Manipal Manual of Surgery [4 ed.] 9788123924168, 812392416X

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Contents Forewords Preface to the Fourth Edition Preface to the First Edition

vii ix xi SECTION I: GENERAL SURGERY

1. Wound, Keloid, Hypertrophic Scar and Metabolic Response to Injury

3

• Types of wounds: Classification • General principles of management of open wounds • Components of wound healing • Factors affecting wound healing • Compartment syndromes • Hypertrophic scar and keloid • Classification of surgical wounds • Healing of specialised tissues • Metabolic response to injury

2. Acute Infections, Sinus, Fistula and

Surgical Site Infection • • • • • • • • • • • • •

Cellulitis Ludwig's angina Lymphangitis Abscess Cervical tuberculous lymphadenitis Boil. carbuncle Erysipelas Chronic abscess Necrotising fasciitis Acute pyomyositis Surgical site infections Asepsis and antisepsis Sinus and fistula

3. Tetanus and Gas Gangrene • • • •

• • • • • •

5. Chronic Infectious Disease • • • • •

15

33

Paronychia Acute lymphangitis Subcutaneous infections Terminal pulp space infections Apical subungual infection Web space infections

41

• • • • • • • • • • • • •

59

Clinical examination Management Wound dressings Traumatic ulcer Venous ulcer Trophic ulcer Tropical ulcer Post-thrombotic ulcer Rare ulcers Diabetic ulcer foot VAC Maggot therapy Pressure sore

7. Lower Limb lschaemia and Popliteal

Aneurysm

• • • • • • • • • • • •

51

Actinomycosis Leprosy Deformities in leprosy Syphilis AIDS

6. Differential Diagnosis of Leg Ulcer and Pressure Sore

Tetanus Gas gangrene Types of gas infections A case report of neck rigidity

4. Hand and Foot Infections

• Deep palmar abscess • Tenosynovitis • Mycetoma pedis Ingrowing toe nail

Lower limb ischaemia Collateral circulation Clinical features Clinical examination Management Differential diagnosis Acute arterial occlusion Critical limb ischaemia Popliteal aneurysm Ainhum Frostbite Reperfusion injuries

76

Manipal Manual of Surgery

xvi • • • •

12. Haemorrhage, Shock and Blood

Fat embolism Air embolism Fontaine classification ICU gangrene

8. Upper Limb lschaemia and Gangrene

Transfusion

98

• • • • • •

Raynaud's disease Thoracic outlet syndrome Axillary vein thrombosis Vasculitis syndrome Gangrene Cancrum oris Acrocyanosis • Drug abuse and gangrene • Iatrogenic drug-induced gangrene • Subclavian steal syndrome

9. Lymphatics, Lymph Vessels and

Lymphoma

• • • • • • • • • • • •

Lymphoedema-anatomy and physiology Lymphatic circulation Primary lymphoedema Secondary lymphoedema Lymphangiography Hodgkin's lymphoma Non-Hodgkin's lymphoma Burkitt's lymphoma Sezary's syndrome Chyluria lmmunohistochemistry Bone marrow and peripheral blood stem cell transplants

10. Varicose Veins and Deep Vein Thrombosis • • • • • • • • • • • •

Classification Premalignant lesions Basal cell carcinoma Squamous cell carcinoma Melanocytic tumours Malignant melanoma Other malignant skin tumours Other skin lesions

• Haemorrhage - Classification - Pathophysiology - Management • Shock - Classification - Pathophysiology - Management • Acute adrenal insufficiency • Hyperbaric oxygen • Central venous pressure • Pulmonary capillary wedge pressure • Blood transfusion • Blood products • Plasma substitutes • Bleeding disorders

13. Burns, Skin Grafting and Flaps • • • • • •

127

• • • • • • • • • • • • • • •

194

Basic definitions Henderson-Hasselbalch equation Regulation of acid-base balance Acid-base disorders Rapid interpretation of an ABG report Normal physiology Water regulation Disturbances of volume Regulation of sodium concentration Disturbances in concentration Disturbances in composition of body fluids Clinical notes Perioperative fluid therapy Special purpose solutions Nutrition

15. Tumours and Soft Tissue Sarcoma

144

185

Burns Free skin grafting Flaps Electrical burns Chemical burns Hydrotherapy

14. Acid-Base Balance, Fluid and Electrolytes

Primary varicose veins Secondary varicose veins Surgical anatomy of venous system of legs Anatomy of the long saphenous vein Clinical examination Treatment Complications Short saphenous varicosity Deep vein thrombosis Recurrent varicose veins Pelvic congestion syndrome Pulmonary thromboembolism

11. Skin Tumours • • • • • • • •

110

17(

• Benign tumours - Papilloma - Fibroma - Lipoma • Neural tumours - Neuroma - Neurofibroma - Neurilemmoma - Chordoma • Malignant tumours - Paraneoplastic syndromes • Soft tissue sarcomas - Differential diagnosis of soft tissue sarcoma

213

Contents

-

Liposarcoma Malignant fibrous histiocytoma Synovial sarcoma Angiosarcoma Rhabdomyosarcoma Kaposi's sarcoma Dermatofibrosarcoma protuberans

16. Cystic Swellings, Neck Swellings and Metastasis Lymph Node Neck

• • • • • , • • • •

Cystic swellings Neck swellings Differential diagnosis of midline swellings Swellings in the submandibular triangle Swellings in the carotid triangle Swellings in the posterior triangle AV fistula Secondaries in the neck Different types of neck dissections Pancoast's tumour

17. Oral Cavity, Odontomes, Lip and Palate

• • • • • • • • • • • • • • • •

272

• Surgical anatomy • Physiology

Thyroid function tests Clinical examination Goitre Multinodular goitre Retrosternal goitre Graves' disease Malignant tumours Anaplastic carcinoma Solitary nodule Thyroiditis Lingual thyroid Ectopic thyroid

• • • • • • • • • • • • • • • • • • •

302

• , • • • •

319

• • • • • • • • •

361

Surgical anatomy Congenital anomaly Physiology-calcium and action of PTH Tetany Hyperparathyroidism Acute hypercalcaemic crisis Adrenal glands-anatomy, physiology Disorders of adrenal cortex Neuroblastoma Phaeochromocytoma lncidentaloma

21. Breast

Surgical anatomy Acute parotitis Submandibular sialoadenitis Salivary gland tumours Summary of malignant tumours Frey's syndrome Sjogren's syndrome Mikulicz disease Parotid fistula Surgery for facial nerve palsy Peripheral nerve repair and transfers

19. Thyroid Gland

• • • • • • • • , • • •

20. Parathyroid and Adrenals

Oral cancer Premalignant conditions General principles Carcinoma of buccal mucosa Carcinoma of tongue Ulcers of tongue Carcinoma of lip Carcinoma maxillary antrum Nasopharynx-cancer Benign lesions in the oral cavity Odontomes Epulis Median mental sinus Vincent's angina Cleft lip and cleft palate Ectopic salivary gland tumour Mucous cysts

18. Salivary Glands

• • • • • • • • • • •

232

xvii

377

Congenital anomalies Surgical anatomy Cystic swellings of breast-classification Acute bacterial mastitis Antibioma Retromammary abscess Phylloides tumour lntracystic carcinoma of breast Aberrations of normal development and involution Fibroadenoma Duct ectasia-plasma cell mastitis idiopathic granulomatous mastitis Macrocysts Galactocoele Galactorrhoea Duct papilloma Carcinoma of the breast Effects of lymphatic obstruction from carcinoma breast Breast reconstruction Carcinoma of male breast Prophylactic mastectomy Mondor's disease Angiosarcoma breast Disorders of augmentation mammoplasty Rare breast cancers A case of carcinoma breast Recent changes in the treatment of carcinoma breast

Manipal Manual of Surgery

xviii

SECTION II: GASTROINTESTINAL SURGERY

22. Oesophagus and Diaphragm

429

• Surgical anatomy • Physiology GORD • Motility disorders • Achalasia cardia Nutcracker oesophagus • Carcinoma • Stricture Perforations • Diverticulum • Dysphagia • Surgical anatomy of the diaphragm • Diaphragmatic hernia • Trachea-oesophageal fistula

23. Stomach and Duodenum • • • • • • • • • • • • • • • • • • •

Surgical anatomy Gastric physiology H. pylori infection Gastritis Peptic ulcer disease Acute complications of peptic ulcer Chronic complications of peptic ulcer Carcinoma stomach Gastrointestinal stromal tumours (GISTs) Gastric lymphoma Complications of gastrectomy Acid function tests Acute dilatation of the stomach Volvulus of the stomach Bezoars Idiopathic hypertrophic pyloric stenosis Chronic duodenal ileus Duodenal anatomy and obstruction Gastric surgery for morbid obesity

24. Liver

• • • • • • • • • • • •

460

Surgical anatomy Physiology Pyogenic abscess Amoebic abscess Hydatid cyst Other cystic diseases Benign tumours Hepatoma Secondaries in the liver Portal hypertension Portal gastropathy Portal bliopathy Ascites in portal hypertension Budd-Chiari syndrome Role of octreotide in surgery Liver transplantation Haemobilia

516

25. Gall Bladder and Pancreas

55(

26. Spleen

617

• • • • • • • • • • • • • • • • • • • • • • • • • •

• • • • • • • • • • • • • • • •

Surgical anatomy Physiology Congenital anomalies Gall stones disease Acute cholecystitis Chronic cholecystitis Obstructive jaundice Stricture CBD Sclerosing cholangitis Choledochal cyst Caroli's disease Chronic pancreatitis Cholangiocarcinoma Congenital biliary atresia Carcinoma of gall bladder Carcinoma of pancreas Endocrine tumours Acute pancreatitis Pseudocyst Annular pancreas Ectopic pancreas Cystic fibrosis Pancreatic divisum Pancreatic fistula White bile Pancreatic ascites

Introduction Surgical anatomy Functions of the spleen Congenital abnormalities Rupture Complications of splenectomy ITP Hereditary spherocytosis Acquired autoimmune haemolytic anaemia Thalassaemia Sickle cell anaemia Splenectomy for other conditions Splenic artery aneurysm Hairy cell leukaemia OPSI Interesting 'most common'

27. Peritoneum, Peritoneal Cavity, Mesentery and Retroperitoneum • • • • • • • •

Peritoneum Acute peritonitis Laparostomy Abdominal compartment syndrome Subphrenic abscess Special types of peritonitis Omentum Mesentery

634

Contents

• • • •

Misty mesentery Mesenteric cyst Retroperitoneum Retroperitoneal cyst, abscess, tumour

28. Small Intestine

• • • • • • • • • • • • • • • • •

Anatomy Physiology Abdominal tuberculosis Tuberculous peritonitis Glandular tuberculosis Intestinal tuberculosis Inflammatory bowel diseases lleostomy Surgical complications of enteric fever Intestinal amoebiasis Radiation enteropathy Peutz-Jegher syndrome GIST Carcinoid tumour Short gut syndrome Diverticula Intestinal fistula

29. Large Intestine

• • • • • • • • • • •

• Surgical anatomy • Carcinoma rectum

Prolapse rectum Surgical anatomy of anal canal Haemorrhoids Anorectal abscess Fistula in ano Fissure in ano

• • • • •

Pilonidal sinus Sacrococcygeal teratoma Malignant tumours of anal canal Stricture of anal canal and rectum Anal incontinence

.

• • • • • •

VAAFT

700

• • • • • • • • • •

729

772

824

Development and anomalies Surgical anatomy Acute appendicitis Differential diagnosis Appendicular mass Complications Neoplasm Mucocoele Faecal fistula Valentino appendix Post-appendicectomy sepsis-case report

34. Hernia

• • • • • • • • • • • • • • • • • • • •

812

Causes Clinical examination Investigations Exploratory laparotomy Haemobilia Angiodysplasia

33. The Appendix

Sigmoid volvulus Meckel's diverticulum Adhesions and bands Gall stone ileus lntussusception Mesenteric vascular occlusion Hirschsprung's disease Atresia and stenosis Arrested rotation with bands Volvulus neonatorum Meconium ileus lmperforate anus Food bolus obstruction Paralytic ileus Malrotation and midgut volvulus

31. Rectum and Anal Canal

• • • • • •

32. Lower Gastrointestinal Bleeding

Surgical anatomy Function Tumours Familial polyposis coli Hereditary nonpolyposis Colorectal cancer Carcinoma Colon screening Diverticular disease Faecal fistula Colonic stricture

30. Intestinal Obstruction

• • • • • • • • • • • • • • •

666

xix

842

Anatomy Indirect hernia Direct hernia Clinical examination Complications Recurrent hernia Special hernias Giant hernia Sportsmen hernia Sliding hernia Femoral hernia Rare types of femoral hernia Umbilical hernia lncisional hernia Epigastric hernia lnterparietal hernia Spigelian hernia Lumbar hernia Obturator hernia Perinea! hernia

35. Umbilicus and Abdominal Wall

• Classification of umbilical diseases

877

Manipal Manual of Surgery

xx • • • • • • • • •

• • • • • • •

Umbilical inflammation Umbilical fistuloe Umbilical neoplasms Abdominal dehiscence Divaricotion of recti Rectus sheath haematoma Meleney's gangrene Desmoid tumour Endometriosis

37. Abdominal Mass

36. Blunt Abdominal Trauma, War and Blast Injuries and Triage • • • •

Pancreatic injuries Renal injuries Retroperitoneol haematoma Blast injuries Warfare injuries Missile wounds of abdomen Triage

885

Liver injuries Small bowel injuries Colonic injuries Duodenal injuries

• • • • • •

900

Clinical examination of abdominal moss Moss in the right iliac fosso Firm to hard nodular moss in the umbilical region The cystic moss in the abdomen Moss in the epigostrium Moss in the right hypochondrium

SECTION 111: UROLOGY

38. Investigations of the Urinary Tract • • • • • • • • • • •

Urine Blood Intravenous urogrom Retrograde pyelogrophy Renal orteriogrophy Cystourethrogrophy Urethrogrophy Ultrosonogrophy Computerised tomography Radioisotope scanning Endoscopy

39. Kidney and Ureter • • • • • • • • • • • •

Surgical anatomy Vesicol calculus Carcinoma of bladder Ectopio vesicoe Acute cystitis Diverticulo Urinary fistuloe Interstitial cystitis Schistosomo hoemotobium Urinary diversion Rupture bladder

• • • • • •

Surgical anatomy of urethra Rupture urethra Stricture urethra Hypospodios Retention of urine Posterior urethral valve

41. Prostate and Seminal Vesicles

932

Surgical anatomy Polycystic kidneys Horseshoe kidney Renal stones Ureteric stone Hydronephrosis Renal tuberculosis Wilms' tumour Pyonephrosis Perinephric abscess Dialysis Renal transplantation

40. The Urinary Bladder and Urethra • • • • • • • • • • •

923

• • • • • •

Surgical anatomy Structural anatomy Benign prostotic hyperplasia (BPH) Carcinoma of the prostate Gleason's score Prostotitis

42. Penis, Testis and Scrotum

954

• • • • • • • • • • • • • • • •

981

Surgical anatomy of penis Phimosis Parophimosis Carcinoma of penis Peyronie's disease Anatomy of the testis Hydrocoele Undescended testis Ectopic testis Varicocoele Spermotocoele Epididymol cyst Torsion testis Testicular tumours Fournier's gangrene Fracture penis

43. Differential Diagnosis of Haematuria • • • • •

972

Causes History and examination Investigations Hoemoturio History

1002

Contents

xxi

SECTION IV: SPECIALITIES 44. Chest Trauma Cardiothoracic Surgery • • • • • • • • • • • • • • •

Chest trauma Blunt trauma Pulmonary injuries Tracheobronchial injuries Myocardial contusion Surgical emphysema Mediastinal emphysema Mediastinal masses Pulmonary aspergilloma Congenital heart diseases Patent ductus arteriosus Coarctation of aorta Coronary artery bypass graft Off pump coronary artery bypass surgery Abdominal aortic aneurysms (AAA)

45. Neurosurgery • • • • • • • • • •

• • • • •

Barium swallow Barium meal Barium meal follow through Barium enema Enteroclysis Angiography Splenoportography Sialography Computed tomography Ultrasonography Magnetic resonance imaging Peripheral venography Myelography lnterventional radiology PET scan Virtual colonoscopy

47. Principles of Clinical Radiation Oncology • Radiation • Dose fractionation

• • • • • • •

Sources and methods Measurement Clinical uses Curative treatment Palliative treatment Radiotherapy reactions Advances in radiation therapy

48. Principles of Anaesthesiology

1035

Classification of head injuries Primary lesions Secondary lesions Extradural/epidural haematoma Chronic subdural haematoma Raised intracranial pressure Fracture skull CSF rhinorrhoea Pott's puffy tumour Hydrocephalus Brain tumours Brainstem death

46. Principles of Radiology • • • • • • • • • •

1009

• • • • • • • • • • •

Preoperative assessment General anaesthetic agents Muscle relaxants Endotracheal intubation Monitoring Local anaesthetics Regional anaesthesia Pain relief Complications of anaesthesia Cardiopulmonary resuscitation What is new?/Recent advances

49. Organ Transplantation

1047

1061

1071

PRINCIPLES OF TRANSPLANTATION • Introduction • Donor selection • Recipient evaluation • Rejection • lmmunosuppression LIVER TRANSPLANTATION • Indications • Contraindications • Selection criteria • MELD score • Types • Techniques • Complications • Role of cellular transplantation SMALL BOWEL TRANSPLANT • Indications • Contraindications • Pre-transplant recipient evaluation • Evaluation and management • Types and operative procedure • Details of the techniques • Complications ISLET CELL TRANSPLANTATION • Indications • Contraindications • Pre-transplant recipient evaluation • Evaluation and management • Types and operative procedure • Details of the techniques • Complications • Indications • Contraindications

1108

Manipal Manual of Surgery

xxii

SECTION V: VIVA VOCE EXAMINATION 50. X-rays and Images • Plain X-rays • Barium swallow • Barium meal • Barium enema • ERCP • T-tube cholangiography • Splenoportovenography (SPV) • CT scan • PET scan

1119

51. Instruments

1129

• • • • • • •

Forceps Retractors Occlusion clamps Dilators Tracheostomy tube Rubber tubes Catheters Sengstaken tube

52. Specimens

• • • • • • • • • • • • •

TB lymphadenitis Hodgkin's lymphoma Carcinoma tongue Chronic gastric ulcer Linitis plastica lntussusception Carcinoma rectum Gangrenous appendicitis Carcinoma colon Meckel's diverticulum Polycystic kidney Renal cell carcinoma Hydronephrosis

Index

1142

• • • • • • • • •

Carcinoma penis Seminoma testis Cholecystectomy Hydatid cyst Carcinoma stomach Lipoma Malignant melanoma Thyroidectomy Wide excision of skin

53. Operative Surgery, Laparoscopic Surgery 1151 and Accessories • Appendicectomy • Herniorrhaphy: Bassini • Surgery for hydrocoele • Incision and drainage (I & D) • Incision of drainage of breast abscess • Circumcision • Venesection or cut down • Vasectomy • Tracheostomy • Suprapubic cystostomy (SPC) • Thyroidectomy • Amputations • Amputations in leg • Upper limb amputations • Open cholecystectomy • Vagotomy gastrojejunostomy (GJ) • Excision of swellings • Colonic surgery • Laparoscopic surgery • High frequency (HF) electrosurgery • Cryosurgery • Lasers in surgery • Staplers in surgery

1183

by Dr. Nigam Rashmi Dhar for free pdf ebooks notes mcq join free whatsapp medical group 009779804202884

Section

General Surgery 1. 2. 3. 4. 5. 6. 7. 8. 9. 10. 11. 12. 13. 14. 15. 16. 17. 18. 19. 20. 21.

Wound, Keloid, Hypertrophic Scar and Metabolic Response to Injury Acute Infections, Sinus, Fistula and Surgical Site Infection Tetanus and Gas Gangrene Hand and Foot Infections Chronic Infectious Disease Differential Diagnosis of Leg Ulcer and Pressure Sore Lower Limb lschaemia and Popliteal Aneurysm Upper Limb lschaemia and Gangrene Lymphatics, Lymph Vessels and Lymphoma Varicose Veins and Deep Vein Thrombosis Skin Tumours Haemorrhage, Shock and Blood Transfusion Burns, Skin Grafting and Flaps Acid-Base Balance, Fluid and Electrolytes Tumours and Soft Tissue Sarcoma Cystic Swellings, Neck Swellings and Metastasis Lymph Node Neck Oral Cavity, Odontomes, Lip and Palate Salivary Glands Thyroid Gland Parathyroid and Adrenals Breast

by Dr. Nigam Rashmi Dhar for free pdf ebooks notes mcq join free whatsapp medical group 009779804202884

1 Wound, Keloid, Hypertrophic Scar and Metabolic Response to Injury • Types of wounds: Classification • General principles of management of open wounds • Components of wound healing • Factors affecting wound healing • Compartment syndromes

• • • • •

Hypertrophic scar and keloid Classification of surgical wounds Healing of specialised tissues Metabolic response to injury What is new?/Recent advances

B. Open wounds • Incised • Lacerated • Penetrating • Crushed

Introduction Wound is a discontinuity or break in the surface epithelium. A wound is simple when only skin is involved. It is complex when it involves underlying nerves, vessels and tendons. Types of wounds

Incised wounds: They are caused by sharp objects such as knife, blade, glass, etc. This type of wound has a sharp edge and is less contaminated. Primary suturing is ideal for such wounds, as it gives a neat and clean scar.

A few classifications have been given below.

I A. Closed wounds • Contusion • Abrasion • Haematoma

Lacerated wounds: They are caused by blunt injury such as fall on a stone or due to road traffic accidents (RTA). Edges are jagged. The injury may involve only skin and subcutaneous tissue or sometimes deeper structures also. Due to the blunt nature of the object, there is crushing of the tissue which may result in haematoma, bruising or even necrosis of the tissue. These wounds are treated by wound excision and primary suturing provided they are treated within six hours of injury.

Contusion: Can be minor soft tissue injury without break in the skin, or major such as when being run over by a vehicle. Generally, it produces discolouration of the skin due to collection of blood underneath. Abrasion: In this wound, epide1mis of the skin is scraped away exposing the dermis. They are painful as dermal nerve endings are exposed. These wounds need cleaning, antibiotics and proper dressings.

Penetrating wounds: They are not uncommon. Stab injuries of abdomen are very notorious. It may look like an innocent injury with a small, 1 or 2 cm long cut but internal organs such as intestines, liver, spleen or mesenteric blood vessels may have been damaged. All penetrating wounds of the abdomen should be admitted and observed for at least 24 hours. Layer by layer exploration and repair, though recommended, may not be possible at times due to oblique track of the wound.

Haematoma: This refers to collection of blood usually following injury. It can occur spontaneously in patients who have bleeding tendencies such as haemophilia. Depending upon the site, it can be subcutaneous, intramuscular or even subperiosteal. A knee joint haematoma may need to be aspirated followed by application of compression bandage. Small haematomas get absorbed. If not, they can get infected.

Crushed or contused wounds: They are caused by blunt trauma due to run over by vehicle, wall collapse, earthquakes 3

Manipal Manual of Surgery

4

or industrial accidents. These wounds are dangerous as they may cause severe haemorrhage, death of the tissues and crushing of blood vessels. These patients are more prone for gas gangrene, tetanus, etc. Adequate treatment involves good debridement and removal of all dead and necrotic tissues. II Tidy and untidy wounds A. Tidy wounds: Incised, clean, healthy tissue and seldom associated with tissue loss (Key Box 1.1). B. Untidy wounds: Crushed or avulsed, contaminated, devitalised tissues and often with tissue loss.

.KEY BOX .1 ••.•••.•.....•....... 0

1

REPAIR IN TIDY WOUND • Nerves: Fascicular repair under magnification (loupe or microscope) using 8-0 or 10-0 monofilament nylon • Artery: To be repaired by using 6-0 prolene • Tendon repaired by monofilament nonabsorbable suture (polypropylene 3-0 or 4-0) • Skin loss: Skin flap/skin graft

Ill Acute wound and chronic wound A. Acute wound: Stab wounds, following RTA and blast injuries. B. Chronic wound: Leg ulcers, pressure sores. General principles of management of open wounds (Fig. 1.1)

Healing of the wound Healing by primary intention occurs in a clean incised wound such as a surgical incision wherein there is only a potential space between the edges. It produces a clean, neat, thin scar. Healing by secondary intention refers to a wound which is infected, discharging pus or wound with skin loss. Such wounds heal with an ugly scar. COMPONENTS OF WOUND HEALING (Table 1 .1) I. Inflammatory phase (lag phase) • Injury results in the release of mediators of inflammation, mainly histamine from platelets, mast cells and granulocytes. This results in increased capillary permeability. • Later kinins and prostaglandins act and they play a chemotactic role for white cells and fibroblasts. • In the first 48 hours, polymorphonuclear (PMN) leukocytes dominate. They play the role of scavengers by removing the dead and necrotic tissue (Figs 1.2 to 1.6). II. Proliferative phase (collagen phase) • Between 3rd and 5th days, polymorphonuclear leukocytes diminish in number but monocytes increase. They are the specialised scavengers. • By 5th or 6th day, fibroblasts appear, proliferate and eventually give rise to a protocollagen which is converted

Wound

Cleaning and bandage

Wound Inflammatory phase

Active bleeding Stop the bleeding

Splint, if there is fracture

Vasoconstriction + Thrombus Haemostasis

IV line, resuscitation

Platelets release Suturing

Transport

Fig. 1.1 : Wound management

• Admission or observation in the hospital. • Monitoring of temperature, pulse and respiration. • Systemic antibiotics depending upon the contamination of wound. • Injection tetanus toxoid for prophylaxis against tetanus. • Treatment of the wound in the form of cleaning, dressing or suturing.

Platelet-derived growth factor (PDGF)

Platelet factor IV

Transforming growth factor � (TGF-�)

Attract PMN + Macrophages Remove devitalised tissues

Fig. 1.2: Inflammatory and proliferative phases

Wound, Keloid, Hypertrophic Scar and Metabolic Response to Injury

into collagen in the presence of an enzyme, protocollagen hydroxylase. 0 2 , ferrous ions and ascorbic acid are necessary for this step. • Fibroplasia along with capillary budding gives rise to granulation tissue. Protocollagen

g n_ hyd_r_ox_yl_as_e _P_r_ot_ocol_la e -----l� Col I agen ,- ....,. ...,.., ....,. hydroxylation

• Secretion of ground substance-mucopolysaccharides by fibroblasts takes place. These are called proteoglycans. They help in binding of collagen fibres. Thus, wound is Fibre + Gel + Fluid system (resembles Iron Rods + Cement + Water used for concrete slab). • Epithelialisation occurs mainly from the edges of the wound by a process of cell migration and cell multiplication. This is mainly brought about by marginal basal cells. Thus, within 48 hours, the entire wound is re-epithelialised. When there is a wound with skin loss, skin appendages also help in epithelialisation. Slowly, surface cells get keratinised. Ill. Remodelling phase (maturation) It starts after 4 days and is usually completed by 14 days. It is brought about by specialised fibroblasts. Because of their contractile elements, they are called myofibroblasts. It is the nature's way of reducing the size of defect, thereby helping the wound healing. Wound contraction readily occurs when there is loose skin as in back and gluteal region. Skin contraction is greatly reduced when it occurs over tibia (skin)

5

or malleolar surface. Corticosteroids, irradiation, chemo­ therapy delay wound contraction. Connective tissue formation: Formation of granulation tissue is the most impottant and fundamental step in wound healing. (It can be compared to concrete slab laying.) IV. Phase of scar formation Following changes take place during scar formation • Fibroplasia and laying of collagen is increased • Vascularity becomes less (devascularisation) • Epithelialisation continues • Ingrowth of lymphatics and nerve fibres takes place • Remodelling of collagen takes place with cicatrisation, resulting in a scar. Complications of wound healing 1. Infection: It is the most important complication which is responsible for delay in wound healing. Majority of bacteria are endogenous. Depending upon pus/culture sensitivity report, appropriate antibiotics are given. 2. Ugly scar: It is the result of infections 3. Keloid and hypertrophic scar (see page 10) 4. Incisional hernia and wound dehiscence 5. Pigmentation of the skin 6. Marjolin's ulcer (see page 145). WOUND CLOSURE OR WOUND SUTURING 1. Primary suturing: Suturing the wound within a few hours following an injury (six hours is ideal) is called primary suturing. Primary suturing can be done provided:

Wound healing Day 0-1

• Tissue lo ? • Exposure of extracellular matrix to platelets

0-2 days

48-96 hours

Macrophages

• Platelet igregation

• Remove dead and necrotic tissue

• Inflammatory mediators

• Epithelialisation

t

Fig. 1.3: Platelets

t

Fig. 1.4: Polymorph

l

Phagocytosis

5-7 days

Fibroblasts appear

t

Granulation tissue

Bridge the transition of inflammatory to proliferative phase • Role of T-lymphocytes is not clear

• Wound contraction takes place

Fig. 1.5: Macrophage

Fig. 1.6: Lymphocytes

6

Manipal Manual of Surgery

• It is an incised or cut wound with a sharp object such as knife or razor blade. • Minimal injury to structures on either side. • There should not be any infection. If a wound is sutured in the presence of infection, the suture material is eaten away (digested) by organisms which results in gaping of the wound. Precautions to be taken while doing primary suturing:

• Foreign body, if present in the deeper aspect of the wound, should be removed. • Associated injury to blood vessels, nerves or tendons should be recognised and repaired. • Wound on the abdomen may have associated visceral injuries. • Prevention of tetanus by administering tetanus toxoid 0.5 ml intramuscularly.

2. Wound excision and primary suturing of skin: This is indicated when: • Wound edges are jagged. • Wound is contaminated with organisms or foreign body. • Tissues are crushed and devitalised. • In such situations, wound is explored and devitalised tissues and foreign body, if present, are removed. The wound is irrigated with antiseptic agents. Thus, lacerated wound is conve1ted into an incised wound and then sutured. Precautions to be taken are:

• It should be done within 6 hours. • Tetanus and gas gangrene prophylaxis. • Repair of tendons and nerves can be done at a later date, if contamination is excessive.

3. Wound excision and delayed primary suturing: This is indicated in lacerated wounds with major crush injuries. Primary suturing within 6 hours is not done in these wounds because of: • Gross oedema of the part • Increased tissue tension • Haematoma • Contamination with bacteria • In such situations, excision of all dead tissues is done. PEARLS OF WISDOM

Saline is increasingly being used to wash the wound as H2 02 and betadine may cause more damage.

• Wound is irrigated with saline and left open without suturing and dressing is applied. • Wound is re-examined 4-6 days later. If there is no infection, or no nonviable tissues, wound is sutured. This two-stage procedure is called delayed primary suturing.

Fig. 1.7: Wound with skin loss

Wound with skin loss (Fig. 1.7): It can follow surgical procedures or accidents, etc. Principles of debridement

• • • • •

Ideally done under general anaesthesia Assess the extent of injury/loss of tissues Control bleeding Excision of devitalised tissue better done with scissors Good saline wash/irrigation is better than betadine/hydrogen peroxide wash.

PEARLS OF WISDOM

Aim is to convert an untidy wound into a tidy wound. Complications of skin loss • Secondary infection of the wound. • The underlying structures such as tendons and nerves are in danger. • Diabetic patients can develop septicaemia. • Deformity and disability can occur at a later date. Hence, skin grafting should be done as soon as possible.

4. Secondary suturing: After operations, sutures may give way because of severe infection with persistent discharge of pus. In such cases 7-14 days later, after controlling infection, the skin is freed from the edge of the wound and the granulation tissue and skin are approximated. This type of suturing is called as secondary suturing. FACTORS AFFECTING WOUND HEALING General factors 1. Age: In children, wounds heal faster. Healing is delayed in old age. Dermal collagen content decreases with aging. Also, collagen fibres show distorted architecture and organisation.

Wound, Keloid, Hypertrophic Scar and Metabolic Response to Injury

2. Debilitation results in malnutrition. Wound healing is delayed probably because of vitamin C deficiency. Following injury, vitamin C deficiency can occur after 3-4 weeks. Vitamin C is necessary for the synthesis and maintenance of collagen. Zinc deficiency is known to delay the healing of pilonidal sinus. Zinc deficiency is rare-it occurs in large burns, severe polytrauma and hepatic cirrhosis. 3. In diabetic patients, wound healing is delayed because of several factors such as microangiopathy, atherosclerosis, decreased phagocytic activity, proliferation of bacteria due to high blood sugar, etc. (Key Box 1.2) Also poor immune response is seen in diabetic patients.

IMl:t----.W� DIABETES AND WOUND HEALING FACTORS

Tissue hypoxia due to atherosclerosis Thickened basement membrane�! tissue perfusion Trauma-repetitive due to neuropathy Tissue metabolism is increased-relative hypoxia Total failure of defence mechanism Observe 5 Ts

Local factors 1. Poor blood supply: Wound over the knee and shin of tibia

heals very slowly but wound on the face heals fast.

2. Local infection: Organisms eat away the suture material, destroy granulation tissue and causes slough and purulent discharge. If the bacterial count exceeds 10 5 organisms/ mg tissue or if any �-hemolytic streptococci are present, wound does not heal. Collagen synthesis is reduced and collagenolysis is increased. Antibiotics should be given immediately or within 2 hours to prevent infection. Infection Prolong inflammatory phase Endotoxins release collagenase Epithelialisation, contraction, collagen depositions are disturbed

Collagen degradation, wound damage

4. Jaundiced and uraemic patients have poor wound healing because fibroblastic repair is delayed.

3. Haematoma precipitates infection.

5. Cytotoxic drugs such as doxorubicin and malignancy delay healing (Key Box 1.3).

5. Tension while suturing.

Nl=t----.r� • • • • •

CHEMOTHERAPEUTIC DRUGS Decrease mesenchymal cell proliferation Reduce number of platelets Reduce inflammatory cells Reduce growth factors Decrease wound breaking strength

6. Generalised infection: Pus in some part of body delays wound healing. 7. Corticosteroids given early may delay wound healing because of their anti-inflammatory activity. Once healing is established, they do not interfere. 8. Malnutrition: Definitely results in delayed wound healing including intestinal anastomotic leakage and wound dehiscence. PEARLS OF WISDOM

Albumin levels must be less than 2 g /dL to have an effect on wound healing.

7

4. Faulty technique of wound closure. 6. Hypoxia: Killing property of macrophages and production of fibroblasts can decrease due to hypoxia. If contamination occurs, oxygen level in tissue decreases. • Collagen synthesis is affected in cases of hypoxia. In anaemic patients, wound healing is delayed because of decreased angiogenesis and decreased collagen production. • Smoking causes vasoconstriction and elevated carbon monoxide levels. 7. Ionising radiation: It causes endothelial cell injury with endarteritis and results in atrophy and fibrosis. • Typically occur in closed lower limb injuries. • Following injuries, inflammatory reaction results in gross oedema of the region. • These are the tight undividing compartments in the leg containing nerves and vessels. PEARLS OF WISDOM

The most prone site is the anterior compartment. Paraesthesia or numbness between 1st and 2nd toes occurs due to pressure on the deep peroneal nerve and is diagnostic.

Manipal Manual of Surgery

8

Clinical features • Severe pain in the leg • Sensory disturbances (Fig. 1.8) • Colour changes-absence of pulses is a late sign. Measuring compartmental pressure • It is measured using a catheter placed in the muscle compartment and a pressure transducer. • Compartmental pressure greater than 30 mmHg is an indication for urgent fasciotomy. Treatment-Fasciotomy • 8-10 cm incisions, each being lateral to subcutaneous border of the tibia (Fig. 1.9). • Once deep fascia is incised, muscle bulges out. Soleus must be detached from tibia to decompress deep flexor compartment. • All compartments of the leg can be approached by these mc1s1ons. • Most important motto should be to preserve blood supply by releasing compression on posterior tibial and peroneal arteries. • Infection and amputation are frequent outcomes. PEARLS OF WISDOM

found. These two conditions represent variations in th� normal process of wound healing (Table 1.2). • Keloid is very common in blacks and least common ir Caucasians (Key Boxes 1.4 and 1.5).

M=t..._..,, "§ TYPES OF SCAR

• Atrophic • Hypertrophic • Keloid

Uf=ff--.....r,, "§ AETIOLOGY OF KELOID

• • • • • •

Key factors: Surgery, burns, vaccinations

Elevated levels of growth factor (more of type 'B' collagen) Laceration or abrasion Over the sternum (incision) Inheritance and injection Deep pigmented skin Remember as KELOID

HYPERTROPHIC SCAR AND KELOID

• Keloid is not a true tumour but has a marked tendency for local recurrence after excision. • Keloid takes the shape of a butterfly over the sternum. It is the commonest site for a keloid. It is extremely difficult to treat the keloid over the sternum. We had one patient who underwent wide excision and grafting 6 times for a sternal keloid. Jaw, vaccination are common sites (Figs 1.10 to to 1.13 and Key Box 1.6).

• As the name suggests, there is hypertrophy of mature fibroblasts in hypertrophic scar. Blood vessels are minimal in this condition. However, in keloid, proliferation of immature fibroblasts with immature blood vessels are

What is a contracture? When scar crosses 'joints or flexion creases, a tight 'web' may form which is referred to as a contracture (see Bums Chapter for more details).

The danger lies in the delay, not in the simplefasciotomy. ln crush injuries who present late to the hospital, it is safer to do amputation because by doing fasciotomy sudden release of myoglobin from dead muscle may cause systemic myoglobinuria, glomerular blockage and renal failure.

Fig. 1.8: Paraesthesia between first and second toes

Fig. 1.9: Fasciotomy

Fig. 1.10: Keloid over the sternum

Wound, Keloid, Hypertrophic Scar and Metabolic Response to Injury

9

Comparison of hypertrophic scar and keloid (Figs 1.11 to 1.13) Keloid

Hypertrophic scar General features

• • • •

It occurs from a prolonged inflammatory phase of wound healing. It never gets worse after 6 months. Itchjng is not usually present. If present, it is not severe. Nontender Not vascular Does not extend beyond the boundary of the original incision or wound. lt rises above skin.

Precipitating factors

• Scar crossing nonnal sbn creases • Over sternum, over joints • Young persons

• It continues to get worse even after I year and up to a few years. • • • •

Severe itching is present Margin is tender Vascular, red, erythematous (immature blood vessels) Extends to normal tissues, has claw-like process. Hence the name.

• • • • • •

Black race Tuberculosis patients Incision over the sternum, ear lobe Equal in both sexes Hereditary and familial Vaccination sites, injection sites, incision sites, piercing sites

Natural history

• May become small

• Does not become small

Complications

• Do not occur

• Ulceration, infection

PEARLS OF WISDOM

Any form of excision has high chance of recurrence

Treatment

• • • • • •

It is often not necessary Stocbng, armlets, gloves Elastic bandage may help Excision can be done Silicone application Topical retinoids

Fig. 1.11: Keloid over the jaw

• It is difficult. Injection of steroid preparation such as triamcino­ lone acetate (Kanacort) has been found to be extremely useful. It flattens the keloid. Intrakeloidal excision and sbn grafting is to be tried last. Recurrence is common. Care should be taken not to extend the incision on to the normal surrounding tissues. • Silicone application • Topical retinoids

Fig. 1.12: Recurrent keloid over the sternum. Excision attempted three times

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Manipal Manual of Surgery

. . . .= . .

lffl !---..r� KELOID SITES

High chances

Least chances

Skin of ear lobe

Eyelid

Presternal

Genitalia

Deltoid

Palm, sole

Upper back

Across joint

SURGICAL WOUNDS Surgical wounds may be classified depending upon the nature of the wound, whether clean or contaminated (Table 1.3 and Figs 1.14 to 1.17).

PEARLS OF WISDOM

Collagen bundles are virtually absent in keloid. It is extremely difficult to treat a keloid.

Fig. 1.14: Excision of neck swelling-clean

Fig. 1.13: Keloid over vaccination site

Fig. 1.15: Cholecystectomy-clean contaminated

Classification of surgical wounds Wounds class Clean (Fig. 1.14)

Clean contaminated (Fig. 1.15) Contaminated (Fig. 1.16)

Dirty (Fig. 1.17)

Definition

.. . . .. . ...

Nontraumatic Elective surgery Gastrointestinal, respiratory or genitourinary tract not entered. Respiratory, genitourinary or gastrointestinal tracts entered but minimal contamination Open, fresh, traumatic wounds Uncontrolled spillage from an unprepared viscus Minor break in sterile technique Open, traumatic dirty wounds Traumatic perforated viscus Pus in the operative field

Examples of typical procedures

... . . .. .

Mastectomy Vascular procedures Thyroidectomy

Gastrectomy Hysterectomy Cholecystectomy Ruptured appendix Resection of unprepared bowel Resection of gangrene

Wound infection rate(%) 2

Usual organism Staphylococcus aureus

200 mg/di), and hypertriglyceridaemia, hypercoagulable state (polycythaemia) and hyperhomocysteinaemia. • Known diabetes: Specially type 2 diabetes mellitus­ increases the risk by two to four times.

Causes of lower limb ischaemia Chronic lower limb ischaemia Fig. 7.1: Atherosclerosis with diabetes resulting in wet gang­ rene - toe has been amputated

Fig. 7.2: Dry gangrene due to atherosclerotic arterial disease­ part is dry, mummified and toe has been amputated

76

Acute lower limb ischaemia

• Acute thrombosis • Atherosclerosis • Thromboangiitis Obliterans • Acute embolism • Trauma to the vessels (TAO) • Collagen vascular disorders • Aneurysm • Diabetes (diabetic foot-neuro ischaemic foot)

Lower Limb lschaemia and Popliteal Aneurysm

• Elderly patients between 60-70 years are vulnerable • Raised blood pressure-hypertension You can remember as SMOKER Hyperhomocysteinaemia It increases the risk of developing PAD by 7% Homocysteine levels more than 15 µmoll! • Increased levels cause endothelial injury and leads to vascular inflammation • Defective gene methylene tetrahydrofolate reductase or MTHFR • Prevention is by eating foods containing B6, B9 and B 12 and with folate, such as potato, green vegetables, fish, etc. It is also a strong risk for myocardial infarction in young patients. Collateral circulation Collateral circulation is present in most of the organs. Hence, even if a major vessel is occluded, the organ can still survive provided collaterals are well-developed. Chronic ischaemia caused by TAO or atherosclerosis, allows sufficient time for collaterals to develop. Hence, necrosis or gangrene that occurs is minimised. Thus, limbs often survive (Figs 7.3 and 7.4). In acute ischaemia caused by thrombus or embolism, there is no time for collaterals to develop. This results in gangrene of the I imb, in untreated cases.

77

,.,�� ......

GRADES OF INTERMITTENT CLAUDICATION-BOYD'S CLASSIFICATION

Grade I : The patient walks for a distance, gets the pain,

continues to walk and the pain disappears. As a result of ischaemia, anaerobic metabolism takes place, which produces substance P, lactic acid, etc. These produce vasodilatation and the pain disappears.

Grade II: The patient walks for a distance, gets the pain and continues to walk with the pain. He has a limp.

Grade Ill: The patient walks and gets the pain. He has to take rest. This grade indicates severe muscle ischaemia.

In late stages: Pain at rest is due to ischaemia of nerves* in addition to ischaemia of the muscles. *Cry of the dying nerves, due to involvement of vasa nervosum Pain is due to ischaemic neuropathy involving small unmyelinated A delta and C sensory fibres.

ischaemia of the foot with impending gangrene. Typically, a patient with rest pain sits on the bed, holds his foot with both hands or may hang the foot out of bed. This gives him some kind of relief. Rest pain is worse at night time 1• It may lead to suicidal tendency. • Claudication distance refers to the distance a patient is able to walk before the onset of pain. A patient with severe claudication may not be able to walk even a few yards. • Site of claudication depends upon the level of arterial occlusion (Table 7.2). • Also see Table 7.3, for other causes of pain in the leg.

Fig. 7.3: Sudden occlusion. Embolic-no time for collaterals to develop

Fig. 7.4: Slow occlusion (atherosclerotic). Collaterals develops

CLINICAL FEATURES-SYMPTOMS 1. Pain in the limb is the chief symptom of lower limb ischaemia. It is a severe cramp-like pain, due to ischaemia of the muscles brought on mainly by exertion. It is called intermittent claudication (Key Box 7.1). Rest pain 1: It is an intractable type of pain usually felt in the foot (instep), toes, etc. It is an indication of severe

2. Nonhe aling ulcer is the next common presenting symptom. It is usually precipitated by a minor trauma and it occurs in the most distal part of the body such as the tip of toes. Ischaemic ulcers are deep and very pain­ ful.

Level of occlusion

Claudication site

Aortoiliac obstruction

Claudication of both gluteal regions, thighs and calves

Iliofemoral obstruction

Claudication of thigh muscles

Femoropopliteal obstruction

Claudication of calf muscles

Popliteal obstruction

Claudication of the foot muscles, instep claudication

10ne of our TAO patients with sleepless nights due to rest pain committed suicide by jumping from the 2nd floor of the hospital.

78

Manipal Manual of Surgery Important causes of pain in the leg

Condition

Aetiology

Nature of pain

Location

• Intermittent claudication • Neurogenic claudication • Venous claudication (DVT) • Varicosity

Inadequate skeletal muscle perfusion Lumbosacral nerve root compression Proximal venous occlusion thrombosis Long saphenous varicosity

Burning or cramp like Diffuse radiating deep ache with or without paraesthesia Bursting

Calf muscles, thigh or gluteal region. Relieved on rest Pain on first step, decreased on sitting or bending over while walking Engorgement during exercise

Diffuse aching/heaviness

Decreases on walking due to calf muscles pump

PEARLS OF

WISDOM

Intermittent claudication in a young patient can be due to some rare causes such as • Popliteal artery entrapment due to abnormal origin of gastrocnemius muscle • Cyst in the media of the popliteal artery • Hyperhomocysteinaemia.

3. Some patients present with gangrenous patches of skin or subcutaneous tissue. Gangrene affects distal parts such as toes. However, gangrene is minimal because of collaterals (Fig. 7 .5). 4. History of bilateral gluteal claudication with impotence can occur in a young patient due to a saddle thrombus at the bifurcation of aorta. It is called Leriche's syndrome. Impotence is due to failure to achieve an erection due to paralysis of LI nerve. • Gluteal claudication is confused for sciatica and many patients are refe1Ted to orthopaedic department. Sciatica causes neurogenic claudication which is present even at rest and is aggravated on movements of the spine. Causes of neurogenic claudication are slipped disc, fracture vertebrae, tuberculosis of spine, etc. • Calf muscle claudication (Key Box 7 .2)

.,---41 SUPERFICIAL FEMORAL ARTERY STENOSIS OR OCCLUSION

�

• It is the most common cause of intermittent claudication • Usually calf muscles are affected • Does not produce life-threatening ischaemia unless profunda femoris is involved. • Single stenosis less than 3 cm is treated by Percutaneous Transluminal Angiography (PTA)

5. Coldness, numbness, paraesthesia and colour changes indicate chronic ischaemia. 6. Majority of patients with peripheral vascular disease are smokers. TAO occurs exclusively in male smokers 1• PEARLS OF

WISDOM

Cauda equina claudication or pseudoclaudication is due to compression of cauda equina.

Classification • Rutherford also categorised chronic lower limb ischaemia into 6 different categories starting from asymptomatic case to a major tissue loss. • Claudication has been classified as mild, moderate and severe depending upon treadmi 11 response and ankle pressure (AP) Mild:

Fig. 7.5: Thromboangiitis obliterans with dry gangrene

Complete treadmill test AP is > 50 mmHg after exercise Moderate: Cannot complete treadmill test. AP after exercise is< 50 mmHg Resting AP S 40 mm of Hg (cannot do tread­ Severe: mi11 test)

1 A 26-year-old female, nonsmoker patient presented to the hospital with ischaemic features of the right upper limb. All causes of upper limb ischaemia were ruled out (Raynaud's, cervical rib, etc.). On careful questioning, she admitted to using SNUFF DIPPING for IO years (snuff contains nicotine).

Lower Limb lschaemia and Popliteal Aneurysm

79

SIGNS (CLINICAL EXAMINATION) Inspection The findings are appreciated better if a comparison is made with the opposite limb. Evidence of chronic ischaemia of the leg are: • Attitude of the limb: Very often, the patient holds the calf muscles or dorsum of foot. • Flattening of the terminal pulp spaces of toes • Fissures, cracks in between the toes • Ulceration of toes, interdigital ulcers • Brittle, flat and ridged nails, shiny skin • Loss of hair and subcutaneous fat • The limb may appear more dark in dark-skinned patients or markedly pale in fair-skinned patients with vasospastic disease such as TAO (Fig. 7.6).

Fig. 7.7: Vasculitis with collagen vascular disorder. It had affected all the ten toes

Palpation 1. Ulcer: Examination should be done as described 111 Chapter 6. Ischaemic ulcers are very tender. 2. Gangrene: It is described according to its size, shape and extent. In dry gangrene, the part is dry and mummified or shrunken. Features of dry gangrene are summarised in Key Box 7.3.

.......

IM=':�v GANGRENE

Fig. 7.6: Attitude of TAO patient-foot is tightly held by hand. Observe gangrene, ulcer, skin changes and ridged nails

• Gangrene is usually dry with a clear line of demarcation. It indicates the junction of dead and living tissue. Since the blood supply to the muscle is better, usually the line of demarcation involves skin and subcutaneous tissue. Line of demarcation is very well appreciated in senile gangrene where it can be skin, muscle or bone-deep. • The limb may show atrophy of muscles. • Multiple toes and finger involvement suggest vasculitis (Fig. 7.7). PEARLS OF

WISDOM

Why is ischaemic pain more in the night? Loss of gravity assistance to arterial supply, reduction in cardiac output at rest, reactive dilatation ofskin vessels to warmth and increased attention of the patient to the leg. 1 ft is better to palpate the entire limb from the thigh downwards. 2Ischaemic limb is like irritable personalities.

• Loss of temperature

• Loss of pulsation

• Loss of sensation

• Loss of colour

• Loss of function

3. Limb above 1 • Ischaemic limb is cold: Careful palpation from above downwards will reveal the change in temperature from warm to the cold area. Temperature changes are appreciated better with the dorsum of the hand which is more sensitive as it has a lot of cutaneous nerve endings. • Tenderness: It is tender due to the presence of inflammation. • Sensation: Ischaemic limb is hypersensitive2 , due to the irritation of nerve endings. • Pitting oedema can be due to thrombophlebitis or due to nonfunctioning of limb. • Feel pulses in all four limbs and head and neck.

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Manipal Manual of Surgery

4. Palpation of pulses' (Table 7.4). • After examining the pulses, results are interpreted in a pulse chart as shown in Key Box 7.4. • In a similar manner, upper limb pulses, head and neck pulses are also recorded in the pulse chart.

tMt,.___,.V PULSE CHART

(For example, classical case of TAO left lower limb) LOWER LIMB PULSES

Right

Left

Dorsalis pedis

++

Posterior tibial

++

Popliteal

++

+

Femoral

++

++

++ : Normal; + : Weak; - : Absent. +++ : Indicates prominent pulse such as water hammer pulse as in aortic insufficiency

Disappearing pulse: When collateral circulation is very good peripheral pulses may be normal. However, when the patien is asked to exercise, the pulse may disappear. Exercis( produces vasodilatation below the obstruction and arteria inflow cannot keep pace with increasing vascular space pressure falls and the pulse disappears. Other tests of minor importance 1. Buerger's postural test is relevant in fair-skinned patients. The patient (supine) is asked to raise his legs vertically upwards keeping the knees straight. In cases of chronic ischaemia, marked pallor develops within 2-3 minutes. The angle at which pallor develops is Buerger's angle of circulatory insufficiency. In ischaemic limb, pallor develops even on elevation of leg up to 15-30 degrees. 2. Capillary refill test: Apply pressure over the tip of the terminal pulp space for a few seconds and release the pressure. Rapid return of circulation is observed in normal persons(< 2 seconds).

Examination of peripheral vessels Artery

Site where it is felt

Remarks

Examination of lower limb pulses 1. Dorsalis pedis is the continuation of anterior tibial artery

At the level of ankle joint lateral to extensor hallucis longus. It should not be felt distally where it dips into the plantar space

In I 0% of cases, it can be absent

2. Posterior tibial artery is a branch of popl iteal artery

Ln between the medial malleolus and medial border of the tendoachilles

For circulation of the foot, any one of these arteries is sufficient

3. Popliteal artery, a continuation of femoral artery, extends from the hiatus in adductor magnus to the fibrous arch in soleus. It is about 20 cm long

It is felt in the prone or supine position with knee flexed. It is felt against lower end of femur or against tibial condyles

The knee is flexed to relax popliteal fascia. Dorsalis pedis, posterior tibial and popliteal artery are usually not palpable in TAO patients

4. Femoral artery is the continuation of external iliac artery

It is felt midway between anterior superior iliac spine and pubic tubercle,just below the inguinal ligament in the upper thigh

Abduction and external rotation of the hip joint may facilitate the palpation in obese patients

Examination of head and neck vessels 1. Subclavian artery arises from the arch of aorta on the left side and brachiocephalic on the right side

lt is felt in the supraclavicular region in the pos­ terior triangle against the first rib

Difficult to feel in obese patients

2. Common carotid artery arises from arch of aorta on the left side and from brachiocephalic artery on the right side

It is felt against the carotid tubercle of sixth cer­ vical vertebra (C6) in the carotid triangle (at the upper border of the thyroid cartilage)

Carotid artery bifurcates at the upper border of the lamina of thyroid cartilage (C3 vertebra)

3. Superficial temporal artery is the terminal branch of the external carotid artery

It is felt in front of tragus of the ear against the zygoma

This is involved in temporal arteritis, a type of giant cell arteritis

1 A thorough clinical examination of pulses includes not only lower limb vessels but also head, neck and upper limb vessels.

.•,__..�

Lower Limb lschaemia and Popliteal Aneurysm

• The test can also be done in the ischaemic foot by asking the patient to sit up and hang his legs down and observe for colour changes. The time taken for the ischaemic foot to become pink is described as capillary filling time. This is prolonged in an ischaemic foot.

Auscultation (Figs 7.8 and 7.9) I. Systolic bruit over the femoral artery can be heard in atherosclerotic occlusion of iliofemoral segment, due to turbulence created by the blood flow. 2. Auscultation of the heart to rule out mitral stenosis (mid­ diastolic murmur, loud 1st heart sound).

...... • •

• • • • • • • • • •

81

CHECKLIST OF FEATURES OF CHRONIC LOWER LIMB ARTERIAL OCCLUSION Cold and numbness Limb elevation: Slow venous refilling. Line of demarcation present Altered/diminished sensation Ulcerations Dead toe-gangrene-Dried and mummified Intractable pain-rest pain Cracks, fissure-interdigital Arterial pulsations decreased or absent Thrill/bruit may be present Intermittent claudication Oedema-If thrombophlebitis or cellulitis occurs Narrow calf muscle girth-muscle atrophy

Remember as CLAUDICATION

......

1m:1r,ot----,'�, Type Type Type Type Type

Fig. 7.8: Auscultation of the heart to rule out valvular heart diseases

Fig. 7.9: Auscultation over the femoral artery to look for bruit

An eighteen-year-old girl was kept in the MBBS examination with ischaemia of lower limb of 6 days duration. The candidate offered collagen vascular disorder as the first diagnosis and failed. He had missed the cardiac history and findings totally by not auscultating the heart. It was a case of mitral stenosis with acute embolic gangrene of the lower limb.

Differential diagnosis • Even though there are many causes of lower limb ischaemia, thromboangitis obliterans (TAO) and atherosclerotic vascular disease are the commonest causes. Hence, they have to be considered first before giving other diagnosis. • TAO is also called Buerger's disease. The details are given in Table 7.5 and Key Boxes 7.5 to 7.7). • Atherosclerotic vascular disease is the commonest cause of lower limb ischaemia. It can manifest as simple ulcer to massive gangrene.

CLASSIFICATION OF BUERGER'$ DISEASE I : Upper extremity Crural (leg and foot) II : Femoral type-femoropopliteal Ill : Aortoiliac IV Generalised type V :

"''*-'� BUERGER'$ DISEASE-SUMMARY

• Male smoker • Progressive, nonatherosclerotic, segmental, occlusive, inflammatory condition • Occlusion of small- and medium-sized vessels, superficial thrombophlebitis and Raynaud's pheno­ menon constitute the 'triad' of TAO. • Microabscesses, polymorphs, giant cells (pathology) are found. • Distal, infrapopliteal, segmental occlusion with skip lesions and corkscrew collaterals in angiogram. • Stop smoking • Start analgesics • Lumbar sympathectomy is of some value Key words can be remembered as PRISON Progressive, Inflammatory, Segmental, Occlusive, Nonathero­ sclerotic

Investigation 1. Complete blood picture: Anaemia definitely delays wound

healing and it also decreases tissue perfusion. High total count indicates secondary infection. • Elevated platelet count suggests risk of thrombosis.

82

Manipal Manual of Surgery Differential diagnosis 1 Atherosclerosis

TAO (Buerger's disease) I. Age 2. Sex 3. Aetiology

Around 50 years and above 20--40 years Females are also affected Exclusively males 1. It is a smoker's disease. Excessive tobacco (nicotine) 1. Atherosclerosis is a rich man's disease, who is usually a smoker, diabetic and hypertensive. produces severe vasospasm of the vessels. 2. Excessive smoking produces increased levels of 2. Strong family history is also present in a few cases. carboxyhaemoglobin which damages these vessels. 3. Low socioeconomic group, recurrent trauma to the 3. Consumption of high fat diet leading to obesity, lack of regular exercises and hypercholestero­ foot, poor hygiene are additional factors. laemia are other factors. 4. Hypercoagulable state 5. Autonomic hyperactivity 6. Autoimmune factors

4. Pathology

Diffuse inflammatory reaction involving all three coats of vessel (panarteritis) causing a thrombus, resulting in occlusion of lumen (obliterans). Polymorphs, giant cells and micro-abscesses are found within the thrombus. In severe cases, vein and nerve are bound by fibrous tissue.

5. Vessels involved 6. Upper limb involvement

Small- and medium-sized vessels such as dorsalis Medium-sized and large vessels such as aorta, pedis, posterior tibial, popliteal are commonly involved. common iliac, femoral, common carotid, arteries are involved. Not uncommon Rare

7. Nature of vessel wall

Not thickened

Thickened

8. Blood pressure

Normal in the normal limb and low in diseased limb.

Hypertension is commonly present.

9. Superficial2 migrating thrombophlebitis

Seen in about 30% of cases of TAO. Veins of lower Not seen limb are involved and are tender and thickened.

Deposition of lipid-rich atheromatous plaque in the intima is the hallmark of atherosclerosis. Plaques tend to be more in lower abdominal aorta, coronary arteries, etc. Plaques may undergo calcification, ulceration and thrombosis, dislodge cholesterol emboli or may weaken the media and produce aneurysm.

10. Raynaud's phenomenon

Can be present

Not seen

l l . Auscultationfemoral artery

Bruit is not heard.

Bruit can be present as in aortoiliac disease.

12. Angiography

Cork-screw pattern of vessels

Shows site of block

• Fasting blood glucose or and glycosylated haemoglobin HbA1c is important test as it reflects the duration of diabetes. • Increased creatinine indicate renal disease. 2. Lipids: Fasting total cholesterol, high density lipoprotein, low-density lipoprotein, and triglyceride concentration­ hyperlipidaemia should be controlled to prevent progression of peripheral arterial disease and death from coronary artery diseases. 3. Hypercoagulable status: • Protein C deficiency is identified as a risk factor for arterial thrombosis especially in patients who will be treated with heparin.

• Heparin induced platelet aggregation and heparin induced thrombocytopaenia • Antiphospholipid antibody (APLA) syndrome (APLS) is also called Hughes syndrome. It is an autoimmune hypercoagulable state resulting in thrombosis of veins (deep vein thrombosis), thrombosis of artery (stroke) and pregnancy related complications. Treated by aspirin and heparin. 4. Homocysteine levels more than 15 µmol/1 5. Doppler ultrasound blood flow detector: This test is based on Doppler principle. An ultrasound signal is beamed at an artery and the reflected beam is picked up by a receiver. Frequency changes of the beam due to moving

1 From MBBS examination point of view, TAO and atherosclerotic vascular disease are to be differentiated. 2Migrating thrombophlebitis is also seen in pancreatic malignancy where it is called Trousseau's sign.

Lower Limb lschaemia and Popliteal Aneurysm

Fig. 7.10: Doppler probe over the femoral artery

Fig. 7.11: Handheld Doppler checking the dorsalis pedis artery

blood are converted into audio signals which can be heard by using a probe. Thus, Doppler probe can be used to detect the pulse even when the pulse is clinically not palpable (Figs 7.10 to 7.13). • By using sphygmomanometer, systolic blood pressure (SBP) of the limb can be measured by positioning the cuff at a suitable level and pressure index can be calculated. This is called ankle brachia! index-AB!. Ankle Brachia) Ankle blood pressure Pressure Index = --------­ Brachia! blood pressure • Normal values are above I. However, in patients with peripheral vascular disease of the lower limb, the values are below 1 which indicate vascular obstruction. • When ankle pressure is less than 30 mmHg, gangrene may be imminent. Uses of Doppler probe • To detect normal pulses as in operation theatres. • To detect clinically nondetectable pulse as in peripheral vascular disease.

83

Fig. 7.12: Colour Doppler showing femoral artery and vein

Fig. 7.13: Colour Doppler showing atherosclerotic narrowing

CFA-78 PR0-60

CFA-88 PR0-68

I SFA: 100 I

SFA: 120

Popliteal-85

Popliteal-80

PTA-P-62 ATA-P-50 CFA-Common femoral artery; PRO-profunda; SFA-Superficial femoral artery; ATA-anterior tibial artery; PTA-posterior tibial artery

Fig. 7.14: Duplex scan report of a patient with the PAD-The number represents the velocity of blood flow in that artery

Manipal Manual of Surgery

84

• To measure BP (blood pressure) in ischaemic limb. • Remeasuring BP in lower limb after exercise to differentiate ischaemic claudication from neurogenic claudication. 6. Duplex scan: • This is the investigation of choice today. Duplex scan is a combination of Doppler with B mode ultrasound (Fig. 7.14). With the availability of colour Duplex, the direction of blood flow can be assessed. Red colour means direction of flow towards transducer and blue means away (Key Box 7.8).

MJMWWIIIIIIIIIIF�, DUPLEX IMAGING SCAN

• It yields both anatomic and blood flow information • No nephrotoxic contrast agent is used • It gives a triphasic wave pattern systolic, diastolic and elastic recoil • Elastic recoil is absent in calcified arteries • Thus biphasic and monophasic wave patterns indicate 'PAD' • Overall sensitivity of 92% and specificity of 99% in occlusive cases • Limiting factors are extensive ulcers, calcification and oedema of leg 7. Angiography (arteriography) (Fig. 7.15) • Before angiogram, follow certain principles (Key Box 7.9) or precautions. • It is not usually indicated in TAO patients where direct arterial surgery is not done. However, a few cases of presenile atherosclerosis who are misdiagnosed as TAO will require angiography to locate the site of obstruction which is suitable for an arterial reconstruction. Angiography is indicated in patients with atherosclerotic vascular disease, to know the exact site of block, type of obstruction, to define the collaterals, so as to plan for arterial reconstruction. PEARLS OF

Fig. 7.15: Angiography showing block in the common iliac arter on the right side (conventional)

Fig. 7.16: Left common iliac artery occlusion

WISDOM

Small vessel bypass is becoming popular today as in selected cases of diabetes and atherosclerosis.

...... '"'�V • • • • • •

PREANGIOGRAPHY-CHECKLIST

Lactic acidosis can occur if creatinine is elevated Anticoagulants: Should be stopped Creatinine: Should be normal Tablet Metformin: It can cause acidosis with contrast dye Intravenous fluids: To prevent renal failure Contrast allergy: Can cause anaphylaxis

Remember as LACTIC

Fig. 7.17: Distal formation at common femoral artery (Courtesy: Dr Chandrakanth Shetty, Prof of Radiology and

Imaging, KMC, Manipal)

Lower Limb lschaemia and Popliteal Aneurysm

Types of angiography A. Percutaneous transfemoral retrograde angiography • This is done in unilateral obstruction. An incision is made in the upper thigh to expose the femoral artery on the normal I side. A Seldinger needle and guide wire are used to introduce arterial catheter and radiopaque dye is introduced after placing the catheter into the aorta. It visualises the entire aortoiliac segment and below. B. Direct translumbar angiography or aortography • It is indicated when obstruction is bilateral, both femoral pulses are not palpable, clinically manifesting as bilateral lower limb ischaemia. Aorta is directly punctured from behind (translumbar) by using ultrasound image intensifier. Results: Arteriography establishes the site of block and nature of collaterals. Complications of angiography2 (Key Box 7.10) • Thrombosis at the puncture site resulting in ischaemia. • Haemorrhage from the puncture site which needs to be stopped by pressure packing. • Arterial dissection if catheter is wrongly placed and advanced. • Anaphylaxis can be avoided by a trial injection. • Paraplegia due to spasm of spinal arteries • Infection: Digital subtraction angiography (DSA)­ This can be done by arterial or venous route. The arterial route is preferred. DSA gives excellent pictures in carotid and large central vessels. However, the peripheral vessels may not reveal adequate information. Thus, conventional arteriography still has an important role in atherosclerotic vascular disease. 8. Magnetic Resonance Angiography (MRA) (Figs 7. l 6 and 7.17) • It is more popular than arteriography because no arterial puncture and no contrast induced nephropathy.

85

• Gadolinium enhanced MRA can visualise entire arterial tree. pattern including small pedal vessels. • Patients with newly placed metallic implants are not the candidates. 9. CT Angiography: It is good for above knee vessel� compared to below knee (Figs 7.18 and 7.19). 10. Carbon dioxide angiography with gas infusion also has been found useful in all sized vessels and is well tolerated.

Fig. 7.18: Right common iliac artery occlusion

Ntd_.,�, Thrombus

COMPLICATIONS OF ANGIOGRAPHY

Rarely paraplegia Arterial dissection Unexpected infection/sepsis Massive bleeding Anaphylaxis Remember as TRAUMA

Fig. 7.19: Right common iliac artery occlusion-another view

1Normal limb is selected for two reasons: • Femoral pulse is palpable. Hence, easy to locate the artery and introduce the catheter. • If a thrombus occurs in the diseased limb due to angiography, it will worsen the ischaemia, may lead to gangrene. 2Because of these complications, angiography should be done carefully only when it is indicated.

-,---�

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Manipal Manual of Surgery

PRINCIPLES OF TREATMENT

k----- Common femoral artery Hf----- Superficial femoral artery

Fig. 7.20: Extensive narrowing of popliteal artery and branches due to atherosclerosis

TREATMENT OF PERIPHERAL VASCULAR DISEASE (TAO AND ATHEROSCLEROSIS) • In all patients with peripheral vascular disease, following general measures must be taken which will help in better perfusion of the lower limb tissues. Anaemia must be treated with haematinics and if necessary, blood transfusion. If ejection fraction is low, drugs are given to improve cardiac output. Principles (Key Box 7 .11) I. To relieve the pain As already discussed, the pain is very severe and distressing. Some amount of pain relief can be obtained as follows: 1. Analgesics: Simple analgesics may not help these patients. • Tramadol (50 mg) one tablet, 3 times a day can be used. • Ketorolac 10-20 mg one tablet, 3 times a day can be given in severe cases.

• • • • •

To relieve the pain To arrest the progression of the disease Role of vasodilators Chemical sympathectomy Surgical methods

• Narcotic analgesics can be used judiciously in cases with rest pain. 2. Buerger's position by elevating head-end of the bed, causes venous congestion and reflex vasodilatation. 3. Buerger's exercises by elevation and dependency of the limb for a few minutes. 4. Heel raise by raising the heels of shoes by 1-2 cm, claudication distance can be increased as it decreases work load on the calf muscles. II. To arrest the progression of the disease Stop smoking: This is more beneficial in TAO patients than atherosclerotic patients. Regular exercise reduces obesity, controls hypertension (Table 7.6). Diet: Avoid fatty food to reduce serum cholesterol. It is more useful in patients with hyperlipidaemia. • Avoid injuries. Ill. Medical management (Table 7.6) Vasodilators have not been found to be useful in atherosclerotic vascular disease. Some degree of reduction of pain and healing of cutaneous ulcers has been found in TAO patients. The drugs are pentoxifylline and prostacyclin. Pentoxifylline • It can be used to treat intermittent claudication • Pentoxifylline improves blood flow through peripheral blood vessels. It can also be used to increase spenn motility

Medical management of peripheral vascular disease-drugs, dosage and their role • Antiplatelet

Aspirin 75 mg with or without clopidogrel 75 mg

Decreases vascular death by 25%

• Antismoking

Stop smoking

Smoking cessation can decrease I 0-year mortality rate from 54% down to 18%

• Anticholesterol

Statins-to decrease LDL cholesterol to at least 100 mg/di

Slow release niacin is emerging as an important therapy in patients with dyslipidaemia

• Antihypertensives

Decrease blood pressure to < 130/85 mmHg

ACE inhibitors, P-blockers

• Antidiabetes

Glycosylated haemoglobin level of< 7%

Oral hypoglycaemic agents or insulin

• Antivasospasm (vasodilator)

Cilostazol-50 mg twice a day (doubtful value)

Inhibits platelet aggregation and is a direct arterial vasodilator

.,__..�

Lower Limb lschaemia and Popliteal Aneurysm

when viable sperms are immotile and are being used in intracytoplasmic sperm injection (ICSI). • Pentoxifylline improves red blood cell deformability (alters the shape of RBCs) reduces blood viscosity and decreases the potential for platelet aggregation and thrombus formation and reduces inflammation and innate immunity. • Dose : 400 mg 3 times a day • It may take 4-8 weeks for the benefit to the patient Prostacyclins • Prostacyclin (also called prostaglandin 12 or PGI 2) is a prostaglandin member of the family of lipid molecules known as eicosanoids. • It inhibits platelet activation and is also an effective vasodilator. • Prostacyclin (PGii) chiefly prevents formation of the platelet plug involved in primary hemostasis (a part of blood clot formation). • It does this by inhibiting platelet activation. • It is also an effective vasodilator. Hence used in peripheral arterial diseases. • Dose: 2 ng/kg/min IV, to start with and increased by 2 ng/ kg/min every I 5 minutes as tolerated to a maximum of 16 ng/kg/min intravenous. IV. Chemical sympathectomy • It acts by producing vasodilatation of the blood vessels of the lower limb. In this procedure, 5 ml of phenol in water is injected beside the bodies of 2nd, 3rd and 4th lumbar vertebrae. The effect of the drug can be judged immediately by feeling for warmth in the feet. • It helps in healing of ulcers and may improve rest pain probably by interfering with afferent sensory circuits. Precautions 1. Lateral injection by using a lumbar puncture needle. 2. Injection should be in front of lumbar fascia which contains the sympathetic trunk. 3. Avoid injuries to aorta and inferior vena cava. 4. Procedure is to be done under X-ray control (screening) Since phenol has replaced lignocaine because of its long lasting effect, it is also called phenol sympathectomy. V. Surgical procedures in TAO 1. Lumbar sympathectomy is the indirect surgery done for TAO patients since direct arterial surgery is not possible (Key Box 7.12). • Indications: Cutaneous ulcer and rest pain. • Structures which can be confused for lumbar sympathetic trunk are: 1. Genitofemoral nerve 2. Tendon strip of psoas muscle 3. Lymphatic chain and fatty tissue

87

SALIENT FEATURES OF LUMBAR SYMPATHECTOMY • Transverse loin incision

• Extraperitoneal approach, and it is a preganglionic sympathectomy. • Lumbar sympathetic trunk is identified in the paravertebral gutter lateral to the psoas muscle as a cord-like structure. • 2nd lumbar ganglion is lar ge and has white rami joining it. • Sympathetic trunk is divided below the first lumbar vertebra and removed up to the 4th lumbar vertebra. • This is a preganglionic sympathectomy because fibres supplying the vessels of the limb have their cell stations in the sacral ganglia which are not disturbed.

•

•

2.

3. 4.

By depriving the sympathetic nerve supply to lower limb blood vessels, vasomotor tone is reduced so that some amount of vasospasm is reduced. Thus, rest pain improves, minor ulcerations heal due to cutaneous vasodilatation. However, the duration of effect of lumbar sympathectomy is not clear. Both sides can be done in one sitting. However, during bilateral operation, the 1st lumbar ganglion on one side should be spared since removal of both ganglia may cause sterility due to paralysis of ejaculatory mechanism. One should be careful not to damage lumbar veins 1 which join inferior vena cava. Omentoplasty has been tried in TAO patients. In most of these patients vasodilator therapy and lumbar sympathec­ tomy has been done with almost no relief of symptoms. In such cases, before a merciful amputation is done, omento­ plasty is attempted. • By careful division of vascular arcade of omentum, it can be lengthened based on one of the epiploic arteries, brought out of the laparotomy incision, tunnelled in the subcutaneous plane and can be brought up to calf or even to the ankle joint level in some patients. • Greater omentum is supposed to produce neovascu­ larisation and thus helps in healing of the cutaneous ulcers. The effect seems to be temporary (not done nowadays). Conservative amputations should be done if the toes are gangrenous. Below knee amputation2 is the last resort. It is indicated in severe rest pain cases where all other modalities of treatment have failed. Risk of amputation after ten years of the disease is around 10%.

1 Lumbar veins, if cut accidentally, retract and troublesome bleeding can occur from inferior vena cava. Pressure packing, waiting for 3-5 minutes and then 'see and ligate' should be the policy. 2Students are advised not to tell this first as a treatment modality.

Manipal Manual of Surgery

IIMtm_..�, MAJOR RISK FACTORS FOR ATHEROSCLEROSIS

Lipids: Dyslipidaemia

•

teflon or dacron, is used. It is also called Y-graft or trouse1 graft. It has commonly 16 mm trunk and two 8 mm diameter limbs (Figs 7.21 and 7.22). In unilateral cases, unilateral graft is applied.

B. Aortoiliac endarterectomy

Inhalation of tobacco: Smoking Insulin deficiency: Diabetes

Indications • Short segment, large artery such as aorta and single artery.

Disordered metabolism: Hyperhomocystinaemia

Types

Pressure: Hypertension

11,---� Remember as LIPID

PROGNOSTIC FACTORS FOR LIMB REVASCULARISATION

• • • •

Severity of the disease Presence of collaterals Presence of diabetes Chronic smoking

• • • •

Site of occlusion Age of the patient Angina pectoris Fitness for anaesthesia

VI. Surgery in atherosclerotic vascular disease • If possible avoid or treat risk factors (Key Box 7.13). • The decision to revascularise the limb is taken after an angiography. The success of reconstruction depends upon a number of factors. They are given in Key Box 7.14. • Intermittent claudication alone is not an indication for surgery. Rest pain and pregangrenous changes in the limb are definite indications for reconstruction with accepted mortality and morbidity. • Surgery can be classified into surgery for aortoiliac disease and surgery for iliofemoral stenotic disease. Treatment of atherosclerotic disease I Aortoiliac disease It is treated by bypass grafts or endarterectomy. A. Bypass grafts Usually, it is bilateral and is treated by using aortobifemoral graft to bypass the stenosis. The graft, made from either

Figs 7.21 and 7.22: Aortobifemoral graft ( Courtesy: Dr Ganesh Karnath, Prof. and Head of Cardiothoracic Surgery, KMC, Manipal)

A. Open endarterectomy: An arteriotomy is done first and

the diseased intima, atheromatous plaque and the thrombus are removed. An arteriotomy incision can be closed directly or a vein patch graft is used to close the defect, so as to avoid narrowing. B. Closed endarterectomy is indicated in a longer-diseased segment. In this procedure, after an arteriotomy, a wire loop is used to strip out a core of atheroma by introducing it through the lower arteriotomy and removing the atheromatous plaque from the upper end. However, results of bypass graft are better than endarterectomy. The modern tendency is to do bypass graft. II lliofemoral stenotic disease •

•

This can be repaired by a bypass graft which is sutured to the normal common iliac artery above and to the normal femoral artery below (Figs 7.23 and 7.24). If patients are unfit for a major vascular bypass, angioplasty can be done. Ideal for short segment stenosis. In this procedure, a balloon catheter is inserted into the artery, inflated and its correct position is confirmed by radiopaque markers which are present in the balloon. By inflation and dilatation technique 2-3 times, the stenosed segment can be dilated (Figs 7.25 to 7.31). Summary of revascularisation surgery is given in Key Box 7.15).

Figs 7.23 and 7.24: lliofemoral graft by using reversed saphenous vein

-,---�,

Lower Limb lschaemia and Popliteal Aneurysm

SUMMARY OF THE REVASCULARISATION SURGERY (Figs 7.25 to 7.31)

Aortoiliac disease

lliofemoral disease Femoropopliteal disease Profunda artery stenosis

Aortobifemoral or aortofemoral graft and endarterectomy lliofemoral bypass graft Balloon angioplasty Bypass graft Profundoplasty

Any arterial stenosis can be dilated in angioplasty. The technique can be repeated if stenosis recurs. The procedure is done under local anaesthesia and 1s indicated in poor-risk patients. •

Ideally suitable for iliofemoral segment, not suitable for stenosed vessels below knee. - Internal dissection, distal embolisation, thrombosis and even rupture of vessel can occur. - Recently, angioplasty combined with laser to drill holes through short stenosis have been employed.

Ill Femoropopliteal occlusion

•

This is treated by using a graft extending from the femoral artery above, to the popliteal artery below. Reversed long saphenous vein is better than other grafts because it is less thrombogenic. Dacron graft, polytetrafluoroethylene graft (PTFE), human umbilical vein graft are the other grafts.

Profunda artery stenosis Significant occlusion of profunda is demonstrated by oblique views in an a1teriography. lf there are no significant vessels available below the stenosis for reconstruction, profundoplasty is considered. It is done by using a patch of Dacron or vein to widen the origin of this vessel after doing endarterectomy. ACUTE ARTERIAL OCCLUSION •

Sudden occlusion of an artery is commonly due to emboli. The source of emboli is from the hea1t or from atheroma. Increased incidence of road traffic accidents, fall or war injuries are other causes. Trauma to the artery also produces occlusion.

Embolic occlusion This occurs commonly in the peripheral arteries such as the common iliac, femoral and popliteal. An embolus is a foreign body to the bloodstream, gets lodged in a vessel and produces obstruction. lt manifests

89

A 75-year-old lady, known case of polycythaemia vera, presented to the hospital with multiple gangrenous patches ofskin in the upper limbs and in the lower limbs. Conservative surgery was attempted by debriding gangrenous portion of the skin. Polycythaemia is also one ofthe causes ofgangrene.

clinically as severe ischaemia or gangrene, resulting in critical limb ischaemia (CLI). See clinical notes above What is critical limb ischaemia (CU)? It is defined as persistently recurring ischaemic rest pain requiring regular, adequate analgesia for more than 2 weeks or ulceration or gangrene of foot or toes with an ankle pressure of< 50 mmHg or a toe systolic pressure of< 30 mmHg. • Atherosclerotic v ascular disease, thromboangitis obliterans, acute embolic ischaemia and even diabetes, etc. at some stage can present as CLI. Pathology (Fig. 7.32) Causes (see Table 7.7) Clinical features (Key Box 7.16) • No previous history suggestive of intermittent claudication • Sudden dramatic symptoms which are described in the form of5 Ps - Pain - Pallor Paresis Pulselessness Paraesthesia 1. Pain is severe, unbearable, burning or bursting type. 2. Limb is pale, cold and superficial veins are collapsed. 3. Paresis: Depending on the level of occlusion, the function of the limb is lost. Movement of the toes becomes difficult, followed by total paralysis. 4. Pulselessness: Characteristically, peripheral pulses below the level of embolism are not palpable.

.,��, • • • • • • •

SIGNS OF ACUTE LOWER LIMB ISCHAEMIA Peripheries are cold Pallor of the limb Poor capillary return Positive Buerger's test Progressive paralysis Pulses are absent Pulse at ankle by Doppler-undetectable Observe 7 Ps

90

Manipal Manual of Surgery

BALLOON ANGIOPLASTY IN ATHEROSCLEROTIC ARTERIAL DISEASE

,

Fig. 7.25: Plain radiograph with guide wire in the aorta

Fig. 7.26: Narrowing of the right common iliac artery

Fig. 7.27: Balloon angioplasty of narrowed right common iliac artery

Fig. 7.28: Post balloon angioplasty

' Fig. 7.29: Mid-SFA showing narrowing

Fig. 7.30: Balloon angioplasty

Fig. 7.31: Postangioplasty image showing restoration of lumen of mid-SFA

( Couttesy: Prof Chandrakanth Shetty and Dr Praharsha, Resident, Department of Radiodiagnosis and Imaging, KMC, Manipal)

Lower Limb lschaemia and Popliteal Aneurysm

91

Neutrophils release products

Leukotrienes and thromboxanes

Proteolytic agents

Oxidising agents

Mannitol, vitamin E, etc.

Cyclo-oxygenase inhibitors

Damage

Endothelial damage

NSAID, steroid calcium channel blockers Tissue damage

Fig. 7.32: Pathogenesis of tissue damage

Fig. 7.33: Critical limb ischaemia (CLI)

Fig. 7.34: CLI due to diabetes and atherosclerosis

Causes of acute lower limb ischaemia Acute lower limb ischaemia

Embolism

• • • • • •

Thrombosis (causes)

Atrial fibrillation Mitral stenosis Myocardial infarction Aneurysm Atheromatous plaques Emboli from myxoma of heart

• • • • •

Common arteries affected

Common sites

• Aortic bifurcation (saddle embolus) • Common femoral bifurcation • Popliteal trifurcation

Polycythaemia rubra vera Thrombocythaemia Leukaemia High oestrogen pill Atheromatous

Fig. 7.35

• Emboli impact at branching points where arterial lumen narrows abruptly.

• External iliac • Profunda • Popliteal

92

Manipal Manual of Surgery

5. Altered sensation in the limb. • If left untreated, necrosis of the muscles followed by gangrene of the limb can occur within a few hours (6-24 hours). • Cardiac examination may reveal the source of emboli. PEARLS OF

Balloon beyond clot

Embolus

WISDOM

Embolectomy done

Intravenous drug abuse remains a major risk factor for endocarditis and embolic complications.

Investigations • Peripheral circulation should be assessed by Doppler ultrasound, which is an excellent noninvasive investigation to judge the severity, level, position and length of superficial femoral artery stenosis. • Digital subtraction angiography in aortoiliac lesions. Treatment I. Angioplasty: Percutaneous transluminal angioplasty (PTA) is indicated in short stenotic lesions in a large vessel, e.g. iliac and femoropopliteal lesions. • First, the balloon catheter is introduced percutaneously over a guidewire across the lesion. Under fluoroscopic control, the balloon is dilated until satisfactory widening of the lumen is achieved. • Relatively safe and simple procedure. • Immediate intravenous infusion of heparin (I 0,000 IU) is necessary to reduce the extension of the thrombo­ embolism. II. Emergency embolectomy is done under GA or local anaesthesia either by direct arteriotomy incision and removal of clot or by using a Fogarty balloon catheter to remove an embolus from a vessel remote from arteriotomy (Figs 7.36 and 7.37). • Embolectomy: Under local or general anaesthesia, a transverse incision is given over common femoral artery. A Fogarty catheter is introduced through the incision for about 1-2 cm and the balloon is inflated.

Arteriotomy is sutured Fig. 7.36: Fogarty embolectomy

Management of acutely ischaemic limb

Anticoagulants-10,000 units Heparin bolus dose IV Try to find out the cause (History, examination, unilateral or bilateral)

Surgical embolectomy

Thrombolysis

Fig. 7.37: Management of acutely ischaemic limb

Emboli in various arteries-symptoms, signs and effects I. Leg

Common femoral, popliteal

Pain/pallor

Gangrene/ischaemia

2. Brain

Middle cerebral artery

Minor or major TIA

Stroke/hemiplegia

3. Retina

Central retinal artery

Amaurosis fugax

Fleeting/permanent blindness

4. Intestine

Superior mesenteric artery

Postprandial pain, shock

Intestinal ischaemia/gangrene

5. Kidney

Renal artery

Pain in loin, haematuria

Renal ischaemia

6. Upper limb

Brachia! artery

Pain, ulcer upper limb

Gangrene

Lower Limb lschaemia and Popliteal Aneurysm

• The catheter is then withdrawn and emboli removed. The procedure is repeated until bleeding occurs. The catheter can be passed by the ankle and distal emboli also can be removed. Postoperatively, anticoagulants are continued. • Intra-arterial thrombolysis: A catheter is passed into the 'clot' after doing arteriography and t-PA (tissue plasminogen activator) is infused through catheter. Repeat angio check films are taken to see the results such as lysis ofclot, revascularisation, etc. III. Thrombolysis • It is indicated in acute or acute on chronic ischaemia. • A fine lysis catheter is passed percutaneously into blocked vessel. • Streptokinase is infused at a rate of 5000 IU at 10 ml/hr. • It will reopen the occluded lumen within 24 hours. • Repeat angiogram is necessary to check for patency. For contraindications see Key Box 7.17. PERIPHERALANEURYSMS • These can affect the popliteal artery, femoral artery, iliac artery, etc. 70% of peripheral aneurysms affect popliteal artery, and two-thirds of them are bilateral. • However, students should realise that aneurysms are uncommon (rare) causes of lower limb ischaemia. Early diagnosis and effective treatment are essential to save the limb. Aortic aneurysms are discussed in Chapter 37 under abdominal mass. POPLITEALANEURYSMS They are the commonest peripheral aneurysms because of following reasons: • Turbulence beyond stenosis at the adductor magnus hiatus • Repeated flexion at the knee.

.,._..�., Absolute

• • • • •

CONTRAINDICATIONS TO THROMBOL YTIC THERAPY

Recent major bleeding Recent major surgery Recent trauma Recent ophthalmologic procedure Recent stroke

Relative • • • •

Active peptic ulcer disease Pregnancy Uncontrolled hypertension Coagulation abnormalities

93

Clinical features • They affect elderly patients and atherosclerosis is the cause. Age at presentation is 65 years. • One-third ofthe cases are associated with aortic aneurysm. • Striking preponderance in males. Male:female ratio is 20-30:1. • Presents as a swelling behind the knee. • Dull aching pain is common. Severe bursting pain indicates rapid expansion and impending rupture. • Pulsatile, tense, cystic, fluctuant swelling behind the knee, in the popliteal fossa and in the line of popliteal artery. • Its size diminishes on extending the knee as the aneurysm is deep to popliteal fascia. • Proximal compression test: On occluding the femoral artery proximally, the swelling may diminish in size. PEARLS OF

WISDOM

In all cases of popliteal aneurysm, please search for femoral and aortic aneurysm.

Investigations • Duplex ultrasonography is the investigation of choice which can measure diameter and determine extent ofmural thrombus. • Angiography can demonstrate the extent of involved segment to look for patency and quality ofrunoff vessels. Complications 1. Thrombosis causes severe acute ischaemia of the lower limb (incidence: 40%). 2. Embolisation causes ischaemic ulceration of the lower limb. 3. Rupture causes pain and haematoma (rare: 2-5%). 4. Compression on the popliteal vein causes pain, tenderness and swelling ofthe leg. 5. Compression on the lateral peroneal nerve causing foot drop, due to paralysis of the peronei and the extensors of the foot. Treatment • Proximal and distal ligation of the artery followed by reversed saphenous vein bypass graft is the treatment of choice. This method results in total obliteration of the sac with revascularisation ofthe limb. • Excision of the sac is better avoided because of chances ofinjury to the popliteal vein and nerves. Lateral popliteal nerve injury causes foot drop and tibial (medial popliteal) nerve injury causes thinning ofthe calfregion, inability to plantar flex the ankle and clawing of the toes due to paralysis of the intrinsic muscles of the foot.

94

Manipal Manual of Surgery

MISCELLANEOUS AINHUM (Fig. 7.38) It affects those who do not use footwear or walk barefooted. It starts as a fissure at the level of interphalangeal joint of a toe, usually fifth. Repeated trauma of minor degree may be present. The tissue becomes a fibrous band resulting in tight constriction and necrosis. If it continues, it may culminate in autoamputation. • The division of band or early Z-plasty may be needed to avoid amputation. • Thrombolysis with catheter in situ, in early cases. FROSTBITE • It occurs due to too much of exposure to cold weather. • High altitudes with excessive cold precipitates vasospasm and damage to the blood vessel wall. It causes sludging of blood and thrombosis. • Malnutrition, ageing process are the other precipitating factors. Severe burning pain, discolouration of foot, develop­ ment of blisters suggest gangrene is imminent (Fig. 7.39). Treatment • Slow warming of the parts and protection with cotton wool. • Analgesics and antibiotics. • Paravertebral injection into the sympathetic chain helps in a few patients. • Elevation of the foot to reduce oedema. • Frank cases of frostbite with gangrene require conservative amputation.

• Most of the injury is believed to be due to 02 derived fre{ radicals. The most important ones include superoxid{ radical, hydrogen peroxide and hydroxyl radical. • These radicals attach unsaturated bonds of fatty acids within the phospholipid membranes resulting in damage. Management • Diagnosis is clinical as suggested by severe pain in the limb, oedema of the leg and muscle tenderness. • Raised intracompartmental pressure measured by transducer cannula will help in the diagnosis. • Creatinine is elevated suggesting renal failure. • Creatine kinase will be elevated suggesting rhabdomyolysis. • Treated by urgent multiple fasciotomy, decompression followed by debridement of dead tissues. FAT EMBOLISM Definition Often a potentially lethal condition which occurs due to blockage of major arteries by aggregation of chylomicrons. Causes • • • •

Fracture femur (long bones) Orthopaedic surgery, multiple fractures Liposuction Sickle cell disease Pancreatitis Diabetes mellitus

REPERFUSION INJURIES OR SYNDROME • This dangerous event follows revascularisation of limbs, resulting in acute compartment syndrome with com­ partmental pressure exceeding capillary pressure (30 mmHg) (Fig. 7.39).

Massive ischaemia Revascularisation Release of intracellular ions, enzymes, proteins into circulation

Haemodynamic instability and lactic acidosis

Intracellular and interstitial oedema

Myocardial irregularity

Myoglobulinaemia (myoglobin, from injured muscle cells) Acute renal failure

Fig. 7.38: Ainhum

Hyperkalaemia

Acute compartment syndrome

Life-threatening dysrhythmias

Fig. 7.39: Pathophysiology of reperfusion injuries

95

Lower Limb lschaemia and Popliteal Aneurysm

PEARLS OF WISDOM

Respiratory failure, con/usion, petechial haemorrhages: Triad of fat embolism syndrome. Early diagnosis and prophylactic administration of oxygen to patients at risk is recommended. Clinical features • Pulmonary: Central cyanosis, tachypnoea, right heart failure, froth in mouth and nostrils. Respiratory distress is the most common presenting feature. CNS: Drowsiness, disorientation, restlessness, constricted pupils, pyrexia and coma. Retinal artery: Striate haemorrhages, fluffy exudates on fundoscopic examination, cotton wool spots. Cutaneous: Petechial rashes in the nondependent parts of the body-chest, axilla and conjunctiva. Pathogenesis • 50% of cases occur within 72 hours. • Free fatty acids (FFA) released at the time of trauma or breakdown of fat in the lung directly affect pneumocytes resulting in acute respiratory of distress syndrome (ARDS). • Other theory is blockage by aggregation of chylomicrons. Investigations Arterial blood gases will show hypoxaemia and respiratory alkalosis from hyperventilation. Chest X-ray will show bilateral interstitial and alveolar infiltrates in severe cases 'snowstorm' pattern. Treatment • Close monitoring with pulse oximetry. • Supportive therapy with oxygen and mechanical ventilation as required. • Surgical-early internal fixation of the fracture. AIR EMBOLISM Definition It is a potentially fatal condition that can occur due to blockage of pulmonary artery by large volume of air in the venous circulation (Key Box 7.18). Causes Neck surgery during which a large vein is inadvertently opened and the patient is in head-elevated position (thyroid surgery) or sitting position (posterior fossa surgery). Rapid infusion of intravenous fluids where emptying of the bottle and infusion set may go unnoticed and a fresh bottle is connected and infused without eliminating air from the infusion set. Effects Formation of air lock within pulmonary artery and right heart failure.

.......

IM!rffl--� AIR EMBOLISM

• • • • •

Large veins get opened in the neck Open heart surgery or if pulmonary vein is punctured Fallopian tube insufflation Illegal abortion {through paravertebral veins) Paradoxical embolism, reaching coronary artery through patent foramen ovale

Treatment Position the patient head down (Trendelenburg position) so that air entrainment is stopped. Tum the patients on their left side so that air will float to ventricular apex reducing its entry into the pulmonary artery. • Administer oxygen and resuscitate as necesssary. FONTAINE CLASSIFICATION • Peripheral artery occlusive disease is commonly divided into stages as introduced by Dr Rene Fontaine in 1954. • Fontaine classification has defined the severity of chronic ischaemia as: Stage I: Asymptomatic Stage 2: Intermittent claudication limiting lifestyle Stage 3: Rest pain due to ischaemia Stage 4: Ulceration or gangrene due to ischaemia • Some have also classified stage 2 into A and B: A - Intermittent claudication > 200 metres B - Intermittent claudication for < 200 metres INTENSIVE CARE UNIT (ICU) GANGRENE • It has been described previously in literature as a rare clinical entity. It was first described by Jonathan Hutchinson in 1891 as multiple extremity ischemia and was termed 'symmetrical peripheral gangrene'. • Possible aetiological factors include sepsis, DIC and use of vasopressor agents. The possible responsible drug is noradrenaline (Fig. 7.40). • Patients in ICU also have multiple arterial punctures for securing intra-arterial line for invasive monitoring in the ICU. The arterial punctures can be a possible aetiological factor. • Sepsis in the presence of sluggish blood flow has been described to be responsible for development of symmetrical peripheral gangrene (Fig. 7.41). • Vasospastic conditions, small vessel obstruction and very low cardiac output states (perfusion pressure falling to 35-60 mmHg) may contribute.

Please note: Aortic aneurysm is discussed in Chapter 44 under cardiothoracic surgery.

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• Medical conditions such as diabetes mellitus, malignancy, protein C or S or antithrombin III deficiency are also contributing factors. • The usual manifestation is pallor or cyanosis, coldness and pain in the extremity. They become erythematous and there is dusky discolouration of skin, bullae or blisters followed by gangrene. • Pulses may be intact in the early stages and large vessels are often spared. Low flow states result in occlusion of the microcirculation of the affected parts. • The first line of management of this gangrene is immediate discontinuation of vasopressors as soon as discolouration 1s seen.

Fig. 7.41: Bilateral symmetrical digital gangrene due tc atherosclerosis, diabetes, sepsis and hypotension

• Aggressive treatment of sepsis with intravenous antibiotic� and anticoagulation for D[C are the suggested measures. • Local debridement and secondary skin grafting have been unsuccessful. • Amputation should be considered only after a clear line of demarcation develops. The condition has a high mortality rate of 40%. WHAT IS NEW IN THIS CHAPTER?/RECENT ADVANCES

Fig. 7.40: ICU gangrene

• A few clinical photographs and Key Boxes have been added. More details about medical management of arterial disease is added. • Transluminal angioplasty has been discussed in more detail. • Fat embolism/air embolism, Fontaine classification are added. • New photographs, Key Boxes are added. • ICU gangrene is added.

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MULTIPLE CHOICE QUESTIONS 1. Which of the following cannot occur in thrombo­ angiitis obliterans? A. Dorsalis pedis and posterior tibial are often affected B. Radial artery can also be affected C. Superficial femoral is also affected D. Bruit present over femoral artery 2. Following are true for atherosclerotic arterial disease except: A. Large sized arteries are affected B. Upper limb is often affected C. It can be associated with aortic aneurysm D. A bruit can be present over the artery 3. Following are features of thromboangiitis obliterans except: A. Raynaud's phenomenon B. Migrating thrombophlebitis C. Segmental panarteritis D. Polymorphs and giant cells are absent rn histopatbological examination

8. Triad of fat embolism includes: A. Respiratory failure, confusion, petechial haemorrhages B. Respiratory failure, confusion, large purpuric spots C. Respiratory failure, alertness, petechial haemorrhages D. Cardiac failure, confusion, petechial haemorrhages 9· Following are true for popliteal aneurysm except: A. It is caused by atherosclerosis B. It is the most common type of peripheral aneurysm C. It is always unilateral D. Foot drop can occur 10. Following are true about lumbar sympathectomy except: A. It is a postganglionic sympathectomy B. Sympathetic trunk is divided below the first sympathetic ganglion C. It is a preganglionic sympathectomy D. Usually done by extraperitoneal approach

4. Following are uses of Doppler probe except: A. Used to feel the nonpalpable pulse B. To look for pressure index C. To measure blood pressure D. To detect tripbasic pattern

11. Infra inguinal bypass surgery for diabetic ulcer­ following are true except: A. Long saphenous vein is inferior to PTFE graft B. Preoperative vein marking is helpful C. 2 years patency is around 70% D. Amputation rate after bypass surgery is still high

5. Lumbar sympathectomy has following advantages except: A. Rest pain improves to a small extent B. Ulcerations heal C. Claudication and claudication distance improves D. Nutritive value of the blood flow improves

12. Following are major risk factors for atherosclerosis except: A. Dyslipidaemia B. Tobacco smoking C. Hyperhomocystinaemia D. Alcohol intake

6. Fogarty catheter is used in: A. Chronic occlusion of artery B. Acute embolic occlusion of an artery C. Vasospastic disease D. Femoral vein thrombosis

13. Following are true for profundoplasty except: A. It is done by using patch of dacron 8. It is done by using vein graft C. Done after endarterectomy D. Done in TAO patients

7. Characteristic feature of critical limb ischaemia has one of these: A. Intermittent claudication B. Ankle pressure is less than 70 mm of Hg C. Toe systolic pressure is less than 30 mmHg D. Absent pulses

14. About tissue plasminogen activator, following are true except: A. First purified from melanoma cells B. It is an endogenous enzyme like urokinase C. It is sometimes elevated in carcinoma stomach D. Streptokinase also has plasminogen activating action

ANSWERS 1 D 11 A

2 B 12 D

3 D 13 D

4 D 14 C

5 C

6 B

7 C

8 A

9 C

10 A

8 Upper Limb lschaemia and Gangrene • • • • •

• • • • •

Raynaud's disease Thoracic outlet syndrome Axillary vein thrombosis Vasculitis syndrome Gangrene, cancrum oris

Acrocyanosis Drug abuse and gangrene Iatrogenic drug-induced gangrene Subclavian steal syndrome What is new?/Recent advances

Introduction Upper limb ischaemia (ULI) is a well recognised clinical entity as lower limb ischaemia, though it is less common. There are certain specific conditions which are responsible for ULI such as cervical rib, Raynaud's disease, etc. It is also important to note that reconstructive surgery is rarely done in the upper limb when compared to lower limb. However, students should be able to identify the ischaemic features in the upper limb early and refer the case to a suitable specialist, so that it can be treated promptly and adequately, thereby avoiding a tragedy such as loss of the limb. Causes of upper limb ischaemia 1. 2. 3. 4. 5. 6. 7.

Raynaud's disease and Raynaud's syndrome Embolic causes Thoracic outlet syndrome (Fig. 8.1) Trauma Buerger's disease Axillary vein thrombosis Vasculitis syndromes • Takayasu's arteritis, giant cell arteritis, polyarteritis nodosa • Systemic sclerosis-sclerodenna-CREST syndrome RAYNAUD'S DISEASE (PRIMARY RAYNAUD'S PHENOMENON)

•

It occurs in young women, commonly. Upper limb is more involved than lower limb

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Fig. 8.1: Gangrene of the tip of fingers due to cervical rib

• Commonly seen in western countries in white-skinned people. Cold climate is possibly a precipitating factor. It was first described by Raynaud as bilateral episodic digital ischaemia of the upper limb on exposure to cold and emotions. It is also referred to as primary Raynaud's phenomenon. • Raynaud's phenomenon is the blanket term used to describe cold-related digital vasospasm (see pathophysiology). Raynaud's phenomenon is subdivided into Raynaud's syndrome where there is an associated disorder and primary Raynaud's disease where there is none. • CREST syndrome:Calcinosis circumscripta, Raynaud's phenomenon, (0) Esophageal defects, Sclerodactyly, Telangiectasia.

Upper Limb lschaemia and Gangrene

Ml............,.� SECONDARY RAYNAUD'S PHENOMENON CAUSES

• Atherosclerosis • Systemic lupus • Scleroderma

• Cervical rib • Carpal tunnel syndrome • Vibrating tools-vibration white finger

• Causes of Secondary Raynaud's phenomenon are given in Key Box 8.1. Currently, both primary and secondary varieties have been grouped together. Pathophysiology On exposure to cold 1, some kind of discomfort and colour changes are observed. This is due to abnom1al sensitivity of the arterioles to cold. Three stages have been described. 1. Stage of syncope: Arterioles undergo constriction as an abnormal response to cold. As a result of this, the part becomes blanched and severe pallor develops. 2. Stage of asphyxia: After a brief period of vasoconstriction, capillaries dilate, filling with deoxygenated blood resulting in bluish discolouration of the part (cyanosis). 3. Stage of recovery or stage of rubor: As the attack passes off, relaxation of the aiterioles occurs, circulation improves and redness occurs. Because of dilatation of capillaries, red engorgement of the part occurs, which causes tingling, burning or bursting pain in the fingers. Clinical features • Affects young women. • Typically causes bilateral episodic digital ischaemia on exposure to cold. • Thumb is usually spared. • Peripheral pulses are normal. • Pallor, cyanosis and rubor are the colour changes occurring during the attack along with pain. • In a few patients, because of recurrent attacks, gangre­ nous patches occur on the tip of the fingers (superficial necrosis). Differential diagnosis • Cervical rib • Vasculitis syndromes • TAO affecting the upper limb usually affects male smokers. Peripheral pulses are feeble or weak. 1Cold refers to temperature-cold climate (winter), cold environment (refrigerator), or cold substance like cold water or ice. Erythromelalgia is a condition wherein heat provokes an attack of burning pain of hands and feet.

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Treatment I. • • • •

Conservative line of treatment Reassurance Avoid unnecessary exposure to cold Avoid smoking Calcium antagonists such as nifedipine I 0-20 mg, two times a day may be beneficial. • If these measures fail, surgery is undertaken. II. Cervical sympathectomy (Key Boxes 8.2 and 8.3) • ln this operation, sympathetic trunk from the lower half of the stellate ganglion to just below the 3rd thoracic ganglion is removed. • Upper 1h of the stellate ganglion is preserved to avoid Homer's syndrome. • All rami communicantes associated with the 2nd and 3rd ganglia are removed. • Nerve of Kuntz, a grey ramus which springs from the 2nd thoracic ganglion to the 1st thoracic nerve is also divided. • Commonly done through a supraclavicular route and axillary route. Thoracoscopic sympathectomy is becoming popular. • Sympathectomy raises the threshold at which spasm occurs (but the effect seems to be temporary). However, the severity of the disease is reduced.

............................. . KEY BOX 8.2

• • • • •

INDICATIONS FOR CERVICAL SYMPATHECTOMY

Raynaud's disease TAO Hyperhidrosis Cervical rib Causalgia

............................. . KEY BOX 8.3

• • • • •

COMPLICATIONS OF CERVICAL SYMPATHECTOMY

Perforation of pleura causing pneumothorax Lymph fistula due to injury to thoracic duct Homer's syndrome Injury to the accessory nerve Haemorrhage

Other vasospastic disorders These are rare (Key Box 8.4)

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............................. . KEY BOX 8.4

OTHER VASOSPASTIC DISORDERS Acrocyanosis

• Women with cyanosis of hands and feet • Cutaneous vasoconstriction is the cause

C8 nerve root

Livedo reticularis

• Spasm of arterioles and dilatation of venules • Worsening by cold • May be associated with systemic lupus erythematosus (SLE}

Subclavian artery

Erythromelalgia

• Burning sensation of hands and feet due to heat First rib

THORACIC OUTLET SYNDROME SURGICAL ANATOMY OF THORACIC OUTLET •

Thoracic outlet is a tight space with bony structures all around (Key Box 8.5) such as manubrium sternum in the front, spine posteriorly and first rib laterally. At the root of the neck, brachia] plexus and subclavian artery pass through scalene triangle into the axilla. Scalenus triangle is the posterior compartment of costoclavicular space. The division into anterior and posterior compartments is by scalenus anticus. The anterior compartment contains subclavian vein.

•

............................. . KEY BOX 8.5

• • • • • •

INTRODUCTION TO THORACIC OUTLET

Tight space with bones all around Brachia! plexus and subclavian artery are chief contents Scalenus anticus muscle is the muscle for landmark Vascular compression is more dangerous Neurological symptoms are often undiagnosed Boundaries of scalene triangle (Fig. 8.2) Base : First thoracic rib Anteromedially : Scalenus anticus Posterolaterally : Scalenus medius If the base (the first thoracic rib) is raised by interposition of cervical rib or any other causes (Key Box 8.6), it results in compression of the subclavian artery.

PATHOPHY SIOLOGY OF CERVICAL RIB WI TH COMPRESSION •

Due to slow compression, artery distal to the compression dilates due to jet-like effect and turbulence of blood flow. This is described as poststenotic dilatation (Venturi effect) (Fig. 8.3 and Key Box 8.7).

Fig. 8.2: Scalene triangle

............................. . KEY BOX 8.6

THORACIC OUTLET SYNDROME-CAUSES �

• Transverse process of C7-long • Hyperabduction syndrome-compression by pectoralis minor • Operative scars-fibrous bands • Rib-cervical rib • Anomalous first rib-abnormal • Costoclavicular syndrome-compression between clavicle and first rib • Insertion of scalenus-anomalous (Scalenus anticus syndrome) • Callus-malaligned fracture clavicle Remember as THORACIC

............................. . KEY BOX 8.7

SUBCLAVIAN ARTERY OCCLUSION EFFECTS Lumen narrowing Fibrosis or thickening of arterial wall Stenosis Poststenotic dilatation Multiple thrombi � Embolism � lschaemia

In this dilated segment, small multiple thrombi develop, which, when dislodged, result in emboli and distal ischaemia. Vascular symptoms are strictly unilateral.

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Clinical features

Dilated segment with thrombus

Common in young females. Even though congenital. symptoms appear only at or after puberty. This is because of the development of shoulder girdle muscles and sagging of the shoulder which narrows the root of the neck. Nerve roots C8, TI are stretched by completion of growth around 25 years. •

Dull-aching pain in the neck is caused by expanded bony end of cervical rib.

•

Features of upper limb ischaemia - Claudication pain is apparent when the arm with muscle wasting is used. Low temperature, pallor, excessive sweating (vasomotor disturbances), splinter haemor­ rhages, ischaemic ulcers in fingers and gangrene of the skin of the fingers are the other features. Peripheral pulses may be absent/feeble. Oedema and venous distension are very rare. These are called vascular symptoms of cervical rib.

•

Features of ulnar nerve weakness (lower nerve roots involvement, mainly first thoracic nerve) manifest as tingling and numbness, or paraesthesia in the distribution of C8, T l . The following are the tests which confirm ulnar nerve weakness. It includes sensory disturbances and motor disturbances (performing fine action-writing, buttoning, etc.) A. Card test 1 : The patient is asked to hold a thin paper or a card in between the fingers. In cases of ulnar nerve paralysis, due to weakness of interossei muscles, the patient will not be able to hold the card tightly (Fig. 8.5). B. Froment's sign: Patient is asked to hold a book between the hand and the thumb. In cases of ulnar nerve paralysis, since the adductor pollicis is paralysed, there is flexion at the distal interphalangealjoint of the thumb.

Fig. 8.3: Poststenotic dilatation

Cervical rib • This is an extra rib present in the neck in about 1-2% of the population. • Commonly unilateral and in some cases it is bilateral. • It is more frequently encountered on the right side. • It is the anterior tubercle of the transverse process of the 7th cervical vertebra that attains excessive development and results in cervical rib. Types of cervical rib (Fig. 8.4) Type I

The free end of the cervical rib is expanded into a hard, bony mass which can be felt in the neck. Type II Complete cervical rib extends from C7 vertebra posteriorly to the manubrium anteriorly. Type III Incomplete cervical rib, which is partly bony, partly fibrous. Type IV A complete fibrous band which gives rise to symptoms but cannot be diagnosed by X-ray.

Type I

Type Ill

Type II

Type IV

Fig. 8.4: Four types of cervical rib (see text) 1Card test and Froment's sign are not seen in cases of cervical rib

Fig. 8.5: Card test

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Fig. 8.6: Froment's sign

This is because flexor pollicis longus, which is supplied by median nerve, contracts (Fig 8.6). • Adson's test: Feel radial pulse, ask the patient to take deep inspiration and turn the neck to the same side. The pulse may disappear or it may become feeble. This test indicates compression on subclavian artery (Fig. 8.7). • Hyperabduction test (Halsted test): This test is done to rule out hyperabduction syndrome caused by pectoralis minor. The radial pulse becomes weak on hyperabduction due to angulation of axillary vessels and brachial plexus, which gets compressed between pectoralis minor and its attachment to the coracoid process. • Military attitude test: When shoulders are set in backward and downward positions the radial pulse becomes weak. This is due to the compression of subclavian artery between the clavicle and the first rib. This is seen in costoclavicular syndrome.

Pallor can be seen in the palm. Now release pressure on the radial artery and watch for the blood flow. Repeat test for ulnar artery. If there is occlusion of either artery, colour changes occur in the fingers slowly (Fig. 8.8). • Elevated arm stress test-EAST (Roos'): Patient is asked to abduct the shoulders to 90 degrees and to flex the elbow. Then he is asked to pronate/supinate forearms continuously. Appearance of symptoms suggests thoracic outlet syndrome. • A hard mass may be visible or palpable in the root of the neck (Type I). • On palpation of supraclavicular region, a thrill and on auscultation, a bruit can be heard in cases of poststenotic dilatation. Differential diagnosis A patient who presents with a few neurological symptoms and signs in the upper limb with a cervical rib may be having some other causes for those symptoms. Hence, it is important to exclude other causes. 1. Cervical spondylosis: This should be considered as a possibility in patients above the age of 40 years. 2. Cervical disc protrusion and spinal cord tumours may mimic cervical rib with predominant neurological features. 3. Carpal tunnel syndrome can occur due to various causes such as myxoedema, rheumatoid arthritis and malunited Colles' fracture. Predominant features of median nerve involvement, more so in menopausal women gives a clue to the diagnosis. 4. Raynaud's phenomenon 5. Costovertebral anomalies 6. Pancoast tumour.

• Allen's test: Ask the patient to clench his fist tightly and compress the radial and ulnar arteries at the wrist with the thumbs. Wait for 1 Os and ask the patient to open his hands.

Investigations 1. X-ray neck may show a cervical rib (Types I, II and III). Interestingly Type IV variety, a fibrous band which cannot be diagnosed by X-ray or by any other investigation, usually gives rise to symptoms (Fig. 8.9).

Fig. 8.7: Adson's test

Fig. 8.8: Allen's test

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Il. Surgery (Key Box 8.8) • Presence of vascular symptoms and signs are the definite indications for surgery. • Excision of cervical rib including periosteum is called extraperiosteal excision of cervical rib (so that it will not regenerate) . This is combined with cervical sym­ pathectomy if vascular symptoms are predominant. • If there is a thrombus in the subclavian artery, it is removed and the artery is repaired (Fig. 8.11 ).

..

........................... .

KEY BOX 8.8

CERVICAL RIB SURGERY (Figs 8.12 and 8.13) � • • • • •

Remove cervical rib Repair subclavian artery Restore circulation Reduce vasospasm-sympathectomy Recognise other causes

Fig. 8.9: X-ray of the neck showing cervical rib

2. Cervical disc protrusion and spinal cord tumours may have predominant neurological features and thus may mimic cervical rib. When in doubt, ask for MRI. 3. Duplex scan of affected limb, to detect any aneurysm. Treatment (Fig. 8.10) I. Conservative • Patients with mild neurological symptoms are managed by shoulder girdle exercises or correction of faulty posture.

I

Fig. 8.11: Subclavian artery is exposed to repair the dilatation and remove the thrombus

Cervical rib j Symptoms j

Local

I

• Pain • Visible lump • Asymptomatic

I

• Visible lump • Hard lump • Fixed lump

I

Neurological

• Claudication pain • Cold and pale hand • Ulcers of the fingers

• Tingling and numbness • Muscle wasting • Muscle paralysis

I

I

Vascular

Signs j

I

I

• Weak pulses • Ulcer or gangrene • Adson's test positive

• Weakness • Wasting of hypothenar muscles • Card test, Froment's sign

[ Treatment ] j Exercises J

Failure

Fig. 8.10: Summary of cervical rib

Cervical sympathectomy

. .

Extrapenosteal exc1s1on

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Fig. 8.13: Cervical rib removed Fig. 8.12: Cervical rib at surgery

• At exploration, ifcervical rib is not found, scalenus anterior muscle is divided. This is called scalenotomy (Key Box 8.9). Ifhyperabduction syndrome is diagnosed, pectoralis minor is divided from its insertion into the coracoid process.

............................. . KEY BOX 8.9

IF CERVICAL RIB IS NOT FOUND

• Scalenotomy • Division of pectoralis minor • Extraperiosteal resection of the first rib

AXILLARY VEIN THROMBOSIS •

• • •

Patients present with swelling ofarm after intense activity from the dominant hand Hypertrophy of subclavius muscle also can cause compression of subclavian-axillary vein (sportsman) Peripheral pulses will be normal Venography to diagnose thrombus Thrombolysis or if necessary venotomy, removal of the thrombus and I st rib (if it is the cause of obstruction) are the treatment modalities. Axillary vein thrombosis is also a complication of axillary block dissection specially where extensive nodal dissection has been done. VASCULITIS SYNDROMES

TAKAYASU'S ARTERITIS (PULSELESS DISEASE) • H is of unknown aetiology. • Commonly affects females (85%).

• It is a panarteritis involving aortic arch and its branches. Subclavian artery is involved in 85% of the cases. Clinical features It starts as a generalised inflammatory disease-fever, body ache, malaise and arthralgia • Upper limb claudication (Key Box 8.10) • Absence of peripheral pulses • Hypertension is common in 50% of the cases due to renal artery involvement. • Bruit may be heard over the subclavian artery • V isual disturbances can occur due to involvement ofretinal arteries. Late blindness can occur. Pathology It is a panarteritis, involving all layers of elastic arteries. Thrombosis and stenosis can occur later.

............................. . KEY BOX 8.10

• • • • •

VASCULITIS SYNDROMES

Aetiology is inflammatory or immunological. Uncommon causes of upper and lower limb ischaemia. Women are affected more than men. Multiple small vessel involvement. Symptoms are confusing, depending upon organ involve­ ment. • lschaemic changes are minimal and superficial when it involves the limbs. • Steroids are useful in controlling the disease. • lmmunosuppression should be tried carefully.

Upper Limb lschaemia and Gangrene

Investigations • C-reactive protein is elevated as part of acute phase response (nonspecific). • Duplex-Doppler ultrasound, MR angiography can diagnose the site of obstruction and blood flow pattern. Treatment • Very early cases benefit with steroid therapy, e.g. tablet prednisolone 30 to 50 mg/day (anti-inflammatory effect). Cyclophosphamide can be tried when other measures fail (immunosuppressive effect). • Vascular reconstruction-difficult. GIANT CELL ARTERITIS • It is also called temporal arteritis. • Elderly women presenting with severe headache is the common presentation. • Fever and malaise may also be present. • Involvement of various arteries will result in various symptoms (Key Box 8.11). • Palpable, pulsatile, tender temporal arteries will clinch the diagnosis.

............................. . KEY BOX 8.11

VESSEL INVOLVED

SYMPTOMS

• • • • •

Headache Jaw pain Sudden blindness Claudication Myocardial infarction

Temporal artery Facial artery Retinal artery Upper limb artery Coronary artery

• Biopsy of the temporal arteries will reveal giant cell granuloma, comprising mainly CD4+ T lymphocytes. • Treated with prednisolone 60-80 mg/day and slowly tapered over 1-2 years. • Relapses and remissions are common. POLYARTERITIS SYNDROME • This includes microscopic polyarteritis (commonly) and polyarteritis nodosa (less often). • This syndrome also has an inflammatory reaction. • Ischaemia of the lower limbs and upper limbs can occur due to involvement of small vessels.

............................. . KEY BOX 8.12

PREGANGRENE-SYMPTOMS • • • •

• Pallor on elevation Rest pain is the main symptom Congestion on dependent position Guttering of veins • Poor capillary filling Thickening or scaling of skin

105

• Abdominal pain is due to involvement of visceral vessels. • Involvement of renal arteries causes loin pain, haematuria and hypertension. • Treatment is similar to other diseases mentioned above. SYSTEMIC SCLEROSIS-SCLERODERMA • Earlier called collagen vascular disorder because of obstruc­ tion of the small vessels by collagen deposition. • Now included under vasculitis syndromes because of their association with inflammatory reaction. • Ischaemic changes occur in the fingers and toes. Necrosis and ulceration are common. • Oesophageal involvement results in dysphagia. • Small bowel sclerosis results in disordered motility and malabsorption. • Sympathectomy and vasodilators may be useful. • Raynaud's symptoms can be controlled using calcium channel blockers and nitrates. GANGRENE Definition Macroscopic death of tissue with superadded putrefaction. It affects the limbs, intestines, appendix, etc. In this chapter differential diagnosis of causes of gangrene of the limbs is considered (Figs 8.14 to 8.19). Pregangrene (Key Box 8.12) Rest pain, colour changes at rest and with exercise, oedema, hyperaesthesia, skin ulcerations are due to inadequate blood supply to the limb. These changes are described as pregangrenous changes in the limb. Classification of gangrene 1. Cardiovascular causes • TAO • Atherosclerotic gangrene • Acute embolic gangrene • Syphilitic gangrene • Raynaud's disease • Cervical rib • Vasculitis syndrome • Polycythemia 2. Neurological causes: Hemiplegia, paraplegia, bedsore 3. Traumatic gangrene • Direct-thrombosis; indirect-crush injuries 4. Physical causes: Sunrays, radiation, corrosive acids 5. Drugs: Ergotamine 6. Diabetic gangrene 7. Acute infective gangrene: Boil, carbuncle, cancrum oris, gas gangrene.

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VARIOUS TYPES OF GANGRENE (Figs 8.14 to 8.19)

Fig. 8.14: Peripheral gangrene due to sepsis and vasopressors

Fig. 8.17: Left medial 2 fingers gangrene due to TAO

Fig. 8.15: Emboli at the brachia! artery resulting in massive gangrene of the hand (wet gangrene)

Fig. 8.18: View of the dorsum of the hand

Fig. 8.16: Case of SLE. Observe pulp of fingers

Fig. 8.19: Embolic gangrene of fingers

Upper Limb lschaemia and Gangrene

Clinical features of gangrene • A part which is gangrenous is a dead portion of the body. It has no arterial pulsations, venous return or capillary filling. • It has no sensation. • The colour initially will be pale and later it changes to dusky grey and finally black. The black colour is due to disintegration of haemoglobin and formation of iron sulphide. • Signs of gangrene (Key Box 8.13). • The gangrenous part has to be treated with surgical excision or debridement, which may amount to either disarticulation of the toe or even an amputation. • The gram-positive, gram-negative and anaerobic organisms multiply in this segment and can produce septicaemia. Thus, this may precipitate multiorgan failure including renal failure, adult respiratory distress syndrome (ARDS), cardiac failure, etc.

............................. KEY BOX 8.13

.

Malnourishment Major diseases such as diphtheria, whooping cough, typhoid, measles and kala azar. • As a result of these factors the oppo1tunistic organisms such as Vincent's organisms-Borrelia vincentii and B. fusiformis multiply and cause multiple ulcers, erosions and later, fibrosis. • Occasionally, as the disease progresses, whole thickness of the cheek may be lost. Treatment of cancrum oris 1. Ryle's tube feeding 2. Improve nutrition 3. Appropriate antibiotics: Metronidazole 400 mg three times a day for 7-10 days. 4. Reconstructive surgery may be necessary later. Complications of cancrum eris I. Fibrosis causing restriction of the movement of jaw. 2. Septicaemia, toxaemia and death.

SIGNS OF GANGRENE • Loss of pulsation • Loss of temperature • Loss of function

• Loss of colour • Loss of sensation

Clinical types Basically there are two types-dry gangrene and wet gangrene. They are compared in Table 8.1.

ACROCYANOSIS • It is also called hereditary cold extremities. • Persistent cyanotic discolouration of hands when exposed to cold is a feature. • This is brought about by intermittent spasm of small peripheral vessels. Commonly affects hands, rarely feet also. • Generally, it is mild and nonprogressive.

SPECIAL TYPES OF GANGRENE CANCRUM ORIS • It is an extensive ulcerative disease of cheek mucosa occurring in malnourished children. • Precipitating factors are:

107

DRUG ABUSE AND GANGRENE • Abuse of the drugs is an important cause of gangrene in the modern days. • Inadvertent injection of drugs into artery can lead to thrombosis of the artery resulting in acute ischaemia­ commonly in the brachia! artery.

Comparison of dry gangrene and wet gangrene Cause

Dry gangrene -------

Involvement of part Local findings

Slow occlusion of the arteries Small area is gangrenous due to presence of collaterals Dry, shrivelled and mummified Usually present

Line of demarcation Crepitus Odour

Absent Absent

Infection Diseases Treatment

Not present TAO, atherosclerosis Conservative amputation

Wet gangrene Sudden occlusion of the arteries Large area is affected due to absence of collaterals Wet, turgid, swollen, oedematous Absent May be present Foul odour due to sulphurated hydrogen produced by putrefactive bacteria Usually present Emboli, ligatures, crush injuries Major amputation is necessary

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• Emergency treatment in symptomatic cases include heparinisation and infusion of dextran. • In severe cases, emergency angiography and intra-arterial thrombolysis is considered. IATROGENIC DRUG-INDUCED GANGRENE • Inadvertent intra-arterial injection of thiopentone into one of the high divisions of brachia] artery, (congenital anomaly) usually the ulnar, will result in severe burning and blanching of the hand (Figs 8.20 and 8.21 ). • If this complication is noticed following steps (measures) have to be taken immediately. Case of inadvertent intra-arterial injection (few important steps) • After the injection, initial signs and symptoms occur very fast (within 15-20 seconds). It consists of intense forearm pain and mottling of the skin on his hand. • Minutes later, discolouration and nail bed pallor became evident. Approximately 3 to 4 hours later, the symptoms had progressed to paraesthesias and pronounced hand weakness. • Rapid development of signs indicative of necrosis (by the eighth day) required the patient to undergo fasciotomies, multiple debridements, and 4 skin grafts for cosmesis. Step 1: If iatrogenic, maintain the intra-arterial catheter in place-do not remove it Step 2: Identify the progress of the disease-colour changes, necrosis, gangrene Step 3: Initiate anticoagulation-diluted heparin intra­ venous and subcutaneous Step 4: Institute symptomatic relief and plan for rehabilitation-analgesics, physiotherapy Step 5: Elevation of extremity, antibiotics Step 6: Perform specific interventions-angiogram, intra­ arterial thrombolysis, vasodilators, prostacyclines, sym­ pathectomy, corticosteroids Step 7: Aim is to save the limb-last will be amputation

Inadvertent brachia! artery injections Do not remove the needle Inject 5 ml of 1 % lignocaine or 2% papaverine sulphate Followed by Dilute heparin (intra-arterial) Intra-arterial thrombolysis using streptokinase

Brachia! plexus block

Intravenous low molecular weight dextran

To decrease the sympathetic spasm

Fig. 8.21: Management of inadvertent brachia! artery injections

MISCELLANEOUS ERGOT AND GANGRENE • Ergot preparations are used by patients with migraine over a long period. • Ergotamine gangrene occurs in those who eat bread infected with Claviceps purpurea. Example: Dwellers on the shores of the Mediterranean Sea and the Russian Steppes. SUBCLAVIAN STEAL SYNDROME • Development of symptoms in either brain or in the arm because of subclavian artery obstruction is referred to as subclavian steal syndrome (Key Box 8.14). • It is most commonly used to describe reversed flow in the vertebral artery ipsilateral to proximal subclavian artery stenosis or occlusion. • Surgical correction is by endarterectomy or bypass graft. • Transluminal balloon angioplasty is another alternative.

............................. KEY BOX 8.14

SUBCLAVIAN STEAL SYNDROME • It is more common on the left side • Vertebrobasilar symptoms-dizziness, vertigo, imba­ lance • Arm symptoms-fatigue, pain with exercise, para­ esthesias, coolness and heaviness on the affected side • Asymmetrical radial pulses or difference of 20 mmHg systolic pressure between the upper limbs. WHAT IS NEW IN THIS CHAPTER?/RECENT ADVANCES

Fig. 8.20: Gangrene following intra-arterial injection

• A few Key Boxes and figures have been added. • Subclavian steal has been discussed. • A few vasospastic diseases have been added. • inadvertent intra-arterial injection

.

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109

MULTIPLE CHOICE QUESTIONS

1. Which of the following is usually not a feature of Raynaud's disease? A.Radial artery pulsations are absent B. Upper limb is involved C. Cervical sympathectomy is helpful D. Pallor, cyanosis and rubor are the colour changes during the attack

7. Following are true for subclavian artery except: A. Cervical rib can compress the artery B. It gives internal mammary a1tery C. Post stenotic dilatation occurs once it is compressed by cervical rib D. It continues as axillary artery at the medial border of first rib

2. FoUowing are true for cervical sympathectomy except: A. It raises the threshold at which spasm occur B. Entire stellate ganglion has to be removed C. It is the treatment of choice in hyperhydrosis D. Up to 3rd thoracic ganglion has to be removed

8. Compression of the subclavian artery is detected by following clinical test: A. Froment's sign B. Adson's test C. Allen's test D. Halsted test

3. Following muscle is the landmark to thoracic outlet: A. Scalenus anticus B. Scalenus medius C. Scalenus posterior D. Levator scapulae

9. Following are causes of splinter haemorrhages except: A. Bacterial endocarditis B. Cervical rib C. Scleroderma D. Aortic regurgitation

4. Following are components of giant cell arteritis except: A. Severe headache is presenting complaint B. Temporal artery is usually not involved C. Biopsy from artery reveals giant cell granuloma D. CD4+ T lymphocytes are found in histopathology

10. Following are true about subclavian steal syndrome except: A. More common on the left side B. Present with vertebrobasilar symptoms C. Both radial arteries are normal D. Significant arm symptoms are present

5. Hypertension is a common finding in following conditions except: A. Polyarteritis nodosa B. Phaeochromocytoma C. Polycystic disease of kidney D. Hyperthyroidism

J l. Following are the causes of upper limb ischaemia except: A. Ergotamine alkaloids B. Cervical rib C.TAO D.Atheroma of the brachia! artery

6. Following is the treatment of choice for cervical rib with ischaemia: A. Excision of the rib B. Excision of the rib with cervical sympathectomy C. Excision of the rib with division of pectoralis minor D. Excision ofrib with division ofscalenus anticus muscle

12. Which is the test to detect the dominant arterial supply in the hand circulation? A. Froment's sign B. Adson's test C. Allen's test D. Halsted test

ANSWERS 1 A

2B

3 A

4 B

5D

6 B

7D

8 B

9D

10C

11D

12C

9 Lymphatics, Lymph Vessels and Lymphoma • • • • • • •

Lymphoedema-anatomy and physiology Lymphatic circulation Primary lymphoedema Secondary lymphoedema Lymphangiography Hodgkin's lymphoma Non-Hodgkin's lymphoma

Burkitt's lymphoma Sezary's syndrome Chyluria lmmunohistochemistry Bone marrow and peripheral blood stem cell transplants • What is new?/Recent advances

• • • • •

Introduction Lymphatics and lymph vessels play the role of draining the waste fluid from the body. Hence, they are vulnerable for various infections. The lymphatics are connected to a group oflymph nodes and then drain into the veins. Hence, infections of the lymphatics give rise to enlargement of lymph nodes. In this chapter, significant surgical diseases affecting the lymphatics and lymph nodes are discussed. LYMPHOEDEMA Definition Accumulation of lymph in the extracellular, extravascular compartment and subcutaneous tissues results in enlargement of the limb. It is protein-rich interstitial fluid. Common sites of lymphoedema • Lower limbs are the most common sites. • Upper limbs • Scrotum: Elephantiasis of the scrotum is caused by filarial organism (Wuchereria bancrofti). • Elephantiasis of penis caused by filarial organisms produces Ram's horn penis (Fig. 9.1). Anatomy and physiology Functions of the lymphatic system • To return protein-rich fluid to circulation through lymphaticovenous junctions in the jugular area.

Fig. 9.1: Complication of filariasis-elephant leg, elephantiasis of scrotum and Ram's horn penis

• This fluid includes water, electrolytes, low molecular weight substances such as polypeptides, growth factors and cytokines. • It also includes macromolecules such as fibrinogen, albumin and globulin. • Transpor t of choleste rol, long chain fatty acids, triglycerides and fat soluble vitamins (A, D, E, K) by intestinal lymph into circulation. They bypass liver and enter circulation through cistema chyli and thoracic duct into the left internal jugular vein (lymph from lower limbs, abdomen and left arm). • Right lymphatic duct drains into right internal jugular vein (lymph from head and right arm).

110

Lymphatics, Lymph Vessels and Lymphoma

........... KEY BOX 9.1

111

__,

.................

.

�

RISK FACTORS FOR LOWER LIMB LYMPHOEDEMA • • • •

Right ----,'--f', lymphatic duct

Inguinal block dissection, e.g. carcinoma penis, melanoma Postoperative pelvic radiotherapy Varicose vein stripping and vein harvesting Obesity RISK FACTORS FOR UPPER LIMB LYMPHOEDEMA

• • • • •

Upper limb lymphatics

Fig. 9.2: Major lymphatics and lymphatic ducts

Components of lymphatic system • Lymphatic channels (Fig. 9.2) • Lymphoid organs-lymph nodes, spleen, Peyer's patches, thymus, tonsils • Circulating cells-lymphocytes and mononuclear immune cells. Lymphatic circulation • Lymph flow is largely due to intrinsic lymphatic contractility by lymphangions (segment of lymphatics). • Transient increase in interstitial pressure secondary to exercise and limb movements also helps to a certain extent. • Valves prevent reflux in the lymphatics (Fig. 9.3). Types of lymphoedema I. Primary lymphoedema 2. Secondary lymphoedema Risk factors of lymphoedema (Key Box 9.1) Initial lymphatics (Blind-ended, larger 50 mm) Drain into Terminal lymphatics (Have bicuspid valves and endothelial cells rich in contractile protein actin)

Lymph trunks

Axillary block dissection, e.g. carcinoma breast Radiation fibrosis, scar formation Advanced cancer-breast with axillary lymph nodes Obesity Chronic infection

PRIMARY LYMPHOEDEMA (CONGENITAL) Introduction • Incidence is 1 in 6000 persons. • Lymphoedema is confined to epifascial plane. • They are due to inherited abnormality of lymphatic system. • A few cases which occur later in life may be due to unnoticed factors-repeated bacterial and fungal infections, trauma to feet, etc. • Loss of venoarteriolar reflux (VAR) which protects lower limb capillaries from excessive hydrostatic forces in the erect posture, occurs in advancing age. Diseases such as diabetes and chronic venous insufficiency may also contribute. Causes of primary lymphoedema I. Hereditary: Associated with syndromes such as Turner's (XO), Klinefelter's (XXY), Down's (Trisomy 21). II. Familial 1. Noone-Milroy-type I • Occurs in 1 :6000 live births • Inherited as autosomal dominant trait • Brawny lymphoedema of both legs-genitalia, arms, face, etc. develops from birth (Fig. 9.4). 2. Meige's disease-type II • Lymphoedema develops between puberty and middle age (50 years). It may involve arms also. • A few are inherited in an autosomal dominant manner. Pathophysiology (Fig. 9.5)

• Accompany neurovascular bundles except in cortical bony skeleton and CNS. • Single layer of endothelial cells • Pulsation of vessels also helps circulation

Fig. 9.3: Lymphatic circulation

PEARLS

OF

WISDOM

Lymphoedema leads to an impairment of immune surveillance and predisposes to other malignancies (Key Box 9.2).

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Types of primary lymphoedema

• Lymphatic aplasia: Number of lymphatic channels am nodes are grossly reduced. • lymphatic hypoplasia: In this variety the lymphati1 channels are of small calibre. • Milroy's disease is a type oflymphoedema congenita whicl runs in families. • Depending upon the time at which lymphoedema appears it can be classified as follows: Lymphoedema congenita. Birth Lymphoedema praecox. Puberty Later life Lymphoedema tarda. SECONDARY LYMPHOEDEMA (ACQUIRED) Fig. 9.4: Congenital lymphoedema-observe the right leg. One attempt has been made to decrease lymphoedema by excision

Lymphatic aplasia, hypoplasia, dysmotility, obliteration, surgical removal Lymphatic hypertension and distension Lymphostasis + lymphotension

Accumulation of proteins, growth factors, particulate matter including bacteria End results Protein rich-fluid + increased deposition of ground substances + subdermal fibrosis, dermal thickening and proliferation Lymphoedema Fig. 9.5: Pathophysiology of lymphoedema

1. Filarial elephantiasis (Fig. 9.6) is caused by Wuchereria bancrofti, transmitted by the mosquito (Culexfatigans). The disease is caused by adult worms which have the affinity towards lymphatic vessels and lymph nodes. Microftlariae do not produce any lesion. • Initially, it causes lymphangitis which clinically presents with high grade fever, chills and rigors, red streaks in the limb, tenderness and swelling of the spermatic cord and scrotum (Table 9.1). • T he lymph nodes are swollen and tender. Retro­ peritoneal lymphangitis produces acute abdominal pain. • Due to such repeated infections, fibrosis occurs resulting in lymphatic obstruction. Later, this gives rise to lymphatic dilatation. Lower limb lymphatics are dilated and tortuous (lymphangiectasis). • To start with, lymphoedema is pitting in nature and progressively becomes nonpitting in nature. Lymph (protein) provides good nourishment for fibroblasts. • After repeated infections, the skin over the limb becomes dry, thickened, thrown into folds and even nodules that break open and result in ulcers, Hence, it is called 'elephant leg'. Lack ofnutrition and infection precipitates lymphoedema. Oedema is also due to reflux of lymph

Clinical manifestations of lymphatic filariasis Site of involvement

Acute

Chronic

L ower limbs

Lymphangitis, lymphadenitis

Lymphoedema, chronic lymphadenitis

Scrotum

Lymphangitis

Lymphoedema, chylocoele

Spermatic cord

Acute funiculitis

Chronic thickened cord

Epididymis and testis

Acute epididymo-orchitis

Chronic epididymo-orchitis

Abdomen

Acute retroperitoneal lymphangitis

Chyluria, lymphadenovarix

Breast

Lymphangitis

Lymphoedema

Lymphatics, Lymph Vessels and Lymphoma

.....

..

. . .

KEY BOX 9.2

.

.................

.

. .

..

. .

...

KEY BOX 9.3

MALIGNANCIES ASSOCIATED WITH LYMPHOEDEMA Skin cancers

. .

.................

113

.

ELEPHANT LEG

Filarial lymphangitis

• Squamous cell carcinoma • Malignant melanoma • Basal cell carcinoma

Lymphatic obstruction

Sarcomas

Lymph stasis

• Lymphang iosarcoma (Stewart-Treves syndrome)­ sarcoma arising in a lymphoedema after treatment of carcinoma breast • Kaposi's sarcoma • Liposarcoma • Malignant fibrous histiocytoma

Recurrent lymphangitis Transudation of albumin

Systemic disease

Lymphoedema (pitting)

• Lymphoma

Coagulation of fluid Repeated infection Lymphoedema (nonpitting)

Fig. 9.6: Filarial lymphoedema

2. 3. 4. 5.

from para-aortic vessels into the smaller lymphatics draining the lower limb. Subcutaneous tissue is grossly thickened. The presence of deep fascia prevents involvement of the deep muscles of the lower limb (Key Box 9.3). After inguinal block dissection for secondaries in the lymph nodes (upper limb lymphoedema following axillary block dissection). Following radiotherapy to lymph nodes Advanced malignancies Repeated infections due to barefoot walking (Fig. 9.7).

Symptoms • Swollen limb, dull-aching pain • Tiredness, pins and needles, cramping pain • Loss of ankle contour • Buffalo hump on the dorsum of the foot • Toes are square (Table 9.2) • Skin on the dorsum of the toes cannot be pinched because of subcutaneous fibrosis-Stemmer 's sign • In early cases, it pits on pressure. Later, oedema does not pit and does not reduce even with elevation. • Advanced cases: Chronic eczema, fungal infections (dennatophytosis) and nails (onychomycosis), fissuring and warts. • Ulceration is unusual. • However, once ulcers start, recurrent bacterial infections are common, thus worsening the disease process.

Clinical features of lymphoedema Early signs

Change into

1. Loss ofankle contour

• • •

2. Buffalo hump on the dorsum of feet 3. Stemmer's sign: Skin on dorsum of 2nd toe cannot be pinched because ofsubcutaneous fibrosis. 4. Early pitting on pressure, less on rest, elevation

Late/advanced cases-signs

1. Chronic eczema Fibrosis Dermal thickening 2. Fungal infection-dermatophytosis 3. Fungal infection ofnails-onychomycosis Hyperkeratosis 4. Fissuring, verrucae, papilla 5. Grossly thickened leg with skin rugosity-elephant leg.

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3. Elephantiasis neuromatosa of the leg can cause diffuse enlargement of the leg. The leg is tender on palpation with soft to finn diffuse swelling. 4. Extensive lipomatosis of the leg. Investigations (Lymphangiography) By and large, no investigations are done in secondary lymphoedema. In selected cases of primary lymphoedema, investigations can be done but are largely of academic interest. Comparison of primary and secondary lymphoedema is shown in Table 9.3. Causes of lymphoedema are shown in Key Box 9.4 and Table 9.4.

.

.

. . .

.

. .

KEY BOX 9.4

..

.

.................

.

SUMMARY OF CAUSES OF LYMPHOEDEMA� Fig. 9.7: Severe lymphangitis

Grades of filarial lymphoedema Grade I

- Oedema-pitting: Completely relieved on rest and elevation. No skin changes. Grade II - Oedema-pitting: Partially relieved on rest and elevation. No skin changes. Grade III - Oedema-nonpitting: Skin involvement, subcutaneous thickening present. Grade IV - Oedema-non pitting: Not relieved, warty projections, elephantiasis, lymphorrhoea present. Differential diagnosis of unilateral elephantiasis of the leg 1. Filariasis is the most common cause of elephantiasis of the leg in endemic areas such as coastal Karnataka, coastal Andhra Pradesh, Tamil Nadu, etc. (Table 9.3). 2. Congenital A-V fistula can present with unilateral gigantism of the leg. Dilated veins, continuous murmur, gigantism, nonhealing ulcer in the leg in a young boy give the clue to the diagnosis.

• • • • • • •

Aplasia, hypoplasia (familial and genetic) Parasitic (filariasis) Lymph node obstruction-advanced malignancies Altered motility-dysmotility (genetic) Surgical extirpation-block dissection Inflammatory/Infection-recurrent After radiotherapy, after barefoot walking 'silica particles'. Remember as APLASIA

LYMPHANGIOGRAPHY Lymphangiography is an investigation wherein a dye is injected into the lymphatics and the entire draining lymphatics and lymph nodes are visualised. Indications for lymphangiography 1. Lymphoedema, if surgery is planned. 2. In cases of lymphoma, to detect pelvic nodes, para-aortic nodes, etc. but now CT scan is preferable. Procedure • Commonly, pedal lymphangiograms are done.

Comparison of primary and secondary lymphoedema Primary lymphoedema

Secondary lymphoedema

It is due to congenital aplasia and hypoplasia

Filariasis is the common cause

Slowly progressive

Rapidly progressive

It is seen in younger age group

Middle age group

Females are more often affected

Males are more commonly affected

Unilateral, begins distally, spreads proximally

Sometimes, it can start proximally-unilateral or bilateral

Capillary haemangioma may be present

Absent

Regional lymph nodes are absent

Lymph nodes are grossly enlarged

Excisional operations are indicated

Excisional operations and other types of surgery

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115

Causes of secondary lymphoedema (Figs 9.8 to 9.10) Cause

Pathology

I. Infections

Filariasis Tuberculosis Chronic lymphadenitis

Recurrent lymphangitis Lymphadenitis Lymphatic destruction

2. Surgery

Lymph node block dissections (inguinal or axillaiy)

Excision of lymph nodes as part of treatment

3. Lymph node obstruction

Postradiotherapy large nodes-metastasis lymphoma

Scarred and obliterated lymphatics

4. Tissue damage

Bums scan"ing

Loss of lymphatics

5. Venous diseases

Chronic venous insufficiency, venous ulcer

Lymphatic destruction

6. Endocrine

Pretibial myxoedema

Oblfreration of initial lymphatics by mucin

Fig. 9.8: A case of carcinoma of the breast presented with lymphangitis

Fig. 9.9: Postmastectomy lymph­ oedema of the right upper limb

Fig. 9.10: Postmastectomy lymphoedema­ progressive since 6 years

• 5-10 ml of methylene blue (patent blue) is injected into the web spaces intradermally between the toes. This delineates the lymphatics of the dorsum of the foot which are identified. Then, an oily dye such as "ultra fluid lipoidol" is injected (10-15 ml). • It may take 12-24 hours to delineate inguinal nodes and para-aortic nodes. • Isotope lymphangiography refers to injection of albumin labelled with technetium 99m colloid or 131 I.

at birth, is progressive, the whole leg is involved and shows variable response to compressive therapy. 2. Distal obliteration (80%) (Key Box 9.5) 3. Proximal obliteration (10%) • It is less common • Obstruction is at aortoiliac or inguinal nodes region. • Whole leg/thigh is involved (Fig. 9.11). • No family history • Rapid progression and poor response.

Results • Metastases appear as iITegular filling defects in the lymph nodes. • It may demonstrate hypoplasia or hyperplasia as in primary 1ymphoedema. • If there is obstruction, the dye may return back (dermal back flow).

Complications of lymphangiography 1. Lymphangitis and toxaemia • It is not being done routinely nowadays, because of availability of ultrasound, CT scan and other noninvasive investigations.

Lymphangiographic classification Three types are recognised 1. Congenital hyperplasia (10%): This is the condition wherein lymphatics are increased in number. These megalymphatics are defective resulting in chylous ascites, chylothorax and end in intestines resulting in protein loss (protein-losing enteropathy). Being congenital, it manifests

..

. . . . .

KEV BOX 9.5

....

.................

DISTAL OBLITERATION (80%) Distal lymphatics are commonly affected In and around puberty-praecox involved Sex: Common in females Treatment is by compression, good response Ankle, calf region is involved Lymphatics are decreased or absent (aplasia) Remember as DISTAL

.

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Manipal Manual of Surgery

• Massage: Manual lymphatic drainage (MLD) • It aims at draining fluid and protein from ISF space. • It also stimulates lymphangion contraction. 2. Care of skin (Key Box 9.7) • Wash daily with warm water • Moisturiser or liquid paraffin should be applied for dr) skin • Avoid injuries • Hyperkeratosis should be treated by keratolytic agern such as 5% salicylic acid. • Avoid ointments/herbal medicines/soaps, etc. which are allergic. • Antifungal treatment • Treat infections early and effectively.

Fig. 9.11: Unilateral limb oedema due to proximal obliteration

2. Rapid infusion is to be avoided for the fear of lipid pulmonary embolus. Lymphoscintigraphy • It has a sensitivity and specificity of92% and 100% respectively. • It has replaced lymphangiography. • Radiolabelled (technetium 99m) colloid is injected into web space between 2nd and 3rd toes or fingers. Limb is exercised periodically and images are taken. • If there is abnormal accumulation of tracer with collaterals, it is a sign of lymphoedema. • MRI and CT scan are the latest investigations in addition to lymphangiography for the evaluation of gross swelling of the limb. Treatment of lymphoedema PEARLS

OF

WISDOM

Surgery has a small role in lymphoedema I. Conservative 1. Control of swelling: Decongestive lymphoedema therapy (DLT) • Bedrest, elevation • Bandaging: Multilayered lymphoedema bandaging (MLLB-Key Box 9.6). • Compression garments

.......... KEY BOX 9.6

.

.................

.

MLLB • Graduated pressure highest at ankle (100%) to lowest in the groin (40%) should be applied. • It requires about 4Q-60 mmHg to leg and 3Q-40 mmHg to arm. • MLLB should be worn at the start of the day and removed at bed time. • However, limb should be kept elevated at night. • The aim is to get a graduated pressure in applying. If it is applied wrongly, it may result in increase in lymphoedema.

3. Relief of pain • Pain is multifactorial. It can be due to swelling, infection, involvement of nerve or bone, etc. • However, it is often psychosomatic and affected by mood and morale. • Antianxiety drugs may help. 4. Control of infections • Usually it is due to streptococci/staphylococci. • Oral or injectable penicillin and flucloxacillin or clindamycin are the drugs of choice. • Other drugs are co-amoxiclavulinic acid, cephalosporins. 5. Exercises • 40% of lymph is formed within skeletal muscle. • It is directly proportional to central inflow. • Slow systemic, isotonic movements such as swimming and massage will increase venous and lymphatic return. • Foot end of the bed should be elevated. 6. Drugs • Oxerutins are the drugs used for venous disease. These are not yet licensed in the UK but are used in India.

.

..........

KEY BOX 9.7

• • • • • • • • • •

.................

GUIDELINES FOR TREATMENT OF A PATIENT WITH LYMPHOEDEMA

L Lymphoedema bandage is multilayered V Manual lymphatic drainage (MLD) or massage Prophylactic antifungal treatment to prevent athlete's foot. Hygiene of skin and foot Advice on diet-weight reduction Treatment of infection-early and aggressive Instructions about exercises Compression garments Surgery consultation as and when required. Remember as LYMPHATICS

.

117

Lymphatics, Lymph Vessels and Lymphoma

ut,t< .. :'.::if 2 cm in greatest dimension (or) tumour of any size with 2 or more high-risk features. Tumour invasion of maxilla, mandible orbit or temporal bone. Tumour with invasion of skeleton or perineural invasion of skull base No regional lymph nodes Metastasis in a single ipsilateral lymph node� 3 cm Metastasis in a single ipsilateral lymph node > 3 cm but < 6 cm; or B/L or contralateral lymph nodes none> 6 cm in greatest dimension Metastasis to lymph node > 6 cm in greatest dimension No metastases Distant metastases

Investigations Wedge biopsy from the edge of the ulcer. The edge is selected because of the following reasons: • Edge is the growing part, malignant cells are numerous • Centre has slough or scab which may not reveal malignancy • Comparison with normal skin is possible. Histology (Key Box 11.2 and Fig. 11.12) Types: 1. Basosquamous 2. Morpheaforrn 3. Adenoid 4. Infiltrative Treatment (Fig. 11.17) • Basal cell carcinoma responds well to radiation. Surgical excision also cures the disease. • Surgery is the first line of treatment for basal cell carcinoma. I. Surgery is indicated when the lesion is: Very close to the eye, adherent to the cartilage or bone • In easily, accessible sites such as neck and hand. • In radiation failure cases. Wide excision is done in all cases: This means excision of the growth with at least 3-4 mm of healthy margin all around including at the base. The resulting defect is closed by: a. Primary suturing of the defect if the lesion is small (Figs 11.18 to 11.20) b. Skin grafting if defect is big as in neck or dorsum of hand c. Rotation flaps as in face for better cosmetic effect (Figs 11.13 to 11.16).

Skin Tumours

. /1)�_ diJ:!i \I \I II V

Fig. 11.13: Reconstruction with nasolabial flap following excision

149

Mohs' micrographic surgery: It is a special surgical technique which involves excision of skin cancer under microscopic control. It minimises recurrence and maximises conservation of surrounding normal tissue. The technique offers complete evaluation of the lateral and deep margins of the tumour excision. Indications for Mohs' procedure • Centrofacially located tumours • Large tumours Poorly defined tumour margins • Recurrent lesions • Lesions with perineural/perivascular involvement • Tumours at site of prior radiation therapy • Tumours in the setting of immunosuppression • High-risk histological subtype of BCC Advantage Better cosmetic results since minimal amount of normal tissue is removed. II. Radiation is indicated in elderly patients who have an extensive lesion which requires a complicated plastic reconstruction. Dosage: 4000-6000 cGy units. Radiation chondritis and osteitis are the complications (see Fig. 11.17 for summary of the treatment of basal cell carcinoma).

Fig. 11.14: BCC excision and reconstruction by using bilobed flap

III. Other forms of treatment for basal cell carcinoma • Small and superficial: Curettage with electrodesiccation • Liquid nitrogen for tumours less than 1 cm in diameter • CO2 laser in basal cell carcinoma (Key Box 11.3)

J Basal cell carcinoma )

I

Rhomboid flap

Surgery • Radiorecurrent cases • Accessible areas • Nearer the eye, nose, cartilage, ear, etc.

I

Radiotherapy • Unfit patients for surgery • Large lesions which need complicated surgical procedure

I

Dosage 4000-6000 cGy units [ Wide excision ] Types Direct closure (small lesion) Banner's flap

Figs 11.15 and 11.16: BCC reconstruction

Split skin grafting (big lesion)

Flaps (better cosmetic effect)

Fig. 11.17: Treatment of BCC

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Fig. 11.18: Pigmented, elevated lesion-5 years Fig. 11.19: Wide excision in progress duration

Fig. 11.20: Primary closure

(Courtesy: Dr Balakrishna Shetty, Professor of Surgery, KS Hegde Medical Academy, Deralakatte, Mangalore, Karnataka)

............................. . KEY BOX 11.3

CO2 LASER IN BASAL CELL CARCINOMA • Useful in superficial BCC that are confined to epidermis and papillary dermis. • At least 4 mm surrounding healthy skin should be removed. • Patients with multiple tumours or in hereditary Gorlin's syndrome-laser is very useful. • Acts by superficial vaporisation • Pathological margins cannot be examined. • Indication in high risk BCC-large (> 2 cm), located near eye, nose, ear. • Precancerous/low grade BCC

Reconstruction following tumour excision 1. Direct closure: In majority of cases, this is possible. 2. Skin graft • Skin graft does not look like normal skin. However, it provides a good skin cover • Cannot be used across a joint • When aesthetic result is important. Skin graft will not take on • Cortical bone without periosteum • Cartilage without perichondrium • Tendon without paratenon • Irradiated tissues. 3. Commonly used skin flaps • Rotation flap: It is a semicircular flap of skin and subcutaneous tissues which rotates about a pivot point into the defect to be closed. Small triangle of skin can be removed to facilitate rotation.

• Advancement flap: Defect is closed by stretching the skin. It can be single, double, or V-Y advancement flap. • Transposition flap: Bilobed and rhomboid flap are few examples. A rectangular piece of skin and subcutaneous tissue is rotated (details below). RECONSTRUCTION OPTIONS IN HEAD AND NECK 1. Healing by secondary intention Simplest method: Suitable only if defect is < 1 cm, superficial defects and located in cosmetically inconspicuous sites. Disadvantage: Not always cosmetically acceptable, might result in contour irregularity, distortion of surrounding structure and unstable coverage. 2. Primary closure • Simple but defect needs to be small. • Possible only if excessive tension and distortion can be avoided. 3. Skin grafts • Cosmetically acceptable but does not give colour and contour match. • Preferred if patient has high risk of local recurrence such as malignant melanoma. • Types: Split and full thickness grafts. 4. Local flaps • Advancement flap • Transposition flaps • Rotation flaps, e.g. cervicopectoral flap

Skin Tumours 5. Local composite flaps Flaps like PMMC flap, deltopectoral flap 6. Free flap Radial artery based free forearm flap, fibular free flap LOCAL FLAPS Advancement flaps • V-Y flap • Good for defects on medial cheek and ala of nose. A. V-shaped incision is made and then advanced to cover the defect and later sutured in a 'Y' shape. B. Transposition flap 1. Banner flap: Provides excellent contour and reasonable colour match. • Can be used for small defects only. • Disadvantage: Produces a conspicuous scar. 2. Bilobed flap: Banner flap uses a single lobe. Instead, in this flap, two lobes are created and then rotated. • Used when defect is large enough not to be closed with banner flap. • Especially useful in area around the nose. • Produces conspicuous scar and is less desirable than banner flap. 3. Rhomboid flap: Geometric modification of Banner's flap. It can be used for medium-sized defects (details in page 149). RECONSTRUCTION OPTIONS FOR LIPS Most important factors that dictates reconstruction is amount of remaining lip vermilion. A. Primary closure for small defects B. Vermilion advancement flap C.Abbe Flap • Mainly used for upper lip • It is harvested based on labial artery

151

• Provides good cosmetic effect • Causes minimal loss of function in lower lip. Modification of Abbe flap like reverse Abbe flap can be used for lower lip defects also. D. Estlander flap • Rotates the upper lip to lower lip • Disadvantage: Produces a round commissure and loss of normal taper of vermilion. E. Webster-Bernard flap • Medial advancement of cheek tissue to create a new lower lip. • Gives a good result • Disadvantage: Results in significant facial scarring. Very large lower lip defects can also be closed with 'Double central severe Abbe's flap' or Bilateral Karapandzic flap (see page 289). SQUAMOUS CELL CARCINOMA (EPITHELIOMA) It is the second common malignant skin tumour after basal cell carcinoma. It arises from prickle cell layer of the Malpighian layer of the skin. It usually affects elderly males. All the premalignant conditions listed earlier apply to this condition. It can also occur de nova in the skin. Basosquamous carcinoma is the term applied when squamous cell carcinoma arises in a pre-existing basal cell carcinoma. It is interesting to note that a variety of names have been given to squamous cell carcinoma when it occurs in different places. They are given in Table 11.2. Pathological types • Ulcerative variety-commonest • Cauliflower like or proliferative growth • Ulceroproliferative type See Table 11.3 for the typical sites of skin, mucous membrane and junction involvement.

Sites of squamous cell carcinoma Site

Name

Reason/explanation

Skin of the abdomen or back of thigh in Kashmiri patients

Kangri cancer

Buttocks, heels, elbows Scrotum

Kang cancer Chimney sweep cancer

Abdominal wall

Saree cancer and Dhothi cancer Countryman's lip

Kangri is the name given to the pot containing hot charcoal which is applied to the abdominal wall because of excessive cold in Kashmir (India) Tibetans sleep on the oven bed due to excess cold Seen in chimney sweepers due to prolonged irritation by chemicals such as tar or pitch in Due to chronic irritation caused by wearing dhothi or saree too tight Carcinoma lower lip is common in agriculturists (outdoor occupation)

Lower lip

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Typical sites of squamous cell carcinoma Skin

Junction

Mucous membrane

• Dorsum of hand • Limbs • Face

• Between skin and mucous membrane • Lip • Penis • Vulva

• Lined by stratified squamous epithelium as in oral cavity and oesophagus • Lined by columnar epithelium, wherein squamous metaplasia occurs, such as gall bladder, bronchus • Lined by transitional cell epithelium with metaplasia as in urinary bladder

• Abdominal wall

Clinical features • Most lesions are preceded by actinic keratosis. Such lesions grow slowly and are locally invasive without metastasis. If not from keratosis, lesions are more aggressive and metastatic. • Typically, it is an ulcerative or cauliflower-like lesion (Fig. 11.21). • The edge is everted and indurated (Fig. 11.22). • The base is indurated and it may be subcutaneous tissue, muscle or bone. • The floor contains cancerous tissue which looks like granulation tissue. It is pale, friable, bleeds easily on touch (Figs 11.23 to 11.27). • Surrounding area is also indurated. • Mobility is usually restricted due to infiltration of underlying structures. In very early cases, ulcer can be moved along with skin over the underlying structures. • Regional lymph nodes such as inguinal lymph nodes (both vertical and horizontal groups) can get enlarged when squamous cell carcinoma affects lower limb or abdominal wall. Hard lymph nodes are suggestive of secondaries. Spread 1. Local spread occurs by infiltration into the surrounding

tissues. Depending upon the site, various structures can be involved. Some examples are: • Tendon involvement in the dorsum of the hand. • Muscle involvement in the abdominal wall. • Bone involvement, e.g. tibia in carcinoma developing in a varicose ulcer or mandible in carcinoma cheek.

2. Lymphatic spread is the chief method of spread even though it occurs relatively late. Regional nodes are involved first. • Nodes which are soft to firm and tender are due to secondary infection. • Nodes which are hard, nontender, with or without fixity are due to secondary deposits. • In untreated cases, nodes start ulcerating through the skin resulting in bleeding and pain.

• As already stated, nodes do not get involved in Marjolir ulcer. 3. Blood spread is rare and late. PEARLS

OF

WISDOM

Epithelial pearls are absent in poorly differentiated carcinomas. Differential diagnosis (Key Box 11.4) Investigations A wedge biopsy from the edge of the ulcer or growth is taken. However, in proliferative lesions punch biopsy can also be taken. Microscopic picture: Eighty per cent of these cancers are well differentiated (Key Box 11.5). It is characterised by central structureless mass of keratin surrounded by normal looking squamous cells which are arranged in concentric manner like onion skin. This whole appearance is called epithelial pearl or cell nest (Fig. 11.28). In 20% of cases, cells are undifferentiated with numerous mitoses, without keratinisation.

............................. . KEY BOX 11.4

DIFFERENTIAL DIAGNOSIS • • • • •

Basal cell carcinoma Keratoacanthoma Papilloma Pyogenic granuloma Tuberculous ulcer

Typical sites Benign self-limiting Benign lesion Painful, short duration Uncommon

............................. . KEY BOX 11.5

BRODER'$ CLASSIFICATION I. II. Ill. IV.

Well differentiated Moderately differentiated Poorly differentiated Anaplastic

75% keratin pearls 50% keratin pearls 25% keratin pearls < 25% keratin pearls

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Fig. 11.21: Squamous cell carcinoma affecting dorsum of the hand-ulceroproliferative lesion

Fig. 11.23: Epithelioma scalp-large ulcerating bleeding lesion

Fig. 11.22: Squamous cell carcinoma-bleeds on touch

Fig. 11.24: Squamous cell car­ cinoma arising in leprosy scar

Fig. 11.26: A large epithelioma of the scalp-biopsy proved squamous cell carcinoma

Fig. 11.25: Squamous cell carcinoma of the sole

Fig. 11.27: Bowen's disease with squamous cell carcinoma

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Fig. 11.28: Classical keratin pearls-well-differentiated squamous cell carcinoma (Courtesy: Dr. Laxmi Rao, Head, Department of Pathology, KMC, Manipal)

Treatment Treatment can be classified as treatment of the primary and treatment of the secondaries. I. Treatment of the primary Squamous cell carcinoma is treated by wide excision or radiotherapy. A. Surgery (Figs 11.29 and 11.30) • It involves removal of growth along with 1 cm of normal healthy tissue from the palpable indurated edge of the tumour, in high-risk cases. • Low-risk lesions can be excised with 4-6 mm margin. Indications for surgery • When the lesion is small and superficial • When the lesion has involved deeper tissues such as muscles, cartilage or bone • Radiorecurrent cases Reconstruction: After wide excision, the defect can be closed primarily, with split skin graft or with a flap to reconstruct the

part depending upon the extent of resection. If the growth i fixed to tibia, below knee amputation is the treatment. B. Radiotherapy Indications for radiotherapy Primary I. Patients unable or unwilling to undergo surgical treatmen for primary lesion 2. When clear margins cannot be obtained by Mohs' o extensive surgery 3. Adjuvant treatment 4. Histologically aggressive, e.g. perineum! invasion 5. High grade 6. Bone invasion (Fig. 11.31) 7. Lymph nodes-adjuvant or palliative intent

II. Treatment of secondaries I. Thirty to forty per cent of the enlarged regional nodes an due to secondary infection. Once the primary is treated and antibiotics are given, lymph nodes may regress. In such cases, 'wait and watch policy' is observed. 2. If lymph nodes do not regress or are hard and mobile, FNAC can be done to confirm the diagnosis followed by radical block dissection. Thus, squamous cell carcinoma of the leg requires inguinal block dissection. 3. If lymph nodes are hard and fixed to the femoral vessels, palliative radiotherapy is given. Even in advanced fungating lesions, the response rate to radiotherapy is reasonably good. • Dose: 3000--4000 cGy units over 3--4 weeks, 200 units/day. Structures removed in inguinal block dissection • The superficial group of nodes consisting of a horizontal chain which lies below inguinal ligament and a vertical chain which lies along upper 5-6 cm of long saphenous vein. These two groups of nodes form the letter T. • The deep glands are located alongside the proximal end of the femoral vein, and one lying within femoral canal. • Fat, fascia, lymphatics are cleared from 2 cm above the inguinal ligament up to 2 cm below saphenofemoral junction. The medial clearance is important up to femoral canal. 8-10 cm long saphenous vein near its termination is removed to facilitate lymph node clearance. Inguinal block dissection • The saphenous vein can be preserved, nodal tissue is removed from the vessel circumferentially; otherwise, it is sacrificed. • Cloquet's node is sent as a separate specimen for frozen section examination PEARLS OF

Fig. 11.29: Wide exci­ sion of squamous cell carcinoma

Fig. 11.30: Wide excision followed by skin grafting on 3rd year follow-up

WISDOM

Fixity to muscle, veins, fascia and bones is not a contraindication for block dissection. Carotid and femoral artery infiltration makes it inoperable.

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155

Fig. 11.31: Large penetrating baso-squamous cell carcinoma with destruction of the left ear and infiltrating mastoid process and external ear

Complications of inguinal block dissection and treatment • Wound infection � Broad-spectrum antibiotics � Adequate drainage • Lymphorrhoea • Haemorrhage � Perfect haemostasis • Flap necrosis � Avoid sharp comer • Femoral blow-out � Sartorius muscle slide to cover femoral vessels at the end of surgery Summary of the treatment of squamous cell carcinoma (Fig. 11.32) Squamous cell carcinoma

Primary

Surgery

or

Wide excision followed by reconstruction (a) Split skin grafting (b) Flap reconstruction

Secondaries in lymph nodes Radiotherapy

1. • 2. •

Mobile lymph nodes Radical block dissection Fixed lymph nodes Palliative radiotherapy is given

Fig. 11.33: Hard lymph node in the deep inguinal region suggestive of metastasis-see clinical notes

Prognostic factors (Key Box 11.6)

1911!1111...........� PROGNOSTIC FACTORS OF sec

• Invasion: Deeper the lesions or SCC less than 2 mm, metastasis is unlikely. If it is greater than 6 mm-15% sec may have metastasised • Broder's grade higher: Worse prognosis • Site: Lip and ear-high chances of recurrence • lmmunosuppression: Poor prognosis • Perineural involvement: Worse prognosis

6000 cGy units

Recurrence occurs

A patient who had undergone wide excision and SSG for a growth behind the knee, presented with oedema of the leg. The MBBS student could not explain oedema. He said the cause is nutritional. Groin had 3-4 hard nodes.

Recurrence occurs

MELANOCYTIC TUMOURS Radiotherapy

Treated by surgery

Advanced cases where both fail Chemotherapy

Fig. 11.32: Summary of the treatment of squamous cell carcinoma

Simple melanocytic tumours These are also called pigmented naevi which are composed of modified melanocytes derived from the neural crest. All naevi have excess melanin pigmentation because of which they are tan brown or black in colour. They are located in the basal layer of the epidermis. They are benign. They are of following types:

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1. Junctional naevus: Located within the epidermis at the dermoepidermal junction. They are common in children. They appear as tan brown to black macules. They are smooth, flat and hairless moles. As they enlarge, they become slightly raised and can evolve into an intradermal or a compound naevus. It has no malignant potential. Junctional naevus commonly occurs on the palm, sole, digits and genitalia. 2. Compound naevus: As the mole enlarges, naeval cells also appear in the dermis along the intraepidermal cells. Such moles are described as compound naevi. These are found usually in adolescents and are usually benign (Fig. 11.34).

Fig. 11.34: Compound naevus Fig. 11.35: Dysplastic naevi over the face

3. Intradermal naevus: It is the most common mole in adults. Because of its deep seated nature, it appears blue. Hence, the name blue naevus. It is seen on the scalp and face. It contains hair and does not become malignant. 4. Spitz naevus: Commonly occur on face and legs. Grows rapidly initially and then remains static. It appears as reddish brown nodule-occasionally deeply pigmented. 5. Spindle cell naevus: Occur in women. Common site is the thigh. It has a malignant potential. 6. Congenital pigmented naevus which is present at birth, has a greater potential for malignant change. It can involve extensive areas of the skin (Giant). Giant congenital pigmented naevus (GCPN) or giant hairy naevus. • It is an example of hamartoma of naevomelanocytes. Involves extensive areas of the skin. • Naevus cells are present not only in epidermis but also in the subdermal fat and muscle • Risk of malignant melanoma is about 10% • Malignant melanoma tends to be axial • Removal is for aesthetic and oncological reasons. 7. Dysplastic naevi are different from acquired naevi in the following ways: • Malignant potential is more (Figs 11.35 and 11.36) • Family members may have such lesions. • Such syndrome is described as familial dysplastic naevus syndrome. MALIGNANT MELANOMA1 (MELANOCARCINOMA) It is a malignant tumour arising from pigment-forming cells (melanoblasts) which are derived from the neural crest. Melanoblasts and melanocytes convert dihydroxyphenyl-

Fig. 11.36: Dysplastic naevus left temporal region

alanine (DOPA) into melanin. This is called positive DOPA reaction (Key Box 11.7). It is a potentially curable tumour in early stages. If left untreated or if not treated properly, it disseminates rapidly, showers the body with tumour emboli and offers a very painful death. Some interesting most have been given in Key Box 11.8. 1Malignant melanoma is the killer skin cancer of whites. "Bronzed Body

Beautiful" concept should be discouraged.

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157

............................. . ............................. . KEY BOX 11.7

KEY BOX 11.9

RISK FACTORS FOR DEVELOPING MALIGNANT MELANOMA (MM)

POSITIVE DOPA REACTION TYROSINE j, f- TYROSINASE DOPA j, f- OXIDASE MELANIN

............................ KEY BOX 11.8

•

• lmmunocompromised-HIV, Hodgkin's disease treated with cyclosporin

.

• Melanoma excised earlier • Early childhood burns and eyes blue, red hair • Tendency to freckle

• Most benign form of melanoma is lentigomaligna melanoma. • Most common form of melanoma is superficial spreading melanoma. • Most malignant type of melanoma is nodular melanoma. • Most reliable independent prognostic indicator in malignant melanoma is tumour thickness based on Breslow classification. • Most of them (majority) arise from pre-existing moles. • Most of the melanoma are pigmented. However, pigmentation is not mandatory for diagnosis of melanoma.

Common sites of malignant melanoma • Head and neck 20-30% • Lower extremity 20-30% • Trunk 20-30% • Remaining cases occur in upper extremities, genitalia, choroid of the eye 1• Aetiopathogenesis (Fig. 11.37) For risk factor study (Key Box 11.9) 1. Ultraviolet rays: It is more common in white-skinned

people. There is a linear correlation between intensity of exposure to sunlight and malignant melanoma in white­ skinned people2 . White-skinned people who live close to

Genetic factors

• Genetic-History of dysplastic naevus

• Naevus: GCPN and total number > 20 naevi

INTERESTING 'MOST' FOR MALIGNANT MELANOMA

Congenital skin conditions

Pigmentosa xeroderma

Sunlight

White delicate skin

Sunlight

High society lady

Fig. 11.37: Aetiopathogenesis 1Choroid has no lymphatics. Hence, excellent prognosis. 2Malignant melanoma is the killer skin cancer ofwhites. "Bronzed Body Beautiful" concept should be discouraged.

Remember as PIGMENT

the equator have increased tendency of developing malignant melanoma. The highest incidence is found in Queensland (Australia). For the same reasons, malignant melanoma is common in the United Kingdom, North America and Australia. 2. Age and sex: Malignant melanoma is more common in females. The higher incidence of the disease is found during reproductive age period. Even though oestrogen and progesterone receptors are found in malignant melanoma in some patients, their true role is not yet established. Leg is the commonest site in females. In general, the incidence of melanoma is higher in men than in women. The incidence of melanoma is 1. 7 times higher for women than men before 39 years of age. After 70 years of age, the incidence of melanoma is 2.2 fold higher for men than women. 3. Genetic factors • Tumour suppressor gene mutation 9p2 l • Deletion or rearrangement of chromosomes l O and 8p. • Familial atypical multiple, mole, melanoma syndrome­ previously known as dysplastic naevus syndrome • Multiple, large> 5 cm, atypical dysplastic nevus in areas covered by clothing. 4. Genetic factors: Increased incidence has been found in patients with familial dysplastic naevus syndrome. The disease is also common in individuals with Celtic race who give family history of malignant melanoma (3-5%). 5. Trauma: Malignant melanoma occurs in the sole of the foot in African blacks. Whether trauma is the cause is not clear. 6. Pre-existing mole: Approximately I/3rd of melanomas arise in a pre-existing mole, remaining I/3rd arise de novo in the normal skin. Malignant change occurs in the junctional or compound naevus. Malignancy should be suspected when following changes occur in a mole (Key Box 11.10).

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............................. . KEY BOX 11.10

GLASGOW-CHECK LIST (Figs 11.38 and 11 .39� 1. Change in size 2. Change in shape 3. Change in colour 4. Inflammation 5. Crusting, ulceration, bleeding 6. Sensory change

7. Diameter> 6 mm

7. Increased incidence of malignant melanoma has been found in patients with renal transplantation and leukaemia as a result of imrnunosuppression. 8. Xeroderma pigmentosum and albinism patients are susceptible for melanomas. PEARLS

OF

Figs 11.38 and 11.39: Only complaint of this 28-year-old lady was an increase in the size and oozing from the lesion

WISDOM

"Bronzed, Blue Eyed, Blond, Body Beautiful-high chances of malignant melanoma" Pathology Microscopic picture: Anaplastic, pigment laden melanocytes confined to epidermis. The cells which have vacuolated cytoplasm (Paget's cells resembling those seen in Paget's disease of the breast) are found. Cells also invade the dermis. Along with pigment-laden macrophages, dermal infiltration of lymphocytes 1 may be present. Rarely, anaplastic melanocytes do not form pigment (amelanotic melanoma). All melanomas ( except nodular) show radial growth initially in the form of intraepidermal growth. However, nodular melanoma has vertical growth phase thus involving dermis leading to nodule formation. This has poor prognosis. Pathological grading of malignant melanoma (Fig. 11 .40) Clarke s level of invasion Level I Tumour cells confined to basement membrane Level 2 Tumour extension into papillary dermis Tumour reaches the interface between papillary Level3 dermis and reticular dermis Level4 Tumour reaches reticular dermis Level 5 Tumour invades subcutaneous fat. It is no longer followed. Breslow described staging depending upon the maximum thickness at the centre of the lesion. 1Presence of lymphocytes may be an indication of host response 'Fight' against cancer.

Fig. 11.40: Malignant melanoma with malignant cells showing dark melanin pigment inside. Second picture is a close-up view (Courtesy: Dr Laxmi Rao, HOD, Pathology, KMC, Manipal)

159

Skin Tumours Clinical types of malignant melanoma (Figs 11.41 to 11.44) Name

Nature

Behaviour

Lentigo malignant melanoma

Arises from Hutchinson's melanotic freckle Occurs in old people on face and temporal region Any part, more in the trunk Any part, more in the leg Located on the palm, sole, digits Difficult to diagnose

Least common (5%) Least malignant Most common (70%) Most malignant, invasive (15-30%) Presents late, aggressive (4-8%) Rare, nonpigmented (5%)

Superficial spreading Nodular Acral lentiginous Amelanotic

Stage I Stage II Stage III Stage IV

Thickness less than 0.75 mm 0.76 to 1.5 mm 1.51 to 3.0 mm More than 3 mm

• Less than 1 mm is regarded as thin melanoma.

Clinical type (Table 11.4) Clinical features •

Malignant melanoma can present as changes in the pre­ existing mole which are already described (Key Box 11.11). • The patient can present as a nonhealing ulcer of the sole of the foot. • It is a painless ulcer • Edges are irregular • Floor is irregular • Bleeds on touch • Typically, the ulcer is pigmented (Fig. 11.41 ). In l 0% of patients, pigment is absent. They are called amelanotic melanoma. • Lesion is firm in consistency and induration is absent. • A halo may be present surrounding the ulcer. • The lesion moves with the skin and is usually not fixed to underlying structures. • Satellite nodules (within 5 cm of the primary ) may be found surrounding the lesion which are due to spread through intradermal lymphatics (Fig. 11.4 7). Such patients will have greatly enlarged, firm, nontender nodes. • In-transit lesion-disease found in the dermis or subcutaneous tissue more than 2 cm away from the primary melanoma but before the regional lymph node basin (Figs 11.45, 11.46 and 11.48) See Key Box 11.12 for acral lentiginous variety.

............................. . KEV BOX 11.11

A B C D E

ABCDE OF EVALUATING A CHANGING MOLE Asymmetry: One half does not match the other Border irregular: Ragged or blurred Colour variation: Tan, black, brown Diameter: > 6 mm Evolving (elevation): Change in a pre-existing lesion

............................. . KEV BOX 11.12

ACRAL LENTIGINOUS (Fig. 11.43) Least common subtype and very aggressive Popularly called hand and foot melanoma Occurs in palms and soles This subtype occurs in dark-skinned people (21-22%). In subungual region-blue black discolouration of posterior nail fold (most common on great toe or thumb). • Pigmentation in proximal or lateral nail folds (Hutchinson's sign)

• • • • •

AMELANOTIC MELANOMA (Fig. 11.44) Lesion appears pink It has poor prognosis than nodular melanoma. PEARLS OF

WISDOM

In all cases of suspected me/anomalous skin lesions examine axillae, scalp, soles, genitalia, oral cavity and interdigital webs also.

TNMstaging

••;jfifif#fil!Hij

AJCC 6th Edition

Tumour TO No tumour Tis in situ tumour T 1a < 1 mm, level II, level Ill no ulceration T 1b < 1 mm, level IV with ulceration T2a 1-2 mm no ulceration T2b 1-2 mm with ulceration T3a 2-4 mm No ulceration T3b 2-4 mm with ulceration T4a > 4 mm no ulceration T4b > 4 mm with ulceration

Node NO-no nodes N1a-one node micrometastasis N 1b-two nodes macrometastasis N2a-2 or 3 nodes micrometastasis N2b-2 or 3 nodes macrometastasis N2c-no nodes but satellite or in-transit lesions N3-4 more nodes; nodes with satellite or in-transits, matted nodes

M-Metastasis MO-no blood spread M1 a-skin, subcutaneous tissue, distant node-normal LDH M1b-lung spread-normal LDH M1c-other viscera or distant spread and increase in LDH with any metastasis

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Fig. 11.42: Nodular

Fig. 11.41: Superficial spreading

Fig. 11.45: Observe the foot, little toe has been amputated five years back for malignant melanoma

Fig. 11.46: Same patient as in Fig. 11.45 presented to the hospital with in-transit deposits

CLINICAL STAGING* Stage O

Tis

Stage IA

T 1a

Stage IB

T1b T2a

Stage IIA

T2b T3a

Stage IIB

T3b T4a

Stage IIC

T4b

NO NO NO NO NO NO NO NO NO

Stage Ill

AnyT

2:. N1

Stage IV

AnyT

Any N

Fig. 11.43: Acral lentiginous variety with early changes-never incise or cauterisesuchmelanomas

Fig. 11.44: The lesion resem bles squamous c ell carci noma. Punch biopsy reportec as amelanotic melanoma

Fig. 11.47: Malignant melanoma operated-recurrence after 8 months with satellite nodule. It is important that the first surgery should be the best surgery. Every attempt should be made to do a three-dimensional excision

MO MO

MO MO MO MO MO MO MO MO M1

*Clinical staging includes microstaging of the primary melanoma and clinical/radiologic evaluation for metastases. By convention, it should be used after complete excision of the primary melanoma with clinical assessment for regional and distant metastases

Fig. 11.48: Whole leg is studded with tumour. In-transit deposits from malignant melanoma of the foot

Skin Tumours

Important changes in the new AJCC staging of malignant melanoma I. Thickness and ulceration in T-category rather than level mvas1on 2. Number of metastatic nodes is more important 3. LDH is included in 'M' category (metastatic spread) 4. If there is ulceration, 3 stages are upgraded. 5. Satellite nodules and in-transit deposits are grouped into stage III disease. Differential diagnosis Pigmented basal cell carcinoma Histiocytoma (sclerosing angioma) • Naevus, Kaposi's sarcoma, cavernous haemangioma. Spread 1. Local spread occurs mainly by continuity and contiguity. Satellite nodules are due to local and lymphatic spread, situated within 5 cm of the primary lesion. Malignant melanoma rarely infiltrates the deep fascia unless and until a 'blunder biopsy' is done. Inadequate local excision can result in local recurrence later. Hence, initial surgery should attempt at cure. 2. Lymphatic spread is the principal mode of spread. Regional nodes get involved very early in malignant mela­ noma, altering the prognosis. Thus, nodes can get enlarged to a large extent even when the lesion looks innocent. Spread occurs both by permeation and embolisation. Permeation produces satellite nodules and in-transit nodules which develop between primary and secondaries. Embolisation occurs rapidly and early producing massive regional nodes (Key Boxes 11.13 and 11.14).

............................. . KEY BOX 11.13

MALIGNANT MELANOMA SOLE Inguinal nodes

I

Iliac nodes

I

Para-aortic nodes

I

Mediastinal nodes

I

Supraclavicular nodes

161

............................. . KEY BOX 11.14

LYMPH NODE METASTASIS IN MALIGNANT MELANOMA

• It is the single-most important prognostic index and it presents stage Ill disease. • Number of nodes is more important than size of nodes. • Poor survival in ulcerated malignant melanoma, even if node-negative. • Extranodal extension has poor prognosis. • Lymph node metastasis proceeds as an orderly process. Thus, evolved the concept of the first node to get involved-sentinel node (SN). • Elective lymphadenectomy is not indicated in thin melanoma (1 mm) • In thick melanoma, do a sentinel node biopsy. If positive, proceed to complete lymphadenectomy (block dissection).

In-transit metastasis appear in the skin as intracutaneous metastasis. They are thought to be due to melanoma cells trapped in lymphatic vessels. 3. Blood spread occurs relatively early and it causes secondaries in liver, lung, brain and bones producing miserable, pathetic situations. They are summarised in Table 11.5. See the interesting clinical notes.

.

...............

CLINICAL NOTES A 55-year-old lady presented to the hospital with enlarged inguinal lymph nodes. Clinical examination of the leg revealed nodules in the leg withfi r m to hard enlarged nodes in the inguinal region. On careful observation, the little toe was missing. On questioning, the patient admitted that the toe had been amputated elsewhere five years back for a painless blackish lesion (Figs 11.45 and 11.46).

Investigations • There is no specific investigation except an excision biopsy of the lesion. Excision with 1 cm of the margin is all that is required. Incisional biopsy is avoided because of the following reasons:

Spread of malignant melanoma Spread Metastasis in Metastasis in Metastasis in Metastasis in Metastasis in

lung liver brain bone bowel

Diagnosis

Problems

Cannonball appearance, pleural effusion Massive hepatomegaly, ascites Raised intracranial tension Bony pains, pathological fractures Bleeding

Respiratory failure Abdominal discomfort Coma Paraplegia, quadriplegia Anaemia

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• •

Manipal Manual of Surgery

- It may injure the deep fascia and it may open up a new plane of spread. - It does not allow the pathologist to perform a detailed histological examination (done in large lesions). Nonspecific investigations to look for metastasis are: - Chest X-ray-cannonball secondary - CT scan is better, however, it is costly (Fig. 11.49) - Ultrasound abdomen-secondaries in liver - X-ray of involved bone-osteolytic lesions FNAC of the regional lymph nodes - Fine needle aspiration of groin nodes is helpful in detecting the spread and to stage the disease (Figs 11.50 and 11.51). A large ulcerated lesion can be subjected to histopathology by punch biopsy or even incision biopsy. Serum LOH levels increase indicate metastatic disease.

Fig. 11.51: FNAC of the recurrent inguinal node secondaries­ bluish black aspirate

• HHB-4S-premelanosomal protein is specific immuno­ histochemical marker for melanoma. Treatment PEARLS OF

WISDOM

Surgery is the main modality of treatment available for malignant melanoma. All other modalities of treatment are only palliative and supportive.

Fig. 11.49: Lung metastasis in CT of the chest. It was not visible in X-ray chest

Fig. 11.50: Hugely enlarged inguinal lymph nodes-the nodes can be firm to hard. Sometimes, they are soft due to degeneration

Types of surgery possible are as follows 1. Excision biopsy-wide excision: A small lesion of 1 mm can be excised even under local anaesthesia with I cm of healthy margin around (narrow excision). Defect can be closed by primary suturing. While excising the tumour, it is better not to handle the tumour. It is possible to remove the tumour by strictly adhering to the principle of 'No touch' technique (Figs J 1.52and 11.53). Wide excision is based on principle­ centrifugal spread of melanoma. Tumour thickness (mm) Wide excision margin < l mm 1 cm 2cm 1-2mm >2mm 2cm Desmoplastic melanoma: Has high rate of recurrence, hence wide margin of excision is required. 2. Clarke's level II lesions are managed by a wider excision along with 2cm of healthy margin around. Resulting defect is closed by split skin grafting (Fig. J 1.54). 3. Subungual malignant melanoma is treated by amputation of the digit. 4. Malignant melanoma of the choroid has good prognosis. Choroid does not have lymphatic drainage. However, metastasis can occur through haematogenous route even after many years. [t is treated by enucleation of the eye. 5. Amputation (advanced and large lesions) (Fig. 11.55).

Skin Tumours

Fig. 11.52: Ulcerated melanoma over the heel-poor prognosis

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Fig. 11.53: Wide excision (minimum 2 cm) specimen

Fig. 11.54: Local recurrence after 2 years probably because of inadequate local excision-you can see the previous skin graft

Fig. 11.55: 8 cm ulcerated pigmented lesion in the foot-malignant melanoma foot required amputation

Fig. 11.56: Lazy S incision is given for inguinal block dissection­ this incision decreases incidence of flap necrosis

Management of lymph nodes I. If lymph nodes are situated adjacent to the primary lesion, block dissection is done along with primary lesion in continuity so as to include 'in-transit' deposits also. 2. If lymph nodes are away, radical block dissection is done. Example: For a lower limb malignant melanoma, inguinal nodes along with iliac nodes are removed. This is called Ilioinguinal block dissection. If these group of nodes are positive at frozen section, lymph node clearance should include lymph nodes of obturator vessels. This is called Ilio-obturator block dissection (Figs 11.56 and 11.57).

Fig. 11.57: Observe pigmented lymph nodes

• Sentinel lymph node m apping: lsosulfan blue is injected intradermally and the node that gets stained is identified and sent for frozen section (Haematoxylin and Eosin stains and immunohistochemical technique). If the node is positive, regional lymphadenectomy is done even when nodes are clinically not palpable. At least

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20-30% of the patients who are in Stage I will be identified as Stage Ill after sentinel node biopsy. There is a definite survival advantage in patients who undergo sentinel node biopsy (SNB) and regional lymphadenectomy when the nodes are not palpable. • If SNS is negative, prophylactic block dissection is indicated only if melanoma has poor prognostic histological factors. TLND-Therapeutic lymph node dissection is done in patients with known lymph nodal metastasis. ELND-Elective or prophylactic lymph node dissection done in patients with no clinical lymph nodal metastasis is beneficial in patients with 1-4 mm thick melanoma - Melanoma without ulceration - Extremity melanoma - Patients younger than 6 years of age • Only 25% of patients slow occult metastasis of age 3. If lymph nodes are enlarged, hard and fixed, palliative radiotherapy is given. Management of advanced malignant melanoma The aim of treating this group of patients is only to afford a reasonable palliation. More than 50% of patients, who have metastasis in the regional nodes are dead by the end of one year. Modes of treatment 1. Radiotherapy • Patients who have been deemed inoperable due to severe medical co-morbidities. 5-year survival is 70% but local recurrence is unacceptablly high • Adjuvant radiation to regional lymph node basin for - multiple +ve lymph nodes - I or more metastatic lymph nodes larger than 3 cm - Extracapsular extension - Recurrent regional disease may benefit from adjuvant radiation. 2. Systemic chemotherapy-dacarbazine (DTIC-dmethyl triazenyl imidazole carboxamide) is the standard agent. The response rate is around 20-30%. Cisplatin, Vinblastine, Bleomycin are also used in combination. The addition of immunomodulators such as BCG or Levamisole to chemo­ therapy have been tried. • Temozolomide is an oral analogue of dacarbazine. It achieves significant CNS penetration. It may decrease incidence of cerebral metastasis. 3. Immunotherapy • Interferon alfa-2b (Key Box 11.15) • Patients with locally advanced, recurrent, nodal, in-transit (Fig. 11.58) or satellite disease should be considered for adjuvant high dose JFN alfa-2b.

Fig. 11.58: Melanoma in-transit deposits

.

............................ .

KEY BOX 11.15

• • • •

INTERFERON-a

It has stimulatory effect on natural killer cells (NK) It has anti-angiogenic activity It is toxic-weight loss and myelosuppression can occur IL2-can cause capillary leak syndrome-hypotension and renal failure.

• Monoclonal antibodies: These are directed against antigen, expressed on the surface of melanoma cells. These antibodies are similar to lgG3 activate the immune system. • Cutaneous nodules can be managed by surgery or CO2 laser excision. • lntralesional injection of BCG has caused regression of the cutaneous nodules in some patients. 4. Hormone treatment: Antioestrogens such as tamoxifen have been tried in the systemic disease with 15-20% response rate. 5. Isolated limb perfusion: This is tried when there are extensive in-transit deposits in the limb (Fig.11.59) or recurrent disease in the limb. Melphalan is the drug of choice. A tourniquet is applied first, femoral vein and artery are cannulated, the blood which comes out is passed through a pump and oxygenator, into which a high dose ofMelphalan or DTIC is given. Input temperature is kept at 41 °C. Therapy is aimed at controlling local disease in the limb and to give a better functional limb even in presence of metastasis. Complications of isolated limb perfusion • Deep vein thrombosis • Pulmonary emboli • Complications of anticoagulants • Damage to the vessels * See Key Box 11.16 for interesting mathematics

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Skin Tumours

............................. . KEY BOX 11.16

MATHEMATICS IN MALIGNANT MELANOMA • • • • • • • • •

1 mm: Thin melanoma 1-4: Intermediate 4 and above: Thick melanoma > 6 mm diameter of a mole: Suspect melanoma 5 cm within primary-satellite nodule 8 to 12%: Familial variety 20 times more common in whites 50 to 60%: Arise from benign naevus 70%: Superficial spreading melanoma Fig. 11.59: Dermatofibroma protuberans upper limb

Summary of skin tumours (Table 11.6) OTHER MALIGNANT SKIN TUMOURS

............................. . KEY BOX 11.17

Dermatofibrosarcoma protuberans

• •

This is a locally malignant tumour arising from the dermis. Common sites are-trunk, flexor region of limbs. It presents as nodular (bosselated) ulcerative lesion of many years duration. Regional lymph node involvement is uncommon. It is less aggressive. Hence, it is curable. Treatment is by local wide excision followed by primary closure or skin grafting.

Kaposi's angiosarcoma (Key Boxes 11 .17 and 11.18) Common in black population

DISEASES ASSOCIATED WITH KAPOSI'S SARCOMA • • • •

Diabetes mellitus Lymphoma Following renal transplantation Acute and chronic immunosuppression (HIV)

............................. . KEY BOX 11.18

TYPES OF KAPOSI'S SARCOMA • • • •

European African Transplant AIDS

Elderly males Young and children Due to immunosuppression Homosexuals

Summary of skin tumours Squamous cell carcinoma

Basal cell carcinoma

Malignant melanoma

Incidence Origin Aetiology Site Types Edge Jnduration Scab Pigmentation Spread

Common (20--30%) Prickle cell layer Chronic irritation Trunk, leg, hand, oral cavity Ulcerative or cauliflower Everted Maximum Never occurs Absent Mainly by lymphatics. Blood spread is rare and late

Most common (60--70%) Basal layer Ultraviolet rays, fair skin Tear cancer Nodular or ulcerative Rolled out and beaded Moderate Occurs Absent Does not spread by lymphatics Blood spread is very, very late Spreads by local spread. Hence the name, rodent ulcer

Less common (10-20%) Melanoblasts Ultraviolet rays, fair skin, pre-existing mole Head and neck, face, digits, palm and sole Nodular or ulcerative Irregular Minimum Never occurs Present in 90% of cases Mainly by lymphatics, also by blood spread, does not infiltrate like rodent ulcer

Cytokeratin

Expression

No

No

Manipal Manual of Surgery

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• • • •

This neoplasm arises from proliferating capillary vessels and perivascular connective tissue cells. Multiple, purplish nodules appear in the limb, which ulcerate and bleed. This is its characteristic feature. Regional lymph node involvement can occur Increasing incidence due to AIDS.

Differential diagnosis 1. Malignant melanoma 2. Soft tissue sarcoma 3. Multiple cutaneous metastasis 4. T cell lymphoma

•

Self-limiting benign neoplasm of viral origin (probably) Arises due to overgrowth of hair follicle and subsequen spontaneous regression is characteristic. It is painless swelling in the skin with central dark brow1 core. After initial rapid growth of2--4 weeks, spontaneou regression occurs in 24 hours. After separation of th, central core, lump diminishes in size leaving a dee1 indrawn scar. Usually single, face is the commonest site. Like sebaceous cyst, it presents as hemispherical swelling Treated by excision. Keratoacanthoma if associated with sebaceous carcinom: and visceral malignancy (colon cancer) constitutes Muir· Torre syndrome (Fig. 11.62).

• •

OTHER SKIN LESIONS They arise from sebaceous glands, sweat glands, hair follicles, etc. See Key Box 11. 19 for types of exocrine glands. • Few examples are syringoma, hidradenoma, trichoepithe­ lioma and sebaceous carcinoma. • They present as localised swelling treated by excision. • They have to be kept in mind as a differential diagnosis for malignant skin tumours. Details of a few skin lesions are given in this chapter.

...

KERATOACANTHOMA: Molluscum sebaceum, Molluscum pseudocarcinomatosum

•

TURBAN TUMOUR (Figs 11.60 and 11.61)

It is the blanket term used to describe a tumour occupying the whole of the scalp resembling a turban. • Most often used to describe multiple cylindromata They produce pink nodular masses

.......................... . ............................. .

KEV BOX 11.19

KEV BOX 11.20

TYPES OF EXOCRINE GLANDS

• Ho/ocrine: Entire cell dies or disintegrates to liberate secretion, e.g. sebaceous gland. • Apocrine: Only the luminal part of the cell disintegrates, cell regeneration takes place from the nucleus and basal portion, e.g. mammary gland. • Merocrine: Secretion is discharged without destruction of the cells. Most of the glands belong to this type.

Fig. 11.60: Turban tumour due to squamous cell carcinoma of scalp

• Very, very rare

TURBAN TUMOUR

Types

• • • •

Multiple cylindromata Multiple nodular basal cell carcinoma Hidradenomata Plexiform neurofibromatosa of scalp

Fig.11.61: Turban tumour (Courtesy: Dr

Sreejayan, Professor of Surgery, Calicut Medical College)

Fig. 11.62: Sebaceous carcinoma

Skin Tumours •

Diagnosis is confirmed by biopsy. For differential diagnosis see Key Box 11.20. Treatment includes excision and reconstruction by skin grafting or rotational flaps.

• • •

CORN It is a popular painful lesion in the plantar surface of the foot (sole of the foot). • It affects the plantar surface of toes and sole of the feet. Corn develops due to intermittent pressure over a limited area. • Basically it is a localised hyperkeratinisation of the skin with a hard central core. • It is a cone-shaped lesion with broad surface and narrow at deeper plane. • They are painful and very tender. Most of these are hard corns. Soft corn can occur in between the toes. Treatment • Diabetic patients have to be carefully explained the consequences of a 'mistreated' corn. Sensations and pulsation have to be checked in those patients. Symptomatic corns have to be excised. Excision of a good cone-shaped tissue is necessary for a permanent cure. Otherwise, recurrence can occur. WART •

A wart is a rough excrescence on the skin Papillomaviruses are responsible for this

167

They are pigmented, keratinised, irregular lesions Common in young adults Common sites: Fingers, feet, genitalia, beard area, etc. Veneral warts: They are also called papilloma acuminata. They can occur in the anal region, perineum and in the coronal sulcus of the penis. Some of the warts may regress spontaneously. Fulguration with diathermy is the treatment.

MERKEL CELL CARCINOMA

• • • • •

It is derived from neuroendocrine cells which function as touch receptors. Highly malignant tumour Elderly white males are affected. Sun-affected areas such as head and neck regions are involved probably due to ultraviolet rays. Surgery, radiation and chemotherapy have been tried. Histopathological report resembles metastatic oat cell carcinoma. WHAT IS NEW IN THIS CHAPTER?/RECENT ADVANCES

• Treatment of basal cell carcinoma has been discussed in more detail including CO2 laser excision. Recons­ truction with various flaps have been discussed, in more detail. • A few lines have been added for malignant melanoma including a-interferon. • A few pearls of wisdom have been added.

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MULTIPLE CHOICE QUESTIONS

1. Which of the following statements is false about basal cell carcinoma? A. It starts as a nonhealing ulcer of many months duration B. Outer canthus of the eye is the common site C. It does spread by lymphatics D. Haematogenous spread is almost unknown 2. Following are true for basal cell carcinoma except: A. It is the most common malignant tumour B. It cannot occur in the lips C. Arsenic used in skin ointments may increase the risk D. Morpheic variety grows very slowly 3. Characteristic feature of basal cell carcinoma is: A. Epithelial pearls B. Mitotic figures C. Orphan Annie nucleai D. Pallisading islands 4. Following are used to treat basal cell carcinoma except: A. Wide excision B. Mohs' micrographic surgery C. Radiation D. Radiofrequency ablation 5. Following are true for basal cell carcinoma except: A. Ultraviolet rays can predispose to this condition B. Mohs' micrographic surgery can minimise the recurrence C. CO2 laser can be used to treat deep lesions D. Gori in's syndrome is associated with multiple basal cell carcinoma 6. Following skin malignancies can spread by lymphatic spread except: A. Epithelioma B. Malignant melanoma C. Sebaceous carcinoma D. Basal cell carcinoma 7. Characteristic feature of epithelioma is: A. Penetrating lesion 8. Elevated beaded edge C. Proliferative growth with everted edges D. Nodular lesion 8. Following are true for epithelioma except: A. Majority are well differentiated B. Cell nest is characteristic C. Broder's classification is used D. Polyhedral cells

9. Following are true for Marjolin's ulcer except: A. It arises from scar tissues 8. It does not spread by lymphatics C. It is rapidly growing D. It is painless 10. Following are true about prognostic factors fo epithelioma except: A. Metastasis is unlikely if the lesion is more tha 10mm 8. Broder's high grade means poor prognosis C. Recurrence is more in lip D. Perineural involvement carries poor prognosis 11. About melanocytes which one of the following is false· A. They are derived from neural crest 8. They convert DOPA into melanin C. Melanocyte stimulating hormone is released fron intermediate lobe of pituitary gland D. Present in the stratum basale layer of the epidermis 12. Following are true for malignant melanoma except: A. Spindle cell naevus has high malignant potential B. Junctional naevus has very high malignant potential C. Superficial spreading variety is the most common fom D. Tyrosinase is absent in albinos 13. Following are true for thin melanomas except: A. Less than I mm thickness 8. Metastasis is about 10-15 % C. 20year survival is almost 95% D. Local wide excision of 2 cm margin is recommended 14. Following are true for acral lentigenous except: A. Least common subtype B. Occur in palm and soles C. Hutchinson's sign may be found D. Rarely found in Blacks 15. Following are true for superficial spreading typf malignant melanoma except: A. Most common subtype 8. Trunk is the common site C. Average age of presentation is 5th decade D. lt has a long vertical growth 16. Which one of the following treatment is recommended in 2 cm melanoma leg with inguinal nodes? A. Wide excision with inguinal block dissection B. Wide excision with ilioinguinal block dissection C. Wide excision with ilioinguinal obturator dissection D. Amputation and inguinal block dissection

Skin Tumours

17. Which one of the following drugs used to decrease cerebral metastasis in malignant melanoma? A. Adriamycin 8. Cisplatin C. Temozolamide D. Alpha-interferon 18. Alpha-interferon has following actions except: A. lt has stimulatory effect on natural killer cells B. It has anti-angiogenic activity C. Does not cause myelosuppression D. Can cause capillary leak syndrome

169

19. Following are true for Merkel cell tumour except: A. It is a highly malignant tumour B. Derived from neuroendocrine tumours C. Fingers and digits are the common sites D. Head and neck are commonly involved 20. Following are true for Kaposi's angiosarcoma except: A. Common in Black population 8. Paclitaxel is the drug of choice with a 75% effective rate C. Homosexuals are affected more as a result of AIDS D. Classic form affects head and neck more than legs

ANSWERS C

2 D

3 D

4 D

5 C

6 D

7 C

11 C

12 B

13 D

14 D

15 D

16 B

17 C

8

D

9 C

10 A

18 C

19 C

20 D

12

.,

Haemorrhage, Shock and Blood Transfusion • Haemorrhage - Classification - Pathophysiology - Management • Shock - Classification - Pathophysiology - Management

• • • • • • • •

Acute adrenal insufficiency Hyperbaric oxygen Central venous pressure Pulmonary capillary wedge pressure Blood transfusion Blood products Plasma substitutes Bleeding disorders

HAEMORRHAGE

B. Chronic haemorrhage: Occurs over a period of time, e.g. haemorrhoids/piles or chronic duodenal ulcer, tuberculous ulcer of the ileum, diverticular disease of the colon.

I. Depending on nature of the vessel involved A. Arterial haemorrhage: Bright red in colour, jets out. Pulsation of the artery can be seen. It can be easily controlled, as it is visible. B. Venous haemorrhage: Dark red in colour. It never jets out but oozes out. It is nonpulsatile and difficult to control because vein gets retracted. C. Capillary haemorrhage: Red in colour, never jets out, slowly oozes out. It becomes significant if there are bleeding tendencies.

IV. Depending on the nature of bleeding A. External haemorrhage/revealed haemorrhage, e.g. epistaxis, haematemesis. B. Internal haemorrhage/concealed haemorrhage, e.g. splenic rupture following injury, ruptured ectopic gestation, liver laceration following injury.

Classification

Pathophysiology of haemorrhagic shock A loss of more than 30-40% blood volume results in a fall in blood pressure and gross hypoperfusion of the tissues leading

II. Depending on the timing of haemorrhage A. Primary haemorrhage: Occurs at the time of surgery B. Reactionary haemorrhage: Occurs after 6-12 hours of surgery. Hypertension in the postoperative period, violent sneezing, coughing or retching, are the usual causes, e.g. superior thyroid artery can bleed after thyroidectomy, if the ligature slips. Hence, it is better to ligate it twice. C. Secondary haemorrhage: Occurs after 5-7 days of surgery. It is due to infection which eats away the suture material, causing sloughing of vessel wall, e.g. bleeding after 5-7 days of surgery for haemorrhoids. Ill. Depending on the duration of haemorrhage A. Acute haemorrhage: Occurs suddenly, e.g. oesophageal variceal bleeding due to portal hypertension.

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A patient who underwent subtotal thyroidectomy for toxic goitre was shifted to the postoperative intensive care unit. Within 10 minutes, the nurse came to inform the surgeon that 450 ml o_fblood was collected in the "Redivac "suction bottle. The dressing was opened and there was no large collection of blood in the surgical wound. The presence of a large haematoma requiring drainage was thus ruled out. The blood pressure (BP) which was previously under control had shot up to 210/110 mmHg postoperatively, possibly due to pain. Careful monitoring and treatment reduced the BP to 1 40/90 mmHg. After 24 hours, the drainage was only JOO ml. The incision did not need re-exploration.. This case illustrates reactionary haemorrhage due to hypertension.

Haemorrhage, Shock and Blood Transfusion

to haemorrhagic shock. The evolution of haemorrhagic shock can be classified into four stages. Class I • When blood loss is less than 750 ml ( < 15% of blood volume), it can be called mild haemorrhage. • 60-70% of blood volume is present in the low-pressure venous system (capacitance vessels). Ten per cent of the blood volume is present in the splanchnic circulation. • When there is blood loss, peripheral venoconstriction takes place and compensates for the loss of blood volume by shifting some blood into the central circulation. Some amount of correction of blood volume also occurs due to withdrawal of fluid from the interstitial spaces. • Apart from a mild tachycardia and thirst, there may be no other symptom or sign suggesting hypovolaemia. The blood pressure, urine output and mentation are all normal m Class I shock. Class II • Loss of800-1500 ml (15-30% of blood volume) results in moderate (Class II) shock. • Peripheral venoconstriction may not be sufficient to main­ tain the circulation. Hence, adrenaline and noradrenaline (catecholamines) released from the sympathoadrenal system cause powerful vasoconstriction of both arteries and veins. • Increased secretion of ADH causes retention of water and salt. Thirst increases. • Clinically, the patient shows a heart rate of 100-120 beats/ minute and an elevated diastolic pressure. The systolic pressure may remain normal. Urine output is reduced to about 0.5 ml/kg/h and the capillary refill is more than the normal 2 seconds. Extremities may look pale and the patient is confused and thirsty. Class Ill • Loss of 1500-2000 ml (30-40% of blood volume) produces Class III shock. All the signs and symptoms of Class TI haemorrhage get worse. • The patient's systolic and diastolic blood pressures fall and the heart rate increases to around 120 beats/minute. The pulse is thready. • The respiratory rate increases to more than 20/minute. Urine output drops to 10 to 20 ml/hour. The patient appears pale and is aggressive, drowsy or confused. Class IV • A blood loss of more than 2000 ml(> 40% of blood volume) results in Class IV shock. The peripheries are cold and ashen. • The pulse is thready and more than 120/minute. The blood pressures are very low or unrecordable.

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• The patient may be moribund. • If persistent, can damage other organs, e.g. GIT: Mucosa) ulcerations, upper GI bleeding, absorption of bacteria and toxins, bacterial translocation and bacteraemia Liver: Reduced clearance of toxins Kidney: Acute renal failure Heart: Myocardial ischaemia, depression Lungs: Loss of surfactant, increased alveolocapillary permeability, interstitial oedema, increased arteriovenous shunting results in acute lung injury (ALI). Multiorgan failure consequent to haemorrhagic shock is associated with a high mortality rate. Early diagnosis of bleeding and appropriate management is crucial to improve survival and outcome. Management of haemorrhagic shock I. Treatment-general measures • Hospitalisation • Care of all critically ill patients begins with A, B and C. A: Airway, B: Breathing, and C: Circulation. • Oxygen should be administered by face mask to all patients who are in shock but are conscious and are able to maintain their airway. • If unconscious, endotracheal intubation and ventilation with oxygen may be necessary. • Haemorrhage control • Intravenous access: Urgent intravenous administration of Ringer lactate to restore blood volume to normal. If there has been massive blood loss as in Class IV shock or the patient is anaemic, blood transfusion is indicated. Colloids such as gelofusine or 5% albumin may also be used. The use of hetastarch may be associated with increased rate of acute kidney injury and mortality, and hence better avoided. • Investigations: Blood is collected at the earliest opportunity for routine investigations as well as for blood grouping and cross-matching. • Cross-matched blood is usually given: lf the haemorrhage is life-threatening, uncross-matched, 0 -ve packed cells may be transfused into the patient. • Use of inotropes and vasoconstrictors is not indicated as they may harm tissue perfusion. • However, if inotropes have been started as a life-saving measure, an attempt should be made to wean them as soon as the volume status is corrected and the patient is stable. II. Treatment-specific measures I. Pressure and packing • To control bleeding from nose and scalp: Packing using roller gauze with or without adrenaline to control bleeding from nose.

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• Bleeding from vein: Middle thyroid vein during thyroidec­ tomy, lumbar veins during lumbar sympathectomy can be controlled using pressure pack for a few minutes. • Sengstaken tube is used to control bleeding from oesophageal varices-intemal tamponade.

• Surgical procedure: Splenectomy for splenic ruptun hysterectomy for uncontrollable postpartum haemorrhagf laparotomy for control of bleeding from ruptured ectopi pregnancy.

2. Position and rest • Elevation of the leg controls bleeding from varicose veins • Elevation of the head-end reduces venous bleeding in thyroidectomy-anti-Trendelenburg position. • Sedation to relieve anxiety-Midazolam in titrated doses of 1-2 mg intravenously may be given.

SHOCK

3. Tourniquets Indications • Reduction of fractures • Repair of tendons • Repair of nerves When a bloodless field is desired during surgery Contraindications Patient with peripheral vascular disease. (The arterial disease may be aggravated due to thrombosis resulting in gangrene.) Types • Pneumatic cuffs with pressure gauge • Rubber bandage Precautions • Too loose a tourniquet does not serve the purpose. • Too tight: Arterial thrombosis can occur which may result in gangrene. • Too long (duration of application): Gangrene of the limb. Hence, when a tourniquet is applied, the time of inflation should be noted down and at the end of 45 minutes to an hour, it has to be deflated at least for 10 minutes and reinflated only if necessary. Complications • Ischaemia and gangrene • Tourniquet nerve palsy 1 4. Surgical methods to control haemorrhage • Application of artery forceps (Spencer Well 's forceps) to control bleeding from veins, arteries and capillaries. • Application of ligatures for bleeding vessels. • Cauterisation (diathenny). • Application of bone wax (Horsley's wax which is bee's wax in almond oil) to control bleeding from cut edges of bones. • Silver clips are used to control bleeding from cerebral vessels (Cushing's clip).

Definition Shock is defined as an acute clinical syndrome characterisec by hypoperfusion and severe dysfunction of vital organs There is a failure of the circulatory system to supply blood ii sufficient quantities or under sufficient pressure necessa1; for the optimal function of organs vital to survival. Classification • Hypovolaemic shock • Cardiogenic shock • Distributive shock • Obstructive shock HYPOVOLAEMIC SHOCK • Loss of blood-haemon-hagic shock • Loss of plasma-as in bums shock • Loss of fluid---dehydration as in gastroenteritis Features (Key Box 12.1) The primary problem is a decrease in preload. The decreased preload causes a decrease in stroke volume. Clinical features depend on the degree of hypovolaemia. Severe (Class III or IV) shock results in tachycardia, low blood pressures and decreased urine output. The peripheries are cold and the patient may be confused or moribund (see pathophysiology of haemon-hagic shock). Treatment • The primary goal is to restore the blood volume, tissue perfusion and oxygenation to normal as early as possible. • Replace the lost blood volume.

............................. . KEY BOX 12.1

HYPOVOLAEMIC SHOCK

Decreased preload Decrease in stroke volume Hypotension and hypoperfusion

1 In MS examination, a candidate was asked to examine a case of radial nerve palsy. The patient had an injury to the wrist 4 months back. The cut flexor tendons had been sutured. The candidate could not correlate the radial nerve palsy to the injury at the wrist. He failed! It was a case of tourniquet palsy.

Haemorrhage, Shock and Blood Transfusion

• Crystalloids: If crystalloids are used to replace blood loss, 2-3 times the volume lost needs to be given. Ringer lactate is the crystalloid of choice. Large volumes of saline infusion can cause hyperchloraemic metabolic acidosis. 5% dextrose is not used to expand the intravascular volume as it is hypotonic once the dextrose is metabolised. • Colloids: When colloids are used to replace lost blood volume, a volume equal to the lost volume may be given. • Crystalloids are preferred in the initial phase of resuscitation. If large volume of blood is lost, colloids such as gelofusine or 5% albumin may be given in order to reduce the amount of volume to be infused. • Blood transfusion may be needed if large amount of blood is lost(> 40% of blood volume) or if the patient is anaemic (Hb < 8 g%). CARDIOGENIC SHOCK The blood flow is reduced because of an intrinsic problem in the heait muscle or its valves. A massive myocardial infarction may damage the cardiac muscle so that there is not much healthy muscle to pump blood effectively. Any damage to the valves, especially acute may also reduce the forward cardiac output resulting in cardiogenic shock. Features • The primary problem is a decrease in contractility of the heart. The decreased contractility causes a decrease in stroke volume. • Left ventricular pressures rise as forward cardiac output reduces. The sympathetic nervous system is activated and consequently, systemic vascular resistance increases. • Clinically, the patient presents with tachycardia, low blood pressures and decreased urine output. • The jugular venous pulse may be raised, a S3 or S4 gallop may be present. • The lung fields may show bilateral extensive crepitations due to pulmonary oedema. • The peripheries are cold and the patient may be confused or moribund. Treatment • The primary goal is to improve cardiac muscle function. • Oxygenation can be improved by administering oxygen, either by face mask or by endotracheal intubation and ventilation as necessary. • Inotropes improve cardiac muscle contractility. • Vasodilators such as nitroglycerine may dilate the coronary arteries and peripheral vessels to improve tissue perfusion. Lowering systemic vascular resistance reduces impedance to forward cardiac output(afterload). However, the patient must be monitored closely to avoid excessive drops in blood pressure due to these drugs.

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• Intra-aortic balloon pump or ventricular assist devices may be used to augment cardiac output. • If hypotension continues to be unresponsive, revas­ cularisation (surgical or interventional) or valve replace­ ments may be considered on an emergency basis. DISTRIBUTIVE SHOCK In distributive shock, the afterload is excessively reduced affecting circulation. Distributive shock can occur in the following situations: • Septic shock • Anaphylactic shock • Neurogenic shock • Acute adrenal insufficiency SEPTIC SHOCK Pathophysiology • Sepsis is the response of the host to bacteraemia/ endotoxaemia. • It may be produced by gram-negative or gram-positive bacteria, viruses, fungi or even protozoa! infections. • Severe sepsis can result in persistent hypotension despite adequate fluid resuscitation and is called septic shock. • Local inflammation and substances elaborated from organisms, especially endotoxin, activate neutrophils, monocytes and tissue macrophages. This results in a cascade of proinflammatory and anti-inflammatory cytokines and other mediators such as IL-1, IL-8, IL-10, tumour necrosis factor-alpha, prostaglandin E 1, endogenous corticosteroids and catecholamines. • Effects of this complex mediator cascade include cellular chemotaxis, endothelial injury and activation of the coagulation cascade (Key Box 12.2). Features • These substances produce low systemic vascu lar resistance (peripheral vasodilatation) and ventricular dysfunction resulting in persistent hypotension. • Generalised tissue hypoperfusion may persist despite adequate fluid resuscitation and improvement in cardiac

. • • • •

............................ .

KEY BOX 12.2

DISTURBANCE OF PROCOAGULANT­ ANTICOAGULANT BALANCE Inflammatory response in sepsis activates tissue factor which in turn activates coagulation Fibrinogen is converted to fibrin Lowered levels of natural anticoagulants such as protein C, protein S and antithrombin Ill Procoagulant-anticoagulant imbalance � diffuse micro­ vascular thrombi

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output and blood pressures. This is due to abnormalities in regional and microcirculatory blood flow. These abnormalities may lead to cellular dysfunction, lactic acidosis (anaerobic metabolism) and ultimately, multi­ organ failure. • Early phases of septic shock may produce evidence of volume depletion such as dry mucous membranes and cool, clammy skin. • After resuscitation with fluids, however, the clinical picture is typically more consistent with hyperdynamic shock. This includes tachycardia, bounding pulses with a widened pulse pressure, a hyperdynamic precordium on palpation and warm extremities. • Signs of possible infection include fever, localised erythema or tenderness, consolidation on chest examination, abdominal tenderness, guarding, rigidity and meningismus. • Signs of end-organ hypoperfusion include tachypnoea, cyanosis, mottling of the skin, digital ischaemia, oliguria, abdominal tenderness and altered mental status. • Often, a definitive diagnosis cannot be made on the basis of initial findings, on history taking and physical examination and empirical treatment for several possible conditions commences simultaneously. Treatment • Removal of the septic focus is an essential step and a priority in the treatment of septic shock, e.g. resection of gangrenous bowels, closure of perforation, appendicectomy. • Early (preferably given in the first hour of arrival) empirical antibiotic therapy to treat the precipitating infection. • Supportive care: Oxygenation and if necessary, endotracheal intubation and mechanical ventilation should be administered. • Intravenous fluids: Restoration of intravascular filling pressures must be done using crystalloids, colloids and blood as necessary. CrystalJoids such as isotonic saline or Ringer's lactate may be used. Large amounts may be required and may contribute to tissue oedema. Colloids restore intravascular volume faster and remain longer in the central circulation. However, they are expensive and are more often used in patients where there is a high-risk of pulmonary oedema due to cardiac dysfunction and thus, may not tolerate large volume of fluids. Avoid hetastarch as it may increase risk of renal injury and mortality. Blood transfusions may be required to maintain the patient's haemoglobin levels at 8-10 g%. • Vasoactive agents such as norepinephrine to produce vasoconstriction and raise the systemic vascular resistance to nonnal may be used. Dopamine, dobutamine or adrenaline may need to be added. Vasopressin infusion may be useful in patients with refractory shock. All these potent drugs are given as infusions under careful and continuous monitoring

of blood pressure as well as cardiac filling pressures (central venous pressures). PEARLS

OF

WISDOM

No matter what antibiotics you use, unless you remove the septic focus by surgical drainage of pus or resection of gangrene, etc. patients in septic shock do not improve.

Summary of septic shock (Key Box 12.3)

............................. . KEY BOX 12.3

SEPTIC SHOCK Early diagnosis of septic shock Empirical antibiotics initially Appropriate antibiotics after culture and sensitivity testing Ul trasonography, CT scan, and chest X-ray are key investigations • Treatment of source of infection - Pneumonia - Drainage of pus Closure of perforation - Resection of gangrene • Early and aggressive resuscitation, supportive care and close monitoring in intensive care unit (ICU). • • • •

ANAPHYLACTIC SHOCK Features Occurs on exposure to an allergen the patient is sensitive to. It may be pollen, foodstuffs, preservatives in the food or a medication. The anaphylactic shock that occurs in the hospital is usually due to some drug, e.g. the patient is allergic to penicillin. Latex allergy is being increasingly recognised. The reaction may be in the form of mild rashes with or without bronchospasm or it may be a full blown anaphylactic shock wherein the patient presents with rashes, generalised oedema including laryngeal oedema, bronchospasm and hypotension and if not treated in time, cardiac arrest. Treatment I. Primary • Oxygen and if necessary, endotracheal intubation and ventilation. • Adrenaline, 0.5-1 mg IM or 50-100 µg intravenous boluses as necessary to maintain blood pressure. • Intravenous fluids-isotonic saline or Ringer lactate • Leg end elevation of bed. II. • • •

Secondary Chlorpheniramine maleate Hydrocortisone 100 mg intravenously If facilities exist, take a 10 ml sample of blood to analyse for serum tryptase levels. If raised, they confirm anaphylactic reaction.

Haemorrhage, Shock and Blood Transfusion

NEUROGENIC SHOCK Causes: High spinal cord injury, vasovagal shock Features: Hypotension without tachycardia that can deteriorate to produce shock and cardiac arrest. Treatment: Intravenous fluids, inotropes and vagolytics as necessary. ACUTE ADRENAL INSUFFICIENCY Causes (Key Box 12.4) Adrenal crisis occurs if the adrenal gland is deteriorating as m: • Primary adrenal insufficiency (Addison's disease) • Secondary adrenal insufficiency (pituitary gland injury, compression) • Inadequately treated adrenal insufficiency. Features • Headache, profound weakness, fatigue, slow and lethargic movement, joint pain.

.

............................

KEY BOX 12.4

• • • •

.

RISK FACTORS FOR ADRENAL CRISIS Infection Trauma or surgery Adrenal gland or pituitary gland injury Premature termination of treatment with steroids such as prednisolone or hydrocortisone.

• Nausea, vomiting, abdominal pain, high fever and chills • Low blood pressure, dehydration, rapid heart rate and respiratory rate, confusion or coma. Treatment • Care of airway, breathing and circulation • Intravenous fluids • Hydrocortisone I 00-300 mg intravenously • Treat the precipitating factor • Antibiotics as necessary OBSTRUCTIVE SHOCK It can be due to cardiac tamponade or due to tension pneumothorax. A. CARDIAC TAMPONADE Features • In obstructive shock, there is impedance to either inflow or outflow of blood into or out of the heart.

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A 54-year-old lady was admitted to the casualty with low blood pressures and dyspnoea since one day. She had a history of fever, vomiting and diarrhoea since 3-4 days, was treated in a local nursing home and when she got worse, was referred to our hospital. In spite of fluid therapy, profound hypotension persisted and within half an hour of arrival to the casualty, she suffered a cardiopulmonary arrest. Her trachea was immediately intubated, cardio­ pulmonary resuscitation (CPR) given and was shifted to the intensive care unit for further management. She required high doses of dopamine, adrenaline and noradrenaline to maintain blood pressures. A blood gas analysis showed severe metabolic acidosis (pH= 7.02, PaC02 = 35 mmHg and HC03- = 12 mmol/L).

Considering the history, a diagnosis of septic shock was made when she continued to have hypotension even after her central venous pressures were normal. Peritoneal dialysis was done as she was in oliguric renal failure. Haemodialysis was not possible as she was hypotensive and on inotropes. A search for septic focus was initiated. An ultrasound abdomen showed dilated kidney and obstructed urinary system. A double J stenting of the ureter, which was done to relieve the obstruction, drained pus. Once the pus was drained, appropriate antibiotics were given and with continued cardiorespiratory support, she showed steady improvement. She was gradually weaned off the ventilator and inotropes, and was discharged from the hospital five weeks later. At discharge, she was fully conscious, stable, ambulant and very grateful to the medical fraternity. This case illustrates the importance of resuscitation, cardiorespiratory support, removal of septic focus and antibiotics in the treatment of septic shock.

• In cardiac tamponade, the pericardium is filled with blood and hampers venous filling as well as outflow. • The fi11ing pressures of the left-sided and right-sided chambers equalise. • The central venous pressure is high and the blood pressure is low. • The patients also have pulsus paradoxus where there is 10% decrease in systolic blood pressure with inspiration. Treatment • To maintain preload with fluids or blood as indicated. • Relief of obstruction, drain the pericardia! cavity as early as possible.

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B. TENSION PNEUMOTHORAX Causes • Injury to the Jung due to trauma • Ventilator-induced barotrauma • Rupture of emphysematous bulla in a patient with chronic obstructive pulmonary disease. Features • Profound cyanosis, distended neck veins • Tachypnoea, dyspnoea or respiratory arrest • No air entry on the side ofpneumothorax, hyper-resonance to percussion • Tachycardia, hypotension and cardiac arrest Treatment • A wide (large) bore needle/cannula must be inserted into the pleural cavity to drain the air. The needle is inserted in the midclavicular line in the 2nd intercostal space on the affected side. This is followed by insertion of a tube thoracostomy. PEARLS

OF

WISDOM

A massive pulmonary embolus is a differential diagnosis for obstructive shock.

HYPERBARIC OXYGEN Hyperbaric oxygen is administered to patients when their own haemoglobin is unable to carry adequate oxygen or when hyperoxia is required. Hyperbaric 02 is administered using either a monoplace (single patient) or multiplace compression chamber. Indications 1. Carbon monoxide poisoning: Hyperbaric 02 at 2-3 atm is given to increase the 02 dissolved in plasma (5-6 ml/dl at 3 atm, whereas it is 0.3 ml/di at 1 atm) which is sufficient to meet the 02 requirement of the body even in the absence of normal haemoglobin (severe CO poisoning). 2. Infections such as gas gangrene: Hyperoxia suppresses the growth of anaerobic organisms. 3. Cancer therapy to potentiate radiotherapy 4. Arterial insufficiency 5. Decompression sickness and air embolism: Hyperbaric oxygen reduces the size ofair bubbles and rapidly eliminates nitrogen from the air bubbles. Contraindications Untreated pneumothorax: Can expand with hyperbaric 02 resulting in life-threatening tension pneumothorax.

CENTRAL VENOUS PRESSURE (CVP) • One of the desirable requirements while treating patient� in shock includes monitoring of CVP. • CVP is a fair indicator of blood volume. Thus, in shock, measurement of CVP is useful so as to plan proper fluid management. • CVP is also affected by contractility of the right ventricle, intrathoracic pressure and intrapericardial pressure. Method • Internal jugular vein (IN) or subclavian vein are preferred to gain access to central veins (Key Box 12.5). • A 16 cm long IV catheter is introduced into the central vein with the patient supine, head down and neck rotated to the opposite side. Head down position helps in engorging the vein. Seldinger's technique is employed and the catheter is advanced up to the junction ofsuperior vena cava and right atrium. • The patency and position of the catheter is confirmed by lowering the saline bottle to check free flow of blood into the connecting tube. • The tube is connected to a saline manometer. Readings of the saline level are taken with the 'zero reference point' at the midaxillary level if the patient is in supine position or at the manubriosternal joint, ifhe is in the semireclining position (45°). An electronic pressure transducer may be used for greater accuracy. • CVP must be measured at end-expiration as it may change with phases of respiration. Uses 1. If CVP is low, venous return should be supplemented by IV infusion, as in cases of hypovolaemic shock. 2. When CVP is high, further infusion of fluids may result in pulmonary oedema. 3. In cardiogenic shock, CVP may be normal or high. Complications 1. Pneumothorax 2. Accidental carotid artery puncture 3. Haematoma in the neck 4. Bleeding 5. Air embolism 6. Infection.

............................. . KEY BOX 12.5

• • • •

ACCESS TO RIGHT HEART/GREAT VEINS Internal jugular vein Subclavian vein Median cubital vein External jugular vein

Haemorrhage, Shock and Blood Transfusion

PULMONARY CAPILLARY WEDGE PRESSURE (PCWP) • This is a better indicator of circulatory blood volume and left ventricular function. • It is measured by a pulmonary artery balloon floatation catheter-Swan-Ganz catheter. Uses of PCWP I. To differentiate between left and right ventricular failure 2. Pulmonary hypertension 3. Septic shock 4. Accurate administration of fluids, inotropic agents and vasodilators. Method of measuring PCWP Swan-Ganz catheter is introduced into the right atrium through a central vein (RIN usually). The catheter has a balloon near its tip which is inflated with air. The catheter is advanced and pressure tracing is monitored. Its entry into the right ventricle and pulmonary artery is identified by changes in pressure tracing. With further advancement, the pressure tracing becomes flat, when the balloon gets wedged in a small branch to give pulmonary capillary wedge pressure (PCWP). When the balloon is deflated, the pulmonary artery pressure is obtained. Normal values: PCWP: 8-12 mmHg. PAP: systolic 25 mmHg; PAP diastolic 10 mmHg. Complications • Arrhythmias • Pulmonary infarction • Pulmonary artery rupture Interpretation in various conditions: CVP and PCWP (Table 12.1) Note: CVP reflects right atrial pressures only. It is low in hypovolaemia and, high in hypervolaemia and in right ventricular failure. PCWP is a better indicator of left ventricular pressures and is a preferred monitor in cardiogenic shock. PCWP is low in hypovolaemia and high in hypervolaemia as well as in left ventricular failure.

Conditions

CVP

PCWP

Hypovolaemic shock Right heart failure Left heart failure Cardiac tamponade Pulmonary embolism

Low High Normal High Normal/ High

Low Normal High High High

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Note: The use of pulmonary artery catheter has declined significantly due to increased complications and unfavourable risk-benefit ratio. An echocardiogram is noninvasive, provides a lot more information and is preferred in the treatment of cardiogenic shock. BLOOD TRANSFUSION Administration of whole blood or its components into a patient is often necessary for various reasons. Since the only available source of blood is by voluntary human donation, and that source is scarce, whole blood is separated into its components, namely packed red blood cells, fresh frozen plasma, platelets and cryoprecipitate. Some coagulation factors may be isolated and separately stored, e.g. Factor VIII for administration into haemophiliacs. Packed red cells are the commonest blood products used. Their transfusion increases the oxygen carrying capacity of blood. Transfusion of250 ml of packed cells raises haemoglobin by 1 go/o in an adult. Indications of blood transfusion (Key Box 12.6) I. Packed red cells are used to replace acute and major blood loss as in: • Haemorrhagic shock • Major surgery-open heart surgery, gastrectomy • Extensive burns II. Packed red cells are also used to treat anaemia due to: • Extensive burns • Chronic blood loss as in haemorrhoids, bleeding disorders, chronic duodenal ulcer, etc. • Inadequate production as in malignancies, nutritional anaemia III. To replace platelets in thrombocytopaenia, fresh frozen plasma in vitamin K deficiency unresponsive to vitamin K replacement as in liver disease or to reverse effects of warfarin, cryoprecipitate to replace fibrinogen in patients with disseminated intravascular coagulation. IV. Whole fresh blood administration is described in massive trauma with heavy blood loss, the rationale being that the patient rapidly loses all components of blood and so all components need to be replaced. Thus, it makes sense to administer whole blood rather than individual components. However, many blood banks are reluctant to allow whole blood transfusion.

............................. . KEY BOX 12.6

GUIDELINES: WHEN TO TRANSFUSE 1. Blood loss > 20% of blood volume 2. Haemoglobin < 8 g/dl 3. Haemoglobin < 10 g/dl in patients with major cardiovascular disease (e.g. ischaemic heart disease)

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BLOOD PRODUCTS Packed red blood cells (PRBC): When whole blood is centrifuged, red blood cells settle down and the platelet rich plasma remains supernatant. The plasma is transferred to another bag while preservative is added to red cells and the bag is sealed. Each bag of packed cells contains approximately 250-300 ml with a red cell concentration of 70%. Red cell transfusion is required for patients whose haemoglobin is < 7 g%, as in patients undergoing chemotherapy, major surgery, delivery, trauma, grossly anaemic neonates and infants or in patients with sickle cell anaemia, especially in sickle cell crisis. A higher transfusion threshold (9-10 g%) may be used for patients with cardiac disease. Each unit of packed cells should increase haemoglobin by 1 g% and haematocrit by 3%. The red cells may be leucodepleted (white blood cells removed) for use in patients requiring multiple transfusions to prevent development of antibodies to leukocytes or in those who are known to react to leukocytes in the past. The rate of blood or blood product administration depends on the quantity and speed at which it is lost from the body. Slow transfusion is indicated in patients with cardiac disease, renal dysfunction, severe chronic anaemia and in paediatric patients. In semi-emergent situations, e.g. treatment of low haemoglobin in patients awaiting surgery or delivery, each bag of packed cells may be transfused slowly. The initial 25 ml can be given slowly to check whether the patient tolerates it. Thereafter, it can be transfused at a rate of 3-4 ml/kg/h (approximately 1.5 h for a bag of red cells). The red cells must be transfused within four hours of removing from refrigerator as bacterial growth may be promoted after that. In emergent situations, the red cells may be transfused much faster in order to keep up with the loss. When large amounts are transfused in a short period (see section on massive transfusion), effects of other components such as citrate become significant. Packed red blood cells must be both ABO and Rh compatible unless the patient has life-threatening massive bleeding, in which case O -ve packed cells may be transfused. Platelets: The bag containing platelet rich plasma is again centrifuged to express off the plasma so that the bag with the remaining platelets can be sealed off. Each unit (50 ml) should contain at least 5.5 x 10 10 platelets (platelet concentrate) and each unit should elevate the platelet count by 5-10,000 cells/ cu mm in a 70 kg person. Platelets may be transfused pro­ phylactically or for therapeutic purposes. Prophylactic platelet transfusion may be done when platelet count is< 10,000 cells/cu mm in oncology patients. In patients who are at high-risk of alloimmunisation, e.g. leukaemia, the threshold for platelet transfusion is even lower at 5000 cells/ cu mm, whereas in patients with clinical instability, the threshold may be raised to 20,000 cells/cu mm. Patients who need to undergo major surgery or requiring invasive procedures

such as spinal anaesthesia, liver biopsy, etc. generally neec their platelet count elevated to> 50,000 cells/cu mm. However patients requiring surgery in critical areas such as neurosurger; or ophthalmic surgery will need their platelet count raised t< > 1,00, 000 cells/cu mm. Therapeutic platelet transfusion is required in patients wh< are known to be thrombocytopaenic and are actively bleedin! (platelet count < 50,000 cells/cu mm). The dose of platelet: may be calculated as follows: One unit of platelets (60 ml) fo: every 10 kg body weight. Platelets must be transfused within four hours o commencement of the infusion. ABO compatibility is no required for platelets. Filters of 170-260 µ must be used tc transfuse platelets as with other blood components. Fresh frozen plasma (FFP): The remaining plasma (200230 ml) may be stored in a frozen form (called fresh frozer plasma) at - l 8° C for one year. It needs to be thawed over halJ hour before use. Fresh frozen plasma transfusion is indicatec' in patients with prolonged INR > 1.5 and are bleeding 01 require surgery. FFP provides coagulation factors to those patients who are actively bleeding and those on warfarin. Fresb frozen plasma is administered in a dose of 10-20 ml/kg body weight. FFPs must be ABO compatible. FFP must be transfused as soon as possible after thawing and definitely within 24 hours of thawing. A hanging unit must be transfused within six hours of commencement of transfusion due to risk of bacterial contamination. Cryoprecipitate: The fresh frozen plasma may be further treated to produce cryoprecipitate rich in fibrinogen. Trans­ fusion of cryoprecipitate (each bag contains 20 ml) is indicated in patients whose fibrinogen levels are < 1 g%, e.g. disseminated intravascular coagulation (DIC). The dose is 0.2 units/kg body weight but usually 10 units are transfused initially and repeated as necessary. Important points to remember before transfusion of blood products: • Always obtain informed consent from patients or immediate relatives (in emergent situations) prior to transfusion. • Recheck the patient's blood group and of the donor blood, preferably along with one other qualified person to ensure that the correct bag is being transfused to the patient. The patient's hospital number and the blood bag number should also be checked. Both people must sign on the transfusion report. • Visually check the blood bag for any obvious abnormalities such as very dark blood, visible clots and if present, do not transfuse but return to blood bank. Check the date of collection and date of expiry before transfusion. The storage shelf life of red blood cells depends on the preservative added. RBCs stored in ACD (acid-citrate-dextrose), CPD (citrate-phosphate-dextrose), CPDA (citrate-phosphate­ dextrose-adenine) and SAGM (saline-adenine-glucose­ mannitol) solutions have a shelf-life of 21, 28, 35 and 35 days respectively (Key Box 12.7).

Haemorrhage, Shock and Blood Transfusion

..

...........................

KEY BOX 12.7

.

STORAGE OF BLOOD Preservative ABC survival (days) 21 ACD (acid-citrate-dextrose) CPD (citrate-phosphate-dextrose) 28 CPDA (citrate-phosphate-dextrose-adenine) 35 SAGM (saline-adenine-glucose-mannitol) 35

• Watch for any transfusion reactions. Whole blood transfusion is not advisable for routine use and is also not available from blood banks. However, there is some evidence in the literature that fresh whole blood transfusion may be helpful in patients with massive trauma as all blood components are replaced simultaneously matching the loss. However, this is difficult practically as the blood products still need to be screened for various antigens and infections. Other blood products available are fibrinogen concentrate (high risk of hepatitis), Factor VIII and Factor IX concentrates (for use in haemophilia and Christmas disease respectively) and Factor VII concentrate (for use tn disseminated intravascular coagulation-DIC). COMPLICATIONS OF BLOOD TRANSFUSION I. Immune complications 1. a. • •

Haemolytic reactions Major (ABO) incompatibility reaction This is the result of mismatched blood transfusion. Majority of cases are due to technical errors like sampling, labelling, dispatching. • This causes intravascular haemolysis. Clinical features • Haematuria • Pain in the loins (bilateral) • Fever with chills and rigors • Oliguria is due to products of mismatched blood transfusion blocking the renal tubules. It results in acute renal tubular necrosis. Treatment • Stop the blood. Send it to blood bank and recheck. • Repeat coagulation profile • IV fluids, monitor urine output, check urine for Hb • Diuresis with furosernide20-40 mg IV or injection mannitol 20% 100 ml IV to flush the kidney. b. Minor incompatibility reaction • Occurs due to extravascular haemolysis • Usually mild, occurs at 2-21 days

179

• Occurs due to antibodies to minor antigens • Malaise, jaundice and fever • Treatment is supportive 2. Nonhaemolytic reactions a. • • •

Febrile reaction Occurs due to sensitisation to WBCs or platelets Increased temperature-no haemolysis Use of20-40 mm filter or leukocyte-depleted blood avoids it. b. Allergic reaction • Occurs due to allergy to plasma products; manifest as chills, rigors and rashes all over. • They subside with antihistaminics such as chlorpheniramine maleate 10 mg lV. c. Transfusion related acute lung injury (TRALI) It is a rare complication, occurring within six hours of a transfusion due to the presence of antileukocyte antibodies in the transfused plasma causing patient's white cells to aggregate in the pulmonary circulation. This leads to degranulation of leukocytes causing increased capillary permeability and noncardiogenic pulmonary oedema. The symptoms may vary from mild dyspnoea to full blown acute respiratory distress syndrome. Proper supportive therapy will see that it resolves within 24-48 hours. d. Congestive cardiac failure (CCF) CCF can occur if whole blood is transfused rapidly in patients with chronic anaemia. Treatment • Slow transfusion, injection furosemide 20 mg IV • Packed cell transfusion is the choice in these patients. II. Infectious complications Serum hepatitis, AIDS, malaria, syphilis are dangerous infectious diseases which can be transmitted from one patient to another through blood transfusion. The danger is increased in cases of multiple transfusions and in emergency situations. "Prevention is better than cure". Hence, it is mandatory to screen the blood for these diseases before transfusion. Ill. Complications of massive transfusion (Key Box 12.8) Massive blood transfusion Definition: Massive blood transfusion has been variously defined-replacement of> 1 blood volume (or> 10 units of packed cells) in 24 hours, half the patient's blood volume in six hours, > 4 RBC units in one hour with ongoing need for transfusion, 500 ml over 5 min or even blood loss> 150 ml/ min with haemodynamic instability and need for transfusion. Massive blood loss may occur with trauma, postpartum haemorrhage or during major surgeries.

