SRB's Manual of Surgery [6 ed.] 9789352709076

Citation preview

6

th Edition

SRB's - - - - - - - - - -

Man uaI of

urgery Sri ram Bhat M MS (General Surgery) Professor and Head Department of Surgery Kastu rba Medical College Mangalore Mangaluru, Karnataka, India Honorary Surgeon Government Wenlock District Hospital Mangaluru, Dakshina Kannada, Karnataka, India e-ma ii: [email protected] Forewords

M Venkatraya Prabhu Thangam Verghese Joshua

JAYPEE BROTHERS MEDICAL PUBLISHERS The Health Sciences Publisher New Delhi I London I Panama

Contents 1. General Surgery

1

A. Wounds and Wound Healing 1 Wounds 1; Classification of Wounds 1; Wound Healing 6; Compartment Syndrome 10; Crush Injury 11; Crush Syndrome 11; Degloving Injuries 12; Scar 12; Keloid 12; Hypertrophic Scar 13; Problems with Wound Healing 14;

B. Ulcer 15 Ulcer 15; Granulation Tissue 18; Investigations for an Ulcer 19; Management of an Ulcer 20; Traumatic Ulcer 22; Trophic Ulcer 22; Ulcer due to Chilblains 23; Ulcer due to Frostbite 23; Martorell's Ulcer 23; Arterial/lschaemic Ulcer 23; Bairnsdale Ulcer 24; Carcinomatous Ulcer 24; Rodent Ulcer 24; Melanotic Ulcer 24; Diabetic Ulcer 25; Meleney's Ulcer 26; Lupus Vulgaris 26; Tuberculous Ulcer 21; Bazin's Disease 21; Tropical Ulcer 21; Venous Ulcer 27; Syphilitic Ulcer 28; Soft Chancre/Soft Sore/Ducrey's Ulcer/Chancroid/Bubo 28; Climatic Bubo/Tropical Bubo 28

C. Sinus and Fistula

30

Sinus 30; Fistula 30; Median Mental Sinus 32; Sequestrum 32; Preauricular Sinus 33

0 . Infectious Diseases

34

Surgical Infection 34; Cellulitis 34; Erysipelas 37; Lymphangitis 37; Abscess 38; Metastatic and Pyaemic Abscess 42; Bacteraemia 43; Septicaemia 43; Pyaemia 43; Boil (Furuncle) 43; Hidradenitis Suppurativa 44; Carbuncle 45; Pott's Puffy Tumour 45; Pyogenic Granuloma 46; Impetigo 46; Erythrasma 46; Scrum Pox 46; Tetanus 47; Gas Gangrene 50; Tuberculosis 52; Leprosy 53; Syphilis (Great Pox) 54; Actinomycosis 54; Madura Foot 55; Rabies 56; Anthrax 59; Nosocomial and Opportunistic Infections 59; Necrotising Fasciitis 60; Acute Pyomyositis 61 ; Surgical Site Infection 62; HIV Infection and AIDS 65

E. Swellings 68 Lipoma 68; Cysts 71 ; Dermoids 72; Sebaceous Cyst 75; Neuroma 77; Fibroma 78; Neurofibroma 78; Neurilemmoma (Schwannoma) 80; Ganglion 80; Papilloma 81 ; Warts 81 ; Glomus Tumour 82; Bursae 82; Semimembranosus Bursa 84; Morrant Baker's Cyst 85; Lymphangioma 85; Lymph Cyst (Lymphatic Cyst) 86; Calcinosis Cutis 86; Chordoma 86; Epignathus 86

F. Electrolyte and Nutrition 87 Normal Physiology 87;

Water Loss 87; Water Excess 87;

Hyponat raemia 88;

Hypernatraemia 88;

Hypokalaemia 89; Hyperkalaemia 89; Hypermagnesaemia 89; Hypomagnesaemia 90; Acid-Base Balance 90; Metabolic Alkalosis 90; Respiratory Alkalosis 90; Metabolic Acidosis 91 ; Respiratory Acidosis 91 ; Anion Gap 91 ; Fluid Therapy 91 ; Nutrition 96; Gastrostomy 97; Jejunostomy 97; Total Parenteral Nutrition 98; Refeeding Syndrome 100; Obesity and Morbid Obesity 100

G. Shock 104 Shock 104; Stages of Shock 105; Effects of Shock 105; Clinical Features of Shock 108; Assessment, Investigations and Monitoring 108; Central Venous Pressure 109; Pulmonary Capillary Wedge Pressure 110; Systemic Inflammatory Response Syndrome 111 ; Multiple Organ Dysfunction Syndrome 111; Oxygen Therapy 111; Cardiac Arrest 111

xviii

H. Haemorrhage and Blood Transfusion 113 Haemorrhage 113; Blood Transfusion 116; Massive Blood Transfusion 118; Artificial Blood 119; Tourniquets 119; Disseminated lntravascular Coagulation 120; Mechanism of Blood Coagulation 120

I. Burns 122 Burns 122; Management of Burns 126; Eschar 128; Contracture in Burn Wound 129; Electrical Burns 131 ; Inhalation Injury 131 ; Chemical Burns 132

J. Trauma 133 Triage 133; Concepts in Trauma Management 135; Spinal Injury 136; Neck Injuries 137; Bullet Injuries 137; Blast Injuries 137; Penetrating Injuries 138; Abdominal Trauma 138; Blunt Trauma of Abdomen 141; Duodenallnjury 143; Small Bowel Injury 143; Colonic Injury 143; Abdominal Compartment Syndrome 144; Seat-belt Injuries 145

K. Hand and Foot 146 Hand 146; Hand Infections 147; Acute Paronychia 149; Chronic Paronychia 150; Apical Subungual Infection 150; Terminal Pulp Space Infection (Felon) 150; Infection of Web Spaces 151 ; Deep Palmar Space Infection 151; Space of Parona Infection 153; Acute Suppurative Tenosynovitis 153; Compound Palmar Ganglion 154; Hand Injuries 154; Dupuytren's Contracture 156; Volkmann's lschaemic Contracture 157; Syndactyly 157; Foot 158; Callosity 158; Corn 158; Plantar Fasciitis (Policeman's Heel) 159; Ingrowing Toe Nail (Onychocryptosis) 159; Athlete's Foot 160; Hallux Valgus 160

L. Arterial Diseases 161 Surgical Anatomy of Thoracic Outlet 161 ; Arteries of Upper Limb 161 ; Arteries of Lower Limb 161 ; Arterial Diseases 162; Intermittent Claudication 162; Rest Pain 163; Limb lschaemia 163; Different Levels of Arterial Obstruction 165; Other Features of Poor Circulation 165; Investigations for Arterial Diseases 166; Diseases of the Arteries 169; Atherosclerosis 169; Thromboangiitis Obliterans 172; Takayasu's Pulseless Arthritis 175; Raynaud's Phenomenon 176; Temporal Arteritis 177; Treatment of Arterial Diseases 177; Subclavian Steal Syndrome 183; Acute Arterial Occlusion 183; Traumatic Acute Arterial Occlusion 184; Embolism 184; Re perfusion Injury 186; Saddle Embolus 186; Embolectomy 187; Fat Embolism 187; Air/Gas Embolism 188; Therapeutic Embolisation 189; Caisson's Disease or Decompression Disease 189; Aneurysm 189; Mycotic Aneurysm 191 ; Abdominal Aneurysm 191 ; Abdominal Aortic Aneurysm 192; Peripheral Aneurysm 196; Carotid Artery Aneurysm 196; Dissecting Aneurysm 197; Erythromelalgia 198; Livedo Reticularis 198; Polyarteritis Nodosa 198; Scleroderma/ Systemic Sclerosis 198; Acrocyanosis 198; Gangrene 199; Diabetic Foot and Diabetic Gangrene 200; Frostbite 201; Ainhum 201 ; Endovascular Surgeries 201 ; Upper Limb lschaemia 202; Arterial Substitutes 204

M. Vascular Lesions

205

Vascular Anomalies 205; Haemangioma 205; Vascular Malformations 208; Cirsoid Aneurysm 209; Arteriovenous Fistula 209

N. Venous Diseases 214 Anatomy of Veins of Lower Limb 214; Physiology of Venous Blood Flow in Lower Limb 215; Deep Vein Thrombosis 216; Varicose Veins 219; Venous Ulcer 232; Compression Therapy for Varicose Veins 234; Thrombophlebitis 235; Klippel-Trenaunay Syndrome 235; Anticoagulants 235; Oral Anticoagulants 236; Pulmonary Embolism 237

0. Lymphatics

239

Surgical Anatomy 239; Lymphangiography 240; Isotope Lymphoscintigraphy 241 ; Acute Lymphangitis and Lymphadenitis 241 ; Lymphoedema 241 ; Lymphomas 248; Mantle Cell Lymphoma 254; Malt Lymphoma (Maltoma) 254; Burkitt's Lymphoma 255; Cutaneous T Cell Lymphoma 255; Chylous Ascites 256; Chylothorax 256; Chyluria 256; Sarcoidosis 257

P. Peripheral Nerves 258 Peripheral Nerve Injuries 258; Tinel's Sign 259; Brachia! Plexus Injuries 260; Causalgia 260; Median Nerve Injury 260; Carpal Tunnel Syndrome 261 ; Ulnar Nerve Injury 262; Claw Hand 263; Radial Nerve Injury 264;

xix

Common Peroneal Nerve Injury 264; Foot Drop 264; Medial Popliteal Nerve Injury 265; Axillary Nerve Injury 265; Long Thoracic Nerve Injury 265; Meralgia Paraesthetica 265

Q. Neoplasm

266

Definition 266; Dysplasia 267; Carcinoma In Situ 267; Aetiologic Factors 267; Spread of Malignant Tumours 268; Grading of Tumour 268; Staging of the Tumour 269; Paraneoplastic Syndromes 270; Investigations for Neoplasm 210: Management Strategy for Cancers 274

R. Skin Tumours

276

Anatomy 276; Clasification of Skin Tumours 277; Skin Adnexal Tumours 277; Dermatofibroma 278; Dermatofibrosarcoma Protuberans 279; Keratocanthoma 279; Rhinophyma 279; Seborrhoeic Keratosis 280; Squamous Cell Carcinoma 281; Marjolin's Ulcer 283; Basal Cell Carcinoma 284; Turban Tumour 286; Naevi 286; Melanoma 287

S. Sarcomas

296

Sarcoma 296; Liposarcoma 303; Fibrosarcoma 304; Malignant Fibrous Histiocytoma 305; Leiomyosarcoma 305; Rhabdomyosarcoma 305; Chondrosarcoma 305; Haemangiosarcoma 305; Synovial Sarcoma 306; Malignant Peripheral Nerve Sheath Tumour 306; Kaposi's Sarcoma 306

T. Amputations

307

Amputation 307; Complications of Amputations 314; Prosthesis 315

U. Reconstruction

316

Graft 316; Skin Grafts 316; Flaps 319; Abdominoplasty 325; Tendon 325; Tendon Repair 326; Tendon Transfer 326; Tendon Graft 326

V. Transplantation

327

Preoperative Evaluation 327; Organ Procurement 327; Graft Rejection 328; lmmunosuppressive Agents 329; Renal Transplantation 330; Liver Transplantation 331 ; Bone Marrow Transplantation 333; Pancreatic Transplantation 333; Small Bowel Transplantation 334; Dialysis 334; Cimino Fistula 334

W. Pain

335

Gate Control Theory 335

2. Faciomaxillary Diseases

338

Diseases of the Palate 338; Orthopantomogram 338; Cleft Lip and Cleft Palate 338; Maxillofacial Injuries 342; Primary Care in Maxillofacial Injuries 343; Fracture Middle Third Area 344; Zygomatic Complex Fracture 345; Fracture of the Mandible 346; Dislocation of the Mandible 348; Jaw Tumours 349; Epulis 350; Ameloblastoma 351 ; Dentigerous Cyst 352; Dental Cyst 352; Osteomyelitis of Jaw 353; Alveolar Abscess 353; Fibrous Dysplasia of Bone/Jaw 354; Cherubism 355

3. Oral Cavity

356

Ranula 356; Sublingual Dermoids 357; Stomatitis 357; Cancrum Oris 358; Syphilitic Lesions of Oral Cavity 358; Leukoplakia 359; Erythroplakia 360; Oral Submucosal Fibrosis 360; Premalignant Conditions of Oral Cavity 361 ; Oral and Upper Aerodigestive Cancers 361 ; Cheek 363; Carcinoma Cheek/Buccal Mucosa 363; Lip 374; Neoplasm of Lip 375; Carcinoma Lip 375; Tongue 378; Tongue Ulcers 379; Benign Tumours of Tongue 380; Tongue Fissure 380; Glossitis 380; Tongue Tie 381 ; Carcinoma Tongue 382; Carcinoma of Posterior One-third/Base of the Tongue 385; Nasopharyngeal Carcinoma 385; Maxillary Tumours 386; Malignant Tumours of Tonsil 388; Carcinoma Hard Palate 388; Laryngeal Tumours 389; Malignant Tumours of Larynx 389; Trismus 391

4. Salivary Glands Anatomy 392; Saliva 395; Sialography 395; Salivary Calculus and Sialadenitis 396; Parotid Abscess 398; Parotid Fistula 399; Recurrent Childhood Parotitis 399; Sjiigren's Syndrome 400; Sialosis 400; Sialectasis 400; Salivary Neoplasms 400: Pleomorphic Adenoma 401 ; Adenolymphoma 403; Oncocytoma 404; Basal Cell Adenoma 404; Mucoepidermoid Tumour 404; Adenoid Cystic Carcinoma 405; Acinic Cell Tumour 405;

392

D

Malignant Mixed Tumour 405; Adenocarcinoma of Salivary Glands 406; Squamous Cell Carcinoma of Salivary Glands 406;

Submandibular Salivary Gland Tumours 406;

Minor Salivary Gland Tumours 409; Parotid

Lymphoma 409; Parotidectomy 410; Frey's Syndrome 411 ; Facial Nerve Injury 412

... Cl)

en

:::I

en

0

5. Neck

414

Anatomy of Lymphatics of Head and Neck 414; Thoracic Outlet Syndrome 415; Cervical Rib 416; Branchial Cyst 419; Branchial Fistula 420; Pharyngeal Pouch 421 ; Laryngocele 422; Cystic Hygroma 424; Ludwig's Angina 425; Parapharyngeal Abscess 426; Retropharyngeal Abscess 426; Subhyoid Bursitis 427; Carotid Body Tumour 428; Torticollis 429; Sterno mastoid Tumour 430; Tuberculous Lymphadenitis 431 ; Cold Abscess 434; Secondaries in Neck Lym ph Nodes 435; Chemotherapy for Head and Neck Cancers 442

6. Thyroid

443

Development 443; Surgical Anatomy 443; Physiology 445; Congenital Anomalies 445; Thyroid Function Tests 449; FNAC of Thyroid 449; Classification of Goitre 450; Diffuse Hyperplastic Goitre 451 ; Multinodular Goitre 451 ; Discrete Thyroid Nodule 453; Solitary Thyroid Nodule 454; Retrosternal Goitre 457; Thyrotoxicosis and Hyperthyroidism 458; Radioactive Iodine 466; Thyroid Neoplasms 467; Papillary Carcinoma of Thyroid 468; Follicular Carcinoma of Thyroid 470; Differentiated Thyroid Carcinoma 472; Anaplastic Carcinoma of Thyroid 474; Madullary Carcinoma of Thyroid 476; Malignant Lymphoma 478; Hashimoto's Thyroiditis 478; De-Quervain's Subacute Granulomatous 478; Riedel's Thyroiditis 478; Thyroid lncidentaloma 479; Thyroidectomy 479; Emil Theodor Kocher 485; Kocher's Test 485; Hypothyroidism 485; Recurrent Laryngeal Nerve Palsy 486

7. Parathyroids and Adrenals

489

Anatomy 489; Calcium 489; Hyperparathyroidism 490; Parathyroidectomy 493; MEN Syndrome (MEA Syndrome) 496; Apudomas 496; Hypoparathyroidism 497; Tetany 498; Adrenals 498; Adrenal Cortical Tumours 498; Adrenocortical Carcinoma 499; Cushing's Syndrome 500; Conn's Syndrome 500; Virilising Syndrome or Adrenogenital Syndrome 500; Neuroblastoma 500; Phaeochromocytoma 502

8. Breast

504

Anatomy 504; Mammography 507; Aberration of Normal Development and Involution of the Breast 508; Fibroadenoma 508; Fibrocystadenosis 510; Sclerosing Adenosis 512; Phylloides Tumour 512; Mastalgia 513; Traumatic Fat Necrosis 514; Galactocele 514; Mastitis 515; Antibioma 517; Duct Ectasia 517; Mondor's Disease 517; Tuberculosis of the Breast 518; Breast Cysts 518; Galactorrhoea 519; Gynaecomastia 519; Duct Papilloma 521 ; Zuska-Atkins Disease 521 ; Mammary Fistula of Atkins 522; Carcinoma Breast 522; TNM Staging of Carcinoma Breast 532; Management of Early Carcinoma Breast 547; Advanced Carcinoma Breast 548; Prognostic Factors in Carcinoma Breast 551 ; Prophylactic Mastectomy 551 ; Carcinoma of Male Breast 551 ; Breast Reconstruction 552; Breast Implants 555; Nipple Retraction 556

9. Peritoneum

557

Anatomy 557; Physiology 557; Acute Peritonitis 558; Spontaneous Bacterial Peritonitis 564; Sclerosing Peritonitis 564; Biliary Peritonitis 564; Postoperative Peritonitis 565; Other Forms of Peritonitis 565; Pelvic Abscess 566; Subphrenic Spaces and Subphrenic Abscess 566; Mesenteric Cysts 569; Mesenteric Panniculitis 570; Acute Mesenteric Lymphadenitis 570; Mesenteric Malignancy 570; Mesenteric Trauma 571 ; Peritoneal Malignancy 571 ; Omental Cyst 572; Omental Torsion 572; Omental Tumour 572

10. Abdominal Tuberculosis

573

Abdom inal Tuberculosis 573; lleocaecal Tuberculosis 574; Ilea! Tuberculosis 578; Peritoneal Tuberculosis 579; Tuberculous Mesenteric Lymphadenitis 582; Ano-recto-sigmoidal Tuberculosis 583; Tuberculosis of the Omentum 583

11 . Liver Surgical Anatomy of Liver 584; Liver Function Tests 585; Alpha Fetoprotein 585; Liver Biopsy 586; Liver Injury 586; Infections of Liver 589; Liver Tumours 598; Liver Cysts 606; Portal Hypertension 607; Oesophageal Varices 610; Emergency Management in Severe Haemorrhage 612; Ascites 617; Ascites in Portal Hypertension 618; Budd-Chiari's

584

Di

Syndrome 619; Hepatic Failure 620; Hepatic Encephalopathy 620; Hepatorenal Syndrome 620; Hepatic Resection 621 ; Portal Biliopathy 622

623

12. Gallbladder Surgical Anatomy 623; Oral Cholecystogram 625; Intravenous Cholangiogram 625; Endoscopic Retrograde Cholangiopancreatography 625; Percutaneous Transhepatic Cholangiography 625; Magnetic Resonance Cholangiopancreatography 626; Radioisotope Scan Study 626; Peroperative Cholangiogram 626; Postoperative T-tube Cholangiogram 627;

Congenital Anomalies of Gallbladder 627;

Choledochal Cysts 628;

Caro li's

Disease 630; Biliary Atresia 630; Gallstones 631 ; Acute Cholecystitis 635; Acute Acalculous Cholecystitis 637; Mirizzi Syndrome 638; Empyema Gallbladder 638; Mucocele of the Gallbladder 639; Chronic Cholecystitis 639; Murphy's Sign 640;

Gallstone lieus 640;

Choledocholithiasis 642;

Cholecystoses 641 ;

Sump Syndrome 645;

Dissolution Therapy for Gallstones 642;

Courvoisier's Law 646;

Surgical Jaundice 646;

CBD

Strictures 649; Sclerosing Cholangitis 650; Gallbladder Polyp 650; Benign Biliary Papilloma 651 ; Carcinoma Gallbladder 651 ; Cholangiocarcinoma 653; Klatskin Tumour 653; Biliary Fistulas 654; Hemobilia 654; White Bile 655; Cholecystectomy 655; Open Approach Cholecystectomy 656; Laparoscopic Cholecystectomy 656; Single Incision Laparoscopic Surgery in Cholecystectomy 657; Bile Duct Injuries 658; Post-cholecystectomy Syndrome 658; Biliary Dyskinesia 659

660

13. Spleen Surgical Anatomy 660;

Functions of the Spleen 660; Splenunculi 661 ; Splenic Injury 661 ;

Atraumatic

Rupture of Spleen 664; Splenomegaly 665; Hereditary Spherocytosis 665; Immune Haemolytic Anaemia 666; Thalassaemia 666 ; Sickle Cell Disease 666; Idiopathic Thrombocytopaenic Purpura 667 ; Thrombotic Thrombocytopaenic Purpura 668; Splenectomy 668; Overwhelming Post-splenectomy Infection 670; Splenic Artery Aneurysm 670; Splenic Abscess 671 ; Hypersplenism 671 ; Splenic Cyst 671

14. Pancreas

672

Surgical Anatomy 672; Serum Amylase 674; Serum Lipase 674; Magnetic Resonance Cholangiopancreatography 675; Pancreatitis 675; Acute Pancreatitis 675; Complications of Acute Pancreatitis 681; Pseudocyst of Pancreas 682; Chronic Pancreatitis 685; Pancreatic Tumours 693; Exocrine Pancreatic Tumours 693; Carcinoma Pancreas 695; Endocrine Pancreatic Tumours 702; lnsulinomas 702; Gastrinomas 703; Glucagonomas 703; Zollinger-Ellison Syndrome 704; Cystic Fibrosis 704; Annular Pancreas 704; Ectopic (Accessory) Pancreatic Tissue 705; Pancreatic Divisum 705; Pancreatic Calculus 705; Pancreatic Ascites 706; Pancreatic Fistulae 706; Pancreatic Necrosis 706; Pancreatic Trauma 101; Cystic Lesions of Pancreas 707; Pancreatic Exocrine Insufficiency 708

15. Retroperitoneal Space

709

Anatomy of Retroperitoneum 709; Retroperitoneal Fibrosis 709; Retroperitoneal Swellings 710; Retroperitoneal Tumours 711 ; Psoas Abscess 714

16. Differential Diagnosis of Mass Abdomen

716

Mass in the Right Hypochondrium 718; Mass in the Epigastrium 719; Mass in the Left Hypochondrium 720; Mass in the Lumbar Region 721 ; Mass in the Umbilical Region 722; Mass in the Right Iliac Fossa 723; Mass in the Left Iliac Fossa 723; Mass in the Hypogastrium 723; Distal Rectal Examination of Prostate and Other Conditions 724

17. Abdominal Wall and Umbilicus

728

Diseases of the Umbilicus 728; Omphalitis 728; Umbilical Granuloma 729; Anomalies of Vitellointestinal Duct 729; Umbilical Sinus 730; Umbilical Adenoma 730; Umbilical Fistula 731 ; Patent Urachus 731 ; Abdominal Wall Tumours 734; Desmoid Tumour 734; Exomphalos 735; Gastroschisis 736; Rectus Sheath Haematoma 737; Abdominal Wall Abscess 737; Meleney's Progressive Synergistic Bacterial Gangrene of Abdominal Wall 737; Diverication of Recti 738

18. Hernia Aetiology 740; Parts of Hernia 741 ; Classification of Hernia 742; Inguinal Hernia 743; Strangulated Hernia 760; Sliding Groin/Inguinal Hernia 762; Pantaloon Hernia 763; Femoral Hernia 763; Ventral Hernia 765; lncisional

739

1111

Hernia 766; Umbilical Hernia 769; Paraumbilical Hernia 770; Epigastric Hernia 771 ; Spigelian Hernia 772; Obturator Hernia 773; Richter's Hernia 773; Lumbar Hernia 773; Sciatic Hernia 774; Complications of Hernia Surgery 774; Parastomal Hernia 775

776

19. Oesophagus Anatomy 776; Lower Oesophageal Sphincter 778;

Dysphagia 778; Contrast Study of Oesophagus 780;

Oesophagoscopy 780; Oesophageal Endosonography 781 ; Third Space Endoscopy 781 ; Gastro-oesophageal Reflux Disease 781 ; Hiatus Hernia 785; Rolling Hernia 786; Reflux Oesophagitis 786; Barrett's Oesophagus 787; Barrett's Ulcer 788; Oesophageal Motility Disorders 788; Achalasia Cardia 788; Plummer-Vinson Syndrome 791 ; Corrosive Stricture of Oesophagus 791 ; Schatzki's Rings 793; Boerhaave's Syndrome 793; Mallory-Weiss Syndrome 793; Trachea-oesophageal Fistula 793; Oesophageal Diverticulum 794; Carcinoma Oesophagus 795; Benign Tumours of the Oesophagus 802; Oesophageal Perforation 802

20. Stomach

804

Anatomy 804; Gastric Physiology 806; Gastric Function Tests 807; Gastrin 807; Barium Meal Study 808; Gastroscopy 808; Congenital Hypertrophic Pyloric Stenosis 811 ; Gastritis 81 2; Acute Peptic Uleer 813; Gastric Ulcer 813; Duodenal Ulcer 816; Pyloric Stenosis due to Chronic Duodenal Ulcer 818; Perforated Peptic Ulcer 820; Bleeding Peptic Ulcer 824; Haematemesis 827; Complications of Gastric Su rgery 828; Trichobezoar 831 ; Chronic Duodenal lieus 832; Dunbar's Syndrome 832; Acute Gastric Dilatation 833; Gastric Volvulus 833; Gastric Polyp 834; Menetrier's Disease 834; Duodenal Diverticula 835; Carcinoma Stomach 835; Gastric Lymphoma 846; Gastric Sarcomas 848; Gastrointestinal Stromal Tumours 848; Pyloroplasty 849; Gastrostomy 849; Gastrectomy 849; Gastrojejunostomy 850; Retrograde Jejunogastric lntussusception 850; Vagotomy 851

21 . Small Intestine

852

Anatomy 852; Meckel's Diverticulum 853; Regional Enteritis 855; Surgical Complications of Typhoid 858; Pneumatosis Cystoides lntestinalis 861 ; Mesenteric Vessel lschaemia 861 ; Necrotising Enterocolitis 863; Small Bowel Tumours 864; Benign Tumours of Small Bowel 865; Carcinoid Tumour 868; Short Bowel Syndrome 870; Small Bowel Enema 871 ; Capsule Endoscopy 871; Small Bowel Enteroscopy 872; Enteric/Gastrointestinal Fistula 872

22. Large Intestine

876

Anatomy 876; Hirschsprung's Disease 877; Diverticular Disease of the Colon 879; Ulcerative Colitis 883; lschaemic Colitis 887; Pseudomembranous Colitis 888; Surgical Complications of Intestinal Amoebiasis 888; Tumours of Colon 889; Carcinoma Colon 892; Angiodysplasia of Colon 900; Ogilvie's Syndrome 900; Colostomy 901 ; Stoma Care 903; Stoma Appliances 904; Faecal Fistula 905; Preparation of Large Bowel for Surgery 906; Surgical Pouches 906; Barium Enema 907

23. Intestinal Obstruction

908

Intestinal Obstruction: Types 908; Dynamic Obstruction 909; Duodenal Atresia 915; Small Intestine Atresia 916; Malrotation 917; Meconium lieus 918; lntussusception 919; Volvulus 922; Sigmoid Volvulus 922; Paralytic lieus 924; Adhesions and Bands 924; Internal Hernias 927

24. Appendix

928

Surgical Anatomy 928; Acute Appendicitis 929; Incidental Appendicectomy 936; Appendicular Abscess 937; Faecal Fistula after Appendicectomy 938; Mucocele of Appendix 938; Neoplasms of the Appendix 939; Laparoscopic Appendicectomy 939

25. Rectum and Anal Canal Surgical Anatomy of Rectum 942; Surgical Anatomy of Anal Canal 943; Per-rectal Examination of the Rectum 945; Proctoscopy (Kelly's) 945; Sigmoidoscopy 945; Colonoscopy 945; Carcinoma Rectum 946; Solitary Ulcer Syndrome 952; Rectal Prolapse 952; Anorectal Malformations 957; Pilonidal Sinus/Disease 958; Piles/ Haemorrhoids 961 ; Anal Fissure 967; Anorectal Abscess 969; Fistula-In-Ano 971 ; Ano rectal Strictures 976; Condyloma Acuminata 976; Anal lntraepithelial Neoplasia 976; Malignant Tumours of Anal Area 976; Sacrococcygeal

942

xxm

Teratoma 978; Anal Incontinence 978; Descending Perinea! Syndrome 979; Proctitis 979; Proctalgia Fugax 979; Hydradenitis Suppurativa of Anal Region 980; Pruritus Ani 980; Gastrointestinal Haemorrhage 980

26. Urology

984 C")

A. Kidney 984

C

::s

CD

::s

Anatomy of Kidney and Ureter 984; Plan X-ray-Kidney, Ureter and Bladder 985; Intravenous Urogram 986; Retrograde Pyelography 987; Renal Angiogram 987; Micturating Cystourethrography 987; Ascending

en'

Urethrogram 988; Isotope Renography 988; Cystoscopy 989; Catheters 990; Nephrostomy 992; Suprapubic Cystostomy 992; Haematuria 993; Horseshoe Kidney 993; Cystic Diseases of the Kidney 994; Duplication of Renal Pelvis and Ureter 995; Retrocaval Ureter 996; Ureterocele 996; Injuries to Kidney 997; Renal Tuberculosis 998; Hydronephrosis 1ooo; Pyonephrosis 1004; Carbuncle of Kidney 1004;

Perinephric Abscess 1005; Renal

Calculus 1005; Ureteric Calculi 1009; Staghorn Calculus 1011 ; Benign Tumours of Kidney 1012; Wilms' Tumour 101 2; Renal Cell Carcinoma 1013

B. Urinary Bladder 1018 Anatomy 1018; Ectopia Vesicae 1019; Urachal Anomalies 1019; Vesical Calculus 1019; Cystitis 1021; Recurrent Cystitis 1021; Interstitial Cystitis 1022; Schistosoma Haematobium 1022; Thimble or Systolic Bladder 1022; Bladder Tumours 1023; Transitional Cell Carcinoma 1023; Ureterosigmoidostomy 1026; Rupture Bladder 1026; Residual Urine 1027; Malakoplakia 1028; Neurogenic Bladder 1028; Vesicoureteric Reflux 1028; Bladder Diverticula 1029; Urinary Diversion 1030; Urinary Fistulas 1031

C. Prostate

1032

Anatomy 1032; Acid Phosphatase 1032; Prostate Specific Antigen 1032; Benign Prostatic Hyperplasia 1033; Prostatitis 1036; Bladder Outlet Obstruction 1036; Carcinoma Prostate 1037

D. Urethra

1040

Anatomy 1040; Urethral Injury 1040; Stricture Urethra 1042; Hypospadias 1044; Epispadias 1044; Posterior Urethral Valve 1045; Urethral Calculi 1045; Urethritis 1046; Extravasation of Urine 1046; Retention of Urine 1047

E. Penis 1048 Phimosis 1048; Paraphimosis 1049; Circumcision 1049; Balanoposthitis 1050; Chordee 1050; Priapism 1051; Peyronie's Disease 1051 ; Ram's Horn Penis 1051; Carcinoma Penis 1051 ; Buschke-Lowenstein Tumour 1055

F. Scrotum 1056 Anatomy 1056; Fournier's Gangrene 1056;

Hydrocele 1057;

Haematocele 1061;

Pyocele 1062; Cyst of

Epididymis 1062; Spermatocele 1063; Varicocele 1063

G. Testis

1065

Anatomy 1065; Undescended Testis 1065; Ectopic Testis 1068; Retractile Testis 1068; Torsion of the Testis 1068; Testicular Tumours 1070; Paratesticular Tumours 1074; Orchitis 1074; Epididymitis 1075

27. Neurosurgery

1076

Head Injuries 1076; Extradural Haematoma 1081; Subdural Haematoma 1082; Subarachnoid Haemorrhage 1083; Fracture Skull 1084; Depressed Skull Fracture 1084; CSF Rhinorrhoea 1085; Hydrocephalus 1085; lntracranial Abscess 1086; lntracranial Aneurysms 1087; lntracranial Tumours 1087; Pituitary Tumours 1090; Craniopharyngiomas 1091; Spinal Dysraphism 1091; Meningocele 1092; Spina Bifida 1092; lntervertebral Disc Prolapse 1093; Spinal Tumours 1096

28. Thorax Chest Injuries 1097; Fracture Ribs 1100; Flail Chest and Stove in Chest 1100; Pneumothorax 1101; Tension Pneumothorax 1101; Haemothorax 1102; Pleural Tap 1102; Bronchoscopy 1103; Empyema Thoracis 1103; Empyema Necessitans 1104; Lung Abscess 1104; lntercostal Tube Drainage 1105; Shock Lung 1101; Pulmonary Embolism 1101; Surgical Emphysema 1108; Lung Cysts 1109; Mediastinal Tumours 1109; Thymomas 1111; Lung Cancers 1111; PancoastTumours 1112; Chest Wall Tumours 1112; Pericarditis 1113; Pericardia! Tap 1113;

1097

DIV

Cardiac Tamponade 1113; Diaphragmatic Hernia 1114; Pulmonary Complications During Postoperative Period 1111; Surgical Management of Pulmonary Tuberculosis 1117; Video-assisted Thoracoscopic Surgery 1117

Cardiac Surgery 1118 Anatomy 1118; Preoperative Assessment and Preparation of the Cardiac Patient 1119; Cardiopulmonary Bypass 1120;

... = en

Congenital Heart Diseases 1120; Patent Ductus Arteriosus 1120; Coarctation of Ao rta 1121 ; Atrial Septal

Cl)

=

Defect 1121 ; Ventricular Septal Defect 1122; Pulmonary Stenosis 1122; Transposition of Great Vessels 1122; Tetralogy of Fallo! 1122; Acquired Heart Disease 1123; Mitral Regurgitation 1123; Aortic Stenosis 1124; Aortic Regurgitation 1124; Valve Replacement Surgery 1124; lschaemic Heart Disease 1124; Cardiac Pacemakers 1125

Q)

'E

C:

==en

1126

29. Adjuvant Therapy

a::i

Radiotherapy 1126; Chemotherapy 1128; Cell Cycle 1128; Antimalignancy Drugs 1129; Hormone Therapy in

1%:

Cl)

Cancer 1129; lmmunosuppression 1129; lmmunotherapy 1131 ; Hybridoma 1131; Gene Therapy 1131

1132

30. Anaesthesia Preoperative Assessment 1132; General Anaesthesia 1133; Regional Anaesthesia 1136; Spinal Anaesthesia 1136; Epidural Anaesthesia 1137

31 . Advanced Imaging Methods

1138

Ultrasound 1138; Doppler 1140; CT Scan 1140; Magnetic Resonance Imaging 1142; Radionuclide Imaging 1143; Positron-Emission Tomography Scan 1143

1144

32. Operative Surgery A. Sterilisation and Instruments 1144 Sterilisation 1144; Instruments 1146; Suture Materials 1153; Diathermy 1155

B. Operative Procedure

1156

Abdominal Incisions 11 56; Vasectomy 1157; Circumcision 1157; Hydrocele 1157; Inguinal Hernia 1157; Appendicectomy 1157; Thyroidectomy 1157; Tracheostomy 1157; Cryosurgery 1159; Lasers in Surgery 1159; Staplers in Surgery 1160; Nasojejunal Tube Feeding 1160; Gossypiboma 1161 ; Advantages of Laparoscopic Surgery 1161 ; Laparoscopic Cholecystectomy 1162; Laparoscopic Appendicectomy 1162; Advanced Laparoscopic Surgeries 1162; Diagnostic Laparoscopy 1162; Retroperitoneoscopy 1163; Natural Orifice Transluminal Endoscopic Surgery 1163

C. Dressings and Bandages 1164 Dressings 1164; Bandages 1164

D. Day Care Surgery 1165 Day Care Surgery 1165; Surgical Audit 1166; Surgeon and Law 1167

33. Miscellaneous

1168

A. Fascinating Signs in Surgery 1168 B. Triads in Surgery 1177 C. Misnomers in Surgery 1178 D. Triangles in Surgery 1179 Further Reading Appendix

1180 1181

Index

1185

Chapter

General Surgery A. Wounds and Wound Healing One having a wound in his eyebrow. An ailment which I will treat. Treatment (of a wound in the eyebrow): Now after thou hast stitched it (thou shouldst bind) fresh meat upon (it) the first day. If thou findest that the stitching of this wound is loose, thou shou/dst draw it together for him with two strips (of plaster), and thou shou/dst treat it with grease and honey every day until he recovers. -[Anonymous], circa 2500 BC

( iHAPT ER OUTLINE

•

• • • •

•

Wounds Classification of Wounds Wound Healing Compartment Syndro me Crush Injury Crush Syndrome

• • • • •

Degloving Injuries Scar Keloid Hypertrophic Scar Problems with Wound Healing

WOUNDS

,. It is incised, clean, healthy wound without any tissue loss. ,. Usually primary suturing is done. Healing is by primary intention. b. Untidy wounds ,. They are due to: Crushing, Tearing, Avulsion, Devitalised injury, Vascular injury, Multiple irregular wounds, Burns. ,. Fracture of the underlying bone may be present. Wound dehiscence, infection, delayed healing are common. ,. Liberal excision of devitalised tissue and allowing to heal by secondary intention is the management. ,. Secondary suturing, skin graft or flap may be needed.

I Wound Definition A wound is a break in the integrity of the skin or tissues often, which may be associated with disruption of the structure and function.

Wound is simply a disruption of any tissues- soft tissue or bone or internal organs. Ulcer is disruption or break in the continuity of any fining- may be skin, mucous membrane or others. Ulcer is one of the types of wounds.

CLASSIFICATION OF WOUNDS I. Rank and Wakefield classification a. Tidy wounds

,,. They are wounds like surgical incisions and wounds caused by sharp objects.

Fig. 1.1A: Tidy wound.

I dressed him and God healed him.- Ambroise Pare

2

underneath. It is more common on the skin over the bones; lax areas like face, scrotum, eyes; vascular areas; children, old aged, and fair skin people.

>... QJ

... :,

C'I

-"' V')

0

:::,

C

"' :E co"'

a:

V')

Fig. 1.18: Untidy

wound.

II. Classification based on the type of the wound

Clean incised wound: It is a tidy, simple, clean cutwound with linear cut edges; usually due to a sharp object like blade, glass piece or knife. It is treated by primary suturing.

Figs. 1.2A and B: Clean incised wounds.

Haematoma: It is a localized collection of blood after blunt trauma or after surgery. Collected fluid blood gets clotted in few minutes to hours; later eventually it liquefies to form a discolored fluid. It may be located in subcutaneous/intramuscular/subfascial/intra-articular regions. Large haematoma may get infected to form an abscess; so large haematoma needs drainage under general or regional anaesthesia. Small haematoma usually gets absorbed (like scalp haematoma). Often haematoma contains reddish plasmatic fluid which should be aspirated using wide bore needle. Complications are-pressure effects and abscess formation; both needs incision and drainage under anaesthesia. Haematoma causing cosmetic problems may require needle aspiration. Haematoma can occur spontaneously in coagulation disorders (haemophilia) or in individuals who are on anticoagulant drug therapy.

Lacerated wound: It has ragged edges with some part of the tissues getting devitalized; viability of the tissues may be impaired; depth of the injury and tissue damage should be carefully assessed. Proper adequate wound excision, thorough warm saline wash and suturing of the wound layer-by-layer is required. Bruise or contusion: It is due to blow or blunt force to the skin and tissues underneath wherein blood vessels or capillaries are damaged underneath. There is skin discolouration without breaking of the skin; broken vessels cause seepage of blood underneath; minor soft tissue injury crushes small vessels without breaking the skin, accumulating the trapped blood

Figs. 1.4A and B: (A) Haematoma of eyelid; (B) Subungual haematoma. Often nail may need to be removed to evacuate the blood clot.

Deeper vital structures or organs may be injured, so should be assessed; foreign body or object may be present in the depth of the wound. Wound should be explored under general or regional anaesthesia to assess the depth and severity of the injury and sutured layer by layer after a through saline wash.

3

n :c )> ""C

-I

m

:0 )>

Fig. 1.5: Haematoma in the groin being aspirated. Haematoma

may get infected or can compress adjacent structures.

Fig. 1.8: Penetrating wound of the abdomen.

Penetrating wounds: They are similar to puncture wounds; occur to stab. Abdomen and chest are common sites. Liver, bowel, spleen, major vessels and other visceral organs may be involved. Ultrasound and CT scan should be done to evaluate deeper organ injuries. Under general anaesthesia wound should be explored properly.

Fig. 1.6: Hematoma leg-after aspiration clot is evacuated.

Abrasion: It is superficial injury (scratch/graze/pressure/contact) and is due to shearing of the skin where the surface is rubbed off. This tangential force causes loss of epidermis exposing dermal vessels and nerves leading to profuse painful oozing. Abrasion heals by epithelialisation. Any dirt or foreign body on the abrasion should be removed to avoid formation of poor tattoo like scar.

Traction and avu/sion injuries: These are complex injuries where the tissues are displaced from their normal anatomical position and alignment. It can occur in single plane like in subcutaneous tissue or in multiple planes like in machinery injuries, major injuries or degloving injuries. lschaemia, bleeding, sepsis, loss of wide tissue area are common problems. Open traction injury occurs on the surface. Closed traction injury can occur in deeper plane like brachia! plexus injury or traction bowel injury. After initial resuscitation, definitive treatment like skin graft or nerve repair should be done. Crush injury: It is due to major wounds, war wounds, natural disaster like earth quake injuries, tourniquet injury. It leads into-compartment syndrome; muscle ischaemia; loss of tissues; gangrene; sepsis. Muscle will lose its viability which is identified by its colour(dark coloured with loss of shining); lose its contractility; becomes turgid, and will not bleed on cutting.

Fig. 1.7: Typical look of abrasion in face.

Puncture wounds and bites: It is usually a stab wound with a pointed object; here depth of the wound is more than the width.

Fig. 1.9: Severe crush injury leg.

A great part, I believe, of the art of medicine is the ability to observe.-Hippocrates, Father of Medicine

4

Gunshot injuries: These injuries may be superficial or deep. Usually entry wound and exit wound will be present. It causes explosive and destructive injuries along with burn injuries in the deeper planes and organs. It can be high velocity inju ries with massive bleeding and major organ injuries. Injuries to bones and joints: It is common in all major injuries; should be identified clinically and confirmed radiologically. It needs specialized management like reduction, plating, etc.

.,,

co a:: V'I

Injuries to nerves, arteries, veins (major vessels), deeper organs: It needs initial resuscitation, later staged management as per individual patient basis depending on the severity and extent of injury and loss of function. Closed blunt injury: It may be due to fall or blunt injury wherein no obvious external injury is seen but deeper injury can occur; it may be often severe enough to cause major injury like in blunt abdominal injury causing bowel/liver/spleen/renal injuries. Note: Wound may be closed (bruise, haematoma, blunt injury); open (abrasion. incised, lacerated, penetrating) or complex (traction/avulsion, crush or gunshot injuries).

Ill. Classification based on thickness of the wound Superficial wound involving only epidermis and dermal papillae. Partial thickness wound with skin loss up to deep dermis with only deepest part of the dermis, hair follicle shafts and sweat glands are left behind. Full thickness wound with loss of entire skin and subcutaneous tissue causing spacing out of the skin edges. Deep wounds are the one extending deeper, across deep fascia into muscles or deeper structures. Complicated wounds are one associated with injury to vessels or nerves. Penetrating wound is one which penetrates into either natural cavities or organs. IV. Classification based on involvement of structures Simple wounds are one involving only one organ or tissue. Combined/complex wounds are one involving mixed tissues.

Fig. 1.10: Acute deep wound adjacent to elbow joint.

matory phase. Diabetes, venous/arterial diseases, nutritional deficiencies are the causes. Hypoxia initially is a potent stimulus for fibroblast activity and angiogenesis; but persistent hypoxia impedes fibroblast and collagen activity and also allows bacterial invasion to make wounds chronic. Chronic wounds are chronically infected with biofilms which interfere mainly with the inflammatory phase of healing, contributing to the non-healing. A biofilm is a complex structure of microorganisms contained in an extracellular matrix of proteins and polysaccharides that adhere to a surface, creating a protected environment for the organisms. Chronic ulcers are unresponsive to dressing treatment; it requires biopsy, culture study, definitive treatment like wound debridement, VAC (vacuum-assisted closure) therapy, skin grafting or flap. Specific conditions like tuberculosis if present should be treated. Malignancy if confirmed is treated by wide local excision and skin graft.

V. Classification based on the time elapsed

Acute wounds are generally defined as those that progress through the normal phases of healing and typically show signs of healing in less than 4 weeks; examples are-surgical or traumatic or burn wounds; progress through the healing phases in a timely and orderly fashion-haemostasis, inflammation, proliferation, and remodeling or maturation. A wound is considered chronic if healing does not occur within the expected period according to its aetiology and localization. Chronic wounds can be classified as typical and atypical. Typical wounds include ischaemic, neurotrophic and hypostatic ulcers and diabetic foot and decubitus ulcers. Atypical chronic wounds can be caused by autoimmune disorders, infectious diseases, vascular diseases and vasculopathies, metabolic and genetic diseases, neoplasm, external factors, psychiatric disorders, drug-related reactions, etc. They do not follow the normal healing process and show no signs of healing. It fails to progress through the normal phases of healing but with prolonged inflam-

Fig. 1.11: Chronic wound over the scalp. VI. Classification of surgical wounds (berard wound classification) 1. Clean wound: Elective, primarily closed; no entrance of normally or frequently colonized body cavities; no break in sterile technique. Examples are-hernioplasty, excisions, thyroidectomy, surgeries of brain, joints, heart and transplant. Infective rate is less than 2%. 2. Clean contaminated wound: Controlled opening of a normally colonized body cavities; minimal spillage or break in sterile technique. Infective rate here is up to 30%. Examples areAppendicectomy, gastrojejunostomy, pancreatic and biliary surgeries.

5

3. Contaminated wound: Acute inflammation ; break in sterile technique; spillage from hollow organs; penetrating trauma less than 4 hours from injury; chronic open wounds. Examples are-acute abdominal conditions. Infective rate is up to 60%. 4. Dirty wound: Purulent abscess; perforation with colonized bacteria; penetrating trauma more than 4 hours from injury. Examples are-abscess, perforated viscous with peritonitis, faecal contamination. Infective rate is more than 60%.

....)>

Fig. 1.14: Contaminated wound-burst appendicitis.

Fig. 1.12: Clean wound of thyroidectomy surgery.

Fig. 1.13: Appendicectomy wound, which is a clean contaminated wound.

T

Wound classifications

• Simple wounds: Only skin is involved • Complex wounds: Vessels, nerves, tendons or bones are involved

• Tidy wounds

• Closed wounds: - Contusion

• Clean wound • Clean contaminated wound • Contaminated wound • Dirty wound

- Abrasion - Haematoma • Open wounds: - Incised wounds - Lacerated wounds - Crush injuries - Penetrating wounds

• Untidy wounds Figs. 1.15A and B: Acute peritonitis with frank pus in peritoneal cavity due to bowel perforation is a dirty wound.

Fig. 1.16: Wound causing extensive skin loss and necrosis.

Start by doing what's necessary, then do what's possible and suddenly you are doing the impossible.

6

WOUND HEALING

I A scab is a beautiful thing-a coin the body has minted, with an

...>a, ...::IC'I

V'I

0

n:s

::I C:

n:s

co a: VI

V'I

invisible motto: In God We Trust. Our body loves us, and, even while

the spirit drifts dreaming, works at mending the damage that we do. -John Updike, 1984

j

Wound healing is complex process to achieve anatomical and functional integrity of disrupted tissue by various components like neutrophils, macrophages, lymphocytes, fibroblasts, collagen ; in an organised staged pathway-haemostasis matrix synthesis (collagen and proteoglycan ground remodelling epithelialisation wound contraction (by myofibroblasts).

I Types of Wound Healing Primary Healing (First Intention) It occurs in a clean incised wound or surgical wound. Wound edges are approximated with sutures. There is more epithelial regeneration than fibrosis. Wound heals rapidly with complete closure. Scar will be linear, smooth , and supple.

Secondary Healing (Second Intention) It occurs in a wound with extensive soft tissue loss like in major trauma, burns and wound with sepsis. It heals slowly with fibrosis. It leads into a wide scar, often hypertrophied and contracted. It may lead into disability. Re-epithelialisation occurs from remaining dermal elements or wound margins.

Fig. 1.18: Healing ulcer with healthy granu lation tissue which is

ready for skin grafting.

B • Stage of haematoma and inflammation • Stage of granulation tissue formation and organisation. Here due to fibroblastic activity, synthesis of of collagen and ground substance occurs • Stage of epithelialisation • Stage of scar formation and resorption • Stage of maturation

I Phases of Wound Healing Inflammatory Phase (Lag or Substrate or Exudative Phase) It begins immed iately after formation and lasts for 72 hours. There is initial arteriolar vasoconstriction, thrombus fo rmation, platelet aggregation due to endothelial damage and release of adenosine diphosphate (ADP). Later vasodilatation and increased vascular permeability develops. Here haemostasis, coagulation and chemotaxis occur. All these cause features of acute inflammatiotrrubor, calor, tumour, dolor and loss of function. Note:

Fig. 1.17: Wound in the abdomen healing with second intention,

which requires secondary suturing once it granulates well. Secondary suturing is done after 10-14 days , once wound granulates well after proper control of infection. Scar in such type is prone to form incisional hernia.

Healing by Third Intention {Tertiary Wound Healing or Delayed Primary Closure) After wound debridement and control of local infection, wound is closed with sutures or covered using skin graft. Primary contaminated or mixed tissue wounds heal by tertiary intention.

Coagulation begins at wound formation of platelet fibrin release of cytokines, PDGF (platelet-derived growth factor), epidermal growth factor (EGF), tran sforming growth factor-~ (TGF-~). platelet activating factor and platelet factor IV, fibrin, serotonin. Chemotaxis causes initially causes initially neutrophil neutrophil migration, and then activation of macrophages, lymphocytes leading into phagocytosis, wound debridement, matrix activation, angiogenesis. Chemotaxis factors are complement factors, interleukin-1, TNF-n (tumour necrosis factor-a) TGF-~ and platelet factor. Activated macrophages produce free radicals and nitric oxide; release cytokine to activate lymphocytes which release interferon and interleukin (called as lymphokines). These factors attract polymorphonuclear leucocytes (PMN-po lymorphonuclear cellsneutrophills) in 48 hours secreting inflammatory mediators and bactericidal oxygen-derived free radicals. Injured tissues and platelet release histamine, serotonin and prostaglandins which increases the vascular permeability by vasodilatation. These actions are reduced in diabetes mellitus, Cushing's syndrome and immunosuppression increasing the sepsis rate.

Proliferative Phase (Collagen/Fibroblastic Phase)

Contd...

It begins from 3rd day and lasts for 3-6 weeks. There will be formation of granulation tissue and repair of the wound. Granulation tissue contains fibroblasts, neocapillaries, collagen, fibronectin and hyaluronic acid. Note:

(1) Initial angiogenesis (growth of new blood vessels) occurs following release of vascular endothelial cell growth factor (VEGF) by keralinocytes; by release of TNF-a, TGF-~. PDGF, FGF by macrophages. (2) Eventual fibroplasia develops by fibroblast activity with formation of collagen and ground substance/glycosaminoglycans. Type Ill collagen is deposited initially in a random fashion. (3) Later re-epithelialisation of the wound surface occurs by migration of basal layer of the retained epidermis which proliferates, differentiates and stratifies to form wound closure.

Remodelling Phase (Maturation Phase) It begins at 6 weeks and lasts for 6 months to 1 or 2 years. There is maturation of collagen by cross linking and realignment of collagen fibers along the line of tension, which is responsible for tensile strength of the scar. There is reduced wound vascularity. Fibroblast and myofibroblast activity causes wound contraction. Type Ill collagen is replaced by type I collagen causing maturation of the collagen. Ratio of type I collagen to type Ill collagen becomes 4:1. Early extracellular matrix contains fibronectin and collagen type 111; eventually it contains glycosaminoglycans and proteoglycans; final matrix contains type I collagen. Scar strength is 3% in 1 week; 20% in 3 weeks; 80% in 12 weeks. Final matured scar is acellular and avascular. Note:

Initially fibrin, fibronectin, proteoglycans deposition occurs; later collagen protein develops to form scar. Normal dermal skin contains 80% type I (20% type Ill) collagen; granulation tissue contains mainly type Ill collagen; scar contains both type I and 111 collagen, initially in equal proportion, later becomes 4:1. Basic essential components of collagen are proline and lysine. Hydroxylation of lysine and later glycosylation of this hydroxylysine decides the type of collagen molecule. Hydroxylation of both proline and lysine as essential step needs adequate concentration of vitamin C, iron and a ketogluteric acid. Collagen deposition in the wound is assessed by quantity of hydroxyproline excreted in urine. There is a balanced activity of collagen production and degradation of collagen (collagenolysis). Collagen is broken down by collagenase and MMPs (matrix metalloproteinases). Procollagen through procollagenase collagen fibril cross linking collagen fiber deposition. Deposited collagen through collagenase degradation and collagenolysis.

B

POINTS TO BE REMEMBERED

• PMN cells survive only for 24 hours; so after 48 hours PMNs would not be found in the wound significantly; PMNs are not needed for wound healing. Activated monocytes called macrophages predominate after 48 hours which will persist until completion of the wound healing. Macrophages are the main cells of wound healing. Macrophages secrete TNF-a, interleukin-1 , fibroblast growth factor (FGF), proteinases (MMPs- matrix metallo proteinases). Contd...

lymphocyte will not have any role in wound healing; T lympho- 1 cytes produce stimulatory cytokines like interleukin-1 supporting the fibroblast activity. Collagen and glycosamines are produced by fibroblasts. Tropocollagen is produced which aggregates to form collagen fibrils. Hydroxyproline and hydroxylysine are synthesized by specific enzymes using iron, a ketoglutarate and vitamin C. Fibroblast requires vitamin Cto produce collagen. Granulation tissue and early scar contains type Ill collagen. Final scar contains type I collagen mainly. Final extracellular matrix contains type I collagen and proteoglycans. Collagen production decreases after 4 weeks of wound healing (declines in 28-42 days). Eighty per cent of tensile strength of normal skin will be achieved finally but not 100%.

Factors Affecting Wound Healing

B Infection Presence of necrotic tissue and foreign body

7

....)> Cl 11)

::::,

....

11) QI

VI C

.... 11) ....

IC

':c: C

::::,

Q. VI QI

::::,

Q.

Poor blood supply Venous or lymph stasis Tissue tension Haematoma Large defect or poor apposition Recurrent trauma X-ray irradiated area Site of wound, e.g. wound over the joints and back has poor healing Underlying diseases like osteomyelilis and malignancy Mechanism and type of wound-incised/lacerated/crush/avulsion Tissue hypoxia locally reduces macrophage and fibroblast activity

Age, obesity, smoking, alcohol, stress Malnutrition, zinc, copper, manganese Vitamin deficiency (Vil C, Vil A) Anaemia, hypoxia Malignancy Uraemia Jaundice Diabetes, metabolic diseases HIV and immunosuppressive diseases Steroids and cytotoxic drugs ._____!':!_europathies of different causes _ _ __

Age: In younger age group wound healing is faster and better. In elderly healing is delayed due to reduction in collagen synthesis, epithelialisation, growth factors and angiogenesis. But final scar will be excellent in old individuals.

Clinical diagnosis is an art, and the mastery of an art has no end: you can always be a better diagnostician -Logan Clendening

C ::::,

Q.

:i:::

11) QI

::::, IC

8

...>.

... ....0

"0

m :c

....

0,

Fig. 1.42: Basal cell carcinoma of face (rodent ulcer). Ulcer edge is raised and beaded in appearance.

C) I'!)

::,

... I'!)

QI

VI

... 1.0 C:

... I'!)

':': C

-;:;;

... I'!)

Figs. 1.40A and B: Maggots seen in a chronic ulcer.

Fig. 1.41: Tuberculous ulcer ankle area. Note the undermined edge. Discharge study, biopsy and later antituberculous drugs are the treatment. They are usually painless.

2. Malignant ulcers: Carcinomatous; rodent (BCC); melanotic. 3. Non-specific ulcers:

,.

Traumatic ulcer. It may be mechanical, physical, chemical-common.

,. ,. ,,. ,.

Arterial ulcer: Atherosclerosis, TAO Venous ulcer: Gravitational ulcer, post-phlebitic ulcer. Trophic ulcer/Pressure sore. Infective ulcers. Pyogenic ulcer.

Fig. 1.43: Squamous cell carcinoma (SCC/epithelioma) leg with typical everted edge. SCC can be ulcerative, ulceroproliferative or proliferative ulcer on clinical look.

Figs. 1.44A and B: (A) Malignant proliferative ulcer; (B) Melanotic ulcer.

,. Tropical ulcers. It occurs in tropical countries. It is callous type of ulcer, e.g. Vincent's ulcer. ,. Ulcers due to chilblains and frostbite (cryopathic ulcer). ,. Martorell's hypertensive ulcer. , Bazin's ulcer. , Diabetic ulcer. ,. Ulcers due to leucaemia, polycythemia, jaundice, collagen diseases, lymphoedema.

Excellence is never granted to man but given as the reward of labour.

18

,. Cortisol ulcers are due to long-time application of cortisol (steroid) creams to certain skin diseases. These ulcers are callous ulcers last for long time and require excision and skin grafting.

Fig. 1.49: Non healing ulcer foot in a diabetic patient with Pseudomonas infection. Note the greenish discharge in the wound. Pseudomonas infection is commonly hospital acquired.

I Wagner's Grading/Classification of Ulcer Fig. 1.45: lschaemic ulcer foot. Middle three toes are already

amputated because of gangrene.

Grade O Grade 1 Grade 2 Grade 3 Grade 4 Grade 5 -

Preulcerative lesion/healed ulcer Superficial ulcer Ulcer deeper to subcutaneous tissue exposing soft tissues or bone Abscess formation underneath/osteomyelitis Gangrene of part of the tissues/limb/foot Gangrene of entire one area/foot

B 1. Stage of extension: Ulcer floor is covered with slough, purulent discharge and inflamed edge and margin. 2. Stage of transition: Floor shows separated slough; healthy granulation tissue; serous discharge. 3. Stage of repair: Fibrosis, collagen deposition, scar formation 1 occurs.

Fig . 1.46: Venous ulcers in both feet. Site is around ankle (Gaiter's zone). There are healthy granulation tissues. It needs skin grafting and definitive procedure for varicose veins after evaluation.

Fig. 1.47: Infective ulcer in the foot. Note the quantity of slough, exposed tendon and gangrenous toes. Patient requires below or above knee amputation.

a. Serous. In healing ulcer b. Purulent: In infected ulcer - Staphylococci: Yellowish and creamy - Streptococci: Bloody and opalescent - Pseudomonas: Greenish colour due to pseudocyanin c. Bloody: Malignant ulcer, healing ulcer from healthy granulation tissue d. Seropurulent e. Serosanguinous. Serous and blood f. Serous with sulphur granules: Actinomycosis g. Yellowish: Tuberculous ulcer

GRANULATION TISSUE It is proliferation of new capillaries and fibroblasts intermingled with red blood cells and white blood cells with thin fibrin cover over it.

I Types

Fig. 1.48: Large ulcer in the foot and leg with exposed tendon.

Healthy granulation tissue: It occurs in a healing ulcer. It has got sloping edge. It bleeds on touch. It has got serous discharge. 5 Ps of granulation tissue-Pink, Punctate haemorrhages, Pulseful, Painless, Pin head granulation. Skin grafting takes up well with healthy granulation tissue.

Streptococci growth in culture should be less than 105/gram of tissue before skin grafting.

INVESTIGATIONS FOR AN ULCER Study of discharge: Culture and sensitivity, AFB study, cytology.

19

('\

::c

)>

-,::,

m

:x,

Fig. 1.50: Healing ulcer with healthy granulation tissue. Note the sloping edge.

Unhealthy granulation tissue: It is pale with purulent discharge. Its floor is covered with slough. Its edge is inflamed and oedematous. It is a spreading ulcer. Unhealthy, pale, flat granulation tissue: It is seen in chronic nonhealing ulcer (callous ulcer). Exuberant granulation tissue (Proud flesh): It occurs in a sinus or ulcer wherein granulation tissue protrudes out of the sinus opening or ulcer bed like a proliferating mass. It is commonly associated with a retained foreign body in the sinus cavity.

Fig. 1.51 : Exuberant granulation tissue (proud flesh) in an ulcer. It should be scooped out using Volkman n's scoop prior to skin grafting.

Sprouting granulation tissue of sinus. Pyogenic granuloma: It is a type of exuberant granulation tissue. Here granulation tissue from an infected wound or ulcer bed protrudes out, presenting as a well-localised, red swelling which bleeds on touching. Differential diagnosis: Papilloma, skin adnexal tumours. Treatment: Antibiotics, excision and sent for biopsy.

I.I\

C

Fig. 1.53: Typical greenish coloured ulcer due to Pseudomonas infection.

11) ....

':'S

C

"....

11)

Fig. 1.54 : X-ray showing osteomyelitis with sequestrum inside. Osteomyelitis prevents ulcer healing. Bone thickening on clinical examination is typical.

Wedge biopsy Biopsy is taken from the edge because edge contains multiplying cells. Usually two biopsies are taken. Biopsy taken from the centre may be inadequate because of central necrosis. X-ray of the part to look for periostitis/osteomyelitis. FNAC of the lymph node. Chest X-ray, Mantoux test in suspected case of tuberculous ulcer. Haemoglobin, ESR, total WBC count, serum protein estimation (albumin). Note: Ulcer will not granulate if haemoglobin is less than 8 go/o and serum albumin is . ,_ (LI

Cl ,_

:::,

VI

0

RJ

:::, C:

RJ

:E VI

c.o

Assessment of wound is important-anatomical site; size and depth of the wound; edge of the wound; mobility; fixity; induration; surrounding area; local blood supply. Wound perimeter may be useful in assessing this Wound imaging is done by tracing it on a transparent acetate sheet at regular intervals Presence of systemic features; regional nodal status; function of the limb/part; joint movements; distal pulses; sensations should be assessed Severity of infection should be assessed-culture of discharge Specific investigations like wedge biopsy; X-ray of part; blood sugar; arterial/venous Doppler; angiogram

away; foam is sealed airtight using a sterile adhesive film. Tube is connected to suction system. Suction is maintained initially continuously later intermittently. Redressing is done only after 4-7 days.

a: VI

MANAGEMENT OF AN ULCER Cause should be found and treated. Correction of the anaemia, deficiencies like of protein and vitamins. Proper investigation as needed. Transfusion of the blood if required . Control the pain and infection. Rest, immobilization, elevation, avoidance of repeated trauma. Care of the ulcer by debridement, ulcer cleaning and dressing. Desloughing is done either mechanically or chemically. Mechanically it is done using scissor by excising the slough. Hydrogen peroxide which releases nascent oxygen is used as chemical agent. Acriflavine is antiseptic and irritant and so desloughs the area and promotes granulation tissue formation. Eusol (Edinburgh University Solution) which contains sodium hypochlorite releases nascent chlorine which forms a water-soluble complex with slough to dissolve it. Use of povidone iodine in ulcer cleaning is controversial (open wound is not suitable; it is mainly for cleaning the surgical field prior to incision). Maggots if present in the wound will cause crawling sensation and are removed using turpentine solution. Removal of the exuberant granulation tissue is also required when present. Ulcer cleaning and dressing is done daily or twice daily or once in 2-3 days depending on the type of ulcer and type of dressing used. Normal saline is ideal for ulcer cleaning. Various dressings are available. Films (opsite/semipermeable polyurethane), hydrocolloids (duoderm), hydrogels (polyethylene oxide with water), hydroactives (nonpectin-based polyurethane matrix), foams (polyurethane hydrophilic or hydrophobic non-occlusive), impregnates (non-adherent fine mesh impregnated with antibacterials), calcium alginates, etc. Topical antibiotics for infected ulcers are not essential but like framycetin, silver sulphadiazine, mupirocin may be used. Vacuum assisted closure (VAC) therapy. It is by creation of negative pressure (25-200 mmHg), continuous or intermittent over the wound surface; it causes reduced fluid in the interstitial space, reduces oedema, increases the cell proliferation and protein matrix synthesis, promotes formation of healthy granulation tissue. Sterile foam is placed over the ulcer bed covering widely; tube drain with multiple holes is kept within it and end of the tube comes out significantly

Fig. 1.55: Vacuum-assisted closure of an ulcer.

Therapy using infrared/short wave/ultraviolet rays to decrease the ulcer size is often used but their benefits are not proved . Amnion to promote re-epithelialisation, chorion to promote granulation tissue formation is also often used. Antibiotics are not required once healthy granulation tissues are formed. Maggot debridement therapy. It is used as biotherapy (but not commonly) by placing cultured live disinfected maggots. Maggots are larvae of the green bottle fly, also known as the green blowfly (Lucilia sericata). They act by dissolving and engulfing dead necrotic tissues; they may reduce the bacterial content in the wound. They can inhibit many bacteria including MRSA (methicillin-resistant bacteria), anaerobic and aerobic bacteria. They secrete proteolytic enzymes to have mechanical effects; secretion of ammonia alters the pH in the ulcer bed which inhibits bacterial growth. They increase the granulation tissue formation also. Once ulcer granulates, defect is closed with secondary suturing, skin graft or flaps. Autologous bone marrow monocytes injection into the ulcer area is new concept by Professor Sribatsa Mahapatra but yet to confirm.

Fig. 1.56: Usually skin grafting is used to cover the defect (healthy ulcer bed).

B To keep ulcer moist To keep surrounding skin dry To reduce pain To soothen the tissue To protect the wound As an absorbent for the discharge

21

....

VI C I.C

... ... '< It>

C

n

...It> Figs. 1.57A and B: (A) Ulcer with slough which is nonhealing; (B) It becomes healing ulcer with healthy granulation tissue which will be covered with split skin graft.

Figs. 1.59A and B: Solutions commonly used for ulcer and surrounding area cleaning and dressing-povidone iodine (brownish) ; EUSOL (colourless); hydrogen peroxide and normal saline. Note the EUSOL bath. Dilute EUSOL solution in a basin is used wherein ulcer foot is dipped and kept in place fo r 20-30 minutes. EUSOL removes the slough and cleans the ulcer bed. Hydrogen peroxide releases nascent oxygen and helps in removing necrotic material. Povidone iodine is not used for open wound, it is only a surface antiseptic. Figs. 1.58A to C: Ulcer leg with exposed bone. Patient underwent local rotation flap to cover. Area from where flap is rotated is covered with split skin graft. When the bone is exposed, skin grafting is not possible.

It is removal of devitalised tissue Small ulcers are debrided in the ward Large ulcers are debrided in operation theatre under general anaesthesia

Cotton dressing--a, e1

:::,

V"I

0

"':::,C:

"' "' co a: V"I

Recurrent infection Trauma, presence of foreign body or sequestrum Absence of rest and immobilization Poor blood supply, hypoxia Oedema of the part Loss of sensation Periostitis or osteomyelitis of the underlying bone Fibrosis of the surrounding soft tissues Lymphatic diseases General/specifi c causes:

Anaemia, hypoproteinaemia Vitamin deficiencies Tuberculosis, leprosy Diabetes mellitus, hypertension Chronic liver or kidney diseases Steroid therapy locally or systemically Cytotoxic chemotherapy or radiotherapy Malignancy

TRAUMATIC ULCER Such ulcer occurs after trauma. It may be mechanicaHental ulcer along the margin of the tongue due to tooth injury; physical like by electrical burn; chemical like by alkali injury. Such ulcer is acute, superficial, painful and tender. Secondary infection or poor blood supply of the area make it chronic and deep. Footballer's ulcer is a traumatic ulcer occurring over the shin of males due to direct knocks on the shin. It is staphylococcal infection with a chronic and deep ulcer. Traumatic ulcers can occur anywhere in the body due to trauma.

Trauma causes infection, necrosis, fasciitis, crush injury, endarteritis of the skin leading into formation of large/deep nonhealing ulcer. Treatment depends on size and extent of ulcer. Regular dressing, later skin grafting is done.

TROPHIC ULCER (PRESSURE SORE/ DECUBITUS ULCER) Pressure sore is tissue necrosis and ulceration due to prolonged pressure. Blood flow to the skin sto ps once external pressure becomes more than 30 mmHg (more than capillary occlusive pressure) and this causes tissue hypoxia, necrosis and ulceration. It is more prominent between bony prominence and an external surface. It is due to-impaired nutrition; defective blood supply; neurological deficit, pressure, anaemia, injury, moisture. It is common in patients with orthopaedic and head injuries, comatose and stroke patients, old age and tetanus patients. Sites: Over the ischial tuberosity; sacrum; heel; heads of metatarsals; buttocks; shou lder; occiput. Due to the presence of neurological deficit, trophic ulcer is also called as neurogenic ulcer/neuropathic ulcer. Init ially it begins as callosity due to repeated trauma and pressure, under which suppuration occurs and gives way through a central hole which extends down into the deeper plane up to the underlying bone as perforating ulcer (penetrating ulcer'). Bedsores are trophic ulcers.

B Normal stimulus to relieve the pressure is absent in anaesthetised patient Nutritional deficiencies worsens the necrosis Inadequate padding over the bony prominences in malnourishedj patients Urinary incontinence in paraplegia patient causes skin soilingmaceration-infection-necrosis

Fig. 1.60: Traumatic ulcers in the upper limb.

Fi g. 1.62: Trophic ulcer- heel. It is deep punched out ulcer.

It often requires rotation flap/transposition flap.

Fig. 1.61 : Degloving ulcer with devitalised exposed

tendons on the dorsum of the hand.

Features: It occurs in 5% of all hospitalised patients. It is painless deep , nonmobile, ulcer which is punched out with base formed by bone.

nutrition; use of water bed/air bed/ripple bed/air-fluid flotation bed and pressure dispersion cushions to the affected area; urinary and faecal care; hygiene; psychological counselling. Regular skin observation; keeping skin clean and dry (using regular use of talcum powder); oil massaging of the skin and soft tissues using clean, absorbent porous clothing; control and prevention of sepsis helps in the management.

23

:::c l>

-0

m

....

::x:, CJ

ULCER DUE TO CHILBLAINS It is due to exposure to intense cold causing blisters and ulcerations in the feet. These ulcers are superficial. It is due to excessive cutaneous arteriolar constriction. The condition is also called as perniosis.

ULCER DUE TO FROSTBITE Figs. 1.63A loC: Bedsore (decubitus ulcer) over the greater trochanter, ischium and sacrum. Bedsore is a trophic ulcer. It is usually with punched out edge.

B • Diabetic neuropathy Peripheral neuritis • Tabes dorsalis • Spina bifida • Leprosy

Spinal injury Paraplegia Peripheral nerve injury Syringomyelia

Investigations: Study of discharge; blood sugar; wedge biopsy from the edge; X ray of the part and spine.

B

STAGING OF PRESSURE SORE

• Nonblanching erythema--early superficial ulcer Partial thickness skin loss-late superficial ulcer • Full thickness skin loss extending into subcutaneous tissue but not through fascia-early deep ulcer • Full thickness skin loss with fasciaand underlying structures like muscle/tendon/bone, etc.-late deep ulcer Treatment ,.. Cause should be treated, correction of diabetes and anaemia. ,, Nutritional supplementation. ,, Rest, antibiotics, slough excision, regular dressings. ,.. Vacuum-assisted closure (VAC). , Once ulcer granulates well, flap cover or skin grafting is done. ,, Excision of the ulcer and skin grafting. , Flaps-local rotation or other flaps (transposition flaps). ,, Cultured muscle interposition. ,.. Proper care: Change in position once in 2 hours; lifting the limb upwards for 10 seconds once in 10 minutes;

It is due to exposure of a part to wet cold below the freezing point (cold wind). There is arteriolar spasm, denaturation of proteins and cell destruction. It leads to gangrene of the part. Ulcers here are always deep.

MARTORELL'S ULCER (1945) It is seen in hypertensive patients often with atherosclerosis. It is seen in calf Otten it is bilateral and painful. Necrosis of calf skin occurs with sloughing away and formation of deep, punched out ulcers extending into the deep fascia. There is sudden obliteration of the arterioles of the calf skin. All peripheral pulses are present. ft takes months to heal. Treatment: Once ulcer granulates well , skin grafting with lumbar sympathectomy is done.

-

Fig. 1.64: Typical Martorell's ulcer.

ARTERIAL/ISCHAEMIC ULCER It is common in toes, feet or legs; often can occur in upper limb digits. It is due to poor blood supply following blockage of the digital or medium sized arteries.

Conflict is the primary engine of creativity and innovation.- Ranold He ifetz

24

...>a, ...:::,Cl

1/\

0

:::, C:

"' co cc:

1/\

Atherosclerosis and TAO (Thromboangiitis obliterans) are common causes of ischaemic ulcers in lower limb. Cervical rib, Raynaud's phenomenon and vasculitis are common causes in upper limb. Ulcer initially occurs after trauma, soon becomes nonhealing, spreading with scanty granulation tissue. Ulcer is very painful, tender and often hyperaesthetic. Digits may often be gangrenous. Intermittent claudication, rest pain are common. Other features of ischaemia are obvious in the adjacent areas. They are-pallor, dry skin, brittle nail, patchy ulcerations, and loss of hair. Ulcer is usually deep, destructs the deep fascia, exposing tendons, muscles and underlying bone. Dead tendons look pale/greenish with pus over it. Management: Specific investigations like arterial Doppler, angiogram, lipid profile, and blood sugar are done. Treatment is done accordingly-drugs like vasodilators; arterial surgeries may be needed.

Hard, discrete regional lymph nodes are often palpable, initially mobile but later become fixed. Lymph nodes can fungate eventually. Ulcer and lymph nodes are initially painless; but becomes painful and tender once there is deeper infiltration or secondary infection. Systemic spread is rare . It is a locoregional malignant disease . Verrucous carcinoma is exophytic, locally malignant welldifferentiated squamous cell carcinoma without lymphatic spread. Management: Wedge biopsy; FNAC of regional lymph nodes are the investigations. Treated with wide local excision with skin grafting and regional lymph node block dissection. Marjolin 's ulcer can occur in in unstable scar of long duration usually after burns or snake bite. it is well differentiated sec wh ich does not spread through lymphatics or blood (Refer Chapter 1 R-Skin Tumours).

Fig. 1.65: lschaemic ulcer foot with gangrene of toes without

evidence of any bleeding with exposed tendons. Fig. 1.66: Marjolin's ulcer in a chronic unstable scar (of long

BAIRNSDALE ULCER It is a chronic, irregular, undermined ulcer due to Mycobacterium ulcerans infection. Deep severe form, with extensive dermal necrosis is called as 'Buruli ulcer'. Discharge study will show acid-fast bacilli. Antituberculous drugs resolve the ulcer usually. Skin grafting may be required later.

CARCINOMATOUS ULCER (EPITHELIOMA, SQUAMOUS CELL CARCINOMA) It arises from prickle cell layer of skin. It may initially begin as a nodule or ulcer; but later forms an ulcerative lesion with rolled out/everted edge. Floor contains necrotic content, unhealthy (tumour) granulation tissue and blood . Ulcer bleeds on touch and is vascular and friable. lnduration is felt at the base and edge. It is usually circular or irregular in shape. Initially ulcer is mobile but becomes nonmobile once it infiltrates into deeper tissues. The typical foul smell is due to necrotic material, infection and release of polyamides from the tumour cells.

duration) in the leg. It does not spread through lymphatics.

RODENT ULCER It is ulcerative form of basal cell carcinoma which is common in face. Ulcer shows central area of dry scab with peripheral raised active and beaded (pearly white) edge. Often floor is pigmented. It erodes into deeper plane like soft tissues, cartilages and bones hence the name-rodent ulcer. As lymphatics are blocked early in the disease by large tumour cells, it does not spread to regional lymph nodes. Blood spread is absent. It is only locally malignant. It is common in face; rarely can it occur over tibia, external genitalia, mucocutaneous junction. It does not occur in mucosa. Management: Wedge biopsy, CT scan of the part to see the depth, wide excision.

MELANOTIC ULCER It is ulcerative form of melanoma. It can occur in skin as de nova or in a pre-existing mole. Ulcer is pigmented otten with a halo around.

Ulcer is rapidly growing, often with satellite nodules and 'in-transit lesions. It is very aggressive skin tumour arising from melanocytes. It spreads rapidly to regional lymph nodes which are pigmented. Blood spread to liver, lungs, brain, and bones is common. It can occur in mucosa, genitalia, and eye. It is a systemic malignant disease. Management: Excision biopsy (usually incision biopsy is not done), FNAC lymph node, US abdomen. Treatment is wide local excision, regional node block dissection, chemotherapy.

25

Note:

Diabetic neuropathy is due to conversion of sugar to sorbitol which causes nerve demyelination: neuropathy 1s distal, diffuse with a stocking distribution: it takes around 1Oyears to develop diabetic neuropathy. Glucose forms sorbitol by aldose reductase enzyme and galactose forms galactitol. If sorbitol level raises it gets trapped in peripheral nerves, retina and lens causing neuropathy, cataract and retinopathy. There may be formation of abnormal arteriovenous communications under the skin leading into nerve ischaemia and neuropathy. Sensory neuropathy causes loss of vibration sense, sense of position, touch, pain and temperature. Sensation may be absent in the entire sole due to medial and plantar nerve involvement. Foot feels dead, senseless with walking like in the sand and prone for minor repeated traumas which are not noticed by the patient. Motor neuropathy causes paralysis of intrinsic muscles of foot leading into claw toes and hammer toe. Longitudinal arch of the foot is lost and it becomes flat causing loss of foot curvature: loss of joint position and loss of foot muscle strength leading into altered gait and positions of foot which in turn lead into deep seated trophic ulcers over the heads of the metatarsals. Autonomic neuropathy causes absence of sweating and loss of skin elasticity making more prone for breakdown and infection. Autosympathectomy is common in diabetics involving foot area. Diabetic angiopathy may be macro-or micro-angiopthy (thickening of the basement membrane of vessels and capillaries). Sepsis in diabetes: Cellulitis, deep seated abscess, ulcer formation , gangrene foot, osteomyelitis of metatarsals, septicaemia, multiorgan dysfunction syndromecan occur faster in diabetes. Phagocytic activity in diabetes is reduced significantly; granulocyte mobilization is reduced in diabetic ketoacidosis. Neuropathy, angiopathy, high tissue glucose level, associated smoking, hypertension, hyperlipidaemia, reduced immunity increases the chances of sepsis in diabetics. Polymicrobial and fungal infections are common.

Figs. 1.67A and B: Melanotic ulcer in the foot with enlarged inguinal lymph nodes.

DIABETIC ULCER Causes , Increased glucose in the tissue precipitates infection. , Diabetic microangiopathy which affects microcirculation. , Increased glycosylated haemoglobin decreases the oxygen dissociation. ,. Increased glycosylated tissue protein decreases the oxygen utilization. ,. Diabetic neuropathy involving all sensory, motor and autonomous components. ,. Associated atherosclerosis. Sites: Foot-plantar aspect-is the most common site; leg, upper limb, back, scrotum, perineum. ,. Diabetic ulcer may be associated with ischaemia. ,. Ulcer is usually spreading and deep. Investigations: Blood sugar both random and fasting. Urine ketone bodies; Discharge for culture and sensitivity; X-ray of the part to see osteomyelitis. Arterial Dopp ler of the limb; glycosylated haemoglobin esti mation.

Figs. 1.68: Foot is th e most common area for diabetic infective problems. It can cause abscess, ulcer, osteomyelitis, gangrene, septicaemia. Initially patient undergoes toe amputation but later eventually land with below knee or above knee amputation.

B

PROBLEMS WITH DIABETIC ULCER

Neuropathy, in foot- -0

m

::0

.....

OJ

C

n

t'D ....

When you cease to dream, you cease to live.

C. Sinus and Fistula

I Causes Fistula

@ HAFTE R OUTLINE • • •

Sinus Fistula Median Mental Sinus

• •

Sequestrum Preauricular Sinus

SINUS It is a blind track lined by granulation tissue leading from an epithelial surface into the surrounding tissues. Sinus means " hollow" or "a bay" (Latin).

1. Congenital: Branchial fistula; Trachea-oesophageal fistula; Congenital AV fistula; Umbilical fistula (patent vitellointestinal duct). 2. Acquired: , Traumatic: Following surgery-intestinal fistulas (biliary, pancreatic, faecal). Following instrumental delivery or difficult labour (vesicovaginal fistula, rectovaginal fistula, ureterovaginal fistula). , Inflammatory-intestinal actinomycosis, tuberculosis. , Malignancy-when the growth of one organ penetrates into the nearby organ (rectovesical fistulas as in carcinoma rectum, vesicouterine fistulas as in uterine cancer). External fistula Orocutaneous • Branchial fistula Thyroglossal fistula Enterocutaneous fistula Appendicular fistula

r• Sinus

Figs. 1.73A and B: (A) Sinus; (B) Fistula.

I Causes of Sinus 1. Congenital: Preauricular sinus. 2. Acquired: Actinomycosis, tuberculosis, pilonidal sinus, chronic osteomyelitis, median mental sinus.

Internal fistula • Tracheo-oesophageal fistula • Cholecystoduodenal fistula Colovesical fistula • Rectovesical fistula

I Clinical Features of Sinus/Fistula Discharge from the opening of sinus. No floor. Raised indurated edge, indurated base, nonmobile. Often sprouting granulation tissue over the sinus opening. Bone thickening in osteomyelitis. Surrounding skin may be erythematous in inflammatory; bluish in tuberculosis; excoriated in faecal fistula; pigmented in chronic sinuses/fistulas. Discharge typical of the cause will be evident which will be obvious after applying pressure over surrounding area. lnduration is afeature of all chronic fistulas excepttuberculosis. Thickening of the bone underneath on palpation if sinus is adherent to bone or if there is osteomyelitis. Enlargement of regional lymph nodes will be evident. Sinus may be single or multiple.

Fig. 1.74: Typical sinus in the thigh due to osteomyelitis of the greater trochanter.

FISTULA It is an abnormal communication between the lumen of one viscus to another or the body surface or between the vessels. Fistula means "flute " or "a pipe or tube."

Fig. 1.75: Multiple discharging sinus foot. It is commonly due to mycetoma (Madura foot). It could also be due to tuberculosis, chronic pyogenic osteomyelitis or malignancy.

B

-

CAUSES OF PERSISTENCE OF A SINUS OR FISTULA

A foreign body or necrotic tissue underneath, e.g. suture, sequestrum Insufficient or nondependent drainage Persistent obstruction in the lumen, e.g. in faecal fistula, biliary fistula (distal obstruction) Lack of rest, persistent infection Wall become lined with epithelium or endothelium Dense fibrosis prevents contraction and healing Specific infections: Tuberculosis, actinomycosis Presence of malignant disease, post-irradiation

31

-

.... ("'\

Fig. 1.79: Pilonidal sinus showing primary and secondary sinus.

Note: The most common cause of sinus in neck is tuberculosis. Commonly it is tuberculous lymphadenitis. It shows yellowish cheesy discharge with bluish margin. Usually tuberculous sinus/ulcer does not show any induration.

Vl

...C:

...

IQ 11)

':':

Vl

:::, C:

II>

DJ

:::,

Q.

Fig. 1.80: Fistula in ano both sides.

::!'!

..... II>

C:

Di"

Figs. 1.76A and B: Secondaries in neck causing discharging sinus. (A) and (8) in two different patients.

Fig. 1.77: Mandibular sinus. It is usually due to infected tooth causing osteomyelitis of mandible. It also could be due to tumour, trauma, actinomycosis and radiation. X-ray (orthopantomogram), study of discharge and biopsy are relevant investigations. Such sinus should be excised with extraction of the causative tooth.

Fig. 1.81: Postoperative gastrointestinal fistula. Note the skin excoriation. It can be controlled by using zinc oxide cream local application.

B Purulent-bacterial infection Gaseous-tuberculous Sulphur granules-actinomycosis Mucus-branchial fistula

Fig. 1.78: Discharging sinus in the neck due to tuberculosis of lymph nodes with a cold abscess underneath.

Saliva--parotid fistula Faeces-faecal fistula Bile-biliary, duodenal fistula Bone-osteomyelitis sinus Urine-urinary fistula

Note: • Streptococcal pus is wate,y with blood stain. • Staphylococcal pus is yellow and creamy. • Green or greenish blue pus s due to Pseudomonas aeruginosa infection. • Anchovy sauce pus is seen amoebic liver abscess. • Gas gangrene produces sickly sweet odour-decayed apple like. • E. coli pus is usually odourless. • Anaerobic bacteria and proteus vulgaris cause typical odour due to proteolysis. Bacteroids cause typical over ripe Camembert cheese like odour. • Faecal fistula causes foul smelling discharge with gas bubble in it. • Tuberculous sinus discharges caseating cheesy material.

When a man looses his health he begins to take care of it.- John Billings

32

I Investigations Fistulogram/sinusogram using ultrafluid lipiodol or water soluble iodine dye (Lipiodol is poppy seed oil containing 40% iodine). Discharge for C/S, AFB, cytology, staining. Biopsy from the edge for tuberculosis and malignancy. Chest X-ray; X-ray of the part; MRI (most reliable) of the part. ESR. CT sinusogram. Probing gently with care. Digital examination of the rectum and proctoscopy in fistula in ano.

I Treatment Treat the cause. Excision of sinus or fistulas. Always specimen should be sent for histology. Antibiotics, antitubercular drugs, rest, adequate drainage.

MEDIAN MENTAL SINUS It is a chronic infective acquired condition wherein there is infection of roots of one or both lower incisor teeth forming root abscess which eventually tracks down between two halves of lower jaw in the midline presenting as discharging sinus on the point of chin at midline.

Treatment: Antibiotics, after doing discharge study (C/S) started; Lay opening and excision of the sinus track with extraction of incisor tooth/teeth.

SEQUESTRUM (SEE TABLE) Sequestrum is dead bone in situ. It can be pyogenic, tubercular (feathery), Salmonella (granular), syphilitic (ivory), tubular and ring (in amputation stump) . It can be unformed-means separation between sequestrum and adjacent normal bone has not occurred or formed-means there is proper adequate separation between normal bone and sequestrum by forming granulation tissue. Radiologically formed sequestrum shows clear lucent area/zone of demarcation. Sequestrum is denser because of the absence of decalcification in the dead bone as there is no blood supply (dead bone is dense bone). Sequestrum should be formed prior to surgical intervention- sequestrectomy and saucerisation.

I Features Usually painless discharging sinus in the midline on the point of chin. Often incisor infection may be revealed (in many patients clinically tooth looks normal even though root is infected invariably). It is often mistaken for infected sebaceous cyst. Osteomyelitis of the mandible is the possible complication. Differential diagnosis: Infected sebaceous cyst; Tuberculous sinus; Osteomyelitis. Investigations: Dental X-ray is diagnostic (Plain X-ray mandible may not reveal the disease); Discharge study for C/S, cytology, AFB.

Sinus track

Figs. 1.83A and B: Diagram and X-ray showing osteomyelitis with sequestrum and sinus. Sequestrum is dead bone in situ.

T

Sinus opening

Fig. 1.82: Median mental sinus. Note the origin of the sinus from the rooVroots of the lower incisor/incisors.

Types of sequestrum

Tubular/diaphyseal

Pyogenic--1:ommon

Feathery

Tuberculosis (vertebra, ribs)

Granular

Salmonella

Ring/tubular

Amputation stump and at Steinmann pin area

Fine sandy

Viral osteomyelitis-rare

Coarse sandy

Tuberculosis

Button like

Small separated sequestrum and in radiation osteomyelitis

Black

In actinomycosis (fungal) and due to H2S

Kiss sequestrum

In peridiscal tu bercutosis

PREAURICULAR SINUS It is a congenital entity occurring due to imperfect fusion of the six tubercles which form ear cartilage. Sinus opening may be seen at the root of the helix or on the tragus. Track is quiet deep running backwards, slightly upwards towards the helix. It usually ends blindly. Outer opening of the sinus often closed causing formation of a cystic swelling (preauricular cyst) which contains fluid wh ich is often infected. Preauricular sinus in no instance will communicate with the external auditory meatus. Bursting of this swelling leads into formation of ulcer li ke lesion. It can be unilateral or bilateral. Occasionally mu ltiple sinuses are seen. Opening of the sinus occurs in a small triangular area in front of the ear at the level of the tragus. Scarring is common around the opening due to repeated infection.

I Features

Figs. 1.84A to C: Osteomyelitis patient with scar. Also showing on table photo of sequestrectomy and saucerisation.

Figs. 1.85A to C: X-ray pictures showing features of osteomyelitis with sequestrum, sinus, cavity of tibia and metatasral bones in diabetic patients.

It is seen since childhood. Often swelling appears and apparently disappears repeatedly. Pain and discharge is common. It causes a cosmetic problem in young individual. Discharge study, ESR, sinusogram to assess the track is needed. MR sinusogram is beneficial. Differential diagnosis: Cold abscess, sebaceous cyst. Investigation: Sinusogram, discharge study. Treatment: Excision under general anaesthesia with removal of entire track is essential. If track is not removed properly recurrence will occur.

Figs. 1.86A and B: Preauricular sinus. During excision, methylene blue is injected into the track initially and later it is excised using elliptical incision.

The drops of rain make a hole in the stone not by violence, but by oft falling.

33

....

t""I C\ /1)

:::,

.... QI

/1)

V,

C: ....

IQ

/1) ....

':":

V,

:::, C:

V,

QI

:::,

Cl. 'Tl

;:;;· ,-+

C:

DJ

D. Infectious Diseases

(9HAPTER OUTLINE

•

--• •

• •

• •

... • •

•

Surgical Infection Cellulitis Erysipelas Lymphangitis Abscess Metastatic and Pyaemic Abscess Bacteraemia Septicaemia Pyaemia Boil (Furuncle) Hidradenitis Suppurativa Carbuncle Pott's Puffy Tumour Pyogenic Granuloma (Granuloma Pyogenicum) Impetigo

-•

Erythrasma Scrum Pox Tetanus Gas Gangrene Tuberculosis Leprosy Syphilis (Great Pox) Actinomycosis Madura Foot (Mycetoma Ped is) Rabies (Hydrophobia) Anthrax Nosocomial and Opportunistic Infections Necrotising Fasciitis Acute Pyomyositis Surgical Site Infection HIV Infection and AIDS

-• • •

-•

-

• •

SURGICAL INFECTION Surgical infection is a major problem in surgical practice. Asepsis (prevention of entry of organisms) and antisepsis (killing of the bacteria in the skin or tissues) has made a difference in surgical practice. Epithelial surfaces act as mechanical barrier and phagocytes, antibodies; complements, macrophages, leukocytes, opsonins, etc. act as protective mechanisms. Malnutrition, diabetes mellitus, obesity, uraemia, jaundice, malignancy, immunosuppression, radiotherapy, chemotherapy, HIV, ischaemia, foreign body, haematoma are the risk factors for surgical infections. Virulence of organisms, blood supply, body immunity and support of antibiotics are the decisive factors in proper response to control infection.

• Grade 0 is normal healing Grade 1 is with bruising/mild erythema Grade 2 is severe erythema with other features of inflammation at or around wound Grade 3 is serous or bloody discharge Grade 4 is presence of pus or deep infection or tissue breakdown or significant haematoma.

ASEPSIS wound score system is used to assess the wound infection.

T

ASEPSIS wound scoring

Parameters • Additional treatment 1. Antibiotics 2. Pus drainage 3. Wound debridement • Serous discharge-for 5 days of first 7 days of wound infection • Erythema-for 5 days of first 7 days of wound infection • Purulent fluid-for 5 days of first 7 days of wound infection • Separation of deep tissues-for 5 days of first 7 days of wound infection • Isolation of bacteria • Stay in the hospital (in-patient) more than 14 days due to infection

Score 10 05 10 0-5 daily

0-5 daily 0-1 0 daily 0-1 0 daily 10 05

Fig. 1.87: Severe sepsis with necrosis in the limb after trauma.

Surgical infection can be superficial surgical site infection in

the wound or deep surgical site infection in deeper fasciomuscular layers or organ space infection like abdomen/ thoracic cavity, etc. Health care associated infection occurs after hospital admission in intensive uniVpostoperative ward, etc.

CELLULITIS It is spreading inflammation of subcutaneous tissue and fascia/ planes. Infection may follow a small scratch or wound or incision or insecVsnake/scorpion bite.

It can be superficial or deep. More common superficial type is easier to diagnose. It is common in diabetics, immunosuppressed people and old age. It is common in face, lower limb, upper limb and scrotum wherein subcutaneous tissue is lax. Note: Cellulitis occurring in children is never primary but secondary to an underlying bone infection-Morison's aphorism (James Morison, 1939Surgeon, Durham, Newcastle).

Diabetes to be treated with insulin. Ketosis if present shou ld be confirmed by assessing urine ketone bodies and treated subsequently with intravenous insulin. Often patient may be in septicaemia; patient in such condition should be treated with higher antibiotics, critical care with fluid management, along with maintaining adequate urine output. Catheterization is required; monitoring is done with-renal function tests, haematocrit (platelet count), liver function tests, prothrombin time and serum electrolyte estimation.

B '

""

:r: )>

-0

--f

m

....,:, 0

C) It)

:::,

... It)

Commonly due to Streptococcus pyogenes and other Gram +ve organisms. Release of streptokinase and hyaluronidase cause spread of infection. Often Gram -ve organisms like Klebsiella, Pseudomonas, E. coli e also involved (usually Gram -ve organisms cause secondary ection).

B

35

0J

VI

...C: I.C ... -:-; It)

:::,

it I'\

...o·

-

SEQUELAE

Infection can get localised to form pyogenic abscess Infection can spread to cause bacteraemia, septicaemia, pyaemia • Often infection can lead to local gangrene Extensive necrosis of skin and subcutaneous tissue-necrotizing fasclilis.

C:

Ill

0

Fig. 1.88: Cellulitis face. Note the oedema of the face and eyelids.

It)

0J

Ill It) Ill

I Clinical Features Fever, toxicity (tachycardia, hypotension). Swelling is diffuse and spreading in nature. Pain and tenderness, red, shiny area with stretched warm skin. Cellulitis will progress rapidly in diabetic and immunosuppressed individuals. Tender regional lymph nodes may be palpable which sign ify severity of the infection. No edge; no pus; no fluctuation; no limit.

I Investigations Total co unt rai ses, differential count , platelet count (decreases) to be done. Liver function tests, blood urea and serum creatinine in severe cases. Blood sugar estimation, urine test for ketone bodies, glycosylated haemoglobin estimation. Deep vein thrombosis (DVT) often may mimic cellulitis of lower limb. Venous Doppler and ultrasound of soft tissues of the limb may require in such situation.

I Treatment Elevation of limb or part to reduce oedema so as to increase the circulation and bandaging. Antibiotics-penicillins, cephalosporins. Dressing (often glycerine dressing is used as it reduces the oedema because of its hygroscopic action glycerine magnesium sulphate dressing).

;;;·

Figs. 1.89A to D: Note the cellulitis in different patients. It is common in lower limbs. There is no edge without any formed pus. It should never be incised with certain exceptions like Ludwig's angina. It is treated by antibiotics. It can cause bacteraemia/septicaemia.

Oedema gives rise to soft pitting, while if pus present, induration can always be felt. -Allan B Kanavel

I Cellulitis in Special Areas

VI

co

a: Vl

Commonest organisms are-Streptococcus viridians, Staphylococcus aureus and anaerobes. Gram-negative organisms Orbital Ce/lulitis can also be involved. Cellulitis may extend into the pharyngomaxil lary space, Cellulitis in orbit causes proptosis, leading to impairment of retropharynx, and superior mediastinum. ocular movements and blindness. Diffuse painful swelling with woody brawny induration of the It can spread through ophthalmic veins into cavernous sinus mouth and anterior neck is seen. Swelling is non-fluctuant causing cavernous sinus thrombosis. It requires admission and immediate aggressive treatment but with redness and tenderness. Bilateral submandibular with higher generation antibiotics (Penicillins, cephalooedema with marked tenderness on palpation at suprahyoid sporins). area with bull's neck appearance. Toxic features like fever, tachycardia, tachypnoea is common. Difficulty in speech, earache, drooling of saliva and putrid halitosis. Involvement of connective tissues, muscles and tascial spaces but not glandular structures. Spread via fascia! planes in continuity not by lymphatics; no lymph node enlargement. Oedema of the tongue with pushing against palate (elevation) upwards and backwards causing airway obstruction, dysphagia and odynophagia. Fig. 1.90: Dangerous area of face-area of upper lip and lower part of nose. Infection from this area spreads through deep facial vein Strider, respiratory distress and cyanosis may develop due pterygoid plexus communicating vein cavernous sinus causing to oedema of tongue and larynx. its life-threatening thrombosis. Investigations-CT scan or MRI is useful to identify airway block, fluid collection and presence of gas. Ultrasound neck is simpler method to identify same. Total count, blood sugar, chest X-ray and often blood gas analysis (in severe cases) is done. Differential diagnoses are-angioneurotic oedema, sublingual haematoma, sialadenitis, lymphadenitis. •·· Complications ,. Laryngeal oedema can occur due to spread of inflammation to glottis submucosa via stylohyoid tunnel. It may require emergency tracheostomy to maintain the respiration. , Mediastinitis due to spread of infection into mediastinum; aspiration pneumonia. , Septicaemia. , Spread of infection into the parapharyngeal space leads to thrombosis of the internal jugular vein which may extend above into the sigmoid sinus which may be fatal. , Mortality is less than 5% unlike in olden days. Fig. 1.91 : Severe cellulitis face involving nose, eyelids and facial skin. Treatment: It can cause septicaemia, cavernous sinus thrombosis or intracranial , Antibiotics (intravenous) like penicillins, piperazillin, tazospread of infection. This area is called as dangerous zone in face. It bactam , clindamycin, metronidazole should be started at needs hospitalisation for treatment. the earliest. , If patient is in respiratory distress, tracheostomy is Ludwig's Angina (Wilhelm Frederick von Ludwig in required as a life saving procedure. 1836) , Whenever distress is severe surgical decompression It is a rapidly progressive polymicrobial cellulitis of the is required. Submental horizontal incision is placed; sublingual and submandibular spaces involving the floor of mylohyoid muscles are cut (both sides); wound may be the mouth and suprahyoid area on both sides of the neck. closed with a loose sutures with a drain or kept open with Commonest cause is dental infection of 2nd or 3rd molar teeth a dressing cover until oedema and sepsis subsides and precipitated by tooth extraction: other causes are submanlater secondary suturing can be done. dibular sialadenitis, trauma, pe ritonsillar abscess, upper , Initial steroid therapy (dexamethasone) may be beneficial respiratory infection, interventions like endotracheal intubation. and is often used to reduce oedema even though it is Predisposing factors are- diabetes mellitus, chemotherapy, controversial. oral cancer, alcohol, neutropenia.

ERYSIPELAS

LYMPHANGITIS

Erysipelas is (Greek-red skin, lgnis sacer, holy fire, St Anthony fire) an acute spreading inflammation of the upper (outer) dermis and superficial lymphatics; it has got typical skin rash presenting on legs, toes, face and fingers due to acute infection by beta haemolytic Streptococcus pyogenes, presenting as raised well demarcated skin rash (rash is due to exotoxin). It is more superficial than cellulitis. In olden days, it was more common in face, now it is common in legs. Infection occurs through a minor trauma. It affects all races; more common in females. There will be always cutaneous lymphangitis with development of rose pink rash with cutaneous lymphatic oedema. Vesicles which form eventually will rupture to cause serous discharge. Sites: Orbit, face and ear lobule-most common ; Hands and scrotum; Umbilicus in infants; Decubitus ulcer of lower limb (legs and feet are now becoming more common site).

I Features Toxaemia is always a feature. Rash is fast spreading and blanches on pressure. It is raised with sharp margin. Redness becomes brown and later yellow with vesicles. Discharge is serous {In cellulitis discharge is purulent). In the face and orbit it causes severe oedema. Milian 'sear sign is aclinical sign used to differentiate erysipelas from cellulitis wherein ear lobule is spared. Skin of ear lobule is adherent to the subcutaneous tissue and so cellulitis cannot occur. Erysipelas being a cutaneous condition can spread into the ear lobule (Gaston Milian-dermatologist Paris, 1945). Tender, regional lymph nodes are usually palpable. Differential diagnoses-herpes zoster, angioneu rotic oedema, contact dermatitis. Complications ,. Septicaemia, localized cutaneous and subcutaneous gangrene are dangerous problems. ,. Abscess, pneumonia, meningitis may develop. , Lymphoedema of face or eyelid or limbs (when involved) can occur due to lymphatic fibrosis. ,. Glomerulonephritis (not rheumatic fever), septic arthritis, necrotizing fasciitis, can occur occasionally. ,. Recurrence rate is 20%. It causes disfiguring sequelae. Treatment: Antibiotics like penicillins, clindamycin, erythromycin, roxithromycin given. Recurrent erysipelas may require injection benzathine penicillin (long-term penicillin) monthly for 2 years.

B

It is an acute nonsuppurativeinfection and spreading inflammation of lymphatics of skin and subcutaneous tissues due to beta haemolytic streptococci, staphylococci, clostridial organisms. It is commonly associated with cellulitis. Erysipelas is a type of lymphangitis. In endemic areas, filariasis is the most common cause (coastal India). It is caused by Wuchereria bancrofti. It is transmitted through bites of Culex mosquito. Microfilaria reaches the lymph node forming adult worm which blocks the lymph node causing obstruction, fibrosis and lymphangitis. Usually lymphangitis due to bacterial infection occurs following a small trauma. Rapidly affected area develops warmness and redness.

I Features Streaky redness which is spreading is typical. On pressure area blanches, on release redness reappears. Oedema of the part, palpable tender regional lymph nodes are obvious. Fever, tachycardia, features of toxaemia. Groin lymph nodes are enlarged and tender in lower limb lymphangitis. In upper limb, as lymphatics are mainly located on the dorsum of hand, oedema and redness develops on the dorsum. Infection in thumb and index finger causes palpable tender axillary nodes; in little and ring finger causes first tender palpable epitrochlear nodes to appear; infection in middle finger causes first deltopectoral nodes to enlarge. Regional lymph nodes (only) may eventually suppurate to form an abscess. Toxaemia, septicaemia may occur. Rapidity may be more in diabetics and immunosuppressed. ••• Chronic lymphangitis occurs dueto repeated attacks of acute recurrent lymphangitis leading into acquired lymphoedema.

ERYSIPELOID DISEASE

Also called as 'Fish handler's disease Occurs following any cuts or scratches • It has features of both erysipelas and cellulitis L!. It is self-limiting with relatively mild symptoms.

,

Figs. 1.92: Typical ear lobule infection after ear prick for ear stud.

Hope puts our feet on the path when our eyes cannot see it.

37

Cl

11)

::,

...

11)

QI

Vl

... '°... C:

11)

::,

iir' n ,+

o· C: VI

9.

VI 11)

QI VI 11) VI

38

Protein exudation causes fibrin deposition and formation of pyogenic membrane. Macrophages and polymorphs release lysosomal enzymes which cause liquefaction of tissues lead ing into pus formation. Toxins and enzymes released causes tissue destruction and pus formation.

Typical redness

!

""C

c. For drainage, dependent incision is used

c. Nondependent incision is used d. Wound is sutured

d. Suturing of the wound is not done e. Drain is placed

e. Drain is not placed (otherwise sinus will form which is difficult to treat)

m

....c:,

:::0

Cl

Procedure

It)

:::,

... It)

Hilton's method of draining an abscess. Initially broad-spectrum antibiotics are started (depending on severity, extent and site of the abscess). Under general anaesthesia or regional block anaesthesia,* after cleaning and draping, abscess is aspirated and presence of pus is confirmed . Skin is incised adequately, in the line parallel to the neurovascular bundle in the most dependent position. Next, pyogenic membrane is opened using Sinus forceps** and all loculi are broken up. Abscess cavity is cleared of pus and washed with saline. A drain (either gauze drain or corru gated rubber drain) is placed. Wound is not closed. Wound is allowed to granulate and heal. Pus is sent for culture and sensitivity. Biopsy should be done in suspected tuberculosis or malignancy. Sometimes secondary suturing or skin grafting is required. Antibiotics are continued. Treating the cause is im portant.

Fig. 1.103: Scrotal abscess, which is well-localised and ready for

drainage. Patient has undergone surgery for hernia earlier.

Ill

VI

... \0 C:

It)

:::,

Fig. 1.104: Abdominal wall abscess being aspirated. Note the aspirated pus in the syringe. Abscess should be aspirated prior to drainage.

it n ..+

o· C:

V,

0

Note: • Counter-incision is placed in breast abscess which is placed in upper

quadrant. Incision should be deeper while draining pus in radial and ulnar bursae, palmar spaces and tenosynovitis. • Problems in drainage: Improper drainage, bleeding, residual abscess or sinus formation. •

Figs . 1.105A and B

• As the pus is acidic local anaesthetic agent will not act and hence it is not used. •• Sinus forceps do not have lock and has got serrations in the tip. It is called as sinus forceps because it was initially designed and used to pack sinuses.

Joseph Lister is father of modern surgery and started antiseptic surgery in 1912.

V,

It)

0,1

V,

It)

V,

42

...>...::, a, O'I

VI

0

IO

::, C: IO

E1

co a: Ill

I]) Abscess

Loculi

Figs. 1.106A and B: (A)lncision for draining an abscess by Hilton's method. Note the longitudinal incision; (8) Abscess with loculi and breaking of loculi using finger for drainage.

VI

Fig. 1.107: Different instruments requ ired for incision and drainage of an abscess.

METASTATIC ANO PYAEMIC ABSCESS

Figs. 1.105C to F

Figs. 1.105A to F: Technique of incision and drainage of a pyogenic abscess. Abscess should be aspirated first to get pus; No. 11 blade is used to incise; using sinus forceps pyogenic membrane is opened; pus is collected for culture; loculi are broken using sinus forceps and little finger; cavity is irrigated with normal saline (ideal); cavity is packed with roller gauze; wound is not sutured.

Metastatic abscess It is an abscess which occurs as a spread from other abscess. For example, lung abscess causing metastatic abscess in the brain (common example). Presentation here is of features of focus abscess and of metastatic abscess (localised features). Pyaemic abscess It is from any infective focus which need not be always from an abscess (from cellulitis or skin infections, etc.) causing pyaemic emboli leading into multiple abscess in different places like brain, kidneys, liver, etc. Presentation here, is mainly of systemic features involving multiple organs with toxicity. These emboli contain bulk of multiplying organisms often derived from infective thrombus or vegetations. Focus may be an abscess, cellulitis, skin infection, acute osteomyelitis, and acute bacterial endocarditis. Acute appendicitis with severe sepsis can cause infective pyaemia in liver called as pyelphlebitis or portal pyaemia. Pyaemic abscess are multiple, deeper, beneath the fascia or in the internal organs. When it is on the surface, it is less tender without any clear signs of inflammation.

Pyaemic abscess carries high mortality with SIRS and MODS. Management: ,.. Evaluation for focus of infection, pus for culture, blood culture (three samples), antibiotics, critical care, systemic therapy, drainage of surface abscesses.

BACTERAEMIA

pyrogenic response; eventual irreversible cold stage develops wherein patient goes for complications like-ARDS; renal, liver and multiorgan failure; disseminated intravascular coagulation (DIC); bone marrow suppression (thrombocytopenia) . Evaluation of septicaemia is done using clinical assessment; blood parameters (haematocrit, liver and renal function tests, coagulation profile, electrolyte estimation, arterial blood gas analysis, C-reactive protein); culture of urine/pus/discharge/bile/ blood; chest X-ray; imaging as per need.

It is the presence of bacteria (live) in the blood circulation (sepsis is the host response to bacteria). It may be primary wherein bacteria are introduced directly into the blood by drug abuse injections, venous catheters or secondary wherein bacteria Treatment: Fluid therapy, antibiotics, monitoring {heart rate, enter blood through some other infection focus like pneumonia, respiration, oxygen saturation, urine output (may need to pass urinary infection, etc. Bacteraemia may be transient or intermit- Foley's catheter), oxygen supplementation, fresh frozen plasma, or tent or persistent. In transient type, bacteria is present in the fresh blood transfusion, critical care with ventilator support, eleccircu lation for only minutes to few hours and gets cleared like trolyte management. CVP line, parenteral nutrition, management in small procedures and instrumentation. In intermittent type, complications (like haemodialysis for renal failure, tracheostomy). periodic bacteraemia occur-seen in pneumonia or abscess, COMPLICATIONS OF SEPTICAEMIA etc. Persistent type is continuous presence of bacteria in blood often seen in infected heart valve or central line or vessel graft Disseminated intravascular coagulation (DIC) or prosthesis etc. Bacteraemia can be Gram positive or Gram ARDS negative. It is more often seen in meningitis, typhoid, brucelLiver dysfunction Renal failure losis, etc. Risk factors are HIV, diabetes, transplant, dialysis and Bone marrow suppression- thrombocytopaenia immunosuppression. Two blood cultures taken from two sepaMultiorgan failure rate sites of the body if show same bacteria it is confirmed as bacteraemia. Condition is treated by effective antibiotic therapy.

B

SEPTICAEMIA

PYAEMIA

Presence of multiplying bacteria in blood as emboli which spread and lodge in different organs in the body like liver, lungs, kidneys, spleen, brain causing pyaemic abscess. This may lead to multiorgan dysfunction syndrome (MODS) . It may endanger life if not treated properly.

It is the presence of overwhelming and multiplying bacteria in the blood with toxins causing SIRS (systemic inflammatory response syndrome) or MODS (multiorgan dysfunction syndrome) which later may progress into multiple system organ failure (MSOF). Actually, sepsis which means body's response to infection evenClinical features ,.. Fever with chills and rigors, Jaundice, oliguria, drowsiness tually causing damage to its own organs. Sepsis is SIRS with ,. Hypotension, peripheral circulatory collapse and later infection. Severe sepsis is sepsis syndrome with MODS or MSOF. coma with MODS Even though two or more of SIRS criteria (Refer Chapter 1GShock) are used to diagnose sepsis, currently other scoring systems Note: Causes, evaluation and treatment of pyaemia is like septicaemia are used (2016). SOFA score [Sequential (sepsis related) organ above. failure assessment] is ideal using six parameters respiratory, neurological, cardiovascular, liver, coagulation and renal systems; each BOIL (Furuncle) having 0,1,2,3,4 scores. Quick SOFA score (qSOFA score) is also It is an acute staphylococcal infection of a hair fol licle with used based on 3 parameters-low blood pressure 22/minute; altered mentation--GCS

""C

-I

m

::JJ

..... 0

44

Boil commonly subsides spontaneously often with the support of suitable antibiotics; occasionally it requires incision and drainage. Regional enlarged tender lymph nodes may be palpable due to secondary infection. Systemic features are not common unless it is multiple/ recurrent/severe or in diabetics and immunosuppressed. Multiple/recurrent boils are common in diabetics.

Stage I: Abscess formation, single or multiple, without sinus tracts or scarring. Stage II: Single or multiple, widely separated, recurrent abscesses with sinus tract formation or scarring. Stage Ill: Diffuse or near diffuse involvement or multiple interconnected sinus tracts and abscesses across the entire area. Note: The Sartorius Hidradenitis Suppurative Scoring is made by counting involved regions, nodules and sinus tracts. __

I

Fig. 1.108: Furuncle/boil are infection of hair follicle with perifolliculitis due to Staphylococcus aureus.

Treatment , Antibiotics given if boil is not resolving spontaneouslycloxaci//in/amoxycillin. ,, Rarely drainage of boil is needed in severe persistent form. Complications , Cellulitis, lymphadenitis. , Hidradenitis (Infection of group of hair follicles). ,. Boil in dangerous zone in the face, can cause cavernous sinus thrombosis.

HIDRADENITIS SUPPURATIVA It is a, chronic scarring inflammatory acne like disease of the skin bearing apocrine sweat glands. Apocrine sweat glands are coiled glands which open into the hair follicles. Hidradenitis suppurativa is also known as 'acne inversa'. It occurs commonly in women of menstruating age group; smoking is the important aetiological factor. It may be associated with acne, pilonidal sinus or Crohn's disease. Aetiology , Smoking, obesity, poor hygiene, polycystic ovarian disease. ,. Diabetes mellitus, steroid therapy. , Genetic causes: It is associated with chromosome 15q24q25. Multiple members of the family may be affected. It may be associated with pyoderma gangrenosum, acne (PASH syndrome) or/and pyogenic arthritis (PAPASHsyndrome). Note:

• Lesion is usually sterile; bacterial infection supervenes in deep abscesses and sin us tracks. Staphylococcus aureus and Propionibacterium acnes are the common bacteria. • Sites of apocrine sweat glands: Axilla, groin, areola, umbilicus, scalp, chest and perineum. • Hidradenitis suppurativa in the anal region rarely my turn into squamous cell carcinoma.

Figs. 1.109A and B: Hidradenitis suppurativa. It is chronic infection of apocrine sweat glands of the skin. It is common in axilla (Courtesy Dr Achaleshwar Dayal, MS, !tarsi, MP).

Pathogenesis Obstruction of duct of apocrine sweat gland by keratin

.1.

Dilatation of the duct of gland

.1.

Infection and abscess formation

.1.

Involvement of subcutaneous tissue and adjacent apocrine glands

.1.

Fibrosis, scarring, sinus formation

.1.

Spread to surrounding tissues

•·· Features , Common in females 4 : 1. , The most common site is axilla. Often it is bilateral. , Multiple discharging sinuses, with nodules in the skin which is tender. , lnduration due to fibrosis.

Investigation: Discharge study-culture/sensitivity and AFB.; Biopsy to rule out tuberculosis or malignancy. Differential diagnosis: ,. Tuberculous sinus. ,. Malignancy (squamous cell carcinoma of skin). ,., Lymph node mass in the region which are in deeper plane. Treatment ,., Topical clindamycin 1%; topical resorcinol 15% cream. ,., Oral antibiotics like clindamycin, erythromycin, doxycycline-needs longer course. ,,. lnfliximab, retinoids (isotretinoin 0.25-0.4 mg/kg) may be used in severe cases. ,., Wide excision (radical excision) of the involved area with skin grafting. ,., Antiandrogen therapy in females using oral estrogen or oral contraceptives or spironolactone or cyproterone acetate or finasteride. ,., Ablative laser (CO2 or erbium YAG) therapy is an alternate therapy often used.

CARBUNCLE (Word meaning of carbuncle is charcoal) It is an infective gangrene of skin and subcutaneous tissue. Staphylococcus aureus is the main culprit. Common site of occurrence is nape of the neck and back. Skin in this area is thick. Condition also can occur in shoulder, cheek, hand, forearm. It is common in diabetics and after forty years of age. It is common in males. Infection

t

Development of small vesicles

t

Sieve like pattern

t

Red indurated skin with discharging pus

t

Many fuse together to form a central necrotic ulcer (ash-grey slough) with peripheral fresh vesicle looking like a "rosette" (cribriform)

t

Skin becomes black due to blockage of cutaneous vessels

t

Disease spreads to adjacent skin rapidly

45

0

Fig. 1.110: Carbuncle in the nape of the neck-typical site. Note the wide area of involvement and dark area-charcoal like. Ash-grey slough is specific.

POTT'S PUFFY TUMOUR It is a misnomer. It is not a tumour. It is formation of diffuse external swelling in the scalp due to subperiosteal pus formation (abscess) and scalp oedema. It originates commonly in frontal region and may extend into other regions. There is acute osteomyelitis of frontal bone. Causes , Chronic frontal sinusitis which eventually suppurates and extends into subperiosteal region. ,. Trauma-subperiosteal haematoma. ,. Chronic suppurative otitis media.

I Features Pain and boggy swelling in frontal region which is warm, tender. Toxicity and drowsiness. Pitting scalp oedema is typical. Investigations: , Total leucocyte count-increased. ESR-raised. ,,. X-ray skull. CT scan. Differential diagnosis: Secondaries in the skull or brain.

Patient is toxic and in diabetics they are ketotic In carbuncle, group of hair follicles are involved. Carbuncle is cluster of furuncles connected subcutaneously, causing deeper suppuration and scarring. Investigations: Urine sugar and urine ketone bodies; Blood sugar; Discharge for C/S. Treatment ,,. Control of diabetes is essential using insu lin. ,., Antibiotics like penicillins, cephalosporins or depending on C/S is given. ,., Drainage is done by a cruciate incision and debridement of all dead tissues is done. Excision is done later. ,,. Once wound granulates well, skin grafting may be required. Note: Renal Carbuncle is an entitywhich occursinkidney due to infection,

forming localized infective mass lesion.

Fig. 1.111: Pott's Puffy tumour. Note the swelling over the frontal

region and eyelid.

Age or youth is not a matter of chronology. we are as young or old as we teel-Sarvapalli Radhakrishnan

46

>,_

"'O

11)

Note:

I Organism Clostridium tetani is a Gram-positive, anaerobic, motile, noncapsulated, organism with peritrichous flagella, and terminal spores (Drum stick/tennis racket appearance). Spore is the infective agent. They are found in soil, manure, dust. Spore can gain entry through any wound, pricks, injuries resulting from road traffic accidents, penetrating injuries.

n

::c

I Toxins

TETANUS

Absence of prior tetanus toxoid immunisation. Trauma with lacerations, deep wounds, crush devitalised wounds, presence of foreign body, wounds with anaerobic environment in the tissues. Chronic suppurative otitis media with perforation, caries teeth. Improper sterilisation in the ward , labour (septic abortion) and operation theatre. Tattooing , rusted nails (there is a myth that only rusted instrument will cause tetanus; this is not true), ear lobe prick, colloquial perianal therapies,

Aggravates the muscle spasm

47

PERIOD OF ONSET

Time between appearance of first symptom and appearance of first sign • Shorter the incubation period of onset the prognosis and vice versa. If it is less than 2 days it is commonly fatal

Even if you're on the right track, you'll get runover if you just sit there.-Arthur Godfrey

"' 11)

"'

I Signs ...>. QI

...C'l :,

V'I

0

IU

:, C: IU

cc a:

VI

V'I

Trismus, due to spasm of masseter and pterygoids. Risus sardonicus (smiling facies), a rigid smile due to spasm of the facial muscle-zygomaticus major. Looks as if patient is smiling. Neck rigidity; spasm and rigidity of all muscles; hyperreflexia. Respiratory changes-due to laryngeal muscle spasm, infection, aspiration. Tonic clonic convulsions. Abdominal wall rigidity often with haematoma formation. Severe convulsion may often lead to fractures, joint dislocations and tendon ruptures. Fever and tachycardia. Retention of urine (due to spasm of urinary sphincter), constipation (due to rectal spasm). Rarely features of carditis are seen due to involvement of the cardiac muscle, which is dangerous, as it often leads to cardiac arrest and death. Here steroids are very useful. It presents with refractory bradycardia. Symptoms will be aggravated by stimuli like light, noise. Diaphragm and other muscles of respiration undergo spasm causing tachypnoea, respiratory distress, respiratory infections, aspiration, cyanosis, respiratory failure with altered P02 and PC02 levels.

Urban tetanus. Due to repeated injections in IV drug abusers. Postoperative tetanus due to improper sterilisation. Even though it is classified as acute tetanus (tetanus develops within 10 days) or chronic tetanus (tetanus develops from 10 days to 3 months) it is only of outcome value as therapy will be same in both . Post-abortion or puerperal tetanus develops due to practice of improper sterilisation during abortion or delivery. If patient is not given tetanus toxoid during the first attack, he can develop second attack of tetanus at a later period called as recurrent tetanus as patient who had once tetanus is not immune for development of second attack of tetanus. In children and adolescents chronic suppurative otitis media (CSOM) with perforation of tympanic membrane can cause tetanus-otitis tetanus.

Opisthotonus: Posterior muscles are acting more, so backward bending. Orthotonus: Straight posture. Both front and back muscles are acting equally. Emprosthotonus: Forward bending as front muscles are acting more. Pleurothotonus: Lateral bending as lateral muscles act more.

I Types of Tetanus

'

Early tetanus: It is a severe form with a short incubation period and poor prognosis. Latent tetanus: Wound is healed and forgotten. After a long incubation period , may be years later, under favourable environment, spores release bacteria and cause tetanus. It carries better prognosis. Latent tetanus is often called as delayed tetanus. Late tetanus: Disease develops many months after injury. Ascending tetanus: Symptoms and signs progress from below upwards. Descending tetanus: Symptoms and signs progress from above downwards. Cephalic tetanus: Facial muscles are involved first (3rd, 4th, 6th and 7th cranial nerves can get involved). Facial nerve is commonly involved in th is type. Oculomotor nerve-3rd nerve (ophthalmoplegia), hypoglossal nerve-12th nerve (spasm of tongue) are other cranial nerves involved. Localised tetanus: Here muscles adjacent to the wound or muscles of one segment or one area develop spasm. It is due to less virulent toxin or released toxins are of less concentration or only one or few segments of anterior horn cells of the spinal cord are affected. Bu/bar tetanus: When muscles of deglutition and respiration are involved. Highly fatal. Tetanus neonatorum (7th day tetanus): Tetanus occurring in neonates. Spread is through umbilical cord. It carries very high, nearly 100% mortality.

·-

----.\

Generalized-commonest; Localized-less severe Cephalic-facial nerve muscles; Neonatal tetanus

Opisthotonus (backward)

------

l Orthotonus (straight}

..........

-............ ______ ........-...... I

,,..'

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

Emprosthotonus (forward)

''

( I

Pleurosthotonus (lateral bend)

Fig. 1.114: Different types of postures seen in tetanus.

B Fracture bones; haematoma Aspiration pneumonia, respiratory failure, ARDS Carditis, arrhythmias-life-threatening DVT, pulmonary embolism Toxaemia; secondary infection-septicaemia Bedsore, malnutrition, stress gastric ulcers Mortality used to be very high in earlier days up to or more than 50%. It is reduced to 15-20% at present; but it is still higher in children and elderly Repeated uncontrollable convulsions can lead into coma and death.

I

B Mildly ill: Rigidity, spasm, trismus and different postures. Seriously ill: Spasm, rigidity, severe respiratory infections, dysphagia. Dangerously ill: Cyanosis with respiratory failure and tonic-clonic convulsions, cyanosis.

j

• I Mild: Mild to moderate trismus; general spasticity; no respiratory embarrassment; no spasms; little or no dysphagia. • II Moderate: Moderate trismus; well-marked rigidity; mild to moderate but short spasms; moderate respiratory embarrassment with an increased respiratory rate greater than 30; mild dysphagia. • 111Severe: Severe trismus; generalised spasticity; reflex prolonged spasms; increased respiratory rate greater than 40; apnoeic spells; severe dysphagia; tachycardia greater than 120. • IV Very severe: Grade Ill and violent autonomic disturbances involving the cardiovascular system. Severe hypertension and tachycardia alternating. Note: Several other severity grading systems are adapted like of Philips, Dakar, Udwadia, Black, Patel and Jong method.

B

DIFFERENTIAL DIAGNOSIS

• Strychnine poisoning (here patient is normal in between): Strychnine blocks neural synapse • Trismus due to other causes like-dental, oral, tonsillar sepsis, oral malignancy, temporomandibular joint dysfunction • Meningitis • Hydrophobia • Convulsive disorders • Metabolic cause like hypocalcaemia and hypomagnesaemia and hypoglycaemia • Drug induced extrapyramidal reaction by metoclopramide, phenothiazines

I Management of the Tetanus Isolation Isolation is advised in a dark quiet room to have afaster recovery; ventilator and all supportive critical care facilities should be available. Tetanus per se being not a communicable disease does not essentially require isolation.

Eliminating the Source of the Toxin Clostridium tetani are sensitive to penicillins, metronidazole, clindamycin, and erythromycin. Injection crystalline penicillin 20 lac 6th hourly; injection gentamicin and metronidazole are given in secondary infection. Only problem with penicillin is that it is GABA antagonist and may increase the convulsions, but still it is the most commonly used drug. Metronidazole is more effective and choice of drug at present-500 mg intravenously 8th hourly for 10 days.

Neutralizing the Unbound and Circulating Toxins It is done ideally by Human Tetanus lmmunoglobulin {HTIG/ ATG)-3000-6000 units. HTIG is given intramuscularly usually to deltoid as a single dose or often in 2 or 3 divided doses. HTIG (ATG) has got long half life (40 days). There is no risk of hypersensitivity; it does not interfere with antibody production; there is no preservative. It does not penetrate

blood-brain barrier. lntrathecal injection of antitoxin is of less value (circulating free toxin in the CSF can be neutralised in first 48 hours during initial development of the spasm). It should not be given intravenously. It is 100 times more potent than anti-tetanus serum (ATS, equinus tetanus antitoxin).

Control of Muscle Spasms Benzodiazepines are used mainly, they are GABA agonists but do not restore glycinergic inhibition. Higher doses of diazepam; lorazepam with a longer duration of action; midazolam with shorter duration of action as infusion (15 mg/hour). Diazepam and lorazepam contains propylene glycol which may cause lactic acidosis; but midazolam does not contain propylene glycol. Diazepam has got cumulative effect by it metabolites oxazepam and desmethyldiazepam. Phenobarbitones (anticonvulsants) and chlorpromazines are also used. Therapeutic paralysis with a nondepolarising neuromuscular blocking agent with mechanical ventilation is often required. Vecuronium and atracurium are used; pancuronium is also used but may cause tachycardia and hypertension. Propofol infusion, dantrolene, baclofen are other agents used. lntrathecal baclofen is also often used (500-200 µg infusion) which is a GABA agonist but is invasive. Centrally acting drugs like methocarbamol, mephenesin or meprobamate is useful. Methocarbamol which is long acting with high potency does not suppress respiration. It may cause severe gastritis and haemorrhagic cystitis. Mephenesin causes hypotension and haemoglobinuria. Meprobamate causes thrombosis, haemolysis and uraemia but is most potent muscle relaxant.

Control of Autonomic Dysfunction Autonomic dysfunction in tetanus causes haemodynamic instability. Fluid therapy (8 liters/day), morphine (20-180 mg), phenothiazines [Chlorpromazine, phenoxybenzamine, phentolamine (a-receptor blocker)], anticholinergics (atropine 100 mg hourly), a2 adrenergic agonists (clonidine, dexmedetomidine) contribute to cardiovascular stability. Beta blockers like propranolol should not be used. Sodium valproate, angiotensin converting enzyme inhibitor, adenosine are other drugs used for this purpose. Magnesium sulphate as presynaptic neuromuscular blocker reduces receptor responsiveness to the catecholamines and also is an anticonvulsant and a vasodilator. Magnesium sulphate infusion is rapidly emerging as a useful therapeutic technique. It controls the autonomic dysfunction; intravenous loading dose of 5 gram given in 20 minutes followed by infusion for maintenance should be given; one should observe for hypermagnesaemia by observing ventilation changes.

Managing Respiratory System Complications Aspiration , pneumonia, ARDS (adult respiratory distress syndrome), respiratory failure are common life-threatening

Ancients discovered much, and yet left much more to be discovered.

49

:::c ""' )> '"C

-t m

:l:>

..... C

"....

ID :::, ID Qj

V\

C:

'....° ID

':':

:::,

io' n ,..,.

o· C: VI

9. VI

ID Qj

VI

ID VI

50

...>-Cl.I

...::::,Cl

VI .._

complications. Ventilator support (/PPR) with endotracheal intubation; paralyzing the patient; tracheostomy, regular suctioning , respiratory physiotherapy for prevention of bronchopneumonia and ARDS; regular monitoring by doing arterial blood gas analysis (ABG), total count, chest X-ray . Steroids may be beneficial in ARDS and in patients with carditis.

0 rQ

::::, C:

rQ

::E VI

co 0::

Active Immunization Tetanus toxoidshould be given as disease will not give immunity against further infection. To start-1st dose, 2nd dose after one month, 3rd dose after 6 months.

VI

Wound Management Wound debridement, drainage of pus when present and injection of HTIG 500 units into wound area to reduce the effects of toxins locally.

• • • • • •

Isolation Avoid noise and light ATG 3,000 units IM ATS-50,000 IM and 50,000 IV- after test dose-not used now Antibiotics like injection penicillin 20 lacs 6th hourly injection tetanus toxoid 0.5 ml IM-to deltoid muscle IV fluids with TPN • Uri nary cathete risatio n • Nasogastric tube is passed to prevent aspiration initially, later for feeding • Regular suction of throat Nasal oxygen when required Prevention of bedsore formation Prevention of DVT by low molecular weight heparin Good nursing care

• l

B

Management of Complications Respiratory (laryngospasm, aspiration, pneumonia, ARDS, pulmonary oedema), renal (infection, failure), cardiac (arrhythmias, failu re), gastrointestinal (stasis, ileus, diarrhoea, haemorrhage), fractu re bones and vertebrae, muscle and tendon injuries, DVT, thromboembolism, sepsis should be managed accordingly.

Other Management Good nursing care, prevention of bedsore and DVT (deep vein thrombosis), nutrition (enteral through nasogastric tube or parenteral using a central line)- are important. Care of urinary catheter, prevention of urosepsis is important. Steroids may be useful in ARDS and carditis. Pyrexia or hyperpyrexia should be treated by tepid sponging, intravenous paracetamol or steroids. It is a poor prognostic indicator. Hyperbaric oxygen even though routinely not used has got beneficial effects in reversing the symptoms. Cardiac pacemaker may be useful in refractory bradycardia and arrhythmias. Following treatment patient may develop muscle spasms or tics which can be prevented by giving oral methocarbamol for one year. •·· Monitoring during therapy: PCO2, PO2, haemotocrit, blood urea, serum electrolytes, chest X-ray, ECG are done at regular intervals. Note:

• Culture media for Clostridium tetaniis ACM (Robertson's Cooked Meat) media and nutrient agar. • Serum antitoxin (antitetanus antibody) level more than 0.01 units/L is protective against tetanus. • When HTIG is not available or financial constraint is the matter then as an alternative, equine tetanus antitoxin serum (ATS, antitetanus serum) can be given. Full dose is 1, 00,000 units; half is given intramuscular and another half given intravenously after initial testing for anaphylaxis by injecting a test dose of 1000 units intravenously.

---

• • • •

IV diazepam 20 mg 6th hourly IV phenobarbitone 30 mg 6th hourly IV chorpromazine 25 mg 6th hourly Endotracheal intubation and ventilator support Tracheostomy if there is severe respiratory secretions Steroids Bronchodilators like deriphylline Wound care--debridement, drainage, and local injection of ATG

B • In adults, fresh immunisation to start-second in one month, next in 6 months period. Tetanus toxoid 0.5 ml IM. Booster dose should be given once in every 4 years or after any significant trauma • In infants-triple antigen (DPD-6 weeks, 10 weeks, 14 weeks, 18 months, and 5 years • To pregnant mother tetanus toxoid injections are given in 4th and 6th months of pregnancy • Additional booster dose given in major injuries or high-risk injuries • ATG 500-1000 units IM given as a prophylaxis in road accidents, severe burns, crush injuries, war wounds, penetrating wounds and wounds of head and face. Here tetanus toxoid also should be given separately at separate site IM

B • Respiratory failure with aspiration pneumonia and ARDS Severe carditis-an ominous sign • Mortality is 45-50%-becoming less now in will equipped centers (15%)

GAS GANGRENE It is an infective gangrene caused by clostridial organisms involving mainly skeletal muscle as oedematous myonecrosis. Earlier it was called as malignant oedema. Source and predisposing factors ,, Contaminated, manu red or cultivated soil, intestines are the sources. Faecal flora commonly contains clostridial organisms enters the wound; in presence of calcium from blood clot or silica (silicic acid) of soil, it causes infection.

,

It is common in crush wounds, following road traffic accidents, after amputations, ischaemic limb, gunshot wounds, war wounds. Injury or ischaemia or necrosis of the muscle due to trauma predisposes infection. ,.. Anaerobic environments in the wound- initial infection with aerobic organism utilises existing oxygen in tissues creating anaerobic environment to cause clostridial sepsis. Organisms-. Clostridium Welchii (perfringens): Gram-positive, anaerobic central spore bearing, nonmotile, capsulated organisms, most common-60%. Others are-Clostridium oedematiens; Clostridium septicum; Clostridium histolyticum. Note: • Various strains include- A, B, C, D, E. 'A' strain is most common. • Non-clostridial gas producing organisms like coliforms can also cause gas gangrene.

B

CLOS TRIO/UM WELCH/I PRODUCE TOXINS

• Alpha (most common); Beta; Epsilon; Iota • Phi toxil}--myocardial depressant • Kappa toxil}--destruction of connective tissue and blood vessels • Bursting factor and circulating factor

I Exotoxins Lecithinase is important toxin which is haemolytic, membranolytic and necrotic causing extensive myositis. It splits lecithin into phosphocholine. Haemolysin causes extensive haemolysis. Hyaluronidase helps in rapid spread of gas gangrene. Proteinase causes breaking down of proteins in an infected tissue. Spores enter through the devitalised tissues commonly in road traffic accidents, crush injury j,

Spores germinate j,

Released bacteria will multiply j,

Exotoxins are released cause their effects

I Effects Extensive necrosis of muscle with production of gas (hydrogen sulphide; nitrogen; carbon dioxide) which stai ns the muscle brown or black anaerobic myositisl myonecrosis. Usually muscle is involved from origin to insertion. Often may extend into thoracic and abdominal muscles. When it affects the liver it causes necrosis with frothy bloodloaming liver, is characteristic. Rapidly spreading infection which is also often fatal.

Limbs are commonly involved; but organs like liver can also be affected. Muscle glycogen is broken down into lactic acid, CO2 and hydrogen. Proteinase released by organism forms amino acids which further releases ammonia and hydrogen sulphide. Acid released earlier is neutralised by ammonia and calcium to progress further multiplication of organisms.

I Clinical Features Incubation period is 1-2 days. Features of toxaemia, fever, tachycardia (out of proportion to fever) pallor. Wound is under tension with foul smelling discharge (sickly sweety/decaying apple odour). Khaki brown coloured skin due to haemolysis. Crepitus can be felt. Jaundice may be ominous sign and also oliguria signifies renal failure. Frequent sites are adductor region of the lower limb and buttocks and subscapular region in upper limb. Clostridium Welchii can infect limbs, abdominal wal l, appendix, gallbladder, common bile duct, intestine, uterus (during septic abortion).

I Clinical Types Fulminant type causes rapid progress and often death due to toxaemia, renal failure or liver failure or MODS or ARDS. Massive type involving whole of one limb containing fully dark-coloured gas filled areas. Group type: Infection of one group of muscles, extensors of thigh, flexors of leg. Single muscle type affecting one single muscle. Subcutaneous type of gas gangrene involves only subcutaneous tissue (i.e. superficial involvement). It is mainly of anaerobic cellulitis type without muscle involvement usually caused by less virulent clostridial organisms other than clostridial welchii. It is usually superficial but may spread and involve fascial planes. It causes necrosis with foul smelling seropurulent discharge.

Fig. 1.115: Gas gangrene in upper limb. It is after effect of an

assault using an axe by a drunken husband.

Peace is so hard to find because it is under your nose.

51

....c:,

52

VI

al a:

V'I

Fig . 1.116: Gas gangrene involving entire leg extensively. Note

the black/khaki coloured area. Patient died of toxaemia.

Septicaemia, toxaemia. Renal failure, liver failure • Circulatory failure, DIC, secondary infection Death occurs in critically ill patients

I Investigations X-ray shows gas in muscle plane or under the skin. Liver function tests, blood urea, serum creatinine, total count, P02, PC02. CT scan of the part may be useful especially in chest or abdominal wounds. Gram's stain shows Gram-positive bacilli. Robertson's cooked meat media is used which causes meat to turn pink with sour smell and acid reaction. Clostridium Welchii is grown in cu lture media containing 20% human serum in a plate. Antitoxin is placed in one-half of the bacteria grown plate sparing the other half. Zone of opacity will be seen in that half of the plate where there is no antitoxin. In the other half part of the plate where there is antitoxin there is no opacity-Nag/er reaction.

Liberal incisions are given. All dead tissues are excised and debridement is done until healthy tissue bleeds. Rehydration and maintaining optimum urine output (30 mU hour) (0.5 mUkg/hour). Electrolyte management. In severe cases amputation has to be done as a life-saving procedure-stum p should never be closed (Guillo tine amputation}. Often ventilator support is required. Once a ward or operation theatre is used for apatient with gas gangrene, it should be fumigated for 24-48 hours properly to prevent the risk of spread of infection to other patients especially with open wounds. Hypotension in gas gangrene is treated with whole blood transfusion. Therapy should be concentrated in managing dehydration, hypotension, infection, toxaemia by hydration, fresh whole blood transfusion, passive immunisation, antibiotics, and hyperbaric oxygen, doing radical wound excision with removal of all dead tissues with foreign body or amputation with critical care.

TUBERCULOSIS Whilst meagre Phthisis gives a silent bow, her strokes are sure, but her advances slow. No loud alarms, nor fierce assaults are shown: She starves the fortress first, then takes the town. -Samuel Garth, 1699

B Proper debridement of devitalised crushed wounds Devitalised wounds should not be sutured Adequate cleaning of the wounds with H20 2 and normal saline Penicillin as prophylactic antibiotic.

I Treatment Injection benzyl penicillin 20 lacs 4th hourly+ Injection metronidazole 500 mg 8th hourly+ Injection aminoglycosides (if blood urea is normal) or third generation cephalosporins or metronidazole. Fresh blood transfusion. Po/yva/ent antiserum 25,000 units given intravenously after a test dose and repeated after 6 hours (Welchii 10,000 IU, oedematiens 10,000 IU, and septicum 5,000 IU). Hyperbaric oxygen is very useful.

Fig. 1.117: Tuberculous lymphadenitis and tuberculous ulcer.

It is commonly caused by Mycobacterium tuberculosis; occasionally by Mycobacterium bovis, M. kansasii, M. fortuitum, M. marinum, M. u/cerans. M. tuberculosis is gram neutral, acid fast, alcohol fast straight or slightly curved rods. It is prevalent in most of the developing countries and has made its resurgence in the developed countries with the advent of AIDS. The characteristic lesion here is 'tubercle', which is an avascular granuloma composed of a central zone containing giant cells, with or without caseation necrosis, surrounded by a rim of epithelioid cells, lymphocytes and fibroblasts.

53

It can occur in almost all organs in the body. Presentation may vary depending on the individual sites. General features are-low-grade fever with evening rise of temperature; loss of appetite; decreased weight.

n

:::c:

I Investigations

)>

"'O

-4

m

....

ESR is often raised; peripheral smear-lymphocytosis. AFB (acid-fast bacillus) staining using Ziehl-Neelsen stain. Chest X-ray is done to rule out pulmonary tuberculosis. Culture of the organism in Lowenstein Jenson media. Mantoux skin test- read after 48 hours of inoculation. Guinea-pig inoculation. Relevant investigations depending on the site of the tuberculosis. Interferon gamma release assay (IFN-y).

:::i,

0

VI

C: .... c.c fl) ....

':':

::,

I Treatment

('\

!:!". 0

Antituberculous drugs are given for 6 months to one year. Specific treatment is given depending on the site of the tuberculosis. (Please refer individual chapters for details about tuberculosis at different locations and also chapter drugs for Anti-tuberculous Therapy.)

C: V'I

0 vi' fl)

0J V'I

fl)

V'I

LEPROSY It is caused by Mycobacterium leprae. It is a Gram-positive, acid fast bacillus. It mainly involves skin, nasal mucosa and peripheral neural tissues. It involves only the cooler parts of the body (So axilla, gluteal region are not involved). Testicular involvement is seen but not the ovary. It does not involve the vital organs. Though not acutely fatal, the disease leaves the victim severely deformed and crippled for life.

TYPES 1. Multibacillary Types

Lepromatous leprosy. It denotes little or no host resistance. Bacilli are seen in large numbers in the superficial nodular lesions and the patient is highly infective. • Borderline /epromatous. Borderline. 2. Paucibacil/ary Types •

Borderline tubercu/oid.

• Tuberculoid leprosy. Here strong host resistance is observed. The disease is more localised, but it causes more deformities due to early involvement of nerves. Bacilli are scanty in the lesion and so infectivity is minimal.

Figs. 1.118A to D: A leprosy patient with the typical face (leonine); skin patches; hand deformities and trophic changes; trophic ulcer in heel.

Investigations: Regular checking of sensation of the suspected area is done. Split skin smear, nerve biopsy taken. Treatment: Dapsone 100 mg daily; Rifampicin 600 mg once a month; Clofazimine 50 mg daily+ 300 mg once a month. Note: • For paucibacillary types, treatment is for 6 months. • For multibacillary types, treatment is for 2 years or more.

B a. Primary Deformities Leonine facies. Collapsed nasal bridge. • Upper branch facial nerve palsy (causes lagophthalmos). Keratitis and blindness. Claw hand either ulnar or combined ulnar and median nerves. Radial nerve palsy-wrist drop (1%). • Clawing of toes due to involvement of posterior tibial nerve. I Foot drop due to involvement of lateral popliteal nerve. (Medial popliteal nerve which supplies the tibialis posterior is never involved.) Contd...

Right is right even if everyone is against it, and wrong is wrong even if everyone is tor it.

54

Contd... ~

Secondary Deformities

Anaesthesia of the part makes it prone to trauma, infection, infective gangrene, destruction, autoamputation and function-

less parts. Trophic ulcers in the foot are common. Treatment of complications: Reconstructive surgeries; Release of contractures; Tendon transfers; Arthrodesis; Ulcer management; Physiotherapy and rehabilitation. Fig. 1.119: Saddle nose .

.,,

SYPHILIS (GREAT POX) lFRENCH DISEASE)

a:

ft is concluded... that this disease which amongst the Italians i~ caned Gafficus, that is to say, the French disease, should now be named Patursa, .. .a disease fifthie and Saturnan. ft is a fifthie disease, because it maketh women to be esteemed unchast and irreligious.... There is a twofold kinde of causes .. .. The first is the only inffuence or corruption of the aire, from whence we must charitably thinke, that it infected those which were religious. The second is conversation, as by kissing and sucking, as appeareth in children, or by carnan copulation.

co V'I

-Juan Almenor, 1502

It is a venereal infection caused by Treponema pal/idum (Refer Chapter 1B-Ulcer).

I Congenital Syphilis Here the infection is transmitted from the mother to foetus through placenta. Early congenital syphilis: It is seen in newborn. • Features are: - Rash, syphilitic snuffles - Nasal discharge, weight loss - Periostitis, meningitis, hepatosplenomegaly - Pneumonia alba

Late congenital syphilis: • Hutchinson's triad: - Interstitial keratitis - 8th nerve deafness - Hutchinson's teeth: Peg shaped upper incisors • Moon's molar, molars with cusps • Congenital neurosyphilis • Cutaneous, skeletal or visceral gummas • Saddle nose • Sabre tibia, Glutton's joint • Perforated palate

1

Congenital syphilis is treated with penicillins.

ACTINOMYCOSIS It is caused by Actinomyces israelii. It is an anaerobic Gram-positive fungal like bacterium, which is a branching filamentous organism. It is called as "Ray fungus" because of sun-ray appearance.

B • Facio-cervical: It is the most common type. Infection spreads either from tonsil or from adjacent infected tooth. Initially an induration develops. Nodules form with involvement of skin of face and neck. It softens and bursts through the skin as sinuses which discharge pus which contains sulphur granules (60%). Thorax: Lungs and pleura get infected by direct spread from pharynx or by aspiration. Empyema develops. Later chest wall nodules appear leading to sinuses with discharge (20%). • In righ t iliac fossa: It presents as a mass abdomen with discharging sinus. • Liver is infected thro ugh portal vein (Honey comb fiver). • Pelvic: Pelvic actinomycosis can occur due to intra-uterine devices.

Organism enters through deeper plane of the tissue, causes subacute inflammation with induration and nodule formation. Eventually discharging sinus forms at the surface. Pus collected in a swab or sterile tube will show sulphur granules. Features: , Discharging sinus with induration and nodules; No lymph nodal involvement. Through blood spread it may cause pyaemia and endanger life. , Pus under microscopy shows branching filaments. , Gram's staining shows Gram-positive mycelia in centre with Gram-negative radiating peripheral filaments. These clubs are due to host reaction which are lipoid material (antigen-antibody complex). , Cultured in brain heart infusion agar and thioglycolate media. Differential diagnosis: Chronic pyogenic osteomyelitis; Carcinomas at the site; Tuberculous disease Treatment ,. Penicillins for longer period (6- 12 weeks). , Tetracyclines, lincomycin, streptomycin. , Dapsone and iodides may be useful. , Antifungals are often given because it is fungal like bacterium. , Su rgical debridement is occasionally required. , Welsh regimen-Injection amikacin 15 mg/kg IV daily fo r 21 days; such cycle is repeated 3 times at a gap of 15 days-along with tablet trimothoprim (7 mg/kg)sulfamethoxazole (35 mg/kg) daily for 6 months.

MADURA FOOT (MYCETOMA PEDIS) It is a chronic granulomatous condition of the foot involving subcutaneous and often deeper tissues causing multiple discharging sinuses. It was first identified in Madurai, Tamil Nadu (India) by Gill (1842, Madura mycosis). It is common in India and Africa. It is common in Tamil Nadu. It can be fungal (more common) or bacterial origin. , Bacterial can be Actinomyces or Nocardia. Among bacterial Nocardia madurae is most common. , Bacterial mycetomas are by Actinomyces (A. israelii or A. bovis) or by Nocardia or Actinomadura (red granules). Occasionally it can be due to pyogenic bacteria like Staphylococcus aureus (Botryomycosis). Bacterial mycetoma shows white/yellow/red granules which on smear delineates thin filaments (1 µm). Nocardia asteroids can primarily involve lungs later spreading to brain, kidney and other organs as metastatic infection. , Fungal eumycotic mycetoma is caused by Madurella mycetomi or Madurella grisa. Fungal mycetoma causes black granules, crushed smear of which shows thick stout filaments (5 µm). Organisms: Nocardia madurae (most common); Nocardia brasiliensis; Nocardia asteroides; Actinomyces israelii.

I Pathogenesis Organism enters through a prick in the foot usually who walks barefoot _j,

Reaches deeper plane in the foot _j,

Evokes chronic granulomatous inflammation _J,

Causes pale, painless, firm nodule .J, Formation of vesicles .J, Burst to form a discharging sinuses.

B

-

-

-

~-

- - --

--

Note:

-

• It is a chronic, granulomatous, progressive inflammatory condition involving subcutaneous tissue, skin and deeper tissues It may be bacterial (actinomycetoma) or fungal (eumycetoma) • Painless subcutaneous tissue; multiple sinuses and seropurulent discharges-triad of mycetoma Causes deformity, disability, destruction 70% occurs in foot; 12% in hands Local hyperhidrosis due to sympathetic over activity can occur • Tiny, shotty regional lymph nodes can get enlarged due to secondary infection or as localized immune response • Osteomyelitis, Kaposi's sarcoma, carcinoma are the differential diagnoses 'Dot in circle sign' in MRI is characteristic FNAC and biopsy is very useful to confirm lmmunoelectrophoresis and ELISA are having diagnostic value.

55

•

I

I Features Painless diffuse swelling in the foot of long duration. Later multiple discharging sinuses develop on the skin. Lymph node involvement will not occur unless secondary bacterial infection is present. Significant limb disability is common. Common in males. 20% develop pain-due to secondary infection or bone involvement. Differential diagnosis: Chronic osteomyeliti; Tuberculous osteomyelitis; Carcinoma, Kaposi's sarcoma.

I Investigations Discharge study will show branching filamentous appearance of the organism. Cultu re in Sabouroud's dextrose agar medium. Gram stain for actinomycosis will show sun-ray appearance with Gram +ve centre and Gram -ve clubs. X-ray of the foot is done to look for osteomyelitis. FNAC and biopsy is confirmative as eumycetoma or actinomycetoma. Ultrasound of area; MRI are useful. Dot in circle sign in MRI is characteristic. lmmunoelectrophoresis and ELISA are having diagnostic value showing antibody titre against causative organism.

Discharging granules may be Black, Red, Yellow. In black type of Madura foot, infection is mainly subcutaneous. In red and yellow types, it burrows into the deeper plane including bone causing bone necrosis (osteomyelitis). Eventually gross swelling of the limb with multiple discharging sinuses with disability will occur. Muscles and bones are involved. Tendons and nerves are spared until very late. Regional lymph nodes are not involved. Condition will deteriorate by secondary bacterial infection. If infection occurs in hand, it is called as Madura hand.

-

MYCETOMA

Fig. 1.120: Madura foot. Note the multiple sinuses.

Life is like a mirror. If you smile at it, it also smiles at you.

c;)

n> n>

... ::I

DJ

VI C:

... ...n>

IQ

::I

it n

.... o· C:

Ill

9.

Ill

n>

DJ

Ill

n>

Ill

56

Celsus found relation of saliva of infected dog to human disease in 1st century AD. Louis Pasteur developed 1st vaccine against Rabies in 1885. Rabies is uncommon in developed countries. It is mainly seen in Indian subcontinent (80%) and Africa. In India, it is not seen in Lakshadweep, Andaman and Nicobar islands. If disease is not seen for 2 years in humans and animals then that area is termed as rabies free. Maldives country does not have human or animal rabies.

...111 ...:::, °' >,.

Vl

0

ta

:::, C:

ta

I Pathogenesis

:lE

"' co a: Vl

Figs. 1.121 A and B: Mycetoma thigh and foot in two different patients. Note the discharging sinuses.

I Treatment Medical In actinomycetoma, amikacin or streptomycin, co-trimoxazole combined therapy is the first line of treatment (5 weeks therapy). Long-term penicillins are useful. Amoxicillinclavulanic acid, rifampicin, sulphonamides, gentamicin and kanamycin are second line of drug treatment. In eumycetoma, ketoconazole, itraconazole, voriconazole are the drugs used for long term up to one year. Dapsone and iodides are often used for long term.

Surgical Wide surgical excision should be done after initial proper medical line of treatment. Improper excision will cause recurrence. Recurrence rate is up to 50%. After wide excision, drug therapy has to be continued. Amputation is needed in severe refractory cases as a life saving option. Amputation rate is 10-25%.

RABIES (HYDROPHOBIA) It is an acute fatal encephalomyelitis caused by a single stranded RNA virus Lyssa virus type 1. It is a zoonotic disease transmitted to humans by bite/lick/ scratch of infected animals (commonly dogs). It is an ancient disease mentioned even in Vedas. Rabies word is derived from Sanskrit word 'Rabhas' means 'to do violence'.

There is no predilection for age or sex even though, it is observed more in children and adult males. Ninety-five per cent of rabies develops due to bite of rabid dog occasionally cat (Urban rabies). Other animals that can transmit rabies are monkey, horse, fox, cows and buffaloes, donkey, pig , sheep, camel, elephant, mongoose, jackal, bear (Wild life/sylvatic rabies). In India, transmission is not observed through bats, rodents and birds. Bat rabies (vampire bat) is seen in parts of USA and Latin American countries. Asymptomatic carrier stage occurs only in animals, but they are unlikely to be infective. Only symptomatic animals are considered to be infective. Rabies virus is bullet shaped envelop virus (75 nm x 180 nm) with numerous glycoprotein spikes to help in attachment of virus and also to induce antibodies. Natural occurring rabies virus is called as street virus which shows long incubation period of 20-60 days. Serial passage of this virus to brain of rabbits creates fixed virus which has got short incubation period of 4-6 days which does not show Negri bodies. This fixed virus which cannot multiply in extraneural tissues is inactivated to prepare vaccine. Infection commonly occurs by animal bite, often by licks, scratches. Licks on abraded skin and licks on abraded or unabraded mucosa can cause infection. Licks are often ignored dangerously. Severity of infection depends on viral load in the animal saliva and class of wound. Aerosol transmission is found in bats or in laboratory workers. Person to person transmission can occur even though it is rare. Rabies may get transmitted through organ/corneal transplantation. Virus multiplies at the site of infection and passes (ascends) through the peripheral nerves into the CNS to develop Negri bodies in the brain leading into fatal encephalomyelitis. From the brain vi rus descends into different tissues like salivary glands, muscles, heart, adrenals and skin. It also involves salivary glands to get secreted in the saliva to cause infection.

I Clinical Features Incubation period is 3-6 weeks; but rarely can be up to many years. Prodromal symptoms like fever, headache. Pain, tingling sensation at the site of bite.

Hyperexcitability and irritability; increased muscle reflexes and spasms. Increased salivation, sweating, lacrimation. Hydrophobia (fear of water) and aerophobia (fear of air) is pathognomonic . Mental instability, dilatation of pupils. Symptoms are aggravated by swallowing water or blowing air on them. Once disease starts, patient die in 72 hours. Fear of water is seen only in affected human beings, not in animals.

B • Class I: Touching or feeding the diseased animal, lick over intact

skin or scratches without oozing of blood. • Class It. Licks on broken skin, scratches with blood ooze, and all bites except over head, face, palms and fingers. Minor wounds less than five in number. • Class Ill: All bites over head, face, palms and fingers, lacerated wounds, wounds more than five in number, wildanimal bites, and contamination of mucous membrane with saliva.

B • All rabid animal bites • If animal is killed or dies during 10 days of observation period • Bite by an unidentified animal • If laboratory tests in animal show positive for rabies • All wild animal bites

I Vaccines for Rabies 1. Nervous Tissue Vaccine (a) BPl inactivated vaccine: It is nervous tissue vaccine. It is 5% emulsion of the infected brain of the sheep containing the inactivated fixed virus. It is Semple vaccine. Dosage of Semple vaccine (as recommended by Pasteur Institute . Coonoor)

Class I Class II Class Il l

Adult

Children

2 ml 3 ml 5 ml

1 ml

Duration 7 days

3 ml 3 ml

10 days 1Odays

Mode of administration : Subcutaneously into the abdominal wall using long needle. Joseph Meister received first anti-rabies vaccine (ARV) in 1885. In Olden days, it was given for 15- 21 days. Antibody develops in 7-30 days. Protection lasts only for 6 months. Booster doses are given if needed. Side effects Headache, palpitation, allergic reactions. Redness, tenderness and swelling at the site of the vaccination.

Post-vaccinal neuroparalysis- a dangerous life-threatening complication. During therapy patient should avoid alcohol and steroids. (b) Nervous tissue vaccine derived from suckling mouse (less than 9 days old) brain (Fuezalida vaccine) is used to reduce neuroparalytic complications as suckling mouse has low myelin neuron. It is given for 1Odaily doses then on 20th and 30th day.

57

n

:I: )>

"'0

-f

m

....

::0

2. Avian Vaccines-Duck Embryo Vaccine (DEV) It has got less neu roparalytic side effects. It can cause egg protein allergy. Purified duck embryo vaccine (PDEV-1 ml} is available in India.

3. Cell Culture Vaccines They are more potent and safer. Human Diploid Cell Vaccine (HDCV-1 ml): Safest vaccine. It is prepared using fixed virus in human diploid fibroblast cells. But it is costly. It is available in India. Second generation tissue culture vaccines: They are potent and cost-effective. They are derived from nonhuman base so urces. Examples are chick embryo fibroblast (Purified Chick Embryo Cell Culture Vaccines-PCECV-1 ml), foetal bovine kidney, hamster kidney cells, vero cells (Purified Vero cell Rabies Vaccine-PVRV-0.5 ml). Dosage: 2.5 IU in 1 ml. 1 ml is given IM into the deltoid on 0, 3, 7, 14, 28 and 90 (optional) days. Side effects: Headache, redness at the site, fever. No other serious side effects. Second generation tissue culture vaccine can be given intradermally also. lntradermal dose is one fifth of the intramuscular 1 dose (0. ml}.

I Wound Treatment

--- - - - - - - - - - - - - - - - - - Proper local wound care reduces the chances of rabies infection by 80%. Immediate cleaning and washing of the wou nd with running water for 15 minutes is essential to reduce the viral load at the wound site. If soap is available soap water is also used. It is better to wash with warm water if available. Wound should be cleaned with virucidal agents like alcohol, tincture, povidone iodine. Savlon or carbolic acid or nitric acid should not be used. Wounds should not be closed. ARS should be injected locally. In deep wounds it may be closed only after 48 hours with loose sutures after thorough washing. ARS (horse or human) should be injectedto all wounds locally. One should not scrub the wound. One should not touch the wound with bare hands. One should wear gloves to touch the wound.

I Passive Immunity It is used in all severe exposures, class II and class Ill and in all wild animal exposure. Present recommendation is injec-

The best rose bush is not the one with the fewest thorns, but that which bears the finest roses.-Jerry Van Dyke

0

G)

ID ::, ID

...

QI

VI C: \0

... ... '< ID

::,

n

o· C: ,-+

VI

0

v;·

ID

QI VI

ID VI

58

tion of ARS with vaccine in all exposed patient irrespective of the class.

I Types of Antirabies Serum (ARS) Horse antirabies serum (horse/equine ARS): It is given on first day with a dose of 40 IU/ kg body weight (maximum up to 3000/units). Half is given into the wound and another half given into the gluteal muscle (IM)-single dose. It prevents the multipl ication of the virus at the wound site. It also prolongs the incubation period. Passive immunity should always be combined with vaccine therapy. ARS needs test dose prior to injection of full dose. Side effects: Serum sickness, anaphylaxis. Human rabies immunoglobulin (HRIG). Dose is 20 units/kg body weight. Part is injected into the wound remaining part into the gluteal muscle (IM)-single dose. Patient should be immunised actively along with serum with additional booster doses. Side effects are rare here.

I Post-exposure Prophylaxis Cell culture and purified duck embryo vaccines are used as they are safe and efficacious. All vaccines should be given to deltoid region (never to gluteal region as due to high fat content vaccine would not get absorbed into circulation rapidly and so immune response may not be optimum). Vaccines should be stored at 4-8°C after reconstitution and should be used immediately.

Mode of Injection

a. Intramuscular into deltoid regiorrEssen regimen . It is common ly used and technically easier but higher dose is required compared to intradermal. It is injected at a schedule of 0, 3, 7, 14, and 28 days and booster at 90 days. First dose should be combined with ARS preferably HRIG. Multisite IM regime is often used as follows-first dose on day Otwo doses of IM vaccine is injected one on each side deltoid. Later single doses on 7 and 21 days (as O{2}, 7 {1}, 21 {1)). b. lntradermal route: (1) Two site intradermal method is used. 1/Sth of the IM dose of selected vaccine is used. Two sites on the day 0, 3, 7 and one site on the days 28 and 90. PVRV 0.1 ml; PCECV 0.2 ml; PDEV 0.2 ml. (2) Eight site intradermal method is used. On day 0, eight sites intradermal injections at both deltoids, both suprascapular, both thighs, both lower quadrants of abdomen are given. On day 7, on 4 sites-both deltoids, both thighs intradermal injections are given. On days 28 and 90 one dose on each day intradermal vaccine is injected at one site. HDCV 0.2 ml is used. It is like o (8); 7 (4); 28 (1 ); 90 (1). In whatever type, on first day (0) rabies immunoglobulin should be injected locally as well as IM. Post-exposure vaccination if individual has been vaccinated earlier. Doses on days o, 3, and 7 are given. But ideally assessed by serum antibody level (should be more than 0.5 IU/ml. Passive immunity is not given in individuals who had vaccination earlier.

I Pre-exposure Prophylaxis It is given to veterinarians, animal handlers. Dose: 1 ml of cell culture vaccine IM or 0.1 ml intradermally on days 0, 7, 28. Serum titre for antibodies should be assessed after 1 month. If it is less than 0.5 IU/ml then one booster dose is injected. Booster doses are given once in every 2 years.

B

RABIES IN DOGS

Incubation period: 10 days to 8 weeks. Types a. Furious type: Here dogs are aggressive like a mad dog. Dog changes its behaviour with loss of fear of human beings; bites objects, eat, mud, etc. Running amok, voice change, inability to bark properly, excessive salivation and foaming, paralysis and death. b. Dumb type: Dog is paralytic and sleepy. There is no aggressiveness at all. Dog sleeps for 3 days and dies. Once symptoms of rabies develop in a dog it rarely survives more than a week. Dog brain is sent for study in 50% glycerol-saline solution. Laboratory Tests to Confirm Rabies in Dogs/Animals • Fluorescent antibody test (FRA test): It is reliable test. If FRA test is negative in brain of animal then even if dog is rabid its saliva does not contain virus. FRA test is positive at any stage of the disease. • Microscopic examination of the brain of the infected dead animal to look for NEGRI bodies. It is seen in 90% of dead rabid dogs. • Mouse inoculation test is very sensitive test. 10% brain tissue emulsion in saline is centrifuged at 2000 rpm for 1Ominutes; 0.03 ml top fluid is injected intracerebrally into the suckling mouse to demonstrate rabies in 8 days in mouse. I • Corneal test is simpler but negative result does not rule out the infection possibility. Immunisation in Animals a. BPL inactivated nervous tissue vaccine (20% infected sheep brain suspension): Single dose 5 ml to dogs; 3 ml to cats. Second dose after 6 months. Then once a year regularly. b. Modified live virus vaccine (33% infected chick embryo suspension): Dose-3 ml single dose which is repeated once in 3 years. c. Oral vaccines are used successfully to control wild foxes in Canada by placing vaccine in food through baits.

B

REMEMBER ABOUT RABIES

• Control of stray dogs and immunisation of all dogs will reduce the incidence of rabies • Prevention is the only way in rabies. Established rabies cannot be treated-100% mortality • HDCV and PCECV dose is 1 ml; PVRV is 0.5 ml- lM into deltoid or anterolateral aspect of thigh in children (NEVER GLUTEAL region) • Day Ois the day of first date of vaccination not day of bite • Interchanging of vaccines is even though acceptable but not well recommended and ideally should be avoided • Vaccine dose is same in all age groups • Reconstituted vaccine should be used immediately • Vaccine dose may be doubled in first dose if ARS is not used in bites of face, head, hands and genitals, in malnourished pal;eat,, ;o pal;eots who ace oo ste,o;ds, aoUmal;gnaocy drngs, antimalarials; in pregnancy, lactation, infants, elderly, HIV and immunosuppressed individuals Contd...

I

I

Contd...

• Consumption of unboiled milk of a rabid cattle amounts for class needs prophylaxis vaccine I• IllPetbitedogandshould be examined periodically by veterinary doctor. • • • •

• I

•

I

••

• • • •

• •

Usual vaccination method used in dog is- at 3rd month of age, 1 month later, and later yearly booster doses. O, 6 months and then yearly is also used. Pet dog should be prevented from coming into contact with stray dogs Dietary or alcohol restriction is not needed during vaccination as it will not alter immune response Concomitant other vaccine injection along with rabies vaccine can be done but at different injection site as there is no interference with immune response There is no contraindication for rabies vaccine as it is life-saving method All cases of dog bite should receiveinitial vaccine and ARS immediately. If the dog remains healthy even after 10 days of observation period vaccine is discontinued. Virus can be present in saliva 3 days before onset; once symptom begins dog cannot live for more than 4 days. Safe period of 3 days is added and so total 1Odays is observed Virus can present in semen of a rabid man. So if he had contact with his wife within 5 days prior to symptoms then wife should be vaccinated as class Ill with HRIG If by mistake vaccine is given to gluteal region, fresh full course of vaccine to deltoid region should be started There are no single shot vaccines available. It is a myth Antiviral drugs are of no use Modern CCVs are very safe and efficacious lntradermal route of 0.1/0.2 ml is the best route Equinus ARS is cheaper and equally effective; but test dose should be given to prevent reaction Rabid dog will never have hydrophobia. Rabid dogs can swim through water or can even drink water. Aerophobia and photophobia are present in rabid dog. Hydrophobia is observed only in human rabies Bite by cats and cows also should be vaccinated Steroids and antimalarials should be avoided during vaccination as it may alter the immune response

ANTHRAX It is caused by Bacillus anthracis, which is a Gram-positive, aerobic, spore forming, capsulated, non motile, nonacid-fast bacillus and is resistant to heat and antiseptics. Disease is common in cattle and seen in people who handle carcasses, wool, hairs. It is often used in biological war.

B

TYPES

a. Cutaneous type (Hide porter's disease) - It is the most common type and occurs within 3-4 days after infection. - lndurated papule with black slough surrounded by vesiclesmalignant pustule. Itching is common in papule-black colour eschar (Anthrax means charcoa~ . - Regional lymph nodes are involved. - Toxaemia is common. b. Respiratory type ( Wool Sorter's disease) is due to inhalation of spores, causing haemorrhagic pneumonia. It is more dangerous nd life-threatening. mentary type due to ingestion of spores. Fatal septicaemia and meningitis can occur in any type.

l:3

Diagnosis: Culture of fluid will show Medusa head appearance. It shows positive M'Fadyean's reaction and positive Ascoli's thermoprecipitation test. Treatment: Antibiotics-Ciprofloxacillin, doxycycline, penicillins given. Alum precipitated Anthrax toxoid is used in humans. Scalvo's serum prepared by active immunisation of asses are used.

59

NOSOCOMIAL AND OPPORTUNISTIC INFECTIONS

I Nosocomial Infection (Hospital-acquired

C)

Infection)

ID

:::,

..,

ID

It is an infection acquired because of hospital stay.

a.,

Vl

Sources

C:

Contaminated infected wounds. Urinary tract/Respiratory tract infections. Opportunistic infections; abdominal wounds with severe sepsis. Spread can occur from one patient to another, through nurses or hospital staff who fail to practice strict asepsis.

':-

...:::,Cl (1/

V)

0

ro

:::, C

ro

cc a: Ill

V)

Figs. 1.129A and B: Picture showing HIV kit and wearing of the HIV kit as a precaution while doing surgery for HIV infected individuals.

HIV suppresses immune response completely directly by suppressing T cell, indirectly by suppressing 'B' cell. Finally it dismantles and destroys the immune system making the individual prone to opportunistic infections.

I General Features in HIV Weight loss more than 10%. Fever more than 1 month. Diarrhoea more than 1 month. Neuralgia, arthralgia, headache. Lymphadenopathy. Cutaneous rashes, dermatitis, fungal (Candida) , bacterial, viral (herpes simplex 1 and 2) infection. Dental infection, gingivitis, candidiasis of oral cavity and oesophagus. Varicella zoster infection. Opportunistic infections. Poor healing after surgery, trauma, infection with more complications.

B

--

TUMOURS IN HIV INFECTION

Kaposi's sarcoma-40% common Lymphomas (NHL common) (3-4%) • Cervical cancer • CNS lymphomas Ano-genital squamous cell carcinoma Testicular tumours (Germ cell types) Lung cancer GIT lymphomas and adenocarcinomas • Squamous cell carcinoma of anal canal and cervix

B

•i1 hi·i!Aitllil i:iil1:HiiiiiHiiiiiili·ii1

1

• Pneumonia Tuberculosis Fungal infections Pneumocystis carinii pneumonia • Cytomegalovirus pneumonia

B Fig . 1.130: Severe infection of ear cartilage in a HIV infected patient. CD4 of T eel!

GIT PROBLEMS IN HIV INFECTION

GIT infections- bacterial, protozoa!, viral • Kaposi's sarcoma, lymphomas, adenocarcinomas Hepatitis ('C' virus), cholestasis Anorectal diseases Abdominal tuberculosis

Activated CD4 T cell Lymphokines 1--------,

CDS cell

Cell k1ll1ng and Lchemokines ,

Neutralising antibody ( Antibody dependent cell killing

I

After HIV infection, antibodies develop to virus envelope and core proteins which persist throughout life.

Fig. 1.131: Kaposi's sarcoma.

7

'B

II

• Encephalitis, aseptic meningitis, myelitis • Neuropathies with demyelination Opportunistic infections like Toxoplasma, Cryptococcus causing severe meningitis • Primary CNS lymphomas • CNS tuberculosis {Tuberculomas) • Visual problems

Management Investigations Tests for HIV; Tests for specific and opportunistic infections. Tests relevant for associated tumours.

Treatment 1. Antiviral therapy: , Nucleoside reverse transcriptase inhibitor (NRTI): Zidovudine, didanosine, abacavir, lamivudine, stavudine. , Non-nucleoside reverse transcriptase inhibitor (NNRT~: Nevirapine, delavirdine. , Protease inhibitors: Ritonavir, indinavir, amprenavir. 2. Treatment of opportunistic infections. 3. Treatment of tumours. 4. lmmunotherapy: , Alpha and gamma interferons. ,. Interleukins. 5. Bone marrow transplantation. 6. Anti-CD3 or IL-2 after HAART (Highly Active Anti-Retroviral Therapy). 7. Psychotherapy. 8. Counselling of HIV patients and their families. 9. Life-expectancy after initial HIV infection is 8-1 Oyears. Prevention Continues to be our best weapon in combating the menace of HIV infection.

• Care in handling sharp objects like needles, blades • All cuts and abrasions in an HIV patient should be covered with a waterproof dressing • Minimal parenteral injections • Equipments and areas which are contaminated with secretions should be wiped with sodium hypochlorite solution or 2% glutaraldehyde

I

•

Contaminated gloves, cottons should be incinerated Equipments should be disinfected with glutaraldehyde Disposable equipments (drapes, scalpels, etc.) should be used whenever possible Walls and floor should be cleaned properly with soap water Separate operation theatre and staff to do surgeries to HIV patients is justifiable Avoid shaving whenever possible before surgery in HIV patients All people inside the theatre should wear disposable gowns, plastic aprons, goggles, overshoes and gloves Surgeons, assistants and scrub nurse should wear in addition double gloves Suction bottle should be half-filled with freshly prepared glutaraldehyde solution Spilled body fluids should be diluted with glutaraldehyde Accidental puncture area in surgeon or scrub nurse should be immediately washed with soap and water thoroughly Theatre should be fumigated after surgery to HIV patient

Safe sex. Condom usage reduces the risk of transmission. •·· Health education. Use of disposable needles to prevent infections.

HIV, hospital and surgeon ••• Isolation per se of HIV patient is not required. Proper care should be taken to prevent transmission of the virus. Open wounds, disposal of excreta, fluids, discharge, pus and other infective materials should be taken care of properly. Risk of HIV infection through needle prick is very less (0.03%). Following measures should be taken while managing HIV patients: Wearing double gloves. Wearing proper spectacles (as HIV can get transmitted through eyes directly). Wearing proper head mask, theatre shoes, apron. Measures to prevent spread of infection from patient to patient in the hospital. Disposal of needles through a sharp disposing container.

Disinfection Autoclave is ideal. Boiling. Sodium hypochlorite solution. 2% glutaraldehyde solution.

The drops of rain make a hole in the stone not by violence, but by oft falling.

67

_. 0 Cl 11)

::,

....

11)

QI

V'l

C: .... IO 11) ....

':': ::,

ro' n ,+

5· C: VI

0 VI 11) QI VI 11) VI

E. Swellings

( ;HAPTER OUTLINE - - - - - - -

• • • •

....

.... •

• •

Lipoma Cysts Dermoids Sebaceous Cyst Neuroma Fibroma Neurofibroma Neurilemmoma (Schwan no ma) Ganglion Papilloma Warts

• • •

..• .. • •

..

Glomus Tumour Bursae Semimembranosus Bursa Morrant Baker's Cyst Lymphangioma Lymph Cyst (Lymphatic Cyst) Calcinosis Cutis Chordoma Epignathus

Figs. 1.133A and B: (A) Swelling chest wall; (B) Multiple swellings in the scalp with ulceration.

LIPOMA 'Swelling' is an obvious enlargement of a part of the body due to various causes like congenital, inflammatory or neoplastic. Mass denotes a lesion which has difficulty to define the extent, e.g. mass abdomen; Lump denotes a defined lesion within an organ or the one causing the alteration in the gross nature/shape of a part or organ, e.g. breast lump.

Fig. 1.132: Swelling scalp; could be sebaceous cyst.

It is a benign neoplasm arising from yellow fat. Often it can be hyperplasia or combination of neoplasm and hyperplasia. A lipoma is composed of mature adipocytes and uniform nuclei that are identical to those seen in normal adult fat. The fat in lipoma is considered unavailable for general metabolism. It is the most common benign tumour (karyotype 12q change). Prevalence is 2.1 per 100 people. It is called as universal tumour (ubiquitous tumour) as it can occur anywhere in the body except brain. It can be localised (encapsulated) or diffuse (nonencapsulated). , Localised lipoma is encapsulated with yellowish orange colour. , Diffuse lipomas are not encapsulated and not localised. It is common in palm, sole, head, neck. It is seen in subcutaneous and intermuscular tissues. It is difficult to remove surgically. Recurrence is high after incomplete removal.

Figs. 1.134A and B: Large lipoma in the nape of neck.

Lipoma can be superficial or deep. , Superficial lipomas are more common; common in subcutaneous plane. It is commonly seen on back, neck, proximal extremities and abdomen. It is commonly less than 5 cm; but can attain large size. There is no gender predisposition. , Deep lipomas are commonly intramuscular, but often may be intermuscular; often both intra- and intermuscular (infiltrating lipoma). They are common in lower limbs (45%), trunk (17%), shoulder and upper limb. They attain large size compared to superficial lipomas. They are more common in men. Lipoma can be single or multiple. , Single lipoma is common. It is usually superficial in subcutaneous plane but can be deep also. , Multiple lipomas are 15% common; common in males (6:1). Common in back, shoulder and upper arm; can be symmetrical. It can be associated with many syndromes like multiple endocrine neoplasia (MEN), Cowden's, Frohlich, Proteus, Bannayan-Zonana syndromes. , Multiple lipomatosis represents a diffuse overgrowth of mature adipose tissue; extensively involves subcutaneous and muscular planes; common in shoulder and pelvic girdles. It is common in younger age group. 30% of them are familial; Adiposa dolorosa (Dercum's disease) is a multipl e lipomatosis mainly seen in females (30:1-female to male ratio); there is tender fat deposition especially in the trunk and girdles; it is common in epileptic and psychiatric patients. Benign tumour arising from brown fat is called as hibernoma (reddish brown), which has got serpentine vascular elements. Lipoma with fibrous component is called as fibrolipoma (most frequent nonlipomatous component in lipoma is fibrous tissue). Fibrolipoma is nonseptal. Lipoma with telangiectasis is called as naevolipoma. Neurolipoma (with nerve tissue and is painful), angiolipoma (with vascular element), myolipoma, chondroid lipoma, spindle cell lipoma, pleomorphic lipoma-are different types depending on the type of nonadipose component associated. Lipoblastoma is a benign tumour of immature fat occurring in infant boys in subcutaneous tissue of extremity. Benign lipomatous lesions may occur focally in a joint or tendon sheath or with diffuse villonodular proliferation in the synovium-/ipoma arborescens. Synovectomy may be needed in this patient. Malignant transformation of lipoma is non-existent. Liposarcoma does not arise from mature fat cells but from primitive mesenchymal cells. Sites

B

I Clinical Features Localised swelling, which is lobular (surface), nontender. Often fluctuant like feel but actually not (because fat in body temperature remains soft). It is usually nontransilluminant. Mobile, with edges slipping between the palpating fingers (slip sign). Skin is free. Lipomas may be pedunculated at times. It is rare in children. Pain in lipoma may be due to neural element or compression to nerves or adjacent structures. Angiolipomas being highly vascular is commonly tender. Trunk is the most common site; nape of neck and limbs are next common. Clinically lipoma can be single, multiple or diffuse.

Figs. 1.135A and B: Diffuse lipoma on the plantar aspect of foot and back.

ANATOMICAL SITES OF LIPOMA

• Subcutaneous; Subfascial • Intramuscular; lntermuscular in anterior abdominal wall Parosteal; Subserosal; Submucosal (GI tract) Extradural (not intradural); Intra-articular; Subsynovial; Subperiosteal, interosseous lntraglandular-breast, pancreas, kidney

Fig. 1.136: Slip sign of lipoma.

Eyes that look are common; eyes that see are rare.-J Oswald Sanders

69

.... m

70

• A diagnostic criterion used by some clinicians for superficial lipomas is hardening of swelling after application of ice. This test is of less important.

Fig . 1.137: Large lipoma in the back over scapula.

Figs. 1.138A and B: Pedunculated lipoma. It is common in axillary region. Lobular surface with narrow pedicle is typical. Often ulceration can occur in the surface due to repeated friction. It often mimics papilloma.

Differential diagnosis: ,. Neurofibroma-it moves horizontally but not longitudinally along the line of the nerve. Neurofibroma is firmer. , Cystic swellings like dermoid, sebaceous cyst. , Liposarcoma-all lipomas are benign. Large lipoma should be differentiated from liposarcoma. ,. Other soft tissue tumours. Investigations: Ultrasound or CT or MR imaging is done in deep or large or intracavitary lipomas; FNAC or incision biopsy is needed in large or deep or intracavitary lipomas to confirm it as benign. Complications ,- Myxomatous changes- occurs in retroperitoneallipoma. ,,, Saponification; Calcification-1 1% mineralization. ,. Submucosal lipoma can cause intussusception and so intestinal obstruction.

Figs. 1.139A and B: Large lipoma in buttock region.

B

LIPOSARCOMA

Common in retroperitoneum, thigh and back Rapid growth; Warm and vascular • Dilated veins over the surface • Infiltration into deeper plane with restriction of the mobility I ~kin fixation and fungation L Blood spread to lungs

---

Treatment: Excision-small lipoma is excised under local anaesthesia and larger one under general anaesthesia. Recurrence is 11 %.

Note:

• It is now considered that all sarcomas are of de nova in origin to begin with at mitochondrial level; so benign soft tissue tumour turning into sarcoma is not existing (except of neural origin like neurofibroma).

Fig. 1.140: Gross feature of specimen of lipoma.

Cyst can be single or multiple. Sebaceous cysts are often multiple.

CYSTS Cyst is a collection of fluid in a sac lined by epithelium or endothelium. Word meaning of cyst is "bladder" (Greek) (Greek word 'KUSTIS means bladder).

71

I True Cyst ....m

Cyst wall is lined by epithelium or endothelium. If infection occurs, cyst wall also will be lined by granulation tissue. Fluid is usually serous or mucoid derived from the secretion of the lining.

C\

It)

:::,

....

It)

I False Cyst

QI

1/'1

C

.... ....

It does not have epithelial lining. Fluid collection occurs as a result of exudation or degeneration.

IQ

It)

':'S

1/'1

Example:

Pseudocyst of pancreas. Wall of cystic swelling in tuberculous peritonitis. Cystic degeneration of tumour. After haemorrhage, in a haematoma, ABC's are lysed, gets absorbed and fluid remains as a false cyst. "Apoplectic cyst" is formed in brain as a result of ischaemia, causing collection of fluid.

:::,

IQ

Figs. 1.1 41A and B: Traumatic cyst thigh with

false cyst wall with clot inside.

I Effects of a Cyst Compression of adjacent structures. Example: Choledochal cyst compressing over the CBD. Infection; Sinus formation; Haemorrhage. Torsion, e.g. ovarian cyst. Calcification, e.g. hydatid cyst, cysticercosis. Cachexia: In malignant ovarian cyst patient goes for severe cachexia.

B a. Congenital cyst -

b.

c. d.

e. f.

Dermoids: Sequestration dermoid. Tubulodermoids: Thyroglossal cyst, postanal dermoid, ependymal cyst, urachal cyst. - Cysts of embryonic remnants: Cysts from paramesonephric duct and mesonephric duct, cysts of urachus and vitellointestinal duct. Acquired cysts - Retention cysts: They are accumulation of secretions of a gland due to obstruction of the duct, e.g. sebaceous cyst, Bartholin's cyst, cyst of parotid, breast, epididymis. - Distention cyst: Lymph cyst, ovarian cyst, colloid goitre. - Exudation cyst: Bursa, hydrocoele, pancreatic pseudocyst. Cystic tumours: Dermoid cyst of ovary, cystadenomas. Traumatic cyst: Due to trauma, haematoma occurs usually in thigh, loin, shin. It eventually gets lined by endothelium containing brown coloured fluid with cholesterol crystals. Degenerative cyst: Due to cystic degeneration of a solid tumour (due to necrosis of tumour). Parasitic cyst Hydatid cyst, trichiniasis, cysticercosis.

1

B (Wiliiiiiitiiiiilf;\i\l:ii1111MiiiillilJihiii1foiiiiJiiiI

r

Ranula

Hydrocoele

Cystic hygroma and lymph cyst

Meningocoele

Epididymal cyst ( Chinese-lantern pattern)

I Cl inical Features of a Cyst Hemispherical swelling which is smooth, fluctuant. nontender, well-localised. Some cysts are transilluminant. Presentation varies depending on its anatomical location and pathology.

Figs. 1.142A and B: Brilliantly transilluminant swellings-

possibly lymph cyst.

There can be no substitute for detailed appraisal of the history and clinical signs.- Harold Ellis

72

Nontransilluminating. Free skin, often adherent into the deeper plane. There will be resorption and indentation of the bone beneath. Impulse on coughing may be present only if there is intracranial extension. Differential diagnosis: Sebaceous cyst, lipoma, neurofibroma. Investigations: X-ray skull or part; CT scan of skull or part Treatment: Excision is done under general anaesthesia. Often formal neurosurgical approach is required by raising cranial osteocutaneous flaps.

DERMOIDS

I Types ...>. C1I

1. Sequestration Dermoids

...:::,Cl

It occurs at the line of embryonic fusion, due to inclusion of epithelium beneath the surface which later gets sequestered forming a cystic swelling in the deeper plane. It is congenital type.

In

0

ro

:::,

C

ro

:E

B a. b. c. [ d.

Forehead, neck, postauricular dermoid. External angular dermoid. Root of nose; Sublingual dermoid. Anywhere in midline or in the line of fusion . Dermoids occurring in the skull may extend into the cranial cavity. When it occurs as an external angular dermoid, it extends into the orbital cavity. It can extend into any cavity in relation to its anatomical location (e.g. thorax, abdomen).

B a. External angular dermoid: It is a sequestration dermoid situated over the external angular process of the frontal bone. Outer extremity of the eyebrow extends over some part of the swelling. This typical feature differentiates it from the swelling arising from the lacrimal gland. It may extend into the orbital cavity also (Frontozygomatic suture). b. Internal angular dermoid: It is a sequestration dermoid cyst in

,0)

1. Cyst outside skull bone

,~O

2a. Cyst with bone indentation 2b. With dura attachment

3

3. Cyst connecting inside skull with a stalk

2a.

~

·G

2b.

4. Entire cyst is intracranial-very rare

Fig. 1.143: Sequestration dermoid in skull-anatomical types.

central position near the root of the nose. It occurs in frontonasal suture line. It is rare. It mimics swelling from lacrimal sac or mucocele of frontal sinus. Mucocele of frontal sinus is due to blockage of frontonasal duct. Dermoid cyst contains putty like desquamated material, hair follicle, sebaceous and sweat glands. It is lined by both dermal and epidermal components. Dermoid cyst in skull region has different anatomical types as it often may extend into cranial cavity. , Cyst which is located entirely outside the skull bone over suture line but without bone indentation. , Cyst located outside the skull bone but with a bone defect underneath. Bone defect may be either on outer table of skull or through both tables of skull with attachment to dura. , Cyst lying partly outside and partly inside the skull with a connecting stalk between the two like a dumb bell. , Cyst is entirely within the skull bone between skull and dura. It is very rare but known.

Features Painless swelling in the line of embryonic fusion. Presents in the second or third decade onwards. Smooth, soft, nontender, fluctuant (Paget's test positive, i.e. swelling is fixed with two fingers and summit is indented to get yielding sensation due to fluid).

Figs. 1.144A and B: Types of angular dermoid. (A) External angular

dermoid; (B) Internal angular dermoid (midline).

73

_, m Fig. 1.148: Dermoid in the ear. It arises due to sequestration at the fusion line of the one of the six developmental ear tubercles (Each ear develops from six ear tubercles). Fig. 1.145: Postauricular dermoid in different patients.

SUBMENTAL DERMOID It Is a congenital sequestration dermo1d occurs dunng fusion of 1st and 2nd branchial arches. It is deep to deep fascia of neck. It presents as soft, cystic, fluctuant, nontransilluminating, swelling in midline in submental region which does not move with deglutition nor moves while protruding the tongue out. It should be differentiated from thyroglossal cyst, cold abscess from submental lymph nodes or sebaceous cyst. It is excised under general anaesthesia with a curvilinear submental incision.

2.

a, Figs. 1.146A and B: Dermoid in the midline-suprasternal space of Burns/sternum. It may extend into deeper plane and so it needs CT chest and evaluation.

Fig. 1.147: External angular dermoid, right sided. ---

COMPLICATION OF SEQUESTRATION DERMOID Infection, haemorrhage, rupture • Surface ulceration, calcification Pressure effects if there is intracavitary extension like into cranial cavity or thoracic cavity, etc.

Tubulodermoids

It arises from the embryonic tubular structures; it is due to accumulation of secretions of the lining of the unobliterated portion of congenital ectodermal tube/duct. Thyroglossal cyst: It is due to persistent thyroglossal duct during development of the thyroid gland. Ependymal cyst:They originate from the sequestration of the neuroectodermal cells. They are benign neuroepithelial cysts lined by ependymal cells. They are most commonly located deep in the parenchyma. Ependymal cysts have been postulated to be the entity responsible for the interhemispheric cysts with the Dandy walker cyst and agenesis of the corpus callosum. Large cyst can cause obstructive hydrocephalous. Post-anal dermoid: It develops from post-anal gut, i.e remnant of neuroenteric canal probably as a teratoma; located in front of the sacrum and coccyx. It is symptomless unless attains a large size or infected or causes pressure symptoms. Rectal examination reveals the cyst. It is treated by complete excision. Coccyx may need to be removed to have an easy access. Urachal cyst: It arises from the remnant of the urachus; presents as swelling in the midline at lower abdomen as extraperitoneal mass. It can get infected, or rupture into umbilicus or peritoneal cavity or calculi can form or adenocarcinoma can develop in the cyst.

3.

Implantation Dermoid Due to minor pricks or trauma, epidermis gets buried into the deeper subcutaneous tissue which causes reaction and cyst formation (trauma is forgotten often). It is an acquired cyst.

Count your blessing not your problems.

:::, 10

74

...>,

GI

El ::::,

V'I

0

IO

::::,

C: IO

:E

... QI

Ol ::,

Ill

0

ra

::,

C:

ra

"' cc a:

Ill

white in colour with brownish haemorrhagic flecks. It is often covered by apparently normal skin because wart is buried into the skin. It looks like a circular pit. It is grey white finger/ filiform like strands in the centre of the lesion and is soft. Plantar warts can be multiple. It is painful and very tender on pressing (more than callosity or corns). A defined clear lump may not be felt. Butcher's wart/pathologist's wart/verrucas necrogenica is due to entry of Mycobacterium tuberculosis through broken skin which are common in milkmaids. It presents as bluish red warty lesion from which fluid oozes out on pressure between projections. It is common over dorsum of hand. Lesion is surrounded by pustules. Senile warts and venereal warts are other types. Venereal warts are same as infective papilloma. Differential diagnoses are true papilloma, callosities, neurofibromas. Treatment: ,. Salicylic acid, podophyllin cream applications are also used. Laser ablation of warts is commonly advocated. ,. Excision is done if other methods fail.

GLOMUS TUMOUR It is also called as glomangioma. It arises from the cutaneous glomus composed of a tortuous arteriole which communicates directly into the venu le (Sucquet-Hoyercanal) and these vessels being surrounded by network of small nerves making this lesion very painful. This communication is called as Sucquet-Hoyer canal. Tumour consists of a mixture of blood spaces, nonmedullated nerve tissue, muscle fibres derived from the wall of the arteriole, with large cuboidal glomus cells-angiomyoneuroma. They are often seen in limbs and common in nail-beds near the finger tips, as purple red swelling. It is 2-3 mm in size. It does not turn into malignancy.

Fig. 1.176: Glomus tumour in finger tip-subungual region.

I Features Severe burning sensation and pain, out of proportionate to the size. The most common site is nail-bed.

Even the slightest pressure will give rise to severe pain. Dilated vessels compress over nerves. It is compressible and pain is more when the limb is exposed to sudden changes in temperature (cold stimulus). On increasing the pressure in the arm above systolic, pain disappears. It looks like a reddish blue spot which does not blanch on percussion. Subungualtype may not be visible but only to cause episodic digital severe pain . It is usually single, but rarely multiplicity is observed as familial. Differential diagnosis , Pyogenic granuloma-bleeds on touch. , Subungual melanoma-painless pigmented lesion. Treatment: Excision cures the condition.

BURSAE Bursa is a sac like cavity co ntaining fluid with in, wh ich in normal location prevents friction between tendon and bone. Bursa secretes synovia like clear fluid in a cavity lined by flat endothelium. It reduces the friction at the site between tendon and bone. Normally fluid content is little to cause a swelling. Minor trauma or infection causes sudden increase in fluid secretion of the bursa making it to enlarge and clinically palpable as pathological bursitis. Bursa is common around knee, elbow, heel and hip. Long-standing bursitis leads into thickening of its wall often with calcification making it feel hard with indurated surface. Lining of bursa may become rough or fluid may contain loose fibrinous particles to create grating sensation (crepitus) on the surface. Often overlying skin becomes thick, cracked and horny due to repeated friction and inflammation. Bursa may get adherent to deeper tissue as well as overlying skin to make it immobile. Bursa is usually well-localised, smooth, fluctuant, nontender swelling. Often it can be bilateral-in knee or elbow. Bursitis can become painful, tender and often with restricted movement once it gets infected. Joint related should be examined. Bursa may be communicating with the adjacent joint. Gout or rheumatoid arthritis can cause bursa. For example, olecranon bursa can develop in gout patient. Bursa should be differentiated from cold abscess, soft tissue tumour, aneurysm, synovial tumou r (sarcoma) at different locations. Complications of bursa: Infection of bursa can occur due to trauma to overlying skin or through blood; can cause mechanical disturbances and discomfort.

83

B • US of the anatomical site, X-ray of the part or MRI are very useful. • Avoiding friction and other aggravating factors may control many bursae. • Aspiration and steroid injection may be useful. • Bursa which is felt indurated with thick wall or calcified or infected or attained large size or which interferes with joint movement or daily activities needs surgical excision. Subcutaneous bursa can be excised under local anaesthesia; large or deeper bursa requires general anaesthesia for excision.

n

:::c l>

"'O

m ::c m

I Different Types It can be anatomical or adventitious.

Anatomical Anatomical bursae are located normally in a particular anatomical site with a purpose of reducing friction. They are commonly deep and adjacent to a bone or joint. They become pathological and clinically significant when it presents with bursitis. They are soft, cystic, well localised, nontransilluminating swelling at known anatomical site. , Subhyoid bursa. An horizontally oval swelling situated below the hyoid bone and in front of the thyrohyoid membrane. , Subacromial bursa: In front and lateral to humeral head in relation to supraspinatus tendon between acromion and greater tuberosity of humerus. , Bicipitoradial bursa. , Olecranon bursa (Student's elbow, Miner's elbow): It is subcutaneous bursa in relation to olecranon which becomes distended due to prolonged periods of leaning over elbow. Gout may involve this bursa.

Figs. 1.178A and B: (A) Prepatellar bursa (Housemaid's knee); (B) lnfrapatellar bursa (Clergyman's knee).

1. , - - -' t - -

,.,_.,..,,....---,-.,____-\-_ Prepatellar bursitis ~ ~----l~ lnfrapatellar bursitis

Fig. 1.179: Prepatellar (Housemaid's knee) and infrapatellar bursae (Clergyman's knee) .

,

Bursa

Fig. 1.177: Location of olecranon bursa (Student's elbow).

,

,

,

Prepatel/ar bursa (Housemaid's knee/miner's beat knee): It lies subcutaneously in front of lower half of patella and upper half of patellar tendon (upper part) undergoes inflammation in people who do much kneeling. Joint is normal here. Subcutaneous infrapatellar bursa occurs between skin and lower part of the tibial tuberosity and ligamentum patellae. It is called as Clergyman 's knee. ('Clergyman' is Christian priest who kneels down during prayer). Suprapatellar bursitis is deep to patella and vastus intermedius, in front of lower end of femur. It communicates with knee joint.

Suprapatellar bursa

, , , ,

Psoas bursa: A tensely cystic swelling situated beneath and below the inguinal ligament, in the lateral aspect of the femoral triangle. But it will not extend above the inguinal ligament into the iliac region (unlike in psoas abscess which extends above and is cross fluctuant). Psoas bursa lies between the psoas tendon and lesser trochanter. When it is enlarged, it causes diffuse swelling over outer part of femoral triangle lateral to femoral vessels. When hip is moved swelling becomes painful. It also should be differentiated from femoral hernia. Brodie's bursa lies deep to medial head of gastrocnemius. Semimembranosus bursa. Bursa anserin;runder the tendons of Guy ropes (sartorius, gracilis and semitendinosus tendons-Goose's too~. Retrocalcaneum burs;rbetween calcaneum and tendoAchilis.

I Adventitious Bursa Adventitious bursa occurs in an unusual place/sftedue to friction/ pressure between two layers of tissue. Once it becomes chronic it may get adherent to overlying skin or tissue underneath.

There is only one pretty child in the world, and every mother has it.- Chinese proverb.

84

,.. Billing gate hump appears over 7th cervical spine deep to overlying skin in people carrying weight over it. Billing gate is a large fish market in London. ,:. Condition should be differentiated from soft tissue tumour, sebaceous cyst, ganglion (depending on the location of bursa). Management: , X-ray of the part and FNAC of swelling should be done. ,.. Later it is excised usually under local anaesthesia.

SEMIMEMBRANOSUSBURSA

cc a: VI

V1

Fig. 1.180: Bursa near elbow joint.

It may get infected, wall gets calcified, and fluid may become thick. It is well-localised, cystic usually nontender swelling. It becomes painful and tender if infected. Lining of bursa may become rough or fluid may contain loose fibrinous particles to create grating sensation/crepitus on the surface.

Fig. 1.181: Adventitious bursa over lateral aspect of foot-

a common site.

Fig . 1.182: Common site of adventitious bursa (Bunion). Examples ,

Bunion is adventitious bursa in patient with hallux valgus

occurring between head of first metatarsal and skin. , Tailor's bursa occurs between lateral malleolus and skin. ,.. Porter's bursa occurs between skin over shoulder and clavicle. ,.. Weaver's bursa occurs between gluteus maximus, ischial tuberosity and skin. ,.. Retro-Achillis bursitis occurs between skin and Achilles tendon. ,.. Subcalcaneal bursitis occurs between calcaneum and heel in long distance runners.

It is a cystic swelling in the upper medial aspect of the popilteal fossa under the semimembranosus tendon. It is said to be due to friction under the tendon causing bursitis. It is located between semimembranosus tendon and femoral condyle above the knee joint line. It is common in you ng adult; in both sexes. It is most common swelling of the popliteal Iossa. It is nontender, cystic/tensely cystic (firm) swelling located above and on medial aspect of the popliteal fossa, fluctuant, noncompressible, often transilluminating, often with a fluid thrill. When it enlarges it comes out of semimembranosus tendon to become subcutaneous. ,:. Content of bursa does not communicate with knee joint. So fluid cannot be reduced into the joint cavity; but often appears flaccid on flexion of knee or by firm pressure probably due to displacement of fluid into deeper recesses of the bursa. The swelling becomes tense when knee is extended. Knee joint is normal. Differential diagnosis: Baker's cyst, popliteal aneurysm. Management , Ultrasound of popilteal fossa shows the cystic swelling under semimembranosus tendon. X-ray knee joint is normal. ,.. Excision is done under general anaesthesia using tourniquet in prone position. Complete excision of the sac is needed to prevent recurrence. Semimembranosus bursa- medial Semimembranosus Semitendinosus Gracilis (Guy ropes)

Bak.er's cyst below midline Medial head7 Gastrocnemius Lateral hea~

Figs. 1.183A and B: (A) Semimembranosus bursa typical location;

(B) Morrant Baker's cyst-typical location.

Management: X-ray of joint shows arthritic changes; MRI is needed; Arthritis is treated and Baker's cyst is excised under general anaesthesia or spinal anaesthesia in prone position.

85

LYMPHANGIOMA It is congenital localised clusters of dilated lymph sacs in the skin and subcutaneous tissue that has failed to join the normal lymph system during development period.

I Types Capillary Lymphangioma

Figs. 1.184A and B: Semimembranosus bursa-typical location. On flexion it only becomes flaccid but does not disappear.

MORRANT BAKER'S CYST It is acystic swelling containing gel like fluid in the lower midline of the popliteal fossa. It occurs due to herniation of the synovial membrane of the knee joint as a result of chronic arthritis. It is pulsion/pressure diverticulum of the synovial membrane towards surface under the gastrocnemius through an opening in the joint capsule. It is below the joint line. It is common in middle-aged individuals. It is smooth, soft and cystic, nontransilluminant, often tender swelling located below (the joint line) and in midline of the popliteal fossa. On flexion swelling disappears and on extension swelling increases in size. Pain and tenderness are present in knee joint with effusion showing positive patellar tap. The knee joint movements are painful and restricted. Baker's cyst may rupture sometimes causing severe sudden pain and swelling in the calf mimicking deep vein thrombosis.

Joint line

It is simple type which can be present at birth but noticeable skin vesicles often develop in few years. It is common at the junction of body to limbs-like near shoulder, axil la, groin or buttock. Skin vesicles contain clear watery or yellow fluid. Bleeding within the vesicle may turn it into brown or black. Its features includes multiple, indistinct white/brown/black coloured vesicles of 0.5-4 mm size at typical locations in children involving around 5- 20 cm area of skin in the particular location. If it is less than 5 cm in size it is called as lymphangioma circumscriptum.

Fig. 1.186: Lymphangioma circumscripta (Courtesy: Dr Balasaraswathy, DVD, DNB, Consultant Dermatologist, Mangaluru)

If it is more than 5 cm in size it is called as lymphangioma diffusum. If it is with reticulated ridges, it is called as lymphoedema ab igne. Area is soft, spongy, often fluctuant with fluid thrill and translucency. It is not compressible. Vesicles will not fade on pressure. Often lesion may get infected to make it painful and tender. Condition will not block the lymph drai nage in normal lymphatics and skin oedema is absent. Regional lymph nodes are not enlarged.

Cavernous Lymphangioma It is soft, lobulated, fluctuant, brilliantly transil/uminant larger lymphatic swelling with often multiple communicating lymphatic cysts. It often extends into deeper plane like muscle. It is common in face, mouth, lips (macrocheilia), tongue (macroglossia). Figs. 1.185A and B: Baker's cyst. It is below the joint line.

Differential diagnosis: Semimembranosus bursa; Thrombosed popliteal aneurysm (often bilateral).

Cystic Hygroma It is collection of clustered sequestered lymph sacs (occurring during developmental period in utero) presenting in newborn as

The mother's heart is child's schoolroom.-Henry Ward Beecher.

....m

88

large swelling which is soft, smooth, fluctuant, brilliantly transilluminant, and compressible (For detail refer Chapter 5-Neck).

LYMPH CYST (LYMPHATIC CYST)

cc"'

a: V'l

EPIGNATHUS This is a type of growth anomaly seen in neonates wherein growth from the base of skull protrudes through the mouth.

It is an acquired type of distension cyst wherein lymphatics form a localised swelling with a capsule around it. It usually occurs in subcutaneous plane, which is smooth, soft, nontender, mobile, and brilliantly transilluminant. It is usually not adherent to the skin. Common sites are neck and limbs. It can get infected and form an abscess. •·· Differential diagnosis: Cold abscess, dermoid cyst. Treatment: Excision.

CALCINOSIS CUTIS It is a type of calcification (dystrophic) in or under the skin. Usually presents as a circumscribed lesion in the skin. Commonly seen in females. Common site is in the waist (Fig . 1.188) Usually bilateral. It is said to be due to friction causing degeneration of skin and immediate deeper structure with increased local alkalinity of the tissue causing precipitation of the calcium leading to solid , hard, swelling in the skin. Cut section shows hard, yellowish material. It may mimic calcified lipoma or neurofibroma. Treatment is excision and closure of defect often with local flaps.

Fig. 1.188: Epignathus. Note: • Enucleation is removal of the swelling within the tissue of origin with normal part of tissue of origin is being retained, e.g. enucleation of prostate in benign prostatic hyperplasia (BPH). • Excision is removal of tissue/tumour entirely with its capsule. • Wide excision is removal of tumour with surrounding tissue margin adequately for clearance. • Compartment excision is removal of tumour/diseased tissue with all adjacent soft tissues in one compartment except neurovascular bundle. It is done in limbs for soft tissue sarcoma as a curative but limb saving procedure. • Radical excision/radical block dissection is removal of tumour widely with adjacent soft tissues with lymph node dissection.

------------------------.::::::=:>

Elliptical incision partially occupying the swelling - includes the punctum in this

------------r'\

Straight incision over swelling

Fig. 1.187: Calcinosis cutis near waist is a common site. It is common in females.

Elliptical incision occupying the entire swelling (circumferential)

CHORDOMA Chrod oma is a slow growi ng tu mour arising from notochord. It can be classical , chondroid and dedifferentiated. It is commonly seen in sacrococcygeal, sphenoid sinus (clivus) and foramen magnum region. It invades the surrounding structures. It often attains large size. MRI is diagnostic. Treatment is wide excision. Radiotherapy is less usefu l but highly focused proton or carbon ion radiation is effective than conventional X-ray radiation.

Fig. 1.189: Different incisions used in surgical approaches to remove swelling.

I~

Psoas ' " " "

, m1oc,lac hydcocoele

• Ranula (plung_i_ng_)_ _ _ _•_ Compound palmar ganglion

j'

F. Electrolyte and Nutrition

....

.... ....

( iHAP TER OUTLINE

..

..• .... •

..•• •

Normal Physiology Water Loss (Volume Loss) Water Excess (ECF Volume Excess) Hyponatraemia Hypernatraemia Hypokalaemia Hyperkalaemia Hypermagnesaemia Hypomagnesaemia Acid-Base Balance Metabolic Alkalosis Respiratory Alkalosis

• • •

.. ....

Metabolic Acidosis Respiratory Acidosis Anion Gap Fluid Therapy Nutrition Gastrostomy Jejunostomy Total Parenteral Nutrition Refeeding Syndrome Obesity and Morbid Obesity Different Surgeries

Total body water is 60% of body weight in males, 50% of body weight in females, i.e. 30 litres. Intracellular water-20 litres (2/3). Extracellular water-10 litres (1/3). ,.. Plasma (1/4) (2.5 litres). , Interstitial fluid (7.5 litres). ICF 10 mmol/L 150 mmol/L

Trace only

I Management Evaluation is done by estimating serum sodium, urinary sodium, and blood urea. Isotonic volume depletion is corrected by infusion of 0.9% normal saline. Pure water depletion is corrected by more water intake/ intravenous 5% dextrose. Monitoring fluid therapy by skin and tongue examination, weight gain, pulse, blood pressure, CVP, PCWP.

WATER EXCESS (ECF VOLUME EXCESS)

NORMAL PHYSIOLOGY

Ion Sodium Potassium Chloride

Only pure water loss occurs due to poor fluid intake and diabetes insipidus. It causes dehydration with proportionate decrease in total body water (213rd ICF, 113rd ECF). As ECF including intravascular fluid loss is less, hypotension is less. Features here are-severe thirst, confusion and convulsions due to hypernatraemia; blood pressure is relatively normal. Dehydration can be mild (weight loss 5%); moderate (10%); severe (15%).

ECF and plasma 140 mmol/L 4.5 mmol/L 105 mmol/L

ECF volume and osmolality regulation is controlled by three hormones. Aldosterone, ADH, atrial natriuretic hormone.

WATER LOSS (VOLUME LOSS) It is decrease in the whole body fluid volume which includes both ECF and ICF. It is usually ECF loss which is more important and assessed. It can be isotonic volume depletion with both salt and water loss leading into hypovolaemia, or only water loss with only min imal loss of electrolytes leading into dehydration.

I Causes and Features Isotonic volume depletion occurs due to diarrhoea, vomiting, and excess diuresis. Here normal or decreased sodium is observed. Fluid loss is only of ECF and so early intravascular volume reduction occurs. This causes hypotension and decreased tissue perfusion. Features are-dry tongue, rapid pulse, cold clammy extremities, sunken eyes, hypotension, oliguria, raised blood urea, decreased urinary sodium. Hypovolaemia can be mild (3 L fluid loss).

It can be divided into water and salt excess or predominantly water excess called as water intoxication. Water and salt excess occurs in CCF, cirrhosis, nephrotic syndrome, hypoproteinaemia, renal failure, excessive saline infusion. Water intoxication occurs in TURP, excess infusion of 5% dextrose only, SIADH secretion, psychogenic polydypsia. It is managed by stopping fluid infusion or procedure (TURP); fluid restriction, and treating the cause. Causes , Excessive amount of intravenous dextrose (5%). , During colorectal bowel wash for preparation of large bowel for surgery, if water is used instead of saline, especially in children. , In transurethral resection of prostate (TURP) when excess irrigating fluid water or glycine is used (commonly used). ,.. In syndrome of inappropriate antidiuretic hormone (SIADH) which is commonly associated with lobar pneumonia, empyema, oat cell carcinoma and head injury.

Drowsiness, weakness; Convulsions and coma • Nausea, vomiting; Passage of dilute urine • Distended neck veins; Pedal oedema • Gain in body weight- most sensitive and consistent sign • Circulatory overload-tachycardia, pulmonary oedema, hypertension Bilateral basal crepitations, ascites; Raised CVP, PCWP Investigations: Haematocrit and sodium level (will show fall in level); Low potassium. Low blood urea. Treatment: , Water and salt restriction and observation.

Move to the rhythm of soul and you'll never miss a beat.-Vicki Virk

88

,. Monitoring in ICU. , Management of fluid and electrolyte balance. , Infusion of hypotonic sodium chloride. Note:

• Administration of diuretics and hypertonic saline should beavoided, as it may cause rapid changes in serum sodiumand water level which will lead to neuronal demyelination and fatal outcome. • ECF loss: Here only ECF loss is present with normal !CF.It is seen in vomiting, diarrhoea, intestinal obstruction. Treatment is infusion of normal saline. • ECF excess: Only ECF excess without an ICFexcess. Features areexcessive infusion of saline with impaired excretion; raised JVP (earliest and best clinical sign), cardiac failure and peripheral oedema. Treatment is fluid restriction and diuretics like frusemide.

HYPONATRAEMIA Sodium level is less than 130 mEq/L. Hyponatraemia is said to be severe if serum sodium becomes lesser than 100 mEq/L in acute type; and lesser than 115 mEq/L in chronic type. It can be due to water overload (dilutional) or sodium loss.

B Acute-presents as neurological manifestations. Chronic-causes pontinemyelinolysis. It presents as behavioural changes, progressive weakness, and cranial nerve palsies.

Types also may be: Hypervolaemic hyponatraemiawherein rapid absorption of fluid occurs into intravascular compartment leading into pulmonary and cerebral oedema. It is due to decreased osmolality causing movement of ECF into the cells. Serum sodium level lesser than 100 mmol/L is called as severe hyponatraemia, causes convulsions. Here urinary sodium will be less than 15 mmol/L. Acute hyponatraemia is corrected by fluid restriction, hypertonic saline, loopdiuretics like frusemide. Monitoring the serum sodium level of the patient is essential. Sodium should be corrected up to above the level of 125 mmol/L. Correction should be slow and gradual at a rate of 2 mEq/L/h with up to 20 mEq/L correction in 24 hours with 4th hourly assessment of serum sodium. Overcorrection of sodium should not be done. Rapid correction can lead into irreversible myelin lysis of pontine. Hypovofaemic hyponatraemia: It is due to hypovolaemia by diarrhoea, vomiting, wherein urine sodium level is less than 20 mmoVL; due to diuresis or renal causes wherein urine sodium level is more than 20 mmoVL or it may be due to correction of hypovolaemia using hypotonic fluid like 5% dextrose. Condition can be treated well using isotonic normal saline. Normovolaemic hyponatraemia: It may be due to renal failure or synd rome of inappropriate ADH secretion (SIADH). In mild asymptomatic patients it is corrected by fluid restriction (1 Uday will raise the serum Na). Vasopressin antagonist demecfocycfine which increases the diluting ability of kidney is used in severe cases. Pseudohyponatraemia: Plasma osmolality is mainly achieved by serum sodium; but small proportion, i.e. 25% of osmolality is due to other solutes like glucose, lipids, plasma proteins, urea which will not move easily between intracellular and extracellular spaces. When concentration of these molecules

raise due to some pathology, proportionately relative concentration of sodium will drop causing pseudohyponatraemia. Here condition causing related to specific solutes mentioned above is treated, than hyponatraemia.

I Causes Intestinal obstruction. Intestinal fistulas-biliary, duodenal, gastric, pancreatic. Gastric outlet obstruction with severe vomiting. Ryle's tube aspiration; Severe diarrhoea due to viral cause, in colitis, colorectal polyps. Syndrome of inappropriate antidiuretic hormone (SIADH). Immediately after surgery and trauma, sodium depletion occurs. Stroke.

B Dry coated tongue; Sunken eyes; Dry wrinkled skin Hypotension; Dark scanty urine; Convulsions Irritability, disorientation and neurological manifestations In chronic hyponatraemia-hypothermia, reduced tendon reflexes, pseudobulbar pasty

Investigations: Serum electrolytes. Urinary sodium is low; Sodium deficit is calculated by: (125 - present serum sodium) x body weight in kg x 0.6. Treatment ,. Intravenous infusion of normal saline as a slow and gradual correction at a rate of 2 mEq/L/hour in acute cases and 150 mEq/L. Excess infusion of normal saline causes overload in circulating salt and water. It is usually due to water deficit. Causes: Renal dysfunction; Cardiac failure; Drug induced like NSAID, corticosteroids. It may be either primary sodium excess or primary potassium excess or primary water deficit.

B

TYPES OF HYPERNATRAEMIA

Euvolem,c (pure water loss). It Is due to failure of water intake like in comatous patients, bedridden people, postoperative patients and in patients with high fever leading into extrarenal loss of water. It can occur in diabetes insipidus or chronic renal failure as renal loss of water. Hypovolaemic (among loss of water and sodium, more water is lost than sodium): It is due to vomiting, diarrhoea, more undue sweating (extrarenal); osmotic diuresis by glucose/mannitol (renal). Hypervolaemic (both sodium and water gain but sodium gain is more than water gain) as seen in more salt intake, excess steroids, sodium bicarbonate/hypertonic saline infusion (salt gain).

Features: Pitting oedema; Puffiness of face ; Increased urination; Often dilated jugular veins; Features of pulmonary

oedema. Investigation: Serum electrolytes, plasma and urine osmolality, renal function tests, haematocrit. Management: , Restriction of saline and sodium. Treatment of pulmonary oedema. , Hypernatraemia should be corrected slowly as follows: Initial infusion of normal saline, then infusion of half strength saline (0.45%) and later with 5% dextrose, i.e. gradual controlled correction is done. Otherwise cerebral oedema and hyperglycaemia can develop. Oral and nasogastric administration of water/fluids.

Causes , Renal failure; Rapid infusion of potassium. , Transfusion of stored blood; Diabetic ketoacidosis. , Adrenal insufficiency; Metabolic acidosis. Potassium sparing diuretics, cyclosporine, beta blockers. ,. Insulin deficiency. , Tissue destruction, burns, trauma, tumour necrosis, crush injury. , In vitro haemolysis, thrombocytosis, tourniquet application, exercise-pseudohyperkalaemia. , Familial hyperkalaemic periodic paralysis. Note: Potassium excess is a dangerous condition which can cause sudden cardiac arrest.

HYPOKALAEMIA

I Sudden Hypokalaemia Serum potassium level less than 3.5 mEq/L. It occurs in patients in diabetic coma treated by insulin and saline infusion.

I Gradual Hypokalaemia Causes Diarrhoea of any causes, villous tumour of the rectum, ulcerative colitis; After trauma or surgery. Pyloric stenosis with gastric outlet obstruction. Duodenal fistula, ileostomy; After ureterosigmoidostomy. Insulin therapy; Poisoning; Drugs like beta agonists. Familial periodic paralysis.

Features Slurred speech; Muscular hypotonia-physical sign. Depressed reflexes; Paralytic ileus. Weakness of respiratory muscles; Cardiac arrhythmias. Inability to produce concentrated urine and so causes nocturia and polyuria. ECG shows prolonged QT interval, depression of the ST segment and inversion of T wave, prominent U wave. Often hypokalaemia is associated with alkalosis. Serum potassium will be decreased. Treatment , Oral potassium 2 g 6th hourly, 15 ml potassium chloride syrup (20 mmol of K). , IV KCI 40 mmol/L given in 5% dextrose or normal saline slowly, often under ECG monitoring [Total dose is 40 mmol (0.2 mmol/kg/hour). Maximum dose per hour is 20 mmol]. , Hypokalaemic alkalosis which occurs in pyloric stenosis should be treated carefully by IV potassium as there will be severe potassium loss.

HYPERKALAEMIA Normal range of potassium is 4.0 to 4.5 mEq/L. Hyperkalaemia manifests when potassium exceeds 6 mEq/L.

I Management High serum potassium level. Peak 'T' wave in an ECG. IV administration of 50 ml of 50% glucose with 10 units of soluble insulin, slowly. Infusion of 10% calcium gluconate slowly (as cardioprotection) intravenously. Calcium chloride is given in severe cases as calcium in this form is released immediately without hepatic metabolism. Diuresis using frusemide injection. Haemodialysis when required-very useful. Continuous ECG monitoring is a must. Polyesterene sulphonate ion exchange resin 30 g/hour in 50 ml of 70% sorbitol as an enema. Salbutamol nebulisation or intravenously 0.5 mg in 4 ml of saline/Albuterol nebulisation. IV sodium bicarbonate-shifts potassium in to cells. 7.5%, with 50-100 ml intravenously in 10 minutes.

HYPERMAGNESAEMIA It is rare. Serum magnesium >2.5 mEq/L. Normal serum magnesium is 1.5-2.5 mEq/L and intracellular magnesium which is more (2nd higher) is 26 mEq/L. Magnesium is mainly deposited in bone (60%). It is a cofactor for many enzymes necessary in phosphorylation of glucose in the cell and ATP utilisation in muscle fiber. Daily required dietary intake of magnesium is 0.4 gram. It is reabsorbed well in proximal renal tubule. Causes: Advanced renal failure treated with magnesium containing antacids, diabetic ketoacidosis; Intentionally produced hypermagnesaemia while treating preeclampsia. Features: ,. Loss of tendon reflexes (most common). ,. Neuromuscular depression; Flaccid quadriplegia. ,. Respiratory paralysis; Somnolence; Hypotension.

You cannot harm a bandaged wound.- Croatian Proverb

89

n

:I: )> "'O

-i

m

:a

....

"T1

VI C: I.C (I)

':':

m

in

n

.-+

0 (I) QI

::::,

z

C:

.-+

;:;:

5· ::::,

90

"' co cc:

V\

HYPOMAGNESAEMIA Serum magnesium '"O

m

....::J:J

'Tl

"....

It)

:::,

It) Qj

VI

C: .... \0 It) ....

':':

!!!

...".... It)

0

It) Qj

:::,

Q.

...;::.·....z C:

o· :::,

• Anaphylactoid reactions can occur Renal impairment should be observed It has no 02 carrying capacity; so should not allow haematocrit to fall below 30% • Impairment of coagulation is possible

.

.

96

Methods of Enteral Feeding

NUTRITION

I Principles of Nutrition QI

...en::::,

-"' 1/'1

0

::::, C:

"' "' c:o a: 1/'1

Avoiding of malnutrition is the basic goal in nutrition therapy as malnutrition increases the morbidity and mortality of the disease process and prevents or delays the recovery. Malnutrition increases the chance of sepsis, delays wound healing, increases the respiratory complications, and decreases the efficacy and tolerance to radiotherapy or chemotherapy. Whenever possible enteral route of nutrition should be used ideally. If that is not possible then parenteral nutrition is used. Overfeeding should be avoided as it leads into hyperglycaemia, hepatic steatosis, raised BUN, and excess CO2 production. Timing and type of nutrition is also important. Nutrition therapy reduces protein wasting. Jmmunomodulators like glutamine, arginine and omega 3 fatty acids are also very useful. Glutamine is a nonessential amino acid synthesised in skeletal muscle. It is essential for cell proliferation during tissue repair. Glutamine helps GI mucosal cell proliferation, maintains mucosal integrity, improves immune function and prevents translocation of bacteria. It is useful in inflammatory bowel disease, short gut syndrome, burns, major trauma, and sepsis. Glutamine is used·commonly by enteral route even though IV preparations are now available (but it is very unstable in solutions).

Gastrointestinal tract is the best route to provide nutrition. Enteral feeding can be delivered by bolus, by gravity or using mechanical pump. , By mouth: Requi res-commo n sense, cleanliness, compassion . , By nasogastric tube: Confirmation of the tube in the stomach is made by injecting 5 ml of air down the tube and listening through a stethoscope for its bubbling entry into the stomach. Feeding rate is 30-50 mUhours. A time gap of 5 hours in the night is given to allow gastric pH to return to normal. Nole:

Problems with tube feeding are: Blockage; nausea and vomiting, aspiration; hyperosmolarity; diarrhoea; tube discomfort; Cholestasis , By enterostomy: Gastrostomy; jejunostomy. Different preparations and formulas are available for enteral feeding. Soluble fibre containing diets along with nutrients are better to prevent diarrhoea.

Nole: • Caloric requirement Neonatal 100 kcal/kg/day; Adult 40 kcal/kg/day;

Adult with catabolism 60 kcal/kg/day.

• It is given as: Carbohydrates 50%; Fat 3D-40%; Protein 10-15%. • Caloric values: Carbohydrate 4 kcal/g; Protein 4 kcal/g; Fat 9 kcal/g.

I Indications for Nutritional Support Preoperative nutritional depletion. Postoperative complications: Sepsis, ileus, fistula. Intestinal fistula: High type wherein output is more than 500 mUday. It may be duodenal, biliary, pancreatic, intestinal. Pancreatitis, malabsorption, ulcerative colitis, pyloric stenosis. Anorexia nervosa and intractable vomiting. Trauma-multiple fractures, fasciomaxillary injuries, head and neck injuries. Burns, malignant disease; renal and liver failure. Massive bowel resection causing short bowel syndrome.

• Body weight • Triceps skin fold thickness Lymphocyte count

• Mid-arm circumference Serum albumin

Nutriti onal requ irements: Carbohydrates, fat, proteins, vitamins (includes fat-soluble vitamins also), minerals, trace elements.

Fig. 1.190: Nasogastric tube passed should be confirmed in place using stethoscope. Tube is used for feeding purpose.

B • Aspiration, wound infection and leak • Diarrhoea due to rapid feeding or hyperosmolarity Hyperglycaemia, hypokalaemia Refeeding syndrome due to severe hypokalaemia and hypophos- 1 phataemia Advantages of enteral nutrition: , Enteral nutrition preserves mucosal protein, digestive enzymes, lgA secretion; prevents mucosa! atrophy and bacterial translocation. , It is more physiological as nutrients pass through liver, the first filter to process and store. Gallstone formation is prevented (unlike long-term TPN) by stimulating gallbladder motility. , It has got less serious complications. It is cost-effective. , It supplies glutamine and short chained fatty acids to gut. Contraindications of enteral nutrition , Intestinal obstruction, GI bleed, paralytic ileus, severe diarrhoea, high output fistula. , Low cardiac output, haemodynamically unstable patient.

,, If safe access to enteral feeding is not present. ,. Anticipated complications if thought to be present should be avoided.

GASTROSTOMY It is the procedure wherein a tube is passed into the stomach per abdominally for the purpose of enteral feeding. Indications: Severe malnutrition; major surgeries; severe sepsis; trauma; major head and neck surgeries; any conditions where tube feeding is required for more than 4 weeks (e.g. burns, severe sepsis). It keeps the small bowel function active. Contraindications: Previous gastric surgeries; intestinal obstruction; gastric outlet obstruction or gastric diseases.

Gastrostomy tube

2. Kader-Senn temporary gastrostomy: It is serosal lined temporary type similar to Stam m's but instead of purse string sutures, seromuscular interrupted sutures are placed from stomach to peritoneum adjacent. 3. Percutaneous endoscopic gastrostomy (popular; Now becoming common method (Figs. 1.1 98A and B).

"'Cl

--t

m

....

"T1

4. Janeway's mucus lined permanent gastrostomy by creating mucosal entire thickness tunnel in the stomach wall. Problems in gastrostomy tube: ,. Leak from gastrostomy site-gastric fistula. ,. Trauma to other organs like colon, spleen. ,. Infection, aspiration and pneumonia. ,. Diarrhoea is common (30%) ; bloating , abdomi nal cramps. ,. Displacement, blockage of the tube.

JEJUNOSTOMY Figs . 1.192A and B: Types of gastrostomy: (A) Temporary gastrostomy; (B) Permanent gastrostomy.

n

:I:

:::0

Figs. 1.193A to C: Percutaneous endoscopic gastrostomy (PEG). It is easier and acceptable method and less invasive.

Fig. 1.191 : Gastrostomy tube in place for enteral feeding.

97

Jejunostomy for enteral nutrition is becoming more popular because of-its comfort, easy to do, can be kept for long time, lesser complication than gastrostomy.

Types ,. Based on duration of use: Temporary or permanent. ,, Based on lining: Mucus lined (permanent) or serosal lined (temporary). ,. Based on technique: 1. Stamm temporary gastrostomy: After opening the abdomen, anterior wall of the stomach is opened . Feeding tu be (Malecot'scatheter) is placed in position. Two layers of purse string suturesare put around the tube. Wound is closed. It is serosal lined temporary type. In Witzel type serosal fold tunnelling is done around the gastrostomy tube.

We will not know unless we begin.

Fig. 1.194: Needle jejunostomy.

98

...>...:::::,C'I QI

V'I

111+-

Gastroscope

0

-

Guidewire

:::::, C:

Abdominal wall

Abdominal wall

cc a: VI

V'I

, •

Gastrostomy c,theter (tube)

.1..

Guidewire Trocar

1:--1-+---

{1,esophagus

"'C

-I

m

::IC

.....

'Tl

C\ l't)

::::,

...

l't)

DI

VI C

\Q

... l't)

-:-;

m

ii>

n ,... 0 -

""C

-I

m

....::::0 C'I

Brain perfusion, when decreases the patient becomes drowsy. Brain is the last organ to get underperfused in shock. Kidneys: GFR decreases and tubular reabsorption of salt and water increases for compensatory response. But in severe cases tubular necrosis sets in leading into irreversible damage. Blood: Alteration in cellular components including platelets leads to Disseminated intravascular coagulation (DIC). It causes bleeding from all organs. Gastrointestinal tract: Mucosa! ischaemia develops causing bleeding from GIT with haematemesis and malaena. It is agg ravated by DIC. Hepatic ischaemia leads into increased enzyme levels.

I Types of Shock 1. Vasovagal Shock It is sudden dilatation of peripheral and splanchnic vessels causing reduced cardiac output and shock. Often it may be life-threatening due to hypoxia.

2. Neurogenic Shock It is usually due to spinal cord injury, which causes dilatation of splanchnic vessels. ,,. This type can safely be treated with vasoconstrictor drugs to bring up the blood pressure. There will be bradycardia, hypotension, arrhythmias, and decreased cardiac output. Blood pressure control, oxygen delivery, maintenance of haemodynamics, airway, fluid therapy, intravenous methylprednisolone therapy should be done. Dopamine and or phenylephrine (a agonist) can be used.

3. Hypovolaemic Shock Haemorrhage, may be due to injury to the liver, spleen, bone fractures, haemothorax, vascular injury, severe bleeding on table during surgeries of thyroid, liver, portal vein or major vessels. Vomiting, diarrhoea due to any cause. Burns.

B a. Covert compensated hypovo/aemia {Mild 40%j. Here all features of hypovolaemia are present like hypotension, tachycardia, sweating, tachypnoea, oliguria, drowsiness, eventually features of SIRS is seen and often if not treated on time leads to MODS, i.e. irreversible shock.

4. Cardiogenic Shock Cardiogenic shock is defined as circulatory failure causing diminished forward flow leading into tissue hypoxia in the setting of adequate intravascular volume with systolic blood pressure 50%. ,,. Cardiogenic shock develops within 24 hours of Ml. It occurs when 50% of left ventricular wall is damaged by infarction. ,. It leads to pulmonary oedema and severe hypoxia. lschaemic necrosis of left ventricular wall causes failure of pump thereby decreasing stroke volume. Diagnosis is established by ECG, echocardiography, arterial blood gas analysis, cardiac enzymes, PCWP and electrolyte estimation (hypokalaemia and hypomagnesaemia are common) are the essential investigations. Management

Proper oxygenation with intubation, ventilator support, cardioversion, pacing, antiarrhythmic drugs, correction of electrolytes, avoiding fluid overload, prevention of pulmonary oedema as immediate measures. Dobutamine (131 receptor agonist) is used to raise cardiac output provided there is adequate preload and intravascular volume (it is peripheral vasodilator and reduces BP). Dopamine is preferred in patients with hypotension. But it may increase peripheral resistance and heart rate worsening cardiac ischaemia. Often both dopamine and dobutamine combination may be required. Careful judicial use of epinephrine, norepinephrine, phosphodiesterase inhibitors (amrinone, milrinone) are often needed. Anticoagulants and aspirin are given. Thrombolytics can be used. 13 blockers, nitrates (nitroglycerine causes coronary arterial dilatation), ACE inhibitors are also used. Intra-aortic balloon pump (IABP) may need to be introduced transfemorally as a mechanical circulatory support to raise cardiac output and coronary blood flow. Relief of pain, preserving of remaining myocardium and its function, maintaining adequate preload, oxygenation, minimizing sympathetic stimulation, correction of electrolytes should be the priorities. Percutaneous transluminal coronary angioplasty (PTCA) and coronary artery bypass graft (CABG) are the final choices.

5. Cardiac Compression Shock

I I

It is probably due to pericardia! tamponade of any cause or kinking of great vessels, massive pulmonary embolism, tension pneumothorax, air embolism causes obstructive shock with reduced preload to heart. Acute massive pulmonary embolism from a thrombus or an air embolism (50 ml of air ), obstructing more than 50% of pulmonary vasculatureleads to severe shock and sudden death. Tachycardia, hypotension, pulmonary oedema, raised JVP, gallop rhythm are the features.

6. Septic Shock Septic shock may be due to gram-positive organisms, gram negative organisms, fungi, viruses or protozoa! origin. Gram-negative septicaemia/gram-negative septic shock is called as endotoxic shock. It occurs due to gram-negative bacterial infections, commonly seen in strangulated intestines, peritonitis, gastrointestinal fistulas, biliary and urinary infections, pancreatitis, major surgical wounds, diabetic wounds and crush injuries. Gram-positive septic shock

Gram-negative septic shock

• Due to exotoxin by gram +ve bacteraemia like Clostridium tetani/welchii, staphylococci, streptococci pneumococci • Fluid loss, hypotension is common; with normal cardiac output

Gram-negative bacteria cause endotoxaemia and its effects. Urinary/gastrointestinal/ biliary and respiratory foci are common

Pathophysiology of septic shock Toxins/endotoxins from organisms like E. coli, Klebsiella, Pseudomonas, and Proteus ,l. Inflammation, cellular activation of macrophages, neutrophils, monocytes ,l. Release of cytokines, free radicals ,l. Chemotaxis of cells, endothelial injury, altered coagulation cascade-SIRS ,l. Reversible hyperdynamic warm stage of septic shock with fever, tachycardia, tachypnoea ,l. Severe circulatory failure with MODS (failure of lungs, kidneys, liver, heart) with DIC ,l. Hypodynamic, irreversible cold stage of septic shock. Septic shock is typically a vasodilatory shock wherein there is peripheral vasodilatation causing hypotension which is resistant to vasopressors. This is due to toxin-induced release of isoform of nitric oxide synthetase from the vessel wall which causes sustained prolonged release of high levels of nitric oxide. Magnitude of infection is quantified as: 1. Mild sepsis which shows fever, tachycardia, leucocytosis. 2. Severe sepsis which shows low tissue perfusion with organ dysfunction (lactic acidosis, dysfunction of liver, kidney, lungs). 3. Septic shock with systemic hypotension (BP 2 mmol/L. Note: • Sepsis is life-threatening organ dysfunction caused by a dysregulated host response to infection identified by the presence of 2 or more SOFA points [scores] (Sequential [sepsis related) organ failure assessment). Quick SOFA (qSOFA) score is also used-low blood pressure 22/minute; altered mentation-GCS '"0

-4

... m

:::0

Cl

11)

::,

11) .... QI

VI

Stages of septic shock a. Hyperdynamic (warm) shock: This stage is reversible stage. Patient is still having inflammatory response and so presents with fever, tachycardia, and tachypnoea. Pyrogenic response is still intact. Patient should be treated properly at this stage. Based on blood culture, urine culture (depending on the focus of infection), higher antibiotics like third generation cephalosporins, aminoglycosides, metronidazole are started. The underlying cause is treated like draining the pus, laparotomy for peritonitis, etc. Ventilatory support with ICU monitoring may prevent the patient going for the next cold stage of sepsis. b. Hypodynamic hypovolaemic septic shock (cold septic shock): Here pyrogenic response is lost. Patient is in decompensated shock. It is an irreversible stage along with MODS (multiorgan dysfunction syndrome) with anuria, respiratory failure (cyanosis), jaundice (liver failure), cardiac depression, pulmonary oedema, hypoxia, drowsiness, eventually coma and death occurs (Irreversible stage). Treatment of septic shock Correction of fluid and electrolyte by crystalloids, blood transfusion. Perfusion is very/most important. Appropriate antibiotics-third generation cephalosporins/ aminoglycosides. Treat the cause or focus-drainage of an abscess; laparotomy for peritonitis; resection of gangrenous bowel; wound excision. Pus/urine/discharge/bile/blood culture and sensitivity for antibiotics. Critical care, oxygen, ventilator support, dobutamine/ dopamine/noradrenaline to maintain blood pressure and urine output. Activated C protein prevents the release of inflammatory mediators and blocks the effects of these mediators on cellular function. Monitoring the patient by pulse oximetry, cardiac status, urine output, arterial blood gas analysis. Short-term (one or two doses) high dose steroid therapy to control and protect cells from effects of endotoxaemia. It improves cardiac, renal and lung functions. Single dose of

ldeas have a short shelf life-that's why we must act before the expiry date. (

C .... 11) ....

':':

VI -::I"

0

n .,,_.

108

methylprednisolone or dexamethasone which often may be repeated again after 4 hours is said to be effective in endotoxic shock.

>. ,._

Note: • Shock index is ratio of pulse rate to blood pressure; normal shock index is 2 mEq/L suggest tissue ischaemia. USG of part, CT/MRI of the location of the pathology of the septic focus should be done; often may require repetition of these imaging to assess progress. Blood urea, serum creatinine, liver function tests, prothrombin time (PT), activated partial thromboplastin time (APTT), ECG monitoring are also should be done. All these tests including platelet count and arterial blood gas analysis (ABG) should be repeated at regular intervals.

I Treatment of Shock B

- -

-

-

GUIDELINES

To treat the cause, to improve cardiac function and to improve tissue perfusion

B First stabilize the patient with initial resuscitation • Next evaluate the patient for cause and severity Lastly treat the specific cause to achieve cure

Initial acute critical care management using A-Airway; BBleeding; C- Circulation; D- Disability, drugs; E- Exposure as primary assessment and resuscitation should be done.

109

Treat the cause, e.g. arrest haemorrhage, drain pus. Fluid replacement: , Plasma, normal saline, Ringer's lactate, plasma expander (haemaccel) (maximum 1 litre can be given in 24 hours). ,,. Initially crystalloidsthen colloids are given. Blood transfusion is done whenever required. ,,. Fluid therapy is ideally done with crystalloids like normal saline, Ringer's lactate, Hartmann's solution. Blood loss should be corrected by blood transfusion only. Crystalloids and colloids do not have 02 carrying capacity. Hypotonic solutions like dextrose are poor volume expanders and so should not be used in shock. :..- Dynamic fluid response is studied by using 500 ml of warm normal saline (or RL) in 10 minutes using two wide bore IV cannulas. Responders show improvement; transient responders show improvement temporarily but revert back to original status probably due to still existing fluid/blood loss or still existing fluid shift from intravascular space; nonresponders will not respond as fluid loss is severe and persistently ongoing. Fluid therapy is continued as 20 ml per kg or 150 ml/hour with adequate monitoring (urine, CVP, MAP). lnotropic agents: Dopamine, dobutamine, adrenaline infusions-mainly in distributive shock like septic shock.

n

B • • • •

Dopamine improves renal and splanchnic blood Dobutamine improves the cardiac output Adrenaline/Levarterenol IV in anaphylaxis Amrinone and milrinone are newer inotropic drugs

Correction of acid-base balance: Acidosis is corrected by using 8.4% sodium bicarbonate intravenously. Steroid is often life-saving. 500-1000 mg of hydrocortisone can be given. It improves the perfusion, reduces the capillary leakage and systemic inflammatory effects. Antibiotics in patients with sepsis; proper control of blood sugar and ketosis in diabetic patients. Catheterisation to measure urine output (30-50 ml/hour or >0.5 ml/kg/hour should be maintained). Nasal oxygen to improve oxygenation or ventilator support with intensive care unit monitoring has to be done. CVP line to perfuse adequately and to monitor fluid balance. TPN is given when required. PCWP to monitor very critical patient. Haemodialysis may be necessary when kidneys are not functioning. Control pain-using morphine (4 mg IV).

J:

.,, )>

-I

m

....

::0

Fig. 1.202: Trendelenburg position-head down position, used in patient in shock.

" G) l'D

::, l'D

Ventilator and ICU/critical care management. Injection ranitidine IV or omeprazole IV or pantoprazole IV. MAST (military antishock trouser): Provides circumferential external pressure of 40 mmHg. It is wrapped around lower limbs and abdomen, and inflated with required pressure. It redistributes the existing blood and fluid towards centre. It should be deflated carefully and gradually. Note: • Activated C protein which prevents the release and action of inflammatory mediators once used is said to be not beneficial now. • The patient is monitored with ECG, pulse oximetry, blood pressure/ invasive blood pressure, CVP/ PCWP, urine output, pupillary reaction (dilated or not), serum electrolytes, arterial P02 and PC02 analysis.

CENTRAL VENOUS PRESSURE (CVP) It is a method to measure the right atrial pressure by placing a venous catheter (20 cm) into the SVC (superior vena cava). Commonly for CVP monitoring, a venous catheter is passed through internal jugular vein or infraclavicular subclavian vein to the SVC (used for TPN purpose). Occasionally a long catheter (60 cm) can be passed through basilic vein (not commonly done). Under radiological guidance, initially a needle is passed 3 cm above the medial end of the clavicle, in the hollow between the two heads of sternomastoid muscles, directing towards the suprasternal notch into the right internal jugular vein. Then through a guide wire, a venous catheter is passed into the SVC through right internal jugular vein, which can also be confirmed by changes in flow during inspiration and expiration. Catheter is connected to saline manometer, taking manubriosternal angle (angle of Louis) as zero point.

• If less than 2 cm, more fluid is infused • If more than 10 cm, fluid infusion should be restricted Note:

It may be useful t o assess the preload and response to fl uid therapy. Even though it is not ideal, it is commonly used in ICU J as it is technically simpler.

Tears shed for self are tears of weakness, but tears shed for others is a sign of strength.

iil V\ C

.... ....

IQ l'D

':c:

V\

=r n 0

-:,::-

110

Complications of CVP: Pneumothorax; Haemothorax; Injury to brachia! plexus and vessels; Bleeding; Sepsis; Catheter displacement.

PULMONARY CAPILLARY WEDGE PRESSURE (PCWP) It is a better indicator of circulating blood volume and left ventricular function. Catheter used is Swan Ganz triple channel pulmonary artery balloon catheter.

B co a: VI

CVP cannula- ~t ===j~~~~.1~ 7---- ,~ Subclavian vein

V\

IT IS USED TO

Differentiate right and left ventricular failure, pulmonary em bolus, septic shock To measure and monitor cardiac output during the use of inotropic agents, vasodilators and fluid therapy

Fig. 1.203: Central venous pressure (CVP) cannula. Note the location of tip of CVP line in the SVC (or can be in the right atrium).

Superior

Lung

Fig. 1.204: CVP line for monitoring and perfusing the patient in shock.

r

Fig. 1.205: Pulmonary capillary wedge pressure. Note the wedged balloon in the tip of the venous catheter in the pulmonary arteriole.

Diflerences between CVP and PCWP

CVP

PCWP

1. Technically easier

1. Requires skilled experts

2. Normal pressure is 2-10 cm of saline

2. 8-12 mmHg

3. Gives gross idea about fluid balance

3. Better and specific

4. Left ventricular function is not assessed

4. Left ventricular function is very well-assessed

5. Not used to differentiate between right and left ventricular function

5. Very well-differentiated

6. Can be kept in situ as long as desired

6. Cannot be kept in situ tor more than 72 hours

7. Catheter tip is in SVC

7. Catheter tip is in pulmonary capillary with wedging

8. Plain tip catheter

8. 1.5 ml air filled balloon tip

9. Can be used for TPN, fluid infusion, etc.

9. Can not be used for TPN, or fluid infusion

10. Complications are easy to tackle

10. Often difficult to tackle

11 . Not as sensitive and specific as PCWP

11 . Sensitive and specific

(SVC: superior vena cava; TPN: total parenteral nutrition; PCWP: pulmonary capillary wedge pressure)

Under strict aseptic precaution, using cannula and guide wire, catheter is passed through internal jugular vein, into the right atrium. Balloon is inflated by 1.5 ml of air and then negotiated into pulmonary artery, until it reaches a small branch and wedges it. Pressure at this point is called as pulmonary capillary wedge pressure. PCWP normally is 8-12 mmHg, considering mid axillary point as zero reference point. After that, balloon is deflated to get pulmonary artery pressure which is normally 25 mm Hg systolic and 1Omm Hg diastolic. PCWP catheter can be kept in situ only for 72 hours. Complications: Arrhythmias; Pulmonary artery rupture; Balloon rupture; Pulmonary infarction; Pneumothorax; Haemothorax; Bleeding, sepsis, thrombosis.

Respiratory, renal , hepatic, circulatory, coagulative and cardiac failure occurs as an end stage MODS. Primary MODS is due to a well-defined cause like pulmonary contusion, rhabdomyolysis, multiple transfusions. Secondary MODS occurs as a result of host response in SIRS. Management of MODS is critical care in ICU with ventilator support, haemodialysis, transfusions, antibiotics, proper nutrition in the form of TPN or enteral. MODS stage has got high mortality.

OXYGEN THERAPY Indications

111

n :c )>

"'C

-t m

:::0

C\

II)

::,

II) ""I

SYSTEMIC INFLAMMATORY RESPONSE SYNDROME (SIRS) SIRS is systemic manifestations of inflammation due to variety of causes like infection, pancreatitis, polytrauma, burns, transfusion reaction, and malignancy. So it is often categorised as infectious cause SIRS or noninfectious cause SIRS. It causes either hyperthermia (>38°C) or hypothermia (90/minute); tachypnoea (>20/minute); total white cell count >12,000/cu mm, or count 4 mmoVL with metabolic acidosis.

•

. '

:,

.,

Progressive coagulopalhy

i

ft, ' •·

Fig. 1.209: Mops used during major surgery should be kept in a rack so that quantity of bleeding during surgery can be assessed approximately.

I.

115

Classification of haemorrhagic shock (circulatory failure) I

BloodlOl8 Up to 15% (40% (>2000 ml)

Palor, thirsty, tachycardia Hypotension, tachycardia, oliguria, confusion Rapid pulse, low BP, anuria, unconsciousness, MODS

Clan

rn • • • • •

....

:I:

EFFECTS OF HAEMORRHAGE

Acute renal shut down; Liver cell dysfunction Cardiac depression; Hypoxic effect; Metabolic acidosis GIT mucosa! ischaemia; Sepsis Interstitial oedema, AV shunting in lung- ARDS Hypovolaemic shock- MODS

I Treatment Immediate Resuscitation

i.e. ligation of the small vessel, suturing the wound part, vessel suturing (anastomosis); topical applications for local ooze-Oxycel, gauze soaked with adrenaline, bone wax for oozing from bone and other local haemostatic agents (collagen, thrombin). In venous haemorrhage, elevation, ligation of vein or in case of large vein suturing of venous wall, pressure bandaging, packing will be helpful. Internal haemorrhage: lntercostal tube placing for haemothorax; laparotomy for liver or spleen or mesentery or bowel injuries, suturing, splenectomy. Just placing intra-abdominal pack to control bleeding especially in liver injury as damage control surgery is very useful. Upper and lower gastro intestinal bleeding is managed differently after initial resuscitation- endoscopic control or angiographic control, etc. Permissive hypotension prevents rebleeding; one should void removing the clot.

Airway; Breathing; Circulation (ABC). Oxygen should be started (15 litres/minute). Correction of hypotension by fluid therapy by fresh warm whole blood transfusion or packed cell, fresh frozen plasma, platelet as 1: 1: 1 ratio (two wide bore IV cannulas should be placed and blood is drawn for blood grouping and cross matching and essential investigations); warm normal saline or Ringer lactate infusion should be started until blood is ready. Crystalloids cause dilutional coagulopathywithout any oxygen carrying Other Measures capacity needed to correct anaerobic metabolism with Oxygen support/intubation/ventilator and critical care. shock; also cause hyperchloremic acidosis worsening the Catheterisation, foot end elevation [head down (Trendelenexisting acidosis. Unwarmed fluid infusion causes hypoburg) position], monitoring. thermia. Isotonic, hypotonic and colloid solutions cause Absolute rest, analgesics, morphine 10-20 mg IM/ IV to leak of fluid into intetstitial space and edema with only a relieve pain, sedation. part remaining within the intravascular system. Haemaccel, Tourniquet is often used in operation theatre for control of SAG-M blood and dextran can also be used initially until haemorrhage in limbs. But it is not advisable as a first aid blood products are available. Injury severity score (ISS) measure. should be assessed (it is the sum of the squares of 3 Total parenteral nutrition (TPN), central venous pressure (CVP) maximum scores of regions). monitoring, electrolyte management are allequally important. Damage control resuscitation (OCR) approaches by earlier Steroid injection, antibiotics, ventilator support are often and more aggressive correction of coagulopathy and metarequired. bolic derangement with-permissive hypotension; the use of Acidosis, hypothermia, hypocalcaemia and coagulopathy blood products over isotonic fluid for volume replacement; should be treated. and the rapid and early correction of coagulopathy with component therapy. End Points of Resuscitation

Control of Haemorrhage Stop the blood Joss:. Pressure; Packing; Position and Rest; Damage control surgery. External haemorrhage: It is controlled differently than internal haemorrhage. Wound exploration and proceeding,

Keeping patient warm (prevention of hypothermia; temperature below 32°C is lethal). Correction and prevention of further coagulopathy (TIC)fibrinogen infusion 4 grams as initial dose then 50 mg/kg; thromboelastography (TEG) and or rotational elastometry (ROTEM) assessment should be done. Trenaxamic acid 15

No problem is too large for God's intervention and no person is too small for God's attention

V'I

...C: IC ...l'D ':c:

:I: 0.1

l'D

3

......0 -::r 0.1

IC l'D 0.1

::,

c.. to

0 0 c.. :;I 0.1

::, Ill

2'

er

Ill

::,

116

mg/kg initial infusion then 5 mg/kg per hou r. Fibrinogen and prothrombin complex concentrate (PCC) transfusion avoids packed cell and platelet transfusions significantly. Cryoprecipitate and recombinant activated factor VIia are also beneficial. Fluid and electrolyte management, sepsis control (antibiotics). Further monitoring by hourly urine output, hemodynamic stability, respiration, and estimation of lactic acid, base deficit, fibrinogen, platelet, prothrombin INR, APTT, haematocrit, arterial blood gas (ABG), liver and renal functions. Chest X ray may be required to check the lungs. Note:

• Blood transfusion is required if Hb%is ,

QI

e'

::I \/'I

0

IQ

::I C: IQ

cc"'

a: \/'I

Uses: To attain bloodless field in limb surgeries- upper and lower limbs, orthopaedic surgeries, soft tissue tumours, amputations. ;. It is used (rubber tourniquet) to access veins for IV injections and IV sampling. , Tourniquet is used in diagnostic tests for varicose veins, purpura (ITP), carpal tunnel syndrome, tetany. , It is used as a first aid in bleeding conditions of limbs, snake bite (it is controversial). Tourniquets are often used for small procedures in fingers and toes. Types: , Rubber tourniquet: Simple red rubber catheter is used for drawing blood, to have access to veins. Martin's tourniquet made up of India rubber. Pneumatic tourniquet: Used in limbs, will give the arterial pressure and also acts as a tourniquet (Sphygmomanometer cuff is simpler and easily available type). , Esmarch rubber elastic bandage tourniquet. , Conn pneumatic tourniquet is manually operated tourniquet where air is pumped up to the required pressure. ;... Specialised sophisticated tourniquets are available which gauge pressure and time accurately-automatic tourniquet. Tourniquet time for upper limb is one hour and for lower limb is two hours. Contraindications: In all peripheral vascular diseases and atherosclerosis. Infection; Deep venous thrombosis. ;... Crush injuries; Sickle cell disease. Complications: Crushing effect on muscles in thigh occurs leading to crush syndrome. ;... Tourniquet palsy in upper limb (radial nerve involvement)-neuropraxia. Improper application of tourn iquet leads to more bleeding. Forgetting the removal of tourniquet or taking more time to release may compromise the blood supply of the limb leading to severe ischaemia and gangrene. It occurs especially when tourniquet is used in finger or toe. Infection, Skin blistering and necrosis.