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............................. . KEY BOX 12.8

1. Predeposit

MASSIVE BLOOD TRANSFUSION 1. > 500 ml over 5 minutes 2. > 1/2 the patient's blood volume in 6 hours 3. > the whole blood volume in 24 hours

Problems

• • • •

Types of autologous transfusion

Citrate toxicity-hypocalcaemia Thrombocytopaenia Clotting factors deficiency Acute lung injury

Massive transfusion protocol: In patients with massive bleeding and anticipated to require massive transfusion, the blood bank should be intimated to activate massive transfusion protocol (MTP). A blood sample must be sent for cross matching with an initial request for 4 units ofO -ve red blood cells. After this, ifMTP is requested, the blood bank releases blood products in different 'boxes' (Table 12.2). The blood products are obtained box by box as necessary. If the bleeding stops, the blood bank should be intimated immediately so that MTP can be terminated. If bleeding continues, box three and four are alternately requested for. In such patients regular and half hourly measurements of acid­ base status, haemoglobin, platelets, prothrombin time (PT), activated partial thromboplastin time (aPTT), fibrinogen and serum calcium are required with an aim to normalise their values. Disseminated intravascular coagulation (DIC)

• Occurs in massive blood transfusion wherein all factors of coagulation are used up resulting in a bleeding disorder. • It produces severe afibrinogenemia • It is treated by replacement with fibrinogen (cryoprecipitate) and other clotting factors.

• 2-5 units of blood may be donated over 2-3 weeks befot elective surgery. 2. Preoperative haemodilution • Cases such as surgery for thyrotoxicosis or abdominopelvi resection wherein one can expect 1-2 units of blood los: Just before surgery, 1-2 units of blood are removed an retransfused after the procedure. 3. Blood salvage Blood which was lost during surgery is collected, mixed wit anticoagulant solution, washed and reinfused. This can be don provided surgery does not involve severe infection, bowE resection or malignancy, revision total hip replacement. Advantage

All the risks involved with blood transfusion are avoided. Disadvantages

• May not be acceptable to the patient • Sophisticated equipment required Blood Product (Fig. 12.1) PLASMA SUBSTITUTES

These are colloidal solutions used to restore normal blooc volume in emergency situations, e.g. polytrauma with seven haemorrhage, massive GI bleed and shock. 1. Albumin

• • • •

It is a rich protein but carries no risk of hepatitis It is available as 5 and 20% Used in severe bums-acute severe hypoalbuminaemia Used in nephrotic syndrome

PEARLS

OF

WISDOM

Packed cells andfreshfrozen plasma transfusion should not be used to treat malnutrition.

AUTOLOGOUS TRANSFUSION

This concept originated to avoid transfusion reactions which can develop when homologous blood is used. • Here, patient's own blood is used.

2. Gelatins

• Good plasma expander • Plasma expansion lasts a few hours

Release of blood products on activation of MTP Box One Two Three Four

PRBC

FFP

2 4 4 4

2 4 4 4

Platelet 1 adult 1 adult

Cryoprecipitate

3

Haemorrhage, Shock and Blood Transfusion

I I

Whole blood

I

Platelet concentrate (pooled platelets) • Used in thrombocytopaenia

Packed red cells • Prepared by centrifugation of whole blood and removing plasma • Used to correct chronic anaemia • Economical and safe

181

I

I

Fresh-frozen plasma (FFP)

I

I

Cryoprecipitate Fibrinogen Factor VIII Factor IX Factor VII Albumin

Fig. 12.1: Blood products

• Severe reactions with urea-linked gelatin, e.g. Haemaccel (I :2,000) but less with succinylated gelatin, e.g. gelofusine (1:13,000) 3. Dextran 40 • Reduces viscosity and red cell sludging • May affect renal function and coagulation 4. Hydroxyethyl starch • Derived from starch • Plasma expansion lasts for over 24 hours • Maximum dose-20 ml/kg • Large doses may interfere with coagulation • Incidence of severe reactions (1: 16,000). • Use in shock is associated with increased risk of renal injury and mortality. BLEEDING DISORDERS HAEMOPHILIA This is the commonest bleeding disorder which occurs due to X-linked genetic disorder of coagulation. Types 1. Haemophilia A: This results from a reduction of factor VIII (antihaemophilic factor) and is carried by a recessive gene. 2. Haemophilia B: This results due to deficiency of factor IX.

HAEMOPHILIA A • Haemophilia "excessive bleeding, unusual bleeding, unexpected bleeding" is due to haemophilia 'A'. • A haemophilic patient's daughters will be carriers but all sons will be normal. • Thus, a carrier woman has 50% chance of producing haemophiliac male or a female carrier. • The level of coagulation factor VIII in the blood may be less than 1% of that in a normal individual. Clinical features "Excessive bleeding, unusual bleeding, unexpected bleeding" is due to haemophilia 'A' (Table 12.3). 1. Bleeding into joints (haemarthrosis) • Large joints such as knees, elbows, ankles, wrists are affected. • Spontaneous bleeding is common. It may also occur due to minor trauma. • Repeated bleeding may result in permanent damage to the articular surfaces resulting in deformity of the joints. 2. Bleeding into muscles • Calf muscle and psoas muscle haematomas are common resulting in contraction and fibrosis of muscle, muscle pain and weakness of limb. • Intramuscular injections should be avoided.

Blood changes in haemophilia A and von Willebrand's disease Haemophilia 'A'

von Willebrand's disease

Bleeding time Prothrombin time Vlll: C

Normal Normal

f Normal

vWF

Normal

HJ.

J. J.

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182

HAEMOPHILIA B (CHRISTMAS DISEASE)

Treatment • Administration of factor VIII concentrate by intravenous infusion is the treatment whenever there is bleeding. • It should be given twice daily since it has half-life of 12 hours. • Any major surgical procedure should be carried out only after raising factor VIII: C levels to I 00% preoperatively and maintained above 50% until healing occurs. • Synthetic vasopressin (DDAVP) produces a rise in factor VIII: C.

• Incidence: One in 30,000 males. The inheritance an, clinical features are identical to haemophilia A. • It is caused by deficiency of factor IX. • Treatment is with factor IX concentrates. VON WILLEBRAND'S DISEASE (vWD) • In vWD, there is defective platelet function as well as facto VIII: C deficiency and both are due to deficiency o abnormality of vWF. • Epistaxis, menorrhagia and bleeding following mino1 trauma or surgery is common. • Haemarthrosis is rare. • Treatment is similar to mild haemophilia. Thus, DDAVF or IV infusion of factor VIII: C or vWF surgery.

Causes of death • Cerebral haemorrhage used to be the most common cause of death. However, today HIV infection seems to be the most common cause of death. • Hepatocellular carcinoma, cirrhosis due to HIV and HCV also are the other causes (due to repeated blood transfusions).

A FEW PHOTOGRAPHS OF PATIENTS WITH SEPTIC SHOCK (Figs 12.2 to 12.7)

Fig. 12.2: Extensive gangrene

Fig. 12.3: Necrotising fasciitis of upper limb Fig. 12.4: Chest X-ray in septic shock

Fig. 12.6A: Intra-arterial blood pressure monitoring •· ··J;·';_.�;, ...,

,r��J... ��i/�

,: ··>�-"! .� ·"'::;" .. ·-· "

Fig. 12.5: Faecal peritonitis

:.


650 mOsm/kg). HYPERVOLAEMIA Causes 1. Excessive infusion of intravenous fluids 2. Retention of water in abnormal conditions such as cardiac, renal and hepatic failure 3. Absorption of irrigation fluid as during transurethral resection of prostate using distilled water. Diagnosis • History and physical examination can lead to the cause. • Physical examination: Distended neck veins, pedal oedema, body weight gain • Circulatory overload: - Hypertension, tachycardia, pulmonary oedema - Confusion, restlessness, convulsions and coma The development of these signs depends on the rate and volume of fluid overload, renal function and cardiovascular reserve. Management I . Treat the cause 2. Restriction of water and salt 3. Diuretics (or dialysis, if necessary) to remove excess water.

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REGULATION OF SODIUM CONCENTRATION Water constitutes the major component of all body fluids but the composition varies with the fluid compartment. The most abundant cation of extracellular fluid is sodium and is the prime determinant of ECF volume. Ninety per cent of the ECF osmolality is due to sodium. The human body has no known mechanism to regulate sodium intake. The body regulates sodium output by: • Regulating glomerular filtration rate • Regulating plasma aldosterone levels Addition or loss of water produces a change in the concentration of the solute. The quantity of solute relative to the volume of water is thereby increased (ECF is concentrated) or decreased (ECF is diluted) with loss or addition of water respectively. Changes in concentration are generally changes in water balance rather than changes in sodium regulation. Since the changes in volume and concentration are interdependent and the changes in water content are not easily

measured, an estimate of the fluid volume and concentration is usually made by using the measured sodium levels and serum osrnolality. DISTURBANCES IN CONCENTRATION HYPONATRAEMIA Hyponatraemia is defined as a sodium level less than 135 mmol/L. It may occur as a result of water retention, sodium loss, or both. True hyponatraemia is always associated with low plasma osmolality. It may be associated with expanded, contracted or a normal extracellular volume. Causes Assessment of hypovolaemia should begin with an estimation of the extracellular fluid volume (clinically and if necessary, using central venous catheters). Thus, true hyponatraemia can be of three types: hypervolaemic hyponatraemia, hypo­ volaemic hyponatraemia and normovolaemic hyponatraemia (Fig. 14.2).

Hyponatraemia

Normal or high 1---------i Pseudohyponatraemia

Evaluation of ECF volume

Normovolaemia

Hypervolaemia

Oedema Extrarenal loss

CCF, cirrhosis, nephrotic syndrome

Diuretics, salt losing kidney

Urinary Na· < 15 mmol/L

Urinary Na• > 20 mmol/L

SIADH, renal failure

Diarrhoea, vomiting, 3rd space loss

Urinary Na· variable

Depletional syndromes Saline required

Fig. 14.2: Evaluation of hyponatraemia

Acid-Base Balance, Fluid and Electrolytes

I. Hypervolaemic hyponatraemia Hypervolaemic hyponatraemia may be associated with clinical features of hypervolaemia such as oedema. Acute hypervolaemia (e.g. TURP syndrome-acute absorption of hypotonic fluids into the intravascular compartment) may result in cerebral oedema and pulmonary oedema. As plasma osmolality decreases, water moves from the extracellular space into the cells leading to oedema. The expansion of brain cells is responsible for the symptomatology of water intoxication: nausea, vomiting, lethargy, confusion, restlessness, etc. If severe ([Na+] < 100 mmol/L), it can result in seizures and coma. Chronic hypervolaemia as in congestive cardiac failure, cirrhosis and nephrotic syndrome may manifest with pedal oedema and elevated jugular venous pressures till de­ compensation occurs. The urinary sodium concentration is less than 15 mmol/L. Treatment Acute hyponatraemia (duration < 72 h) can be safely corrected more quickly than chronic hyponatraemia. The following factors must be evaluated: patient's volume status, duration and magnitude of the hyponatraemia and the degree and severity of clinical symptoms. Fluid restriction, diuretics and correction of the underlying condition may be adequate in most cases. A combination of intravenous normal saline and diuresis with a loop diuretic (e.g. frusemide) also elevates serum sodium concentration. Acute symptomatic hyponatraemia is a medical emergency. It should be treated with hypertonic saline (1.6% or 3%). Concomitant use of loop diuretics increases free water excretion and also decreases the risks of fluid overload. The sodium concentration must be corrected to relieve symptoms and to a concentration of 125 mmol/L. Patients who are acutely symptomatic, the treatment goal is to increase the serum sodium by approximately 1-2 mEq/L/h until the neurologic symptoms subside. The correction should be slow and over a period of 12-24 hours with frequent checks of sodium concentration (every 2--4 h) to avoid overcorrection. Avoid an absolute increase in serum sodium of more than 15-20 mEq/L in a 24-hour period. lf sodium correction is undertaken too rapidly, the resulting osmolality changes in the extracellular fluid can cause central pontine myelinolysis. This condition is serious and can be irreversible. The following equation can aid in the estimation of a sodium deficit to help determine the rate of saline infusion: Calculated sodium deficit = (125 - current serum Na+) x (body weight in kg) x 0.6

203

A litre of normal saline (0.9%) contains 154 mEq sodium chloride (NaCl) and 3% saline 500 mEq NaCl. In chronic severe hyponatraemia (i.e. serum sodium Posm and j, urine output � Extrarenal causes (e.g. diarrhoea, fistulae.) • Uosm > Posm and i urine output � Osmotic diuresis • Uosm < Posm and i urine output � j, ADH or j, renal response to ADH. Treatment I. Administration of water orally/nasogastric tube 2. Administration ofIV fluid-5% dextrose or 0.45% saline 3. Change in serum sodium should not be more than 2 mmol/L/h. Rapid rehydration can cause cerebral oedema.

DISTURBANCES IN COMPOSITION OF BODY FLUIDS POTASSIUM BALANCE The normal potassium level is 3.5-5.5 mmol/L. Hypokalaemi, and hyperkalaemia are two clinically important disturbances. Hypokalaemia (Key Box 14. 7) This is defined as a plasma concentration of potassium less than 3.5 mmol/L.

............... CAUSES OF HYPOKALAEMIA

�

• Reduced intake • Tissue redistribution: Insulin therapy, alkalaemia, �2 adrenergic agonists, familial periodic paralysis. • Increased loss: Gastrointestinal losses-diarrhoea, vomiting, fistulae • Renal causes: Diuretics, renal artery stenosis, diuretic phase of renal failure

Symptoms • Anorexia, nausea • Muscle weakness, paralytic ileus • Altered cardiac conduction: Delayed repolarisation, reduced height of 'T' wave, presence of 'U' wave, wide QRS complexes and arrhythmias. Management • Diagnosis and treatment of the cause • Repletion of body stores • Potassium supplements, in the form of milk, fruit juice, tender coconut water. • Syrup potassium chloride orally-15 ml contains 20 mmol of potassium. • If the patient cannot take orally or the hypokalaemia is severe, intravenous potassium chloride is usually given at a rate of 0.2 mmol/kg/h. If there are life-threatening arrhythmias, it may be given at a rate not exceeding 0.5 mmol/kg/h under electrocardiographic monitoring and serial measurements. Hyperkalaemia • This is defined as a plasma concentration of potassium more than 5.5 mmol/L. Clinical features • Vague muscle weakness, flaccid paralysis Electrocardiographic changes • Tall, peaked 'T' waves with shortened QT interval (6-7 mmol/L)

205

Acid-Base Balance, Fluid and Electrolytes

• Wide QRS complex, widening and then loss of 'P' wave (8-10 mmol/L) Wide QRS complex, merge into 'T' waves (sine wave pattern) • Ventricular fibrillation (K+ > IO mmol/L) Treatment of hyperkalaemia I. Calcium gluconate (10%): 10-30 ml. 2. Sodium bicarbonate: 1-2 mmol/kg over 10-15 minutes. 3. 100 ml of 50% dextrose with 10-12 units of insulin over 15-20 minutes. 4. Hyperventilation 5. Salbutamol nebulisation 6. Calcium exchange resins 7. Peritoneal or haemodialysis MAGNESIUM • Magnesium is the second most abundant intracellular ion. The normal intracellular concentration of magnesium is about 26 mEq/L and extracellular concentration is 1.5-2.5 mEq/L. Almost 60% of the magnesium is deposited in the skeleton. Magnesium is required as a cofactor for several important enzymatic reactions including the phosphoryla­ tion of glucose within cells and the use of ATP by contracting muscle fibres. • A daily dietary intake of 0.3-0.4 g (approximately 20-30 mEq) is required. The proximal convoluted tubule reabsorbs magnesium very effectively. CALCIUM • Calcium is the most abundant mineral in the body. Ninety­ nine per cent is deposited in the skeleton. In addition, calcium ions are important for the control of muscular and

A 21-year-old lady was found to be collapsed as she was feeding her 15-day old baby in the nephrology ward. On arrival, the cardiac arrest response team found her to have ventricular tachycardia without pulse. Cardiopulmonary resuscitation was given and she was shifted to the intensive care unit after return of spontaneous circulation. Investigations showed that her potassium level was 1.6 mmol/L. She had been admitted to the nephrology unit for postpartum acute renal failure. She had been dialysed three times following which she had gone into the diuretic phase of recovery from acute renal failure. She was putting out about 5 litres of urine per day in the last two days. Her hypokalaemia was corrected over 2-3 days. She recovered completely and could be discharged from the ward in 5 days time.

neural activities, in blood clotting, as cofactors for enzymatic reactions and as second messengers. • Calcium homeostasis reflects a balance between reserves in the bone, rate of absorption across the digestive tract, and rate of loss from the kidneys. The hormones parathyroid hormone (PTH), vitamin D and calcitonin maintain calcium homeostasis in the ECF. Parathyroid hormone and vitamin D raise Ca2+ concentrations and calcitonin lowers it. • Calcium absorption from the digestive tract and reabsorp­ tion along the distal convoluted tubule are stimulated by P TH from the parathyroid glands and calcitriol from the kidneys. The average daily requirement of calcium in an adult is 0.8-1.2 g/day. Hypercalcaemia (Key Box 14.8) Hypercalcaemia exists when the Ca2+ concentration of the ECF is above 11 mg%. Features Severe hypercalcaemia ( 12-13 mg%) causes symptoms such as fatigue, confusion, cardiac aIThythmias, and calcification of the kidneys and soft tissues throughout the body. Hypocalcaemia (Key Box 14.9) Hypocalcaemia exists when calcium level is < 9 mg%. However, the serum calcium level should be related to the albumin levels. Half the serum calcium is bound to albumin and as albumin levels become low, this bound fraction is lower leading to a low total serum calcium concentration. Free ionic calcium is important for the electrical activity of the nerves and muscles and is more reliable (Normal: l .0-1.4 mmol/L). Features Muscle spasms, stridor, generalised convulsions, myocardial depression, cardiac arrhythmia and osteoporosis.

.............. CAUSES OF HYPERCALCAEMIA

�

• Hyperparathyroidism • Malignant cancers of the breast, lung, kidney or bone marrow

............... CAUSES OF HYPOCALCAEMIA • Hypoparathyroidism • Vitamin D deficiency • Chronic renal failure

�

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Manipal Manual of Surgery

PERIOPERATIVE FLUID THERAPY A patient undergoing surgery needs intravenous fluids to replace volume deficit acquired during starvation, normal maintenance for the duration of surgery and volume lost during surgery. Depending on the extent of dissection, fluid accumulates in these tissues in the form of oedema (third space losses). In addition, blood loss also needs to be replaced. Perioperative fluid therapy in a patient whose body homeostasis is normal The replacement is as follows: 1. Fluid requirement during starvation: Patients awaiting anaesthesia and surgery need to be kept fasting for a few hours prior to and after the surgery. Except in very minor surgery, fluid lost during this period needs to be replaced. This volume is calculated at the rate of 2 ml/kg/h for the number of hours of fasting and is then replaced over 2-3 h. 2. Maintenance requirement: The average daily requirement of water for an average-sized adult is 2000 ml. In general, a volume of 30-35 ml/kg/day meets the daily maintenance needs. This is calculated as 2 ml/kg/h of surgery. 3. Third space losses: • 4 ml/kg/h for surgery with minimal dissection, e.g. herniorrhaphy • 6 ml/kg/h for surgery associated with moderate dissection, e.g. gastrojejunostomy and vagotomy • 8 ml/kg/h for surgery associated with large amount of dissection, e.g. Whipple's procedure. 4. Blood loss is replaced by compatible blood transfusion (homologous or autologous), if the haematocrit falls below 25%. Blood loss is replaced with an equal amount of colloids or three times the volume with crystalloids if the haematocrit is > 25% in an otherwise healthy individual. Crystalloids are electrolyte solutions that distribute themselves throughout the body water and hence, a larger volume needs to be given. A patient undergoing surgery should receive fluid deficit due to starvation + maintenance fluids + third space losses + replacement of blood loss (as detailed above). Adequacy of fluid replacement should be checked with haemodynamic stability and urine output in major procedures. When very large fluid shifts are expected (oncologic surgeries), and the patient has compromised cardiac status or has renal insufficiency, it may be necessary to monitor fluid status using central venous pressure monitoring. Perioperative fluid therapy in patients with disturbed fluid balance Derangements of fluid therapy can be classified as: a. Disturbances of volume

b. Disturbances of concentration c. Disturbances of composition. In the evaluation of a patient with a suspected problem ir fluid and electrolyte or acid-base balance, careful sequential analysis of the volume, concentration and composition (in tha1 order) followed by appropriate therapy protects the patien1 from severe, perhaps fatal errors in management. Types of intravenous fluids These can be broadly divided into three groups: crystalloids, colloids and special purpose solutions. Crystalloids: These are essentially solutions of electrolytes in water, e.g. Ringer lactate. Some also contain dextrose, e.g. dextrose saline, 5% dextrose and paediatric maintenance solutions. They vary in the content of different electrolytes. Colloids: These are solutions of large molecules which tend to remain in the intravascular compartment, e.g. gelatine, hetastarch, pentastarch, dextran 40, dextran 70. They are all plasma expanders since the molecules tend to exert osmotic forces and draw fluid from the interstitial compartment into the intravascular space. The colloids vary in their magnitude of volume expansion and duration of action. Dextran 40 reduces viscosity of blood and maintains blood rheology better. Hence, it is used as continuous infusion after microvascular surgery. It also reduces platelet aggregation which along with reduced viscosity helps in maintaining blood supply to free flaps and vascular grafts. Volume deficits and losses are usually replaced using Ringer lactate. However, if the patient has disturbances of concentration and composition, other solutions may need to be given. SPECIAL PURPOSE SOLUTIONS Sodium bicarbonate: It is available as 7.5% (0.9 mEq/ml) and 8.4% (1 mEq/ml) of sodium bicarbonate. Uses • • • •

As an alkalinising agent To treat metabolic acidosis To treat hyperkalaemia Forced alkaline diuresis.

Disadvantages • Increased sodium load • Alkalosis with a shift of oxygen dissociation curve to the left (increased affinity of haemoglobin to 02, reducing its unloading) • Increased i ntracran ial pressure and intra ven tricu I ar haemorrhages in neonates

Acid-Base Balance, Fluid and Electrolytes

• Circulatory overload leading to cardiac failure • Carbon dioxide load leading to respiratory failure Mannitol (10 and 20%): It is an osmotic diuretic. Its main use is to reduce intracranial pressure by producing diuresis. It is also used to reduce intraocular pressure. Mannitol expands intravascular volume initially by drawing fluid from the interstitium. This is followed by diuresis. It should be used with caution in patients with cardiac failure, renal failure, etc. Hypertonic saline (1.6, 3, and 5%): These solutions are available to treat hyponatraemia.

•

•

Albumin: 5% albumin is used as a plasma expander. 20% albumin can be used to replace lost albumin in severe hypoalbuminaemia in addition to plasma expansion. NUTRITION

•

Nutritional support to patients Cells can perform their function only when they get nutrients and oxygen. When the nutrients are metabolised in the cells, the tissues get energy to perform their physiological functions. Gastrointestinal system (GIT) is the source of supply of nutrients (energy) to all the tissues. It has to supply nutrients on a day-to-day basis because the body has limited expendable reserves (stores). Lack of nutrients results in energy crisis. In critical care units, priority is given to treatment of hypoxia, haemorrhage, haemodynamic instability, fluid, electrolyte, acid-base imbalance, and sepsis. These deserve their priority, but hyponutrition and the consequent energy crisis should not be ignored. • Pathophysiology of GI failure-autocannibalism: When GIT fails, the stores are consumed to supply energy. Glycogen stored in the liver undergoes glycogenolysis and gets depleted in 24 to 48 hours. Normal physiological functions of the organs of an average adult at rest (BMR/ Resting energy expenditure-REE) need about 20 kcal/kg/ day. Therefore, when GIT fails, the fat in the adipose tissue and protein in the muscles and viscera are mobilised and metabolised to supply and sustain REE. This is called autocannibalism (eating one's own tissues to survive). It weakens the muscles (e.g. respiratory and cardiac muscles), viscera (liver, kidneys, etc.) and immune system, resulting in increased morbidity and mortality. • Patients receiving intravenous (IV) fluids are semistarving: 500 ml of 5% dextrose containing 25 g of dextrose provides about I 00 kcal (each gram of carbohydrate provides about 4 kcal), and therefore an adult taking nil by mouth (NBM), receiving 4--5 bottles of 5% dextrose/day gets about 400-500 kcal; the balance amount of REE (for a 50-kg individual, REE= 50 x 25= 1250 kcal;

•

•

•

207

1250 - 500 = 750 kcal) comes from glycogen for 24 to 48 hours and thereafter from autocannibalism which is detrimental. Fasting in healthy persons versus fasting in patients: When resting healthy persons are fasting, the metabolic rate drops to basal level. Although critically ill patients who are kept N BM are also resting, their basal metabolic rate is accelerated (hypennetabolic) and their REE is increased proportionate to the level of stress due to injury or illness. Assessment of nutritional status: A healthy adult can withstand semistarvation (receiving IV fluids only) for about 4 to 5 days without ill-effects. Beyond that they need nutritional support in order to prevent the adverse effects of autocannibalism. Many patients are nutritionally depleted at the time of admission and will need nutritional support much earlier. Weight loss is an important indicator of nutritional status. A recent h/o 20% weight loss indicates mild, 20 to 40% moderate and more than 40% severe undernutrition. Body mass index (BMI) is another easily calculated indicator. Mid-arm circumference, triceps skin fold thickness are anthropometric indices that are useful. Low serum albumin (3-3.5 g%-mild, 2 g%-moderate and < 2 g%-severe undemutrition) is not a sensitive indicator because of the long half-life and a large pool size. Prealbumin, retinol­ binding protein and transferrin have shorter half-life and smaller pool size and are more sensitive indicators. How much nutrition to be given: Resting energy expenditure (REE) can be more accurately calculated by the Harris-Benedict equation. The REE x stress factor equals total caloric requirement. The stress factor depends upon the severity of illness, injury or operation and ranges from 1.2 to 2.0. For practical purposes, it is easier to remember that the adult REE is 20 kcal/kg/day and it increases to 25, 30, and 40 kcal/kg/day in mild, moderate and severe stress. Sixty per cent of the total calories should come from carbohydrates and 40% from fats. These calories are nonprotein calories. Calories obtained from proteins should not be taken into account for calculating the energy needs because they are building blocks in tissue repair and are not meant for burning for calories. The protein requirement of the resting adult is 0.8 g/kg/day, and it increases to 1.1, 1.5, and 2.0 g/kg/day in mild, moderate and severe stress. Recommended daily allowances of vitamins, minerals and trace elements are added to the formulations. The daily intake and output of fluids should be balanced. Nutrition can be administered by either the enteral or parenteral route.

Enteral nutrition It is paradoxical that sick patients who need to eat more to meet the increased metabolic demands are often unable to eat.

Manipal Manual of Surgery

208

They have anorexia, nausea, vomiting and altered sensorium. Oral feeding is impossible in patients with faciomaxillary injuries or those on ventilators. In many of them, the intestines are working. Enteral route is best for providing nutrition. Hence the dictum, "When the gut is working, use it". • Enteral Access: Following are the routes to introduce nutrients into the GIT (Table 14.3): • Nasogastric feeding: When the stomach emptying is normal and swallowing is impossible or contraindicated, nasogastric feeding (Ryle's tube) provides nutrition. • Nasojejunal feeding: In gastric stasis, feeding can be given through a nasojejunal (NJ) feeding tube introduced either blindly, or under radiologic or endoscopic guidance to place its tip in the jejunum (postpyloric). • Feeding gastrostomy: By open method or by percutaneous endoscopic gastrostomy (PEG) when RT or NJ tube insertion is impossible. • Percutaneous Endoscopic Gastrostomy (PEG): With the help of an endoscope, a gastrostomy tube is placed in a retrograde manner and brought out through a skin incision. It is technically very easy and can be done under local anaesthesia. It has replaced feeding gastrostomies (open method). It is popular nowadays (Fig. 14.3). Complications include colonic perforation, sepsis, bleeding, wound infection, etc. • Feeding jejunostomy: After major/complex operative procedures on the oesophagus, stomach and pancreas, a feeding jejunostomy is frequently established.

• What to feed: A number of preparations are commercial I: available but most cost-effective ones are the blenderise1 kitchen feeds. Enteral feeds are hyperosmolar and provid, 1.2 to 2.0 kcal/ml. • Polymeric feeds: These are commonly prepared in th1 kitchen. Liquid and powder preparations ar1 commercially produced. These contain polysaccharides polypeptides and oils. Soups of dal, vegetables anc chicken are examples of polymeric feeds. • Elemental feeds: These are predigested in vitro anc contain oligosaccharides, oligopeptides and mediurr and long chain triglycerides (MCT and LCT). The) are useful in patients with irritable bowel disease anc short bowel. • Modular feeds: Contain monosaccharides, amino acidi and fatty acids. • Disease-specific feeds: The composition of the feed� needs to be altered in certain disease states. Renal failure-low protein, low/ no electrolytes; Hepatic failure-more branched chain amino acids (BCAA) and less aromatic amino acids; Respiratory failure-more fats (55% cal) and less carbohydrates. • How to feed: The feeds can be gravitated, injected with a syringe or pumped into the tubes either continuously or intermittently. To start with 50 ml every two hours on the first day and if tolerated may be increased gradually to 200 ml every two hours until the target is reached.

Feeding methods (Figs 14.4 and 14.5) Ryle's tube (RT) feeding

Gastrostomy

Feeding jejunostomy

Easy, quick, cheap method Indicated in stroke, comatose patients, etc.

Indicated when RT cannot be passed, e.g. inoperable carcinoma oesophagus, stricture

Indicated after major oesophageal surgeries, high duodenal fistulae

Chances of aspiration are high. Hence, 30° propped up position is recommended

Malecot's catheter is introduced into the stomach and kept in place using a purse string suture (Stamm's gastrostomy)

A Ryle's tube is introduced into the jejunum under vision (during surgery) and kept in place using a purse string suture

Fig. 14.3: Percutaneous endoscopic gastrostomy

tube

Fig. 14.4: Feeding gastrostomy

Fig. 14.5: Feeding jejunostomy

Acid-Base Balance, Fluid and Electrolytes

• Advantages of enteral nutrition: I. The integrity of gut mucosa depends on provision of nutrients into the gut lumen. If the fasting period exceeds more than a few hours, the gut mucosa! cells start disintegrating and the villi get destroyed. This may permit the intestinal bacteria to enter the circulation leading to sepsis. Translocation of bacteria from the intestines into the circulation has been identified as the 'motor of multiorgan failure'. 2. Use of natural route of nutrition requires less nursing superv1s1on. 3. Infection rate is lower with enteral nutrition. 4. Greater insulin response is seen with enteral nutrition. 5. There is a lower tendency to retain salt and water. 6. Enteral nutrition is cheaper. • Complications of enteral nutrition: Nausea, vomiting, abdominal distension and diarrhoea are common. However, intractable diarrhoea should be investigated for Clostridium difficile infection. Electrolyte imbalance, hyperosmolar coma, refeeding syndrome and aspiration are other problems. Tube clogging, displacement, leak and erosion are mechanical complications. lmmunonutrition Glutamine is an essential nutrient for the maintenance of integrity of intestinal cells and there is evidence to show that its inclusion in enteral feeds enhances patient immunity. PARENTERAL NUTRITION When enteral nutrition is not possible for more than a few days, parenteral nutrition (PN) may need to be considered. When all nutrition is done by the parenteral route, it is termed total parenteral nutrition. Partial parenteral nutrition may be given to supplement inadequate enteral nutrition. Parenteral nutrition (PN) to GI failure is like dialysis to renal failure and ventilator support to respiratory failure. When enteral feeding is impossible, parenteral nutrition should be given. Prolonged ileus, intestinal obstruction, malabsorption, short gut, inflammatory bowel disease, high output intestinal fistulae are some common indications for PN. PN formulations are marketed as 1. Dextrose (10, 20, 25 and 50%), amino acid (5 and 10%) and lipid (10 and 20%) solutions in separate bottles. 2. Dextrose + amino acid solutions 3. All in one solutions. Insulin is commonly added to PN solutions. Heparin 1s occasionally added.

209

Administration of nutrients Carbohydrates One gram of glucose provides 4 kcal. The entire energy requirement of the day can be given in the form of glucose. This needs to be administered in a concentration of 50%, so as not to exceed the daily fluid requirement. I000 ml of 50% dextrose contains 500 g of dextrose providing 2000 kcal. Problems • Hyperglycaemia must be prevented by addition of appropriate amount of insulin. • Potassium supplements need to be added to avoid hypokalaemia. • Increased CO2 production may increase work of breathing and results in difficulty in weaning patients from ventilator. • Hypertonic solutions produce thrombophlebitis • Infusion of dextrose-containing solutions do not take care of daily protein and other nutrient requirements of the body. Thus, parenteral nutrition should not be given using dextrose alone. Lipids One gram of fat provides 9 kcal. Since, it is a more concentrated form of energy, half the daily caloric requirement can be given using 10 or 20% lipid emulsions. Problems • Occasionally lipid infusion can cause impaired pulmonary diffusing capacity. • Its clearance from plasma may be delayed with impaired liver function. • Lipid emulsions are expensive. Proteins The average protein requirement is I g/kg/day. Amino acid replacement solutions are available as 10 and 20% solutions. The calorific value of proteins should not be counted as they are the building blocks of the body. They are not infused to be metabolised for energy. Electrolyte requirements The daily requirement of various electrolytes are given below. Electrolyte mmol/kg/day 1-2 Sodium Potassium 1 Calcium 5-10 Magnesium 5-10 Vitamins are given separately. Trace elements are given weekly to patients on long-term PN. One ampule of water­ soluble vitamins must be infused daily, over a period of time exceeding 30 minutes to avoid urinary loss. Folic acid, vitamins B 1 2, K, A and D also need to be given once a week.

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Manipal Manual of Surgery

The total volume of PN should be 2000-3000 ml/day. Alternately, commercially available PN solutions which are well-balanced with all nutrients can be used. Although expen­ sive, good nutrition may reduce the morbidity and duration of hospital stay of the patient thus proving cost-effective.

Routes of administration 1. Peripheral vein: Solutions with less than 800 mOsm/L may

be administered through a peripheral vein. This is suitable for short-term PN. 2. Central vein: Either internal jugular or subclavian vein is cannulated. PN solutions with higher osmolality must be given through a central vein. 3. Peripherally inserted central vein catheter: This also may be used for shmi-term purposes. 4. Subcutaneously implanted central vein catheter ports are especially suitable for long-term, domestic or ambulatory PN. PEARLS OF

WISDOM

The central venous access should be dedicated to PN and should not be used for administration of drugs or other fluids. The line should be handled with strict asepsis.

Methods of administration • The solutions can be gravitated but the rate of infusion is better controlled if given through pumps. Smaller volumes are given initially and is gradually increased to reach the target volume/day. Absolute aseptic precautions are observed while handling the catheters and the PN formulations, since central vein catheter infection is a dangerous complication. Central vein catheter should be used exclusively for administering PN solutions and should not be used for any other purpose. Monitoring Aim I. To identify excess or deficiency of individual nutrients. 2. To identify complications.

Daily • Blood sugar, serum electrolytes, blood urea and serurr creatinine. Biweekly • Liver function tests, coagulation profile, complett haemogram. Complications of PN 1. Technical complications: Injury to subclavian/carotid artery, brachia! plexus, haemo or pneumothorax. 2. Catheter-related: Central line sepsis is the most dangerous, at times, life-threatening and yet, preventable complication and its incidence is a measure of patient safety. It may not be possible to control sepsis with antibiotics alone without removing the central catheter. Thrombosis and catheter clogging are other problems.

3. Gut mucosal atrophy: Patients on total parenteral nutrition develop atrophy of the intestinal mucosa which loses its barrier function and becomes permeable to bacteria. The consequent bacterial translocation leads to sepsis and multiorgan dysfunction syndrome (MODS). This is not seen in patients on partial PN supplemented with EN. 4. Cholestasis: Some patients on long-term TPN develop cholestasis, jaundice and gallstones which resolve on starting oral/EN. 5. Fluid, electrolyte and acid-base imbalances are common. Assured delivery of nutrients, accurate and rapid correction of fluid, electrolyte and acid-base imbalances are the merits of PN but it is complicated and expensive. PEARLS OF

WISDOM

Nutrition is an important component of the care of the surgical or critically ill patient. Malnutrition can be fatal. Overnutrition can also be harm/ul. Provision of services of a physician, dietician, microbiologist and good nursing care are vital to patient recovery.

Acid-Base Balance, Fluid and Electrolytes

211 ·,

MULTIPLE CHOICE QUESTIONS 1. The normal hydrogen ion concentration of plasma is ---- nmol/L: A. 30 B.40 C. 50 D. 60 2. The most important buffer system in the plasma is: A. Phosphate buffer system B. Ammonia buffer system C. Proteins D. Bicarbonate-carbonic acid buffer system 3. pH is the negative logarithm to the base 10 of hydrogen ion concentration expressed in ____ B. mmol/L A. mol/1 C. nmol/L D. µmol/L 4. The solubility coefficient of carbon dioxide in plasma is ml/mm Hg/dL A. 0.3 B. 0.03 D. 3 C. 0.003 5. The following is true in primary metabolic acidosis with secondary respiratory alkalosis: A. pH and PC02 are decreased B. pH decreases but PaC02 increases C. pH and PaC02 are increased D. pH increases but PaC02 decreases 6. The following is one of the causes of metabolic acidosis with increased anion gap: A. Diarrhoea B. Intestinal fistula C. Pancreatic fistula D. Diabetic ketoacidosis 7. The following equation represents the nonlogarithmic form of acid-base equation: A. Harrison B. Henderson C. Hutchinson D. Hanson 8. The following may be a cause of respiratory alkalosis: A. Morphine overdose B. Chronic obstructive pulmonary disease C. Salicylate poisoning D. Curare poisoning 9. In the normal oxyhaemoglobin dissociation curve, a saturation of90% corresponds to mm Hg P02· A. 40 B. 60 D. 120 C. 80

10. Metabolic acidosis with normal anion gap is also called: A. Hyperchloraemic acidosis B. Hyperkalaemic acidosis C. Hypematraemic acidosis D. Hypercalcaemic acidosis 11. The following electrolyte contributes most to the osmolality of plasma: A. Sodium B. Potassium C. Magnesium D. Calcium 12. The normal plasma osmolality is ____ mOsm/kg: B. 290 A. 190 C. 160

D. 260

13. Plasma osmolality is determined in the laboratory using: A. Freezing point B. Boiling point C. Saturation point D. Isoelectric point 14. The second major intracellular cation is: A. Sodium B. Potassium C. Calcium D. Magnesium 15. In chronic hyponatraemia, the sodium concentration should be increased at a rate not exceeding ___ mmol/L/h. A. I B. 5 C. 10 D. 15 16. The following is one of the drugs used to treat hyperkalaemia: A. Digoxin B. Magnesium sulphate C. Atropine D. Insulin 17. Tall 'T' wave in the electrocardiogram is a feature of: A. Hypokalaemia B. Hyperkalaemia C. Hypocalcaemia D. Hypercalcaemia

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18. Rapid infusion of the following fluid can ca use intraventricular haemorrhage in neonates: B. 8.4% sodium bicarbonate A. 5% dextrose C. 0.9% saline D. Ringer lactate 19. Translocation of bacteria from the following organ can lead to multiorgan failure and thus this organ is called 'motor of multiorgan failure': B. Brain A. Kidney C. Gut

20. Hypercalca emia may be seen in the followin conditions except: A. Hyperparathyroidism B. Malignant cancers of the breast and lung C. Vitamin D toxicity D. Chronic renal failure

D. Liver

ANSWERS 1 B 11 A

2 D

3A

48

5A

60

7 B

8 C

9 B

10 A

12 B

13 A

14 D

15A

16 D

17 B

18 B

19 C

20 D

15 Tumours and Soft Tissue Sarcoma • Benign tumours - Papilloma - Fibroma - Lipoma • Neural tumours - Neuroma - Neurofibroma

- Neurilemmoma -Chordoma • Malignant tumours - Paraneoplastic syndromes • Soft tissue sarcomas - Differential diagnosis of soft tissue sarcoma

Introduction A tumour is a new growth consisting of cells of independent growth arranged atypically and serves no function. Broadly classified into: • Benign • Malignant BENIGN TUMOURS PAPILLOMA This is a benign tumour arising from skin or mucous membrane. It is characterised by finger-like projections with a central core of connective tissue, blood vessels, lymphatics and lining epithelium (Fig. 15.1). It can be called Hamartoma or a skin tag. It is an example of overgrowth of fibrous tissue (Key Box 15.1). It can be pedunculated with narrow base or broad base. PEARLS OF

WISDOM

Acrochordons (skin tags) are fleshy, pedunculated masses located on the axillae, trunk and eyelids.

• Keloid

: Page 8

• Desmoid tumour

: Page 910

• Hypertrophic scar

: Page 8

-

Liposarcoma Malignant fibrous histiocytoma Synovial sarcoma Angiosarcoma Rhabdomyosarcoma Kaposi's sarcoma Dermatofibrosarcoma protuberans

Types

1. Skin papilloma a. Squamous papilloma occurs in the skin, cheek, tongue, etc. • Soft papillomas are squamous papillomas. They are seen in elderly patients on the eyelid as small, soft, brownish swellings. • Squam o u s papilloma can also be congenital, sometimes multiple in number and can be sessile or pedunculated. b. Basal cell papilloma (seborrhoeic keratosis) is seen on the trunk of elderly patients as brownish elevated patch of skin and gives a semitransparent, oily appearance. 2. Arising from mucous membrane of visceral organs a. Transitional cell papilloma in the urinary bladder as a cause of haematuria. b. Columnar cell papilloma in the rectum as a cause of mucous diarrhoea. c. Cuboidal cell papilloma in the gall bladder. d. Squamous papilloma in the larynx can cause respiratory obstruction. e. Papilloma ofbreast (duct papilloma) causes bleeding per nipple. Treatment • Excision, only if papilloma causes discomfort, or if it is symptomatic.

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Fig. 15.1: Structure of papilloma

Manipal Manual of Surgery

Fig. 15.2: Pedunculated papilloma thigh-broad base

Complications 1. Skin papilloma can get secondarily infected resulting in pain and swelling. 2. Ulceration and bleeding (trauma) 3. Papilloma in the breast, rectum, tongue and gall bladder can undergo malignant change. FIBROMA1 Fibroma is a benign tumour, consisting of connective tissue fibres only. Clinically, it presents as a firm, subcutaneous swelling. However, a truefibroma is rare. They are combined with neural elements, muscle tissue or fatty tissue. Types 1. Soft fibromas: Less fibrous tissue 2. Hard fibromas: More fibrous tissue • Neurofibroma: Fibroma mixed with nerve fibres • Fibrolipoma: Fibroma mixed with fat

Fig. 15.4: Lipoma in the flank-commonest site of lipoma

Fig. 15.3: Papilloma thigh with a narrow base-easy to remove it. Ulceration can be a problem here

• Myofibroma: Fibroma mixed with muscle fibres • Angiofibroma: Fibroma mixed with blood vessels Treatment They are treated by excision because of the possibility oJ developing into a sarcoma. LIPOMA: UNIVERSAL TUMOUR Lipoma is a benign tumour arising from fat cells of adult type. It is also called 'universal tumour' because it can occur anywhere in the body where there is fat (Key Box 15.2). Types 1. Single encapsulated lipoma

• This is a single, soft, slow-growing, painless and semi­ fluctuant swelling (Figs 15.4 and 15.5).

Fig. 15.5: Giant lipoma in the chest wall ( Courtesy: Prof Raj iv Shetty, HOD, Bangalore Medical College, Medical Superintendent, Bowring Hospital, Bangalore)

1Students should not give the diagnosis of fibroma because in majority of cases, it is neurofibroma or fibrolipoma.

Tumours and Soft Tissue Sarcoma

Fig. 15.6: Multiple lipomas-being subcutaneous in location, they become prominent on contract ion of muscles

Fig. 15. 7: Retroperitoneal lipoma. It can grow to large dimensions. Mostly asymptomatic, and hence patients come late to the hospital

............................. KEY BOX 15.2

• • • • •

.

DIAGNOSTIC FEATURES OF LIPOMA Subcutaneous-commonest type Soft to firm lobular swelling 'Slip' sign positive-a pathognomonic sign Semifluctuant swelling 'Smart' dimple sign on movement of the skin

• The swelling is soft, may feel cystic with fluctuation. This is also called pseudofluctuation because fat at body temperature behaves like fluid. • Surface is lobular. Lobulations are better appreciated with firm palpation of the swelling. Due to the pressure, lobules bulge out between the fibrous tissue strands. • The edge slips under the palpating finger which is a pathognomonic sign of Lipoma.

Fig. 15.8: Mediastinal lipoma. The swelling was deep to pretracheal fascia and partly substernal. It was soft and lobular-confused for com­ pressible swelling. Candidate offered haemangioma as diagnosis. (Courtesy: Dr Salim, HOD, Surgery, Trivandrum Medical College, Kerala (PG exam case 2009)

215

Fig. 15.9: Lipoma forehead. An undergraduate gave the diagnosis as dermoid cyst. You see the location. It is not the classical site of dermoid cyst and the 'slip sign' was positive. Even though face is not a common site, lipoma can occur in this location. Another differential diag­ nosis for this swelling is sebaceous cyst

• Commonly present as a subcutaneous swelling. It is freely mobile. The flank is the commonest site. Shoulder region, neck, back, upper limbs are the other common sites. (For various locations see Table 15.1, Figs 15.8 and 15.9.) Some lipomas from the chest wall can be of large size. • Dimpling sign: Fibrous bands connect a lipoma to the skin. When the skin moved, a dimple appears on the skin. 2. Multiple lipomatosis (Fig. 15.6) • Such lipomas are multiple and very often tender because of nerve elements mixed with them. Hence, they are called multiple neurolipomatosis. Dercum s disease is one example of this variety (Adiposis dolorosa) wherein tender, lipomatous swellings are present in the body, mainly the trunk. 3. Uncapsulated lipoma (diffuse) • Diffuse variety is a rare type of lipoma. It is called pseudolipoma. It is an overgrowth of fat without a capsule. Histological types of lipoma 1. Fibrolipoma: Since fibrous tissue is mixed with fat, lipoma feels hard. 2. Neurolipoma: Painful Jipoma, because of presence of nerve elements. 3. Naevolipoma: Lipoma is usually relatively avascular but this variety is vascular.

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Manipal Manual of Surgery

Various types of lipoma Location

Presentation

Differential diagnosis

Significance

I. Subcutaneous Shoulder Flank

Mobile Lobular Edge slips under palpating fingers.

Neurofibroma

The most common variety

2. Subfascial Limbs, palm, sole

Difficult to appreciate the edge and lobulation

Implantation dermoid, TB tenosynovitis

Subfascial lipoma of the scalp-erodes bone.

3. Subsynovial,

Swelling in relation to knee joint, elbow joint (Figs 15.12 and 15.13)

Bursa Baker's cyst

Intra-articular lipomas are rare

4. lntermuscular Thigh Shoulder region

Swelling of the thigh. On contraction of the muscles, it becomes more finn due to transmitted pressure

Fibrosarcoma Haematoma

Chances of developing liposarcoma are more

5. Parosteal

Under the periosteum of bone, feels hard

Bony tumour

Very, very rare

6. Submucous Intestines, larynx

Asymptomatic or stridor or pain abdomen

Intestinal tumour Laryngeal tumour

Intussusception

7. Subserosal Retroperitoneum

Retroperitoneal swelling (Fig. 15.7)

Hydronephrosis Retroperitoneal cyst

Liposarcoma

8. Extradural

Very rare

9. lntraglandular

Breast, pancreas

Cystic lesions

Very rare

intra-articular

Treatment I. An incision is given over the swelling. Dissection is canied out all around, separating it from underlying tissues and it is excised (Fig. 15.11). 2. Small Iipoma can be removed by incising the skin followed by squeezing the lipoma out (no dissection method). Complications 1. Liposarcoma: The cuITent view is that lipomas are benign and do not turn into malignancy. However, a few retroperitoneal lipomas and lipoma in the thigh can tum into liposarcoma after many years of growth. Malignancy should be suspected when: • The swelling grows rapidly (Fig. 15.10). • It becomes painful due to infiltration of nerves. • The swelling becomes vascular and red coloured with dilated veins over the surface. • Surface is warm due to increased vascularity. • Skin fungation or fixation occurs later • Mobility gets restricted because of infiltration into deeper planes such as muscle. • Liposarcoma spreads via blood. It rarely spreads via lymphatics. Metastasis in the lung can rarely occur from liposarcoma producing multiple chest secondaries. • Liposarcoma is treated by wide excision followed by reconstruction either by split skin graft or by flaps. In the thigh, sometimes radical surgery may amount to compartmental excision. Chemotherapy and radiotherapy can also be used but the benefit is doubtful (see page 663).

Since 3 months, it is rapidly growing, observe dilated veins and shiny s�n.There � loc� rise of temperature. These features are suggestive of sarcoma.

Fig. 15.10: Pedunculated lipoma of the back of 15 years duration with features suggestive of sarcomatous change

2. Calcification 3. Myxomatous degeneration: Occurs only in retro­ peritoneal lipoma. 4. Intussusception-due to submucosal lipoma of terminal ileum is an abdominal emergency. 5. Saponification (see Key Box 15.3 and Figs 15.12 and 15.13)

217

Tumours and Soft Tissue Sarcoma

............................. . KEY BOX 15.3

SOME 'RARE' FACTS IN LIPOMA • It is rare in children • Rarely gives rise to transillumination (if size is big) • Rarely gets infected (because it is relatively avascular) • Diffuse variety is rare • Neurolipomas are rare • Rarely they turn into malignancy

NEURAL TUMOURS NEUROMA Fig. 15.11: Excised specimen of lipoma-see the lobularity

A 32-year-old man presented with gross swelling of the right leg. He had seen two surgeons earlier who had told him that he had deep vein thrombosis but no treatment was offered. Examination revealed an obvious mass which was palpable in anteromedial and posterior compartment. MRI revealed an intermuscular mass. At exploration, an intermuscular /ipoma weighing 700 g was excised (Figs 15.12 and 15.13).

They are uncommon benign tumours which arise from sympathetic nervous system or spinal cord. They can be classified into true neuromas and false neuromas. True neuroma 1. Ganglioneuroma: It consists of ganglion cells and nerve fibres of sympathetic chain. They are slow-growing tumours. When present in the neck as a parapharyngeal mass, it can cause dysphagia. These tumours can occur in the neck, retroperitoneum or mediastinum. Excision of the tumour is the treatment (see clinical notes). 2. Neuroblastoma: It consists of poorly differentiated cells. It occurs in young children. It is interesting to know that this tumour can undergo spontaneous regression. 3. Myelinic neuroma: It is very rare. It arises in relationship with spinal cord made up of myelinic fibres. • Does not contain any ganglion cells. All these three tumours are called true neuromas. False neuroma These tumours arise from the connective tissue of the sheath of nerve endings. They occur following nerve injuries, lacerations or after amputation. They are of two types:

Fig. 15.12: Compare both the legs. Local gigantism can also be caused by extensive lipomatosis involv­ ing the leg. This was treated initially as filariasis

Fig. 15.13: Soleus muscle is cut, lipoma is seen coming out of the deeper plane. Luckily no major neurovascular bundle was invol­ ved. The patient had a smooth recovery

An 18-year-old engineering student who had backache was examined by an orthopaedician and referred to general surgery. CT scan of the abdomen revealed mass in the paraspinal region in the retroperitoneum. Laparotomy and excision of the mass was done. It was a ganglioneuroma. Paraspinal region is one of the common sites of ganglioneuroma.

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Manipal Manual of Surgery

1. End-neuroma occurs after amputation due to proliferation

of nerve fibres from the distal cut end of the nerve. This produces a bulbous swelling. If it is caught in the suture line or due to pressure of the prosthesis, it produces severe neuralgic pain. To avoid this, when an amputation is being done, the nerve is pulled downwards and cut as high as possible so that it retracts upwards (Fig. 15.14). 2. Lateral neuromas occur due to partial injury to the nerve on the lateral aspect (Fig. 15.15). Treatment Excision of the neuroma. NEUROFIBROMA

It is a benign tumour arising from the connective tissue of the nerve sheath. Typically, it produces a fusiform swelling in the direction of the nerve fibres. The tumour contain both neural (ectodermal) and fibrous (mesodermal) elements. Clinical types 1. Single subcutaneous neurofibroma (local) Commonly affects the peripheral nerves such as ulnar nerve, median nerve or cutaneous nerves. Occurs in adults. Clinical features • Presents as a painful, subcutaneous nodule. • Tingling and numbness, paraesthesia in the distribution of the nerve, specially when the nodule is compressed. • Round to oval swelling in the direction of nerve fibre. • Smooth surface, with round border. The swelling moves at right angles to the direction of nerve fibres. Vertical mobility is absent. • Consistency is firm. Sometimes, it is hard. • Being a subcutaneous swelling, the skin can be lifted up. Differential diagnosis of subcutaneous nodule 1. Neurofibroma, 2. Metastatic nodule, 3. Cutaneous T cell lymphoma, 4. Chronic abscess

Fig. 15.14: End neuroma

Fig. 15.15: Lateral neuroma

Treatment • It is treated by excision. • In most of the cases, excision is easy as the tumour is we encapsulated. 2. Generalised neurofibromatosis: von Reckling hausen's (vR) disease (Type I) (Figs 15.16 to 15.18) • This is an autosomal dominant disorder transmitted by bot] sexes. The whole body is studded with cutaneous nodule of varying sizes. They are soft and nontender. • Coffee brown pigmentation is characteristic of this conditio1 (cafe au lait spots, Fig. 21.). Cafe au lait spots can b, associated with involvement of cranial nerves-V IIIth nerv, (auditory nerve) acoustic neuroma-a cerebellopontin. angle tumour. Popularly called vestibular schwannoma. • Fibroepithelial skin tags are often present. • Type I is caused by gene mutation on chromosome 17. • The presence of skin pigmentation is an indication of th( common neuroectodermal origin of nerve sheath cells anc melanocytes. • Skeletal deformities such as kyphoscoliosis or osteoporosii are common.

Fig. 15.16: von Recklinghausen's with plexiform neurofibromatosis ( Courtesy: Dr Prashanth Shetty, Professor, Dept. of Surgery, KMC, Manipal)

Fig. 15.17: Case of vR disease with multiple nodules, pigmentation and schwannoma of vagus nerve ( Courtesy: Dr Siddarth Bhandary, Professor, Dept. of Surgery, KMC, Manipal)

Tumours and Soft Tissue Sarcoma

• It may be associated with phaeochromocytoma (high blood pressure). • Sarcomatous changes do occur PEARLS OF

WISDOM

Record the bloo d pressure i n all c ases of von Recklinghausen 's disease because it may be associated with phaeochromocytoma.

Bilateral vestibular schwannoma or acoustic neuromas are pathognomonic of neurofibromatosis type 2, a syndrome resulting from chromosome 22 mutation. It is also associated with increased incidence of meningiomas and gliomas. 3. Plexiform neurofibromatosis (Trigeminal) (Fig. 15.19) • In this condition, the branches of 5th cranial nerve are commonly affected. It can also involve the peripheries. • The affected part is grossly thickened due to fibro­ myxomatous degeneration. • When it involves the branches of trigeminal nerve, following problems can occur: - Tingling paraesthesia in the distribution of V th nerve, especially ophthalmic division. - When it attains a huge size, it can obstruct the vision. As it grows bigger in size, it hangs in front of the neck, as a grossly thickened pendulous fold of skin. • Treatment: Very difficult. Excision can be attempted with plastic surgery repair. 4. Elephantiasis neuromatosa • This condition affects the limbs. It represents an advanced stage of plexiform variety. Gross thickening of subcuta-

Fig. 15.18: von Recklinghausen's d isease-Tumours are multiple, congenital, familial

219

neous tissue gives the appearance of elephant's leg. The skin is dry and coarse (Fig. 15.20). Complications of neural tumours (Key Box 15.4)

lltMJ-..W� COMPLICATIONS OF NEURAL TUMOURS • Atrophy of muscles • Dumb-bell tumours from dorsal spinal nerve root can cause backache or paralysis • Acoustic neuroma-Deafness • Cystic degeneration • Sarcomatous change

5. Pachydermatocoele • This refers to the plexiform lesions mainly found in the neck as a thickened, coiled single mass. NEURILEMMOMA (SCHWANNOMA) • This is a benign tumour arising from Schwann cells. • Commonest site is the acoustic nerve. However, vagus nerve is the most common peripheral site (Fig. 15.22). • They can be single or multiple and present with a fusiform swelling in relationship with the nerves. • They can also arise from a peripheral nerve. Sensory branches are affected more frequently (Table 15.2). • They can also be seen in mediastinum and retroperitoneum. • They are soft, lobulated, well encapsulated tumours. • They are benign and do not turn into malignancy.

Fig. 15.19: Plexiform neurofibromatosis ( Courtesy: Dr Roh it Jain, Asst. Professor, Department of Surgery, KMC, Manipal)

Fig. 15.20: Elephantiasis neuromatosa ( Courtesy: Dr Balakrishna Shetty, Prof of Surgery, KS Hegde Medical Academy, Mangalore)

Manipal Manual of Surgery

220

Comparison between neurofibroma and schwannoma

• • • • • • • •

Neurofibroma

Schwannoma

More common Ectodermal and mesodermal origin Subcutaneous (forearm) nerves are the commonest site Multiple lesions are common Feels finn or hard Tender Can turn into sarcoma Often nerve fibres are entangled with tumour. Hence, excision involves sacrificing nerve also

• • • • • • •

Less conm1on Ectodermal origin Acoustic nerve is the commonest site Very rare Soft Nontender Does not turn into sarcoma • Well encapsulated. Hence, enucleation is possible without sacrificing nerve

Treatment • Excision of the tumour can be done without sacrificing thf nerves because the tumour is well encapsulated anc displaces the nerve.

HAMARTOMA • It is a tumour-like developmental malformation of the tissues of a particular part of the body wherein it is arranged haphazardly. • Hamartoma is a Greek word which means fault or misfire. It is not a clinical diagnosis.

Fig. 15.21: The cafe au lait spots. More than five such spots will appear by early life ( Courtesy: Dr Prashanth Shetty, Professor, KMC, Manipal)

A few examples of hamartoma Haemangioma, neurofibroma, glomus tumour, benign naevus, lymphangioma. Characteristic features • Being a developmental anomaly, they are seen at birth or in early childhood. • In adults, there is a long history of swelling. • Being a malformation (not a tumour), it does not have a capsule. • They can be single or multiple • Some may regress as in strawberry angioma. • They are benign lesions. Treatment • Excision is not only curative but also gives a correct diagnosis. • Care should be taken when it contains vascular tissue such as haemangioma or neural tissue as in cases of neuro­ fibroma. • Facial nerve and its branches may be damaged while excising hamartomatous lesions over the face.

Fig. 15.22: Vagal schwannoma at surgery-you can see the displaced carotid artery and internal jugular vein. Second picture showing the specimen ( Courtesy: Prof Balakrishnan, Dept. of ENT, Kasturba Hospital, Manipal. Initially it was diagnosed as solitary nodule of the thyroid gland but it was not moving with deglutition)

CHORDOMA

• Rare tumour • Remnant of notochord (origin) • Sacrococcygeal region (common site)

Tumours and Soft Tissue Sarcoma

• Carcinoma cheek-fixity to mandible Significance: May necessitate removal of mandible along with wide excision (Fig. 15.23A). • Squamous cell carcinoma-fixed to tibia may necessitate an amputation.

• Resection is difficult, chances of neurological deficit and bleeding are high. • Radioresistant MALIGNANT TUMOURS Types of malignant tumours • They are of two types: Carcinoma and sarcoma. Carcinoma arises from epithelium-ectodermal, endoderrnal or rnesodennal in origin. • Sarcomas arise from soft tissues or bone and are derived from mesoblast or mesenchymal tissues. • It may be observed that mesoderm can give rise to carcinoma and mesenchymal sarcoma also.

2. Lymphatic spread: It is one of the most important features of carcinoma (Key Box 15.5). As you complete reading this book, you will come across many cases and many examples of lymphatic spread of malignant tumours. A few sarcomas also spread by lymphatics (see page 225). Different types of lymphatic spread are given below: • Embolisation: More aggressive tumour means more aggressive spread-by embolisation wherein nodes can be enlarged in a far away station, e.g. malignant melanoma (Fig. 15.23B). • Permeation: Refers to tumour cells travelling along the lymphatic vessel, e.g. carcinoma tongue with sub­ mandibular node enlargement (Fig. l 5.23C).

Pathology (Table 15.3) Spread 1. Local spread: Generally, local spread occurs into adjacent structures. Few examples are given:

A

Local spread

Embolisation

...•

E

Haematogenous

221

�t

C

Permeation

•.

·:

Transcoelomic

F

Seeding

Fig. 15.23: Spread of malignant tumour (see text)

Pathology of tumours Terminology

Explanation

Examples

1. Well differentiated

Cells that resemble very closely their normal counterparts Loss of structural and functional differentiation Loss in the uniformity, loss in the architectural orientation Dysplastic changes involving entire thickness

Well-differentiated squamous cell carcinoma

2. Undifferentiated (anaplasia) 3. Dysplasia 4. Carcinoma in situ

of the epithelium

Poorly-differentiated carcinoma (anaplasia) Barrett's columnar cell lined oesophagus Cheek, tongue, breast, etc.

5. Apoptosis Programmed cell death Seen in malignant tumours ---------------------------------

Manipal Manual of Surgery

222

............................. . KEY BOX 15.5

CARCINOMA

ORIGIN

Comparison of benign and malignant tumours is shown i Table 15.4. PARANEOPLASTIC SYNDROMES (PNS)

• Ectodermal-skin cancer • Endodermal-gut cancer • Mesodermal-renal carcinoma TYPES

• Squamous cell carcinoma • Basal cell carcinoma • Glandular

• Retrograde lymphatic spread: When main lymphatic pathway is blocked retrograde spread can occur and a node in an unusual location may get enlarged, e.g. Irish node (left axillary node enlargement in carcinoma stomach). 3. Haematogenous spread: This is most important method of spread of sarcomas. Also, a few malignancies such as renal cell carcinoma, follicular carcinoma thyroid, carcinoma prostate, carcinoma breast (Fig.15.23D) and malignant melanoma commonly spread by blood. Bony metastasis and lung metastasis results from blood spread. Bone metastasis can vary from mild form with only bone pain to severe form with quadriplegia/pathological fracture. 4. Transcoelomic spread: Spread through peritoneal cavity by dislodgment of malignant cells, e.g. Ca stomach with Krukenberg's tumour-Bilateral bulky ovarian metastasis, commonly seen in premenopausal patients (Fig. l 5.23E). 5. Seeding (Fig. I 5.23F): A few examples are given below: • Cancer of lower lip spreading to upper lip, also called kiss cancer. Other example: Cancer of vulva. • Incision and 'port' site metastasis (port refers to laparoscopic port).

These are interesting syndromes and are listed i1 Table 15.5. Certain cancers produce some specific clinical syndrome (symptom complexes other than cachexia) which cannot b1 explained by their local or distant spread or by the hormone, produced by the tissue of origin of these tumours. These an called Paraneoplastic syndromes (Key Box 15.6). Just to give an example, hypercalcaemia due to skeleta metastasis from carcinoma breast is not considered as PNS but if it occurs without skeletal metastasis, it is considered a: PNS.

...

.......................... .

KEY BOX 15.6

IMPORTANT FEATURES OF PNS

• Incidence : 10-15% of patients with cancer • It may be the earliest manifestation (primary can be occult)

• Bronchogenic cancer and breast cancer are most commonly associated with PNS. • Hypercalcaemia and Cushing's syndromes are the most common clinical syndromes associated with PNS.

• PNS can be a major clinical problem and can be treated. Aetiology of carcinoma in general

1. Tobacco is the most important factor in the development of lung cancer, upper respiratory tract cancer, gastrointestinal tract and genitourinary tract cancer. Carcinoma pancreas is found more commonly in smokers. Passive smokers also have increased incidence of development of cancers.

Comparison of benign tumours and malignant tumours Feature

Benign

Malignant

Growth

Very slow

Rapid

Duration

Long

Short

Pain

Usually not a feature

Pain can be present due to local infiltration

Mobility

Present

Restricted

Fixity

No

Can be present

Consistency

Firm/soft

Hard, irregular

Spread

No

Spreads

Capsule

Capsulated

Uncapsulated

Recurrence after surgery

Does not occur

Can occur if wide excision is not done

Tumours and Soft Tissue Sarcoma

223

Paraneoplastic syndromes Clinical syndrome 1.

Underlying major cancer

Mechanism

Small cell Ca lung, Ca pancreas Small cell Ca lung Hepatoma Squamous cell Ca lung Ca breast, Ca kidney Bronchial carcinoid, Ca pancreas, Ca stomach Renal cell Ca Cerebellar haemangioma

ACTH or ACTH-like substance ADH or atrial natriuretic factor Insulin or insulin-like substance PTH like substance

Thymoma

Immunologic

Adenocarcinoma lung

Unknown

Carcinoma pancreas

Hypercoagulability

Ca pancreas, lung, prostate

Tumour products

Endocrinopathies Cushing's syndrome Hyponatraemia Hypoglycaemia Hypercalcaemia Carcinoid syndrome •

Polycythaemia

Serotonin, bradykinin Erythropoietin

2. Muscle syndrome Myastbenia gravis 3. Bone and soft tissues Hypertrophic osteoarthropathy and clubbing of fingers 4. Vascular Venous thrombosis Disseminated intravascular coagulation

2. Alcohol: Smoking with alcohol increases the permeability of the upper digestive tract mucosa and respiratory mucosa to the carcinogens. Thus, they increase the incidence of cancer. Hepatocellular cancer is commonly found in alcoholic cirrhotic liver. 3. Ionising radiation: Atomic bomb blasts in Japan have definitely resulted in increased number of cases of breast cancer in premenopausal women and leukaemia in children. 4. Ultraviolet radiation: Causes all types of skin cancers. 5. Genetic causes (Key Box I 5.7) 6. Hereditary causes • MEN syndrome: Medullary carcinoma thyroid (Multiple Endocrine Neoplasia) • FPC: Colonic cancer (Multiple) (Familial Polyposis Coli)

............................. KEV BOX 15.7

GENETIC / DEFECTIVE DNA REPAIR Skin cancer 1. Xeroderma pigmentosa 2. Bloom's syndrome Acute leukaemia, various cancers Acute leukaemia, squamous 3. Fanconi's anaemia cell carcinoma, hepatoma Acute leukaemia, lymphoma, 4. Ataxia-telangiectasia breast cancer

.

I Ca = Carcinoma

• Li -Fraumeni syndrome: Familial breast cancer. • Retinoblastoma. 7. Dietary factors • Red meat: Carcinoma colon, carcinoma breast. • Fat: Carcinoma breast, carcinoma colon. • Smoked, charred fish: Carcinoma oesophagus, carcinoma stomach. 8. Chemicals • Benzanthracenes: Skin cancer when painted on the skin • Benzopyrenes: Lung cancer • �-naphthylamine: Bladder cancer • Nitrosamines and amides: Cancer stomach • Aflatoxin 8: Hepatocellular carcinoma • Asbestos: Lung cancer 9. Viral factors • Human T-cell leukaemia virus type 1 (HTLV-1): T cell leukaemia/lymphoma. (RNA virus) • Human papillomavirus (HPV) Cancer cervix, cancer urogenital region • Epstein-Barr virus: Burkitt's lymphoma. SOFT TISSUE SARCOMAS (STS) These are malignant tumours arising from soft tissues. Thus, they can occur in any part of the body. Examples are given in Table 15.6.

Manipal Manual of Surgery

224 Soft tissue sarcoma Tissue of origin

Name

• Mesenchymal tissue

Malignant fibrous histiocytoma (MFH)

• Adipose

Liposarcoma

• Smooth muscle

Leiomyosarcoma (GlST)

• Striated muscle

Rhabdomyosarcoma

• Synovial tissue

Synovial sarcoma

• Neural tissue

Malignant schwannoma

• Uncertain

Epithelioid sarcoma

• Blood vessels

Angiosarcoma

• Lymph vessels

Lymphangiosarcoma

Introduction • These are malignant tumours which are fatal if untreated or mistreated. Most of them occur in young patients as painless lumps. CT scan, MRI and incision biopsy ( details later) are key investigations. Early diagnosis and curative resection have a major role in the management of soft tissue sarcomas. In addition to TNM staging, pathological grading of the tumour has been included GTNM staging. Aetiology/epidemiology of STS (Key Box 15.8) 1. Genetic factors: Genetic mutations/gene rearrange­ ments have been implicated in the pathogenesis of STS. Two genes that are most relevant to soft tissue tumours are retinoblastoma (Rb) tumour suppressor gene and p53 tumour suppressor gene. Various familial syndromes are associated with STS such as Li-Fraumeni syndrome, Gardner's syndrome and von Recklinghausen's disease. Several oncogenes have been identified. 2. Exposure to radiation: 8 to 50-fold increase in the incidence of STS is reported in patients who are treated for cancer of the breast, cervix and ovary by radiation. 3. Neurofibromatosis type I: Specially mentioned here because it is the only benign tumour progressing to STS. 4. Exposure to chemicals: Thorium oxide, vinyl chloride, arsenic have been implicated in hepatic angiosarcomas. 5. Trauma: Chronic tissue trauma is blamed as a triggering factor for development of STS. Others believe that trauma draws attention of the patient to the STS. 6. lmmunosuppression: Kaposi's sarcoma occurs in HIV­ AIDS patients. 7. Chronic lymphoedema can give rise to lymphangio­ sarcoma. Postmastectomy lymphangiosarcoma is called Stewart-Treves syndrome. Clinical features Sarcomas are rapidly growing vascular growth (Table 15.7).

............................. KEY BOX 15.8

AETIOLOGY OF STS-SUMMARY Genetic factors Exposure to radiation Neurofibromatosis (NF-1) Exposure to chemicals Trauma Immunodeficiency Chronic lymphoedema Remember as GENETIC

iifafibi§iitj

Staging of soft tissue sarcoma

1. Grade (G) G1 - Well differentiated G2 - Moderately differentiated G3 - Poorly differentiated G4 - Undifferentiated

2. Primary Tumour (T) a: Superficial tumour b: Deep tumour T1: Tumour < 4-5 cm in greatest dimension T2: Tumour > 5 cm in greatest dimension 3. Regional lymph nodes (N) NO : No nodal metastasis N1 : Regional lymph node metastasis present. 4. Distant Metastasis (M) MO : No distant metastasis M1 : Distant metastasis present G - TNM staging system

Stage grouping II Ill

IV

G1 G2 G3 G1-G3 G1-G3

T1-T2 T1-T2 T1-T2 T1-T2 T1-T2

NO NO NO N1 N1

MO MO MO MO M1

Investigations/diagnostic imaging • Routine blood tests • Chest radiography: Presence of cannon ball metastasis alters the staging, treatment policy and prognosis. PEARLS

OF

WISDOM

If STS is more than 5 cm (T2), computed tomography (CT) of the chest should be considered.

• CT scan is useful in evaluating retroperitoneal sarcomas. It can define structures, infiltration into neighbouring structures, hydronephrosis, etc. CT can guide a core biopsy also.

Tumours and Soft Tissue Sarcoma

225

Comparison of carcinoma and sarcoma Carcinoma

Sarcoma

I. Cell of origin

Ectodermal or endodermal (epithelial)

Mesodermal (mesenchymal)

2. Age group

Elderly, 40-60 years

Young, 10--30 years

3. Rate of growth

Slow

Fast

4. Presentation

Nonhealing ulcer, cauliflower-like growth with everted edges and induration. Fixity is a late fea­ ture

Fleshy mass, red and vascular, dilated veins over the surface, local rise of temperature. Ulceration is a late feature

5. Spread

Lymphatic spread is very common, both by em­ boli and permeation. Blood spread does occur as in renal cell carcinoma, thyroid carcinoma, breast carcinoma, etc.

Blood spread occurs very early and results in cannon ball secondaries in lung. Rhabdomyosarcoma, synovial sarcoma, epithelioid sarcoma. Malignant fibrous his­ tiocytoma, angiosarcoma spread by lymphatics

6. Microscopy

Cell nests or epithelial pearls are seen in well­ differentiated cancers

Malignant cells resemble their cell of origin. Thus, spindle-shaped cells are found in fibrosarcoma

7. Treatment

Surgery is the main treatment of ectodermal cancers, surgery or radiotherapy for ectodermal lesions. Chemotherapy is not very useful

Surgery is the main modality of treatment. Radio­ therapy and chemotherapy are also beneficial when the tumour is more than 5 cm

• MRI is the investigation of choice when STS occurs in extremities to delineate muscle groups, bones, vascular structures, etc. • Biopsy: FNA - Acceptable first investigation to prove the diagnosis. - Useful to detect metastatic disease - To detect local recurrence - Ideal for superficial lesions - Disadvantage: Cannot assess tumour grading. Tissue is not sufficient for diagnostic tests. • Core needle biopsy - Safe and accurate - Tissue is sufficient for grading, electron microscopy and flow cytometry - With CT guidance, core biopsy can be taken from deeper structures also. • Incisional biopsy - When core biopsy tissue is not adequate, incisional biopsy is indicated (Key Box 15.9).

............................. . KEY BOX 15.9

• • • • •

GUIDELINES WHILE DOING AN INCISIONAL BIOPSY Incision should be oriented longitudinally in STS of extremities to facilitate removal of biopsy site, scar and tumour en bloc Flaps should not be raised Perfect haemostasis should be achieved Prevent dissemination Avoid drain

Treatment (see next page) • The aim is to achieve local control and to treat metastasis including subclinical metastasis, thus trying for a cure. • Surgery is the first line of treatment varying from a wide local excision to amputation or disarticulation (5%), when it occurs in the extremities. Low-grade tumours can be treated on 2 cm wide excision of surrounding normal tissue and high-grade tumours by4 cm margin (Key Box 15.10). • Small tumours less than 5 cm have not been associated with recurrence. Hence, radiotherapy may not be required but if grade is high, RT is required (Key Box 15.11). • Tumours do respond to radiotherapy and chemotherapy. Role of chemotherapy • High-grade tumours have high potential ofmetastasis. Hence, combination chemotherapy is to be considered before or after surgery. • The most favoured combination chemotherapy drugs include Mesna, Adriamycin, Ifosfamide and Dacarbazine (MAID). • The success rate is around 10-20%. Sarcomas that metastasise to lymph nodes* • Rhabdomyosarcoma • Angiosarcoma • Clear cell carcinoma • Epithelioid sarcoma • Synovial sarcoma *Remember as RACES

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Manipal Manual of Surgery

A FEW SURGICAL PROCEDURES DONE FOR SOFT TISSUE SARCOMA (Figs 15.24 to 15.29)

Fig. 15.24: A 24-year-old lady underwent excision of a swelling diagnosed as lipoma in a peripheral hospital which was reported as sarcoma

Fig. 15.27: A large (8 cm) liposarcoma of the thigh being exploreo with a longitudinal incision of the thigh. He had undergone an incision biopsy earlier. The biopsy site is removed along with the skin

Fig. 15.25: The swelling was explored-as you can see it was infiltrating the muscles-the first surgeon had not excised the swelling completely

Fig. 15.28: Synovial sarcoma of the toe-disarticulation being done-highly malignant sarcoma

Fig. 15.26: Excised specimen showing skin, tumour and deeper muscle fibres. STS was 3 cm in diameter. Histopathology reported as synovial cell sarcoma. Margins were negative ( Courtesy: Dr Ankur Sharma, Asst Professor, Dept. of Surgery, KMC, Manipal)

Fig. 15.29: Specimen of the growth along with the toe: Wide excision. No nodes were palpable in the leg

Tumours and Soft Tissue Sarcoma

............................. . KEY BOX 15.10

..

227

........................... .

KEY BOX 15.11

SURGERY FOR SOFT TISSUE SARCOMA • Surgery is the primary and most effective therapy • Local wide excision with 2 cm to 4 cm of surrounding normal tissue should be removed. 2 cm for low grade and 4 cm for high grade tumour. • Not to dissect along pseudocapsule which is composed of tumour cells. • If necessary, excision should include nerves and vessels followed by nerve grafts and arterial reconstruction. • STS rarely involve bones and skin. Hence, wide resections of these structures are infrequently necessary. • It should also include previous scars. • If amputation can be avoided by giving preoperative radiotherapy, it is preferred first. It is then followed by wide excision/compartmental excision and postoperative radiotherapy. Dose of pre- and postoperative radiotherapy is 50-60 Gy given in 25 fractions. Brachytherapy is also given. , Margin negative resection should be the aim.

•

RADIOTHERAPY It is not the first modality of treatment in STS.

• Radiotherapy is given after margin negative resection. • Surgery + RT has resulted in local control rates up to or more than 90%. • External beam radiation therapy (electrons, protons or neutrons) is given with a margin of 5 to 7 cm or even more depending on size of tumour. Dose is 50 Gy given in 25 fractions-as preoperative dose. • Dose is 60 to 70 Gy for postoperative treatment. • In brachytherapy, multiple catheters are placed in tumour resection bed. Catheters are loaded with seeds containing lridium-192. • Dose of brachytherapy is 42-45 Gy to tumour bed over 4-6 days. • Short period of treatment time and less systemic toxicity are advantages of brachytherapy.

• Almost 80% of STS metastasise to lungs within 2-3 years of the diagnosis. • Prognosis depends on metastatic STS, grade of the tumour, size of the tumour, margins after resection and anatomical location. • If pulmonary metastasis is resectable, 30% survival may be expected at 3 years.

• The compression on blood vessels may result in oedema of the limbs when it occurs in retroperitoneum. • Well-differentiated myxoid liposarcomas are notoriously known to recur many times before spreading to lungs. Hence, prognosis is good (Fig. 15.42). • Pleomorphic and lipoblastic liposarcomas tend to be of higher grade and often present with metastasis. • Wide excision/surgery is the primary treatment. • They do respond to radiotherapy.

DIFFERENTIAL DIAGNOSIS OF SOFT TISSUE SARCOMA

Hl¥MJ,-w7,.4�

Prognosis

LIPOSARCOMA (Figs 15.36 and 15.37)

RETROPERITONEAL SARCOMA (RPS)

�

• Most common RPS is liposarcoma or leiomyosarcoma. • They constitute 15% of adult soft tissue sarcomas. • May present as large masses of more than 20 cm at the time of presentation. • Local recurrence and intra-abdominal spread is more common. • Progressive abdominal distension, pedal oedema, young age and firm to hard retroperitoneal mass clinches the diagnosis. • CT scan of chest and abdomen followed by FNAC/True cut biopsy for histology/grade of the tumour.

• •

It is a malignant fatty tumour (see page 550). Common sites: Proximal extremity, trunk or retro­ peritoneum (Fig. 15.30). • They are generally large at the time of diagnosis, e.g. retroperitoneum. It results in gross swelling, which is firm to hard (more than 50% will be of> 20 cm size) (Key Box 15.12)

• Margin negative-complete surgical resection is the treatment of choice. • Chemotherapy has not been effective against RPS.

MALIGNANT FIBROUS HISTIOCYTOMA (MFH) • Fig. 15.30: Retroperitoneal liposarcoma

It is a malignant tumour of mesenchymal tissue (fibrous tissue). This is the recent nomenclature of sarcoma.

Manipal Manual of Surgery

228

• • • •

Fibrosarcoma or pleomorphic rhabdomyosarcomas are included under this. Most of the so-called fibrosarcomas are presently included under MFH (Fig. 15.31). These are high-grade tumours that lack differentiation. It can also arise from bone. The MFR: Superficial type rarely metastasises and carries good prognosis. Locations: Retroperitoneum, trunks and limbs (inter­ muscular septae of adductors, scapulohumeral and pectoral muscles).

Clinical features • Common in elderly patients (50 years) but can occur at any age. • Slow-growing, firm to hard mass with restricted mobility. • As the tumour is locally invasive, it infiltrates the muscles and adjacent structures. Thus, it can cause muscle weakness or pain, etc. • Spread: Local spread is common. Distant metastasis by blood is late (lungs). Lymph node metastasis is rare. • Like other sarcomas, dilated veins, local rise of temperature, restricted mobility and hardness will clinch the diagnosis. • MRI is the investigation of choice to know the extent of the disease (Figs 15.32 to 15.35). • Margin negative surgery should be the aim. SYNOVIAL SARCOMA (Fig. 15.40) • Any rapidly growing tumour in the region of joint or near the tendons in young patients (20-40 years), synovial sarcoma is to be considered. • Common site: Shoulder, wrist, knee, etc. • Age: Young between 20 and 40 years. • Clinical features are similar to the other sarcomas-hard, painful mass. • In addition to the local and blood spread, it also spreads by lymphatic route.

Fig. 15.31: Recurrent fibrosarcoma (MFH)

Figs 15.32 to 15.35: T1 - and T2-weighted (W) axial MRI. lsointense to hyperintense mass lesion noted in the anterior compartment of right thigh. Sagittal T2-W MRI shows supero-inferior extent of the lesion

• Plain X-ray: It may show characteristic calcification. • It is aggressive, with high rates of recurrence. • In the G-TNM staging system, they are grade 3. ANGIOSARCOMA (Fig. 15.41) • I to 2% of soft tissue sarcomas. • Affects elderly patients. • They are high grade and aggressive tumours. • They arise from skin and subcutaneous tissue rather than deeper tissues. • Most of them occur in the head and neck, breast and liver. • Surgery (excision) followed by radiotherapy/combination chemotherapy may have to be given. RHABDOMYOSARCOMA • It is the most common soft tissue sarcoma seen in children, even though they are rare ( under the age of 15). • It arises from striated muscle-painless enlarging mass. • Resection/Chemotherapy/Radiotherapy (combination) is tried depending on location. • Sites: Head and neck (30%), genitourinary system (25%), extremities (20%). • All three varieties: Embryonal, alveolar and pleomorphic are considered as Grade 3 in GTNM staging. Hence, prognosis is not good (Fig. 15.43).

Tumours and Soft Tissue Sarcoma

Fig. 15.36: Early stage of liposarcoma-this swelling had local rise of temperature and restric­ ted mobility

Fig. 15.37: Advanced case of liposarcoma-the swelling was hard and fixed. Dilated veins over the surface and location were characteristic

Fig. 15.38: Swelling in the elbow region for two years duration presented to the hospital with bleeding and fungation-advanced case of epithelioid sarcoma

229

Fig. 15.39: Ulcerated lesion in the elbow region of two years duration presented to the hospital with bleeding-a case of dermatofibro­ sarcoma protuberans

Fig. 15.40: Synovial sarcoma left shoulder region. See the secondary varicosity due to pressure effects. Nonextremity sarcomas have poor prognosis

Fig. 15.41: Bleeding vascular lesion in the ankle region­ angiosarcoma (Courtesy: Dr Mallikarjuna Desai, HOD, Surgery, SDMC, Dharwar, Karnataka)

Fig. 15.42: Six times recurrent myxoid liposarcoma since 8 years. It has good prognosis ( Courtesy: Dr Shashi, HOD, Surgery, Calicut Medical College, Calicut, India)

Fig. 15.43: Spindle cell sarcoma ( Courtesy: Dr Laxmi Rao, Head of the Dept. Pathology, KMC, Manipal)

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Manipal Manual of Surgery

• Complete tumour resection should be the aim. Chemo­ therapy and RT are also used. • Rhabdomyosarcomas have a high propensity for lymph node metastasis. KAPOSI'S SARCOMA • Vulnerable section of people include Jews, immunocom­ promised patients such as transplant recipients and AIDS. • Typical sites: Legs. Other sites include chest, arm, neck in epidemic form (Africa). • It presents as multiple pigmented sarcoma nodules in the leg. • It is interesting to note that Kaposi's sarcoma is "not seen" in transfusion related 'AIDS '. • It manifests with purplish to red subcutaneous nodules in the leg followed by ulceration and bleeding. • Combination chemotherapy with doxorubicin, etoposide and interferon have been used to control the disease. DERMATOFIBROSARCOMA PROTUBERANS (Fig. 15.39) • Clinically presents as nodular exophytic 'mass' lesion­ protuberans. • Locally aggressive tumour which does not metastasise • Wide excision should be the aim with negative margin to prevent local recurrence. • Mohs' micrographic surgery such as basal cell carcinoma has been advocated to get negative margin and thus to get a low recurrence rates. • Has good prognosis, if treated early. USEFUL TIPS IN A CASE OF SOFT TISSUE SARCOMA (Key Box 15.13) In undergraduate clinical examination, students are advised to offer soft tissue sarcoma as the diagnosis. When asked the possible type, they only give a possible histological type based on various clinical features mentioned above. Ask following questions to yourself to get ready for the clinical exams. 1. Is it soft tissue sarcoma? Tumour arising from soft tissue, dilated veins, reddish skin, increase in local temperature, firm to hard, rapidly growing swelling, with late involvement of skin (carcinoma starts in the skin) (Key Box 15.13). 2. What is the age of the patient? • In children Rhabdomyosarcoma Undifferentiated sarcoma Liposarcoma • In 20-40 years Synovial sarcoma Kaposi's sarcoma • In elderly patients - Angiosarcoma Chondrosarcoma (bone) Fibrosarcoma (Fig. 15.23)

3. Which site has it occurred? • Head and neck Angiosarcoma Rhabdomyosarcoma Osteogenic sarcoma Uaw) • Distal extremity Synovial sarcoma (Fig. 15.25) (limbs) Epithelioid sarcoma (Fig. 15.38 Clear cell sarcoma • Retroperitoneum - Liposarcoma (Fig. 15.24) and Mesentery MFH, leiomyosarcoma 4. Has it spread to lymph nodes? • Rhabdomyosarcoma • Synovial sarcoma • Epithelioid sarcoma 5. Has it spread to lungs or liver? • Chest X-ray • Ultrasound 6. Can I preserve the limb? How? • Wide excision • Compartmental excision • Preoperative radiotherapy combined with surgery anc postoperative radiotherapy.

............................. . KEY BOX 15.13

• • • • • • • • • • •

INTERESTING 'MOST' TO REMEMBER IN SOFT TISSUE SARCOMA Most of the soft tissue sarcomas arise de novo. Most originate in an extremity (60%) Most common soft tissue sarcoma in adults is MFH (28%). Most common soft tissue sarcoma in children is rhabdomyosarcoma. Most of soft tissue sarcomas metastasise by blood. Most of the extremity soft tissue sarcoma metastasise to lungs. Most of the retroperitoneal soft tissue sarcoma metastasise to liver. Most important prognostic factor for soft tissue sarcoma is size and grade of the primary tumour. Most accurate imaging modality for soft tissue sarcoma is MRI. Most effective modality of treatment is surgery. Most effective chemotherapeutic drugs are doxorubicin, dacarbazine and ifosfamide.

A 34-year-old nondiabetic male patient had a nonhealing ulcer great toe (RT) of 6 months duration. History of minor trauma was present. Examination revealed ulceration, thickening and induration with 'mass' lesion. It was MBBS exam case. The student offered osteomyelitis, foreign body granuloma and tuberculous synovitis as diagnosis. He qualified but he was asked to examine the groin. The patient had 3 hard nodes in the groin. The final diagnosis-Synovial cell sarcoma with metastasis in the groin nodes-the student had not examined the groin!

Tumours and Soft Tissue Sarcoma

231

MULTIPLE CHOICE QUESTIONS 1. When do you suspect lipoma turns into liposarcoma: A. When lipoma gets infected B. When lipoma causes lymphangitis C. When it becomes fixed D. When it undergoes myxomatous degeneration 2. Intussusception is caused by: A. Submucosal lipoma B. Subserosal lipoma C. Retroperitoneal lipoma D. Intraperitoneal lipoma 3. Myxomatous degeneration occurs only in which Lipoma? A. Retroperitoneal B. Subfacial D. Subcutaneous C. Submucosal 4. Following are true for von Recklinghausen's disease except: A. It is an autosomal dominant disorder B. Skin pigmentation is a feature C. Cafe au lait spots are characteristic D. Does not tum into sarcoma 5. Following are true for Schwannoma except: A. Commonest site is acoustic nerve B. Sensory branches are affected more often C. Well encapsulated tumour D. Highly premalignant tumour 6. Following are radiosensitive tumours except: A. Oral cancer B. Seminoma testis C. Carcinoma breast D. Malignant chordoma 7. Which one of the following does not have a capsule? B. Schwannoma A. Hamartoma C. Fibroadenoma D. Branchial cyst 8. Which one of the following is commonly associated with paraneoplastic syndrome? A. Carcinoma stomach B. Carcinoma colon C. Carcinoma pancreas D. Carcinoma lung 9. Which one of the following condition is more commonly associated with paraneoplastic syndrome? A. Hypernatraemia B. Hyponatraemia C. Hypercalcaemia D. Hypocalcaemia

10. Polycythaemia is a paraneoplastic syndrome seen in which condition? B. Wilms' tumour A. Hepatoma C. Apudoma D. Renal cell carcinoma 11. Post mastectomy lymphoedema is called: A. Stewart-Treves syndrome B. Bloom's syndrome C. Fanconi 's syndrome D. Sturge-Weber syndrome 12. Precautions to be taken while doing a open biopsy in soft tissue sarcoma include following except: A. Incision should be longitudinal B. Perfect haemostasis should be achieved C. Drain should be kept D. Flaps should not be raised 13. Sarcomas metastasise to lymph nodes include following except: A. Rhabdomyosarcoma B. Angiosarcoma C. Synovial sarcoma D. Liposarcoma 14. Following are true for the treatment of soft tissue sarcomas except: A. Surgery is the best line of treatment B. Radiotherapy is given after surgery C. Chemotherapy is also given D. Radiotherapy is the first line of treatment 15. Following are true for the treatment of retroperitoneal sarcoma except: A. Surgery is the best line of treatment B. Radiotherapy is extremely helpful C. Chemotherapy is not very useful D. Most of them are liposarcoma 16. Following are high grade soft tissue sarcomas except: A. Angiosarcoma B. Synovial sarcoma C. Malignant fibrous histiocytoma D. Liposarcoma 17. Following are true for glomus tumour except: A. It can turn into malignant B. It is an angioneuromyoma C. It is radio resistant D. It is concerned with heat regulation

ANSWERS 1 C 11 A

2 A

3 A

4D

5D

6D

7 A

12 C

13 D

14 D

15 B

16D

17 A

8D

9 C

10 D

16 Cystic Swellings, Neck Swellings and Metastasis Lymph Node Neck • • • • • •

Cystic swellings Neck swellings Differential diagnosis of midline swellings Swellings in the submandibular triangle Swellings in the carotid triangle Swellings in the posterior triangle

Classification of cyst I. Congenital cyst • Sequestration dermoid cyst • Branchial cyst • Thyroglossal cyst • Lymphangioma • Cysts of embryonic remnants: Cyst of urachus, vitello­ intestinal duct cyst II. Acquired cyst • Retention cyst: Sebaceous cyst, galactocoele, spermatocoele, Bartholin 's gland cyst • Distension cyst: Thyroid cyst, ovarian cyst • Exudation cyst: Hydrocoele • Degenerative cyst: Tumour necrosis

AVfistula Secondaries in the neck Different types of neck dissections Pancoast's tumour What is new?/Recent advances

• Traumatic cyst: Haematoma, implantation dem1oid cys1 • Cystic tumours: Cystadenoma of pancreas, cystadenoma of the ovary

CYSTIC SWELLINGS A cyst is a swelling containing fluid. True cysts are lined by endothelium or epithelium. They contain clear serous fluid, mucoid material, pus, blood, lymph or toothpaste like material. The false cysts do not have lining epithelium. They can be degenerative cysts as in the case of tumours which undergo tumour necrosis or tumour degeneration, or merely a collection of fluid which is walled off by coils of bowel as in tuberculous encysted ascites or an exudation cyst as in pseudopancreatic cyst.

• • • • •

III. Parasitic cyst • Cysticercosis • Hydatid cyst Clinical examination of cysts in general Students are requested to follow the standard practice of examination of the swelling in the form of inspection, palpation, percussion and auscultation in the clinical examination. Some important tests for cystic swellings are given in the next page. 1. Location: Most of the congenital cystic swellings have a typical location wherein diagnosis can be made with fair accuracy. A few examples are as follows: • Branchial cyst occurs at the junction of upper one-third and lower two-thirds of the stemocleidomastoid muscle whereas opening of branchial fistula occurs at junction of upper two-thirds and lower one-third of sterno­ cleidomastoid muscle. • Dermoid cyst: Midline, outer or inner canthus of the eye • Meningocoele: Swelling in the newborn at lumbosacral region • Ganglion: On the dorsum of the hand and foot

232

Cystic Swellings, Neck Swellings and Metastasis Lymph Node Neck

Fig. 16.1: Fluctuation should be Fig. 16.2: Transillumination elicited using both hands and in test: It should be done in a both directions dark room

2. Shape: Majority of the cystic swellings are round or oval • Subhyoid bursitis: Transverse c,val cystic swelling in the midline of the neck. • Thyroglossal cyst: Vertically placed oval swelling in the midline of the neck. • Sebaceous cyst: Hemispherical swelling. 3. Surface: Almost all the cystic swellings in the skin and subcutaneous tissue have smooth surface. 4. Consistency: Fluctuation is positive in all cystic swellings (Fig. 16.1 and Key Box 16.1). However, depending on the contents, the fluctuation may be different, which an experienced surgeon can diagnose. • Soft cystic: Thyroglossal cyst, meningocoele, lymph cyst. • Tensely cystic: Ganglion, tensely cystic swellings in the neck may feel finn or solid, e.g. tense thyroid cyst. Cyst in the breast may feel firm or hard. • Yielding in cases of lipoma, as fat at body temperature behaves like fluid (pseudofluctuation). • Soft with firm thickened periphery: Cold abscess • Half filled like a rubber hot water bottle: Branchial cyst • Putty or tooth paste: Sebaceous cyst (true fluctuation is not found). • Cross fluctuation for swellings having two components connected to each other, e.g. plunging ranula. 5. Transillumination test: Cystic swellings which contain clear fluid show positive transillumination (Fig. 16.2). 6. Mobility: Almost all the cystic swellings in the skin, subcutaneous tissue or in the deeper plane are benign and as a rule, they should have free mobility. However, this is not true due to various anatomical factors. • Branchial cyst: Restricted mobility is due to its adherence to the sternomastoid muscle. • Thyroglossal cyst: Transverse mobility is absent because the cyst is tethered by remnant of the thyroglossal duct. • Sebaceous cyst: Limited mobility due to the adherence to the skin. 7. Sign of compressibility: The swellings which have communication with a cavity or with tissue spaces give the positive sign of compressibility. Thus, a steady pressure is

233

applied over the swellings. The swelling may disappear completely or may partially disappear. However, when pressure is released the swelling fills up slowly. Hence, it is also called the 'sign of refilling' (Key Box 16.2). 8. Plane of the swelling • Almost all significant cystic swellings in the neck are deep to deep fascia. Thus, contracting sternomastoid for laterally placed swellings and bending the chin against resistance for centrally placed swellings must be done to define the plane of swelling. • Subcutaneous swellings become more prominent when the underlying muscles are contracted as in limbs. • Swelling due to semimembranous bursitis, almost dis­ appears on flexion of knee and becomes more prominent on extension of the knee. • Sebaceous cysts are attached to the skin at the site of punctum. 9. Pulsations a. Expansile: Aneurysms are characterised by expansile pulsations. When two fingers are placed over the swelling on the sides, the fingers are not only elevated but are also separated. Popliteal aneurysms typically give this sign (Key Box 16.3). b. Transmitted: When the swelling is situated over a vessel, the fingers are raised but not separated, e.g. pseudo­ pancreatic cysts. When the swelling pushes the vessel anteriorly, transmitted pulsation can be obtained, e.g. cervical rib pushing the subclavian artery. c. Pulsation can also be present in vascular tumours such as osteogenic sarcoma or secondaries from carcinoma thyroid, etc.

lftJl$f!IIMWWllilr,1 RULES OF ELICITATION OF FLUCTUATION � • Mobile swelling has to be fixed. • Both hands should be used. • With the index finger and thumb of one hand the swelling is pressed-these are 'active' fingers and the impulse is received by the thumb and index finger of the other hand (passive fingers). • Fluctuation should be elicited in both directions, as fleshy muscle in the thigh can be fluctuant across but not in the longitudinal direction. • When swelling is smaller than 2 cm in size, Paget's test is done. Cystic swellings feel soft in the centre and firm at the periphery. Solid swellings feel firm at the centre than periphery.

............................. . KEY BOX 16.2

COMPRESSIBLE SWELLINGS • Haemangioma • Lymphangioma • Meningocoele

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Manipal Manual of Surgery

ll$•Wllllllllllllll,1 • • • •

ANEURYSM TESTS

�

Expansile pulsations: Finger separation sign Proximal compression test: Decreased size Distal compression test: Size may increase Thrill and bruit are present and distal pulses may be weak.

Effects of aneurysm (Key Box 16.4 and Figs 16.3 and 16.4)

m&MIWllllllllllllll,1 EFFECTS OF ANEURYSM: TIPS

Fig. 16.4: Radial artery aneurysm-with clot-excised specimen ( Courtesy: Prof L Ramachandra, Professor of Surgery, KMC, Manipal)

�

• Thrombosis, lschaemia • Pressure, Skin changes

Some useful TIPS 1. Thrombosis: It is one of the common effects of aneurysm particularly aortic and popliteal resulting in ischaemia in the distal territory. 2. Ischaemia: Distal parts may become gangrenous and or can have ischaemic ulcers or claudication. 3. Pressure effects a. Effect on bone: Erosion of the vertebral body as in aortic aneurysm. This does not happen in TB spine. b. Effect on nerves: Popliteal aneurysm can give rise to foot drop due to pressure on lateral popliteal nerve. c. Effect on the veins: Results in congestion and oedema of the leg. d. On the oesophagus: Dysphagia as in aortic aneurysm. 4. Skin changes may be in the form of oedema and redness. Complications of cysts in general l. Infection, e.g. sebaceous cyst 2. Calcification, e.g. haematoma, multinodular goitre with cyst, hydatid cyst 3. Pressure effects: Ovarian cyst pressing on the iliac veins. 4. Haemorrhage within thyroid cyst. 5. Torsion: Ovarian dermoid. 6. Transformation into malignancy. 7. Ovarian cachexia: Large ovarian tumour with pedal oedema, anorexia, loss of weight, lordosis. DERMOID CYST This is a cyst lined by squamous epithelium containing desquamated cells. The contents are thick and sometimes toothpaste-like which is a mixture of sweat, sebum and desquamated epithelial cells and sometimes even hair.

Fig. 16.3: Radial artery aneurysm

Clinical types of dermoid cyst (Key Box 16.5) I. Congenital/sequestration dermoid • They occur along the line of embryonic fusion, due to dermal cells being buried in deeper plane. • The cells which are sequestrated in the subcutaneous plane proliferate and liquefy to form a cyst. • As it grows, it indents the mesoderm (future bone) which explains the bony defects caused by dermoid cyst in the skull or facial bones. • Even though they are congenital, they manifest as a swelling during childhood or later in life. Often they can be mistaken for lipoma and sebaceous cysts (Fig. 16.5). • They can occur anywhere in the midline of the body or the face (Key Box 16.5) l . External and internal angular dermoid cyst: At the fusion lines of frontonasal and maxillary processes (Fig. 16.6). 2. Median nasal dermoid cyst: At the root of the nose at the fusion lines of frontal process (Fig. 16.7). 3. In the suprasternal space of Burns 4. Sublingual dermoid cyst (Fig. 16.8) 5. Preauricular dermoid cyst-in front of the auricle 6. Postauricular dermoid cyst behind the auricle (Fig. 16.9). PEARLS OF

WISDOM

Pinna is formed by the fusion of 6 cutaneous tubercles. Both preauricular and postauricular dermoid cysts occur because of failure of fusion of one of the tubercles with the others as they form pinna.

18$MIWllllllllllllll,1 ORIGIN OF DERMOID CYST

�

• The face is developed from 5 processes-2 maxillary, 2 mandibular and 1 frontonasal process. • Dermoid cyst occurs in the line of embryonic fusion of these processes.

Cystic Swellings, Neck Swellings and Metastasis Lymph Node Neck

Complications of dermoid cyst 1. Infection 2. Suppuration: Abscess 3. Ovarian dem1oid: Torsion Clinical features • Though congenital, the cyst manifests in childhood or during adolescence. A few cases also manifest in 30--40 years age group. • Typically, the patient presents with a painless, slow-growing swelling. • Soft, cystic and fluctuant; transillumination is negative. • Rarely, it may be putty like in consistency. • The underlying bony defect gives the clue to the diagnosis. • Classical location of the cyst (along the line of fusion) is a feature of sequestration dermoid cyst.

Fig. 16.5: Median frontal dermoid cyst-l ipoma, sebaceous cyst are other differential diagnosis

Fig. 16.6: External angu­ lar dermoid cyst-look for bony depression-it is pathognomonic of this swelling

•'

:·\,

'

. ,, lt.;

235

II. Implantation dermoid cyst (Figs 16.1 O and 16.11) • This is common in women, tailors, agriculturists whc sustain repeated minor sharp injuries. • Following a sh arp injury, few epidermal cells get implanted into the subcutaneous plane. There, they develop into an implantation dermoid cyst. Hence, it is typically found in the fingers, palm and sole of the foot. As the cyst develops in the areas where the skin is thick and keratinised, it feels firm to hard in consistency. Ill. Teratomatous dermoid cyst (Fig. 16.12) • Teratoma is a tumour arising from totipotential cells. Thus, it contains ectodermal, endodermal and mesodermal elements-hair, teeth, cartilage, bone, etc. • Common sites are ovary, testis, retroperitoneum and mediastinum.

Fig. 16.7: Median frontal Fig. 16.8: Subl ingual dermoid cyst at the root of dermoid ( Courtesy: Dr the nose.Test tor cough Sreejayan, Professor of impulse Surgery, Calicut Medical College)

Fig.16.9: Post-auricular dermoid cyst. Location and soft consistency are characteristic

.

Fig. 16.10: Implantation dermoid cyst-Classical sites are hand and foot which are prone for sharp injuries

Fig. 16.11: Implantation dermoid cyst-TB synovitis and chronic abscess are the other differential diagnosis

Fig.16.12: Ovarian teratomatous dermoid cyst. Usually they are bilateral-large ones with pe dicles are vulnerable for torsion ( Courtesy: Dr Rajesh Bhakta, Associate Professor, Dept. of OBG, KMC, Manipal)

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Manipal Manual of Surgery

A 24-year-old female patient was admitted with acute lower f abdominal pain o 2 days' duration. There was guarding and f rigidity o the abdominal wall with rebound tenderness. Per vaginal examination was normal. At exploration, there was a twisted ovarian teratoma on the left side with gangrene. It was excised. The opposite ovary, on careful examination revealed a small teratomatous dermoid cyst which could be enucleated. The significance of this case report lies in the fact that both ovaries should be examined in cases of ovarian dermoid cysts.

IV. Tubulo-embryonic dermoid cyst • They arise from ectodermal tubes. A few examples are thyroglossal cyst, post-anal dermoid cyst. • Ependymal cyst of the brain. Treatment of dermoid cyst • Excision of the cyst. EPIDERMAL CYST (WEN) • This is popularly called sebaceous cyst. It is a misnomer. This occurs due to obstruction to one of the sebaceous ducts, resulting in accumulation of sebaceous material. Hence, this is an example of retention cyst. • Sites: Scalp, face, back, scrotum, etc. It does not occur in palm and sole, where sebaceous glands are absent. In the back, scalp and scrotum (Figs 16.15 to 16.19), multiple cysts are often found. Clinical features • They are slow-growing and appear in early adulthood or middle age.

Fig. 16.13: Cut open specimen of epidermoid cyst showing cheesy material

• Hemispherical or spherical swelling located in the dermis. A dark spot in the centre (punctum) filled with keratin is a diagnostic feature of this cyst. The punctum indicates blockage of the duct (Fig. 16.14). • In 20-30% of cases, instead of opening into the skin, sebaceous duct opens into the hair follicle. Hence, punctum is not seen. • It has a smooth surface, round borders, soft and putty consistency and is nontender. • The cyst can be moulded into different shapes which is described as sign of moulding. • Sign of indentation refers to pitting on pressure over the swelling (Table 16.1). • The swelling is mobile over the deep structures, and the skin is free all around except an area of adherence at the site of punctum. • In the scalp, loss of hair is a feature over the swelling because of constant slow expansion of the cyst. Treatment • Incision and avulsion of cyst with the wall. Very often, during dissection, the cyst wall ruptures. Care should be taken to excise the entire cyst wall. If not, recurrence can occur. • When it is small it can be excised along with the skin. Complications 1. Infection can occur due to injury or scratch resulting in an abscess. The cyst will be tender, red and warm to touch. It should be treated like an abscess by incision and drainage. After one to two months, the cyst can be excised. 2. Sebaceous horn results due to slow drying of the contents which are squeezed out, specially if a patient does not wash the part. Thus, it is not common to find a large sebaceous horn nowadays because of better ways of living and sanitation (Fig. 16.20).

Fig. 16.14: Sebaceous punctum is diagnostic of sebaceous cyst

Fig. 16.15: Multiple sebaceous cysts on the back-troublesome situation

Cystic Swellings, Neck Swellings and Metastasis Lymph Node Neck

Fig. 16.16: Multiple seba­ ceous cysts on the scrotum­ one of the common sites

Fig. 16.18: Strawberry scrotum ( Courtesy: Dr Umesh Bhat, Surgeon, Kundapur, Karnataka, India)

Fig. 16.17: Multiple seba­ ceous cysts on the scrotum­ calcified. Requires excision

237

Fig. 16.19: Sebaceous cyst on the scalp-loss of hair is a feature. When punctum is absent, the differentiated diagnosis is lipoma

Comparison of congenital dermoid cyst and sebaceous cyst

• Aetiology • • • • • • •

Location Sign of indentation, moulding Punctum Skin fixation Bony defect lntracranial communication Treatment

Congenital dermoid cyst

Epidermal cyst

Congenital-sequestration of the dermal cells in the subcutaneous plane Midline of the body, along the line of fusion Uncommon Absent Absent Present in majority of cases Rare, can be diagnosed by cough impulse test Excision

Acquired-retention cyst due to accumulation of sebaceous contents Face, scalp, scrotum, back Very common Present in 50% of cases-diagnostic Skin is fixed at the site of punctum Absent Absent Excision or avulsion

3. Calcification 4. Cock's peculiar tumour 1 refers to infected, ulcerated cyst of scalp with pouting granulation tissue and everted edge resembling epithelioma (Key Box 16.6). 5. Rarely, basal cell carcinoma can arise in a long-standing sebaceous cyst.

............................. KEY BOX 16.6

• • • •

INTERESTING-SEBACEOUS CYST Syndrome: Gardner's syndrome Tumour: Cock's peculiar tumour Parasitic worm: Demodex folliculorum Strawberry scrotum: Multiple sebaceous cysts of scrotum

.

Fig. 16.20: Sebaceous horn

1 Pott's puffy tumour refers to osteomyelitis of the frontal bone with oedema of scalp secondary to frontal sinusitis. This and Cock's peculiar tumour are favourite viva questions.

Manipal Manual of Surgery

238 PEARLS OF

WISDOM

Meibomian cyst: They are epidermal cysts found on the free edge of eyelid. Chronic Meibomian cyst is called chalazion. GANGLION It is a tense, cystic swelling and occurs due to myxomatous degeneration of the synovial sheath lining the joint or tendon sheath. They are common around joints because of abundant fibrous tissue. They contain gelatinous fluid. Common sites • The dorsum of the hand is the common site, at the scapholunate articulation. • In the foot, dorsal or lateral aspect. • Small ganglion in relation to flexor aspect of fingers. Clinical features • Majority of patients are between 20 and 50 years. • A round to oval swelling in the dorsum of the hand, with smooth surface and round borders. Skin over the swelling is normal. • The swelling is tensely cystic and fluctuant. Trans­ illumination is negative. It is mobile in the transverse direction. • W hen the tendons are put into contraction, the mobility of the swelling gets restricted. • Ganglion is not connected with the joint space. Sometimes, it gives an impression of becoming small due to slipping away between bones. Treatment 1. Asymptomatic ganglion is better left alone. 2. Aspiration of the ganglion and injection of sclerosants may reduce the size of ganglion. 3. Sometimes, rupture of the cyst due to trauma may result in permanent cure. 4. Surgical excision can be done. However, recurrence rate is high. Differential diagnosis (DD) 1. Implantation dermoid cyst, when it occurs in the feet or hand. 2. Exostosis of the bone, has to be considered if swelling is very hard. 3. Bursa (vide infra) COMPOUND PALMAR GANGLION Aetiology • Tuberculous tenosynovitis of the tendon sheaths affecting the flexor tendons. This is a common cause in India (Key Box 16.7)

• Rheumatoid arthritis with involvement of multiple joints causing thickening of synovial membrane-common cause in Western countries. Pathology • As a result of tuberculous tenosynovitis, typical caseous material collects within the flexor tendon sheaths. The tendons get matted, a swelling develops in the palm and another swelling develops in lower aspect of forearm. The thickening of synovial membrane, fibrin particles in the fluid and melon seeds, are characteristic of this condition. Clinical features • Majority of patients are below 40 years of age. • Concavity of the palm is obliterated. • Soft, cystic, fluctuant, transillumination-negative swelling situated above and below the flexor retinaculum. • Cross fluctuation test between these two swellings is positive, which is diagnostic of compound palmar ganglion. • Restricted mobility of the fingers due to matting of the tendons. • Wasting of the small muscles of the hand. • Paraesthesia due to compression on median nerve. Investigations I. The ESR may be increased if it is due to tuberculosis. 2. Aspiration of the swelling and fluid can be sent for acidfast bacilli. 3. Synovial biopsy. Treatment 1. Antituberculous treatment {ATT) in case of tubercular pathology. If the response rate is not satisfactory­ exploration, decompression, synovectomy and release of matted tendons is the treatment. 2. Control of rheumatoid arthritis, with complete excision of the synovial sheath, in cases due to rheumatoid arthritis. Summary of compound palmar ganglion • Tuberculosis and rheumatoid arthritis-common causes. • Synovial thickening will clinch the diagnosis. • Cross fluctuation test is an important clinical finding. • Antituberculous treatment if it is due to tuberculosis. • Decompression or synovectomy may be required in both conditions mentioned above. GLOMUS TUMOUR (Key Box 16.7) • This is also called glomangioma or angioneuromyoma. • Glomus is a specialised organ. Structure of glomus (glomus body) Abundant arteriovenous anastomosis surrounded by large clear cells (glomus cells) and medullated and non-medullated nerve fibres in between the cells is characteristic of glomus.

Cystic Swellings, Neck Swellings and Metastasis Lymph Node Neck

Clinical features of glomus tumour (Figs 16.21 and 16.22) 1. Typical site: Under the nail beds of hands and feet. 2. It is purple red in colour, usually single, the size does not exceed 1 cm in diameter. 3. Glomus tumour is usually seen in the 5th decade. 4. Excruciating pain either at rest or on movement of the finger or on pressure is pathognomonic feature of this tumour. Pain is due to compression of the nerve fibres by dilated glomus vessels. 5. The tumour is compressible.

.239

IM&MCWWllir,1 GLOMUS TUMOUR � • Rare and benign tumour • The most painful tumour • The smallest benign tumour, does not turn malignant

• Nail bed is the commonest site • Histologically, it is an angioneuromyoma • It is radioresistant

• Excision gives permanent cure • Function of glomus is concerned with heat regulation.

Treatment • Surgical excision results in permanent cure. Differential diagnosis 1. Subungual melanoma: Painless and pigmented 2. Granuloma pyogenicum: Mild pain, bleeds on touch and evidence of infection is present. 3. Chronic infection with granuloma. BURSA • Bursa means a sac or a sac-like cavity containing fluid lined by endothelium. It is meant to reduce the friction between tendons of the muscle and the bone. • Bursitis refers to inflammation of a bursa resulting in accumulation of excessive fluid inside the bursa. This results in a swelling in the anatomical sites of normal bursa. • The causes of chronic bursitis includes constant pressure, constant irritation or minor injuries. • Some examples of bursitis are given in Table 16.2. Figs 16.21 and 16.22: Glomus tumour: The most painful condition in the finger ( Courtesy: Prof Bhaskarananda Kumar and Dr Anil Bhat, Department of Orthopaedics, KMC, Manipal)

Clinical features • A cystic swelling in a known anatomical site of a bursa is a chronic bursitis unless proved otherwise.

Bursae and bursitis Anatomical site

Popular nomenclature

I . Prepatellar bursa

Housemaid's knee

2. 1n front of patella tendon (infrapatellar)

Clergyman's knee

3. Olecranon bursa

Student's elbow

4. Under the insertion of tendons of sartorius, gracilis and semiten­ dinosus muscle

Bursa anserina ( extension of the bursa along the sides of tendon­ resembles goose's foot)

5. Between the tendon of the semimembranosus and the medial condyle of tibia

Semimembranosus bursitis

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• Bursitis produces a soft, cystic, circumscribed or oval swelling with fluctuation. • As majority of bursitis contains inflammatory fluid, they do not show transillumination. • In a few cases, signs of inflammation may be present. Complications 1. Secondary infection may result in an abscess 2. Frequent friction may result in ulceration 3. Cosmetic deformity. Treatment • Excision is indicated only in the presence of symptoms such as pain or complications mentioned above. • Chances of recurrence are high. SEMIMEMBRANOSUS BURSA (Figs 16.23 to 16.25) This is the commonest swelling in the popliteal space. It presents as a tensely cystic swelling �11------;.:J--Semimembranosus when the knee is extended bursa and it becomes flaccid on flexion of the knee. It is not compressible as it does not communicate with thejoint. The differential diag­ nosis for semimembrano­ sus bursitis is Morrant­ Baker's cyst, which is a herniation of the synovial membrane. The differences between these two swellings Fig. 16.23: Popliteal fossa swelling are given in Table 16.3. Adventitious bursae • This refers to a cyst which develops in an anatomical area where no bursa is present. These also occur due to constant pressure or friction. They are summarised below. 1. Tailor's ankle: Above the lateral malleolus

Fig. 16.24: Constant pressure on the lateral aspect of foot resulting in bursa due to the habit of 'squatting position'. Second picture showing the bursa with pigmentation all around

Figs 16.25A and B: (A) Semimembranosus cyst and (B) cyst disappears on flexing

Comparison of semimembranosus bursa and Baker's cyst (Fig. 16.23)

I. 2. 3. 4. 5.

Aetiology Age Location in the popliteal fossa On flexion of the knee On extension of the knee 6. Patellar tap 7. Compressibility 8. Knee movements

Semimembranosus bursa

Baker's cyst

Friction or pressure Young patients Higher up and more medial

Rheumatoid or osteoarthrosis of knee joint

Disappears Appears and is tense (Figs 16.25A and 8) Absent Absent Normal

Middle aged Below and midline Increases Diminishes Present Present partially Restricted

Cystic Swellings, Neck Swellings and Metastasis Lymph Node Neck

2. Porter's shoulder: Between clavicle and skin 3. Weaver's bottom: Between gluteus maximus and ischial tuberosity 4. Bunion: Between prominent head of the first metatarsal and skin due to hallux valgus • The complications and treatment of adventitious bursae are similar to chronic bursitis (Fig. 16.24) TRANSILLUMINANT SWELLINGS IN THE BODY These are the cystic swellings containing clear fluid characterised by fluctuation and transillumination. 1. Lymphangioma 2. Ranula 3. Meningocoele 4. Epididymal cyst 5. Vaginal hydrocoele. L YMPHANGIOMA (Figs 16.26 to 16.29) • Failure of one of the lymphatics to join the major lymph sac of the body results in a lymphangioma. Hence, it occurs in places where lymphatics are abundant. • They are dilated lymphatics that project onto the skin surface. • Common sites: Posterior triangle of the neck, axilla, mediastinum, groin, etc. (Key Box 16.8). • In the neck, it is called cystic hygroma of the neck. As the sac has no communication with lymphatics by the time swelling appears, the lymph is absorbed and is replaced by thin watery fluid (mucus) secreted by endothelium. Hence, it is also called hydrocoele of the neck. • When it is largely confined to subcutaneous plane, it is called cystic hygroma.

Fig.16.26: Lymphangioma involving chest wall, neck. The two were different swellings

241

............................. . KEY BOX 16.8

LYMPHANGIOMA • Jugular lymph sac • Posterior lymph sac • Cisterna chyli

SITES Neck

Groin Retroperitoneum

Types of lymphangioma 1. Lymphangioma circumscriptum: If it is less than 5 cm across. 2. Lymphangioma diffusum: If they are more widespread. 3. Lymphoedema ab igne: If they form a reticulate pattern of ridges. Clinical features • Usually, cystic hygroma presents during infancy or early childhood. Occasionally, present since birth and rarely before birth. They can also present as small vesicles. • When the child cries or strains the swelling increases in size and becomes prominent due to increased intra­ thoracic pressure which is transmitted through root of the neck. • Typical locations-lateral aspect of neck (posterior triangle), groin, buttocks. • Soft, cystic, fluctuant, partially compressible swelling. Lymphangioma is a multilocular swelling consisting of aggregation of multiple cysts. These cysts may inter­ communicate and may occasionally insinuate between muscle planes. Hence, it gives the sign of compressibility! However, complete reducibility is not a feature. • The swelling is brilliantly transilluminant because it contains clear fluid (watery lymph) (Key Box 16.9).

Fig. 16.27: Lymphangioma involving chest wall. Brilliantly transilluminant

Fig.16.28: Lymphangioma involving neck and axilla-cross fluctuation was positive

Manipal Manual of Surgery

242

............................. . KEY BOX 16.9

TRANSILLUMINATION TEST • Should be done in a dark room • Avoid surface transillumination • Transillumination may be negative because of infection, sclerotherapy and haemorrhage

Treatment • Surgical excision is the treatment of choice. All the loculi or cysts should be removed. Careful search has to be made for the extension of lymphangioma through the muscle planes so as to avoid recurrence (Figs 16.29 and 16.30). Sclerotherapy was being used earlier for lymphangioma. Since, tissue planes are distorted by sclerosants, dissection becomes difficult. Thus, injection type of treatment is not favoured at present.

2. Lipoma: This is a soft lobular swelling with fluctuation because fat behaves I ike fluid at body temperature. However, the edge slips under the palpating fingers. Both trans­ illumination and compressibility tests are negative with lipoma. 3. Cold abscess ( details follow in a later page). Complications 1. In neonates and infants, lymphangioma can cause difficulty in breathing due to its large size. 2. Occasionally, secondary infection can occur. 3. Lymphangioma in the mediastinum can give rise to dyspnoea, dysphagia due to compression on the trachea/ oesophagus. RANULA Ranula is a cystic swelling arising from sublingual salivary gland and from accessory salivary glands which are present in the floor of the mouth called glands of Blandin and Nuhn. The word ranula is derived from the resemblance of the swelling to the belly of frog-Rana hexadactyla. Aetiology I. Ranula occurs due to obstruction to the ducts secreting mucus. Hence, it is an example for retention cyst. 2. Some surgeons consider it as an extravasation cyst.

Fig. 16.29: Lymphangioma twice excised-residual lesion. It can be left alone if it is asymptomatic

Clinical features • Seen in young children and adults. • The swelling is typically located in the floor of the mouth or under surface of the tongue, to one side of the midline (Fig. 16.31). • Soft, cystic, fluctuant swelling, which gives brilliant transillumination. • It is covered by thin mucosa containing clear, serous fluid. Hence, it is bluish in colour (Fig. 16.32).

Fig. 16.30: Lymphangioma-multiple pockets at surgery-all those have to be removed to prevent local recurrence

Differential diagnosis 1. Haemangioma: Posterior triangle of the neck is one of the

common sites for haemangioma. Haemangioma is soft, cystic and fluctuant but transillumination is negative and the sign of compressibility is positive.

Fig. 16.31: Ranula in the floor of the mouth

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243

Differential diagnosis • Sublingual dermoid cyst is a thick-walled cyst, whitish in colour and not transilluminant. • Mucus cyst Complications I. Rupture of the cyst decreases the size but it can reappear at a later date. 2. When the swelling is big, the tongue is pushed upwards and may cause difficulty in speech or swallowing. MENINGOCOELE

Fig. 16.32: Ranula-observe the bluish colour

• Surface is smooth, borders are diffuse, nontender swelling. • Plunging ranula: It is an intraoral ranula with cervical extension, where it passes on the side ofmylohyoid muscle and produces a swelling in the submandibular region. Thus, one swelling in the floor of the mouth and the other in the neck gives rise to plunging ranula. The diagnosis 1s confinned by cross-fluchiation test (Key Box 16.10).

mm••�,, CROSS-FLUCTUATION TEST

�

• Indicated when a cyst has two interconnected components • Gentle pressure on one component, impulse felt on the other component • Demonstrated by bidigital palpation • Plunging ranula, compound palmar ganglion, iliopsoas abscess, hydrocoele en bisac will give a cross-fluctuation test. Treatment 1. Complete excision of the ranula is the treatment of choice in plunging ranula. Since the cyst wall is very thin, it should be carefully dissected and removed. 2 Marsupialisation is indicated in simple ranula. The ranula is incised and the wall of the cyst is sutured to the mucosa of the floor of the mouth, so as to leave an opening to the exterior (Marsupials, e.g. kangaroo). • After 5-10 days, the cyst gets collapsed, fibrosis occurs and the entire cavity gets obliterated. • Marsupialisation avoids surgical dissection and chances of injury to the submandibular duct. • Plunging ranula can be excised by intraoral approach. Once the intraoral dissection is completed the cervical extension can be mobilised by the same incision dissecting close to the cyst wall. However, rupture and chances of leaving behind a portion of the cyst wall are high.

Meningocoele is a herniation of the meninges through a weak point in the spine (neural arch) where the bony fusion has not taken place effectively (Fig. 16.33 and Key Box 16.11). The swelling is covered by pia mater and arachnoid mater without a dural covering. The swelling contains cerebrospinal fluid (CSF). Meningocoele is an example of spina bifida cystica. Clinical features • The swelling is present since birth. Soft, cystic, fluctuant with brilliant transillumination are the typical features of the swelling. • Sign of compressibility is present due to displacement of CSF. • When the child cries or coughs, an expansile impulse is present. • On palpating the edge of the swelling, a bony defect is usually found.

............................. . KEY BOX 16.11

MENINGOCOELE: SITES

• Lumbosacral: The commonest • Occipitocervical: Second common • Root of the nose: Rare

Fig. 16.33: Meningocoele

244

Manipal Manual of Surgery SPINA BIFIDA OCCULTA

Fig. 16.34: Encephalocoele ( Courtesy: Prof Vijaykumar, Paediatric Surgeon, KMC, Manipal)

Treatment (Key Box 16.12) • CT scan is done to look for hydrocephalus. If it is present, a ventriculoperitoneal shunt is done which will reduce the meningocoele. • Excision of the rneningocoele should be done as early as possible to prevent the rupture and secondary infection. Complications • Skin covering the swelling is very thin and so is prone to ulceration. Due to ulceration, secondary infection and rneningoencephalitis can occur. • Haemorrhage.

............................. . KEY BOX 16.12

EXCISION OF MENINGOCOELE

• In this condition, the neural arch is defective posteriori: There is no visible swelling. • It can be suspected when there is a tuft of hair, lip01rn naevus, pigmented patch of skin overlying the lumbosacn region. • Child is normal at birth. Neurological symptoms such a weakness, sciatica-like pain may start appearing at pubert (neurogenic talipes equinus-club foot). • During this time because of growth there may be tractio1 on the spinal cord by a ligament called membrane reunien.s • X-ray can demonstrate the bifid spine. • Surgical excision of the membrane gives permanent curi to the patient, if there are symptoms. Types of spina bifida cystica 1. Menigocoele } 2. Meningomyelocoele See Table 16.4 for companson. • Protrusion of meninges, with nerve root of spinal cord or disordered spinal cord results in meningo­ myelocoele. • Neurological deficit such as foot drop, talipes, trophic ulcer of the foot (S I root) may be present. • Surgical excision may be followed by residual neurological deficit. 3. Syringomeningomyelocoele • In this condition, in addition to the meninges, the central canal of spinal cord is also herniated out. • Most of the children are stillborn. • Very difficult to treat, if the child survives. Encephalocoele (Fig. 16.34): It is also known as cranium bifidum. It is a neural tube defect characterised by sac-like protrusion of brain and meninges through an opening in the skull.

• Surgery: As early as possible after birth

DIFFERENTIAL DIAGNOSIS OF MIDLINE SWELLING IN THE NECK (see page 246)

• Early closure prevents infection • Transverse elliptical incision • Excision of the sac

Midline swellings: From above downwards I. Ludwig's angina 2. Enlarged submental lymph nodes

• Closure of the defect by plication • Approximation of the muscles

Comparison of meningocoele and menigomyelocoele Meningocoele

Meningomyelocoele

Contents

Membranes

Membranes with nerve roots

Consistency

Soft and cystic

Soft to firm

Transillumination

Brilliant

Partially transilluminant

Longitudinal furrow

Absent

Present due to adherence of the nerve roots to the skin

Neurological deficit

Absent

Trophic ulcers, bladder and bowel incontinence, locomotor prob­ lems are present

Prognosis after repair

Good

Residual neurological deficit is present

Cystic Swellings, Neck Swellings and Metastasis Lymph Node Neck

3. 4. 5. 6. 7. 8. 9. 10.

Sublingual dermoid cyst Subhyoid bursitis Thyroglossal cyst Enlarged isthmus of thyroid gland Pretracheal and prelaryngeal lymph nodes Retrosternal goitre Thymic swelling Swelling in the suprastemal space of Bums: Lipoma/ cold abscess/aneurysm. LUDWIG'S ANGINA

• This is an inflammatory oedema of the floor of the mouth. It spreads to the submandibular region and submental region. • Tense, tender, brawny, oedematous swelling in the submental region with puh·id halitosis is characteristic of this condition. ENLARGED SUBMENTAL LYMPH NODES The three important causes of enlargement: 1. Tuberculosis: Matted submental nodes, firm in consistency, with enlarged upper deep cervical lymph nodes, with or without evening rise of temperature are suggestive of tuberculosis. 2. Non-Hodgkin's lymphoma can present with submental nodes along with other lymph nodes in the horizontal group of nodes such as submandibular, upper deep cervical, pre­ auricular, post-auricular and occipital lymph nodes (external Waldeyer's ring). Nodes are firm or rubbery, discrete without matting. 3. Secondaries in the submental lymph nodes can arise from carcinoma of the tip of the tongue, floor of the mouth, central portion of the lower lip. The nodes are hard in consistency and sometimes, fixed. SUBLINGUAL DERMOID CYST • It is a type of sequestration dermoid cyst which occurs due to sequestration of the surface-ectode1m at the site of fusion of the two mandibular arches. Hence, such a cyst occurs in the midline, in the floor of the mouth (Key Box 16.13). • When they arise from 2nd branchial cleft, they are found lateral to the midline. Hence, lateral variety. • The cyst is lined by squamous epithelium and contains hair follicles, sebaceous glands and sweat glands. It does not contain hair. Clinical features l . Young children or patients between the age of IO and 20 years present with painless swelling in the floor of mouth.

245

M$M1,-,,1 SUBLINGUAL DERMOID CYST

• • • • •

�

Origin: At the site of fusion of 2nd branchial arches Site: Midline-common; Lateral-uncommon Supraomohyoid variety is common Bidigital palpation for demonstration of fluctuation Soft, cystic, fluctuant, transillumination negative swelling

Differential diagnosis • Ranula: Transillumination is positive • Thyroglossal cyst: Moves with deglutition

2. Swelling is soft and cystic. Fluctuation test is positive. Bidigital palpation gives a better idea about fluctuation with one finger over the swelling in the oral cavity and the other finger in the submental region. 3. Transillumination test is negative as it contains thick, cheesy, sebaceous material. Differential diagnosis 1. Ranula: When a sublingual dermoid cyst is in the mid­ line in the floor of the mouth and above the mylohyoid muscle, ranula is considered as differential diagnosis. However, ranula is bluish in colour, brilliantly trans­ illuminant. 2. Thyroglossal cyst should be considered as differential diagnosis when the sublingual dermoid cyst is below the mylohyoid muscle. Thyroglossal cyst moves up with deglutition whereas a sublingual dermoid cyst does not. Treatment • Through intraoral approach, excision can be done for both types of sublingual dermoid cyst. SUBHYOID BURSITIS • Accumulation of inflammatory fluid in the subhyoid bursa results in a swelling and is described as subhyoid bursitis. • The bursa is located below the hyoid bone and in front of thyrohyoid membrane. Clinical features • The swelling is in front of the neck, in the midline below the hyoid bone (Fig. 16.43). • The swelling is oval in the transverse direction. • It moves up with deglutition. • Soft, cystic, fluctuant and transillumination negative swelling (turbid fluid). • The swelling may be tender as it contains inflammatory fluid.

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246

A FEW MIDLINE SWELLINGS IN THE NECK (Figs 16.35 to 16.42)

Fig. 16.35: Submental lymphadenitis: Caused by tuberculosis-not a common site

Fig. 16.36: Submental swelling of 30 years duration ( Courtesy: Prof. KK Rajan, Calicut Medical College)

Fig. 16.38: Incompletely treated thyroglossal cyst. Recurred, about to rupture and form thyroglossal fistula

Fig. 16.39: Thyroid nodule: common swelling-moves with deglutition

2008: MS Examination short case, KMC, Manipal ( Courtesy: Professor BH Ananda Rao, Department of Surgery) Fig. 16.41: Cold abscess in the suprasternal space of burns: One of the common sites of cold abscess

Fig. 16.37: lntraoral examination of patient (Fig. 16.36) reveals swelling also in the floor of mouth-sublingual dermoid cyst

Fig. 16.40: Pretracheal lymph nodes and upper neck nodes: Case of non-Hodgkin's lymphoma

2008: MS Examination short case, Govt. Medical College, Goa ( Courtesy: Prof Dilip Amonkar, HOD, Dept. of Surgery) Fig. 16.42: Aneurysm of innominate artery. Atherosclerosis is the cause. Before incising a cold abscess in this location, make sure that it is not an aneurysm

247

Cystic Swellings, Neck Swellings and Metastasis Lymph Node Neck

Treatment Complete excision

Q>---

- Foramen caecum

Complication It can develop into an abscess Differential diagnosis 1. Thyroglossal cyst is a vertically placed oval swelling, whereas subhyoid bursitis is transversely placed oval swelling (Fig. 16.44).

0

• Thyroglossal cyst moves on protrusion of the tongue outside (subhyoid bursitis does not). 2. Pretracheal lymph node swelling. 3. Ectopic thyroid enlargement.

Pyramidal lobe I I I I

�Thyroglossal l;}-�yst I I I I I I

Fig. 16.43: Subhyoid bursitis: Transversely placed oval swelling

THYROGLOSSAL CYST

Fig. 16.45: Anomalies/fate of thyroglossal tract

• This is an example for tubulo­ embryonic dermoid cyst. • It arises from thyroglossal tract/ duct which extends from foramen caecum at the base of the tongue to the isthmus of the thyroid gland. Hence, the thyro-glossal cyst can develop anywhere along this duct. For anomalies are shown in Fig. Fig. 16.44: Thyroglossal 16.45. cyst: Vertically placed oval • It is lined by pseudostrati-fied, swelling ciliated, columnar or squamous epithelium which produces desquamated epithelial cells or mucus at times. Sites of thyroglossal cyst (Fig. 16.46) 1. Subhyoid: The most common type 2. At the level of thyroid cartilage: 2nd common site 3. Suprahyoid: Double chin appearance 4. At the foramen caecum: Rare 5. At the level of cricoid cartilage: Rare 6. In the floor of the mouth

Fig. 16.46: Sites of thyroglossal cyst (numbers see text)

Clinical features • Even though congenital, thyroglossal cyst appears around the age of 15-30 years. • They are more common in females who present with painless, midline swelling. However, in the region of thyroid cartilage, the swelling is slightly deviated to the left side.

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Manipal Manual of Surgery

• The cyst is soft, cystic, fluctuant, transillumination­ negative swelling (very rarely, it can give rise to transillumination). It can be firm if the tension within the cyst is high. • Mobility: Thyroglossal cysts ex._hibit 3 types of mobility which are characteristic of this condition: - The cyst moves with deglutition. - Moves with protrusion of the tongue (Fig. 16.47 and Key Box 16.14): Hold the thyroglossal cyst with the finger and thumb and ask the patient to protrude the tongue outside. The movement of the cyst upwards is described as a tug because of its attachment with the hyoid bone. - The swelling moves sideways but not vertically as it is tethered by the thyroglossal duct. Treatment • Before excision of cyst, a thyroid scan is mandatory since it may be the only functioning thyroid tissue. • Sistrunk operation: Excision of the cyst along with the entire thyroglossal tract which may include part of the hyoid bone, is the recommended treatment. The intimate relationship of hyoid bone can be explained by its development from 2nd and 3rd branchial arches. Complication 1. Recurrent infection: The wall of the thyroglossal cyst sometimes contains lymphoid tissue which can get infected, resulting in an abscess. If it ruptures or is incised it results in thyroglossal fistula (Key Box 16.15). 2. Rarely, a papillary carcinoma can occur in the thyroglossal cyst (see page 313). 3. Fistula.

Fig. 16.47: Thyroglossal cyst: It moves upwards with protrusion of the tongue

............................. KEY BOX 16.14

MOVEMENT ON PROTRUSION OF THE TONGUE • The cyst is attached to the hyoid bone. Hence, it gives a classical TUG. • Not always present, cyst below the thyroid cartilage-tug is absent. • Better appreciated on holding the swelling. Examination of thyroglossal cyst • Cyst proper, mobility • Base of the tongue to rule out lingual thyroid and lymph nodes. PEARLS

OF

WISDOM

Do not forget to feel base of the tongue for lingual thyroid/ectopic thyroid. Whenever a patient has one congenital anomaly, examine thoroughly for 'more' associated anomalies.

THYROGLOSSAL FISTULA Thyroglossal fistula is never congenital. It is always acquired due to the following reasons (Fig. 16.48 and Key Box 16.16): I. Infected thyroglossal cyst rupturing into the skin. 2. Inadequately drained infected thyroglossal cyst. 3. Incompletely excised thyroglossal cyst. • The track is lined by columnar epithelium. Clinical features • Previous history of swelling in front of the neck, which is now painful, red and ruptured resulting in discharging pus. Once the pus is drained, the opening closes. However, after an interval of time, the 'pain and discharge' reappear.

Fig. 16.48: Thyroglossal fistula

Cystic Swellings, Neck Swellings and Metastasis Lymph Node Neck

............................. KEY BOX 16.15

• • • • • • •

.

KEY BOX 16.16

• • • •

............................. . KEY BOX 16.17

SISTRUNK'S OPERATION

RECURRENT ABSCESS: RUPTURE FISTULA OR SINUS Thyroglossal fistula Osteomyelitis Stitch abscess Pilonidal sinus Median mental sinus Cold abscess Umbilical sinus

............................. . THYROGLOSSAL FISTULA Always acquired Fistulous opening is in the midline Semilunar sign or hood sign It gets pulled up with protrusion of the tongue

• When there is no infection, the fistula discharges only mucus and the surrounding skin is normal. Infected fistulae are tender, discharging pus and the skin is red hot. • Majority of the patients presenting are young in the age group of IO to 20 years. • A fistulous opening in the centre of neck which is covered by a hood of skin can occur due to increased growth of the neck, when compared to that of fistula. This is described as semilunar sign or hood sign. Treatment • Infection is controlled with antibiotics. • Surgical excision should include the fistula with removal of the entire tract up to the foramen caecum. Otherwise, recurrence will occur. • The central portion of the hyoid bone is removed due to close proximity of the fistula. • An elliptical incision is preferred as it gives a neat scar. • This operation is called Sistrunk's operation. See Key Box 16.17 for details about Sistrunk's operation. ANOMALIES OF THYROGLOSSAL DUCT • Thyroglossal duct extends from foramen caecum to thyroid cartilage. • Various anomalies have been given in Key Box 16.18. • However, thyroglossal cyst is common. Lingual thyroid and ectopic thyroid tissue are uncommon swellings.

249

• Fistula with entire thyroglossal tract is excised. • Central portion of the hyoid bone and lingual muscle are removed. • Removal is facilitated by pressing the posterior 113rd of the tongue. • Do not perforate thyrohyoid membrane. • Incomplete removal results in recurrence.

.

............................ .

KEY BOX 16.18

THYROGLOSSALDUCT ANOMALIES • • • •

Lingual thyroid Levator glandulae thyroidae Ectopic thyroid tissue Thyroglossal cyst

• They have to be kept in mind as a differential diagnosis of the swellings in the midline of the neck. SWELLING ARISING FROM ISTHMUS OF THE THYROID GLAND Almost all the diseases of the thyroid gland result in enlargement of the isthmus. However, a solitary nodule and cysts can occur in relation to isthmus. The swelling moves with deglutition. However, it does not move on protrusion of the tongue.

PRETRACHEAL AND PRELARYNGEAL LYMPH NODES These lymph nodes produce nodular swelling in the midline. One or two discrete nodes are palpable. They can enlarge due to the following conditions: 1. Acute laryngitis: The nodes are tender, soft. 2. Papillary carcinoma of thyroid: The nodes are firm without matting, with or without evidence of thyroid nodule. 3. Carcinoma of the larynx: The nodes are hard in consis­ tency. 4. Jn India, tuberculosis should be considered as a possible diagnosis when other diseases are ruled out.

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SWELLINGS IN THE SUPRASTERNAL SPACE OF BURNS 1. Lipoma: Soft and lobular, edge slips under the palpating finger. 2. Sequestration dermoid cyst is a midline, soft, cystic, fluctuant swelling. 3. Gumma produces a firm swelling with evidence of syphilis else­ where in the body. 4. Thymic swellings, an aneurysm of innominate or subclavian artery are the other causes (Fig. 16.49).

Anterior triangle l. 2. 3. 4.

Submental triangle Digastric (submandibular) triangle Carotid triangle Muscular triangle

Posterior triangle 1. Occipital triangle 2. Supraclavicular triangle SWELLINGS IN SUBMANDIBULAR TRIANGLE Fig. 16.49: Aneurysm

A lady of 65 years presented with swelling in the suprasternal space (Fig. 16.92). Candidate gave the diagnosis of lymph node swelling-probably cold abscess. He failed. It had expansi/e pulsations. It was a case of aneurysm of innominate artery. MS. exam case 2008, JNMC, Belgaum (Courtesy: Professor Ashok Godhi, HOD of Surgery)

DIFFERENTIAL DIAGNOSIS OF LATERAL SWELLINGS IN THE NECK Before we discuss the swellings in the lateral side of the neck, it is essential to know the various triangles in the neck. These are discussed below.

• The submandibular triangle is a pa1t of anterior triangle. • This is bounded inferiorly by anterior and posterior bell: of digastric muscles with their tendon, superiorly by th< attachment of deep fascia to the whole length of mandible • This triangle is covered by deep fascia. • The floor is formed by mylohyoid muscle which arisei from mylohyoid line of the mandible, thus closing tht space. • Swellings in the submandibular triangle are (Fig. 16.51): 1. Enlarged submandibular lymph nodes-common 2. Submandibular salivary gland enlargement-common 3. Plunging ranula-not uncommon 4. Ludwig's angina-not uncommon 5. Lateral sublingual dermoid cyst-rare 6. Tumours of the mandible-rare

TRIANGLES OF THE NECK Each side of the neck is a quadrilateral space subdivided by sternocleidomastoid into anterior triangle and posterior triangle. They are further subdivided as given below (Fig. 16.50).

Fig. 16.50: Triangles of the neck

Fig. 16.51: This swelling in the submandibular triangle was bidigitally not palpable. However, it exhibited a doubtful sign of movement with deglutition. It turned out to be an ectopic thyroid swelling (Courtesy: Prof Sampath Kumar, Dept. of Surgery, KMC, Manipal)

Cystic Swellings, Neck Swellings and Metastasis Lymph Node Neck

ENLARGED SUBMANDIBULAR LYMPH NODES

They fonn a nodular swelling which is deep to deep fascia. They are palpable only in the neck (not intraorally). The nodes can get enlarged due to the following conditions: 1. Acute lymphadenitis: Very often, poor oral hygiene or a

caries tooth produces painful, tender, soft enlargement of these lymph nodes. Extraction of the tooth or with improvement of oral hygiene, lymph nodes regress.

2. Chronic tuberculous lymphadenitis can affect these nodes along with upper deep cervical nodes. The nodes are finn and matted. 3. Secondaries in the submandibular lymph nodes arise from carcinoma of the cheek, tongue, palate. The nodes are hard with or without fixity. 4. Non-Hodgkin's lymphoma can involve submandibular lymph nodes along with horizontal group of nodes in the neck. The nodes are firm or rubbery in consistency. SUBMANDIBULAR SALIVAR Y GLAND ENLARGEMENT (Key Box 16.19)

The various causes of submandibular salivary gland enlarge­ ment have been discussed under the salivary gland chapter. The common causes are chronic sialadenitis with or without a stone, tumours of the salivary gland or enlargement due to autoimmune diseases. They form irregular or nodular swelling. The diagnosis is confirmed by bidigital palpation of the gland. Enlarged submandibular gland is bidigitally palpable because the deep lobe is deep to mylohyoid muscle.

251

Some important swellings in this triangle are as follows: 1. Branchial cyst 2. Lymph node swelling (cold abscess) 3. Aneurysm of carotid aitery. 4. Enlargement of the thyroid gland 5. Carotid body tumour- rare Carotid 6. Laryngocoele-rare triangle 7. Stemomastoid tumour-rare Fig. 16.52: Carotid triangle 8. Neurofibroma of the vagus BRANCHIAL C YST Aetiology

• Branchial cyst arises from vestigeal remnants of 2nd branchial arch. • The cyst is lined by squamous epithelium and contains desquamated epithelial cells which slowly forms a tooth paste-like material.

Clinical features

• Even though congenital, majority of patients are young between the age group 15 and 25 years. • The swelling is typically located in the anterior triangle of the neck partly under cover of the upper 113rd of anterior border of sternomastoid. This can be explained because of the development of sternomastoid muscle from the myotome in the ridge of second bronchial arch (Fig. 16.53).

MMMM,_,� • Calculus

SUBMANDIBULAR SALIVARY GLAND ENLARGEMENT

• Chronic sialoadenitis • Cancer • Chronic diseases: Autoimmune

J '

DIFFERENTIAL DIAGNOSIS OF SWELLINGS IN THE CAROTID TRIANGLE

The carotid triangle has following boundaries (Fig. 16.52): Laterally by sternomastoid muscle, superomedially by digastric muscle and stylohyoid muscle and inferomedially by omohyoid muscle.

....

__ _..., � ,

Fig. 16.53: Branchial cyst-typical location-partly covered by sternocleidomastoid muscle. Differential diagnosis for this swelling will be cold abscess

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Manipal Manual of Surgery

• The swelling has smooth surface and round borders. It is soft, cystic, fluctuant and transi1Jumination-negative 1. The consistency is that of a rubber bag half filled with water. The swelling is very often firm due to thick inspissated content. In such situations, it is very difficult to elicit fluctuation. The mobility of the swelling is also restricted because of its adherence to the sternomastoid muscle. • Sternomastoid contraction test: The swelling becomes less prominent. • If contents are aspirated, it contains cholesterol crystals (Key Box 16.20). • No other lesion is found in the neck (lymph nodes).

.

............................ .

KEY BOX 16.20

SWELLINGS CONTAINING CHOLESTEROL CRYSTALS • • • •

Branchial cyst Dental cyst Dentigerous cyst Hydrocoele

Treatment • Excision of the cyst along with its entire epithelial lining with a curved incision centred over the swelling. One must ensure that epithelial lining should be removed completely or else recurrence will occur. • Sometimes cyst may grow backwards in between 'fork' of common carotid artery as far as pharyngeal constrictors. Complications Since the wall is rich in lymphatic tissue, it can undergo secondary infection with pain and swelling. Hence, the swelling has to be excised (Fig. 16.54). Differential diagnosis (Fig. 16.55) There is no differential diagnosis in a classical case of branchial cyst. However, a few swellings have to be considered as differential diagnosis. l. Cold abscess occurs in young patients due to tuber­ culosis ofjugulodigastric nodes. Presence of multiple lymph nodes in the neck with or without fever gives clue to the diagnosis. 2. Lymphangioma is a brilliantly transilluminant, partially compressible swelling. However, anterior triangle is not a common site for lymphangioma. 3. Lipoma can also occur in the neck, though it is an uncommon site (Fig. 16.54).

Fig. 16.54: Swelling mimicked branchial cyst. However, it was , subcutaneous swelling with lobularity-it turned out to be lipom, ( Courtesy: Dr P Rajan, Calicut Medical College, Calicut, Kerala)

Swelling upper neck: Anterior triangle Cystic (fluctuation positive)

Solid (fluctuation negative)

Transillumination

Metastatic nodes-hard Lymphoma-firm Tuberculosis-matted nodes

Positive 1-----1 Lymphangioma ruled out Slip sign negative Lipoma ruled out

Lymphangioma Compressibility negative

1-------1 Haemangioma ruled out Classical location

1. Branchial cyst • Young age group 20-30 years • Smooth, soft, nontender • Deep to sternomastoid

2. Cold abscess • Young age • Smooth, soft, nontender • Deep to sternomastoid • Lymph nodes (matting)

Fig. 16.55: Branchial cyst-one example of diagnosing a case using clinical methods. It is important that each clinical method elicited carefully, gives a clue to the diagnosis

1 Branchial and thyroglossal cysts rarely give rise to transillumination if contents are clear and unless the cyst is small, it is difficult to elicit fluctuation. These cysts can be firm or hard swellings also.

Cystic Swellings, Neck Swellings and Metastasis Lymph Node Neck

BRANCHIAL FISTULA (Key Box 16.21) • This is always congenital and occurs due to persistent 2nd branchial cleft. 1 • External opening is situated at the junction of middle 113rd and lower 113rd of sternomastoid (Fig. 16.56). • The tract from the skin passes through the fork of common carotid artery deep to the accessory and hypoglossal nerve and opens in the anterior aspect of posterior pillars of tonsils (Fig. 16.56). The tract is lined by ciliated squamous epithelium and discharges a mucopurulent discharge. Sometimes, the upper end is blind resulting in a SlllUS.

• The patient may complain of a dimple, discharging mucus and the dimple becomes more obvious when the patient is asked to swallow. • Usually seen in growing adults (30% of cases). • Can be unilateral or bilateral, equally common in males and females. • It is also called lateral fistula of the neck. (Thyroglossal fistula is called median fistula of the neck.)

253

Treatment • Fistulogram can be done by injecting methylene blue into the external opening and defining the tract (Fig. 16.57). This is followed by exploration of the tract. At surgery, it should be carefully dissected up to the internal opening and then excised. May have to be done by two different incisions: Upper incision at upper border of thyroid cartilage and lower incision encircling fistula and dissecting upwards. Complication Recunent infection of the fistula. COLD ABSCESS DUE TO TUBERCULOSIS • ln India, this is the commonest cystic swelling in the carotid triangle. The cold abscess occurs as a result of caseation necrosis of the lymph nodes. This forms a soft, cystic, fluctuant swelling with negative transillumination. Presence of other lymph nodes in the neck or sinuses in the neck gives the clue to the diagnosis. • Loss of appetite, weakness and fever with chills may be other features. ANEURYSM OF THE COMMON CAROTID ARTERY • Atherosclerosis is the most common cause of aneurysm. This weakens the vessel walls uniformly and produces fusiform dilatation of the blood vessel. Hypertension is another factor which adds to the aneurysm. • Abdominal aorta is the commonest site for aneurysms followed by popliteal artery. Types A. Fusiform: Atherosclerosis, hypertension (Fig. 16.58A). B. Saccular: Due to injury (Fig. 16.588).

Fig. 16.56: Sites of branchial fistula-commonly bilateral

Fig. 16.57: Contrast study

demonstrating fistulous tract

C. False: In this condition there is a sac lined by cellular tissue which communicates with the aitery through an opening in its wall (Fig. 16.58C).

M$MfJWIIIIIIF,1 • • • • •

BRANCHIAL FISTULA PASSES SUPERFICIAL ·� TO FOLLOWING STRUCTURES Internal carotid artery Internal jugular vein Derivatives Hypoglossal nerve J of 3rd arch Glossopharyngeal nerve Stylopharyngeous muscle Pierces superior constrictor muscle and opens on posterior pillar of the fauces behind tonsil.

1 Persistent first branchial cleft results in external auditory meatus.

Figs 16.58A to C: Fusiform, saccular, false aneurysm

254

Manipal Manual of Surgery

A

F

B

Figs 16.59A to F: Different methods of operation for aneurysm. (A) Excision and end-to-end graft, (B) Excision and end-to-side graft,

(C) Excision and side-to-side graft, (D) Excision and bypass grafting, (E and F) Matas aneurysmorrhaphy Causes (Key Box 16.22)

............................. . KEY BOX 16.22

• • • •

ANEURYSM:CAUSES

Congenital: Berry aneurysm in the circle of Willis Traumatic Degenerative: Atherosclerosis Rare causes:

Syphilis: Endarteritis obliterans Mycotic: Infective emboli Subacute bacterial endocarditis Marfan's syndrome Polyarteritis

Clinical features of aneurysm 1. Elderly patients are commonly affected. 2. Evidence of atherosclerosis in the form of thick walled vessel is present. 3. Tensely cystic (feels firm), fluctuant, transillumination negative swelling with expansile pulsation (when the fingers are kept over the aneurysm they are not only elevated but they are separated). 4. Compressibility is positive 5. On exerting pressure proximally the swelling diminishes in size-classically it happens in a case of popliteal aneurysms on compression of the femoral artery. 6. Bruit/thrill is characteristic of this condition. PEARLS

OF

CAROTID BODY TUMOUR (CHEMODECTOMA) Introduction • This is a benign tumour arising from chemoreceptors in the carotid body (Key Box 16.23). They are situated in the tunica adventitia at the bifurcation of common carotid artery (Fig. 16.60). • Hence, such a tumour is called chemodectoma. • Function of the carotid body is regulation of pH. • It may be associated with phaeochromocytoma. • Chronic hypoxia can lead to carotid body hyperplasia. Hence, there is a higher incidence of chemodectoma in people living at higher altitudes. Clinical features (Key Box 16.24) • Middle-aged or elderly patients are affected (5th decade). • The patient gives long history of painless, slow-growing swelling for many years. • Typical location: In the upper part of the anterior triangle of the neck, at the level of the hyoid bone, beneath the anterior edge of the stemomastoid muscle (Fig. 16.61). • Surface is smooth or lobulated, borders are round, and is an oval, vertically placed swelling. Consistency is firm to hard. Hence, called classical potato tumour.

WISDOM

Classical signs of an aneurysm described above may be absent, if thrombus is present within it.

Treatment of aneurysm • Angiography to confirm the diagnosis followed by repair of aneurysm with graft-PTFE graft (polytetrafluoro­ ethylene graft, Fig. 16.59).

Fig. 16.60: Location of

carotid body

Fig.16.61: Carotid body tumour­

classical site

Cystic Swellings, Neck Swellings and Metastasis Lymph Node Neck

............................. . KEY BOX 16.23

J

-

255

CHEMORECEPTORS: SITES • • • • •

The carotid body The aortic body Brainstem Pulmonary receptors Myocardial receptors

Hormonally not active cells

• Homer's syndrome and unilateral vocal cord paralysis can occur due to involvement ofthe nerves. • Pressure on the tumour gives rise to syncopal attack due to decrease in the pulse rate (carotid body syndrome) • Moves in the transverse direction. • Carotid artery is stretched over the swelling and so, transmitted pulsations are felt (Fig. 16.62). • Intra-oral examination shows prolapse ofipsilateral tonsil, unless it grows in parapharyngeal space. Diagnosis • Carotid angiography (Fig. 16.63A) should be done ifthere are neurological symptoms such as syncopal attack. rt may demonstrate separation of the carotid bifurcation. • Lyre sign: Splaying of carotid artery can be seen (Fig. 16.63B) • Incision biopsy is dangerous • Colour Doppler should be the first investigation. Treatment • Excision of the tumour with reconstruction • No role for radiotherapy.

Figs 16.63A and B: Carotid angiography-observe separation of internal and external carotid artery by the tumour (A) and vascular blush (B)

............................. . KEY BOX 16.24

• • • • • • •

CAROTID BODY TUMOUR

Rare tumour Rarely malignant Rarely bilateral Rarely grows fast Rarely patient presence early Rarely metastasises Experience of a general surgeon with this tumour is very, very rare.

PEARLS OF

WISDOM

It is important to preserve cerebral circulation during surgery. Vascular surgeon's help is necessary.

Complications • Very rarely, it can turn into a malignant carotid body tumour with lymph nodal metastasis. Precautions • Do not biopsy from within mouth-carotid body tumour can displace tonsil medially. • Should not do FNAC • Should not do open biopsy • When you feel some pulsations over a lymph node in the carotid triangle-remember carotid body tumour. STERNOMASTOID TUMOUR • This is not a tumour, it is a misnomer. • Injury to the sternomastoid during birth causes rupture of few fibres and haematoma. Later, healing occurs with fibrosis, resulting in a swelling in the middle ofsternomastoid muscle. • The other possible theory is that this is a congenital anomaly-short sternomastoid muscle.

Fig. 16.62: Carotid body tumour in a 45-year-old lady-duration 5 years (Courtesy: Dr MR Srivatsa, Prof and Head, Prof Bagali Babasaheb, Prof Bharathi, Dr Srikar Pai, Department of Surgery, MS Ramaiah Medical College and Hospital, Bangalore)

Clinical features • This is seen in infants or children. Firm to hard, 1-2 cm swelling in the middle ofthe sternomastoid muscle.

Manipal Manual of Surgery

256

• Tender and mobile sideways. Medial and lateral borders are distinct but superior and inferior borders are continuous with the muscle. • Many cases are associated with torticollis. Treatment • Gentle manipulation of child's head • Physiotherapy to stretch the shortened sternomastoid muscle. • Division of lower attachment of sternomastoid from clavicle and sternum with or without removal of lump is the surgical treatment. LARYNGOCOELE (Key Box 16.25) • ft occurs due to herniation of the laryngeal mucosa (Fig. 16.64, external laryngocoele) • When it enlarges within the larynx, it may displace vocal cord, produce hoarseness and is called internal la ryngo­ coele. Causes • Glass blowers, musicians, wind instruments and trumpet players are commonly affected. • Chronic cough may be one of the predisposing factors. Clinical features • Smooth, oval, boggy swelling which moves upwards on swallowing, in relation to thyrohyoid membrane (subhyoid position). • Swelling becomes prominent when the patient is asked to cough or blow (Valsalva manoeuvre).

Thyrohyoid ----Hr-7s:--­

membrane

• Expansile cough impulse is present. • Tympanitic note on percussion (resonant) Treatment • Excision of the sac-in external laryngocoele • Marsupialisation-in internal laryngocoele. Differential diagnosis Other cystic swellings such as branchial cyst and lymphangioma should be ruled out. Complications Secondary infection results in laryngopyocoele. The opening in the thyrohyoid membrane may be blocked by mucopus in such cases. PHARYNGEAL POUCH • Herniation or protrusion of mucosa of the pharyngeal wall through Ki IIian's dehiscence. • Killia n's dehiscence is a potential area of weakness in between the two parts of the inferior constrictor muscle (Fig. 16.65): (A) Upper oblique fibres (thyropharyngeus) and (B) Lower horizontal fibres (cricopharyngeus). Aetiopathogenesis Due to increase in the intra- Fig. 16.65: Pharyngeal pharyngeal pressure, mucous pouch membrane bulges in between parts of inferior constrictor muscles due to neuromuscular imbalance. Hence, it is a pulsion diverticulum. Course of the diverticulum Pulsion diverticulum deviates to one side mostly to the left because of the rigid vertebral column in the midline posteriorly.

Fig. 16.64: Laryngocoele

M¥81*'-W',1 • • • •

LARYNGOCOELE

�

Very rare Increased laryngeal pressure Expansile impulse on cough Treatment: Ligation of its neck and division of the whole sac.

Diagnosis • Initially foreign body sensation is present in the throat. Later, gurgling sound regurgitation of food on turning to one side, sense of suffocation, cough or dysphagia is present. • Aspiration may cause dyspnoea later. PEARLS OF

WISDOM

Pharyngeal pouch is a swelling behind sternocleido­ mastoid below the level of thyroid cartilage-soft swelling which can be emptied.

Cystic Swellings, Neck Swellings and Metastasis Lymph Node Neck

Treatment • Barium swallow followed by excision of the pouch • Cricopharyngeal myotomy may also be done. SCHWANNOMA OF THE VAGUS NERVE (Fig. 16.66) • This condition produces swelling in the carotid triangle in the region of thyroid swelling. • It is a vertically placed oval swelling • It is firm to hard in consistency • On pressure over the swelling, dry cough and in some cases bradycardia may occur.

.............................

257

KEY BOX 16.26

.

BOUNDARIES OF POSTERIOR TRIANGLE

• • • •

Anteriorly Laterally Above Below

Sternomastoid (posterior border) Trapezius (anterior border) Mastoid process Clavicle

Most ofthe swellings have been discussed under appropriate chapters. Haemangioma, metastasis in the cervical lymph nodes and Pancoast's tumour have been discussed below. Classification (Table 16.5) Common swellings in the posterior triangle are given below. HAEMANGIOMA Definition This is a swelling due to congenital malformation of blood vessels. It is an example of Hamartoma.

Fig. 16.66: Schwannoma of the vagus nerve ( Courtesy: Prof P Rajan, Calicut Medical College, Kerala. This swelling was not moving with deglutition-Candidate offered thyroid nodule as diagnosis-MS exam case 2007)

DIFFERENTIAL DIAGNOSIS OF SWELLING IN THE POSTERIOR TRIANGLE The posterior triangle (Key Box 16.26) is an interesting area as far as swellings are concerned. It is the commonest area of metastasis in lymph nodes from occult prima1y. Lymphangiomas, haemangiomas, cold abscess, lymphomas commonly occur here. Interesting cases of cervical rib, Pancoast's tumour, aneurysms also occur here.

Classification A. Depending on the origin • Capillary • Cavernous • Arterial B. Depending on behaviour of the lesion Terms in common use Strawberry naevus I. Involuting haemangioma • Superficial Capillary haemangioma • Deep Cavernous • Combined (superficial+ deep) Strawberry naevus Capillary haemangioma Cavernous haemangioma II. Noninvoluting • Portwine stain Portwine stain Capillary haemangioma Naevus tlammeus • Cavernous haemangioma • Arteriovenous fistula

Swellings in the posterior triangle I. Solid swellings

II. Cystic swellings

III. Pulsatile swellings

l.

Metastasis in the lymph nodes

Lymphangioma

Subclavian artery aneurysm

2.

Tuberculosis

Haemangioma

Vertebral a11ery aneurysm

Lymphoma

Cold abscess

3.

4. Lipoma 5. Cervical rib 6. Pancoast's tumour

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Manipal Manual of Surgery

CAPILLARY HAEMANGIOMA (Key Box 16.27) They consist of dilated capillaries and proliferation of endothelial cells. Hence, it commonly occurs in the skin (Figs 16.67 and 16.68). They can be of the following types: 1. Salmon patch is a bluish patch over the forehead, in the

midline, present at birth and disappears by 1 year of age. Hence, no treatment is required.

2. Port-wine stain is an extensive intradennal haemangioma. This is bluish purple in colour, commonly affects the face or other parts of the skin, is present at birth and usually progresses and does not regress (Key Box 16.28). • It is a noninvoluting capillary haemangioma (dilatation due to defective maturation of cutaneous innervations during embryogenesis). • Area supplied by sensory branches of the fifth cranial nerve is involved. • Start with light red colour and progress to deep colour. • Pulsed dye laser using light with specific wavelength of 585 or 595 nanometres is one of the best treatments available. This process is called as 'photothermolysis'. • It may be associated with Sturge-Weber syndrome (page 259) PEARLS

OF

WISDOM

Flat,patchy lesion on the/ace in the area offifth cranial nerve that does not fade is port-wine stain.

............................. . KEY BOX 16.27

• • • • •

CAPILLARY HAEMANGIOMA Skin and soft tissue involvement Salmon patch: Midline-forehead Port-wine stain: Head and neck Strawberry angioma: Compressible Wait and watch policy is the best.

3. Strawberry angiomas produce swelling which protrud, from the skin surface. The child is nonnal at birth. After; month, a bright red swelling appears over the head am neck region, which exhibits sign of compressibility. Th< lesion consists of immature vascular tissue. Even thougl the lesion grows initially, by 5-7 years of age, swellin� regresses and colour fades. Hence, no specific treatmen is necessary. The treatment is indicated only when th( swelling persists. 70% resolve by 7 years of age. VENOUS (CAVERNOUS) HAEMANGIOMA This occurs in place where venous space is abundant, e.g. lip. cheek, tongue, and posterior triangle of the neck (Fig. 16.65 and Key Box 16.29). Clinical features • History of a swelling in the neck of long duration. History of bleeding is present when it occurs in the oral cavity. • The swelling is warm and bluish in colour but not pulsatile. • Soft, fluctuant, transillumination is negative. • Compressibility is present. This sign is also called 'sign of emptying' or 'sign of refilling'. When the swelling is compressed between the fingers, blood diffuses under the vascular spaces and when pressure is released, it slowly fills up. Compressibility is a diagnostic sign of haemangioma. Differential diagnosis 1. Lymphangioma is brilliantly transilluminant. If a lymphangioma is infected or has been treated with preliminary injections, it may not show transillumination. 2. Lipoma is not compressible. 3. Cold abscess 4. Branchial cyst when it is in anterior triangle.

............................. . KEY BOX 16.28

PORT-WINE STAIN • Port-wine colour even though to start with it is red in colour • Occurs usually in the face, can also occur on the shoulder and trunk • Regression does not occur • Treatment: Pulsed dye laser, photocoagulation, derma­ brasion • Worrying because it becomes more keratotic and nodular as age advances • Injection of sclerosants may be needed • Noninvoluting haemangioma is its another name • Extensive intradermal capillary dilatation Remember as PORT-WINE

Fig. 16.67: Capillary haemangioma ( Courtesy: Prof Pramod Kumar, HOD of Plastic Surgery, KMC, Manipal)

Cystic Swellings, Neck Swellings and Metastasis Lymph Node Neck

M=!t • • • •

CAVERNOUS HAEMANGIOMA

259

,1 M¥MC,-w',1 �

SWELLINGS: TREATED WITH SCLEROSANTS

�

• Haemangioma

Compressible swelling Bluish warm, nontender swelling Associated with arteriovenous communication Associated with lipoma: Naevolipoma

• Haemorrhoids • Prolapse rectum • Oesophageal varices • Varicose veins

Gluteal region is one of the sites of haemangioma, AVfistula, aneurysm and neuri/emmoma. A patient presented with this swelling which wasfirm with a few cystic areas. It was excised in toto. Remember to check whether it is a low pressure (venous) or a high pressure arterial lesion before excising a haemangioma. Massive bleeding can occur if it is a high pressure lesion.

Syndromes associated with haemangioma (Table 16.6, Figs 16.76 and 16.77) Complications of haemangioma (Key Box 16.32)

Treatment of cavernous haemangioma Principles 1. Injection is the first line of treatment of cavernous haemangioma. It makes the swelling fibrotic, less vascular and small. Thus, excision can be done at a later date (Key Boxes 16.30 and 16.31). 2. Excision of haemangioma in the oral cavity is more difficult than in the neck. 3. It is better to have a control of external carotid artery in the neck, while excising haemangiorna in the oral cavity. If necessary, external carotid artery should be ligated in order to control the bleeding. 4. Adequate blood to be arranged. 5. Previous embolisation into the feeding artery decreases the size of the haemangioma (therapeutic embolisation).

18¥Mi�,1 INJECTION LINE OF TREATMENT

6. Large haemangiomas in the oral cavity should be excised only after preliminary sclerotherapy and taking all the precautions mentioned above.

�

• Boiling water, hypertonic saline or sodium tetradecyl sulphate (STD solution) can be used. • In multiple spaces, in multiple sittings. • Obliteration occurs due to aseptic thrombosis and fibrosis. • Lesion becomes flat.

M¥Mi�,1 COMPLICATIONS OF HAEMANGIOMA

�

• Ulceration and bleeding: Commonly occurs with capillary haemangioma • Infection: Septicaemia usually precipitated by a small ulcer • High output cardiac failure

CONGENITAL ARTERIOVENOUS (AV) FISTULA (Arterial Haemangioma) • An abnormal communication between artery and vein, results in AV fistula(Key Box 16.33). • AVfistula can be congenital or acquired(Figs 16.73, 16.79 and 16.80). • Such AV fistula has got structural and functional effects (Figs 16.78A and B) Structural effect • Since high pressure blood from an artery flows into the vein, the veins get dilated, tortuous and elongated. This arterialisation of the vein results in secondary varicose veins.

Syndromes associated with haemangioma and its findings Syndromes associated with haemangioma

Findings

1. Klippel-Trenaunay-Weber syndrome

I. Naevus flammeus, osteohypertrophy of extremities, AV fistula and varicose veins

2. Osler-Rendu-Weber syndrome

2. Haemangioma of lip associated with haemangioma of GIT

3. Sturge-Weber syndrome

3. Haemangioma of brain, mental retardation, Jacksonian epilepsy, glaucoma

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Fig. 16.68: Capillary haemangioma of the nose

Fig. 16.69: Cavernous haemangioma of the cheek

Fig. 16.71: Sign of compressibility was positive in this case. It was initially diagnosed as post-auricular dermoid cyst Note: Importance of complete clinical examination

Fig. 16.74: AV malformation being excised

Fig. 16.70: Local gigantism due t, haemangioma involving gluteal region

Fig. 16.72: Haemangioma tongue causing macroglossia. She also had haemangioma of the cheek and skin

Fig.16.73: AV malformation since birth and growing since 3 years. The patient had bleeding due to trivial trauma. Local rise of tempera­ ture and machinery murmur was present

Fig. 16.75: Sclerosants were injected initially to reduce vascularity. This was followed by wide excision

Cystic Swellings, Neck Swellings and Metastasis Lymph Node Neck

Figs 16.76 and 16.77: Large haemangioma involving cheek, lip and palate. He also had haemangioma of the liver

Fig. 16.79: Most common type of AV fistula you see today is in the nephrology ward-created to facilitate haemodialysis

Fig. 16.82: Congenital AV fistula of 25 years duration

Fig. 16.78A: Traumatic AV fistula. Seethe arteria­ lisation of the vein

Fig. 16.80: X-ray of the hand­ traumatic AV fistula

261

Fig. 16.788: DSA picture

Fig.16.81: TraumaticAVfistula affecting wrist and hand. Kindly observe prominent veins and swollen fingers

Fig. 16.83: After therapeautic embolisation it has regressed by over 70%. Patient is waiting for another course of embolisation. ( Courtesy: Dr Umesh Bhat, Surgeon, Kundapur and Dr Subhaschandra, lnterventional cardiologist, Manipal Hospital, Bangalore)

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KEY BOX 16.33

ARTERIOVENOUS FISTULA: TYPES • Congenital • Traumatic • Iatrogenic: Created in cases of renal failure

Physiological effect • Increased pulse rate, increased cardiac output, increased pulse pressure result due to increased venous pressure and arteriovenous shunt. Functional effect • Soft, cystic, fluctuant, transillumination-negative, pulsatile swelling. • A continuous bruit/murmur is characteristic (Fig. 16.73) • Nicoladoni's sign or Branham's sign - On compressing the feeding artery, the venous return to the heart diminishes, resulting in fall in pulse rate and pulse pressure. - On compressing feeding artery, pulsation or continuous murmur may also disappear and swelling will diminish in size. • If the AV fistula is big, a high output cardiac failure can occur. • The affected part is swollen (because of high pressure) than-local gigantism (Figs 16. 70 and 16. 72). Thus, overgrowth of the limb or toe can occur. • Distal to the AV fistula, there are ischaemic ulcers, due to comparative reduction in the blood supply.

This lady visited many general practitioners for a swelling near the coccyx region (adjacent to natal cleft). The diagnosis which was made in this case was subcutaneous neurofibroma, pilonida/ sinus abscess, boil and haemangioma. Careful examinations revealed pulsations. History dates back to 20 years duration. It was a case of congenital AVfistula. Wide excision was done. She has been asymptomatic for 2 years now. [Courtesy: Dr B Hartimat, Asst. Prof of Surgery, KMC, Manipal (Figs 16.74 and 16.75)}.

................

CLINICAL NOTES A 30-year-old patient who had an injury to the dorsum of hand, had pain and swelling in dorsum of hand for 60 days duration. Many had missed the diagnosis. However, swelling had local rise of temperature, pulsations and reducibility (Fig.

16.81).

Fig. 16.84: Traumatic AVM at surgery

Investigations • Angiography with DSA pictures (digital subtraction angiography) are essential before treating these patients (Fig. 16. 78B). Treatment • Therapeutic embolisation is the treatment of choice for arteriovenous fistula, in congenital cases (Figs 16.82 and 16.83). • Acquired lesion need to be observed or treated by quadruple ligation (Fig. 16.84). CIRSOID ANEURYSM • Not an aneurysm • lt is an AV fistula occurring in older people affecting the temporal region. • The arteries and the veins are dilated and tortuous and are compared to pulsating bag of worms. COLD ABSCESS IN THE POSTERIOR TRIANGLE (Fig. 16.85) Causes I. Posterior c ervical lymph nodes pri­ marily involved­ route of infection from adenoids or other lymph nodes in the anterior triangle. 2. Lower posterior lymph n o d e s o r Sc a l e n e 1 node-route of infec­ Fig. 16.85: Cold abscess in the tion from lungs. posterior triangle

Cystic Swellings, Neck Swellings and Metastasis Lymph Node Neck

3. From tuberculous cervical spine: Caries spine • Clinically it presents as pain in the back, cold abscess and neurological presentation. • Rust's sign: Child with caries spine will support the head by holding the chin. • Cold abscess from caries spine can rupture anteriorly or posteriorly. A. Anterior rupture: It ruptures deep to prevertebral layer of deep cervical fascia. From here, it can take the following routes: • Upper cervical region: Presents as deep seated abscess in the posterior wall of the pharynx in the midline. • Lower cervical region: Pus will press on oesophagus and trachea forwards. • Laterally pus passes deep to prevertebral fascia behind carotid sheath in the posterior triangle (Fig. 16.86). B. Posterior rupture • Pus may enter spinal canal and then can travel along anterior primary division of the cervical spinal nerves. Diagnosis • Cervical spine X-ray to rule out spinal tuberculosis. • Chest X-ray to rule out pulmonary tuberculosis. • Nondependent aspiration of the cold abscess followed by AFB staining. Treatment • Antituberculous treatment • Nondependent aspiration if cold abscess is present. • Please refer orthopaedic books for specific treatment of TB spme. Differential diagnosis • Haemangioma-compressible • Lymphangioma-transilluminant • Schwannoma (Fig. 16.86)

-

263

Fig. 16.86: Schwannoma in the posterior triangle. Soft tissue sarcoma is another diagnosis. Undergraduate clinics at JNMC, Belgaum, Karnataka ( Courtesy: Dr Aditya Patil, Prof. Ashok Godhi, HOD of Surgery)

SECONDARIES IN THE LYMPH NODES OR LYMPH NODE METASTASIS IN HEAD AND NECK Introduction Very often, the patients present to the surgeon with lymph node swelling in the neck with or without any complaints. If there is an obvious lesion in the oral cavity, the diagnosis is easy. On the other hand, difficulty arises in locating the primary malignancy, which is hidden or occult. It is important to know the anatomical location of the lymph nodes in the neck and drainage area, so that drainage areas can be investigated. Classification • They can be divided into horizontal (Table 16.7) and vertical groups of lymph nodes. Interestingly, very often circular group of nodes are enlarged in non-Hodgkin's lymphoma on both sides.

Horizontal group of lymph nodes Location 1. Submental 2. Submandibular

3. Parotid 4. Preauricular 5. Postauricular 6. Occipital

Drainage area

Common diseases

Central part of the lower lip, floor of the mouth, tip of the tongue Cheek, tongue, whole of the upper lip, outer part of the lower lip, gums, angle of mouth, side of nose, inner angle of the eye Nasopharynx, back of nose, eyelids, front of scalp, enamel, auditory meatus, tympanic cavity Outer surface of pinna, side of scalp Temporal region of scalp, back of pinna Back of the scalp

Infections, metastasis Infections, carcinoma Eyelid tumours, parotid tumours, tuberculosis Scalp infection, tuberculosis Scalp infection Scalp infection, secondary syphilis

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VERTICAL GROUP OF LYMPH NODES (Fig. 16.87) They can be classified into central and lateral group of lymph nodes. Most of the central group of lymph nodes are in relation to laryngeal cartilages or the trachea. The lateral lymph nodes are the most popular lymph nodes called deep cervical nodes. Majority of them are in close relationship with internal jugular vein. Jugulo-omohyoid and jugulodigastric lymph nodes belong to this category. The details of the vertical group of lymph nodes are given in Table 16.8.

3'>----.a----ll=-i ��-t-tJ

Memorial Sloan-Kettering cancer centre: Latera lymph node classification Lymph nodes in the submental triangle anc submandibular triangle. Level II Upper jugular nodes from posterior belly o digastric superiorly to hyoid bone inferiorly. Level III Middle jugular nodes from hyoid superiorly tc cricothyroid membrane inferiorly. Level IV Lower jugular nodes from cricothyroic membrane superiorly to clavicle inferiorly. Posterior cervical region from anterior borde1 Level V of trapezius posteriorly to posterior border o1 sternocleidomastoid anteriorly and clavicle inferiorly. Level VI Anterior compartment nodes from hyoid bone superiorly to suprasternal notch inferiorly and laterally by medial border of carotid sheath. Level VII: Upper mediastinal nodes inferior to suprastemal notch (no longer used). • It is advisable to know these various levels of lymph nodes and their drainage areas. • When block dissection is being done, you will find the lymph nodes being mentioned in the fotm of levels. Factors which accelerate local and regional spread of malignant tumour (Key Box 16.34). Level I

,.:,,.,___,,,

6�-------'� z�-----a..rw.

FACTORS WHICH ACCELERATE LOCAL AND REGIONAL SPREAD OF MALIGNANT TUMOUR

Fig. 16.87: Vertical group of lymph nodes (see Table 16.8)

• • • •

�

Angiogenesis Lymphangiogenesis Lack of basement membrane on lymphatic vessels Extracapsular breakout

Vertical group of lymph nodes Location

Drainage area

Common diseases

1. Prelaryngeal (on the cricothyroid ligament)

Larynx

Laryngeal carcinoma

2. Pretracheal, paratracheal (in front of trachea)

Trachea, thyroid

Papillary carcinoma thyroid, tuberculosis

3. Upper anterior deep (jugulodigastric)

Tonsil, posterior 1/3 tongue, oropharynx, pyriform recess

Tonsillitis, carcinoma posterior 1/3 tongue, oropha1yngeal carcinoma, tuberculosis

4. Upper posterior deep

Adenoids, posterior pharynx, retropharyngeal region Thyroid, supraglottis

Tuberculosis, nasopharyngeal carcinoma

6. Lower anterior (jugulo-omohyoid)

Tongue, thyroid, subglottis

Papillary carcinoma thyroid Carcinoma tongue, carcinoma thyroid

7. Lower posterior (supraclavicular)

Thyroid, postcricoid, oesophagus, lungs breast

Bronchogenic carcinoma, intra-abdominal ma­ lignancy, lymphoma

5. Middle group of lymph nodes

Note: True vocal cords are devoid of lymphatics.

Cystic Swellings, Neck Swellings and Metastasis Lymph Node Neck

• Example: In papillary carcinoma thyroid, level III, level IV, level V and level VI lymph nodes are removed (see next page). Clinical presentation of metastatic deposits in the lymph nodes 1. Majority of patients are elderly males(> 50 years), present with painless swelling in the neck of a few months duration. 2. The symptoms with which a patient presents to the hospital gives the clue to the site of origin of the primary. Few examples are given below: • Difficulty in swallowing: Carcinoma posterior 1/3 tongue, oropharyngeal carcinoma or carcinoma oesophagus • Difficulty in breathing: Laryngeal cancer • Hoarseness of voice: Larynx or thyroid • Obvious growth in oral cavity: Carcinoma cheek, alveolus, tongue, etc. • Haemoptysis, difficulty in breathing: Bronchogenic carcinoma • Epistaxis, ear pain or deafness: Nasopharyngeal carcinoma(Key Box 16.35) Clinical signs • Lymph nodal metastasis appears as a hard, nodular or irregular mass in the anatomical location of the lymph nodes. • Early cases may have some mobility. However, in majority of cases nodes get fixed and they attain a huge size. Very often, what appears as one lymph node, is a complex mass of multiple lymph nodes. • On stemomastoid contraction test or chin test, these nodal swellings become less prominent. • Skin ulceration is a late feature. A prominent skin fold is due to infiltration into the platysma-platysma sign. • The primary malignancy may be evident in the anterior third of tongue, cheek, alveolus, etc. • Posterior one-third of the tongue should be palpated with gloved finger (Key Box 16.36).

M$M1,-,,1 NASOPHARYNGEAL CARCINOMA 1:

�

TROTIER'S TRIAD

• Conductive deafness • Homolateral immobility of soft palate • Pain in the side of the head due to involvement of 5th cranial nerve

265

M$m1,-,� OCCULT PRIMARY SITES

• Posterior 113rd of the tongue, oropharynx • Nasopharynx, sinuses • Upper oesophagus, bronchus, thyroid • Secondaries in the lymph nodes can cause pressure effects or may cause paralysis of nerves. Upper anterior deep cervical lymph nodes can cause hypoglossal nerve paralysis (Key Box 16.3 7) where in the tongue points towards the side of lesion. When there is no evidence of the primary lesion clinically, the situation is described as occult primary with secondaries in the neck. • Pain in the distribution of trigeminal nerve (face) suggests nasopharyngeal malignancy infiltrating skull base(foramen lacerum). How do you suspect metastasis in the neck? Any elderly patient presenting to the hospital with finn to hard lymph node in the neck of short duration with or without fixity. He has no signs and symptoms of inflammation such as fever or pain to begin with. Having suspected a metastatic deposit, remember the following facts: • 80% of them are metastatic deposits. • Majority of malignant neoplasms are epithelial in ong111. • Nodes in the upper half (Level I and II) can be due to primary in the oral cavity, tongue, oropharynx, larynx. • Nodes in the lower half(Level JTl and IV) can be due to primary in the thyroid, tongue. • Nodes in the supraclavicular region(Level V): Carcinoma in the GIT, genitourinary tract, lungs and nasopharynx. • Nodes in the pretracheal, suprasternal region(Level VI): Papillary carcinoma thyroid.

•t•l,-,,1 CLINICAL EXAMINATION IN A CASE OF LYMPH NODES IN THE NECK

• Lymph nodes: All groups • Drainage areas Pressure effects on:

• Hypoglossal nerve • Accessory nerve • Cervical sympathetic chain

1 More details about nasopharngeal carcinoma are given in page 291 in the chapter on oral cavity.

�

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METASTASIS IN CERVICAL LYMPH NODES-VARIOUS LEVELS (Figs 16.88 to 16.96)

Fig. 16.88: Level I and II nodes. Carcinoma floor of the mouth

Fig. 16.91: Level V nodes: Post­ cricoid carcinoma

Fig. 16.94: Level I, II, Ill and V nodes: Primary could not be identified-a case of occult primary

Fig. 16.89: Level II and Ill nodes: Carcinoma alveolus

Fig. 16.92: Predominantly Level II, Ill and V nodes: From nasopharyngeal carcinoma

Fig. 16.95: Scalene node: In between the two heads of sternomastoid-a case of bronchogenic carcinoma

Fig. 16.90: Level Ill, IV and V nodes Carcinoma posterior 1 /3rd of tongue

Fig. 16.93: Predominantly Level II, Ill anc V nodes: From nasopharyngeal carcinom,

Fig. 16.96: Hugely enlarged lymph nodes which are

bluish in colour, pushing the carotid arteries anteriorlypapillary carcinoma thyroid

Cystic Swellings, Neck Swellings and Metastasis Lymph Node Neck

NECK DISSECTION (Fig. 16.103) Introduction • Crile in 1906, first described a standardised dissection of cervical nodes by applying anatomical and oncological principles including removal of surrounding fibrofatty tissue from various compartments of the neck. • Radical neck dissection causes significant functional and cosmetic morbidity. • Today adjuvant chemoradiotherapy is an important modality of treatment. • Thus with better understanding of tumour biology, natural history of disease and availability of adjuvant radiotherapy, radical neck dissection is not frequently done but its modifications are done. • Rule of 80 in neck masses (Key Box 16.38) Indication • Carcinoma tongue, carcinoma floor of mouth • Malignant melanoma • Metastatic lymph nodes from pharynx and upper oesophagus. Contraindications • Fixed nodes, evidence of distant metastasis • Untreatable primary cancer Types (see next page) I. Classical radical neck dissection (Crile's operation) Removal of level I to V nodes+ IJV, stemomastoid+ spinal accessory and submandibular salivary gland + Tail of parotid. Incision 1. MacFee: Two incisions are given (Fig 16.97) • Upper incision extends from mastoid process to hyoid bone up to the point of chin across intermediate tendon of digastric muscle. • Lower incision is given 2 cm above clavicle-from anterior border of trapezius to midline. • Gives a very good exposure and vascularity of flaps are good. No comers and hence, no necrosis. 2. Crile's incision • Upper incision is similar to MacFee. The other incision is oblique along the length of sternocleidomastoid inclining more in the posterior triangle (Fig. 16.98). • When stemocleidomastoid has to be removed as a part of neck dissection or to clear the lymph nodes which are badly stuck to jugular vein or to accommodate PMMC flap, Crile's is a better incision (Fig. 16.99).

267

• Here all the cervical lymph nodes from Level 1 to Level VI are removed along with nonlymphatic structures such as stemocleidomastoid muscle, internal jugular vein, accessory nerve (XI), submandibular salivary gland and cervical sympathetic plexus. A few examples wherein radical neck dissection is done are carcinoma tongue, oropharyngeal carcinoma, etc. II. Modified radical neck dissection (MRND-Fig. 16.100) Type I • Preserve one structure: Spinal accessory nerve • Classically done for squamous cell carcinoma of upper aerodigestive tract with clinically positive neck dissection. Type II MRND Preserve two structures: Spinal accessory and IN. Type III MRND or BOCCA's functional neck dissection (Fig. 16.101) • Preserve three structures: Spinal accessory, sterno­ cleidomastoid and internal jugular vein. • Done for metastatic well differentiated carcinoma thyroid. In this all the lymph nodes from Level I to Level V are removed but nonlymphatic structures are preserved. • Dissection is from lower border of mandible to clavicle and from anterior border of trapezius to midline. III. Selective neck dissection • Here any of the lymphatic compartments is preserved (which should have been removed as part of classic RND). A few examples are given below a. Supraomohyoid dissection (Fig. 16.102): Removal of nodes in Level I, II and III is done for carcinoma oropharynx, carcinoma cheek. Usually done for carcinoma floor, lateral tongue, etc. b. Lateral neck dissection: Level II, III, IV are removed as in carcinoma larynx and cervical oesophagus. c. Posterolateral neck dissection: Level II to V are removed as in cutaneous malignancy of posterior scalp and neck. Can also be done for thyroid malignancies. IV. Commando's operation RND, hemimandibulectomy with radical glossectomy. It is a very radical and aggressive surgery done for carcinoma tongue.

.

............................ .

KEY BOX 16.38

RULE OF 80 IN NECK MASSES • • • • •

80% of neck masses are neoplastic 80% of neoplastic masses occur in males 80% of neck masses are malignant 80% of malignant neck masses are metastatic 80% of metastatic neck masses are from primary sites above clavicle.

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• Double transverse incision • 3 point intersection is avoided • Subsequent blow outs of carotid artery can be avoided • Furcate incision­ otherwise called Y incision • 3 point intersection occurs which may give rise to necrosis

Figs 16.97 and 16.98: Incisions for block dissection: MacFee and Crile

Fig. 16.99: Sternomastoid retracted

Fig. 16.100: Radical neck dissection

Fig. 16.101: Functional neck dissection-blue nodes of papillary carcinoma thyroid are unmistakable

Fig. 16.102: Supraomohyoid neck dissection

Cystic Swellings, Neck Swellings and Metastasis Lymph Node Neck

Investigations 1. Complete blood picture 2. Chest X-ray can provide the following information: • Secondaries in the lungs with cannonball appearance as in cases of malignant melanoma of head and neck. • Bronchogenic carcinoma can be suspected by an irregular dense shadow in the peripheral lung fields. • Large mediastinal node mass may be seen, with or without tracheal shift. 3. Biopsy from clinically obvious lesion(tongue, cheek, etc.). 4. Triple endoscopy includes • Direct and indirect laryngoscopy • Oesophagoscopy • Bronchoscopy and biopsy of the suspicious area. 5. X-ray base of the skull may show destruction of the bone by the tumour. 6. CT scan of the sinuses, or nasopharyngeal area, or skull base to detect a primary growth, its extension, etc. 7. FNAC of the lymph nodes can give a diagnosis is more than 90% of the cases, avoid incision biopsy as it will result in tumour recurrence and wound necrosis. 8. If primary tumour cannot be detected on endoscopy, a blind biopsy is taken from posterior wall of the fossa of Rosenmiiller and of the pyriform fossa on the same side.

269

9. When aspiration cytology is negative, an excision biopsy is advised as a last resort. Treatment 1. When the primary tumour is obvious with enlarged lymph node metastasis, radiotherapy is the preferred line of treatment. However, see Chapter 17 for management of oral malignancy. 2. Treatment of occult primary with metastatic lymph nodes • If the lymph node or nodes are mobile, radical block dissection is done. If histopathologic report detects poorly differentiated carcinoma, extracapsular spread, or nodes are large(> 3 cm), radiotherapy is given. • If there are large, bulky, bilateral secondaries or fixed secondaries, radiotherapy is given to the neck masses. If the nodes do not completely regress or the tumour becomes bigger in size after 6-8 weeks of radiotherapy, radical neck dissection would be considered. Follow-up • About 30--40% of treated patients with occult primary with metastatic nodes die with no evidence of the primary later. • In about 30% of patients, the primary will manifest within 1-2 years time. • About 10% of patients are cured but primary is not detected.

Neck dissection

Classical RND-crile

Modified RND

Selective neck dissection

Type Ill

Supraomohyoid

Lateral neck

• Extended radical-Radical (Levels I to VI} with clearance of superior mediastinal nodes (Level VII}

Fig. 16.103: Types of radical neck dissection

Posterolateral

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Manipal Manual of Surgery

PANCOAST'$ TUMOUR • Pancoast's tumour or superior sulcus tumour is a bronchogenic carcinoma arising from the apex of lung. • Typically, the patient is an elderly male around 70 years, chronic smoker who presents with cough, weight loss, dyspnoea and chest pain. • As the tumour grows, it compresses the lower roots of brachial plexus CS and Tl and results in tingling, pain and paraesthesia in the distribution of ulnar nerve. • The tumour is felt in the lower part of the posterior triangle. It is hard in consistency, fixed, irregular and sometimes tender. The lower border of the mass cannot be appreciated. The Pancoast's syndrome refers to the following components: 1. Pancoast's tumour 2. Erosion of the first rib 3. Paralysis of C8 and T l nerve roots 4. Horner's syndrome due to paralysis of cervical sympathetic chain. The preganglionic sympathetic fibres of the head and neck are given from the 1st and sometimes the 2nd thoracic segments of the spinal cord. These nerve fibres synapse with the cells in the three cervical sympathetic ganglia. They give rise to postganglionic fibres to the head and neck region. Thus, anywhere along this pathway, disruption, damage or infiltration of the nerve roots results in Homer's syndrome. The causes of Homer's syndrome are depicted in Key Box 16.39.

• Pseudoptosis: Drooping of upper eyelid (Fig. 16.104). • Enophthalmos: Regression of the eyeball • Nasal vasodilatation: Nasal congestion Investigations 1. Chest X-ray: May demonstrate a dense mass or collaps� of the lobe, etc. 2. CT scan may demonstrate infiltration of the tumour intc ribs or vertebra. 3. Sputum for malignant cells 4. Flexible bronchoscopy: Tissue biopsy or sputum sample can be collected. 5. FNAC of the tumour gives the diagnosis in majority of cases. Treatment Palliative radiotherapy. The response rate is poor.

Components of Homer's syndrome • Miosis: Small pupil • Anhidrosis: Absence of sweating.

Mtm1,-w7,1 HORNER'$ SYNDROME COMMON CAUSES

• Posterior inferior cerebellar artery {PICA) thrombosis • Cervical sympathectomy • Pancoast's tumour UNCOMMON CAUSES • • • •

Syringomyelia Injury to lower roots of brachia! plexus Tumour in the neck Aneurysm of carotid artery

�

Fig. 16.104: Pseudoptosis due to Pancoast's tumour

WHAT IS NEW IN THIS CHAPTER?/RECENT ADVANCES

• All topics have been updated. • Many interesting short cases and photographs are inserted. • Simple flow charts which help in diagnosing many short cases in the examination have been included.

Cystic Swellings, Neck Swellings and Metastasis Lymph Node Neck

271

MULTIPLE CHOICE QUESTIONS 1. Following are true for ranula except: A. It is a swelling in the floor of the mouth B. It is a retention cyst C. It is transilluminant D. Plunging ranula produces one more swelling in the submental region 2. Which one of the following does not give rise to cross fluctuation: A. Iliopsoas abscess B. Compound palmar ganglion C. Sebaceous cyst D. Hydrocoele en bisac 3. Following are true for thyroglossal fistula except: A. Always congenital B. It is lined by columnar epithelium C. Semilunar sign is seen in adults D. Surgery done for this is called Sistrunk's operation 4. Following are derived /arises from 2nd branchial arch except: A. Sternomastoid muscle B. Branchial cyst C. Facial muscles D. Anterior belly of the digastric 5. Which one of the following swelling does not contain cholesterol crystals? A. Branchial cyst B. Sebaceous cyst C. Dental cyst D. Hydrocoele

6. Most important stimulus for carotid body tumour is: B. Hyperbaric oxygen A. Hypoxia D. Hypercarbia C. Hypothermia 7. Tuberculous cervical spine can give rise to cold abscess in following locations except: A. Posterior wall of the pharynx in the midline B. Behind the carotid sheath C. Front of the carotid sheath D. Along the anterior primary division of the cervical spinal nerves 8. Pancoast' tumour has following features except: A. It is a superior sulcus tumour B. It can given rise to Homer's syndrome C. It can erode first rib D. It is ususually resectable 9. Components of Horner's syndrome are following except: B. Anhydrosis A. Miosis D. Exophthalmos C. Pseudoptosis 10. Following organs drain to posterior triangle lymph nodes B. Thyroid A. Adenoids D. Tonsil C. Retropharynx 11. Content of sebaceous cyst includes A. Desquamated epithelial debris B. Keratin C. Sebum D. Pus

ANSWERS D 11 B

2 C

3 A

4 D

5 B

6A

7 C

8 D

9 D

10 D

17 Oral Cavity, Odontomes, Lip and Palate • • • • • • • • •

Oral cancer Premalignant conditions General principles Carcinoma of buccal mucosa Carcinoma of tongue Ulcers of tongue Carcinoma of lip Carcinoma maxillary antrum Nasopharynx-cancer

• • • • • • • • •

Introduction Oral cavity is bounded by the lips anteriorly, the cheek on each side, tonsils posteriorly, superiorly by the palate and inferiorly by the floor of the mouth (Key Box 17. l ). It is lined by squamous epithelium. Oral cavity is a common site of malignancy because it is insulted by various agents such as alcohol, smoking, tobacco chewing. Oral cancer is the commonest malignant neoplasm in the head and neck. Hence, it is discussed first in this chapter. Abuse of tobacco and alcohol are the most common preventable risk factors for development of head and neck cancers (Key Box 17.2). Those who smoke 2 packs/day and drink 4 units of alcohol/day have an odds ratio of 35 for development of carcinoma. Tobacco quid is very dangerous and highly carcinogenic. An interaction between redox-active metals in saliva and low reactive free radicals in cigarette smoke occur. Net result is that saliva loses its antioxidant capacity and instead becomes a potent pro-oxidant milieu. Field cancerisation is a concept based on prolonged exposure of oral and pharyngeal mucosa to carcinogens. 15 to 20% of the survivors of one cancer of head and neck develop another primary head and neck cancer.

Benign lesions in the oral cavity Odontomes Epulis Median mental sinus Vincent's angina Cleft lip and cleft palate Ectopic salivary gland tumour Mucous cysts What is new ?/Recent advances

.,............. • • • • • • • •

ORAL CAVITY SUB SITES

�

Lip Buccal mucosa Lower alveolus Retromolar trigone Oral tongue Floor of mouth Upper alveolus Hard palate

.,........... • • • • • • •

� RISK FACTORS ASSOCIATED WITH CANCER OF HEAD AND NECK Tobacco quid 1 -'Pan Masala' Oropharyngeal Ca-Plummer-Vinson syndrome Barr-Epstein virus Alcohol, areca nut Cigarette smoking and reverse smoking2 Chronic irritation-Dentures Oral hygiene poor and poor nutrition Remember as TOBACCO

Betel nut chewed in combination with lime and cured tobacco is called 'quid'. It is commonly placed in gingivolabial sulcus. It is highly carcinogenic. This type of carcinoma is very common in India called Indian oral cancer. 2 Reverse smoking: Smoking a cheroot with burning end inside mouth. The risk of hard palate carcinoma is 4 7 times more in these patients. 1

272

Oral Cavity, Odontomes, Lip and Palate

ORAL CANCER Incidence Tongue Cheek Floor • Gums

of oral cancer 50% : 20-25% : I 0-15% : 10%

Definitions • Hyperkeratosis refers to increase in keratin layers. It occurs due to constant irritation. Once the cause is removed, it is reversible. It is a microscopic diagnosis. For example, Smokers' hyperkeratosis of the palate and lips. Once the aetiological agent is withdrawn, the lesion returns back to normal. • Leukoplakia appears clinically as a white patch in the mouth and cannot be scraped off. It is irreversible and not attributable to any known disease. It is important to biopsy leukoplakic portion to rule out malignancy. PEARLS OF

WISDOM

Sump area or 'Coffin corner' at the posterior tongue/floor of mouth is a common site for cancer-may be missed on cursory inspection. PREMALIGNANT CONDITIONS FOR ORAL CANCER 1. Leukoplakia The causes for leukoplakia are as follows: • Smoking results in hyperkeratosis. Nicotine in the form of cigarettes, chewed tobacco 1, powdered snuff produces premalignant changes in the oral cavity. • Spices • Spirits have synergistic action with smoking

Fig. 17.1: Leukoplakia of the tongue­ biopsy was positive for malignancy. He was treated with radiotherapy

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• Sharp tooth, sepsis, poor oral hygiene • Sunlight actinic rays • Syphilis causes endarteritis obliterans and results in chronic superficial glossitis of the tongue which is a precancerous condition (rare these days). • Susceptibility 1 of a person • Betel nut, and slaked lime with betel leaf and tobacco (pan) is eaten and usually kept inside the cheek for many hours. Over the years, it brings about chronic irritation of mucosa of the cheek and causes leukoplakia. Tobacco contains multiple carcinogens including aromatic hydrocarbons. Stages in the development of leukoplakia I. Keratosis appears as a milky blush on the surface. 1 I. Acanthosis refers to elongation of rete pegs. This appears as a smooth, white, dry patch. III. Dyskeratosis means the formation of keratin cell layer in the deeper aspect of epidermis, before they reach the surface. IV. Speckled leukoplakia appears as multiple, small white p"atches on an erythematous base. It has the highest rate of malignant transformation (Figs 17.1 and 17 .3 ). V. Carcinoma in situ

•.............

�

CHRONIC HYPERPLASTIC CANDIDIASIS (Fig. 17.2) Commissures of the mouth commonly affected Albicans Candida invasion No response to drugs, then surgery/laser treatment Dense plaques of leukoplakia Immunodeficiency can precipitate this condition Dangerous because of malignant potential Antifungal treatment-Topical application may help Remember as CANDIDA

Fig. 17.2: Chronic hyperplastic candidiasis affecting palate

Fig. 17.3: Carcinoma arising from leukoplakia

( Courtesy: Dr Keerthilatha Pai, Head, Oral Medicine, College of Dental Sciences, Manipal) 1 An 18-year-old girl, nonsmoker, nonalcoholic, with good oral hygiene had carcinoma tongue. What was the cause?

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Treatment of leukoplakia • About 10% of leukoplakia patients develop oral cancer. Hence, superficial excision of the lesion followed by skin grafting should be done. • Even though leukoplakia is irreversible, Isotretinoin (13cis-retinoic acid) can reverse some cases of leukoplakia and possibly reduce the development of squamous cell carcinoma. 2. Erythroplakia is a red, velvety lesion with an incidence of malignancy around 15% (17 times more malignant than leukoplakia). It is irregular in outline and may be nodular. 3. Chronic hyperplastic candidiasis (Key Box 17.3). 4. Submucous fibrosis • This is supposedly due to use of pan masala , arecanut with or without alcohol. • Initially it produces ulceration of mucosa of the cheek. These ulcers heal resulting in a dense submucous fibrosis, which appear clinically firm to hard. It can affect the tongue also. It is a progressive disease entirely confined to Asian population. • Chances of malignancy are around 10-15%. • Mouth opening may be restricted • It is treated by excision with reconstruction. 5. Sideropenic dysphagia (Plummer-Vinson and Paterson­ Kelly syndrome). Iron deficiency occurs in the absence of anaemia in these patients. Common in Scandinavian women. Iron supplements reduce epithelial atrophy. 6. Papilloma of the tongue or cheek 7. Discoid lupus erythematosis 8. Dyskeratosis congenita 9. Syphilitic glossitis: Tertiary syphilis produces chronic superficial glossitis which can lead to carcinoma of the tongue. However, it is rare these days. 10. Human papilloma virus is a epitheliotropic virus. Its oncoproteins suppress tumour suppressor gene. It can give rise to tonsillar carcinoma and oropharyngeal carcinoma. 11. Miscelllaneous saw dust-Sinonasal adenocarcinoma. • U-V rays-lip cancer Reverse cigarette smoking-palatal cancer. Upper aerodigestive tract cancers • Most of them are squamous cell carcinomas. • Tobacco and alcohol are the most common aetiological factors. • Most common premalignant lesion is leukoplakia. • Multiple anatomic sites can be involved simultaneously (synchronous). • Second primary cancers develop in 10-15% of cases (metachronous). • Clinical presentation can be peculiar/misleading depending on anatomic site.

• Generous biopsy and MRI (if necessary) are the investigations of choice. • Surgery, radiotherapy and chemotherapy are used singly or in combination in appropriate cases. PEARLS OF WISDOM

Distant metastasis is more common with nasopharyngeal carcinoma than with any other head and neck cancer. Common symptoms and sites (Table 17.1) Common symptoms and common sites of oral cancer (Ca = Carcinoma) Symptom

Site of cancer-Ca

Pain around the eyes Pain in the ear (otalgia) Hoarseness Trismus Dysphagia Loss of hearing

Nasopharynx Base of tongue, hypopharynx Glottis Extension of cancer into pterygoid muscles Base of tongue, hypopharynx, oesophagus Auditory canal or nasopharynx

GENERAL PRINCIPLES IN THE TREATMENT OF ORAL CANCER Aim of the treatment 1. Cure of the patient: Cure of the cancer, if possible, with wide excision of the tumour which includes removal of the tumour with 2 cm of the normal tissues, with or without bone. 2. Palliation: If cure is not possible, palliation should be attempted by surgery or radiotherapy. 3. Preservation of function such as swallowing, speech and vision, should also be taken into consideration. 4. Cosmetic function: Following wide excision, the cosmetic function must be maintained by reconstruction with myocutaneous/osteomyocutaneous flap. 5. To achieve minimal mortality and morbidity. 6. Metastatic lymph nodes are treated by neck dissection or curative radiotherapy (RT). Even when nodes are not palpable, following guidelines are given (Key Boxes 17.4 and 17.5).

•,•.........,.� CLINICALLY 'NODE-NEGATIVE' NECK FROM ORAL CANCER

• Carcinoma lateral tongue, floor of mouth and mandibular alveolus commonly cause occult metastasis. • Occult metastasis is seen in up to 30% of patients. • Hence selective neck dissection of levels I, II and Ill are indicated in continuity with tumour excision in these cases.

Oral Cavity, Odontomes, Lip and Palate

N1 {N2a N2b} N2c N3

CLINICALLY NODE POSITIVE NECK

: Selective supra-omohyoid neck dissection : Modified radical or radical neck dissection followed by postoperative radiotherapy : Bilateral radical neck dissection-preserve at least one Internal Jugular Vein (IJV) + postoperative radiotherapy : Preoperative radiotherapy, if feasible radical neck dissection later.

ORAL CANCER-American Joint Committee Cancer (AJCC) PRIMARY TUMOUR (T) • •

TO: No evidence of primary tumour Tis: Carcinoma in situ T l : :::; 2 cm T2: > 2 cm and:::; 4 cm T3: > 4 cm T4: Any cancer invading adjacent structures such as cartilage, cortical bone, deep (extrinsic) muscles of the tongue, skin or soft tissue of the neck. T4a: Moderately advanced local disease T4b: Very advanced local disease (skull base, pterygoid plate, internal carotid artery, masticator space)

REGIONAL LVMPH NODES (N) • Nx: Nodes cannot be assessed • NO: No lymph node metastasis

• •

Nl: Single positive ipsilateral node less than or equal to 3 cm in greatest dimension N2a: Single positive ipsilateral node more than 3 cm but less than or equal to 6 cm N2b: Multiple ipsilateral nodes but all less than 6 cm N2c: Bilateral or contralateral lymph nodes but all less than 6 cm N3: Lymph node more than 6 cm.

DISTANT METASTASIS (M) • MO: No distant metastasis • M l : Distant metastasis present STAGE GROUPING Stage I Stage II Stage III Stage IV

T l , NO, T2, NO, T3, NO, T4, NO, TO, NO,

MO MO MO, Tl-3, NI, MO MO , T, N2-3, MO Ml

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7. Treatment of advanced tumours-T3 and T4 lesions: These are managed by combination of surgery with postoperative RT. Usually, surgery is the principal therapeutic modality of treatment followed by postoperative radiotherapy. The treatment depends upon general condition of the patient, risks of anaesthesia, adequate intensive care management, etc. Chemotherapy also has been tried before or after surgery. However, response rate has improved but it has not affected the survival. Flowchart showing treatment of primary tumour and metastasis is given in Figs 17.4 and 17.5 These are guidelines only, individualise the treatment. Role of chemotherapy in head and neck cancers • The most important benefit of chemotherapy has been in the treatment of laryngeal and nasopharyngeal carcinomas. • Cisplatin is clearly the most effective drug. Other drugs such as carboplatin, 5-fluorouracil (5-FU), bleomycin, gemcitabine, etc. are also used. • Induction chemotherapy: In advanced cases, chemo­ therapy is given before surgery or RT. In more than 80% of cases, tumour regression can occur. • Concurrent chemoradiotherapy (CCRT): It improves both local and regional controls, specially in those patients with high risk cancers, e.g. locally advanced cancers of the oral cavity, larynx, oropharynx. Drug used in CCRT can be high dose cisplatin-100 mg/m2 IV for 3 cycles every 21 days concomitantly with RT (for other dosage, kindly refer to oncology manual). Side effects include severe mucositis, xerostomia. Gastrostomy may be necessary for feeding. Radiotherapy (RT) (Key Box 17.6) • Irradiation of the oral cancers achieves a cure in about 80-90% of patients. It preserves anatomical part and also preserves the function. • The dose of RT: 6500-7500 cGy units is required to eradicate squamous cell carcinoma of head and neck. It is usually given in the daily dose of 180-200 cGy units.

IHHIIIIIIIIIIMIIIIIIIIIIIIIII"� RT:ADVANTAGES

• • • • •

Easy, safe with minimal mortality Preservation of an organ Function of the part is preserved Cure rate is around 80 to 90% First line in early cases.

• • • •

Long stay in the hospital (can also be taken as an out patient) Tumour cure cannot be assesed by pathology Soft tissue fibrosis resulting in ankylostomia Adverse effects on skin, loss of hair, mucositis of oral cavity, xerostomia, etc.

RT: DISADVANTAGES

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I I • • • • •

SURGERY

PRIMARY TUMOUR r1, r2 j

I

I

[oRJ

Accessible area Well-differentiated Tumour close to the bones Invasive tumours Radiorecurrent

In T1 or T2 lesions surgery and RT both have equal results

RADIOTHERAPY (RT)

l

.. ..

• Inaccessible area High grade tumours Risk for surgery Patient's choice, organ preservation Recurrence after surgery

: METASTASIS-LYMPH NODES: [oRJ Surgery as above + Radical neck dissection + Postoperative RT

In advanced lesions, surgery is found to be superior to radiotherapy as a first line of treatment

Radiotherapy + Post RT surgery

Fig. 17.4: Treatment of primary tumour and metastasis

Complete resection-composite resection Selective neck dissection Reconstruction Adjuvant therapy Radiation alone

Concurrent chemoradiation

Dose: 60 Gy to lymph nodes + primary and 50 Gy lower risk nodal stations in the neck

Indications Good performance status • Few or no comorbidities

Drugs Cisplatin-100 mg/m2 on days 1, 22 and 43 of 7-week course of 70 Gy of radiation • Ceruximab, an EGFR* antibody 200-400 mg/m' weekly 8 weeks * EGFR: Epidermal growth factor receptor

Fig. 17.5: Management of locally advanced nonmetastatic disease (111 to IV B)

• Radiotherapy is given in Tl and T2 lesions as the first line of treatment and postoperatively in T3 and T4 lesions after surgery. Role of surgery (Key Box 17.7) • Surgery is done in all stages of oral cancers. It may be in the form of wide excision or wide excision with removal of the bone (composite resection). In advanced stages it may be palliative surgery such as excision of a fungating, ulcerating, bleeding mass. Surgery is also done for the lymph nodes in the form ofradical neck dissection (RND), or modified RND. • The pectoralis major myocutaneous flap (PMMC flap): It is the most widely used flap for the reconstruction of oral cancers.

MMII...........� SURGERY:ADVANTAGES • It removes a fungating, ulcerating, bleeding lesion. • It relieves the pain. • The specimen is available for histopathological examination for cancer clearance. • 80-90% cure is possible.

• • • •

SURGERY: DISADVANTAGES Loss of an organ-total glossectomy Functional and cosmetic disability Significant morbidity Mortality: 8-10%.

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Indications for surgery • Early disease, bulky disease • Tumour involving mandibular alveolus • Tumour invading bone • Advanced disease • Fungating and bleeding lesion • Radiorecurrence • Multiple tumours • Extensive premalignant changes of oropharyngeal mucosa CARCINOMA OF BUCCAL MUCOSA Carcinoma of the buccal mucosa is very common in India due to the habit of keeping tobacco quid in the cheek pouch (gingivoalveolar sulcus). Pathological types 1. A nonhealing ulcer, with slough in the centre of the lesion 2. An exophytic growth, or a proliferative growth-verrucous carcinoma. 3. An infiltrative lesion slowly involves the adjacent structures such as tongue, mandible, floor of the mouth and skin. Skin infiltration results in orocutaneous fistula. Clinical features • A nonhealing ulcer or cauliflower like growth. Verrucous carcinoma is an exophytic growth. • Edges are everted (Fig. l 7.6) with induration at the base as well as at the edge. lnduration clinically presents as a hard feeling. Pathologically, it is due to fibrosis, caused by malignancy (carcinomatous fibrosis). It is a diagnostic feature of squamous cell carcinoma. Possibly, it is a host reaction indicating good immunity. Due to fibrosis, some lymphatics get obliterated. This delays spread of the disease, thereby improving the prognosis. • Proliferative lesions are often verrucous carcinoma (Key Box 17.8). • Ulcer bleeds on touch. Due to secondary infection most of the oral cancers are tender to touch (Fig. 17.9).

Fig. 17.6: Carcinoma alveolar margin. Look at teeth stains-everted edge

• • • • •

PECULIARITIES OF VERRUCOUS CARCINOMA Very slow-growing Growth is exophytic (than infiltrative) Rarely spreads by lymphatics It is a well-differentiated carcinoma Surgery is the treatment of choice.

• Fixity to the underlying structures such as mandible may be present. • Surrounding area may also show induration. • Evidence ofleukoplakia may be present in the oral cavity. • Trismus is due to involvement of pterygoid muscles and masseter. This occurs when carcinoma buccal mucosa extends into the retromolar trigone. Trismus can also be due to soft tissue fibrosis caused by radiation. Once perineural lymphatics are involved, spread can occur in infratemporal fossa resulting in trismus (Key Box 17.9 and Fig. 17.10). • Halitosis is very characteristic.

..............

�

TRISMUS Difficulty in opening the mouth is called trismus. Normal mouth opening ranges from 35 to 45 mm. Grades: • Grade I : Mouth opening is between 2.5 and 4 cm • Grade II : Between 1 and 2.5 cm • Grade Ill: Less than 1 cm

Common causes of trismus 1. Temporomandibular joint involvement-such as ankylosis, dislocation, synovitis, etc. 2. Mandible fractures 3. Pterygoid muscle infiltration by growth in the retromolar region 4. Acute inflammatory lesions in the oral cavity 5. Tetanus and tetany 6. Radiation fibrosis of soft tissues/muscles of mastication

Fig. 17.7: Carcinoma cheek with orocutaneous fistula

Fig. 17.8: Carcinoma buccal mucosa infiltrating skin-'warning' of fistula

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• Assessment of fixity to mandible: Severe pain over the jaw indicates periostitis. • Bidigital palpation of mandible is done by examining with index finger on the outer aspect of the mandible and the thumb on the under surface of the mandible. This test should be done on the opposite side first. Only then, the thickening of the mandible can be appreciated. • Gingival cancers (Fig. 17.11) - Early cases present as mucosa! change in leukoplakia - Loosening of tooth may be a presenting feature. - Can present as bleeding and pain - Bone involvement occurs early - Spread to adjacent structures occurs early. Spread 1. Local spread: Once it involves the entire thickness of the cheek it results in orocutaneous fistula (Figs 17.7 and 17.8). Involvement of mandible results in sinus (Key Box 17. I 0).

··-� CARCINOMA BUCCAL MUCOSA AND MANDIBLE

• • • • •

Direct infiltration by the tumour Through mandibular canal Through periodontal membrane Orthopantomogram or spiral CT can be used for imaging Loss of central part of mandible results in pouting of lower lip and continuous drooling of saliva. It is called as Andy Gump deformity1.

2. Lymphatic spread: Submandibular nodes and upper deep cervical nodes get enlarged (Levels l and II). In 50% of the cases, lymph node enlargement is due to infection and remaining 50%, it is due to metastasis. Metastatic deposits are hard in consistency, indurated and with or without

Fig. 17.9: Carcinoma buccal mucosa - advanced

fixity. Significant oedema of face can occur due t, lymphatic spread. 3. Blood spread: It is very rare and it occurs late. PEARLS OF

WISDOM

The mandibular canal is close to occlusive surface, in edentulous elderly patients due to decrease in the vertical height of horizontal ramus thus facilitating easy spread (Figs 17.12 and 17.13) of oral cancer to mandible. Investigations 1. Wedge biopsy from the edge of the ulcer is taken becausE of the following reasons: • Tumour cells are concentrated more in the growing edge • Comparison with the normal tissues is possible. • Centre of the ulcer has slough. • Histopathological report shows squamous cell carcinoma and in majority of the cases it is well­ differentiated. 2. Orthopantomography: X-ray of mandible to rule out mandibular involvement 3. Chest X-ray to detect inhalation pneumonia. 4. FNAC of the lymph node. 5. Magnetic resonance imaging (MRI) • Large advanced lesion can be better assessed by MRI. Soft tissue infiltration can be assessed correctly thus dictating the extent of resection especially in patients with restricted mouth opening. • It is the investigation of choice to look for involvement of skull base, brachia! plexus, spinal nerve roots and lymph nodes. • MRI has no radiation hazards. Treatment of carcinoma buccal mucosa It can be classified into early disease and advanced disease (Figs 17.9 to 17.11).

Fig. 17.10: Carcinoma buccal mucosa infiltrating mandible - severe trismus. Also observe tobacco stains

Fig. 17.11: Carcinoma alveolus

1 Andy Gump was one of the characters in a popular comic strip, 'The Gumps' created by Sidney Smith in 1917. The character's face seems to end at the upper lip due to 'absence of mandible' and is chinless. A statue of this comic character is on display at Lake Geneva Museum

Oral Cavity, Odontomes, Lip and Palate

Early disease

.,.........,.� Two modalities are given in Key Box 17. I I.

• • • • •

EARLY CARCINOMA BUCCAL MUCOSA

T1, T2 lesions-surgery/RT T1 lesion near commissure-RT T2-exophytic and superficial-RT T2-deep-surgery is better Early disease-no nodes-surgery is better-no other treatment is necessary. • Early disease-positive lymph nodes-same modality to be used for primary and secondary.

I. Surgery 1. A small superficial ulcer (T 1, T2) is treated by wide excision followed by split skin graft (SSG). 2. An infiltrative lesion is treated with wide excision followed by a flap reconstruction. Usually, PMMC (pectoralis major myocutaneous flap) is used. PMMC flap: This is the most widely used flap now for head and neck reconstruction. The flap is raised along with muscle and an island of skin based on pectoral branch of thoracoacromial artery. It is tunnelled under the skin of chest wall and neck and brought to the area of the defect. It has been described as the 'workhorse' for head and neck reconstruction. • Radial artery based flap is the workhorse of microvas­ cular reconstruction. II. Radiotherapy As mentioned earlier, early lesions can be managed with radiotherapy (RT). The advantage of RT is that it cannot only cure the disease but also preserve the organ and its function.

-+

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Indications for radiotherapy 1. Patient not willing for surgery 2. Patient not fit for surgery 3. T l and T2 lesions 4. Lesion near the commissure. Types 1. External radiotherapy: Large total dose of 6000-8000 cGy units are given at the rate of 200 cGy units/day. 2. Interstitial radiotherapy is indicated in infiltrative small lesions. Caesium 137 or iridium wires are placed within the tumour. Advantage of this method is minimal tissue reaction. Advanced carcinoma buccal mucosa (Figs 17.14to 17.19) Surgery: T3 and T4 lesions require surgery as the main modality of the treatment followed by postoperative radio­ therapy. Most of the lesions require full thickness resection leaving behind large defects. Such defects can be repaired using myocutaneous flap. PEARLS OF

WISDOM

When the primary lesion is removed en bloc with mandible and cervical lymph nodes, it is called composite resection. Examples of surgeries 1. Carcinoma buccal mucosa fixed to the mandible: Wide excision of the growth along with segmental resection of the mandible or hemimandibulectomy is done depending upon the infiltration of the tumour. Very often, whole thickness of the cheek is lost which is reconstructed by using PMMC flap (see next page).

Mandibular canal

Fig. 17.12: Mandibular canal in a normal person is well away from occlusive surface

Fig. 17.13: Mandibular canal in an edentulous patient is close to occlusive surface explaining easy spread in carcinoma buccal mucosa

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Fig. 17.14: Carcinoma cheek with infiltration into the skin and mandible-locally advanced. Good case for composite resection

Fig. 17.17: Gross lymphoedema of left side of the face with enlargement of sub­ mandibular lymph nodes

Fig. 17.15: Neglected carcinoma buccal m ucosa infiltrating floor of the mouth, commissure and lower lip

Fig. 17.18: Carcinoma alveolus-excavating ulcer Fig. 17.19: Carcinoma buccal with slough, infiltrating retromolar trigone. RT followed mucosa on the right side arising from submucous fibrosis by surgery is the ideal choice of treatment

• Segmental mandibulectomy: Indications A. Clinical and radiological involvement of bone B. To obtain wide margins C. To facilitate reconstruction when one has to use 'bulky' PMMC flap D. Excision of full thickness of cheek. • Marginal mandibulectomy: It can be done for carcinoma floor of the mouth or tongue. In this removal of either inner or outer table of the mandible or excising the superior rim of the mandible. However, in large lesions, it is better not to do marginal mandibulectomy. • Hemimandibulectomy: Very advanced lesion may necessitate removal of mandible. Various methods of reconstruction of mandible following excision • Soft tissue • Soft tissue with bone • Nonvascularised bone grafts • Vascularised bone grafts

Fig. 17 .16: Advanced carcinoma buccal mucosa with involvement of half of lower lip

: PMMC flap : 2.4 mm reconstruction alloplastic material plate and PMMC flap : Titanium tray and cancellous chips from iliac crest : Fibula, iliac crest

2. Orocutaneous fistula is treated by wide excision which refers to removal of the entire thickness of the cheek along with the growth. Reconstruction is done by using PMMC flap. Radiotherapy should not be given as it results in persistence of fistula. 3. Carcinoma of the buccal mucosa with lymph nodes: Along with the primary, submandibular nodes and upper deep cervical nodes (Levels I, II and III) are removed, along with submandibular salivary gland. This is called supraomohyoid block dissection. If surgery has been used to treat the primary, the lymph nodes also should be treated by surgery in the form of neck dissection. 4. Carcinoma of buccal mucosa with fixed lymph nodes: Both primary lesion and lymph nodes should be treated by radiotherapy and reassessment done after 3-4 weeks. If residual glands persist or if the glands become mobile, neck dissection can be done at a later date. Fixity to internal jugular vein or stemocleidomastoid muscle are not contra­ indications for radical block dissection. Those structures can be removed. However, when the lymph nodes are fixed to the carotid artery, radiotherapy is preferred.

Oral Cavity, Odontomes, Lip and Palate

Prophylactic neck dissection • It is advocated in T3 and T4 lesions irrespective of nodal status. This amounts to minimal supraomohyoid neck dissection with removal of Levels I, II and Ill lymph nodes. It has shown survival benefits. • It is also indicated in carcinoma mandibular alveolus or buccal mucosa extending into the floor of mouth.

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Complications of carcinoma buccal mucosa 1. Orocutaneous fistula 2. Trismus-It can be due to direct infiltration of pterygoid muscles or masseter or soft tissue fibrosis following RT. 3. Recurrent respiratory tract infection 4. Cancer cachexia.

STEPS OF PECTORALIS MAJOR MYOCUTANEOUS FLAP (PMMC) (Figs 17.20 to 17.25) WORKHORSE OF HEAD AND NECK RECONSTRUCTION

Fig. 17.20: Amount of tissue to be removed

(wide excision) is marked. Horizontal incision is given in the neck for block dissection

Fig. 17.23: Elevation of flap

Fig. 17.21: Skin paddle outlined prior to

elevation

Fig. 17.22: Skin paddle mobilised

Fig. 17.24: You can see here wide excision Fig. 17.25: Elevated flap is being mobi­ of growth of mandible. The specimen is about lised to be removed

( Courtesy. Prof. Satadru Ray, Head of the Department, Surgical Oncology, KMC, Manipal)

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PMMC FLAP-CONTINUED (Figs 17.26 to 17.31)

Fig. 17.26: Flap being turned out (bipedaled) to provide inner and outer lining for the tissue loss

Fig. 17.27: Composite resection: Inner aspect

Fig. 17.28: Composite resection: Outer aspect

Fig. 17.29: Modified radical neck dissection is being done

Fig. 17.30: Completely mobilised PMMC flap ready for reconstruction. Main vessels in the paddle are pectoral branches of acromiothoracic artery. Mandible reconstruction is not mandatory

Fig. 17.31: Healing after 10 days

Oral Cavity, Odontomes, Lip and Palate

CARCINOMA OF TONGUE Pathological types 1. Nonhealing ulcer, commonly on lateral border of tongue in 60% of cases, with slough (Fig. 17.32) 2. A proliferative growth, with everted edge 3. Frozen tongue or indurated variety (Fig. 17.33) In this variety, there is maximum induration and sometimes it is more than the size of tumour. The tongue is converted into a hard woody "mass". 4. Fissure variety: The tongue is indurated with deep fissure. Clinical presentation • A bleeding ulcer

Fig. 17.32: Carcinoma tongue lateral border-the most common site

283

• Pain in the tongue is due to involvement of lingual nerve. In such cases, pain from the tongue can be referred to the ear and lower temporal region. 1 • Ankyloglossia is restricted mobility of the tongue. It is due to infiltration of the floor of the mouth or mandible, or due to an advanced lesion (Figs 17.33 and 17.34). • Disarticulation-difficulty in talking is due to inability of the tongue to move freely. • Dysphagia is a common presentation from carcinoma of posterior 113rd (in 20% cases). An elderly gentleman sitting in the outpatient department spitting blood-stained saliva is suggestive of carcinoma posterior 113rd of the tongue (Figs 17.35 and 17.36). • Foetor oris is due to infected necrotic growth.

Fig.17.33: Frozen tongue-ankyloglossia and dysphagia are present

Fig. 17.36: Carcinoma tongue with absolute dysphagia, fixed lymph nodes in the neck and involvement of mediastinal lymph nodes­ receiving radiotherapy-on Ryle's tube feeding Fig. 17.34: Carcinoma tongue with ankyloglossia, disarticulation, dysphagia

Fig. 17.37: Carcinoma tongue­ excavating ulcer

Fig. 17.35: Carcinoma posterior 113rd easily missed

Fig. 17.38: Carcinoma tongue left lateral border everted edges and excavating

Fig. 17.39: Carcinoma lateral border tongue in a 40-year-old man-elevated and everted lesion

1 Auriculotemporal nerve and lingual nerve are posterior branches of mandibular division of trigeminal nerve.

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• Bilateral massive enlargement of lower deep cervical nodes in an elderly patient is suggestive of carcinoma of posterior I/3rd. The patient may not be aware of growth at all. PEARLS OF

Basal vessels Central vessels

WISDOM

Tongue cancers tend to be more rapid in their onset than other cancers in the oral cavity. Compared with other cancers within the oral cavity, tongue cancers have greater potential of lymph node metastasis. Clinical examination • Inspection and palpation of the growth or the ulcer should be described in the same manner as that of carcinoma cheek. Typically, the ulcer bleeds on touch with central slough. The edge, base and sun-ounding area are indurated. Carcinoma of the tongue and carcinoma of the penis are two places in the body wherein induration can be much more extensive than the primary growth or an ulcer. In some cases, induration may be the only finding. Everted edge is commonly seen (Figs 17.37 to 17.39). • Digital palpation of posterior I/3rd of tongue should be done with a glove. Test for mobility of the tongue. - Forward protrusion-genioglossus. This is the muscle commonly involved. - Backward movement-styloglossus - Elevation-palatoglossus - Depression-hyoglossus All these muscles are supplied by hypoglossal nerve except palatoglossus which is supplied by glosso­ pharyngeal nerve. Bidigital palpation of the mandible should be done which may show thickening. Lymphatic spread (Fig. 17.40) 1. Apical vessels drain the tip of the tongue into submental lymph nodes, bilaterally. 2. Lateral vessels drain into submandibular lymph nodes, from here to the lower deep cervical lymph nodes and jugulo-omohyoid nodes-level III. 3. Central vessels drain into submandibular nodes. 4. Basal vessels drain the posterior 113rd of the tongue. There is criss-crossing of the lymphatics on both sides. Hence, they drain into bilateral lower deep cervical lymph nodes. (Some lymphatics on their way to the nodes, pass through the mandible which explains the frequent involvement of mandible in carcinoma tongue. This is a debatable issue now.) • In 50% of cases, the lymph node enlargement is due to secondary infection. Such nodes are tender and firm and respond to antibiotics. In remaining cases, they are hard and fixed and hence, significant.

Lateral --;-----..+---1 vessels

Apical vessels

Sternomastoid Internal jugular vein

Fig. 17.40: Lymphatic drainage of the tongue-see text for numbers PEARLS OF

WISDOM

Posterior 1/3 tongue has very less cornification but has abundant lymphatics which explains massive nodes (Key Box 17.12). Investigations 1. Wedge biopsy from edge of the ulcer can be taken under local anaesthesia. In cases of proliferative growth, punch biopsy is recommended. In cases of growth arising from posterior I/3rd of the tongue, biopsy can be taken under general anaesthesia. It also provides an opportunity to examine in detail the posterior spread of the disease into tonsils, pharynx, etc. 2. Or thopantomogram: X-ray of the mandible can demonstrate an irregular defect due to invasion, erosion or pathological fracture.

•.............

�

CARCINOMA POSTERIOR 113rd • It presents with dysphagia or with a change in voice. • Easily missed in a clinical examination • Biopsy should be done under general anaesthesia to avoid aspiration and to assess the spread posteriorly. • Palpation will give the diagnosis-induration • It is one of the occult primaries for lymph node secondaries in the neck. • Criss-crossing of the lymphatics explain bilateral lymph nodes in the neck. • Blood spread is more common. • Prognosis is bad because well-differentiated carcinoma in this location is rare.

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3. Chest X-ray is taken to rule out aspiration or inhalation pneumonia. 4. Routine investigations such as complete blood picture, fasting and postprandial sugar estimation to rule out diabetes and electrocardiography to assess cardiovascular function should be done. 5. Very advanced lesions may require CT scan/MRI scan. Treatment Carcinoma of the tongue is managed similar to a cancer in the oral cavity. However, to preserve the function of the tongue, widespread disease in the posterior one-third tumours, general health of patient (elderly with bad bronchopneumonia) may decide the treatment in favour of radiotherapy. However, results of surgery or radiotherapy for early carcinoma of tongue are equivalent. Various types of surgery 1. Carcinoma in situ: This type is uncommon in our country. Wide excision with 1 cm margin and a depth of 1 cm is sufficient. Reconstruction of the tongue is not necessary. 2. Partial glossectomy is indicated when the lesion is less than 2 cm (Tl) and confined to the lateral border of the tongue. About I/3rd of the anterior 213rd of the tongue is removed. The wide excision should include at least 2 cm of tissue away from the palpable indurated edge of the tumour (Figs 17.41 and 17.43). • Alternatively, radiotherapy can be given. 3. Hemiglossectomy refers to removal of around 50% of the tongue. This is indicated in a radio-residual tumour, radio­ recurrent tumour or where radiotherapy facilities are not available (Fig. 17.42). Reconstruction of the tongue can be done by nasolabial flap and division of pedical at later date. Radial forearm free flap can also be used. 4. Total glossectomy: Indications are similar to those mentioned above. However, very extensive growth involving the entire tongue are given radiotherapy initially, to reduce the size of the tumour. Surgery can then be undertaken. Total glossectomy carries significant mortality and morbidity.

Fig.17.43: Partial glossectomy done with the help of laser­ excellent tool for haemostasis (Courtesy: Dr Balakrishnan, Prof of ENT and Head and Neck, KMC, Manipal)

5. Commando's operation: This is indicated when carcinoma of tongue is fixed to the mandible with infiltration of the floor of the mouth. Hemiglossectomy with hemimandibulectomy, removal of the floor of the mouth and radical neck dissection is described as Commando's operation (Key Box 17.13). • However, in a few selected cases, removal of the haemimandible is not necessary. Growth which is close to the margin of the mandible without infiltration (confirmed by X-ray) needs to be treated by marginal mandibulectomy. Carcinoma of the tongue with involvement of only a small portion of mandible can be managed by segmental excision. Advantage of this method is that it is not only cosmetic but also preserves the function of the tongue by preserving genio­ glossus. Hence, the tongue may not fall backwards after surgery.

.,............ STRUCTURES REMOVED IN RADICAL BLOCK DISSECTION OF THE NECK

Fig.17.41: Partial glossectomy

Fig. 17.42: Hemiglossectomy

�

• The fat, fascia, lymphatics from midline to the anterior border of trapezius, from mandible to clavicle below. • The lymph nodes-submental, submandibular, upper and lower deep cervical nodes, posterior group of nodes (Levels 1-V). • Submandibular salivary gland, sternomastoid, one side internal jugular vein (IJV) are sacrificed • Spinal accessory nerve is removed • Lower pole of parotid is removed to facilitate lymph node clearance.

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Manipal Manual of Surgery External carotid artery

Internal carotid artery

Common--­ carotid artery

Superior thyroid artery

Fig. 17.45: Ligation of external carotid artery

CARCINOMA OF LIP Fig.17.44: Modified radical neck dissection is in progress-internal jugular vein, carotid artery and vagus nerve are seen

TREATMENT OF LYMPH NODES (Fig. 17.44) • Lymph node metastasis in the neck from squamous cell carcinoma can be managed both by surgery as well as radiotherapy. Radiotherapy can be given in all stages of secondaries in the neck. However, its main indication is a large primary tumour with neck nodes. In such situations both the primary and secondary can be managed with radiotherapy alone which carries minimal morbidity and mortality. • If the general condition of the patient is good and the lymph nodes are hard and mobile, hemiglossectomy with excision of the floor of the mouth with radical dissection of the neck is done (Commando's operation). • If radical neck dissection has to be done on both sides, the JJV should be preserved at least on one side to prevent cerebral oedema. In such cases, radiotherapy is a very good alternative.

•

Incidence of carcinoma of the lip is about 10 to 12%. lt is common in the western, elderly, white people, specially those exposed to sunlight. The actinic rays produces actinic cheilitis-inflammation of the lip, especially lower lip, which over a period of years can turn into malignancy. Since this is common in agriculturists, who are constantly exposed to sunlight, it is called Countryman's lip (Key Box 17.14).

............. COUNTRYMAN'S LIP

�

Sunlight J,

Actinic rays J,

Cheilitis J,

Erythema J,

Cracks

Please note: Details about radical neck dissection is given in Chapter 16, page 267.

Causes of death in carcinoma tongue l . Recurrent aspirational pneumonia 2. Gross local recurrence, fungation, ulceration, cachexia. 3. Uncontrolled haemorrhage from growth: In such cases ligation of external carotid artery above superior thyroid branch should be done (Fig. 17.45). If ligature is applied below the origin of superior thyroid artery, it results in eddy currents and thrombus at bifurcation of common carotid artery.

Fig. 17.46: Advanced carcinoma lip-a proliferative lesion, treated with radiation

Oral Cavity, Odontomes, Lip and Palate

Fig. 17.47: Carcinoma lip-everted edge and exophytic growth-also observe coating of the tongue

• • •

Fig. 17.48: Carcinoma lip with infiltration of buccal mucosa. Requires lip and cheek reconstruction

Carcinoma lip includes growth arising from vermilion surfaces and mucosa. Leukoplakia is also responsible for squamous cell carcinoma. Smoking, spirits and spices are the common precipitating factors. Genetic factors also may play a role. Blacks are less susceptible. On the other hand, increased incidence of carcinoma lip has been found in Caucasians. Khaini chewers are more susceptible for carcinoma of the lip (khaini is a mixture of tobacco and lime). It can also present as ven-ucous carcinoma of lip.

Clinical features (Figs 17.46 to 17.50) Elderly males are affected in 90% of cases. Nonhealing ulcer or growth is a common presentation. Edge is everted and indurated. Induration of the edge and the base is characteristic (Fig. 17.47). • Floor is covered with slough. Bleeding spots may be visible. • Mobility: Ulcer or the growth moves with the lip, it is fixed to the subcutaneous structures of the lip. The entire upper lip and lateral portions of the lower lip drain into upper deep cervical nodes. Central portion of the lower lip drains to submental nodes and submandibular nodes. Like elsewhere in the oral cavity, in 50% of the cases, nodes are enlarged due to secondary infections. In remaining 50% of the cases, they are enlarged due to

Fig. 17.50: Very large lesion started in the upper lip-later involving the lower lip also-case of kiss cancer

287

Fig. 17.49: A bleeding exophytic lesion. Better managed by surgery than radio­ therapy

metastasis. Such nodes are hard, with or without fixity. Blood spread is uncommon. Differential diagnosis (Key Box 17.15)

MH...........W:� • • • •

DIFFERENTIAL DIAGNOSIS OF CARCINOMA LIP Keratoacanthoma Ectopic salivary gland tumour Pyogenic granuloma Leukoplakia.

In a classical case of carcinoma of the lip with everted edges and induration, there is no differential diagnosis. However, following are a few conditions to be remembered: 1. Keratoacanthoma • It is a cutaneous tumour arising from hair follicles on the lips. It is common in white, western, males between 50 and 70 years of age. • Sunlight (actinic rays), chemical carcinogen, viral factors may be responsible for this lesion. • The central portion of the nodule may ulcerate. The lesion may progress for 6 weeks and may resolve spontaneously within 4-6 months. 2. Ectopic salivary gland tumour • The lip is one of the common sites of malignant salivary gland tumours. This presents with submucous nodules that grow slowly and ulcerate and may mimic squamous cell carcinoma (Fig.17.51). • They are also indurated lesions. • However, the characteristic everted edge may not be seen. • These are adenocarcinomas which are treated by surgery. 3. Pyogenic granuloma (Fig. 17.52) • Recun-ent infections or trauma produces a polypoidal mass with significant bleeding. • It is rich in granulation tissue and resembles a polyp. • It is devoid of epithelium • Histologically, it is a capillary haemangioma • Absence of induration gives the diagnosis.