DISSEMINATED INTRAVASCULAR COAGULATION Disseminated intravascular coagulation (DIC) is a manifestation due to widespread intravascular coagulation resulting in microthrombi formation, consumption of platelets and clotting factors and production of breakdown products eventually leading into severe bleeding and tissue ischaemia. Causes: , Major trauma causes DIC due to release of tissue thromboplastin. Burns, major surgery can also cause DIC. , Sepsis is the most common cause of DIC. Common sepsis causing DIC are gram-negative, meningococcal, malarial, histoplasmosis, aspergillosis, etc.

;.- Acute pancreatitis can cause DIC by releasing proteolytic enzymes which activate prothrombin and factor X. Septic abortion, abruption, retained dead foetus, amniotic fluid embolism are obstetric causes of DIC. Carcinoma of pancreas, prostate, acute promyelocytic leucaemia often cause DIC. Haemolysis, snake bite, liver dysfunction are other causes. Types of DIC: , Acute DIC presents with bleeding in gums, GIT, venepuncture site, haematuria, petechiae, oozing from su rgi cal or traumatic wounds. Massive bleeding also can occur. ,,. Chronic DIC is a low grade type with thrombotic features. Investigations: ;... In DIC-bleeding time, platelet counts are reduced . Thrombin time (TT), prothrombin time (PT) and activated partial thromboplastin time (APTT) are prolonged. Fibrinogen degradation product (FOP), D-dimer test are raised. ,,. Complete haematocrit, investigations relevant to cause, renal function tests, LFT, electrolyte estimation, blood/ discharge/pus/urine culture. Treatment of DIC: , Treatment of specific cause as per protocol. Correction of haemodynamic instability by fluid therapy, transfusion of packed cells or whole blood. Dopamine/dobutamine therapy. Factor replacement-specific therapyfor DIC- FFP, cryoprecipitate, platelet concentrate transfusions are essential. FFP is given at a dose of 15 ml/kg. Cryoprecipitate is used to raise fibrinogen level at a dose to make plasma fibrinogen level 150 mg/dl. Platelet is transfused at a dose of 0.1 unit/kg, when platelet drops below 20,000/ or with episodes of bleeding. ,,. Heparin use is often controversial. It is used mainly in chronic DIC, DIC with purpura, DIC of obstetric cause, cancer induced DIC, DIC due to acute antiphospholipid antibody syndrome. EACA, tranexamic acid can be used but with questionable benefits.

MECHANISM OF BLOOD COAGULATION (HAEMOSTASIS) Haemostasis is the spontaneous arrest of bleeding. When an injury occurs platelet adhesion occurs to injured vessel/capillary wall which activate the release of ADP (Adenosine diphosphate) which makes more platelet to aggregate (platelet aggregation). These activated platelets release thromboxane A2 which further increases the adhesion and aggregation of platelets. Circulating fibrinogen binds to an activated platelet receptors glycoprotein llb and Illa and fibrinogen gets converted into fibrin. Clotting factors are proteins synthesised by the liver which with a series of cascade reaction activates clotting factors and achieves blood coagulation by a complex mechanism. Factor

11, VI I, IX and X are vitamin K dependent for their synthesis in liver (carboxylation of glutamic acid). In the process of coagulation each factor gets activated to an enzyme by partial proteolysis, which in turn, activates other needed coagulation factors. Eventually fibrinogen gets converted into soluble fibrin and later into insoluble fibrin. Two types coagulation system are there: Intrinsic pathway Extrinsic pathway In vitro coagulation occurs by intrinsic coagulation system. Cascade gets activated by vessel wall injury, shear stress of vessel or other factors. It activates the cascade to get final result.

I Coagulation Cascade System

Factor No.

Extrinsic

Xia 1x - L X

{VII , 111 (tissue thromboplastin ), Ca, phosphoipids}

lr a---t-J--

++ X X Ca ~--VI II, platelet factor

!

IX

Fibrinogen II Prothrombin 111 Thromboplastin IV Ionic calcium V Hereditary labile factor, activator (AC) globulin, proaccelerin VI Accelerin, supposed to be active form of factor V VII Proconvertin; serum prothrombin-conversion accelerator (SPCA)

("\

:::c > "'C --t

m ::c

....

:::c Cl ID :,

... ID 1:11

VI C:

IX Plasma thromboplastin component (PTC; Christmas factor)

IC

X Stuart-Prower factor

:I:

XII Hageman factor XIII Fibrin stabilizing factor, fibrinase XIV Prekallikrein XV Kallikrein XVI Platelet factor

i

Classic haemophilia is called haemophilia A is caused by deficiency of factor VIII Cwith X linked recessive trait. It occurs Fibrinogen (I)- - • Fibrin soluble la in males or homozygous females. Recurrent haemarthroses is a common presentation. Petechiae and echymoses are absent. XIII Ila Xllla - · Bleeding time is normal but coagulation time is prolonged . Fibrin insoluble Treatment is replacement of factor VIII haemophilic factor. If not known patient may go for a life-threatening bleeding even Haemophilia and Von Willebrand's Disease after dental extraction. They are the two most common inherited bleeding disorders Von Willebrand's disease is deficiency of larger component due to deficiency of factor VIII. (99%) of the factor Vlll-vWF. It is an autosomal dominant Factor VI11 has two components; smaller one-factor VI11 disease with normal bleeding time and normal platelet count. C is needed for activation of factor X in intrinsic coagula- Common presentations are spontaneous bleeding from mucous tion pathway; its deficiency leads to classic haemophilia. It membrane, excessive bleeding from wounds and severe is inherited as an X-linked recessive trait, thus it occurs in menorrhagia. Haemarthroses is not common in von Willemales and homozygous females. The larger component of brand's disease. Treatment is replacement of specific factors. factor VI11 called van Wiflebrand's factor, facilitates the adheHaemophilia B also called as Christmas disease is due to sion of platelets to subendothelial collagen, hence crucial for factor IX deficiency is inherited as X linked autosomal receshaemostasis, its absence leads to van Wiflebrand's disease. sive trait. Prothrombin - - • - - - (II) V, Ca++, platelet factor

121

Common name

XI Plasma thromboplastin antecedent (PTA)

x 1i--x 11a XI

Blood clotting factors

VII I Antihemophilic factor (AHF)

Intrinsic Prekallikrein, kininogen

•!

T

• Thrombin Ila

I

Our greatest glory is not in never faffing, but in rising every time we faff.

... ID

':c:

1:11 ID

3

...... ':S'

0

1:11

IC

ID 1:11

:,

Q.

0 0

Q.

:;I 1:11

:,

Ill

2'

o·

Ill

:,

I. Burns Burns which are not involving eyes, ears, face, hand, feet, perineum.

(iH APTER OU TL INE • • • •

Burns Management of Burns Eschar Contracture in Burn Wound

• • •

Electrical Burns Inhalation Injury Chemical Burns

BURNS

Major (severe): Second degree burns more than 25% in adults, in children more than 20%. All third degree burns of 10% or more. Burns involving eyes, ears, feet, hands, perineum. All inhalation and electrical burns. Burns with fractures or major mechanical trauma.

B

I. Depending on thickness of skin involved

• Thermal injury - Scald-spillage of hot liquids - Flame burns - Flash burns due to exposure of natural gas, alcohol, combustible liquids - Contact burns-contact with hot metals/objects/materials • Electrical injury • Chemical burns-acid/alkali • Cold injury-frost bite • Ionising radiation • Sun burns

I Classification of Burns Depending on the Percentage of Burns (Burn Severity Classification) Mild (Minor): •

Partial thickness burns ,

QI

V'I

0

"'C: ::,

"' :E

"' a::a er: V'I

Fig. 1.220: Third degree burns with charred skin, with thrombosed veins and eschar.

There is increased capillary permeability, decreased plasma oncotic pressure causing loss of protein and fluid from intravascular space. Vasoconstriction occurs due to raised capillary hydrostatic pressure leading into cellular aggregation. Blockage of lymphatics causes poor clearance of fluid and proteins from interstitial spaces. Cell membrane function is impaired causing intracellular fluid accumulation. Activation and release of various complement factors, histamine, and prostaglandins results in myocardial dysfunction, oedema of tissues, reduced immunoglobulin synthesis. Catecholamine levels are raised drastically in patient with burn. There will be lipolysis, proteolysis, increased release of glutamine and alanine from skeletal muscles. Urea production is increased due to more proteolysis.

Zone of stasis

--.J

Zone of - ~ = t= coagulation Zone of - +---.,.__ hyperaemia Coagulation

Fig. 1.221: Face burns in an adult-severe third degree.

I Clinical Features History of burn. Pain, burning, anxious status, tachycardia, tachypnoea, fluid loss. In severe degrees features of shock. Tolerable temperature to human skin is 40°C for brief period.

I Pathophysiology Heat causes coagulation necrosis of skin and subcutaneous tissue J, Release of vasoactive peptides j,

Altered capillary permeability J, Loss of fluid Severe hypovolaemia j,

I

Decreased output Dec~eased renal blood flow (Renal failure)

Decreased myocardial fun ction Oliguria

Fig. 1.222: Burn zones (Jackson's).

Massive oedema in the body is due to altered pressure gradient because of the injury to basement membrane. Cardiac dysfunction is due to: Hypovolaemia. Release of cardiac depressants. Hormonal causes like catecholamines, vasopressin, angiotensins. Renal changes are due to: Release of ADH from posterior pituitary to cause maximum water reabsorption . Release of aldosterone from adrenals to cause maximum sodium reabsorption. Toxins released from the wound along with sepsis causes acute tubular necrosis. Myoglobin released from muscles (in case of electric injury or often from eschar) is most injurious to kidneys. Pulmonary changes are due to: Altered ventilation-perfusion ratio. Pulmonary oedema due to burn injury, fluid overload, inhalation injury. ARDS; Aspiration; Septicaemia.

INFECTIONS ARE COMMONLY DUE TO +

+ +

•

• •

Occurs in burns around face and neck, or trapped in burning room Presents with hoarseness or stridor Inhaled burning gases can cause upper airway burns and laryngeal oedema Smoke inhalation can cause chemical alveolitis, pulmonary oedema, ARDS and respiratory failure Steam inhalation can cause damage to respiratory epitheliumand subglottic oedema Carbon monoxide inhalation more than 10% is dangerous as it forms carboxyhaemoglobin (CO has got 240 times more affinity to haemoglobin than oxygen) which blocks oxygen transport completely causing respiratory arrest, hypoxia and metabolic acidosis Chest wall burn causes mechanical block of ventilation-needs escharotomy in chest wall and the procedure is painless Airway burn may require early elective intubation or tracheostomy or emergency cricothyroidotomy as a life-saving method

Streptococci (Beta haemolytic-most common) Pseudomonas + Staphylococci • Other gram-negative organisms Candida albicans

125

n

:::c l> m

....

::ti

"...

11)

::, 11) QI

...r::

V'I

IC

...

11)

':c:

c:,

...::,

r::

GIT changes are due to:

Acute gastric dilatation which occurs in 2-4 days. Paralytic ileus, Curling's ulcer {due to decreased mucosa! defence; not due to increased HCI). Cholestasis and hepatic damage; Acute acalculous cholecystitis, acute pancreatitis can occur. Bowel mucosa/ ischaemia causes poor motility, reduced food digestion and absorption with increased translocation of bacteria causing peritoneal oedema, septicaemia and abdominal compartment syndrome.

I Metabolic Changes Hypermetabolic rate (BMR). Negative nitrogen balance; Electrolyte imbalance. Deficiencies of vitamins and essential elements. Metabolic acidosis due to hypoxia and lactic acid.

I Sepsis in Burn Patient Focus may be at the burn site, catheter site, cannula/CVP line site, or respiratory infection. Low immunity, loss of proteins and immunoglobulins, loss of barrier causes sepsis. Opportunistic infection is also common. Associated conditions like diabetes, HIV infection, old age, respiratory diseases worsen the sepsis in burn injury. It may be local infection commonly by Staphylococcus aureus in early period, Pseudomonas, Candida, Aspergillus, herpes simplex virus in partial thickness nasolabial burns. It may be suppurative thrombophlebitis also. Systemic infection like pneumonia, bacteraemia, septicaemia can occur. Burns itself creates immunosuppression (cell-mediated immunity). Sepsis is identified by fever, lethargy, leucocytosis, thrombocytopenia.

VI

Fig. 1.223: Burns over chest wall with eschar. Note the extent of involvement.

I Effects of Burn Injury Shock due to hypovolaemia-15% of burns causes shock. Renal failure. Pulmonary oedema, respiratory infection, adult respiratory distress syndrome (ARDS), respiratory failure. Infection by Staphylococcus aureus, beta haemolytic Streptococcus, Pseudomonas, Klebsiella leads to bacteraemia, septicaemia. Fungal and viral infections of dangerous types can also occur. GIT: Hypovolaemia, ischaemia of mucosa, erosive gastritisCurling's ulcer (seen in burns >35%). Fluid and electrolyte imbalance. Postburn immunosuppression predisposes to severe opportunistic infection. Eschar formation and its problems like defective circulation, ischaemia when it is circumferential. Electrical injuries often cause fractures, major internal organ injury, convulsions. Development of contracture is a late problem. It leads to ectropion, microstomia, disability of different joints, defective hand functions, growth retardation causing shortening. Inhalation burn causes pulmonary oedema, respiratory arrest, ARDS. Chemical injury causes severe GIT disturbances like erosions, perforation, stricture oesophagus (alkali), pyloric stenosis (acid), mediastinal injury. Other problems commonly seen are DVT, pulmonary embolism, urinary infection, bed-sores, severe malnutrition with catabolic status, respiratory infection.

Success lies not in achieving what you aim at, but in aiming at what you ought to achieve.

126

Complications of burns contracture itself like hypertrophic scar, keloid formation. Toxic shock syndrome: It is a life-threatening exotoxin mediated disease caused by Staphylococcus aureus. It is common in children, presents with rashes, myalgia, diarrhoea, vomiting, and multiorgan failure with high mortality.

... ... °' :, >,. QI

-

m

V'I

0 ta :,

CAUSES OF DEATH IN BURNS

Hypovolaemia (refractory and uncontrolled) and shock Renal failure Pulmonary oedema and ARDS Septicaemia Multiorgan failure • Acute airway block in head and neck burns

C:

ta

"' C0

a: V'I

Fig. 1.224: Deep burns face with eyelid involvement.

MANAGEMENT OF BURNS I First Aid Stop the burning process and shift the patient away from the burning area. Cool the area with tap water by continuous irrigation for 20 minutes (not cold water as it can cause hypothermia).

m

INDICATIONS FOR ADMISSION IN BURNS

Any moderate and severe burns • Airway burns of any type • Burns in extremes of age All electrical/deep chemical burns

m

INITIAL MANAGEMENT

Figs. 1.225A and B: Burns hand both palmar and dorsal aspect.

-

Clothing should be removed Cooling of the part by running water for 20 minutes Cleaning the part to remove dust, mud, etc Chemoprophylaxis-tetanus toxoid; antitetanus globulin (ATG500 units, IM); antibiotics; local antiseptics Covering with dressings by different methods Comforting with sedation and pain killer

I Definitive Treatment

Fig. 1.226: Deep burn in the scalp involving extensively.

Admit the patient. Maintain airway, breathing, circulation (ABC). Emergency endotracheal intubation may be required in early period itself; in such situation succinylcholine should not be used. Assess the percentage, degree, and type of burn. Keep the patient in a clean environment. Sedation and proper analgesia. Patient should be placed in burns unit (ideally air-conditioned) with barrier nu rsing, sterile clothes, bed sheets with all aseptic methods.

Sodium is assessed by formula: 0.52 mmol x kg body weight x % body burns, given at a rate of 4.0 to 4.4 ml/kg/hour.

Fig. 1.227: Extensive burn patient of fluid therapy

with CVP line.

I Fluid Resuscitation Formulas to calculate the fluid replacement: Parkland regime: Commonly used: 4 mU% burn/kg body weight/24 hours. Maximum percentage considered is 50%. Half the volume is given in first 8 hours, rest given in 16 hours. Muir and Burclay regime: For colloid after 12-24 hours. % Burns x Body weight in kg R t· = 1 a 10n

2

3 Rations given in first 12 hours. 2 Rations in second 12 hours. 1 Ration in third 12 hours.

B • Galveston regime (pediatric): 5000 mUm 2 burned+ 1500 mUm 2 total • Modified Brooke formula: First 24 hours: RL: 4 ml/kg/% burns in 24 hours (first half in first

8 hours) Colloid- none. Second 24 hours: Crystalloids-to maintain urine output; Colloids-0.3 ml to 0.5 ml/kg/burns in 24 hours; (Albumin in Rl solution) (Albumin alone should be given with care if really indicated only}. Evan's formula: In first 24 hours: Normal saline 1 mUkg/% burns; Colloids 1 mu

kg/% burns; 5% dextrose in water, 2000 ml in adult. In second 24 hours: Half of the volume used in first 24 hours. Fluids used are Ringer lactate, Hartmann fluid, plasma. Ringer

lactate is the fluid of choice. Blood is transfused in later period (after 48 hours). First 24 hours only crystal/aids should be given (Crystalloids are one which can pass through capillary wall like saline either hypo, iso or hypertonic, dextrose saline, Ringer lactate).

After 24 hours up to 30-48 hours, colloids should be given to compensate plasma loss (colloids are one which are retained in intravascular compartment). Plasma, haemaccel (gelatin), dextrans, hetastarch are used. Usually at a rate of 0.35- 0.5 mLJkg/% burns is used in 24 hours. Human albumin is ideal colloid. Urinary catheterization to monitor output; 30-50 mUhour should be the urine output. Tetanus toxoid. Monitoring the patient: Hourly pulse, BP, PO2, PCO2, electrolyte analysis, blood urea, nasal oxygen, often intubation is required . IV ranitidine 50 mg 8th hourly. Ryle's tube insertion initially for aspiration purpose later for feeding (Enteral feeding). For burns>15%. Antibiotics: Penicillins, aminoglycosides, cephalosporins, metronidazole. Culture of the discharge; total white cell count and platelet count at regular intervals are essential to identify the sepsis along with fever, tachycardia and tachypnoea. In burns of oral cavity tracheostomy may be required to maintain the airway. Total parenteral nutrition (TPN) is required for faster recovery, using carbohydrates, lipids, vitamins (through a CVP line). Tracheostomy/intubation tube may be required in impending respiratory failure or upper airway block. Intensive nursing care.

I Local Management Dressing at regular intervals under general anaesthesia using paraffin gauze, hydrocolloids, plastic films, vaseline impregnated gauze or fenestrated silicone sheet or biological dressings like amniotic membrane or synthetic biobrane. Open method with application of silver sulfadiazine without any dressings, used commonly in burns of face, head and neck. Closed method is with dressings done to soothen and to protect the wound, to reduce the pain, as an absorbent. Tangential excision of burn wound with skin grafting can be done within 48 hours in patients with less than 25% burns. It is usually done in deep dermal burn wherein dead dermis is removed layer by layer until fresh bleeding occurs. Later skin grafting is done. Advantages of tangential excision: It reduces-the chance of secondary infection, the hospital stay, and formation of hypertrophic scar or contracture, the cost. In burns of head and neck region, exposure treatment is advised. Slough excision is done regularly. After cleaning with povidone iodine solution silver sulfadiazine ointment is used. It is an antiseptic and soothening agent. It causes neutropenia.

Life's disappointments are opportunity's hidden appointment.

127 n

::I:

l>

"'O

--4

m

....

::c

128

Top/cal agent

Advantage,

Problems

Silver sulfadiazine (1 %)

• Antiseptic (G +ve and G-ve)

• Neutropenia, pseudoeschar • It diffuses poorly through the eschar

• Soothening, good penetration, • Hydration and softening of eschar occurs

• Causes hypertrophic granulation and so not used once wound is clean and eschar is separated

• It does not cause pain

• Causes wound maceration • It should be avoided in pregnancy, nursing mother and infants below 2 years of age

Sulfamylon (5%/11 %)

• Antipseudomonal, anticlostridial

• Very irritant, painful

(Mafenide acetate)

• Penetrates very well into tissues • It is water soluble • It is good antipseudomonal agent

• Causes acidosis, by inhibiting the carbonic anhydrase

Silver nitrate (0.5%)

• Antiseptic, antipseudomonal

• Stains burnt area

Povidone iodine (5%)

• Antiseptic (G +ve, G-ve)

• Irritant and painful

• Used on granulation tissue after eschar separation

• Not used in partial burns

• Used in perineum and buttocks to prevent maceration Silver sulphadiazine and cerium nitrate

• Boosts cell-mediated immunity and forms sterile eschar

Other agents used are Sulfamylon (Mafenide acetate) and Silver nitrate. , Sulfamylon is antipseudomonal and anticlostridial agent. It penetrates well into the tissues but it is very irritant. It causes acidosis. , Silver nitrate causes staining of burnt area. , 0.025% sodium hypochlorite (Dakin's solution) is effective against Gram +ve organisms; 0.25% acetic acid is effective against Gram -ve organisms, but both mildly inhibit epithelialisation. Regular culture and sensitivity for bacteria is required, to see for streptococcal growth which should be less than 1,00,000 (1a5) per gram of tissues.

I Wound Coverage Once the area granulates well, in 3 weeks usually, split skin grafting is done (SSG, Thiersch graft). For wider area MESH split skin graft is used. If there is eschar, escharotomy is required to prevent compression of vessels. In certain areas like face and ear, full thickness graft (Wolfe graft) or flap is required . Cultured skin: Full thickness skin biopsy of patient's skin is done immediately after admission. By specialized culture technology sheets of skin can be manufactured in 3 weeks as cultured epithelial grafts. It can cover skin of almost entire body. It is usually useful in burns of >80%. Take up of cultured graft is 60-75%. Limitations are-time taken to develop cultured graft; more vulnerability for mechanical trauma; costly; time taken to manufacture; scarring. Synthetic dressings in burn wound Vaseline impregnated gauze dressing prevents stiffness of eschar.

Hydrocolloid dressing (duoderm) helps moist environment, proper epithelialisation. It is useful in mixed deep burns. It is changed once in 3 days. Opsite is less expensive, with less pain, creates moist barrier. But it does not have antimicrobial effect and it causes accumulation of exudates. Biobrane is collagen coated silicone sheet which gets adherent to wound acting as barrier without any pain. But it does not have antimicrobial effect and it causes accumulation of exudates. It is used for 2nd degree burns. Transcyte has similar features of biobrane. It contains growth factor derived from cultured fibroblasts which promotes wound healing. lntegra contains deeper collagen matrix as dermal substitute; outer silicone sheet as epidermal substitute. Inner collagen matrix acts as dermis whereas outer silicone sheet is removed 2 weeks after dressing and additional autograft should be placed. It provides complete wound cover. Scarring after healing is reduced significantly. Biologic dressings for burn wound

It is used to cover the wound temporarily as a barrier and also to have some immunologic function. Eventually graft will slough. Later wound is covered with auto-skin graft. It is used for massive burn injuries more than 50%. Possible problem is transmission of viral diseases. Xenograft is of pig skin. Allograft is of cadaver skin (homograft)-it gives all existing normal skin function for temporary period . It may leave a dermal equivalent in the wound later.

ESCHAR It is charred, denatured, full thickness, deep burns with contracted dermis.

It is insensitive, with thrombosed superficial veins. Circumferential eschar in the upper limb, lower limb, neck, thorax can cause more oedema which initially causes venous compression and later arterial compression causing ischaemia, gangrene of the distal part. So distal area should be monitored for circulation. If required deep longitudinal full thickness incisions are made in different areas so as to prevent collection of oedema fluid and also to prevent compression over the vessels. This is called as escharotomy. Escharotomy causes large quantity of blood loss and so blood transfusion is needed while doing escharotomy. Incision should be of adequate length and depth during escharotomy. It should be placed in such a way so as to avoid injury to major neurovascular system. Release of muscle compartment is needed often in these patients.

CLASSIFICATION OF BURNS CONTRACTURE IN THE NECK (BM ACHAUER ) Mild (less than 1/3rd)-inability to see ceiling. Moderate (113rd to 2/3rd)-flexion is possible but not extension. Severe (more than 2/3rd)-fully contracted in flexed position with pull on lower lip. Extensive--contraction is extensivewith mentosternal adhesions. /feanyichukwu has classified neck contracture into: Type 1-mild anterior with narrow contracting band less than fingerbreadth (1a) or broad band (1 b); type 2-moderate anterior with narrow band (2a) or broad band (2b); type 3-severe anterior mentosternal adhesion with supple neck skin (3a) or without supple skin (3b); type 4-posterior with narrow band (4a) or multiple or broad band (4b). Reconstruction territories in neck in burn contracture based on functional benefits are--central above; central below; central above and below; lateral.

I Complications of Burns Contracture Ectropion of eyelid causing keratitis and corneal ulcer. Disfigurement in face. Narrowing of mouth microstomia. Contracture in the neck causing restricted neck movements. Disability and nonfunctioning of joints due to contracture. Hypertrophic scar and keloid formation. Repeated breaking of scar and infection, ulcer, cellulitis. Pain and tenderness in the scar contracture. Marjolin's ulcer: Refer Chapter 1B.

I Treatment for Contracture Fig. 1.228: Extensive eschar involving both thighs.

Multiple incisions or incisions over the joints may be needed. Early rapid separation of eschar indicates severe sepsis underneath. Eventually eschar should be excised and the area is allowed to granulate and skin grafting should be done. Pseudoeschar is thickened burnt skin due to repeated silver sulphadiazine application.

CONTRACTURE IN BURN WOUND

Release of contracture surgically and use of skin graft or "Z" plasty or different flaps. Different flaps used are-transposition flaps, vertical or transverse; laterally based flap; bilobed flap; bipedicled flap; advancement flap; regional flap; random cutaneous flap (Epaulette flap, Charretera flap) ; fasciocu taneous/myocutaneous flap; tube flap; expanded skin flap; combined skin graft and flap; microvascular free flap. Proper physiotherapy and rehabilitation is essential. Pressure garments to prevent hypertrophic scars. Management of itching in the scar using aloe vera, antihistamines and moisturizing creams.

I Problems in Managing Burn Contracture Giving proper anaesthesia is challenging. Scar excision can cause significant bleeding.

Contracture in burns can occur anywhere. It is more common wherein flexibility and mobility is present like along the joint, eyelids, cheeks, lips, neck, elbow, knee, etc. Contracture can be intrinsic by loss of tissue or extrinsic by pull during healing phase contraction. Contracture proceeds towards position of comfort until it meets or closely reaches opposite surface. There is clearly wound shortening. Disorganised over formation of compact collagen (3 times normal) causes hypertrophic scar leading further contracture. Deficit of neck extension is graded, normal> 110°; E, 95-110°; E2 is 85- 95°; E3 is "'C

6

I Injury Severity Score (ISS) It is an anatomical scoring system that provides an overall score for patients with multiple injuries. Each injury is assigned with AIS with one of the six body regions-Head , Face, Chest, Abdomen, Extremities including pelvis, External. The highest AIS score in each body region is used. The 3 most severely injured body regions have their score squared and added together to produce the ISS score. A major trauma is defined as the Injury Severity Score being greater than 15; it is associated with mortality of 10% or more. ISS ranges from 1 to 75.

m

Fig. 1.238: Degloving injury involving entire left lower limb, perineum,

..... ,.__

and left groin. Patient has lost scrotum and both testes. There were no internal injuries and vessels and nerves were intact. Patient underwent wound excision extensively andcolostomy was done to promote healing of perinea! wound and prevent contamination.

"

::ti

It>

g C:

3 QI

Fig. 1.239: Class Ill dog bite on face in a boy.

Note: • Values indicative of critical physiologic derangement include:

Temperature 4 mmol/L; ionised calcium 1.5 x normal; fibrinogen level 15; 15% of injured patients will have major trauma.

I Revised Trauma Score (RTS)

Glasgow coma scale

Blood pressure systolic

Respiratory rate

Value

13-15 9-12 6- 8 4- 5 3

>89 76-89 50-75 1--49 0

10- 19 >29 6-9 1-5 0

4 3 2 0

iil VI

CRAMS score (Circulation; Respiration; Abdomen ; Motor; Speech) is used often; score

Fig. 1.240: Degloving of scalp with bone periosteum exposing the skull bone. Outer table was actually dead and required bone removal and graft after healthy granulation tissue was formed.

CONCEPTS IN TRAUMA MANAGEMENT Concept of 'golden hour' to treat the trauma patient is important. Multidisciplinary approach. Planning, setting up, organizing, team work. Assess respiratory system; circulation; bleeding areas-as priority. Assess also whether patient is haemodynamically stable or unstable.

Surgeons are Chromophobic-They don 't like Red (blood}; Green (bile}; Blue (organ ischaemia}; Yellow (Pus, fecal matter}.

136

Arrange fluids, blood, catheters, ventilator, etc. Further definitive therapy depending on severity and site of injury.

B

Ill

co

a:

V'I

• Primary management using ABCDEF; assessment of patient as stable or unstable with defined parameters • One should follow ATLS or other guidelines • "Timeline principle/concept" in which clinician should act within the 'critical time window' in the compensatory stage only patient should be addressed properly to prevent the patient from going to decompensatory stage. We have to look everywhere and focused exclusion of specified anatomical regions; check for hidden injuries • Patient should be taken up for Damage Control Surgery (DCS) with following criteria-hypothermia . ...:::, QI

C'I

Vl

0 ,u

:::, C ,u

cc cc VI

Vl

Figs. 1.258A to F: Different types of hand infections. Note the oedema of hand even on dorsal aspect. Small infective focus can aggravate rapidly and so early proper drainage from deeper plane is important in managing the hand infections. Often it may cause extensive destruction exposing the tendons.

B

----

-

REMEMBER

Hand should be flexi ble and strong; sensitive and pain free and coordinated to show all fine and powerful functions Pinch (picking a small object); power grip (holding a hammer); key grip (holding a key); chuck grip (holding a pen); hook grip (carrying a bag)-are the functions of hand Hand should be properly examined clinically for tendon functions; neurological problems-sensations (sweat test, two point discrimination test); for circulation (Allen's test); joint movements; examination of entire upper limb; opposite hand; axillary lymph nodes and other relevant systemic examinations

Contd...

Contd... Nerve conduction studies; electrophysiology; MR I hand; radioisotope bone scan; selective angiograms; X-ray hand are the relevant investigations other than systemic investigations Principles of treatment-elevation to reduce oedema; splintage to prevent contracture; early movements once inflammation subsides; early exploration of wound or surg ical drainage of infective area; reg ional anaesthesia; usage of tourn iquet; incisions when done across the flexor creases, should be at 45° angle

149

Position of rest

Position of function

Fig. 1.259: Hand positions.

Fig. 1.263: Hypertrophic scar and keloid in hand and

forearm after burn contracture.

ACUTE PARONYCHIA It is the most common hand infection. It occurs in subcuticular area under the eponychium. Minor injury to finger is the common cause. Suppuration occurs very rapidly. It tracks around the skin margin and spreads under the nail causing hang nail or floating nail. Organisms are Staphylococcus aureus and Streptococcus pyogenes. Quantity of pus is very less around 0.5 ml but it should be drained to relieve sympotoms.

I Features and Management Figs. 1.260A and B: Hand positions in immobilisation and function.

Severe throbbing pain and tenderness {dependent throbbing) with visible pus under the nail root. Nail on touch is very tender {paronychia means "Run around "). Pus is sent for culture and sensitivity. Antibiotics like cloxacillin, amoxicillin; Analgesics. The pus is drained by making an incision over the eponychium. Digital block usi ng xylocaine 2% plain { without adrenaline as end artery supply to digits can develop arterospasm) is given as anaesthesia.

Fig. 1.261: Hand infection. Infection of ring finger

extending into the palm.

Fig. 1.262: Infection of little finger with dorsal oedema.

Figs. 1.264A and B: Pointing pus in acute paronychia. Quantity of

pus is very less usually around 0.5 ml.

Don't be afraid to learn; Knowledge is weightless, a treasure you can carry easily.

Cl

lb ::I lb QI "'

Vl C:

"' IO lb

"' ': n>

:::,

Ill

VI C

.... n> ....

10

Figs. 1.267A to C: (A) Anatomy of the terminal pulp space; (B) Pulp space infection; (C) Incision for pulp space drainage.

Drainage of terminal pulp space by an oblique deep incision. If there is osteomyelitis of the terminal phalanx, it has to be amputated. Complications: , Osteomyelitis of the terminal phalanx. :.- Pyogenic arthritis of distal interphalangeal joint and tenosynovitis of flexor sheath. ,. Septicaemia-in immunosuppressed individuals.

':':

:I:

, ,

Ill

:::,

a. Ill

:::,

a. Fig. 1.268: Incision for web space drainage.

I Surgical Anatomy

Two deep palmar spaces are present

There are three triangular web spaces filled with fat between the dorsal and volar skin. When the space is filled with pus it straddles the deep transverse ligament. Even though pus is volar, it points out dorsally. Infection of originates from: + Abrasion; Callosities; Trauma. Infection of proximal volar space of finger. Infection of proximal spaces. Spread from other palmar spaces and from llexor sheaths through lumbrical canal.

I

7

1. Midpalmar space. 2. Thenar space. Midpalmar space is bound in front by palmar aponeurosis,

behind by medial three metacarpals, laterally by a vertical line from lateral margin of the middle linger. It contains flexor tendons, neurovascular bundles and lumbricals. It is the common site of the infection. Thenar space is located anterior to lateral two metacarpals. Infection here is usually due to extension from midpalmar space.

J I Midpalmar Space Infection

- - --Bacteria: Staphylococcus, Streptococcus, Gram-negative organisms. Features and management: , Fever; Pain and tenderness. , Oedema of dorsum of hand. , Maximum tenderness is on the volar aspect. :.- 'V' sign- separation of fingers. ,. If untreated, infection may spread into other web spaces and hand spaces. ;.. Elevation of hand. , Antibiotics and analgesics. ,. Drainage under regional or general anaesthesia. A horizontal incision is placed on volar skin of the web and deepened to reach the space by dividing fibres of palmar fascia.

0

0 ....

DEEP PALMAR SPACE INFECTION

INFECTION OF WEB SPACES

I Surgical Anatomy

.,,

Causes ,. Trauma. ,. Spread from infection of finger spaces and web spaces. ,. Haematogenous spread. ,. Spread from tenosynovitis. Features ,. Pain and tenderness in the palm. ,. Oedema of dorsum of hand (frog hand) . , Loss of concavity of palm. ,. Painfu l movement of metacarpophalangeal joint (but interphalangeal joint movements are normal and painfree). ,. Fever. ,. Palpable tender axillary lymph nodes. ,. Eventually pus may come out of palmar aponeurosis forming collar stud abscess and later sinus formation. ,. X-ray of the part is required. Treatment: ,. Elevation of the affected limb.

Be ordinary in your life in all aspects; you will become extraordinary in future.

...>...Cl Q)

:I

V'I

0

ro

:I C:

ro

:E Ill

co

a:

Ill

, - - --Palmar aponeurosis , - - - - Superficial palmar arch ~ -- Digital nerves

Flexor pollicis - - ~ longus tendon Thenar muscles

Hypothenar muscles

Flexor tendons of index finger

Flexor digitorum superficialis tendons

Thenarspace l-----\:\:~~~~, Dorsal subcutaneous space Dorsal - - -subaponeurotic space

Flexor digitorum profundus tendons

~ Midpalmar space

• Skin incision parallel to crease line • Never cross the crease line • Palmar aponeurosis is opened vertically • Space can be approached through lumbrical canal through horizontal web space incision

Fig. 1.270: Incisions to drain midpalmar space infection.

m

COMPLICATIONS

Osteomyelitis of metacarpals Stiffness of hand Suppurative arthritis Extension of infection into other spaces

I Thenar Space Infection

First lumbrical canal

Thenar space

- -+-

Lumbrical canals2nd, 3rd, 4th Midpalmar space

-

Flexor retinaculum

~ -

11----.-1--=- Incisions to drain pus from space of Parona

- -+-- - Space of Parona

Figs. 1.269A to C: Anatomy of pal mar spaces of the hand and forearm. Midpalmar space is on the medial aspect; thenar space is on the lateral aspect. Space of Parona is on the lower forearm.

,, Antibiotics and analgesics. ,, Drainage: It is drained under regional/general anaesthesia by placing horizontal/oblique incision parallel to the palmar crease. One should avoid crossing the crease line as much as possible. Pal mar aponeurosis is carefully incised vertically to avoid injury of the neurovascular bundles. Alternatively one of the interdigital web spaces is incised horizontally; lumbrical canal(3rd or 4th) is opened to reach the deep palmar space. Pus is drained and sent for culture and sensitivity. Thorough saline irrigation is very essential. Drain is placed through the wound.

Thenar space (triangular shape) is located anterior to the lateral two metacarpals and fascia over transverse head of adductor pollicis; behind the short muscles of thumb, flexor tendons of index finger and 1st and 2nd lumbricals. Thenar muscles and flexor pollicis long us are lateral to it; fibrous vertical septum from palmar aponeurosis to 3rd metacarpal bone is medial to it. It is on the outer half of the hollow of the palm. Proximally it extends from flexor retinaculum; distally it extends to transverse palmar crease. It communicates to fascial sheath of 1st lumbrical. It is often associated with midpal mar space infection. Incision through first web space-radial side of index finger- through first lumbrical

Fig. 1.271 : Incision and drainage of thenar space abscess.

It is drained similarly by placing incision on the lateral aspect

of the palm or through the first web space incision is done along the first lumbrica/ canal on the radial side of the index finger. Olten incision is made parallel to cleft between index and thumb on the posterior aspect. In some patients, thenar space infection may spread distally to the first web and then dorsally over the first dorsal interosseous muscle, referred to as a pantaloon abscess. In such situation an additional counter incision over the dorsal aspect of the hand is needed while draining.

I Features

153

Symmetrical swelling of entire finger. Flexion of finger-Hook sign. Severe pain on extension. Tenderness over the sheath. Oedema of whole hand, both palm and dorsum (due to lymphatic spread). As ulnar bursa extends into the little finger its infection results in pain and tenderness extending up to little finger but not much to other fingers.

m

SPACE OF PARONA INFECTION

ACUTE SUPPURATIVE TENOSYNOVITIS It is the bacterial infection of flexor tendon sheaths.

I Surgical Anatomy Radial bursa is synovial sheath of flexor tendon of thumb which extends to the digit. Ulnar bursa is synovial sheaths of medial four flexor tendons of hand which extends into the digit of the fifth (little) finger.

In infection of radial bursa thumb is swollen with pain and tenderness over the sheath of the flexor pollicis long us and there is inextensibility of interphalangeal joint. Swelling just above the flexor retinaculum is common. Incisions for digital synovial sheath drainage

1:---1

___,_ Ulnar bursa

~

- - Synovial sheath exposed in the digit Irrigation polythene catheter ,.._...---Exposed tendon Synovial sheath (infected) ,....,;::--_ Incision in the palm

Fig. 1.272: Bursae hand.

• Extensor tendons are devoid of sheaths. • Radial and ulnar bursa communicate with each other in 80% of cases.

rn Figs. 1.273A and B: Suppurative tenosynovitis is drained through

Common bacteria: Staphylococcus aureus, Streptococcus incision at proximal part and another at digital sheath. Often by placing

pyogenes.

m

.... Cl

... II)

Incision for ulnar bursa

_

--t

::0

II)

Swollen finger held in flexion Exquisite pain on passive extension Tenderness precisely over the tendon sheath Area of greatest tenderness over the part of ulnar bursa lying between transverse pa/mar creases

Incision for~ radial bursa

)> "tJ

::,

KANAVEL SIGNS

Forearm space of Parona is a rectangular space situated in the lower part of the forearm above the wrist, in front of pronator quadrates and deep to long flexor tendons. Above it extends up to oblique origin of flexor digitorum superficialis, below up to flexor retinaculum communicating with mid palmar space. Flexor tendon sheath proximally extends into this space. Pus in this space is drained through lateral incisions in the lower part of the forearm.

""

::c

fine polythene catheter into thesheath, saline wash is given into the area.

When a hand is seriously inflamed it rakes up the position ofgreatest ease, which is, in fact, the position of rest.-frederic Wood Jone

0ol

1/'1 C:

IQ

... II)

': Antibiotics and analgesics. Position of rest. > Drainage under general anaesthesia. Incisions are placed over the site of maximum tenderness and flexor sheath should be opened up. Many a times multiple incisions are required. It is drained through two incisions-one over the proximal part of the sheath ; other over the distal part of the sheath in the digit-along the crease lines. A fine catheter is passed into the sheath from proximal incision and irrigated with normal saline through this catheter. This catheter is left in situ for further regular irrigations, splinting of hand is necessary with boxing glove dressing.

virus infection. Contracted via direct contact with infected sheep or goats or fomites with orf virus. Human-to-human transmission is not known. It causes apurulent-appearingpapule locally and generally without systemic symptoms. Infected locations can include the finger, hand, arm, face and even the penis. It may become progressive and life-threatening in immunocompromised host. Treatment-1 % cidofovir in progressive disease. MILKER'SNOOES/NOOULES {Milkmaid Blisters): It is cutaneous condition caused by Paravaccinia virus; transmitted from udders of infected cows. Disease in humans is nearly identical to orf. Usually has got self-limiting course, running from 14-72 days, with infrequent systemic symptoms and little or no scarring.

HAND INJURIES

m

CLASSIFICATION

•

COMPLICATIONS

• Spread of infection proximally into forearm-to space of Parona Stiffness of fingers and hand • Suppurative arthritis Osteomyelitis • Median nerve palsy Bacteraemia and septicaemia

COMPOUND PALMAR GANGLION It is chronic tenosynovitis of flexor tendon sheaths due to tuberculosis (tuberculous tenosynovitis) or rheumatoid arthritis. It can be unilateral or bilateral. Flexor tendon sheath on either side of the wrist is involved , i.e. both in the volar surface of palm and lower forearm. Swelling contains fluid with typical melon seed bodies. Condition is often bilateral in case of rheum atoid arthritis. Swelling in the palm and lower forearm which is smooth, soft, nontender, fluctuant and also cross-fluctuant across flexor retinaculum, nontransilluminating. Wasting of hand and forearm muscles are seen. Matted axillary lymph nodes may be palpable. Primary focus may be present in lungs. Investigations. ESR, chest X-ray, MRI hand. FNAC of axillary lymph node and swelling itself. Treatment: , Start antituberculous drugs: INH, rifampicin, ethambutol and pyrazinamide for 9 months. ,. Excision of flexor tendon sheath is done along with scraping of caseating material, tubercles, melon seed bodies. Care should be taken not to injure median and ulnar nerves.

Tidy injuries: They are clean incised wounds and are usually treated by primary suturing but depends on the tissues involved like nerves, tendons and muscles. Untidy injuries:They are lacerated wounds. Treated by debridement and later by delayed primary or secondary suturing. Compartment injuries. Oegloving injuries lndetermined injuries which could not be assessed.

I Assessment of Injury It should include: Number, extent, depth, deformity and disability, neurovascular injuries, tendon injuries, muscle injuries bone and joint injuries.

I Principles of Treatment Haemostasis; Use of tourniquet. Wound debridement and cleaning. Antibiotics and antitetanus treatment (toxoid and antitetanus globulin).

Note: ORF: It is a rare, benign, self-limiting exanthematous disease, also known

as contagious pustular dermatitis or infectious labial dermatitisor ecthyma contagiosum or thistle disease or scabby mouth caused by a parapox

Figs. 1.274A and B: Indeterminate and untidy hand injuries.

Skin grafting or flaps for skin loss. Tendon suturing or tendon graft for tendon injuries. Rest and elevation of the affected parts. Management of fractures by splint, wiring. Nerve repair for nerve injuries. Immobilisation up to 21 days. Later physiotherapy with warm, exercise, was bath active movements. Microsurgical restoration of digits. Reimplantation of the digits. Amputation of digits or metacarpals on ly when inevitab le. Fig. 1.275: Hand injury exposing tendons. Note the marker stitch in the tendon. It needs local transposition flap or groin flap to cover. Skin grafting is not possible over tendons.

155

....

"... rt)

Primary repair of tendons and nerves are of lesser priority in untidy injuries. Priority is wound debridement/wound excision and early skin cover. Cut ends of nerves and tendons are tagged with coloured stitches for future identification purpose.

IC

I]] COMPLICATIONS ANO MORBIDITY OF HANO INJURIES

':c: ::c

Infection; Osteomyelitis Arthritis of joints; Stiffness Loss of function due to disability

::::s rt)

QI

Ill C

... ... rt)

QI

::::s

Q. QI

::::s

Q. "T1

0 0 ,+

Fig. 1.276: Stuck finger by a ring. It is removed by applying soap, fat, and wax. String method is winding and unwinding a thread under and across the stuck finger. Sawing is done only when every method fails.

Fig. 1.278: Hand injury which is healing but with severe deformity of fingers.

Fig. 1.277: Avulsion injury of finger causing raw area.

•·· Primary suturing if it is a incised wound or delayed primary suturing if there is oedema.

Fig. 1.279: Typical deformity of finger which needs correction for proper function.

Disease is the fate of poor, but also punishment of rich.- lvo Andrick

156

T

In hand

Do's

Don'ts

Do examine hand carefully

Do not incise every infected digit

Do think of other diagnosis

Do not make puncture incisions or over pads

Do wait for abscess to localise

Do not injure the digital nerves or vessels

Do place adequate length and depth of incisions

Do not place incisions crossing the crease line

Do immobilise, elevate the hand

Do not close human bites or lacerated wounds

Do give antibiotics and proper dressings

Do not forget to send pus for culture and sensitivity

OUPUYTREN'S CONTRACTURE Individuals pre-disposed to the affection we are describing, observe that it is more difficult to extend the fingers of the affected hand.. . The first (interphalangeal joint) is flexed at nearly a right angle ... the most powerful efforts are insufficient to extend it.. .. Hence, it was natural to conclude, that the commencement of the disease was in the unusual tension of the pa/mar aponeurosis. -Guillaume Dupuytren, 1833

It refers to localised thickening of palmar aponeurosis and later formation of nodules with severe permanent changes in metacarpophalangeal and proximal interphalangeal joints. Terminal interphalangeal joint is not involved as palmar aponeurosis does not extend to terminal phalanx. It is common in males (10:1 ). It starts in ring and little fingers, with flexion of ring and little fingers. Later involving all fingers. There is thickening and nodule formation in the palm with adherent skin. It is often familial and bilateral 45%. Pads (of fat) develop in knuckles and are called as Garrod's pads (in proximal IP joints) . CONDITIONS OFTEN ASSOCIATED WITH

Plantar fasciitis 5%-Ledderhose's disease Mediastinal and retroperitoneal fibrosis • Peyronie's disease of penis 3% • Nodules in the face and ear • Pellegrini-Stieda's disease Note:

Dupuytren's contracture typically affects ring finger; years later little finger is affected; but in 30% of cases little finger is primarily affected - Peter F Early.

Aetiology: ;., Repeated minor trauma, use of vibrating tools. ;., Cirrhosis, alcoholism, smoking, ,,. Epileptics on treatment with phenytoin sodium. ,,. Diabetics, pulmonary tuberculosis, acquired immunodeficiency syndrome (AIDS). ;.. Other metabolic conditions. ;.. Familial-autosomal dominant.

Figs. 1.280A and B: Dupuytren's contracture in the hands. Note the involvement of the ring finger. Note the fibrous band on the other hand as early finding.

Complications: , Restriction of hand function and so disability. , Arthritis of metacarpophalangeal (MCP) and proximal interphalangeal (IP) joints. Treatment: , Fasciotomy of palmar aponeurosis and later physiotherapy, Z plasty. ;.. In severe cases fasciectomy partial or complete. Treatment of the cause. Recurrence can occur in 5-50% cases.

Fig. 1.281 : Z plasty done for Oupuytren's contracture.

VOLKMANN'S ISCHAEMIC CONTRACTURE It is a vascular injury leading to muscular infarction and subsequent contracture. •·· Causes: ,, Supracondylar fracture of the humerus. , IV chemotherapy; Burns. Closed forearm crush injuries. Tight plaster after reduction of fracture. Pathogenesis: Injury of brachia! artery (tear, contusion, spasm, compression)

Results in infarction Injury to median nerve of forearm flexor (mainly) and ulnar nerve muscle both by ischaemia and J, infarction Aseptic muscle necrosis J, Fibrosis of flexor muscle of forearm J, Contracture Features: Acute phase: Pain (persistent pain in forearm, hand, fingers- ominous symptom). Pallor; Puffiness (due to oedema).

Pulseless (absence of radial pulse; but its presence does not rule out the onset of impending contracture). Paresis. Late phase: Deformity ~

)>

I - -·w~i;t joint extended.

Extended metacarpophalangeal joints. Flexed interphalangeal joints. Volkmann's sign: In early stage, the fingers can be extended at the interphalangeal joints, only when the wrist is flexed fully. The fingers tend to flex if any attempt to extend the wrist is made.

Treatment: In acute phase: ;.. Removal of plastic cast applied after fractu re reduction. , Correction of fracture. Exposure of brachia! artery and application of 2.5% papaverine sulphate to relieve the spasm if any. ;.. Suture of arterial tear if present, often with placement of arterial graft. , Lateral incision over the deep fascia of forearm is placed to decompress the oedema. In late phase (once deformity occurs): :..- Physiotherapy, Dynamic splints. ;.. Max-Page operation: Release of flexor muscles (forearm muscles) from their origins from the bone and allowing it slide down until full extension. , Excision of fibrou s tissue and damaged muscles along with tendon transfer; Arthrodesis.

SYNDACTYLY It is webbing or fusion of fingers. Causes: Congenital and hereditary-common; Traumatic like burns. TYPES

--

Cutaneous-simple. Fibrous. Bony-complex. It can be unilateral or bilateral. Often all fou r limbs may be involved with webbing of toes. It may be associated with polydactyly or visceral anomalies. If bony type is suspected, X-ray of the part should be taken.

Treatment: ,. If cut ised which leads to gangrene of the digit. If fibrous, release can be done. ;..

If bony type, release is difficult because blood supply may

be compromised which leads to gangrene of the digit. Note: • Malle/ finger {Base ball finger): The terminal phalanx can not be

extended because of tear at insertion of extensor tendon or avulsion fracture of the base of the terminal phalanx.

"Impossible" is a word found only in the dictionary of fools.~apoleon

'"'

::x:

ty {due to injury to median nerve):

m

157

-0 --f

m

:;%:>

158

...>-

• Hsberden's nodes: These are seen in osteoarthritis, occurring behind the distal interphalangeal joints of index, middle, little and ring fingers. • Spina ventosa: Refers to phalangeal tuberculosis ( Tuberculous dactylitis). It is called as spina ventosa because of its appearance as "air- filled balloon".

QI

ei

Nerve supply is by saphenous, sural, posterior tibial, superficial and deep peroneal nerves.

CALLOSITY It is a hard, thickened skin occurs as a protective measure seen in wider area usually over heel and heads of metatarsals.

::::,

....0

Ill

,a ::::, C:

,a Ill

cc

a:

Ill

Fig. 1.282: Syndactyly. Fig. 1.286: Callosity in the foot.

Fig. 1.283: Syndactyly and polydactyly of both hands and both feet.

A callosity protrudes outwards from the skin. It is greyish-brown , raised, protruded outwards, thickened, hypertrophic skin occurs due to occupation and skeletal structure. It is painless. It is wider lesion. Paring the top layer exposes the shiny translucent dead skin beneath. It is grayish brown hypertrophic raised thick protective phenomenon which occurs commonly in areas of wear and tear like hands and feet. Top roughened layer when peeled off, shiny, translucent, homogenous dead skin can be exposed beneath. It is painless; it can get rubbed easily to create a sore. As it is a protective phenomenon it is best left alone. Corn

Fig. 1.284: Arthritis of joints of hands.

Dermis

Fig. 1.287: Diagram showing differences between corn and callosity.

CORN Types: (1) Hard corn, (2) Soft corn. Fig. 1.285: Spina ventosa.

FOOT Foot contains 7 tarsal bones, 5 metatarsals, 14 phalanges (total 26 bones). Two sesamoid bones of 1st metatarsal bone are common. There are 4 layers of muscles in foot. Ligaments, muscles, joints, maintain the stability of toot complex. Blood supply is by anterior tibial, posterior tibial and peroneal arteries.

Fig. 1.288: Corn in the plantar aspect of the foot.

Hard Corn It is localised area of thickening over a bony projections like heads of metatarsals. Histologically it differs from callosity by having severe keratoses with a central core of degenerated cells and cholesterol. It presses over the adjacent nerves causing pain. It can get infected causing severe pain and tenderness with inability to walk. It is sma//erlesion which is pushed deep into the skin forming a localised palpable painful/tender nodule with a central yellow-white core of dead cornified skin. Corn is common if there is deformity or by wearing tight fitting shoes/foot wears. Corn is narrow, deep and painful/tender. It is common in females. Corn is usually white/gray/yellow coloured, deep seated lesion. Infection, abscess formation and ulceration can occur especially if patient is diabetic. Corn may be associated with bursae causing bursitis. Corn often recurs after excision. Treatment: ,. Excision. ,. Local application of salicylic acid preparations or mixture of salicylic acid/lactic acid/collodion may be helpful. Skin softening agents are also tried. .,. Eliminating the pressure is very important to prevent recurrence. ,. Avoid excision of corn unnecessarily in diabetic (especially with neuropathy) and in ischaemic foot. Soft Corn It usually occurs between 4th and 5th toes due to friction of bases of adjacent proximal phalanges.

Both medial and lateral sides of the toe can be involved. Recurrent attacks of acute and subacute paronyc hia occurs. Pain , tenderness, swelling of margins of the toe, often along with granulation tissue and foul smelling discharge. Staging: (1) Embedded toenail edge with swelling and erythema; (2) Infection, painful discharge from nail edge; (3) Soft tissue hypertrophy, chronic infection, granulation tissue formation. Treatment: ,. Regular dressing and packing. Antibiotics. Discharge is sent fo r culture and sensitivity. ,. Nails should be cut concavely or straight without leaving lateral spikes towards soft tissues. ,. Partial or complete matrlxectomy: In recurrent disease or in stage 3 disease partial or complete matrixectomy is done. In partial matrixectomy, soft tissue resection is done; then ingrown toe nail with that part of the nail root (germinal matrix) is excised properly. In complete matrixectomy, entire nail with its root is removed. Incisions may be 45° angled with root flap (Zadik's/Fowler's) or with L shaped incision (Frost) or 'D' shaped excision of the nail root (Winograd) or 'H' shaped incision with removal of the entire nail, nail root, nail bed with diseased soft tissue resection (Kaplan). Zadik-Fowler's radical nail excision (1950) as partial or complete matrixectomy is commonly used with flap elevation at angles. Phenol matrixectomy, laser and radiosurgical matrixectomy are also currently in use.

PLANTAR FASCIITIS (Policeman's Heel) It occurs due to friction or tear of the ossified posterior insertion of the plantar fascia which is common in people who stand or walk for long-time. Treatment Analgesics, rest, steroid injections to the site.

Hyponychium (Subungual space)

INGROWING TOE NAIL (Onychocryptosis) It is also called as embedded toe nail. It is due to curling of the side of nail inwards, causing it to form a lateral spike resulting in repeated irritation and infection of overhanging tissues in the nail fold. Causes: Tight shoes; Improper cutting of nails (very short and convex). It is common in great toe and is often bilateral.

Hypertrophied infected tissue

Incision for ingrowing toe nail

Fig. 1.289: Zadik's or Fowler's operation.

You become successful by helping others to become successful.

159

Ill

al

Fig. 1.291 : Hallux valgus deformity.

Nail

c::

[!I

Figs. 1.290A to C: (A and B) Ingrowing toe nail. Note the granuloma caused by repeated infection and inflammation; (C) Incision tor nail excision. Note the germinal matrix. Removal of nail, entire or partial with adjacent germinal matrix is called as radical nail excision. Note: • Onychogryphosis: It is curving of nail upwards (Ram's Hom Nai~; It

occurs due to repeated trauma or fungal infection. • Onychomycosis: It is fungal infection of the nail.

ATHLETE'S FOOT It is the fungal infection of the skin between thetoes-Tinea pedis. Fungi enter th rough cracks; survive due to moisture in between toes. Skin is swollen, red, with sticky fluid, macerated with blisters. Itching, deep cracks, pain and discharge are common. Part should be kept dry. Cotton, clean socks should be worn. Oral antifungals, antihistaminics and topical antifungals are used. Condition is contagious.

HALLUX VALGUS Here great toe is deviated laterally at first metatarsophalangeal joint. There is outward deviation of great toe with medial deviation of first metatarsal head. It may be due to persistent lateral force or occasionally hereditary. Condition is often bilateral. It is common in females. Thick walled bursa (bunion) over medial aspect of the head of the first metatarsal bone is common. Undue prominence of head of first metatarsal bone is typical

Fig. 1.292: Hallux varus deformity. It is opposite of

hallux valgus deformity. often forming an exostosis at this point. Osteoarthritis of 1st metatarsophalangeal joint can occur. Lateral deviation of proximal phalanx over 2nd toe causing crowding of the toes. Initially it is painless; but eventually pain and tenderness develops with infection of bu nion and splaying of forefoot. X-ray shows deviation with often osteoarthritis of the metatarsophalangeal joint.

Treatment

,

Keller's operation: Proximal 113rd of the proximal phalanx of great toe and medial part of head of 1st metatarsal bone is excised through medial curved incision. Soft tissue interposition is done. ,. Mayo's procedure: Medial part of base of the proximal phalanx of great toe and head of 1st metatarsal bone is excised- opposite of Keller's. Simmond's procedure: Varus osteotomy at the base of 1st metatarsal bone with reinsertion of adductor hallucis tendon is done. , McBride procedure: Transfer of adductor hallucis tendon and lateral head of flexor hallucis brevis from proximal phalanx of great toe to the lateral part of head of 1st metatarsal bone. ,. Arthrodesis of metatarsophalangeal joint is done to relieve pain. ,. Excision of bunion, deformity correction, osteotomy, muscle transfers are also done as a combined approach.

L. Arterial Diseases

( iHAPTER OUTLINE

• • • • •

• • •

•

.. • •

•

.. ..• • .. •

•

..•

Surgical Anatomy of Thoracic Outlet Arteries of Upper Limb Arteries of Lower Limb Arterial Diseases Intermittent Claudication Rest Pain Limb lschaemia Different Levels of Arterial Obstruction Other Features of Poor Circulation Investigations for Arterial Diseases Diseases of the Arteries Atherosclerosis Thromboangiitis Obliterans Takayasu's Pulseless Arteritis Raynaud's Phenomenon Temporal Arteritis Treatment of Arterial Diseases Subclavian Steal Syndrome Acute Arterial Occlusion Traumatic Acute Arterial Occlusion Embolism Reperfusion Injury

• • • • •

• •

• • •

..•

..• ..• • ..• .... ..•• •

Saddle Embolus Embolectomy Fat Embolism Air/Gas Embolism Therapeutic Embolisation Caisson's Disease or Decompression Disease Aneurysm Mycotic Aneurysm Abdominal Aneurysm Abdominal Aortic Aneurysm Peripheral Aneurysm Carotid Artery Aneurysm Dissecting Aneurysm Erythromelalgia Livedo Reticularis Polyarteritis Nodosa Scleroderma/Systemic Sclerosis Acrocyanosis Gangrene Diabetic Foot and Diabetic Gangrene Frostbite Ainhum Endovascular Surgeries Upper Limb lschaemia Arterial Substitutes

SURGICAL ANATOMY OF THORACIC OUTLET Thoracic outlet is bounded by manubrium sternum in front, spine posteriorly, and the first rib laterally. At the superio r aperture of thorax subclavian vessels, brachia! plexus traverse the cervicoaxillary canal to reach the upper limb. Cervicoaxillary canal is divided into proximal Costoclavicular space and distal axil/a (divided by first rib) .

Costoclavicular space is bounded superiorly by clavicle, inferiorly by first rib, anteromedially by the costoclavicular ligament, and posterolaterally by scalenus medius muscle along with long thoracic nerve. Scalenus anticus muscle divides the costoclavicular space into two compartments, the anterior one containing subclavian vein and the posterior one containing subclavian artery and brachia! plexus. This posterior compartment is called as Scalene triangle bounded by scalenus anticus anteriorly, scalenus medius posteriorly, and the first rib inferiorly. Cervical rib narrows this triangle and causes compressive features of the C8, T1 nerve roots and subclavian artery. Anything that narrows costoclavicular space causes Thoracic outlet syndrome.

ARTERIES OF UPPER LIMB Right subclavian artery begins from brachiocephalic trunk (innominate artery) whereas left subclavian artery arises directly from the arch of aorta. From underneath the sternoclavicular joint artery arches over the pleura and apex of lung about 2.5 cm above the clavicle and then reaches the lateral border of first rib to continue as axillary artery. Subclavian artery is divided into three parts by scalenus anterior muscle. Axillary artery is divided into three parts by pectoralis minor muscle. At the lower border of teres major muscle it enters the arm and continues as brachia/ artery. About 2.5 cm below the crease of the elbow joint, it bifurcates into radial and ulnar arteries which run in the forearm. Ulnar artery forms the superficial palmar arch which is completed by superficial palmar branch of radial artery . Radial artery after passin g through the anatomical snuff box enters the dorsum of hand and first intermetacarpal space to form deep palmar arch . It is completed by deep palmar branch of ulnar artery and is 1 cm proximal to superficial palmar arch.

ARTERIES OF LOWER LIMB Abdominal aorta bifurcates at the level of fourth lumbar vertebra (corresponds to the level of the umbilicus in anterior abdominal wall) into two common iliac arteries.

To be calm is the greatest manifestation of the power-Swami Vivekananda.

...>QJ

...::::,Cl

V'l

0

::::, C:

:E _.,, cc

0:: V'l

Common iliac artery is about 5 cm in length passes downward and laterally; and at the level of lumbosacral intervertebral disc, anterior to sacroiliac joint, it divides into external and internal iliac arteries. Internal iliac artery supplies pelvic organs. External iliac artery continues as common femoral artery at the level of inguinal ligament. About 5 cm below the inguinal ligament common femoral divides into superficial femoral and deep femoral (Profunda femoris) arteries. Deep femoral artery provides collateral circulation around the knee joint and also communicates above with gluteal vessels to maintain collateral circulation around the gluteal region. Superficial femoral artery at the hiatus in the adductor magnus, continues as popliteal artery up to the inferior angle of the popliteal fossa where it divides into anterior and posterior tibial arteries. Anterior tibial artery supplies anterior com partment of leg and ankle, continues as dorsalis pedis artery which forms dorsal arterial arch of the foot. Posterior tibial artery supplies posterior compartment of leg and ends as medial and lateral plantar arteries which forms plantar arterial arch of the foot. Posterior tibial artery gives peroneal artery which runs close to fibula supplying calf muscles.

,., In the abdomen causing pain, bloody stool. In the kidney causing haematuria. Dilatations are aneurysms. Arteritis. Small vessel abnormalities .

INTERMITTENT CLAUDICATION Claudio means "I limp" a Latin word. It is a crampy pain in the muscle seen in the lim bs. Due to arterial occlusion, metabolites like lactic acid and substance P accumulate in the muscle and cause pain. The site of pain depends on site of arterial occlusion: The most common site is calf muscles. Pain in foot is due to block in lower tibial and plantar vessels (70%). Pain in the calf is due to block in femoropopliteal segment. Pain in the thigh is due to block in the superficial femoral artery. Pain in the buttock is due to block in the common iliac or aortoiliac segment (30%), often associated with impotence and is called as Leriche's syndrome. Pain commonly develops when the muscles are exercising. Cause for pain is accumulation of substance Pand metabolites. During exercise increased perfusion and increased opening of collaterals wash the metabolites. BOYD'S CLASSIFICATION OF CLAUDICATION

Grade I: Patient complains of pain after walking, and distance in which pain develops is called as 'claudication distance'. If patient continues to walk, due to increased blood flow in muscle and opening of collaterals metabolites causing pain are washed away and pain subsides Grade II: Pain still persists on continuing walk; but can walk with effort Grade 111: Patient has to take rest to relieve the pain

\\ Figs. 1.293A and B: CT angiogram of aortoiliac segment showing aortoiliac block due to atherosclerosis. Collaterals are also welldeveloped.

B Arterial-typically develops after walking for certain distance and resolves rapidly within 5 minutes once walking is stopped Neurogenio-pain develops in standing or walking and disappears immediately after stopping walk; normal feeling pulses without ischaemic changes are present. It is usually due to narrow lumbar canal (spinal canal stenosis) Venous-it is rare but definitely occurs. It is observed in chronic pelvic venous obstruction as a mechanical high venous pressure. It is usually due to iliac vein thrombosis. Peripheral pulses are normal

ARTERIAL DISEASES Stenosis due to trauma, atherosclerosis, emboli. It may be: , In the brain causing transient ischaemic attacks. , In the limbs causing claudication and rest pain.

Note:

• Beta blockers may aggravate claudication. • Claudication is not that common in upper limb but can occur during writing or any upper limb exercise.

•

Criteria to diagnose intermittent claudication: (1) Cramp like pain

in a muscle-calf muscle. (2) Pain develops only when muscle is exercised. (3) Pain disappears when exercise stops within 5 minutes. • Intermittent claudication is due to anaerobic muscle metabolism. • Superficial femoral artery is most commonly affected (70%) causing calf claudication. • ABPI decrease by 0.1 below the 0.9 carries 10% increase in relative risk (RR) of major cardiovascular disease in patient with claudication.

REST PAIN It is continuous aching in calf or feet and toes or in the region even at rest depending on site of obstruction.

FEATURES OF ARTERIAL STENOSIS/BLOCK IN LIMBS

163 1

Intermittent claudication, rest pain Cold periphery, numbness, paraesthesia Colour changes, ulceration, gangrene Altered sensation and decreased function/movements Diminished/absent arterial pulsation Thrill/bruit over the stenosed artery Altered venous filling-normally it is in few seconds; it is delayed in arterial stenosis; it is rapid in AV fistula

l

("\

:I:

l>

'"O

-I

m

:x, -'

'

LIMB ISCHAEMIA

I

It is 'cry of dying nerves' due to ischaemia of the somatic Causes nerves. It signifies severe decompensated ischaemia. Pain Atherosclerosis. gets aggravated by elevation and is relieved in dependent Embolism (acute). position of the limb. Arteriopathies-Bu erg er's disease, Raynaud's disease, Pain is more in the distal part like toes and feet. It gets aggraTakayasu's disease. vated with movements and pressure. Diabetes. Hyperaesthesia is common association with rest pain. Scleroderma. Rest pain is increased in lying down and elevation of foot; it Physical agents-trauma, tourniquet, radiation injury. may be reduced on hanging the foot down. Rest pain is worst at night and so patient is sleepless at Classification of Limb lschaemia night. During sleeping at night heart rate and blood pressure diminishes which further aggravates the hypopertusion and m ~FONTAINE CLASSIFl~ATION OF LIMB ISCHAEMIA rest pain. Stage 1: No clinical symptoms Rest pain is apparently reduced by holding the foot with Stage 2: Intermittent claudication hand , probably due to suppression of transmission of pain - 2a: Well-compensated-can walk >200 metres sensation. - 2b: Poorly compensated- walk only 2 weeks or ulceration or gangrene of the foot or toes with an ankle systolic pressure , ...:::ICl (I)

V'I

0

tO :::I C tO

_.,, a:i

cc: V'I

normal atheroprotective features of endothelium (barrier function; antiadhesive effect; antiproliferative effect on smooth muscles of arterial wall). Progressive atheromatous plaque formation, thrombosis, migration and proliferation of vascular smooth muscle cell occur. Migrated smooth muscle cells into intima act as neointima and this migration is stimulated by PDGF (platelet-derived growth factorreleased by endothelial smooth muscles, platelets) ; which (this migrated smooth muscle) newly becomes secretory to produce large quantity of matrix of the plaque. Lipid (LDL) gets oxidised to release factors which promote inflammation and coagulation and factors which prevent production of protective nitric oxide. Macrophages stabilise the plaque. Pathology constitutes of atherosclerotic plaque wh ich contai ns smooth muscle cells, connective tissue matrix, macrophages and lipid (the feature of atherosclerosis) . Ulceration and calcification occurs in these plaques. Ulcerated plaque is highly thrombogenic causing thrombosis and further critical block of the vessel leading to tissue ischaemia and infarction distally. Plaques are more at the dividing junctions of the artery where stress and shear force of the blood flow is more. Plaques are dynamic in nature with progression and regression phases. Plaque progression has got a unique ability of adaptation so that as the plaque progresses, lumen caliber is been tried to be preserved until critical stage occurs. Stenosis more than 40% is said to be critical. Beyond this, compensatory mechanism fails causing rapid progression and further stenosis of lumina. Stenosis more than 40% causes atrophy of tunica media making arterial wall mechanically unstable leading into dilatation and aneurysm. Common arteries involved are-infrarenal part of abdominal aorta, coronary arteries, iliofemoral vessels, carotid bifurcation , popliteal arteries. It is less common in upper limb arteries, common carotid, renal and mesenteric arteries.

I Features and Evaluation It is common after 50 years, but can occur at earlier age group. It occurs in males and females. Family history is common. Smoking, hypertension, diabetes, raised cholesterol are common causes. Veins are not diseased. Arterial wall is thickened on palpation. Thrill and bruit over femoral, renal, carotid arteries may be felVheard. It suggests localised stenosis with turbulence of blood flow. Features of ischaemia in the affected limb seen. Absence/ feeble pulses including main arteries of the limb-femorals. Abdomen should be examined for aortic aneurysm. Transient ischaem ic attacks, chest pain, eye problems, mesenteric ischaemia, altered renal function may be associated.

Blood sugar, fasting lipid profile, Doppler, angiogram (CT/ DSA), US abdomen, ECG, echocardiography are essential investigations. Angiogram shows typical narrowed artery, site, extent, percentage of stenosis, and collaterals. Antiphospholipid antibody (APLA) estimation is done to identify antiphospholipid antibody syndrome (APLS/Hughes' syndrome). It is an autoimmune, hypercoagulable state caused by antiphospholipid antibod ies. APLS provokes thrombosis in both arteries and veins as well as causes pregnancy-related complications such as miscarriage, stillbirth, preterm delivery, and severe preeclampsia. It causes lower limb DVT (venous) and arterial thrombosis (causing stroke); it is diagnosed by ELISA test for APLA. It is treated by aspirin and anticoagulant (Warfarin).

B • Narrowing of the arteries: Coronary, cerebral, renal, mesenteric, iliofemoral. > 70% narrowing (severe) causes reduced blood flow. lschaemia, ulcerations, gangrene can occur • Embolism: From atheromatous plaques Aneurysm formation: Mainly abdominal aorta and also in peripheral arteries • Fatty streaks: Fibrous atheromatous plaque with fibrous, lipid and basal Complicated plaque with calcification, ulceration, narrowing

I Management Risk factor modification: Avoid smoking; control of hypertension, diabetes, hypercholesterolaemia; weight reduction by diet, and exercise. Drugs: Antiplatelet agents (aspirin 75 mg, clopidogrel 75 mg); cilostazol 50 mg bd; atorvastatin to reduce cholesterol; pentoxiphylline. Percutaneous transluminal angioplasty (PTA) is very useful for iliac blocks and lower limb blocks. Surgeries: Thrombectomy, endarterectomy, profundaplasty. ,, Reverse/saphenous vein graft. , By pass grafts-iliofemoral, aortofemoral, iliopopliteal, femorofemoral grafts. ,. Amputations if limb is gangrenous-toe/below knee, above knee. Forefoot and Syme's amputations are not feasible in vascular conditions. Note:

Lumbar sympathectomy and omentoplasty are not much useful in atherosclerotic limb. 0mental vessels as such are often poorly perfused in atherosclerotic patients dueto involvement of coeliac trunk.

I Aortoiliac Occlusive Disease Common site of symptomatic atherosclerotic occlusive arterial disease of lower limb is infrarenal abdominal aorta and iliac arteries. Aortic bifurcation is the most common site of occlusion. Often disease may also extend into infrainguinal level.

I Types of Aortoiliac Occlusive Disease Type I: Disease localised to distal abdominal aorta and common iliac arteries. Type II: Wide spread aortic and iliac disease. Type Ill: Multiple level diseases along with infrainguinal diseases.

leak, aortovenacaval/aortoduodenal fistula, mesenteric ischaemia (colonic), impotence. ,. Indirect extra-anatomical bypass: It is quicker and technically easier and is suitable to patients who cannot tolerate anatomical bypass. Axillo-bifemoral graft is used, only in such occasional situation. ,. Nonoperative catheter-based endovascular procedure: If stenosis is less than 5 cm percutaneous transluminal angioplasty (PTA) with or without intravascular stents can be done. It is useful for single or multiple short focal stenoses. It is now proved that long-term patency of PTA is equal to surgical intervention.

171

n

:I: )> ""C

-t

m

::D

.....

,C\ (I)

::,

...

(I)

I lnfrainguinal Arterial Occlusive Disease Type I

Distal aorta and common iliac arteries

Type II

Widespread abdominal aorta and iliac arteries

Type Ill

Multiple level diseaseabdominal, iliac, infrainguinal arteries

Fig. 1.309: Types of aortoiliac occlusive disease.

I Features Common in 5th and 6th decades. Common in males. Claudication in buttock, Leriche syndrome with impotence, distal ischaemia are the features. Femoral artery pulsations below are absent. Systolic bruit over aorta and iliac arteries may be heard suggesting stenosis. Atheromatous plaque may dislodge and may cause em bolus causing acute presentation. Aortic angiogram is diagnostic.

It is either part of type 111 aortoiliac disease (aortoiliac femoral) or femoropopliteal tibial disease. Superficial femoral artery is most commonly involved. Involvement of long segment of the artery is common. Occasionally, a short stenotic segment may be present.

I Management If the popliteal artery below knee is patent femoropopliteal bypass is the ideal procedure used. Otherwise one of the patent branches is used for bypass. In situ saphenous vein graft is ideal; reverse saphenous vein graft or synthetic femoropopliteal graft can also be used. Profundaplastymay be done to improve the collateral ci rculation through profunda femoris (deep femoral).

Management Treatment for diabetes, hyperlipidaemia, etc. Surgical treatment is the mainstay. Direct anatomical reconstruction - Aortoiliac endarterectomy is reboring/disobliteration procedure useful for type I disease. Diseased intima, plaquewith thrombus is removed by arteriotomy along the entire length which is closed later using 4-zero/5zero polypropylene continuous sutures (open endarterectomy). In lengthy disease, after making two small arteriotomies at proximal and distal diseased parts, endarterectomy loop is passed to remove the intima with diseased plaque (semi-closed endarterectomy). Advantages: It avoids prosthetic graft and its complications. Problem is- reocclusion and restenosis. Aortofemoral bypass graft is the gold-standard surgical procedure for type I and II disease. Long-term patency rate is 70- 80%. Woven Dacron graft is used. Complications are-bleeding, thrombosis, embolisation, graft blockage, graft failure, graft infection, graft

Figs. 1.310A and B: Aortofemoral bypass graft.

Character is like a tree; reputation is like its shadow. Theshadow is the one we think of but actually tree is the real thing.

QI

VI C:

... \0 ... ':': ... ...iii' (I)

)>

( I)

172

Note: Percutaneous transluminal angioplasty (PTA) in infrainguinal blocks is occasionally useful, only when stenosis is short and well-localised; otherwise it is not a good option. Angioplasty with laser drilling is often tried.

THROMBOANGIITIS OBLITERANS Syn. Buerger's Disease The disease (occurs) in young adults between the ages of twenty and thirty-five or forty years .... Upon examination we see that one or both feet are markedly blanched, almost cadaveric in appearance, cold to the touch, and that neither the dorsalis pedis nor the posterior tibial artery pulsates.. .. After months... trophic disturbances make their appearance .... Even before the gangrene, at the ulcerative stage, amputation may become imperative because of the intensity of the pain. - Leo Buerger ___ 1908 (Professor of Urology, 1879 to 1943)

It is adisease very commonly seen in young and middle-aged males; seen in smokers and tobacco users; not usually seen in females due to genetic reasons (but can occur in females very rarely). It is more common in Israel, Japan and India. The disease is most common in South Asian. Almost always starts in lower limb, may start on one side and later on the other side. Upper limb involvement occurs only after lower limb is diseased. Only upper limb involvemnt can occur (not uncommon) but it is rare. It is nonatherosclerotic inflammatory disorder involving medium sized and distal vessels with cell mediated sensitivity to type I and type 111 collagen. It is common in Jewish people; it is rare even in female smokers. Hormonal influence, familial nature, hypersensitivity to cigarette, altered autonomic functions are probable different causes. Lower socioeconomic group, recurrent minor feet injuries, poor hygiene are other factors. •·· It is segmental, progressive, occlusive, inflammatory disease of small and medium-sized vessels with superficial thrombophlebitis often may present as Raynaud's phenomenon with microabscesses, along with neutrophil and giant cell infiltration, with skip lesions.

I Pathogenesis Smoke contains carbon monoxide and nicotinic acid -J.. r

Carboxyhaemoglobin

Causes initially vasospasm and hyperplasia of intima J, Thrombosis and so obliteration of vessels occur, commonly medium-sized vessels are involved. J, Panarteritis is common Usually involvement is segmental J,

Eventually artery, vein and nerve are together involved J, Nerve involvement causes rest pain J, Patient presents with features of ischaemia in the limb Once blockage occurs, plenty of collaterals open up depending on the site of blockage either around knee joint or around buttock Once collaterals open up, through these collaterals, blood supply is maintained to the ischaemic area J, It is called as compensatory peripheral vascular disease J, If patient continues to smoke, disease progresses into the collaterals, blocking them eventually, leading to severe ischaemia and is called as decompensatory peripheral vascular disease. It is presently called as critical limb ischaemia. It causes rest pain, ulceration, gangrene. Note: • There is vasospasm intimal panarteritis obliteration; tender, cord like veins with superficial migratory thrombophlebitis (30%); with nerve involvement due to vasa nervorum block/ spasm. Arterial lumen is blocked but not thickened like atherosclerosis. • In 10% disease is bilateral; 10% females may get the disease (but rare); 10% seen in upper limbs. • Large arteries are usually not involved by TAO.

Smoking index (SI)= Number of years of smoking

Number of cigarettes smoked per day SI >300 is a risk factor

Pack Years Index (PYI) = Number of years of smoking

Number of packets of cigarettes per day PYI >40 is a risk factor

Shianoya's criteria for Buerger's disease Tobacco use. Only in males Disease starts before 45 years Distal extremity involved first without embolic or atherosclerotic features Absence of diabetes mellitus or hyperlipidaemia With or without thrombophlebitis

----

_J

I Classification of TAO Type I: Upper limb TAO- rare. Type II: Involving leg/sand feet-crural/infrapopliteal. Type 111: Femoropopliteal. Type IV: Aortoiliofemoral. Type V: Generalised.

I Clinical Features Common in male smokers between the 20-40 years of age group. It is a smoker's disease.

173

n

:I: )>

-0

m

:x,

..... r-

".... Ill

:::,

Fig. 1.311: Dry gangrene of leg. Patient needed above knee amputation.

Fig. 1.3128 Figs. 1.312A and 8: Bilateral lower limb TAO causing gangrene of both feet. Patient needs amputation on both sides.

Intermittent claudication in foot and calf progressing to rest pain, ulceration, gangrene. Recurrent migratory superficial thrombophlebitis. Absence/Feeble pulses distal to proximal ; dorsalis pedis, posterior tibial, popliteal, femoral arteries. May present as Raynaud's phenomenon.

Ill Ill

V\ C:

IQ

....

Ill

':':

)> .... r+ Ill ....

iii"

0

ij;" Ill

I Investigations

Ill

"'

Ill

"'

Hb¾. Blood sugar, ABPI. Arterial Doppler and Duplex scan (Doppler+ B mode US). CT angiogram is useful especially when intervention is planned. • Transfemoral retrograde angiogram through Seldinger technique: - Shows blockage-sites, extent, and severity. - Cork screw appearance of the vessel due to dilatation of vasa vasorum. - Inverted tree/spider leg collaterals. - Severe vasospasm causing corrugated/rippled artery. - Distal run of/is amount of dye filling in the main vessel distal to the obstruction through collaterals. If distal run off is good then ischaemia is compensated. If distal run off is poor then ischaemia is decompensated.

Fig. 1.312A

Fig. 1.313: Gangrene foot.

•·· Transbrachial angiograrrr. If femorals are not felt, then trans· brachia! angiogram (through left side brachia! artery-left subclavian artery-and so to descending aorta) should bedone. Ultrasound abdomen to see abdominal aorta for block/ aneurysm. Vein, artery, nerve biopsy.

Fig. 1.314: Gangrene of all toes in a foot in TAO patient.

The art of seeing invisible things is called as 'clinical vision '.

_vi

a::i

0:::

Vl

Fig . 1.315: lschaemic ulcer foot in a TAO patient.

Fig. 1.319: Angiogram showing block in main vessel with opened up

collaterals and adequate distal run off.

Fig. 1.316: lschaemic features in upper and lower limbs (four limbs).

I Treatment Stop smoking. "Opt for either cigarette or limb, but not both."

Fig. 1.320: Angiogram showing adequate collaterals.

Fig. 1.317: Skip ischaemic ulcers are common in vascular diseases. It suggests severe ischaemia up to most proximal ulcer level.

Drugs

Fig. 1.318: CT angiogram of lower limb (leg area) showing segmental block.

Low dose of aspirin 75 mg once a day-antithrombin activity. Prostacyclins, ticlopidine, praxilene, carnitine. Clopidogrel 75 mg; atorvastatin 10 mg; parvostatin 40 mg; ci/ostazo/e 100 mg bid- is a phosphodiesterase inhibitor which improves circulation (ideal drug). All drugs act at the collateral level than on the diseased vessel. Analgesics, often sedatives, antilipid drugs like atorvastatin may be needed. Complamina retard (xanthine nicotinate) tablet which was used daily once earlier, is presently not in use. However, graded injection of xanthine nocotinate 3000 mg from day 1 to 9000 mg on day 5 is often practiced to promote ulcer healing, helps to increase claudication distance as a temporary basis. Low molecular dextran may be also used.

175

Naftidofuryl is useful in intermittent claudication; it alters the tissue metabolism. Gene Therapy: Intram uscular injection of vascular endothelial growth factor (VEGF) which is an endothelial cell mitogen that promotes angiogenesis.

n

:::i: )> ""C

Note: • Vasodilators and anticoagulants are of no use in TAO. • Drugs like pentoxiphylline increases the flexibility of ABC's and helps them reach the microcirculation in a better way so as to increase the oxygenation. Its efficacy is more in venous ulcer than arterial diseases (now).

m

.... r-

:::0

Care of the Limbs Buerger's position and exercise-regular graded isometric exercises up to the point of claudication improves the collateral circulation. In Buerger's position, head end of bed is raised; foot end of bed is lowered to improve circu lation.

B Patient is in supine position, legs elevated to 45 degree. Time taken for blanchi ng is observed and for 2 more minutes limb is kept elevated. Patient is made to sit in high sitting position with limb in lowered position for 2 minutes. Lastly patient is made in supine position for 5 minutes. This seq uence is done 5 times/session with 3 sessions a day.

Care of feet (Chiropady): Exposure of feet to more cold and warm temperature should be avoided; trauma even minor like nail paring or pressure at pressure points in feet shou ld be avoided. Dryness of feet and legs should be avoided by applying oil to the feet and legs. Footwear should be selected carefully. It is better to wear socks with footwear. Heel raise by raising the heels of shoes by 2 cm decreases the calf muscle work to improve claudication.

Chemical Sympathectomy Sympathetic chain is blocked to achieve vasodifatation by injecting local anaesthetic agent (xylocaine 1%) paravertebrally beside bodies of L 2, 3 and 4 vertebrae in front of lumbar fascia, to achieve temporary benefit. Long time efficacy can be achieved by using 5 ml phenol in water. It is done under C-arm guidance. Feet will become warm immediately after injection. Problems are-possible risk of injecting phenol into IVG/aorta, spinal cord ischaemia.

Surgery Omentoplastyto revascularise the affected limb. Profundaplasty is done for blockage in profunda femoris artery so as to open more collaterals across the knee joint (It often makes better perfusion to the knee joint and flap of below-knee amputation). Lumbar sympathectomyto increase the cutaneous perfusion so as to promote ulcer healing. But it may divert blood from muscles towards skin causing muscle more ischaemic.

1/l

... \0 ... ':c: ...... C: ll)

lschae ic ulcer fopt

Fig. 1.321: Bilateral TAO. Patient has undergone right-sided above knee amputation and left-sided lumbar sympathectomy. lschemic ulcer on left-sided foot is seen.

Amputations are done at different levels depending on site, severity and extent of vessel occlusion. Usually either belowknee or above-knee amputations are done. flzarov method of bone lengthening helps in improving the rest pain and claudication by creating neo-osteogenesis and improving the overall blood supply to the limb.

TAKAYASU'S PULSELESS ARTERITIS (Mikito Takayasu, 1938-0phthalmolo ist, Ja an It is progressive, initially symptomless panarteritis involving aortic arch and branches of aorta of unknown etiology, probably immunological. It is common in young females (85%) ; common in Japan; commonly subclavian artery is involved (85%); involves all layers of arteries of upper limb and neck; often bilateral. It remains unnoticed for long time.

I Features Fever, myalgia, arthralgia, upper limb claudication. Absence of pulses in upper limb/limbs, neck; hypertension. Fainting on turning the neck or change in position; atrophy of face. Thrill/bruit along major arteries of upper limb and neck are the features. Optic nerve atrophy without papilloedema. Weakness and paraesthesia of upper limb. Cerebral softening, convulsions, hemiplegia can occur. Occasionally it can be life-threatening. Myocardial infarction; embolism, ischaemia are other complications. DSA; MR angiography and Doppler are the investigations.

Learn to see, learn to hear, learn to feel, learn to smell-that is clinical method.

)> ll)

:?. QI

9. ll) "' QI

"'ll) VI

176

...>(l/

:::,

Treatment: , To suppress immunity prednisolone 50 mg/day and cyclophosphamide daily is given. , Vascular reconstruction.

RAYNAUD'S PHENOMENON

V'I

0

IO

:::, C:

IO

_.,,

co c:::

V'I

der the influence of a very moderate cold, and even at the height J summer, she (case 1) sees her fingers become ex-sanguine, mpletely insensible, and of a whitish yellow colour.... One might indeed have suspected that the local asphyxia was connected with a spasmodic state of the vessels,...a functional trouble localised to the arterioles immediately contiguous to the capillaries. _ _ _ _ _ _-_ M _ aurice Raynaud, 1862 (French Surgeon)

Fig. 1.322: Vasculitis can cause arterial insufficiency. "Raynaud's phenomenon" is an episodic, localized, vasospasm Collagen vascular diseases: Like scleroderma, rheumatoid (vasoconstriction) of small vessels (arteriolar -vasospasm) diseases causing vasculitis (all autoimmune diseases). of hands and feet that leads into temperature and colour Other causes: Cervical rib, Buerger's disease, Scalene changes with sequence of clinical features called as 'Raynaud's syndrome. syndrome' as initial pallor (blanching; vasospasm); then blue/ It is often associated with CREST syndrome (Calcinosis cutis, cyanosis (venous congestion); lastly red (congestion due to Raynaud's phenomenon, Esophageal defects, Sclerodactyly, rapid blood flow, reactive hyperaemia). Telangiectasia).

Sequence of clinical features due to arteriolar spasm. Local syncope: It is due to vasospasm, causing white cold palm and digits along with tingling and numbness • Local asphyxia: It is due to accumulation of deoxygenated blood as the result of vasospasm causing bluish discolouration of palm and digits with burning sensation (due to accumulated metabolites) • Local recovery: It is due to relief of spasm in thearteriole, leading to return of blood to the circulation causing flushing and pain in digits and palm (pain is due to increased tissue tension) Local gangrene: If spasm persists more than ischaemic time (more than one hour in upper limb), then digits go for ulceration and gangrene. Does not occur regularly but is an occasional phenomenon in the cycle

Coffman criteria for Raynaud's syndrome-"episodic attacks of well-demarcated reversible self-limiting colour changes for 1-20 minutes on exposure to cold/emotional stimuli and is symmetrical/bilateral lasting for 2 years".

I Causes for Raynaud's Phenomenon Raynaud's disease: , It is seen in females, usually bilateral. ,, It occurs in upper limb with normal peripheral pulses. ,.. It is due to upper limb (hand) arteriolar spasm as a result of abnormal sensitivity to cold. Patient develops blanching, cyanosis and later flushing as in Raynaud's syndrome. Occasionally, if spasm persists it results in gangrene. , Symptoms can be precipitated and observed by placing hands in cold water. Working with vibrating tools: Like pneumatic road drills, chain saws, wood cutting, fishermen travelling in machine boats-vibration white finger.

Raynaud's phenomenon can be: Primary Raynaud's phenomenon is an idiopathic vasospastic disorder without underlying identifiable causes. Usually there is no significant pain in primary type. Primary is probably due to increased sensitivity of alpha 2 receptors to nonepinephrine; decreased nitric oxide and endothelin 1 in endothelial cells; increased serotonin and thromboxane. It is common in females and younger age group. Usually it is bilateral involving all digits. Criteria for primary ar&-Vasospastic attacks precipitated by cold or emotional stress; Symmetric attacks involving both hands; Absence of tissue necrosis or gangrene; No history or physical findings suggestive of a secondary cause; Normal nail-fold capillaries; Normal ESR; Negative serologic findings (for antinuclear antibodies). Secondary Raynaud's phenomenon is vasospasm due to some underlying cause. Significant pain will be present especially during rewarming stage. There are positive autoantibodies; equal in both sexes; occurs at any age group; need not be bilateral.

I Features (of Raynaud's Disease) Commonly bilateral. Common in young females (5%); 10% of population. Raynaud's disease is common in western white women. Usually medial four digits and palm are involved. Thumb is spared. Features of pallor/blanching (syncope), dusky cyanosis (asphyxia), rubor/painful red engorgement(recovery) are the

presentation. Occasionally, if vasospasm becomes longer, gangrene or ischaemic ulceration supervenes along the tips of the fingers. Peripheral pulses (radial/ulnar) are normallyfelt. These pulses will be absent in upper limb TAO. Repeated attacks are common.

TEMPORAL ARTERITIS There is localised inflammatory giant cell infiltration of arterial wall (giant cell arteritis) involving superficial temporal, facial, retinal, upper limb, coronary and vertebral arteries. It is common after 50 years. Common in females (2:1). Claudication of facial muscles, ischaemic severe headache, tende r, thrombosed superficial temporal artery and its branches are the features. Retinal ischaemia leading into irreversible blindness is dangerous feature. Involvement of coronary artery may cause myocardial infarction. Temporal artery biopsy is diagnostic-shows giant cell granuloma with CD4+ T lymphocytes. High dose long-term prednisolone 80 mg/day is needed. In involvement of retinal artery IV hydrocortisone/methylprednisolone may be needed initially.

I Investigations Type is identified by angiogram of hand (DSA/MR angiogram), arterial Doppler/Duplex scan. Other investigations required are X-ray of the part, antinuclear antibody (ANA assay) tests specific for different conditions. Assessment of segmental blood pressure gradient from brachial-forearm-wrist-fingers; finger tip thermography; cold recovery time (normal is less than 10 minute, but in Raynaud's it is more, often up to 30 minutes); reactive hyperaemia time (pneumatic cuff is inflated and kept for 5 minutes and released to observe hyperaemia); nail fold capil lary microscopy; laser Doppler flux to assess microvascular perfusion of finger skin-are special methods of eval uations. Nailfold capil/oscopy (capillary microscope) will show abnormal capillaries; it distinguishes primary and secondary Raynaud's phenomenon. Other routine investigations for arterial diseases like blood sugar/lipid profile/hypercoagulability status.

TREATMENT OF ARTERIAL DISEASES

I a.

Medical

General Measures Stop smoking, reduction of weight, exercise. Change in lifestyle, care of feet. Control of diabetes and hypertension. Buerger's position and exercise.

I Treatment

Drugs Treat the cause. Avoid precipitating factors-protect from cold/proper dress/ Nifedipine, praxilene, pentoxiphylline, low dose aspiri n, hand warmer electrical or chemica l/hand gloves. Avoid prostacycline, dipyridamole, ticlopidine. smoking even though it is not direct etiological cause (other Clopidogrel (75 mg). than upper lim b TAO), but it may possibly aggravate the Cilostazol (type 111 phosphodiesterase inhibitor) 100 mg disease. Avoid vibrating tools. BO- inhibits platelet aggregation. Vasodilators/pentoxiphylline/low dose aspirin (75-100 mg Oral anticoagulants are used only it there is history of emboper/day). Calcium antagonist (nifedipine 20 mg) is useful. lism or atrial fibrillation. Steroids may be useful in case of secondary Raynaud's. Prostaglandins, growth factors, vascular endothelial growth ACE inhibitors, nitrates, endothelin inhibitors (bosentan), factor (VEGF), Eldecoy (blocks intimal and smooth muscle epoprostenol-prostaglandin a potent vasodilator (lloprost, cell proliferation), mesoglycan (breaks blood clot), testosa prostaglandin analogue) and antiplatelet drug (continuous terone, herbals like garlic (reduces viscosity of blood) are intravenous infusion can be given), iloprost-prostacycline other newer drugs under use and trial. analogue, PG E1, misoprostol (oral PG E1)-are all tried at B vitamins and folic acid reduces homocysteine level (which different stages of the disease. 1% topical glycerine trinitrate is a risk factor). is useful. Inositol, L-carnitine (1500 mg), magnesium 500 mg (not Cervical sympathectomy-is used for nonhealing digital in renal failure or with diarrhoea), vitamin E and Care other ulceration . Not very beneficial to Raynaud's syndrome. Localized digital sympathectomy by stripping off the advenagents often used to improve walking distance. titia of digital vessels often with that of radial and ulnar Heparin is used only in acute phase or embolism. arteries with resection of nerve of Henle (a branch of ulnar b. Surgery nerve in the forearm-is sympathetic innervation of the ulnar artery) is said to be effective. Procedure is done using Percutaneous transluminal balloon angioplasty (PTA}: operative microscope. Through transtemoral Seldinger approach, initially angiogram Nole: is done. Then under guidance (fluoroscopic) stenosed area Avoid oral contraceptives, beta blockers and ergot preparations in Raynaud's is approached. First guidewire is introduced through which

I

disease.

Not just 'Go' through the life, but better 'Grow' through the life.

177

.... rC)

/1)

... ::J

/1)

DJ

VI C:

... ... ....... /1)

)> /1)

::::!. DJ

S?. "' /1) DJ "'/1) V,

178

balloon catheter is passed. Balloon of the angioplasty catheter is inflated at stenosed area for one minute and repeated if required. Plaques should rupture. Catheter is withdrawn. It is useful in cases of localised stenosed areas. Note:

• Often nonexpandable or self-expandable stents are used if stenosed segment is not dilated adequately through balloon- PTA with stenting. • PTA for carotid artery stenosis is risky and not ideal as there will be possible release of microemboli during dilatation procedure which can precipitate stroke. Specialised balloon catheters with umbrella tip which can trap the microemboli may be used in these places.

Types ,.. Conventional: Here balloon is inflated along the lumen to break the plaque circumferentially. , Subintimal: Here balloon is inflated after passing subi ntimal plane to break the plaque.

Fig. 1.324: Percutaneous transluminal balloon angioplasty (PTA). Note the inflated balloons on both side iliac arteries.

Conventional PTA - -1111111- Medial/adventitia ~ =>---,-....__ . Intima a llii•lll!!ll.;--PTA guidewire PTA balloon Atheroma plaque -

- 11111!! ! -~~~---,~---c Subintimal PTA

Medial/adventitia lntima PTA guidewire PTA balloon ~ - - - -- - - - - A t h e roma plaque

Fig. 1.323: Conventional and subintimal types of PTA.

Complications ,., Thrombosis, bleeding, sepsis. , Embolism, dissection, retroperitoneal haematoma. , Pseuodoaneurysm formation. Advantages , It is done under local anaesthesia. ,., Procedure can be repeated if needed. ,., Stent can be placed at a later stage if needed. ,.. It is done when stenosis is less than 5 cm. In ideal indications its efficacy is equal to surgery.

Figs. 1.325A and B: DSA showing left-sided aortoiliac block and correction after doing PTA.

Disadvantages ,., It is less useful for lengthy blocks or stenosis more than 5 cm. ,..

It is dangerous to do in stenosis of carotid artery where

endarterectomy is ideal. Atherectomy: It is removal of atheroma either through open surgery or by percutaneous route from the wall of the vessels. Thrombectomy: It is removal of thrombus through an arteriotomy of larger vessels. Done in aortoiliac, femoropopliteal region. Endarterectomy: , It is removal of thrombus along with diseased intima through an arteriotomy. Endothelium of the vessel is removed, hence the name.

Figs. 1.326A and B: DSA showing superior mesenteric artery stenosis. It is corrected by PTA.

179

("\

:x:

.,,-l

)>

m

Internal iliac artery

:::0

r-

Inguinal ligamen Common femoral artery

Fig. 1.327: Thrombectomy. Atheroma

Superficial - - - femoral artery (blocked)

Fig. 1.329: Profundaplasty for deep femoral block.

9. "'IT> 0J

Atheroma intima removed _ _ _ _ _ _ _ _ _:,_ Ring stripper

Fig. 1.328: Endarterectomy technique. Both thrombus and diseased

intimaare removed through an arteriotomy. Often ring stripper is used to remove the atheromatous plaque.

,

It is done in carotid, aortoiliac and occasionally aortofemoral blocks. It is also called as disobliteration/reboring. There are three methods-(1 ) Open method-Arteriotomy is done along the entire diseased seg ment; endarterecto my is do ne by remo ving th rombu s, disease d inti ma with plaque along th e plane of media. Arteriotomy is closed using 5-zero polypropylene suture and patient is heparinised. (2) Semiclosed method- Here two art erioto mies are done on either ends of the level of obstruction ; loop endarterectomy stripper is passed from one end to complete the endarterectomy; two arteriotomies are cl osed. (3) Wiley's eversion endarterectomy-Here artery is cut transversely at the junction of diseased and normal nondiseased segment; diseased intima with plaque is circumferentially dissected; artery is

Profundaplasty: , It is done when there is localised block in opening of profu nda femoris (deep femoral). Profunda femoris is opened, thrombus if present, is removed. Opening is widened using either venous or synthetic (Dacron or PTFE) grafts. This procedure allows collaterals across the knee joint to open through profunda femoris and so gives good blood supply below-knee level and may prevent patient going in for above-knee amputation.

(May be able to save knee joint with below-knee amputation with better prosthesis.) , Lateral angiogram view is needed to identify the orifice of profunda femoris. Disease involves invariably only at the orifice without extending distally towards 1st perforator branch. Endarterectomy at the junction and closure with a venous patch widens the opening adequately. Reverse saphenous vein graft: In case of femoropopliteal block, saphenous vein is dissected out, reversed and sutured above to the femoral artery and below to popliteal segment so as to bypass the blood through reverse saphenous vein graft. Saphenous vein is reversed to nullify the action of valves so as to allow easy flow of blood. In situ saphenous vein graft: It is arterialisation of saphenous vein. Saphenous vein intact in same position is sutured above and below the blocked

everted ou t to extract the diseased intima like a tube;

femoropopliteal region to bypass the blood across. Venous

everted artery is reduced and sutured to normal end of the artery. Advantages are- it avoids prosthetic graft and its complications. Problem is- reocclusion and restenosis. Placement of intraluminal stent tor localised stenosis.

valves are removed through valvulotomy instrument so as to allow the blood to pass. Here nutrient su pply of vein is left intact with proximal part of the vein sutured to wider part of the femoral artery, narrow distal part is sutured to narrow part of the artery.

Absolute diagnosis are unsafe and are made at the expense of the conscience.-William Osler

"'IT> "'

180

Arterial/venous grafts: Synthetic:

Dacron woven graft Dacron knitted graft Polytetrafluoroethylene (PTFE) graft Natural:

Internal mammary artery (ideal one) Long saphenous vein either reverse or in situ Umbilical vein graft (cryopreserved)-3 mm vein is the minimum diameter required Grafts of different length and size are available

,

Different procedures: Aortofemoral bypass graft (end to side)-5% mortality; lleofemoral bypass graft; Femorofemoral bypass graft; Femoropopliteal graft; Femorodistal graft; Axillofemoral graft. ,, Problems with grafts: Leak, infection, thrombosis, cost factor, availability, reblock.

Cervicothoracic preganglionic sympathectomy: It is removal of 2nd and 3rd thoracic ganglia which contains cells of postganglionic fibres supplying the upper limb. Preganglionic white rami communicantes fibres from 2nd and 3rd sympathetic nerves enter thoracic T1 ganglion and supplies head and neck region through upper part of the stellate ganglion. Preganglionic sympathetic nerve entering the 2nd and 3rd ganglia from below, supplies sym pathetic fibres for upper limb through the lower part of the stellate ganglion. In cervical sympathectomy for upper limb ischaemia, lower part of stellate ganglion with Kuntz nerve is divided. For head and neck hyperhidrosis entire stellate ganglion should be removed which leads to development of Homer's syndrome. For hyperhidrosis of axillary area, along with stellate ganglion upper four thoracic ganglia has to be removed.

Nole: Angioscope is used to visualise the valves in saphenous vein or to visualise

the completion of the by pass grafts like femorodistal graft. Arterial graft

EIA Occluded vessel Bypass graft

Internal iliac artery

Fig. 1.330: Bypass graft (aortoiliac).

Fig. 1.332: Aortofemoral bypass graft (end to side). (EIA: external iliac artery; CIA: common iliac artery; IIA: internal iliac artery)

Common iliac artery Internal iliac artery External iliac artery

Inguinal ligament

Fig. 1.333: On table picture of aortofemoral arterial graft. Graft

Fig. 1.331 : Femoropopliteal bypass graft.

INDICATIONS

Cervical rib with vascular manifestations-useful Raynaud's phenomenon-useful Hyperhidrosis-very useful Upper limb vasospasm due to other causes- useful Acrocyanosis- useful Causalgia- very useful Sudeck's osteodystrophy

Approaches Supracfavicufar approach: Through a supraclavicu lar incision sternomastoid , (omohyoid is retracted or divided) scalenus anterior muscles, are divided. Phrenic nerve is displaced medially; subclavian artery is pushed downwards; thyrocervical trunk is identified and ligated securely, suprapleural membrane is depressed, stellate ganglion is identified in the neck of the first rib. All rami communicantes from second and third ganglia are divided. Grey ramus from second ganglion to first thoracic nerve called as Kuntz nerve, is also divided.

B Bleeding Injury to subclavian artery and nerves Pneumothorax and haemopneumothorax Homer's syndrome with ptosis, miosis, anhydrosis, enophthalmos Chylous fistula, chylocele ~ ost-sympathetic neuralgia

,

Transthoracic/Axiffary approach (Hedley Atkins): This gives better visibility and easier removal of rami, lower down compared to supraclavicular approach. Patient is placed in lateral position; transverse incision is made just below the hair bearing line; intercostobrachial nerve is preserved. Thorax is opened at 2nd space. Sympathetic chain is identified at the neck of 1st rib. ,. Thoracoscopic sympathectomyis the choice, and popular approach at present. Advantages are better visibility with magnification, less trauma of access (wound), faster recovery, and precise.

Lumbar sympathectomy. Indications , Peripheral vascular disease like TAO. , To promote healing of cutaneous ulcers. , To change level of amputation and to make flaps to heal better after amputation. , Causalgia of lower limb (it is common in upper limb). ,. Hyperhidrosis of lower limb is rare. Principle ,. It increases the cutaneous blood su pply thereby promoting healing of ulcer and skin flaps in amputation. It is a preganglionic sympathectomy. Ganglion L2 and L3 supplies legs below knee level. L1 supplies upper part of thigh and buttock region. L1 lies under the crus of diaphragm. L4 lies under the common iliac vessels below. , It increases the blood flow for 2-4 weeks by abolishing constriction of arterioles and precapillary sphincters (basal and reflux). It produces transient small increase in distal perfusion; increases the nutritive perfusion to promote ulcer healinq, alters the pain perception and pain impulse transmission temporarily. Procedure Under general or spinal anaesthesia, ganglia are approached through a transverse incision in the loin at the level of umbilicus, through extraperitoneal approach, by dividing external oblique, and internal oblique, and splitting transverse abdominis muscles. Interior vena cava on right side, aorta on left side are identified. Sympathetic chain is identified by its rami, over transverse processes of lumbar spines. L2, L3, L4, L5 ganglia are removed. L2 is identified by its size (Larger) and more number of rami. L1 is retained on one side in bilateral cases. If both are removed it will lead to failure of ejaculation and so sterility (Dry ejaculation).

B Injury to IVG or aorta Bleeding lumbar veins Spinal vessel spasm and so ischaemia of spinal cord and paraplegia, dry ejaculation Injury to bowel and ureter Wound infection and abscess formation Post-sympathetic neuralgia Paradoxical gangrene of opposite leg and foot

,. Its effects are only temporary (3-4 weeks). Long-term results aredoubtful. It can be combined with omentoplasty. , It can also be done along with below-knee amputation to increase the blood supply of skin flap so as to have better healing. , Limb will become warmer immediately after sympathectomy. Fig. 1.334: Cervical sympathectomy scar in the neck and patient has developed Homer's syndrome. Healed ulcer over tip of index finger on left side is seen. Patient is asymptomatic after sympathectomy.

Note: Lumbar sympathetic chain may be mistaken for lymph nodes, fat, tendon of psoas muscle, genitofemoral nerve.

Einstein died of ruptured aortic aneurysm. Nissen did cellphone wrapping of the aneurysm in Einstein

181

VI

...C \C ...

11)

':':

.........

)>

11)

iii. 0

iii. 11)

DJ

1/1

11)

1/1

182

Fig. 1.336C Figs. 1.336A to C: Omentoplasty procedure for

revascularisation of the ischaemic limb. Omentum is supplied by omental vessels Fig. 1.335: Lumbar sympathectomy scar with ischaemic ulcer foot

showing healing sign.

~ --

Coeliac artery .____ _

7

I ,.._ __ Gastroduodenal artery ~ ugh common hepatic artery2_l

Chemical sympathectomy. ,, It is done in lateral position using a long spinal needle under local anaesthesia. Position is confirmed by injecting dye under fluoroscopy. Later 5 ml of phenol in water or absolute alcohol is injected lateral to the vertebral bodies of fourth and second lumbar vertebrae. Care should be taken to see that the needle does not enter IVC or aorta. , Procedure is contraindicated in patients with bleeding disorders and in patients who are on anticoagulants. Omentoplasty:

B Peripheral vascular disease-to improve circulation For lymphoedema, it helps by providing lymphatics and so to drain lymph from the limb It is also tried for revascularisation of pharynx, cranial cavity

Right gastroepiploic

,...__

sptenic ~

I

M~- - ~ -

~ l

C

Omental vessels

Left gastroepiploicl a~ry

--_J

1

Four layers of omentum has got omental arcades of vessels (arc of Burkow). Omentum is also rich in lymphatics. It has got adhesive property. By retaining one of the pedicles, omentum can be mobilised so as to reach the limb to maintain the circulation. It can also be mobilised up to the ankle. It promotes ulcer healing, reduces the pain and controls the features of ischaemia. It can also be used in upper limb ischaemia. But, if patient continues to smoke, disease spreads to these omental vessels also. Often it can be mobilised to both lower limbs in bilateral diseases.

_J

Fig. 1.337: Incisions for omentoplasty in theabdomen

(upper midline) and in lower limb.

Abdominal sepsis lncisional hernia, whereomental pedicleis tunneled into the limb from the abdomen Adhesions and intestinal obstruction

_J

Procedure: Under general anaesthesia, abdomen is opened

Figs. 1.336A and B

with upper midline incision. Omental vessels are identified. Omentum with its blood supply is carefully mobilised to get an adequate length. Lengthened, mobilised omentum is brought into the subcutaneous plane through abdominal wall, lateral to the lower part of rectu s muscle. Later this pedicle is mobilised in the subcutaneous tunnel across the leg, burried in the deep fascia. Other treatment methods-. , Amputations are done at different levels depending on extent of gangrene, site of block, amount of collaterals.

Fig. 1.338: Below knee amputation stump which is also ischaemic.

Patient underwent below knee amputation for gangrene foot. But patient might need above knee amputation.

SUBCLAVIAN STEAL SYNDROME Following obstruction of the first part of the subclavian artery, vertebral artery provides collateral circulation to the arm by reversing its blood flow through basilar artery (from opposite vertebral artery and circle of Willis). It affects the blood supply of posterior part of the brain and affected side upper limb. Steal may be occult, partial or complete. Causes: Atherosclerosis (95%), embolism, Takayasu's arteritis, dissecting aortic aneurysm. It is more common on left side (3:1 ); common in males (2:1 ). Vertebrobasilar insufficiency symptoms. Dizziness, syncope, visual disturbances, diplopia, nystagmus, pulsatile tinnitus, vertigo, hearing loss. Upper limb ischaemia: Pain, heaviness, paraesthesiaand fatigue in the arm which is aggravated by exercise (arm claudication often with exercise). Sudden turning of the neck to the affected

side may precipitate symptoms. Radial pulses on both sides are asymmetrical (affected side it is feeble). Blood pressure on the diseased side will be 20 mmHg less compared to normal side. Javid test: Here compression of ipsilateral carotid artery makes ipsilateral radial pulse feeble as compression reduces the reversal blood flow through basilar artery. Bruit in supraclavicular and suboccipital area is often evident. Investigations: Duplex scan; neck and transcranial Doppler; CT carotid and vertebral angiogram; DSA (very is useful). Treatment: Drugs (aspirin, beta blockers, ACE inhibitors); Transluminal balloon angioplasty, Endarterectomyor Surgerrbypass graft (Common carotid-subclavian graft).

Left vertebral artery

Right - -subclavian artery

• Left subclavian artery

Arch of aorta

Fig . 1.339: Subclavian steal syndrome. (CCA: common carotid artery)

n

:::c )>

m

....::::0 rCl It)

::,

It) ..,

QI

ACUTE ARTERIAL OCCLUSION It is a condition of acute lack of tissue perfusion due to sudden cessation of circulation. Main axial artery of the limb is blocked presenting within minutes to hour after occlusion. It is common in lower limb, upper limb; but can occur in mesenteric, cerebral, coronary arteries.

I Causes

VI C

.., It) ..,

IQ

':

.., ,.. It) ..,

iii' 0 VI

It) QI VI

Embolism is the most common cause in developing country. Trauma. Thrombosis of an artery: Normal artery can develop sudden acute thrombosis in certain special situations with hypercoagulable status like malignancy, leukaemia, antiphosholipid antibody syndrome, protein C/protein S/antithrombin deficiency; polycythaemia rubra vera, thrombocytosis. It is commonly observed in external iliac artery, profunda femoris artery and popliteal artery. Thrombosis of a bypass graft is common cause in western countries which occurs at the site of anastomosis.

I Pathophysiology

Right vertebral artery

183

Distal ischaemia begins immediately after acute obstruction. Most sensitive peripheral nerves are first involved, and then muscles, subcutaneous tissue and skin are affected in order. Irreversible ischaemia occurs in 6 hours. Golden period is 1-6 hours. lschaemia may get aggravated by-propagation of thrombus below and above the block occluding the orifices of collaterals, fragmentation of embolus, associated thrombosis, acute compartment syndrome. Acute ischaemia causes endothelial injury of capillaries, arterioles and venules with luminal obliteration. Raised capillary permeability causes fluid leakage into extravascular space forming massive tissue oedema deep to deep fascia which by raising the intracompartmental pressure further reduces the perfusion leading into acute compartment syndrome.

I Features Pain which is continuous, severe, steady, bursting. Pallor of the distal part with extreme cold limb.

In early painful stage the ischemic foot (gangrene threatened) is nearly always pink, the skin being atrophied as though it were stretched tightly over underyling structure -Wilfrid G Oakley

It) VI

184

Pulselessness-sudden loss of earlier palpable pulse. Paraesthesia-sensory disturbances like tingling, numbness or complete loss of sensation. Paresis- damage to motor nerve and muscle leading into paralysis as a late grave feature. Poikilothermia-change in the temperature (cold). Pain, paraesthesia, paresis are due to ischaemia of peripheral nerves which are sensitive to hypoxia.

B Cardiac source (80%): - Due to mural thrombus following mitral stenosis and atrial fibrillation (50%); myocardial infarction (25%); others (5%) like prosthetic valves, endocarditis, intracardiac tumours (atrial myxoma) Noncardiac (10%): - Aneurysms (5%); atheromatous plaque in proximal artery, paradoxical (1%) Idiopathic is 10% Others (4%) like: - Cervical rib causing poststenotic dilatation of subclavian artery can cause emboli Cryptogenic---an unknown source (5%)-after investigationsj source is not found ---

TRAUMATIC ACUTE ARTERIAL OCCLUSION Causes:Thrombus due to trauma; Subintimal haematoma; Acute compartment syndrome; During femoral or brachia! arterial catheterisation for either diagnostic or therapeutic procedures. Features: History of trauma, pain, swelling at the site, pallor, pulselessness, cold limb. Investigation: Duplex scan, angiogram. Treatment: ,, Wound is explored and tear in the artery is identified. It is sutured using nonabsorbable monofilament material, polypropylene 6-0. Often venous or dacron graft is required for interposition. ,, Proper antibiotics and heparin are required to prevent thrombosis of the vessel. Later patient is advised to take oral warfarin for maintenance. ,, Compartment syndrome is common in anterior compartment of leg and in front of forearm. Here because of the closed compartment, pressure increases following fracture, haematoma which compresses over the vessel. It leads to blockade of vessel causing acute ischaemia of the limb presenting with severe pain, pallor, pulselessness. - Treatment: Immediate decompression by longitudinal fasciotomy, is the treatment of choice, wherein deep fascia is cut adequately to relieve the compression. Otherwise limb may go for severe ischaemia, gangrene and may land in amputation. , Associated fractures, haematoma, vessel tear has to be managed accordingly.

EMBOLISM ('Embolus' means in Greek-peg; first this term was used by Virchow in 1854) It is due to a solid, liquid or gaseous, material which is floating and travelling in the bloodstream, eventually blocking the vessel on its pathway. Arterial emboli. Venous emboliare due to DVT causing pulmonary embolism. Venous-arterial paradoxical emboli: Seen in intra-cardiac shunt (ASD) or intrapulmonary shunts (AV malformations) (Osler-Weber-Rendau syndrome). Fat embolism. Air embolism.

I

I

I Effects of Arterial Embolism

l ~

Brain: Blockage at middle cerebral artery causes hemiplegia, transient ischaemic attacks (TIA), visual disturbances Blockage at central retinal artery causes amaurosis fugax or permanent blindness Blockage at mesenteric vessels causes intestinal gangrene Blockage at renal artery leads to haematuria, loin pain Blockage at limb vessels causes pain, pallor, pulseless, paraes~~esi_a, paresis, ulceration, gangrene common site of arterial emboli is common femoral artery

I

I Sites of Lodging of Emboli The most common site is lower limbs (75%). 10% brain; 10% upper limb; 5% superior mesenteric and renal arteries. In the lower limb the most common site is at the bifurcation of common femoral artery (40%); popliteal artery (15%); common iliac artery (12%); aortic bifurcation (10%).

I Features of Embolism Earlier history of claudication is absent but history suggestive of disease for source of emboli will be present. Sudden, dramatic, rapid development of pain with numbness. Limb becomes rapidly cold and mottled with blebs. Loss of sensation and movements. Absence of distal pulses but forcible, expansile, prominent proximal pulse. For example-prominent femoral artery pulsation with embolic bock at popliteal level. Toxic features. Collapsed veins, cold limb distal to the level of block, oedema and presence of blebs distally. Muscle which is soft normally while palpating will feel doughy initially but later becomes stiff. Once stiffness of muscle is found embolectomy benefit is bleak.

T

Differences between embolism and thrombosis

Fsatures

Embolism

Thrombosis

Source Pulse

Present Proximal and contralateral pulses normal Severely cold Sharp cut off sign Very few-not well-developed

Not present lpsilateral and opposite side pulses may be absent

Temperature Angiography Collaterals

Cold or normal Diffuse tapered disease Well-developed

I Investigations for Arterial Embolism Emergency Doppler angiogram, ECG and echocardiography. Angiogram is gold standard in all acute limb ischaemia. It differentiates between embolism and thrombosis; status of vessel proximally and distally. Angiography should ideally be done from contralateral limb or through left brachia!.

Fig. 1.340: Right leg showing features of acute embolism.

Relevant tests for origin of emboli. Prothrombin time, APTT, BT, CT, platelet count should be done. Note:

Once embolism occurs irreversible changes occur distally in 6 hours, so ideal period for intervention is within 6 hours.

B

CLASSIFICATION OF SEVERITY OF ACUTE LIMB ISCHAEMIA

Class I: Viable-no pain; no neurological deficit; Doppler shows audible signal. Venous flow present. Class Ila. Marginally threatened-no pain; numbness/paraesthesia; no audible Doppler signal. Venous flow present. Class /lb: Immediately threatened- persistent pain; sensory and motor loss; no Doppler signal. Venous flow present. Class Ill: Irreversible-paralysis and anaesthesia. No venous flow. lschaemia up to class llb with normal venous flow is called as Early; ischaemia which is class Ill, with muscle rigor, marbled skin and without any venous flow is Late. This late ischaemia is more likely to land with amputations even though revascularisation can be ~

I Treatment Treatment of Embolism and Thrombosis of Acute Limb lschaemia Immediate infusion of 5000-10,000 units of IV heparin and relief of pain are needed first.

Surgical

Embolectomy (surgical exploration and removal of clot) is the choice for embolus. It is done either by interventional balloon 5 French (Fogarty, 1963) embolectomy or open method. It is the standard treatment for arterial embolism. It can be repeated several times until adequate bleeding occurs. For acute thrombosis causing acute limb ischaemia, open thrombectomy with or without bypass may be the surgical treatment; but it is not the standard treatment fo r acute thrombosis (Standard is thrombolysis, Dotter and co, 1974).

185

("\

:::c J> ""O -l

m

:::0

.....

r-

Endovasculartherapy

lntrarterial thrombolysis using urokinase. Percutaneous mechanical thrombectomy- it is done either by suctioning clot via catheter or dissolution of thrombus by pulverisation and aspiration by high speed motors or fluid jets. Ultrasound accelerated thrombolysis using catheter based or transdermal using acoustic cavitation to ablate thrombus.

Embolectomy ,. It is done as early as possible as an emergency operation . ,. Under fluoroscopic guidance, Fogarty catheter (interventional radiology) is passed beyond the embolus and balloon is inflated. Catheter is withdrawn out gently with embolus. Procedure has to be repeated until embolectomy is completed and good back bleeding occurs. Angiogram is repeated to confirm the free flow. ,. Postoperatively initially heparin and later oral anticoagulants are used. Procedure is done under general anaesthesi a or local anaesthesia. ,. Open arteriotomy and embolectomy can be done by direct approach and later the arteriotomy has to be sutured. Postoperatively anticoagulants and antibiotics are given. Intra-arterial thrombolysis using fibrinolysins After passing arterial catheter, angiogram is done and agents are injected intra-arterially through the arterial catheter. Drugs used are: ,. Streptokinase (Here lysis occurs in 48 hours): Dose is 2.5 lac IV over 30 minutes; or intra-arterialy 20,000 units/ hour followed by one lac unit in 24 hours. ,. Urokinase: It is commonly used for thrombolysis. It converts plasminogen to plasmin which breaks fibrin clots. Initial bolus of 2,50,000 IU is given followed by an infusion of 4,000 IU/min for 4 hours, later continuous infu sion of 2,000 IU/min to complete the lysis. Even though controversial, it is of usual practice to infuse 1000 IU/hour of heparin to prevent new thrombus formation. Check angiography should be done during therapy. Multiholed catheter (5 French) is used for infusion. , Tissue plasminogen activator (TPA): Altep lase , Reteplase-here lysis occurs in 24 hours. TPA is better

Unless you try to do something beyond what you have already mastered, you will never grow.