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Fig. 17.51: This lesion was diagnosed as carcinoma lip. However, it did not have everted edges. It was indurated. Biopsy reported as ectopic salivary gland tumour. On careful questioning, patient says it started as a swelling not as an ulcer

Fig. 17.52: Pyogenic granuloma due to caries tooth (Courtesy: Dr Keerthilatha Pai, HOD, Department of Oral Medicine, College of Dental Sciences, Manipal)

I. Surgery • T l and T2 lesions can be excised followed by direct suturing without much functional problems. This is described as "V" excision which includes removal of growth with I cm healthy margin. Care should be taken to excise full thickness of the lip. • When removal of more than 113rd of the lip is required, flap reconstruction may be necessary. The primary goal in lip reconstruction surgery is oral competence. Examples I. Abbe flap: Based on upper labial artery-a pedicled flap is rotated down and sutured to the defect at the lower lip (Fig. 17.55). 2. Estlander's flap: Wedge-shaped flap is used to reconstruct carcinoma of lower lip, when it involves the angle (Fig. 17.54). Larger tumours: T3 and T4 lesions are irradiated first. If the tumour persists after radiotherapy, excision of the entire lip may be necessary followed by PMMC flap reconstruction. • Significant lymph nodes can be removed along with the primary tumour-supraomohyoid block dissection. II. Radiotherapy It is indicated in all stages of carcinoma of the lip. Radio­ therapy produces tumour necrosis resulting in a slow healing rate. Treatment lasts for several weeks and it delays the wound healing. Elderly patients who are not fit for surgery and carcinoma lip with fixed nodes are treated by irradiation. • Commissure involvement is treated with RT than surgery. • Dose: 4000-6000 centigray (cGy) units

4. Leukoplakia A slow developing leukoplakia presents as whitish nodule or an ulcer. However, biopsy confinns the diagnosis.

Reconstruction of the lips • There are various methods available to reconstruct the lip Up to I /3rd of the lip can be sacrificed with direct closure Details are given in page 289 and summary (Table 17.2).

Treatment Surgery and radiotherapy are the two modalities available for the treatment of carcinoma of the lip.

Please note: Students are advised to refer plastic surgery books for more details. Knowledge of some of these flaps will help you in getting more marks in the examinations.

Reconstruction following resection of oral malignancy. Ca - carcinoma Anatomic site of cancer I. 2. 3. 4. 5. 6. 7. 8.

Ca lip(less than 2 cm) Ca lip (large lesion) Advanced Ca lip Small lesions lateral tongue Large defect tongue(> 2 cm) Advanced lesions tongue Floor of mouth Tl or T2 Floor of mouthT3 or T4

9. Buccal mucosa 10. Lower alveolus

Surgery

Reconstruction

Vor W excision Wide excision Total excision of lip(chin also) Partial glossectomy Partial glossectomy Total glossectomy Wide excision Wide excision with removal of anterior mandible

Direct closure Bernard rotational flap; Advancement flap Total lip reconstruction by using forearm flap Primary closure or secondary healing Reconstruction by radial forearm flap Reconstruction by using rectus abdominus flap Radial artery forearm flap Fibula flap or iliac crest graft-deep circumflex iliac artery (DCIA) is used Direct closure or radial artery forearm flap

Wide excision with removal of underlying buccinator muscle also Wide excision with segmental resection of mandible

Primary reconstruction with fibula flap for edentulous mandible and DClA for dentate mandible.

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LIP RECONSTRUCTION (Figs 17.53 to 17.58)

(• }

Fig. 17.53: Karapandzic flap-used for defect up to 213rd of lip size. Good functional result, good colour and texture match

Fig. 17.54: Estlander flap. Used for lateral lip defects up to 113rd size. Good functional result

Fig. 17.55: Abbe's flap. Used for up to 1 /3rd lip defect. Can be modified for both upper and lower lip. Produces good functional and cosmetic result

Figs 17.56 to 17.58: Wide excision of lip followed by reconstruction using nasolabial flap (Courtesy: Dr Biswas, Dr JK Jha, Dr SP Nayak, Chittaranjan National Cancer Institute (CNCI), Kolkata)

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OHNGREN's line • Antral carcinoma can be divided by a line joining medial canthus of eye to the angle of mandible. This line is called as Ohngren's line • The region above the plane is suprastructure and one below is tenned infrastructure • Poor prognosis in suprastructure because of proximity to skull base and pterygoid region • These suprastructure lesions as usually inaccessible and unresectable. CARCINOMA MAXILLARY ANTRUM (Key Box 17.16) It is rare in western countries but common in Asia. The workers in furniture industries, chromic and nickel industries are more prone for the development of carcinoma maxillary antrum. Clinical presentation (Table 17.3) I. Growth originating on the floor of antrum may result in bulge of the hard palate. This results in pain in the teeth and they may become loose. 2. When medial wall is involved, nasal obstruction and epiphora occurs due to obstruction of the lacrimal duct. Bleeding from the nose can also occur, if there is ulceration. 3. If anterolateral wall is involved, asymmetry of the face results in pain in the cheek. Anaesthesia over the skin of the cheek including upper lip occurs due to involvement of infraorbital nerve, a branch of maxillary division of the trigeminal nerve. 4. If the roof is invaded, proptosis and diplopia occurs. 5. Posterior extension of the growth is difficult to assess clinically. When it involves the pterygoid muscles, it results in trismus. Paraesthesia over the cheek, gums, lower lip, postnasal discharge are the other features of these tumours. They carry poor prognosis because of late presentation.

Fig. 17.59: Maxillary antral carcinoma

Lymphatic spread • Nodal metastasis are uncommon in sinonasal malignancies. Investigations 1. Computed tomography (CT scan) can define a lesion, its extent, bony destruction, posterior extension, etc. Hence, it is the first investigation of choice.

•,............. CARCINOMA MAXILLARY ANTRUM

�

• Majority are squamous cell carcinoma

• Most important method of spread is contiguous spread • Early presentation is rare because maxillary antrum serves no important function • Tissue diagnosis is by biopsy of the mass protruding through nasal cavity/oral cavity or transnasal needle through medial wall of the maxilla. • CT scan for bone involvement and MRI for soft tissue involvement are investigations of choice

Clinical examination of a case of carcinoma maxillary antrum • Palatine bulge

"'7 Growth in the floor

• Unilateral nasal obstruction (He is asked to breathe, closing the nostrils one by one)

"'7 Growth in the medial wall

• Asymmetry of the face

"'7 Growth expanding anteriorly, superficial surface

• Change in the level and sharpness of inferior orbital margin, proptosis "'7 Growth on the roof (orbital surface) • Swelling (indurated) temporal region

"'7 Posterior extension into infratemporal region and then into temporal region.

• Hard, fixed or mobile submandibular nodes

"'7 Metastasis

Oral Cavity, Odontomes, Lip and Palate 2. Sinoscopy: Fenestration will provide tissue for biopsy followed by curettage to reduce the tumour bulk and to drain necrotic contents outside. Treatment • Radiotherapy is the main modality of treatment in carcinoma maxillary antrum. Curative rate is around 70% in early cases. In advanced cases, radiotherapy is given first. This reduces the tumour bulk so that an unresectable lesion becomes resectable and maxillectomy can be done. • Surgery can be done in the form of total maxillectomy when the growth involves entire maxilla or it is of high grade followed by postoperative radiotherapy. • Tumours of the lower half of the antrum are treated by partial maxillectomy. It includes removal of the entire hard palate, alveolus and medial wall of the antrum up to and including middle turbinate. Indications and contraindications for surgery and radiotherapy are in similar lines as discussed earlier in this chapter. NASOPHARYNX-CANCER •

Carcinoma arises in a small anatomic site bordered by nasal fossae, posterior wall continuous with posterior wall of oropharynx, body of the sphenoid and basilar part of occipital bone and soft palate. • > 90% are squamous cell carcinoma out of which 40-50% are undifferentiated (lymphoepithelioma) and 5% are lymphomas. Incidence is higher in Asia-Southern China, Malaysia, etc. May be associated with Epstein-Barr virus (EBV) • Present as high posterior cervical lymphadenopathy • Cranial nerve syndromes are common due to tumour invasion of base of skull. 1. Retrosphenoidal syndrome from involvement of cranial nerves II through VI manifests as unilateral ophthalmoplegia, trigeminal neuralgia, ptosis, etc.

2. Retroparotid syndrome: Occurs due to compression of cranial nerve IX through XII and causes various symptoms depending on nerve involvement including Homer's syndrome. • Diagnosis by endoscopy, biopsy, CT and MRI. • Mainly managed by radiotherapy and chemotherapy BENIGN LESIONS IN THE ORAL CAVITY Differential diagnosis of ulcer in the tongue (Fig. 17.60 and Key Boxes 17.17 and 17.18) 1. Aphthous ulcer • Small, multiple, very painful ulcers, can occur at any age group. More common in females at the time of menstruation. These are called as minor aphthous ulcers. • When they are larger, deeper and painful, they are called major aphthous ulcers. • They are due to viral infection. These ulcers are super­ ficial ulcers with erythematous margin. • They subside within a few days. Temporary relief can be obtained by applying salicylate gel. • Vitamin B complex is usually given for the satisfaction of the patients. 2. Dental ulcer • These ulcers occur due to broken tooth, sharp tooth, ill­ fitting dentures, prosthesis, etc. They are very painful ulcers. • Such ulcers are common on the lateral margin and they heal when the tooth is removed. This is an example for traumatic u lcer. It should not be confused with carcinomatous ulcer which commonly occurs on the lateral margin.

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11¥111..........� PAINFUL ULCERS IN THE TONGUE • Aphthous ulcers • Dental ulcers • Tubercular ulcers

.,•...........,.� PAINLESS ULCERS IN THE TONGUE • Carcinomatous ulcers • Gummatous ulcers • Systemic diseases

291

® ® Sharp tooth

--(f)

---® ----0)

Fig. 17.60: Ulcers of the tongue-see text for numbers

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3. Tubercular ulcer of tongue • Tuberculosis affects tip of the tongue. These ulcers are very painful with enlargement of regional nodes. • It occurs in patients with fulminating pulmonary tuberculosis. • Ulcers have undermined edges. These ulcers are sometimes multiple with serous discharge. 4. Gummatous ulcer • Gumma is a complication of tertiary syphilis resulting in a firm swelling in the midline in the anterior 213rd of the tongue. Induration is absent. Ulcer is nontender. Severe endarteritis obliterans results in the necrosis of gumma giving rise to gumrnatous ulcer. It has punched out edges and wash leather slough on the floor. Other sites of gumma include testis, palate, clavicle and liver. These ulcers are rare these days. 5. Systemic diseases • Pemphigus • Systemic lupus erythematosus (SLE) • Lichen planus 6. Post-pertussis ulcer • It occurs in children due to repeated coughing. Typical location of the uleer on the under surface of the tongue and on the frenulum clinches the diagnosis. 7. Carcinomatous ulcer • Lateral margin • Nonhealing ulcer • Everted edge • Edge and base are indurated • Bleeds on touch • Fixity • Significant lymph nodes in the neck. MACROGLOSSIA Diffuse painless enlargement of the tongue is described as macroglossia. It is a rare condition and can occur due to various causes. l. Lymphangioma: In this condition, the tongue diffusely enlarges. Sometimes, it is a localised swelling. It may be associated with lymphangiomas elsewhere in the body such as cheek mucosa, lips, etc. The tongue becomes larger, indurated and gives rise to severe discomfort to the patients. Due to repeated trauma, the surface becomes ulcerated. Tt is treated by injecting sclerosants such as ethanolamine oleate, hypertonic saline. Partial excision may be necessary in cases of large lymphangioma. 2. Haemangioma: Cavernous haemangiomas occur in the tongue, lips, etc. It is present since bi1th but manifests during childhood. It presents with soft, cystic, fluctuant swelling and at times, pulsatile. Trauma due to teeth or food results

in bleeding. Haemangioma of the tongue is treated on the same lines as lymphangioma. It is much more difficult to excise it, especially a large haemangioma. Preoperative angiography and ligation of lingual artery on both sides is necessary. 3. Neurofibroma: It may be associated with von Recklinghausen's disease. It is treated by hemiglossectomy. 4. Muscular macroglossia: This condition, though rare, is seen in cretins. The tongue is thickened and cannot be held in place. Hence, it protrudes outside. It is treated by partial exc1s1on. SYPHILITIC LESIONS OF THE TONGUE 1. Primary syphilis: Primary chancre that occurs in the tongue is highly contagious. It affects the tip of the tongue. It produces a painful ulcer with large significant enlargement of regional lymph nodes. 2. Secondary syphilis produces a white patch in the tongue, lips and on the anterior pillars of fauces. In the tongue, these are multiple which coalesce to form snail track ulcers. The ulcers heal with fine tissue paper scar. In some cases syphilitic organisms produce a flat, hypertrophied epithelium which is described as condyloma. This is called as Hutchinson's wart. 3. Tertiary syphilis: It produces gumma. Syphilis also produces chronic superficial glossitis, which is characterised by bald tongue with loss of papilla and fissured tongue. It is a precancerous condition. ODONTOMES Definition: Odontomes are the cysts, malformations arising from epithelial or mesothelial elements of tooth resulting in swelling of the jaw. As a developmental anomaly, few epithelial cells proliferate, persisting as epithelial debris of Mallasez. Three important odontomes have been given in Table 17.4. DENTAL CYST: RADICULAR CYST/PERIAPICAL CYST Pathogenesis This arises from a normally erupted, chronically infected, pulpless caries tooth. The caries tooth produces a low grade, chronic inflammation which stimulates epithelial debris to proliferate. Later this brings about degeneration of epithelial and mesothelial cells resulting in a cyst within the maxilla. Clinical features • Common in women around 3rd-4th decades • Commonly affects the upper jaw (maxilla)

Oral Cavity, Odontomes, Lip and Palate

• It presents as a slow-growing swelling in the maxillary region resulting in deformity of the face. Diagnosis • Presence of caries tooth with expansion of maxilla • X-ray-large, unilocular cyst in maxilla or orthopantomogram showing cyst in the mandible • Aspiration of the cyst demonstrates cholesterol crystals. Treatment Excision of cyst with its epithelial lining through intraoral approach. After excision of the epithelium the cyst wall should be curetted, followed by soft tissue 'push-in' to obliterate dead space. DENTIGEROUS CYST: FOLLICULAR ODONTOME • Common in lower jaw (mandible) in women 30-40 years (Fig. 17.61). • It occurs in relation to unerupted, permanent, molar tooth, most commonly the upper or lower third molar tooth. • This unerupted tooth constantly irritates the cells, produces degeneration of the cells resulting in a dentigerous cyst. The cyst is lined by squamous epithelium surrounded by connective tissue. Within the cyst, the tooth lies obliquely or sometimes is embedded in the wall of the cyst. As it grows further, the cyst displaces the tooth to which it is attached. Thus, the tooth is displaced deeper and deeper and prevented from eruption. Clinical features • Absence of molar tooth

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• Expansion of mandible-since the inner table of the mandible is strong, the expansion mainly occurs in the outer aspect of mandible. The bone gets thinned out resulting in egg-shell crackling. Diagnosis X-ray mandible (Figs 17.62 and 17.63) I. Tooth in the cyst 2. Soap-bubble appearance due to multiple trabeculations of the bone 3. Radiolucent well-defined swelling. Treatment • Small cyst-excision of the cyst by intraoral approach. • Large cysts-managed by marsupialisation. ADAMANTINOMA • It is also called multilocular cystic disease, ameloblastoma, Eve's disease (Fig. 17.64). • This tumour arises from ameloblasts (enamel f01ming cells). • It is a benign tumour, very slow-growing and behaves like a basal cell carcinoma. Inadequate treatment results in local recurrence and later metastasis, Hence, even though the tumour is benign, it has to be treated like malignant tumour. Sites • Mandible is the most common site • Tibia is the 2nd common site. It can be explained by inclusion of abnormal embryonic epithelium. • Pituitary is another common site where adamantinoma can occur. Both pituitary stalk and enamel arise from oral epithelium. Clinical features • Patients in 4th or 5th decade are commonly affected. • This is a slow growing jaw tumour in the region of angle of mandible and horizontal ramus of the mandible.

Fig. 17.61: Dentigerous cyst

Fig. 17.62: X-ray of the denti­ gerous cyst

Fig. 17.63: Orthopantomogram showing unerupted tooth

Fig. 17.64: Adamantinoma left jaw

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• As the tumour grows, it undergoes cystic degeneration resulting in multiple cystic spaces. Hence, it is called multilocular cystic disease. • As it grows it causes expansion of the outer table of the mandible and causes fracture mandible. • Patient may present with complaints of falling teeth. Diagnosis X-ray: A large cyst and small multiple cysts due to the trabeculations giving it a 'honeycomb' appearance. Treatment • Even though it is benign, simple curettage or enucleation may result in recurrence and chances of recurrent adamantinoma turning into malignancy are high. Hence, wide excision with 1 cm of healthy normal tissue should be removed. It may amount to segmental excision of the mandible or hemimandibulectomy (Key Box 17.19 and Table 17.4).

............. ADAMANTINOMA

�

• Locally invasive solid tumour • Nonfunctiona l, intermittent in growth, unicentric, multilocular

• • • •

Spreads within the medullary bone Invades soft tissues Should not fragment the tumour cells Subperiosteal excision should not be done as it may result in recurrence • Incomplete excision results in recurrence and metastasis to the lung • Hence, even though it is a benign tumour it is treated by wide excision or hemimandibulectomy.

Differential diagnosis 1. Giant celled reparative granuloma (Jaffe tumour It is a benign tumour which occurs due to haemorrhage withii the bone marrow. Pathology • It affects antral part of maxilla or mandible causin1 enlargement of the jaw. Stroma is vascular consisting of thin-walled blood vessels scanty collagen, connective tissue cells. Microscopic features mimic giant cell epulis or browr tumour of hyperparathyroidism. Clinical features Unlike an adamantinoma, this tumour affects females ir the age group of 10-25 years. Painless enlargement of the jaw is the presenting feature X-ray demonstrates radiolucent artery. Treatment Calcitonin 0.5 mg (100 units) daily subcutaneous injection over a period of one year has been recommended as a firs1 line of treatment. It has shown resolution of the tumour. Curettage is the surgical line of treatment. 2. Osteoclastoma • This is a rare tumour seen in the lower jaw. • Males between the age of25 and 40 years are commonly affected. Unlike adamantinoma, it is a rapidly growing tumour. As the tumour enlarges, both tables of the lower jaw are thinned out. X-ray may show a large, radiolucent cyst with pseudo­ trabeculation. Even though benign, it is radiosensitive. However, recurrence can occur and can turn into malignancy like that of adamantinoma.

Comparison of three common odontomes I. 2. 3. 4.

Aetiology Site Age Palpation

Dental cyst

Dentigerous cyst

Adamantinoma

Caries tooth Maxilla 30-50 years Smooth, thin bone-eggshell crackling

Unerupted tooth Mandible 20-30 years Smooth, expansion of the outer table of mandible Radiolucent unilocular cyst with unerupted teeth Subperiosteal excision Does not occur

True neoplasm-ameloblasts Mandible 40-50 years Expansion of mandible in the 3rd molar region, cystic areas Large radiolucent area with fine honey­ combing Wide excision even though benign Local spread, recurrence, late spread to the lung are the features

5. X-ray

Radiolucent unilocular cyst

6. Treatment 7. Spread

Subperiosteal excision Does not occur

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EPULIS •

Epulis means "upon the gum". It refers to solid swelling situated on the gum (Key Box 17.20). It arises from alveolar margin of the jaw.

•

Very often patients present with swelling on the gum which is painless.

Types Granulomatous epulis (Fig. 17.65) Precipitating factors are caries tooth, dentures, poor oral hygiene. It manifests as a mass of granulation tissue around the teeth on the gums. It is a soft to firm, fleshy mass and bleeds on touch. •

Pregnancy epulis refers to this variety (gingivitis gravidarum).

Fibrous epulis (Fig. 17 .66) It is the commonest form. A simple fibroma arising from periodontal membrane, presents on the gum. It may undergo sarcomatous change. It is a firm polypoidal mass, slowly growing and nontender. Giant cell epulis It is also called myeloid epulis. It is an osteoclastoma arising in the jaw. It presents as hyperaemic vascular, oedematous, soft to firm gums with indurated underlying mass due to expansion of the bone. It may ulcerate and result in haemorrhage. X-ray shows bone destruction with ridging of walls (pseudo­ trabeculation). Small tumours are treated by curettage. Large tumours are treated by radical excision.

Fig. 17.65: Granulomatous epulis

MltMiftWllir,J -

• Soft epulis • Firm epulis • Hard epulis • Malignant epulis • Dangerous epulis

-

EPULIS Granulomatous Fibrous Giant cell Carcinomatous Carcinomatous Fibrosarcomatous

�

Carcinomatous epulis This is an epithelioma arising from mucous membrane of the alveolar margin. • Typically, it presents as a nonhealing, painless ulcer. It slowly infiltrates the bone. Hard regional lymph nodes are due to metastasis. Treated by wide excision which includes removal of segment of the bone. MEDIAN MENTAL SINUS This is a sinus in the midline just beneath the mentum. Aetiopathogenesis It is produced by an apical abscess of lower incisors which penetrate buccal c01tical plate below the origin of mentalis muscle. This muscle takes origin from labial surface of alveolar process just above the labial sulcus. Hence, pus discharges through a sinus in the centre of chin. Clinical presentation (Fig. 17.67) Patients present with recurrent swelling in the submental region which bursts open spontaneously discharging at times mucus and seropurulent fluid. Repeated history of swelling, discharge and healing are common presentations.

Fig. 17.66: Fibrous epulis

Fig. 17.67: Median mental sinus

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CLEFT LIP AND CLEFT PALATE We had a patient with discharging sinus in the region of mentum for 1 � years who had seen many practitioners including a surgeon who curetted the lesion twice. Howeve,; the lesion reappeared soon. This case is a classical example of what the mind does not know, eyes cannot see.

Diagnosis is established by examination of the oral cavity. which reveals evidence of caries tooth. Treatment Once the caries tooth is extracted, sinus will heal sponta­ neously. VINCENT'S ANGINA It is an acute ulceromembranous stomatitis or acute ulcerative gingivitis and stomatitis. The disease is caused by Vincent's organisms-Borre/fa vincentii, an anaerobic spirochaete and fusiformis. These are gram-negative rods which are the normal pathogens of oral cavity. Precipitating factors Malnutrition, diabetes mellitus, caries tooth, wam1 seasons, winter, etc.

CLEFT LIP Cleft lip results from abnormal development of the mediar nasal and maxillary process. Cleft palate results from a failure of fusion of the twc palatine processes. Types of cleft lip (Figs 17.68 and 17.69) l. Central: It is very rare and occurs due to failure of fusior of two median nasal processes. II. Lateral: It is the commonest variety wherein there is a cleft between the frenulum and the lateral part of the upper lip. This is due to imperfect fusion of maxillary process with median nasal process. The lateral variety can be unilateral or bilateral. ill. Complete or incomplete: In cases of complete variety, cleft lip extends to the floor of the nose. In cases of incomplete variety, the cleft does not extend up to the nostril. IV. Simple or compound: Compound refers to cleft lip associated with a cleft in the alveolus. Clinical features (Figs 17.70 and 17.71) 1. In 80% of the cases, cleft lip is unilateral and in about 60% of the cases it is associated with cleft palate.

The disease starts in the intergingival defects as a deep penetrating ulcer which results in a spontaneous gingival haemorrhage. There is a thick membrane covering the ulcer. Once infection spreads to tonsillar region, it is called as Vincent's angina-very severe painful condition. Clinical features Common in children and young adults between 20 and 40 years of age. It presents with very painful gums with fever, malaise and toxaemia. Gums are swollen, red, inflamed with or without slough. Difficulty in swallowing, painful swallowing ( odynophagia), foetor oris, features of toxaemia and high grade fever are characteristic of this condition. Treatment Improve nutrition. Mouth washes with hydrogen peroxide help in washing away the membrane. Injection Penicillin: 10 lakh units, IM 6th hourly for 6-7 days. Since they are anaerobic organisms, metronidazole 400 mg thrice/day for 7-10 days, should be given.

Figs 17.68 and 17.69: Types of cleft lip (I and II)

Figs 17.70 and 17.71: Cleft lip ( Courtesy: Dr CG Narasimhan, Senior Consultant Surgeon, Mysore, Karnataka)

Oral Cavity, Odontomes, Lip and Palate

2. In many cases, nostril is widened. 3. Maldevelopment or malalignment of the teeth in relation to the cleft is common. Functional effect 1. Presence of cleft lip does not interfere much with suck­ ing. However, there may be some difficulty in bottle feeding. 2. Some degree of difficulty in speech (disarticulation) is present. CLEFT PALATE Development of palate • Palate is developed around 6-8 weeks of intrauterine life from 3 components. The premaxilla is developed from the median nasal process and maxillary process contributes one palatine process on each side. • The line of fusion of these processes is in the form of a letter Y. • Imperfect fusion or developmental anomalies results in cleft palate. Types (Fig. 17.72) I. Complete: Failure of fusion of palatine processes and premaxilla results in complete cleft palate. In such situations, the nasal cavity and mouth are interconnected. When premaxilla is not fused with both palatine processes, it hangs down from the septum of nose. Thus, complete cleft can be of two types as shown below in the diagram. II. Incomplete: When the fusion of three components of palate takes place, it staits from uvula and then backwards. Thus, various types of incomplete fusion results. • Bifid uvula. • The whole length of soft palate is bifid. • The whole length of soft palate and the posterior part of hard palate are involved. On the other hand, anterior part of palate is normally developed. In about 25% of cases, cleft palate alone and in 50% of cases, both cleft palate and cleft lip are encountered.

Fig. 17.72: Types of cleft palate (I, Ila and llb)

297

Effects of cleft palate I. Presence of cleft palate interferes with swallowing to some extent. 2. They are unable to pronounce the consonant sounds such as B, D, K, P, T. 3. Teeth: Upper lateral incisors may be small or even absent. The maxilla tends to be smaller. Teeth are crowded. 4. Nose: Oral organisms contaminate the upper respiratory mucous membrane through cleft palate. 5. Hearing: Even with repair, acute and chronic otitis media and hearing problems can occur. Management of cleft lip and palate • A multidisciplinary approach involving plastic surgery, orthodontics, speech pathology, ENT department, prosthodontics and paediatrics department is needed to rehabilitate the cleft palate cases. This approach to the problem results in aesthetically acceptable end result without much functional deficiencies. • Feeding advice: Cleft palate babies are unable to suck mothers' milk because intraoral negative pressure cannot be created due to communication between oral and nasal cavity. Thus, expressed mother's milk is given by spoon with head end of baby elevated by 45 degrees. Swallowed air during feeding is released frequently by burping. Cleft lip repair (Key Box 17.21) Timing: Majority of surgeons follow "RULE OF 1O" as a guide for timing of lip and anterior palate repair. At the time of repair, haemoglobin should be more than IO g%, age approximately 10 weeks, weight more than l O lb (4.54 kg) and total leukocyte count less than I 0,000/cu 111111 (i.e. no infection).

MIMIWlllllllll1IW� • • • •

RULE OF 10 Hb > 10 g% Age approx : 10 weeks Weight > 10 lb (4.54 kg) TC < 1 O,OOO/mm3

Types of cleft lip repair • For unilateral cleft lip repair most commonly used methods are Millard rotation advancement flap and Tennison-Randall triangular flap method. • Bilateral cleft lip can be repaired in single stage or in two stages at the interval of 3-6 months. For two stage repair, any one of the methods described for unilateral cleft lip can be used. For bilateral repair in one stage, Veau Ill method is simple and gives satisfactory results. Other single stage methods which give good results are Millard's single stage procedure and Black procedure.

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Figs 17.73A and B: Basic steps of lip repair Basic steps of lip repair Markings are made according to the method selected (e.g. Millard's repair, Fig. 17. 73A and Tennison-Randall repair, Fig. 17.73B). Adrenaline-saline solution (1:200,000) is injected in the lip and labial sulcus for haemostasis. •

Full thickness of lip is incised along the marking. Lip repair is done in three layers-mucosa, muscle and skin. For better aesthetic result, Cupid's bow should become horizontal, white line continuity should be repaired and there should be no vermilion notching.

Cleft palate repair Timing: Early repair results in retarded maxillary growth due to surgical trauma to growth centre and periosteum. Delay in repair results in speech defect. Best balanced result is achieved by repairing between one and a half years. Types • Palate repair: Palate is repaired by palatal shelves. Mucoperiosteal flaps raised from various methods are available for palate repair. Most commonly used method is V-Y, pushback palato­ plasty. Steps of 'V-Y' pushback palatoplasty (Fig. 17.74) Palate is infiltrated with I : 2 lakh adrenaline-saline solution. • Two mucoperiosteal flaps are elevated, one from either side of palatal shelves. Then, nasal layers are mobilised. Palate is closed in three layers-nasal layer, muscle layer, oral layer. • In V-Y push back palatoplasty, palatal lengthening is achieved by V-Y plasty. Hook of hamulus can be fractured to relieve tension on suture line by relaxing the tensor palati muscle.

Fig. 17.74: V-Y palatoplasty MISCELLANEOUS ECTOPIC SALIVARY GLAND TUMOUR • Palate is the most frequent site • These tumours can also occur anywhere in the mouth or pharynx • It is a slow-growing and painless tumour. At this stage, it may feel firm or hard without ulceration of mucous membrane. • Slowly it ulcerates. Thus, it can have the shape of a 'verrucous' carcinoma. Such lesions are ulcerated, hard, and painful with irregular margins. • They are of low-grade malignancy. • Neglected cases can invade base of skull and spread to lymph nodes. • Wide excision with or without reconstruction is the treatment of choice. MUCOUS CYSTS • They are examples of retention cyst • They occur due to obstruction of the duct of many mucous secreting glands which cover the inner surface of the lips and whole of inside of mouth (Fig. 17.75). • They are also a type of extravasation cyst. Clinical symptoms and signs • Painless, slow growing swelling on the inner side of lip or cheek. • Most common on the lower lip • Typically round, soft, fluctuant, pale pink swelling or blue domed. • Transillumination is positive but difficult to demonstrate as the cysts are small. • Mucous membrane is free over the swelling.

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299

A case of malignant melanoma of the oral cavity (see the clinical notes below)

This 48-year-old man (Figs 17.76 to 17.78) presented with an innocent looking, painless submandibular lymph node enlargement. It was firm and nontender. Initially, it was thought to be due to dental caries. Examination of the floor of the mouth revealed an interesting me/anomalous lesion. Diagnosis was malignant melanoma with metastasis in nodes. This is just to remind that melanoma can also occur in the oral cavity (mucosa and firm skin). Fig. 17.75: Mucous cyst in the floor of the mouth

Differential diagnosis • Pyogenic granuloma: It is red in colour, soft and bleeds. It may be associated with trauma or persistent infection. • Ectopic salivary gland tumours: They are firm and non­ tender swellings. Treatment • Excision can be done under local anaesthesia • Once mucous membrane is incised, swelling can be dissected all around separating it from orbicularis oris/ buccinator muscle and it is removed. • The mucous membrane is closed with absorbable sutures.

Fig. 17.76: This patient presen­

ted with right submandibular lymph node enlargement

Fig. 17.77: Oral cavity exami­ nation reveals a pigmented ulcerated lesion

Fig. 17.78: Closeup view reveals it is a malignant melanoma WHAT IS NEW IN THIS CHAPTER?/ RECENT ADVANCES

• All the topics have been updated • New photographs and key boxes have been added • Reconstruction of the oral cavity has been dealt in detail including lip • PMMC flap has been discussed in more detail.

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MULTIPLE CHOICE QUESTIONS 1. Which one of the following has highest chance of malignancy? B. Dyskeratosis A. Acanthosis D. Speckled leukoplakia C. Leukoplakia 2. Drug used to treat leukoplakia is: B. Isonicotonic acid A. Vitamin E C. Isotretinoin D. Niacin

10. Which is the most effective chemotherapeutic drug fm head and neck cancers? A. Carboplatins B. 5-Fluorouracil C. Bleomycin D. Cisplatin

3. Following are true for verrucous carcinoma except: A. Very slow growing B. Poorly differentiated carcinoma C. Spread by lymphatics is not common D. Surgery is the best treatment

11. Best treatment of carcinoma buccal mucosa-Tl NO MO: A. Radiotherapy only 8. Radiotherapy followed by surgery C. Surgery only D. Surgery followed by radiotherapy

4. Carcinoma tongue spreads to following lymph nodes except: A. Submandibular B. Submental C. Jugulo-omohyoid D. Supraclavicular node

12. Khaini chewers more susceptible for development of which carcinoma? A. Carcinoma lip B. Cracinoma tongue C. Carcinoma buccal mucosa D. Carcinoma floor of the mouth

5. Following malignant lesions in the posterior third of the tongue can occur except: A. Squamous cell carcinoma B. Adenocarcinoma C. Lymphoepithelioma D. Sebaceous carcinoma

13. Estlander flap is used to cover: A. Central defects of lip-lower lip B. Central defects of lip-upper lip C. Lateral defects of more than 50% D. Lateral defects up to 30%

6. The investigation of choice in oral cancer to find out the skull base involvement is: A. CT scan B. Angiography C. MRI D. Ultrasound 7. Which cancer in the oral cavity-Tl NO MO - requires supraomohyoid block dissection? A. Carcinoma buccal mucosa B. Verrucous carcinoma lower lip C. Carcinoma upper lip D. Carcinoma floor of the mouth 8. Following are true for chronic hyperplastic candidiasis except: A. Invasion of Candida albicans B. Antifungal treatment helps C. High malignant potential D. Floor of the mouth is affected 9. Which one of the following condition has high incidence of distant spread? A. Carcinoma buccal mucosa B. Carcinoma tongue C. Carcinoma floor of the mouth D. Nasopharyngeal carcinoma

14. Following are true for precancerous lesions of lip/oral cavity except: A. Keratoacanthoma B. Leukoplakia C. Erythroplakia D. Submucous fibrosis 15. Following are features of carcinoma maxillary antrum except: A. Can cause asymmetry of face B. Can cause proptosis C. Can cause infraorbital nerve paralysis D. Can cause buccal branch of fascia! nerve paralysis 16. Following are the boundaries of nasopharyngeal space except: A. Nasal fossae B. Basilar part of occipital bone C. Body of sphenoid D. Cribriform plate of ethymoid bone 17. Following are true for nasopharyngeal carcinoma except: A. Presents as high anterior cervical lymphadenopathy B. Can present as trigeminal neuralgia C. Compression on IX and X cranial nerves D. Can present as ophthalmoplegia

Oral Cavity, Odontomes, Lip and Palate

18. Following are painless ulcers in the tongue except: A. Gummatous ulcers B. Carcinomatous ulcers C. Systemic diseases D. Tuberculous ulcers 19. Following are painful ulcers in the tongue except: A. Gummatous ulcers B. Tuberculous ulcers C. Aphthous ulcers D. Dental ulcers

301

22. Which one of the following swelling does not contain cholesterol crystals? A. Branchial cyst B. Sebaceous cyst C. Dental cyst D. Hydrocoele 23. Following are true for dentigerous cyst except: A. Upper jaw is commonly involved B. Produces egg shell crackling C. X-ray shows soap bubble appearance D. Arises from unerupted tooth

20. Following are true for syphilitic lesions of the tongue except: A. Snail track ulcers B. Gumma C. Hutchinson's wart D. Hunterian chancre

24. Adamantinoma of the jaw has following features except: A. It is a malignant tumour B. It spreads within medullary bone C. It is treated by wide excision D. Mandible is the most common site

21. Following are true for dental cyst except: A. Upper jaw is commonly involved B. It is a large unilocular cyst C. Cyst contains cholesterol crystals D. Arises from unerupted tooth

25. Which of the following is unilocular cyst? A. Adamantinoma B. Dentigerous cysts C. Epididymal cyst D. Dental cyst

ANSWERS 1 D 11 C 210

2 C

3 B

4 D

5 D

6C

7 D

8 D

9 D

10 D

16 D

17 A

18 D

19 A

20 D

12 A

13 D

14 A

15 D

22 B

23 A

24 A

25 D

18 Salivary Glands • • • • • •

Surgical anatomy Acute parotitis Submandibular sialoadenitis Salivary gland tumours Summary of malignant tumours Frey's syndrome

• • • • • •

Sjogren's syndrome Mikulicz disease Parotid fistula Surgery for facial nerve palsy Peripheral nerve repair and transfers What is new?/Recent advances

Introduction There are three pairs of salivary glands-parotid, submandibular and sublingual. In addition to these, there are many (450) minor salivary glands located in the cheek, mucosa, lips, palate and base of the tongue. Parotid, the "big brother of 3 ", suffers mainly from three diseases-infection, enlargement and tumour. Submandibular salivary gland suffers from mainly two diseases-sialoadenitis and tumours. Other salivary glands are of minor importance. However, it should be remembered that the commonest tumour of minor salivary glands is malignancy. Fig. 18.1: Two lobes of parotid gland

SURGICAL ANATOMY OF THE PAROTID GLAND Parotid gland is present on the lateral aspect of the face, divided by the facial nerve into superficial lobe and deep lobe. Superficial lobe overlies the masseter and the mandible. Deep lobe is wedged between the mastoid process and the styloid process, ramus of the mandible and medial pterygoid muscle. The superficial lobe also receives a duct from the accessory lobe which is in the region of zygomatic arch/zygomatic process. The duct of parotid, Stensen's duct, 2-3 mm in diameter, receives tributaries from superficial, deep and accessory lobes, passes through the buccinator muscle and opens in the mucosa of the cheek opposite the upper 2nd molar tooth. Parotid gland is covered by a true capsule which is a condensation of fibrous stroma of the gland and a false

capsule formed by parotid fascia, a part of the deep cervical fascia. Facial nerve After emerging from stylomastoid foramen, it hooks around the condyle of mandible, enters the substance of the parotid and divides into 2 major branches, zygomaticotemporal and cervicofacial. Facial nerve along with retromandibular vein (which is formed by the union of superficial temporal vein and maxillary vein, formed from branches of pterygoid plexus of veins) are present in this plane. This plane is called the fasciovenous plane of Patey (Figs 18.1 and 18.2). The facial nerve then gives rise to 5 branches which are interconnected

302

.,.,,.........

Salivary Glands Facial nerve and its branches Branches of facial nerve I. Temporal

Muscles supplied �

2. Zygomatic � upper lower 3. Buccal

� �

4. Mandibular � 5. Cervical �

Auricularis anterior and superior portion of frontalis Frontalis and upper half of orbicularis oculi Lower half of orbicularis oculi and muscles below the orbit Buccinator, orbicularis oris and a few fibres of elevators of the lower lip Muscles of the lower lip Platysma

CAUSES OF ACUTE PAROTlTIS

1. Viral

2. Bacterial 3. Recurrent parotitis of childhood 4. Specific infections 5. Allergic 6. Sexual diseases 7. Postradiation 8. Postoperative

303

�

Mumps-Commonest Coxsackie A and B Parainfluenzae 1 and 3 Echovirus Lymphocytic choriomeningitis Usually ascending infection Staphylococcus aureus Recurrent, mistaken for mumps Resolves at puberty

Mycobacterial, cat-scratch disease, syphilis, toxoplasmosis Food and drugs HIV-related Reduction in the salivary juice Due to dehydration.

Fig. 18.2: Surgical anatomy of the parotid gland

like the foot of a goose, called Pes anserinus. Branches of facial nerve in the face and the muscles supplied by these nerves are given in Table 18.1. ACUTE PAROTITIS Acute inflammation of the parotid can occur due to bacterial or nonbacterial causes. It can be unilateral or bilateral (Key Box 18.1). Three important causes and their treatment are given below: 1. Mumps parotitis: Mumps' is an acute generalised viral disease with painful enlargement of salivary glands, chiefly the parotids. The virus belongs to Paramyxoviridae family and only one serotype is known. The disease spreads from a human reservoir by direct contact, airborne droplets or fomites contaminated by saliva and possibly by urine (Fig. 18.3). Clinical manifestation • Incubation period is 10-24 days. Fever, headache and muscular pain are usually found. Both parotids are enlarged with pain and temperature. • Swelling starts subsiding by 3-7 days of time. 1 It causes parotitis, orchitis and pancreatitis.

Fig. 18.3: Acute parotitis due to viral infection

Treatment • If symptomatic: Maintenance of good oral hygiene and hydration is useful. Antibiotics may be given to prevent secondary infection. One episode of infection confers lifelong immunity. 2. Acute bacterial parotitis: Staphylococcus aureus infection of parotid produces serious illness with marked engorgement of parotid. Typically, it produces parotid abscess. Diabetes, malignancy, malnutrition increase the risk. Decreased salivary secretion is an important predisposing factor. 3. Reduction in salivary juice: It can occur due to various factors mentioned in the box. Postoperative parotitis can be prevented by good mouth care and good oral hygiene. Due to poor oral hygiene, ascending infection occurs from the oral cavity resulting in parotitis (Key Box 18.2).

304

M!M......W� • • • • •

CAUSES OF ..j, SALIVARY FLOW Postoperative Poor oral hygiene Dehydration Enteric fever, septicaemia Postradiotherapy, for oral cancer

.....,......... SWELLINGS WHEREIN ONE SHOULD NOT WAIT FOR FLUCTUATION Parotid abscess Breast abscess lschiorectal abscess Pulp space infection Any deep seated abscess

�

• • • •

Clinical features • A patient who is recovering in the postoperative period may complain of pain and swelling in the parotid region. Presence of severe pain with a very sick, toxic look and high grade fever, chills and rigors indicates parotid abscess. Diffuse brawny swelling is characteristic. • The swelling is due to inflammation of parotid and since it is enclosed by parotid fascia, the swelling takes the shape of parotid gland. However, it is not common for a parotid abscess to raise the ear lobule. For the reason mentioned above, fluctuation is a late feature. If the abscess is not drained, it is likely to rupture into the external auditory canal (Key Box 18.3). • The opening of the parotid duct may be inflamed and on gentle compression of the parotid gland, pus can be seen coming out of the parotid duct.

• • • • •

•..,.,...........

Manipal Manual of Surgery

�

Treatment I. Conservative line of management • Indicated in a stage of cellulitis with no abscess. • Maintaining good hydration of the patient in the post­ operative period. • Improvement in the oral hygiene-mouth washes with potassium pennanganate (KMn04 ) solution. • Appropriate antibiotics against staphylococci, such as cloxacillin, is administered in the dose of 500 mg, 6th hourly along with metronidazole 400 mg, 8th hourly to treat anaerobic infections. • It takes about 3-5 days for the inflammation to settle down. II. Surgical treatment when there is pus • Under general anaesthesia, an adequate vertical incision is made in front of the tragus of the ear up to deep fascia.

DRAINAGE OF PAROTID ABSCESS Should not wait for fluctuation High grade fever, toxicity are indications Vertical incision Hilton's method is preferred to break multiple loculi

Open the deep fascia in two or three places and drair with blunt haemostat so as to avoid damage to facia nerve. This is described as Blair's method of drainagE of parotid abscess. A drainage tube has to be kept whicl: can be removed after 3-4 days (Key Box 18.4). RECURRENT PAROTITIS OF CHILDHOOD • Children between ages of 3 and 6 years are commonly affected. • Aetiology is unknown, may be due to sialectasis (dilatation of branches of salivary duct) • Recurrent pain and swelling of one or both parotids is common. • Each attack may last for 3 to 7 days. • It is self-limiting (if the attack is minor). • Sialography shows punctate sialectasis, called snowstorm appearance. • A short course of antibiotics has to be given to cover Streptococcus viridans. • Rarely, superficial parotidectomy may be necessary (Fig. 18.4).

Fig. 18.4: Superficial parotidectomy for recurrent parotitis. Please remember chronic parotitis in children is pathognomonic of HIV infection

SURGICAL ANATOMY OF THE SUBMANDIBULAR SALIVARY GLAND (Fig. 18.5) • Submandibular salivary gland is located in the sub­ mandibular triangle. It lies partly below and partly above the mandible. • It is in very close contact with the belly of the digastric muscle. At surgery, once the deep fascia is opened, the intermediate tendon of digastric is located and when it is retracted downwards, mobilisation of the gland becomes easy.

Salivary Glands

Intermediate tendon of digastric muscles

305

infection. Despite control of acute symptoms with antibiotics, the gland becomes chronically inflamed. • Calculi (80% of them occur in the submandibular salivary gland) commonly occur in the duct and also within the gland and produce recurrent sialoadenitis. Calculi are more common in the submandibular salivary gland than in the parotid gland because of the following reasons: 1. Higher mucin content in the submandibular salivary gland secretions. 2. Calcium and phosphate content in the secretion is high. Hence, 80% of them are radiopaque and are detected by plain X-ray (Fig. 18.6). 3. Nondependent drainage of the secretions. Gland is in the neck and the duct opens into the oral cavity. 4. Kinking or hooking of submandibular duct by lingual nerve.

Submandibular salivary gland

Fig. 18.5: Anatomy of the submandibular salivary gland

• Submandibular salivary gland is divided into a superficial and a deep part by the mylohyoid muscle which forms the oral diaphragm. During excision of the gland, a few fibres of mylohyoid are also removed. When submandibular salivary gland enlarges, it is bidigitally palpable because the deep portion is deep to mylohyoid and it is in the floor of the mouth. • Facial artery enters the gland from its posterolateral surface and deeply grooves the gland. It is ligated at this place first during excision of the gland. After grooving the gland, it ascends laterally and curls around the lower border of mandible to enter the face. It is ligated at this place also. • Main duct of submandibular gland, Wharton's duct arises from deep part of gland and opens on a papilla beside the frenulum of the tongue in the oral cavity. • In a deeper plane, the gland is related to two nerves­ lingual and hypoglossal. CHRONIC SUBMANDIBULAR SIALOADENITIS • Obstruction is the most important cause of submandibular sialoadenitis. Trauma to the floor of the mouth is another cause. • Obstruction can be due to stone, disease, stricture of the duct, or fibrosis of the papilla (calculus - most common). • The causative organism is Staphylococcus. SIALOADENITIS DUE TO CALCULI • The disease starts with acute bacterial sialoadenitis which occurs secondary to obstruction. The submandibular gland has a poor capacity for recovery following

Fig. 18.6: Submandibular calculi in the duct-removed (intraoral approach)

Clinical features (Key Box 18.5) • Salivary colic: It is a severe pricking type of pain which is exaggerated at the time of meals. Salivary secretions are induced by a meal or lemon (Lemon juice test). As a result of blockage due to a stone, the tension within the gland increases, resulting in pain. • Lingual colic: If a calculus is situated within the sub­ mandibular duct where it is hooked by lingual nerve, the

di!.111..........� • • • •

SUBMANDIBULAR SALIVARY GLAND ENLARGEMENT Location-submandibular region Lobular, firm swelling Bidigitally palpable Stone may be palpable within the duct, intraorally

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Fig. 18.7: Submandibular sialoadenitis

Fig. 18.8: Inflamed opening of submandibular duct

pain can radiate to the tongue as a result of irritation to the lingual nerve. • Enlargement of salivary gland during meals is the characteristic feature of salivary calculus. Classically submandibular salivary gland swelling is located in the submandibular region. It is firm in consistency with a lobular surface. It is tender and both lobes are enlarged. It is bidigitally palpable (submandibular lymph node is palpable only in the neck) both inside the oral cavity and in the neck. The swelling reduces in size once the stimuli are withdrawn (after meals). • The stone may be palpable within the gland (in the neck), within the duct (intraorally), or sometimes it may be seen at the orifice of the submandibular duct on the side of lingual frenulum. • It is not uncommon to get a severe septic sialoadenitis with gross swelling of the gland and inflammatory oedema almost like Ludwig's angina (Fig. 18.7).

is followed by gush of old dirty contents of the submandibular gland (Fig. 18.8). 2. Chronic sialoadenitis: This requires excision of submandibular salivary gland. Three steps of dissection of the gland include incision, mobilisation and excision (Manipal Rule of 2-Table 18.2). • Incision: It should be a skin crease incision over the lower pole of the gland, the posterior limit of the incision should be at least 2 cm away from the angle of the mandible, to avoid damage to the cervical branch of facial nerve. The incision is deepened till the deep fascia is opened. • Mobilisation of the gland: Division of the facial artery twice, once in a deeper plane on the posterolateral aspect and another at the superolateral aspect close to the lower border of the mandible is an important step which permits mobilisation of the gland. Separation of the gland from fibres of mylohyoid muscle by dividing small arteries completes the mobilisation (Fig. 18.9). • Excision of the gland: It is done by ligating and dividing submandibular duct.

Treatment • An oblique lateral or posterior oblique occlusal radiography may demonstrate a stone. 1. Stone in the submandibular duct: This can be removed by incising the mucosa over the floor of the mouth, after stabilising the stone. Removal of the stone

Complications • Damage to lingual nerve, marginal mandibular nerve or even to hypoglossal nerve. Seroma and infection are the other complications.

Submandibular salivary gland excision-Manipal Rule of 2 2 common indications

�

Stone and as a part of radical neck dissection

2" long incision

�

Curved incision over the swelling

Protect 2 superficial nerves

�

Cervical and marginal mandibular branches of facial nerve

Protect 2 deep nerves

�

Lingual and hypoglossal nerve

Ligate facial artery 2 times

�

First at deeper plane and then at superficial plane

Divide 2 muscles

�

Superficial-platysma; Deep---fibres of mylohyoid

Remove 2 lobes

�

Superficial and deep

Incision is 2 cm medial to mandible, 2 cm anterior to angle of the mandible

�

To protect 2 superficial nerves

Salivary Glands

307

Incidence: 80% of salivary gland tumours are found in the parotid gland. Out of these, 80% are benign, of which 80% are pleomorphic adenomas. In the submandibular salivary gland, 50% are benign and 50% are malignant. In the minor salivary glands, 90% are malignant. Thus, the incidence of malignancy increases from major to minor salivary glands. PLEOMORPHIC ADENOMA OF PAROTID GLAND (MIXED TUMOUR) Fig. 18.9: Submandibular stone and gland

• Transection of the nerve to mylohyoid muscle produces anaesthesia of the submental skin. SALIVARY GLAND TUMOURS INTERNATIONAL CLASSIFICATION I. Epithelial tumours II. Nonepithelial tumours. I. Epithelial tumours A.Adenoma 1. Pleomorphic adenoma 2. Monomorphic adenomas • Adenolymphoma (Warthin's tumour) • Oxyphilic adenoma (oncocytoma) • Other types B. Mucoepidermoid tumours C. Acinic cell tumour D. Carcinoma 1. Carcinoma in pleomorphic adenoma 2. Adenoid cystic carcinoma 3. Undifferentiated carcinoma 4. Adenocarcinoma 5. Epidermoid carcinoma 6. Acinic cell tumour 7. Mucoepidermoid carcinoma 8. Malignant mixed tumour. II. Nonepithelial tumours 1. Lipoma 2. Lymphoma 3. Neurofibroma 4. Lymphangioma 5. Sarcoma Salivary gland tumours are not uncommon. There are dozens of histological types of salivary gland tumours. However, pleomorphic adenoma and adenolymphoma are the common benign types. Carcinoma arising in pleomorphic adenoma, mucoepidermoid tumours and adenoid cystic carcinoma are important malignant tumours.

It is the most common benign salivary gland neoplasm. Pathology • Epithelial cells proliferate in strands, or may be arranged in the form of acini or cords. • There are also myoepithelial cells which proliferate in sheets. They are called spindle-shaped cells. • The tumour produces mucoid material, which displaces and separates the cells resembling cartilage in histological section. PEARLS OF

WISDOM

Because of the presence of epithelial cells, myoepithelial cells, mucoid material, pseudocartilage and lymphoid tissue, the tumour is called pleomorphic adenoma. • As the tumour grows, it compresses the normal parotid tissue and the branches of the tumour penetrate the thin capsule and enter the substance of the parotid. Simple enucleation will result in a recurrence. Hence, superficial parotidectomy has to be done. Clinical features 1. Middle-aged women, around 40 years, are commonly affected (Fig. 18.10) (female, fifth decade and fullness near ear lobule).

Fig. 18.10: Pleomorphic adenoma-classical signs

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2. Typically, a history of a very slow growing swelling (for a few years) is usually present. 3. The swelling is painless. Any painless swelling near the ear is best assumed to be parotid gland neoplasm unless proved otherwise (Fig. 18.11 ). Signs (Key Box 18.6) 1. Parotid swelling has the following classical features: • It presents as a swelling in front, below and behind ear. • Raises ear lobule. • Retromandibular groove is obliterated. 2. It is rubbery or firm. Soft areas indicate necrosis. In long­ standing cases, it can be hard. Surface can be nodular or

sometimes bosselated. Skin is stretched and shiny. Howevc being a benign tumour, it is neither adherent to the skin n1 to the masseter (Figs 18.12, 18.13 and 18.17). 3. After a few years, pleomorphic adenoma may show featun of transformation into malignancy (carcinoma exple< morphic adenoma). It should be suspected when • lt staiis growing rapidly • Skin infiltration occurs • Facial nerve paralysis occurs • Gets fixed to masseter muscle • Red, dilated veins over the surface • Presence of lymph nodes in the neck • Tumour feels stony hard. INTRAORAL EXAMINATION (Fig. 18.14)

• • • • • • •

CLINICAL EXAMINATION OF PAROTID TUMOURS Swelling firm, nodular Facial nerve involvement (80-90% cases of malignancy) Fixity to mandible and masseter Deep cervical nodes Opening of parotid duct Shift of tonsil and pillar of the fauces Other salivary glands (both sides).

• Approximately 10% of the parotid tumours are behind th facial nerve in the deep lobe. • This is appreciated by intraoral examination wherein th tumour presents with a parapharyngeal mass whic displaces the tonsil or soft palate medially. • Deep lobe tumours present as dysphagia. Such tumours ma: not show gross swelling on the outer aspect but as the: grow, they pass through the stylomandibular tunnel of Pate:

Fig. 18.11: Gross enlargement of parotid gland of 30 years duration

Fig. 18.12: Lateral view showing nodular surface

Fig. 18.13: Bosselated surface. Ear lobule is raised

Fig. 18.14: Deep lobe tumour. Importance of intraoral examination

( Courtesy: Dr Sreejayan, Prof of Surgery, Calicut Medical College, Calicut, Kerala)

Salivary Glands

Fig. 18.15: Large sebaceous cyst in the preparotid region. It had sebaceous punctum

Fig. 18.17: Nodular surface of pleomorphic adenoma

UHi¥.........,� DIFFERENTIAL DIAGNOSIS OF SWELLING IN THE PAROTID REGION 1. When it involves the preparotid region and angle of mandible • Preparotid lymph node enlargement: It may be enlarged due to tuberculosis, metastasis or in non-Hodgkin's lymphoma. Sebaceous cyst in pre-parotid region (Fig. 18.15) • Preauricular dermoid cyst (young patients) • Mesenchymal tumours such as lipoma, neurofibroma • Haemangioma, lymphangioma (Fig 18.16) 2. When it involves lower pole of parotid • Upper jugular chain lymph node enlargement­ metastatic or tubercular • Branchial cleft cyst • Epithelial inclusion cyst

and push the pharyngeal wall, tonsil and soft palate. These tumours are called Dumbbell tumours (Key Box 18.7) DIFFERENTIAL DIAGNOSIS OF SWELLING IN PAROTID REGION Investigations Slow growing parotid tumours should not be subjected to biopsy for 2 reasons:

309

Fig. 18.16: Parotid cyst in a young boy-It was fluctuant and transillumination-negative. It was a haemangioma

• Injury to the facial nerve • Seeding of tumour cells in the subcutaneous plane which causes recurrence in about 40-50% of cases. 1. Fine needle aspiration cytology (FNAC) is done to confim1 the diagnosis and rule out malignancy. 2. CT scan is done when the tumour is arising from the deep lobe. It helps to define the extraglandular spread, the extent ofparapharyngeal disease, cervical lymph nodes and bony infiltration. 3. FNAC of the lymph nodes that are palpable in the neck in cases of malignancy of the parotid gland. 4. X-ray of the bones (mandible and mastoid process) to look for bony resorption, if malignancy is suspected. 5. MRI is a better investigation. However, it is expensive­ CT Scan and MRI lack specificity for differentiating between benign and malignant lesions. Indications for CT scan 1. Suspected bone destruction at skull base 2. Suspected involvement of mandible 3. To assess neck nodes. Indications for MRI 1. Delineating the interface between tumour and normal salivary gland 2. Better imaging of the parapharyngeal space 3. Evaluating perineural spread, e.g. adenoid cystic carcinoma 4. Facial nerve status may be better appreciated. Treatment • Conservative superficial parotidectomy (Fig. 18.18 and Key Box 18.8) • It is the standard surgery done for benign pleomorphic adenoma. It means removal of the entire lobe containing the tumour which is superficial to the facial nerve. Facial nerve should always be preserved. Enucleation should never be done as it causes recurrence and can injure facial nerve. It is difficult to remove a recurrent tumour.

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3.

4. 5. Fig. 18.18: Conservative s uperficial parotidectomy: Facial nerve divides the gland into superficial and deep lobes. The entire superficial lobe is removed preserving the facial nerve

• • • • •

CONSERVATIVE SUPERFICIAL PAROTID EC TOMY Indicated in pleomorphic adenoma and other benign neoplasms Tumour along with the normal lobe is removed Preserve the facial nerve, even in malignant tumours unless grossly involved. Avoid rupture of the gland (Fig. 18.19) Enucleation should not be done as it ca uses recurrence.

Some important steps of superficial parotidectomy (Key Box 18.9) 1. Adequate exposure by an incision which starts in front of tragus of ear, vertically descends downwards, curves round the ear lobule up to the mastoid process and is carried downwards in the neck ('Lazy S' incision). 2. Recognising the facial nerve at surgery (Fig. 18.19) • Facial nerve lies 1 cm inferomedial to the bony cartilagenous junction of external auditory canal (Conley's pointer).

6.

• Trace the posterior belly of digastric up to the mastoid process. Facial nerve is in between the muscle and tympanic plate. • A nerve stimulator may be used. Developing a plane: Facial nerve and retromandibular vein divide the parotid gland into superficial and deep lobes. Benign tumours do not invade this faciovenous plane of Patey. Gentle handling, good suction and perfect haemostasis help in clear recognition of the nerve. In toto removal-The tumour along with the lobe to avoid spillage (which is one of the causes of recurrence). Good suction drainage of the wound is necessary to avoid haematoma, wound infection, etc.

MIMI..........� • • • • • •

WHAT SHOULD BE DONE IN PAROTID SURGERY Wide exposure by an adequate skin flap Always identify facial nerve (best way to avoid injury). Minimum surgery to be done is superficial parotidectomy (enucleation can cause recurrence and injury to facial nerve) Always try to preserve facial nerve even in malignancies unless it is directly infiltrated. If facial nerve is excised, try to reconstruct immediately by nerve graft-greater auricular or sural nerve. Always 'drain' the cavity.

Total conservative parotidectomy excision of superficial and deep lobe of parotid gland while preserving the facial nerve. Total parotidectomy with the excision of facial nerve when nerve is involved. Radical parotidectomy involves excision of other structures along with parotid gland and facial nerve • Skin • Infratemporal fossa • Mandible • TM joint • Petrous bone

Indications for postoperative radiotherapy • If the deep lobe is involved • If the lymph nodes are involved • High grade tumours • If margins are positive • Perineural invasion • Lymphovascular invasion Fig. 18.19: Superficial parotidectomy specimen

Postoperative radiotherapy • T3/T4 cancer: Adenoid cystic carcinoma recurrent tumours

Salivary Glands

ADENOLVMPHOMA (WARTHIN'S TUMOUR, PAPILLARY CYSTADENOMA LYMPHOMATOSUM) • Adenolymphoma is not a lymphoma. It is a misnomer (vide infra). • It is a benign parotid tumour and next common to pleomorphic adenoma. It constitutes about 10% of parotid tumours. • Origin of adenolymphoma: During development some parotid tissues get included within lymph nodes (preparotid) which are present within the parotid sheath. Histology • It is composed of double layered eosinophilic epithelium. The inner cells are columnar. • Presence of lymphatic tissue in the stroma, and lymph follicles is characteristic of adenolymphoma (hence the name). Clinical features (Table 18.3) • Middle aged or elderly males are commonly affected usually they are smokers. • Can be bilateral, in some cases (10%). • It has smooth surface, round border with soft, cystic consistency (Fig. 18.20). • Classically, situated at the lower pole of parotid elevating the ear lobule. Sometimes it may be multicentric. • This tumour affects only parotid gland (very, very rarely other glands may be affected). Treatment • It has got a well-defined capsule. Hence, enucleation used to be done earlier but not now. Superficial parotidectomy is the treatment of choice.

311

MUCOEPIDERMOID TUMOUR • As the name itself suggests, it consists of sheets of epidermoid cells and cystic spaces lined by mucus secreting cells (Fig. 18.21 ). • In childhood, it is the commonest parotid tumour. They are benign, slow-growing but hard in consistency. (Adenolymphoma and mixed tumours are firm but mucoepidermoid tumour is hard). Parotid is the commonest site. In cases of minor salivary glands, palate is the commonest site. • Mucoepidermoid tumours can infiltrate local tissues, lymph nodes or skin. Hence, a few consider that mucoepidermoid tumours are always carcinomatous. • Well-differentiated tumours behave like benign tumours, intermediate ones are aggressive and undifferentiated tumours metastasise early. Mucoepidermoid carcinoma is the most common malignant epithelial neoplasm of salivary gland. • The low grade tumours are composed of predominantly mucus secreting cells. High grade tumours have pre­ dominantly epidermoid cells. • Benign tumours need excision and malignant tumours need radical parotidectomy. Radiation is required in the post­ operative period. OTHER TUMOURS Acinic cell tumour • !hese are the uncommon parotid tumours. Commonly occur tn women. • The cells resemble those of serous acini and this tumour also has properties of invasion such as mucoepidermoid tumour. It tends to be soft and sometimes cystic. Oxyphil adenoma • Also called oncocytoma. It occurs exclusively in the parotid gland. It is a solid tumour and occurs in the sixth decade of life.

Comparison between pleomorphic adenoma and adenolymphoma Features

Pleomorphic adenoma Adenolymphoma

1. Incidence 2.Sex 3. Number 4.Site 5. Clinical feature 6. Histology

70-80% Common in females Single Unilateral Nodular, firm

10% Common in males Sometimes multiple Bilateral Smooth, soft cystic

Pleomorphism

7. 99mTc-Pertechnetate scan 8. Treatment

Cold spot

Double layer epithelium and lymphoid tissue Hot spot

Superficial parotidectomy

Superficial parotidectomy

Fig. 18.20: Adenolymphoma Fig. 18.21: Mucoepidermoid

carcinoma

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Adenoid cystic carcinoma • It is a highly malignant tumour consisting of cords of dark staining cells with cystic spaces containing mucin. It also consists of myoepithelial cells and duct epithelium. • Even though slow-growing, it spreads along the perineural tissue, may invade periosteum or medullary bone at a distance. This bone resorption results in bony tenderness. • These tumours have a high incidence of distant metastasis but in general they display indolent growth. Skip lesions are common as it spreads along the nerve tissue, which leads to treatment failure. • Local infiltration, lymphatic and blood spread, and local recun-ence are important features. • It is hard and fixed and can produce anaesthesia of the skin overlying the tumour. • Early cases are treated by radical parotidectomy with block dissection of the neck. However, many cases present late to the hospital. Thus, palliative radiotherapy is given to reduce pain and to an-est progress of the disease. Complications of parotidectomy (Key Box 18.10)

............................ KEY BOX 18.10

.

COMPLICATIONS OF PAROTIDECTOMY • Flap necrosis-avoid acute bending of the incision and to use gentle retraction • Facial nerve palsy-careful identification • Fluid collection: Blood or seroma-perfect haemostasis and drain should be used • Fistula salivary-duct should be ligated • Frey syndrome-occurs in 10% of the cases Observe 5 Fs.

Summary of malignant salivary gland tumours (Figs 18.22 to 18.29 and Table 18.4) To find out the exact type of malignant tumour is of interest to pathologists. Clinically, one can suspect malignancy when a salivary tumour has one of the following features: • Rapidly growing neoplasm • Change in consistency (the tumour tends to be hard) • Fixity to underlying muscle such as masseter as in parotid tumours • Fixity to mandible as in parotid or submandibular tumour • involvement of facial nerve as in 80% of cases of malignant parotid tumours. • Resorption of adjacent bone such as mastoid, tenderness as in adenoid cystic carcinoma. • Significant hard nodes in the neck. • They are treated by radical sialadenectomy with radical block dissection of the neck. Radiotherapy is used as a palliative treatment. 'Most common' for salivary glands (Key Box 18.11)

MHMINIIIWIIIIIIIIII� 'MOST COMMON' FOR SALIVARY GLANDS • Most common benign parotid tumour in adults­ pleomorphic adenoma • Most common benign parotid tumour in children­ haemangioma • Most common type of cancer arising in the parotid glands is mucoepidermoid cancer. • Most common malignant tumour in submandibular gland­ adenoid cystic carcinoma • Most common minor salivary gland tumour is adeno­ carcinoma • Most common site of squamous cell carcinoma is sub­ mandibular salivary gland • Most common response to radiotherapy among the malignant tumours is adenoid cystic carcinoma.

Comparison between submandibular and parotid tumours

• Incidence

Submandibular tumour

Parotid tumour

Uncommon

Common

• Malignancy

50% of tumours

10-20%

• Location

Submandibular triangle

Parotid region

• Deep lobe involvement

Intraorally felt in the floor of mouth

TonsilJar shift, can also be felt in the lateral wall

• Surgical treatment of malignant tumour

Small tumours-intracapsular submandibular excision Large tumours-radical excision with or without sacrifice of '2' nerves-lingual and hypoglossal

Total conservative parotidectomy for malignant tumours. Facial nerve should always be preserved.

• Lymph nodes

Selective neck dissection-supraomohyoid neck dissection

Selective neck dissection (lateral) levels II, Ill, IV and V lymph nodes

• Warthin's

Does not occur here

Exclusively occurs in the parotid gland.

Salivary Glands

MALIGNANT PAROTID TUMOURS

313 _,.-

Fig. 18.22: Low grade mucoepidermoid carcinoma-had restricted mobility due to fixity to masseter

Fig. 18.23: Adenoid cystic carcinoma- Fig. 18.24: High grade mucoepidermoid perineural spread is common with this carcinoma-rapidly growing and dilated veins over the surface tumour

Fig. 18.25: Facial nerve paralysis-one of the strong clinical signs of malignancy in a parotid tumour

Fig. 18.26: Adenoid cystic carci­ noma-hard and fixed receiving radiotherapy

Fig. 18.28: Carcinoma parotid-late stage with involvement of platysma-platysma sign

Fig. 18.27: Malignant mixed tumour. Ulcerated, late cases

Fig. 18.29: Same patient in Fig. 18.28 also had facial nerve paralysis

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FEW OPERATIVE PHOTOGRAPHS (Figs 18.30 to 18.35)

Fig. 18.30: Malignant mixed tumour- Fig. 18.31: Methylene blue is used to mark position of patient the site of incision and part to be raised

Fig. 18.33: Strap muscles are divided as a part of supraomohyoid block dissection

Fig. 18.34: Wide excision and block

dissection

MISCELLANEOUS FREY'S SYNDROME-GUSTATORY SWEATING • It occurs after surgery for parotid tumours, surgery in the region of temporomandibular joint or due to injury to the parotid gland. Injury to the auriculotemporal nerve can occur at a site where it turns around the neck of the mandible. The injury manifests at a later date, e.g. 2-3 months (Fig. 18.36). • Because of the injury, postganglionic parasympathetic fibres from otic ganglion unite with sympathetic fibres of superior cervical ganglion which supplies the vessels and sweat glands over the skin overlying parotid region (Key Box 18.12).

.,.,:1-,, PARTS SUPPLIED BY AURICULOTEMPORAL NERVE • Auricular part: - External acoustic meatus - Tympanic membrane surface - Skin of auricle above external acoustic meatus • Temporal part: Hairy skin of the temple.

Fig. 18.32: Flaps are elevated

Fig. 18.35: Specimen of tumour witl surrounding fat, fascia and lymphatic tissut

• As a result of this, whenever the act of chewing 01 mastication is started, there is increased sweating and hyper­ aesthesia in the region supplied by auriculo­ temporal nerve (cuta������ neous branch of cted affe mandibular division of trigeminal nerve). Hence, it is called auricu l o t e m p o r a l Fig. 18.36: Frey's syndrome syndrome. • Diagnosis: Starch iodine test-paint the affected area with iodine and allowed it to dry before applying the dry starch. • The starch turns blue on exposure to iodine in the presence of sweat. Prevention • Principle is to provide a barrier between the skin and parotid bed by using temporalis fascia! flap or sternomastoid muscle flap.

Salivary Glands

Treatment 1. Reassurance, aluminium chloride-antiperspirant which is a useful astringent 2. Denervation by tympanic neurectomy 3. Latest treatment includes injection of botulinum toxin into the affected skin (see page 441 also). RARE CAUSES OF SALIVARY GLAND ENLARGEMENT

............................

315

KEY BOX 18.13

.

PAROTID FISTULA

• Any surgery on the parotid gland-superficial parotidectomy, drainage of abscess, surgery for carcinoma cheek, facio­ maxillary trauma are the causes. • Discharging watery fluid, exaggerated by keeping lime in the mouth. • Fistulogram confirms the diagnosis. • Exploration and excision of fistula and ligation of duct is required.

1. Sjogren's syndrome It is the diffuse infiltration of salivary and lacrimal glands with lymphocytes resulting in enlargement of glands and slow destruction of acini. Thus, clinical features include dry eyes (keratoconjunctivitis sicca) and dry mouth (xerostomia). These along with a third component rheumatoid arthritis, form the triad of Sjogren's syndrome (primary). • 30% of patients with systemic lupus erythematosus and all patients with primary biliary cirrhosis develop Sjogren 's syndrome. This is termed secondary Sjogren's syndrome. • Other features: This disease is 10 times more common in females and presents with painful enlargement of the glands. • Complications 1. Lymphomatous transformation (high in primary). 2. Oral candidiasis. 2. Mikulicz disease Due to autoimmune mechanism, symmetrical enlargement of all salivary glands and lacrimal gland enlargement occur. Dry mouth and narrow palpebral fissures are diagnostic of this condition.

MINOR SALIVARY GLAND TUMOUR (Fig. 18.37) • Even though they are called minor, numberwise they are major (many), about 450 in number. • They are mucus-secreting. • They can present as mucus retention cyst (common in lip) or as malignant tumour. • 90% of minor salivary gland tumours is malignant. • Since they are submucosal, they start as a submucous nodule (very important point in the history) to differentiate from carcinoma buccal mucosa/lip, etc. • As they grow, they ulcerate. Ulceration is a feature of malignancy. • Slowly lymph nodes get enlarged. • Treatment of benign cyst/tumour is by simple excision and malignant tumour is by wide excision.

3. Drugs Carbimazole and thiouracil can cause enlargement of salivary glands. 4. Metabolic disorders Diabetes and acromegaly are the other causes. 5. Granulomatous sialoadenitis These are rare, painless swellings. Following are the causes: • Tuberculosis • Sarcoidosis-commonly affects parotid gland wherein it is called pseudotumour • Toxoplasmosis • Cat-scratch disease • Wegener's granulomatosis PAROTID FISTULA It is an uncommon condition which commonly occurs after surgery on the parotid gland (Key Box 18.13).

Fig. 18.37: Minor salivary gland tumour in the palate. Laser excision

was done

SURGERY FOR FACIAL NERVE PALSY Indications for different types of surgery 1. Early immediate nerve repair, in case of injury to the nerve. 2. Late nerve crossing by suturing peripheral branches of facial nerve to one of the following nerves: • Hypoglossal nerve, spinal accessory nerve • Phrenic nerve. 3. Surgery to achieve movement in long standing facial palsy (usually after 1 year).

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A. Static procedures • Suspension of lips, cheek and angle of mouth to zygomatic bone or temporal fascia using fascia lata, palmaris longus tendon or other alloplastic materials. • Medial canthoplasty to reduce epiphora • Lateral tarsorrhaphy (canthoplasty) to prevent exposure keratitis due to widened palpebral fissure. B. Dynamic procedures • Muscle transfer with carefully preserved muscle nerve and vessel, e.g. temporalis muscle transfer, masseter muscle transfer. • Cross face nerve transplantation using sural nerve. Using microscope, sural nerve is sutured to the two or three relatively insignificant branches of facial nerve (selected by intraoperative electric stimulation) on normal side. Other end of the sural nerve is sutured to distal end of the divided facial nerve on paralysed side. • Free neurovascular gracilis muscle graft using microvascular techniques. PERIPHERAL NERVE REPAIR AND TRANSFERS PRINCIPLES OF NERVE REPAIR 1. Preoperative assessment of motor and sensory system 2. Microsurgical tools should be available 3. Tension free repair should be done. If tension free repair is not possible, then nerve graft can be used 4. Primary repair when conditions permit 5. Delayed repair should be done in case of extensive injury. Microsurgical techniques • 4X magnification needed • 9-0/10-0 nylon is used for repair • Ends prepared with microscissors. First suture to be loose to ease the alignment of other sutures. • Avoid postural manoeuvre to avoid tension. Nerve can be mobilised 1-2 cm proximally and distally for tension free suturing but not more than that. Conduits • To place a polyglycolic acid tube to bridge the gap rather than to perform nerve grafting. • Indication - Donor nerve harvest not possible due to availability and associated morbidity - Patients who decline autogenous nerve graft harvest - Nerve gap of not more than 3 cm. • Principle of conduits - Nerve regeneration and proximal and distal axonal matching result from a combination of neurotropism and contact guidance.

TYPES I. Nerve grafting • Indication: - If a tension free repair is not possible - If the nerve gap is more than 3 cm - Can be used in place of conduits • Principle - The graft should be oriented in a reverse fashion frorr its native position so that the regenerating fibres are no1 diverted from the distal neurorrhaphy site and distal stump. Misalignment should be avoided. • If the nerve gap is small, fascicular matching can be done • If the nerve gap is long, techniques of awake stimulation or histochemical staining may be used. • Donor sources for nerve grafting - Posterior interosseous nerve, medial antebrachial cutaneous nerve for small digital nerves, sural nerve for nerve gaps involving larger nerves, greater auricular nerve. II. Nerve transfer • It is used for reconstruction of proximal nerve injuries. • Drawbacks of nerve graft that become the merits of nerve transfer: • Grafting across a proximal nerve injury has poor functional outcomes • Timely re-innervation may not be possible if the target muscle is too far from the regenerating site. • A nerve transfer converts a high level nerve injury to a low level nerve injury by recruiting expendable nerve fascicles from the donor nerve to innervate critical nerves close to their target end organs. • Donor nerves are preferentially selected according to their proximity to the target site • Criteria for nerve transfers - An expendable donor nerve - Donor nerve with a large number of pure axons - Donor nerve near the target organ - Donor motor nerve that innervates a muscle that is synergistic to the target muscle • Nerve transfers are done for both motor and sensory nerves.

WHAT IS NEW IN THIS CHAPTER?/RECENT ADVANCES

• All topics have been updated. • A few photographs and key boxes have been added. • Granulomatous sialoadenitis, peripheral nerve repair and transfers have been added.

Salivary Glands

'317

MULTIPLE CHOICE QUESTIONS

1. Commonest tumour of minor salivary gland is: A. Pleomorphic adenoma B. Warthim 's tumour C. Malignant tumour D.Mucoepidermoid tumour 2. Parotid duct opens opposite: A. Upper canine tooth B. Lower canine tooth C. Upper 2nd molar tooth D. Lower 2nd molar tooth 3. Chronic parotitis in children is pathognomonic of which infection? A. HCV infection B. HBV infection C. HIV infection D. Syphilis 4. Which is the common site of calculi in submandibular salivary gland? A. Superficial lobe B. Deep lobe C. Accessory lobe D. Duct 5. Deep lobe tumours of parotid presents as following features except: A. Dysphagia B. Can push tonsils C. Can push soft palate D. Can block the external auditory meatus 6. The investigation of choice in deep lobe tumours of parotid gland is: A. CT scan B. Angiography C.MRI D. Ultrasound

9. Following benign tumours have high incidence of recurrence except? A. Adamantinoma B. Deep lobe tumours of parotid gland C. Desmoid tumours D. Diffuse lipomata 10. Recommended treatment of pleomorphic adenoma is: A. Enucleation B. Excision C. Superficial parotidectomy D. Wide excision 11. Which benign tumour of parotid produces Hot Spot in Technetium scan? A. Pleomorphic adenoma B. Adenoid cystic carcinoma C. Mucoepidermoid tumour D. Adenolymphoma 12. Commonest parotid tumour in children is: A. Pleomorphic adenoma B. Warthim's tumour C. Mucoepidermoid tumour D. Lymphangioma 13. Most common malignant tumour in submandibular salivary gland is: A. Pleomorphic adenocarcinoma B. Adenoid cystic carcinoma C. Mucoepidermoid tumour high grade D. Acinic cell tumour 14. Following are true for precancerous lesions of lip/ oral cavity except: A. Keratoacanthoma B. Leukoplakia C. Erythroplakia D. Submucous fibrosis

7. Conley's pointer refers to: A. Location of facial nerve in relation to tragal cartilage B. Location of facial nerve in relation to posterior belly of diagstric C. Location of facial nerve in relation to retromandibular vem D. Location of facial nerve in relation to pinna

15. Following are features of carcinoma maxillary antrum except: A. Can cause asymmetry of face B. Can cause proptosis C. Can cause infraorbital nerve paralysis D. Can cause buccal branch of facial nerve paralysis

8. Following are true for chronic hyperplastic candidiasis except: A. Invasion of Candida albicans B. Antifungal treatment helps C. High malignant potential D. Floor of the mouth is affected

16. Following are the boundaries of nasopharyngeal space except: A. Nasal fossae B. Basilar part of occipital bone C. Body of sphenoid D. Cribriform plate of ethymoid bone

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17. Following are true for nasopharyngeal carcinoma except: A. Presents as high anterior cervical lymphadenopathy B. Can present as trigeminal neuralgia C. Compression on IX and X cranial nerves D. Can present as ophthalmoplegia 18. Following are painless ulcers in the tongue except: A. Gummatous ulcers B. Carcinomatous ulcers C. Systemic diseases D. Tuberculous ulcers J 9. Following are painful ulcers in the tongue except: A. Gummatous ulcers B. Tuberculous ulcers C. Aphthous ulcers D. Dental ulcers 20. Following are true for syphilitic lesions of the tongue except: B. Gumma A. Snail track ulcers C. Hutchinson's wart D. Hunterian chancre 21. Following are true for dental cyst except: A. Upper jaw is commonly involved B. It is a large unilocular cyst C. Cyst contains cholesterol crystals D. Arises from unerupted tooth

22. Which one of the following swelling does not contai cholesterol crystals? A. Branchial cyst B. Sebaceous cyst C. Dental cyst D. Hydrocoele 23. Following are true for dentigerous cyst except: A. Upper jaw is commonly involved B. Produces egg shell crackling C. X-ray shows soap bubble appearance D. Arises from unerupted tooth 24. Adamantinoma of the jaw has following feature: except: A. It is a malignant tumour B. It spreads within medullary bone C. It is treated by wide excision D. Mandible is the most common site 25. Which of the following is unilocular cyst? A. Adamantinoma B. Dentigerous cysts C. Epididymal cyst D. Dental cyst

ANSWERS 1 C

2 C

3 C

4 D

5 D

11 D

12 C

13 B

14 A

15 D

21 D

22 B

23 A

24 D

25 D

6 C 16 D

7 A 17 A

D

9 D

10 C

18 D

8

19 A

20 D

19. Thyroid Gland • • • • • • •

• • • • • • •

Surgical anatomy Physiology Thyroid function tests Clinical examination Goitre Multinodular goitre Retrosternal goitre

Introduction Thyroid gland is an endocrinal gland present in the neck secreting T3 and T4 hormones. It is richly vascular and highly functional. Effects of hormonal changes affect every part of the body such as central nervous system, cardiovascular system, gastrointestinal system and reproductive system. It is also the site of various diseases-a simple enlargement, toxicity and malignant transformation.

Graves' disease Malignant tumours Anaplastic carcinoma Solitary nodule Thyroiditis Lingual thyroid Ectopic thyroid

Pyramidal lobe

SURGICAL ANATOMY OF THYROID GLAND Development and Anatomy It develops from median down growth (midline diverticulum) of a column of cells from the pharyngeal floor between first and second pharyngeal pouches. • The descent is anterior to structures that form hyoid bone and larynx (Fig. 19.1). By 6 weeks of intrauterine life, the central column, which becomes the thyroglossal duct, gets reabsorbed. The duct bifurcates to form thyroid lobes. Pyramidal lobe is formed by a portion of the duct. Calcitonin producing parafollicular or C cells originate from the fourth branchial pouch. Thyroid gland is present in the neck, enclosed by pretracheal fascia which is a part of deep cervical fascia. It has a right and left lobe joined by the isthmus in front 319

....,._______ Lateral lobe of thyroid

Fig. 19.1: Development of thyroid gland

of 2nd, 3rd and 4th tracheal rings. It weighs about 20-25 g. A projection from the isthmus usually on the left side is called pyramidal lobe. It is attached to the hyoid bone by a fibrous band or muscle fibres called levator glandulae thyroideae. Suspensory ligament of Berry: This pair of strong condensed connective tissue binds the gland firmly to each side of cricoid cartilage and upper tracheal rings.

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Pretracheal fascia, which is part of deep cervical fascia splits to invest the gland. These structures (ligament of Berry and pretracheal fascia) are responsible for thyroid gland moving with deglutition.

Arterial supply (Fig. 19.2) • Superior thyroid artery, a branch of the external carotid artery, enters the upper pole of the gland, divides into anterior and posterior branches and anastomoses with ascending branch ofinferior thyroid artery. Since the upper pole is narrow, ligation is easy. Inferior thyroid artery is a branch ofthyrocervical trunk' and enters the posterior aspect ofthe gland. It supplies the gland by dividing into 4 to 5 branches which enter the gland at various levels (not truly lower pole). Inferior thyroid artery used to be ligated well away from the gland to avoid damage to RLN. However, ligation of these arteries on both sides will cause permanent hypopara­ thyroidism. Hence, the current practice is to identify and ligate the branches of inferior thyroid artery (3-4) separately. • Thyroidea ima artery is a branch ofeither brachiocephalic trunk or direct branch of arch of aorta and enters the lower part of the isthmus in about 2 to 3% of the cases. Venous drainage Superior thyroid vein2 drains the upper pole and enters the internal jugular vein. The vein follows the artery.

External-------, carotid artery Superior--­ thyroid artery

•

•

Middle thyroid vein is single3 , short and wide and draim into internal jugular vein. Inferior thyroid veins form a plexus which drain into innominate vein. They do not accompany the artery. Kocher's vein is rarely found (vein in between middle and inferior thyroid vein).

Nerves in relationship with thyroid gland 1. Superior laryngeal nerve: The vagus nerve gives rise to superior laryngeal nerve, which separates from it at skull base and divides into two branches. The larger internal laryngeal nerve is sensory to the supraglottic larynx. The smaller external laryngeal nerve runs close to the superior thyroid vessels and supplies cricothyroid. • This nerve is away from the vessels near the upper pole. Hence, during thyroidectomy, the upper pedicle should be ligated as close to the thyroid as possible. 2. Recurrent laryngeal nerve (RLN) is a branch ofvagus, hooks around ligamentum arteriosum on the left and s ubclavian artery on the right and r uns in the tracheoesophageal groove near the posteromedial surface. Close to the gland, the nerve lies in between (anterior or posterior) branches of inferior thyroid artery (Figs 19.3 and 19.4 in the Riddle's triangle). • On the right side, it is I cm within the tracheoesophageal groove (Key Boxes 19. I A and B).

....................... KEY BOX 19.1A

Superior thyroid vein Internal jugular vein

External--­ laryngeal nerve Inferior thyroid artery Inferior thyroid vein

Subclavian artery '------ lnnominate vein

• • • • • •

RECURRENT LARYNGEAL NERVE AT SURGERY

Lack of colour-white in colour Lack of elasticity Lack of pulsation Longitudinal vein on the surface Longitudinal course Location-Riddle's triangle-it is between inferior thyroid artery superi orly, carotid artery laterally and trachea medially. From here RLN runs up­ wards to enter larynx at greater cornu of thyroid cartilage

Fig. 19.3A Fig. 19.2: Surgical anatomy of the thyroid gland 'Branches of thyrocervical trunk can be remembered as SIT-suprascapular, inferior thyroid and transverse cervical artery. 2Superior thyroid artery and vein are like newly married couple, they go together, hand in hand. 3Middle thyroid vein is single, a bachelor and inferior thyroid artery and vein are a divorced couple.

.

Thyroid Gland

321

Lymphatic drainage of thyroid

__,________ Superior thyroid artery

----�- Mobilised gland

Inferior ----l__. thyroid artery

Fig. 19.38: Course of recurrent laryngeal nerve-gland is mobilised. The branches of inferior thyroid are ligated (not the main trunk)

Right vagus

•

-

Subcapsular lymphatic plexus drains into pretracheal nodes (delphic nodes means uncertain) and prelaryngeal nodes which ultimately drain into lower deep cervical nodes and mediastinal nodes (Fig. 19.5). The chief lymph nodes are middle and lower deep cervical lymph nodes (Levels III and IV). Supraclavicular nodes and nodes in the posterior triangle can also be involved in malignancies of the thyroid gland, especially papillary carcinoma thyroid.

Histology (Fig. 19.6) • Microscopically, it is divided into lobules. Each lobule has 20-40 follicles. • Each follicle is lined by cuboidal epithelial cells. • In the centre, colloid is present which is secreted from epithelial cells in response to calcitonin. • Parafollicular cells are present in the interfollicular stroma.

Left vagus Pretracheal lymph nodes Middle and lower deep cervical lymph nodes

Left recurrent laryngeal nerve

Right recurrent laryngeal nerve

Left subclavian artery

CX>'=""'--------- Mediastinal node

Fig. 19.5: Lymphatic drainage

Right subclavian artery Arch of the aorta

Fig. 19.4: Course of recurrent laryngeal nerve and origin

............................. . KEY BOX 19.18

RECURRENT LARYNGEAL NERVE ANOMALIES

• The nerve traverses through the gland in about 5-8% of cases. • The nerve may be very closely adherent to the posteromedial aspect of the gland. • Nerve not seen-may be far away in the tracheo-oesophageal groove. • Nonrecurrent, recurrent laryngeal nerve is found in about 1 in 1,000 cases. The nerve has a horizontal course. • In 25% of the cases, it is within the ligament of Berry.

Fig. 19.6: Diffuse colloid goitre-Acini lined by cuboidal follicular cells with luminal colloid ( Courtesy: Dr Laxmi Rao, HOD Pathology, KMC, Manipal)

Manipal Manual of Surgery

322 PHYSIOLOGY

Tri-iodothyronine (T3) and thyroxine (T4) are the hor­ mones secreted by the thyroid gland. Dietary requirement of iodine per day is 100-200 micrograms or 0.1 mg. Sources of iodine are milk, dairy products and sea food including fish. Steps involved in the synthesis of these hormones

1. Iodide trapping from the blood into the thyrocyte is the first step in the formation of T3 and T4. • Thiocyanates and perchlorates block this step.

2. Oxidation of iodide to inorganic iodine: This step needs the enzyme peroxidase. • Drugs which block this stage (thioamides) are sulfonamide, PAS (para-amino-salicylic acid), carbimazole and propylthiouracil. 3. Formation of iodotyrosines • Iodine + Tyrosine = MIT (monoiodotyrosine) and diiodotyrosine (DIT) • This step is inhibited by thiourea group of drugs, i.e. carbimazole. 4. Coupling reactions • Coupling of two molecules of DIT results in T4 and one molecule of DIT and MIT results in T3. • This stage is blocked by carbimazole. 5. Hydrolysis • The hormones combine with globulin to form a colloid­ thyroglobulin. They are stored in the thyroid gland and released as required by process of hydrolysis. • T3 is an important physiological hormone and is fast­ acting (few hours). T4 is a slow-acting hormone and takes about 4-14 days to act. THYROID FUNCTION TESTS Serum T3 and T4 estimation is most commonly performed. Other tests are not commonly done and some of them are obsolete (Table 19.1). 1. Serum T3: Normal levels-1.5-3.5 nmol/L • Most (80%) T3 is produced by deiodination of T4 in the liver, muscle, kidney and anterior pituitary. • T3 is 3 to 4 times more potent than T4. • The half-life of T3 is approximately 24 hours, whereas half-life of T4 is about 7 days.

Levels of T3, T4 and TSH in some common conditions Disease

. . .

T3

T4

TSH

Thyrotoxicosis

i

i

T3 toxicosis

ii

Suppressed or undetectable

Normal

Suppressed

Hypothyroidism

Low or normal

Low

i

• Free T3 (3 to 9 pmol/L) is most useful in confirming the diagnosis of early hyperthyroidism, especially in pregnancy where in levels of free T4 and free T3 rise before total T4 and T3. 2. Serum T4: Normal levels-55 to 150 nmol/L • They are measured by radioimmunoassay. • In euthyroid state, T4 is the predominant hormone produced by the thyroid. • Total T4 levels reflect output from the thyroid gland. Both T3 and T4 increase cell metabolism, nonnal growth, facilitate normal mental development and increase local effects of catecholamines. 3. Serum TSH (thyroid stimulating hormone) 0.3-5 IU/ml of plasma. Table 19.1 shows the levels of T3, T4 and TSH in a few common conditions. 4. Serum thyroglobulin • It is produced by thyroid tissue only. Hence, the levels should be low after total thyroidectomy. • The most important use of this test is to monitor patients after total thyroidectomy for well-differentiated carcinoma. • It is not nom1ally released into circulation in large amount but increases suddenly in thyroiditis, Graves' disease or toxic multinodular goitre (MNG). 5. Serum cholesterol: It is increased in hypothyroidism and decreased in hyperthyroidism. 6. Thyroid autoantibody levels: More than 90% of the patients with Hashimoto's thyroiditis and 80% of patients with Graves' disease have antibodies which are called as 'LATS' (long acting thyroid stimulator). The detection of these antibodies help in the diagnosis of such cases and also to suspect these diseases before clinical mani­ festation. 7. Thyroid scintigraphy (Table 19.2 and Fig. 19.7): Uptake by both lobes. Table 19.3 shows a few terminology of the thyroid diseases.

Thyroid Gland

323

Thyroid scintigraphy Substance 131I

99m Tc (technetium)

Dose

Half-life

Ideal case

High dose radiation (500 mrad)

Long 8-10 days

Lingual thyroid, retrostemal goitre; images are better

Low dose radiation (30 mrad )

Shorter 8-10 hours

Well differentiated carcinoma for bony metastasis.

Lowest radiation (5 mCi)

Shorter half-life 6-8 hours

Sensitive for nodal metastasis Lingual thyroid.

Fig. 19.7

Nomenclature of certain thyroid diseases • Ectopic thyroid Diagnosis by isotope scan or CT scan

• Thyroid tissue along the line of descent Example: Floor of the mouth, submental region, mediastinum

• Lingual thyroid Diagnosis by isotope scan or CT scan

• Swelling in the region of foramen caecum in tongue

• Dyshormonogenesis It is familial.

• Autosomal recessive condition with deficiency of peroxidase or dehalogenase (enzymes)

• Pendred's syndrome

• Dyshormonogenesis with congenital deafness

• Struma ovarii

• Malignant ovarian teratoma containing thyroid tissue

• Jod Basedow's disease (German word)

• Excessive iodine given for hyperplastic goitre resulting in hyperthyroidism

CLINICAL EXAMINATION OF THYROID SWELLING Diseases of the thyroid are very common and thyroid swellings are very often common cases in an undergraduate and postgraduate clinical examination. Hence, before discussing the various diseases of the thyroid gland, various aspects of the "CLINICS" are discussed in detail below. COMPLAINTS-HISTORY TAKING (Fig. 19.8) 1. Swelling: Long duration of thyroid swelling indicates benign condition, e.g. multinodular goitre (MNG), colloid goitre. • Short duration with rapid growth indicates malignancy such as anaplastic carcinoma. Majority of thyroid swellings do not produce pain. 2. Rate of growth: Usually slow-growing in benign disease. • If it is a rapid growth, it can be 'de novo' malignancy or malignancy developing in a benign lesion, e.g. follicular carcinoma in MNG. • Sudden increase in the size of swelling with pain indicates haemorrhage in the MNG (multinodular goitre). 3. Dyspnoea: Difficulty in breathing in a patient with goitre can be due to following reasons (Key Box 19.2). • Small goitre, rapid growth-anaplastic carcinoma infiltrating the trachea. • When lower border is not seen, retrostemal goitre.

............................. . KEY BOX 19.2

• • • •

DYSPNOEA IN GOITRE-CAUSES Infiltration of trachea Anaplastic carcinoma Lower border not seen Retrosternal goitre Tracheomalacia Long-standing MNG Cardiac failure Secondary thyrotoxicosis

• Hyperthyroidism causing arrhythmias leading to congestive cardiac failure can cause dyspnoea and orthopnoea. • Long-standing MNG compresses on the tracheal cartilages and produces pressure atrophy of tracheal cartilages. This is called tracheomalacia. 4. Dysphagia is relatively uncommon because oesophagus is a posterior structure and it is a fibromuscular tube. 5. Hoarseness of voice indicates malignancy. It always occurs in carcinoma thyroid infiltrating the recurrent laryngeal nerve (never in benign diseases of thyroid). 6. Toxic features suggestive of hyperthyroidism A.CNS symptoms are predominantly seen in Graves' disease (primary thyrotoxicosis-Key Box 19.3) • Tremors of the hand • Sweating • Intolerance to heat • Preference to cold • Excitability

Manipal Manual of Surgery

324

I

I

Swelling

• Duration • Progress • Rapid growth • Pain

HISTORY TAKING IN A CASE OF THYROID SWELLING

I

I

Local effects

Toxicity

• Dyspnoea • Dysphagia • Hoarseness of voice • Pain

• CNS with eye • CVS •GIT • Menstrual

I

I

Hypothyroidism

• Lethargy • Deposition of fat • Deep, husky voice • Intolerance to cold

Fig. 19.8: Summary of history taking

.

............................ . ............................. . KEY BOX 19.4

KEY BOX 19.3

GRAVES' DISEASE

• Goitre • Ophthalmic symptoms • Irritability • Tremors • Restlessness • Excitability Remember as GOITRE

• Irritability • Prominent eyes are observed by other persons. Double vision, oedema of the conjunctiva can be the presen­ ting complaints in late cases. B. Cardiovascular symptoms (CVS) are predominantly seen in secondary thyrotoxicosis. Even though both forms of thyrotoxicosis produce palpitations, it is a significant complaint in multinodular goitre with thyrotoxicosis (secondary thyrotoxicosis). Precordial chest pain and dyspnoea on exertion are late manifestations ofsecondary thyrotoxicosis (Summary-Fig. 19.8) ON EXAMINATION Inspection 1. The location ofthe swelling is in front of the neck, extend­ ing from one sternomastoid to the other sternomastoid, vertically from suprastemal notch to the thyroid cartilage. 2. The size and shape have to be mentioned. 3. Surface: Thyroid swellings can have the following types of surfaces a. Smooth-adenoma, puberty goitre, Graves' disease b. Irregular-carcinoma of the thyroid c. Nodular-multinodular thyroid 4. Borders are usually round. 5. Swelling moves up with deglutition because of the following reasons (Key Box 19.4): • Thyroid is enclosed by pretracheal fascia which is condensed to form a ligament posteromedially called

• • • • •

SWELLINGS WHICH MOVE UPWARDS WITH DEGLUTITION Thyroid swellings Subhyoid bursitis Pretracheal and prelaryngeal lymph nodes Thyroglossal cyst Laryngocoele

ligament of Berry. These are pairs ofligaments attached above to cricoid cartilage. During deglutition, the cricoid cartilage moves upwards and with it, the thyroid gland (give the patient a glass of water and check for movement with deglutition). • If there is restriction of movement, it can be due to - Malignancy with fixity to the trachea - Retrostemal goitre - Large goitre because of the size - Previous surgery 6. Movement on protrusion of the tongue suggests thyroglossal cyst. This test should be done when there is a nodule or a cyst in the region of isthmus of the thyroid gland. This test has no relevance in cases ofMNG or other thyroid swellings. Palpation It should be done from behind. 1. Size, shape, surface and border should be confirmed. Local rise of temperature is a feature of toxic goitres. Very large nodular surface is described as bosselated surface (Figs 19.9 and 19.10). 2. Consistency • Soft: Graves' disease, colloid goitre. • Firm: Adenoma, multinodular goitre. • Hard: Carcinoma, calcification in the MNG. 3. Confirm the movement with deglutition by holding the thyroid gland. 4. Intrinsic mobility of the gland is very much restricted in carcinoma because of infiltration into the trachea.

Thyroid Gland

325

Figs 19.9 and 19.10: Endemic goitre of 35 years duration turned into multinodular goitre. She presented with 3 months history of rapid increase in the size of the swelling. She underwent total thyroidectomy. Final histopathology report was follicular carcinoma thyroid. Observe large nodular surface-bosselati ons. (Courtesy: Dr Chidananda KV, Prof and Head of the Department and Dr Gopinath Pai, Prof of Surgery, KVG Medical College, Sullia, Dakshina Kannada, Karnataka. MBBS exam case 2007)

5. Sternomastoid contraction test is done when only one lobe is enlarged. In this situation the examiner keeps the hand on the side of the chin, opposite the side of the lesion and asks the patient to push his hand against resistance. If the gland becomes less prominent (as with thyroid swellings), it indicates the swelling is deep to the stemomastoid muscle. 6. Chin test (neck fixation test) is classically done in multi­ nodular goitre, wherein both lobes are enlarged. The patient is asked to bend the chin downwards against resistance. This produces contraction of both stemomastoids and strap muscles and the gland becomes less prominent (Fig. 19.11). 7. Special tests or methods of examination of thyroid gland: a. Crile's method is indicated when there is a doubtful nodule. Keep the thumb over the suspected area of the nodule and ask the patient to swallow. The nodularity is appreciated better with this test (Fig. 19.12). b. Lahey's method of examination of thyroid can be done from front as well as behind. In order to palpate the right lobe, push the gland to the right side and feel the nodules in the posteromedial aspect of the gland. The lobe becomes more prominent and thus nodules are appreciated better. c. Pizzillo's method is indicated in obese patients especially short-necked individuals. The patient is asked to clasp her hands and press against her occiput with head extended. Thyroid gland becomes more prominent and thus, palpation becomes better. d. Kocher's test: If gentle compression on lateral lobes produces stridor, it is described as positive. This is due to scabbard 1 trachea. Long-standing multinodular

Fig. 19.11: Chin test-neck flexion test-thyroid swellings become less prominent

Fig. 19.12: Crile's method to detect nodule wherein thumb is used

goitres causing tracheomalacia and carcinoma with infiltration into trachea may give rise to stridor. 8. Position of trachea: In cases of solitary nodule confined to one lobe, trachea is deviated to the opposite side. However, in cases ofmultinodular goitres, trachea need not be deviated because of symmetrical enlargement of both lobes. 9. Palpation of lymph nodes in the neck. If lymph nodes are significant, it indicates papillary carcinoma of the thyroid. 10. Palpation of common carotid artery: Draw a line from mastoid process to sternoclavicular joint. Then draw a horizontal line from upper border of thyroid cartilage. The point where these two lines meet is the site of bifurcation of common carotid artery. This artery should be palpated just below this point.

1 Scabbard is a sheath for holding a sword or other large blade (it is narrow and curved).

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Manipal Manual of Surgery

• In large multinodular goitres, the artery may be pushed laterally. Hence, pulsations are felt in the posterior triangle. Carcinoma of the thyroid engulfs the carotid sheath. Consequently, pulsations may be absent. Absent carotid artery pulsation is called Berry sign positive. Since the lumen is not narrowed, superficial temporal artery pulsations are felt normally. • Summary of the palpation (Key Box 19.5).

............................. KEY BOX 19.5

• • • • • • • • •

.

PALPATION TESTS Local rise of temperature, size, shape, surface, borders Consistency, movement with deglutition Intrinsic mobility test Sternomastoid contraction test and chin test Position of trachea Palpation of lymph nodes Pulsations of common carotid artery Special tests Evidence of toxicity

Percussion Percussion over the sternum gives a resonant note in normal cases. In retrosternal goitres, it gives a dull note. Auscultation • It should be done in the upper pole because of following reasons: Superior thyroid artery is a direct branch of external carotid artery. It is more superficial than inferior thyroid artery. • Presence of thrill and bruit are the features of toxic goitre. Systemic examination This includes CNS and eye signs, as in Graves' disease, examination of skeletal system to rule out metastasis as in carcinoma of the thyroid, and examination of cardiovascular system in cases of toxic goitre. These have been dealt with in detail in the corresponding topics. Deep tendon reflexes also have to be elicited-there is a slow relaxation phase in hypothyroidism. Diagnosis It is based on its anatomical location and features. It should be noted that the neural tumours arising from vagus nerve can present in the same location but it will not move with deglutition (Key Box 19.6).

•*•........_,,, 1. 2. 3. 4.

ANATOMICAL FEATURES OF THE THYROID GLAND� Thyroid gland is in front of the neck Deep to pretracheal fascia Moves up with deglutition Butterfly shaped when whole gland is enlarged.

Differential diagnosis • Simple goitre • Toxic goitre • Malignant goitre • Solitary nodule • Thyroiditis • Other rare causes of thyroid enlargement. GOITRE Definition Diffuse enlargement of the thyroid gland is described as goitre (it is derived from the Latin word, Guttur = the throat). Classification of goitre I. Simple goitre • Puberty goitre • Colloid goitre, iodine deficiency goitre (Figs 19.13 and 19.14) • Multinodular goitre (Fig. 19.15). II. Toxic goitre • Graves' disease; diffuse toxic goitre • Secondary thyrotoxicosis in multinodular goitre • Toxic nodule; other causes. III. Neoplastic goitre • Benign adenoma (follicular adenoma) • Malignant tumours: They are further classified as follows: A.PRIMARY • Well-differentiated carcinoma - Papillary carcinoma, follicular carcinoma. • Poorly differentiated carcinoma - Anaplastic carcinoma • Arising from parafollicular cells - Medullary carcinoma • Arising from lymphatic tissue - Non-Hodgkin's lymphoma. B. SECONDARY (Metastasis) Malignant melanoma, renal cell carcinoma, breast carcinoma produce secondaries in the thyroid, due to blood spread. IV. Thyroiditis • Granulomatous thyroiditis • Autoimmune thyroiditis • Riedel's thyroiditis. V. Other rare causes of goitre • Acute bacterial thyroiditis • Thyroid cyst

-

327

Thyroid Gland

Fig. 19.13: Observe smooth surface­ c olloid goitre of 15 years duration

Fig. 19.14: Colloid goitre showing minor nodularity

• Thyroid abscess • Amyloid goitres. PEARLS OF WISDOM

Multinodular goitres, malignant goitres, puberty goitres are common causes of goitre. Bacterial thyroiditis is rare. Riedel 's thyroiditis is very rare. MULTINODULAR GOITRE • A multinodular goitre is the end-stage result of diffuse hyperplastic goitre. Excessive metabolic demands cause an excessive enlargement of the thyroid. Therefore, it is common m women. • Metabolic demands increase during puberty. A goitre appearing during that period is called puberty goitre. A goitre can develop during pregnancy and is called pregnancy goitre. Both of them are physiological but may eventually develop into multinodular goitre (MNG). Aetiopathogenesis Multinodular goitre occurs due to continuous stimulation by TSH secreted from the anterior pituitary. 1. Puberty goitre, pregnancy goitre • It is seen in girls at puberty or during pregnancy when the metabolic demands are high and the production of T3, T4 are comparatively normal. Due to feedback mechanism, TSH levels increase, which stimulate thyroid gland and causes diffuse hypertrophy and hyperplasia. • This is also called physiological goitre and can be treated by giving tablet thyroxine (T4) 0.2 mg/day to suppress TSH. • Goitre may disappear if treatment is given in the stage of diffuse hypertrophy.

Fig. 19.15: Multinodular goitre showing nodules

2. Iodine deficiency goitre • Daily iodine requirement is about 100-125 micrograms. • Common in hilly/mountainous or low-lying areas because of decreased iodide content of water. This causes iodine deficiency goitre mediated by the same feedback mechanism. • This is treated with iodised salt (which is used in food) and iodine-containing preparations. • If the iodine deficiency status continues for a long time, it results in accumulation of colloid material in the gland and causes colloid goitre. • All these three types of goitre if left untreated will change to multinodular goitre (Fig. 19.16). • Stage I: Stage of diffuse hypertrophy and hyperplasia of thyroid. • Stage II: Due to fluctuating levels of TSH because of pregnancy, lactation, menstruation, etc. Some areas in thyroid are overstimulated and are converted to active follicles. • Stage III: The active follicle ultimately undergoes necrosis and many such necrosed follicles join to form a nodule. Many such nodules form a multinodular goitre. Nodules contain necrosed tissue, i.e. inactive tissue. The intemodular tissue is active. 3. Goitrogens such as cabbage (contains thiocyanates), drugs such as PAS and sulfonamides, cause goitre by preventing oxidation of iodide to iodine. Excess iodides inhibit organic binding of iodine and produce goitre. 4. Dyshormonogenesis (see Table 19.3) Clinical features • Common in females. Female: male ratio is 10: 1. Seen in the age group of 2�0 years.

328

Manipal Manual of Surgery Puberty

I

Genetic defect

Iodine deficiency

J1----------,

Goitrogens

Low levels of T3 and T4

I

Feedback mechanism J

.--------------1 TSH stimulation Diffuse hyperplasia 1----------, Mixed pattern 1------------1 Necrosed follicles

lnternodular tissue has follicles and it is functioning

Multinodular goitre .----�----, [After a few yearsJ Malignancy

Toxic MNG

Fig. 19.16: Pathogenesis of MNG

• Long duration of swelling in front of the neck, dyspnoea due to tracheomalacia and dysphagia are the presenting features. T he gland is nodular, firm in consistency and both the lobes are enlarged. Hard areas may suggest calcification and soft areas, necrosis. • Sudden increase in size with pain is mainly due to haemorrhage in a nodule. PEARLS OF

WISDOM

The most common site of a nodule is at the junction of isthmus with one lobe. Complications of multinodular goitre 1. Calcification in long-standing MNG. 2. Sudden haemorrhage in one of the nodules causes dyspnoea. 3. In 10-20% of cases, patients can develop secondary thyrotoxicosis with CVS involvement. Toxic multinodular goitre is also called Plummer's disease. Tachycardia can be graded as follows-Crile's grading Grade I < 90/min-mild Grade II 90 to 1GO/min-moderate Grade Ill> 110/min-severe 4. Follicular carcinoma in a long-standing goitre (8%).

Management of multinodular goitre Investigations 1. Complete blood picture (CBP), routine urine examination and fasting and postprandial blood sugar to rule out diabetes mellitus. 2. X-ray of the neck: Anteroposterior and lateral view. • To look for compression of trachea-to check feasibility of intubation during anaesthesia (Fig. 19.17). • To rule out retrostemal extension-soft tissue shadow seen. • Calcification in long-standing MNG. 3. Flexible laryngoscopy is done to see vocal cord mobility (this has replaced indirect laryngoscopy). 4. Ultrasonography: High frequency ultrasound is a very useful investigation specially in cases of solitary nodule. Even in multinodular goitres, ultrasound guided FNAC can be done. It can also detect clinically impalpable lymph nodes in the neck (suggest malignancy) (Key Box 19.7). • Ultrasound examination is inexpensive, easily done and has more advantages than disadvantages. Thus, it is often the first investigation in thyroid swellings. 5. Fine needle aspiration cytology (FNAC): It can be done in suspected hard nodule of multinodular goitre. It is a simple and useful investigation which can detect

329

Thyroid Gland

M$blWW',� "N INVESTIGATIONS-GOITRE 1. Simple goitres • Routine-blood and urine tests, chest X-ray • Indirect laryngoscopy-flexible laryngoscopy is better 2. Toxic goitres • Routine • T3, T4, TSH 3. Malignant goitre • Routine • Ultrasound, CT scan of the neck • FNAC

............................. . KEY BOX 19.9

PREVENTION OF MNG • Puberty goitre: 0.1 mg to 0.2 mg of thyroxine • Iodine deficiency goitre: Use iodised salt, sea food, milk, egg, etc. • Goitrogens: Avoid cabbage, drugs.

Fig. 19.17: X-ray of the neck-lateral view

IM$-WW',1 ULTRASOUND

�

• It has become the investigation of choice (front line) in thyroid diseases. • High frequency sonogram is used. • It can distinguish solid from cystic lesions. • It can measure the size of the nodule. • It can reveal multicentric nature of the goitre.

•

It can detect lymphadenopathy and can guide FNAC. • It can detect microcalcification-a feature of malignancy

• i echogenicity means i chances of tumour/malignancy • i sonolucent means i chances of cyst (benign).

malignancy. Since the treatment of MNG is often total thyroidectomy and an ultrasonogram can also help to rule out malignancy, FNA C is done only in suspicious cases ofMNG. 6. CT scan is done when you suspect retrostemal extension, doubtful resectability or large lymph nodes in the neck. They may also have intrathoracic lymph nodes (Fig. 19.55). Classification of investigations (Key Box 19.8) Prevention Prevention is mainly important in endemic area and is done by supplementing iodine (Key Box 19.9).

Why do we ask for ultrasound examination? Following clinical notes will give answers.

1. A 22-year-old lady was referred to us for a nodule in the right side of neck in the thyroid region that was moving with deglutition. She also had epigastric pain since 6 months. Clinical diagnosis of solitary thyroid nodule with hyperacidity was made. An ultrasound examination of the neck was requested and the report was a surprise. She was having a parathyroid adenoma with calcific pancreatitis and nephrocalcinosis. You know the diagnosis now-A case of hyperparathyroidism. 2. We sent a case of MNG to thyroid scan. The scan detected jugular lymphadenopathy on both sides. The diagnosis changed from MNG (benign) to malignancy (papillary carcinoma because of lymph nodes). Lymph nodes were not palpable on clinical examination in this patient. 3. A 21-year-old lady had a multinodular goitre with the entire gland replaced by nodules. Case was posted for a total/near total thyroidectomy. A senior experienced Professor asked one question, 'what is the duration of this swelling?' It was JO years. As per his advice, FNAC was done. FNAC report was lymphocytic thyroiditis. Surgery was cancelled. She was put on T-Eltroxin 0.1 mg, 2 tablets /day. She is on follow up now. Gland size is reduced to 30% in the last 2 years.

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Manipal Manual of Surgery

Treatment Three choices can be given to patients. • Subtotal thyroidectomy: In this operation, parts of right and left lobes and entire isthmus are removed in flush with tracheal surface leaving behind a little tissue in the tracheoesophageal groove to protect recurrent laryngeal nerve and parathyroid gland (Fig. 19.18). Some surgeons treat these patients with 0.2 mg ofthyroxine to suppress the TSH stimulation in the postoperative period, for a period of 2-5 years. Total thyroidectomy is the choice today p rovided complications such as recurrent laryngeal nerve, paralysis and hypocalcaemia due to removal of parathyroid glands can be avoided. Thus, it is desirable to do a total thyroidectomy if experience of the surgeon is good and in a high volume centre (Fig. 19.19). Medical: Small nodules-treat with Tab thyroxine (Eltroxin) 0.1 to 0.2 mg/day.

I r

· Early cases • Asymptomatic cases

I

• Observe, nodules may regress • Explain to the patieni about regular followup • Tab Eltroxin 0.1-0.2 mg/day

r

· If no improvement I • If growing I

• British favour this • No chances of recurrence • Avoids resurgery if carcinoma is detected after histopathological report TOTAL THYROIDECTOMY

Fig. 19.18: Subtotal thyroidectomy specimen

MULTINODUL AR GOITRE

I

• MNG with pressure symptoms • MNG with cosmetic worries • Patient desires surgery due to fear of malignancy

I•

Retrosternal extension J

1 Surgery 1

I

J

13 optionsJ • Americans favour this • Avoids permanent hypoparathyroidism • Avoids RLN paralysis • Incidence of recurrence is very less (argument for subtotal thyroidectomy)

SUBTOTAL THYROIDECTOMY

• Total lobectomy on one side, subtotal on other side is indicated if one entire lobe is involved • It preserves some thyroid tissue • Can avoid hypothyroidism, hypoparathyroidism

DUNHILL PROCEDURES

Fig. 19.19: Management of multinodular goitre-for diagrams see operative surgery chapter on thyroid diseases

Thyroid Gland

RETROSTERNAL GOITRE Very often, it is a multinodular goitre that develops in the neck and is slowly pulled down behind the sternum due to following reasons. 1. Negative intrathoracic pressure 2. Pretracheal muscles are strong in men 3. Short neck, obesity Rarely, it arises from an ectopic thyroid tissue. Classification I. Primary:It arises from ectopic thyroid tissue in the mediastinum.It also derives blood supply from media­ stinum.It is rare (1%) II. Secondary:It is the common variety-It is MNG which gets pulled down into thorax. Clinical types • Substernal: The most common type where the lower border of the gland is behind the sternum. • Intrathoracic: No thyroid is seen in the neck, diagnosed by radio-iodine scan. • Plunging goitre: When patient is asked to cough, intrathoracic pressure increases. As the thyroid plunges out, the lower border of gland is clearly seen in the neck. Clinical features of retrosternal goitre • It can be suspected when the lower border of the swelling is not seen. Most of the patients have difficulty in breathing or even stridor. Dysphagia is more common. • Engorgement of neck veins and superficial veins. These become more prominent when the hands are raised above the head, and the arms touch the ears-Pemberton's sign (Fig. 19.20).

Fig. 19.20: Observe the engorged veins due to retrosternal goitre. Such veins are also seen when mediastinal nodes are enlarged as in papillary carcinoma thyroid

331

Investigations They are similar to MNG. However, isotope scan is very useful in the diagnosis of intrathoracic goitres. • CT scan to localise and to know the size and extent Treatment It can be easily explored through the neck incision and removed. • Very rarely, a sternal split may be necessary. Summary (Key Box 19.10).

............................. . KEY BOX 19.10

• • • • • • •

RETROSTERNAL GOITRE Very often, it is an MNG with the lower border unseen Rarely from ectopic thyroid tissue Severe breathlessness even though small Drugs should not be given if it is toxic Pressure effects diagnosed by Pemberton's test Surgical excision is the treatment No radioiodine therapy

TOXIC GOITRE-THYROTOXICOSIS It is a complex disorder which occurs due to increased levels of thyroid hormones (hyperthyroidism) and manifests clinically with various signs and symptoms involving many body systems. Following are the causes of thyrotoxicosis: 1. Primary thyrotoxicosis (Graves' disease, exophthalmic goitre, diffuse goitre) 2. Secondary thyrotoxicosis: Secondary to nodular goitre (multinodular) 3. Solitary toxic nodule: Autonomous nodule which is not under the influence ofTSH but occurs due to hypertrophy and hyperplasia of gland (tertiary thyrotoxicosis). 4. Other causes of thyrotoxicosis • Thyrotoxicosis factitia: False thyrotoxicosis occurs due to overdosage of thyroxine, given for puberty goitre. • Jod-Basedow's thyrotoxicosis: Jod means iodine in the German language, Basedow means toxic goitre.Iodine­ induced thyrotoxicosis (iodine given for hyperplastic endemic goitres). •Initial stage ofthyroiditis: Hashimoto's thyroiditis,viral thyroiditis can produce temporary thyrotoxicosis features. • Very rarely, malignant goitres can be toxic (differentiated carcinoma). • Neonatal thyrotoxicosis occurs in babies born to thyrotoxic mothers. • TSH-secreting tumours of pituitary • Struma ovarii

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................

• Drugs: Amiodarone is an iodine containing preparation given as antiarrhythmic drug. Please note: In the clinical examination, primary thyrotoxicosis and secondary thyrotoxicosis are commonly kept as long cases. Hence, these two have been discussed below in detail and also see Table 19.5.

CLINICAL NOTES Following are three case reports which highlight the clinical symptomatology of primary thyrotoxicosis. 1. An 18-year-old girl visited many doctorsfor her complaint of loss of weight. She was investigated for tuberculosis (common disease in India), malignancy, etc. She was given unnecessary tonics. After nearly 6 months, when eye-signs started developing, it was proved to be Graves' disease. 2. A bank clerk's only complaint was that he could not sign the cheque because of excessive sweating. Thyroid gland was not palpable. His pulse rate was very high, investigations revealed that it was a case of primary thyrotoxicosis. On careful questioning, he admitted that he was a "nervous character". 3. A 24-year-old lady was being asked by her friends every day why her eyes were prominent. Her only complaint was prominent eyes. On careful questioning, she admitted having anxiety, tension, excitability.

GRAVES' DISEASE Aetiopathogenesis The exact aetiological factors responsible for the disease are not clear. Following are considered as possible aetiological factors (Key Boxes 19.11 and 19.12): 1. Autoimmune disorder is the first possible cause due to the demonstration of auto-antibodies in the circulation. Example: TSH receptor antibodies. It can also be associated with other autoimmune disorders like vitiligo. 2. Familial: The disease can run in families. Familial/genetic Graves' disease has been documented in identical twins. 3. Thyroid stimulating immunoglobulins (TSI) and long­ acting thyroid stimulator (LATS) are responsible for pathological changes in the thyroid gland in Graves' disease. They stimulate thyrocytes to grow and synthesise excess thyroid hormones. 4. Exophthalmos producing substance (EPS) is responsible for "ophthalmopathy" seen in Graves' disease. 5. Female sex, emotions, stress, young age also have been considered as other factors responsible for the disease. Pathology As a result of continuous stimulation, acinar hypertrophy and hyperplasia take place. The acinar cells which are normally flat, become tall columnar. The normal colloid

.

............................ .

KEV BOX 19.11

TRIGGERING FACTORS FOR GRAVES' DISEASE • • • •

Postpartum state Iodine excess Lithium therapy Infection-bacterial and viral

disappears and the cells are empty. However, rich vascularity is seen. Thus, small follicles with hyperplastic columnar epithelium is characteristic. Clinical features (see Clinical Notes) Primary thyrotoxicosis is 8 times more common in females than in males, especially in the age group of 15-25 years. Symptoms, signs and swelling appear simultaneously. Very often young women present with unexplained loss of weight in spite of good appetite. Diarrhoea occurs due to increased smooth muscle activity of small intestines. Intolerance to heat, preference to cold, fine tremors, excitability, hyperkinetic movements, excessive sweating are the other features. Free steroid hormone levels decrease Graves' disease. This results in decreased effective oestrogen at the ceU level which in turn causes oligomenorrhoea. Signs of primary thyrotoxicosis I. Signs of thyroid gland in Graves' disease Uniformly enlarged (mild degree) Smooth surface-no nodules (treated cases may have nodularity) Gland is soft or firm in consistency It is warm-highly vascular (Key Box 19.13) Auscultation-a bruit is usually heard.

............................. . KEV BOX 19.12

FEATURES OF GRAVES' DISEASE • • • • •

Female with strong family predisposition Extrathyroidal manifestations Middle or young age (30 to 50 years) Autoimmune mechanism Leukocyte antigen human (HLA) and T-lymphocyte antigen may contribute. • Enlargement of gland is diffuse Remember as FEMALE

.

............................

KEV BOX 19.13

PULSATILE THYROID SWELLINGS • • • •

Primary thyrotoxicosis Secondary thyrotoxicosis Follicular carcinoma Vascular malformations

.

Thyroid Gland

Fig. 19. 21: She was 42-year-old with a 2-month history of thyrotoxicosis that developed after viral infection. ( Courtesy: Dr Sreejayan, Prof. of Surgery, Calicut Medical College, Calicut, Kerala)

Fig. 19.22: He was 31-year-old with loss of weight of 12 kg in 3 months. There was diffuse enlargement of the gland with exophthalmos

II. Central nervous system (CNS) signs • Tremors of the tongue when the tongue is within the oral cavity and tremors of the outstretched hands are characteristics. A piece of paper may be placed on the fingers in doubtful cases for demonstrating the tremors of the hand. Extensor surface of the hand is used because extensors are weak when compared to flexors. •

Hyperkinetic movements. Always a moist, warm hand (shake hands).

Ill. Cardiovascular system (CVS) signs Pulse rate is always raised and rapid indicating tachycardia. Depending upon the pulse rate, thyrotoxicosis can be classified as follows: mild-90-100/minute, moderate­ ! 00-110/minute, severe-more than 110/minute. Palpitation and extrasystoles can also be found in primary thyrotoxicosis even though other cardiac features such as fibrillation and cardiac failure are rare. IV. Eye signs Prominent eyeballs and retraction of the eyelid result in thyrotoxic exophthalmos. This is due to retrobulbar de­ position of inflammatory cells and round cells with venous congestion resulting in oedema (Figs 19.21 to 19.23). • Levator palpebrae superioris muscle is innervated by oculomotor nerve which also carries sympathetic fibres derived from cavernous plexus for the smooth muscle part of the levator. Contraction of this muscle produces lid spasm. • This is aided by spasm ofMiiller's muscle, a sympathetic muscle which lies adjoining the levator palpebrae superior

333

Fig. 19.23: She was 48-year-old lady who had multi nodular goitre of 10 years duration. She also had lid-lag sign (rare in toxic MNG)

muscle. This is responsible for keeping the eyeball forwards. All these factors together produce a classical stare. 1. Assessment of exophthalmos • Upper sclera is seen above the limbus (upper margin of the cornea and conjunctiva-Dalrymple's sign. • Naffziger's method: Stand behind the patient and look at the supraciliary arch, by tilting the patient's head backwards. In normal cases, eyeball is not seen. In cases of exophthalmos, eyeball is protruded outside and hence it is seen. 2. Moebius' sign: Loss of convergence of eyeball occurs due to muscle paresis as a part of thyrotoxic ophthalmoplegia. Diplopia is due to weakness of extraocular muscles (inferior oblique-elevators). 3. Stellwag's sign: Infrequent blinking and widening of palpebral fissure is due to spasm of sympathetic fibres in the levator palpebrae superioris. 4. Joffroy's sign: Absence of wrinkling of the forehead when the patient is asked to look upwards. This occurs due to increase in the field of vision due to exophthalmos. 5. von Graefe's sign (Lid lag sign): When the patient is asked to look up and down, upper eyelid cannot cope up with the speed of movement of the finger because of the lid spasm. Hence, the lid lags behind. 6. Enroth sign: Oedema of eyelids and conjunctiva 7. Gifflord's sign: Difficulty in everting the upper eyelid. 8. Kocher's sign: When an attempt is made to lift the eyes higher, upper eyelid springs up more quickly than the eyebrows

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Summary (Table 19.4 and Key Boxes 19.14 to 19.16) Malignant exophthalmos • This occurs in untreated cases of Graves' disease. • If the disease continues, infrequent blinking secondary to exophthalmos results in constant exposure of the cornea to the atmosphere. This results in keratitis, corneal ulcer, conjunctivitis, chemosis and may even lead to blindness. This is called malignant exophthalmos.

.

............................ .

KEY BOX 19.14

THYROTOXIC EXOPHTHALMOS

• • • • • •

Proptosis and lid retraction result in exophthalmos Sciera is visible beyond limbus Naffziger's method to examine Staring look Typically seen in Graves' disease Rarely seen in secondary thyrotoxicosis

............................. . KEY BOX 19.15

INTERESTING 6 Ps OF GRAVES'OPHTHALMOPATHY

• • • • • •

.

Prominent eyes Periorbital oedema Papilloedema Proptosis Palpebral fissure widening Progression to blindness

............................ .

KEY BOX 19.16

GRADING OF THYROID EYE DISEASES

Grade O Grade 1 Grade 2 Grade 3 Grade 4 Grade 5 Grade 6

No signs or symptoms Only signs, no symptoms Both signs and symptoms Proptosis Extraocular muscle involvement Corneal involvement Loss of vision with optic nerve atrophy

• Malignant exophthalmos is probably due to autoimmum disease. • In late stages, optic nerve damage and blindness can occur. Important causes of exophthalmos

1 . Thyrotoxicosis 2. Primary CNS tumours: Meningioma, optic nerve glioma, haemangioma, lymphoma, etc. 3. Metastatic tumours: Neuroblastoma, central carcinoma. 4. Vascular: Cavernous sinus thrombosis-aneurysm of ophthalmic artery Causes of pulsating exophthalmos

1. 2. 3. 4.

Cavernous sinus thrombosis Carotid-cavernous sinus, A-V fistula Orbital vascular tumour Ophthalmic artery aneurysm

Treatment of thyrotoxic ophthalmopathy 1. Massive doses of steroids-methylprednisolone and metronidazole 2. Lateral tarsorrhaphy 3. Orbital decompression may be necessary in late cases 4. Guanethidine eyedrops are useful to decrease lid spasm and lid retraction. 5. Head end elevation and disease control. 6. Dark spectacles, 7% methylcellulose eye drops. V. Thyrotoxic myopathy Mild weakness of proximal limb muscles, ocular and frontalis muscles is not uncommon. On careful questioning, patient may admit difficulty in climbing steps. Weakness of extraocular muscles results in double vision (diplopia). Features suggestive of myaesthenia gravis, periodic paralysis can be found. • Myopathy improves with treatment. VI. Thyrotoxic dermopathy (Key Box 19.17) • Popularly called as pretibial myxoedema-is seen in thyrotoxicosis patients treated with surgery or antithyroid

Symptoms of thyrotoxicosis Symptoms of hyperthyroidism

Symptoms of increased adrenergic stimulation

In females

In children

Heat intolerance

Palpitations

Amenorrhoea

Rapid growth

Increased sweating, thirst

Nervousness, fatigue

Decreased fertility

Weight loss in spite of good caloric intake

Emotional !ability

Miscarriages

Hyperkinesis Tremors

Prominent stare is due to catecholamine excess.

Thyroid Gland

drugs. It is always associated with exophthalmos. It is seen in 1-2% of patients. • Bilateral symmetrical deposition of myxomatous tissue (glycosaminoglycans) mainly in the pretibial region, may also affect the foot and ankle, sometimes the entire leg below knee. Skin is dry and coarse (thickening ofskin by mucin-like deposits). Swelling is due to the obliteration of initial lymphatics by mucin (see page 111). • Pretibial myxoedema (misnomer) is nonpitting in nature and may be associated with clubbing of fingers and toes called thyroid acropachy (Key Boxes 19.18 and 19 .19). Responds to topical steroids and thyroid disorder treatment Thus 4 important features are seen only in primary thyro­ toxicosis, not in secondary thyrotoxicosis (Key Box 19.20).

11$-PWlllllllir,1 SKIN CHANGES

• • • • •

Pretibial myxoedema Pruritis Palmar erythema Thinning of hair Dupuytren's contracture

11$ • • • • • • • • •

W

335

lftl$-PWlllllllir,1 '"81 SOME MISNOMERS

• • • • • •

Pretibial myxoedema Mycosis fungoides White bile Adenolymphoma Sternomastoid tumour Malignant hydatid

Not seen in myxoedema Not a fungal infection Not white, not bile Not a lymphoma Not a tumour Not malignant

•,.,._,,, EXTRATHYROIDAL MANIFESTATIONS OF GRAVES' DISEASE

• • • •

�

Pretibial myxoedema Proximal myopathy Pachy (acropachy) Progressive ophthalmoplegia

At the end of clinical examination, commonly asked question is, how will you differentiate primary thyrotoxicosis from secondary thyrotoxicosis (Table 19.5).

PRETIBIAL MYXOEDEMA-MISNOMER

,, �

Acropachy-Clubbing of fingers and toes Coarse hair Red shiny skin Obliteration of initial lymphatics by mucin, oedema nonpitting Pretibial region, foot and ankle After a few years of toxicosis, it develops Cyanotic when cold H aluronic acid deposition } Y in dermis-bilateral and symmetrical Remember as ACROPACHY

Management of primary thyrotoxicosis Routine isotope scanning has been abandoned in toxic goitres except when toxicity is associated with nodularity. Investigations Routine investigations such as complete blood picture, fasting and post-prandial blood sugar estimation, flexible laryngoscopy are done. Serum T3, T4 and TSH are measured. T3 or T4 levels are high and TSH levels are low. The normal level of T3 is 1.3-3.5 nmol/L and normal level ofT4 is 55-150 nmol/L. • Sleeping pulse rate is counted after the patient is sedated with 30 mg of phenobarbitone. In a case of toxic goitre, the pulse rate remains high even during sleep because of increased metabolism. This is a simple bedside

Differences between primary and secondary thyrotoxicosis

I. 2. 3. 4. 5. 6. 7. 8. 9. I0. 11.

Age Symptoms and signs Skin over thyroid Consistency Surface Auscultation Eye signs Predominant symptoms Pretibial myxoedema Proximal myopathy Malignant exophthalmos

----

Primary thyrotoxicosis (Graves)

Secondary thyrotoxicosis (Toxic MNG)

20-40 years Appear simultaneously, duration is short Warm Soft or firm Smooth Bruit is common Commonly found CNS Seen in 1-2% patients Seen in 5% patients Can be seen

35-50 years Long duration of a swelling and short duration of signs Not warm Firm or hard Nodular Bruit uncommon Rarely found (lid lag) CVS Never seen Never seen Never seen

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investigation in cases of toxic goitre. In anxiety states, pulse rate may be high in the waking hours and it comes back to normal during sleep. Thyroid stimulating antibodies are elevated (TSH-RAbs) Measurement oflgG immunoglobulins (TSH-RAbs) is not essential to make the diagnosis of thyrotoxicosis. Treatment of primary thyrotoxicosis Aim of treatment I. To restore patients to euthyroid state II. To reduce the functioning thyroid mass to a very critical level (about 6-8 g of thyroid tissue) III. To minimise complications I. To restore the patient to euthyroid state (Table 19.6) • Other drugs such as potassium perchlorate are given in the dose of 200 to 400 mg daily. Propylthiouracil in the dose of 200 mg three times a day can also be given in patients who develop neutropaenia due to carbimazole. • Propranolol inhibits peripheral conversion of T4 to T3. This results in rapid control of tachycardia and surgery can be scheduled in a few days (within one week). It should be continued for one week after surgery because it does not interfere with synthesis of hormones. Please note • Iodine containing antiarrhythmic drug amiodarone may worsen thyrotoxicosis. • Propyl thiouracil is safe in pregnancy with Graves' disease. • Role of Lugol's iodine is doubtful. • Antithyroid drugs will not cure the disease. In selected patients (30-40%) remission is possible with regular intake of drugs. They may be continued for a maximum period of 2 years. If toxicity persists or recurs on stopping drugs, surgery is recommended. However, majority of the patients ultimately require surgery or radioiodine.

Block and replace treatment • If a small dose of T3 (20 µg up to 4 times/day) or T4 (0.1 mg/day) is given along with antithyroid drugs, there is less incidence of development of hypothyroidism and increase in the size of goitre.

II. To reduce the functioning thyroid mass 1. Subtotal thyroidectomy: In this procedure, the amount of thyroid gland removed is more than toxic multinodular goitre. However, being a toxic goitre, very little gland should be left behind so as to avoid persistent toxicity in the postoperative period. This quantity is difficult to measure. Hence, some surgeons advocate leaving thyroid tissue as small as the tip of the little finger, on both sides. By convention it is said that a total of about 6 8- g of the thyroid tissue should be left on both sides of the tracheo­ esophageal groove. 2. Total thyroidectomy can be offered to young patients with a small-sized gland. It controls the toxicity very faster. Hypothyroidism occurs but it is easy to treat. 3. Radioiodine therapy: This is a suitable alternative to surgery in cases of primary thyrotoxicosis in patients above the age of 30 (Key Box 19.21). III. To minimise complications Good preoperative preparation of the patient, good anaesthetic and surgical techniques, and good postoperative care wiJI reduce the complications of surgery. Thus, antithyroid drugs, subtotal thyroidectomy and radio­ iodine therapy are the three different modalities available for the treatment of primary thyrotoxicosis. The indications, merits and demerits of each treatment are given in Fig. 19.24. Treatment of secondary thyrotoxicosis Patients with severe cardiac damage entirely or partly due to hyperthyroidism are middle-aged or elderly with secondary thyrotoxicosis and the hyperthyroidism is not very severe. These patients develop atrial fibrillation and cardiac failure, if left untreated. In elderly patients, when the operative risk is unacceptable, radioiodine is given. Treatment with antithyroid drugs is started 48 hours later and continued until radioiodine has had its effect (6 weeks). If the cardiac symptoms are controlled well and anaesthesia risk is acceptable, subtotal thyroidectomy is done. However, the gland that is left behind should be equal to the distal phalanx of the thumb of the patient.

Antithyroid drugs: Routine preoperative preparation Drugs and mode of action

• Carbimazole: It blocks oxidation of iodide to iodine and coupling reactions thus reducing T3 and T4 levels. It is metabolised to methimazole after ingestion • Propranolol is a nonselective �-blocker, reduces tachycardia. • Lugol's iodine is given to reduce vascularity of the gland before surgery

Dose

Precautions/Side effects

10 mg, 6th hourly and maintenance dose of IO mg two to three times a day

Takes 12 weeks for its action It should be given 3-8th hourly interval Dangerous agranulocytosis can manifest as sore throat

20-40 mg, two or three times a day depending on the pulse rate 10---12 drops (minimum three times a day for 10 days before surgery)

Congestive cardiac failure Can precipitate bronchial asthma. T3 and T4 levels are not decreased It is bitter, has to be taken with orange juice

Thyroid Gland

I

I

I. Continuation of I anti-thyroid drugs

I J

TREATMENT OF GRAVES' DISEASE

[

'I. Surgery-subtotal/ total thyroidectomy

f Advantages

f Advantages

1. Avoids surgery 2. Less risk to life 3. Economical

I

[ Disadvantages

I

J

1. Long duration of treatment 1-2 years 2. Agranulocytosis, fever, skin rash 3. Missed doses 4. Relapses common-50%

I

I [ Ill. Radioiodine150 microcuries

J

f Advantages

1. In good hands, permanent cure to the patient 2. Cure rate is high

J

337

Disadvantages

J

1. Associated with mortality and morbidity 2. Side-effects of thyroidectomy 3. Recurrence

1. 2. 3. 4.

J

J

No surgery No drugs Easy, more cost-effective No RLN palsy

J

Disadvantages

J

1. Contraindicated in pregnancy, lactating women. 2. Permanent hypothyroidism in 10-15% patients. 3. Exacerbation of cardiac arrhythmias.

[indications[

[indications[

[indications[

1. Patient not willing for surgery 2. Postponement of surgery 3. Recurrence after surgery

1. Young patients, aged 25-35 years who are fit for surgery 2. Toxicosis with large goitre 3. Retrosternal goitre 4. Pregnant patients

1. Any age group after 25, very appropriate after 45 years of age 2. Recurrent thyrotoxicosis after surgery 3. Patients with cardiac symptoms 4. Small to moderate enlargement.

Fig. 19.24: Indications, merits and demerits of different modalities of treatments for primary thyrotoxicosis

Solitary toxic nodule Treatment • This is not under control of TSH. It is an automous nodule • The control of toxicity is in the usual manner--carbimazole and propranolol • This is an indication to do 1 131 scan or preferably IV 99mTc scan. Hot nodule takes up isotope (also in carcinoma). Rest of the gland does not. Treatment is either by hemithyroidectomy or radioactive iodine therapy-1 131 . Some specific thyroid conditions I. Thyrotoxicosis in children: Initially antithyroid drugs are given for 10-15 years followed by surgery. • Radioiodine is absolutely contraindicated as there is a fear of carcinoma and growth retardation (Key Box 19.21). 2. Thyrocardiac: It refers to a condition wherein cardiac dam­ age has resulted due to hyperthyroidism (Key Box 19.22). • Classically it happens in secondary thyrotoxicosis. It is usually seen in middle aged or old aged patients.

11$

RADIOIODINE THERAPY

,, �

• Today there is 'no restriction of age and gender'. (However, this is preferred in children only after completion of growth and in adults only after family is complete) • Absolute contraindication is pregnancy • Conception must be avoided for a period of 4 months after radioiodine therapy

.,.,_,,, • • • • • •

SECONDARY THYROTOXICOSIS EFFECTS ON CVS Tachycardia Wide pulse pressure Systolic scratch (Means-Lerman scratch) 1 Extrasystoles Atrial fibrillation Cardiac failure

�

Means-Lerman scratch is occasionally heard in left 2nd ICS during expiration due to hyperdynamic circulation in thyrotoxicosis (Described by J. Lerman and J.H Means of Massachusetts General Hospital)

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Manipal Manual of Surgery

Summary of toxic goitre Disease

First line

Treatment

1. Primary thyrotoxic cases

Antithyroid drugs

2. Toxic nodular goitre 3. Solitary toxic nodule

Antithyroid drugs Antithyroid drugs

4. Recurrent thyrotoxicosis after surgery 5. Toxic goitre in pregnancy 6. Toxic goitre in children

Antithyroid drugs

< 45 years-antithyroid drugs > 45 years-radioiodine or surgery Subtotal/total thyroidectomy > 45 years-radioiodine > 45 years-hemithyroidectomy Antithyroid drugs/radioiodine therapy

Propylthiouracil Antithyroid drugs

1st trimester drugs 2nd/3rd-surgery Continue drugs/surgery in selected cases

• Propranolol controls the disease very well. • Radioiodine therapy is the treatment of choice. 3. Hyperthyroidism in pregnancy • Invariably it is Graves' disease. • In the first trimester, surgery and radioiodine are contraindicated. Carbimazole and propylthiouracil cross the placenta. • Surgery, if necessary, can be done in 2nd trimester. 4. Apathetic thyrotoxicosis • Thyrotoxicosis in elderly wherein pulse rate is low, they appear to be hypothyroid rather than hyperthyroid. Thyroid gland is rarely palpable. • Lethargy and behaviour changes. Summary of the toxic goitre (Table 19.7) NEOPLASTIC GOITRE Adenoma • The benign tumours of the thyroid gland are not uncommon. They present as a solitary nodule, thus causing a worry to the clinician. Adenomas are of follicular type. • The diagnosis is established by histological examination. • Adenomas are treated by hemithyroidectomy/lobectomy. • However, FNAC cannot distinguish between a follicular adenoma and follicular carcinoma. Hence, a frozen section has to be done. MALIGNANT TUMOURS1 Thyroid is the only endocrine gland wherein malignant tumours are easily accessible to clinical examination. Thyroid is the only endocrine gland wherein malignant tumours occur in children, young age, middle age, old age, and in both sexes. Thyroid is the only endocrine gland wherein malignant tumours spread by all possible routes-local, lymphatic and blood spread.

Thyroid is the only endocrine gland wherein malignant tumours are usually nonfunctional. Malignant tumours of the thyroid are common. They are interesting tumours, having good prognosis if diagnosed early. Papillary and follicular carcinoma are well differentiated, medullary carcinoma are poorly differentiated. PAPILLARY CARCINOMA THYROID (PCT) Aetiology 1. Irradiation to the neck during childhood: In olden days radiotherapy was given for benign conditions such as acne in teenagers or enlarged tonsils or thymus gland. Those children had increased risk of papillary carcinoma thyroid. These indications are obsolete now. However, accidental radiation to the neck or radiation given to Hodgkin's lymphoma can precipitate the development of papillary carcinoma thyroid. 2. It can be a complication of Hashimoto's thyroiditis. 3. Papillary cancer of thyroid occurs more often in patients with Cowden's syndrome, Gardner's syndrome or Camey's syndrome. 4. Associated mutations: Chromosomal translocation involving RET proto-oncogene (tyrosine kinase) chromosome l Oq 11. PEARLS OF

WISDOM

Neck radiation increases risk not only for carcinoma thyroid but also for parotid gland tumours and hyperparathyroidism. Pathology It is made up of colloid-filled follicles with papillary projections. In some cases, calcific lesions are found which are called psammoma bodies. These are diagnostic of papillary carcinoma of thyroid. Characteristic pale, empty, nuclei are present in a few cases which are described as

1These were the teachings or sayings of Late Prof Sharath Chandra, FRCS, Professor of Surgery, Madras Medical College, Chennai (Conveyed to me by Late Prof. Subbu, MMC, Chennai, when for the first and last time, l sat with Prof. Subbu as a co-examiner at Govt. Medical College, Coimbatore, 1998).

339

Thyroid Gland

•

•

Orphan Annie eye nuclei. Thyroid gland has very rich intrathyroidal lymphatic plexus. Papillary carcinoma can be unifocal or multifocal (Figs 19.25 and 19.26). Papillary microcarcinoma: They measure 1 cm or less in diameter. Distant metastasis is extremely rare. Hence a simple hemithyroidectomy is the treatment of choice. Follicular variant of papillary cancer: This is a mixed lesion with a predominance of follicles over papillae. These are treated by total thyroidectomy. It is called Lindsay tumour. Tall cell papillary cancer: This is an aggressive and rapidly growing tumour. It occurs in elderly patients and should be treated by total thyroidectomy. Other details are given in Key Box 19.23.

Clinical presentation Young females are commonly affected (in the age of 20--40 years). It can present as a solitary nodule. Very often, the lymph nodes in the lower deep cervical region are involved and thyroid may or may not be palpable.

............................. . KEV BOX 19.23

• • • • • • • • • •

PATHOLOGY OF PAPILLARY CARCINOMA MICROSCOPY Calcification Cystic changes/necrosis Cuboidal pale cell with grooving Crowded nuclei Cytoplasmic inclusions-intranuclear Cartoon character-Orphan Annie Calcified deposits-Psammoma bodies Observe 7 Cs Types-Woolner classification Minimal/Occult/Microcarcinoma: Tumour of 1 cm or less in size with no invasion/no lymph node metastasis lntrathyroidal: Confined to thyroid gland Extrathyroidal: Invasion of adjacent structures.

When thyroid gland is not palpable, it is called occult (hidden). However, papillary carcinoma less than 1.5 cm in diameter is also called 'occult' (Key Box 19.24). A few patients present late to the hospital with fixed nodes in the neck, and fixed thyroid to the trachea with or without recurrent laryngeal nerve paralysis (Figs 19.27 to 19.32). PEARLS

OF

WISDOM

'It should be noted that papillary carcinoma can be offered as a clinical diagnosis only in the presence of significant lymph nodes in the neck along with a thyroid swelling.'

............................. . KEV BOX 19.24

Fig. 19.25: Orphan Annie-eyed nuclei

1. 2. 3. 4. 5. 6. 7.

Fig. 19.26: PCT showing papillae with fibrovascular cores lined by follicular cells with Orphan Annie nucleus ( Courtesy: Dr Laxmi Rao, Head of the Department, Pathology, KMC, Manipal)

PAPILLARY CARCINOMA-LYMPH NODE � METASTASIS-PECULIARITIES They may be palpable even when thyroid gland is not palpable-occult primary Very slow growing Very often, they are intracapsular They need not be hard, are often cystic and firm in consistency At operation, they are bluish in colour because of rupture of the papillae Presence of lymph node metastasis does not affect the prognosis Mostly central neck nodes are cleared . Dissection of posterior triangle and suprahyoid dissection is not necessary.

Prognostic criteria There are many prognostic criteria that have been used in cases of well-differentiated carcinoma. Various scoring systems are available for well-differentiated carcinomas. Some examples are given in the next page.

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Manipal Manual of Surgery

Fig. 19.27: Papillary carcinoma thyroid presenting as solitary nodule. FNAC gave the correct diagnosis. Total thyroidectomy was done in this patient

Fig. 19.28: This lady underwent total thyroidectomy for MNG. It was reported as PCT. After one year she presented with a solitary lymph node

Fig. 19.30: Papillary carcinoma thyroid in a 75-year-old lady-it behaves aggressively in elderly patients. Total thyroidectomy was done (uncommon presentation)

Fig. 19.31: Another case of papillary carcinoma thyroid with huge lymph node secondaries-advanced, neg­ lected case

AMES scoring A: Age less than 40 years-better prognosis M: Distant metastasis-poor prognosis E: Extent of tumour extracapsular spread-poor prognosis S: Size less than 4 cm, good prognosis

Fig. 19.29: T his lady underwenl hemithyroidectomy two years back ano came with recurrence in the opposite lobe. Completion thyroidectomy was done

Fig. 19.32: Same patient as in Fig. 19.31underwent total thyroidectomy with functional block dissection of the neck. See also the displacement of carotids

dilemma exists as to how to proceed. Straightaway, one can advice the patient for completion (total) thyroidectomy. According to another school of thought, if the patient is in low risk category with intrathyroidal malignancy and other factors favouring good prognosis, then 'wait and watch' policy can be undertaken.

AGES scoring A: Age less than 40 years better prognosis G: Grade of the tumour-high grade-poor prognosis E: Extracapsular spread-poor prognosis S: Size less than 4 cm, good prognosis

Treatment It can be discussed under three headings. Treatment of the primary, treatment of the secondaries in the lymph nodes and suppression of TSH.

MACIS scoring M: Metastasis A: Age C: Completeness of resection I: Invasion S: Size

I. Treatment of the primary/2 choices (A) Total thyroidectomy and (B) Lobectomy. A. Total thyroidectomy is the treatment of choice. It means removal of the entire thyroid gland. It has the following advantages (Key Box 19.25 and Fig. 19.33). PEARLS OF

Histological surprise If a patient undergoes hemithyroidectomy for suspicious adenoma and histopathology reported is papillary carcinoma,

WISDOM

After total thyroidectomy, thyroxine is not given for a period of 4 weeks so that thyroid remnants can be ablated with radioiodine-till TSH are levels are above 30 MIUIL

Thyroid Gland

18$•�,, PAPILLARY CARCINOMA TOTAL THYROIDECTOMY ADVANTAGES

Structures such as internal jugular vein, sternomastoid muscle, accessory nerve are not removed because lymph nodes are slow growing and they rarely spread/ outside the capsule of the lymph node. However, in exceptional cases of papillary carcinoma with infiltration to these structures, there should not be hesitation to remove these structures. Lateral aberrant thyroid: Initially believed to be thyroid tissue. It is actually metastasis into levels 3 and 4 lymph nodes from papillary carcinoma thyroid. No role for prophylactic neck dissection except in children See Figs 19.35 to 19.38 for various pictures of papillary carcinoma thyroid.

�

1. Easy to detect and treat residual or metastatic disease. 2. Easy to assess recurrence by thyroglobulin level estimation. 3. Eliminates contralateral occult cancer (multifocal in 80% patients) 4. Eliminates resurgery. 5. Eliminates risk of recurrence, thus improving survival. 6. TSH suppression can be done.

• •

Fig. 19.33: Total thyroidectomy-in majority of cases

Fig. 19.34: Lobectomy-low risk groups (hemithyroidectomy)

Dose of radioiodine 30-100 mCi. B. Lobectomy (hemithyroidectomy) means removal of one lobe and entire isthmus. Those who favour lobectomy have following arguments (Fig. 19.34): 1. Total thyroidectomy has high complication rates such as RLN paralysis, permanent hypothyroidism, permanent hypoparathyroidism, etc. 2. Recurrence in opposite lobe is only 5% which means in 95% of the cases, removal of the opposite Jobe is unnecessary. 3. Even if it occurs later, since it is not dissected at the time of initial surgery, the lobe can be removed easily. 4. Tumour multicentricity has little prognostic signi­ ficance. Thus in a few selected cases, lobectomy can be done. Advantages of lobectomy 1. No hormone replacement 2. No hypoparathyroidism 3. Need not test thyroid function regularly. II. Treatment of secondaries in the lymph nodes Mostly central neck nodes are cleared. If nodes are enlarged in the anterior triangle, they are dissected and removed en bloc along with fat and fascia. This is called functional block dissection (Berry picking means removal of enlarged lymph nodes only. It is no longer followed).

341

III. Suppression of the TSH This is an important aspect in the postoperative period because papillary carcinoma is a TSH dependent tumour. To prevent the patient developing hypothyroidism in the postoperative period and to suppress TSH, thyroxine 0.3 mg/ day is given. • Failure of suppression of TSH to a level < 0.1 mU/litre suggest inadequate dose of thyroxine or noncompliance. When to give tri-iodothyronine (T3) in the follow-up period of well-diflerentiated thyroid cancers? Patients who require regular radio-iodine for scanning and ablation should be given T3 because it acts quickly and it can be stopped and restarted quickly. On the other hand, T4 has to be stopped for almost 30 days prior to scanning and ablation rendering patients severely hypothyroid for 4 weeks. (Dose of T3 is 40-60 µg/day. It is very costly; not freely available). Management protocol of papillary carcinoma of the thyroid (Fig. 19.39) Summary of papillary carcinoma thyroid as shown in Key Box 19.26.

...

..........................

KEY BOX 19.26

.

SUMMARY OF PAPILLARY CARCINOMA THYRO� • • • •

Most common histological type of thyroid cancer (60 to 65%) Most often it is multifocal (80%) Commonly it spreads by lymphatic spread. Psammoma bodies are diagnostic of papillary carcinoma of thyroid. • Most often it presents as solitary nodule and with or without lymph node metastasis • Most commonly done procedure for papillary carcinoma thyroid is total thyroidectomy with or without functional neck dissection • Most of the patients (>95%) have 10-year survival rate.

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Fig. 19.35: This patient had undergone hemithyroidectomy in a peripheral hospital 15 years back. He did not receive any medication in these years. He presented with ulcerated nodules on the right side of the neck (lymph nodal mass). He success­ fully underwent completion thyroidectomy with functional neck dissection on the right side. Also observe sebaceous cyst on the right side of the chest wall ( Courtesy: Dr Annappa Kudva, Prof of Surgery, KMC, Manipal)

Fig. 19. 36: This 24-year-old lady presented with swelling in the submandibular region. FNAC revealed salivary tissue. Hence, submandibular sialoadenitis was diagnosed. At surgery, the salivary gland was separate from this mass which was excised. Histo­ pathology reported as papillary carcinoma arising from thyroglossal duct cyst in ectopic area. She is on L-thyroxine 0.3 mg/day for last 18 months. As of now she has no compli­ cations. ( Courtesy: Dr Padmanabha Bhat, Prof of Surgery, KMC, Manipal)

TNM STAGING IS GIVEN IN NEXT PAGE

Fig. 19.37: Functional neck dissectior is done for papillary carcinoma thyroid Routine central compartment dissectior is not required. Figure shows vagu� nerve, carotid artery and internal jugula1 vein. Sternocleidomastoid is retracted All these structures are preserved. Hence, the name functional neck dis­ section ( Courtesy: Dr Gabriel Rodrigues, Prof of Surgery, Dr Suresh BP, Asst Professor, Dept. of Surgery, KMC, Manipal)

PAPILLARY CARCINOMA THYROID

Total thyroidectomy with central neck dissection (no prophylactic lateral neck dissection)

Discontinue T4 for 4-6 weeks To achieve TSH above 30 mU/L Radionuclear scan Significant residual disease

Radio-iodine 30-100 µCi

Radio-iodine 100-200 µCi

START T4-0.3 mg/day

Fig. 19.38: Observe bluish colour lymph nodes at surgery and specimen of lymph nodes

Fig. 19.39: Management protocol of papillary carcinoma

343

Thyroid Gland

iiMJid§iiti

FOLLICULAR CARCINOMA

Thyroid cancer

•

Tumour Primary cannot be assessed Tx No evidence of primary TO Limited to thyroid, 2 cm or less T1 T2 Limited to thyroid > 2 cm but < 4 cm T3 Limited to thyroid > 4 cm Extending beyond capsule any size T4 Nodes Nx Cannot be assessed NO : No regional node metastasis N1 : Regional node metastasis Metastases Mx Cannot be assessed MO : No metastasis M1 : Metastasis is present

Incidence: Constitutes 17% of cases (Key Box 19.27). Follicular adenoma 20% are malignant and 80% are benign

Aetiology • Follicular carcinoma usually arises in a multinodular goitre, especially in cases of endemic goitre. It should be suspected when MNG starts growing rapidly. • It can present as solitary nodule also (Figs 19.40 and 19.41).

Anaplastic carcinoma zs always stage IV

Separate stage groupings are recommended for papillary or follicular (differentiated), medullary, and anaplastic (undifferen­ tiated) carcinoma

18$ • • • • •

INCIDENCE OF THYROID MALIGNANCY

Papillary carcinoma Follicular carcinoma Anaplastic carcinoma Medullary carcinoma Others

,, �

60-65% 15-20% 10-12% 5-10% 10%

Papillary and follicular thyroid cancer (age< 45 years): M T N Stage I AnyT Any N MO II Any T Any N M1 Papillary and follicular; differentiated (age .2'. 45 years): M N Stage T I T1 MO NO II T2 MO NO T3 Ill MO NO MO N1a T1-T3 IVA MO T4a N1b IVB T4b Any N MO IVG AnyT Any N M1 Anaplastic carcinoma (all anaplastic carcinomas are considered stage IV): M N Stage T Any N T4a MO IVA T4b Any N IVB MO IVG Any N M1 AnyT Medullary carcinoma (all age groups): N T Stage I T1 NO II T2, T3 NO Ill T1-T3 N1a IVA NO T4a N1a T4a N1b T1 N1b T2 N1b T3 T4a N1b NO, N1b T4a N1b T1-T4a IVB Any N T4b IVG Any N AnyT

M MO MO MO MO MO MO MO MO MO MO MO MO M1

Fig. 19.40: Follicular carcinoma thyroid-clini­ cally suspected-ultra­ sound showed microcalci­ fication-FNAC revealed follicular cells. She under­ went total thyroidectomy. Final report was follicular carcinoma thyroid

Fig. 19.41: A 35-year-old female with solitary nodule. FNAC revealed papillary carcinoma

Pathology Depending upon the property of invasion it is classified into: Noninvasive which means minimal invasion. Invasive refers to angio-invasion and capsular invasion, necessary for the diagnosis of follicular carcinoma of

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thyroid. The tumour cells line the blood vessels and get dislodged into the systemic circulation producing secondaries in the bones. Microscopically, most of the tumours are well-encapsulated (Fig. 19.42). Clinical presentation (Figs 19.49 to 52) It can present as a solitary nodule. The diagnosis is considered only after an ultrasound scan reveals some features of malignancy such as microcalcification. Peak age group is around 40 years (Fig. 19.43). In case of long-standing multinodular goitres, if the goitre is rapidly growing, hard or has restricted mobility, follicular carcinoma can be considered. Metastasis in the flat bones: The only clinical situation wherein a follicular carcinoma can be considered as the diagnosis is when a patient with a thyroid swelling presents with metastasis in the bone in the fom1 of bony swelling or pathological fractures. Commonly, secondaries develop in the flat bones such as skull, ribs, sternum, vertebral

Fig. 19.44: Chest X-ray show­ ing osteolytic lesion in the left Fig. 19.45: This lady presentec clavicle with secondary in the left seconc rib. She did not have thyroid swel ling. A case of occult follicula carcinoma

Fig. 19.46: Carefully watch right sternoclavicular joint-she had a tender bony swelling

Fig. 19.42: Follicular carcinoma thyroid showing well differentiated

follicles invading the capsule and capsular veins. Please remember follicular carcinoma cannot be diagnosed by FNAC but by histopathology only (Courtesy: Prof Laxmi Rao, HOD, Pathology, KMC, Manipal)

Fig. 19.43: Secondary deposit in the sternum in a patient who underwent near-total thyroidectomy for follicular carcinoma 5 years back

column because the flat bones retain red marrow for a longer time. When bony swelling is obvious and thyroid is not palpable clinically, it is called occult primary site. • The clinical features of secondary in the skull are: • They are rapidly growing • They are wann • Vascular and pulsatile • Underlying bony erosion may be present (Figs 19.44 to 19.52 and clinical notes).

A patient with a diagnosis of "Lipoma of the scalp" was posted for excision in the prone position. As the chief surgeon scrubbed and was about to paint the part, he could see the pulsatile nature of the swelling, which he had missed in the outpatient department. Fortunately, it was not excised. A needle was introduced and frank blood was aspirated. Surgery was cancelled. X-ray skull showed osteolytic lesion. She had a small thyroid nodule.

Thyroid Gland

Investigations • Routine investigations Ultrasound scan is done (Figs 19.53 and 19.54) to demonstrate nature of the nodule, whether solid or cystic and to guide FNAC. FNAC of the nodule. It should be remembered that FNAC cannot differentiate a follicular adenoma from follicular carcinoma. Hence, if FNAC reports as follicular cells, overtreat the patient by total thyroidectomy. Some follow with frozen section and proceed. This is not favoured by many. If it is reported as follicular carcinoma, no further surgery is required. If it is reported as benign, patient requires to be treated for hypothyroidism. Open biopsy is not taken in operable cases (Key Box 19.28).

............................. . KEY BOX 19.28

• Thyroid • Parotid • Testis

MALIGNANT TUMOURS WHEREIN OPEN BIOPSY IS NOT TAKEN

A 38-year-old lady underwent total thyroidectomy for a solitary nodule, with FNAC revealing follicular carcinoma. After 3 years, she came with pain on the right side of sternum. Careful examination revealed a tender, warm, bony swelling arising from sternoclavicular joint. The diagnosis was follicular carcinoma of the thyroid. No wonder it is said that a surgeon should have eagle's eyes! (Fig.19.48) MS exam case 2008, JNMC-Belgaum, Contributed by Prof Ashok Godhi, HOD of Surgery

Fig.19.47: Erosion of skull bone

345

MfflMIJWW',1 CAUSES OF SECONDARY IN THE SKULL

1. 2. 3. 4. 5.

• •

�

Follicular carcinoma of thyroid Renal cell carcinoma Hepatocellular carcinoma Prostatic carcinoma Bronchogenic carcinoma

CT scan in appropriate cases (Fig.19.55). Alkaline phosphatase-if increased, bone scan should be done. Plain X-ray of the involved bone can reveal osteolytic lesions (Fig. 19.48 and Key Box 19.29). When primary is not found, bone biopsy is required to find out the site of the primary.

Treatment of follicular carcinoma of thyroid I • Treatment of the primary Situation I: When a patient has enlarged thyroid gland and scalp swelling, total thyroidectomy is the treatment of choice. Secondaries do not take up the radioisotope ( 131 I) in the presence of primary tumour. Hence, lobectomy or hemithyroidectomy should not be done. • Situation II: A patient undergoes subtotal thyroidectomy for MNG and final report is follicular carcinoma thyroid. In such cases, it is better to do completion thyroidectomy within 7 days or after 4 weeks. • If done, within 7 days, or after 4 weeks it is relatively easy to do. Between 7 days and 4 weeks, the inflammatory process would have produced dense fibrosis, chances of injuring RLN, parathyroids are high. Situation III: A patient with solitary nodule-high suspicion of malignancy after sonography and FNAC reported as follicular cells, it is better to go ahead with total thyroidectomy.

Fig. 19.48: Total thyroidectomy in progress. This is the best option for most of Indian patients who present with fairly big lesions. The need to take a small dose of thyroid hormone lifelong is a disadvantage but if the report is 'malignancy', it is the treatment

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FOLLICULAR CARCINOMA THYROID-INTERESTING PICTURES

Figs 19.49 and 19.50: This lady presented with thyroid swelling of 5 years' duration with swelling in the scalp and sternum of 2 months duration. Bony swellings were painful-it was a case of follicular carcinoma of thyroid with multiple bony metastasis: Lady with three swellings

Fig. 19.51: Ulcerated secondary in the scalp bone from follicular carcinoma thyroid. See dilated veins in the neck. No other differential diagnosis in such patients

Fig. 19.52: This lady, who had undergone hemithyroidectomy for follicular carcinoma thyroid 3 years back , presented with recurrence. Total thyroidectomy could be done

Fig. 19.53: Ultrasound revealing recurrence (nodules) in both lobes of the thyroid gland ( Courtesy: Dr Chandrakanth Shetty, Prof of Radiodiagnosis, KMC, Manipal)

Fig. 19.54: Colour Doppler showing the relationship of the gland to major vessels of the neck ( Courtesy: Dr Chandrakanth Shetty, Professor of Radiodiagnosis, KMC, Manipal)

Fig. 19.55: CT neck and chest showing the large mass displacing the carotid artery, internal jugular vein thrombosis and mediastinal lymph nodes. Lymph nodes were cleared after sternotomy

Thyroid Gland

II. Treatment of the metastasis After total thyroidectomy, a whole body bone scan is done to look for metastasis in the bone. A single secondary can be treated by oral radioiodine therapy, followed if necessary by external radiotherapy depending upon the response of the tumour. Multiple secondaries, if present, are treated by oral radioiodine therapy. Ill. Postoperative thyroxine • In the postoperative period, patients should receive thyroxine 0.3 mg/day to suppress TSH and to supplement thyroxine. Prognosis: 15% mortality in 10 years. HURTHLE CELL CARCINOMA • Hiirthle cell carcinoma is a variant of follicular carcinoma (Key Box 19.30). It is more aggressive than follicular carcinoma. • These tumours are defined by the presence of more than 75% of follicular cells having oncocytic features. Tumour contains sheets of eosinophilic cells packed with mitochondria. They secrete thyroglobulin. • Even if Hiirthle cell adenoma is well encapsulated, it is potentially malignant. • It does not take up 131 1. Hence, it is less likely to respond to 131 1 ablation. • Higher mortality (20% at 10 years). • 99mTc-sestamibi scan can detectHiirthle cell carcinoma. Criteria to diagnose Hiirthle cell carcinoma Capsular/vascular invasion, distant metastasis. Higher chance of spread to lymph nodes compared to follicular thyroid carcinoma. Higher chances of spread to distant sites also. Treatment • Total thyroidectomy is the treatment of choice. In many cases ofHiirthle cell carcinoma, lymph nodes are enlarged. Hence, modified radical neck dissection is done (MRND). • TSH suppression and follow-up are regularly required. PEARLS

OF

WISDOM

All Hiirthle cell neoplasms are almost malignant and all adenomas are almost follicular In all suspicious solitary nodules of follicular cell origin, it is better to overtreat them by total thyroidectomy than relying upon the frozen section, to avoid second surgery. Follow-up of patients with papillary and follicular carcinoma thyroid-differentiated thyroid cancer •

Serum thyroglobulin (Tg): Thyroid is the only organ which produces thyroglobulin. Levels greater than 1 to 2 mg/ml in patients receiving replacement thyroxine

347

............................. . KEY BOX 19.30

• • • • •

HURTHLE CELL CARCINOMA More aggressive follicular carcinoma More than 75% malignant cells More chances of lymphatic spread More chances of distant spread More chances of mortality

therapy indicates presence of metastasis. Hence, assess the serum Tg response to injected recombinant human TSH, every year. In thyrotoxicosis and thyroiditis, thyroglobulin levels may be increased (Key Box 19.31).

............................. . KEY BOX 19.31

SERUM THYROGLOBULIN • Normal value: < 1-35 µgm/litre • Above 50 µgm/litre suggest malignancy • Above 100 µgm/litre suggest pulmonary or skeletal metastasis • It is produced by follicular cells of the thyroid. Hence elevated in well-differentiated carcinoma. Not in medullary carcinoma thyroid. • Hence it is a tumour marker of well-differentiated carcinoma • Anti-thyroglobulin antibodies (ATA) TgAb are found in Hashimoto's thyroiditis and in Graves' disease

Ultrason ography or MRI scans of the neck for localisation of residual or recurrent tumour. ANAPLASTIC CARCINOMA Incidence 10-12% of cases Clinical features (Key Box 19.32) Common in elderly women around 60-70 years of age. • Majority of the patients present with rapidly-growing thyroid swelling of short duration. The surface is irregular and consistency is hard. Early infiltration of the trachea results in stridor (scabbard trachea).

............................. . KEY BOX 19.32

ANAPLASTIC CARCINOMA THYROID • • • • • •

The most rapidly growing thyroid malignancy Advanced age group at presentation Advanced nature of presentation Gross local infiltration-Berry's sign positive No form of treatment is successful Intrinsic carcinoma of larynx spreading outside and infiltrating the skin should be considered as a differential diagnosis

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•

Manipal Manual of Surgery

Infiltration of carotid sheath: In such cases, common carotid artery pulsation will not be palpable. This is described as 'Berry sign positive'. Early fixity is characteristic. Thus, the resectability rate is almost nil (Figs 19.56 and 19.57).

Diagnosis It is established by FNAC, other investigation being CT scan (Fig. 19.58).

Fig. 19.58: Highly pleomorphic malignant cells. (Courtesy: Pr
4

• • • •

• • • •

• • • •

Early menarche, late menopause Nulliparous Age> 35 at first birth Post-menopausal obesity

Radiation exposure Breast cancer in one breast Mammographic dense breast One first degree relative (Mother/sister) with breast cancer

• Alcohol> 2 units/day • Hom10ne replacement therapy • Proliferative benign breast disease (LCIS and ADH)

Radiation to mantle field in Hodgkin's lymphoma Ductal carcinoma in situ Lobular carcinoma in situ Two first degree relatives with breast cancer

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Sex-Female

White race

History of irradiation of the chest

Cancer breast

Carcinoma in one breast

Incidence of cancer

Increasing age

0

10 20 30 40

Hereditary

50Yr

Fig. 21.37: Carcinoma breast-probable risk factors

18$••1WW',.'� BENIGN BREAST DISEASE WITH RISK OF MALIGNANCIES

• • • • •

•

�

Lobular carcinoma in situ!LCIS Ductal carcinoma in situ-DCIS-10 fold increase Atypical ductal hyperplasia- ADH/atypical lobular hyperplasia/ALH-5 fold increase Florid hyperplasia-2 fold increase lntraductal papilloma-2 fold incresae Radial scar and sclerosing adenosis: Both are microscopic findings. They can be felt as hard lesions. Radial scars are less than 1 cm. Doubtful role in malignancy or no increased risk. Fibroadenoma: No increased risk

7. Diet: Increased risk has been found in postmenopausal obese women and is due to increased synthesis of oestrogen (oestradiol) in the body fat. As a result of aromatisation of androgens in adipose tissue, circulatory oestrogen levels are increased. Alcohol intake is associated with a 1.5 fold increased risk of breast cancer. Vitamin C may have protective value. Increased intake of saturated fats and reduced intake of phyto-oestrogens increases risk. 8. Endocrinal causes: a. Longer the cumulative period of menstruation more the risk (early menarche and late menopause) b. More the cumulative period of lactation better the protection (more children, each child breastfed longer)

c. More abortions and each occurring later increases risk. d. Hormone replacement therapy (HRT) in postmenopausal women increases incidence of breast cancer particularly when a combination of oestrogen and progesterone is used. • Oral contraceptive pills are thought not to increase breast cancer. 9. Chest wall radiation: Young children/women who have received mantle radiation for Hodgkin's disease have increased risk ( 19%) by the age of 50. 10. Geographical: Carcinoma of the breast is the disease of white, western women. It is rare in Japan and Taiwan. Genetic predisposition exists in a few cases, especially in bilateral breast carcinoma. Pathology • Majority of the tumours arise in the ductal epithelium (90%) which is called the ductal carcinoma and about 10% arise within lobular epithelium (lobular cancer). DESCRIPTION OF THE TERMS USED PREVIOUSLV Scirrhous carcinoma (Fig. 21.38) • It is the most common form seen in about 60-75% of patients. In this variety, there is increased fibrotic reaction and less cellular reaction. It presents as a hard lump. Hence the name, sciIThous carcinoma. It produces grating sound when it is cut. Cut surfaces are concave. The chalky white necrosis and calcification may be visible occasionally. This

-

Breast

is the type of lesion which produces retraction of the nipple, infiltration of the skin and fixity to the chest wall. • Malignant cells are round to polygonal with dark nuclei. Perivascular and perineural space infiltration is common. • Atrophic scirrhous is an infiltrating duct carcinoma seen in elderly patients when there is atrophy of the breast. Medullary carcinoma of the breast (Fig. 21.39) It is seen in around 15% of cases. It tends to occur in well­ formed breasts in the reproductive age group and it feels more soft than hard. In addition to undifferentiated cells, occasionally well differentiated gland formation is present. Hence, the name, medullary adenocarcinoma. Presence of lymphatic infiltration is thought to represent a good host response, thus indicating a good prognosis. Inflammatory carcinoma (Fig. 21.40) • It constitutes less than 1% of all cases of carcinoma breast. • Dermal lymphatic invasion is characteristic. • Predominantly seen during pregnancy and lactation. • Malignancy grows so rapidly that it invades more than half of the breast tissue. It comes under locally advanced breast cancer (LABC).

Fig. 21.38: Scirrhous carcinoma-with nipple retraction

395

• Redness, pain and enlargement appear so suddenly that it is diagnosed as breast abscess. Hence the name, mastitis carcinomatosa. It is differentiated from breast abscess by absence of fever and presence of gross peau d'orange due to blockage of subdermal lymphatics. • Blunder biopsy (incision) should not be done thinking that it is an abscess (Fig. 21.41). • This variety has the worst prognosis. • High angiogenic and angioinvasive capability. Most of them are ER negative. PEARLS OF

WISDOM

Mastitis carcinomatosa comes under Stage T4D. Paget's disease of the nipple (Fig. 21.42) • It is a misnomer. It is not a disease of the nipple but an intraductal carcinoma involving excretory ducts that infiltrates nipple and areola early. • Nipple can be ulcerated, fissured and cracked. Oozing is present. • In advanced cases, entire nipple is destroyed (ulcerated). • Lump appears much later than changes in the nipple.

Fig. 21.39: Medullary carcinoma-common in middle age

Fig. 21.41: Another case of inflammatory carcinoma. It has been incised mistaking it for an abscess

Fig. 21.40: Inflammatory carcinoma­ stage T4D

Fig. 21.42: Paget's disease of the nipple-totally destroyed nipple

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• Microscopically, large hyperchromatic cells with clear cytoplasm or clear halo are seen. • It is due to intracellular accumulation of mucopoly­ saccharides. These cells are called Paget's cells. • It is differentiated from eczema as follows (Key Box 21.13).

Multifocality refers to occurrence of a second in situ breas cancer within same breast quadrant as the primary in sill cancer (Figs 21.43 and 21.44) Upper and outer quadrant is commonly involved (60-65% because breast tissue is more there.

19¥11•,__.,,, ECZEMA • Seen in lactating women • Bilateral • Itching is present • Responds to antibiotics and local treatment

• • • •

PAGET'S DISEASE Seen in elderly women Unilateral Itching is absent Does not respond to antibiotics

�

Colloid carcinoma • It is diagnosed because of production of mucin, intracellu­ larly and extracellularly. • Prognosis of this variety of carcinoma breast is better than other infiltrating duct carcinomas. Definitions-current nomenclature • Carcinoma in situ: This term is used when cancer cells do not invade through basement membrane. Examples: Lobular carcinoma in situ (LCIS) and ductal carcinoma in situ (DCIS) (Table 21.7). • Invasive carcinoma: This term is used when cancer cells invade through basement membrane. A few examples are Paget's disease of the nipple, adenocarcinoma (scirrhous, simplex, medullary carcinoma, etc). PEARLS OF

WISDOM

Multicentricity refers to occurrence of a second in situ breast cancer outside the breast quadrant of primary in situ carcinoma.

Figs 21.43 and 21.44: Multicentric and multifocal-this is the reason behind getting a mammogram before doing a breast conservative surgery

Invasive breast carcinoma Depending upon specific histologic features, they are further classified into subtypes which are given below. However, it is interesting to note that 80% of invasive breast cancers are described as invasive, ductal carcinoma of 'no special type' (NST). Foot-Stewart classification of invasive breast cancer I. Paget's disease of the nipple JI. Invasive ductal carcinoma: 85% (Fig. 21.47) a. Adenocarcinomas [scirrhous, simple, NST (80%)] b. Medullary carcinoma 4% c. Mucinous (colloid) carcinoma 2% d. Papillary carcinoma 2% e. Tubular carcinoma 2% III. Invasive lobular carcinoma I 0% IV. Rare types: Adenoid cystic, squamous cell, apocrine.

Comparison of lobular carcinoma in situ (LCIS) with ductal carcinoma in situ (DCIS)

• • • • • • • • • •

Origin Incidence in males Multicentricity Bilateral occurrence Incidence of invasive cancer Change into invasive cancer Age (years) Incidence Clinical sign Mammographic signs

LCIS

DCIS

Terminal Duct Lobular Units (TDLU) Not found 60 to 90% 50 to 70% 25 to 35% of women LCI can give rise to both lobular and ductal invasive carcinoma 44-47 2-5% None None

Epithelium that lines minor breast ducts In 5% male breast cancer 40 to 80% 10 to 20% Increased by 5-fold DCIS gives rise to invasive ductal cancer 54-58 5-10% Mass, pain, nipple discharge Microcalcification

Breast

Fig. 21.45: Comedocarcinoma in situ-it is a DCIS-dilated terminal duct lobular units (TDLU) lined by malignant epithelial cells with luminal necrosis. No invasion into stroma. Slowly, the lumen gets occluded, can present as cyst and later calcification

397

Fig. 21.47: Infiltrative ductal carcinoma: Infiltrating islands of malignant cells forming glands and trabeculae surrounded by desmoplastic stroma (Courtesy: Dr Laxmi Rao, HOD, Pathology)

PEARLS OF

WISDOM

There is no role for chemotherapy in DCIS. Modified Van Nuys Prognostic Index Score 2 l 16-40 Size (mm) .:S 15 1-9 Margins (mm) > 10 Non-high Pathology Non-high grade without grade with necrosis necrosis > 61 Age (years) 40-60

3

.:S 41 >1 High grade with or without necrosis .:S 39

5-year disease free survival for VNPI 4 to 6 = 100%. Fig. 21.46: Normal vs carcinoma in situ: Here you can see normal breast lobule (left) and in situ carcinoma (right) (Courtesy: Both Figs 21.45 and 21.46 by Professor Laxmi Rao, Head, Dept. of Pathology, KMC, Manipal)

NONINFILTRATIVE LESIONS Carcinoma in situ • It can be ductal-ductal carcinoma in situ (DCIS) • Lobular-lobular carcinoma in situ (LCIS) This is a type of cancer without infiltrating epithelial basement membrane. No lymph nodes will be enlarged. Hence, no role for axillary block dissection. 5-years disease free survival for DCIS is calculated by using modified Van Nuys prognostic index for ductal carcinoma in situ (DCIS).

PEARLS OF

WISDOM

Lobular carcinoma is more dangerous because it is multifocal and bilateral. • Lobular carcinoma refers to a lesion developing from the acini or terminal ductules of the lobule. • More in situ carcinomas are diagnosed because of mammography and most of the breast conserving surgery is done in this group of patients. Comedocarcinoma of breast (Figs 21.45 and 21.46) • Comedos means cast or plug. It is a peripheral carcinoma wherein the tumour cells block the ductules by forming a case or plug producing a small cystic lesion. It is an example for intracystic carcinoma.

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CLINICAL FEATURES OF CARCINOMA BREAST • Lump in the breast is the most common presentation. It is the breast tissue in the upper and outer quadrant which is most frequently involved (65%). Typically it is hard and irregular but it can also be firm. Fixation to the skin, ulceration, peau d'orange, fixation to pectorals and chest wall occurs late ( Figs 21.48 and 21.49). • Bleeding per nipple is an uncommon symptom of carcinoma of the breast. It involves multiple ducts and is unilateral. • About 5% of patients present with bony metastasis give rise to bony pains, i.e. pathological fractures, paraplegia, quadriplegia, etc.

Fig. 21.48: Scirrhous carcinoma presenting as hard lump with peau d'orange

• Centrally retracted nipple: Recent retraction indicate malignancy. It is due to fibrosis caused by extension o growth along the lactiferous duct. Remote retraction i either idiopathic or due to recurrent mild infectio1 (mastitis). PEARLS

OF

WISDOM

Circumferential retraction is due to carcinoma. Slit­ like retraction is due to duct ectasia orperiductal mastitis.

Causes of retraction of the nipple (Figs 21.50 to 21.59: • Carcinoma of the breast • Chronic mastitis • Congenital • Chronic disease-tuberculosis • Level of the nipples: The nipple may be elevated and retracted (Fig. 21.50) because of fibrosis induced by the growth. One should carefully watch for the level of nipple to detect an early case of carcinoma. Destruction of the nipple is a feature of Paget's disease of the nipple. 2. Areola • Presence of peau d' orange indicates the tumour infiltrating the areola. It has been compared to orange skin because of folJowing reasons: - The areola becomes thick because of lymphatic obstruction giving rise to lymphoedema. - Fixation of hair folJicles and sweat glands to the underlying malignancy. 3. Skin over the breast • Puckering or dimpling of skin is due to thin fibrous bands which are embedded in the subcutaneous fat and are attached to the skin and pectoral fascia called ligaments of Cooper which are infiltrated by the malignancy. Multiple nodules indicate advanced disease. 4. Lump-if visible, give details 5. Oedema of the arm is due to lymphatic blockage caused by lymph nodes in the axilla.

Fig. 21.49: Carcinoma breast presenting as lump and ulceration

CLINICAL EXAMINATION OF A CASE OF CARCINOMA OF THE BREAST (Figs 21.50 to 21.59) Inspection should be done in three positions: I. Hands by the side of the patient 1. Nipple • Bloody discharge indicates duct carcinoma.

II. Hands raised above the head 1. Peau d'orange (on elevation of hands), becomes more prominent. Ill. Bending forward In cases of carcinoma infiltrating the chest wall, the breast will not fall forward on bending. Palpation 1. Local rise of temperature and tenderness are usually not found in cases of carcinoma of the breast. However, rapidly growing carcinoma and inflammatory carcinomas do exhibit local rise of temperature, redness and tenderness.

Breast

Fig. 21.50: Slit like retraction

399

Fig. 21.51: Another case of nipple retractionchronic abscess

Fig. 21.53: Large lump with ulceration and bleeding

Fig. 21.54: Retracted nipple

Fig. 21. 56: Retraction of the nipple and peau d' orange

Fig. 21.58: Paget's disease of the nipple ( Courtesy: Dr Sreejayan, Prof. of Surgery, Calicut Medical College, Calicut, Kerala)

Fig. 21.52: Retraction of the nipple-classical feature of intraductal carcinoma

Fig. 21.55: Peau d' orange

Fig. 21.57: Right nipple is elevated and retracted

Fig. 21.59: Cancer en cuirasse ( Courtesy: Dr Sreejayan, Prof. of Surgery, Calicut Medical College, Calicut, Kerala)

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2. Describe the lump: The lump is the commonest presentation of carcinoma of the breast. The upper and outer quadrant is the commonest site of carcinoma of the breast because of more breast tissue in that quadrant. Typically, the lump is hard and irregular. However, very often carcinoma breast can present as a firm lump. In mastitis carcinomatosa the lump can be soft due to tumour necrosis. 3. Intrinsic mobility may be present but it moves with the breast tissue (fibroadenoma moves independent of breast tissue). 4. Plane of the swelling • Lift the skin. If it is not possible, it indicates that the tumour is fixed to skin. • Pectoralis major contraction test: Ask the patient to keep the hands on the flanks and press against the hip. If the lump cannot be moved after contraction, it indicates fixity to pectoralis major. • Fixity to the chest wall is assessed by 2 methods: 1. A tumour which is fixed to the chest wall will not be mobile when pectoralis major is relaxed. 2. Breast will not fall forwards. • Serratus anterior contraction test by pressing the hand against the wall. The test has to be done when the tumour is situated in the outer and inferior quadrant. Axillary lymph nodes examination There are 5 groups of nodes in the axilla which are described under lymphatic drainage of the breast. However, very often, central group of nodes and pectoral nodes are enlarged. It is very difficult to feel the apical group of nodes. If the axillary nodes are hard, with or without fixity, they are significant. • Soft to firm nodes need not be malignant but can be due to secondary infection because of fungating, ulcerating growth. PEARLS OF

WISDOM

Presence of supraclavicular lymph nodes does not indicate metastatic disease. Examination for distant metastasis I. Opposite axilla and opposite breast 2. Abdominal examination for secondaries in the liver which present as nodular liver, ascites and Krukenberg's tumour­ bilateral bulky ovarian metastasis. 3. Rectal examination to rule out deposits in rectouterine pouch 4. Respiratory system examination to rule out effusion.

5. Bony tenderness should be looked for in the spine, Ion; bones, skull, etc. Spread of carcinoma of the breast

1 . Local spread As the tumour grows in size, it infiltrates the skin causini ulceration, fungation, bleeding, foul smelling discharge Later, it involves pectoral muscles, chest wall, etc. 2. Lymphatic spread • Central group, pectoral, lateral, subscapular anc supraclavicular nodes. • Medial quadrant tumours may involve the interna mammary glands in the upper three or four intercosta spaces, close to the sternum. • Lymphatics from inner medial quadrant of the breast penetrate the rectus sheath and join the intraperitonea lymphatics, thus producing ascites, Krukenberg's tumoun (in premenopausal patients, ovary is vascular and fertile), rectovesical deposits, secondaries in the liver. 3. Blood spread • Secondaries in flat bones are common (vertebral column, femur, ribs, scalp, etc). • Secondaries in brain results in headache, vomiting and blurring of vision. • Malignant pleural effusion is the common cause of death in carcinoma of breast. Staging (see page 401) INVESTIGATIONS l. Complete blood picture: Hb% may be decreased. 2. Increased ALP levels in the blood suggests bone metastasis or liver metastasis 3. Mammography (Figs 21.60 to 21.63) • This has become the first diagnostic investigation • Diagnostic accuracy is about 90-95%. • It should always be combined with a clinical examination • Mammography is done when there is a doubt about the diagnosis • Mammo gram cate gorie s are grouped under­ Bl-RADS-Breast imaging reporting and data system

This is a classification system that is currently used in clinical radiology practice now. Procedure • A selenium coated X-ray plate is used directly in contact with the breast and the breast is exposed to low voltage and high amperage X-ray. • 2 views: (a) Mediolateral and (b) craniocaudal Indication • Coarsely nodular breast • Fibroadenosis • Woman, aged 40 years with family history of breast cancer

Breast

fillt':fitdilUd

Breast cancer (Ca)

Tx primary tumour cannot be assessed. no evidence of tumour TO Tis carcinoma in situ Tis(DCIS) ductal carcinoma in situ Tis(LCIS) lobular carcinoma in situ Tis(Paget's) Paget's disease of nipple with no tumour T1 2 cm or less than in dimension T1mic microinvasion 0.1 cm or less in greatest dimension 0.1-0.5 cm in greatest dimension T1a 0.5-1 cm in greatest dimension T1b T1c 1-2 cm in greatest dimension T2 2-5 cm in greatest dimension > 5 cm in greatest dimension T3T4extension into chest wall including intercostal T4a muscles, ribs, serratus anterior (not pectoralis major) extension into skin-peau d' orange, ulceration, T4b cancer en cuirasse/satellite lesions (no puckering/ dimpling) T4c 4a + 4b T4d inflammatory carcinoma/mastitis carcinomatosa Nx NO N1 N2 N2a N2b N3 N3a N3b N3c Mx MO M1

cannot be assessed no regional lymph nodes metastasis to mobile ipsilateral axillary lymph node metastasis to ipsilateral axillary lymph node fixed/matted to one another or other structures ipsilateral internal mammary nodes in the absence of clinically evident axillary lymph node metastasis to ipsilateral infraclavicular lymph node ipsilateral internal mammary and axillary lymph node metastasis in ipsilateral suraclavicular lymph node cannot be assessed no metastasis metastasis present

iii4ii§;M@IUi Stage O Stage I Stage Ila

Stage llb Stage Illa Stage lllb Stage Ille Stage IV

401

Tis T1 TO T1 T2 T2 T3 TO,T1,T2 T3 T4 AnyT AnyT

NO NO N1 N1 NO N1 NO N2 N1, N2 N0-2 N3 Any N

MO MO MO MO MO MO MO MO MO MO MO M1

Regional lymph node - pN (pathologic)-done after axillary block dissection and i - immunohistochemistry Cannot be assessed pNx pNOb no regional lymph node metastasis histologically pNO(i-) no regional lymph node metastasis histologically, negative IHC pNO(i+) no regional lymph node metastasis histologically, positive IHC pNO(mol-) no regional lymph node metastasis, negative molecular findings (RT-PCR ) pNO(mol+) no regional lymph node metastasis, positive molecular findings metastasis in 1-3 axillary lymph nodes pN1 pN1mi micrometastasis (0.2 mm - 2.0 mm) pN1a 1-3 axillary lymph nodes pN1b internal mammary nodes (not clinically apparent), microscopic disease in sentinel lymph nodes pN1c metastasis in 1-3 axillary lymph nodes and in internal mammary nodes (pN1a+pN1b) pN2pN2a 4-9 lymph nodes present pN2b internal mammary nodes + (clinically apparent) pN3a > 1O lymph nodes metastasis or metastasis to infraclavicular lymph nodes pN3b metastasis in clinically apparent internal mammary lymph nodes + positive axillary lymph node pN3c metastasis in ipsilateral supraclavicular lymph node Instructions • From undergraduate point of view, TNM classification is important • What is given here is staging after an axillary block dissection. Hence, it is called pathological staging. If you know this, you may score higher marks.

Advantages of mammography • Noninvasive • Minimum hazards of radiation Disadvantages of mammography • False positive cases are around 5%. Hence, mammo­ graphic positive cases should undergo FNAC to confirm the diagnosis. • If a lesion is detecte d by mammogra phy, but is impalpable, the following special techniques will help. PEARLS OF

WISDOM

In young women, microcalcifications are the only mammographic abnormalities suggesting malignancy.

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Fig. 21.60: Architectural distortion

Fig. 21.61: Spiculation­ medio lateral view-right side

Bl-RADS scores range from O to 6 0: This score identifies a mammogram study that is still incomp­ lete. The X-ray may have been cloudy, making it difficult to read the images. This can happen, for example, if the patient moved at the precise moment the picture was taken. 1: This score means that mammogram is negative (that is, no evident signs of cancer were found). 2: This score also means that mammogram is normal, with no apparent cancer, but that other findings (such as cysts) are described in the report. 3: A score of 3 means that mammogram is probably normal but that there is an approximately 2 percent chance of cancer. Hence, follow-up with a repeat mammogram in six months. And if there is a family or personal history of breast cancer, the radiologist may opt to do more tests at this stage rather than wait. 4: This score means that the findings on the mammogram are suspicious and that there is an approximately 20 to 35 percent chance that a breast cancer is present. Core biopsy is a must in such cases for tissue sample. 5: This score means that the mammogram results are highly suspicious, with a 95 percent chance of breast cancer. 6: This means that patients have already been diagnosed with breast cancer and the pathologist has confirmed the diagnosis.

Technique of mammography A. Double dye technique of injecting contrast medium and patent blue.

B. A hooked wire is pushed down the needle left in the breast

tissue which acts as a guide to the surgeon-mammographic needle localisation and needle biopsy. For mammography findings see Key Box 21.14. C. Biopsy from nonpalpable lesions: First mammography is d one which may show suspicious areas such as microcalcification. Then ultrasound or stereotactic localisation is done, followed by FNAC. This is almost

Fig. 21.62: Spiculation­ craniocaudal view-right side

Fig. 21.63: Mammograph} showing a cystic lesion

lftl:1"1itWWIIW,� MAMMOGRAPHY

�

• Screening: Asymptomatic women of more than 40 years. • Diagnostic: Women with pain in the breast, mass, discharge, family history of breast cancer • 2 views-Mediolateral oblique view is for outer quadrant and axilla. Craniocaudal view is for medial quadrants. • Radiation dose is 0.1 centigray (cGy)-4 times that of chest X-ray dose but no side effects. • Benign lesions are round, punctate, popcorn like, etc. • Highly suspicious-pleomorphic, heterogeneous - Solid mass with irregular edges, spiculation - Long tentacles-tentaculation - Fine scattered calcification-microcalcification - Distortion of architectural pattern of the breast. - Asymmetrical thickening of breast tissues. I 00% accurate in the diagnosis of breast cancer (see under image guided biopsy). PEARLS OF

WISDOM

45% of breast cancers can be seen on mammography before they are palpable.

4. FNAC (Fine Needle Aspiration Cytology) is quick, safe and

easy method in which a cytological diagnosis can be made. The accuracy is more than 95% with an experienced cytologist. • False negative 15% (infiltrating lobular carcinoma, tubular carcinomas may mimic fibroadenoma) • False positive 1 % (at y pical ductal hypoplasia, papilloma, radiation changes) 5. Trucut biopsy: If FNAC is negative, a trucut biopsy or vacuum-assisted biopsy (VAB) using 11 gauge biopsy probe can be taken. The advantage of these biopsies is

Breast

preoperative assessment of hormone receptors can be done. False negative (25%) and no false positive (0%). 6. Incisional biopsy: Under local anaesthesia, a small incision is made over the lump and a biopsy is taken, which is sent for frozen section. lf frozen section report is malignant, an appropriate mastectomy is done. If the frozen section is negative, a lumpectomy is done and if report comes later as malignant, a mastectomy is done. 7. Image guided biopsy in indeterminate lesions a. Ultrasound guided biopsy: Microcalcifications are seen infrequently but if found, they can be biopsied using ultrasound as a guide. Success rate of positive histo­ pathology is high (Fig. 21.64) b. Wire localisation (Figs 21.65 to 21.67) • Indication: Localising nonpalpable breast lesions. • Procedure: Mammogram is done and the suspicious area is localised (microcalcification). A hooked wire enclosed within a needle is placed within the breast under guidance. The needle is removed. Patient is shifted to OT. In the OT incision and dissection follows the wire till 'Hook' is reached and tissue is excised and sent for histopathology.

Fig. 21.64: Ultrasound detecting a lump in the breast and a wire is being introduced within

403 c. Stereotactic biopsy: It is used to biopsy mammo­ graphically-suspicious lesions. Stereotactic biopsy techniques include-vacuum-assisted core biopsy. d. Mammotome: It is a hand held vacuum-assisted biopsy instrument which can be used under image (ultrasound or stereotactic) guidance. It gives accurate diagnosis of nonpalpable mammographic abnormalities. 8. Chest X-ray: Rule out pulmonary secondaries, effusion, or mediastinal widening (2 types) (Fgis 21.68 and 21.69) • Cannon ball type (haematogenous)-presents late • Lymphangiectatic type (lymphatic spread))-presents with intractable cough. 9. Abdominal ultrasonography is done to rule out secon­ daries in the liver, ascites, rectouterine deposits. • Incidence of liver metastasis in Ca breast is 6%. Routine USG abdomen is indicated only if hepatomegaly is present or any abnormality in LFT or in stage III or IV. 10. Bone scan: Incidence of bone metastasis (Fig. 21.70). • T l , T2-< 2%, no role for scan • T3, T4-25%. Even then, 60% of skeletal tissue should be demineralised before it is evident. Hence, bone scan is indicated if symptomatic, increased ALP or stage II and above.

Figs 21.65 to 21.67: Mammography directed wire localisation ( Courtesy: Dr Sampath Kumar, Professor of Surgery, Dr. Chandrakant Shetty, Professor of Radiology and Imaging, Dr Poornima, Resident, Dept of Surgery, KMC, Manipal)

..

Fig. 21.68: Cannon ball secondaries

Fig. 21.69: Carcinoma breast with metastasis in the clavicle

Fig. 21.70: Bone scan Ca breast

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11. Steroid hormone receptors-immunohistochemistry A. Intracellular steroid hormone receptor proteins, primarily ER (oestrogen receptors) and PR (progesterone receptors) have shown to be indicators of prognosis and a guide to hormone and endocrine therapy. About 50 to 85% of invasive breast cancers contain measurable amounts of ER. The concentration of ER increases with age with both having their highest levels in postmenopausal patients. • Normal value of ER is < IO fmol/mg proteins. It is consi­ dered positive> IO fmol/mg proteins. Upper levels as high as 1000 fmol/mg proteins may be there. • The presence of ER implies that normal cellular mechanism for processing oestrogen has been maintained despite the malignant change. Patients with ER positive tumours have longer disease-free survival after primary treatment, superior overall survival and longer survival after recur­ rence compared with patients with ER-negative tumours. • Before starting hormonal therapy steroid hormone receptors have to be checked and measured. Depending upon the levels, whether positive or negative, the hormonal therapy is given (details below). • All patients may benefit from tamoxifen except premenopausal ER/PR negative patients. Tamoxifen is started only after chemotherapy is completed. It should be given for 5 years. B. HER-2/neu receptor: It is a membrane tyrosine kinase receptor and a marker of cellular proliferation, expressed in up to 50% of cases. It is usually associated with ER negativity and high grade tumour, poor prognosis but there is a better response to adriamycin (Key Box 21.15).

[

............................. KEY BOX 21.15

HER-2 • Measured by immunohistochemistry • Test for gene amplification-fluorescent in situ hybridisation (FISH) is gold standard. • 25% of all breast cancers overexpress HER-2 • It is a transmembrane tyrosine kinase receptor • High grade tumours usually express this • ER negative-poor prognosis • Responds to 'TRASTUZUMAB'

Thus 'FISH' is done only when HER-2/neu is 2+

Interpretation of HER-2/neu result interpretation by IHC I + means negative 2 + means equivocal result-to confirm by 'FISH' 3 + positive or over expressed/amplified C. Detailed histopathological examination and followed b) immunohistochemistry (IHC) and DNA microarrays forrr an important part of treatment or the breast cancer. Th� new marker for cellular proliferation has been identifiec and named Ki 67 (Key Box 21.16).

.,,.,,_,,� • • • • •

� Ki67 Also known as MK167 It is a marker for cellular proliferation It depicts growth fraction of all proliferation Higher values suggest aggressive tumour A cut off value of< 14% is generally used to denote low and high values

lnvestigational Algorithm in a 40-year-old female with 5 cm lump in the outer upper quadrant I Clinical examination (suspicious of carcinoma)-no lymph nodes in axilla T2 NO MO

I

Routine investigations • Complete blood count: It may show anaemia • Chest X-ray may show cannon ball metastasis • Liver function testsalkaline phosphatase

Diagnostic tests • Mammography-suggestive of malignancy (Bl-RADS Grade IV)

I

I

Microcalcifications, spiculations

I

l

Fine needle aspiration cytology )

I

I No evidence of malignancy (clinically suspicious)l

I

[ TRUCUT or CORE biopsy

I

l

IHC-ER, PR, HER-2/neu, SBR grade, Ki67 index DNA microarrays

Fig. 21.71: Investigations in carcinoma breast

l

Metastatic workup • Chest X-ray • Ultrasound if hepatomegaly • Bone scan if ALP is increased or symptomatic

Breast

D. DNA microarrays: They compare DNA from nom1al cells to breast cancer cells. They are very expensive. Examples: 1. Oncotype Dx-it is 21 gene assay 2. Mammaprint-70 gene assay Based on these, treatment is divided into specific groups referred to as 'luminals'-this is the latest treatment and is given in page 422.

Pathological evaluation of carcinoma breast • Tumour, node, metastasis (TNM), oestrogen receptor (ER) • Progesterone receptor (PgR) • HER-2, Ki 67 index. • Tumour grade: Scarff-Bloom-Richardson (SBR). SBR grade Tumour grade: Depending upon three factors such as nuclear pleomorphism, tubule fotmation and mitotic rate, tumours are given scores and graded. Grade Ill tumours means they are poorly differentiated. Grading system is popularly called 'SBR' system-Scarff Bloom Richardson-Elston-Ellis modified 'SBR'grading system. Nuclear pleomorphism: Score I-small uniform nucleoli Score 2-intermediate nucleoli Score 3-large prominent nucleoli Mitotic count: Score 1-< 10% mitosis in 10 HPF Score 2-10%-20% mitosis Score 3-> 20% mitosis Tubule formation Score 1- > 75% cells in tubule form Score 2- > 10-75% cells in tubule form Score 3-< I0% cells in tubule form Grade Score I Favourable Unfavourable up to 3 3-5 Grade I Grade II 6-7 8-9 Grade III Examples Well-differentiated: Moderately differentiated: Poorly differentiated:

Grades Grades Grades

3 , 4, 5 6, 7 8-9

12. Role of ultrasonography in breast lump • It is second only to mammography. • Define cystic lesions • Demonstrate echogenic qualities of solid abnormality

405

• Well-circumscribed lesions are cysts with smooth margins (benign) • Benign lesions are round to oval-shaped with smooth contours. • Cancers have irregular walls. • It can guide FNAC, core biopsy, etc. • Painless, cost effective, can be reproducible but operator dependent. • Not ideal for lesions which are 1 cm or less in diameter. 13. Role of MRl 1 Particularly useful in detecting malignancy when mamrnographically subtle or occult (lobular carcinoma). • It can differentiate scar tissue from cancer. Hence can detect local recurrence after surgery. • MRI is better than mammogram in assessing the response of the tumour to neoadjuvant chemotherapy. It is also better investigation in dense breasts and m pregnancy. • Also for imaging of the breast with implants. Summary of investigations in Ca breast (Fig. 21. 71) PEARLS OF

WISDOM

When axillary nodes are positive for adenocarcinoma, but the primary is unknown, MRI is the ideal investigation to detect an impalpable breast lump. 14. Bone marrow aspiration in cases of unexplained cytopaenia or leukoerythroblastic blood smear. PEARLS OF

WISDOM

Thus 'TRIPLE ASSESSMENT' is the most important method for the diagnosis of carcinoma. It includes: • History and clinical examination • Imaging: Below 40 years-Ultrasound; Above 40 years-Mammogram • Fine needle aspiration cytology. Students are hereby instructed to follow the investigation which are available and routinely done in your hospital from the clinical point of view and select the investigation based on staging of the disease. TUMOUR BIOLOGY • Halsted did the first radical mastectomy for carcinoma breast in 1878. This was the gold standard operation for carcinoma breast for nearly I 00 years. Carcinoma breast is considered to be a systemic disease to begin with according to Fischer's theory if we consider the biology of tumour growth.

1 From practical point of view, the clinician should select the best investigation in each patient and not all the investigations given here.

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population and size of the tumour 1 cm. Also, the other modalities of treatment such as radiotherapy, chemotherapy, hormonal therapy are playing a major role together with surgery. Hence, the current approach is to do minimal local surgery and aggressive approach towards management of lymph nodes or metastasis. Stage wise carcinoma breast is divided into a. Early breast cancer-Stages I and IIA b. Locally advanced-Stages IIB and III c. Advanced/metastatic-Stage TV Surgical management is thus based on the stage of the disease. There is no confusion regarding management of either early breast cancer or metastatic breast cancer (MBC). Controversy exists in the management of LABC (III) which has both operable and inoperable diseases which is given in page 411. TREATMENT OF EARLY BREAST CANCER­ STAGES I, IIA A. Primary: Growth and Axilla • Locoregional control of primary disease is by surgery in the forrn of modified radical mastectomy (MRM) or breast conservative surgery (BCS). B. Adjuvant • Locoregional control of residual disease is by radiotherapy • Systemic control of disease is by hormonal therapy or chemotherapy.

Surgery (Table 21.8) 1. Wide local excision (lumpectomy) is indicated in tumour5 less than 4 cm in size and with well-differentiated histology. It includes removal of the tumour with a rim of at leas1 I cm of normal breast tissue. If the nodes are palpable and enlarged, this is combined with axillary block dissection, using separate incision. Currently, this procedure has become more popular. It is also called Breast Conservative Surgery (Key Box 21.17). Contraindications for BCS are given in Key Box 21.18.

M$11iPWllllllr,1 W SALIENT FEATURES OF BCS Tumour size-4 cm or less Should be able to get 1 cm negative margin Availability of frozen section is desirable Should not do undermining of the flaps as we do in MRM Absolute haemostasis No suction drain to be kept Direct approximation of the skin with no attempt to close the fatty tissue. • Decision about cosmetic acceptance to be done by patient specially to 4 cm

• • • • • • •

.,,..,_,,, • • • • • • • •

CONTRAINDICATIONS FOR LUMPECTOMV (BCS) Multicentric disease (rule out by mammogram) Pregnancy Central quadrant tumour (relative) Prior radiotherapy to the breast Prior chest irradiation Collagen vascular diseases Recurrence after BCS Skin fixation, involvement of nipple, chest wall

�

Different types of surgery for carcinoma breast Types of surgery

What does it mean?

Comments

• Local wide excision

• Tumour along with 1 cm of normal tissue is removed with an ellipse of the skin over lump • Quadrant containing the tumour is removed Entire breast tissue is removed. Both pectoralis minor and major are preserved

• Most popular breast conservative surgery done in Tl and T2 cases • Not done Frequently done-good retraction o f pectoralis major and minor are necessary for the axillary clearance • Mostly done and removal ofpectoralis minor helps the axillary dissection • Not necessary as carcinoma breast is considered as systemic disease today.

• Quadrantectomy Total mastectomy and axillary clearance • Patey mastectomy • Halsted radical mastectomy

• Breast tissue + pectoralis minor is removed and axillary block dissection • Entire breast tissue and both the pectoral muscles are removed.

407

Breast TEN COMMANDMENTS A few important tips about local wide excision­ lumpectomy (Figs 21.72 and 21.73) 1. Incision is made directly over the tumour 2. Pectoral fascia is usually not opened unless the tumour is adherent 3. Perfect haemostasis 4. Tumour removed with 1 cm margin all around 5. Ideally specimen mammogram and frozen section is done to look for clearance 6. Cavity should not be closed or obliterated by sutures as small seromas get absorbed 7. For better orientation of the tumour a long silk is tied on the lateral side. Remember a long lateral, short suture posteriorly. 8. Metal clips or coloured beads can be applied. 9. Drain should not be kept 10. Anterior and posterior margins are less important if a full thickness of breast tissue is removed.

Fig. 21.72: Local wide excision is done-if the skin is involved, it is also removed, undermining of the flaps is not required

Fig. 21.73: Local wide excision is in progress. 1 cm of normal breast tissue of the cancerous lesion is all that is necesary

PEARLS OF

WISDOM

Radiotherapy to the breast tissue is mandatory in all cases of breast conservation surgery. 2. Total mastectomy with axillary clearance (MRM) is equally good (good retraction of pectoralis minor facilitates axillary dissection-Auchincloss modification) 3. Patey mastectomy: This is the most acceptable and most widely practised surgery. [t is also called modified radical mastectomy. In this, the entire breast including nipple and areola (simple mastectomy) are removed with, pectoralis minor, followed by axillary block dissection. A complete axillary block dissection should include node clearance up to level III (Figs 21. 74 to 21. 79). • Level I Extends from axillary tail to the lateral border of pectoralis minor. Extends from lateral border of pectoralis • Level II minor to medial border of pectoralis minor. • Level III : Up to the apex of axilla. 4. QUART therapy by Veronesi: It includes Quadran­ tectomy (the entire segment of the breast containing tumour is removed), Axillary block dissection and Radiotherapy to the breast or axilla. • However, quadrantectomy, by removing large amount of breast tissue, gives rise to poorer cosmetic results. It seems unnecessary to remove entire segment of the breast, when in reality breast cannot be strictly divided into quadrants (unlike hepatectomy). Hence, it is not very popular. Advantages of Patey mastectomy over Halsted radical mastectomy • Cosmetically better accepted as axillary fold is maintained. • Function of the shoulder is better, and it gives a stronger and more useful arm. 5. Radical mastectomy: In this operation, following structures are removed • Entire breast including nipple and areola, skin overlying the tumour along with fat, fascia and lymphatics. • Axillary block dissection, including complete clearance of axillary fat and up to Level III nodes clearance. • Stemocostal portion ofpectoralis major, entire pectoralis minor, few fibres and aponeurosis of internal oblique, serratus anterior, latissimus dorsi and subscapularis. Three important structures should be preserved • Axillary vein • Bell's nerve (long thoracic nerve which supplies serratus anterior) • Cephalic vein. Disadvantages of radical mastectomy • Mutilating surgery • Poor cosmetic results • Lymphoedema of arm • High morbidity rate

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Fig 21.74: Classical MRM incision which includes nipple areola complex and slightly extending into axilla to facilitate axillary block dissection

Fig. 21.76: Tumour with degeneration

Fig 21.78: Same patient as shown in Fig. 21.77. Axillary block dissection is complete

Fig. 21.75: Patey mastectomy specimen: In this operation, entire breast including axillary tail with all the axillary group of lympr nodes and pectoralis minor are removed

Fig. 21. 77: You can see the axillary vein and thoracodorsal nerve as the axillary dissection is in progress

Fig 21.79: Initially local wide excision was planned in this patient. Mammogram revealed multicentricity. Hence, MRM was done

Breast

Please note: In our country, modified radical mastectomy is more frequently done. However, an early carcinoma is being treated more and more by breast-conserving surgery nowadays. Radical mastectomy is obsolete now. Management of axilla • In early breast cancer with no clinically apparent nodes and if the disease is not multicentric, then sentinel lymph node (SLN) biopsy can be considered. Otherwise, axillary dissection is recommended. Following surgery adjuvant treatment should be instituted in all tumours > 1 cm. Presence of metastatic disease within axillary lymph nodes remains the best single marker for prognosis. Sentinel node biopsy • Senital means guard. Senital node is the first lymph node to get enlarged in malignancies. • What does it mean? If no node is detected in this procedure in the axilla, axillary block dissection is not required. This will be of great significance in early breast carcinomas wherein lymph nodes are not clinically palpable nor detected by investigations such as ultrasound/CT scan of the axilla. • Indication: Early breast cancer with (Tl or T2 No) clinically node negative axilla. Contraindications 1. Palpable lymph node 2. Prior axillary sampling 3. Post-chemotherapy, post radiotherapy 4. Multifocal breast carcinoma 5. Allergy to blue dye Localisation (procedure) Peroperative is better and followed more commonly. Patent blue (Isosulfan vital blue dye 2.5-7.5 ml) or 99mTc radioisotope labelled albumin is used. Where to inject • Near the tumour: Peritumour area • Into subdermal plexus around nipple. Detection • By visually blue staining after making an incision • Hand held gamma camera. The node is biopsied and sent for frozen section biopsy or imprint cytology. Where exactly to detect the sentinel lymph node (SLN)? • A 2-4 cm incision is made in between the pectoralis major and lattissimus dorsi, after 5-7 minutes after the injection. • Blue stained lymphatics are identified. • 2-3 lymph nodes have to be removed. Interpretation • If sentinel lymph node (SLN) is negative for malignant cells, no axillary block dissection is required.

409 • If SLN is positive for malignant cells, axillary block dissection is done. Detection rates • Blue dye: 90% • Radioisotope: > 95%. Complications of MAM 1. Seroma//ymph collection: (30 to 50%). In spite of adequate drainage of the chest wall and axilla, drainage occurs for about 5-10 days. Just patiently reassure the patient. 2. Secondary infection: It manifests as redness, discharge, fever, etc. Appropriate antibiotics are necessary. 3. Flap necrosis: True mastectomy requires elevation of both upper and lower flaps (almost skin thin). Thus it predisposes to flap necrosis. This requires debridement, antibiotics, suturing and rarely skin grafting also. 4. Haemorrhage: Not common. If infection is severe especially in the axillary tissue, it is possible that the blood vessels may get eroded and bleeding can occur. This usually stops with pressure but may require ligation of bleeders in the operating theatre. 5. Pain and numbness in the axil/a, medial side of arm. It is usually due to irritation of intercostobrachial nerve. Generally subsides by a few days of time. They require simple analgesics. 6. Shoulder dysfunction can occur especially when pectoral muscles are injured or retracted resulting in haematoma or when pectoralis muscles are removed. It improves over a period of time. Incidence is about 8-10%. 7. Injury/thrombosis of axillary veins. It manifests as severe pain in the hand and swelling. Treated by low molecular weight heparin. 8. Injury to axillary vein, needs to be repaired by 5 or 6-0 prolene sutures. 9. Winging scapula is due to injury to long thoracic nerve of Bell. The good anatomical knowledge is essential to prevent this complication. 10. Lymphoedema of the arm appears a few months later. Adjuvant treatment-2 types A. Radiotherapy (Key Box 21.19} • Following either MRM or BCS, the residual disease is controlled by administering radiotherapy in a dose of 50 gray in 25 fractions. • In the absence of nodal involvement axilla may be spared radiation. • If 4 or more nodes are involved, then the field of radiation should include supraclavicular and internal mammary chains. • Boosts to lumpectomy causes are given by adding 10-16 grays

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111$11•,_,,, INDICATIONS FOR POSTOPERATIVE RADIOTHERAPY

• • • • • • •

·�

Tumour margin is positive Pectoralis major is involved Inner quadrant tumour High grade tumours Axillary clearance not satisfactory Breast conservative surgery Tumour size more than 5 cm, 4 or more nodes are positive

Brachytherapy in breast cancer (Figs 21.80 and 21.81) 1. It refers to use of radiation sources in or close to the tumour. 2. Breast is one of the organs which can be treated with brachytherapy. • Details about brachytherapy are given in Chapter 47. B. Systemic therapy-2 types I. Adjuvant hormonal therapy Hormonal therapy is administered to patient in whom the tumour expressed steroid hormone receptors, i.e. ER/ PR.Those who are negative for ER/PR, will not benefit from these drugs. The drugs are categorised as follows • 1st line-anti-oestrogens-tamoxifen.Commonly, tamoxifen in a dose of 20 mg is used for 5 years, to be started only after completion of chemotherapy. It is given in premenopausal patients. • Raloxifene is an oral selective oestrogen receptor modulator (SERM) that has oestrogenic actions on bone and anti­ oestrogen actions on the uterus and breast. It is used in the prevention of osteoporosis in postmenopausal women. Studies have proved that no specific advantage of raloxifene in the adjuvant treatment of breast cancer rather than established drugs such as tamoxifen.

Fig. 21.80: Brachytherapy

• 2nd line-Aromatase inhibitors. They prevent synthesi of endogenous oestrogens/steroids by blocking th, aromatase enzyme which converts androstenedione l< oestradiol in the adrenals. They are: 1st generation­ aminoglutethimide and 2nd generation-anastrozole letrozole, exemestane given to postmenopausal patients. • Letrozole is costlier than tamoxifen, reduces oestroge1 levels by 98%, and thus slows down oestrogen sensitiv, breast cancers. It is given in a dose of 2.5 mg per day. Side effects include vaginal bleeding, vaginal dryness, nigh sweats, hot flushes, osteoporosis, etc. (Key Box 21.20). • 3rd line-progestogens-megestrol acetate 400 mg per da) can be given • 4th line-androgens such as fluoxymesterone in the dos{ of30 mg daily is another drug (last 2 drngs are rarely given) II. Adjuvant chemotherapy Should be considered in all cases of early breast cance1 irrespective of menopausal status, hormone receptor statui and nodal status.

MHte...........---; AROMATASE INHIBITORS

• Aromatase is an enzyme which synthesises oestrogen • It converts androgens into oestrogen by process of aromatisation • Aromatase inhibitors inhibit this process • T hus in premenopausal women-it can be used to decrease production of oestrogen by ovary. However, tamoxifen is better • In postmenopausal woman-oestrogen is produced in peripheral tissues such as fat, liver and muscle • Hence, drug of choice in postmenopausal patients is an aromatase inhibitor • Anastrazole, letrozole, exemextane are the drugs • Cardiac problems, osteoporosis are side-effects.

Fig. 21.81: Brachytherapy is being given

Breast

Classification of drugs 1st line agents: Cyclophosphamide, adriamycin, 5-fluorouracil (FAC). Because of cardiotoxicity of adriamycin, epirubicin is preferred as in FEC regimen. The preferred regime is anthracyclines (adriamycin) which have a better response rate. Either CAF or FEC every 21 days x 6 cycles or at every 21 days x 4 cycles or just adriamycin with cyclophosphamide (AC). Howeve,� the CMF (cyclophosphamide,mitomycin, fluorouracil) regimen still continues to be used widely due to economical reasons. 2nd line agents: Taxanes-paclitaxel and docetaxel 3rd line: Gemcitabine Indications for adjuvant chemotherapy • Tumour > 1 cm OR Tumour< 1 cm with ER-ve, HER-2 +ve, high grade. Adjuvant trastuzumab therapy Trastuzumab: A monoclonal antibody against tyrosine kinase receptor (HER-2 receptor) is administered in patients with HER-2 +ve patients since it has been shown to improve disease free survival (DFS) by 50%, when it is combined with taxane­ based chemotherapy. It potentiates effects of chemotherapy. Dose: Loading 4 mg/kg. Maintenance 2 mg/kg/week for 9 weeks. Treatment of early carcinoma of the breast is summarised in Fig. 21.82.

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LOCALLV ADVANCED BREAST CANCER (LABC) Many patients with breast carcinoma come to the hospital with gross skin involvement, pectoral muscles involvement or chest wall involvement. Even though the cure rates are low, with proper planning, good control of the disease, good palliation in majority of patients and 'cure' in a small group can be achieved. They do not have metastasis. Hence, grouped under locally advanced breast carcinoma (T3 and T4). With neoadjuvant chemotherapy (NACT), survival rates have improved (Figs 21.83 and 21.84). Aim • Good locoregional control Attempt at 'cure' by chemotherapy. Salient features of LABC (Figs 21.83 to 21.87) • Any tumour more than 5 cm with or without skin and chest wall involvement are included under LABC. • Under TNM, any stage IIB and stage III can be included under LABC. Isolated supraclavicular metastasis is also included in the stage III/LABC category. • Mastitis carcinomatosa (without metastasis) is also included. Types A. Operable LOBC (large operable breast cancer) (IIB and III A): Ulceration, limited skin oedema, fixation to the pectoralis muscles and bulky axillary nodes are grave signs but resection can be done.

Locally advanced breast cancer Mammogram + trucut biopsy + bone scan + chest X-ray (if necessary, CT chest and abdomen) Receptors positive

LABC-inoperable Neoaduvant chemotherapy (NAG) Complete clinical response (cCR)

Chemotherapy + radiotherapy

MRM (or local wide excision) �--------- Trastuzumab for one year

Microscopic disease Partial pathologic response (pPR)

No microscopic disease Complete pathologic response (cPR)

Fig. 21.82: Treatment of early carcinoma of the breast

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Fig. 21.83: Carcinoma of the breast-See the puckering of the skin, nipple retraction-Core biopsy or incision biopsy also can be taken here. This case is not ideal for resection. Hence, neoadjuvant chemotherapy should be given first (T4 N1 MO)

Fig. 21.86: LABC-tumour was fixed to skin with peau d'orange tumour was 8 cm in size ( Courtesy: Dr Basavaraj Patil, Associate Professor, Dept of Surgery, KMC, Manipal)

Fig. 21.84: Large lump displacing the nipple areola complex downwards-core biopsy for receptor studies. MRM may not give negative margin. Hence, this is an ideal case for neoadjuvant chemotherapy followed by surgery (T4 N1 MO) Fig. 21.87: LABC ( Courtesy: Dr Satish Deshmukh and Dr Murtaza Akhthar, NKP Salve Institute of Medical Sciences, Nagpur)

B. Inoperable (IIIB): Extensive breast skin oedema, intercostal nodes, arm oedema, or inflammatory breast cancer. Investigations • Tissue diagnosis is established by core biopsy. • Core biopsy tissue is also evaluated for ER, PR, HER 2/neu status, P53 overexpression, etc. • Biological markers important because if positive, NACT is offered in the first place. Those who respond to NACT, are subjected to mastectomy or BCS. Fig. 21.85: LABC-Tumour was fixed to chest wall with fungating ulcer and oedema arm. ( Courtesy: Dr Ankur Sharma, Assistant Professor, Dept. of Surgery, KMC, Manipal)

NEOADJUVANT THERAPY IN BREAST CANCER This modality is used for treated locally advanced breast cancer (LABC) wherein due to the advanced nature of the disease in

Breast

the breast and/or axilia, the surgeon is unable to operate upon the patient initially. The exact TNM stage for defining LABC remains controversial (II B versus Ill A). However, any breast cancer in which the operating surgeon feels that he will be unable to completely resect the tumour in the breast and/or axilla can be treated with neoadjuvant chemotherapies. If the growth becomes impalpable clinically after finishing ALL rounds of chemotherapy, it is called a Complete Clinical Response (cCR). However, if the resected specimen shows viable microscopic disease in a patient with cCR, it is termed Partial Pathological Response (pPR). If no microscopic growth is seen in resected specimen, then it is termed Complete Pathological Response (cPR). The various modalities available are (Key Box 21.21) 1. Neoadjuvant chemotherapy • Patient should have a good general condition to receive chemotherapy. • Full course of chemotherapy should be completed whether or not the growth has completely resolved, e.g. 4 cycles of AC followed by 4 cycles of paclitaxel followed by surgery. • Patients who have received full course of neo-adjuvant chemotherapy before surgery need not receive any more chemotherapy in the adjuvant setting. • However, patients who did not receive full course of neoadjuvant chemotherapy before surgery should finish their remaining cycles after surgery. • Anti-HER2 (tratuzumab) can be given alongwith in the neoadjuvant setting till all courses of chemotherapies are over and should be continued in the adjuvant setting for a total duration of one year. 2. Neoadjuvant hormonal therapy • Postrnenopausal patients who are not fit to receive systemic cytotoxic therapies should undergo a trial of this modality. • A prior assessment of the ER/PR status should be done to make sure that the tumour is hormone responsive. This modality does not work in honnone negative tumours. • Tamoxifen/letrozole/anastrozole all can be used.

N¥M.-wr,1 • • • • • •

SUMMARY OF LABC � Trucut or open biopsy is done to confirm the diagnosis followed by identification of receptor status. Neoadjuvant chemotherapy is given first which will shrink the tumour. Surgical resection is carried out to achieve a clear margin (mastectomy or breast conservation surgery). Radiotherapy is given to chest wall and supraclavicular area. Additional chemotherapy can also be given (Adjuvant). If the tumour is fungating and the surgeon feels adequate cancer clear margin can be achieved, toilet mastectomy can be done first followed by chemoradiotherapy.

413

• Duration of the treatment should be up to the achievement of maximal response. 3. Neoadjuvant radiotherapy • Patients not responding to the above mentioned treatment may be given a trial of this modality. • Neoadjuvant therapy apart from making inoperable tumours operable also act as prognostic marker as in patients who achieve cPR have a far better outcome of the disease that those who do not. • Even in LABC, if the response to treatment is favourable breast conservation is possible, even in T4 tumours. PEARLS

OF

WISDOM

• ER/PR negative responds better with NACT. • ER/PR positive has the option of NAHT (Neoadjuvant hormonal therapy). • HER-2/neu positive responds to Trastuzumab with taxanes, and response is better (treatment is costly)

Advantages of neoadjuvant chemotherapy • Downstages the disease • Increases chances of breast conservation. • Inoperable tumours may become operable. • Systemic treatment (chemotherpy) starts early • Assess response in vivo. • Inhibits a potential postsurgical growth spurt. • Chemotherapy is delivered through an intact vasculature. CT Regimens • Anthracycline (doxorubicin, epirubicin) + cyclopho­ sphamide (AC/EC) with or without addition of 5 fluorouracil is found to be superior to CMF regimen. • Addition of taxanes has been beneficial. • One example of sequential AC + Taxane (HER-2/neu negative case is given below: Doxorubicin 60 mg/m2-IV, and Cyclophosphamide 600 mg/m2 IV day 1, cycled every 21 days for 4 cycles followed by Paclitaxel 175 mg/m2 by 3 hour IV injection day/cycled every 21 days for 4 cycles. Radiation therapy after LABC RT is given to the chest wall in case of: 1. Tumour size > 5 cm before neoadjuvant therapy 2. Positive margins after mastectomy/BCT 3. More than 4 axillary LN +ve if axillary dissection done 4. Lymphovascular invasion 5. ER-negative 6. High grade tumour. PEARLS OF

WISDOM

Any recurrence following lumpectomy-mastectomy has to be done.

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Role of "toilet" total mastectomy • Controversial: It is not mentioned in most current literature. • Fungating and offensive smelling-for local control to improve quailty of life and alleviate social and psycho­ logical factors in patients with poor physiological reserve. • 30- 40% local recurrence. • Increased incidence of distant metastasis without systemic therapy. • Increased rates of wound dehiscience, wound infection, seromas-when associated with preoperative radiotherapy Treatment of metastatic carcinoma of the breast • Breast cancer is a systemic disease at the onset. Even at early stages, patient may harbour micrometastasis at some sites, which may remain dormant for years together or may get treated with adjuvant therapy. Metastatic breast cancer (MBC) may be seen in almost all organs but some organ involvement is seen more frequently than others. Soft tissue recurrence, bone, liver and lung metastasis is seen more commonly. Metastasis to brain, pleura and pericardium, altogether less common, may have devastating consequences and require specific treatment. • Aim of the treatment is to give palliation in the form of treatment of symptoms. • As these patients have advanced disease/disseminated disease, chemotherapy is given first followed by excision of lump/simple mastectomy to get rid offungating tumour. However, if the lump disappears totally, there is no indication for excision of the lump or mastectomy. • Following chemotherapy, tamoxifen is given for a period of five years. Repeated courses of second and third line chemotherapeutic drugs may have to be given. • The treatment of individual organ involvement or system involvement is given below. Patients with oestrogen receptor +ve status are more likely to have a bony recurrence, whereas ER -ve tumours tend to recur in liver and brain. After diagnosis of MBC, patients live an average of 16-30 months.

Treatment options in MBC 1. Hormonal treatment 2. Chemotherapy 3. Trastuzumab 4. Supportive treatment. Hormonal treatment In general, hormone treatment is better tolerated as it is associated with fewer side effects. Hence, it is preferred in patients who have steroid receptor positive status and/or asymptomatic visceral metastasis.

Chemotherapy • Preferred in patients with steroid receptor negative diseast and in symptomatic visceral metastasis. • Both single and combination chemotherapy regimen car be used. Trastuzumab • Patients who have overexpression ofHER-2 receptor, ma) also receive trastuzumab in addition to the above. Supportive treatment-3 organs 1. Malignant pleural effusion • Once effusion is confirmed by aspiration and cytology, an intercostal drain is left in place for a few days. Once the drainage is nil or completely dry, talc insufflation is done, to achieve pleurodesis. Talc is the most effective agent, followed by tetracycline or bleomycin, etc. In selected cases, effective pleurodesis has given an asymptomatic period up to 1-2 years. 2. Cerebral metastasis • Patients present with features of raised intracranial pres­ sure such as headache, nausea, vomiting and papilloedema. • Treatment includes corticosteroids and cranial radio­ therapy. However, treatment is distressing. 3. Bone metastasis • Eventually 60-70% of patients with carcinoma breast develop bone metastasis. Bone metastasis produces intractable pain, pathological fractures, quadriplegia and paraplegia, etc. (Figs 21.88 and 21.89 and Key Box 21.22). • Localised bone lesions are treated by palliative radiotherapy or decompression as in quadriplegia, etc. • Bisphosphonates, e.g. oral clodronates have been found to arrest progression of bone disease, given at the dose of 1,600 mg/day (Key Box 21.23). Causes of death • Malignant pleural effusion • Bony metastasis • Cerebral metastasis • Cancer cachexia. Management of metastatic breast cancer is summarised in Fig. 21.90.

.,...........

�

BONE METASTASIS

• • • •

Pain-due to stretching of periosteum Pathological fractures Paralysis Perish-due to hypercalcaemic crisis

Breast

415 Metastatic Breast Cancer

ER or PR +ve

ER or PR-ve

Hormonal treatment

Chemotherapy

No response

Fig. 21.88: Pathological frac­ ture humerus-treated by internal fixation followed by radiotherapy

MMI..........,.� • • • • • • •

BISPHOSPHONATES Important drugs in addition to radiotherapy to treat bone metastasis in malignancies. Used to treat hypercalcaemia. Inhibits osteoclast formation Decreases bony resorption and induces apoptosis of osteoclasts Decreases bony pain, increases mobility Helps in recalcification of lytic bone metastasis IV-pamidronate or zoledronic acid 4 mg IV monthly for 1 O courses are used

Fig. 21.89: Whole body bone scan showing disseminated bony metastasis-99mTc-labelled phosphate compounds are agents of choice for detection of osseous metastasis

See Fig. 21.90 for flow chart of management of metastatic breast cancer

Response present HER-2 neu +ve Trastuzumab

Fig. 21.90: Management of metastatic breast cancer

EFFECT OF LYMPHATIC OBSTRUCTION FROM CARCINOMA OF BREAST

1. Peau d'orange: It occurs due to lyrnphoedema due to sub­ areolar lymphatic obstruction and fixation of hair follicle to the underlying malignancy. 2. Oedema of the arm is also called elephantiasis chirurgens (Figs 21.91 and 21.92). • It is a complication of radical mastectomy when all the nodes in the axilla are removed, especially when postoperative radiotherapy is given in the axilla. • It occurs due to destruction of all lymphatics and lymph nodes. • It does not pit on pressure. Some amount of infection also plays a role. Treatment • Difficult. Elastic bandage, exercise, massage and antibiotics may help. 3. Brawny oedema of the arm • This occurs in inoperable carcinoma of breast with fixed nodes in the axilla. Arm is oedematous and does not pit on pressure. • It is brawny, indurated, painful. • It is treated like above. 4. Cancer-en-cuirasse (Figs 21.93 to 21.95) • In this, the entire chest wall is studded with cancerous nodules which are hard and fixed to the skin and to the chest wall. The condition has been compared to an armour used by the soldier. It indicates advanced carcinoma of breast. Palliative treatment is given in the form of radiotherapy or chemotherapy and analgesics to relieve the pain. 5. Lymphangiosarcoma: It is a rare complication after mastectomy and arises from lymphoedematous limb. It is treated by forequarter amputation.

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Fig. 21.91: Lymph oedema right upper limb-troublesome

c omplication after axillary blo ck dissection-more after radiotherapy to axilla

Fig. 21.94: Extensive lymphoedema and lymphangitis due to car­ cinoma axillary tail of the breast

Fig. 21.92: Po stmastectomy lymph oedema ( Courtesy: Dr Sreejayan, Professor of Surgery, Calicut Medical College, Calicut)

Fig. 21.95: Cancer-en-cuirasse

BREAST RECONSTRUCTION

• The ideal candidate for breast reconstruction is a patient who has undergone modified radical mastectomy. Fig. 21.93: Cancer-en-cuirasse-Chest wall studded with

cancerous nodules-very painful bleeding lesion

Mastectomy results info/lowing changes in a woman such as: • Psychological stress • Mood disturbances and anxiety

417

Breast

• Increased consciousness about clothes • Decreased sexual interest and satisfaction • Negative body image Reconstruction (Key Box 21.24) • Improves self confidence • Better social life • Decreases concern about cancer • Better sexual life • Feel "whole again" Timing: Immediate or delayed • Immediate reconstruction: Should be done if no contraindications. It has proven psychological benefits and patient satisfaction, it is cost effective, it does not delay adjuvant treatment and recurrence detection. Reconstruction options 1. Pedicled flaps • Latissimus dorsi myocutaneous flap (with implant) • TRAM flap 2. Free flaps • TRAM flap

IIMl!NIIIIIIIMIIIIII� COMPONENTS OF BREAST RECONSTRUCTION • Chest wall reconstruction: This can be achieved by using latissimus dorsi musculocutaneous flap or contralateral transversus abdominis muscle (TRAM) flap. • Creation of a mould: A silicone gel implant is inserted under pectoralis major muscle (subpectoral pocket). • Reconstruction of nipple and areola: This can be achieved by skin taken from inner thigh, labia minora or from opposite breast (nipple sharing). However, this reconstruction is done 4-6 weeks later, once the implant settles down. • Symmetry with the opposite breast: It is achieved by doing reduction mammoplasty of the other breast.

• Gluteus maximus myocutaneous flap • Anterolateral thigh flaps 3. Silicon compound gel/saline • Expandable • Adjustable • Lesser risk of complications

BREAST RECONSTRUCTION WITH TRAM FLAP (Figs 21.96 to 21.101)

Fig. 21.96: Mastectomy site is marked

Fig. 21.97: TRAM flap marking is done

Fig. 21.98: TRAM flap is raised-flap design: Zone I-IV

Fig. 21.99: TRAM flap is shown with its blood supply

Manipal Manual of Surgery

418

Fig. 21.100: Mastectomy bed-pectoralis muscle fibres

Fig. 21.101: Healing after 20 days

(Courtesy: Dr Bhaskar KG, Senior Consultant, Plastic Surgeon, Medical Trust Hospital, Cochin, Kerala (next page photos also)

Breast reconstruction with TRAM flap Transversely oriented rectus abdominis myocutaneous flap popularly known as TRAM flap is the most common re­ constructive option in postmastectomy breast recon­ struction. This flap can be pedicled based on the superior pedicle or based on inferior pedicle. It can be used for breast reconstruction as a free flap using microvascular technique.

Superior epigastric artery is the artery supplying the flap. If it is used as a pedicled flap for breast reconstruction, the contralateral side is used for reconstruction. If TRAM flap is taken with inferior pedicle using microvascular technique, deep inferior epigastric artery forms the main vascular basis of the flap.

BREAST RECONSTRUCTION WITH LD FLAP (Figs 21.102 to 21.104)

Fig. 21.102: Latissimus dorsi (LD) flap is marked

Fig. 21.103: LD flap is raised

Fig. 21.104: LD flap is brought to the mastectomy site

Breast

419

BREAST RECONSTRUCTION WITH LD FLAP WITH SILICON IMPLANT (Figs 21.105 to 21.108)

Fig. 21.105: Silicone implant

Fig. 21.106: Silicone implant placed

Fig. 21.107: Silicone implant placed in the subpectoral pocket

Fig. 21.108: Wound closed after implant

Latissimus dorsi musculocutaneous flap Indications: Breast or skin reconstruction, post mastectomy or wide excision of large chest wall Contraindications: 1. Previous lateral thoracotomy 2. Very large breast in patient who does not desire reduction 3. Planned postoperative radiation therapy Positioning: • Lateral decubitus position • Marked before mastectomy Vasculature!Blood supply: Thoracodorsal pedicle Procedure: Skin island of about 5-6 cm wide, outlined transversely. Divide the paraspinal origins of the muscle preserving the pedicle. Rotated on its humeral insertion towards the anterior chest wall beneath a bridge of thoracic skin below the axilla. Breast implant can be placed in a submuscular pocket.

MALE BREAST CARCINOMA

Advantages: 1. Proximity to the breast 2. Reliable/robust circulation (rich blood supply) Disadvantages: 1. Large scar on the back. 2. Cannot be used if there is an injury to thoracodorsal pedicle.

Introduction Constitutes less than 1% of all cancer in men. Minimum age is around 65 years Probable aetiological factors 1. Radiation exposure 2. Oestrogen therapy 3. Gynaecomastia 4. Klinefelter's syndrome XXY 5. Testicular feminising syndrome 6. Obesity 7. Increases incidence in prostate cancer patients 8. Bantumen Pathogenesis Male breast cancers tend to be more advanced at the time of presentation because of less subcutaneous fat. More than 90% cases are infiltrating duct carcinoma, about 10% are ductal carcinoma in situ (DCIS).

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Fig. 21.109: Male breast carcinoma in pre-existing gynaecomastia. You can see destruction of the nipple and infiltration into the skin: Commonly it is infiltrating ductal carcinoma

Fig. 21.110: Male breast carcinoma-tends to ulcerate and infil· trate chest wall early ( Courtesy: Dr Surya Prakash Saxena, Dis­ trict Surgeon, Durg, Chhattisgarh)

Clinical features (Figs 21.109 and 21.110) • Lump in the breast which is hard and irregular. • Nipple retraction • Early skin involvement • Metastatic axillary nodes.

• BRCA mutations: Highest incidence in Ashkenazi Jews • High risk of ovarian cancer • High risk of contralateral breast cancer • Multiple ipsilateral primaries • Adenocarcinoma of invasive type • High grade • BRCA 1-ER, PR, HER-2-neu negative • High risk for a recurrence.

Differential Diagnosis • Chest wall sarcoma • Bony lesions other bony metastasis, tuberculosis • Gynaecomastia. Investigations • FNAC/Trucut biopsy • Chest X-ray. Treatment • MRM with radiation to the chest wall • 80% of these cases are ER positive, hence role for tamoxifen • Goserelin 3.6 mg/28 days for 2 years-it is a LHRH agonist. It causes reversible chemical castration. MISCELLANOUS PROPHYLACTIC MASTECTOMY It is also called 'risk reduction mastectomy'. Counselling before risk reduction mastectomy • Loss of breast is irreversible • Sexual and psychological function • Reconstruction has its complications • Does not guarantee 100% safety against cancer. Cancer risks in BRCA mutation carriers • BRCA I: 65 to 85% (long arm of chromosome I7) • BRCA 2: 40-85% (long arm of chromosome 13)

Aim • To decrease the risk of developing invasive cancer • Also to decrease the risk of dying. Risk assessment 2 specific genes-BRCA I andBRCA 2 are responsible for 5% of all breast cancers. Why prophylactic mastectomy • BRCA I associated cancers are invasive, high grade, ER, PR and HER-2/neu negative. Hence, more aggressive and carry poor prognosis. Other risk reduction strategies • Lifestyle modification: Exercises, decrease alcohol intake, avoid HRT. • Early detection by annual mammogram • Tamoxifen as a primary chemoprevention drug. Types of risk reduction mastectomy • Total mastectomy • Subcutaneous mastectomy. Indications (Pennisi, 1986) I. Biopsy proved • Noninvasive intraductal carcinoma in situ • A single focus of lobular carcinoma in situ • Florid cystic disease 2. A mammogram that is suspicious of moderate to severe marnma1y dysplasia

Breast

421

3. Persistant breast nodules that do not vary with menstrual cycles and are of concern to the patient and physician 4. Familial or hereditary breast carcinoma. Procedure • Submammary incision is given. • Breast tissue is dissected from the pectoral muscles. • Skin flap along with even thickness of subcutaneous fat which is retained under the skin, is raised (helps in retaining vascularity of the flap). Haemostasis is obtained. Immediate reconstruction or reconstruction at a later date can be done. • Skin sparing mastectomy • Remove entire breast including axillary tail. Flaps should be thin. Axillary block dissection is not necessary. Results • Prophyhlactic mastectomy reduces incidence of breast cancer by 90%. MONDOR'S DISEASE Mondor's disease is spontaneous thrombophlebitis of the superficial veins of the breast and anterior chest wall. The common causes of thrombophlebitis such as injury or infection are not found in these cases. Signs 1. Indurated subcutaneous thrombophlebitic cord about 3 mm diameter of varying length is situated in the subcutaneous plane with an attachment to the skin. Consistency is like that of vas deferens. 2. W hen the skin over the breast is stretched by raising the arm, a narrow, shallow subcutaneous groove along the side of the cord becomes apparent. Treatment • Restricted arm movements (otherwise it is very painful). Spontaneous recovery is expected within a few days. ANGIOSARCOMA OF THE BREAST • • • •

They are uncommon malignant tumours of the breast. Aetiology is not clear Breast is one of the sites of angiosarcoma. Clinically it presents as rapidly growing lump in the breast with infiltration of the skin producing ulceration. • On careful observation, the edges are not everted and the lesion is very vascular. • It is difficult to make a clinical diagnosis of angiosarcoma of the breast (Fig. 21.111). However, with careful examina­ tion it is possible to suspect because of increased vascularity of the tumour and the features mentioned above. • Confirmation of the diagnosis is by FNAC or by biopsy from the lump. Chest X-ray and CT scan of the chest are other helpful investigations.

Fig. 21.111 : Angiosarcoma of the left breast

• Treated by course of chemotherapy followed later by mastectomy. DISORDERS OF AUGMENTED BREAST • This is called augmentation mammoplasty • Implant used is outer silicone shell filled with silicon gel, saline or a combiantion of both • Site of implant A. Subpectoral: More often this is the side. Even if a cancer develops in this breast, mammography can detect early lesion. After mastectomy-if recurrence develops, easy to detect if it is subpectoral B. Subcutaneous: Not many advantages: Easy to keep and easy to remove. It interferes with detection of malignancy. Easy to rupture. • Complications I. Implant rupture: 5 to 10% 2. Bleed RARE BREAST CANCERS 1. Squamous cell (epidermoid) carcinoma • Rare cancer from metaplasia within duct system and devoid of distinctive clinical/radiological characteristics. • Regional metastasis occurs in 25% of patients • Distant metastasis is rare 2. Adenoid cystic carcinoma • Rare, less than 0.1%. • Well circumscribed and usually 1-3 mm in diameter. • Axillary lymph node metastasis are rare • Death usually occurs from pulmonary metastasis. 3. Apocrine carcinoma • Well circumscribed cancers have rounded vesicular nuclei and prominent nucleoli. • Usually aggressive.

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Manipal Manual of Surgery

4. Sarcomas • Large painless breast mass with rapid growth • Diagnosis: Core/open biopsy. • Sarcomas graded on-eccentricity, degree of differentia­ tion, nuclear atypia, mitotic activity • Axilla is addressed only if palpable lymph nodes are present • Usually in patients with history of adjuvant RT. 5. Lymphomas: 2 variants (good prognosis) a. Women < 39 years-bilateral, associated with Burkitt's lymphoma. Axillary dissection is done for staging and clearance of the disease. Recurrence is treated with CT/ RT b. Women > 40 years-B cell type. Rarely Hodgkin's lymphoma AN EXAMPLE OF A CASE OF CARCINOMA BREAST A 59-year-old post-menopausal lady complains of lump in the right breastof3 months duration. Lump was hard in consistency. Sonomamrnogram revealed ill-defined hypoechoic lesion with spiculations seen in the superolateral quadrant of the breast. F NAC was suggestive of malignancy. Whole body bone scan after 3 hours IV inj. of 20 mCi of Tc-99 m MDP showed few osteoblastic lesions-so degenerative changes? Treatment: She underwent local wide excision followed by axillary block dissection. Final report was tubular carcinoma30% arising in a background of proliferative breast disease (70%). All margins were free. Lymph nodes were free from metastasis Immunohistochemistry: Tumour cells are ER positive PR positive Quick score 2. Her-2/neu 1+ Ki 67: 12%-Hormone responsive Next plan: External beam radiotherapy of 60 Gy in 30 fractions over 6 weeks, including boost to the lumpectomy side. • She also received tablet letrozole 2.5 mg HS and 6 monthly Zoledronate - Zoledic acid Based on DNA microarrays, breast cancer can be divided into specific groups referred to as luminals depending on which the choice of the treatment can be given. However, since these tests can be very expensive, IHC results (ER/PR/HER/Ki 67) can be used as surrogate markers to define various categories of breast cancer. RECENTADVANCES IN THE MANAGEMENT OF CARCINOMA BREAST (Table 21.9) 1. LuminalA Tumour characteristics should include all of the following features: ER and PR positive

HER-2 negative Ki 67 'low' (i.e. 25 score 'Luminal B-like (HER-2 positive)' ER positive HER-2 over-expressed or amplified Any Ki 67 Any PR 3. Non-luminal ( Erb B2 over-expression) HER-2 over-expressed or amplified ER and PR absent 4. Basal-like-triple negative (ductal) ER and PR absent HER-2 negative. Duration and type of chemotherapy 1. Anthracycline based chemotherapies (e.g. doxorubicin) should be preferred over the first generation chemotherapy regimens like CMF. 2. Taxols should be added sequentially to anthracycline based agents especially when the tumour is HER-2 positive and/ or more than 4 lymph nodes are positive or triple negative disease. 3. 6-8 cycles should be the number of cycles given to the patient. One example would be 4 cycles of adriamycin (doxorubicin) + cyclophosphamide given 3 weekly followed by 4 cycles of single agent paclitaxel given 3 weekly. 4. Duration of trastuzumab should be 1 year. It should not be given along with anthracycline based chemotherapy but can be given along with taxols. 5. Dose dense chemotherapies are the ones where the overall duration of chemotherapy cycles is reduced, e.g. instead of conventional three weekly gap between two cycles, the gap is reduced to 2 weeks. These are typically used in aggressive breast cancers like triple negative breast cancers. Duration and types of hormonal therapies 1. Tamoxifen can be used in both pre- and post-menopausal ladies, the total treatment duration is now recommended to be 10 years (earlier it was 5 years), especially when the chances of recurrence is high.

Breast

..

.

423

Other molecular subtypes may include varieties like 'molecular apocrine', 'normal breast like' , 'claudin low' .

Subtype

Type of therapy

Notes

Luminal A

Hormonal therapy (e.g. tamoxifen, letrozole, anastrozole)

Luminal 8 (HER-2 negative)

Adjuvant chemotherapy followed by hormonal therapy Adjuvant chemotherapy+ anti HER-2 (trastuzumab) + hormonal therapy Adjuvant chemotherapy+ trastuzumab Adjuvant chemotherapy

Relative indications of adding chemotherapy to hormonal therapy include: 1. High recurrence score on oncotype DX, i.e. > 25 2. High score on MammaPrint 3. Grade 3 disease 4. Involvement of 4 or more axillary lymph nodes. 5. Age< 35 years

Luminal 8 (HER-2 positive) Nonluminal Triple negative

2. Aromatase inhibitors are typically used in post-meno­ pausal ladies and should not be used in pre-menopausal females unless it is given along with ovarian suppression agents like Goserelin. 3. Other agents like Fulvestrant should only be used in the second line setting. • SERMs: Selective estrogen receptor modulators (agonistic in some tissues while antagonist in others) • Example: Tamoxifen is agonist on bone and uterus but antagonist in breast. Other example: Raloxifen • Fulvestrant, on the other hand is a pure antagonist. Novel agents in breast cancer 1. Trastuzumab-monoclonal antibody against HER-2/neu receptor on the breast cancer cell surface. 2. Lapatinib-dual tyrosine kinase inhibitor 3. Pertuzumab-HER dimerisation inhibitor 4. Ado-trastuzumab emtansine (T DMl )-antibody drug conjugate 5. Ixabepilone-microtubule stabilizer 6. Denosumab-a RANK ligand inhibitor used for palliation of bone metastasis.

Trastuzumab should be given in any tumour size greater than 5 millimeters Aggressive regimens like DOSE DENSE chemotherapy may be used.

INTERESTING 'MOST COMMON' IN BREAST DISEASES • Most common organism in lactational mastitis is Staphylococcus aureus. • Most common organisms in nonlactational mastitis are anaerobes. • Most effective drug used to treat cyclical mastalgia is evening primrose oil. • Most common cause of gynaecomastia is idiopathic. • Most breast cancers arise in ductal epithelium {90%) • Most breast cancers occur in upper outer quadrant (60%) • Most common presentation of carcinoma breast is with a lump • Most common type of carcinoma breast is scirrhous carcinoma (60 to 70%). • Most malignant form of carcinoma breast is mastitis carcinomatosa. • Most common reconstructive option in postmastectomy breast reconstruction is TRAM flap. • Most commonly used hormonal treatment in carcinoma breast is tamoxifen WHAT IS NEW IN THIS CHAPTER?/RECENT ADVANCES

• All topics have been updated. • The understanding of pathology and treatment of breast abscess has been updated. • Prophylactic mastectomy, sentinal node biopsy, flap breast reconstruction, has been edited • Latest treatment guidelines 'LUMINAL' has been added in carcinoma breast.

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Manipal Manual of Surgery

MULTIPLE CHOICE QUESTIONS 1. Following are true for lymphatic drainage of the breast except: A. Apical nodes are also called infraclavicular nodes B. Apical nodes drain into subclavian lymph trunk C. Posterior third of the breast drain into supra-clavicular nodes D. For detection of sentinel node, ideal site is subdermal plexus around nipple 2. Following are true for advantages of MRI in the breast except: A. It is the best modality for women with breast implants B. Screening in women with strong family history C. It is also better than ultrasound to image axilla D.It can distinguish scar from recurrence in women who have undergone breast conservative surgery 3. Following are true for retraction of the nipple except: A. Slit-like retraction is seen in duct ectasia 8. Circumferential retraction is seen in carcinoma breast C. Extension of the growth along lactiferous duct causes retraction of the nipple D. Horizontal retraction can occur at puberty suggests fibroadenosis 4. Following are true Lactational mastitis except: A. Retracted nipple is one of the cause B. Majority of the cases are due to anaerobic infection C. Repeated aspiration is recommended treatment D.Fluctuation is a late sign 5. Smoking is associated with which of the following breast disease? B. Breast abscess A. Tuberculosis D. Mondor's disease C. Duct ectasia 6. Following is not the common sign of Periductal mastitis: A. Discharge per nipple B. Indurated mass C. Fistula D. Circumferential retraction of the nipple

9. Following are true for Phylloides tumour except: A. Usual age of presentation is 20 years B. Large tumour with bosselated surface C. It may have high mitotic index D. Rarely develop into sarcoma 10. Which one of the following is the treatment of choic1 for early breast cancer in a 30-year-old lady who is � months pregnant? A. Chemotherapy B. Tamoxifen C. Local wide excision D. Modified radical mastectomy 11. Which is the drug used in a patient to prevent breas1 cancer with positive family history but unlikely carrier of breast cancer gene? B. Tamoxifen A. Adriamycin D. Trastuzumab C. Letrozole 12. Which one of this operative step is not done in Modified radical mastectomy? A. Total mastectomy 8. Axillary block dissection C. Removal of pectoralis major D. Removal of pectoralis minor 13. Following are the components of breast conservative surgery except: A. Excision of tumour plus a rim of 1 cm of normal breast tissue B. Axillary block dissection C. Routine removal of the skin over the tumour D. Sentinel node biopsy in selected patients 14. Following are true for radiotherapy in carcinoma breast except: A. It should be given after breast conservative surgery B. Large tumours after surgery require radiotherapy C. Extensive lymphovascular invasion is an indication D.Routinely given to axil la after a complete block dissection

7. The widely used first investigation of choice in a lady of 25 years with lump breast is: A. Ultrasonography B. CT scan C.MRI D.FNAC

15. Following are true for aromatase inhibitors except: A. Maximum use in the premenopausal patients 8. Relapse free survival is prolonged C. Reduction in the contralateral disease D. Increase in the bone density loss

8. Which one of the following is not the treatment for mastalgia? A. Evening primrose oil B. Danazol C. Steroids D. Bromocriptine

16. While of the following is the most important aetiological factor for carcinoma breast? A. Oral contraceptive pills B. Childhood i1Tadiation for Hodgkin's lymphoma C. Duct ectasia D. Racial factors

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Breast

17. Following are true for Ductal carcinoma in situ (DCIS) except: A. It is usually unilateral B. Multifocal C. Excision with or without radiotherapy is the treatment of choice D. No role for chemotherapy 18. Following are true for inflammatory carcinoma except: A. It results in blockage of dermal lymphatics B. It is considered as locally advanced breast cancer C. Neoadjuvant chemotherapy is the treatment of choice D. Severe degree of inflammation present pathologically 19. Following are true for lobular carcinoma in situ except: A. It is usually unilateral B. Multifocal C. Excision with or without radiotherapy is the treatment of choice D. 20 % will develop into invasive carcinoma 20. Following are true about internal mammary lymph nodes except: A. They drain posterior part of the breast B. They communicate with subdiaphragmatic lymph nodes C. They are included in the staging system now D. Their involvement indicates metastatic disease 21. Following are true for sentinel node biopsy in carcinoma breast except: A. Injection of patent blue localises the sentinel node B. Injecting into subdermal plexus around the nipple is ideal

C. Hand held gamma camera detects this D. It is ideal in clinically node positive axilla 22. Following is an indication for radiotherapy to chest wall in carcinoma breast except: A. Extensive lymphovascular invasion B. Large number of positive nodes in the axillla C. Base is involved by the tumour D. Skin is infiltrated 23. Following are true for tamoxifen except: A. It is widely used as hormonal treatment m premenopausal patients B. It decreases the annual recurrence by 25% C. It also has benefit in preventing contralateral breast cancer D. It is not recommended in patients who have family history of carcinoma breast but unlikely carriers of breast cancer gene 24. Treatment option for carcinoma breast in pregnancy is: A. Local wide excision B. Radiotherapy C. Chemotherapy D. Mastectomy 25. Which one of the following is not true in male breast cancer? A. Most commonly it is infiltrating duct carcinoma B. Mostly it presents as bleeding per nipple C. Mostly mastectomy is required D. Gynaecomastia is a predisposing factor

ANSWERS C

2 C

3 D

4 B

5 C

6 D

7 A

16 B

17 B

11 B

12 C

13 C

14 D

15 A

21 D

22 D

23 D

24 D

25 B

C

9 A

10 D

18 D

19 A

20 D

8

Gastrointestinal Surgery 22. 23. 24. 25.

26.

27. 28. 29.

30. 31. 32.

33. 34. 35. 36. 37.

Oesophagus and Diaphragm Stomach and Duodenum Liver Gall Bladder and Pancreas Spleen Peritoneum, Peritoneal Cavity, Mesentery and Retroperitoneum Small Intestine Large Intestine Intestinal Obstruction Rectum and Anal Canal Lower Gastrointestinal Bleeding The Appendix Hernia Umbilicus and Abdominal Wall Blunt Abdominal Trauma, War and Blast Injuries and Triage Abdominal Mass

22 Oesophagus and Diaphragm • • • • • • • •

Surgical anatomy Physiology GORD Motility disorders Achalasia cardia Nutcracker oesophagus Carcinoma Stricture

• • • •

Perforations Diverticulum Dysphagia Surgical anatomy of the diaphragm • Diaphragmatic hernia • Tracheo-oesophageal fistula • What is new?/Recent advances

SURGICAL ANATOMY • Oesophagus is 25 cm in length, extending from the cricopharyngeal sphincter to the cardio-oesopbageal junction (Fig. 22.1). • Cardio-oesophageal junction lies to the left ofT 11 vertebra. It is identified at endoscopy by a Z-line.

• It is a jagged line where the oesophageal mucosa changes to gastric mucosa. • Collar of Helvitius: It is the site at which the circular muscles of oesophagus turns to oblique muscles of the stomach at the incisura. • It runs in the posterior mediastinum as a continuation of pharynx. 2 cm of this tube lies below the diaphragm. • Physiological constrictions (Table 22.1)

From incisor teeth

!1scm Cervical

t

---- Cricopharyngeus

3 cm

Upper thoracic

24 cm

32 cm

Midthoracic

Lower thoracic/abdominal

.. .

Cardio-oesophageal junction

Fig. 22.1: Division of oesophagus into 4 parts based on distance from incisor teeth Physiological constrictions

Constrictions I. Cricopharyngeal 2. Aortic and bronchial 3. Diaphragmatic sphincter

Distance from incisor teeth

Diameter of oesophagus

15 cm 25 cm 40cm

14mm 15-17 mm 16--19 mm

429

Problems Foreign body lodgement Perforations during endoscopy Malignancy

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Manipal Manual of Surgery

Muscle layers (Figs 22.2 and 22.3) • It has an inner circular layer and an outer longitudinal layer. • Upper 113rd has striated muscle fibres. • Lower 213rd has smooth muscle fibres. PEARLS OF WISDOM Clinically significant motility disorders affect lower two-thirds (smooth muscle) of oesophagus.

Mucosa • The entire oesophagus is lined by squamous epithelium except last 3 cm which is lined by columnar cells. The columnar cells are similar to gastric mucosa but oxyntic and peptic cells are absent. • Mucosa is the toughest coat of oesophagus. • The smooth oesophageal mucosa gets transformed into rugal folds. Lymphatic drainage (Key Box 22.1) • Upper oesophagus drains into the left and right supra­ clavicular nodes. • Middle oesophagus drains into the tracheobronchial nodes and paraoesophageal nodes. • Lower oesophagus drains into lymph nodes along the lesser curvature of stomach and then into coeliac nodes. Involve­ ment of coeliac nodes indicate inoperability. Nerve supply The parasympathetic nerve supply is by vagus nerve through extrinsic and intrinsic plexuses. The intrinsic plexus has no Meissner's network and Auerbach's plexus is present only in the lower two-thirds.

............................. . KEY BOX 22.1

LYMPHATIC DRAINAGE OF OESOPHAGUS

• Extensive lymphatic plexus in the submucosa which explains the easy spread • Like in the colon they are classified as perioesophageal, paraoesophageal and lateral oesophageal lymph nodes • Thus throughout the entire length of oesophagus various groups of lymph nodes are enlarged such as deep cervical, mediastinal, subcranial, hilar, diaphragmatic, para and peri­ oesophageal, gastric, coeliac, etc. • Presence of coeliac nodes indicate inoperability • Longitudinal lymphatics are 6 times more than transverse. Hence, distant spread occurs fast-satellite nodules occur proximal and distal to the growth. This is the reason why 1 O cm margin must be given in resection of the oesophagus • One of the advantages of transhiatal resection over Ivor Lewis operation (see page 449) Blood Supply (Figs 22.4 and 22.5) Arterial • Cervical oesophagus: Mainly from branches of the inferior thyroid artery. • Upper thoracic oesophagus: Mainly from branches of the inferior thyroid artery and less consistently from anterior oesophagoh·acheal branch from aorta. • Mid and lower thoracic oesophagus: Supplied by bronchial arteries. • Lower oesophagus: Small branches of the left gastric artery. Rich internal arterial anastomosis is present in the oesophagus and in the stomach. Hence, extensive mobilisation of oesophagus can be done without compromising viability.

Fig. 22.2: You can see the multilayered structure. Endosonography detects all these layers

Helicoidal -....-'-:;-:---muscle

..

,)-:�

··- ·.,

Fig. 22.3: Helicoidal muscle

Fig. 22.4: Oesophageal anatomy­ relationships

Fig. 22.5: Oesophageal anatomy­ segmental blood supply

Oesophagus and Diaphragm

431

Surgical anatomy of the oesophagus-key points Surgical anatomy

Significance

Surgical points of interest

1. Upper oesophageal sphincter 2. Lower oesophageal sphincter

Dense cricopharyngeus muscle High pressure zone at gastro-oesophageal junction Toughest coat of oesophagus Requires mobilisation Helps in peristalsis but it recoils due to elasticity

Zenker's diverticulum through Killian triangle Weakness causes reflux oesophagitis

3. Oesophageal mucosa 4. No serosa, no mesentery 5. Helicoidal muscle 6. Segmental arterial supply 7. Lower end of oesophagus-veins

Extensive mobilisation can be done without compromising blood supply Rich intercommunicating veins between portal and systemic veins Can get injured and bleed

8. Azygos vein crossing oesophagus in thorax 9. Mucosa! and submucosal lymphatics Oesophageal tumours extend over a long out number capillaries distance within oesophageal wall

Venous drainage Veins accompany corresponding arteries. Importantly, thoracic oesophagus drains into azygos and hemiazygos veins. • Table 22.2 PHYSIOLOGY (Fig. 22.6) The main function of the oesophagus is to transfer food from the mouth to the stomach. Voluntary contraction of the oropharynx pushes food into the upper oesophagus through a relaxed cricopharyngeal sphincter (Key Box 22.2). Then, due to primary and secondary peristalsis, the food bolus is transferred to the stomach.

M$MtwllllW'� • • • • •

THE PHARYNGEAL STAGE-REFLEX Food stimulates mechanoreceptors Soft palate is pulled upwards so that food will not enter nasal cavities Vocal cords adduct and larynx is pulled upwards against epiglottis so that food will not enter trachea Cricopharyngeal sphincter opens Muscular wall of the pharynx then contracts from above downwards.

• Cricopharyngeal sphincter is closed at rest. It helps in preventing regurgitation of oesophageal contents into the respiratory passage. • Lower oesophageal sphincter (LOS): It is a physiological sphincter at the gastro-oesophageal junction. It is 3--4 cm

Important in oesophageal anastomosis Leak rate is high, easy spread of carcinoma Hence, once resection takes place-specimen 'shortens' or retracts-anastomosis may become difficult especially in abdominal anastomosis Hence, transhiatal esophagectomy (THE) is done with ease Oesophageal varices occur here Resection of midoesophageal tumours Need for total oesophagectomy and cervical anastomosis + lymph node clearance

Masticated food

...J 3 mg% B. Tumour thrombus in the main portal trunk C. Early hepatocellular carcinoma D. Inoperable tumours 20. Which of the following is true criteria for secondaries in the liver? A. Single lobe enlargement B. Rounded border C. Soft in consistency D. Nodular liver 21. Commonest gastrointestinal tract primary in a nodular liver secondaries is from: A. Stomach 8. Pancreas C. Gall bladder D. Duodenum 22. Portal hypertension is defined as a portal venous pressure exceeding __ mmHg. B. 10 A. 5 C. 15 D. 20

23. Portal vein normally carries ____ % of blood supply to liver. B. 50 A. 25 D. 100 C. 75 24. Causes of bulky secondaries include the following except: A. Malignant melanoma B. Carcinoid tumours C. Colloid carcinoma rectum D. Testicular tumours 25. Which of the following is true about the use of vasopressin in variceal bleeding? A. 20 units is diluted in 200 ml of saline and given over 20 min B. Causes coronary vasodilatation C. Is a powerful inotrope D. Is a splanchnic vasodilator and reduces pressure in the splanchnic vessels 26. Gastric transection of Tanner is an operation performed for: A. Carcinoma stomach B. Carcinoma pancreas C. Portal hypertension D. Hepatocellular carcinoma

ANSWERS 1 D

40

2 C

3A

11 B

12 C

13 C

14A

21 A

22 C

23 C

24 D

6A

7A

8 C

9 D

10 A

15 D

16A

17 C

18 B

19 D

20 D

25 A

26 C

5 D

25 Gall Bladder and Pancreas • • • • • • • • •

Surgical anatomy Physiology Congenital anomalies Gall stones disease Acute cholecystitis Chronic cholecystitis Obstructive jaundice Stricture CBD Sclerosing cholangitis

• • • • • • • • •

Choledochal cyst Caroli's disease Chronic pancreatitis Cholangiocarcinoma Congenital biliary atresia Carcinoma of gall bladder Carcinoma of pancreas Endocrine tumours Acute pancreatitis

INTRODUCTION

• One may wonder why gall bladder and pancreas are discussed together!! Yes, biliary tract (gall bladder is an important part of that) ends after joining with pancreatic duct by opening into 2nd part of duodenum. • Gall stones, when they block/pass through ampulla ofVater, produce acute pancreatitis. • An important surgical condition-surgical jaundice or obstructive jaundice can be discussed better only if you know gall bladder and pancreas. • Any surgery involving sphincter of Oddi in the form of sphincteroplasty/or removal (as in Whipple's pancreati­ coduodenectomy), the gall bladder does not function and hence it needs to be removed. • Embryologically, liver, biliary tree, ventral pancreas, gall bladder-all developed from a diverticulum on the ventral aspect of the foregut of the embryo. • And gall bladder will be palpable in cases of periampullary/ carcinoma head of the pancreas.

• • • • • • • • •

Pseudocyst Annular pancreas Ectopic pancreas Cystic fibrosis Pancreatic divisum Pancreatic fistula White bile Pancreatic ascites What is new?/Recent advances

• Fundus: It is the dilated portion of the gal !bladder adheren1 to undersurface of liver from which it can be separated easily. • Neck: The narrow angulated distal portion of the neck is called Hartmann 's pouch-common site where stones occur and tend to stay for a long time (also called infundibulum of gall bladder) (Fig. 25.1). • Gall bladder drains into the common bile duct (CBD) through cystic duct, which is 3 cm long. It is lined by cuboidal epithelium. There are prominent mucosal folds within the cystic duct due to the presence of prominent

Fundus Body

SURGICAL ANATOMY OF THE GALL BLADDER AND BILE DUCTS

Gall bladder is a pear or globular shaped organ present in the right hypochondrium on the inferior surface of the liver, situated in the gall bladder fossa. It is about 8-12 cm long.

550

Valves of Heister---� Fig. 25.1: Surgical anatomy of the gall bladder

Gall Bladder and Pancreas

circular muscle fibres underneath. Its lumen is usually 1-3 mm in diameter. Contraction of gall bladder produces a functional valve called valve ofHeister which prevents the migration of stone into the CBD. The wall of cystic duct is surrounded by a sphincter structure called sphincter of Lutkens. A spiral fold keeps cystic duct open for drainage of bile. Cholecystohepatic triangle or Calot's triangle boundaries • Lateral: Cystic duct and gall bladder • Medial: Common hepatic duct • Above: Inferior surface of right lobe of the liver. It is an important landmark in the identification of cystic duct, and cystic artery during cholecystostomy so as to avoid damage to extrahepatic biliary tree.

551

accounts for frequent spread of carcinoma gall bladder to the liver. Anatomy of the bile ducts • Common hepatic duct (CHD) is formed by the union of right and left hepatic ducts. It is 3 cm long, receives cystic duct and continues as common bile duct (CBD). • Common bile duct is about 8 cm long. It has four parts: Supraduodenal, retroduodenal, infraduodenal and intraduodenal. Along with pancreatic duct, it forms ampulla of Yater. Controlled by sphincter of Oddi, it ends by an opening into the second part of duodenum (Fig. 25.3).

Contents • Right hepatic artery and its branch, the cystic artery • Cystic lymph node of Lund.

SupraduodenalCBD

J-:f--'�-- Retroduodenal CBD lntraduodenal -..-, �IIMl�.JI!!�� CBD

Blood supply of gall bladder Cystic artery, a branch of right hepatic artery arises behind the common bile duct. Soon, it branches out over the surface of gall bladder. Cystic artery is an end artery (Fig. 25.2). Multiple small veins from the surface of gall bladder join the liver surface. There is also a cystic vein, from the neck of gall bladder draining into portal vein directly. This explains early spread of gall bladder malignancy to the liver. Lymphatics 1. Subserosal and submucosal lymph nodes drain into cystic lymph node of Lund and from here they drain into nodes in the hilum of liver and coeliac nodes. 2. Subserosal lymphatic vessels of gall bladder are also connected to subcapsular lymph channels of liver, which

Right hepatic artery

Left hepatic artery

Common bile duct

-----

---Common hepatic artery

Fig. 25.2: Blood supply of the gall bladder

Sphincter of Oddi

�-- lnfraduodenal CBD

Fig. 25.3: Surgical anatomy of the bile ducts. Note how close gall bladder is to the duodenum (chances of injury in laparoscopic cholecystectomy)

PHYSIOLOGY Functions of the gall bladder • Reservoir for bile: Bile excreted by the liver is stored in the gall bladder as total of about 500 to 1000 ml per day. At fasting, the tone of sphincter of Oddi is high. Food contents in the duodenum stimulates release of cholecystokinin, which causes gall bladder to contract. • Concentration: Bile is 98% water. Due to active absorption of water, sodium chloride and bicarbonate, bile gets concentrated 5-10 times. Thus, a relative increase in bile salts, bile pigments, cholesterol and calcium occurs. • Mucus secretion: It secretes about 20 ml/mucus per day. Obstruction to the cystic duct causes mucocoele of the gall bladder. Bile • Secreted from hepatocytes • pH is more than 7.0

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Manipal Manual of Surgery

• 500-1000 ml/day, 98% is water • Concentrated in gall bladder because of absorption of water. Capacity of gall bladder is 40-50 ml. • Fatty food stimulation releases cholecystokinin, which stimulates gall bladder to contract and at the same time, sphincter of Oddi to relax. • It also has inorganic ions (more than plasma) and hence, severe electrolyte imbalance is seen in biliary fistula. • Cholesterol, synthesised in the liver, gives rise to bile acids-cholic and chenodeoxycholic acids. They are meta­ bolised in the colon to deoxycholic acid and lithocholic acids. • Main function of bile acids in the bile is to maintain cholesterol in solution. CONGENITAL ANOMALIES OF GALL BLADDER Absence of gall bladder: Very rare; other variations include (Fig. 25.4): A. Floating gall bladder: Results due to long mesentery. It is more vulnerable to torsion-a rare cause of recurrent upper abdominal pain. Such a gall bladder can be easily removed. B. Phrygian cap: Cap which was worn by people of Phrygia (ancient Asian country, Mongolia). It is an anomaly connected with the fundus of the gall bladder. C. Double gall bladder: The second one is always intrahepatic (rare). D. Absence of cystic duct: Cholecystectomy becomes difficult. There are high chances of injury to the common bile duct. E. Low insertion of cystic duct: Cystic duct opens into the common bile duct near the ampulla. This anomaly should

be kept in mind when operating on cases of obstructiv jaundice. F. An accessory or aberrant cholecystohepatic duct i present in about I 0% of the patients. It may be the cause o significant bile leakage after cholecystectomy. It is th segmental duct that joins biliary system outside the live instead of within it. G. Diverticulum of gall bladder H. Cystic duct joining right hepatic duct I. Anomalies of blood supply 1. Very, very tortuous hepatic artery: Caterpillar turn o Moynihan's hump. It runs in front of the origin of cysti, duct. 2. Cystic artery is given anteriorly from right hepati, artery. GALL STONE DISEASE (CHOLELITHIASIS) Aetiology 1. Metabolic causes • Cholesterol is produced from the liver which gives rise tc bile acids. Cholesterol is insoluble and it must be transportec within the bile salt micelles and phospholipid (lecithin: vesicles. Normal ratio of bile acids: cholesterol is 25: l (Fig. 25.5). • This ratio is necessary to maintain the cholesterol in liquic form by fanning micelles. When the ratio drops down tc 13: 1 (which is called critical ratio), cholesterol crystals will nucleate and stones will form. • Obesity, high calorie diet and medications which increase cholesterol secretion can result in stone formation.

1-2

Fig. 25.4: Congenital anomalies of the gall bladder (see text A to I)

Gall Bladder and Pancreas Cholesterol

Concentration of phospholipid

Secreted from canalicular membrane in phospholipid vesicles +

Bile acids in bile

Micelle formation 1--------1 Supersaturated bile or 1--------; Bile acid concentration is low Unstable phospholipid vesicles form

Cholesterol crystals formation

Cholesterol stones

Fig. 25.5: Flow chart showing formation of cholesterol stones

2. Infection • It is the most common cause responsible for a gall stone in 80% of patients. Sources of infection are tonsils, tooth, bowel, etc. • Organisms such as E. coli, Proteus, anaerobic organisms, Streptococci, etc. reach the gall bladder wall through the blood stream and form a focus/nidus around which cholesterol and bile salts get precipitated. • Over a period of many years, this results in a mixed stone. They are usually multiple and occur in infected bile. PEARLS

OF

WISDOM

"A gall stone is a tomb stone erected in the memory of organisms within it. "-Lord Moynihan

3. Bile stasis and decreased bile acid pool • Pregnancy, oestrogens, following vagotomy and prolonged total parenteral nutrition are associated with bile stasis. • They are prone to mixed stones as a result of bile stasis (Key Box 25.1).

............................. . KEY BOX 25.1

1. 2. 3. 4. 5. 6.

CAUSES OF DECREASED BILE ACID POOL � Cirrhosis of liver-pigment stones Gastrectomy lleal resection Malabsorption Obesity Hypercholesterolaemia

553

4. Haemolytic anaemia • Examples: Hereditary spherocytosis, sickle cell anaemia. • Bilirubin production is increased because of increased break down of RBCs. Since the production is more, they cannot conjugate with glucuronic acid, which is produced at normal levels. • Such unconjugated bilirubin combines with calcium and is excreted in the biliary tree resulting in calcium bilirubinate stones (pigment stones) not only in the gall bladder but also in the entire ductal system. 5. Saint's triad Gall stones ( can occur along with two other conditions mentioned below) • Diverticulosis of colon • Hiatus hernia 6. Parasitic infestation • In Oriental countries, Clonorchis sinensis (Chinese liver fluke) infestations can cause stone in the biliary tree. • Ascaris lumbricoides in the biliary tree may produce stones in our country (India). 7. Due to abnormal mucus It is produced in congenital cystic fibrosis. Gall stones occur in these children due to impairment of bile flow. Other diseases associated with gall stone disease • Diabetes mellitus • Type IV hyperlipoproteinaemia • Cirrhosis of liver • Fistulae on treatment with total parenteral nutrition • Gastric surgery Risk factors for gall stone disease (Key Box 25.2 and Fig. 25.6)

............................. . KEY BOX 25.2

• • • •

RISK FACTORS Female sex Obesity Maturity onset diabetes Age > 40 years

Types of gall stones (Figs 25. 7 to 25.16) 1 . Cholesterol stones • Constitutes about 10% of the gall stones. • Occur in patients with increased cholesterol levels. • Fatty women are commonly affected. • It is single, solitary, occurs in aseptic bile. Sometimes they can be multiple. Precipitation of cholesterol gives rise to stone.

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Clinical features (complications of gall stones) Clinical presentation of these patients vary from dyspepsia tc severe forms such as pancreatitis and perforation of th1 gall bladder (Key Box 25.3). Classified as in the gall bladder in the CBD and in the intestines.

............................. KEY BOX 25.3

.

IN THE GALL BLADDER Silent stones Flatulent dyspepsia Gall stone colic Acute cholecystitis Chronic cholecystitis Mucocoele Empyema Perforation Carcinoma of the gall bladder Mirizzi's syndrome IN THE BILE DUCT • Obstructive jaundice • Cholangitis • White bile • Acute pancreatitis IN THE INTESTINE Acute intestinal obstruction (gall stone ileus) • • • • • • • • • •

Gall stones

Diverticular disease

Septic focus in the oral cavity

Fig. 25.6: A few risk factors for gall stone disease

• Such stones can be silent for many years. They are radiolucent. • Pigment can also get precipitated along with cholesterol. 2. Brown pigment stones • Rare in gall bladder, occurs in bile duct. • Composed of calcium bilirubinate, calcium palmitate and calcium stearate + cholesterol. • Occur due to bile stasis caused by foreign bodies, endo­ prosthesis, Clonorchis sinensis and Ascaris lumbricoides. 3. Mixed stones • They constitute about 80% of gall stones. • They contain alternating layers of cholesterol and pigment with epithelial debris or vegetations, from infective organisms. • They are multiple, small, faceted by mutual pressure. 4. Pigment stones • They are found in 5 to 10% of patients. • They are calcium bilirubinate stones. • Commonly occur due to haemolysis. Hence, they are black, multiple, small, irregular concretions or sludge particles. • For reasons not clear, cirrhotic patients have increased incidence of black pigment stones. • Bacteria also have a major role to play in the formation of pigment stones. Patients with pigment stones have more sepsis than patients with cholesterol stones.

COMPLICATIONS IN THE GALL BLADDER Silent stones • This is usually a single, silent, cholesterol stone which is symptomless. • It is accidentally discovered, may be by an ultrasound or plain X-ray abdomen (since calcium content is low in a cholesterol stone, it is very rarely visible in a plain X-ray). This stone rarely causes obstructive jaundice. • Hence, it is left alone without treatment. Flatulent dyspepsia If an obese woman (fatty, fertile, flatulent, female in forties) complains of gaseous distention, intolerance to fatty food and discomfort in the abdomen, heartburn and belching, she probably has gall stones. These patients benefit from cholecystectomy.

Gall Bladder and Pancreas

Fig. 25.7: Mixed stones-cause of flatulent Fig. 25.8: Pigment stones­ case of hereditary spherocytosis dyspepsia

Fig. 25.10: Mixed stones-observe faceting-cause of gall stone colic

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Fig. 25.9: Double GB with stones

Fig. 25.11: Gall stones- see the colour

Fig. 25.12: Contracted thick­ walled gall bladder

Fig. 25.13: Multiple 2 mm stones. This lady had 25.14: Gall bladder Figs 25.15 and 25.16: Gall stones: Multiple stones are was opened to responsible for obstructive jaundice 3 attacks of gall stone pancreatitis extract stones

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Gall stone colic • It usually occurs at night wherein a stone tends to block the cystic duct or neck of gall bladder in the supine position. • It is a severe colicky upper abdominal pain felt in the right hypochondrium, may shoot to the back or between shoulder blades. The pain is continuous and lasts for a few hours. Pain may radiate to chest also. - The pain is due to spasm of gall bladder - It is associated with vomiting due to reflex pylorospasm, restlessness and sweating. - There is tenderness in the right hypochondrium. • Pain may last for a few minutes to a few hours. • For differential diagnosis (Key Box 25.4).

............................. . KEY BOX 25.4

DIFFERENTIAL DIAGNOSIS OF GALL STONE COLIC

• Chronic duodenal ulcer • Reflux oesophagitis (some cause can present as precordial chest pain) • Pancreatitis • Myocardial infarction ACUTE CHOLECYSTITIS Definition: Acute bacterial inflammation of the gall bladder with or without stone. Types 1. Calculous: Obstructive cholecystitis. It is the commonest variety. Calculi cause bile stasis. 2. Acalculous: Nonobstructive cholecystitis. It is not uncommon and is seen in patients who are recovering from major illness (Key Box 25.5). 3. Acute emphysematous cholecystitis. Bacteriology of acute cholecystitis 1. Majority of the cases of calculous cholecystitis are due to organisms such as E. coli, Streptococci, Salmonella, Klebsiella, etc.

............................. .

2. Typhoid fever can also cause Typhoid Cholecystitis arourn 2nd week of infection (Key Box 25.6). 3. Clostridial infection of the gall bladder produces acut1 cholecystitis with toxaemia.

............................. . KEY BOX 25.6

TYPHOID INFECTION OF THE GALL BLADDER • Salmonella typhi or S. typhimurium are the organisms

• Acute cholecystitis can occur in the 2nd week of typhoid fever. • Long lasting infections-chronic cholecystitis can occur • Bacilli can be present in the bile for a long time • Obstruction by a pre-existing stone or any other cause • Necrosis of GB, ulceration, perforation can be dangerous • Local tenderness, on the right side of abdomen, guarding can occur. • Antibiotics against Salmonella must be given Pathogenesis (Key Box 25. 7) • Acute calculous cholecystitis appears to be caused by obstruction to bile flow from gall bladder by stone or oedema formed as a result of local mucosa! erosion and inflammation caused by stone. Once mucosa is eroded, tissue planes are exposed to bile salts. Toxic bile salts destroy cells by their detergent action leading to necrosis and perforation of gall bladder. • At the same time, bacterial infection adds to the morbidity of acute cholecystitis. Positive bile cultures are found in 70% of cases of acute calculous cholecystitis. Pathology 1. Inflammation: Entire gall bladder is inflamed, swollen and is friable. When the inflammatory exudate surrounding the gall bladder collects under the diaphragm, it results in pain radiating to the right shoulder (C3, 4) due to phrenic nerve

............................. . KEY BOX 25.7

STONE

KEY BOX 25.5

PERCUTANEOUS CHOLECYSTOSTOMY

• In life-threatening situations with severe sepsis due to gall stones, percutaneous cholecystostomy seems to be a very good alternative to save the life of the patient. • Indicated in acalculous cholecystitis in which the patient's condition is serious with sepsis with co-morbid conditions. • Using ultrasound or CT guidance, a pigtail catheter can be inserted into the gall bladder, ideally transperitoneally. • It is the treatment of choice for acalculous cholecystitis. • 3-4 days later, when sepsis improves, laparoscopic/open cholecystectomy can be done.

Outlet obstruction

Mucosal erosion

Destruction of cells by toxic bile salts Bacterial proliferation

Bacterial proliferation

[ Necrosis and perforation J

Gall Bladder and Pancreas Stone blocking cystic duct

Inflamed edematous wall

Gall stone colic

Acute cholecystitis

Empyema

Contracted thickened wall

�

Chronic cholecystitis

Cancer

557 Mucus is accumulated

Mucocoele

Perforation

CHO

Obstructive jaundice

White bile

Gall stone pancreatitis

Gall stone ileus

Fig. 25.17: Diagrammatic representation of the complications of gall stones. They are discussed in the subsequent pages

irritation. It may undergo complete resolution with antibiotic therapy but such recurrent attacks are common at a later date (Fig. 25. I 7). 2. Perforation: Extensive ulcerations of gall bladder may result in perforation with biliary peritonitis and carries a very high mortality rate. Perforation can occur when the stone is impacted in the Hartmann's pouch. 3. Obstruction to the neck of gall bladder results in muco­ coele or pyocoele (empyema). Empyema of the gall bladder can occur in diabetic patients and is associated with high grade fever, chills, rigors and even septicaemia. 4. Gangrene of gall bladder can occur if the blood vessels get thrombosed. All these features are more in an obstructive variety. • If there is clostridial infection as can occur in diabetics because of extensive gas production in biliary tree and associated toxicity, perforation is likely even without a stone. Clinical features • A fatty, fertile, female is the typical victim who presents with severe upper abdominal pain (Figs 25. I 8 and 25. I 9). The pain is colicky in nature and more prolonged because of inflammation. Severe nausea and vomiting are present. In the initial phase, there is low grade fever, except in clostridial infection where there is high grade fever. • You may be surprised to find a young boy, a girl or even a child with gall stones-suspect haemolytic anaemias. Signs 1. Murphy's sign • Keep the fingers in the right hypochondrium and ask the patient to take a deep inspiration.

Figs 25.18 and 25.19: Large 2 stones blocking lumen-cause for severe colic

• At the height of inspiration there is a sudden catch in the inspiration. • [t is due to inflamed gall bladder coming in contact with the abdominal wall under the fingers and producing pain. This is called Murphy's sign positive. It is a diagnostic sign of acute cholecystitis (Fig. 25.20). 2. Boas' sign: An area of hyperaesthesia between 9th and 11th ribs posteriorly on the right side is a feature. 3. Upper abdominal guarding, rigidity. 4. Vague mass consisting of inflamed gall bladder, omentum, inflammatory exudate can be felt at times. Hence, even if a perforation occurs, generalised peritonitis is uncommon.

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5. Lobar pneumonia (basal): Can cause right hypochondriac guarding and rigidity. It is a referred pain.

•

Fig. 25.20: Eliciting Murphy's sign

Differential diagnosis {DD) (Fig. 25.21) 1. Perforated peptic ulcer: Severe sudden pain, severe tenderness in the right hypochondrium, guarding and rigidity caused by perforated peptic ulcer, can mimic acute cholecystitis. Obliteration of liver dullness, coffee ground vomitus, generalised guarding and rigidity clinches the diagnosis of perforated duodenal ulcer. 2. Acute pancreatitis: A severe pain in upper abdomen, tenderness in the right hypochondrium and epigastrium mimic cholecystitis. One should remember that pain of pancreatitis is more severe and classically radiates to back. 3. High retrocaecal appendicitis: Especially when appendix is in the subhepatic position. Once inflammatory fluid spreads in the general peritoneal cavity, there will be more difficulty in diagnosing clinically. 4. Amoebic liver abscess: Can also mimic very closely. It is more common in male alcoholics. Liver is enlarged and one can feel the round lower border of liver very closely. Liver will be extremely tender.

Fig. 25.21: Differential diagnosis of acute cholecystitis (see text)

Investigations 1. Total WBC count is always raised. 2. Blood and urine sugar estimation to rule out diabete� mellitus. 3. Plain X-ray abdomen erect position (Fig. 25.22) • Gall stones can be demonstrated in 10% of the patients as radio-opaque shadows in the right hypochondrium. fn lateral view, the stone is seen in front ofvertebral bodies. • To rule out other causes such as perforated peptic ulcer (air under diaphragm). • Rarely, it may show calcified gall bladder (porcelain gall bladder). 10% Gall stones are radio-opaque, 90% are radiolucent. Centre of stones may contain radiolucent gas, either triradiate (Mercedes Benz sign) or biradiate (Sea Gull sign). 4. Emergency ultrasonography (Fig. 25.23) • To demonstrate stones, which cast posterior acoustic shadow. • Success rate is > 95% • lt can demonstrate inflamed, thickened organ, in cases of acalculous cholecystitis. • Demonstration of Murphy's sign, with the help of ultrasonography is possible which adds to the diagnosis. • Ultrasound can also measure gall bladder function by using ultrasonic dimensions of the gall bladder. • It can detect gall bladder polyps. 5. HIDA scan/PIPIDA scan (Fig. 25.24) • HIDA is hepatic iminodiacetic acid. • 99111Tc labelled HIDA agent is excreted in the biliary tree, within one hour following IV administration.

Fig. 25.22: Plain X-ray abdomen showing radio-opaque shadows

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559

2. Aspiration with Ryle's tube: Aspiration of HCl decreases the stimulus to the secretion of bile. Spasm of gall bladder may come down. 3. Antispasmodics: Injection morphine 8-10 mg IM as analgesic along with injection atropine 0.6 mg to relieve spasm of sphincter of Oddi. 4. Antibiotics: Broad spectrum antibiotics are given against gram +ve, gram -ve and anaerobic organisms. Cefazolin, cefuroxime or amikacin are the drugs of choice. The patient is kept nil orally for 2-3 days and during this time IV fluids are given. After 2-3 days, pain comes down, signs (tenderness) disappear and abdomen becomes soft. Ryle's tube is removed, clear oral fluid is given for 2-3 days followed by soft diet. After 6 weeks, the patient is advised to undergo elective cholecystectomy. Reason for conservative treatment is in majority of cases, inflammation will settle down. Fig. 25.23: Ultrasonography showing posterior acoustic shadows

Fig. 25.24: HIDA scan showing nonvisualisation of gall bladder­ a case of acute cholecystitis (CT is more specific)

• In acute cholecystitis, even though the dye is excreted in the biliary tree, it does not enter the gall bladder due to oedema of the cystic duct. Hence, nonvisualisation of gall bladder is diagnostic of acute cholecystitis. • Its importance lies in the diagnosis of acalculous cholecystitis and when diagnosis is in doubt. 6. CT Scan: • It is done when ultrasound findings are not clear • It not only diagnoses gall stones, but also detects other complications such as perforation, stones in the CBD, etc. • Renal halo sign due to fluid around • Obliteration of psoas shadow • Air fluid level in duodenum are the features. Treatment: School of thought I. Conservative treatment is followed in majority of the cases (60 to 70%) I. Admission in the hospital

II. Early cholecystectomy • Patients in the first group need two admissions, cost is increased and return to work is also delayed. Hence if a surgeon is experienced and the set up is good, one can proceed to early cholecystectomy from 2nd day to 7th day. • It has been proved that even though gall bladder is inflamed, complications are no way more than elective cholecystec­ tomy in the hands of experienced surgeon while performing laparoscopic cholecystectomy. • Thus, if a firm preoperative diagnosis is established and some of the comorbid conditions are corrected (diabetes, hypertension, etc.), surgery can be done safely. This is called early cholecystectomy. Ill. Emergency cholecystostomy About 10% of cases of acute cholecystitis require emergency cholecystostomy. • In these patients, high grade fever, sepsis, shock, high leukocyte count are the deciding factors. • Acalculous cholecystitis and perforated gall bladder with peritonitis are definitely strong indications for emergency cholecystectomy. IV. What is prophylactic cholecystectomy? It means removal of gall bladder with stones without symptoms (Key Box 25.8). Prognosis • Overall death rate is 3-5%. • Contributing factors for death are diabetes, age above 60 years, cardiovascular or pulmonary disease. • Uncontrolled sepsis, intra-abdominal abscess.

............................. . KEY BOX 25.8

PROPHYLACTIC CHOLECYSTECTOMY • Diabetic patients • Congenital haemolytic anaemia • Patients undergoing bariatric surgery

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CHRONIC CHOLECYSTITIS • Recurrent attacks of cholecystitis will convert the gall bladder into a fibrosed, nonfunctioning, contracted, shrunken, small gall bladder. Gall bladder wall is grossly thickened. Stones are invariably present. Such patients present with classical fatty food intolerance. Murphy's sign is positive. • They are diagnosed by ultrasound which reveals a small, contracted gall bladder. Otherwise, oral cholecystography (OCG) can be done to know the function of the gall bladder. Treatment Cholecystectomy (Fig. 25.25)

Types 1. Cholesterosis (Strawberry gall bladder): Aggregations o cholesterol crystals in the mucosa/submucosa. Yellov specks of cholesterol crystals are seen when gall bladde is opened (xanthogranulomatous) (Key Box 25.9 an< Figs 25.26 and 25.27). 2. Cholesterol polyposis (gall bladder polyp): Polypoida projections of mucosa in the gall bladder. Polyp longer that. 1 cm or change in size require surgery. 3. Cholecystitis glandularis proliferans: Granulomatom thickening and hyperplasia of the gall bladder. All layen of gall bladder are thickened. 4. Diverticulosis of gall bladder 5. Gall bladder wall with fistula Clinical features Dyspepsia, upper abdominal discomfort, Murphy's sign is positive. Management Ultrasound to confirm the diagnosis followed by chole­ cystectomy.

Fig. 25.25: Cholecystectomy specimen-observe thickened gall bladder wall

CHOLECYSTOSES

Fig. 25.26: Xanthogranulomatous cholecystitis with stone

Definition Uncommon group of conditions affecting gall bladder in which there are chronic inflammatory changes and hyperplasia of all tissue elements.

............................. . KEY BOX 25.9

XANTHOGRANULOMATOUS CHOLECVSTITIS • It is a pathological diagnosis • Gall bladder is chronically thickened, irregular with extension of yellow xanthogranulomatous inflammation to adjacent organs. • It is thought to be due to bile penetrating gall bladder wall

Fig. 25.27: Xanthogranulomatous cholecystitis at surgery

Gall Bladder and Pancreas

MUCOCOELE (Figs 25.28 to 25.30) • It occurs when there is a stone blocking the cystic duct and the bile is not infected. • As a result of obstruction, all the bile within the gall bladder is absorbed and is replaced by the mucus secreted from gall bladder epithelium. • Clinically, it results in a soft, fluctuant, globular mass in the right hypochondrium which moves with respiration. It needs cholecystectomy.

561

EMPYEMA AND PERFORATION OF GALL BLADDER • These are uncommon. Impacted stone, diabetes, virulent organisms precipitate pyocoele and perforation. • Patients present with high grade fever with chills and rigors, toxicity, high leukocyte count. • Perforation can cause local abscess, if there are adhesions due to previous inflammation. • Perforation into the general peritoneal cavity is rare but produces diffuse biliary peritonitis which has a high mortality rate. • Urgent laparotomy, aggressive resuscitation, good antibiotic cover may help in reducing mortality. At laparotomy, removal of gall bladder is difficult. Hence, drainage of the pus and cholecystostomy with removal of gall stones can be done (Figs 25.3 lA and B). 3 Types (Fig. 25.32)

Fig. 25.28: Obstruction by stone

l . Localised perforation with pericholecystic abscess­ managed by catheter drainage.

• Fig. 25.29: Mucocoele

Fig. 25.31A: Empyema of

gall bladder

Fig. 25.30: Mucocoele

Fig. 25.31 B: Percutaneous chole­ cystostomy for empyema of gall bladder

Fig. 25.32: Empyema of gall bladder-complications (see the text

above)

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2. Free perforation into peritoneal cavity and peritonitis­ urgent laparotorny drainage. 3. Hollow viscous perforation (duodenum). CARCINOMA OF GALL BLADDER (page 589) Long-standing gall stones can bring about squamous metaplasia ofgall bladder epithelium and can cause carcinoma of gall bladder. However, the incidence is very low. Hence, routine cholecystectomy is not advised for silent gall stones. MIRIZZI SYNDROME (Figs 25.33 and 25.34) Type I:Compression ofCBD without lumen na1rnwing. Type II:Compression ofCBD with lumen narrowing. Type IJI:Compression causingCBD wall necrosis. Type IV: Stone ulcerating intoCBD resulting in cholecysto­ choledochal fistula.

TREATMENT OF GALL STONES ERCP (Fig. 25.34) followed by open cholecystectomy wai the most popular method till recently, now replaced b) laparoscopic cholecystectomy. Laparoscopic cholecystectomy It has become the most popular choice today. More than 95o/c ofgall bladders can be removed through a laparoscope. Some principles and procedure oflaparoscopic cholecystectomy are discussed below (Figs 25.35 and 25.36). Conversion to open when: • Very badly contracted, fibrosed gall bladder. • Very difficult gall bladder-Calot's triangle anatomy is not defined well. Dissection may cause injury to the bile ducts and can cause stricture. Partial cholecystectomy is a safe alternative (Fig. 25.37). Procedure • 1 cm incision is made below the umbilicus, through which a pneumoperitoneum is maintained byCO2 insufflation.

Retraction--"""--� '-.:../ of neck to RIF 112 cm Fundic traction---+-� 1/2 cm

•

1 cm

Camera port---1----�

Fig. 25.33: Mirizzi syndrome Fig. 25.35: Incisions for laparoscopic cholecystectomy

Fig. 25.34: ERCP showing Mirizzi syndrome

Fig. 25.36: Laparoscopic view of gall bladder

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• Following this, a laparoscope is introduced and a camera is attached. Three small, 1 cm incisions are made in epigastrium and 1/2 cm in the right hypochondrium. These are used for suction, instrumentation, cauterisation, dissection, retraction purpose-dissection port. • Cystic duct and cystic artery are clipped and gall bladder is removed using gall bladder-holding forceps and is brought outside through the epigastric port (Figs 25.38 to 25.40). • Bleeding from liver is controlled using lasers/cautery. • The procedure is done under general anaesthesia. It may take 1-3 hours depending upon the experience of the surgeon.

and all stones were cleared-asymptomatic since 3 years. It is a safe alternative to complete cholecystectomy. It is not a failure

Advantages 1. Hospital stay is 1-2 days, recovery is very fast. 2. Pain is minimal. Hence, mobilisation of the patient is much better and easy. 3. It gives an acceptable and better cosmetic result. 4. Complications such as adhesions and incisional hernia are rare.

Fig. 25.38A: Cystic artery and cystic duct are identified and

Fig. 25.388: Cystic artery is isolated

Fig. 25.39A: Cystic artery is being clipped

Fig. 25.398: Cystic artery is divided

Fig. 25.37: Partial cholecystectomy could be done in this patient

separated

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Fig. 25.40A: Calot's triangle is completely dissected

MEDICAL TREATMENT OF GALL STONES It is indicated for pure cholesterol stones only. Patient selection • Patients with functioning gall bladder proven by OCG or scintigraphy. • Young, thin, female patients. • Tiny(< 5 mm), translucent, floating stones Drawbacks • Recurrence of stones once treatment is stopped • Life-long maintenance is needed. • After dissolution of the stones, lithotripsy or extracorporeal shock wave Iithotripsy should be done. Types of medical treatment 1. Oral dissolution treatment • Drugs used: CDCA-chenodeoxycholic acid UDCA-ursodeoxycholic acid • Mechanism of action: They inhibit HMG-CoA, a rate limiting step in the synthesis of cholesterol, thereby increasing the bile salt pool. UDCA also acts by decreasing cholesterol absorption in GIT. 2. Direct contact dissolution MTBE-methyl terbutyl ether is the drug which is given through a catheter placed in gall bladder percutaneously. Drawbacks: Explosive and toxic if it enters bile duct or duodenum. Indications • High-risk patients with symptomatic stones, refusing surgery. • The patient must have patent cystic duct(proven by OCG/ scintigraphy). Side effects: Haemorrhage and catheter displacement.

Fig. 25.408: Gall bladder is mobilised from liver bed

Thus we have completed gall stones and their complications in the gall bladder. Now we will study the complication of gall stones in the common bile duct, mainly obstructive jaundice. OBSTRUCTIVE JAUNDICE (SURGICAL JAUNDICE) Definition • Jaundice that occurs due to obstruction to the outflow of bile is called obstructive jaundice. • Since these cases have to be managed by surgical intervention, it is also called surgical jaundice. • However, haemolytic jaundice cases are not obstructive but a few are managed by splenectomy(surgical). • Before we start a detailed discussion of obstructive jaundice, we will study anatomy of the pancreas. This is an important long case in the university examination. For a better understanding of obstructive jaundice, students are requested to understand various causes of obstructive jaundice which are given in the subsequent pages. SURGICAL ANATOMY OF THE PANCREAS The pancreas is both endocrine and exocrine organ situated retroperitoneally behind the stomach. It is a soft and fleshy gland(pancreas-all flesh), extending from the duodenum on the right side to the spleen on the left side, the entire length being 6 inches(Fig. 25.41). It weighs approximately 80 g. Parts • The head lies within the C-loop of duodenum. The uncinate process projects from the left inferior portion of the head over which course the superior mesenteric vessels. There are 5-6 small thin veins connecting this portion of the head with superior mesenteric veins. These veins have to be carefully divided during pancreaticoduodenectomy.

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• The head is drained by superior pancreaticoduodenal vein which drains into portal vein and inferior pancreatico­ duodenal vein drains into superior mesenteric vein. Islets of Langerhans (endocrine) • 75% : � Cells • 20% : a Cells • 4% : 8 Cells

Fig. 25.41: Blood supply and parts of pancreas: (1) Uncinate process, (2) Superior pancreaticoduodenal, (3) Coeliac artery, (4) Splenic artery, (5) Inferior pancreaticoduodenal, (6) Head of pancreas, (7) Body of pancreas, (8) Tail of pancreas and (9) Spleen

Superior mesenteric vein continues above as po1tal vein after joining the splenic vein. During pancreatico­ duodenectomy for periampullary carcinoma, infiltration into the portal vein should be ruled out before any major structure is divided. This is done by inserting a finger between the portal vein and head of pancreas, both from above and below. The neck is about 2 cm and is related posteriorly to superior mesenteric vessels. • Body and tail: The head and neck continue as body which is placed transversely. It slopes upwards across the aorta and ends as tail of the pancreas, which is enclosed within lienorenal ligament along with splenic vessels. A large cystadenoma arising from the tail of the pancreas can move with respiration because of its contact with the spleen. Blood supply of the pancreas Arterial supply (Fig. 25.41) • Splenic artery is the chief artery supplying the neck, body and the tail. Arteria pancreatica magna refers to one large branch of splenic artery. • Superior and inferior pancreaticoduodenal a1teries supply not only head of pancreas but also the adjacent duodenum. Thus, during any surgery which involves excision of the head, the C-loop of the duodenum is also removed. Thus, pancreaticoduodenectomy becomes a major surgery. Venous drainage • Body, neck and tail drain into splenic vein by means of multiple small veins.

Pancreatic duct (see page 608) • The main pancreatic duct (duct of Wirsung), a tubular structure drains entire pancreas from tail to the head. It joins the common bile duct and fonns ampulla of Yater. This ampulla opens on the duodenal papilla (a nipple-like elevation) in the 2nd pa1t of the duodenum. Normal diameter of pancreatic duct is 2-3 mm. When it is dilated more than 6-8 mm, as in chronic pancreatitis, longitudinal pancreatico­ jejunostomy can be done. • Accessory pancreatic duct of Santorini drains the uncinate process and lower portion of the head and opens into the duodenum 2 cm above the opening of the main duct. The two ducts communicate with each other at many sites. • The main pancreatic duct is lined by columnar epithelium which becomes cuboidal in the ductules. PANCREATIC JUICE • Bicarbonate rich, protein rich, alkaline fluid • 2.5 litres/day • Rich in proteins 15 g of protein/day • Thus acidic chyme in the duodenum is alkalinised by pancreatic juice • Protein is secreted by acinar cells, fluid and electrolytes by ductal cells • Secretions are stimulated by 3 phases a. Cephalic phase is mediated by acetylcholine-10% of secretion b. Gastric phase-mediated by gastrin and vagus-15% c. Intestinal phase-75% release mediated by release of secretion from duodenal acidification and stimulated release of bile into 2nd part of duodenum following entry of fat and proteins. A CASE OF OBSTRUCTIVE JAUNDICE Choledocholithiasis and carcinoma head of pancreas periampullary region are two important causes of obstructive jaundice. They constitute more than 90% of cases of obstructive jaundice treated surgically. Presence of jaundice, high-coloured urine, clay-coloured stools and itching gives the clue to the diagnosis. Palpable liver, palpable mass (cholangiocarcinoma, carcinoma head of pancreas) and palpable gall bladder (periampullary carcinoma) supports the diagnosis. Early diagnosis by imaging, endoscopy, ERCP, MRCP is possible. Since causes are many, they are treated

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accordingly. Relief of pain, relief of jaundice with or without resection (in malignancies) should be done early to prevent complications such as cholangitis, liver abscesses, septicaemia, renal failure and even death. AETIOLOGY (Fig. 25.42)

Klatskin's tumour/ lymph node mass

I. Causes in the lumen 1. Stones in the common bile duct 2. Ova, cysts, ascaris worms 3. Hydatid cyst of the biliary tree 4. Stone in the pancreatic duct/CBD junction Causes in the wall Periampullary carcinoma Bile duct stricture Stenosis of sphincter of Oddi (papillary stenosis) Klatskin's tumour-carcinoma of the bile duct where right and left ducts join 5. Choledochal cyst 6. Post laparoscopic cholecystectomy

�--- Choledochal cyst ....a---- Stricture CBD

II. 1. 2. 3. 4.

Ill. Causes from outside (due to pressure) 1. Carcinoma head of pancreas 2. Chronic pancreatitis 3. Lymph nodes at the porta hepatis obstructing the biliary tree.

.. .

6. 7. 8.

Fig. 25.42: Various causes of obstructive jaundice CLINICAL FEATURES First study to differentiate choledocholithiasis from carcinoma periampullary/head of pancreas (Table 25.1).

Differences between stone in the CBD and periampullary carcinoma (Ca)/Ca head of pancreas

1. Age 2. Sex 3. Duration of symptoms 4. Symptoms Pain

5.

Chronic ----'a.=""--, pancreatitis

Stone in the CBD

Carcinoma periampullary/head of pancreas

30-50 years More common in females Long duration

50-70 years Equally common in both sexes Short duration (1-3 months)

It is due to a stone blocking the CBD resulting in spasm of CBD. It is severe colicky pain like gall stone colic. As a result of obstruction, multiplication Fever of organisms results in fever. Occurs due to obstruction. Once inflammation Jaundice subsides all these 3 symptoms are relieved partly but they occur after sometime. Hence, intermittent pain, intermittent fever, intermittent jaundice are classical of a stone in CBD-Charcot's triad. Since the obstruction is never complete, Stools clay-coloured stools are not commonly seen. May be present but mild Pruritus Loss of appetite No Loss of weight No Signs Deep yellow Jaundice Absent Anaemia Liver can be enlarged due to back Per abdomen pressure. It is smooth, with round border, finn in consistency. As a rule, gall bladder is not palpable Gall bladder No Metastasis

There may be some discomfort in abdomen but colicky pain is not a feature. Pain is relatively rare in carcinoma head of the pancreas. When obstruction becomes severe, there is bile stasis. Cholangitis, fever with chills and rigors can occur. As a result of slow developing obstruction in periampullary region, jaundice is persistent, progressive, painless, pruritic. In 5% of cases, growth may ulcerate into the duodenum. It can cause melaena and jaundice may temporarily subside. Clay-coloured stools are common and when mixed with blood, it is called silvery stools or aluminium paint stools. Severe--due to bile salts in the circulation. Significant Significant Sometimes, greenish yellow It is usually present Liver can be enlarged due to back pressure. If it is nodular, with sharp border, hard in consistency, it is due to secondaries in the liver. Gall bladder is palpable in 70-75% cases. Left supraclavicular node, ascites, etc. may be seen.

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Courvoisier's law (Figs 25.43 and 25.44) • In a jaundiced patient, if the gall bladder is palpably enlarged, it is not due to stones (Key Box 25.10). In case of stones, previous inflammation would have made gall bladder fibrotic and hence, will not be palpable. • From clinical point of view, 90% of cases of obstructive jaundice are due to stones, periampullary carcinoma or carcinoma of the head of pancreas.

.-- - lntrahepatic dilatation of biliary radicals

............................. . KEY BOX 25.10

CLINICAL FEATURES OF GALL BLADDER MASS

• Egg-shaped mass/pyriform shape • Moves with respiration • Tensely cystic, feels firm, tender and sometimes located in the right hypochondrium • Superficially placed • Intra-abdominal, intraperitoneal Exceptions to Courvoisier's law 1. Double impaction: One stone in the CBD and one stone in the cystic duct. 2. Periampullary carcinoma in a patient who has undergone cholecystectomy. 3. Primary oriental cholangiohepatitis causing stones in the CBD (gall bladder is normal in these cases). CHOLEDOCHOLITHIASIS (Table 25.1)

Contracted gall bladder

Stones

Fig. 25.43: Contracted thick gall bladder due to stones. Hence,

not palpable

�--- lntrahepatic dilatation of biliary radicals

Types of CBD stones I. Primary stones: These stones are formed in the CBD or within intrahepatic ducts. They are multiple, pigment stones or often mixed stones (Key Box 25.11). Various causes are: 1. Infections of biliary tree and infestation-parasites such as clonorchiasis. 2. Congenital-Caroli's disease or choledochal cyst. 3. Biliary dyskinesia-defective pathophysiology of biliary tree. 4. Other causes-diabetes, malnutrition. II. Secondary stones: These stones originate from gall bladder and stay in CBD-usually supraduodenal portion­ then get enlarged to attain large size over a period of time. These stones can give rise to cholangitis. Cholangitis Bacterial infection of bile duct is called cholangitis.

N&MJ,-w',J CBD STONES

Distended---' gall bladder

Growth

Fig. 25.44: Enlarged palpable gall bladder in cases of

periampullary carcinoma

�

• Only 4-12% of all CBD stones • These stones do form primarily in common bile duct • Almost all primary CBD stones are pigment type (brown stones). • Associated with bile duct stasis and infection (bacteria) • Soft, easily crumble when manipulated • Associated with biliary stricture, papillary stenosis or sphincter of Oddi dysfunction. • Mechanism; Stasis-bacteria secrete bacterial glucuronidase which causes deconjugation of bilirubin diglucuronide. Bilirubin gets precipitated as calcium salt.

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Predominant organisms • E. coli, Klebsiella, Pseudomonas, Enterococci, Proteus. • Bacteroides and other anaerobes (C/ostridium perfringens) Important causes: Cholangitis. • Choledocholithiasis • Biliary stricture • •

•

Neoplasm Less important causes are pseudocyst of pancreas, chronic pancreatitis stenosing sphincter of Oddi, biliary parasitic infections, etc. Post ERCP, if stent cannot be passed as in obstructive jaundice cases.

Symptoms and signs • Biliary colic, jaundice and chills and rigors are called Charcot's triad. • Tenderness may be present in the upper abdomen. Investigations • Leukocytosis, high bilirubin levels and alkaline phosphatase levels are diagnostic tests. • Ultrasound, CT scans, ERCP are indicated to confirm/rule out the various causes. Treatment • Intravenous antibiotics • Emergency endoscopic sphincterotomy, extraction of stones in choledocholithiasis, endoscopic stoning in cases of stenosis or stricture. • Percutaneous transhepatic biliary drainage (PTBD) in high obstructions. • Laparotomy-drainage of CBD-T-tube insertion. Reynold's pentad of acute obstructive cholangitis Few cases of CBD stones present with serious problems of cholangitis described as Reynold's pentad 1. Persistent pain 2. Fever 3. Persistent jaundice 4. Shock 5. Altered mental status INVESTIGATIONS IN OBSTRUCTIVE JAUNDICE 1. Hb% is low in malignancy. 2. TC, DC are increased in case of infections. 3. BT, CT, PT are altered in case of obstructive jaundice. 4. Urine for urobilinogen is negative in obstructive jaundice.

5. Serum alkaline phosphatase: Normal value 60--300 units/1 More than 500 units is suggestive of obstructive jaundice • These are the enzymes which bring hydrolysis o phosphate esters in alkaline medium. • Sources of alkaline phosphatase include liver, biliar: tree, bone, intestine, kidney. • Excretion is mainly through biliary tree (Key Box 25.12)

............................. . KEY BOX 25.12

ALKALINE PHOSPHATASE

• It is the product of epithelial cells of cholangioles-i levels are due to increased enzyme production. • lntrahepatic cholestasis, cholangitis, extrahepatic obstruction are the chief factors causing elevation. • Focal lesions in the liver-single hepatic metastasis or liver abscess or a tumour can cause increased levels without jaundice. • In cholangitis, bilirubin may be normal but alkaline phosphatase may be very high.

• Gross elevation

Mild elevation

Obstructive jaundice, biliary Metastasis in the liver hepatic cirrhosis, bone disease abscess hepatitis

6. Abdominal ultrasound: It is the most useful, noninvasive, reliable and quick investigation for obstructive jaundice. Dilated biliary radicles, both intrahepatic and extrahepatic can be demonstrated (First clue in obstructive jaundice). • Stones can be diagnosed with their posterior acoustic shadow. • Mass lesion in the head region can be seen in cases of chronic pancreatitis or carcinoma head of the pancreas causing obstructive jaundice. • Ultrasound can detect multiple secondaries in the liver, thus, favouring the diagnosis of malignancy. Endosono­ gram can detect lymph nodes also. 7. CECT (contrast enhanced CT) scan: A head mass of even 2-3 cm in size and portal vein infiltration can be demonstrated by CT scan. Obliteration of fat plane between the mass and superior mesenteric vessels can be demons­ trated by CT scan which decides the operability of periampullary carcinoma or carcinoma head of the pancreas (Figs 25.45 to 25.48). • CT scan cannot differentiate head mass of carcinoma from chronic pancreatitis (PET scan may differentiate). • CT scan can also detect coeliac nodes, presence of which is a contraindication for radical resections (Key Box 25.13). • Take precautions against contrast induced nephro­ pathy.

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Fig. 25.45: CT scan showing hugely distended gall bladder and common bile duct-periampullary carcinoma. Growth was resectable. He underwent Whipple's operation

Fig. 25.46: Large 6 cm mass in the head of pancreas-patient not only had obstructive jaundice but also duodenal obstruction. He underwent triple bypass

Fig. 25.47: 6 cm mass abutting mesenteric vessels and involving root of the mesentery. Such masses are usually nonresectable

Fig. 25.48: Large head mass with obstructive jaundice with intrahepatic dilatation. When in doubt, diagnostic laparoscopy will help-to operate or palliate nonsurgically

..

...........................

KEY BOX 25.13

.

COMPLICATIONS OF PERCUTANEOUS CT-GUIDED BIOPSY OF HEAD MASS

Death Enzymatic autodigestion Abscess Traumatic fistula Haemorrhage Remember as DEATH

8. Endoscopy is useful to diagnose a periampullary carcinoma which may be seen as an ulcerative lesion in the second part of the duodenum. Biopsy can also be taken which shows adenocarcinoma (Fig. 25.49).

• In case of obstructive jaundice due to stones, smooth bulge can be seen in the second part of the duodenum. In carcinoma, ulcerated lesion can be seen. 9.Barium meal follow through to see the C-loop of duodenum (Fig. 25.50). • In periampullary carcinoma, there may be distortion of the medial border of the duodenum giving rise to Inverted 3 sign. • In carcinoma head of the pancreas there may be widening of C-loop of duodenum-Pad sign. 10. ERCP (endoscopic retrograde cholangio-pancreato­ graphy) (Figs 25.51 to 25.53) • With the help of a side viewing endoscope, ampulla of Yater is cannulated and a radio-opaque dye is injected. • It fills up the biliary and pancreatic system.

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Fig. 25.49: Endoscopy showing growth in the periampullary region. These lesions are usually resectable Fig. 25.51: ERCP showing stones in the CBD, stones are extracted and stent has been placed in the common bile duct

Fig. 25.50: Barium meal showing widening of C-loop of duodenum. It is not done nowadays

Interpretation

Fig. 25.52: ERCP showing stones in the CBD and contracted gall bladder

Uses If stones in the CBD are diagnosed, they can be treated in the following ways: • Extraction by using a basket. • Large stone can be crushed by using a lithotripter and can be extracted.

Fig. 25.53: ERCP showing stones in the CBD and contracted gall bladder

• Stones appear as filling defects in the CBD or in the common hepatic duct (CHD) (Figs 25.51 to 25.53), which may be mobile ( change position if patient is moved). • A periampullary carcinoma gives rise to an irregular filling defect or there may be total cut off in the flow of dye. • Chronic pancreatitis may show the dilated duct and stones in the pancreatic duct-'chain of lakes' appearance.

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• Sphincterotomy (incision of sphincter of Oddi) can be done to facilitate extrusion of small stones. • In patients with cholangitis with obstructive jaundice, stenting of common bile duct can be done to relieve obstruction. Stent removal is necessary at a later date. • In selected patients with biliary strictures, stent is placed after ERCP to relieve obstructive jaundice (sometimes permanent in malignancies). • ln selected patients with chronic pancreatitis, pancreatic duct can be stented to relieve pain. PEARLS OF

WISDOM

ERCP and stenting is NOT indicated in operable cases of carcinoma periampullary/head of pancreas unless cholangitis is present. Complications Severe infection of biliary tree (cholangitis) and acute pancreatitis can occur in 1-2% of the patients. Hence, prophylactic antibiotics are given before the procedure. 11. Endosonogram: Endoscopy aided ultrasound can detect missed stones in the CBD. It can also detect pancreatic head mass, lymph nodes. Endosonoguided FNAC can also be done (Fig. 25.54). I 2. Percutaneous transhepatic cholangiography (PTC): Using an ultrasound image-intensifier, a dilated biliary radicle is identified within the liver and a fine needle (Chiba needle 1 ) is introduced into it. The stylet is then removed and a radio-opaque dye injected. Chiba needle is 15 cm long and 0.7 mm in diameter (Figs 25.55A and B, Key Box 25.14).

Fig. 25.54: Endosonography showing pancreatic tumour with coelic lymph nodes (Courtesy: Dr Ganesh Pai, HOD, Medical Gastroen­ terology, KMC, Manipal)

Precautions • BT, CT, PT should be normal. Otherwise, vitamin K injection 10 mg is given IV or SC for 3 days. • If there is a bleeding tendency, this procedure should not be done. • Broad spectrum antibiotics are given before the procedure. Complications • Infection, cholangitis, septicaemia • Biliary leak can be significant producing abdominal pain and guarding. Hence, PTC should be done just prior to the surgery. • Haemorrhage

Needle injects contrast medium into the dilated right intrahepatic bile ducts

Dilated biliary ducts Klatskin's tumour in hepatic duct bifurcation

1Chiba: It is the name ofa university in Japan.

Figs 25.55A and B: (A) Diagrammatic representation of percutaneous transhepatic c holangiography (PTC); (B) Dye had failed to fill-up the bifurcation of the common hepatic duct suggesting the obstruction

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Fig. 25.55C: PTBD catheter inside CBD

............................. .

Fig. 25.56: MRC (magnetic resonance cholangiogram) showing high stricture-possibility of Klatskin's tumour (cholangiocarcinoma)

KEY BOX 25.14

FACTS ABOUT PTC 1. In the diagnosis of high strictures, PTC is better than ERCP. 2. It can also be used in ERCP failure cases. 3. In the diagnosis of Klatskin's tumour, PTC is extremely useful. It can also delineate the dilated proximal duct, which helps in planning for a biliary-enteric anastomosis. 4. Catheter can be kept in the bile ducts to provide external drainage as in strictures or in inoperable malignancies with obstructive jaundice (Fig. 25.55C).

13. MRI scan (MRCP) (Figs 25.56 and 25.57) • It is the investigation of choice in cases of obstructive jaundice or of high strictures and cholangiocarcinomas. • It is noninvasive and delineates the bile ducts very well so that a biliary bypass can also be planned. 14. Diagnostic laparoscopy • It is used in many GI malignancies • Tt can be aided by laparoscopic ultrasound • It can complement the staging by CT, MRI, etc. It can improve prediction of resectability to about 98% accuracy. • It is a simple but invasive investigation, requires 3 ports. • Specially excellent to detect peritoneal metastasis (which cannot be picked by other tests) which is the sign of inoperability. 15. CA 19-9: Often head mass can be due to carcinoma/ chronic pancreatitis. Biopsy is not mandatory. Clinical suspicion of a head mass may be treated with Whipple's pancreaticoduodenectomy. However, gross elevation of CA 19-9 (carbohydrate antigen), will suggest carcinoma. Figure 25.58 shows investigations and treatment of obstructive jaundice.

Fig. 25.57: MRC intrahepatic dilatation, another case of cholangio­ carcinoma-biopsy is not possible with MRI. Brush cytology is possible while doing ERCP

TREATMENT OF OBSTRUCTIVE JAUNDICE Preoperative preparation 1. Correction of fluid and electrolyte status and adequate hydration before surgery for 2-3 days is essential, especially when patients have vomiting or have developed sepsis. 2. Injection dopamine 2 µg/kg/min can be given to improve the urinary output (diuretic dose). 3. Injection vitamin K, 10 mg, subcutaneously or intra­ venously for 3 days is given to correct the prothrombin time. If prothrombin time is not corrected with this treat­ ment, fresh frozen plasma should be given. 4. Broad spectrum antibiotics are given before, during and after surgery. 5. Adequate blood transfusion to correct anaemia.

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OBSTRUCTIVE JAUNDICE 1------; • Direct bilirubin > 50% of total • ALP > 3 times rise • Urine bilirubin positive-urobilinogen absent USG abdomen (for cause and level of block) IHBR dilatation

Distended GB

GB not distended

GB wall is thick ± stone in GB

Level of block below insertion of cystic duct

Level of block above insertion of cystic duct

CBD stone Look for any mass lesion in head of pancreas or evidence of chronic pancreatitis

Klatskin's tumour

ERCP for confirmation, sphincterotomy and basketing

MRCP/CT

No If ERCP fails

l

• Endo USG for better depiction of lesion and advantage of taking biopsy • CT abdomen • Tumour marker CA 19-9

lap/open Choledocholithotomy + cholecystectomy

UGI endoscopy

Lesion in periampullary region Ca head of pancreas

Chronic pancreatitis

Biopsy

Operable

CT scan for operability

Triple bypass Whipple's pancreaticoduodenectomy

Resectable

Unresectable

1. Choledochectomy and HJ 2. Hepatectomy depending on involvement of portal veins (PVs) and hepatic artery (HA)

PTBD or Surgical bypass

• IHBR: lntrahepatic biliary radicles • HJ: Hepaticojejunostomy • Ca: Carcinoma • ERCP: Endoscopic retrograde cholangio­ pancreatography • CT to look for liver metastasis, lymph nodes, superior mesentric artery, portal vein or hepatic artery infiltration and extension into hepatic parenchyma • PTBD: Percutaneous transhepatic biliary drainage

Fig. 25.58: Investigations and treatment of obstructive jaundice ( Courtesy: Dr Ankur Sharma, Asst. Professor, Dept. of Surgery, KMC, Manipal)

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Treatment of CBD stones • Cholecystectomy is done first. This is followed by introduction of a cannula into the cystic duct and a radio­ opaque dye is injected. • This is called OTC (On Table Cholangiography). If the dye goes freely into the duodenum without filling defect, it is a normal OTC. If there are filling defects in the CBD, it is explored. • Precautions while doing OTC - There should not be any air bubble in the syringe. - 5-10 ml of dye has to be injected. - Leakage of the dye should not occur.

............................. . KEY BOX 25.15

1. 2. 3. 4. 5.

INDICATIONS FOR EXPLORATION OF CBD History of Charcot's triad Ultrasound proven stones in CBD CBD dilated more than 1 cm in size OTC shows filling defect Palpable stones in the CBD (open method)

Types of surgery for stones in the CBD There are three options available for treatment of stones in the CBD. Depending upon the merit of the case and experience of the surgeon any one of the methods can be selected. 1. Supraduodenal choledocholithotomy: Supraduodenal CBD is explored through an incision over the anterior wall and the stones are removed. Operating choledochoscope is passed into the common hepatic duct and its branches and the stones, if present, are removed. Closure of the CBD is done after inserting 'T-tube'. After 8-10 days, a T-tube cholangiography is done and if the dye goes freely into duodenum and no filling defect is seen in the CBD, the T­ tube is removed by gentle traction. By 10-12 days, the track is well formed. Hence, even if minor leak occurs, bile flows outside without causing peritonitis (Key Box 25.15, Figs 25.59A and B).

Fig. 25.59A: Cholecystectomy, CBD exploration followed by insertion of T-tube

Fig. 25.598: T-tube cholangiogram showing residual (retained) stone in the lower CBD-it was removed through endoscopic papillotomy and basketing

2. Cholecystectomy + choledocholithotomy + choledochoduo­ denostomy: It can be done when CBD is dilated more than 1.5 cm in diameter and stoma should be at least 2-3 cm in size (Key Box 25.16 and Fig. 25.60).

............................. . KEY BOX 25.16

INDICATIONS • Recurrent stones in the CBD • Multiple intrahepatic stones (Caroli's disease) • Stricture of the lower CBD

Fig. 25.60: Choledochoduodenostomy

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Advantages ofcholedochoduodenostomy (Key Box 25.16)

• Biliary leak is negligible • There is no worry even if there are retained stones in the CBD. • It is a permanent solution for stenosis, stricture or multiple intrahepatic stones. 3. Preoperative ERCP, sphincterotomy + extraction of stones followed by laparoscopic cholecystectomy This method has become the choice today. Expertise and sophisticated equipment are necessary for this (Fig. 25.61).

Shaded area resected

Fig. 25.62: Diagrammatic representation of Whipple's resection

Fig. 25.61: Stone was removed through endoscopic papillotomy

followed by basketing. There is a small risk of perforation and pancreatitis in these patients (Courtesy: Dr Ganesh Pai, Prof and HOD of Medical Gastroenterology, KMC, Manipal)

Choledocho­ jejunostomy

Natural history of CBD stones (Key Box 25.17)

............................. . KEY BOX 25.17

NATURAL HISTORY OF CBD STONES % of patients Asymptomatic 40% Cholangitis 20% Jaundice 20% Biliary colic 15% Pancreatitis 5%

Pancreatico- _,.,.........._..., jejunostomy

Symptom

• • • • •

Treatment of periampullary carcinoma I. Surgical treatment (Figs 25.62 to 25.69) I. Radical pancreaticoduodenectomy-'Whipple's operation' (Figs 25.62 and 25.63). • In this operation, the growth along with 'C' loop of duodenum up to DJ flexure, proximal jejunum, head of the pancreas up to the neck are removed and partial gastrectomy is done. This is followed by: • Pancreaticojejunal anastomosis (PJ), gastrojejunostomy (GJ) and choledochojejunostomy (CJ).

Fig. 25.63: Reconstruction following Whipple's operation

PEARLS OF WISDOM Preoperative tissue diagnosis is NOT necessary with appropriate clinical and imaging findings, specially in head masses.

• This is a major operation and carries 5-10% mortality due to pancreatic leakage or biliary leakage. • Whipple's operation is indicated in cases of mobile growth with no metastasis and where the general condition of the patients is reasonably good. 2. Pylorus-preserving pancreaticoduodenectomy (PPPD) In this operation, pylorus is preserved. Thus, gastric motility is not disturbed (Fig. 25.64).

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Pancreas

Fig. 25.64: Pylorus-preserving pancreatico­ duodenectomy (PPPD)

Fig. 25.659: Whipple's specimen (Courtesy: Dr Satyanarayana N, Associate Professor, Yenepoya Medical College, Mangalore)

Choledochojejunostomy

Fig. 25.66: Pancreaticogastrostomy (PG) is an alternative to PJ -----,,.-�----,,-.-- Carcinoma head of the

Jejunostomy

Fig. 25.67: Palliative triple bypass

'TUNNEL OF LOVE'

Fig. 25.68: Distended gall bladder ready for anastomosis

During resectional surgery for pancreatic head and uncinate process tumours (Whipple's pancreaticoduodenectomy), after a preliminary search for metastases, a tunnel (the so-called tunnel of love) needs to be developed behind the neck of the pancreas and anterior to the underlying visceral vessels before concluding that the lesion can be resected.

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Bismuth classification of postoperative stricture (Fig. 25.71) Type I: Low common bile duct; stump> 2 cm Type II: Middle common hepatic duct, stump < 2 cm Type III: Hilar-confluence of right and left duct intact Type IV: Right and left ducts separated Type V: Involvement of the intrahepatic ducts

I

Fig. 25.69: Palliative stenting for carcinoma head of the pancreas

3. Triple bypass: Cholecystojejunostomy + enteroentero­ stomy + gastrojejunostomy • This is a palliative surgery in which distended gall bladder is anastomosed to a long loop of jejunum (40 cm) to relieve jaundice. To prevent food particles entering into the gall bladder, enteroenterostomy is done (Fig. 25.61). • Most of the patients develop duodenal obstruction caused by the growth in the postoperative period. Hence, a palliative GJ is done at the same time. • In the absence of duodenal obstruction, if surgeon thinks that patient may live longer, beyond 6 months, GJ is indica­ ted (for a possible duodenal obstruction occurring later).

Too much traction on gall bladder­ CBD gets pulled and CBD appears like cystic duct and gets divided

Fig. 25.70: Laparoscopy bile duct injury Type I

Type II

II. Nonsurgical treatment Very elderly patients (age criteria not clear) who are not fit candidates for surgery and patients who have metastasis can be treated by palliative stenting. However, results of a surgical bypass is superior to stenting. Also, the stent needs to be changed frequently. OTHER CAUSES OF OBSTRUCTIVE JAUNDICE

Type V

STRICTURE OF THE CBD • 80% of strictures occur following surgery on the biliary tree. They are called postoperative strictures. 20% are due to inflammatory pathology. • lt gives rise to slowly progressive, painless jaundice. • Strictures account for 1-2% cases of obstructive jaundice.

Fig. 25.71: Classification of benign biliary stricture-Bismuth classification (see text)

Causes 1. Postoperative post-traumatic (Fig. 25.70) • Difficult cholecystectomy: When the gall bladder is fibrosed, densely stuck to the right hepatic duct or to the common bile duct or as in early cholecystectomy due to oedema around Calot's triangle, injury can occur to the right hepatic duct or to the CBD or CHO resulting in stricture.

2. Post-inflammatory Post-inflammatory strictures follow recurrent attacks of cholangitis due to: • Stones in the CBD or CHO • Parasites like Ascaris lumbricoides in the biliary tree or Asiatic cholangiohepatitis produced by Chinese liver fluke infestation ( Clonorchis sinensis) (page 591).

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• Primary sclerosing cholangitis wherein the cause is not known. PEARLS OF WISDOM

Difficult cholecystectomy, dangerous cholecystectomy and faulty dissection are the important factors for postoperative bile duct strictures. • Dangerous cholecystectomy: Sudden bleeding from cystic artery can occur due to traction on the gall bladder or due to lack of gentleness in ligating cystic artery. Sudden application of an artery forceps to control the bleeding may injure CBD. In such situations, packing the area, good suction and visualisation of the bleeding artery and ligation should be done. If the bleeding continues, the hepatic artery can be compressed between the finger and the thumb in the lesser omentum through the foramen of Winslow. This is called Hogarth-Pringle manoeuvre. • Dissection at fault: Ignorance of anomalies such as short cystic duct or too much traction on the gall bladder distorts CBD and predisposes to injury (Figs 25.72 and 25.73). • It is the duty of the surgeon to show his assistants the Y junction which is formed by the cystic duct, common hepatic duct above and common bile duct below before dividing any structures in this area.

Fig. 25.72: ERCP showing complete transection of bile duct­ type IV injury

3. Malignant strictures Malignant strictures are due to cholangiocarcinoma. Clinical features • History of cholecystectomy in the past with or without profuse discharge of bile in the postoperative period. • A slowly progressive, painless jaundice deepening day by day. • Hepatomegaly due to back pressure. • Recurrent cholangitis due to stasis of bile.

Fig. 25.73: CBD clipping-here, it was not transected. It was explored, clip removed and endoscopic stent was inserted

Investigations • USG-to rule out residual stones in CBD, to demonstrate intrahepatic dilatation. • ERCP or PTC may demonstrate a stricture in the CBD or CHD with proximal dilatation. • T-tube cholangiography, if T-tube is in place. • MRC is noninvasive and is better than PTC. Treatment • If it is due to laparoscopic clipping without transection of the CBD, it is better to re-explore and remove the clips and a T-tube or an endoscopic stent can be placed in the CBD (Fig. 25.74). • Late cases can be managed by choledochojejunostomy or hepaticojejunostomy by anastomosing a loop of jejunum to the dilated portion above the stricture. However, the general condition of the patient should be improved before surgery (Figs 25.75 to 25. 79).

Fig. 25.74: Same patient as in Fig. 25.73. You can see the stent in the common hepatic duct. It was removed after 6 months

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Fig. 25.75: Cut CHO-took one hour to reach this area, it is trimmed for biliary enteric anastomosis

Fig. 25.76: Roux jejuna! loop is prepared for hepaticojejunostomy

Figs 25.78 and 25.79: PTBD catheter was passed from above across strictured hepaticojejunostomy and dilatations were done in high strictures PEARLS OF

WISDOM

Prevention is better than treatment. Try to prevent bile duct injuries during laparoscopic cholecystectomy. SCLEROSING CHOLANGITIS It is characterised by development of multiple strictures and dilatation ofCBD with features of fibrous thickening ofCBD.

Fig. 25.77: Hepaticojejunostomy was done in another patient with type IV injury

Types • Primary No cause is found. However, it can be associated with following conditions (Key Box 25.18). • Secondary It is due to stones or injuries.

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IIMI • • • •

ASSOCIATED CONDITIONS

,_

�

Ulcerative colitis Crohn's disease Graves' disease Sjogren's syndrome

Complications Due to long-standing obstruction, biliary cirrhosis and cholangiocarcinoma can develop (Fig. 25.80). Diagnosis • Ultrasound can demonstrate intrahepatic dilatation. • MRCP is a noninvasive investigation which can demon­ strate multiple strictures and dilatation. • ERCP is the investigation of choice which can demonstrate the strictures in the CBD and dilatation which is described as having a beaded appearance. However, the risk of suppurative cholangitis is present. Treatment: It is difficult Stenting is the choice although stents may have to be replaced or changed if blockage occurs or if infection sets in.

Fig. 25.80: Sclerosing cholangitis

CHOLEDOCHAL CYST • It is a congenital cyst occurring in the CBD due to pa11ia: or complete weakness of the wall of the CBD. • Majority of cases manifest by 1-2 years of age.

Classification (Fig. 25.81 and Key Box 25.19)

Type I

Type Ill

Fig. 25.81: Types of choledochal cysts

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581

KEY BOX 25.19

TODANI CLASSIFICATION Types Type I: Fusiform dilatation of CBD-commonest Type II: Lateral saccular diverticulum of the CBD Type Ill: Dilatation of intraduodenal segment of CBD (choledochocoele) Type IV: Dilatation of CBD + intrahepatic biliary dilatation. Type V: Multiple intrahepatic cysts-Caroli's disease

Recent advances: Type VI-cystic dilatation of cystic duct

Clinical features • Age: Majority of cases manifest in children within 1-2 years of age. It can also present in adults. • More common in females 4: 1. • Abdominal distension can be due to a large cyst. The cyst can be palpated per abdomen in the right hypochondrium. • Slow progressive jaundice, recurrent attacks with abdominal pain and pyrexia. Investigations 1. USG will confirm presence of abnormal cyst. It is usually unilocular cyst. 2. MRC: It will define the relation between lower end of the bile duct and pancreatic duct to know basic anatomy (Figs 25.82 and 25.83). 3. CT is also useful to know intrahepatic and extrahepatic dilatation. 4. ERCP may be done but it will not give any more informa­ tion than MRC. Treatment • This anomaly is premalignant. Change to carcinoma is a well-recognised complication and it carries poor prognosis. Hence, excision of the cyst and reconstruction is the treatment of choice (Figs 25.84 to 25.87).

Fig. 25.82: MRC showing choledochal cyst

Fig. 25.83: Choledochal cyst-MR cholangiogram showing gross dilatation of the common bile duct and common hepatic duct

• Type I: Excision of the cyst followed by Roux-en-Y hepaticojejunostomy. • Type II: Excision of the diverticulum with suturing of CBD. • Type III: Endoscopic sphincterotomy is adequate ( choledochocoele ). • Type IV: They are difficult to treat. Due to recurrent cholangitis, if total excision is not possible due to adhesions between the cyst and portal vein, posterior wall of the cyst can be left behind, after removal of mucosa. This is described as Lilly's technique. Type V will be described later.

Complications l . Recurrent cholangitis with high-grade fever, resulting in biliary cirrhosis.

Fig. 25.84: ERCP showing choledochal cyst-type Ill treated by sphincterotomy

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CAROLl'S DISEASE • It is a hereditary condition wherein there is dilatation o intrahepatic ducts with stenotic segments in between. • Multiple, irregular saccular dilatations are characteristic. • It is also an example of type V choledochal cyst. Two types: 1. Simple: Presents later with abdominal pain and sepsis. 2. Periportal: It occurs in childhood, presents as recurren cholangitis. • Diagnosed by ultrasound and CT scan. MRI and ERCP arc other investigations (Fig. 25.88). • Associated lesions are given in Key Box 25.20.

Fig. 25.85: Choledochal cyst at surgery. It was excised completely and hepaticojejunostomy was done ( Courtesy: Dr Ramesh Rajan, Associate Professor, Surgical Gastroenterology, Trivandrum Medical College, Kerala)

Complications • Cholangitis: It occurs due to constant obstruction • Stones: Obstruction and stasis precipitate stone formation • Biliary cirrhosis • Cholangiocarcinoma Treatment • Hepatectomy (Fig. 25.89) • Liver transplantation

...

.......................... .

KEY BOX 25.20

ASSOCIATED LESIONS • Congenital hepatic fibrosis • Medullary sponge kidney • Polycystic liver

Fig. 25.86: Type I cyst

Excision and simple suturing

Fig. 25.88: Caroli's disease showing large cyst and intrahepatic dilatation Fig. 25.87: Type II-excision and suturing

CHRONIC PANCREATITIS

2. Rupture of the cyst resulting in biliary peritonitis. 3. CBD stones 4. Carcino ma in the cyst (25-30% of cases). It 1s a cholangiocarcinoma common in types I and V.

Definition Diffuse inflammatory process of pancreas involving head, body and the tail resulting in permanent structural and functional damage to the pancreas.

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Gall Bladder and Pancreas

Fig. 25.89: Left lobectomy with extraction of stone-Caroli's disease

(Courtesy:

Bangalore)

Dr Nagaraj Palankar, Consultant, Manipal Hospital,

Causes 1. Alcohol: High alcohol consumption is the most frequent cause. Alcohol stimulates pancreatic secretion rich in protein. This forms plugs in the pancreatic duct and results in stasis of secretion and stone formation. Alcohol also causes spasm of sphincter of Oddi. 2. Idiopathic: It is common in Kerala, and was thought to be due to consumption of tapioca (Fig. 25.90). It is also called Kerala pancreatitis or tropical pancreatitis. It is now thought to be due to malnutrition. It is also called fibrocalculous pancreatic diabetes. 3. Hereditary pancreatitis: It is a genetic disorder transmitted as a Mendelian-dominant trait. 4. Cystic fibrosis: Generalised dysfunction of exocrine glands cause secretions to precipitate in the lumen. 5. Hyperparathyroidism favours precipitation of calcium intraductally. It can also activate pancreatic enzymes. 6. Autoimmune pancreatitis: Diffuse enlargement of pancreas and narrowing of pancreatic duct is seen. Auto­ antibodies may be present. IgG4 is increased. Pathology There is destruction of pancreas by ductal sclerosis, ductal strictures, glandular fibrosis and calcification, both intraductal and parenchymal (Fig. 25.91 and Key Box 25.21). PATHOGENESIS Tapioca (cassava) Derivatives of cyanide Detoxified in liver (by sulphur-containing amino acids) If these enzymes are low or absent Cyanogen toxicity Chronic pancreatitis

Fig. 25.90: Aetiopathogenesis of cassava pancreatitis

Strictures--�

Fig. 25.91: Chronic pancreatitis showing pathological changes

............................. . KEY BOX 25.21

• • • • • •

CHRONIC PANCREATITIS IDIOPATHIC TYPE Common in warm climates (Kerala) Common in young age High incidence of diabetes High incidence of stones in the duct Increased chances of parenchymal calcification Increased chances of pancreatic cancer

Clinical features (you can remember as MOPED) • Malabsorption occurs due to damage to exocrine glands resulting in steatorrhoea-10 to 15 stools per day, bulky, frothy, rich in fat, foul-smelling. Malabsorption indicates late disease and results in weight loss. Creatorrhoea refers to excessive loss of protein (Figs 25.92 and 25.93). • Obstructive jaundice can occur due to oedema of the head of pancreas. Later, fibrous constriction of CBD due to fibrotic indurated mass in the head region can cause jaundice by compressing the CBD. • Pain abdomen-upper abdominal pain radiating to the back in the region of L 1 and L2 due to retroperitoneal inflammation (Key Box 25.22). Pain may be severe, sometimes radiating to both right and left sides. The pain

..

........................... .

• • • • •

Parenchymal hypertension Perineural inflammation Ductal hypertension Pseudocyst formation Stenosis of bile duct

KEY BOX 25.22

PAIN IN CHRONIC PANCREATITIS

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Manipal Manual of Surgery

is due to multiple strictures in the pancreatic due increasing the intraductal pressure. It is relieved 01 stooping forward. • Exploratory laparotomy-many cases are diagnosed a laparotomy where iITegularity and hardness involving tht entire pancreas are seen. Exploration is done for evaluatior of obstructive jaundice or for chronic abdominal pain. • Diabetes-incidence of diabetes is about 10-20%. It shoulc be suspected in diabetic patients with pain abdomen. Investigations • Plain X-ray abdomen can demonstrate stones in the pancreatic duct or parenchymal calcification (Fig. 25.94). • USG can detect the stones, stricture, dilatation and associated cysts. • ERCP (Fig. 25.95) - Ductal distension, ductal stricture - Dilated pancreatic duct (diameter of the normal duct is 4-6 mm) - Demonstration of stones-appear as regular filling defect. • CT scan: It can reveal ductal anatomy, head mass, size and configuration of pancreas (Figs 25.96 and 25.97). Corn pl ications • Obstructive jaundice due to a mass lesion in the head region • Carcinoma of pancreas • Pseudocysts • Steroids in autoimmune pancreatitis

Figs 25.92 and 25.93: This 21-year-old girl was weighing 32 kg

in 2006 when she underwent longitudinal pancreatico­ jejunostomy (LPJ) for failed medical treatment including stents. After 3 years, she has put on 22 kg weight

Fig. 25.94: Plain X-ray abdomen showing extensive calcification

Treatment I. Conservative • Pain relief by analgesics, epidural analgesia, or splanchnic nerve block. Slow release opioid skin patches are useful. • Supplement pancreatic enzyme-diet should be low in fat and vitamin D supplements should be given.

Fig. 25.95: ERCP showing dilated pancreatic duct-'chain of lakes' appearance

Gall Bladder and Pancreas

Fig. 25.96: CT scan showing chronic pancreatitis with head mass. This case should be treated with head-coring operation

585

Fig. 25.97: CT shows dilated pancreatic duct of 1 O mm in size with stones. ERCP is not required in such patients. This patient underwent LPJ

• Pancreatic enzymes not only help in treating exocrine insufficiency but they also denature CCK releasing peptide, thereby diminishing the release of CCK. Thus, pain is decreased. Six capsules ofpancreatin are given. • Control diabetes, stop alcohol consumption and tobacco smoking, antioxidants may help. II. I. 2. 3.

Surgery: Indications Unrelieved pain Suspicion of carcinoma Complications • Ascites • Cysts • Abscesses • GI bleeding-left-sided, portal hypertension, pseudo­ aneurysm • Obstructive jaundice • Duodenal obstruction

Types of surgery (Figs 25.98 to 25.107) I. Chronic pancreatitis involving tail of pancreas • Distal pancreatectomy with removal of spleen. 2. Diffuse chronic pancreatitis with dilated (large duct) pancreatic duct • Duct is laid open widely, strictures are cut open, stones are removed and it is anastomosed to a loop of jejunum1 ongitudinal pancreaticoj ej unostomy-Puestow's operation (Roux-en-Y jejuna! segment). The duct should be at least 8 mm in diameter. Sutures hold very nicely because of fibrosis of pancreas. Pain relief is obtained in about 80% of the cases. Pancreatic fistula is a complication of this surgery. Majority of the fistulae close spontaneously. • This is a bypass procedure which preserve endocrine and exocrine functions.

Fig. 25.98: Distal pancreatectomy. Chronic pancreatitis confined to tail of pancreas

Fig. 25.99: Pancreaticojejunal anastomosis is being done

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Manipal Manual of Surgery

Fig. 25.100: Dilated duct is being aspirated to identify the duct followed by laying it open

Fig. 25.101: Diagrammatic representation of LPJ

Superior mesenteric vein

�-- Pancreatic duct

Fig. 25.102: The dark shaded area is called pacemaker of chronic pancreatitis. It should be removed in cases of head­ coring operation. It is a triangle between lower CBD, pancreatic duct and superior mesenteric vessels

Fig. 25.103: Head-coring is completed. You can watch thin rim of pancreatic tissue all around

Fig. 25.104: Specimen of the portion of the head which was excised-Frey's procedure ( Courtesy: Dr Girish MS, MCh, Surgical Gastroenterologist, KMC, Manipal, 2007-2008)

Fig. 25.105: Specimen of the portion of the head along with multiple stones

Gall Bladder and Pancreas

4.

5. 6. Fig. 25.106: Head-coring gives rise to bleeding which can be

7.

controlled with cautery (Key Box 25.23). Pain relief is the main aim of surgery of chronic pancreatitis and a new carcinoma developing. Both these problems are dealt with in this operation

587

Hence, pancreaticoduodenectomy is advised, provided experience of the surgeon is good and the mortality rate is less than 5%. Chronic pancreatitis with bile duct obstruction • If malignancy is ruled out, a bypass procedure is the treatment of choice. - Choledochojejunostomy is the ideal treatment. - Pancreaticoduodenal resection (Whipple) can also be done (as mentioned above). Chronic pancreatitis with duodenal obstruction Here also, resection of the head mass or gastrojejunostomy is the treatment of choice. Chronic pancreatitis with ascites: Treatment of choice is Puestow's operation (stenting may also relieve ascites). Resection: A duodenum-preserving pancreatic head resection is called Hans Beger's Procedure. Head-coring procedure is called Frey procedure (Figs 25.103 to 25.106 and Key Box 25.23). CHOLANGIOCARCINOMA-BILE DUCT CARCINOMA

• Patients having folJowing diseases have increased risk of developing cholangiocarcinoma (Key Box 25.24).

............................. . KEY BOX 25.24

RISK FACTORS

Cholangitis-primary sclerosing Colitis-ulcerative Clonorchiasis Choledochal cyst Caroli's disease Chemical carcinogens like thorium, nitrosamines, diosmin

Fig. 25.107: Bilateral subcostal roof top incision gives adequate exposure to all pancreatic surgery

3. Chronic pancreatitis with a head mass In this situation, doubt arises whether it is malignancy or not. Even trucut biopsy and frozen section are not foolproof.

............................. . KEY BOX 25.23

HEAD-CORING-FREY PROCEDURE

• Pathologic key to recurrent pancreatitis is localised to pancreatic head. It is called pacemaker. • It is located in the crucial triangle (see Fig. 25.102) which has to be excised. • Pain and recurrent pancreatitis will decrease after Frey procedure. • Surgery can be done with minimum blood Joss. • Try avoiding injury to lower CBD-if necessary, CBD can be opened and a sound can be introduced.

Observe 6 Cs

• • • •

It is an uncommon cause of obstructive jaundice. Elderly males > 60 years are commonly affected. Obstructive jaundice is the presenting feature. Tender hepatomegaly is present due to congestion and may be due to cholangitis. • Gall bladder is not palpable in cases of cholangio­ carcinoma unlike in periampullary carcinomas. • Cholangiocarcinoma at the bifurcation of the hepatic duct is called Klatskin's tumour. Types 1. Perihilar is the most common, Klatskin's tumour comes under this. 2. Intrahepatic (10%) 3. Distal (CHD-CBD) 25%

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Management • Abdominal ultrasound, CT scan, ERCP will help in loca­ lising the site of obstruction (Figs 25.108 and 25.109). ERCP and stenting the CBD is indicated in inoperable cases. However, MRI is better. • Treatment is difficult because most of the lesions are high up in the hilar region infiltrating the liver, portal vein, etc. Resection and hepaticojejunostomy or endoscopic stent placement are the treatment modalities available. • Resection involves liver for hilar growth and for lower end growth.

CONGENITAL BILIARY ATRESIA Aetiopathogenesis A disease of unknown aetiology, though rare, is fatal. Vira aetiology and defective embryogenesis have been blamed fo the development of biliary atresia. Types (Fig. 25.11 O) • Type I: Common bile duct is involved

• Type II: Common hepatic duct is involved • Type III: Atresia of right and left hepatic ducts.

Clinical features • It presents as jaundice at birth or in the neonatal period.

• Due to absence of bile in the gut, meconium is not bile­ stained. Hence, stools are pale. • Gradually, due to backpressure, liver enlarges.

• Enlargement of the spleen may follow if there is develop­ ment of portal hypertension. • Steatorrhoea, pruritus and clubbing are the other features. Treatment Surgical drainage of bile is the only available treatment provided patent bile duct or radicle is seen. Anastomosis of Roux-en-Y loop of jejunum to the dilated bile duct or sometimes excision of bile duct tissue up to the liver capsule should be done followed by Roux-en-Y anastomosis. This is called Kasai's portoenterostomy.

Figs 25.108: ERCP showing cholangiocarcinoma

• Liver transplantation is the choice when there is atresia of the intrahepatic duct. Complications 1. Recurrent cholangitis giving rise to hepatic fibrosis 2. Biliary cirrhosis and portal hypertension

Figs 25.109: CT scan showing extensive cholagiocarcinoma with

ascites

Fig. 25.110: Congenital biliary atresia-types I, II and Ill

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Gall Bladder and Pancreas

CARCINOMA OF THE GALL BLADDER Incidence: It is common in North Eastern India. Occurs in elderly patients in their sixties/seventies. Aetiology • Gall stones 80-90% of gall bladder cancers are associated with gall stones. Calcification of gall bladder is associated with carcinoma gall bladder. • Chemicals High incidence of gall bladder and biliary cancer is noted in people who work in rubber industries. • Gall bladder polyp (Fig. 25.111) • Dietary Adulterated mustard oil for cooking is found to precipitate carcinoma gall bladder. Pathology • 85% of cases. It is usually adenocarcinoma. Undifferen­ tiated carcinoma and squamous cell carcinoma may also occur. The tumour is most commonly nodular, infiltrates the entire gall bladder and adjacent tissues. • 3 types of adenocarcinomas have been identified: nonpapi­ llary adenocarcinoma, papillary adenocarcinoma and mucinous adenocarcinoma. Majority are nonpapillary adenocarcinoma. Clinical features • Significant weight loss,jaundice and mass in the right upper quadrant are common presentations. Clinically, it is palpable as a hard irregular mass. • A few cases can present as chronic cholecystitis with a mass or acute cholecystitis. Ultrasonography may diagnose as stones and they are subjected to cholecystectomy. During surgery or postoperatively, the diagnosis gets confirmed.

Fig. 25.112: Advanced case of carcinoma gall bladder with ascites and liver infiltration

• Obstructive jaundice, bleeding, ascites are late features. Investigations (Table 25.2 for staging) • CA 19-9 is elevated in 80% patients. • Ultrasonography and endosonography are very useful investigations. Ultrasonography-guided FNAC can be done for histological diagnosis in suspected cases of gall bladder mass. • CT scan is useful for staging-lymph nodes metastasis in the liver (Fig. 25.112). • ERCP if there is obstructive jaundice to localise the exact site and nature of obstruction. • Diagnostic laparoscopy. If peritoneal metastasis is present, it is not worth resecting. • MRCP can be done. It visualises bile duct better than CT scan. Treatment • [f gall bladder cancer is found at cholecystectomy and if mucosa alone is involved, then cholecystectomy is sufficient.

............................. . KEY BOX 25.25

GALL BLADDER CANCER IS THE WORST, WHY?

Fig. 25.111: You can see extensive carcinomatous polypoid lesions inside the gall bladder

• Biologically very aggressive cancer (unlike basal cell cancer, carcinoma colon, etc.) • High incidence of lymphatic spread • Easily spreads into liver by direct infiltration • Spreads by blood, neural, intraperitoneal routes • lntraductal extension into CBD causes obstructive jaundice • Infiltration into stomach, colon, duodenum and liver can occur because of its location • Radiation and chemotherapy is rarely of any benefit

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Manipal Manual of Surgery

TNM staging Tumour Tis - carcinoma in situ Tl - spread to mucosa or muscle layer Tia - only mucosa] involvement T3 - spread > 2 cm to liver or 2 or more adjacent organs - CBD, stomach, duodenum, colon, omentum Stage I: Tl NO MO (up to muscle) Stage II: T2 NO MO (up to serosa) Stage Ill: T3 NO - beyond serosa, liver< 2 cm, I adjacent organ 1/2/3 NJ-hepatoduodenal ligament Stage IV: T4 N0/1/MO, N2 M l

Nodal spread NO- no nodes NI - spread to cystic/nodes in portal area N2 - spread to parapancreatic/coeliac/superior mesenteric nodes Metastasis MO - no metastasis Ml - distant spread is present Simple cholecystectomy Extended cholecystectomy Different procedures

• If gall bladder wall is involved, then extended cholecystec­ tomy is done (Key Box 25.25 and Fig. 25.113). • Radiation has very small benefits. • Chemotherapy also has been tried. 5-FU, mitomycin C, doxorubicin are the drugs used.

.

............................ .

KEY BOX 25.26

EXTENDED CHOLECYSTECTOMY

I

Cholecystectomy

I Open theIspecimen I

I

I Growth penetrating the wall I

I

Segmental wedge excision of liver

I

I

Regional lymphadenectomy (lymph nodes in and around CBD, duodenum, pancreaticoduodenal regions)

Fig. 25.113: Extended lymphadenectomy

causes needs some concentration power and practice (Key Box 25.26). Parasitic infestations causing obstructive jaundice (Table 25.3)

The usual blunder committed is doing cholecystectomy for a diagnosis of stone when it is actually a case of carcinoma gall bladder-inadequate surgery results in biliary fistula and hastens death.

Prognosis In general, five-year survival is very poor (Key Box 25.26). Aggressive surgery and complete clearance give best result (Fig. 25.113). Thus, we have discussed various causes of obstructive jaundice. Students should be able to differentiate between all these different causes and to offer an appropriate diagnosis in the examination. Each of these short topics can be asked as short notes in the theory paper. Understanding of all these

Fig. 25.114: Ascariasis of the biliary duct. (Courtesy: Dr C.G. Narasimhan, Surgeon, Mysore, Karnataka)

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591

Parasitic infestation of the biliary tract BiJiary ascariasis 1. Causative organism Ascaris lumbricoides (Fig. 25.114)

Clonorchiasis

Biliary hydatid

Clonorchis sinensis

Echinococcus granulosus Rupture of hydatid cyst of liver into biliary tree

2. Route of entry

Ampulla of Yater

Ampulla of Yater

3. Complications

Stricture, suppurative cholangitis, liver abscess, empyema

Biliary pain, stone cholangitis, block, Obstructive jaundice, cholangitis cirrhosis, bile duct carcinogenic

4. Management

• Ultrasound, CT scan

• Ultrasound, CT scan

• Ultrasound

• ERCP-worm can be extracted • Open surgery • Deworming

• Cholecystectomy and T-tube drainage • Choledochojejunostomy for a better drainage

• ERCP, sphincterotomy, stent

4. Cystadenocarcinomas are rare, slow-growing and tend to attain a large size.

CARCINOMA OF PANCREAS Introduction • 70% of the cases occur in the head of the pancreas including periampullary region. • 30% occur in the body and the tail. • 70% of cases are adenocarcinoma of duct cell origin. • It is 4th leading cause of death due to cancer in males, after lung, colon, prostate. AETIOLOGY 1. Chronic tropical pancreatitis and hereditary pancreatitis are associated with pancreatic cancer-such malignancies can be multifocal. 2. Haemochromatosis produces extensive calcification of pancreas. It is also a precancerous condition. 3. Diabetes: Diabetic patients are 10 times more vulnerable to develop carcinoma of pancreas. 4. Other possible aetiological factors: • Alcohol and smoking: It is related to tobacco specific nitrosamines (including smokeless tobacco). • Westernisation of diet: Fatty food, rich in animal proteins can cause pancreatic cancer. • Industrial carcinogens: B 11-naphthylamine, benzidine, gasoline are the possible agents. PATHOLOGY • Periampullary refers to carcinoma arising from ampulla of Yater, the duodenal mucosa or the lower end of the common bile duct. • Microscopically, the types are: 1. Mucus secreting carcinoma of ductal origin. 2. Nonmucus secreting carcinoma of acinar origin. 3. Anaplastic carcinomas are poorly differentiated and tend to arise from the body of the pancreas.

• Appropriate surgery

CLINICAL FEATURES • Periampullary and carcinoma head of pancreas present as obstructive jaundice (discussed already). Discussion here is on carcinoma body and tail of pancreas. • Microscopically, the tumour shows malignant cells with a variable degree of differentiation, mitotic figures, etc. Not only it invades vessels and lymphatics, but also perineural spread occurs fast which explains backache (Key Box 25.27).

............................. . KEY BOX 25.27

• • • • •

CARCINOMA OF PANCREAS-PATHOLOGY�

More than 80% are ductal adenocarcinomas. Solid and scirrhous tumours by nature. Plenty of neoplastic tubular glands are seen. Desmoplastic reaction is extensive (feels hard). Infiltrates locally-spreads along nerve sheaths, lymphatics and into blood vessels. • Being retroperitoneal in location, manifests late, spreads fast and carries poor prognosis.

Symptoms • Severe pain radiating to the back in the region of Ll and L2. It is due to infiltration of retroperitoneal nerve plexuses or pancreatic duct obstruction. • Pain is so severe, often patient requests for narcotic analgesics. • Gross weight loss in 3-6 months. • Anorexia, asthenia and generalised weakness. • Jaundice cannot occur in carcinoma body and tail of pancreas unless there are secondaries in liver or lymph nodes at porta hepatis. • Symptoms of vomiting suggest duodenal obstruction.

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• Trousseau's sign 1 (thrombophlebitis migrans) - Migrating thrombophlebitis of the legs can occur in visceral malignancies particularly from carcinoma of pancreas, rarely carcinoma stomach, colon, etc. - It is supposedly due to sluggish blood flow resulting in thrombus formation. - It is superficial and affects the leg veins such as long saphenous vein. PEARLS OF WISDOM

Sudden development of diabetes mellitus is an early manifestation in 25% ofpatients. Signs • Anaemia may be present as in any other malignancy. • Jaundice is not a feature. Left supraclavicular node may be palpable. • Per abdomen findings (Table 25.4): Majority of these cases are advanced, fixed and are felt as a mass in the upper abdomen. I. Characteristics of a pancreatic mass • It is situated on the left side involving left hypochon­ drium, umbilical region and epigastrium. I t does not move with respiration because it is retroperitoneal. • It does not fall forwards in knee elbow position. • Can get above the swelling. • On percussion, it gives a resonant note because of anterior position of stomach. II. Features of carcinoma • It is common in elderly, male patients. • Hard, irregular, fixed lump.

III. Evidence of metastasis Secondaries in the liver, ascites, rectovesical deposits DIFFERENTIAL DIAGNOSIS 1. Carcinoma stomach infiltrating the pancreas • Such mass may not be mobile. It does not move witl respiration because it is fixed to pancreas. • These patients will have vomiting first followed b) backache at a later date. 2. Carcinoma transverse colon • Produces constipation and bleeding per rectum. Vertica mobility may be present. • Right to left peristalsis may be present. 3. Para-aortic lymph node mass may be due to: • Intra-abdominal malignancies, lymphoma, testicula1 tumour, etc. INVESTIGATIONS I.Investigations for periampullary carcinoma and carcinoma of head of pancreas (discussed before). II.Investigations for carcinoma of body and tail I. USG: First investigation of choice • Rules out calculous obstruction • Can detect a mass as small as 2 cm • Intraoperative ultrasound can be used to take a biopsy. 2. Contrast-enhanced CT scan (Figs 25.115 and 25.116) • Retroperitoneal invasion • Lymph node enlargement • Invasion of hepatic artery, superior mesenteric artery, ascites, liver metastasis-unresectable. • Infiltration of portal vein which makes it inoperable • In more than 70% of cases pancreatic duct is dilated and in more than 60% of cases bile duct is dilated. • When both these ducts are dilated, it is most likely pancreatic cancer. • CT-guided aspiration biopsy is indicated for lesions to start neoadjuvant chemotherapy/RT (infiltrative lesions).

Differences between pancreatic mass and stomach mass

1

Carcinoma body of the pancreas

Carcinoma stomach

1. Pain radiating to the back is the presenting feature 2. Haematemesis is not a feature 3. The mass does not move with respiration 4. Mobility is not a feature 5. Knee elbow test-the mass does not fall forwards 6. Retroperitoneal organ 7. Percussion: Resonant note is obtained because of stomach/intestine anterior to it

Vomiting, dyspepsia is present. Pain is not a feature Haematemesis is a common feature Usually moves with respiration Mobility is present Stomach mass falls forward (intraperitoneal), unless it is fixed Intraperitoneal Impaired note because of growth in the stomach

Trousseau's sign is also found in Buerger's disease

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593

4. Carbohydrate antigen CA 19-9: These are glycoproteins which are elaborated by malignancies. It is a tumour marker of pancreas to monitor carcinoma pancreas. Increased in 75% of patients with carcinoma pancreas and l 0% of the patients in benign disease of pancreas, liver and bile ducts. Hence, it is not a diagnostic investigation. However, very high values suggest malignancy even when histology is inconclusive.

Fig. 25.115: Carcinoma body of the pancreas with para-aortic nodes and ascites

TREATMENT I. Periampullary carcinoma-Whipple's operation II. Carcinoma of body and tail A. If the tumour is very small and diagnosed very early, they are ideally treated with total pancreatectomy with removal of involved lymph nodes. B. Many cases are diagnosed late. They are inoperable either due to fixity to portal vein or due to metastasis. Hence, there is no role for curative surgery. For the confinnation of diagnosis, transabdominal USG-guided fine needle aspiration cytology can be done. Surgery is not indicated in such cases; only palliative treatment can be offered. Prognosis is very poor. • Palliative radiotherapy: 4000-6000 cGy units can be given. Response rate is 5-10%. It reduces size of the tumour and some pain relief is obtained (Table 25.5). • Palliative treatment is an important aspect of carcinoma pancreas even though such patients succumb to the disease within 3 to 6 months. Palliation is mainly to relieve pain. TRUE CYSTIC PANCREATIC NEOPLASM (CPN)

Fig. 25.116: Large mass in the head and body of pancreas

3. ERCP is indicated in the absence of a mass. Findings can be stenosis/obstruction.

• These are uncommon tumours present predominantly in women (Key Box 25.28). • Other rare tumours are cystadenocarcinoma, acinar cell cystadenocarcinoma, etc. • These tumours can be confused with pseudocyst of the pancreas. Hence, they should be differentiated at surgery (Figs 25.117 to 25.120). Otherwise, gross blunders can take place (Table 25.6). • Serous and mucinous are important. They are discussed in Table 25.7.

Palliation for carcinoma pancreas-3 chief symptoms Pain

Jaundice

Duodenal obstruction

• Sustained release oral narcotic drugs-morphine • Stenting-recurrent obstruction and cholangitis • Occurs in 20% patients chances are decreased sulfate Posterior dependent GJ is pre­ ferable-indicated in cases of • Coeliac plexus nerve block (pain is due to invasion • Choledochojejunostomy, cholecystojejunostomy (if cystic duct enters CBD well above the symptoms such as vomiting ofretroperitoneal nerve trunks) growth) • lntraoperative coeliac plexus block by injecting 50% • Surgical bypass has increased mortality and alcohol directly into tissues along side ofaorta morbidity initially • Palliative RT and gemcitabine-survival improved by l -2 months

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594

f§ijffii

Differences between cystic neoplasm and pseudocyst Cystic neoplasm

Pseudocyst

I.

History of pancreatitis

Absent

Present

2.

Imaging

May demonstrate solid component

Usually absent

3.

ERCP

Mucin egress from bulging papilla in cases of IPMTs

No

4.

Mucin content of fluid

Present

Absent

5.

CEA in fluid

Increased in mucinous cystic neoplasms

Absent

6.

CA 19-9

Increased in malignant cystic neoplasms

Not elevated

Epithelial lining can be present

No epithelial lining

7. Percutaneous wall biopsy

Cystic neoplasms Serous

Mucinous

• Gender

• No prediction

• More common in women

• Nature

• Multiple cysts are present

• More often multilocular

• Cut surface

• Microcystic adenomas with cut surface

• Macrocystic adenomas with smooth lining with papillary

appearance of a sponge

projections

• CT scan

• CT may show cysts with calcification

• Septa! on CT scan are characteristic

• Nature of fluid

• Cystic spaces contain serous fluid

• Cystic spaces contain mucus

• Lining epithelium

• Lining is by cuboidal epithelium

• Lining is tall columnar and Goblet cells

• Malignant potential

• No malignant potential

• Most of them turn into cyst adenocarcinoma

............................. . KEY BOX 25.28

TRUE CYSTIC PANCREATIC NEOPLASMS (CPNs) A. Serous Cystadenoma: Benign • Common in women, located in head of pancreas. They account for 30% of all CPNs. They can be observed if asymptomatic. B. • • • • • •

Mucinous Cystic Neoplasms (MCNs) More common in women More often found in body and tail More incidence than serous (40%) Considered premalignant Do not communicate with ductal system Lesions more than 2 cm need to be resected

C. lntraductal Papillary Mucinous Tumours (IPMTs) • Slightly more common in males • Communicates with duct • Incidence is about 25% • High malignant potential • More common in head involving ampulla of Vater • Treated by Whipple's resection

Fig. 25.117: Cystadenoma of the pancreas

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595

Fig. 25.118: Ultrasound showing cystic mass in the body of pancreas

Fig. 25.120: Cystadenoma of the pancreas resected specimen

INSULINOMA (f3-CELL TUMOUR)

Fig. 25.119: Cystadenoma of the pancreas at surgery

ENDOCRINE TUMOURS OF THE PANCREAS These tumours are members of APUDOMA arising from APUD (Amine Precursor Uptake Decarboxylation) cells. They are neuroectodermal in origin. Accordingly, following tumours can occur (Table 25.8). Types • Sporadic: Usually occurs as a single tumour. • Familial: Occur with other adenomas as in multiple endocrine neoplasia syndrome-MEN TYPE I: Pituitary, parathyroid, pancreatic adenoma.

• Most common functioning islet cell tumour. • Majority of the insulinomas occur in the tail and body of pancreas and majority of them are benign, one-third are malignant and one-third are multiple. • It can be a single adenoma, can be due to diffuse hyperplasia or due to carcinoma. • Clinical features are that of hypoglycaemia (Key Box 25.29 for other causes of hypoglycaemia). • In early stages, it can mimic a duodenal ulcer. Due to hypoglycaemia, hunger pain develops and a tendency to ask for food is present. • As it becomes severe, giddiness, dizziness, syncopal attacks, blurring of vision and diplopia can occur. • Late stages-epilepsy, semiconsciousness and coma.

............................. . KEY BOX 25.29

• • • • • •

PERSISTENT HYPOGLYCAEMIA-CAUSES lnsulinoma Hepatoma Hepatocellular damage Hypopituitarism Addison's disease Large mesenchymal tumour

Summary of islet cells lslet cells

Active agent

Syndrome

Presentation

Alpha Beta Delta G

Glucagon Insulin Somatostatin Gastrin

G lucagonoma lnsulinoma Somatostatinoma Gastrinoma

Hyperglycaemia Hypoglycaemia Hyperacidity

Manipal Manual of Surgery

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Whipple's triad of insulinoma 1. Attack of hypoglycaemia in morning hours, in the fasting state. 2. Symptoms are relieved on taking glucose. 3. Blood sugar in the fasting state is less than 45 mg% during the attack.

The author remembers a case of recurrent epilepsy getting admitted to the department of neurology. He was found to have hypoglycaemia and was diagnosed as insulinoma only during the 4th admission. A 3 cm benign insulinoma was enucleated from the pancreas. The patient is asymptomatic till date.

Investigations I. Serum insulin levels done by immunoassay method are found to be very high. Proinsulin levels and C-peptide level are also very high. 2. Persistent hypoglycaemia-blood sugar level of less than 50 mg/di relieved by glucose is suggestive of insulinoma. 3. USG and CT scan ofabdomen can demonstrate the tumour ifit is more than 2 cm (intraoperative ultrasound is a useful test). 4. Selective angiography will demonstrate tumour blush as majority of them are very vascular. Treatment • Enucleation is the treatment of choice. • Resection of the tumour and if necessary distal pancrea­ tectomy can also be done in selected patients. • Diazoxide is given to suppress insulin release. • For inoperable islet cell carcinoma, streptozocin is the best chemotherapeutic agents.

GASTRINOMA (ZOLLINGER-ELLISON SYNDROME Two types are recognised • Type I (rare): These have antral G cell hyperplasia, wherei gastrin is stored. Hypergastrinaemia with chronic pepti ulceration is a feature. Pancreas is normal. • Type II: May be due to an ulcerogenic, non B-islet eel tumom or sometimes diffuse hyperplasia of the islet cell1 Tumour secretes gastrin and is usually found in the tail o the pancreas. 50% of them are malignant. • Intractable peptic ulceration, hypergastrinaemia witl massive acid hypersecretion ofup to 500 ml/hr can occw • Diarrhoea, steatorrhoea and hypokalaemia occur due t1 acid irritating the small bowel activity (Key Box 25.30)

............................. KEY BOX 25.30

GASTRINOMA SHOULD BE SUSPECTED IN FOLLOWING SITUATIONS 1. Unusual ulcer: Peptic ulcer not responding to intensive

medical treatment.

2. Unusual recurrence: Multiple recurrences in spite of the treatment . 3. Unusual number: Multiple ulcers scattered in the GIT. 4. Unusual sites: Ulcers present in the 2nd part of duodenum, an ulcer just distal to the ligament of Treitz. 5. Unusual age: Sudden development of an ulcer in a young boy or in a very elderly patient. Site It commonly occurs in the gastrinoma triangle also called Passaro's triangle. Following three points form Passaro's triangle. 1. Junction of the cystic duct with the common bile duct (Fig. 25.121).

lnsulinoma-interesting most

• • • • • • • • • • •

The most common functioning islet cell tumour of pancreas. Mostly are single (75%) Mostly mutiple in 'MEN' syndromes Mostly benign (70%) Mostly occur in tail and body of pancreas. Mostly present as bizarre behaviour confusing type due to hypoglycaemia. Most useful diagnostic test-very high (disproportional) levels of insulin during fasting hypoglycaemia. The most important test to localise the tumour before surgery is CT/MRI. The most important test to localise at surgery is intra­ operative ultrasound. Mostly they can be enucleated when occur in the head. Most insulinomas are small, less than 2 cm

Fig. 25.121: Passaro's triangle of gastrinoma ( Courtesy: Dr. Vidushi, Undergraduate student, KMC, Manipal)

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Gall Bladder and Pancreas

2. Junction between head and neck of pancreas. 3. Junction between second and third parts of the duodenum. Diagnosis

1. Endoscopy will show prominent mucosa] folds and large amount of acid in the stomach. 2. Serum gastrin is increased above normal levels (normal value < 150 ng/dl). 3. CT scan and arteriography may localise the tumour. Treatment

1. Type I: Partial gastrectomy to remove G cells bearing area. 2. Type II: If the tumour is small, removal of the tumour can be done (enucleation). 3. If the tumour is large, omeprazole 20 mg twice a day or somatostatin derivatives have been used to control acidity. Total gastrectomy is the last res011, if gastrinoma is not found. GLUCAGONOMA

• It arises from alpha cells producing glucagon. Around 90% are in the body and tail. • Clinically, it presents with extensive necrolytic migratory erythematous rashes. Rashes occur due to low amino acid levels due to neoglucogenesis brought about by glucagon. Rashes involve legs and perineum. • Age range is 20-70 years • This is common in women • Mild diabetes, weight loss, diarrhoea are the other features. • Glucagon levels are increased in serum diagnostic. • Localisation is by elective arteriography and CT scan • Treatment is enucleation

• • • •

Acute pancreafitis drinks like afish (produces dehydration). f Acute pancreatitis eats like a wo! (pancreatic necrosis). Acute pancreatitis burrows like a rodent (producesfistula). Acute pancreatitis kills like a leopard (life-threatening).

Marseille's classification of pancreatitis

I . Acute pancreatitis 2. Acute relapsing pancreatitis • In both these conditions, pancreas returns back to normal. 3. Chronic pancreatitis 4. Chronic relapsing pancreatitis • In both these conditions there is always permanent damage to the pancreas. AETIOLOGY (Figs 25.122 to 25.124)

1. Alcohol abuse (40 to 50%): It is the major cause of acute pancreatitis in our country and is seen in about 50% of the cases. Alcohol stimulates pancreatic secretions rich in protein, forms protein plugs and results in obstruction to the pancreatic duct. Alcohol stimulates trypsinogen. It causes spasm of sphincter of Oddi. It also has direct toxic effect on the pancreas. Tobacco smoking contributes to its effects. 2. Biliary tract disease: Stone in the biliary tree (gall stone pancreatitis) is the major cause of acute pancreatitis in the Western world (40%). In our country, it may be responsible for pancreatitis in about 20-30% of patients.

Mechanism of pancreatitis

Treatment principles of glucagonoma

• Correction of malnutrition by TPN (total parenteral nutrition). • Treatment with somatostatin analogues • Enucleation is easy if it is in the head • Considerable palliation even if subtotal removal is achieved. • Low dose heparin-before and after surgery-because of high risk of DVT-pulmonary embolism. • Streptozocin and dacarbazine are the most effective chemo­ therapeutic agents. Thus, endocrine tumours of the pancreas are rare but when they occur, they can present with varying clinical manifesta­ tions which confuse a clinician. A high index of suspicion is necessary. They may be benign but can be fatal if left untreated. ACUTE PANCREATITIS

Definition: It is defined as acute nonbacterial inflammatory condition caused by activation, interstitial liberation and auto­ digestion of pancreas presenting as acute abdominal pain. • Acute pancreatitis stings like a scorpion (produces severe pain).

Injury to acinar cells

Premature activation of pancreatic enzymes within pancreas

Local • Pancreatic oedema­ mild • Severe haemorrhage • Severe necrosis

Systemic • Haemodynamic instability • Bacteraemia due to translocation of gut flora •ARDS • GI haemorrhage, renal failure • Disseminated intravascular coagulation (DIC}

Fig. 25.122: Pathogenesis of tissue damage (see text)

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Pancreatic divisum Autoimmune

Hyperparathyroidism Viral

Multiple gall stones

Fig. 25.123: A few causes of acute pancreatitis

7.

8. 9. Gall stones in---tl"--'11 the duodenum and one stone blocking the sphincter of Oddi

10. 11.

Fig. 25.124: Diagramm atic representation of gall ston e pancreatitis

3. Collagen vascular disorders: Autoimmune disease such as polyarteritis nodosa can be a causative factor in acute pancreatitis. 4. Drugs: Corticosteroids, tetracycline, oestrogens, azathio­ prine, valproic acid and diuretics can cause pancreatitis. 5. Endoscopic procedures: Sphincterotomy, cannulation of CBD or pancreatic duct, or basketing of stones from CBD can precipitate acute pancreatitis by duct disruption and enzyme extravasation. 6. Familial or genetic factors have been blamed for acute pancreatitis. Hereditary pancreatitis may be due to

12.

13. • • • • •

mutation of cationic trypsinogen gene. Symptoms begir in early childhood. Hyperparathyroidism causing hypercalcaemia ma) stimulate pancreatic juices and can cause pancreatitis. l also facilitates precipitation of calcium in the ducts. Hyperlipidaemia (> 1000 mg/di of triglycerides) can alsc cause pancreatitis. Dietary control can cure pancreatitis. Hypothermia and hypotension can cause ischaemia to the pancreas resulting in acute pancreatitis. Injury to the pancreas either postoperative or following penetrating injury can result in pancreatitis. Infection: Virus such as Mumps and Coxsackie can cause pancreatic. Scorpion sting can also cause pancreatitis abnormality. Ductal: Around 20%. Some of them are due to pancreatic divisum seen during ERCP. The opening of the minor papilla is inadequate for drainage of pancreatic juice. Postoperative pancreatitis It follows operations on CBD-open or laparoscopic. More common after T-tube insertions (now it is less). Sphincteroplasty, ERCP, stone extractions Pancreatic biopsy Gastrectomy (distal/total) Cardiac surgery. Here risk factors are perioperative administration of calcium, postoperative hypotension, pre­ operative renal failure.

Gall Bladder and Pancreas

14. Idiopathic: It is seen in about 15% of cases. Even though, classified as idiopathic (no cause is found), they are found to have sludge/gall stones undetected by ultrasound examination (Key Box 25.31).

3.

............................. . KEY BOX 25.31

• • • •

4.

QUICK REFERENCE TO COMMON CAUSES OF PANCREATITIS

Pancreatitis in teens-suspect hereditary or APBDJ 1 Pancreatitis in women-suspect gall stones Pancreatitis in males-alcoholic pancreatitis Pancreatitis with fleeting joint pains-autoimmune pancreatitis. • Pancreatitis with bony lesions/cysts-hyperparat hyroidism • Pancreatitis with fever-viral 1 Abnormal pancreatico-biliary duct junction

Pathogenesis (Fig. 25.125) • Autodigestion is the final common pathway leading to pancreatitis, a few theories have been explained as possible factors for autogenesis. 1. Obstruction/secretion: Recurrent attacks of pancreatitis as in chronic cases can be due to multiple strictures, stasis, resulting in autodigestion. 2. Common channel theory: In about 90% of cases, bile duct and pancreatic duct converge into ampulla of Yater. However, only in 10% of cases they have common channel.

5.

• • •

599

Reflux of bile into pancreas has been blamed for 'gall stone pancreatitis'. However, strong evidence is lacking. Duodenal reflux: Activation of enzymes takes place in the duodenum through action of enterokinase. Hence, it is postulated that duodenal reflux is one of the causes of acute pancreatitis. Back diffusion: Back diffusion of pancreatic enzymes through the ductal epithelium is a possible factor, when damaged by alcohol/bile acids, etc. Systemic sepsis: Organ failure - ARDS, renal failure - Endotoxins originate from bacteria. Acute pancreatitis is an autodigestion following activation of trypsinogen. This is brought about by various agents mentioned above. It may also be due to reflux of bile into the pancreas. Trypsinogen is converted into trypsin. It acts and stimulates:

1. Lipase: Lipase splits the fat into fatty acids and glycerol. Fatty acids combine with calciwn to form calcium soap. This is represented as fat necrosis seen in the omentum, subsynovial pockets of knee joint, etc. This also explains hypocalcaemia and tetany seen in acute pancreatitis. 2. Elastase: It digests the elastic fibres of the blood vessels resulting in rupture and haemorrhage into the peritoneal cavity.

Trypsin Acts and stimulates

Lysolecithinase

Digests elastic fibres of blood vessels

Fatty acid + Glycerol

Membrane damage

Phospholipase A2

Prostaglandins bradykinins

Pseudoaneurysm Diffuse inflammation

Haemorrhage Fat necrosis

Necrotising pancreatitis

Acute hypovolaemic shock

Death ---------, Infected necrosis 1------1 Renal failure

Fig. 25.125: Activation of trypsinogen to trypsin-pathogenesis of pancreatitis

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Fig. 25.126: Fat necrosis in the omentum Fig. 25.127: Abdominal distension is due to paralytic ileus in thE early stages. It can also be due to pancreatic ascites

Fig. 25.129: Grey Turner's sign-discolouration in the flanks

Fig. 25.128: Cullen's sign-discolouration around the umbilicus

3. Lysolecithinase: This is derived from the bile. It produces extensive tissue necrosis resulting in destruction of pancreas. 4. Prostaglandins, bradykinins, kallikrein, etc.: These are the inflammatory mediators. They produce profound hypotension, shock and collapse, due to loss of fluid in the retroperitoneum (TII space loss). 5. Extensive necrosis of pancreas: It produces MDF (myocardial depressant factor) which depresses ventricular contraction resulting in cardiac failure. Ultimate result is the development of multi-organ failure.

Clinical features (Figs 25.126 to 25.129) Symptoms • Severe upper abdominal epigastric pain radiating to the back increases over a period of hours-illimitable agony is a characteristic feature. It is partially relieved on stooping and bending fo1wards (Mohammedan Prayer sign). A meal or alcohol triggers the pain. • Vomiting-frequent and effortless due to reflex pyloro­ spasm. • Fever-low grade • Haematemesis and rnelaena can occur due to necrosis of duodenum. It is a poor prognostic sign. Signs • Febrile, tachypnoeic patient in agony. • Cyanosis-irnproper perfusion of lungs. • Faint jaundice due to oedema of the head of the pancreas. • Features of shock-feeble pulse, tachycardia, hypotension, cold extremities.

Gall Bladder and Pancreas

• Abdominal findings - Tenderness in epigastrium - Upper abdominal guarding and rigidity - Distension of the abdomen - Mass in epigastrium - Muscle guarding - Abdominal distension due to either accumulation of blood or fluid in the peritoneal cavity or due to paralytic ileus. • Cullen's sign (Fig. 25.128) - Bluish ecchymotic discolouration seen around umbilicus (first described for ruptured ectopic pregnancy). • Grey Turner's sign (Fig. 25.129) • Bluish discolouration in the flanks • Both these signs are due to peri-pancreatic and retroperitoneal haemorrhage and seepage of blood along fascia! planes, into the anterior abdominal wall and spread through falciform ligament. • Evidence of respiratory signs: Tachypnoea, dullness, effusion, crepitations, rhonchi (pulmonary oedema, ARDS). Investigations 1. Haemogram (CBP): Hb% may be low due to haemorrhagic pancreatitis. • Total count is raised above 15,000 cells/mm 3 due to inflammation. 2. Blood for urea, creatinine to rule out renal failure 3. Serum amylase (widely used test) (Key Box 25.32). • Normal levels are 40-80 Somogyi units • Values around 400 are suggestive and values more than 1000 Somogyi units are diagnostic of acute pancreatitis. • It is increased in the first 24-48 hours and returns to normal within 3-4 days (Key Box 25.32). • Persistent high level of amylase in acute pancreatitis indicates: - Unresolving inflammation - Recurrent attacks of pancreatitis - Complications-pseudocyst, pancreatic abscess - Serum lipase levels-more specific but difficult to measure. Lipase is only secreted by pancreas.

60

4. Blood and urine sugar estimation: Glycosuria is present in almost 100% of patients. PEARLS OF WISDOM Lipase levels tend to be higher in alcoholic pancreatitis and amylase levels are higher in gall stone pancreatitis.

5. Serum calcium levels: Hypocalcaemia is seen, due to hypoalbuminaemia or fat necrosis. 6. Total proteins are usually low, especially albumin. 7. Plain X-ray abdomen ( erect position) (Fig. 25.130) • Calcification suggests chronic pancreatitis. • To rule out perforation of peptic ulcer. • Sentinel loop sign-one dilated jejuna! loop of intestine which is seen in the region of pancreas. • Colon 'cut-off' sign refers to mild distension of transverse colon with collapsed descending colon. 8. Abdominal ultrasound-can demonstrate oedematous pancreas, fluid in the abdomen or biliary tract disease. 9. Contrast enhanced CT scan of abdomen is done after 3-5 days in patients who fail to respond to conservative treatment. IfCT scan demonstrates infected necrosis, an urgentCT-guided FNAC is done and Gram stain is sent. If Gram stain is positive, it has to be treated urgently (Key Box 25.33, Figs 25.131 and 25.132). Indications for CT scan in acute pancreatitis • Patients with severe pancreatitis • When the diagnosis is in doubt • Patients with organ failure • Patients with sepsis • Localised complications such as pseudocyst, pseudo­ aneurysms. • Clinical deterioration

............................. . KEY BOX 25.32

• • • • • • •

INCREASED AMYLASE LEVELS ARE SEEN IN� Acute pancreatitis and its complications Parotitis Afferent loop obstruction Spasm of sphincter of Oddi Biliary peritonitis-duodenal injuries Mesenteric infarction Ruptured ectopic gestation

Fig. 25.130: Plain X-ray abdomen erect showing dilated jejunal loop due to paralytic ileus-sentinel loop sign

.

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............................ .

KEY BOX 25.33

CT A B C D E

BALTHAZAR CT SCAN SCORING SYSTEM OF ACUTE PANCREATITIS Points Grade 0 Normal 1 Oedematous 2 Mild extrapancreatic collection 3 Severe extrapancreatic collection 4 Extensive/multiple extrapancreatic collection

Fig. 25.132: CT scan showing infected necrosis

PEARLS OF WISDOM

Early aggressive fluid replacement is the key in the management of acute pancreatitis.

Fig. 25.131: Contrast enhanced CT (CECT) scan is the gold standard investigation in cases of acute pancreatitis. Useful after 7 days of acute pancreatitis. This photograph shows extensive necrosis. CT scan also helps in detecting pseudocysts, abscesses, presence of gall stones or any other unsuspected pathology in the abdomen. Modern thinking is to wait for 4-6 weeks for separation of necrosis before surgical necrosectomy

Management • Almost always conservative. However, a few scoring systems have been followed (Table 25.9).

• Even though management principles of any acute abdomer includes 'nil per oral' so as to give rest to the part, in casei of pancreatitis it has been divided broadly into 3 categories It is desirable to know the treatment followed fo1 these categories. It has been given in Table 25.10 and Key Box 25.34. • Those of you who find it difficult can remember at least few fundamentals of managing acute pancreatitis. They are given in the fonn of simple headlines: ABCDEF A: Aspiration with Ryle's tube, to give rest to the pancreas (may be for one or two days). B: Blood transfusion if Hb% is low, or albumin and amino acids if proteins are low. C: Charts-increasing pulse, increasing temperature indicates pancreatic abscess which needs laparotomy and drainage.

Prognostic indicators/severity predictors Ranson score ON ADMISSION Age> 55 years WBC count> 16 x J09/L Blood glucose> IO mmol/L LOH> 700 units/L AST> 250 Sigma Frankel unit/unit WITHIN 48 HOURS Increase in blood urea nitrogen levels> 5 mg% Drop in haematocrit> I 0% Arterial oxygen saturation (Pa02 < 60 mmHg) Serum calcium < 2 mmol/L Base deficit> 4 mmol/L Fluid sequestration> 6 L

Glasgow Scale Age> 55 years WBC count> 15 x 109/L Blood glucose > IO mmol/L Serum urea> 16 mmol/L Pa02 < 60 mm Hg Serum calcium < 2 mmol/L Serum albumin < 32 g/L LOH> 600 units/L AST/ALT> 600 units/L

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Gall Bladder and Pancreas Management principles of acute pancreatitis Acute oedematous (Mild-80%)

Necrotising pancreatitis (Sterile necrosis-10%)

Infected necrosis (Very severe---5%)

Admission

Acute ward with monitoring of vitals Intensive care unit

Intensive care unit

IV fluids/ hypotension

Early correction of hypotension, hypovolaemia-crystalloids

IV fluids and inotropic* support may be required

May require inotropes and vasopressors for a long period

Blood transfusion

Rarely required

May be required

Definitely required

Antibiotics

No antibiotics

Oral/nutrition

Oral fluids, soft diet by 3-4 days, once pain and ileus settle down

Early antibiotic prophylaxis is required Broad spectrum antibiotics-imipenem, ciprotloxacin, metronidazole, etc. If pain is still present even after 4 days, Enteral feeding/nasojejunal feeding. nasojejunal feeding to be done If calories are not sufficient, total parenteral nutrition is required

Hypomagnesaemia/ hypocalcaemia

Usually will not be a problem

Correction is required

Correction is required

Oxygen

By nasal cannula/face mask may be required

Early ARDS-ventilatory support

Ventilatory support may be required

Role of surgery/ natural course

Majority of patients will not require surgery. If gall stones are impacted at sphincter of Oddi, endoscopic basketing and laparoscopic cholecystectomy should be done

By I 0-15 days, may resolve completely Ideal time to operate is after 4 weeks or may develop into pseudocyst or when necrosis is demarcated well infected necrosis whjch require surgery

* Dopamine 2-5 µg/kg/min can help in renal perfusion. 5-10 µg/kg/min acts as an inotrope and l 0 20 - µg/kg/min as a vasopressor. Noradrenaline 50-200 ng/kg/min as a vasopressor.

D: Drugs: Prophylactic antibiotics-used in cases of severe pancreatitis for prevention of local or general complication. IV cefuroxime or imipenem or ciprofloxacin with metronidazole are given. Low molecular weight dextran (lomodex) 500 ml can be used to increase renal perfusion. Alternately, dopamine 2 µg/kg/min can be given IV which helps in renal perfusion (in case of oliguria).

............................. . KEY BOX 25.34

• • • • •

INDICATIONS FOR SURGERY IN ACUTE PANCREATITIS Infected necrosis Pancreatic abscess Diagnosis is in doubt-perforated viscus cannot be ruled out. Complications such as massive bleeding not responding to conservative treatment. Cholangitis not responding to treatment.

E: Exploratory laparotomy, only when diagnosis is in doubt, when patient is not improving or when there is a complication of pancreatitis such as pancreatic abscess, fistula or necrosis. With the advent of wonderful imaging techniques, surgical intervention is rarely being done in the initial phase of acute pancreatitis. In early cases, pancreas should not be handled. Peritoneal lavage is done followed

by insertion of tube drain. Lavage has shown some benefit. In cases of infected necrosis, necrosectomy is done. • The wound can be left open as laparotomy or with mesh or with zip. F: Fluid should be given early. Rapid infusion of3--4 litres of Ringer lactate is used to treat hypovolaemic shock. Plasma or albumin may also be given. • Factors predicting severe pancreatitis (Key Box 25.35).

MtMJ,-w',1 IDENTIFICATION OF SEVERE PANCREATITIS • Age : > 70 years • Obesity

: BMI > 30 kg/m2

• Pleural effusion

: Present

• CT necrosis

: > 50

• CT severity index

: CTSI

• Persistent

: Organ failure

PEARLS OF

�

WISDOM

In cases of infected necrosis proved by CT-guided FNAC and Gram stain, necrosectomy should be done.

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PANCREATIC NECROSECTOMY (Figs 25.133 to 25.139) • Ideally done 4-6 weeks later when sepsis is still present. • CT-guided FNAC/bacterial culture is a must. • A midline laparotomy is done • Lesser sac is entered • Thorough debridement of the dead tissue is done. • All fluid and tissue should be sent for aerobic and anaerobic culture. • Blunt dissection rather than sharp dissection is done to minimise bleeding. • Antibiotics of choice should be carbapenems and quino­ lones. • Ileostomy should be done for retrocolic necrosis

• Cholecystectomy should be done for gall stone pancreatiti cases. • Perfect haemostasis should be achieved usmg manua compression, sutures and ligatures. • In cases of early necrosectomy, lesser sac is packed reoperation done after 48 hours-zipper closure of th1 abdomen is used. • Continuous lavage of the lesser sac and retroperitoneum i: done. • Other alternate methods are closed packing. • Today all these cases are done through minimally invasivt methods. • Endoscopic necrosectomy, retroperitoneoscopic necrosec­ tomy is also done.

Fig. 25.133: Acute necrotising pancreatitis. Patient had high temperature. Nasojejunal tube feeding is being done

Fig. 25.134: Acute necrotising pancreatitis with massive upper GI bleeding. Patient had high temperature, angiogram revealed pseudoaneurysm. He underwent therapeutic embolisation followed by necrosectomy

Fig. 25.135: Acute necrotising pancreatitis with massive upper GI bleeding. Patient had haematemesis of one litre of blood with clots

Fig. 25.136: You can see necrosectomy being done. It was done through lesser sac. Significant blood loss was present. Fingers are used to separate necrosis and it is removed with a spoon

Gall Bladder and Pancreas

605

Fig. 25.139: After necrosectomy, it is better to perform zip laparotomy so that it can be opened any number of times for peritoneal toilet ( Courtesy: Dr BH Ananda Rao, Prof and Head, Department of Surgery, Dr Siddhartha Bhandary and Dr Prashanth Shetty, Associate Professors, KMC, Manipal) Fig. 25.137: After necrosectomy, pancreatic bed is washed with saline and tubes or sump drains are put so as to be able to irrigate in the postoperative period

Treatment of gall stone pancreatitis (Key Box 25.36) If gall stone is obstructing the ampulla of Yater and causing pancreatitis, endoscopic sphincterotomy may be done within days. It has shown some benefit to the patients. After 2-3 weeks, once the jaundice subsides, cholecystectomy is recommended.

............................. KEY BOX 25.36

.

ACUTE PANCREATITIS • Lipase is a more reliable test because it is more pancreas­ specific and remains elevated for long after onset of symptom. • However, amylase is widely used • It is not necessary to demonstrate CBD stone to prove the diagnosis of gall stone pancreatitis. Often stone has passed into duodenum.

COMPLICATIONS OF AClJTE PANCREATlllS (Table 25.11) Systemic complications 1. Shock • Hypovolaemia and hypoperfusion are the major factors responsible for renal failure. Due to collection of large

Fig. 25.138: Necrosectomy specimen

Complications of acute pancreatitis Local

General

• • • • • • • • •

• • • • • •

Pancreatic necrosis (sterile) Pancreatic necrosis (infected) Pancreatic abscess Pancreatic ascites Pancreatic pseudocyst Peritoneal fluid collection Pleural effusion Pseudoaneurysm Partial/splenic vein thrombosis

Pulmonary-ARDS Cardiac-shock, arrhythmias Renal-renal failure Gastro-ileus, intestinal colonic necrosis Metabolic-hypercalcaemia, hypoglycaemia, hyperlipidaemia Haematological

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amount of fluid in the third space-peritoneal cavity, pleural cavity and extravascular space, shock occurs. Fluid replacement with blood or albumin should be done at appropriate time to treat the shock. • Electrolyte abnormalities should be corrected. 2. Respiratory insufficiency: Factors responsible for this are given in Key Box 25.37.

ll$D!J�,, W RESPIRATORY FAILURE • Abdominal distension and elevation of diaphragm. • lntravascular coagulation in the lung. • Lecithin present in the pulmonary surfactant is altered due to lecithinase resulting in defective capillary alveolar exchange. • Defective ventilation caused due to pain. • Left-sided pleural effusion not responding to treatment.

Measurement of arterial blood gas values and administration of oxygen is enough in the initial stages. In late stages, pulmonary insufficiency needs to be treated with ventilatory support. 3. Hypocalcaemia needs to be treated with calcium IV. It is due to hypoalbuminaemia and due to calcium soap. 4. Pleural effusion is treated by pleural tap (ultrasound­ guided), if it is symptomatic. 5. ARDS, MODS: Some mediators such as phosphatase damage alveolar membrane of lungs causing 'ARDS'. It manifests as respiratory failure.

• It has to be drained by CT-guided aspiration. • Laparoscopy may be necessary not only for the diagnosi but also as a therapeutic means for removal of necroti, pancreas. • Otherwise, open drainage of the abscess is required. 2. Pseudocyst of pancreas (vide infra) • This complication is encountered after 2nd week followin! an attack of acute pancreatitis. • It is seen in about 20% of the patients. 3. Perforation of colon or stomach 4. Pseudoaneurysm resulting in massive upper gastro­ intestinal or lower gastrointestinal bleeding. Bleeding intc the pancreatic duct is called haemosuccus pancreaticus. • This condition occurs due to enzymatic digestion of the blood vessels in the vicinity of pancreas. Thus splenic artery, gastroduodenal artery, etc. are commonly involved. • It has very high mortality. Prompt angiography followed by embolisation is the treatment. • Otherwise, laparotomy, ligation of pseudoaneurysm with or without intracystic ligation of the bleeders is the treatment (Figs 25.141 to 25.143).

Local complications l. Pancreatic abscess (Fig. 25.140) • It develops after 3-4 weeks of pancreatitis. Secondary infection in a pseudocyst results in pancreatic abscess. It usually points out on the left flank.

Fig. 25.141: Gastroduodenal pseudoaneurysm at surgery

Fig. 25.140: Aspiration of pus from pancreatic abscess

Fig. 25.142: Diagnosis by angiography

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Type II. Post necrotic pseudocyst that occur after an episodic of acute on chronic pancreatitis and have a diseased but not strictured pancreatic duct and there is after---communication between the duct and pseudocyst. Type III (also called retention cyst) occur in chronic pancreatitis, unifonnly associated with a duct stricture and a communication between duct and pseudocyst.

Fig. 25.143: As a cause of massive upper gastrointestinal bleeding (Courtesy: Dr Annappa Kudwa, Professor of Surgery, KMC, Manipal)

PSEUDOCYST OF PANCREAS Definition Collection of amylase-rich fluid in the lesser sac, due to pancreatic pathology. Fluid collection in the first 4 weeks is an acute fluid collection. After 4 weeks, it becomes an acute pseudocyst. PEARLS OF

WISDOM

It is called pseudocyst because it has no epithelial lining.

• Fluid is enclosed by a wall of fibrous granulation tissue and is called a pseudocyst. Aetiology 1. Fallowing an attack of acute pancreatitis, it usually appears after 4 weeks, as upper abdominal swelling. 2. Blunt injury of abdomen causing a ductal disruption wherein the pancreatic duct in the region of body is crushed against vertebral body results in a pseudocyst. 3. Some cause of chronic pancreatitis may be associated with pseudocyst.

Clinical features 1. Tensely cystic mass in the epigastrium, umbilical region or in left hypochondrium. Tensely cystic mass feels firm on palpation. Classically upper border of the mass is not felt. 2. Does not move with respiration because it is retroperitoneal in location. 3. It may have transverse mobility 4. It does not fall forwards 5. Percussion: It gives a resonant note because of stomach or intestine anterior to it. 6. Transmitted pulsations from aorta can be felt 7. If a Ryle's tube is passed, it can be felt over the swelling. It is called Baid sign. 8. Depending upon the tension within the cyst, it can be tender or nontender. Investigations 1. USG/CT: It can detect size, location of cyst and wall thickness. Confirm that there is no neoplasm of the pancreas before doing cystogastrostomy (Fig. 25.144). 2. Barium meal stomach: In a lateral picture, the stomach is pushed anteriorly and increased vertebrogastric interval is seen (not done nowadays, Fig. 25.145). 3. ERCP may demonstrate communication of the cyst with the duct (routinely not done).

Locations of pseudocyst 1. Between stomach and transverse colon 2. Between stomach and liver 3. Behind or below the transverse colon D'EGIDIO CLASSIFICATION OF PSEUDOCYST Type I. Acute postnecrotic pseudocyst that occurs after an episode of acute pancreatitis and is associated with normal duct anatomy and rarely communicate with pancreatic duct.

Fig. 25.144: CT scan: Classical pseudocyst pushing the stomach anteriorly-cystogastrostomy is ideal treatment

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Manipal Manual of Surgery

Fig. 25.145: Large pseudocyst behind the stomach demonstrated

by barium meal

Fig. 25.146: Posterior gastric wall is sutured to anterior cyst wal after creating a stoma of 6 cm

Treatment I. Conservative line of treatment • Majority of the pseudocysts following acute pancreatitis resolve spontaneously within 3-4 weeks. • Hence, regular ultrasound examination is done to observe the pseudocyst. Flowchart of management of pseudocyst Pseudocyst

Symptomatic

Infected

ERP (MRP)

Percutaneous drainage Normal duct

Communication

Endoscopic transpapillary stent

Fig. 25.147: Cystogastrostomy

Endoscopic internal drainage

Radiologic external percutaneous drainage

Surgical internal drainage ± resection

II. Surgery Increase in size of cyst, severe pain, no response to conser­ vative line of treatment are indications for surgery. 1. Cystogastrostomy (Figs 25.146 to 25.149) Indications: Pseudocyst in relation to head and body of pancreas. • Timing: Surgery is done after 6 weeks because that is the time required for the wall to become fibrous.

Fig. 25.148: Aspirated fluid is dark in colour, rich in enzymes­ 'engine oil' appearance

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--+----- Cyst

compressing stomach

D

Cyst-opened into stomach

Figs 25.149A to D: Steps of cystogastrostomy (Courtesy: Miss Vidushi, MBBS student, KMC, Manipal)

• Size of the cyst should be at least 6 cm. • Procedure: Anterior gastrostomy is done and an incision in the posterior wall of stomach opens into the cyst cavity. The contents are drained, opening is enlarged and cut end of stomach in posterior wall is sutured to cut edge of cyst wall. After one week, the cyst collapses. For reasons not known, the food does not enter the cyst cavity. Size of

cystogastrostomy stoma is about 6 cm. This procedure can also be done by endoscopic method. 2. Distal pancreatectomy: Cyst confined to the tail of the pancreas is treated with removal of the tail and the cyst. 3. Cystojejunostomy: By using Roux-en-Y loop can be done, for large cysts by suturing jejuna I loop to the cyst in the most dependent area (Figs 25 .150 to 25 .152).

Fig. 25.150: CT scan showing large pseudocyst with multiple pockets. Cystojejunostomy is the ideal treatment

Fig. 25.151: Roux-en-Y cystojejunostomy at surgery, inset shows the opened cyst wall which is thick

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Fig. 25.154: Pancreatic ductal anatomy

ANNULAR PANCREAS

Fig. 25.152: Cystojejunostomy-diagrammatic representation

It is a rare anomaly which occurs due to persistence of a po1tion of the ventral pancreatic anlage which fails to rotate. As a result of this, second part of the duodenum is surrounded by a thin rim of pancreatic tissue. Hence the name, annular pancreas (Key Box 25.38). Associated anomalies Intrinsic duodenal atresia or stenosis.

Fig. 25.153: Endoscopic cystogastrostomy-ideal to drain acute pseudocysts. Inset-after draining a nasobiliary catheter can be left in the cyst cavity ( Courtesy: Dr Filipe Alvares, Medical Gastro­ enterologist, KMC, Manipal)

Ill. Endoscopic drainage This is easy, less invasive and ideal in draining acute pseudocysts. This procedure can also be repeated. However, chances of introducing infection are present. Endoscopic method of draining the pseudocysts is also being done for chronic cysts (Fig. 25.153).

Clinical features • Neonatal type: It manifests early in life. It produces symptoms of acute intestinal obstruction with vomiting and inability to take food. • Adult type: It manifests after the age of 20. Vomiting is bile-stained. Due to stasis in the pyloric antrum, features of duodenal ulcer may be present. Investigations • Plain X-ray abdomen: Double bubble appearance occurs due to dilated stomach and dilated proximal duodenum. • Barium meal: X-ray can demonstrate obstruction to second pait of duodenum. Treatment • Duodenoduodenostomy is the treatment of choice. Other­ wise, duodenojejunostomy can be done.

............................. . KEY BOX 25.38

CONGENITAL ANOMALIES OF THE PANCREAS Embryological anatomy • Pancreas is formed by fusion of ventral and dorsal buds. This will occur by 6 weeks. Malrotation of ventral bud causes annular pancreas. • Ventral bud forms head and uncinate process (Fig. 25.154).

• • • • • •

ANNULAR PANCREAS Persistent ventral 'anlage' Neonatal or adult age Barium meal-obstruction to second part of duodenum Plain X-ray-'double bubble' Duodenoduodenostomy is the treatment Never divide the annulus

Gall Bladder and Pancreas PEARLS OF

WISDOM

It is tempting to divide the thin pancreatic rim. This should not be done as it will result in a pancreaticfistula.

Differential diagnosis 1. Pyloric stenosis 2. Wilkie's disease (chronic duodenal ileus) ECTOPIC P ANCREAS • This condition is not uncommon • Occasionally, at laparotomy for unrelated conditions, a soft to firm irregular tissue or nodule, is found on the intestinal surface. • Biopsy of this may come later as ectopic pancreas. Most of them are asymptomatic. • It may be found in the submucosa of the stomach, duodenum, small intestines, Meckel's diverticulum or in the hilum of the spleen. Complications 1. In the stomach: It may undergo cystic degeneration. 2. In the intestine: It can cause intussusception. Sometimes, it may be the source of gastrointestinal bleeding. 3. In the Meckel's diverticulum: Any of the complications mentioned above. CONGENITAL CYSTIC FIBROSIS • It is inherited as an autosomal recessive disorder. • It is a generalised dysfunction of exocrine glands resulting in defective mucus secretion. • Malabsorption due to pancreatic insufficiency is a feature. • Pulmonary disease due to bronchiolitis occurs later (Key Box 25.39).

11¥MJ,-aar.,i • • • •

VISCID MUCUS Meconium ileus Cystic fibrosis of pancreas Respiratory tract infection Increased Na+ loss in the sweat

�

Pathology The viscid mucin which is produced results in obstruction of the ducts and ductules. Stasis of pancreatic secretions, alveolar rupture due to increased pressure take place later. As a result of alveolar rupture, pancreatic enzymes leak outside resulting in pancreatitis.

611

Clinical features l . At birth: Meconium ileus or meconium peritonitis is an important manifestation of cystic fibrosis of pancreas. 2. In infants • Recurrent respiratory tract infection in the form of bronchiolitis and bronchiectasis results in cough with expectoration and dyspnoea. • Emaciation, steatorrhoea. Stools are pale and sticky. 3. Older children Steatorrhoea, gross emaciation and wasting are the features. Due to poor nutritional status, cirrhosis of the liver with portal hypertension can be a feature. 4. In adult Lucky survivors will suffer from gross wasting, diabetes, bronchiectasis, cirrhosis of the liver, sialoadenitis and choroiditis. Investigations • Normal contents of the electrolytes in the sweat: Na+-70 mmol/L, c1--60 mmol/L, K+- 20 mmol/L. • In these patients, sodium excretion may be three to four times more than normal. Treatment Only symptomatic I. Nutritional support • Fat intake should be low, protein should be increased. • Pancreatic enzyme preparations (5 to 10 g) are given two to three times per day, to supplement pancreatic enzymes. II. Control infection Respiratory tract infection is treated with antibiotics, bronchodilators, mucolytic agents, etc. III. Role of surgery Indicated in meconium ileus to relieve intestinal obstruction. PANCREATIC DIVISUM AND DUCTAL ANOMALIES • Pancreatic divisum is the most common congenital abnor­ mality of the pancreas. It is found in 5-10% of patients. • It is caused by failure of fusion of the dorsal and ventral portions of the developing pancreas. In the majority of patients, this anomaly is of no clinical importance. • In a certain subset of patients, however, estimated to be approximately 5-20%, pancreatic divisum is a clinically important cause of abdominal pain, acute recurrent pancreatitis or chronic pancreatitis. • The frequency of pancreatic divisum in patients with pancreatitis in recent studies is approximately 10% (Key Box 25.40).

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Manipal Manual of Surgery

............................. . KEY BOX 25.40

PANCREATIC DIVISUM • It is one of the uncommon causes of acute pancreatitis. • In 10% of patients. Majority of pancreas drains through duct of Santorini through lesser papilla. • Outflow obstruction-treated by sphincteroplasty of minor papilla.

Pathophysiological consequences The cause of pancreatitis in this common anomaly is hypo­ thesised to be caused by minor papillary insufficiency caused by papillary fibrosis and subsequent stenosis. Diagnosis • The diagnosis is established by ERCP. • MRCP is a noninvasive and accurate method of establishing the diagnosis. However, a negative MRCP does not exclude the diagnosis of divisum as the concordance with ERCP ranges from 50% to more than 70%. • The accuracy of the MRCP may be improved by administration of secretin. Treatment • In patients with minimal symptoms, treatment with pancreatic enzymes may reduce pancreatic secretion and hence, pain. Pain management referral may be necessary. • Patients with acute pancreatitis are estimated to benefit from either surgical minor sphincteroplasty or endoscopic minor papillotomy.

D. Absent accessory duct E. Blind end of accessory duct with main duct communication MISCELLANEOUS PANCREATIC FISTULA Most common after pancreatic trauma (Fig. 25.156). Causes l . External fistulae occur due to operative injury to the pancreas or due to a pancreatic anastomotic leak. • Injury to the tail of pancreas during splenectomy or adrenalectomy. • Injury to the head and body during radical gastrec­ tomy. • Pancreaticojejunostomy for chronic pancreatitis or following Whipple's operation can also give rise to fistula in the postoperative period. • External drainage of an infected pseudocyst. 2. Internal fistulae: It can occur following a blunt injury abdomen wherein the neck of the pancreas is crushed against lumbar spine resulting in injury. Internal fistulae can communicate with pleural space resulting in a pancreaticopleural fistula. Clinical presentation • In many cases, the patients present with a discharge of straw­ coloured fluid from the drain site in the postoperative period. • Internal fistula can manifest in a totally unexpected manner, sometimes, as a case of pleural effusion.

A few anomalies of pancreatic duct are given below (Figs 25.155A to E) A. Normal anatomy B. Terminal end of accessory duct fibrosed C. Blind ending of accessory duct

Investigations • Amylase levels in the pleural fluid, peritoneal fluid and in the discharge will be high.

Figs 25.155A to E: Anomalies of pancreatic duct

Fig. 25.156: Pancreatic fistula following acute pancreatitis

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• Abdominal ultrasound is done to rule out a pseudocyst of the pancreas. • ERCP: It can demonstrate leakage of the dye from the pancreatic duct into the surrounding area or along the fistulous tract and proximal obstruction, if any. • CT fistulogram can define the exact site of communication to pancreas. It can define the length of the fistula also. • CT angiogram is indicated in cases of bleeding associated with pancreatic fistula. Treatment 1 . Conservative treatment • In majority of the cases following surgery, the fistulous discharge stops within one to three weeks of time. During this period, the skin is protected by application of zinc oxide cream. Electrolytes have to be checked frequently. • Injection octreotide in the dose of 50-100 mg, 8th hourly will help in decreasing fistula output by more than 80-90%. 2. Surgical treatment • If the fistula persists in spite of conservative treatment, fistulectomy with removal of involved part of the pancreas and body or tail has to be done. • In very difficult cases (plastered abdomen) or in fistulas on the anterior abdominal side, fistulogastrostomy can also be done. • Pancreaticojejunostomy is another option. Complications of pancreatic fistula 1. Secondary infection 2. Massive bleeding: It occurs due to digestion of elastin fibres of blood vessels resulting in pseudoaneurysms. Morbidity is high. 3. Ramification which means branching pattern of the fistula renders the surgery difficult. 4. Bronchopancreatic fistula-difficult to treat

A patient was admitted to the hospital with acute breathless­ ness. He was found to have left-sided pleural effusion (massive). He had blunt injwy abdomen 2 months back during which time laparotomy and closure of a proximal jejuna! perforation had been done. There was no obvious sign of pancreatic injury. Aspiration of pleuralfluid revealed high amylase. It was a case of pancreaticopleural fistula.

WHITE BILE • It is a misnomer (Fig. 25.157) • In long-standing cases of obstruction to the CBD, the bile in the CBD gets absorbed and is replaced by mucus secreted from the CBD.

Fig. 25.157: White bile suggestive of severely impaired liver cell

function

• It is not white but straw coloured • It is not bile but is mucus Significance • It indicates a long-standing obstruction. • It has to be relieved as an urgent procedure. • White bile is seen in: - Long-standing stricture of the CBD - Due to the stones in CBD - Rarely, seen in periampullary carcinoma. PANCREATIC ASCITES Definition • Accumulation of 'enzyme rich' pancreatic exudate in the peritoneal cavity is called pancreatic ascites. • It is a protein-rich, noninfected fluid with protein levels greater than 25 g/L. Causes • Acute pancreatitis mainly alcoholic • Chronic pancreatitis • Trauma to pancreas • Ruptured pseudocyst Pathogenesis • Disruption of ductal system of pancreas followed by spread of enzyme-rich fluid both anteriorly and posteriorly results in pancreatic ascites. • Anterior rupture results in ascites. • Posterior spread results in pleural effusion. Clinical features • History suggestive of pancreatitis, gross abdominal distention. • Shifting dullness • Breathlessness-due to pleural effusion.

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Manipal Manual of Surgery Investigations

• Serum amylase, may be elevated due to reabsorption acros� parietal membrane. • Ultrasound/CT: It can detect fluid, pancreatic duct and guide aspiration. • ERCP: May slow ductal communication. Treatment

Fig. 25.158: Pedal oedema-severe hypoalbuminaemia

• Repeated tapping-follow with albumin infusion. Most of the cases are associated with hypoalbuminaemia (Figs 25.158 and 25.159). • Octreotide to decrease pancreatic secretion. • Total parenteral nutrition • ERCP-pancreatic duct stenting • Surgical: If there is no response by 2-3 weeks, resection (for tail lesion) or drainage for body lesion is indicated. ISLET CELL TRANSPLANTATION

• This is emerging as a fine treatment for diabetes and chronic pancreatitis after pancreatectomy. • Many patients have undergone islet cell transplantation with minimal complications (see Chapter 49). WHAT IS NEW IN THIS CHAPTER? /RECENT ADVANCES

• All the topics have been updated. • Treatment of acute pancreatitis has been given in more detail with more illustrations. • Islet cell transplantation, percutaneous cholecystostomy and pancreatic divisum have been added. Fig. 25.159: Clinical presentation-tense ascites

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MULTIPLE CHOICE QUESTIONS 1. Which of the following statements is false for pseudocyst of pancreas? A. It occurs in lesser sac B. Cystogastrostomy is usually the treatment of choice C. Better to operate after 2 weeks D. It has no epithelial lining 2. Which of the following is not the cause for gall stone formation? A. Saint's triad B. Haemolytic anaemia C. Infection D. Gastrojejunostomy 3. Gall bladder carcinoma has following features except: A. It is squamous cell carcinoma B. It does cause jaundice often C. It does not respond to radiation D. Prognosis is very poor 4. Most ideal and quick method for the diagnosis of gall stones is by: B. MRI scan A. Endosonogram C. CT scan D. Ultrasound 5. Following is not the feature of acute cholecystitis: A. It causes pain in the right hypochondrium B. If it perforates, gas under diaphragm is detected by percussion C. Shoulder pain can occur D. Acalculous cholecystitis is due to hypotension 6. Following is the most important sign of acute cholecystitis: A. Pain in the right shoulder B. Intercostal oedema and tenderness C. Positive Murphy's sign D. Hyperaesthesia of the abdominal waJJ 7. Cystic duct joins supraduodenal CBD in what percentage of the cases? B. 90% A. 80% D. 95% C. 70% 8. Porcelain gall bladder has following features except: A. It is calcification of the gall bladder B. Plain X-ray is not very useful to detect this C. CT scan will detect this D. It may be associated with carcinoma gall bladder 9. Which one of the following is true for choledochal cyst? A. Acquired dilatation B. Not premalignant C. Type I cyst is the most common variety D. Does not cause pancreatitis

10. Which one of the feature is true in gall stone ileus? A. Obstruction is in terminal ileum B. Stone reaches ileum via ampulla of Yater C. Obstruction and features of perforation of gall bladder are found D. Cholecystectomy and extraction of the stones by enterotomy is done 11. Following are true for acalculous cholecystitis except: A. It can be seen in septic shock due to hypotension B. Some features of cholecystoses may also be found C. Salmonella typhi can also give rise to acute acalculous cholecystitis D. Rare to get chronic cholecystitis in Salmonella cholecystitis 12. Following are indications for common bile duct exploration except: A. Palpable stones in the CBD B. Dilated common bile duct C. Jaundice D. Thickened common bile duct 13. Following are risk factors for cholangiocarcinoma except: A. Choledochal cyst B. Caroli 's disease C. Primary sclerosing cholangitis D. Biliary stricture 14. Which one of the following is true for carcinoma gall bladder? A. It is common in males B. Disease occur around 5th decade C. Gall stone disease does not predispose to carcinoma gall bladder D. Majority are adenocarcinoma 15. Caterpillar hump is an anomaly of what structure? A. Cystic artery B. Hepatic artery C. Coeliac artery D. Gastroduodenal artery 16. Portal veins is formed at: A. Posterior to head pancreas B. Posterior to body of pancreas C. Anterior to neck of pancreas D. Posterior to neck of pancreas 17. Following are true about anatomy of the pancreas except: A. 30% is by the head of pancreas B. 75% of islet cells are beta-cells producing insulin C. 80-90% pancreatic tissue is exocrine pancreatic tissue D. 5% of islet cells are A cells producing glucagon

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18. To avoid spillage of cells after FNAC of the pancreatic head mass which is the best investigation? A. Endoscopic biopsy B. CT-guided biopsy C. MRI-guided biopsy D. Endosonographic biopsy 19. Which one of the following is true for pancreas divisum? A. Dorsal pancreatic duct become accessory duct B. It does not cause pancreatitis C. MRCP is not an ideal investigation for diagnosis D. Endoscopic sphincterotomy has a definitive role 20. Following are true for annular pancreas except: A. It is more prevalent in children with Down syndrome B. [t surrounds 2nd part of duodenum C. Duodenal obstruction causes vomiting in neonate D. Gastrojejunostomy is the treatment of choice 21. The use of ultrasound in the diagnosis of acute pancreatitis is mainly to: A. Rule out perforation B. To look for oedematous pancreatitis C. To rule out gall stones D. To look for pancreatic stones 22. Role of CT scan in acute pancreatitis include following except: A. To detect gall stones B. Diagnostic uncertainty C. To detect necrotizing pancreatitis D. To find out the localized complication 23. Following are features of acute pancreatitis except: B. Cullen's sign A. Shock C. Grey Turner's sign D. Kehr sign 24. Which one of the following is not routinely recom­ mended in severe pancreatitis? A. Aggressive fluid resuscitation B. Nasogastric feeding C. ICU monitoring with oxygenation D. Antibiotic prophylaxis

25. Which one of the following is true for pancreatil necrosectomy? A. Usually done within 2-3 weeks of acute attack 01 pancreatitis B. Gall stones-if present, should not be removed along with necrosectomy C. Blunt dissection is the best technique D. Feeding jejunostomy may not be useful 26. Sudden rise in platelet counts in acute pancreatitis suggests: A. Pancreatic fistula B. Bleeding C. Portal vein thrombosis D. Pancreatic necrosis 27. Following are true about pseudocyst fluid except: A. Ultrasound can easily detect pseudocyst B. CEA levels are usually above 400 ng/ml C. High amylase levels D. Inflammatory cells in the aspirate 28. Following are true for tropical pancreatitis except: A. Starts in young age B. High incidence of stone formation C. High incidence of diabetes mellitus D. Does not predispose to pancreatic cancer 29. The best surgical treatment for chronic pancreatitis with dilated duct is: A. Frey's operation B. Whipple's operation C. Longitudinal pancreaticojejunostomy D. Distal pancreatectomy 30. Following are the risk factors for carcinoma pancreas except: A. Cigarette smoking B. Hereditary pancreatitis C. Chronic pancreatitis D. Female sex

ANSWERS 1 C

2 D

3 A

4 D

5 B

6 D

7 A

8 B

9 C

10 A

11 D

12 D

13 D

14 D

15 B

16 D

17 D

18 D

19 D

20 D

21 C

22 A

23 D

24 B

25 C

26 C

27 B

28 D

29 C

30 D

26 Spleen • • • • • • • • •

Introduction Surgical anatomy Functions of the spleen Congenital abnormalities Rupture Complications of splenectomy ITP Hereditary spherocytosis Acquired autoimmune haemolytic anaemia

Introduction Diseases of the spleen and causes of enlargement of the spleen are a great concern for physicians. Dozens of differential diagnosis of splenomegaly are being taught in medicine. However, a surgeon's role in the treatment of an enlarged spleen is minimal. However, it is he who does this 'magic' of removing the spleen within minutes and saves the life of a patient with a ruptured spleen. A surgeon plays a major role by treating hypersplenism with splenectomy. In this chapter, only topics related to surgeon's interest are dealt with. Other topics are mentioned briefly. SURGICAL ANATOMY (Fig. 26.1) The spleen is an anatomically small organ, hidden underneath the 9th to 11th ribs (dull note on percussion) measuring 1 x 3 x 5 inches and weighing about 7 oz (80-300 gm). It lies in intimate contact with the undersurface of the diaphragm. This explains why a splenic abscess can rupture through the diaphragm causing empyema. When blood collects due to splenic injury, it in-itates the diaphragm causing refen-ed pain to the shoulder tip (Kehr's sign). The anterior border is notched and is in contact with the stomach. It is enclosed by the gastrocolic omentum. The surgical importance of this is that when traction is applied on the stomach during vagotomy, a splenic capsular tear can occur, resulting in bleeding. The two leaves of gastrocolic omentum

• • • • • • • •

Thalassaemia Sickle cell anaemia Splenectomy for other conditions Splenic artery aneurysm Hairy cell leukaemia OPSI Interesting 'most common' What is new?/Recent advances

pass backwards in front of the kidney forming the lienorenal ligament (Fig. 26.2). This ligament is responsible for some part of the posterior fixation of the spleen. When this ligament is divided, the spleen can be brought to the surface of the wound during splenectomy. The inferior part of the hilum of the spleen is (just) in contact with the tail of the pancreas. Often, during an emergency splenectomy, the tail of pancreas can get injured, resulting in a pancreatic fistula (Figs 26.2 and 26.3) Splenic parenchyma • It is highly vascular • Splenic pulp has red pulp and white pulp. • Red pulp is a honeycombed vascular space which is made up of cords of reticular cells and sinuses. • White pulp consists of lymphatic tissue and lymphoid follicles containing lymphocytes, plasma cells and macro­ phages. Blood supply • Arterial supply: Splenic artery is a branch of coeliac artery. At the hilum of the spleen, it divides into 4-5 trabecular arteries and then branches into central arteries. Central arteries pass through white pulp and give radial branches to periphery including distal red pulp. Anatomical knowledge of these branches helps when performing partial splenectomy for ruptured spleen. (The blood flow rate through spleen is about 300 ml/min.)

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• Venous drainage: Four to five branches of the vein joir and fonn the splenic vein. This forms the portal vein b1 joining with superior mesenteric vein behind the 2nd par1 of the duodenum. Lymphatic drainage The lymph drains into hilar nodes, then into retropancreatic nodes and finally into coeliac nodes. Since it has intimate contact with the fundus ofthe stomach and tail ofthe pancreas, spleen also used to be removed (nowadays not done) along with the stomach and tail of the pancreas during radical gastrectomy and distal pancreatectomy. Fig. 26.1: Location of the spleen in relation to the fundus of the

stomach and the diaphragm

Spleen

Left suprarenal gland

Splenic flexure of colon

Splenic artery

Tail of pancreas

Splenic vein Pancreas Left kidney

Fig. 26.2: Visceral relations of the spleen

Lienorenal ligament containing tail of pancreas and splenic artery Tail of pancreas

Accessory ""--------�­ spleens

FUNCTIONS OF THE SPLEEN 1. Destruction: Removal of abnormal aged, damaged red blood cells by a process is called culling. Culling, the Greek word implies sorting a collection of two groups. One that will be kept and the other that will be rejected. For example, spherocytes, in hereditary, spherocytes are broken down in spleen. This happens in red pulp. 2. Production: Production of lymphocytes, monocytes and plasma cells. Lymphopoiesis continues throughout life. Lymphocytes take part in immune response of the body­ largest. 3. Antibody production: Spleen synthesises antibody in its white pulp. It removes antibody-coated bacteria and anti­ body-coated blood cells via blood and lymph node circulation. 4. Immune response: White pulp is composed ofmalpighian corpuscles. They are composed of B lymphocytes and T lymphocytes-which are in periarteriolar lymphoid sheaths (PALS). Thus white pulp plays a major role in active immune response through humoral and all mediated pathways. 5. Reservoir of platelets: It is a storehouse ofplatelets, which are released into circulation in an emergency situation. Thus when spleen is enlarged, 80% of the platelets are sequestrated resulting in thrombocytopenia.

Superior vascular segment

PEARLS OF

Spleen

6. Immune function: It is the major site for synthesis of tuftsin, a peptide that stimulates phagocytic activity of leucocytes. Opsonins are antibodies produced by spleen against bacteria and fungi. 7. Removal: Heinz bodies, Howell-Jolly bodies, etc. are removed from RBCs. These cells are non-defo1mable intra­ cellular substances. 8. Erythrocyte production: During foetal development. White and red blood cells production occur till 5th month ofgestation. In adults in case ofmyelofibrosis erythopoiesis can occur.

Avascular plane Inferior vascular segment

Fig. 26.3: Peritoneal ligaments attached to the spleen and com­ mon sites of accessory spleen

WISDOM

Splenomegaly typically involves expansion of red pulp.

Spleen

CONGENITAL ABNORMALITIES 1. Accessory spleens: Splenunculi: They are found in about 10-20% of the population. - They can be found near the hilum of spleen, tail of pancreas, splenic ligaments or in the mesocolon. - In diseases of the spleen (haemolytic anaemia, thrombocytopaenic purpura), all these accessory spleens have to be removed along with spleen. 2. Splenic agenesis is rare. 3. Polysplenia is rare. It occurs due to failure of splenic fusion. 4. Wandering spleen is due to loose ligaments. It is more vulnerable for torsion. 5. Hamartoma: Rare, small masses of I cm in diameter. It may in lymphoid tissue also (Hamartoma = misfire). RUPTURE OF THE SPLEEN CAUSES

619

Grade II:

Grade ill:

Grade IV:

Grade V:

Nonexpanding subcapsular haematoma 10-50% surface area. Nonexpanding intraparenchymal haema­ toma 50% surface area Intraparenchymal haematoma >2 cm Parenchymal laceration >3 cm deep Ruptured intraparenchymal haematoma with active bleeding. Laceration involving segmental or renal vessel> 25% spleen volume devascularised Completely shattered or avulsed spleen. Hilar laceration which devascularises entire spleen.

• Blunt injury abdomen - Injury to the left side of chest, left lower rib fractures, due to fall from a tree, road traffic accidents can be associated with splenic rupture. - Retroperitoneal haemorrhage, fracture spine and renal injuries may be associated with splenic injury. • Penetrating injuries to the abdomen may cause rupture of spleen. • Spontaneous rupture of the spleen is seen in malaria and infectious mononucleosis, rarely in sarcoidosis, haemolytic anaemia and leukaemia. • Iatrogenic: Splenic capsule may be torn during surgical procedures such as vagotomy or gastrectomy due to traction on the stomach. TYPES OF INJURIES Most of these injuries are diagnosed by CT scan today (CT classification is given). 1. Subcapsular haematoma: A capsular tear or parenchymal tear results in subcapsular haematoma. 2. Clean (incised wounds): This can happen in sharp injuries, staal injuries (not common) or operative injuries. 3. Lacerated wounds: Often they are multiple as in blunt injuries abdomen. 4. Hilar injuries with pedicle avulsion are the most serious injuries. Need urgent laparotomy and ligation of bleeders and splenectomy. Otherwise, death will occur. 5. Splenic injury with other organs. Tail of pancreas, diaphragm, left kidney, left colon. Splenic injury CT scan grading (Figs 26.4 to 26.6) Nonexpanding subcapsular haematoma Grade I: < 10% surface area Nonbleeding capsular laceration with < 2 cm deep parenchymal involvement.

Grade II

Grade IV

Grade V

Fig. 26.4: Various grades of splenic injury

Fig. 26.5: Lacerated spleen

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• A subcapsular haematoma which had developed ruptures after some time. • Associated injury to the tail of pancreas causes release of enzymes which digests the tissues at a later date. Thi5 variety is called "delayed type of shock" or delayed rupture of spleen. PEARLS OF

WISDOM

In polytrauma, if a hypotensive patient responds to intravenous fluids, it is a hypovolaemic shock. Head injury is rarely a cause of hypovolaemic shock. Fig. 26.6: Lacerated spleen-Grade 111-splenectomy specimen

CLINICAL PRESENTATION It can be divided into three groups: 1. Tearing of splenic vessels: Avulsion of splenic pedicle can result in severe haemorrhage and shock. Death can occur within a few minutes. Even in the best of the situations, death cannot be prevented often. 2. A slow developing haemorrhagic shock followed by recovery. Examination reveals evidence of intraperitoneal bleeding. This can occur due to a capsular tear or injury to the splenic parenchyma. Its clinical features are as follows: • Anaemia-pallor • Pulse-tachycardia, more than 100/min • BP-low/hypotension • Cold, clammy extremities • Abdominal distension • Paralytic ileus develops slowly • Guarding and rigidity • Kehr's sign is positive. Irritation of undersurface of the diaphragm by the blood causes irritation of phrenic nerve (C3, 4). Thus, this pain is referred to shoulder region (supraclavicular nerve C4). PEARLS OF

WISDOM

Kehr's sign can be elicited by elevation of the foot end of bed for about 10 minutes.

• Ballance's sign: Blood in the vicinity of spleen is fresh blood which is coagulated and blood in the periphery is not coagulated. Hence, there will not be shifting dullness on the left side of the abdomen but can be present on the right side. • Saegesser's splenic point of tenderness: An area of tenderness on the left side between stemomastoid and scalenus medius (rare sign). 3. Initial features of haemorrhagic shock, recovery and sudden haemorrhagic shock after a few hours to a few days. It is due to the following reasons: • Greater omentum seals off a tear which gets reopened after some time. Time period is called "laten period ofRandet".

COMPLICATIONS OF SPLENIC INJURIES 1. Haemorrhagic shock-if not treated-multi-organ failure results. 2. Disseminated intravascular coagulation due to massive bleeding and blood transfusion. 3. Injuries to the tail of pancreas-manifesting later as pancreatic ascites, pancreatic fistula or pseudocyst of pancreas. 4. Splenic artery pseudoaneurysm. 5. Splenic arteriovenous fistula. INVESTIGATIONS 1. Rb% estimation, PCV (packed cell volume) estimation

(however, in appropriate cases it should be repeated at frequent intervals to detect continuing haemorrhage). 2. Four quadrant tap/aspiration by using a fine needle (23 gauge) demonstrates blood either fresh or old. However, diagnostic peritoneal lavage (DPL) is more reliable (vide infra). 3. Emergency USG can reveal a splenic tear, a subcapsular haematoma and can rule out other injuries. It is the most important investigation in suspected cases of splenic injury. 4. CT scan is more reliable. It should be used in cases of doubtful diagnosis and stable patients. 1 It also rules out hollow viscus perforation, pancreatic injuries, etc (Fig. 26.7).

Fig. 26.7: CECT showing splenic and liver injuries

1 Deaths have been reported in CT scan room, in unstable patients with haemoperitoneum.

Spleen

5. Diagnostic peritoneal lavage (DPL) • It is indicated in blunt injury abdomen where there are equivocal signs or doubtful signs of peritonitis. Indications • Unconscious patient with polytrauma with signs of abdominal injury. • Unexplained shock • Associated spinal cord injury Procedure (Fig. 26.8) • A l cm incision is made in the subumbilical region under local anaesthesia (LA) after emptying the bladder. The peritoneum is opened and a 12 or 14 Fr peritoneal dialysis catheter is introduced into the peritoneal cavity. • The skin is closed and 1000 ml normal saline is allowed to flow over 30 minutes. • The patient is turned to the right and left sides and fluid is allowed to flow out. • DPL is positive when 1. Fresh blood of more than 20 ml is removed immediately after inserting dialysis catheter and it does not clot.

621

2. RBC count l,OO,OOO/mm3 or WBC > 500/mm3 . 3. Amylase level of over 175 units/di. 4. Bile, food or any other fresh contents. 5. Gram stain is positive in the contents of lavage. • Diagnostic accuracy is around 95%. DPL is useful to rule out intestinal injury in addition to intraperitoneal bleeding. PEARLS OF

WISDOM

DPL is more sensitive for mesenteric injury than CT scan. However in early diagnosis of hollow viscus injuries, DPL is superior to CT scan. 5. Plain X-ray abdomen 1 erect (should not be done in an emergency situation) when patients are in shock. • Splenic outline may not be seen clearly. • Fundic air bubble may be indented by the haematoma. • Psoas shadow is obliterated (KUB X-ray). • Evidence of fracture of lower ribs. • Evidence of fluid in the peritoneal cavity-'ground glass' appearance • It may detect free gas under the dome of diaphragm. • Elevation of left dome of diaphragm. TREATMENT (Key Box 26.1 and Fig. 26.9)

IISIIWlllllllir,1 W

•

• • • •

Fig. 26.8: Diagnostic peritoneal lavage

I • Clinical - haemorrhagic shock • FAST - splenic injury • Four quadrant tap • Shock continuing

I

FAST decision making j Clinical - haemorrhagic shock response to volume replacement

• Clinical - haemorrhagic shock • FAST - spleen + liver injuries Urgent resuscitation and

FAST- splenic injury CT scan - proves splenic injury

CT scan

Unstable patient Explore

TREATMENT-4 HEADINGS Emergency splenectomy - Massive bleeding and unstable Splenorrhaphy - Stable Partial splenectomy - Stable - Stable patient Nonoperative

l

I

FAST: Focussed abdominal sonography in trauma

Unstable patient Explore j

I

I Stable I

I Conservative]

Fig. 26.9: Managment of splenic rupture 1 Please do not make an unstable patient stand for the sake of X-ray. He may collapse due to hypotension.

[Unstable]

I f Explore l

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Manipal Manual of Surgery

1. Emergency splenectomy When there is active bleeding with hypotension due to large lacerations, emergency splenectomy is the treatment of choice since it is quick, easy to perform and can be life­ saving. The splenic artery is ligated first at the upper border of pancreas followed by splenic vein. In desperate situations, the spleen is mobilised by incising the lienorenal ligament, a large arterial clamp is applied at the splenic hilum and splenectomy is done (Figs 26.10 and 26.11 ).

Fig. 26.12: Partial splenectomy

Fig. 26.10: Large laceration of the spleen

Fig. 26.13: Splenorrhaphy

3. Splenorrhaphy (Fig. 26.13) • It is a method of preservation of spleen when the general condition of the patient is reasonably good. • In this, a small tear is sutured by using chromic catgut and can be wrapped using greater omentum.

Fig. 26.11: Haemoperitoneum-emergency splenectomy

2. Partial splenectomy (Fig. 26.12) • It can be done because the splenic artery gives an upper polar branch and a lower polar branch which again divides into 2 branches. Hence, when one of the branches of splenic artery is ligated, the bleeding stops and a partial splenectomy can be done comfortably. • After splenectomy, the spleen can be cut into multiple pieces and can be implanted within the greater omentum. The spleen survives because of neovascularisation and functions like a spleen in the production of antibodies. This can be done in children.

4. Nonoperative or conservative treatment • It is safe in selected adult and paediatric patients with isolated splenic injuries. • Small risk of delayed rupture should be explained to the patient (Key Box 26.2).

1. 2. 3. 4. 5. 6.

NONOPERATIVE TREATMENT PATIENT SELECTION Haemodynamically stable patients No other associated organ injuries which require laparotomy. No extra-abdominal injuries which may preclude assessment of abdomen. Facilities for emergency resuscitation, ultrasonography, CT scan should be available. Patient must be willing for hospitalisation and close observation. Regular monitoring by duty doctors, expert nursing staff who maintain hourly vital charts.

Spleen

• Patients are admitted in intensive care unit. Serial monitoring of haemoglobin, total WBC counts are done. Abdominal girth and blood pressure are monitored. If there is any doubt of hypotension, exploration is done. EARLY: COMPLICATIONS OF SPLENECTOMY 1. Pancreatic injury (0-6%): Treated by drain placement and measuring drain amylase. Usually it is a minor discharge, generally settles over a few days. 2. Vascular injury (2-3%): Slippage of ligature (splenic artery), hilar dissection, capsular tear during retraction. Needs to be identified and sutured or coagulated or clamped. 3. Injury to bowel: Colon, stomach. These can be avoided with careful dissection techniques. During ligation of sh01t gastric arteries too close to the stomach side, injury to the stomach can occur. It is repaired in two layers. Colonic injury can occur during dissection to seperate the lower pole. It is also repaired in the similar manner. 4. Diaphragmatic injury: This can happen when adhesions are present between spleen and diaphragm due to perisplenitis or recurrent infarctions. Simple suturing is enough if recognised. 5. Splenosis: This refers to multiple small implants of splenic tissue on the peritoneal smface following traumatic rupture of spleen. They can give rise to adhesions. 6. Pulmonary: These are common complications. Generally get unnoticed. It can range from minor cough to atelec­ tasis to pneumonia and pleural effusion. Basal atelectasis is one of the common causes of postoperative fever. 7. Paralytic ileus: Treated by drip and suction 8. Haematemesis can occur due to congestion of tbe gastric mucosa as a result of ligation of the short gastric vessels. 9. Subphrenic abscess: Not common. If it occurs, percutaneous drainage and antibiotics (Fig. 26.14). 10. Wound problems: Haematomas, seromas and wound infection. 11. Thrombocytosis and thrombotic complications: Sometimes it can be dangerous. They can present as abdominal pain and vomiting which is confused for incision pain, gastritis, etc. Ultrasound examination is a must. One of our patients bad portal vein thrombosis

623

which was managed by anticoagulants. Platelet coun after splenectomy was 8 lakh/mm3 . INDICATIONS FOR SPLENECTOMY I. Always indicated (a) Primary splenic tumour (b) Hereditary spherocytosis ll. Usually indicated (a) Primary bypersplenism (b) Chronic ITP (c) Splenic vein thrombosis (d) Splenic abscess III. Sometimes indicated (a) Splenic injury (b) Autoimmune haemolytic disease (c) Elliptocytosis haemolysis (d) Nonspherocytic congenital haemolytic anaemia (e) Hodgkin's disease with anaemia (f) Thrombotic thrombocytopenic purpura (g) Idiopathic myelofibrosis (h) Splenic artery aneurysm (i) Wiskott-Aldrich syndrome (Key Box 26.3) (j) Gaucher's disease

.......

1mmm1lillllllillllll,� WISKOTT-ALDRICH SYNDROME

�

• X-linked disorder predominantly presents as features of thrombocytopaenia such as epistaxis, bloody diarrhoea, petechiae in young age. • Combined 'B' and 'T' cell immunodeficiency causes eczema • More incidence of malignancies • Spleen destroys platelets counts as low- 20,000 or 40,000/µL • The best treatment is human leukocytic antigen (HLA) matched sibling bone marrow transplantation. • Otherwise-splenectomy and antibiotics are the treatment of choice.

IV. Rarely indicated • Chronic leukaemia • Thalassaemia major • Sickle cell anaemia • Felty's syndrome • Hairy cell leukaemia HAEMATOLOGICAL INDICATIONS FOR SPLENECTOMY IDIOPATHIC THROMBOCYTOPAENIC PURPURA (ITP)

Fig. 26.14: Post-splenectomy large collection

• This condition occurs due to development of autoantibodies against patient's own platelets (Key Box 26.4). • Normal levels of platelets are between 1,50,000 and 4,00,000 cells per mm3 .

Manipal Manual of Surgery

624

llf.MI-....W,1 � OTHER CAUSES OF PURPURA • J, Production: Cytotoxic drugs, aplastic anaemia. • i Consumption of platelets: DIC as in septicaemia. • i Destruction: SLE, infectious mononucleosis. • i Capillary fragility: Steroid induced or Henoch-Schonlein purpura.

• The spleen is probably responsible for sequestration of platelets and for production of antibodies. Types Type 1: Acute-children: Follows an acute infection and resolves spontaneously in about two months. Type 2: Chronic-adults: Longer than six months. No cause is identified. Clinical features • Condition is common in females, F : M :: 3 : 1. • Purpuric patches (ecchymosis refers to skin discolouration due to extravasation of blood) are found on the buttocks and petechial haemorrhages (spots) are found on the limbs. These are dependent areas having high intravascular pressure (Figs 26.15 to 26.17). • All types of haemorrhages are common and can be mild or moderate (Key Box 26.5).

Treatment In children • Spontaneous regression occurs in majority of cases. • Short course of corticosteroids is beneficial. Tablet pred­ nisolone in the dose of 10 mg/day over a period of 6 weeks is given. In adults Splenectomy is indicated in the following situations: • When ITP has presented for more than 6-9 months. • When ITP has relapsed in spite of steroids, the patient is given a trial of prednisolone, 1 mg/kg/day. Platelet count rises within 7 days of starting steroids after which splenec­ tomy can be done (Key Box 26.6). • Also indicated if a patient has two relapses on steroid therapy or if the platelet count is less.

........ l!fflff.ffll-....W� • • • • • •

COMMON TYPES OF HAEMORRHAGES Epistaxis Menorrhagia Haematemesis Bleeding gums Haemarthrosis Haematuria

Fig. 26.15: Purpuric spots in the forearm

• Intracranial haemorrhage is found in 1-2% of patients but it may be the cause of death. • In 25% of cases, the spleen is just palpable (small size). • Hess's tourniquet test: More than 20 petechiae in a circle of 3 cm diameter in cubital fossa suggests purpura. PEARLS OF

WISDOM

If the spleen is big, it is not ITP. Investigations • Bleeding time is prolonged in purpura. Clotting and prothrombin times are normal. • Platelet count is reduced. • Bone marrow biopsy: Precursor of platelets (mega­ karyocytes) are increased.

Fig. 26.16: Purpuric spots on the abdomen

Spleen

Fig. 26.17: Severe sepsis with thrombocytopaenia

....... 1«amm1111111111111111� SURGERYINITP • Can be done even when platelets are as low as 10,000 cells/mm 3. • Diathermy is used to open layers of the abdomen. • Ligate splenic artery first at the upper border of pancreas after opening lesser sac. • Splenectomy is not difficult. Accessory spleens (15-30%) if present, should be removed. • Platelet transfusion may be required postoperatively, if there is bleeding. • Two-thirds of patients will be cured by surgery. • 15% will show improvement. • Remaining will not benefit.

Results 70-80% of patients respond permanently and do not require any further treatment. Counts rise above 1,00,000/mm3 in 7 days. Even if counts are not raised, recurrent bleeding rarely occurs. Haemolytic anaemia This type of anaemia results from an increased red cell destruction which occurs in the spleen. The life-span of red blood cells is also shortened. Hence, irrespective of the cause of haemolytic anaemia, these cases can benefit from splenectomy. Anaemia, jaundice and splenomegaly are the triad of haemolytic anaemia. Causes of haemolytic anaemia 1. Hereditary spherocytosis 2. Acquired autoimmune haemolytic anaemia 3. Thalassaemia 4. Sickle cell disease HEREDITARY SPHEROCYTOSIS This hereditary disorder is transmitted by either parent as Mendelian autosomal dominant. The disease is characterised by a defect in cell wall protein of the red cell, namely spectrin, which increases reflux of sodium into the cell. This causes the biconcave red cell to swell and become spherical. Hence, the name spherocytosis.

625

Consequences of spherocytes in circulation 1. The spherocyte is already weak. Adding on to this, there is a greater loss of membrane phospholipid which results in a delicate fragile spherocyte. 2. Because of the altered shape, oxygen and energy requirements ofRBCs increase which cannot be met by the spleen. In the spleen, they are destroyed and release excessive haemoglobin which is converted into bilirubin. • The amount of bilirubin produced is increased and most of it is unconjugated. It gets attached to albumin as it is lipid soluble and therefore, cannot be excreted in the urine. Hence, this type of jaundice is also known as acholuric jaundice. PEARLS OF

WISDOM

Most of this bilirubin is excreted in the biliary tree resulting in pigment stones in the biliary tree. Clinical features (Figs 26.18 to 26.21) • Commonly seen in young children but can manifest in adults also. • Seen equally in both sexes. • History of recurrent attacks of jaundice can be elicited from childhood. • Jaundice is mild, never deep, not associated with itching or bradycardia. • Pallor is an important feature due to destruction of the red cells. • Acute haemolytic crisis: It is precipitated by an acute infection or stress. Abdominal pain, nausea, vomiting, pyrexia, pallor are the features. The condition can be confused for an abdominal emergency. In severe cases, anaemia, thrombocytopenia, leukopaenia can occur. • Spleen is moderately enlarged. The liver is also palpable in a few cases. • In adults, gall stone colic and obstructive jaundice due to CBD stone can also complicate the disease process. Incidence of gall stone disease is around 50%. It is common after the age of 10. • Leg ulcers may occur as a result of anaemia. PEARLS OF

WISDOM

Young,pale,jaundiced,fatigued child with gall stones­ Think of hereditary spherocytosis. Investigations 1. Peripheral smear: Spherocytes are present. 2. Reticulocyte count is increased (15-25%): These are immature red cells which are discharged by the bone marrow due to loss of red cells. 3. Coombs' test is negative. • Serum bilirubin is mildly elevated and most of it is unconjugated.

Manipal Manual of Surgery

626

Treatment Splenectomy is the treatment including removal of accesso� spleen. • Ideally, it should be done from the age of 6 to 10 years Why after 6 years? The incidence of post-splenectom) sepsis is more when splenectomy is done within 6 years Oj age. Why before 10 years? After 10 years of age, the incidence of gall stones increases.

Fig. 26.18: Pigment stones

4. Fragility test • Normal red cell haemolysis occurs in 0.47% saline solution. Here, it occurs at 0.6% or even in weaker solution. 5. Ultrasound abdomen is done to rule out gall stones.

Splenectomy helps in the following ways (Figs 26.19 to 26.22� 1. By decreasing erythrocyte destruction, anaemia improves, thus avoiding blood transfusions. 2. Erythrocytes achieve normal lifespan. Hence,jaundice also disappears. 3. Leg ulcers heal quickly. • If gall stones are also found in these cases, laparoscopic splenectomy and cholecystectomy can be done in the same sitting. PEARLS OF

WISDOM

Hereditary elliptocytosis is also a genetic disorder wherein RBCs are oval in shape. It also responds to splenectomy.

Fig. 26.19: Case of hereditary spherocytosis with faint jaundice in a 22-year-old girl

Fig. 26.20: She also had 2 cm enlarged liver and gall stones

Fig. 26.21 : Splenomegaly-its size was 5 cm

Fig. 26.22: Ideal case-she underwent laparoscopic splenectomy and cholecystectomy

Spleen

ACQUIRED AUTOIMMUNE HAEMOLYTIC ANAEMIA Due to an autoimmune reaction, the red cell surface is damaged and it gets destroyed in the splenic red pulp. Antibodies develop against red cell antigens. In 50% of cases, the cause is not known. The following are the probable causes: • SLE, lymphoma, chronic lymphocytic leukaemia • Drugs: Methyldopa, mefenamic acid • Exposure to chemicals or infections Clinical features • More common in females after 50 years of age. • Splenomegaly, moderate in size • Gall stones are found in around 25% of the cases. Investigations • Anaemia is present • Spherocytes may be present because of damage to the red cell surface. • Coombs' test is positive Treatment • It is a self-limiting disorder. Reassurance plays an important role. • Corticosteroids: A short course of prednisolone 60 mg/day is given. • Splenectomy is done when steroid response is not satisfactory. 80% ofpatients respond to splenectomy. THALASSAEMIA It is also called Cooley's anaemia, Mediterranean anaemia, Heinz body haemolytic anaemia. Aetiopathogenesis • It is a hereditary disorder, transmitted as a dominant trait. It is characterised by a defect in haemoglobin peptide chain synthesis. Depending on type of peptide chain involvement, it is classified into ex, � and y. However, �-thalassaemia is more common. There is a decrease in haemoglobin and red cells will be destroyed prematurely due to intracellular precipitates (Heinz bodies). • When it is a heterozygous disorder, it is called beta­ thalassaemia minor and when homozygous, it is cal led beta­ thalassaemia major (Cooley's anaemia). The abnormal gene is inherited from either or both the parents. Clinical features • Severe anaemia resulting in weakness, lethargy, leg ulcers, etc. • Splenomegaly is mild to moderate in size. Sometimes, it is massive resulting in abdominal discomfort or may cause abdominal distension. • Jaundice due to haemolysis. Liver can also be enlarged. • To compensate for anaemia, bone marrow hyperplasia occurs resulting in bossing of skull and prominent malar

627

bones. Thalassaemia major usually manifests in the firs1 year of life. Complications 1. Haemosiderosis (deposition of iron in tissues) of pancreas results in chronic pancreatitis and diabetes. 2. Hepatic cirrhosis due to liver haemosiderosis. 3. Aplastic crisis with severe life-threatening infections. 4. Gall stones occur in about 20% of cases. Investigations • Peripheral smear demonstrates microcytic hypochromic anaemia. Red cells are small, thin and are resistant to osmotic lysis. • Haemoglobin electrophoresis reveals reduction or absent levels of haemoglobin A (HbA). There is a compensatory increase in foetal haemoglobin (HbF). Treatment • Blood transfusion: Multiple repeated transfusions may be necessary. • Surgical: Splenectomy is indicated in a few cases who require multiple transfusions and patients with gross splenomegaly. SICKLE CELL ANAEMIA OR SICKLE CELL DISEASE This is a hereditary haemolytic disorder in which haemoglobin A is replaced by haemoglobin S (HbS). This results in crescent­ shaped erythrocytes. This HbS molecule crystallises if there is hypoxia, strenuous work or due to dehydration. As a result of this, red cells are distorted and elongated. Therefore, blood viscosity is increased, causing obstruction in the splenic circulation (Key Box 26.7). Effect of HbS blockage • Splenic microinfarcts • Splenomegaly or autosplenectomy due to repeated infarcts (Fig. 26.23). Clinical features • The disease is very common in Africans. Most of the patients in India have high HbF levels which protect HbS. Hence, symptoms are not seen in the first few weeks.

....... w:n,mm,-.....r� FUNCTIONAL ABNORMALITIES IN SICKLE CELL ANAEMIA • Spleen sometimes acts as a large reservoir for red cells and the sickle cells are destroyed. • Antibody production is decreased. • Spleen's ability to filter Streptococcus pneumoniae is reduced.

Manipal Manual of Surgery

628 Summary of haemolytic anaemias Aetiology

ShapeofRBC

Spl een

Investi gation

Treatment

I. Hereditary spherocytosis

Increase in red cell permeability to sodium

Spherocyte

Large

Reticulocyte count

Splenectomy very beneficial

2. Autoimmune haemolytic anaemia

SLE, drugs

Spherocyte

Medium size

Coomb's test is positive

Steroids: splenectorny in steroid failure cases

3. Thalassaemia (Cooley's anaemia)

Reduction of �-chain of haemoglobin

Small, thin and no shape

Moderate size

Resistance to osmotic lysis Electrophoresis HbA

Splenectomy for bulky spleen and in patients requiring frequent blood transfusion

4. Sickle cell

HbA is replaced by HbS

Sickle shape

Mild and later not palpable

Electrophoresis HbS

If hypersplenism +, splenectomy

CM 6

7

• Splenectomy has a doubtful value. It is indicated only when excessive numbers of RBCs are sequestrated causing anaemia (Table 26.1 for comparison). Complications • Infarct of cerebrum resulting in hemiplegia. • Mesenteric infarction resulting in gangrene of the bowel. • Pulmonary infarction resulting in chest pain. SPLENECTOMY FOR OTHER CONDITIONS

8

Fig. 26.23: Sickle cell anaemia-Nugget spleen (3 cm size). An example for autosplenectomy

• Abdominal pain is due to recurrent infarcts or due to gall stones in a few patients. • Skin ulcers occur due to anaemia or hypoxia. PEARLS OF

WISDOM

Microinfarction of carpal and tarsal bones resulting in bony pains is described as hand-foot syndrome. Investigations • Peripheral smear demonstrates sickle cell. • Haemoglobin electrophoresis to identify the type of haemoglobin. Treatment • Folic acid supplements

1. Hypersplenism It is defined as pancytopaenia in the presence of normal or hypercellular bone marrow. Following are the causes of hypersplenism: • It usually involves red pulp expansion. It is to be remembered that in cirrhosis of the liver, 15% of patients develop hypersplenism. Anaemia and thrombocytopaenia are mild. Rarely requires splenectomy (a few causes are given below): 1. Tropical splenomegaly due to malaria, kala-azar or schistosomiasis, etc. Since these diseases are endemic in tropical countries, it is called tropical splenomegaly. Gross enlargement of the spleen and hypersplenism are indications for splenectomy in such cases. 2. Myeloproliferative disorders: Splenectomy reduces need for transfusion and m ay relieve abdominal discomfort. 3. Portal hypertension: Maximum benefit is in segmental portal hypertension with oesophagogastric varices (Fig. 26.24). 4. Genetic: Gaucher's diseases 5. Neoplastic infiltration 6. Inflammatory disease: Sarcoidosis, lupus erythematosus. 7. Chronic infections such as tuberculosis, brucellosis and malaria.

Spleen

629

8. Chronic haemolytic diseases-spherocytosis, thalas­ saemia, glucose-6-phosphate dehydrogenase deficiency, elliptocytosis. 2. Cysts of the spleen (Figs 26.25 and 26.26) • Parasitic cyst is rare. Typically, it is caused by echino­ coccal disease (hydatid disease). • Traumatic cysts are due to a haematoma giving rise to liquefaction. These are false cysts of the spleen. • Congenital cysts can be due to a dermoid cyst, haeman­ gioma or lymphangioma. • Better save the spleen by deroofing cyst wall.

Fig. 26.24: Splenectomy done for left-sided portal hypertension due to splenic vein thrombosis

Fig. 26.25: Simple cyst of the spleen resulting in compression of the spleen-appearance like that of a globe

Fig. 26.26: Splenic cyst coronal view

3. Tumours of the spleen/neoplasms (Fig. 26.27) • Among neoplasms, lymphoma is the commonest cause of enlargement of spleen. Spleen used to be removed as a part of a staging laparotomy. Now, it is very rarely removed. Patients with large spleen of chronic myeloid leukaemia, Gaucher's disease and hairy cell leukaemia (details later) will also benefit from splenectomy. • Fibrosarcoma or angiosarcoma are rare malignant tumours. • Haemangioma is the most common benign tumour of the spleen. It may turn into haemangiosarcoma. • Spleen is removed in oesophagogastrectomy or upper carcinoma stomach-only when it is directly infiltrated. Sometimes it happens in carcinoma tail of pancreas/ splenic carcinoma. 4. Other indications for splenectomy (Figs 26.28 to 26.31) • Splenic abscess can occur due to infected septic emboli from otitis, typhoid fever or due to thrombosis of splenic vein causing infarction followed later by infection

Fig. 26.27: Spleen removed with left hemicolectomy-advanced carcinoma

630

Manipal Manual of Surgery

Fig. 26.30: Incision of the abscess after splenectomy Fig. 26.28: CT scan showing multiple splenic abscesses

Fig. 26.29: Same patient as in Fig. 26.28 with multiple abscesses in the spleen

(Key Box 26.8). Tubercular abscess of the spleen is rare. It is usually affected secondary to abdominal or pulmonary tuberculosis. • Felty's syndrome refers to splenomegaly, neutropenia with rheumatoid arthritis. After splenectomy, leg ulcers heal quickly and neutropenia also improves. The patient may respond to steroids better and the incidence of recurrent infection becomes less (Fig. 26.32).

Fig. 26.31: Splenic abscess adhered to diaphragm and pre-renal fascia. In ITP, there will not be adhesions

....... lfflwms: -.alllllr! , CAUSES OF SPLENIC ABSCESS Salmonella infection Paratyphi infection Labour-puerperal sepsis Ear infection-otitis Necrosis of pancreas Infection of bone-osteomyelitis Chronic disease-tuberculosis Remember as SPLENIC

Fig. 26.32: Felty's syndrome. The patient had received steroids for one year. See the Cushingoid facies

Spleen

631

Figs 26.33 to 26.35: A 28-year-old man presented with pain abdomen. CT scan showed splenic artery aneurysm with haematoma and thrombus suggestive of ruptured splenic artery aneurysm. He also had splenomegaly and features of portal hypertension. He was alcoholic. He might have had pancreatitis and developed splenic artery aneurysm (Courtesy: Prof Sampath Kumar, Dr Basavaraj Patil, Dr Dinesh, Dr Pawan Kumar Addala, Department of Surgery, KMC, Manipal)

5. Splenic artery aneurysm It is an uncommon condition. More common in women, and some of them occur during pregnancy. Causes • Atherosclerosis: Commonly seen in elderly patients • Congenital: Young patients. These patients may present for the first time during pregnancy with enlargement or rupture. • Acute pancreatitis: Inflammatory process may give rise to pseudoaneurysm specially because of pancreatic necrosis. Clinical features • Pain and vomiting-haematemesis • Thrill or bruit • Calcification in a routine X-ray • Rupture and features of haemorrhagic shock. Investigations (Figs 26.33 to 26.35) • Ultrasound/CT scan • Angiography Treatment • Nonoperative: Embolisation of splenic artery. • Operative: Ligation of splenic artery at the upper border of pancreas after opening lesser sac followed by splenectomy. • Rupture: Carries high mo1tality rate. 6. Hairy cell leukaemia (HCL) (Fig. 26.36) • The cells on a blood smear have an irregular outline due to the presence of filament-like cytoplasmic projections. Hence, the name hairy cells. • HCL is a clonal proliferation of abnormal B cells (very rarely T cells).

Fig. 26.36: Patient had pyrexia of unknown origin with pancytopaenia. Spleen was enlarged. Splenectomy was done and the biopsy report was non-Hodgkin's lymphoma

Clinical features • Anaemia • RecmTent infection • Massive enlargement of spleen. Investigations • Blood cow1ts are low. White cell count may be raised with circulating hairy cells. • Bone marrow has increased cellularity with char­ acteristic infiltration by hairy cells. Treatment • Drug: 2-Chloradenosine acetate (2-CDA) has been beneficial. It can induce rem1ss1on. • Splenectomy is indicated in cases of diagnostic difficulties and in very large spleens (Fig. 26.37).

Fig. 26.37: Splenectomy was done for large spleen causing discomfort, reported as hairy cell leukaemia

632

Manipal Manual of Surgery j Splenectomy-benefits j

I

I

Maximum

Moderate

1. Rupture of the spleen 2. Hereditary spherocytosis 3. Idiopathic thrombocytopaenic purpura (ITP) 4. Cysts of the spleen 5. Tumours of the spleen 6. Hypersplenism 7. Left-sided portal hypertension

1. Tropical splenomegaly 2. Autoimmune haemolytic anaemia 3. Felty's syndrome

Mild 1. 2. 3. 4. 5. 6.

Chronic myeloid leukaemia Thalassaemia Sickle cell disease Gaucher's disease Hodgkin's lymphoma Hairy cell leukaemia

Fig. 26.38: Benefits of splenectomy

OPSI Overwhelming postsplenectomy infection (OPSI): Incidence of meningococcal and pneumococcal infections is more in children after splenectomy. Hence, pneumococcal vaccine must be administered in the postoperative period. • Following splenectomy, especially children are vulnerable for opportunistic infections such as Pneumococcus, H. influenzae and Meningococcus. Hence prophylaxis is necessary. • OPSI can also be a major problem in adults who undergo splenectomy for different conditions. Following are the recommendations; to avoid OPSI (Key Box 26.9).

llttll-......r,, HOW TO AVOID OPSI?

�

• Offer education-about spleen, role of splenectomy, side effects of splenectomy-especially in children. • Prophylactic antibiotics before splenectomy. • Speedy (prompt) treatment of infection. Early control of 'sepsis' is the 'key' to success. • Immunisation must be timely Remember as OPSI

I. Elective splenectomy • All children who undergo splenectomy before 5 years of age, should receive daily dose of penicillin until 10 years. • Haemophilus injluenzae type B vaccination for all irrespective of age and pneumococcal vaccine to be given (repeated once in 5 years). • Meningococcal protection for only those who travel to high-risk areas. P EA RLS OF

WI SDOM

Vaccination should be given at least 2 weeks before elective splenectomy.

II. After emergency splenectomy • Vaccines should be given as early as possible. However protection is not always guaranteed. • Daily dose of oral prophylaxis with penicillin erythromycin or amoxycillin to all children till 10 yean who undergo splenectomy before the age of 5. Benefits of splenectomy (Fig. 26.38) INTERESTING 'MOST COMMON' FOR SPLEEN • Haemangioma is the most common benign tumour of the spleen. • Lymphoma is the commonest cause of enlargement of spleen (in India, malaria is the cause). • It is the most common solid organ injured in blunt abdo­ minal trauma. • Most common cause for splenic rupture is blunt abdominal trauma. • Most significant benefit of doing a splenectomy for hyper­ splenism is from segmental left-sided portal hypertension. • Most commonly performed investigation for suspected splenic trauma is abdominal ultrasound. • Most common malignancy affecting spleen is lymphoma • Most common complication of splenectomy is pulmonary complication • Most common neoplasm of spleen-lymphoma • Most common primary tumour of spleen-Haemangioma • Most common primary malignant tumour of the spleen­ haemangiosarcoma • Most common infection after splenectomy is by Strepto­ coccus pneumoniae • Most common indication for splenectomy-splenic trauma • Most common indication for splenectomy in elective setting-lTP. WHATISNEWIN THIS CHAPTER?/RECENTADVANCES

• Splenic injury grades are added • All topics have been updated • New key boxes are added

Spleen

633

MULTIPLE CHOICE QUESTIONS l. In splenic injury, Kehr's sign refers to: A. Absence of shifting dullness on the left side of abdomen B. Tenderness on the left side between sternocleido­ mastoid and scalenus medius muscle C. Tender left iliac fossa D. Shoulder pain due to irritation of the under-surface of the diaphragm 2. Spontaneous rupture of the spleen is seen in the following except: A. Idiopathic thrombocytopenic purpura B. Malaria C. Infectious mononucleosis D. Leukaemia 3. Diagnostic peritoneal lavage is said to be positive in all of the following except: A. Fresh blood> 20 ml is aspirated after inserting dialysis catheter B. Gram stain is positive in the contents of the lavage C. RBC count > 100,000 cells/cu mm D. Amylase level of 75 units/di 4. The following is not true about nonoperative treatment in splenic trauma except: A. Hemodynamically stable patient. B. Hollow viscus injury C. Close monitoring is available D. Hospitalisation is required 5. Big spleen is not a feature of: A. Malaria B. Idiopathic thrombocytopaenic purpura C. Portal hypertension D. Leukaemia

6. The following is true about purpura: A. Bleeding time is prolonged B. Clotting time is prolonged C. Prothrombin time is prolonged D. Activated partial thromboplastin time is prolonged 7. Which of the following vaccination has to be given after splenectomy to prevent overwhelming p ost­ splenectomy infection? A. Measles vaccine B. Pneumococcal vaccine C. Hepatitis vaccine D. Varicella vaccine 8. The following is true about splenectomy in idiopathic thrombocytopaenic purpura (ITP): A. Is required in all cases of ITP B. Must be done when platelet count is > I 00,000 cells/ cu mm C. Accessory spleens, if present must be left behind D. Two-thirds of patients will be cured by surgery 9. The following is true about splenectomy for hereditary spherocytosis: A. Splenectomy should be done as a last resort B. Splenectomy must be done either before 6 years or after ten years of age C. Accessory spleen, if present must be left behind D. Jaundice disappears after splenectomy 10. The most common benign tumour of the spleen is: A. Haemangioma B. Lymphoma C. Tuberculoma D. Lipoma

ANSWERS 1 D

2 A

3 D

4 8

5 8

6A

7 8

8 D

9 D

10 A

27

Peritoneum, Peritoneal Cavity, Mesentery and Retroperitoneum • • • • • • •

Peritoneum Acute peritonitis Laparostomy Abdominal compartment syndrome Subphrenic abscess Special types of peritonitis Omentum

Introduction Peritoneal cavity is the largest cavity in the body accommodating various viscera. It is divided into greater and lesser sac (omental bursa) which communicate through the foramen of Winslow or epiploic foramen. The peritoneum lining inner side of the parietes is called parietal peritoneum. It is very sensitive and is innervated by both somatic and visceral afferent nerves. This explains the sharp, localised, cutting pain of peritonitis. Diaphragm and central part of the peritoneum is supplied by phrenic nerve (C4) and partly by intercostal nerves. Rest of the peritoneum is supplied by intercostal nerves and lumbar nerves. Lesser omentum: It is also called hepatoduodenal ligament. It extends from the duodenum to the liver. This has two layers and within these layers are the common bile duct, hepatic artery and hepatic portal vein. THE PERITONEUM Lining of peritoneum The peritoneum is lined by a single layer of flattened cells and a thin layer offibroelastic tissue. It is parietal peritoneum. A large peritoneal defect heals within a few hours because of these mesenchymal cells (flattened polyhedral cells­ mesothelium). Applying this principle, some surgeons do not close the peritoneal layer after laparotomy. When parietal peritoneum is reflected into viscera, it is called visceral peritoneum.

• Mesentery • Misty mesentery • Mesenteric cyst • Retroperitoneum • Retroperitoneal cyst, abscess, tumour • What is new?/Recent advances

It covers viscera and is supplied by autonomic nervous system. Hence, it is not sensitive. Thus, gastrojejunostomy can be done under local anaesthesia but distension and traction to the bowel causes pain. During herniorrhaphy under spinal anaesthesia, handling of bowel or traction on the bowel can produce uncomfortable upper abdominal pain. Fluid Peritoneal surface is a semipern1eable membrane with an area comparable to that of cutaneous body surface. Nearly 1 m2 of the total 1.7 m 2 area participates in fluid exchange with extracellular fluid space at the rate of 500 ml or more per hour. It normally contains less than 50 ml fluid. When it is insulted by infection, a large amount of fluid can collect in this space giving rise to severe fluid and electrolyte imbalance. This is described as III space loss, e.g. peritonitis,pancreatitis. Peritoneal fluid helps in smooth gliding of intestines. Absorption of fluid and secretion of fluid are some important functions of peritoneum (Key Box 27 .1).

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• • • • •

�

NORMAL PERITONEAL FLUID-TRANSUDATE Specific gravity below 1016 Protein concentration less than 3 gm/dl White blood cell count less than 3000/µL Complement mediated bacterial activity Lack of fibrinogen related clot formation

Peritoneum, Peritoneal Cavity, Mesentery and Retroperitoneum

Absorption and exudation This takes place through capillaries and lymphatics present in between the two layers of peritoneum. This principle is applied i n dialysis. The direction of circulation is towards subdiaphragmatic lymphatics. Protective function It secretes prostaglandins, interferons and free radicals which help in some protection against peritonitis. ACUTE PERITONITIS Definition: Inflammat ion of the peritoneum is called peritonitis. Causes: They can be classified into primary or secondary. I. • • • •

Primary peritonitis Spontaneous peritonitis of childhood Spontaneous peritonitis of adults Tuberculous peritonitis Peritonitis associated with dialysis

II. Secondary peritonitis This term refers to peritonitis from an intra-abdominal source and is the most common form of peritonitis. The following are the causes for secondary peritonitis (Fig. 27.1 ): 1. Perforation of a hollow viscus • Perforated duodenal ulcer, gastric ulcer • Perforated enteric ulcer, tubercular ulcer • Perforated Meckel 's diverticulum • Perforated colonic ulcer 2. Direct spread: Post-inflammatory • Acute cholecystitis-gangrenous • Acute appendicitis • Gangrene of the intestine • Acute necrotising pancreatitis 3. Penetrating injuries to the abdomen, where the organisms gain entry from outside. 4. Postoperative peritonitis is due to the introduction of infection during surgery which might be due to: • Postoperative leaks • Foreign body (mop) in the abdomen 5. Parturition peritonitis: It refers to peritonitis after pregnancy and delivery. 6. Blunt injuries to the abdomen • Fluid which is spilled into the peritoneal cavity (example: Blood and bile can travel along paracolic gutter and manifest as pain in the right iliac fossa, causing guarding

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and rigidity. This has been called Valentino syndrome (see later in page 637). PATHOGENESIS (Fig. 27.2 and Flowchart 27.1) Due to any one of the reasons mentioned above, infection sets in and the causative organisms multiply in the peritoneal cavity. • Gram-negative organisms: Escherichia coli (E. coli), Proteus, Klebsiella. They are present in the small and large bowel. They are the commonest organisms producing peritonitis (Key Box 27.2). • Enterococci: Streptococcus faecalis needs bile to grow. It is present in the urinary tract, genital tract and also in the intestines. However, both aerobic and anaerobic streptococci are the second most common organisms producing peritonitis. They are the chief organisms in puerperal sepsis. • Bacteroides: They are anaerobic organisms, present mainly in the lower intestine. • Bacteria from outside alimentary canal: Gonococci, pneumococci, tubercular organisms, etc. • These organisms proliferate in the peritoneal cavity resulting in peritonitis. As a result of this, there is secretion of a large amount of fluid into the peritoneal cavity resulting in 3rd space loss which leads to severe hypovolaemic shock. This fluid is rich in proteins, bacteria and toxins. Due to powerful endotoxins released by gram-negative bacteria, endotoxic shock or septic shock (refer to shock), ensues. • The fluid is rich in fibrinogen which forms fibrin and helps in localisation of infection (Fig. 27.3). • Peritoneum loses its shiny surface, becomes reddish and oedematous and is covered with thick fibrinous exudate. • Omentum: It is a fatty apron with rich blood supply. A mobile double-layered peritoneal fold acts like a policeman to seal the area of infection or perforation. Examples: Perforated duodenal ulcer, acute appendicitis, acute diverticulitis, etc. Probably it also serves to supply collateral blood supply to the ischaemic viscera. It also has immunological functions such as supply of phagocytes which destroy unopsonised bacteria.

N$MlWIIIIIIW'� ORGAN-SPECIFIC ORGANISMS • Gastric perforations: Sterile with minimum gram-positive organisms. • lleal perforation, appendicitis-aerobic bacteria in 30% patients, anaerobic in 10% patients. Predominant aerobic bacteria include gram-negative E.coli, Streptococci, Proteus and Klebsiella. • Colonic-rectum: Faecal spillage produces a load of 10 12 or more-gram-negative and anaerobic bacteria per gram of stools.

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Common causes of perforation 1. Perforated duodenal ulcer 2. Perforated gastric ulcer 3. Perforated appendicitis 4. Perforated enteric ulcers 5. Perforated tubercular ulcers 6. Gangrene intestine 7. Perforated carcinoma left colon

Uncommon causes of peritonitis 8. Perforated gall bladder 9. Ruptured amoebic liver abscess 10. Necrotising pancreatitis 11. Perforated Meckel's diverticulum 12. Sigmoid diverticular perforation

Fig. 27.1: Common causes of generalised peritonitis

PERFORATION, POSTINFLAMMATORY, POSTOPERATIVE Multiplication of bacteria in the peritoneal cavity-E. coli, Enterococci, Bacteroides

Peritoneal irritation

Endotoxins

Exudate collects, 3rd space loss Septic shock Hypovolaemic shock Multiorgan failure 1.----; Renal failure Death

Fig. 27.2: Pathophysiology of peritonitis

Fig. 27.3: Fibrin plaques: Early cases of peritonitis with fibrin plaques all over the peritoneal cavity

Peritoneum, Peritoneal Cavity, Mesentery and Retroperitoneum Flowchart 27 .1: Pathogenesis of peritonitis Peritonitis-tissue damage-capillary leak-discharge of histamine and other vasoactive substances Protein (fibrinogen rich) fluid into ECF Fibrinogen rich fluid helps in destruction of bacteria Tissue thromboplastin released from mesothelial cells converts fibrinogen to fibrin, thus forms fibrous adhesions Tissue oedema Paradoxical intravascular hypovolaemia Hypotension 3rd space sequestration

•

Ce11--........i•

e

Interstitial fluid

Fluid sequestration

Types of peritonitis A. Local peritonitis: Ifa perforation is small and ifit is sealed off immediately by omentum, it will give rise to local peritonitis. Examples: Small gastric ulcer perforation or diverticular perforation, gall bladder perforation. Anatomical factors also play a role in local peritonitis. Examples: Retrocaecal appendicitis with perforation. It is behind the caecum and in retroperitonaeum. Signs are confined to right iliac fossa only. In posterior gastric perforations or acute pancreatitis, signs are limited to upper abdomen. Pelvic peritonitis is another example-occur following septic abortions or salpingo-oophoritis. B. Generalised peritonitis: If the contents of the viscus leak into the peritoneal cavity with force, as it occurs in intestinal perforation or due to perforation of a free lying organ­ example: Meckel's diverticular perforation. Virulence of bacteria is more as in colonic perforations with generalised peritonitis. Duodenal ulcer perforation can manifest as severe pain in the right iliac fossa mimicking appendicitis.

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Many have been operated as appendicitis. Reason being a broad right paracolic gutter and the contents travel down into right iliac fossa. This has been referred to as Valentino syndrome (Key Box 27.3).

.,.,___.,� VALENTINO SYNDROME-VALENTINO APPENDIX

• Rudolph Valentino was an Italian actor who lived in early 20th century. • On August 15, 1926, he was admitted with the diagnosis of appendicitis and gastric ulcers, with peritonitis. • He underwent appendicectomy. • Continued to have peritonitis • He developed pleural effusion and sepsis • Dies after a few days of surgery. • In retrospect what he had was duodenal ulcer perforation. • It is also called Valentino syndrome because in any inflammation of the upper abdominal viscera, contents can travel down the right paracolic gutter into the right iliac fossa resulting in pain and tenderness mimicking acute appendicitis.

FACTORS DECIDING THE SEVERITY OF PERITONITIS • Clean perforation: Upper GI-Gastric juice remain sterile for 6-8 hours. Hence, in early stages, there will be mild chemical peritonitis and early treatment is give good results. • Distal gut perforation and infected bile peritonitis: Very dangerous and severe, causing sepsis and septic shock early. • Postoperative peritonitis that usually occurs due to anastomotic leak is also dangerous. • A perforation sealed off early by omentum causes mild peri­ tonitis. Retrocaecal appendicitis produces minimal local peritonitis • On the other hand, perforated Meckel 's diverticulitis produces diffuse peritonitis soon (Key Box 27.4). • A few causes ofperitonitis are shown in Figs 27.4 to 27.18B.

Mtfflt--..r� FACTORS AFFECTING DIFFUSE PERITONITIS

• Speed of peritoneal contamination, e.g. perforation of Meckel's diverticulum • Stimulation by purgatives • Virulence of organisms • Perforation in a closed loop obstruction • lmmunocompromised status • Young children. Omentum is thin and small Clinical features It depends upon whether 1t 1s localised periton1t1s or generalised. In cases ofretrocaecal appendicitis, the abdominal signs may be minimal but guarding and rigidity of the back muscles is characteristic. Features of generalised peritonitis are as follows:

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A FEW CAUSES OF PERITONITIS

Fig. 27.4: Meckel's diverticulitis

Fig. 27.5: Peritonitis due to ileal perforation consequent to tuber­ culosis. Usually it is an ulcerative variety

Fig. 27.6: Transverse colon injury due to steering wheel-simple closure in early cases without much peritonitis. Otherwise resec­ tion/closure with or without diversion ileostomy may be required

Fig. 27.7: Enteric perforation which is 4 days old-very friable edges. Re-leak after suturing is common

Fig. 27.8: Pancreatic necrosis-necrosectomy is done-you can see spoon has been used to remove necrotic tissue. Patient was in sepsis and peritonitis. Dramatic recovery took place

Fig. 27.9: Chronic duodenal ulcer perforation resulting in biliary peritonitis. Golden time to operate is within 6 hours before bacte­ rial peritonitis sets in

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639

A FEW CAUSES OF PERITONITIS

Fig. 27.10: Postoperative peritonitis due to a mop left behind fol­ lowing caesarean section. Always count the mops and instruments before closure of the abdomen. It is a good practice

Fig. 27.11: Fibrous band causing gangrene of the terminal ileal loop resulting in peritonitis. Patient had pelvic peritonitis due to tuberculous salpingitis. That resulted in bands

Fig. 27.12: Sigmoid diverticular perforation causing peritonitis. Sigmoid colon is the most common site of acquired diverticuli

Fig. 27.13: Faecal peritonitis and faecal fistula due to anasto­ motic leak following right hemicolectomy. lschaemia and tension are the two common causes for the leak

Fig. 27.14: Proximal jejunal transection following blunt abdomi­ nal trauma. It is one of the common sites affected in blunt ab­ dominal trauma

Fig. 27.15: Colostomy gangrene -the intra-abdominal segment was also gangrenous. It is important to always check for the vas­ cularity of the colostomy site before closing abdominal incision

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Manipal Manual of Surgery

Fig. 27.16: Retracted colostomy-intra-abdominal gangrenous segment of the intestine causing peritonitis. It was resected

Fig. 27.188: Massive gangrene following superior mesenteric arterial occlusion-patient had severe acidosis

Fig. 27.17: Necrosectomy specimen-acute pancreatitis

• Severe abdominal pain which is cutting in nature, becomes worse on movement of the abdominal wall. Hence, the patient lies still on the bed. • Persistent vomiting is due to irritation of parietal peritoneum. • The pulse rate is increased. An increase in the pulse rate may be an early indication of peritonitis, in cases of gangrene of the bowel or peritonitis following perforation of bowel. • High-grade fever with chills and rigors indicates a septicaemic process. • Cough tenderness indicates parietal peritoneal infla­ mmation. Abdominal tenderness is elicited in all quadrants of the abdomen. • Rebound tenderness (Blumberg's sign): Abdomen is pressed for a few seconds. The patient experiences pain. Sudden release of pressure causes severe pain. It is due to sudden movement of the sensitive parietal peritoneum (Fig. 27.19). • Guarding and rigidity of abdominal wall. • Bowel sounds are absent. Distension of the abdomen occurs within a few hours due to accumulation of fluid and paralytic ileus. • End-stage disease: Hippocratic facies (Key Box 27.5 and Fig. 27.20). Investigations

Fig. 27.18A: Mesenteric ischaemia giving rise to gangrene

t. Complete blood picture shows high total count with predominant neutrophil count. 2. Blood examination for sugar is done to rule out diabetes mellitus. Empyema gall bladder with or without perforation can present as septic shock. Often, they are diabetic. 3. Plain X-ray abdomen, chest and upright • Gas under the diaphragm-perforation (Figs 27.21 to 27.23)

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641

Mtllt-.r'� HIPPOCRATIC FACIES

• • • • •

Hollow, bright eyes Pale and pinched face Cold perspiration in the head and brows Blue lips Dry, cracked tongue

Fig. 27.21: Chest X-ray showing free gas under diaphragm

Fig. 27.19: Rebound tenderness is the diagnostic sign of peritonitis

Fig. 27.22: Lateral decubitus X-ray showing free gas in the peri­ toneal cavity

Fig. 27.20: Hippocratic facies: Sunken eyes, drawn in cheeks, dehydrated, blood in the Ryle's tube

• Ground glass appearance-a smooth homogeneous appearance due to accumulation of fluid (Fig. 27.24). • Air in the bowel wall-gangrene (Fig. 27.25) • Obliteration of psoas shadow and preperitoneal fat planes. 4. Abdominal USG to detect fluid in the abdomen. Following are different fluids which may give clue to the diagnosis • Frank pus-peritonitis of more than 48 hours old

Fig. 27.23: Diaphragm is elevated-fundic air bubble is in the chest-traumatic diaphragmatic hernia

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Fig. 27.24: Subphrenic

Manipal Manual of Surgery

Fig. 27.25: Gas in the bowel wall

abscess-mild pleural effusion indicates gangrene of the bowel on both sides

• Bile-green coloured-duodenum, stomach, gall bladder perforation • Faeculent-dark green coloured thick aspirate with faecal odour-ilea! perforations, postoperative anastomotic leaks • Serous-exudative-early acute pancreatitis, tuberculous peritonitis • Haemorrhagic-haemorrhagic pancreatitis • Food particles-hollow viscus perforation Thus ultrasound has so many advantages even though it may not point at the specific site. However, probe tenderness with fluid in the right iliac fossa may suggest acute appendicular perforation. Very thick contents such as anchovy sauce from ruptured amoebic liver abscess cannot be aspirated. However, ultrasound will give clue about the liver abscess. 5. Abdominal tap • Aspiration of blood indicates haemoperitoneum or gangrene of the bowel. • Aspiration of bile indicates biliary peritonitis due to perforation of duodenal ulcer, gall bladder or intestine.