0

iii' (I) QI

"' (I)

"'

186 ,

and ideat, but it is costly. It has very less side effects. TPA dose is 50 mg over 2 hours IV. TPA pulse-spray method. Here lysis occurs in 6 hours. CONTRAINDICATIONS FOR THROMBOLYSIS

Recent stroke, Recent eye surgery, Pregnancy Recent major surgery or major bleed like of varices I History suggestive of or confirmed active duodenal/gastric ulcers L!_ Uncontrolled hypertension or coagulation disorders

T

Advantages and disadvantages of thrombolysis

Advantages

Disadvantages

• Gentle angiographic clot • Useful only for class I and removal Ila acute ischaemia • Survival and limb salvage is • Bleeding at the site and equal elsewhere is possible • It is mainly useful for acute • 25% rate of failure thrombus • For embolus it is used often as an adjunct along with embolectomy • It avoids surgery

I Anticoagulant Therapy It is to prevent recurrent emboli formation. Immediate infusion of heparin 5,000 units intravenously is helpful to prevent further extension of thrombus. Later oral anticoagulants may be added.

Complications of Revascularisation in Acutely lschaemic Limb Reperfusion injury 'No ref/ow' phenomenon: It is due to severe capillary oedema causing poor peripheral tissue hypoperfusion in spite of major vessel revascularisation. Acute compartment syndrome can occur due to massive ischaemic oedema especially of skeletal muscles deep to deep fascia which compress on venu les exceeding tissue interstitial pressure causing further compromise in tissue perfusion. Other complications are-sepsis, reblock, bleeding and catheter-related complications.

Severe ischaemia causes oedema in the muscular compartment with raise in compartment pressure more than the essential capillary perfusion pressure causing acute compartment syndrome. It is com mon in the anterior compartment of the leg. It is basically in the skeletal muscles deep to deep fascia. Compartment pressure when measured using transducer needles will be more than 40 mm Hg or >30 mmHg for 3 hours or above the mean arterial pressure. Muscle weakness, sensory changes, leg pain which is aggravated by dorsiflexion of toes. 'No re-flow' phenomenon due to tissue oedema causes capillary perfusion block. Even though compartment syndrome and 'no reflow' phenomemenon are separate entities they are always seen together along with reperfusion injury. Metabolic acidosis, acute tubular necrosis causing acute renal failure and cardiac arrhythmias may set in and become life-threatening. Features are-toxaemia; oliguria; persistent pain and oedema in the leg with muscular tenderness; raised blood urea and serum creatinine with features of acute ischaemia in the limb. Raised creatinine level (renal failure}, creatine kinase (muscle lysis) are typical. Treatment: ,.. Mannitol to prevent renal failure; fluid therapy. ,, Fasciotomy to reduce raised compartment pressure. All four compartments of lower limb should be decompressed surgically. Long vertical lateral deep fasciotomy incision in the calf behind the fibula along the deep fascia and its fibular attachments is a must. Bleeding is common after fasciotomy as patient is heparinised. Infection of the wound can occur. Later, once the patient is stabilised and oedema subsides with healthy wound , secondary suturing or skin grafting is done. If after fasciotomy, patient survives then it is with eventual development of Volkmann's ischaemic contracture. ,. Antibiotics and supportive therapy.

SADDLE EMBOLUS It is an embolus blocking at bifurcation of aorta. Causes: Mural thrombus after myocardial infarction; Mitra! stenosis with atrial fibrillation; Aortic aneurysm.

REPERFUSION INJURY It occurs after reestablishment of arterial flow to an ischemic tissue bed which further leads to tissue death causing specifically peripheral muscle infarction. It is due to sudden release of oxygen free radicals which blocks the microcirculation, with release of high levels of potassium (hyperkalaemia) and myoglobin (myoglobinaemia and myoglobinuria}. Haemodynamically patient becomes unstable with lactic acidosis, intracellular changes, interstitial oedema and cardiac dysfunction. It is often life-threatening. •·· Haimovici triad of revascularisation injury (1960)-(1) Muscle infarction; (2) Myoglobinuria; (3) Acute renal failure.

Saddle embolus

Fig. 1.341 : Saddle em bolus blocking the bifurcation of abdominal aorta.

It causes severe, rapid, dramatic symptoms. (EIA: external iliac artery; CIA: common iliac artery; IIA: internal iliac artery)

The embolus which blocks at aortic bifurcation is usually large. Features: ,, Features of sudden , rapidly progressive ischaemia in both lower limbs. ,, Gangrene of both lower limbs. ,, Features of associated infection. Investigations: Arterial Doppler, aortic ang iogram; Ultrasound abdomen. Treatment ,, Initially, heparin is injected intravenously-10,000 units and later 5,000 units subcutaneously 8th hourly. ,, Embolectomy can be done using Fogarty's catheter. ,, Open arteriotomy and embolectomy can also be tried. ,, Antibiotic prophylaxis is given to prevent infection. Note: In aortic bifurcation thrombus, there is earlier history of claudication in the buttock often with Leriche's syndrome. Symptoms are slow and gradual but not dramatic. Collaterals between aorta and iliac arteries have well-formed and so sudden, rapid development of gangrene will not occur.

EMBOLECTOMY Indications ,, Acute embolic blockade of artery commonly seen in common femoral, cranial vessels, mesenteric vessels. ,, It should be done within 6 hours as after 6 hours irreversible changes occur- Golden hour.

Fogarty f--- tube/catheter _ _,.__.

Balloon passed through

Balloon inflated

-

Fig . 1.342: Embolectomy technique.

Fig. 1.343A:

n

:::c )>

"'O

-I

m

...r-

:,:,

Fig. 1.343B: Figs. 1.343A and B: Fogarty's catheter. It is 80 cm in length with 4 to 7 French size. It is used for embolectomy. Note the inflated balloon at the tip.

Cl

lb

:::,

.... QI

lb

V'I

,, It is usually done under local anaesthesia under C-arm guidance with anaesthetist monitoring the patient. It can be done under spinal or general anaesthesia. Methods: ,, Jnterventional method is usually employed using Fogarty's catheter. Good back bleed signifies completeness of embolectomy. ,, Open arteriotomy method is done directly over the artery followed by suturing the artery. Complications: Bleeding; sepsis; thrombosis; narrowing; incomplete removal. After embolectomy: Patient is placed in ICU care; Monitoring with-PTT, thromboplastin time. Note: • lntraoperative arteriogram is a must to confirm the adequacy of blood flow and completion. • lntraoperative thrombolysis as an adjunct to save the limb using urokinase 2,50,000 IU for minutes into distal artery may be beneficial. • Prophylactic tasciotomy is needed in delayed cases to prevent reperfusion injury. • Postoperative systemic heparin and later oral anticoagulant is given. • Treatment for atrial fibrillation, atherosclerotic stenosis and other causes is needed.

Clot (embolus)

l~

187

FAT EMBOLISM (Ernst Von Bergmann, in 1873) 90% of major trauma especially with fractures develop fat embolism from aggregation of fat globules and chylomicrons derived from bone marrow. Fat globules release fatty acids which act as toxins. It is common in fracture long bones, and multiple fractures. It is observed after intramedullary nailing, liposuction, joint reconstruction , parenteral lipid infusion, cardiopulmonary bypass, and pathological fractures. Only 5- 10% will develop tat embolism syndrome (FES). FES shows respiratory distress (ARDS), and skin manifestations. Approximately 20- 30% of the population have a patent foramen ovale; fat emboli pass through the pulmonary circulation causing the systemic manifestations of FES, particularly involving the brain and kidneys. As a result of the occluded cerebral vasculature, patients exhibit encephalopathy, localised cerebral edema. FES has got 20% mortality.

Food is the unavoidable necessity of existence.

C: .... lb ....

10

'


.... .... lb

....

iii'

9.

"'lbQI "'lb "'

188

...>.

...:::,Cl ....0 QI

V')

ro

:::, C:

ro

_.,, a::i 0::

V')

I Features Pulmonary: Cyanosis, tachypnoea, right heart failure, froth in mouth and nostrils, fat droplets in sputum, eventually respiratory failure. Cutaneous: Petechial haemorrhages in the skin. Cerebral: Drowsy, restlessness, disoriented, constricted pupils, pyrexia and coma. Retinal artery emboli is the earliest sign to appear, causing striae haemorrhages, fluffy exudates confirmed on fundoscopic examination. Kidney: Blockage in renal arterioles results in fat droplets in urine.

T

Gurd and Wilson criteria for FES

Major criteria

Minor criteria

• Symptoms and radiologic evidence of respiratory insufficiency • Cerebral sequelae unrelated to head injury or other conditions • Petechial rash-over upper body, axillae

• Tachycardia (heart rate >1 10/min) • Pyrexia (>38.5°C) • Retinal changes or petechiae • Renal dysfunction • Jaundice • Acute drop in hemoglobin level • Sudden thrombocytopaenia • Elevated erythrocyte sedimentation rate • Fat microglobulinaemia

Differential diagnosis: Pulmonary embolism; Thrombotic thrombocy1openic purpura. Investigations: ,, Presence of fat lobules in the blood obtained by pulmonary capillary wedging is diagnostic. :.- Haemoglobin estimation, platelet count and total WBC count. ,. Chest X-ray shows snow storm appearance. ,, CT chest is useful; CT head is done to rule out causes of intracranial injuries. ,. Transesophageal echocardiography (TEE) may be of use in evaluating the intraoperative release of marrow contents into the bloodstream during intramedullary reaming and nailing. ,. Bronchoalveolar lavage with staining for fat will show lipid inclusions. Treatment: , Adequate oxygenation with ventilator support (ICU care). ,. Hydration, nutrition, achieving haemodynamic stability, prevention of DVT, avoiding volume overload with proper fluid therapy. , Methylprednisolone may be useful; but use of heparin, low molecular dextran and other steroids are controversial even though commonly used. , Albumin transfusion may be helpful as it binds with fatty acids to reduce the lung injury.

,. Early fixation of the fractures; placement of IVG filters will prevent the chances of fat embolism or emboli reaching into the lungs.

AIR/GAS EMBOLISM

I Causes

Through venous access like IV cannula, most common cause. During artificial pneumothorax. During surgeries of neck and axilla. Traumatic opening of major veins sucking air inside, causing embolism. During fallopian tube insufflation; laparoscopic surgeries. During illegal abortion.

I Features It causes respiratory distress, haemoptysis, convulsions, unconsciousness, visual and hearing disturbances, fatigue and numbness, paralysis, haemodynamic instability and coma. Amount of air required causing venous air/gas embolism is 15 ml. To precipitate symptoms in venous embolism 100 ml of air required. 100 ml ofair at a rate more than 100 mUsecond is fatal. When the air enters the right atrium, it gets churned up form ing foam which enters the right ventricle and blocks the pulmonary artery. Mill-Wheel murmur (machinery) heard over the precordium through a stethoscope is diagnostic. During open heart surgery/therapeutic pneumothorax, by accidental pulmonary vein puncture or in atrial septa! defect (ASD) air may enter left side of the heart (paradoxical air embolism) causing coronary block or cerebral air embolism. Arterial gas embolism is more dangerous and often early fatal. 2 ml of gas/air is fatal in cerebral circulation; and 0.5 ml is fatal in coronary arteries. Through paravertebral veins also air embolism to brain can occur.

I Treatment Patient is placed in Trendelenburg left lateral decubitus position. The Trendelenburg position keeps left ventricular air bubble away from the coronary artery ostia (which are near the aortic valve) so that air bubbles do not enter and occlude the coronary arteries. Left lateral decubitus positioning helps to trap air in the non-dependent segment of the right ventricle (where it is more likely to remain instead of progressing into the pulmonary artery and occluding it). The left lateral decubitus position also prevents the air from passing through a potentially patent foramen ovale (present in as many as 30% of adults) and entering the left ventricle , from which it could then embolise to distal arteries. Hyperbaric oxygen is useful in both venous and arterial gas/air embolism as it reduces the ischaemia, reduces the bubble size; in arterial gas embolism it removes the nitrogen from the bubble so that to improve perfusion and oxygenation.

By passing a needle, the air has to be aspirated from the right ventricle. Often requires life-saving open thoracotomy to aspirate the excess air causing the block.

THERAPEUTIC EMBOLISATION

I Indications

Haemangiomas; AV fistulas. Malignancies like renal cell carcinoma, hepatoma. Cerebrovascular problems. To arrest haemorrhage from GIT, urinary and respiratory tract. ,. In bleeding duodenal ulcer or gastric ulcer, embolisation is done to occlude gastroduodenal artery or left gastric artery respectively. ,. It is also useful in bleeding oesophageal varices, secondaries in liver (mainly due to carcinoids), hepatoma.

Blood clot Gel foam Balloons Quick setting plastics Stainless steel coils

Human dura Plastic microspheres Ethyl alcohol Wool

False aneurysm contains single layer of fibrous tissue as wall of the sac and it usually occurs after trauma.

B Fusiform-vniform dilatation of entire circumference of arterial wall Saccular-iJilatation of part of circumference of the arterial wall Dissecting-through a tear in the inlima blood dissects between inner and outer part of tunica media of the artery ____J

"'ti

-I

m

....r-

:::0

l

True aneurysm

False aneurysm

Fig. 1.344: True and false aneurysms. In true type, all layers are intact.

CAISSON'S DISEASE OR DECOMPRESSION DISEASE (BEND'S DISEASE)

Fusifo rm type

Saccular type

Fig. 1.345: Fusiform and saccular types of aneurysms.

ANEURYSM There is no disease more conducive to clinical humility than aneurysm - William Osler, Circa 1900 of the aorta.

It is an abnormal permanent dilatation of localised segment of arterial system. Diameter will be 50% more than expected normal diameter of that artery in aneurysm. Atherosclerosis which is the most common (90%) facilitating cause of aneurysm is due to destruction and loss of stability of tunica media. •·· True aneurysm contains all three layers of artery.

n

:c )>

Soft tissue

In false type all layers breached with haematoma having a false capsule.

It occurs due to rapid decompression from high altitude, aircraft, compressed air chambers, deep sea divers causing bubbling of nitrogen which blocks the small vessels. It is classified as Typel/(Simple) involving musculoskeletal system, skin, lymphatics and Type II (Serious) involving also major organs. Commonest part involved is musculoskeletal system (90%) mainly major joints like elbow, shoulder, hip, wrist, knee and ankle. In joints and muscles it causes excruciating pain (bends). Spinal cord ischaemia causing neurological deficits. Lungs may be affected causing choking with chest pain, tightness and dry cough . Treatment: ,. Oxygen therapy; Recompression and gradual decompression in special chamber.

189

Fig. 1.346: Thoracic aortic aneurysm.

When you cease to dream, you cease to live.

9. "'

(I)

Ill

"' "' (I)

Causes , Acquired: - Degenerative: Atherosclerosis (most common cause- 90%); mucoid degeneration of intima and media (in South African young Negroes). - Traumatic-. Direct; indirect like in post-stenotic dilatation by cervical rib; traumatic AV aneurysmal sac; aneurysm due to irradiation (due to dryness and destruction of vasa vasorum causing weakening). - Infective: Syphilis; mycotic; tuberculosis (in lung); arteritis; acute sepsis. Collagen diseases like Marfan's syndrome, polyarteritis nodosa, Ehler-Danos syndrome. ,. Congenital: - Berry aneurysm; cirsoidaneurysm; congenital AV fistula. Sites: Aorta; Femoral; Popliteal; Subclavian; Cerebral, mesenteric, renal, splenic arteries. The most common is true, fusiform, atherosclerotic, aortic aneurysms. Berry aneurysms are multiple aneurysms occurring in ci rcle of Willis.

Distal oedema due to venous compression. Altered sensation due to compression of nerves. Erosion into bones, joints, trachea or oesophagus. Aneurysm with thrombosis can throw an embolus causing gangrene of toes, digits, extending often proximally also.

B Thrombosis and distal ischaemia Release of emboli causing acute arterial occlusion Pressure effects on bone (erosion); skin; veins (oedema); nerves (pain, paraesthesia); stomach (erosion-haematemesis); oesophagus (dysphagia) Rupture; Infection of aneurysm

Fig . 1.347: Chest X-ray showing aortic aneurysm.

I Differential Diagnosis

I Clinical Features of Aneurysms Swelling at the site which is pulsatile (expansile) , smooth, soft, warm , compressible, with thrill on palpation and bruit on auscultation. Swelling reduces in size when pressed proximally.

Pyogenic abscess: Abscess has to be always confirmed by aspiration; especially in axilla, popliteal region, groin. Vascular tumours. Pulsating tumours: Sarcomas, pulsating secondaries. Pseudocyst of pancreas mimics aortic aneurysm. AV fistula.

m

,.:., Matas aneurysmorrhaphy for saccular aneurysm

Ligation and exclusion using autologous vein bypass graft

Excision and graft in fusiform aneurysm (Dacron graft)

Excision of aneurysm and bypass using autologous vein graft

Figs. 1.348A to C: Different methods of aneurysm repair. (A) Matas aneurysmorrhaphy for saccular aneurysm; (B) Excision and Dacron grafting; (C) Ligation and exclusion of the aneurysm using autologous vein graft and excision of aneurysm and bypass using autologous vein graft.

I Investigations Doppler study, duplex scan, CT angiogram, DSA. Tests relevant for the cause, like blood sugar, lipid profile, echocardiography.

Fig. 1.349: Right renal artery aneurysm on CT angiogram.

Fig. 1.350: Cerebral CT angiogram showing (intracranial)

Berry's aneurysm.

I Treatment Reconstruction of artery using arterial grafts. Arterial endoaneurysmorrhaphy-MATAS. It is done usually for peripheral saccular aneurysm. Matas aneurysmorrhaphy may be restorative or endo-obliterative or reconstructive. Therapeutic embolisation. Clipping the vessel under guidance (e.g. cranial aneurysms). Older methods which are now not used but popular earlier were-wiring of the aneurysmal sac/wrapping of the aneurysmal sac/ligatures at different levels (ligation just proximal to aneurysmal sac-Anet's; ligation proximally proximal to an arterial branch-Hunter's; ligation just distal to aneurysmal sac-Brasdor's; ligation distally distal to an arterial

branch-Wardrop's; ligature one proximal and another distal to aneurysmal sac-Antylus).

MYCOTICANEURYSM (INFECTIVE ANEURYSM) (3%) It is a misnomer. It is mushroom shaped aneurysm, hence the name (William Osler, 1885). It is not due to fungus but due to bacterial infection of the arterial wall. It is actually an endovascular infective vasculitis. Common bacteria are grampositive organisms like Staphylococcus aureus (most common) and Streptococcus. Salmonella infection also can cause infective aortic aneurysm. Infective aortic aneurysm is common in Taiwan. Common aetiology is bacterial endocarditis but could be any infective site. Cholecystitis, urinary infection, osteomyelitis, diverticulitis and pneumonia also occasionally cause infective aneurysm. ,:. It is seen in diseased atherosclerotic artery (like aorta) or traumatized disrupted inti ma of the artery (femoral). Common vessels involved are aorta, visceral, head and neck and intracran ial. Commonly it is saccular, multilobed, with a narrow neck. Patient presents with fever, back pain (if aorta), toxaemia and tender pulsatile mass if it is in the periphery. Torrential haemorrhage and sepsis can occur as complication. It has got 50% mortality. Investigations: Leucocytosis and raised ESR; positive blood culture (70% cases), positive culture from aneurysmal sac, MR or CT angiogram are relevant. Treatment: Broad-spectrum antibiotics (often for long term, >6 weeks); resection of the aneurysm; debridement and drainage of the infected aneurysm with adequate blood transfusions; extra-anatomic bypass through uninfected tissue planes to avoid contamination of the graft. Endovascular aneurysm repair (£VAR) often with covered endovascular reconstruction of aortic bifurcation ( CERAB) is becoming more commonly used nowadays as less invasive effective therapy. Nole: Microbial arteritis with aneurysm is adifferent entity is due to bacteraemia occurring in an atherosclerotic vessel due to Salmonella infection.

ABDOMINAL ANEURYSM Abdominal aortic aneurysm is the most common aortic aneurysm. Splenic artery aneurysm is the 2nd most common type. Incidence is 2%. It is more common in males. Transverse diameter of aorta in an aneurysm should be 3 cm or more. Common in elderly; common in males (4:1); chance of getting aneurysm in genetically related first degree relatives is 10 times more. Common in smokers (8:1 with nonsmokers); in 55% of patients Chlamydia pneumoniae is identified.

Today's impossibilities are tomorrow's miracle.

191

(')

I

)> -0

..... m

:::c .... ,... G\ rt)

:::,

... rt)

ll)

VI

C:

13 rt)

... ...,.. ...iii'

':':

)>

rt)

0

"' rt) ll) "' rt)

"'

192

...>...:::,Cl

QI

V'I

0 n, :::, C:

n,

_.,,

co Q: V'I

ABDOMINAL AORTIC ANEURYSM (AAA) AAA is a localised, progressive and permanent dilatation usually larger than 3 cm in diameter of the abdominal aortic wall.

I Causes Atherosclerosis (as degenerative process) is the most common facilitating cause (95%)-aortic wall contains smooth muscle cell matrix, elastin, collagen; elastin (in tunica media) is the main load bearing part with collagen (in adventitia) as safe net in the wall to provide tensile strength preventing aneurysm formation. Elastin in medial layer of aorta is degraded and reduced significantly in infrarenal aorta in relation to collagen, absence or less vasa vasorum in infrarenal aorta and atherosclerotic unstability of the medial wall of aorta cause infrarenal aorta more prone to develop aneurysm. Increased proteolytic activity of aortic medial wall due to increased matrix metalloproteinases (MMP) (derived from aortic smooth muscle cells and macrophages) cause elastin and collagen degradation and increase in diameter of aneurysm. Collagen degradation in adventitia causes rupture. Familial aortic aneurysm (associated with 25% of AAA) is more prevalent in females to reduce male-to-female ratio to 2:1. It is related to decrease in type Ill collagen, a1 antitrypsin and lysyl oxidase. Marfan's, Ehler Dan los syndromes are related genetically. Others: Syphilis, dissection, trauma, collagen diseases, infection, arteritis, cystic medial necrosis, association with Chlamydia pneumoniae (55%). Classification I

- lnfrarenal-most common (95%). - Suprarenal- 5%. Isolated suprarenal type is rare; it is usually associated with thoracic and or infrarenal types. Classification II

- Asymptomatic. - Symptomatic. - Symptomatic ruptured.

Asymptomatic Type It is found incidentally either on clinical examination or on angiography or on ultrasound. Repair is required if diameter is over 5.5 cm on ultrasound. It is identified during routine abdominal palpation or while assessing or operating for some other abdominal conditions.

Symptomatic without Rupture

(Clinical features/presentations) It presents as back pain, abdominal pain, mass abdomen which is smooth, soft, nonmobile, not moving with respiration, vertically placed above the umbilical level, pulsatile both in supineas well as knee-elbow position with same intensity, resonant on percussion. Common in males (4: 1); common in smokers. ,:. GIT, urinary, venous symptoms can also occur. Hypertension, diabetes, cardiac problems should be looked for and dealt with.

In infrarenal type upper border is clearly felt. Lower limb ischaemia and embolic episodes can occur. Being a retro peritoneal mass back pain is common-may be due to retroperitoneal stretching, nerve irritation or vertebral erosion . 5% present as inflammatory aneurysm adherent to ureters, left renal vein, inferior vena cava and duodenum. Expanding aneurysm blocks lymphatics causing inflammation and fibrosis; or it may be due to infection and fibrosis of earlier localised ruptured abdominal aortic aneurysm. Such chronically inflamed aneurysm will not rupture further; but it is always symptomatic with fever and severe pain in abdomen and back. It needs surgical repair through retroperitoneal approach. Aortocaval fistula, presents as high output cardiac failure with continuous bruit in abdomen and severe lower limb ischaemia (steal phenomenon). Aortoenteric fistula is due to erosion of aneurysm into 4th part of duodenum presenting as gastrointestinal (G I) bleed, malaena, shock. It is treated by duodenal closure, aortic ligation, aneurysmal exclusion with extra-anatomic bypass graft with gastrojejunostomy. Contamination is the major threat here. Aneurysm in a patient with horseshoe kid ney wh ich is anterior to aorta is difficult to manage. Left retroperitoneal approach is needed. Endovascular aneurysm repair (EVAR) is not possible.

I Investigations Blood urea, serum creatinine. US (most widely used noninvasive test; but neck of the aneurysm, dimensions and relation to renal arteries are difficult to assess), aortogram, DSA, CT scan (most precise). US is an effective screening tool. Screening is done in cardiovascular patients in men (60-85 years), in women (60-85 years); men and women above 50 years with family history; annually in asymptomatic AAA with 4.0-4.5 cm size, with size >4.5 cm once in every 6 months. CT angiogram, MR angiogram. Blood sugar, lipid profile, other relevant investigations like ECG, echocardiography, cardiac and pulmonary assessment. Note:

X-ray will show eggshell calcification. CT scan is morereliableand precise investigationof choice-gives better information regarding extent on sides/ neck, size, dimensions, sizeand site of the thrombus, calcification, relation of renal arteries, inflammation and fibrosis and adjacent tissues. MRI may be better only in renal failure patients.

B Rupture, infection Thrombosis, embolism, distal ischaemia/gangrene Aortocaval fistula, aortoenteric fistula formation Erosion of vertebra, spinal cord ischaemia when thrombosis develops

impairment and noninflammatory aneurysm); if aneurysm size is

"'O

m

:,:,

External iliac artery

Fig. 1.354: lnfrarenal aortic aneurysm repair. It is the most common site of aortic aneurysm. Adventitia of aorta is opened; aneurysm is excised;

graft is sutured above and below; adventitia is wrapped around.

Superstiff guidewire Catheter

lpsilateral attached branch

9. "' 11) QI "' 11)

Contralateral separate modulus branch

• lpsilateral aortic and iliac single unit modulus is passed through same side CFA. • Opposite side (contralateral side) branch CIA graft is deployed through superstiff guidewire. • Through opposite C FA.

"'

• Completion of procedure with graft in place. Arteriotomy is closed and skin incision is closed.

Figs. 1.355A and B: Endovascular aneurysm repair (EVAR). Aortic and one side iliac stent is used as one unit modulus which is passed

through same side common femoral artery; opposite side iliac part is inserted as separate modulus through opposite CFA.

REMEMBER

COMPLICATIONS OF SURGERY M/is the most common cardiac complication in perioperativeand in first 2 days of postoperative period Haemorrhage and haemodynamic complications • Renal failure-is most common noncardiac complication Colonic ischaemia-10% due to poor IMAcirculation • Sexual dysfunction Aortoduodenal fistula; Aortovenacaval fistula Spinal cord ischaemia- paraplegia Paralytic ileus Distal thromboembolism- blue toe syndrome DVT, limb ischaemia Graft leak, graft thrombosis, graft failure Anastomotic disruption, pseudoaneurysm formation Prosthetic infection/migration

Pulsation of an aneurysm is expansile. Pulsation may be absent if it is thrombosed Abdominal aneurysm of any size which is painful or tender should be operated Abdominal aortic aneurysm of any size causing embolus should be operated Abdominal aortic aneurysm more than 5.5 cm should be operated In ruptured abdominal aortic aneurysm emergency surgery is the only choice operation with rapid resuscitation; immediate opening and repair using graft. Systolic pressure in this patient should be just adequate to maintain the cardiac function but should not be more than 1OD mm Hg as it will cause more bleeding Anterior rupture is more dangerous than posterior rupture Endoluminal stenting is becoming popular

Move to the rhythm of soul and you'll never miss a beat.

196

PERIPHERAL ANEURYSM Peripheral aneurysms are less common compared to aneurysms in the cavity. Such surface aneurysms are easily visible and better amenable for clinical examination. But same time it may be mistaken for abscess and inadvertent wrong attempt of incision and drainage can occur leading to disastrous consequences. Popliteal type is the most common one. Peripheral aneurysms occur in descending order offrequency in popliteal, femoral, subclavian, axillary and carotid arteries. Expansile pulsation which is confirmed using two finger placement with thrill and bruit is typical. Infection, thrombosis make it less pulsatile mimicki ng an abscess. Erosion into adjacent bone and skin, rupture are known to occur. Distal emboli may lead into digital gangrene. Pressure on the affected artery proximally reduces the size, and eliminates the thrill/bruit; pressure distal to aneurysm increases the prominence of the aneurysm swelling with bounding pulsation. False aneurysm is common in femoral artery. It is treated with thrombin injection if size is 3 cm surgical repair of artery with or without graft is indicated. True femoral aneurysms are uncommon; when it occurs complications are also rare (2 cm; symptomatic aneurysm; aneurysm which has caused emboli. Surgeries are-exclusion with bypass inlay Dacron graft through medial approach; aneurysmectomy and graft if there is neurovascular compression. If there is thromboses aneurysm, intra-arterial thrombolysis is needed using Fogarty catheter but amputation rates are very high (>50%).

CAROTID ARTERY ANEURYSM (EXTRACRANIAL) Incidence is less than 4% of peripheral aneurysms. Most common site: Common carotid artery bulb, often extends into the internal carotid artery.

Fig . 1.356: Femoral artery aneurysm with impending ruptureneeds emergency surgical intervention. It is rare type.

Fig. 1.359: Carotid aneurysm. Fig. 1.357: Radial artery aneurysm.

Causes: Atherosclerosis, trauma ; Syphilis, Marfan 's syndrome; Ehler-Danlos syndrome; Congenital.

B

-

CLINICAL FEATURES

10% bilateral Swelling in the neck at the level of the thyroid cartilage, below the angle of mandible, Pulsatile (expansile pulsation), Smooth, soft, nontender, horizontally mobile, with bruit Neurological features due to embolic episodes (50%) Hoarseness of voice, Homer's syndrome Dysphagia due to swelling extending into the tonsillar bed.

Differential diagnosis: Carotid body tumour; Neurofibroma arising from the vagus; Abscess in neck.

I.Classification (DeBakey's) _ _ I Type I: Dissection begins in ascending aorta extends into descending thoracic aorta (70%) Type JI: Dissection originates in ascending aorta and extends only up to the origin of the major vessels. It is safer type with less complications Type Ill: Dissection begins in the descending thoracic aorta beyond the origin of the left subclavian artery II. Stanford classification Proximal- includes DeBakey's Type I and II Distal-includes DeBakey's Type Ill Ill. Dissecting aneurysm can be: Acute Chronic Healed dissecting aneurysm which communicates distally again to aorta as double barrelled aorta

Figs. 1.360A and B: Basilar artery aneurysm- angiogram (Courtesty: Dr Muralidhar Pai, MCh, Mangaluru).

Complications: Rupture; Thrombosis; Hemiplegia. Investigations: Doppler of neck, carotid angiogram; DSA, CT angiogram. Treatment: ,, Reconstruction of the artery using vascular graft. ,. Ligation of the bulb as a life-saving procedure, but results in haemiplegia. ,, lntravascular stents.

Typvpe Type A

DISSECTING ANEURYSM

II

Type Ill

T"''•V'•I

Type B (Stanford)

Fig. 1.361 : Dissecting aneurysm.

It is a misnomer. It is not an aneurysm, only an aortic dissection. It is the dissection of media of the aorta after splitting through inti ma creating a channel in the media of the vessel wall. Causes: Hypertension (It is associated in 80% of dissecting aneurysms); Cystic medial necrosis; Marian's syndrome and collagen diseases; Trauma; Weakening of the elastic layers of the media due to shear forces. Features: ,, It is always seen in thoracic aorta, common in ascending aorta (70%). ,. It is uncommon in other parts of aorta or other vessels. ,. It can occur in aortic arch or thoracic descending aorta. ,. This dissected aortic channel gets lined by endothelium, often reopens distally into the aorta causing doublebarrelled aorta which, in fact, prevents complications. ,. It is commonly associated with aortic insufficiency. Atherosclerosis is not a usual cause for dissecting aneurysm.

B Acute: Rupture into the pericardium or pleura-dangerous type Chronic: Blockage of coronary vessels and major vessels like carotid and subclavian arteries with aortic insufficiency

,. Pain in the chest, back which is excruciating. ,, Features of ischaemia dueto blockage of different vessels. Investigations: Chest X-ray shows mediastinal widening; Arterial Doppler; CT angiogram. Treatment: , Antihypertensives. ,. Surgery: Using Dacron graft reconstruction of aorta has to be done with cardiopulmonary bypass.

B ~

Progressive disease ignificant ischaemia

Imagination is the highest kite one can fly.

n

:::c

)>

,:, -i

m ::0 _.

,...

198

ERYTHROMELALGIA (ERYTHRALGIA) It is also called as Mitchell's disease; an episodic attack precipitated by heat, exertion and stress; common in lower limbs. Primary type is familial and autosomal dominant. Secondary type is seen in gout, erythrocyanosis frigida, polycythaemia, viral infection, mushroom poisoning, drug induced (verapamil , ergots, fluoroquinolones) . There is microvascular and neuropathic changes. There is severe burning pain in the limbs with redness and sensation of heat. Warmness in the skin; often excoriation or ulceration, flu shing, prominent veins, severe hyperesthesia are the usual features. Treatment: Mechanical cooling by elevating the limb (Note: Should not place the affected limb in cold water which may flare up the problems); aspirin; pregabalin, gabapentin, IV lignocaine are different drugs used in this condition.

LIVEDO RETICULARIS It is a condition with arteriolar spasm along with obstruction of capillaries by tiny blood clots with dilatation of venules, causing lace like purplish discoloration. Persistent, mottled, reddish-blue dermal streaks that do not blanche is typical. It may be idiopathic or related to vasculitis due to autoimmune conditions along with SLE or drug induced. Condition worsens by cold. Treating the cause; PUVA bath; warming the limb; exercise are different therapies.

POLYARTERITIS NODOSA It is a necrotising inflammatory reaction with commonly microscopic polyarteritis and nodule formation, often of small and medium-sized arteries (not capillaries), causing ischaemia of lower and upper limb. Visceral arterial (mesenteric) involvement (70%) can cause abdominal pain, GI bleed; mucosa! ulceration and perforation of small bowel. Massive hepatic infarction, cholecystitis can develop. Renal artery can cause loin pain , haematuria, and renal hypertension. Coronary artery also can get involved causing myocardial infarction. Disease is common at bifurcation of medium/small sized arteries leading to localised aneurysms. It is common in males (3: 1); fever, weakness, myalgia, arthralgia are early features. Presents with localised small aneurysms, like multiple5-1 0 mm nodules, palpable along the course of the artery. In late stage presents with myocardial infarction, renal failure, sepsis, GI bled. HBsAg is positive in 40% patients of polyarteritis nodosa. Angiogram of renal , mesenteric, peripheral arteries will show aneurysms at branching points. Biopsy of tender nodule, tender muscle is useful for diagnosis. Treatment is prednisolone 60 mg daily with cytotoxic drugs. Prognosis is poor with rapid death in early years.

SCLERODERMA/SYSTEMIC SCLEROSIS It is a progressive disease causing fibrosis of skin, GI tract, lungs, heart and kidney. It is common in females (4:1) at 4th/5th decade. It is considered as vasculitis even though earlier considered as collagen disease. Pathology consists of cytotoxic endothelial injury causing interstitial oedema, severe fibroblast proliferation causing fibrosis of affected vessels, and dilatation and proliferation of remaining capillaries as telangiectasis. Thin epidermis, thick dermis with more collagen with absence of appendages and rete pegs are typical. Lower 213rd oesophagus is sclerosed (50%) with increased collagen in submucosa with atrophied mucosa and muscularis. Dysphagia is common. Diffuse interstitial fibrosis, thickening of alveolar memb rane and pulmonary hypertension occurs. Synovial thickening causes arthritis; fibrosis of skeletal muscles; interstitial myocardial fibrosis causes bundle branch block, pericardia! effusion. Glomerulosclerosis in kidney is common (50%). Renal failure is common. Fibrosis of thyroid, periodontal membrane can occur. Malabsorption synd rome is common due to small bowel involvement. Involvement of digital arteries present as Raynaud's phenomenon . Calcinosis, Raynaud's, oesophageal hypomotility, sclerodactyly, and telangiectasia are the presentation of CREST syndrome. Investigations- anaemia, raised ESR, elevated lgG, presence of antinuclear antibodies and anticentromere antibodies (in CREST)-are different laboratory findings. Skin and peripheral arterial biopsy is confirmative. Treatment-is difficult. Drugs like D pencillamine, colchicines, p amino benzoic acid , vitamin E, dimethyl sulfoxide, ranitidine are tried at various levels. Vasodilators, warming and massaging skin, avoiding detergent soaps, oil and hydrophilic ointment application are used. Steroids, oxygen therapy for irreversible pulmonary fibrosis; haemodialysis for renal failu re; digitalis and other drugs for cardiac failure are needed later. Death is due to cardiac/pulmonary/renal failure.

ACROCYANOSIS (CRURUM PUELLARUM FRIGIDUM) It is persistent, painless cyanosis seen in fingers and often in legs with paraesthesia and chilblains affecting young females. It is chronic persistent arteriolar constriction with slow rate of blood flow. Trophic changes and ulcerations are not seen. Cyanosis which is persisting may aggravate on exposure to cold. It may be associated with endocrine dysfunction. Treatment:Vasodilators; Cervical sympathectomy (effective).

Raynaud's phenomenon

Acrocyanosis

• Episodic

• Persistent

• Painful

• Painless

• Acute arteriolar spasm

• Chronic constricti on

• lschaemic changes are common

• lschaemic changes are not seen

199

.....

GANGRENE

r

If the tips of (the patient's) fingers are falling off and are black, he will die. - (Anonymous), circa 2000 BC I

It is macroscopic death of tissue in situ (in continuity with adjacent viable tissue) with putrefaction (there will be loss of function also). Sites: Limbs; Appendix; Bowel; Testes; Gallbladder.

I Causes Secondary to arterial occlusion like atherosclerosis, emboli, diabetes, TAO, Raynaud's disease, ergots. Infective: Boil, carbuncle, gas gangrene, Fournier's gangrene, cancrum oris. Traumatic: Direct, indirect. Physical: Burns, scalds, frostbite, chemicals, irradiation, electrical. Venous gangrene.

I Clinical Features Colour changes. Pallor, greyish, purple, brownish black due to disintegration of haemoglobin to sulphide. Absence of pulse, loss of sensation, loss of function. Line of demarcation between viable and dead tissue by a band of hyperaemia and hyperaesthesia along with development of a layer of granulation tissue.

G) II)

::::,

.... DJ

II)

Figs . 1.36 4A and B: Gangrene of foot and leg. Note the line of demarcation. But skip lesion proximally shown signifies more proximal spread of gangrene which may need above knee amputation.

In dry gangrene the separation occurs by aseptic ulceration with minimum infection and the gangrene is dry and mummified. In wet gangrene, separation takes place by septic ulceration. Often demarcation is vague with skip lesions more proximally and so landing with higher level of amputations. Even after amputation skin flap may show die back process, leading to failure of taking up of flap of amputation and so requiring still higher level of amputation. Proximal ischaemic features may be present with rest pain, colour changes, hyperaesthesia-pregangrene.

Dry gangrene is due to slow, gradual loss of blood supply to the part causing dry, desiccated, wrinkled, mummified part with proper line of demarcation from the viable adjacent tissues Wet gangrene is due to infection with putrefaction, causing oedematous, swollen, discoloured part, spreading proximally, L -with vague line of demarcation from the adjacent viable tissues

I Investigations Hbo/o, blood sugar. Arterial Doppler, angiogram (Seldinger tech nique), CT angiogram. US abdomen to find out the status of aorta.

Fig . 1.362: Gangrene foot with ischaemic ulcer.

Fig. 1.363: Dry gangrene-great toe.

Fig. 1.365: lschaemic features of right hand; compare to left side which is normal.

Today well lived makes every yesterday, a dream of happiness and tomorrow, a vision of hope.

VI

.... .... C:

\Q

II)

':c;

)> .... ,... ....

II)

iii' 0 vi'

II)

DJ

"' II) "'

200

_.,, a:,

a:

Ill

T

Differences between dry gangrene and wet gangrene

Dry gangr,ns

Wst gangr,ne (Moist)

Clear line of demarcation is seen Dry, shriveled, mummified Slow, gradual loss of blood supply Separation is by aseptic ulceration Limits to the demarcation Causes are atherosclerosis, thromboangiitis obliterans (TAO)

Line of demarcation is vague Oedmatous, putrified, discoloured (H2S) Sudden loss of blood supply Septic ulceration causes separation Can extend proximally rapidly Emboli, trauma are the causes

I Treatment Limb saving methods: Drugs: Antibiotics, vasodilators, pentoxiphylline, praxilene, dipyridamole, small dose of aspirin, ticlopidine. Care of feet and toes: ,. The part has to be kept dry. , Any injury has to be avoided. , Proper footwear is advised (microcellular ru bber footwear, MCA). ,. Measures for pain relief is taken. , Nutrition supplementation is done. , The limb should not be warmed. , Pressure areas has to be protected. ,. Localised pus has to be drained. Cause is treated; Diabetes is controlled. Surgeries to improve the limb perfusion: Lumbar sympathectomy, omentoplasty. Profundaplasty, femoropopliteal thrombectomy or endarterectomy, arterial graft bypass are done according to the need. Life-saving procedures: Amputations may have to be done ofen. Level of amputation is decided on skin changes, temperature, line of demarcation, Doppler study. Below-knee amputation is a better option as BK prosthesis can be fitted better and also the movements of knee joint are retained. There is no need of external support and limp is absent. In above-knee amputation range of movements are less, limp is present, and often requires third (stick) support to walk. Different amputations done are Ray amputation, below-knee amputation (Buerger's amputation), Gritti-Stokes transgenial amputation, above-knee amputation. Lisfranc's, Chopart's, Symes', modified Symes' amputations are not commonly used in ischaemic limb as flaps will not survive.

DIABETIC FOOT AND DIABETIC GANGRENE Foot is a complex structure with many layers of muscles, ligaments, joints, arches, fat, thick plantar fascia, vascular arches, neurological system which maintains weight-bearing, gravity, normal walk, stability and gait (swing and stance phases). Problems in diabetic foot: Callosities, ulceration, Abscess and cellulitis of foot, Osteomyelitis of different bones of foot like metatarsals, cuneiforms, calcaneum, Diabetic gangrene, Arthritis of the joints

• • • •

Grade 0: Foot symptoms like pain, only Grade 1: Superficial ulcers Grade 2: Deep ulcers Grade 3: Ulcer with bone involvement Grade 4: Forefoot gangrene Grade 5: Full foot gangrene

I Pathogenesis of Diabetic Foot/Gangrene High glucose level in tissues is a good culture media for bacteria. So infection is common. Diabetic microangiopa thy causes blockade of microcirculation leading to hypoxia. •·· Diabetic neuropathy Due to sensory neuropathy, minor injuries are not noticed and so infection occurs. Due to motor neuropathy, dysfunction of muscles, arches of foot and joints occurs. And loss of reflexes of foot occurs causing more prone for trauma and abscess. Due to autonomic neuropathy, skin will be dry, causing defective skin barrier and so more prone for infection. Diabetic atherosclerosis itself reduces the blood supply and causes gangrene. Thrombosis can be precipitated by infection causing infective gangrene. Blockage occurs at plantar, tibial, and dorsalis pedis vessels. Increased glycosylated haemoglobin in blood causes defective oxygen dissociation leading to more hypoxia. At tissue level there will be increased glycosylated tissue proteins, which prevents proper oxygen utilisation and so aggravates hypoxia. Features: ,. Pain in the foot; Ulceration; Absence of sensation. ,. Absence of pulsations in the foot (posterior tibial and dorsalis pedis arteries); Loss of joint movements. , Abscess formation; Change in temperature and colour when gangrene sets in. , Patient may succumb to ketoacidosis, septicaemia or myocardial infarction. Investigations: , Blood sugar, urine ketone bodies. , Blood urea and serum creatinine. , X-ray of part to look for osteomyelitis. , Pus for culture and sensitivity.

:.- Doppler study of lower limb to assess arterial patency. ,. Angiogram to look for proximal blockage. ,. Ultrasound of abdomen to see the status of abdominal aorta. :.- Glycosylated haemoglobin estimation. Treatment: Foot can be saved only if there is good blood supply. ,, Antibiotics-decided by pus C/S. :.- Regular dressing. :.- Drugs: Vasodilators, pentoxiphylline, dipyridamole, low dose aspirin. ,, Diabetes is controlled by insulin only. :.- Diet control, control of obesity. Surgical debridement of wound. ,, Amputations of the gangrenous area. Level of amputation has to be decided by skin changes and temperature changes or Doppler study. , Care of feet in diabetic: - Any injury has to be avoided. - MCR footwears must be used (microcellular rubber). - Feet has to be kept clean and dry, especially the toes and clefts; Hyperkeratosis has to be avoided.

,, Warm drinks, analgesics, paravertebral injections to sympathetic chain, hyperbaric oxygen are effective. If gangrene develops, amputation is needed.

n

:c )>

AINHUM Ainhum also known as 'dactylolysis spontanea' is a painful constriction of the base of the fifth toe frequently (occasionally other toes also) followed by bilateral spontaneous autoamputation a few years later. Grooving pain constriction tendon, nerve and vessel bone gets cut spontaneously without any bleeding (auto-amputation) in many (2-5) years. Commonly affects males (can also occur in females). Common in blacks, in Negroes. History of running barefoot in childhood is common. Fifth toe is commonly affected. A fissure develops at the interphalangeal joint which becomes a fibrous band, that encircles the digit causing necrosis (Gangrene of little toe). Often it can be bilateral.

FROSTBITE

m

....r-

::ti

0

(I)

Qj

"' (I) "'

Treatment: It is early "Z" plasty. Amputation is often required later. Most often autoamputation occurs. Note-.

Yoruba people of Nigeria named ainhum.

ENDOVASCULAR SURGERIES

It is due to exposure to cold wind or high altitude. It is common in old age during cold spells. Damage to vessel wall occurs causing oedema, blistering, gangrene formation. Part is painless and waxy. Cells get frozen at - 5°C. Initially redness and oedema (1st degree); blister formation (2nd degree); skin necrosis (3rd degree); gangrene (4th degree) develops gradually. Treatment: ,, Gradual warming is done. Part should be wrapped with cottonwool and rested. Warming is gradually done with 44°C in 30 minutes with warm water. Limb elevation is done to reduce oedema. Intra-arterial vasodilators may help.

""C

;;;·

Fig. 1.367: Bilateral Ainhum involving 4th and 5th toes of both feet. Note the constriction ring in the toes.

Fig. 1.366: Gangrene of 3rd and 5th toes in a diabetic. Patient already underwent amputation of 4 the toe earlier for gangrene.

201

It is mainly used in peripheral vessels like femoropopliteal, renal, coronary, cerebral vessels.

Aneurysm for stenting and grafting Aortoiliac constrictive disease Renal artery stenosis; Carotid occlusive disease AV fistulas; Management of pseudoaneurysm

-----

•·· Balloon angioplasty: It is useful in short segment stenosis in large vessles like renal vessels, iliofemoral, coronary vessels. It is less effective compared to open surgery. lntravascular stenting: Balloon expandable and self-expanding stents are used at stenosed area.

Don't wait for your ship to come; swim out to it.

202

... ...::,

>,

QI

O'I

-"' V'I

0

::,

Endovascular grafts (PTFE, DACRON). Endovascular atherectomy. Angioscopy: Flexible, small, fibreoptic scopes to visualise vessel wall with sufficient irrigation to avoid opacification by blood. lntravascular ultrasound: To evaluate the vessel wall morphology. Complications:Thrombosis; Rupture; Sepsis; Fluid overload; Air embolism.

C

"' :E ,VI co a::

V'I

UPPER LIMB ISCHAEMIA It is a rare uncommon entity compared to lower limb ischaemia but important because of its difficulty in managing. Higher-level amputations are rare in upper limb ischaemia. Its incidence is rare (5%) due to abundant collateral supply, infrequency of atherosclerosis, decreased metabolic demand and smaller muscle mass. It mostly affects distal small arteries (90%). Symptoms are usually delayed. Types of upper limb ischaemia: Acute; Chronic.

Atherosclerosis-most common cause in USA. TAO of upper limb. Others- fibromuscular dysplasia; postirradiation-lung, breast; occupational injuries; vibration injury, hypothenar hammer syndrome; hypercoagulable states; APLA, polycythemia, cold agglutinins. Raynaud's phenomenon and disease. Symptoms of chronic ischaemia Upper limb 'Claudication' Weakness and wasting Digital ischaemia-ulcer, gangrene in finger tips Raynaud's phenomenon Signs ol chronic ischaemia Wasting of arm, forearm and hand muscles lschaemic changes in skin; tapering of finger tips Drop in systolic pressure >20 mmHg Proximal thrill or bruit Mass in the neck, thrill and bruit in the neck in supraclavicular region Adson test, hyperabduction (Halsted) test, Roos test, Allen's tests are important

a. Acute type Causes: Embolism-common: 30% of the peripheral emboli lodge in upper extremity. The most common site is at the bifurcation of brachia! artery (40%); next common is at axillary artery (12%). Embolism is due to: , Cardiac origin (70%)-valvular lesions (atrial fibrillation, endocarditis), IHD, paradoxical. , Others- aneurysms, thoracic outlet syndrome, plaque. Trauma-most common: Brachia! artery injury is seen in 30% of civilian trauma with arterial injuries, blunt injuries, fractures and dislocations, penetrating injuries. Iatrogenic.

Fig. 1.369: Gangrene of left index finger. Patient has undergone cervical sympathectomy.

I Investigations in Upper Limb lschaemia Fig. 1.368: Upper limb ischaemia with gangrene extending proximally towards elbow joint.

b. Chronic Type Causes: Arteritis-aortoarteritis, Takayasu arteritis, giant cell arteritis, connective tissue disease/vasculitis- scleroderma, SLE, RA, PAN, etc.

Laboratory tests for vasculitis, hypercoagulable states, and atherosclerotic risk factors. X-rays-for cervical rib; clavicular and first rib fractures; fractures and dislocations in extremity; pulmonary lesions of connective tissue disorders. Arterial Doppler study. Angiogram (subclavian angiogram)-CT/MR; conventional. CT scan neck and thorax. Blood sugar, lipid profile, cardiac evaluation.

Cilostazol-suppresses cAMP phosphodiesterase Ill rise in cAMP levels with antiplatelet, antithrombotic effects; induces vasodilatation; increases plasma HDL cholesterol; decreases plasma triglycerides. Catheter-based interventions , Atherectomy. , Angioplasty ± stenting by conventional or subintimal approach. , Stent grafts. , Cryoplasty. Surgery , Endarterectomy; Bypass surgery; Sympathectomy, extraperiosteal resection of the cervical rib.

Bypass Surgeries in Upper Limb /schaemia

Fig. 1.370: Upper limb angiogram showing blocks in subclavian artery.

I Management of Upper Limb lschaemia Treatment of the cause.

Treatment of Embolus

Conventional bypass , Aorto-subclavian/axillary bypass. , Subclavian-Axillary/brachial bypass. , Brachiodistal bypass. Extra-anatomical bypass ,, Carotid: Subclavian/Axil/ary bypass. , Subclavian: Subclavian bypass. , Axillary: Axillary bypass. Subclavian: Carotid transposition.

Time since the first symptom is very important. Clinical assessment of extent of ischaemia, immediate anticoagulation with heparin, Doppler study and angiogram of the arterial system, evaluation for the source of embolus-are the protocols. Embolectomy , Brachia/ embolectomy Local/regional/general anaesthesia is used. Longitudinal incision in the arm is used for proximal embolus; Lazy S-shaped incision across the elbow is done for embolus extending into the bifurcation and to expose the branches.

Treatment in Trauma General evaluation and resuscitation. Control of bleeding in open wound: Pressure bandage/manual compression (DO NOT USE TOURNIQUET) . Time since the event and clinical assessment of limb perfusion. Stabilisation of fractures and dislocations. Doppler study of arterial system, angiogram if required. Arterial repair; bypass graft either venous or synthetic.

Treatment of Chronic lschaemia Medical management , Risk modification-diabetes, hypertension, dyslipidemia, smoking, homocystinaemia, exercise training. :,.. Antiplatelets-aspirin/ticlopidine/clopidogrel. , Anticoagulants-heparin/warfarin. , Xanthines/pentoxiphylline/cilostazol.

Figs. 1.371A and B: (A) Carotid subclavian bypass; (B) Subclavian carotid transposition.

Treatment of Raynaud's Phenomenon Avoiding triggering agents. Drugs (vasodilators)-£alcium channel blockers, angiotensin II receptor blockers, alpha-1 adrenergic blockers, Sildenafil, prostaglandin E1. Surgery-sympathectomy. Note:

Individual topics about causes of upper limb ischaemia are discussed in different places.

He who attempts the absurd can achieve the impossible.

203

n

::c ):,, ""O

m

:::0 -' ,-

C\ n,

:::,

...

n, QI

VI C

... IO ... ':': n,

.......)>

...iii" n,

0

"' n, QI

"'n, "'

204

I Classification of Arterial Substitutes

B Trauma/cervical rib are the common causes Opposite limb, lower limbs should be examined Cardiovascular system should be examined Neck should be examined Wasting/girth should be checked All relevant clinical methods are equally significant Auscultation over neck/axilla/carotids for bruit are important Doppler; angiogram; nerve conduction studies; CT neck and thorax are essential investigations Arterial repair; therapy for cervical rib; scalenotomy; cervical sympathectomy are the different modalities of treatment Digital amputation may be required

~"' cc

a: I.I\

ARTERIAL SUBSTITUTES Ideal arterial substitute is not yet developed. Ideal arterial substitute should be strong, durable for patient's life, biocompatible, nonthrombogenic, should be resistant for infection, easily available, should have a long-term patency rate, and should have elastic property of normal artery.

T

Arterial allograft-not used. Arterial autograft-internal mammary artery (common), internal iliac artery. Arterial xenograft-bovine carotid artery graft-not used. Venous autograft-long saphenous vein (common) , small saphenous vein, basilic vein, cephalic vein. Venous allograft-umbilical vein graft. Prosthetic grafts , Textile grafts Dacron graft-knitted or woven or crimping or velour types. Dacron is polyethylene terephthalate. - Teflon graft-knitted or woven crimping or velour types. , Non-textile semi-inert polymer graft: - ePTFE graft-expanded polytetrafluoroethylene graft. Pree/offing the noncoated knitted or woven Dacron graft is done to seal the graft and to prevent leak, and to create a smooth lining at graft-blood interface. This step is not necessary for PTFE or newer grafts.

I Complications of Graft

Features of ideal graft

Strong

Should be leak proof on restoration of blood flow

Durable for patient's life

Should not chemically or physically degenerate

Nonthrombogenic

Should not cause any abnormal reaction to surrounding tissues

Biocompatible

Should not occlude when flexed

Resistant to infection

Should not damage blood contents

Flexible Should maintain long-term patency Should have elastic property of normal artery

Carrel and Guthrie first did venous autograft into arteries of dogs. They did extensive histological study of viable and nonviable grafts. Lexer in 1907 used saphenous vein for axillary artery repair. Murray started to use intraoperative heparin. Enaz Moniz and dos Santos originated technique of arteriography. Gross and his colleagues in 1948 started to use viable arterial allografts. Later it was found that, as of graft is considered tissue viability is not essential for success of graft uptake.

Neointimal fibrous hyperplasia at suture lines of the graft is due to surgical trauma, PDFG, arterial smooth muscle proliferation. Graft infection-incidence is 2%. It is more in lower limb graft than abdominal graft. Peroperative cephalosporin administration reduces the rate of graft infection. If infection occurs graft should be removed and revascularisation is achieved using a saphenous vein graft. Graft failure is rare but can occur. It is due to fiber degeneration, manufacturing defect, diffuse dilatation of graft (is due to expansion of the knit in knitted Dacron). Anastomotic false aneurysm (3%) occurs just adjacent to suture line towards host artery. It is tearing of the artery adjacent to suture line due to mismatched graft artery compliance, improper suture placement, and arterial degeneration. There will be partial or total separation of the graft from the host with blood collection in a covering of fibrous capsule. Eventually it will rupture/may cause thrombosis and embolism. Treatment is graft- artery reanastomosis with insertion of additional piece of graft.

M. Vascular Lesions

@ HAPTER OUTLINE • • •

Vascular Anomalies Haemangioma Vascular Malformations

• •

c.=.=.-::_------Cirsoid Aneurysm Arteriovenous Fistula

VASCULAR ANOMALIES It is a collective term used for haemangioma and vascular malformations. Haemangioma is a benign tumour containing hyperplastic endothelium with cellular proliferation and increased mast cells. Growth in tissue cultu re is observed. It is absent at birth, seen by 1 month in 30%. It usually shows biphasic growth phase with slow involution. 95% of cases achieve spontaneous involution. Fast growing type can cause platelet trapping and thrombocytopenia. Associated skeletal changes are not common but can occur. But bone erosion by the lesion can occur. It is common in girls (3:1). Vascular malformations are single layer endothelium lined spaces derived from arterial, capillary, venous or lymphatic system. There is no growth in tissue culture. Raise in mast cells is not seen. 90% cases are seen at birth; only few at later period. It is equal in both sexes (1 :1 ). Quiescent endothelium with vessels showing progressive ectasia is the featu re. Intravascular coagulation and mild thrombocytopenia can develop. Skeletal changes and overgrowth are common. Spontaneous involution is not common. Disfigurement, tissue destruction, deformity, dysfunction, telangiectasia, skin scarring are common. Szilagyi classification-(1} Cavernous haemangioma; (2) Microfistulous AV communications ; (3) Macrofistulous communications; (4) Anomalous mature vascular channels. Humburg classification-{1) Predominantly arterial/venous/ lymphatic defects with aplasia or obstructive dilatation which

is limited or infiltrative; (2) Predominantly AV shunting defects with deep/superficial limited or infiltrating lesions; (3) combined vascular defects-arterial, venous and haemolymphatic which may be limited or infiltrating. Diagnosis is made clinically and by radiological imagingcoloured Doppler, DSA, MRI. MRI is better than CT to identify the flow (MR angiogram is ideal}. Haemangiomas show intense parenchymal staining; vascular malformations show ecstatic vessels without much parenchyma; AVM shows rapid venous shunting.

HAEMANGIOMA It is the most common tumour in children (in 10% of term deliveries}. It is benign vascular endothelial tumour, common in girls (3 : 1}. It shows cellular endothelial hyperplasia with increased mast cells. Onset is few weeks after birth with biphasic growth showing initial rapid growth with gradual involution over 5-7 years. It is commonly seen in skin and subcutaneous tissue but can occur anywhere in the body like in liver, brain, lungs or other organs. It grows rapidly in first year and 70% involutes in 7 years. Early proliferative lesion is bright red, irregular; deep lesion is bluish coloured. Involution causes colour fading, softness, shrinkage leaving crepe paper like area. Commonly it is central; common in head and neck region (60%}. Often large haemangiomas may be associated with visceral anomalies. Head and neck haemangioma is associated with ocular and intracranial anomalies; sacral with spinal dysraphism. Multiple cutaneous haemangiomas may be associated with haemangioma of liver causing hepatomegaly, cardiac failure (CCF}, anaemia. 1

Vascular anomalies

Vascular anomalies

Haemangiomas Multllayered Mast cells

Vascular malformations Single layer No mast cells

Arterial/venous proliferation/ lymphatic ectasia

Aplasia/dilatation Limited/infiltrative

I

AV shunting defects

Superficial/deep Limited/infiltrative

You may be disappointed if you fail, you are doomed if you don't try.

Combined vascular defects

Arterial + venous + lymphatic Limited/infiltrative

.__

---

206

Ulceration, bleeding, airway block and visual disturbances are common complications. A definitive even though rare, but important life-threatening complication is platelet trapping and severe thrombocytopenia presenting as ecchymosis, petechiae, intracranial haemorrhage massive GI bleed. Raised angiogenic (fibroblastic) growth factor which is secreted in patient's urine is useful lab investigation to differentiate it from vascular malformations. HAEMANGIOMA CLASSIFICATION (OLD) Capillary - Strawberry haemangioma • Cavernous haemangioma

:,. It is actually a capillary malformation even though considered under haemangioma. It results from defect in maturation of sympathetic innervation of skin causing localised vasodilatation of intradermal capillaries. ,,. It is often associated with Stu rge-Weber syndrome, Klippel-Trenaunay-Weber syndrome and Proteus syndrome. ,, It needs treatment-laser (pulsed dye/diode); excision and grafting; cosmetic coverage. Expected result by treatment is not possible many times. Note: Presently word 'capillary haemangio ma' is restrict ed to straw berry type only; salmo n patch and po rt-wine stain are actually classified under vascular malformation s.

Note: Salmon patch and port-wine stain are actually capillary vascular malformations even though they were earlier classified under capillary haemangioma.

Vascular Malformations Salmon patch (stork bite): , It is actually capillary vascular malformation also called as naevus simplex; it is very commonly seen in 40% of newborns. , It presents at birth. It commonly occurs in nape of the neck (50%), face, scalp and limbs. It usually involves wide area of skin. It is caused by an area of persistent fetal dermal circulation. With age, it goes for spontaneous regression and disappears completely (usually in one year). Hence masterly inactivity is the treatment.

Fig. 1.373: Port-wine stain (Naevus flammeus).

I Capillary Haemangioma Strawberry haemangioma: , It may start at birth or child is normal at birth; between one to three weeks it appears as red mark which rapidly increases in size in 3 months to form strawberry/raspberry haemangioma. It contains immature vasoformative tissues. There will be eventually intravascularthrombosis, fibrosis and mast cell infiltration .

Fig. 1.372: Salmon patch.

Port-wine stain (Naevus flammeus): ,,. It presents at birth and persists throughout life without any change. Spontaneous regression will not occur. It presents as smooth, flat, reddish blue/intensely purple area; common in head, neck and face; often with maxillary and mandibular dermatomes of 5th cranial nerve. Eventually surface becomes nod ular and keratotic. , It persists throughout life. It is less common; seen in 0.3% of all newborns. It is also a capillary vascular malformation.

Fig. 1.374: Strawberry haemangioma.

, :., ,, , , ,

, ,

It is a true capillary haemangioma. It is 20 times more common than port wine stain. It is common in white girls. Male to female ratio is 1 : 3. It is common in head and neck region. It is clinically compressible, warm with bluish surface. Bleeding can occur after minor trauma and also ulceration. It involves skin, subcutaneous tissues and often muscles also. After 1 year of age, it slowly begins to disappear, and completely in 7- 8 years (70% in 7 years). It is the most common haemangioma.

!Haemangioma in periorbital region obstructs the vision in newborn with amblyopia and if it persists for 7 days causes permanent visual damage. Astigmatism also can occur Haemangioma in nasal area in newborn may obstruct nasal airway seriously (as newborn cannot breathe through mouth-obligatory nasal breathing) Skin ulceration may cause haemorrhage Infection can occur which may lead into sepsis, necrosis or rarely septicaemia • Systemic steroid for 3 weeks induces involution • Usually there is no role for surgery. Surgery is done only for retained tissue after involution

I Treatment

'--------

They are treated by wait and watch policy commonlyallowed for spontaneous regression. Pulsed dye laser (diode laser), surgical excision and reconstruction. Feeding vessels may need to be ligated after wide exposure before achieving complete extirpation. Sclerotherapy/cryotherapy/C02 snow therapy cause unpleasant scarring. Preoperative embolisation facilitates surgical excision and reduces the operative blood loss. When once embolisation done, surgery should be done as early as possible otherwise recurrence occurs and much more worried formation of enlarged collaterals can occur. Materials used are-foam, plastic spheres, stainless/platinum steel coils, ethanol, polyvinyl alcohol foam of 1000 µ meters size, and rapidly polymerizing acrylic. Problems are tissue necrosis, reaction, normal tissue embolisation. Procedure is done with interventional radiology under image intensifier guidance. Rapidly growing haemangioma may need systemic/oral and intralesional steroidtherapy. Antiangiogenic interferon 2a may be useful. Life-threatening platelet trapping may be controlled by cyclophosphamide chemotherapy. Haemangioma with drug resistant CCF can be treated with radiotherapy.

207 INDICATIONS FOR SURGERY OR INTERVENTION

Uncontrolled growth; Accidental haemorrhage l__Functional impairment like vision or hearin_g_ _ _ _

_J

Klippel-Trenaunay-Weber syndrome: Naevus flammeus + osteohypertrophy of extremities (soft tissue and bone hypertrophy) + varicose veins of lower limbs. If there is an association of arteriovenous fistula (AV fistula), it is called as Parkes-Weber syndrome Kasabach Merritt syndrome: Capillary haemangioma + DIC (Disseminated intravascular coagulation) with thrombocytopenia Sturge-Weber syndrome: Haemangiomas (Naevus flammeus) + hemiplegia and Jacksonian epilepsy (calcified vascular cerebral and meningeal deposits) + glaucoma Maffucci syndrome: Cavernous haemangioma + dyschondroplasia Proteus syndrome: Naevus flammeus + regional gigantism with lymphaticovenous malformation (asymmetrical hypertrophy) Osler-Rendu-Weber syndrome: Haemangioma of skin and lip with gastrointestinal tract haemangioma (hereditary haemorrhagic telangiectasia), (autosomal dominant)

I Cavernous Haemangioma It is present at birth and consists of a multiple venous channels. Its size increases gradually and may cause problems. It often contains feeding vessels which is of surgical importance. Sites. Head, neck, face, limbs, tongue, liver and other internal organs. Large or multiple cavernous haemangiomas can cause congestive heart failure (hyperdynamic) due to shunting of large quantity of blood. Cavernous haemangioma with dyschondroplasia is called as Maffucci syndrome. Cavernous haemangioma is often mixed with lymphatic component also (mixed vascular and lymphatic).

Figs. 1.375A and B: Cavernous haemangioma in (A) tongue and (B) knee.

When I was young, I observed that nine out of ten things I did were failures. So I did ten times more work. -George Bernard Shaw

....

"' lb :::, lb

... QI

... ... ':c;

V'I C:

IC lb

Ill

n

C:

...

iiT r lb

!!?. 0

:::, Ill

208

Features ;.. It is smooth, soft, well-loca lised, warm, fluctuant, compressible, nonpulsatile swelling with bluish surface occurring in skin and su bcutaneous tissue (often in mucosa like oral cavity) without any transillumination. , Compressibility and bluish surface is diagnostic. When swelling is pressed it reduces partially/often completely but when pressure is released it slowly attains its original size and shape. Vascular and lymphatic malformations are compressible. , It is usually nontender unless it gets infected or undergoes thrombosis or causes haemorrhage. Differential Diagnosis , Lymphangioma: It is brilliantly transilluminant unless it is infected or fibrosed. Lipoma, cold abscess, lymph cyst-clinically it is easier Fig. 1.377: Laparoscopic view of cavernous haemangioma of liver. It to differentiate. is the most common benign tumour of the liver.

Complications: Haemorrhage; DIC; Thrombosis; Infection, ulceration and septicaemia; Erosion into the adjacent bone; High output cardiac failure. Investigations: Ultrasound, Doppler; CT angiogram to find out feeding vessel; Platelet count; MRI/MR angiogram to see feeding vessels and deeper extension. Treatment , Sclerosant therapy: It is the initial first line of therapy. It causes aseptic thrombosis and fibrosis of the cavernous haemangioma with less vascularity and smaller size. It is directly injected into the lesion . Sodium tetradecyl sulphate/hypertonic saline are used. Often multiple injections are needed to achieve complete required effect. Later excision of the lesion is done. , Ligation of feeding arte,yand often at later stage excision is done once haemangioma shrinks. ,, Therapeutic embolisation. , If small and located in accessible area, excision is the initial therapy. ;.. Laser ablatio~diode pulsed laser is becoming popular because of good control of bleeding. COi Nd:YAG laser is also equally effective.

VASCULAR MALFORMATIONS

Figs. 1.376A and B: Cavernous haemangloma in the cheek near angle of the mouth and in the tip of the tongue. Haemangioma tongue is one of the causes of macroglossia.

Secondary to defect in development of vascular components, in 8th week of intrauterine period. Single layer endothelium lined spaces derived from arterial, capillary, venous or lymphatic system showing ectasia. There is no growth in tissue culture. Raise in mast cells is not seen. Associated with many syndromes. Can be located in skin or in deeper planes.

Present at birth and grows in proportion to child's growth. Pale skin which later darkens over the age or faint blue mass is the presentation. Spontaneous involution is not common. Capillary malformation (CM) type is due to lack of sympathetic control. Venous malformation (VM) type is most common vascular malformation which shows hypoplasia, hyperplasia or aplasia of superficial or deeper system. It is seen in subcutaneous plane as faint blue compressible mass with morning pain and stiffness of the area. Lymphatic malformation (LM) type can be microcystic (lymphangioma) or macrocystic (cystic hygroma). It can cause lymphoedema, soft tissue and bony hypertrophy, asymmetry (face), macrochelia, macroglossia, macrotia, cellulitis. Lowis/ow flow malformations can cause skeletal hypoplasia; high/fast flow malformations can cause hypertrophy. AVM is high flow type. Consumption coagulopathy (DIC) can occur. It is equal in both sexes (1:1). Doppler is commonly used investigation; but MRI (MR angiogram is with contrast is ideal to identify and to differentiate low and high flow types. Treatment-conservative with compression garments and sclerotherapy. Laser photocoagu lation is the choice for superficial malformations; multiple sittings may be needed; complete clearance may not be achieved. Surgical excision can be done. Preoperative embolisation may be needed. Vin rose patch: It is a congenital intradermal pale pink vascular malformation with dilatation of vessels in subpapillary dermal plexus. It may be associated with haemangiomas; AV malformations in limbs; congenital lymphoedema.

CIRSOID ANEURYSM It is actually a rare arteriovenous fistula I malformation of the scalp usually of congenital origin (80%) but occasionally can be traumatic. 90% occur in relation to superficial temporal artery but few occur additionally also in relation to occipital arteries. It should be differentiated from the true aneurysm of the superficial temporal artery. Cirsoid means varix. It is a rare variant of capillary haemangioma occurring in skin, beneath which abnormal artery communicates with the distended veins. Common ly seen in superficial temporal artery and its branches.

209 Often the underlying bone gets thinned out due to pressure. Occasionally extends into the cranial cavity. Ulceration is the eventual problem which will lead to uncontrollable haemorrhage.

Fig. 1.378: Typical cirsoid aneurysm involving superficial temporal artery region; it is commonly congenital.

I Features Pulsatile swelling in relation to superficial temporal artery, which is warm, compressible, with arterialisation of adjacent veins and with bone thinning (due to erosion). It feels like a 'pulsating bag of worms'. Investigations: Doppler study, CT scan; Angiogram, X-ray of the part. Treatment , Ligation of feeding artery and excision of lesion, often requires preliminary ligation of external carotid artery. , lntracranial extension requires formal neurosurgical approach. , Endovascular therapy is also useful. , Percutaneous direct puncture embolisation often with transarterial embolisation is also said to be effective method of treatment currently.

ARTERIOVENOUS FISTULA (AVF) It is an abnormal communication between an artery and vein.

Iii Types of AVF

Congenital: AV malformation Acquired: Traumatic

High flow: >800 ml/minute Normal flow: 400-800 mUminute Low flow: ,. QI

VI

Rl

Artery

::, C Rl

Vein

Vein

_.,,

cc a:

VI

Figs. 1.384A and 8: Acquired arteriovenous fistula in the wrist over

radial vessels. It should be palpated for compressibility and thrill. It should be auscultated for bruit.

AV fistula with aneurysmal sac

Dacron graft

Fig. 1.386: Reconstruction of AV fistula using graft.

Fig. 1.385: AV fistula created for treating chronic renal failure has formed an aneurysm. It may rupture to cause severe haemorrhage. Thrombosis or sepsis also can occur in this.

In emergency situation, quadruple ligation, i.e. both artery and vein above and below are ligated without touching the fistula and sac. Patient recovers well from cardiac failure. Therapeutic embolisation may be tried . Hunter's ligation should be avoided. It is used as life-saving measure because it invariably causes limb ischaemia and gangrene even though patient recovers from cardiac failure. It is ligation of artery proximally so as to make cardiac function normal. But it invariably steals the blood from the limb leading to gangrene. Artery

Vein

Changes Below the Level of the Fistula Because of diversion of arterial blood distal part becomes ischaemic. Because of high pressure arterialisation of veins and valvular incompetence occurs causing varicose veins.

Changes Proximal to the Fistula Hyperdynamic circulation causes cardiac failure. Cardiac failure may be very severe in traumatic AVF (often resistant to drug therapy). If pressure is applied to the artery proximal to the fistula, swelling will reduce in size, thrill and bruit will disappear, pulse rate and pulse pressure becomes normal. This is called as Nicoladoni's sign or Branham's sign.

Figs. 1.387A and 8: (A) Quadruple ligation of AV fistula. (B) Hunter's ligation is ligation of artery proximal to AV fistula. It should not be done as it causes diversion of all blood from periphery leading to gangrene of distal part.

Note: Other rare conditions

Campbell de Morgan spots. It is usually smaller (0.2 to 6 mm) in size, circular, elevated and bright red swelling. It is common in tru nk; common in elderly; also called as cherry angiomas. It usually does not require treatment; when needed, excision or electrodesiccation or laser removal is done. Parry-Romberg disease: It is hemifacial atrophy of skin, soft tissue and bone; common in females; usually begins at twenties. Atrophy of skin, fat, muscle, cartilage and bone causing coupe de sabre deformity- are the features.It is a self-limiting disease. Aesthetic reconstruction is offered when severe deformity develops. Hamartomata: Hamartano/hamartia means 'I miss' (Greek); or 'fault' or 'm isfire' or 'error'- (missing the mark in spear

throwing). Presently this terminology is not very much in use. It is a benign lesion with aberrant differentiation producing a mass of disorganised but mature specialised cells or tissue indigenous to the particular site. It is tumour like overgrowth of tissue or tissues proper to that part. It may be single lesion or multiple lesions; haemangiomas, lymphangiomas, AV malforma· tions, neural malformations are the examples. It is commonly observed in lungs, heart, brain (hypothalamus), skin, stomach, spleen, etc. It can be vascular, bone forming, cartilage forming, bronchial, neurofibromatosis, nevi, etc. Problems with hamartomas are-pressure symptoms, bleeding, infection, gigantism, cosmetic problem. Treatment: Depends on site, type, extent; cryotherapy, ligation of feeding vessels, sclerotherapy, excision or laser therapy are options.

213

C\ 11)

::J

...

11) QI

V'I C:

...

10

... ':c; 11)

VI

n

C:

...r-er 11)

~-

0

::J

VI

Attitudes are more important than tacts.

N. Venous Diseases

. ..

..

( iHAPTER OUTLIN E

.... ..

Anatomy of Veins of Lower Limb Physiology of Venous Blood Flow in Lower limb Deep Vein Thrombosis Varicose Veins Venous Ulcer

....

.... ..

Compression Therapy for Varicose Veins Thrombophlebitis Klippel-Trenaunay Syndrome Anticoagulants Oral Anticoagulants Pulmonary Embolism

ANATOMY OF VEINS OF LOWER LIMB

I Deep Veins

Tibial , popliteal, femoral veins are cal led as "veins of conduits"which drain blood into iliac veins and then to IVG. Pumping veins: They are venous sinuses existing in the calf muscles which pump blood towards major veins. They are better termed as muscufovenous pumps. They are also called as the peripheral heart. Paired veins of anterior tibial, posterior tibial and peroneal with soleal and gastrocnemius veins join together to form popliteal vein which at subsartorial canal (Hunter's) continue as femoral vein where it is joined by profunda (deep) femoral vein . Later femoral vein continues as external iliac vein beyond above the inguinal ligament.

Fig. 1.388: Bilateral varicosity of great saphenous veins. Superficial epigastric -

,

-

~

:..__ Superficial external pudenda!

~ - - Posterior vein

I Superficial Veins

Long (Great) saphenous vein (LSV I GSV): It begins from the medial part of the dorsal venous arch of the foot runs in front of the med ial malleolus ascends up to the posteromedial aspect of the knee joint, and then ascends Fig. 1.389: Great saphenous vein-anatomy and tributaries. upwards in the th igh towards the sap henous opening. Saphenous opening lies 3. 75 cm below and lateral to the of the popliteal fossa and ends into the popliteal vein. It has pubic tubercle. It pie rces the cribriform fascia to enter the got 6-12 valves. Sural nerve runs closely along the SSV which femoral vein. LSV contains 12-20 valves. It is the longest may get injured during surgery. Saphenopopliteal junction is vein in the body. In the lower part of the leg LSV is closely variable, but usually located at popliteal fossa; it can be above associated with saphenous nerve which can get damaged or below the actual site. during surge ri es to LSV varicosity. Tributaries of GSV Posterior arch vein of Leonardo from medial ankle to the are- posterior arch vein, anterior vein of the leg, anteroLSV below. lateral vein, posteromedial vein and sometimes accessory Anterior arch veins to popliteal veins. saphenous vein. Short (Small) saphenous vein (SSV): Lateral marginal Note: vein of the foot behind the lateral malleolus continues as • Superficial veins have got multiple valves; blood always drains from superficial veins towards deep veins. Inferior vena cava (IVG) and iliac SSV ascending upwards along the lateral margin of the veins do not have valves. Superficial veins of lower limb drains skin and tendoachilles. It runs along the middle of the back of the leg subcutaneous tissues (10% of blood). Veins can accommodate large between two heads of the gastrocnemius into the lower part quantity of blood hence called as capacitance vessels.

I Communicating Veins

ssv

Per/orators

m

215

They are veins in the subcutaneous plane communicat ing between different superficial veins of the leg. They usually do not perforate the deep fascia. Long intersaphenous communicating vein often exists between cranial extension of SSV to join GSV and can be varicose and pathological and is called as communicating vein of Giacomini-Cruveilhier. Anterior accessory great saphenous vein (AAGSV, Anterolateral vein of thigh) is communicating vein into the GSV anteriorly and laterally. AAGSV communicates into GSV usually just proximal to preterminal valve (60%); often at confluence (39%); rarely onto femoral vein (1%). In many patients with varicose veins it is this vein which is diseased than GSV. It often receives superficial circumflex vein before joining the GSV. Communicating veins are often called as anastomotic veins.

Figs. 1.390A and B: Short saphenous vein varicosity.

Skin Subcutaneouscompartment

-

- --

- --Tributary saphenous vein Fascia Aponeurosis

I Perforator Veins

Perforator vein Valve

Muscular compartment

Deep vein

f'J Perforator vein Valve

Subcutaneous compartment

Deep vein

B • • • • • •

Ankle perforators (May or Kuster) Bassi's paraAchillean (5 cm above calcaneum) perforator Lower leg perforators: I, 11, 111(of Cockett) Gastrocnemius perforators (of Boyd)- below knee Mid-thigh perforators (Dodd) Hunter's perforator in the thigh Common femoral vein

Great saphenous vein (GSV/LSV)

Hunter's (Hunterian)

Boyd's

Small saphenous vein

Cockett's May and Kuster's (ankle)

~

-+- Tributary

•r-,.:..;c.--=IIHHI---+- Muscular I

Compartment

Figs. 1.392A and B: Communicating and perforator veins in the leg.

PHYSIOLOGY OF VENOUS BLOOD FLOW IN LOWER LIMB Dodd's

Popliteal vei

Saphenous compartment

GSV

m

Bassi's (paraachilis)

Fig. 1.391: Perforator veins and different compartments in the leg.

):,,

"'0

-t

m

....

:::0

z

G\ l'1) :,

...

l'1)

GI

VI

...C IC ... l'1)

':c:

< (I)

:,

• Spider veins (pin veins/dermal veins/telangictasia/hyphen reticular vein (feeder vein/subdermal superficial deep vein. Malleolar flare (ankleflare/corona phlebectatica) is a fan shaped venous flare is an early sign of advanced venous disease.

They are the veins which connect superficial to deep veins at various levels. They travel from superficial fascia through an opening in the deep fascia before entering the deep veins. The direction of blood fl ow here is from superficial to deep veins. These perforators are also guarded by valves so that the blood flow is unidirectional, i.e. towards deep veins. Reversal of flow occurs due to incompetence of perforators which will lead to varicose veins.

("'\

::I:

Veins are thin-walled collapsible vessels, that assu me an elliptical configuration in collapsed state and circular configuration in the filled state. Venous valves are abundant in the distal lower extremity and the number of valves decreases proximally, with no valves in superior and inferior vena cava.

I Factors Affecting the Venous Return Arterial pressure across the capillary increases the pumping action of vein.

The fool is never satisfied while the wise man finds wealth in contentment

0 C

VI

VI l'1) GI VI l'1) VI

216 4----

Perforator

Fig. 1.393: Long saphenous vein with perforator incompetence with venous ulcer. Calf muscu/ovenous pump: During contraction phase of walking, pressure in the calf muscles increases to 200-300 mmHg. This pumps the blood towards the heart. During relaxation phase of walking, pressure in the calf falls and so it allows blood to flow from superficial to deep veins through perforators. Normally while walking, pressure in the superficial system at the level of ankle is 20 mmHg. During walking, foot pump mechanism propels blood from plantar veins into the leg. Gravity. Note:

Pressure in arteriolar end of the capillary is 32 mmHg; venular end of capillary is 12 mmHg. FACTORS RESPONSIBLE FOR VENOUS RETURN

Negative pressure in thorax Peripheral pump- calf muscles Vis-a-tergo of adjoining muscles Nonrefluxing valves in course of veins

- - - - - -- - ~

DEEP VEIN THROMBOSIS Deep vein thrombosis (DVT) is called as phlebothrombosis. It is semisolid clot in the vein which has got high tendency to develop pulmonary embolism and sudden death. Common site of beginning of thrombus is soleal veins which later propagate proximally, often gettin g detached to cause acute massive pulmonary embolism or moderate sized emboli can cause pyramidal/wedge shaped pulmonary infarcts.

I Causes Following childbirth. Trauma- to leg, ankle, thigh, pelvis.

Muscular violence. Immobility: Bedridden patients, individuals on long duration air or bus travel (Traveller's thrombosis). Debilitating illness, obesity, immobility, bed rest, pregnancy, puerperium , oral contraceptives (increases the risk by 5 times), estrogens. Postoperative thrombosis (Most common cause): Common after the age of 40 years. Incidence following surgeries is 30%. In 30% of cases both legs are affected. Usually seen after prostate surgery, hip surgery, major abdominal surgeries, gynaecological surgeries, cancer surgeries. Bedridden for more than 3 days in the postoperative period increases the risk of DVT. Spontaneous thrombosis is common in visceral neoplasm like carcinoma pancreas or carcinoma stomach. It is often migrating type. Thrombus may start in a venous tributary which eventually may extend into the main vein causing DVT. Axillary vein thrombosis ,. It can occur spontaneously, following compression by cervical rib, by various causes of thoracic inlet syndrome, or arm being in the hyperabduction state for prolonged period (e.g. painting the ceiling), after axillary lymph node block dissection, after radiotherapy to axilla, occasionally as a complication of venous cannulation. , Upper limb DVT is rare compared to lower limb DVT (5% of all DVT). It may be axillary or subclavian vein or both. , But 30% of upper li mb DVT can cause pulmonary embolism. , Primary upper limb DVT is Paget-Schroetter syndrome, is due to subclavian vein compression that occurs in thoracic outlet syndrome. It may be precipitated by exertion of arms, swimming, exercise, etc. , Idiopathic upper limb DVT is rare. Occult underlying malignancy should be thought of. , Secondary upper limb DVT is due to CVP line, pacemaker thrombocytosis, malignancy, surgeries, radiotherapy, etc. , Unilateral arm, forearm swelling with bluish discolouration, pain, pitting oedema, often with skin blebs are the features. , Investigations are- Duplex scan, MR venography (as clavicle obscures proper duplex evaluation), BT, CT, PT, APTT, platelet count estimation. , Treatment is similar, with heparin/LMWH/warfarin, thrombolysis using tissue plasminogen activator, elevation of the arm, using compression stockings. Polycythaemia vera, thrombocytosis. Deficiencies of antithrombin 111, protein C, protein S, factor V of Leiden, thrombophilia. Recent myocard ial infarction, heart fai lure, nephrotic syndrome. Thrombosis can occur in individuals who sit with computers for long time-'ethrombosis'.

Sites a. Pelvic veins-not uncommon; involves internal iliac veins. It is

more common in PIO (pelvic inflammatory disease) in females. In males prostatic veins may be the site of origin. It is difficult to identify clinically even though rectal or vaginal examination may help. b. Leg veins- very common in veins of soleus muscle in calf. Femoral vein/iliofemoral vein thrombosis can occur along with calf veins or independently without calf vein thrombosis which shows adductor canal tenderness. lliofemoral vein thrombosis is common on left side due to its lengthy course/compression by right iliac artery/often due to presence of web at its entry into IVG. Incidence of bilateral leg DVT is 30% which should be 1 differentiated from bilateral pedal oedema due to other causes I like hypoproteinaemia, renal failure and cardiac causes. ~ er limb veins-not uncommon (Axillary vein thrombosis).

Phlegmasia Alba Do/ens It is DVT of femoral vein (deep femoral vein commonly) causing painful congestion and oedema of leg, with lymphangitis, which further increases the oedema and worsens the situation ( white leg).

Fig. 1.394: Deep vein thrombosis (DVT) in both legs. 30%

cases of DVT are bilateral.

217

FEATURES OF OVT

Commonly it is asymptomatic-60% Fever-most common Tense, tender, warm, pale/bluish, shiny swelling calf Positive Haman's, Mose's or Neuhof's signs Inverted champagne bottle sign Features of pulmonary embolism

--------~

I Features Fever-earliest symptom. Pain and swelling in the calf and thigh (often). Pain is often so severe that the patient finds it difficult to flex (or move) the leg. Leg is tense, tender, warm, pale or bluish with stretched and shiny skin. Positive Haman's sign: Passive forceful dorsiflexion of the foot with extended knee will cause tenderness in the calf. Mose's sign: Gentle squeezing of lower part of the calf from side-to-side is painful. Gentleness is very important otherwise it may dislodge a thrombus to fo rm an embolus. Neuhof's sign: Thickening and deep tenderness elicited while palpating deep in calf muscles. Most often, DVT is asymptomatic and presents suddenly with features of pulmonary embolism like chest pain, breathlessness and haemoptysis. After applying tourniquet at saphenofemoral junction, patient is made to walk and without removing the tourniquet, limb is elevated- persisting prominent superficial veins will be observed in DVT- Linton's test. Differential diagnosis for DVT: Ru ptured Baker's cyst; Ruptured plantaris tendon; Calf muscle haematoma; Cellulitis leg; Superficial thrombophlebitis.

I Investigations Venous Doppler with Duplex scanning: It shows noncompressible vein which is wider than normal. On compression over calf muscles, it does not show any augmentation of flow. Normal venous sound at the area of femoral vein which disappears during inspiration is conspicuously absent in DVT.

Fig. 1.395: Right leg venous gangrene. Note the discolouration,

blebs and oedema.

Phlegmasia Caerulea Do/ens It is extensive DVT of iliac and pelvic veins causing blue leg with either venous gangrene or areas of infarction.

Fig. 1.396: US showing IVG thrombosis.

Peace begins with a smile. Beauty is power; smile is its sword.

n

:::c

l>

.... m .... "'C

z

Cl /1)

::i

...

/1)

Qj

V'I

... ...

C: I.C /1)

':c:

::i

0

C:

V,

9.

V,

/1) Qj V,

/1) V,

...>. ...::,Cl QI

V\

0

::, C:

, V'I

cc a:

V\

Venogram ,. Contrast material is injected into venous system to get detailed idea of the veins after applying tourniquet into superficial system. Occlusive and nonocclusive thrombus can be differentiated by this. But as it is invasive one, it is not commonly done at present. MR venogram is under trial at present. Impedance plethysmography is used to measure the rate of venous emptying. Vein occlusion is done using cuff around upper thigh which is confirmed by flat electrical wave pattern . When cuff is released rapid flow of wave is observed in normal; sluggish flow of wave is seen in DVT. Radioactive 1125 fibrinogen study ,. Sodium iodide 100 mg orally is given to the patient 24 hours before the test to block the thyroid activity. 1125 labelled fibrinogen 100 µ Curies is injected intravenously. First radioactivity of heart is measured by placing the scintillation counter over precordium. Reading obtained by this is adjusted as 100%. After that legs are elevated using adjustable stands and to prevent venous pooling, scintillation counter is placed over the calf. Counting in the leg is done from below upwards at 5 cm intervals. Procedure is done in preoperative period; on 1st, 3rd and 6th postoperative days. A 20% or more raise in percentage value suggests deep vein thrombosis in leg. 1125 labelled fibrinogen is used (earlier 1131 labelled fibrinogen was used) because it has got shorter radioaction; its detectability is done with much lighter and mobile apparatus. ••• Haemogram with platelet count, 0-dimer test/analysis of fibrin degradation products (FOP) are relevant tests used. D-dimer test is measurement of cross-linked degradation products which interprets the plasmin activity on fibrin. Negative D-dimer test is of more value. Ventilation-perfusion scanning with mismatched defects; pulmonary artery CT scan with filling defect; pulmonary angiography are the investigations to confirm the pulmonary embolism.

I Treatment Rest, elevation of limb, bandaging the entire limb with crepe bandage. Anticoagulants: Heparin/low molecular weight heparin, warfarin, phenindione. For fixed thrombus:

,

Initially high dose of heparin of 25,000 units/day for 7 days is given. Then later patient is advised to continue warfarin for 3- 6 months. Dose is controlled by assessing Activated Partial Thromboplastin Time (APTT). Duration of heparin treatment is usually for 7-10 days. Dose of heparin is often calculated as- 80 units/kg bolus of heparin followed by 15 units/kg of infusion. .,, Low molecular weight heparin is preferred to heparin. , Warfarin should be started as early as possible (same day of heparin therapy). Day one and day two- 10 mg each day; day three-5 mg. On day three prothrombin

time should be done. Warfarin is given for 3-6 months with regular monitoring, depending on the cause, risk group, and severity of DVT. INR should be maintained between 2.0 to 3.0. , Oral anticoagulants being teratogenic cannot be used during pregnancy. LMWH is the drug of choice used during pregnancy and postpartum period. For free thrombus:

B Fibrinolysins Thrombectomy using Fogarty's catheter IVG filter

,

Fibrinolysins: Streptokinase, 6 lakhs to start with and later one lakh hourly. It is commonly infused directly into the affected vein through a venous catheter. Urokinase or tissue plasminogen activator may also be used to dissolve thrombus (it should not be given when patient is on heparin).

B

FIBRINOLYSINS

Tissue plasminogen activator-100

1.5

Immobilization more than 3 days or surgery within last 4 weeks

1.5

Previous DVT or PE

1.5

Haemoptysis

1.0

Malignancy in last 6 months

1.0

Score 4 =suggestive of PE.

Low dose heparin is given in suspected cases, in major surgeries and continued during postoperative period till the patient is ambulated. 5,000 units is given subcutaneously 2 hours before surgery. Low molecular weight heparin is preferred drug than heparin as it can be used once a day; it does not require monitoring; it does not cause thrombocytopenia (like heparin); and there is lesser risk of bleeding. Various measures like graduated static compression, elastic stockings, electrical stimulation of calf muscles, pneumatic compression are used to prevent sluggish flow of blood. Dextran 70, intravenously 500 ml during surgery and another 500 ml postoperatively in 24 hours can also be used to prevent DVT. Smoking increases the viscosity of blood and so should be stopped. Patients on oral contraceptives or oestrogens should stop the drug 6-8 weeks prior to any elective surgery.

, - - - -lliofemoral block

LSV

EFFECTS ANO SEQUELAE OF DVT

Fig. 1.397: Palma operation for iliofemoral block. Using opposite saphenous vein femoral vein is connected to other femoral vein.

Pulmonary embolism-15% Infection; venous gangrene Partial recanalisation, chronic venous hypertension around the ankle region causing venous ulcers-chronic venous insufficiency-CVI Recurrent DVT-30% Propagation of thrombus proximally-20-30%

VARICOSE VEINS Fig. 1.398: Crepe bandages applied to both legs in bilateral varicose veins and DVT.

Palma operation: In iliofemoral thrombosis, common femoral vein below the block is communicated to opposite femoral vein through opposite long saphenous vein. May-Husni operation: When blockage is in popliteal vein, popliteal vein below the block is anastomosed to long saphenous vein (end-to-end) so as to bypass the blood across the popliteal block.

It is permanently elongated, dilated vein/veins with tortuous path causing pathological circulation. There is reversal of blood flow through its faulty valves. Risk factors being heredity; female sex; occupation that demands prolonged standing; immobility; raised intraabdominal pressure like in sports, tight clothing, pregnancy, raised progesterone level and altered estrogen-progesterone ratio, chronic constipation, high heels. Prevalence of varicose veins is 35%; severe varicose veins is 10%; chronic venous insufficiency (CVI) is 8%; ulcer is 2%.

I Prevention of DVT Categorise the patient as low/moderate and high-risk. Low-risk young patients undergoing surgery for less than 30 minutes. Moderate-risk-patients above 40 years of age undergoing major surgery. High-risk-one who had existing cardiac diseases, stroke, previous history of DVT, suffering from malignancy Mechanical methods-elastic compression bandage; elevation; external pneumatic bandage Pharmacological- low molecular weight heparin-once a day

I

Care has to be taken to see for proper positioning of legs with no pressure on the calf muscles. Pressure bandage to the legs has to be applied during major surgeries, laparoscopic surgeries. During postoperative period, elevation, massaging, pressure bandage, early ambulation, maintaining hydration are essential measures.

Figs. 1.399A to C: Typical varicose vein with skin changes and venous ulceration.

Every heart has pain: some hide it in eyes; some hide in their smile!

n

:I:

l>

"'C

-I

m ::c

.... 2

220

;::(I)

I Classification I Long/great saphenous vein varicosity. Short/small saphenous vein varicosity. Varicose veins due to perforator incompetence.

O'I ....

::J VI

0

(ti

::J C:

Fig. 1.402: Atrophic blanche.

(ti

_.,,

• Small varicose vein is 4 mm in diameter. • Coronaph/ebectatica are blue telangiectasias on the medial aspect of the foot below the malleolus around ankle level. More than 5 such lesions are the best independent predictor of the skin changes. • Pulsatile varicose veins in lower limb is seen in Klippel-Trenaunay syndrome

cc 0::

VI

I Classification Ill CEAP Classification of Lower Limb Varicose Veins (2004)

m

CEAP CLASSIFICATION

Figs. 1.400A to C: (A) Great saphenous vein varicosity. (8) Small saphenous vein varicosity. (C) Perforator incompetence (blow outs).

I Classification II Thread veins (or dermal flares/telangiectasis/spider veins/ Hypen veins are 0.5-1 mm in size): Are small varices in the skin usually around ankle which look like dilated, red or purple network of veins ( Venulectasia). Spider naevi/ venous flares are common in females. Reticular varices (1-3 mm in size): Are slightly larger varices than thread veins located in subcutaneous/subdermal region. Varicose veins: They are dilated , tortuous, elongated superficial veins located in the subcutaneous tissue (saphenous compartment) equal or more than 3 mm in diameter measured in standing position. Combination of any of the above.

C-Clinical signs (grade 0-6) ; (A) for asymptomatic or (S) for symptomatic presentation E- Etio/ogica/ classificat ion: Congenital (Ee), Primary (Ep), Secondary (Es), No venous etiology (En) A-Anatomic distribution: Superficial (As), Deep (Ad) or Perforator (Ap), No venous location identified (An) P-Pathophysio/ogic dysfunction: Reflux (Pr), Obstructive (Po), Both, or No pathophysiology identified (Pn)

GRADING OF CLINICAL SIGNS (C) 0- No visible or palpable signs of venous diseases 1- Telangiectases, reticular veins or malleolar flare 2-Varicose veins 3-Oedema without skin changes 4- Skin changes due to venous diseases like pigmentation, eczema or lipodermatosclerosis 4a-pigmentation; 4b-lipodermatosis, atrophia blanche 5- Skin changes as above with healed ulceration 6-Skin changes as above with active ulceration ANATOMICAL DISTRIBUTION (A)

Fig. 1.401 : Thread veins are up to 1 mm diameter; reticular veins are 1- 3 mm in diameter. Note: • Atrophic blanche is localized white atrophic skin surrounded by hyper pigmentation and dilated capillaries. • Saphena varix is a large groin varicosity at SFJ which disappears on lying down and imparts an impulse and thrill on coughing.

As-superficial system: 1-Telangiectases, reticular veins 2-Great saphenous vein above the knee-ostial and preterminal 3-Great saphenous vein below the knee 4- Small saphenous vein 5-Nonsaphenous--43% Ad-deep system: From 6 to 15 Ap-perforator system: 17-Perforator vein (PV) of the thigh 18- Perforator (PV) of the calf and leg An- no anatomical lesion identified

I Pathogenesis • Fibrin cuff theory • White cell trapping theory Incompetence of venous valves .J, Stasis of blood

having superficial vein incompetence (30%) with or without perforator incompetence or deep vein incompetence (30%) or having previous DVT with complete obliteration or partial recanalisation with incompetence called as post-thrombotic syndrome (30%). Varicose vein is a condition of progressive deterioration even often with interventions.

221

(')

:::r:: )>

"'0

-f

m

:x,

z

,j,

Chronic ambulatory venous hypertension -1,

C)

Defective microcirculation

It)

,j,

It)

:::,

....

QI

RBC diffuses into tissue planes

Vl C: \Q

-1,

.... ....

Lysis of ABC's

It)

-1,

':':

Release of haemosiderin ,j,

Pigmentation .J, Dermatitis .J, Capillary endothelial damage -1,

::::, 0

C: VI

S?. VI

It)

Fig s. 1.403A and B: Lipodermatosclerosis is pigmentation,

thickening, and induration of the skin due to venous diseases.

Prevention of diffusion and exchange of nutrients -1,

Severe anoxia Chronic venous ulceration (Fibrin cuff theory) .

Inappropriate activation of trapped leucocytes release proteolytic enzymes which cause cell destruction and ulcerationWhile cell trapping theory. Fibrin deposition, tissue death, scarring occurs together, called as lipodermatosclerosis. Secondary valvular venous reflux venous wall effects. Weakening of the venous endothelial wall and valves occur due to raised venous wall tension by-(1) Shearing stress pressures of blood flow, (2) Increased matrix metalloproteinases (MMPs) activity on endothelium and smooth muscle cells reducing structural integrity of venous wall with decreased elastin content in the media of the vein, (3) Changes in normal venous constriction and relaxation properties, (4) Recu rrent inflammation. Venous system in the lower limb is maintained by-(1) Valvular competence, (2) Venous patency, (3) Calf muscle pump which is venous channel/plexus within the soleus muscle. Any change in any of these systems can cause venous insufficiency. Chronic venous insufficiency (CV/) is a syndrome resulting from continuous chronic venous hypertension/ambulatory venous hypertension [AVP] (>80 mmHg venous pressure at ankle) in the erect posture either on standing or exercise (in normal people venous pressure in superficial system falls during calf contraction). CVI consists of postural discomfort, varicose veins, oedema, pigmentation, induration, dermatitis, lipodermatosclerosis and ulceration. CVI patients may be

Predisposing factors for varicose veins are-age, sex, race, obesity, height, left > right, occupation, family history, erect posture.

Fig. 1.404: Typical site of venous ulcer. Note the

m

pigmentation and chronicity.

SITES WHERE VARICOSITIES CAN OCCUR

Lower limb, Sites of portosystemic anastomosis Vulva, perineum, Pampiniform plexus of veins

m

REMEMBER

20% of population suffers from varicose veins. Prevalence of varicose veins increases with age (from 11 % at 18 years of age to 55% at 55 years). Veins are three times of arteries. 70% of blood in the ,;,__ of time. __, circulation is in veins at a given point Contd...

One negative thought can burn all positive thoughts.

________

QI

VI It) VI

Contd...

...>. ...Cl QI

:I VI

0

ro

:I C:

ro

_.,, a:)

a:: VI

Superficial veins carry 10% of venous system. Superficial veins of the lower limb have thick muscular wall and are located in condensed superficial fascia (between skin and deep fascia called as saphenous fascia), have low pressure; have plenty of valves to propagate blood. Blood from these veins enter into the femoral vein through SFJ and through SPJ and through perforators into the deep veins. Long saphenous vein (LSV) is the longest vein in the body has 10-20 valves. Posterior arch vein of Leonard starts behind the medial malleolus ascending upwards to join the LSV at knee level; anterior superficial vein (along the shin joining the LSV at knee level) ; posteromedial and anterolateral accessory veins in the thigh join LSV near SFJ. Superficial epigastric, superficial circumflex iliac and superficial external pudenda! veins are tributaries at SFJ. Deep venous system in the leg comprises femoral and popliteal veins; pairs of venae commitantes of the tibial, posterior tibial and peroneal arteries (total 6 venae comitantes, soleal and gastrocnemius veins join to form popliteal vein); being valveless blood takes within the calf muscles communicates with superficial system via saphenofemoral junction, mid-thigh perforators, short saphenopopliteal junction and calf perforators. Primary varicose veins are often familial; with unknown cause could be due to weak valve or wall. It is seen in younger age, main saphenous veins are usually affected; deep veins are usually patent; only Trendelenburg test-I is positive; complications are less and late to occur. Occupation prolonged standing and muscular weakness are contributory factors. Congenital is; due to absence valve; it is uncommon. Secondary type is acquired one; it occurs in adult and old; saphenous, perforators and often deep veins are affected; both Trendelenburg tests I and II are often positive; complications are common and quick to develop. Perforators normally drain only from superficial to deep veins across a valve (through deep fascia); in perforator incompetence flow reverses from deep veins towards superficial veins as 'blow outs' which are more prominent during calf contraction. Communicating veins are one which communicates between one or more superficial venous system; example is communicating vein between long and short saphenous veins. Symptom classification: Asymptomatic is group I with venous telangiectasia, thread/reticular/varicose veins; symptomatic group II with cramps, heaviness, tenderness, itching, swelling; complicated group Ill is with haemorrhage, superficial thrombophlebitis, eczema, lipodermatosclerosis and venous ulcers. CEAP classification is also important. Symptoms are less severe in morning than at evening. In atrophie blanche white patches develop in skin due to severe varicose veins. Dermal flares are thread veins seen in the skin with 3 mm in diameter. INR (International Normalised Ratio): Ratio of measured PT to a mean lab control PT corrected for the sensitivity of thromboplastin

I Aetiology of Varicose Veins Varicosities are more common in lower limb because of erect posture and long column of blood has to be supported which can lead to weakness and incompetency of valves. Incidence is 5% of adult population. Primary varicosities due to: ,. Congenital incompetence or absence of valves. ,. Weakness or wasting of muscles-defective connective tissue and smooth muscle in the venous wall. ,. Stretching of deep fascia. , Inheritance (family history) with FOXC2 gene. ,. Klippel-Trenaunay syndrome, avalvulia, Parkes-Weber syndrome. Here varices are of atypical distribution. Secondary varicosities: ,. Recurrent thrombophlebitis. ,. Occupational-standing for long hou rs (traffic police, guards, sportsman). ,. Obstruction to venous return like abdominal tumour, retroperitoneal fibrosis, lymphadenopathy, ascites. ,. Pregnancy (due to progesterone hormone), obesity, chronic constipation. ,. AV malformations-congenital or acquired. ,. Iliac vein thrombosis. ,. Tricuspid valve incompetence.

I Features SYMPTOMS IN VARICOSE VEINS

Dragging pain, postural discomfort Heaviness in the legs Night-time cramps-usually late night Oedema feet, itching (feature of CVI) Discolouration/ulceration in the feeVpainful walk CAUSES OF PAIN/CRAMPS IN VARICOSE VEINSNENOUS DISEASES Increased venous wall tension-chronic venous hypertension Hypoxia of tunica media of the venous wall due to altered function of vasa vasorum Increased capillary pressure Hyperviscosity of red cells-haemorrheological disorders Platelet hyperaggregation Reduction in capillary permeability causing capillary functional d;sorder Altered cutaneous microcirculation due to leucocyte adhesion and accumulation into the venous wall; release of free radicals cause microvascular lesional disease

B Can be localised or generalised Localised oedema is due to ankle flare or dilatation of medial marginal vein Cellulitis and lymphangitis association causes oedema Scarring and thickening of dermal and subdermal tissues-lipodermatosclerosis (brawny induration) Ankle becomes narrower due to contraction of skin and subcutaneous tissues but calf remains prominent- champagne bottle appearance Pale atrophic skin with white patches surrounded by dilated capillaries and pigmentation-atrophic blanche

-------'

It is more common in females (10:1). Often it is familial. Familial varicose veins begin in younger age group, seen bilaterally, involves all veins including deep veins.

I Signs

Brodie-Trendelenburg test: Vein is emptied by elevating the limb and a tourniquet is tied just below the saphenofemoral junction (or using thumb, saphenofemoral junction is occluded). Patient is asked to stand quickly. When tourniquet or thumb is released, rapid filling from above signifies saphenoemoral incompetence. This is Trendelenburg test I. In Trendelenburg test II, after stand ing tourniquet is not released. Filling of blood from below upwards rapidly can be observed within 30-60 seconds. It signifies perforator incompetence.

..... z C)

SF junction

II)

:::,

.... cu

II)

lo"

C: .... I.C II) ....

': "'ti

-f

m

....

::0

z

Cl (1)

::::,

(1) .... Cl.I

V'I

C: .... (1) ....

\Q

':': Fig. 1.411 : Venous haemodynamic mapping (VHM) of the lower limb.

::::, 0 C:

V,

To find out OVT-very important To find out saphenofemoral, saphenopopliteal incompetence To find out perforator incompetence Uniphasic signals signify flow in one direction-normal Biphasic flow signifies reversal flow with incompetence To find out thrombus/thrombophlebitis in superficial veins

Figs. 1.410A and 8: Duplex scan for venous diseases of lower limb

should also be done in standing position. Duplex scan: .,, It is a highly reliable UIS Doppler imaging technique (here high resolution B mode ultrasound imaging and Doppler ultrasound is used) which along with direct visualisation of veins, gives the functional and anatomical information, and also colour map. Examination is done in standing, lying down position and also with Valsalva manoeuvre. Hand-held Doppler probe is placed over the site and visualised for any block and reversal of flow. DVT is very well-identified by this method. ,. Venous haemodynamic mappingNHM/Cartography is essential prior to surgery. Note:

• Proper venous haemodynamic mapping (VHM) is essential. • Saphenous eye (Egyptian eye) at SFJ should be identified in duplex US. • Reflux is defined as retrograde blood flow in reverse direction lasting for more than 0.5 seconds. • 'Mickey mouse sign' is the duplex scan transverse 8 mode image showing great saphenous vein (GSV) and femoral vein medially and femoral artery laterally.

J

,,. Digitally coded free flow (B flow) USG (Professor Feder Lurie of Hawaii) allows simultaneous visualisation of flowing blood/blood cells and surrounding stationary structures to give proper haemodynamic imaging with functioning mechanism of venous valves, valve leaflets and flow across leaflets. This may be the ideal tool of investigation in future . Ultrasound abdomen, peripheral smear, platelet count, other relevant investigations are done depending on the cause of the varicose veins. If venous ulcer is present, then the discharge is collected for culture and sensitivity, biopsy from ulcer edge is taken to rule out Marjolin's ulcer. Plain X-ray of the part is taken to look for periostitis. IVUS (Intravenous ultrasound) is often used.

l

•·· Plethysmography: ,, It is a noninvasive method which measures volume changes in the leg. It gives tunctionafinformation on venous volume changes and calf muscle pump insufficiency. ,. Photoplethysmography: Using probe transmission of light through the skin, venous filling of the surface venules which reflects the superficial venous pressure is measured. Initially patient performs dorsiflexion at ankle for 1Otimes to empty the venules and pressure tracing falls in photoplethysmography. Patient takes rest

Everybody is in favour of progress. ft is the change they don't like.

0 iii' (1)

CI.J

V,

(1)

V,

226

and refilling occurs. In normal people, it occurs through arterial inflow in 20-30 seconds. In venous incompetence • Ascending phlebography defines obstruction filling also occurs by venous reflux and so refilling time Descending phlebography identifies valvular incompetence is faster than normal. j Regularly not required to be done Disadvantage: Site of reflux cannot be localised by this ~ R venography is new m...:.e_ th_od_ _ _ _ _ _ _ _ ___, method. ;.. Air plethysmography: Patient is initially in supine position Varicography: Here nonionic, iso-osmolar, nonthrombogenic contrast with veins emptied by elevation of leg. Air filled plastic is injected directly into the variceal vein to get a detailed pressure bladder (inflatable polyurethane cuff filled with anatomical mapping of the varicose veins. It is used in recurair) is placed on calf to detect volume changes. Minimum rent varicose veins. volume is recorded. Patient is turned to upright posi· tion and venous volume is assessed. Maximum venous volume divided by time required to ach ieve maximum venous volume gives the venous filling index (VF/). VFI Lymphoedema; AV malformation; Orthostatic oedema Renal, cardiac and hepatic diseases is a measure of reflux. Ejection fraction is volume change Vasculitis and metabolic diseases like gout, myxoedema, and measured prior and after single toetip manoeuvre which is morbid obesity a measure of calf pump action. Residual venous fraction Chronic infections like tuberculosis, syphilis _ _ _ ___, is an index of overall venous function which is venous volume in the leg after ten toetip manoeuvres divided by Treatment venous volume prior to manoeuvre. Increased VF/ and Conservative treatment: diminished ejection fraction in a patient will benefit from :.- Elastic crepe bandage application from below upwards or surgery. use of pressure stockings to the limb-pressure gradiant Ambulatory venous pressure (AVPJ: It is an invasive method. Needle inserted into dorsal vein of of 30-40 mm Hg is provided. foot and is connected to transducer to get its pressure which ,, Elevation of the limb-relieves oedema. Two short times, is equivalent to pressure in the deep veins of the calf. Ten during day and full night, elevation of foot with feet above tiptoe manoeuvres are done by the patient. With initial rise the level of heart and toes above the level of nose is the in pressure, pressure decreases and eventually stabilises method. with a balance. Pressure now is called as ambulatory venous ,,. Unna boots-provide nonelastic compression therapy. It pressure (AVP). After stopping exercise, veins are allowed comprises a gauze compression dressings that contain to refill with return of pressure to baseline. Time required zinc oxide, calamine, and glycerine that helps to prevent for pressure to return to 90% of baseline is called as venous further skin break down. It is changed once a week. refilling time (VRT). Raise in AVP signifies venous hyperten,, Pneumatic compression method-provide dynamic sion. Patients with AVP more than 80 mmHg has got 80% sequential compression. chances of venous ulcer formation. .,, These methods reduce the AVP, reduce transcapillary Arm-toot venous pressure: fluid leakage by increasing SC pressure and improve Foot pressure is not more than 4 mmHg above the arm prescutaneous microcirculation. sure. Drugs used for varicose veins: Venography: , Calcium dobesilate: 500 mg BO. Calcium dobesilate , Ascending venographywas very common investigation improves lymph flow; improves macrophage mediated done before Doppler period. proteolysis; and reduces oedema. A tourniquet is applied above the malleoli and vein of ,, Diosmin: It is micron ized purified flavanoid fraction. It dorsal venous arch of foot is cannulated. Water soluble dye protects venous wall and valve, and it is anti-inflammainjected, flows into the deep veins (because of the applied tory, profibrinolytic, anti-oedema, lymphotropic. Diosmin tourniquet). X-rays are taken below and above knee level. 450 mg + Hesperidin 50 mg (DAFLON 500 mg). Mainly Any block in deep veins, its extent, perforator status can used in relieving night cramps but not to improve healing be made out by this. of ulcers. It is a good reliable investigation for DVT. ,, Toxerutin 500 mg BO, TIO. Antierythrocyte aggregation If DVT is present, surgery or sclerotherapy are contrain· agent which improves capillary dynamics. dicated. Benzopyrones, saponins, plant extracts, Ruscus (venular , ,,. Descending venogram is done when ascending venogram a 1-adrenergic receptor partial agonist) coumarins are is not possible and also to visualise incompetent veins. different drugs used. Here contrast material is injected into the femoral vein Note: through a cannu la in standing position . X-ray pictures are taken to visualise deep veins and incompetent veins. Benefits of all these drugs are doubtful.

B

B

I

I

lnjection-sclerotherapy: ,. Fegan's technique: By injecting sclerosants into the vein, complete sclerosis of the venous walls can be achieved. Indications - Uncomplicated perforator incompetence. - In the management of smaller varices-reticular veins, thread veins (telangiectasis), Recurrent varices, Isolated varicosities. Aged/unfit patients.

- Advantages: Cheap, technically easy, easily available, OPD procedure, can be repeated many times, anaesthesia is not needed, can be used along with other procedures for varicose veins. US guided foam sclerotherapy (USFS) is the current standard. Complications: Headache, transient blindness, stroke, air embolism, thrombophlebitis, pain over injected site, pigmentation. - Contraindications: Peripheral arterial disease, DVT.

227

.... z

"....

ID :::, ID QI

VI C: IQ ID

.... ....

':':


. Qj

en ...

:::i V1

0

!ti :::i C: !ti

_.,, cc a:

V1

Microsclerotherapy: Very dilute solution of sclerosing agent like STDS (0.1% of 0.1 ml- dilute) Polidocanol is injected into the thread veins and reticu lar veins followed by app lication of compression bandage (30 G needle). Dermal flare will disappear well by this method . ;,.. Transillumination microsclerotherapy (vein-lite): It is better imaging of the veins using light gene rated by halogen bulb with high quality fibre illumination over the skin uniformly and passing 30 gauge needle for microsclerotherapy. Echosclerotherapy: Sclerot herapy is done under duplex ultrasound image guidance. Catheter directed sclerotherapy: It is devised at Miami vein clinic with specific catheter for sclerotherapy. This catheter has got side holes all around the specific length for uniform contact of venous wall with the foam. It also has got a balloon at the tip which after inflation blocks the SFJ thus preventing embolization of foam. It has got three external ports one for balloon inflation; one for bladder valve port; one for injection. This technique is under trial.

ITrifurcated hub I Balloon inflation port-~ Bladder valve port-

------=

c{]t==~=tt::;::==

Infusion port- - -

Current place of sclerotherapy is mainly for recurrent varicosities and threadltelangiectatic veins Sodium tetradecyl sulphate (STDS) is most common ly used sclerosant Hyperpigmentation is common after STDS Anaphylaxis is common after sodium morrhuate Anaphylaxis is least with polidocanol Extravasation (presence of pain/irritation/burning) should be avoided as it will cause skin necrosis Postsclerotherapy walking immediately after injection for 30 minutes with elastic bandage in place prevents/minimises the chances of DVT Compression bandage should be worn for minimum period of 6 weeks Sclerotherapy can be-macrosclerotherapy; microsclerotherapy; echo (ultrasound guided) sclerotherapy; foam sclerotherapy; transilluminated sclerothera..!p- "ti

-4

m ::c

.... z

Detachable handle

Cl

lb :::, lb

iiJ

Ill C 10 lb

...

/

...

ron heads and handle can be screwed to the both ends as needed

-:,;

:::, 0 C

Olive tips (knobs)

Ill

Handle

9.

Ill

lb

1),1 Ill

lb

Ill

Detachable tip

m Figs. 1.415 and B: Photo of Myer's stripper with Olive tips and also diagrammatic look of the same.

Figs. 1.417A to C: Saphenofemoral junction and its ligation. Tributaries are well seen .

• Antero-superior iliac spine

/ /-'---+- Inguinal ligament

Fig. 1.416: Incision for juxtafemoral flush ligation of long saphenous vein (Trendelenburg operation).

Figs. 1.418A and B

Know more than others; work more than others; expect less than others.

230 Stripper end {knob)

...

>,

QI

e'

:::,

VI

0

IIJ

:::,

C

IIJ

_.,,

- Stripping of the short saphenous vein is done from ankle below upwards after passing stripper from above downwards. It obliterates the mid calf perforator vein which is the common reason for recurrences. - Complications of stripping: Saphenous neuralgia (1-7%) due to saphenous nerve injury/avulsion; numbness and tingling along femoral nerve distribution; haematoma; infection; ulceration; recurrence of the disease is common (30%) which is not due to any technical default but due to progression of disease itself by neo-angiogenesis and re-vascularisation.

C0

Note:

VI

• Stripping of LSV up to knee level reduces the need for re-inteNention for recurrent varicose veins; stripping up to the ankle will not benefit additionally but may increase the chance of saphenous neNe injury. So stripping is not at all done in lower part of the leg for LSV. • Stripping of the vein is more effective than just ligation of the vein at the SFJ/SPJ. • Stripping of SSV reduces the recurrence but may damage sural neNe.

a:

Figs. 1.418C and D Figs. 1.41 BA to D: Long saphenous vein stripping. Note the stripped vein. Stripping is better than just ligation at the junction.

- 'Inverting or invaginating stripping' using rigid Oesch pin stripper is better as postoperative pain and haematoma is less common and also tissue damage. Vein should be very firmly fixed to the end of the stripper and pulled out to cause the inverting of the vein. - Stripping of short saphenous vein is more beneficial than just ligation at saphenopopliteal junction. It is done from above downwards using a rigid stripper to avoid injury to sural nerve. , Subfascia/ ligation of Cockett and Dodd (1938): - Perforators are marked out by Fegan's method. Perforators are ligated deep to the deep fascia th rough incisions in anteromed ial side of the leg. , Ligation of short saphenous vein at saphenopopliteal junction. It is done in prone position with horizontal incision. Variations in SP junction are common. But stripping is better. ,., Linton's vertical approach (1938)-subfascial ligation of perforators.

junction. Anterior accessory saphenous vein (AASV) and posteromedial thigh vein are also often seen distally as tributaries. , Stripping of vein - Using Myer's stripper vein is stripped off. Stripping from below upwards is technically easier. Immediate application of crepe bandage reduces the chance of bleeding and haematoma formation. Stripping avulses the vein as well as obliterates thetributaries. Babcock's stripper and rigid metal pin stripper can also be used. - Two methods of stripping are used: (1) Extraluminal collision technique using Myer's stripper (Acorn head stripper, 73 cm long) is practiced since long time; but it damages the adjacent tissue, causes infection, postoperative pain, discomfort and haematoma along the stripped tract with possibility of revascularisation of the tract haematoma. (2) lnvagination technique is Figs. 1.419A and B: A. Cockett and Dood subfascial ligation of better with less damage to adjacent tissue. Codman's perforators using multiple small horizontal incisions. 8. Linton's vertical approach for subfascial perforator ligation in the leg. stripper is used for invagination technique.

"

Stab avu/sion of varicose vein and pertorators: Avulsion is done using mosquito forceps or avulsion hooks-hook phlebectomy. It is popular method, also used along with other minimal invasive techniques like EVLA, RFA. Multiple incisions are made and veins are carefully and gently avulsed/teased to clear it. Postoperative compression bandage is a must.

Fig. 1.420: Typical perforator ligation.

Figs. 1.421A and B: Avulsion of the per/orator using hook or mosquito forceps through a small skin incision-stab avulsion.

Minimally invasive methods: a. Subfascial endoscopic perforator ligation surgery (SEPS) Through working channel clip applicator or diathermy instrument 1s passed

may be connected to a monitor Incision for SEPS

Fig. 1.422: Subfascial endoscopic per/orator ligation surgery (SEPS).

- A special telescope is introduced deep to deep fascia through a single small vertical incision at proximal leg selecting healthy skin. Potential space between muscle and deep fascia with loose areolar tissue is easy to dissect using endoscope. Technique is done under tourniquet 300 mmHg pressure. Endoscope is advanced down along the medial border of the tibia. Perforators travelling in subfascial plane are identified and fulgurated using bipolar cautery or clips can be applied into the perforators. It is recommended in chronic venous insufficiency (CVI). But its limitation is difficulty in getting 'lift off' skin in cases with severe lipodermatosclerosis to identify the perforators. b. Radiofrequency ablation (RFA) method (VNUS closure method) (VNUS medical technologies Inc; Sunnyvale, CA, USA) (by Goldman 2000): This procedure is done under general or regional anaesthesia. A RFA catheter is passed into long/ short saphenous vein near saphenofemoral or saphenopopliteal junction under guidance. 85°C temperature is used for longer period of time to cause endothelial damage, collagen denaturation and venous constriction. 7 French catheter is used and 7 cm segment vein is ablated sequentially. Phlebectomy is done while withdrawing the catheter. Wall of the vein is destroyed through its full thickness. Vein forms a cord, which gets dissolved by macrophages and immune cells. CELON RF/TT-Radio Frequency Induced Thermal Therapy (2007) is newer type. c. Trivex method". Under subcutaneous illumination and local anaesthesia, a large quantity of fluid is injected percutaneously to identify the superficial veins under. Tumescent anaesthesia created causes hydrodissection. Trivex resector and illuminator are placed under the skin. Resector gently extracts veins by suction and morcellation. Further stages of tumescence flushes all blood and delivers vasoconstriction solutions. Solution is passed through 18 gauze needle to clear all blood underneath. Method is cosmetically acceptable; removes all sized veins; achieves good pain relief; with minimal complications like bruising, induration which gets resorbed eventually; and can be used when there are venous ulcers. d. Endovenous laser ablation (EVLA): It is done as an outpatient procedure or as day-care surgery. Patient lies supine with diseased leg flexed, hip externally rotated and knee flexed. With aseptic precaution, under U/S guidance LSV is cannulated guide wire is passed beyond SFJ and 5- French catheter is passed over guide wire and tip is placed 1 cm distal to the junction. 200 ml of 0.1 % lignocaine (crystalloid with local anaesthetic) is infiltrated along the length of the LSV. Laser flexible glass fibre is inserted up to the tip of the catheter and catheter is withdrawn for 2 cm and laser fibre protrudes

A man without purpose is like a ship without rudder.

231

.....

z

"::s It)

It)

ii1 V'l

C

IQ

...

It)

-:-;

::s 0

C

"' 0

iii'

It)

QI

"' It)

"'

232

for 2 cm. Laser fibre is fired step by step using diode laser (810-1470 nm diode laser energy), 1 mm withdrawal in 2 seconds. Once procedure is over catheter is removed and pressure bandage is applied for 2 weeks. Heat produced (729-1000°c at tip) by the laser produces steam bubbles with thermal damage of endothelium leading into occlusion of the vein. Laser energy acts on the blood within the vein rather directly through the wall and heats the blood and in turn heats the vein wall. Drawback of laser therapy is inability to create flush occlusion allowing tributaries to open up to cause possible recurrence. Complications of EVLA: Pain; ecchymosis, haematoma, skin burns, difficulty in cannulating the unsuitable vein if selected; DVT; sensory disturbances, infection.

e. Other methods: - Transilluminated phlebectomy is done by passing transilluminating light under the skin and passing a rotating blade through another small incision. Veins are grasped and removed by rotating movements. Ambulatory phlebectomy is done through tiny small incisions using special phlebectomy instruments. Electrodessication using weak electric current through a fine needle directly into the spider veins (telangiectasis) is also used. Endovenous glue (cyanoacrylate-0.1 ml) injection through a guided venous catheter to seal the lumen.

B Infection- most common complication Haematoma formation, bruising, oedema limb Nerve injury is the most common serious complication: - Saphenous nerve injury in LSV stripping is 1-7% - Sural nerve injury in SSV stripping is 20% - Common peroneal nerve injury in SSV stripping is 4% Recurrence of varicose veins-20-30%. - Causes are-angiogenesis and revascularisation; improper surgery; reflux in residual axial vein; new reflux; axial vein recanalisation - Recurrence is more with SSV disease than LSV; in obese patient - Complications are high in surgeries for recurrent varicose veins-40% DVT is rare complication -

-0

-t m

....::x, z

Cl

11)

::::,

...

11) Ql

VI C: I.C

... ... ':":

Fig. 1.424: Venous ulcers around both ankles (bilateral).

rational which is due to hydrostatic pressure by weight of blood column from the right atrium (hydrostatic reflux) which is maximum at foot and ankle OR dynamic which is due to muscular contraction across the incompetent perforator with a high pressure up to 200 mm Hg (hydrodynamic reflux). There is a peculiar recycling of blood from deep femoral vein spillage of blood across incompetent SFJ into LSV/GSV passage of same blood across perforators into the deep veins to reach femoral vein again to enter the LSV as spillage. White cell trapping' theory and 'fibrin cuff' theory; release of free radicals; increased matrix metalloproteinases (MMPs); abnormal fibroblast activity; inhibition of growth factors; are other causes of venous ulcer formation. Area where venous ulcer commonly develops, is around and above the medial mal/eoli because of presence of large number of perforators which transmit pressure changes directly into superficial system. This area is called as Gaiter's zone. It can also be on both malleoli. Ulcer is often large, nonhealing, tender, recurrent with secondary infection. Vertical group of inguinal lymph nodes are usually enlarged and tender. Often it leads to scarring, ankylosis, Marjolin's ulcer formation. Slough from the ulcer bed may give way causing venous haemorrhage. Periostitis is common which also prevents ulcer from healing. Most of the venous ulcers have surrounding lipodermatosclerosis. Lipodermatosis is chronic inflammation, fibrosis, thickening, induration of the skin and subcutaneous tissue of the lower leg (calf) and ankle with pigmentation and a tight contracted woody leg often with Achilles tendon contracture. It suggests severe chronic venous disease.

11)

Fig. 1.425: Gaiter's zone. It is handbreadth area around malleoli where complications of venous disease occurs. Word gaiter (French) is a leather/cloth covering for lower leg and ankle.

•

Fig. 1.426: Champagne bottle sign/inverted beer bottle sign is seen in lipodermatosclerosis due to prominent calf with narrow ankle, contracted skin and subcutaneous tissue. Sign is often observed in DVT also.

Fig. 1.427: Marjolin's ulcer in a chronic venous ulcer.

Due to regular walking on toes so as to relieve the pain causes contraction and extra-articular fibrosis of achilles tendon. Proper exercise is the remedy for-talipes equinovarus. Note:

70-80% of leg ulcers are venous ulcers.

Strong convictions precede great actions.

::::, 0 C:

"' 0 "' 11) Ql "' 11)

"'

234

Differential diagnosis: lschaemic ulcer, diabetic ulcer; Rheumatoid ulcer; Traumatic ulcer; Neuropathic ulcer; Neoplastic ulcer. Investigations ,. Discharge from the ulcer for culture and sensitivity. ,. X-ray of the area to look for periostitis. , Wedge biopsy from the ulcer edge to rule out Marjolin's ulcer. ,. Investigations to rule out other causes of leg ulcers like arterial; neurological; diabetes; sickle cell disease and other haemolytic diseases. ,. Erythrocyte sedimentation rate; C-reactive protei n, peripheral smear; red cell counts. ,. Doppler-venous and often arterial. Treatment , Bisgaard method of treating venous ulcer: - Measures to reduce oedema, increase venous drainage, so as to promote ulcer healing. - Elevation. - Massage of the indurated area and whole calf. - Passive and active exercise. ,. Care of ulcer by regular cleaning with povidone iodine, H2O2. Dressing with EUSOL. ,. Four layer bandage (45 mmHg pressure) technique to achieve high compression pressure. It is changed once a week. ,. Antibiotics depending on culture and sensitivity of the discharge. ,. Once ulcer bed granulates well, split skin graft (SSG) is placed (Thiersch Graft), or pinch graft. , Specific treatment tor varicose veins should be undertaken-Trendelenburg operation, stripping of veins, perforator ligation.

Fig. 1.428: Skin graft is done over a venous ulcer once ulcer has shown healthy granulation tissue. COMPLICATIONS OF VENOUS ULCERS Haemorrhage Marjolin's ulcer Infection Talipes equinovarus

Periostitis is common over the tibia Disability Calcification

Note-; • Present concept is to treat the ulcer first by compression bandage; regular dressing; skin grafting. Once ulcer has healed definitive procedure for varicose veins is done. Studies show that rapidity of healing of ulcer perse is not dependent on the surgery for varicose veins. • Recurrence rate of venous ulcer after proper therapy is 30%. Reulcer formation is more in post-phlebitic/ thrombotic limb. • EUSOL is Edinburgh University solution of lime containing boric acid, sodium hypochlorite, calcium hydroxide.

COMPRESSION THERAPY FOR VARICOSE VEINS

Charing-Cross (hospital London) elastic multilayered compression bandage, once a week Low compression bandaging Graduated elastic compression stockings with a leak proof absorbent dressing beneath Unna boots Crepe bandage/stockings

Compression reduces the venous wall tension; prevents reflux; controls the venous over-distension. Compression diverts the blood towards deep veins through perforating veins; prevents the outward flow of blood in perforator incompetence; improves the efficacy of calf muscle pump. Compression reduces the oedema and improves the venous and lymphatic drainage; improves venous elasticity; improves the microcirculation and more important is it prevents further damage of the venous wall. Compression may be elastic/inelastic/combination of elastic and inelastic (Unna boot)/multilayered (four layered) compression system which can provide sustained high compression for several days-usually up to a week/intermittent pneumatic compression. Unna boot is three-layered paste gauze compression dressing containing calamine, zinc

Fifty percent of venous ulcer occurs as a result of recanalisation of DVT, and the leg is commonly called as postphlebilic limb (leg). It presents with all complications of venous diseases like eczema, ulceration, lipodermatosclerosis and venous ulcers. Here surgery for superficial varicose veins are contraindicated. Venous valve repair (Kistner's valvuloplasty) or valve transplantation or drugs like Stanazolol, which reduces the fibrous tissue thereby increasing the oxygenation are beneficial.

has mainly inelastic inner component with partly elastic outer layer wrap. Recommended pressure in mild varicose veins, pregnancy and postoperative period is 20 mmHg; in symptomatic varicose veins and after sclerotherapy is 30 mmHg; in venous ulcer and post-phlebitic leg it is 4G-45 mm Hg; in phlebolymphoedema (venous oedema with lymphatic oedema which is more dangerous) it is more than 45 mm Hg. Skin maceration, excoriation, dryness, infection, ulceration and failure are the complications of compression therapy.

DIFFERENT BANDAGES USED ARE:

- - -- - - -

oxide, glycerin, sorbitol, gelatin and aluminium silicate which

Fig. 1.429: Compression stockings should be worn in varicose vein disease even after intervention to reduce the chances of recurrence.

c. Spontaneous: Polycythaemia vera, polyarteritis, Buerger's disease. d. Thrombophlebitis migrans (Trousseau's sign, 1876): It is spontaneous migrating thrombophlebitis seen in visceral malignancy like pancreas, stomach. e. Mondor's disease. It can be-SVT with varicose veins (V-SVT) or SVT without varicose veins (NV-SVT). It can also be- primary or secondary. Duplex ultrasound Doppler of both limbs is a must.

235

....

z

G) IT>

:::,

...

IT> QI

I

• Compression improves ulcer healing rate Supplementation of phlebotonic drugs in conjunction with compression therapy is accepted Inelastic compression (50% stretch) is more commonly used because it is better accepted Multilayered bandage system is most effective • Ankle-arm pressure index less than 0.6 is contraindication for compression therapy as it may precipitate ischaemic ulcer formation Exercise may improve the muscle-pump action

VI

... ... '


Fig. 1.430: Superficial thrombophlebitis with clot and infection.

I Features Pain, redness, tenderness, cord like thickening of veins, fever. It can be seen either in upper limb or lower limb. Complications: Destruction of venous valves resulting in varicose veins. DVT, embolism, infection. Treatment ,, Elevation. ,, Anti-inflammatory drugs, antibiotics. , Application of crepe bandage-compression therapy. ,, AnticoagulatiorrLMWH for SVT >3 cm in length.

B British standard Class I: 14-17 mmHg Class II: 17-24 mmHg Class Ill: 24-35 mmHg

International (European) standard Class I: 20- 30 mmHg Class II: 30-40 mmHg Class Ill: 40-50 mmHg Class IV: 50-60 mmHg

KLIPPEL-TRENAUNAY SYNDROME

COMPRESSION BANDAGES Type I: Light weight confirming stretch bandages. These comprise light weight elastomer with high elasticity but little power. It is used to retain dressings Type II (short stop): Light support bandages. Minimal stretch. Exhibit limited elasticity but tends to lock out on minimal extension. In ambulant patient CVI they form an essentially inelastic covering to the leg which will exert pressure during calf systole but not during diastole. They are unsuitable for control of oedema • Type Ill (Long stop): These are extensible elastic and powerful to a varying degree

•

j

THROMBOPHLEBITIS

It is a nonfamilial mesodermal anomaly with skin naevus, varicose veins, soft tissue and bone hypertrophy. Deep veins are often aplastic. It is usual ly managed with compression bandages. If patient is undergoing surgery for some other condition, then LMWH should be started. Condition itself occasionally can be treated with EVLA for varicose veins if only deep veins are normal; bone length discrepancy correction of leg is done. Parkes-Weber syndrome is a differential diagnosis. PW synd rome presents with varicose veins, multiple AV fi stulas, chronic venous hypertension, high output cardiac failure and ulceration.

ANTICOAGULANTS

It is the inflammation of veins, usually of superficial, veins due to different causes. It is actually superficial vein thrombosis (SVT) with inflammation (slight).

I Types a. Acute: Due to IV cannulation, trauma, minor infections, hypercoagulability. b. Recu rrent.

These are the agents used to prevent and treat thrombosis and thromboembolic events. It may be used in arterial thrombus (white in colour) like in myocardial infarction, stroke, ischaemia of different parts; or in venous thrombosis of limb veins or pelvic veins or mesenteric veins or neck veins. Embolism is the eventual problem along with perfusion changes. Thromboem bolism is a major problem in venous thrombosis. Anticoagulants may be in vivo-parenteral (heparin, LMWH,

Everything has its beauty but not everyone sees it.

:::, 0 C: VI

VI

IT> QI VI

IT> VI

236

...>... :, (II

C'I

Vl

0

Rl

:, C

Rl

_,.,,

co a: Vl

fondaparinux) or oral (dicumarol, warfarin, rivaroxaban); in vitro-heparin , sodium citrate, sodium oxalate and EDTA (Ethylene diamine tetra acetic acid). Contraindications for anticoagulant therapy. Ongoing bleeding; recent surgery/invasive procedure; severe trauma; bleeding tendency (clotting factor deficiency); intracranial haemorrhage; pericarditis/pericardial effusion; patient prone to fall.

I Heparin (Unfractionated/UFH) It is a natural anticoagulant, a mucopolysaccharide. It activates plasma antithrombin Ill and so blocks extrinsic pathway. It has got antiplatelet action. It prevents clotting of blood both in vivo and in vitro by acting on all three stages of coagulation. It prolongs clotting time and activated thromboplastin time in specific (by 1.5-2.0 times the control). Heparin also causes hyperkalaemia, thrombocytopenia and osteoporosis. Commercial heparin is derived from lung and intestinal mucosa of pigs and cattle. The onset of action is immediate after administration lasting for 4 hours. It is metabolised in the liver by heparinase. It does not cross placental barrier and is not secreted in breast milk. Indications: As prophylaxis in major surgeries, postoperative period, puerperium; as therapy in DVT; in vascular diseases. Dose: For prophylaxis - 5,000 units/subcutaneously 8th hourly. For therapy - 10,000 units/IV 6th or 8th hourly; later changed to subcutaneous dose. In severe cases, 5,000 units to 20,000 units is given daily through IV infusion at a rate of 1,000 units per hour. Daily dose should not exceed 25,000 units. Heparin should not be given intramuscularly and it is better not to combine with penicillins, hydrocortisone. Heparin is not given orally. Heparin administration should always be monitored with APTT. Complications: Allergy, bleeding, thrombocytopenia, alopecia, osteoporosis. Contraindications: Bleeding disorders, severe hypertension, GIT ulcer, piles, malignancy, ocular and neurosurgery, chronic alcoholism, cirrhosis, etc. Note: • Danaparoid is an antifactor Xa, heparinoid is an anticoagulant used in patients where heparin is contraindicated. • Heparin antagonist: 50 mg of 1% protamine sulphate solution is given slow intravenous. 1 g reverses 100 units of heparin. It is given only after doing activated thromboplastin time. Overdosing or infusion without indication may itself precipitate bleeding.

I Low Molecular Weight Heparin (LMWH) It is a commercially prepared heparin with a molecular weight of 4,000 to 6,500. It acts by inhibiting factor Xa. It shows lesser antiplatelet action and lower incidence of haemorrhagic complications. It has got better bioavailability on subcutaneous administration (once daily).

Drugs are - Enoxaparin; Dalteparin; Parnaparin; Reviparin; Fraxiparine. It is used as subcutaneous injection. Uses: (1) Prophylaxis of DVT and Pulmonary embolism in surgery, stroke and immobilized patients (2) DVT (3) myocardial infarction (4) Rheumatic heart disease (5) Haemodialysis patients. Advantages: It has got longer du ration of action once a day; has better anticoagulant effect; less interaction with platelets; less antigenic; usage is easier and more acceptable; monitoring is not necessary. Disadvantages: They are expensive; only partially reversible by protamine sulphate; it is monitored by anti-Xa assay which is not freely available.

I Fondaparinux It is a synthetic pentasaccharide factor Xa inhibitor. It acts by binding antithrombin Ill. Fondaparinux (Arixtra) is injected subcutaneously into the abdominal wall as once daily dose (2.5 to 1O mg). It is used for initial treatment of deep vein thrombosis (DVT) and pulmonary embolism (PE) and for prevention of venous thromboembolism in patients undergoing surgery for hip fracture or hip/knee replacement.

ORAL ANTICOAGULANTS They are given orally and are slow-acting.

I Types Coumarin derivatives: Bishydroxycoumarin (Dicoumarol): First coumarin drug derived from sweet clover. Warfarin sodium: Most common oral anticoagulant used. lndandione derivative: Phenindione, anisindione.

I Mode of Action of Oral Anticoagulant Therapy By suppressing synthesis of prothrombin, factors VII, IX and X. By inhibiting vitamin K mediated carboxylation of glutamic acid. Oral anticoagulant does not have in vitro action. They are slow-acting, and long-acting. Control of oral anticoagulant therapy is by monitoring prothrombin time. PT comes to normal only 7 days after cessation of the drug. They cross placental barrier and are known to cause teratogenicity when given in 1st trimester. They are secreted in breast milk.

I Indications In DVT after cessation of heparin for maintenance therapy. After valve replacement surgery. To achieve adequate anticoagulant effect and to prevent thromboembolic episodes the INR has to be maintained within 2- 3.

I Side Effects

Direct factor Xa inhibitors , Rivaroxabarr-lt is given orally once or twice daily as 15-20 mg dose per day. It is given initially 15 mg twice daily, later 20 mg once daily. It shows rapid bioavailability;

Bleeding-it may require blood transfusion/FFP or vitamin K injection intramuscular or oral to control. Cutaneous gangrene. shows rapid onset of action; coagulation monitoring is Fetal haemorrhage and teratogenicity. not required. Apixaban (2.5 mg) and edoxaban are other Alopecia, urticaria, dermatitis. drugs. Drug interactions: with NSAIDs, cimetidine, omeprazole, Contraindications for NOACs: Renal impairment; disorders metronidazole, cotrimoxazole, erythromycins, barbiturates, of haemostasis; active bleeding; prosthetic heart valve; liver rifampicin, griseofulvin. disease; pregnant and breastfeeding women; children less than 18 years.

I WARFARIN

WAR FARIN ( Wisconsin Alumn i Research f oundation + coumARIN) derivative) SODIUM is the most common drug used. It has got lesser side effects. It has got cumulative action and so given in tapering dose. Dose is 5 mg, once a day. It should be discontinued 7 days before any surgery like tooth extraction and prothrombin time should return to normal level. During surgery, if excess bleeding occurs, fresh frozen plasma may be given. Theeffects of warfarin sodium is reversed by injection vitamin K; the dose depends on INR and emergency of reversal (takes 24 times to reverse).

B • Streptokinase; urokinase; anistreplase • Altepase - Recombinant tissue plasminogen activator (rtPA); half life is 5 minutes; given as 10 mg IV bolus followed by 90 mg infusion in 90 minutes. Tenecteplase: genetically engineered, higher fibrin selectivity.

T

Dillerences between oral anticoagulants and heparin Oral anticoagulant Heparin

Monitored by

Slow-acting

Immediate

Long-acting

Short-acting

Only in vivo action

Both in vitro and in vivo action

Prothrombin time

Partial thromboplastin time

Crosses the placental barrier

Does not cross the placenta

Secreted in milk

Not secreted in milk

Administration Orally

Intravenously/subcutaneously

Non-vitamin K antagonist oral anticoagulants (NOACs) Direct thrombin inhibitors ,. Recombinant hirudin and hirudin analogues- derived from leeches, are direct inhibitors of thrombin. , Synthetic direct thrombin inhibitors (factor Ila) -Argatroban, dabigatran etixilate (Pradaxa 110 mg).

Note: ldarucizumab (Praxbind) is used to reverse the anticoagulant effects of

dabigatran. Reversal agents for apixaban and rivaroxaban are currently not available.

I Anti platelet Drugs Small dose aspirin-inhibits platelet synthesis of thrombaxane A2.

Ticlopidine (125 mg BD)-alters platelet membrane, thereby platelet aggregation. Clopidogrel-action similar to ticlopidine. Dextran-decreases platelet aggregation. Abciximab-glycoprotein Ilb/IIla inhibitors, block platelet aggregation, and platelet adhesion to fibrin. Dipyridamole-xanthine oxidase inhibitor.

PULMONARY EMBOLISM It is commonly due to lower limb DVT (15% of lower limb DVT). It can also occur after pelvic vein DVT or upper limb DVT (30% of upper limb DVT). Chest pain, cough, haemoptysis, dyspnoea are the features. Often site of DVT may be asymptomatic. When symptomatic, fever, pain, tense, tender calf, with positive Haman's sign may be evident. Massive embolism causes sudden cardiac arrest and death due to pulmonary artery block. Moderate embolism causes pyramidal wedge-shaped infarcts in lungs. Duplex scan of limb, CT angiogram of thorax, pulmonary angiogram (gold standard), X-ray chest, ventilation perfusion scan, ECG, echocardiography are useful investigations. Treatment is thrombolysis, heparin/LMWH, compression bandage. Occasionally surgical removal of clot from pulmonary artery is done if possible. IVG filter placement is essential in recurrent DVT with anticoagulation, DVT with contraindication for anticoagulation, pulmonary hypertension. Greenfield IVG filter is ideal with 95% patency rate. Complications are-bleeding, haematoma, migration of filter into pulmonary artery, thrombosis at filter level, IVC perforation. Retrievable IVG filters are newer method used to prevent longterm filter complications. It is used in young patients who

Attitudes are more important than facts.

237

..... z C) 11)

::,

... 11) QI

... ... '
""0

Para-aortic

nodes

Mesentenc--.::t- -- - ---•~•nodes

Ovaries

Fig. 1.457: Staging laparotomy for Hodgkin's lymphoma. Needle and wedge biopsies from liver/nodal biopsies from para-aortic, celiac, mesenteric, iliac nodes/splenectomy/ovarian translocation/iliac crest biopsy are the components of staging laparotomy. This can be very well-achieved through laparoscopy also now. Staging laparotomy is not commonly done now.

biopsy. Laparoscopyand biopsy of different abdominal lymph nodes is also a good option. Lower limb lymphangiographyto look for the pelvic and retroperitoneal lymph nodes. It shows reticular pattern in node; it can be used to assess the therapeutic response and prognosis. Lymphoscintigraphy is better and acceptable. It is prognostic tool also. Staging laparotomy The abdomen is opened. Splenectomy is done mainly to remove the tumour bulk, as spleen is commonly involved and also to avoid irradiation to splenic area which often causes unpleasant pulmonary fibrosis. Biopsies are taken from both lobes of the liver (needle biopsy) from para-aortic, celiac mesenteric, iliac nodes. In females, ovaries are fixed behind the uterus to prevent radiation oophoritis (oophoropexy/ ovarian translocation). Staging laparotomy is not routinely done now. It is done only if it benefits the patient to have better plan of treatment or better result. It is done in stage 1/IIA lymphoma (HL) in selected patients. Note:

Staging laparotomy, splenectomy (requires for immune function), lymphangiogram, IVU are no longer/very rarely done now for HL. Figs. 1.456A and B: Mediastinal nodes involved in lymphoma as seen in chest X-ray and chest CT scan.

MRI and PET scanare very useful to identify extranodal tissue involvement. Gallium scan is less useful.

Bone marrow biopsy or aspiration to stage the disease and to see the response to treatment. Usually iliac crest biopsy is done under local anaesthesia. It gives the staging. Chamberlain's mediastinoscopy and biopsy of mediastinal lymph node is done if peripheral nodes are not available for

B

PROBLEMS IN HL

Pleural effusion-respiratory discomfort SVC obstruction Spine if involved-not very common but can occur Opportunistic infection-mycobacteria, cytomegalovirus, herpes zoster Bronchopneumonia, septicaemia lmmunosuppression and its effects Risk for other malignancies in later life

Man cannot discover new oceans unless he has courage to lose sight of the shore-Anonymous

-f

m

....

:::i::,

0

252

...>...::::, °' (11

Ill

0

n:I

::::, C

n:I

_.,,

cc a:

Ill

T

Dillerences between HL and NHL

Age: Pattern of involvement: Cervical lymph node: Splenomegaly: Peripheral lymph node involvement (e.g. epitrochlear nodes) Treatment:

Prognosis:

HL (more common) Young and elderly

NHL Middle age and elderly

Symmetrical and consecutive Commonly involved

Asymmetrical Any group can be involved Not common Common

Common Not common

I Treatment for Relapses Mainly radiotherapy Chemotherapy (ABVD regime) Better

Mainly chemotherapy

Poor

I Treatment for HL :

Stage I and 11: ,, Mainly radiotherap~ external high cobalt RT. Above the diaphragm-" Y" field therapy, covering cervical, axillary, mediastinal lymph nodes. It may cause carcinoma of breast. Below the diaphragm, mantle or inverted "Y" field therapy, covering para-aortic and iliac nodes. It may cause infertility. ,, Chemotherapy is also given. Stage Ill and IV: Mainly chemotherapy.

Autologous stem cell transplantation. High dose chemotherapy. High dose chemotherapy with autologous stem cell transplantation. MOPP/ABV hybrid regime. Single dose vinorelbine (new vinca alkaloid); gemcitabine; immunotherapy; tumour vaccination; gene therapy.

Prognosis

TREATMENT STRATEGY FOR HL

Stage I and 11-80%. Stage Ill A-70%. Stage Ill Band stage IV-

m

::i::,

..... 0

Cl

ID

::::,

... ID QI

Vl C

... ...

IQ ID

Note: • Diffuse large B cell (DLBCL) type is the most common type of NHL (3D-40% of cases); 50% of patients have organ involvement at the time of diagnosis. Subtypes are-Primary mediastinal Bcell lymphoma (PMBL); Primary CNS lymphoma; EBV-positiveDLBCL; T-cell/histiocyterich large B cell lymphoma, etc.

• High grade

T

253

B

R- Maxi - CHOP is used in Mantle cell lymphoma as 21 days interval regime alternating with R-HDAC (Rituximab with high dose cytarabine). In other NHL excluding aggressive forms, R-CHOP standard dose is first line therapy.

Laugh at obstacles and cry at your success.

3

-0

':J" QI

~n "'

254

•

Bulky disease is defines as-Maximal diameter >7 cm in transverse or coronal planes (MSKCC).

•

International Prognostic Index: Based on 5 variables-(1) Age >60 years; (2) Stage Ill, IV; (3) Performance status: ECOG ~2; (4) Number of extranodal sites "'O

m

....

:x,

0

Fig. 1.475: Squamous cell carcinoma scalp. Note the everted edge. It is ulceroproliferative lesion.

Fig. 1.473: Soft tissue sarcoma thigh. Note the dilated vessels on the surface.

B Xeroderma pigmentosa Ataxia telangiectasia Fanconi anaemia, Bloom syndrome

_J

It is more common in elderly. But it is variable. Tumour suppressor gene p53 plays important role in prevention of the cancers. Its function is prevention of replication of damaged DNA. If damaged DNA replicates there is high chances of abnormal mitotic activity and cancer transformation of the tissue. Loss or reduction of ability/function of p53 suppressor gene can lead into cancers. Common cancers associated with p53 loss are cancers of breast, colorectum, retina, bone, brain, soft tissues, blood and familial related type. Fig. 1.474: Carcinoma heel region. Note the everted edge with proliferative cauliflower like look.

DYSPLASIA It means "disordered growth". There is loss in the uniformity of the cells with pleomorphism and hyperchromatism, as well as loss in their architectural orientation.

CARCINOMA IN SITU Here dysplasia involves the entire thickness of the epithelium, and is preinvasive. Basement membrane is intact in carcinoma in situ.

AETIOLOGIC FACTORS

I Age

B

ACQUIRED CAUSES

Chronic atrophic gastritis Solar keratosis Leucoplakia of oral cavity Ulcerative colitis

B Alkylating agents Hydrocarbons Smoking-lung, aerodigestive system, bladder cancer Asbestos-lung cancer Alcohol- liver cancer Amides, Az.o dyes-bladder cancer Aflatoxin B1 Arecoline, collagenases and tannins (present in Betel nuts) Nitrosoamines, vinyl chloride, insecticides

B

B Familial: Familial polyposis of colon MEN syndrome Neurofibromatosis von-Hippel-Lindau syndrome Familial breast and ovarian cancers

J

Japan-Carcinoma stomach China, France-carcinoma oesophagus Hong Kong-Nasopharyngeal carcinoma Australia-Melanoma India-Carcinoma oral cavity, gallbladder carcinoma New Zealand- Small bowel tumours

We travel not to find ourselves in the world, but to remember that we have a world inside us.

268

,

RADIATION CARCINOGENS

UV rays, ionising radiation.

...>. C1I ...:::,0\

B

-

MICROBIAL CARCINOGENS

Human papilloma virus-carcinoma cervix Epstein-Barr virus-Burkitt's lymphoma, nasopharyngeal carcinoma Hepatitis 'B' virus-liver cancer Human T cell leukaemia virus Type I Helicobacter pylori can cause carcinoma of stomach and is associated with lymphomas [Mucosa Associated with Lymphoid Tissue (MALT)J

V,

0

n:I

:::, C n:I

,Vl

ca

0::

_J

V,

SPREAD OF MALIGNANT TUMOURS 1. Local spread: Into adjacent structures like soft tissues, vessels, bone. 2. Lymphatic spread , By permeation: Here malignant cells proliferate through lymphatic vessels up to lymph node level. For example, in carcinoma breast malignant cells permeate into axillary lymph nodes. , By embolisation: Here cells get dislodged from lymphatic vessels and freely travel to spread into further level of lymph nodes. In carcinoma breast spread occurs from axillary lymph node to supraclavicular lymph node by embolisation. , Retrograde lymphatic spread occurs once lymph vessel get blocked by malignant infiltration. In carcinoma breast retrograde spread occurs to opposite breast, opposite axilla, or to mediastinum. In melanoma, through dermal lymphatics and retrograde spread 'in transit nodules' occur in the skin. 3. Blood spread , Occurs through veins, as veins are thin-walled and infiltration is easier (arteries contain elastic fibres in their wall, which resist malignant infiltration). , Blood spread is commonly to lungs, bone (upper end of femur and humerus, ribs, skull), liver, brain, adrenals and other organs. Both by permeation (e.g. in renal cell carcinoma permeation through renal vein is common) and by embolisation (in other malignancies). In carcinoma prostate, due to increased pressure and venous blockade, retrograde venous spread occurs through vertebral venous plexus which causes osteoblastic secondaries in pelvic bones and vertebrae. 4. Seedling: For example , From lower lip cancer to upper lip as kiss cancer.

B

KISS CANCER

Lip; Bladder; Cervix; Vocal cord; Vulva

Recurrence in the scar after surgery for malignancy, e.g. deposition of malignancy in the scar of SPC from bladder tumour. ,.. Seedling in the peritoneal cavity from abdominal malignancy is common causing intractable ascites. 5. Transcoelomic spread: Spillage or dislodge of malignant cells occurs from primary site resulting in seedling on other organ, e.g. in carcinoma stomach secondaries in ovary (Krukenberg tumour) occurs due to transcoelomic spread. Here cells get deposited on the raw su rface of ovary during ovulation (So it occurs in menstruating age group only) .

GRADING OF TUMOUR It signifies aggressiveness of tumour. It is based on differentiation of tumour cells and mitotic activity. Broder's grading of squamous cell carcinoma: Based on keratin/ epithelial pearls Grade I: Well differentiated->75% epithelial pearls Grade II: Moderately differentiated-50-75% epithelial pearls Grade Ill: Poorly differentiated- 25-50% epithelial pearls Grade IV: ""O

-t m ::c

0 Fig. 1.484: Soft tissue tumour of buttock. It could be sarcoma.

G) (I)

::, (I)

.....

11,J

Vl

C: .....

IC (I)

.....

'


m ::c

....

:c

V,

C:

10 ID

...

':c;

V,

C.

::,

3 0

...C:

"'

278

...>. QI

e"

::I

V,

0

ta

::I C: Ill

_.,, a:,

cc:

Fig. 1.504: Malignant skin tumour with ulceration of the skin over chest wall. Axillary nodes were enlarged in this patient.

V,

Fig. 1.501: Skin adnexal tumour (benign) in the face.

DERMATOFIBROMA (Sclerosing Angioma or Subepithelial Benign Nodular Fibrosis) It is a benign tumour arising from skin from dermal dendritic cells. It is formation of firm , single or mu ltiple nodules occurring commonly in extremities (limbs). It can be red, brownish yellow (due to lipid}, or bluish black (due to haemosiderin). Dermatofibroma is common in females (4:1). It typically shows dimple/buttonhole sign wherein applying lateral pressure creates a central depression. Histologically, spindle cells are arranged in 'mat like' or 'cart-wheel' pattern. It is also called as dermal histiocytoma/dermal dendrocytoma. Treatment Excision. Differential diagnosis: Squamous cell carcinoma of skin; Melanoma; Basal cell carcinoma; Skin adnexal tumour.

Fig. 1.502: Skin adnexal tumour (malignant) turned out to be of hair follicle origin.

Figs. 1.503A and B: Skin adnexal tumour-preauricular region; (B) Skin adnexal tumour. Vascularity is increased and it is malignant.

Fig . 1.505: Dermatofibroma in leg.

DERMATOFIBROSARCOMA PROTUBERANS It is a low grade fibrosarcoma which grows slowly but persistently. It arises from dermal fibroblasts. Occurs in head and neck, limbs, abdominal wall and back. Trunk is the commonest site (50%). It is not a rare entity, often attains a large size with multiple, nodular, hard, swelling with often involvement of lymph nodes. Malignant spindle cells are seen histologically. Rarely it spreads into lungs through blood. It mimics squamous cell carcinoma of skin and skin adnexal tumour. Positive for CD34 and ring chromosome. With melanin pigmentation it is called as Bednar's tumour. Diagnosis: Biopsy of the lesion; Chest X-ray, CT scan; FNAC of the lymph node.

Causes: Human papilloma virus; genetic; immunosuppression; UV rays, trauma are considered. It presents as a rapidly growing, painless, single swelling in the skin with central brown area. It grows usually for 4 weeks and later shows spontaneous regression in 4 months. It is a pseudomalignancy. During regression phase, central area separates from the lesion leaving a deeply seated scar. Even though it is usual ly benign , 6% of cases may be invasive squamous cell carcinoma and often called as keratoacanthoma variant. Commonly keratoacanthoma is solitary, but rarely can be multiple, giant or generalised.

279 n

:::c )> "'ti

--i

m

....

;o

...C: l!l ... ':c;

VI

ID

VI 7':' ::J

2 3 0

...

C:

"'

Epidermis Dermis

Figs. 1.508A and B: Typical keratoacanthoma with central brownish area. It is self-limiting disease. It is crateriform ulcer.

I Features

Fig. 1.507: Dermatofibrosarcoma protuberans.

Treatment: Wide excision and follow up; Recurrence is common- 50%; Prognosis is good.

KERATOACANTHOMA (MOLLUSCUM SEBACEUM) It is an overgrowth and subsequent spontaneous regression of pilosebaceous glands with proliferation of squamous cells protruding out of the duct which are common in adult males (3:1) and places where more sebaceous glands are found . Typical locations are sun exposed areas. It is common in Caucasians after 50 years of age.

Mobile, hard, painless, nontender, lump with a central brownish volcano like area. It is common in face. It can be recurrent in lips and fingers. Lymph nodes are not enlarged. Differential diagnosis: Squamous cell carcinoma. Treatment Excision. The tissue is sent for histopathological study.

RHINOPHYMA (Potato Nose) (Bottle Nose) It is a glandular form of acne rosacea causing immense thickening of distal part of skin of nose with visible openings of sebaceous follicles. Nose is bluish red in colour with dilated capillaries. It is due to hypertrophy and adenomatous changes in sebaceous glands. Cosmesis is the main problem. Male to female ratio is 12:1. Three per cent cases may have occult BCC in it. But rhinophyma itself will not cause sec. Treatment Excision of excess tissue and reconstruction , dermabrasion, laser resurfacing, cryosurgery.

It's when the fish opens his mouth that he gets caught.

280

...>CV ...:::,C'I

V"I

,,, :::, ,,, 0

C:

_vi

al

a: V"I

Fig. 1.510C Figs. 1.510A to C: Seborrhoeic keratosis in face, ear and in periumbilical region. It is not a premalignant condition.

Figs. 1.509: Rhinophyma. Typical look and site.

SEBORRHOEIC KERATOSIS (Seborrhoeic Wart, Basal Cell Papilloma) It is a benign overgrowth of the basal layer of epidermis with excess of small darkly stained basal cells, which protrudes from the surface of the epidermis to give oily appearance.

I Features It is common in elderly. Common sites are the back, face, neck. It grows slowly with widening in area without altering in thickness. It often gets infected but uncommon to bleed on touch. It is pigmented due to melanin and so mimics naevus or melanoma. It is common Caucasians. It is familial-autosomal dominant gene related. Often when it falls off, it leaves a pale pink patch on the skin with visible small surface capillaries. It is not a premalignant condition. Note: Solar keratosis is a premalignant condition. It is hard and stiffer than normal skin. Lymph nodes are not involved. It does not occur in palms and soles. It can be picked off from the skin. 'Stuck on' appearance is characteristic. Differential diagnosis: Melanoma, pigmented BCC, naevus, Treatment , Excision cures the condition . :..- Shave excision or curettage or cautery or cryosurgery can be done. Scarring may be a problem.

B

Figs. 1.51 DA and B

Bowen's disease of skin: It is an intradermal precancerous condition. It presents as brownish induration with a well-defined edge. Microscopically it contains large clear cells. Eventually, it will turn into carcinoma (10%). Entire epidermis is disorganised and irregular. It shows parakeratosis, acanthosis, hyperkeratosis. Chronic solar exposure; arsenic; human papilloma viru s 16 are the aetiologies. Topical 5 fluorouracil or imiquimod; 4 mm margin surgical excision; MOHS; laser are the therapeutic options

Erythroplasia of Oueyrat is Bowen's disease occurring over glans penis Paget's disease of nipple Leukoderma Senile or solar keratosis: It is multiple, dry, hard, scaly, lesions on the face and back of hands due to exposure to sunlight, occurs after middle age. This is sunray induced hyperkeratosis with irregular, firm, raised or flat patch. Squamous cell carcinoma develops later after 1Oyears. Lesion in such situation becomes non-healing, indurated with central crust with everted edge; hard enlarged regional lymph nodes may be palpable Radiodermatitis, arsenic dermatitis Chronic scars develop into Marjolin's ulcer Albinism Xeroderma pigmentosa wherein there is defective DNA excision repair mechanism. It turns into malignant melanoma, BCC, sec Chronic lupus vulgaris Prolonged irritation of skin by various chemicals like dyes, tar, soot.

..... :::0

Fig. 1.512: Squamous cell carcinoma eyelid. Note the involvement.

Fig. 1.513: Squamous cell carcinoma on the labia.

Fig. 1.511: Bowen's disease (John T Bowen, 1912).

SQUAMOUS CELL CARCINOMA(Epithelioma) It occurs in premalignant conditions like Bowen's disease, Paget's disease, leukoplakia, chronic scars, chemically induced chronic irritation, radiodermatitis, senile keratosis, e.g. Kangri cancer in Kashmir, Chimney scrotal cancer, Kang

cancer of Tibetans. It arises from squamous layer of the skin. Usually it occurs in a pre-existing predisposing lesion; occasionally can develop in de nova skin. It can be grossly proliferative/ulcerative/ulceroproliferative/ red plaque like. Proliferative type is cauliflower like. It expresses cytokeratins one and ten. •·· It is the 2nd (20%) most common skin cancer. It is common in males. Exposure to UV 8 rays (ultraviolet rays are A, B, C types) causes sec by direct carcinogenic effects on keratinocytes, unrepaired mutations , decreased immune surveillance response, inhibition of tumour rejection, mutation of p53 suppressor gene (seen in 90% SCC).

Other risk factors are-inheritance, human papilloma virus (HPV 16-18), premalignant conditions (see box) , ionising radiation, carcinogens, chronic scar (Marjolin's), immunosuppression (azathioprine, etc.), chemicals (arsenic).

Bowen's disease. Exposure to UV B rays Chronic scars and sinuses (burns; osteomyelitis; venous ulcer) Lu pus vulgaris Solar keratosis-20% chances of sec Senile keratosis Xeroderma pigmentosa Tobacco use Viral cause-human papilloma virus (HPV) five and sixteen Chemically induced chronic irritation Radiodermatitis Ka11gri cancer-it is due to constant placing of the hot charcoal pot (kangri) over the abdominal wall to control cold. Seen in Kashmir Kang cancer is seen in buttocks and heel of Tibetans due to sleeping over oven bed to control cold

Chimney sweep cancer is observed in scrotum due to constant irritation by tar SCC is more common in immunosuppressed individuals, immunosuppressive drugs like azathioprine, cyclosporine, prednisolone; risk becomes 10% in 10 years of intake of these drugs and 40% in 20 years.

When door is closed, many more is open. Money can't buy life.-Bob Marley

282

Common sites are: Oorsum of hand, limbs, face, and skin of abdominal wall sec can occur in external genitalia, mucocutaneous junction, oral cavity, respiratory system, oesophagus, gallbladder, in urinary bladder as metaplasia from transitional cell lining.

I Features An ulcerative or ulceroproliferative or proliferative lesion. Raised and everted edge; lndurated base and edge; Bloody discharge from the lesion. Regional lymph nodes are commonly involved, which are hard, nodular, initially mobile but eventually fixed to underlying structures. Usually blood spread does not occur. 5% can develop metastatic SCC; risk factors are-immunosuppression, large growth, perineural and deep infiltrative, poorly differentiated.

Figs. 1.514A and B: Squamous cell carcinoma foot-ulceroproliferative lesion with fungating inguinal node secondaries. It is an advanced disease. Opposite inguinal nodes are also significantly enlarged.

Figs. 1.516A and B: Carcinoma cuniculatum-in heel and under great toe which is a low grade verrucous type of SCC.

Fig. 1.517: Verrucous carcinoma of penis. It is an exophytic, dry, warty, locally malignant lesion (Squamous cell carcinoma).

Figs. 1.515A and B: Recurrent SCC in amputated stump. Note the enlarged significant lymph nodes in the groin.

B Marjolin's ulcer which occurs in chronic scar is a type of squamous cell carcinoma without lymph node spread. Verrucous carcinoma is a squamous cell carcinoma, commonly occurring in mucous membrane or mucocutaneous junction without lymph node spread. It is dry, exophytic, warty, indurated growth. It has good prognosis. It is a curable malignancy. A rare multiple self-healing sec is observed usually in face as familial autosomal dominant (Ch 9q) disease in Western ScotlandFerguson-Smith syndrome. SCC often associated with BCC also.

Fig. 1.518: Squamous cell carcinoma and BCC in face of a patient.

Histology , Malignant whorls of squamous cells with epithelial or keratin pearls are characteristic feature. ,., Spindle cells, invasion. deep and peripheral margin clearance. ;., Desmoplastic, mucinous, pigmented, acantholytic- are other variants. ,., High risk features are-depth >2 mm, poorly differentiated, perineural invasion.

B I: II:

Well differentiated: 75% or more keratin pearls Moderately differentiated: 50-75% keratin pearls Ill: Poorly differentiated: 25-50% keratin pearls I IV: ~ndifferentiated/anaplastic: 2 cm is worse Tumour border-ill-defined border is worse Lymphovascular invasion worsens the prognosis Associated immunosuppression is worse Differentiation-poorly differentiated is worse Perineural involvement has worse prognosis Invasion; depth < 2 mm has got better prognosis; depth more than 6 mm has got worst prognosis Local recurrence rate is 20%. Recurrence period is 5 years, not beyond Fig. 1.520: Squamous cell carcinoma in the foot. It is proliferative cauliflower like lesion. Wedge biopsy has to be done.

283

MARJOLIN'S ULCER (RENE MARJOLIN, PARIS-1828) It is well-differentiated squamous cell carcinoma which occurs in chronic scars like burn scar, scar of venous ulcer. It occurs in unstable scar of long duration. It is slowly growing, locally malignant disease. It is a curable malignancy. As it develops in a scar due to chronic irritation and there are no lymphatics in scar tissue, it will not spread to lymph nodes.

Adversity reveals genius, prosperity conceals it.

284

...>.

(11

e'

::I VI

0

ttl

::I C: ttl

_.,,

co a:: VI

As scar is relatively avascular it grows slowly. As scar does not contain nerves, it is painless. Once it reaches the normal skin it may behave like any other squamous cell carcinoma, i.e. it will spread to lymph nodes. lndurated, painless, nontender, ulcer with raised and everted edge with scar in surrounding area is typical. There is marked fibrosis. Wedge biopsy from the edge, MRI of the part and regional node area confirms the diagnosis. Treatment: Wide local excision; in case of large ulcer, amputation is required. Note: RT should not be given as it may turn into poorly differentiated SCC.

Basal cell naevus syndrome ( Gorlin syndrome) with BCC; medulloblastoma; bifid ribs. It is only locally malignant. It does not spread through lymphatics nor through the blood. But it erodes deeply into local tissues including cartilages, bones causing extensive local destruction. Hence the name "rodent ulcer".

I Types Nodular-common in face, classic type. Cystic/nodulocystic. Ulcerative-Rodent (Jacob) ulcer. Mu ltiple, often associated with syndromes and other malignancies. Pigmented BCC-mimics melanoma. Geographical or field fire or forest fire BCC is wide area involvement with central scabbing and peripheral active proliferating edge. Basisquamous- behaves like squamous cell carcinoma which spread into lymph nodes. BCC which has not been treated for long time can turn into basisquamous carcinoma. Note:

Fig. 1.521: Marjolin's ulcer foot in a pre-existing burns scar. It is a well-differentiated squamous cell carcinoma occurring in an unstable scar of long duration.

Nodulocystic and noduloulcerative is the commonest form (70-90%).

BASAL CELL CARCINOMA (Rodent Ulcer) It is a low grade, locally invasive, carcinoma arising from basal layer of skin (or adnexal basal layer of hair follicle) or mucocutaneous junction. It does not arise from mucosa. It is the commonest (70%) malignant skin tumour. It is more common in white-skinned people than blacks. Common in places where exposure to UV light is more (Australia). Other causes are- arsenics, coal tar, aromatic hydrocarbons, skin tumour syndromes, xeroderma pigmentosa, albinism. It is common in males, common in middle-aged and elderly. Common site is face-above the line drawn between angle of mouth and ear lobule (90%)-Onghren 's line. It is called as tear cancer because it is commonly seen in area where tears roll down. Often it can occur in mucocutaneous junctions.

Fig. 1.524: Sebaceous epidermal naevus. It is common in females, often extensive, begins in childhood. It needs surgical excision and skin grafting. It has got 10% chances of turning into BCC.

I Clinicopathological Types Fig. 1.522: Note the common site of BCC-in the face above the line drawn between angle of mouth and ear lobule- Onghren's line.

a. Superficial type-small buds of tumour masses, common in young people. b. Morpheic type-dense stroma with basal cells and type IV collagen ; spreads rapidly; sclerosing BCC.

c. Fibroepithelioma type of Pinkus shows elongated cords of basaloid cells with mesh work, common in lower back. It contains outer palisading columnar cells with central polyhedral cells but no prickle cells or keratinisation.

....

:z::,

Fig. 1.525: BCC lesions in different places (multiple).

I Features Ulcer on the face in a middle-aged man which is nontender, dry, slowly growing, nonmobile, with raised and beaded edge withcentral scab, often with central depression or umbilication. Site of beading signifies the area of active proliferating cells. In between beaded areas dormant nonactive cells are present. No lymph node or blood spread occurs. Due to large sized tumour cells/tumour cluster, it does not spread through lymphatics. sec can be low risk or high-risk.

Size >2 cm Near the eye/nose/ear Ill-defined margins

Recurrent tumours • lmmunosuppressed individuals

Differential diagnosis: Squamous cell carcinoma; Melanoma; Keratoacanthoma; Seborrhoeic keratosis. Investigations: Wedge biopsy, X-ray of the part, CT scan dermoscopy is very useful. Treatment: , It is radiosensitive. If lesion is away from vital structure (like away from eyes), then curative radiotherapy can be given. Radiotherapy is not given, once it erodes cartilage or bone . RT is not given to sec of ear and close to lacrimal canaliculi. ,,. Surgery:

m

INDICATIONS FDR SURGERY

Rodent ulcer eroding into cartilage or bone BCC close to the eye Recurrent BCC after RT

Wide excision (4-6 mm clearance) with skin grafting, primary suturing or flap (Z plasty, rhomboid flap, rotation flap) is the procedure of choice. ,. Cryosurgery. ,

m Figs. 1.526A and B: Basal cell carcinoma in different locations. Note the nodularity and ulceration. Note the beaded edge.

,. MOHS (Microscopically Oriented Histographic Surgery) (Federic E Mohs, American Surgeon) is useful to get a clearance margin and in conditions like sec close to eyes, nose or ear, to preserve more tissues. MOHS is becoming popular in BCC/dermatofibrosarcoma protuberans/melanoma. Procedure is done by dermatological surgeon along with a histotechnician/histologist. Under local anaesthesia, a saucerised excision of the primary tumour is done and quad rants of the specimen are mapped with different colours. Specimen is sectioned by histotechnician from margin and depth, and it is stained using eosin and haematoxylin. It is studied by MOHS surgeon or histologist. Residual tumour from relevant mapped area is excised and procedure is repeated until clear margin and clear depth are achieved . Clearance must be complete and proper in sec otherwise there will be very high chance of recurrence (70%).

Other Methods Laser surgery, photodynamic therapy (using aminolevulinic acid lotion), cryosurgery, curettage and electrodesiccation, 5 fluorouracil local application (5% cream), topical 5% imiquimod, immunotherapy using Euphorbia pep/us.

Your mind will give back exactly, what you put into it.

286

1. Hairy mole is a mole with a hair growing on its surface.

TURBAN TUMOUR It is a descriptive term wherein entire scalp looks like a turban because of multiple scalp swellings. It can be due to multiple cylindroma; multiple hidradenomas; subcutaneous neurofibromas; nodular multiple basal cell carcinoma. Multiple cylindroma is usually considered disease under this term. Cylindroma is a variant of eccrine spiradenoma (skin adnexal tumor). Mu ltiple firm pinkish nodules in the scalp are the presentation in multiple cylindroma. They are rare, often locally malignant, grows slowly over the span of many years to cover entire scalp with reddish lobulated lesion. Hidradenoma is a rare benign sweat gland tumour. Multiple tumours commonly look like a turban in the scalp. They are painless, disfiguring, cosmetically problematic, soft, boggy, non-tluctuant, non-com pressible cutaneous swellings; commonly observed in middle age group. Multiple sebaceous cysts over the scalp mimic the same. Management is initial biopsy to find out the cause; then wide excision with skin grafting.

Fig. 1.528: Hairy naevus.

2. Nonhairy mole. 3. Blue naevus. It is seen in children. It is located deep in the dermis, hence appears blue. It is common in buttock (Mongolian spot), hand, feet. 4. Junctional naevus. It lies centred in the junctional layer (basal layer) of the epidermis as clusters. It is immature, unstable and premalignant. Microscopically there is proliferation of melanocytes at the epidermal junction. Features of malignant transformation are-change in the size, colour, bleeding, ulceration, crusting, satellite spots.

NAEVI (MOLES) It is excessive proliferation of melanocytes. It is hamartoma of melanocytes due to excessive stimulation. The risk in a small or medium-sized mole turning into melanoma is under 1%. It may present during birth or appear later.

I Types

e,;,,~;,i §

Fig. 1.529: Junctional naevus.

Melanocytes

Basal layer Dermis .._

5. Compound naevus. It is combination of intradermal and junctional naevus. lntradermal part is inactive but junctional part is potentially malignant.

Normal skin Epidermis : _._ _ Basal layer ~ Dermis.._

. .___. '

•

•

•

lntradermal naevus Epidermis + Basal layer Dermis .._

'1[l!i

Compound naevus Epidermis +Basal layer

---- 1:z:

Dermis + -

Junctional naevus

Fig. 1.527: Different types of naevi.

Fig. 1.530: Compound naevus.

6. Juvenile melanoma (Spitz naevus) (It is a misnomer). It appears as junctional like mole before puberty. It is seen in children on face. They present as brownish red nodular lesion which needs always excision. 7. Hutchinson's freckle. It is seen in elderly with large area of dark pigmentation. In the macular stage it is smooth and brown. In the tumour stage it is dark and irregular. It can turn into melanoma commonly. 8. Halo naevus-: Halo of depigmentation around the pigmented naevus. This halo is due to antibody response to melano-

287

cytes. Halo naevus is often seen along with vitiligo. Similar halo may develop around a malignant melanoma lesion. 9. lntradermal naevus: Cluster of dermal melanocytes is seen without junctional component. Common in face.

..... :::0 C) l'D

::,

l'D

ii.1 V\ C

Fig. 1.531: lntradermal naevus.

10. Spindle cell naevus-. It is dense, black pigmented lesion containing spindle cells and atypical melanocytes at dermoepidermal junction; seen in females on high with malignant potential. 11. Naevus spi/us: It is hyperpigmented speckles throughout, also called as speckled lentiginous naevus. Malignant potential is rare. 12. Naevus of Ota is dermal melanocytic hamartoma seen in distribution area of trigeminal nerve (ophthalmic /maxillary). It is seen in oriental and African race adolescent females (thigh) with a hormonal influence. 13. Naevus of Ito is similar lesion occurring in shoulder region. 14. Oysplastic naevus is proliferation of atypical melanocytes from epidermal basal layer having variegated irregular look; it is usually >5 mm in size; can be familial; 10% cases may turn into superficial spreading melanoma. 15. Congenital naevus: It is present since birth.

Fig. 1.533: Giant congenital naevus (Courtesy: Dr MuraliKeshav, MD, Pediatrician, KMC Mangaluru).

I Treatment

V\

Q. ::,

3

MELANOMA

0

It is a malignant tumour arising from epidermal melanocyte which is most aggressive cutaneous malignant tumour. It is of neural crest (ectodermal) origin. •·· It is 20 times more commonly seen in whites than blacks. Melanoblast Primitive cell derived from the neural crest. Melanocyte-. The cell which synthesises melanin is located in the basal layer (melanocyte : basal cell : : 1 : 10). Melanophores are pigment melanin carriers through dendrites into the epidermis. Melanophages are macrophages having melanin pigment. Melanoblasts and melanocytes contain DOPA oxidase enzyme and synthesise melanin. They show +ve DOPA reaction. Melanophores and melanophages show -ve DOPA reaction. Melanin synthesis is controlled by melanocyte stimulating hormone, ACTH and sex hormones. Dopa is 3,4-dioxyphenylalanine.

--i-,----

DOPA reaction:

DOPA

Tyrosinase

___i___

Melanin

Oxidase

Fig. 1.532: Congenital naevus.

Note:

':':

Excision. Always should be sent for histopathology.

Tyrosine

• Giant naevus is naevus more than 1% of body surface area or more than 20 cm in size. Giant congenital pigmented naevus (GCPN) often shows dermatomal distribution. Pigment cells spread from epidermis to fat and muscle often. It may turn into melanoma (5% risk). Such melanomas are usually axial; is usually associated with retroperitoneal / intracranlal melanosis. Curettage, dermabrasion, laser, excision and SSG are the treatment options. • Mongolian spot is a blue brown/grey pigmented macular area which is seen in sacral region during birth and after initial intense pigmentation regresses fully in 7 years. • Normal number of melanocytes releasing abnormally higher number of melanin granules is called as frecklelephilis.

., .,

\C n,

Its incidence is equal in both sexes. Its incidence is increasing over the years. Five per cent of skin cancers are melanomas. It is most common in Queensland, Australia. Auckland, New Zealand .

I Sites B

U

ead and neck-25% Trunk-25% Other sites-14%

Don't sit back and take what comes; go after what you want.

C ., "'

288

In females, leg is the commonest site. In males, the front or back of the trunk. In the Bantu tribe, sole is the commonest site. Other sites: Eyes (iris, ciliary body, choroids), mucocutaneous junction (anorectal region, genitalia}, head and neck (meninges, oropharynx, nasopharynx, paranasal sinuses).

I Risk Factors Exposure to sunlight (exposure to UV light; more common in white-skinned-20 times). Ethnic factors, socioeconomic status (high society), lifestyle, climate. Albinism. Xeroderma pigmentosa--f>.R is 1000 (by Kaposi in 1874): It is an autosomal recessive (Ch 9q) disease with defect in DNA excision repair mechanism causing formation of aberrant nucleotide causing 'ultraviolet rays' intolerance, erythema, pigmentation , photophobia, premature skin ageing, severe sunburn, painful sun sensitive eyes with corneal ulcers, freckling and blistering of skin, dry, scaly, irregular skin, multiple malignancies with 60% mortality at the age of 20. Incidence is one in 1,00,000 people; more common in Japan. DNA repair assessment of skin and blood, amniocentesis or chorionic villous sampling in fetus can confirm it.

Patients who are on immunosuppressive drugs or after renal transplantation or NHL (RR - 30). MALIGNANCIES WHICH SPREAD FRDM MOTHER TO FOETUS t

I

•

I

•

I Classifications Breslow's classification (1970): Based on thickness of invasion measured by optical micrometer-most important prognostic indicator until nodal spread I: Less than 0.75 mm II: Between 0.76 to 1.5 mm Ill: 1.51 mm to 4 mm IV: More than 4 mm

Relation of Tumour Thickness to Nodal Spread-Based on AJCC Classification Lesion Thin Intermediate Thick

Tumour thickness

Nodal spread

4 mm

1 mm thick lesion)

...>O'I ... ::, QI

VI

0

B

tO

::, C: tO

_.,,

Fig. 1.541: Amelanotic melanoma; often mimics soft tissue sarcoma.

VI

• Oesmop/astic melanoma has high affinity for perineural invasion;

co a:

is common in head and neck with higher recurrence rate. It is often amelanotic melanoma with thicker lesion carrying poor prognosis due to neural invasion. • Subungual melanoma which was earlier thought ofacral lentiginous type is now considered as superficial spreading type. It is involvement of nail fold matrix (not nail plate). Triangular, macular, progressively widening pigmentation of nail fold with nail dystrophy is typical-Hutchinson's sign. It should be differentiated from benign racial melanonychia which are dark streaks under the nail. Biopsy ofnail matrix should be done here. • Ocular melanoma: It is the commonest malignancy arising in eye. It may arise from retina, iris, ciliary body, choroid. It rarely metastasize or only at late stage as it is devoid of lymphatics. Ocular melanoma commonly shows its distant spread to liver. Massive hepatomegaly is typical and is often seen many years after the treatment of primary ocular lesion. Condition is treated with enucleation, radiation, photocoagulation. • Clark's concept-Two phases of growth: Initial radial growth phase occurs horizontally, later vertical growth phase occurs with invasion.

I• •• I I• ~

Asymmetry Border irregularity - coast of Marine sign Colour variation Diameter> 6 mm Evolving (changing _ Pr_o_gr_e_ss_iv_e_lY_)_ _ _ _ _ _ _ _

I Spread Through lymphatics it spreads to regional lymph nodes either by permeation or by embolisation.

•·· In-transit nodules are seen in the skin between the primary lesion and regional lymph node area, and is due to retrograde spread to dermal lymphatics. Through blood: To lungs, liver (huge liver), brain, skin, bones. Secondaries are typically black.

I

Brain-convulsions, localising features and raised intracranial pressure / • Lung-cannon ball secondaries, pleural eHusion-haemoptysis, chest pain and cough / • Liver (massive liver), ascites I • Skin-cutaneous nodules often pigmented Bones-bone pain, pathological fracture. Paraplegia/neurological deficits in spine metastasis Extensive visceral involvement causes melanuria

I

L____ - - - - - - - - - - - - - - - - -

Melanoma in choroid has got better prognosis, because as there are no lymphatics, spread is delayed. ••• Sometimes primary is very small and unnoticed (in anus, subungual region). They present with features of secondaries only. Fig. 1.542: Aggressive melanoma in the axilla; could be

desmoplastic type.

I Clinical Features It can stat in a pre-existing naevus (commonly junctional naevus)-50-60% or as de novo in a normal skin--40-50%. Melanoma is unknown before puberty.

•·· lnduration is not seen in melanoma. Pigmentation with irregular surface and margin with rapid growth.

Ulceration, bleeding, itching, change in the colour. Note: When a mole turns malignant, following changes should be observed (Glasgow criteria):

Figs. 1.543A and B: Primary melanoma with lymph node secondaries

in two different individuals. Note the pigmented ulcerated secondaries in one.

Anus Genitalia Scalp Eye

External auditory canal Adrenal medulla Nail bed -

B

Figs. 1.544A and B: (Al 'In-transit' nodules in melanoma. They are secondary depositions in dermal lymphatics; (B) Satellite nodule in melanoma occurs within 2 cm of primary. Note the primary in the heel and satellite nodule adjacent to it.

Note: • Satellite nodule occurs within 2 cm of the primary melanomawhereas in - transit nodules occur in between primary and lymph node secondaries.

• Infiltration into deep fascia by melanoma is rare in initial stages as deep fascia acts as a strong barrier. • Melanoma can also occur in places other than skin liketongue, mucous membrane or genitalia.

- -

-

-

MELANOMA

5% of all skin cancers-incidence 20 times more common in whites than blacks Mucosa! melanoma has got poor prognosis Can spread from mother to foetus Multiple melanomas are 1% common Melanoma in choroid will not cause lymph node involvement, as it has no lymphatic drainage. But late massive liver secondaries even after 10-20 years is known to occur 10% of melanomas are familial-in whites Satellite nodules are secondary skin nodules within 2 cm of primary In transit' nodules are secondary skin nodules beyond 2 cm of primary any where up to lymph node region Melanoma may present as secondary (in liver, lungs, bone, brain) with occult primary when primary is situated in anus, genitalia, eye, external auditory canal, adrenal gland, nail bed and scalp-7% Pigmentation is not mandatory to diagnose melanoma even though it is commonly present Melanoma also can occur in fishes, dogs and horses

I Investigations

Fig. 1.545: Melanoma in tongue.

Figs. 1.546A and B: Melanoma in vagina-in mucocutaneous

junction. On table and excised specimen of melanoma.

No incision biopsy. It can cause early blood spread. Only in large lesions or already metastatic lesions incision biopsy is advocated. Excision biopsy of primary. It is done with 2 mm margin with deeper fatty tissue. One should avoid using cautery and avoid crushing the tissues as much as possible. Instead of excision biopsy, punch biopsy is done in case of large primary tumour very close to pinna, eye, nose. Punch biopsy assesses the depth/thickness of the lesion. Punch should be done at the most elevated part of the lesion to get proper depth. FNAC of lymph node. US abdomen to look for liver secondaries (usually huge hepatomegaly occurs). Chest X-ray to look for secondaries in lung ("cannon ball" appearance). HRCT of chest is ideal. Relevant other methods depending on site and spread, e.g. CT scan of head, chest, abdomen, pelvis. ••• Urine for melanuria signifies advanced disease. Sentinel lymph node biopsy (SLNB). Tumour markers-LOH; MeIan - A; S 100; tyrosinase; HMB 45 are the tumour markers used. Human melanoma black 45 (HMB 45) is a monoclonal antibody against specific antigen (Pmel 17) present in melanocytic tumours. HMB 45 has got 92% sensitivity. MRI of the area; PET scan to detect the spread- in seleted patients only. Desmoscopy in early cases.

In time of test, family is best.

....

::c

292

TNM STAGING (AJCC, 8TH EDITION , 2018)

T (Tumour) TO - No evidence of primary tumor; Primary site of tumor is unknown Tis- in situ carcinoma {thickness, ulceration not applicable) T1 : < 1 mm; T1a - < 0.8 mm without ulceration; T1 b - < 0.8 mm with ulceration or > 0.8 to 1 mm with or without ulceration. T2: 1-2 mm; T2a without ulceration; T2b is with ulceration. T3: 2-4 mm; T3a is without ulceration; T3b is with ulceration. T4 > 4 mm; T4a is without ulceration; T4b is with ulceration. N - Nodes

NO: No regional nods involved. N1 : N1a - Clinically occult One node detected by SLNB: N1 b - Clinically detected one node; N1 c - No nodes but presence of in transit, satellite or microsatellite metastasis. N2: N2a - 2 or 3 clinically occult nodes; N2b - 2 or 3 nodes with at least one clinically detectable node; N2c - One clinically occult or detectable node with in transit, satellite or microsatellite metastasis. N3: N3a - 4 or more clinically occult nodes; N3b - 4 or more nodes with at least one clinically detectable nodes; N3c - 2 or more clinically occult or detectable nodes with in transit, satellite or microsatellite metastasis. M - Metastases

MO - No distant spread M1 : M1 a - Skin, soft tissue, non regional node spread without (M1 a (0)) or with LOH elevation (M1a (1 )). M1 b - Distant spread to lungs with or without M1 a without raise in LOH (M1 b (0)) or with raised LOH (M1 b (1)). M1 c - Distant spread to Non CNS viscera with or without M1 a or M1 b without raise in LOH (M1 c (0)) or with raise in LOH (M1 c (1 )). M1 d - Distant spread to CNS with or without M1a,b,c without raise in LOH (M1c (0)) or with raise in LOH (M1c (1)). Staging Stage 0: Tis, NO, MO. Stage IA: T1 a, T1 b, NO MO. Stage 18: T2a, NO MO. Stage IIA: T2b, T3a, NO, MO. Stage 118: T3b, T4a, NO, MO. Stage IIC: T4b, NO, MO. Stage IIIA: T1a/b, T2a; N1a or N2a; MO. Stage 1118: T1 a/b, T2a; N1 b/c, N2b; MO. T2b, T3a, N1a, N2b, MO; TO, N1b/c, MO. Stage IIIC: T1a to T3a, N2c or N3a/b/c, MO; T3b/ T4a, any N (I/

ei

::::,

....0 VI

tU

::::, C: tU

_.,,

c:o a: VI

Preoperative lymphoscintigraphy is done on the day of surgery for primary tumour. 0.5 mCi Technetium 99 sulphur colloid is injected into the normal dermis within 0.5 cm from the margin of melanoma. Dynamic and static images are taken to identify lymphatic channels, sentinel node (s). interval node (like popliteal node in lower and epitrochlear node in upper limb). After that under general anaesthesia, just before wide local excision (WLE) of the melanoma, 1-5 ml of isosufan blue dye is injected into the dermis similarly. By this, senile lymph node is identified in 99% of patients. SLN is defined as a node which indicates adirect lymphatic drainage pathway from primary; it is the most radioactive node in nodal basin; shows radioactive count 10% more than other nodes; takes blue dye adequately; on table this node will be palpably suspicious of tumour. In melanoma nodal basin usually 2 SLNs are identified (as first echelon nodes in nodal basin). Axillary and inguinal SLNs are easier to identify than head and neck SLNs which are usually located adjacent to spinal accessory nerve. Entire SLN (s) should be removed and sent for histology, immuno- I histochemistry-S-100 and HMB-45. Interval lymph nodes like popliteal or epitrochlear when involved 1 should be cleared surgically.

I

I

I I

I

I I

I I

I

L_

--

--

- - - - --- --- --- .

B rcinoma breast rcinoma penis

-- -- -------

4. Prophylactic regional block dissection which was previously advocated is now controversial. But still used in many centres. Elective lymph node dissection (ELND) is done when tumour thickness is 1-4 mm. 5. Management in unknown primary(2%) presenting as nodal secondaries is by nodal radical dissection at the region with adjuvant chemotherapy. They have better prognosis than with known primary. Patient should be monitored and evaluated to identify the primary site during every follow up. Once primary is identified it is treated accordingly depending on its location.

For Loco Regional Recurrent Melanoma: Local recurrence is one which recurs within 5 cm radius of the primary tumour in skin or subcutaneous tissues. Risk of local recurrence is 0.2% if primary tumour is less than 0.75 mm; 2% if it is between 0.75-1.5 mm; 6% if it is 1.5-4 mm; 12% if it is more than 4 mm. ,, Isolated limb perfusion (ILP) (Creech et al, New Orleans, 1958) using cytotoxic agents like Melphalan (M for M), interleukin 2, tumour necrosis factor (TNF). Concentration used here is 15-25 times more than that is used for systemic therapy. Melphalan dose is 10 mg/I perfusion solution in leg (13 mg/I in arm). With transverse incision, extremity vessels (artery and vein like femorals) are exposed for 3 cm; secure tourniquet is applied; major artery and vein are cannulated or

arteriotomy and venotomy done transversely; Melphalan (phenylalanime mustard, 1 mg/kg) and Actinomycin D (0.5 gram) is injected at high temperature of 42°C with a pump and oxygenator through cannulas in femoral artery and vein (with a proximal tourniquet in situ). Drugs are administered as single bolus through the pump along the extracorporeal circuit. Patient is heparinised initially later reversed using protamine after vascular suturing. One hour pump run wash out is given. Hyperthermia and oxygenation increase the metabolic activity of tumour cells to make it more vulnerable to melphalan. Procedure controls the local disease well with preserving the functioning limb. It is used in local recurrence or 'In-transit' deposits. It shows 80% response rate with 15% complete response; but only of short period. Complications like DVT, bleeding, sepsis can occur. ,, Isolated limb infusion {ILi) (Thompson, 1993): Vascular catheters are passed and placed across femoral artery and vein through opposite femoral vessels or through arm vessels. The limb is warmed ; patient is anaesthetised 2 hours later and also heparinised; papaverine is injected into arterial catheter and tourniquet is applied in the thigh/arm. Melphalan 7.5 mg/I, actinomycin D 75 µg/1 in 400 ml saline (10 mg and 100 µg/1 in 300 ml NS in upper limb) is infused into the isolated limb for 6 minutes which is pumped around the limb repeatedly for 30 minutes with a hypoxia in limb; drugs are washed out using a Hartmann's solution; tourniquet is removed and normal circulation is regained with removal of vascular catheters. Protamine is given to reverse heparin action. Here extracorporeal circuit and oxygenator are not required (unlike in limb perfusion). Procedure is also used in extremity sarcomas. Laser ablation of multiple small cutaneous lesions but with doubtful benefits.

For Distant Spread Brain, lung and liver are the most common sites; skin, bone, GI are less common sites. But melanoma is one of the commonest tumours to spread to intramural GITto present as intussusception. Distant spread when found or suspected, CT scan of head, chest, abdomen and pelvis are needed. PET scan and tumour markers are very useful. ,.. Chemotherapy and immunotherapy is the main treatment. , Isolated lung or liver metastasis can be resected. ,, Radiotherapy is useful for bone and brain secondaries. Stereotactic program using gamma knife is better for brain secondaries.

Chemotherapy for Melanoma Indications: a. Secondaries in lungs, liver, bones. b. After surgery for melanoma. Usually it is given intravenously.

Drugs are: a. DTIC: Diethyl triamine iminocarboxamide. b. Melphalan (Phenyl alanine mustard) (Melphalan for melanoma). c. Carboplatin , vindesine. d. CVD regime-is cisplatin, vinblastine and dacarbazine. e. Tamoxifen as a ceramide sensitizer rather than part of the chemotherapy regime (Dartmouth regimen-DTIC, BCNU, cisplatin and tamoxifen) may be beneficial.

lmmunotherapy/Biological Therapy

G) I!)

It is done using specific tumour antibodies, BCG, levamisole, Corynebacterium parvum, alpha interferon, interleukins and tumour vaccines are tried with some success rate up to 40% in advanced melanomas. Biochemotherapy is com bination of CVD with interferon a and interleukin 2. Interferon a is acytokine which is antiangiogenic and stimulator of natural killer NK cells. Dose is 20 mU/m2 IV 3 times a week for 4 weeks then maintenance dose of 10 mU/m2 subcutaneously 3 times a week for one year. Severe myelodepression and fulminant liver necrosis are the toxicity and so often dose is reduced to 3 mU/m2 three times weekly for 2 years. lmiquimod is applied over the tumour as cream. Talimogene laherparepvec (T-VEC), BCG, IL-2, IFalpha 2b, BCG are used as liquid injected into the tumour. GM2 ganglioside based vaccine (stimulates production of lgM antibodies), Melacine (contains melanoma cell lysates) and cancerVax are three vaccines under trial at present. lpilimumab is a monoclonal antibody that boosts the body's immune response against melanoma cells. Pembrolizumab and nivolumab are two other drugs. They are PD 1 receptor inhibitor (Programmed Death Receptor 1, seen in T cells) drugs.

Endolymphatic Therapy •·· It can be done to control disease in the nodes using radioactive 1131 or P32 with ultrafluid lipiodol along with lymphangiography.

Targeted therapy Signal transduction inhibitor therapy using-(1) BRAF inhibitors-Vemurafenib, dabrafenib; (2) MEK inhibitorsTrametinib and cobemetinib; (3) C-Kit inhibitors-imatinib, nilotinib are used. Usually combinations both groups are used tablets (pill) to be swallowed . Other targeted therapies are-oncolytic virus therapy; monoclonal antibody therapy. Note:

:I

I!)

@ \II

... ...

C:

Fig. 1.552: Melanoma involving face extensively with destruction. Note the maggots over the surface of tumour. Melanoma is most aggressive cutaneous malignancy.

I.C I!)

':c:

\II

:I

--l

I Prognosis for Melanoma

C:

3 0

It is not good since it is a very aggressive tumour. Old age has worse prognosis. Females show better prognosis. Extremity melanoma has better prognosis than head and neck. Pro11no1tic factors-ovsra/1 poor • Tumour thickness-very important factor • Nodal spread-once regional nodes are positive, 85% of patients will have occult distant spread. Number of positive nodes is also important • Ulceration-poor • Angiogenesis, vascular invasion-poor In-transit nodules- poor • Vertical growth-poor prognosis • Metastatic disease-poor • Staging

Staging as pro11nostic factor Stage I: >90% prognosis Stage II: 70% Stage Ill: 35% Stage IV: ,

...Cl QI

:I 1/\

0

11:1

:I C 11:1

_,.,, co a:

1/\

T - Tumour (T) TX - Primary tumor cannot be assessed TO - No evidence of primary tumor T1 - Tumor 5 cm or less in greatest dimension T2 - Tumor more than 5 cm and less than or equal to 1Ocm in greatest dimension T3 - Tumor more than 10 cm and less than or equal to 15 cm in greatest dimension T4 -Tumor more than 15 cm in greatest dimension T Suffix (m) Select if synchronous primary tumors are found in single organ N - Nodes (N) NO - No regional lymph node metastases or unknown lymph node status N1 - Regional lymph node metastasis N Suffix (sn) if regional lymph node metastasis identified by SLN biopsy only (f) if regional lymph node metastasis identified by FNA or core needle biopsy only M - Distant Metastasis (M) MO: No distant metastases M1 : Presence of disatant metastases The terms pMO and MX are not valid categories in the TNM system. Assignment of the M category for clinical classification may be cMO, cM1, or pM1 cMO - No distant metastasis cM1- Distant metastasis pM1 - Distant metastasis, microscopically confirmed

Definition of FNCLCC (Federation Nationale des Centres de Lutte Contre le Cancer) Histologic Grade (G) The FNCLCC grade is determined by three parameters: Differentiation, mitotic activity, and extent of necrosis. Each parameter is scored as follows differentiation (1-3), mitotic activity (1-3), and necrosis (0 -2) The scores are added to determine the grade GX - Grade cannot be assessed G 1 - Total differentiation, mitotic count and necrosis score of 2 or 3 G 2 - Total differentiation, mitotic count and necrosis score of 4 or 5 G 3 - Total differentiation, mitotic count and necrosis score of 6, 7, or 8 Tumor Differentiation score 1 - Sarcomas closely resembling normal adult mesenchymal tissue (e.g. low grade leiomyosarcoma) 2 - Sarcomas for which histologic typing is certain (e.g. myxoid/round cell liposarcoma) 3 - Embryonal and undifferentiated sarcomas, sarcomas of doubtful type, synovial sarcomas, soft tissue osteosarcoma, Ewing sarcoma/ primitive neuroectodermal tumor (PNET) of soft tissue Mitotic Count (Score) In the most mitotically active area of the sarcoma, 10 successive high-power fields (HPF; one HPF at 400 x magnification = 0.1734 mm2) are assessed using a 40 x objective. Mitotic Count Score: 1: 0-9 mitoses per 1OHPF. 2: 10- 19 mitoses per 1OHPF. 3: 2!20 mitoses per 1OHPF. Tumor Necrosis Score 0 - No necrosis. 1 - 5 cm-important factor More than one compartment involvement Neurovascular invasion Clearance margin

V\

High grade Deep tumours and multicentric Lung secondaries

Figs. 1.566A and B: Recurrent soft tissue sarcoma over scapular region and forearm in two different patients. Old surgical scar is seen.

"::s... f1) f1)

QI

l.n C:

IQ

... f1)

': ""O

-i

Bones

m

....-i

:::0

BK amputated limb

Cl

Ill

:::,

...

Ill 11,1

"' ... C:

Incision for Ray amputation Walking component

Fig. 1.577: Pylon is plaster cast with walking component used fo r immediate mobilisation after below-knee amputation.

Fig. 1.579: Ray amputation for toes- racquet incision.

...... er

-0 C:

11,1

:::,

"'

I Different Amputations

1. Ray's 3. Tarsometatarsal (Lisfranc's)

5. Syme's

7. Transcondylar

2. Transmetatarsal (GIilies) 4. Midtarsal (Chopart's) 6 . Below-knee (Burgess) 8. Above- knee

Fig. 1.578: Different levels of amputation in lower limb.

l>

3

-:- Stumps can be side bearing (sutures are on the side); end bearing/conical (sutures are on the end) or cylindrical. -:- Postoperatively active exe rcise sh ould be given to the proximal joint so that prosthesis can be fit to it properly. If there is sepsis especially in gangrene limb, flaps should be left open or loose suture applied otherwise flap necrosis occurs. -:- Proper anatomy of muscles and neurovascular bundle around should be known in all amputations.

Ray amputation Amputation of toe with head of metatarsal or metacarpals. -:- Transmetatarsa/ amputation (Gillies') Here amputation is done proximal to the neck of the metatarsals, distal to the base.

10Ill

Fig. 1.580: Ray's amputation done for gangrene of great toe. It is usually not sutured.

Fig. 1.581 : Forefoot amputation.

-:- lisfranc's amputation ( Tarsometatarsal amputation) Here tarsometatarsal joint is disarticulated with a long volar flap. It needs a surgical boot. But there is inevitable develop-

Asking for help is strength, not weakness.

310

...>. ...:::, QI

C,

V'I

0

IO

:::, C:

IO

_.,.

al

a:

V'I

ment of equinovarus deformity. So stabilisation of midtarsal and ankle joints is needed. In Hey's modification, 2nd metatarsal is cut at base instead of disarticulation. Chopart's amputation (Midtarsal amputation) Here talonavicular joint and calcaneo-cuboid joints are disarticulated. Tibialis anterior muscle is sutured to drilled talus bone. A long volar flap is used and immobilised for 6 weeks after surgery. Syme's amputation It is removal of the foot with calcaneum and cutting of tibia and fibula just above the ankle joint with retaining heel flap (dividing both malleoli). Heel flap is supplied by medial and lateral calcaneal vessels (branches of posterior tibial artery). Elephant boot is used for the limb after Syme's which is inexpensive. Many patients walk well with Syme's stump without difficulty. It is presently mainly used in trauma (crush injury) and malignancies in distal part of the foot. In vascular diseases, calcaneal vessels may not be adequate to maintain the viability of the flap. While raising the flap, knife should be very close to the calcaneum so as to avoid injury to calcaneal vessels and to maintain the viability of the flap. In Wagner's method deep fascia of heel is sutured to drill holes made on the anterior edge of tibia and fibula. Above-knee cast is essential. Advantages are-it is an end-bearing stump having good proprioception. Patient can walk without prosthesis. Low energy consumption ambulation is possible. ,. Disadvantages- posterior displacement of heel pad; poor cosmesis. ),., Boyd's amputation-anterior part of the calcaneum is excised (osteotomy) just distal to the peroneal tuberosity and calcaneotibial arthrodesis is created. It is done to prevent posterior migration of heel pad.

Level of bone cutting - ~,___-• (tibia and fibula just above malleoli)

Fig. 1.583: Incision for Syme's amputation.

Fig. 1.584: Elephant boot used after Syme's amputation.

Modified Syme's amputation Here heel flap is elliptical. Tibia and fibula are divided slightly higher. But variation here is the elliptical flap.

Level of bone cutting (tibia and fibula just

-

-,1;~-~

a bove malleoli)

Incision fo r Syme's amputation

Fig. 1.582: Syme's incision and level of amputation.

Incision for modified Syme's amputation

Fig. 1.585: Modified Syme's incision is elliptical one.

311

Pirogoff's amputation It is like Syme's amputatio-n except posterior part of the calcaneum is retained along with heel flap. It provides longer stump than Syme's amputation.

n :c "'ti

-I

m

....-I

::0

" /1)

:::, /1)

Fig. 1.588: Below-knee amputation-long posterior flap. Note the placing of the drain.

VI

...C:

IQ /1)

)>

3

"C C:

.... .... o· :::,

Calcaneum part

QI

Fig. 1.586: Pirogoff's amputation.

Below-knee amputation Here we use a long-posterior flap with scar placed over anterior aspect. Prosthesis placement is better here with greater range of movements without limp and without support. It is also called as Burgess amputation. Fibula should be divided first, higher than the proposed site of cut of tibia otherwise its sharp end will press on the skin flap. Tibial stump should be beveled anteriorly. Posterior muscles are sutured across the bone end, to the periosteum in front. In more proximal type of below-knee amputation, fibula often is removed to allow the proper use of flap. Length of the flap should be 11/2 times the circumference of the site (around 12 cm). Stump length is 14-17 cm from knee joint. Minimum length required for prosthesis is 8 cm. If there is need to extend more proximally, it is better to do above-knee amputation. Modern artificial limbs like suction socket prosthesis are used now.

"'

Fig. 1.589: Below-knee amputation flap necrosis has occurred which eventually granulated with repeated dressings. Area is ready for skin grafting.

Fibula

Muscle

Myocutaneous nap Incision

Tibia

Flap

Anterior view

Posterior view

Fig. 1.587: Below-knee amputation technique-incision, flap and levels of cutting bones.

Fig. 1.590: Bilateral below-knee amputations done for vascular causes with diabetes.

Imagination is the highest kite one can fly.

312

'Peg-leg' amputation , It is amputation 5 cm below the knee level-proximal most below-knee amputation. It is not practiced nowadays. Here anterior flap is rotated posteriorly like a hood and patient kneels and bears weight which is wellaccustomed to pressure. It is done whenever prosthesis cannot be used probably due to economic causes (in developing countries).

rehabilitation is difficult, and fitting of prosthesis is not proper, patient needs a third support for walk with often a limp. Skin incision

Fig. 1.592: Above-knee amputation, incision and approach.

Fig. 1.591 : 'Peg-leg' amputationis below-knee amputation done close to the knee joint (5 cm stump) with folding flap and metallic support down. It is not used now.

Transcondylar-Gritti-Stokes amputation with long posterior flap. , Femur is divided just above the articular surface and patella is anchored to the divided femur. There is risk of nonunion between patella and femur. This procedure is no longer performed. Above-knee amputation , Usually equal anterior and posterior flaps are used. Lower th ird and middle third level amputations are done. Ideally the required length of the femu r as stump is 25 cm from the tip of the trochanter. Femur length lesser than 1O cm is not possible and here one needs to do hip disarticulation. In children as growing epiphysis of femur is in lower end , it is essential to preserve as much length of fem ur as possible. It is done in ischaemia, trauma, sepsis, gangrene which is spread ing above. Often patient might earlier have undergone below-knee amputation but now as indicated need above-knee amputation. It is usually contraindicated in children (done only in undue definitive indication) or if stump is less than 7.5 cm. :.- Advantages are technically easy, healing chances are better and faster. Disadvantages are cosmetically poor,

Fig. 1.593: Above-knee amputation wound which is infected in a

diabetic patient. Hip disarticulation It is done whenever it is not possible to save the minimum 10 cm length of the femur. Incision used is either single posterior flap- Solcum's approach (better) or anterior racquet incision-Boyd's approach. Hind quarter amputation lnter-innominate abdominal amputation (Sir Gordon Taylor's amputation): Removal of one side pelvis with innominate bone, pubis, muscles and vessels. Original ligation of common iliac artery is modified to individual ligations of external and internal iliac vessels. Internal iliac artery is

ligated beyond the origin of the superior gluteal artery to keep the large posterior flap viable. Now hind quarter name is replaced by hemipelvectomy. It may be standard hemipelvectomy with classic gluteal flap; extended hemipelvectomy with removal of posterior part of the sacrum; conservative hemipelvectomywith retaining part of the pubis, ilium bones on that side. Internal hemipelvectomyis new method wherein hemipelvectomy is done with preserving the limb.

It is removal of entire upper limb with scapula and lateral 213rd of the clavicle and muscles attached to it. It is done in malignancies involving scapula, upper part of humerus and near shoulder joint. In emergency conditions like severe sepsis, gas gangrene and machinery entrapment, Guillotine amputation is done without suturing. Suturing is done at later period. All tissues are divided at same level.

313

G"\

11)

::::,

11)

@ 1/1 C

IQ

...

11)

':c:

)>

3

't,

C ,.,. ,.,. 0,1

c>' ::::, 11'1

Fig. 1.596: Upper limb Guillotine amputation-above elbow done for

trauma induced gas gangrene. fig . 1.594: Hindquarter amputation done for run over of vehicle over pelvis, right thigh. Patient also underwent colostomy. Patient survived with severe morbidity.

Krukenberg's amputation Done in upper limb following any trauma. Here forearm amputation is done in such a way that it creates a gap between radius and ulna like a claw to have a hold or grip. lnterscapulothoracic amputation (Forequarter amputation) (Littlewood'sposterior approach or Berger's anterior approach):

figs. 1.595A and B: (A) Forequarter amputation done for electric burn which caused extensive damage to upper limb, (B) Forequarter amputation done for sarcoma proximal to shoulder joint.

I Postoperative Period Physiotherapy is advised. Regular dressings are done. Crutch is used initially, after 3 months prosthesis is placed. Rehabilitation is important.

i

Fig . 1.597: Patient with axillary crutches.

Love the life you live; Live the life you love.

314

COMPLICATIONS OF AMPUTATIONS

I Early Haemorrhage, haematoma, infection.

I Late Pain, ulceration of stump, ring sequestrum formation, flap necrosis, painful scar, Phantom limb-feeling of amputated part in toto or partially with pain over it.

_.,.

Haematoma

vi

It is identified by pain, swelling over the stump underneath the flap. It is aspirated using a wide bore needle. Haematoma may delay healing; may precipitate infection or flap necrosis due to pressure. After aspiration, pressure dressing is needed. If haematoma reforms after 2-3 aspirations, it should be drained by opening the wound on one corner and inserting haemostat into the wound.

cc a:

Stump Neuroma It can occur due to proliferation of the nerve fibrils beyond the point of nerve division and is usually due to failure of cutting of the nerve more proximal to the level of division of the bone. It causes pain and tenderness over the stump. It is usually relieved by analgesics, re-assurance and prosthesis. Occasionally, it may require re-exploration of the wound, excision of end neuroma and also cutting nerve more proximally.

Infection of the Stump It may cause abscess formation, delay in wound healing, flap necrosis, giving way of the wound. Removing few or all sutures to relieve pressure and draining the pus underneath is needed. Infection may also lead into poor scar, adherent scar which causes difficulty in placing the prosthesis.

Figs. 1.599A and B: (A) Below-knee amputation stump infected. (B)

tibia is exposed.

Stump Pain after Amputation It is a common problem. Causes are-infection, poor blood supply, causalgia, stump neuroma, phantom pain/limb, deep vein thrombosis, adherent scar, formation of spurs and osteophytes at amputated bone end. Scar adhesion to bone is prevented by keeping adequate length of deep fascia underneath intact. Spurs and osteophytes are confirmed by X-ray and needs removal using bone nibbler after appropriate skin incision.

Phantom Limb

Fig. 1.598: Above-knee amputation stump having small sinus.

Flap Necrosis It is a common complication. Main causes for flap necrosis are poor blood supply, infection, haematoma underneath, inadequate length of the flap causing stretching of flap. Small area of necrosis can be excised. Wider area needs laying opening of the wound or revision of the stump or higher level amputation. Anaemia, poor nutrition, nutritional deficiencies, diabetes mellitus, immunosuppression, smoking, old age are other factors causing flap necrosis.

It is typical awareness of sensation that as if amputated part is still exists partly or in toto; often such part may be painful or disturbing or hyperaesthetic. Exact cause is not known; but it is probably due to severe pain at the amputated part just prior to amputation making brain area for that part in alert situation causing phantom limb. Reassurance, prosthesis, analgesics help to control the condition. It is said that it can be prevented by proper pain control for 24 hours prior to amputation; but it is often difficult. It is common in upper limb.

Ulceration over the stump It is not uncommon. It is due to necrosis, infection, lengthy bone stump pressing on the summit of the flap, prosthesis, nutritional deficiencies, diabetes mellitus, ischaemia. Ulcer may be small/large; superficial/deep. Callous chronic ulcer at

the end of the stump is called as Douglas ulcer. Small ulcer is later treated by regular dressings and suturing. Large ulcer needs flap to cover the defect. Osteomyelitis of the stump should be ruled out in chronic stump ulcer. Ring sequestrum may be typical in such situation. Revision amputation is needed for the stu mp.

Contracture of the Joint Contracture of the joint proximal to the amputated stump is common. It is mainly due improper positioning after amputation due to pain, poor exercise and occasionally due to inflammation of surrounding soft tissues. Contracture interferes with proper fitting and functioning of the prosthesis and delays rehabilitation . Proper positioning, passive stretching and exercises, strengthening exercises with help to correct it. Rarely needs surgical release of the contracture.

Other Complications Scar hypertrophy, skin thickening, hyperkeratosis, papilloma, eczema,lymphoedema, boils, bursae over bony point can occur which are treated accordingly. Spu r, osteophyte formation, causalgia, jactitation of the stump, stump aneurysm, stump fractu re- are other complications.

Computer-assisted designing and computer-assisted manufacturing (CAD - CAM) socket is an automated processing method whichfulfills all above criteria accurately with modifications. It is more comfortable and is made up of thermoplastic or laminated plastic with closed cell polyethylene foam. Suspension for below knee amputation prosthesis is leather cuff strap above femoral condyles. Exo- or endoskeleton is used. For athletics endoskeleton is preferable. SACH (Solid Ankle Cushion Heel) foot is used. It is multiaxial, optimizes gait, and facilitates walking on rough ground. It needs minimal maintenance. It is preferred in old people. Energy sto ring foot is often used wherein ankle joint is replaced by a plastic spring.

I In Upper Limb

AOVANTA~ES OF PROSTHESIS

-

Cosmetic Function of the part relatively can be got Ambulation in lower limb prosthesis

I Prosthesis Types Exoskeletal prosthesis. Endoskeletal prosthesis with modular system. Internal prostheses are one used inside. They are placed by open surgery. They are nonreactive, long-durable materials, e.g. hip prosthesis in total hip replacement.

Figs. 1.600A to C: Below-knee amputee with prosthesis. It gives good range of movements, normal walk and comfort. Note: Most popular jaipur foot is designed by PK Sethi.

We make a living by what we get; we make a life by what we give.

"'C

-4

m ::c

Cl 11)

::I

.... QI 11)

.... 11) ....

'


3

...... 5·

"C C: QI

::I

-

It is the substitution to a part of the body to achieve its optimum function. (Orthroses are supplement for limb function.)

Syme's amputatiorr. Elephant boot, Canadian Syme's prosthesis. Below-knee amputation: Patellar-tendon bearing (PTB) prosthesis and solid ankle cushion heel (SACH). Above-knee amputation: Suction type prosthesis. It is placed above the stump. It is better and well-tolerated. Nonsuction type prosthesis: It is placed at the ends . It requires additional support. Hind-quarter amputation: Tilting table prosthesis (TTP) or Canadian prosthesis is used here. Patellar tendon-bearing prosthesis (PTB prosthesis): Here all the weight bearing is done below knee; movement is controlled by his own knee joint. Patellar tendon is the main key weight-bearing area within the socket; stump posteriorly up to the popliteal fossa is also important to provide counter pressure so that patellar tendon is kept in place. Medial and lateral paratibial areas and medial condylar flare also bear significant weight. Head of the fibula, tibial tubercle, cut ends of tibia and fibula are pressure intolerant areas. Socket should have proper relief areas to these intolerant parts.

l>

IC

PROSTHESIS

I In Lower Limb

n

:::c

VI C:

1. Above-elbow prosthesis is a high technology prosthesis. It is sophisticated device with harness, socket, elbow joint unit, control cable, forearm and wrist device. 2. Below-elbow prosthesis. Krukenberg's amputation does not require any prosthesis.

m

315

V,

U. Reconstruction

(iHAPTE R O UTLI NE

1

... Graft ... Skin Grafts ... Flaps ... Abdominoplasty

... Tendon

... Tendon Repair ... Tendon Transfer ... Tendon Graft

GRAFT Graft: It is transfer of tissue from one area to other without its blood supply or nerve supply.

I Contraindications SSG cannot be done over bone, tendon, cartilage, Joint.

m

PREREQUISITE

Healthy granulation area 13-haemolytic streptococci load less than 105 per gram of tissue, otherwise graft failure will occur

Technique Donor area-Commonly thigh, occasionally arm, leg, forearm; Knife used is Humby's knife; Blade is Eschmann blade, Down's blade; Using Humby's knife graft is taken, punctate bleeding is observed which says that proper graft has been obtained.

Autograft: It is tissue transferred from one location to another on the same patient. lsograft: It is tissue transfer between two genetically identical individuals, i.e. between two identical twins.

m

Allograft: It is tissue transfer between two genetically different members, e.g. kidney transplantation (Human to human) (Homograft). Xenograft: It is tissue transfer from a donor of one species to a recipient of another species (Heterograft).

,

DIFFERENT INSTRUMENTS USED TO HARVEST THE SKIN GRAFT Humby's knife Watson modification of Humby's knife Power dermatome is also used (Brown) Sterilised razor blade can be used with a specialised device to harvest small grafts under local anaesthesia

Donor area is dressed and dressing is opened after 10 days, not earlier.

SKIN GRAFTS Skin Grafting: It is transfer of skin from one area (donor area) to the required defective area (recipient area). It is an autograft.

I Types 1. PARTIAL THICKNESS GRAFT (Split-thickness skin graft-SSG) Also called as Thiersch graft, is removal of full epidermis + part of the dermis from the donor area. It may be: (1) Thin SSG; (2) Intermediate SSG; (3) Thick SSG (depending on the amount of thickness of dermis taken).

m

INDICATIONS

Well-granulated ulcer Clean wound or defect which cannot be apposed After surgery to cover and close the defect created For example: - After wide excision in malignancy - After mastectomy - After wide excision in squamous cell carcinoma Graft can survive over periosteum or paratenon or perichondrium

Figs. 1.601A and B: Split skin grafting knife and set; and harvesting.

317

... C

" l'D

:, l'D

ii.'l

Fig. 1.602: Commonest donor area for spilt skin grafting is thigh.

VI

...C: ...l'D

IQ

Recipient area is scraped well and the graft is placed after making window cuts in the graft to prevent the development of seroma. Graft is fixed and tie-over dressing is placed. If graft is placed near the joint, then the part is immobilised to prevent friction which may separate the graft. On 5th day, dressing is opened and observed for graft take up. Mercuro chrome is applied over the recipient margin to promote epithelialisation.

':':

::a:, l'D

n

0

:,

...C:

V,

..+

n

:::!'. 0

:,

I Stages of Graft Intake 1. Stage of p/asmatic imbibition: Thin, uniform, layer of plasma forms between recipient bed and graft. 2. Stage of inosculation: Linking of host and graft which is temporary. 3. Stage of neovascularisation: New capillaries proliferate into graft from the recipient bed which attains circulation later. Note:

Graft is stored at low temperatureof 4°Cfor not more than 21 days.

I Disadvantages of SSG Contracture of graft. Two types: A. Primary contracture means SSG contracts significantly once graft is taken from donor area (20- 30%). Thicker the graft more the primary contracture. B. Secondary contracture occurs after graft has taken up to recipient bed during healing period, due to fibrosis. Thinner the graft more the secondary contracture. •·· Seroma and haematoma formation will prevent graft take up. Infection; Graft failure. Loss of hair growth, blunting of sensation. Dry, scaling of skin due to nonfunctioning of sebaceous glands. So after healing, oil (coconut oil) should be applied aver the area.

I Advantages Technically easier. Wide area of recipient can be covered. To cover large area like burns wound, graft size is increased by passing the graft

Figs. 1.63A to D

Happiness is not destination, it is a manner of travelling.

318

...>... CII C"I

:::::I

V,

0

tO

:::::I

C: tO

_.,,

co a:: V,

Fig. 1.603E

Figs. 1.603A to E: Technique of split skin grafting.

rn Figs. 1.607A and B: Donor area of split skin graft in the thigh and graft placed over raw area in the leg. Fig. 1.604: Skin stapler can be used to fix the SSG to the margin of the recipient bed.

Fig. 1.605: Humby's knife with Eschmann blade.

Humby's knife Skin graft

Fig. 1.606: Harvesting a skin graft.

Figs. 1.608A to C: Mesher used in split skin grafting to increase Its surface area to cover wider area like burns wound. A large defect can be covered by this. It can cause expansion of skin up to six times.

through a Mesherwhich gives multiple openings to the graft, which can be stretched on the wider area like a net. It can cause expansion up to 6 times. Graft take up is better; Donor area heals on its own. Note: Mercurochrome/merbromin once used as a local applicant to the edge of the grafted area (SSG) and small raw areas to promote epithelialisation. It is applied once a day. But it is no longer used now.

2. FULL THICKNESS GRAFT (Wolfe Gratt)

It includes both epidermis+ full dermis. It is used over the face, eyelid, hands, fingers and over the joints. It is removed using scalpel blade. Underlying fat should be cleared off properly. Deeper raw donor area is closed by primary suturing. If large area of graft is taken, then that donor area has to be covered with SSG which is a disadvantage in full thickness graft.

B

I

,

Sensation, functions of sebaceous glands, hair follicles are retained better compared to SSG. ,. Functional and cosmetic results are better. Disadvantages , It can be used only for small areas. ,. Wider donor area has to be covered with SSG to close the defect.

B Composite graft which includes skin+ fat +other tissues like cartilage Tendon graft; Bone graft; Nerve graft; Venous graft; Corneal graft Combined graft (allograft + autograft) Reverdin graft (Jacques-Louis Reverdin, Swiss surgeon): It is a pinch graft taken from the skin and seeded in to the needed raw area.

-I

m

.... G)

11)

::::, 11)

al VI C: IO

...

... 11)

FLAPS

': ""C

-4

m

....

;JJ

'-- ~ Fig. 1.624: Waltzing of flap.

Forehead flap through anterior branch of superficial temporal artery

Bipedicle forehead flap through both anterior and posterior branches of superficial temporal artery

:

Figs. 1.625A and B: Forehead flap based on superficial temporal artery. It is fasciocutaneous flap.

Flap is rotated towards the defect area in the cheek. Inner area of the flap is covered with split skin graft. Donor flap area is covered with another split skin graft. After 3 weeks, base of the flap is disconnected; remaining proximal part of the flap can be replaced into forehead donor area. SSG over donor flap area takes up well. This flap often can be rotated under (deep to) the zygoma also. A lined forehead flap can be used. After flap elevation, under surface of the flap is lined by split skin graft prior to rotation. This grafted lined flap is resutured into the donor area for 2 weeks until undersurface of graft takes up well; after 2 weeks flap is rotated towards the defect area (cheek). Delaying of the flap is often done in forehead flap. Flap after elevation, is replaced into the original position to have optimum vascular reorientation; after 2 weeks it is again rotated towards defect. Delayed flap reduces the flap necrosis chances. Often bipedicled forehead flap (Narayanan's flap) is used taking both from anterior and posterior branches of the superficial temporal artery. This flap is moved to defect in cheek with anterior branch part staying outside and posterior part will line the mucosa! area. A different type-middle forehead flap is used for nasal reconstruction. Problems- poor color match, contraction of flap and donor area is cosmetically nonacceptable.

Deltopectoral cutaneous flap (Bakamjian flap) It is based on first three perforating branches of the internal mammary artery (mainly 2nd perforator). Flap runs horizontally across the chest wall anteriorly towards shoulder tip from its base over the sternal border. Its upper border is along the line of the clavicle; its lower border is along the line of anterior axillary fold line. Raw area often requires a spilt skin grafting. It is usually rotated upwards often with waltzing. It is tubed and attached above. Tube is drained to prevent any collection to occur.

Fig . 1.626: Deltopectoral cutaneous flap.

Great achievements begin with small opportunities.

C

324

...>...::,en QI

V'I

0

11:1

::, C 11:1

::2E , VI

co a: V'I

Rotation angle is important to prevent any kinking in the pedicle. It is usually used to cover the defects in cheek, chin, mastoid and parotid region. Often flap is delayed to get adequate length. Groin flap It is based on superficial circumflex iliac artery which is 2-3 cm below and parallel to the inguinal ligament. Artery originates from femoral artery over medial border of the sartorius and ends at anterior su perior iliac spine. 1:1 rectangular flap with deep fascia is used. Secondary defect can usually be closed with sutures. It is used mainly for defects in wrisV forearm where positioning is easier.

Figs. 1.628A and B: Pectoralis major myocutaneous flap used for carcinoma cheek.

Gastrocnemius muscle flap It is either medial or lateral and is commonly used to cover the upper part of the tibia and knee joint. It is technically easier and functional deficit occurring at donor area is insignificant. It is rarely used as myocutaneous flap. Figs. 1.627A and B: Groin flap used for burns defect in the hand. Transverse rectus abdominis muscle flap (TRAM flap) It is either superior pedicle based on the superior epigastric vessels or inferior pedicle based on the inferior epigastric Latissimus dorsi muscle/myocutaneous flap It is based on thoracodorsal artery, a branch of subscapular vessels. Superior pedicle based flap is used to cover postmasartery. Skin over the upper and anterior border of latissimus tectomy area or chest wall defect. To cover post-mastectomy dorsi is used for transfer. It is commonly used to cover the defect area opposite side superior pedicle is used to reduce the arc through which flap has to rotate (but rotating from opposite after mastectomy. But it does not give the bulk. It is technically side is technically difficult). Inferior ped icle flap is used to easier. It can be used as muscle flap also. It helps as skin cover. cover the defects in groin and thigh. Proper marking of the Prosthesis is needed to place underneath to provide bulk in flap is essential. Skin incision is made like an ellipse. Antepost-mastectomy defect. rior rectus sheath is cut in the line of incision and is raised upwards carefully of the rectus muscle up to the xiphisternum. Pectoralis major myocutaneous flap (PMMF) It is based on the pectoral branches of thoracoacromial artery. Muscle is gently separated of the posterior rectus sheath with Usually skin below and medial to nipple over the muscle is care not to injure the epigastric vessels. Once dissection is used. Muscle pedicle is made as broad as skin. It is used to complete lower part of the muscle is cut in superior pedicle cover the defect over the cheek/neck/pharynx/intraoral lesions to rotate upwards carefully. In the upper part again anterior after wide excision with removal of skin over the tumour. Vessel rectus sheath is opened to pass the flap towards the defect marking is 2 cm medial to coracoid process, obliquely below in subcutaneous plane. TRAM flap gives bulk and contour to the defect. But it is technically difficult. Usually opposite the clavicle at the junction between middle third and outer third. side of the defect is taken as flap as it is easier to rotate from Skin with muscle is dissected from the deeper structures like opposite side. Defect in the abdomen usually needs mesh to ribs, intercostal muscles and pectoralis minor. Flap is raised support and close. It is not possible to do this flap in obese upwards up to the coracoid. Lateral pectoral vessels if possible individuals and if patient has undergone laparotomy earlier are retained, otherwise can be sacrificed. Defect below is usually (with a lengthy scar). Inferior cut end of the inferior epigastric closed primarily with sutures. Often it needs spilt skin grafting. artery in superior pedicle flap can be anastomosed to a vessel Pectoralis major flap can be used along with deltopectoral flap in recipient bed to improve the perfusion (supercharging). with proper planning. Inferior epigastric artery in inferior pedicle can be additionally perfused using opposite inferior epigastric artery (recharging).

Radial forearm flap It is perfused from the radial vessels and raised on the flexor aspect of the forearm. Perforating branches of these vessels supply deep fascia and skin over it. Flap can be fasciocutaneous or osteofasciocutaneous/osteomyofasciocutaneous if radial bone is also used as part of the flap. Radial forearm free flap is commonly used for mandible defects. It is technically easier and safer. Flap is raised along with skin, segment of the radius along its intermuscular septum through which vessels pass and brachioradialis as components. Care is taken in dissecting vessels of the bed and not to injure the radial nerve. In free flap artery is sutured to the recipient artery like facial artery using microscope. Other similar flaps are-ulnar forearm flap, scapular flaps, and vascularised fibular transfer. Limberg flap It is a type of rhomboid flap used in pilonidal sinus with base at gluteal skin.

325

I

(")

:::c )>

""C

--i m :x,

....

Fig . 1.630B

C

Figs. 1.630A and B: Cross leg flap. Cl

ABDOMINOPLASTY It is reconstructive su rgery done in morbid obesity to reduce abdominal fat and skin as liporeduction procedure to maintain the abdominal contour. It is often done along with bariatric surgery. Lower horizontal lengthy incision with creation of new umbilicus is done after excising adequately and abdominal wall wound is closed. Complications are: Wound infection and dehiscence; Meleney's gangrene mainly in diabetics, fat necrosis, septicaemia.

ID

:::,

...

ID 0J

...

V'l

C 10

...

ID

'
...::::, QI

C'I

.... V'I

0 n, ::::, C n,

co"' a:

V'I

Millard cleft lip repair by rotating the local nasolabial flaps. Management of associated primary or secondary cleft palate deformity. Proper postoperative management like control of infection, training for sucking, swallowing and speech . Tenninson's 'Z' plasty (Teneninson-Randa/1 triangular flap) . Note: Delaire timing of the cleft surgery- Unilateral/bilateral cleft lip alone, in one stage operation done in 4-6 months. For cleft palate alone involving only soft palate, in one stage, surgery is done in 6 months. For cleft palate alone but involving both soft and hard palates-soft palate in 6 months; hard palate in 18 months. In combined cleft lip and palate, unilateral or bilateral, in two stages-cleft lip and soft palate in 6 months; hard palate in 18 months.

Figs. 2.5A and B: (A) Bilateral cleft lip. (B) Cleft lip with cleft lip.

Figs. 2.6A and B: Cleft lip and cleft palate in an adult.

Cleft lip lateral

Figs. 2.4A and B: (A) Lateral type of cleft lip (Type II variety- it is commonest). It is due to imperfect fusion of maxillary process and median nasal process. It can be unilateral or bilateral; (B) unilateral cleft lip (commonest). It is due to imperfect fusion of maxillary process and median nasal process.

m

PRINCIPLES OF CLEFT LIP REPAIR

"Rule of 10' should be fulfilled Before 6 months, it should be operated Infection should not be present Millard advancement flap is commonly used for unilateral cleft lip repair Bilateral cleft lip repair can be done either in a single or two stages (with 6 months gap between each stage) One stage bilateral cleft lip repair is done using Veau Ill method/ Millard's single stage/Black method Proper markings are made prior to surgery and incision should be over full thickness lip Often 1:2,00,000 adrenaline injection is used to achieve haemostasis Three-layer lip repair should be done (mucosa, muscle and skin) Cupid's bow should be horizontal

Figs. 2.7A and B: Cleft palate only. Lip is normal. Pre-maxilla is not involved.

Fig. 2.8: Steps of cleft lip repair.

Continuity of white line should be maintained

Vermilion notching should not be there

I Cleft Palate It is due to failure of fusion of the two palatine processes. Defect in fusion of lines between premaxilla (developed from median nasal process) and palatine processes of maxilla one on each side. When premaxilla and both palatine processes do not fuse, it leads into complete cleft palate (Type I cleft palate).

Figs. 2.9A and B: (A) Cleft palate with cleft lip in a child. Child also is suffering from congenital cardiac defects; (B) Complete cleft palate Type I.

341

Incomplete fusion of these three components can cause incomplete cleft palate beginning from uvula towards posteriorly at various lengths. So it could be Type II a-bifid uvula, Type II b-bifid soft palate (entire length) or Type II c -bifid soft palate and posterior part of hard palate (but anterior part of hard palate is normal). Small maxilla with crowded teeth, absent/poorly developed upper lateral incisors. Bacterial contamination of upper respiratory tract with recurrent infection is common.

n

:::c l>

"'O

-I

m ::c IV

cG

Fig. 2.12: Bilateral congenital craniofacial cleft

Cleft palate II b bifid soft palate

m Figs. 2.10A and B: (A) Cleft palate Type II a-bifid uvula; (B) Cleft palate Type II b-bifid soft palate (entire soft palate). Cleft palate II c-bifid soft palate

'"" cie~

''"''

Figs. 2.11A and 8: (A) Cleft palate Type II c-bifid soft palate entire length with cleft of posterior hard palate (anterior palate is normal). (B) Adult complete cleft palate without cleft lip.

Chronic otitis media with deafness may occur. Swallowing difficulties to certain extent and speech problems can occur. Cosmetic problems can occur.

I Treatment for Cleft Palate B

• 10 kg weight • 1Omonths of age (10- 18 months)

(Courtesy Dr Sathish Bhat, Plastic Surgeon, Mangaluru).

Cleft palate is usually repaired in 12- 18 months. Early repair causes retarded maxillary growth (probably due to trauma to growth center and periosteum of the maxilla during surgery if done early). Late repair causes speech defect. Both soft and hard palates are repaired. Abnormal insertion of tensor palati is released. Mucoperiosteal flaps are raised in the palate which is sewed together. If maxillary hypoplasia is present, then osteotomy of the maxilla is done. With orthodontic help teeth extraction and alignment of dentition is needed. Regular examination of ear, nose and throat during follow up period. Postoperative speech therapy. Whenever complicated problems are present, staged surgical procedure is done. Wardi/1- Kilner push back operation-by raising mucoperiosteum flaps based on greater palatine vessels. Secondary management: ,. Hearing support is given using hearing aids if defect is present; control of otitis media. ,. Speech problems occur due to velopharyngeal incompetence; articulation problems also can occur-speech therapy is given. It is corrected by pharyngoplasty, veloplasty, speech devices. ,. Dental problems like uneruption, unalignments are common. They should be corrected by proper dentist opinion, and reconstructive surgery. ,. Orthodontic management with alveolar bone graft, maxillary osteotomy-done in 8-11 years of age. ,. Veloplasty, dental implants, rhinoplasty, orthognathic surgeries, etc.

B Timing is between 10-18 months Mucoperiosteum flap is raised \ Palatal defect is closed using 3 layers- nasal, muscle and oral layers Hook of pterygoid hamulus is fractured to relax tensor palatej muscle to relieve tension on suture line

-- ---------- ----

Bleeding gums in uraemia are not so spongy as in scurvy: in fact, they may look nearly normal.-Frederic J Wright

342

...>, ...::ICl (I/

Ill

0

ro

::I C:

ro

"' cc a:

Ill

Cleft palate repair V-Y palatoplasty

Cleft palate with palatine artery

Fig. 2.13: V-Y palatoplasty.

MAXILLOFACIAL INJURIES It may be due to road traffic accidents, assaults, bullet injuries or sport injuries.

I Classification Fracture in maxillofacial region can be grouped as: Fracture lower third that comprises mandible. Fracture middle third that comprises maxilla, zygoma and nose. Fracture upper third of the face involving part of the orbit, frontal bones.

Fractures of the face which do not involve the dental occlusion-fractures of zygoma and nose. Fracture which involves the dental occlusion-fracture mandible and maxilla.

Frontal bone

Occlusal plane

...I

_.J

Mandible

Soft Tissue Injuries Lacerations, contusions, cut wounds, etc. Eyelid injuries with black eyes. Facial nerve injury. Primary repair is required. Parotid duct injury. Here primary anastomosis of the injured duct is done, with a fine polythene cannula is kept as a stent inside the duct which will be removed in 14 days. Lacrimal apparatus injury. Here the duct is sutured with a fine nylon thread in the canaliculus which is kept for 3 months.

Injuries to the Facial Bones

Maxiflofacial fracture also can be grouped as:

Fig. 2.14: Relation between middle third and cranium in 45° plane.

Fig. 2.15: Posterior gagging of occlusion due to backward displacement of fracture segment in middle third fracture.

Fracture nose: Nasal bones are most commonly injured bones in face. Patient presents with pain and swelling in the nose with deviation and displacement. Here reduction of the fractured nasal bones and nasal septum under general anaesthesia is done. Later position is maintained by nasal packs from inside (which is removed in 7 days) and by a nasal plaster from outside (which will be kept for 14 days). Procedure is done using Walsham's and Asch's forceps. Injuries to the maxilla. Zygomatic bone injuries. Mandibular bone fracture and mandibular dislocation. Orbital bone fracture-. Presents with diplopia, enophthalmous, sensory loss in the area of infraorbital nerve. lnfraorbital ecchymosis of the orbit is called Panda sign. Features

, Localised swelling due to haematoma. ,, Facial oedema; Bleeding with open wounds. , Asymmetry which is clinically confirmed by observing supraorbital ridges, nasal bridge. ,, Localised tenderness; Step deformity; Trismus; Diplopia. , Features of associated injuries like intracranial, abdominal or thoracic injuries. Investigations: X-ray face; CT scan of head and jaw.

Suturing of soft tissues Control of infection Airway maintenance Treating the individual Control of bleeding fractures • Pain relief ' - - - - - - - - - - - - - -- ~ - ~ - -

PRIMARY CARE (EARLY CARE) IN MAXILLOFACIAL INJURIES Injury can be isolated as single bone fracture or multiple bone fractures. Real primary care is usually not required except when there is mechanical respiratory block causing airway obstruction.

abdomen or other areas, or they may interfere with pupillary reaction and neurological signs in the presence of intracranial injuries. Antibiotics are needed. Tetanus toxoid and often antitetanus globulin (ATG 3000 units IM) are required.

I Haemorrhage in Maxillofacial Injuries Haemorrhage in maxillofacial injuries is usually not life-threatening. But it should be identified and controlled properly. In association with other internal injury, such haemorrhage may be important to cause the circulatory failure.

I Respiratory Obstruction

Haemorrhage may be due to: Soft tissue bleeding. Bleeding from inferior alveolar artery, palatine vessels. Nasal bleeding.

Causes

I Control of Bleeding

Oronasal airway block can occur by blood clot, vomitus, foreign body, dentures, teeth, saliva, bone pieces, etc. Backward falling of tongue can cause obstruction of the nasopharynx and oropharynx. It is common in bilateral mandibular fracture. Occlusion of the nasopharynx and oropharynx can occur in fracture maxilla with posterior and inferior displacement. Haematoma in floor of the mouth or posterior oral cavity can cause airway block. Oedema of larynx/tongue/posterior third of oral cavity/ pharynx. Surgical emphysema.

Treatment Cleaning of the oral and nasal cavities to remove obstructing agents like clot, dentures, teeth or bone. Gauze swabbing and suction. Fallen tongue should be placed forward using finger and often temporary alignment of the occlusion may be needed. Maxillary disimpaction is done when needed in fracture maxilla. Positioning of the patient is important. Prone/semiprone position with head towards one side is the safest position. If this is not possible, then patient may be placed in sitting position which also improves the breathing. Placing the patient flat on his back in supine position should be avoided as much as possible. Tracheostomy should be done when needed without delay as it will be life-saving by facilitati ng the easy airway and breathing.

I Control of Pain and Infection Analgesics like NSAIDs are used to control pain. Morphine and analogues are not used as they may suppress the respiration. They may mask the pain of alarming severe injury in chest,

Blood transfusion, IV fluids, resuscitation. Nasal packs; Fracture correction; Ligation of the bleeder. Cauterisation; Packing the area. Under running the bleeding field; Embolisation. External carotid artery ligation above the level of the origin of the superior thyroid artery. fractures not involving occlusion Central

• Fracture nasal bones and/or nasal septum • Fracture of frontal process of maxilla • Fractures of above two extending into ethmoidnasoethmoid • Fractures above three which extend into frontal bone-fronto-orbito-nasal dislocation

Fractures involving occlusion Dentoalveolar Subzygomatic

• Le Fort-I-low level either unilateral or bilateral • Le Fort II-pyramidal either unilateral or bilateral Suprazygomatic

• Le Fort Ill- high level • Craniofacial disjunctionunilateral or bilateral

Lateral

• Fractures involving zygomatic bone, arch and maxilla excluding the dentoalveolar component Le fort classification

(Rene Le Fort- French surgeon classified these fractures by dropping rocks on the face of the cadavers and later dissected the area for study and research and published paper in 1911 ) Types Le Fort I (Guerin's fracture/ow level) (floating fracture, horizontal fracture of maxilla)

Features

• Bleeding from nose • Posterior gagging of occlusion • Upper lip swelling • Palatal ecchymosis • Occlusion derangement • Floating maxilla

If you like me I am your heart; if you hate me I am in your mind. - Swami Vivekananda

344

I Associated Injuries

• It runs horizontally above the floor of the nasal cavity involving lower third septum, palate, alveolar process of maxilla and lower third of pterygoid plates of maxilla Le Fort II (pyramidal fracture)

• Oedema of middle third face • Both sides circumorbital and subconjunctival ecchymoses

• From the nasal bones at top-most, fracture runs laterally towards lacrimal bones, medial wall of orbit, infraorbital margin, through medial to infraorbital foramen and backwards below the zygomaticomaxillary area through lateral wall of maxillary sinus and pterygoid plates. Zygoma is intact with skull base

• Nasal bleeding/obstruction/ deformity • Deformity of face (dish face), diplopia • Retroposition of maxilla with posterior gagging • Limitation of ocular movements, CSF rhinorrhoea • Tenderness and separation of infraorbital margin

Le Fort Ill (craniofacial disjunction, high level)

• Lengthening of face • Enophthalmos, ocular level depression • Here fracture runs parallel to • Hooding of eyes, occlusal skull base. It passes through plane tilting the nasal bone, lacrimal • Entire facial skeleton moves bone, ethmoid bone, optic as a single block foramen, inferior orbital • Tenderness and separation fissure, pterygomaxillary of suture line fissure and lateral orbital wall with frontozygomatic suture • Diplopia • Trismus, teeth mat-alignment with zygomatic arch Guerin sign: Haematoma at greater palatine foramen

Dentoalveolar fracture

All associated injuries should be assessed properly and individually. On priority basis it should be treated. Soft tissue injuries. Cranial injuries. Orbital injuries. Intra-abdominal/thoracic/pelvic injuries.

FRACTURE MIDDLE THIRD AREA It includes: Maxillae, zygomatic bones, palatine bones, nasal bones, lacrimal bones, inferior conchae (one on each side). The vomer, ethmoid and its attached conchae, pterygoid plates of sphenoid. Note: Fracture middle third includes fracture maxilla, zygomaand nasal bones.

I Features Oedema face, subconjunctival haemorrhage, ocular ecchymosis. Bleeding from the nose. Diplopia due to trapping of the extra-ocular muscles in the fracture segments. Anaesthesia of the cheek. Trismus and malalignment of teeth. Guerin 's sign: Haematoma at greater palatine foramen. Always patient should be examined and observed for CSF leak and intracranial injuries. Investigations: CT scan head; X-ray skull.

Le Fort fracture I

Fig. 2.17: Fixation of the splint to skull. It is often used with gunning

splints to fix it to skull.

l'!I

Le Fort fracture II

Le Fort fracture Ill

Figs. 2.16A to D: Le Fort classification-different types and also dentoalveolar fracture (Refer table for details).

Treatment , ,

It should be managed in a center for maxillofacial injuries. Antibiotics.

, ,. ,. ,.

Tracheostomy. Associated zygoma and nasal fractures are reduced first. Direct wire suturing of the zygomaticofrontal region. Fixation of teeth in occlusion using eyelet wires, bars or cap splints. , Once reduced , fracture bones are immobilised using extra-oral rods called as Mount Vernon box frame. ,. Initially intravenous fluids and blood transfusions are required. Later Ryle's tube feeding is done. , Proper ophthalmic consultation is necessary when there are orbital injuries.

Fig . 2.18: Photograph showing bilateral maxillary fracture.

I Features

345

Swelling and bruising in the cheek with subconjunctival haemorrhage. Flattening of the cheek prominence. Step in the margin of the bony orbit at the infraorbital foramen. Sensory loss over the supply of the branches of the superior orbital nerve- teeth on the affected area are anaesthetic on percussion. Sensory loss over the supply of the infraorbital nerve usually over infraorbital region, upper lip and alar region of the nose-common. Enophthalmos is due to herniation of the orbital fat across the fracture floor of the orbit into the antrum. Diplopia is due to entrapment of the inferior rectus muscle preventing upward rotation of the eyeball while looking up. Trismus with marked restriction of the lateral movements. Epistaxis, lowering of pupil level. lnfraorbital ecchymosis of the orbit is called as Panda sign. Investigations , 30° occipitomental X-ray is used commonly but often obliquity of X-ray may be increased to 60°. In X-ray, findings observed are: Fracture line near infraorbital foramen, zygomatic arch and lateral wall of the antrum. Fracture site (undisplaced)

ZYGOMATIC COMPLEX FRACTURE

I Classification Simple fracture which is stable and undisplaced- here fracture line passes across the infraorbital foramen downwards over anterior wall of the antrum. Simple fracture which is displaced medially. It may be associated with rotation/tilt in vertical axis, either medial tilt or lateral tilt. lnfraorbital nerve may get compressed or branches of superior dental nerve may get torn . Unstable fracture with rotation around horizontal axis with medial tilt or lateral tilt. Comminuted fracture extending into the floor of the orbit. Fracture of the zygomatic arch causes a localised depression of the arch which displaces medially and tends to impinge on the coronoid process of the mandible. 'Blow-out' fracture of the orbit is due to direct blunt trauma on the eyeball causing depressed comminuted fracture of the orbital floor with herniation of the orbital fat into the antrum. Enbloc dislocation of zygomatic bone medially/inferiorly/ posterolaterally.

Maxillary sinus

Figs. 2.19A to C: {A and B) Diagrams showing different types of zygomatic fractures, (C) Blow-out fracture.

Ankylosis of mandible joint causes receding of chin giving a characteristic shrew mouse profile. -Leon Dufourmentel

346

Orbital floor line for fracture. - Opacity in the antrum due to blood. ,, CT scan is done to see orbital depression and herniation of orbital fat.

Treatment ,, Every patient with zygoma fracture need not require surgical correction. ,, Need for surgery is decided based on clinical features.

B lnfraorbital anaesthesia, trismus Oiplopia, enophthalmos Flattening of the cheek Undisplaced fracture with infraorbital anaesthesia

I Surgical Approaches 1. Closed reduction of the zygomatic arch through Gillies temporal approach: Disimpaction forceps

Figs. 2.21A and B: Disimpacti on of maxilla using Rowe's disimpaction forceps by downward leverage action.

Temporalis muscle

Fig . 2.20: Technique showing method of temporal reduction using disimpaction forceps.