Fig. 27.26: Diagnostic tap showing pus following rupture of empyema of gall bladder

• CT can diagnose unsuspected and unexpected lesions in the abdomen including diverticular perforations, internal herniation and gangrene, acute pancreatitis, etc. • Adequate hydration and normal renal function (as indicated by normal creatinine values) are important before a contrast-enhanced CT scan.

Fig. 27.27: CT showing air in the bowel wall

• Aspiration of frank pus indicates peritonitis due to gram-negative bacteria. Foul-smelling pus is due to anaerobic bacteria producing free fatty acids and their esters. Always send the fluid for culture sensitivity (Fig. 27.26). • Amylase estimation should be done to rule out pancreatitis. 6. Contrast-enhanced CT scan • When the signs and symptoms are equivocal, CT is the ideal investigation. • CT can diagnose hollow viscus perforation, especially when there is no gas under the diaphragm. • CT can detect ischaemic changes due to gangrene of the bowel-gas in the bowel wall (Figs 27.27 and 27.28)

Fig. 27.28: CT showing hypodense lesion in the left iliac fossa­ a case of sigmoid diverticular perforation

Peritoneum, Peritoneal Cavity, Mesentery and Retroperitoneum

7. Diagnostic laparoscopy can be used in suspected cases of peritonitis (Key Box 27.6).

M$MIWIIIIIW',1 W DIAGNOSTIC LAPAROSCOPY

• It can be used to reconfirm peritonitis • It can diagnose pancreatitis (laparotomy may be avoided) • It can also treat primary cause, e.g. laparoscopic closure of duodenal ulcer perforation • Peritoneal toilet can be given • It can rule out other causes • In blunt injury, it can detect diaphragmatic injury­ herniation of bowel, etc.

PEARLS OF

WISDOM

When in doubt, do laparoscopy: It can reveal 'hidden' pathology.

Treatment 1. Aspiration: Nasogastric aspiration with Ryle's tube helps in decreasing gastrointestinal secretion. Thus it reduces abdominal distension. It also prevents vomiting and gives rest to the gut. lndirectly it reduces 'bacterial load' contaminating peritoneum. 2. Bowel care and blood: Purgatives should not be given as it may result in perforation. Blood is arranged for surgery. 3. Charts: Temperature, pulse rate, respiratory rate, intake­ output charts are maintained. 4. Drugs are given against gram-positive, gram-negative and anaerobic organisms (Key Boxes 27.7 and 27.8).

ll$mXWIIIIIW'� SELECTION OF ANTIBIOTICS

• 2nd or 3rd generation cephalosporins should be started as early as possible • Once culture and sensitivity reports are available (after surgery), antibiotics can be changed • Antibiotics should also cover aerobes and anaerobes • Should not have serious toxicity, especially amikacin which has nephrotoxicity. Hence, to be used carefully (check creatinine) 5. Exploratory laparotomy and appropriate surgery is done followed by thorough peritoneal toilet/wash with normal saline. 6. Fluids-IV fluids are given before, during and after surgery. Central venous cannulation and measurement of central venous pressure (CVP) is indicated in unstable patients to guide fluid therapy. If not possible, an emergency cut down

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(venesection)-cephalic or basilic vein, is done followed by fluid infusion. Preoperatively the aim is to maintain at least 30 ml/hr of urine output. • Ringer lactate solution is an ideal replacement.

ll$mlWIIIIIW'� EARLY AGGRESSIVE RESUSCITATION

• Restore intravascular volume. Crystalloids: Ringer lactate or isotonic saline stay in the intravascular space for a short period, larger volumes required Colloids: Longer duration of action, smaller volumes are sufficient and can be used in cardiac patients • Restore oxygenation by face mask or mechanical ventilation, as necessary • Restore perfusion: Dopamine/dobutamine/noradrenaline. • Restore normality by 'war' against sepsis-ANTIBIOTICS and surgical removal of SEPSIS Principles of surgery for peritonitis (Key Box 27.9, Figs 27.29 to 27.33) I. Generous incision is used 2. As soon as the peritoneal cavity is opened, purulent fluid comes out. The fluid is collected and sent for culture and sensitivity. Greenish fluid indicates a hollow viscus perforation. All the fluid is drained, the source of peritonitis is identified and appropriate surgical procedure is done. Examples are • Appendicectomy for appendicitis. • Closure of perforation for perforated peptic ulcer. • Closure or resection for ilea! perforation. • Resection of the bowel for gangrene. Control of sepsis: This is the most important step of treatment of peritonitis. Removal of septic focus is a primary aim-examples: Appendicectomy, perforation closures (dudodenal ulcer) or resection (intestinal or colonic perforation) or cholecystostomy in difficult perforated gall bladder diseases. However, all the septic foci in the abdomen have to be removed-necrotic material, pus pockets and food pa1iicles. Thorough irrigation with warm saline cleans up subhepatic spaces, pelvic spaces and interloop collections. Primary anastomosis in presence of sepsis may

M$MiWIIIIIW',1 � PRINCIPLES OF SURGERY

• • • • • • • •

Incision Establish the diagnosis Exploration Pus culture and sensitivity Treat the cause-control of sepsis Peritoneal toilet Drain Closure

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.......

IMIJVI,._,,� PERITONEAL LAVAGE

°'8l

• Used in diffuse peritonitis • 3-5 litres of isotonic crystalloid solution is used. • Avoid antibiotic solution or povidone iodine solutions-they may induce more adhesions. • Aminoglycoside lavage may cause respiratory depression due to neuromuscular blocking action of these drugs. Mops must be used to dry the peritoneal cavity. If fluid is left over, it may dilute the opsonins and thus decrease phagocytosis.

Fig. 27.29: Laparoscopy showing pus

Fig. 27.31: Tension sutures

Peritonitis

Fig. 27.30: A late case of duodenal ulcer perforation who presented with peritonitis with sympathetic pleural effusion. Laparotomy, closure of perforation and feeding jejunostomy were done. Drains in the subhepatic space and right pleural cavity were placed. Patient had stormy postoperative period with biliary fistula, which was closed after 6 weeks

result in leak and postoperative peritonitis. It is better to do colostomy or ileostomy in such cases. Incision can be partially closed leaving the skin open-sutures can be tied after 2 days in the ward. In a few cases, laparostomy is done if you suspect abdominal compartment syndrome. 3. It is better to use nonabsorbable suture material such as silk to do an intestinal anastomosis or for closure of perforation. In the presence of infection, absorbable sutures such as catgut get absorbed very fast. 4. A thorough peritoneal wash/lavage is given by using warm saline (up to 3-5 litres) to avoid intraperitoneal abscesses. Antiseptic agent such as betadine solution should be avoided because they can cause adhesions (Key Box 27 .10). 5. Peritoneal cavity is drained to the exterior by using tube drains. These are kept in the subhepatic space and in the pelvic cavity.

Fig. 27.32: Zip closure of peritoneum

Resuscitation Diagnosis Exploratory laparotomy Treat accordingly Some examples

Closure of perforation

Appendicectomy Diverticulectomy

Intestinal resection

Sigmoid colectomy

Also drain the peritoneal cavity-soft s11111p drain wit!, multiple hole and co11tin11011s suction.

Fig. 27.33: Principles of management of peritonitis-identifying the source of sepsis and elimination is the key to success

Peritoneum, Peritoneal Cavity, Mesentery and Retroperitoneum

6. The wound is irrigated with antiseptic agents. 7. Tension sutures are put depending upon the severity of the peritonitis to prevent burst abdomen. 8. Laparostomy (vide infra): This method of exposing the peritoneal cavity can be done in selected cases, if you suspect abdominal compartment syndrome. LAPAROSTOMY This refers to leaving peritoneal cavity exposed to outside without approximation of the anterior abdominal wall. Some situations arise, especially in emergency cases, where this is required. Hence, it is important to know how to deal with this situation. Types 1. Open laparostomy: Abdominal fascia and peritoneum are not sutured. Advantages: Abdominal compartment syndrome can be prevented. Details are given later. Disadvantages: Significant fluid loss and secondary infection. 2. Closed laparostomy or mesh laparostomy: Here the fascia! layer is closed by using marlex mesh or prolene mesh or even a zip to protect exposed viscera. Advantages: One can minimise infection. Disadvantages: Abdominal compartment syndrome and perforation of bowel can occur. Indications of laparostomy When a second look procedure is contemplated, e.g. acute pancreatitis, mesenteric ischaemia. Nonoperative treatment 1. Too sick a patient to tolerate the surgical procedure. 2. Sealed perforation 3. Localised peritonitis-may resolve with treatment. ABDOMINAL COMPARTMENT SYNDROME Introduction The phrase "abdominal compartment syndrome" was coined in 1984 when Irving Kron, described the measurement of intra­ abdominal pressure as a means of developing criteria for abdominal decompression to improve organ function. Definition • Abdominal compartment syndrome (ACS) is defined as a sustained increase in IAP more than 20 mmHg [with or without an abdominal perfusion pressure (APP) < 60 mmHg)] that is associated with new organ dysfunction/ failure (Fig. 27.34).

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Pathophysiology

lschaemia + soft tissue injury Inflammatory response and capillary leak Oedema and increasing tissue pressure Decreased tissue perfusion Increasing cellular ischaemia and oedema with ongoing fluid resuscitation Intra-abdominal hypertension

Fig. 27.34: Pathophysiology of abdominal compartment syndrome

• Intra-abdominal pressure (IAP) is the pressure concealed within the abdominal cavity. Normal IAP is 0-5 mmHg showing phasic variation with respiration. • Intra-abdominal pressure is measured to detect abdominal compartment syndrome and to decide on the requirment of a decompression so as to improve organ function. Final effects • The adverse physiological effects of intra-abdominal hypertension (IAH) affect almost every organ system resulting in ACS. The major systems affected, in decreasing frequency of incidence and morbidity are: Pulmonary, cardiovascular, renal, splanchnic, central nervous system. Thus the end result can be: Intractable hypoxia, hypercarbia, ARDS Cardiac insufficiency and cardiac arrest Oliguria, anuria, acute renal failure Cerebral oedema and anoxia Grading of IAP Grade I : Grade II : Grade III : Grade IV :

(Burch) IAP 12-15 mmHg lAP 16-20 mmHg IAP 21-25 mmHg IAP > 25 mmHg

Risk factors A. Diminished abdominal wall compliance I. Acute respiratory failure with elevated intrathoracic pressure. 2. Abdominal surgery with primary fascia! or tight closure. Example: Reduction of massive hernia (Figs 27.35 and 27.36). 3. Major trauma/bums 4. Prone positioning, head of bed >30° 5. High BMI, central obesity

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Fig. 27.35: This boy had abdominal compartment syndrome after reduction of intestinal contents from left thoracic cavity and we could not close the abdomen. It was covered with a thin plastic sheet-you can see the intestine. It took two months for granula­ tion tissue to cover the defect. Eventually, he recovered with an incisional hernia. Bogota bag or VAC are the other alternative meth­ ods of closure

Fig. 27.37: Close monitoring of patient with vital signs and blood transfusion in addition to antibiotics play a major role in the management of septic shock

Fig. 27.36: Loss of abdominal layers following reduction of a mas­ sive ventral hernia

B. Increased intraluminal contents I. Gastroparesis 2. Acute gastric dilatation 3. lieus 4. Colonic pseudo-obstruction C. Increased abdominal contents I. Haemoperitoneum/pneumoperitoneum 2. Ascites/liver dysfunction 3. Laparoscopy IAP measurement-principles • Expressed in mm Hg ( I mm = 1.36 cm of water). • Measured at end expiration • Performed in supine position • Zeroed at mid-axillary line at the level of iliac crest. • Performed with an instillation volume of no greater than 25 ml of saline (for bladder technique). • Measured 30-60 secs after to allow bladder detrusor muscle relaxation (for bladder technique).

Fig. 27.38: Blood transfusion

TREATMENT • Close monitoring of patient's vitals (Fig. 27.37) • Blood transfusion when required (Fig. 27.38) SURGERY I. Temporary abdominal closure • Towel clip closure, only skin closure. • Mesh-commercially available meshes with absorbable surface facing intraperitoneum and non-absorbable facing outer aspect of the wound are used. Example being polygalactin (vicryl inside) and polypropylene (prolene) outside can be used. • PTFE mesh repair: Expanded polytetrafluoroethylene. (ePTFE) is another mesh which is used. It is a surgical biomaterial with two antimicrobial preservative agents­ chlorhexidi ne diacetate and silver carbonate. It also enhances tissue ingrowth.

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PELVIC ABSCESS A young boy of 18 years, who suddenly had breathlessness was found to have diaphragmatic hernia. After reduction and closure, patient developed A CS. Reopening of abdomen was done and peritoneum was not closed but a 'cover' was given by using 'urosac' bag which was split open. Wound was allowed to heal by granulation tissue. • Bogota bag: A Bogota bag is a sterile plastic bag used for closure of abdominal wounds. 1 It is generally a sterilised, 3 litre genitourinary irrigation bag that is sutured to the skin or fascia of the anterior abdominal wall. The Bogota bag acts as a hermetic barrier that avoids evisceration and loss of fluids. Another advantage to the Bogota bag, is that the abdominal contents can be visually inspected which is particularly useful in cases of ischaemic bowel. Thus useful in resections following mesenteric ischaemia. In our country we can use urosac bag (which can be split open) or even a thin plastic sheet can be used like a Bogota bag (Fig. 27.35). • Vacuum assisted closure (VAC): It has been extensively used in the management of leg ulcers specially diabetic ulcers. It has been used in a few cases of severe pancreatitis. Here it is called open abdomen negative pressure therapy system. It removes debris, inflammatory exudates. II. Definitive abdominal closure • Primary closure. It is done layer by layer. Non-absorbable suture is usually selected • Synthetic mesh • Biologic mesh • Component separation • Plastic surgery PEARLS OF

This refers to accumulation of pus in the rectovesical pouch or pouch of Douglas (rectouterine pouch). Causes • Any peritonitis, commonly following perforation due to acute appendicitis or following salpingo-oophoritis. The rectovesical pouch is the most dependent part in the body. Hence, the septic emboli accumulated in peritoneal space give rise to pelvic abscess. • Anastomotic leakage is also an important cause. • Perforated duodenal ulcer, perforated ilea! ulcers on the other common causes. Clinical features • History of surgery/peritonitis • Postoperative high-grade fever • History of discharge of mucus per rectum for the first time in a patient who is recovering from peritonitis suggests pelvic abscess. It occurs due to irritation of the rectum. Increased frequency of micturition occurs due to irritation of bladder. • Deep tenderness in the suprapubic region. • Surgery done for peritonitis • Continuing infection even after surgery-leak from an anastomotic line. • Inadequate peritoneal toilet • Inappropriate antibiotics • Pelvic surgery • Diabetes

WISDOM

No doubt, drainage of the sep tic fo cus is the mo st important step.

COMPLICATIONS OF PERITONITIS 1. Severe hypovolaemic shock giving rise to renal failure. It can be prevented by adequate hydration of the patients and careful usage of antibiotics such as gentamicin. 2. Septic shock, multiorgan failure and death occur in late cases of peritonitis. 3. Subacute intestinal obstruction due to postoperative adhesions. 4. Pelvic abscess 5. Subphrenic abscess 1 Bogota bag's use was first described by Oswaldo Borraez in Bogota, Colombia.

A 36-year-old lady underwent vaginal hysterectomy for dysfunctional uterine bleeding. To control the bleeders, several gauze pieces were used without a proper count. After 2 weeks, purulent discharge per vagina, feve1� ill health was reported. Ultrasound done showed a pelvic abscess. CT scan done in our hospital showed foreign body with air trap suggesting gauze pieces. Her abdomen was explored, abscess drained and the gauze pieces were removed. Diagnosis • Confirmed by per-rectal examination. A tender boggy swelling is felt in the anterior wall of recturn. Ultrasound can define an abscess and can detect the size of the abscess. • CT scan is very useful in defining pelvic abscess, its extent and to detect presence of a foreign body (Fig. 27.39).

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intraperitoneal and one is extraperitoneal. The spaces, boun­ daries and the common causes of pus in these spaces are described in Table 27.1 and Figs 27.41 to 27.43.

Fig. 27.39: CT scan showing gauze pieces-she had persistent foul-smelling vaginal discharge

Treatment (Fig. 27.40) • Under general anaesthesia, a proctoscope is introduced and a nick is made in the anterior wall of the rectum to open into the abscess cavity. Pus is drained with a sinus forceps through the rectum. There is no peritoneal contamination. The cavity collapses after a few days. Postoperatively, the patient is given broad spectrum antibiotics. • In females, pus can be drained through posterior fomix.

1. 2. 3. 4.

Right subhepatic space Right subphrenic space Left subhepatic space Left subphrenic space

Fig. 27.41: Subphrenic spaces in sagittal section Falciform ligament

Upper layer of right coronary ligament

Left triangular ligament Lesser omentum

Lower layer of right coronary ligament

Inferior vena cava Gall bladder

Fig. 27.42: Subphrenic spaces (Table 27.1 for numbers)

Fig. 27.40: Drainage of pelvic abscess through the rectum

SUBPHRENIC ABSCESS

Pancreas

Introduction • As a result of peritonitis, residual abscess can collect in the intraperitoneal cavity. Pus that collects under the diaphragm is described as subphrenic abscess. Subphrenic abscess is the commonest intra-abdominal abscess. • Gastrointestinal perforations, postoperative leaks, penetrations, traumas, puerperal sepsis, are the common causes of subphrenic abscess. • Blood clots, bacteria laden fibrin, neutrophil contribute to an abscess. Surgical anatomy There are 5 subphrenic spaces between the diaphragm and the liver bounded by various peritoneal folds. Four are

Right posterior Fig. 27.43: Cross-section showing subphrenic spaces: (1) Right anterior intraperitoneal space, (2) right posterior intraperitoneal space, (3) left anterior intraperitoneal space, (4) left posterior in­ traperitoneal space (lesser sac)

Aetiopathogenesis (Fig. 27.44) • The causative organisms of peritonitis are Escherichia coli, Enterococci, Klebsiella, Enterobacter, Proteus, Bacteroides, etc.

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Subphrenic space boundaries and common causes of involvement Spaces

Boundaries

Common causes

1. Right anterior intra­ peritoneal space

It lies between right lobe of the liver and diaphragm Posteriorly-anterior layer of the coronary ligament and right triangular ligament. On the left side is the falciform ligament

Perforated duodenal ulcer, gastric ulcer, cholecystitis

2. Right posterior intra­ peritoneal space (Rutherford Morrison's hepatorenal pouch)

It lies below the right lobe of the liver Inferiorly-hepatic flexure and transverse colon Medially-second part of the duodenum Laterally-abdominal wall. This is the biggest intraperitoneal space

Appendicitis, cholecystitis Perforated duodenal ulcer Upper abdominal surgery

3. Left anterior intra­ peritoneal space

Above-diaphragm. Posteriorly-left triangular ligament, left lobe of the liver, lesser omentum, anterior surface of the stomach. Right side-falciform ligament Left side-spleen

Surgery on stomach­ gastrectomy. Distal pancreatectomy. Left hemicolectomy

4. Left posterior intra­ peritoneal space (lesser sac)

In front-by lesser omentum and posterior surface of the stomach. Behind-pancreas, suprarenal, left kidney On the right side-foramen of Winslow through which it communicates with the greater sac

Pseudopancreatic cyst Perforated gastric ulcer

5. Midline extraperitoneal space (bare area of the liver)

Above-upper layer of coronary ligament Below-lower layer of coronary ligament Left-inferior vena cava

Amoebic hepatitis Pyogenic liver abscess

Liver abscess Perforated --t-----.d!!!m:"""'1-� duodenal ulcer Acute---+----pancreatitis

Appendicitis --1---+-+ � (retrocaecal)

2. Left paracolic gutter is narrow and colophrenic ligament is present on the left side. 3. Majority of diseases affect right side (perforation, liver abscess, appendicitis, gall bladder disorders, etc.) (Fig. 27.44). Clinical features • A patient who is recovering from peritonitis complains of fever with sweating. Initially, fever is low grade, continuous. Later, there is high-grade fever with chills and rigors. • Deterioration of health occurs very fast with wasting and anorexia. • Shoulder pain is due to irritation of undersurface of the diaphragm by the pus (sensory fibres of the phrenic nerve are irritated-CJ, 4). • Postoperative patient is not doing well-prolonged ileus. • Anorexia, wasting, hiccup, dry cough. PEARLS OF

Fig. 27.44: Common causes of subphrenic abscess

• The high incidence of subdiaphragmatic abscess is due to constant circulation of fluid from below upwards because of following reasons: 1. Upward movement of diaphragm during expiration. 2. Decreased intra-abdominal pressure 3. Capillary action Subphrenic abscess is common on the right side because of the following reasons: 1. Right paracolic gutter is wide and deep and colophrenic ligament is absent.

WISDOM

A postoperative patient who has pyrexia, prolonged ileus, poor appetite and progressive deterioration of health has subphrenic abscess.

• Tenderness is present in the epigastrium on deep palpa­ tion. • Common causes of postoperative fever are absent, e.g. thrombophlebitis, urinary tract infection. PEARLS OF

WISDOM

Pus nowhere, pus somewhere, pus under the diaphragm­ Harold Barnard.

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Investigations 1. Total count with neutrophil count 2. Plain X-ray abdomen (erect)-may show gas and fluid level under the diaphragm (Figs 27.45 and 27.46) 3. Fluorescent radiography may reveal absence of movement of right side of diaphragm on inspiration. 4. Ultrasonography confirms the site of abscess, number of abscesses, loculations, etc. • Abscess is characterised by hypoechogenic cavity surrounded by sharp distinct echogenic wall. It can be therapeutic to insert catheter for drainage.

Fig. 27.47: CT scan showing left subphrenic collection followin� acute pancreatitis-a pigtail catheter is inserted (also see Fig. 27.48)

5. CT scan demonstrates well defined, low density mass, the rim of which is enhanced after intravenous injection o1 contrast medium. The mass tends to be round because of centripetal expansion-highly sensitivity > 95% (Fig. 27.47). Fig. 27.45: Plain X-ray-gas and fluid level under diaphragm: PA view and lateral view

6. Isotope imaging using Gallium 67 citrate or Iridium 111. Gallium binds to proteins-lactoferrin and transferrin which are present in high concentration in an abscess. Goals of the treatment (Flowchart 27.2) Treatment Today with the availability of sophisticated imaging facilities, percutaneous drainage has become the choice of therapy rather than surgery. Both have been described in next column. I. Percutaneous drainage can be done with the help of ultrasound or CT scan, provided the abscess cavity is unilocular, and the track is safe.

f

Types 1. Pigtail catheter (using Seldinger's technique): It is a small

Fig. 27.46: Plain X-ray-gas and fluid level under diaphragm: PA view and lateral view

tube used to drain bile, urine, pancreatic fluid or abscess. 2. Trocar catheter: 12-16 F trocar is used.

Flowchart 27.2

I

I

Goals of the treatment

I

I

I

Drainage abscess

Control of primary cause

Effective antibiotics/nutrition

I. Percutaneous drainage II. Open drainage

I. Postoperative leaks-treat II. Good drainage

I. Broad spectrum antibiotics II. Nutritional support

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SPECIAL TYPES OF PERITONITIS POSTOPERATIVE PERITONITIS Introduction: This is not an uncommon problem encountered in the surgical wards. Very often patient who undergoes an intestinal or a biliary surgery and a few days later vague symptoms and signs develop. Hence it is difficult to diagnose if one takes a casual approach. It has high mortality. Hence requires early detection and demands early effective solution. It should be suspected following surgery on intestines or biliary tract, when a patient who is recovering from paralytic ileus starts deteriorating or when paralytic ileus does not return back to normal. Fig. 27.48: Pigtail catheter drainage of left subphrenic abscess

following gastric ulcer perforation

3. Sump catheter: It has a double lumen which permits irrigation as well as drainage and allows a good suction (Key Box 27.11). PEARLS OF

WISDOM

More than 90% of subphrenic abscesses are managed by percutaneous drainage successfully.

.......

IMfflW�� INDICATIONS FOR REMOVAL OF CATHETER

• Drainage less than 10 ml/day • No fever, no pain • WBC counts return to normal

II. Open drainage (Key Box 27.12) • The anterior subcostal or posterior (bed of 12th rib) approach is used. Both are extraserous approaches. • However, lesser sac abscess and abscesses connected with the bowel, discharging pus or bile, are drained by intra­ peritoneal route. • Open drainage is ideal in cases of multiloculated abscesses. • Surgery is always done under the cover of broad-spectrum antibiotics.

........

IMfflM,,_.,� INDICATIONS FOR OPEN DRAINAGE 1. Multiloculated abscess 2. Persistent fistula discharging pus-communication with bowel. 3. T hick viscid content 4. Failure of percutaneous aspirations 5. Abscess very close to IVG/diaphragm

Etiology (Figs 27.49 to 27.52) • Leakage from anastomotic line • Iatrogenic visceral trauma • Foreign bodies • Others Causes of delay in the diagnosis • Presence of fever is attributed to other sources of infection such as urinary tract infection, thrombophlebitis, etc. • Presence of pain and tenderness is attributed to recent laparotomy scar. • Tachypnoea, hypotension are attributed to pre-existing medical conditions such as COPD, cardiac failure. • Steroid therapy masks the local signs and symptoms. • Administration of antibiotics would have reduced the severity of peritonitis (masking effect) only to manifest as septicaemia some time later. Bacteriology 1. Common org anisms in po stoperative peritonitis

(Figs 27.49 to 27.52) A. Gram -ve Bacilli Klebsiella Pseudomonas B. Anaerobes Clostridium Fusobacteria C. Gram +ve cocci Staphylococci

E.coli

Proteus Bacteroides Peptostreptococci Enterococci Streptococci

2. Foreign bodies responsible for postoperative peritonitis Macroscopic Microscopic

• • • • • • •

Gossypiboma Textiloma Surgical drains Suture materials Surgical clips Implants Instruments

3. Presentation of foreign body

• Abdominal pain • Mass abdomen • Granuloma, fever

• • • • •

Barium Cloth piece Faecal matter Necrotic tissue Talcum powder

• Intestinal obstruction • Fistula and sinus • Extrusion-sometimes the foreign body may be visible to the exterior

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Fig. 27.49: Postoperative peritonitis due to post-cholecystectomy

Fig. 27.50: Postoperative peritonitis due to anastomotic leak with faecal peritonitis

Fig. 27.51: Postoperative peritonitis due to enteric ulcer perforation

Fig. 27.52: Postoperative peritonitis due to anastomotic leak with faecal peritonitis-look at the temperature chart

How to suspect postoperative peritonitis • Deterioration after 3-5 days of operation (the time when anastomotic dehiscence takes place). • Delay in recovery from paralytic ileus-abdominal distension. • Evidence of toxaemia-tachycardia, tachypnoea. • Free drainage of bile and faecal matter or pus from the drain site or the main wound. • Oliguria may be an early indicator of postoperative sepsis. • Tenderness, guarding, rigidity.

• Avoid using packs-fascia! closure • Intraoperative radiology • High degree of suspicion (see clinical notes) 2. Specific treatment (Key Box 27.13)

leak

Treatment (Figs 27.53 and 27.54) 1. Prevention of gossypiboma (MOP) • Double counting • Sponges with radio-opaque markers • No hurried counting • A dditional counting-when change of OT personnel

In 1973, when ultrasound facilities were not available, a 35-year-old male who had undergone appendicectomy for a gangrenous appendix, was found to have high spiking fever on the 3rd postoperative day. All possible causes of postoperative fever including malaria were ruled out. On the I 0th postoperative day, the patient developed a purulent discharge of I 00 ml through the lower part of the main wound following which he had a spontaneous and dramatic recovery.

Peritoneum, Peritoneal Cavity, Mesentery and Retroperitoneum

,.,,.,_,� .......

TREATMENT • Danger lies in delay, not in reoperation • Leak or abscess cavity is confirmed by abdominal ultrasound. • Exploration (preparation similar to a routine laparotomy) • Resection or resuturing, ileostomy, colostomy

• Drainage of abscess cavity • Refashioning of colostomy, if it is retracted • Once abscess is drained or leakage is prevented, recovery is wonderful. • Appropriate antibiotics • Delayed closure of skin • Peritoneal lavage

653

Poor prognostic factors in postoperative peritonitis • Increasing age • Organ(s) failure • Colonic perforation • Multiple abscess • Lesser sac abscess • Malnutrition • Postoperative pneumonia • Anergy: Anergy (immunologic tolerance) refers to the failure to mount a full immune response against a target. BILIARY PERITONITIS • Leakage of bile into the peritoneal cavity results in biliary peritonitis. • It will be more obvious and can be detected early if a drainage tube has been kept. Causes 1. Surgery on the gall bladder • Leakage from the cystic duct • Injury to the right hepatic duct • Leak from accessory cholecystohepatic duct 2. Surgery on the CBD • Retained stones in the lower CBD • Loose sutures over CBD • T-tube not anchored properly

Fig. 27.53: Pus pouring out as soon as peritoneum is opened. First step is to send a pus for culture sensitivity

3. Surgery on the duodenum • Sphincteroplasty • Partial gastrectomy • Reperforation of sutured duodenal ulcer 4. Injury to the duodenum • During nephrectomy, hemicolectomy • Blunt injury 5. Instrumentation ERCP, stenting or following duodenal polypectomy 6. Diseases of the gall bladder Perforation or gangrene of the gal I bladder

Fig. 27.54: Postoperative peritonitis following due to leak following right hemicolectomy for carcinoma caecum. There were 2 sites of leak: 1. lleocolic anastomotic site, 2. proximal jejunum. Both were exteriorized. You can see the wound left open with loose skin sutures-they will be tied after 3-4 days. This is to prevent wound infection (almost inevitable)

Clinical features (Figs 27.55 and 27.56) • In majority of the cases, the local signs are confined to one quadrant of the abdomen in the form of guarding and rigidity. • There may be excoriation of the skin due to drainage of bile to the exterior (Fig. 27.57) • However, when the anastomosis gives way, generalised peritonitis can occur. • In untreated cases, septicaemic shock can develop. • Final stage will be multiorgan failure and death. • It has a very high 11101iality rate.

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Fig. 27.55: Post-cholecystectomy bile leak due to injury to the common bile duct. Majority of such leaks can be managed conservatively. If the leak continues, ERCP and treat the cause accordingly

Fig. 27.57: Injury to the terminal ileum during tubectomy resulted in faecal fistula. To prevent the excoriation of the skin, zinc oxide cream has been applied

Fig. 27.56: Biliary peritonitis following duodenal ulcer perforation.

It requires urgent laparotomy. Closure of the ulcer perforation, peritoneal lavage followed by drainage tube and closure of the incision

Fig. 27.58A: Feeding jejunostomy-a small incision is made in the proximal jejunum and a plastic tube is introduced into the lumen and advanced to about 60cm

Treatment

I. Most of the biliary fistulae heal within 2-3 weeks with conservative line of treatment. 2. If it does not heal, re-exploration and resuturing or resection has to be done. 3. Feeding jejunostomy is a very useful procedure in all cases of reperforation of sutured duodenal ulcers or difficult duodenal ulcer closures (Fig. 27.58). It should be remembered that feeding jejunostomy is temporary and is get till it is assured that there is no leak from

Fig. 27.580: Feeding jejunostomy intussusception

the operated site. If there is no peritonitis, patient has passed flatus and stools, jejunostomy tube is removed. Feeding jejunostomy should be done carefully with proper placement of catheter within the small bowel, fixing it firmly both inside and outside the abdominal wall and fixing to the bowel wall. Complications of feeding jejunostomy • Bile leak from the entry point • Tube blockage if it is not flushed properly after feeding. • Bleeding, displacement and intussusception.

.,,.,,_,�

Peritoneum, Peritoneal Cavity, Mesentery and Retroperitoneum

PNEUMOCOCCAL PERITONITIS • Primary variety is more common. Girls of3-6 years of age are usually affected. Infection spreads from female genital tract through vagina. • Malnourishment precipitates pneumococcal peritonitis. • In boys, blood spread can occur following upper respiratory tract infection. Clinical features • High grade fever with features of toxaemia. • Bloody diarrhoea and frequency of micturition are indicative of pelvic peritoneal inflammation. • Other features of peritonitis are present. Diagnosis Aspiration of peritoneal fluid demonstrates high WBC count30,000/mrn3 . More than 90% are polymorphs. Treatment Laparotomy and drainage of pus (odourless and sticky initially and creamy or purulent in the later stages), to be followed by appropriate antibiotics. PRIMARY STREPTOCOCCAL PERITONITIS • Infants and children less than 4 years are commonly affected. • Peritoneal exudate is cloudy and contains flakes of fibrin. • Symptoms of gastroenteritis-greenish watery stools are present. • Source of infection is tonsillitis, pharyngitis, etc. • Treated with injection crystalline penicillin. PARTURITION PERITONITIS • This occurs after delivery if proper aseptic precautions are not taken. The incidence has come down in the recent years. Attempted abortions by using instruments which are not sterile results in peritonitis. Most of the time, peritonitis is confined to pelvis with paralytic ileus, mucous diarrhoea and offensive lochia (Key Box 27.14). Late cases develop generalised peritonitis, intra-abdominal abscess, intestinal obstruction and infertility. Table 27.2 gives summary of various types of peritonitis. • Other name for this type of peritonitis is abortion peritonitis.

• • • • •

655

ABORTION PERITONITIS Instrumentation Puerperal sepsis Offensive lochia Pelvic peritonitis Infertility

SPONTANEOUS (PRIMARY) BACTERIAL PERITONITIS (SBP) As the name suggests, in this condition, there is no demon­ strable intra-abdominal disease responsible for peritonitis. Types 1. In infants: It is more common in female children. Spread is by haematogenous route. The causative organisms are Streptococcus pneumoniae. It may follow respiratory tract or urinary tract infection (Key Box 27.15). 2. In adults: Male alcoholic patients are commonly affected followed by patients with chronic liver disease. Causative organisms are E. coli, S. faecalis, etc. • Portal hypertension increases permeability of gut wall, thus increasing bacterial migration. These bacteria which colonise in the small bowel reach systemic circulation because of shunting of blood around liver sinusoids. Portal lymph also gets contaminated giving rise to increased ascitic fluid.

lltMIWW',1 RISK FACTORS

�

CHILDREN • • • •

Malnutrition Malignancy Chemotherapy Splenectomy

ADULTS • Cirrhosis • Nephrotic syndrome • Chronic renal failure

Summary of various types of peritonitis Type

Age

Route

Exudate

Pneumococcal �-haemolytic streptococci Parturition Tuberculous Perforation peritonitis

3-6 years Children 20-40 years 20-40 years 20-40 years

Vagina Vagina Vagina Pulmonary tuberculosis Hollow viscus

Odourless, sticky, turbid pus with high-grade fever Cloudy and fibrin flakes+ Thick pus+ Straw-coloured fluid+ tubercles + Foul-smelling exudate +

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Clinical features • Dull aching pain in the abdomen with low-grade fever. • Rebound tenderness is present, bowel sounds are absent or sluggish-abdominal distension • Cirrhotic patients may develop coma with onset of primary bacterial peritonitis. • Septic shock is a late feature with a high mortality rate.

Tumours of the peritoneum • Primary: Mesothelioma is the tumour to be remembered It is more common from the pleura. In the abdomen, pelvi, peritoneum is the common site. • Secondary tumours are-pseudomyxoma peritonei an< carcinoma peritonei.

Investigations • Leukocytosis, !albumin, lprothrombin time suggests sepsis. • Peritoneal tap and Gram staining of the fluid. • Laparoscopy may help to rule out intra-abdominal emergencies such as perforations, etc. • Ultrasound can detect nature of the liver and amount of fluid in the abdomen. • CT scan when in doubt about the diagnosis.

• In this condition, peritoneal cavity is filled with mucoic substance (jelly-like) brownish or yellowish. • Mucinous cystadenoma appendix is the cause and late1 spreads to ovaries (Fig. 27.59). Some also believe diseas� starts in ovary and later spreads to other organs. • Primary tumour is very slow growing and metastasis ii exceptional.

PSEUDOMYXOMA PERITONEI

Treatment • Conservative treatment is followed, provided secondary bacterial peritonitis is ruled out. • Broad-spectrum antibiotics such as aminoglycosides with 3rd generation cephalosporins is the ideal choice. Metronidazole is always added. • Instillation of antibiotic solution into ascitic fluid to achieve a quick and high concentration. • If laparotomy is done, peritoneal wash or toilet is given. PERIODIC PERITONITIS • • • •

Also called familial Mediterranean fever. It is of unknown aetiology It affects children, young adults and females. Presents as abdominal pain, tenderness, pyrexia and increased total WBC count. • When in doubt, laparotomy should be done. Some salient features (Observe 11 Ps) • Pyrin-a protein product from the gene-MEFV gene mutation causes this. • Pain abdomen and tender abdomen • Pyrexia • Pain in the thorax • Period of 2-3 days, it occurs and slowly remission occur. • Patients are usually children • Principally occurs in Arabs, Armenians and Jews. • Primarily runs in families • Peritoneal inflammation is present • Polymorph leukocyte count is increased • Prevent recurrence-colchicine therapy

Fig. 27.59: Rupture of mucocoele of the appendix (Courtesy: Dr Stanley Mathew, FRCS, Professor, Department of Surgery, KMC, Manipal}

Clinical features (Key Boxes 27.16 and 27.17) • The patients can present with slow, painless and progressive abdominal distension. No shifting dullness. • It is more common in females. • It can also present with features of intestinal obstruction. Treatment • Aggressive surgery is the main mode of treatment. Masses of tumour tissue (jelly) should be resected or scooped out. Thus debulking, bilateral oophorectomy, appendicectomy, omentectomy are done. • Combination chemotherapy by using cisplatin and/or intraperitoneal alkylating agents have been used.

....

...

IMIW�� SITES

• • • •

Ovary-common site Appendix-common site Intestines-not uncommon sites Uterus, urachus-rare sites

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657

PSEUDOMYXOMA PERITONEI Women Operation: Surgery is the main treatment Mucinous cyst of appendix/ovary is the cause Extraperitoneal spread never happens No shifting dullness-material is jelly-like At surgery Masses of jelly scooped out Appendicectomy should be done Debulking of mass to be done OVAriectomy, omentectomy is done Radioactive isotopes can be instilled into peritoneal cavity Yellow apron-omentum should be removed Remember as WOMEN have MAD OVARY

• Recurrence can occur because the tumour is locally malignant. CARCINOMA PERITONEI • This name is applied to an advanced stage of intra­ abdominal malignancy involving the entire peritoneal cavity. • The causes are shown in Key Box 27.18.

COMMON CAUSES Carcinoma of the: • Stomach, colon, pancreas • Breast, ovary

Features at laparotomy • Multiple firm to hard nodules on the visceral and parietal peritoneum. • Dense adhesions between the intestinal loops and other viscera. • Plaques on the intestinal surface • Widespread secondaries in the liver • Entire omentum will be studded with hard nodules. It is called omentum cake (Fig. 27.60). • Ascites: Straw-coloured or haemorrhagic • Greater omentum being the policeman and a great drain pipe of the abdomen with rich lymphatics, is studded with nodules. • Low protein ascites is more vulnerable for risk of developing peritonitis. Incidence of p eritonitis in malignant ascites cases is low because of increased immunoglobulin levels in malignant ascites and increased opsonic activity.

Fig. 27.60: Omental cake in colorectal cancer

Differential diagnosis • The most common differential diagnosis is tuberculosis (peritoneal). These nodules are firm and greyish. • Acute pancreatitis with fat necrosis: This is due to calcium soap. They are yellow and soft. • Ruptured hydatid cyst • Lymphomatous nodules Treatment I. Radioactive gold (Au 198) instillation into peritoneal cavity. 2. Tamoxifen is useful for ascites due to carcinoma of the breast. GRANULOMATOUS PERITONITIS • Talc, gauze, starch, etc. are causative factors. • It occurs many weeks after surgery. • Low-grade fever, weight loss, distension, crampy abdominal pain are the features. • Laparoscopy is the key investigation, can visualise granuloma and biopsy can be taken. Also, fluid can be aspirated and sent for histopathology. High concentrations of lymphocytes are present in both. • Symptomatic treatment-sinus, fistula needs to be treated. • Intravenous prednisolone followed by oral prednisolone for 2-3 weeks is given. Prevention • Cleaning the gloves by wiping before handling bowel, prevents many cases of granulomatous peritonitis. • In the initial surgery all the foreign bodies including ova, cysts of parasites, ascariasis, ingested food particles have to be removed.

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THE OMENTUM Surgically important diseases in the omentum are: I. Tuberculous peritonitis: Here omentum is involved. It becomes nodular because it is studded with tubercles. Classically seen in children who are brought with abdominal distension. On palpation, omentum is felt as granular mass in the upper abdomen which moves with respiration. Laparoscopy, biopsy and antitubercular treatment is given in Fig. 27.61. 2. Metastasis: Carcinoma stomach, colon, pancreas commonly result in metastasis and they give rise to omental cake (Fig. 27.62). 3. Tumour (cyst): Omentum is the site of omental cyst which is a lymphatic cyst (Fig. 27.63). It is a slow-growing, painless swelling in the upper abdomen. On examination, the patient is a child or an adult, with a smooth, firm mass in epigastrium which moves with respiration. Excision of the lymphatic cyst is the treatment. Massive cyst can be confused for ascites.

•

•

•

Fig. 27.65: Pus-like milky fluid once abdomen is opened (NHL)

4. Torsion: It is a rare surgical emergency wherein torsion of omentum occurs due to old adhesions or it is primary due to lengthy mobile omentum. It produces symptoms/signs similar to that of appendicitis. Laparotomy and excision of gangrenous omentum have to be done. 5. Non-Hodgkin's lymphoma can affect ometum and can give rise to granularity (Figs 27.64 and 27.65). Complications: Haemorrhage into the cyst. MESENTERY • Anatomy: Given in page 666 • Mesenteric tear has been discussed in page 667 • Mesenteric lymphadenitis has been discussed in page 672

Fig. 27.61: Rolled up omentum in tuberculosis

Fig. 27.62: Omental cake in metastasis

Fig. 27.63: Omental cyst (lymphatic)

Fig. 27.64: Omentum in non-Hodgkin's lymphoma (NHL)

MISTY MESENTERY • It means increase in the mesenteric fat and it is a finding in the multi-detector CT scan. • A few pathological conditions which may give rise to this entity are: Acute pancreatitis, retroperitoneal haemorrhage, malignancies. • Once the source is treated, the findings may reduce or may disappear. • Hence, follow-up CT scans are required. • Weber-Christian disease: Lipodystrophy and mesenteric lipogranuloma are the features. In this condition, it should be called mesenteric panniculitis. Findings include inflammation of the mesentery, fibrosis, shortening and ischaemia.It is difficult to treat. MESENTERIC CYST • These are congenital cysts, enterogenous or chylolymphatic. It manifests in young children or during adolescence. Typically, the cyst is located in the umbilical region which moves at right angles to the direction of mesentery (Fig. 27.66).

Peritoneum, Peritoneal Cavity, Mesentery and Retroperitoneum

•

( \

/

/

.

/

659

•

--+-+---+-- Line of attachment of mesentery

Fig. 27.69: Chylolymphatic cyst-it can be excised with­ out resection of the bowel Fig. 27.66: Diagrammatic representation of mesenteric cyst and its movements at right angles to the direction of mesentery

Types of mesenteric cysts (Figs 27.67 to 27.70, Key Box 27.19) A. Chylolymphatic cyst is a lymphatic cyst arising from mesentery of ileum. It is a thin-walled cyst with clear fluid or chyle. It has a separate blood supply. Hence, enucleation is the treatment without sacrificing the bowel. B. Enterogenous cyst is a duplication cyst from the intestine or due to diverticulum of the mesenteric border of the intestine. It is thick walled and contains mucus. This cyst is treated by excision of cyst with bowel segment because both share the same blood supply.

Fig. 27.70: Enterogenous cyst-it requires excision of intestine along with the cyst

Complications • Torsion of the cyst resulting in acute abdominal pain. • Rupture of the cyst due to trauma • Haemorrhage into the cyst

.,.,_,,, • • • •

MESENTERIC CYST-TYPES Chylolymphatic cyst Enterogenous cyst Urogenital remnant Teratomatous dermoid cyst

"'81

RETROPERITONEUM ANATOMY

Fig. 27.67: Enterogenous cyst at surgery Tillaux's Triad 1. Fluctuant swelling near the umbilicus. 2. Movement perpen­ dicular to the line of mesentery. 3. It is dull surrounded by a zone of resonance and traversed by band of resonance. Fig. 27.68: Mesenteric cyst excised in toto

Retroperitoneal space: The retroperitoneal space lies between the peritoneum and the posterior parietal wall of the abdominal cavity and extends from the diaphragm to the pelvic floor. Superiorly-12th thoracic vertebra and lateral lumbocostal arch. Inferiorly-base of the sacrum, iliac crest, and iliolumbar ligament. Anteriorly-posterior parietal peritoneum Posteriorly-fascia overlying the quadratus lumborum and psoas major muscles. Important anatomical organs (Fig. 27.71) 1. Urinary: Adrenal glands, kidneys, ureter, bladder 2. Circulatory: Aorta, inferior vena cava 3. Digestive: Oesophagus (thoracic part), rectum (part of middle third and lower third is extraperitoneal). 4. The head, neck, and body of the pancreas, the duodenum, except for the proximal first segment, ascending and descending portions of the colon.

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Posterior abdominal wall includes the study of the following structures Abdominal aorta 2

Inferior vena cava

3

Right and left kidneys with ureters

4

Duodenum on the right side

5

Head of pancreas in the concavity of duodenum

6 Body and tail of pancreas across the posterior abdominal wall towards the left kidney

Fig. 27.71: Anatomical organs in the retroperitoneal space

J

IDIOPATHIC RETROPERITONEAL FIBROSIS-ORMOND'S DISEASE • This is one of a group of fibromatosis ( other being Dupuytren 's contracture and Peyronie 's disease). • Other types of fibrosis such as mediastinal fibrosis, sclerosing cholangitis may also be associated features. • Riedel's thyroiditis may be associated with this condition • As collagen encases ureters, they present with ureteric obstructions, requiring ureteric stenting. • Steroid therapy is the treatment of choice • Tamoxifen may help • It can present with lower back pain, renal failure, hypertension, deep vein thrombosis and other obstructive features. See Key Box 27.20 and clinical notes.

.,.,_,� IDIOPATHIC RETROPERITONEAL FIBROSIS-CAUSES

ldiopathic-Ormond's disease Drugs-chemotherapy, methysergide, �-adrenoreceptor antagonists Irritation-blood, urine, bowel contents Others-autoimmune Peritoneal disease-secondaries Aortic aneurysm-inflammatory type Trauma Hereditary/familial Inflammation-chronic Carcinoid Remember as IDIOPATHIC

• One day I was urgently called to assist the urosurgeon who had opened the abdomen for ureterolysis. Following was the clinical picture. • A 76-year-old lady was admitted under urology for the diagnosis of renal failure. An ultrasound abdomen showed bilateral hydronephrosis. She was diagnosed to have idiopathic retroperitoneal fibrosis and posted for ureterolysis. To my surprise, when I explored, the patient had carcinoma colon in three places (synchronous), carcinoma caecum infiltrating right ureter, carcinoma sigmoid colon infiltrating left ureter and carcinoma transverse colon. She underwent total colectomy with ileorectal anastomosis. Ureteric reimplantations were done into bladder. She lived for 3 years and then succumbed due to liver metastasis.

RETROPERITONEAL CYST • Very often, it is a painless, smooth, firm enlargement. • May have minor degree of mobility. • These cysts are either lymphatic cysts or derived from the remnant of Wolffian duct. A few of them are teratomatous dermoids. • Benign retroperitoneal cysts are usually mesothelial or mesonephric in origin; rarely, rupture of the biliary tree can result in bile-filled cysts. • Gross: These cysts are not connected to the kidney or adrenal; usually filled with clear or straw-coloured fluid. • Histology: These cystic structures may be lined by mesothelial, enteric (glandular), or columnar epithelium.

Peritoneum, Peritoneal Cavity, Mesentery and Retroperitoneum

• They can be unilocular or multilocular. • CT scan is required to differentiate it from hydronephrosis. • Excision is the treatment.

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Paravertebral abscess

RETROPERITONEAL ABSCESS Causes 1. Renal source: Pyonephric abscess 2. Spine: Tuberculosis (details below) 3. Haematoma: Fracture spine/pelvis 4. Acute pancreatitis (on right side) 5. Retrocaecal appendicitis (on right side) 6. Sigmoid diverticulitis Diagnosis • Ultrasound or CT scan-guided aspiration • Culture of the pus and antibiotic sensitivity. Treatment Aspiration, appropriate antibiotics and open drainage. PSOAS ABSCESS Three types have been recognised 1. Primary psoas abscess: It is caused by haematogenous spread of Staphylococcus aureus. Source may be occult­ tonsils, middle ear, etc. More common in children and young adults. Poor nutrition, may be a contributing factor­ It is monomicrobial. 2. Secondar y psoas abscess: Secondary to intestinal perforation, e.g. Crohn's disease. It is polymicrobial. Other causes are given below. Clinical features Fever, flank pain, flexion of the hip joint are triad of psoas abscess. Pain on extension confirms the diagnosis. Management • CT scan is the diagnostic test. Gas bubbles are diagnostic of an abscess. • Treatment include percutaneous catheter drainage, treatment of the source of infection with antibiotics. • If necrotic tissue does not drain well or if patient is not improving, open drainage should be done. 3. Tuberculous spine: Lower thoracic (Tl 0) and upper lumbar spine are commonly affected (Fig. 27.72). Clinical features • Pain in the back (localised to lesion) or referred pain if there is a collapse. • Evening pyrexia • Protective muscular spasm, especially of sacrospinalis. • Collapse of the anterior portion of vertebral body results in angular defom1ity-gibbus.

Fig. 27.72: Various sites of tubercular cold abscess arising from tuberculosis of the spine

Route of psoas abscess • Pus enters psoas sheath and tracks downwards and causes mass in the iliac fossa. • From here, it traverses beneath inguinal ligament. • If untreated, it collects in the subcutaneous plane. Investigations • Chest X-ray, ESR, sputum AFB • Spine X-ray-AP and lateral view. The earliest sign is a decrease in the intervertebral space. • MRJ can detect spine lesion, cold abscess • CT/MR-guided aspiration of pus/biopsy to prove histological diagnosis. Treatment • Cold abscess-aspiration followed by antituberculous treatment. • Unstable/collapse spine-costotransversectomy-lateral thoracotomy. RETROPERITONEAL TUMOUR Definition • The term retroperitoneal tumour (RPT) is usually confined to primary tumours arising in other tissues in this region, e.g. muscle, fat, lymph nodes, nerves (Tables 27.3 to 27.5). (Jean Lobstein, French pathologist and surgeon in 1829coined the term retroperitoneal tumour.) • Other tissues refer to-tumours of retroperitoneal organs such as kidneys, ureters, pancreas and adrenals are conventionally not included in retroperitoneal tumours.

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Tumours of mesodermal origin (75%) Fatty tissue origin Smooth muscle origin Connective tissue origin Lymphatic origin Mesenchymal origin Vascular origin Unknown origin

..

.

Lipoma/liposarcoma Leiomyoma/leiomyosarcoma Fibroma/fibrosarcoma Lymphangioma/lymphangiosarcoma Myxoma/myxosarcoma Haemangioma/haemangiosarcomal haemangiopericytoma Xanthogranuloma

Tumours of notochordal or embryonic rests origin

Embryonic origin Notochordal origin

Benign or malignant teratomas chordomas

Tumours of neurogenic origin (25%) Germ cell origin Sympathetic origin Adrenochromaffin origin

Benign or malignant teratomas Chordomas Cortical carcinoma/ paraganglioma/ pheocromocytoma

Introduction • Uncommon (0.2-0.6% of all tumours) • Malignant in 80-85% of cases (of these, 35% are sarcomas). • Sex: No differences • Age: Most occur between the sixth and seventh decade of life. • Because of their location, these lesions usually demonstrate indolent growth and present as relatively large lesions. • Their proximity to vital structures (especially vascular) makes resection difficult. Aetiology 1. Idiopathic: The actual cause is not known 2. History of radiation: Accidental or given for lymphoma. 3. Exposure to vinyl chloride/thorium dioxide 4. Familial disorders: Gardner's syndrome, familial neuroblastoma, neurofibromatosis, Li-Fraumeni syndrome. Symptoms of RPT • Abdominal mass (80%): Slow growing painless mass is the most common presentation. Typically confined to one side rather than centre. • Nausea, vomiting and weight loss in about 20 to 30% of patients. • Compressive symptoms: Abdominal pain, constipation, recent haemorrhoids, haematochezia can occur. Back pain and sciatica (30%) are common and are confused for spine pathology.

Unilateral lower extremity oedema and pressure symptom including secondary varicosities are common. Acute urine retention, dysuria and increased frequency cai occur due to compression on urinary bladder • Paraneoplastic syndrome such as intermittent hypo glycaemia can occur in liposarcoma/fibrosarcoma an< catecholamine excess in paragangliomas (Key Box 27.21)

....... 1m1w�,� • • • • • •

WHEN TO SUSPECT RETROPERITONEAL SARCOMA�

Recent finding of large abdominal mass Recent lower limb swelling Recent varicosities Recent varicocele Recent haemorrhoids Recent loss of weight

Signs of RPT • Usually large abdominal mass, finn to hard, irregular. • Nonmobile, restricted mobility • Not moving with respiration • Does not fall forward (knee elbow position) • Resonant tone on percussion-due to bowel anteriorly. • Transmitted pulsations may be felt. Differential diagnosis • Lymphoma • Germ cell tumours • GIST • Metastatic testicular cancers Please note: When you suspect anyone of these as differential

diagnosis a CT-guided biopsy may be required which alters the management.

Investigations P reoperative planning to assess the extent of the disease is essential because successful surgery is defined by complete excision of the mass with adequate margins of nom1al tissue. 1. CBP 2. LFT: Increase alkaline phosphatase may indicate secondaries. 3. RFT: Compressive uropathy with high urea and creatinine. 4. Tumour markers: AFP, beta-HCG-germ cell tumour. 5. LDH in lymphoma/GCT: LDH levels indicate tumour burden and growth rate. 6. CT Scan (Key Box 27.22) • Delineate the anatomic limits of the lesion. • Vascular involvement-vena cava, aorta, renal vessels. • Assess the integrity and function of adjacent organs­ renal function is one of the important advantage of CT scan. • Visceral metastases+/-. If present, it is inoperable but still worth trying a resection after giving neoadjuvant therapy. • Para-aortic, iliac, mesenteric lymphadenopathy. • Axial skeleton and renal involvement (Key Box 27.22).

,-,-�,, ....... • • • • • • • • •

Peritoneum, Peritoneal Cavity, Mesentery and Retroperitoneum

� CT SCAN: TYPICAL FINDINGS Lipoma: Homogenous fatty density Malignant fibrous histiocytoma: Calcifications Neurofibroma: Homogenous low density Teratoma: Mixed components Paraganglioma: Para-aortic location Neuroblastoma: Calcified tumour, usually in children Leiomyosarcoma: Large areas of necrosis Liposarcoma: Heterogenous fatty density Hemangiopericytoma: Hypervascularity

7. CT-guided core biopsy • Reserved for cases in which a diagnosis will change therapy, such as the need for neoadjuvant chemotherapy for I. GIST-imatinib mesylate 2. Germ cell tumours 3. Lymphomas 8. Laparoscopic biopsy/retroperitoneoscopy • Equivocal history and diagnostic dilemma • Unusual appearance of the mass • Unresectable tumour • Distant metastasis Treatment: Surgery is the main modality and the most effective modality of the treatment. It can be curative if R-0 resection is achieved. Chemotherapy, radiotherapy are complementary to surgery. A few cases are also managed by neoadjuvant therapy. I. Surgery Principles: Extirpative surgery is the principal and most effective form of therapy for primary retroperitoneal tumours. Tumour histology, tumour size, or patient age not significant factors in survival in multiple-variable analysis. Therefore carefully planned and skillfully executed surgical therapy is critical for any chance at long-term success. Adjuvant therapy High local recurrence rate and eventual mortality from this disease has prompted the exploration of adjuvant therapeutic modalities. Postoperative radiation increased toxicity to surrounding structures. II. • 1. 2.

Radiotherapy Radiotherapy: Two types EBRT-external beam radiotherapy Brachytherapy for retroperitoneal leiomyosarcoma.

Ill. Chemotherapy Doxorubicin is the foundation for chemotherapy m advanced sarcoma.

663

TEN COMMANDMENTS 1. Complete R-0 resection should be the aim-single most important positive predictive feature. 2. Generous incision should be given to get into good access to the tumour 3. Intra-abdominal adhesions should be released to separate the tumour. Should use of sharp, curved Mayo scissors for meticulous dissection. 4. Should never do enterotomy (accidental) because an enterotomy and subsequent fistula formation can cause major morbidity. 5. Should preserve all important vessels. 6. Should preserve important organs such as kidney, ureter colon, etc. 7. Should resect the organs if found infiltrated and if R-0 resection is possible. 8. Should do a centripetal dissection. It allows one to dissect those areas that are amenable to dissection. 9. Should use surgical clips, should be placed to mark the periphery of surgical field and other relevant structures to help guide potential future radiation therapy. 10. Both radiotherapy and chemotherapy are given.

• MAID regimen: Mesna, adriamycin (doxorubicin), ifosfamide, and dacarbazine has been successful in neoadjuvant programmes for sarcomas of the extremities compared with historical controls. Yet less data are available with regard to other soft tissue sites. Neoadjuvant therapy for retroperitoneal sarcoma. Advantages • The viscera are often displaced by the tumour volume and a lack of surgical adhesions further reduces dose to the bowel. • Effective radiation dose is lower in the preoperative setting. DIFFERENTIAL DIAGNOSIS OF RETROPERITONEAL SARCOMA These are pathological variants. It is difficult to consider them as a diagnosis on clinical grounds. However, more common ones have to be considered first such as liposarcoma. Patients with von Recklinghausen's disease may have neural tumours. A few differential diagnosis are given below. LIPOSARCOMA Most common of primary RPT, 20% from retroperitoneum. • Histological types I. Well-differentiated liposarcoma (low grade) 2. Myxoid/round cell liposarcoma (50%-most common) 3. Pleomorphic liposarcoma (10-15%-high grade) 4. Dedifferentiated liposarcoma-the rate of metastasis depends on the degree of tumour differentiation, with nearly 90% of poorly differentiated tumours meta­ stasizing.

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• Pathology: The key feature of a liposarcoma is the lipoblast, which is essentially an immature fat cell. LIPOBLAST have multiple fat vacuole which compress the nucleus, creating a scalloped appearance. LEIOMYOSARCOMA • 50% from retroperitoneum-Female: Male-2: 1 • Site of origin (soft tissue, vascular or superficial), although many of the soft tissue lesions are believed to originate from smaller blood vessels. • Immunohistochemistry stain for smooth muscle myosin, vimentin, and actin and less often for desmin. (Leiomyomas stain positive for desmin, which separates them from their malignant counterpart.) • They stain negative for S-100. MFH (MALIGNANT FIBROUS HISTIOCYTOMA) • Less common in the retroperitoneum. • Derived from fibroblast differentiation (previously defined as a sarcoma of primary histiocytic origin) • Storiform-pleomorphic (40-60%) and myxoid type (25%) are subtypes. Other types being giant cell type and inflammatory. RETROPERITONEAL TERATOMA • These are the tumours arising from totipotential cells. Thus, they can have ectoderm, mesodenn or endoderm elements. • l 0% of all primary RPT • Rare in adults because of its congenital nature. • Solid teratoma malignant (likely). • Malignant mature cystic teratomas (0.2 to 2% of cases) have the potential to metastasise to sites such as the retroperitoneal lymph nodes and lung parenchyma.

RHABDOMYOSARCOMA • 6% in retroperitoneum • More common in children • Sporadic-most common - Genetic risk factor-10-33%: Li-Fraumeri syndrome, neurofibromatosis. SCHWANNOMA • Majority has mutations in NF2 gene. • Majority are sporadic tumours. • A minority (l 0%) are associated with syndromes such as neurofibromatosis type 2, schwannomatosis and multiple meningiomas. • Hallmark of schwannoma is alternating areas of cellular (Antoni A) and hypocellular (Antoni B) areas. • Retroperitoneal tumours are larger and often show degenerative changes such as cystic change, haemorrhage and calcifications. • Immunohistochemistry: Diffuse S-100+ is characteristic.

WHAT IS NEW IN THIS CHAPTER?/RECENT ADVANCES

• All topics have been updated with flowcharts and coloured pictures. • More details on mesentery and retroperitoneum are added. • Retroperitoneal mass/sarcoma and abdominal compartment syndrome are discussed in more detail. • Misty mesentery valentine syndrome and mesenteric cyst have been added.

MULTIPLE CHOICE QUESTIONS 1. One need not close peritoneal layer after laparotomy because: A. The peritoneum can get stuck to the bowel B. Flattened mesothelial cells heal within a few hours C. The peritoneum tears when closure is attempted D. It is very painful postoperatively

3. Which of the following is an example of primary peritonitis? A. Tuberculous peritonitis B. Perforation peritonitis C. Postoperative peritonitis D. Parturition peritonitis

2. Peritoneum can be used for dialysis because: A. It is close to kidney B. It is faster than haemodialysis C. Capillaries and lymphatics between two layers of peritoneum help in absorption and exudation D. It covers entire abdomen

4. Which of the following organisms are most commonly involved in secondary peritonitis? A. Enterococci B. Streptococci C. Staphylococci D. Pneumococci

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5. The following are the typical features of acute generalised peritonitis except: A. Abdominal pain B. Persistent vomiting C. Bradycardia D. High-grade fever with chills

13. Following are features of tuberculous peritonifo except: A. Tubercles over peritoneal surface B. Encysted form C. Can be a military form D. Transudate

6. Abdominal tap is done in peritonitis for all of the following roles except: A. Aspiration of blood to indicate haemoperitoneum B. Aspiration of pus indicating infection with gram­ negative bacteria C. Aspiration of bile indicating biliary peritonitis D. Aspiration of urine indicating ureterocoele

14. Nonoperative treatment for peritonitis may be followed in the following except: A. Moribund patients B. Sealed perforation C. Localised peritonitis D. Generalised peritonitis

7. The following suture material is best suited for closure of bowel perforation: A. Silk B. Catgut D. Thread C. Nylon 8. History of discharge per rectum for the first time in a patient who is recovering from peritonitis suggests: B. Pelvic abscess A. Anal prolapse D. Colitis C. Proctitis 9. What forms the anterior relationship of Rutherford Morrison's space? B. Kidney A. Liver D. Duodenum C. Diaphragm 10. Subphrenic abscess is common on the right side because of the following reasons except: A. Majority of the diseases affect right side B. Right lung is larger C. Left paracolic gutter is narrow and colophrenic ligament is present on the left side D. Right paracolic gutter is large and colophrenic ligament is absent on the right side 11. Indications for open drainage of subphrenic abscess include the following except: A. Persistent fistula discharging pus B. Thick viscid pus C. Abscess very close to IVC/diaphragm D. Single loculus 12. Intra-abdominal pressure exceeds ____ cm H2 0 in abdominal compartment syndrome. B. 25 A. 15 D. 45 C. 35

15. The following catheters are commonly used for percutaneous drainage of subphrenic abscess: B. Trocar catheter A. Pigtail catheter C. Sump catheter D. Foley's catheter 16. More reliable sign of peritonitis is: A. Cough tenderness B. Tenderness on pressure C. Rebound tenderness D. Guarding 17. Presence of sunken eyes, pale and pinched face, dry cracked tongue, cold perspiration and cyanosis are all typical features of: B. Gargoyle facies A. Hippocratic facies D. Mask like facies C. Marshall hall facies 18. Following are risk factors for spontaneous bacterial peritonitis except: A. Cirrhosis B. Nephrotic syndrome C. Chronic renal failure D. Carcinoma stomach 19. Which of the following is true for pneumococcal peritonitis? A. Common in young boys B. Age is around 15 years C. Peritoneal fluid is transudate E. It is typically odourless 20. Following are about pseudomyxoma peritonei except: A. Common in women B. Ovary is the main source C. Surgery cannot cure the disease D. Chemotherapy is also used

ANSWERS B

2 C

3 A

4 A

5 C

6 D

7 A

11 D

12 C

13 D

14 D

15 A

16 C

17 A

8

B

9 D

10 B

18 D

19 D

20 B

28 Small Intestine • • • • • • • • •

Anatomy Physiology Abdominal tuberculosis Tuberculous peritonitis Glandular tuberculosis Intestinal tuberculosis Inflammatory bowel diseases lleostomy Surgical complications of enteric fever

Introduction Truly speaking small intestines extends from pylorus to ileo­ caecal junction. However, for all practical purposes it is discussed as starting from duodenojejunal flexure till caecum. Small intestines play an important role not only in the transfer of food contents distally but in the digestion, absorption and secretion of the contents. Being the central portion of the GI tract with long length, many diseases affect the intestine. Surgically important topics have been covered in this chapter.

Intestinal amoebiasis Radiation enteropathy Peutz-Jegher syndrome GIST Carcinoid tumour Short gut syndrome Diverticula Intestinal fistula What is new?/Recent advances

• Differences between jejunum and ileum have been given in Key Box 28.1.

MIii,........,.� DIFFERENCES BETWEEN JEJUNUM AND ILEUM

• •

. . .

ANATOMY • Small intestine consists of proximal 2/5 jejunum and distal 3/5 ileum. It is about 6 metres in length. • Small intestine starts at duodenojejunal flexure just to the left of the inferior mesenteric vein. Surgical importance: To identify the first (short) loop of jejunum for gastrojejunostomy. • Small intestine ends at ileocaecal junction. In cases of intestinal obstruction, trace up to ileocaecal junction. If caecum is distended, it is a case of large bowel obstruction. If caecum is collapsed, it is a case of small bowel obstruction. • Jejunum resides in the left side of the peritoneal cavity and ileum on the right side.

• • • • • • • • •

• •

Length Diameter Wall Colour Peyer's patch Blood supply Mesentery

Jejunum 2/5 Wider (2-4 cm) Thick and double (mucous membrane can be felt) Deep red Very, very less

Ileum 3/5 Less (2-3 cm) Thin

Long and few vasa rectae (1 or 2) Transparent, less fat

Short and numerous (5 or 6) More fat

Pale pink More

Blood Supply (Fig. 28.1) • Superior mesenteric artery is the artery of the midgut which supplies the entire midgut (entire small intestine). Jejuna! arteries are end-arteries. • Mesenteric border of the intestine gets more blood supply when compared to anti-mesenteric border. Hence in cases of diminished blood supply, anti-mesenteric border become ischaemic first. • Venous drainage is through superior mesenteric vein.

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Small Intestine

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Microscopic Anatomy • Basic unit of small bowel mucosa is the villus, which is a finger-like projection. Each villus is covered with tall columnar epithelium. • Goblet cells, Paneth cells and endocrine cells are seen in the crypts. Goblet cells are mature mucous cells. Endocrine cells (enterochromaffin cells) have cytoplasmic granules which secrete 5-hydroxytryptamine, neurotensin, glucagon and motilin. Importantly, mucosa! cell mediated immunity is brought by mucosa! T lymphocytes. Jejunum

Ileum

Fig. 28.1: Blood supply-jejunum and ileum

Mesentery • It is a fan-shaped fold of peritoneum which attaches jejunum and ileum to posterior abdominal wall. • Blood vessels and lymphatics course in between the folds of peritoneum. • It extends from the left of duodenojejunal flexure (left of L2) vertebra to the right sacroiliac joint, thus fixing the ileocaecal junction there. • The importance of direction of the mesentery is appreciated in the following examples: A. Mesenteric cyst moves at right angles to the direction of the mesentery. B. Mesenteric lymph nodes can be clinically palpable as a nodular or a smooth mass. C. Horizontal tear in the mesentery causes more gangrene of the bowel than vertical tear (Fig. 28.2). • Structures crossed by mesentery: Duodenum, aorta, inferior vena cava, right ureter, right psoas major and right gonadal vein.

FUNCTIONS - PHYSIOLOGY • Motility: Two types of muscle contractions occur-one which does not propagate-it exposes the food contents to the absorptive surface for a longer time by causing segmentation thus better absorption of the food. Another type is peristaltic which propagates the food contents. Control of peristalsis is done by myenteric plexus. Time taken by the solid food contents to reach from mouth to colon is about 4 hours (Key Box 28.2). • Absorption and digestion: Except calcium and iron almost everything is absorbed in the small intestines. To give a few examples: out of 6-10 litres of water, almost 80% water is absorbed in the small intestine and only I 0-20% is discharged into colon. Thus in cases of terminal ilea! obstruction, about 8-10 litres of fluid accumulate resulting in gross distension of the abdomen and dehydration. Carbohydrates and fat are mainly absorbed in duodenum and proximal jejunum. Proteins require pancreatic enzymes. Hence, they are broken down in the jejunum into amino acids and peptides. Conjugated bile acids are absorbed in the terminal ileum wherein enterohepatic circulation takes place and again they are secreted in the bile. Thus in ilea! resections or diseases like Crohn's disease, more amount of bile acids enter colon resulting in diarrhoea due to increased secretion of water and electrolytes.

M!IMWIIIIIIIIIIIIIIIW�

Fig. 28.2: Observe blood supply of the mesentery and it is but natural that a horizontal tear causes more gangrene

FUNCTIONS OF THE SMALL INTESTINE • Digestion and absorption • Synthesis of lipoproteins • Secretion of regulatory peptides - Secretin - Cholecystokinin - Somatostatin -VIP • Immune function: Production of immunoglobulins (lgA). The B cells and T cells help in phagocytosis and secretion of cytokines.

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Diseases of the small intestines Following are the various diseases of the small intestines described in the texts. • Abdominal tuberculosis which includes intestinal, mesenteric and peritoneal tuberculosis. We should realise that all three types are very much connected to each other in one way or other and hence are discussed together. • Inflammatory bowel disease includes ulcerative colitis and Crohn's, both are important health problems in the Western world. Other inflammatory conditions include enteric fever and its manifestations, intestinal amoebiasis, HIV associated enteropathy and radiation-induced enteropathy. • Tumours include benign and malignant tumours including carcinoid tumours. • Small intestinal obstruction, Meckel's diverticulum, superior mesenteric vascular occlusion and necrotising enterocolitis have been discussed in Chapter 30 on intestinal obstruction. ABDOMINAL TUBERCULOSIS (TB) Introduction Abdominal tuberculosis is a common extrapulmonary mani­ festation of tuberculosis. Incidence in the West has increased due to immigrant population and increased incidence of HIV infections. In India, extrapulmonary TB is also showing re­ emergence due to incomplete treatment and occurrence of multidrug-resistant strains. Hence, it is a major health problem. Definition • The term abdominal tuberculosis includes tuberculous infection of gastrointestinal tract, mesenteric lymph nodes, peritoneum, omentum and solid organs related to gastrointestinal tract such as liver and spleen. Classification (Key Box 28.3) The commonly encountered four forms of tuberculosis are given below: 1. Tuberculous peritonitis 2. Tuberculous mesenteric lymphadenitis-glandular tuberculosis 3. Intestinal tuberculosis 4. Tuberculosis of solid viscera such as liver and spleen. Routes of spread of infection and pathogenesis 1. Intestinal tuberculosis is caused by Mycobacterium tuber­ culosis from swallowed sputum (pulmonary tuberculosis) or milk (milk-borne infection-Mycobacterium bovis). From intestinal tuberculosis, mesenteric nodes get involved and later, the peritoneum can get involved. 2. Blood spread: Infection from pulmonary tuberculosis can spread through blood during bacteraemic phase. 3. Lymphatic spread from tuberculosis of intestines.

MIP#l........_.� VARIOUS FORMS OF ABDOMINAL TB I. Peritoneal tuberculosis: Acute, chronic A. Chronic forms 1. Ascitic type (wet) • Generalised • Localised 2. Fibrous type (dry) • Adhesive, plastic • Miliary nodule type B. Tuberculosis of peritoneal folds 1. Mesenteric adenitis 2. Mesenteric cysts/abscesses 3. Bowel adhesion II. Gastrointestinal 1. Ulcerative 2. Hyperplastic 3. Sclerotic/plastic Ill. TB of solid viscera 1. Liver 2. Spleen

4. Genitourinary tuberculosis: From here, cephalad spread occurs and thus, peritoneum gets affected. 5. From bile: Granuloma in liver. Bacilli are excreted in bile. Pathology and pathogenesis (Fig. 28.3). Investigations It is important to realise that there are so many investigations for abdominal tuberculosis. Although it is essential to have a theoretical knowledge about all these investigation, all of them need not be done during clinical management. To give an example: If chest X-ray and sputum AFB are positive, one should start ATT that need not do costly investigations such as CT scan or even diagnostic laparoscopy, etc. Investigations done by the clinician should be complementary to each other. 1. Complete blood picture (CBP) which include Hb%, TC, DC and ESR. Haemoglobin may be low indicating anaemia. Anaemia has to be corrected before surgery. 2. ESR will help in equivocal cases. High values and the clinical situation may force the clinician to start anti­ tuberculous treahnent in selected patients. However, with treatment, if ESR comes down and patient is symptomatically better with weight gain, settling fever, improving appetite, it suggests tubercular pathology. 3. Sputum AFB (acid fast bacilli): Demonstrated by Zeihl­ N eelsen method. Many patients with abdominal tuberculosis will not have pulmonary tuberculosis. However, if sputum is present, it must be tested for AFB 4. Chest X-ray may suggest tuberculosis in the form of cavity, calcification, etc.

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MYCOBACTERIUM TUBERCULOSIS

Infected sputum

Haematogenous

lleocaecal region

lleocaecal tuberculosis

Mesenteric node enlargement

Fibrosis

• Rolled up omentum • Loculated ascites

Perforation

Abscess

Abscess • Napkin ring stricture • Intestinal obstruction

Peritonitis Matting-coccon

Fig. 28.3: Abdominal tuberculosis-pathology and pathogenesis

5. Mantoux test is nonspecific but a strong ulcerated Mantoux test result suggests tuberculosis. 6. Ultrasound, being a noninvasive investigation is an imaging of choice. • Ascites can be demonstrated and the aspirated fluid is sent for analysis • Focal ascites between loops of bowel-Club sandwich sign may be seen • Enlargement of mesenteric lymph nodes (common) and retroperitoneal nodes (uncommon) can be detected • Dilated loops and sometimes peritoneal tubercles are seen as echo-poor shadows • Thickening of omentum, mesentery, peritoneum can be found out. (However ultrasound is not the best investigation to detect these findings). • Pseudokidney sign: Pulled caecum identified in the right hypochondrium • Hepatosplenomegaly may be present 7. Ascitic fluid analysis: • Ultrasound guided fluid is aspirated and about 20--40 ml is sent for analysis (Key Box 28.4). 8. CECT: Contrast enhanced CT scan of the abdomen: • CT scan is objective. All the findings which can be detected by ultrasound can be confirmed by CT scan. Addition of the contrast is definitely more superior in detecting strich1res, dilatations, perforations, and more importantly loculated ascites and intra-abdominal

ll!MHIWlllllllllllllllllllf'� ASCITIC FLUID ANALYSIS (STRAW-COLOURED FLUID)

• Specific gravity is increased-2020 or more • Glucose< 30 mg% • AFB is rarely demonstrated 500/cells/cumm), predominantly lymphocytic • Increased total protein (> 2.5 g/dl) • Serum/ascitic fluid albumin gradient (SAAG*) < 1.1 g% units • LDH > 90 units/L • Decreased pH • Increased adenosine deaminase • Bacterial isolation and culture is possible in 20-45% of patients. • SAAG = Serum albumin level-ascitic fluid albumin level

collections. CT guided biopsy can be done. If dis­ tension or matting of loops and adhesions are present, it is not safe. 9. Barium studies: These are not done routinely. If diagnosis is possible by the various investigations mentioned above, there is no necessity to do them. In fact it can harm the patient by precipitating obstruction and barium peritonitis if there is a perforation.

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10. • • • 11.

• • • • • • • •

• Enteroclysis: Dilatation and narrowed segments in partial obstruction. • Narrowing of terminal ileum (Fleischner's sign), Fibrotic terminal ileum opening into the contracted caecum (Stier Jin's sign) • Barium enema: Pulled up caecum, normal acute ileocaecal angle becomes obtuse or sometimes straightening of the ileocaecal angle. Endoscopy: Upper gastroduodenoscopy may detect tubercles in the stomach or duodenum - rare Push enteroscopy: Ulcers in the proximal jejunum can be detected and biopsy can be taken-chances of perforation are high. Colonoscopy can detect nodular lesions, ulcerations in the colon-caecum and terminal ileum (last 10 cm of ileum should be entered and biopsy should be taken). Laparoscopy: This is a diagnostic investigation as it gives the tissue diagnosis (Figs 28.4 and 28.5). One can also evaluate all possible viscera, peritoneum, omentum and pelvic organs. Biopsy is possible under direct vision. Findings may include the following (Key Box 28.5). Peritoneal cavity: Fluid collection-clear or straw­ coloured or abscess secondary to perforation Omentum: Tubercles, rolled up omentum Peritoneum: Tubercles, nature of the tubercles, caseation. Intestines: Matting of the loops of bowel, adhesions, bands, strictures, dilatations, perforations of bowel Root of the mesentery: Short mesentery, caseation of lymph nodes-cold abscess Lymph nodes: Mesenteric lymph nodes enlargement, caseation Caecum: Pulled up caecum Hepatosplenomegaly, tubercles on the surface of the liver. Laparoscopy can also be therapeutic, if a stricture is identified, the diseased loop is isolated, brought out and resection and anastomosis/stricturoplasty done.

LAPAROSCOPY-IMPORTANT FINDINGS • • • • • •

Lymph nodes Adhesions, matting Peritoneal nodules Ascites - aspiration - AFB staining Rolled up omentum Obstruction, stricture You can remember as LAPARO

_,___ Peritoneal surface tubercles

.+-----'r-- Tubercles on the viscera

Fig. 28.4: Various sites from where laparoscopic biopsy can be taken in suspected case of abdominal tuberculosis. Biopsy from peritoneal surface and omentum is safe. Otherwise lymph node biopsy can be done

Fig. 28.5: Extensive abdominal tuberculosis. Laparoscopic biopsy being done

12. Polymerase chain reaction (PCR): • It is a technique used in medical and biological research labs. • One can do functional analysis of genes; useful in the diagnosis of hereditary diseases; • Helps in detection and diagnosis of infectious diseases such as tuberculosis • Laparoscopically biopsied tissue can be sent for PCR. It can detect 1-2 organisms or 8 fg of mycobacterial DNA • Positive PCR indicates infection but it need not be active infection. Hence, it is inferior to tissue diagnosis • PCR has 97% sensitivity and 99% specificity When to use what investigations? Tn the diagnosis of the abdominal tuberculosis, investigations can be classified as follows. 1. Routine in all cases: CBP including ESR, chest X-ray and sputum for AFB. Lymph node biopsy-If lymph nodes are palpable (peripheral-cervical, axillary preferable -

Small Intestine

inguinal last choice). Advantage being lymph node biopsy can be done under local anaesthesia. If it is positive, all other invasive and costly investigations can be avoided (see clinical notes below). Imaging: Ultrasound examination followed by guided ascitic fluid analysis has become a routine imaging test. It may not be diagnostic but since it has more advantages than any side-effects, it can be done. 2. Selected cases: PCR, CT scan, small bowel enteroscopy, barium studies, colonoscopy. If any one of these gives a diagnosis, treatment is started. In the presence of obstruc­ tion, avoid barium studies, enteroscopy and colonoscopy. The decision is made on clinical grounds to proceed with laparotomy. 3. Finally: Diagnostic laparoscopy/tissue diagnosis-this is the investigation of choice-often done as a last choice when all investigations are equivocal or have failed to give a diagnosis. Antituberculous Treatment • Details are given in medicine textbooks. However, 4 drug regimen for 2 months followed by 2 drug regimen for 4 months is recommended as a first line of treatment. • First line of drugs include INH, rifampicin, ethambutol and pyrazinamide given for 2 months. This is followed by rifampicin and INH for 4 months. Refractory cases are treated by kanamycin, ofloxacin, ciprofloxacin, amikacin, etc. see clinical notes. TUBERCULOUS PERITONITIS It can be of two types: Acute and chronic. Basically, it produces the following pathological changes: I. Intense exudation which causes ascitic form

An 18-year-old girl presented with colicky abdominal pain, low grade fever, gross emaciation, etc. Barium meal follow through demonstrated strictures of the small bowel. Laparotomy was done. There were dense adhesions. Minimal dissection resulted in two openings in the terminal ileum. Peritoneum was studded with tubercles from which biopsy was taken. Abdomen was closed with a drainage tube. Postoperatively, the patient was given antituberculous treatment. After three months, the patient came to the outpatient department and had put on 14 kg weight. She was asymptomatic. This case history illustrates the presence of tuberculosis as a major cause of illness in India. It also highlights the importance of antituberculous treatment and recovery.

671

2. Exudation with minimal fibroblastic reaction-loculated form 3. Extensive fibroblastic reaction-plastic form 4. Fibroblastic with secondary infection-purulent form • In most of the cases, tuberculous peritonitis results from reactivation of latent primary peritoneal focus. Types 1. Ascitic form (Fig. 28.6) (generalised variety) • It is common in children and young adults. The child is brought to the hospital with abdominal distension. • Omentum can be felt as a rolled up transverse mass, which is nodular due to extensive fibrosis. Abdomen has a doughy feel with fluid giving rise to shifting dullness. • Aspiration of peritoneal fluid reveals exudate, which is rich in lymphocytes (Key Box 28.6). • Peritoneal cavity contains pale-straw-coloured fluid and the peritoneal surface is studded with tubercles. • Umbilical hernia or congenital hydrocoele appears in children due to increased intra-abdominal pressure. 2. Loculated or encysted form (Fig. 28.7) • In this variety, ascitic fluid is present in one quadrant of the abdomen which is sealed off by matted intestinal coils surrounded by omentum. It gives rise to localised swelling. These patients have no shifting dullness. • It commonly presents in adults. • Differential diagnosis: Other cystic swellings in the abdomen such as pseudocyst of the pancreas, mesenteric cyst, retroperitoneal cyst.

M!ffl,t..........r� CAUSES OF EXUDATIVE ASCITES-INCREASE IN PROTEIN Pancreatic ascites Rare cause: Meig's syndrome Occlusion: Budd Chiari syndrome Tubercular peritonitis Excess of chylomicrons Infective: Peritonitis Neoplasm of peritoneum (carcinoma peritonei) Remember as PROTEIN

+ +

+ +

Fig. 28.6: Ascitic form

Fig. 28.7: Encysted ascites

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3. Fibrous peritonitis (plastic) (Fig. 28.8) • In this variety, there is no ascites but there is extensive fibrosis which results in dense adhesions between the coils of intestines. Intestines are matted, distended and not able to empty properly due to adhesions and bands. It is associated with strictures. • This gives rise to blind loop with steatorrhoea and emaciation. • Usually, it presents with intestinal obstruction at a later date due to fibrous band which needs to be divided to relieve the obstruction. In some occasions, it is not possible to enter the peritoneal cavity, due to dense adhesions. • It is not uncommon to create openings in the bowel at laparotomy and end with a helpless situation wherein one will not be able to close the perforation. The net result is fistula formation. 4. Purulent variety (Fig. 28.9) • Seen in females as a complication of genitourinary tuberculosis (tuberculous salpingitis). • The spread occurs through the female genital tract and there is always secondary infection. • It presents with acute peritonitis at laparotomy, the peritoneal cavity is seen studded with tubercles, cold abscesses and pus. • Laparotomy, drainage of pus, followed by antitubercu­ lous treatment is the choice of therapy. • It carries poor prognosis because of complications such as toxaemia and faecal fistula formation. • Tuberculous peritonitis can be associated with infections of pleural space and pericardia( space (effusion). It is called polyserositis syndrome. • See also Figs 28.10 to 28.13

Fig. 28.11: Adhesive form

Fig. 28.12: Tubercles on the peritoneal surface

• Fig. 28.13: Tuberculous peritonitis Fig. 28.8: Fibrous peritonitis

Fig. 28.9: Purulent type

Fig. 28.10: Fibrous bands

TUBERCULOUS MESENTERIC LYMPHADENITIS Clinical presentation 1. As a calcified lesion (Fig. 28.14) along the line of mesentery, which extends from L2 vertebra, at the left of vertebral column to the right sacroiliac joint. In 50% of cases, there is no active infection but in the remaining, there is infection. If the symptoms are that of tuberculosis, antituberculous treatment should be given. The shadow caused by lymph nodes are round to oval, mottled and may be regular or irregular.

Small Intestine

673

INTESTINAL TUBERCULOSIS

•

Fig. 28.14: Mesenteric lymph nodes

Fig. 28.15: Acute lymphade­ nitis

Ileocaecal region is commonly involved in tuberculosis because of the following reasons: 1. Rich lymphatics in Peyer's patches. 2. Presence of alkaline media favours the growth of the organisms. 3. Presence of ileocaecal valve precipitates stasis. 4. Terminal ileum is the area of maximum absorption. Types (Table 28.1 for comparison) 1. Ulcerative variety 2. Hyperplastic variety 3. Mixed (sometimes) Clinical features (Figs 28.18 to 28.20)

•

Fig. 28.16: Chronic lymphade­ nitis

Fig. 28.17: Pseudomesen­ teric cyst

2. Acute mesenteric lymphadenitis (Fig. 28.15) • Common in children, clinically mimics acute appendi­ citis. • Pain in the right iliac fossa, vomiting, fever, rigidity can be present. • On palpation, tender mass of swollen lymph nodes can be felt in the right iliac fossa. • Laparotomy, appendicectomy and biopsy of the lymph node is the procedure of choice. 3. Chronic lymphadenitis (Fig. 28.16) in children presents as failure to thrive. Fever, loss of weight, loss of appetite, emaciation and pallor are present. Abdomen is protuberant. On deep palpation, nodes can be felt in the right iliac fossa. These nodes have to be differentiated from nodes that enlarge due to lymphoma. 4. Pseudomesenteric cyst (Fig. 28.17) • T his is due to caseation of mesenteric lymph nodes confined within two leaves of mesentery. • Due to adhesions, intestines can get kinked or twisted causing intestinal obstruction. Treatment • Antituberculous treatment (details in medicine books).

In general, intestinal tuberculosis presents with the following symptoms: • Abdominal pain: It is the most common symptom. It can be a dull, vague pain or colicky pain (stricture) which increases after taking food or relieved by vomiting. • Diarrhoea: Watery, small quantity, abnormally foul smelling. It may alternate with constipation. • Nonspecific symptoms such as flatulence, noisy sounds in the abdomen (borborygmi) are not uncommon. • Abdominal distension: It is due to ascites and subacute intestinal obstruction. • Weight loss is very common. Anorexia, tiredness, pallor may be the presenting features. Signs • Typically patients are malnourished and pale. • Visible intestinal peristalsis may be seen. • Distended bowel loops can be palpated. • Doughy abdomen in case of peritoneal involvement. • Rolled up omentum, mass in the right iliac fossa or lumbar region, loculated ascites, etc. are other features (Fig. 28.21). Management of intestinal tuberculosis 1. No evidence of intestinal obstruction: Antituberculous treatment. 2. With obstruction (stricture) (Figs 28.22 and 28.23) A. Solitary stricture: It is best treated by stricturoplasty by incising the stricture longitudinally and suturing it transversely (Fig. 28.28). B. Multiple strictures at long intervals: Stricturoplasty is the ideal treatment. C. Multiple strictures within a short segment. Resection is the ideal treatment (Fig. 28.30). PEARLS OF

WISDOM

Stricture within 10 cm from the ileocaecal junction is best resected.

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Comparison of two forms of intestinal tuberculosis (TB)

Ulcerative variety Secondary to pulmonary TB. Occurs due to swallowing of TB bacilli (Mycobacterium TB) Terminal ileum 2. Site 3. Virulence of the organism More virulent Very poor 4. Resistance of body Multiple ulcerations in the terminal 5. Pathology ileum with/without involvement of lymph nodes. Ulcers are transverse. Serosa is reddened and oedematous. Symptoms of pulmonary TB, blood 6. Clinical features and mucus in stool resulting in gross emaciation and cachexia. Diarrhoea is also a feature. • Acute TB, ulcer perforation-ulcers 7. Complications are transverse because they follow the lymphatics. Treatment is laparo­ tomy and resection of bowel. • Chronic: Healing of ulcer results in stricture of terminal ileum and subacute intestinal obstruction. Demonstrates a stricture, or multiple 8. Barium meal follow strictures. In the initial stages, through (Figs 28.24 ileum is not seen due to hypermotility. and 28.25) Positive 9. Chest X-ray and sputum AFB (Fig. 28.26) 10. Colonoscopy and biopsy To confirm the diagnosis (Fig. 28.27) 1. Aetiology

Fig. 28.18: Step ladder peristalsis is seen in a patient who underwent appendectomy for pain in the right iliac fossa. The diag­ nosis made for acute appendicitis and appendicectomy was done. Postopera­ tively patient has step ladder peristalsis. The case was ileocaecal tuberculosis

Hyperplastic variety It is a primary intestinal TB, due to M bovis. Milk-borne infection or due to Mycobacterium TB-low grade infection Ileocaecal region Less virulent Good It is low grade, chronic continuous inflammation involving ileocaecal region resulting in cicatrising granuloma in right iliac fossa (mass in right iliac fossa). Abdominal pain and diarrhoea may be the initial symptoms for a long time and later fever, weight loss and subacute intestinal obstruction occur. Nodular, mobile, firm mass in the right iliac fossa which later produces subacute intestinal obstruction.

Barium enema can demonstrate (i) contracted caecum, (ii) pulled up caecum (subhepatic), (iii) luminal obstruction, and (iv) obtuse ileocaecal angle. Negative To confirm the diagnosis

Fig. 28.19: Distension of the abdomen due to ascites

Fig. 28.20: Severe hypoproteinaemia­ you can see bilateral pedal oedema

Small Intestine

Fig. 28.21: Mass in the right iliac fossa-hyperplastic variety

Fig. 28.24: Enteroclysis showing stricture of the jejunum with proximal dilatation

Fig. 28.26: Plain X-ray showing dilated air fluid levels

675

Figs 28.22 and 28.23: Tuberculous stricture with intestinal obstruction-observe the stricture, tubercles and massive proximal dilatation

Fig. 28.25: Barium enema-'pulled up' caecum

Fig. 28.27: Colonoscopy showing nodular leison in the ascending colon. Biopsy proved tuberculosis

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3. Surgical treatment ofhyperplastic tuberculosis: Limited resection is the treatment of choice. It includes removal of terminal 8-10 cm of the diseased ileum, caecum with appendix, diseased po1tion of the ascending colon, followed by ileocolic anastomosis. All these cases have to be given antituberculous treatment for a period of 9-12 months. Nutritional supplementation to improve albumin and haemoglobin levels and if necessary, blood transfusion before and after surgery help in smooth recovery in the postoperative period (Fig. 28.29).

Caecum

Fig. 28.30A: Limited resec­ tion-resection of the diseased segment

Fig. 28.308: Opened bowel showing ulcers and narrowed segment Fig. 28.28: Stricturoplasty

Fig. 28.30C: Tubercular stricture of jejunum Fig. 28.29: Limited colectomy followed by ileocolic anastomo­ sis. (Courtesy: Miss Vidushi Kapil, MBBS student, KMC, Manipal)

Complications of abdominal tuberculosis 1. Intestinal obstruction: Usually it is ilea! or jejuna!

obstruction-details have been already discussed 2. Perforation: It is not uncommon. Carries 6-8% mortality rate specially in late cases with peritonitis. 3. Malnutrition: Diarrhoea, loose stools, blind loop syndromes contribute for malnutrition

4. Faecal fistula is usually due to operated cases of intestinal tuberculosis by resection and anastomosis. In such cases, anastomotic dehiscence may result in faecal fistula. The faecal fistula is not due to a direct internal involvement of viscera unlike in Crohn 's disease. 5. Disseminated tuberculosis per se from abdominal tuberculosis is not common. Most of such cases have fulminant pulmonary tuberculosis. This happens in untreated cases and immunocompromised cases.

Small Intestine

INFLAMMATORY BOWEL DISEASES Introduction These are the diseases involving small and large bowel, of unknown aetiology, characterised by multiple ulcerations in the bowel, clinically manifesting as blood and mucus in stools. Ulcerative colitis and Crohn's disease (regional enteritis) are included under this heading. However, both these diseases are uncommon in India. Hence, amoebic dysentery, intestinal TB, enteric fever with intestinal manifestations can also be included in this. Both diseases can present has acute abdomen with intestinal obstruction and complications such as perforation toxaemia, etc. Also both are premalignant conditions for carcinoma colon. ULCERATIVE COLITIS Aetiology 1. Autoimmune factor: Even though exact mechanism of ulcerative colitis is not clear, there are some factors which may point out to autoimmune reaction. They are: • Presence of cytotoxic T lymphocytes against colonic epithelial cells in the lamina propria of the bowel. • Presence of anticolon antibodies. • Whatever be the immune mechanisms, activation of inflammatory mediators such as cytokines, growth factors and arachidonic acids takes place and they are responsible for the disease. 2. Dysfunctional immunoregulation in the intestinal wall results in inappropriate production of cytokines. This creates an imbalance between various interleukins resulting in inflammatory changes. 3. Psychosomatic and personality factors: Ulcerative colitis is more common in western women. Emotional stress, family stress, stress from divorce are the contributing factors. PEARLS OF

WISDOM

Western, white, worrying women's disease is ulcerative colitis. 4. Dietary factors • Westernisation of the diet which is rich in red meat has been blamed. Vegetarian diet is supposed to protect the colonic mucosa. • Allergy to milk protein is responsible for ulcerative colitis in a few patients. 5. Defective mucin production and a defective mucosa! immunological reaction is considered as a chief factor responsible for ulcerative colitis.

677

PEARLS OF

WISDOM

Appendicectomy and smoking have been protective factors for development of ulcerative colitis. 6. Genetic: 15% of patients have first degree relatives with ulcerative colitis. Pathology (Key Box 28.7) • The disease always starts in the rectum and spreads in a backward manner, thus involving the entire colon in majority of cases. In 5% of cases, tenninal ileum can also be involved-back wash ileitis. • Anus is not involved in ulcerative colitis. • The disease manifests as multiple, small superficial ulcers­ pinpoint ulcers. • As the disease progresses, inflammation spreads into the submucosa of the colon. Attempt at healing may produce pseudopolyp. There are areas of epithelial hypertrophy in between the ulcers, resembling polyp. Healing with fibrosis results in a narrow, contracted colon, called pipe stem colon. • Microscopy: Pus (abscess) in the crypts and pus cells (inflammatory cells) in the lamina propria are typical of ulcerative colitis. Clinical features • More common in females. Female:male ratio is 2: 1. • Age: Common age of presentation is 3rd decade followed by 4th and 2nd decades. • The disease is characterised by passage of 15-20 stools per day and contains blood and mucus. Sometimes, it may be watery diarrhoea (Key Box 28.8). As the rectum loses elasticity and lumen collapses, tenesmus occurs. • Relapses and remissions are common and are related to emotional disturbances.

.,.•�,:, PATHOLOGY OF ULCERATIVE COLITIS Pinpoint ulcers Pseudopolyposis Observe 4 Ps Pipe stem colon Pus cells

�

IMft,MI:_.,_� TYPES-DEPENDING UPON SEVERITY

�

• Mild colitis: Refers to< 4 stools/day without systemic signs and symptoms. • Moderate colitis: Refers to< 6 stools/day without systemic toxicity. • Severe colitis: Refers to > 6 stools/day with systemic toxicity-fever over 37.5 ° C, tachycardia more than 90/min, hypoalbuminaemia less than 3 g/dl, weight loss more than 3 kg

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• Severe dehydration, m alnutrition, anaemia, hypo­ proteinaemia are late features. • Acute fulminating attack is associated with high grade fever, bloody dysentery, distension and tenderness all over the abdomen with profound weakness. Hypokalaemia, acidosis, anaemia and shock are the other features. Types of ulcerative colitis (depending upon the extent of the colon involved) 1. Proctitis: In about 20-25% of the patients, the disease involves only rectum. In such patients, stools are semisolid because of absorption of water by normal colon. Also, the intensity of the disease is not severe and risk of cancer is 2-5%. 2. Left-sided colitis: It is found in 15% of patients. It presents as severe recunent attacks of dianhoea with blood in stools, without systemic toxicity (Fig. 28.31A). 3. Total proctocolitis: It is seen in about 25% of the patients. Severe bloody diarrhoea and hypoproteinaemia are its features. Chances of cancer and complications are high in this group (Fig. 28.3 JB). Complications 1. Toxic megacolon: It is an abdominal emergency encoun­ tered with fulminating colitis. Severe abdominal pain and tenderness, toxaemia, high fever, tachycardia and leucocytosis are the features. Plain X-ray abdomen which shows colon with a diameter more than 6 cm gives the diagnosis. It requires emergency treatment by laparotomy and colec­ tomy. Supportive treatment and intravenous corticosteroids are necessary (Key Boxes 28.9 and 28.10, Figs 28.32 and 28.33).

Fig. 28.32: Plain X-ray abdomen showing dilated transverse colon more than 6 cm toxic megacolon

lflft.•*-�L:, FACTORS PRECIPITATING TOXIC MEGACOLON� • Barium enema • Opiates, antidiarrhoeal drugs, anticholinergic agents • Not known

••111111111111111 TOXIC MEGACOLON-CAUSES • Ulcerative colitis, salmonella colitis • Pseudomembranous colitis, amoebic colitis

Fig. 28.31A: Sharp demar­ cation at the splenic flexure may be due to an incomplete marginal artery ending at point of demarcation. Case of left-sided colitis

Fig. 28.31 B: Fully mobilised colon. Observe loss of haustra­ tions. Total proctocolectomy with ileal pouch to anal anastomosis was done-total proctocolitis

Fig. 28.33: Total colectomy for toxic megacolon specimen showing pseudopolyposis

Small Intestine 2. Massive haemorrhage per rectum is uncommon. It is treated by blood transfusion. 3. Perforation is treated as peritonitis with resection of colon. Mortality rate is around 25-50%. Steroids may mask the symptoms. In an emergency situation, saving life is more important. Hence, resection followed by ileostomy should be done. 4. Carcinoma of the colon (Key Box 28. l J) • Overall incidence is 3% when the disease has been present for 15 years. • At the end of 25 years, the incidence may be around 20%. • Hence, routine sigmoidoscopy and biopsy have to be done when the disease is present for more than l O years and if it shows epithelial dysplasia, it should be considered as premalignant. • Incidence is more in total proctocolitis and when the disease has started in the early age group. 5. Recurrent perianal abscess resulting in perianal fistula, in about 15-20% of patients. 6. General complications (extraintestinal) • Protein malnutrition resulting in cirrhosis. Primary sclerosing cholangitis is also found in many cases. Fatty acid infiltration is seen in 40% of cases. It is reversible after control of disease. • Skin ulcerations, pyoderma, erythema nodosum, etc. reflects protein malnutrition. • Conjunctivitis, iritis, arthritis involving large joints are also other features. • Incidence of bile duct cancer is high in these patients.

679

and bleeding are more common with ulcerative colitis than Crohn's disease. • Dysenteries: Bacillary dysenteries, Shigellosis, Salmo­ nellosis, Amoebiasis and other dysenteries have to be kept in mind specially in developing countries including tuberculosis which has been already discussed. • Diverticular disease of the colon: However, when complications including bleeding occur, diverticular disease of the colon and in cases of perforations, malignant perforation must be considered as a differential diagnosis. Investigations 1. Stool • It is done mainly to rule out various causes of infective diarrhoea-amoebiasis, Shigella, Clostridium difficile. • Most common cause of infective colitis in UK is Campylobacterium. 2. Sigmoidoscopy (Key Box 28.12) • Can demonstrate inflammatory changes in the mucosa. • Mucus, pus and blood are visible. • Multiple ulcers are visible with bleeding. 3. Barium enema findings in ulcerative colitis (should not be done in acute cases) (Fig. 28.34) • Contracted colon/pipe stem colon • Absence of haustrations and mucosa! irregularity • Pseudopolyposis appears as stippled appearance

PEARLS OF WISDOM

Peripheral arthritis and ankylosing spondylitis are the two most common extraintestinal manifestations.

Differential diagnosis • Crohn's disease should be ruled out first. The differences between the two inflammatory bowel diseases have been discussed at the end of the chapter. In general, diarrhoea

•.•_, Fig. 28.34: Barium enema showing pipe stem colon

• • • • • •

ULCERATIVE COLITIS (UC) AND COLORECTAL CANCER (CRC) CRC is more aggressive Multicentric and synchronous UC patients with primary sclerosing cholangitis (PSC) have increased risk of CRC More advanced stage at the time of presentation Risk increases with duration of the disease Malignancy develops on a background of dysplasia [dysplasia associated lesion or mass (DAL-M)]

SIGMOIDOSCOPY-FINDINGS

Amoebic ulcer

• Large • Deep, flask shaped • Mucosa in between ulcer is healthy

Ulcerative colitis

• Small, pinpoint • Superficial • Mucosa in between ulcers unhealthy

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Manipal Manual of Surgery

Figs 28.35 and 28.36: Colonoscopy-pinpoint ulcers with bleeding ( Courtesy: Dr Filipe Alvares, Medical Gastroenterologist, KMC, Manipal)

• Salazopyrines are given in the dose of2 g/day. Mode of action: When given orally, it gets split into 5-amino­ salicylic acid and sulphapyridine in the colon. This suppresses activity ofprostaglandins El and E2 and thus reduces inflammation. They are used mainly to induce rem1ss1on. • Corticosteroids: Less severe cases not responding to salazopyrines are given a trial of oral prednisolone 60 mg/day. They decrease the frequency ofstools. The dose is tapered off over 3-4 weeks. • In acute attacks, IV hydrocortisone 100 mg is given. • Prednisolone retention enema: 20 mg in 200 ml saline in intractable diarrhoea. Tt avoids systemic toxicity. Prednisolone 20-40 mg/day can also be given orally for 3-4 weeks. • Role of cyclosporine: Those patients who do not respond to corticosteroids, can be given IV cyclosporin 4 mg/kg/ day. It can induce remission.

4. Colonoscopy (Figs 28.35 and 28.36)

• To confinn the diagnosis by biopsy • To find out the extent ofinvolvement ofcolon • To follow up patients who are on treatment • To rule out carcinomatous changes 5. Plain X-ray abdomen: To rule out megacolon and perforation. 6. C-reactive protein: Its levels are very high in case ofacute fulminating attack or toxic megacolon. 7. Electrolytes, albumin levels are low. They have to be con-ected especially in severe cases. Treatment of ulcerative colitis

I. Conservative line of management (Table 28.2) • Hospitalisation and bedrest • Con-ection of fluid and electrolyte imbalance • Blood transfusions to correct anaemia and TPN for hypoproteinaemia (Fig. 28.3 7).

II. Surgery Indications for surgery

• • • • • •

Complications-toxic megacolon, perforation. Active disease in spite ofmedical line of management Severe disease not responding to medical treatment Dysplasia on biopsy Steroid dependence Haemorrhage

PEARLS OF

Fig. 28.37: Total parenteral nutrition (TPN) before and after surgery for ulcerative colitis plays an important role in the recovery of the patient, as these patients are grossly emaciated and hypo­ proteinaemic

WISDOM

Failure to improve within 7 days of maximum medical treatment with steroids and cyclosporine is an indication for surgery.

681

Small Intestine Pharmacotherapy in ulcerative colitis Drug

Dose

Mode of action

I. Salazopyrines-first choice

• • • • • • •

• Suppresses PGE 1 and E2

2. Steroids-refractory cases

3. Jmrnunomodulators • Cyclosporine used in refractory cases 4. Monoclonal antibody Antitumour necrosis factor-alpha-(TNF a) lnfliximab 5. Anti-inflanunatory (salicylic acid)

2-4 g/day Anti-inflammatory lmmunosuppressive IV hydrocortisone LOO mg 8th hourly Prednisolone enema After acute illness prednisolone 40-60 mg/day Azathioprine 6-mercaptopurines

• Anti-inflammatory

• Act at DNA level and inhibit T-lymphocyte functions • Antitumour necrosis • Directly targeted/released into the colon

• Olsalazine (Dipentum) • 500 mg 1-2 times/day

1. Total proctocolectomy followed by permanent ileostomy (ileoanal anastomosis should not be done because of incontinence). Ileostomy is connected to ileostomy bag. Adhesive obstruction and chronic perinea! sinus are late complications. This is the procedure with least complica­ tions (Figs 28.38 and 28.39). 2. Restorative proctocolectomy with ilea! pouch: • This can be done as one- or two- stage procedure. • Total proctocolectomy is done first. • A mucosectomy of the upper anal canal is done. • A pouch is created by anastomosing the loops of ileum. A J shaped pouch is the most popular followed by W pouch (Figs 28.40 to 28.43). • The pouch is anastomosed to the dentate line Uunction of upper and lower anal canal) by using stapler or by hand sutures. • Protective ileostomy is done and it can be closed after two months.

Fig. 28.40: J pouch

Fig. 28.41: W pouch

Advantages of a pouch 1. Avoids ileostomy

•

Fig. 28.38: Total colectomy

Fig. 28.39: lleostomy

The length of the pouch is generally 15 cm and capacity around 300 ml when distended. Note that it is a two-layered anastomosis to create the pouch. The same can be achieved by linear stapling device. Fig. 28.42: J pouch ( Couttesy: Dr Prasad Babu TlVD Ramcharan Thyagarajan, Srikanth Gadiyaram, Prof Sadiq S Sikora, Department of Surgical Gastroenterology, Manipal Hospital, Bengaluru)

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Treatment of ulcerative colitis Mild

Moderate

Severe

Admission Rectal steroid Prednisolone

Not required Yes Oral20--40 mg

Not required Yes Yes

S ASA Nutrition Fluid/electrolyte balance Blood transfusion

Can be given Oral Normal No

Can be given Oral Non11al Yes

Required No IV hydrocortisone 100-200 mg/4 times/day Not given TPN/oral/high calorie diet Imbalance is very common Yes

Surgery

Not done

Not done

No response for 3-5 days-surgery

SASA: amino salicylic acid TPN: total parenteral nutrition

• To avoid: High fibre high residue diet -thus to control diarrhoea • To avoid: Caffeine-Coffee, dried fruits and nuts, alcohol, meat, spicy food, oily food, soda, etc. ILEOSTOMY Wound was sutured after 48 hours because wound infection is a common complication after this surgery Fig. 28.43: Total proctocolectomy with a pouch and protective

ileostomy was done here

2. Continence is preserved and patient is able to pass the stools via naturalis. 3. A t the same time, all the diseased mucosa has been removed. Thus, the risk of cancer is negligible. • Pouchitis is a complication. • See Table 28.3 also. Prognosis • In general, emergency colitis is required in about 25% patients with severe attacks. Perforation has a mortality rate of up to 40% because of faecal contents and toxicity. Incidence of carcinoma colon is about 10-15%. Longer the duration of the disease, higher is the incidence. Dietary advice • High protein, carbohydrates, whole grains, and good fats. Meat, fish, poultry, and dairy products breads and cereals; fruits and vegetables may be consumed. • For vegetarians: Dairy products and plant proteins- such as soya bean products may be consumed.

Definition • Ileostomy is a surgical procedure wherein a loop of the ileum or end of the ileum is brought to the exterior (surface of the body). • Two types are usually done: End ileostomy and loop ileosotmy. • End ileostomy is done following total proctocolectomy. It is a pennanent ileostomy. The ileum is brought out through the lateral edge of rectus abdominis. It should project at least 5 cm outside (Fig. 28.44). • Loop ileostomy is done to divert gastrointestinal contents to protect ileo-pouch anastomosis. It is a temporary ileostomy which is closed after 6-8 weeks. • Permanent ileostomy is also required following total colectomy for carcinoma colon. It is an end ileostomy (Figs 28.45 to 28.47). • Ileostomy care includes maintenance of fluid and electrolyte balance, use of disposable ileostomy bag and skin protec­ tion. • Complications of an ileostomy are similar to that of colostomy-retraction, prolapse, bleeding, stenosis. • Precautions-living with an ileostomy 1. Ileostomy bag has to be fitted well to the body surface. It needs to be adjusted often 2. Ileostomy has to emptied 4-6 times depending upon the requirement 3. Chewed and masticated food is better 4. Avoid gas forming diet. Patients will learn slowly what to take and what not to take 5. Long-tenn complications include-gall stones, kidney stones, adhesions and intestinal obstruction.

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Small Intestine

CROHN'S DISEASE

•

Fig. 28.44A: lleostomy: It

should project out for at least 5cm

Fig. 28.448: End ileostomy: following total protoco­ lectomy

Fig. 28.45: Doubtful viability-complication of the ileostomy

Fig. 28.46: 18-year-old girl was diagnosed with acute appendicitis at exploration, tuberculosis of the ileum made and limited ileo­ colectomy was done fol­ lowing which ileum was brought outside as tempo­ rary ileostomy

It was called regional ileitis because the disease was first reported in the terminal ileum. However, today it is called regional enteritis because the disease can occur in jejunum, ileum, colon, oesophagus, etc. Involvement of ileum is more common, followed by colon. Definition • Crohn's disease is a chronic transmural inflammatory disease of the gastrointestinal tract of unknown aetiology. Aetiology 1. Infectious agents: Mycobacterium paratuberculosis and measles virus have been proposed as potential causes of Crohn's disease. However, it should be noted that antimicrobial therapy has not been effective in eradicating Crohn's (unlike ATT in TB). Also, no immunological reaction has been found. 2. Immunologic factors: Similar to UC. Focal ischaemia due to autoimmune reaction has also been considered. 3. Genetic factors: Single strongest risk factor for development ofCrohn's is a relative with Crohn's disease. • Relatively high incidence is found in Ashkenazi jaws. 4. Smoking: It increases the risk of Crohn's disease three­ fold unlike its protective effect against ulcerative colitis. Pathology (Key Box 28.13) • Disease starts in terminal ileum as ulcerations of intestine in about 60% of cases. • There is extensive inflammatory oedema and mucosa! ulcers are present. Fibrotic thickening of the intestine results in hose pipe rigidity of the intestine. • There are skip areas which are characteristic of Crohn's disease (segments of intestine are normal in between). • Mesenteric nodes are enlarged. They can be calcified but do not show any caseation.

___ PATHOLOGY OF CROHN'S DISEASE

Fig. 28.47: Loop ileostomy is done following resection of perfo­ rated enteric ulcer. Distal ileum was friable and oedematous. Hence it is brought out. This is a safer option

Transmural inflammation Reticulation cobblestone Aphthoid ulcers Noncaseating cicatrising granuloma Skip lesions Mesenteric lymph nodes enlarged Ulcers are in ileum, jejunum, colon, etc. Rigidity-Hose pipe Adhesions occur soon Linear ulcers Remember as TRANSMURAL

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Ileum

Fig. 28.48: Crohn's disease-creeping fat ( Courtesy: Dr Ramesh Rajan, Gastrointestinal Surgeon, Trivandrum Medical College, Kerala)

• Intense infiltration of mononuclear cells and lymphoid hyperplasia is common. • As the disease progresses, there is cicatrising granuloma of the bowel wall. This results in narrowing of lumen causing intestinal obstruction. Caseation is char­ acteristically absent. • Once inflammation spreads to the serosa, adhesions develop between bowel loops or other structures. Abscesses occur in the mesentery which rupture resulting in internal fistula (Fig. 28.48). PEARLS OF

WISDOM

Transmural inflammation is characteristic of Crohn 's disease. Clinical features (Key Box 28.14 and Figs 28.49 to 28.51) The disease is often insidious, slowly progressive with a protracted course and commonly affects young adults in the second or third decade of life. Intermittent colicky lower abdominal pain, diarrhoea and weight loss are common. Depending upon symptoms, it can be classified as follows: (Table 28.4) 1. Stage of ileocolitis: Clinically, it presents as pain abdomen and mucus in stools. It is seen in younger patients. It may be associated with fever. Presence of pain and tenderness in the right iliac fossa mimics appendicitis. If there is a mass, it may be confused for an appendicular mass. 2. Stage of subacute intestinal obstruction occurs due to stricture of the terminal ileum. Strictures can be multiple. They are not reversible.

Perianal abscess

Fig. 28.49: Clinical features of Crohn's disease ( Courtesy: Ms Vidushi, MBBS student, KMC, Manipal)

VARIOUS STAGES/COMPLICATIONS OF CROHN'S DISEASE Colitis-ileocolitis Rectal-anorectal disease Obstruction due to stricture Hollow viscus fistulae Nutritional deficiencies Remember as CROHN

Fig. 28.50: An emaciated patient who underwent appendicectomy, only to present later with features of Crohn's disease

Fig. 28.51: Congested bowel loops, area of perforation, dilated loops of bowel are seen in this picture

Crohn's disease Colitis

Obstruction

Mass

Nutrition

Anorectal disease

Diarrhoea

Cramps, distension

Right iliac fossa

Emaciation

Fissure, abscess

Water Weight loss

Vomiting

Gurgling, tender, obstruction

Dehydration

Fistula in ano

j, j,

j,

j,

j, j,

j,

j,

j,

j,

Small Intestine

3. Stage of fistula formation: It can be enteroenteric or enterocutaneous. Following are the examples of fistulae encountered in Crohn's disease-ileovesical, ileocolic, ileoileal, ileovaginal, etc. 4. Perianal disease in the form of multiple ulcers in the anorectal region, perianal abscesses, multiple fistulae in ano are much more common than in ulcerative colitis. Repeated infection of anal crypt due to diarrhoea is common. • Extraintestinal complications are similar to ulcerative colitis. PEARLS OF

WISDOM

Analfissure is the most common anal problem in Crohn 's disease. Noncaseating granuloma is most common in anorectal disease. Differential diagnosis 1. Ulcerative colitis: However, colonic symptoms are more with ulcerative colitis. 2. Tuberculosis: In India, tuberculosis should be considered first and ruled out. 3. Appendicitis: Acute pain in the right iliac fossa can be confused for acute appendicitis. For benefit of the doubt, the patient can undergo laparotomy. Laparoscopy and appendicectomy will be better 4. Intestinal obstruction: Other causes of obstruction have to be kept in mind. Investigations 1. Small bowel enema: Enteroclysis. • Cobblestone reticulation because ofmultiple ulcers with islands of normal mucosa in between. • Absence of peristalsis in terminal ileum. • String sign of Kantor is demonstrated in terminal ileum due to narrowing of the lumen. • Multiple strictures and dilated segments in between can be demonstrated. 2. Sigmoidoscopy and colonoscopy may demonstrate inflamed mucosa, which is granular with aphthoid ulcers, which are discrete (Fig. 28.52). 3. Fistulography to localise the internal fistula. 4. CT scan: It is done to detect thickening of bowel and extraintestinal disease. 5. Remember to investigate upper GI tract with gastroduodenoscopy and capsule endoscopy (see page 817). Treatment I. General principles • Complete rest, avoid stress and emotions • A low residue, high caloric diet such as high protein diet • Some patients may require total parenteral nutrition-some at home called home parenteral nutrition

685

11. Conservative (med ical) treatment is similar to ulcerative colitis. • Steroids are the mainstay of treatment. They are effective in inducing remission in 70 to 80% of cases. • Steroids are most effective in treating small intestinal disease. They are anti-inflammatory. They do control diarrhoea, induce remissions. Prednisolone is used for short term treatment. Long-term use can give rise to toxicity such as immunosuppression, bone loss, delayed wound healing, etc. • Salazopyrines can be used especially in maintenance cases also. • Even though salazopyrines and corticosteroids have been beneficial in Crohn's disease, salazopyrines do not induce remissions. They are used in acute ileocolitis. Steroids can be used for anorectal disease. • Immunosuppressive therapy using azathioprine and 6-mercaptopurine are also effective. • They are effective in treatment of colonic disease. The main concern is bone marrow toxicity. 6-mercapto­ purines are known to produce pancreatitis. • Most recent and promising drug is infliximab-a mono­ clonal antibody to tumour necrosis factor (TNFcx). It mainly helps in closure of fistula. It is given intravenously and is used for intestinal and perianal disease. • Metronidazole has shown some benefit. UL Surgical treatment: Resection is not the aim of surgery but it may have to be done in cases of obstruction, perfora­ tion, intra-abdominal abscesses, internal fistulae, bleeding and malignancies. Depending upon the involvement of the bowel, various resections are possible. Examples: 1. Stricture-stricturoplasty or resection 2. Ileocaecal resection (Figs 28.53 and 28.54) 3. Colectomy and ileorectal anastomosis 4. If the fistulae are present, they are disconnected from the bowel and excised.

Fig. 28.52: Colonoscopic view of Crohn's disease

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Fig. 28.53: Resected specimen of small intestine. The patient had intestinal obstruction

Fig. 28.54: Opened specimen showing stricture. Biopsy proved Crohn's disease

Comparison of intestinal tuberculosis, ulcerative colitis and Crohn's disease is given in Table 28.5.

• Both small bowel and large bowel in 50-60% patients • Duodenum, stomach, oesophagus can also be involved. • Inflammatory cells and mediators of the inflammation such as cytokines, interleukins, tumour necrosis factors produce the inflammatory changes resulting in granuloma • Sometimes, very difficult to differentiate clinically and pathologically between tuberculosis and Crohn's disease. • Anus is involved in Crohn 's colitis, not in ulcerative colitis.

Prognosis • In spite of various treatment, there is no cure for the disease. About 10-20% patients come with relapses and recurrent symptoms • But for repeated treatment including surgical procedures, survival is still good as compared to general population without the disease. A few observations in crohn's disease • Ileum is the most common site • Small bowel alone is affected in 20-30% of the patients

SURGICAL COMPLICATIONS OF ENTERIC FEVER During the third week of enteric fever, Salmonella typhi, paratyphi (enteric bacilli) multiply in Peyer's patches and can give rise to following problems:

Summary of tubercular ileocolitis, ulcerative colitis and Crohn's colitis

Tuberculosis

Crohn's

Ulcerative colitis

1. Bacteria

Mycobacterium

No

No

2. Pathology

Continuous spread. Caseating granuloma, lymph node caseation. Transmural inflammation present

Skip areas. Cobblestone granuloma present. No caseation. Deep longitudinal ulcers. Transmural inflammation

Continuous, pseudopolyposis. Pinpoint ulcers. Crypt abscesses. Superficial ulcers. Mucosa and submucosa involved

3. Site

lleocaecal

Terminal ileum and colon

Rectum and colon

4. Bowel wall

Thick and fibrotic

Thick fibrotic

Thin

5. Clinical Bleeding Diarrhoea Fever Mass Anal disease Toxic megacolon Fistulae Stricture 6. Malignancy

Uncommon Uncommon Common Very common Rare Never Uncommon Common No

Uncommon Very common Common Common Very common Rare Very common Common Can predispose (low)

Very common Very common Rare Rare Rare More common Uncommon Rare High incidence

Small Intestine

1. Haemorrhage is seen in about 5-10% of cases due to ulceration of Peyer's patches. It can be occult, obvious or rarely massive bleeding. It is managed conservatively in majority of cases. 2. Perforation of terminal ileum: An oval, vertical perforation results in peritonitis. Enteric perforation need not give rise to all signs of peritonitis. Guarding and rigidity can be minimal because of poor, immunocompromised nature of the diseases and due to Zenker's degeneration of abdominal wall muscles. It is a single perforation in about 85% of the cases. It is situated in the antimesenteric border of the terminal ileum. Typically, it occurs in the third week of enteric fever. Bradycardia, dehydration, toxicity are the other features. • Hyperplasia of reticuloendothelial system including lymph nodes, liver and spleen is characteristic of typhoid fever (Key Box 28.15). • Diagnosis of perforation is based clinically on the acute abdominal pain, bleeding per rectum with/without guarding and rigidity. High grade fever, toxicity and bradycardia are other features that help in the diagnosis. • Plain X-ray abdomen may not reveal gas because of small sealed off perforation. • The most useful investigation is CT scan which can reveal not only pneumoperitoneum but also pericolic collection, which can be missed by ultrasound (see the clinical notes below).

687

in the right iliac fossa. Wound infection is common in such cases. Multiple fistulae are also common (Figs 28.55 to 28.58) • Small bowel exteriorisation: This can be considered in cases after resection when both ends of the intestine are friable. This is a very safe option. After 1-4 weeks, relaparo­ tomy and anastomosis of resected ends is done. 3. Paralytic ileus due to toxic dilatation of intestine results in distension of abdomen. It is managed by drip and suction. 4. Typhoid cholecystitis is not uncommon. Its starts within 2 to 4 days fever. Chances of gallbladder perforation are present. 5. Typhoid pyelonephritis, cystitis, epididymo-orchitis. 6. Typhoid osteomyelitis 7. Typhoid conjunctivitis 8. Thrombosis of the common iliac vein occurs probably due to sluggish blood flow. Enteric Ulcer Perforation

PEARLS OF WISDOM • When you suspect peritonits, no guarding and no rigidity • When you suspect perforation, no gas under diaphragm • When you suspect toxicity, no tachycardia but brady­ cardia, suspect enteric ulcer

Fig. 28.55: Single ulcer,

simple suturing

Fig. 28.56: Multiple ulcer­ resection and anastomosis

Treatment • Third generation cephalosporins are used • Emergency laparotomy, resection of bowel and end-to-end anastomosis or closure of the perforation by using non­ absorbable sutures. Abdomen is closed with a tube drain kept

A patient with partially treated enteric fever presented on the I 0th day with acute abdominal pain and fever. Lower abdominal guarding and rigidity were present. Plain X-ray abdomen did not reveal gas under the diaphragm. Ultrasonography did not reveal pericolic collection. A CT scan of the abdomen was done. It revealed pneumo­ peritoneum and pericolic collection. Immediate /aparotomy and closure of the perforation was done and peritoneal cavity was drained. The patient recovered dramatically. When in doubt, request a CT scan.

I

)

Fig. 28.57: Both ends brought outside - small bowel exteriorisation (Courtesy: Dr Vidushi, MBBS student, KMC, Manipal)

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8 8 Fig. 28.58: Multiple bud fistulae developed following resection and anastomosis of typhoid ulcers treated with ostomy bags

9. Perforation of large intestine can occur in paratyphoid 'B'

infections. • Most of these complications occur due to bacteraemia produced in the early septicaemic phase of enteric fever. Liver, spleen, bone and bowel are commonly affected. Metastatic abscesses are common.

TYPHOID FEVER-SALIENT FEATURES • Toxic-Dehydrated delirious patient with diarrhoea­ Green pea soup stools • Young patient • Poor tone of the abdominal muscles due to Zenker's degeneration • High grade fever • Other-hepatosplenomegaly, Rose spots • lmmunocompromised status • Decreased pulse rate-Faget sign You can remember as TYPHOID

8 8

Fig. 28.59: Quadrinucleate cyst of entamoeba

• Lower sigmoid and upper rectum are involved in 75% of cases. Clinical features J. Amoebic typhlitis: Inflammation of the caecum by amoeba is described as amoebic typhlitis. It produces pain in the right iliac fossa and it can be confused for appendicitis. However, in this condition there is also tenderness in the left iliac fossa. A point on the left side corresponding to McBurney point on the right side is called Sir Philip Manson-Bahr's amoebic point of tenderness (Fig. 28.60) and is suggestive of involvement of rectosigmoid. 2. Amoebic dysentery: It can be acute or chronic. An acute

attack is associated with gripping pain abdomen with blood and mucus in stool, an urgency to pass stools. High grade fever and tenesmus are the other features. Chronic dysentery is more common with 2--4 foul-smelling stools per day and mild to moderate colicky abdominal pain.

3. Amoeboma: Chronic, low grade, persistent inflammation

of the caecum produces granulomatous hyperplasia of the caecum, with thickening of the pericaecal tissue (producing mass in the right iliac fossa) (Fig. 28.6 l ). Amoeboma can

INTESTINAL AMOEBIASIS • This disease is caused by Entamoeba histolytica and transmitted mainly by contaminated drinking water. • After the cysts are swallowed, they are broken down in the intestine by trypsin into trophozoites which produce inflammation of the colon. Trophozoites swallow red blood cells and multiply by mitosis. They enter into crypts of Lieberkuhn. They produce multiple submucous loculi which later result in multiple ulcers. These ulcers are flask-shaped (bottle neck) ulcers with healthy intervening mucosa. • Some trophozoites are transformed into cysts and excreted outside (Fig. 28.59).

•

Fig. 28.60: Sir Philip Manson- Fig. 28.61: Amoeboma is a Bahr's amoebic point tender thickened gurgling mass

Small Intestine

•

• 4. 5.

also occur in rectosigmoid junction. This is, however, uncommon nowadays because of effective treatment with metronidazole. Clinically, this manifests as mass in the right iliac fossa causing dull aching pain, vague ill health, tender palpable caecum with guarding. It can be confused for ileocaecal tuberculosis or carcinoma caecum. It responds very well to metronidazole. Amoebic perforation of caecum or sigmoid can occur resulting in pericolic abscess. Peritonitis needs emergency surgery. Massive bleeding per rectum is rare. It occurs due to separation of the slough.

Treatment 1. Metronidazole 400-600 mg, 3 times a day for IO days. It acts on amoebae present in the lumen and tissue. 2. Diiodohydroxyquin 650 mg, 3 times a day for 20 days is another alternative. 3. Diloxanide furoate is ideal for chronic cases in the dose of 500 mg, 3 times a day for 10 days. It acts on luminal amoebae. It is the drug of choice in chronic cyst passers. • Supportive treatment in the form of hospitalisation, correction of dehydration, antispasmodics and bedrest is also advised. Stool culture must be done before and after treatment with antiamoebic drugs.

689

• If there is obstruction, laparotomy with resection or bypass or any other treatment depending upon the findings. • Surgery can be difficult because of dense adhesions. • Difficult to prevent these changes. However, an absorbable mesh can be placed over the small intestines after pelvic surgery when radiation is contemplated. SMALLBOWEL TUMOURS • Benign tumours such as lipoma, hamartoma, polyps can occur. • Malignant tumours such as adenocarcinoma, gastro­ intestinal stromal tumours (GIST), carcinoid and lymphoma can occur in the small intestine. PEUTZ-JEGHER SYNDROME (FAMIL I AL H AMARTOMATOUS POLYPOS IS) This syndrome is characterised by: 1. Familial tendency

RADIATION ENTEROPATHY • These are the changes which occur in the intestines after they are exposed to radiation. • Typically happen when radiation is given to the pelvis after rectal resections or radiation given to treat carcinoma cervix • Incidence is 5% after 4500 cGy units to 30% after 6000 cGy units. • Chief factor is radiation induced damage to the blood vessel supplying the vessel wall. • Chronic intestinal ischaemia occurs resulting in stricture and intestinal obstruction. Some cases are due to fibrosis, dense pelvic adhesions, bowel getting entrapped into the fibrosis, fistula formation and pelvic abscess. • When there is no obstruction, conservative line of treatment to be followed.

Fig. 28.62: This patient presented to hospital with abdominal pain. The Registrar who examined the case reported that there was a mass abdomen. However, when he presented the case next day, there was no mass. The patient had undergone laparotomy 10 years back for similar complaints. A segment of bowel had been resected earlier. This photograph gives the clue to the diagnosis­ pigmentation of lips and oral mucosa. The mass (appearing and disappearing) was due to intussusception ( Courtesy: Prof Sreevatsa, HOD, Prof Bharathi, Prof Bagli, Dr Srikar Pai, Department of Surgery, MS Ramaiah Medical College, Bengaluru, CME 2008)

Comparison of two hamartomatous polyposis syndromes Peutz-Jegher syndrome

Cronkhite Canada syndrome

• • • • •

• • • • •

Hamartomatous polyps Polyposis of small intestine (mainly) Melanin spots on the buccal mucosa and lips Bleeding and intussusception are common complaints Rarely malignancy can occur

Hamartomatous polyps Gastrointestinal polyposis Cutaneous pigmentation, finger nails atrophy, alopecia Intestinal obstruction can occur Severe malabsorption-protein-losing enteropathy

690

-·--

Manipal Manual of Surgery

We had 3 interesting cases ofPeutz-Jegher syndrome. The first case was of a boy of 14 years, who presented with acute intestinal obstruction. At laparotomy, there were 3 intussusceptions in the jejunum due to polyps. About 15 large polyps were removed after doing an enterotomy. The second case was a 50-year-old lady, who presented with duodenal carcinoma. Endoscopy revealed polyps in the stomach and duodenum. Specimen of pancreatico­ duodenectomy revealed it as a case of Peutz-Jegher syndrome. This lady did not have pigmentation ofthe oral mucosa. The third case was of a 35-year-old male who has been coming to our hospital with intermittent bleeding and anaemia. Endoscopy revealed multiple polyps in the stomach and duodenum. Small bowel enema demonstrated multiple polyps in the small bowel. Even proctoscopy showed multiple polyps in the rectum. He is being managed conservatively.

• • • •

MALIGNANCY IN SMALL INTESTINE IS RARE-WHY?

No stasis, rapid transit of food Secretion of immunoglobulins-lgA Various carcinogens are diluted by secretions Comparatively low and inactive bacteria in the small bowel

2. Melanosis of mucosa of lip, cheek, interdigital space and even perianal skin (Fig. 28.62 and Table 28.6). 3. Multiple polyps in the small bowel and large bowel mainly in the jejunum. They are hamartomatous polyps. • It is an autosomal dominant disease. Clinical presentation • Runs in families. • As a cause of bleeding per rectum, results in chronic anaemia. • Can cause adult intussusception. • Rarely, it can turn into malignancy. • Female patients have increased chances of breast and cervical cancer (see clinical notes)

Fig. 28.63: CECT scan showing jejuna! growth with narrowing of the lumen of the jejunum

Treatment • Blood transfusion to correct anaemia • Resection of that portion of bowel containing polyp, in cases of bleeding or intussusception. ADENOCARCINOMA • Incidence is 40% of small bowel tumours. Overall, small bowel tumours are rare. Reasons have been given in Key Box 28.16 • Duodenum is the commonest site of adenocarcinoma. If it arises from first part and second part, it may require Whipple's pancreaticoduodenectomy. From the third part, early cases of adenocarcinoma can be resected without removal of pancreas. • Some familial diseases predispose to adenocarcinoma.They are familial polyposis coli,Adenomas, Crohn's disease, etc.

Figs 28.64A and B: Push enteroscopy showing ulcerative lesion in the proximal jejunum

• Clinical features include vague features like nausea, poor appetite, crampy abdominal pain, bleeding and intestinal obstruction. • Diagnosis is by CT scan (Fig. 28.63). • Push enteroscopy has the advantage of visualisation of the growth and to take biopsy (Figs 28.64A and B). • Other option is capsule endoscopy. It is time consuming and biopsy is not possible

Small Intestine

691

• Resection with at least 7 cm margin with lymphadenectomy is the treatment of choice (Figs 28.65 to 28.67). • Hardly any role for radiotherapy and chemotherapy GIST: GASTROINTESTINAL STROMAL TUMOUR

Fig. 28.65: Ulcerative lesion at surgery

• Earlier called as leiomyoma and leiomyosarcoma, they occur in jejunum or ileum. These are uncommon mesenchymal tumours (Figs 28.68 to 28.70). • Stomach is the commonest site. GISTs are rare in the oesophagus whereas leiomyoma is more common. • They arise from interstitial cells of Cajal which are pacemaker cells that regulate motility and peristalsis. • They present as bleeding/mass/perforation. • Very often massive bleeding may be the only presentation • Carney's triad 1. GIST 2. Pulmonary chondromas 3. Extra-adrenal paragangliomas.

Fig. 28.66: Resection of the growth done with 6 cm margin with lymph nodes Fig. 28.68: GIST involving terminal ileum, caecum and ascending colon (Courtesy: Dr Stanley Mathew, FRCS, Professor, KMC, Manipal)

Fig. 28.67: This patient presented with abdominal pain, melaena and loss of weight. CT scan of the abdomen revealed mass in the jejunum. It was resected (inset). Mucosa! surface showed ulceration. Histopathology report was adenocarcinoma

Fig. 28.69: GIST involving jejunum-resected specimen showing mucosa! ulceration (Courtesy: Dr Padmanabha Bhat, Dept. of Surgery, KMC, Manipal and Prof HD Shenoy, Father Muller's Hospital, Mangalore)

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Sites (Table 28.7) • Appendix: 65%. It is the most common site. It occur more commonly in females. When the tumour occurs i the appendix, it is usually benign, hard and occurs in dist� one-third of appendix (Key Box 28.17). • Terminal ileum: 30%. Most of them are malignant. When the tumour occurs in the ileum it is usually malig nant and produces multiple bulky secondaries in the live1 even when primary is very small. Fibrosis of the mesenter results in kinking of bowel causing periodic abdominal pair

.,.•-� • • • • •

Fig. 28.70: GIST from small intestine ( Courtesy: Dr Ramesh Rajan, MS, MCh, Surgical Gastroenterologist, Trivandrum, Kerala)

'MOST COMMON' FOR CARCINOID APPENDIX

Most common neoplasm of the appendix Mostly found in distal one-third of the appendix Mostly tumour is yellow staining for immunohistochemistry Mostly benign-very rarely metastasise Mostly appendicectomy is sufficient. Rarely, right hemico­ lectomy may be required.

• Diagnosis is by endoscopy and CT scan • They express CD 117/CD34 antigen. CD117 is known as C-kit receptor. • Treatment is in the form of resection. • Diagnosis of malignancy is by mitotic figures:< 10 mitoses/ high power field (HPF) suggests a low grade malignancy. • > 10 mitoses/HPF suggests high grade malignancy. • Increased incidence of C-kit has also been found in patients with neurofibroma.

Secondaries in the liver

CARCINOID TUMOUR (ARGENTAFFINOMA) AND CARCINOID SYNDROME It arises from argentaffin/chromaffin cells which are present in the crypts of the villi of intestine. These cells are called as Kulchitsky cells. These cells stain with ammonical silver salt solution to black colour. Hence, they are called as argentaffinoma or chromaffinoma. They secrete 5-HT (sero­ tonin or 5-hydroxytryptamine). They can be single or multiple and can be associated with adenocarcinoma.

Fig. 28.71: Carcinoid syndrome

Comparison of carcinoid tumours at various sites Foregut

Midgut

Hindgut

Site

Duodenum, stomach, bronchus, pancreas, etc.

Jejunum, ileum, right colon

Rectum

Incidence

Rare

Common

Uncommon

Hormones

No serotonin

5-HT, prostaglandins, insulin, ACTH

Do not secrete

Bronchial carcinoid produces bronchial obstruction and produces carcinoid syndrome without secondaries in live,'.

.,.•,-,.:,

Small Intestine

• The hormones produced by the tumour 5-HT (serotonin) are not metabolised because of the secondaries. So, they are absorbed into the circulation and produce various symptoms. This is called as carcinoid syndrome (F ig. 28.71). • Carcinoid syndrome consists of following features: 1. Carcinoid tumour which is malignant 2. Secondaries in the liver 3. Symptoms are red-blue cyanosis of skin, flushing attack and asthmatic attacks, intestinal hyperperistalsis causing dimThoea, pulmonary and tricuspid stenosis with CCF. Diagnosis • 24-hour urine for 5-hydroxyindole acetic acid. Normal levels 2-9 mg/24 hours. Very high values are found in this condition. • Ulh·asound and CT scan are other investigations which will help in detecting mass lesion or liver metastasis. Treatment 1. Resection of the tumour with a wide margin along with lymph nodes. Otherwise debulking should be done. 2. Bromocriptine 2.5 mg twice a day can be given to reduce the symptoms. Other agents which can be used are methysergide and diphenoxylate hydrochloride. 3. Secondaries in the liver are treated by intra-arterial (hepatic artery) streptozocin. Localised liver metastasis can be treated with resection. 4. Therapeutic embolisation of hepatic artery by using gel foam, etc. will reduce the size of the liver thereby decreasing discomfort to the patient. 5. Injection octreotide 100 µg IV is the drug of choice in cases of carcinoid crisis (severe bronchospasm). Octreotide in carcinoid syndrome 1. It is used to suppress the tumour growth 2. Control symptoms - flushing, wheezing, diatThoea 3. It controls the release of GI hormones 4. Dose is: 100 micrograms subcutaneously tds in patients with mild/moderate, non-life-threatening carcinoid syndrome (Key Box 28.18). MALIGNANT LYMPHOMA Primary: Arising from lymphoid tissue Secondary: Part of systemic lymphoma. Ileum is the most common site of lymphoma (Key Box 28.19). Precipitating factors 1. Crohn's disease 2. Coeliac disease 3. Immunosuppression-AIDS-Usually it 1s a non­ Hodgkin's lymphoma of 'B' cell origin.

• • • •

• • • • • • •

693

CARCINOID TUMOURS � They are APUDOMAS. Arise from enterochromatfin cells. They may be associated with multiple endocrine neoplasia (MEN ) type 1 and 2 About 15% of carcinoid patients may have carcinoma elsewhere, example: carcinoma stomach, carcinoma lung or carcinoma colon. Common age is 25-45 years of age Majority arise from midgut-almost 85% cases. They secrete 5-HT Appendix is the most common site. Often appendicectomy is enough for malignant carcinoid tumour. Foregut carcinoids do not secrete serotonin Flushing and diarrhoea can be controlled by octreotide. Secondaries can be multiple and big, but slow growing like primary. Arterial embolisation has been tried in many cases. Since they are slow growing and prognosis is good­ aggressive treatment in the form of surgery, octreotide, liver resections and rarely liver transplantation has been done

___ MALIGNANT LYMPHOMA

1. Western type

• Non-Hodgkin's, B-cell type • Annular ulcerating lesion • Bleeding, obstruction, perforation and weight loss 2. Primary lymphoma with coeliac disease • Increased incidence of lymphoma • It is a T-cell lympoma • Diarrhoea, pyrexia are other features 3. Mediterranean-associated with alpha chain disease.1 1Alpha chain disease is a disorder characterised by the secretion of a defective a-heavy chain. Patients present with steatorrhoea, often progressive and fatal.

•._, SHORT GUT-CAUSES

• Mesenteric infarction • Midgut volvulus • Necrotising enteritis • Crohn's disease1 • Radiation enteritis

1 It is common cause in western countries

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SHORT GUT SYNDROME

resulting in mucosa! thinning, ulceration followed b: oedema and later, fibrosis are characteristics of thi: condition. Endarteritis and vasculitis also add to thes1 changes resulting in stricture, perforations, abscess, mal absorption and multiple resection, etc.

Causes of short gut syndrome (Key Box 28.20)

• Short gut syndrome occurs due to massive resection of the bowel resulting in loss of length of the bowel, loss of absorptive area of the bowel and loss of valves. Superior mesenteric artery being an end artery, thrombosis at its origin is invariably fatal. • Midgut volvulus of neonates is congenital due to arrested rotation resulting in floating caecum and mobile intestine. We had an interesting case of midgut volvulus in an 18-year-old boy consequent to a laparotomy done for perforated duodenal ulcer. While replacing the coils of bowel within the abdomen, the mesentery was probably twisted resulting in massive gangrene. This boy now has about 100 cm of the small bowel and surviving on "baby food" for the last 5 years. • Necr o tising enteritis (enteritis necroticans) is a complication of infection of small bowel by Clostridium perfringens. It usually occurs after a heavy feast where pork is consumed. There is extensive suppuration of mucosa! and submucosal layer of jejunum (also ileum). Serosa may show multiple dark bluish patches. Massive resection is done for a necrotic, perforated, unhealthy bowel which results in short gut syndrome. • Radiation enteritis or radiation enteropathy results in patients who receive radiotherapy to the abdominal and pelvic regions, e.g. carcinoma cervix. Arrest of cell division

Pathophysiological effects

(Fig. 28.72 and Key Box 28.21)

It depends upon: • Extent of resection • Site of resection • Presence/absence of ileocaecal valve • Age of the patients • Infants tolerate extensive resections better than adults. Patients with less than l 00 cm of the small bowel wi11 develop severe nutritional deficiencies and may require parenteral nutrition.

••.........,�, • • • • •

EFFECTS OF SHORT GUT Severe malabsorption Gall stones Fatty infiltration of liver Urinary stones Gastric hyperacidity

Massive resection

i Transit time

lleal resection

.J. Absorptive surface

Enterohepatic circulation .J.

Malabsorption of fat

.J. Absorption of bile salts

Malabsorption of calcium and magnesium

Bile salts enter colon

1------ Colonic bacteria Precipitate as soaps + unabsorbed fatty acids

Secondary bile salts

.J. Absorption of water and electrolytes

Diarrhoea

Fig. 28.72: Nutritional deficiency in short gut syndrome

Small Intestine

1. Malabsorption of fat and fat soluble vitamins: Can occur after ilea! resections due to interruption of enterohepatic circulation of bile salts. These bile salts enter the colon and are converted into secondary bile salts. These bile salts block absorption of water and electrolytes. 2. Gastric hypersecretion: Due to delayed clearance of gastrin, as in proximal jejuna! resections, there is increased gastric secretion of acid resulting in hyperacidity. 3. Liver disease: Fatty infiltration of the liver and mild hyperbilirubinaemia is a feature in massive resections and jejunoileal bypass. Acute fulminant hepatic failure can also occur. 4. Gall stone formation: There is increased incidence of cholesterol stones as a result of reduced bile salt pool, after ilea! resection and jejunoileal bypass. 5. Urinary stones • All types of urinary stones are common due to low levels of calcium excretion in the urine and high levels of oxalate. • Water and salt depletion, and loss of K + causes hyponatraemia and hypokalaemia. Adaptation (Key Box 28.22) • As a result of loss of significant bowel, dilatation of the remaining intestine and villous enlargement takes place. This is brought about by a humoral agent, enteroglucagon. In children, length of the bowel is increased. Also, the number of cells in the villi is increased (work hypertrophy). There is also evidence to suggest gradual slowing of the transit time.

•.•_, ADAPTATION TO SHORT GUT SYNDROME

• • • •

Villus-size Length of bowel Transit time Absorption from colon

Treatment • Treatment of short gut patients is difficult. It needs a special set up of dieticians who plan "proper food" for these patients in consultation with treating surgeons. It is a gradual process of feeding the patient beginning with parenteral nutrition and progressing to normal, low fat diet after a few months. • In the initial 2-3 months following massive resection, total parenteral nutrition including supplementation of fluid and

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electrolytes is the ideal treatment. Sips of plain water or oral hypotonic solutions can be allowed. • After 2-3 months, when adaptation of bowel takes place, enteral feeding is started gradually with baby food, fat-free, fibre-free, protein rich, liquid diet. Essential fatty acids should be supplied. Diarrhoea is a common problem and is treated with loperamide tablets. • Enteral feeding can contain low fatty diet in addition to the other nutrients mentioned above. • They occur in the mesenteric border unlike Meckel's which occur in the antemesenteric border. INTESTINAL FISTULAE Introduction • Intestinal fistulae are abnormal communications between two portions of the intestine, between the intestine and some other hollow viscus, or between the intestine and the skin of the abdominal wall. • When it involves skin and intestines, it is called enterocutaneous fistula. • Despite significant advances in their management, intestinal fistulae remain a major clinical problem, with an overall mo1iality rate of 15 to 25%. Classification Anatomical • Internal: Colovesical fistula • External: Duodenal, jejuna! fistula • Mixed: Crohn 's disease Depending on the contents • Low output < 200 ml • Moderate output 200-500 ml • High output > 500 ml Aetiology • Iatrogenic (70%)-postoperative: Injury to intestines unnoticed at the time of surgery or injury recognised at the time of surgery, sutured but has given way are prime causes of enterocutaneous fistula. Anastomotic leak, partial or complete, is another important cause of entero­ cutaneous fistula. Meticulous surgical techniques, gentle handling of the bowel, usage of the proper suture material (Vicryl and silk), ensuring adequate blood supply to the intestines which have to be anastomosed will largely prevent anastomotic leak (Fig. 28.73). • Stump blow out: Duodenal blow out: Typically happens 4-5 days following surgery (see page 506).

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• Distal obstruction • Multiple fistulae that do not heal as in Crohn's or typhoic perforations. • Lateral duodenal fistula does not heal because large volume of contents are poured into the second part o1 duodenum and there is no rest to the part. • When the defect is more than 1 cm, and the track is morE than 2 cm, fistula does not close (see clinical notes). Management: Principles

Fig. 28.73: Small intestinal fistulae due to anastomotic disruption and wound dehiscence

• Inadequate resection of the diseased segment: In such situations, anastomosis may be in unhealthy bowel. • Instrumentation: Oesophageal perforations are dangerous ones and can occur even with flexible scopies. • Spontaneous (30% ): Crohn's disease, diverticular disease of the colon, appendicitis are also known to give rise to fistulae. Local factors precluding spontaneous closure • Bleeding • End fistula: Bowel discontinuity • Foreign body: Swabs, tips of the suction tubes (detached), retained sponges or instruments. • Radiation • Inflammation/infection/inflammatory bowel disease • Epithelialisation of the tract is another important cause of the persistent fistula. • Neoplasms

A 60-year-old man was operated for appendicitis. On the fifth postoperative day, he developed faecal dischargefrom the wound. Conservative management was done for 7 days with total parenteral nutrition and other measures. Fistula persisted. CT scan was done on the 14th day. It revealed a growth in the hepatic flexure almost encir cling the lumen. Colonoscopy and biopsy proved adenocarcinoma colon. He underwent re-exploration and right hemicolectomy was done. The.fistula stopped and he was discharged from the hospital after 10 days. This case highlights the fact that in the presence of a distal obstruction, fistula will not close. More impor tantly, what the patient had was not appendicitis but subacute intestinal obstruction I The pain was due to colic.

It can be discussed under the following headings. 1. Recognition and aetiology 2. Phase of stabilisation 3. Nutrition-more details on page 207 4. Investigative phase 5. Phase of definitive management a. Surgery b. Skin care c. Abdominal wall defect 1. Recognition and aetiology • Delay in recovery from paralytic ileus or a common phrase used-when the patient is not doing well are early indications of a leak or a breakdown of anastomosis. Once the fistula establishes, diagnosis is easy because the drain will start draining intestinal contents including food particles (if oral intake is started). Intra-abdominal collections, abscess, fever, sepsis are other features of leak. • Recognising the cause is important because it can dictate treatment. To give an example: If any foreign body is left in the abdomen, exploration and removal may be urgently required. • If a specific disease is suspected such as tuberculosis, anti­ tuberculous treatment may cure a fistula. 2. Phase of stabilisation • Nil by mouth, total bowel rest, introduction of a nasogastric tube (Ryle's tube) with continuous drainage decreases fistula output. • Proton pump inhibitors decrease gastric secretions. • Protection of skin is by liberal zinc oxide application. • Common fluid and electrolyte problems seen in patients with GI fistula include dehydration, hyponatraemia, hypokalaemia and metabolic acidosis. T hey have to be corrected. • Drainage of collections by CT or ultrasound-guided aspiration or pig tail catheter insertions. • Broad-spectrum antibiotics, with anaerobic coverage. 3. Nutrition (more details on page 207) • Nutrition has been discussed in detail in the chapter on fluid­ electrolyte balance and nutrition. A few points worth

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mentioning are that more distal the fistula, less is the requirement of total parenteral nutrition (TPN). TPN is safe today. One may have to wait for 4-6 weeks with TPN for the final repair of complicated fistula such as lateral duodenal fistula or ilea! fistula. • Placement of nasogastric, nasojejunal tubes, per­ cutaneous gastrostomy or jejunostomy for nutrition is an important step which can be done with the help of radiology and imaging department. 4. Investigative phase a. • • • • •

Routine biochemical and haematological investigations Blood urea, serum creatinine, blood sugar Serum electrolytes Serum albumin, transferrin Blood culture helps in giving appropriate antibiotics Chest X-ray to rule out static pneumonia or ARDS, pleural effusions (source of sepsis also). b. Imaging studies • USG: Drainage of collections by catheters. If drainage persists, suspect fistulous communication. • Fistulograms to define the exact site of fistula-proximal or distal-gastric or intestinal (Key Box 28.23).

Fig. 28.74: Small bowel exteriorisa­ tion was done for enteric perfora­ tion after resection-safe option Fig. 28.75: Severe nutritional when there is gross contami- deficiency following duodenal nation fistula-autocannibalism

•._, FISTULOGRAM/CT/CONTRAST GI SERIES

• What is the cause of the fistula? • Is the bowel completely disrupted or is it a lateral fistula with the bowel in continuity? • What is the length of the fistula tract? • Is there an abscess cavity? • What is the size of the bowel wall defect? • Is there a distal obstruction?

• Contrast CT abdomen: To detect collections, disconti­ nuity, diseased segments, distal obstruction foreign body. Extremely useful investigation in intestinal fistula. • Colonoscopy: Barium enema are other investigations used in specific situations such as colonic fistulae. 5. Phase of definitive management a. Surgical intervention • Drainage or aspiration of pus urgently when there is sepsis before doing an elective repair. • Feeding jejunostomy as in high fistulae, e.g. duodenal or oesophageal fistula. • Diversion/exclusion/colostomy/bypass are various other treatments depending upon the location of fistula (Fig. 28.74). • Definitive treatment: It includes restoration of continuity­ resection and anastomosis, internal diversion or exteriori­ sation of bowel as in suspicious viability (mesenteric ischaemia or enteric perforations-friable bowel, known for leak and releak).

Fig. 28.76: Extensive excoriation of the skin in a faecal fistula. Zinc oxide has been applied to the abdominal wall

b. Methods of skin care (Figs 28.75 and 28.76) Pouches, stoma bags of different sizes are available. • Creams and ointments-zinc or petroleum-based • Suction catheter placed in situ: Low pressure suction 60-80 mmHg. Generally, dressings need to be changed every 4th hourly. Pouch/reservoir system should be employed. c. Abdominal wall defects • Depending upon the size of the defect, immediate or delayed closure can be achieved with tension-releasing incisions on the external oblique or laparostomy and secondary suturing, skin grafting or by using prosthetic mesh such as Marlex or Prolene. SMALL INTESTINAL DIVERTICULA Introduction Small intestinal diverticula are far less common than colonic diverticula. Multiple sac-like mucosa( herniations occur

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through weak points in the intestinal wall where blood vessels penetrate. Incidence Diverticula are more common in duodenum than jejunum or ileum (Fig. 28.77). Pathophysiology • It is believed to develop as a result of abnormalities in peristalsis, intestinal dyskinesis and high segmental intra­ luminal pressures. • They occur in the mesenteric border unlike Meckel's which occurs in the antimesenteric border. • Diet low in fibre and rich in fat and some visceral myopathy have been blamed for development of dive1iicula. Clinical • Asymptomatic • Occult blood in the stools-positive

• Anaemia and symptoms such as fatigue, weakness, peda oedema. • Malabsorption-diarrhoea, flatulence, weight loss • Pain abdomen, signs of peritonitis such as tenderness guarding or localised abscess. Investigations • Specific investigations are enteroclysis and enteroscopy. • CT with contrast is the ideal investigation. • Often dive1iicula are not suspected but on a CT scan obtaine< for some pathology in the abdomen may reveal a localisec abscess. Later, at laparotomy it can tum out to be diverticuh (Figs 28.78 and 28.79). Treatment • Resection and anastomosis of the bowel containinf diverticula. INTERESTING 'MOST COMMON' FOR INTESTINES

Fig. 28.77: Diverticula form mesenteric border of jejunum. It was the cause of anaemia due to occult blood loss

• Most common site of gastrointestinal tuberculosis is ileocaecal region. • Most common site of ulcerative colitis is rectum. • Most common extraintestinal manifestations of ulcerative colitis are peripheral arthritis and ankylosing spondylitis. • Most common site of gastrointestinal perforation in enteric fever is terminal ileum. • Most common neoplastic lesion of small intestine is lymphoma. • Most common anal problem in Crohn's disease is fissure in ano. • Most common site of adenocarcinoma in intestines is duodenum. • Most common site of malignant carcinoids is terminal ileum. • Most common site of carcinoid in intestines is appendix.

WHAT IS NEW IN THIS CHAPTER?/ RECENT ADVANCES

Figs 28.78 and 28.79: Enteroclysis. First picture showing filling defect and a clear view of diverticula in the second picture

• All topics have been updated with flowcharts and coloured pictures. • Small intestinal divericula, intestinal fistulae and tumours have been discussed in more detail. • Radiation enteropathy has been added. • Multiple choice questions have been added

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699

MULTIPLE CHOICE QUESTIONS 1. Pathology of ulcerative colitis includes all of the following except: A. Punched out ulcers B. Pseudopolyposis C. Pipe stem colon D. Pus cells 2. Toxic megacolon is seen in the following except: A. Intestinal tuberculosis B. Ulcerative colitis C. Amoebic colitis D. Salmonella colitis 3. String sign of Kantor is seen in: A. Crohn 's disease B. Tubercular enteritis C. Typhoid enteritis D. Amoebic colitis 4. Guarding and rigidity can be minimal in enteric perforation of terminal ileum because: A. The perforation is usually small B. It is self-sealing and self-limiting C. Zenker's degeneration of abdominal muscles D. Occurs in the antimesenteric border of the tetminal ileum 5. Flask-shaped (bottle neck) ulcers are a feature of: A. Intestinal tuberculosis B. Crohn's disease C. Intestinal amoebiasis D. Typhoid enteritis 6. Malignancy in small intestine is rare because: A. Bacteria in the intestine are protective B. Blood supply is very good C. No stasis, rapid transit of food D. Low levels of immunoglubulins 7. Most common site of gastrointestinal perforation in enteric fever is: A. Duodenum B. Jejunum C. Colon D. Terminal ileum 8. Adaptation to short gut syndrome includes all of the following except: A. Decreased transit time B. Increased villus size C. Absorption from colon D. Increased length of bowel 9. The symptoms of carcinoid syndrome is due to: A. Histamine B. Serotonin C. Prostaglandins D. Epinephrine

10. Tenderness in the Sir Philip Manson-Bahr's point is a feature of: A. Rectosigmoid involvement in amaoebic colitis B. Acute appendicitis C. Acute cholecystitis D. Crohn's disease 11. The commonest site for gastrointestinal stromal tumour is: A. Oesophagus B. Stomach D. Jejunum C. Duodenum 12. Carney's triad includes all of the following except: A. Gastrointestinal stromal tumours B. Pulmonary chondromas C. Extra-adrenal chondromas D. Familial hamartomatous polyposis 13. One of the drugs used in the treatment of carcinoid syndrome is: A. Metronidazole B. Bromocriptine C. Streptomycin D. Serotonin 14. The most common site for a carcinoid tumour is: B. Duodenum A. Stomach D. Rectum C. Appendix 15. Causes of short gut syndrome include all of the following except: A. Mesenteric ischaemia B. Necrotising enterocolitis C. Crohn's disease D. Radiation enteritis 16. C-kit receptor is expressed in: A. Gastrointestinal stromal tumour B. Peutz-Jegher syndrome C. Carcinoid syndrome D. Crohn's syndrome 17. The most recent and promising drug used in the treatment of Crohn's disease is: A. Trastuzumab B. Infliximab D. Fab antibodies C. Monteleukast 18. Most common anal problem in Crohn's disease is: A. Anal fistula B. Haemorrhoids D. Perianal abscess C. Anal fissure 19. Transmural inflammation is characteristic of: B. Tuberculous enteritis A. Crohn 's disease C. Typhoid enteritis D. Ulcerative colitis 20. 'Hose pipe rigidity' is a feature of: B. Tuberculous enteritis A. Crohn 's disease C. Typhoid enteritis D. Ulcerative colitis

ANSWERS A

2 A

3A

11 B

12 D

13 B

4C

5C

6C

7 D

8A

9 B

10 A

14 C

15 D

16A

17 B

18 C

19 A

20 A

29 Large Intestine • • • • • •

Surgical anatomy Function Tumours Familial polyposis coli Hereditary nonpolyposis Colorectal cancer

• • • • • •

Carcinoma Colon screening Diverticular disease Faecal fistula Colonic stricture What is new?/Recent advances

Splenic flexure

SURGICAL ANATOMY • Large intestine extends from ileocaecal valve to anus. It has five segments: right colon, left colon, transverse colon, sigmoid colon, rectum and anal canal (Fig. 29.1). • Average length is about 135-150 cm. • Interestingly, alternating portions of the colon are mobile and fixed. Ascending colon and descending colon are fixed but caecum, transverse colon and sigmoid colon are mobile. Mobile structures can undergo twisting (volvulus). • Layers of colon: Mucosa, submucosa, muscularis propria and serosa. Inner circular and outer longitudinal muscle layer constitute muscularis propria. Inspite of4 layers, wall ofthe colon is thin. Hence it distends much more in obstruction. Ascending colon

Caecum • 7.5 cm in both length and breadth • Blind pouch • Completely covered by peritoneum • Mobile Diseases • Carcinoma • Tuberculosis • Volvulus-rare • Amoebic typhlitis • lntussusception

Superior mesenteric artery

Sigmoid colon

Fig. 29.1: Anatomy of the colon and parts

Ascending colon-15 cm long • Caecum continues as ascending colon. • Covered by peritoneum in front and on both sides. • In 25% of patients, it has a mesentery. 700

Large Intestine

• Right paracolic gutter is deep on the lateral aspect of ascending colon-space for para-colic abscess in cases of perforation peritonitis. Transverse colon-SO cm long • Most mobile part of large intestine • It is suspended by transverse mesocolon, loops down and is adherent to the posterior wall of omental bursa. Diseases • Cancer • Ulcerative colitis Descending colon-25 cm long • Continues as sigmoid colon • Retroperitoneal (like ascending colon) • Also has paracolic gutter

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Recognise large intestine by 1. Taenia coli 2. Omental appendices-appendicular epiploicae 3. Haustrations 4. Large diameter (calibre) Names of three taenia coli 1. Mesocolic: Transverse and sigmoid colon are attached by this. 2. Omental: To which omental appendices attach. 3. Libra (free): Nothing is attached. PEARLS OF

WISDOM

Since taenia are shorter than intestine, the colon becomes sacculated between taenia forming haustra. Arterial supply (Fig. 29.2) I. Superior mesenteric artery, a branch of abdominal aorta arises at the level of first lumbar vertebra (Ll ). It supplies the entire small bowel and the right colon up to proximal 2/3rds of transverse colon. Branches of superior mesenteric artery (SMA) supplying colon are: A. Middle colic artery supplies ascending colon, hepatic flexure and the transverse colon mainly. It divides into right and left branches.

Sigmoid-40 cm long • S-shaped • Ends as rectosigmoid junction where taenia coli ends. Diseases • Volvulus • Diverticulosis • Cancer Right colon (Table 29.1) Big and hepatic flexure is broad. Left colon • Small and splenic flexure is acute. Hence, ischaemic colitis commonly affects splenic flexure (Table 29.1). • Splenic flexure is deeply situated. T herefore, malignancy in this area can be easily missed. Muscle coat Outer longitudinal muscle is arranged in the form of three strips called taenia coli. All three join at the rectosigmoid junction and form a complete longitudinal layer of the rectum. These three taenia coli converge at the base of the appendix. T his is an important method to localise appendix. Inner circular muscle coalesces distally to form internal anal sphincter.

Middle colic---��------- Inferior pancreatico­ duodenal

�''=r-----:c1':"'-+f,.._.--- JejunaI and ,__..._,.,,,.,.____, ileal branches

Fig. 29.2: Blood supply of the right colon

Comparison between right colon and left colon Right colon

Left colon

1. 2. 3. 4. 5. 6. 7. 8. 9.

I. 2. 3. 4. 5. 6. 7. 8. 9.

Big Hepatic flexure is broad Hepatic flexure is not deep Paracolic space is broad Luminal diameter is big Contents are liquid Growth pattern is usually ulceroproliferative Obstruction is not common More common in women

Small Splenic flexure is acute and narrow Splenic flexure is deep Left paracolic space is narrow Luminal diameter is narrow Contents are solid Growth is annular stricture Hence, obstruction is common More common in men

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B. Right colic artery supplies right colon. C. Ileocolic artery supplies the terminal ileum and ascending colon. It divides into anterior and posterior caecal branches and supplies caecum and appendix through appendicular artery. 2. Inferior mesenteric artery (IMA), a branch of abdominal aorta arising at the level ofL3 supplies the left colon up to mucocutaneous junction at the lower end of anal canal (Hilton's line). Its branches are (Fig. 29.3): A. Left colic artery which anastomoses with branches of middle colic artery. It divides into upper and lower branches supplying the descending colon. B. Three sigmoidal branches supply sigmoid colon C. Superior haemorrhoidal artery (rectal) • The anastomotic branches form marginal artery of Drummond, which is relatively nanow in the region of sple­ nic flexure (another reason for development of ischaemia). • Arc of Riolan is the anastomotic arcade formed between branches of IMA and SMA. Venous supply • Follows the corresponding artery and empty into superior and inferior mesenteric veins, ultimately draining into the portal vein (Fig. 29.4). • Thus, any inflammatory conditions of the colorectal/ surgical procedures if they infected, infection can easily spread into portal vein and result in portal pyaemia.

Ascending branch of left colic artery Descending branch of left colic artery

Left colic artery

mesenteric artery

Sigmoid arteries '---- Sigmoid colon

Fig. 29.3: Arterial supply of left colon

Lymphatic drainage (Fig. 29.5) • Nl: Epicolic, paracolic nodes are the first to get involved. • N2: Nodes at the origin of ileocolic and middle colic arteries-intermediate nodes. • N3: Nodes at the origin of superior and inferior mesenteric arteries. They are involved in approximately 50% of the

Superior pancreaticoduodenal vein-----=" Portal vein ----..�_,...._.Ill

Right external iliac vein-------. Middle rectal vein-----------....... Internal pudenda! vein --- ------....-

�--------- Superior rectal vein

Inferior rectal vein--------�

Fig. 29.4: Venous drainage

Large Intestine

Fig. 29.5: Lymphatic drainage (see text for description of N1, N2

and N3)

patients with carcinoma colon at presentation to the hospital. These are called principal nodes. Colorectal nerve supply • Sympathetic (inhibitory) arise from T l 0-Tl 2 and L l-L3 • Parasympathetic (stimulation). A. Vagus nerve supplies right and transverse colon. B. Sacral nerves (S2-S4 which fonn nervi erigentes) supply distal colon, i.e. splenic flexure onwards. Significance Colonic pseudo-obstruction starts from splenic flexure. Transition zone of vagal supply to sacral nerve supply.

Motility Colon has 4 types of motility. Propulsive, retropulsive, mass peristalsis and gastrocolic reflux. Thus, contents travel aborally. Retropulsive activity is more in the right colon, these allowing the contents to 'churn' more and more. Mass contractions are found more in left colon-specially after meals. Factors which stimulate the colonic motility • Dietary fat • Rich fibre diet • Physical activity-walking, change in posture, exercises • Emotional activity • Less water intake Constipation (Key Box 29.1) • It depends upon several factors such as food habits, genetic, social customs. • Generally a patient is said to have constipation if he passes less than 2 stools per week. • In addition to the low fibre diet, emotional feelings and many rectal diseases also cause constipation. Example­ prolapsed rectum, solitary rectal ulcer syndrome. Colonic disease such as megacolon-Hirschsprung disease is an important cause of constipation in children. • Increasing constipation in elderly patient suggest carcinoma in left colon. Needs to be evaluated by colonoscopy. Recycling • Recycling of various nutrients takes place in the colon. Examples: Fennentation of carbohydrates, short-chain fatty acids and urea cycling. • Butyrate is the main product of bacterial fermentation. It is required mainly as a fuel for colonic epithelium. • To accomplish this, the colon depends highly on its bacterial flora, especially for degeneration and fermentation ability. PEARLS OF

Secretion Colon also secretes K+ and c1-. It is increased in colitis. Chloride secretion is increased in cystic fibrosis.

WISDOM

More distal the colon-more is the protein metabolism and putrefaction resulting in carcinogens and greater exposure to colonic mucosa. Hence, two-thirds of colonic cancer occur in the left colon.

COLONIC FUNCTION Absorption Water content of faecal matter is reduced to 1000-1500 ml per day. Thus stools become solid. Similarly, sodium, potassium and bile salts are also absorbed. Significance of this is in cases of diarrhoea, there is loss of water, sodium, potassium and other electrolytes. Amino acids and fatty acids are also absorbed slowly in the colon.

703

MHdilllllllWIIIII� • • • • • • •

CONSTIPATION

Digestion and absorption In a child-Hirschsprung's disease Adult women-idiopathic/following child birth Middle aged women-following hysterectomy Elderly man-carcinoma left colon Constipation with severe pain-anal fissure Depression patient-psychotropic drugs-used to treat schizophrenia, antidepressants and antiepileptic drugs.

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Colonic bacteria • Anaerobic bacteria: They constitute more than 99%. The most common pathogen is bacteroides fragilis (10 10 lg of faeces). Other organisms are clostridia, cocci, etc. • Aerobic bacteria: Escherichia coli is the most common organism about 10 7lg of faeces. Other organisms are Klebsiella, Proteus and Enterobacter. • Normal function: Bacteria degrade bile pigments thus resulting in brown coloured stools. They also help in colonic motility and absorption. Fatty acids produced by bacteria supply nutrition to colonic epithelium. Bacteria also supply vitamin K to the host. Prebiotics and probiotics • Prebiotics are non-digestible food ingredients that stimulate the growth and/or activity of bacteria in the digestive system in ways claimed to be beneficial to health. Traditional dietary sources of prebiotics include soybeans, inulin sources (such as Jerusalem artichoke, jicama, soya and chicory root), raw oats, unrefined wheat, unrefined barley, and yacon. • Probiotic is defined as a "live microbial feed supplement which beneficially affects the host animal by improving its intestinal microbial balance". They are non-degradable oligosaccharides. They stimulate the growth of beneficial intestinal bacteria. • Probiotics are dietary supplements which contain live cultures of bacteria and yeast that are beneficial to colonic and host function. The common species used as probiotics are Lactobacillus and Bifidobacterium. Probiotics stimulate immune function, exhibit anti-inflammatory property and suppress pathogenic organism. • Clinical application: When a person takes antibiotics, both the harmful bacteria and the beneficial bacteria are killed. Bacterial change in flora alters carbohydrate metabolism with decreased short-chain fatty acid absorption and result in osmotic diarrhoea. In a similar fashion, antibiotic therapy causes increase in the growth of Clostridium difficile. Thus, probiotics have been recommended in antibiotic induced diarrhoea. Also, they have been used to treat diarrhoeas in ulcerative colitis, in pouchitis (inflammation of the pouch after total proctocolectomy for ulcerative colitis) and in necrotising colitis in children. TUMOURS OF THE LARGE INTESTINE Benign tumours (Fig. 29.6) are usually referred to as polyp, which means elevated from the surface. They are as follows: ADENOMATOUS POLYP (Key Box 29.2) • It may be a villous adenoma which is a flat lesion or a tubular adenoma having a pedicle. Tubular is more common.

Normal intestinal fold

Fig. 29.6: Polyp

Fig. 29.7: Carcinoma in polyp

• They give rise to bleeding, mucus diarrhoea and hypokalaemia. • They can be single or multiple • They are dysplastic • They are premalignant and the risk of malignancy is greater with increase in the size of the adenoma. • They can be removed with the colonoscope-polypectomy. • Malignant potential of villous adenoma is more than tubular adenoma (Fig. 29.7). • Adenoma less than 1 cm-risk of malignancy is 1 %; 1-2 cm is 10%; > 2 cm is 30%.

11$

,

ADENOMATOUS POLYPS • Most of the neoplastic polyps occur in elderly patients (> 50 years). • Most of them are pedunculated • Most pedunculated polyps are removed by colonoscopic snaring. • Adenoma larger than 5 mm in diameter carry risk of malignant potential. • More the polyps, more chances of synchronous carcinoma • Flat adenomas also carry malignant potential.

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• Symptoms and signs of polyps: Bleeding per rectum is the most common symptom. Fresh bleeding is seen in rectal polyps. Typically it is painless. It is intermittent. If it is associated with change of bowel habits means probably a malignant change. These changes include mucus discharge, tenesmus, sometimes constipation. In children, polyp may project outside the anus. In such cases, it has to be distinguished from prolapsed rectum. Treatment • Colonoscopy and polypectomy is the standard treatment. • If specimen shows invasive carcinoma, radical surgery needs should be done. PEARLS OF WISDOM

Although most neoplastic polyps do not evolve to cancer, most colorectal cancers originate as a polyp. HAMARTOMATOUS POLYP (JUVENILE POLYP) • This can occur in the colon as in Peutz-Jeghers syndrome. Risk of malignancy is very limited. Symptomatic polyps need to be treated. • Juvenile polyps are usually single and occur in children. They give rise to bleeding and are easily resected. They do not have malignant potential.

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FAMILIAL POLYPOSIS COLI (FPC) OR FAMILIAL ADENOMATOUS POLYPOSIS (FAP)

• FAP is a genetic disorder inherited as a Mendelian dominant. The gene APC (adenomatous polyposis coli) is located on the short arm of chromosome 5. Prevalence: 1 in 10,000. lt is clinically defined by the presence of more than I 00 colorectal adenomas (Figs 29.8 and 29.9). • It is transmitted from both sexes. The incidence is same in either sex. • When it is associated with desmoid tumour, craniofacial osteoma, epidermoid cysts, congenital hypertrophy of retinal pigment epithelium, it is described as Gardner's syndrome (Key Box 29.3). • When familial polyposis coli is associated with central nervous system tumour and glioblastoma, it is called Turcot's syndrome. • 50% of them have benign gastric polyps and 90% of them have duodenal polyps.

Clinical features

• Runs in families; other members of the family are affected. • Manifests at the age of 20 in the form of blood and mucus in the stool, loose stools, etc. It produces crampy lower abdominal pain. • Anaemia, weight loss and protein malnutrition occur slowly. • Mean age of development of carcinoma is 39 years.

Complications of FAP

Malignancy ( 100% risk) Investigations

Colonoscopy-details in page 707 Treatment

NSAID: Sulindac 300 mg, twice a day and aspmn 325 mg once a day have been found to decrease the size of polyps.

Fig. 29.8: Thousands of polyps. One of them is 2 cm and is pedunculated

Fig. 29.9: Close-up view of the polyps

•,............ FAMILIAL POLYPOSIS COLI-SUMMARY

�

• Polyps are more than 100 (colorectal adenomas) • Other mesodermal tumours-desmoid tumours, osteoma, epidermoid cysts can be present (Gardner's syndrome). • Large bowel is predominantly involved. • Year of development of carcinoma-mean age 39 years. • Polyposis gene-autosomal dominant APC gene. • Other syndrome-Turcot • Sigmoidoscopy from age of 15 at intervals is the investigation of choice. • lleoanal anastomosis with pouch-restorative proctoco­ lectomy-advisable above age of 30. • Surgery is the only means of preventing colonic cancer. Remember as POLYPOSIS

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• Patients with FAP who are above the age of 30 have high chances of having a carcinoma in the colon. Hence, even when there is no malignancy, surgery is advisable. Types of surgery (Figs 29.10 and 29.11) • Many patients do not like ileostomy. Hence, a subtotal colectomy with ileorectal anastomosis can be done. This is done provided that rectum is examined frequently and endoscopic snaring of the polyps is done regularly, especially in a young patient. • Restorative proctocolectomy with ileoanal anastomosis by using a pouch is another alternative. However, it is a major surgical procedure and should be undertaken only by an experienced surgeon. Screening Starts from the age of 15 years using sigmoidoscopy. PEARLS OF

WISDOM

I/there are no adenomas by the age of 30 years, FAP is unlikely.

METAPLASTIC POLYP Also called hyperplastic nodules. They are of viral aetiology. They do not have malignant potential.

Fig. 29.12: Synchronous carcinoma in adenomatous polyps

HNPCC (HEREDITARV NONPOLVPOSIS COLORECTAL CANCER) • Autosomal dominant, no polyps • Lynch's syndrome I: Site specific colorectal cancer. • Lynch's syndrome 11: Cancer family syndrome-they have extracolonic cancers such as endometrial cancer, ovarian cancer, transitional cell cancer, etc. • Lifetime risk of developing colorectal cancer is 80%. • Synchronous carcinoma means more than one cancer at the time of diagnosis. Metachronous carcinoma means appearance of second carcinoma after 6 months can occur here (Fig. 29.12). Diagnostic criteria (Amsterdam criteria 11) I. At least 3 members in a family should have colorectal cancer-two of whom are first degree relations. 2. At least two consecutive generations 3. At least one relative should have colorectal cancer by less than 50 years of age. 4. Exclusion of FAP Screening Increased incidence of proximal colonic cancer. EXAMINATION OF COLON

Figs 29.10 and 29.11: Familial adenomatous polyposis with two malignancies-lower rectum and hepatic flexure. This patient was being treated for chronic diarrhoea for 7-8 years with various medications. 'He underwent colonoscopy for the first time in our hospital.Total proctocolectomy specimen (Courtesy: Dr Challa Srinivas Rao, Professor, Dept of Surgery, and Dr Ravi, Konaseema Institute of Medical Sciences (KIMS), Amalapuram-Andhra Pradesh)

Anoproctoscopy: One can examine up to 10-12 cm of anal canal and rectum. Rubber band ligation (for piles) and polypectomy can be done with this instrument. Flexible sigmoidoscopy: The scope measures about 60 cm in length. One can easily reach up to splenic flexure. Bowel wash or an enema is given before the procedure. No sedation is required (Figs 29.13 and 29.14).

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(more than 1 malignancy at the time of diagnosis) is found in about 5% of the patients. Colon is also one of the sites of metachronous cancer (new malignancy appearing after 6 months of curative surgery). More than 95% are adeno­ carcinoma and surgery remains the most effective treatment. Survival has improved because of early diagnosis and multimodality of the treatment. Over a period of years the understanding of development of carcinoma has changed and more and more molecular biology of colonic cancer is being discussed. The Fearon Vogelstein adenoma-carcinoma multistep model of colorectal neoplasia represents one of the best-known models of carcinogenesis (Key Box 29.4).

............................. . KEY BOX 29.4

FEARON-VOGELSTEIN MODEL

Normal [ tissue

Fig. 29.13: Rigid sigmoido- Fig. 29.14: Flexible sigmoidoscope scope

Fibreoptic colonoscopy can assess the entire colon. It is 100-160 cm in length. Usually there will be multiple polyps varying from a few millimetres to centimetres. Biopsy has to be taken. Polyps are visible after 15 years and certainly by the age of 30 years. • It is the investigation of choice in most of the large intestinal lesions. • It permits examination of entire colon and terminal ileum. • Colon is prepared by polyethylene glycol given orally. • Risk of perforation of colon is Jess than 0.1%. Indications Diagnostic • Lower gastrointestinal bleeding • Inflammatory bowel diseases • Abnormal finding in barium enema • Family history of colorectal cancers • Biopsy of caecum/ileum in suspected cases of cancer. • Tleocaecal tuberculosis-to take biopsy Therapeutic • Control of bleeding-coagulation or injection sclerotherapy. • Snaring of polyps • Removal of foreign body • Detorsion of volvulus • Decompression of pseudo-obstruction CARCINOMA COLON Introduction It is the second most common cancer and cancer related death cases in the western world next only to lung cancer. The incidence increases with age. Multiple synchronous lesions

Normal colonic epithelium APC

Tumour [ initiation

Dysplastic aberrant focci Early adenoma K-RAS

Tumour [ progression

Intermediate adenoma DCC Late adenoma p53

Neoplasia [

Carcinoma

APC, K-RAS, DCC and p53 are oncogenes that convert normal cell to cancerous cell

• Terminology: Before we start the discussion on carcinoma colon, we shall study a few tenninologies used in carcinoma colon. They are synchronous carcinoma, metachronous carcinoma, familial colorectal carcinoma. SYNCHRONOUS CARCINOMA • Moertel 's definition: Synchronous cancers as those occurring within 6 months of the first primary cancer, or two or more histologically distinct simultaneously detected malignancies or more than one malignancy at the time of initial diagnosis. This will happen especially in cases of colon and upper aerodigestive tract wherein the stimulus or aetiological factor for malignancy affects different parts of the organ. • Colon, head and neck, oesophagus are the site of synchronous carcinomas. • In cases of carcinoma colon with specific aetiological factors such as familial polyposis coli, ulcerative colitis, hereditary non-polyposis cancer, often carcinoma is synchronous. • Thus, it is important to do a complete colonoscopy when a patient comes with colonic carcinoma because he/she may be having another synchronous carcinoma elsewhere. See the case report in the next page.

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• Certain criteria have been laid upon for the diagnosis o: these conditions which have been discussed already.

CLI N I C AL N OTES A 68-year-old lady was admitted with large bowel obstruction. Plain X-ray abdomen showed intestinal obstruction. Exploratory laparotomy was done. A 3 cm constricting growth was identified at rectosigmoid junction and high anterior resection and anastomosis was done. On the 4th postoperative day, the patient was allowed liquid diet. Distension increased. For another 3 days, distension went on increasing. Plain X-ray abdomen revealed obstruction with more gas than before. The patient was having colicky abdominal pain. Explorato,y laparotomy was done. Findings at 2nd laparotomy­ anastomosis was intact. Transverse colon was hugely dilated. Careful palpation of splenicflexure revealed one more growth. A resection and anastomosis was done again. Patient was discharged after 10 days. The first surgeon agreed that after finding out the rectosigmoid growth, he did not look for any other lesions (mistake). This was obviously a case of synchronous carcinoma.

METACHRONOUS COLONIC CANCER • Metachronous cancer was defined as those cancer occurr­ ing more than 6 months following resection of one malignancy. • A few examples of metachronous site are: Colorectum, breast, kidney. • Family history of hereditary, nonpolyposis colorectal cancer (HNPCC or Lynch syndrome), an autosomal dominant disease, also can present with both synchronous or metachronous colorectal cancers. • It is more common in females • Common usually at young age. • The associated genetic defect lies at the mismatch repair genes, responsible for the correction of DNA bases mismatch. • The coexistence of adenomatous polyps is also considered a risk factor for the development of metachronous lesions. Thus, after treating one carcinoma, example-carcinoma sigmoid, annual colonoscopy is recommended. If polyps are detected, patients have to be informed about the polyps and their potential of malignancy. • Survival is better FAMILIAL COLORECTAL CANCER • All these have a carrier gene and thus, run in families. Malignancies occur in young age group. Often they are synchronous. Metachronous lesions are not uncommon.

• Genetic instability is the chief factor responsible. Th� instability can be at chromosomal level called chromosomai instability or at DNA level called microsatellite instability­ MSI. As a result of this after the cell division by duplication, mismatched genes develop. These genes cannot be repaired. This predisposes to mutation, results in a cancer gene. • Familial polyposis coli accounts for about 1% of colorectal cancers. However, incidence of malignancy is 100%. Gardner's and Turcot's syndrome are the variants ofFPC. • HNPCC accounts for about 5 to 10% of colorectal cancers. They also have extracolonic cancers such as endometrial, ovarian and urinary bladder cancers. • Other familial syndromes are Cronkhite Cana da syndrome. It is more common in females. Multiple polyps develop in stomach, duodenum and in the colon. Diarrhoea is the clinical presentation. Other features include pigmentation, alopecia, loss of weight and cachexia. Chances of developing malignancy is about 15%. Precancerous conditions (Key Box 29.5) 1. Polyps: Environmental and genetic factors favour the development of colonic polyps and their transfonnation into malignancy. The incidence of malignancy is increased when the polyp is more than 1 cm, polyps are multiple or flat (Table 29.2).

•=t•WW',1 RISK FACTORS ASSOCIATED WITH COLON CANCER

Risk factor Familial polyposis coli HNPCC Ulcerative colitis Crohn's colitis Adenomatous polyps

�

Incidence of cancer 100% chances of colorectal cancer 80% chances 10-20% after 20 years 5% after 10-20 years 1-20% depending upon the size

Summary of colonic polyps Type

Cause

Malignant potential

Features/syndrome

1. 2. 3. 4.

Benign tumour "Misfire" Genetic disorder Hyperplasia

1-10% Negligible 100% Nil

Hypokalaemia, diarrhoea Peutz-Jeghers syndrome Gardner's and Turcot's syndrome Asymptomatic

Adenomatous polyp Hamartomatous polyp Familial polyposis coli Metaplastic polyps

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PEARLS OF WISDOM

PEARLS OF WISDOM

Familial polyposis coli has 100% chance of carcinoma. 2. Inflammatory bowel disease A. Ulcerative colitis is a definite precancerous condition. Presence of dysplasia diagnosed by colonoscopic biopsy is an indication for colectomy. B. Crohn's involving colon also has a mildly increased risk of developing carcinoma when compared to ulcerative colitis. C. Schistosomal colitis: The risk of colorectal cancer is increased in patients with long-standing schistosomal colitis. Long-standing cases are associated with mild to severe grades of colonic epithelial dysplasia. Thus ulcers or pseudo-polyps can occur. These dysplastic changes are considered as premalignant. D. Radiation exposure: Usually it is mucin secreting adenocarcinoma with poor prognosis. E. Ureterosigmoidostomy increases risk of colonic cancer over 100-500 times. Aetiological factors 1. SAD factors: It is sad to know that SAD factors are responsible for carcinoma colon. They are S-Smoking, A-Alcohol, D-Dietary factors. Diet rich in red meat has high animal fat. This alters intestinal bacteria, which convert primary bile acids into secondary bile acids. This is the beginning of formation of carcinogenic polycyclic aromatic compounds. After cholecystectomy, there is increase in free bile acid concentration, thus increasing the risk of colonic cancer. Thus increased roughage is associated with increased transit time which in turn reduces exposure of mucosa to carcinogens. • Increase incidence is found in western countries wherein diet rich in animal fat is consumed in large quantity. Incidence is more after the age of 50 years. Obesity, poor exercise and smoking are the contributing factors. • Some interesting observations are also found in females with colonic cancer which have been depicted in Key Box 29.6.

Calcium salts are protective. They form insoluble bile salt complexes, thus reducing the concentration of bile acids in the colon. Pathological types (Fig. 29.15) • It is an adenocarcinoma-columnar. Rectum (40%) and sigmoid (20%) take a major share in colorectal carcinoma followed by caecum (12 to 15%). Multiple synchronous cancers are also common in the colon. A. Annular stricture: Common in left colon (splenic flexure, pelvic colon). B. Tubular stricture: Common in left colon and at the rectosigmoid junction. C. Ulcerative lesion: Ascending colon or caecum. D. Proliferative growth: More in right colon, the least malignant, fleshy and bulky polypoid lesion (Fig. 29.16). • It is a columnar cell adenocarcinoma. In about 5% of cases, it undergoes mucoid degeneration. Such tumours carry poor prognosis. They spread to the liver very fast and secondaries produce mucoid material.

A

B

C

D

Figs 29.15A to D: Types of carcinoma colon

•=11NW111W,� WOMEN AND COLONIC CANCER

�

• Caecum is more commonly involved than other parts. • Women with breast cancer have increased incidence of colonic cancer. • Women smokers have increased chances of colonic cancer. • Women who have undergone cholecystectomy have increased chances of colonic cancer. • Metastasis to ovary is mostly haematogenous (1 to 10%).

Fig. 29.16: Ulceroproliferative growth in the descending colon

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•

•

A

•

•

B

C

Figs 29.17A to C: (A) Carcinoma caecum, (B) Carcinoma cae­ cum with secondaries in the liver, (C) Carcinoma left colon with intestinal obstruction

Fig. 29.18: Large secondary in the liver. Look at the umbilication (central necrosis)

Clinical features of carcinoma colon (Figs 29.17A tc C and Table 29.3) (Mnemonic TMA Pai) 1 I. Tumour: The mass produced by carcinoma caecum and even hepatic flexure is palpable. It is firm to hard, irregular and with or without fixity. • Occasionally, growth at pelvirectal junction can be felt on rectal examination. • However, on left-sided constrictive lesions, growth is not often felt. It is the hard faecal matter and lymph nodes which are felt as a mass. 2. Metastasis: 5-10% of the patients present with metastasis to liver (mucoid adenocarcinoma), ascites, etc. Distant metastasis is not common (Fig. 29.18). 3. Anaemia is an imp01iant feature of carcinoma caecum. It may be due to blood loss or a proliferative growth secreting toxins causing suppression of bone marow. Asthaenia and anorexia are the other features. 4. Pain abdomen: Dull aching pain may be present. Colicky pain is due to chronic obstruction as in left-sided growths (napkin ring stricture). 5. Alteration in the bowel habits: A recent constipation, increase in the dose of laxatives followed by attacks of diarrhoea can be due to carcinoma colon. Diarrhoea is due to hard faecal balls, irritating the colonic mucosa resulting in increased secretion of mucus produced by proximal colon. 6. Intestinal obstruction is caused by constricted left-sided lesions (Fig. 29.19). On the left side, diameter of the colon is narrow, contents are solid and growth is constrictive. Lower abdominal distension, right to left peristalsis are the

Fig. 29.20: lntussusception due to carcinoma caecum

Fig. 29.19: Tubular stricture at rectosigmoid junction-cut opened specimen after resection

1 Late Padmashree Dr TMA Pai, was the founder of Kasturba Medical College which he started in 1953. He was also a banker, educationist. He is called 'Modern Architect ofManipal', where 'Manipal University' is situated.

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11111111...........� PECULIARITIES OF CARCINOMA OF CAECUM (Figs 29.20 to 29.26)

• Incidence is more in females • Presents with anaemia and a mass • Can present as acute appendicitis when the lumen of appendix is obstructed (in elderly patients). • It is a cause of intussusception (secondary) • Can present with flexion of hip due to infiltration of iliopsoas.

NIIMI�� PECULIARITIES OF CARCINOMA SPLENIC FLEXURE • Presents as obstruction • It is easily missed unless carefully looked for • Carries poor prognosis as many cases present with obstruction. • Often, it is inoperable

29.21: Pallor disproportionate to the blood loss-typical of carcinoma caecum

late features. Carcinoma sigmoid can cause colovesical fistula. To summarise • Early cases: It can be easily missed-such as change in bowel habits like diarrhoea, vague ill health, weakness (due to anaemia), intermittent bleeding per rectum, often attributed to piles or some other cause. Mass is usually not palpable. • Late cases: It can present with obstruction (rectosigmoid junction growth), perforation, intussusception (right-sided tumours), mass abdomen, secondaries in the liver, left supraclavicular nodes (Troisier's sign), etc. See Key Boxes 29.7 to 29.9.

.. . • • • • • • • •

MHIIIIIIWIIIIIIIIII� PECULIARITIES OF CARCINOMA SIGMOID (Figs 29.27 and 29.28)

• Commonly presents as obstructive lesion-constipation and intestinal obstruction. • Colovesical fistula: Carcinoma sigmoid is the 2nd commonest cause. • It can cause colovaginal fistula • It can also infiltrate ureter, uterus and ovary. • It can present as abscess in the lateral abdominal wall. PEARLS OF

WISDOM

Obstructed and perforated carcinoma colon have poor prognosis.

Spread 1. Local: For a long time, the lesion is confined to mucosa and submucosa. They grow in annular fashion and later

Carcinoma right colon and carcinoma left colon

Presentation Bleeding Abdominal discomfort Incidence Frequency Pathology Investigation Complication

Carcinoma right colon

Carcinoma left colon

Unexplained weakness, anaemia Occult blood in stools Right side and dyspeptic symptoms also More common in women About 10-20% Ulcerative/proliferative lesion Colonoscopy Obstruction-less common

Change in bowel habits Gross blood in stools Constipation and obstruction More common in men About 60-70% (including rectum) Strictures (rectosigmoid) Flexible sigmoidoscopy Obstruction, perforation, pericolic abscess-common

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longitudinally. Once serosa is involved, spread occurs rapidly into neighbouring structures such as ureter, bladder, uterus, etc. The involvement of these structures is not a contraindication for surgery (TNM staging). • Local perforation may result in pericolic abscess. • Hollow viscus perforation results in internal fistula. 2. Lymphatic spread (see page 702) 3. Blood spread: It occurs late, resulting in secondaries in the lungs, liver, etc. Cannon ball in lung nodule in the liver. PEARLS OF

WISDOM

Because of the drainage into the portal system, colonic cancers spread to the liverfirst. On the other hand, rectal cancers spread to the lungs because of drainage into inferior vena cava. STAGING/CLASSIFICATIONS There are many classifications and staging for carcinoma colon/rectum. They are not important. A few important ones have been given here. I. Dukes' staging for colorectal cancer • Stage A: Invasion of but not breaching the muscularis propria. • Stage B: Breaching the muscularis propria but not involving the lymph nodes. • Stage C: Lymph nodes are involved Few authors describe a stage D for metastatic disease. Since it has not been described by Duke, it is called modified Dukes' staging. II : Astler-Coller's modification of Dukes' staging Stages (Fig. 29.29) A Limited to mucosa-no nodes B1 Extension into muscularis propria-no nodes B2 Extension into entire bowel wall-no nodes B3 Extension into adjacent organs-no nodes C l Extension into muscularis propria-positive nodes C2 B2 + Lymph nodes C3 B3 + Lymph nodes D Distant metastasis Ill. WHO classification-it is based on histology • Majority are adenocarcinoma-90% • Mucinous adenocarcinoma-5-10% • Signet ring cell carcinoma • Small cell carcinoma • Squamous cell carcinoma • Undifferentiated carcinoma IV. TNM staging (see text on the right side) A few clinical photograph, staging pictures and operative pictures have been given in next page.

I

TNM STAGING OF COLORECTAL CANCER Tumour-T Tx-Primary tumour cannot be assessed TO-No evidence of tumour Tis-Carcioma in silu-intraepithelial/invasion into lamina propria Tl-Invasion into submucosa T2-Invasion into muscularis propria T3-lnvasion into pericolorectal tissues/fat T4a-lnvasion into surface of the visceral peritoneum T4b-Direct invasion or adherent to adjacent structures/organs Regional nodes-N Nx-Nodes cannot be assessed NO-No nodal spread N I-Regional nodes 1-3 involved -Nla-1 regional node -NI b-2 to 3 regional nodes - Nle-Tumour deposits in serosa/mesentery/non-peritonealised pericolic or perirectal tissue without regional nodes N2a-Regional nodes 4 or more involved - N2a--4-6 regional nodes - N2b-7 or more regional nodes Distant metastases M MO No distant spread Ml Distant spread present Spread confined to one organ or site-liver/lung/ovary/ Mia nonregional nodes Spread to more than one organ or site/peritoneum Mlb Histological grade G Gx-Grade cannot be assessed GI-Well-differentiated G2-Moderately differentiated G3-Poorly differentiated G4-Undi fferentiated Residual tumour R R O-No residual tumour after resection RI-Microscopic residual tumour after resection R 2-Macroscopic residual tumour after resection Stage Group 0-Tis NO MO I-Tl NO MO; T2 NO MO IIA-T3 NO MO IIB-T4a N O MO IIC-T4b NO MO IIIA-Tl-2 NI-le MO; Tl N2a MO IIIB-T3-4a NI-le MO; T2-3 N2a MO; Tl-2 N2b MO 11IC-T4a N2a MO;T3-4a N2b MO;T4b Nl-2 MO IVA-Any T any N Mia IVB-Any T any N MI b V O-No venous invasion;VI-presence of venous invasion LO-No lymphatic vessel invasion; Lt-presence of lymphatic vessel invasion

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Fig. 29.22 Fig. 29.22: A 34-year-old lady presented to the hospital with mass in the right iliac fossa with slight flexion of the right hip. Mass was hard and irregular. She also had anaemia Fig. 29.23: Exploration of the mass (caecum). It was mobile Fig. 29.24: Right hemicolec­ tomy specimen-a case of carcinoma caecum

Fig. 29.28: Intestinal obstruction due to rectosigmoid stricture­ a common complication. Tumour rarely goes beyond 2 cm from the edge of the tumour unless there is concomitant spread to lymph nodes A

81

Mucosa Fig. 29.25: Carcinoma hepatic flexure with partial obstruction

Fig. 29.26: Extended right hemi­ colectomy specimen

---, Muscularis i--�---t---t-----t-----1---mucosae Submucosa Muscularis propria Subserosa Serosa

Fig. 29.29: Astler-Coller staging

Fig. 29.27: Rectosigmoid stricture. Patient underwent high ante­ rior resection. 5 cm proximal margin is enough for radical cure

Fig. 29.30: Limited colectomy leak-managed by refashioning of the stoma followed by lleo­ stomy

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Complications (Fig. 29.30) 1. Intestinal obstruction 2. Pericolic abscess: Pain is present in the tumour site and may radiate to back, leg or hip as in caecal perforations. It is due to irritation of the psoas muscles or due to irritation of femoral nerve. • Diagnosis is confirmed by ultrasound/CT scan. • Percutaneous aspiration, followed by elective resec­ tion is the best treatment. 3. Faecal fistula (Fig. 29.31) • Pericolic abscess when it is incised or drained to the exterior may result in faecal fistula if there is malignancy. • Carcinoma caecum may Fig. 29.31: Carcinoma result in appendicitis colon perforated-pericolic abscess which on draining and appendicectomy may invariably result in faecal resulted in faecal fistula fistula. 4. Internal fistula: Colovesical, colocolic, coloenteric are not uncommon complications of malignancies. They are managed by resection. However, preoperative assessment of fistulae by investigations should be done. PEARLS OF

PEARLS OF

Occult blood in the stools may be thefinding which gives a 'clue' in many cases of 'anaemia for evaluation'.

3. Double contrast barium enema may show irregular filling defect-intrinsic, persistent. It may also show an apple core deformity (Figs 29.33 and 29.34 and Key Box 29.10).

.,............ Fig. 29.33: Barium enema showing apple core deformity

WISDOM

Involvement of local structures is not a contraindication for radical resection.

Routine tests/general fitness 1. Complete blood count • Hb%: Low indicating anaemia • TC, DC: If it is high, it indicates perforation. pericolic abscess • ESR: May be increased 2. Stool: Occult blood positive 3. Liver function tests, renal function tests and blood sugar estimation 4. Cardiac ECHO/ECG for fitness before surgery

Fig. 29.34: Intrinsic, irregular, persistent filling defect

�

BARIUM ENEMA

• Gives good anatomic and topographic information.

Investigations (Fig. 29.32) I. Complete blood picture-demonstrates low Hbo/o 2. Occult blood in stools

J

WISDOM

• Can detect associated diverticular disease. • Small ulcerative lesions can also be diagnosed.

Carcinoma colon-investigations

J

I

Diagnostic tests 1. Ultrasound: Simple, baseline noninvasive investigation • Can pick up 'colonic mass' • Can detect liver metastasis • Can demonstrate ascites, para-aortic nodes 2. CT scan • Objective, more precise about mass, infiltration to vascular pedicles, lymph nodes. ureters 3. Colonoscopy • Invasive but diagnostic and Gold standard investigation • Biopsy should be taken for final confirmation

Fig. 29.32: Carcinoma colon-investigation

I

Metastatic workupinvestigations to know spread 1. Chest X-ray to look for cannon ball secondaries 2. CEA: Gross elevation may suggest advanced stage 3. CT is also done to know local and distant spread 4. PET scan: In follow-up of cases of carcinoma colon. If CEA levels start increasing

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CARCINO-EMBRYONIC ANTIGEN-CEA

Figs 29.35A and B: Colonoscopy shows growth and biopsy is being taken (Courtesy: Dr Filipe Alvares, Consultant, Medical Gastroenterologist, KMC, Manipal}

4. Flexible sigmoidoscopy: 60 cm of colon can be visualised. It is an outpatient procedure. It is indicated in rectal bleeding. An enema is given before the procedure. 5. Colonoscopy is done to take a biopsy from growth and also to rule out synchronous malignancy as seen in 5% of the cases (more than one malignancy at the time of diagnosis). If biopsy cannot be taken, as in obstruction, brush cytology can be taken (Fig. 29.35). • Small risk of perforation is present and it is invasive procedure. • Virtual colonoscopy can pick up polyp of 6 mm size also but biopsy cannot be taken. 6. Ultrasound: It is the baseline investigation to be done first. • It can detect colonic mass • It can detect hydronephrosis, liver metastasis, ascites, para­ aortic nodes. • Ultrasound guided biopsy is possible in advanced cases. • Definitely it cannot pick up early mucosal or lesions which have penetrated up to muscularis mucosa or serosa. 7. CT scan in carcinoma colon • Other than a biopsy, it has all the advantages and it is the investigation of choice after colonoscopy. • Anatomical location of the tumour, involvement of serosa • Infiltration of local adjacent structures like ureter (right side)-preoperative stenting is required in such cases. • Metastasis in the liver, ascites, para-aortic nodes. • Other associated diseases specially in elderly such as aortic aneurysm, gall stones, hiatus hernia, etc. • 90% and 95% sensitivity and specificity in detecting liver lesions greater than 1 cm. 8. Carcino-embryonic antigen (CEA) (Key Box 29.11). • It is a foetal glycoprotein, not present in normal human beings (minute quantities). It is present in the cell membranes of many tissues including colorectal cancer. • It is present in the last trimester in the foetus. • It has a prognostic rather than a diagnostic value. After treatment of the primary, CEA level should come back to nornrnl. If it is increased, it suggests either recurrent tumour or secondaries in the liver.

• Discovered by Gold and Freedman • It is a surface glycoprotein • Produced by colorectal epithelium but cleared by Kupffer cells of liver. Its half-life is prolonged (normal-10 days) in cholestasis and hepatocellular dysfunction. • Normal levels: 0-4 mg/ml • Significant increase in the levels is also found in pancreatic carcinoma, gastric carcinoma, lung carcinoma, breast carcinoma. • Increased CEA in the follow-up period of colorectal cancer suggests metastasis. PET scan may help in these patients when CT/US are normal. • It has very low sensitivity • If preoperative CEA is increased in node-negative colonic cancer, chemotherapy is recommended. 9. Role of PET scan • Routine use of PET-positron emission tomographic scanning in the primary management of colorectal cancer is not recommended. • It is useful in the follow-up cases wherein CEA levels are increasing and the actual cause for the rise is being evaluated. • Chest X-ray: Colonic carcinoma spreads more often to lungs than carcinoma of stomach giving rise to cannon ball secondaries. Prognostic factors of carcinoma colon • Spread: If it is limited to mucosa and there are no nodes, 5-year survival is 90-100%. • Age: Younger patients have poor prognosis. • Grade: Poorly differentiated tumours have worse prognosis. • Obstruction and perforation: Poor prognosis due to dissemination of malignant cells. • Blood transfusion: Perioperative blood transfusion has poor prognosis. PEARLS OF

WISDOM

Blood transfusion increases number of suppressor T-lymphocytes thus causing immunosuppression. Hence, follow bloodless surge,y or autologous blood transfusion.

Preoperative preparation 1. Mechanical bowel preparation is necessary to reduce the bacterial load in the colon. This reduces incidence of anastomotic leakage. • Whole gut irrigation by oral polyethylene glycol is found to be superior than enemas. It is the method of choice today. It is mixed with 2 litres of water and is given 12 hours before surgery.

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2. Antibiotics: Oral antibiotics neomycin/metronidazole or neomycin/erythromycin are given in the afternoon and evening before surgery. They act locally and are given mainly to decrease wound infection. Intravenous antibiotics such as ciprofloxacin and metro­ nidazole or 3rd generation cephalosporins such as ceftria­ xone is given one hour before the surgery. The target bacteria are E. coli and Bacillus fragilis. 3. Improvement of general condition by correcting albumin levels and if necessary, TPN will definitely decrease the incidence of anastomotic leakage. 4. A fat-free diet, low residue diet is prescribed two to three days before surgery. 5. Prophylactic fractionated heparin enoxaparin or dalteparin is given subcutaneously, to prevent deep vein thrombosis. Otherwise subcutaneous heparin is given. Principles of surgery Colonic surgery is a very common problem encountered in the practice or institution. Every surgeon should be aware of basic principles of colonic surgery and technique of anastomosis. I have grouped them together under the heading of ten commandments so as to understand better. Surgery Over a period of years, radical resections for carcinoma of colon have become less radical pertaining to the extent of bowel resection. For example: One need not remove terminal 30 cm of ileum for carcinoma caecum today. Only 6-8 cm of ileum removal is sufficient in a right hemicolectomy.

Different types of surgery I. Carcinoma right colon including caecum: If it is operable, the treatment is right radical hemicolectomy. Structures removed in this operation are (Fig. 29.36 and Key Box 29.12). • Terminal 6-8 cm of ileum 1 • Caecum, appendix and ascending colon • One-third of transverse colon • Fat, fascia, lymphatics and lymph nodes like ileocolic nodes, pericolic nodes, nodes at the original of SMA. • If the growth is fixed to posterior abdominal wall, common iliac vessels, palliative ileotransverse anastomosis is done (ileotransverse colostomy). 2. Carcinoma transverse colon-'V' resection. The area supplied by middle colic artery is removed followed by end to end anastomosis. The patient may need removal of entire transverse colon depending upon lesion. • When lesion is at hepatic flexure or in the transverse colon, extended right hemicolectomy should be done. 3. Carcinoma left colon-left radical hemicolectomy. • Left half of the transverse colon and descending colon are removed followed by anastomosis of transverse colon to sigmoid colon-This is the area supplied by left colic artery (Fig. 29.37).

MM.............� STRUCTURES THAT CAN GET INJURED DURING RIGHT HEMICOLECTOMY • Duodenum • Ureter • Gonadal vessels

TEN COMMANDMENTS OF SURGERY FOR CARCINOMA COLON-OPEN SURGERY 1. Should mark the ostomy site preoperatively-in cases of emergency colectomy. 2. Should give an adequate incision. 3. Should explore the peritoneal cavity for metastasis. 4. Should remove the growth with at least 7 cm margin, with all groups of regional nodes, fat fascia and lymphatics called en block resection-R-0 resection. 5. Should do the resection without touching or handling the tumour-follow no touch technique of Turnbull. 6. Should divide the vascular pedicle first and should ligate the vessels as high at the origin-High tie. 7. Should ensure the cut ends bleed well before anastomosis. 8. Should ensure there is no tension at the suture line. 9. Should do one stage procedure in all elective cases­ resection and anastomosis. 10. Should consider temporary ileostomy after resection anastomosis in obstructed colon cancer.

Fig. 29.36: Right radical hemicolectomy 1 Removal of a foot of ileum is not necessary unless it has a doubtful vascularity after ligation of pedicles.

Large Intestine

Fig. 29.37: Left radical hemicolectomy including removal of the spleen as the growth was infiltrating the spleen-routine removal of spleen should not be done

4. Carcinoma sigmoid colon-radical sigmoid colectomy

followed by anastomosis of descending colon to the rectum (colorectal anastomosis). Or in a few cases, left hemico­ lectomy may have to be done. 5. Left-sided colonic tumours with intestinal obstruction­ an emergency temporary transverse colostomy is done to divert the faecal matter and to relieve intestinal obstruction (for more details on colostomy see page 785). Resection is not done because many patients are elderly with comorbid illness such as diabetes, hypertension, cardiac illness. • General condition of the patient is poor with gross abdominal distension and dehydration. • Left colon is loaded with faecal matter. Hence, high chances of anastomotic leakage and faecal peritonitis are present. • After 2 weeks, laparotomy is done once again. The primary tumour is resected and end-to-end anastomosis done. • This is followed by closure of the colostomy 8 weeks later-three-stage operation. • Single-stage resection can also be done provided, thorough colonic irrigation through appendicular stump (after appendicectomy), is given and it should be successful in cleaning the entire colon. This is the concept of on-table irrigation and lavage. ON-TABLE IRRIGATION AND LAVAGE (Fig. 29.38) Indication In cases of left-sided colonic obstructions-classical example being carcinoma rectosigmoid with obstruction. Procedure • Resection is done first. Clamps are applied to both ends. • Appendicectomy is done and purse string suture applied but not tied. • Through the appendicular stump lumen, a 30 Fr Foley catheter is passed into colon.

717

Fig. 29.38: 'On-table' irrigation through appendicular stump

• The position of the catheter is checked to be lying safely in the caecum and its balloon is inflated. A purse string suture, previously applied to the appendix base is tied. • Saline is irrigated at the rate of 50 to 100 ml per hour speed. Proximal clamp is opened into a container (kidney tray). • It takes about an hour or so for the whole gut irrigation. • This is done till the returning fluid is clear. Advantage It avoids a stoma, decreases stay in the hospital and thus less expensive. Caecostomy tube Once anastomosis is completed, Foley catheter is brought out through an opening in the abdominal wall and connected to a bag. It is sutured to the inside of the parietes by vicryl sutures and kept open. Tube removal Tube is removed after 7-10 days provided there is no leak from the suture line. ENHANCED RECOVERY PROGRAMME These are the various changes in the management of these patients over conventional methods. A few are given below which will help the patient recover fast and get discharged­ no quality compromise. • Nasogastric tube is not required unless operating on obstructed colons. Evidence is that the sutures /staplers are good enough to prevent the leaks. • Improve the nutrition preoperatively by increased intake of carbohydrates-oral or TPN. • Laparoscopy has the definite advantage of small incisions, less pain, easy breathing and less respiratory complications. • Early mobilization, early feeds, early recovery and early removal of catheters. • Epidural catheters to relieve pain. • Few diagrammatic representation of the colectomies are shown in the next page (Figs 29.39 to 29.44).

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SURGERIES IN A CASE OF CARCINOMA COLON (Figs 29.39 to 29.44 and Table 29.4)

Figs 29.39A and B: Right hemicolectomy followed by end-to-end anastomosis-ileocolic

Figs 29.40A and B: Palliative ileotransverse anastomosis inoperable case

B

Figs 29.41A and B: Extended right hemicolectomy-growth in the hepatic flexure

Figs 29.42A and B: Transverse colectomy followed by colocolic anastomosis

B

Figs 29.43A and B: Left hemicolectomy for growth in the splenic flexure

B

Figs 29.44A and B: Resection of rectosigmoid growth followed by colorectal anastomosis

Figs 29.39 to 29.44: All figures are contributed by Ms Vidushi Kapil,

MBBS

student, KMC, Manipal

719

Large Intestine Summary of the resections for carcinoma colon Site of the tumour Name of surgery

Part of the bowel resected

Artery supplying

Safe margin

Caecum

Right hemicolectomy

Terminal ileum to midtransverse colon

Ileocolic, right colic RJGHT branch of middle colic

5 cm

Ascending colon

Right hemicolectorny

Terminal ileum to midtransverse colon

Ileocolic, right colic RJGHT branch of middle colic

5 cm

Hepatic flexure

Extended right bernicolectomy

Terminal ileum to descending colon

Ileocolic, right colic MIDDLE colic

5 cm

Transverse colon

Extended right hernicolectomy

Terminal ileum to descending colon

Ileocolic RIGHT colic MIDDLE colic

5 cm

Splenic flexure

Extended left hemicolectomy

Right flexure to rectosigmoid

Middle colic LEFT colic IMA

5 cm

Descending colon

Left hemicolectorny

Left flexure to sigmoid

IMA LEFT branch of middle colic

5 cm

POSTOPERATIVE CHEMOTHERAPY l. pTl-2NOMO do not require any adjuvant treatment, such patients can be kept on follow-up with routine 3 monthly CEA and annual CECT thorax/abdomen/pelvis. 2. pT3NOMO or node positive disease requires adjuvant treatment in the form ofconcurrent chemoradiotherapy and chemotherapy. 2 cylces of FOLFOX (5-FU + Leucovorin + Oxaliplatin) �Concurrent 5-FU/Leucovorin and radiation � 2 more cycles of FOLFOX. Oral Capecitabine can be used in place ofIV 5-FU. 3. It is preferable to add Oxaliplatin in the chemotherapy regimen ifnodes are positive for metastatic disease. In older population(> 65-70 years), it might of less benefit. 4. Oxaloplatins have been shown to down size liver metastasis. Chief complications of Oxaliplatins is peripheral neuropathy. • Indications for chemotherapy has been given in Key Box 29.13.

11$111�, INDICATIONS FOR CHEMOTHERAPY

• All node positive patients • In node negative patients if - T4 lesions are involving free mesothelial surface - Major microscopic vein involvement - Signet cell carcinoma - High preoperative CEA - Aneuploidy on flow cytometry - Microsatellite instability

Postoperative radiotherapy Adenocarcinoma colon does not respond well to radiation. Routinely it is not given. Surgery remains the gold standard

for carcinoma colon. Soft tissue infiltration into psoas muscle or abdominal wall or inoperable recurrent tumours are indications for radiotherapy. Metastatic disease without obstruction Patients with isolated liver/lung secondaries should also undergo treatment with a radical approach as even in these cases with resection of the primary and adequate liver/lung resection, a good disease control can be achieved. 1. A typical course of neo-adjuvant therapy comprises concurrent 5-FU/Capecitabine and radiation in cases of large lesions abutting the abdominal wall or down into the pelvis. A dose of 45-50 Gy is used to treat the pelvis including the growth and the draining lymphatic regions followed by 5 Gy boost to the tumour itself. 2. Following neo-adjuvant therapy, patient should be re­ evaluated using CT/MRI for possibility of resection. 3. Surgery is usually considered after 6-8 weeks following neo-adjuvant therapy as the maximal response to the treatment may take up to 2 months. 4. Further adjuvant treatment is to be given following surgery depending upon the histopathological report. Management of liver secondary • CT scan and PET scan are done to evaluate local/systemic disease. Provided there is no systemic spread, liver secon­ daries have to be treated aggressively. Pattern ofrecurrence in colonic carcinomas is more commonly distant, that is they tend to recur more commonly at distant sites such as liver, and lungs. As a result, systemic treatment is more necessary. • Liver directed therapies such as hepatic arterial chemo­ therapy infusion/embolisation, radiofrequency-ablation, radiotherapy should be used in treahnent of isolated liver metastasis. • Indications for liver resection is given in Key Box 29.14.

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INDICATION FOR RESECTION OF LIVER METASTASIS

• Solitary metastasis or metastasis confined to one lobe. • < 3 metastasis in both lobes • Absence of extrahepatic disease Novel agents in colorectal cancers I. Bevacizumab-anti-VEGF (vascular endothelial growth factor) monoclonal antibody. It has anti-angiogenesis property thereby controlling the tumour growth. 2. Cetuximab anti-EGFR (epidermal growth factor) monoclonal antibody. Follow-up (Key Box 29.15) Most of the colonic cases are curable if diagnosed and treated early. Also metachronous lesion can occur in the rest of the colon. Hence, certain tests are necessary during follow-up.

2. Dietary fibres: Fibres decrease the transit time, they dilut1 the carcinogens and are used to present development o cancer. Cellulose, hemicelluloses and pectin are a fe� examples. Fibres also produce short chain in fatty acid: causing fermentation by faecal flora. Thus colonic pl­ becomes more acidic which in turn inhibits carcinogenesis 3. Aspirin, calcium, and sulindac also have been used tc prevent cancer developing in an adenoma. COLON SCREENING • Large bowel is the 4th most common site for cancer after lung, stomach and breast • More common in North America, North Europe and Australia. Lowest rates in Africa, India. • 75% of CRC develop in people with no known risk factors apart from older age.

Treatment of recurrent or metastatic cancer • Recun-ence or metastasis is suspected during follow-up by abnormal values of investigation. • Recurrent tumour should be resected en bloc-it may amount to a more radical procedure including resection of duodenum, liver, kidney (Figs 29.45 and 29.46) • Metastasis in the liver (Key Box 29.14).

............................. . KEY BOX 29.15

FOLLOW-UP OF COLORECTAL CANCER Tests

1. Haemo-occult 2. Colonoscopy

Duration

Once in 3-6 months 6 months after surgery, later once in a year 3. Alkaline phosphatase Once in 3-6 months for 3 years 4. CEA Once in 6 months 5. Chest X-ray Yearly

In years

3 3 3 3

CHEMOPREVENTION OF COLONIC CANCER I. Folic acid: It is an important vitamin with many functions. In the absence of folic acid, hypomethylation can occur. As a result of this overexpression of proto-oncogenes such as K-ras and c-Myc can occur. Deficiency of folic acid causes imbalances in the nucleotide pool leading to DNA break and mutation. Thusfolic acid supplementation should be given in adenoma specially when baseline levels offolic acid is low.

Figs 29.45 and 29.46: A colorectal cancer patient who was

operated 3 years back for carcinoma caecum presented with recurrence in the abdominal wall. He underwent excision of the tumour. He did not have any other site of metastasis. Postoperative radiotherapy was given

Large Intestine

Screening options 1. Faecal occult blood test (FOBT) • It is guaiac test which will detect elevated level of blood in stool. It requires 2 samples from each of 3 consecutive stools which are smeared onto cards. • False positive: Vegetables, fruits, red meat, aspirin or any other bleeding lesion proximal to colon screening, FOBT has shown to decrease mortality by 20 to 30%. 2. Flexible sigmoidoscopy (FS) • Reduces incidence and mortality ofdistal CRC by around 60%. • Single FS at the age of 60 is recommended in UK. • In US, 5-yearly screening is being done. 3. Colonoscope screening • Should be done if there is distal adenoma (chances of proximal adenoma are high). • 70% ofall advanced colorectal neoplasia will be detected with this strategy. • Procedure is painful, requires sedation and analgesics. • Chances of perforation are 1 in 500 to 2000 cases. • It requires skills of an experienced endoscopist. 4. Virtual colonoscopy • It is an alternative but not yet become popular because of time, cost and preparation. • It is done with the help of CT scan. • Biopsy cannot be taken DIVERTICULAR DISEASE OF COLON It is an acquired condition, in which colonic mucosa herniates through the circular muscle fibres at weak points, where blood vessels penetrate the colonic wall. Since it is acquired it lacks the muscle coat. They are thin, more prone for infections and perforation. Hence, they are termed pseudodiverticuli. Aetiopathogenesis • The disease is common in western population wherein diet is very poor in fibres because ofrefining ofsugar and flour. Nonstarch polysaccharides (NSP) or low dietary fibres are the chief factor for diverticulosis of colon (Key Box 29.16). • It requires very high pressure for propulsion offaecal matter and this is believed to cause characteristic thickening of muscles and herniation of mucosa (Figs 29.47 and 29.48). • In Africans and Indians, the disease is rare because ofhigh fibre content of the diet. • The disease starts after the age of40. Any stress or emotional disorders may add to the constipation already caused by dietary factors and result in diverticular formation. • 90% of them affect sigmoid colon. Rectum is spared in majority. Rarely, it affects right colon.

721 True diverticulum

Pseudodiverticulum

Through the muscle Examples: Meckel's normal appendix

Examples: Zenker's oesophageal common colon "ticks"

Figs 29.47 and 29.48: Diverticulum

• Diverticulae project between antimesenteric and mesenteric borders with taenia but they never penetrate taenia (Fig. 29.49). • There is muscle hypertrophy, which project into the lumen as obstructive folds. The mucosa is essentially normal. Slowly, luminal diameter is narrowed. • Inflammation occurs in the pericolonic tissue with or without abscess formation.

NSP AND DISEASES • Diverticular disease • Obesity and diabetes mellitus • Constipation, and piles • Breast cancer and colonic cancer

Structural changes in colonic wall of patients with diverticulosis • Mycosis • Thickening (neither hypertrophy nor hyperplasia) of the circular muscle layer. • Shortening of the taenia coli • Luminal narrowing • i elastin deposition in taenia coli • i type III collagen synthesis • i collagen cross-linking Segmentation • Law of Laplace: Pressure = K x Tension/Radius • Sigmoid colon has small diameter resulting in highest pressure zone. • Segmentation = motility process in which the segmental muscular contractions separate the lumen into chambers. • Segmentation �increased intraluminal pressure� mucosa! herniation �diverticulosis.

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Fig. 29.49: Sigmoid diverticulae

• May explain why high fibre prevents diverticuli by creating a larger diameter colon and less vigorous segmentation. • Collagen-connective-tissue diseases such as Ehlers­ Danlos syndrome, Marfan's syndrome, and autosomal­ dominant polycystic kidney disease result in structural changes in the bowel wall, leading to decreased resistance of the wall to intraluminal pressures and thus allowing protrusion of diverticula.

Clinical features 1. Diverticulosis: It refers to presence of diverticulosis without much symptoms. But on careful questioning, patients do have lower abdominal distention, heaviness, flatulence, etc. Vague abdominal pain is also felt in the left iliac fossa. 2. Diverticulitis: Left-sided lower abdominal pain, moderate to severe is associated with passage of loose stools. The pain is partially relieved on passing flatus. • Bleeding per rectum can be the presenting feature, sometimes it can be massive. • Low-grade fever, tenderness, rigidity and even mass may be present in the left iliac fossa (like left-sided appendi­ citis). The mass is thickened, inflamed, tender and sigmoid. Such attacks result in abscess which rupture into hollow organs and give rise to fistulae (Fig. 29.50).

Fig. 29.50: Perforated diverticula at surgery-resected specimen (Courtesy: Dr Ramesh Rajan, Surgical Gastroenterologist, Trivandrum Medical College, Kerala)

3. Internal fistulae: Colovesical fistulae give rise to pneumaturia (flatus in the urine) and rarely faeces in the urine (Key Box 29.17).

MH..........,.� CAUSES OF INTERNAL FISTULAE

• Diverticular disease of colon • Carcinoma of colon • Crohn's disease • Radiation • Tuberculosis For summary of diverticular disease see Fig. 29.51

Classification/staging system Hinchey classification (Fig. 29.52) I. Pericolic abscess IL Walled off 3 pelvic abscess III. Generalised purulent peritonitis JV. Generalised faecal peritonitis

Symptoms

Diverticulitis

Diverticulosis • Bloating • Heaviness • Flatulence •Abdominal distension

Perforation

Generalised Abscess

Peritonitis

Bleeding •Anaemia • Massive

Internal fistula • Colovesical • Colovaginal

Low grade Stricture

Diverticulosis of the colon is one of the common causes of massive lower GI tract bleeding specially in Western patients

Fig. 29.51: Summary of symptoms of diverticular disease

723

Large Intestine

·I! II

Fig. 29.53: Colovesical fistula (arrow)

IV

Ill

Fig. 29.52: Hinchey classification

Hinchey stages I and II may be treated by sigmoid colectomy and primary anastomosis (a one-stage operation). Hinchey stages III and IV are treated by sigmoid colectomy followed by end-colostomy and Hartmann pouch. PEARLS OF

WISDOM

The most common fistula in acute diverticulitis is colovesical followed by colovaginal fistula.

Investigations 1. Sigmoidoscopy: Mucosa may be normal or may show erythematous and oedematous changes. Ulcers are absent. Opening of diverticulae can be seen. 2. Barium enema: Contraindicated in acute cases. • It may show saw-tooth appearance due to muscle hypertrophy. • It may show a long stricture • Champagne glass sign: Partial filling of diverticula by barium with stercolith inside the diverticula. 3. Colonoscopy to confirm the findings and to rule out carcinoma colon (Figs 29.53 and 29.54). PEARLS OF

WISDOM

Sigmoidoscopy, colonoscopy and barium enema are contraindicated in acute diverticulitis.

Fig. 29.54: Colonoscopy showing opening of diverticula

4. Ultrasound and CT scan are the investigations of choice in acute diverticulitis (Fig. 29.55). COMPLICATIONS 1. Massive haemorrhage per rectum: Haemorrhage is due to vessels in the base of diverticulae, more so in atherosclerotic or hypertensive patients.

CT SCAN ACUTE DIVERTICULITIS • • • •

Can detect thick muscular folds-confirms the diagnosis. Detects an abscess and can confirm complication. Detects extraluminal air or contrast-confirms perforation. Can rule out other causes-acute pancreatitis with pericolic collection, etc. • It is the investigation of choice in acute diverticulitis. • Thickened colonic wall > 4 mm • Pelvic abscess can be diagnosed

Manipal Manual of Surgery

724

TREATMENT

Fig. 29.55: CT scan showing pericolic abscess on the left paracolic

space

2. Stricture of sigmoid colon can develop due to recurrent attacks resulting in intestinal obstruction. 3. Perforation may result in peritonitis, pericolic abscess or pelvic abscess (Fig. 29.56 and Key Box 29.19). 4. Fistula formation: Internal fistulae occur due to inflammatory adhesions and abscess formation which ruptures resulting in fistula. Thus, colovesical, colovaginal, colointestinal fistula can occur.

1. Stage of diverticulosis or in those patients who have recovered from one attack of diverticulitis. • High residue diet • Fruits and vegetables • Whole meal bread and flour • Bulk purgative To avoid constipation Diet • High fibre diet, optimal amount of daily fibre is unknown. • 20 to 30 g per day is a widely recommended. • Recommendation to avoid seeds, nuts and popcorn. 2. Acute diverticulitis with pericolic abscess • Rest, hospitalisation, correct hydration • IV antibiotics: Bactericidal against gram -ve and anaerobes. • Abscess is aspirated under ultrasound guidance. • After 4-6 weeks, elective sigmoid colectomy and anastomosis is done. 3. Diverticulitis with peritonitis A. Hartmann's procedure is the choice: Sigmoid colon is resected, end-colostomy is done by using descending colon followed by closure of rectal stump (Figs 29.57A and B). • After 4-6 weeks, colorectal anastomosis is done. B. However, if a perforation is small and the general condition is good after the resection, the colon is irrigated with 8-10 litres of saline till the contents are clear. This is followed by colorectal anastomosis in the same sitting.

............................. . KEY BOX 29.19

PERICOLIC ABSCESS

Fig. 29.56: Pericolic abscess

Differential diagnosis • Carcinoma of the colon • Inflammatory bowel disease • Ischaemic colitis • Irritable bowel syndrome • Pelvic inflammatory disease Indications for surgery 1. Failure to respond to conservative/dietary advice 2. 2 attacks of diverticulitis 3. Complications

• Abscess depends on the ability of the pericolic tissues to localise the spread of the inflammatory process. • Intra-abdominal abscesses are formed by-anastomotic leakage: 35%-diverticular disease: 23%. • Limited spread of the perforation forms an inflammatory phlegmon, while further (but still localised) progression creates an abscess. • Signs and symptoms: High grade fever with or without leukocytosis despite adequate antibiotics, tender mass. • Treatment: A. Small pericolic abscess: 90% will respond to antibiotics and conservative management alone. Often it is retrocolic. May present as swelling in the loin. B. Percutaneous abscess drainage (PAD) is the treatment of choice for small, simple, well-defined collections. 100% success in simple unilocular abscesses. C. Open drainage: In cases of multilocular collection, abscesses associated with enteric fistulas, and abscesses containing solid material. Drainage of pus, resection, Hartmann's operation is ideal choice. Closure of the colostomy after 6-8 weeks is done.

Large Intestine

• A

B Figs 29.57A and B: Hartmann's procedure

4. Treatment of fistulae: As an elective procedure, with good preparation, after confirming the site of fistula, resection of the sigmoid colon with closure of fistula can be done.

• • • •

TYPES OF FISTULA RELATED TO DIVERTICULAR DISEASE Colovesical: 65% Colovaginal: 25% Colocutaneous Coloentero

MISCELLANEOUS FAECAL FISTULA Classification (Key Box 29.20) Depending upon the nature of the disease or a surgical procedure. I. Primary or type I fistula: It develops as a result of an underlying disease affecting gut. Example: Carcinoma colon infiltrating urinary bladder resulting in colovesical fistula or rupture through the skin resulting in pericolic abscess, fistula. II. Secondary or type II fistula: Occurs after injury to otherwise normal gut. Examples: Left colonic/right colonic injury in percutaneous nephrolithotomy (PCNL) for renal stones or following anastomotic leak. Depending upon the site of the fistula A. Lateral fistula: It occurs after a colocolic or ileocolic anastomosis wherein a few sutures would have given way in the immediate postoperative period. B. End fistula: This means both ends of the intestines are open following resection and anastomosis giving rise to postoperative peritonitis.

725

Bacterial flora • More than 99% of faecal bacterial flora are anaerobic. • Most common anaerobe is Bacteroides fragilis, with a count of 10 10 /g of wet faeces. Clostridia, Lactobacillus arE other organisms. • E. coli is the predominant aerobic organism. Count is l O 7lg of faeces. Klebsiella, Proteus, Enterobacter are other aerobes. St rept ococcus faeca lis is the principal enterococcus. Clinical manifestations • Postoperative patient 'not doing well'-prolonged paralytic ileus, faeculent discharge from wound or drainage site, etc. is an indication of faecal fistula. • Features of septic shock: Often patient may not complain of abdominal pain but manifestations can be of renal failure, tachycardia, tachypnoea and hypotension. Diagnosis • Total counts are elevated-it indicates infection. • Creatinine and urea values are high indicating renal failure. • Ultrasound to detect any collection. • CECT can detect intraperitoneal collection, pneumo­ peritoneum, anastomotic leak. However, creatinine should be normal before doing CT scan. Often patients are in sepsis with renal failure. Treatment (Figs 29.58 to 29.60) • Control of infection • Drainage of sepsis-laparotomy, resection and anastomosis, ileostomy or colostomy may be necessary. • Lateral fistula may heal with total parenteral nutrition provided there is no distal obstruction (TPN). • End fistula may require resuturing with or without proximal diversion colostomy/ileostomy. ·

Fig. 29.58: This patient underwent three surgical procedures for treatment of fistula. Initial surgery was right hemicolectomy for ileocaecal tuberculosis. Second surgery was resection and a proximal diversion ileostomy because of the leak and finally after two months, closure of ileostomy was done followed by ileotransverse anastomosis

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Manipal Manual of Surgery

COLONIC STRICTURE

Fig. 29.59: lleostomy following leak in the case mentioned in Fig. 29.58

Causes l . Malignant: Adenocarcinoma colon is the commonest cause of stricture colon (rarely lymphomas, carcinoid). 2. Tuberculosis: Uncommon cause of stricture in the ascending colon. 3. Ischaemic: Uncommon/rare cause-left colon may be affected. 4. [nflammatory bowel disease: Any ulcers, including amoebic, may heal with fibrosis resulting in stricture. 5. Diverticular stricture 6. Radiation stricture 7. Endometriomas: Ectopic endometriosis tissue responds to cyclic hormonal stimulation causing inflammation and fibrosis. Clinical features • Progressive constipation • Change in bowel habits Bleeding per rectum • Features of large bowel obstruction • Mass may/may not be felt Investigation Colonoscopy and biopsy Treament • Single-stage resection and end-to-end anastomosis. • Treatment of the cause WHAT IS NEW IN THIS CHAPTER?/RECENT ADVANCES

• All topics have been updated. • More and more cases of large bowel obstruction are treated by single-stage resection after giving an 'on­ table' lavage. • Role of chemotherapy for carcinoma has been updated.

Fig. 29.60: A case of faecal fistula following ileal resection for

obstruction caused by a band. Following resection anastomosis on the fifth postoperative day air mixed faecal matter started coming out from the wound. CT scan revealed small lateral fistula. The patient responded to conservative treatment with TPN given for fourteen days. Fistula subsided

• Faecal fistula, colonic stricture have been added. • Chemoprevention of colonic cancer is added.

• Enhanced recovery programme has been added. • Ten commandments of colonic resection have been added.

Large Intestine

727

MULTIPLE CHOICE QUESTIONS 1. The following feature is true of adenomatous polyps of the large intestine: A. Most are sessile B. Most can be removed by colonoscopic snaring C. Adenoma smaller than 15 mm in diameter do not carry the risk of malignant potential D. Young people are more likely to have these polyps 2. Common premalignant conditions for colonic cancer include the following except: A. Familial polyposis coli B. Ulcerative colitis C. Adenomatous polyp D. Peutz-Jeghers syndrome 3. Flexion of the hip can be present in: A. Carcinoma splenic flexure B. Carcinoma caecum C. Carcinoma sigmoid colon D. Carcinoma hepatic flexure 4. Abscess in the lateral abdominal wall can be a feature of: A. Acute pancreatitis B. Perforated carcinoma caecum C. Diverticular perforation D. Meckel's diverticular perforation 5. Mechanical bowel preparation is best given using: A. Plenty of saline B. Oral mannitol C. Glycerine enema D. Whole gut irrigation using oral polyethylene glycol 6. Which one of this is true in colonic surgery? A. No touch technique of Turnbull B. Right-sided lesions are treated by colostomy C. Left-sided lesions are treated by resection D. Removal of 30 cm of ileum along with colon 7. Following is true about prognostic factors of carcinoma colon except: A. Elderly patients have poorer prognosis B. Perioperative blood transfusion has poor prognosis C. Survival is good if it is limited to mucosa and there are no nodes D. Obstruction and perforation is associated with poor prognosis 8. Regarding colonoscopic screening, which one of the following is true? A. It is done if there is distal adenoma B. It is painless and easy to perform C. It is not useful for screening of colonic cancer D. Detection of all advanced colorectal neoplasia is only up to 20%

9. Proliferative growth is more common in th{ ----- colon: A. Right B. Left C. Rectosigmoid D. Splenic flexure 10. The 'Gold standard' investigation for detection of colonic cancer is: A. Ultrasound abdomen B. CT scan C. Barium enema D. Colonoscopy and biopsy 11. Following are the features of intestinal tuberculosis except: A. It can be secondary to pulmonary tuberculosis B. Terminal ileum and caecum are commonly involved C. Ulcers are transverse D. Ulcers do not result in structure 12. In which of the following malignancy anaemia is an important method of presentation? A. Malignant melanoma B. Carcinoma breast C. Carcinoma caecum D. Carcinoma pancreas 13. The most common fistula in diverticulitis is: A. Colovesical B. Colovaginal C. Colorectal D. Colocolic 14. Which of the following diverticula is true diverticula? A. Sigmoid diverticuli B. Meckel's diverticuli C. Parabronchial dive1iiculum D. Laryngeal diverticula 15. In hereditary nonpolyposis colorectal cancer, which carcinoma is more often seen? A. Rectum B. Rectosigmoid C. Transverse colon D. Caecum and ascending colon 16. Incidence of malignancy in familial polyposis coli is: A. 10% B. 30% C. 50% D. 100%

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Manipal Manual of Surgery

17. Following ar e true for carcinoembryonic antigen except: A. Jt is a glycoprotein B. It is tumour marker for carcinoma colon C. It should be done in al I cases of carcinoma colon before surgery D. Produced by colorectal epithelium and cleared by kidney 18. Following ar e true for right hemicolectomy for carcinoma caecum except: A. Right one-third of transverse colon is also removed B. Greater omentum should be removed C. Tem1inal 30 cm of the ileum should be removed D. Duodenum can get injured during surgery

19. Indications for postoperative chemotherapy following colectomy for carcinoma include following except: A. Signet ring carcinoma B. Lymph nodes are positive C. Lymphovenous involvement D. Involvement of muscularis propria 20. The most common aerobic organism present in a sigmoid colonic faecal fistula is: A. Clostridia B. Lactobacillus C. Bacteroides fragilis D. E.coli

ANSWERS B

2 D

3 B

4 D

5 D

11 D

12 D

13 A

14 B

15 D

6 A

7 A

16 D

17 D

A

9 A

10 D

18 C

19 D

20 D

8

30 Intestinal Obstruction • • • • • • • •

Sigmoid volvulus Meckel's diverticulum Adhesions and bands Gall stone ileus lntussusception Mesenteric vascular occlusion Hirschsprung's disease Atresia and stenosis

• • • • • • • •

Introduction • Intestinal obstruction is a challenging surgical emergency encountered by general surgeons. This can affect any age group starting from neonate to an old man. It can affect a school going boy, working woman or a man during their peak of life. Sometimes it can be fatal either due to delay in the diagnosis, delay in the treatment or complications related to surgery. Abdomen is a Pandora's box. Sometimes, it is difficult to pinpoint the cause of obstruction. • Ad]:iesions and hernia are the two most common causes of intestinal obstruction. Adhesions are more common than hernias nowadays. Laparoscopic surgery has definitely decreased incidence of adhesions. In Western countries more than 50% cases of intestinal obstruction are due to adhesions and only l 0-15% are due to obstructed hernia (Fig. 30.1). However, students should be able to diagnose intestinal obstruction, resuscitate the patients and refer the patient for further surgical treatment. With the availability of sophisticated investigations such as CT scan, diagnosis can be established in majority of cases before surgery. However, in other cases, 'exploratory laparotomy' will give the diagnosis.

Arrested rotation with bands Volvulus neonatorum Meconium ileus lmperforate anus Food bolus obstruction Paralytic ileus Malrotation and midgut volvulus What is new?/Recent advances

• 20% occur in large bowel • Majority (more than 80%) of small bowel obstructions are benign in nature. In the large bowel, more than 70% of colonic obstruction is due to malignancy-others being inflammatory bowel diseases, ileocaecal tuberculosis, volvulus, etc. CLASSIFICATION I. Depending upon the nature of obstruction (Key Box 30.1) A. Dynamic obstruction/mechanical obstruction (Fig. 30. l ) B. Adynamic obstruction-paralytic ileus o r neurogenic ileus.

Definition When the intestinal contents fail to move distally, it is called intestinal obstruction. It is the most common surgical disorder (emergency) of the intestines. Few important facts about intestinal obstruction: • 80% occur in small bowel

Fig. 30.1: Obstructed incisional hernia ( Courtesy: Dr CG Narasimhan, Senior Consultant Surgeon, Mysore, Karnataka)

729

.,.............

Manipal Manual of Surgery

730

C OMMONLY USED TERMINOLOGY • Mechanical obstruction

�

There is a physical barrier which prevents the abnormal progress of intestinal contents.

• Paralytic ileus

There is no physical barrier but failure of peristalsis to propel intestinal contents due to neurogenic causes.

• Simple obstruction

It refers to obstruction to lumen only (early cases)

• Strangulated obstruction

It refers to obstruction with impairment of blood supply to the gut.

• Closed loop obstruction

In this condition, the intestine is occluded in two places. More chances of gangrene and perforation are present, e.g. volvulus. • Pseudo-obstruction: No mechanical cause.

II. Depending on the blood supply A. Simple obstruction: Blood supply is not seriously impaired. B. Strangulated obstruction: Blood supply is seriously impaired. C. Closed loop obstruction: It means both proximal and distal ends are blocked. This occurs in carcinoma of the right colon with constrictive lesions. If the ileocaecal valve is competent and the obstruction is total, the intra­ luminal pressure within the colon increases. As a result of this, the caecum may perforate. Thus, closed loop obstruction can be dangerous ( Fig. 30.2). Another example is sigmoid volvulus.

I. Depending upon the cause of obstruction A. In the lumen of the gut Gall stones ileus Food bolus obstruction Roundworm mass Foreign body (rare) Meconium ileus B. In the wall of the gut Stricture, e.g. tuberculosis Crohn 's disease Carcinoma Atresia Adhesions C. Outside the wall of the gut Yolvulus, intussusception Congenital bands Meckel's diverticulum with band Obstructed hernia II. Depending upon severity of obstruction A. Acute obstruction: Signs and symptoms appear very early. Usually, it affects small bowel, obstructed hernia, bands. B. Chronic obstruction (e.g. carcinoma colon) affects large bowel (colic comes first, distension later). Diverticular disease also produces chronic obstruc­ tions (Fig. 30.3). C. Acute on chronic obstruction develops in carcinoma colon, wherein an acute obstruction suddenly results due to the accumulation of faecal matter in the proximal bowel (see Fig. 30.4 for various causes of intestinal obstruction). Pathophysiology (Fig. 30.5) • As a result of obstruction, the proximal bowel undergoes hyperperistalsis which is responsible for colicky pain abdomen. The peristalsis may continue for a few days and later the intestine may be paralysed and flaccid. After 3-4 hours, distal to the obstruction, all physiological activities of the bowel are stopped. Intestine becomes contracted, pale and does not exhibit peristalsis. After a few hours, the proximal bowel gets dilated secondary to obstruction. • The causes of distension of intestinal loop are:

Fig. 30.2: Closed loop obstruction in 40% of patients ileocaecal valve is competent

Fig. 30.3: Dilated small intestinal loops in a case of ileal obstruction

A. Gaseous distension • Swallowed air (70%). Because of colic and anxiety, the swallowed air is increased. Oxygen is absorbed and nitrogen remains as it cannot be absorbed. This results in distension. • Diffusion of air from the blood into bowel lumen increases carbon dioxide which diffuses very rapidly. • Gas due to bacterial activity releases H2S, NH3 etc. ,

Intestinal Obstruction

731

Gall stone

� ::-----+--- Carcinoma splenic flexure

Carcinoma hepatic flexure

Incidence of common causes

----+---

Adhesions: 40 to 50% Tuberculosis/Crohn's, Diverticulitis: 15 to 20% • Malignancy: 15% Obstructed hernia: 15% Others: 10-15%

Stricture ileum (TB) -+---- Faecoliths r-1---- Meckel's diverticulum Band ---+---.IF----"1��!11,,;::__, Gall stone ileum Carcinoma caecum (advanced)

--+-----

--1------.-.___:::--"-'7

Obstructed hernia ------=--�-=--.....,.J

COMMON CAUSES OF ILEAL OBSTRUCTION

COMMON CAUSES OF COLONIC OBSTRUCTION

• Adhesions

• Carcinoma colon

• Volvulus

• Obstructed hernia

• Sigmoid volvulus

• lntussusception

• Stricture

• Faecal impaction

• Obstructed hernia

• lntussusception

• Mesenteric ischaemia

• lleocaecal tuberculosis

• Hirschsprung's disease

• Mesenteric vascular occlusion

• Bands

• Anorectal malformations

• Worm ball-in children

• Stricture colon-rare

COMMON CAUSES OF GANGRENE

• Twisting around a band • Necrotising enterocolitis

• lleal atresia-in children

Fig. 30.4: Differential diagnosis of intestinal obstruction-diagrammatic representation

Manipal Manual of Surgery

732

• In doubtful cases of viability, if facilities are available, a test called fluorescein test can be done. 1000 mg of fluorescein is injected into peripheral vein and bowel is inspected under Wood light. If loops are nonviable, resection and anastomosis is done.

OBSTRUCTION Distension Venous compression

Strangulation (Fig. 30.6 and Key Box 30.2)

Congestion and oedema bowel wall turns purple Progressive arterial compromise blackish discolouration Twisting around band

r-�----Gangrene

Proliferation of bacteria Transmigration

Toxins

,____----1 Peritoneal cavity �-Septic shock

Fig. 30.5: Pathophysiology of intestinal obstruction

B. Distension due to fluids • 1500 ml of saliva • 2 litres of gastric juice • 3 litres of intestinal secretions • l litre of bile and pancreatic juice Normally, all this fluid is absorbed in the bowel. In cases of intestinal obstmction, this fluid absorption is delayed. It accumulates in the intestinal loop. Excretion of water and electrolytes into the lumen is also increased. C. Role of nitric oxide Activated neutrophils and macrophages accumulate within the muscular layer of the bowel wall due to dilatation and inflammation of the bowel wall. This damages the secretory and motor processes by release ofreactive proteolytic enzymes and cytokines. Net result is increase in the local release of nitric oxide, itself a potent inhibitor of smooth muscle tone. It further aggravates the intestinal dilatation. D. Role of bacteria • Bacterial colony count increases following obstruction resulting in stasis. From less than 106 in jejunum and from I 0 8 in ileum, counts increase. • Bacterial translocation can occur even in simple obstruc­ tion without strangulation. Thus, bacteria can enter into lymph nodes and into systemic circulation. Abdominal distension, hypovolaernia, renal failure and sepsis set in. In addition to these changes, diaphragm gets elevated, respiration is impaired which result in respiratory complications such as atelectasis and basal pneumonia.

• Interference with blood supply: As the tension within the loops becomes more and more, venous congestion takes place resulting in oedema of the bowel wall. • If the obstruction is not relieved, capillary rupture and haemorrhage into bowel may ensue. In cases of volvulus and intussusception, the arterial supply gets compro­ mised rapidly causing gangrene of bowel wall very early. Bacterial proliferation takes place and endotoxins are released. • Transmigration (translocation) of gram-negative organisms, anaerobes and gram-positive organisms through the gangrenous bowel results in peritonitis. • The organisms release powerful endotoxins which are absorbed from the peritoneal surface and cause gram-negative shock or septic shock. It carries very high mortality rate (30%). • Early gangrene without obstruction is a feature of mesen­ teric thrombosis or embolism. • Loss of blood volume is an important feature of massive gangrene.

.,.............. Fig. 30.6: Gangrene of the intestine due to bands

• • • • • •

FACTORS PREDISPOSING ISCHAEMIA

Volvulus Mesenteric ischaemia Necrotising enterocolitis lntussusception Progressive distension Extrinsic compression by adhesions, bands, etc.

�

Intestinal Obstruction

733

PEARLS OF WISDOM

Vomiting of faeculent contents indicates terminal ilea/ obstruction. 3. Distension of the abdomen: It may be central abdominal distension as seen in ilea] obstruction, peripheral abdominal as in large bowel obstruction, or localised to one or two quadrants as in sigmoid volvulus. 4. Constipation occurs because the distal bowel does not move. Constipation to faeces and flatus is called obstipation. Exceptions are given in Key Box 30.4. Signs 1. General signs of dehydration such as dry skin, dry tongue,

Fig. 30.7: Faeculent vomiting is pathognomonic of terminal ilea!

obstruction

Clinical features (Key Box 30.3)

sunken eyes, feeble pulse, low urinary output are seen. Dehydration occurs due to persistent vomiting and sequestration of fluid and electrolytes. Hypokalaemia is an important finding. 2. Abdominal findings • Distension, tympanitic note on percussion • Step ladder peristalsis is seen in terminal ilea! obstruction. Right to left colonic peristalsis is seen in left-sided colonic obstruction, large bowel obstruction. • On auscultation-loud, noisy intestinal sounds are heard. They are called borborygmi. • Hernial orifices have to be examined, especially for a femoral hernia in females.

1. Pain abdomen: Central abdominal pain is a feature of small

intestinal obstruction and peripheral pain is a feature of large intestinal obstruction. The pain is colicky in nature, lasts for 5-10 minutes and is intermittent. On pressure, it decreases. 2. Vomiting is due to reverse peristalsis. Vomitus consists of stomach contents initially, then bile, followed by faeculent matter. Faeculent is not faecal matter but terminal ileal contents which undergo bacterial degradation and fermentation resulting in the smell of faecal matter. Vomiting of altered blood indicates haemorrhage and gangrene. Frequent vomiting reflects jejuna! obstruction (Fig. 30.7 and Table 30.1).

Signs of strangulation • It should be suspected when features of obstruction are present along with features of shock.

dtMl!,I..........� dt!l!l,I..........� • • • •

CARDINAL FEATURES OF INTESTINAL OBSTRUCTION

• • • •

Colicky abdominal pain Abdominal distension Vomiting Absolute constipation

INTESTINAL OBSTRUCTION WITH DIARRHOEA

Faecal impaction Richter's hernia Gall stone ileus Mesenteric vascular occlusion

: : : :

Page 731 (Fig. 30.4) Page 858 Page 747 Page 753

Comparison of clinical features at different levels of intestinal obstruction

Vomiting Distension Pain Constipation Peristalsis

High Uejunum)

Distal (ileum)

Frequent, bilious No Intermittent, not crescendo type Not initially Not seen

Moderate bilious, faeculent Moderate distension Intermittent, crescendo type, colicky Not initially Step ladder peristalsis

Low (colon) Late vomiting, faeculent Marked distension Variable pain, not classical crescendo Initially present Right to left peristalsis may be seen

Manipal Manual of Surgery

734

Fig. 30.8: Dilated intestinal loops

Fig. 30.10: Gangrene due to volvulus

• Features of septic shock-fever, hypothem1ia, renal failure, respiratory failure (Key Box 30.5). • Rebound tenderness: It is called Blomberg's sign. It is a classical sign of peritonitis. • Guarding and rigidity of the abdominal wall. • Absent bowel sounds because rest of the bowel loops undergo paralytic ileus. • Sudden symptoms-spasmodic pain (due to peristalsis) and continuous pain suggest strangulation (Fig. 30.11 ). • Features of strangulation and perforation occur quickly in cases of closed loop obstruction (Figs 30.8 to 30.11 and Key Box 30.6).

MIMI,!........,.� FEATURES OF STRANGULATION

• • • •

Tachycardia Tenderness Temperature-fever Acidosis

Fig. 30.9: Perforation due to obstruction

.,............. Fig. 30.11: Step ladder peristalsis

CLOSED LOOP OBSTRUCTION

�

• This occurs when the bowel is obstructed at both proximal and distal points. • Proximal bowel is not distended as much in this condition • Gangrene and perforation can occur fast • Retrograde thrombosis of mesenteric vein, can result in distension of the bowel. • A few examples of closed loop obstruction include sigmoid volvulus, strangulated hernia, carcinoma right colon.

Rectal examination • In small bowel obstruction, rectum is empty and is often ballooned out. • Carcinomatous growth with or without stools can be felt. • The finger may be stained with blood.

Intestinal Obstruction

735

• The small intestine is considered dilated if loops of bowel measure more than 3 cm in diameter. Measurements for the large bowel vary among different anatomic segments, with a relative threshold of 9 cm in diameter for the proximal colon and 5 cm for the sigmoid colon. INVESTIGATIONS • Complete blood picture: Low Hb% indicates underlying malignancy. Increased total WBC count indicates infection and sepsis (perforation). • Electrolytes: Most of the electrolytes are low in cases of intestinal obstruction and require con-ection preoperatively. Strangulation may be associated with deranged potassium, amylase or lactic dehydrogenase. • Plain X-ray abdomen in the erect position may show multiple gas fluid levels. Gas levels appear earlier than fluid level. Normally, two insignificant fluid levels can be present, one in the terminal ileum and one in the first part of the duodenum (Key Box 30.7). Supine films indicate the distal limit of obstruction (Figs 30.12 to 30.16). PEARLS OF

Fig. 30.12: Multiple gas fluid levels-plain X-ray erect abdomen

WISDOM

Enteroclysis is rarely performed in acute intestinal obstruction but it has greater sensitivity in the detection of partial small bowel obstruction (Fig. 30.17). • Ultrasound/CT scan-See Fig. 30.18, Key Boxes 30.8 and 30.9

Mi!l,I.........,.� PLAIN X-RAY FINDINGS UPRIGHT AND SUPINE

• First get supine films. They indicate distal limit of obstruction. Erect films are asked if any doubt exists about obstruction. • Jejunum is characterised by regularly placed mucosa! folds called valvulae conniventes (Fig. 30.14) placed opposite to each other (Herring bone pattern). They are produced by valves of Kerckring. • Large bowel is characterised by haustrations (Fig. 30.15): Incomplete, large mucosa! folds, not placed opposite to each other. • Caecum has no haustrations. It appears as a round gas shadow in the right iliac fossa. • Ileum has no characters-characterless loop of

Fig. 30.13: Multiple gas fluid levels seen with a large dilated loop in the centre

Fig. 30.14: Valvulae conniventes

Wangensteen.

• Plain X-ray may demonstrate gall stone ileus or foreign body. • Gas is absent in the small bowel as in mesenteric vascular ischaemia. • Sigmoid volvulus appears as a large dilated loop-inverted 'U' shape.

Fig. 30.15: Haustrations

736

Manipal Manual of Surgery

MIMIHIIIIIIWIIIIIIIIW� CT SCAN IN INTESTINAL OBSTRUCTION • Can detect dilated intestines proximally and collapsed bowel distally. • If bowel wall is thick and air is present (pneumatosis), strangulation is likely. • It can detect portal venous gas (suggesting gangrene) • CT can detect mass lesions-carcinoma sigmoid, caecum or ileocaecal mass (TB). • CT has low sensitivity in detecting low grade or partial small bowel obstruction. Sensitivity increases in total obstruction.

Fig. 30.16: Plain X-ray abdomen supine-distended intestines. Supine X-ray will indicate distal limit of obstruction

Fig. 30.18: lleocolic intussusception-observe 'target sign' on the right side

MANAGEMENT Preoperative preparation includes correction of dehydration, electrolytes and broad spectrum antibiotics. Principles in the management of intestinal obstruction are as follows: Fig. 30.17: Jejunal stricture due to tuberculosis-enteroclysis picture

MHM,l.........."J ULTRASOUND • It is not the investigation of choice or it may not be required However, a good sonologist can diagnose: a. Dilated loops of intestine b. Presence of fluid in the abdomen c. Gall stone ileus d. lnfarcted/ischaemic bowel/gas in the portal vein or intrahepatic gas. e. lntussusception and can assess vascularity with the help of duplex scan. f. It can also rule out other causes Being a noninvasive investigation, it has more benefits

A. Aspiration with Ryle's tube. This is the most important step in the management of intestinal obstruction. It helps in decreasing the distension and also prevents vomiting. This will help in preventing respiratory complications, such as aspiration following general anaesthesia. B. Bowel care: No purgatives because purgation can cause perforation. C. Charts: Temperature, pulse, respiration and intake-output chart. In cases of conservative management such as obstruction due to adhesions, change in temperature and increasing pulse rate suggests perforation or gangrene. These cases have to be explored immediately. D. Drugs to cover gram-positive, gram-negative and anaerobic organisms. E. Exploratory laparotomy is done and depending upon the findings, obstruction is treated. A few examples are given in Key Box 30.10 and Fig. 30.19.

737

Intestinal Obstruction

F. Fluids should be given before, during and after surgery. It forms the most important treatment of intestinal obstruction.

e41.,�, • • • • • • • • • • •

PRINCIPLES OF EXPLORATORY LAPAROTOM\' Ideally done within 6-8 hours Long midline incision Resection of gangrene and anastomosis Adhesion-release Bands-divide Gall stone ileus-remove the stone Volvulus-untwist or resection Obstructed hernia-reduce Gangrene-resect Stricture-resection or stricturoplasty Advanced malignancy-bypass Long midline incision

Caecum is collapsed

Caecum is distended

Small intestinal obstruction

Large intestinal obstruction

No gangrene

Viable bowel Treat the cause

Resection

Treat the cause

Treat accordingly (e.g. perforation)

Fig. 30.19: Principles of management of intestinal obstruction

APPROACH TO THE MANAGEMENT OF INTESTINAL OBSTRUCTION

Ask the following questions to yourself and proceed. 1. What is the probable cause of obstruction? 2. Is it small bowel obstruction at laparotomy? 3. Is it large bowel obstruction at laparotomy? 4. Is it simple obstruction? 5. Is it strangulation? 6. Is it some kind of a surprise or a difficult case? 7. Can I manage conservatively? 1. Probable cause of obstruction • A previous laparotomy scar may indicate that it could be an adhesive obstruction (most common). • An obvious obstructed hernia (inguinal or femoral) can be managed with inguinal approach. • An elderly man, hypertensive and atherosclerotic, with features of blood in the stools and acute abdominal pain may be having superior mesenteric ischaemia. • A constipated, elderly man in poor health, with acute or chronic obstruction may be having carcinoma of the colon.

2. Diagnosis of small bowel obstruction at laparotomy

• Caecum is collapsed • Dilated loops of small intestine are present. • A stricture or a mass lesion may be obvious at laparotomy.

3. Diagnosis of simple obstruction

• It is done when bowel is not gangrenous. • In doubtful cases, because of long-standing ischaemia, wrapping the bowel with warm and moist pack and adminis­ tration of pure oxygen may help the bowel to recover from ischaemia.

4. Diagnosis of large bowel obstruction at laparotomy

• Caecum is distended. • A growth may be palpable and obvious in the transverse colon or in the hidden colon, i.e. splenic flexure. • It is very important to examine the entire colon (synchronous carcinoma is more common). 5. Diagnosis of strangulation

(Fig. 30.20 and Key Box 30.11) • Black, dark, foul-smelling bowel is seen as soon as laparo­ tomy is done. • Peritoneal fluid contains blood-stained fluid. • Precautions must be taken not to contaminate peritoneal cavity when gangrenous segment is removed. Do not hesitate to take the help of senior experienced surgeons in treating an uncommon situation such as massive ischaemia and gangrene of small bowel and colon (due to mesenteric vascular occlusion), synchronous carcinoma and ileosigmoid knotting, etc.

Ml!ffl.............� • • • •

VIABLE BOWEL-FEATURES Normal peristalsis Normal peritoneal sheen is present Normal pulsations are visible or felt at the mesentery Normal pink colour is present

Fig. 30.20: Viable and nonviable bowel

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Manipal Manual of Surgery

6. It is a surprise • Surprises are well known in intestinal obstruction. Congenital bands, foreign bodies, internal herniation, lymphomatous strictures are a few examples. • The detailed management of individual cases is discussed below.

4

7. Can I manage conservatively? • In these cases, a long intestinal tube called Miller-Abbott tube can be passed to decompress intestines (Key Box 30.12).

11111,1........._.� INTESTINAL OBSTRUCTION CONSERVATIVE TREATMENT 1. Partial small bowel obstruction mostly due to adhesion:

Wait for 48 to 72 hours. They may show improvement. If not, surgery is required. 2. Early postoperative obstruction: It rarely progresses to strangulation. Hence, nonoperative management can be extended to many days (3-7) provided there is no evidence of peritonitis. 3. Intestinal obstruction in Crohn's disease: Aim in Crohn's disease is to 'preserve' bowel as it may respond to medications. 4. Carcinomatosis: Disseminated malignancy with obstruc­ tion. The aim is nonoperative treatment as nothing much can be achieved with laparotomy. DIFFERENTIAL DIAGNOSIS OF INTESTINAL OBSTRUCTION VOLVULUS OF THE SIGMOID COLON • Common in North India (Punjab), Eastern Europe, Uganda. • In certain parts of India as mentioned above, it is one of the common surgical emergencies in elderly population (Key Box 30.13).

MIii,........._.�

Fig. 30.21: Causes of sigmoid volvulus (see text for numbers)

3. Long, redundant, pendulous sigmoid 4. Loaded colon due to high residue diet 5. Diverticulitis with a band, or adhesions Sigmoid volvulus is a definite occurrence in mentally disturbed patients, hypothyroidism, Parkinson's disease, multiple sclerosis, etc. probably due to severe constipation due to medications. PEARLS OF

WISDOM

Ogilvie 's syndrome precipitates volvulus.

Clinical features 1. Acute sigmoid volvulus (fulminant) presents as intestinal obstruction. It starts usually after straining at stools. Volvulus is usually in anticlockwise direction and after one and a half turns, the entire loop becomes gangrenous. Enormous distension of the abdomen takes place, which gives a tympanitic note all over the abdomen. It is due to diffusion of CO2 (Fig. 30.22). Due to gross distension,

CERTAIN FACTS ABOUT SIGMOID VOLVULUS

• More common in males • 2/3 of the cases are sigmoid volvulus and 1 /3 are caecal volvulus. • Common in middle age and > 60 years • It consists about 10 to 15% of cases of intestinal obstruction in India. • More common in rural population • It is not an uncommon event during pregnancy Precipitating factors (Fig. 30.21) 1. Long mesentery of the pelvic colon 2. Narrow attachment at the base

Fig. 30.22: Uneven distension due to sigmoid volvulus

Intestinal Obstruction

severe hypovolaemic shock develops within 6-8 hours of volvulus. Gangrene sets in, which gives rise to features of strangulation. A dilated loop can be seen and felt. Features of peritonitis are seen within 1-2 days. Per rectal examination shows rectum to be empty. PEARLS OF

WISDOM

Distended tympanitic drum like abdomen-sigmoid volvulus.

2. Chronic (indolent) recurrent sigmoid volvulus: It occurs due to partial twisting and untwisting of the bowel. Elderly patients present with recuITent lower abdominal pain on the left side and distension of the abdomen which is relieved on passing large amount of flatus. Diagnosis • Plain X-ray abdomen erect shows a hugely dilated sigmoid loop which is described as 'bent inner tube sign'. The dilated loop may be visible on the right side, centre and to the left of abdomen, having two fluid levels, one on right side and one on left side. This is also described as 'omega sign' (Figs 30.23 and 30.24). • Contrast enema: As the barium enters the rectum, it tapers into the sigmoid colon-Bird's beak sign.

739

II. Operative treatment I. Single-stage resection: This can be done, provided general condition of the patient is good. If the loop is gangrenous, resection followed by end to end anastomosis is done, after giving 'on table' lavage using saline washes till the contents of the colon are clear. Sigmoid colon is hugely dilated (Figs 30.25 and 30.26). 2. Hartmann's procedure: If the loop is gangrenous and proximal bowel is loaded with faecal matter, resection of the sigmoid colon is done. Proximal descending colon is brought out as an end colostomy and rectum is closed (Hartmann 's procedure). After 6 weeks, colorectal anastomosis is done. 3. Sigmoidopexy: If the loop is not gangrenous, untwist the sigmoid loop and fix the sigmoid to the posterior abdominal wall (sigmoidopexy). If the mesentery is long, it can be made short by plication. 4. Exteriorisation: Paul-Mickulicz procedure is done when general condition of the patient is poor as in elderly patients, in severely dehydrated patient with impending septicaemia. In such cases, the gangrenous loop is brought outside and resected, with a proximal colostomy and a distal mucous fistula (Fig. 30.27).

Treatment I. Nonoperative • A successful passage of flatus tube or sigmoidoscope up to 25-30 cm results in release of a large amount of flatus and fluid and obstruction is relieved. If obstruction is completely relieved or if there is no gangrene and the general condition of the patient improves, an elective resection is done after 7 days. If resistance is found while passing flatus tube, instill barium for guidance.

Fig. 30.25: Sigmoid colon at surgery-huge distension results in severe hypovolaemic shock

Fig. 30.23: Plain X-ray abdomen Fig. 30.24: An 84-year-old showing distended sigmoid patient with sigmoid volvulus, loop managed by sigmoidoscopic decompression

Fig. 30.26: Distended sigmoid with venous congestion

Manipal Manual of Surgery

740

• In a plain X-ray abdomen, caecum produces round shado, in the centre of the abdomen. • Resection is the ideal treatment. PEARLS OF WISDOM

If loop is gangrenous, do not derotate-clamp the mesentery first, then detorsion can be done to avoid reperfusion injury.

Fig. 30.27: Paul-Mickulicz procedure

CAECAL VOLVULUS AND BASCULE • It is a rare cause of intestinal obstruction (Figs 30.28 to 30.32 and Key Boxes 30.14 and 30.15). • Presumably, it is more likely to occur following any surgical procedure which might require some degree of medial visceral rotation or disruption of the fusion plane between the caecum or ascending colon with the lateral peritoneum, providing sufficient mobility allowing caecal volvulisation to occur. • A constant feature of caecal bascule is the presence of a constricting band across the ascending colon. This may be found at laparotomy.

Fig. 30.28: Supine X-ray findings in caecal volvulus

COMPARISON OF CAECAL VOLVULUS AND SIGMOID VOLVULUS Caecal volvulus

Sigmoid volvulus

• Rare

• Common

• Clockwise twist

• Anticlockwise

• Mobile caecum

• Long mesentery is the cause

• Middle-aged

• Elderly, debilitated

• Kidney-shaped gas shadow with single fluid level on the left side.

• Omega sign or coffee bean­ shaped. Two fluid levels can be found.

• Treated only by surgery

• Nonoperative treatment should be attempted in all cases, provided there is no ischaemia.

Fig. 30.29: Erect X-ray showing distended loop. Caecum was palpable in the left hypochondrium

Fig. 30.30: CT X-ray showing dilated coils of bowel

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Intestinal Obstruction

MECKEL'S DIVERTICULUM WITH A BAND It is a congenital diverticulum which occurs due to persistent intestinal end of vitellointestinal duct. Being congenital, it has all the layers of the bowel. Hence, a true diverticulum. Anomalies of vitellointestinal duct (Fig. 30.33) A. Fibrous band results when entire duct is obliterated and bowel can twist around the band, resulting in volvulus. B. Persistent intestinal end: Meckel 's diverticulum (Key Box 30.16). C. Meckel's diverticulum with the band attached to the umbilicus can give rise to intestinal obstruction. D. Umbilical fistula results when entire duct is patent. Even though it is connected to the tenninal ileum, the opening is very small. The discharge is rarely faecal. Often, it is the mucus secreted from the lining of the duct (omphaloenteric fistula). E. Umbilical sinus results due to persistent umbilical end discharging mucus. Slowly umbilical adenoma occurs and epithelial lining of the sinus gets everted. F. Intra-a bdominal cyst results when both ends are obliterated. The central portion of the duct persists and secretes mucus. This is very, very rare.

Fig. 30.31: Distended caecum at surgery ( Courtesy: Dr Basavaraj Patil, Fig. 30.32: Caecal volvulus with Dr Hartimath, Dr Rasheed, gangrene Dr Nikil, Department of Surgery, KMC, Manipal)

IIMtl,I.........,.� CAECAL BASCULE

• Caecum folds anteromedial to the ascending colon, with production of a flap-valve occlusion at the site of flexion. • Caecum will be markedly distended and will be found in the centre of the abdomen.

MHII!,.........�

• Occasionally, it is associated with malrotation of the gut • There will be a constricting band across the ascending colon.

MECKEL'S 1 DIVERTICULUM AND OTHER ASSOCIATED ANOMALIES

• It is also argued that caecal bascule can also be due to lack of fixation of large bowel. Resection is ideal even though fixation (caecopexy) is also another alternative.

• Angiodysplasia of the caecum • Anorectal atresia, atresia of the oesophagus

• Bascule is a French term for see-saw and balance

D

1The most common congenital anomaly of small intestine

E

F

Figs 30.33A to F: Anomalies of vitellointestinal duct (A to F see text for details)

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MECKEL'$ DIVERTICULUM • It is present in 2% of the cases, 2 inches long, 2 feet away from ileocaecal region in the antimesenteric border (ilea! duplication can occur in the rnesenteric border). It is two times more common in females. • Symptomatic cases are below 2 years of age. • In 12% of the patients, heterotopic gastric tissue is found which can produce peptic ulceration. In a few other patients, it can contain pancreatic and colonic tissue. Rule of 2 for Meckel's diverticulum • • • • •

Incidence: 2% Location: 2 feet proximal to ileoceacal junction Length: 2 inches long Ectopic tissue: 2 types-gastric and pancreatic Presentation: 2 years or below 2 years is the most common age • Male: female ratio-1 :2

Clinical presentation • Massive bleeding per rectum: In the form of melaena, it is not uncommon. ln many other patients, mild chronic bleeding can result in anaemia. • Acute Meckel's diverticulitis: Factors which precipitate diverticulitis are: - Peptic ulceration due to ectopic gastric mucosa of the diverticulum - Ingested foreign material, e.g. stalk of vegetable, seeds, fish or chicken bones - Faecolith (not common in ileum), tumours, worms causing stasis and bacterial infection - Inflammation and ischaemia caused by torsion due to an associated band is called mesodiverticular band - Association with acute appendicitis Such cases can perforate very often. It is impossible to differentiate it from ruptured appendix. This is treated by laparotomy and resection of diverticulum along with adjacent intestine. In majority of appendicular perforations, local abscess will occur because of retrocaecal position of the appendix (70%). However, perforation of Meckel 's diverticulum, even though a rare cause of peritonitis, has a high mortality rate. This is because infection spreads very fast as diverticulum is intraperitoneal and contents are faeculent. PEARLS OF

WISDOM

Older child, deformed umbilicus, scarless abdomen with intestinal obstruction-cause may be Meckel's diverticulum. • As a cause of intestinal obstruction, when it is associated with a band or due to volvulus. It is the most common presentation in adults (Key Box 30.17).

• • • • • •

MECKEL'S DIVERTICULUM AND INTESTINAL OBSTRUCTION lntussusception Band Volvulus due to band Internal herniation beneath mesodiverticular band Diverticulitis with band Littre's hernia

• As a cause of intussusception: Here also, inflamec heterotopic tissue can be found in the diverticulun (2% cases). • Pain can occur due to chronic peptic ulceration. • Neoplasm: Carcinoids and GIST are more common ir Meckel's diverticulum than elsewhere in the small intestine although presence ofMeckel's diverticulum itsel1 1s rare. PEARLS OF

WISDOM

Hernia of Littre is a hernial sac containing Meckel's diverticulum (Key Box 30.17). Investigations 1. No investigation can prove diagnosis of Meckel's diverticulum. Small bowel enema may demonstrate the diverticulum if opening is wide (fluoroscopy is more ideal). 2. 99 mTc-labelled pertechnetate given IV, may localise the heterotopic gastric mucosa in the Meckel's diverticulum, in about 90% of patients. • This radionuclide is taken up by mucin-secreting cells and parietal cells and it is secreted immediately. Thus, if 99111Tc appears in the stomach as well as in other part of bowel, it indicates functioning heterotopic tissue. • Even when bleeding is at a rate of 0.1 ml/minute, it can detect Meckel's diverticulum. Hence, it is superior to angiography. • Very useful in children with bleeding. PEARLS OF

WISDOM

T he pertechnetate anion 99111Tc is selectively taken up by gastric mucosa/ cells, thyroid, salivary glands and choroid plexus.

Treatment (Fig. 30.34) 1. Incidentally found Meckel's during laparotomy for some other causes can be left alone, provided it has a wide mouth. However, a note of it must be made in the operation register (Key Box 30.18). 2. Meckel's diverticulum with bleeding, band, perforation and narrow mouth is treated by removal of diverticulum with

Intestinal Obstruction

743

Fig. 30.34: Meckel's diverticulectomy should include normal

intestine also

Fig. 30.36A: Resected Meckel's diverticulum with normal intes­ tine ( Courtesy: Dr Pramod K, Dr Deviprasad Shetty, Kasturba Hos­

pital, Manipal)

Fig. 30.35: Inflamed Meckel's diverticulum

adjacent intestine because the gastric tissue may often line the intestine also (Figs 30.35 and 30.36). Fig. 30.368: Meckel's diverticulum with band and obstruction

• • • • •

INDICATIONS FOR REMOVAL OF INCIDENTALLY FOUND MECKEL'S DIVERTICULUM

Children under 2 years of age Meckel's with a band Meckel's with adhesions Meckel's with narrow base Long Meckel's diverticulum

ADHESIONS AND BANDS ADHESIONS Introduction: Intra-abdominal adhesions develop after abdominal surgery as part of the normal healing processes that occur after damage to the peritoneum. The early balance between fibrin deposition and degradation seems to be the critical factor in adhesion formation. They also cause

significant morbidity, including adhesive small bowel obstruction, infertility and increased difficulty with reoperative surgery. Thus, high chances of causing intestinal fistula after re-exploration. Definition: Peritoneal adhesions can be defined as abnormal fibrous bands between organs or tissues or both in the abdominal cavity that are normally separated.

They are common causes of intestinal obstruction in the western world, Chinese population of Malaysia, etc. In India, adhesions and obstructed hernia are the two common causes in adults. PEARLS OF

WISDOM

It is important to realise that seconda,y infertility in women and ectopic gestation can occurs due to adhesions.

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.............................

Causes

1. Infection: Laparotomy done for acute appendicitis with or without perforation, perforation peritonitis, intra-abdominal abscess have higher incidence of adhesions. Surgery is the commonest cause of peritoneal adhesions. 2. Iatrogenic: It refers to talc, silk thread, foreign body (mop), etc. used for surgery which can induce extensive adhesions due to foreign body reaction (spilled gall stone also). 3. Jschaemia: Lack of blood supply, particularly venous occlusion can cause adhesions, e.g. mesenteric vascular occlusion.

KEY BOX 30.19

PATHOGENESIS OFADHESIONS Peritoneal injury/ischaemia/irritation Inflammation Infiltration of inflammatory cell, release of cytokines, activation of complement system and activation of coagulation cascade Fibrinous exudate

4. Injury to the bowel can result in adhesions. 5. Irradiation enteritis is becoming common due to iITadiation for carcinoma of the cervix. Remember the important 5 Is. Pathogenesis

Fibrous adhesions

Complete resolution

Dense fibrous adhesions

• Peritoneal plasminogen activator release results in lysis of adhesion.

Ischaemia and irritation of the intestines are the chief factors responsible for adhesions (Key Box 30.19). Types (Fig. 30.37)

1. Fibrinous adhesions (bread and butter adhesions): They are the causes of early postoperative obstruction, which settles down within 3-5 days. Majority of them disappear in due course of time. 2. Fibrous adhesions: If the infection is continuing or if foreign body is present, the fibrinous material is convetied to fibrous band. They also occur at the site of ischaemia. They will cause late intestinal obstruction (Figs 30.38 and 30.39). 3. Tuberculous adhesions are dense adhesions that result in matting of intestinal coils. Separating them at laparotomy is extremely difficult (Fig. 30.40). • Congenital band is the 4th type of band (not due to adhesion). P EARLS

OF

Fig. 30.37A: Bread and butter adhesions-case of perforation peritonitis

WI SDOM

A congenital band, omental band, a string band of bacterial peritonitis or afibrous band of tuberculosis is responsible for obstruction (Figs 30.41, 30.42 and Key Box 30.20). Clinical features

• Recurrent abdominal pain, vomiting and distension are the typical features. Often the attacks are mild and selflimiting. But persisting symptoms require monitoring and treatment. • There may be peristalsis as in cases of terminal ileum. • Gangrene is not common in cases of adhesive obstruction (Figs 30.42 and 30.43). However, these cases have to be closely monitored for any changes in the type of pain or

Fig. 30.378: Band following tubectomy-has been divided

Intestinal Obstruction

745

Fig. 30.38: Band causing obstruction - proximal dilated and distal collapsed bowel

Fig. 30.41: Congenital band causing obstruction

Fig. 30.39: Postappendicectomy band causing obstruction

Fig. 30.42: Adhesive obstruction

Fig. 30.40: Tuberculous adhesions and bands causing intestinal obstruction

Fig. 30.43: Close up view of gangrene

Manipal Manual of Surgery

746

Treatment I. Conservative treatment • ln the form of nasogastric aspiration, IV replacement c fluids and electrolytes to correct dehydration may b successful in early postoperative obstruction. If it is nc successful, reoperation is required. Generally 48-72 hour is the waiting period in patients who present to the hospitf as late adhesive obstruction. Further delay may result ii perforation or gangrene of the bowel.

Fig. 30.44: Band secondary to peritonitis causing gangrene

Mi¥1,..........,, • Congenital • Acquired

TYPES OF BAND

�

: Transduodenal band of Ladd : Vitellointestinal duct associated band : Following peritonitis : Diverticulum : Greater omentum as a band

new abdominal signs. Severe pain, tachycardia, tempera­ ture, tacbypnoea and tenderness in the abdomen indicates gangrene or perforation (Figs 30.43 and 30.44). • Gilroy Benan triad of adhesive pain: •

•

Pain gets aggravated or relieved on change of posture.

Pain in the region of old abdominal scar. • Tenderness elicited by pressure over the scar.

Investigations • Plain X-ray abdomen and small bowel enema are very useful investigations to prove the obstruction. • Computed tomography (CT) enhanced with oral contrast: -

Detects air-fluid level: Complete obstruction The absence of mass lesion Dilated and collapsed loop junction Thickening and oedema of the bowel wall suggests intestinal ischaemia. Presence of intramural air is a late sign (gangrene) - CT has a sensitivity of 90% and specificity of 88% - Thus, CT and MRI are very helpful in patients with small bowel obstruction.

• Record pulse rate, blood pressure, abdominal girth arn intake output. Increasing pulse rate, hypotension, increasini abdominal girth and oliguria in spite of adequate IV fluid: will suggest gangrene. Such cases need to be explore< immediately. II. Surgical methods (Key Box 30.21) • Where fibrous bands are the cause, they need to be dividec to relieve obstruction. • Laparoscopic adhesiolysis is more often being used and i1 is indicated in pelvic adhesion, selected cases of abdominal adhesion, single band adhesion and obstruction with mild distension. Ill. Prevention of adhesion (Key Box 30.22) A. Recently absorbable and nonabsorbable membrane barriers such as expanded polytetrafluoroethylene (PTFE) and membrane composed of hyaluronic acid and carboxy­ methyl cellulose have been used. B. Noble's plication: By suturing loops together so that they are fixed in a suitable relation to one another (not very successful) (Fig. 30.45). See Key Box 30.23 for summary of adhesive obstruction.

11111...........� HOW TO DECREASE ADHESIONS? • Handle the bowel carefully. Good suturing without tension. Avoid anastomotic leak. • Raw peritoneal areas should not be sutured

• Thorough peritoneal toilet in cases of peritonitis with saline or dextran to drain pus, bile, blood clots. • Avoid spillage of contents-bile, faecal matter. • Prefer a Pfannenstiel incision than midline incision. • Noble's plication (Fig. 30.45)

• Laparoscopic method produces decreased adhesions than laparotomy. • Membrane barriers

Intestinal Obstruction

747

--:::----.-��-- Plication sutures

Fig. 30.45: Surgery for adhesions

Fig. 30.46: Cholecystoduodenal fistula caused by gall stones

MUI,..........� MEMBRANE BARRIERS • The bioresorbable membrane called seprafilm is currently the most effective membrane barrier. • It consists of hyalorunic acid and carboxymethylcellulose • At the completion of surgery, these films are placed at the potential sites of adhesion formation-such as pelvis, between the intestinal loops. • Mechanism of action: Within the next 24 to 48 hours, the seprafilm membrane hydrates to form a gel-like barrier. It slowly resorbs within 7 days. • These barriers should not be used to cover the anastomosis-chances of leak rates are high.

Fig. 30.47: Gall stones ileus {both these figures are contributed by Ms Vidhushi, Kasturba Medical College, Manipal) ADHESIVE INTESTINAL OBSTRUCTION • It is the most common cause of intestinal obstruction (in the West). • The most common cause of adhesion is inflammatory­ peritonitis. • It is the cause of recurrent intestinal obstruction-often partial. • Conservative treatment is successful in majority of cases. • One can wait for 3-4 days with careful monitoring before a decision of laparotomy is undertaken (worsening situation). • Repeated X-ray abdomen and CT scan will show changes and progression of the obstruction. • Most valuable clinical signs of ischaemia/gangrene are tachycardia and tenderness. • Adhesiolysis/resection is the treatment.

GALL STONE ILEUS-GALL STONE OBTURATION Introduction: Gall stone ileus is more common in women and accounts for 1-4% of all presentations to hospital with

small bowel obstruction. T he term gall stone ileus is a misnomer, as the condition is a mechanical obstruction of the gut and not a true ileus. • It should be suspected in a patient who has gall stones and present with intestinal obstruction. • Elderly females above the age of 60 are usually affected. • Gall stone reaches the terminal ileum by forming 'cholecystoduodenal fistula' due to recurrent attacks of cholecystitis (Figs 30.46 to 30.49). • Due to recurrent inflammation, adhesions develop between gall bladder and duodenum (common) or gall bladder and colon or stomach (rare). Large stones cause pressure necro­ sis, resulting in formation of a cholecystoduodenal fistula. • Terminal ileum is the narrowest portion of the gut wherein gall stone gets impacted. Sometimes, the stone may ulcerate from gall bladder into jejunum, colon, etc.

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Manipal Manual of Surgery

--�-- Distended intestine

Fig. 30.48: Gall stone ileus (Courtesy: Prof MG Shenoy and Dr GN Prasad, KMC, Manipal)

Fig. 30.50: Rigler's triad

Investigations

1. Plain X-ray abdomen (erect position) may demonstrate multiple gas and fluid levels and stone in the gall bladde1 and also in the lower abdomen suggesting gall stone ileus. • Air may be found 1 in the biliary system. 2. Small bowel enema may demonstrate partial obstruction. 3. Computed tomography (CT) scanning is the investiga­ tion of choice. It has preoperative diagnosis of gall stone ileus with a sensitivity of 93%. It will also indicate any inflammation of the gall bladder, fistula-air pockets in the gall bladder, distal stone and proximal dilatation. Treatment

• There is difference of opinion regarding what is the ideal surgery to be done in case of gall stone obstruction. It is

Fig. 30.49: 17 Gall stones were removed in this case

• Clinical features: They are suggestive of small intestinal obstruction-abdominal pain is severe, vomiting and distension. Step ladder peristalsis may be seen. History suggestive of recurrent cholecystitis may be present. • Rigler's triad: Pneumobilia, the presence of an aberrant gall stone and enteric obstruction (Fig. 30.50). * Bouveret syndrome: It is a gastric outlet syndrome secondary to stone lodged in proximal duodenum due to cholecystoduodenal fistula. Proximal migration of the stone will precipitate obstruction.

Fig. 30.51: Enterolithotomy and removal of stone

1Cholecystoduodenal fistula, choledochoduodenostomy, sphincteroplasty, emphysematous cholecystitis are a few other conditions wherein air is found within biliary tree.

Intestinal Obstruction

749

because many patients are elderly, with co-morbidity and they have intestinal obstruction (dehydration, bowel oedema, bacterial proliferation, etc). Whatever it is, the first step is always to do enterolithotomy by incising the ileum and deliver the stone/stones. It may be possible to crush the stone and pass it onto caecum, avoiding enterolithotomy. Search the proximal intestine for any other stones. What must be done next? Two types are described. 1. Single stage procedure: If general condition of the patient is good, and gall bladder is gangrenous or inflamed (more chances of perforation) enterolithotomy, cholecystectomy followed by closure of the duodenal fistula is done. 2. Two stage procedure: If general condition is not good or with no active inflammation of the gal I bladder, enterolithotomy is the procedure of choice, followed 6 weeks later by cholecystectomy with closure of the duodenal fistula (Fig. 30.51 ). INTUSSUSCEPTION Definition Invagination of one segment of intestine into another (usually the proximal into distal) is called intussusception. PEARLS OF

WISDOM

It is the most common cause of intestinal obstruction in infants aged 6 to 18 months. Incidence: 2-4/1000 live births. Types

Fig. 30.52: Parts of intussusception (see the text for numbers)

AETIOPATHOGENESIS 1. Idiopathic intussusception: Actual cause is not known. It is seen in infants. Possible factors (Key Box 30.24). • Dietary factor: Around the age of6-9 months, weaning of breast milk is done. Weaning causes alteration in the bacterial flora in the GIT, causing enlargement of the Peyer's patches. These protrude into the terminal ileum and may precipitate intussusception.

.,,.,-,,, • • • • • •

�

PREDISPOSING FACTORS

Recent viral infection (upper respiratory ) Recent operation Henoch-Schonlein purpura Cystic fibrosis Coeliac disease Haemophilia

• Infective factor: It usually follows upper respiratory tract infection with virus (adenorotaviruses) which produce inflammation of Peyer's patches. 2. Adult intussusception (secondary): In adults, there is always a cause for intussusception (Key Box 30.25 and Figs 30.53 to 30.62).

1. Simple ileocolic is the most common type, followed by ileoileal or colocolic. 2. Compound-ileoileocolic 3. Retrograde jejunogastric intussusception, a complication of gastrojejunostomy (GJ) is a rare but interesting type of intussusception (see page 503).

.,,...........

Parts (Fig. 30.52) 1. lntussuscipiens: It is the outer tube (distal bowel which receives the intestine). 2. lntussusceptum: Proximal bowel (inner tube) which enters the distal segment. 3. Apex is the part which advances further into the distal bowel. 4. Neck, the narrowest portion of intussusception, is the junction of entering layer with the mass. • The whole mass that develops is called intussusception.

Pathophysiology • As the apex advances, it drags the mesentery containing blood vessels which get obstructed at the neck resulting in mucosa! ulcers and haemonhages. Marked lymphadeno­ pathy and hypertrophy of Peyer's patch is found at operation. • If the neck is too tight, gangrene sets in very early, as in ileocolic intussusception. • All other features of strangulation, dehydration, distension and septicaemic shock develop later.

�

ADULT INTUSSUSCEPTION

• Meckel's diverticulum • Polyps, tumours, submucous lipoma • Carcinoma colon (caecum, transverse colon)

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Manipal Manual of Surgery

Fig. 30.53: lntussusception

Fig. 30.54: Carcinoid of the ileum with intussusception

Fig. 30.55: Carcinoid of the ileum causing intussusception­ resected specimen

Fig. 30.56: lntussusception due to polyp

Fig. 30.57: Jejunal carcinoma-CT coronal section

Fig. 30.58: Opened specimen showing polyp

751

Intestinal Obstruction

Fig. 30.59: lleocolic intussusception CT

Fig. 30.61: lntussusception due to polyp at surgery

Fig. 30.62: lleocolic intussusception at surgery

Clinical features • First born male infants between 6 and 9 months are commonly affected. Boys : Girls - 3 : 2. • Child screams with abdominal pain 1 (intestinal colic) which is associated with facial pallor. • One attack of red currant jelly stools is characteristic. Bleeding is due to mucosa! ulcer (venous infarction). Mucus secretion is due to irritation of intestines. This is followed by absolute constipation. Red currant jelly stools are not found in adult intussusception. • Vomiting 3-4 times, initially due to pylorospasm. Later, due to obstruction. • In between the spasms, the child sleeps but gets up suddenly with pain. 1

Fig. 30.60: lntussusception due to polyp

Hindi speaking mother says, 'Baccha sota hai aur rota hai'

Fig. 30.63: Sausage-shaped mass-resonant, mobile

Signs • The mother is asked to feed the baby in sitting position and examination of the baby's abdomen is done with the left hand, standing in front of the mother. • A contracting, hardening mass in and around the umbilical region can be felt (sausage-shaped, Fig. 30.63). • Emptiness in the right iliac fossa (Dance's sign-signe de dance). • There may be a visible step ladder peristalsis. • Rarely, intussusception can be seen outside the anus due to long mesentery. • Rectal examination reveals blood-stained mucus on the examining finger. • Features of peritonitis occur in untreated cases.

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Manipal Manual of Surgery

Investigations • Barium enema: 'Claw (pincer) ending' (Fig. 30.64) is diagnostic ofintussusception. This is also called 'meniscus sign'. Ifthere is any suspicion ofgangrene, this test should not be done. In many cases, the diagnosis is established on clinical grounds.

is introduced into the rectum and 1 2 - litres ofsaline from l height of 1-2 metres is allowed to run. Catheter is removec and buttocks are pressed together. 50- 70% of cases an reduced by this method, I :3 barium sulphate in wam isotonic saline can also be used. • Air contrast enema will not reduce gangrenous bowel. Ai, is pumped into the colon at a pressure of 60-80 nunHg. Contraindications: • Peritonitis with shock • Total intestinal obstruction

PHI,........,.� HYDROSTATIC REDUCTION IS SUCCESSFUL WHEN

1. Flatus and faeces are passed with barium 2. Child is symptom-free and comfortable 3. Small bowel loops are filled with contrast Fig. 30.64: Claw ending

• Ultrasound (Figs 30.65 and 30.66) is the investigation of choice nowadays. It can detect target sign and detect mass (Doughnut sign) with Doppler. It can be used to assess vascularity of the bowel also. Thus, barium enema has become obsolete. • CT scan: It is the most sensitive imaging modality in the diagnosis of intussusception. Sausage-shaped mass, with blood vessels within bowel lumen are typical findings (see Fig. 30.59). Treatment I. Conservative treatment (Key Box 30.26) • Hydrostatic reduction can be attempted when the gangrene is ruled out as in early intussusception. A lubricated catheter

Advantage • Easy, nonoperative method Complication • Rarely, colonic perforation

II. Surgical treatment (Figs 30.67 to 30.69) Laparotomy and reduction of intussusception • lntussusception is reduced by milking (squeezing) the colon in opposite direction, which is facilitated by breaking the adhesions at the neck using the little finger. Appendicec­ tomy is also done, as it avoids any future confusion as to the reason for the abdominal scar. Fixing the caecum is not necessary because idiopathic intussusception rarely recurs. If the loop is gangrenous, resection and ileocolic anasto­ mosis is done. • Recurrent intussusception is rare: If it occurs, terminal ileum is sutured to the side of the ascending colon.

Figs 30.65 and 30.66: Ultrasound-pseudokidney sign, target sign, Duplex-assesses vascularity also

Intestinal Obstruction

753

See Key Box 30.27 for some interesting "more common" about intussusception.

11¥11!,dllllllllllllll� ACUTE INTUSSUSCEPTION-MOST COMMON • Most common in children between 5 and 10 months of age • Most common cause of intestinal obstruction in children • Most common cause is idiopathic {90%) • Most incidence of adenovirus infection, common in midsummer and midwinter • Most common variety is ileocolic variety • Most commonly hypertrophied Peyer's patches • Most common type of nonoperative reduction is by using air and barium enema • Most commonly done surgical procedure is reduction • Most commonly used noninvasive test for diagnosis is ultrasound-pseudokidney sign, target sign, Duplex­ assesses vascularity also.

MESENTERIC VASCULAR OCCLUSION

Figs 30.67 and 30.68: Adult intussusception due to jejuna! lipomatosis. This patient was 24-year-old male who presented with intestinal obstruction. A mass was palpable in the umbilical region at laparotomy. This mass was resected. Opened specimen showed extensive segmental lipomatosis (Courtesy: Dr Gabriel Rodrigues, Dr Mahesh Gopa Setty, Dr Lavanya K, KMC, Manipal)

Definition Acute mesenteric ischaemia is an abrupt reduction in blood flow to the intestinal circulation of sufficient magnitude to compromise the metabolic requirements and potentially threaten the viability of the affected organs (Key Box 30.28). Clinical features • It is common in elderly patients who are hypertensive and usually obese. • It is due to atherosclerosis causing thrombosis of the superior mesenteric arte1y or due to emboli which originate from atheromatous plaques or from the infarcted heart. • Smokers are more often affected by this condition. • Superior mesenteric vein can also get thrombosed due to injury during pancreatectomy or thrombosis as a result of oral contraceptive pills.

MHII!,...........� TYPES

Fig. 30.69: lntussusception is reduced at surgery

1. Acute mesenteric ischaemia (AMI): It is a sudden occlusion of artery or vein resulting in gangrene. It has a mortality rate of 60 to 90%. 2. Chronic mesenteric ischaemia (CMI): It is associated with stenosis of coeliac artery, superior mesenteric artery or inferior mesenteric artery.

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Manipal Manual of Surgery

Effects

The pathological effects of arterial occlusion and venous occlusion are the same. Superior mesenteric artery supplies the entire midgut starting from the duodenojejunal flexure to right one-third of the transverse colon. It is an end-artery. As a result of thrombosis, the entire small bowel and po11ion of the large bowel becomes gangrenous (if there is a thrombus at the origin of superior mesenteric artery). Causes of acute mesenteric ischaemia

I. Arterial emboli are the most frequent cause of AMI and are responsible for approximately 40 to 50% of cases. Most mesenteric emboli originate from a cardiac source. They lodge in the superior mesenteric artery (SMA) because it emerges from the aorta at an oblique angle. Few emboli lodge in the origin of middle colic artery. 2. Acute mesenteric thrombosis accounts for 25 to 30% of all ischaemic cases. Atherosclerotic disease is the cause of thrombosis. lt typically occurs at the origin of the superior mesenteric artery. Gangrene is more extensive in cases of thrombosis than embolism. 3. Nonocclusive mesenteric ischaemia (NOMI): Typically happens in cases of hypotensive patients. Low cardiac output, ICU patients on vasoconstrictors and inotropes precipitate the problem. Splanchnic vasoconstriction occur in response to hypovolemia. Vasoactive drugs, particularly digoxin, have been implicated in the pathogenesis ofNOMI. 4. Mesenteric venous thrombosis (MVT) is the least common cause of mesenteric ischaemia, representing up to l 0% of all patients with mesenteric ischaemia related to primary clotting disorders. Thrombi usually originate in the venous arcades and propagate (Key Box 30.29).

MIi,�� AETIOLOGY OF MESENTERIC VENOUS THROMBOSIS

• Hypercoagulable states (e.g. polycythaemia vera, protein C and S deficiencies). • Visceral infection • Portal hypertension • Blunt abdominal trauma • Malignancy: Pancreatic • Pancreatitis and in patients who smoke • Women taking oral contraceptives are also at increased risk of venous thrombosis. • Patients who have undergone splenectomy. To summarise the important causes

1. Atherosclerosis: Hypotension, haemorrhage into atheroma 2. Hypercoagulable state 3. Fibromuscular dysplasia

4. Takayasu 's arteritis 5. Embolic: Majority-emboli originate in the heart • Valvular heart disease • Dilated LA • Recent MI • Atrial arrhythmias • Ventricular dilatation with mural thrombus 6. Aorta: • Aneurysm • Atheroemboli Splanchnic circulation

• It is better to have some idea about the splanchnic circula­ tion to understand the effects of ischaemia to the gut (Key Box 30.30). • The splanchnic circulation receives approximately 25% of the resting and 35% of the postprandial cardiac output. • 70% of the mesenteric blood flow is directed to the mucosa! and submucosal layers of the bowel. Hence early cases present with bleeding due to mucosa! ulcerations. • 30% supplies the muscularis and serosal layers. • Blood flow is regulated by intrinsic (metabolic and myogenic) and the extrinsic (neural and humoral) factors. • Reactive hyperaemia, and hypoxic vasodilation are consi­ dered intrinsic controls and are responsible for instanta­ neous fluctuations in splanchnic blood flow. • An imbalance between tissue oxygen supply and demand will raise the concentration of local metabolites (e.g. hydrogen, potassium, carbon dioxide, and adenosine), resulting in vasodilation and hyperaemia.

Mlffl,I.........� ISCHAEMIC INJURY

Vascular occlusion

Inflammatory cell infiltrate

i

Loss of capillary integrity

i

Bowel wall oedema

i

Breakdown of mucosa! barrier

i

Bacterial translocation

i

Endotoxaemia

i

Sequestration of fluid within bowel lumen

Intestinal Obstruction

755

Reperfusion injury • Oxygen free radicals Cytokines from PMN leucocytes • Myocardial depression • Progressive inflammatory response • Increased capillary permeability • Tissue oedema and MODS. Clinical features

Symptoms 1. Abdominal pain: Severe, poorly localised, unresponsive to narcotics, out of proportion to the physical findings (Fig. 30.70). 2. Onset may be abrupt (embolism) or insidious (thrombosis) 3. Gastrointestinal emptying: Vomiting, diarrhoea, with occult or frank bleeding once infarction set in.

Fig. 30.70: Attitude of a patient with severe ischaemia due to superior mesenteric vascular occlusion. In these cases, the pain is disproportionate to the abdominal signs

Signs 1. Early: Soft, nontender, nonnal bowel sounds. 2. Late: Distension, tenderness, rebound tenderness, rigidity, absent bowel sounds. 3. Terminal: Cold clammy extremities--dehydration, thready pulse, hypotension, shock, acidosis. PEARLS OF

WISDOM

Sudden event, sudden bleeding, sudden collapse, severe pain, silent abdomen, shock progressing to sepsis is mesenteric ischaemia.

Investigations 1. Total counts are raised, in about 75% of cases > 15,000 cells/mm 3 . 2. Plain X-ray abdomen (erect) reveals absence of gas within the bowel loops and intramural gas. Blunt plicae are seen. This is called as thumb printing (Figs 30.71 and 30.72). 3. Metabolic acidosis in > 50% of cases. 4. Serum phosphate levels are raised within 3-4 hours following ischaemia as smooth muscle layer of small bowel is rich in phosphates. 5. D-dimer is elevated in all cases. 6. CT with or without angiogram is the investigation of choice. It can detect intramural air pockets in the bowel wall, air within biliary radicle, perforation, etc. It can also detect thrombus or nan-owing of superior mesenteric artery or superior mesenteric vein (Figs 30.73 and 30.74). 7. Emergency angiography is the test of choice and can be done within 6 hours of ischaemia. It can demonstrate arterial thrombosis at the origin or distally in one of the branches. (It can also be therapeutic.) 8. Hypercoagulability disorders - protein C and protein S antithrombin III, factor V, anticardiolipin antibody (MVT).

Figs 30.71 and 30.72: Plain X-ray showing presence of air in the bowel wall

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Manipal Manual of Surgery

c. Nitroglycerine d. Nitroprusside e. Prostaglandin E f. Phenoxybenzamine g. lsoproterenol 5. Sympathetic epidural block Assessing bowel viability • Doppler (84%) - Absent arterial flow on antimesenteric border of bowel. Absent mesenteric arterial flow • Fluorescein test (100%) Fig. 30.73: CT showing SMA thrombosis

- Sodium fluorescein 1 g administered IV over 30 to 60 seconds and the bowel examined using a hand-held long wave UV Wood's lamp (yellow). Recent advances in cases of mesenteric ischaernia • Catheter directed thrombolysis • Percutaneous transluminal angioplasty • Endovascular fenestration of aortic dissection: Techniques to assess bowel viability Pulse oximetry Infrared photoplethysmography - Bowel surface oximetry Quantitative fluorescence using perfusion fluorometer.

Fig. 30.74: CT confirming the same. A case of superior mesen­ teric arterial thrombosis with massive gangrene (See Fig. 30.75)

Treatment 1. Majority of the patients present late with massive gangrene. Massive resection of the gangrenous bowel followed by end to end anastomosis is done. These patients suffer from short bowel syndrome (vide infra), if they survive. 2. If patients come within 4-6 hours of ischaemia, emergency angiography followed by papaverine infusion (30 to 60 mg/h) into the superior mesenteric artery can be tried. Otherwise, emergency laparotomy is done and the superior mesenteric artery is explored. A Fogarty catheter is introduced and embolectomy is done. These patients may require a second look operation within 24-48 hours to rule out gangrene developing later due to rethrombosis of the artery. 3. Heparinisation-in all cases 4. Other vasodilator agents used are: a. Tolazoline b. Glucagon

Prognosis Majority of the cases present with massive gangrene. Even after massive resection, they succumb to the sepsis and multiorgan failure (Fig. 30.75). See next page for a few causes of intestinal obstruction (Figs 30. 76 to 30.81).

Fig. 30.75: Massive gangrene of small intestines and right side

of the colon due to thrombosis at the origin of superior mesen­ teric artery

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757

A FEW CAUSES OF INTESTINAL OBSTRUCTION

Fig. 30.76: Tubercular stricture-annular stricture

Fig. 30.77: Sigmoid stricture due to carcinoma

Fig. 30.78: Obstructed hernia Fig. 30.79: Nonspecific stricture Fig. 30.80: Peritoneal metastasis causing obstruction opened lesion Fig. 30.81: Laparoscopy detected a lesion in the small intestine

STRICTURES • Common causes are tubercular stricture of the ileum or jejunum in India and Crohn's disease in the Western world.

• Radiation stricture, ischaemic strictures and nonspecific strictures are the other causes. • Small bowel enema or enteroscopy are very useful investi­ gations (Figs 30.82 to 30.85). • Malignant structures

. .. "'�'' ; . .,� , ..

.

f

�

""

•

"'

Fig. 30.82: Enteroclysis showing jejunal narrowing and aortic calcification

Fig. 30.83: Double balloon enteroscopy representation

Fig. 30.84: Double balloon enteroscopy

Fig. 30.85: Balloon enteroscopy showing jejunal ulcers

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NEONATAL INTESTINAL OBSTRUCTION Causes 1. Hirschsprung's disease-congenital megacolon 2. Atresia and stenosis 3. Arrested rotation with bands 4. Volvulus neonatorum 5. Meconium ileus 6. Imperforate anus

Types of Hirschsprung's disease (Fig. 30.87) 1. Ultrashort segment: Anal canal and terminal rectum is aganglionic. 2. Short segment: Anal canal and entire rectum is completely involved. 3. Long segment: Anal canal, rectum and part of colon involved. 4. Total colonic: Anal canal, rectum and whole length of colon is involved.

HIRSCHSPRUNG'S DISEASE: CONGENITAL MEGACOLON It is also called congenital megacolon, aganglionic megacolon or primary megacolon. It is one of the common causes of neo­ natal intestinal obstruction. Pathophysiology (Fig. 30.86) • The disease always involves the anus and rectum wherein parasympathetic ganglion cells are absent in the neural plexus of the intestinal wall. The defect involves internal sphincter. • As a result of this, there is a terminally constricted, non­ relaxing segment, in the rectum and sigmoid (lower part), above which the pelvic colon (sigmoid) is enormously dilated. Rectosigmoid area is involved in 80% of cases. • Circular muscle hypertrophy, mucosa! hyperaemia and ulcers are present in the dilated segments. • In between, there may be a transition zone (cone), which contains a few parasympathetic ganglion cells. • Rarely, Hirschsprung's can also involve the entire sigmoid colon or even the entire colon. • Hirschsprung's rarely occurs in adults also.

Shaded area indicates aganglionic segment

Fig. 30.87: Types of Hirschsprung's disease

Clinical features • Male children are commonly affected, when compared to females. • Incidence: 1 in 4000 to 5000 live births. PEARLS OF ---'7""-7'---

Normal intestine

-----Transition zone (cone) -+--------Terminally constricted segment

Fig. 30.86: Pathophysiology

WISDOM

The most common associated anomaly with Hirschsprung '.5 disease is Down's syndrome (5 to 10%).

• The child presents with acute neonatal intestinal obstruc­ tion as manifested by failure to pass meconium or delay in passing meconium with abdominal distension. • Within 12-24 hours, all features of intestinal obstruction can be found. If it is complicated by enterocolitis, it may result in perforation and septicaemia. A severe diarrhoea with blood and mucus, abdominal distension and vomiting can occur within a few hours, followed by hypovolaemic shock.

Intestinal Obstruction

• Rectal examination reveals that the rectum is empty, finger is gripped by anal sphincter and there is no perianal soiling (Fig. 30.88). On the other hand, in acquired mega­ colon, rectum is loaded with faecal matter, perianal soiling is present and there is no sphincter activity (Fig. 30.89). • Chronic variety: Chronic constipation manifesting in the first few weeks of life. The child may be brought with abdominal distension. Stools are goat pellet-like.

Hard stools

__,.e:::____ Patulous

sphincter

Fig. 30.88: Rectal examination in congenital megacolon

Fig. 30.89: Acquired megacolon

Differential diagnosis (Key Box 30.31) • Acquired megacolon: Usually manifests by one to two years of age. Rectum is loaded with faecal matter. CompI ications • Intestinal obstruction, perforation, peritonitis • Enterocolitis • Growth retardation Investigations 1. Full thickness rectal wall biopsy under GA demonstrates absence of parasympathetic ganglion cells and hypertrophic nerve fibres in the nerve plexus. It should be taken above the anorectal junction. PEARLS OF

WISDOM

Today submucosal suction biopsy is more popular than biopsy since it avoids haemorrhage, infection and scarring. 2. Barium enema: 3.6% solution of barium is used, the

intermediate zone appears as a cone with proximal dilatation and a distal narrow zone which is characteristic of Hirschsprung's disease.

Treatment I. Emergency cases • Right transverse loop colostomy: In most of the cases, aganglionic segment is limited to rectosigmoid region.

759

Mt!ll!,I...........� DIFFERENTIAL DIAGNOSIS

• • • •

Hypothyroidism Meconium plug syndrome Intestinal pseudo-obstruction Colonic neuronal dysplasia

• A full thickness biopsy of the colostomy is sent for histopathological examination. JI. Definitive surgery • Can be done usually between the age of 3 and 6 months (8 to l O kg of weight). • Resection of aganglionic bowel (anorectum) followed by a pull-through procedure. Maintaining continence is the main aim. • A few points of comparison between 'Duhamel's and Swenson's pull-through' are mentioned below. Duhamel's Swenson's I. Endorectal pull-through 1. Retrorectal pull-through 2. Difficult 2. Teclmically easy Steps of Duhamel's pull-through surgery 1. The rectum is transected above the peritoneal fold and is closed. 2. The proximal ganglionic segment is pulled down behind the rectum (retrorectal space created by using blunt dissector). 3. An incision is made in the posterior wall of the anorectum above the dentate line and is deepened through the entire bowel wall. 4. The end of the proximal colon is sutured to the opening in the posterior anal canal all around. 5. The adjacent walls of rectum (posterior wall) and colon (anterior wall) are crushed by using a Kocher's forceps which falls off by itself by the 14th day. 6. The open end of the rectum (above) is closed end to side to the colon. Other types of surgery: Soave's mucosectomy and pull­ through operation. ATRESIA AND STENOSIS • Commonly, it affects the duodenum, followed by ileum and jejunum (Fig. 30.90). • There may be single/multiple atresia. • Incidence: 1 in 10,000 I ive births. PEARLS OF

WISDOM

Duodenal atresia is the most common cause of intestinal obstruction in neonates.

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760

• Clinical features of obstruction manifest within 48-72 hour in the form of obstruction. • Atresia means imperforation; stenosis means narrowing. • Duodenal atresia presents as vomiting with or without bile minimal distension and visible gastric peristalsis. • Jaundice • X-ray abdomen erect: D ouble-bubble in duodena atresia. Treatment

Duodenal atresia: Duodenojejunostomy by anastomosinf dilated duodenum above the atresia to the jejuna! loop. SMALL INTESTINAL ATRESIA Salient features

Fig. 30.90: Ilea! atresia at surgery

DUODENAL ATRESIA (Key Box 30.32)

Types Type I: Complete atresia: It is the commonest atresia. The proximal dilated segment and distal collapsed segment are completely separated. Types 2: There is no separation of the two parts. However, a fibrous band is in between. Type 3: It is incomplete obstruction: There may be a web or stenosis. Windsock deformity: An incomplete diaphragm, with central aperture (hole) with proximal dilatation • Anastomosis: A special anastomosis named after Kimura is being described. It is a diamond shaped anastomosis between upper pouch (duodenum which is opened transversely and lower pouch (distal duodenum) which is opened longitudinally. • Needless to mention here that general condition has to be taken care of with supportive therapy including fluid and electrolytes, total parenteral nutrition, nasogastric decompression and adequate urinary output.

MIIIII,..........�

• It can affect jejunum (common) or ileum • Like duodenal atresia, it can be associated with maternal hydramnios or malrotation of the gut. • Exact reasons for atresia are not known. However, vascular variations in the mesentery such as V-shaped mesentery or due to occlusion of vessels in the intrauterine life are the possible factors. • Like any obstruction, proximal bowel is dilated and distal bowel is collapsed. The colon is very small-microcolon.

GRIES FIELD MODIFICATION OF MARTIN'S CLASSIFICATION

• Type I: Simple stenosis. Mesentery is normal. It is mucosa( atresia (Fig. 30.91). • Type II: Proximal and distal bowel are connected by a fibrous band which has no lumen-atretic segment. Here again, mesentery is normal (Fig. 30.92).

Fig. 30.91: Type I

DUODENAL ATRESIA-ASSOCIATED LESIONS • Annular pancreas, incomplete rotation of the gut • Down's syndrome (trisomy 21)

• Maternal hydramnios • Congenital heart disease • Anorectal malformations

Fig. 30.92: Type II

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761

Figs 30.93A and B: Type Ill

Fig. 30.95: Arrested rotation­ malrotation

Fig. 30.96: Volvulus neona­ torum

• One such band is called transduodenal band of Ladd which compresses the second part of duodenum and gives rise to obstruction (features similar to duodenal atresia). • Differential diagnosis is duodenal atresia. Treatment Laparotomy, division of band and fixation of the caecum in the right iliac fossa.

Fig. 30.94: Type IV

• Type II l: Further divided into 2 types. Illa refers to atresia with V-shaped loss of mesentery in between, IUb refer to complete jejuna) atresia with coiled ileum. It has been called Christmas tree deformity (Figs 30.93A and B). • Type IV: Multiple atretic segments including mesentery (Fig. 30.94). Please note: Apple-peel or Christmas tree deformity. Here, obstruction is usually in the proximal jejunum, which is supplied by the entire superior mesenteric artery (SMA). This results in a gap in the mesentery. So, rest of the small intestine is coiled around the ileocolic branch of the SMA.

Treatment Resection, anastomosis�aim is to conserve as much as possible. ARRESTED ROTATION WITH BANDS • It is a congenital anomaly wherein the caecum and right colon are found on the left side (Fig. 30.95). • As a result of this, there are peritoneal bands which run across from left to right and cause intestinal obstruction (duodenal obstruction).

VOLVULUS NEONATORUM It is a complication of arrested rotation with bands which pre­ disposes to midgut volvulus (small bowel). These unfortunate infants undergo massive resection of the bowel if it is gangrenous (Fig. 30.96). Such massive resections give rise to short gut syndrome. Thus, the patient becomes a digestive cripple. If the loop is not gangrenous, it is treated by laparotomy, untwisting of bowel and division of bands. MECONIUM ILEUS • It is a neonatal manifestation of mucoviscidosis of the pancreas wherein the mucus is thick and viscid. This, along with meconium, produces obstruction. There may be ileal atresia, which might have precipitated meconium ileus (Fig. 30.97). Majority of meconium ileus is coupled by complications such as gangrene, perforation and peritonitis. As a sequel to peritonitis, calcification and adhesive meconium obstruction develop. • Infants present with abdominal distension, bilious vomiting and failure to pass meconium. • Plain X-ray shows distended bowel and mottling due to calcification. • Soap bubble sign or Neuhauser's sign: Ground glass appearance in the right lower quadrant due to viscid meconium mixed with air.

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• Ultrasonography: Dilated loops of bowel filled with echogenic material are highly suggestive of meconium ileus rather than ilea! atresia. • If perforation is ruled out, barium enema can be done which shows microcolon. Treatment I. Conservative treatment It is indicated if there is no peritonitis, general condition of the child is reasonably good or if there is partial obstruction. Dilute gastrograffin is introduced into the colon as enema. It fills up terminal ileum. It absorbs fluid from the interstitial space into the lumen because it is hyperosmolar. Consequently, the meconium becomes soft and it is rejected naturally. Hypervolaemia is to be corrected during this. II. Nonresectional procedures A. Bishop-Koop operation (Fig. 30.98) • Ileum is divided in the proximal healthy part. This proximal ileum is anastomosed to the ascending colon to relieve obstruction-end-to-side anastomosis. • Distal ileum containing thick meconium pellets is brought outside as a fistula and regular saline washes are given to dilute the meconium. Mucous fistula needs to be closed after a few weeks. B. Santulli operation (Fig. 30.99) • In this operation, proximal ileum is brought out as ileostomy.Distal ileum is anastomosed to proximal ileum as end-to-side anastomosis. Ill. Resection Resection is the surgery of choice today as more cases present with short segment obstruction. Long-term results and complications are much less than nonresectional procedures. ANORECTAL ANOMALIES Developmental anatomy • To sta1t with, there is a common chamber called cloaca, which is later divided into 2 chambers, anteriorly allantois

Fig. 30.97: Meconium ileus Fig. 30.98: Bishop-Koop operation

Distal ileum brought out as ileostomy

Fig. 30.99: Santulli operation

gives rise to urinary bladder and posteriorly postallantoic gut gives rise to rectum and upper 2 cm of anal canal. • Postallantoic gut fuses with proctodeum, thus giving rise to anal canal. If there is a defective fusion of this, it results in imperforate anus. IMPERFORATE ANUS Incidence 1 :4500 live births. Common in female children. Types of imperforate anus I. Low anomalies: [t refers to termination of the bowel below the anorectal bundle. 1. Covered anus (Fig. 30.1OOA) The anal orifice is covered by a tag of skin. 2. Membranous anus (Fig. 30.1OOB) Covered with a thin membrane 3. Anterior ectopic anus (Fig. 30.1OOC) Anus is situated anteriorly 4. Stenosed anus (Fig. 30.1OOD) Anal orifice is microscopic II. High anomalies: They are supralevator-the bowel terminates above the anorectal bundle. 1 . Anorectal and agenesis, with fistula: Rectovesical, rectovaginal, rectourethral fistulae (Fig. 30.101A). 2. Rectal atresia: Colon ends as a blind pouch below (Fig. 30.10 lB). 3. Cloaca: In this variety, bowel, urinary and genital tracts open into a common chamber. This occurs in females only. Diagnosis-Wangensteen's invertogram 12 hours after birth, the child is held upside down (] 2 hours is the time for the gas shadow to reach the distal portion of the gut). A metal coin is strapped to the site of anus and X-ray is taken. If the gas shadow is above the pubococcygeal

Intestinal Obstruction

763

B

Figs 30.101A and B: High anorectal anomalies

D

Figs 30.1OOA to D: Low anorectal anomalies

line, it is a high anomaly. If the distance between the coin and gas shadow is more than 2.5 cm, it is a high anomaly. If the gas shadow is below the pubococcygeal line, it is a low anomaly. Pubococcygeal line is called as Stephen's line. Treatment I. Low anomaly: Easy to treat, division of membrane or skin followed by dilatation is all that is required with some amount of plastic reconstruction (anoplasty is necessary). II. High anomaly: Repaired by 3-stage procedure: • 1st stage: Preliminary transverse colostomy to relieve intestinal obstruction. • 2nd stage: When the child is 8-IO kg of weight, a "pull­ through" operation is done with division of fistula. • 3rd stage: After 2 months, colostomy is closed. CAUSES OF INTESTINAL OBSTRUCTION AS PER AGE Neonates: Oto 7 days (Figs 30.102 to 30.109) 1. Atresia and stenosis 2. Hirschsprung's disease 3. Arrested rotation with bands 4. Volvulus neonatorum 5. Meconium ileus 6. lmperforate anus Young patients: Up to 30 years 1. Obstructed hernia 2. Adhesions 3. Tuberculous stricture ileum 4. Crohn's disease of ileum 5. Tuberculous peritonitis with adhesions 6. Meckel's diverticulum with band 7. Adult intussusception

Middle-aged patients: 30-60 years 1. Adhesions 2. Obstructed hernia 3. Carcinoma left colon 4. Diverticulosis with stricture left colon 5. Gall stone ileus 6. Sigmoid volvulus 7. Mesenteric vascular occlusion 8. Adult intussusception FOOD BOLUS OBSTRUCTION This complication can occur, particularly when a GJ or partial gastrectomy is done. Factors precipitating this condition • Unmasticated, undigested particles • Coconut pieces, jackfruit seeds and gulped coins, etc. • They get impacted in the terminal ileum which is the narrowest portion of the gut. Treatment Squeeze the bolus into the caecum. Otherwise, enterotomy and removal may be necessary. OBSTRUCTION DUE TO INTERNAL HERNIA Syn: Stammer's hernia 1. These are rare causes of intestinal obstruction. Due to some congenital defect in the mesentery, the floating, mobile intestines can herniate (Key Box 30.33). The defect may be in: • Mesentery • Transverse mesocolon or • Broad ligament • F oramen of Winslow Hence, whenever a surgical procedure is done for resection of the bowel or GJ, etc. Once the anastomosis is completed, the rent in the mesocolon as in GJ or rent in the small bowel mesentery should be closed. 2. Herniation can also occur through one of the potential spaces (fossae) in and around a viscus.

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Fig. 30.102: Worm ball obstruction

Fig. 30.103: Anorectal malformation

Dialated zone

Transition zone

Fig. 30.104: lleal obstruction Fig. 30.105: Double­ bubble appearance

Fig. 30.108: lleocaecal intussusception

Fig. 30.106: Hirschsprung's Fig. 30.107: Various segments of the large intestine in Hirschsprung's disease disease

Fig. 30.109: Necrotising enterocolitis with patchy gangrene

( Courlesy: Professor Vijaykumar, Department of Paediatric Surgery, KMC, Manipal)

765

Intestinal Obstruction

Duodenal fossa • Left paraduodenal fossa: Inferior mesenteric vein lies very close to the free border here. • Right duodenojejunal fossa: Superior mesenteric artery runs in its free border. Colonic fossa • Superior ileocaecal fossa • Inferior ileocaecal fossa • Sigmoid fossa PEARLS OF WISDOM

These are the rare causes of intestinal obstruction to be kept in mind. PARALYTIC ILEUS (NEUROGENIC ILEUS) In this condition, there is a failure of transmission of para­ sympathetic impulses from one segment to the other. That means, there is a failure of parasympathetic mechanism, :¥hich results in paralysis of bowel. It gives rise to a large collection of fluid and gas within the bowel resulting in distension. Causes 1. Postoperative: Exposure of the intestines, handling of the intestines, contamination with blood, foreign body, etc. causes temporary suppression of parasympathetic activity and results in paralytic ileus. This gets aggravated by allowing oral fluids too early. 2. Following peritonitis Pus is the chief cause. Bile, blood, etc. are other causes. Bacterial toxins also prevent movement of the bowel. 3. Reflex: Following fracture spine, retroperitoneal haemor­ rhage, etc. 4. Hypokalaemia: K+ is the chief intracellular ion of all cells including muscles. Hypokalaemia causes generalised muscle weakness including muscles of the bowel. Clinical features I. Abdominal distension is gross. Tympanitic note all over is a feature. Respiratory and cardiac functions are impaired. 2. No colicky pain abdomen (in dynamic obstruction, colicky pain is a feature). Dull pain occurs due to distension of the abdomen. 3. Failure to pass flatus, effortless vomiting is also characteristic of paralytic ileus. 4. On auscultation, tinkling sounds are heard due to shift of fluid from one coil of bowel to the other (Key Box 30.34). 5. Severe fluid, electrolyte and protein depletion occur.

MHll!,I..........� I NTERNALHERNIA

• Wherever a defect is present which is an anatomically present recesses or congenital or following surgery, loop of small intestine can herniate resulting in hernias. • They are difficult to diagnose. The diagnosis is by exclusion • Triggering factors: Inflammation in the vicinity with or without an adhesion and band trigger herniation and complications. • Most of these cases present as acute abdomen with features of intestinal obstruction. • CT scan is the best investigation in such cases • It should be remembered that an important vessel runs in the close vicinity of these sites in most of the cases. • Treatment is reduction, suturing the defect or resection anastomosis if the intestine is gangrenous taking care not to damage the vessel. Decompression without dividing the constricting ring may be required.

11111!.I.........� THREETYPES OFBOWELSOUNDS

• Normal: Once in 20 seconds, low pitch • Borborygmi: Loud , noisy, which occurs once in 5-1 O seconds. • Tinkling sounds: Mild metallic sounds as in paralytic ileus

Investigations 1. Plain X-ray abdomen erect demonstrates distended loops of bowel (Fig. 30.110). 2. Electrolyte study and correction of any abnormality. Treatment • Basic principle of treating paralytic ileus is drip and suction. • The cause of paralytic ileus has to be treated first, e.g. if there is hypokalaemia, supplement potassium. If there is pus in the peritoneal cavity drain it. • Ryle's tube aspiration, to give rest to the gut. • Intravenous fluids, supplementation of ions, correction of dehydration, oliguria, etc. Such treatment is continued for 3-4 days. • Ryle's tube is removed when abdomen is soft, bowel sounds are heard and patient has passed flatus. Clear oral fluids are started for 2-3 days followed by soft diet. Small bowel activity returns within 12-18 hours, followed by colon which starts functioning within 36-48 hours. However, gastric functions may return ranging from 18 hours to 4 days. PSEUDOINTESTINAL OBSTRUCTION Acute colonic pseudo-obstruction (ACPO) is also called Ogilvie's syndrome. It is massive colonic distension in the absence of a mechanically obstructing lesion.

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CAUSES OF COLONIC PSEUDO-OBSTRUCTION�

1. Retroperitoneal irritation • Blood • Urine • Fracture spine and pelvis

2. Drugs • Levodopa • Tricyclic antidepressants 3. Metabolic • Uraemia • Diabetes • Myxoedema • Hypokalaemia 4. Viral infections

Fig. 30.110: Plain X-ray abdomen showing dilated bowel

loops

Pathogenesis It occurs mainly due to malfunctioning of sacral para­ sympathetic nerves (S2-S4). It results in atony of the descending colon resulting in functional obstruction. It is interesting to note that the junction of the dilated and collapsed bowel is near the splenic flexure. This is the place wherein parasympathetic supply by vagus ends and sacral autonomic nervous system starts. An increased sympathetic tone results in colonic dilatation due to inhibition of contraction (Key Box 30.35).

• Rarely, even after colonoscopic decompression, caecal tenderness continues. If distension persists, laparotomy followed by tube caecostomy may have to be done. INTESTINAL OBSTRUCTION-SPECIAL CAUSES 1. Adhesive obstruction • Firstly, strangulation should be ruled out by clinical and radiological tests. Extended nonoperative therapy may be advised, e.g. if one can wait for 48 hours in a case of intestinal obstruction. In these cases 4-6 days of waiting is sometimes worth, especially in a patient who has been operated many times earlier. • During this extended period, careful monitoring is important to look for any new symptoms/signs of strangulation. • Early postoperative adhesions (bread and butter adhesions) can also be given an extended nonoperative therapy because mostly it is partial obstruction.

Clinical features • Elderly bedridden patient with cardiac/lower respiratory illness are the victims. Aerophagia and drugs which decrease colonic mobility are precipitating factors. • Failure to pass faeces and flatus for several days. • Tachypnoea due to elevation of the diaphragm due to distended colon is common. • Rectal examination reveals some faeces (in cases of mechanical obstruction, rectum is empty). • Plain X-ray abdomen erect may or may not show one or two air fluid levels. Distension is mainly colonic. • Carcinoma colon is to be differentiated by barium enema. • Caecal perforation is a dangerous complication. Hence, look for right iliac fossa of tenderness.

2. Intestinal obstruction in Crohn's disease • As far as possible, resection should be avoided in Crohn 's disease because the aim is to save as much as possible. • 30% of patients eventually develop obstruction which requires resection. • Again, if possible do strictureplasty • Strictureplasty should not be done in patients with intra­ abdominal abscesses or intestinal fistulae. • Some cases of Crohn 's obstruction also respond well to medications-one more reason for nonoperative treatment.

Treatment • It is conservative, provided acute abdomen is ruled out. • Colonoscopic decompression is the method of choice. • Prokinetic drugs such as cisapride or mosapride have been tried in selected cases.

3. Intestinal obstruction in pregnancy • The commonest cause of intestinal obstruction in pregnancy and puerperium is adhesive bands. • Volvulus is the second commonest cause of intestinal obstruction-intestine volvulates around an adhesive band

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767

• The diagnosis and treatment of a pregnant patieni suspected of having a bowel obstruction should be no different from those given to a nonpregnant one.

4. lleosigmoid knotting It is rare type of knotting between sigmoid and ileum carrying significant mortality if not treated timely (Key Box 30.36). 5. Iliac crest graft hernia • This is also a rare hernia following bone graft removal from the iliac crest for treatment of fractures.

MMffl,I...........� Fig. 30.111: Pregnant lady with adhesions causing intestinal obstruction

• •

• • •

•

•

• • • •

and that is why the primary cause is adhesive bands (Fig. 30.111). Inguinal hernia and intussusception are the other causes. The consequences of intestinal obstruction in pregnancy do not differ from those found in nonpregnant women except that in the pregnant patient, a second entity, the foetus, is also threatened. Most of the intestinal obstructions tend to occur in the third trimester. Maternal mortality for intestinal obstruction in pregnancy (10-33%) is higher than in nonpregnant patients. In the first half of the pregnancy, nausea, vomiting and episodes of constipation are quite common. Hence, they are confused for hyperemesis gravidarum, acute duodenal ulcer and gastritis. In the second half of the pregnancy, symptoms can be confused for toxaemia, constipation, Braxton Hicks contractions, etc. Ultrasound can help in the diagnosis by detecting dilated intestinal loops, fluid in the peritoneal cavity and intus­ susception. It also helps to rule out ovarian torsion, gall stone disease, etc. Premature labour can be prevented with tocolysis (abolition of uterine contraction). Abdominal surgery in the third trimester does not induce labour. Negative laparotomy carries a small risk of disturbing the pregnancy. Volvulus and intussusception are the two major causes of small intestinal obstruction in pregnancy. Both can give rise to gangrene. Hence an intervention is required before gangrene sets in.

ILEOSIGMOID KNOTIING • It is also called compound volvulus. • Predisposing factor is long pelvic mesocolon. • The ileum twists around the sigmoid colon resulting in gangrene of ileum or sigmoid colon or both. • Even though sigmoid is also twisted, features are similar to small intestinal obstruction-not massive distension as seen in sigmoid volvulus. • Resection anastomosis of gangrenous segment followed by anastomosis of the bowel (ileoileal/colic and colocolic).

• Iliac crest is the common donor site of bone graft. If the bone graft removed is large, the intestines can herniate resulting in intestinal obstruction. • Tenderness in the surgical site scar can cause diagnostic difficulty. • It is confused for haematoma • CT scan gives the diagnosis Following is the case report of a patient who had undergone iliac crest bone graft and developed hernia (Figs 30.112 to 30.114). 6. Acute large bowel obstruction Details about the causes of large bowel obstruction, patho­ physiology and the treatment is given in Chapter 29. However a few important points have been given in ten commandments.

A 50-year-old man presented to the casualty with abdominal pain, vomiting and distension. Features were suggestive of intestinal obstruction. On examination, there was a buldge in the region of right iliac fossa-more lateral. T here was a scar of bone graft incision. On questioning the patient he says that he hadfracture humerus. Two months back, nailing and bone grafting had been done. Ultrasound revealed bowel loops. CT scan showed a large bone graft defect with herniation of intestines resulting in intestinal obstruction. Emergency surgery, reduction of hernia contents and mesh repair was done. Recovery was uneventfid.

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TEN COMMANDMENTS

Fig. 30.112: Iliac bone graft site hernia

1. Should rule out pseudo-obstruction before exploring the abdomen. 2. Should do limited contrast study or CT scan 3. Should resuscitate the patient before surgery 4. Should take into account, the general condition of the patient before resection anastomosis. 5. Should consider right hemicolectomy or extended hemicolectomy for right-sided growth which is operable. 6. Should consider single stage resection anastomosis also for left-sided growth provided general condition of the patient is good and on table lavage is given before the anastomosis. 7. Should consider exteriorisation of gangrenous bowel in a very sick patient. 8. Should consider a simple diversion colostomy in moribund patients in rectosigmoid obstructions (it is a common problem). 9. Should not do anastomosis in cases with faecal contamina­ tion, peritonitis, haemodynamic instability or possible ischaemia of the remaining colonic segments. 10. Should mark the probable stoma site

MALROTATION AND MIDGUT VOLVULUS

Fig. 30.113: Caecal herniation

Fig. 30.114: Caecal hernia through iliac bone graft defect-mesh

repair

Introduction • The incidence of malrotation is 1 in 500 infants. • The male to female ratio is 2 : 1. • Malrotation with midgut volvulus may become rapidly life­ threatening. The previously healthy infant with bilious vomiting is a characteristic presentation of malrotation. Usually it presents with bilious vomiting, failure to thrive and features of intestinal obstruction (Fig. 30.115). • In approximately 60% of patients, malrotation presents by one month of age. Another 20-30% of patients present at age 1-12 months. Thereafter, it can present at any age, and is seen in adults and even the elderly. Basic pathophysiology • A volvulus is a complete twisting of a loop of intestine around its mesenteric attachment site. This can occur at various locations of the GI tract, including stomach, small intestine, caecum, transverse colon, and sigmoid colon. Midgut malrotation refers to twisting of the entire midgut about the axis of the superior mesenteric artery (SMA). Malrotation is any deviation from the normal 270 ° counterclockwise rotation of the bowel that occurs during embryogenesis (Figs 30.115 and 30.116). • At the fourth week of gestation, the gastrointestinal system is a straight tube centrally located in the abdomen. During the ensuing 8 weeks, the midgut rotates and becomes fixed to the posterior abdominal wall. Arrest of development at any stage narrows the mesenteric base and impairs fixation, leaving the bowel at high risk for volvulus.

Intestinal Obstruction

769

over (but more reassuringly lateral to) the left vertebral pedicle and at the same height as the duodenal bulb on a well-centred view. If the DJJ does not meet these twc criteria, malrotation is diagnosed. • Signs of midgut volvulus include an abrupt termination, or break of the contrast column and the corkscrew (apple peel, or barber pole) sign. • On ultrasound (US) and computed tomography (CT), the superior mesenteric artery (SMA) and superior mesenteric vein (SMV) relationship may be reversed. • Normally, the SMV is to the right of the SMA; with malrotation, the SMV may occupy a position directly anterior or to the left of the SMA.

Fig. 30.115: Midgut volvulus: (A) Clockwise rotation resulting in superior mesenteric ischaemia shown in red line, (B) Anticlockwise untwisting to be done at surgery is shown as green line

Treatment • The Ladd procedure remains the cornerstone of surgical treatment for malrotation today. • A classic Ladd procedure is described as reduction of volvulus (if present), division of mesenteric bands, place­ ment of small bowel on the right and large bowel on the left of the abdomen, and appendicectomy. • A laparoscopic variation of the Ladd procedure has been used in some centres, with the general advantage of decreased adhesions and scarring, but good visualisation of the entire bowel is necessary. Laparoscopy is a particularly suitable investigative procedure for children presenting with acute abdomen. 'Clues' to the causes of intestinal obstruction are mentioned in Key Box 30.37.

•,•.1...........� • • • •

Fig. 30.116: Malrotation with band

• The resultant shortened mesenteric pedicle predisposes to midgut volvulus, a clockwise rotation around the superior mesenteric artery axis that can lead to bowel ischaemia. Investigations • Conventional radiographs are neither sensitive nor specific for malrotation. • On the upper GI series, it is crucial to locate the position of the duodenojejunal junction (DJJ). The DJJ must be at least

INTESTINAL OBSTRUCTION 'Clues' to the causes of obstruction

Operative scar Groin swelling Gross distended intestinal loop Step ladder peristalsis

• Red currant jelly stools • Deformed umbilicus in adult child • Melanosis of lips, mucosa • H/o constipation/bleeding per rectum • Emaciated, young patient with loose stools/weight loss/fever • Elderly, hypertensive severe abdominal pain, tachycardia, tachypnoea, acidotic breathers

-Adhesions -Obstructed hernia - Sigmoid volvulus -Small intestinal obstruction -lntussusception -Meckel's diverticulum with band -lntussusception in adults (PJ syndrome) - Colonic obstruction (cancer) -lleocaecal tuberculosis TB stricture -Mesenteric vascular occlusion

No wonder 'a surgeon should have an eagle eye'!

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INTERESTING 'MOST COMMON' IN INTESTINAL OBSTRUCTION • Most commonly encountered disorder of intestines is intestinal obstruction. • Most common cause of small intestinal obstruction is intra­ abdominal adhesion. • Most commonly performed, simple diagnostic investigation in case of obstruction is plain X-ray abdomen in erect position. • Most common abdominal symptom of intestinal obstruction is colicky abdominal pain. • Most commmon congenital anomaly of small intestine is Meckel's diverticulum. • Most common presentation of Meckel's diverticulum in children is bleeding. • Most common cause of intestinal obstruction in infants between 6 and 18 months is intussusception.

WHAT IS NEW IN THIS CHAPTER?/ RECENT ADVANCES

• All topics have been updated • Malrotation and special causes of intestinal obstruction have been added. • Atresias have been updated • Iliac crest graft hernia and ileosigmoid knotting have been added. • Ten commandments of acute large bowel obstruction have been added.

MULTIPLE CHOICE QUESTIONS 1. Which of the following is true for closed loop obstruction? A. Can occur with constrictive growth in the hepatic flexure B. Ileocaecal valve is incompetent C. Perforation of the sigmoid colon is common D. Occurs with partial obstruction 2. Which of the following is not the cause of gangrene in intestinal obstruction? A. Ileocaecal tuberculosis B. Mesenteric vascular occlusion C. Necrotising enterocolitis D. Volvulus

6. Features of strangulation includes all of the following except: A. Tachycardia B. Disappearance of pain abdomen C. Fever D. Acidosis 7. The features of viable bowel includes all of the following except: A. Normal peristalsis B. Normal pulsations are visible C. Normal pink colour is present D. Peritoneal sheen is absent

3. Faeculent vomiting is pathognomonic of: A. Jejuna! obstruction B. Terminal ilea! obstruction C. Duodenal obstruction D. Colonic obstruction

8. Conservative treatment is advocated in intestinal obstruction when there is: A. Disseminated malignancy with obstruction B. Complete obstruction with adhesions C. Postoperative obstruction with peritonitis D. Crohn's disease unresponsive to medications

4. Cardinal features of intestinal obstruction include all of the following except: A. Colicky abdominal pain B. Vomiting C. Diarrhoea D. Abdominal distension

9. 'Bent inner tube design', 'Omega sign', 'Bird's beak design' are all seen in: A. Sigmoid volvulus B. Caecal volvulus C. Meckel's diverticulum D. Bascule

5. The following is true in a plain X-ray of abdomen in intestinal obstruction: A. Caecum can appear as round shadow B. Ileum has valvulae conniventes C. Colon has haustrations D. Sigmoid appears shapeless

10. The most common cause of intestinal obstruction in infants aged 6-18 months is: A. Worms B. Bands C. Intussusception D. Adhesions

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771

12. Dance's sign (signe de dance) is a feature of: A. Worms B. Bands C. Intussusception D. Adhesions

19. Common factor precipitating sigmoid volvulus in patients with parkinsonism, multiple sclerosis, hypothyroidism is: A. Diarrhoea B. Constipation C. Drugs D. Long mesentery of the colon

13. The investigation of choice in mesenteric vascular occlusion is: A. Ultrasound abdomen B. Plain X-ray abdomen C. CT with or without angiogram D. MRI

20. Investigation of choice for detecting bleeding Meckel's diverticulum is: A. CT scan B. Pet scan C. MRI scan D. Technetium scan

14. The most common cause of intestinal obstruction in neonates is: A. Bands B. Duodenal atresia C. Imperforate anus D. Meconium ileus

21. Presence of intramural air is diagnostic of: A. Gall stone ileus B. Sigmoid perforation C. Duodenal atresia D. Intestinal gangrene

11. Red currant jelly stools are characteristic of: B. Bands A. Worms C. Intussusception D. Adhesions

15. The most common congenital anomaly of small intestine is: A. Bands B. Duodenal atresia C. Stenosis D. Meckel's diverticulum

22. Following complications can occur after gastro­ jejunostomy except: A. Dumping syndrome B. lntussusception C. Volvulus D. Stomal ulcer

16. Features of paralytic ileus include the following except: A. Gross abdominal distension B. Pain abdomen C. Failure to pass flatus D. Tinkling sounds

23. Following are the causes of adult intussusception except: A. Meckel's diverticulum B. Submucous lipoma C. Carcinoma caecum D. Hypertrophy of Peyer's patches

17. Melanosis of lips and mucosa with intestinal obstruction should arouse the suspicion of: A. Gardner's syndrome B. Turcot's syndrome C. Peutz-Jeghers syndrome D. Down's syndrome

24. The most common anomaly associated with Hirschsprung's disease is: A. Down's syndrome B. Hypothyroidism C. Meckel's diverticulum D. Anorectal atresia

18. Which is the factor precipitate sigmoid volvulus? A. Short colon B. Broad attachment at the base C. Empty colon D. Long mesentery of the colon

25. Following are causes of paralytic ileus except: A. Anastomotic leak B. Retroperitoneal irritation C. Hyperkalaemia D. Fracture spine

ANSWERS A

2 A

3 B

4 C

5 A

6 B

7 D

8 A

9 A

10 C

11 C

12 C

13 C

14 B

15 D

16 B

17 C

18 A

19 B

20 D

21 D

22 B

23 D

24 A

25 C

31 Rectum and Anal Canal • • • • • • • •

Surgical anatomy Carcinoma rectum Prolapse rectum Surgical anatomy of anal canal Haemorrhoids Anorectal abscess Fistula in ano Fissure in ano

• VAAFT

• • • • • •

• Valves of Houston: Despite the name rectum means straight, it is never straight in adults. It has one convexity on the left and two convexities on the right side. There are 3 valves of Houston (prominent mucosa! folds), two on the left and one on the right. • That portion of the rectum resting on the pelvic floor is called ampulla-dilated portion of the mid rectum.

SURGICAL ANATOMY OF THE RECTUM The rectum starts at the rectosigmoidjunction, opposite the third piece of sacrum. It descends in the sacral hollow, passes through the pelvic floor, and ends in the anorectal junction, which is about 4 cm away from the anal verge. Anorectal junction is enclosed by puborectalis muscle posteriorly and in the lateral aspects. The rectum is 12-15 cm in length (Key Box 31.1).

Pilonidal sinus Sacrococcygeal teratoma Malignant tumours of anal canal Stricture of anal canal and rectum Anal incontinence What is new?/Recent advances

Arterial supply

Peritoneal covering • Upper one-third is completely covered by peritoneum (> 11 cm from anal verge) (Figs 31.1 and 31.2) • Middle one-third is covered in front and lateral aspects (6-11 cm). • Lower one-third (0-6 cm) has no extraperitoneal covering but has two fascia! condensation layers. Posteriorly, the strong Waldeyer's layer separates the rectum from lower sacral pieces and coccyx. At surgery, stripping of this fascia results in uncontrollable bleeding from sacral plexus of veins, which is underneath the Waldeyer's fascia. • Anteriorly, the weak Denonvillier's fascia separates the rectum from prostate and bladder. Stripping of this fascia results in troublesome bleeding from prostatic venous plexus. • Rectum is attached to side wall of pelvis by lateral ligaments, which contain middle haemorrhoidal vessels. These need ligation or coagulation during mobilisation of lower rectum.

772

1. Superior haemorrhoidal artery is a branch of the superior rectal artery which is the continuation of the inferior mesenteric artery. It divides into right and left branches. The right branch divides into anterior and posterior branches which supply the rectum (Fig. 31.3).

...

.......................... .

KEY BOX 31.1

SOME INTERESTING FEATURES OF RECTUM�

1 . Rectum means straight but it is not 2. Coverings of the peritoneum different in different levels 3. Eventhough it is a part of the large intestine, taenia, appendices epiploicae and sacculations are absent. 4. Middle curve marks the anterior peritoneal reflection. It is about 12-15 cm above anus. 5. Rectal carcinoma has high recurrence because of lack of serosal layer and close relation to other pelvic viscera. 6. Principal route of lymphatic drainage is upwards towards para-aortic nodes.

Rectum and Anal Canal

773 Rectum

Sacrum

Rectovesical pouch

•

• • • • • • • •

After investing the upper rectum, pelvic peritoneum is reflected anteriorly in males urinary bladder-thus form rectovesical pouch and in females it reflects onto uterus and thus form rectouterine pouch. It is one of the sites of transcoelomic spread of malignant cells. Maligant cells settle down in this most dependent part of the abdominal cavity and grow. They are palpable by rectal examination, a shelf like feeling-popularly called Blumer's shelf. Thus, if per rectal or per vaginal examination findings suggest presence of Blumer's shelf-it means hard deposits are felt and the case is inoperable. Rectovesical pouch is also the site of pelvic abscess It is diagnosed by per rectal or per vaginal examination Pus can be drained through rectum or through posterior fornix. Aspiration of blood from rectovesical pouch through posterior fornix also indicate intraperitoneal bleeding-may be ruptured ectopic.

2. Middle haemorrhoidal artery, a branch of internal iliac artery, runs in the lateral ligament of the rectum. 3. Inferior haemorrhoidal artery, a branch of internal pudenda! artery supplies the lower rectum. Venous return (Fig. 31.4) The rich submucous plexus of veins surrounding the ampulla forms external rectal plexus. The venous drainage from here flows in two directions. 1. Upwards to drain into superior rectal veins. These join inferior mesenteric veins, which in turn drain into the portal system.

Area covered with ---+­ peritoneum

Urinary bladder

Perineal---membrane Urethra--� Perinea! body

Fig. 31.1: Sagittal section through the male pelvis showing the location of the rectum and some of its anterior and posterior relations

2. Across to drain into middle rectal veins, which run in the lateral ligament of the rectum along with middle rectal artery. Hence, the lateral ligaments have to be ligated and divided during resection of rectum. These veins drain into internal iliac veins (systemic circulation). Hence, rectum is a site of portosystemic anastomosis. Lymphatic drainage of rectum (Fig. 31.5) • Upper 113rd of rectum is completely enclosed by peritoneum and the middle I/3rd of rectum is covered in front and on the sides by peritoneum. From these areas, lymphatic drainage always occurs in the upward direction, first to (A) pararectal nodes of Gerota followed by superior haemorrhoidal nodes, middle haemorrhoidal nodes and nodes at the origin of inferior mesenteric artery. • From lower I/3rd of rectum, lymphatics spread in the lateral direction and can involve (B) internal iliac nodes.

Area not covered with peritoneum

�

Upp" 113,d of cectom Peritoneum

Middle 113rd of rectum � '------ Peritoneum

A

8

@-

Figs 31.2A and B: Peritoneal relations of the rectum

Lower 113rd of rectum

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Manipal Manual of Surgery

Portal vein----""""="-"'=-= Superior haemorrhoidal artery

2 convexities to the left

Fig. 31.3: Arterial supply of rectum

Fig. 31.6: Digital rectal examination

Fig. 31.4: Venous return

Fig. 31.7A: Proctoscopy

• Lymph nodes are also present in the hollow of sacrum along median sacral artery (C). Lymphatics are present in the muscularis mucosa. Nerve supply • Sympathetic: The fibres come from hypogastric plexus, which is located at the aortic bifurcation at the level of L 5 _ Injury to this can cause absence of erection or dry orgasm. Fibres also come along with inferior mesenteric artery and superior rectal artery. • Parasympathetic: (S2, S3, S4) by means of nervi erigentes from the hypogastric plexus and supply motor fibres to detrusor. Pain and ability to distinguish flatus and faeces is because of these fibres. Loss of mucosa of the rectum results

Fig. 31.5: Lymphatic drainage

Fig. 31.78: Sigmoidoscopy

Fig. 31.8: Colonoscopy

in the loss of these sensations. During division of lateral ligaments or during anterior dissection of the bladder base, injury to nervi erigentes can occur. • External anal sphincter and puborectalis are innervated by inferior rectal branches of internal pudenda! nerve. Examination of rectum and anal canal (Table 31.1) Figure 31. 9 shows common diseases of ano rectum. CARCINOMA RECTUM Aetiopathogenesis Similar to carcinoma colon. However, precancerous conditions and risk factors are given as follows.

Rectum and Anal Canal

Haemorrhoids-painless

775

Anal fissure-painful

Thrombosed external haemorrhoids-painful

Perianal abscess-painful

Fistula in ano--painless/painful

Perianal warts-painless

Rectal prolapse-painful/painless

Pedunculated polyp-painless

Carcinoma anal canal-painless

Carcinoma rectum-painless

Fig. 31.9: Common diseases of ano rectum These are the common anal/perianal conditions which can be diagnosed by inspection (only), or palpation or per rectal examination/ proctoscopic examination

776

Manipal Manual of Surgery Examination of rectum and anal canal

Digital rectal examination (Fig. 31.6)

Proctoscopy (Kelly's) (Fig. 31.7)

Flexible sigmoidoscopy (Fig. 31.8)

•

•

•

• •

• •

Lubricate the finger with lignocaine jelly Sim's position: Left lateral is ideal position Explain to the patient what you plan to do

•

Gently apply pressure on the external sphincter (anal opening) and slowly introduce the finger

•

Polyps, carcinomatous growths, strictures, thrombosed piles can be felt

• •

Poor sphincter tone as in prolapse rectum, spinal cord paralysis

•

Do a per rectal examination and rule out painful condition (Fissure in ano) Proctoscope with obturator is introduced to full length Piles are seen bulging into lumen as obturator is withdrawn Biopsy from growth can be taken Piles can be injected with sclerosants Pelvic abscess can also be drained

Flexible sigmoidoscope is 60 cm long • An enema is given before procedure • Growth, ulcers, bleeding diverticulae, polyps, colitis can be diagnosed and biopsy can be taken • Do not force instrument-it may perforate colon • It may deflate and derotate sigmoid volvulus

Fig. 31.10: Annular constricting lesion-patient presents with colonic obstruction

Fig. 31.11: Fibreoptic sigmoidoscopy showing growth in the upper rectum-biopsy proved signet ring carcinoma

Precancerous conditions • P olyps in FAP, villous adenoma (see page 704) • Ulcerative colitis (see page 677) • Crohn's disease (see page 683)

4. Diffuse variety is similar to linitis plastica. It develops from ulcerative colitis. It has a poor prognosis. 5. Colloid variety is rare. The tumour contents are gelatinous due to increased mucus production. This variety is seen in young patients. The cell is filled with mucus and nucleus is displaced. It is called 'Signet Ring' carcinoma. It is associated with poor prognosis (Fig. 31.12).

Risk factors • Smoking, Alcohol, Diet (see page 709) • Genetic • Colorectal family (see page 705, 706) Pathological types 1. Annular variety is common at the rectosigmoid junction. It presents with constipation and intestinal obstruction. It takes about a year for the growth to completely encircle the lumen of the gut (napkin ring deformity) (Fig. 31.10). 2. Polypoidal lesions are common in the ampulla of the rectum (Fig. 31.11). 3. Ulcerative lesions can occur anywhere in the rectum with raised edges and the growth occurs in the transverse direction.

Clinical features of carcinoma rectum • Constipation requiring increasing doses of purgatives due to annular growth at rectosigmoidjunction. Always a sense of incomplete evacuation and altered bowel habits. • Bleeding per rectum, frank blood or mixed with stools is common. It is painless, never massive and is the earliest symptom of carcinoma rectum. Very often, it is confused for haemorrhoids. • Early morning spurious diarrhoea is due to accumulation ofmucus overnight in the ampulla ofrectum (dilated middle portion of rectum), which causes an urgency to pass stools but results in passage of only mucus with minimal stools. There is always a sense of incomplete defaecation.

Rectum and Anal Canal

777

Clinical examination

Fig. 31.12: Signet ring carcinoma colon-relatively poor prognosis ( Courtesy: Dr Laxmi Rao, Head, Department of Pathology, KMC, Manipal)

• Tenesmus - Painful, incomplete defaecation associated with bleeding is called tenesmus. - This symptom is common with stricturous growths. • Bloody slime (Key Box 31.3): An attempt at defaecation results in mucus mixed with blood. • Loss of appetite, loss of weight due to liver secondaries, abdominal distension due to obstruction are late features. ABCDEF of rectal carcinoma-symptoms 1. Altered bowel habits 2. Bleeding per rectum and bloody slime-upper rectum. 3. Constipation increasing-annular carcinoma at rectosigmoid junction. 4. Incomplete defaecation 5. Early morning spurious diarrhoea-midrectum 6. Fatigue, weight loss

11:!tiiiWW'� SYMPTOM

• • • • • • • • •

Constipation Bleeding Tenesmus Early morning spurious diarrhoea Bloody slime Sciatic-like pain Abdominal distension Loss of weight/ abdominal distension Strangury

1. Rectal examination: In every patient with bleeding per rectum, rectal examination has to be done. More than 90% of cases of carcinoma rectum can be diagnosed by rectal examination. Always feel for the ulcer or growth, nodularity, induration, fixity to posterior sacrum, anterior bladder base and laterally to lateral ligaments. Look for the blood stains specially in ulcerative cases. It is also possible to feel the lymph nodes in the mesorectum in cases of lower third carcinomas. 2. Vaginal examination: When the growth is situated in the anterior wall of the rectum, accurate assessment of the growth can be done with one finger in the rectum and the other in the vagina. Large Krukenberg tumours if present can also be felt by vaginal and rectal examinations. 3. Evidence of metastasis: Palpable nodular liver, para-aortic lymph nodes, ascites and supraclavicular nodes (Troisiers sign). PEARLS OF

WISDOM

Rectal cancer presenting as a fistula in ano is the equivalent to a perforated colonic cancer. It is a bad prognostic sign. Differential diagnosis (Key Box 31.4) 1. Villous adenomas (benign) present as bleeding per rectum with mass coming out sometimes of anal canal. They have frond like appearance. They are very friable, bleeds on touch easily and they are bulky. Biopsy is a must. lf it is benign, they can be removed through the rectum-submucosally. 2. Proctitis due to inflammatory bowel diseases: Both ulcerative colitis and Crohn 's disease produce diarrhoea, blood in the stools and multiple ulcers. They are not indurated. Whatever it is, biopsy is a must before doing a major surgical resection. Ulcers in ulcerative colitis are typically described as pinpoint ulcers. In Crohn's they are fissure type or patchy with a cobblestone appearance. 3. Amoebic granuloma: It is not common nowadays. It presents with soft mass at rectosigmoid colon with or

PROBABLE SITE OF LESION/EXPLANATION

Rectosigmoid Cauliflower-like growth Rectosigmoid stricture Growth in the ampulla of rectum Blood and mucus Sacral plexus infiltration Large bowel obstruction Liver metastasis, ascites, etc. Infiltration of the bladder base anteriorly

A 22-year-old girl was treated with iron tablets for anaemia due to occasional bleeding per rectum. She was treated with metronidazole because she was passing mucus along with the stools. She developed intestinal obstruction after 6 months during which time a surgeon was consulted. Rectal examination revealed a large growth, fixed all around. She died after 6 months because of advanced disease. The case illustrates the importance of rectal examination and that carcinoma of the rectum often occurs in young patients also.

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Manipal Manual of Surgery

.......................... .

KEY BOX 31.4

RECTAL ULCERS 1. Carcinoma rectum 2. Amoebic ulcers 3. Ulcerative colitis 4. HIV infections 5. Solitary rectal ulcers Fig. 31.13: Solitary rectal ulcers 6. Radiation proctitis

without obstruction. An ulcer over the surface will mimic carcinoma. Biopsy is mandatory because amoebomas are completely curable with antiamoebic treatment. 4. Tuberculous proctitis: Usually patients have pulmonary tuberculosis. Submucosal abscess rupture and result in ulcers with undermined edges. Hypertrophic tuberculosis with stricture can also occur. Biopsy is mandatory before resection. 5. Endometrioma: It presents as constipation, bleeding per rectum especially bleeding during menstruation. Typically young females between the age of20-40 years are affected. It produces constricting lesion in the rectosigmoid junction. Mucosa is intact as seen by sigmoidoscopy. Treatment is biopsy followed by treatment of endometriosis. 6. Solitary rectal ulcer syndrome (SRUS) • Site: Commonly occurs in the anterior wall oflower rectum, an area of mucosa! change. • Mucosa: It is erythematous, heaped up and bleeds on touch. • It is a single, depressed ulcer. The cause, even though not clear is probably due to trauma by anal digitation. Today, it is believed that it is due to internal intussusception or anterior wall prolapse. • Clinical features are passage ofblood and mucus in stools. Mucosa! prolapse may also be a feature. • A biopsy must be done to rule out carcinoma rectum. • Treatment is conservative: Avoidance ofconstipation and straining may treat the prolapse. Spread of carcinoma rectum 1. Local spread • It takes eighteen months for a growth to encircle the lumen of rectum, as in annular strictures at the rectosigmoid junction. • Then, it involves muscle coat and spreads into extrarectal tissues. • Anteriorly, it involves prostate, seminal vesicles and bladder base in males, vagina and uterus in females. • Posteriorly, sacral plexus gets involved in late cases and causes sciatica-like pain. 2. Lymphatic spread-chief nodes are para-aortic nodes.

3. Blood spread • It results in secondaries in the liver, lungs, etc. They an common in young patients with anaplastic variety and ir colloid carcinoma. 4. Peritoneal spread • It results in ascites, carcinomatous nodules over the peritoneum, etc. STAGING I. Modified Dukes' staging of carcinoma of rectum (Fig. 31.14) Stages A Growth confined to the rectal wall B Growth involving perirectal pad offat and tissues. No nodes are involved. B 1: Invading muscularis mucosa B2: Invading to or through serosa C Nodes are involved C1: Local lymph nodes-pararectal C2: Distal lymph nodes-along the course of blood vessels D Distant spread-liver, lungs, etc.

Fig. 31.14: The three cardinal stages of progression of the neoplasm

II. Astler-Coller's modification of Dukes' system Stages A Limited to mucosa-no nodes B 1 Extension into muscularis propria-no nodes B2 Extension into entire bowel wall-no nodes B3 Extension into adjacent organs-no nodes C l Extension into muscularis propria-positive nodes C2 B2 + Lymph nodes C3 B3 + Lymph nodes D Distant metastasis Prognosis as per Dukes' staging • Dukes' A: 5-year survival is 90 to 100% • Dukes' B: 5-year survival is 50 to 80% • Dukes' C: 5-year survival is less than 50%

Rectum and Anal Canal

iiMHMiWi

T represents the extent of local spread and there are four grades T1 tumour invasion through the muscularis mucosae, but not into the muscularis propria T2 tumour invasion into, but not through the muscularis propria T3 tumour invasion through the muscularis propria, but not through the serosa (on surfaces covered by peritoneum) or mesorectal fascia T4 tumour invasion through the serosa or mesorectal fascia N describes nodal involvement

• NO No lymph node involvement • N1 Between one and three involved lymph nodes • N2 Four or more involved lymph nodes M indicates the presence of distant metastases

• MO No distant metastases • M1 Distant metastases The prefix 'p' indicates that the staging is based on histopathological analysis and 'y' that it is the stage after neoadjuvant treatment, which may have resulted in downstaging Ill. TNM staging Key points in pathology, staging and spread • They are adenocarcinomas-well differentiated, moderately differentiated and poorly differentiated. • Signet ring carcinoma and colloid carcinoma carry poor prognosis • Spread occurs by-local, lymphatic, haematogenous and transperitoneal routes • Posterior sacral infiltration and anterior bladder base infiltration - surgery can be very difficult and dangerous (uncontrollable bleeding from sacral plexus of veins and prostatic plexus of veins in males. Hence preoperative chemoradiotherapy followed by surgery is done • Involvement of mesorectum carry poor prognosis hence the circumferential resected margin is important (see page 780) • Among the staging TNM is internationally accepted system.

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Investigations (Table 31.2) 1. Proctoscopy: It should be done in all cases of bleeding per rectum. It is done as an out patient procedure. The left lateral position with buttocks elevated on a small pillow is the ideal position for proctoscopy. However, knee-elbow position can also be used. The growth appears as an ulcer with everted edges. A biopsy is taken to prove the diagnosis. The histological grading of the tumour is as follows: A. Well-differentiated carcinoma: Low-grade variety (10-15%). B. Moderately differentiated carcinoma: The most common variety (65%). C. Undifferentiated carcinoma: The most aggressive variety (20-25%). 2. Sigmoidoscopy: To take a biopsy from rectosigmoid growths, sigmoidoscopy is essential. 3. Barium enema: It is indicated when proctoscopy and sigmoidoscopy fail to give a diagnosis due to spasm of the colon. When carcinoma arises in multiple polyposis coli or ulcerative colitis, barium enema is done to rule out synchronous malignancies. 4. Colonoscopy: If synchronous carcinoma exists (8 to 10%) biopsy can be taken to prove the diagnosis. 5. CEA: Increased levels of carcinoembryonic antigen indicates metastasis. 6. Ultrasound: Some cases of carcinoma rectum present with metastasis such as secondaries in the liver, ascites with para­ aortic nodes, etc (colloid carcinoma). 7. Endorectal ultrasonography (EUS) (Key Box 31.5) Endoscopic ultrasound staging of rectal tumors UTI Invasion confined to the mucosa and submucosa UT2 Penetration of the muscularis propria but not through to the mesorectal fat UT3 Invasion into the perirectal fat UT4 Invasion into the adjacent organ UNO No enlargement of lymph nodes UNI Perirectal lymph nodes enlarged 8. Computed tomography (CT) scan/MRI • It helps to detect the lesion, detect metastasis in liver. • To know the extension of the tumour • To know the fixation to adjacent structures (ureter, uterus, bladder base, etc. hydronephrosis). • Importantly, to know nodal status

Importance of each investigation and how it alters the treatment plan in a case of biopsy-proven carcinoma rectum. APR-abdomino-perineal resection, HAR, LAR-high and low anterior resection

I. Carcinoma lower rectum 2. Carcinoma upper rectum 3. Carcinoma lower rectum 4. Carcinoma rectum

5. Carcinoma lower rectum

Plan

Investigation finding

Changed plan

APR HAR APR

CT-metastasis in liver Colonoscopy growth in transverse colon M Rl/endosono-extensive T4 lesion

Palliative colostomy/chemoradiation Subtotal/total colectomy First chemoradiation followed by APR or LAR Cystoscopy-ureteric stenting-LAR No APR

LAR APR

CT scan-hydronephrosis PET scan-bone metastasis present

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............................ . KEY BOX 31.5

• • • •

EUS

It is also called Trans Rectal Ultra Sonography (TRUS) To know the level of penetration Detect perirectal lymph node enlargement Invasion of adjacent structures-levator ani, bony pelvis, etc. • It is superior in T-staging of rectal cancers.

TEN C O MMANDMENTS OF T O T AL MESORECTAL EXCISION 1. 2. 3. 4. 5.

9. MRI: Both MRI and EUS are good for assessment for T-staging, MRI has following advantages • It is better for T-3 and T-4 stage • High resolution MRI is better for assessment of circum­ ferential resected margin (CRM). • It is also good for nodal staging TREATMENT PRINCIPLES

1. Aim is to have a curative resection 2. Palliative resection is worth doing even in the presence of metastasis, when there is obstruction. 3. Even though surgical treatment is the main modality, radiotherapy and chemotherapy are beneficial. 4. At surgery, ligation of vascular pedicle is done first to prevent tumour embolisation. 5. Ligation of bowel, proximal and distal to the tumour helps to prevent transluminal dissemination. 6. 40% ethanol is used as tumouricidal agent to prevent suture line recun-ence. Solutions such as dilute povidone iodine have been used to irrigate rectal bed after APR to prevent recurrence but without much success. 7. Distal surgical margin should be about 2.5 cm to 3 cm. Proximal margin-minimum 5 cm. 8. An attempt should be made to perform a Total Mesorectal Excision (TME)-which improves quality of life (see Ten commandments and Key Box 31.6). 9. Colonic pouch: The splenic flexure is mobilised fost. A 6 cm limb of sigmoid or descending colon is folded and a pouch is created. A colotomy is made at the apex of the pouch. Linear cutter is used to staple the pouch on itself to create a common lumen. A second fire of the stapler may be necessary. This pouch now acts like a neorectum. • A double-stapled anastomosis as described or a hand-sewn anastomosis is then perfom1ed. A dive1ting loop ileostomy is used routinely for these ultra low anastomoses. LAPAROSCOPIC MESORECTAL EXCISION Introduction

Laparoscopic anterior resection and total mesorectal excision are well established procedures now. It is possible because of

6. 7. 8. 9. 10.

Should do TME in all cases of mid and lower carcinoma rectum Should excise the entire mesorectum (contains fat, lymph nodes and superior rectal blood vessels) Should d o the dissection with electrocautery o r scissors Should open the posterior plane between visceral and parietal layers of endopelvic fascia-Holy plane of Heald

or avascular plane

Should exert good traction and counter traction to develop the planes Should excise the entire mesorectum circumferentiallyminimum of 5 cm of the CRM Should be inside the pelvic plexus laterally Should excise Oenonvilliers fascia anteriorly Should excise rectosacral ligament so as to reach the pelvic floor Should do proximal diversion ileostomy

•=tilt_.�, TOTAL MESORECTAL EXC ISION-ADVANTAGES

1. Mesorectum is the perirectal fat surrounding the rectum. 2. It preserves autonomic nerves 3. Impotence, urinary incontinence and retrograde ejaculation are lesser after TME.

advances in the laparoscopic instruments, high definition cameras and improved technology. Advantages

• A 30 ° camera allows a beautiful magnified view of the entire dissecting field specially low down in the pelvis. Thus, no part of the laparoscopic procedure is blind. • Laparoscopic surgery allows the surgeon to adopt the principle of no touch technique. At the same time with better visualisation of the rectum and mesorectum all around, it permits a good dissection. • It is not uncommon in the open method that the specimen gets torn due to traction. It has been proved that this tearing is much less in laparoscopy. • Recovery is very fast after laparoscopy. • Better preservation of the pelvic autonomic nerves, low anastomotic leak and low mortality rates. • For laparoscopic low resections, one need not mobilise splenic flexure but sigmoid can be used for anastomosis. • Wound infection, paralytic ileus are much less after laparoscopic surgery than open surgery. Disadvantages • Very low resection-because of the limited space and the lack of proper curved instruments-open method has slight advantage.

Rectum and Anal Canal

• Port site recurrence: Local tissue trauma due to trocars, tumour manipulation, tumour behaviour are the factors responsible for port site recun-ence. Tumour spillage, tumour cell aerosolisation due to sudden loss ofpneumoperitoneum, tumour spillage during extraction and immunosuppression during pneumoperitoneum are a few factors responsible for port site recurrence. This can be minimised by using bag for extraction, minimal handling of the tumour, to avoid tearing the specimen. TREATMENT OF CARCINOMA RECTUM

Cancers arising in the distal 15 cm of the large bowel are included under this heading. These cancers behave almost like colonic cancers. The resection is the best treatment in early stages for cure. However, anatomy of the rectum, with its retroperitoneal location, narrow pelvis in males and proximity to the urogenital organs, autonomic nerves, and anal sphincters, makes surgical access and resection relatively difficult. In advanced cases neo-adjuvant therapy-chemo­ radiotherapy is given to downstage the disease and then resections are done. The treatment of rectal cancer has changed significantly over the past 20 years with the aim ofcure with rnultimodality treatment and preserving the sphincter. Broadly the various surgery done for carcinoma rectum are as follows. 1. Anterior resection: Refers to removal of rectum and sigmoid colon. Indicated in cases of carcinoma rectum above peritoneal reflection: It can be low anterior resection when colorectal anstomosis is done below the peritoneal reflection or high anterior resection where the anastomosis is above peritoneal reflection. In this procedure rectum and sigmoid colon is removed along with mesorectum which contain lymphatic channels. Sphincter function is preserved. Sigmoid colon has to be removed

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because in cases of inferior mesenteric artery ligation, blood supply becomes inadequate and anastomosis may leak. Colorectal-colo anal anastomosis is done with or without pouch. Stapler anastomosis is the choice for low resections. Bowel is clamped and transected just proximal to this point (Fig 31.15 to 18). When the anastomosis is very low Gust above anorectal ring), a protective diversion ileostomy is done which is closed after IO weeks. Patient will have increased frequency of stools, incontinence or soilage. Diet restrictions may help these patients. Total mesorectal excision should be the aim. It improves the survival rates, decreases incidence of local recurrence. 2. Abdominal Perinea! Resection (Figs 31.19 to 22): It is also called as Miles-Walker's operation It is indicated when the growth is too low involving the anal sphincters, poorly differentiated cancers which are very low. The patient is put in Lloyd Davis position (supine with lithotomy). Two surgeons operate simultaneously, one from the abdomen and one from the perineum. Abdomen is opened first and the growth is mobilised from the sacrum and from the urinary bladder. Pelvic dissection is carried

Fig. 31.16: Circular stapler used for anterior resection

Fig. 31.15: Carcinoma upper rectum

Fig. 31.17: Diagrammatic representation

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0

A

B

I I I I I I I I I

�--------------------� Fig. 31.19: The field of clearance in APR

D

Figs 31.18A to D: (A to C) Parts of the stapler, (D) shows as the stapler is tightened, upper and lower rectum come together and the anvil fits in very well in the circular stapler

MJ!MI...........� STRUCTURES REMOVED IN APR • Growth with entire rectum and anal canal. • Fascia propria with pararectal nodes. • Two-thirds of the sigmoid colon and mesocolon with lymphatics and lymph nodes. • Muscles and peritoneum of pelvic floor. • Wide area of perianal skin, with part of ischiorectal fossa.

by abdominal surgeon till levator ani muscles. At this stage, anus is closed by a purse string by perinea! surgeon. Rectum and anal canal is mobilized from below. The entire specimen of rectum with meso rectum and anal canal and the nodes are removed. It is followed by Permanent End­ Colostomy by bringing the sigmoid colon outside in the left iliac fossa (sphincter sacrificing surgery).Thus complete excision of the rectum, anal canal, mesorectum, and lymph nodes are done through abdomino-perineal

Fig. 31.20: APR specimen. Observe that the entire meso­ rectum has been removed

Fig. 31.21: Nodular lesion 3 cm away from the anal verge

Fig. 31.22: Permanent end-colostomy following APR

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VARIOUS PHOTOGRAPHS OF CARCINOMA RECTUM AT SURGERY (Figs 31.20 to 31.24)

Fig. 31.23: This patient with carcinoma midrectum presented with bleeding per rectum. Low anterior resection was done. At least 3 cm margin could be achieved distally

Fig. 31.24: Low anterior resection­ see the lower margin

Fig. 31.258: Proximal colon is mobilised up to the transverse colon so that it can be easily brought down without tension

A

Fig. 31.25A: Low anterior resection is in progress. The rectum has been mobilised from urinary bladder anteriorly and sacrum posteriorly -carcinoma midrectum

Fig. 31.26: Low anterior resection specimen-Stapler anastomosis has been done-you can see the complete doughnuts

B

C

Figs 31.27A to C: Schematic representations of resection of the rectum. (A) AP resection followed by colostomy, (B) High anterior resection followed by anastomosis, (C) Lo w anterior resection with colostomy and closure of rectal stump-Hartmann's procedure­ in large bowel obstruction. The colostomy is closed and colorectal anastomosis is done after 4 weeks

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M!tlt..........,.� • • • • • •

LOCAL EXCISION OF CARCINOMA RECTUM Mobile tumours less than 4 cm in diameter Less than 40% of rectal wall involvement Located within 6 cm of anal verge Lesion should be T1 or T2 No vascular or lymphatic invasion No nodal involvement-preoperative MRI or EUS.

incision. Usually one drainage tube is inserted brought out through perinea! wound. 3. Local excision: If the tumor is confined to the submucosa (uTI, NO), excision can be done by a transanal approach. The explanation being these cases will have less than 8%,lymphatic metastasis. In our country majority of patients do not fall in this category. It is indicated for mobile early tumors that are less than 4 cm in diameter, that involve less than 40% of the rectal wall circumference, with good histology like well or moderately differentiated histologically, and that are located within 6 cm of the anal verge. and with no vascular or lymphatic invasion. Recent change in APR: In the conventional APR dissection is within the levator ani and levator ani is preserved. The change is APR with removal of levator ani-it is called extra levator APR. The resected specimen has a waist-it is called cylindrical APR.

Inoperable cases Locally advanced growths present with severe pain, bleeding and with subacute intestinal obstruction. Temporary loop colostomy is done in the left iliac fossa by bringing the sigmoid colon outside. Postoperatively, radiation and chemotherapy are given. Hartmann's operation (Fig. 31.28) This is indicated in old and debilitated patients who may not withstand APR. The rectum is excised, the lower end of the rectum is closed and a colostomy is performed. When the growth is slow-growing, this operation gives good palliation. Other types of surgeries

suturing. It can be done by transanal, trans-sacral or trans sphincteric approach. • Trans-sacral (Kraske's) is for posterior rectal tumour. ENDOSCOPIC TREATMENT OF COLORECTAL LESIONS • Colonoscopic polypectomy: All adenomas and potentia adenomas should be removed. Pedunculated polyps an removed by snare. Small polyps can be removed by colc or hot forceps and hot or cold snare. Bleeding can be controlled by injection of epinephrine, electrocautery, clip� or endoloops. • Endoscopic mucosa! resection: It is indicated for flat, sessile lesions, not more than 2.5 cm in diameter. First the lesion is elevated by injecting saline or hypertonic dextrose with epinephrine. The lesion is elevated, cut and removed or sucked and cut. • Endoscopic submucosal dissection: Technically more difficult. Principle of the procedure is same. More chances of perforation. • Endoscopic stents for decompression for malignant obstruction: Large bore colorectal decompression tubes are available. They can be passed with or without endoscope with a guidewire. They are used to relieve obstruction so that bowel preparation can be done and one stage treatment­ resection and anastomosis can be done. Self expandable metal stents-SEMS has been used as palliation in cases of large gut obstruction. Quality of life is slightly better but no increase in survival rate of patients. Stent migration, tumour ingrowth, overgrowth, perforation and bleeding are the other complications. • Laser therapy: Like oesophageal carcinoma, lower rectal carcinoma with obstruction can be treated as a palliation by Nd:YAG laser.It cannot be used in acute cases. Compli­ cations are perforation, bleeding, fistula formation, etc. ROLE OF RADIOTHERAPY AND CHEMOTHERAPY

•

Fig. 31.28: Hartmann's operation

• TEM: Transanal endoscopic microsurgery (BUESS) • lt involves wide retraction, followed by good visualisation of lesion followed by complete thickness excision and direct

Postoperative Management 1. pT l -2NOMO do not require any adjuvant treatment, such patients can be kept on follow-up with routine 3 monthly CEA and annual CECT thorax/abdomen/pelvis. 2. pT3NOMO or node positive disease requires adjuvant treatment in the form of concurrent chemoradiotherapy and chemotherapy. Example: 2 cycles of FOLFOX (5-FU + Leucovorin + Oxaliplatin) � concurrent 5-FU/Leucovorin and radiation � 2 more cycles of FOLFOX. Oral Capecitabine can be used in place ofIV 5-FU. 3. It is preferable to add Oxaliplatin in the chemotherapy regimen if nodes were positive for metastatic disease. Although in older population(> 65-70 years) it might be of less benefit.

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Rectum and Anal Canal

4. Radiation portals should include the postoperative tumor bed, pre-sacral nodes, internal iliac nodes. External iliac nodes can also be included in T4 tumors. Radiation dose is usually 45-50 Gy given over 5 days a week for 5 consecutive weeks. Another 5-9 Gy boost to the tumor bed ONLY can be considered especially with adverse features like lymphatic emboli, close margins. Preoperative-Neoadjuvant chemoradiation (Key Box 31.9) Rationale: In locally advanced cases where the surgeon feels that complete resection may not be feasible or sphincter saving will not be possible, neoadjuvant chemoradiation can be attempted thus saving the patient from having a permanent colostomy bag and also a better curative outcomes.

M:t•e--..r� ADVANTAGES OF PREOPERATIVE RADIOTHERAPY

• Decreased tumour seeding at surgery • Increased radiosensitivity due to more oxygenated cells • Conversion of APR to LAR • Generally clinically T3-4 tumors which may or may not be node positive are eligible candidates for neoadjuvant chemotherapy. 1. A typical course of neoadjuvant therapy comprises concurrent 5-FU/Capecitabine and radiation. A dose of 45-50 Gy is used to treat the pelvis including the growth and the draining lymphatic regions followed by 5 Gy boost to the tumor itself. 2. Following neoadjuvant therapy, patient should be re-evalua­ ted using CT/MRI for possibility of resection. 3. Surgery is usually considered after 6-8 weeks following neoadjuvant therapy as the maximal response to the treat­ ment may take up to 2 months. 4. Fmiher adjuvant treatment is to be given following surgery depending upon the histopathological report. PEARLS OF

WISDOM

Brunschwig's operation) are alternative treatments available. COLOSTOMY Opening of the colon to the exterior, either temporary or permanent, for the drainage of faecal matter is called colostomy. TYPES 1. Temporary colostomy A. In cases of acute left-sided colonic obstruction, proximal half of right transverse colon is brought out through the upper part of the right rectus abdominis muscle. Later, radical resection of the left colon is done followed by closure of the colostomy. B. In cases of traumatic or congenital fistula affecting the left colon, temporary colostomy is indicated (Fig. 31.36). The loop of the colon which is brought outside is held in place by a glass rod. This is passed through the transverse mesocolon and held by rubber tubing. This rod is removed after 10 days. C. Colostomy/Ileostomy is done if a distal colorectal anastomosis gives way (Figs 31.29 and 31.30).

Fig. 31.29: Sigmoid colon is brought out and a glass rod connected to rubber tube is used to hold it out

Rectal cancers are more radiosensitive and colonic cancers are more chemosensitive. LOCALLY RECURRENT RECTAL CANCER • Major cause is a positive margin on pelvic side wall. This is the reason why preoperative chemoradiotherapy should precede excision of T3 and T4 lesions with TME. • Usually develops within 18 months. • Presents as pelvic pain, mass and rectal bleeding. • Pelvic CT, MRI, CEA levels are the required investigations. • Chemoradiation, surgery, local palliative treatment, pelvic exenteration (resection of rectum and bladder-

Fig. 31.30: Faeculent leak from failed anterior .___. resection

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PEARLS OF WISDOM Transverse colostomy is bulky, contents are semiliquid and difficult to manage-Try to avoid it. TEN COMMANDMENTS 1. Should mark the colostomy site in the standing and sitting positions 2. Should be at least 3 cm away from the bony landmark (anterior superior iliac spine) 3. Should be at least 3 cm away from the midline incision. Otherwise, colostomy will contaminate the incision site (Fig. 31.31) 4. Should close the paracolostomy space within the abdomen to prevent herniation of the small intestines 5. The colostomy should be matured after the midline incision is completely closed and sterile dressings applied 6. Should be in flush with skin surface in the left iliac fossa 7. Should excise a disc of skin for a permanent colostomy 8. The stoma should be brought out through the rectus muscle

Fig. 31.31: End colostomy away from the main incision

9. Should avoid bringing stoma outside through scar tissue

10. Should examine the end of the colostomy for vascularity (Fig. 31.32)

2. Permanent colostomy It is indicated after abdominoperineal resection where the end of sigmoid colon is brought outside in the left iliac fossa as permanent colostomy (Figs 31.31 and 31.32, Key Box 31.10). The colostomy site should be 3 cm away from anterior superior iliac spine so that colostomy bag can be fitted properly (Figs 31.33 and 31.34). 3. Double-barreled colostomy In this the adjoining walls of the intestine are crushed. Both ends of the loop are defunctioned. This type of colostomy is not frequently done now. It was done earlier for sigmoid volvulus, resection of colonic stricture, etc.

Fig. 31.32: Inspect for vascularity of colostomy following APR

Indications for colostomy • Congenital: In Hirschsprung's disease and anorectal anomalies, temporary colostomy is done first. • Carcinoma: Following APR, permanent end-sigmoid colostomy is done. • Colonic fistulae: Fistulae due to diverticulitis, Crohn 's disease or due to tuberculosis. • Colonic injuries: Trauma due to stab injuries or due to operative injuries following nephrectomy, pelvic operations, PCNL (percutaneous nephrolithotomy). Advantages of colostomy Distal bowel takes complete rest, regains normal size and bacterial colonisation is reduced. It becomes empty and sterile so that chances of leakage at a later operation is reduced.

Fig. 31.33: Disposable colostomy bags are available with or without flange. Depending upon the size of the stoma, it can be cut open so as to fit into the colostomy, Fig. 31.34: A colostomy bag has been applied. It can be reused

Rectum and Anal Canal

Fig. 31.35: Paracolostomy hernia: Herniation of bowel from the side of colostomy

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Fig. 31.38: Burst abdomen due to contamination of the wound from colostomy-proper care should be taken to isolate laparotomy site and colostomy till complete healing of laparotomy wound takes place

Complications of colostomy (Figs 31.35 to 31.38) • Bleeding, necrosis, retraction, prolapse, parastomal hernia and colostomy diarrhoea are some complications. • Colostomy obstruction, gangrene. PEARLS OF WISDOM Colostomy should be done by an experienced surgeon as patient has to live with the colostomy life long. A Figs 31.36A and B: Colostomy gangrene. It is mandatory to inspect the end of the colsotomy everyday in the postoperative period to check for vascularity. If it is gangrenous and if it is re­ tracted it is better to open the patient again and refashion colos­ tomy

MHMNIIIIIIIIIIIIII� PERMANENT COLOSTOMYLEFT ILIAC FOSSA

• • • • •

Avoid bony prominences Avoid belt lines Avoid marking in lying down position. Avoid scars Avoid bringing out stoma lateral to rectus.

PROLAPSE RECTUM Protrusion of the mucous membrane or the entire rectum outside the anal verge. This condition is common in children and elderly patients. Types Prolapse can be of two types: Partial prolapse and complete prolapse.

Fig. 31.37: Colostomy prolapse because sutures would have given way where it was anchored to aponeurotic layer. The patient also had violent postoperative cough

Partial prolapse • In this variety, the protrusion is between 1.25 and 3.75 cm outside the anal verge (Fig. 31.39). • It is usually a mucosa! prolapse.

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COMPLETE/TOTAL PROLAPSE • Full thickness prolapse is also called procidentia. • It is defined as protrusion of the rectum for more than 3.7. cm outside the anal verge. Very often, it is the entire rectun which protrudes out on straining, sometimes along wit] the peritoneal sac. • Often, it is associated with prolapse uterus.

Fig. 31.39: Partial prolapse

The pelvic floor-surgical anatomy (Fig. 31.41) • It is composed of the two levator ani and a puborectafo muscle. • Levator ani originate from pelvic side walls anc sacrospinous ligament. It suspends the rectum in a muscula1 sling which ends when puborectalis angulates rectum. • Puborectalis muscle takes origin from posterior aspect o1 pubis, forms a sling around rectum and return to posterio1 pubis. • Contracted puborectalis is responsible for normal acute anorectal angle and it is critical for maintaining continence. Thus during coughing, sneezing, anorectal angle becomes more acute, increasing continence. Supports of the rectum and surgical importance Various supports of the rectum keeps the rectum in place. Failure of one or more of these factors may precipitate rectal prolapse (Fig. 31.42). They have been enumerated in the following lines. 1. Pelvic floor: Weakness of pelvic floor can be due to birth

Fig. 31.40: Rectal prolapse in a child

Causes 1. In infants, it is due to undeveloped sacral curve and in children it can be secondary to habitual constipation. 2. It can follow an attack of whooping cough or excessive straining (Fig. 31.40). 3. It can follow an attack of dian-hoea resulting in loss of fat in the ischiorectal fossae, which supports the rectum. 4. In adults it is common in females mostly due to Torn perineum caused by obstetric trauma. Treatment I . Digital reposition: In infants, partial prolapse is tempora1y. The mother is advised to push the prolapse inside after lubricating with lignocaine jelly. 2. Injection of ethanolamine oleate into the submucosa of the rectum. It causes aseptic fibrosis. Thus, mucosa gets tethered to the other layers. 3. Partial prolapse can be excised, after applying Goodsall's ligature.

injuries or due to defective collagen maturation. 2. Lateral ligaments: These ligaments are due to condensation of pelvic fascia on each side of the rectum. Excessive mobility of these ligaments may be the contributing factor for prolapse rectum. 3. Fascia of Denonvilliers (rectovesical fascia): Deep rectovesical pouch is often found in prolapse rectum. In all cases of complete prolapse rectum, please look for the deep rectovesical pouch and if it is present it should be obliterated. 4. Fat supports the rech1m. Hence, any chronic illness and loss of fat may contribute for prolapse rectum. Anorectal physiology and investigation These are useful in patients who have complaints of prolapse rectum, constipation, incontinence. 1. Anorectal manometry • Normal resting pressure in the anal canal---40-80 mmHg (it is the function of internal anal sphincter.) • Squeeze pressure: It is maximum voluntary contraction pressure minus resting pressure. It is 40-80 mm above resting pressure. ft reflects function of external anal sphincter.

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A FEW PHOTOGRAPHS OF PROLAPSE RECTUM Pubic symphysis

Levator ani

Rectum--�

Fig. 31.41: Pelvic floor anatomy-weakness of the pelvic floor is an important cause of prolapse rectum

INTERNAL INTUSSUSCEPTION

Prolapse rectum • Rectal fullness • Incomplete evacuation

Solitary rectal ulcer • Rectal bleeding • Tenesmus • Mucus discharge

Progressive incontinence Pain Prolapse

Further progression

Descending perineum syndrome Irreducible prolapse """"7 lschaemia

Gangrene

Fig. 31.43: Pathogenesis of prolapse rectum

Fig. 31.42: Prolapsed rectum-diagrammatic representation, sometimes confused for prolapsed haemorrhoids

2. Function of pudenda( nerve and puborectalis nerve: These are nerve conduction studies which can reflect injuries to the nerve. Causes (Fig. 31.43) 1. Common in elderly women who are multipara. Probably, it is due to repeated birth injuries to the perineum causing damage to the nerve fibres. As age advances, muscles become weak. This, together with fatty degeneration of the muscle, results in prolapse rectum. 2. Excessive straining causes weakness of the supports of the rectum. 3. Defective collagen maturation results in failure ofrectal support by levators and pelvic fascia. 4. Presence of deep rectovesical pouch and excessive mobility of the rectum (mesorectum) predisposes to prolapse of the rectum. 5. Many people believe that prolapse of the rectum starts as an intussusception in the first stage, initiated by certain factors such as diarrhoea, constipation and disorder of the pelvic floor. The process starts with anterior wall of rectum, where supporting tissues are weakest (Fig. 31.45).

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Uterine prolapse

Fig. 31.44: Complete prolapse-should be tested by asking the patient to squat and strain Uterus

Rectal prolapse

Fig. 31.46: Complete prolapse of the rectum with prolapse of uterus-procidentia

• Some degree of incontinence of faeces and flatus is alway1 present. It gives rise to urgency and perianal soiling. • Rectal examination-lax anal sphincter and wide gaping on straining. • Procidentia (Fig. 31.47)

Urinary--- 4 Small stitches at close intervals Aponeurosis only Minimal tension Monofilament, slowly absorbable suture

2. • • •

Prevent wound infections Correct suture technique Attention to hygiene measures Additional measures, e.g. antibacterial sutures

3. Surgeon-related factors See Key Box 35.4

.,........... • • • • • • • •

Figs 35.12 and 35.13: Wound dehiscence due to gangrene of

the intestine-laparotomy was done, resection followed by closure of the abdomen with tension sutures

5. Closer of mid line incision: In all cases of midline incision it is the linea alba with or without anterior fascia! layer is approximated to the corresponding fascia on the other side. • "Fascia" usually refers to the anterior rectus fascia, the fascia above the rectus muscles. This fascia holds the abdomen together and is the most important layer of closure. • The fascia can extend beyond the muscles and bind to other fascia. • An extension of the fascia is called an aponeurosis. Complications of wound closure I. Early complications: • Infection • Dehiscence II. Late complications: • Incisional hernia • Suture sinus • Wound pain

SURGEON'S ROLE IN PREVENTING INCISIONAL HERNIA

�

Continuous vs interrupted sutures Knotting technique-secure knots Suture length/wound length ratio Bite size Mass closure vs aponeurosis only Tensionless sutures Suture material-nonabsorbable Anti-SSI (surgical site infection) measures-prophylactic antibiotics

4. Continuous sutures See Key Box 35.5

MHMI..........� • • • •

CONTINUOUS SUTURES

Continuation suturing is faster Accommodates wound lengthening due to distension Bursting strength of wound is significantly higher Minimises number of knots-equivalent or lower incidence of incisional hernia • Disadvantages (theoretical): Wound security depends on single strand of suture and limited number of knots. DIVARICATION OF RECTI • In this condition, the two rectus abdominal muscles are widely separated (not in the midline). • Repeated pregnancy in quick succession is the most impor­ tant cause. Chronic constipation or overstraining may be another factor. Obviously women are commonly affected. • Exercises and abdominal corset are helpful. • Symptomatic cases are operated-divaricated recti are brought towards midline.

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RECTUS SHEATH HAEMATOMA Collection of blood in relation to rectus sheath and muscles occurs due to tearing of one of the branches of inferior epigastric artery. A parietal haematoma occurs usually at the level of the arcuate line. It is an uncommon condition. However, the causes can be as follows: 1. Trauma: A sudden blow to the abdominal wall. 2. Straining: Sudden straining such as violent cough or vigorous exercise in a muscular man can cause haematoma. 3. Pregnancy: Rarely, the cause of haematoma can be pregnancy, in late trimester. The exact cause is not known. Clinical features • History of sudden straining or coughing, etc. • A tender lump develops just below and to the side of umbilicus at the level of arcuate line where posterior rectus sheath is absent. • Nausea, vomiting, pyrexia are the other features. Diffe rential diagnosis Spigelian hernia (it is rare) Treatment • The condition is self-limiting. With antibiotics and analgesics, a haematoma subsides within 5-7 days. If it persists or progresses or if there is a doubt about the diagnosis, exploration and evacuation of haematoma should be done and the bleeding vessels are ligated. The results and recovery are excellent. MELENEY'S PROGRESSIVE POSTOPERATIVE SYNERGISTIC GANGRENE This dangerous complication is rare nowadays, thanks to the good pre- and postoperative antibiotics. Aetiopathogenesis • It is caused by synergistic action of microaerophilic non­ haemolytic Streptococcus and Staphylococcus aureus. • Surgical operations which have increased risk ofMeleney's gangrene include perforated appendix, biliary tract surgery, colectomy, etc. • Atherosclerosis, diabetes are the other precipitating factors. • Starts as cellulitis with reddish skin and postoperative fever. • The spread may occur within 3-5 days, with extensive gangrene and sloughing of the skin of the abdominal wall with purulent discharge. Clinical features • Postoperative patient with cellulitis of abdominal wall • Fever of moderate degree, an extremely tender abdominal wall and purulent discharge. • Toxicity and deterioration of general health may follow soon.

Treatment 1. At the stage of cellulitis: Broad spectrum antibiotics to cover not only the organisms mentioned above but to cover anaerobic organisms. Thus, a combination of benzyl penicillin, gentamicin and metronidazole is used. 2. At the stage of gangrene: Emergency aggressive debride­ ment is the treatment. Dead skin and subcutaneous tissue is excised, pus drained and the slough is removed. 3. Hyperbaric oxygen may be very useful. 4. Skin grafting is done, once the wound is healed with granulation tissue. FIBROMATOSES: DESMOID TUMOUR Classification • Superficial or deep. Deep fibromatoses, also called Aggressive fibromatoses and desmoid tumours is an uncapsulated fibroma which occurs in the abdominal wall. • It arises from muscles and aponeurotic layer of the abdominal wall. Incidence • In children, most desmoid tumours are extra-abdominal with a female predominance. • In young adults desmoid tumours almost always occur in the abdominal wall (of women). Hormonal effects and pregnancy are believed to influence the growth of this tumour. • Some tumours express hormone receptors (oestrogen and progesterone), and therefore tamoxifen and other hormonal modulators are among the adjuvant therapies for this tumour. Aetiopathogenesis • Childbirth, trauma or operative scars are the possible aetiological factors. • Desmoid tumour is one of the components of Gardner's syndrome. • It is benign but has a tendency to infiltrate the muscles. Some fibromas exhibit dysplastic changes. The cut surface is compared to an onion-whorled fibroma with spindle­ shaped cells. • Sarcomatous changes and metastasis do not occur. Clinical features • Abdominal fibromatosis is far less prone to recurrences than desmoid tumours in other sites. • It usually occurs in the abdominal wall of women of childbearing age during or after pregnancy. • Clinically the lesions present as deep-seated, firm, nonencapsulated, slow-growing, locally invasive and painless masses.

.,.,............

Umbilicus and Abdominal Wall

• It typically manifests as a slow-growing, progressive mass that becomes more prominent on abdominal muscle contraction. Mass is in the abdominal wall and is firm to hard in consistency. • Mesenteric fibromatosis, probably the commonest among the group, usually presents as a slow-growing mass that involves small bowel mesentery or retroperitoneum. • The recurrence rate of mesenteric fibromatosis seems to be substantially higher in patients who have Gardner's syndrome than in patients who do not have this syndrome. Treatment • Simple excision results in recurrence. Hence, wide excision with 2-3 cm of nonnal healthy margins is necessary with reconstruction of the abdominal wall (Figs 35.14 and 35.15). • In spite of adequate surgery, I 0-20% chances of recurrence occurs (Key Box 35.6).

• • • • • •

PECULIARITIES OF DESMOID TUMOUR Uncapsulated fibroma Infiltrates muscles, even though benign Does not change into sarcoma It may be a part of Gardner's syndrome Simple excision results in recurrence Wide excision is recommended

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•,.,............ SITES OF ENDOMETRIOSIS • Laparoscopic port - Umbilicus • Gynaecological surgery - Abdominal incision • Episiotomy - Perineum

�

Fig. 35.14: Wide excision of recurrent desmoid tumour in the abdominal wall

Fig. 35.16: Scar endometriosis

Fig. 35.15: Specimen of desmoid tumour which is removed along with normal tissue

ENDOMETRIOSIS OF THE ABDOMINAL WALL Drugs like sulindac and tamoxifen have also been used here, with some success. • It occurs due to mechanical implantation of endometrial cells during surgery (sites-Key Box 35.7 and Fig. 35.16). • Painful, palpable swelling, more symptomatic at the time of menstruation are characteristic features. - Perimenstrual cyclical bleeding can occur • Oral contraceptive pills may control the symptoms. • Otherwise, excision of the nodule has to be done (Fig. 35.17).

Fig. 35.17: Wide excision of scar endometriosis WHAT IS NEW IN THIS CHAPTER?/RECENT ADVANCES

• Desmoid tumour and fibromatosis have been updated. • A few coloured photographs have been added.

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MULTIPLE CHOICE QUESTIONS

1. The following is not a cause of umbilical faecal fistula: A. Persistent vitellointestinal duct B. Tuberculosis C. Carcinoma D. Raspberry adenoma

6. The principles of surgery for burst abdomen include! all of the following except: A. Layer by layer careful suture B. Adequate exposure C. Tension sutures D. Trimming of edges

2. The following statement is true about burst abdomen: A. It is very painful B. It is associated with shock C. Straining can produce it D. Conservative management is the choice

7. The pathognomonic sign of burst abdomen is: A. Shock B. Pink or brown serosanguinous discharge C. Pain D. Occurs on the second postoperative day

3. About desmoid tumour: A. Is a capsulated fibroma B. Infiltrates muscles C. Often changes to sarcoma D. Simple excision is recommended

8. Umbilical adenoma: A. Sessile swelling B. Is a premalignant condition C. Can be treated with ligature D. Occurs due to persistent umbilical vessels

4. The following is true of Meleney's postoperative synergistic gangrene except: A. Is progressive B. Can occur after appendicectomy C. Starts as cellulitis D. Is painless

9. Pilonidal sinus is known to occur in the following places except: A. Internatal cleft B. Umbilicus C. Interdigital cleft D. Ax.ilia

5. The following is true about rectus sheath haematoma except: A. It can occur with pregnancy B. Sudden straining can precipitate it C. Presents as painless lump above umbilicus D. Nausea, vomiting and fever are other features

10. Meleney's gangrene is a synergistic gangrene caused by: A. Streptococcus and Staphylococcus B. E. coli and Klebsiella C. Clostridium and Pseudomonas D. Salmonella typhi and paratyphi

ANSWERS 1 D

2 C

3 B

4 D

5 C

6 A

7 B

8 C

9 D

10 A

36

Blunt Abdominal Trauma, War and Blast Injuries and Triage • • • • • •

Liver injuries Small bowel injuries Colonic injuries Duodenal injuries Pancreatic injuries Renal injuries

• • • • • •

Introduction Blunt abdominal trauma (BAT) is one of the common surgical emergencies encountered by general surgeons. Increasing number of vehicles, high speed and poor maintenance of the roads are the contributing factors. Blunt injury abdomen with polytrauma is one of the commonest causes of death in the younger population. Thus, it is important for a house officer to recognise a polytrauma patient, to diagnose and to suspect intra-abdominal injury, so that urgent resuscitation and treatment can be offered to the patient at the proper time, at the proper hospital and by a proper surgeon. Major systems involved are given in Key Boxes 36. l and 36.2. Craniospinal and chest injuries are discussed in their respective chapters. Pelvic and skeletal injury is beyond the limits of this book. In this chapter, blunt injury of the abdomen is discussed.

Retroperitoneal haematoma Blast injuries Warfare injuries Missile wounds of abdomen Triage What is new?/Recent advances

RH........_..� • • • • • • •

COMMON VISCERA INVOLVED IN BLUNT INJURY Spleen Significant bleeding Liver Significant bleeding Kidney Significant bleeding Intestines Perforation-peritonitis Pancreatico­ duodenal injuries Usually missed-bleeding Diaphragm M issed-tachypnoea Urinary bladder Urinary peritonitis

NMIBIIIIIIIWIIIIIIIW�

Causes of blunt injury abdomen I. Rail and road traffic accidents (most common) 2. Fall from a height and dashing against an object 3. Seat belt syndrome 4. Assault Mechanism of injury/pathophysiology • Vehicular trauma is by far the leading cause of blunt abdominal trauma. • Broadly BAT can be explained by three mechanisms: 1. Rapid deceleration: It causes differential movement among adjacent structures. Shearing forces are created;

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• • • • •

MAJOR SYSTEMS INVOLVED Craniospinal Chest Abdomen Pelvis Skeletal

they cause solid, visceral organs and vascular pedicles to tear at relatively fixed points ofattachment. Examples: Renal pedicle injury - Injury to distal aorta than proximal mobile aorta as former is attached to thoracic spine. 2. Crushing effect: Here solid viscera are crushed between anterior abdominal wall and vertebral column or posterior thoracic cage.

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3. Sudden dramatic rise in the intra-abdominal pressure due to external compression • Hollow viscus ruptures (in accordance with principles of Boyle's law). Investigations 1. Complete blood count, coagulation studies, grouping and cross-matching. Fall in haemoglobin is an indication of on­ going haemorrhage-especially while managing a patient with liver/splenic injury on conservative line of management. 2. Serum electrolyte analysis 3. Serum amylase/lipase • May be elevated because of pancreatic ischaemia due to hypotension • Persistent elevation may be indication of intra­ abdominal injury. 4. Plain X-rays (Fig. 36.1) • Chest X-ray: Pneumoperitoneum-fundic, stomach (air bubble in thorax as in diaphragmatic injury, retro­ peritoneal air-duodenal perforation. • Pelvic fractures 5. Role of ultrasound (Fig. 36.2) FAST: Focussed assessment with sonography for trauma 6. Diagnostic peritoneal lavage (DPL) It is indicated in BAT in following situations: • Multiple injuries and shock • Spinal cord injury • Obtunded patient with possible abdominal injury • Intoxicated patient Also see splenic trauma (page 619). Types • Open: Infraumbilical skin incision and open peritoneum. • Semiopen: Infraumbilical skin incision deepen up to linea alba. • Closed: Blind insertion of needle.

Fig. 36.1: Chest X-ray showing diaphragmatic hernia on the lef" side

Precautions • Foley's catheter to empty bladder • Ryle's tube to empty stomach • X-ray pelvis to detect pelvic fracture. Positive DPL • 10 ml of gross blood aspirate before infusion of lavage fluid. • More than 100,000 RBC/ml • More than 500 WBC/rnl • Bile and bacteria are demonstrated • Vegetable matter PEARLS

OF

WIS D O M

Positive DPL means intraperitoneal injury is present. It does not mean that the patient should be shifted to operation theatre immediately.

BLUNT ABDOMINAL TRAUMA (BAT) FAST: Focussed Assessment with Sonography for abdominal Trauma

Fluid detected

Stable

Unstable

4 Acoustic windows • Pericardiac • Perihepatic • Perisplenic • Pelvic

Operation theatre CT scan • Intoxicated patient • Spinal injuries • Pelvic injuries , Determine source of bleeding r---+----1 I C f T of the abdomen is equivocal, and the patient is not improving, consider LAPAROTOMY

Negative/inconclusive Diagnostic peritoneal lavage

Blood +ve

Stable

Fig. 36.2: Algorithm of investigations in BAT (see FAST)

Observe

Bile Faecal Vegetable Matter

Laparotomy

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Blunt Abdominal Trauma, War and Blast Injuries and Triage

7. CT scan (Fig. 36.3) • Gold standard for solid organ injuries • CT also can reveal other associated injuries such as vertebral or pelvic fractures. • CT can also pick up diaphragmatic injury (CT chest). • It can detect source of haemorrhage • CT is an excellent scan for pancreas, duodenum, etc.

PEARLS OF

WISDOM

Stable patients with solid organ injuries are managed more often by nonsurgical methods with clos e monitoring. Remarks With the availability of FAST and CT scan, role of DPL is now limited to unstable patients whose FAST results are negative or inconclusive. ON ARRIVAL AT CASUALTY Primary survey-to identify and treat life-threatening injuries ABCDE (ATLS protocol) Airway Breathing Circulation Disability Exposure

Fig. 36.3: Diaphragmatic hernia-CT scan ( Courtesy: Dr Yashdeep Sharma, Associate Professor, KMC, Manipal)

8. Diagnostic laparoscopy: Done when CT scan is negative, suspicion of diaphragmatic injury is present (Fig. 36.4).

Fig. 36.4: Diaphragmatic hernia with gangrene of the stomach

AMPLE (History) Allergy Medication Past medical illness Last meal Events leading to incident

Secondary survey-physical examination Initial clinical assessment in an alert patient: Pain, tenderness, gastrointestinal haemorrhage, hypovolaemia. Clinical evaluation alone has an accuracy rate of only 65% for detecting blood in peritoneal cavity. • Abdominal examination: Abrasions, contusions, ecchymosis may indicate underlying visceral damage. 1. London's sign: Bruising of abdominal wall underlying hollow visceral perforation. 2. Seat belt sign: Contusion across lower abdomen. 3. Saree sign (Fig. 36.28): Across upper abdomen may be associated with pancreatic/bowel injuries. 4. Cullen's sign: Ecchymosis in the umbilicus 5. Grey Turner's sign: Ecchymosis in the flanks 6. Rib fractures on right side: Look for liver injury 7. Rib fractures on the left side: Look for splenic injury 8. Auscultation of bowel sounds in the thorax: Diaphragmatic injury 9. Rectal examination: Floating prostate, bony penetra­ tion-pelvic fracture, high riding prostate. • Vermooten's sign: Urethral injury-in cases of rupture of membranous part of urethra, prostate will not be palpable as it is displaced upwards. It is called high riding prostate. Tertiary survey Repeat primary survey, secondary survey and repeat laboratory/imaging studies (see Key Box 36.3 for wisdom lines in blunt abdominal trauma).

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1.

2. 3.

4. 5.

BLUNT ABDOMINAL TRAUMA: WISDOM LINES First priority in BAT is to assess and manage airway, breathing, with cervical spine precautions and circulation. Immediate splinting of fractures and control haemorr­ hage. Resuscitation using wide bore cannulae-'2 litres of fluid' If a hypotensive patient responds to fast transfusion of warm crystalloid solution, it means it is a hypovolaemic shock. It rules out head injury. FAST/ CT scan and decide-unstable patient will be taken to surgery immediately. Frequent, repeated examination-any new signs in a stable patient such as • Tachycardia Continuing • Hypotension } haemorrhage • Temperature • Oliguria } Early sepsis, intestinal • Jaundice gangrene or perforation • Tachypnoea • Hazy chest X-ray } Septic shock or diaphragmatic injuries • Nasogastric bleeding • Immediate laparotomy is indicated

LIVER INJURIES Liver injury should be suspected when a patient with suspected blunt injury abdomen is brought with the following features: • Right lower ribs fracture • Injury marks on the lower chest or upper abdomen. • Patient with persistent hypotension or patient who had shock following blunt injury abdomen. • A child can have liver injury without fracture ofribs because of elastic nature of the rib cage.

Fig. 36.5: Massive bleeding-challenging task ( Courtesy: Dr Suni Krishna, Assistant Professor, KMC, Manipal)

Clinical presentation • The most common presentation is features ofintraperitonea haemorrhage, which includes hypotension, thready pulse abdominal distension as in Fig. 36.5. Peritoneal signs an minimal as early bleeding does not produce much peritonea irritation. • However, massive lacerations of the liver including stellatE fractures present with rapidly developing hypotension anc shock, which are life-threatening. Investigations • Ultrasonography and more precisely CT scan should be done in all patients who are haemodynamically stable with or without support (Figs 36.6 to 36.9 and Key Box 36.4). • Also see Table 36.1

•••...........,.� CT SCAN WITH IV CONTRAST It can grade the liver injury It can guide a conservative or operative treatment. It also rules out other injuries Grade I and Grade II injuries can be managed by non­ operative treatment. • Free contrast in and around the liver is indicative of active bleeding.

• • • •

Liver injury scale Grade

II

III IV V VI

Injury

Description

Haematoma Laceration Haematoma diameter Laceration Haematoma

Subcapsular haematoma;< 10% surface area Capsular tear, < I cm parenchymal depth Subcapsular, I 0-50% surface area; intraparenchymal extension < IO cm

Laceration Laceration Laceration Vascular Vascular

< 10 cm long; 1-3 cm parenchymal depth Subcapsular,> 50% surface area; expanding intraparenchymal haematoma of> 10 cm or expanding > 3 cm, intraparenchymal depth Parenchymal disruption of 1-3 Couinaud's segments within a single lobe Parenchymal disruption> 3 Couinaud's segments within a single lobe Retrohepatic vena cava/central major hepatic veins Hepatic avulsion

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Figs 36.6 and 36.7: CECT arterial phase-Grade IV laceration of the liver with haemoperitoneum-interestingly patient was stable, managed conservatively, got discharged on 10th day

Fig. 36.10: Liver injury-use of omentum Fig. 36.8: Coronal section liver and kidney injury

Fig. 36.9: Liver injury Grade II

Treatment (Key Boxes 36.5 and 36.6) 1. Simple lacerations which are not bleeding at laparotomy: A drain is kept in the liver bed, blood and clots are sucked out and peritoneal wash is given. 2. Simple laceration with bleeding: It is sutured by interlocking horizontal mattress sutures by using special liver suturing needle. If too much tension is applied while

suturing, cutting through can occur. Omentum can be used as a Plug in between the laceration (Fig. 36.10). Absorbable sutures are used. 3. Subcapsular haematoma: If present, should be evacuated. 4. Deep laceration with bleeding: In such situations, wound should be opened. Dead liver parenchyma is removed, bleeding vessel at depth and biliary radicle are ligated. It is described as tractotomy. 5. Severe lacerations: These injuries present with massive bleeding. Temporary control is obtained by compression of portal vein and hepatic artery in gastrohepatic omentum in front of foramen of Winslow (Pringle manoeuvre). If bleeding stops, portal veins or branches of hepatic artery are damaged. If bleeding continues, hepatic veins are the source of bleeding. Visualisation of source of bleeding with debridement of avascular liver tissue is done by finger fracture method. Perihepatic packing can be used to compress the liver as a temporary measure to buy time for resuscitation, to explore rest of the abdomen or as a definitive treatment when other measures fail. Pack is usually removed after 24--48 hours (Fig. 36.11 ). • Nonanatomical resection may have to be done, in a few cases.

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HAEMOSTATIC TECHNIQUES AT SURGERY

Chromic catgut liver suture Perihepatic packing Resection Argon beam coagulator followed by fibrin glue and sheet of Surgicel (haemostatic agent) • Selective arteriography and embolisation in arteriovenous fistula or haemobilia.

MHrR..........,.� SUMMARY OF MANAGEMENT OF LIVER INJURIES

• Laceration Suturing • Expanding subcapsular haematoma Evacuation Suturing biliary radicles and • Deep laceration portal radicles, packing, etc. • Severe laceration Debridement Tractotomy; hepatic artery • Stellate fracture ligation Ligation of hepatic vein, portal • Complex injuries vein branches or lobectomy, etc. 6. Complex liver injuries: These injuries involve hepatic veins, retrohepatic vena cava or branches of portal vein resulting in massive haemorrhage. This type of massive injury can be managed by a large thoracoabdominal incision or abdominostemal incision by doing stemotomy. Division of the right triangular ligament helps in visualising bleeding from hepatic veins. Schrock shunt: Failure of Pringle manoeuvre means juxtahepatic and retrohepatic vena caval injuries. In such cases, Heaney manoeuvre clamping both infra and suprahepatic vena cava followed by atriocaval shunt or venovenous bypass can be done.

Fig. 36.11: Perihepatic packing

Complications of liver injuries 1. Massive bleeding, hypovolaemia and cardiac arrest. 2. Haematoma can get infected resulting in an abscess. 3. Haematoma can rupture into the peritoneal cavity resultin in leakage of bile-biliary peritonitis. 4. Haemobilia refers to rupture of the haematoma into the bil duct-it may result in massive haematemesis or melaena.

................

CLINICAL NOTES

A 24-year-old male patient was operated for blunt injury abdomen by laparotomy. Liver laceration was found. It was sutured. After 15 days, he came to our hospital with haematemesis. Initially it was diagnosed as erosive gastritis. Ultrasono­ graphy revealed a pseudoaneurysm of one of the branches of middle hepatic artery. CT angiography and embolisation was tried but not successful. He had another bout ofhaema­ temesis. He underwent exploratory laparotomy and ligation of branches of middle hepatic artery. Bleeding stopped. Now since 6 months, nofurther attacks of haematemesis (Fig. 36.12). It was a case of haemobilia (Fig. 36.13).

Fig. 36.12: Laparotomy scar of the patient who had liver laceration which was sutured. He presented with massive haematemesis later-a case of haemobilia

Fig. 36.13: Branch of middle hepatic artery ligated

Blunt Abdominal Trauma, War and Blast Injuries and Triage

SMALL BOWEL INJURIES • The shearing injuries produce either disruption or laceration of the bowel between fixed and mobile points, i.e. at the duodenojejunal flexure or at ileocaecal junction. These are the most common sites of small bowel injuries. • Injury to the small bowel can also occur due to crush injury between spine and a steering wheel or handle bars, etc. • Bruising on the abdominal wall may suggest perforation (Fig. 36.14). • Mesentery and its vessels also get damaged and bleeding can be sufficient to produce hypovolaemia and shock (Fig. 36.15).

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Clinical presentation 1. Acute abdominal pain: Features are like that of any

perforation peritonitis with guarding and rigidity. Erect abdominal X-ray shows gas under the diaphragm.

2. Features of peritonitis with haemoperitoneum is the result of bowel injury with bleeding from the mesentery. 3. Occult or hidden perforation: A small perforation gets sealed off by coils of bowel and omen tum. Most of these patients present with abdominal pain. However, very often, features of peritonitis are missed as a result of other associated injuries such as fracture pelvis or retroperitoneal haematoma. After 3-4 days, a localised abscess may form and rupture into the peritoneal cavity, resulting in peritonitis. This is aggravated by intake of oral fluids which stimulate peristalsis. Repeated examination is the most honoured, most fruitful investigation in blunt injuries of the abdomen (see clinical notes). Investigation • X-ray abdomen, erect or lateral decubitus (Fig. 36.16) demonstrates free gas under the right dome of the diaphragm in majority of cases. Four quadrant tap or diagnostic peritoneal lavage is also useful. • When in doubt, CT scan of abdomen should be requested to diagnose hollow viscus perforation and bleeding.

Fig. 36.14: Observe London's sign: Bruising (imprint abrasion) over the abdominal wall signifying hollow viscus perforation

Fig. 36.15: Gangrene of t he jejunal loop due to injury to mesenteric blood vessels in the same patient in Fig. 36.14

A 23-year-old male with fracture femur and pelvic fracture was admitted to the hospital after 12 hours of injury. A general surgeon was consulted to rule out an intra-abdominal injury. Pulse rate was 100/min and on deep palpation, there was tenderness in the right iliac fossa. Keeping in mind associated pelvic injury, it was decided to treat him conservatively. X-ray abdomen left lateral decubitus (Fig. 36.16) (erect film could not be taken as patient could not stand) film did not show free intraperitoneal air (gas). Ultrasound revealed a retroperitoneal haematoma of 8 cm x 3 cm. The patient was treated conservatively with Ryles tube for 3 days. On the 4th day, oral fluids were started, as patient passed stools once. On 7th day morning, patient had tachypnoea. Pulse was 120/min, BP was 90/60 mm of Hg. Previous 24 hours urine output was only 450 ml. Abdominal examination revealed guarding and rigidity in the right iliac fossa. It was decided to do a laparotomy. At laparotomy, there was a small 2 cm perforation in the ileum with bilioma surrounded by intestinal loops and gross contamination of peritoneal cavity. The perforation was closed and the peri­ toneal cavity was drained. The patient made a good recovery from septicaemia, thanks to early antibiotics and surgery.

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PHOTOGRAPHS OF SMALL INTESTINAL PERFORATION (Figs 36.17 to 36.21)

Fig. 36.16: Lateral decubitus picture is extremely useful in polytrauma cases when patient is unable to stand due to fractured limbs. However, CT scan is the best investigation in blunt abdominal trauma because it not only detects pneumoperitoneum but also other injuries

Fig. 36.17: Jejunal transection-6 cm away from DJ flexure. It is better to resect and anastomose in this type of cases ( Courtesy Dr Saurabh Aggarwal, Associate Prof, KMC, Manipal)

Fig. 36.18: 72 hours old perforation close to ileocaecal junction. Limited colectomy done

Fig. 36.19: Anastomotic leak following limited colectomy. lleostomy done. It was closed after 8 weeks. You can see the wound infection

Fig. 36.20: Food particles-definite indication if found in DPL for laparotomy

Fig. 36.21: Bile and blood in the peritoneal cavity

Blunt Abdominal Trauma, War and Blast Injuries and Triage

Fig. 36.22: Peritonitis following ilea! perforation. Golden time to operate is within 6 hours of perforation

Treatment Golden time to operate is within 6 hours • Perforation: Single or multiple, have to be closed, after trimming the edges by using nonabsorbable sutures such as silk. • A lacerated or a macerated bowel has to be resected. • Bleeding mesenteric vessels have to be ligated, haematoma must be evacuated and bowel should be inspected for any ischaemia. Food particles and bile should be evacuated (Figs 36.17 and 36.23). • A perforation of ileum close to the ileocaecal junction is treated by ileocolectomy rather than simple closure for the fear of enterocutaneous fistula, due to suture line leakage.

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Fig. 36.23: Colonic perforations. The patient presented 7 days later with abdominal distension and early sepsis-steering wheel injury to the transverse colon

Fig. 36.24: Sigmoid perforation

IIMN..........� • • • • •

SMALL BOWEL INJURY

Acute abdominal pain Guarding and rigidity Rebound tenderness Gas under diaphragm in plain X-ray abdomen erect Laparotomy and closure of perforation COLONIC INJURIES

• Blunt injury of the colon is not uncommon. • Mobile sigmoid is more prone for injury than fixed parts. • Steering wheel injury can directly crush the transverse colon and can cause perforation. • Bruise or laceration of the colon can undergo ischaemic necrosis and it can present after 5-7 days with signs of peritonitis/sepsis (Figs 36.23 and 36.25). • Diagnosis is by clinical examination/contrast enhanced CT scan. • Depending upon the contamination, contusion or laceration and duration of injury, treatment can be resection and

Fig. 36.25: Traumatic faecal fistula

anastomosis within 6-8 hours of the injury or simple suturing or diversion colostomy if gross contamination is present. • Even in penetrating injury, primary closure can be done.

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DUODENAL INJURIES

PANCREATIC INJURIES

• Retroperitoneal duodenum is commonly injured. • Steering wheel, belt or a blow in the epigastrium may injure the duodenum as it is crushed against the spine.

Because of anatomical close approximation of pancreas with vertebral column, blunt injury abdomen in the epigastrium, kicks or seat belt injuries crush the pancreas against the vertebral column (Key Boxes 36.8 and 36.9).

Clinical features • Peritonitis features are not common as it is the retro­ peritoneal duodenum (part II and III) that is injured. • Tenderness is present on deep palpation. • Being retroperitoneal, these injuries manifest late with abscess formation or fluid in lesser sac, etc. Investigations • X-ray abdomen - Obliteration of psoas shadow -Air outlining the kidney-Chilaiditi's sign (Fig. 36.26) -Absence of air in the duodenum • Raised serum amylase is one of the biochemical parameters that should arouse a suspicion of pancreatic injuries along with duodenal injury. Treatment • Golden time to operate is within 6 hours. • When in doubt, about narrowing of lumen, duodeno­ jejunostomy may be indicated. • When in doubt regarding duodenal fistula, tube duodenostomy is done. • Duodenal haematoma is managed conservatively.

Mill belt: Saree or churidar cloth may be caught in the belt of a running conveyor belt and result in compression force in the centre of the abdomen (Figs 36.27 to 36.31 and clinical notes see next page). Diagnosis • Pancreatic injury alone is diagnosed when patient presents with a pseudocyst of the pancreas 2-3 weeks following an lnJUry. • Very often, laparotomy is done for haemorrhage or perforation. In such situations, retroperitoneal bleeding, collection of bile or collection of fluid in the lesser sac arouses suspicion of pancreatic injuries. Treatment 1. Pseudocyst following blunt injury abdomen invariably requires surgical drainage, e.g. cystogastrostomy because of injury to pancreatic duct. 2. Injury to body and tail require subtotal pancreatectomy with splenectomy. 3. Rarely, pancreaticoduodenectomy may be required for significant injury to the head of pancreas with injury to the duodenum. Complications • Pancreatic fistula • Pancreatic pseudocyst • Pleural effusion

MMM..........,.� • • • • •

PANCREATIC INJURIES Anatomically hidden Very often, injuries missed Peritonitis features are not seen Dangerous because of enzymatic activation Can manifest as pleural effusion

IMtllWlllllliF 1 Fig. 36.26: Air outlining small portion of the kidney and air pockets under the diaphragm-Chilaiditi's sign

• • • • • • •

PANCREATICOOUOOENAL INJURIES Diagnosed late Peritonitis features are minimal Shock is very rare At laparotomy, they are missed Surgical treatment needs more skill and experience. Feeding jejunostomy is very useful Mortality and morbidity around 50%

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Indian women-vulnerability (Figs 36.27 to 36.31)

The saree and churidar are traditional Indian dresses. One end of the saree is tied around the waist and the other draped freely along the shoulder. If care is not taken, the free end often gets caught in the mill belt and causes injuries around the waist (blunt abdominal trauma) due to the drag created. The impression created on the abdomen is by the saree and not the mill belt. The shawl of the churidar (has two free ends) can get caught in a belt or a wheel to cause injuries around the neck. The drag may be sufficient to cause even strangulation. The author remembers a case of scalp avulsion due to long hair of a woman getting caught in a mill belt.

Fig. 36.29: In the OT

Fig. 36.27: Grinding mill having a conveyor belt (Courtesy: Dr Rakesh Hegde, Associate Professor, Department of Surgery, KMC, Manipal, 2006-2009)

Fig. 36.28: Impression of the saree all around waist in a lady who was dragged by the conveyor belt when her saree got caught

Fig. 36.30: On laparotomy, blood gushing out (Courtesy: Dr Raghunath Prabhu, Associate Prof, KMC, Manipal)

Fig. 36.31: Pancreas split into 2 parts

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RENAL INJURIES Types (Fig. 36.32) I. Minor injuries: Subcapsular haematoma, mrnor laceration and renal contusions. II. Major injuries: Bleeding into renal pelvis from laceration ofmedulla, corticomedullary rupture, hilar injury. Clinical features • Haematuria is the most important (80-90%) sign ofrenal injury. It may be mild, or sometimes can be massive depending upon the extent of injury. It may be absent in renal pedicle avulsion. • Loin bulge due to perinephric haematoma • Bruising ofsoft tissue in the loin • Retroperitoneal haematoma compressing on splanchnic nerves (meteorism) results in paralytic ileus, which causes abdominal distension. • Associated injuries such as fractures of the transverse process oflumbar spine may be present. Investigations

Fig. 36.33: Renal trauma: Right kidney is transected and upper pole is displaced by a large haematoma. Left kidney is normal (Courtesy: Dr Padmaraj Hedge, Prof and Head, Dept. of Urology, KMC, Manipal)

1. Intravenous pyelography can demonstrate • Intrarenal extravasation • Extrarenal extravasation (pararenal pseudohydrone­ phrosis due to extravasated blood and urine, slowly occluding pelviureteric junction). • Function of injured kidney • Function of opposite kidney 2. Ultrasound and CT scan are other investigations which are useful when there is an expanding haematoma (Figs 36.33 and 36.34).

2

3

Fig. 36.32: Closed renal trauma: (1) Subcapsular haematoma, (2) laceration, (3) avulsion of one of the poles and (4) avulsion of renal pedicle

Fig. 36.34: Liver and kidney injury

Treatment 1. Conservative: Minor injuries are managed conservatively with close monitoring of vital signs such as pulse, blood pressure, temperature and respiration, Hb% and PCV. • Sedation and analgesics are also given 2. Surgical exploration • Small laceration sutured over gel foam or by using detached muscle. • Major lac eration involving one pole-a partial nephrectomy is done. • Major m u ltiple lac erations, avulsions, require nephrectomy.

897

Blunt Abdominal Trauma, War and Blast Injuries and Triage

RETROPERITONEAL HAEMATOMA • It is quite common because of accidents, fall from height. • Fracture vertebrae, fracture pelvis, injury to retroperitoneal veins. • Bleeding from vena cava and aorta can be fatal. • Haematomas which are not expanding should not be disturbed. Diagnosis and management of retroperitoneal haematoma as shown in Fig. 36.35. Pelvic fractures and retroperitoneal haematoma • Pelvic fractures are also an important cause of retroperi­ toneal haematoma. • The most frequent mechanisms causing pelvic fractures are motor vehicle accidents, motorcycle accidents, falls and accidents involving pedestrians. Associated injuries to urethra in males should be ruled out first. Per rectal examination should be done to evaluate the position of the prostate. CT scan is done to assess pelvic fracture and also to assess retroperitoneal haematoma (Figs 36.36 and 36.37). • Retroperitoneal bleeding can be arterial, venous, or osseous in origin. Unstable pelvic fractures are generally associated with increased blood loss. Posterior fractures with involvement of the sacroiliac joint are frequently associated with arterial bleeding, which can be controlled by embolisation of the bleeding vessel, usually branches of the internal iliac artery. Unstable fractures should be fixed by external fixation. Expanding haematoma should be explored. Control the bleeders, otherwise pack the pelvis. Nonexpanding haematoma should not be explored. Wait and watch.

I Zone I: Central abdominal A. Supramesocolic haematomas are: Usually due to injury to suprarenal aorta, coeliac plexus Requires exploration and repair

. .

B. lnframesocolic haematomas are usually due to infrarenal artery or vein, vena caval injuries

. .

I

.

Fig. 36.37: Retroperitoneal haematoma

Retroperitoneal haematoma

I

Ultrasound/CT scan

Zone II: Flank haematomas

.

Fig. 36.36: Retroperitoneal haematoma contrast CT

They are due to injury to renal artery, renal vein or renal injuries If they are not expanding, they can be observed Many patients require partial or total nephrectomy Pedicle injury can be repaired if renal angiogram is done within 6 hours

I

I

I

Zone Ill: Pelvic haematoma • Usually due to fracture pelvis • Iliac artery injury should be suspected when there is absent groin pulse and when haematoma is expanding • Bleeding can be massive in fracture pelvis-aggressive resuscitation nonoperative is the gold standard • Therapeutic embolisation of bleeder, and external fixation of pelvic fraclure are other methods to control haematoma

Fig. 36.35: Diagnosis and management of retroperitoneal haematoma

I

Zone IV Portal retrohepatic area-packing

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GENERAL PRINCIPLES IN A BLUNT INJURY ABDOMEN • A patient should be admitted to the hospital and carefully monitored if there is a slight doubt regarding blunt injury abdomen. • Repeated examination, careful monitoring of pulse rate, temperature and blood pressure, chest X-ray, estimation of Hb%, frequently help in many cases of silent blunt injuries (Fig. 36.38). • Most of the cases today are polytrauma cases, Hence, all systems should be examined. Among all these, priority should be given to life-threatening, salvageable injuries such as extradural haematoma, haemothorax, splen ic injuries, liver injuries. • It is easier to make a diagnosis of fracture 1 (revealed injuries) which can be treated later. FRACTURE CAN WAIT BUT NOT RUPTURE. Concealed injuries should be carefully looked for. • Undoubtedly, diagnostic peritoneal lavage, ultrasound (CT scan is the immediate noninvasive investigation) help in diagnosis of more than 90% of cases of blunt injury abdomen. • Adequate blood, appropriate antibiotics, aggressive resusci­ tation before surgery to treat hypovolaemia and shock are the major factors which decide the outcome of surgery. • In a major accident involving many patients and limited resources, quick decision should be taken regarding triage-who can be saved, who cannot be saved (Figs 36.39 and 36.40).

Fig. 36.39: Pelvic fracture with ileal injury-frequent clinical examination revealed guarding and rigidity ( Courtesy: Dr Rajesh Sisodia, Associate Prof, KMC, Manipal)

Fig. 36.40: Splenic haematoma-initially managed by conservative method. However, patient was developing hypotension-importance of careful monitoring of the patient in intensive care unit

BLAST INJURIES

Fig. 36.38: A case of missed liver injury with haemoperitoneum.

Repeated examination revealed deteriorating general condition. CT scan provided the diagnosis

• Bursting of bombs or shells rupture their casing and impart high velocity to resulting fragments. These fragments cause more devastating injuries than bullets. All explosives are accompanied by a complex blast wave. • The two main components are: Blast pressure wave (dynamic overpressure) with positive and negative phase and mass movement of air (blast wind). • Positive phase of blast wave lasts few milliseconds (close to the explosion it may be over 7000 kN/m2 ) (tympanic membrane ruptures at 150 kN/m2 ).

1 Even a grandmother can diagnose fractures. However, it needs lot of experience and skills in diagnosing blunt injury abdomen.

Blunt Abdominal Trauma, War and Blast Injuries and Triage

• Like sound waves blast, pressure waves flow over and around an obstruction and affect persons sheltering behind a wall. The pressure affecting such a person is known as incident pressure (pressure at 90° to direction of travel of blast shock front). • Person standing in front of a wall facing an explosion is subject to added effect of reflected pressure. Mass movement of air displaces air at supersonic speed. This disrupts environment, hwting debris and people. Blast wave under the water travels at great speed and to greater distance. Injuries tend to be complex and severe. PEARLS OF

WISDOM

Structures injured by primary blast wave are ears, lungs, heart and gastrointestinal system.

• Most will have combination of blunt, blast and thermal injuries. Deafness, lung contusion, capillary leakage and haemorrhage into alveoli and ARDS precipitated by over transfusion are the features. Perforation of the intestines and penetration injuries to the eye are the other features. • Management consists of resuscitation in a well-equipped trauma unit, blood transfusions, intensive care monitoring, antibiotics and appropriate surgical procedures. WARFARE INJURIES Penetrating missile wounds, injuries from blast phenomena and burns are typical features of modem conventional war. The most common wounding agent in surviving casualties is a fragment wound and not a bullet wound as many erroneously believe. The aim in modem war is to incapacitate and not to kill. Hence, large number of surviving casualties are a major financial and logistic burden on a nation engaged in war. Wound Ballistics and Mechanisms of Injury Bullets fired from hand guns are propelled at low velocity, have low available energy and result in low velocity transfer wounds ( l 00-500 J), whereas those from assault rifle have high velocity and have high available energy (2000-3000 J) and they cause high energy transfer wounds. Low energy transfer wounds leave injury confined to wound tract. High energy transfer wounds causes local laceration, crush injury and also cause remote injury from wound tract due to temporary cavitation phenomena. Management • Entrance and exit wounds do not indicate considerable damage that may have occurred to deeper structures. • Resuscitate as per ATLS guidelines. • Record the wounds in case sheets, take photographs if necessary.

899

• Under anaesthesia, excise skin around ently and exit wounds. give liberal longitudinal incision through skin and deep fascia, which allows proper visualisation of underlying structures. • Debride (cut till healthy tissues are seen) all dead tissuesdead muscle does not bleed or contract, looks dusky. • Identify neurovascular bundles and examine them. • Dissect and mark injured nerves for possible future repair. • Repair arteries and veins if injured • Give thorough wash and Jet out all the dirt. • Injured tendons are trimmed and tied for easy identification at future surgery. • Fix bones by appropriate methods. • Cover the wound with absorbable dressing. • Appropriate antibiotics and injection tetanus toxoid are given. • Amputation may be necessary if limb is grossly mutilated • Delayed primary closure is done (4-6 days later) once the wound starts healing. MISSILE WOUNDS OF ABDOMEN • Every penetrating and perforating missile wound of the abdomen should be explored by laparotomy. A full midline incision from xiphistemum to pubis is recommended and it may be extended to thorax if necessary. • The rest of the treatment depends on the nature of injury. Bleeding mesenteric vessels are ligated, injured small bowel is repaired by suturing or by resection and anastomosis. In colonic injuries, simple closure or closure with protective colostomy is necessary depending upon the nature of the colonic injury and contamination. • Liver, splenic, pancreatic and renal injuries have been discussed in respective chapters. PENETRATING TRAUMA OF THE ABDOMEN Today all penetrating injuries of the abdomen need not be explored by laparotomy. Good physical examination of the patient, entry and exit point, and haemodynamic status of the patient followed by investigations such as ultrasonogram and CT scan will guide the decision for Japarotomy.

IIMINMIWIIIIIIIIIW� • • • •

INDICATIONS FOR LAPAROTOMY Tenderness, guarding, rigidity Unexplained shock Evisceration of contents Positive investigations - Positive DPL - Gas under diaphragm - IVP, cystoscopy, cystogram - Ultrasonogram - CT scan

Manipal Manual of Surgery

900 TRIAGE

• The tenn triage is derived from the French word trier, meaning to sort. This is a method of sorting out injured patients, during mass casualties depending on the severity of injury. If you are the first on the scene, first priority is to get expert help (call up fire services, regional trauma centre, etc). • Triage is a skilled activity by a Trauma team, wherein there is a leader (usually senior-most doctor/surgeon) and there are many assistants. The leader sorts out patients (people) depending upon their severity of injury. • Each of the patients can be colour coded (a coloured flag is attached to them). Examples: 1. Immediate help is necessary-Red: Otherwise the person will die in a few minutes if no treatment is offered, e.g. obstructed airway, tension pneumothorax. These patients may 2. Urgent help is necessarydie in 1-2 hour if no treatment is given. Examples are cases of massive bleeding and hypovolaemia. 3. Delayed-Green: These patients can wait. Examples are minor fractures. 4. Expectant-Blue: Very severe injury and unsalvageable. An attempt to treat them, may delay treatment to other patients who are salvageable. 5. Dead people are flagged-white or black. PEARLS

OF

WISDOM

The leader assesses the patient-flags and moves forward and it is his assistant who carries out the necessary resuscitation. The leader should not resuscitate any patient. There are others waiting for his expert help.

............................. . KEY BOX 36.11

FUNDAMENTAL STEPS • Airway management • Breathing • Circulation • Disability and assessment of level of consciousness by Glasgow Coma Scale. • Exposure of the patient fully for thorough examination. • Finger evaluation and tubes

A common scheme 1. Can the patient walk? Yes-Delayed (green) No-Check for breathing 2. Is the patient breathing? No: Open the airway Are they breathing (ventilating) him? Yes-immediate (red) No-DEAD (white)

Yes: Count or estimate respiratory rate (over 15 sec). < 10 to> 30 per minute-immediate (red) 10-30 per minute-check the circulation. 3. Check the circulation Pulse>120/min (capillary refill>2 s)-immediate (red) Pulse< 120/min (capillary refill< 2 s)-urgent (yellow). • If any regional trauma centre is well equipped and nearby one has to transport all the injured patients to hospita: (scoop and run), where expert help is available. Meanwhile trauma centre can be alerted about the aITival of casualties. If expert help is far away, then one may have to treat the patients at the accident site (stay and play). Resuscitation is done as per ATLS guidelines.

MIMIMll-11111-IW� QUICK REMINDER MNEMONIC AT TREATMENT CENTRE

• Lift jaw • Intubation: Airway • Fallen tongue/foreign body to be checked • Tracheostomy/cricothyrotomy/oropharyngeal or naso­ pharyngeal throat suction • Jaw fractures to be ruled out Remember as LIFTJAW

• During these exercises do not forget to take care of your own safety, in burning vehicles, burning or falling buildings, etc. PEARLS OF

WISDOM

An initial quick evaluation of the patient for anaemia, level of consciousness, ifnecessary volume replacement, application of cervical (C) collar to the neck, etc. are done. Management (Key Boxes 36.11 and 36.12) These six initial steps are included under primary survey and resuscitation inATLS (advanced trauma life support) are given below. Airway with cervical spine protection • Rapid assessment of signs of obstruction-foreign body, laryngeal and faciomaxillary fracture, fallen back tongue. • Lift the jaw, introduce airway, good suction of throat, intubation or even tracheostomy if necessary. • In polytraurna, assume cervical spine fracture and cervical collar is applied immediately (Fig. 36.41). Breathing and 02 administration • Rule out tension pneumothorax, multiple fractures of ribs and haemothorax, surgical emphysema, etc. • All trauma patients should receive high-flow oxygen.

.,............

Blunt Abdominal Trauma, War and Blast Injuries and Triage

• • • •

DISABILITY: AVPU SYSTEM

901

�

Awake Open eyes to Voice Open eyes to Painful stimulus Unarousable

Disability Glasgow Coma Score and assessment of pupils can help immediately to assess the status of the patient. Exposure with control of environment Complete inspection of the patient by taking out clothing. Keep patient warm. Fig. 36.41: Management of a polytrauma patient in an intensive care unit-cervical collar has been applied and patient is being ventilated PEARLS OF

WI SDOM

Tension pneumothorax is a clinical diagnosis. Do not delay the treatment while waiting for chest X-ray.

Circulation and control of bleeding • Immediate and quick assessment of circulation status, evidence of shock and evidence of internal or external bleeding should be looked for. • Feeble thready pulse, hypotension indicates volume or blood loss. Splenic, liver or mesenteric injuries can be diagnosed by ultrasound. Bleeding into the pleural space or pericardium needs further tests-CT scan or echo cardiography, etc. • Assess the class of haemorrhage • Two large bore intravenous lines to be placed. • Initial bolus of 2 litres of crystalloid solutions. PEARLS OF

WI SDOM

T he immediate goal is to arrest bleeding rather than replacing the blood.

Fingers and tubes • Quick examination of all orifices. Examples: Look for bleeding from the ear, nose, oral cavity, rectum, vagina, and urethra. • Nasogastric tube, endotracheal tube, catheter, intravenous line or central line are the immediate requirements of a polytrauma patient. Please note Remember following mnemonics in blunt injury abdomen: • AMPLE - page 887 • ABCDE

- page 887

• FAST

- page 886

• LIFT JAW

- page 900

• AVPU

- page 901

WHATISNEWI N THISCHAPTER?/RECE N TADVANCES

• All investigations have been updated. • Liver and pancreatic injury have been discussed in more detail. • More colour photographs and key boxes have been included.

902

Manipal Manual of Surgery

MULTIPLE CHOICE QUESTIONS 1. The most common bedside investigation done for suspected blunt abdominal trauma for bleeding is: A. CT scan B. MRI scan C. Diagnostic peritoneal lavage D. Ultrasound 2. Which one of the followings is a definite indication for laparotomy in blunt injury abdomen? A. Splenic injury B. Pancreatic injury C. Liver injury D. Aspiration of bile in the peritoneal aspirate 3. If air bubble like picture is found within the thorax following blunt injury abdomen what do you suspect? A. Splenic rupture B. Liver injury C. Injury to the stomach D. Diaphragmatic injury 4. How do you rule out a head injury with factors given below? A. Hypotension responding to fluid B. CSF rhinorrhoea C. Fracture skull D. Hypertension and bradycardia 5. Which is an important sign of hollow viscus perforation? A. Cullen's sign B. Grey Turner's sign C. Mallet Guy sign D. London sign

6. The following are true for conservative manageme01 of liver injury except: A. Hollow viscus injury should not be there B. Free contrast in and around liver in CT scan C. Grade I and Grade II injury D. Haemodynamically stable patient 7. Pringle manoeuvre refers to: A. Compression of left gastric artery to stop the bleeding from giant gastric ulcer B. Compression of hepatic artery to stop the bleeding during liver resection C. Compression of hepatic artery and portal vein in front of foramen of Winslow D. Compression of gastroduodenal artery during Whipple's procedure 8. The salvage procedure to buy time in massive bleeding from liver include following: A. Pringle manoeuvre B. Plug by ornentum C. Perihepatic packing D. Portovenous shunt 9. Perforation within 4 cm of the ileocaecal junction following blunt injury is better treated by: A. Suturing and drainage B. Resection and anstornosis and drainage C. Suture and bypass D. Exteriorisation 10. Which of the following is not a feature of retro­ peritoneal duodenal perforation? A. Can occur with steering wheel injury B. Chilaiditi sign may be present C. Free gas under diaphragm D. Guarding and rigidity is minimal

ANSWERS 1 D

2 D

3 D

4 D

5 D

6 B

7 C

8 C

9 B

10 C

37 Abdominal Mass • Clinical examination of abdominal mass • Mass in the right iliac fossa • Firm to hard nodular mass in the umbilical region

Introduction The abdomen is like Pandora's box. However, a student who is examining a case of abdomen is like an investigating CBI officer. He has to collect information at every level of examination, i.e. history, past history, general examination and abdominal examination. An attempt has been done here to highlight the importance of history and clinical examination. Ten points in the history, if taken and analysed properly may give a definite clue in majority of cases. After getting this clue, clinical examination of the mass may become easy.

• • • •

The cystic mass in the abdomen Mass in the epigastrium Mass in the right hypochondrium What is new?/Recent advances

3. Left hypochondrium 4. Right lumbar region 5. Umbilical region 6. Left lumbar region 7. Right iliac fossa 8. Hypogastrium 9. Left iliac fossa

CLINICAL EXAMINATION OF ABDOMINAL MASS (CLINICS) 2

REGIONS IN THE ABDOMEN • Abdomen is divided into nine regions (quadrants) by two horizontal lines and two vertical lines. • Upper horizontal line or transpyloric line is midway between xiphisternum and umbilicus. • Lower hoizontal line (transtubercular line) is the line joining iliac crest tubercles of each side, about 5 cm behind anterior superior iliac spine. • The vertical lines are drawn on either side through midpoint between anterior superior iliac spine and symphysis pubis. Following are the nine regions of the abdomen (Fig. 37.1). 1. Right hypochondrium 2. Epigastrium

3

5

4

7

•

6

8

9

Fig. 37.1: Regions of the abdomen (see text)

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Manipal Manual of Surgery

904

HISTORY 1. Abdominal pain: It is present in most of the cases of abdominal mass. Abdominal pain can be of the following types: A. Dull aching pain: ft suggests a solid organ enlargement. It is a continuous pain felt in the anatomical location of the swelling. Patients often describe it as a discomfort rather than pain. Examples • Liver enlargement: Pain in the right hypochondrium. It occurs due to stretching of parietal capsule (Glisson's) (Fig. 37.2A) • Splenic enlargement: Pain in the left hypochondrium • Renal enlargement: Pain in the back and costal region or costovertebral pain (Fig. 37.2B) • Enlarged lymph nodes (para-aortic), pancreatic tumours: Backache B. Colicky pain suggests hollow viscus obstruction. This pain is due to hyperperistalsis. It is severe and intermittent (comes and goes). Each attack may last for 5-10 minutes. The patient bends on himself, holds the abdomen and puts pressure on the abdomen which gives some kind of relief. Being visceral type of pain, it is not ve1y well localised. Following are a few examples: • Mass in the right iliac fossa (carcinoma caecum or ileocaecal tuberculosis). Initially there may be a vague discomfort. However, when partial obstruction occurs, it results in a colicky abdominal pain which is centrally located and sometimes unbearable. • Ureteric colic and biliary colic (Fig. 37.2C).

�---- Shoulder pain in acute cholecystitis

Pain from CBD colic Pancreatic pain Renal colic

/

y

\

Pain from pelvic organs

Fig. 37.3: Referred pain (posterior view)

• Carcinoma pyloric antrum or pyloric stenosis produces colicky upper abdominal pain with gastric peristalsis. However, this type of pain is not an unbearable one. C. Referred pain: Tuberculosis of spine is a common problem in India. Often patients present with iliopsoas abscess. Patients can complain of referred pain in the lower abdomen (Fig. 37.3). 2. Sensation of fullness/early satiety • Carcinoma of the stomach and pyloric obstruction. Also hepatoma or large pancreatic tumours can cause extraluminal compression on the stomach resulting in sensation of fullness in the abdomen. • Early satiety is due to loss of receptive relaxation of stomach due to malignant infiltration of muscle layer. 3. Vomiting • Persistent, profuse, projectile and nonbilious vomiting suggests pyloric stenosis. Chronic duodenal ulcer and carcinoma stomach are the common causes of pyloric obstruction (pain is absent/negligible). • Persistent, profuse, projectile, bilious vomiting­ intestinal obstruction. For example, ileocaecal tuberculosis, stricture of the small bowel, adhesions (pain is severe and colicky). 4. Haematemesis • Epigastric mass suggests carcinoma stomach • Splenomegaly may be an indication of portal hypertension.

A

Renal cell carcinoma

Figs 37.2A to C: Source of the pain

5. Bleeding per rectum • Fresh blood with or without melaena----carcinoma rectum • Melaena-carcinoma stomach, portal hypertension 6. Loss of appetite and loss of weight • These are common symptoms of GI malignancies. Please note that these two symptoms are seen not only in intra­ abdominal malignancies but also in many diseases such

Abdominal Mass

as tuberculosis. However, it should be noted that one of the earliest signs of carcinoma stomach is loss of appetite. Severe weight loss is an early and important feature of carcinoma body of the pancreas. • Significant weight loss refers to loss of 10 kg or more in the last 6 months. 7. Bowel habits • Fresh bleeding per rectum: Carcinoma rectum • Blood and mucus (bloody slime): Carcinoma rectum • Alternate constipation and diarrhoea: Carcinoma colon 8. Jaundice • Progressive, persistent, pruritic jaundice: Periampullary carcinoma or carcinoma head of pancreas. However, in periampullary carcinoma, fluctuation can occur if growth ulcerates. • Mild recunent jaundice: Haemolytic anaemia. • Intermittent jaundice, pain, fever: Charcot's triad­ stone in the common bile duct. 9. Haematuria: Fresh bleeding/clots: Renal cell carcinoma. 10. Fever • High-grade fever, with chills and rigors: Stone in common bile duct • Low-grade fever: Hepatoma, renal cell carcinoma, lymphoma. Fever is due to some pyrogens released into circulation or due to tumour necrosis. • In a tropical country like India, hepatomas with fever are often diagnosed as amoebic liver abscess and mistreated. ON EXAMINATION Inspection

• The patient is asked to breathe well with mouth open. • Sh1dents should spend a few minutes watching the abdomen carefully. 1. Shape of the abdomen • Scaphoid in normal cases • Protuberant in fatty abdomen. • Generalised distension with fullness in the flanks is usually due to ascites. • Localised distension can be due to a mass • Presence of step ladder peristalsis indicates small bowel obstruction, visible gastric peristalsis indicates pyloric stenosis and right to left peristalsis indicates colonic obstruction.

905

2. Restricted movement of any one region of the abdomeu indicates an inflammatory pathology. 3. Umbilical nodule (Sister Joseph's) indicates intra­ abdominal malignancy (carcinoma of stomach, colon, pancreas) 4. Details about the mass such as size, shape, surface, borders, movement with respiration have to be mentioned if mass is visible. If the details about the mass cannot be appreciated or if mass is not clear on inspection, it is better to say "there is fullness" rather than trying to manipulate the details about the mass. 5. Inspection of male genitalia: If scrotum is empty, it could be a case of undescended testis 1•2. Palpation

Methods of palpation Following are the methods of palpation available to the clinician and done depending upon the merits of the case: 1. Superficial palpation: Gentle superficial palpation of the abdomen gains confidence of the patient. It can detect superficial lesion of the abdominal wall such as lipomatosis, neurofibromas or fibromas, etc. It can also detect an area of tenderness, so that clinician is careful while doing deeper palpation. Superficial palpation is done with the flat of the hand or fingers. 2. Deep palpation: These are important requirements for deeper palpation: • Patient should be well-relaxed, with flexion of the knee for about 45 degrees. • The patient's face should be turned to the opposite side and he is asked to breathe comfortably with open mouth. • Deep palpation should be started from the quadrant situated diagonally opposite to the site of pain. • Palpation should cover not only the 9 quadrants of the abdomen, but also 2 more quadrants, i.e. the 2 renal angles and 12th quadrant-external genitalia in males3 • • Deep palpation is carried out with the palmar surface of the fingers and some degree of angulation depending upon the depth of palpation. Tests

I. Movement with respiration: This test is done by placing the fingers (hand) over the lower border of the swelling and the patient is asked to take a deep breath. Movement with respiration is positive when there is "up and down" movements not anteroposterior movement. Any structure

1 A case of mass abdomen diagnosed to be soft tissue sarcoma or lymphoma of the para-aortic node region by U/S proved to be a seminoma in an undescended testis. Patient said that 6 months back his right testis was removed by a 'groin' incision. 2Inspection and palpation of external genitalia is important in males only, NOT TO BE DONE in females. 3Don't forget the 12th man in a cricket match. He is also an important player.

Manipal Manual of Surgery

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............................. KEY BOX 37.1

.

MOVEMENT WITH RESPIRATION • Liver, spleen and stomach masses move freely with respiration • Gall bladder mass also moves freely with respiration because of its proximity to liver • Mass arising from hepatic flexure and splenic flexure of colon also have some mobility because it is in contact with liver and spleen • Renal masses exhibit minor degree of movement with respiration because of indirect attachment to diaphragm

in contact with diaphragm moves with respiration (Key Box 37.1). For example: • Liver, stomach, spleen, gall bladder move very well with respiration. • Splenic flexure growth, due to contact with the lower pole of the spleen and hepatic flexure growth due to contact with liver move with respiration.

•

• C

3. Size, shape and surface • An egg-shaped mass or globular mass suggests gall bladder lesion (Fig. 37.4A). • A horseshoe shape may indicate a horseshoe kidney (Fig. 37.4B) with pathology, e.g. hydronephrosis. • Reniform-shape suggests a renal swelling (Fig. 37.4C). • Large nodular surface is seen in following conditions (Fig. 37.4D): - Polycystic kidney - Secondaries in the liver - Group of lymph nodes • Smooth surface usually indicates a benign lesion. - Splenomegaly, hydronephrosis, ovarian cyst, gall bladder swelling. • Irregular surface is an important feature of malignancy such as carcinoma of the stomach, carcinoma caecum. 4. Consistency • Hardness is a feature of malignant lump. Thus, hepatoma, carcinoma stomach, pancreatic carcinoma present as hard

• D

Figs 37.4A to D: Shapes of the intra-abdominal mass

• Renal swelling moves with respiration because kidney is enclosed by fascia of Gerota which is attached above the diaphragm. 2. Finger insinuation test: This test has relevance in an upper abdominal mass. • Liver and spleen are under right and left costal margins respectively. Hence, it is not possible to get the upper margin or upper border of these organs. An attempt to invaginate between the costal margin and these masses is not possible. On the other hand, finger invagination under the costal margin is possible in a stomach mass.

B

A

• • •

• •

lump. However, it should be remembered that often the malignant lump is firm and not hard. Firm consistency is found in ileocaecal tuberculosis, nodes of lymphoma. A peculiar doughy feel is described for tuberculous abdomen. It is difficult to elicit fluctuation test for intra-abdominal swellings, and often tensely cystic swellings feel firm on palpation, e.g. pseudocyst of pancreas, hydro­ nephrosis, etc. Indentation or pitting on pressure can be found in a colon loaded with faeces. Temporary contraction of a stomach (visible gastric peristalsis) should not be confused as a mass.

5. Margins or borders (Fig. 37.5) • Upper border cannot be made out in liver, splenic and renal swellings. • Lower border is not appreciated in pelvic masses, e.g. uterine fibroid, ovarian cyst (pelvic). • A characteristic notch is felt in the anterior border of splenic swelling. • Lower border is sharp as in a malignant liver swelling. 6. Intrinsic mobility test (Key Box 37.2) • An intra-abdominal mass can be mobile if it has loose attachments or if it is not within the bony cage. Thus, liver, spleen, uterine mass are not mobile because of their location within bony cage.

907

Abdominal Mass

Line of mesentery

��:::------ Sharp border (liver)

-------- Round border (cyst)

A. Ovarian cyst

B. Mesenteric cyst

t

�+--- Irregular border (carcinoma)

• Fig. 37.5: Various types of borders

PEARLS

OF

WISDOM

Check for intrinsic mobility in different positions.

• Carcinoma pyloric antrum can exhibit movements in different positions-left lateral, right lateral or even in the sitting position. • Pancreatic carcinoma, advanced malignancies and lymph nodal masses also may not have intrinsic mobility. • However, there are a few swellings which have characteristic mobility. Examples A. Ovarian cyst is a freely mobile swelling which can be moved in all directions (Fig. 37.6A). B. Mesenteric cyst moves at right angles to the direction of the line of mesentery (Fig. 37.6B). C. Pseudopancreatic cyst may have a minimal side-to-side mobility (Fig. 37.6C). D. Carcinoma transverse colon has vertical mobility unless it is advanced (Fig. 37.6D).

............................. . KEY BOX 37.2

INTRINSIC MOBILITY- MASS • Side to side

Gall bladder

• Vertical

Transverse colon

• All directions

Ovarian cyst

• Right angle to the direction of mesentery

Mesenteric cyst

• Push back to renal

Kidney pouch

• Tree top mobility

Pancreatic cystadenoma

C. Pseudopancreatic cyst

D. Carcinoma transverse cyst

E. Tree top mobility

Figs 37.6A to E: Intrinsic mobility

E. Pancreatic masses: Even though they do not exhibit mobility, a cystadenoma of the pancreas because of the size and a nairnw base, will exhibit tree top mobility (Fig. 37.6E). Any big mass abutting the undersurface of the diaphragm also moves with respiration. F. Renal mass comes down during inspiration. As it comes down, it can be held back and can be pushed back to the renal pouch. 7. Plane of the swelling A. Leg raising test or head raising test • The purpose of this test is to contract rectus abdominis muscles (also other abdominal wall muscles). Intra­ abdominal swellings become less prominent. On the other hand, abdominal wall swellings become more prominent, e.g. fibroma, neurofibroma, or lipoma in the abdominal wall. • This test is done by asking the patient to raise his legs without bending at the knee (extended legs) or by raising the shoulders from the bed with arm folded over the chest.

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B. Nose blowing test or straining test • This test can be done by asking the patient to blow through the nose with mouth closed. The lateral abdominal muscles are more contracted with this test. • It should be remembered that a swelling or the mass which moves with respiration is obviously an intra­ abdominal mass. C. Knee-elbow test • This test differentiates an intraperitoneal mass from retroperitoneal mass. It is more useful when there is a mass in the centre of the abdomen-more so in the upper abdomen. To give a few examples, intraperitoneal cyst or intraperitoneal mass falls forward. On the other hand, pancreatic mass or a lymph node mass will not fall forward. The test has significance only in 'selected' cases. • However, knee-elbow test helps to differentiate expansile pulsation from transmitted pulsation. • Examples: A pseudocyst of pancreas will give transmitted pulsations because it overlies aorta. In the knee elbow position, pulsation disappears as it gets separated from the aorta. On the other hand, aneurysms exhibit expansile pulsations. • Intraperitoneal mass with pulsations over it is likely to be hepatoma. Retroperitoneal mass with pulsations over it is pancreatic mass. Special tests 1. Bimanual palpation: Grossly enlarged swellings may

be bimanually palpable such as liver, spleen, kidney.

2. Ballottability: 'Ballot' means to toss about. To ballot, the swelling should be bimanually palpable and there should be a gap or space between hands which are kept anterior and posterior to the mass. Typically, renal swellings are ballottable. This test is done when the patient is in supine position, by keeping one hand anteriorly in the lumbar region over the swelling and the other hand posteriorly in the renal angle. A gentle push is given from behind and the swelling touches the hand which is placed anteriorly and it goes back. Ballottability is because of perirenal pad of fat and due to 'pedicle'. Percussion 1. To demonstrate mild ascites, the patient is put in a knee­ elbow position and percussion is done around umbilicus. It gives a dull note if minimal fluid is present (normally area around the umbilicus is resonant). • Significant or moderate fluid in the abdomen is demonstrated by percussion of the centre and flanks of the abdomen in the lying down position and in the left or right lateral position.

• In the supine position, flanks give a dull note due to fluid. However, in the lateral position, fluid shifts down and coils of bowel float up. 2. Liver dullness is elicited in the 5th intercostal space and the dullness is continuous with the mass, if it is arising from the liver. 3. Splenic dullness is elicited in the 9th intercostal space in the left midaxillary line. 4. Percussion over the mass (Key Box 37.3): • Splenic and liver masses classically are dull to percuss. • Retroperitoneal masses may give resonant note because of intestines anterior to it. However, when they attain large size, e.g. sarcomas, they push the bowel to one side and hence, they are dull to percuss. • Stomach mass may give impaired resonant note because of solid growth and due to the presence of air in the stomach. • Renal angle percussion: In cases of enlargement of kidney, there will be a band of resonance anteriorly due to the colon but posteriorly it gives a dull note. • Hydatid thrill: It is demonstrated by placing 3 fingers over the swelling and percussing the middle finger. Due to the fluid in the cyst, the fluid thrill (after-thrill) is felt by the other two fingers. This clinical sign is rarely demonstrable.

............................. . KEY BOX 37.3

• Dull note • Resonant • Impaired • Shifting dullness

PERCUSSION Liver, spleen, renal angle Bowel anterior to the mass (e.g. retroperitoneal mass) Stomach mass Ascites

Auscultation (Fig. 37.7) 1. Loud noisy sounds (borborygmi) with or without peristalsis may indicate subacute obstruction. Such patients may be having ileocaecal tuberculosis or carcinoma caecum. This should be done at right iliac fossa to listen bowel sounds. 2. Auscultation over the liver mass may reveal a bruit as in a rapidly growing hepatoma. 3. Succussion splash is a splashing sound in cases of pyloric obstruction either due to carcinoma or due to chronic duodenal ulcer. 4. Perisplenitis and perihepatitis give rise to friction rub as in sickle cell anaemia due to repeated infarction and adhesions. 5. Ao1tic aneurysm will give a continuous murmur in the upper abdomen. 6. Auscultopercussion or auscultoscraping test is done to assess lower border of the stomach or greater curvature of the stomach.

.,,,...----- -------

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•

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internal jugular vein and subclavian vein on the left side This explains the significance of enlargement o Virchow's node. In 20% of cases, thoracic duct is sing!, and 10-15% of cases, it is double. • Significance of right supraclavicular node: Thi lymphatics from the right mediastinal lymph trunk, a11< from the posterior right thoracic wall which form th1 right upper lymph trunk drain into the commencemen of the right brachiocephalic vein. SYSTEMIC EXAMINATION

Fig. 37.7: Auscultation sites: 1-5 (see text in previous page)

Rectal examination Should be done in a case of intra-abdominal mass. 1. It can detect a carcinoma or a growth in the rectum in a case of secondaries in the liver. 2. It can detect secondaries in the rectovesical pouch. Blumer's shelf-as hard, nodular mass and rectal mucosa is free during digital examination. Vaginal examination Should be done to rule out carcinoma cervix or to detect lymph nodes in the pouch of Douglas. Bimanual examination This should be done in cases of pelvic masses. One hand (left) is placed over the mass in the hypogastrium and right index finger or fingers inserted in the vagina or rectum in virgin females and the left hand is pressed downwards and backwards above the pubic symphysis. By this manoeuvre, details of the pelvic mass, solid or cystic, uterine or ovarian, free or fixed can be made out. Examination of lymph nodes In cases of abdominal masses arising from lymph nodes, a thorough search of the body should be done to rule out other group of lymph nodes such as axillary, iliac, inguinal, neck nodes (lymphoma). Significance • Left supraclavicular nodes (Virchow's) are enlarged very often in visceral malignancies mainly from gastrointestinal tract. It indicates "inoperable" nature of the disease. Entire gastrointestinal lymph drains into the thoracic duct which joins the point of confluence of

Systemic examination should include respiratory system anc cardiovascular system. Evidence of tuberculosis of the ches gives a clue about the mass in the abdomen, which may be i tubercular mass. Differential diagnosis: Students are requested to refer clinica books for details. However, mass arising from five differern quadrants are discussed below. MASS IN THE RIGHT ILIAC FOSSA Parietal swelling A. Parietal wall abscess B. Desmoid himour Intra-abdominal A. Arising from nonnal structures B. Arising from abnormal structures FROM NORMAL STRUCTURES I. Intestines 1. Appendicular mass 2. Appendicular abscess 3. Ileocaecal tuberculosis 4. Carcinoma caecum 5. Amoeboma 6. Intussusception 7. Actinomycosis II. Lymph nodes 1. Acute lymphadenitis 2. Lymphoma 3. Secondaries III. Retroperitoneal structures 1. Sarcoma 2. Aneurysm 3. Iliopsoas abscess 4. Chondrosarcoma IV. In females 1. Ovarian cyst 2. Fibroid 3. Tubo-ovarian mass

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FROM ABNORMAL STRUCTURES 1. Undescended testis: Seminoma 2. Unascended kidney Differential diagnosis of mass in the right iliac fossa (Figs 37.8 and 37.9) I. Parietal swelling: They are extra-abdominal. On head or leg raising test, they become more prominent. They are uncommon swellings. A. Parietal wall abscess: It is a pyogenic abscess which can occur in a haematoma, or a pyaemic abscess which can occur as a part of pyaemia as in diabetic patients. Such abscesses are very tender, with warm surface and are associated with fever, chills and rigors. B. Desmoid tumour: It is an unencapsulated fibroma occurring in the abdominal wall. • Occurs in multiparous females. Repeated stretching of abdominal layers (due to pregnancy) is supposed to initiate formation of tumour. • It can also occur following abdominal wall injury including laparotomy. • It is a firm to hard swelling. • It has no capsule. Hence, it should be treated with wide exc1s1on. • It does not undergo sarcomatous change. • After wide excision, the abdominal wall has to be reconstructed by using mesh. 11. Intra-abdominal swelling: A. Arising from structures normally present in the right iliac fossa 1. Appendicular mass (Fig. 37. l 0): It is a tender, soft to firm mass which develops 48-72 hours following acute appendicitis. It is nature's attempt to limit the spread of infection by forming a mass consisting of omentum, terminal ileum, caecum with pericaeca/ fat and inflammatory oedema. It is managed conservatively by Oschner-Sherren's regime

Fig. 37.8: Mass in the right iliac fossa

because an attempt to remove the appendix may result in faecal fistula. 6-8 weeks later, an elective appendicectomy can be done. 2. Appendicular abscess: It will be a very tender, firm, fixed mass. Such patients will have fever with chills and rigors. 3. lleocaecal tuberculosis (Fig. 37.11): Hyperplastic variety of tuberculosis forms a chronic cicatrising granulomatous reaction involving terminal ileum, caecum and part of ascending colon resulting in a mass in right iliac fossa. It is a chronic, nontender, firm, nodular mass, may have mobility, situated slightly (lumbar) on the higher side. Features of tuberculosis are usually present. It is treated by limited resection followed by ileocolic anastomosis. 4. Carcinoma caecum (Figs 37.12 and 37.13): • More common in females, around 40-50 years of age. • It produces bleeding per rectum, severe anaemia, etc. • Hard, irregular mass in right iliac fossa with fixity or restricted mobility is a usual feature. Psoas spasm indicates infiltration into psoas muscle. It is treated by right radical hemicolectomy. 5. Amoeboma: Can be acute or chronic. It follows an attack of amoebic typhlitis (inflammation of the caecum). Amoeboma is tender and soft to firm. It is not common to find amoebomas nowadays because of effective treatment of amoebiasis with metronidazole, tinidazole, etc. 6. Intussusception: Acute or chronic intussusception can give rise to a mass in the right iliac fossa which is tender and soft to firm. When acute intussusception occurs in children, it is described as idiopathic intussusception. Chronic intussusception may disappear spontaneously. 7. Actinomycosis: This is a rare mass in the right iliac fossa which usually develops 2-3 months after appendicectomy. A woody hard, indurated tender mass with multiple sinuses is characteristic of this condition. Sinuses discharge sulphur granules which can trickle down. Unlike tuberculosis, narrowing of lumen of the gut and lymph node enlargement does not occur.

Fig. 37.9: Carcinoma caecum (right iliac fossa mass)

Abdominal Mass

Fig. 37.10: Appendicular mass-tender, laterally placed fixed mass

Fig. 37.11: lleocaecal tuberculosis-caecum is pulled up and more medial

8. Lymph node mass • Acute mesenteric lymphadenitis is common in children. It produces tender, nodular and firm mass in right iliac fossa. The child usually has fever. Acute lymphadenitis can also involve external iliac nodes as in filariasis. • Lymphoma involving external iliac nodes, nodular, finn to hard mass with involvement of other nodes, liver, spleen, etc. • Secondaries in lymph nodes (external iliac) from carcinoma ovary, cervix, etc. Nodes are hard and fixity is a feature. 9. Retroperitoneal sarcoma (Fig. 37.14) • Common in young patients • Huge, nodular, fixed lump involving lumbar, umbilical and right iliac fossa. Recent increase in size draws the attention of the patient. • Fixed to posterior abdominal wall • Later, obstruction of inferior vena cava results in oedema of legs. • Pressure on the ureter can give rise to hydronephrosis. • Liposarcoma is the commonest and may arise from pre­ existing lipoma. • Fibrosarcoma, haemangiosarcoma, leiomyosarcoma are other sarcomas. • It is treated by wide excision followed by radiotherapy.

Fig. 37.14: Retroperitoneal sarcoma

Fig. 37.12: Carcinoma cae­ cum-irregular, nontender hard mass-more lateral

911

Fig. 37.13: Proliferative lesions of carcinomas produce mass

• Chemotherapy is also helpful, when it is not possible tc remove the entire mass. • Debulking even if it is an advanced case is recom­ mended. 10. Aneurysm: Iliac artery aneurysm is rare and occurs in old-aged patients. It produces a soft, pulsatile swelling in the right iliac fossa. Bruit or thrill is usually present. 11. Iliopsoas abscess (Fig 37.15) • It is the result of tuberculosis of thoracolumbar spine. It should be suspected when a young patient complains of pain in the back referred to abdominal wall. • Spine movements are limited. • Gibbus is present. • Initially, it forms paravertebral abscess and later it gravitates down beneath the medial arcuate ligament and forms psoas abscess. Psoas abscess burrows into the thigh under inguinal ligament and forms iliopsoas abscess. • Fluctuation is present on both sides of the inguinal ligament. It is described F.19. 37.15: as cross-fluctuation test (Fig. 37 .16). lliopsoas abscess

Fig. 37.16: Cold abscess-the swelling was partly above and partly below the inguinal ligament

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12. Chondrosarcoma of the iliac crest: • It is a hard, fixed tumour which cannot be separated from the bone. In females 13. Ovarian cyst: To sta1i with, the cyst develops in the pelvis and gives rise to discomfort in the lower abdomen. As the cyst grows, it comes out of the pelvis and forms a mass in right iliac fossa. It has smooth surface, round borders, is cystic, freely mobile and can be pushed back into the pelvis. Sometimes, the cyst can attain huge size. Such freely mobile ovarian cysts have a long pedicle. Per vaginal examination gives the clue to the diagnosis. 14. Fibroid of the uterus: It presents as a firm to hard nodular mass in the suprapubic region and in the right iliac fossa. 15. Tubo-ovarian mass • It is usually tender • Pelvic infection is present • It is soft to firm • It can be bilateral B. Arising from structures which are not normally present 1. Unascended kidney: It can be either in the pelvis or in the iliac fossa. Such kidney is usually not very well-developed. It presents as a lobular mass. 2. Normal mobile kidney: It can be felt in lumbar region, iliac fossa and can be pushed back into the loin. 3. Undescended testis: It is palpable in right iliac fossa only when it is involved by Seminoma. It is an intra-abdominal testis and is hard, irregular, fixed mass. Absent testis in the scrotum clinches the diagnosis. Patient may have palpable para-aortic nodes, supraclavicular nodes, etc. FIRM TO HARD NODULAR MASS IN THE UMBILICAL REGION 1. Mass arising from lymph nodes a. Metastasis or secondaries is one of the common lymph node masses in the abdomen. Mass can be due to para­ aortic nodes from testicular tumour, melanoma, carcinoma of ovary, carcinoma of penis, carcinoma of rectum, colon, stomach in late cases. • A para-aortic lymph node mass has the following features: 1. Fixed 2. Does not move with respiration 3. No intrinsic mobility 4. Does not fall forward

5. Coils of bowel can be felt over the mass 6. Percussion may be resonant because of intestinal coils. b. Lymphoma: The mass is enlarged para-aortic group of lymph nodes. It has all features of nodes mentioned above. Presence of lymph nodes in the neck along with palpable liver and spleen clinches the diagnosis. c. Tuberculosis can affect para-aortic nodes. However, it is uncommon. 2. Retroperitoneal sarcoma • Common in young patients • Rapidly growing, enlarging mass in the abdomen of short duration. • It is firm, hard, nodular, fixed, does not fall forward and has intestinal coils anterior to it. • Large sarcomas can cause compression on inferior vena cava or on the ureter. Therefore, pedal oedema and hydronephrosis can occur. • Liposarcoma and fibrosarcoma are common. • Radical surgery should be attempted and it is the only hope of cure. (Many cases may require debulking and follow-up with radiotherapy and chemotherapy.) 3. Carcinoma body of pancreas (Fig. 37.17) • Cystadenocarcinoma of pancreas can attain a huge size. Otherwise, it is uncommon to get a large nodular pan­ creatic mass. However, carcinoma pancreas presenting as a palpable nodular mass indicates nonresectability. Presence of pulsations over the mass (transmitted) clinches the diagnosis. • Men in the 6th decade are usually affected. • Severe backache, loss of weight, recent development of diabetes suggest pancreatic pathology. • Jaundice does not occur unless and until liver secondaries develop. • It has all the features of retroperitoneal mass. • These cases are advanced with ascites, rectovesical deposits, etc. 4. Carcinoma transverse colon (Fig. 37.18) • Elderly patients present with constipation and bleeding per rectum. • Firm to hard nodular mass occupying umbilical region may be found. • It may have vertical mobility and being intra-abdominal, it falls forward. • Caecum may be distended. Right to left peristalsis may be visible.

Abdominal Mass Mass in the umbilical region

Fig. 37.17: Carcinoma body of pancreas

913

for details). Getting above the swelling is possible. Transmitted pulsations of the ao1ta can be felt over the mass which disappears on knee-elbow position. History of acute pancreatitis or blunt injury abdomen gives the clue to the diagnosis (Fig. 37.22).

Fig. 37.18: Carcinoma transverse colon

Fig. 37.22: Pseudocyst of pancreas (large)

Fig. 37.19: Omental mass

Fig. 37.20: Carcinoma stomach

Fig. 37.21: Pseudocyst pancreas

5. Tuberculous abdomen (Figs 37.19 and 37.20) • The mass can be rolled up omentum, with lymph nodes and coils of intestines which are matted. • This is common in children and also occur in young adults in India. • History of evening rise in temperature, loss of weight, loss of appetite, emaciation and improper digestion gives the clue to the diagnosis. • Ascites is present in almost all cases. • Features of subacute intestinal obstruction can also be present. THE CYSTIC MASS IN THE ABDOMEN Intra-abdominal cystic swellings are interesting swellings. They occur in young children, adults, middle-aged persons. There are many cases of cystic swellings which have given a surprise at laparotomy (notoriously so in females). In children cysts have confused many competent paediatricians!! Being intra-abdominal cysts, it is not possible to elicit fluctuation and very often they are film due to increased tension. The details of important cystic swellings are given below. 1. Pseudocyst of pancreas (Figs 37.21 and 37.22) Tensely cystic upper abdominal mass may feel fim1 and tender, and does not move with respiration (see page 607

2. Hydatid cyst of liver (Fig. 37.23) This swelling is of long duration, is symptomless or with dull pain in the upper abdomen. The cyst is spherical with smooth surface, rounded borders and feels firm. Since it is a mass arising from liver, it moves with respiration and getting above the swelling is not possible. Classical hydatid thrill, mentioned in the books, is rarely appreciated. Simple cyst of the liver can also present as a cystic mass (Fig. 37.24). 3. Mesenteric cyst These are congenital cysts, enterogenous or chylolymphatic, manifests in young children or during adolescence. Typically, the cyst is located in the umbilical region which moves at right angles to the direction of mesentery. Types of mesenteric cyst (Key Box 37.4 and Figs 37.25 to 37.27, see page 659 also) A. Chylolymphatic cyst is a lymphatic cyst arising from mesentery of ileum. It is a thin-walled cyst with clear fluid or chyle. It has a separate blood supply. Hence, enucleation is the treatment without sacrificing the bowel. B. Enterogenous cyst is a duplication cyst from the intestine or due to diverticulum of the mesenteric border of the intestine. It is thick walled and contains mucus. This cyst is treated by excision of cyst with bowel segment because both share the same blood supply. Complications • Torsion of the cyst resulting in acute abdominal pain. • Rupture of the cyst due to trauma. • Haemorrhage into the cyst.

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Fig. 37.23: Hydatid cyst

Fig. 37.24: Intra-abdominal cyst: Serous cyst in the liver

............................. . KEY BOX 37.4

• • • •

MESENTERIC CYST-TYPES Chylolymphatic cyst Enterogenous cyst Urogenital remnant Teratomatous dermoid cyst

Fig. 37.25: Enterogenous cyst at surgery

pelvis will have free mobility. On pushing the mass upwards there will be traction on the pedicle, which may result in pain (Fig. 37.28). In any female patient who presents with lower abdominal mass, ovarian mass has to be considered first, and only then consider other possibi I ities. 6. Retroperitoneal lymphatic cyst Retroperitoneal cyst is one of the commonest of lymphatic cysts, which grows slowly to attain large size. Typically it is painless, seen in young patients and is tensely cystic. The bowel loop may be felt over the mass (retroperitoneal mass), or bowel loops may be pushed to the side. 7. Encysted ascites This consists of ascitic fluid loculated by many loops of intestine along with omentum. Loss of weight, fever, anorexia, emaciation are the other features. 8. Abdominal aortic aneurysm (AAA) • Majority of cases are due to atherosclerosis and most of the aortic aneurysms are infrarenal. Hence, they present

Fig. 37.26: Chylolymphatic cyst-it can be excised without resection of the bowel

Fig. 37.27: Enterogenous cyst-it requires excision of intestine also along with the cyst

4. Hydronephrosis Large hydronephrosis can attain a huge size without producing any symptoms. Bulk of the swelling is confined to one side of abdomen, with prominent bulge in the loin. It is difficult to elicit fluctuation in a tensely cystic intra­ abdominal mass. Bimanual palpation and ballotability give the clue to the diagnosis. One of the large cysts of polycystic kidney can present as a large renal cyst. 5. Ovarian cyst It is a freely mobile, firm or soft mass in any quadrant of the abdomen. Such ovarian cysts, once they come out of

Fig. 37.28: Ovarian cyst

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915

with a swelling in the umbilical region or epigastric region and are associated with backache (Key Box 37.5). • Often they contain clotted blood. Hence, they feel firm, not compressible, fixed and tender. • Characteristic feature of an abdominal aortic aneurysm is expansile pulsation. This can be appreciated by palpating the swelling gently all around. In the knee­ el bow position, the pulsations do not disappear. (Transmitted pulsations disappear in the knee-elbow position.) • The femoral pulses may be normal unless there is thrombosis or rupture of the aneurysm, giving rise to features of acute ischaemia. • Pressure effects such as venous oedema due to pressure on the inferior vena cava or erosion of vertebrae may be found. • Ultrasound to confirm the aneurysm and also to rule out suprarenal aneurysm.

Fig. 37.29: Large lymphatic cyst arising from omentum

• It is treated by repair of aneurysm, by incising the aneurysm and suturing a dacron graft end to end, inside the aneurysmal sac. 9. Rare cystic swellings in the abdomen (Fig. 37.29) • Omental cyst: This is usually a lymphatic cyst which occurs in children and can attain a huge size. Sudden enlargement indicates haemorrhage. Excision is easy. • Large mucocoele of the gall bladder can present as a tense cystic, slightly tender mass in the upper abdomen.

............................. KEY BOX 37.5

.

AORTIC ANEURYSM Elderly males > 60 Hypertensive Expansile pulsation + Anterior rupture: 20%-haemoperitoneum Posterior rupture: 80%-retroperitoneal haematoma • > 6 cm size-dangerous

• • • • •

PEARLS

OF

WISDOM

One should not forget distended bladder in the lower abdomen as a cause of cystic swelling.

MASS IN THE EPIGASTRIUM Mass in the epigastrium is one of the common long cases kept in the examination. Students should consider mass arising from liver and stomach first. Other possibilities must be considered later because common cases are common.

A female child, aged 6 years, was examined by a paediatrician for generalised abdominal distension. All the investigations were normal. The child was put on antituberculous treatment, as the treating paediatrician diagnosed this case as tuberculous ascites. Child was brought back after 9 months with no improvement and having abdominal pain since 2 days due to sudden increase in the size of the swelling. A paediatric surgeon was consulted, who palpated the abdomen and said she does not have ascites but has a cyst and that the wall of the thin cyst can be felt. At exploration, a large cyst arising from the omentum and occupying all the 9 quadrants of the abdomen, was excised. Histopathological report was lymphatic cyst (Fig. 37.29).

CLASSIFICATION I. MASS ARISING IN THE ABDOMINAL WALL • First do the head raising test. If the mass becomes more prominent, it is extraperitoneal (abdominal wall). • Lipoma, neurofibroma or desmoid tumour arising from the rectus sheath can present as a mass in the epigastrium. • Also note epigastric hernia occurs in this region. It is a hernia, not a mass. PEARLS

OF

WISDOM

Any hard subcutaneous swelling in the abdominal wall of recent origin can be a metastasis.

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II. INTRAPERITONEAL MASS 1. Mass arising from the liver A. Hepatoma (Fig. 37.30): Liver is enlarged, hard, irregular, and nontender. However, rapidly growing hepatomas are tender, finn and even a bruit is heard over the swelling. Rapid deterioration of health in a cirrhotic patient is usually due to the development of a hepatoma. B. Secondaries in the liver (Fig. 37.31): Usually both lobes are enlarged, have nodular surface without a bruit. Jaundice is a late feature in secondaries of the liver. The primary may be obvious as a colonic mass, a stomach mass or a testicular tumour, etc. (see page 533). C. Hydatid cyst (Fig. 37.32): It is a benign swelling. History of contact with a dog is usually present. Epigastric swelling is due to enlarged liver which is smooth or irregular, nontender with rounded borders. Classical hydatid fremitus and thrill are rarely elicited. General health of the patient is usually good. D. Simple cyst (Fig. 37.33): It is not a clinical diagnosis but is mentioned here only for discussion. It is a serous cyst. Single big cyst can also be a part of polycystic disease of the liver. 2. Mass arising from the stomach • For all practical purposes, the only mass arising from the stomach in the epigastrium is carcinoma stomach. It is hard, i1Tegular and moves with respiration. Usually the patient is a male with loss of appetite and weight. Vomiting is a feature. If there is a growth in the pyloric antrum, visible gastric peristalsis can be seen in the epigastrium. (Students are hereby requested not to offer lymphoma of the stomach or GIST of the stomach as a clinical diagnosis unless asked for by the examiner, for a differential diagnosis.)