An oblique skin incision of 2 cm length temporal is made between the two branches of the superficial temporal artery. Care is taken to avoid injury to artery. Whitish glistening temporal fascia is identified and incised . Zygoma elevator is introduced beneath the zygoma and fracture fragments are manipulated and elevated into proper position. An audible snap is heard when fracture gets reduced into position. Reduced, disimpacted fracture is always stable. Additional corrections in other parts can be done by different leverage actions of the elevator. Orbital rim and zygomatic arch are palpated for completion of correction. Skin wound is closed with sutures. Elevators used are Bristow's periosteal elevator, Rowe's zygomatic elevator. 2. Internal fixation by open reduction and fixation is needed , When fracture is unstable or ,, Comminuted or , Zygoma fracture with middle third fractures. By proper incisions, infraorbital and zygomaticofrontal fracture sites are exposed; after open reduction, they are fixed using wires/plates and screws.

Fig. 2.22: Fracture zygoma showing open reduction and fixation using wires directly. Two types of incisions are shown depending on the site of the fracture.

3. Exploration of the orbital floor is necessary whenever there is ,, Comminuted fracture in orbital floor. , Orbital fat herniation. , Diplopia with entrapment of the inferior rectus muscle.

FRACTURE OF THE MANDIBLE -~~---• Types I. At the neck of the condyle (35% ), as it is the weakest point. The condyle is displaced in front and medially often with dislocation. Painful jaw movement is the clinical features. It may be unilateral or bilateral. II. At the angle of the mandible: If fracture is upwards and inwards, it is impacted and undisplaced. So it is a favourable fracture. If fracture is downwards and outwards, it gets displaced and so it is an unfavourable fracture. It needs open reduction using wires.

347

n

:::c )> ""0

Medial pterygoid

-i

m

::x, N "Tl

DJ

'"' o·

Fig. 2.23: Types of mandibular fractures.

3

~DJ

Ill. Fracture near the mental foramen through the canine fossa. This fracture causes displacement. Such bilateral fractures can cause pull on digastric and geniohyoid muscles precipitating fall of tongue backwards which will block the airway.

...

'< 0

Fracture site

"'

/1)

I Other Classifications T

DJ

"' /1)

"'

Classification of the fracture mandible

Depending on the type

Depending on the anatomical site

• Simple

• Dentoalveolar fracture

• Compound

• Condylar fracture

• Comminuted

• Coronoid fracture

• Pathological

• Fracture ramus of the mandible

• Green slick fracture in children

• Fracture angle of the mandible

Fig. 2.24: Muscle actions in mandible fracture causing different displacements. Fracture unreduced

Fracture reduced

• Fracture in the body of the mandible • Symphyseal region fracture

Guardsman fracture is direct fracture of symphysis and indirect fractures of both the condyles of the mandible. In olden days guards of the queen who are in attention position used to faint and fall forward to get these fractures.

T

Teeth not occluded

Fig. 2.25: Unreduced and reduced fracture mandible.

Dentoalveolar fracture

f,atures • Horizontal fracture below the alveolar margin

Teeth well occluded

Fracture coronoid

Management • Look for other injuries in face

• Dentoalveolar segment will be • X-ray face to see injuries freely mobile • Tooth may get split vertically/ • Dentoalveolar segment reduction and placing jaws in horizontally central occlusion position • Derangement in occlusion and alignment

• Stabilisation using inter-

• Gingival laceration • Bleeding • Infection and late osteomyelitis of mandible

• Liquid diet for 3--4 weeks

dental wires or arch bars

Condyle fracture

Ramus fracture

Symphyseal fracture

Body fracture Angle fracture

Fig. 2.26: Different sites of fracture mandible.

I Clinical Features Pain and tenderness in the lower jaw with bruising over the surface.

The first two letters of goals are go.....

348

• Simple arch bar fixation

Erich arch bar fixation with cleats

Fig. 2.27: Arch bar wiring. Figure shows both simple and Erich arch bar wiring with cleats to pass wire.

Haematoma in the floor of the mouth is called as Coleman's sign. Difficulty in opening the mouth, speech and swallowing. Anaesthesia of the lower lip due to compression of inferior dental nerve. Deranged dental occlusion. Step deformity. Investigations: X-ray of the mandible; Orthopantomogram (OPG), CT head and face. Treatment ,. Antibiotics to prevent formation of osteomyelitis of the mandible. ,, Open fixation of the fracture segments using silver wires for 4-6 weeks. ,. Fixation by: lnterdental wiring; Using arch bars; Silver alloy or plastic caps. Only fluid diet for 6 weeks. Irrigation wash to the oral cavity to maintain the hygiene. Complications of fracture mandible: Obstruction of the airway; Osteomyelitis of the mandible; Trismus; Speech disturbances

DISLOCATION OF THE MANDIBLE It occurs at temporomandibular joint. Unilateral dislocation after trauma is common.

Upper border wiring

Fig. 2.28: Circummandibular wiring used in gunning splints.

~~( '"\-')~- ~ ' ~/J

'--- -A ; - ,, r

J../

'

Fig. 2.29: lnterdental wiring. It is commonly used and accepted method of wiring.

Bilateral dislocation occurs during yawning and it is recurrent. Clinical features are difficulty in opening the mouth with pain and tenderness over the joint. Treatment: Reduction of dislocation under general anaesthesia; If there is associated fractu re mandible, it should be dealt with accordingly.

Lower border wiring

Fracture site

Fracture site

Fig. 2.30: Upper and lower border wiring. It is used to fix the mandibular fractures and is often done together with other fracture fixations in the face.

349

Fracture mandible

Compression plate fixed

,, QI

n

o·

3 QI X

Axial compression

Fig . 2.31: Compression plating of a mandibular fracture. Note the different methods.

B Mandibulofacial dysostosis Hypoplasia of the zygomatic bone and mandible Antimangoloid slant to the palpebral fissure Coloboma of lower eyelid • Low ear lobule with deficient middle ears Familial-3rd arch syndrome (Mandibulofacial dysostosis)

,.- .. .

•-,

-

Fig. 2.32: Microtia which is corrected later (Courtesy. Dr Sathish Bhat, Plastic Surgeon, Mangaluru).

JAW TUMOURS The term 'Jaw tumours' is a gross terminology which denotes any tumou r which arises from jaw either benign or malignant; from upper or lower jaw; from any tissues (layers) of the jaw from mucosa to soft tissues. Swelling arising from the gums (Epu/is): Congenital epulis, Fibrous epulis, Pregnancy epulis, Giant cell epulis, Myelomatous epulis, Sarcomatous epulis, Carcinomatous epulis.

Swelling arising from the dental epithelium and ectomesenchyme (Odontomes): Ameloblastoma, Compound odontome, Enameloma, Cementoma, Dentinoma, Odontogenic fibroma and myxoma, Radicular odontome, Composite odontome. Cysts arising in relation to dental epithelium: Dental cyst, Dentigerous cyst. Swelling arising from the mandible or maxilla: Osteoma and osteoblastoma, Torus palatin us and mandibularis , Fibrous dysplasia, Osteoclastoma (Common in mandible), Osteosarcoma; Secondaries; Giant cell reparative granuloma . Surface tumours: Tumours from the surface which extend into the jaw-Ossifying fibroma, Osteofibrosis of maxilla, Ivory osteoma of jaw, Leontiasis ossea (diffuse osteitis), Carcinoma extending into the jaw. It can be odontogenic or non-odontogenic. Odontogenic tumour can arise from (A) odontogenic epithelium like-(1) Ameloblastoma; (2) Pindborg's tumour; (3) Clear cell; (4) Squamous cell type. (8) from odontogenic epithelium and ectomesenchyme like-(1 ) Ameloblastic fibroma; (2) Adenomatoid odontogenic tumour; (3) Compound odontome; (4) Complex odontome. (C) From odontogenic ectomesenchyme like-(1) Odontogenic fibroma; (2) Myxoma; (3) Benign cementoblastoma.

Nonodontogenic tumours are classified as- (A) Ossifying neoplasm like cementa-ossifying fibroma. (B) Nonneoplastic bone lesions like fibrous dysplasia, cementa-ossifying dysplasia. (C) Cementa-osseous dysplasias like cherubism, central giant cell granuloma. (D) osteoma, osteoblastoma, osteoclastoma, osteosarcoma. Other lesions like haemangioma, neurofibroma also can occur in jaw. Pindborg's tumour is calcifying epithelial odontogenic tumour (CEOT). It arises from epithelial remnant of enamel; it is common

Many receive advice, only wise profit from it.

350

Figs. 2.33A to C: Lower jaw tumour with images.

Figs. 2.34A and B: Lower (B) and upper (A) jaw tumours in two different patients.

in mandibular molar. 50% or more arises from unerupted tooth. It presents as painless slow growing jaw tumou r. Image shows scattered flaks of calcification with driven snow appearance. It can be uni or multilocular lesion. Treatment is wide excision; shows 15% recurrence. Investigations: Open incision biopsy is preferred. CT of the part; MRI to evaluate soft tissues. CT chest and abdomen are done in malignant cases to assess metastases. Treatment: Wide excision with part of the bone with 2 cm clearance. Reconstruction is needed with bone graft and reconstruction prosthesis and flaps. Maxillectomy, mandibulectomy are often needed depending on location , size and extent.

EPULIS (GREEK-MEANS UPON GUM) Swelling arising from the mucoperiosteum of gums (gingiva). It is gross terminology but still term is used in many conditions.

Fig. 2.35: Epulis.

I Types Congenital Epulis: It is a benign condition seen in a newborn arising from gum pads (Neumann's tumour). It is a variant of granular cell myoblastoma originating from gums. It is more common in girls. It is more common in upper jaw, common in canine or premolar area. It is not a malignant condition. Clinical features are-Well localised swelling from the gum which is firm and bleeds on touch. Treatment: Excision. Fibrous Epulis: It is a benign condition, can occur in any individual. It is red, firm/hard, sessile/peduncu lated. It is the commonest type. It is fibroma arising from periodontal membrane. Clinical features are-Painless, well localised, hard, non-tender, grey pink swelling in the gum which bleeds on touch. Differential diagnosis: Squamous cell carcinoma

from the gum. lnvestigations-X-ray jaw, Orthopantomogram, Biopsy from the lesion. Treatment: Excision with extraction of the adjacent tooth. Recurrence can occur if root is not removed properly. Pregnancy Epulis: It occurs in pregnant women du... to inflam matory gingivitis. Usual ly, it occurs durin g 3rd month of pregnancy. Clinically, it resembles fibrous epulis or pyogenic granuloma. It usually resolves after delivery. Otherwise, it should be excised. Epulis fissuratum: It is a benign hyperplasia of fibrous tissue developing as a reactive lesion to chronic irritation to ill-fitting dentures. Myelomatous Epulis: It is seen in leukaemic patients. It is investigated for leukaemia by peripheral smear, bone marrow biopsy. Treatment is for leukaemia. Granulomatous Epulis: It is a mass of granulation tissue in the gum around a caries tooth. It forms a localised soft/firm/ fleshy mass in the gum which bleeds on touch. Giant Cell Epulis: Osteoclastoma causing ulceration and haemorrhage of gum. Carcinomatous Epulis: Squamous cel l carcinoma of the alveolus and gum presenting as localised, hard, indurated swelling with ulceration. Fibrosarcomatous Epulis: Fibrosarcoma arising from fibrous tissue of the gum.

351

'Tl QI

I"\

o· 3

~-

QI

... '< QI

Fig. 2.36: Large jaw tumour-could be adamantinoma/ osteoclastoma.

Carcinomatous Myelomatous Fibrosarcomatous

11)

V,

Fig. 2.37: Orthopantomogram (OPG) X-ray showing unilocular odontogenic tumour in lower jaw.

OOONTOGENIC TUMOURS Epithelial tumours Ameloblastoma Calcifying odontogenic tumour Odontogenic adenomatoid tumour Composite odontoma, which may be either complex or compound. It is odontogenic hamartoma contains all 4 layers, dentin, enamel, cementum and pulp Mesodermal tumours Odontogenic fibroma, myxoma Cementoma, dentinoma Malignant odontogenic tumours Malignant ameloblastoma Fibrosarcoma

AMELOBLASTOMA (Adamantinoma, Eve's Disease, Multilocular Cystic Disease of the Jaw) It arises from the dental epithelium probably from the enamel/ dental lamina. It occurs commonly in mandible (5:1) or maxilla. Occasionally, it is seen in the base of the skull in relation to Rathke's pouch or in tibia. It is a locally malignant tumour. Histologically, it is a variant of basal cell carcinoma. It neither spreads

11) QI

V,

B Congenital Fibro us-commonest Granulomatous Pregnancy

9.

V,

Figs. 2.38A and B: X-ray (two different X-rays) showing typical honeycomb/multiloculated features of adamantinoma (Courtesy: Dr Veena Jagadish, MOS).

Rudeness is a weak imitation of strength.

352

through lymph node nor through blood. Hence it is curable. It is usually unilateral. It can occur in a pre-existing dentigerous cyst. It is usually multilocular cystic spaces but can be unilocular also. Histologically cords of odontogenic epithelium, connective tissue, stellate reticulum like cells with columnar ameloblast like cells.

I Features Swelling in the jaw usually in the mandible near the angle which attains a large size, extending to vertical ramusEggshe/1 crackling. It is a gradually progressive, painless swelling which is smooth and hard with intact inner table (enlarges externally). Lymph nodes are not enlarged. Outer table expansion. It is common in males, common in 4th to 5th decades. Differential diagnosis , Osteoclastoma of the mandible: Here inner table is not intact. , Dentigerous cyst; Dental abscess. , Giant cell reparative granuloma (Jaffe's tumour): It is a swelling which occurs due to haemorrhage within the bone marrow. It contains vascular stroma, collagen and connective tissue cells. It is common in women. It causes painless enlargement of jaw. It can be treated by calcitonin (100 units/0.5 mg subcutaneously daily for 12 months) or surgical curettage. Investigations: ,. Orthopantomogram (OPG) shows multiloculated lesionHoneycomb appearance. CT scan of the region in ideal. , Biopsy from the swelling. Treatment: Segmental resection of the mandible. OR Hemimandibulectomy with reconstruction of the mandible.

Figs. 2.39A and B: (A) Dentigerous cyst; (B) Orthopantomogram showing dentigerous cyst.

Complication: It can turn into adamantinoma. •·· Investigation: Orthopantomogram. Tooth within the cyst, which is well-defined. Treatment , If it is small, excision of the cyst is done. , If it is large, initial marsupialisation and later excision is done. , Unerupted tooth should be extracted.

DENTAL CYST (Radicular Cyst, Periapical Cyst) It occurs under the root of the chronically infected dead erupted tooth. It is lined by squamous epithelium derived from epithelial debris of Mallassez. Clinical feature: As a smooth, tender swelling in the jaw in relation to caries tooth which causes expansion of the jaw bone.

Note: • Curettage and bone grafting should not be done. It is a curable condition. • Recurrent adamantinoma can spread through blood into lungs.

DENTIGEROUS CYST (Follicular Odontome) It is a unilocular cystic swelling arising in relation to the dental epithelium from an unerupted tooth. Common in lower jaw, but can also occur in upper jaw. It occurs over the crown of unerupted tooth. Commonly seen in relation to premolars or molars. It causes expansion of outer table of the mandible. Clinical feature: Pain less swelling in the jaw which is smooth and hard. Differential diagnosis: Adamantinoma; Dental cyst; Osteoclastoma

Fig. 2.40: Dental cyst with orthopantomogram x-ray.

Complication: It can cause osteomyelitis of the jaw. Differential diagnosis: Dentigerous cyst. Investigation: Orthopantomogram. Treatment: Antibiotics; Drainage or excision of the cyst with extraction of the infected tooth is done.

Verrucous carcinoma

Papillary carcinoma thyroid Marjolin's ulcer Carcinoma colon

T

Differences between dental cyst and dentigerous cyst

a. Site of occurrence b. Infection c. Complication d. Treatment

Dental cyst

Oentlgerous cyst

Erupted tooth under the root Common Osteomyelitis Excision and extraction of tooth

Over the crown of an unerupted tooth Not common Adamantinoma Marsupialisation, excision and then extraction of tooth

OSTEOMYELITIS OF JAW It is an inflammatory process in jaw; acute or chronic. It can be in the maxilla or mandible. Causes: , Alveolar abscess leading into osteomyelitis. , Recurrent dental infection; Trauma. , After dental extraction; surgeries of the jaw. , Postradiotherapy osteomyelitis (osteoradionecrosis).

Management , X-ray jaw; CT scan of jaw; discharge study; ESR are essential investigations. Biopsy from the sinus is needed often. , It is often difficult to treat. In acute phase, antibiotic coverage, treatment of cause is done. In chronic type, sequestrectomy, mandibulectomy is needed.

I Actinomycosis of Jaw (Refer Chapter 1D) Faciocervical is the commonest type; lower jaw is commonly involved; infection begins at carious tooth; indurated gums multiple discharging sulphur granules with normal X-ray (Ray fungus). Actinomycosis israelii is the causative agent. It is treated by penicillins.

ALVEOLAR ABSCESS (Dental Abscess) It is due to spread of infection from root of the tooth into the periapical tissue. Initially, it forms periapical abscess which later spreads through the cortical part of the bone into the soft tissues around forming an alveolar abscess.

I Types Acute is common in children; maxilla or mandible may get involved; swelling, redness, fullness are the features; pus may trickle through nostril if it is in maxilla. Subacute type is the commonest type; com mon in adult; apical sepsis, endarteritis, bone necrosis is the pathology; common in mandible; rare in maxilla due to existing network vasculature which prevents endarteritis. Compression over inferior dental nerve causes numbness in chin in area of distribution of mental nerve. Pain, swelling, tenderness, irregularity, bone thickening are typical. Chronic type is also common in mandible; apical abscess, alveolar abscess, trauma, radiation, chemicals like phosphorus, tuberculosis, syphilis, actinomycosis are the causes. Pain, bone thickening, irregularity, discharging sinus, sequestrum in the discharge, discomfort are the features. Infection from lower incisor causes median mental sinus. X-ray shows features of osteomyelitis with new bone formation and sequestrum.

Fig. 2.42: Tooth infection causing large dental abscess. It needs antibiotics, proper drainage and tooth extraction. Patient may develop trismus/retropharyngeal infection/chronic osteomyelitis of the mandible.

Disease begins in the pulp of tooth pulpitis spread to root localized osteitis abscess formation spread into soft tissues outside in initially diffused later localised swelling in the jaw with redness and oedema of gum. Initial dull continuous pain later becomes severe excruciating pain. Bacteria: Staphylococci, streptococci, anaerobic-bacteria and gram-negative organisms.

I Features Fig. 2.41: Osteomyelitis of the mandible. Discharging sinus is obvious.

353

Deep, throbbing pain in the jaw and adjacent oral cavity with diffuse swelling over the cheek. Tender soft tissue swelling in the jaw which eventually bursts spontaneously leading to sinus formation.

Open confession is good tor the soul.

-n Qj

0. 0

3 Qj

~. iii ....

'< 0

;:;;· (I) Qj

"' (I) "'

354

Oedema, pain and tenderness in the floor of the mouth. Trismus and dysphagia. Fever and features of toxaemia. Tender palpable lymph nodes in the neck. Investigations: X-ray of the mandible or maxilla; Discharging pus for culture study; CT mandible.

B Septicaemia. • Spread of infection into other spaces like parapharyngeal spaces; sublingual and submandibular spaces causing Ludwig's angina; oedema of epiglottis and respiratory distress; spread to pterygoid space and along pterygoid muscles through emissary vein cavernous sinus thrombosis; upper canine tooth abscess medial corner of eye angular vein thrombophlebitis cavernous sinus thrombosis; submasseteric abscess. Lower incisor abscess can cause abscess in the chin and later median mental sinus; chronic osteomyelitis of the jaw with discharging sinuses. Osteomyelitis is common in mandible - horizontal process near the mentum, presenting with pain, swelling, discharging sinuses, bone thickening, loose tooth, and trismus. Sequestrum is commonly seen. It is treated by antibiotics, sequestrectomy, mandibulectomy. • Treatment: Antibiotics, sequestrectomy, mandibulectomy.

Treatment: Antibiotics; Drainage of the abscess under general anaesthesia; Extraction of the tooth at a later period; Excision of the sinus whenever required.

FIBROUS DYSPLASIA OF BONE/JAW It is ben ign self-limiting non-capsulated lesion of bone wherein normal bony architecture is replaced by collagen, fibroblasts, osteoid and calcified tissue. It is often classified as benign tumour with localized developmental arrest, with bone being not differentiated into a mature bone tissue. It is seen in childhood and adolescents.

I Types It may be polyostotic or monostotic. Condition can occur in long bones, ribs and jaw bones, either mandible or maxilla. Disease is either metaphyseal or in the shaft, never in epiphysis.

1. Monostotic (70%) It is equal in both sexes. It occurs in children and adolescents; stops once growth plate is closed. Femur is the commonest bone involved; tibia, ribs, jaw bones, skull and humerus can get involved. It can present as asymptomatic diffuse hard bony swelling or can be painful due to fracture. Discrepancies of the part with asymmetry are common. ,:. Monostotic will not turn into polyostotic type. Monostotic will not turn into sarcoma.

2. Polyostotic Fibrous Dysp/asia (27%) without Endocrine Dysfunction It begins in earlier age group than monostotic.

It is common in femur, skull, tibia, humerus, ribs, fibula, radius, ulna, mandible and vertebrae. Craniofacial bones are involved in more than 50% of patients. It may continue to grow in adulthood (progressive). There is no evidence of hyperparathyroidism. It should be differentiated from primary hyperparathyroidism of bone. Involvement of shoulder and pelvis causes severe deformity. Severe involvement of femu r causes 'shepherd crook' deformity. Recurrent spontaneous fractures are common. Polyostotic occasionally turns into sarcoma.

3. Polyostotic Fibrous Dysplasia with Endocrinopathies (3%) Polyostotic fibrous dysplasia with skin pigmentation ( Cafe au lait, on same side of the disease in neck, chest, back, shoulder, pelvis, larger) with sexual precocity in females (McCune Albright's syndrome); often with hyperthyroidism, growth hormone secreting pituitary adenoma and primary adrenal hyperplasia is 3% common. It is due to mutation of guanyl nucleotide binding protein gene (GNAS gene). Fibrous dysplasia is most common in femur-Shepherd Crook deformity; metaphyseal • In the jaw, mandible is the common site, vertical ramus, outer table expansion • Monostotic is more common • Polyostotic is more problematic-discrepancies, pathological fracture, sarcoma changes I •• Monostotic ceases with cessation of growth Surgery should never be done during growing period

I Fibrous Dysplasia of Jaw In the jaw, it can occur in maxilla or mandible; but mandible is more common site. It presents with diffuse swelling of vertical ramus of the mandible or maxilla. Gritty white, hard cartilages with cysts are the pathology. Diffuse hard, painless swelling which causes asymmetry is the usual presentation. It progresses with the growth of the bone. It is commonly monostotic but can be polyostotic. Monostotic ceases once bone develops completely. Polyostotic may continue to grow. Teeth are normal. Expansion is towards outer cortex of the mandible. Polyostotic occasionally turns into sarcoma (but not monostotic). Complications of fibrous dysplasia: Deformity and cosmetic problems; Pathological fractures; Sarcomatous transformation in polyostotic type only.

Differential diagnosis: Osteoclastoma, adamantinoma; Osteitis fibrosa cystica of primary hyperparathyroidism. Investigations X-ray is diagnostic showing ground glass/smoke screen appearance. Serum alkaline phosphatase may be slightly elevated.

,

Biopsy may be needed to confirm the condition and to rule out other conditions. Parathormone assay, serum calcium estimation in suspected parathyroid pathology. Treatment: It shou ld not be operated during growing period as if intervened there may be chances that it may turn into osteosarcoma. As it is a self-limiting disease it can be left alone once the growth stops or can be corrected by restorative excision to maintain facial contour. Thorough curettage with grafting of cancellous bone may be done. Bisphosphonates are often used to relieve pain.

CHERUBISM (Cherub-Angelic Being) It is an autosomal dominant condition that occurs in first year of life. Giant cell granuloma with fibrous tissues in the jaw. It is commonly bilateral. Commonly seen in the angles of the mandible and also in maxilla. It is familial fibrous dysplasia of jaw commonly involving both halves of the mandible with bulging outwards near the angle of the jaw causing 'winged face' appearance of angelic babies. Diffuse enlargement of maxilla and both sides of the mandible. Bulging of the cheek causes pull of the lower eyelid. Hence, child appears like, as if looking upwards. Interference with the development and eruption of the teeth. Treatment: It is a self-limiting disease. Often requires dental care and treatment for proper dentition .

Fibrous dysplasia mandible

Fibrous dysplasia maxilla Figs. 2.43A and B: Fibrous dysplasia of mandible and maxilla in two different patients. Mandible is common site in jaw. Overall fem ur is the commonest site.

• • • • • • •

Congenital condition Cleft palate alone Mandibular hypoplasia Cyanotic episodes Deficiency in transforming growth factor Defective sucking and tongue falling backwards in infants Cryptorchidism

Attitudes are more important than tacts.

355 n

:c l>

""O

-I

m

"" "T1 1:11

n

o· 3

~-

1:11

...iii '< 0 iii"

II)

1:11

"' II)

"'

Chapter

Oral Cavity ()HAFTER OUTLINE • • • • • • • • • • • • • • • •

Ranula Sublingual Dermoids Stomatitis Cancrum Oris Syphilitic Lesions of Oral Cavity Leukoplakia Erythroplakia Oral Submucosal Fibrosis Premalignant Conditions of Oral Cavity Oral and Upper Aerodigestive Cancers Cheek Carcinoma Cheek/Buccal Mucosa Lip Neoplasm of Lip Carcinoma Lip Tongue

• • • • • • •

• • • • • • •

Tongue Ulcers Benign Tumours of Tongue Tongue Fissure Glossitis Tongue Tie Carcinoma Tongue Carcinoma of Posterior One-Third/Base of the Tongue Nasopharyngeal Carcinoma Maxillary Tumours Malignant Tumours of Tonsil Carcinoma Hard Palate Laryngeal Tumours Malignant Tumours of Larynx Trismus

causes rupture of the acini due to increased pressure leading into extravasation cyst. Cyst contains saliva. The paired sublingual salivary glands are located beneath the mucosa of the anterior part of the floor of the mouth, anterior to the submandibular ducts and above the mylohyoid and geniohyoid muscles. It is closely related to lingual nerve and submandibular salivary gland duct.

Oral cavity includes lips, buccal mucosa, alveolar margins (gingiva), retromolar trigone, hard palate, floor of the mouth, anterior 213rd of the tongue (oral/mobile tongue).

RANULA (Rana= Frog, Ranula looks like belly of frog, hence the nameLatin). Ranula is an extravasation cyst arising from sublingual gland. It is actually a pseudocyst. Occasionally, it can arise from submandibular salivary gland also. Initially, there is blockage of the duct (of sublingual gland) causing retention cyst, which

Figs. 3.1A to C: Ranula. Note the bluish discolouration, brilliantly transilluminant and the location.

I Features

357

Presents as a bluish smooth, soft, fluctuant, brilliantly trar:silluminant swelling in the lateral aspect of the floor of the mouth. Plunging ranula: Ranula often extends into the submandibular region through the deeper part of the posterior margin of mylohyoid muscle and is called as plunging ranula. It is intraoral ranula with cervical extension. It is cross fluctuant across mylohyoid. It can arise from both submandibular and sublingual salivary glands as a mucus retention cyst initially, which reaches neck by passing across the mylohyoid muscle presenting as soft, fluctuant, non-tender, dumbbell-shaped swelling in the submandibular region. It is bidigitally palpable. Differential diagnosis: Lymph cyst; sublingual dermoid; retention cysts arising from glands of Nuhn and Blandin or from ducts of Rivinus. Investigations: US neck or MRI neck and oral cavity is diagnostic.

I Treatment

0 ii.1 r'I QI

Figs. 3.2A and B: Sublingual dermoid. It lies usually in midline. It is not transilluminant. Note the extension into the neck in submental region. Excised specimen is also shown.

I Types 1. Median sublingual dermoid:

Marsupialisation (unroofing) can be done in itially, and later once the wall of the ranu la is thickened it is excised completely (Marsupial means pouch where baby is kept, carried and sucked on the mother's belly, like in Kangaroo). If ranula is small it can be excised without marsupialisation. Excision of sublingual salivary gland is often needed. In plunging ranula, submandibular salivary gland needs to be excised occasionally. Note: • Ranula has a delicate fibrous capsule and is lined by macrophages. It contains clear fluid/saliva. It is thin walled. • It may get infected; it may get infected; occasionally, it may extend posteriorly along parapharyngeal space and may cause dysphagia. • It may interfere with speech andswallowing. It may damage Wharton's duct. • Only Marsupialisation even though often is sufficient but has got higher recurrence rate than sublingual gland excision. • Usually transoral route is sufficient; one should avoid injuring the lingual nerve and submandibular salivary duct.

SUBLINGUAL DERMOIDS They are sequestration dermoids lined by squamous epitheliu m containing keratin. It is smooth, soft, fluctuant, non-transilluminant bidigitally palpable swelling. They are congenital in origin; commonly occurs as midline swelling; lateral dermoid can occur but rare. Swelling may often attain large size presenting both sublingually, intraorally and midline submentally on external side. -:- Occasionally it can cause trismus, dysphagia, pain, odynophagia.

Differential diagnoses are---haemangioma, lymphangioma, sublingual dermoid, lipoma and Ludwig's angina. MRI is useful investigation. Excision is done through intraoral approach usually; large cyst extending under geniohyoid muscle may require external approach.

;.. It is derived from epithelial cell rests at the level of fusion of two mandibular arches. It may be supramylohyoid or inframylohyoid. It is located between two genial muscles, in relation to mylohyoid muscle. It is a mid line swelling which is smooth, soft, cystic, nontender, nontransilluminant. ;., Treatment is excision through oral approach. ;.. Complication is abscess formation. 2. Lateral sublingual dermoid: ;.. It develops in relation to submandibular duct, lingual nerve and stylohyoid ligament. It is derived from first branchial arch. It forms a swelling in the lateral aspect of the floor of t he mouth. ;., Treatment: Small one is removed per orally. Larger one, through submandibular incision.

STOMATITIS It is inflammation of oral mucosa by trauma, radiotherapy, chemicals, nutritional deficiency or infection. Traumatic stomatitis may be due to dentures, teeth bite, and brushing of teeth harshly which presents as painfu l thin covering of furr with increased salivation. Proper mouth wash will cure the condition. Aphthous stomatitis is seen in malnutrition, debility, steroid usage. Present as multiple hyperaemic painful vesicles later forming deep round painful ulcers. It is treated with mouth wash and if needed by antibiotics. Recurrent aphthous stomatitis with ulcers is often familial, more common in women, common in lip, cheek, tongue which are very painful with more salivation. It heals spontaneously. But during active period , it interferes with speech, swallowing distressfully. It is treated by many drugs like levamisole, antibiotics, vitamin Band C, local applications of anaesthetics (xylocaine)/choline salicylate/benzalko nium ch lo ride.

If you think you are right all the time, you learn nothing from life. -Sir Henry T Buutlin

< ;:;:

'
3 cm carry poor survival >3 in number of nodes involved is poor sign

Poor Prognostic Factors Size of the tumour >4 cm caries poor prognosis. Site of tumour (posterior third has got poor prognosis). Tumour crossing the midline. Lymph nodes status. Poor differentiation. Bone involvement.

CARCINOMA OF POSTERIOR ONETHIRD/BASE OF THE TONGUE Lesion may remain asymptomatic for long time. Clinically may be missed easily. Earlier symptoms are features mimicking sore-throat and throat discomfort. Dysphagia and change in voice (hot potato voice) occurs later. Referred pain in the ear, bleeding from mouth, visible mass in posterior third of tongue is late local features. lnduration on palpation in posterior third tongue is diagnostic of the carcinoma. As posterior third tongue has got abundant lymphatics which cross communicates on either side, lymph node spread is common (70%). Bilateral nodal spread is common. Massive nodes and involvement of jugulodigastric node are also common. Infiltration into the tongue muscles like genioglossus, epiglottis, pre-epiglottic space , tonsillar pillars and hypopharynx are common. Carcinoma posterior third of the tongue is often poorly differentiated and so caries poor prognosis. Blood spread can occur into bones, liver and lungs in posterior third cancers. Palpation under anaesthesia gives better idea about the tumour, its spread and also allows the biopsy. Presentation as unknown/occult primary and often with blood spread can occur. CT scan/MRI is always needed to plan the staging and therapy. T1, T2, N0 and N1 diseases are treated by surgical wide excision or often by total glossectomy using midline mandibulotomy incision (mandible split) with neck dissection on both sides (MRND one side). Post-surgery radiotherapy is needed if it is a poorly differentiated type or nodal status is more than N1. Advanced lesions need palliative radiotherapy or chemotherapy. T4 lesions are often treated by total glossectomy with laryngectomy and neck dissection but overall outcome is not good.

In many centres primary curative radiotherapy is used. Lymphoepithelioma and transitional cell carcinoma can occur in posterior third tongue (rarely).

B

385

("\

::c

It is squamous cell carcinoma arising from gums It is common in males It is common in India It is commonly due to tobacco/pan chewing Features and precipitating factors are similar to other oral carcinomas There will be invariable bone involvement by direct extension Nodal spread is also common • Wide excision with mandibulectomy and block dissection of neck is the treatment

It is usually aggressive tumour It is rare in India It is 2nd common site of oral carcinoma (SCC) in western countries It invades hyoglossus, mylohyoid, genioglossus and anterior mandible early Bilateral neck nodes are commonly involved Rim resection of mandible with wide excision of tumour with muscles and soft tissues and bilateral neck dissection is necessary Often visor anterior approach with anterior mandible resection followed by proper reconstruction with bone graft and plates is needed Post-operative radiotherapy and later chemotherapy is used to prevent recurrence Prognosis is poor and also has poor cosmetic results

Fig. 3.74: Carcinoma of floor of the mouth.

It has got poor prognosis.

NASOPHARYNGEAL CARCINOMA Nasopharynx lies above the level of the soft palate which divides it from oropharynx below. It is also called as post-nasal space or epipharynx. Eustachian tube opens on its anterolateral wall. Fossa of Rosen muller is located above and behind the opening of the Eustachian tube as a small depression. Nasopharyngeal carcinoma is common in China and Mongolia. In Ind ia it is common in North-East region. It is

Hope sees the invisible, feels the intangible and achieves the impossible.

l>

m :::0 w 0

@ f"I

DJ

< ;:;:

'
... :, VI ....0 QI C'l

n:s

:,

C

n:s

co a:: VI

VI

commonly squamous cell carcinoma (85%). Lymphoma, minor salivary tumours and sarcoma are other malignancies that can occur rarely in nasopharynx. It can be of proliferative, ulcerative, and infiltrative types. Commonest site is fossa of Rosenmuller in lateral wall of pharynx. It is three times common in males. HO's triangle in supraclavicular fossa (bounded by medial and lateral ends of clavicle and point where neck meets the shoulder) is the site where metastatic nodes commonly exist in nasopharyngeal carcinoma. In 50% of cases nodal involvement is bilateral. Often cervical lymphadenopathy may be the first presentation. Clinical features may be nasal, otogenic, ophthalmoneurogenic (involving most of the cranial nerves with facial pain, squint, di plopia, exophthalmos , and ophthalmoplegia) , jugular foramen syndrome (cranial nerves IX, X, XI spread), nodal spread and distant spread to bones, lungs and liver. Unilateral serous otitis media may be the only presentation.

It is common in people working in furniture industries, mustard gas industries, and leather industries. It is common in Bantus in South Africa where snuff with nickel and chromium is commonly used.

I Types Squamous cell carcinoma 80%. Adenocarcinoma. Transitional cell carcinoma. Salivary gland tumours. Sarcomas and melanoma. Burkitt's lymphoma.

I Features Epistaxis, nasal speech, post-nasal discharge and nasal obstruction. Pain in the ear with unilateral deafness due to compression of Eustachian tube with fluid collection in the middle ear. Elevation and immobility of soft palate on the same side. Pain in the area of distribution of trigeminal nerve due to direct infiltration of the nerve at foramen lacerum. Palpable secondaries in upper deep cervical lymph nodes (70%). TROTTER'S TRIAD

Unilateral deafness Immobile elevated soft palate • Pain in the distribution of trigeminal nerve

Differential Diagnosis: Lymphoma; Lymphoepithelioma; Minor salivary gland tumour. Investigations: Biopsy from the primary site; FNAC from the neck lymph nodes; X-ray of the skull to visualise erosions; CT scan skull. Treatment: External irradiation for primary. RT is the main modality of treatment. Radical block dissection of cervical lymph nodes. In N2a, N2b and N2c contralateral neck dissection is needed. Spinal accessory nerve is never preserved while doing block dissection in nasopharyngeal carcinoma. ,. Chemotherapy: Methotrexate, Vincristine. ;.. Skull base surgeries are useful.

MAXILLARY TUMOURS They are rare. Maxillary sinus is the commonest site for malignancy of paranasal sinuses. Ethmoids, frontal and sphenoids are next in order.

Fig. 3.75: Carcinoma maxillary sinus in a boy. Note the extension into the palate.

I Behaviour and Presentation

Initially may be symptomless or may present with epistaxis or features of chronic sinusitis. When it spreads to the floor, loosening of the teeth, necrosis, antro-oral fistula can occur. Extension medially causes nasal block, fungation, nasal discharge, blockage of nasolacrimal duct (epiphora). Extension anteriorly causes pain, anaesthesia and swelling in the cheek, ulceration and fungation in the skin of cheek. Spread above into the orbit causes epiphora, diplopia, proptosis. Posterior spread is most dangerous as it is not revealed easily. It causes postnasal discharge, pain, trismus, limitation of temporomandibular joint movement. Involvement of upper deep cervical lymph nodes in later stage is common. Differential diagnosis: Chronic sinusitis.

I Classification 1. Ohngren's classification An imaginary plane is drawn extending between medial canthus of eye and the angle of mandible. Growth situated above this plane is called as suprastructuralwhich has got poor prognosis. Growth below this plane is called as infrastructural and has got better prognosis.

2. Lederman's classification Two horizontal lines are used, one passes through the floor of the orbit, another passes through the floor of the antra. These lines are called as line of Sebileau. Suprastructure type: In this type olfactory area of nose, ethmoidal, sphenoid, and frontal sinuses are involved. Mesostructural type-. This involves maxillary sinus and nasal respiratory part. Infrastructural type-. This type involves alveolar process. Lederman's classification is further divided by two vertical lines over medial walls of the orbit to separate ethmoid sinuses and nasal tossa from maxillary sinuses.

T4 - T4a: Moderately advanced local disease-Tumour invading anterior orbital contents, skin of cheek, pterygoid plates, infratemporal Iossa, cribriform plate, sphenoid and frontal sinuses. T4b: Very advanced local disease-Tumour invading orbital apex, dura, brain, middle cranial Iossa, cranial nerves other than maxillary division of trigeminal nerve, nasopharynx or clivus N - Nodes: same as oral cavity. Staging Stage D: Tis NO MO. Stage I: T1 NO MO. Stage II: T2 NO MO. Stage Ill: T3 NO MO; T1 , T2, T3 N1 MO. Stage IV: IVA: T4a NO/N1 MO; T1, T2, T3, T4a N2 MO. Stage IVB: Any N3 MO; T4b Any N MO. Stage IVG: Any T Any N M1. ~ --------'

I Diagnosis

Medial canthus ---,,-.,;.;...of eye Infrastructure

387

Contd...

f.t>-;=-o,;;;;=7

- ~ff- Suprastructure

Maxillary sinus

X-ray of the part- pacity of the involved sinus with destruction of bony walls is seen-Water's PNS view. CT scan-ideal method. Sinus endoscopy for detailed examination of sinus and tor biopsy. Biopsy is done through nasal/oral or on early stage through Caldwell-Luc operation.

I Treatment Fig . 3.76: An imaginary plane is drawn extending between medial canthus of eye and the angle of mandible and line in this plane is called as Ohngren's line. Vertical lines Suprastructure Orbit

Pre-operative megavoltage radiotherapy is given. After six weeks, total maxil/ectomyis done. Reconstruction of maxilla along with dental reconstruction is required. When lymph nodes are involved radical neck lymph nodes dissection is done. Post-operative radiotherapy and chemotherapy is given as an adjuvant therapy. Overall prognosis is 30-40%. Lip

Caldwell-Luc incision ,,----..--

Canine fossa

Fig. 3.77: Diagrammatic representation of Lederman's classification.

Tx - Primary tumour cannot be assessed Tis - Carcinoma in situ T1 - Tumour limited to maxillary sinus mucosa with no erosion or destruction of bone T2 - Tumour causing bone erosion or destruction including extension into the hard palate and or middle nasal meatus except extension to posterior wall of the maxillary sinus and pterygoid plates T3 - Tumour invades either the bone posterior wall of the maxillary sinus, subcutaneous tissue, floor or medial wall of the orbit, pterygoid and ethmoid sinuses Contd...

Lip

Fig. 3.78: Incision for Caldwell-Luc operation which is used for taking biopsy from maxillary tumour. Incision is not used for definitive therapy for carcinoma maxilla. Incision also used in benign conditions to approach maxillary sinus. Gingivobuccal mucosa is incised and mucoperiosteum is raised. Bone of canine Iossa is cut to reach the maxillary antrum.

Efforts and energies should be consistent, constructive and compassionate.

...0 0.1

n 0.1


"'0

-f m :,:,

Anatomical Types

Fig. 3.83: Carcinoma hard palate extending laterally.

389

Oesophagus Fig. 3.84: Anatomy of larynx showing supraglottic, glottic and subglottic regions.

Your feelings are your God; our doubts are traitors and make us lose the good we oft might win.

w

...

0

QI

("I QI




"'ti

-t

m

::J:J .i::,.

400

SJOGREN'S SYNDROME It is an autoimmune disease causing progressive destruction of salivary and lacrimal glands, leading to keratoconjunctivitis sicca (dry eyes), and xerostomia (dry mouth).

en a::i

a:

Cl)

Types: 1. Primary. 2. Secondary. Primary Sjogren 's syndrome (Primary Glandular sicca syndrome) presents with severe dry mouth and dry eyes; widespread dysfunction of exocrine glands. It has got high incidence of developing lymphomas. But there is no association of connective tissue disorders. Secondary Sj6gren's syndrome presents with dry mouth and dry eyes; with association of connective tissue disorders like-Primary biliary cirrhosis (near 100%), SLE (30%), Rheumatoid arthritis (RA) (15%). Female to male ratio is 10: 1.

I Features It is common in middle aged females who present with dry eyes, dry mouth, enlarged parotids and enlarged lacrimal glands which are often tender. Superadded infections of the mouth, Candida albicans is common. Investigations: Autoantibody estimation-rheumatoid factor, antinuclear factor, salivary duct antibody, sialography; estimation of salivary flow; slit lamp test of eyes; Schirmer test-to detect lack of lacrimal secretion; FNAC of parotids and lacrimal glands; 99Technetium pertechnetate scan for gland function. Treatment: It is conservative. Artificial tears; artificial saliva; frequent drinking of water; treat the cause. Note: • Mlkulicz disease: It is a clinical variant of Sjogren's syndrome. It is an autoimmune disorder of salivary and lacrimal glands, resulting in infiltration of the glands with round cells. It may be due to or associated with sarcoidosis, Sjtigren's syndrome, leukaemia and lymphoma. • Triad of Mikulicz disease: (1) Symmetrical and progressive enlargement of all salivary glands. (2) Narrowing of palpebral fissures due to enlargement of the lacrimal glands. (3) Parchment-like dryness of the mouth. • Primary extra-glandular sicca syndrome: Dry mouth, dry eyes, hyperglobulinaemic purpura, vasculitis, Raynaud's phenomenon or B cell lymphoma. • In benign /ymphoepilhelial lesion, 20% bilateral diffuse parotid swelling develops; 20% develop lymphoma.

SIALOSIS It is enlargement of the salivary gland due to fatty infiltration, as a result of various metabolic causes like diabetes, acromegaly, obesity, liver disease, alcoholism, bulimia, idiopathic, drug induced (sympathomimetics, carbimazole, thiouracil). It is diffuse, bilateral, non-inflammatory, non-neoplastic asymptomatic swelling of the salivary gland mostly of parotids with fatty infiltration and acinar cell hypertrophy. Clinical features: Bilateral diffuse enlargement of parotids, which is smooth, firm, non-tender. Treatment: The cause is treated.

SIALECTASIS It is an aseptic dilatation of salivary ductules causing grapelike (cluster-like) dilatations. It is a disease of unknown aetiology with destruction of parenchyma of gland accompanied by stenosis and cyst formation in the ductu les. It is common in parotids; often bilateral. Presents as a smooth, soft, fluctuant, nontransilluminating swelling which increases in size during mastication. It is tender initially. It lasts for many days with a long symptomfree period of the disease. Sialogram is diagnostic (grape or cluster-like dilatations). Treatment is conservative (nonsurgical).

SALIVARY NEOPLASMS

I Aetiology

Genetic-loss of alleles of chromosomes in 12q, Bq, 17q. Eskimos are more prone for salivary neoplasm. Infective-mumps, Epstein-Barr vi rus, chronic sialadenitis may be the cause; but not proved emphatically. Recurrent inflammation can cause duct dysplasia and carcinoma. Radiatiorr-it is more common in survivors of atomic bomb explosion; mucoepidermoid carcinoma is more in these patients. Smoking-adenolymphoma of Warthin's shows 40% risk in smokers. Sex-benign tumours and many malignancies are common in females; Warthin's and some malignancies are common in males. Environment and diet-Arctic-Eskimos show dietary deficiency of vitamin A and develop salivary tumour. Industrial agents like nickel, cadmium, hair dyes, silica, preservatives may increase the risk of salivary tumours.

I Classification (WHO) a. Epithelial (90%): 1. Adenomas - Pleomorphic adenoma. - Monomorphic adenomas. • Adenolymphoma (Warthin's tumour). • Oncocytoma (oxyphil adenoma) seen in elderly; seen in parotid gland. • Basal cell adenoma-it is a rare benign tumour. 2. Carcinomas - Mucoepidermoid carcinoma-most common malignancy. - Acinic cell carcinoma-1%. - Adenoid cystic carcinoma- very aggressive-10%; common minor salivary glands. - Adenocarcinoma. - Squamous cell carcinoma- 2%. - Carcinoma in ex pleomorphic adenoma. - Undifferentiated carcinoma.

be diffuse/focal/capsulated/unencapsulated . It may be associated with Mikulicz's disease or Sjogren's syndrome. ;..- Malignant-it is rare tumour occurs in parotid and submandibular glands (ESKIMOMA).

Note:

Carcinoma can be: • Low grade (acinic cell; adenoid cystic; low grade mucoepidermoid) or • High grade (adenocarcinoma; squamous cell carcinoma; high grade mucoepidermoid) mesenchymal.

I Incidence Eighty per cent salivary neoplasms are in the parotids of which 80% are benign; 80% of th ese are pleomorphic adenomas; 80% occur in superficial lobe. Fifteen per cent of salivary tumours are in the submandibular salivary gland, of which 50% are benign. 95% of these are pleomorphic adenomas. Ten per cent of salivary neoplasms are in the minor salivary glands-palate, lips, cheeks, etc. Of these only 10% are benign. Note:

Fig. 4.17: Parotid enlargement in a boy of 11-year-old.

b. Nonepithelial: Haemangioma-common/y seen in infants, usually in parotids. Spontaneous regression is common. Most common benign salivary gland tumour in paediatric age group. Lymphangioma. Neurofibromas and neurilemmomas. c. Malignant lymphomas-NHL type: Common in parotids. Common with HIV, Sji:igren's syndrome (44 times more chances than normal people). d. Secondary tumours from head and neck region; bronchus and skin.

• • • •

Parotid tumours are common but only 20% are malignant. Submandibular tumours are uncommon but 50%of themare malignant. Minor salivary gland tumours are rare and 90% of them are malignant. Sublingual salivary tumours are very rare but almost all sublingual salivary tumours are malignant. • Incidence of malignancy in salivary glands is inversely related to size of the gland; in parotid it is 15%; in submandibular it is 50%; in sublingual it is 85%.

PLEOMORPHIC ADENOMA (Mixed Salivary Tumour) Commonest of the salivary gland tumour in adult. It is 80% common. More common in parotids (80%). 10% in submandibular salivary gland; 0.5% in sublingual salivary gland. It is mesenchymal, myoepithelial and duct reserve cell origin. Grossly it contains cartilages, cystic spaces, solid tissues. Microscopically it is biphasic in nature with epithelial and stromal components. Benign tumours will usually not show necrosis.

m

HISTOLOGICALL~- ,T SHOWS~ - - - -

Epithelial cells • Myoepithelial cells

Figs. 4.18A and B: Neoplasm of parotid gland (Malignant).

e. Lymphoepithelial tumours: ,

Benign-it is 5% of all benign sa livary tumours (Godwin's tumour) . It is common in females; can be bi lateral. Benign lymphoepithelial lesion (BLEL) is of unknown etiology characterized by replace ment of salivary parenchyma with lymphoid tissue. It may

---

-

Mucoid material with myxomatous changes • Cartilages/pseudocartilages

Even though it is capsulated, tumour may come out as pseudopods and may extend beyond the main limit of the tumour tissue. When disease occurs in parotid, commonly it involves superficial lobe or superficial and deep lobe together. But sometimes only deep lobe is involved and then it presents as swelling in the lateral wall of the pharynx, soft palate and posterior pillar of the fauces. There may not be any visible swelling in the preauricular region . It is called as 'dumbbell tumour . This tumour is in relation to styloid process, mandible, stylohyoid, styloglossus, stylopharyn geus muscles.

Selecting wisely generally follows experience of selecting unwisely; from this experience comes sound judgement usually based on bad judgement.

401

402

I Features B

CLINICAL FEATURES OF PAROTIO TUMOUR

Swelling, pain, ulceration, dysphagia (if deep lobe is involved) Raised ear lobule Cannot be moved above the zygomatic bone-curtain sign Deviation of uvula and pharyngeal wall towards midline in case of deep lobe tumour Facial nerve, masseter, skin, lymph node and bone involvement eventually occurs in case of malignancy

Common in females (3:1 ). Occurs in any age group. But common in 4th and 5th decade Usually unilateral. Present as a single painless, smooth, firm lobulated, mobile swelling in front of the parotid with positive curtain sign (As the deep fascia is attached above to the zygomatic bone, it acts as a curtain, not allowing the parotid swelling to move above that level. Any swelling superficial to the deep fascia will move above the zygomatic bone). Obliteration of retromandibular groove is common. The ear lobule is raised. When deep lobe is involved, swelling is commonly located in the lateral wall of pharynx, posterior pillar and over the soft palate-10%. Deep lobe tumour passes through Patey's stylomandibular tunnel pushing tonsils, pharynx, soft palate often without any visible swelling or only small swelling when only deep lobe tumour is present; it also presents as dysphagia. Bidigital palpation of parotid is significant in such occasion with one finger inside mouth. Facial nerve is not involved. Long-standing p/eomorphic adenoma may turn into carcinoma (carcinoma in ex pleomorphic adenoma). Its features are: Recent increase in size Pain and nodularity Involvement of skin, ulceration Involvement of masseter Involvement of facial nerve-lower facial nerve palsy-(Difficulty in closing eyelid, difficulty in blowing and clenching teeth) Involvement of neck lymph node Restriction of jaw movements

Fig. 4.19: Pleomorphic adenoma showing curtain sign.

Fig . 4.20: Deep lobe tumour of parotid is examined per orally.

Fig. 4.21 : Typical parotid swelling with ear lobe raised. Facial nerve

should be tested by clenching the teeth.

Fig. 4.22: Parotid tumour showing typical raise in earlobe

pleomorphic adenoma.

B Benign tumours are usually painless Sudden onset of pain denotes malignant transformation Pain is dull boring at primary site or referred to ear through auriculotemporal nerve •

Pain is due to:

-

Capsular distension by tumour Obstruction to free flow of saliva Nerve infiltration Inflammation like in Warthin's Tumour necrosis

• Incomplete excision, 10% of tumours which are highly cellular are other causes for recurrence. • RT is given after surgery eventhough it is benign (controversial; a debate). • Inexplicable metastasis can occur even though it is benign. • Tumour may implant due to spillage whilesurgical removal into retained residual parotid (deep lobe in superficial parotidectomy). • Recurrence after parotidectomyin pleomorphicadenoma is 5%. It is due to spillage, improper technique, inadequate margin, retained pseudopods, multicentricity. Recurrent tumour is multinodular without any capsule. Expression of MUC1/DF3 in the tumour is marker to predict recurrence.

I Investigations FNAC is very important and diagnostic. CT scan to know the status of deep lobe, local extension and spread. MRI is better method.

Fig. 4.23: CT picture of pleomorphic adenoma. Note:

• Incision biopsy of parotid tumour is contraindicated as chances of seedling and recurrence are high and also there is a chance of injuring the facial nerve, and chance of developing parotid fistula while doing the biopsy. • Malignant transformation in pleomorphic adenoma in early tumours is 3-5%; in long standing cases (more than 15 years) and recurrent cases it is more than 15%.

ffl OPEN BIOPSY IS CONTRAINOICATEO IN PAROTIO 11:.i TUMOURS DUE TO: • Chance of injury to facial nerve Seedling and high chance of recurrence • Chance of parotid fistula formation

I Treatment Surgery- first line treatment. If only superficial lobe is involved, then superficial parotidectomy is done wherein parotid superficial to facial nerve is removed. If both lobes are involved, then total conservative parotidectomy is done by retaining facial nerve. Note:

• Enucleation is avoided as it causes high recurrence (50%) due to extension of tumour outside as pseudopods across the capsule.

Figs. 4.24A and B: Recurrent parotid tumour which has attained large size. Note facial nerve is intact. Duct orifice should be inspected using retractor.

ADENOLYMPHOMA (Warthin's Tumour, Papillary Cystadenoma Lymphomatosum) It is a misnomer. It is not malignant, it is not lymphoma. It is a benign tumour that occurs only in parotid, usually in the lower pole. It is said to be due to trapping of jugular lymph sacs in parotid during developmental period. It composed of double layer of columnar epithelium, with papillary projections into cystic spaces with lymphoid tissues in the stroma. It usually involves only superficial lobe of parotid gland. It may also be multicentric. Smoking (40%/8 times more risk than nonsmokers) and radiation exposure may be the cause.

Remember that the faith to move a mountain always carries a pick. -Anonymous

403

404

I Features It presents as a slow growing, smooth, soft, cystic, fluctuant swelling, in the lower pole, often bilateral and is nontender. It is common in males-4:1. Common in smokers. Common in old people-60 years. Its incidence is 10%. Common in Whites. It is 2nd most common benign tumour. It is often bilateral-10%. Investigations: Adenolymphoma produces a "hot spof' in 9 9Technetium pertechnetate scan-it is diagnostic (Due to high mitochondrial content); FNAC. Treatment: Superficial parotidectomy. Note: Adenolymphoma does not turn into malignancy. But occasionally it can

simultaneously be associated with pleomorphic adenoma, carcinoma or lymphoma of parotid.

Site of adenolymphoma

Fig. 4.25: Warthin's tumour. It is seen in parotid gland usually in superficial lobe, in lower pole.

Predilection for 99Tc with hotspots and FNAC are the investigations.

BASAL CELL ADENOMA It is rare, benign , now classified under monomorphic adenoma containing isomorphic basaloid cells with basal layer and basement membrane. It is common in minor salivary glands; in major salivary glands it is multicentric. Grossly it looks like lymph node. Microscopy-isomorphic basaloid cells with solid/trabecular/ tubular/membranous pattern. Canalicular adenoma is its variant with bilayered ribbons of columnar cells separated by vascular stroma.

MUCOEPIDERMOID TUMOUR It is the commonest malignant tumour in parotid. It is 2nd common malignant tumour in submandibular and sublingual salivary glands. It is commonest malignant tumour of parotid in childhood. Incidence is 9% of salivary tumours; 20% of malignant salivary tumours. It occurs both in major as well as minor salivary glands. Parotid is the commonest site; palate is the commonest minor salivary gland site (In the palate adenoid cystic carcinoma is common). Radiation is the commonest etiological factor. Gross-unencapsulated solid tumour with cystic spaces. Microscopy-biphasic with mucin secreting (+ve for PAS, -ve for diastase) low grade and epidermoidwith high grade; clear eel/ with intermediate type. It is slowly progressive, often attains a large size and spreads to neck lymph nodes. It contains malignant epidermoid and mucous secreting cells.

Fig. 4.26: Warthins tumour of parotids. It is common in males; often bilateral; common in elderly.

ONCOCYTOMA (Oxyphil Adenoma) It is 7 mm at C2 and >22 mm at C6; CECT is very useful which shows retropharyngeal mass, hypodense lesion with ring enhancement, obliteration of fat planes. CT is useful for follow up also. Chest x-ray to confirm aspiration pneumonia if develops. Total count and C reactive protein will be raised. Treatment: Antibiotics intravenously is essential (penicillins, clindamycin, piperacillin and tazobactam). Managing airway obstruction by emergency cricothyrotomy or tracheostomy may be needed if endotracheal intubation fails. Drainage is done usually through per oral incision under careful general anaesthesia. Only occasionally drainage may be done through a neck incision. Pus should be sent for culture.

I Chronic Retropharyngeal Abscess It is invariably due to tuberculosis of cervical spine (common in C6).

Fig . 5.22: X-ray neck showing retropharyngeal abscess with tuberculosis lesion involving the C2 cervical spine (Courtesy. Dr Navin Chandra Shetty, MD, Radiologist, Mangaluru).

Abscess is in the m;dline behind the prevertebral fascia. There is destruction of the body of the vertebra due to tuberculosis. It presents as midline swelling in the posterior pharyngeal wall, which is smooth and nontender. Features of tuberculosis of cervical spine will be observed. Often abscess may point in the neck in relation to sternomastoid. Neurological manifestations may occur in severe disease. Investigations: X-ray spine, chest X-ray, ESR, MRI of cervical spine are essential investigations. Treatment: Antitubercular drugs; Drainage of the abscess should be done through neck approach (never intraoral approach). Decompression of the vertebra and stabilization is also often required.

SUBHYOID BURSITIS (Retrohyoid Bursa/Boyer's Bursa) Subhyoid bursa is space between posterior surface of the body of hyoid bone and thyrohyoid membrane. It lessens friction between these two structures during swallowing. Due to constant friction inflammatory fluid collects in the bursa leading to bursitis, which presents like a horizontally placed midline swelling between lower part of the hyoid bone and thyrohyoid membrane.\ Smooth, soft, cystic, fluctuant, nontransilluminating swelling which moves upwards with deglutition but not while protrud;ng the tongue out. It should be differentiated from thyroglossal cyst and prelaryngeal lymph nodes. It contains turbid fluid often may get infected to make the swelling tender or to form an abscess. Treatment Excision under general anaesthesia.

Happiness is a direction not a destination.

428

...>-

...OI:, QJ

VI

0

Histologicallycells are arranged in a classical pseudoalveolar r>,attern known as cell ball - Zellballen. It contains type I chief cells with catecholamine granules and type II sustentacular cells without granules.

- - - - -Subhyoid bursa

CAROTID BODY

t + - - - Thyrohyoid membrane

re

:,

Thyroid cartilage

C

re

co"' a:

Cricoid-------Sagittal view

Front view

VI

Fig. 5.23: Sagittal and front view showing location and relation of subhyoid bursa.

CAROTID BODY TUMOUR (Potato Tumour, Chemodectoma, Nonchromaffin Paraganglioma) It arises from thecarotid body, which is located at the bifurcation of the common carotid artery. Carotid body is derived from neural crest which is essential for adaptation in fluctuation in the 02, CO2 and pH. Carotid body tumour can be sporadic (75%); familial (20%, common in young, can be multiple); or hyperplastic (5%) which is associated with the chronic hypoxia seen in high altitude (5,000 feet from sea level), COPD, cyanotic heart disease. The tumour is situated in the adventitia of the artery. They are benign or locally malignant tumours (10%), but in 20% cases spread can occur to the regional lymph nodes and lungs. Blood supply to the tumour is from ascending pharyngeal artery and external carotid artery. Tumour does not secrete epinephrine or any endocrine substances. Blood supply comes through Meyer's ligament on the posteromedial wall of the carotid at bifurcation.

ICA - ' , - - - ----1

Carotid body tumour- -- - Splaying of carotid - - - - - . ,- -

Fig. 5.24: Carotid body tumour-note the site and location. Splaying of the carotid is common.

Pathologically, it is well-encapsulated, hard creamy yellowish tumour with dense fibrous tissue. Carotid body tumour cells are not hormonally active.

Normal carotid body is 3-5 mm sized, 15 mg weight, flat brownish nodule in the adventitious of common carotid artery It consists of chief cells (contains catecholamine granules) and supportive cells Its nerve supply is from Hering nerve, a branch of Glossopharyngeal nerve These chemoreceptors are sensitive to changes in pH and temperature in the body especially in hypoxia, help in autoregulation of respiration and circulation Carotid body hyperplasia can occur in people residing in high altitudes who are exposed to chronic hypoxia Other chemoreceptors in the body are-aortic bodies in the arch of aorta; glomus jugular in the bulb of the internal jugular vein; glomus intravagale in relation to ganglion nodosum of the vagus nerve and others like pulmonary (near pulmonary artery) and myocardial (near coronary artery origin) receptors

I Features 0.5%-lncidence. Usually unilateral; 5% bilateral. More common in middle age. Common in females. Swelling (75%) in t he carotid region of the neck which is smooth, firm, pulsatile (transmitted pulsation-due to pulsatile carotid vessel overlying its surface) and moves only side to side but not in vertical direction (Fontaine sign). It can often compress over oesophagus and larynx. Headache, neck pain (35%), dysphagia, and syncope are other presentations. 10% may present with cranial nerve palsy (hypoglossal, glossopharyngeal, recurrent laryngeal or spinal accessory) or sympathetic chain; so present as pain, tongue deviation towards same side while protruding, dysphagia, unilateral vocal cord palsy, hoarseness of voice, drooping shoulder and Homer's syndrome. Features of transient ischaemic attacks due to compression over the carotids, "carotid body syncope."

Thrill may be felt and bruit may be heard. It is located at the level of hyoid bone deep to anterior edge of the sternomastoid muscle in anterior triangle, vertically placed, round, firm 'potato' like swelling. Often tumour may extend into the cranial cavity along the internal carotid artery as dumbbell tumour.

B Type I: Localised, easily resectable (26%) Type II: Adherent, partially surrounding the carotids (46%) Type Ill: Adherent, encased carotids completely (27%)

I Investigations Arterial Doppler. Angiogram to see the 'tumour blush'- DSA. Widening/ splaying of the carotid artery with tumour blush in an angiogram is called as Lyre sign. CT scan, MRI, MR angiography. MIBG scan in useful in multiple familial and functioning tumours (they are smaller in size); in nonfunctioning tumours pentetreotide scan using radiolabeled somatostatin analogue is used. No FNAC, No trucut biopsy, No partial excision.

I Differential Diagnosis Carotid artery aneurysm. Soft tissue tumour (Sarcoma). Lymph node enlargement. Neurofibroma of the vagus nerve presents as swelling in the carotid triangle in the region of thyroid as vertically placed, oval, hard swelling. On palpation of the swelling, patient often develops bradycardia and dry cough. It does not move with deglutition and has only transvers~ mobility. As the tumour lies behind the carotid it can stretch the carotid in front causing transmitted pulsation (Figs. 5.25A to C).

I Treatment If it is small, then it can be excised easily as the tumour is situated in the adventitia. When it is large, as commonly observed, complete excision has to be done followed by placing a vascular graft.

During resection a temporary shunt (diversion of blood) is placed between common carotid below and internal carotid above to safeguard cerebral perfusion; external carotid artery is ligated. Venous or prosthetic graft is placed between common carotid and internal carotid arteries. Carotid body tumour is not radiosensitive (controversial).

I Complications of the Surgery Bleeding. Blockage of common carotid artery, leading to contralateral side hemiplegia (3%). This can be prevented by stenting the common carotid artery towards internal carotid artery and is done during surgical excision of the tumour. Cranial nerve injury X and XI (40%). Injury to vagus causes hoarseness of voice; injury to superior laryngeal nerve alters the pitch of voice.

Cold abscess Cystic hygroma Branchial cyst Thyroglossal cyst

TORTICOLLIS (WRY NECK) It is turning of the neck to one side with chin pointing towards opposite side.

I Causes Sternomastoid tumour. Trauma-spinal injury, disc prolapse, spondylosis. Inflammatory: Lymphadenitis either tuberculous or suppurative; tuberculosis of cervical spine. Spasmodic-due to spasm of sternomastoid muscle of same side or spasm of posterior cervical muscles of opposite side.

Figs. 5.25A to C: Vagal Neurofibroma: (A) Large neurofibroma of vagus-clinical look; (8) CT image showing tumour; (C) On table look of

tumour with anteriouly displaced carotid artery.

Direction is a matter of fact; ideas are matter of opinion.

429

n

:I: )>

--4

m

:::0 Vt

z

(I)

n

;,:;-

430

Reflex. Rheumatic-after exposure to cold/draught. Burns-causing contracture. Ocular causes. Compensatory due to scoliosis.

STERNOMASTOID TUMOUR It is due to birth injury to the sternomastoid muscle. It is a misnomer. It is not a tumour.

Figs. 5.26A and B: Boy having right-sided torticollis.

Fig. 5.28: Sternomastoid tumour-typical location.

Fig. 5.27: Torticollis (right-sided) with chin towards opposite side.

Features Restricted neck movements. Chin pointing towards opposite side. Squint. Features relevant of the causes. Treatment:The cause is treated. Benik Contralateral Torticollis Bracing System; TOT collar used only child is awake; Kinesio taping technique-are used. Nole

One-third of congenital torticollis is due to sternomastoid tumour and twothirds due to abnormal position in utero which recovers spontaneously in a few weeks. - Kenneth F Hulbert.

During child birth, injury to the sternomastoid muscle causes haematoma in the muscle which gets organ ised to form sternomastoid tumour. Common in breech delivery. It is seen in infants of 3-4 weeks age. Swelling of about 2 cm size, in the sternomastoid muscle which is smooth, hard, nontender and adherent to the muscle-in the middle part. Chin pointing towards opposite side. Head towards same side (Scoliosis capitis). In later age groups it causes hemifacial atrophy due to less blood supply as a result of compression of the external carotid artery by sternomastoid tumour and dueto kinking by position of neck. Distance between the outer canthus of eye to angle of mouth is reduced, with less arched eyebrow, flat or less filled cheek and flat nose compared to opposite side. Compensatory cervical scoliosis. Compensatory squint. Differential diagnosis-. Other causes for torticollis.

I Treatment Division of the lower end of the sternomastoid muscle or excision of the muscle. Both sternal and clavicular heads of sternocleidomastoid muscle should be divided under general anaesthesia using horizontal incision. One should not injure IJV, carotid, vagus, spinal accessory nerve. Additional all fibrous bands are also cut. Usually over correction is done. Physiotherapy exercise and toricollis harness is used for 6-12 months. Exercise and active stimulation of muscles in early cases.

T

Differential diagnosis for neck lymph node enlargement

• Tuberculous lymphadenitis • HIV infection

• Secondaries in lymph nodes • Lymphomas

• Chronic lymphatic leukaemia

• Nonspecific lymphadenitis

• Infectious mononucleosis

• Sarcoidosis

• Actinomycosis

• Brucellosis

Disease can also occur in other lymph nodes like-axillary, para-aortic, mesenteric, inguinal, mediastinal, intercostal. Disease may be associated with HIV infection, lymphomas.

431

':I:"' "m )>

--4

::0 V1

• Toxoplasmosis

z

rt)

n

';I:"

Fig. 5.29: Lymphoma neck involving both sides. Lymphomas are smooth, nontender, firm/India rubber consistency.

Fig. 5.30: Swelling in the neck well-localised. Note the scar of previous biopsy/excision. It could be lymph node enlargement due to lymphoma or tuberculosis.

TUBERCULOUS LYMPHADENITIS (Earlier called as Scrofula). It is the commonest form of extrapulmonary tuberculosis. Causative organ ism: Mycobacterium tuberculosis.

I Sites Common in neck lymph nodes-80% Common in upper deep cervical (jugulodigastric-54%). lymph nodes 20% bilateral. Next common is posterior triangle lymph nodes (22%).

[!I

Figs. 5.31A to C: Cold abscess due to caseating tuberculous lymphadenitis in neck. Collar stud abscess in the neck. Tuberculous sinus formation after drainage.

It takes 5 years to learn when to operate, and 20 years to learn when not to.

432

I Features Mode of Infection: Usually through the tonsils, occasionally through blood from lungs. Tonsillar infection shows multiple tubercles on its surface; from here infection spreads into jugulodigastric nodes (anterior triangle nodes) then to other nodes. Infection reach lymph node first into subcapsular space/ sinus then to lymph node cortex which contains plenty of lymph follicles. Matting is due to periadenitis involving subcapsular sinus/ space of lymph node. In children infection to neck node can come from either tonsils or adenoids or both. When it occurs from adenoids, lymph nodes in posterior triangle are involved through retropharyngeal lymphatics. It may be associated with pulmonary tuberculosis or renal tuberculosis. Through blood infection reaches medullary cords of lymph node and so medulla of lymph node. Rarely spread can occur from tuberculous lesion of the apex of lung through suprapleural Sibson's fascia/membrane to supraclavicular nodes. Often fibrosis and calcification can occur with or without treatment. Gross Pathology: Firm, matted, lymph node, with cut section showing yellowish caseating material. Microscopic Features: Epithelioid cells with caseating material are seen along with Langhans type of giant cells.

------

Stage 1: Lymphadenitis (Discrete nodes, nontender, firm/hard mobile)

...-----.... -------

Stage 2: Matting (Firm, nontender, move together en mass - due to periadenitis)

00 00

Stage 3: Cold abscess (Deep-to-deep fascia)

Stage 4: Collar-stud abscess (Rupture through deep fascia under the skin, cross fluctuant, adherent to skin)

Stage 5: Sinus formation

Fig. 5.33: Stages of tuberculous lymphadenitis (Hogarth's Rake) .

Acute type: Seen in infants and early childhood below 5 years Hyperplastic type: Lymphoid hyperplasia is typical; it is seen in patients with good resistance; hard discrete mobile lymph nodes; 15-20% common Caseating type: 75-80% common; matted nodes often with cold abscess; poor body resistance; seen in young adults Atrophic type: Rare type; seen in elderly; small lymph nodes but caseating type with atrophied nodes

Fig. 5.32: Tuberculous lymphadenitis-cut section of the specimen. Note the yellowish caseating material with periadenitis (matting). Caseating type is more common than hyperplastic type.

B 1. 2. 3. 4. 5.

Stage of infection, and Jymphadenitis Stage of periadenitis with matting Stage of caseating necrosis and cold abscess formation Stage of formation of collar stud abscess Stage of formation of sinus which discharges yellowish caseating material

_J

Swelling in the neck which is firm, matted. Cold abscess is soft, smooth, nontender, fluctuant, without involvement of the skin. It is not warm. As a result of increased pressure, cold abscess ruptures out of the deep fascia to form collar stud abscess which is adherent to the overlying skin. Once collar stud abscess bursts open, discharging sinus is formed. It can be multiple, wide open mouth, often undermined, nonmobile with bluish color around the edge. It is usually not indurated. Tonsils may be studded with tubercles and so clinically should always be examined. Associated pulmonary tuberculosis should also be looked for. In 20% cases of tuberculous lymphadenitis, there may be associated pulmonary tuberculosis or it may be a primary focus. Cervical spine is examined for tuberculosis.

T

433

Types

1. Hyperp/a,tlc

2. C11eatlng

a.

20% common

a.

80% common

b.

Discrete, firm or hard

b.

Matted due to periadenitis

::c

C.

Occurs in the cortex of lymph node

c.

Involves medulla with periadenitis

-0 --4

d.

Host immunity is good

d.

Body resistance is not adequate

:::tJ

e.

Drugs act better

e.

Drugs do not reach in proper concentration and may not be effective

f.

Drug resistance is uncommon

Drug resistance is common

g.

No cold abscess or sinus formation

h.

Blood spread

I. g. h.

Axillary nodes, when involved, is due to retrograde lymphatic spread from neck nodes or blood spread. Inguinal lymph nodes are involved occasionally through blood. Bluish hyperpigmented involved overlying skin is called as scrofuloderma. Tuberculous pus with caseating cheesy creamy material is infective as it contains multiplying organisms. Atypical mycobacterial tuberculosis can occur occasionally. Such disease may be resistant to drug therapy. Sinus may persist due to-fibrosis, calcification, secondary infection, inadequate reach of drug to maintain optimum concentration in caseation. Differential diagnosis: :.-- Nonspecific lymphadenitis. , Lymphomas, and chronic lymphatic leukaemia. , Secondaries in the neck. , Branchial cyst mimics cold abscess. , Lymph cyst mimics cold abscess.

Deep-to-deep fascia No evidence of signs of inflammation Not warm, nontender, smooth, soft and fluctuant, non-transilluminating Not adherent to skin (skin is free); no redness Contains cheesy caseating material It is seen in caseating tuberculous lymphadenitis due to caseation necrosis It may form collar stud abscess and later sinus FNAC, AFB, culture are useful investigations Differential diagnosis are branchial cyst, lymph cyst Treated by Antituberculous drugs 'Zig-zag' aspiration by wide bore needle in nondependent area to prevent sinus formation Drainage using nondependent incision; later closure of the wound without placing a drain

HIV with lymph node involvement. When there is discharging sinus-actinomycosis.

Cold abscess or sinus are common Spread from tonsils

I Investigations Haematocrit, ESR, peripheral smear. Ultrasound neck: It shows node size, matting, cold abscess, track, number of nodes; Doppler USG helps in demonstrating the vascularity (hilar vascularity in tuberculous node; peripheral capsular vascularity in metastatic node). FNAC of lymph node and smear for AFB and culture. FNAC is very useful but not as superior as open node biopsy. False negative, false positive results and altering the node architecture, and so eventual need of open biopsy are the problems. Epithe/ioid cells (modified histiocytes/macrophages) are diagnostic. Langhans giant cells, lymphocytes, plasma cells are other features. Open biopsy when FNAC is inconclusive. Open biopsy is more re/iab/efor tuberculosis (and also in lymphoma; but it is contraindicated in node secondaries); entire node (ideally two nodes if possible) has to be taken intact; one in formalin for pathology, other in normal saline for microbiology (AFB) and culture. HIV test (ELISA and Western blot), CD4 count Lowenstein-Jensen media is used for culture which takes 6 weeks to give result; so selenite media is often used which shows growth in 5 days. Mantoux test may be useful; but not very reliable. Chest X-ray to look for pulmonary tuberculosis. Polymerase chain reaction (PCR) is very useful method. CT neck and chest to study nodal status.

I Treatment Drugs Antitu bercular drugs has to be started: Rifampicin 450 mg OD on empty stomach. It is bactericidal. It discolours urine red. It is also hepatotoxfc. INH: 300 mg OD. It is bactericidal. It causes intolerance of GIT. Neuritis, Hepatitis (I NH). Ethambutol 800 mg OD. It is bacteriostatic. It causes GIT intolerance, retrobulbar neuritis (green colour blindness). Pyrazinamide 1500 mg OD (or 750 mg BO). It is bactericidal. It is hepatotoxic, also causes hyperuricaemia and increases psychosis. Duration of treatment is usually 6-9 months.

Happiness is when what you think, what you say, and what you do are in harmony. --Mahatma Gandhi

n

>

m V,

434

Aspiration When there is cold abscess, initially it is aspirated. [Wide bore needle is introduced into the cold abscess in a nondependent site along a "Z" track (in zig-zag pathway) so as to prevent sinus formation].

Fig. 5.35: Tuberculous sinus excision with excision of

diseased lymph nodes.

Figs. 5.34A to C: Typical cold abscess in the neck in which pus is aspirated. Pus should be sent for cytology (for epithelioid cells), staining (Ziehl-Neelsen-AFB) and culture.

Incision and drainage If it recurs, then it should be drained. Drainage is done through a nondependent incision. After draining the caseating material, wound is closed without placing a drain. Surgical removal Surgical removal of tubercular lymph nodes are indicated, when 1. There is no local response to drugs or 2. When sinus persists. It is done by raising skin flaps and removing all caseating material and lymph nodes. Care is taken not to injure major structures. Excision of the sinus track It is often essential when sinus develops. Note: " Paradoxical upgrade reactions" (PUR) is worsening of the symptoms

during treatment period with development new nodes, nodes become larger, more sinus formation in patients who have received at least 1O days of therapy. It is due to rapid killing of mycobacteria which releases mycobacterial products like cytokines, TNF-alpha, interleukins etc causing extensive local inflammation and necrosis. It shows negative stains for AFB. Baseline peripheral monocytosis is a significant predictor. Non-tuberculous Mycobacterial Lymphadenitis (NTML): It is seen in developed countries like Australia, Canada, and USA; commonly due Mycobacterium avium, M. scrofulaceum, M. kansasii. Level 1 neck nodes are involved;

truly localized; unilateral; biopsy, NTM antigen specific¥ interferon and skin tests. Surgical excision is the main treatment. BCG Jymphadenitis: It occurs as a complication of the BCG vaccination. It can be simple nonsuppurative which regresses spontaneously or suppurative which forms often an abscess which is nontender, not warm without fever. Needle aspiration or often incision and drainage help. There is no role of antitubercular drugs. Mycobacterium bovis also can cause tuberculous lymphadenitis but is rare now. Infection occurs through dairy products and droplets. It is more aggressive with high mortality in HIV patients. Pyrazinamide is not useful for M. bovis lymphadenitis. BCG vaccine is derived from M. bovis (1919).

COLD ABSCESS Cold abscess is common in neck. It can also occur in groin, intercostal space, loin or any site where tuberculous caseating material with cheesy content can get collected and localised. Cold abscess may originate from tuberculosis of spine (thoracic or cervical spines), lymph node, internal organs, bone, etc.

I In the neck Tuberculous lymphadenitis is common cause. Here cold abscess is commonly seen in anteriortriangle. Tuberculosis of cervical spine is also an important cause. Commonly here cold abscess occurs in posterior triangle. Caseating material from the cervical spine collects in front of the vertebra behind the prevertebral fascia which eventually ruptures either anteriorly or posteriorly. , Anterior rupture allows passage of caseating material below and behind the prevertebral fascia reaching superior mediastinum; laterally behind the prevertebral fascia and carotid sheath to form cold abscess in posterior triangle; in midline upper part, protruding forwards from behind the prevertebral fascia in midline presenting as chronic retropharyngeal abscess; in midline lower part

,

protrudes into oesophagus; caseation runs along the axillary sheath and neurovascular plane to reach axilla and arm to cause cold abscess in axilla and arm/cubital fossa. Posterior rupture occurs towards spinal canal facilitating the passage of caseation along the cervical nerves towards posterior triangle and brachia! plexus and so axilla and arm.

435

B Secondary infection of the cold abscess making it tender. Formation of collar stud abscess, once pressure increases inside the cold abscess which will give way through the deep fascia to reach the subcutaneous plane to get adherent to skin. Sinus formation. Spread of disease to multiple lymph nodes and other organs. Differential Diagnosis Branchial cyst and other cystic swellings in neck. Secondaries in neck lymph nodes. Secondaries in cervical spine.

I

I Treatment

Tuberculous sinus

l

Antituberculous drugs. Nondependent aspiration or drainage of the cold abscess. Excision of the diseased neck nodes. Immobilization of cervical spine by plaster jacket/collar for 4 months. Cervical spine fusion by open surgical method, if diseased spine is unstable.

SECONDARIES IN NECK LYMPH NODES

Cold abscess

Levels in Neck Nodes (Memorial SloanKettering Cancer centre levelling of neck nodes)

Fig. 5.36: Tuberculous cold abscess and sinus in the neck.

I Features It is common in young but can occur in any age group. Equal incidence in both sexes. Swelling in the neck, which is smooth, nontender, soft, fluctuant, nontransilluminating, with restricted mobility but is not adherent to skin. Neck pain, neck rigidity, restricted movements of cervical spine in case of cervical spine tuberculosis. With every change of position and often when patient is seated he supports his head with his hands and forearm-Rust's sign (Jan N Rust, surgeon, Poland). Evening fever, loss of weight and appetite, anaemia. Features of systemic disease, if present like of pulmonary tuberculosis-cough, haemoptysis. Matted lymph nodes adjacent to cold abscess may be palpable. Oral cavity, tonsils, chest should be examined. Raised ESR, positive Mantoux test, anaemia, lymphocytosis, chest x-ray may show pulmonary tuberculosis, aspiration of cold abscess (FNAC) to see microscopically epithelioid cells. Acid-fast bacilli may be identified from the aspirated fluid using Ziehl-Neelsen stain. X-ray neck in case of cervical spine tuberculosis to identify reduced joint space, vertebral destruction, soft tissue shadow. MRI of cervical spine, US/CT scan neck are needed to confirm the anatomical location, number of lesions.

Level I: Submental (la) and submandibular (lb) lymph nodes. Le vel II: Lymph nodes in upper deep cervical region . (It extends from base of skull to hyoid bone and from lateral margin of sternohyoid to posterior margin of sternomastoid muscle). Level Ila is below and in front of the line of the spinal accessory nerve in the upper part; 11 b is above and posterior. Level Ill: Lymph nodes in middle cervical region (from hyoid bone to omohyoid muscle or cricothyroid membrane). Level IV: Lymph nodes in lower cervical region (from omohyoid muscle/cricothyroid membrane to clavicle). Level V: Lymph nodes in posterior triangle including supraclavicular region from posterior border of sternocleidomastoid muscle to anterior border of trapezius muscle. Level Va is above the line of spinal accessory nerve in the lower part; Vb is below. Level VI: Lymph nodes in the midline neck-pretracheal and prelaryngeal from hyoid bone above to suprasternal notch below, medial border of carotid sheath on either side. Level VII: Lymph nodes in the mediastinum. inferior to suprasternal notch to innominate artery below. Note: •

Level I node from oral cavity, lip, salivary gland, skin; level II node from oral cavity, oropharynx, nasopharynx, salivary gland; level Ill node from oral cavity, oropharynx, hypopharynx, larynx, thyroid; level IV node from oropharynx, hypopharynx, larynx, t hyroid, cervical oesophagus; level V node from nasopharynx, scalp, GIT, breast, lungs.

Example isn't the best way to teach, it's the only way.

436

Note: • •

... ...en:::,

>-

QI

1/\

•

0

,0

:::,

•

C: ,0

"' al

20% of metastatic squamous cell carcinoma is from aerodigestive tract. Source of primary from head and neck region usually have frequency like this- nasopharynx, tonsil. base of tongue, thyroid, larynx, floor of the mouth, cheek, palate, pyriform fossa. Non-head and neck source of primary are bronchus, oesophagus, breast, stomach. Histologically it is (80%) or non-SCC. In non-SCC it may be poorly differentiated carcinoma (10- 15%), adenocarcinoma (5- 10%) or others like melanoma, poorly differentiated neoplasm (5%). In poorly differentiated neoplasm immunohistochemistry/ immunoperoxidase staining, electron microscopy and chromosome analysis

sec

are needed to rule out lymphoma, neuroendocrine tumours, undifferentiated sarcomas.

a:

1/\

• •

Fig. 5.37: Levels of neck nodes.

• • • •

3 years after treatment of one primary, if recurrence occur it is called as new primary in aerodigestive tract. Lower lip, tongue, soft palate and supraglottis can cause bilateral secondar ies in neck. Soft palate, retro molar region, nasopharynx, hypopharynx posterior and lateral oropharynx can involve retropharyngeal nodes. Nasopharyngeal carcinoma spreads to level 11-V, retro and pa rapharyngeal nodes. 1 5% of secondaries are from infraclavicular primaries- lung, pancreas, oesophagus, stomach, breast, ovaries, testis, prostate. Nodal spread below the lower border of cricoid (level IV and V; Lower) carries poor prognosis compared to nodes above cricoid (Upper).

Branchiogenic carcinoma It is a primary squamous cell carcinoma which is uncommon, arising from remnants of branchial cleft or arch. It is a differential diagnosis for secondaries in neck. It is common in men. It is common near the area of carotid bifurcation. Histologically it contains malignant squamous cells with lymphoid tissues around. It spreads to lymph nodes and can infiltrate into adjacent soft tissues. Treatment Wide excision.

I Features of Secondaries in Neck

Figs. 5.38A to D: Typical secondaries in the neck. Note the different levels involved in different patients. Note the skin involvement and sinus formation in few photos.

Common in adulVelderly male (Male to female ratio is 4:1), presents as painless rapidly increasing localised swelling in the neck. •·· Nodular surface and hard in consistency, often fixed when it is advanced. Fungation, ulceration, haemorrhage can occur in advanced disease.

COMMON SITES OF PRIMARY Oral cavity, tongue, tonsils

Oesophagus

Salivary glands

Lungs

Pharynx-oasopharynx Larynx

GIT Thyroid

It is commonly from squamous cell carcinoma, but can also be from adenocarcinoma or melanoma. Squamous cell carcinoma is mainly from oral cavity, pharynx. Adenocarcinoma is usually from GIT, commonly involving left supraclavicular lymph nodes. Breast, lungs, thyroid abdominal viscera are other areas where primary may cause secondaries in neck which should be examined when suspected.

Figs. 5.39A and B: (A) Typical secondaries in the neck-well localized, hard swelling which is fixed to the sternocleidomastoid muscle; (8) Secondaries in the neck nodes from laryngeal carcinoma.

Secondaries from papillary carcinoma of thyroid can be soft, cystic with brownish black fluid. Secondaries can infiltrate into carotids, sternomastoid , posterior vertebral muscles, spinal accessory nerve (shrugging of shoulder is affected), hypoglossal nerve (tongue will deviate towards the same side), cervical sympathetic chain (Homer's syndrome). Secondaries spread into adjacent soft tissues and also to the skin causing fungation and ulceration. Often because of tumour necrosis, softer area develops in the hard node. Skin fold prominence due to infiltration of the platysma is typical. In advanced cases tumour may infiltrate into the major vessels like carotids, or branches of external carotid artery causing torrential haemorrhage. Dysphagia, dyspnoea, haemoptysis, hoarseness of voice, ear pain, deafness are other features depending on the primary site.

Secondaries, when mobile are treated by radical lymph node block dissection in the neck.

Figs. 5.41A and B: Advanced secondary carcinoma neck with

ulceration and fungation.

Fig. 5.42: Secondaries in neck with nodules and ulceration. Note the deviation of tongue towards same side due to hypoglossal nerve

involvement.

2. Secondaries in the neck with clinically unidentified primary Hard neck lymph nodes are the secondaries, but primary has not been identified clinically. FNAC of the neck node is done and secondaries is confirmed. Hoarseness-carcin oma larynx, thyroid Then search for the primary is done by various investigations. Dysphagia-carcinoma posterior 113rd of the tongue, pharynx, oesophagus They are: Haemoptysis, cough, dyspnoea-carcinoma lung a. Panendoscopy Ear pain, deafness-nasopharyngeal carcinoma ,. Nasopharyngoscopy. Spinal accessory nerve-shrugging of shoulder is difficult ,. Direct laryngoscopy. Hypoglossal nerve-tongue deviates to same side with wasting , Oesophagoscopy. Sympathetic chain-Horner's syndrome withmiosis, anhidrosis, , Bronchoscopy- if needed only. upper eyelid droop (pseudoptosis), enophthalmos, loss of spinoJ iary reflex b. Blind biopsies are taken from fossa of Rosenmuller, lateral -- -wall of pharynx, pyriform fossa, tonsillar bed, base of tongue, region (larynx). It is called as surveillance biopsy subglottic Types of Secondaries in the Neck and is done to reveal unknown primary in 15% of cases of 1. Secondaries in the neck with known primary secondaries in neck. If this surveillance biopsy is negative, Here secondaries are present and primary has been identithen ipsilateral tonsillectomy may be needed. Presently fied clinically in the oral cavity, pharynx, larynx, thyroid or bilateral tonsillectomy is recommended. c. FNAC of thyroid and suspected areas. other areas. Biopsy from the primary and FNAC from the secondaries d. CT scan. Once the biopsy confirms the primary, it is treated either by are done. Primary is treated accordingly either by curative radiotherapy surgery or by curative radiotherapy. Secondaries in the neck is treated by radical neck dissection. or by surgery (wide excision). Fig. 5. 40: Fungating secondaries in the neck-terminal illness.

---

-

=i

I

Listen to the faintest sound; for opportunities knock only once.

437

438

...::, (II

C'I

V,

0

3. Secondaries in the neck with an occult (unknown) primary It is biopsy (FNAC) proven cancer of the neck node, which even after a complete clinical and radiological workup (that includes physical examination, CT scan, oesophgoscopy, laryngoscopy, bronchoscopy and multiple surveillance biopsies) reveals or yields no demonstrable primary lesion.

ro

::, C:

ro

:rE

B Fossa of Rosenmaller Lateral wall of pharynx Posterior third of the tongue Thyroid

V'l

co a: V,

L-

--

--

--

costly. Chromosomal analysis for tumour specific genes is used in B, T and germ cell lymphomas. Initially the secondaries in the neck are treated by radical neck dissection, then regular follow-up is done (at three monthly intervals) until the primary reveals. Once primary is revealed it is confirmed by biopsy and treated accordingly, either by curative radiotherapy or by wide excision depending on location of revealed primary. This type is usually less aggressive and has got better prognosis.

Paranasal sinuses Bronchus Oesophagus ---

N1- ...:::, °' ....0 QI

11'1

IO

:::, C:

IO

m

USG IN THYROID

To identify nodules, number, size, vascularity, echogenicity To do USG-guided FNAC To identify neck lymph nodes To find out solid or cystic nature Benign lesion is hyperechoic, often cystic with well-defined margin; shows peripheral egg shell calcification with sonolucent rim (halo) around nodule Malignant lesion is hypoechoic with poorly defined margin, with high vascularity, with microcalcification without any halo around j

:E

"' cc a:: 11'1

Figs. 6.11A and B: Note the goitres in (A) male and

CLASSIFICATION OF GOITRE Goitre is enlargement of thyroid gland. ( 'goiter'-Latin-guttur-throat) 1. Simple nontoxic a. Diffuse hyperplastic: Physiological · Puberty. - Pregnancy. Primary iodine deficiency (Endemic; dietary iodine intake less than 100 µg/day). Secondary iodine deficiency: Goitrogens of Brassica family, e.g. cabbage, soya bean. Common in hill stations. · Excess dietary fluoride. - Drugs: PAS, lithium, phenylbutazone, thiocyanates, potassium perchlorate, antithyroid drugs, radioactive iodine. Dyshormonogenetic goitre. b. Colloid goitre. c. Nodular goitre (Multinodular). d. Solitary nontoxic nodule. e. Recu rrent nontoxic nodule. f. Wolff-Chaikoff effect (1948): Intake of large quantity of iodides inhibits the further release of thyroid hormones (inhibits organification) by autoregulatory mechanism; but later may cause escape phenomenon. g. Hokkaido goiter: Hokkaido is northern island in Japan where iodine rich seaweeds are the main diet intake of which causes goitre in these individuals. Patients are euthyroid. 2. Toxic a. Diffuse (Primary)-Graves' disease. b. Multinodular (Secondary)-Plummer's disease. c. Toxic nodule (solitary) (Tertiary). d. Recurrent toxicosis. 3. Neoplastic a. Benign-adenomas: follicular, Hurthle cell. b. Malignant: Carcinomas: Papillary, follicular, medullary, anaplastic. Lymphomas. 4. Thyroiditis a. Hashimoto's autoimmune thyroiditis. b. de-Quervain's autoimmune thyroiditis. c. Riedel's thyroiditis. 5. Rare causes: Bacterial (suppurative), amyloid.

(B) female patient.

Figs. 6.12A and B: Familial goitre. Goitres in brother and sister.

Figs. 6.13A and B: Familial goitre running in sisters.

B Grade 0: No visible or no palpable goitre. Grade 1: Palpable thyroid/goitre but not visible in normal positioned

neck. Grade 2: goitre which is visible in normal positioned neck.

Figs. 6.14A and B: Large goitre in two female patients.

IN A CASE OF THYROIO OISEASE, FOLLOWING THINGS SHOULD BE MADE VERY CLEAR Functional status-hyperthyroid/euthyroid/ hypothyroid Compression on trachea/recurrent nerve Neck lymph nodal status Tracheal deviation Carotid infiltration Retrosternal extension Systemic features like toxicity or malignant spread to different organs like bone/liver/lungs

DIFFUSE HYPERPLASTIC GOITRE Initial persistent increase in TSH level causes diffuse active lobules. In late stages of diffuse hyperplasia,TSH stimulation decreases and many follicles become inactive, get filled with colloid and it is called as colloid goitre. As diffuse hyperplastic goitre is a reversible stage, L-thyroxine is beneficial.

J, Nodule formation. J, Centre of the nodule is inactive and only margin is active, i.e. internodular tissue is active. J, Formation of many nodules. J, Multinodular goitre (MNG). Other factors involved are growth-stimulating immunoglobulins and growth prone cell clones.

B Stage of hyperplasia and hypertrophy Stage of fluctuation in TSH Stage of formation of nodules (inactive); (inter-nodular tissues are active) Colloid goitre is a goitre due to long-standing iodine deficiency with I localised accumulation of significant colloid in the gland.

I

Fig. 6.15: Diffuse hyperplastic goiter.

MULTINODULAR GOITRE (MNG) MNG is discordant growth with functionally and structurally altered thyroid follicles presenting as multiple nodules in thyroid. It may be due to mainly fluctuation in TSH level; other causes may be iodine deficiency, goitrogens, hereditary, dyshormonogenesis.

Fig. 6.16: Examination of the thyroid gland from behind for any

nodules. Thyroid enlargement in male is rare. Nodule in a male could be malignant.

I Pathogenesis Persistent TSH stimulation. J, Diffuse hyperplasia of gland (all active lobules). J, Later with fluctuation of TSH level. .1. Mixed areas of active and inactive lobules develop. It is also probably due to increased sensitivity of follicular cells to TSH. J, Active lobules become more vascular and hyperplastic. J, Haemorrhages occur with necrosis in the centre.

Multiple nodules

Thyroid

Fig. 6.17: Multinodular goitre.

Your purpose is more powerful than anyone's opinion. -Billy Cox

451

I Clinical Features

452

cc"'

a:

V\

Fig . 6.18: Pizzillos method to make the thyroid more prominent while examining.

More common in middle aged females (10:1). It is a slowly progressive disease with many years of history. Multiple nodules of different sizes are formed in both lobes, also in isthmus, which is firm, nodular, nontender, moves with deglutition. Recent increase in size signifies malignant transformation or haemorrhage. Positive Kocher's test is due to compression over trachea (tracheomalacia/scabbard trachea) in a long-standing MNG. Nodule when calcified becomes harder; necrosis softens the nodule.

l

Secondary thyrotoxicosis (30%) Follicular carcinoma of thyroid (10%) Haemorrhage in a nodule Tracheal obstruction, calcification Cosmetic problem -- -- --

Note: Thyropharyngeus and cricopharyngeus parts of inferior constrictor muscle are attached to thyroid and cricoid cartilages respectively; during swallowing these muscles will contract to move thyroid and cricoid cartilages upwards. Thyroid is attached to larynx (cricoid) through condensed pretracheal fascia, Berry's ligament. So thyroid moves upwards with deglutition.

Fig. 6.19: Recurrent nodule thyroid. Note scar of previous surgery.

Fig. 6.21: Multinodular goitre. Note the nodules marked.

Fig. 6.20: Thyroid swelling moves on deglutition because of attachment of pretracheal fascia through Berry's ligament to cricoid cartilage and to constrictor muscles.

Fig. 6.22: Large multinodular goitre.

I Investigations T3, h TSH, free T4, US neck, FNAC. FNAC is done from most dominant and suspicious nodule. FNAC from more than one nodule is better; US-guided FNAC is more reliable. High resolution US identifies impalpable nodules, number, nature of nodule, vascularity of nodule. Nodule less than 0.3 cm is identifiable in US. X-ray neck shows ring or rim calcification; also reveals the position (displacement) and compression of trachea. Indirect laryngoscopy to see vocal cords prior to surgery (This is mainly for documentation and legal purpose as even in individual with normal voice, one of the vocal cords may have been paralyzed by viral infection like mumps, probably during childhood and have compensated)-occu!t recurrent laryngeal nerve (RLN) palsy). Radioisotope iodine scarr-in selected patients when indicated only. Routine blood investigations, serum calciu m. CT scan/MRI are routinely not indicated. It is done in retrosternal extension.

I Treatment Usually surgery is preferred. Reason for doing surgery in nodular goitre is-it is an irreversible stage and chances of complications like development of toxicity, haemorrhage and follicular carcinoma are high and also for cosmetic reason. When entire gland is diseased total thyroidectomy is a better option. Subtotal thyroidectomyis done depending on the amount of gland involved, amount of normal gland existing and location of nodules-commonly done procedure in multinodular goitre. Eight grams of thyroid tissue is retained in each lateral lobe. Often partial thyroidectomy or Hartley Dunhi/1 operation (isthmus + one entire lateral lobe and opposite side subtotal

or partial) is also done depending on the amount of diseased gland and normal tissues behind. Partial thyroidectomy is not well approved now. Postoperative L thyroxine is often given to prevent any fluctuation in TSH level which may cause recurrent nodule formation. Prevention of multinodular goitre is possible by supplementing with L-thyroxine (0.1-0.2 mg) when patient develops goitre in puberty. Formation of nodular goitre can be prevented by correcting iodine deficiency by using iodine-rich diet like eggs/seafood/milk or iodized salts and also avoiding goitrogenic drugs and diet. Suppressive dose of L-thyroxine alone may be used occasionally in small nodules with limited results. TSH level should be suppressed consistently below 0.5 mlU/L. Problems are need of periodic monitoring with TSH estimation; hormone insensitive part of thyroid tissue continue to grow; indefinite period of treatment; high recurrence after stopping L-thyroxine. So it is not ideally accepted therapy. It is found that TSH suppression is of no use in treating residual/recurrent MNG. Note: • Adequate thyroidectomy is essential, either total or subtotal, complete removal of diseased thyroid without retaining is considered, in view of chances of high recurrence in 10 or more years due to incomplete removal. Re-surgery for recurrent MNG is difficult due to loss of anatomical plane and fibrosis; hence total thyroidectomy is often advised. • Fine calcification is seen in papillary carcinoma (Psammoma bodies); coarse calcification is seen in multinodular goitre. • Radioactive iodine therapy may be beneficial after subtotal thyroidectomy to prevent or treat recurrent nodules.

DISCRETE THYROID NODULE Discrete thyroid nodule (4% of adult population) is a clearly palpable nodule in thyroid. It can be solitary (isolated) nodule (70%) if clinically only anodule is felt without palpable remaining gland OR it can be dominant nodule (30%) if clinically a nodule is felt in a palpable remaining one or both lateral thyroid lobes. A discrete thyroid nodule is common in females. Discrete nodule may be solid or cystic. USG, CT, MRI may confirm the diagnosis. 15% solitary/isolated nodule may be malignant; 40% may be follicular adenoma. Other causes are thyroid cyst, thyroiditis or colloid degeneration. Incidence of malignancy in dominant nodule is 50% less than that of solitary nodule. Risk factors for malignancy in discrete thyroid nodule are(1) solitary is 2 times more risk than dominant; (2) male is 4 times more than female (in solitary-48% to 12% in solid, 24% to 6% in cystic; in dominant 24% to 6% in solid, 12% to 6% in cystic); (3) solid nodule is 2 times more risk than cystic-Rule of 12.

Fig. 6.23: Multinodular goitre surgical specimen-total thyroidectomy was done.

Some people speak from experience; others, from experience, don't speak.

453

"":::c )>

m :c

°'

454

...>Cl/

E::,

Vl

0 re,

::,

SOLITARY THYROID NODULE It is a single palpable nodule in thyroid on clinical examination, in an otherwise normal gland. Rest of the gland is impalpable. Current definition - A discrete lesion/nodule within the thyroid gland that is palpably and or radiologically (Ultrasound neck) distinct from surrounding thyroid parenchyma (in an otherwise normal gland). The importance of thyroid nodule rests with the need to exclude thyroid malignancy which occurs in 5-15% cases.

C: re,

:E

Ill

cc a: Vl

m Figs. 6.24A and B: Solitary nodule thyroid in one lateral lobe and isthmus.

I Causes Thyroid adenomas Almost all thyroid adenomas are follicular. Follicular adenoma can be - Colloid (do not have potential for microinvasion; commonest type); Fetal (microfollicular - has potential for microinvasion); Embryonal (atypical - has potential for microinvasion); Hurthle ce/1/oxyphil or oncocytic (has potential for microinvasion); hyalinising trabecular adenoma. Carcinomas - papillary (commonest carcinoma), follicular, medullary (non-familial or familial or with MEN syndrome), anaplastic. Thyroid cysts. Thyroiditis presenting as solitary nodule (localized form). Note: • Papillary adenoma earlier called, was actually papillary carcinoma; papillary adenoma is very rare or probably non existing. • Earlier definition was only clinically palpable nodule which also included only palpable nodule in an underlying multinodular goitre (other nodules are not palpable clinically) accounted for 50%; now radiological finding is added to definition. Hence MNG cause is not considered.

, ,

Nodule is overactive. It is 5% common of which only 5% can be malignant. Warm : Normally functi oni ng nodu le. Nodu le and surrounding normal thyroid will take up the isotope (active). It is 10% common of which 10% can be malignant. Cold: Nonfunctioning nodule; may be malignant (need not be always). Nodule will not take up isotope (underactive). It is 80% common of which 20% are malignant.

I Features Single nodule palpable in one or other lobes of the thyroid which is usually smooth and firm. Lahey's test does not show any other nodules in posterior part of the gland. Hot or warm in 99mTc scan but cold in 1123 scan {discordant nodule} commonly they are malignant. Thyroid nodule in children and elderly can be malignant. Rapid enlargement of thyroid nodule can be malignant. Tracheal deviation towards opposite side is commonconfirmed by trail sign, th ree-finger test, auscultation and X-ray neck. 30% of solitary nodules are cystic. 20% of cold nodules are malignant. Cold nodule may be due to malignancy, thyroiditis, thyroid cyst or haemorrhage, benign adenoma. Commonest site of a nodule is at the junction of isthmus with one of the lateral lobes. Solitary thyroid nodule is the most common thyroid surgical disease.

Any nodule can be malignant whether it is hard/ firm/cystic/small/ large/asymptomatic Rapid onseVrapid recent increase in size Hoarseness of voice/dysphagia/stridor/dyspnoea Fixity of the nodule Palpable significant neck nodes Nodule in a male patient Nodule in a child (50% are malignant) Nodule in extremes of age group

SOLITARY NODULE IS ALSO CLASSIFIED AS Benign-70% Malignant-5% lndeterminate-10%-suspicious and follicular neoplasm Nondiagnostic-15%

I Types Based on function: (1) Toxic solitary nodule-3-5% of solitary nodules of thyroid; (2) Nontoxic solitary nodule. Based on radioisotope study (Technetium 99mPertechnetate scan): Hot: Means autonomous toxic nodule. Normal surrounding thyroid tissue is inactive and so will not take up isotope.

Fig. 6.25: Solitary nodule thyroid involving isthmus.

I Investigations Ultrasound neck (very useful). ,, It shows size, number, echogenicity, solid or cystic, vascularity, presence of significant lymph node (irregular, thick walled, without halo, more/chaotic vascularity, absence of hilus, microcalcification, cystic/necrotic changes). ,, TIRADS-ACR 2015 (Thyroid Imaging Reporting And Data System: American College of Radiology) based on-(1) Composition (2) Echogenicity (3) Shape (4) Size (5) Margin (6) Echogenic foci. Tl RADS 1-Benign; TIRADS 2-Not suspicious; TIRADS 3-Mildly suspicious; TIRADS 4-Moderately suspicious; TIRADS 5Highly suspicious. ,, If sonologically, Nodule >1 cm with high suspicious of malignancy or >1.5 cm with low suspicious of malignancy or >2.0 cm with very low suspicious of malignancy needs FNAC. Suspicion high (solid hypoechoic, irregular, microcalcification, taller than wide, extrathyroidal extension) has got >90% malignant risk; intermediate (hypoechoic solid but not other features) has 20% risk; low (iso- or hyperechoic solid or partially cystic but no other features) has 10% risk; very low (spongiform or partially cystic) has got 3% risk. ,, Irregular nodule, microcalcifications, taller than wide are suspicious features for malignancy. FNAC shows benign (70%); indeterminate (10%-it can be suspicious or follicular neoplasm); malignant (5%); nondi-

indicates malignancy in that nodule. Malignant nodule shows anarchical angiogenesis. Flow patterns are: Type 0- no flow; Type 1: only peripheral flow; Type 2: peripheral with small central flow; Type 3: peripheral with extensive central flow; Type 4: only central flow. Serum calcitonin estimation if FNAC confirms medullary carcinoma. Thyroidal uptake on F18-fluorodeoxyglucose position emission tomography (18FDGPET) scan is often useful in large nodule. CT scan or MRI neck is not done routinely, but only in selected cases. Large swelling/to see vascularity/retrosternal extension are the indications. X-ray neck to see tracheal deviation.

Thyroid nodule

Figs. 6.27A and B: Trachea should be clinically examined to find out whether it is central or deviated. In goitre involving both lobes, it is central. One lateral lobe enlargement usually causes deviation of trachea towards opposite side. Tracheal compression should be checked by Kocher's test. It confirms the scabbard trachea. Note:

True incidence of solitary nodule will come down to 50% from its original clinical diagnosis after investigations and surgical exploration.

I Treatment Malignant nodule Follicular neoplasm Toxic nodule in young Nodules with obstruction

Recurrent cystic nodule Complex cyst (both solid and cystic components) Cosmetic reason

Benign Nodule Fig. 6.26: Solitary nodule thyroid.

agnostic (15%; reaspiration should be done in 4 weeks). US-guided FNAC is the investigation of choice due to its safety and accuracy. T3, T4, TSH. Radioisotope study (l I23;1131; 99mTc). Power Doppler is done to know the vascularity of the gland. Vascularity is described in resistive index (RI) (Harley De Nicola 2005). Normal RI is 0.65-0.7; if RI is more than 0.7 it

Nontoxic benign nodule is treated with observation without any therapy. There is no role of any hormone therapy (L thyroxine). Annual clinical examination and ultrasou nd neck is needed during essential follow up- any nodule of 20% increase in size or more than 2 mm increase in diameter warrants a repeat FNAC and hemithyroidectomy (removal of entire one lateral lobe and entire isthmus) may be considered . Compressive symptoms and cosmesis are the indications for surgery-hemithyroidectomy.

A genius is someone who shoots at a target which no one else sees and hits it.

455 n

::c

)> "'O

-f

m

;:I:,

°'

451

Nodule (carcinoma)

Old scar Fig. 6.30: Patient earlier operated for nodular goitre. Note recurrent nodule and scar of previous surgery.

Figs. 6.28A and B: Ultrasound pictures of thyroid. It is often used to see nodules, content-solid/fluid, size and extent.

is needed. Later suppressive dose of L thyroxine (0.2 to 0.3 mg daily) is given orally in morning time on empty stomach. If FNAC report says follicular adenoma, then hemithyroidectomy is done. If histology report says follicular carcinoma (capsular and vascular invasion), then completion total thyroidectomy is done. Completion thyroidectomy is done usually within 7 days or after 3 weeks. If frozen section biopsy proves carcinoma, then total thyroidectomy is done. If FNAC report says medullary carcinoma of thyroid, then total thyroidectomy with bilateral neck nodal dissection including central compartment is done.

Indeterminate Nodule

Fig. 6.29: FNAC of thyroid (Courtesy: Dr Krishna Upadhya, Pathologist, Nandikoor Laboratory, Mangaluru).

If FNAC shows follicular neoplasm, then hemithyroidectomy, paraffin section confirmation for capsular and vascular invasion; completion thyroidectomy in 7-14 days is done; later radioactive iodine therapy is given. If FNAC shows suspicious variety, then repeat FNAC is done. If it is also suspicious then hemithyroidectomy is done; frozen section biopsy is done to confirm if it is papillary carcinoma; if so, total thyroidectomy with ipsilateral central node neck dissection is carried out. (Frozen section is not suitable for follicular neoplasm). -

Solitary toxic nodule needs initial antithyroid drugs and then radioactive iodine therapy (5 m curie); occasionally surgery is done-hemithyroidectomy. Colloid nodule can be observed or hemithyroidectomy is done for cosmesis or just in pain or increase in size; thyroxine therapy even though is commonly used, its benefit is not proved.

Risk of malignancy in follicular lesion of undetermined significance (FLUS) is 10%. Total thyroidectomy is indicated here. If there is a nodule in the isthmus, isthmectomy is done with excision of part of adjacent lateral lobes.

Malignant Nodule If FNAC comes as papillary carcinoma of thyroid, then total or near total thyroidectomy is done. If tumour is more than 2 cm, with extracapsular spread then radioactive iodine therapy

-

-

- -

-

--

-

-

THYROID CYST It is thyroid swelling which is cystic in nature eliciting positive fluctuation But tensely cystic swelling can be hard (thyroid paradox-with cellular tumour of thyroid can be soft also) Common cause is colloid degeneration-50%. There will be absence of epithelial lining Involution in follicular adenomas present like a cyst; 15% of such cysts may be malignant 30% of solitary nodules are cystic 15% cystic swellings in thyroid are malignant Cyst formation is common in papillary carcinoma of thyroid Acyst if contains both solid and cystic areas, is called as complex cyst which is more likely to be malignant FNAC may cause regression of simple cyst. Even after three repeated aspirations if recurrence occurs, surgery is needed Surgery is indicated in complex cyst and if cyst is more than 4 cm in size. Percutaneous ethanol injection (PEI) may be useful in benign cyst.

457

Nondiagnostic Nodule Cyst that recurs and repeat FNAC also becomes nondiagnostic then hemithyroidectomy and proceed is the better option especially if nodule is more than 4 cm in size and or in highrisk group. Repeat FNAC becomes diagnostic in 50% of initial nondiagnostic.

RETROSTERNAL GOITRE Retrosternal goitre is defined as having >50% goitre below the suprasternal notch, i.e. below the plane of thoracic inlet. Major intrathoracic extension requiring mediastinal dissection, extension into the anterior mediastinum more than 2 cm in depth or mass reaching the level of 4th thoracic vertebra are other criterias considered. Primary is rare-1%. Primary retrosternal goitre arises from ectopic thyroid tissue from mediastinum. It gets its blood supply from mediastinum itself, not from the neck. And also it is not related to the existing thyroid in the neck. Secondary is common. It is extension from the enlarged thyroid from the neck. Usually arises from the lower pole of a nodular goitre. Commonly seen in short neck or obese individuals. Due to negative intrathoracic pressure, nodule gets drawn into the superior mediastinum.

I Types 1. Substernal type: Part of the nodule is palpable in the lower neck. 2. Plunging goitre-. An intrathoracic goitre is occasionally forced into the neck by increased intrathoracic pressure. 3. lntrathoracic goitre itself. Neck is normal. Common in men.

I Features Dyspnoea at night during lying down or neck extended . Cough and stridor (strider is harsh sound on inspiration). Dysphagia.

Fig. 6.31 : Superior vena cava obstruction due to thyroid extending into the retrosternal area. Note the dilated veins over neck, chest wall and arm.

Engorgement of neck veins and superficial veins on the chest wall. Lower border is not seen on inspection and not felt on palpation. Pemberton 's sign is positive. The patient is asked to raise the arm above the shoulder level. Dilated veins are seen over neck and upper part of chest wall. Stridor and rarely dysphagia may occur. (When patient raises the arm above the shoulder level, retrosternal goitre compresses over the easily compressible structures like SVC and trachea causing dilated veins and dyspnoea respectively). Dull note over the sternum on percussion. Retrosternal goitre can be either nodular, toxic or malignant. Rarely recurrent nerve palsy can occur. Retrosternal goitre has got similar chance of turning into malignancy alike cervical goitre; but identifying by US or by FNAC is difficult. Differential diagnosis: Mediastinal tumours.

Figs. 6.32A to C: (A and B) X-rays (PA view and lateral view) showing retrosternal goitre. (C) Diagrammatic representation of the retrosternal goitre.

Success consists of a series of little daily efforts.

458

I Investigations Radioactive iodine study is diagnostic (1 123 or technetium). ,:. CT/MRI is very useful investigation. CT neck and chest is ideal. T3, T4, TSH estimation. Chest X-ray will show soft tissue shadow; barium swallow X-ray will show oesophageal indentation. Lung function tests are useful.

I Treatment Surgical removal of retrosternal thyroid is done. Commonly, it can be removed through an incision in neck (as blood supply of retrosternal goitre is from neck}, but in case of large retrosternal extension or in malignant type median sternotomy is required (rarely). Even asymptomatic retrosternal goitre needs surgery; total thyroidectomy with careful extirpation of the retrosternal extension through cervical incision is the choice. Note: • Radioiodine therapy is not accepted in retrosternal goitre.

• Stridor due to compression of tracheobronchial tree by retrosternal goitre is very dangerous because it is often not possible to clear the airway either by intubation or by tracheostomy. • Surgical removal should be complete because recurrent retrosternal goitre is very difficult to re-operate. • In the case of retrosternal goitre, hemoptysis unmixed with sputum is due to rupture of an engorged tracheal vein.-Peter Burgess BREATHING DIFFICULTIES IN THYROID SWELLING

Retrosternal goitre-positive Pemberton's sign Multinodular goitre of long duration-positive Kocher's testcompressive stridor Secondary toxic goitre-congestive cardiac failure Carcinoma infiltrating the trachea-stridor on rest-without compression with fingers

THYROTOXICOSIS AND HYPERTHYROIDISM I have lately seen three cases of violent and long-continued palpitations in females, in each of which the sample peculiarity presented itselfviz., enlargement of the thyroid gland .... A lady, aged twenty, became affected with some symptoms which were supposed to be hysterical .... II was now observed that the eyes assumed a singular appearance, for the eyeballs were apparently enlarged, so that when she slept, or tried to shut her eyes, the lids were incapable of closing. -Robert James Graves, 1835 (Irish physician)

L

Thyrotoxicosis is symptom complex due to raised levels of thyroid hormones. Thyrotoxicosis refers to biochemical and physiological manifestations of excessive thyroid hormones. Hyperthyroidism is the term used for overproduction of the hormones by thyroid gland. In hyperthyroidism pathology is in thyroid gland itself. Hyperthyroidism is one of the causes of thyrotoxicosis. Thyrotoxicosis can also occur due to other causes other than hyperthyroidism. Other causes of thyrotoxicosis without hyperthyroidism are-ectopic functioning thyroid, struma ovarii, functioning

metastatic follicular carcinoma, trophoblastic tumours, thyrotoxicosis factitia.

I Types 1. Diffuse toxic goitre-(Graves' disease, Basedow's disease. Primary thyrotoxicosis)-60%. 2. Toxic multinodular goitre (Secondary thyrotoxicosis) (Plummer disease)-20%. 3. Toxic nodule (Goetsch's disease)-5%. 4. Thyrotoxicosis due to rarer causes: a. Thyrotoxicosis factitia-drug induced. Due to intake of L-thyroxine more than normal. b. Jod-Basedow effect/phenomenon is hyperthyroidism occurs in a goitre patient (not normal thyroid gland) after administration of increased doses of iodides (in hyperplastic endemic goitre). It is also seen after administration of iodine contrast agents or amiodarone drug. Jod in Germany means iodine. c. Autoimmune thyroiditis or de Quervain's thyroiditis. d. Neonatal thyrotoxicosis. It subsides in 3-4 weeks as TsAb titres fall in the baby's serum. e. Struma ovarii. f. Drugs like amiodarone-an antiarrhythmic agent. Amiodarone is rich in iodine having structural similarity to T4 causing thyrotoxicosis. g. Very rarely, well-differentiated carcinoma can cause thyrotoxicosis-metastatic type. h. Patients with hydatidiform mole or choriocarcinoma with high levels of 13 HCG which can stimulate TSH receptor and can cause thyrotoxicosis.

Graves Disease Graves disease is an autoimmune disease with increased levels of specific antibodies in the blood (TSH receptor antibodies). Normal feedback mechanism is absent. It is often associated with vitiligo. It is often familial. Thyroid stimulating immunoglobulins (TSl)/thyroid stimulating antibodies (Ts Ab) and longacting thyroid stimulator (LATS) cause pathological changes in the thyroid. Histologically, there is acinar cell hypertrophy and hyperplasia with absence of normal colloid in the tall columnar epithelium (normal is flat epithelium with colloid). As cells are empty, they look vacuolated. Tissues are highly vascular. Exophthalmos producing substance (EPS) causes Graves ophthalmopathy. Diffuse goitre, thyrotoxicosis and autoimmune manifestations like infiltrative ophthalmopathy, dermopathy, myopathy are essential components of Graves disease. Thyroid stimulating immunoglobulins (TSls) are produced against thyroid antigen in Graves disease which is directed to TSH receptor acting as TSH receptor antibody. This TSHR Ab is observed only in Graves disease. Puberty, pregnancy, emotion and infection are the precipitating factors for primary thyrotoxicosis. Familial/genetic cause is also attributed in Graves disease (50%); both identical twins can develop Graves disease.

T3 Toxicosis Here Taalone is raised; TSH is decreased; T4 is normal. Free T3 estimation is important. Subclinica/ Hyperthyroidism Subclinical hyperthyroidism is defined as a state of decreased TSH level but not undetectable with Ta, T4, free Taand free T4 are within the normal range without any clinical symptoms. Its incidence is 1% of hyperthyroidism. It is one of the causes of infertility in females (both subclinical hyper or hypothyroidism can cause infertility). It may present as cardiomyopathy or arrhythmias. Hormone assay, radioisotope scan and US neck, ECG are used for evaluation.

Figs. 6.33A and B: Graves disease in a female and male patient. Note the exophthalmos with clearly visible lower sclera.

There is hyperplasia and hypertrophy of entire thyroid due to prolonged continuous action by binding of abnormal thyroid stimu lating antibodies to TSH receptor sites. Scalloped pattern of vacuolated colloid is typical.

Toxic Adenoma (Toxic Nodule) It is benign functioning monoclonal thyroid tumour, usually more than 3 cm in size. It usually presents as functioning (toxic) solitary nodule of thyroid. It is autonomous functioning tumour; not TSH responding. Toxic adenoma secretes large quantity of thyroid hormones suppressing the function of the remaining normal thyroid tissue. There are no eye signs and other features of Graves disease. It com monly shows higher T3 levels than T4. TSH receptor or G protein genes show somatic mutation. US neck, T3, T4 , TSH and radioi sotope scan (shows hot nodule)-are the relevant investigations. Treatment: ,, Initial control of toxicity with antithyroid drugs; later hemithyroidectomy is done after 6 weeks. Once thyroidectomy is done, suppressed remaining normal thyroid tissue starts functioning to secrete normal level of thyroid hormones. ,, Radioactive iodine therapy can be used which selectively concentrates and ablates the thyroid adenoma; later remaining normal thyroid starts functioning. Note:

• Radioactive iodine therapy after initial control of toxicity using antithyroid drugs, has become the standard mode of treatment after the age of 10 years. But RAI therapy should not be used in pregnancy, lactation as it crosses placental barrier and get secreted in milk causing newborn or infant hypothyroidism.

Struma Ovarii Ovarian teratoma with thyroid differentiation will secrete T3 and T4 and suppress TSH. Function of normal thyroid in neck is suppressed. Radioisotope scan shows uptake in pelvis with no or less uptake in neck. Note: The River Struma arises in Bulgaria and flows into Aegean Sea. Endemic goitre exists in area along its banks;·Struma' means goitre. Hashitoxicosis It is due to autoimmune Hashimoto's thyroiditis. Mild toxic features develop during initial stage of hyperplasia. Already formed thyroid hormones are released by inflamed gland causing toxicity. It eventually leads into euthyroid and later hypothyroidism in Hashimoto's disease. Thyrotoxicosis Factitia Intake of L thyroxine without indications to lose weight or overdose intake causes toxicity. Postpartum Hyperthyroidism It is exacerbation of previously confirmed or undiagnosed hyperthyroidism during pregnancy or after delivery due to increased autoimmune factors. It is associated with HLA DR3 and HLA DR5. Neonatal Thyrotoxicosis It is seen in infants born to mother with Graves disease due to crossing of the thyroid stimulating antibody (TSH RAb) across placental barrier. Infant will be toxic for 3-4 weeks which subsides gradually. Trophoblastic Thyrotoxicosis HCGsecreted from vesicular mole, choriocarcinoma or metastatic embryonal carcinoma in females, acts like TSAb causing toxicity. Amiodarone-induced thyrotoxicosis Amiodarone is antiarrhythmic drug. It can cause thyrotoxicosis. Type I is treated with thionamide, potassium perchlorate, beta blockers often with glucocorticoids. Type II is destructive and so eventually patient recovers; glucocorticoids and beta blockers are needed initially. Apathetic Hyperthyroidism It lacks all usual clinical features of toxicity. It is commonly observed in old people. Thyroid gland is not enlarged.

Men take only their needs into consideration, never their abilities.

Contd...

459

460

QI

...C'I

::,

.....0

\I\

ro

::, C

ro

cc a: VI

\I\

Contd... 1 Patient presents with behavioral problems; often considered as psychiatry patient with decreased appetite. Such lethargic individual also often shows features of recent angina and atrial fibrillation. Unless serum T3, T4 and TSH are done, diagnosis is masked. Subacute Thyroiditis (Toxic Phase) Other common cause of thyrotoxicosis is subacute thyroiditis (15-20% of cases), a destructive release of preformed thyroid hormone. Radioactive iodine scan does not show any radioactive iodine uptake in the thyrotoxic phase of the disease. Thyroid hormone levels can be highly elevated. Low ESR, low T3 T4 ratio are other features.

I Clinical Features of Thyrotoxicosis •·· It is eight times more common in females. It occurs in any age group. Primary type is seen commonly in younger age group. Secondary type is common in older age group. Graves's disease often presents without any obvious thyroid swelling in the neck. Whenever, there is unexplained behav-

Diffuse - ~ ~ toxic goitre

Thyroid

Fig. 6.34: Diffuse toxic goitre (primary) involves both lobes with hyperfunctioning acini.