•

Fig. 37.30: Hepatoma

•

Fig. 37.31: Secondaries in the liver

3. Omental mass • Omentum gets involved in tuberculosis as a finn, nodular mass (Fig. 37.34) or in secondaries from intra-abdominal malignancies as a hard, nodular mass (Fig. 37.35). Classically it moves with respiration. • Rarely, omental cyst can present as a tensely cystic mass in the epigastrium. 111. RETROPERITONEAL MASS A. Pseudopancreatic cyst (Fig. 37.36): Jt fonns a tense cystic mass, felt as finn mass in the epigastrium. Its upper border can be made out. It does not usually move with respiration. It has smooth surface and round borders. History of acute pancreatitis or blunt injury abdomen is usually present. Pulsations over the mass (transmitted) suggest that it is a mass close to the aorta. In such a case, it is a pseudocyst. Gurgle heard anteriorly suggests distended stomach. B. Cystadenoma: Cystadenomas of pancreas are benign and can attain huge sizes. It can present as a mass in the epigastrium, left hypochondrium or umbilical region. They exhibit what is described as 'tree top mobility'. C. Carcinoma body of pancreas can present as a mass in the lower part of epigastrium or upper umbilical region. The mass is hard, irregular, fixed and does not move with respiration. Presence of severe backache and loss of weight are important features. D. Abdominal aortic aneurysm (AAA) (Fig. 37.37): An elderly patient, usually a hypertensive presents with features of abdominal pain, swelling or features of ischaemia of the lower limb. On examination, tender swelling in the epigastrium with a characteristic expansile pulsation is present. Knee-elbow test will help differentiate it from transmitted pulsations. Presence of a bruit and weak or absent lower limb pulses (due to thrombus) also helps in establishing the diagnosis. E. Lymph node mass (Fig. 37.35)

•

Fig. 37.32: Hydatid cyst

•

Fig. 37.33: Simple cyst

Abdominal Mass

+ +

•

•

Fig. 37.36: Pseudocyst of pancreas: Large enough- in contact with diaphragm

Fig. 37.37: Abdominal aortic aneurysm: Pulsatile firm mass

•

•

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+ +

Fig. 37.34: Tuberculous abdomen-ascites and rolled up omentum

Fig. 37.35: Lymph nodes nodular, firm to hard fixed

MASS IN THE RIGHT HYPOCHONDRIUM (Fig. 37.39)

I. Parietal: On head raising test, the lump becomes more prominent. A. Lipoma, neurofibroma: They can be part of multiple lipomatosis or multiple neurofibromatosis. If pain and pigmentation are present, it is neurofibroma. B. A hard nodule in the parietal wall can be due to a. Secondary deposit in the skin/subcutaneous tissue specially when skin is infiltrated and ulcerated. Common primaries are malignant melanoma, bronchogenic carcinoma, hepatoma. b. Non-Hodgkin's lymphoma: 'T' cell type c. Cold abscess: Spine tenderness with or without history of tuberculosis gives the clue to the diagnosis. II. Intra-abdominal swellings: On head raising test, the lump becomes less prominent. 1. Liver: Only chronic masses are discussed A. Secondaries in liver (Fig. 37.38 and Key Box 37.6) • Entire liver is enlarged (both lobes) • Nodular surface • Sharp border • Hard in consistency • Rare umbilication sign, evidence of primary, emaciated patient, poor health, loss of appetite and weight are other features. B. Hepatoma (Key Box 37.7) • One lobe is enlarged • Finn to hard, irregular • Very tender liver • Bruit/thrill may be present • Evidence of chronic liver disease such as serum hepatitis or cirrhosis is usually present. C. Polycystic disease of the liver • Both lobes are enlarged • Nodular

Fig. 37.38: Large secondary in the liver from carcinoma stomach

............................. . KEY BOX 37.6

ANATOMIC FEATURES OF LIVER MASS • Location: Hypochondrium (right and left) and epigastrium • Moves with respiration • Finger-insinuation between costal margin and mass is not possible • No intrinsic mobility and dullness • Dull note over the liver which will continue with the mass

............................. . KEY BOX 37.7

• • • • •

TENDER LIVER MASS Hepatoma Amoebic liver abscess Suppurative pylephlebitis Congestive cardiac failure Infected hydatid cyst

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Fig. 37.39: Differential diagnosis of mass in the right hypochondrium: (1) Smooth hepatomegaly in lymphoma or due to medical causes, (2) secondaries in the liver-hard and nodular, (3) hepatoma-irregular, hard or firm, (4) polycystic disease of the liver-firm and nodular with round borders, (5) with splenomegaly-could be portal hypertension, (6) hepatomegaly, splenomegaly, para-aortic lymph nodes and iliac nodes-Hodgkin's lymphoma, (7) carcinoma ascending colon-hard irregular mass, (8) palpable gall bladder­ smooth, round borders, and (9) renal mass

• Nontender • Round borders • General health is good The patient would have presented to the hospital with pain due to haemorrhage in a cyst. D. Hydatid cyst • One or both lobes are enlarged • Smooth or nodular surface • Round borders, nontender • General condition of the patient is good • Hydatid thrill-rare 'physical sign' may be present.

E. Cirrhosis of liver • Liver may be enlarged: Firm and irregular in pre-cirrhotic cases. Splenomegaly, ascites will help in the diagnosis. • Other features ofliver cell failure such as gynaecomastia, spider naevi, palmar erythema may be present. F. Lymphoma • Liver is palpable, one or two finger-breadths, firm or hard, smooth or irregular, nontender. • Splenomegaly and lymphadenopathy will help m the diagnosis.

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Abdominal Mass

G Congenital Riedel's lobe • It is a tongue-shaped projection from the inferior border of liver. It is on the right side, can be mistaken for gall bladder. 2. Gall bladder mass (Key Box 37.8) Causes of gall bladder enlargement • Back pressure: Distal obstruction periampullary carcinoma. Such gall bladder is firm, smooth and asso­ ciated with jaundice. • Carcinoma gall bladder: Hard, irregular, fixed • Acute cholecystitis: Tender, vague, well-defined mass. • Mucocoele: Nontender, palpable gall bladder without jaundice • Empyema: Very tender, gall bladder mass 3. Colonic mass A. Carcinoma hepatic flexure • Firm to hard irregular mass • Restricted mobility • Moves with respiration because of its contact with liver

............................. . KEY BOX 37.8

CLINICAL FEATURES OF A GALL BLADDER MASS a. It is oval, e.g. egg-shaped swelling b. It is tense. Hence, feels more firm in consistency c. Moves freely up and down with respiration-better seen in thin patients d. May have slight side-to-side mobility e. It is felt slightly posterior to (step deep) inferior border of the liver

• Resonant or impaired resonant note on percussion (liver is dull on percussion) • Caecum may be distended if there is obstruction. B. Large ileocaecal tuberculosis with pulled up caecum may also be palpable. Such masses may be bimanually palpable but not ballotable. 4. Renal mass • Importantly renal mass is palpable mainly in the lumbar region, loin and in the right hypochondrium. • Carcinoma kidney is hard and irregular • Upper border is usually not palpable-it is under cover of the 12th rib. • Hydronephrotic kidney will be finn and smooth 5. Suprarenal mass • Clinically they have all features of a renal mass • Hence, symptoms of the patient may have to be correlated. A few examples are given here: - Cushing's syndrome - Phaeochromocytoma P EA RLS OF

WI SDOM

Offer renal mass as diagnosis. When asked fo r differential diagnosis, mention suprarenal mass.

WHATISNEWIN THISCHAPTER?/ RECENTADVANCES

• All topics have been updated. • Mass in the right hypochondrium has been added. • New simple diagrams have been added.

Manipal Manual of Surgery

920

MULTIPLE CHOICE QUESTIONS 1. The most diagnostic sign of a renal mass is: A. Moves with respiration B. Upper pole cannot be felt C. It enlarges downwards D. Ballotability 2. Which one of the following lower horizontal lines divides abdomen into regions? A. Transpyloric line B. Transcolic line C. Transtubercular line D. Transanterior superior iliac spine line 3. Which of the following masses does not move with respiration? A.Kidney B. Hepatic flexure C. Tail of the pancreas D. Para-aortic lymph node mass 4. Notch is a diagnostic sign of which mass? B. Liver A. Spleen C. Kidney D. Adrenal gland 5. Which of the following does not have intrinsic mobility? A. Fibroadenoma breast B. Ovarian cyst C. Mesenteric cyst D. Multinodular goitre 6. The following are true for renal masses except: A. It is bimanually palpable B. It is ballotable C. It does not move with respiration D. Upper border is usually not felt

9. Following appendicectomy, after 2 months, if wood indurated mass develops in the right iliac fossa wit sinuses, what is the diagnosis? A. Tuberculosis B. Crohn's disease C. Amoeboma D. Actinomycosis 10. Acute intussusception mass has following feature except: A. Mass is tender B. Mass is felt in the umbilical region C. It is a sausage-shaped mass D. In the right iliac fossa caecum gurgles 11. Which of the following masses does not have cross· fluctuation? A. Iliopsoas abscess B. Plunging ranula C. Collar stud abscess D. Branchial cyst 12. Following are true for retroperitoneal sarcoma except; A. Conm1on in young patients B. Mass does not move with respiration C. It can attain a hard and large mass D. Free fluid is usually present in the abdomen 13. The diagnostic feature of mesenteric cyst is: A. It is present in the umbilical region B. It is dull to percuss C. It falls forward D. Moves at right angle to the direction of rnesentery

7. Blumer's shelf refers to: A. Rectouterine pouch B. Rectovesical pouch C. Rectosacral pouch D. Rectoprostatic pouch

14. Which of the following masses is nontender? A. Hepatoma B. Appendicular mass C. Carcinoma stomach D. Cholecystitis

8. Following tumours can occur in the abdominal wall except: A. Desmoid tumour B. Endometriosis C. Dermoid tumour D. Fibromatosis

15. Murphy's triad of symptoms include the following except: A.Pain B. Vomiting C. Fever D. Jaundice

ANSWERS D 11 D

2 C 12 D

3 D 13 D

4 A 14 C

5 D 15 D

6 C

7 B

8 C

9 B

10 A

Section

Ill

Urology 38. 39. 40. 41. 42. 43.

Investigations of the Urinary Tract Kidney and Ureter The Urinary Bladder and Urethra Prostate and Seminal Vesicles Penis, Testis and Scrotum Differential Diagnosis of Haematuria

38 Investigations of the Urinary Tract • • • • • •

• • • • • •

Urine Blood Intravenous urogram Retrograde pyelography Renal arteriography Cystourethrography

URINE EXAMINATION Specific gravity: lt varies from l .005 to 1.040 according to

the state of hydration of the patient. In chronic renal failure, the concentrating ability of the kidneys are lost and the specific gravity remains fixed at 1.010.

pH: The urinary pH nomrnlly ranges from 4.5 to 8. It varies depending on serum pH. Urine pH influences the type of stone formed, e.g. alkaline urine-infection by urea splitting organisms, infection stones are formed and in acidic urine, uric acid and cystine stones occur.

Nonnally, urine is devoid of blood, protein and sugar. Proteinuria: It is defined as excretion of protein > 150 mg/

day. Protein, especially albumin, appears in urine following exercise, glomerulonephritis and nephrotic syndrome. Sugar: It appears in urine in diabetes mellitus. Transient

postprandial glycosuria may be seen when serum glucose levels exceed the renal threshold.

Blood: This is seen as presence of red blood cells (RBCs) in urine. Presence of more than 3 RBCs/high power field is considered significant. Haematuria, haemoglobinuria and myoglobinuria give rise to red colour in urine.

The causes of haematuria may be glomerular or nonglomerular. Glomerular causes are nephritis, analgesic nephropathy, IgA nephropathy and connective tissue disorders. Nonglomerular causes are stones or tumours involving the

Urethrography Ultrasonography Computerised tomography Radioisotope scanning Endoscopy What is new?/Recent advances

urinary tract ( e.g. renal cell carcinoma, transitional cell carcinoma). More details are given in Chapter 43, page l 002. Ketones: Are seen in urine in diabetic ketoacidosis, starvation

and pregnancy.

White blood cells (WBCs): Presence ofWBCs in urine is called pyuria (>5 cells/hpf). The causes are urinary tract infection, stones, glomerulonephritis, foreign bodies, tuberculosis (sterile pyuria) and malignancies (sterile pyuria).

CASTS AND CRYSTALS Casts: Tamm-Horsfall protein is a mucoprotein from renal tubular cells. It forms nucleus of all casts. It entraps REC/ WBC/epithelial cells to form respective casts. Hyaline casts are without cells and are normal. Crystals: Different types of crystals are formed in acidic and

alkaline urine and act as precursors for stone formation. Calcium phosphate and struvite crystals form in alkaline urine. Calcium oxalate, uric acid and cystine crystals form in acidic unne. URINE CYTOLOGY Urine cytology refers to identification of malignant cells under the microscope. If positive, it indicates transitional cell carcinoma of bladder. Cytology is not useful in the detection of other types of carcinoma such as squamous cell or adeno­ carcinoma.

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Manipal Manual of Surgery

24-hour urinary studies T hese are indicated in metabolic evaluation of stone disease (identifies various abnormalities of electrolyte homeostasis) and in the evaluation of recurrent pyeloneplu·itis in children and diabetic nephropathy (degree of proteinuria). BLOOD TESTS • Prostatic surface antigen (PSA): A glycoprotein, it is elevated in prostatic diseases. Its estimation is used for screening of carcinoma prostate but is not specific for this condition. Normal levels 0-4 ng/ml. Carcinoma should be suspected if levels exceed 4 ng/ml (see page977). • Testicular tumour markers Alp hafetoprotein (AFP): Elevated in embryonal carcinoma, yolk sac tumour P-human chorionic gonadotropin (P-hCG): Very high levels in choriocarcinoma Lactate dehydrogenase (LOH): Also elevated in embryo­ nal carcinoma and seminoma. lt indicates bulk of the disease or tumour burden. It is helpful in diagnosis, staging, follow up during/after treatment and prognostication. • Sex hormones such as follicular stimulating hormone (FSH), testosterone and prolactin may also be measured. X-RAY KUB • Plain X-ray KUB is a baseline investigation in suspected cases of calculous disease. • It should be taken in supine position which should cover pubic symphysis and lower two ribs. • A fat free, low residue diet, dimol 2 tablets, 3 times daily for 2-3 days prior to the X-ray is required. • Stones appear as a white shadow (images are given in renal stones, see page936,937) IMAGING Today renal imaging (Fig. 38.1) is the most important part of investigating a case of urinary tract. T here are many investigations, important ones being given below: Iodinated contrasts Ionic: Diatrizoate, metrizoate, ioxaglate (more side-effects, cheaper). Nonionic: Iopamidol, iopromide (fewer side-effects, costly). INTRAVENOUS PYELOGRAPHY (IVP) AND INTRAVENOUS UROGRAM (IVU) (Figs 38.1 to 38.5) Aim 1. To study renal function 2. To detect any pathology of kidneys, ureters and bladder 3. To study any anatomical variations of the renal system.

Fig. 38.1: IVU 5 minutes picture

Procedure • A fat-free, nonresidual diet is given for 2 to 3 days prior to the procedure to avoid intestinal gas shadows. • Dimol 2 tablets, 3 times daily for 2-3 days prior to the procedure to expel the gas. • T he patient should not take oral fluids 6 hours before the procedure. • Radiological contrast dye: 45% sodium diatrizoate, 20-40 ml is injected through median cubital vein. Requirements before IVP l . Normal renal function is a prerequisite for IVU. Serum creatinine is reliable, not urea because of variations in urea levels depending on hydration. Normal value of serum creatinine is 0.5 to 2 mg%. 2. Plain X-ray kidney ureter bladder (KUB) region to look for a renal stone-90% of the renal stones are radio-opaque (only 10% of gall stones are radiopaque). • To distinguish between renal stones and gall stones on plain abdominal radiograph, in case of doubt, take lateral film, stones anterior to the vertebral column are gall stones and posterior to it are renal stones. Precautions while injecting the dye 1. The dye should be given very slowly 2. The dye should not extravasate 3. Tf bronchospasm occurs, in addition to inhale broncho­ dilator, hydrocortisone 100 mg and an antihistaminic should be administered IV. 4. In cases of urticaria and skin rashes, an antihistaminic must be given. Radiography 1. Early films taken after 2 or 5 minutes, demonstrate the kidney outline (nephrogram).

925

Investigations of the Urinary Tract

Fig. 38.2: IVU after 20 minutes

Fig. 38.3: Observe bowel gas, inadequate preparation

Fig. 38.4: IVU showing hydronephrosis

2. 5 minutes later, pelvicalyceal system is visualised (Fig. 38. l ). 3. 15-20 minutes later, ureter, bladder can be visualised (Fig. 38.2). 4. Post-voiding picture is taken to demonstrate any residual contrast in the urinaiy bladder. • Abdominal compression has to be applied to demonstrate pyelograms better. Contraindications for IVP 1. Idiosyncrasy to iodine: Test dose should be given before hand. 2. Renal failure: Kidneys fail to excrete the drug. 3. Multiple myeloma: The dye precipitates myeloma proteins, blocks the ureter and kidney, and causes anuria. 4. Hyperuricaemia: Uric acid crystals deposit in the renal tubules. 5. Sickle cell anaemia: Precipitates sickle cell crisis. 6. Dehydration. Uses of IVU l . Diagnosis of congenital abnormalities such as polycystic kidney, horseshoe kidney, single kidney, duplication of kidneys and ureters. 2. Diagnosis of hydronephrosis, hydroureter (Fig. 38.4). 3. Obstruction to pelviureteric junction, ureters, primary obstructed megaureter. 4. Diagnosis of renal, ureteric stones and bladder stones. 5. To diagnose renal tuberculosis, tumours. lntraoperative one-shot IV pyelogram In ureteral injuries, when delayed contrast images are not possible because of haemodynamic instability, an intra­ operative one-shot (2 mg/kg IV contrast material given IO min before flat plate abdominal X-ray) IV pyelogram (!VP) is recommended in patients with hypotension or a history of significant deceleration, despite absence of gross haematuria.

Fig. 38.5: IVU showing double ureter

RETROGRADE PVELOGRAPHV(RGP)OR RETROGRADE URETEROGRAPHV(RGU) Indications I . When the kidney is not visualised by IVU a. Gross hydronephrosis (Fig. 38.6) b. Very high blood urea 2. To selectively collect the urine sample from renal pelvis, e.g. renal tuberculosis. 3. History of allergy to intravenous contrast materials 4. Prior to ureteroscopy. Procedure • A cystoscopy is done first. • Ureteric orifices are identified and cannulated by a flexible, catheter which is introduced up to the pelvis of kidney and

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Manipal Manual of Surgery

MUI,...........,� 1. 2. 3. 4.

COMPLICATIONS Tubular necrosis of the kidney Paraplegia due to spasm of spinal arteries Haematoma Thromboembolism

Fig. 38.6: RGP: Retrocaval ureter with hydronephrosis

the dye is injected. X-rays are taken at 5 minutes, 15 minutes and 30 minutes. Uses 1. Anatomical evaluation of pelvicalyceal system. 2. Early diagnosis of renal tuberculosis. 3. Since the dye is injected directly into the pelvis, pelvi­ calyceal system can be identified better which helps in the diagnosis of early transitional cell carcinoma of kidney. Complications of RGP I. It is an invasive procedure and hence, urinary tract infection can occur. Prophylactic antibiotics are given before the procedure. 2. Rarely chances of perforation of the bladder or perforation of the ureters may occur. RENAL ARTERIOGRAPHY: ANGIOGRAPHY Technique The technique used now is digital subtraction angiography (DSA). There are two methods in which the test can be performed. 1. Retrograde arteriography using Seldinger technique. Selective renal angiography can be done by using a catheter over a guide wire passed into renal artery. 2. Translumbar aortography is done wherein aorta is punctured with a needle from behind, above the renal arteries at the level of 1st lumbar vertebra. Dose For aortography 30 ml of contrast (hypaque) and for selective renal angiography 6-8 ml is used. Uses (Key Box 38.1) 1. To demonstrate pathological anatomy of the renal artery when renal artery stenosis or aneurysm is suspected.

Fig. 38.7: Renal angiogram-aneurysm

2. In renal cell carcinoma, tumour vascularity and extension of the tumour into the renal vein can be diagnosed during the venous phase. 3. Bleeding from the kidney due to trauma, post-percutaneous nephrolithotomy (PCNL) bleeding or arteriovenous malformation. 4. Therapeutic application: • Transluminal angioplasty can be done by inflating the balloon in cases of renal artery stenosis. • Embolisation of bleeding vessels, aneurysms (Fig. 38.7). MICTURATING CYSTOURETHROGRAPHY {MCU) In this procedure, the dye is injected into the urinary bladder via an indwelling catheter and X-rays are taken when patient passes urine. Indications 1. 2. 3. 4.

In children, to demonstrate vesicoureteric reflux Posterior urethral valve Vesical trauma Vesicovaginal or vesicocolic fistula.

Procedure A catheter is passed into the urinary bladder in a child and the dye is injected. The catheter is removed and the child is screened for vesicoureteric reflux during voiding of urine (Fig. 38.8).

Investigations of the Urinary Tract

927

Fig. 38.8: MCU voiding phase: Observe the ureter due to reflux

Fig. 38.9: Ascending urethrogram and micturating cy sto­ urethrogram-stricture urethra

Films

it can cause oil embolism. Conray 280 is injected slowly into the urethra.

Filling phase, full bladder, voiding and postvoiding phase films are taken. Newer Direct or indirect radionuclide cystography wherein isotopes are used. It can pick up minute reflux also. Complications Due to the invasive nature of the procedure, urinary tract infection can occur. Hence, prophylactic antibiotics should be used.

UL TRASO NOGRAPHY(USG) (Fig. 38.10) • This is a noninvasive investigation, using ultrasonic waves (sound waves with frequency> 20,000 Hz). These waves cannot be heard by the human ear but are reflected or absorbed by tissues to various degrees which helps to diagnose different conditions. Ultrasound can be used through different approaches: • Transabdominal • Transrectal (Key Box 38.2) • Transvaginal (used mostly by gynaecologists).

ASCENDI NG URETHROGRAPHY(ASU) OR RETROGRADEURETHROGRAPHY(RUG) In the diagnosis of urethral stricture, to know the length of stricture, proximal dilatation or diverticulum, urethrography is used (Fig. 38.9). Indications • Evaluation of urethral injury • Investigation of urethral stricture Contraindication Urethral haemorrhage Precaution Barium and medium containing oil such as lipiodol should not be used because if there is a urethral mucosa! tear or breach,

Fig. 38.10: Ultrasound showing hydronephrosis

Manipal Manual of Surgery

928

1111u•1111111111111111111w� TRANSRECTALULTRASONOGRAPHYIN CARCINOMA PROSTATE

• Disruption of the architecture of echoes • Invasion of capsule • Biopsy-ultrasonography guided

Limitations of USG • Operator-dependent • Air precludes adequate imaging: Bowel gas may prevent satisfactory imaging of pancreas/kidneys. • Obesity: Poor visualisation. Uses of USG A. Fluid can be differentiated from solid tissue. Hence, cystic swellings can be made out. B. Stones can be diagnosed. Stones appear as hyperechoic lesions and postacoustic shadowing. C. Tn an enlarged kidney thickness of cortex, disruption of the architecture of echoes can be made out as in hydronephrosis. D. Residual urine in the bladder can be found out, which may be an indication of enlarged prostate. The volume of the prostate can be measured. Ultrasonography has become the investigation of choice to diagnose foetal hydronephrosis due to various reasons. This has got dual advantages. Firstly, the management of the disease causing hydronephrosis can be planned at the early stage thereby preventing damage to the kidney and secondly intra­ uterine interventions are also possible if the need arises. COMPUTERISED TOMOGRAPHY (CT) SCANNING • CT can be done with or without contrast (Fig. 38.11 ). • CT without contrast (plain CT) is the investigation of choice for evaluation of renal/ureteric colic. • CT scan can visualise most urinary tract calculi, even radiolucent stones not seen on plain X-ray KUB or IVU. • Contrast CT gives information about the function also­ similar to IVU. However, unlike IVU, which gives information only about the renal parenchyma and collecting system, CT can provide valuable information about perinephric events (e.g. urinoma, abscess, lymph nodes compressing ureters causing hydronephrosis). • CT angiography is replacing conventional angiography for diagnostic evaluation of renal vascular anatomy. • lt is more useful than arteriography to assess and display the images of the body at selected levels. • It is useful in the diagnosis of kidney tumour and its extent, spread and infiltration.

Fig. 38.11: CT scan showing normal kidneys: It gives information about function of kidney. Now more commonly used than IVU

• To stage cancer of prostate, bladder, kidney, testicular tumours and renal trauma (Figs 38.12 and 38.13). PEARLS OF

WISDOM

Presently, CT with contrast is fast replacing /VUfor evalua­ tion of urinary tract because of its obvious advantages.

RADIOISOTOPE SCANNING Gamma camera screening following injection of technetium 99m gives information about proximal tubular function. To assess differential renal functions, diethylene triamine penta­ acetic acid (99mTc DTPA) or dimercapto-succinic acid (99111Tc

Fig. 38.12: CT scan showing left renal cell carcinoma

Investigations of the Urinary Tract

929

posterior urethral valves, etc. This scan is also useful ir: assessing the improvement in relative function ofthe kidne) after surgery for above conditions. The yield of DTPA scan can be improved by injecting IV lasix. This is known as diuretic renography. This will unmask marginal pelviureteric junction (PUJ) obstruction (Fig. 38.14). mTc DMSA: Dimercapto-succinic acid It is used most commonly for cortical imaging. It shows details of renal parenchyma. It is patiicularly useful when looking for segmental abnormalities of kidney (e.g. renal scarring secondary to conditions such as chronic pyelonephritis and renal tumours-Fig. 38.15 and Key Box 38.3). 99

ENDOSCOPY • Cystourethroscopy: Bladder and urethral mucosa can be visualised • Procedure is done under surface anaesthesia • Preparation: The external genitalia is cleaned with soap solution or an antiseptic agent and 1 % lignocaine jelly is injected into urethra, to provide lubrication and anaesthesia. This should be left in place for 10 minutes for its action.

Fig. 38.13: CT scan showing bladder carcinoma

DMSA) is used. It is filtered and secreted into the tubular lumen. Two types are given below. mTc DTPA: Diethylene triamine penta-acetic acid This scan is done to find out relative functions ofboth kidneys; it also tells about the total GFR and what percentage of total GFR is contributed by each kidney. Relative function of45±2% is considered acceptable for each kidney. The main indication for DTPA is long-term hydronephrosis. Examples are newborn with antenatally diagnosed hydronephrosis, children with 99

Uses of cystoscopy I. Diagnosis of bladder cancer, papilloma, cystitis 2. Position and character ofureteric orifices-in tuberculosis involving the urinary bladder, the ureteric orifices are shifted upwards and gaping-golf hole ureter.

2

3

4

5

6

7

8

9

10

11

12

13

14

15

16

17

18

19

20

21

22

23

1-----· (.)

(LT kidney-BKG)

180 120

0

,,_______________________ --,

,.

60 0

Uptake interval

(RT kidney-BKG) I

I

0

2

4

6

8

10 12 14 Min

16

18

20 22

24

26

Function

Fig. 38.14: DTPA scan showing sluggish clearance of contrast in the right kidney. Left kidney is normal in clearance and function

930

Manipal Manual of Surgery

URETHROSCOPV It refers to visualisation of urethra by introducing cystoscope. Types of urethroscopy 1. Anterior urethroscopy is done in urethral stricture or in cases of chronic urethritis. It can rule out strictures due to granuloma. 2. Posterior urethroscopy: To visualise prostatic urethra and verumontanum • Verurnontanum is red in cystitis • In chronic prostatitis, prostatic ducts may be seen discharging pus • When lateral lobes of prostate gland are enlarged, they project into the internal meatus and produce an inverted 'V' appearance. Fig. 38.15: DMSA scan

IIIIHIIIIIIMIIIIIIIIIIIIIIW� RADIOISOTOPE SCANNING IN UROLOGY • 99mTc DTPA - Renal function, drainage - Renal parenchymal images • 99mrc DMSA - Bone secondaries • 99mTc Diphosphate • 131 1 - Phaeochromocytoma MIBG - Parathyroid adenoma • 99mTc Sestamibi

3. Indigocarmine test: 7 ml of 0.4% dye is injected intravenously. Observe ureteric orifice through cystoscope. Unilateral delay in appearance of dye suggests obstruction. If there is bilateral delay, it indicates impaired renal function. 4. As a preliminary step to do RGP. 5. To rule out involvement of bladder in gynaecological cancer (e.g. cancer cervix). 6. To remove bladder stones 7. For transurethral resection of bladder tumour (TURBT) in early bladder cancers.

MAGNETIC RESONANCE (MR) UROGRAPHV MRI of the genitourinary system is useful in many situations. It is more expensive compared to other investigations. A few uses have been given below. Uses of MR urography • For accurate evaluation of inferior vena cava (IV C) thrombus in renal cell carcinoma (RCC). • Extrinsic causes of ureteric obstruction causing hydronephrosis (e.g. retroperitoneal fibrosis, pelvic tumours) • MR urethrography for accurate delineation of urethral 1t1Junes. WHAT IS NEW IN THIS CHAPTER?/ RECENT ADVANCES

• All topics have been updated. • Urine examination and CT scan have been discussed in more details. • MR urography has been added.

Investigations of the Urinary Tract

931

MULTIPLE CHOICE QUESTIONS 1. When do you say there is significant haematuria? A. Presence of more than 2 RBCs/high power field B. Presence of more than 3 RBCs/high power field C. Presence of more than 1 RBC/high power field D. Presence of RBCs in the urine 2. Which one of the following can be detected by urine examination? A. Transitional cell carcinoma B. Squamous cell carcinoma C. Adenocarcinoma D. Adenosquamous cell carcinoma 3. When do you suspect carcinoma prostate? A. If patient has urgency of micturition B. Rectal examination reveal grade 2 enlargement of prostate C. If prostate specific antigen levels are more than 4 ng/ml D. If patient has recurrent urinary tract infection 4. Spider leg deformity in intravenous pyelography is a diagnostic sign of which disease? A. Hydronephrosis B. Polycystic kidney C. Horseshoe kidney D. Carcinoma kidney 5. Which of the following is a contraindication for intravenous pyelography (IVP)? A. Staghom calculi B. Tuberculosis of kidney C. Horseshoe kidney D. Multiple myeloma

6. The following are the advantages of retrogradf pyelography over intravenous pyelography (IVP) except: A. Selectively urine sample can be collected from renal pelvis B. Can be done when the kidney is not visualised by JVP C. Intravenous contrast need not be given D. It is an invasive procedure 7. Investigation of choice in foetal hydronephrosis is: B. MRI scan A. CT scan D. DTPA scan C. Ultrasound 8. Investigation of choice to know the renal parenchymal/ cortical function or damage is: A. Intravenous pyelography B. Contrast enhanced CT scan C. DTPA scan D. DMSA scan 9. Investigation of choice to know the renal function/ drainage is: A. CT scan B. MRI scan C. DTPA scan D. DMSA scan 10. Indigo carmine test is done to study: A. Ureteric obstruction B. Prostatic obstruction C. Urethral obstruction D. Renal obstruction

ANSWERS 1 B

2 A

3 C

4 B

5 D

6 D

7 C

8 D

9 C

10 A

39 Kidney and Ureter • • • • • •

• • • • • • •

Surgical anatomy Polycystic kidneys Horseshoe kidney Renal stones Ureteric stone Hydronephrosis

Renal tuberculosis Wilms' tumour Pyonephrosis Perinephric abscess Dialysis Renal transplantation What is new?/Recent advances

SURGICAL ANATOMY OF KIDNEY • Kidneys are the retroperitoneal organs, two in number on either side of vertebral column. Each kidney is bean or reniform in shape. Owing to the presence of liver, right kidney is 1-2 cm lower than the left kidney, extending from L l-L3 and the left kidney extends from T l2-L3 • These relationships are very important for surgeons, as the structures mentioned above may get injured during operations on kidney (Table 39.1). Also they may get directly involved by renal malignancies (local spread).

Fascial attachments Both the kidneys and associated adrenal glands are surrounded by varying degrees of perirenal or perinephric fat and these are enclosed loosely together by the perirenal fascia, commonly called Gerota's fascia.

Spleen

Fig. 39.1: Anterior relationships of the kidney

• Perinephric fat with renal pedicle are responsible for classical ballotability of renal swelling. • The anterior and posterior sheaths of Gerota's fascia, become fused on three sides around the kidney laterally, medially and superiorly.

Relations of kidneys Right

Left

Anterior (Fig. 39. l)

Below: Hepatic flexure of colon Medial: 2nd part of duodenum

Below: Splenic flexure, pancreas and splenic vessels, below the pancreas is jejunum, above the pancreas is stomach and spleen

Medial

Above: Adrenal, liver, IVC

Above: Adrenal, duodenojejunal flexure, inferior mesenteric vein, ureter

Lateral

Below: Ascending colon

Below: Descending colon

Above: Liver

Above: Spleen

Posterior

Same ia both kidneys. Each kidney rests upon four muscles: Psoas, transversus abdominis, quadratus lumborum, diaphragm

932

Kidney and Ureter

933

Superiorly Gerota's fascia fuses with the diaphragm (as a result ow which kidney moves with respiration). Students should remember here that movement with respiration is a characteristic feature of intraperitoneal organs and masses, and many times this feature is used to support the diagnosis of intraperitoneal organ. However, even though the kidney is a retroperitoneal organ, it also moves with respiration. Medially Gerota's fascia of one side crosses the midline and fuses with Gerota's fascia of the opposite side. Inferiorly Gerota's fascia remains an open potential space containing ureter and gonadal vessels. • Gerota's fascia forms an important anatomical barrier and tends to confine pathological processes originating from the kidney. • However, because of its deficiency inferiorly, a collection within Gerota's fascia may track down and extend into pelvis. Some points to remember Renal artery is an end artery and the entire renal arterial system is composed of end-arteries (without anastomosis and collateral circulation). Occlusion of any branches of renal artery (within kidney known as segmental arteries) results in infarction of area supplied by it. • In contrast, the renal parenchymal veins anastomose freely with each other and even with perinephric veins. POLYCYSTIC KIDNEYS (CONGENITAL CYSTIC KIDNEYS) • This is an autosomal dominant disease transmitted through chromosomes from anyone of the parents. Early onset con­ genital cystic kidneys are found to be autosomal recessive. • More common in women Types • ADPKD: Autosomal Dominant Polycystic Kidney Disease (Figs 39.2 and 39.3). • ARPKD: Autosomal Recessive Polycystic Kidney Disease. Associated lesions • Congenital polycystic disease of liver (18%). • Congenital polycystic disease of pancreas. • Congenital polycystic disease of ovary or testis. • Berry aneurysms

Fig. 39.2: Autosomal dominant polycystic kidney disease

Pathology • During development, some of the uriniferous tubules fail to join with the collecting ducts. Such uriniferous tubules develop into cysts. The important pathological features are as follows: • Both kidneys are affected (Fig. 39.2) • They enlarge often to 3-4 times the normal size. • Cysts are distributed evenly throughout cortex and medulla. - Content of cyst varies but it is not urine. • The kidneys are studded with multiple large cysts • When the cyst ruptures into the pelvis of the kidney, it results in haematuria. • As the disease progresses, cysts progress in size which lead to pressure atrophy of the functional renal parenchyma leading to renal failure. Types (for recessive) I. Foetal type: Autosomal recessive disease may cause intra­ uterine death due to obstructed labour. 2. InfantiJe type: Autosomal recessive, early renal failure with death within 3-5 years of life. 3. Adult type: Autosomal dominant type. Presents in the 3rd or 4th decade with symptoms (Fig. 39.3). Clinical features • Even though congenital, it manifests at middle age around 40 years.

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Fig. 39.3: Autosomal dominant polycystic kidney disease:

Cut section

• Dull aching pain in both loins is due to stretching of the renal capsule (dragging pain). • Microscopic or macroscopic haematuria in 70-80% cases. • Hypertension (secondary) (75%) is due to renal ischaemia which stimulates juxtaglomerular apparatus to secrete renin. It may also be related to a separate genetic factor. • Bilateral renal mass: Both kidneys are enlarged, surface is nodular or bosselated, firm to hard and some times cystic. • Features of renal failure: Thirst, vomiting, abdominal distension due to paralytic ileus, anuria, uraemic smell, coated tongue, anaemia. • Infection, pyelonephritis • Acute pain: If there is haemorrhage into or infection of a cyst. Colicky pain is due to blood clot in the ureter. Diagnosis 1. Serum urea and creatinine to rule out renal failure. Nonna! creatinine levels: 0.8-1.6 mg%. Normal urea: 20 to 40 mg%.

Flg.39.4: USG of autosomal dominant polycystic kidney

disease

Fig. 39.5: CT of autosomal dominant polycystic kidney disease

2. Plain X-ray KUB: Enlarged kidney can be seen because of changes in the density between the perirenal pad of fat and the kidney. 3. Abdominal USG/CT scan to confirm the diagnosis (Figs 39.4 and 39.5). 4. IVU: The spider leg deformity of the calyces. Treatment • Asymptomatic polycystic kidney does not require any treatment other than regular follow-up. • Polycystic kidney with hypertension: Control of hypertension with drugs. When hypertension becomes uncontrollable, bilateral nephrectomy followed by renal transplantation should be done.

My MS Exam Case, June 1986, Wenlock Hospital, KMC, Mangalore. A 45-year-old lady with mass abdomen was allotted as my long case. She had undergone open cholecystectomy 3 months back (ultrasound was not available at that time). She had a vague renal mass in the right loin. I was not sure. When I recorded BP it was 200/110 mm of Hg. I carefully palpated the opposite side where I was able to palpate a renal mass. The diagnosis became evident. It was polycystic kidney. In fact one of the examiners was the surgeon who had operated on this patient for open cholecystectomy. During that surgery, he discovered enlarged kidneys. The patient was asymptomatic.

Kidney and Ureter

• Infected cyst or if pyelonephritis develops: Appropriate antibiotics are given and if necessary, the cyst should be aspirated using ultrasound guidance. • Polycystic disease with renal failure, emergency dialysis followed by renal transplantation is the treatment of choice. The related donor should be screened for polycystic trait. HORSESHOE KIDNEY • During development, 2 mesonephric buds appear on the side of the future vertebral column and grows into metanephros. Mesonephric buds form ureter and metanephros kidneys. • If fusion occurs at lower pole, it results in a classical horseshoe kidney (Fig. 39.6). • Rarely, upper polar fusion can occur giving rise to reverse horseshoe kidney. • Inferior mesenteric artery crosses the isthmus at the level of L3-L4. Hence, horseshoe kidney cannot ascend. It is felt lower down in the abdomen. Clinical features • It can be asymptomatic for many years. • A palpable mass below and to the right and to the left of umbilicus or umbilical region can be a horseshoe kidney. • Recurrent urinary tract infection (UTI) is common because the ureters are angulated over the kidney isthmus. • They are more prone for hydronephrosis due to the angulation of ureters.

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• Rovsing's sign: Hyperextension of the spine results in abdominal pain, nausea or vomiting due to stretching of the capsule. Diagnosis 1. Ultrasonography (USG), to locate the kidney. 2. IVU: Upper and middle calyx are directed laterally but lower calyx is directed medially where there is fusion, which is characteristic of horseshoe kidney. 3. CT scan or isotope renogram are confirmatory. Treatment • Indicated only when there are complications. • Removal of the stone, or repair and reconstruction of the hydronephrosis are done in the usual manner. RENAL STONES Aetiopathogenesis 1. Infection: Organisms such as Proteus, Pseudomonas, Klebsiella produce recurrent UTI. These organisms produce urea, cause stasis of urine and precipitate stone formation. Nucleus of the stone may harbour these bacteriae (Fig. 39. 7). 2. Hot climates cause increase in concentration of solutes, resulting in precipitation of calcium and formation of calcium oxalate stones. 3. Dietary factors • Diet rich in red meat, fish, eggs can give rise to aciduria. • Diet rich in calcium-tomatoes, milk, spinach, rhubarb produce calcium oxalate stones. • Diet lacking in vitamin A causes desquamation of renal epithelium which precipitates calcium, alters it and fonns stones. Epitaxy: Growth of one type of stone over another type.

Fig. 39.6: Horseshoe kidney

.............. ASSOCIATED ANOMALIES

• • • •

Spina bifida Congenital hemivertebra Turner's syndrome Cleft lip and cleft palate

�

Fig. 39.7: Chronic pyelonephritis with calculi ( Courtesy: Prof Sasidharan, Head, Dept of Urology (2002-2008), KMC, Manipal}

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Manipal Manual of Surgery

4. Metabolic causes • Hyperparathyroidism increases serum calcium levels resulting in hypercalcinosis and pelvic stones. • Gout increases uric acid levels and causes multiple uric acid stones. 5. Immobilisation: Paraplegic patients secrete large amounts of calcium in the urine resulting in calcium oxalate stones (they pass skeletons in urine). 6. Decreased urinary citrate: Citric acid (300-900 mg/24 hours) keeps the urinary pH low. When citric acid levels decrease, it promotes precipitation of urinary calcium. Citrate excretion is under hormonal control. 7. Inadequate urinary drainage as in cases of horseshoe kidney, unascended kidneys are more vulnerable for development of stones due to stasis.

4. Cystine calculus • Cystinuria is an inborn error of metabolism which occue due to decreased resorption of cystine from the rena tubules. • Occurs in young girls at puberty • Increased excretion of cystine in urine results in cystim calculus. • Stones are hard and radio-opaque due to sulphur. Clinical features • Renal pain: Dull aching to pricking type of pain posteriorly in the renal angle formed by the sacrospinalis and 12th rib. Murphy's kidney punch test demonstrates tenderness at renal angle. The same pain may some times be felt anteriorly

8. Randall's plaques: Randall has suggested that initially a small erosion or an ulcer develops at the tip of renal papilla on which minute concretions or minor calcium particles get deposited and give rise to stone formation. Types of renal stones 1. Calcium oxalate stone • Called as mulberry calculi • Common type of stone • It is irregular having sharp projections • Oxalate stone is bard and single • Produces haematuria very early, resulting in deposition of blood over the stone giving a dark colour to the stone. • It occurs in infected urine (Fig. 39.8) • Contains alternate layer of calcium and bacterial vegetation. It is visualised in plain X-ray KUB.

Fig. 39.8: Oxalate stone

Fig. 39.9: Staghorn calculi

Fig. 39.10: Uric acid stone

2. Uric acid stone (Fig. 39.10) • Multiple, small, hexagonal, faceted, yellow coloured. • Contain calcium oxalate which makes them opaque. Pure uric acid stones are radiolucent. • Occur in acidic urine • Common in patients who consume red meat • Best responsive to lithotripsy 3. Phosphate stone (Figs 39.11 to 39.13) • Smooth, round • Consists of triple phosphate of calcium, magnesium and ammonium. • Dirty white to yellow in colour • Commonly occur in renal pelvis and tend to grow in alkaline urine. • As it enlarges in the pelvis, it grows within the major and minor calyces and slowly fom1s staghorn calculus. This calculus produces recurrent urinary tract infection and haematuria and slowly damages the renal parenchyma (Fig. 39.9).

Figs 39.11 to 39.13: Staghorn calculi and phosphate calculi

Kidney and Ureter

in the costal margin. Hence, it is described as costovertebral pain. Nausea and vomiting is due to intense sympathetic stimulation caused by stretching of renal capsule mediated by coeliac plexus. Ureteric colic: When the stone is impacted in the pelviureteric junction or anywhere in the ureter, it results in severe colicky pain originating at the loin and radiating to the groin, testicles, vulva and medial side of the thigh. This may be associated with strangury. The referred pain is due to irritation of the genitofemoral nerve. Haematuria is common with renal stone because the majority of stones are oxalate stones. T he quantity of blood lost is small but it is fresh blood (Key Box 39.2). Recurrent UTI: Fever with chills and rigors, burning micturition, pyuria may occur, along with increased frequency of micturition. Guarding and rigidity of the back and abdominal muscles during severe attack of pain.

•

• • •

937

Fig. 39.14: Plain X-ray KUB showing large stone in the pelvis

Complications 1. Calculous hydronephrosis occurs due to back pressure producing renal enlargement. Stretching of the renal capsule results in pain. In such cases, an associated palpable kidney mass suggests hydronephrosis. 2. Calculous pyonephrosis: Infected hydronephrosis where in the kidney is converted into a bag of pus. 3. Renal failure: Bilateral staghorn stones may not be symptomatic until they present with uraemia and renal failure. 4. Squamous cell carcinoma: Long-standing stones increase the risk of carcinoma. Investigations 1. Blood urea and creatinine to rule out renal failure. 2. Plain X-ray KUB (Figs 39.14 and 39.15) • To diagnose stones 90% of the renal stones are radio­ opaque. • Enlarged renal shadow can be seen. 3. USG • Presence of the stone can be diagnosis • Exact size and location of the stone can be evaluated.

··-� CAUSES OF HAEMATURIA: RENAL CONDITIONS

1. 2. 3. 4. 5. 6.

Polycystic disease of kidney Renal stone, ureteric stone Renal tuberculosis Carcinoma kidney Papilloma of kidney Renal infarction

Fig. 39.15: Plain X-ray KUB showing bilateral staghorn calculus

4. IVP • To locate the stone exactly in relation to kidney and ureter and to assess renal function. A nonradiopaque stone can be seen as a filling defect. Hydronephrosis and hydro­ nephroureterosis can also be seen. • Now noncontrast CT scan and contrast CT scan are used for more accurate detection of causes of abdominal colic. 5. Urine for culture and sensitivity. Treatment The treatment of renal stones can be divided into nonoperative treatment and operative treatment.

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I. Nonoperative treatment 1. Conservative: Small stones less than 5 mm in size pass off with intake of copious amount of fluids and at times forced diuresis. Intravenous hydration followed by intravenous frusemide may help pass the stones spontaneously. 2. Extracorporeal shock wave lithotripsy (ESWL): After cystoscopy, a ureteric stent (Double J stent) is placed into the ureter on the side of a large renal stone (Fig. 39.16). Shock waves are generated (around 500-1500 shock waves) which blast the stone. The stones get crushed and most of the stones will come out by the side of the stent (Key Box 39.3). Small stones can be removed without prior crushing. Steinstrasse • It means "stone street" • It is a condition which follows the use of ESWL. • Small pieces of fragmented calculi collect and obstruct in the distal ureter. Laser lithotripsy • Holmium-YAG laser (Ho-YAG) is the most effective with good margin of safety. • Wavelength 2100 nm • Ho-YAG laser lithotripsy occurs primarily through a photothermal mechanism that causes stone vaporisation. • They have the ability to fragment all types of stones regardless of the composition. ESWL is not effective in fragmenting cysteine stones because of its crystal lattice.

II. Operative treatment l . Endoscopic procedures 2. Open procedures 3. Special situations 1. Endoscopic procedures Percutaneous nephrolithotomy (PCNL): Retrograde pyelography (RGP) is done when the stone is located in the pelvis of the kidney. With a small I cm incision in the loin, the PCN needle is passed into the pelvis of the kidney and is confirmed by fluoroscopy. A guide wire is passed through the needle into the pelvis of the kidney. The needle is withdrawn, with the guide wire left within the pelvis. Dilators are passed over the guide wire and a working sheath is introduced into the pelvis. A nephroscope is passed into the pelvis and if the stone is small, it can be taken out. If it is big, it may have to be crushed using ultrasound probes and the fragments are removed. Ultrasound or pneumatic energy is used for fragmenting. Complications of PCNL • Injury to the colon, sepsis • Injury to the blood vessels • Urinary leak may persist for a few days.

Fig. 39.16: After clearance, DJ stent in place

2. Open surgical procedures Depending upon the location of the stone, various types of procedures are done. They are as follows: A. Pyelolithotomy: When there is extrarenal pelvis. B. Nephrolithotomy: When there is intrarenal pelvis, the stone has to be taken out through the kidney parenchyma. C. Extended pyelolithotomy: By retracting the kidney parenchyma laterally, the incision over the pelvis can be extended over to the calyx and the stone can be extracted from the calyx. Even a large staghom calculus can thus be removed. D. Pyelonephrolithotomy: Stone is extracted through an incision in the pelvis as well as the renal parenchyma. E. Partial nephrectomy: When the stone from a lowermost calyx is impacted, a lower pole neplu:ectomy can be done. F. Nephrectomy: When the kidney is destroyed by pyonephrosis, following obstruction by stone. 3. Special situations A. Bilateral renal stones: Kidney with better function has to be operated first. 1-2 weeks later, the opposite side can be operated. B. If there is pyonephrosis with a severe degree offever, pain and tenderness, nephrostomy is done and a tube drain is

..............

�

ESWL

Advantages • No incision • No pain

Disadvantages • Cost factor • Availability

Kidney and Ureter

placed in the pelvis of the kidney for drainage of pus and urine. Once the pus is cleared, fresh assessment of renal function is done. I f the kidney is nonfunctioning, nephrectomy is done. If the kidney is functioning ESWL/ PCNL/open procedure is done. This is known as percuta­ neous nephrostomy (PCN). PEARLS OF WISDOM

Open procedures for the management of stone disease have become obsolete and are found in old surgery text­ books. URETERIC STONE Stones come down from pelvis of the kidney and may get impacted at any site of anatomical narrowing of ureter, namely: 1. Pelviureteric junction 2. Crossing of the iliac artery 3. Crossing of the vas deferens or broad ligament. 4. Site of entry into the bladder wall 5. Ureteric orifice This may lead to hydroureteronephrosis, renal parenchymal atrophy, infection and pyonephrosis. Clinical features • Pain in the loin radiating to groin: Pain is severe, colicky, intolerable and lasts for a few hours. When stone descends into lower ureter, pain radiates to the testicles, labia majora and to the upper portion of thigh due to irritation of genitofemoral nerve. Colic lasts for about 4-6 hours and is relieved by antispasmodics, narcotics and NSAID. • An attack of haematuria or pyuria • Guarding and rigidity of the abdominal wall if present on the right side, is confused with acute appendicitis. Investigations Same as renal stone Treatment 1. Most of the ureteric stones pass via naturalis (urine). The patient is asked to consume a lot of water and antispasmodics. 2. Flushing therapy: About 2 L of IV fluid, with 20-40 mg inj frusemide (Lasix). It can be repeated for a few days. 3. Stone in the upper ureter: ESWL is the ideal treatment. Retrograde intrarenal surgery (RIRS) for ureteric, renal and calyceal stones. Flexible ureteroscopy with laser fulguration can also be done. 4. Middle ureteric stone: ESWL, ureteroscopy basketing or open surgery (ureterolithotomy).

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5. Lower ureteric stone: Ureteroscopic removal. With the usage of ureteroscope passed through the urethra, direct visualisation and manipulation of the stone even if it is impacted can be done. A laser lithotripter or ultrasonic lithotripter can be used to disintegrate the stone. 6. Vesicoureteric junction: Ureteroscopic removal or endoscopic meatotomy of vesicoureteric junction. For a stone impacted at ureterovesical junction, cystoscopy is performed. Ureteric orifice is identified and a cut is given at its mouth. Under fluoroscopic monitoring, the stone can be manipulated and basketed out using a dormia basket or other types of baskets available. 7. An impacted stone which is not amenable to ESWL, fluoroscopic or ureteroscopic manipulation have to be extracted by ureterolithotomy (open surgical method). Prevention of stone disease I. Metabolic work up of urine and blood for identifying metabolic causes. Example: Hyperparathyroidism to be ruled out by 24 hours urine analysis for calcium, phosphate and uric acid levels. 2. Fluid management: 1.5 L/day 3. Dietary adjustments: Red meat to be avoided (rich in uric acid). 4. Drug treatment • Xyloric, sodium bicarbonate: Uric acid stones Potassium citrate: Calcium stones • Thiazides small dose: Calcium stones • D-penicillamine: Cystine stones • Protease inhibitors: Infection stones against E.coli 5. Ultrasound to be done once in 6 months. HYDRONEPHROSIS Definition Aseptic dilatation of the whole or part of the pelvicalyceal system of the kidney due to partial or intermittent interruption to the outflow of urine. Causes of unilateral hydronephrosis

I. lntraluminal (within the lumen) • Stones and papillary necrosis in diabetes mellitus, analgesic nephropathy. II. Intramural (in the wall) 1. Congenital A. Pelviureteric junction (PUJ) abnormality (PUJ dyskinesia or achalasia of PUJ) is a congenital lesion where hydronephrosis occurs due to failure of transmission of neuromuscular impulses through the narrow PUJ. It can

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Manipal Manual of Surgery

also be bilateral. Male : Female ratio is 2 : 1. More common in males on the left side. B. Ureterocoele, congenital narrow ureteric orifice. 2. Acquired A. Carcinoma of the ureter or carcinoma of the bladder involving the ureteric orifice. B. Stricture of the ureter secondary to stone: After dislodge­ ment of the stone there can be inflammatory stricture of the ureter. C. Tuberculosis of the ureter and bladder. Ill. Extramural 1. Involvement of ureter by carcinoma cervix, rectum, bladder, the retroperitoneal tumours, primary or secondary deposits in lymph nodes. 2. Obstruction by aberrant vessels (Fig. 39.18) • Aberrant renal artery going to the lower pole of kidney can cause obstruction to the ureter causing hydronephrosis. 3. Retrocaval ureter 4. Horseshoe kidney Causes of bilateral hydronephrosis Lower urinary tract obstruction below the level of bladder neck will give rise to bilateral hydronephrosis (Ormond's disease). Any of the causes for unilateral hydronephrosis when present bilaterally cause bilateral hydronephrosis (Fig. 39.17).

�-- Aberrant renal artery

dyskinesia Ureteric stone Benign stricture�

Lymph node mass

Carcinoma ureter

Carcinomatous infiltration

Lower ureteric stone

•--- Ureterocoele

Bladder cancer--�

�----- Bladder neck contracture

BPH or----1-carcinoma prostate 1111-,�---- Posterior urethral valve Urethral----­ stricture Meatal stenosis-

---�o-9--- --- Phimosis

Fig. 39.17: Causes of hydronephrosis

Fig. 39.18: PUJ obstruction with aberrant crossing vessel

I. Causes in children I. Phimosis 2. Meatal stenosis 3. Posterior urethral valve 4. Bilateral vesicoureteric reflux II. In young adults I. Stricture urethra is commonly due to gonococcal urethritis. Iatrogenic strictures following instrumentation of urethra or following a rupture urethra later is becoming more common. 2. Bilateral aberrant vessels: Often a time, these may be the branches of renal artery and vein which cross the ureters. Ill. Causes in the middle age and above 1. Benign prostatic hypertrophy (BPH) • Common cause in middle age 2. Contracture of bladder neck 3. Idiopathic retroperitoneal fibrosis (Onnond's disease). IV. Physiological: Pregnancy Due to growing foetus and partly due to the hormone progesterone. Pathogenesis The back pressure effect depends upon the type of the pelvis (Figs 39.19 and 39.20). I. In patients with intrarenal pelvis the kidney gets damaged very early. As the time goes on, the urine gets diluted. All the salts are absorbed and is replaced by a watery type of fluid having a specific gravity of 10 I 0. 2. Patients with extrarenal pelvis have minimal damage to the renal parenchyma for a long time. 3. Even if there is complete obstruction to the urinary flow in a hydronephrotic kidney, some amount of urine is secreted, some of it is absorbed by the renal pelvis and the collecting

Kidney and Ureter

Fig. 39.19: Kidney with intrarenal

pelvis

Fig. 39.20: Kidney with extra-

renal pelvis

tubule and some by the lymphatics of the interstitial tissue of the kidney. The urine is thought to enter the interstitial space of the kidney from the pelvis through microscopic discontinuity (pyelo-sinus backflow-break) in the covering epithelium. • If the disease progresses further, it leads to a nonfunctional kidney. • If the disease is bilateral, it may give rise to uraemia. Clinical features 1. Painless enlargement of the kidney. A renal mass is felt in the loin with a smooth surface and firm in consistency (tensely cystic). 2. A dull-aching pain in the loin 3. Previous history of calculus disease 4. Hypertension and haematuria are rarely seen in hydro­ nephrosis. 5. Dietl's crisis: • It is intermittent hydronephrosis • This is common in calculous hydronephrosis. • Following an attack of renal colic, ureteric obstruction occurs due to stone which results in enlargement of the pelvis ofthe kidney resulting in a palpable mass in the loin. After a few hours, the mass disappears due to the passage ofa large quantity ofurine due to reflux polyuria or due to slipping of the stone. 6. The symptoms of primary cause may be evident in the history, e.g. colicky radiating abdominal pain due to stones and haernaturia.

Fig. 39.21: Right hydronephrosis-CT picture. Left kidney

normal

2. USG can detect enlarged kidneys and can detect the cause for hydronephrosis in majority of cases. 3. CT scan is the investigation ofchoice. It can assess anatomy and function more accurately than IVU (Fig. 39.21 ). 4. Intravenous pyelography (IVP) (Fig. 39.22) • Normally calyces are concave. • They become flat and later convex/club shaped followed by dilatation of pelvis and ureter depending upon the level of obstruction. • In hydronephrosis with gross impainnent ofrenal function, the dye may not be visible for a few hours in the X-ray. In such cases, large quantity of dye (100-200 ml) may have to be used and the pictures may have to be taken even after 24 hours. Such a situation is seen in PUJ dysfunction. 5. Isotope renography: Technetium 99m-labelled DTPA (diethylene triamine penta-acetic acid) scan using a Gamma camera. The above Gamma radiation emitter is injected intravenously and it can be detected to have been trapped in the urinary tract above the level of obstruction. It does not get washed off even after giving frusemide injection.

Investigations 1. Plain X-ray KUB • Enlarged renal outline can be made out • Demonstration of stone

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Fig. 39.22: Normal, flattened and club-shaped calyx

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Manipal Manual of Surgery

6. Retrograde pyelography (RGP) • When the IVP fails to demonstrate the kidneys, RGP is a useful investigation. It can be done just prior to definitive surgery for confirmation of the site of obstruction. • Less quantity of dye is required and better configuration of calyces can be made out. 7. Blood urea, creatinine is estimated to rule out renal failure Treatment of hydronephrosis I. Hydronephrosis secondary to a cause Treatment of the cause has to be done. Examples: a. Stones: Pyelolithotomy, ureterolithotomy b. Stricture: Strictureplasty or excision and end-to-end anastomosis. c. Aberrant vessel: Transection of the ureter and anastomosis in front of the vessel. d. Phimosis: Circumcision e. Meatal stenosis: Meatoplasty f. Posterior urethral valve (PUV): Transurethral fulguration of the valve. g. Benign prostatic hypertrophy (BPH): Transurethral resection of the prostate (TURP). h. Carcinoma of prostate: TURP+ bilateral orchidectomy + hormonal therapy. i. Stricture urethra: Visual internal urethrotomy or urethro­ plasty. Principles of surgery 1. Nonfunctioning kidney with thinned out cortex and hydronephrosis/pyonephrosis-nephrectomy. 2. If the cortical thickness is adequate (0.5 cm) by ultra­ sonography, even though it is a nonfunctioning kidney, a preliminary nephrostomy to decompress the system has to be done. Reassessment of the renal function is done after a few days. If the renal function improves, definitive surgery for hydronephrosis can be done. If it remains a nonfunctioning kidney and the opposite kidney is nonnal, nephrectomy is done. 3. In B/L hydronephrosis, kidney which is functioning better should be operated first. II. Patients with congenital hydronephrosis­ pelviureteric junction (PUJ) dysfunction Congenital hydronephrosis needs special mention here. With increasing use of obstetric ultrasound, the incidence of antenatally detected foetal hydronephrosis is on the rise. In the present scenario, antenatal detection of foetal hydronephrosis has become the most common mode of presentation of congenital hydronephrosis.

Fig. 39.23: Gross hydronephrosis due to PUJ obstruction

Congenital hydronephrosis is defined as the antero­ posterior diameter of renal pelvis > 10 mm at > 20 weeks of gestation. PUJ obstruction is the main cause (Fig. 39.23). These foetuses undergo serial ultrasound monitoring during the rest of pregnancy and based on the increase or decrease in the pelvic diameter during this period, postnatal management can be planned even before the child is born. Recently one study has graded antenatal hydronephrosis due to PUJ on the basis of pelvic diameter and proposed the management guidelines (Dr Vikas Jain, Professor Sasidharan et al-KMC, Manipal). INDICATIONS FOR SURGERY (Key Box 39.4)

MWMtl............� INDICATIONS FOR SURGERY

Pain Atrophy of kidney (damaged) Infection Nephrosis-hydronephrosis Remembered as PAIN

Grades of renal pelvic diameter and management I Mild 11-20 mm II Moderate 21-35 mm >35 mm III Severe • Grade I hydronephrosis can be managed conservatively by serial monitoring of pelvic diameter by ultrasound and renal functions by DTPA scan. This is known as conservative or nonoperative management. These kidneys improve over a period of time. • Grade II hydronephrosis: Majority (almost 80-90%) can be managed conservatively. However, close monitoring of

Kidney and Ureter

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3. Attempt at healing produces calcification-pseudocalculi in the parenchyma of kidney. 4. Tubercular hydronephrosis is very rare. It is due to tubercular stricture of the pelviureteric junction.

Figs 39.24A and B: Anderson-Hynes reduction pyeloplasty (A) Excision of the redundant pelvis along with PUJ, (B) Completed ureteropelvic anastomosis

the patient is required to detect any deterioration in renal function. Any deterioration in renal function is an indication for surgical intervention. In this group, 10-20% patients benefit from early surgery (patients with renal function of involved kidney< 40%). • Grade III hydronephrosis: All these patients should be operated early-Anderson-Hynes pyeloplasty to prevent permanent damage to kidney. Types of pyeloplasty 1. Anderson-Hynes pyeloplasty Principles 1. Excision of redundant pelvis 2. Disconnection of PUJ which is not functioning. 3. New ureteropelvic anastomosis is done in such a way that urine should drain by gravity. • This is the most popular type of dismembered pyeloplasty (Figs 39.24A and B). 2. Nondismembered pyeloplasty • They are Foley's Y-V plasty or flap pyeloplasty. The PUJ is not transected. However, they are not very popular. RENAL TUBERCULOSIS • This is secondary to pulmonary tuberculosis/lymphatic tuberculosis. The primary focus is often difficult to identify - Common in males, in the 20-40 age group. - Infection is always haematogenous. Often one may not find any active lesion in the lung or in the lymph nodes. - Usually unilateral. Pathology (Figs 39.25 to 39.33) 1. Tubercles develop and coalesce over the papilla which may ulcerate-ulcerative form. 2. The tubercles may caseate and rupture over the renal papilla and communicate with the pelvis. This variety is called as ulcerocavernous form.

5. The opening of one of the calyces may get fibrosed leading to hydrocalyx which may distort rest of the calyces. 6. Cortical abscess ruptures into the perinephric space and forms a tubercular perinephric abscess. This may even point at the loin and rupture forming a sinus in the loin. 7. Tubercular pyonephrosis (caseous kidney, putty kidney, cement kidney). When it gets calcified, it is called cement kidney. The entire kidney is converted into a bag of pus which is tubercular caseous material with or without secondary infection. The complete ureteric stricture due to tuberculosis cutting off the pelvicalyceal system may result in autonephrectomy because of fibrosis. 8. Small fibrosed, contracted functionless kidney. 9. As a part ofmiliary tuberculosis-multiple small tubercles may be seen in the renal parenchyma. Clinical features 1. Frequency is the earliest symptom of tuberculosis. It is due to renal tubular inflammation and later due to tubercular cystitis. 2. Abacterial acid pyuria: The urine is opalescent, pale or yellow, acidic in reaction and no organisms/bacteria are grown on repeated culture. PEARLS OF

WISDOM

Sterile pyuria is seen in tuberculosis, stones and in carcinoma in situ. 3. Haematuria is not uncommon. Usually it is a small quantity due to ulcerocavernous variety. 4. Evening rise of temperature. 5. Loss of appetite and loss of weight. 6. Evidence of pulmonary or lymph node tuberculosis (TB) may be present. Investigations 1. Urine for acid-fast bacilli (AFB) • Early morning sample of urine has to be examined which gives the highest concentration of AFB, for 3 days. • Ziehl-Neelsen stain and Gram staining • Lowenstein-Jensen media culture • Guinea pig inoculation is positive in 90% of cases. PEARLS OF

WISDOM

Newer easy methods like polymerised chain reaction (PCR) and radioisometric culture are also used.

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Manipal Manual of Surgery

A FEW PHOTOGRAPHS OF RENAL TUBERCULOSIS

Fig. 39.25: Ulcerative form

Fig. 39.28: Hydronephrosis

Fig. 39.31: Contracted kidney

Fig. 39.26: Ulcerocavernous form

Fig. 39.29: Tuberculous perinephric abscess

Fig. 39.32: Multiple tubercles­ miliary tuberculosis

Fig. 39.27: Pseudocalculi

Fig. 39.30: Tuberculous pyonephrosis

Fig. 39.33: Renal tuberculosis-entire kidney is a mass of caseous material

Kidney and Ureter

2. Cystoscopy A. Initially small ulcers are seen around ureteric orifice. They join together resulting in a big ulcer. • Due to extensive periureteric fibrosis, the ureter becomes thickened, sho1tened and straight. The ureteric openings are lifted upwards and they are gaping which means it does not contract/does not close when bladder contracts. Such contracted, elevated, permanently opened, lower end of ureter is called as golf hole ureter (Fig. 39.34). • As a result of this, with each act of contraction of bladder, there is reflux of urine into the kidney causing damage, B. When the disease involves the urinary bladder, it results in fibrosis. It becomes small and contracted with ineffective function. Storage capacity is lost resulting in intractable frequency, with a few drops of urine. There is bleeding. Micturition is painful and is called strangury. There is also severe pain in the suprapubic region which is referred to the tip of the penis. Such a small nonfunctioning urinary bladder is called as thimble bladder (Fig. 39.35). Treatment 1. Conservative line of management with antituberculous treatment is successful, provided kidneys are functioning as in early stages. 2. Nephroureterectomy is indicated if the kidney is nonfunctioning (Fig. 39.36). 3. Renal cavernotomy of Henley • Indicated when there is stricture of calyces which results in a hydrocalyx. • In this operation the stricture is divided so that the drainage becomes better. 4. Treatment of thimble bladder-ileocystoplasty. • In this 10-15 cm of ilea! loop is isolated based on the blood vessels, the fibrosed bladder dome is excised, intestine is split open and sutured to urinary bladder (Fig. 39.37). • This is to increase the capacity of bladder so as to store urine and reduce frequency.

945

Fig. 39.35: Genitourinary tuberculosis: Left kidney is normal. Right kidney shows hydroureteronephrosis. The urinary bladder is contracted-thimble bladder

RENAL NEOPLASMS Classification • Benign: Adenoma, cortical adenoma, papilloma arising from pelvis, haemangioma. • Malignant: Nephroblastoma, renal cell carcinoma • Transitional cell carcinoma (rare) • Squamous cell carcinoma (extremely rare)

Small-contracted ____,___ urinary bladder

Fig. 39.34: Thimble bladder with golf hole ureter (TB)

Fig. 39.36: Nephroureterectomy for renal tuberculosis ( Courtesy: Professor Sasidharan, Manipal)

Fig. 39.37: lleocystoplasty

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WILMS' TUMOUR (NEPHROBLASTOMA) • This is a malignant tumour of the kidney occurring in children. • The tumour is composed of epithelial and mesothelial elements. Thus, it may contain bone, cattilage, muscle, etc. Hence, it is called nephroblastoma (immature embryonic tissue). • The tumour arises in one of the poles, distorting the reniform shape of the kidney. It is greyish white or pinkish white in colour. At places, there may be areas of haemorrhage/necrosis. • Microscopic features include connective tissue elements cartilage, spindle cells, smooth striated muscle cells and epithelial elements. Clinical features • Common in female children, around 2-4 years. - Less than I year of age carries good prognosis - Upper limit of age is 7 years - Rarely it may occur in adolescents • The child is brought with abdominal distension, due to hugely enlarged kidney which on palpation feels nodular. • Rarely, Wilms' tumour can be bilateral • Haematuria is a bad prognostic symptom. It is an indication of rupture of tumour into the pelvis of kidney. Most of such children die by 2 years of age. • Low grade fever can occur in rapidly growing tumour due to tumour necrosis, which releases pyrogens. • Rapid deterioration of health is characteristic. Investigations 1. Abdominal USG can detect a solid tumour in the kidney. Ultrasound also rules out opposite kidney tumour. 2. CT scan to know extent of lesion and spread to the adjoining structures (Fig. 39.38). 3. IVP is done to study distortion of calyces and to evaluate the function of the opposite kidney. 4. FNAC is done to confirm the diagnosis preoperatively.

Fig. 39.38: A 3-year-old male child had presented with left loin mass. CECT abdomen and pelvis was done which showed a large tumour in the left kidney. Left r adical nephrectomy was done and the histopathology was reported as Wilms' tumour

Differential diagnosis 1. Neuroblastoma arises from adrenals. This is more common than nephroblastoma. 2. Retroperitoneal tumours 3. Adrenal tumour (Fig. 39.39)

Fig. 39.39: CECT abdomen and pelvis showing a large right heterodense adrenal tumour

Differentiating features between Wilms' tumour and neuroblastoma (NBL) 1. Calcification-foci of calcification seen in NBL (85%). Less common in Wilms' (15%). 2. Intraspinal extension-seen in NBL 3. Aorta and IVC invasion by Wilms' 4. Location Wi 1 ms' -intrarena 1 Neuroblastoma-seen above kidney pushing it downwards and outwards. 5. Crossing midline-neuroblastoma 6. Homovanillic acid (HVA) and Vanillylmandelic acid (VMA) increase in neuroblastoma. Spread 1. Direct infiltration of the capsule 2. Lymphatic spread occurs to the hilar lymph nodes, para­ aortic lymph nodes, mediastinal and left supraclavicular lymph nodes. 3. Haematogenous spread occurs to the lungs, liver, bones, brain, etc. The tumour thrombus can extend to the renal vein and inferior vena cava. Treatment The anaemia has to be corrected at the earliest. 1. For tumours confined to renal capsule or perirenal soft tissue not infiltrating the adjacent organs, radical nephrec­ tomy followed by chemotherapy with actinomycin D and vincristine are given for 6 months.

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2. For tumours which have gone beyond renal capsule and perirenal soft tissue, local infiltration to adjacent tissue, lymphatic metastasis, nephrectomy followed by local radio­ therapy and chemotherapy is given with actinomycin D and vincristine for 15 months. 3. If the tumour is found to be unresectable by CT scan or magnetic resonance imaging (MRI), preoperative FNAC to confirm diagnosis is indicated followed by preoperative radiotherapy ( l 000 cGy) or chemotherapy. Once the tumour regresses in size, nephrectomy has to be done. Postoperative chemotherapy is given with actinomycin D, vincristine and doxorubicin. 4. Bilateral Wilms' tumour: Radical nephrectomy on the side of the larger tumour and partial nephrectomy on side of the smaller tumour should be done. As much of renal tissue as possible should be preserved after leaving a tumour free margin. Postoperatively the patient has to be treated with chemotherapy. If the surgery is not feasible, only radiotherapy and chemotherapy has to be given. Growth disturbances, cardiac and pulmonary toxicities are complications of radiotherapy.

PATHOLOGY • Nearly all renal cancer in adults are adenocarcinoma • Cell of origin: Proximal renal tubular epithelium. • Starts in one of the poles, commonly in the upper polf and usually ruptures outside the capsule because of which reniform shape of kidney is maintained (Wilms' tumour grows within the capsule. Hence, the kidney shape is los1 very early) (Fig. 39.40A). • On the outer surface, it is homogenous (Wilms' tumour is pleomorphic) and yellow in colour due to position of lipids. • A few haemorrhagic areas are common because the tumour is very vascular (Fig. 39.40B). • Microscopy: Alternate clear cells and dark cells. • Various subtypes of RCC 1. Clear cell carcinoma • Most common type (70-80%) • It can be familial, associated with von Rippel-Lindau syndrome or sporadic (95%). 2. Papillary-both familial and sporadic forms.

RENAL CELL CARCINOMA (RCC) • It is also called Hypernephroma or Grawitz tumour. • Commonly found in the age group 40-60. • Male : female ratio 2 : l AETIOLOGY (Key Box 39.6) • Not known. Rarely familial RCC is known. Specific oncogenes have been identified. Smokers are twice as often affected than nonsmokers. • Leather workers are more prone for RCC. • Analgesic abusers are also more prone.

MHmtl...........�

Fig. 39.40A: Renal cell carcinoma upper pole

RISK FACTORS OF RCC • Diabetes mellitus • Chronic dialysis

.............. 1. 2. 3. 4.

RENAL CELL CARCINOMA: AETIOLOGY Chronic cystic disease Chromosomal defect Cadmium exposure Cigarette smoking

5. Coffee drinking 6. Congenital disease-von Hippel-Lindau disease Observe6 Cs

�

Fig. 39.408: Upper pole RCC-see the fleshy tumour with haemorrhage

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948

RENAL CELL CARCINOMA I CLINICAL PRESENTATION

TUMOUR Pain Haematuria Loin mass

I

BONY Fractures Secondaries Bony pain

I

HAEMATOLOGICAL Anaemia Polycythaemia Hyperglobulinaemia

I

ENDOCRINAL Hypertension Hypercalcaemia

Fig. 39.41: RCC showing clinical presentation

3. Chromophobe-these tumours exhibit multiple chromosome losses and extreme hypoploidy. 4. Renal medullary carcinoma • new subtype • associated with sickle cell trait • Tumour cells line the blood vessels which are responsible for early blood spread from renal cell carcinoma (like follicular carcinoma of thyroid-angioinvasion and capsular invasion). CLINICAL FEATURES (Fig. 39.41) I. Triad of renal cell carcinoma Triad is seen in only 9%, but if present, strongly indicates metastatic disease. 1. Pain: Dragging or intermittent clot colic due to blood clot blocking the ureter. 2. Intermittent haematuria 3. Palpable mass: Hard, nodular, ballotable and bimanually palpable, loin mass moving with respiration. II. Other manifestations 1. Pathological fractures, e.g. fracture femur, humerus vascular, pulsatile, secondaries are common in the flat bones, e.g. scalp, vertebra, rib, sternum because they contain red marrow for longer time. 2. Anaemia disproportionate to amount of haematuria is due to decreased production of erythropoietin. 3. Mild elevation of temperature is due to tumour necrosis producing pyrogens. It can present as pyrexia of unknown origin (PUO) and hence it is called Internist's tumour. 4. Nephrotic syndrome-like features are rare. 5. Endocrinal disturbances are rare (paraneoplastic syndromes). Increased ESR-most common paraneo­ plastic syndrome. • Renin producing tumours are responsible for hyper­ tension. • Polycythaemia is due to increased erythropoietin secretion.

• Other hormones produced by the tumour are parathormone, adrenocorticotrophic hormone, human chorionic gonadotropin, glucagon, prolactin. 6. Hypertension 7. Liver dysfunction: Nonmetastatic liver dysfunction also known as Stauffer's syndrome and this improves after nephrectomy. Robson's staging of renal cell carcinoma • Stage I: Tumour limited to kidney • Stage II: Tumour invades perinephric tissues or adrenal gland, but does not extend beyond Gerota's fascia.

liMlid@Ui Tx TO T1 T2 T3a T3b T3c T4

NO NX N1 N2 MO M1

RENAL CELL CARCINOMA

Primary tumour cannot be assessed No tumour < 7 cm, within capsule, i n greatest dimension > 7 cm, within capsule Extracapsular but within Gerota's fascia extension Into adrenal/perinephric Extension into renal vein/IVG Extension above diaphragm-direct invasion beyond Gerota's fasica No nodes Cannot be assessed Single regional lymph node More than one regional lymph node No metastasis Distant metastasis present

Stage grouping Stage I Stage II Stage Ill Stage IV

T1 NO MO T2 NO MO T1 N1 MO T2 N1 MO T3 N1 MO T4 any N, MO Any T, N2, N3, MO Any T, any N, M1

Kidney and Ureter

949

• Stage Ill : Tumour extends into major veins or lymph noda involvement • Stage IV: Tumour invades beyond Gerota's fascia or distan metastasis. INVESTIGATIONS

Fig. 39.42: Right RCC with left adrenal metastasis

1. Urine examination is done when the patient has haematuri, to look for malignant cells. 2. Plain X-ray KUB region: Enlarged kidney can be seen. 3. IVP: Distortion of calyces, missing of calyces or loss oJ architectural pattern of kidney. 4. USG: • Enlarged kidney • Locate tumour, site and extent • USG-guided FNAC can be done • Can detect thrombus in inferior vena cava (IVC) 5. Contrast enhanced CT scan is the investigation of choice for staging (Key Box 39.7 and Figs 39.42 to 39.46).

ll$NWW',� CT SCAN

• • • • •

�

Mixed density mass lesion Enhancement after contrast Secondary changes such as tumour cell necrosis Local extent can be evaluated IVG thrombus and lymph node involvement identified.

PEARLS OF

WISDOM

Any renal mass which enhances after contrast on a CT scan in an elderly person should be considered as RCC until proved otherwise.

Fig. 39.43: Chest X-ray showing cannon ball

6. Renal angiography is done by retrograde transfemoral approach. Features are as follows: • Neovascularisation: Tumour blush inside the tumour. • Venous phase has to be observed to rule out tumour extension in the vein.

Fig. 39.44: CT scan showing left RCC

Fig. 39.45: Very extensive left RCC

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Fig. 39.46: IVC thrombus

Fig. 39.48: Renal cell carcinoma with tumour thrombus removed

Fig. 39.47: Renal cell carcinoma with tumour thrombus in the renal vein, inferior vena cava

Fig. 39.49: Renal cell carcinoma-radical nephroureterectomy specimen

• Infradiaphragmatic tumour thrombus can be removed with proximal control over the vena cava. The supra­ diaphragmatic IVC thrombus requires cardiopulmonary bypass (Figs 39.48 and 39.49). • In the absence of distant metastasis after removal of the thrombus these patients survive for a long duration.

7. MRI scan: MRI scan is the investigation of choice to know the extent of IVC thrombosis (better than CT). 8. Venacavogram: It is done to know extent of tumour in the IVC and presence of collateral circulation.

TREATMENT 1. Radical nephrectomy • En bloc removal of entire Gerota's fascia with its contents, i.e. kidney, proximal ureter, adrenal gland. • Retroperitoneal lymph node dissection does not improve the survival rate. • Routine removal of ipsilateral adrenal gland is uncommon. Unless tumour invovles large portion of upper pole of kidney or there is suggestion of adrenal gland involvement on preoperative radiologic exams. 2. Radical nephrectomy with extraction of tumour thrombus • The tumour thrombus can extend along the renal vein into IVC and even into the right atrium (Fig. 39.47).

3. Nephron sparing surgery It is done when tumour length is less than 5 cm, in bilateral tumours and in cases of renal cell carcinoma in solitary kidney.

..............

�

• • • • •

TRANSITIONAL CELL CARCINOMA PELVIS Uncommon tumour arises in the pelvis Multiple sites of urothelial mucosa involvement often. Low grade tumours Discovered late Haematogenous spread is common.

Kidney and Ureter

4. Therapeutic embolisation • This can be used as a palliative measure in advanced carcinoma to relieve symptoms. This can also be used preoperatively to regress the size of the large tumour. • A catheter is placed in the renal artery and substances such as gel foam, blood clot, crushed muscle are injected. • They block the lumen of the vessel and reduce size of the tumour so that radical nephrectomy can be undertaken later. 5. Radiotherapy Not of much use. However, it is a good form of palliation for secondaries in the lung, bone and brain. 6. Immunotherapy Administration of interferon or interleukin-2 has been found to improve the survival rate. RENAL MASS IN SURGICAL WARD (Table 39.2) Clinical features of kidney mass • Moves with respiration because the fascia of Gerota encloses the kidney and fuses above with the diaphragm. • Kidneys enlarge in the upward and downward direction. • Bimanually palpable and ballotable. It is ballotable because of renal pedicle and perirenal pad of fat. • Colonic band of resonance is obliterated when the kidney enlarges, as the colon is pushed laterally. • Upper border is not palpable because it is under the 12th rib.

951

ACUTE SURGICAL INFECTIONS OF THE KIDNEYS PYONEPHROSIS In this condition, the entire kidney is converted into a sa, containing pus or purulent urine-almost always the rena parenchyma is destroyed totally. Causes 1. Renal calculous disease is the most common cause o­ pyonephrosis. 2. Acute pyelonephritis is more common in children and ir females. Inadequately treated cases may develop intc pyonephrosis, especially when pyelonephritis is associatec with urinary tract obstruction. 3. Infection of a hydronephrosis Clinical features • Anaemia and fever • Renal swelling • Large swelling with high grade fever with chills and rigon suggest an imminent danger of septicaemia and calls fo1 an immediate drainage of the pus. Investigations • Urine examination may be positive for coliforms and other gram-negative organisms. • Plain X-ray KUB may reveal a stone or an enlarged renal outline. • Ultrasound can confirm hydronephrosis.

Renal mass in surgical ward Hypernephroma

Hydronephrosis

Polycystic kidney

1. Chief symptoms

Haematuria, pain in the loin, renal mass

Asymptomatic, distension, abdomen pain

Mass abdomen, hypertension, haematuria

2. Age of the patient

Over 50 years

20-30 years

30-40 years

3. Sex incidence

Common in males

Common in females

Common in females

4. Anaemia

Present

Absent

May be present

5. Features of renal failure

Absent

Can be present in bilateral cases (rare)

May be present

6. Renal mass 7. Features of kidney mass

Unilateral, nodular, hard,

Can be bilateral, smooth, cystic,

Bilateral, bosselated, nodular, not

may be fixed, nontender

feels firm

fixed, nontender

May not have free mobility

Not fixed, nontender

Present

due to fixity

8. IVU

Irregular calyces

Gross dilatation of pelvicalyceal system

Spider-leg deformity of calyces

9. CT Scan

Enhancing mass

Dilated pelvicalyceal system with

Multiple hypodense areas without

uniform filling of contrast

enhancement

Pyeloplasty

Symptomatic-renal transplantation

10. Treatment

Radical nephrectomy

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Manipal Manual of Surgery

• Intravenous urogram demonstrates poor function of the kidney on the diseased side. As a rule the opposite kidney is normal.

• X-ray spine: Scoliosis with concavity towards abscess • Screening chest: Diaphragm is immobile and elevated or the diseased side.

Treatment • Broad-spectrum antibiotics (parenteral) should be started immediately once the urine and blood is sent for culture and sensitivity. • Ultrasound-guided aspiration of pus or a percutaneous nephrostomy (better), and drainage of pus greatly improves the general condition of the patient. • If any obstruction or causative agent such as a stone is found, it should be removed. • Nephrectomy should be considered if the kidney is non­ functioning with significant damage.

Treatment • Pus is drained by an incision in the loin, breaking all tht loculi. • Dialysis and renal transplantation are discussed ir page 1111.

PERINEPHRIC ABSCESS It refers to the collection of pus in the perirenal area. Causes • Infection in a perirenal haematoma • Pyonephrosis when it ruptures • Tubercular perinephric abscess • Pus from retrocaecal appendicitis can extend into loin, perinephric area and may present as abscess. Clinical features • High swinging temperature • Rigidity, tenderness, fullness in the loin • Oedema in the loin

MISCELLANEOUS INTERESTING 'MOST COMMON' FOR RENAL CELL CARCINOMA • Most common renal cancer in adults are adenocarcinomas. • Most common site is upper pole of the kidney. • Most common presentation of renal cell carcinoma is with mass abdomen. • Most common investigation of choice is contrast enhanced CT scan. • Most common method of spread is by haematogenous route. • Most common intra-abdominal malignancy which spreads within vena cava and into atrium is renal cell carcinoma. • Most common cell of origin is proximal renal tubular epithelium.

WHAT IS NEW IN THIS CHAPTER?/RECENT ADVANCES

Investigations • Total count: Raised above 20,000 cells/mm 3 • Urine analysis: No organisms are usually found

• All the topics have been updated. • New photographs and key boxes have been added.

MULTIPLE CHOICE QUESTIONS 1. Following is not the feature of adult polycystic kidney: A. It can give rise to renal failure B. Hypertension is seen in about 75% patients C. It is autosomal recessive D. It is always bilateral 2. Relationships of right kidney include following except: A. Posteriorly are muscles B. Anteriorly pyloric antrum C. Lateral ascending colon D. Medial adrenals

3. Which of the following is the feature of horseshoe kidney? A. Classically it is the upper polar fusion of both kidney B. It does not cause angulation of the ureter causing hydronephrosis C. It can be associated with Down's syndrome D. Hyperextension of the spine results in pain, nausea and vomiting 4. Gout results in: A. Calcium stones C. Phosophate stones

B. Cystine calculi D. Uric acid stones

Kidney and Ureter

5. Which type of stone typically causes haematuria? B. Phosphate stone A. Oxalate stone C. Cystine stone D. Uric acid stone 6. Following are true for calcium oxalate stone except: A. They are called mulberry calculi B. It is smooth and round C. It is hard D. It is visible in X-ray 7. About uric acid stone following is true except: A. It is common in those who consume red meat B. Small and multiple C. Also seen in gout D. Pure uric acid stone is radio-opaque 8. Staghorn calculi is a: A. Calcium oxalate stone B. Phosphate stone C. Uric acid stone D. Cystine calculus 9. Following are true for upper ureteric stone except: A. ESWL is the ideal treatment B. Percutaneous removal is an ideal treatment C. Ureteroscopic removal is an ideal treatment D. Cystoscopic removal is ideal 10. Patient has renal stones and sweJling in the neck, the diagnosis can be: A. Medullary carcinoma thyroid B. Hyperparathyroidism C. Hyperthyroidism D. von Recklinghausen's disease 11. Which of the following is the cause for bilateral hydronephrosis? A. Idiopathic retroperitoneal fibrosis B. Carcinoma ureter C. Tuberculosis of urinary tract D. Aberrant artery 12. Following are features of Dietle's crisis except: A. It is called intermittent hydronephrosis B. It is usually seen in calculus hydronephrosis C. Does not produce a mass D. It happens due to slippage of stone 13. Which is the investigation of choice in hydronephrosis? B. Intravenous pyelography A. Ultrasound C. CT scan D. Isotope renography

953

14. Definite indication of pyeloplasty include followin� except: A. Calculus hydronephrosis B. Grade III hydronephrosis C. Nonfunctioning kidney with cortical thicknesi 0.1 cm D. Tuberculous hydronephrosis 15. Following are true for renal tuberculosis except: A. It is primary tuberculosis B. Infection occur through lymphatic spread C. Commonly causes hydronephrosis D. It causes bacterial acid pyuria 16. Which of the following is an uncommon symptom ol renal tuberculosis? A. Haematuria B. Evening rise of temperature C. Cystitis D. Pyuria 17. Following are the result of urinary tuberculosis except: B. Golf hole ureter A. Thimble bladder C. Putty kidney D. Hydronephrosis 18. Following are true for Wilms' tumour except: A. Tumour contain only epithelial elements B. Common age group is less than 1 year of age C. Prognosis is poor if it occurs within l year D. Haematuria is a bad prognostic sign 19. About renal cell carcinoma all are true except: A. Majority in adults are adenocarcinoma B. Reniform shape is maintained C. Clear cells and dark cells are present in microscopy D. Tumour cells do not line the blood vessels 20. Polycythaemia in renal cell carcinoma is due to: A. Renin production B. Erythropoietin production C. Prolactin production D. Glucagon production 21. The diagnostic CT finding of a renal cell carcinoma in an elderly patient is: A. Dense mass lesion B. Tumour necrosis C. Lymph nodes D. Enhancing mass after intravenous contrast 22. Following are true for transitional cell carcinoma except: A. Low-grade tumours B. Haematogenous spread is common C. Arise from urothelium D. Multiple sites are not commonly involved

ANSWERS 1 C 11 A 21 D

2 B 12 C 22 D

3 D 13 C

4 D 14 B

5 A 15 D

6 B 16 A

7 D 17 D

8 B 18 D

9 A 19 D

10 B 20 B

40 The Urinary Bladder and Urethra • • • • • • • • •

Surgical anatomy Vesical calculus Carcinoma of bladder Ectopia vesicae Acute cystitis Diverticula Urinary fistulae Interstitial cystitis Schistosoma haematobium

• • • • • • • • •

Urinary diversion Rupture bladder Surgical anatomy of urethra Rupture urethra Stricture urethra Hypospadias Retention of urine Posterior urethral valve What is new?/Recent advances

• Distal urethral sphincter is a somatic striated muscle which is supplied by S2-S4 fibres via pudenda! nerves.

SURGICAL ANATOMY OF THE BLADDER Lining epithelium • Urinary bladder is lined by transitional epithelium that covers a connective tissue known as lamina propria. • Bladder cancers are transitional cell carcinomas. • However, due to changes in the epithelium caused by chronic irritation (stone), one can get other malignancies such as squamous cell carcinoma. • Bladder cancer can easily spread through lamina propria into the muscle coat (detrusor muscle).

Supports of the bladder • Posteriorly endopelvic fascia, which is continuous with lateral ligaments of the rectum, need to be divided during radical cystectomy. • Anteriorly puboprostatic ligaments also need to be divided during radical cystectomy.

Detrusor muscle It is a smooth muscle, fibres of which are intermingled. Hence, in cases of bladder neck obstruction, changes such as trabeculations/sacculations due to hypertrophy of this muscle is found. Trigone It is a triangular area lying between the internal urethral orifice and the orifices of the ureter. It is the most sensitive part of the bladder, irritation of which is mainly responsible for frequency of micturition (Fig. 40.1 ). Bladder neck • Internal sphincter is the smooth muscle which surrounds the bladder neck. It is innervated by a-adrenergic fibres and prevents retrograde ejaculation.

954

Fig. 40.1: Surgical anatomy of the urinary bladder

The Urinary Bladder and Urethra

• False ligaments: Urachus and obliterated hypogastric arteries together with the fold of peritoneum overlying these structures are called as false ligaments (median and lateral ligaments). Blood supply • Superior and inferior vesical arteries are derived from anterior trunk of internal iliac artery as the main source of arterial blood supply. Minor blood supply comes from obturator, inferior gluteal and in females from uterine and vaginal arteries also. • Veins form plexus on lateral and inferior surfaces of the bladder. Hence during suprapubic cystostomy, these structures have to be avoided while entering into the bladder. • Vesical plexus is continuous with prostatic plexus of veins in males which drain into the internal iliac vein. Lymphatics • Internal iliac nodes are the first level of lymph nodes. • Obturator and external iliac lymph nodes get involved later.

955

• Secondary: Stone develops in the presence of infectior and stasis due to obstruction to the urinary flow. The) develop secondary to bladder pathology. Types (Figs 40.2 and 40.3) 1. Oxalate stone: Moderate size, uneven surface, mulbeIT) stone is dark brown or black because of incorporation 01 blood pigment in it. 2. Uric acid stone: Round to oval, smooth, pale yellow, 1101 opaque to X-rays. They are primary stones. 3. Cystine: Radio-opaque due to high sulphur content. 4. Triple phosphate: These stones consist of ammonium, magnesium and calcium phosphates. They occur in urine infected with urea-splitting organisms. Sometimes, they grow rapidly. The nucleus of the stone can be made up of bacteria, desquamated epithelium or a foreign body. Dirty white in colour. Clinical features

• Primary: Stone which develops in sterile urine. They develop in the absence of bladder pathology. These also include renal stones which have migrated to the bladder.

• Frequency of micturition is the earliest symptom of bladder stone. It is due to cystitis. • Pain at the end of micturition referred to tip of penis in young boys suggests bladder stone. In school-going children, pain is aggravated by jumping and jolting. Pain is decreased on lying down because stone falls away from the trigone of the bladder. Typically, oxalate stones produce pain. Painful ineffective micturition is described as strangury. • Haematuria, is due to stone causing abrasions in the bladder mucosa. • Acute retention of urine due to the calculus obstructing the internal meatus. • Males are affected 8 times more frequently than females.

Fig. 40.2: Bladder stone-triple phosphate stone

Fig. 40.3: Bladder stone with copper-T (see clinical notes in page 970)

Innervation • Parasympathetic comes from anterior divisions of sacral nerves-S2,S3,S4 through inferior hypogastric plexus. Following excision of rectum, disturbance of micturition and sexual function can occur due to damage to the pelvic plexus. • Sympathetic supply comes from TIO to L2 segments. VESICAL CALCULUS

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956

Investigations • Urine: Red blood cells may be present-microscopic haematuria • Envelope-like crystals: Oxalate stone • Hexagonal plates: Cystine calculi • Radiography: In 90% of cases, the stone is visible. However, it is important to look for stones in the entire urinary tract. • Cystoscopy: Stone can be visualised. A click can be heard when the stone comes into contact with the instrument. Treatment • Ultrasound lithotripsy - very safe, but only for small stones. • Laser lithotripsy (Holmium laser)-can break most large stones. • Percutaneous suprapubic litholapaxy-using needle, guidance and metal dilators. I. Litholapaxy • By introducing a cystoscopic lithotrite, stone is grasped firmly and broken. Small fragments of stone are evacuated by using evacuator. Contraindications for litholapaxy 1. Urethra: Obstruction such as stricture, enlarged prostate. 2. Bladder: Cystitis, contracted bladder, carcinoma. II. Suprapubic cystolithotomy • Can be done when the stone is too big, too hard to crush or too soft. Transitional cell Ca - 90% Squamous cell Ca - 5-10% Adenocarcinoma - 2% CARCINOMA OF THE BLADDER Aetiology (Key Box 40.1) • Incidence is more in aniline dye workers. Products such as benzidine and 3-naphthylamine are carcinogenic. • Cigarette smoking • Chronic irritation by stones, catheter, also can produce carcinoma: 95% of the tumours originate in mucous membrane.

Mllffl,........� BLADDER CANCER

Aetiology • • • •

Bilharziasis Chronic irritation Chronic smoking Cyclophosphamide

High risk-occupations • • • •

Aniline dye workers Leather industry workers Paint industry workers Rubber industry workers

• Bilharziasis or schistosomiasis increases the chances oJ bladder cancer (squamous cell carcinoma). • Congenital anomalies associated with increased risk o1 carcinoma bladder a. Patent urachus b. Exostrophy bladder Pathology 1. Malignant villous tumours: They are transitional cell carcinomas. Multiple primaries are found in 25% of patients with bladder cancer. a. The villi are stunted, swollen and resemble cauliflower. They are slow growing. b. Can be sessile. Such tumours are high grade. c. Bladder wall is more vascular. d. Submucous lymphatic nodules appear around the growth. 2. Solid tumours are always malignant: They are sessile, lobulated. 3. Carcinomatous ulcer: It arises in leukoplakia. Histological types I. Transitional cell carcinoma-90% of tumours 2. Squamous cell carcinoma 3. Adenocarcinoma arises from urachal remnants and urethral glands 4. Mixed variety 5. Undifferentiated. Clinical features • In 90% of cases, initial symptom is painless, intermittent haematuria. • Severe cystitis like symptoms occur in carcinomatous ulcer. • Later painful, blood-stained micturition can occur. • Strangury: Painful micturition with bleeding and incomplete emptying of bladder. • Loin pain is due to ureteric obstruction with hydronephrosis. • Suprapubic pain, groin pain, perinea! pain are due to infiltration of nerves. This indicates advanced nature of the growth. Investigations I. Urine: Cytology of a 3-hour specimen. 2. IVP: Filling defect in bladder, dilatation of ureter can be found out (Fig. 40.4). 3. Ultrasound is a very useful investigation which can detect a bladder carcinoma (Figs 40. 7 and 40.8). It can also detect liver metastasis. 4. CT scan is the investigation of choice especially to know the spread of the disease (Figs 40.5, 40.6 and 40.9 to 40.11). • Specially it is useful to know the infiltration of the muscle, perivesical tissue and also the prostate and pelvic wall. 5. Cystoscopy: It is the gold standard investigation to locate the lesion and to take biopsy which is very much required for further management of the case (Key Box 40.2).

The Urinary Bladder and Urethra

Fig. 40.4: IVU showing filling defect

957

Fig. 40.5: CECT abdomen and pelvis showing a large tumour ir the left lateral wall of bladder with left hydroureteronephrosis

Fig. 40.6: CECT abdomen and pelvis showing a large tumour in the right lateral wall of bladder with right hydroureteronephrosis

Fig. 40.7: Ultrasound suggesting carcinoma urinary bladder

Fig. 40.8: Ultrasound suggesting carcinoma urinary bladder (another case)

Fig. 40.9: Plain CT of the bladder showing filling defect in the bladder

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Manipal Manual of Surgery

Fig. 40.1 O: Contrast CT of the bladder showing filling defect suggestive of carcinoma bladder

WIN,........,.� INDICATIONS FOR CYSTOSCOPY • Haematuria with normal IVP • Lower urinary tract symptom • Malignant cells in the urine cytology.

6. Bimanual palpation, rectoabdominally in males and vaginoabdominally in females is done under general anaesthesia. Thickening of bladder wall, mobility, fixity and hardness can be made out. Staging of bladder cancer 1. Clinical staging: Jewett, Strong and Marshall system

• Clinically the tumours are broadly classified into three groups: Superficial or noninvasive, infiltrating or invasive and carcinoma in situ. 2. TNM staging

iiMfiLHiiti

TNM staging of carcinoma bladder

Tis - Tumour in situ Ta - Tumour involving mucosa without invading the lamina propria T1 - Tumour involving mucosa, invading lamina propria and submucosa T2 - Tumour involving the muscle layer T3a - Tumour involving the muscle layer throughout the thickness T3b - Tumour extending to the perivesical fat or peritoneum and involving the adjacent organs T4 - Involvement of the rectum and prostate NO - No lymph nodes N1 - Lymph nodal metastasis No distant metastasis MO M 1 - Distant metastasis present

Fig. 40.11: Contrast CT of the bladder showing filling defects in two places suggestive of carcinoma bladder

Treatment of carcinoma urinary bladder I. Carcinoma not involving muscle layer (Tis Ta, T l ) a. Transurethral resection (TUR) of tumour (resected base to be screened for tumour by microscopy). b. Postoperative intravesical chemotherapy with Thiotepa/ Adriamycin/Mitomycin retained inside the bladder for 1 hour. Such 6-8 courses at weekly interval are given to reduce recurrence. c. BCG or interferon immunotherapy given postoperatively intravesically to prevent tumour recurrence. II. T2-T4 lesions: Radical cystectomy followed by systemic chemotherapy (MVAC: Methotrexate, Vinblastine, Adriamycin, Cisplatin) (vide infra). Radical cystectomy: Removal of the bladder with pericystic fat and the prostate and seminal vesicles, urethra in men and bladder and pericystic fat, cervix, uterus, anterior vaginal vault, urethra and ovaries in women. It is a major surgery with 3 to 8% mortality rate. III. Any T, N 1, MO or any T, NO, MI-Systemic chemotherapy (MVAC) followed by radiation therapy has to be given. IV. Small lesion involving muscle in the vault of bladder or posterolateral wall of the bladder, partial cystectomy (segmental resection) of that part of the bladder containing the growth with a wide margin of2-3 cm. This should be followed by intravesical chemotherapy. Role of radiotherapy I. Local: If lesion is not anaplastic, is 4 cm or less, after open diathermy excision, radiotherapy can be given. a. Implantation of radioactive Gold grains-198 Au b. Radioactive Tantalum wire-192Ta II. Deep X-ray therapy • Indication: Undifferentiated carcinoma • By using Cobalt 60 or linear accelerator.

959

The Urinary Bladder and Urethra

ECTOPIA VESICAE (EXOSTROPHY OF THE BLADDER) • A rare congenital anomaly seen in I: 50,000 births • Male: Female = 4 : 1 Aetiopathogenesis • T his occurs due to failure of development of lower abdominal wall and anterior wal1 of the urinary bladder. • As a result, the posterior bladder wall is seen protruding out below the umbilicus. Hence, it is exostrophy of the bladder. Types 1. Complete: Pubic symphysis is not formed, complete epispadias in male or bifid clitoris in female. 2. Incomplete: Pubic symphysis, penis or clitoris are normal. Clinical features (Key Box 40.3) • More common in male children • Posterior bladder wall is seen in the lower abdomen as a pink to red mucosa, partially inflamed. • Umbilicus is usually absent. • Penis is rudimentary and epispadias may be present. • Testis descends normally into a well developed scrotum. • Pubic symphysis is widely separated. It has the advantage in female patients in that it facilitates the delivery. • In female children-umbilicus is absent, external genitalia are poorly developed, and clitoris is bifid. • Constant dribbling of urine outside-therefore, they smell of urine. • Recurrent urinary tract infection (UTI).

Complications I. Renal failure due to recurrent UTI 2. Adenocarcinoma of bladder and in early age (Fig. 40.12) 3. Ammoniacal dermatitis of the skin.

............... • • • • •

ECTOPIA VESICAE

�

Penis is rudimentary Posterior bladder wall is seen in lower abdomen Pubic bone is widely separated Poorly developed external genitalia Poor health-UT!, renal failure, Ca bladder

Treatment l. Incomplete: Reconstruction of the anterior wall of the bladder with reconstruction of the bladder sphincter. 2. Complete: Total cystectomy followed by urinary diversion by implantation of ureters in the sigmoid colon (ureterosigmoidostomy) followed by reconstruction o1 anterior abdominal wall if the patient has urinary incontinence. ACUTE CYSTITIS Aetiology and pathogenesis Acute uncomplicated bacterial cystitis predominantly affects women. By definition these are infections occurring in the absence of any anatomic or functional abnormality of the urinary tract. The ascending faecal-perineal-urethral route is the primary source of infection. Men are somewhat protected from ascending infection because of long urethra and antibacterial properties of prostatic secretions. Causative organisms 80% of bladder infections in women are caused by E. coli followed by other gram-negative organisms such as Klebsiella and Proteus species. Clinical features Irritative voiding symptoms (frequency, urgency, dysuria) are the hallmarks of cystitis. Low backache and suprapubic pain are other complaints. Fever and other constitutional symptoms are usually present. Physical examination is frequently unremarkable except for suprapubic tenderness.

Fig. 40.12: Carcinoma bladder in a case of ectopia vesicae. ( Courtesy: Dr PS Aralikatti , Associate Professor, SIMS, Belgaum,

Karnataka)

Diagnosis Urinary microscopy is the mainstay of diagnosis. Diagnosis is strongly considered positive if microscopy shows > 5 WBCs/high power field in females and 2-3 WBCs/high power field in males. • Urine culture not only confirms the diagnosis but also identifies the causative organisms.

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Manipal Manual of Surgery

• Other tests and imaging studies are not indicated in an uncomplicated infection, unless patient presents with recuITent episodes.

PEARLS OF

Management • Antibiotic therapy based on the culture and sensitivity repott given for a period of 7-10 days, is curative. • Symptomatic treatment in the form of antipyretics, urinary analgesics and antispasmodics may help.

Investigations

DIVERTICULA OF THE BLADDER Types • Congenital (situated midline anterosuperiorly): Rare and usually symptomless. They represent the unobliterated vesical end of the urachus. May require excision if chronic infection persists. • Acquired: They are pulsion diverticula and occur due to bladder outflow obstruction. Pathology • The diverticulum is lined by bladder mucosa • The opening (mouth) is situated above and to the outer side of one ureteric orifice. Clinical features-most commonly in males (95%) >50 years of age • Symptoms of recurrent urinary infection: Suprapubic pain, frequency of micturition, fever with chills, etc. • Symptoms of lower urinary obstruction: Frequency, urgency, hesitancy, etc. • Symptoms of pyelonephritis: Backache, fever and renal angle tenderness, etc.

WISDOM

Presence of diverticula is not an indication for surgery

1. Cystoscopy: Full bladder distension is necessary to search

for diverticulum. 2. Intravenous urography: It can detect the site of dive1ticulum. lt can also detect hydronephrosis (Fig. 40.13). 3. Ultrasonogram • It can detect residual urine • It can detect diverticulum • It can detect associated stone Treatment Combined intravesical and extravesical diverticulectomy Complications (Key Box 40.4)

ll!M,I........,.� COMPLICATIONS

• Recurrent urinary infections • Bladder stone can occur and it may give rise to haematuria • Hydronephrosis and hydroureter occur due to peridiverticular inflammation and fibrosis • Neoplasm: Squamous metaplasia and leukoplakia URINARY FISTULAE Introduction Urinary fistulae are not an uncommon problem encountered by surgeons. Broadly classified into congenital and acquired. Congenital causes are a few of them (Key Box 40.5) which have been discussed in more detail in their respective chapters. More important ones are acquired fistulae which are given below. Among these vesicovaginal fistula is discussed in more detail. Acquired fistulae 1. Traumatic urinary fistula Perforating wounds, penetrating wounds or following surgery in the pelvis.

MH!l!,I........_.� CAUSES OF CONGENITAL URINARY FISTULAE

Fig. 40.13: Bladder diverticulum arising from the posterior wall. Ureter is seen entering the diverticulum

• Ectopia vesicae • Patent urachus • Association with imperforate anus

The Urinary Bladder and Urethra

2. Vesicovaginal fistula Causes

• Protracted or neglected labour • Gynaecological operations such as total hysterectomy and anterior colporrhaphy • Radiation causing avascular necrosis of the bladder • Carcinoma cervix infiltrating into the bladder. PEARLS OF

WISDOM

leakage due to necrosis oftissues manifests usually after 7 days. Clinical features • Leakage of urine from vagina • Excoriation of the vulva Diagnosis • Digital vaginal examination may reveal thickening on the anterior wall of vagina. • Vaginal speculum examination: Dribbling of urine into vagma. • Swab test: Methylene blue is injected into the urethra and ifvaginal swab is coloured blue, it is vesicovaginal fistula. Treatment

• Low fistula: Transvaginal repair • High fistula: Suprapubic approach and repair. 3. Fistula from renal pelvis to skin or gut

• Tuberculosis causes caseation and may result in fistula in the loin • Large staghorn calculi • Pyonephrosis • Crohn 's disease of the renal pelvis. INTERSTITIAL CYSTITIS

• Initial symptoms are increased frequency and pain is relieved by micturition and aggravated by overdistension of bladder. • First described by Guy Hunner (Gynaecologist) in 1914. • The characteristic linear bleeding ulcer is caused by splitting ofmucosa when the bladder is distended under anaesthesia. • It is also called Runner's ulcer. • It is common in western female patients. Many ofthem are psychiatric patients. • There is severe fibrosis of the urinary bladder due to pancystitis, resulting in a small thimble bladder. (In India, tuberculosis must be considered.) • Frequency ofmicturition and pain due to decreased bladder capacity are the features. lt causes sterile pyuria.

961

• Cystoscopy and biopsy will confirm the diagnosis • Treatment is difficult-hydrostatic dilatation, instillation ofdimethyl sulphoxide or surgical procedures such as ileo­ cystoplasty have been tried. SCHISTOSOMA HAEMATOBIUM

• Commonest cause of calcification in the bladder wall. • It is called as urinary bilharziasis. • The disease is caused by e mbryos (cercariae) of schistosoma, which enter the body through penetration of the skin and reach the bladder via the portal vein in a retrograde manner. In the bladder, ova are released which are excreted back into the fresh water via the urine. Fresh water snail is the intermediate host. • Multiple pseudotubercles, nodules, granulomas, fibrosis are the prominent pathological features. • Diagnosis is suspected by painless terminal haematuria, which lasts for 5 days (swimmer's itch) fever and eosinophilia. • Cystoscopy and biopsy to confirm the diagnosis. • Treated by long-term praziquantel and surgery may be required (ileocystoplasty). PEARLS OF

WISDOM

Urinary bilharziasis is a premalignant condition. URINARY DIVERSION

Patients with lower urinary tract cancers or severe functional or anatomic abnormalities of the urinary bladder may require urinary diversion. The most commonly used method of urinary diversion is by incorporating various intestinal segments into the urinary tract. Virtually every segment of the intestinal tract has been used. 1. lleal conduit: 18-20 cm of ileum is used as a conduit. Ureters are directly implanted into it. The end of the ilea! conduit is brought through lateral aspect ofrectus abdominis muscle and stoma is made. This simply acts as a conduit carrying urine from the renal pelvis or ureter to the skin, where urine is collected in an appliance attached to the skin surface. It is not a continent mechanism. 2. U reterosigmoidostomy is an example ofcontinent urinary reservoir, wherein ureters are anastomosed into the sigmoid colon. PEARLS OF

WISDOM

The most worrisome complication of this procedure is development ofadenocarcinoma, at the site where ureters are implanted.

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• Routine sigmoidoscopy is recommended annually, to be started after 5 years of procedure. • Newer method of continent diversion-orthotopic bladder substitution. 3. Nephrostomy: ft is required for drainage and decom­ pression of the upper urinary tract and is indicated in following situations: • Retrograde ureteral catheterisation is not advisable (e.g. in sepsis secondary to ureteral obstruction). • Retrograde ureteral catheterisation is impossible (e.g. complete ureteral obstruction by stone, tumour or stricture). • It is done by percutaneous approach. 4. Uretero-ureterostomy: It means anastomosis of ureter to ureter following resection. It can be on the same side, if the resection length of the ureter is 4 cm or less or to the opposite side ureter, if the ureter excised is more than 4 cm in length. Indications a. Trauma to ureter b. Ureteric involvement by neoplastic conditions, e.g. colonic carcinoma, which requires resection of ureter. • Mainly indicated for upper and midureteral involve­ ment. The procedure of choice for lower ureteric involvement is reimplantation into the bladder. RUPTURE OF THE URINARY BLADDER Causes of urinary bladder rupture 1. Surgical (iatrogenic): Bladder can be injured mostly during pelvic surgery, i.e. excision of the rectum or during gynaecological procedures, etc. 2. Trauma: Blunt injury abdomen due to road traffic accidents. • Kick or blow on abdomen, with a full bladder • Penetrating injury (very, very rare).

Types of rupture and clinical features I. lntraperitoneal rupture When there is surgical trauma or trauma to a distended bladder the rupture will be intraperitoneal (Fig. 40.14A). Clinical features • Sudden severe suprapubic pain, hypotension/syncope and shock. • Lower abdominal guarding and rigidity occur after a few hours of injury. • Distension • Even though the patient has not passed urine for a few hours there is no desire to micturate. • Shifting dullness may be elicitable. II. Extraperitoneal rupture (Fig. 40.14B) • Trauma either penetrating or blunt injury with fracture of pubis gives rise to this type of injury. • Difficult to distinguish clinically from an injury to the membranous urethra. Investigations 1. Plain X-ray abdomen: Lower abdomen shows ground glass appearance. 2. IVP: Extravasation of the dye into the peritoneal cavity or extraperitoneally. 3. Retrograde cystourethrogram: Confirms the site of leak. 4. However, CT cystogram is the investigation of choice today. Treatment 1. lntraperitoneal rupture: Laparotomy, repair of the bladder in two layers with vicryl. Drain the suprapubic space with a tube drain. An indwelling urethral catheter has to be placed for IO days to 2 weeks to keep the bladder decompressed. 2. Treatment of extraperitoneal rupture: Extraperitoneally expose the bladder with a suprapubic midline incision and repair of the bladder. Drainage, as mentioned above, has to be carried out. SURGICAL ANATOMY OF THE URETHRA (Fig. 40.15)

Fig. 40.14: Rupture bladder: (A) lntraperitoneal; (B) Extra­ peritoneal

• Male urethra is divided into anterior urethra (bulbop enile) and posterior urethra (prostatomembranous urethra). • Male urethra functions as a conduit for urine and semen. Anterior urethra is covered with erectile tissue of corpus spongiosum. Anterior urethra penetrates the urogenital diaphragm to enter the pelvic cavity as prostatomembranous urethra. • Since its margins are attached to the perinea! membrane it is vulnerable to tear at this point in pelvic bone fracture. • Length of male urethra is about 14-16 cm. • Entire urethra is supplied by internal pudenda! artery.

The Urinary Bladder and Urethra

• Veins drain into Santorini's plexus around the bladder neck and prostate. • Female urethra is short, drains only urine and is not vulnerable for injuries. • Narrowest part of male urethra is the external meatus. • Prostatic urethra has 2 sphincters at each end. Internal sphincter at the bladder neck is composed of smooth muscle fibres. The external sphincter is a rhabdosphincter, about 2 cm long, sun-ounding the membranous urethra. • Normally, urinary continence is maintained by the external sphincter. When the external sphincter is damaged (e.g. trauma, postsurgical) the internal sphincter maintains continence but to a lesser degree. • Continence is not affected by ablation of internal sphincter (e.g. post-transurethral resection of prostate (TURP) but it results in retrograde ejaculation, i.e. the semen goes back into the bladder instead of exiting through the urethra. RUPTURE URETHRA Types I. • • •

Rupture bulbar urethra The most common urethral injury Urethra angulates in the perineum, where it gets injured. Superficial extravasation of urine.

Clinical triad of rupture of bulbar urethra 1. Perinea] haematoma 2. Urethral haemorrhage: Blood at the urethral meatus 3. Distended bladder: Diagnosed by percussion over suprapubic region which gives dull note. Treatment • Advise the patient not to try to pass urine. • Urinary antibiotics • Should be shifted to the operation theatre and with aseptic precautions a catheter is passed gently, if it enters the bladder, it is kept in place for 2 weeks and perineal haematoma is drained. • If catheter does not reach the bladder, an incision is made in the perineum and the catheter is guided into the urethra. • If all the measures fail, emergency suprapubic cystostomy/catheter is done to drain the urine and later repair of urethra is undertaken. II. Rupture membranous urethra: Extraperitoneal rupture of the bladder • Always (70%) associated with fracture of the pelvis. • Occurs in major road traffic accidents. • There may be disruption of pelvic bones, fracture symphysis pubis, with avulsion of the puboprostatic ligament leading to floating prostate. • Deep extravasation of urine.

91

Types of rupture of membranous urethra I. Complete transection results in floating bladder. In t condition, urethra is completely transected at the apex the prostate. As the puboprostatic ligament is avulsed, 1 prostate falls back and migrates upwards. On rec examination prostate is felt as though it is floating Floating prostate (Vermooten's sign). 2. Incomplete transection 3. Associated with injury to bladder: Extraperitoneal ruptu of the bladder is seen here (intraperitoneal rupture ofbladd occurs in a distended bladder). Clinical features • History of injury • Features of shock due to significant blood loss (arour: 1-2 litres) • Haematuria • In cases of extraperitoneal rupture of the bladder, it wi not be palpable due to extravasation of urine into perineurr • Suprapubic tenderness and dullness • Rectal examination: Floating prostate can be felt whicl is tender. Investigations 1. X-ray pelvic bones may show a fracture or separation o; pubic symphysis. 2. Ascending urethrography (ASU) to confirm the rupture (Figs 40.16 and 40.17). 3. Once the stricture develops, voiding cystourethrogram (VCUG) is done to know the exact location of the stricture. Treatment (Figs 40.18 and 40.19) • Urgent blood transfusion to treat shock • Suprapubic cystostomy is done and degree of damage assessed. • A bougie/sound is passed from above and another similar sound is passed through the external meatus (penis). When the two meet, a click is appreciated. With both sounds in contact , the sound from bladder is withdrawn slowly. The lower one is advanced at this stage and 2nd sound appears in the bladder. A red rubber catheter is tied to it and the sound is withdrawn through external meatus. • To this red rubber catheter which is seen outside, a Foley catheter is tied and is drawn into the bladder and is kept in place for 15 days. This is called rail roading technique. • Associated injuries such as rupture bladder are treated by suturing. • Antibiotics are given

Manipal Manual of Surgery

964

MIMI,!............� COMPLICATIONS OF RUPTURE URETHRA

1. Extravasation of urine into scrotum, beneath superficial fascia of the penis, beneath Scarpa's fascia 2. Urethral stricture 3. Haematoma 4. Recurrent urinary tract infection

Bulbar urethra--+-+-+-�

Complications of rupture urethra Most dangerous complication is stricture urethra (Key Box 40.6)

Penile urethra

Fig. 40.15: Surgical anatomy of the urethra

Ruptured pubo-prostatic ligament

Fig. 40.16: Rupture of membranous urethra with floating prostate. Note the ruptured puboprostatic ligaments

STRICTURE URETHRA Causes (Figs 40.20 to 40.22) 1. Congenital-very rare 2. Post-inflammatory A. Post-gonococcal urethritis • Within 48 hours of exposure to the venereal disease gonorrhoea, there is involvement of periurethral glands. They are concentrated more in the bulbar urethra. Hence strictures are more in bulbar urethra. • It causes periurethral fibrosis, resulting in multiple dense strictures within I year of infection but may not cause difficulty in micturition for 10-15 years (Key Box 40.7). B. Tuberculosis 3. Post-instrumentation • Catheterisation • Dilatation • Transurethral procedures 4. Postoperative • Prostatectomy • Repair of rupture urethra 5. Rupture urethra 6. Schistosomiasis

................ ACUTE GONOCOCCAL URETHRITIS

�

• Pain during micturition • Burning micturition • Gleet: White flakes due to desquamated urethral epithelium in the early morning urine

Fig 40.17: Plain X-ray showing extravasation of the dye

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The Urinary Bladder and Urethra

SUSPECTED RUPTURE URETHRA

Patient has passed urine after trauma

Patient has not passed urine

No extravasation No rupture or partial rupture • No catheterisation

No blood at meatus

Blood at meatus

• Start the patient on antibiotics • Observe Try passing urethral catheter gently and without force

Catheter can not be passed Passes urine Discourage the patient to pass urine

[Observe]

Does not pass---+ Endoscopy and treat like stricture

DoASU

7 days ------� ---------1

(2-3 months)

Could be contusion

Patient is allowed to void

VCUG

VCUG

Demonstrates site of extravasation

Early repair (open)

No extravasation

Extensive extravasation

Minor Extravasation

Voiding normal without pain

Late repair

Observe

Foley's catheter for a few days

Fig. 40.18: Algorithm for management of bulbar urethral rupture (Courtesy: Dr Vikas Jain, Asst Professor, Department of Surgery, KMC, Manipal, 2007-2008)

Manipal Manual of Surgery

966

SUSPECTED INJURY OF POSTERIOR URETHRA P/R: High lying prostate Pelvic X-rays: • Pelvic fracture • Displacement of pubic bone Cystogram to confirm (MCU +ASU) Rupture urethra (may be associated with bladder rupture) CT cystogram

No bladder rupture

Intraperitoneal rupture with developing peritoneal signs

SPC

Extraperitoneal approach

(2-5 months later)

Laparotomy

VCUG +ASU

Repair bladder using absorbable sutures

Short segment stricture Introduce a SPC catheter and maintain for 3 months

Pelvic haematoma will subside and prostate/bladder will slowly course back to normal anatomical positions

Extraperitoneal rupture

Remove SPC (urethral injury alone heals spontaneously)

Repair bladder with absorbable sutures Long segment stricture

lntraoperatively do SPC

Urethroplasty

1--------------------------------'

VCUG Confirm the site of stricture Single stage repair of stricture of the defect at the site of ruptured urethra with direct suturing or anastomosis of bulbar urethra directly to the apex of prostate

Fig. 40.19: Algorithm for management of posterior urethral rupture ( Courtesy: Dr Vikas Jain, Asst Professor, Depar tment of Surgery, KMC, Manipal)

Fig. 40.20: ASU and VCUG showing stricture urethra

Fig. 40.21: ASU: Total cut off seen due to stricture urethra

Fig. 40.22: ASU with MCU showing stricture

The Urinary Bladder and Urethra

967

Clinical features Previous history of exposure to gonorrhoea, history of instrumentation or history of injury to urethra is usually present. • Common in young age (20--40 years). • History of straining while passing urine. • Suprapubic pain and swelling due to distended bladder. • Stricture urethra may be felt in the perineum as a button hole. It should be remembered that gonococcal urethritis is not common nowadays because of effective treatment of the disease. Treatment Usually cut at 12 o'clock position. 1. Visual internal urethrotomy (VIU) by using urethrotome 2. Open method is indicated in long strictures not responding to less invasive procedures. They are grouped under urethroplasty. a. Excision and end-to-end urethroplasty b. Reinforcement urethroplasty-buccal mucosa c. Substitution urethroplasty-buccal mucosa, skin d. Two step urethroplasty 3. Regular dilatation by using Lister's dilators Complications 1. Acute retention of urine either following alcohol or due to postponement of micturition. 2. Secondary stones due to stasis of urine proximally. 3. Recurrent periurethral abscesses (multiple) which rupture and open externally in the perinea! skin. When such a patient is asked to pass urine, urine can be seen coming out of multiple holes in the perineum (Watercan perineum). 4. Recurrent epididymo-orchitis. HYPOSPADIAS In this condition, some portion of distal urethra is not developed, as a result of which external meatus is situated in the under surface of penis. Usually this is associated with chordee and hooded prepuce. Types (Fig. 40.23) 1. Glandular variety • In this external meatus is situated few mm away from normal site within the glans. 2. Coronal variety • It occurs due to failure of development of urethra which runs in the glans penis. • As a result of this, urethra opens at the corona glands­ junction of glans and shaft of penis.

Fig. 40.23: Various types of hypospadias

• Both these varieties do not give major problems functionally. It can be left alone without treatment. 3. Penile hypospadias • In this the external opening is situated somewhere in the under surface of the penis. 4. Peno-scrotal/perinea! hypospadias • In this condition, the entire urethra is not developed • Penis is rudimentary. • Urethral opening is seen in between two halves of the scrotum and often it is split. • Cases may be associated with undescended testes • In such cases it is difficult to differentiate the sex of the child Clinical features • Occurs in I: 350 males. • Micturition: Stream is good, but it wets the clothes in 3rd and 4th varieties. • Chordee: Many of the cases are associated with bending of penis. • Sexual intercourse will be difficult. • Hooded prepuce. In severe hypospadias, possibility of intersex problem is settled by karyotyping. Treatment l. One-stage urethroplasty • Chordee correction: Always confirm by inducing artificial erection • Urethral tube formation by tubularising urethra • Inner prepuceal island tube urethroplasty. 2. Two-stage urethroplasty • When the child is 6-12 months old, chordee is corrected by straightening the penis. This is called orthoplasty.

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Manipal Manual of Surgery

• When the child is 5-6 years old, reconstruction of the urethra is done by using locally available skin either from the prepuce or from penile shaft. This is called urethroplasty. Hence, circumcision should not be done in hypospadias. DIFFERENTIAL DIAGNOSIS OF RETENTION OF URINE Causes of retention of urine I. Acute retention of urine A. In males • Benign prostatic hypertrophy (BPH): In elderly patients over 50 years of age. • Stricture urethra: In young patients • Postoperative retention of urine: Operations like haemorrhoidectomy, fistulectomy, etc. produces reflex spasm of internal sphincter, which precipitates retention of urine. Management of such cases is given in Key Box 40.8. B. In females • Hysteria • Retroverted gravid uterus • Urethral stenosis Residual urine Normal-30 ml >SO-abnormal/significant >200 in BPH requires surgery C. In children • Meatal stenosis due to meatal ulcer with scab (due to scratching habit of child). D. In general • Spinal anaesthesia • Spinal injuries • Blood clot in the bladder following prostatectomy • Bladder stone in school-going children: Pain referred to tip of penis • Acute urethritis and acute prostatitis due to bacterial infection • Faecal impaction in the rectum

MJMl,I...........� 1. 2. 3. 4. 5.

POSTOPERATIVE RETENTION OF URINE: TREATMENT

Hot water fomentation to the suprapubic region Provide privacy Run a tap nearby Make the patient to stand and pass urine Catheterisation should be done as the last resort

• Contracture of the bladder neck • Urethral calculus • Drugs: Atropine, carbachol, bethanechol. II. Chronic retention of urine (Key Box 40.9) 1. Benign prostatic hypertrophy 2. Bladder neck contracture 3. Stricture urethra

IIIM,I...........� • • • •

CHRONIC RETENTION OF URINE

BPH: Most common cause Painless Suprapubic dullness Slow decompression is recommended

POSTERIOR URETHRAL VALVE • They are congenital, symmetrical valves in the posterior urethra. • It is one of the common causes of vesicoureteric reflux and hydronephrosis in infants. • Bladder wall is thickened due to obstruction and hypertrophy. The urinary bladder is palpable, hard and felt in the suprapubic region-cricket ball bladder. • Due to stasis, recurrent urinary tract infection occurs commonly. • It is also one of the common causes of renal failure in infancy and childhood. Investigations • Ultrasound: Bilateral hydronephrosis, thickened bladder, etc. • Micturating cystourethrography: Dilated proximal urethra is highly suggestive of posterior urethral valve. It is a diagnostic investigation (Figs 40.24 to 40.27). Treatment • Cystoscopic posterior urethral valve fulguration • In very ill patients, initially vesicostomy is done to improve renal function and stabilise the patient. Fulguration is done after 1-2 weeks. • These patients should be monitored for renal failure by serial creatinine measurements. PEARLS OF

WISDOM

It can be associated with an orectal and vertebral malformations.

The Urinary Bladder and Urethra

Fig. 40.24: PUV with grade IV reflux

Fig. 40.25: MUC showing grade V vesicoureteric reflux

969

Fig. 40.26: Bilateral vesicoureteric reflux

• Pyelonephritis and scarring: Reflux of infected urine causes pyelonephritis. It can be acute or chronic. Recurrent episodes can lead to scarring of kidneys and decreased function, proteinuria and hypertension. Severe scarring bilaterally can lead to chronic renal failure (CRF) and end­ stage renal disease (ESRD).

Fig. 40.27: Micturating cystourethrogram showing bilateral grade V vesicoureteric reflux (VUR)

VESICOURETERIC REFLUX (VUR) Vesicoureteric reflux is the retrograde flow of urine from the bladder into the kidneys. Normally urine flows from the kidneys into the bladder and backward flow is prevented by a complex anatomy at the vesicoureteric junction, most importantly a good length of submucosal tunnel of ureter. Lack of adequate submucosal tunnel leads to VUR. The VUR can be of mild to severe grades (graded I -mild to V-severe). Clinical features • Child might be diagnosed to have hydronephrosis detected antenatally, depending on the severity of reflux. • Recurrent UT[: Due to increased amount of residual urine. When the child voids, some urine refluxes back into the kidneys, which comes back into bladder after voiding ceases. This will lead to a high post-void residue (PVR), causing recurrent infections.

Investigations • Urine analysis for proteinuria and infection. • Complete blood picture: Anaemia in CRF and leukocytosis in acute pyelonepbritis. • Renal function tests: Increased urea and creatinine in renal failure. • Ultrasonography (USG): Renal size, parenchyma and hydroureteronephrosis with PVR can be assessed. • Mic turating cystourethrogram (MCU) or voiding cystourethrogram (VCUG)-investigation of choice for diagnosis. Procedure: Bladder is filled with contrast after catheterisation and X-rays taken during filling and full bladder to look for reflux. After full bladder, catheter is removed, patient is asked to void and X-rays are taken in the voiding phase. • DMSA scan-for extent of renal scarring and function. Helps in choosing the management strategy (medical vs surgical). Treatment I. Conservative management: Low grade reflux (grades I-III) can be managed conservatively. The aims of conservative therapy include: • Prophylactic/suppressive antibiotics. • Regular or timed voiding and double voiding to keep PVR as low as possible. • To avoid constipation-reduces the incidence of UTI. • With these measures, most low grade reflux resolves spontaneously. • Higher grade reflux requires endoscopic or surgical management.

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Manipal Manual of Surgery

II. Endoscopic management: Injection of a bulking agent to provide support to VUJ, helps in a few cases. JU. Surgical management: Ureteric reimplantation is done for severe reflux. The principle of surgery is to lengthen the sub mu cosal tunnel. Tf one kidney is poorly functioning, nephrectomy can be done. In some cases, bilaterally scarred, nonfunctioning kid­ neys can lead to ESRD. These patients require renal transplan­ tation. MISCELLANEOUS INVESTIGATION OF CHOICE •

In carcinoma bladder-cystoscopy

•

In rupture bladder-CT cystogram

•

In rupture urethra-ascending urethrography

•

In posterior urethral valve-micturating cystourethrography (MCU).

•

In vesicoureteric reflux-MCU or VCUG

A 45-year-old multiparousfemale patient was diagnosed to have bladder stone. She underwent vaginal examination because she also had pain in her genitalia.She underwent dilatation and curettage. On dilatation, tail of Cu-T with thread was seen in the uterus. On trying to remove Cu-T, only thread could be extracted. She underwent laparotomy. Uterus was found plastered to urinary bladder. On opening the bladder, a stone of6 x 6 cm with Cu-T in pregnated in it was present. The Cu-T had perforated anterior wall of uterus and posterior wall of the bladder. It was removed. (Courtesy: Prof Rajiv Shetty, Professor Shivaswamy, Professor Durganna, Bangalore Medical College, Bengaluru) WHAT IS NEW IN THIS CHAPTER?/ RECENT ADVANCES

• All the topics have been updated • Vesicoureteric reflux has been added • New images are added

The Urinary Bladder and Urethra

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MULTIPLE CHOICE QUESTIONS

1. Following type of malignant tumour can occur in urinary bladder except: A. Transitional carcinoma B. Adenocarcinoma C. Squamous cell carcinoma D. Leiomyosarcoma 2. Following are true for internal sphincter of the urinary bladder except: A. It is a smooth muscle B. It is innervated by adrenergic fibres C. It prevents retrograde ejaculation D. It is supplied by pudenda! nerve 3. Cystine calculi in the urinary bladder are radio-opaque because of: A. High calcium content B. High sulphur content C. High triple phosphate content D. High oxalate content 4. Following are clinical features of urinary bladder stone except: A. Pain referred to the testis B. Pain aggravated by jumping C. Strangury D. Haematuria 5. Which of the following parasitic infestations is a strong risk factor for carcinoma urinary bladder? A. Bilharziasis B. Ascariasis C. Leishmaniasis D. Clonorchis sinensis 6. Following are true for carcinoma urinary bladder except: A. Aniline dye workers have high risk B. Transitional cell carcinomas are more common C. Strangury D. Adenocarcinoma of the bladder is due to bilharziasis 7. Following are true in ectopia vesicae except: A. Penis is normal B. Posterior wall of the urinary bladder is seen C. Pubic symphysis is widely separated D. Adenocarcinoma urinary bladder occurs very early 8. Most common organism causing acute cystitis are: B. Klebsiella A. E.coli D. Pseudomonas C. Proteus

9. Following are the causes for vesicovaginal fistula except: B. Anterior colporrhaphy A. Protracted labour C. Radiation D. Crohn's disease 10. The most worrying complication following uretero­ sigmoidostomy is: A. Acidosis B. Adenocarcinoma C. Alkalosis D. RecmTent infection and septicaemia 11. Which of the following is not the feature of intra­ peritoneal rupture of the urinary bladder? A. Shock B. Urgent and frequent desire to pass urine C. Suprapubic pain D. Hypotension 12. Which is the narrowest portion of male urethra? B. External meatus A. Penile urethra C. Perinea! urethra D. Bulbar urethra 13. Which of the following is not part of the triad of rupture of bulbar urethra? A. Perinea! haematoma B. Blood at urethral meatus C. Distended bladder D. Floating prostate 14. What is the first advice given to the patient in rupture bulbar urethra? A. Blood transfusion B. Intravenous antibiotics C. Not to try pass urine D. Immediate catheterisation 15. Floating prostate--Vermooten's sign is classical of: A. Rupture bulbar urethra B. Rupture penile urethra C. Rupture intraperitoneal urinary bladder D. Rupture membranous urethra 16. Which of the following is true for posterior urethral valves? A. They are acquired B. Bladder is thin walled and more prone for rupture C. Renal failure is uncommon D. They are symmetrical valves

ANSWERS D 11 B

2 D 12 B

3 B 13 D

4 A 14 C

5 A 15 D

6 D 16 D

7 A

8 A

9 D

10 B

41 Prostate and Seminal Vesicles • Surgical anatomy • Structural anatomy • Benign prostatic hyperplasia (BPH)

SURGICAL ANATOMY Embryology and lobes • The prostate develops around 12th week of intrauterine life. Primitive buds given from urethra form the glandular tissue and surrounding mesenchyme forms the fibromuscular stroma. Developmentally, the prostate has 5 lobes: Anterior, posterior, 2 lateral and 1 middle lobe. • Middle lobe (median lobe) is situated in between the two ejaculatory ducts and the urethra. The enlargement of this lobe in benign hypertrophy of the prostate is responsible for the obstruction of urethra. This lobe enlarges upwards into the bladder (Fig. 41.1).

Fig. 41.1: Prostate-lobes

• • • •

Carcinoma of the prostate Gleason score Prostatitis What is new?/Recent advances

• In benign prostatic hypertrophy (BPH), the glands of the inner adenomatous zone hypertrophy and lead to urinary outflow obstruction. Carcinoma usually occurs in the outer nonadenomatous zone. • New terminology for BPH arising zone is transitional zone and carcinoma arising zone is peripheral zone. Structural anatomy • Prostatic urethra is surrounded by a fibroadenomatous gland. • Urethral glands open into the prostatic urethra. These submucosal glands are responsible for BPH. • When the prostate enlarges, it compresses the outer zone resulting in a false capsule. • The outermost zone is the zone of prostatic glands proper which is responsible for carcinoma of prostate (Fig. 41.2).

Fig. 41.2: Prostate: (A) Outer carcinomatous zone, (B) Inner

adenomatous zone

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973

Prostate and Seminal Vesicles

• In between the fasciculations, there are depressed areas which are called sacculations. • Since the sacculi are thin, as the pressure increases, herniation occurs outside resulting in diverticuli. • In the diverticuli, there is stasis of urine resulting in secondary infection and stone formation.

• Surrounding this, there is fascia of Denonvilliers which is a part of pelvic peritoneum. • Between the anatomical capsule and pelvic peritoneum, prostatic venous plexus is present which may give rise to massive haemorrhage, if injured. BENIGN PROSTATIC HYPERPLASIA (BPH) AETIOPATHOGENESIS There are 2 theories to explain BPH. I. Hormonal theory • It has been compared to fibroadenosis in female patients. • As the age advances, the levels of androgens come down. There is a corresponding increase in the oestrogen which stimulates the prostatic gland and producesBPH. 2. Neoplastic theory According to this theory there is proliferation of all the elements of prostate: Fibrous, muscular and glandular resulting in fibromyoadenoma.

3. Changes in the ureter and kidney (Key Box 41.1) Bilateral hydronephrosis and bilateral hydroureter are the end result ofBPH, which may result in renal failure.

............................. . KEY BOX 41.1

BPH-BACK PRESSURE EFFECTS ON THE BLADDER Bladder muscle hypertrophy Fasciculations Sacculations Diverticuli

SECONDARY EFFECTS OF BPH I. Urethral changes • Urethra gets compressed, elongated and gets converted into a narrow, longitudinal slit. • The effect is more with median lobe enlargement which is due to enlargement of subcervical glands. Lateral lobes enlarge when there is involvement of submucous glands. 2. Changes in the bladder (Fig. 41.3) • As a result of obstruction, the bladder musculature undergoes hypertrophy. Very prominent thick bundles of the muscle can be seen, which are called fasciculations or trabeculations.

Stasis, infection, stone

CLINICAL FEATURES OF BPH (Key Box 41 .2) • Frequency, urgency, hesitancy are triad of BPH - Frequency: To start with, frequency is present during the day time followed by day and night (5-10 times during the night). It is due to ineffective emptying of the bladder. It results in residual urine in the bladder precipitating cystitis. - Urgency: As the prostate enlarges there is vesical introversion of sensitive mucous membrane of prostatic urethra within the bladder. This causes the internal sphincter to stretch and prevents contraction. This results in a few drops of the urine trickling down the posterior urethra resulting in an urgent desire to pass urine (urgency). - Hesitancy: Patient hesitates to pass urine because it is so ineffective due to obstruction.

.....,....,.______ Bladder muscle hypertrophy

MIii!........._.� BPH-CLINICAL FEATURES

Fig. 41.3: Secondary changes in the urinary bladder due to BPH

• • • • •

Frequency Urgency Hesitancy Acute retention of urine Chronic retention of urine

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Manipal Manual of Surgery

• Haematuria is rare - It is due to congestion of prostatic venous plexuses resulting in hyperaemia and haematuria. • BPH with acute retention of urine - This occurs due to postponement of micturition, following alcohol or drugs like mydriatics. • BPH with chronic retention of urine - Many of the patients present with chronic retention of urine, with painless enlargement of the urinary bladder. COMPLICATIONS • Stones • Diverticuli • Renal failure • Recurrent urinary tract infection DIAGNOSIS OF BPH Digital rectal examination: Enlarged lateral lobes can be easily felt. Rectal mucosa is free (In an enlarged prostate gland, in case of carcinoma of prostate, the mucosa of the rectum cannot be moved if it has infiltrated into the rectum.). Grading of prostate is done as follows I. The prostatic Jobes protrude minimally into the rectal lumen by 1-2 cm, the median sulcus is palpable. II. Prostatic lobes protrude> 2 cm but < 3 cm into the rectal lumen and the median sulcus is obliterated. HI. 3--4 cm protrusion IV. > 4 cm protrusion of lobes, most of the rectal lumen is filled by the projecting prostatic lobes. INVESTIGATIONS l. Blood urea and creatinine: Raised levels indicate renal failure. 2. Uroflowmetry: The person is asked to void urine from his full bladder into the flowmeter. The flow rate is assessed. Peak flow rate • Normal peak flow rate: 20 ml/sec. • Doubtful peak obstruction: 10 to 15 ml/sec. • Definite peak obstruction: Less than 10 ml/sec. • Thus, the degree of bladder outlet obstruction (BOO) can be secured by uroflowmetry in case of BPH. 3. Ultrasonogram: To assess the size and weight of prostate, to assess the residual urine and to look for hydro­ ureteronephrosis, bladder wall changes. TREATMENT It can be classified into medical treatment and surgical treatment. I. Medical treatment of BPH If the patient has mere frequency of micturition and if the residual urine is not much (< 150 ml), uroflowmetry shows more than 15 ml/sec of urine flow and if there are no back

pressure effects on the kidney, the patient can be reassure< advised to avoid heavy alcohol consumption which may lea to prostatic congestion and acute retention of urine. To avoi over distension of the bladder, he has to void the urine as an when he feels the urinary sensation of micturition, and shout, not postpone micturition. Drugs a. Finasteride acetate 5 mg daily for 6 months. It is a Sa reductase inhibitor. It helps in prevention of hyperplasi: of the prostate. It is given for large prostates. b. a-adrenergic blockers: It is supposed to relax the intema sphincter for better drainage of the bladder. They are give1 when the prostate is less than 40 g (small) as measured b: ultrasound.Tamsulosin (most selective), terazosin, alfazocir are a few drugs used today. II. Surgical treatment of BPH Indications for surgery 1. Acute retention of urine 2. Chronic retention of urine with postvoid residual urim more than 200 ml. 3. If frequency of micturition is so much that it disturbs the normal lifestyle during daytime. 4. Complications such as haematuria due to congestion o1 prostatic venous plexuses, hydroureteronephrosis, prostatic diverticulosis, vesical calculus and recurrent infections. SURGICAL METHODS 1. Transurethral resection of the prostate (TURP) • This is the most popular method today. • A resectoscope is passed through the urethra and under vision with constant irrigation with water or glycine, the prostate is resected into multiple pieces and removed. • Haemostasis is obtained with the help of a cautery. Advantages 1. Postoperative recovery is smooth and rapid. 2. Incontinence is rare because the chances of damage to the internal sphincter are very low. Disadvantages 1. TURP syndrome with water intoxication and electrolyte imbalance, if water is used as irrigating fluid. 2. If there is BPH with diverticuli and stone, TURP has to be followed by litholapaxy. 2. Transvesical suprapubic prostatectomy • This method is now restricted to glands more than 100 g in weight and associated with calculus. • Ankylosis of hip/other orthopaedic conditions (difficulty in positioning).

Prostate and Seminal Vesicles

Through an extraperitoneal approach the bladder is opened, prostate is enucleated with finger, bleeding is controlled by inflating the Foley bulb with about 3050 ml of air and by ligatures. • Bladder is drained by a Malecot's catheter which is wider than Foley's so that it can drain well if the bleeding occurs in the bladder. • During the process, the prostatic urethra is also avulsed. • After about 7-10 days, a tract develops along the length of Foley catheter which heals by granulation, fibrosis and forms the future prostatic urethra. Disadvantages • Blind resection • Chances of haemorrhage are more • Stricture of prostatic urethra 3. Retropubic prostatectomy Done by extraperitoneal approach without opening the bladder, pushing the bladder to one side and excision of the prostate. 4. Perineal prostatectomy Not done nowadays Newer treatments • Holmium: YAG laser. • Intraurethral stents-in men who are grossly unfit (ASA Grade IV) for surgery. CARCINOMA OF THE PROSTATE • Carcinoma of the prostate is common after the age of 65 years. The incidence increases with age. • In Western countries, it is the second most common type of carcinoma in males after 65 years, first being bronchogenic carcinoma. • Prostatectomy done for BPH does not give protection against development of carcinoma of prostate because during prostatectomy the outer zone is left undisturbed (not resected). CLINICAL FEATURES 1. Histological surprise Prostatectomy done with diagnosis of BPH but histology reveals carcinoma of the prostate. 2. Multiple bone pain, confused for rheumatism, is due to multiple metastasis. 3. Rectal examination: Reveals a hard nodule on the anterior wall of the rectum, obliteration of median sulcus. The rectal mucosa cannot be moved over the prostate but it is not ulcerated (fascia of Denonvilliers prevents the spread of carcinoma prostate into the rectum). 4. Elderly man with bilateral sciatica with metastasis in the thoracolumbar vertebrae. 5. Acute retention of urine occurs in 5-10% of cases of carcinoma of prostate.

975

6. Difficulty in passing urine, painful micturition and sometimes with haematuria is due to involvement of prostatic urethra. SPREAD (Key Box 41 .3) 1. Haematogenous spread • This is due to retrograde tumour embolisation which occurs through prostatic venous plexus which communicates through the emissary veins with the bone (Batson 's paravertebral plexus of veins). Peculiarities of secondary deposit from carcinoma of prostate: • They are multiple • Moth-eaten appearance • Osteoblastic (in most of other secondaries, they are osteolytic). • Most common site of origin for skeletal metastases.

MHMI�� 1. 2. 3. 4. 5.

BONES INVOLVED IN CARCINOMA PROSTATE Thoracolumbar vertebrae (Fig. 41 .4) Pelvic bone, iliac crest Femur Scalp Ribs

2. Lymphatic spread • Prostatic chain of lymphatics drain into internal iliac nodes. • When spread occurs along seminal vesicle, external iliac nodes are enlarged. • From this group of nodes, para-aortic nodes, mediastinal nodes, followed by left supraclavicular nodes get involved.

Fig. 41.4: Plain X-ray showing destruction of vertebrae

Manipal Manual of Surgery

976

3. Local spread • On the medial side it can involve the prostatic urethra and give rise to retention of urine. • When it spreads upwards, the bladder can get involved resulting in painful haematuria. • Superiorly it can also involve seminal vesicle. • Rectum is involved very late in carcinoma of prostate because of the tough Denonvillier 's fascia.

..

-

STAGING TNM STAGING

Staging using the tumour, node, metastasis (TNM) system (Fig. 41.5)

..

1. T1 a, T1 b and T1 c. These are incidentally found tumours in a clinically benign gland after histological examination of a prostatectomy specimen. T1 a is a tumour involving less than 5 per cent of the resected specimen; these tumours are usually well or moderately well differentiated. T1 b is a tumour involving > 5 per cent of the resected specimen. Tic tumours are impalpable tumours found following investigation of a raised PSA. 2. T2a disease presents as a suspicious nodule on rectal examination confined within the prostate capsule and involving one lobe. T2b disease involves both lobes. 3. T3 tumour extends through the capsule (T3a, uni- or bilateral extension. T3b, seminal vesical extension). 4. T4 is a tumour that is fixed or invading adjacent structures other than seminal vesicles-rectum or pelvic side wall

TO

Fig. 41.6: Bone scan showing extensive metastasis

T1

Fig. 41.7: Lytic lesions in pubic bones

T2b

T4

G

T2a

T3

Fig. 41.5: Tumour, node, metastasis (TNM) staging for prostate cancer

INVESTIGATIONS 1. Transrectal trucut biopsy

Report-adenocarcinoma

2. X-ray of bones (Figs 41.6 and 41.7) Which are likely to be involved (already mentioned). 3. Serum acid phosphatase (Key Box 41.4) • The enzymes which split the organic phosphates are concentrated in the prostate which are responsible for acidic pH in the prostatic urethra. • Normally they are drained in the urine so that they are not detectable in the serum.

MIMI...........� ACID PHOSPHATASE INCREASED IN

1. 2. 3. 4.

Paget's disease of bone Acute prostatitis Cirrhosis of liver Carcinoma prostate

Prostate and Seminal Vesicles

• In carcinoma prostate because of the ductal blockage; it gets absorbed into the blood and thus high levels are reached, especially with metastasis. • 1 to 3 King-Armstrong units-suggestive of carcinoma of prostate. Requirements before estimation of acid phosphatase • Early morning blood sample • On empty stomach • Avoid fatty food • Per rectal examination should not be done before drawing the sample of blood. Significance of acid phosphatase Levels come down with the treatment of carcinoma of prostate, especially when bone metastasis disappears. 4. Serum alkaline phosphatase It is increased if there is extensive liver metastasis or bone metastasis. 5. Prostate-specific antigen • Prostate-specific antigen (PSA) is a neutral protease, elaborated by columnar prostatic acinar epithelial cells. • Ifit is more than 4 mnol/ml, carcinoma is to be suspected; 10 nmol/ml is suggestive of prostatic carcinoma; 35 nmol/ml: Disseminated carcinoma. • The highest concentration of PSA occurs in the lumen of the prostatic acini and ducts(up to million times greater than in systemic circulations). The prostatic luminal cells are normally surrounded by basal cells, prostatic basement membrane and prostatic stroma. • A number of diseases disrupt some of the barriers to absorption resulting in elevation of serum PSA, notably prostatic cancer, prostatic inflammation and infarction. PSA is also transiently elevated (up to 24 hours) after ejaculation and cycling. • PSA measw·ement is the most efficient screening test for prostate cancer and it increases further if the measurement is combined with digital rectal examination (DRA). • PSA measurement is also vital in staging prostate cancer and assessing response to treatment. PEARLS OF

WISDOM

Thus, PSA is organ-specific but not cancer-specific. 6. Abdominal and transrectal USG For staging of the disease 7. CT scan or MRI scan These are done before proceeding on to radical surgery to assess the extent of the tumour. 8. Bone scan Is indicated in cases of carcinoma prostate especially who have bony pains, raised alkaline phosphatase and also very high PSA levels(> 20 ng/ml) (see clinical notes).

977

A 75-year-old gentleman was admitted to the hospital because of giddiness and syncope. He was a diabetic. Initially, it was thought to be due to hypoglycaemia. Investigations revealed creatinine of 7. 5 mg%. Diagnosis of renal failure was made. Urgent ultrasound revealed bilateral hydronephrosis due to large para-aortic nodes compressing both ureters. He underwent emergency stenting of both ureters. Rectal examination revealed a hard prostate. PSA was 20 nmol/ml suggestive of dissemination. CT scan done later showed involvement of pelvic bones, thoracolumbar vertebrae and iliac bones. It was a case of carcinoma prostate. Retrospective analysis revealed that the patient was having backache since the last 6 months.

TREATMENT OF CARCINOMA OF PROSTATE

(Fig. 41.8)

It can be classified under following headings-early malignancy and late malignancy. I. Early malignancy

It refers to Tl or T2, NO, MO A. Early prostatic malignancy with PSA levels less than 20 nmol/ml • Radical prostatectomy is done for TI and T2 and in men with life expectancy of> IO years. Metastasis should be excluded by a negative bone scan, chest radiograph and serum PSA < 20 nmol/mL. Radical prostatectomy involves pelvic lymphadenectomy and removal of the prostate, seminal vesicle including the distal urethral sphincter followed by anastomosis of urethra to the bladder neck. • Most commonly injured vessel-dorsal venous complex. • Radical radiotherapy for prostate and pelvic nodes is given postoperatively. Disadvantages of radical prostatectomy Impotence and stress incontinence may complicate the surgery. B. Early prostatic malignancy with PSA is 20 nmol/ml or more and the patient is already beyond 65 to 70 years of age, surgery is not favoured. Radical radiotherapy is given. II. Late malignancy It means T3 lesions, involvement of regional nodes or presence of metastasis. 1. Androgen ablation in the form of bilateral orchidectomy is done as the tumour is androgen-dependent followed by

Manipal Manual of Surgery

978

I Age < 65 years General condition good Radical prostatectomy

T1 or T2, NO, MO

I

I

I

Age < 65 years General condition not good Radical radiotherapy (RT)

I

T3orN1,or M1 J

Bilateral orchidectomy + Stilbestrol therapy

BONY METASTASIS

+

l

Local radiotherapy

• Pathological fracture femur: Internal fixation + Palliative RT • Extensive bony metastasis: Hemibody irradiation

Fig. 41.8: Summary of the treatment of carcinoma prostate

anti-androgenic measures. Anti-androgenic measures are given below. A. Low orchidectomy Subcapsular orchidectomy used to be done earlier wherein tunica albuginea was left behind. It is now obsolete because it has been proved beyond doubt that some testicular tissue can still be left behind. Hence, a low bilateral orchidectomy is done. B. Drugs-(LHRH agonists, anti-androgens) 1. Oral stilboestrol therapy, dose 20-25 mg/day; phosphorylated diethylstilboestrol (Honvon) IV initially later, orally. Within 48 hours of treatment, dysuria improves, pain disappears, bone pains improve and metastasis may disappear. Disadvantages • Thromboembolism • Nausea and vomiting • Gynaecomastia: Can be prevented by preoperative radiation to the breast-800 rads. 2. Phosphorylated diethylstilboestrol is an excellent drug which can be given orally and IV, and the drug is not broken down until it reaches prostate. Hence, systemic toxicity of stilboestrol does not occur. In the prostate it is broken down by acid phosphatase to release stilbestrol locally. 2. Radiotherapy A. For localised metastasis in bones or brain, radiotherapy relieves the symptoms. B. For generalised metastasis, hemibody irradiation is excellent to improve the symptoms and the survival time. C. If carcinoma of the prostate is diagnosed only by a biopsy of the prostate removed during TURP, no surgery needs to be done. Local radiotherapy to the prostatic bed 1s sufficient.

D. If the diagnosis of carcinoma of prostate is made on rectal findings, surgeons have no role to play because many a time it is an advanced cancer and there is a danger of bleeding from prostatic venous plexus. Hence, radiotherapy is administered by using linear accelerator (cobalt). 3. Palliative treatment of carcinoma prostate A. Local radiotherapy to the enlarged para-aortic nodes and to the bone. B. Chemotherapeutic drugs • Mitomycin and nitrogen mustard are being tried. • Docetaxel-new drug with better results. Recent advances in carcinoma prostate P rostate cancers exhibit heterogenecity within tissue, so two histological areas of prostate are each scored between I and 5. The two scores are added to give an overall Gleason score of between 2 and 10. The score correlates well with the likelihood of spread and prognosis. Gleason score • 6 or lower-often candidates for an active surveillance program (previously referred to as watchful waiting therapy). • ?-usually associated with a critical decision-making step (usually some form of definitive therapy). • 8-10-often candidates for adjuvant therapy or radiation treatment.

PROSTATITIS • Inflammation of prostate can be acute or chronic. • However, in both types, in addition to the prostate, seminal vesicles and posterior urethra are involved. • Often the diagnosis is delayed because of varying symptoms which will be attributed to a different cause and they are being wrongly treated.

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• If the treatment is not effectively given, infection persists and later it is difficult to eradicate. ACUTE PROSTATITIS Aetiology • Causative organisms are Escherichia coli, Staphylococcus aureus and Staphylococcus a/bus. • The infection is usually due to haematogenous spread from a distant focus or secondary to urinary tract infection. • Instrumentation or invasive urological procedures are also the factors. Clinical features • The patient is ill with high grade fever, chills and rigors. • Pain all over the body, more so in the back. • Perinea! heaviness or pain, rectal irritation and urethral discharge are also the features. • Pain on micturition is common and initial samples of urine contain 'threads'. • Rectal examination finding: Tender, boggy enlarged prostate. Fluctuation indicates prostatic abscess (rare). Treatment • Hospitalisation, intravenous fluids, antipyretics. • Antibiotics such as ciprofloxacin or norfloxacin should be given for 2-3 weeks. Otherwise, recurrent attacks of prostatitis can occur. • If abscess is suspected, diagnosis can be confirmed by transrectal ultrasound, it can be drained by transurethral unroofing of abscess cavity similar in technique to transurethral resection of prostate (TURP). CHRONIC PROSTATITIS Chronic prostatitis results due to inadequately treated acute prostatitis (Key Box 41.5). Clinical features • Elderly men are affected and they complain of perinea! heaviness, perinea! discomfort or pain on sexual intercourse. • Intermittent fever is also a feature • Rectal examination may reveal a boggy and tender prostate. • Low backache

Diagnosis Prostatic massage is given by bidigital method-index finger in the rectum and the thumb in the perineum to one side. Now the patient is asked to void the urine. Presence of prostatic threads or mucopus in the postprostatic massage urine is diagnostic of chronic prostatitis. Treatment Prolonged antibiotic therapy-norfloxacin, trimethoprim and metronidazole are used.

•.............. • • • • • • •

CHRONIC PROSTATITIS Follows recurrent prostatitis Elderly men are affected Symptoms-misleading Perinea! heaviness, back pain Postprostatic massage-threads in urine Pus cells and bacteria-prostatic fluid Prolonged antibiotics

�

INTERESTING WISDOM LINES • Do not injure prostatic venous plexus (between anatomical capsule and pelvic peritoneum) during prostatectomy or abdominoperineal resection (APR) done for carcinoma rectum. • Frequency of micturition is not an indication for prostatectomy. • Most commonly done surgery for BPH is transurethral resection. • Prostatectomy for BPH does not give protection from development of prostatic carcinoma. • Androgen ablation is the chief form of treatment in advanced prostatic cancers.

WHAT IS NEW IN THIS CHAPTER?/RECENT ADVANCES

• All the topics have been updated. • New photographs and key boxes have been added. • Gleason score is added

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MULTIPLE CHOICE QUESTIONS 1. Following are true for surgical anatomy of the prostate except: A. Developmentally it has 5 lobes B. Median lobe enlargement causes carcinoma prostate C. Develops around 12th week of intrauterine life D. Between prostate and rectum, Denonvillier's fascia exists 2. Following are the changes in the urinary bladder due to benign prostatic hypertrophy except: A. Facsiculations B. Sacculations D. Carcinoma C. Diverticuli 3. Vesical introversion of the sensitive prostatic urethra within urinary bladder causes: B. Urgency A. Frequency C. Hesitancy D. Haematuria 4. Following are complications of benign prostatic hypertrophy except: A. Stones B. Renal failure C. Recurrent urinary tract infection D. Carcinoma urinary bladder 5. Which of the following drug is used to treat benign prostatic hypertrophy? A. a adrenergic blockers B. � adrenergic blockers C. Oral stilboestrol D. Oral prednisolone 6. Following are true for role of ultrasound in benign prostatic hypertrophy except: A. Can assess the size B. Can assess the weight C. Can assess residual urine D. Can assess the flow rate 7. Following are true for carcinoma prostate in digital rectal examination except: A. Hard nodule is felt B. Median sulcus is obliterated C. Rectal mucosa cannot be moved D. Ulcerated mucosa 8. The reason for early bone spread from carcinoma prostate to bones is spread through: A. Batson 's plexus B. Santorini plexus of veins C. Waldeyer's plexus of veins D. Denonvillier's plexus of veins

9. Following are the features of bone secondaries fror carcinoma prostate except: A. Thoracolumbar vertebrae are involved B. Moth-eaten appearance in X-ray C. Osteolytic D. Multiple bones are affected 10. Which structure is affected late in carcinoma prostate A. Prostatic urethra B. Seminal vesicles C. Rectum D. Urinary bladder 11. Which of the following is not true for aci4 phosphatase? A. It is responsible for acidic pH in the prostatic urethr B. High values suggest carcinoma prostate C. It is elevated not only in carcinoma prostate but als1 other conditions D. Early morning urine sample is the best to measure thi. 12. Following are true for prostate specific antigen except A. Released from columnar prostatic acinar epithelia cells B. More than 4 nmol/ml suggest carcinoma prostate C. Prostatitis also can increase in the levels D. It does not help in assessing the response to treatmen 13. Which of the following is not done in cases ol carcinoma prostate? A. Radical prostatectomy B. Radical radiotherapy C. High orchidectomy D. Stilboestrol therapy 14. What is the treatment to follow after prostatectomy done for benign prostatic hyperplasia is reported as carcinoma prostate? A. Orchidectomy B. Stilboestrol C. Bisphosphonates D. Local radiotherapy 15. Following are true for acute prostatitis except: A. Usually it is haematogenous infection B. Instrumentation can also cause this C. Usually it is mild self-limiting D. Urine sample may contain 'threads' 16. Which of the following is true for posterior urethral valves? A. They are acquired B. Bladder is thin walled and more prone for rupture C. Renal failure is uncommon D. They are symmetrical valves

ANSWERS 1 B

2 D

3 B

4 D

5A

60

11 D

12 D

13 D

14 D

15 D

16 B

7 D

8A

9 C

10 C

42 Penis, Testis and Scrotum • • • • • • • •

• • • • • • • • •

Surgical anatomy of penis Phimosis Paraphimosis Carcinoma of penis Peyronie's disease Anatomy of the testis Hydrocoele Undescended testis

SURGICAL ANATOMY OF PENIS • It consists of two corpora cavernosa and one corpus spongiosum (Fig. 42.1 ). • Corpora cavernosa are vascular spaces wherein arterioles open directly. They are corkscrew shaped (helicine arteries) which allow their elongation in erection. • Corpora spongiosum is perforated by urethra and is continuous distally with the glans. • Each corpus is enclosed by tough fibrous membrane-the tunica albuginea of the corpus. • The fused fibrous sheaths are attached to the under surface of the symphysis pubis by a triangular sheet of fibrous tissue called as suspensory ligament. It has to be divided during total amputation of the penis. Blood supply • The artery to the bulb supplies corpus spongiosum and the glans. • Deep artery supplies corpus cavemosum alone-its sole function is erection. • Dorsal artery supplies skin, fascia and glans • Superficial dorsal vein drains into superficial external pudenda! veins. Deep dorsal vein enters prostatic venous plexus. Penile urethra • Entire penile urethra is lined with transitional epithelium except dilated anterior part in the glans-fossa navicularis-lined by stratified squamous epithelium. 981

Ectopic testis Varicocoele Spermatocoele Epididymal cyst Torsion testis Testicular tumours Fournier's gangrene Fracture penis What is new?/Recent advances

Lymphatic drainage • Medial group of superficial inguinal nodes. • Some from glans pass directly to the node of Cloquet Nerve supply • Parasympathetic nerves-nervi erigentes: stimuli resulting in erection of the external genitalia. • Sympathetic system-hypogastric nerves: helps 111 ejaculation. PHIMOSIS Inability to retract the prepucial skin is described as phimosis.

Deep��-������� dorsal ___:;:,.:;�;:---- Superficial vein dorsal vein Fascia penis Tunica albuginea

Urethra

---corpus cavernosum Corpus spongiosum

Fig. 42.1: Surgical anatomy of the penis

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Causes I. Congenital: Most common type seen in young patients. 2. Secondary to chronic balanoposthitis: Balanitis means inflammation of glans penis and posthitis means inflammation of the prepuce. Balanoposthitis is common in diabetic patients. 3. Chancre also can cause phimosis. 4. Carcinoma of the penis can present as a recent phimosis. Clinical features I. Inability to retract the prepuce. 2. In children, ballooning of the prepuce (second bladder) can be seen, which is diagnostic of phimosis. 3. Because of the phimosis, they are more prone for balanoposthitis because of inability to clean the glans.

Treatment 1. Sedation 2. Injection hyaluronidase 250 units in 10-15 ml of saline i injected into the constriction ring (prepucial skin which i retracted) 5-10 minutes later, when oedema is reduced, wit! gentle manipulation it is possible to reduce th1 paraphimosis. 3. Dorsal slit is given so that reduction can be done Circumcision follows later. Complications 1. Ulcers of glans 2. Gangrene of glans in later stages CARCINOMA OF PENIS Most common type - squamous cell carcinoma.Othe1 varieties are-melanoma, adenocarcinoma, basal cell carcinoma (BCC)

Complications I. Carcinoma of the penis (Fig. 42.2) 2. Paraphimosis

Premalignant lesions

Treatment Circumcision: Removal of the prepuce

1. Genital warts: Buschke-Lowenstein tumour is a giant penile condyloma and resembles squamous cell carcinoma. It is a cauliflower-like lesion, may have foci of malignancy.

PARAPHIMOSIS In this condition, the retracted skin of the glans penis (prepuce) cannot be pulled forwards. As a result of this, the retracted skin acts like a tight constricting agent which compresses the corona resulting in venous congestion. As venous congestion increases, glans swells up resulting in paraphimosis. Causes (Key Box 42.1) I. During catheterisation, if the retracted prepuce is not pulled forwards, it results in paraphimosis. 2. This can follow after a sexual intercourse.

2. Erythroplasia of Queyrat or Paget's disease of penis: a persistent red, raw lesion which is a precancerous condition. 3. Leukoplakia: Persistent nonspecific patch in the glans or in the prepucial skin. Interestingly, leukoplakic patches are not white in the penis. 4. Bowen's disease: This presents as a small eczematous plaque. Carcinoma in situ is a complication which develops within this plaque.

Clinical features 1. Severe pain in the glans penis 2. Gross swelling of retracted prepucial skin and oedema of the distal glans penis.

•,..........,,� • • • •

PHIMOSIS-CAUSES Cancer Chancre Congenital Chronic disease: Diabetes mellitus

Fig. 42.2: Ca penis proliferative growth. Patient had phimosis

Penis, Testis and Scrotum

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Fig. 42.3: This patient presented with fungating inguinal lymph nodes. Observe the penis-he has undergone partial amputation of the penis 2 years back.

Fig. 42.4: This patient had a large foul smelling proliferative lesion in the glans penis with fungating inguinal lymph nodes. Such cases are advanced cases with poor prognosis

Fig. 42.5: Carcinoma penis difficult to find penis-ask the patient where he passes urine-he will show you the urethral opening

Fig. 42.6: Carcinoma penis-proliferative lesion with gross oedema due to secondary infection

AETIOLOGY

Clinical features

Phimosis

l . Carcinoma penis is common in 6th decade. Majority of patients present with nonhealing ulcer. 2. Foul smelling discharge is common and occasionally it is blood stained (Figs 42.3 to 42.6). 3. Recent phimosis due to growth underneath the prepuce. 4. Haematuria, pain while passing urine indicate locally advanced tumours. 5. On examination, very often there is an ulceroproliferative growth with everted edges and induration of the base and edge. The induration is much more extensive than the lesion. Hence, entire shaft has to be examined for evidence of induration. 6. Urethra is rarely involved in carcinoma of penis because it is protected by the tough Buck's fascia, which is a part of pelvic fascia. In large fungating lesions, it may be

1. Extremely rare in Jews who practise circumcision immediately after birth. 2. Carcinoma penis is rare in Mohammedans who practise circumcision few years after birth. 3. Carcinoma penis is common in Hindu and Christian population who do not practise circumcision. Due to the prepucial skin, smegma collects within, which is responsible for chronic irritation giving rise to carcinoma of penis. PEARLS OF

WISDOM

Circumcision done within 1 year confers immunity against Ca penis.

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difficult to identify the external urinary meatus. In such situations the patient will point out at the external urinary meatus. SPREAD

1. Direct spread: Involves the glans, prepucial skin and shaft. 2. Lymphatic spread: Inguinal nodes are enlarged. 30% cases are due to infection. Nodes are fom and tender in infection. Hard nodes suggest metastases. Later, internal iliac and para­ aortic nodes can also get enlarged. In advanced cases the lymph nodes may show fungation as in Fig. 42.4. Cause of death

Cause of death in carcinoma penis: Erosion of femoral vessels by inguinal lymph nodes. STAGING

I. II. III. IV.

Tumour confined to the glans or prepuce Tumour involving the penile shaft or corpora cavemosa Mobile regional nodal metastases, with stage I or II Tumour beyond penile shaft, fixed regional lymph node or distant metastases.

INVESTIGATIONS

l . Wedge biopsy from the edge of the growth, proves the diagnosis of squamous cell carcinoma nodes. 2. FNAC of enlarged inguinal lymph nodes. 3. Ultrasound and MRI are helpful in lesions invading corpora cavernosa (soft tissue details). Ultrasound guided FNAC of lymph nodes. 4. CT scan is useful in obese patients wherein clinical examination of inguinal nodes is difficult 5. CT guided FNAC of pelvic nodes DIFFERENTIAL DIAGNOSIS

TREATMENT

It can be divided into treatment of the primary and treatmer of the secondaries. I. Treatment of the primary

Stage I

1. Growth confined to the prepuce-circumcision. Regula follow up is necessary. 2. Growth involving the glans: Partial amputation with a least 2 cm margin from the palpable indurated edge of th1 tumour. Stage II 1. Partial amputation: After having a macroscopic tumour free, 2 cm margin proximally, if there is adequate length o penile shaft (minimum 2.5 cm) to carry out the sexua function and for directing the urinary stream, a partia amputation can be done (Figs 42.7 and 42.8). 2. Total penectomy with perinea! urethrostomy, if adequatE shaft cannot be retained. This is a major operation, patien1 has to be clearly instructed about the consequences anc complications of perinea! urethrostomy. Complications of perinea! urethrostomy

1. Ammoniacal dermatitis of scrotum. To prevent this, patient has to lift the scrotum to pass urine. 2. Stricture of perinea! urethra which can be dilated by Hegar's dilators. Stage Ill • Circumcision, partial amputation or total penectomy followed by ilioinguinal block dissection.

1. Condyloma acuminatum 2. Buschke-Lowenstein tumour 3. Balanitis xerotica obliterans

Fig. 42.7: Partial amputation in progress-veins are exposed

Fig. 42.8: Stump seen with catheter in place

Penis, Testis and Scrotum

if node is positive for malignancy, a complete inguinal block dissection is indicated. In case of negative nodes, nothing else is required and patient is kept under regular follow­ up.

II. Treatment of inguinal lymph node secondaries Before discussing ilioinguinal block dissection, it is necessary to know the concept of sentinel lymph node biopsy (SLNB). • The concept of SLNB was first described by Cabana in 1977. He demonstrated consistent drainage of the penile lymphatics into a sentinel lymph node or groups of lymph nodes, located superomedial to the junction of saphenous and femoral veins in the area of superficial epigastric vein. He postulated that this sentinel lymph node is the first to get involved in the penile malignancy and if this sentinel lymph node is negative for tumour, metastasis to other inguinal lymph nodes will not occur. Metastasis to this lymph node will indicate the need for complete ilioinguinal block dissection. • Technique: A dye, isosulphan blue, is injected at the site of primary tumour. After sometime, the inguinal dissection is done to expose the sentinel lymph node area and the lymph nodes, which take up the dye. These are removed and sent for pathological examination. Based on the report,

985

• Only 50% of patients presenting with palpable lymph nodes actually have metastatic disease, the remainder have lymph node enlargement secondary to inflammation. So, subjecting all the patients with inguinal lymphadenopathy to surgery is not recommended. Hence, a course of antibiotic is given and wait for a period of 4-6 weeks, if the nodes are still palpable, block dissection is carried out. • However this antibiotic policy can be followed in low grade tumour such as in situ carcinoma and Tl lesions. If the lymph nodes regress, wait and watch. If the grade of the tumour is high, do not give antibiotics. FNAC is done and treat accordingly. • Also, if the nodes are not palpable, and if primary tumour is poorly differentiated, superficial lymph node dissection is done. If the nodes are positive, then modified inguinal block dissection (Catalona) can be done. • Algorithm (Fig. 42.9) to help in the management of these patients is given below.

Primary surgery for carcinoma penis

Palpable nodes

Treat with antibiotics for 4-6 weeks +-- Follow up after 6 weeks

Nodes not palpable

Primary lesion well differentiated 5 cm in greatest dimension M: Distant metastasis Mx: Distant metastasis cannot be assessed MO: No distant metastasis M1: Distant metastasis present M1a: Nonregional nodal or lung metastasis M1b: Nonpulmonary visceral metastasis (M2) 1Staging also considers serum tumour markers -8-LDH, HCG, AFP

SPREAD • Seminoma spreads by lymphatic route. Along the testicular vessels it spreads to para-aortic lymph node mass, through thoracic duct, to mediastinal nodes and left supraclavicular nodes. Spread does not occur to inguinal nodes unless scrotum is incised. • Malignant teratomatous tumours spreads predominantly by blood. STAGING OF TESTICULAR CANCER • Stage I: Tumour confined to the testis only. • Stage IIA: Tumour and lymph nodes below the diaphragm­ size less than 2 cm. • Stage IIB: Tumour and lymph nodes below the diaphragmsize more than 2 cm. • Stage III: Tumour and lymph nodes above the diaphragm. • Stage IV: Blood spread to lungs/liver/elsewhere. INVESTIGATIONS

Fig. 42.40: Seminoma testis with para-aortic nodes-clinical examination of testis is very important in an upper abdominal mass

1. No biopsy should be done through scrotal route because if the scrotal skin is involved, the spread occurs to inguinal lymph nodes opening up one more channel of lymphatics. Even FNAC is not recommended. 2. Chest X-ray: To rule out cannon ball secondaries as in teratoma.

Penis, Testis and Scrotum

3. Ultrasound of the testis: Seminoma appear as hypoechoic lesion and nonseminomatous tumours as inhomogenous. 4. Abdominal ultrasonography to see for enlarged lymph nodes, secondaries in the liver, or to detect a tumour in an undescended testis. However, CT scan is a better investigation. 5. CT scan: Heterogenously enhancing testicular mass with retroperitoneal lymph nodes and liver metastasis can be made out (Fig. 42.41). 6. 24 hours urine sample for HCG • Normal levels-less than 100 JU More than I 000 JU is diagnostic of choriocarcinoma • Hence, it is the tumour marker of choriocarcinoma. 7. Human chorionic gonadotrophin: Serum HCG (Key Box 42.6) • As the name suggests, it is made by chorionic elements. • HCG as a hole (a- and �-HCG) may be increased in testicular neoplasm, melanoma, lymphoma, etc. It can also be raised in nonmalignant conditions such as cirrhosis, peptic ulcer disease.

997

• It is the �-HCG which is more important in diagnosing testicular neoplasms and also useful in the postoperative period to know residual tumour or recurrent tumour. • The blood levels of �-HCG is O ng/ml. 8. a-fetoprotein: Increased in nonseminomatous germ cell tumours. 9. Lactate dehydrogenase (LOH): Increased in nonsemino­ matous germ cell tumours. LOH levels indicate tumour load. It is not specific for any tumour. 10. Placental alkaline phosphatase is increased in seminoma testis. TREATMENT I. Treatment of the tumour • Radical inguinal orchidectomy is the treatment of choice in all testicular tumours irrespective of the histological type and stage. When a patient presents with rapidly growing testicular swelling and the neoplasm is doubtful, testis is explored through an inguinal incision. It is delivered out and a soft clamp is applied to the testicular vessels at the level of deep ring while doing the procedure so that tumour embolisation does not occur. Testis is split open, the suspicious area is biopsied and sent for frozen section. If the frozen section is positive, the cord and testicular vessels are divided at the level of deep ring and testis is removed. This is called as high orchidectomy. If frozen section is negative, the testis is sutured back and replaced in scrotum. This kind of procedure done through inguinal route is called as Chevasu's procedure. II. Treatment of the retroperitoneum and metastasis (Key Box 42.7) 1. Seminoma a. Stage 1-IIA (Low stage): Radiotherapy to retroperitoneum (2500-3000 cGy) is the treatment of choice:

MHM.........,�

Seminoma testis

RETROPERITONEAL LYMPH NODE DISSECTION

Fig. 42.41: Testicular tumour with para-aortic lymph nodes and liver metastasis

.,........... • • • • •

P-HCG

�

Never found in normal persons Secreted by syncytiotrophoblasts Choriocarcinoma: 100% cases,Seminoma: 10% of cases Embryonal carcinoma: 65% of cases Increased levels after orchidectomy indicate recurrence, residual tumour. • Tumour marker of teratocarcinoma.

• It is recommended in all cases of teratoma including stage I. Also indicated in residual disease in lymph nodes in seminomatous tumours also. • All groups of lymph nodes draining the tumour such as precaval, paracaval, interaortocaval, retroaortic, para­ aortic, common iliac nodes are removed. • It also involves removal of gonadal vein and fibrofatty tissue around the vein from its origin near the internal ring to its insertion to renal vein on the left side and inferior vena cava on the right side. • The procedure is done on both sides. Haemorrhage, injury to ureter and bowel are other complications. • Retrograde ejaculation is one common problem after RPLND • Haemorrhage, bowel and ureter injury are common.

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• 5-year survival rate is around 95%. • Relapse after radiotherapy is managed by chemotherapy. b Seminoma stage IIB, III, IV • Radical orchidectomy + chemotherapy-PVB regimen­ cisplatin, vincristine, bleomycin. • BEP: Bleomycin, etoposide and cisplatin c. Treatment of residual disease in lymph nodes • In stage IIB and Ill, if there is residual lymph nodal mass 3 cm in size even after chemotherapy, then, a retroperi­ toneal lymph node dissection (RPLND) has to be done. • Stage IIB and Ill: Survival for 5 years-75% • Stage IV: Poor survival. II. Teratoma a. Stage I, IIA (Low stage) • Radical orchidectomy and RPLND 5-year survival-85% b. Stage IIB, III Radical orchidectomy and chemotherapy (PVB), post chemotherapy residual tumour in the retroperitoneum and if the tumour markers levels regress-retroperitoneal lymph node dissection should be done. 5-year survival rate is around 60%. c. Stage IV Orchidectomy + chemotherapy-poor prognosis. Comparison of seminoma with teratoma (Table 42.4) Other tumours are given below:

• Good prognosis because it behaves almost like a benign lesion b. Sertoli cell tumours • They are feminising tumours: Gynaecomastia, loss of libido, and aspermia are other features. • Increased oestrogen production is responsible for feminisation. • Postpubertal tumour • Orchidectomy is the treatment of choice. FOURNIER'S GANGRENE (IDIOPATHIC GANGRENE OF SCROTAL SKIN) Aetiopathology Even though Fournier's gangrene is called as idiopathic gangrene, certain factors precipitate the scrotal gangrene. 1. Low socio-economic group patients. 2. Unhygienic conditions Following perianal abscesses, urogenital instrumentation a scratch, cut or bruise in the scrotal skin (instrumentation, injury, infection-Key Box 42.8).

............................ KEY BOX 42.8

INTERSTITIAL CELL TUMOURS • • • a.

2 types are important clinically. Leydig cell tumour and Sertoli cell tumour. They should be treated like teratoma. Leydig cell tumour • It is prepubertal tumour • Causes masculinisation due to increased androgens­ infant Hercules • Spreads to lymph nodes and lungs

FOURNIER'$ GANGRENE

Trauma, cuts, scratch, infection Organism Gross inflammation Obliterative arteritis Scrotal gangrene

Comparison of seminoma with teratoma

I.

Cell of origin

2. Incidence

3. 4. 5. 6. 7. 8. 9. I 0.

Age group Shape of testis Surface Cut surface Consistency Spread Tumour markers Radiation response

Seminoma

Teratoma

Seminiferous tubules in the mediastinum of the testis 35-40% 30-40 years Retained Smooth Smooth Firm Mainly lymphatic

Totipotential cells in the rete testis 30% 20-30 years Not retained Irregular Variegated-nodules, cysts Firm or soft (cystic) Predominantly blood HCG-malignant teratoma Less sensitive

Excellent-melts like snow

.

Penis, Testis and Scrotum

Fig. 42.42: Fournier's g angrene ( Courtesy: Prof Santhosh Pai, Manipal)

999

Fig. 42.43: Fournier's gangrene precipitated by perianal abscess

Fig. 42.44: Perinea! phle g mon (Courtesy: Dr Ramachandra L, KMC, Manipal)

Causative organisms • Microaerophilic Haemolytic streptococci

• Staphylococci • E. coli

• Anaerobes: Clostridium welchii (Can be compared to Meleney's ulcer-sy nergistic gangrene-affecting abdominal wall skin. Today it is grouped under necrotising fasciitis). Clinical features I . Common in young apparently healthy individuals 2. Sudden appearance of scrotal inflammation-red, swollen, very painful. Patient is toxic with fever, prostration. 3. Within one/two days, extensive gangrene of the scrotal skin occurs resulting in sloughing of the scrotal skin exposing the testicles. In few cases, the gangrene can involve skin of the penis, anterior abdominal wall, medial side of thigh, perianal region. In such situations, it is described as perinea! phlegmon (Figs 42.42 to 42.44). 4. Luckily, the testis does not get involved in Fournier's gangrene because of thick tunica albuginea. Treatment 1. Broad spectrum antibiotics are started, once pus is sent for culture and sensitivity, e.g. • Metronidazole for anaerobes • Gentamicin for gram-negative organisms • Ampicillin for gram-positive organisms • Cephalosporins may have to be added if required. 2. Gangrenous portion of the scrotum has to be excised, as soon as possible which brings a dramatic reversal of general condition of the patient from toxic to near normal. 3. If the testicles are exposed, they can be implanted in the thigh or once the inflammation subsides, skin grafting is done to cover testicles.

Fig. 42.45: Healing with skin grafting ( Courtesy: Dr Ramachandra L, KMC, Manipal)

4. If penile skin is gangrenous, it is excised and covered with split skin graft later. Surprisingly, results are better than expected!! (Fig. 42.45) MISCELLANEOUS FRACTURE OF PENIS It is a misnomer • It is the traumatic rupture of corpora cavernosum. It is considered a urologic emergency. • Sudden blunt trauma or abrupt lateral bending of penis in an erect state can break the markedly thinned and stifftunica albuginea.

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Manipal Manual of Surgery

• One or both corpora may be involved and concomitant urethral (38%) injury if both corpora involved. • Causes: Sexual activity, masturbation, gun shot wounds, industrial accidents, mechanical trauma. • Diagnosed clinically. • In equivocal cases - cavemosonography or MRI. • Preoperative retrograde urethrography urethral injury is suspected. Treatment a. Medical: Fluids, antibiotics. If surgical therapy is delayed due to urethral injury, initial medical therapy consists of cold compresses, pressure dress­ ings, anti-inflammatory medications and suprapublic cystostomy. b. Surgical: Evacuate haematoma, identify site of injury, correct the defect in tunica albuginea and repair urethral injury urethral catheter removed after 2 weeks. Complications Erectile dysfunction, abnormal curvature, painful erections, urethrocutaneous fistula, corpora-urethral fistula and painful nodules. PRIAPISM • Priapism is a pathologic condition of a penile erection that persists beyond or is unrelated to sexual stimulation. • It can occur in all age groups including the newborn but peak incidence: 5-10 years 20-50 years. Causes 1. Low flow: Sickle cell trait/disease, chronic myeloid leaukemia, total parenteral nutrition, medications (sildenafil, cocaine), malignant penile infiltration, spinal cord injury, spinal anaesthesia or general anaesthesia. 2. High flow: Perinea! or penile trauma.

Two types Type I: Low flow priapism (veno-occlusive) Decreased venous outflow Increased intracavemosal pressure Painful, fully erect penis Local hypoxia and acidosis It is the most common variety Type II: High flow priapism High inflow and high outflow Penis is erect but nontender Corporal blood gas analysis can differentiate low flow and high flow priapsim. Treatment 1. Low flow priapism: Corporal irrigation with normal saline and cx-adrenergic intracorporal injections ( epinephrine/ phenylephrine/ephedrine) every 5 min till detumescence. In severe cases-shunts ( corporoglandular), corporo­ spongiosal or corporosaphenous) may be necessary. If patient has sickle cell disease, IV bicarbonate and blood transfusions required. 2. High flow priapism: Doppler ultrasound done to identify arterial-lacunar fistula. Arterial embolisation or open surgical arterial ligation done. INTERESTING 'MOST COMMON' FOR TESTICULAR CANCER • • • • •

Most testicular tumours are malignant Most common cancer in young adults is testicular cancer Most common solid cancer in young man is seminoma Most common tumour is seminoma Most common teratoma is malignant teratoma intermediate: Teratocarcinoma WHAT IS NEW IN THIS CHAPTER?/ RECENT ADVANCES

• All the topics have been updated • New photographs and key boxes have been added

MULTIPLE CHOICE QUESTIONS 1. Following are true for surgical anatomy of the penis except: A. Corpora cavemosa are vascular spaces B. Arterioles are corkscrew shaped C. Attached to symphysis pubis by suspensory ligament D. Deep artery supplies corpus spongiosum alone 2. Balanitis refer to: A. Inflammation of the glans penis 8. Inflammation of the prepuce C. Inflammation of glands in the fossa navicularis D. Inflammation of the urethral glands

3. Dorsal slit is given for which condition? A. Phimosis 8. Paraphimosis C. Carcinoma penis D. 8alanitis 4. Following are complications of phimosis except: A. Paraphimosis 8. Carcinoma penis C. Balanoposthitis D. 8uschke-Lowenstein tumour

Penis, Testis and Scrotum

5. Which of the following is rare feature of carcinoma penis? A. Foul smelling discharge B. Recent phimosis C. Urethral involvement D. Induration is extensive 6. Treatment of carcinoma penis confined to prepuce is: A. Circumcision B. Dorsal slit and excision C. Partial amputation D. Radiotherapy 7. Following are true for partial amputation of penis except: A. Partial amputation is the treatment for growth confined to glans penis B. At least 2 cm proximal shaft is necessary in partial amputation C. Long ventral flap is required to cover D. Perineal urethrostomy may be required 8. Few important consideration sin total amputation of the penis include following except: A. Suspensory ligaments have to be divided B. Perinea! urethrostomy has to be done C. Severe excoriation of the scrotum is a complication D. Advantage being no stricture urethra after surgery 9. Following are the features syphilitic chancre of the penis except: A. Single ulcer B. Hard chancre C. Firm painful lymph nodes in the axilla D. Contagious 10. Testosterone is produced by? B. Sertoli cells A. Germ cells D. Clear cells C. Leydig cells 11. Testicular artery is a branch of: A. Aorta B. Common iliac C. Internal iliac D. Early morning urine sample is the best to measure this 12. Following are true for prostate specific antigen except: A. Released from columnar prostatic acinar epithelial cells B. More than 4 nmol/ml suggest carcinoma prostate C. Prostatitis also can increase in the levels D. It does not help in assessing the response to treatment

1001

13. Which of the following test differentiate encystec hydrocoele from spermatocoele? A. Fluctuation B. Getting above the swelling C. Impulse on cough D. Traction test 14. What is the classical clinical feature of tuberculou: epididymo-orchitis? A. Thickened vas B. Atrophy of the testis C. Craggy epididymis D. Anterior sinus 15. Chinese lantern type of transillumination is classica of: A. Spermatocoele B. Epididymal cyst C. Ranula D. Cystic hygroma 16. Which of the following is true in cases of retractih testis? A. It is premalignant B. Testis is generally not palpable C. Scrotum is well developed D. Testis cannot be brought down into scrotum 17. In torsion testis following are true except: A. Scrotum is empty B. Tender lump at external abdominal ring C. Elevation of scrotum increases pain D. Upper abdominal pain and vomiting are features 18. Which one of the following is more prone for testicular tumour? A. Gardner's syndrome B. Down's syndrome C. Klinefelter's syndrome D. Sipple syndrome 19. Human chorionic gonadotrophin elevation is diagnostic of ................. A. Choriocarcinoma B. Seminoma C. Leydig cell tumour D. Sertoli cell tumour 20. Following are true for increased LDH levels in tumours: A. It suggests liver metastasis B. It suggests germ cell tumours C. lt suggests malignant melanoma D. It suggests tumour load

ANSWERS 1 D

2 A

3 B

4 D

5 C

11 A

12 D

13 D

14 C

15 B

6A 16 C

7 D

8 D

9 C

17 D

10 C

18 C

19 A

20 B

43 Differential Diagnosis of Haematuria • Causes • History and examination • Investigations

• Haematuria • History • What is new?/Recent advances

CAUSES OF HAEMATURIA (Fig. 43.1 and Key Box 43.1) I. In the kidney 1. Infection • Acute glomerulonephritis • Tuberculosis 2. Infarction • SBE with emboli causing renal infarction • Massive haemolysis with acute renal tubular necrosis • Mismatched blood transfusion 3. Injury • Stab/blunt injury 4. Tumours • Wilms' tumour: Nephroblastoma • Hypemephroma: Renal cell carcinoma (RCC) • Transitional cell carcinoma (TCC) 5. Stones 6. Polycystic kidney

Ill. In the urinary bladder

I. 2. 3. 4. 5. 6. 7.

Carcinoma of bladder Carcinoma prostate Cystitis Tuberculosis Bilharziasis Stone: Common in school-going children Benign prostatic hyperplasia (BPH)

IV. Urethra 1. Stone

MmhWIIIIIIIIIF,1 HAEMATURIA Common causes • Urolithiasis • Tumours Uncommon causes • Tuberculosis • Cystitis, bladder tumours • Polycystic kidney

II. In the ureter 1. Stone 2. Cancer-rare

V. Rare causes 1. Patients on anticoagulants 2. Sickle cell anaemia 3. Bleeding disorders HIS TORY AND EXAMINATION

�

1. Age and sex Young children Young adults Elderly patients 2. Occupation Aniline dye workers 1002

Vesical calculus Renal stones, tuberculosis (TB) Renal cell carcinoma (RCC) Carcinoma bladder

Differential Diagnosis of Haematuria

1003

Glomerulonephritis -------=--"-' Stone---_J Infarction----

Fig. 43.1 : Various causes of haematu ria

3. Haematuria • Bright red • Altered blood • Profuse • Small quantity

• • • •

• • • • •

• • • • •

Beginning of micturition End of micturition Mixed with urine Painless haematuria Painful haematuria

Lower urinary tract Kidney Papilloma Renal cell carcinoma, TB, stone Urethral pathology Bladder pathology Renal Papilloma or carcinoma Renal stone, bladder stone

4. General physical examination • Gross pallor • Significant blood loss • Gross pallor with minimal • Renal cell carcinoma blood loss • Hypertension • Polycystic kidney • Bony pains • Carcinoma (prostate) 5. Abdominal examination • Palpable kidney • Polycystic kidney, Wilms' tumour, renal cell carcinoma • Carcinoma prostate, • Distended bladder enlarged prostate • Bladder stone, cystitis • Suprapubic tenderness • Craggy epididymis • Genitourinary tuberculosis and beaded vas

6. Rectal examination • Enlarged smooth prostate • Hard irregular prostate • Hard, thickened seminal vesicles • Advanced growth

• BPH • Carcinoma prostate • Genitourinary tuberculosis • Carcinoma rectum infiltrating urinary bladder

INVESTIGATIONS (Table 43.1 and Fig. 43.2) 1. Urine examination • Worm-like clots • Flat disc-like • Pieces of tumour

• Growth in the ureter • Urethra • Papilloma of the bladder

2. Urine microscopy • Pus cells • Abacterial acid pyuria • Malignant cells positive

• Urinary tract infection • TB • RCC or papilloma bladder

3. Plain X-ray KUB • Enlarged kidney • Radio-opaque shadows

• Polycystic kidney, renal cell carcinoma • Renal stones, ureteric stones, bladder stone

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4. Cystoscopy • Growth in the bladder • Inflammation of the bladder • Ulcers, hyperaemia, golf-hole ureter 5. • • •

Papilloma bladder/TCC Cystitis TB

Intravenous urography Spider leg calyces Irregular calyces Missing calyces

6. Ultrasound • Enlarged kidney

PEARLS OF

Polycystic kidney R CC TB R enal cell carcinoma Polycystic kidney Wilms' tumour Stones

WISDOM

Differential diagnosis of haematuria is a major theory question in an undergraduate examination. I have given some ideas about h ow to eval uate these cases by analysing history, physical examination and investiga­ tions. This gives a good exercise to the students.

HAEMATURIA Haematuria is defined as passage of blood mixed with urine. It can be classified as 1. Microscopic 2. Macroscopic

Also 1. Glomerular 2. Nonglomerular (urological-Table 43.2). Macroscopic or gross haematuria Urine may vary in colour from red to stale brown or chocolate coloured. May be associated with clots. The nature of clots may give a clue to the source or site of bleeding. Serpiginous or vermiform clots indicate bleeding from the kidneys. Amorphous clots are suggestive of lower tract bleeding. Most patients with gross haematuria have some pathology in the kidneys or the genitourinary tract. It can be initial, terminal or throughout the stream. Urethral bleeding usually manifests as the first 10-15 ml of blood-stained urine, which gradually clears. Terminal haematuria usually indicates trigonal irritation (e.g. bladder stone). It is usually associated with dysuria and strangury. Bleeding from the upper urinary tract (kidneys and ureters) is seen throughout the stream as the blood mixes with the urine stored in the bladder. Microscopic haematuria It is defined as the presence of more than 3 RBCs per high power field on microscopic examination. It is usually detected incidentally on urine dipstick testing and subsequent microscopic examination. Nonnal RBC excretion is up to 2 million RBCs/day. Aetiology Evaluation: Numerous investigative modalities are available for evaluation of haematuria. The choice of the correct modality depends on the clinical suspicion and important clues on clinical examination.

Utility of various investigative modalities for evaluation of haematuria Investigations

Advantages

Disadvantages

Intravenous urography (IVU)

Evaluation of kidney collecting system and ureter for stones, masses and obstruction

Poor assessment of bladder pathology Nephrotoxicity

Cystoscopy

Investigation of choice for bladder and urethral assessment

Invasive Significant patient discomfort Expensive equipment required Potential for complications (e.g. urethral stricture)

Urinary cytology

Diagnosis of transitional cell carcinoma (TCC) Grading of TCC

Not useful for squamous or adenocarcinoma

CT scan

Investigation of choice for colic (noncontrast CT scan) Cost Radiation Best investigation for renal masses Contrast nephrotoxicity Better than IVU in most situations Other organs affecting the urinary tract can also be imaged (e.g. carcinoma cervix causing ureteric obstruction) Invasive Aneurysms Not widely available (skilled) A-V fistulas/malformations High cost Postoperative bleeding for localisation and angioembolisation

Angiography

1005

Differential Diagnosis of Haematuria

Initial evaluation includes Ultrasonography: It is noninvasive and less expensive • Diagnosis of upper or lower urinary tract lesions a. Stones b. Tumours c. Hydronephrosis

• Renal parenchymal changes suggest glomerular cause. • Ultrasound guides towards choosing further investigations • Less sensitive for evaluation ofureteral causes ofhaema turia.

Distinguishing between glomerular and urological causes of haematuria Glomerular

Nonglomerular

Colour (if macroscopic)

Pale red, smoky brown or stale tea

Red or pink

Clots

Rare

More common

Proteinuria (dipstick/mg per day)

3+ or more (> 500 mg/day)

< 2+ (< 500 mg/day)

RBC morphology

Dysmorphic RBCs

RBC casts

May be present

Normal Absent

HAEMATURIA

Microscopic haematuria > 3 RBCs/hpf

Gross

• lgA nephropathy • Drug-induced • Infections

Rule out benign causes (exercise, sexual activity, menstrual contamination)

History and physical examination Complete urinalysis(physical characteristics, protein, dysmorphic RBCs, pus cells) RFT

Dysmorphic RBCs(-ve) Pus cells(+)

Dysmorphic RBCs(-ve) Pus cells(-ve)

S/o glomerular disease

Urological causes

Look for proteinuria, RFT

+ve(UTI) Antibiotics

Dysmorphic RBCs(+ve) ± pus cells

Sterile pyuria •TB • Stones • Malignancy

USG/IVU/CT + Urine cytology

Renal biopsy Treat the cause

Cystoscopy

Fig. 43.2: Haematuria-management algorithm

WHAT IS NEW IN THI CHAPTER?/RECENT ADVANCES

• Haematuria has been discussed with greater detail.

i!'!

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Manipal Manual of Surgery

MULTIPLE CHOICE QUESTIONS 1. Following is the cause of haematuria in bacterial endocarditis: B. Renal sepsis A. Renal necrosis D. Coagulopathy C. Renal infarction 2. Haematuria in polycystic kidney is due to: A. Hypertension B. Renal infarction D. Cyst rupture into renal pelvis C. Renal ischaemia 3. Which of the following conditions rarely gives rise to haematuria? A. Renal stones B. Renal cell carcinoma C. Bilharziasis D. Benign prostatic hypertrophy

4. Palpable renal masses in 40-year-old hypertensivE patient with one attack of haematuria is: A. Hypernephroma B. Hydronephrosis C. Adenomyolipoma D. Polycystic kidney 5. Which of the following pathologies is reflected as terminal haematuria? A. Trigonal irritation B. Posterior urethral irritation C. Ureteric irritation D. Renal irritation

ANSWERS

1 A

2 D

3 D

4 D

5 A

Specialities 44. 4S. 46. 47. 48. 49.

Chest Trauma, Cardiothoracic Surgery Neurosurgery Principles of Radiology Principles of Clinical Radiation Oncology Principles of Anaesthesiology Organ Transplantation

44

Chest Trauma, Cardiothoracic Surgery • • • • • • • •

Chest trauma Blunt trauma Pulmonary injuries Tracheobronchial injuries Myocardial contusion Surgical emphysema Mediastinal emphysema Mediastinal masses

CHEST TRAUMA Introduction In chest trauma, the mortality is very high unless promptly recognised and properly treated. The margin of safety is very slim initial care dictates the final result. With varying degree of s:verity, chest injuries occur in almost 80% of road traffic accidents (Key Box 44.1 ). MAIN AIMS OF RESUSCITATION The standard method ofresuscitation in all cases ofpolytrauma is as follows: A. Airway • Aspiration ofblood and secretions from oral cavity, pharynx and trachea • Introduce plastic airway • Endotracheal intubation

ll=tMtWIIIW'� • • • • •

COMMON CAUSES

Automobile accidents Gunshot wounds Stab injuries Blast injuries Crush injuries

• • • • • • • •

Pulmonary aspergilloma Congenital heart diseases Patent ductus arteriosus Coarctation of aorta Coronary artery bypass graft Off pump coronary artery bypass surgery Abdominal aortic aneurysms (AAA) What is new?/Recent advances

• Cricothyroidotomy as necessary • Tracheostomy as necessary B. Breathing • Intubation • Intercostal chest tube (ICT) • Closure of any open chest wounds These two steps (A and B) help in re-aerating the lung C. Circulation • Control of major and life-threatening bleeding • Volume infusion D. Disability • Neurological E. Exposure • All clothing to be cut open without moving the patient

All the above steps are taken by the trauma centre team simultaneously and not one by one. • Relieve pain. Do not sedate • All open wounds of the chest to be covered • Life-threatening injuries should be identified and treated immediately. ASSESSMENT OF INJURY

History • Time since the injury • Details of the injury from the bystander or the police

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• High-speed deceleration injury (aortic and cardiac rupture to be ruled out). • Crushing accidents (tracheobronchial and oesophageal tear). • Sudden abdominal compression (ruptured diaphragm). • In stab injuries, length of the knife and direction of stab. Examination • The clothing should be removed carefully without moving the patient. • Palpate for clinical evidence of fracture ribs, surgical emphysema, any paradoxical movement of the ribs and auscultate for air entry in both lungs. • Even if it is a trivial injury, the patient should be admitted and observed for a minimum of 24 hours before discharge. • It is reasonable to do unilateral or bilateral closed tube thoracostomy (ICT) (Fig. 44.1) on suspicion of haemothorax or pneumothorax when the patient is in respiratory distress.

Fig. 44.1: Fracture ribs with haemothorax left side-intercostal tube has been inserted

BLUNT TRAUMA TOTHE CHEST Causes • Road traffic accidents • Fall from a height • Crush injuries • Assault with blunt object SIMPLE RIB FRACTURE Rib fracture can be single or multiple (Key Box 44.2). Single rib fracture • Often regarded as a trivial injury but should be treated with respect in elderly patients. • Occurs due to direct injury or excessive flexion. • The common site is at the costal angle or middle of the shaft. • Patients will have pain on breathing, coughing and on palpation.

lltM�� POINTS TO REMEMBER • First rib fracture is a marker of severe trauma-injury to brachia! plexus, subclavian artery and vein • Displaced fracture of 8th-10th ribs - Injury to liver and spleen • Fracture of 11th and 12th ribs-injury to kidney • Penetrating injury to left lower chest wall-injury to heart, lung, diaphragm, stomach and spleen

• They are treated with analgesics, intercostal nerve block and assurance. Multiple rib fractures • When there are multiple rib fractures without any pneumo­ or haemothorax and no other organs are involved, intercostal nerve block and small amount of narcotics are required. • Strapping is occasionally necessary in young adults. • In elderly patients, consider hospitalisation for observation, pain control and pulmonary toilet. • Chest X-ray to be repeated after 24 hour and at the time of discharge, to rule out late onset pneumo- and haemothorax. • Intermittent use of velcro belt rib support. • Inform the patient of deep breathing and coughing using the rib belt. • Epidural analgesia is becoming the standard of care for pain management in patients with multiple rib fractures. STERNAL FRACTURE • Commonly due to steering wheel injury-blunt trauma. • Usually occurs at the sternal angle. • Associated with costochondral dislocations. • Classified as displaced and nondisplaced fractures. • Localised swelling, tenderness and deformity are the clinical findings. Treatment • Displaced fracture-requires surgical fixation. • Nondisplaced fracture-conservative management. FLAIL CHEST This results from severe chest injuries with multiple rib fractures. Here there are fracture of four or more ribs at two places, anteriorly and posteriorly, so that certain segments of ribs will have no attachment to the chest wall. These ribs become indrawn due to intrathoracic negative pressure as the patient inhales and is driven outwards on expiration producing instability. This is called paradoxical respiration. It results in hypoventilation, carbon dioxide retention and respiratory failure. Flail chest is of three types: Anterior, posterior and lateral.

1011

Chest Trauma, Cardiothoracic Surgery

Anterior flail • Fracture of the costochondral junction on both side of the sternum Posterior flail • Fracture ribs of posterior chest wall Lateral flail • Fracture shaft of the ribs Treatment I. Commonly done procedures 1. Anterior flail: Seagull-shaped prosthesis introduced to stabilise the flail segments. 2. Posterior flail: No treatment is required as the scapula acts as a support to stabilise the flail segment. 3. Lateral flail: It is treated by chest wall stabilisation, reduction of the respiratory dead space, management of the pulmonary contusion and pain control. Epidural analgesia is recommended for pain management. Intercostal nerve blocks may also be used. II. Other methods • Surgical stabilisation is rarely indicated, i.e. open reduction of rib fracture or osteofixation. • Recent method is to intubate and stabilise the flail segments with positive pressure ventilation, which has to be done for at least one week. This is called internal pneumatic fixation. • Physiologic stabilisation with intubation and IPPV must be initiated before hypoxia develops. IPPV produces satisfactory ventilation and helps the fractured ribs to unite in the position of inspiration, thereby reducing the deformity and improving the pulmonary function. 'STOVE IN' CHEST • Localised, severe, blunt or crush injury produces depression of a portion of the chest. • Treatment is same as that for flail chest. Sometimes, thoracotomy may be needed if there are internal injuries. PULMONARY INJURIES CONTUSION OF THE LUNG • Deceleration injuries or crush trauma often produces extensive parenchymal damage. Haemorrhage and interstitial oedema result in obliteration of alveolar spaces and consolidation of large areas of pulmonary tissue. • Contusion of the lung can be unilateral or bilateral. The contusion can be in the form of a small area of damage with oedema and extravasation of the blood, or it may be widespread damage. Haemoptysis and excessive tracheobronchial secretions give the clue to the diagnosis (Key Box 44.3).

Mtm1--..r� PULMONARY INJURIES

• • • •

Contusion Pneumothorax/haemothorax Laceration Chest wall injuries

• Chest X-ray: Early patchy consolidation. It must b1 differentiated from adult respiratory distress syndrome (ARDS). • CT scan is more specific (Fig. 44.2).

Fig. 44.2: CT scan showing rib fracture and lung injury

Treatment • Fluid restriction, pulmonary care • Chest physiotherapy • Steroids and rarely ventilation Usually self-limiting, if there are no other severe injuries. Complications • Pneumonia • Atelectasis • Respiratory failure • ARDS PNEUMOTHORAX • Pneumothorax is the most common cause of respiratory insufficiency following chest trauma. • Usually if there is a rib fracture and evidence of subcutaneous emphysema, pneumothorax is certainly present (Fig. 44.3). • Pneumothorax can be closed (simple), open and tension. • Small simple pneumothorax does not need any treatment. • A repeat chest X-ray after 12-24 hours is essential to confirm that it is not progressing.

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............................. . KEY BOX 44.4

TENSION PNEUMOTHORAX: CLINICAL FEATURES Tachypnoea Tachycardia Tympanic note on percussion Total absence of breath sounds Tracheal shift Observe 5 Ts

.

............................ .

KEY BOX 44.5

TENSION PNEUMOTHORAX Injury to the lung Valvular air leak

Fig. 44.3: Pneumothorax right lung

Lung collapse Mediastinal shift to opposite side Increased intrapleural pressure Decreased venous return Decreased ventilation Hypoxia and cardiac arrest

............................. KEY BOX 44.6

.

TENSION PNEUMOTHORAX

Fig. 44.4: Large bullae

• • • • •

It can be confused for a large bullae (Fig. 44.4). Small pneumothorax can be missed easily. Bilateral pneumothorax is an emergency. Late pneumothorax can also occur. Open chest wound will produce complete collapse of lung and paradoxical shift of the mediastinum with each respiration (mediastinal flutter) causing hypoventilation and reduced cardiac output. • Treatment is by closure of the wound, intercostal tube drainage (ICD) and surgery.

Tension pneumothorax (Key Boxes 44.4 to 44.6) • Injury to the lung results in continuous valvular air leak. The accumulating air collapses the lung on the same side and pushes the mediastinum to opposite side. As a result of

• Diagnosis immediately • Do not wait for CXR • Thoracocentesis in 2nd intercostal space, midclavicular line. • Then, follow with intercostal chest tube insertion.

this tension, the intrapleural pressure increases, till it is above atmospheric pressure at the time of expiration. This reduces the venous return to the heart as well as compromises the ventilation. • Tension pneumothorax is an emergency which should be treated urgently with needle thoracocentesis in the second intercostal space in the midclavicular line to release the tension. Thoracocentesis converts tension pneumothorax to simple pneumothorax. • This should be followed by urgent ICD insertion and connected to underwater seal. • Do not wait for chest X-ray.

Chest Trauma, Cardiothoracic Surgery

Tension gastrothorax This occurs due to herniation of dilated and obstructed stomach into the mediastinum due to diaphragmatic tear resulting in haemodynamic compromise. It should be treated by reducing the hernial contents and repair of diaphragmatic tear. HAEMOTHORAX • May be missed in chest X-rays in the supine position. • Results from injury to internal mammary artery, intercostal artery and vascular lung adhesions. • Classical signs are reduced-chest expansion, dullness to percussion and absent breath sounds on affected side. • Treated by intercostal chest tube insertion (Figs 44.5 and 44.6). • If bleeding continues or features of shock develop, thoracotomy has to be considered. • The bleeding may be delayed or may recur after several days.

Fig. 44.5: Right haemothorax

1013

Indications for thoracotomy • Initial volume of blood loss is not as important as the amount of ongoing bleeding. • Drainage is more than 1000 ml or 100 ml each hour for 4 hours. • If clotted haemothorax is suspected (opacity persisting on chest X-ray even after ICD). Complications Failure to adequately drain a haemothorax initially results in residual clotted haemothorax and empyema or late fibro­ thorax. INTERCOSTAL CHEST TUBE (ICT) INSERTION (CLOSED TUBE THORACOSTOMY) (Flowchart 44.1) • Second intercostal space-anteriorly midclavicular line is ideal for pneumothorax and sixth space in the midaxillary line for haemothorax. • Triangle of safety - Above the level of nipples - Anterior to midaxillary line - Below and lateral to pectoralis major muscle • Can be introduced from sixth intercostal space midaxillary line for pneumothorax also but the chest tube should reach the apex of the lung (Figs 44.7 and 44.8). • Infiltrate local anaesthetics up to the parietal pleura • 2-3 cm incision parallel to the ribs-deepened, suture taken • Insert the chest tube with trocar into the pleural cavity • Then the trocar is removed and the chest tube is connected to underwater seal. The ICT is fixed • Intercostal drainage can also be done by connecting to 2 bottles (Fig. 44.9) • Removal ofICT (Key Box 44.7) For ten commandments ofI CT-see the next page.

---------Fig. 44.6: Bilateral haemothorax-detected early and treated with ICT on the right

Fig. 44.7: lntercostal tube (JCT) connected to underwater seal

Fig. 44.8: Underwater seal (diagrammatic)

Manipal Manual of Surgery

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TEN COMMANDMENTS OF PRECAUTIONS WHILE USING ICT-ICD 1. Should select triangle of safety 2. Should direct the tube towards apex in cases of haemothorax and pneumothorax 3. Should direct the tube towards base in empyema 4. Should confirm that all the holes are inside 5. Should be connected to under water seal 6. Should observe for movement of the fluid column with respiration 7. Should take an X-ray after the insertion of the tube 8. Should avoid kinking of the tube 9. Should not clamp the ICD if there is air leak 10. Should be always lower than patient level in the supine position. Otherwise, contents of the bottle will enter the pleural space

Flowchart 44.1: Important steps of intercostal tube insertion Position of the patient-supine with head end up Select the triangle of safety Infiltrate local anaesthesia Sharp dissection only to cut the skin Blunt dissection with finger and artery forceps till you reach pleura (serratus and intercostal muscle fibres are separated) Introduce the intercostal tube obliquely-make a short tunnel

Retain the drain with a good suture and vertical mattress suture for wound closure

............................. . KEY BOX 44.7

!CT-REMOVAL • Chest X-ray-lung fully expanded and drainage should be less than 100 ml for two days and no air leak. Patient should not be on a ventilator • Clamp the tube for 24 hours • Removal after 24 hours of clamping, provided the patient is comfortable and lung remains fully expanded.

Fig. 44.9: ICD with 2 bottles

LUNG LACERATION • Minor laceration: Haemopneumothorax. Usually inter costal chest tube is enough. • Major laceration: Haemopneumothorax 1. Introduce intercostal chest tube 11. Continuous air leak or bleeding through JCT and lun� not expanding. Requires thoracotomy and repair 01 resection of lobe. SHOCK LUNG Definition • Alveolar collapse due to shock as a result of oedema, impaired perfusion, and reduction in alveolar space resulting in respiratory failure is called shock lung. • It is also called Acute Respiratory Distress Syndrome (ARDS). Causes • Major chest trauma with multiple rib fractures and lung contusion • Septic shock and septicaemia • Disseminated intravascular coagulation • Massive blood transfusion • Major bums • Cardiopulmonary bypass (also called 'Pump lung') • Acute pancreatitis • Aspiration of gastric contents Pathogenesis • Diffuse inflammation of the lung • Extensive intravascular coagulation due to m1cro­ thromboembolism • Focal disorders of the circulation, primarily because of sluggish microcirculation due to leukostasis, sludge, leading to extensive hyaline thromboses • Increased capillary penneability due to damaged capillaries • Diffuse alveolar damage, decreased production of surfactant by type II pneumocytes leading to bilateral extensive fine atelectasis • Net result is pulmonary consolidation, decrease in the lung compliance, poor gas exchange leading to stiff lung. Investigations • Arterial blood gas analysis • Chest X-ray • CT scan Treatment • Ventilatory support (intermittent positive pressure ventilation) • Antibiotics-broad spectrum • Low to moderate doses of steroids may help in early ARDS, in patients requiring high doses of vasopressors to maintain blood pressure.

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Summary of blunt chest injury Tracheobronchial

Diaphragm

Contusion

Mediastinal emphysema

Breathlessness

Pneumothorax

Shock

Coils of bowel within thorax

Subclavian injury

Haemothorax

Airway obstruction

Surgical emphysema

Laceration

Death

Rib fractures

Pulmonary injuries

Flail chest Stove-in chest

• Intensive care-supportive therapy can be remembered as FASTHUG F-Feeding (usually enteral) A-Analgesia S-Sedation once a day and check neurological status T-Thromboembolism prophylaxis H-Head-end elevation (20-30°) U-Ulcer (gastric) prophylaxis, and G--Glucose (blood) control INJURY TO TRACHEA AND MAJOR BRONCHI TRACHEAL INJURIES Injuries occur as a result of trauma sustained due to crush injuries. Common in cervical trachea.

INJURY TO THE DIAPHRAGM Features • Due to crush injury • Industrial-due to fall of heavy weights over the abdomen. • Commonly, left diaphragm tear occurs. • Missed in chest X-ray in the supine position. • Diagnosed by CECT of the abdomen or chest X-ray reveals the Ryle's tube in the chest or coils of bowel (gas shadow) in the chest and elevated fundic air bubble (Figs 44.10 to 44.12). Diagnostic laparoscopy is also a good investigation in cases of rupture diaphragm. • When in doubt, do diagnostic laparotomy.

Symptoms • Acute airway obstruction • Emphysema-mediastinal and cervical • Pneumothorax voice impairment Treatment Intubation, tracheostomy, surgery and repair MAJOR BRONCHIAL INJURIES • Blunt trauma produces stereotype injury to main bronchus of either side. Lobar bronchi are less commonly injured (Table 44.1). • Lesion is often a circumferential laceration with complete separation or a partial tear. Symptoms • Pneumothorax, uncontrolled air leak • Haemoptysis • Surgical emphysema • Bronchoscopy for confirmation Surgery • Endobronchial intubation • Bronchoplasty, injury to the lobar bronchus-resection of the lobe of the lung.

Fig. 44.10: Chest X-ray left haemothorax with elevated fundic air bubble

INJURY TO THE AORTA (RUPTURE OF THE AORTA) • • • •

Deceleration injuries Gets avulsed at the region of ligamentum arteriosus Complete rupture-immediate death Incomplete rupture-shock, widening of mediastinum, unequal pulses.

Surgery • Repair is done using cardiopulmonary bypass.

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Manipal Manual of Surgery PEARLS OF

WISDOM

Palpable crepitus is diagnostic of surgical emphysema.

X-ray chest may show pneumothorax or air in the sub­ cutaneous plane (Fig. 44.14). Treatment • Localised: Not extending, no pneumothorax-observatior • Pneumothorax-ICT • Extensive: ICT to be introduced on the side where it i� maximum. • ICT may be required on both sides • Two ICT, one apical and the other, basal, may need to be introduced if there is continuous air leak.

Fig. 44.11: Fundic air bubble in the thorax

Fig. 44.13: Surgical emphysema

Fig. 44.12: ICT in the stomach

MYOCARDIAL CONTUSION • Deceleration injuries • Anterior chest impact • Clinical feature include arrhythmias, reduced cardiac output, cardiac tamponade. SURGICAL EMPHYSEMA Types 1. Localised 2. Extensive from the eyelids to the scrotum, quite alam1ing in appearance. Indicates lung injury (Fig. 44.13).

Fig. 44.14: Chest X-ray showing 10th rib fracture with surgical emphysema on the right side

Chest Trauma, Cardiothoracic Surgery

............................. .

MEDIASTINAL EMPHYSEMA

KEY BOX 44.8

• • • • • •

SURGICAL EMPHYSEMA

Air in the subcutaneous tissue Palpable crepitus Sometimes, can be gross Disfigurement is more than symptoms and signs Rib stabbing the lung Rupture of bronchus-mediastinal emphysema

• The emphysema usually subsides within a week. Multiple incisions and expelling the air from the subcutaneous space manually are not required but may be carried out for cosmetic purposes or if patient has respiratory distress (Key Box 44.8). PENETRATING THORACIC WOUNDS Stab wounds They depend on type of weapon, length and direction of the stab (Key Box 44.9). Projectile wounds • Relatively low velocity revolver bullets may perforate one or two lobes of the lung with a little damage and only drainage of the pleural space is required. High velocity perforating wounds cause more damage to the tissue, adjacent to the tract. If the damage is extensive, lobectomy or pneumonectomy has to be considered. • Bomb fragments, because of irregular shape, commonly carry with them pieces of ribs and are associated not only with severe haemorrhage and air leak from the torn pulmonary vessels and bronchus but also with haemorrhage from irregular entry and exit wounds. Urgent thoracotomy is necessary. Treatment Emergency thoracotomy is a life-saving procedure in the trauma centre. Thoracotomy for polytrauma has a poor prognosis than for isolated thoracic injuries. Indications • Decompression of cardiac tamponade • Control of bleeding, allow for internal cardiac massage • Clamping of descending thoracic aorta for exsanguinous bleeding in the abdomen. Surgery Anterolateral thoracotomy through 5th intercostal space, entered in 1-2 minutes.

............................. . KEY BOX 44.9

PENETRATING WOUNDS

• • • •

Represents mainly stab and gunshot wounds Immediately pack the wound lntercostal chest tube Thoracotomy mandatory

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• The emphysema is mainly suprasternal. • On auscultation, p ericardia! crunching soundi synchronous with the heart beat, are heard. • If there is haemodynamic instability, bilateral intrapleura JCT has to be introduced as a precaution against tensior pneumothorax. • Rule out oesophageal and tracheal injury. Principles of managing chest injuries 1. Pulmonary physiotherapy: Most important in all ches1 injury patients. 2. Aspiration of secretions: Tracheal aspiration, nasotracheal suction, aspiration of oral cavity and pharynx. 3. Relieving pain: Oral narcotics, parenteral narcotics, thoracic epidural analgesia, intercostal nerve block. 4. Physiotherapy assistance • Encourage coughing • Chest percussion and vibration • Deep inspiratory efforts • Humidification of air, nebulisation • Early mobilisation 5. Treatment of pneumothorax or haemothorax: Insertion of intercostal chest tube. 6. Treatment of shock: First of all, the causes of shock in chest injuries has to be determined by thorough assessment of the patient. Once the cause is found out, depending upon the nature of the problem, the patient is treated in an intensive care unit (Key Box 44.10).

............................. . KEY BOX 44.10

CAUSES OF SHOCK

• • • • • • •

Tension pneumothorax Massive haemothorax Cardiac tamponade Myocardial contusion Air embolism Ruptured diaphragm Injury to the great vessels

7. FAST (focussed abdominal sonography in trauma) ultrasound: Focussed assessment with ultrasonography for trauma and rapid assessment for ruling out fluid collection in the abdomen, chest and pericardium. 8. Surgery: Depending upon the severity and location, surgery is done. 9. Treatment of complications (Key Box 44.11) • Thromboembolism • Tracheostomy complications • Prolonged ICU complications.

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.

............................ .

KEY BOX 44.11

COMPLICATIONS • • • • • • •

• CT scan may show split pleura sign. Tuberculous spine can be diagnosed as a cause of empyema (Figs 44.17 to 44.18)

Empyema Bronchopleural fistula Bronchial stenosis Chylothorax Clotted haemothorax ARDS Atelectasis

Nontraumatic rib fractures (Key Box 44.12)

............................. . KEY BOX 44.12

NONTRAUMATIC RIB FRACTURES • • • • • •

Stress fractures Metastatic disease Metabolic disease such as hyperparathyroidism Osteogenesis imperfecta Consider child abuse, 'shaken baby' syndrome Older patients after violent coughing

EMPYEMA

Fig. 44.15: Effusion

Definition

Collection of the pus in the pleural space is called empyema. Aetiopathogenesis

• It is the end stage result of pleural effusion and infection. A few examples are-following haemothorax, lung abscess, pneumonia. In India, tuberculosis is an important cause of empyema (Figs 44.15 and 44.16) • Oesophageal perforations-iatrogenic or spontaneous also result in empyema. • Rupture of subphrenic abscess, rupture of hydatid cyst, rupture of amoebic liver abscess also can result in empyema. • The classical events which follow empyema following pneumonia are exudative phase with pleural effusion, followed by thickening of fluid-fibropurulent stage and when the lung is covered by thick cortex, it is called organising phase.

Fig. 44.16: Consolidation

Diagnosis • History of fever diagnosed as pneumonia or tuberculosis • Pain in the chest, difficulty in breathing • Tenderness over the chest • Toxic features in acute empyema specially in children • Presence of thick pus with thick cortex of fibrin and coagulum over the lung. Investigations

• Chest X-ray may show collapse of the lung, tracheal deviation, evidence of pneumonia or tuberculosis. • Aspiration of pleural fluid and analysis-exudative in cases of pneumonia. Send for bacterial culture.

Fig. 44.17: CT right lung empyema

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Fig. 44.18A: Right empyema split pleura sign

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Fig. 44.18B: Empyema left with Koch's spine-split pleura sign

( Courtesy: Figs 44.15 to 44.18 are contributed by Dr C.S. Rajan, Senior Consultant and Head, Dept of Thoracic Surgery, St. Martha's Hospital, Bangalore)

Surgical management of pleural effusions and empyema • Video Assisted Thoracoscopic Surgery (VATS): With 3-4 ports, video assisted procedures have become very popular. Minimal incision, less pain and recovery is fast. Drainage, pleural biopsy, talc pleurodesis, debridement of empyema, intercostal tube drainage (ICD) can all be done. • Rib resections, and drainage through a window-Eloisier's method. • Decortication: More radical procedure involves thoracotomy, debridement, excision of thick cortex or the covering of the lung, so that lung will expand. This is done by posterolateral thoracotomy. Complications • Toxicity, septic shock, multiorgan failure in untreated cases • Damage to the lungs-lobectomy, pneumonectorny Empyema necessitans: It is a type of empyema which presents as a swelling in the subcutaneous plane with communication to the pleural space/cavity. It is a tense, tender, fluctuant swelling with local rise of temperature. lntercostal bulge is also seen. On asking the patient to cough, expansile impulse is felt. Otherwise, management is similar to that of empyema such as drainage, treating the cause, ATT in tuberculosis and antibiotics in pyogenic infections.

BRONCHOPLEURAL FISTULA Definition It is a fistulous communication between the pleural space and the lung. Causes • Following pneumonectomy or an infection.

• It may occur when large airways are in communication with the pleural space following a large pneumothorax. Pathogenesis • There will be a large empty space left behind following pneumonectorny. This space will be filled with air. Over a period of time air is absorbed. The gaping of bronchial stump occurs resulting in bronchopleural fistula. • Invariably the fluid which accumulates later will be infected-purulent. Clinical features • History and clinical features suggestive of empyema • History of lung surgery • Persistent air leak in the intercostal drain • Pus in the ICD Treatment is extremely difficult and disappointing • Propped up/sitting position and turn to the disease side so as to get a dependent drainage • Intercostal drain connected to under water seal • Pleurocutaneous window drainage can be done • Specific treatment includes control of infection, treat the primary cause, suturing, etc. SURGICAL ANATOMY OF MEDIASTINUM AND MEDIASTINAL MASSES The mediastinum is a broad central partition that separates the two laterally placed pleural cavities. It extends from the sternum to the bodies of the vertebrae; and from the superior thoracic aperture to the diaphragm. Contents: The thymus gland, pericardia! sac, heart, trachea and major arteries and veins. It also serves as a passageway for structures such as the oesophagus, thoracic duct and various components of the nervous system as they traverse the thorax on their way to the abdomen.

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Division of mediastinum (Fig. 44.19) • A transverse plane extending from the sternal angle (the junction between manubrium and the body of the sternum) to the intervertebral disc between vertebrae thoracic 4 and 5 spaces separates the mediastinum into the superior mediastinum and inferior mediastinum. • Inferior mediastinum is further partitioned into the anterior, middle, and posterior mediastinum by the pericardia! sac. • Anterior mediastinum: It lies between the back of the sternum and the anterior aspect of the great vessels and pericardium. It contains the thymus, internal mammary arteries, lymph nodes, connective tissue and fat. • Middle mediastinum: It extends from the pericardium anteriorly to the ventral surface of the thoracic spine posteriorly. It contains the pericardium, heart, great vessels, airway and oesophagus. • Posterior mediastinum: It is made up of the spine and includes the costove1tebral sulci. The posterior compartment contains the proximal intercostal neurovascular bundles, the spinal ganglia, the sympathetic chain, lymphatic tissue, and connective tissue.

Vertebral column---------� Inlet of thorax------� Superior mediastinum------� Manubrium---�.rr Inferior mediastinum- --.- -1-+--�--� Branches of internal thoracic artery Body of sternum Anterior mediastinum Sternopericardial ligament Xiphisternum Diaphragm--� Middle mediastinum---� Posterior mediastinum-

-

--.J

Fig. 44.19: Subdivisions of the mediastinum

Differential diagnosis of mediastinal masses is shown m Table 44.2.

Differential diagnosis of mediastinal masses Anterior mediastinum

Middle mediastinum

Posterior mediastinum

Thymus • Thymoma • Thymic cyst • Thymic hyperplasia • Thymic carcinoma

Bronchogenic cyst

Neurogenic tumours • Neurofibroma • Neurilemmoma • Neurosarcoma • Ganglioneuroma • Ganglioneuroblastoma • Neuroblastoma • Chemodectoma • Phaeochromocytoma Meningocoeles

Lymphoma

Pericardia! cyst

Germ cell tumour • Teratoma/dem1oid cyst • Seminoma • Nonseminoma - Yolk sac tumour - Embryonal carcinoma - Choriocarcinoma

Lymphadenopathy • Lymphoma • Sarcoid • Metastatic lung cancer

lntrathoracic thyroid • Substemal goitre • Ectopic thyroid tissue Parathyroid adenoma Haemangioma Lipoma Liposarcoma Fibroma Fibrosarcoma Foramen of Morgagni hernia

Enteric cyst Oesophageal tumours Vascular masses and enlargement

Thoracic spine lesion (e.g. Pott's disease)

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ANTERIOR MEDIASTINAL MASSES "Terrible Ts":Thymic tumours, Teratoma/germ cell tumour, (Terrible) lymphoma, and Thyroid. 1. Thymoma • Most common anterior mediastinal primary tumour; 20% of adult mediastinal neoplasms. • Presentation between ages 30 and 50 (most patients are > 40 years old). • 50% are asymptomatic but others have symptoms secondary to compression: Chest pain, cough, dyspnoea, compression of the superior vena cava resulting in head and neck venous congestion, facial oedema. • Myasthenia gravis: Seen in 30-50% of thymoma patients; others can have hypogammaglobulinemia (10%), endocrine disorders, connective tissue disorders. Staging (Table 44.3)

Investigations

• Chest X-ray may show a rnediastinal mass. • Contrast CT scan of the thorax is the investigation of choice which will detect a mass lesion and its relationship to the adjacent structures (Fig. 44.20). • MRI can also be done • Preoperative biopsy is not necessary (Key Box 44.13). Surgery • Surgery is the main modality of the treatment-a complete resection of the thymus. Median sternotomy is done to remove the tumour. Masaoka's clinical stage Stage l: Stage II: Stage lll: Stage IVa: Stage !Vb:

Macroscopically completely encapsulated and micro­ scopically no capsular invasion Macroscopic invasion into surrounding fatty tissue or mediastinal pleura, or microscopic invasion into capsule Macroscopic invasion into neighbouring organ, i.e. peri­ cardium, great vessels, or lung Pleural or pericardia! dissemination Lymphogenous or haematogenous metastasis

............................. KEY BOX 44.13

.

PREOPERATIVE BIOPSY IS NOT NECESSARY� FOR THE RESECTION OF FOLLOWING TUMOURS 1. 2. 3. 4. 5.

Thymoma Pleomorphic adenoma Testicular tumours Renal cell carcinoma Hepatocellular carcinoma

Fig. 44.20: Thymoma

• A minimally invasive (thoracoscopic or robotic) approach is another option. 2. Germ cell tumours (GCT) • The mediastinum is the most common location for extragonadal germ cell tumours in adults. • They are classified as benign (teratomas, dermoid cysts) or malignant (seminomas, nonseminomatous G CTs). • Seminomas are more common than nonseminomatous GCTs. • Occur in the second decade-late teens • 20% are malignant • Cystic teratomas are dermoid cysts, less malignant compared to solid teratomas which have more chances of malignancy. • Diagnosis is by tumour markers: Alfa fetoproteins (AFP) and beta-hCG. AFP is normal in teratoma and "pure" seminomas. Ninety percent of nonseminomatous germ cell tumours have elevated AFP and/or beta-hCG. • They are highly radiosensitive (Key Box 44.14).

...

.......................... .

KEY BOX 44.14

TUMOURS WHICH ARE HIGHLY RADIOSENSITIV� 1. Germ cell tumours 2. Seminoma 3. Lymphoma 4. Squamous cell carcinoma

3. Lymphomas • The most common are nodular sclerosing Hodgkin's lymphoma and primary mediastinal B-cell lymphoma. • May present with systemic symptoms such as fevers, weight loss or night sweats but can also present with symptoms such as chest pain, dyspnoea, wheezing, stridor, hoarseness,

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• • • •

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dysphagia, or superior vena cava syndrome due to compression of mediastinal structures. Core biopsy is required for the diagnosis CT scan will help in ruling out other diseases. Mediastinal mass ratio (MMR)-the ratio of the mass to the chest diameter is calculated (see page 119 of Chapter 9) Chemotherapy is the treatment of choice.

4. Thyroid masses • lntrathoracic thyroid tissue-ectopic or substernal, typically causes symptoms of shortness of breath or dysphagia. Sometimes they produce dangerous airway obstruction, deviation of the trachea or narrowing. • The intrathoracic mass is usually continuous with the thyroid gland in the neck; only 2 percent of cases are separate from the cervical thyroid and are truly intrathoracic. • Diagnosis is by CT scan and technetium scan. • Majority can be managed with cervical incision-a few may require sternotomy. • Less commonly, intrathoracic mobilisation via VATS is done. • Possibility of collapse of trachea should be kept in mind after thyroidectomy-one should be ready with re­ intubation/tracheostomy. MIDDLE MEDIASTINAL MASSES • Lymphadenopathy is the most common lesion presenting as a mass in the middle compartment of the mediastinum. • The most common causes include lymphoma, sarcoid and metastatic lung cancer. • Mediastinoscopy is a very useful technique to biopsy lymphadenopathy in this region. • Cystic masses comprise approximately 20 percent of middle mediastinal masses. 1. • • •

Bronchogenic cysts Bronchogenic cysts are the most common cystic lesion Secondary to abnormal lung budding during development. Bronchogenic cysts are more common in men, in the right paratracheal region and in the subcarinal location. • Often presents with substernal pain, cough, recurrent infection symptoms or dyspnoea. 2. Enteric cysts • They are the third most common benign oesophageal masses after leiomyomas and polyps, and are usually asymptomatic. • Three criteria are required to establish their diagnosis: (a) Oesophageal attachment (b) The presence of two layers of muscularis propria (c) Epithelium characteristic of the gastrointestinal tract.

3. Pericardial cysts • Seventy percent arise in the right cardiophrenic angle. • Symptoms can include shortness of breath, right heart failure secondary to compression, infection and bleeding. Management • Bronchogenic and enteric cysts need surgical resection to establish a definitive diagnosis and to decrease the risk of infection or malignant degeneration. • Pericardia! cysts can typically be observed if asymptomatic but resection can be utilised if there are symptoms or if the diagnosis is not completely established by imaging. • Simple drainage is generally not recommended because these cysts typically recur without complete resection. POSTERIOR MEDIASTINAL MASSES Neurogenic tumours represent more than 60 percent of posterior mediastinal masses. These lesions are classified based upon their neural cell of origin. 95% of posterior mediastinal masses arise in the intercostal nerve rami or the sympathetic chain region. 1. Schwannomas and neurofibromas • These are benign lesions that arise from the intercostal nerve sheath and make up 90 percent of adult neurogenic tumours. • Neurilemmomas or schwannomas constitute 75% of this group of masses. These tumours are firm, encapsulated masses consisting of Schwann cells. • Neurofibromas are nonencapsulated, soft, friable and are associated with von Recklinghausen neurofibromatosis. • The surgery of choice for removal of these tumours is by thorocoscopy or thoracotomy. • Postoperative complications include Homer's syndrome, partial sympathectomy, recurrent laryngeal nerve damage and paraplegia. 2. Malignant tumours of nerve sheath origin • Malignant nerve sheath tumours are spindle cell sarcomas of the posterior mediastinum and include malignant neurofibromas, malignant schwannomas and neurogenic fibrosarcomas. • They affect men and women equally in the third to fifth decade of life and are closely associated with neuro­ fibromatosis, with a 5% risk of sarcomatous degeneration. • Pain and nerve deficits are common. • Complete surgical resection is the optimal treatment but in patients with unresectable tumours, adjuvant chemotherapy and radiation are options. 3. Autonomic ganglionic tumours • Neuroblastomas and ganglioneuroblastomas are malignant tumours that occur most commonly in children and originate from the sympathetic ganglia.

Chest Trauma, Cardiothoracic Surgery

• Ganglioneuromas are benign lesions that arise from the sympathetic ganglia, and are most common in young adults. Lesions that arise from paraganglionic cells include phaeochromocytomas and paragangliomas. • Some neurogenic tumours are "dumb-bell shaped" and arise near intervertebral foramen, and have a posterior mediastinal and intraspinal component. • Resection usually requires a combined approach with neurosurgery and thoracic surgery. PULMONARY ASPERGILLOMA • Pulmonary aspergilloma, is a mycetoma or fungus ball, caused by fungus of Aspergillus species • The most common place affected by aspergillomas is the lung • Tuberculosis of the lungs and immunocompromised conditions are risk factors. • The fungus settles in a cavity and grows to a big ball­ called fungal ball • Majority of the cases are asymptomatic • Cough, chest pain, haemoptysis, abscess formation are the features • Diagnosis is by chest X-ray and CT scan (Fig. 44.21), majority of the cases do not require any treatment • In symptomatic cases (rarely), removal of the lesion, lobectomy may be required (Fig. 44.22).

Fig. 44.21: CT chest showing crescent sign-pu lmonary

aspergilloma

Fig. 44.22: Pulmonary aspergilloma­ cut open specimen (Courtesy: Figs 44.21 and 44.22, Dr C.S. Rajan, Senior Consultant and Head, Dept of Thoracic Surgery, St. Martha's Hospital, Bangalore)

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BRONCHOGENIC CARCINOMA Introduction Bronchogenic cancer is the most frequent cause of cancer death in men and women and accounts for 14.5% of all cancer diagnoses and 27.6% of all cancer deaths in the United States. Cigarette smoking is unequivocally the most important risk factor in the development of lung cancer. In India, bronchogenic carcinoma has become number one cancer in men overtaking other cancers. The diagnosis is often delayed. Many patients present as metastasis in bones. Surgical resection alone cannot be curative in many cases because of late presentation of the cases. Prognosis is poor in spite of the treatment. Risk factors • Cigarette smoking: It is described as pack years-a pack containing 20 cigarettes. • Industrial carcinogens: Asbestos, arsenic, chromium or nickel, organic chemicals, radon. • Radon is the second leading cause of lung cancer. Radon is a natural radioactive gas released from the normal decay of uranium in the soil. Pathology-types 1. Adenocarcinoma (ADA) of the lung is the most frequent histologic type and accounts for approximately 45% of all lung cancers. Women smokers have more incidence of adenocarcinoma. Adenocarcinoma of the lung develops from the mucus-producing cells of the bronchial epithelium. Cells are cuboidal to columnar cells. Most of these tumours (75%) are peripherally located. Adenocarcinoma of the lung tends to metastasise earlier than squamous cell carcinoma of the lung and more frequently to the central nervous system (CNS)-cases can present as weakness of the limbs, hemiparesis, etc. 2. Bronchioloalveolar carcinoma is an adenocarcinoma. Sometimes it can be a more indolent disease. It is well differentiated and spreads along alveolar walls without invasion of stroma, blood vessels or pleura. 3. Squamous cell carcinoma (SCCA) of the lung occurs in approximately 30% of patients with lung cancer. Approximately 75% of these tumours are centrally located and tend to expand against the bronchus, causing extrinsic compression. These tumours are prone to undergo central necrosis and cavitation. Squamous cell carcinoma tends to metastasise later than adenocarcinoma. Microscopically, keratinisation, stratification and intercellular bridge formation are exhibited. SCCA may be more readily detected on sputum cytology than ADA. 4. Large cell undifferentiated carcinoma may be made in approximately 10% of all lung tumours. These tumours tend to occur peripherally and may metastasise relatively early. Small cell lung cancer represents approximately 20% of

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Differences between adenocarcinoma and squamous cell carcinoma Factors

I. 2. 3. 4.

Incidence Metastasis Location Metastasis to brain

Adenocarcinoma

Most common---45% Early Peripheral More frequent

Squamous cell carcinoma

30% Later Central Less frequent

all lung cancers; approximately 80% are centrally located. The disease is characterised by an aggressive tendency to metastasise. It often spreads early to mediastinal lymph nodes and distant sites, especially bone marrow and brain. 5. Small cell lung cancer appears to arise in cells derived from the embryologic neural crest. Microscopically, these cells appear as sheets or clusters of small dense cells, with dark nuclei and little cytoplasm. This oat-like appearance under the microscope gives the term oat cell carcinoma to this disease. These tumours are often advanced at presentation, with an aggressive tendency to metastasise both by lymphatics and by blood. Chemoradiotherapy is generally used for treatment. However, the 5-year survival rate is only 5%. Table 44.4 shows differences between adenocarcinoma and squamous cell carcinoma.

Fig. 44.23: CT of lungs showing bronchogenic carcinoma

Clinical features • Cough and haemoptysis: lt is a nonspecific symptom in many patients and diagnosis is delayed because it is often thought to be a smoker's cough. • Dyspnoea is due to pleural effusion or due to restrictive pulmonary disease. • Bloody effusion, clubbing of the fingers, localised chest pain are other features. • Hoarseness due to recurrent laryngeal nerve paralysis, back ache due to metastasis in vertebrae, or neurological features due to metastasis in brain are also presenting features in many cases. • Myopathy similar to myasthenia gravis can occur in small cell carcinoma. Diagnosis and spread • In addition to chest X-ray, CT scan, sputum for malignant cells, diagnosis can also be made with bronchoscopic biopsy and CT-guided biopsy. Spread is common PET scan: 18F­ fluorodeoxyglucose positron emission tomography (FDG­ PET) is the investigation of choice to detect the distant lesions (Figs 44.23 to 44.25). • Brain CT or magnetic resonance imaging (MRI) (Fig. 44.26) • Mediastinoscopy

Fig. 44.24: Sagittal section of the CT showing bronchogenic carcinoma right lung

-

•..

••

••

PET-CT of a patient who was diagnosed to have carcinoma lung on bronchoscopy. PET CT shows a hilar mass with a nodule anteriorly on left side of pleura. It also shows pneumonic patch on lower zone of left lung which is FDG avid

Fig. 44.25: PET scan of lung showing bronchogenic carcinoma

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UMiif49iiti Stage Occult cancer 0 IA IB

IIA

IIB IIIA

IIIB IV

T TX Tis T1a/b T2a T2b T1a/b; T2a T2b T3 AnyT1; T2 T3 T4 T4 AnyT AnyT AnyT

N NO NO NO NO NO N1 N1 NO N2 N1/N2 NO/N1 N2 N2 NS Any N

M MO MO MO MO MO MO MO MO MO MO MO MO MO MO M1a/b

• CT of the chest, abdomen; MRJ brain: Mediastinal lymph node masses, metastases to the adrenal glands, brain, lung and bone are common. Fig. 44.26: MRI brain showing metastasis from bronchogenic carcinoma

• Bone metastases are osteolytic. Whole body bone scan is needed.

TNM STAGING The international tumour-node-metastasis T (Primary tumour) TX: Primary tumour cannot be assessed, or tumour proven by the presence of malignant cells in sputum or bronchial washings but not visualised by imaging or bronchoscopy TO: No evidence of primary tumour Tis: Carcinoma in situ T1: Tumour :;:; 3 cm in greatest dimension, surrounded by lung or visceral pleura, without bronchoscopic evidence of invasion, more proximal than the lobar bronchus (i.e. not in the main bronchus) • T 1a tumour :;:; 2 cm in greatest dimension • T 1b tumour > 2 cm but :;:; 3 cm in greatest dimension T2 tumour > 3 cm but :;:; 7 cm or tumour with any of the following features: • Involves main bronchus � 2 cm distal to the carina • Invades visceral pleura associated with atelectasis or obstructive pneumonitis that extends to the hilar region but does not involve the entire lung •T2a tumour > 3 cm but :;:; 5 cm in greatest dimension • T2b tumour > 5 cm but :;:; 7 cm in greatest dimension T3 tumour > 7 cm or one that directly invades any of the following: chest wall (including superior sulcus tumours), diaphragm, phrenic nerve, mediastinal pleura, parietal pericardium; or tumour in the main bronchus < 2 cm distal to the carina but without involvement of the carina or associated atelectasis/obstructive pneumonitis of the entire lung or separate tumour nodule(s) in the same lobe

T4 tumour of any size that invades any of the following: Mediastinum, heart, great vessels, trachea, recurrent laryngeal nerve, oesophagus, vertebral body, carina; or separate tumour nodule(s) in a different ipsilateral lobe N (Regional lymph nodes) NX: Regional lymph nodes cannot be assessed NO: No regional lymph node metastasis N1: Metastasis in ipsilateral peribronchial and/or ipsilateral hilar lymph nodes and intrapulmonary nodes including involvement by direct extension N2: Metastasis in ipsilateral mediastinal and/or subcarinal lymph node(s) N3: Metastasis in contralateral mediastinal, contralateral hilar, ipsilateral or contralateral scalene or supraclavicular lymph node(s) M (Distant metastasis) MX: Distant metastasis cannot be assessed MO: No distant metastasis M1: Distant metastasis • M1a separate tumour nodule(s) in a contralateral lobe; tumour with pleural nodules or malignant pleural (or pericardia!) effusion • M1b: Distant metastasis

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Treatment Can be grouped into three major categories 1. Stages I and II tumours are contained within the lung and may be completely resected with surgery. 2. Resectable stages IIJA and IIIB tumours are locally advanced tumours with metastasis to the ipsilateral mediastinal (N2) lymph nodes (stage IIIA) or involving mediastinal structures (T4NOMO). These tumours, by their advanced nature, may be mechanically removed with surgery followed by systemic chemotherapy. Radiotherapy can also be given to locally advanced disease. 3. Stage lY disease includes metastatic disease and is not typically treated by surgery, except for patients requiring surgical palliation. Systemic therapies-chemotherapy for metastatic disease are common. CONGENITAL HEART DISEASES Introduction • Abnom1al heart structures since birth. • It develops between 3rd and 8th week of foetal life. • 1st operation for congenital heart disease was ligation of PDA by Cross in 1938. • With new advances in neonatal cardiopulmonary bypass (CPB), outcomes have improved. Development • At 12 weeks, primitive vascular tube is fully developed. • Foetal circulation differs from adult in that right and left ventricles pump blood in parallel rather than in series to receive increasingly oxygenated blood. • 3 important structures are ductus venosus, foramen ovale and ductus arteriosus. Changes at birth • Pulmonary vascular resistance falls (breathing) • Pulmonary vasodilation and ductus arteriosus constricts within 30 minutes of delivery (due to increased oxygen levels). • Reversal of pulmonary and systemic pressure gradient. Tennination of blood flow from pulmonary artety into aorta. • Taping/cutting umbilical cord • Venous blood from placenta stops • JVC and right atrial pressure decrease and left right atrial pressure increases (due to increased systemic vascular resistance) • Foramen ovate closes Abnormalities • Persistence of normal channels (patent ductus arteriosus­ PDA, patent foramen ovale). • Failure of septation (atrial septa I defect-ASD, ventricular septa) defect-VSD, tetralogy offallot-TOF).

• Stenosis (intracardiac-supravalvular, valvular, infra. valvular, coarctation of aorta). • Atresia/abnonnal connections (transposition of grea arteries-TGA, total anomalous pulmonary venom connections-TAPVC). Incidence Eight cases per 1000 live births in UK Aetiology Unclear: Infection/Envirotoxins/Genetics Diagnosis In utero foetal ECHO Classification • Cyanotic ( 1/3) and acyanotic (2/3) • Right to left shunts (similar to TOF) • Left to right shunts (similar to ASD, VSD, PDA) • Parallel systemic and pulmonary flow (similar to TGV) • Aortic stenosis, coarctation of aorta • Mixing of systemic and pulmonary flow (similar to TAPVC): Heart failure in infancy. CYANOTIC CONGENITAL HEART DISEASE 1. Tetralogy of Fallot (TOF) Most common cyanotic congenital heart disease Child adopts squatting posture during hypoxic spells which increases systemic vascular resistance and venous return to heart. This directs the blood into pulmonaty circulation and oxygenation improves. Chest X-ray shows boot-shaped heart. Treatment-single or two stage surgery (Key Box 44.15).

....

......................... .

KEY BOX 44.15

1. 2. 3. 4.

COMPONENTS VSD Overriding aorta Pulmonary stenosis Right ventricular hypertrophy

2. Transposition of great vessels (TGA) • Most common cause of cyanosis in newborn. Features • Aorta arises from right ventTicle and pulmonary atiery from left ventricle. • Incompatible with life unless associated with ASD or VSD. • Chest X-ray-'Egg on side' appearance. Treatment • Rashkind percutaneous balloon atrial septoplasty. • Definitive repair-aiierial switch (2 stage) • Mustard or Senning surgery.

Chest Trauma, Cardiothoracic Surgery

3. Total anomalous pulmonary venous drainage • Pulmonary venous drain disconnected from left atrium and drains to systemic circulation, e.g. inferior vena cava (IVC) or superior vena cava (SVC). • Presents with failure to thrive. • Treatment-surgery

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............................. . KEY BOX 44.16

4. Eisenmenger's syndrome • It occurs following reversal of a previous left to right shunt as with ASD or V SD. • Similar to pulmonary hypertension. • Closure of shunt contraindicated.

DIAGNOSIS • Hyperdynamic precordium and bounding peripheral pulses in large shunt • Machinery or continuous murmur (Gibson's murmur) in the left second intercostal space, radiating to the left infraclavicular area • ECG: Left ventricular hypertrophy • Chest X-ray: Cardiomegaly • Pulmonary congestion in large ductus and hilar dance on fluoroscopy • Two-dimensional echo is diagnostic-in suprasternal view, the PDA is seen

ACYANOTIC CONGENITAL HEART DISEASE

PEARLS OF WISDOM

1. Patent ductus arteriosus Definition Persistence of the foetal ductus arteriosus in the postnatal period. Embryology • Derived from the 6th aortic arch • Essential for the foetal circulation • Blood ejected by right ventricle flows exclusively through the ductus to the lower extremity and placenta bypassing the high resistance pulmonary circulation in the foetus. At birth • Physiological closure occurs in 1-6 days • Anatomical closure occurs in 2-3 weeks Mode of closure Smooth muscle constriction in response to rising arterial oxygen tension. Pathophysiology Shunt occurs across the ductus both in systole and diastole resulting in left ventricle overload and pulmonary plethora. • Shunt depends on the size of the ductus and pulmonary and systemic vascular resistance. Continuous murmur is due to shunt during both systole and diastole. Clinical features • Incidence: M : F = l :2 • It depends on the size of the ductus, pulmonary vascular resistance, age at presentation and associated anomalies. a. Small ductus: Asymptomatic b. Infants: Congestive heart failure c. Children: Dyspnoea on exertion, repeated respiratory tract infection (Key Box 44.16)

Occurs 1 in 5000 live births. 50% in premature babies Complications • Reversal of shunt • Infective endocarditis • Congestive heart failure Treatment It can be divided into surgical and nonsurgical methods. I. Surgical methods • Presence of PDA is sufficient indication for surgery even without symptoms. • In infants: Large PDA with congestive cardiac failure not responding to antifailure measures, surgery is indicated. Surgery • Triple ligation • Division and suturing • Right lateral position and posterolateral thoracotomy • Thorax entered through 4th intercostal space • It can also be done by thoracoscopy and video-assisted thoracoscopic surgery (VATS) and clipping of PDA. Care during surgery Left recurrent laryngeal nerve must be carefully preserved Complications during surgery • Haemorrhage, left recurrent laryngeal nerve palsy • Chylothorax II. Nonsurgical methods • Pharmacological closure: In preterm infantsindomethacin • Transcatheter closure: lnterventional cardiology • Double umbrella device-Rashkind • Gianturco coils • Lock Clamshell occluder.

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2. Coarctation of aorta Definition Congenital narrowing of the descending thoracic aorta usually occurring just distal to the left subclavian artery origin, adjacent to the site of insertion of the ductus arteriosus. Incidence • 0.2-0.6 per 1000 live births • 5-8% of all cases of congenital heart diseases. Aetiology Flow theory: Reduced flow in the aorta due to multiple abnormalities in the heart during the foetal period. Ductal sling theory: When there is isolated coarctation, this theory is applicable. Abnormal extension of contractile ductal tissue into the adjacent aorta which results in coarctation.

............................. KEY BOX 44.17

ASSOCIATED CONGENITAL ANOMALIES • • • •

• CXR: Notching of the ribs (Dock's sign) from third rit onwards, above four years of age. Diagnosis • Two-dimensional echocardiogram, cardiac catheterisation • Transoesophageal echocardiogram (TEE) • Angiogram PEARLS

Types Infantile-preductal and adult-juxtaductal. Classification • Group I: Isolated coarctation • Group II: Coarctation with VSD • Group III: Coarctation with complex intracardiac anomalies (Key Box 44.17). Clinical features Depend on: Age and symptoms at presentation, location of the coarctation, severity of the coarctation, associated anomalies. Symptoms • Visual disturbances, exertional dyspnoea • Upper extremity hypertension • Headache, epistaxis • Claudication of the lower limbs Signs • Systolic murmur heard over the precordium and posteriorly between the scapula. • Feeble femoral pulses. • Enlarged collateral vessels are seen (Suzman's sign) and palpable between scapula and bruit can be heard here. • Systolic gradient between arm and leg blood pressure. Collaterals • Subclavian artery and its branches • Internal mammary artery • Intercostal artery • Scapular, cervical, vertebral, epigastric and spinal arteries. Investigations • ECG: LVH with LV strain

Ventricular septal defect Bicuspid aortic valve Patent ductus arteriosus Mitral valve abnormalities

OF

WISDOM

Classic 'three' sign: Formed by dilated left subclavian artery, narrowing ofthe coarctation and the poststenotic dilatation of aorta. Complications (Key Box 44.18)

............................. . KEY BOX 44.18

• • • • • •

COMPLICATIONS Circle of Willis' aneurysm rupture Aortic dissection Early coronary atherosclerosis Bacterial endocarditis Congestive heart failure Spontaneous rupture of aorta

Treatment A. Nonsurgical (infants) B. Surgery • Left posterolateral thoracotomy • Thorax entered through fourth intercostal space • Arterial line in the right upper limb • Preserve the vagus and recurrent laryngeal nerves • Arterial line in the lower limb • Lower limb pressure during cross-clamping of aorta, to be kept above 45 mmHg • If the pressure is low, then create a shunt from the aortic arch to the descending thoracic aorta with the help of a cannula. Complications of surgery • Haemorrhage, RLN injury, phrenic nerve injury • Homer's syndrome, chylothorax • Paradoxical hypertension, paraplegia, stroke.

Chest Trauma, Cardiothoracic Surgery

Late complications • Recoarctation, aneurysm and left arm ischaemia (when subclavian flap aortoplasty is done).

............................. . KEY BOX 44.19

I. Nonmodifiable

Pseudocoarctation Rare condition which results from congenital elongation of the aortic arch which leads to redundancy and kinking of the aorta and may appear similar to coarctation but there is no obstruction to blood flow.

Treatment Open heart surgery with CPB and closure of defect with sutures or with pericardia! or synthetic patch. 4. Ventricular septal defect Types: 4 types • Perimembranous defect (in membranous septum): 70-80% • Muscular defect (multiple): 10% • Atrioventricular defect: 5% • Subarterial defect: 5-10% 0/E: Pansystolic murmur Treatment: Surgical repair CORONARY ARTERY BYPASS SURGERY Introduction Often simply called bypass surgery, it is the most commonly performed open heart surgery all over the world. The incidence of coronary artery disease is rapidly rising in the developing countries. A vein graft from the lower limb or an arterial graft (internal mammary artery or the radial artery) is used to bypass the obstructed coronary artery. The bypass is done from the root of the aorta to the distal coronary artery. • The obstruction or the block is not touched. Risk factors for coronary artery disease (Key Box 44.19)

Incidence Least in Japan and highest in Finland.

RISK FACTORS

• Age, sex, family history II. Modifiable, controlled or treated by medicines: • Smoking, uncontrolled diabetes • Uncontrolled hypertension • Obesity, overweight, lack of physical exercise • Individual response to stress • High blood cholesterol or lipid abnormalities

3. Atrial septal defect Defect in septum between left and right atrium leading to left to right shunt. Types:3types • Ostium secundum: Defect in floor of fossa ovalis. Presents late in life. • Ostium primum: Endocardial cushion defect associated with mitral valve defects (mitral regurgitation) and trisomy 21. Presents earlier in life. • Sinus venosus: Defect near junction of SVC and atrium.

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Indications for surgery I. Usually indicated in symptomatic or for prognostic reasons (i.e. balance between expectant benefit and risk faced by patient). a. > 50% stenosis of left main stern artery b. > 70% stenosis of proximal left anterior interventricular artery c. Triple vessel disease d. Poor ventricular function associated with CAD. 2. Chronic stable angina 3. Acute coronary syndrome 4. Surgery for complications of MI such as ventricular septa! rupture, etc. PEARLS

OF

WISDOM

With interventional cardiology progressing rapidly the indications for surgery are getting blurred. Investigations • ECG/cardiac enzymes-Trop T and Trop I • 2D echocardiograrn-new stress ECHO • Holter monitoring, thallium scan-201 or 99mTc • Stress test (exercise tolerance test) • Coronary angiogram • Radionucleotide studies and cardiac MRI • CT (multislice high resolution) Contraindications of CABG • • • •

Small, diffusely diseased arteries Diffuse disease and heart failure Acute myocardial infarction over 6 hours old Moribund patients after resuscitation

Surgery • • • •

Bypass surgery with vein or artery graft Endarterectomy Venous patch Transmyocardial laser revascularisation (TMLR).

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1030 Bypass surgery

l. Techniques of bypass • Cold cardioplegia with moderate hypothermia and cardiopulmonary bypass. • Ischaemic cross-clamp with moderate hypothermia with cardiopulmonary bypass. • Empty beating heart, normothermic with cardio­ pulmonary bypass. • Beating heart surgery, no heart lung machine. 2. Grafts • Veins-lower limbs (long saphenous vein) • Upper limb (cephalic vein) rarely used • Arteries-radial (Key Box 44.20) • Internal mammary artery both right and left • Gastroepiploic • Inferior epigastric • Inferior mesenteric

............................. . KEY BOX 44.20

• • • • •

ADVANTAGES OF ARTERIAL GRAFT

It is artery to artery anastomosis Compatible size Flow adaptation IMA advantages: No vasa vasorum Dense nonfenestrated intact internal elastic lamina that inhibits cellular migration and subsequent initiation of hyperplasia • Thin medial layer with a few smooth muscle cells which provide a little vasoreactivity • Produces more prostacyclines which is a vasodilator and platelet inhibitor 3. Nonautogenic conduits • Cryopreserved human saphenous vein allograft • Processed bovine sacral artery • Polytetrafluoroethylene graft (Gore-Tex) Standard surgical procedure • • • • • • • • • • • •

Median sternotomy Internal mammary artery dissection done Pericardium opened Systemic heparinisation 3 mg/kg Aortic and venous cannulation. Cardiopulmonary bypass staited Aorta cross-clamped and cold K+ rich solution given in the aortic root Heart arrested in diastole to achieve immobile surgical field Distal anastomosis completed Proximal anastomosis done with the heart beating Weaned off CPB Protamine given and decannulation carried out Sternum closed, extubation 6-8 hours later.

Complications of surgery 1. Perioperative MI (2-3%) 2. Stroke 3. Arrhythmias (30%) 4. Sinus tachycardia 5. Atrial fibrillation 6. Reinfarct 7. Wound infection 8. Bleeding 9. General weakness 10. Mortality (2-3%) 11. Persistent poor cardiac output requiring inotropes o mechanical support Mechanical support to low cardiac output patients post CABG: Intra-aortic balloon pump (IABP) • Inserted percutaneously in common femoral aitery, threade1 into aorta till its tip lies in distal arch vessels. • Balloon is triggered by the ECG, deflating during ventricula systole (decreasing afterload) and inflating during diastol1 (increasing diastolic pressure and blood flow to coronaries) Prognosis • Ability to return to normal lifestyle • Improvement in ventricular function • Work capacity better than preoperative • Risk of arrhythmias unchanged • Minimal medications • Complete and dramatic relief of chest pain. Patency Year 1 5 10

Artery 97 95 90

Vein 90 60 40

Recent advances • Age limitation changed (CABG done in 80 years old also) • Improved myocardial preservation • Composite arterial grafts • Sutureless anastomosis • Endoscopic vein harvesting • lntracoronary injections of vascular endothelial growth factor (VEGF) • All major cities have the centres and expertise • TMLR TMLR (transmyocardial laser revascularisation) • • • • •

Severe CAD not treated by PTCA or CABG Emulates reptilian circulation in the mammalian heart No CPB CO2 or excimer laser Limited thoracotomy

Chest Trauma, Cardiothoracic Surgery

• Creation of channels I mm in size in the myocardium from epicardium to endocardium • Allows perfusion of blood directly from the LV cavity to the intramyocardium • Creation of intramyocardial channels, neoangiogenesis and denervation are the factors which improve perfusion to the ischaemic myocardium.

4. Vascular intimal hyperplasia: lmp011ant cause of graft occlusion. Gene-based therapies to prevent this before grafting the veins. 5. Minimally invasive direct coronary artery bypass (MIDCAB) surgery: Anterior submammary incision. LIMA dissected down with the aid of a thoracoscope and grafted to the LAD.

Composite arterial grafts • Arterial grafts have long-term patency. • Patients with total arterial grafts may not require future surgery. • Arterial grafts are used in the shape of T or Y. • It can revascularise almost all blocked major vessels.

ABDOMINAL AORTIC ANEURYSMS (AAA) • Aneurysms are defined as a focal dilatation of at least 50 % larger than the expected normal arterial diameter • For AAA-transverse diameter 3 cm or greater PEARLS OF

Only two small incisions With help of a subcutaneous tunnel, the vein is dissected Requires expensive equipment Major branches are tied and smaller ones are controlled by pressure • Cosmetic advantage • Takes longer time OFF PUMP CORONARY ARTERY BYPASS SURGERY Procedure • Beating heart surgery, no heart lung machine • Sternotomy, stabilisation devices used • Short-acting beta-blockers to reduce the heart rate. • Proper positioning of the heart with mechanical stabilisation device for proper visual presentation • CPB standby, all vessels can be bypassed by proper position of heart, quick recovery, early extubation • No CPB, less trauma, reduce risk of bleeding and kidney failure, reduced hospital stay. Advantages • Avoids physiological stress associated with CPB. • Aortic manipulation leading to neurological complications avoided. Whole body inflammatory response that occurs due to CPB is decreased. • Incidence of postoperative renal failure due to CPB is decreased. Future developments/Recent advances 1. Bypass graft coupling devices to facilitate anastomosis (clips, stents). 2. Normalising left ventricular geometry with intracardiac devices. 3. Myocardial protection during CPB (ventricular assist devices). lschaemic preconditioning pharmacologically preoperatively.

WISDOM

Most common type of true aneurysm with high propensity to rupture and it is the 15th overall leading cause of death.

Endoscopic vein harvesting • • • •

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Risk factors for AAA (Key Box 44.21)

.

............................ .

KEY BOX 44.21

MOST IMPORTANT RISK FACTORS OF AAA � 1. Age: Peak incidence at 80 to 85 years 2. Male : Female ratio 4 : 1 3. Genetic: First-degree relatives: 11-fold increase in relative risk 4. Tobacco use: 8 : 1 preponderance of aneurysms in smokers 5. Other factors: 55% demonstrate C. pneumoniae by immunohistochemistry.

Site 95%-infrarenal aorta, 5%-suprarenal aorta. Salient features Fates of aneurysm

I. 2. 3. 4.

Infection Rupture (free/contained) T hrombosis Embolism

Growth rates < 5 cm diameter-0.3 cm/year > 5 cm diameter-0.5 cm/year. Clinical features Incidental finding 1. Pulsatile abdominal mass on palpation 2. Undergoing evaluation for another abdominal pathology 3. Surgery for unrelated abdominal or pelvic operation. Classical triad I. Sudden-onset midabdominal or flank pain 2. Shock 3. Presence of a pulsatile abdominal mass (expansile pulsation).

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Findings on palpation 1. Finn mass 2. Expansile pulsation over the mass 3. If upper border is palpable, then the origin is probably infrarenal. Diagnosis 1. Radiograph-Eggshell pattern of calcification 2. Abdominal ultrasound a. Detail of the vessel wall b. Presence of plaques c. Size 3. CT-Most precise a. Proximal and distal extent b. Amount and location of mural thrombus c. Calcifications d. Adjacent structures

4. Risk factor modifications 5. Drugs used: ex-blockers, NSAIDs-inhibits elastase • MMP inhibitors-doxycycline • Repair is indicated when it is symptomatic or> 1 cm/yei growth. High-risk patients • Delay in repair until larger diameter • Endovascular aneurysmal repair (EVAR) can be tried. Techniques of open repair 1. Transperitoneal approach 2. Retroperitoneal approach is indicated in cases of hostil abdomen, suprarenal aneurysm, horseshoe kidne) peritoneal dialysis, inflammatory aneurysm and in ascite� 3. Minimal incision aortic surgery 12 to 15 cm incision, 9 cm proximal to the umbilicus. Endovascular aortic aneurysm repair

4. MRI/MRA

5. Contrast arteriography Less frequently used

Stent-graft is introduced into the aneurysm through th1 femoral arteries and fixed in place to the nonaneurysmal aorti1 neck and iliac arteries with self-expanding or balloon expandable stents (Palmaz stents), or with barbs, pins o hooks.

Complications (Key Box 44.22)

Special considerations

............................. .

1. Inflammatory aneurysm • Jncidence is about 5% • Adherent to: Duodenum, IVC, left renal vein, ureters-th� reason being lymphatic obstruction during aneurysrr expansion and secondary fibrosis and infection from chronic contained rupture • Rupture is uncommon (as it is often symptomatic and treated before rupture) • Retroperitoneal approach is preferred.

a. Less frequently used b. Used if patient has renal failure

KEY BOX 44.22

COMPLICATIONS

1. Most frequent-nonfatal Ml and renal failure 2. Bleeding 3. Most serious GI complication-ischaemia of the left colon and rectum 4. Lower extremity ischaemia 5. lschaemic injury to the spinal cord or lumbosacral plexus 6. Postoperative sexual dysfunction 7. Deep vein thrombosis (DVT)

2. Aortocaval fistula a. Continuous abdominal bruit+ b. High-output cardiac failure

Management Low-risk AAAs • Followed with serial size measurements. Reduce expansion rate and rupture risk by conservative treatment such as: I. Smoking cessation 2. Blood pressure control 3. Reduction of cholesterol

c. 'Steal' phenomenon-ischaemia to the lower limbs. Treatment: Fistula closure followed by AAA repair. 3. Horseshoe kidney a. Kidney usually fused anterior to the aorta b. Left retroperitoneal approach c. Endovascular repair is not possible.

Chest Trauma, Cardiothoracic Surgery

RUPTURED ABDOMINAL AORTIC ANEURYSM Types of rupture Anteriorly-into peritoneal cavity Posteriorly-into retroperitoneum Clinical features Back and abdominal pain, pallor, diaphoresis and syncope. If untreated, it is fatal in all cases. Treatment • Immediate surgical repair • If patient is unstable + previously diagnosed or a pulsatile mass, he is transferred immediately to OT. • If stable + questionable diagnosis, a CT and open surgical repair, control the haemorrhage+ resuscitation+ aneurysm repair. Early postoperative mortality rate is 45%. Differential diagnosis of ruptured AAA • • • •

Angina pectoris, perforated peptic ulcer Acute pancreatitis, acute cholecystitis Acute diverticulitis, mesenteric vascular occlusion Prolapsed lumbar intervertebral disc, sciatica

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INTERESTING 'MOST COMMON' IN THIS CHAPTER • The most common cause of respiratory insufficiency following chest trauma is pneumothorax. • Most common cyanotic congenital heart disease is tetralogy of Fallot. • Most common cause of cyanosis in newborn is transposition of great vessels. • Most commonly performed open heart surgery all over the world is CABG. • Most common type of true aneurysm with high propensity to rupture is abdominal aortic aneurysm. • Most common type of AAA is infrarenal • Most commonly done investigation for AAA is CT scan • Most common noncardiac complication after repair of AAA is renal failure.

WHAT IS NEW IN THIS CHAPTER?/RECENT ADVANCES

• All topics have been updated. • Mediastinal tumours, bronchogenic carcinoma have been included. • Coronary artery bypass graft and abdominal aortic aneurysm have been discussed in more detail.

MULTIPLE CHOICE QUESTIONS 1. Fracture of the following rib is a marker of severe trauma: A. First B. Fourth C. Eighth D. Tenth 2. Treatment of posterior flail segment is: A. Strapping B. Open reduction and fixation C. No treatment is required D. External fixator application 3. Posterior flail segment does not require treatment because: A. Scapula supports the flail segment B. It does not cause complications C. It heals by itself D. It has no physiological implications 4. 'Internal pneumatic fixation' for flail chest is the term used for: A. Insertion of a balloon into the chest B. Internal fixation with screws C. Endotracheal intubation and positive pressure ventilation D. Valsalva manoeuvre

5. Tension pneumothorax should immediately be treated with: A. Intercostal tube insertion B. Needle thoracostomy C. Thoracotomy D. Thoracoscopic drainage 6. Features of tension pneumothorax include all of the following except: A. Tachypnoea B. Hypotension C. Dull note on percussion D. Tachycardia 7. Indications for thoracotomy in haemothorax include all of the following except: A. Drainage more than 1000 ml B. Drainage of more than 100 ml/hour for 4 hours C. If clotted, haemothorax is suspected D. Coagulation abnormalities 8. The intercostal drain can be removed in all of the following situations except: A. Lung is fully expanded B. Drainage< 100 ml C. No air leak D. The patient is on a ventilator

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9. The following is diagnostic of surgical emphysema: A. The lungs are emphysematous B. Always follows surgery C. Palpable crepitus D. Infiltrates on chest X-ray 10. The following is true about mediastinal emphysema except: A. The emphysema is mainly suprasternal B. Mediastinal drain needs to be inserted C. Pericardia! crunching sounds can be heard on auscultation D. Oesophageal and tracheal injury need to be ruled out 11. Nontraumatic rib fractures may be seen in all of the following except: A. Hypoparathyroidism B. Metastatic disease C. Older patients after violent coughing D. Osteogenesis imperfecta 12. The classic 'three' sign of coarctation of aorta is formed by all of the following except: A. Dilated left subclavian artery B. NatTOwing of coarctation

C. Post-stenotic dilatation of aorta D. Dilated pulmonary artery 13. Most common type of aneurysm with a propensity ti rupture: A. Abdominal aortic aneurysm B. Carotid artery aneurysm C. Radial artery aneurysm D. Cerebral artery aneurysm 14. The most common cause of noncardiac complicatior after abdominal aortic aneurysm repair is: A. Pulmonary insufficiency B. Cerebral insufficiency C. Renal insufficiency D. Hepatic insufficiency 15. The most common cause of respiratory insufficienc) following chest trauma is: A. Pulmonary contusion B. Pneumothorax C. Flail chest D. Haemothorax

ANSWERS A 11 A

2 C 12 D

3 A 13 A

4 C 14 C

5 B 15 B

6 C

7 D

8 D

9 C

10 B

45 Neurosurgery • • • • • • •

Classification of head injuries Primary lesions Secondary lesions Extradural/epidural haematoma Chronic subdural haematoma Raised intracranial pressure Fracture skull

Introduction Head injuries derive their importance because of the fact that many patients who die or who are disabled belong to the younger age groups. Head injuries account for 1 % of all deaths, one-fourth of deaths due to trauma and they are responsible for half of all deaths from road traffic accidents. Majority of the patients are young, adult males. HEAD INJURIES Classification I. Based on clinical type 1. Open 2. Closed

• CSF rhinorrhoea

• Pott's puffy tumour • Hydrocephalus • Brain tumours • Brainstem death • What is new?/Recent advances

are likely to develop an infective complication following the head injury and he can initiate measures to prevent it. Blunt injuries, depending on the severity of impact can result in an open or closed head injury. Missile injuries tend to result in an open head injury most often. • The brain is protected by a bony box which has a vault and base of the skull. The base of skull in contrast to the vault is a rough terrain due to the various bony prominences, ridges and foraminae. • This factor is important in causing extensive brain damage to the brain in acceleration/deceleration type of injuries. In addition to the linear acceleration/deceleration, rotational acceleration is also capable of producing damage to the brain as the brain swirls about inside the skull. Such injuries result in maximal damage at interfaces between structures of different densities such as grey matter-white matter junctions.

II. Based on type of injury 1. Blunt injury-acceleration, deceleration 2. Missile injuries • The term open head injury is used to denote a type of injury in which there is a fracture of the skull associated with tear of the dura and arachnoid, resulting in cerebrospinal fluid leak either to the external environment or into one of the potentially infective areas in the base of the skull, e.g. CSF rhinorrhoea or otorrhoea.

I. • • •

• A closed head injury is one where there is no such leakage. The advantage of this classification is that it helps the treating physician to recognise a group of patients who

II. Secondary lesions • Swelling, haemorrhage • Extradural haematoma

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Pathology The pathological changes due to trauma to the brain can be classified into primary and secondary. Primary lesions Diffuse neuronal damage Shearing lesions Contusions and lacerations

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• Subdural, intracerebral haematoma • Infection

II. Secondary lesions (Key Box 45.3)

I. Primary lesions (Key Box 45.1) • Diffuse neuronal damage is the most constant feature of blunt injuries. Immediately after an injury no changes may be seen but changes begin after 14 hours of injury and maximum effects may last up to one week. Prolonged unconsciousness may follow injuries which produce only diffuse neuronal damage without any obvious macroscopic changes. Shearing lesions of the nerve fibres account for some severe injuries without any conspicuous changes to naked eye examination of the brain. A few important primary lesions are discussed below. • Cerebral concussion: Alteration in consciousness without structural damage as a result of non-penetrating traumatic brain injury. There might be loss of consciousness, confusion and amnesia. These are the features. Widespread degeneration of white matter occurs without much changes in the nervous system cortex or brainstem. These patients have spasticity in all four limbs after injury and when they regain consciousness, they are found to be severely demented (Key Box 45.2). • Contusion and lacerations are the obvious naked eye changes seen after injuries and were thought to be the main injuries before diffuse neuronal damage and shearing lesions were described. Contusions are seen on the summit of the gyri which get injured against the bone. The overlying pia is torn and the blood seeps into the subarachnoid space. A bleeding cortical vessel may result in the formation of the acute subdural haematoma or intracerebral haemorrhage. Brain oedema which develops surrounding the contusion and lacerations is the one that determines the outcome. Most often contusions are seen at the tips of the frontal and temporal lobes, under surface of frontal and temporal lobes, over corpus callosum, superior and anterior surfaces of cerebellum and anterior surface of brainstem.

.

............................ .

KEY BOX 45.1

PRIMARY LESIONS • Diffuse neuronal damage • Cerebral contusion • Cerebral laceration

............................. KEY BOX 45.2

• • • • •

CEREBRAL CONCUSSION Temporary physiological paralysis of the nervous system Loss of consciousness Post-traumatic amnesia Recovery may be complete Some can develop complications

.

Brain swelling: This is a vague term applied to increase ir brain bulk due to both oedema and venous congestion. It ii aggravated by hypoxia or respiratory insufficiency which ma) be due to associated lung injury or obstruction to uppe1 respiratory passages. Sometimes such a swelling can lead tc severe brain compression which is difficult to relieve, sine{ there is no single mass lesion. PEARLS

OF

WISDOM

Malignant cerebral oedema has close to 100% mortality. This is more common in children. Intracranial haemorrhage: Extradural or subdural haemorr­ hages may develop as a clean cut secondary event, even though bleeding may have started at the time of injury. These cause compression of brain, secondary rise in intracranial pressure and can cause death if not detected and treated early. Infections: All open head injuries are liable to result in intracranial infection either as generalised meningitis or focal infection such as subdural empyema or brain abscess, osteomyelitis of skull. After closed head injuries, infection of a subpericranial blood clot may result in Pott's puffy tumour. When infection supervenes on an already injured brain, it may retard the recovery or may even lead to death. Hence, it becomes mandatory to treat all infections vigorously.

Cause of death in head injuries • It is instructive to consider the pathological findings in fatal cases and to speculate the deaths which might have been prevented. For example, earlier many deaths which had occurred as a result of aggravation of brain swelling due to hypoxia could have been prevented by ventilation and anti-oedema measures. It should be emphasised that the role of the treating physician is to anticipate and take

.

............................ .

KEY BOX 45.3

SECONDARY LESIONS 1. Brain swelling • Oedema • Venous congestion • Hypoxia 2. lntracranial haemorrhage • Extradural • Subdural 3. Infections A. Open head injury • Generalised meningitis • Subdural empyema B. Closed head injury • Pott's puffy tumour

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appropriate measures to prevent the patient from succumbing to the secondary injuries (damages-seizures, hypoxia). In extensive primary damage to the brain, apart from supportive treatment, one may have to wait and hope. • Extensive injury to vital areas such as diencephalon, or patients with diffuse damage are not likely to survive. These are patients who are unconscious from the time of injury with bilateral, dilated, fixed pupils, flaccidity in all 4 limbs and autonomic disturbances. • Sometimes a head injury associated with extensive injuries to chest, abdomen or the limbs by their sheer severity can cause death. • Intracranial complications such as haematomas, brain swelling, infection, and extracranial complications such as chest injury/metabolic abnormalities, if recognised and treated early, can go a long way in saving the life of the patients. INTRACRANIAL HAEMATOMA • Most of the head injuries are mild or minor and irrespective of how they are managed, the patient recovers on his own. All those who are unconscious, even if briefly, run the risk of respiratory obstruction. Some of the so-called trivially injured run the risk of developing an intracranial haematoma. Hence, all head injuries must be taken seriously. A complicated head injury is one where anyone of the secondary pathological changes may occur and threaten the life of the patient. Uncomplicated head injury is one where no such events occur. However, it could be a severe one where the unconsciousness is prolonged. • These haematomas could develop in any one of the planes intracranially. Extradural (epidural), subdural, intracerebral haematoma, or a haemorrhagic contusion. • The clinical presentation of these haematomas are due to either increase in the intracranial pressure or due to signs of cerebral compression. In the case of acute subdural haematoma or intracerebral haematoma, the clinical picture and the outcome of treatment is also dependent on associated brain damage. EXTRADURAUEPIDURAL HAEMATOMA (Fig. 45.1) • The clot collects between the dura and the inner table of skull. A majority of them occur in the middle cranial fossa, since injwy to middle meningeal vessels (vein and artery) is the commonest cause. However, about 20-25% of the extradural haematomas can occur in the frontal, parietal regions, at the vertex or in the posterior fossa. Injuries to the dural venous sinuses or a large diploic venous channel are the other causes for the formation of a haematoma. Depending upon the source of bleeding, the haematoma could collect rapidly (hyperacute type) or slowly over a

Fig. 45.1: CT scan showing extradural haematoma

period of a few hours to a few days and present as a chronic lesion. 60-80% of these patients have an associated fracture of the skull bone and only a few of them may present with classical symptoms with lucid interval. In the remaining patients, the initial picture can vaiy from an unconscious state to a fully conscious person with or without a history of post-traumatic amnesia. With the widespread availability and use of CT scan, the diagnosis has become much simpler nowadays. However, a few clinical features are worth mentioning. 1. Deteriorating consciousness level • This is one of the hallmarks for the diagnosis of intracranial haematoma. The term 'lucid interval' is used when a patient recovers from an initial period of unconscious state. Though in earlier days this was said to be associated with intracranial haematomas, it can occur in other conditions such as brain oedema, multiple contusions. To assess the consciousness level properly, instead of using vaguely defined terms such as semiconscious, obtunded, etc. the 'Glasgow Coma Score' is widely used, to avoid observer errors in the observation of such patients. • Restlessness in a previously quiet patient indicates increasing intracranial pressure, which again needs to be investigated. At the earliest appearance of focal neuronal deficit, the patient has to be taken up for explorato1y burr holes. • Progressive neurological deficit indicates cerebral compression and the manifestation may depend on the area of the brain affected. 2. Pupillary abnormalities These are to be considered as a late manifestation. It is due to pressure by the herniating temporal lobe on the ipsilateral third nerve at the tentorial hiatus. In the early stages due to irritation of the nerve, there is constriction. Since it is a transient phenomenon, the early constriction goes unnoticed most of time. The patients are often detected in the next stage,

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i.e. pupillary dilatation, caused by paralysis of pupilloconstrictor fibres in the third nerve. However, if the cerebral compression is unrelieved, this may go onto bilateral. Pupillary dilatation is due to ischaemia of third nerve nucleus at the midbrain which is caused by pressure on the posterior cerebral artery. These series of pupillary changes have been termed Hutchinsonian pupils. The dilated pupil has a definite localising value in that if an exploratory burr hole has been decided upon, it should be done on the side of the initially dilated pupil. 3. Autonomic disturbances Bradycardia, though said to be a definite sign, is a late and not an early sign. Initially, there may be a rise in the pulse rate (tachycardia) which may progress to bradycardia, when the systolic blood pressure increases. At times there may be a rise in the diastolic pressure also. These changes occur due to changes in the cerebral blood flow as a consequence of increased intracranial pressure. Respirations become deep and slow rate (bradypnoea) and later patients may develop Cheyne-Stokes ventilation due to brainstem ischaemia. PEARLS OF

WISDOM

Cushing's triad of increased intracranial pressure (ICP). • Bradycardia • Hypertension • Irregular respiration • Local scalp swelling is seen in more than half of the cases. Thus, examination of the head for any such swelling becomes important. • Some of these patients may have a stiff neck either due to increased intracranial pressure or due to associated injury to neck muscles. Mild fever may, at times, occur and this sometimes confuses the observer. In such a case, the patients must be investigated with a definitive investigation like CT scan. If much time is not available, one should not hesitate to proceed to exploratory burr holes or a 'trauma craniotomy flap' has to be employed to rule out a haematoma. • Though in adults, 'shock' is a rare complication of head injury, in children with intracranial haematoma and associated cephalhaematomas, due to volume depletion 'shock' may be encountered. Even in adults, if there is a large scalp injury which is not sutured immediately, shock can occur. • Posterior fossa haematomas in any plane are dangerous because of the lesser space available for the haematomas. As a result of this, rapid brainstem compression can occur which may prove fatal. The availability of CT scan has made detection of these so-called 'unusual haematomas' more frequent. In a suspected case, even if facilities are not available, the treating physician should explore the posterior fossa, if the clinical features suggest haematoma,

or if the skull X-rays show a fracture line extending acros� the occipital bone towards the foramen magnum. Investigations (Key Box 45.4) As has been pointed out earlier, the advent of CT scan of the head has made the diagnosis easier and more specific. However, it should be emphasised that in the absence of CT scan, if adequate clinical features point out to the possibility of an intracranial haematoma, the patient must be taken up immediately for an exploratory surgery rather than wait and allow him to develop irreversible brainstem damage. Since 60-80% of patients with an intracranial haematoma have a skull bone fracture, irrespective of his consciousness level has to be observed for at least 24-48 hours. Occasionally, one may have to resort to old investigations such as angiography not only to establish the haematoma but also to rule out associated dural venous sinus injury.

............................. . KEY BOX 45.4

INDICATIONS FOR SKULL RADIOLOGY • Loss of consciousness • Obvious depression on the skull • Compound fracture • Laceration or contusion of the scalp • Focal neurological signs

TREATMENT OF HEAD INJURIES IN GENERAL I. Resuscitation and support 1. Admission is indicated when: a. Definite history of unconsciousness b. Fracture temporal bone c. Person who cannot be attended by the doctors immediately, i.e. no medical facilities nearby. d. Post-traumatic seizures-patient should be admitted. 2. Casualty reception a. Airway • Mouth gag-to prevent tongue falling backwards. • Endotracheal intubation with positive pressure ventilation. Hypoxia is an important cause of cerebral oedema which worsens the level of consciousness. b. General assessment of patient • To rule out abdominal injuries such as splenic rupture. • Haemothorax-may need an intercostal tube. • Long bone fractures c. General assessment of the degree of shock by pulse, blood pressure monitoring and treatment. d. Neurological assessment by Glasgow Coma Scale 1. Eyes open 4 Spontaneously To speech 3

Neurosurgery

To pain None 2. Best verbal response Oriented Confused Inappropriate words Incomprehensible sounds None 3. Best motor response Obeys commands Localises the pain Withdrawal to pain Flexion to pain Extension to pain

2 5 4 3 2

6 5 4 3 2 (severe damage with increase of ICP) 1 None • Total score is 15; minimum score is 3. Any patient who has a coma score of 8 or less than 8 is said to be in coma.

.............................

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KEY BOX 45.5

.

EXTRADURALHAEMATOMA

• 3 cm vertical incision immediately above the midpoint of zygoma • Strip the pericranium • Burr hole with Hudson's brace • Evacuate 'black-currant jelly' clot • Extend the burr hole and control bleeding middle­ meningeal artery by bipolar diathermy • Dural hitch sutures to prevent stripping of dura

II. Care of the unconscious a. Ryle's tube aspiration or feeding b. Care of the eyes-padding c. Catheter for drainage of urine d. Change of position to avoid bedsores. Ill. Surgical treatment for extradural haematoma Immediate surgery for removal of haematoma and relief of cerebral compression is a must. Extradural haematoma, in particular, is a neurosurgical emergency and patient survival will depend upon the speed with which the compression is relieved. It is not an exaggeration to state that even if decompression has to be done with unsterile instruments, at the bedside it may be worth the effort. In every neurosurgeon's career, at least one such situation might have occurred and a live patient may justify the means employed. Once consciousness is lost, pupils are dilated and decerebrate rigidity and periodic breathing develop, it may be only a few minutes that may be available to save the life of the patient and one should not wait and waste time. In the case of extradural haematoma the outcome is dependent on the size of the haematoma and the stage in which the patient was taken up for surgery. In the case of acute subdural and intracerebral haematoma it depends on associated brain damage. If the associated brain damage is very severe, patients succumbs to the brain damage (Key Box 45.5 and Fig. 45.2).

Fig. 45.2: Site of temporal burr hole

May occur in people who are on anticoagulant therapy. Mortality- 50-90% Outcome is better if surgery is done within 4 hours. CHRONIC SUBDURAL HAEMATOMA • Common in old people • In the elderly, the distance between the dura and the brain increases because of the shrinkage of the brain. Even a minor trauma can tear the cortical veins resulting in collection of blood • Bleeding is never progressive and the blood in the subdural space slowly compresses the brain causing features of raised intracranial pressure (ICP).

Acute subdural haematoma Impact damage is more as compared to epidural haematoma. There is associated underlying brain injury. Symptoms are due to compression of underlying brain with midline shift, in addition to parenchymal brain injury.

Clinical features • Elderly patients with history of minor trauma • Bilateral headache, mental apathy • Slowness, confusion-later alteration in the level of consciousness may progress to unconsciousness. • Waxing and waning of the level of consciousness is seen in some patients. If such a history is elicited, one should always suspect chronic subdural haematoma.

Causes • Parenchymal laceration bleed • Tom cortical bridging vessel

Diagnosis CT scan or, if feasible, MRI scan are the ideal investigations (cerebral angiography had been used and is still being used in

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some centres where access to the latest imaging facilities are not available). Treatment • BmT hole and drainage of the haematoma usually under local anaesthesia or occasionally under general anaesthesia is often the practised mode of treatment. • At times, the patient may need two or more burr holes to ensure adequate evacuation. • If the brain fails to expand and obliterate the cavity, especially in older people or in persons with a very thick inner membrane, a large craniotomy and wide excision of the subdural membrane has to be carried out to remove the constricting effect. • Adequate bedrest and plenty of fluid administration are also important postoperative measures. RAISED INTRACRANIAL PRESSURE Normal ICP is 8-12 mmHg Measures to reduce the raised ICP Aim is to keep ICP 20 mmHg • Head and elevation • Hyperventilation • Sedation-with or without muscle relaxant • Use of diuretics-furosemide, mannitol • Thermoregulation • Use of barbiturates-thiopentone-reduces brain metabolic rate • Maintaining fluid and electrolyte balance • Seizure control • Steroids in severe head injury are associated with increased mortality and should not be used. FRACTURE SKULL Anterior fossa fracture

I. Fracture cribriform plate can result in CSF rhinorrhoea. 2. Fracture may extend to the orbit-subconjunctival haemorrhage. 3. Olfactory nerve involvement-partial anosmia. 4. Optic nerve may be contused or fracture may involve the optic foramen resulting in partial or total loss of vision. 5. Rarely, 3rd nerve palsy gives rise to dilated pupil.

Middle cranial fossa fracture

1. Epistaxis due to fracture venous/sphenoid sinuses 2. CSF from the ear: Blood mixes with CSF and so, does not clot. 3. 7th nerve palsy 4. Rarely 6th and 8th nerves are also involved. Posterior cranial fossa fracture

I. Extravasation of blood in the suboccipital region causing boggy swelling in the nape of the neck.

2. 9th, I 0th, and 11th cranial nerves may be involved. 3. Battle sign: Discolouration of skin and collection of blooc occur in the region of mastoid process. CSF RHINORRHOEA • There should be a communication between the intradural cavity (subarachnoid space) and the nose. • It indicates tear of the dura mainly in the basal region and a fracture involving paranasal sinuses-frontal, ethmoidal or sphenoidal. • There is always an injury to a small portion of the brain. It (the portion of brain) plugs the tear, preventing the dura from healing. Thus, the rhinorrhoea persists for many days. • This leads to complication, i.e. infection and meningitis. • Two types I. Traumatic: It can be iatrogenic following surgery or can be post-traumatic (62 to 80%). 2. Nontraumatic: It can be due to high pressure (hydrocephalus) or congenital. • Confirmation of CSF rhinorrhoea is on the bed side by following clinical signs. 1. Ring sign: Onto linen, ring of blood with 1 layer ring of clear fluid. 2. Reservoir sign: Gush of CSF in certain position of the head. • �2 transferrin is the most accurate method. Treatment • Acetazolamide 250 mg three times a day • Lumbar drainage can be done • Prophylactic antibiotics • If the rhinorrhoea persists, repair of the dural defect alone, (or) at times with a shunt procedure will be needed. POTT'S PUFFY TUMOUR • This is sub periosteal infection usually caused by osteomyelitis of the underlying skull. • It is common in the frontal region and the frontal bone is commonly involved. • The cause of infection is through frontal sinusitis. • Another common cause of infection of a subpericranial haematoma following needle aspiration. • It can also follow chronic suppurative otitis media. • Pus collects in the subpericranial space and extradural plane, which communicate with each other (dumb-bell type abscess). • It causes a boggy swelling in the frontal region and tenderness over the scalp.

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• Pitting oedema over the scalp is conclusively called Pott's puffy tumour. • Severe headache, vomiting and blurring of vision should clinch the diagnosis. Treatment 1. CT scan to confirm the diagnosis 2. A burr hole and aspiration of pus can be done followed by 6-8 weeks of antibiotics. 3. In chronic cases, the wall of the abscess may have to be removed. The associated osteomyelitic skull bone requires a radical removal under cover of antibiotics.

Foramen of ___.:,,__=+­ Monro

HYDROCEPHALUS Definition This is a condition that occurs due to disturbances of CSF flow and imbalance between CSF production and absorption resulting in the accumulation of CSF and dilatation of ventricles. CSF production CSF is mainly produced by the choroid plexus of lateral ventricles by an active autoregulated process. Daily production: 450 ml-total volume is 150 ml CSF circulation (Fig. 45.3)

l

Lateral ventricle

via foramen of Monroe

3rd ventricle

l

via aqueduct of Sylvius

4th ventricle CSF leaves the 4th ventricle by foramen of Luschka and Magendie to circulate over the convexity where it is finally absorbed over arachnoid granulations. Aetiopathology Hydrocephalus occurs due to two reasons: • If there is overproduction of CSF, or CSF circulation • If there is decreased absorption A. Overproduction: True overproduction is rare and occurs in cases of choroid plexus papilloma. B. Decreased absorption: Failure ofCSF absorption is much more common due to infection and haemorrhage. Other causes being: Structural abnormality occurring in CSF pathway-tumour, congenital malformation such as aqueduct stenosis.

Fig. 45.3: Ventricular system

Types 1. Communicating: Due to obstruction in subarachnoid space. 2. Noncommunicating: Due to obstruction in ventricular system. Clinical features I. Infantile hydrocephalus • Difficulty in delivery of large head (Key Box 45.6) • Craniofacial disproportion • Increase in head circumference more than 2 cm/month • Scalp is thin, shiny and prominent veins • Fontanelles: Bulging and tense especially on crying • Sutures: Open, excessive irritability • Macewen's sign--cracked pot sound on percussing over dilated ventricles. • Inability to retain feeds, mental retardation, delayed milestones, hypothalamic disturbances. • Sun-set sign: Weakness of upward gaze II. Childhood/adult hydrocephalus • By this time fontanelles have closed • Features of increased intracranial pressure: Headache, nausea, vomiting. • l!Titability, indifference, apathy, drowsiness

............................. . KEY BOX 45.6

DIFFERENTIAL DIAGNOSIS OF LARGE HEAD 1 . Megalencephaly lntracranial pressure is normal 2. Chronic subdural Enlargement of parietal haematoma region 3. Cerebral atrophy May cause ventricular enlargement 4. Cerebral tumours

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Manipal Manual of Surgery

• Blindness is not the sign of papilloedema • Blindness is due to ophthalmoplegia • Bradycardia, systemic hypertension, altered respiratory rate is due to distortion of brainstem. Untreated cases also develop unilateral or bilateral abducens palsy or upward gaze palsy. Treatment Aim 1. To decrease CSF production by using phannacological agents: • Acetazolamide • Furosemide • lsosorbide • Glycerol 2. Direct removal of cause of obstruction 3. Diversion of CSF to another viscous for reabsorption by means of various shunt procedures. SHUNTS Ventriculoperitoneal shunt and ventriculoatrial shunt (Figs 45.4 and 45.5) Complications of shunts (Key Box 45.7)

............................. KEY BOX 45.7

1. 2. 3. 4. 5. 6. 7.

.

COMPLICATIONS OF SHUNTS

Shunt obstruction Shunt infection Seizures Extracerebral fluid collection Subdural haematoma Spontaneous pneumocephalus Ascites

TUMOURS Eight percent of all primary cancers arises in the CNS. Ir adults, it constitutes the sixth largest group of cancers and ir children the CNS is the commonest site for solid tumours. PEARLS OF WISDOM

Most adult brain tumours are supratentorial but 60% of tumours in children are infratentorial and arise in the posterior fossa. Presenting feature 1. Progressive neurological deficit: In up to 2/3rds of the patients, a focal motor weakness is the most common presentation. This deficit arises from the direct neuronal damage or are due to compression on brain or cranial nerves by the tumour. Sudden change may be due to haemorrhage into a pre-existing tumour. 2. Headache: It occurs in over 50% of patients caused by raised intracranial pressure (ICP). Only I 0% patients have the classic presentation of headache with early morning worsening, associated with nausea and a temporary relief by vomiting. 3. Generalised or focal seizures occur in 25%. Focal seizures may help to localise the site of tumour. A high index of suspicion is required for patients past the second decade with recent onset epilepsy. 4. Other presentations include visual disturbances, visual field defects, mental changes, etc. depending on the site of tumour. Investigations 1. CT scan: The best initial investigation. This shows where the tumour alters the attenuation of the X-ray beam as it passes through the brain. It also shows distortion of the ventricular system or obliteration of the pattern of the sulci. 2. MRI: Often gives extra information and may eventually supersede CT completely. It is the investigation of choice for imaging the posterior fossa and the spine as it can 'see through' bone. 3. X-rays are only of anecdotal interest picking up incidental calcifications or erosion ofparts of the skull. Newer imaging modalities including SPECT and PET scans are increasingly being used in higher centres. SPECIFIC TYPES OF BRAIN TUMOURS Gliomas

Fig. 45.4: Ventriculoperitoneal

shunt

Fig. 45.5: Ventriculoatrial

shunt

• Astrocytoma • Oligodendroglioma • Mixed tumours

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Astrocytoma: WHO classification Pilocytic Grade I Diffuse Grade II Anaplastic Grade III Grade IV Glioblastoma multiforme • This is the most common primary brain tumour arising from the supporting glial cells and diffusely infiltrates brain tissue early on. • Two grading systems are available: WHO and St Anne/ Mayo. Grade I and Grade 2 (low grade) tumours are slow growing and compatible with good quality survival. Grades 3 and 4 are rapidly growing. Grade 4 tumours are also called glioblastoma multiforme are highly radio and chemo­ resistant with median survival of only 12 months even with optimal treatment. Even low grade astrocytomas may evolve over time into secondary glioblastomas. Glioblastoma Multiforme • Most common in 5th and 6th decade. • Treatment includes surgery to confom the diagnosis and achieve a macroscopic excision, followed by a high dose (60 gy) of irradiation. • Chemotherapy wafers impregnated with carmustine must be inserted. Oligodendroglioma • This type of glioma is usually a slow-growing tumour and over half arise in the frontal lobes. • There may be a history of epilepsy or even focal neurological signs of many years' duration. • They may show calcification, both microscopically and macroscopically. Ependymoma • These glial tumours arise from cells that line the ventricles of the brain and the central canal of the spinal cord. • They are most common in the fourth ventricle in children and young adults where they block CSF flow and often present with hydrocephalus. Embryonal tumours • Primitive neuroectodermal tumours (PNETs) are a group of highly malignant tumours of which the cerebellar PNET or medulloblastoma is the archetype. • Commonest in children and young adults. It may originate from primitive cell nests which have undergone malignant transformation. Medulloblastoma is the most common brain tumour in children. • The patient presents with truncal ataxia, headache, vomiting and sometimes diplopia. All PNETs are prone to spread within the CNS producing 'sugarcoating metastasis' which are best seen on MRI of the spine.

Schwannoma (neurilemmoma) • Peripheral neurons get their myelin sheaths from Schwarn cells. Occasionally, these form slow growing benig1 tumours on cranial or spinal nerves. • The cranial nerve most affected is the vestibular divisioi of the 8th cranial nerve and the tumour is then usuall: called acoustic neuroma. PEARLS

OF

WISDOM

Even though it does not arise from the acoustic division and is not a neuroma, it is still called an acoustic neuroma.

• It causes progressive deafness, hydrocephalus and ataxia It is slow growing. Less often it may involve the trigemina or the vagus nerve. Meningioma • Eighty percent are supratentorial • These tumours arise from the arachnoid layer of the meninges and the arachnoid villi. They are commonest over the falx and the convexity of the skull but rarely may also arise from the skull base (especially from the sphenoid wing and olfactory groove) or inside the lateral ventricle. • Commonest in middle-aged women and may occur at the site of a previous radiation field. Most are benign and slowly growing. Signs occur based on the site of tumour (Key Box 45.8). • They tend to provoke endosteal hypertrophy or exostosis of the overlying skull and is still occasionally detected on an incidental skull film or even by palpation of the skull. Patient with neurofibromatosis type 2 often have multiple meningiomas. Rarely, they are malignant. Pituitary tumours • • • • • •

I 0-15% of all intracranial tumours Majority are benign adenoma Prolactinoma-30% Nonfunctioning adenoma-20% GH secreting adenoma-15% ACTH secreting adenoma-10%

. . . . . . . . . . . . . . . . . . . . . . . . . .. . . . . KEY BOX 45.8

• • • • • •

SITES OF MENINGIOMA Convexity of skull 20% Parafalcine arc 20% Sphenoid ridge 10% Tuberculum sella 10% Olfactory groove 10% 2-5% Ventricle

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Manipal Manual of Surgery

• May produce mass effect-bitemporal hemianopia or cranial nerve dysfunction. • Endocrine dysfunction such as galactorrhoea, primary/ secondary amenorrhoea, Cushing's syndrome, acromegaly. • Pituitary apoplexy-results in sudden onset of headache, visual loss, ophthalmoplegia and altered conscious level. Caused by haemorrhagic infarction of a pituitary tumour. Other tumours They are pineal region tumours, pituitary adenomas, craniopharyngiomas, choroid plexus tumours, etc. Metastases One-fourth of all cancer patients have intracerebral metastases at the time of death. Common primary sites include the bronchus (50%), breast (15%), and melanoma (10%). Treatment They can be divided into medical and surgical line of treatment. I. Medical • Acutely raised intracranial pressure (ICP) is treated with IV mannitol 0.5-1 g/kg body weight. • Hydrocephalus can be relieved using a CSF diversion system (closed external ventricular drainage or a ventriculoperitoneal shunt). • Seizures are treated with lorazepam and phenytoin commonly and maintained on phenytoin. • Corticosteroids, especially dexamethasone 4 mg qid is given to reduce symptoms of raised ICP, which may make surgery easier. II. Surgery • This is the mainstay of treatment. The aim of surgery is to obtain a complete tumour excision without producing a neurological deficit. • This is not always possible due to the site of the tumour, in which case compromises must be made and a debulking procedure is done. • Any residual tissue may be observed or treated with adjuvant radiotherapy. • Stereotactic guided surgery is now a well-established concept in brain surgery allowing more targeted treatment of the lesion with minimal surrounding damage. III. Radiotherapy • Jntracranial tumours are relatively radioresistant, and radiotherapy is primarily a palliative treatment.

IV. Chemotherapy • No definite benefit of chemotherapy for the treatment oJ brain tumours. Temozolomide is a promising new drug for the treatment of brain tumours. Outcome Surgery offers good prospects for the treatment of benign brain tumours such as meningioma or pituitary adenoma, but outcome of treatment of malignant tumours is still poor. TRIGEMINAL NEURALGIA • Superior cerebellar artery or multiple sclerosis plaque can cause this condition. • Severe episodic lancinating facial pain occurring in the distribution of V cranial nerve. • Vascular compression of the nerve near the root entry zone, multiple sclerosis may be causative factors. • Investigation-MRI Treatment • Carbamazepine or gabapentin • 75% will not respond to medical treatment. • Surgery: Refractory cases 1. Percutaneous glycerol injection, radiofrequency ablation, thermocoagulation, balloon compression, local nerve block. 2. Microvascular decompression via craniotomy 3. Stereotactic radiosurgery BRAINSTEM DEATH Irreversible loss of consciousness, loss of brainstem reflexes and apnoea. Diagnosis of brainstem death is done in three stages: 1. Identification of the cause of irreversible coma. 2. Exclusion of reversible causes of coma. 3. Clinical demonstration of absence of brainstem reflexes. Brainstem reflexes • Pupillary reaction to light, corneal reflexes, vestibular ocular reflex, cough reflex, gag reflex, motor response to pain. • Apnoea test-apnoea despite a CO2 increase to> 6.65 kPa or 50 mrnHg. • All reflexes must be absent and are tested independently twice by 2 doctors.

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A FEW CT SCANS OF HEAD INJURIES (Fig. 45.6 to 46.14)

Fig. 45.6: Right temporo-parietal extra- Fig. 45.7: Right temporal extradural Fig. 45.8: Postoperative scan-no EDH. Pos1 dural haematoma(EDH) showing mass haematoma right temporal craniotomy with pneumocepha· effect with midline shift with subfalcine lous herniation

Fig. 45.9: Left frontoparietal acute subdural haema- Fig. 45.10: Traumatic right more than left toma. Left frontal and temporal nonhaemorrhagic frontoparietal and anterior interhemispheric contusion with subfalcine herniation with midline subarachnoid haemorrhage shift

Fig. 45.11: Diffuse axonal injury­ right frontal, left temporal contusion, right frontoparietal region-subara­ chnoid haemorrhage and intraven­ tricular haemorrhage

Contributed by Dr. Sunil Upadhyay - Asst. Prof., Dept. of Neuro Surgery, KMC, Manipal

Fig. 45.12: Right temporal depressed fracture with Fig. 45.13: Left frontoparietal hypodence Fig. 45.14: Left FTP decompressive area suggestive of left middle cerebral craniectomy brain herniation outside the temporal EDH with pneumocephalous artery stem infarct skull

Manipal Manual of Surgery

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VERY IMPORTANT WISDOM LINES IN HEAD INJURY

WHAT IS NEW IN THIS CHAPTER?/RECENT ADVANCES

• If an exploratory burr hole has been decided upon, it should be done on the side of the initially dilated pupil.

• All topics have been updated • Trigeminal neuralgia, brainstem death have been included.

• Bradycardia is due to raised intracranial pressure. • If hypotension responds to volume replacement in trauma centre in a comatose patient, the coma is most likely not due to head injury. • Admit the patient if there is fracture temporal bone. • Hypoxia is an important cause of cerebral oedema which worsens the level of consciousness. • Steroids in severe head injury are associated with increased mortality and should not be used. • CSF rhinorrhoea indicates tear of the dura mainly in the basal region and a fracture involving paranasal sinuses. • Glasgow Coma Score of less than 8 means patient is in coma.

MULTIPLE CHOICE QUESTIONS 1. Following is not the feature of Glasgow Coma Scale: A. Eyes opening B. Motor response C. Verbal response D. Sensory response 2. Closed head injury include following except: A. Head injury with fracture skull B. Head injury with black eye C. Head injury with facial nerve palsy D. Head injury with CSF rhinorrhoea 3. Which are the following is included under secondary lesions following head injury? A. Diffuse neuronal damage B. Contusions C. Lacerations D. Swelling 4. Post-traumatic amnesia is a feature of: A. Raised intracranial tension B. Fall from a height C. Fracture skull D. Cerebral concussion 5. Which is an important cause of brain swelling following head injury? A. Infection B. Oedema C. Acidosis D.Bleeding

6. The cause of extradural haematoma is bleeding from: A. Venous sinuses B. Cavernous sinus bleeding C. Basal veins bleeding D. Middle meningeal vessels bleeding 7. Lucid interval is typically seen in: A. Extradural haematoma B. Acute subdural haematoma C. Chronic subdural haematoma D. Pontine haematoma 8. Which nerve is paralysed after herniation of temporal lobe in extradural haematoma? A. Occulomotor nerve B. Ophthalmic nerve C. Trigeminal nerve D. Facial nerve 9. Pupillary dilatation following head injury is due to ischaemia of the third nerve caused by: A. Middle cerebral artery B. Posterior cerebral artery C. Inferior cerebral artery D. Anterior cerebral artery 10. Following are definite indications for admission in a head injury patient except: A. Fracture skull B. CSF rhinorrhoea C. History of unconsciousness D. Scalp bleeding

ANSWERS 1 D

2 D

3 D

4 D

5 B

6D

7 A

8 B

9 B

10 D

46 Principles of Radiology • • • • • • • •

• • • • • • • • •

Barium swallow Barium meal Barium meal follow through Barium enema Enteroclysis Angiography Splenoportography Sialography

Computed tomography Ultrasonography Magnetic resonance imaging Peripheral venography Myelography lnterventional radiology PET scan Virtual colonoscopy What is new?/Recent advances

Introduction These radiological investigations have become an integral part of the management of the patients. A clear understanding of these will help the students to read the images accurately, interpret well and score good marks. BARIUM STUDIES This is the study of the gastrointestinal tract by instillation/ ingestion of barium suspension made up of/made from pure barium sulphate. BARIUM SWALLOW (Fig. 46.1) It is the contrast study from the oral cavity up to the fundus of the stomach. Indications • Dysphagia and obstruction • Odynophagia • Assessment of mediastinal masses • Motility disorders of oesophagus-achalasia, scleroderma. Relative contraindications • Tracheo-oesophageal fistula • Perforation Procedure • One mouthful of contrast media is given and the act of deglutition is observed fluoroscopically. After a mouthful of barium, films are exposed to the region of interest.

Fig. 46.1: Barium swallow: Opacified thoracic oesophagus showing an intraluminal mass with mucosal irregularity in its lower third suggestive of malignancy

Interpretation of study 1. Malignant obstructions are seen as annular constrictions, shouldering cranial and caudal to the lesion, mucosa! destruction, ulceration and fistulae formation. 2. Benign strictures are long segment narrowings with no mucosa! abnormalities.

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3. Achalasia cardia is evident as 'rat tail' appearance of the lower end of the oesophagus, with gross dilatation of the oesophagus proximally and thin streaks of contrast entering the stomach. 4. Scleroderma shows dilatation, atonicity, poor or absent peristalsis and free gastro-oesophageal reflux. BARIUM MEAL (Fig. 46.2) This is the radiological study of oesophagus, stomach, duodenum and proximal jejunum. Indications • Symptoms of vomiting, epigastric pain, heart burn, dyspepsia • Upper abdominal mass • Gastrointestinal haemolThage • Gastric or duodenal obstruction • Malignancies Contraindications • Suspected perforation • Suspicion of aspiration • Large bowel obstruction Procedure An undiluted barium suspension is given and deglutition is seen under fluoroscopy. Once barium reaches the stomach, the patient is rotated so as to coat the entire stomach and filming is done. More barium is given to distend the stomach wall. Filming is done as contrast enters the duodenum and opacifies proximal jejunum. Interpretation of study 1. Hiatus hernia is evident as presence of the stomach above the oesophageal hiatus. In addition gastro-oesophageal

reflux will be evident. Mucosa! ulceration and stricturei may be demonstrable in long-standing cases. 2. Gastric and duodenal ulcers appear as projections frorr the nonnal contour with pooling of contrast. Benign ulcen usually project out and have the mucosa! folds radiating up to the edge of the ulcer. Deformity of the stomach and duodenal cap are seen in chronic stages. 3. Bezoars of stomach are seen as radiolucent masses in the stomach and the barium fills the crevices between the particles fonning a characteristic appearance. 4. Infantile hypertrophic pyloric stenosis: Thin streak of barium is seen extending across pylorus-indentation of barium-filled antrum is seen. BARIUM MEAL FOLLOW THROUGH It is the radiographic examination of the GIT-oesophagus, stomach, small bowel and ileocaecal junction, by oral administration of contrast media. Indications • Symptoms of small bowel disease such as diarrhoea, abdominal pain, weight loss, Crohn's disease. • Small bowel obstruction (chronic) • Gastrointestinal bleeding • Palpable mass possibly involving the small bowel • Malabsorption Contraindications • Colonic obstruction • Suspected perforation Procedure A small mouthful of barium is given after which the stomach is studied and films are taken. 500-800 ml barium is then given to the patient and filming is done after 15-20 minutes to demonstrate the jejunum and the proximal ileum. Subsequently, films of the ileum and ileocaecal junction are taken. Interpretation of study 1. Intestinal malignancies such as lymphomas are evident as strictures which may be short or long segment with or without proximal dilatation. The mucosa shows irregularity and ulcerations. Fistulous communications may be evident. Displacement of bowel loops in large extraluminal masses may be seen. 2. Inflammatory strictures are evident as sites of nalTowing with mucosa[ abnormality and ulcerations, proximal dilatation and mucosa! fold thickening.

Fig. 46.2: Barium meal: Contrast opacified stomach demons­ trating a projection from lesser curvature due to a benign gastric ulcer (niche)

Complications • Perforation of the bowel • Aspiration

Principles of Radiology

BARIUM ENEMA This is the radiographic study of the large bowel by administration of contrast media through the rectum. Types Single contrast barium enema and double contrast barium enema. Indications 1. Change in bowel habit 2. Melaena 3. Mass suspected to be arising from colon 4. Features of large gut obstruction (subacute) Contraindications 1. Toxic megacolon 2. Pseudomembranous colitis 3. Rectal biopsy done recently (procedure withheld for 7 days) Procedure • Bowel is prepared with low residue diet, purgation and cleansing water enema. High density barium suspension is allowed to flow up to the ileocaecal junction and reflux into the terminal ileum. Single contrast filming is done. The patient is asked to evacuate the barium and a post evacuation film is taken. Once barium is evacuated properly, air insufflation is carried out so as to distend colon up to the ileocaecal junction. • Filming is done to demonstrate the double contrast of large bowel with additional spots of hepatic, splenic flexures and rectosigmoid junction in oblique positions so as to open up these regions.

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5. Malabsorption Dilution of barium, segmentation of the column of barium 'Moulage sign' (barium in a featureless tube) and jejuna dilatation are the findings. SMALL BOWEL ENEMA OR ENTEROCLYSIS (Fig. 46.3) (Key Boxes 46.1 and 46.2) This is the radiological study of the small bowel (from jejunurr to the ileocaecal junction) by intubation of the jejunum and instillation of contrast media through the tube. Procedure • Bilbao Dotter tube is inserted with the guide wire through one of the nostrils and advanced caudally with the swallowing action till the tip reaches the stomach. The tube is then advanced through the antrum of the stomach to the pyloric canal. Then it is advanced under fluoroscopic guidance to about 4-5 cm distal to the Trietz ligament (duodenojejunal junction).

Interpretation of study A few examples are: 1. Ulcerative colitis • Loss of haustral pattern • Fine granularity of mucosa • Fine ulcerations resulting in spiking of colonic surface • Strictures • Pipe stem colon, increase in presacral space 2. Malignant lesions • Circumferential/eccentric growth narrowing the lumen • Hold up of barium proximal to the lesion • Mucosa! abnormality-ulcerations 3. Tuberculosis Ileocaecal region is the commonest site. Deformed, elevated caecum, stricture and ulceration involving ascending colon and ileum. 4. Crohn's disease Multiple ulcerations, thickening and distortion of valvulae conniventes, short or long strictures, cobblestone pattern and separation of bowel loops are the features.

Fig. 46.3: Small bowel enema showing stricture terminal ileum

mtlMIIIIIIWIIIIIII� • • • • •

INDICATIONS Mechanical obstruction GIT bleeding Tumours of small intestine Unexplained abdominal pain Diarrhoea

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•=t • • • • •

CONTRAINDICATIONS Complete obstruction Suspected perforation Massive dilatation of small bowel Duodenal obstruction Gastrojejunostomy

�, �

• 200 ml barium suspension is injected at a rate of 75 ml/ min followed by 5% of rnethylcellulose at a rate of 100 ml/min. The head of the barium column is followed with intermittent fluoroscopy and films exposed wherever necessary. lleocaecal spot films are taken when the junction is opacified and distended. Interpretation of study 1. Normal small bowel shows a decrease in calibre from jejunum to ileum and the change of prominent valvulae conniventes to featureless ileum is evident. 2. Malignancies and lymphomas show evidence of strictures, proximal dilatations and mucosa! abnormality. Large mesenteric nodal masses displace the bowel loops. 3. Strictures and ulceration of terminal ileum: Dilatation of the segment proximal to the narrowed segment and conical shrunken caecum are seen in ileocaecal tuberculosis. In later stages, ilea! strictures, fistulae, etc. may be seen.

Fig. 46.4: Arch aortogram: Contrast in the arch of aorta demonstrating the major vessels arising from it

Complications • Perforation • lnspissation of barium • Transient bacteraemia ANGIOGRAPHY (Figs 46.4 and 46.5) Definition This is the study of blood vessels by injection of a contrast medium containing iodine into the vessel. Indications 1. Primary vascular diseases such as vaso-occlusive disease, aneurysm, arteriovenous malformation (AVM). 2. Vascularity assessment of a tumour. 3. Congenital vascular conditions such as coarctation. 4. Percutaneous interventional vascular procedures. Contraindications 1. Bleeding tendencies 2. Skin infections at site of entry 3. Cardiovascular disease such as recent myocardial infarction, overt congestive cardiac failure. 4. Hepatic failure

Fig. 46.5: Aortogram: Contrast in the thoracic aorta showing narrowing

Procedure • Local anaesthesia at site of puncture is preferred except in children or restless patients, wherein general anaesthesia is preferred. Using a Seldinger needle the artery is punctured. • The catheter of appropriate dimension is placed into the artery and negotiated into the desired vessel to be studied. Contrast is injected and filming is done. Puncture sites Femoral artery, axillary artery and brachia! artery. Interpretation of study 1. Aneurysms are seen as focal dilatations of vessel or projecting from the main vessel through a neck.

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2. Tumour vessels show abnormal branching pattern, vascular encasement, displacement, arteriovenous shunting and pooling of contrast in the lesion. 3. AVM shows evidence of a dilated feeding artery/abnormal blush and early draining vein. 4. Vascular occlusions are seen as abrupt or gradual tapering of vessel with collateral supply distally. Complications • Damage to arterial walls at the site of puncture • Severe hypotensive reactions • Thrombosis of arteries, catheter clot embolus, haematoma at puncture site • Vagal inhibition • Allergic reactions to contrast • Damage to nerves and to organs SPLENOPORTOGRAPHY (Fig. 46.6) This is the contrast study of the portal venous system by percutaneous splenic puncture. Indications • Demonstration of the anatomy of the portal system in patients with portal hypertension prior to surgery • Check the patency of a portosystemic anastomosis Contraindications • Abnormal prothrombin time • Ascites Procedure Splenoportography needle is introduced into the spleen in the midaxillary line under local anaesthesia. After confirming the

placement of needle in the splenic pulp, injection of iodine containing contrast media is done and films are taken to includ, the splenic vein, portal vein and the portal radicles in liver. Interpretation of study • Contrast injected into the splenic pulp drains into the hilun through the splenic radicles and then through the spleni1 vein and portal vein into liver. • Thromboses are seen as filling defects in the contras opacified splenic and po1tal vein. • In severe pottal hypertension multiple collaterals are seer as branching channels from the normal pathway. Complications l . Haemorrhage 2. Perforation of adjacent structures (pleura, colon) 3. Splenic rupture 4. Infection SIALOGRAPHY This is the contrast opacification of the duct and the glandulat acini by cannulating the ducts of salivary glands. Indications Pain, recurrent swelling Contraindications Acute infection or inflammation Procedure • The orifice of the parotid duct or submandibular duct is cannulated depending on the indication. Iodine-containing contrast medium is injected. • Injection is terminated immediately, if any pain is experienced. • Films are taken to demonstrate the duct and the glandular branching pattern. • Post-secretory films are taken 5 minutes after administration of a sialogogue to demonstrate sialectasis. Interpretation of study 1. Stones in the gland are identified as filling defects and those in the ductal system result in proximal obstruction and dilatation. 2. Glandular enlargement due to inflammation result in pooling of the contrast injected. 3. Tumours of the salivary gland result in irregular filling defect with duct distortion and pooling of contrast medium.

Fig. 46.6: Splenoportography showing dilated splenic vein

Complications • Pain • Damage to duct orifice • Rupture of ducts • Infection

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COMPUTED TOMOGRAPHY (CT) This is an imaging procedure where detailed infonnation is obtained from thin sections in collimated X-rays. Indications • Structural evaluation of intracranial lesions (Fig. 46.7) • Detailed evaluation of lung, mediastinal pathologies • Intra-abdominal and pelvic masses where exact site of origin and relation to adjacent structures can be evaluated. • Extra-osseous and soft tissue extension of bone tumours. • Vascularity of the normal organ and the abnormal tissue can be evaluated and compared. Contraindications (relative) • Pregnancy • Restless patients

Interpretation of images • Structures imaged appear densely white to densely black depending on the absorption of X-rays and the emerging resultant X-rays which are detected. The composite picture is actually a collection of Hounsfield numbers. Each Hounsfield number being assigned a specific shade of grey, thus producing a picture that might be easily understood. Some of the common densities to be encountered in practice are as follows (Hounsfield units = HU): Air -1000 HU Fat -50 to -100HU Water OHU Oto+3HU CSF +22 to +32HU W hite matter Grey matter +36 to +46HU +60 to +80 HU Clotted blood Calcification bone +80 to+ 1000 HU • In order to increase the contrast that may exist between the structures in the body, intravenous contrast (iodine containing) is administered. Certain tissues show enhancement of their density and various pathologies also show fairly characteristic contrast uptake patterns (Figs 46.8 to 46.11). • In abdominal scanning, oral contrast is administered to the patient before the procedure, to enable the operator to accurately separate the bowel loops from the other intra­ abdominal structures. • The advantages of CT over conventional radiology are that it can visualise extremely small pathology, not evident on conventional films, is cost-effective as multiple X-ray films and procedures can be avoided. It is noninvasive and the radiation levels applied to the patient are extremely low. ULTRASONOGRAPHV (Figs 46.12 and 46.13)

Fig. 46.7: Cranial CT: Hypodensity of the left (L) frontoparietal

cerebral parenchyma suggestive of a (L) middle cerebral artery territory infarct

Principle This imaging modality is based on the piezoelectric effect which is the prope1ty of certain substances to convert electrical

Figs 46.8 to 46.10: CT scan axial section through liver shows large mass in plain scan. Brightly enhancing in arterial phase of contrast study. Finally wash-out of contrast in portovenous phase which is a classical feature of hepatocellular carcinoma in multiphase contrast CT scan

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Fig. 46.13: Ultrasound of liver shows hydatid cyst which is anechoic with multiple daughter cysts Fig. 46.11: CT scan of abdomen: Contrast-enhanced scan demonstrating a hypodense lesion in right lobe of liver posteriorly due to an abscess

energy to sound energy. These are the active portions of the ultrasonic transducers. The commonly used substance in the transducer is lead zirconate titanate (PZT). Applications • Ultrasonic beam of high frequency gives excellent resolu­ tion images of only superficial structures. This is used for study of musculoskeletal system, joints, thyroid, scrotum, etc. • For imaging deeper structures of abdomen, a low frequency probe with greater penetrancy is used. Interpretation of images • Images are dependent on the intensity of echoes received back by the transducer.

• Structures which reflect all the sound waves back an depicted as bright echoes and termed hyperechoic. • Structures which reflect moderate level of sound wavei appear as unifonn grains and are termed isoechoic. • Fluid filled structures which transmit all the sound waves. do not reflect any echoes and are termed hypoechoic. • The reflection of sound waves in the form of echoes dependE on the density of the organ and the transmission of sound through the same. Advantages of ultrasonography I. It is a cost-effective investigation 2. It is widely available 3. Noninvasive 4. Owing to the relatively small size of the apparatus, it is fairly portable, and can thus be brought to the bedside of the moribund patient. 5. It does not involve the use of ionising radiation, and can therefore be safely used in a pregnant patient and can be repeatedly used as a follow-up modality. Limitations of ultrasonography I. Its use is limited in thorax 2. Limited use in the abdomen when there is gaseous distension 3. Operator expertise is all important 4. It cannot image bone MAGNETIC RESONANCE IMAGING (MRI) Principle

Fig. 46. 12: Ultrasound of liver and gall bladder demonstrating an isoechoic mass lesion occupying the lumen of gall bladder which was due to malignancy

Certain atomic nuclei, which possess unpaired protons or neutrons, have an inherent spin. The nucleus is positively charged and therefore creates a small magnetic field around itself, when it spins. The human body contains in abundance such spinning nuclei in the atoms of hydrogen which is found in water and lipids (Figs 46.14 to 46.18).

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Fig. 46.14: MRI: T1 and T2 weight axial and coronal scans of normal brain

Fig. 46.15: Soft tissue sarcoma involving the muscle of thigh. The lesion is hyperintense in appearance

Fig. 46.16: Carcinoma tongue. MRI image shows hyperintense lesion is involving the posterior and base of the tongue on the left side. The other bright structures seen bilaterally are parotid glands

Fig. 46.17: lntersphincteric fistula - axial section of MRI pelvis showing hyperintense fistula (arrow)

Fig. 46.18: Axial section of pelvis shows wall thickening of the rectal wall (arrow)

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Principles of Radiology

When the tissues containing these nuclei are within a strong magnetic field, the nuclei tend to align themselves along the lines of the force. The spinning protons now tend to precess, i.e. wobble about the axis of the main magnetic field. Now a radiofrequency (RF) is applied, being of the same frequency as the processing but at right angles to the main magnetic field. This excites the protons at low energy states into higher energy states. Thus, an absorption of energy takes place, which is used, as the excited protons 'relax' back to their original energy level when the radiofrequency is switched off. The relaxation of protons back to equilibrium and lower energy state is termed spin-lattice relaxation or longitudinal relaxation. It is exponential and referred to by the time constant T l . When the RF pulse is applied the protons process together in synchronism or in phase with each other. During relaxation, however, they go quickly out of phase due to small variations in local magnetic fields. This loss of phase is termed spin-spin relaxation or transverse relaxation. It is also an exponential and referred to by the time constant T2. Depending on the type of tissue under study, the T 1 and T2 relaxation times will differ, thus giving rise to differences in the image. The MR] image depends upon 4 main factors: 1. The T 1 relaxation time 2. The T2 relaxation time 3. The proton density 4. The blood flow Depending on the characteristics of the above four parameters, the signal intensity of the image will vary, thus deciding the appearance that any given tissue will finally cast. Advantages of MRI 1. It is noninvasive 2. It does not involve the use of ionising radiation. Hence, it is safe in that respect. 3. It gives high intrinsic contrast 4. Direct transverse, sagittal and nonnal imaging possible 5. No bone/air artefact 6. It has no known biological hazard Disadvantages of MRI 1. The imaging time is long. Hence, movement of the patients may produce artefacts. 2. Due to variety of protocol options during scanning, the final image is highly operator-dependent and this requires expert technical staff. 3. Expensive 4. Poor bone and calcium detail 5. Patients with pacemakers, metallic implants and critically ill patients cannot be scanned. PERIPHERAL VENOGRAPHY This is the contrast study of the veins of the limbs.

Indications 1. Deep venous thrombosis 2. Demonstration of incompetent perforators 3. In case of suspected venous obstruction by tumour 4. To outline venous malformations. Procedure • Tourniquet is applied just above the ankle or elbow t, occlude the superficial venous system • A 19 g butterfly needle is inserted into a distal vein arn contrast media (iodine containing) is injected • Filming is done up to the region of interest Interpretation of study • Thrombosis of deep veins are seen as filling defects • Incompetent perforators are evident as refluxing of contras from deep to superficial system and prominent tortuou: collaterals • Displacement of the opacified veins are seen at sites o tumour. Tumour encasement of veins and thrombosis alsc may be demonstrable. Complications • Complications due to contrast media • Thrombophlebitis • Tissue necrosis due to extravasation of contrast • Pulmonary embolus due to dislodged clot MYELOGRAPHY (Fig. 46.19) Definition This is the study of the spinal canal and spinal cord by injection of contrast medium into the thecal subarachnoid space. Indications Suspected intraspinal or nerve root abnormalities Contraindications • Papilloedema • Recent lumbar puncture, since CSF collected outside the thecal space may be tapped. • Previous adverse reactions to intrathecal injection of contrast medium. Procedure • Lumbar puncture is most preferred. The puncture is done at L2-L3 level.

Fig. 46.19: Thoracic myelogram: Contrast in the thecal sac showing a total interruption in the flow of contrast cranially due to an intradural mass lesion

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• Once good flow of CSF is obtained, low osmolar nonionic contrast media is injected. • Filming of lumbar region is done and with adequate tilt towards head end, thoracic and cervical regions are also filmed. • Cisternal puncture is done when lumbar puncture is a failure. Interpretation of study • Disc lesions are seen as impressions on contrast column and cut off the nerve roots at a particular level. • Intraspinal tumours cause widening of the contrast column with or without total obstruction to flow. • Vertebral and paravertebral lesions result in displacement of the contrast column and cut off at a particular level. Complications • Headache • Convulsions-rare • Transient increase in lumbar or sciatic pain • Hypotension • Spinal cord damage in cisternal puncture INTERVENTIONAL RADIOLOGY The role of radiology was limited as only a diagnostic art until mid 1970s. However, now radiology has taken on an exciting new aspect and has entered the field of interventional radiology. Two main types of interventional procedures: Vascular and nonvascular. Vascular 1. Angioplasty: This is performed by the use of intraluminal balloon catheters and may be performed for almost any diseased vessel in the body. The more commonly treated vessels are the coronaries, renal arteries, peripheral limb vessels, etc. 2. Embolisation: This procedure is performed either preoperatively to reduce the vascularity of certain t umours, or as a curative treatment for vascular malformations, aneurysms, GI bleeding, etc. Temporary embolisation may be achieved by using gel foam or autologous clots and pern1anent embolisation by using balloons, steel coils, ethanol, etc. Inferior vena cava (IVC) umbrella placement, IVC membranotomy are also done. 3. Intravascular ultrasound: The use of ultrasound inside a blood vessel to visualise the interior of the vessel in order to detect problems inside the blood vessel. 4. Stent placement: A tiny, expandable coil, called a stent, is placed inside a blood vessel at the site of a blockage. The stent is expanded to open up the blockage.

•

Important types of stents and stent selection: Self-expanding stents are compressed within a catheter device and released by removing a constraining sheath or membrane. The final diameter of the stent is a function of the outward elastic load of the stent and the inward recoil of the elastic wall. Balloon expandable stents are mounted on angioplasty balloons in a compressed state and then deployed by balloon inflation. These stents retain the diameter imposed by angioplasty balloon unless externally compressed.

5. Foreign body extraction: The use of a catheter inserted into a blood vessel to retrieve a foreign body in the vessel. 6. Needle biopsy: A small needle is inserted into the abnormal area in almost any part of the body, guided by imaging techniques, to obtain a tissue biopsy. This type of biopsy can provide a diagnosis without surgical intervention. 7. Blood clot filters: A small filter is inserted into a blood clot to catch and break up blood clots. 8. Injection of clot-lysing agents: Clot-lysing agents, such as tissue plasminogen activator (tPA) are injected into the body to dissolve blood clots, thereby increasing blood flow to the heart or brain. 9. Catheter insertions: A catheter is inserted into large veins for giving chemotherapy drugs, nutritional support, and haemodialysis. A catheter may also be inserted prior to bone marrow transplantation. 10. Cancer treatment: Administering cancer medications directly to the tumour site. Nonvascular 1. Hepatobiliary: Percutaneous transhepatic biliary drainage (PTBD) is widely accepted in cases of biliary obstruction, along with percutaneous biliary calculus removal. Biliary stent placement across a malignant lesion is widely being done in inoperable cases as a palliative procedure. 2. Urinary: Percutaneous nephrostomy, percutaneous stenting and percutaneous nephrolithotomy are being performed. 3. Guided biopsy: Fluoroscopy, ultrasound or CT-guided biopsy of various lesions are now part of routine technique. 4. Other interventional procedures: Percutaneous gastrostomy, catheter drainage of abscesses, pseudocysts, ultrasound­ guided intrauterine foetal surgeries, etc. Advantages of interventional procedures 1. Patient compliance is high as surgery is avoided 2. Cost-effectiveness is high

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• PET scan is a medical imaging technique that combines computed tomography (CT) and nuclear scanning. It is used to determine the metabolic or biochemical activity in the brain, heart and other organs by tracking the movement and concentration of a radioactive tracer injected into the blood stream. • A camera records the tracer's signal as it travels through the body and collects information about the organs. A computer then conve1ts the signals into 3D images of the

examined organ, which provide a clear view of a1 abnormality. • One of the main differences between PET scan and othe imaging tests like CT or MRI is that the PET scan reveal: the cellular level metabolic changes occun-ing in an orga1 and functional changes at cellular level. A PET scan ofter detects these changes very early, whereas CT or MRI detec changes a little later as the disease begins to cause structura changes in organs or tissues. • Positron emission tomography (PET-CT) constitutes majo1 progress in management of cancer patients for the initia diagnosis, staging and follow-up of various malignancies PET-CT is also useful in the follow-up of patients followinf chemotherapy or surgical resection of tumour, since, mos of them have a confusing appearance at CT or MR imaginf due to postoperative changes or scar tissue (Figs 46.2( to 46.25).

Fig. 46. 20: Carcinoma breast with metastasis

Fig. 46. 21: Carcinoma oesophagus

3. r nfection rates are low 4. Can be repeated as it is relatively noninvasive 5. Certain untreatable conditions are treated palliatively with interventional procedures. POSITRON EMISSION TOMOGRAPHY (PET SCAN)

Fig. 46. 22: Carcinoma lung-PET-CT scan

-

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II

I

Fig. 46. 23: Carcinoma nasopharynx-PET-CT scan

Fig. 46.24: Another case of carcinoma nasopharynx

VIRTUAL COLONOSCOPY • It is a recently developed technique that uses a CT scanner and computer virtual reality software to look inside the body without having to insert a long tube (conventional colonoscopy) into the colon or without having to fill the colon with liquid barium (barium enema). • More formally known as three-dimensional CT colonography, the virtual procedure allows radiologists to obtain 3D images

Fig. 46.25: Carcinoma rectum-PET-CT scan

from different angles, providing a sort of movie of the colon's interior without having to inse1t an endoscope into the bowel. Advantages • Noninvasive procedure, well tolerated by patient • Requires no sedation, Jess time-consuming • Useful in elderly who are frail and infirm • Useful when a tumour is large enough to block passage of scope (Figs 46.26 and 46.27).

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Figs 46.26 and 46.27: Virtual colonoscopy shows opened up view to look for small mass or polyp

Disadvantages • Exposure to radiation, less detail of inner lining of colon • Small polyps are located more reliably by colonoscopy • Strictly a diagnostic procedure (unlike colonoscopy).

WHAT IS NEW IN THIS CHAPTER?/RECENT ADVANCES

• All topics have been updated.

• PET scan, virtual colonoscopy have been upgraded • A few photographs have been added

MULTIPLE CHOICE QUESTIONS 1. Niche and a notch is diagnostic of: A. Chronic duodenal ulcer B. Chronic gastric ulcer C. Carcinoma stomach D. Stromal tumour 2. Trifoliate/Clover deformity is diagnostic of: A. Chronic duodenal ulcer B. Chronic gastric ulcer C. Carcinoma stomach D. Stromal tumour 3. The substance used in barium studies is: A. Barium chloride B. Barium sulphate C. Barium carbonate D. Barium sulphide 4. Barium follow through extends up to: A. Proximal duodenum B. Fundus of the stomach C. Ileocaecal junction D. Proximal jejunum 5. Which of the following is a contraindication for barium study? A. Dysphagia and odynophagia B. Motility disorders of the GIT C. Perforation of gastric mucosa D. Assessing mediastinal masses

6. Achalasia cardia shows the following findings on barium swallow except: A. Shouldering effect cranial and caudal to the lesion B. Rat tail appearance of lower end of the oesophagus C. Gross dilatation of the proximal oesophagus D. Thin streaks of contrast entering the stomach 7. 'Moulage' sign (barium in a featureless tube) is a feature of: B. Intestinal TB A. Crohn's disease C. Ulcerative colitis D. Malabsorption 8. Which of the following statements is false? A. Angiographic studies use 123 1 as contrast substance B. Enteroclysis is done for mechanical obstruction of the intestine C. Peripheral venography done for deep vein thrombosis uses 125 1 D. Papilloedema is an absolute contraindication for myelography 9. Which of the following statements is false? A. Ultrasound is based on the principle of piezoelectric effect, the most common substance used in ultrasonic transducers being lead zirconate titanate (PZT) B. MRI uses ionising radiation, hence it is unsafe C. Patients with pacemakers and critically ill patients cannot be scanned using MRI D. CT scan gives good bone and calcium detail

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10. The most common side effect of peripheral veno­ graphy is: A. Complications due to contrast B. Tissue necrosis C. Thrombophlebitis D. Pulmonary embolism due to dislodged clot 11. Which of the following statements is false about PET scan? A. PET scan uses protons for radiological examination B. It combines CT and nuclear scanning C. It detects changes at the cellular level D. It helps in early detection of changes m vanous pathologies. 12. BI-RADS score of 5 indicates which of the following? A. Normal mammogram, no evidence of cancer B. Mammogram nonnal, some evidence of cancer present C. Suspicious findings on mammogram, 20-35% chance of cancer D. Mammogram findings highly suspicious, 95% chance of cancer

13. The following statements about virtual colonoscopy ar true except: A. It is also called CT pneumocolon, purely diagnostic B. It is invasive, requires sedation and contraindicated i1 elderly C. It involves exposure to radiation D. It cannot identify polyps measuring betweer 2 mm and 10 mm 14. Which of the following in future may be a gold standard test for screening of colorectal cancer: A. PET scan B. Sigmoidoscopy and colonoscopy C. MRI D. Virtual colonoscopy 15. The following is true about MRI except: A. It is noninvasive B. Gives high intrinsic contrast C. Imaging possible in transverse, sagittal and normal views D. Bone/air artefact can be a problem

ANSWERS B

2 A

3 B

4C

5C

11 A

12 D

13 B

14 D

15 D

6A

7 D

BA

9 B

10 A

47

Principles of Clinical Radiation Oncology • • • • •

• • • • •

Radiation Dose fractionation Sources and methods Measurement Clinical uses

Introduction Radiation oncology is that discipline of human medicine concerned with the generation, conservation, and dissemination of knowledge concerning the causes, prevention, and treatment of cancer and other diseases involving special expe1tise in the therapeutic applications of ionising radiation. Leopold Freund was the first to report in 1897, the use of ionising radiation to "cure" a large nevus pigmentosus on the back of a young girl. With time, ionising radiation became more precise; high-energy photons, electrons, protons, neutrons, and carbon ions became available; and treatment planning and delivery became more accurate and reproducible. Advances in computer and electronic technology fostered the development of more sophisticated treatment-planning and delivery techniques, leading to the development and eventually broad implementation of three-dimensional conformal radiation therapy (3DCRT) and intensity-modulated radiation therapy (IMRT), stereotactic body radiation therapy (SBRT), etc. RADIATION The tenn radiation applies to the emission and propagation of energy through space and material medium. Radiation travels with the speed of light in a vacuum, and interacts with living or nonliving matter resulting in varying degrees of energy transfer to the biological medium. This process of deposition of energy within the cells is brought about by ionisation (removal of an orbital electron) of atoms and

Curative treatment Palliative treatment Radiotherapy reactions Advances in radiation therapy What is new?/Recent advances

molecules. Ionising radiations are off very high frequency (3 x 10 21 hertz) and short wavelength (10 13 m) electro­ magnetic waves. Ionisation can occur within the nuclear DNA molecule of a cell (directly acting) or interaction with other molecules, mainly water (H 20) to produce free radicals (indirectly), which in tum can damage DNA and result in cell death or mutagenesis. By damaging DNA, radiation interferes with cell division and can result in reproductive death of a cell. This process in a malignant tumour could mean the loss of its ability for uncontrolled cell division or proliferation. This process is unselective; it occurs both in cells of normal tissues and in those of tumours. Therapeutic usefulness of radiotherapy, therefore, depends on the differential sensitivity of tissues (normal vs tumour cell), on careful treatment planning and dose prescription to minimise normal tissue damage and the patient's tolerance to radiation. DOSE FRACTIONATION The five Rs of radiobiology provide the basis for fractional radiotherapy (Key Box 47.1). In clinical practice dividing a dose into a number of fractions has the following advantages: 1. The acute effects of single doses of radiation can be decreased with fractionation. The patient's symptomatic tolerance improves with fractional radiation. 2. Fractionation exploits the difference in recovery rate between normal tissues and tumours. Effects on normal tissues are less because of repair of sublethal damage between dose fractions and normal cellular repopulation.

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3. Radiation-induced redistribution of cells within the cell cycle tends to sensitise the rapidly proliferating cells, which is seen more in tumours. 4. Radiosensitivity of cells depend markedly on the phase of the cell cycle at which they receive the radiation. Cells in mitosis and G2 phase are the most sensitive and cells in early GI, and late S phases are the most resistant. 5. Reduction in the number of hypoxic cells is brought about through cell kill and reoxygenation. Also blood vessels compressed by a growing cancer are decompressed as the cancer shrinks, permitting better oxygenation.

RHMIIIIIIIIMIIIII� • • • • •

BASIS OF FRACTIONAL RADIOTHERAPY

Repair Redistribution Reoxygenation Repopulation/recovery Radiosensitivity

Oxygen effect The biologic effects of ionising radiations are greatly influenced by the presence of normal oxygen concentration within the cells. The absence or low oxygen content conveys a resistance to radiation requiring about three times the dose to produce the same biologic effects. Certain solid tumours and large tumours are likely to contain 10-15% of hypoxic cells. For routine practice the "Conventional or Standard" dose fractionation schedule is followed. This consists of 180- 200 cGy fraction per day, and 5 fractions per week over 4-6 weeks (depending on the total dose). The choice of optimal dose/ time/fractionation schedules for various tumours should be individualised according to the cell kinetic characteristics and clinical observations. Radiocurability refers to the eradication of tumour at the primary or regional site and reflects the direct effect of irradiation, which may or may not parallel the patient's ultimate outcome. PROBABILITY OF TUMOUR CONTROL (Fig. 47.1) It is axiomatic in radiation therapy that higher doses of radiation produce better tumour control, and numerous dose-response curves (sigmoid in shape) in a variety of tumours have been published. For every increment of dose a certain fraction of cells will be ki!led. Therefore the total number of surviving cells will be proportional to the initial number present and the fraction killed with each dose. Thus, it is apparent that various levels of irradiation yield a different probability of tumour control, depending on the extent of lesion or number of clonogenic cells present.

100%

e c 8 :i 0 E

TP

--NP

I I I I I I I I

�

II

100% cn C .Q

a. E 0 ()

Q) :, cn en :;::;

ro E

o I / I '----=---....:..J=----"___,_, ________J z

Dose in cGy

TP = Tumour control probability NP = Normal tissue complication probability

Fig. 47.1: The graph showing tumour probability control

For subclinical disease ( I 0 3-4 cells) in squamous cell carcinoma of the upper respiratory tract or for adenocarcinoma of the breast, doses of 4500-5000 cGy result in control of disease in over 90% of patients. For microscopic residual disease or cell aggregates greater than 106/109 are required for the pathologist to detect them. Therefore, these volumes must receive higher doses of radiation in the range of 6000-6500 cGy in 6-7 weeks for epithelial tumours. For clinically palpable tumours (Gross disease), doses of6000 cGy (for Tl) to 7500 cGy to 8000 cGy for T4 tumours) are required (200 cGy/day/5 fractions weekly). This dose range and probability of tumour control have been documented for various tumours. RADIOTHERAPY-SOURCES AND METHODS OF DELIVERY Radiotherapy is the therapeutic use of high-energy ionising radiation in the treatment/management of malignant disease. These are either electromagnetic waves, X-rays, gamma rays or corpuscular (subatomic particles) electrons, protons, neutrons, alpha particles, or heavy ion nuclei. Ionising radiation penetrates tissues to different depths according to its type of energy and physical nature. Radiotherapy treatment planning is an important part of the radiation oncologist's work. SOURCE OF RADIATION (Fig. 47.2) Gamma and beta rays from radioactive isotopes (cobalt 60, caesium 137, indium 197) and X-rays and electrons from a high energy X-ray machine (linear accelerator). Protons, neutrons and heavy ion nuclei from cyclotrons. X-rays and gamma rays are identical in properties but are produced by different sources. Ionising radiations can be classified according to their density of ionisations per unit length of the distance in the absorbing media as low and high LET (linear energy transfer) radiation.

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Advantages of megavoltage beam RT 1. Deeper penetration 2. Sharp beam edges 3. Skin sparing 4. Equal absorption in bone and soft tissues 5. Improved dose distribution within tissue. II. Brachytherapy (Key Box 47.2) Brachytherapy refers to use of radiation sources in or close t< the tumour. Use of sealed (closed containers) radioactive sources fo radiation treatment from a short distance. Types of brachytherapy Fig. 47.2: Cobalt teletherapy unit

IONISING RADIATION • Low LET-X-rays, gamma rays and electron. • High LET-neutrons, protons, a-particles and negative 10ns. • High LET radiation has a mass heavier than electrons. Hence, they cause dense ionisation and are biologically more effective (damaging) than low LET radiation. They are also less dependent on repair of sublethal damage, cell cycle phases and oxygen content of the cells for radio­ sensitivity. • High LET radiation is available in limited cancer centres around the world, require expensive equipment to produce and are undergoing clinical trials. METHODS OF DELIVERY Ionising radiations may be delivered clinically in three ways: I. External beam irradiation From sources at a distance (usually 80-100 cm) from the body surface. This includes 6°Co Teletherapy Units andX-ray sources, such as linear accelerators (Fig. 47.3).

1. Intracavitary (within a cavity), e.g. uterine cavity, vagina cavity, oesophageal and bronchial lumen. 2. Interstitial when radioactive needles and wires are insertec into and around a tumour. 3. Surface moulds or plaques as radioactive surfac{ applicators, e.g. skin cancer, eye cancers. With this mode of therapy a high dose can be deliverec locally to the tumour with rapid dose fall off in th{ surrounding normal tissue. In the past, brachytherapy wai carried out mostly with radium or radon sources. Currently use of artificially produced radionuclides such as 137C� (caesium137), 192Ir (iridium 192), 198Au (gold 198), anc 125 I (iodine 125) is rapidly increasing. N ew technical devel opments have stimul ated increased interest in brachytherapy 1. Introduction of improved artificial isotopes 2. Manual afterloading devices to reduce personnel exposure 3. Remote afterloading, high dose rate (HDR) machines, have increased accuracy, improved dosimetry, reduced (short) treatment time, treatment on outpatient basis and have improved patient compliance. Brachytherapy is used very often. Combined with external beam treatment and rarely alone (early stage). The rationale behind combining the two is to treat the primary site and regional spread (very often subclinical disease) with external RT and to deliver a higher dose (boost) to the primary (gross disease) with brachytherapy. Aim is not to exceed the normal tissue tolerance

.,•..........,.� 1. 2. 3. 4. 5. 6.

Fig. 47.3: Linear accelerator

ADVANTAGES OF BRACHYTHERAPY

High localised dose to limited volume Minimal dose to adjacent tissues Spares deeper normal tissues Continuous RT in a single course Short overall time (high dose rate) Alone: Rarely (early stage) Combined: Often (as boost)

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and at the same time the tumour should receive adequate curative dose. Brachytherapy can also be used as palliative therapy, e.g. bronchial and oesophageal obstruction.

Advantages I . Characteristic sharp dose fall off beyond the tumour 2. Dose uniformity within the target volume

Ill. Internal or systemic irradiation From unsealed radioactive sources (i.e. 131 1, 32 P, 89 Sr) administered enterally, intracavitarily or intravenously, for diagnostic (nuclear medicine) and therapeutic purposes, e.g. carcinoma thyroid, bone tumours and thyrotoxicosis.

Principal applications l . Treatment of skin and lip cancers 2. Chest wall irradiation for breast cancer 3. Boost dose to nodes and tumour bed 4. Head and neck cancers.

MEASUREMENT OF IONISING RADIATION 1. The Roentgen is a unit of exposure. It is a measure of ionisations produced per unit volume of air by X-rays and gamma rays and cannot be used for photon energies above 3 Mev. • The SI unit for exposure is Coulomb per kilogram (C/kg) • 1 R = 2.58 x I 04 C/kg air 2. Radiation absorbed dose (RAD) • Absorbed dose is a measure of the biologically significant effects produced by ionising radiation. Absorbed dose = De/dm, De is the mean energy imparted by the ionising radiation to material of mass dm. The old unit of dose is rad and represents the absorption of 100 ergs of energy per gram of absorbing material. • 1 rad== I 00 ergs/g = I 02 J/kg • The SI unit of absorbed dose is gray (Gy) and is defined as • I Gy = l J/kg Thus the relationship between rad and gray is 1 Gy = I 00 rad or I cGy = 1 rad.

Clinical use of radiotherapy (Key Box 47.3) Like surgery and chemotherapy, radiation therapy (RT) has definite indications and contraindications for its application. It can be used alone to cure or, in combination with other methods, as an adjuvant. Currently 50-60% of all patients with cancer receive RT during the course of their illness. If properly used, 50% of these patients could get cured. For the other half, incurable by any current method, palliation of specific symptoms and signs can improve quality of life (Table 47.1). Before treating a patient with radiotherapy, the radiotherapist must be satisfied that the working diagnosis is correct, pretreatment investigations and staging have to be complete. Then the radiation oncologist must address two questions: 1. Is the treatment intent curative or palliative? 2. What is the best approach to achieve this goal? The first question is vital, for there are important differences between radical and palliative radiotherapy. The second question recognises that cancer can be treated by surgery, radiotherapy and drugs. In many instances, radiotherapy is the best approach. In view of the increasing complexity of curative cancer management for many tumours, with different combinations of surgery, radiotherapy and chemotherapy for different stages of the disease, require a co­ ordinated Multidisciplinary approach. The correct initial choice gives the best prospect for cure or good palliation.

ELECTRON BEAM THERAPY Source Mainly linear accelerators Energy Most useful range for clinical use is 6 to 20 Mev.

AS A PRIMARY CURATIVE MODALITY (Key Box 47.4) A. RT frequently may be the sole agent used with curative intent for anatomically limited tumours of the retina, optic nerve, brain (craniopharyngioma, medulloblastoma,

Use 1. Superficial tumours up to a depth of 5 cm 2. Local boost

Differences between radical and palliative radiotherapy Radical radiotherapy

Palliative radiotherapy

• Treatment is intended to eradicate all clonogenic malignant

• Treatment is intended to control symptoms to improve quality of life.

cells. •

High dose curative courses of RT needed and considerable nonnal tissue morbidity (acute) may be associated.

• Late side-effects of adjacent normal tissues may be dose limiting. • Patient often needs high doses and long courses of treatment.

•

Minimum doses of RT to achieve maximum control with minimal side-effects.

• Short patient survival times, less concern with long-term limiting, morbidity. • Patient prefers few hospital visits, so short courses of treatment used.

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..............'V IIHMl..........� 1. 2. 3. 4.

As As As As

RADIOTHERAPY USE IN FOUR SETTINGS a primary curative modality an adjuvant for curative therapy (combined modality) prophylactic radiation palliative treatment

Rl4Ml..........'V INDICATIONS FOR CURATIVE RT Stages I and II Testis Seminoma Ovary Dysgerminoma Skin Basal cell and squamous cell carcinoma Lymphatic Hodgkin's lymphoma Cervix Cervix, uterus, vagina Bladder Transitional cell Ca Prostate Adenocarcinoma Anal canal Carcinoma Head and neck Cancers Oesophagus Cancer Lung Non-small cell cancer Brain Medulloblastoma

ependymoma), spinal cord (low-grade glioma), skin, oral cavity, pharynx, larynx, oesophagus, uterine cervix, vagina, prostate and reticuloendothelial system (Hodgkin's disease, stages I, II and III A). B. When no other potentially curative treatment exists. Some cancers remain localised for all or much of their natural history. These cancers might also be unresectable by virtue of their anatomical location or because of local infiltration into surrounding nom1al/vital structures, which would mean that surgery will severely affect physiological function, e.g. locally advanced head and neck cancer, cervical cancers stage Tlb-III, medulloblastoma (alternative to surgery for inaccessible and inoperable malignancies). C. Where alternative therapy is considered more "toxic". Carcinoma of the larynx, anal canal, breast can all be managed by ablative surgery and in each case the anatomy and physiology of the respective organ is lost. Each of these cancers can be managed by irradiation with preservation of anatomy and function. Preservation of organ and its function (larynx, breast, anal canal, limbs, cervix, tongue, bladder) ADJUVANT FOR CURATIVE THERAPY (COMBINED MODALITY) (Key Box 47.5) RT is combined with surgery for advanced cancers of the head and neck, cancers of the lung, uterus, breast, urinary bladder, testis (seminoma), rectum, soft tissue sarcomas and primary bone tumours.

• • • • • • • •

ADJUVANT RT (SURG+RT+/-CT) Head and neck cancer locally advanced Brain tumours Breast cancer Rectal cancer Soft tissue sarcoma Bone sarcoma Endometrial Ca Paediatric solid tumours (Wilms', rhabdomyosarcoma, neuroblastoma)

RT is an adjuvant to chemotherapy for some patients will lymphomas, lung cancers and cancer in children (rhabdomyo­ sarcoma, Wilms' tumour, neuroblastoma). In some clinical situations the combined benefits of surgery RT and chemotherapy might be exploited. It has been mos useful in the management of breast cancer, bone sarcoma anc Wilms' tumour. COMBINED TREATMENT (SURGERY AND RADIATION THERAPY) In many situations radiation therapy alone is inadequate fo, achieving maximum cure levels. This can be because the number of tumour stem cells is too large, some or all of the cancer cells are radioresistant, or tolerance of the contiguom normal tissues is too low. The rationale for combining surgery and radiation therapy is the differing mechanisms of the two disciplines. Radiation therapy fails at the centre of the tumour where the concentrations of the tumour cells is the largest and the conditions may be hypoxic (less sensitive to RT). Surgical resection fails because the tumour extends further than the margins of excision, infesting contiguous tissues with undetectable microscopic foci. Radiation therapy is efficient in the sterilisation of these tumour cell numbers that are well vascularised, and the surgical resection is efficient in removing the gross necrotic tumour masses. Radiation can be combined with surgery either preoperatively or postoperatively. Aims and advantages of preoperative RT l . Unresectable cancer to resectable cancer 2. Prevent iatrogenic metastases 3. Reduction of size and vascularity 4. Destroys microscopic foci beyond surgical margin Disadvantages l . Delay in surgical (primary) treatment 2. Delay in wound healing 3. Pathologic downstaging to influence other adjuvant treatment.

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3. Concurrent: During a course of radiotherapy. 4. Combinations of the above.

4. Alters anatomical staging (precise pathological extent) 5. Inability to tailor RT to high risk areas.

•••........,� Postoperative RT (Key Box 47.6)

AIM AND ADVANTAGES 1. Exact disease extent known so as to tailor the treatment individually. 2. Operative margins are well-defined-gross or microscopic. 3. Less postoperative complications-wound healing intact. 4. GI anastomoses and ileal conduits can be done in a non­ irradiated field. 5. Potential for unnecessary irradiation in some patients is reduced DISADVANTAGES

1. Delay in RT due to postoperative complications-delay wound healing. 2. Decreased radiosensitivity of the tumour due to rich oxygen in vascular tumour. 3. Postoperative adhesions of organs/structures increase RT complication rate. 4. No effect on dissemination during surgery. 5. Volume of normal tissue to be irradiated is more. Usually all tissue planes are potentially contaminated by surgery.

Clinical situations may indicate different sequences but such combinations of surgery and radiation therapy improve the local tumour control rate for many advanced cancers. Combined therapy may also improve the cure rate at the same time reducing the morbidity associated with more aggressive single modality treatment. COMBINATION OF RADIOTHERAPY WITH CHEMOTHERAPY

In general, chemotherapy is used in an adjuvant way to control subclinical disease elsewhere in the body or in an additive way to enhance the local effects of the radiation to achieve higher rate of local control. Many other agents will act in both ways. Agents of choice are those whose toxic effects are in organs not included in the radiation target volume. An example is the combination of the cisplatin compounds with radiation therapy for head and neck cancers. Here, the toxicity of chemotherapy is primarily haematogenous and renal. The toxicity of RT is on the oral mucosa. Chemotherapy could be combined with RT in three main ways 1. Neoadjuvant: 1-3 courses before definitive RT. 2. Adjuvant: After completion of definitive RT.

PROPHYLACTIC CRANIAL RADIATION

Certain cancers have a high incidence of developing brair (CNS) metastases even after their primary disease is controlled since the CNS because of the blood-brain barrier can act as , sanctuary site for relapse. Among such patients, it is possiblt to reduce their local CNS relapse rate and improve surviva by treating the CNS by prophylactic cranial RT± intrathecal chemotherapy. The total dose needed is low (18-24 Gy) anci has minimal side effects, e.g. acute lymphoblastic leukaemia; high grade lymphomas.

-·-.......... PALLIATIVE TREATMENT

�

Objectives of palliative irradiation include:

• Relief of pain, usually from metastases to bone • Relief of headache and neurological dysfunction from intracranial metastases • Relief of obstruction, such as tumours involving ureter, oesophagus, bronchus, lymphatic and blood vessels. •

Promotion of healing of surface wounds by local tumour control.

• Haemostatic for cervical/bladder cancers.

MANAGEMENT: RADIOTHERAPY REACTIONS

{Table 47.2)

The incidence of systemic symptoms from radiotherapy is variable. In broad terms, the larger the treatment yield, the fraction size and the total given dose, the greater will be the chance of the patient developing problems. The dose of radiation that can be delivered is limited by acute reactions and by late irreversible organ/tissue damage. Each organ has a known tolerance which should not be exceeded. However, in order to achieve a given level of tumour control probability certain amount of normal tissue sequelae are unavoidable. During a course of radiotherapy mild to moderate grade acute reactions occur frequently and can be usually conser­ vatively managed and might require a short break in the treatment whereas chronic reactions are usually the dose limiting complications. Severe grade reactions should be avoided using proper time dose fractionation regimens, accurate treatment planning and execution. Some of the important acute reactions following RT, their threshold doses and management are shown in Table 4 7.2.

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Complications and management of RT Organ toxicity

Approx. dose threshold Gy

Specific management points

Skin

Erythema

10-12

No specific treatment required

Skin Shin

Dry desquamation Moist desquamation

40-50 45-55

Proflavine and emollients may produce symptomatic relief Keep the affected area dry. Gentian violet may be helpful in drying the affected area

Mucous Mucositis

30-40

Topical benzydamine hydrochloride mouth rinse or spray. Stop smoking. Mucaine for oesophageal mucositis. Always exclude candidiasis

Hair

Alopecia

30-40

Warm patient prior to starting treatment. Advise a wig fitting

Lung

Pneumonitis with cough, dyspnoea

20

Consider systemic corticosteroids

GI tract

Nausea, vomiting

Any dose

Regular antiemetics may be required

GI tract

Diarrhoea

30-40

Advise low fibre diet when starting treatment. Antidiarrhoeal preparations may be required for symptomatic relief

40-50 10-20

Exclude urinary tract infection and consider antimuscarinic drugs Check full blood count regularly

Bladder Urinary frequency and dysuria Suppression especially of Bone marrow white blood cells and platelets during wide-field radiotherapy

ADVANCES IN RADIATION THERAPY Current research in radiation oncology is of such significance that it promises a new standard of care for patients with cancer. Recent advances in radiation therapy include efforts to improve the effectiveness of radiation and to improve the quality of life of treated patients. Innovations in radiobiology, imaging technology, computer technology and treatment machine technology has resulted in marked changes in the way radiotherapy is practised at present. The newer methods aim at increasing the accuracy of treatment, planning and dose delivery using the highly sophisticated features offered by the modem day equipment (Key Box 47.8). 1. 3-D conformal radiotherapy (three-dimensional treatment planning and conformal dose delivery). In3-D CRT, patient immobilisation, image-guided treatment planning and computer-controlled treatment delivery can create a radiation dose distribution that conforms to the shape of the tumour volume (Key Box 47.9). The tumour volume containing the cancer and areas of potential cancer is much more accurately outlined as are normal tissues to be avoided. The target radiation dose can be increased when necessary without increased toxicity to normal tissue. This is accomplished using volumetric CT data in a3-D treatment planning computer.

2. IMRT: IMRT is an advanced form of3-DCRT. It is one of the technologically most advanced treatment methods available in external beam radiation therapy. IMRT allows

............ 1. 2. 3. 4. 5. 6. 7. 8.

ADVANCES IN RADIATION THERAPY

�

30 conformal radiotherapy (3-dimensional) IMRT (intensity modulated radiation therapy) SRS/SRT (stereotactic radiosurgery and radiotherapy) IGRT (image guided radiation therapy) SBRT (stereotactic body radiation therapy) Proton therapy lntravascular brachytherapy CyberKnife

very precise external beam radiotherapy treatments. Rather than having a single large radiation beam pass through the body, with IMRT the radiation is effectively broken up into thousands of tiny pencil-thin radiation beams of varying

w,m,.........,.� CANCERS BEING TREATED WITH IMRT

• Prostate cancer, pancreatic tumours, lung cancer • Metastatic brain tumours, primary brain tumours (glioblas­ tomas, gliomas, etc) • Liver tumours (metastases, hepatocellular carcinoma) • Head and neck cancer (larynx, tongue, sinus, base of skull, mouth, etc) • Radiosurgery (single fraction) and stereotactic radiation therapy (fractionated)

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intensity with millimetre accuracy. These beams enter the body from many angles and intersect on the cancer. This results in a high dosage to the tumour and a lower dose to the surrounding healthy tissues. • Intensity modulation radiotherapy can allow us to treat tumours to a higher dose, retreat cancers that have previously been irradiated, and safely treat tumours that are located very close to delicate organs like the eye, spinal cord, or rectum. Simply put, this can translate into a higher cancer control rate and a lower rate of side effects. 3. SRS/SRT (stereotactic radiosurgery and radiotherapy) High-dose highly focused radiation therapy for small (SRS = Single fraction, SRT = Multiple fractions) target lesions(< 2-4 cm) can be accomplished by either gamma knife (multiple, fixed, precisely aimed cobalt teletherapy beams) or stereotactic radiation therapy(multiple rotational arcs of photon beams from a linear accelerator). Both techniques are similar in their use of standard energy photon beams for treatment and rely on meticulous patient immobilisation to deliver treatment to a precisely localised target within a co-ordinate mapping system. These techniques have been widely used and well described for the treatment of intracranial neoplasms (meningiomas, acoustic neuromas and metastatic tumours) and for the ablation of arteriovenous malformations and ocular melanomas. [Equipment used: 1. Gamma Knife(Multiple 6 °Co sources) or 2. X- knife(modified linear accelerator)] 4. IGRT • Image guided radiation therapy(IGRT), is one of the most cutting-edge innovations in cancer technology available. Tumors can move, because of breathing and other movement in the body. Real-time imaging of the treatment target and normal organs during each treatment allows for minimisation of reduction of irradiated volumes, as well decreases the chance of missing a target, helping to limit radiation exposure to healthy tissue and reduce common radiation side effects. • In IGRT, the linear accelerators are equipped with imaging technology that take pictures of the tumour immediately before or even during the time radiation is delivered. There are numerous types of imaging modalities that can be incorporated into an IGRT system. Examples include: ultrasound images, low-energy(kV) CT scan images, high­ energy(MV) CT scan images, etc. 5. SBRT Stereotactic body radiation therapy(SBRT) is a technique that utilises precisely targeted radiation to a tumour while minimising radiation to adjacent normal tissue(Fig. 47.4). This targeting allows treatment of small or moderate sized

Fig. 47.4: Stereotactic radiosurgery brain (metastasis)

tumours in either a single or limited number of dose fractions. SBRT has been used in hepatocellular carcinoma, lung cancer, prostate cancer, pancreatic cancer. As with any form of radiation therapy, careful attention to matters of patient selection and technical quality assurance is essential for the effective and safe implementation of SBRT. 6. Proton therapy • Proton beam: Proton radiation reduces the dose to normal tissues by allowing for more precise dose delivery because of the unique physical properties of heavy particles. Protons penetrate tissue to a variable depth, depending upon their energy, and then deposit that energy in the tissue in a sharp peak, known as a Bragg peak. This rapid dose fall off at a depth that can be controlled by the initial energy of the protons allows for decreased radiation to adjoining normal tissue by a factor of 2 to 3. • Protons are used for uveal melanoma(ocular tumours), skull base and paraspinal tumors (chondrosarcoma and chordoma), and unresectable sarcomas, pediatric neoplasms (such as medulloblastoma) and prostate cancer. 7. lntravascular brachytherapy: Arterial renarrowing after angioplasty or restenosis occurs in 30 to 40 percent of patients and results from neointimal proliferation and constrictive remodelling of the angio-injured artery. Coronary stenting has led to a 30 to 50 percent decrease in the rate of restenosis primarily by preventing the consh·ictive remodeling of the artery but at the cost of an increase in neointimal proliferation. The system used for intra-a1ierial beta-radiation therapy has Yttrium-90 beta ray emitting source(half-life, 64 hours; maximal energy, 2.284 Mev), a centering balloon and an automated delivery device. An 18-Gy dose not only prevents the renarrowing of the lumen typically observed after successful balloon angioplasty but actually induces luminal enlargement.

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Drawback (all techniques) • High cost of treatment (expensive equipment+ time and labour-intensive) • Lack of long term data (survival/late morbidity). 8. CyberKnife: CyberKnife radiosurgery is the noninvasive alternative to surgery for the precise treatment and effective removal of cancerous tumours (Fig. 47.5) from the body. • The system has a miniaturised linear accelerator mounted on a robotic arm with 6 different points of axis where it can bend, tum, tilt or swivel with sub millimetre accuracy. • Destroys tumours with highly precise beams of radiation, tumours virtually anywhere in the body, quickly, painlessly and without downtime or hospital stay in one to five sessions (hypofractionation). • Thus offers new hope to patients who have inoperable or surgically complex tumours and for those who may be looking for an alternative to more invasive surgery.

Fig. 47.5: CyberKnife WHAT IS NEW IN THIS CHAPTER?/ RECENT ADVANCES

• All topics have been updated. • New key boxes have been added. • Recent advances in radiotherapy have been upgraded.

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MULTIPLE CHOICE QUESTIONS 1. The following statements about radiation are correct except: A. Fractional radiation reduces the acute effects of single doses of radiation on normal tissues B. Cells in mitosis and G2 phase are most sensitive to radiation C. Oxygen concentration within cells is directly proportional to the radiation dose required to kill them D. Standard dose of radiation is 180-200 cGy per day and 5 per week over 4-6 cycles 2. A dose-response curve for radiation is of which shape? A. Sigmoidal B. Linear-rising C. Bell-shaped D. Irregular 3. The following are the pr inciples of brachytherapy except: A. It is a type of method of delivery of ionising radiation from sources inside or close to the tumour B. External RT is used to deliver a higher dose (boost) to treat the primary site while brachytherapy treats the regional spread C. It is highly localised, specific to a given tumour volume D. It has a high dose rate, given as continuous RT in a single course 4. 1 cGy is: A. 1 rad B. lOrad C. 100 rad D. 1000 rad 5. The most commonly used chemotherapeutic agent with radiation for head and neck cancers is: A. Vincristine B. Doxorubicin C. Cisplatin D. Etoposide

6. Wh ich of the f o llowing statements regarding chemotherapy adjuvant to radiotherapy is false? A. Agent of choice will have minimum toxic effects on the organs included in the radiation target volume B. Neoadjuvant chemotherapy refers to 1-3 courses of CT after definitive RT C. Adjuvant CT refers to a course after completion on definitive RT D. Intrathecal CT is a prophylactic measure to prevent CNS metastasis in cancers like ALL or high grade lymphomas 7. IMRT refers to: A. Intensity modified radiation therapy B. Intensity modulated radiation therapy C. Intensive method of radiation therapy D. lntravascular method of radiation therapy 8. Intra-arterial brachytherapy uses which of the following? A. Technetium-99 B. Yttrium-90 C. lodine-123 D. Cobalt-60 9. The following is true about electron beam therapy except: A. The source is mainly a linear accelerator B. Most useful range is 6-20 Mev C. Deeply situated tumours at a depth of> 5 cm are treated with this D. Provides dose uniformity within the target volume 10. The following is true about postoperative RT: A. Exact disease extent is known and treatment can be individualised B. Postoperative complications, especially wound healing is affected C. Operative margins are ill-defined D. Potential for unnecessary radiation increases

ANSWERS 1 C

2 A

3 B

4A

5 C

6 B

7 B

8 B

9 C

10 A

48 Principles of Anaesthesiology • • • • • •

Preoperative assessment General anaesthetic agents Muscle relaxants Endotracheal intubation Monitoring Local anaesthetics

• Regional anaesthesia • Pain relief • Complications of anaesthesia • Cardiopulmonary resuscitation • What is new?/Recent advances

Surgery has been practised for ages. However, the advent of modern techniques of anaesthesia has allowed surgery to develop by leaps and bounds. If there is a well-informed, vigilant and safe anaesthesiologist taking care of the patient, the surgeon is able to concentrate on the surgical procedure unhindered. T he anaesthetist of yore who was treated often as a technician assistant of the surgeon has metamorphosed into the present day anaesthesiologist, who is also a perioperative physician. An anaesthesiologist's understanding of anatomy, physiology and pharmacology, and his expertise of management of critical illness and pain have taken him into new fields such as Intensive Care, Pain and Palliative Care. Even then, the main realm of the anaesthesiologist remains the operation theatre. A good understanding of the physiology and pharmacology, complemented by continuous and vigilant monitoring has made the practice of anaesthesia safer. Safe practice of anaesthesia comprises the following steps: a good rapport with the patient, a thorough preoperative preparation and premedication, monitoring and perioperative care.

Anaesthesia is associated with changes in the internal homeostasis. Normally these are well tolerated by the different systems. However, if the patient has a pre-existing derangement, his capacity to withstand changes in his internal milieu may be limited. It is thus very important to know the preoperative condition of the patient. History A detailed history of the patient with symptoms pertaining to the various systems must be elicited (Table 48.1). Physical examination A detailed physical examination is done and the relevant history specially borne in mind. General physical examination includes: Vital signs: Blood pressure, heart rate, respiratory rate, temperature (and oxygen saturation, in relevant cases). PICCLE: Pallor, icterus, cyanosis, clubbing, lymph­ adenopathy, oedema and raised jugular venous pulse. Airway: Examine carefully to detect any difficult airway.

PREOPERATIVE ASSESSMENT AND PREMEDICATION

Spine: To rule out infection over the skin covering the spine, tenderness, stiffness or fractures of spine, to check spaces.

Every patient is anxious when he comes into the operating room. The causes of anxiety can be varied: fear of the disease condition, surgery, anaesthetic and the anticipated pain. A good rapport developed between the patient and the anaesthesiologist can build up his confidence and help allay many of these fears.

Veins: Ease of obtaining venous access is also assessed. Systemic examination A detailed examination of the various systems is then carried out and relevant findings noted.

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History taking prior to anaesthesia Body system

History-Points to note

I. Cardiovascular system

Dyspnoea, angina, syncope, palpitations, pedal oedema, previously diagnosed to have a cardiac problem, effort tolerance

2. Respiratory system

Cough, fever, breathlessness, chest pain, recent onset upper respiratory tract infection

3. Central nervous system Consciousness level, convulsions, orientation, ability to walk, speech, movement of all four limbs, bowel and bladder habits, any paraesthesia or altered sensation in the limbs. 4. Renal system Decreased urine output, haematuria 5. Hepatic Jaundice Easy bruising, increased blood loss with trivial injuries 6. Haematology 7. Musculoskeletal H/o weakness in the limbs 8. Previous medical Previous surgeries/ anaesthetics, any problems during the previous experience. H/o hospitalisation in the past, history H/o tuberculosis, asthma, diabetes, hypertension, epilepsy, H/o current medications, steroid intake in the last 6 months 9. Allergy Allergy to any drug or other substances 10. Addictions Smoking, alcohol, drug abuse 11. Pregnancy Last menstrual period/possibility of pregnancy (in female patients) 12. Family

Relevant family history, particularly pertaining to anaesthesia

Assessment of airway Whenever a person becomes unconscious, the tongue and epiglottis fall back on to the pharynx and obstruct the airway. Since the anaesthesiologist makes the patient unconscious during a general anaesthetic, it is his duty to ensure that the patient's airway patency is maintained. Hence, assessment of the airway becomes important before a patient is made unconSCIOUS.

Class I

Class II

Class Ill

Class IV

Fig. 48.1: Mallampati classification of the airway

The assessment is done as follows: The 1-2-3 test • When a person opens his mouth, one should be able to insinuate one finger in the temporomandibular joint. • There should be at least two finger breadths' distance between his incisors. • There should be at least three finger breadths' distance between the chin and the thyroid cartilage (thyromental distance) of the patient.

Grade I

Grade II

Grade Ill

Grade IV

Mallampati test The patient is made to sit upright, open his mouth wide and protrude his tongue. The structures visualised are classified as given in Fig. 48.1. These classes roughly c01Telate with the following grades of laryngoscopic views (Fig. 48.2). Difficult airway may be anticipated in Mallampati Class III and IV and the anaesthesiologist must be prepared to secure airway in such patients.

F"19. 48. 2 : Mo d'1f'1e d c ormac k and Le hane grad'ing of 1 aryngo-

scopic view

Principles of Anaesthesiology

Neck movements Restriction of neck movements, especially extension makes endotracheal intubation more difficult. Investigations There is no place for routine investigations to be ordered before any surgery. Investigations that are required to assess the baseline status of the patient and have a bearing on the perioperative course must be done. Thus, they should be tailored to the individual patient. The common investigations ordered are as follows: Haemoglobin estimation: All patients Electrocardiogram, blood sugar estimation, blood urea, serum creatinine and electrolytes: Male patients> 40 years, smokers and female patients above 50 years. Total and differential white cell count: If infection is suspected. Platelet count and coagulation profile: If any abnormality is suspected or bleeding is anticipated. Chest X-ray is requested for if a major abdominal or thoracic surgery is planned, postoperative ventilation is expected or cardiorespiratory disease is suspected. Stress test, echocardiogram, pulmonary function test or a blood gas analysis are obtained as necessary. Blood grouping and cross-matching are requested if the surgery may be associated with major blood loss.

ASA Physical Status Classification The patient's preoperative physical status can be classified into five different categories. The American Society of Anaesthesiologists classification of Physical Status is as follows: ASA I : Healthy patient, no medical problems ASA II : Mild systemic disease ASA III : Severe systemic disease, but not incapacitating ASA IV : Severe systemic disease that is constant threat to life ASA V : Moribund, not expected to survive without the operation A suffix E is added if the surgery is of emergent nature. A sixth category called ASA VI is given to the patient who is declared brain dead whose organs are being removed for donor purposes. The risks associated with anaesthesia in a patient with concurrent diseases must be assessed. Algorithms by different organisations are available to help decision-making in individual patients: For example, American Heart Association recommendations for evaluation of ischaemic heart disease in a patient posted for noncardiac surgery. The goal of preoperative assessment and preparation is thus to ensure that the patient is optimised and is in the best possible condition prior to surgery and anaesthesia.

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Informed consent The patient is explained about the planned anaesthetic, th� problems anticipated and the risks involved in his owr language and a written and informed consent is obtained. Preoperative instructions The patient is permitted to take solids and milk up to 6-8 houn and clear fluids up to 3 hours prior to surgery. This may be relaxed for neonates and infants where a four-hour fast for breast milk is sufficient. Fonnula feeds and milk from any other source are treated as solids. If the last food intake contained a considerable amount of fat, gastric emptying time may be delayed (Key Box 48.1).

lft4M.........,.� FASTING INSTRUCTIONS • Adults Solids and milk: 6 hours and older children Clear fluids: 3 hours • Infants and neonates Formula feeds: 6 hours Breast milk: 4 hours Clear fluids: 2 hours

Premedication Premedication is the term given to the administration of certain medications prior to anaesthesia. The drugs used for premedication and their objectives are as follows: I. To allay anxiety: Certain degree of anxiety is felt by most patients before surgery. A good rapport developed between the patient and the anaesthesiologist helps relieve this anxiety. Any of the following medications may be used to reduce anxiety. Adults: (Night before and morning of surgery) Tab Diazepam 0.1-0.2 mg/kg Tab Lorazepam 2-4 mg orally Children: To enable easy separation from parents. Midazolam 0.5 mg/kg (maximum 10 mg) mixed with 5 ml of paracetamol syrup is given orally 15-20 minutes prior to the procedure. Triclofos syrup, I 00 mg/kg, one hour prior to surgery 2. To relieve pain: If the patient has any painful condition that could get aggravated on movement, a narcotic is often added to reduce the pain during shifting from the ward, e.g. fractures. Morphine (0.1 mg/kg), pethidine (0.5 mg/kg) or tramadol (0.5-1 mg/kg) given IM are often used. 3. To dry secretions: An anticholinergic such as glycopyrrolate (0.2 mg) is added if a fibreoptic intubation is planned so that oral secretions do not hinder vision. Local anaesthetic agents produce better local anaesthesia of the upper airway when the mucosa is dry. It may be given intravenously just prior to surgery in ENT surgeries and oral surgeries.

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4. To help anaesthesia induction: Premedication with a narcotic provides analgesia and helps induce anaesthesia more smoothly. 5. To blunt baroreceptor reflexes: A small dose of� blockers or clonidine may be given in certain patients to blunt baroreceptor reflexes during intubation. 6. To reduce gastric volume and acidity: Some patients are at risk of regurgitation of gastric contents and aspiration. They may be premedicated with a prokinetic such as metoclopramide ( 10 mg) and a H2 blocker such as ranitidine (150 mg orally). Pantoprazole 40 mg may be given instead of ranitidine. The consequences of aspiration of gastric contents depends on its quantity and acidity. Particulate matter, if aspirated can cause mechanical blockage of the airways. Metoclopramide reduces residual gastric content by hastening gastric emptying and ranitidine reduces the acidity. Sometimes, a nonparticulate antacid such as sodium citrate may be given to neutralise existing gastric acid. Multiple factors during anaesthesia and surgery increase the chances of nausea and vomiting. Since anaesthesia involves blunting of airway reflexes, patients are at risk of aspiration. This is why patients are kept fasting before elective surgery. This may not be possible for patients who are posted for emergency surgery. It is assumed that these patients are 'full-stomach' and appropriate precautions are taken. TYPES OF ANAESTHESIA Anaesthesia can be classified into two main categories: General anaesthesia and regional anaesthesia. General anaesthesia (GA) The patient is unconscious and there is a generalised and reversible depression of the central nervous system. Regional anaesthesia This involves injection of local anaesthetic agents in close proximity to the nerves or nerve bundles supplying the site of surgery. Regional anaesthesia can be central neuraxial block or peripheral nerve blocks. Central neuraxial block When regional anaesthesia is induced by injecting the local anaesthetic agents around the spinal cord, it is called central neuraxial block. Peripheral nerve block When regional anaesthesia is induced by injecting the local anaesthetic agents around nerve plexuses or individual nerves, it is called peripheral nerve block.

The choice of anaesthesia depends on several factors: • The site and duration of surgery • General condition of the patient • Expertise of the anaesthesiologist and • Preference of the patient. Regional anaesthesia has a small failure rate and certair surgeries may outlast the duration of block provided by the regional block, in which case general anaesthesia may need tc be given. Thus even though the primary anaesthetic contemplated is regional anaesthesia, patients must also be fi1 for general anaesthesia. GENERAL ANAESTHETIC AGENTS The term general anaesthesia includes: • • • •

Hypnosis (sedation) Amnesia Analgesia and Muscle relaxation. General anaesthetic agents are of two main types: Inhalational anaesthetic agents or intravenous anaesthetic agents.

INHALATIONAL ANAESTHETIC AGENTS • Volatile anaesthetics: The volatile anaesthetic agents need a vaporiser to calibrate and deliver the vapour accurately in measured doses, e.g. halothane • Nonvolatile anaesthetics: e.g. nitrous oxide Classification I. Agents of mainly historical interest 1. Ethyl chloride 2. Chloroform 3. Trichloroethylene 4. Cyclopropane 5. Methoxyflurane 6. Enflurane II. Agents in occasional use 1. Diethyl ether III. Agents in clinical use 1. Halothane 2. Isoflurane 3. Sevoflurane 4. Desflurane 5. Nitrous oxide IV. Agent undergoing clinical trials-Xenon A comparison of clinical effects of common inhalational anaesthetic agents in use is given in Table 48.2. Only important effects have been mentioned for the benefit of students.

Principles of Anaesthesiology

HALOTHANE This was a very popular anaesthetic agent till recently but is gradually being replaced by isoflurane. Reasons for dwindling popularity are: • Myocardial depression • Arrhythmogenicity • Remote possibility of halothane hepatitis • Easier availability of isoflurane (a safer agent). Halothane hepatotoxicity Two types of halothane induced hepatic dysfunction are recognised: Type 1: It is mild, self-limiting and more common. It is associated with mild increases in liver enzymes but not jaundice. It is caused by reductive metabolism of halothane. Type II: On extremely rare occasions (widely quoted incidence l :35,000), the administration of halothane may be associated with the production of hepatitis. This entity, known as halothane hepatitis is largely a diagnosis of exclusion. It is fulminant with a high mortality rate (up to 50%). It is associated with fever, jaundice and grossly elevated liver enzymes. The toxic metabolite, trifluoroacetic acid reacts with liver proteins and triggers immune-mediated reaction in genetically susceptible individuals. Risk factors for development of halothane hepatitis are female gender, obesity, prior history of postanaesthetic jaundice, genetic susceptibility, repeated administration and enzyme-inducing drugs such as phenobarbitone. Halothane is metabolised up to 20%, and sevoflurane (2%) and isoflurane (0.2%). Thus, hepatic dysfunction, although reported with sevoflurane and isoflurane, is extremely rare.

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ISOFLURANE • It is a halogenated ether. • lsoflurane causes less cardiac depression. Hence, it is usec in patients with cardiac disease. • It causes less cerebral vasodilatation than halothane. Hence, it is preferred during neurosurgery. • Isoflurane is now routinely used in all cases in many centres because it produces less arrhythmias and myocardial depression, is associated with only a remote risk of hepatitis and is not expensive. • It has a pungent smell and hence cannot be used for inhalation induction. SEVOFLURANE (Fig. 48.3) • • • • •

It is a newer general anaesthetic agent and has a sweet smell. Useful for inhalation induction, especially in children. Induction and recovery are faster than with halothane. It produces minimal myocardial depression. It is also a useful agent for induction of anaesthesia in patients with difficult airways. • Its use is limited only by its cost. DESFLURANE • • • •

It is another new volatile anaesthetic agent. It causes vasodilatation and increase in heart rate. Induction and recovery are very fast. However, it is irritant to the respiratory tract and hence is not suitable for inhalational induction. • It also requires a specially constructed heated vaporiser because of its high volatility.

Comparison of different inhalation anaesthetics Parameter Smell Inhalation induction Effect on CNS CNS depression Induction and recovery Effect on RS Minute ventilation Bronchodilatation Effect on CVS Myocardial contractility Heart rate Blood pressure Arrhythmias Effect on uterine muscle tone Effect on skeletal muscle

Halothane

lsotlurane

Sevotlurane

Pleasant Suitable

Pungent Not suitable

Pleasant Suitable

Rapid, progressive Fast

Rapid, progressive Faster

Rapid, progressive Fastest

Depressed +++

Depressed ++

Depressed ++

Relaxes

Relaxes

+++

H

Relaxes

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INTRAVENOUS ANAESTHETIC AGENTS Intravenously administered anaesthetic agents are more popula for induction of anaesthesia because it is more rapid and smootl than that associated with inhalational agents. They can also b, used for maintenance of anaesthesia, sedation during regiona anaesthesia, sedation in the ICU and treatment of statu epilepticus. They can be classified into rapidly-acting (acting withi1 one arm-brain circulation time) and slow-acting (those tha take longer than one arm-brain circulation time) (Key Bm 48.2).

MUM............� Fig. 48.3: Inhalation induction of anaesthesia in a child using sevoflurane. Note how the child is reassured by the comforting arm of the anaesthesiologist

INTRAVENOUS ANAESTHETICS

• Rapidly-acting: Thiopentone Propofol Etomidate

NITROUS OXIDE • Slower-acting: Ketamine High dose opioids • It is an anaesthetic gas which is compressed and supplied as a liquid in cylinders. Benzodiazepines • It is sweet smelling and nonin-itant. • It provides analgesia but is insufficient to produce an THIOPENTONE SODIUM adequate depth of anaesthesia when used alone. • It enhances induction of anaesthesia with the volatile It is an ultra-short acting barbiturate, available as a yellowish powder. It is used as a 2.5% solution (25 mg/ml). It is used in anaesthetics and reduces their requirement. • It does not produce significant depression of the a dose of 4-5 mg/kg intravenously. cardiovascular system. Clinical effects Uses CNS • It is used along with volatile anaesthetic agents as part of • Generalised depression of the CNS is observed within 15 balanced anaesthesia. to 20 s ofIV injection of thiopentone. Loss of eyelash reflex • A combination of 50% nitrous oxide and 50% oxygen is is used as an end-point. available as Entonox. It is used to provide labour analgesia. • It is a potent anticonvulsant. • It may also be used to provide analgesia for small procedures in dentistry. It is not an analgesic. To the contrary, 1t increases pain sensation (antanalgesic). Consciousness is regained within Side-effects 5 to 10 minutes. • It can diffuse into closed gas spaces such as pneumothorax, obstructed intestines, sinuses and middle ear, and can cause CVS barotrauma. The volume of a cavity can increase 3 to 4 • It produces myocardial depression, peripheral vaso­ dilatation and hypotension especially when large doses are times within 1-2 hours. Hence, nitrous oxide is best avoided administered rapidly. in patients in whom such expansion may be anticipated. • Its use is associated with increased incidence of • Profound hypotension may occur, especially in a patient postoperative nausea and vomiting. with hypovolaemia or cardiac disease. l t may induce • In prolonged administrations, it can affect vitamin B 1 2 tachycardia. synthesis causing megaloblastic anaemia. • Teratogenicity: This has been observed in pregnant rats RS • It reduces respiratory drive. A short period of apnoea is exposed to nitrous oxide for prolonged periods but not common. proved in human beings. Nitrous oxide is best avoided in • It can precipitate asthma in susceptible individuals. early pregnancy.

Principles of Anaesthesiology

Skeletal muscle There is poor muscle relaxation with thiopentone. Uterus and placenta • There is little effect on resting uterine tone. • It crosses placenta rapidly, although foetal blood concentration is far less than that observed in the mother. Eye • It reduces intraocular pressure. • The corneal, eyelash and eyelid reflexes are abolished. Side effects • Hypotension, tachycardia • Respiratory depression • Irritant to veins and can cause thrombophlebitis. If it extravasates, can cause tissue necrosis. • Intra-arterial injection: Usually inadvertent, can cause severe arterial spasm and pain. This may be treated with vasodilators and heparin. • Allergic reactions: Very rare. Contraindications Absolute 1. Airway obstruction 2. Acute intermittent porphyria 3. Previous hypersensitivity reaction

Relative

1. Hypovolaemia 2. Asthma

PROPOFOL This drug became commercially available in 1986. It is comparable to thiopentone but is five times more expensive. It is highly lipid soluble and is formulated in a white, aqueous emulsion containing soy bean oil and egg phosphatide. It is used in a dose of 2 to 2.5 mg/kg intravenously. The dose should be reduced in the elderly and in haemodynamically unstable patients. Clinical effects CNS • Propofol depresses the central nervous system within 20 to 40 s of injection. Loss of verbal contact is used as an end­ point. • Recovery is rapid and there is a minimal "hang-over" effect even in the immediate post-anaesthetic period. CVS • The arterial pressure decreases more than with thiopentone. This is due to peripheral vasodilatation. The degree of hypo­ tension can be reduced by slowing the rate of administration. • Heait rate increases slightly.

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RS • After induction, apnoea occurs commonly and for a longe1 duration than with thiopentone. • It causes ventilatory depression, particularly with opioids. • It has no effect on bronchial muscle tone. GIT, uterus and placenta: Propofol has no effect on Gl motility but causes mild transient decrease in hepatorenal function. Uses (Table 48.3) • As an induction agent • As an infusion, can be used to provide total intravenous anaesthesia • To provide conscious sedation • As continuous infusion to sedate patients in the ICU, since its half-life is very short. Adverse effects • Cardiovascular depression • Respiratory depression • Pain on injection • Allergic reactions. ETOMIDATE • Etomidate is a rapidly acting intravenous anaesthetic agent with a short duration of action of 3-5 minutes. • It is a very cardiostable agent and is used to induce anaesthesia in cardiac patients and in haemodynamically unstable patients. • However, continuous infusions are not advisable as it is known to depress synthesis of cortisol by the adrenal gland and impair response to ACTH. • It is used in a dose of 0.2-0.3 mg/kg IV. Adverse effects 1. Suppression of synthesis of cortisol with infusions. 2. Excitatory phenomena: Involuntary movements, cough 3. Pain on injection and venous thrombosis 4. Nausea and vomiting KETAMINE HYDROCHLORIDE Ketamine differs from other intravenous anaesthetic agents in many respects and produces dissociative anaesthesia, rather than generalised depression of the central nervous system. It is used in a dose of 1-2 mg/kg IV or 4-5 mg/kg IM. Clinical effects CNS • It induces anaesthesia within 30-60 s of intravenous injection and lasts for l 0-20 mins. It is effective within 3--4 mins of intramuscular injection and lasts for 15-25 mins.

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Comparison of different intravenous anaesthetics Parameter

Thiopentone

Propofol

Ketamine

Rapid

Rapid

Slow

Hl H

Hl H

Dissociative anaesthesia

Pain sensation

Antanalgesia

No change

Profound analgesia

Hallucinations

None

None

Present

Minute ventilation

l

H

H

Bronchodilatation

Can precipitate bronchospasm

No effect

Bronchodilator

Not blunted

Blunted

Maintained

l i l

l i H

ii ii ii

H

H

l l

l l

Onset Effect on CNS CNS depression Cerebral blood flow

Effect on RS

Airway reflexes Effect on CVS Myocardial contractility Heart rate Blood pressure Arrhythmias Muscle tone Intraocular pressure

• It produces anaesthesia by dissociating the cerebral cortex from the limbic system. • It is a potent analgesic. • It increases cerebral blood flow and intracranial pressure. CVS The heart rate, blood pressure and cardiac output increase. RS • Respiration is usually well-maintained with ketamine although transient apnoea may occur occasionally. • Ketamine is a good bronchodilator. Skeletal muscle There is increased muscle tone. Some spontaneous and involuntary movements may occur. GIT Increased salivation can occur and can be prevented by using anticholinergic agents. Eye lntraocular pressure increases. Uterus and placenta It readily crosses the placental barrier and hence should be given in lower doses in pregnant patients. Adverse effects • Emergence delirium • Hypertension and tachycardia • Increased intracranial pressure

ii

i i

• Vivid and unpleasant hallucinations are known with keta­ mine and can be prevented by prior injections of benzodiazepines. Uses (Table 48.3) • Patients in severe hypotension, shock • Paediatric anaesthesia • Analgesia and sedation • Bronchial asthma • Difficult locations: Accident sites, war casualties. PHYSIOLOGY OF NEUROMUSCULAR JUNCTION When a nerve impulse arrives at the neuromuscular junction through the motor neuron, acetylcholine (ACh) molecules are liberated from the nerve endings into the junctional cleft. Acetylcholine molecules act as neurotransmitters by interacting with the nicotinicACh receptors on the postjunctional muscle membrane at the motor end plates. This induces the opening of ionic channel of the receptor to allow ionic (sodium, calcium) flux into the muscle. The sudden influx of sodium results in depolarisation and muscle contraction. Acetylcholine receptor The acetylcholine receptor is flower-shaped with five petal­ like structures. These five subunits are named a(2), �(l), £(1) and 8(1). E ach alpha subunit must be occupied by an acetylcholine molecule to open the channel. This acetylcholine

Principles of Anaesthesiology

receptor at the neuromuscular junction is nicotinic in nature and thus different from those in the rest of the body. MUSCLE RELAXANTS These are drugs that interfere with the combination of acetyl­ choline molecules with their receptors. These block neuro­ muscular transmission and cause relaxation of the muscle resulting in muscle paralysis. Neuromuscular blockers are of two types: Depolarising and nondepolarising muscle relaxants. DEPOLARISING MUSCLE RELAXANTS (SUCCINYL CHOLINE) It is the only depolarising muscle relaxant in clinical use. It has a molecular structure similar to acetyl choline. It combines with the alpha subunit oftheACh receptor and produces muscle contraction. However, unlike acetyl choline, it has a prolonged action. Continued depolarisation of a muscle results in accommodation blockade and the muscle relaxes. Dose: 1-1.5 mg/kg intravenously. Onset of action: Within 60 seconds Duration of action: 3-5 minutes Metabolism: By plasma cholinesterase. Uses Succinyl choline is the only muscle relaxant which has the shortest time to onset of action (60 seconds) and shortest duration of action (3-5 minutes). It is used: • To facilitate endotracheal intubation in 'full-stomach' patients. • It is useful in patients with difficult airway because it gives very good relaxation to facilitate intubation but ifintubation fails, the patient is likely to resume spontaneous breathing early and hypoxic brain injury may be avoided. • To maintain paralysis for short procedures. Adverse effects • Muscle pains: The initial depolarisation that occurs due to succinyl choline causes uncoordinated contraction (fasciculations) of different groups of muscle fibres. This can cause severe muscle pain postoperatively. • Bradycardia, especially ifa second dose ofsuccinyl choline is given. It is easily avoided by pretreatment with atropine. • Hyperkalaemia in patients with renal failure, burns, massive crush injury, etc. • Increase in intracranial pressure • Increase in intraocular pressure

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_,.,,.

Prolonged action in patients deficient in pseudo· cholinesterase (plasma cholinesterase). This occurs as , genetic problem in a small number of patients but may bt an acquired problem as in severe liver disease. • Malignant hyperthermia is a disorder that is unique anc life-threatening disorder precipitated by exposure tc inhalation anaesthetics and succinyl choline in susceptible patients. In malignant hyperthermia, the metabolic rate of muscle cells is increased tremendously due to a defective ryanodine receptor which is necessary for reuptake of calcium after a depolarisation. • Those with a family history of anaesthetic mishaps, neuromuscular diseases such as muscular dystrophy may be susceptible to this disorder and succinyl choline is best avoided in these patients. It is not always possible to identify latent muscular dystrophies in infants and children. Administration of succinyl choline in these patients can be disastrous. Hence, the use of succinyl choline should be avoided in children less than 2 years unless an indication such as a full stomach exists. Even then, rocuronium may be considered as an alternative. NONDEPOLARISING MUSCLE RELAXANTS Pancuronium, vecuronium, rocuronium, atracurium and cisatracurium are drugs belonging to this group in clinical use. The nondepolarising muscle relaxants combine with the ACh receptors but do not have any intrinsic effect on the muscle. They cause muscle paralysis by preventing the acetyl choline molecules that are released from the nerve terminal from combining with the acetyl choline receptors on the postsynaptic membrane and producing their action (competitive inhibition). Even if one alpha subunit is combined with a molecule of nondepolarising muscle relaxant, the muscle cannot contract in response to a nerve impulse and gets paralysed. The muscle regains its power when the muscle relaxant gets metabolised. The nondepolarising muscle relaxants can be classified according to their duration of action as follows: • Short-acting (10-20 min): Mivacurium • Intermediate-acting (20-30 min): Atracurium, vecuro­ nium and Rocuronium • Long-acting(> 45 min): d-Tubocurarine and pancuronium. Uses 1. To facilitate endotracheal intubation 2. To maintain paralysis during anaesthesia and in the ICU. REVERSAL OF NEUROMUSCULAR BLOCKADE At the end of anaesthesia, the muscle relaxation produced by the nondepolarising muscle relaxant is usually reversed. This is to ensure good recovery ofmuscle power to maintain airway and respiration.

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Management of airway is one of the basic skills acquirec Acetylcholine molecules are broken down by choline­ sterases. Anticholinesterases such as neostigmine block the by an anaesthesiologist. This is relatively easy in mos action of cholinesterase, thus allowing acetyl choline molecules individuals. Difficult airway is the term given when there i: to accumulate in the neuromuscular junction. The block difficulty with mask ventilation, endotracheal intubation, o p roduced by the nondepolarising muscle relaxants is both. competitive. When acetyl choline molecules increase in number due to the action of the anticholinesterase, and BAG-MASK VENTILATION nondepolarising muscle relaxant molecules decrease in number When a person becomes unconscious, the tongue and thf due to metabolism, muscle power returns. Neostigmine is the epiglottis fall back and obstruct the airway. Since they an only anticholinesterase in clinical use. Its dose is 0.05 mg/kg attached to the mandible, lifting up of the mandible also lifti body weight. Neostigmine increases the amount of acetyl these two structures and opens airway. This is usually done b ) choline not only at the neuromuscular junction but also in the head-tilt and chin-lift method as during cardiopulmonar ) entire body. It can cause the muscarinic effects of acetyl choline resuscitation. Jaw thrust is more popular with anaesthesio­ such as bradycardia, bronchoconstriction, etc. Hence, logists. The patient may be ventilated using a 'bag' (either a neostigmine is always combined with atropine (0.025 mg/kg) self-inflating bag or anaesthetic circuit) and a facemask or glycopyrrolate (0.01 mg/kg) to counter the muscarinic (Fig. 48.4). The mask is triangular in shape, the narrow portion effects. of which is placed at the bridge of the nose and the base placed in the depression between the lower lip and chin. The mask is Adequate recovery from neuromuscular blockade held in place by an E-C technique (Fig. 48.5). Clinical indicators • Opening of eyes without furrowing of forehead • Good hand grip • Raising arms against gravity • Good cough • Ability to lift head against gravity (sustained head-lift) for at least five seconds. Objective criteria The amount of neuromuscular blockade can be checked using a peripheral nerve stimulator. The ulnar nerve is stimulated and the response of the adductor pollicis muscle is checked. If good muscle contractions are seen in response to the nerve stimulation, the muscle power is said to have returned. Similarly, posterior tibial nerve and facial nerve may also be used to monitor neuromuscular junction. The patient is allowed to breathe spontaneously and his trachea extubated only when there is clinical evidence of com­ plete recovery from neuromuscular blockade. If there is inade­ quate recovery of muscle power, the patient may need to be ventilated artificially till muscle power is normal.

ENDOTRACHEAL INTUBATION Endotracheal intubation is the most definitive way of maintaining airway in patients who require muscle paralysis and require intermittent positive pressure ventilation. It involves introduction of a tube into the trachea for maintaining the patency and protecting the airway as well as to ensure adequate oxygenation and ventilation. Whenever general anaesthesia is induced and needs to be maintained for long periods, endotracheal intubation is done. Indications • To administer general anaesthesia for long(>1-2 h) periods. • To maintain patency of the airway in unconscious patients.

AIRWAY MANAGEMENT General anaesthesia involves induction of unconsciousness during which the patient's ability to maintain his airway and breathing are impaired. It thus becomes necessary that the anaesthesiologist maintains and protects the patient's airway. In addition, not only anaesthesia but also oxygenation and ventilation are maintained well only if the airway is patent and secured.

Fig. 48.4: Maintenance of airway and ventilation using bag (anaesthetic circuit) and mask

Principles of Anaesthesiology

Fig. 48.5: The E-C technique of holding a face mask to get airway seal. The thumb and index fingers form a 'C' at the top of the mask holding it firmly down on the face. The middle, and ring fingers lift up the mandible as well as provide head tilt. The little finger aids in providing jaw thrust. Note that the finger pressure should be given on the bony part and not on the soft tissues under the chin

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Fig. 48.6: Macintosh laryngoscope. Note the curved blade. Sizes 1, 2 and 3 are displayed

• To protect lungs from aspiration of regurgitated gastric contents. • To ensure delivery of adequate tidal volumes to the lungs. • To clear excessive and retained secretions from the lungs. Contraindications Endotracheal intubation may be extremely difficult and even dangerous. A tracheostomy may be better in such situations. • When the upper airway integrity is lost as in extensive maxillofacial injury with bilateral fractures of mandible and maxillae. • Injuries to the neck with laryngeal rupture • Large tumours of the upper airway. Equipment Laryngoscopes These consist of a handle and a blade. The handle contains batteries. The blade has a flange to push the tongue towards the left side. This ensures more room for visualisation of the glottis. A bulb nearer the tip of the blade lights up when the handle and blade are at right angles to each other and electrical contact is made. • There are several types of laryngoscopes to aid in different situations. - Macintosh type blade (Fig. 48.6) is curved and is popular for use in adults.

Fig. 48.7: McCoy laryngoscope. Note the retractable tip of the laryngoscope blade

- The Miller blade is straight and is used in children and in adults with difficult airway. The McCoy laryngoscope (Fig. 48.7) has a tiltable tip. Bullard laryngoscope for use in patients with cervical spine immobility. - Fibreoptic laryngoscopes are flexible. - Videolaryngoscopes have a small camera on the blade a little proximal to the tip and provide a better view of 'difficult to see' larynx. - Short-handled laryngoscopes are available for use in difficult airways, e.g. pregnant women, obese patients.

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Manipal Manual of Surgery

Fig. 48.8: Endotracheal tube. (A) Standard 15 mm connector (B)

Pilot balloon (C) Inflatable cuff (D) Beveled patient-end (E) Black line to indicate optimum positioning of the tube. This line is usually placed just above the glottis Endotracheal tubes (Fig. 48.8) It is a 'C'-shaped tube and is commonly made of polyvinyl chloride (PVC). The machine end has a standard 15 mm diameter connector. The patient end is bevelled and has an opening on the side just proximal to the tip called the Murphy's eye. This ensures patency of the tube even if the bevelled tip is against the tracheal wall.

MUM..........,� • • • •

ENDOTRACHEALTUBES�E

Adult male Adult female Children < 6 years Children > 6 years

8 or 8.5 mm/D 7 or 7.5 mm/D Age/3 + 3.5 mm/D Age/4 + 4.5 mm/D

Endotracheal tube size Endotracheal tubes are available in different sizes. Their size is indicated as the internal diameter in mm (Key Box 48.3). However, the correct size will depend on the growth of the child. Depth of insertion • The distance of several points on the tube from the patient end is marked in cm along the tube. The tube is fixed at 22 or 23 cm in adult men and at 20 or 21 cm in adult women. • In children, the following formula is used: Age/2 + 12 cm. The tube should be positioned such that its tip must lie above the carina but well below the glottis. Position A pillow (7-10 cm) under the patient's head enables mild flexion at the cervical spine. The head is then extended at the atlanto-occipital joint. This is called the intubating position or "sniffing position".

Fig. 48.9: Oral endotracheal Fig. 48.10: Nasal endotracheal

intubation

intubation

Route Endotracheal intubation can be done either orally or nasally (Figs 48.9 and 48.10). It can be done either under direct vision or indirectly using a fibreoptic scope. It may need to be done blindly when visualisation of the glottis by direct means is not possible and a fibreoptic scope is not available. In such cases, if the regular antegrade technique (mouth or nose to larynx) is not possible, retrograde intubation (larynx to mouth) may be tried. In the retrograde technique, a guide wire is passed from the cricothyroid membrane upward into the mouth or nose and the endotracheal tube is guided over it. Procedure (Key Box 48.4) The patient's head is placed in the sniffing position. The mouth is opened and the laryngoscope blade is introduced through the right angle of mouth along the tongue into the pharynx. Once the epiglottis is seen, the tip of the laryngoscope blade is pressed into the vallecula. This lifts up the epiglottis to reveal the glottis. The glottis is identified by the two pearly white vocal cords. Once the cords are seen, the endotracheal tube is inserted between them into the trachea. It may also be done with the patient awake after administer­ ing local anaesthesia to the upper airway when a difficult intubation is anticipated.

NHMtllllllllllllli� ENDOTRACHEAL INTUBATION

• • • • •

Orotracheal or nasotracheal Direct or indirect (using fibreoptic laryngoscope) Under vision or blind Anaesthetised or awake Antegrade or retrograde

Principles of Anaesthesiology

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Many airway adjuncts are available for use when a difficult airway is encountered, especially when it is unanticipated. These include oropharyngeal airway, nasopharyngeal airway, laryngeal mask airway and Combitube.® Confirmation of correct placement of endotracheal tube Correct position of the endotracheal tube may be confirmed by the following • Endotracheal intubation under vision • Bilateral visible chest rise • Bilateral equal air entry in the lungs • Absent breath sounds in the epigastrium • A square wave normal capnogram-gold standard • Prompt inflation of the deflated bulb of oesophageal detector device.

Fig. 48.11: Oropharyngeal airway

Complications The complications of endotracheal intubation may be classified as follows Immediate • Trauma to teeth, lips, tongue, pharynx or larynx • Haemodynamic changes-tachycardia, hypertension, myocardial ischaemia • Misplaced tube-accidental extubation, oesophageal intubation Delayed Laryngeal granuloma, laryngeal or subglottic stenosis Oropharyngeal airway (Figs 48.11 and 48.12) • It is available in various sizes. The correct size is chosen such that when it is placed along the side of the patient's face, it should extend from the angle of mouth up to the tragus. This is inserted along the tongue and reaches up to the posterior pharyngeal wall. • Care should be taken not to push the tongue backwards with the airway itself. • To avoid that, the airway is inserted with its concavity towards the palate and then turned once 50% of it is inserted and passed further. • Please note that an oropharyngeal airway is excellent in patients who are completely unconscious. Patients who are conscious do not tolerate it. In patients who are semiconscious, it may initiate a gag reflex and induce vomiting. In such patients, a nasopharyngeal airway 1s a better choice. Nasopharyngeal airway (Figs 48.13 and 48.14) • It is a soft tube made of latex or silicon. It is available in various sizes. The correct size is the same size as an endotracheal tube for that patient. • The correct length is chosen such that when it is placed along the side of the patient's face, it should extend from

Fig. 48.12: Insertion of oropharyngeal airway. Take care not to push the tongue backwards

Fig. 48.13: Nasopharyngeal airway

Fig. 48.14: Insertion of nasopharyngeal airway

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the nostril up to the tragus. This is inserted along the nose and reaches up to the posterior pharyngeal wall. Laryngeal mask airway (Fig. 48.15) • This is a supraglottic airway that has revolutionised airway management during anaesthesia. It has a tube or a shaft with a standard 15 mm connector at the proximal end to connect to the anaesthetic circuit. The patient end has an oval-shaped inflatable cuff which when inserted rests just above the larynx and creates an airtight seal.

Fig. 48.15: Laryngeal mask airway Uses

• To administer anaesthesia • To maintain airway in patients with difficult airway where endotracheal intubation is not possible and are at risk of hypoxia • It may also be used as a conduit for passage of endotracheal tube, either blindly or with the use of fibreoptic broncho­ scope. Endotracheal extubation Endotracheal extubation is as important as intubation. A patient's trachea may be extubated when the following criteria are met • Oxygenation and ventilation are satisfactory • Patient is haemodynamically stable. • Patient is fully conscious • Able to maintain his airway patency • Airway reflexes are intact • Able to cough and clear airway. Equipment for reintubation and personnel skilled in intubation should be readily available. After a good oropharyngeal suction to clear the secretions, the cuff is deflated and the tube removed. Oxygen should be administered by face mask and the patient monitored till he is stable and ready to go to the ward. MONITORING IN ANAESTHESIA The administration of anaesthesia is associated with changes in the internal homeostasis of the patient, especially the cardiac and respiratory systems. Constant monitoring of the various body systems is necessary to ensure the well-being of the patient and prompt recovery from anaesthesia at the end of

surgery. Identification and prompt treatment of any untoward changes should alleviate complications due to anaesthesia. • Monitoring used in anaesthesia may be classified into: noninvasive and invasive monitoring. The extent of monitoring depends on the patient's preoperative condition, the extent of surgery, the type of anaesthesia and the facilities available. • Most patients are noninvasively monitored. Invasive monitoring becomes necessary when considerable haemodynamic instability exists or is expected to occur perioperatively. • Minimum monitoring standards (according to guidelines from Indian Society of Anaesthesiologists) for a patient undergoing any type of anaesthesia are: Noninvasive blood pressure (NIBP) Electrocardiogram (ECG) Pulse oximetry • Monitoring capnography although not mandatory in India, is an important monitor in patients undergoing anaesthesia. In addition, monitoring of body temperature, anaesthetic gases and neuromuscular blockade is desirable. NONINVASIVE MONITORING Basic noninvasive monitoring includes clinical observation of the patient and monitoring of the patient's haemodynamic parameters. Adequate cardiac output is associated with good urine output, warm and well-perfused peripheries and good capillary refill. Heart rate may be monitored with a finger

on the pulse. Blood pressure may be measured using a standard sphygmomanometer. More conveniently and accurately, heart rate and rhythm are continuously monitored using the electrocardiogram and blood pressure using automated noninvasive blood pressure monitoring systems. Baseline readings of the heart rate, blood pressure and oxygen saturation prior to induction of anaesthesia are recorded. After induction, the patients are continuously monitored and a record of these is made at least every five minutes thereafter. When a patient undergoes an extensive or a long procedure associated with large fluid shifts, his urinary bladder is catheterised so that hourly urine output can be measured. A normal person produces at least 0.5-1 ml/kg/hour of urine output. ELECTROCARDIOGRAM (ECG) The objectives of electrocardiographic monitoring (Fig. 48.16) are to monitor heart rate and rhythm and to look for myocardial ischaemia. Lead II is monitored commonly as it is best for identifying an-hythmias. VS detects left ventricular ischaemia. Hence, both Lead II and VS should be monitored continuously for patients suspected to have ischaemic heart disease. Subtle changes in the electrocardiogram on the monitor must be confirmed by a 12 lead-ECG.

Principles of Anaesthesiology

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oximeter consists oftwo light emitting diodes (LEDs), em ittini light at around 660 nm and 940 nm. Oxyhaemoglobin (OxyHb absorbs more oflight at 940 nm whereas reduced haemoglobi, (Hb) absorbs more light at 660 nm. The amount ofOxyHb i, tissues increases with each pulse wave.

Fig. 48.16: Multimodular monitor

NONINVASIVE BLOOD PRESSURE (NIBP) Most automated devices use an oscillotonometric technique and as a result the most accurate pressure is the mean arterial pressure. They tend to overestimate at low pressures and underestimate at high pressures. They may also give erroneous results in patients with atrial fibrillation or with other anhythmias. The cuff width (Fig. 48.17) is the most important determinant of the accuracy of the pressure reading. The cuff width should be 40% of the mid-circumference of limb (the length should be twice the width). Cuffs which are too narrow tend to overestimate BP while those which are too wide tend to underestimate BP.

Fig. 48.18: A portable pulse oximeter and capnograph. (A) The capnograph and adaptor, (B) Pulse oximeter probe, (C) Graphical display window that can be programmed to show either a plethysmograph (as seen in the photograph) or a capnogram

Fig. 48.19: The plethysmograph and oxygen saturation (Sp02) obtained by a p�lse oximeter

Fig. 48.17: Blood pressure cuffs of various sizes

PULSE OXIMETRY

The differential absorption of light by OxyHb and reduced Hb and the varying concentration of these two substances with arterial pulsations are utilised in the construction of the pulse oximeter. The pulse oximeter has one LED on, then the other on, then both off. This happens several times a second. The ratio of absorption of these two wavelengths of light by OxyHb and reduced Hb is related to the oxygen saturation using a nomogram.

The pulse oximeter displays the oxygen saturation of haemoglobin continuously, noninvasively and in real-time Significance (Figs 48.18 and 48.19). Its use has revolutionised the way • The normal oxygen saturation of a person breathing room air is around 98-100%. patients are monitored, both perioperatively and in the ICUs. • If the saturation dips below 90% or lower, hypoxia is said Working principle to be imminent. Saturation of 90% conesponds to Pa02 of The pulse oximeter works on the principle ofBeer-Lambert's 60 mmHg. law. This states that the amount oflight absorbed by a solution • Hence, the inspired oxygen concentration is adjusted to is directly proportional to the amount ofsolute in it. The pulse maintain a Sp02 of> 95% during anaesthesia.

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Sites of monitoring Fingers, toes, ear lobes; soles and palms in neonates/infants. Limitations • Movement: The pulse oximeter may not be reliable when there is movement of the part monitored. • Electrical interference may occur with concurrent use of diathermy. • Vasoconstriction: It may fail to pick up a pulse with profound vasoconstriction as in hypothermia or low cardiac output. • COHb: It may over-read in patients with significant carboxyhaemoglobin concentrations. • Meth-Hb: It may under-read in patients with significant methaemoglobin concentrations. CAPNOGRAPHY Working principle The amount of carbon dioxide in the exhaled gases can be measured using a capnograph. The capnograph (Fig. 48.20) passes infrared light through the gases to be analysed. The carbon dioxide in the gases absorbs the infrared light in proportion to its concentration. This absorption is measured and the carbon dioxide concentration is displayed according to a nomogram. When the carbon dioxide (CO2 ) concentration is displayed against time, a capnogram is obtained. The normal capnogram • The normal capnogram has four different phases as illustrated in Fig. 48.20. • Phase I: Dead space gases • Phase II: Mixed alveolar and dead space gases • Phase III: Alveolar gases • Phase IV: Inspiration • The CO2 concentration at the end of expiration is called end-tidal CO2 concentration (normal range: 35-40 mmHg) which represents the alveolar carbon dioxide concentration. Arterial CO2 concentration is 0-5 mmHg more than the end-tidal CO2 concentration.

Information obtained Carbon dioxide is excreted from the body only through one organ in the body, i.e. lungs. A normal capnogram obtained from the exhaled gases helps identify the correct position of the endotracheal tube. • If the capnograph registers zero levels of carbon dioxide, the endotracheal tube is malpositioned as in oesophageal intubation or accidental extubation. Mechanical ventilator disconnection also results in zero ETC02 levels. • The ETC02 levels may also reduce to near zero levels if no carbon dioxide returns to the lungs from the tissues as in profound hypotension and an impending cardiac arrest may be suspected. • However, the ETC02 will register zero if the patient is in cardiac arrest since there is no circulation and no carbon dioxide is brought to the lungs. In such situations, a capnograph cannot be used to identify the correct position of endotracheal tube. The capnograph will register CO2 only if effective CPR is being given. • However, a capnograph is extremely useful in assessing the effectiveness of CPR. If ETC0 2 is > 15 mmHg during CPR, chances of successful resuscitation are higher. • A capnograph is also useful to detect hypo or hyperventi­ lation, bronchospasm and recovery from neuromuscular blockade. Limitations • When there is an increase in physiological dead space ventilation (that part of ventilation that does not take part in gas exchange), the arterial to ETC02 gradient increases. • In such situations, arterial blood gas analysis can be done to determine the gradient and then its trend can be followed. INVASIVE MONITORING DIRECT ARTERIAL PRESSURE MONITORING Direct arterial pressure monitoring is preferred whenever haemodynamic instability exists or is expected to occur perioperatively. In such situations, invasive measurement enables beat to beat monitoring of arterial pressure. A cannula is inserted into an artery and connected to a pressure transducer. The monitor displays beat to beat blood pressure both numerically as well as graphically.

Fig. 48.20: The normal capnogram: Phase I-dead space gases, Phase II-mixed dead space and alveolar gases, Phase Ill­ alveolar gases, Phase IV-inspiration. The arrow is pointing towards the end-tidal carbon dioxide concentration {ETC02)

Sites • Radial artery: Most common (Fig. 48.21) • Dorsalis pedis artery: When radial arteries are inaccessible or they have already been used.

Principles of Anaesthesiology

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• Afterload: The dicrotic notch is lower on the downstrok, of arterial waveform when afterload is reduced. What size cannula? • A 20-gauge cannula may be used for the radial, brachia! o dorsalis pedis arteries in adults. • A long 18 gauge nonkinking cannula is preferred for femora arterial lines in adults. • A 22-gauge cannula in children. Complications • Ischaemia distal to cannula: Normally collateral circulatior is adequate to maintain perfusion distal to the arteria cannulation site. Occasionally ischaemia may occur wher associated with low cardiac output, sepsis, shock, high-dost vasopressors or vasculitis. • Exsanguination: In case of accidental disconnection, blooc loss of up to 500 ml/min can occur (through 18 G cannula) • Spurious result: Wrong position or calibration of transducen • Infection • Intra-arterial injection of drugs. CENTRAL VENOUS PRESSURE MONITORING A catheter inserted into the central vein with its tip positioned in the superior vena cava enables measurement of the right atrial pressure or central venous pressure. It also provides a good venous access. Normal values: 2-5 cmH20 in a spontaneously breathing patient. 5-10 cmH20 in a mechanically ventilated patient. Figs 48.21A to C: Radial arterial cannulation: (A) Cannulation,

(8) Catheter securely taped in place and labelled 'Arterial line­ Do Not Inject!', (C) The arterial waveform and pressure

• Femoral artery: If the peripheral circulation is sluggish, blood pressures are too low and the radial arteries are not felt, cannulation of brachial or femoral arteries may be considered. However, these sites should be changed to more peripherally placed catheters as early as possible to avoid complications such as distal ischaemia. • The distal circulation must be assessed periodically by capillary refill time, colour of the digit, nail bed, pulse oximetry, etc. What information is obtained? The invasive arterial pressure monitoring provides the following information • Systolic, diastolic and mean blood pressures • Heart rate and rhythm • Contractility: The upstroke of the arterial waveform is steep if the heart has good contractility. • Preload: The systolic variation in arterial pressure > 10% with respiration indicates hypovolaemia.

Indications of central venous cannulation 1. To guide fluid therapy as in hypovolaemia, low cardiac output states, renal failure 2. For total parenteral nutrition 3. To infuse potent drugs such as antibiotics, vasopressors, chemotherapeutic agents 4. When peripheral venous access is not available 5. For PA catheterisation and cardiac output measurement. Sites of insertion • Internal jugular vein (IJV): Right IJV is preferred over left IN because: - RIN is more in line with the superior vena cava. - Injury to thoracic duct is avoided. • Subclavian veins (Figs 48.22 and 48.23). • Peripherally placed central venous catheters may also be useful in measuring CVP if the tip of the catheter is intrathoracic. • Femoral vein catheters may also be used provided the tip of the catheter is close to the diaphragm in the inferior vena cava. However, it is not preferred as it is close to the inguinal region and maintaining asepsis is difficult. lt also interferes with the patient's mobility.

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• It can change with changes in right ventricular complianc{ also. For example CVP does not reflect left ventricula1 filling in patients with pulmonary hypertension or in righ· ventricular dysfunction. In such cases, dynamic changes ir CVP are more useful than absolute values.

Fig. 48.22: Subclavian venous cannulation: Locating subclavian vein

Complications of central venous catheterisation Associated with insertion • Bleeding • Carotid artery puncture • Pneumothorax • Haemothorax • Air embolism • Arrhythmias Associated with use • Catheter-related infection and sepsis • Disconnection, leading to bleeding or air embolism • Pleural or pericardia! effusion due to misplaced catheter

Fig. 48.23: Subclavian venous cannula in place

NHM.........,.�

PULMONARY ARTERIAL (PA) CATHETERISATION These are long catheters placed in the PA through the central veins, right atrium and right ventricle (Fig. 48.24). The tip of this catheter ends in a small distal branch of the pulmonary artery. When the balloon on its distal tip is inflated, the pressure distal to the catheter reflects the left atrial pressure which may reflect left ventricular end-diastolic pressure and hence the preload more accurately (Fig. 48.25). PA catheter insertion may be associated with a number of complications and must be used judiciously.

FLUID CHALLENGE TEST Amount of fluid given over 10 min CVP (cmH20) 8 200 ml 8-13 100 ml >13 50ml • If CVP rises by > 5cmH20, stop fluid administration • If CVP rises by < 5 but > 2 cmH 20, stop fluids, wait for 10minutes and then give a smaller volume • If CVP rise is < 2 cmH20, repeat fluid administration.

CVP as a guide to fluid replacement (Key Box 48.5) CVP may be used as a guide to right ventricular filling. The 5-2 rule is a useful way of assessing fluid responsiveness. Baseline CVP is recorded and then fluid is infused (50-200 ml of colloid over 10 min) if hypovolaemia is suspected. Limitations • The CVP displayed depends on correct positioning of the tip of the catheter. • The position and calibration of the transducer are important. The transducer must be placed at the level of the midaxillary line (supine patient) and the sternal angle (sitting patient) and then zeroed to atmospheric pressure.

Fig. 48.24: Pulmonary artery catheter

Uses • If a PA catheter is in place, 7-3 rule can be used to guide fluid challenge similar to the 5-2 rule with a CVP catheter. • A rise in PCWP may be the earliest indicator of left ventricular dysfunction. A new onset mitral regurgitation can also be detected in the wedge pressure trace. • Some PA catheters can be used to pace the left ventricle • Cardiac output measurements are possible.

Principles of Anaesthesiology

RA

RV

PA

PCWP

40

20

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pKa The pKa of a drug is the pH at which the ionised and nonionisec portions of the drug are equal. The lower the pKa, lower is th, degree of ionisation for any given pH. The nonionised portio1 is lipophilic and crosses the cell membrane more easil; hastening the onset of nerve blockade. For example, lignocainc has a pKa of 7.9 and acts faster than bupivacaine with a pK: of 8.1. pH A cidosis decreases the proportion of the nonionised drug anc reduces the amount of drug able to cross the membrane. Tha is why local anaesthetics do not work optimally when injectec into an infected tissue (pH is acidic in these tissues).

Fig. 48.25: Insertion of a PA catheter with the pressure trace display as a guide. The corresponding position of the catheter in the heart is shown diagrammatically below

LOCAL ANAESTHETICS Local anaesthetics are drugs when injected around the nerves block impulse conduction distal to the site of injection and produce analgesia and anaesthesia in that area. Classification Local anaesthetics consist of a hydrophilic tertiary amine group linked to a lipophilic aromatic group. They are classified into two main categories based on this linking group: the aminoamides and the aminoesters. • Aminoesters: Procaine, chloroprocaine, tetracaine • Aminoamides: Lignocaine, bupivacaine, ropivacaine. Mechanism of action A nerve impulse is transmitted by progressive opening of sodium channels across the membrane and sudden influx of sodium into the intracellular fluid. Local anaesthetics produce sodium channel blockade. They block the fast sodium channels and the sodium influx, thus blocking all impulse transmission across the membrane. Local anaesthetic exists in two forms: Ionised and nonionised. The nonionised form is lipophilic and crosses the phospholipid membrane more easily. The ionised form is hydrophilic and blocks the channel in the open state and blocks nerve transmission (use-dependent blockade). The drug blocks the channel from the intracellular direction. Factors influencing activity Lipid solubility Higher the lipid solubility, higher is its ability to penetrate the lipoprotein membrane and hence greater is its potency.

Protein binding The greater the degree of protein (membrane proteins) binding. longer is the duration of action. Choice of local anaesthetic agents • Lignocaine: Skin infiltration-0.5-1 % Minor nerve block-I% Epidural-1.5-2% Spinal anaesthesia-5% hyperbaric (heavy) Two topical preparations-2% lignocaine jelly and 4% lignocaine spray for the mucosa! surfaces of the body. Note: l % means each ml contains IO mg of the drug, 2% to 20 mg/ml, and so on. • Bupivacaine: Skin infiltration, epidural-0.25%, 0.5% Spinal anaesthesia-OS%, hyperbaric (heavy). • Ropivacaine: Available as 0.2% for providing postoperative analgesia, labour analgesia and as 0.75% for spinal and epidural anaesthesia and nerve blocks. Maximum recommended doses of local anaesthetics for infiltration and blocks • Lignocaine-5 mg/kg • Lignocaine with adrenaline-7 mg/kg • Bupivacaine-2.5 mg/kg Ropivacaine-5 mg/kg, not to exceed 200 mg for minor nerve blocks. • Lignocaine (preservative free) when used as an antiarrhythmic agent is given IV, in a dose of 1-2 mg/kg. Clinical effects Local effects Local anaesthetics block the sodium channels in the neuronal membrane and thus the propagation of impulses across it. Systemic effects These drugs can produce systemic effects when high plasma levels of the drug are achieved. This may also be deliberate as when lignocaine is used as an antiarrhythmic agent. It is

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classified as Class Ia drug (sodium channel blockers) in the Vaughan Williams classification of antiarrhythrnic agents. When high plasma concentrations oflocal anaesthetics are achieved, either due to accidental intravenous injection ofthe drug or due to intravascular absorption oflarge amount ofdrug infiltrated in a region, systemic toxicity can occur. PEARLS OF WISDOM

Adrenaline containing preparations should not be used for nerve blocks offingers, toes and penis as it can cause ischaemia. Toxicity of local anaesthetics

The likelihood oftoxicity oflocal anaesthetics depends on several factors: 1. Amount of drug injected 2. Site of injection-vascularity 3. Addition of vasoconstrictors 4. Rapidity of injection 5. Nature of drug given 6. Presence ofassociated conditions such as low cardiac output or renal failure. PEARLS OF WISDOM

Bupivacaine and preservative-containing lignocaine are not for intravenous use. Amount and nature of drug injected

Systemic toxicity

Ifsignificant amount oflocal anaesthetics reach the tissues of heart and brain, they exert the same membrane stabilising effect as on peripheral nerve, resulting in progressive depression of function. The toxicity oflocal anaesthetics is dose-dependent. These drugs always produce central nervous system (CNS) toxicity first. As the plasma level rises, cardiovascular toxicity and collapse occur. The plasma levels of lignocaine required to produce cardiovascular collapse (CVS toxicity) is seven times that required to produce convulsions (CNS toxicity). Bupivacaine requires only three times the plasma level to produce cardiac toxicity as for CNS toxicity. Thus, bupivacaine has a greater potential for cardiotoxicity. PEARLS OF WISDOM

With all local anaesthetics, central nervous system toxicity always comes first. This is followed by cardiac toxicity. Clinical features oflocal anaesthetic toxicity are related to the plasma level of the local anaesthetic. The clinical effects and their relation to plasma level of lignocaine are given in Table 48.4.

Lignocaine is mostly used to produce peripheral neural conduction blockade. It can be used in doses not exceeding 5 mg/kg body weight for plexus blocks or infiltration. When combined with vasopressors such as adrenaline (I :200,000 = 5 µg/ml), the dose can be increased up to 7 mg/kg. The intravenous dose of lignocaine is 1-1.5 mg/kg when used as an antiarrhythmic. However, even a small dose such as 20 mg in an adult when injected accidentally into the carotid artery may be sufficient to produce convulsions. Bupivacaine is used only for nerve blocks or infiltration. It is not an antiarrhythmic drug. Bupivacaine is cardiotoxic and care should be taken not to exceed the prescribed doses. Circulatory collapse and cardiac arrest due to large doses of bupivacaine can be very resistant to resuscitation. It can be used in a dose ofup to 2.5 mg/kg body weight. Ropivacaine is a newer amide local anaesthetic agent which is similar to bupivacaine but with less cardiotoxicity. A levo isomer of bupivacaine, called levobupivacaine is also less cardiotoxic and has been put into clinical use recently. Site of injection

Certain sites are very vascular as compared to others. A higher plasma level of local anaesthetic is reached when the same amount of local anaesthetic is used for multiple intercostal nerve blocks as compared to brachia! plexus block.

The clinical effects and their relation to plasma level of lignocaine Plasma

CNS toxicity

CVS toxicity

Concentration (µg/ml)

5 5-10 Slurred speech, muscle twitching 10 10-15 15 20

25

Tingling, numbness, tinnitus, light-headedness. Loss of consciousness Convulsions Coma Respiratory arrest

Myocardial depression Cardiac arrhythmias Ventricular arrest

Principles of Anaesthesiology

Prevention of toxicity • Do not exceed recommended doses. • Aspirate to rule out presence of the needle tip in a vessel before injecting the drug. • Avoid injecting large boluses at once. Small boluses, given slowly to achieve the desired effect are safer. Treatment of local anaesthetic toxicity The toxicity of local anaesthetics manifests as CNS depression and convulsions. Maintenance of airway, breathing and circulation must be a priority. These convulsions generally last for a short period of time. • Patency of the airway must be maintained. • Oxygen by face mask. • Ventilation, if apnoea occurs. • Convulsions are treated with intravenous diazepam or thiopentone in incremental doses. • Cardiovascular collapse with ephedrine, inotropes and vasoconstrictors, and CPR as needed. • Arrhythmias must be treated appropriately. SPINAL AND EPIDURAL ANAESTHESIA • Injection of local anaesthetics around the spinal cord to produce a reversible blockade of impulses that pass through it, is called central neuraxial blockade. • When the local anaesthetic is injected into the cerebrospinal fluid bathing the spinal cord, it is called spinal anaesthesia (subarachnoid block). • When the local anaesthetics are injected into the epidural space to block the nerves that emerge from the spinal cord, it is called epidural anaesthesia. SPINAL ANAESTHESIA Physiological effects Nervous system The local anaesthetics spread from the site of injection by mixing with the cerebrospinal fluid (CSF). They reach the nerve fibres in the spinal cord and block transmission of impulses below the highest level of spread. The concentration of the drug is higher in the caudal regions and reduces with increasing distance cranially. A lower concentration of the drug is sufficient to block smaller fibres (C and A8) whereas the thick motor fibres (Aa) require a larger concentration to get blocked. Thus, a differential blockade is seen after a spinal anaesthetic and is as follows: • Motor block up to a certain level (depends on the dose of drug injected) • Sensory level of block 2 segments higher than motor block.

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• Sympathetic block about two to four segments above th, level of sensory block. Cardiovascular system • Hypotension: Due to the blockade of sympathetic nerve below the level of spinal block, there is profound vaso dilatation in the affected areas. A relative hypovolaemi: occurs and hypotension is usually seen. The extent o hypovolaemia depends on the preoperative volume status level of block and ability to compensate for the vaso dilatation. The vessels in the upper limbs constrict tc compensate for vasodilatation in the lower limbs. This i: described as pink trousers, blue jacket phenomenon. • Bradycardia: Heart rate is maintained in low blocks, but ir higher blocks (high thoracic), sympathetic nerve block of th( cardioaccelerator nerves can occur causing unopposed actior of the parasympathetic system and bradycardia. • Cardiac output also reduces in high spinal anaesthetics. Respiratory system • No changes in respiratory function are seen in spina: anaesthetics below T l O level. • When the level of spinal anaesthesia ascends, the intercostal nerves are gradually blocked. • The diaphragm is not easily paralysed as the phrenic nerve is a thick and strong nerve and arises from cervical nerve roots (C3,C4,C5). • In high spinals, the alveolar ventilation reduces and may lead to hypoxia and hypercarbia. Gastrointestinal system • Unopposed parasympathetic activity leads to constriction of gut with increased peristaltic activity. • Nausea, retching or vomiting may occur and may be the symptom of impending hypotension. These symptoms disappear when the hypotension is corrected. Occasionally, it may need administration of an anticholinergic or an antiemetic agent. • However, since the bowel is contracted and small and the skeletal muscle relaxation produced is greater, the surgeons find it easier to operate on such a gut. Indications • Any surgery below the level of umbilicus Contraindications Absolute • Patient refusal • Infection at the site of injection • Bleeding tendencies Relative • Hypovolaemia • Severe stenotic valvular heart disease

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Limitation Limited duration of block Preprocedure check A preprocedure check of the anaesthesia equipment, resuscitation equipment and drugs is made. An intravenous line is placed and monitoring commenced. The patient is then positioned for spinal anaesthesia. Position (Figs 48.26 and 48.27) The spinal anaesthetic may be administered with the patient in lateral or sitting position. Lateral: The patient lies either in the left or right lateral position. The back should be parallel to the edge of the operating table and perpendicular to the ground. The legs should be flexed at the hips as much as possible. Sitting position: The patient sits on the table, with the back bent forward. He is allowed to rest his arms on pillows. The back is cleaned with spirit and betadine and draped. Under aseptic precautions, the vertebral spines are identified in the lumbar region. The highest point of the iliac crest corresponds

Fig. 48.27: Spinal anaesthesia in the sitting position

3. 4. 5. 6.

Supraspinous ligaments Interspinous ligaments Ligamentum tlavum Dura and arachnoid

Paramedian approach: The needle is inserted a finger-breadth lateral to the spine and advanced in a slightly cephalad direction towards the midline. If the needle touches the lamina, it should be redirected medially. The correct position of the needle is identified by obtaining a free flow ofCSF. This approach helps access the subarachnoid space in those patients whose interspinous and supraspinous ligaments are calcified or in patients unable to bend enough to open the interspaces well. The local anaesthetic is now injected into the CSF, taking care not to displace the needle.

Fig. 48.26: Spinal anaesthesia in the lateral position, spinal needle is in place and CSF drop is seen at the hub

to L3-4 space. The L2-3, L3-4, L4-5 intervertebral spaces can also be used. A space higher than this is not used as the spinal cord ends at LI in adults. This point is lower in children and should be borne in mind in paediatric spinals. Approach The subarachnoid space may be approached either from the midline or by a paramedian technique. A subcutaneous wheal of local anaesthetic is raised in the chosen intervertebral space. Midline approach: The lumbar puncture needle is inserted in the midline, midway between the spines and perpendicular to the skin. The spinal needle passes through the following structures to reach the subarachnoid space: 1. Skin 2. Subcutaneous tissue

Complications These may be classified into Minor and Major based on the reversibility and seriousness of the complication. Minor Hypotension This is treated with intravenous fluids to compensate for the vasodilatation. If necessary, incremental doses of a vasoconstrictor may also be used. Bradycardia If the cardioaccelerator nerves (T1-T4) are blocked. This is usually easily treated with an anticholinergic such as atropine or glycopyrrolate. If profound, a small dose of adrenaline may be required (very rare). Postdural puncture headache (PDPH) The incidence of PDPH depends on the size of the needle used, number of punctures made, fluid status and ambulation. With finer (25 and 26# needles) and good hydration of the patient,

Principles of Anaesthesiology

PDPH is uncommon. This may be treated with rest, increased fluid intake, plenty of coffee and NSA!Ds. Rarely, an epidural blood patch (vide infra) may be required. Respiratory depression If the level of spinal anaesthesia is high and all intercostal muscles are paralysed, respiratory depression may occur. However, diaphragm, the principal muscle of respiration is supplied by the thick phrenic nerve which does not get blocked easily. Any respiratory depression seen during spinal anaesthesia is more due to hypoperfusion of the respiratory centre ( due to hypotension). This can be treated with respiratory support as required and stabilisation of blood pressure.

109�

at injection of this air or saline is made as the needle advance through the tissues. The entry of the needle into the epidun space is heralded when it penetrates the ligamentum flavur and there is a loss of resistance to injection of air or salinE This is taken as the end-point. An epidural catheter is passe, through this needle and advanced to about 3-4 cm into th space. The needle is removed and the catheter taped and fixe, to the back. A bacterial filter is attached to the injection por of the catheter (Figs 48.28A to C).

Retention of urine Backache: This is not a problem of spinal anaesthesia per se but may be due to faulty positioning during surgery.

Major 1. Infection: Arachnoiditis, meningitis 2. Nerve injury: Cauda equina syndrome

EPIDURAL ANAESTHESIA (Table 48.5) In this type of central neuraxial blockade, local anaesthetic is injected in the space around the dura (epidural space). The local anaesthetic blocks the nerves as they emerge through the intervertebral foramen. Some of it diffuses through the meninges into the spinal cord and acts on the spinal cord. Preprocedure check A preprocedure check of the anaesthesia equipment, resuscitation equipment and drugs is made. An intravenous line is placed and monitoring of heart rate, electrocardiogram, blood pressure and oxygen saturation is established and the baseline noted. Position The epidural puncture can be done with the patient in sitting position or in the lateral decubitus position. Technique The patient's back is cleaned with an antiseptic and then draped. Under aseptic precautions, epidural puncture is done using a 16 or 18# Tuohy needle. This needle has a blunt tip to reduce the risk of dural puncture. The needle is inserted either in the midline or by a paramedian approach. It passes through the same tissues as in lumbar puncture except the subarachnoid space. The needle is inserted along with its stylet and always advanced slowly from skin onwards. Once the subcutaneous tissue is entered, the stylet is removed. A 2 ml or 5 ml syringe with a freely moving plunger and containing either air, saline or both is then connected to the needle hub. A gentle attempt

Figs 48.28A to C: Epidural anaesthesia: (A) Eliciting Joss of resistance to air, (B) Epidural catheter attached to bacterial filter, (C) Epidural catheter taped to the back

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The epidural needle or catheter may accidentally enter an epidural vein or the subarachnoid space. To avoid injecting a large dose of local anaesthetic into either of these spaces, a test-dose containing a small amount oflocal anaesthetic (3 ml of2% = 60 mg lignocaine) and 15 µg ofadrenaline is injected. Any sensory or motor block following this dose would suggest an accidental dural puncture resulting in spinal anaesthesia. An accidental intravascular injection is identified by an increase in the heart rate and blood pressure within a minute of the injection. In either situation, the epidural catheter may need to be withdrawn or replaced. If neither response is seen, an epidural placement is assumed and the full dose of local anaesthetic is injected in divided doses. The patient should be continuously monitored till the block wears off. Complications 1. Postdural puncture headache (PDPH) The epidural needles are large and dural puncture results in a larger leak of cerebrospinal fluid (CSF). This results in low CSF pressures. Whenever the patient sits up or becomes ambulatory, a drag occurs on the brain and the meninges due to gravity and loss of CSF. This results in a typical postural headache referred to the occipital region. The pain disappears when he lies down supine. This is more common in obstetric patients. It may occur up to 2 to 7 days after lumbar puncture and may persist for up to 6 weeks.

Treatment Plenty oforal fluids may increase CSF production. Rest, plenty ofcoffee and NSAIDs may also help. Rarely, an epidural blood patch may be required. Epidural blood patch: If the headache is very severe, an epidural blood patch may be given. 10 to 15 ml of patient's own blood is drawn under aseptic precautions. Simultaneously, epidural puncture is made in the same space as the previous epidural puncture. The freshly drawn blood is injected into the epidural space which clots and seals the puncture hole. This is nearly 100% effective in relieving the headache. 2. Total spinal block When a large dose oflocal anaesthetic is injected intrathecally inadvertently, all spinal nerves are blocked, causing profound hypotension, bradycardia and collapse. If the patient is continuously monitored and treated promptly, this is completely reversible. Treatment 1. Volume infusion and vasopressors 2. Endotracheal intubation and ventilation as necessary. 3. Urinary retention 4. Meningitis, if aseptic precautions are not followed. 5. Cauda equina syndrome, adhesive arachnoiditis: Extremely rare.

Comparison of spinal and epidural anaesthesia Spinal anaesthesia I. Done in the lumbar region only 2. Confirmation of correct placement of needle by ensuring free flow of CSF 3. A small amount of local anaesthetic is used 4. 23-29 # LP needles used 5. Test doses not required 6. Onset of neural blockade is fast. So also side-effects 7. All the nerves are blocked below the level of anaesthesia 8. Limited duration. Continuous spinals are not routinely used. 9. Postdural puncture headache possible I0. No such problem

Epidural anaesthesia Can be done in the lumbar, sacral (caudal), thoracic or cervical regions. Placement confirmed by using loss of resistance to injection of air, saline or both Larger mass of local anaesthetic is injected Larger needles (16-20 #) are required Use of test doses advisable Onset slow as drugs have to penetrate the dura. So, less hypotension The local anaesthetic spreads both caudad and cephalad. Segmental block can be achieved Epidural catheters are routinely introduced and hence duration of anaesthesia can be prolonged with repeated boluses or continuous infusion through catheters PDPH not seen, unless dura is inadvertently punctured Inadvertent intrathecal or intravascular injection of large amount of drugs possible, with consequent complications such as total spinal blockade and local anaesthetic toxicity respectively.

Principles of Anaesthesiology

OTHER REGIONAL TECHNIQUES CAUDAL ANALGESIA This is a very popular technique in providing postoperative analgesia in children. It involves injection of local anaesthetics with or without opioids in the caudal epidural space. Procedure Position The patient is positioned in lateral position with the knees flexed and the back perpendicular to the ground. It can also be given with the patient in prone position. The area over the sacrum and the gluteal region is cleaned and draped. Needles A 22 or 23 G hypodermic needle or a scalp vein set is used to administer the block. Technique The needle is inserted at the apex of the sacral hiatus at a 60° angle to the skin. A distinct 'pop' or a 'give way' is felt as the needle punctures the sacrococcygeal membrane. The angle of the needle is then changed to about 15° to 20° to the skin and advanced a little further into the sacral epidural space. The latter step is optional and has to be done with caution as the dural sac may end relatively low in infants. After careful aspiration to rule out blood or CSF, a small dose of local anaesthetic is injected. There should be no resistance to injection. A subcutaneous injection should also be ruled out. Drugs 0.25% bupivacaine in a dose of 0.5 ml/kg is sufficient for perinea) and low sacral procedures, 1 ml/kg for lumbosacral procedures and 1.5 ml/kg for lower abdominal procedures. However, a total volume of 20 ml and a total dose of 2.5 mg/ kg of bupivacaine may not be exceeded.

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BRACHIAL PLEXUS BLOCK Injection of local anaesthetics injected around the brachi� plexus produces analgesia and even surgical anaesthesia i the upper limb. The brachia! plexus can be blocked by fou different approaches: interscalene, supraclavicular, infra clavicular or the axillary. Of these, the supraclavicular and th axillary techniques are the most popular. Supraclavicular brachia! plexus block Position The patient is positioned in supine position with the head tume, to the opposite side of the block. The area over the lower par of the neck is cleaned and draped. Needles A 22 G hypodermic needle, a scalp vein set or an insulate< needle with an internal nerve stimulator may be used tc administer the block. Ultrasound guidance is becomin� increasingly popular to guide nerve blocks. Technique The needle is inserted at a point I to 1.5 cm above the midpoin of the clavicle. The subclavian arterial pulsations are felt witl the thumb of one hand and the needle advanced posterior to i1 with a medial and caudal direction. Paraesthesia must be sough1 in the upper limb and the injection of local anaesthetic made at the point of paraesthesia. Undue pain during injection suggests intraneural injection. Careful aspiration for absence of blood to rule out intravascular injection is mandatory before injection of the local anaesthetic. Drugs • Lignocaine plain not exceeding 5 mg/kg • Lignocaine with adrenaline 7 mg/kg or • Bupivacaine not exceeding 2.5 mg/kg may be used.

Indications • Postoperative pain relief in children for perinea! and lumbosacral procedures. • It is also used to supplement general anaesthesia for perianal procedures in adults.

Indications • Intraoperative analgesia and postoperative pain relief in adults and children. • Sole anaesthetic in adults for procedures on the upper limb.

Contraindications • Absence of consent from parents/patients • Local infection • Bleeding tendencies.

Contraindications • Absence of consent • Local infection • Bleeding tendencies.

Complications

Complications • Haematoma • Intravascular injection of local anaesthetics • Pneumothorax.

• Intrathecal injection is possible, especially in smaller infants who have extension of dural sac down to S3. • Intravascular injection of large dose of local anaesthetics.

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AXILLARY BRACHIAL PLEXUS BLOCK Position The patient is positioned in supine position with the arm abducted to a right angle and the elbow bent at right angles to the arm. The area over the axilla is cleaned and draped. Needles A 22 G hypodermic needle, a scalp vein set or an insulated needle with an internal nerve stimulator may be used. Technique • The needle is inserted at a point just above the point of maximum pulsations of the axillary artery but parallel to the artery. This point should be as high in the axilla as possible. Paraesthesia must be sought in the upper limb and the injection of local anaesthetic made at the point of paraesthesia. Undue pain during injection suggests intraneural injection. Careful aspiration for absence of blood to rule out intravascular injection is mandatory before injection of the local anaesthetic. •

A single injection of the local anaesthetic can be made at this point. Alternately, half the total volume of injection can be made at a point on the other side of the artery.

Drugs Either bupivacaine not exceeding a total dose of 2.5 mg/kg; lignocaine plain 5 mg/kg or with adrenaline 7 mg/kg may be used. A volume 35-40 ml may be required. Indications Intraoperative analgesia, anaesthesia and postoperative pain relief in children and adults for procedures on the upper limb. Contraindications Absence of consent, local infection, bleeding tendencies. Complications Haematoma, intravascular injection of local anaesthetics ANKLE BLOCK This is a popular technique in providing intra- and post­ operative analgesia in adults undergoing procedures on the foot. Position The patient is positioned supine. The foot is raised by an assistant and the area around the ankle is cleaned and draped. Needles A 22 G hypodermic needle is used to administer the block.

Technique Ankle block involves blocking of 5 nerves. l . The posterior tibial nerve is blocked with 3-5 ml of local anaesthetic at a point midway between the medial malleolus and the heel, just behind the posterior tibial arterial pulsations. 2. The sural nerve may be blocked at a point midway between the lateral malleolus and the heel,just lateral to the Achilles' tendon. 3. The deep peroneal nerve is blocked at a point midway between the lateral and the medial malleoli lateral to the tendon of extensor hallucis longus and anterior tibial artery. 4. The saphenous nerve and the superficial peroneal nerves are easily blocked by raising a subcutaneous wheal of local anaesthetic between the malleoli anteriorly. Drugs Lignocaine plain not exceeding 5 mg/kg or bupivacaine not exceeding 2.5 mg/kg may be used. Indications Postoperative pain relief in adults for procedures on the foot. Contraindications Absence of consent from patients, local infection, bleeding tendencies. PAIN AND ITS RELIEF Introduction One of the main anxieties of undergoing an operation is the pain that is anticipated along with it. An anaesthesiologist's responsibilities do not end with providing safe perioperative care. He must also ensure that the pain associated with the surgery is relieved adequately so that the surgery itself is not so unpleasant an experience. Definition Pain is defined as an unpleasant sensory and emotional experience associated with actual or potential tissue damage, or described in terms of such damage. Pain can be acute or chronic. The pain that is seen immediately after surgery is acute postoperative pain and is easier to treat. The mechanism of perpetuation of chronic pain is less well understood and is more difficult to treat. Pain pathways Pain begins with injury to the tissue. Both the peripheral and the central nervous systems are involved in the ultimate pain perception (Fig. 48.29).

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ACUTE PAIN Four basic mechanisms are involved in the appreciation of pain: transduction, transmission, modulation and perception. Pain relief can be achieved by blocking each of these steps. Transduction The transduction of noxious stimuli begins with peripheral nociceptors. These nociceptors convert the stimulus (transducer) of injury (heat, pressure, mechanoception) to pain sensation. This is also assisted by the production of prostaglandins and leukotrienes in response to the injury. Nonsteroidal anti-inflammatory analgesics can be used to reduce their production. Local application of local anaesthetics also reduces transduction. Transmission Signals from these nociceptors travel along myelinated Ao and unmyelinated C fibres. Their axons synapse in the dorsal horn of the spinal cord, where the neurons of laminae (of Rexed) I, II and V are most involved in the perception of pain. These fibres can be blocked at the nerve plexus level using local anaesthetics. Modulation Large fibre inputs from other sensory modalities and descending pathways can modulate activity in the dorsal horn. The extent of this modulation results in differing levels of pain experienced by patients. This can be further modified using

Opioids Alpha-2 agonists

local anaesthetics (epidural anaesthesia), opioids and alpha-'. agonists. Perception The signals then travel along the spinothalamic tract of th1 spinal cord to the thalamus and the cortex which ultimate!� cause activity in the appropriate portion of the sensory corteJ and the limbic system. The response of the cerebral cortica cells and the limbic cells can be modified using opioids anc alpha-2 agonists. Often, a combination of all these techniques is used tc achieve good pain relief. Relief of pain helps the patient hea faster and return to normal activities early. The complicatiorn of inadequate pain relief such as respiratory infections hypertension, myocardial infarction and increased catabolism are avoided or minimised. Assessment of pain Pain is more difficult to measure as compared to othe1 parameters such as temperature or heart rate. The measuremen1 of pain is mainly dependent on the patient's perception of i1 and may be done in any of the following ways: 1. Visual Analogue Scale (VAS): The pain is assessed along a l 00 mm scale where O corresponds to no pain at all and l 00 to the worst imaginable pain. The patient scores his pain at any point he finds appropriate. The pain relief is assessed thereafter from this point. The VAS is the most popular tool used to assess pain and its relief.

No pain

Hurts little bit

Hurts Hurts little more even more

Hurts a lot

Hurts worst

Fig. 48.30: Wong-Baker faces pain rating scale for children Descending modulation Dorsal horn

Local anaesthetics Opioids Alpha-2 agonists --- Dorsal root ganglion

Local anaesthetics

2. Category rating scale: The different ratings are no pain, mild, moderate or severe. 3. Wong-Baker Faces pain rating scale: A scale with different faces, a happy face at one end and a face in severe pain and crying at the other with four other faces in between may be used for children (Fig. 48.30). 4. Biological measures such as tachycardia, hypertension, tachypnoea or inadequate breathing are relatively inaccurate but useful estimates of pain. CHRONIC PAIN

Peripheral receptors

------t-=====_.

Local anaesthetics Anti-inflammatory drugs

Fig. 48.29: Pain pathways and their modulation using drugs

The initial response to a noxious stimulus is brief and correlates with the sharp, well-localised initial pain. The second phase of the response is more prolonged and correlates with the dull, diffuse pain experienced after the initial injury. This second phase is associated with a growing region of hypersensitivity

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around the point where the noxious stimulus was initially applied and is said to result in chronic pain. The response to noxious stimuli can be modulated by their repeated application. Peripheral nociceptors become more responsive with the repeated application of noxious stimuli. Their sensitivity can be further enhanced by many tissue factors and inflammatory mediators released in the course of tissue injury. The process through which the neurons of the dorsal horn of the spinal cord become sensitised by prior noxious stimuli is often referred to as "windup" or "central sensitisation." Much less is known about pain-induced sensitisation of the supraspinal components of the CNS. This phenomenon leads to chronic pain where the patient experiences pain, often, excruciating, and long after the stimulus that initially caused the pain is removed. The relief of chronic pain is more difficult. Various techniques used to treat chronic pain are as follows: Nonpharmacological techniques • Transcutaneous electrical nerve stimulation (TENS) • Acupuncture Pharmacological techniques • Paracetamol • Nonsteroidal analgesics Sympathetic blockade • Sympathetic ganglion blocks-stellate ganglion block, coeliac plexus block, etc. • Epidural local anaesthetics • Epidural neurolytic such as phenol, alcohol injections. COMPLICATIONS IN ANAESTHESIA The practice of anaesthesia has become very safe due to better preoperative evaluation and preparation, careful choice of patients, better monitoring, availability of safer drugs and safer anaesthetic techniques. The incidence of complications has come down drastically. However, complications can still occur. The perioperative (pre-, intra-, and postoperative periods) complications can be classified as follows: Respiratory • Airway obstruction • Bronchospasm • Respiratory failure. Cardiovascular • Hypertension • Hypotension • Arrhythmias • Shock • Cardiac arrest.

Central nervous system • Postoperative drowsiness • Postoperative nausea and vomiting • Neurologic complications of regional blockade. Renal and hepatic failure RESPIRATORY COMPLICATIONS Airway obstruction Airway obstruction may occur during the induction of general anaesthesia. When a person becomes unconscious, the tongue and the epiglottis fall back and can obstruct the airway. The patency of the airway is usually maintained fairly easily by the anaesthesiologist by using chin lift or jaw thrust manoeuvres. A definitive airway such as an endotracheal tube may then be inserted into the trachea. Occasionally, the chin lift or jaw thrust manoeuvres are inadequate to maintain a patent airway as in patients with abnormal airways. Insertion of an endotracheal tube may also prove to be difficult in certain individuals. An oral or nasopharyngeal airway may be inserted to overcome this obstruction and maintenance of the airway for a short duration. If there is difficulty in inserting the endotracheal tube due to supraglottic causes and oral or nasopharyngeal airways are not sufficient to relieve the obstruction, insertion of a laryngeal mask airway or a Combitube® may be attempted. If the problem is at the glottis or subglottis and the airway is obstructed, an emergency cricothyrotomy or a tracheostomy may be required. Perioperative airway obstruction may also be due to any of the following causes • Trauma-maxillofacial, head injury • Foreign body aspiration • Laryngospasm • Infection-Ludwig's angina, retropharyngeal abscess • Oedema-laryngeal/pharyngeal oedema • Neurological-recurrent laryngeal nerve injury • Endocrine-thyroid enlargement • Tumour-malignancy of the airway (tongue, cheek, larynx or the pharynx) A thorough assessment of the airway must be done preoperatively and a management plan A formulated. Plan B and Plan C also should be considered in the eventuality of failure of Plan A. Generally loss of life occurs not because of inability to intubate but due to an inability to oxygenate and ventilate the patient. This situation, also called, 'cannot intubate-cannot ventilate' (CVCI) is one of the most dreaded situations faced by an anaesthesiologist. Bronchospasm Bronchospasm may occur in a patient under anaesthesia. The possible causes are as follows:

Principles of Anaesthesiology

Irritable airways as in a known asthmatic, chronic obstructive airways disease, i.e. exacerbation of pre-existing bronchospastic disease. • Endobronchial intubation and cranial stimulation • As part of allergic reaction to anaesthetic drugs, antibiotics • Aspiration of regurgitated gastric contents • Pneumothorax • Upper airway obstruction and laryngospasm can reflexly stimulate bronchospasm. Treatment involves treatment of the precipitating cause and bronchodilators. Respiratory failure A patient is said to be in respiratory failure if he is unable to maintain adequate oxygenation and ventilation, i.e. arterial blood gas tension of 0 2 less than 60 mmHg (when patient is breathing 60% 02) and CO 2 more than 50 mmHg. This could be acute or acute exacerbation of chronic respiratory failure. Causes Central • Head injury • Depressant drugs-anaesthetics, opioids • Hypoxic encephalopathy • Metabolic causes such as hyponatraemia, hypoglycaemia, hypokalaemia, hyperglycaemia. Peripheral • Lung parenchymal disorders such as pneumonia, atelectasis, aspiration, pneumothorax, pulmonary embolism • Inadequate respiratory excursion due to pain or thoracic cage abnormalities such as kyphoscoliosis. • Weakness of muscles, e.g. prolonged effect of muscle relaxants, myasthenia gravis Treatment • Treat the cause • Intermittent positive pressure ventilation and ventilatory support till the patient improves. CARDIOVASCULAR COMPLICATIONS Hypertension, hypotension and arrhythmias occur perioperatively due to various reasons such as inadequate preoperative treatment, surgical stress, inadequate anaesthesia, metabolic or endocrine causes or even drug interactions. Brief periods of haemodynamic instability, although common are well-tolerated by healthy individuals. Continuous and appropriate monitoring and prompt treatment should avoid long-term complications. Hypotension may be due to decreased preload, reduced contractility or increased afterload to the left ventricle. If not identified or treated in time, it may progress to shock and cardiac arrest. Hypovolaemic shock is the commonest type of

shock encountered during surgery. However, cardiogenic shoe due to perioperative myocardial infarction, anaphylactic shoe due to allergic reaction to anaesthetics or neurogenic shoe due to vasovagal attack, high spinals may also occur i susceptible individuals. Perioperative myocardial infarction (Ml) is a comp! cation that occurs in susceptible individuals. It may be cause due to extreme and sustained variations in haemodynamic such as hypotension, hypertension or tachycardia. It may als be caused due to thrombosis as the patient is in hypercoagulable state due to stress of surgery. The highe! incidence of perioperative MI is seen not on the day of surger (when the patient is under the vigilant care of th anaesthesiologist) but on the second or the third day when th attention given to him is less in terms of pain relief o haemodynamic changes. CENTRAL NERVOUS SYSTEM COMPLICATIONS Awareness Rarely, a patient under GA might recall events that occurrec during the procedure. This is termed awareness durini anaesthesia and is one of the most dreaded complications b: both anaesthesiologist and the patient. This is more likely t< occur if the patient's haemodynamics are very unstable (a: in postpartum haemorrhage, trauma) and the anaesthesiologis fears further cardiac depression may occur with the use o inhalation agents. The use of opioids and nitrous oxide ma) provide analgesia but not the amnesia and anaesthesia required With increased awareness of this complication amon� anaesthesiologists coupled with the use of benzodiazepinei and modem anaesthetic agents which are more cardiostable the incidence of this is reduced. A new monitor called BI� index monitor provides some information of the consciom state of the patient but is not widely available yet. Postoperative drowsiness A patient may be slow to awaken after anaesthesia due to persistent effect of the anaesthetic agents or opioids administered during the anaesthesia. However, it may be due to metabolic causes such as hypoxia, hypothermia, hypo- or hypematraemia, hypo- or hyperglycaemia. If all these causes are ruled out, a neurological consultation is obtained to rule out any space-occupying lesions in the central nervous system, stroke or hypoxic encephalopathy. Postoperative nausea and vomiting (PONV) PONY is a frequent complication of anaesthesia. It is common in women, after laparoscopic surgeries, squint surgeries and is associated with the use of nitrous oxide and opioids or even early oral intake postoperatively. It may be treated with IV metoclopramide ( 10 mg), ondansetron ( 4-8 mg) or dexamethasone (4-8 mg) in an average adult.

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Nerve injuries Nerve injuries may arise as a complication of regional blockade. Complications such as adhesive arachnoiditis, cauda equina syndrome or paraplegia have been reported after spinal and epidural anaesthesia but are extremely rare. The use of tourniquet, if prolonged or if very high pressures are used, can also cause nerve injuries. Peripheral nerve injuries may occur perioperatively due to improper positioning under regional or general anaesthesia. The common peroneal nerve and the sciatic nerve can be injured during lithotomy position. The ulnar and the radial nerves may be affected in the arm or at the elbow due to inadequate attention to positioning. Brachia! plexus stretch injury can occur if the arms are allowed to be abducted more than ninety degrees. Injury to optic nerves or the retina may occur in prone position due to compression of the eyeball. Corneal injury may occur due to exposure in an unconscious patient. RENAL FAILURE Renal failure, usually prerenal is associated with large fluid shifts or major haemodynamic instability. Direct injury to the kidneys (acute tubular necrosis) may occur if adequate attention is not given to prerenal failure. Methoxyflurane, an inhalation anaesthetic agent can cause renal failure but is no longer in clinical use. Renal failure can also occur as part of hepatorenal syndrome or after a mismatched blood transfusion. HEPATIC FAILURE A patient with compromised hepatic function may proceed to hepatic failure perioperatively, e.g. cirrhosis of liver, obstructive jaundice. Hepatic failure may also occur due to infective complications such as hepatitis or sepsis. Massive hepatic necrosis has been reported after repeated use of halothane. The incidence of this is very rare and must be a diagnosis of exclusion. CARDIOPULMONARY RESUSCITATION (CPR) Cardiac arrest is an inevitable and natural event in every one's life. However, this event may have been precipitated prematurely due to some underlying but treatable cause. If prompt intervention is made and the patient's heart and respiration are supported at this stage, precious time may be gained to treat this precipitating cause. Many victims of cardiac arrest may lead normal lives once this calamity is tided over. This is the basis of cardiopulmonary resuscitation. CPR when given without the help of any equipment is called Basic CPR (also called basic life support-BLS). When equipment and drugs are used in CPR, it is called Advanced CPR (advanced cardiac life support-ACLS). CPR must be given promptly and in the correct manner to be effective. While

basic CPR alone may not be sufficient in resuscitating a victim of cardiac arrest, it is this intial response that keeps the patient alive till advance help can be provided. Success of resuscitation as well as health-related quality of life depends largely on the promptness and quality of basic CPR. Hence, knowledge of CPR is mandatory for all medical and paramedical personnel. Points to note • CPR is only a symptomatic therapy.Attention must be given to the precipitating cause and must also be treated. • The best of CPR can deliver only one-third of the normal cardiac output. Hence, it is important to aim for return of spontaneous circulation as early as possible. • 'Time is brain': Effectiveness of advanced cardiac life supp011 as well as intact survival largely depends on prompt, early and good quality basic CPR. Respiratory arrest The patient stops breathing due to a primary respiratory cause. The heart continues to beat till all the oxygen in the lungs is removed. The heart will stop once hypoxia occurs. This may take 1-2 minutes to occur. If the respiration is assisted promptly, it is possible to avert cardiac arrest. However, this is possible only in a witnessed respiratory arrest. Cardiac arrest The heart stops beating and there is no circulation. Oxygen delivery to the tissues stops. Within 15 seconds of a cardiac arrest, a person loses consciousness. The brain stops functioning within 3 minutes. This is called survival time. If resuscitation is not done in another five minutes, brain death occurs. This is called revival time. Many factors influence these survival and revival times. The important 'take home' message is that prompt and effective treatment is necessary for successful resuscitation. BASIC CPR The sequence of steps of basic CPR has been changed in the 2010 guidelines of American Heart Association (AHA). Approach to CPR is currently done in the following order: C, A and B. C for cardiac compressions, A for airway and B for breathing. There are six steps in basic CPR. STEP 1: ASSESS UNCONSCIOUSNESS. "SHAKE GENTLV AND SHOUT LOUDLV" In cardiac arrest, cerebral circulation comes to a standstill and the person becomes unconscious within 15-20 seconds. Thus, a person in cardiac arrest will not be conscious. Shortly thereafter, the victim of cardiac arrest will also stop breathing. On shaking gently and calling out loudly, if there is no response, assume unconsciousness. Turn the patient in to a supine

Principles of Anaesthesiology

position. Take care to turn the head, neck and body together in a trauma victim who may possibly have a cervical spine injury. Observe for any sign of breathing such as chest movement. Jf there is no breathing, it confirms that the patient needs assistance. STEP 2: CALL FOR HELP AND AED

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shoulders directly above the sternum. The rescuer can avo1 fatigue during compressions by allowing his or her body weigl to be transmitted to the chest to create the required depth < compression rather than use muscle power. Thus, th movement occurs at tbe rescuer's hips and not at the elbow Only one hand may be used for compression in children lei than 8 years of age.

The chances of resuscitation are lower with basic CPR and it is important to get help (in the form of ambulance services for advanced CPR) as early as possible. It is also difficult for a single rescuer to administer CPR for long periods. The rescuer may take up to a minute to call for help. Since the majority of adult, nontraumatic cardiac arrests are due to ventricular fibrillation or pulseless ventricular tachycardia (shockable rhythms), a defibrillator, either manual or automated, must be obtained as quickly as possible. STEP 3: FEEL CAROTID PULSE Check for pulse or other signs of circulation such as coughing, movement in response to the rescue breaths. When circulation is inadequate, tbe body tries to conserve circulation to the vital organs as long as possible. Thus, the carotid artery pulsations are the last to disappear when cardiac output gradually reduces. Hence, it is important to feel the carotid pulse to identify cardiac arrest (Fig 48.31). Infants and neonates have short necks and it is difficult to feel their carotid arteries. ln them, the brachia! artery may be used to identify cardiac arrest. If no pulse is felt, the patient is in cardiac arrest.

Fig. 48.31: Feel carotid pulse

STEP 4: GIVE CHEST COMPRESSIONS Site of compression Compressions should be given with the heel of the hand placed on the lower half of the sternum. Care should be taken not to compress on either side of the sternum as it may cause rib fractures. Technique (Fig. 48.32) Place the heel of one hand over the lower half of the sternum in the midline. Clasp this hand with the heel of the other hand, keep the elbows straight and move forward so as to place the

Fig. 48.32: Provide chest compressions

Rate of compression: Provide 30 compressions at a rate of a least 100/minute. Depth of compression: 2 inches (5 cm) in an adult. Chest compressions are the most important part of basic CPR. Hence they should be initiated as soon as cardiac arres1 is recognised. 'Push hard, push fast' is what the AHA recommends. There should be minimal interruption in ches1 compressions for any other interventions such as defibrillation, airway management or even calling for help. The patient should be lying on a relatively hard surface. If not a hard board may be placed underneath the patients chest so that the compressions become effective.

STEP 5: OPEN AIRWAY When a person loses consciousness, the muscles of the tongue, epiglottis and pharynx relax. The tongue and the epiglottis fall back and obstruct the glottis. Unless the airway is opened, it is not possible to check accurately whether respiration is present or not and if present, whether it is adequate or inadequate. The airway is opened using a "head-tilt and chin lift method" (Fig 48.33). The palm of one hand is placed on the forehead of the patient and the head tilted posteriorly to extend the head as much as possible. The chin of the patient is lifted up manually with the thumb and fingers of the other hand so that the lower incisors of the victim override the upper incisors. Since the tongue and

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Fig. 48.33: Opening airway with 'head tilt and chin lift'

the epiglottis are attached to the mandible, chin lift also lifts the tongue and the epiglottis. STEP 6: GIVE TWO BREATHS The rescuer opens his/her mouth wide, makes a mouth to mouth seal and delivers a large breath into the victim so as to expand the chest adequately. He/she takes a breath in from the atmosphere and gives another breath to the victim. Take two seconds to deliver each breath. Compression-ventilation ratio Provide cycles of 30 compressions and 2 breaths (5 cycles should be complete in two minutes). Continue cycles of 30 compressions and two breaths until help arrives or if the patient shows some sign of life such as breathing or movement. AED If the second rescuer arrives (with an AED), he proceeds to switch on the AED, attach the defibrillator pads to the bare chest of the patient, placing one pad in the right infraclavicular region and the other on the precordium. He allows the AED to analyse the cardiac activity for a shockable rhythm. During analysis, the rescuers should not touch the patient. If the AED confirms the presence of a shockable rhythm, it automatically charges the pads and requests the user to press the shock button so that the shock can be delivered to the patient. The rescuer ensures that nobody is touching the patient or the bed, including himself, announces loudly and delivers the shock. The second rescuer takes over chest compressions whereas the first rescuer (who may be tired by then) would take care of airway and breathing. The two rescuers give chest compressions and ventilations at a ratio of 30 : 2 (Fig 48.34). Rescuer fatigue can affect the quality of CPR. After five cycles of CPR (2 min), the rescuer must switch roles to provide compression and ventilation. The heart rhythm is analyse every two min to see whether there is a return of spontaneous cardiac activity. If the AED declares that there is no shockable rhythm, carotid pulse should be felt. If a palpable pulse is present, stop chest compressions and continue rescue breathing as necessary

Fig. 48.34: Two rescuer CPR

(1 breath every 5 seconds). If breathing also returns and is adequate, the patient may be allowed to breathe spontaneously. The victim may be turned to a lateral position (recovery position) and monitored. The victim must then be shifted to a hospital for further evaluation and care. BLS is usually continued till help arrives and advanced life support measures can be attempted. However, if no help is at hand, and if the rescuer is unable to continue due to fatigue or if the victim is obviously dead, CPR may be stopped. Advanced CPR (advanced cardiac life support ACLS) Advanced CPR involves using airway adjuncts, other equipment and drugs for resuscitation. A. Airway Bag-mask ventilation (Fig 48.35), if effective in achieving chest expansion is adequate in the initial phase of CPR. Airway is best maintained with endotracheal intubation. However, chest compressions should not be interrupted to facilitate endotracheal intubation. If it can be achieved without interrupting chest compressions, then one may proceed to secure the airway with an endotracheal tube. The route of choice for intubation is through the mouth because it is faster and less traumatic. Endotracheal intubation has the following advantages: I. The trachea is protected from aspiration of regurgitated gastric contents. 2. The tidal volume can be assured 3. There is no distention of the stomach. In case endotracheal intubation is difficult either due to difficult airway or inexperience in the technique, oxygenation and ventilation can be achieved by the use of an oropharyngeal

Principles of Anaesthesiology

VENTRICULAR FIBRILLATIONNENTRICULAR TACHYCARDIA Most of the adult nontraumatic cardiac arrests are due t ventricular fibrillation (VF)/ventricular tachycardia (VT). l untreated, VF/VT will degenerate into asystole. The onl. treatment of ventricular fibrillation is electrical defibrillatior The success rate of defibrillation reduces by 10% every passin. minute. Hence, defibrillator must be obtained as early a possible (Fig 48.36).

Fig. 48.35: Bag-mask ventilation

or nasopharyngeal airway along with a self-inflating bag and face mask. Usually this is adequate but occasionally a laryngeal mask airway or a Combitube® along with a self-inflating bag may also be used. B. Breathing After the airway is secured, ventilation must be provided at a rate of 8-10 breaths per minute using a self-inflating bag attached to the endotracheal tube. Oxygen must be attached to the bag to improve oxygenation. These breaths need not be interposed between cycles of30 compressions. Once the airway is secured with an endotracheal tube, chest compressions are given uninterrupted at a rate of at least 100/min while breaths are given every 6-8 seconds. It is important not to hyperventilate during CPR as it can produce constriction of cerebral arteries, reduce cerebral blood flow and adversely affect outcome. A capnograph may be attached to the endotracheal tube to monitor end-tidal carbon dioxide. A capnograph has three uses in CPR: 1) To confirm the placement of endotracheal tube 2) To monitor the effectiveness of CPR. If the end-tidal carbon dioxide concentration is > 10 mm Hg, the cardiac compressions can be deemed effective and3) A sudden increase in end-tidal carbon dioxide to normal or near normal levels heralds a return of spontaneous circulation (ROSC). Thus, when an endotracheal tube is in place, efforts must be made to obtain and use a capnograph, whenever possible. C. Circulation Continue cardiac compressions as in BLS. Obtain peripheral intravenous access quickly. D. Drugs/Defibrillation Cardiac arrest rhythms are one of the following three types: Ventricular fibrillation/Ventricular tachycardia (VF /VT), pulseless electrical activity (PEA) and asystole. A monitor is obtained as early as possible in CPR to diagnose these rhythms.

Fig. 48.36: A manual defibrillator

If the rhythm shows ventricular fibrillation (VF) 01 ventricular tachycardia (VT) (Figs 48.37 and 48.38), the following algorithm must be followed: • Provide Basic CPR. Once VF/VT is confirmed, interrupt CPR briefly to deliver a single shock of360 J (monophasic) or 200 J (biphasic). Resume CPR immediately and continue for another two minutes (5 cycles of compressions and ventilations) before reanalyzing the rhythm.

Fig. 48.37: ECG rhythm showing ventricular fibrillation. Note the saw tooth shaped waves that are irregular and chaotic in pattern

Fig. 48.38: ECG rhythm showing ventricular tachycardia which is more regular and shows wide ORS complex with inverted T waves

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Manipal Manual of Surgery

• If VF/VT persists, deliver another single shock of 360 J (monophasic) or 200 J (biphasic) and resume CPR. Administer adrenaline 1 mg or vasopressin 40 IU (one dose only). Reassess after two minutes. • If VF/VT persists, deliver another single shock of 360 J (monophasic) or 200 J (biphasic) and resume CPR. An antiarrhythmic drug such as Amiodarone 300 mg may be given IV. • Thus, analysis of the rhythm is repeated every two min and if a shockable rhythm is present, shock is given. Epinephrine 1 mg is repeated every 3-5 min. A second dose of arniodarone 150 mg may be considered for persistent VF/ VT. Always remember to look for h·eatable causes (Key Box 48.6) of arrest.

• Check whether all leads are connected correctly. Improper connections may manifest as asystole. • Change the Lead and check whether it is asystole in Lead I, II and III. Occasionally, fine VF may be seen as asystole in one lead but on changing the lead, VF may be seen. lf VF is seen, defibrillation must be attempted. There is no role for defibrillation in asystole. In fact, it could make it worse. • Commence basic CPR immediately. • Administer adrenaline, 1 mg IV, every 3-5 minutes as necessary till return of spontaneous circulation is achieved. Look for a treatable cause and treat. • lf there is no response to resuscitation in spite of all the above efforts even after a reasonable period of time (30-60 min) consider abandoning resuscitation efforts.

PULSELESS ELECTRICAL ACTIVITY (PEA)

DRUGS USED IN CPR

lf the monitor showed no VF/VT, but a discernible rhythm, 1. Adrenaline sinus or nonsinus with the patient manifesting no pulse (no This is the drug of choice in cardiac arrest. It is given in a dose carotid pulsations), it is termed pulseless electrical activity of 1 mg, diluted to 10 cc (1: I 0,000) intravenously every 3-5 (PEA). This means that electrical activity is present while minutes: This should be followed by at least 20 cc of 'chaser' mechanical activity is absent. solution (0.9% saline or Ringer lactate) and limb elevation for While PEA may be caused by massive myocardial infarction 15-20 s. A peripheral intravenous access is adequate but if a where the myocardial necrosis is so severe that ventricular central line is in situ, the drug may be injected in the central contractions are absent (electromechanical dissociation - line. If intravenous access is not available, an interosseous EMD), it may also be caused by the following factors which access may be secured (anteromedial aspect of tibia or lower may result in ineffective contractions while electrical activity end of femur may be used). Alternately, adrenaline may be is still present (pseudo-EMO). In pseudo-EMD, the causes injected through the endotracheal tube but higher doses need are reversible. lf the cause is identified and treated promptly, to be used (2-5 mg diluted to 10 cc). the chances of successful resuscitation are high. lntracardiac administration of adrenaline is not advisable l . Commence Basic CPR for the following reasons: 2. This must be followed by adrenaline, I mg lV, every 3-5 a. Possible injury to coronary arteries minutes as necessary till return of spontaneous circulation b. Possible injury to the pleura is achieved. c. Accidental injection into the myocardium can precipitate 3. Look for a treatable cause (Key Box 48.6) and treat promptly. resistant venh·icular fibrillation d. Since the right ventricle is anterior, intracardiac injection is usually into this chamber from which it has to traverse the pulmonary circulation to reach the coronary arteries. This may well be achieved by an intravenous injection, running it in a drip and avoiding the complications of REVERSIBLE CAUSES OF CARDIAC ARREST intracardiac injection. Intracardiac injection is advisable only 1. Hypoxia 1. Tension pneumothorax when done under vision as during open heart surgery or 2. Hydrogen ions (acidosis) 2. Tamponade (Cardiac) thoracotomy. 3. Thrombosis (Pulmonary, 3. Hypovolaemia

MUM..........,� Coronary) 4. Toxins 4. Hyper/hypokalaemia 5. Trauma 5. Hypo/hyperthermia Remember 5 Hs, 5 Ts

ASYSTOLE lf the monitor shows a flat line, it is asystole. Whenever asystole is seen on a monitor the following things should be done (flat line protocol). Confirm that it is asystole and then commence therapy.

2. Vasopressin This is a potent vasoconstrictor that can be given as a single dose of 40 lU and as an alternative to the fust or the second dose of adrenaline in a victim of cardiac arrest. The vasoconstriction produced raises the diastolic pressure and hence, the coronary perfusion pressure during CPR. 3. Amiodarone If VF/pulseless VT persists in spite of the adrenaline/ vasopressin followed by a DC shock of 360 J, amiodarone

Principles of Anaesthesiology

may be given in a dose of 300 mg intravenously followed by 150 mg after five min, as necessary. Other drugs that may be used are: Lignocaine: l-2 mg/kg intravenously or magnesium sulphate: l-2 g intravenously. Not more than two anti­ arrhythmic drugs may be used to treat an arrhythmia as all antiarrhythmics are also proarrhythmics and may also produce myocardial depression. 4. Sodium bicarbonate An alkalinising agent is used in the following situations: • When there is documented metabolic acidosis • The cardiac arrest is due to hyperkalaemia or • The arrest period is prolonged beyond 10 minutes. The dose is 1-2 mmol/kg intravenously. 5. Calcium chloride/gluconate Calcium is given only in the following situations: • lf the cardiac arrest is due to hyperkalaemia • There is documented hypocalcaemia or • The patient has been on calcium channel blockers. The dose is IO ml of 10% calcium chloride or gluconate. Thus, both calcium and sodium bicarbonate are indicated when there is hyperkalaemic cardiac arrest. Their routine use in cardiac arrest is not indicated as they can cause more harm.

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PRECARDIAC ARREST RHYTHMS Often cardiac arrest follows a period characterized by sorr abnormal rhythms such as symptomatic bradycardia (heart ra1 < 50/min or symptomatic tachycardia (heart rate > 150/min The symptoms are usually chest pain, dizziness and syncop, palpitations or generally feeling weak. ff these are identifie and treated promptly, cardiac arrest may be averted. Oxyge must be administered. Attach a monitor and secure a intravenous access. Symptomatic bradycardia Symptomatic bradycardia may be due to sinus bradycardia c heart blocks. Atropine (boluses of 0.5 mg IV, repeated to maximum of 3 mg) is the drug of choice followed by a infusion of adrenaline (2-10 mg/min) or dopamine (2-10 mf kg/min). If the bradycardia is unresponsive to atropirn dopamine or adrenaline, a transcutaneous or transvenou pacemaker may be required to pace the heart. Always look fa and treat the cause. Symptomatic tachycardia

POSTRESUSCITATION CARE

Cardiac arrest may also be preceded by symptomati, tachycardia (hea1t rate > 150/min). The symptoms are due tc inadequate circulation or the tachycardia itself such as ches pain, dizziness and syncope, palpitations or generally feelini weak.

lf return of spontaneous circulation is achieved, it is important to ensure it is sustained. Cardiac compressions are stopped when the pulses begin to be felt without the compressions. Intravenous fluids and/or an infusion of an inotrope such as adrenaline or dopamine may be required to sustain normal blood presasure. A blood sample may be taken to quickly analyse the cause of cardiac arrest such as acidosis, disturbances in potassium levels, poor oxygenation, and for cardiac enzymes. A 12-lead electrocardiogram may be obtained to rule out ST-elevation Ml. If present, the patient will require cardiology intervention such as coronary revascularisation. The patient will need further care in the intensive care unit where respiratory and renal support can be provided. It is important to preserve cerebral function by maintaining normal blood pressures, normal blood gases and pH, and normal cerebral perfusion pressures. If the patient does not regain consciousness after CPR and ROSC, consider induced hypothermia by infusing one litre of cold saline (4 ° C) with a target body temperature of 32 to 34 °C. This is done in an attempt to reduce cerebral metabolism so as to better preserve brain function while the patient recovers from the after-effects of cardiac arrest and CPR. Thus, cardiopulmonary resuscitation should be aimed at recovery of all functions of the body including the brain and is more aptly called cardiopulmonary cerebral resuscitation (CPCR).

Symptomatic tachycardia may be due to sinus tachycardia This may be due to causes such as pain, fever, sepsis, fear hypovolaemia, etc. Identify and treat the cause. • If the patient is having symptomatic tachycardia, which i: not sinus in origin, measure blood pressure. If it is lo� (