T

Fig. 6.35: Primary thyrotoxicosis. Note the exophthalmos.

Figs. 6.36A and B: Note the dilated veins in an enlarged vascular thyroid gland. Auscultation is important to hear a bruit.

iou ral problem, insomnia, myopathy, unexplained diarrhoea or loss of weight, tachycardia, Graves's disease should be suspected and evaluated. The degree of thyrotoxicosis is determined by estimation of thyroid hormone levels; the severity of clinical manifestations may not correlate with the degree of thyroid hormone elevation.

Differentiating points between primary and secondary hyperthyroidism

Primary thyratoxlcosis 1. Symptoms appear first. then thyroid swelling

Secondary thyrotox/cosis 1. Thyroid swelling appears fi rst

2. Goitre is diffuse, smooth, firm or soft, both lobes are involved with thrill and bruit 3. Features are much more severe compared to that of secondary toxicosis of short duration

2. Swelling is large nodular, obvious 3. Symptoms appear after long time, which is less severe and slowly progressive compared to primary toxicosis

4. Eye signs and exophthalmos are common

4. Eye signs are not common

5. As it is an autoimmune disease, there may be hepatosplenomegaly

5. Cardiac features are more common

6. It occurs in younger age group

6. It occurs in adult and elderly individual

7. Entire gland is overactive

7. lnternodular tissues are overactive

8. There is no preexisting goitre

8. Occurs in a long-standing pre-existing multinodular goitre

Histologically, there is hyperplasia of acini, lined by columnar epithelium, often containing vacuolated colloid scalloping with pseudopapillary formation

A. Symptoms of Hyperthyroidism/Toxicosis Gastrointestinal system: Weight loss in spite of increased appetite; diarrhoea (due to increased activity at ganglionic level). Cardiovascular system: Palpitations; shortness of breath at rest or on minimal exertion; angina; irregularity in heart rate; cardiac failure in the elderly (CCF). Neuromuscular system: Undue fatigue and muscle weakness; tremor. Skeletal system: Increase in linear growth in children. Genitourinary system: Oligo or amenorrhoea; occasional urinary frequency. Integument: Hair loss, gynaecomastia; pruritus; palmar erythema. Psychiatry: Irritability; nervousness; insomnia. Sympathetic overactivity: It causes dyspnoea, palpitation , tiredness, heat intolerance, sweating, hyperactivity, iriitability, nervousness, increased appetite and decrease in weight. Because of the increased catabolism, they have increased appetite, decreased weight and so also increased creatinine level which signifies myopathy (due to more muscle catabolism). Fine tremor is due to diffuse irritability of grey matter.

T

Wayne 's diagnostic indices (clinical) of thyrotoxicosis

461

Symptoms

Present

1.

Dyspnoea on effort

+1

2.

Palpitation

+2

:I:

3.

Tiredness

+2

-I

4.

Preference to heat

5.

Preference to cold (Heat intolerance)

+5

6.

Excessive sweating

+3

7.

Nervousness

+2

8.

Appetite increased

+3

9.

Weight decreased

+3

Signs

Present

1.

Bruit over thyroid

+2

2.

Exophthalmos

+2

3.

Lid retraction

+2

4.

lid lag

+1

5.

Hyperkinetic movements

+4

B. Signs of Hyperthyroidism/Toxicosis

6.

Fine finger tremors

+1

1. Eye signs in toxic goitre

7.

Hands hot

+2

-2

Moist

+1

-1

8.

Atrial fibrillation

+4

-3

9.

Pulse rate 80/minute

0

80-90/minute

+3

More than 90/minute

+3

Eye signs are common in primary thyrotoxicosis. Lid lag, lid spasm can occur in secondary thyrotoxicosis also. 1. lid retraction: Here upper eyelid is higher than normal; lower eyelid is in normal position. It is due to sympathetic overactivity causing spasm of involuntary smooth muscle part of the levator palpebrae superioris (Muller's muscle). It is a sign of thyrotoxicosis, not a sign of exophthalmos. 2. von Graefe's sign {lid Lag's sign): It is inability of the upper eyelid to keep pace with the eyeball when it looks downwards to follow the examiner's finger. It is contraction/ overactivity of the involuntary part of the levator palpebrae superioris muscle-Muller's muscle 3. Oalrymple's sign: Upper eyelid retraction, so visibility of upper sclera. 4. Stellwag's sign: Absence of normal blinking-so staring look. Fi rst sign to appear. It is due to widening of palpebral fissure due to lid retraction and also due to contraction of voluntary part of levator palpebrae superioris muscle. 5. Joffroy's sign: Absence of wrinkling on forehead when patient looks up (frowns) with head in bent down/flexed position. 6. Moebius sign: It is lack of convergence of eyeball. Defective convergence is due to lymphocytic infiltration of inferior oblique and rectus muscles in case of primary thyrotoxicosis. There will be diplopia. It may be an early sign of eventual ophthalmoplegia.

10.

Absent

n

l>

-5

11-19 equivocal

..

0

a:

Absent

-2

>19 pointstoxic

7. Naffziger's sign: With patient in sitting position and neck fully extended , protruded eyeball can be visualized when observed from behind. 8. Jellinek's sign: Increased pigmentation of eyelid margins. 9. Enroth sign: Oedema of eyelids and conjunctiva. 10. Rosenbach's sign: Tremor of closed eyelids. 11. Gifford's sign: Difficulty in everting upper eyelid in primary toxic thyroid. Differentiates from exophthalmos of other causes. 12. Loewi's sign: Dilatation of pupil with weak adrenaline solution. 13. Knie's sign: Unequal pupillary dilatation. 14. Cowen's sign: Jerky pupillary contraction to consensual light.

Hope sees the invisible, feels the intangible and achieves the impossible.

°'•:• -I ':T '


"ti

-t

m

::0

Radioisotope study ,. Radioisotope study by 1131 (Diagnostic dose -5 microcurie is used) shows more uptake, i.e. hot nodules or hot areas. This is very useful in autonomous solitary toxic nodule. 131 ,. 1 causes more irradiation and its half-life is 8 days. So intravenous 99mTc is used for diagnostic purpose. 99m technetium has become isotope of choice for diagnosis as it is cheap, less radiation, scanning is done 20 minutes after IV injection of 99mTc (half-life is 6 hours). Drawback of technetium is that it concentrates in carcinoma, so forms hot nodule (means hot nodule need not be benign in Tc scanning). Warm nodule in Tc scan may appear as cold nodule in RAI scan and so is called as discordant nodule which suggests malignancy. If radioactive iodine is used for diagnosis, then 1123 is better as it has got short half-life (13 hours). ,. Autonomous toxic nodule is absolute indication for radioisotope scan in toxic thyroid showing hot nodule. Graves disease shows diffuse overactivity (uniform); hypofunctioning cold nodule in Graves disease cou ld be malignant. In secondary thyrotoxicosis internodular tissues are overactive (heterogeneous activity). Non-hyperthyroid toxicosis shows increased uptake in non-thyroid areas of toxicity like struma ovarii in pelvis. TRH estimation. ECG-to look for cardiac involvement; if required opinion from cardiologists is taken and cardiac problems are managed. Total count and neutrophil count are very essential base-line investigations before starting antithyroid drugs (as it may cause ag ranulocytosis). Thyroid antibodies estimation-antithyroglobulin antibody, TSH receptor antibody, antithyroid peroxidase (anti-TPO) antibody.

I Treatment for Thyrotoxicosis --

MEDICAL Relief of symptoms: - Beta blockers-propranolol, nadolol, metoprolol-Control cardiovascular and hyperadrenergic manifestations. Bronchial Asthma, heart Block, Cardiac failure are contraindications. - Calcium channel blockers (e.g. verapamil and diltiazem) can be used - Oral rehydration

One person with passion is greater than ninety-nine who have only an interest.

Contd...

°' -I '
30 mCi); administration is oral (solution/capsule [one capsule = 50 mCiJ; solution is better to take and easier to adjust the dose). RAI dose: (Thyroid mass [gram] X 80-200 uCi)/% uptake.

T

Dose ol radioactive iodine

Diagnostic

Therapeutic

• For thyroid-5-50 microcurie • For whole body iodine scan 5-10 millicurie in 72 hours

• Residual thyroid ablation-50 mCi • Bone secondaries from FCT-200-240 mCi • Lung secondaries from FCT-180 mCi

T

Radioactive isotopes used in thyroid

Isotopes 1123 ,124

Route of administration Oral

12s 1

PET scan in thyroid cancer Oral

1131

Oral

11 32

Oral Tc 99 scan* IV

Half-life 13 hr

THYROID NEOPLASMS A. Benign Follicular adenoma can be - Colloid (do not have potential for microinvasion; commonest type); Fetal (microfollicular - has potential for microinvasion) ; Embryonal (atypical - has potential for microinvasion); Hurthle ce///oxyphil or oncocytic (has potential for microinvasion); hyalinising trabecu/aradenoma. Note:

Type of rays y rays

• All adenomas are invariably follicular. • Colloid adenoma is the commonest. • The existence ol papillary adenoma is doubtful; it is invariably a lowgrade papillary carcinoma.

y rays

a. Differentiated-BO%

4 days 60 days 8 days

• Toxic thyroid in children: Radioiodine therapy is absolutely contraindicated in children below 5 years. Recurrence rate is also very high after surgery. So proposed treatment is initially antithyroid drugs are given until adolescent period andthen subtotal thyroidectomy OR Radioactive iodine therapy (after the age of 10 years). • Thyrocardiac: Severe cardiac damage (partly or wholly) resulting from hyperthyroidism, usually secondary type, requires proper opinion from cardiologists and treatment with propranolol. Subtotal thyroidectomy is the treatment. • In a patient with thyrotoxicosis, with recent onset ol proptosis: Early thyroidectomy has to beavoided, because early surgery may precipitate malignant exophthalmos. Here the patient has to be treated initially with antithyroid drugs and if required with steroids, until the proptosis remains static for six months. Then subtotal thyroidectomy is done. • Since half-life of L-thyroxine is 7 days, propranolol and antithyroid drugs have to be continued for 7 days after thyroidectomy. • T3 Thyrotoxicosis should be suspected if the clinical picture is suggestive of toxicosis, but routine tests for thyroid function are within normal range.

B. Malignant (Dunhill classification).

B, y rays

2.3 hr 6 hr

• Used mainly for malignancy in thyroid itself. It is sensitive, convenient, low radiation exposure, inexpensive, with good images but nonspecific and not used for therapy. lithium is also used as isotope for diagnosis in thyroid diseases. Technetium is better to identify nonfunctioning secondaries.

REMEMBER High dose of retinoic acid will make 1131 to concentrate in tumour cells (70 mg/daily for 2 weeks) Fertility should be avoided for 1 year after 1131 therapy Avoid contrast CT in thyroid diseases as much as possible because 1131 study in later period will be difficult MRI is ideal when radioiodine therapy is needed Note:

• Toxic thyroid in pregnancy: Radioiodine therapy is absolutely contraindicated in pregnancy (High-risk to foetus). Antithyroid drugs can be administered carelully. But, the problem here is that both TSH and antithyroid drugs cross the placental barrier and baby born may be hypothyroid and goitrous. Propylthiouracil is preferred in pregnancy. SubtotaVtotal thyroidectomy can be done in second trimester.

1. Papillary carcinoma (60%) . 2. Follicular carcinoma (17%). 3. Papillofollicular carcinoma behaves like papillary carcinoma of thyroid. 4. Hurthle cell carcinoma behaves like follicular carcinoma.

b. Undifferentiated-20% Anaplastic carcinoma (13%)

c. Medullary carcinoma (6%) d. Malignant lymphoma (4%) e. Secondaries in thyroid (rare)-from colon, kidney, melanoma, breast.

I Incidence and Spread Annual incidence of thyroid cancers is 3.7 per 1,00,000 population. It is common in females (3:1 ). Papillary carcinoma mainly spreads through lymphatics; follicular through blood; anaplastic through lymphatics and blood.

I Aetiology of Thyroid Malignancy Radiation either external or radioiodine can cause papillary carcinoma thyroid. There was increased incidence of thyroid

Dissatisfaction and discouragement are not caused by the absence of things but the absence of vision.

487

468

carcinoma among children following exposure to ionising radiation after the Chernobyl nuclear disaster in Ukraine in 1986; in children in Marshall island after atomic bomb testing. Earlier irradiation was practised to head and neck region to treat benign conditions like tonsillitis, adenoids, thymus enlargement, acne vulgaris, haemangiomas during first two decades of life. As a consequence papillary carcinoma of thyroid became common in these individuals. Radiotherapy received in adolescent period for Hodgkin's lymphoma may predispose to papillary carcinoma. Pre-existing multinodular goitre. It can turn into follicular carcinoma of thyroid. Medullary carcinoma thyroid is often familial. Hashimoto's thyroiditis may predispose to NHUpapillary carcinoma of thyroid. Familial. Elevated TSH is observed in papillary carcinoma of thyroid. Genetic-Cowden syndrome is differentiated thyroid carcinoma, carcinoma breast, multiple hamartomas. It is due to germ cell mutation of PTEN tumour suppressor gene. Oncogenes-C myc, Cerb, Cfas, Ras are associated thyroid neoplasms.

B

DIFFERENTIAL DIAGNOSIS FOR CARCINOMA THYROID I I

r

• I

f

•

PAPILLARY CARCINOMA OF THYROID (PCT) It is 70-80% common. Common in females and younger age group.

I Aetiology (see above) Radiation either external or radioactive iodine therapy. TSH levels in the blood of these patients are high and so it is called as hormone dependent tumour.

Papillary carcinoma

.,•'· ••

Thyroid Trachea

Enlarged lymph nodes

.::

Fig. 6.40: Papillary carcinoma of thyroid with nodal infiltrationdiagrammatic representation.

B Types

l

_________

i. Occult primary (.,·,;

·.

-

Fig. 6.48: Total thyroidectomy specimendone for carcinoma thyroid.

I Follow-up It is by radioisotope 1123 scan done at regular intervals (6 months to one year) to look for secondaries. Thyroglobulin estimation is a good follow-up method to decide for radioisotope study. Normal value (3-40 ng/mL). TG >50 ng/mL is abnormal. It should be done once in 3-6 months. Its raise sig nifies recurrent/metastatic disease. Serum thyroglobulin level estimation is of no value in preoperative assessment. After thyroidectomy thyroglobulin secretion is stopped, hence its level should not be traceable after total thyroidectomy. Ultrasound neck or MRI neck to identify early relapse. MRI neck is better.

Further Treatment If secondaries are detected therapeutic dose Ra 1131 is given orally. L-thyroxine has to be stopped 6 weeks prior to RT, then required dose of Ra 1131 is given (50-150 m curie). Radio remnant ablation (RRA) ,, Initially thyroid radioisotope scan is done. If patient is on 300 µg suppressive dose of L-thyroxine, it should be stopped for 3-6 weeks so as to achieve serum TSH level above 30 M IU/I or two intramuscular injections of 0.9 mg of recombinant human TSH is given.

I Hurthle Cell Carcinoma Hurthle cell carcinoma is a variant of follicular carcinoma of thyroid which contains abundant oxyphi/1 cells. It spreads more commonly to regional lymph nodes than follicular carcinoma of thyroid. Note:

• • • • • • • •

Hurthle cell carcinoma does not take up 1131 It secretes thyroglobulin It has got poorer prognosis than follicular cell carcinoma 30% multicentric 99 mrc sestamibi scan is very useful for Hurthle cell carcinoma Regional nodes are more commonly involved than follicular carcinoma 20% show distant spread Abundant oxyphill cells (Askanazy) are specific Total thyroidectomy, MRND and TSH suppression is the treatment

DIFFERENTIATED THYROID CARCINOMA (OTC) OTC is a spectrum of disease derived from follicular cells. Both papillary and follicular carcinomas are grouped under this. 90% of thyroid malignancies are differentiated one. Papillary (PTC), follicular (FTC) and Hurthle cell carcinomas are DTCs. Insular variety is poorly OTC having intermediate position between OTC and anaplastic; it can cause bone and lung spread; it is common in younger age group; p53 and p21 are negative; with 40% 10-year survival. AGES (Mayo Clinic, Hay); AMES (Lahey clinic); MACIS; Sloan Kettering scoring - are different scoring systems used for DTCs. Sloan Kettering scoring includes low, intermediate and high-risk groups. First three scoring systems have low- and high-risk groups. Papillary spreads through nodes; follicular through blood. FCT causes pulsatile vascular secondaries in skull. Incidence of thyrotoxicosis in DTCs is 2%.

Galectin - 3, RET/PTC rearrangements, CD44, leukocyte common antigen (LCA), cytokeratin are the probable tumour markers under evaluation. For indeterminate, FNAC molecular markers are used to identify RAS and BRAF mutations. TNM staging tor DTCs (AJCC 2018) are: ,., T - Tumour: Tx - Primary cannot be assessed; TO - no evidence of primary tumour; T1 -

""O

-i

m

;J:>

°'

474

... ...:::,C'I >, QJ

V'I

0

IU

:::, C: IU

cc a: Ill

V'I

I

and recurrent laryngeal nerve injury. Lateral cervical node Follow-up dissection (LCND. Nodes -IIA, Ill, IV and VB) is done only Proper clinical examination in the neck for residual/nodal when it shows the evidence of involvement (by FNAC or US); disease and for distant spread. there is no role for prophylactic LCND. Serum thyroglobulin measurement - s0.2 ng/mL (IRMA Only hemithyroidectomy is done if tumour is 1 cm, extracapUSG-guided FNAC of lymph node and Tg measurement in sular thyroid invasion or locoregional extension, high-risk FNAC fluid washout (FNAC-Tg). group, unfavorable histological subtype (follicular, diffuse When there is negative I131 scan with elevated TG during sclerosing, or tall cell-variant papillary cancer), multifocal follow up, PET scan is indicated. disease; BRAF positive tumor in the specimen. Criteria for RAI are-TSH should be >30 mU/ml. L-Thyroxine replaceCentral Node Compartment Neck Dissection ment is stopped 3- 4 weeks before radioiodine treatment; OR - in time being switch over to T3 80 µg/day and RAI in It is removal of paratracheal, tracheo-oesophageal, pretracheal 7 days; OR - When L-thyroxine withdrawal is not advisable, and prelaryngeal nodes along with thymus and thyroid enbloc TSH stimulation was achieved using Recombinant Human extending from hyoid bone above, brachiocephalic vein below, Thyrotropin (rhTSH) (0.9 mg IM one dose-2 consecutive carotids on both sides. days; after 24 hours RAI given). Dose of 1131 - usually-3700 Completion Thyroidectomy MBq (100 mCi); for metastases, it is 200 mCi. It is done after hemithyroidectomy if histology confirms as Suppression hormone therapy using high dose L thyroxinedifferentiated thyroid cancer, either papillary or follicular. dose is to make TSH level 50% of MTG patients. CEA >30 mg/ml indicates incurability by surgery; CEA> 100 mg/ ml suggests extensive nodal spread; raising CEA with stable calcitonin indicates dedifferentiation and poor prognosis. CT neck, chest, abdomen should be needed for metastatic work up. CT abdomen is also done for pheochromocytoma. Urinary metanephrine, VMA, (24 hours) shou ld be done in suspected pheochromocytoma. Serum, calcium and parathormone (PTH) estimation for hyperparathyroidism. 11 Hndium octreotide scanning is useful in detecting MCT (70% sensitivity). It is also useful in postoperative follow up to find out residual or metastatic disease. Genetic testing for RET mutations.

I Treatment Surgery is the main therapeutic modality. Total thyroidectomy with bilateral central node dissection and ipsilateral lateral neck dissection if primary tumour is > 1 cm or central nodes are positive. Positivity of central nodes is 81 %. Opposite lateral neck node dissection is done if US neck shows opposite nodes or extensive ipsilateral neck nodes when present or bilateral primary tumours (multifocal/ bilateral). Thyroxine replacement/maintenance therapy 100 ug in the morning before food daily, is needed. No role of suppressive hormone therapy or radioactive iodine therapy. External beam radiotherapy for residual tumour disease. Somatostatin/octreotide for diarrhoea.

Adriamycin is the drug used as chemotherapy with limited results. If there is associated phaeochromocytoma, it should be treated surgically by adrenalectomy first and later only total thyroidectomy is done. All family members of the patient should be evaluated tor serum calcitonin and if it is high they should undergo prophylactic total thyroidectomy (Can also be assessed by genetic evaluation). If there is positive RET proto-oncogene in MCT with MEN II A and familial MCT types, prophylactic total thyroidectomy is done at the age of 5 years. In positive RET proto-oncogene in MCT with MEN 11 B, prophylactic total thyroidectomy is done at the age of one year. MCT with associated parathyroid hyperplasia (30%) in MEN IIA, total thyroidectomy with central nodal dissection with total parathyroidectomy and autotransplantation of half of gland in sternomastoid or nondominant forearm brachioradialis muscle is done. Other therapies External RT; Somatostatin analogues; lmatinib mesylate tyrosine kinase inhibitor; Chemotherapy-less successadriamycin, capacetabine, 5 FU, irinotecan; newer targeted therapies.

I Prognosis Sporadic MCT and MCT with MEN syndrome II are aggressive. Familial MCT not associated with MEN II syndrome has got better prognosis. Presence of nodal disease carries poor prognosis. Survival is overall good- 1Oyears-85%; depends on type, familial nature, association for MEN syndrome; status at the time of presentation-size, nodal status, distant spread. Incidence of recurrence is 50% in MCT.

I Follow-up Serum calcitonin and CEA level. Imaging as needed-USG/CT neck and mediastinum. Recurrence in the neck should be treated with resurgery exploration and disease clearance. External beam RT may be useful also. PET scan; MIBG scan are also useful. Follow-up for late onset pheochromocytoma or hyperparathyroidism should be regularly assessed, annually.

I Calcitonin It is a polypeptide of 32 amino acids. It is derived from ultimobranchial body. It is secreted from C cells of thyroid (parafollicular cells). It lowers the plasma calcium and phosphorus levels. It blocks the PTH-induced bone resorption. Calcium from the

Only in advanced cases of hypothyroidism there is a substantial loss of hair.-Richard AJ Asher

477

478

...>...:::,C'I QI

V'I

0

ro

:::, C

ro

co"' a:

V'I

circulation is shunted into the bone. It increases the excretion of calcium , phosphorus, sodium and potassium. It rapidly lowers the serum calcium. Normally, it is less than 0.08 ng/L (u ndetectable). It is increased in medullary carcinoma of thyroid. It is very good tumour marker for MCT. It confirms the relapse/metastases/residual disease. Increased levels in family members confirm the genetic relation and such relatives should undergo prophylactic total thyroidectomy. Calcitonin level will further increase after injection of calcium 2 mg/kg or pentagastrin 0.5 µg/kg. In disease-free individual, after therapy for MCT calcitonin level decreases. Calcitonin as a therapeutic agent is used in hypercalcemia, Paget's disease, bone pain of neoplastic diseases, menopausal osteoporosis. Calcitonin (pork), 4 units/kg is given SC or IM three times a week. Salmon calcitonin SC/IM/nasal spray-50-400 units can be given three times a week or daily depending on need.

MALIGNANT LYMPHOMA It is NHL type. Occurs in a pre-existing Hashimoto's thyroiditis (Not proved well). FNAC is useful to diagnose the condition (Often trucut biopsy). Chemotherapy and radiotherapy is the main treatment. Rarely total thyroidectomy is done to enhance the results.

HASHIMOTO'S THYROIDITIS (Struma Lymphomatosa) Also called as diffuse non-goitrous thyroiditis. It is an autoim mune thyroiditis-common in women (15 times more common). There is hyperplasia initially, then fibrosis, eventually infiltration with plasma cells and lymphocytic cells. Askanazy cells are typical (like Hurthle cells). The river Struma arises in Bulgaria and flows into Aegean Sea. Struma means goitre. Banks of this river are endemic area for goitre. Painful, diffuse, enlargement of usually both lobes of thyroid which is firm, rubbery, tender and smooth (occasionally one lobe is involved). Initially they present with toxic features, but later, they manifest with features of hypothyroidism. Hyperplasia Hyperthyroid-Hashitoxicosis Euthyroid. Fibrosis Hypothyroid. There may be hepatosplenomegaly, It is often associated with other autoimmune diseases. In 85% cases significant rise in the thyroid antibodies (microsomal, thyroglobulin, or colloid antibodies) is observed. Common in perimenopausal females. It can predispose to papillary carcinoma of thyroid .

Often condition may be associated with or may predispose to malignant lymphoma. It is, at present, not well-proved. Investigations: FNAC, T3, T4, TSH. Thyroid antibodies assay. Usually ESR is very high (over 90 mm/hour). Treatment: ,. L-thyroxine therapy. , Steroid therapy often is helpful. , If goitre is large and causing discomfort, then subtotal thyroidectomy is done.

DE-QUERVAIN'S SUBACUTE GRANULOMATOUS THYROIDITIS It is due to viral aetiology either mumps or coxsackie viruses causing inflammatory response with infiltration of lymphocytes, neutrophils, multinucleated giant cells. Painful diffuse, swelling in thyroid which is tender Commonly seen in females. Initially there is transient hyperthyroidism with high T3 and T4 but poor radioiodine uptake. FNAC is useful. It is usually a self-limiting disease. Prednisolone 20 mg for 7 days helps.

RIEDEL'S THYROIDITIS 0.5% Common. A very rare benign entity wherein thyroid tissue is replaced by fibrous tissue which interestingly infiltrates the capsule into surrounding muscles, paratracheal tissues, carotid sheath. (' Woody Thyroiditis', 'Ligneous Thyroiditis). It is often associated with retroperitoneal and mediastinal fibrosis and sclerosing cholangitis. There is both intrathyroidal as well as extrathyroidal fibrosis. It also encroaches parathyroids and recurrent laryngeal nerves. It may be unilateral or bilateral. Swelling with irregular surface, stony hard consistency, strider, with positive Berry's sign (absence/impalpable carotid pulsation); small goitre; common in males. Differential diagnosis: Anaplastic carcinoma of thyroid. Investigations: , T3, T4 may be low due to hypothyroidism. , Radioisotope scan will not show any uptake. , FNAC to rule out carcinoma. Treatment: , lsthmectomy is done to relieve compression on the airway. They require L-thyroxine replacement later, as hypothyroidism is common. , High dose of steroid often used. ,. Thyroidectomy is not necessary.

B Previous MNG undergoing malignant transformation Haemorrhage into a nodule Anaplastic carcinoma of thyroid

other purpose as "non-thyroid "imaging modality, a thorough specific sonographic evaluation of the thyroid gland should be performed in all thyroid incidentalomas. A sonographically confirmed thyroid nodule should be managed like any other clinically identified thyroid nodule/disease.

THYROIDECTOMY

• Scabbard trachea in longCarcinoma of thyroid standing MNG • Riedel's thyroiditis • Retrosternal goitre REMEMBER Goitre is enlargement of the thyroid gland • Solitary nodule is single palpable nodule on clinical or sonological examination without palpable rest of the gland • Dominant nodule is single nodule with palpable enlargement of the remaining thyroid gland • Thyroid swelling is confirmed by its movement with deglutition due to attachment of enclosed pretracheal fascia to inferior constrictor muscle which is attached to trachea and cricoid cartilage and so moves with deglutition • Berry's ligament is condensed vascularised pretracheal fascia postero-supero-medially. It is important as it is close to recurrent laryngeal nerve Any thyroid swelling can be malignant unless proved otherwise Ultrasound neck, FNAC, estimation of T3, T4, TSH are essential investigations MRI neck is needed in large goitre and fixed or malignant thyroid Radioisotope study 1123 is done only in selected cases like borderline toxicity, ectopic thyroid, retrosternal goitre and after thyroidectomy in follicular carcinoma thyroid to see secondaries during follow-up period • Normal thyroid gland is usually not palpable • A rare entity called as black thyroid shows lipofuscin deposition in thyroid in a patient who is on longstanding tetracycline therapy which may interfere with thyroid function

• Anaplastic carcinoma thyroid-often • Carcinoma thyroid with extensive local infiltration into soft tissues, trachea/larynx and posterior muscles • lntrathoracic retrosternal extension with infiltration/impaction • Riedel's thyroiditis with encasement of trachea • Massive thyroid wherein upward movement is difficult to observe and appreciate

THYROID INCIDENTALOMA Thyroid incidentaloma is defined as an unsuspected, asymptomatic thyroid lesion that is discovered on an imaging study or during an operation unrelated to the thyroid gland. Thyroid incidentalomas are most commonly detected on ultrasound , followed in frequency by CT scan and MRI, carotid duplex scanning and PET scan. The incidence of carcinoma in incidentaloma is not insignificant. As these imaging methods are done for some

I Types

1. Hemithyroidectomy Along with removal of one lobe, entire isthmus is removed. It is done in benign diseases of only one lobe. It is also done in follicular neoplasm involving only one lobe. Solitary toxic or nontoxic nodule, thyroid cyst are other indications. 2. Subtotal thyroidectomycommonly done in toxic thyroid either primary or secondary and also often for nontoxic multinodular goitre. Here about 8 grams, or a tissue, size of pulp of finger is retained on lower pole, on both sides and rest of the thyroid gland is removed. It is also done in MNG. 3. Partial thyroidectomy (By Thomas) is removal of the gland in front of trachea after mobilisation. It is done in nontoxic multinod ular goitre. Its role is controversial. 4. Near total thyroidectomy Here both lobes except the lower pole (one or other sides) which is very close to recurrent laryngeal nerve and parathyroid is removed (To retain blood supply to parathyroids). It is done in case of papillary carcinoma of thyroid . Here less than 2 grams of thyroid tissue is left behind near its lower pole on one side usually opposite side of the diseased, occasionally on both sides. 5. Total thyroidectomy Entire gland is removed. It is done in case of follicular carcinoma of thyroid, medu llary carcinoma of thyroid. 6. Hartley Dunhi/1 operation is removal of one entire lateral lobe with isthmus and partial/subtotal removal of opposite lateral lobe. It is done in non-toxic multinod ular goitre . 4 grams of tissue is left behind only on one side.

Blood grouping and cross matching. Keep the required blood ready • Indirect laryngoscopy. Patient is asked to tell' E' to check the abduction of vocal cord Videostroboscopy - it is special method to visualize the vocal cord vibration. 70 degree rigid strobolaryngoscope is used with mounted microphone. Flexible laryngostroboscope also can be used. Patient in sitting position, videostroboscope is passed to visualize vocal cords and patient is asked to tell eeee to examine vocal cord vibration as a slow motion. Serum calcium estimation

• • • •

T3, T4 , TSH Thyroid antibodies ECG and cardiac fitness especially in toxic goitre Lugol's iodine 10 days prior to surgery to make gland firm and less vascular.

Everything has its beauty but not everyone sees it.

479

rocedure >. ,.. Q)

...:::, ....0

C'I

VI

Its

:::, C

Its Ill

a:i 0:: VI

Position: ,. Under general anaesthesia patient is put in supine position with neck hyperextended by placing a sand bag under shoulder-with table tilt of 15 degree head up to reduce venous congestion (Rose position). Incision: Horizontal crease incision is done, two-finger breadth above the sternal notch, from one sternomastoid to the other (Kocher's thyroid incision) (Posterior margin of sternomastoid). Procedure: Skin and platysma are incised (Subplatysmal plane)upper flap raised up to thyroid cartilage, lower flap up to sternoclavicu lar joint. Deep fascia is opened vertically in the midline. Strap muscles are retracted; in large goitre they are often divided in upper part to retain their nerve supply ansa cervicalis. Often anterior jugular veins need to be ligated using 3 ze ro vicryl.

Sternomastoid muscle

Pulp of the finger or 4-8 gm thyroid in lower pole both sides

Fig . 6.55: Subtotal thyroidectomy-it is done in toxic/ nontoxic multinodular goitre. Most of the gland except lower pole (4·8 gram) on both sides is removed. (Thyroid tissue in tracheo-oesophageal groove) Part retained

Part removed

l

Part of both lateral lobes and entire isthmus

--f-Sternomastoid muscle

Two-finger breadth

i

Fig. 6.56: Partial thyroidectomy- it is done in nontoxic nodular goitre if there is adequate normal gland posteriorly. Tissue in the tracheooesophageal groove is retained. Isthmus and gland with nodules in front is removed. It is not commonly done now.

Fig. 6.53: Note the incision for thyroid surgery. Except small tissue in lower pole near recurrent laryngeal nerve and parathyroids (one or both sides-1 g/1 cm)

Part retained

\U IEI;Jf-:--'---

laryngeal nerve

Part removed

t \

Both lateral \ lobes and isthmus\

-'t;H '- Recurrent

---

n::.-=--"l"''r:::~ s...:::,C'I (I/

111

0

:::, C:

"'

IX)

a:

111

T

Complications ol thyroidectomy

Metabolic Hypoparathyroidism • Temporary hypoparathyroidism • Temporary hypocalcaemia without hypoparathyroidism (hungry bone syndrome) Permanent hypoparathyroidism Spurious hypoparathyroidism (total calcium is less but ionized calcium is normal) Thyroid crisis Hypothyroidism/thyroid failure/myxoedema lntraoperative Haemorrhage Nerve injuries

.. ..

Nerve Injuries External laryngeal nerve injury - pitch of the voice is lost Recurrent laryngeal nerve injury Vascular complicati ons Haemorrhage - primary and reactionary Haematoma formation Compromised trachea-oesophageal blood supply

.. ..

Immediate postoperative

..

. . .. .

.. Haematoma

..

Laryngeal oedema Respiratory obstruction - stridor Hypoparathyroidism

Other causes are-infection, trauma, pre-eclampsia, diabetic ketoacidosis, emergency surgery, stress, drugs like anticholinergics or antiadrenergic or NSAIDs or chemotherapy, diabetes mellitus.

Features They present 12-24 hours after surgery; but on table also, it can occur occasionally. Hyperpyrexia (>41°C), severe dehydration, circulatory collapse, hypotension, palpitations, tachycardia, tachypnoea, hyperventilation, cardiac arrhythmias, cardiac failure. GI symptoms like vomiting, diarrhoea, jaundice. Restlessness, irritability, delirium, tremor, convulsions and coma can occur. Bailey's symptom complex of thyroid storm are - insomnia, anorexia, diarrhoea, vomiting, sweating, emotional instability, fever, tachycardia, aggravated toxic features, multiorgan dysfunction. Burch-Wartofsky score (1993) is used to identify or predict the thyroid storm using different parameters - score of below 25 excludes storm; sc'ore 25-45 suggests impending storm; more than 45 means thyroid storm. Death may ensure suddenly. Differentia diagnoses are- malignant hyperpyrexia, septic shock, anxious status, cardiac disorders. Investigations: Raised T3, T4, suppressed TSH, raised serum calcium, ECG and echocardiography shows changes, raised total count, altered liver function tests, arterial blood gas (ABG) shows changes; altered electrolytes.

Treatment Supportive measures: Rehydration by proper fluid therapy, tepid sponging , cooling blankets, antipyretics like paracetamol IV infusion, glucocorticoids (Hydrocortisone 500 mg IV or dexamethasone injections), IV dextrose infusion as there is more metabolic demand, correction of electrolytes, treating cardiac arrhythmias, ICU care with ventilator support. Central line, CVP monitor, nasogastric tube, urinary catheter should be placed.

Other complications Respiratory obstruction Skin flap necrosis - rare Infection Discharge and sinus formation • Wound granuloma Keloid formation

.

Late postoperative Hypothyroidism Recurrent nodule • Recurrent toxicity • Wound infection

Antiadrenergic drugs: Propranolol 80 mg orally or through nasogastric tube 6th hourly, or IV propranolol 1 mg IV in 1O minutes followed by 2 mg in 10 minutes as per need . When propranolol is contraindicated in asthma, heart block and failure, cardioselective beta blockers like atenolol, metoprolol can be used; esmolol IV loading dose as 500 ugm/kg followed by 100 ug/kg minute infusion is also effective. Reserpine 500 ug/kg loading dose, then 5 ug/kg/minute; heart rate should be

"'ti

-t

m

::::c

......

C

... -0.5. Assay of ·Intact PTH' is more relevant; Intact PTH is biologically active PTH molecules. "whole" or "biointact" parathyroid hormone assays using two different antibodies against two different segments of parathyroid hormone is still more specific. Serum phosphate level will be decreased in primary HPT; but in secondary HPT serum phosphate level is increased. Bone density assessment. Vitamin D estimation. Increased urinary calcium level >250 mg/24 hours. Raised serum alkaline phosphatase level. X-ray skull shows salt and pepper appearance; X-ray phalanges (subperiosteal bone resorption and tufting of distal phalanges) and jaw are also specific showing osteitis fibrosa cystica; X-ray spine may show Rugger jersey spine. Dual-energy X-ray absorptiometry (DXA) for evaluation of

bone mineral density (BMD) is very useful.

B Pepper lesions in the skull Sub-periosteal erosion of radial side of middle phalanx Calcification in bones

Ultrasound abdomen to find out problems in kidney, pancreas. Ultrasound neck and CT (Multiphase 4 D CT) MRI scan neck and mediastinum.

Figs. 7.3A and B: X-ray of humerus bone and hand bones showing bone features-brown tumour-osteitis fibrosa cystica.

Selective venous sampling for PTH is also very useful. Thallium-Technetium scan shows hot spots which is diagnostic of parathyroid adenoma. Technetium-99m labelled Sestamibi isotope scan is better and sensitive (80%) than Thallium-Tc scan. As it is very expensive, it is used in parathyroid re-exploration. It is often combined with single photon emission computerised tomography (SPECT). Urinary cAMP level increases in 90% cases. Angiography, venous sampling, USG-guided biopsy are other methods.

I Differential Diagnosis Secondaries in the bone-due to secretion of PTH related polypeptide by tumour. Actual PTH is suppressed. Multiple myeloma. Vitamin D intoxication.

Sarcoidosis. Functioning carcinoma. Familial hypocalciuric hypercalcaemia is an autosomal dominant disease with mild raise in serum calcium and PTH levels secondary to mutation in the cell membrane calcium receptor. Urinary calcium excretion is low. It does not require parathyroidectomy. Calcium creatinine clearance ratio is less than 0.01 in this condition whereas it is >0.02 in primary HPT. It is due to heterozygous mutation in calcium receptor gene (chromosome 3).

I Treatment B • • • • • • •

Severe symptoms Young age group Markedly reduced bone density Serum calcium more than 11 mg% Urinary calculi Neuromuscular presentations Urinary calcium more than 400 mg/24 hours Surgical removal of the all four glands and implantation of fragments of the gland in forearm muscle is done, i.e. to brachioradialis muscle (commonly used muscle) or necksternomastoid (not commonly used now). Marker stitch is placed at the transplantation site. 1/2 or 1/3rd of one gland or 100 mg of parathyroid gland is autotransplanted-total parathyroidectomy. Three and half glands are removed and half of one parathyroid gland (most normally appearing gland [commonly one half of one inferior parathyroid]) is retained in situ with marker stitch using non absorbable suture material- subtotal parathyroidectomy. If it is carcinoma, additional hemithyroidectomy with postoperative radiotherapy is required. Adenoma when occurs in one gland with normal other glands, removal of that gland with adenoma may be sufficient. When all four glands are diseased, transce rvical thymectomy is also added along with total parathyroidectomy to reduce persistent and recurrent disease. In familial and MEN syndromes, total parathyroidectomy is better. If it is mediastinal parathyroid adenoma, after proper localisation thoracoscopic removal may be sufficient. Nonsurgical care like good hydration, regular exercise, avoiding immobilisation, daily calcium and vitamin D intake is done in patients who have recovered from acute crisis. Estrogens reduce the calcium level and maintain bone density. Raloxifene, a selective estrogen receptor modulator also reduces the serum calcium level. Biphosphonates mainly alendronate improves the bone mineral density (BMD) in primary HPT but without altering the levels of

PTH or calcium. Cinacalcet, a calcimimetic drug activate calcium receptor and reduces the levels of PTH and calcium but not increasing the BMD. Calcitonin, mithramycin are other drugs used. Mithramycin is used once a week but is hepatotoxic and also causes thrombocytopenia. But medical treatment for primary HPT is less effective and not popular. PROBLEMS IN PARATHYROIDECTOMY

• Permanent hypoparathyroidism Persistent hyperparathyroidism- 5% Recurrent hyperparathyroidism-hypercalcaemia recurs 12 months after first parathyroid surgery Recurrent laryngeal nerve injury-1 % Often needs additional thyroidectomy • Variations in positions of the gland especially lower-may be in mediastinum Sudden drop in calcium level after surgery due to increased L absorption of calcium by bones-hungry bone syndrome _

493

C"")

:::c

>

m

:::z:, ..., C

... -...:::r ""C

I» I»

'< 0

C.

en

I» ::I C.

...>

C.

a, ::I I»

vi

PARATHYROIOECTOMY

I Indications in Pri mary HPT Symptomatic HPT Asymptomatic HPTwith-Criteria (2002) for surgical intervention are: :.- Raise in serum calcium level more than 1 mg/dl of upper limit of the normal calcium range; , 24 hour urinary calcium if more than 400 mg; creatinine clearance when reduced more than 30%; , Bone density greater than 2.5 standard deviations below peak bone mass in lumbar spine/hip/lower end of radius; , Age below 50 years; , When medical therapy is not possible.

I Indications in Secondary HPT Here indication for removal of all four glands with auto-transplantation of parathyroid is only in severe cases or with renal osteodystrophy.

I Preoperative Preparation Vocal cords should be assessed by preoperative indirect laryngoscopy. High calcium levels preoperatively may require treatment with hydration; diuresis; steroids (prednisolone 20 mg TIO for 5 days before surgery); 100 mmol phosphate infusion in 6 hours: 200 units calcitonin subcutaneous injection for 5 days twice daily before surgery; diphosphanate-etiodronate disodium 7.5 mg/ kg daily as slow IV infusion for 3 days; mithramycin 25 µg/kg as single dose.

Listen for what is felt as well as said; focus on the present; be willing to forgive.- Charles Edward Brown-Sequard

494

I Anaesthesia and Position General anaesthesia is used with neck hyperextension by placing rolled sheet under the shoulder blades. Head is placed on the head ring; head end of the table is raised to semi-erect position (Semi-Fowler position).

I Incision and Dissection It is same as for thyroidectomy. Flaps are raised in similar way. Strap muscles are separated after opening the deep fascia in the midline. Thyroid gland is mobilised to identify the parathyroid adenoma. Parathyroid having adenoma is mobilised which is close to recurrent laryngeal nerve. End artery of the parathyroid is identified and ligated. Adenoma is separated from adjacent thyroid tissue using gauze dissection. Either on table venous sampling for PTH assay is done or venous sample from cubital vein is done for PTH assay. Parathyroid may be confirmed by frozen section biopsy or on table aspiration of parathyroid tissue which is analysed for PTH assay which will be more than 1500 pg/ml (confirms that removed tissue is parathyroid). Total parr,thyroidectomy is done for parathyroid hyperplasia by removing all fou r glands and one-third of one gland is autotransplanted into the forearm muscle (brachioradialis) or sternocleidomastoid muscle with marker stitch. Gland to be transplanted is sliced into 1 mm pieces and around 18 pieces are embedded in decided muscle with a marker stitch or clip. If in postoperative period patient still presents with features of primary HPT; transplanted area is re-explored and further reduction in parathyroid tissue is done. Wound is closed with proper haemostasis.

I Complications Haemorrhage, recurrent laryngeal nerve palsy, hypocalcaemia and hungry bone syndrome are known to occur. Persistent HPT(serum calcium does not normalise immediately after surgery leading to total failure) or Recurrent HPT (serum calcium after surgery becomes normal but in 6-12 months, it again increases) may be a problem. Hypoparathyroidism with severe hypocalcaemia is a problem when all glands are removed (3½) with bilateral neck exploration. 1O ampoules of calcium gluconate is diluted with one liter of normal saline and given as continuous infusion at a rate of 30 ml/hour- initial method of management. Hypomagnesemia should also be corrected. Migration or inability to identify the transplanted parathyroid is often a problem in autotransplantation of parathyroid. Hungry bone syndrome ,. It occurs usually in patients with preoperative hyperthyroidism. They have increased bone breakdown in their hyperthyroid state. When a patient's thyroid hormone level

drops acutely after surgery, stimulus to break down bone is removed. The bones are now "hungry" for calcium, remove calcium from the plasma rapidly. :.- It usually occurs after parathyroidectomy; thyroidectomy for toxic thyroid; prostate cancer patients on estrogen therapy. Sudden cessation of existing increased bone breakdown makes bones to absorb calcium, magnesium and phosphorus rapidly. Calcium levels in blood prior to operation cannot predict hungry bone syndrome. , Hypocalcaemia, hypophosphataemia, hypomagnesemia and hyperkalaemia are four typical features in these patients. Estimation and correction of all these four factors is essential. ECG changes can occur. Hyperkalaemia should be treated judicially. Magnesium infusion is needed. There is an unusually high need for calcium, with a low calcium excretion in the urine. , Bone-specific alkaline phosphatase (ALP) continues to rise in the first few weeks indicating increased bone reconstruction. , Supplementation of vitamin D and elemental calcium is needed during discharge for 6 months. , Postoperatively they need calcitriol (gradually increased to 16 mg in 1 month, then gradually reduced) with 2 g calcium supplement. Monitoring is done by evaluating serum calcium, albumin, magnesium, phosphorus and bone specific alkaline phosphatase. Injury to recurrent laryngeal nerve, oesophagus can occur.

I Effects of Surgery Among neuromuscular symptoms of primary HPT, proximal muscle weakness responds better than respiratory muscle weakness by parathyroidectomy. Among psychiatric illnesses, depression and spatial learning and processing improve well by surgery. Bone mineral density in hip and lumbar spine becomes better. Nephrocalcinosis is improved by surgery; but hypertension and renal excretion will not improve much. Half life of PTH is 4 minutes. On table serial PTH assays are done at several intervals-before dissection of the gland, during dissection and after dissection. 50% reduction in PTH level from baseline in post-removal sample is a 96% predictor of complete removal. Operative failure rate is 1.5 to 6%. lntraoperative PTH assay improves the success rate very much (76-94%). Cure rate is defined as normocalcaemia in 6 months postoperative period. Radio-guided parathyroidectomy using intravenous injection of 20 mCi of 99mTc sestamibi 2 hours before surgery is not routinely practiced but may be useful for removal of adenoma appropriately. Hand-held quantitative gamma counter is used intraoperatively on the neck over all parathyroid tissues.

I Surgical Approaches

Endoscopic Parathyroidectomy

Classic Approach (Traditional Approach) It is under general anaesthesia exploring bilateral neck to remove

parathyroid tissue which is confirmed by frozen section biopsy. It shows 95% cure rate with 2% complication rate.

Entire parathyroidectomy is done using laparoscopy. It is first used (1994) for a mediastinal parathyroid adenoma. In 1996, it was used for neck parathyroid hyperplasia by Gagner for removing 3½ glands. Now technique is limited to single adenoma to remove tumour and gland. Low pressure insufflations with 5 mm four trocars are used. Placement of trocars is dependent on the need of the operating su rgeon. Many place trocars on one side only; but few prefer to place working trocars on opposite side.

Median Sternotomy (3%) Extension Median sternotomy is often needed when parathyroid is in anterior mediastinum along with thymus. Often parathyroids may be 5, 6 or 7 in numbers instead of four.

495

C")

:::c

>

"'Cl -4

m

::J:I --.J C

... :::r 50% patients they are bilateral and multicentric. MEN II b is more aggressive, commonly seen in

infancy and early childhood. Total parathyroidectomy with total thyroidectomy and adrenalectomy is the treatment. Evaluation of MEN syndrome: Family history; biochemical screening with parathormone, serum calcium, prolactin, growth hormone, blood sugar, insulin, proinsulin, pancreatic polypeptide, glucagons, gastrin levels, calcitonin, urinary catecholamines estimation; genetic screening from isolated DNA from peripheral blood white cells for ret proto oncogene.

APUDOMAS APUD (Amine Precursor Uptake Decarboxylation) cells are cells having specific cytochemical characteristics. They are: ,, High amine content. ,, Capacity of amine precursor uptake. ,, Property of decarboxylation of these precursors to form amines. Initially it is thought that APU D cells are derived from neural crest cells (Pearse) but now found to be from endoderm. Cells share similarities in structure, properties, histological, histochemical, immunocytochemical and electron microscopic appearance. Neuron specific enolase enzyme is specific for these cells. These cells have got capacity to synthesize peptides which has got different modes of action. They are: 1. Endocrine action where peptides get secreted into circulation to have distant target actions. 2. Paracrine action where peptides get secreted locally to have action at local sites. 3. Neurocrine action where peptides act as neurotransmitter at neuronal synapses. 4. Neuroendocrine action where peptides stimulate release of peptide product of the neuron into the circulation. Apudomas can also be: , Orthoendocrine: (1) Tumors secreting normal polypeptides of their cells of origin, e.g. lnsulinoma, astri noma, pancreatic glucagonoma, calcitoninoma. (2) Tumors secreting normal amines of their cells of origin , e.g. Phaeoechromocytoma, neuroblastoma and ganglioneuroma (adrenal medulla) and carcinoid tumor. , Paraendocrine Apudoma Syndrome (PES): (1) Tumors of endocrine glands secreting hormones characteristic of other glands (other APUD cells). (2) Tumors of organs, not usually regarded as endocrine in nature, secreting hormones, e.g. oat cell carcinoma of bronchus producing ectopic ACTH. Tumours arising from these cells are grouped as Apudomas. Many of parathyroid tumours, pancreatic tumours are under this group. Their presentations are commonly due to increased secretions of these neuroendocrine hormones. Commonly presentation is like syndromes. lnsulinoma, glucagonoma, gastrinoma, VIPoma are different examples. Tumours are of entopic type, if they secrete hormones normal to the tissue of origin like insulinoma/glucagonoma. They are ectopic type if they produce hormones which are not normal to the tissue of origin like gastrinoma/VIPoma. APUDOMAS are commonly associated with MEN syndrome (commonly type I). Radioimmunoassay, MRI abdomen, CT neck are useful investigations.

Treatment is of individual diagnosed components of the condition.

I Features of Hypoparathyroidism

HYPOPARATHYROIDISM Hypoparathyroidism is defined as a PTH level 6 cm), >100 g weight are more likely to be malignant. Cytologic criteria alone are not diagnostic but should find capsular infiltration and vasc ular invasion. Weiss scoring system is used based on architectu re (1,2,3 scores), the nucleus (1,2,3 scores), the type of invasion (1,2,3 scores); total 9 scores; score >3 suggests carcinoma. Proliferation index, as Ki67 immunomarker or mitotic count, can help to define the diagnosis and prognosis. A mitotic count >20 mitoses/50 HPF is "high grade tumour" with a worst prognosis; whereas "low grade tumour" with ~20 mitoses/50 HPF. Often presents with no symptoms or only vague symptoms. May present with mass effect and compression of adjacent structures. Increased secretion of one or more steroid hormones can occur.

Diagnosis is by endocrine work up, CT/MRI; MR angiography to identify IVG tumour thrombus. The determination of steroidogenic factor 1 (SF-1) expression has proved as the most valid marker. Secondaries occur commonly in lungs. So HRCT of lungs is usually done. Mcfalane staging: Stage I-tumour 5 cm; Stage Ill-tumour with local invasion; Stage IV- distant spread. In stage I and 11, 5 year survival is 25%; in stage Ill and IV, 5-year survival is 5%. It can be 'localized' or 'Metastatic' tum our. Treatment: ,,. Surgery is the mainstay of treatment-en block adrenalectomy; adjacent organs like kidney, spleen may be removed if needed; removal of tumour thrombus from vena cava if present. ,,. Chemotherapy using mitotane-Op-000 (choice) with cisplatin, etoposide and doxorubicin. Mitotane is a derivate of the insecticide dichlorodiphenyltrichloroethane (DOD), with adrenolytic and cytotoxic activity. Mitotane plasma level ~14mg/L is requ ired for clinical efficacy and better survival. Mitotane with etoposide, cisplatindoxorubicine (M-EDP) is the first line of chemotherapy; mitotane-streptozotocin (M-Sz) is the second line of chemotherapy. ,,. Adjuvant radiotherapy may be used to prevent recurrence (little role). ,. Recurrence is treated by debulking and chemotherapy. Metastatic disease is treated by RFA or TACEor debulking with chemotherapy. Target therapy is also tried. ,,. Laparoscopic adrenalectomy is not advisable in adrenocortical carcinoma. •·· Prognostic factors-tumour size>12 cm; high mitotic activity >6 mitoses/high power field; intratumour haemorrhage. Low risk group factors for recurrence are-RO resection, absence of metastases and Ki67 3.

I Treatment Medical: Metapyrone; ketaconazole; aminoglutethamide; mitotane. Surgical For pituitary disease: Trans-sphenoidal microsurgery; Pituitary irradiation-conventional fractionated therapy; Sterotactic radiosurgery; Bilateral adrenalectomy.

For adrenal disease: Unilateral adrenalectomy in adenoma, carcinoma; Bilateral adrenalectomy in macronodular adrenal hyperplasia and primary pigmented nodular adrenal disease.

CONN'S SYNDROME (Jerome Conn, 1954) It is primary hyperaldosteronism with excessive secretion of aldosterone from the adrenal gland associated with suppression of plasma renin activity. Aldosterone secretion is related to angiotensin I and II and plasma rennin with angiotensin converting enzyme. Causes: Aldosterone producing adrenocortical adenoma (Aldo ste ronoma)-65 %; Idiopathic hyperaldosteronism-30%. Features: Hypertension; hypokalaemia; Hypernatremia or normal sodium; metabolic alkalosis; In primary hyperaldosteronism-hypertension of early onset which is difficult to control and is with hypokalaemia. Diagnosis: Hypokalemia; increase in urinary potassium excretion; Elevated plasma aldosterone concentration (PAC); suppressed plasma renin activity (PAA); PAC: PAA >30; it is confirmed by suppression test by oral or IV salt loading; Localising tests-CECT abdomen; Selective adrenal venous sampling-Gold standard to differentiate between unilateral versus bilateral aldosterone hypersecretion. Treatment: Adenoma-unilateral adrenalectomy; Idiopathic hyperaldosteronism-medical treatment with spiranolactone.

VIRILISING SYNDROME OR ADRENOGENITAL SYNDROME Virilising tumours/syndromes. Such tumours are excised. , In female: Virilism, ambiguous external genitalia, clitoral enlargement. , In male: Precocious puberty, premature fusion of epiphysis, short stature. Congenital adrenal hyperplasia (adrenogenital syndrome) is-enzyme 21 hydroxylase deficiency; autosomal recessive. It is treated by-replacement of deficient steroids. Adrenal hyperplasia does not require surgical intervention but the genital manifestations of excess androgen production, particularly in women, may require specialised surgery.

NEUROBLASTOMA Commonest childhood abdominal tumour. It is a tumour of adrenal medulla. An aggressive malignant tumour in childhood usually below the age of 5 years. Incidence is equal in both sexes. A reddish-grey tumour gets invaded early into kidney, pancreas and adjacent tissues. It can also cause distant spread to liver, bones (skull), orbit. Note: It can occur anywhere in sympathetic chain but common in adrenal gland (40%).

I Types 1. Pepper type is right side adrenal neuroblastoma with liver secondaries. Common in infants. 2. Hutchinson's type is left side adrenal neuroblastoma with secondaries in orbit and skull. Common in late childhood. Secondaries in the skull mimics spicular osteogenic sarcomas.

I Pathology Gross-tumour with vascularity, necrosis, haemorrhage and often calcifications. Histologically, it contains uniform round cells with hyperchromatic speckled nucleus with Homer-Wright rosettes with central fibrillar core. PAS stain is negative and NSE stain is positive. Often histochemistry is needed to differentiate from other tumours. Shimada et al. histopathological classification is based on (1) the degree of neuroblast differentiation, (2) the presence or absence of Schwannian stromal development (stroma-rich, stroma-poor), (3) the index of cellular proliferation (known as mitosis-karyorrhexis index [MKI]), (4) nodular pattern, and (5) age. Favorable type will be-well differentiated, stroma rich; MKI index is less than 200/5000; without nodularity; younger age group. Unfavorable type carries poor prognostic factors.

I Clinical Features Child presents as a huge mass per abdomen, in the loin which is non-mobile. Not moving with respiration. Knobby (nodular) surface, crosses the midline. Dancing eye syndrome and opsomyoclonus. Racoon's eye sign is infraorbital ecchymosis due to secondaries in retroorbital region .

Fig. 7.6: Left-sided adrenal neuroblastoma with secondaries in orbit. Note scar of left-sided adrenalectomy.

Hypertension, fever, weight loss, anaemia, flushing (due to catecholamine release) and sweating. Diarrhoea, hypokalaemia due to release of Vasoactive Intestinal Polypeptide (VIP). Other hormones like ACTH are also released.

501 n

:::z:::

>

m Stage I: Localised tum our with gross complete excision. Same side representative nodes are negative on microscopy Stage II A: Localised tumour with incomplete gross excision. Same side representative nodes are negative on microscopy Stage II B: Localised tumour with or without complete gross excision. Same side nonadherent representative nodes are positive but contralateral nodes are (may be enlarged) negative on microscopy Stage Ill: Unresectable unilateral tumour infiltrating across midline/localised tumour with opposite side node spread/bilateral nodal spread/midline tumour with bilateral infiltration Stage JV: Any primary tumour with spread to distant nodes, bone, bone marrow, liver, skin and other organs Stage IVS: Localised primary tumour (stage I, IIA, 11B) with spread to skin, liver or bone marrow-limited to infants younger than one year

--------------~

I Risk Groups

Low risk groups-Stage I disease; Stage II disease with single N myc value; Stage II with favourable Shimada histology. Intermediate risk groups-Stage Ill without N myc amplification; Stage Ill with favourable Shimada's histology. High-risk groups-all patients with N myc amplification; stage IV neuroblastoma.

I Differential Diagnosis Wilm's tumour, which is mobile, with smooth surface, moves with respiration , does not cross the midline.

Figs. 7.7A and B: Typical secondaries in skull and orbit with primary in adrenal gland. Such patients carry poor prognosis. Racoon's eye sign is infraorbital ecchymosis due to secondaries in retro-orbital region. Dancing eye syndrome and opsomyoclonus are other eye features. CT picture of the same patient shows obvious secondaries.

One only gets to the top rung of the ladder by steadily climbing up one at a time.

_,

:::z:, Cl

"ti

.... I» I»

-.... =




a. .... (I)

::,

I»

en

502

... Q)

en :::I

en

c

I Investigation Ultrasound, CT to assess the mass and secondaries in liver. MRI is better than CT. Plain X-ray abdomen shows stippled calcification. Urinary VMA and Homovanillic acid (HVA) estimation in 90% cases. MIBG scan. Bone marrow biopsy may be positive in 60% cages. It is taken from the iliac bones both sides under general anaesthesia PET scan is done if MIBG is not beneficial. Elevated ferritin, LOH and NSE (neuron specific enolase) is observed

I Treatment Adrenalectomy (Complete surgical excision). In inoperable cases, debulking is beneficial. Postsurgical radiotherapy and folic acid supplements are useful. Chemotherapy is useful adjuvant. Drugs used are carboplatin, doxorubicin, cyclophosphamide and etoposide. Rarely spontaneous regression of tumour is known to occur. Low risk groups are treated by surgery. Intermediate risk groups are treated by surgery and multidrug chemotherapy. High risk groups are treated by high dose multidrug chemotherapy and later surgery. Other treatment modalities-retinoids (fenretinide), immunotherapy (G 02), radionuclide targeted therapy, angiogenesis/ tyrosine kinase/aurora kinase inhibitorsare on trial. Bone marrow or stem cell transplantation are newer methods used.

I Prognosis It depends on staging of the neuroblastoma-1 , 2A, 2B, 3, 4, 4S. Low risk has got 3 years survival-90%; intermediate risk-70% and high risk group has got 30%. Factors are-age of the child; stage of the disease; Shimada's histology; Nmyc amplification status (more means high risk); DNA ploidy; neurotrophin receptor Trk A (increased favourable); neutrophin receptor Trk B (increased unfavourable).

PHAEOCHROMOCYTOMA It is a tumour arising from chromaffin cells, commonly from the adrenal medulla but occasionally can arise from extraadrenal chromaffin tissues (Organ of Zuckerkand1. The organ of Zuckerkandl (Emil Zuckerkandl, Professor of Anatomy, Vienna, 1901) comprises a mass of chromaffin cells derived from neural crest located along the aorta beginning above cranial to the superior mesenteric artery or renal arteries and extending below to the level of the aortic bifurcation; highest concentration is seen at the origin of inferior mesenteric artery. It is catecholamine secreting tumours that arise from chromaffin cells of sympathetic origin derived from neural crest representing a potentially curable form of hypertension.

Incidence is 0.005--0.1 % of general population; 0.1-0.2% of adult hypertensive population. It is a soft, brownish grey pink tumour, mainly secretes noradrenaline or other catecholamines. It may also secrete calcitonin, ACTH, VIP (vasoactive intestinal polypeptide), PTH-related polypeptide. Prevalence of phaeochromocytoma is 0.05%. In patients with hypertension it is up to 0.6%. 4% of incidentalomas are phaeochromocytoma. Often it is difficult to differentiate between benign and malignant types. Necrosis, haemorrhage, high Ki-67 positive cells, size of the tumour, increased phaeochromocytoma of adrenal gland scale score (PASS), capsular invasion and vascular invasion, nuclear DNA ploidy and increased neuron specific enolase (NSE) level are possible features of malignant phaeochromocytoma. Currently mutations in at least six distinct genes predispose to phaeochromocytomas-RET, NF1 , VHL, SDHB, SDHC, SOHO. Extra-adrenal pheochromocytoma-10% common; occurs in organ of Zuckerkandl, urinary bladder, paravertebral or para-aortic area, thorax, neck. It secretes norepinephrine rather than epinephrine because they laci< the enzyme PNMT. Commonly benign (90%). Tumour is:

10% malignant 10% extra-adrenal 10% bilateral 10% familial

10% childhood 10% multiple 10% not associated with hypertension 10% calcified

I Clinical Features Clinical manifestations are due to increased secretion of epinephrine and norepinephrine. Commonest presentation is severe headache. Palpitation, dyspnoea, weakness, pallor, blurred vision and other symptoms of sympathetic overactivity. They may present as persistent or paroxysmal hypertension (90%). As an abdominal mass which is nonmobile, smooth , does not move with respiration, crossing the midline, palpation may cause fluctuation in BP. It may precipitate hypertensive encephalopathy, cardiac arrhythmias or cerebral haemorrhage. Panic attacks and sudden death are known to occur. It may be associated with MEN-Ila or MEN-1/b syndromes which includes medullary carcinoma of thyroid and mucosal neuromas. It may be associated with familial multiple neurofibromatosis with cafe au lait spots in the skin (von Recklinghausen's disease). Or with von Hippel-Lindau syndrome (cystic lesion of pancreas, non-functioning islet cell tumour, phaeochromocytoma), RCC, CNS and retinal haemangioblastoma. Familial paraganglioma syndrome may be an association with carotid body and extra-adrenal paraganglioma.

Differential diagnosis: Hyperthyroidism; Anxiety status; Cardiac conditions; Carcinoids (functioning)

I Investigations VMA excretion in urine in 24 hours will be >7 mg/24 hr in pheochromocytoma. U/S abdomen, IVU, CT scan. MRI is preferred to CT as contrast used for CT scan can precipitate paroxysms. Measurement of plasma-free metanephrines is the recommended test of choice for excluding or confirming diagnosis of phaeochromocytoma. Urinary normetadrenaline or other catecholamines estimation. Arteriography. Iodine labelled metaiodo-benzylguanidine (I, MIBG). MIBG is useful to find out extra-adrenal involvement-SPECT scan is very useful. 1131 MIBG scan is safe, noninvasive with 100% sensitivity and 95% specificity. Measurement of plasma free metanephrine and normetanephrine has the highest sensitivity and specificity and appears to be the best initial test for screening patients with pheochromocytoma.

Note: • Laparoscopic adrenalectomy is becoming popular. It is choice of approach for benign functioning or non-functioning adrenal tumours that are less than 6 cm in size. It is contraindicated in malignant tumours and tumour more than 6 cm. • Recent trends are-cortical sparing adrenalectomy; thoracoscopic transdiaphragmatic approach; Robotic-assisted laparoscopic adrenalectomy. • Specimen should be sent for bichromate staining which stains the specimen brown. • Metyrosine is indicated in pheochromocytoma who are awaiting for surgery or when surgery is contraindicated or in refractory to phenoxybenzamine therapy. Metyrosine inhibits tyrosine hydroxylase thus reducing the catecholamine biosynthesis.

C")

:::z:::

>

rn

:::z,

...... C

... = ... "'tJ Q) Q)

'
.

Q.I

V'I

0

::, C

.,,

co a: V'I

Note:

• 5% of breast cancers are missed in total mammograhic screening. • Breast imaging reporting and data system (BIRADS) have got its own categories, assessment (0-6) and recommendations. • Digital mammography is computerised electronic image of the breast with enhanced magnified pictures. • 3 O mammography (breast tomosynthesis) is newer one and may be beneficial in identifying early lesions and in reducing the false positive results. • Digital spot-view mammography allows faster and more accurate stereotactic biopsy. • The condition when lump is clinically not palpable but mammogram shows identifiable carcinoma is ideal for breast conservative surgery. • Mammography should be done one week after menstruation; not just before menstruation. 1

Xeromammography is same as above, but here a photoconductor is used to produce a final image on a Selenium paper rather than on X-ray film. Advantages: Edge enhancement effect, therefore, useful in dense breasts. Disadvantage: Exposure to high radiation dose and selenium plates are needed.

ABERRATION OF NORMAL DEVELOPMENT AND INVOLUTION (ANDI) OF THE BREAST ANDI includes variety of benign breast disorders occurring at different reproductive periods in females-early, matured and involution phase of reproductive age group. It was first coined at Cardiff breast clinic in 1987 by LE Hughes. All conditions under AND I should be carefully clinically examined and often mammography and FNAC/core cut biopsy should be done to rule out malignancy. ANDI includes different aberrations and diseases. It is based on change in normal three phases of physiology of breast-(1) Lobular development; (2) Cyclical hormonal modifications; (3) Involution. In early reproductive age group (15-25 years): Normal lobule formation may cause aberration as fibroadenoma. If it is more than 5 cm it is called as giant fibroadenoma as a diseased status. It is AND of a lobule. Normal stroma may develop juvenile hypertrophy as aberration and multiple fibroadenoma as diseased status. In mature reproductive age group (25-40 years): Normal cyclical hormonal effects on glands and stroma get exaggerated by aberration causing generalised enlargement.

Its diseased status is cyclical mastalgia with nodularity also called as fibrocystadenosis. Involution age group (40-55 years): Lobular involution with microcysts, fibrosis, adenosis, apocrine metaplasia and eventual aberrations as macrocysts and cystic disease of breast. Macrocyst is an aberration of normal involution (ANI). Sclerosing adenosis is also a type of aberration. Ductal involution may cause ductal dilatation and nipple discharge as aberration. Later disease status develops with periductal mastitis, bacterial infection , nonlactational breast abscess and mammary duct fistula. Periductal fibrosis may cause partial nipple retraction. Epithelial changes leads into epithelial hyperplasia and atypia.

FIBROADENOMA It is a benign encapsulated tumour occurring commonly in young females of 15-25 years age group-older definition. Presently, it is considered as hyperplasia of a single lobule of the breast (classified under ANDI). It is the most common benign tumour of the breast below 30 years of age in females. It is aberration in normal development (AND) of a lobule. It shows similar hormonal activities of normal breast like lactation, perimenopausal involution. Incidence is 15% of palpable breast lumps. It is common in blacks and Negroes. •·· It is bilateral in 20% of cases. 20% are multiple. Juvenile fibroadenoma occurs in adolescent girls, rarely (variant). Even though it shows rapid growth with stromal and epithelial hyperplasia, it does not show any alteration in stromal epithelial balance or cellular atypia or periductal cellular concentration. It may clinically mimic phyllodes tumour. But it does not turn into phyllodes tumour or carcinoma. Complex fibroadenoma (Oupon et al} is a condition (variant) having typical fibroadenoma with fibrocystic changes like apocrine metaplasia, cyst formation, sclerosing adenosis. 15% of proven fibroadenomas are complex. It occurs in older age group. Occasionally it may turn into malignancy unlike usual fibroadenomas. Core biopsy is needed to confirm the condition. 30% of fibroadenomas may disappear or reduce in size in 2-4 years. 10-15% will increase in size progressively. It does not occur after menopause unless women are on hormones. Fibroadenoma does not turn into malignancy.

I Types Gross: 1. Soft-common after 30 years; more cellular; often bilateral. 2. Hard-common below 30 years; more fibrous. 3. Giant (> 5 cm in size)-common in Africa.

509

Microscopy: 1. lntracanalicular-large and soft-mainly cellular. Stroma with distorted duct. 2. Pericanalicular-small and hard- mainly fibrous. Stroma with normal duct.

Fig. 8.10: US picture showing fibroadenoma left breast.

I Treatment Excision through a circumareolar incision ( Webster's) or submammary incision (Gaillard Thomas incision) is done under general anaesthesia. Fig. 8.8: Fibroadenoma, on table look-well-capsulated neoplasm. It is part of ANDI.

I Clinical Features It presents as a painless swelling in one of the quadrants, which is smooth, firm, nontender, well-localised and moves freely within the breast tissue (mouse in the breast). Axillary lymph nodes are not enlarged.

Fig. 8.9: Large fibroadenoma left breast in a 14-year-old female.

I Investigations Mammography (well-localised smooth regular shadow). It may show popcorn calcification on mammography. FNAC or core biopsy. Ultrasound (to confirm solid nature) well defined with smooth outline. MRI if necessary.

Figs. 8.11A and B: Multiple fibroadenomas.

Peace is so hard to find because it is under your nose.

510

Fibroadenoma which is small (

I. Conservative line of management is preferred . 1. Reassurance, avoid caffeine, chocolate, salt. 2. Medical (Drugs) - Goal: To stop progression. To relieve pain. To reverse changes. To soften breast tissue. - Indicated when: Fibroadenosis is not increasing in size. No nipple discharge especially blood. No psychological effect. Drugs are:

Intractable pain

t'"'I

::t:

Persistent bloody discharge Bloodgood cyst

Clothe yourselves with compassion, kindness, humility, meekness and patience.

Psychological reason

m

::0 00

...

c::, /1)

QI VI

....

512

Note: • Subcutaneous mastectomy is removal of enti re breast with retaining skin over the breast, areola and nipple. It is done through a submammary Gaillard Thomas incision. Adequate skin flap containing subcutaneous fat is raised which maintains the blood supply of the flap and prevents flap necrosis. After haemostasis drain is placed. Breast implant can be placed in subcutaneous/submuscular plane either immediately or as delayed reconstruction. Indications for subcutaneous mastectomy are-fibrocystadenosis with epitheliosis, sclerosing adenosis, persistent nodules, gynaecomastia and DCIS. • Macrocysts (> 1 cm) is an advanced form of fibrocystic disease; occurs in women in their forties and pericystic fibrosis develops later making cyst harder.

Phylloides tumour is the most commonly occurring nonepithelial neoplasm of the breast, although it represents only about 1% of tumors in the breast. It can also often be fibroepithelial. They can be benign, borderline or malignant. Gross: Large capsulated area with cystic spaces and cut surface shows soft, brownish, cystic areas.

SCLEROSING ADENOSIS It is a benign proliferative condition of terminal duct lobular units with increased number of acini. It occurs in 30-50 years of age group. It may be diffuse or focal. Fig. 8.14: Cystosarcoma phylloides of right breast. Note the dilated Multiple, small, firm, nodules with fibrous tissue and tiny veins. Tumour occupies the entire breast. cysts are common pathology. Recurring pain alike cyclical mastalgia and often breast mass Microscopy: (20% cases) is the presentation. It contains cystic spaces with leaf like projections, hence the Tender breast often with palpable tender firm mass may be name (Phy//oides-Greek-leaf-like). felt with granular surface. Cells show hypercellularity and pleomorphism. It contains proliferative terminal ductules and acini with prolifIt may be a variant of intracanalicular fibroadenoma of breast eration of stroma often with deposition of calcium. Number ( Giant type). of acini per terminal duct is increased more than double the number of normal lobule. There is lobular enlargement, Features fibrous stromal proliferation and distortion. They occur in middle aged or elderly. Complex type is with papilloma and epithelial hyperplasia It is usually unilateral, grows rapidly to attain a large size with often with fibroadenoma. Radial scar is a variant of this. bosselated surface. Condition mimics carcinoma clinically, radiologically and Swelling is smooth, nontender, soft, f/uctuant with necrosis histologically. of skin over the summit due to pressure. It is at present included in ANDI. Sclerosing adenosis can co-exists with both or either invasive and in situ carcinoma breast. Mammography may show distortion, asymmetrical density, amorphous calcifications mimicking carcinoma breast. MRI is better investigation with guided trucut or stereotactic or vacuum assisted biopsy. It is considered as an independent risk factor for developing breast cancer with 1.5-2 higher risk. But it is not a precancerous condition. Treatment: Like ANDI. Regular follow is needed.

I

PHYLLOIDES TUMOUR (CYSTOSARCOMA PHYLLOIDES/SEROCYSTIC DISEASE OF BRODIE) They are not simply giant fibroadenoma. It is commonly nonepithelial; occasionally fibroepithelial. They show a wide spectrum of activity, varying from almost a benign condition (85%) to a locally aggressive and sometimes metastatic tumour (15%). Depending on mitotic index and degree of pleomorphism they are graded as low grade to high grade tumours. When malignant (sarcoma) spreads to lungs or bone.

Fig. 8.15: Phylloides tumour of left breast (Cystosarcoma phylloides of left breast).

Skin over the breast is stretched, red and with dilated veins over it. Tumour is warmer, not fixed to skin or deeper muscles or chest wall. Nipple retraction is absent. Lymph nodes are usually not involved. These are the differentiating features from carcinoma.

Tumour grows rapidly; undergoes necrosis at various places; causes cystic areas. Recurrence is common. Differential diagnoses: Juvenile/giant fibroadenoma, angiosarcoma, breast abscess and carcinoma breast.

Investigations: , Ultrasound; Mammography. , FNAC, core biopsy ,. Chest X-ray CT chest in malignancy to see secondaries.

I Treatment Wide excision with 1 cm margin or subcutaneous mastectomy to avoid recurrence. If malignant (sarcoma) total mastectomy is indicated with adjuvant chemotherapy; but carries poor prognosis. Nole:

• Enucleation should not be done. • Benign type is 70% common; 3 mitoses/10 HPF; modest cellularity with uniform stroma; low Ki 67 index; negative !MP3; 10%recurrence. • Borderline type is 15%; 4-9 mitoses/10 HPF; modest cellularity with heterogenous stroma: intermediate Ki 67 index; negative !MP3: 20% recurrence. • Malignant type is 15%: 10 or more mitoses/1OHPF; cellularitry, stromal overgrowth and nuclear pleomorphism is marked; infiltrative margin with heterogenous elements; high Ki 67 index; positive !MP3; 50% recurrence; metastases can occur. • AMOS criteria for predicting recurrence: Atypia; Mitoses; OvergroW1h of stroma; surgical margin. • Core biopsy is ideal. • Surgery is the mainstay of treatment Fig. 8.16: Operated specimen of cystosarcoma phylloides.

MASTALGIA ("Pain in the Breast") 45% of women report breast pain, 21 % severe. An entity that is ubiquitous; has an unknown aetiology, and a poorly understood. Mastitis, carcinoma presenting with only mastalgia (8%). Patients who are on HRT, caffeine, tobacco, large pendulous breasts, etc.

I Types

Cyclical-65%; Noncyclical-30%; Chest wall pain- 5%.

Cyclical Pain related to menstrual cycles. Usually seen in ANDI like fibrocystadenosis. Present in women of menstruating age group. Pain is more during menstruation. It is bilateral, diffuse with "heavy feeling".

Figs. 8.17A and B: Recurrent cystosarcoma phylloides.

513

Treatment: Diclofenac gel-is very useful as local application. Evening primrose oil 325 mg BO. Gamolenic acid 120 mg BD. Danazol (100-200 mg BD)-antigonadotrophin agent. Bromocriptine (2.5 mg BD)-prolactin inhibitor. Tamoxifen (20 mg daily). Gn RH analogue 3.6 mg injection depot-monthly. Testosterone undecanoate 40 mg BD. Vitamin 86 , 812 . Analgesics.

Gratitude, joy, love, passion, excitement, enthusiasm, hope, goal, satisfaction, humility, contentment - are different needs of happy life but difficult to acquire.

...

CD l'D CJ

"'r+

514

Noncyc/ical Other causes of breast pain are periductal mastitis, malignancy, cervical root pain , musculoskeletal pain, previous surgery, Tietze's syndrome, idiopathic, Mondor's syndrome. It is unilateral, chronic, burning or dragging in nature, occurs both in pre- and postmenopausal age group. 5% of breast cancers present as pain during first presentation.

cc"' a: Ill

Treatment: Cause has to be identified. Malignancy has to be ruled out. Avoid coffee and stress. Proper support to breasts.

Tietze's syndrome: Costochondritis of second costal cartilage, commonly seen in females, mimics mastalgia.

TRAUMATIC FAT NECROSIS It may be due to either direct or indirect trauma (trauma may not be noticed many times).

I Pathogenesis Capillary ooze causes triglyceride in the fat to dissociate into fatty acids. It combines with calcium from the blood resu lting in saponification which causes inflammatory reaction and later presents as a nonprogressive swelling in the breast.

I Features

Figs. 8.18A and B: Traumatic fat necrosis.

Painless swelling in the breast which is smooth, hard, nontender and adherent to breast tissue. It is nonprogressive, nonregressive. Investigations: FNAC shows chalky fluid with fat globules. Mammography to rule out malignancy. Differential diagnosis: Carcinoma breast. Treatment: Excision.

GALACTOCELE Seen in lactating women. Occurs during cessation of lactation. Often up to 1Omonths after lactation. It is due to the blockage of lactiferous duct resulting in enormous dilatation of lactiferous sinus. It contains milk and epithelial debris within. It is a retention cyst in subareolar region attaining large size.

Fig. 8.19: Galactocele.

I Features

515

Lump in the lower quadrant of the breast which is usually unilateral, large, soft, f/uctuant, with smooth surface. It is usually nontender. It may get precipitated, inspissated or get calcified. When it is calcified it mimics carcinoma breast. If it gets infected it will form an abscess. When it is cystic other cystic swelling in the breast should be ruled out. Investigations: Ultrasound; FNAC-Aspiration shows thick, creamy, greenish/brown fluid. Treatment: Aspiration of the content; Excision (submammary incision); Abscess when formed should be drained under general anaesthesia under cover of antibiotics.

MASTITIS

I Types

Fig. 8.21 : Subareolar abscess.

1. Subareolar. 2. lntramammary. 3. Retromammary (submammary).

Subareolar Mastitis It is the infection under the areola due to cracks in the nipple or areola. It results from an infected gland of Montgomery or a furuncle of the areola. There is blockage of the ducts of these glands. Often it is associated with duct ectasia-causing formation of abscess, sinus and fistula. It is common in nonlactating women. Risk factors are - diabetes, smoking, nipple cracks. Clinical features: Red, inflamed, edematous areola with a tender swelling underneath; Nipple retraction may develop. Differential diagnosis, Paget's disease of the nipple. Treatment: Under cover of antibiotics pus is drained by making a subareolar incision.

lntramammary Mastitis (Breast Abscess) a. Lactational abscess of the breast. Commonly seen in lactating women. Usually up to 6 months of lactation period. It occurs in 3% of breastfeeding mothers.

B Cracked nipple; Retracted nipple Improper cleaning of the nipple Inadequate milk sucking by baby or milk expression causing stasis Infection from the mouth of the baby Haematoma getting infected

Mode of infection: Bacteria (Staph. aureus- most common) enters the breast during sucking through the cracked nipple. Occasionally, it can oe from haematogenous spread. Staphylococcus epidermidis, streptococci, anaerobic bacteria also can cause acute mastitis. Gram-negative and other bacterial infections can supervene later. Staphylococcus aureus causes clotting of milk in the blocked duct and multiply. Duct initially gets blocked by epithelial debris or by retracted nipple. Initially it begins in one quadrant but later involves entire breast. Features:

Fig. 8.20: Diagram showing subareolar, intramammary and retromammary abscess.

Continuous throbbing pain in the breast and high grade fever. Diffuse redness, tenderness, warmness and brawny induration in the breast. Purulent discharge from the nipple. Entire breast may get involved eventually. Occasionally tender fluctuant swelling (10%) may be felt; ulceration and discharge can occur at a later period. Tender axillary lymph nodes may be palpable.

Saying 'no' to something is actually much more powerful than saying 'yes'. - Tom Hanks

516

Often takes very long time to heal after surgery causing distress to patient and surgeon as well.

• Mastitis not resolving with antibiotics in 48 hours • Persistent fever and progression of mastitis • Brawny induration Do not wait for abscess to form (fluctuation to develop)

Complications: Antibioma formation. Sinus formation, skin necrosis, milk fistula formation. Recurrent infection, bacteraemia, septicaemia. Fig. 8.22: Typical breast abscess with features of acute

inflammation. It is difficult to differentiate initial stage of mastitis (stage of cellulitis) from stage of breast abscess formation. When it is treated with antibiotics without incision and drainage eventually it may get organised to form a nontender, hard breast lump with sterile pus inside-stage of antibioma formation. Differential diagnosis: Inflammatory carcinoma of breast. Investigations: Blood total and differential count; US breast to identify an abscess, its location, size, loculi. US guided aspiration can be done; pus should be sent for culture and sensitivity. Treatment: ,. Antibiotics- cephalosporins, flucloxacillin and amoxicillin.

Note:

• Proper antibiotics are needed. • Support to breast is done to relieve pain. • Milk from affected breast should be removed either manually or using breast pump. • Occasionally milk suppression may be needed if milk fistula or recurrent abscess develops. It is achieved by giving bromocriptine (2.5 mg BO for 2 weeks) or stilbestrol.

Drain Incision

Counterincision

Figs. 8.24A and B: (A) Incision and counter-incision for breast abscess; (B) Drain should be placed after incision and drainage in such abscesses. Often gauze drain can be used.

b. Nonlactational abscess of the breast. ,. It commonly occurs in duct ectasia and periareolar infections. Common organisms are bacteroides, anaerobic streptococci, enterococci and Gram-negative organisms. It is commonly recurrentwithtender swelling under the areola. ,. It is common in diabetes mellitus and immunosuppression. Treatment: Antibiotics; Repeated aspirations; Drainage and later cone excision of the duct is done.

Retromammary Mastitis Fig. 8.23: Breast abscess in a male patient. Breast abscess eventhough

is uncommon in males, it can occur in puberty and middle age.

,. Repeated US guided aspirations (using 18 gauge needle with saline lavage) can be tried which avoids surgery and scar-ideal and standard now. ,. Drainage under general anaesthesia, a counter incision may be needed. It is not advisable to wait till the formation of abscess.

It is commonly due to tuberculosis of the intercostal lymph nodes or ribs beneath or suppuration of the intercostal lymph nodes. Empyema necessitans or infected hematoma in the chest wall cal also is the cause. Presentations: Pain and swelling in the chest wall deep to breast which is non mobile. Investigations: Hematocrit, ESR, peripheral smear; Chest X-ray; US of breast and chest wall. But CT scan chest is ideal.

Treatment: Cause has to be treated. Drainage through submammary/retromammary incision is done. Note-.

• Mastitis of infancy ( Witch's mi/K) is due to maternal hormone in infant blood. It usually subsides but may cause suppuration. • Mastitis of infancy affects both sexes equally. • Mastitis of puberty is common in boys, is invariably unilateral with tender, swollen and inflamed breast. • Mastitis of mumps is usually unilateral and can occur in both sexes. • Bacterial mastitis is seen in adult women, commonly lactating and is due to staphylococci infection. • Subareolar mastitis is due to infection of gland of Montgomery or due to areolar furuncle.

ANTI BIO MA If intramammary mastitis is not drained but only treated by antibiotics, pus localises and becomes sterile (flaques) with a thick fibrous tissue cover and it is called as antibioma.

I Features Previous history of mastitis treated with antibiotics. Swelling which is painless, smooth, nontender, hard, fixed to breast tissue without involving the pectorals and chest wall. Differential diagnosis: Carcinoma breast (Scirrhous carcinoma breast). Investigations. FNAC; mammography; US breast. Treatment Excision (Submammary incision). Later antibiotics are given. It should be sent for histology.

DUCT ECTASIA It is dilatation of lactiferous ducts due to muscular relaxation (myoepithelial relaxation) of duct wall with periductal mastitis.

Fig. 8.25: Duct ectasia-ductography.

It is also called 'plasma cell mastitis' as periductal inflammation contains plasma cells. Commonly many ducts are involved.

Hormonally induced myoepithelial relaxation with poor ductal absorption of secretions and desquamated cells causing obstruction are the probable other causes and features.

I Features Greenish discharge or creamy/paste like from the nipple. lndu rated mass under the areola which is often tender. Retraction of nipple which occurs at later stage of the disease. Slit like retraction of nipple due to fibrosis occurs. Eventually it forms an abscess and fistula. Often they are bilateral and multifocal. More common in smokers-in relation to arterial pathology. Co mmon in multiple pregnancies, perimenopausa/ age, hyperprolactin status. May present as mastalgia. Axillary nodes may be palpable as nonspecific. Secondary bacterial infection (anaerobic) is common. Differential diagnosis: Carcinoma breast. Investigations: ,. Discharge study, FNAC. ,. Mammography.

Treatment

, It is important to stop smoking. ,. Cone excision of involved major ducts (Adair-Hadfield operation). Antibiotics. ,. Me/hem Novel modified breast ductal system excision.

MONDOR'S DISEASE (Henri Mondor-Paris, 1939) Mondor's disease is spontaneous thrombophlebitis of the superficial veins of the breast and anterior chest wall. Cause is not known . History suggestive of injury or infection is not observed. Presents as a thrombosed subcutaneous cord (2-3 mm sized) which is attached to the skin. On raising the arm above, a narrow, shallow subcutaneous groove appears alongside the cord like thrombosed vein. The thoracoepigastric vein, the lateral thoracic vein, and the superior epigastric vein- are involved. The upper, inner portions of the breast are never involved. Trauma, infection, surgery may be the cause but clearly not proved and so controversial. Rarely penile Mondor's disease and Mondor's in the arm are observed. It is often a self-limiting disease without any recurrence, complication or deformity. It often mimics th e lymphatic permeation of carci noma breast. Restriction of arm move ments, brassiere support and anti -inflam matory drugs may be needed. Occasionally refractory cases need surgical excision of involved segment of vein.

Cultivation of mind should be the ultimate aim of human existence. - BR Ambedkar

517

n

J:

)> "'C

--i m ::x:, 00

518

...>Cl ...::::, QJ

Ill

0

re,

::::, C: re,

:E

cc a: Ill

Ill

Contd.. .

TUBERCULOSIS OF THE BREAST It is relatively rare. Even though it is rare, often seen in India (4% of benign breast diseases). It may be due to high resistance offered by mammary gland tissue to the survival and multiplication of the tubercle bacillus, a resistance similar to that offered by spleen and skeletal muscle. Usually associated with active pulmonary tuberculosis. Infection reaches through blood or retrograde lymphatic spread from lymph nodes of axilla. Common in lactation. Nipple and areola are not commonly involved. Lump-irregular ill-defined; Peaud' orange; discharge, sinus, matted axillary nodes often with sin us are the features. Presents as a swelling in the breast with cold abscess, sinuses and a typical bluish appearance of surrounding skin w(th matted lymph nodes in the ipsilateral axilla. Differential diagnosis: Carcinoma breast. Investigations: FNAC; Frozen section biopsy is usefu l to differentiate from carcinoma; Excision biopsy. Treatment: Antituberculous drugs-I NH, rifampicin, ethambutol, pyrazinamide; Drainage of cold abscess. Note:

• Mastectomy is not done.

Clinically/sonologically/mammographically mimics carcinoma or tuberculosis. Tissue trucut biopsy shows granuloma but negative for tuberculosis, AFB and carcinoma. • Diagnosed by method of exclusion; higher prolactin; niigative tests for tuberculosis. • Treatment: Doxycycline 100 mg for 6 months; Two cycles of 10 days each of amikacin; Deflazacort 12 mg initially then 6 mg OD for 6 months; Radical excision with closed or open drain; Anti-inflammatory and antibiotics; Prolactin lowering drugs-bromocryptine, cabregoline; Colchicine, hydrocortisone, methotrexate, azathioprine in recurrenVrefractory cases; Role of antituberculous drugs is questioned?

BREAST CYSTS They are cavities lined by epithelium in the breast containing fluid. It arises from destruction and dilatation of breast lobule and terminal ductules. It is due to nonintegrated stromal and epithelial involution. Cyst may be microscopic or macroscopic. It contains straw coloured or green or opaque fluid. Incidence is very high (1 in 14 females). It is common after the age of 35 years up to menopause. It is uncommon after menopause. Hormone replacement can cause cyst formation in old women. Cyst size varies with menstruation due to influence of ovarian hormones.

Fig. 8.26: Tuberculosis of breast showing undermined lesion.

B • It is damage to the ductal epithelium with luminal secretions escaping into the lobules causing intralobular granulomatous mastitis. Exact cause is not known-trauma, prolonged breast feeding, autoimmune response, hyperprolactinaemia (cytokine like effect) are possible causes-mimics tuberculosis of the breast often. • Negative history of tuberculosis with negative for PPD, ADA and culture; higher levels of prolactin are special features. Histology-granulomas with microabscess formation without caseating necrosis. It is seen in multipara; prolonged breastfeeding. • lndurated discharging sinuses with tenderness; positive lymph nodes in axilla-often seen. __,

____________

1

Contd...

Fig. 8.27: Retention cyst in areola.

Cysts can be multiple (50%). Often bilateral. Cysts can be recurrent (50%). Risk of breast cancer in breast cyst is very less (0.1 %). But incidental associated carcinoma may be present in 3% of breast cysts. Clinically-smooth, soft, fluctuant often transilluminating well-localised swelling may be felt. Differential diagnoses are: Bloodgood cyst, haematoma, cystic necrosis in a carcinoma, Brodie's disease, galactocele, lymph cyst, hydatid cyst.

Investigations: US of breast; FNAC. Mammography to rule out associated carcinoma.

I Treatment Aspiration for two times. Surgical excision is done if cyst recurs after two aspirations or if there is bloody discharge or residual mass if felt after aspiration.

• After aspiration one should examine for the residual lump. FNAC of this residual lump should be done. • Cyst when recurs (30%) reaspiration should be done. • Patient should be examined for refilling of the cyst in 6 weeks.

)>

Bloodgood cyst

• Breast abscess • Haematoma in breast Galactocele

Lymph cyst

Serocystic disease of Brodie Cystic necrosis in carcinoma breast

GALACTORRHOEA It is secretion of milk not related to pregnancy or lactation. It is always bilateral. Primary galactorrhoea is due to: Stress and other factors. It is physiological in puberty or menopause. Reassurance is the treatment. Secondary galactorrhoea is due to:

Dopamine receptor blocking agents like haloperidol, methyldopa, chlorpromazine, metoclopramide or by hyperprolactinaemia due to pitu itary tumours. It enhances the prolactin activity. Hypothyroidism. Drugs like oral contraceptives, atenolol, clonidine, ranitidine. Ectopic prolactin secreting tumours usually from lungs (bronchogenic carcinoma). Chronic renal failure.

I Management Estimation of serum prolactin, T3, T4, TSH, CT/MRI head. Bromocriptine therapy. Treatment of cause. Causative drug shou ld be stopped and different drugs should be used for the needed condition. Note:

Note: • Fluctuation in the cyst is checked by examiner standing behind the patient. • Mammogram should be done if patient is more than 35 years. • 21 Gauge needle is used for aspiration. • Fluid is sent for analysis (cytology); evidence says that if fluid is clear no need to send for cytology but in practice it is commonly sent and safer to send also. • All fluid which is altered should be sent for cytology.

n

:r:

Hydatid cyst

Figs . 8.28A and B: Ultrasound look of breast cysts-simple and complex type.

519

Witch's milk is secretion of milk in both male and female infants due to maternal hormonal effects in foetus. It lasts up to three weeks after chi ld birth.

GYNAECOMASTIA (Greek-Women Breast) It is hypertrophy of male breast more than usual due to increase in ductal (epithelial) and connective tissue (stromal)

elements often attaining features of female breast. It can be unilateral or bilateral. Bilateral can be symmetrical or asymmetrical.

Knowledge should be gained; more importantly gained knowledge should be utilized effectively and efficiently in a right way for a right cause. -William J Moore

m

:::i:,

00

520

It is nonphysiological stimulatory oestrogen excess or inhibitory androgen deficiency. There is nonphysiological hypertrophy of male breast attaining the size of female breast with increase in connective and ductal tissues.

,, Inhibition of testosterone synthesis-cimetidine, phenytoin, spironolactone.

ldiopathic- 25% Teratoma testis-3% Ectopic hormonal production in bronchial carcinoma Anarchism, after castration Adrenal and pituitary disease Leprosy, because of bilateral testicular atrophy Drugs (25%): Stilbestrol, digitalis, cimetidine, spironolactone, INH, phenothiazides Liver diseases and liver failure- 10% Klinefelter's syndrome (XXY Trisomy), Kallman syndrome Primary or secondary hypogonadism Hyperthyroidism Renal diseases, dialysis induced (1%) Fig. 8.29: Gynaecomastia-right side breast (diffuse type). Compare

with opposite side to note the difference in size.

It can be diffuse- involving all quadrants or small well localised hard subareolar nodule.

TYPES Neonatal gynaecomastia is due to action of placental estrogen Pubertal (25%) in young boys is due to excess estrogen ;level in relation to testosterone. It is usually unilateral. Here breast tissue will be more than 2 cm in diameter in nonobese young male • Senescent in old is due to fall circulating testosterone causing relative hyperestrinism Prepubertal-may also be seen in girl child It can be-. Puffy nipples Pure glandular Adolescent hereditary Adult-most common Androgenic Simon's classification of gynaecomastia: Group 1-Minor visible type without skin redundancy Group 2 A-Moderate without skin redundancy Group 2 8- Moderate with minor skin redundancy Group 3-Gross pendulous breast

I Features Fig. 8.30: Right-sided gynaecomastia (well-localised-puffy nipple).

I Aetiology Oestrogen excess-increased estradiol secretion due to testicular tumour (Leydig cell) or nontesticular tumours from adrenal cortex, lung, liver; hyperthyroidism (increases conversion of androgen to estrogen) , liver diseases, oestrogen therapy for prostate cancer. Androgen deficiency-agin g, Klinefelter 's/ Kallmann syndromes, eunuch, ACTH deficiency. Secondary testicular failure-cryptorchidism, orchitis, trauma, CRF, etc. Drugs (25%): ,, Increases estrogen activity (digitalis, anabolic steroid)/ estrogen synthesis (reserpine, theophylline, frusemide).

Initially there will be florid proliferative stage of ductal epithelium with oedematous stroma without acini (1 year); later there will be quiescent stage with ductal dilatation and stromal fibrosis. Always one should examine genitalia and liver. It is physically embarrassing; psychologically devastating. Oifferential diagnosis: , Pseudogynaecomastia- adipose tissue deposition as a part of obesity; needs liposuction. , Pectoral muscle hypertrophy. ,, Lipoma, dermoid, haematoma. Presentations: ,,. Diffuse enlargement of breast occupying all quadrants. ,,. Often well-localised, small, firm or hard nodule under the areola which is often painful and tender. Investigations: Relevant to the cause, e.g. liver function tests, DNA study, hormone assay, FNAC, USG breast.

521

Treatment: , Well-localised type which is symptomatic or patient is psychologically worried is treated by surgical excision using circumareolar incision. Large diffuse gynaecomastia needs Gaillard Thomas submammary incision for excision. ,. Reduction mammop/asty; nipple reduction surgeries. ,. Drugs-tamoxifen, clomiphene , androgens (dihydrotestosterone), danazol, testolactone as aromatase inhibitor-but less useful. Drugs are given only for 6 months. Gynaecomastia more than 2 years duration is less likely to show benefit from drugs. ,. Breast irradiation to prevent gynaecomastia in prostatic cancer patients who are on oestrogen therapy. ,. Causative drugs should be stopped. , Testicular tumours, hyperthyroidism should be treated if they are the causes. ,. Testosterone can be given if hypogonadism is the cause. , Endoscopic assisted subcutaneous mastectomy is done and under trial mainly for cosmetic purpose.

("\

:::c

)>

m

:x, 00

Figs. 8.31A and B: Papilloma breast which is large.

Usually it is small intraductal.

Nolfr.

• Rarely gynaecomastia may turn into malignancy. Gynaecomastia is a differential diagnosis for male breast cancer. • 80% of Klinefelter's syndrome shows gynaecomastia; it has got very high-risk (20 times) of developing carcinoma.

DUCT PAPILLOMA It is the most common cause of bloody discharge from nipple It is usually single, from a single lactiferous duct It blocks the duct causing ductal dilatation They are epithelium lined true polyps of breast lactiferous ducts. Usually, it is 50% cases (5 fold). 5% of male breast cancers are DCIS. Minor ductal epithelial proliferation is the typical histology. Invasive ductal cancer forms in the same breast and same quadrant of OCIS un li ke LCIS. DCIS is an anatomical precursor of invasive DC. Presence of > 25% of DCIS component is present in the main invasive tumour or if DCIS is present elsewhere in the surrounding breast tissue, it is called as extensive in situ component. Nipple discharge and often small swelling are main presentations. US assisted FNAC and mammography are the needed investigations. Risk of lymph node spread in DCIS is less than 4%. So axillary dissection is not necessary. Sentinel Lymph Node Biopsyand proceed is the preferred method.

T

CLASSIFICATION OF PRIMARY BREAST CANCER

Van Nuy-s prognostic index for DCIS Score (VNPI score) 2

3

16-40 mm 1-9 mm

>40 mm

"C

--i

m

:::0 00

532

There is 5 fold increase in 2nd breast cancer on opposite side in a female, who has had breast cancer on ipsilateral side. LCIS, multifocal breast cancer, family history are other risk factors. Choudary Millis criteria that help to differentiate primary contralateral breast cancer from metastatic-in situ change, different histological pattern, different differentiation shown in opposite breast, clinically or by evaluation ipsilateral tumour has not shown any features of spread . It is investigated like any breast cancer, staged accordingly and treated. If tumour on the opposite side is metastatic then it will show separate histology and differentiation with mammography showing less infiltrative, diffuse, without microcalcifications but with oedema. If it is metastatic, it is stage IV disease.

TNM STAGING OF CARCINOMA BREAST (AJCC 8TH EDITION , 2018)

I Primary Tumour (T) Tx Primary tumour cannot be assessed (already treated elsewhere without documentation). TO - No evidence of primary. Tis - Carcinoma in situ. Tis (DCIS) Ductal carcinoma in situ. Tis (Paget's) - Paget's disease of the nipple not associated with invasive carcinoma or with DCIS T1 - Tumour less than 2 cm (20 mm) T1 mi - Microinvasion 1 mm or less in greatest dimension T1a-1 - 5mm T1b- 5-10 mm T1c - 10 -20mm T2 - 20 - 50 mm in greatest dimension T3 - >50 mm in greatest dimension T4 - Any size with direct extension to the chest wall or skin or both. T4a - Tu mour of any size extending into the chest wall, not including on ly pectoralis muscle invasion/adhesion (chest wall means ribs, intercostal muscles and serratus anterior but not pectoral muscles). T4b - Ulceration or ipsilateral satellite nodules and/or oedema including peaud'orange of the skin which do not meet the criteria for inflammatory carcinoma. T4c - T4a and T4b. T4d - Inflammatory carcinoma.

I Regional Lymph Nodes (N) Nx - Regional nodes cannot be assessed NO - No regional nodes involved N1 - Metastases to mobile ipsilateral level 1 and 2 axillary nodes N2N2a - Metastases in ipsilateral level 1 and 2 axillary nodes which are fixed to one another (matted) or other structures

N2b - Metastases only in clinically detected ipsilateral internal mammary nodes and in the absence of clinically evident level 1 and 2 axillary nodes N3N3a - Metastases to ipsilateral infraclavicu lar lymph nodes (level 111axillary) with or without level 1 and 2 axillary lymph node involvement. N3b - Metastases to ipsilateral internal mammary lymph nodes with clinically evident level 1 and 2 axillary lymph nodes involvement. N3c - Metastases to ipsilateral supraclavicular lymph nodes with or without axillary or internal mammary lymph node involvement. Nole: •

Clinically detected is defined as detected by imaging studies (excluding lymphoscintigraphy) or by clinical examination and having characteristic highly suspicious for malignancy or a presumed pathologic micrometastases based on the fine needle aspiration biopsy with cytologic examination. Confirmation of clinically detected metastatic disease by fine needle aspiration without excision biopsy is designated with f suffix. • Lobular carcinoma in situ (LCIS) is a benign entity and is removed from TNM staging in the AJCC Cancer Staging Manual, 8th Edition.

I Distant Metastases (M) MO- No clinical or radiological evidence of distant metastases cMO(i+ )-No clinical or radiological evidence of distant spread metastases but deposits of molecularly or microscopically detected tumour cells in circulating blood, bone marrow or other non-regional nodal tissue that are no larger than 0.2 mm in a patient without symptoms or signs of metastases. M1-Distant detectable metastases as determined by classic clinical and radiographic means and/or histologically proven larger than 0.2 mm. Note: • • •

Mx is removed in 7th edition of TNM staging. c: stage given by clinical examination of a patient. p: stage given by pathologic examination of a surgical specimen. • y: stage assessed after chemotherapy and/or radiation therapy; in other words, the individual had neoadjuvant therapy. • r: stage tor a recurrent tumor in an individual that had some period of time free from the disease.

I Stage Groups Tis NO MO T1/T1 mi NO MO TO N1 mi MO; T1 /T1 mi N1mi MO TON1 MO; 1 N1 MO; T2 NO MO T2 N1 MO; T3 NO MO TO N2 MO; T1 N2 MO; T2 N2 MO; T3 N1 MO; T3 N2 MO Stage 1118: T4 NO MO; T4 N1 MO; T4 N2 MO Stage IIIC: Any T N3 MO Stage IV: Any T any N M1

Stage O: Stage IA: Stage 1B: Stage IIA: Stage 118: Stage IIIA:

Note: • T1 includes T1 mi • TO and T1 with nodal micrometastases are excluded from stage IIA and is classified as stage 18. • If patient presents with M1 prior to neoadjuvant systemic therapy, the stage is considered as stage IV and will remain stage IV regardless of response to neoadjuvant therapy. • Isolated tumour cluster is cluster of tumour cells wherein each cluster is less than 0.2 mm in size. • Micrometastasis is deposit tumour 2 mm. • Circulating tumour cells (CTC) are assessed using 7.5 ml of blood through cell tracks analyzer II with a DNAstain DAPI. >1 cell/7.5 ml for primary tumour and >5 cells/7.5 ml for metastatic disease is considered as unfavorable. • Disseminated tumour cells (DTCs) in bone marrow are assessed by liquid biopsy from bone marrow. One cell or more is unfavourable.

I Oncotype Ox: 21 genes are assayed (16 cancer related and 5 normal genes). It is assessed by RT-PCR using tissue paraffin section. Score < 11 yields very low risk for recurrence. It used to predict the risk of recurrence of early-stage, hormone-receptor-positive breast cancer and they will benefit from chemotherapy after surgery. The Oncotype DX DCIS test is used to predict the risk of recurrence of DCIS and/or the risk of a new invasive cancer developing in the same breast; how a woman diagnosed with DCISwill benefit from radiation after surgery. Oncotype OX demonstrated both Prognostic significance (the capability of predicting distant recurrence) and Predictive significance (the capability of the test to assess the potential benefit of additional adjuvant chemotherapy). PAM50 gene signature (ProSigna): it is a genomic test; it creates score from 0-100 for prediction of distant recurrence. 50 genes are assessed. It is categorized in node negative patients as low (up to 40), intermediate (41-60) and high (61-100). In node positive patients-it is low (0-40) and high (41-100). It is performed on RNA isolated from FFPE breast tumor tissue. It measures the expression levels of 50 genes

I

B • Basal-like (15-25%): ER-ve, PR-ve and Her-2-ve; also called triple negative breast cancer (TNBC). Most BRCA1 breast cancers are basal-like TNBC. They are high grade, aggressive with poor prognosis. Common in black and young individuals; high incidence of lung and brain secondaries. Luminal A (50%): ER+ Her-2 - ve and low grade. They are slow growing and occur in postmenopausal women. They respond well hormone therapy. Luminal B (15-20%): ER+ PR+ve Her-2 +ve (triple positive) but often high grade. They respond to chemotherapy. • Her2 rich (10%): ER -ve. Poorly differentiated, aggressive with higher incidence of brain secondaries. Treated with chemotherapy and trastuzumab. l aptinib is used if brain metastases are present as trastuzumab will not cross the blood-brain barrier. Normal breast like (5%): ER +ve, Her-2 -ve. • Luminal ER-IAR+: recently identified and rogen responsive subtype which may respond to antihormonal treatment with bicalutamide. ERBB-2/Her-2+: has amplified Her-VNeu. • Claudin-low: a more recently described class; often triplenegative, but distinct in that there is low expression of cell-cell junction proteins including E-cadherin and frequently there is infiltration with lymphocytes.

1B

533

Contd...

cane~

:I:

l>

"'0

-I

m

:D 00

I

'--

Note: Manchester staging and Columbia classification are obsolete (not used now). Readers can refer 4th edition if needed. Manchester staging is-(1) Mobile tumour in the breast; no deeper fixation ; skin if involved lesser the size of the tumour; lymph node spread is absent. (2) Same as stage 1 with mobile discrete axillary nodes. (3) Fixed to pecto ralis major or skin involvement more the tumour size or fixed/adherent node. (4) Tumour fixed to chest wall/involvement same or opposite supraclavicular nodes, opposite axillary nodes, opposite breast, cancer en cuirasse, distant blood spread.

B Fibroadenosis Traumatic fat necrosis Tuberculosis of breast Bloodgood cyst Filariasis breast

Mastitis Antibioma Galactocele Mondor's disease Cystosarcoma phyllodes

I Investigations in Carcinoma Breast Mammography.

Genomic tests analyze a sample of a certain genes are. The activity level of these genes affects the behavior of the cancer, including how likely it is to grow and spread. Breast cancer index: It is used to predict the risk of node-negative, hormone-receptor-positive breast cancer patients coming back 5 to 1Oyears after diagnosis. Endopredict is a gene expression numerical scores from Oto 15. Up to 5 is low risk; >5 is high risk. MammaPrint: 70 genes are assessed to predict breast cancer recurrence. . ...:::,C'I QJ

V'I

0

11'

:::,

C 11'

:E

"' a:i a: V'I

Soft tissue shadow is irregular Spiculations

j

Bilateral mammography is done to identify multicentricity, to have guideline for assessing eventual chemotherapy or RT in LABC. Stereotactic mammography guided biopsy is very useful. LCIS may be missed in mammography. Fifty per cent of breast cancers can be seen on mammography before they are palpable. It is noninvasive with less radiation exposure. Five per cent false-positive rate in mammography; hence, biopsy is a must. Mammogram is used during follow-up period after conservative breast surgery and of opposite side. Ideally specimen mammography is a must after conservative breast surgery to assess the completion; and after core biopsy to confirm the sample tissue.

Fig. 8.59: Ultrasound picture of right breast showing breast mass.

Note:

Lead time and length time bias in breast screening: The preconceived notion, not definitively proven, that early discovery of tumours usually allows for curative treatment and is always superior to later discovery, creates a lead time bias favouring early detection. Length time bias suggests that individual tumours reach the point of no return based upon distinct and separate sojourn times, and it is assumed that detecting a tumour by screening is superior to discovering a tumour by physical examination. It is possible that survival outcome and death will be near same in such tumours that death time may be same. So screening controversy still continues in carcinoma breast. Preclinical detectable phase will give length bias. Lead time varies from women's age and natural history of individual women's breast cancer; this leads into lead time bias.

Ultrasound of breast: ,,

To find out whether the lesion is solid or cystic.

B To look for whether the lesion is solid or cystic, margin of the lesion, internal echoes, retro-tumour acoustic shadowing, compressibility, dimensions. Irregular margin, irregular internal echoes, irregular posterior shadowing, noncompressibility, ratio between anteroposterior to width (lateraVhorizontal} dimensions more than 1are the features of carcinoma. Doppler will show high frequency signals with continuous flow. It is hypoechoic with more vertical taller growth. Benign lesions are smooth, rounded with well-defined margins with weak internal echoes and compressibility. It can be elliptical, hyperechoic/hypoechoic smooth lesion. Cyst is anechoic, oval/round, well circumscribed lesion. Disadvantage is lesions less than 1 cm may not be identified. FNAC can be done under US guidance. It is cheaper, easily available and there is no risk of radiation. It is preferred method of screening in young females where mammography is not done and in pregnancy and early lactation. US of axilla also can be done to assess axilla and to do guided FNAC of node.

Fig. 8.60: Ultrasound breast is basic noninvasive simple investigation to assess breast lump.

FNAC (Martin and Ellis 1930): It is very useful in diagnosing the carcinoma breast. US guided FNAC is also used. But negative results are difficult to interpret because it may be due to sampling errors and so requires further diagnostic methods. FNAC of opposite breast, lymph nodes, opposite axillary lymph nodes are also often required. It is done with 23 gauge needle using FNAC aspiration special syringe (aspiration gun). With the lump held firmly, the needle is passed into the lump and with negative pressure continuous aspiration is done until adequate material comes through the needle (suction pressure of 40 cm of H20 is created into the syringe). Needle with syringe is removed without negative pressure. Material is collected on a slide; a smear is made using 100% alcohol. Cytology is studied after staining it under microscopy. Minimum six aspirations are done. Giemsa, Papanicolaou, hematoxylin and eosin stains are used. Repeat FNAC can be done for further 2 times. But it gives only cytological diagnosis. Receptor study cannot be done.

taken using vacuum. A clip may be placed under guidance at the site of the lesion as marker. Mammography of core biopsy specimen is done to confirm the sample tissue. If stereotactic biopsy is inconclusive then a wire localised surgical excision should be done. Core needle biopsy is the method of choice.

It is difficult to differentiate between in situ and invasive breast cancer by FNAC.

Advantages FNAC is least painful, can be done on OP basis, reliable and cheaper. Malignant deposits will not occur along FNAC track (only contraindication for FNAC is testicular tumour).

,

Fig. 8.62: Trucut biopsy from carcinoma breast. It is the ideal

investigation for confirmation. Reliability of FNAC and mammography

Sensitivity (true positivity) Specificity (without false-positive) False-negative False-positive

Mammography

90% 90% 10% 10%

B Co: No epithelial cells C3 : Atypical cells C1 : Scanty epithelial cells, benign C4 : Suspicious cells C2 : Benign cells_____ C5 : Malignant cells

I

Corecutnrucut biopsy: , It is done under local anaesthesia. It gives clear histological evidence and also confirms DCIS (FNAC cannot confirm DCIS). This allows proper neoadjuvanVprimary chemotherapy, receptor status of the tumour. Wide bore needle biopsy with vacuum is also used. , 14- 18 gauge spring loaded needle is used. Mu ltiple punctures are needed. US guided biopsy is also done. , Large core biopsy is done using 6- 14 gauge needle; sing le puncture is sufficient; large single sample of tissue is obtained. , Vacuum-assisted core biopsy is also done. , Stereotactic mammographic/MRI/US guided core biopsy are also used in small/impalpable lesions. Stereotactic core biopsy is done in prone position with compressed breast. Under local anaesthesia, 3 mm incision is made and 11 gauge core needle is passed under digital mammographic gu idance. Multiple core biopsies are

-0 --4

m

FNNAC is Fine NeedleNon-Aspirating Cytology.

FNAC 90- 98% 98- 100% 2-10% Near 1- 5%

':r.J>"' :::ti 00

Note:

Fig. 8.61 : FNACof breast lump.

535

B It is donewhen lump is not clearly palpable. US guided core needle biopsy. • Stereotactic mammographic core needle biopsy. Mammography guided wirelocalisation using needle sheath over the tumour andthroughan incision under local anaesthesia hook is reached and biopsy is done. It is used if core needle biopsy fails in localising nonpalpable lesion. MRI guided core needle biopsy.

Frozen section biopsy If FNAC fails even after two trials or in cases of negative FNAC, then on table frozen section biopsy is done for diagnosis. Frozen section biopsy also has got drawbacks. It has got 20% false-negative results. So its use at present is under debate eventhough it is practiced in few oncocenters. It is not ideal method. Excision biopsy: , It is done only when FNAC is inconclusive and a facility for frozen section is not available. Incision should be planned in such a way that it will be included in eventual mastectomy. Edge biopsy: , Done only when t here is ulceration and fungatio n. Diathermy should be avoided in incision biopsy as it may distort the histology of tumour and study of hormone receptor status may not be possible. Oestrogen receptor study: , They are oestrogen sensitive receptors, which are cytosolic, glycoprotein present in the breast and tumour tissue. It is an important indicator of prognosis of carcinoma breast.

If you don't know whereyou're going, you may miss it when you get there.

536

>,_ QI

C'I ,_

::,

V,

0

Ill

::, C

Ill

co"'

ex:

V,

,

Tissue for receptor study is sent at low temperature in ice radioisotope labelled albumin (one mCi on previous flasks. It is assessed by quantitative analysis (Frozen -70°). day)/sulphur colloid (6 hours before) near the tumour , If value is more than 10 units (f/mols) per n gram of tissue (peritumour area) or into subdermal plexus around the it is called as ER +ve status. ER positivity is common in nipple. Marker will pass th rough the sentinel node which postmenopausal women (60%) compared to premenocan be visually detected as blue staining or with a hand pausal women (30%). If value is less than 10 units per held gamma camera; and is biopsied with a small incigram of tissue it is called as ER-ve status. Currently IHC sion made directly over it. Frozen section biopsy or touch is used to measure ER/PR expression. Staining of 1% or imprint cytology is done for presence of malignant cells. more is considered as positive. If there is no involvement of sentinel node by tumour In ER +ve status then further axillary dissection is not required as skip - Prognosis is good. lesions (skipping sentinel node) occur only in less than Hormone therapy including tamoxifen is more beneficial. 3% cases. Response to treatment is better. , Detection rate of sentinel node for blue dye and radioisoIn ER -ve status tope is 90% and 98%, respectively. Subdermal/subareolar Prognosis is poor. injection of radioisotope has got better sentinel node Hormone therapy is not very beneficial (but used) as localisation than peritumour injection. But better imaging compared to ER +ve patients. is obtained by peritumour injection and so peritumour - Response to treatment is not good. injection is usually practiced. Radioisotope tracer injecProgesterone receptor (PR status) study or Her-2/Neu tion done in the early morning of the day of surgery into receptor status or cErb 82 (growth factor receptor study) peritumour area and perioperative injection of patent blue are other studies done at present to plan the therapy and dye in subareolar region-as a combined method is often assess the prognosis. used in many centres. After injection of patent blue, breast Her-2/ Neu receptor (Human epidermal growth factor is massaged continuously to enhance the uptake. Incision receptor-2 Neu oncogene [Neuropilin 21) is a tyrosine kinase is made after 5-7 minutes between pectoralis major and receptor and is associated with ER negative, high grade, tumours. It carries poor prognosis. Over expression of Her-2/ latissimus dorsi to identify blue stained lymphatics which Neu shows good response to adriamycin. are traced to 2-3 blue lymph nodes. Hand-held radioHer-2/Neu is also called as c Erb 82 (cell surface Erythroprobe is used to identify the sentinel node which is later blastic oncogene B2) . excised. Often 2-3 nodes are removed. Study of discharge from the nipple. , Paraffin section histology is better than frozen section , Nipple discharge is usually unilateral in carcinoma to identify positive sentinel lymph node. If report comes breast. Ductal lavage may be useful in some patients. negative immunohistochemistry test is done to confirm Microcatheter of 1 cm length is introduced gently into that lymph node is negative for tumour. Sentinel lymph the ductal opening. 10 ml saline is infused through the node biopsy should be done before wide local excision catheter. Fluid is withdrawn into the syringe and cytoof the primary tumour. logical analysis is done. , Wide local excision of the primary tumour is done after Sentinel lymph node biopsy (SLNB): SlNB in the same sitting. , The first axillary node draining the breast (by direct , SlNB is less invasive than axillary dissection. It is ideal in drainage) is designated as the sentinel lymph node {SLN). early invasive carcinoma. Positive SlNB is again classified SlN is first node involved by tumour cells and presence as macrometastasis (>2 mm) or micrometastasis ( "'0

-4

m

::l:> 00

Fi g. 8.63: Sentinel lymph node biopsy (SLNB) of breast. Note: Spread by skipping the sentinel node is less than 3%.

Axillary sampling-. ,

It is often done with an adequate axillary incision. 10-15 nodes are removed for sampling. It is not commonly practiced now (Minimum 10 nodes should be removedlevel I nodes). MRI of breast: , To differentiate scar from recurrence. , To image breasts of women with implants. ,, To evaluate the management of axilla and recurrent disease. It is useful in screening females with high-risk group and young women and in pregnancy. , T1 and T2 weighed images are taken . , Irregular mass with spiculations, changes in skin and nipple, lymphoedema are the findings in carcinoma breast. , Lesion indeterminate by US/mammography is assessed by MRI. , Ionising radiation is not there with MRI. ., It is the method choice of imaging breasts in pregnancy . ., It is better in dense breasts. ., Patient lies in prone position with breasts placed over breast coils; both precontrast and postcontrast (gadolinium) MRI are taken. ,, Disadvantages are-it is costly; nonavailability; not sensitive for premalignant lesions. It cannot be done in patients who are having incompatible metal prosthesis in the body. It is not accurate if done within 9 months of the radiotherapy for carcinoma breast. Tumour markers: CA 15/3 (normal value

-0

m

:::0 00

Fig. 8.68: Gray incision for mastectomy in carcinoma breast which extends to opposite side.

(

__) .-

Fig. 8.69: Greenough incision for mastectomy. lodsioo \

,

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

_J

/................ .

\ ......

\.........,...

___J ...

,\.,·-

Incision

Tumour

Incision

_..../ Incision

//.............1

m

·::.:.:\··.·r l ·············.., \

..

··.•.

[

Figs. 8.66A and B: (A) Horizontal incision for total mastectomy; (8) Oblique incision extending into the anterior axillary fold for radical mastectomy.

··..,

.

..

...... ...... ·:::,

r Incision

•• ••

·•..

• ...........'L

Tumour

m 1- 2 cm \ '' '' •:.:•., -1.:.:· ·······.,\

l

Figs. 8.70A and B: (A} Kocher's incision for mastectomy; (8) Orr incision for mastectomy. Tumour

t) . . ., ' ,.,j 8 1nbision

_,

...... Incision

Figs. 8.67A and B: Stewart incision fo r mastectomy.

OJ .... (D 0.1 ,.,, ,-+

Note: • Mastectomy specimen should be carefully inspected and sent to pathology department. Specimen is sent in formalin fo r histology and grading, margin clearance. Lymph nodes should beassessed as number, involvement, capsular breach and which levels. • It is sent in saline in low temperaturefor ER/PR/Her-2/Neu status study (histochemistry). Ki 67 index should also be assessed. • Specimen mammography is often done.

__) '"="' \

539

C)

Fig. 8.71: Rodman incision for mastectomy.

The place to improve this world is first in one's own heart, head and hands.

C)

540

Figs. 8.72A to E: Different steps of Paley's mastectomy with incision, flap raise, dissection in breast and axilla and specimen after surgery.

3. Conservative breast surgeries: ,. Wide local excision: It is removal of unicentric tumour with 1 cm clearance margin. Incision is made directly over the tumour. Skin flaps should not be raised. Normal breast tissue of 1 cm clearance with excision of tumour is done. Pectoral fascia is usually not opened in wide local excision unlike in total mastectomy. The specimen is marked after placing in orientation grid and mammography of the specimen is done followed by frozen section biopsy to look for clearance. At least 1 mm clearance is needed for adequacy. Margins where clearance is less than 1 mm need reexcision at that particular site. If margins show no clearance then patient requires probably total mastectomy. So prior consent for mastectomy should be taken. Drain is not placed; deeper cavity is usually not closed/obliterated as small seroma gets absorbed without any problem. Skin is closed cosmetically. It is often called as lumpectomy or partial mastectomy; but better term is wide local excision. It is ideal breast conservative surgery. Along with this, axillary dissection through separate incision and RT to breast and chest wall area is given. It is done in low grade tumour with 2.5 cm diameter; oedema of -

..

(I)

Cl

lymphangiosarcoma (Stewart-Treve's syndrome) of upper limb lfxiernal radiotherapy is given over the breast area, axilla (in selected can develop in patients who have developed lymphoedema after I p~tients like if axillary dissection is not done or more than 4 posItIve mastectomy with axillary clearance. Usually, it occurs 3-5 years axillary nodes), internal mammary and supraclavicular area after development of lymphoedema. Such patient may require fore• Total dosage 5000 cGY units quarter amputation. It has got poor prognosis. It presents as multiple I ~ 0-cGY units daily 5 days a week for 6 wee_ks_ _ _ __ subcutaneous nodules.

r

:::,

0

T

ro

To chest wall

C:

• T3 tumour >5 cm • 4 or more • RT is a must after nodes positive conservation of breast • Residual • Extranodal • Local with or without disease-LABC spread to axilla • Positive margin/ • Axillary status • Tangential fields 50 close surgical not known/not Gy/25 fractions/ 5 weeks margin of 60 years, node negative, invasive ductal, T1 tumours.

Principles: It is used in ER/PR positive patients in all age groups (earlier it is used in perimenopausal age groups). It is relatively safe, easy to administer. It gives prophylaxis against carcinoma of opposite breast. It is useful in metastatic breast carcinoma. Hormone therapy (tamoxifen) is useful in breast cancer in elderly (positive ER) after wide local excision or occasionally as tamoxifen alone. It is now not used in ER negative patients. Menopausal status; nodal status; chemotherapy used are not factors to defer the use of hormone therapy. Hormone therapy reduces the recurrence rate and so probably improves the life span and quality of life. Includes: Oestrogen receptor antagonists- tamoxifen. Ovarian ablation by surgery (Bilateral oophorectomy) or by radiation. LHRH agonists (Medical oophorectomy). Oral aromatase inhibitors for postmenopausal women . Adrenalectomy or pituitary ablation. Progesterone receptor antagonist. Androgens-lnj testosterone propionate 100 mg IM three times a week. Amin oglutethimide- blocks the synthesis of steroids by inhibiting conversion of cholestero l to pregnenolonemedical adrenalectomy. Progestogens, e.g. medroxyprogesterone acetate.

Tamoxilen It is an antioestrogen. It blocks cytosolic oestrogen receptors. Dose is 1Omg BID or 20 mg OD for 5 years. Half life of tamoxifen is 7 days; it takes 4 weeks to show its benefits. It reduces the cholesterol and also cardiovascular morbidity. Adverse effects: Tamoxifen flare-flushing, tachycardia, sweating , genital itching, vaginal atrophy and dryness (premenopausal), vaginal discharge (postmenopausal), fluid retention, weight gain. Occasionally it causes bone pain which should be differentiated from pain due to bone metastasis. It is due to loss of bone density in premenopausal women. It increases the incidence of endometrial cancer. DVT (3%), pulmonary embolism, CVA, TIA, cataract, fractures. Side effects and endometrial cancer are less in selective drugs like raloxifene.

Advantages: It reduces the recurrence rate by 25%. It improves the prognosis. It is used presently in all age group, ER +ve patients. Preservation of bone density in postmenopausal women. But it causes bone loss in premenopausal women.

T

Drugs used in hormone therapy

Drug

Mechanism of action

Tamoxifen

Antioestrogen 20 mg

Medroxyprogesterone Aminoglutethimide

Progestogen

Arimidex Letrozole

Aromatase inhibitor Aromatase inhibitors Aromatase inhibitor

Dose

Adverse sffscts

Nausea, weight gain, hot flushes, vaginal bleeding, bone pain, amenorrhoea 400 mg Nausea, flushing, vaginal bleeding 250 mg Rash, dizziness, QID lethargy, Cushing facies 1 mg Lethargy, GI upset 2.5 mg

Vaginal dryness, hot flushes, vaginal bleeding, cardiovascular problem LHRH agonist 3.6 mg Amenorrhoea, nausea. Zoladex (Goserelin) monthly It is expensive Diethylstilbestrol Oestrogen 15 mg Fluid retention, vomiting, thrombosis, daily hypercalcaemia Fluoxymestrone Androgen 30 mg Masculinization, daily nausea It is more beneficial in ER +ve patient; perimenopausal patient with node +ve; postmenopausal patient. Presently Tamoxiten and Oophorectomy are the commonly used methods. OD

Cheap, easily available, less toxic effects, very effective. It is equally effective in carcinoma male breast. It is presently also under trial for certain benign diseases of breast (ANDI, cyclical mastalgia).

Selective oestrogen antagonists Do not cause endometrial hyperplasia or endometrial carcinoma. Drugs include droloxifen, toremifen, raloxifene. TAMOXIFEN USED IN:

Carcinoma breast Benign diseases of the breast like fibroadenosis Infertility in males; Desmoid tumour Risk reduction therapy in high-risk femalesin premenopausal age.

Letrozole It is a nonsteroidal competitive inhibitor of the enzyme 'aromatase'. This enzyme converts adrenal androgens to oestrogen (aromatization). So it is an aromatase inhibitor. It is expensive but more effective than tamoxifen. It is also used in recurrent disease. Letrozole is used as an adjuvant endocrine therapy in postmenopausal women with hormone sensitive breast cancer (In premenopausal women this will cause rise in gonadotrophins and ovarian aromatase is not well suppressed). It can also be used in metastatic and recurrent cases. It slows down and stops the growth of oestrogen sensitive breast tumours. It reduces oestrogen level by 98%. Its half life is 45 hours. It decreases the bone density. ,, Dosage of letrozole is 2.5 mg once daily. ,, It is given for 5 years or for 2 years following 3 years of tamoxifen. ,, Side effects of letrozole are vaginal dryness, night sweats, hot flushes, vaginal bleeding, cardiovascular problems and osteoporosis. Note:

• Aromatase inhibitors can be non steroidal (Letrozole, anastrozole) or steroidal (exemestane). Aminoglutethemide (1st generation), formestane, fadrazole(2nd generation), anastrozole, letrozole, exemestane (3rd generation) are different aromatase inhibitors. Trastuzumab (Herceptin) It is a monoclonal antibody that blocks HER-2/Neu receptors thereby preventing growth of cancer cells. It is a new drug. It is presently marketed as herceptin. It is c-ErbB2 (growth factor receptor) inhibitor. It is a newer biological agent. Her-2/Neu receptor is tyrosine kinase receptor. It has very little effect on HER-2/Neu negative cancers. It is useful only in HER-2/Neu positive cancers. It is given as intravenous infusion. Studies have shown substantiate improvement in disease free and overall survival rate.

Don't sit back and take what comes; go after what you want.

545

t""I

:c )> "'ti

-I

m

::x, 00

548

Pertuzumab is another similar agent. But both do not cross the blood-brain barrier. Margetuximab is the newer lgG monoclonal antibody against Her-2/Neu receptors. Dose is 4 mg/kg as loading; 2 mg/kg as maintenance for 1 year. It has got cardiac side effects. NOTE Tamoxlfen interferes with oestrogen receptors • Letrozole interferes with oestrogen production + Transtuzumab (Herceptin) interferes with Her-2/Neu receptors • Bevacizumab-vascular growth factor receptor inhibitor-newer

agent • Lapatinib-is dual Her-2/Neu and EGF receptor inhibitor; it

crosses the blood-brain barrier.

D. Chemotherapy in Carcinoma Breast Adjuvant chemotherapy refers to administration of cytotoxic drugs to women after breast cancer surgery in the hope of eliminating clinically undetectable distant spread. Reduced recurrence rate and improved survival rate is observed in all women with invasive breast cancer. Chemotherapy is usually not indicated in DCIS. Neoadjuvant chemotherapy refers to adm inistration of cytotoxic drugs in a large operable tumour to reduce the locoregional tumour burden to make it better amenable for surgical resection, usually after 3 doses. It downstages the disease; may ach ieve breast conservative surgery in selected patients (only); early systemic control is achieved. Palliative chemotherapy is used in advanced and metastatic carcinoma breast.

Lapalinib

Indications:

Lapatinib ditosylate is dual tyrosine kinase inhibitor which interrupts with Her-2/Neu and EGFR by inhibiting autophosphorylation. It is oral drug used in breast carcinoma with over expression of Her-2/Neu (Her-2/Neu positive). It is used in postmenopausal metastatic tumour with over expression of the Her-2/Neu. It is often combined with capacitabine. It is used in diseases which has progressed after using anthracyclines, taxanes and trastuzumab. ,:. Dose- lapatinib at a dose of 1,250 mg daily and capecitabine at a dose of 2,000 mg per square meter of body-surface area daily on days 1 through 14 of a 21-day cycle). ,:. Lapatinib has got better cardiac safety compared to trastuzumab. Lapatinib crosses the blood-brain barrier.

All node positive patients. Primary tumour more than 1 cm in size. Presence of poor prognostic signs of any tumour-vascular and lymphatic invasion; high nuclear and histologic grade; Her-2/Neu overexpression; negative hormone receptor status. In advanced carcinoma breast as a palliative procedure. In postoperative period after simple mastectomy in stage Ill carcinoma breast with fixed axillary nodes. In inflammatory carcinoma of breast. ,:. In stage IV carcinoma breast with secondaries in bone , lungs, liver. In preme nopausal age group with poorly differentiated tumours.

Is

iiiiiiii@ill11i1!JMiii·i~§:M§i-

ll111 M11

/n premenopausal women + Tamoxifen-antioestrogen

Ovarian ablation by surgery/by Goserelin/by radiation

Progestogens-medroxyprogesterone 400 mg I•+ Androgensfluoxymestrone In postmenopausa/ women

• • • ., •

Tamoxifen Aromatase inhibitor like letrozole 2.5 mg OD Progestogens Androgens Medical adrenalectomy using aminoglutethimide (Mitotane)-as major source of oestrogen after menopause is adrenal gland. Cortisone supplement is also needed to prevent feedback rise of ACTH which may block the effect of aminoglutethimide.

B • Bilateral oophorectomy (Beatson-1896) in premenopausal age • Bilateral adrenalectomy; Pituitary ablation Note:

Present changing concept-is letrozole and tamoxifen are continued even after 5 years (probably life long) to reduce the chance of late recurrence.

Different regimes CMF regime (Non-anthracycline regime): Cyclophosphamide, methotrexate and 5 fluorouracil (CMF) is the good old regime used in olden days (still practiced in many places). It is cheaper and easier to administer. Side effects are-alopecia, bone marrow suppression, megaloblastic anaemia, cystitis, GIT disturbances and nephritis. These drugs are used now along with anthracyclines or taxanes as different combination regimes. Anthracyclines (1960 ): First anth racycline extracted is daunorubicin from streptomyces; later doxorubicin (adriamycin, red colour) is extracted from another strain of streptomyces. Adriamycin is the most commonly used anthracycline. It is used often with other chemotherapeutic agents and also with trastuzumab. It is cardiotoxic; cardiotoxicity can develop as late as more than one year post-chemotherapy period. Epirubicin is another anthracycline used. ldarubicin (in AM L), valurubicin (in bladder cancer) and mitoxantrone are other anthracyclines. Anthracyclines are first line drugs with different combinations. Taxanes (1990): Paclitaxel and docetaxel are the two taxanes commonly used. It blocks the G2/M phase of cell cycle. Paclitaxel is derived from Pacific yew trees (Taxus brevifolia); docetaxel, is a semisynthetic derivative of paxlitaxel (from Europen Yew tree-Taxus baccata). Taxanes are used

CMF

TAC/AC

Others

Cyclophosphamide

CEF Cyclophosphamide

Cyclophosphamide

For Her-2/Neu positive -

Adriamycin (Doxorubicin)

Epirubicin

Methotrexate

Taxane (Docetaxel/ Paclitaxel) Adriamycin

5-Fluorouracil

5-Fluorouracil

5-Fluorouracil

Cyclophosphamide

Docetaxel + tratuzumab with CEF

CAF

commonly for locally advanced (LABC) and metastatic breast cancer (MSC). Nab-paclitaxel (Abraxane) is newer derivative which is more effective. Nanoparticle albumin-bound (nab)-paclitaxel, is paclitaxel linked to albumin nanoparticles which makes it soluble and is an example of application of nanotechnology to cancer treatment. Taxanes have no cross-resistance with anthracyclines and so can be used sequentially or concurrently with anthracyclines. Taxanes are 2nd line drugs. Gemcitabine (synthetic pyrimidine nucleoside prodruganticancer drug): It is used often in selected cases of MSC and LABC for better results. It may be 3rd line drug . Note: • Neoadjuvant (primary) chemotherapy is useful in largeoperable primary tumour to make it amenable for conservative breast surgeries. Postoperative radiotherapy and chemotherapy is a must in this situation. • High dose chemotherapy with stem cell rescue is not found to bebeneficial by trials in patients with heavy large nodal spread. • Hormone therapy is given after completion of chemotherapy for additive effect. Concurrent therapy of both (together) is not used at present. It is sequential-chemotherapy then hormone therapy. Chemotherapy gives quicker and better response initially and so it is used early and later only hormone therapy is continued. • Adriamycin shows better response with Her-2/Neu overexpression patients. • Paclitaxel may be combined with trastuzumab in Her-2/Neu (Human epidermal growth receptor 2) over expression patients. • Chemotherapy and radiotherapy can be given concurrently or as sandwich therapy. • Presently one day dose of all drugs of the regimen used as a standard at 3 weekly cycles for 6 cycles for stage 11 I-CAFregime. For metastatic paclitaxel single day dose cycles or etoposide and cisplatin 3 days cycle-6 cycles. • CAF regime is better than CMF. ACregime (Adriamycin and cyclophosphamide) with taxanes or AC regime 4 cycles followed by taxanes 4 cycles are also used. • Gompertzian model is used in chemotherapy principle. Benjamin Gompertz (18th century mathematician) told "Law of mortality" asgrowth rate of populations are exponential at early stages of development and slower at later stages. Later Norton and Simon hypothesis proved that-tumours follow Gompertzian growth functions; smaller tumours grow faster than larger ones; rate of cell-killing by many drugs is proportional to tumour growth rates; tumours given less time to regrow between treatments are more likely to be destroyed. So shortening of the time interval of the doses and high density dosing improved survival. • Endocrine-responsive breast cancer: Luminal A: Node negative-no benefit from adjuvant CT; Node positive benefit from CT. •

•

Luminal 8: High Ki67 index, aggressive, risk of relapse; CT and endocrine (hormone) therapy are indicated. Choice of CT is based on risk of relapse. Sequential anthracylines and taxanes are used. Regular intake of aspirin may prevent recurrence of breast cancer or

breast cancer- on trial.

AC followed by paclitaxel + trastuzumab

547

n

::::c )>

"'ti

Complications of chemotherapy-bone marrow suppression, alopecia, GI side effects, cardiac toxicity. Monitoring wit h haematocrit, blood urea, serum creatinine, LFT are needed. Cytoprotect ive agents like GMCF (granulocyte macrophage colony stimulating factor), GCSF (granulocyte colony stimulating facto r) are given to reduce severity and duration of neutropenia. It is given in 24 hours as 30 million units.

Fig. 8.80: Patient with carcinoma breast on chemotherapy having alopecia.

MANAGEMENT OF EARLY CARCINOMA BREAST Early breast cancer (EBC) is the stage I, II A and only T2N 1MO of 118. Tumour N1

• Tumour margin is not free of tumour after breast • Poorly differentiated tumour; Multicentric tumour • Tumour/breast size ratio is more (central tumour) • Earlier breast irradiation • Tumour beneath the nipple • Extensive intraductal carcinoma

Auchincloss operation or Patey's (MRM) or total mastectomy whenever BCS is not possible (see Table)-lndications for Total Mastectomy in Early Breast Cancer are-when tumour is more than 4 cm; multicentric tumour; poorly differentiated tumour- high grade; tumour margin is not cleared after breast conservative surgery. Sentinel node biopsy when required should be an option. Skin sparing mastectomy with axillary dissection can also be done. Oncoplastic surgery with breast reconstruction of different types should be done with individual case basis.

Adjuvant Therapy Postoperative radiotherapy to breast after BCS or chest wall after total mastectomy or MRM and in high-risk patients. It reduces the chances of local recurrence. Chemotherapy: CMF, GAF regime commonly used. Taxanes are also used. It is used in all with node positive patients, young women, lymphovascular invasion, high proliferative index, close surgical margins, ER/PR negative. Endocrine manipulation: (1) Tamoxifen (receptor antagonist)-20 mg/day for 5 years. (2) Aromatase inhibitorsblocks oestrogen production. Letrozole 2.5 mg OD. (3) LHRH agonists-Goserelin 3.6 mg/28 day's cycle for 2 year. (4) Ovarian/pituitary ablation.

Follow up Regular follow-up is done with clinical examination, ultrasound breast and axilla/mammography, radioisotope bone scan, liver function tests, chest X-ray, ultrasound abdomen, CT chest and abdomen if needed, and CEA tumour marker.

ADVANCED CARCINOMA BREAST Refers to: Locally inoperable (adherent to chest wall) tumour. Inflammatory carcinoma of breast. Fixed axi llary lymph nodes, or supraclavicu lar lymph nodes, or opposite axillary nodes. Bilateral carcinoma breast. Metastatic carcinoma of breast, i.e. spread to bones, liver, lungs, brain.

I Locally Advanced Carcinoma of Breast (LACB) It means locally advanced tumour with muscle/chest wall involvement, extensive skin involvement or fixed axillary nodes. It will be T3, T4a, T4b, T4c or T4d or N2 LACB is stage II Band Ill disease. lnfraclavicu lar (level Ill) and supraclavicu lar lymph node (n3) involvement is LACS. It is investigated by FNAC of tumour/core needle biopsy/ incision biopsy/mammography of opposite breast, chest CT, CT abdomen or whole body bone scan. Biopsy is needed to assess receptor status (ER/PR/Her-2/Neu). Bilateral mammography is done to assess tumour size and multicentricity which is needed to check the chemotherapy response at a later period. FNAC of axillary node is required. If bone scan is positive it becomes metastatic carcinoma of breast not LACB. Only when 60% of bone is dimeneralised in metastatic bone disease, plain X-ray bone will detect the lesion. Even though X-ray may be normal in stage Ill disease, in 30% of these patients bone scan will be positive making the disease stage IV metastatic.

Treatment of LACB Neoadjuvant chemotherapy (NACT), mastectomy either total or modified radical mastectomy (MAM-usually after 3 cycles of initial chemotherapy), further chemotherapy, radiotherapy [local breast field and axilla (concurrent)], hormone therapy (as sequential-trastuzumab for Her-2/Neu positive/ tamoxifen/letrozole) is becoming more popular. Evaluation with bone scan and HRCT chest is a must to rule out metastatic type. Survival rate will be increased to 50% by these modalities. Targeted therapy like trastuzumab in Her-2/Neu positive patient with neoadjuvant chemotherapy can be safely administered prior to mastectomy. This anterior (neoadjuvant) chemotherapy helps in downstaging the diseases by cytoreduction; controls the micrometastases first which will be present mostly in LACB; it allows the chemosensitivity; also provides systemic chemotherapy.

Remaining chemotherapy should be started within 6 weeks of surgery. If BCS is done RT should be given first then chemotherapy. After completion of chemotherapy hormone therapy is started. LACB with entirely fixed fungating masses, chemotherapy, palliative total mastectomy (often with skin graft to cover the defect), hormone therapy using tamoxifen or letrozole or trastuzumab. Axillary dissection is not necessary in this type. Only chemotherapy and radiotherapy (to breast and axilla) is also advocated in few; later with hormone therapy. Treatment for LACB is targeted at present as curative; but it is achieved only in 50% of patients. So in 50% of patients therapy becomes palliative. During treatment period for LACB , disease may become metastatic (systemic) and so evaluation at regular intervals with CT chest, bone scan, MRI spine, liver function tests, CT abdomen is needed. Breast conservative surgery (BCS) has got limited role as it will effect on the survival rate (even though many proponents practice, this may not be ideal). 5-year su rvival is 50% with proper therapy and 10-year survival is 25% or less.

m

PRESENT STRATEGY OF TREATMENT FOR LACB

•

•

•

•

Initial neoadjuvant (anterior) chemotherapy is given to downstage and achieve cytoreduction, to target possible micrometastases first, to assess chemosensitivity. FEC, CMF, GAF regimes are used. Response to chemotherapy is assessed by complete responders without palpable tumour; partial responders with >50% decrease in tumour size; nonresponders with 25% increase in size. Patients with non responders and progressive disease are treated by RT to breast, chest wall, axilla and supraclavicular region; taxanes; hormone therapy; surgery if operable. Responders are later treated with total mastectomy/MRM, occasionally BCS. After surgery remaining 3 or 4 cycles of chemotherapy are completed. Later hormone therapy should be given for 5 years (tamoxifen 20 mg OD). ER +ve (> 1Ofmol/mg) patients show 75% positivity. Stage IIIA patients are classified as operable and inoperable.

Inflammatory Carcinoma of Breast Inflammatory carcinoma of breast is a clinicopathological entity characterized by diffuse erythema and oedema/peau d'orange involving one third or more of the skin over the breast with rapid evolution of less than 6 months. Inflammatory carcinoma is T4d LACB (Stage 11IB). It is a clinical diagnosis. It is also called as mastitis carcinomatosis or lactating carcinoma of breast. It is 2% common. It is observed in younger age group usually in pregnancy or lactating period. There will be extensive skin involvement with pain. It often mimics mastitis of lactation. FNAC or incision biopsy concludes diagnosis. It can be ductal or lobular. It is initially treated by chemotherapy or

radiotherapy and later if tumour reduces in size total mastectomy with axillary clearance can be done. But most often it is inoperable. After surgery, chemotherapy and tamoxifen is given. 5-year survival for inflammatory carcinoma of breast is 25-30%.

I Metastatic Carcinoma of Breast It is blood spread into different places like bone , lungs and pleura, liver, soft tissues, brain and adrenals. It is evaluated by FNAC/i ncision biopsy, chest CT, LFT, US abdomen, CT abdomen, whole body bone scanning, CT brain , tissue study for ER/PR/Her-2/Neu receptor status. It is stage IV disease. Bone is the most common site of metastasis. Spread to vertebra is through posterior intercostal vein and Batson's venous plexus (valveless). Vertebrae, ribs, upper end of humerus and femur are common bones involved. Lungs and pleural spread causes 'cannon ball' secondaries, effusion, consolidation, chest wall secondaries. Liver secondaries may develop either by haematogenous or through lymphatics across diaphragm from lower inner quad rant of breast. Brain secondaries present with headache, vomiting, convulsions, localising features. Soft tissue secondaries has got better prognosis, visceral secondaries has got worst prognosis. Response to treatment decreases with number of organs involved with secondaries. Receptor negative secondaries are more aggressive. Median survival time for metastatic breast cancer is 24 months. TREATMENT CONCEPT IN METASTATIC CARCINOMA OF BREAST • To improve quality of life. To relieve pain of secondaries like bone, lungs. To relieve neurological problems like convulsions, space occupying cranial problems; Other symptomatic relief.

B

Treatment strategy in metastatic carcinoma of breast Chemotherapy-CMF, GAF, Taxanes in combination. Chemotherapy in metastatic breast cancer is useful (indicated/ commonly used) in rapidly spreading visceral and skin secondaries, lymphangitis or when predicted disease free survival is less than 2 years; if response is negative for first line hormone therapy; in receptor negative status. :.- High dose of chemotherapy using cyclophosphamide, cisplatin, carmustine, melphalan is tried in view to get high response rate of 55- 70% along with bone marrow transplant. But toxic effects are often life-threatening. Its advantages are now doubtful when compared to toxic effects. , Haemopoietic growth factor is also used along with chemotherapy to enhance the cell kill with less bone marrow toxicity. It may also allow multiple high dose chemotherapy to increase the response rate.

He who reigns within himself and rules his passions, desires and tears is more than a king.

549

n

:c

)>

"O

--4

m

:::0 00

550

Radiotherapy is used in bone metastasis, brain secondaries, to prevent paraplegia in spine involvement, and advanced axillary nodes. It is also used in painful bone secondaries, chest wall secondaries. Hormone therapy has got important role. Tamoxifen , oophorectomy, adrenalectomy, androgens, progestogens, aminoglutethimide are used. It is useful in slow growing soft tissue or bone secondaries; or predicted disease-free survival is more than 2 years or age more than 35 years. Blockage of over expression of epidermal growth factor (EGF)/transforming growth factor alpha (TGF-alpha) which are related to ErbB1/ErbB2 receptors in relation to aggressive carcinoma factor is tried.

decompression to prevent paraplegia, lung resection in case of localised secondaries, bilateral oophorectomy. Trastuzumab (herceptin) is monoclonal antibody used in cancers with good results. It blocks the Her-2/Neu and ErbB2 receptors. Bevacizumab and lapitinab are other newer biological agents of different actions. Metabolic complications like hypercalcaemia may be lifethreatening emergency in metastatic breast cancer. It is treated with hydration, steroids, biphosphonates (pamidronate, clodronate) 90 mg intravenously once a month. It also reduces the demineralisation of bone, fracture, pain and paraplegia.

• • • • • •

Most common site of blood spread (70%) Lumbar vertebrae, femur, pelvis Pathological fracture can occur Spinal compression and paraplegia Radiotherapy, internal fixation, spinal decompression is required Biphosphonates 1600 mg/day

B • It signifies terminal event • It has got poor prognosis Respiratory distress and failure is the main feature • HRCT is ideal diagnostic tool • Treated by

• lntercostal tu be drainage • Pleurodesis using talc/tetracycline(1.5 g)/bleomycin (60 units) • Chemotherapy

B Fig. 8.81: Carcinoma of breast operated earlier with skull secondaries.

It is metastatic carcinomaof breast. Skull secondaries are not common. Common site of bone secondaries are lumbar vertebrae, pelvis, upper part of femur, ribs, etc.

• Secondaries in lung-haemoptysis, respiratory failure • Spine involvement-quadriplegia • Secondaries in brain; Cancer cachexia

I Treatment Strategy for Carcinoma Breast Stage 0-DCIS

Stage I and II (Early)

Stage Ill

Fig. 8.82: Secondaries in brain- primary is from breast.

Palliative surgeries done are total/toilet mastectomy, fixation of bones in case of pathological fractures, spinal cord

Stage IV

Less than 4 cm- wide local excision; RT to breast; hormone therapy (Tamoxifen 20 mg OD for 5 years). More than 4 cm-total mastectomy with SLNB (if +ve axillary dissection); hormone therapy Breast conservative surgery (BCS) +RT+ if SLNB +ve axillary dissection. If tumour >1 cm, +ve axillary nodes, high nuclear grade, vascular/lymphatic invasion, ER/Pr - ve with over expression Her-2/Neu, then adjuvant chemotherapy Neoadjuvant chemotherapy + MRM + RT+ CT + Hormone therapy Hormone therapy (Trastuzumab/Lapatinib) with chemotherapy using taxanes or capacetabine + palliative mastectomy (toilet mastectomy if needed when fungation is present)

PROGNOSTIC FACTORS IN CARCINOMA BREAST

s,,,, I II Ill IV

5 years survival

90% 70% 40%

20%

Spread to axillary nodes is the most important prognostic indicator. Age: Younger the age worser the prognosis. Sex: Carcinoma male breast has got worser prognosis compared to female breast. Because of early spread in carcinoma male breast. LYMPH NOOE-AS A PROGNOSTIC FACTOR

• • • • • •

Number of nodes: >2 carries poor prognosis Location of nodes Capsular invasion Extranodal status Size of the node: >2.5 cm has poor prognosis More than 4 nodes/level Ill (apical nodes) involvement has got worst prognosis (5-year survival is 30%) and also decides for radiotherapy to axilla.

Stage I and II has got better prognosis. Atrophic scirrhous has got best prognosis. Medullary carcinoma has got better prognosis than scirrhous carcinoma because of lymphocytic infiltration. Invasive carcinoma has got worser prognosis. Inflammatory carcinoma breast has worst prognosis. ER and PR positive tumours has got better prognosis. Differentiation also decides prognosis. Presence of elastic fibres in histology has got better prognosis. Tumour proliferation stages, growth factor and oncogene factors. Tubular, colloid, papillary types has got better prognosis. Tumour grade, growth factor and oncogene factors. ErbB2Her-2/Neu positive has got poor prognosis. ErbB1 with overexpression of epidermal growth factor (EGF), TGF alpha and cathepsin D has got poor prognosis. DNA flow aneuploid status has got poor prognosis. Low S phase fraction (90% ("'\

70-90%

::c J>

"tJ

30,000/mm3.

I Treatment Diagnostic tapping, tube peritoneal drainage and laparoscopic drainage and wash are useful methods. Laparotomy and peritoneal toilet. Broad spectrum antibiotics including combination of aminoglycosides, cephalosporins and metronidazole. Local instillation of antibiotics, into the peritoneal cavity to achieve quick and effective results. Mortality is high.

I SECONDARY PERITONITIS It is secondary to any bowel or other visceral pathology, e.g. perforation, appendicitis. Escherichia coli (70%) is the most common organism involved. Other bacteria are-aerobic and anaerobic streptococci, Clostridium welchii, bacteroides, staphylococci, Klebsiella, Salmonella typhi. Duodenal perforation and burst appendicitis are th e commonest causes of secondary peritonitis.

I TERTIARY PERITONITIS It occurs after any abdominal surgeries, which is usually severe and the patient may go in for SIRS or MODS early. Tertiary peritonitis is defined as persistent or recurrent intraabdominal infection after an adequate treatment for primary or secondary peritonitis-usually after 48 hours.

It is common in immunosuppressed individual with ineffective peritoneal host defenses against microbes. Infection due to f. faecalis, E. faecium, S. epidermidis, P. aeruginosa, C. albicans are common in such patients. Virulence and resistance to drugs are other factors. It is difficult to diagnose clinically causing delay in therapy. CT abdomen, total and platelet count, LFT, monitoring of renal functions, hourly urine output assessment, chest X-ray are required investigations. Treatment is aggressive antibiotic therapy, antifungal therapy, TPN, maintaining of haemodynamic stability, exploration of abdomen, thorough wash, colostomy/ileostomy or exteriorisation of bowel segment. FFP, packed cells, platelet transfusions may be required. Ventilator, ICU care is often needed. Often these bacteria like enterococci, Candida, Staphylococcus epidermidis, Enterobacter, Pseudomonas shows multidrug resistance and so finding difficulty in managing such patients. Mortality rate is >50%. Problems are-DIC, septicaemia, uraemia (haemodialysis may be needed), haemorrhage, pneumonia, ARDS.

I Mode of Infection Perforation of the GIT-duodenal/gastric/enteric/colonic ulcers; Meckel's diverticulitis perforation. Penetrating or blunt trauma. Surgery; Drains; Dialysis. Foreign body. Appendicitis, cholecystitis, diverticulitis. Intestinal obstruction with strangulation. Via fallopian tubes. Through blood spread-in septicaemia. Transmural spread. Following uterine perforation/injury during abortion or termination of pregnancy.

I Bacteria Causing Peritonitis a. Bacteria from GIT-E. coli, aerobic streptococci, Streptococcus faecalis, Staphylococcus, anaerobic streptococci, anaerobes (bacteroides), Klebsiella, Cl. welchii. b. Bacteria not from GIT-Gonococcus, Pneumococcus, are from fallopian tubes, commonly occurs in young females. Chlamydia, 13-haemolytic streptococci, Mycobacterium are few other bacteria which can cause peritonitis.

r 1. Primary 2. Secondary 3.

Tertiary peritonitis

Monomicrobial, extraperitoneal source, blood spread Most common, polymicrobial, intra-peritoneal source Due to superadded infection following treatment of secondary/primary

Fig . 9.2: Duodenal ulcer perforation causing peritonitis.

Most common bacteria during the phase of peritonitis is E. coli and duri ng abscess formation is B. fragilis. Mortality tor diffuse peritonitis is 10%.

Fig. 9.1: On table finding in a case of peritonitis, where the peritoneal cavity is filled with pus.

Fig. 9.3: Jejuna! perforation due to trauma. Trauma is the common cause for jejuna! perforation. It is commonly blunt abdominal injury due to shearing force (traction injury) causing jejuna! tear near duodenojejunal junction.

Attack the problem, not the person.

559

...

""C

CD

::. 0 C'0

C:

3

560

Bacterial load (2 x 108 CFU/mL) and virulence overwhelm the host defense. It interfere with the phagocytosis, neutrophil activity leading into bacterial multiplication and toxaemia and pus formation. Localised peritonitis- initially localisation of peritonitis occurs based on the anatomical factors like supracolic/ infracolic compartments; greater omentum; paracolic gutters; dilated small bowel, etc. Pathological factors are thickened peritoneum, fibrin deposition, omental adhesions, reduced bowel peristalsis. Localised periton itis may resolve by proper therapy. It may form abscess- pelvic/subphrenic. If it progresses it may form generalised peritonitis. Diffuse peritonitis sets in due to poor localisation, rapid peritoneal contamination, violent peristalsis, virulent organisms, immunodeficiency status, and small omentum in children (not properly developed in children). Diffuse peritonitis may occasionally get localised to form abscess. Mortality in diffuse peritonitis is 10%.

0

n, ::I C n,

:e

-"'

cc

cc Cl)

I Clinical Features Figs. 9.4A and B: lleal perforation with severe peritonitis. Note the

closure done horizontally. It could be typhoid perforation.

Rapidity by which the pus is gushed into the peritoneal cavity, e.g. burst appendix, perforations Amount of peristalsis (more the peristalsis more the spread) Virulence of the organism, bacterial load and nature Localising action of the omentum (in children localisation is poor as omentum is small) lmmunosuppression-HIV, steroids Anatomical nature of the peritoneal cavity Age, associated diseases like malignancy, malnutrition, anaemia

I Pathogenesis Lot of fluid is secreted into the peritoneal cavity which is infected, containing bacteria and toxins causing shock, toxaemia and its subsequent effects. Fibrinogen forms fibrin which tries to localise the infection. Bowel gets adhered to each other with fluid collecting between the loops. Thick flakes are formed adhering to the surface of the bowel. Peritoneum is thick, oedematous, becomes velvety and reddish with loss of its glistening appearance. Omentum is thickened, adherent. Often site of perforation may be identified by the location of the end of the omentum. Dilated bowel loops with site of obstruction/gangrene may be found. Pus, often with pockets may be present in subphrenic, paracolic and pelvic spaces. Peritoneal contents are initially sterile but eventually become infected in certain situations, as in acute pancreatitis, in haemoperitoneum, and in ruptured urinary bladder. It is due to transmural migration of bacteria. In perforated duodenal or yastric ulcers, contents in the peritoneal cavity are initially sterile but later get infected to form bacterial peritonitis.

Sudden onset of pain which is severe. Fever, vomiting. Tenderness-initially localised later becomes diffused. Rebound tenderness-Blumberg sign. Guarding and rigidity, dull flanks on percussion. Tachycardia, tachypnoea. Tenderness on P/R examination. Distension with silent abdomen. Eventually leading to Hippocrates facies, septicaemic shock and loss of consciousness. Bowel sounds are absent due to paralytic ileus. Fever may be absent in severe peritonitis due to loss of pyrogenic reaction. Total count also may be very low in severe peritonitis. APACHE II (Acute Physiology And Chronic Health Evaluation [using 12 physiological variables]) and Mannheim peritonitis index (8 parameters) scoring systems are used to assess the severity and predicting the outcome in peritonitis.

Fig. 9.5: Small bowel perforation with peritonitis with thick plaques

on the bowel surface.

I Investigations Chest X-ray in standing position with abdomen (erect) will show ground glass appearance of the abdomen with gas

under diaphragm suggesting hollow viscous perforation. Left lateral decubitus X-ray is very useful in very critical patient where patient cannot be made to stand in erect posture. It should be differentiated from the rare Chilaiditi syndrome wherein interposition of bowel in front and above the liver looks radiologically like gas under diaphragm.

fluid will be low in bacterial peritonitis but will show raised protein and LOH (greater than serum LOH). Diagnostic laparoscopy. It can also be used as therapeutic tor duodenal ulcer perforation or primary peritonitis to give peritoneal wash. CT scan is useful to confirm the cause or to rule out conditions like pancreatitis. CT scan detects bowel ischaemia, gangrene, perforation, internal hernias and quantity of pus/ fluid in the peritoneal cavity. MRI is reliable in identifying intra-abdominal abscess.

I Differential Diagnosis

Fig. 9.6: Plain X-ray abdomen showing gas under diaphragm.

Pancreatitis: In pancreatitis back pain is common. But it is often difficult to differentiate clinically from acute peritonitis. Serum amylase is increased. CT abdomen may help in differentiating from peritonitis. Intestinal obstruction: There will be distension, vomiting and pain. Plain X-ray shows dilated bowel loops. CT scan confirms intestinal obstruction and its cause.

Perforation is the most common cause of peritonitis.

Fig. 9.7: Plain X-ray abdomen showing ground glass

appearance-a feature of peritonitis. Total count is increased. US abdomen-shows fluid in the abdominal cavity. Also clinches the other causes like haemoperitoneum, pancreatitis. Electrolyte study, blood culture Blood urea and serum creatinine. Serum amylase if four times the normal value, it is significant. LFT also should be done. Platelet count, bleeding time, clotting time, prothrombin time are to be assessed in severe peritonitis. Four quadrant abdominal tap-reveals pus or infected fluid. In suspected pancreatitis fluid should be analysed for amylase level (which will be high). Often US guided aspiration is better and more accurate. Fluid collected should be sent for culture. Diagnostic peritoneal lavage (DPL) is useful. OPL with more than 500 WBCs/mL suggests peritonitis; fluid should be assessed for pH, sugar, protein, LOH, cell count, Gram stain, culture. Fluid is analysed for amylase or bilirubin or creatinine in suspected cases of pancreatitis or biliary leak/peritonitis or urinary leak. pH ( -a

-4

m

:l:J C0

"'tJ

(D

; :. 0

::,

(D

C

3

568

...> ...en Q)

::I

en C

::I

r::

:: en

CCII

a:

en

Tenderness in right hypochondrium. Sympathetic right sided pleural effusion due to congestion and hyperaemia of the diaphragm. Collapse of the lung/basal atelectasis-right side may be evident. Tachypnoea and respiratory distress can be observed . Pain in the right shoulder due to irritation of phrenic nerve . Hiccough, tachycardia. Hoover's sign: Scoliosis towards same side in subphrenic abscess. Wasting and anorexia is common. Occasionally tender mass in the right hypochondrium may be palpable. Tenderness over 11th intercostal space may be evident in right subhepatic space abscess. Differential diagnosis; Amoebic liver abscess; Pylephlebitis; Empyema; Pulmonary collapse.

drained. Catheters used are pigtail catheter, 16 French trocar catheter or sump catheter. 90% of subphrenic abscess are drained percutan eously.

B Malecot's catheter/wide Foley's catheter when open drainage is done Pigtail catheter Catheter placement through trocar under guidance (16 French trocar catheter) Sump catheter (double lumentubewhichallows irrigation as well as suction drainage) Note: Catheter is removed when pus drainage from the tube is Always evaluate for associated injuries-fractures, thoracic and head injuries. ,.. Haematocrit, electrolyte esti mation, blood grouping. Treatment , Emergency laparotomy and resection of bowel or suturing of the mesentery.

Peritoneal mesothelioma is the 2nd common type of mesothelioma; first being the pleura. Asbestos is the prime cause; by inhalation or ingestion. It is 20% of all mesotheliomas. It is very aggressive primary type with survival rate of 6-12 months. It is often difficult to diagnose; vague presentation mimicking ovarian pathology or inflammatory bowel syndrome. Presentation is abdominal distension (commonest), abdominal pain, ascites, weight loss, and cachexia. Omentum is involved with malignant nodules presenting like a nodular abdominal mass. Peritoneal cavity is studded with nodular deposits. Entire peritoneal cavity is involved often involving the organs like liver or their surfaces. •·· It is often difficult to differentiate it from peritoneal carcinomatosis. However, extra-abdominal spreads to lungs and bone is uncommon in malignant mesothelioma. Histologically it can be epithelioid (75%, better prognosis), sarcomatoid and biphasic. Staging is done through laparoscopy or laparotomy. Stage I: Localised disease without nodal spread; II: Localised with mi nimal nodal spread; Ill: Localised to one side of the body with involvement of nearby organs and nodes; IV: Spread to both sides of body with distant spread. Peritoneal cancer (carcinomatosis) index(PCI, Sugarbaker's) is used to assess the peritoneal mesothelioma based on 13 sectors with total score of 39. PCI scores of 1- 10, 11-20, 21-30, and 31-39 correspond to T stages of 1, 2, 3, and 4, respectively. Stage I disease included T1 N0M0, stage II included T2-3N0M0, and stage Ill included T4N0M0 and any N/M positive disease. Investigation: CT abdomen, ascitic fluid analysis, laparoscopy are the diagnostic methods. Treatment: Cytoreduction surgery (CRS/surgical peritonectomy) with Hyperthermic intraperitoneal chemotherapy (HIPEC), using mitomycin C, intraperitoneal chemotherapy of cisplatin, early post operative intraperitoneal chemotherapy (EPIC)-are treatment options.

Power tends to corrupt and absolute power corrupts absolutely-TS Subrahmaniam

3

572

... Cl)

en

-

:::I Cl)

0

n, :::I

C n,

:E _vi

a::I

a: Cl)

I Pseudomyxoma Peritonei •·· It is a rare malignant condition that develops due to ruptured malignant ovarian cyst or adenocarcinoma of appendix. Peritoneum is coated with mucus secreting tumour with tenacious gel like mucus with cystic masses. It is common in old aged females. Vague abdominal pain with distension and dullness which is not shifting are typical findings. CT scan shows bowel displacement with fluid content in the peritoneal cavity. Treatment: If origin is known then it is treated accordingly. In patients operated by appendicectomy, removal of all mucus gel with right hemicolectomy with omentectomy is done. If ovarian tumour is the cause then panhystrectomy with omentectomy is done. In cases with unknown cause, after laparotomy, panhystrectomy, right hemicolectomy, omentectomy are done. lntraperitoneal chemotherapeutic agents like 5 FU, mitomycin C, oxaliplatin; intraperitoneal instillation of mucolytic agents like dextran, uro kinase after surgery is beneficial. It shows better prognosis compared to mesothelioma and carcinomatosis.

Entire peritoneal cavity is studded with carcinomatous nodulesboth parietal and visceral peritoneum Haemorrhagic ascites, interloop bowel adhesions, hard studded nodules in the omentum are common. Intestinal obstruction can occur It may be primary peritoneal carcinomatosis (mesothelial) or secondary from GIT, ovary, breast, pancreas Liver secondaries are often present It mimics peritoneal tuberculosis, acute pancreatitis, and multiple lymphomatous nodules, multiple peritoneal hydatids As malignant ascitic fluid contains high immunoglobulin, peritonitis is not common lnstillation of radioactive gold and chemotherapy are the treatment It has got poor prognosis

I I

When it attains large it presents as mass in central or lower abdomen which is smooth well localised mobile often moves with respiration. It can cause torsion, ruptureand infection. Extrinsic compression of bowel can occur. US/CT shows cyst with internal septations. Differential diagnoses are-mesenteric cyst, retro peritoneal cyst and desmoid tumour. Treatment is laparotomy and cyst excision which can also be done laparoscopically.

OMENTAL TORSION Torsion of greater omentum is twist of omentum along its longitudi nal axis; it usually occurs on right side of omentum. Primary torsion is torsion occurring without any existing cause. Secondary torsion is occurring due to some cause like omentocele in a hernial sac or tumour or adhesion. Torsion is more common in men (2:1) in 5th decade. Sudden severe abdominal pain, mainly right sided often mimicking acute appendicitis, cholecystitis, ureteric stone or twisted ovarian cyst. Often signs of peritonitis may be present. CT abdomen is diagnostic. Total count will be raised. Treatment is laparotomy and omentectomy which can also be done laparoscopically.

OMENTAL TUMOUR Metastatic omental deposits are common; usually from GIT cancers. Primary neoplasm of omentum is rare. It may be soft tissue tumour arising from fat of omentum.

L

OMENTAL CYST It arises from obstruction of omental lymphatics either congenital or acquired. It is un ilocular or multilocular cyst lined by endothelium. It is common in children.

Fig. 9.26: Omental secondaries.

Chapter

Abdominal Tubercu losis In man the balance between immunity and susceptibility to tuberculosis is delicately adjusted: There is a small factor of safety. - Milton Rosenau , 1927

...... ..

..

-:-.lllflA P TE R OUTLINE

..

Abdominal Tuberculosis lleocaecal Tuberculosis lleal Tuberculosis Peritoneal Tuberculosis Tuberculous Mesenteric Lymphadenitis

..

Ano-recto-sigmoidal Tuberculosis Tuberculosis of the Omentum

ABDOMINAL TUBERCULOSIS It is common in India and developing countries. It is the 6th most common type of extrapulmonary tuberculosis. Its incidence is high in HIV infected patients.

I Types 1. Intestinal ,,. lleocaecal region: (a) Ulcerative-60%; (b) Hyperplastic; (c) Ulcero-hyperplastic. ,. lleal region, 40% commonly: Strictu re type. 2. Peritoneal tuberculosis a. Acute. Sits spectrum

3. 4.

5. 6. 7.

b. Chronic. i. Ascitic type. ii. Encysted (loculated) type. iii. Plastic (fibrous/adhesive) type. iv. Purulent type. Tuberculosis of mesentery and its lymph nodes. Ano-recto-sigmoidaJ-present as fistula, fissure, abscess, mass. Involvement of liver, spleen and other organs as a part of miliary tuberculosis. Tuberculosis of the omentum. Rare types: Oesophageal (0.2% of abdominal tuberculosis)/ gastroduodenal (1 % of abdominal tuberculosis)/retroperitoneal tuberculosis. Because of hydrochloric acid (acid media) gastric tuberculosis is rare.

Note: • Chronic peritoneal tuberculosis may be associated with pleural ettusion and pericardia! ettusio n. • Tuberculosis is not common in stomach, duodenum and jejunum. • Diffuse tuberculous colitis is less commonly seen (4%), mimics ulcerative colitis in every respect even in colonoscopy. Patient recovers well with antituberculous drugs. • Intestinal tuberculosis is called as Koenig's syndrome ( 1892). • Isolated or segmental colonic tuberculosis is 5% common without ileocaecal tuberculosis; it is often multiple; colonoscopy is diagnostic.

• Luminal GI tuberculosis-55-60% • Abdominal solid organ tuberculosis-liver, spleen, pancreas-1 % • Peritoneal tuberculosis-adhesive (cocoon/doughy/rolled up omentum)/ascitic (wet)- loculated. It could be acute or chronic40%.

1/socaecal and terminal ileum-most common site Jejunum and colon-less common Stomach, duodenum, oesophagus-rare Pathology: • In malnourished with poor resistance patients-ulcerative and or stricture types

• Abdominal lymph node tuberculosis-mesenteric/periportal/ para-aortic/retroperitoneal. It could be caseating/hyperplastic/ calcified-5%

• In well nourished with good resistance-hyperplastic and nodular types • Combination types-ulcerostricture type; ulcero-hyperplastic type

Phthisis is an ulcer of the lung that consumes the whole body. --aernarll lie Gorllan, 1495

574

T

Different clinical presentations of abdominal tuberculosis

Clinical presentation may be:

Types:

• Acute • Acute on chronic • Chronic

• Ulcerative-{liarrhoea and malabsorption • Stricture-subacute or acute intestinal obstruction • Hyperplasti54 U/1; asctic ADA >33 U/1; ascitic fluid: serum ADA >0.985. • Bactec MGIT broth culture: It is Mycobacterium growth indicator tube cultu re system containing Middlebrook Broth with silicon base; fluorescent technology is used.

Method used to isolate tuberculous bacteria ZiehlNeelsen stain microscopy LowensteinJenson solid media culture Saelee MGIT broth culture COBAS Taqman PCR

Time AFB/ml specimen taken for reporting 5,00010,000/ ml 100/ml

577

Sensitivity Specificity

1-2 hours

75%

85%

4-8 weeks

80%

90%

C")

::c

> "'tJ -4

m

:::c

......

= 10/ml

2-6 weeks

Less than 24-48 10/mL hours

94%

92%

97%

99%

> er

C. 0

3 ::I I»

.....

= er

Exudate with protein level ~3.0 g/dL Serum-ascitic fluid albumin gradient is 250/cumm AFB in ascitic fluid is seen only 1.016 • Glucose er

1. Acute Typ&--Mimics Acute Abdomen

3

Exploratory laparotomy reveals straw-co loured fluid with tubercles in the peritoneum, greater omentum and bowel wall. It is an on-table diagnosis. Fluid is evacuated and collected for AFB study and culture. Omental biopsy is taken. Abdomen is closed (without a drain) with tension sutures to prevent burst abdomen. ATD is started.

r;---Peritoneal tuberculosis is not uncommon; many patients I present with peritoneal tuberculosis. There will be thickening and fibrosis of the peritoneal wall which is studded with multiple tubercles. It can be 'wet' type or 'dry' type. Wet type is common (95%) with formation of different types of ascites. Ory type (5%) shows typical fibrosis and adhesions. It may be post primary as activation of long-standing latent focus. It can be from blood spread or can develop from diseased mesenteric lymph nodes or intestine or Fallopian tubes. Presentations ar&--Distension of abdomen, recurrent subacute intestinal obstruction , pain abdomen, recurrent fever, loss of weight and appetite. Different types are-abdominal cocoon, ascitic, loculated, plastic, acute and purulent. • Evaluation-Chest X-ray, USG abdomen, CT abdomen , ascitic fluid analysis, peritoneal biopsy or diagnostic laparoscopy. Serum ascitic fluid gradient is less than 1.1 g/dl; ascitic fluid/blood glucose ratio is less than 0.96; cells mainly of lymphocytes will be more than 1000/ul. Achieving positive AFB staining in ascitic fluid is only 3%; fluid culture will j show Mycobacterium tuberculosis in 20% cases; centrifuged ascitic fluid once cultured will show 80% positivity for AFB. Ascitic fluid ADA raises (33 units/litre is very sensitive) due to stimulation of T cells by tubercular antigen. It is compared to serum ADA level. Serum ADA >54 units/litre and ascitic fluid to serum ADA ratio >0.985 is considered as tuberculosis. It is low or normal in HIV; it is high in malignancy. Interferon y raises in ascitic fluid along with ADA. • Treatment-Antitube rcular drug therapy is the main modality. Surgery is only indi_ cated if the~e is complete I or recurrent intestinal obstruction, perforation or multiple strictu res. Stricturopla~ty, l~calised rese~tions ar~ done. , Sepsis, abscess formation, fistula formation, surgical site infection are the complications of surgery.

579

ACUTE PERITONEAL TUBERCULOSIS

Rare, On-table diagnosis Features of peritonitis Due to perforation or rupture of mesenteric tuberculous lymph nodes

2. Chronic Tuberculous Peritonitis Present as abdominal pain, fever, ascites, loss of weight and appetite, abdominal mass, doughy abdomen (10%). Peritoneum is thickened with multiple tubercles. Omentum is thick, fibrosed, rolled up. Infection is usually from mesenteric lymph nodes, ileocaecal tuberculosis, from Fallopian tubes rarely blood born (from lungs). Laparoscopy is very useful in this type to diagnose. a. Ascitic form

Ascitic form shows enormous distension of abdomen with dilated veins. It presents with congenital hydrocele in male with patent processus vaginalis, umbilical hernia, rolled up omentum , shifting dullness, fluid thrill, and mass abdomen. Ascitic tap reveals straw coloured fluid from which AFB can be isolated. Fluid is pale yellow, clear, rich in lymphocytes, with high-specific gravity. Chest X-ray, Mantoux test are other required investigations. ATDs for one year is required. Repeat tapping may be required initially as part of the treatment. Note:

Ascites may be free or loculated; clear or complex; debris or septae; 'sliced bread' appearance.

b. Encysted (loculated) ascites Ascites gets locu lated because of the fibrinous deposition. Dullness, which is not shifting, is the typical feature.

Great things are done by a series of small things brought together.

Cl. 0

:::, Q)

-i C

...n

er (D

C 0

en

c;;·

580

...::s Q)

en

v.,

Figs. 10.13A and B: US picture showing ascites. Straw coloured

ascitic fluid tapping done under US guidance.

c. Plastic type Here there are widespread adhesions between the coils of the intestine (ileum commonly), abdominal wall, omentum , with distension of the small bowel, leading to blind loop, ileus, intestinal obstruction (subacute, acute), thickened parietal peritoneum.

Figs. 10.11A to C: On-table findings in intestinal tuberculosis,

of extensive involvement with multiple tiny tubercles, thickening, adhesions and involvement of mesenteric lymph nodes. Fig. 10.14: CT scan abdomen showing ascites due to tuberculosis.

They get recurrent colicky abdominal pain, diarrhoea, wasting, and loss of weight, mass abdomen, and doughy abdomen. Differential diagnosis: Peritoneal carcinomatosis. Open/ laparoscopic peritoneal biopsy is very useful tool to diagnose. They respond well for drug treatment. Surgery is indicated if obstruction occurs.

Fig. 10.12: Abdominal distension with ascites due to abdominal

tuberculosis. They may present as intra-abdominal mass, which may mimic ovarian cyst, retroperitoneal cyst or mesenteric cyst. Treatment is U/S guided aspiration along with ATDs.

d. Purulent form It is invariably due to tuberculous salpingitis, presenting as a mass in the lower abdomen containing pus, omentum, fallopian tubes, small and large bowel. Cold abscess gets adherent to the abdominal wall, umbilicus and may form an umbilical fistula. Patient commonly has got genitourinary tuberculosis. US, discharge study, X-ray abdomen and other investigations are useful.

581

::r» er

Cl. 0

3

::I Q)

C

Figs. 10.15A and B: Laparoscopic picture of loculated ascites due to abdominal tuberculosis. Fluid is getting aspirated using a needle under laparoscopic vision.

•·· Treatment: ATDs are started exploration of umbilicus, exploration of fistula and bowel bypass is done. •·· Prognosis is poor in this type.

Figs. 10.17A and B: Laparoscopy showing adhesions between bowel and abdominal wall due to tuberculosis.

Ascitic form Insidious onset Fatigue, weight loss, lever, anorexia

Figs. 10.16A to C: Laparoscopy showing plastic type of abdominal tuberculosis and multiple tubercles over parietal and visceral surface of the peritoneum.

Loculated form Loculated abdominal swelling Ascites which is not shifting Abdominal pain usually absent Diagnosis-US guided aspiration Abdominal distension usually without Antituberculous drugs pedal oedema Rolled up omentum is common association as mass Fibrous form Purulent form Widespread adhesion of bowel loops Due to tuberculous salpingitis Presenting as lower abdoAdhesions, matting and dilatation minal mass Act as blind loop-malabsorption Umbilical fistula, abdominal wall abscess can occur Abdominal pain, loss of weight, Cold abscess adherent to abdominal wall doughy abdomen due to thickened parietal peritoneum, diarrhoea, often mass abdomen-are usual features

Example isn't the best way to teach, it's the only way.

er

nl ..,.

n C

0 Ill

c:;;·

582

... ...

>,

Cl>

Cl

::I Cl)

c

Cl.I

ca

a: Cl)

TUBERCULOUS MESENTERIC LYMPHADENITIS Infection is usually through the Peyer's patches of the intestine (i.e. through oral cavity). Usually several lymph nodes are often involved causing massive lymph node enlargement. Commonly right-sided lymph nodes are involved, but left sided nodes can also get involved. It presents with general symptoms (fever, malaise, weight loss). Pain in umbilical region and right iliac fossa, mass in right iliac fossa, which is matted, nonmobile. It may present with features of acute appendicitis. Often coils of intestine get adherent to the caseated mesenteric lymph nodes leading to intestinal obstruction. Most often caseating material may collect between the layers of the mesentery, forming a cold abscess, mimicking a mesenteric cyst (Pseudomesenteric cyst). Massive enlargement of mesenteric lymph nodes due to tuberculosis is called as tabes mesenterica. Mesenteric tuberculous adenitis is more common in chi ldren. Present with anaemia, fever, loss of appetite and reduced weight, failure to thrive, palpable mass in right iliac fossa which is firm and nodular.

Fig. 10.18C

Figs. 10.18A to C: Mesenteric tuberculous lymphadenitis on table findings.

Lio, of

r.:.:~--

,.,•

,,,,,'

,.

,•

r.J

Tuberculous mesenteric lymphadenitis

Figs. 10.19A and B: Note the location of mesenteric tuberculous lymphadenitis in the right iliac Iossa. But it can occur anywhere in the line of mesentery. On-table caseating tuberculosis lymphadenitis is also shown.

B Mesenteric tuberculous adenitis-acute/chronic in right iliac Iossa- mass Pseudomesenteric cyst

Figs. 10.18A and 8

• Cold abscess within the mesentery Tabes mesenterica

Differential Diagnosis: Carcinoma caecum; Lymphoma; Retroperitoneal tumour; Nonspecific lymphadenitis. (Acute nonspecific mesenteric lymphadenitis is called as nurses' syndrome).

Investigations: ,. X-ray abdomen shows calcification. ,. U/S may confirm the diagnosis. ,. Mantoux test may be positive. ,. Diagnostic laparoscopy-is very useful in TB lymphadenitis. Mesenteric cold abscess can be drained safely through laparoscopy. Treatment: ATDs; Laparoscopy and proceed. Prognosis is good.

ANO-RECTO-SIGMOIDAL TUBERCULOSIS It mimics carcinoma rectum. Haematochezia is the most common symptom (90%); 40% present with constipation; condition may mimic carcinoma rectosigmoid. It presents as tenesmus, diarrhoea, and discharge from the fistula and occasionally as mass per abdomen. Rectal tuberculosis occurs usually within 10 cm of anal verge. Fistulas are painful and characteristically not indurated. Tuberculous fistulas are commonly multiple. Tuberculous anal ulcers when occur are shallow, bluish, with undermined edges. Sigmoidoscopy, U/S, discharge study, fistulectomy and biopsy confirms the diagnosis. Treatment is ATDs, fistulectomy, often sigmoid resection.

TUBERCULOSIS OF THE OMENTUM It usually occurs as a part of the other types of abdominal tuberculosis. Rolled up omentum with thickening is characteristic. Often cold abscess can develop per se in the omentum. If it is so, it can be dealt with laparoscopy safely under the cover of ATDs. In all abdominal tuberculosis, drug treatment is for one year. In adrenal tuberculosis and severe adhesions, steroids may be beneficial.

Fig. 10.20 : Omental tuberculosis showing tubercles.

B

INDICATIONS FOR STEROIDS IN TUBERCULOSIS

• Miliary tuberculosis Tuberculous meningitis Adrenal tuberculosis Tuberculous pericarditis Pleural and endobronchial tuberculosis In abdominal tuberculosis to prevent adhesions Tuberculosis of the mediastinal lymph nodes (Rapidly progressive) Genitourinary tuberculosis Ocular tuberculosis

I Remember AFB staining of fixed tissue in abdominal tuberculosis is possible only in 5% cases. Ziehl-Neelsen staining is done. (Carbal fuchsin-steam heat for 7 minutes-water washdecoloraisation with 20% H2S04-95% ethanol wash-counterstain with Loeffler's methylene blue/1 % picric acid/0.2% malachite green. Tuberculous bacilli are bright red rods seen under oil immersion lens). Culture takes 8 weeks to get the result. 35% of abdominal tuberculosis shows positive in culture. Lowenstein Jenson media is used. Guinea pig inoculation is also useful method. WHO now recommends antituberculous drugs for 6 months. Uncomplicated cases-4 drugs for 2 months and 2 drugs for 4 months. Complicated cases-4 drugs for 2 months and 2 drugs for 7 months. But many patients need one year treatment. Because of complications and difficulty in managing recurrent cases of abdominal tuberculosis one year therapy is C"mmonly used in developing countries. First line drugs are-isoniazid-5 mg/kg; rifampicin-10 mg/kg; ethambutol-15 mg/kg ; pyrazinamide-25 mg/kg. Second line drugs are-amikacin; kanamycin; PAS (Paraamino sulphuric acid); ciprofloxacin; ofloxacin; clarithromycin; azithromycin; rifabutin. Follow-up and prognosis is monitored by-regular weight check to see for gain; improvement in appetite; reduction of abdominal pain and distension; absence of fever; normal bowel habits; normal haemoglobin; ESR becoming normal; and US abdomen shows improvement in sonological features. Patients who are not responding in 6 weeks should be reassessed again for-drug resistance; associated other diseases like malignancy (carcinomas or lymphoma), Crohn's disease, eosinophilic enteritis. During therapy, patient who is responding for drug therapy can also go for intestinal obstruction due to fibrosis during healing stage. It needs surgical intervention. Repeated surgery in abdominal tuberculosis is difficult and dangerous as chances of developing faecal fistula, further adhesions are more likely.

The same beam of light can illuminate two objects and produce two different effects.

583 n

:::c

> -,::,

-I

m

:::D ..4

c:::>

>

C"

CL 0

3

::I

I»

-i

C: CT

...n C1)

C: 0

en en

Chapter

Liver The liver... that great maroon snail.. .. No wave of emotion sweeps it. Neither music nor mathematics gives it pause in its appointed tasks. -Richard Selzer, 1976

.. ..•

~HAPT ER OUT LINE

• • •

.

Surgical Anatomy of Liver Liver Function Tests Alpha Fetoprotein Liver Biopsy Liver Injury Infections of Liver • Amoebic Liver Abscess • Pyogenic Liver Abscess • Portal Pyaemia (Pylephlebitis) • Hydatid Cyst of Liver • Actinomycosis of Liver Liver Tumours • Benign Tumours of the Liver • Primary Malignant Tumours of the Liver

.. • • •

•

.. • •

• • •

•

• Hepatocellular Carcinoma (Hepatoma) • Secondaries in the Liver Liver Cysts • Congenital Liver Cysts • Neoplastic Liver Cysts • Traumatic Liver Cyst Portal Hypertension Oesophageal Varices Emergency Management in Severe Haemorrhage Ascites Ascites in Portal Hypertension Budd-Chiari's Syndrome Hepatic Failu re Hepatic Encephalopathy Hepatorenal Syndrome Hepatic Resection Portal Biliopathy

Hepatic artery commonly originates from coeliac axis. There are many variations of hepatic artery, both in its origin and its pathway and is considered surgically important. Hepatic artery ligation is one of the surgical palliation for advanced hepatocellular carcinoma and secondaries in liver as tumour tissue is supplied exclusively by hepatic artery but normal tissue is also supplied by portal vein. Superior mesenteric vein and splenic vein join dorsal to the neck of pancreas to form portal vein. Hepatic artery, portal vein and bile duct are located in the free edge of the lesser omentum until it enters the liver. Venous drainage is usually through the three major hepatic veins, right, left, and middle which drain into the IVG. Often inferior hepatic vein may be present. The liver parenchyma is entirely covered by a thin capsule called 'Glissons capsule' and by visceral peritoneum all over but the posterior surface of liver termed 'bare area'.

SURGICAL ANATOMY OF LIVER Liver has four lobes- right lobe, left lobe, quadrate lobe and caudate lobe. Supporting structures are right triangular ligament, left triangular ligament and falciform ligament. Liver weighs around 1.5 kg.

It is a unique organ with dual blood supply. Hepatic blood flow is around 1500 ml/minute of which portal vein contributes 80% and hepatic artery 20%.

m Figs. 11 .1A an d B: Anatomical location of liver and its lobes.

Segmental lobes Rt Lobe

I

LIVER FUNCTION TESTS (LFT)

Lt Lobe

Gallbladder

Fig. 11 .2: Segmental anatomy of the liver.

I Segmental Anatomy of Liver Liver is divided into functional right and left lobes by a line passing from the left of the gallbladder fossa to the left of IVC- Cantlie's line creating Couniaud's segments. There are eight segments: ,. Segments I, II, 111, and IV are of left lobe. ,, Segments V, VI , VII , VIII are of right lobe. ,, Segment I is the caudate lobe of the liver and has independent supply of portal and hepatic veins. This hepatic vein directly joins IVC. Right lobe is having right hepatic artery, right branch of portal vein, and right hepatic duct. Left lobe is having left hepatic artery, left portal vein, left branch of bile duct. Bismuth 's classification is similar bu t separation of the sectors is by hepatic veins. Functional unit is called as hepatic lobule and it contains central hepatic vein and portal triad (hepatic arteriole, portal venule, bile ductule).

I Lymphatic Drainage of the Liver Superficial vessels drain in four directions; from ligaments into the thoracic duct; porta hepatis to the hepatic nodes then to celiac nodes; from rest of the ri ght lobe to the celiac nodes; posterior part of the lett lobe drains to paracardial nodes near oesophageal hiatus; rest of the left lobe to celiac nodes. Deep lymphatic vessels form ascending and descending trunks; ascending trunk drains to nodes adjacent to IVC; descending trunk drains into hepatic nodes and to celiac nodes. FUNCTIONS OF THE LIVER

Removal of gut endotoxins and foreign antigens-liver acts as the first filter Drug and hormone metabolism Formation of bilirubin and its metabolism Formation of urea from protein catabolism Glucose metabolism, glycolysis, and gluconeogenesis Clotting factors synthesis pH balance and correction of lactic acidosis Maintaining body temperature Storage of vitamin B12, vitamin A, Cu, Fe

1. Serum bilirubin which includes both direct and indirect. Test is known as van den Bergh's test. 2. Serum albumin, globulin and A: G ratio; serum albumin is the indicator of chronic liver disease. 3. Prothrombin time: Normal value is 12-16 seconds. Difference between control and test more than 4 seconds or test being more than 1½ times the control is significant. When it is altered it is corrected by injecting vitamin K, 10 mg IM for 5 days or by fresh frozen plasma (FFP)- For cell synthesis. 4. Alkaline phosphatase-Secretaty function. 5. Aspartate amino transaminase 5-40 IU//itre (AST, SGOT)signifies inflammation. 6. Alanine transaminase 5-40 IU/litre (ALT, SGPT), liver specific. 7. 5 nucleotidase. 8. Gamma glutamyl transpeptidase (GGT) 10-48 IU/L. 9. Immunological tests: Antimitochondrial or antinuclear antibodies. 10. Alpha-fetoprotein (AFP). 11. Specific tests: (a) For haemochromatosis: Serum iron, total iron binding capacity, serum ferritin. (b) Wilson's disease: Serum copper, urinary copper, serum ceruloplasmin. 12. Fluorodeoxyglucose-positron emission tomography (FOG· PET): It is to find out the uptake of labelled glucose which varies in different diseases of liver, i.e. benign, malignant and inflammatory. 13. Technetium-99m labelled radioisotope scan shows the uptake and excretion of bile. 14. A sulphur colloid liver scan shows specifically Kupffer cell activity. Sulphur colloid will not show uptake in adenoma and haemangioma as Kupffer cells are absent in these lesions. 15. Urine for bile salts (Hay's tesQ, for bile pigments (Fouchet's tesQ and for urobilinogen (Ehrlich's aldehyde test). Hepatocyte function

AST, ALT

Synthetic function and metabolism

PT-INR, factor V. VII, album in, bilirubin

Biliaty canalicular function ALP, 5' nucleotide, gamma glutamyl transferase, bilirubin

B

OTHER INVESTIGATIONS FOR LIVER DISEASES , I t I '

I I I

• • I

Laparoscopy and laparoscopic US Liver biopsy

ALPHA FETOPROTEIN (AFP) It is normal component of the plasma protein of the fetu s secreted by yolk sac and embryonal hepatocytes (fetal liver),

I prefer to learn and to teach anatomy, not from books but from dissections; not from the positions of philosophers but from

the fabric of nature. -William Harvey

585

(")

:c

>

m

::J:J

_,,

_,,

586

...=

Q)

en Cl)

0

which usually reduces after birth and persists as a low value level. Gene is located in the q arm of chromosome 4- 4q25. -== ========= ===~ ~ a It binds to copper, nickel, fatty acids and bilirubin. a. Stilette b. Biflanged needle It is a g/ycoprotein containing a carbohydrate moiety with 33 591 amino acids. AFP in fetus protects fetus from maternal :> b estradiol. Normal value is up to 10 ng/ml. Immediately after f'..:..1 birth it will be higher but in a year it reaches to normal value. It is an important tumour marker for HCC more often to a -..r-L......,...1,~~::;;;;:::=;5 HCC with cirrhosis (100-1000 ng/ml) where it increases enormously. In HCC, it is also prognostic indicator to identify relapse or residual disease. HCC less than 4 cm may show a. Needle b. Locking pin c. Stilette only raise up to 200 ng/ml. 20% of HCCs and variants like rn Figs. 11.3A and B: Liver biopsy needles. (A) Vim-Silvermann; fibrolamellartype may have normal AFP. Serum lens culinaris (B) Menghini. agg lutinin reactive fraction of AFP, an isoform of AFP (AFPL3) is also used as tumour maker for HCC. It also increases in benign conditions also to certain extent LIVER INJURY but never more than 100 ng/ml. Liver is most vulnerable abdominal organ for injury. That is due It also increases in nonseminomatous testicular tumours, to its size, anterior location, mobility, fragile nature and thin hepatoblastoma, and some ovarian tu mours (yolk sac Glisson's capsule. It can be blunt injury, stab and gunshot injury. tumour). About 5% of all trauma admissions will have liver injury. It is Serum value in adults, serum and amniotic fluid value in most common organ injured in penetrating abdominal trauma; pregnant women are assessed. it is the 2nd most common organ injured in blunt abdominal It is useful in diagnosing the omphalocele, ataxia telangiectrauma first being spleen. 40% of blunt liver trauma is associated tasia, neural tube defects. with splenic injury; 30% of it with rib fractures. AFPep is a peptide derived from AFP which shows anticancer features. Types

- .::;:=========~~ c--==========::::::5

I

LIVER BIOPSY Prerequisites: Prothrombin time should be normal before doing liver biopsy, otherwise severe bleeding can occur. Normal value of prothrombin time is 12-16 seconds (It is compared to control and difference should be less than 4 sec between test and control). It is corrected by giving injection vitamin K 10 mg IM, for 5 days. After that if repeat PT is still high then fresh frozen plasma (FFP) is required to correct it. Indications: ,.. Cirrhosis, chronic hepatitis, haemochromatosis, Wilson's disease, metabolic diseases, and neonatal conditions. , Only advanced hepatocellular carcinoma (HCC), in selected liver secondaries where primary is not detected. Complications: Haemorrhage, Infection; Bile leak and biliary peritonitis. Contraindications: Hydatid disease, where it will precipitate anaphylaxis; Haemangioma, bleeding disorders, ascites.

I Procedure Needle used here is either Vim-Silvermann needle or Menghini needle (better option). Needle is usually punctured through right midaxillary line in 9th intercostal space or under US guidance into any place as required. Presently, guided liver biopsy is commonly recommended and popular, i.e. US guided, CT scan guided, laparoscopy guided.

It can be contusion, laceration, avulsion, extension into thorax and biliary tree may be associated with other organ injuries (spleen, kidney, duodenum, bowel, IVG) and with fracture ribs. Liver injury can be: Penetrating: It often requires surgical intervention . After laparotomy with rooftop incision, laceration is assessed, clots and blood in the peritoneal cavity is removed. Using Pringle manoeuvre bleeding is temporarily controlled. Often suprahepatic IVG and infrahepatic IVG control may be required. Liver wound is sutured using specialized needle with vicryl using co-opting sutures. Gelfoam or Surgicel is placed over it. Other injuries like of diaphragm, biliary system, bowel should be looked for. Adequate amount of blood, FFP should be kept for transfusions. Postoperative assessment of BT, CT, PT, platelet count, observation for sepsis is needed. Ideal antibiotic coverage using 3rd generation cephalosporins or higher are needed. Blunt trauma: It is assessed by CT scan. It is usually treated conservatively. Indications for surgical intervention are-progressive deterioration and bleeding, grade 5 liver injury on CT scan, associated bowel injury. Commonly used procedure after laparotomy is keeping packs between liver and diaphragm which is removed in 48-72 hours. Occasionally venovenous bypass, hepatic resection may be required.

I Clinical Features Features of shock due to severe torrential bleeding (pallor, hypotension, tachycardia, and sweating).

Distension of abdomen with dull flank, guarding, tenderness and rigidity. Oliguria. Tachypnoea, respiratory distress and often cyanosis. Rupture of right lobe is more common than left lobe leading to haemoperitoneum. Occasionally can cause localized haematoma which may form an abscess. Bile leak from the injured site can lead to biliary peritonitis.

I Investigations Chest X-ray to look for rib fractures. US abdomen, FAST (Focused assessment with sonography for trauma) CT scan of chest and abdomen (CT is ideal). Diagnostic peritoneal lavage: 10 ml gross blood on initial aspiration, >1,00,000 cu mm RBCs, >500 WBCs, and presence of enteric contents suggest positive DPL. Diagnostic peritoneal aspiration only can also be done. Hb¾, PCV, blood grouping and crossmatching. Adequate amount of blood (5-10-15 bottles of blood) must be kept ready for transfusion, i.e. requires massive transfusion. Arterial blood gas analysis. Coagulation profile. Thromboelastography (TEG) is dynamic form of assessing the coagulation status on table. Arteriography to visualise the bleeding branch/vessel in the liver and embolisation can be tried.

I Treatment Initial resuscitation using ABC (Airway; Breathing; Circulation) of acute critical care then further management is done depends on eventual evaluation and liver injury grade. General measures IV fluids, blood transfusion (massive), FFP. Have both central venous access, and peripheral venous access. Bladder catheterisation has to be done to measure the urine output. Factor Vl/-proconvertin (SPCA-0.6 mg/ml up to 4.8 mg); very effective, makes INR normal; but very expensive. Initial conservative nonoperative management It is done in-nonprogressive liver injuries in patients who are haemodynamically stable, low grade (1-111) liver injury, need of less than 2 units of transfusion, without peritoneal signs, normal mental status. However, CT abdomen (absence of extravasation of contrast during arterial phase can be treated nonoperatively) and repeat CT or regular ultrasound follow-up is a must. Replacement of lost blood ; prevention of sepsis; regular monitoring by haematocrit, liver function tests, prothrombin time are needed.

Angiographic embolisation increases the success rate of nonoperative therapy. Intensive care unit (ICU) management for 2-5 days; repeat CT scan after 5 days; bedrest to be continued; patient can have normal activity only after 3 months.

T

CT grading of liver injury 1994-Association of American Society for Trauma (AAST)

Grade and type

Injury

I- Haematoma - Laceration

Subcapsular haematoma less than 10% surface area. Capsular laceration less than 1 cm in depth. Subcapsular haematoma 10-50%; intraparenchymal less than 10 cm diameter. Capsular laceration 1-3 cm in depth; less than 10 cm length. Subcapsular haematoma more than 50%; intraparenchymal more than 1Ocm diameterexpanding. Capsular laceration >3 cm in depth. Parenchymal disruption 25-75% hepatic lobe; 1-3 Couinaud segments. Parenchymal disruption more than 75% or more than 3 Couinaud segments. Major hepatic veins or retrohepatic venacaval injuries. Hepatic avulsion.

II - Haematoma - Laceration

Ill - Haematoma - Laceration

IV - Laceration V- Laceration - Vascular

VI - Vascular Note:

Mervis et al. proposedto add thickness of subcapsular hematoma ingrading system as-3 cm. When at any time patient's condition worsens, he should be taken up for laparotomy. Success rate of nonoperative treatment is 85% in grade 1-111 injuries; 40% in grade IV- VI injuries. It is the associated injuries which decides the success. Intervention radiology methods like angioembolisation through hepatic artery are used especially when there is pseudoaneurysm. But life-threatening hepatic necrosis and gallbladder necrosis can occur. Gallbladder necrosis warrants cholecystecto my.

Specific treatment Push (direct compression); plug (plugging the deep track injuries using silicone tube or SB tube); Pringle's manoeuvre; pack (liver wound is directly packed with mop).

B Phase I: Laparotomy control of bleeding by various methods, closure ' with pack. Phase II: Resuscitation, managing metabolic insult (hypothermia with temperature 3.5 mg%, serum albumin 500 ml, anaemia, diabetes.

I PYOGENIC LIVER ABSCESS I Aetiology 1. Biliary sepsis 35%; commonest route. , Empyema gallbladder; Cholangitis. ,. After biliary tract surgery; Instrumentation. ,. Stone disease, Caroli's disease, biliary ascariasis, biliary enteric anastomosis.

2. Portal vein sepsis 20%: ,. Appendicitis 2 %; Diverticulitis. ,.

Inflammatory bowel disease, pancreatitis, perforation, PIO, colorectal carcinoma. ,. Omphalitis in newborn . 3. Distant infections (through hepatic artery 15 %): ,. Pneumonia; Upper UTI. ,. Endocarditis, osteomyelitis, bacteraemia.

Due to laminar blood flow right lobe (75%) is commonly involved; left lobe (20%), caudate lobe (5%) are also often involved. Usually solitary---60%; occasionally it can be bilobar and multiple. Cavity contains pus with virulent organisms. Usually abscess is acute. In cryptogenic type chronic presentation is known to occur. Ascites and splenomegaly is not common. It is more common in diabetics. Male to female ratio is 1:1. It is more common in old people after 55 years of age. Blood culture commonly shows positive for bacteria.

I Features Pain in the right hypochondrium---60%. High fever, with rigors---90%. Weight loss. Jaundice-occasionally---20%. lntercostal tenderness. Tender, soft liver-60%. Features of toxicity. Constitutional symptoms like malaise, lethargy, vomiting. Diagnosis: ,. Ultrasound abdomen, CT scan. Sensitivity is 90% fo r USG; 97% for contrast CT scan. ,. LFT, total count. ,. Ultrasound guided aspiration of pus after controlling PT. ,. Chest X-ray shows elevated diaphragm often with right sided pleural effusion. ,. Blood culture is very relevant. Differential diagnosis: Amoebic liver abscess, hydatid cyst, subphrenic abscess. Treatment: ,. Systemic antibiotics- combination of third generati on cephalosporins and metronidazole.

,. Ultrasound guided aspiration/pigtail catheter-Percutaneous drainage is the treatment of choice at present.

Some men are born great, some achieve greatness, and some have greatness thrust upon them. -William Shakespeare

593

594

Drainage tube/catheter are placed under US/CT guidance into the liver abscess. Pus should be sent for culture and sensitivity. Follow-up USG, LFT, assessment of quantity of daily drainage-should be done to assess the response. 75% of pyogenic abscess is drained percutaneously. Percutaneous aspiration without drainage tu be placement is also used; but repeated guided aspirations are required; otherwise success rate is similar to drainage. ,. Occasionally, open drainage is required. Open drainage is indicated in recurrent abscess, failure of percutaneous drainage, large abscess of size more than 5 cm. Open drainage is becoming less common at present; but in selected patients it may be a life-saving essential therapeutic modality. ,. Treating the primary cause is very essential. Complications: ,. Septicaemia, liver failure; Rarely rupture and peritonitis can occur. , Klebsiella hepatic abscess can cause dangerous endogenous endophthalmitis commonly in diabetic patients impairin g vision.

I PORTAL PYAEMIA (PYLEPHLEBITIS) It usually follows after severe infection of areas drained by portal vein. Causes: Appendicitis; Diverticulitis; Any severe abdominal sepsis. Presently it is becoming rare because of availability of good effective antibiotics. Infective thrombus in the vein (draining the infective area) J, It dislodges as infective emboli J, Reaches the liver J, Causes multiple abscesses in liver parenchyma J, Pylephlebitis. E. coli is the most common organism. Often staphylococci, anaerobes, or combined infections may be involved. Note: • Gas and thrombus in portal vein is diagnostic of portal pyaemia. • Blood culture is a useful investigation.

B High fever with rigors Toxicity Drowsiness, jaundice

I Treatment Antibiotics-. Combination of third generation cephalosporin + aminoglycoside, ceftriaxone sodium, cefoperazone, ceftazidime, amikacin, tobramycin, metronidazole.

IV fluids, blood transfusion, FFP. Ventilator support. Mortality is very high and usually patients die of hepatic failure, septicaemia, MODS. It is better to prevent portal pyaemia by prior good antibiotics in suspected cases. Treatment of primary cause is very important.

I HYDATID CYST OF LIVER Word meaning is 'dew drop' (Latin). In Greek it means 'watery vesicle'. Echinococcus means 'hedge hog berry' in Greek. Caused by Echinococcus granu/osus (EG) , dog tape worm, a parasite. Life cycle: Infected offal of sheep J, Eaten by the dog (definitive host) J, EG released, develops in the dog's intestine into a parasite of 1 cm long with a head and three segments, last of which contains about 500 ova. J, Ova expelled from the dog's intestine to grass and vegetables J, Eggs are ingested by sheep, cattle or human beings (intermediate host) J, Through portal vein- liver-larva form J, Hydatid cysts (70% in liver). It takes few years to evolve into a complete hydatid cyst. Most commonly involved segment is segment Vll-27%. Commonly right lobe-66%; both lobes in 16% and only left lobe is involved in 17%.

I Pathology It has got three layers: 1. Adventitia (pseudocyst) is an inseparable fibrous tissue due to reaction of the liver to the parasite. 2. Laminated membrane (ectocyst), formed by the parasite itself is whitish, elastic, containing hydatid fluid, which can be peeled off readily from the adventitia. Germinal layer Brood capsule Protoscolex

, -~ ::...---1-- Brood capsule Daughter cyst

Fig. 11.15: Anatomy of hydatid cyst.

3. Germinal epithelium is the only living part, lining the cyst (endocyst). This layer secretes hydatid fluid, brood capsules with sea/ices (heads of future worms) .

Jaundice and pain. Features of anaphylaxis. Discomfort in right upper quadrant area; dyspepsia; hydatid cachexia in children; weight loss; fatigue; vomiting. Occasionally splenomegaly, pleural effusion, cholangitis, allergic asthma, fever. Came/latte sign: Following intrabiliary rupture, gas enters into cyst causing partial collapse of the cyst wall.

B Figs. 11 .16A and B: Hydatid cyst of the liver-typical look.

B

DIFFERENTIAL DIAGNOSIS

Hepatoma L.-

Amoebic liver abscess

- -

Cystic disease of the liver

- -

- -

_J

B

FEATURES OF HYDATID FLUID

Anaphylaxis; Rupture Obstructive jaundice-ERCP sphincterotomy may be needed Infection; Calcification; Liver failure

• Clear High specific gravity (1.005-1.009) • Shows hooklets and scolices

Once brood capsules disintegrate, it grows into daughter cysts. Note: • Liver is commonly involved (65%); lungs (25%); muscles (5%); bones (3%); rarely kidneys, brain, spleen. • Pressure in the cyst is very high 70 mm ot H20; so leak and anaphylaxis becomes rapid when punctured. • Separated laminated membrane fall in the cyst causing " water lily sign" which is more often observed in lung hydatid but can be seen in liver also. • ERCP is done in presence of hydatid debris in CBD, biliary cyst communication lasting more than 3 weeks, presence of jaundice. • PAIR becoming popular; risk of anaphylaxis should be taken care off. • Radical surgery is not commonly advocated; but pericystectomy is very effective therapy. • Liver resection is occasionally required. • Laparoscopic approach gained acceptance for hydatid cyst of the liver.

I Course of the Disease The parasite may die and cyst eventually may get calcified. Commonly cyst enlarges and is palpable per abdomen. It may cause complications like jaundice due to pressure over biliary tree. Rupture into the peritoneal cavity causes anaphylactic reaction which may cause life-threatening shock, requiring proper management with steroids. Rupture into biliary channels is commonest (60%). Rupture into bowel, pleural cavity can occur. Secondary infection causing suppuration and septicaemia. Secondary cysts in the lung, spleen, mesentery, retroperito· neum and other organs can occur. Hepatic dysfunction. Disseminated abdominal hydatidosis can occur after silent rupture.

_l~l_ nv_e_s_ ti=ga_t_io_n_s___________ Ultrasound is diagnostic. It reveals rosettes of daughter cysts, double contoured membrane of the cyst due to detachment of the cyst membranes, and calcification of cyst wall. Intraoperative ultrasound (IOUS) is very useful tool.

Type 1 Type 2 Type 3 Type 4 I Tvpe 5 -

L..

Pure fluid collection Fluid collection with split wall (Membrane separation) Fluid collection with septa Heterogeneous appearance (hypo + hyperechoic) Reflecting thick walls (completely calcified)

--

--

--

---

X-ray often shows calcification. CT scan abdomen is more accurate in identifying cyst characteristics- cart wheel like- multivesicular rosette like. Primary serological tests-ELISA; indirect haemagglutination test; latex agglutination test; immunofluorescence antibody test; immunoelectrophoresis. 80-95% sensitivity for liver hydatid.

I Clinical Features Asymptomatic palpable liver with classical thrill (hydatid thrill) elicited by three-finger test.

Fig. 11.17: CT scan of hydatid disease of the liver.

Today's impossibilites are tomorrow's miracle.

595

- - - - - - - - - - - - --

596

Contraindications Large cysts; Honeycomb cysts (with septae) • Infected cysts; Calcified cysts Pregnancy. -- -- --

---,

I L

Fig. 11.18: MRI picture of multiple hydatid cyst of liver-MRI is very useful investigation in hydatid cyst of liver.

Secondary laboratory tests-Detection of precipitation linearc 5 in immunoelectrophoresis is most specific and virtually diagnostic of hydatid disease; immunoblotting; polymerase chain reaction (PCR). More specific, very useful in extrahepatic hydatid disease and calcified non-fertile liver hydatid. Liver function tests-altered in 20% cases. Casoni's test (intradermal test-75% sensitive); complement fixation test-historical interest. MRI when there is jaundice to visualise biliary tree and its relation to hydatid cyst; to find out cystobiliary communication; biliary hydatids in bile duct and hepatic ducts. ERCP can also be done to find out the communications. Other method to find out the cystobiliary communications is intraoperative cholangiogram. ERCP to find out biliary communications but not as useful as MRI. MRI is superior to CT also. Aspiration of the cyst should not be done due to risk of anaphylaxis but presently PAIR (Puncture-Aspiration-lnjectionReaspiration) is done effectively.

Type - CL - Active; unilocular; no cyst wall; early stage; not fertile Type - CE 1 -Active; cyst wall present; hydatid sand present; fertile Type - CE 2 - Active; multivesicular rosette like cyst wall; fertile Type-CE 3 - Transitional; detaching laminated membrane; water-lily sign; beginning of degeneration Type - CE 4 - Inactive; degenerative contents; no daughter cysts; not fertile Type - CE 5 - Inactive; thick calcified wall; not fertile WHO-IWGE USG classificatioff. Group 1: Active-cyst >2 cm; fertile. , Group 2'. Transitional group - may contain viable scolices. Group 3: Inactive - degenerated, calcified, non viable cyst.

I I

I Treatment

Drugs used are: ,. Albendazole 4-week cycles with 2 weeks drug free interval. It is ovicidal/larvicidal/ vermicidal. Commonly used. 3 such cycles are used; dose is 400 mg twice daily/1O mg/kg/day. Albendazole may alter LFT, cause fever, alopecia, leucopenia; it is contraindicated in pregnancy. Drug therapy is less effective in hydatids of bone, brain and eye. ,. Praziquantel-60 mg/kg along with albendazole for 2 weeks. , Mebendazole-600 mg daily for 4 weeks-not used now. ,. Side effects of drugs: Altered liver enzymes, alopecia, bone marrow suppression leading into aplastic anemia.

PAIR (Puncture-Aspiration-lnjectionReaspiration -Percutaneous! Done 1980) Technique of PAIR Done under US/CT guidance. Under local anaesthesia cyst is punctu red using a cholangiography 22 gauge needle through thickest route/part of cyst wall. Cyst is entered through non-dependent wall and 50% of fluid is aspirated. All mu ltiple/daughter cysts are aspirated. Radiopaque dye is injected to see if any communications are present. Scolicidal agents-15-20% hypertonic saline is injected into the cyst. After 20 minutes reaspiration is done. A sclerosant is injected. If cyst is 6 cm or more, a drainage catheter is placed for 24 hours for complete drainage and later alcohol sclerosant is injected-PAI RD. Double-puncture aspiration and injection (D-PAI) or modified PAIR/PEVAC (Percutaneous EV Acuation of Cyst) are other procedures done. PAIR has gained wide acceptance as it is safe, less invasive, and easier to do, with low morbidity and mortality. Complications and results are same as open surgery. Indications for PAIR

R,sults and problems of PAIR

• Inoperable patients

• Complication rate-1D-40%

• Patients who refuse surgery

• Mortality rate-0.9-2.5%

• CL, CE 1, CE 2 and CE 3 types/Gharbi type 1 and 2

• Fever-35%-disappears in 72 hours

• Relapse cysts

• Anaphylaxis-0.Hl.2%Same as open surgery-but drugs should be kept ready for anaphylaxis

Drugs Indications for drug therapy 10 days prior to intervention and to continue it for 1 month (albendazole) to 3 months after the intervention Inoperable cysts; Multiple or multiorgan cysts Recurrent hydatids; Surgically unfit patients Cysts in lungs ---~

• I

L

_J

• Infected cysts • lnfection-10% well • In pregnant women; children controlled by antibiotics less than 3 years • Cysts more than 5 cm in different liver segments

• Local recurrences- 4% Repeat PAIR can be done

Contd...

Contd... Contraindications for PAIR

• • • •

Inaccessible cysts; Hyperechogenic cysts Cysts with multiple septae-honeycomb cysts Communicating cysts to bile duct Calcified cysts; Cysts in the lung

I Surgery Surgery is still the choice and gold standard therapy for hydatid disease. The abdomen is opened, and the peritoneal cavity is packed with mops [black or coloured mops are used to identify white scolices clearly so as to pick up all and prevent any spillage]. Fluid from the cyst is aspirated and scolicidal agents [cetrimide, chlorohexidine, alcohol, hypertonic saline (15-20%), 10% povidone iodine or H202] are injected into the cyst cavity (formalin should not be used). Hypertonic saline should be left within the cavity for 15-20 minutes to have effective scolicidal effect.

Laparoscopic pericystectomy is becoming more popular. Contraindications are deeply situated cyst, densely adherent cyst, and inaccessible cysts; more than 3 cysts; calcified cysts and cysts in other organs. Main problem with laparoscopic pericystectomy is spillage and difficulty in preventing it. Liver resection-only occasionally segmental or hemihepatectomy is done.

B Cetrimide-can cause acidosis Alcohol 80%-can cause cholangitis Hypertonic saline (15%)-hypernatraemia Sodium hypochlorite-hypernatraemia Hydrogen peroxide In cases with biliary communication only hypertonic saline (15-20%) is used (not other agents) Principles in surgical trsatmsnt

• Removal of all infective components of the cysts • The avoidance of spillage of cyst • Management of communications • Management of the residual cavity • Minimizing the risks and complications

Types of surgsriss

Complications of surgery

Conservative

Bile leakminor/major leak Sepsis, cholangitis Recurrence-3-10% Mortality6-10%

• Laparotomy Open aspiration Evacuation of cyst injection of scolicidal agent residual cavity management by marsu pialization/ capitonage/omentoplasty/ introflexion • Partial pericystectomy • Laparoscopic approach using perforator-grinderaspi rator apparatus Radical surgical procedures

• Pericystectomy or lobectomy or hepatectomy • Surgical treatment for communications

Figs. 11.19A to D: On table finding of hydatid cyst of liver. Black mops are used to identify and isolate scolices. Note the cavity containing bile suggestive of biliary communication.

Detection of cystobiliary communications is very crucial as it may cause caustic sclerosing cholangitis when scolicidal agent like formalin is used. Communicating openings may be single or multiple. Cyst more than 10 cm is likely to have cystobiliary communications. Often clinical features of communications may not be present. Preoperativelythere may be recurrent cholangitis; dilated bile duct. Factors important are-its size; number; site; involvement of hepatic/bile ducts; liver dysfunction. Finding of bile stained cyst on table during aspiration is highly suggestive of communication. White mops soaked with hypertonic saline are kept in the cyst cavity and gallbladder is gently squeezed to see for the bile staining of the mop in the cavity which confirms communication.

Procedures used to correct the cystobiliary communications and to obliterate the cavity are: Suturing of the communication-simple suture using vicryl/ PDS suture with T tube drain of bile duct. Pericystectomy (Pericystectomy is done by peeling off the cyst wall and abdomen is closed with or without a drain); marsupialization. Tube drainage of the cavity; capitonnage-spira l suturing of the bottom of the cavity upward from base of cavity to the edge of the cyst wall; introflexion-inverting the rim of the cyst edge without apposition; omentoplasty. Internal drainage procedures like choledochojejunostomy; transduodenal sphincteroplasty; Roux-en Y cysto/intracystic hepatico-jejunostomy; Roux-en-Y hepatico-jejunostomy. In cystobiliary communication (fistula), cystobiliary disconnection is achieved anatomically between cyst and fistula and a multiperforated transparenchymal drain is placed into the fistula through the cyst and another mu lti perforated drain is

I don't dawdle. I'm a surgeon. I make an incision, do what needs to be done and sew up the wound. There is a beginning, a middle and an end. - Richard Sezler

597

0

::c

> -a -4

m

.... .... :x,

r-

e' CD

....

...= Q)

Cl

Cl)

0

placed into the cyst cavity. AT tube drain is placed into the bile duct (perdomo procedure) to achieve complete external drainage with facilitation of closure of the communication. Bipolar drainage-a drain is placed to cyst cavity and aT tube is placed into common bile duct. Reconstructive procedure like pericysto-jejunostomy, bile duct repair. ERCP sphincterotomy.

B It is a misnomer as it is a benign condition It is caused by Echinococcus multilocularis (Alveolaris). It presents with multiple small cysts in both lobes of the liver, all over It is difficult to treat, and mimics clinically and prognosis-wise to j malignancy, hence the name They die of liver failure

------

I ACTINOMYCOSIS OF LIVER Actinomyces israelii reaches the liver-via portal vein from ceacum (60%) or via hepatic artery from distant focus-from faciocervical region-30%. Disease progresses gradually and slowly to destroy the liver tissue causing multiple actinomycotic abscesses which produce a typical 'honeycomb liver.' Complications: Secondary infection; Liver failure. Investigations ,, Liver function tests. , Ultrasound guided aspiration and staining of pus shows 'sun-rays' appearance. Treatment Penicillins for 3-6 months.

LIVER TUMOURS

I BENIGN TUMOURS OF THE LIVER It is two times common than malignant liver tumour It is seen in 20% of population Haemangioma is the most common benign tumour CT and MRI are the essential investigations MRI shows 95% accuracy Diagnostic accuracy of liver biopsy is low-40% Hepatic adenoma is potentially malignant FNH and haemangiomas are not premalignant Primary (HCC) tumour or secondaries are differential diagnosis; AFP and CEA are helpful to differentiate

1. Haemangiomas They are the commonest benign tumour of the liver. It is usually solitary but can be multicentric. Compressibility of tumour is diagnostic. It is common in females (3:1) in 5th decade. Commonly they are asymptomatic (85%). They can occur in right and left lobes. Calcification is seen in 10% cases.

Fig. 11 .20: Solitary benign cyst of the liver. It is a rare entity. Polycystic liver also can occur. Polycystic liver is associated with polycystic kidney disease. Condition is a rare differential diagnosis for hydatid cyst, hepatoma, and solitary secondary.

It is congenital in origin and enlarges by ectasia. Involution of a large haemangioma can occur forming a dense fibrosis which mimics metastasis. It may compress over gastroduodenum to cause abdominal pain and vomiting. Acute thrombosis with sudden onset of pain and fever may mimic liver abscess; but symptoms subside gradually in 3-4 weeks. Spontaneous rupture is very rare. Differential diagnoses-. HCC, vascular solitary secondaries, hepatic adenoma, liver abscess. In children it is 10-15% of liver tumours. They are multifocal, often involves other organs also. Large one can cause AV shunting leading to CCF. Large childhood hepatic haemangiomas have got 70% mortality due to rupture, DIC, thrombocytopenia. It is confirmed by MRI. It is treated by embolisation, radiotherapy and often resection. Cavernous haemangioma, consumption coagulopathy, thrombocytopenia is called as Kasabach-Merritt syndrome. LFT, AFP and CEA are normal. Complications: Bleeding rupture; Thrombosis; DIC; Infection Complication usually occurs if haemangioma is more than 8 cm in size. Needle aspiration and biopsy are contraindicated. US and CT scan are confirmative. Angiogram may be needed if surgical intervention is planned. MRI is 100% sensitive and shows 97% accuracy. Giant haemangioma is of size >5 cm. Treatment: Prior radiotherapy is given to reduce the size and then hemihepatectomy is done. Surgery is done when size is large, symptomatic, impending rupture or any other complications. Enucleation or anatomical resection with inflow control is needed. Note:

It has got very little or no malignant potential.

2. Hepatic adenomas They are common in females (10:1 ). They present as a solitary nodular lesions in the liver.

It is said to be due to use of oral contraceptive pills (OCPs). It is uncommon in males, but can occur with androgen use. It is relatively rare compared to FNH and haemangioma. It is commonly solitary; but 20% of cases can be multiple; situation where >10 adenomas are present is termed as adenomatosis. Multiple adenomas are not related to OCP intake and not more common in females. Multiple type may occur in glycogen storage disease type I. Upper abdominal pain (65%) is the commonest presentation. AFP is normal but rises if there is malignant transformation. They might turn into malignancy, hence resection is advised. US, CT scan are diagnostic but angiography is needed prior to resection, as it is vascular. MRI is ideal tool. Haemorrhage is more common . Hepatic adenoma cells are larger, contain glycogen and lipids. They are devoid of bile ducts whereas FNH contains bile duct components. Surgical resection is the needed treatment. Limited resection is needed. It is better to do resection prior to pregnancy. In adenomatosis either hemihepatectomy or transplantation may be required. OCP should be stopped.

It also should be differentiated from HCC, hepatic adenoma, nodular regenerative hyperplasia of liver (NRH). Vague pain abdomen may be the presentation. Rupture, bleeding are very rare. Histologically it can be typical ( with central scar') or atypical ( without central scar')- 15%. Usually it does not require any treatment. OCP should be stopped. Note:

Von Meyenburg's complexes are benign liver malformations that originate from embryonic bileducts that fail to involutewith cystic dilatation. It mimics metastatic liver disease. MRI is essential to diagnose.

I PRIMARY MALIGNANT TUMOURS OF THE LIVER 1. Hepatocellular carcinoma/Hepatoma/HCC (80%). 2. Cholangiocarcinoma (20%). 3. Hepatoblastoma in infants and children. Hepatoblastoma occurs within 2 years of life. It is most common primary malignant tumour of liver in children. It is common in male child. It is derived from fetal or embryonic hepatocytes. Serum AFP is elevated in 90% of cases. CT scan shows vascular mass with speckled calcification. It is highly sensitive to chemotherapy (vincristine, doxorubicin, 5FU). If it is resectable, it is the initial choice of therapy. Liver transplantation is also beneficial.

I HEPATOCELLULAR CARCINOMA (HEPATOMA)

B Rupture of adenomas-50% If there is possibility of turning into malignancy {large adenomas)

I

Note:

Kuptter cells are not present in hepatic adenomas.

3. Focal nodular hyperplasia (FNH) It is a benign condition of unknown aetiology, seen in females showing focal overgrowth of functioning liver tissue with fibrous stroma support. It is hyperplasia of liver containing all components of liver in disorganized pattern. It is 8 times more common in females. It contains hepatic cells as well as Kupffer cells which is characteristic. It is 2nd most common benign tumour. Usually it presents as solitary nodule. A sulphur colloid liver scan is diagnostic, shows 'hot spot' with a spoke wheel pattern-85%. It is a harmless condition. CT scan shows central scar with stellate distribution of the blood vessels, Kupffer cell activity, without calcifications with homogeneous mass lesion in arterial phase enhancement. Telangiectatic FNH and hyperplastic with adenomatous FNH are non-classic forms of FNH, which are 20% common and more common in men. It is totally benign tumour; but often difficult to differentiate from fibrolamellar type of HCC. AFP is normal.

Hepatocellular carcinoma (HCC) is a pri mary malignant tumour of the liver with hepatocellular differentiation. Its incidence is increasing; becoming 5th most common malignancy worldwide. HCC is the commonest primary liver malignancy (90%). But in the liver, secondaries is the most common malignancy (20 times more common than primary). It is common in cirrhotics and hepatitis B and hepatitis C virus infection. It is common in Japan, Mozambique, South East Asia, tropical Africa, Taiwan. In Mozambique it is often seen in younger age group-below 30 years. Male to female ratio is 4:1. It is usually unicentric but occasionally can be multicentric. Right lobe is commonly involved.

Fig. 11 .21: Hepatocellular carcinoma (HCC/hepatoma) is usually solitary, common in right lobe, attains large size.

Every Job is self portrait of the person who did it.

599

600

I Aetiology Aflatoxin B1, a product of fungus aspergillus. It is powerful carcinogen. Hepatitis Band hepatitis Cvirus infection. It is more common in individuals who have chronic positive status for HBs Ag and chronic carriers. Vertical transmission of virus at birth raises HCC rate. In Europe, Japan, USA, HCV infection is more common cause. It is not necessary to develop cirrhosis to cause HCC. It is the infection which is the cause. Alcoholic cirrhosis. It is co-carcinogen. Clonarchis sinensis infestation; Smoking. Haemochromatosis, a 1 antitrypsin deficiency. Hepatic adenoma-potentially malignant. Environment related chemicals like DDT, nitrite and nitraterelated food products; trichloroethylene (dry cleaning solvents), biphenyls, carbon tetrachloride, herbicides , solvents like dioxane. Tannins, griseofulvin, bush tea (pyrrolizidine), rice toxins. Anabolic steroids, polyvinyl chloride. Non alcoholic fatty liver disease (NAFLD), diabetes and obesity. Primary biliary cirrhosis; Wilson's disease.

I Pathology Gross It is highly vascular with indistinct margin. Often it is well demarcated by fibrous tissue. Haemorrhage and necrosis are common. Hanging type of tumour-tumour attached to normal liver by a small vascular stalk- always very well resectable. Pushing type of tumour- large, well demarcated, displaces normal vasculature- resectable. Infiltrative type of tumour- indistinct, infiltrative-often difficult to resect.

Unifocal - Expanding with sharp demarcation-portal vein invasion is high - Pedunculated- type 1 is intrahepatic; type 2 is extrahepatic - Spreading type-without a clear demarcation (Engel's type) Multifocal-synchronous Indeterminate- different features in different parts of the liver Diffuse Okuda classification-Okuda staging is different Multifocal; Indeterm inate; Spreading; Expanding NCCN classification Nodular; Massive; Diffuse Note: • Small HCC nodule is less than 2 cm in size. • PV extension as tumour thrombosis indicates inoperability and cause portal hypertension. • Infiltration into hepatic veins is not common but when it occurs, tumour thrombus extends rapidly into IVC and right atrium. • Invasion into biliary system causes haemobiliaand obstructive jaundice. • Lymphatic spread occurs to hilar nodes, celiac nodes, pericardia! nodes, paracaval nodes.

• • • •

Local infiltration into diaphragm and adjacent organs can occur. Blood spread occurs to lungs, bones, meninges, kidney and adrenals. Dysplastic nodules and hepatic adenomas are premalignant conditions. Histologically it can be well/moderate or poorly differentiated tumour. • Variantsof HCC are-fibrolamellar(good prognosis); mixed hepatocellular cholangiocellular (poor); clear cell variant (better); giant cell variant; (bad) sarcomatoid variant (carcinosarcoma).

I Fibrolamellar Variant of HCC (FL HCC) FL HCC occurs in younger age group. Incidence is equal in both sexes-5% common. It does not show any elevated levels of serum AFP. Tumour marker for FL HCC type is increase in serum vitamin 8 12 binding protein and increase in neurotensin levels. It is not related to viral hepatitis or cirrhosis. Left lobe is commonly involved. It involves lymph nodes more commonly than HCC. CT scan shows characteristic central scarring. Fibrous stromas with thin hyaline bands are typical. Sheets of well-differentiated hepatocytes are seen which are sandwiched between collagen and fibroblasts. It is fairly resectable (50-75%) with better prognosis than HCC.

I Staging of HCC Note:

There are many more staging systems; students don't require remembering or memorizing these staging systems. Staging is based on tumour factor and liver function.

T

Barcelona Clinic Liver Cancer (BCLC) staging systemcurrently used

Ve,yearly stage 0

Early stage A

Intermediate Advanced stage B stage C

Terminal stageO

Child Pugh A

Child Pugh A-8

Child Pu gh A-8

Child Pugh C

Child Pugh A-8

Single nodule 1-3 nodule 1 cm in size in ultrasound and AFP >20 nglmL should trigger to evaluation for HCC. • Tumour markers like GPC3, HSP70 and GS are helpful to identify HCC from high grade dysplasia.

Contrast MRI/CT scan 1- 2 weeks following intrahepatic infusion of lipiodol (ethiodized oil emulsion 5-15 ml) with contrast agent through hepatic artery is very useful. Such Lipiodol enhanced CT/MRI even though is ideal and gold standard, but not practiced commonly; during selective angiogram lipiodol is injected into the hepatic artery which is taken up by the tumour, follow-up CT can be done to assess tumour in 2-4 weeks.

MRI-T2 weighted studies are useful for small HCC. MR angiography is also done to see tumour thrombus in portal vein, hepatic vein and IVG. Ascitic tap when ascites is present for cytology. Laparoscopic evaluation and laparoscopic US is useful for proper assessment of the tumour. Investigations in relation to hepatitis B and hepatitis C virus infections. Metastatic work up-HRCT of chest is essential. FOG-PET scan and bone scans are useful in detecting the early metastatic diseases. Surveillance screening for high-risk group like cirrhosis is done using USG once in 6 months and AFP.

I Treatment Definitive Treatment When limited to one lobe, hemihepatectomy is done (Removal of 70% liver is compatible with life). Laparoscopic resection is becoming popular. It is done only to Child's grade A and favourable Child's B group patients. It is the treatment of choice for operable HCC in non-cirrhotic patients. There is no regenerative capacity in cirrhotic patients with poor coagulation status, portal hypertension, varices, and ascites.

Figs. 11 .23A to D: Hepatocellular carcinoma-CT and resected specimen.

In cirrhotic patients with HCC, total hepatectomywith orthotopic liver transplantation is required. Now it is found that even resection candidates will do better by transplantation than resection. But waiting period for transplantation is long. To bridge this waiting period for transplantation radiofrequency ablation, transarterial embolisation, ethanol/acetic acid injections are used.

B • • • • •

Patient who is not a candidate for resection Tumour less or equal to 5 cm Tumour less than 3 in number Tumour without portal/hepatic vein invasion Tumour without extrahepatic spread

_J

Note:

• 30%of liver tissueor at least two segments should be retained inliver resection when underlying liver is normal: hepatic reserve is assessed by indocyanine green clearance test and CT or MR volumetry. • Resectability is based on-number and anatom ical location of the tumour, hepatic reserve, liver function (normal bilirubin, HVPG1,00,000/, sufficient functional liver remnant (FLR). • MELD (Model for End Stage Liver Disease) scoreis used for resectability using creatinine, bilirubin, PT- INR. • Milan's criteria are tumour less than 5 cmor number less than 3 with each less than 3 cm. • University of California, San Francisco criteria-single tumour 3 cm and not >3 nodules). Ethanol injection is minimally invasive, can be injected using fine needle, and is cheaper. Local recurrence is lower with acetic acid injection than ethanol. But radiofrequency ablation has got better result than ethanol/acetic acid injection. RFA is costly and not available in many centers. Trans -arterial chemotherapy (TAC) using Ad riamycin/ cisplatin/mitomycin through gastroduodenal artery. It is often given along with lipiodol to make dru gs to stay longer time in the liver tissue so that better results are achieved. This regional chemotherapy is much more successful in HCC. Trans-arterial embolisation (TAE) using gelfoam, microspheres, gelatin sponge, or chemoembolisation (TACE). TACE is combined TAE with chemotherapy using lipiodol, doxorubicin, mitomycin and cisplatin. TACE can be conventional or Drug eluted beads (particles) TACE [DEB-TACE]. Microwave or cryoablations similar to RFA. As it is considered that tumour tissue excl usively gets its blood sup ply from hepatic artery, ligation of hepatic artery can be a good palliation (to achieve tumour necrosis) for HCC. Normal tissue will still have its blood supply from portal vein. Transarterial 1131 lipiodol, Yttrium 90 microspheres. Targeted Iodine 131 with lipiodol injection into hepatic artery is used as targeted RT as adjuvant therapy after liver resection. It is also makes disappearance of the portal vein thrombosis in HCC and used as neoadjuvant therapy prior to liver resection.

B Large primary tumour-downstaging/downsizing may make resection possible. • Transarterial chemoembolisation (TACE) • Chemotherapy and radiotherapy (SBRT-stereotactic body RT) • Hepatic artery infusion of chemotherapy (HAI) • Radioimmunotherapy • Fractioned radiotherapy Transarterial 90Y microspheres (TARE-transarterial radioem. bolisation)

• I

B

• Surgical resection, only 20% have resectable liver Liver transplantation • Radiofrequency ablation • Percutaneous ethanol/acetic acid injection • Transarterial embolisation/chemoembolisation • Microwave/cryoablation Transarterial radiotherapy • Transarterial Yttrium90 microspheres/1 131 lipiodol Hepatic artery ligation • Adjuvant systemic chemotherapy lmmunotherapy/hormone therapy/growth factors

The best bridge between despair and hope is a good night's sleep.- EJoseph Cassman

603

604

n:i

::I C

n:i

:ii: _rA

CCI

a:

C'-1

PET scan is done to identify occult extrahepatic metastases. Common aetiologies are aflatoxins, hepatitis B and hepatitis C virus infection, alcoholic cirrhosis, haemochromatosis, smoking, hepatic adenoma, clonorchis sinensis, polyvinyl chloride Unicentric and right lobe involvement is more common Fibrolamellar variant is common in left lobe, not related to hepatitis or cirrhosis without AFP level raise. There are increased serum vitamin B12 binding capacity and neurotensin levels Invasion to portal veins is common and it has got negative prognosis with lesser chances of resection or transplantation. It may cause portal vein thrombosis and so splenomegaly It can be multifocal/indeterminate/spreading/expanding-Okuda

I

I

classification

Presents as large, smooth, hard liver mass-later jaundice, fever, pain and tenderness, ascites and bruit over mass Spreads by lymphatics, blood and direct extension Mimics amoebic liver abscess, secondaries, hydatid cyst, polycystic Iive r disease LFT, CT scan, raised AFP, liver biopsy are the investigations

I

CEA is reliable indicator of spread or recurrence. Resection, chemotherapy, intra-arterial chemotherapy are therapeutic modalities. Contraindications for liver resection are-extrahepatic spread, nodal involvement along with primary, short disease free interval less than 1 year, four or more secondary deposits, bilobar spread, CEA more than 200 ng/dl, secondary more than 5 cm in size, involved histologic margin. Colorectal liver secondaries show good prognosis when secondary are solitary, and with absence of all poor prognostic indicators. 5 FU with irinotecan or oxaliplatin, bevacizumab (anti-vascular endothelial antibodies) are also useful.

• Liver mass, confirmed by CT/MRI and serum AFP >500 ng/dl is almost diagnostic and treatment can be started without tissue

diagnosis Liver biopsy is done (FNAC) only in patients with nondiagnostic level of AFP and not a candidate for curative surgery but only for palliation Hemihepatectomy is the treatment in early operable growth • Liver transplantation is the good surgical option Hepatic artery ligation/intra-arterial chemotherapy/chemo- I embolisation/percutaneous ethanol or acetic acid injection/ radiofrequency ablation/chemotherapy using adriamycin, carboplatin, gemcitabine-are palliative procedures

I

Fig. 11 .24: Secondaries in liver-solitary type from colonic primary.

I SECONDARIES IN THE LIVER It is the commonest malignant tumour in liver. The incidence of primary: secondary : : 1 : 20.

B In brain

I Causes Abdominal: Carcinoma in stomach, colon , pancreas, small bowel, kidney, abdominal oesophagus, rectum, and carcinoids. Extra-abdominal: Melanoma. Carcinoma breast, lung, t ho racic oesophagus, blad der, prostate. Testicular and adrenal tumours. Follicular carcinoma thyroid (FCT). Commonly secondaries in liver are multiple, multiple in one lobe or in both lobes. It can be solitary (rare).

I Classification Colorectal Colorectal carcinoma has got special interest in relation to its secondaries in liver. It carries good prognosis. Secondaries can be synchronous or metachronous. Resection of secondaries in liver often gives good outcome.

Neuroendocrine Gastrinoma, glucagonoma, somatostatinoma, nonfunctioning neuroendocrine tumours can cause liver secondaries. These tumours are slow growing. Main symptoms are often due to secreting peptides causing different clinical syndromes. Hormonal assay, CT abdomen, CT angiogram are needed. Hormone controlling therapies, chemotherapy and cytoreduction using liver resection gives good 5-year survival up to 75% in these patients.

Non-colorectal and Non-neuroendocrine These are aggressive primary tumour causing liver secondaries like from stomach, pancreas, small bowel, oesophagus, gallbladder, melanoma, urinary bladder and prostate. They carry poor prognosis. Treatment is chemotherapy. Early primary like-in stomach with isolated liver secondaries without extrahepatic spread can be benefited with liver resection. But overall prognosis is guarded.

I Other Classifications Precocious: Secondaries are presented/identified before primary is suspected-carcinoid, colorectal cancer.

Synchron~us: Both p~imary and secondaries are identified at the same time-carcinoma stomach. Metachronous. Secondaries develop much later may be long time after the treatment for primary-melanoma, breast carcinoma.

I Route of Spread a. b. c. d.

I

D_ittere~tial diagno_sis_:Mul~icentric hepa~oma; Macronodular ::irrhos,s; Polycystic liver disease, hydat1d cyst of liver.

I Investigations

--- --=- - - - - - - - - - -- - -- -

Primary is identified by-gastroscopy, colonoscopy, contrast X-rays, CT scan.

C")

:c ):I,,

m

:::D

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

Direct spread: Stomach, colon , gallbladder, bile ducts. Hepatic artery: Melanoma. Portal vein: Carcinoid tumours, other GIT malignancies. Lymphatic spread: Breast, lung.

C:

...
13 mm; presence of •cherry red spots' or •white nipple sign' (platelet fibrin plug on the summit of the varix), suggests rebleed. Endosonography is also useful. Liver biopsy to identify the aetiology once coagulation profile is corrected using FFP and vitamin K injection. Ascitic tap is useful; fluid is evaluated for cells, proteins. Serum ascitic albumin gradient (SAAG-serum albumin minus ascitic fluid albumin) if more than 1.1 suggests high gradient means portal hypertension.

guidance through left 9th intercostal space in midaxillary lineto reach the pulp of the spleen. Needle is connected o manometer to measure pressure. Water-soluble iodine dyeis injected under Carm guidance to get splenoportogram films to assess the portal system. Complications are - bleeding, infection. left shoulder pain.

I Management of Portal Hypertension a. General measures: ,. Anaemia to be corrected. , Nutrition supplementation. , lnj. vitamin K-10 mg IM for 5 days. , Blood and blood product transfusions as required. b. Specific measures: , Treatment of oesophageal varices. , Prevention of hepatic encephalopathy. , Treatment of ascites. , Measures to reduce portal pressure: - Surgeries-Portosystemic shunt. · Nonselective. - Selective. - TIPSS. - Drugs to reduce the portal pressure like propranolol, nadolol, isosorbide-5-mononitrate. r Liver transplantation.

OESOPHAGEAL VARICES May be asymptomatic. May present with haematemesis or melaena or as recurrent bleeding. Varices begin to bleed when portal pressure exceeds 12 mmHg. When present with severe haematemesis, patient shows features of shock. Mortality in bleeding varices is 25-30%. Factors related to variceal bleed are-portal venous pressure; gastro-oesophageal reflux causing ulceration; variceal size; variceal wall tension.

I Types of Varices 1. Oesophageal varices which is in the lower 1/3rd of the oesophagus, usually 3 or more in number, graded as I, II, Ill, IV (based on gastroscopic findings) (80%).

B

I. Minimal varices without luminal prolapse, visible on Valsalva, nontortuous. • Transient elastography ultrasound based-is non-invasive method to II. Moderate varices, with luminal prolapse and with minimal detect clinically significant portal hypertension. obscuring of 0-G junction-occupy

"'C

m

........

:ti

r
3 mg%.

ENDOSCOPIC RETROGRADE CHOLANGIOPANCREATOGRAPHY (ERCP)

This test is done to study function of the gallbladder. Patient is advised to have fat-free diet for 3 days. Previous night 6 tablets of iopanoic acid (Telepaque) is given orally. On the day of OGG, plain X-ray abdomen in erect posture is taken to visualise the gallbladder.

t

Fig. 12.5: Oral cholecystogram with smooth filling defect (Cystic duct stone).

Later, fatty meal is given and one more X-ray is taken to see the change in the size of the gallbladder (which should be less in size compared to the earlier film, as the gallbladder contracts on stimulation with fat-rich meal if it is functioning normally). Smooth filling defect signifies non-opaque stone. Contraindications: Patients with serum bilirubin >3 mg%; acute cholecystitis; vomiting. Note:

• One tablet of lopanoic acid is 500 mg.

INTRAVENOUS CHOLANGIOGRAM

Through a side viewing gastroduodenoscope, sphincter of Oddi is cannulated , and dye is injected. Biliary and pancreatic trees are visualised. It is done under C-arm guidance and sedation like midazolam or propofol anaesthesia. Patient is placed in prone position with the head turned towards right. After passing gastroduodenoscope, sphincter is identified and cannulated . Under visualisation 3 ml of water-soluble iodine contrast is injected into the bile duct and pancreatic duct. When cannula goes upwards beside vertebra, it signifies that it is in bile duct; and if cannula goes across the vertebra, it is in pancreatic duct. Indications (Figs. 12. 7A to E) , Malignancy-irregular filling defect. , Chronic pancreatitis-'chain-of-lakes' appearance. , Congenital anomalies-Stones. , Stricture of biliary tree-Choledochal cyst. , For sampling of biliary and pancreatic juices for analysis and cytology. , Brush biopsy from tumour site.

B

THERAPEUTIC USES

Extraction of stone from biliary duct Nasobiliary drainage Stenting of tumour in the CBD or in the pancreas Dilatation of the biliary stricture • Endoscopic papillotomy

_J

Complications , Pancreatitis; Cholangitis , Duodenal injury, perforation; Sphincter stenosis , Bleeding from pancreaticoduodenal artery. Relative contraindications: Acute pancreatitis; Previous gastrectomy.

PERCUTANEOUS TRANSHEPATIC CHOLANGIOGRAPHY (PTC) It is done in case of severe obstructive jaundice under the cover of antibiotics and after control of any bleeding tendency-by giving injection vitamin K or FFP transfusion.

B Fig. 12.6: Cholecystocholangiogram. Note the dilated CBD, complete block in lower CBD, dilated biliary radicles.

Failure of ERCP; High biliary strictures Klatskin tumour; Stenting in high tumours I High blocks when external and internal catheter drainage is ~._ n_ee_d_ed

To be a successful surgeon, you need to have eye of a hawk, heart of a lion and hands of a lady.

625 n :c > "'ti

...

m ::::c N

626

...= Cl)

en

Ci)

0

en a:i

a: Ci)

Figs. 12.7A to E: ERCP being done. Note the gastroduodenoscope with injection of dye. (A) Shows filling defect in the CBD. (B) Shows dilatation of biliary radicles. (C) Shows radiolucent stone (smooth filling defect) in distal CBD which can be removed through ERCP. Antibiotics should be given to prevent cholangitis.

With the help of fluoroscopy (C-arm)/US/CT guided, Chiba or Okuda needle which is long, flexible, thin, blunt, without a bevelled end (15 cm long and 0. 7 mm in diameter) is passed into the liver through right 8th intercostal space in midaxillary line. Once needle is in the dilated biliary radicle, bile is aspirated {sent for culture, cytology, analysis) and then water-soluble iodine dye is injected into the same so as to visualise the dilated biliary radicles, also the site and extent of the obstruction, i.e. tumour, stricture. Procedure can be used for therapeutic stenting across the biliary tree through the obstruction either in the hepatic ducts or in the CBD into the duodenum. It is used whenever ERCP fails, in high strictures {in CHO), in Klatskin tumour, in catheter drainage (external) in high blocks, in stenting high tumours. Prerequisites for PTC ,. Should have normal prothrombin time (or should be made normal by injection of vitamin K or FFP). ,., Should be a last and final inevitable method for evaluation or therapy. , Blood for transfusion in case of bleed , consent for intervention in need, antibiotic prophylaxis-are a must prior to PTC. Complications: Bleeding ; Biliary leak and biliary peritonitis; Septicaemia Note: • Chiba is a University in Japan. Okuda is the name of the person. • PTBD is percutaneous transhepatic biliary drainage. It can be external or internal. It is done through PTC.

MAGNETIC RESONANCE CHOLANGIOPANCREATOGRAPHY (MRCP) It is a non-contrast non-invasive imaging method, better than ERCP as diagnostic tool in biliary and pancreatic diseases.

RADIOISOTOPE SCAN STUDY 1131 Rose Bengal and Tc99 labelled iminodiacetic acid (HIDA, PIP/DA) are very useful in diagnosing acute cholecystitis and other biliary disorders, like biliary atresia. Note: • HIDA is Hippuran lminodiacetic Acid. • Technetium 99 m IDA scan using bilirubin analogue like choletec or hepatolite is done currently. It is a dynamic scintiscan that assesses function of the gallbladder and cystic duct patency.

PEROPERATIVE CHOLANGIOGRAM It is done during CBD exploration for stricture, residual CBD stones, atresia, choledochal cyst, cholangitis. Fine polythene catheter is passed into the CBD through cystic duct and dye is injected. Under C-arm image-intensifier, any block or stricture can be identified and completion of the procedure can be confirmed. Complications: Infection; bile leak. Precaution: Air should not be present in the syringe, as it may mimic stones.

627

CONGENITAL ANOMALIES OF GALLBLADDER r--

Fig . 12.8: Peroperative cholangiogram. Fine catheter is passed through cystic duct into the CBD. Care should be taken to avoid air getting into the CBD (it will mimic stone). Dye is injected under C-arm guidance.

POSTOPERATIVE T-TUBE CHOLANGIOGRAM

--

Absence of gallbladder-rare Phrygian cap-cap-like projection and bend over the fundus of the gallbladder (Phrygia is an ancient Asian country- Mongolia) (Liberte cap of French revolution). It is identified in cholecystogram. It is 6% common Double/triple gallbladder-additional one may be intrahepatic. Cystic ducts may join to form a common cystic duct which joins the CBD or cystic duct of each gallbladder may join separately into the CBD Mobile/floatinglmesenteric gallbladder-gallbladder has a long mesentery attached. It may cause torsion. It can cause recurrent abdominal pain. Its removal is easier through laparoscopic or open method Long cystic duct with low insertion of the cystic duct into CBD near the ampulla. This is important in all pancreatic/biliary/laparoscopic biliary surgeries Absence of cystic duct with gallbladder directly entering the CBD through a wide opening near infu ndibulum Accessory cholecystohepatic duct (duct of Luschka) may be present(10%) which, if not identified and ligated during cholecystectomy, will cause biliary fistula/ peritonitis due to continuous leak Cystic artery may originate anteriorly (15%) from right hepatic artery/from common hepatic artery Very tortuous common or right hepatic artery in front of the origin of the cystic duct is called as Moynihan's caterpillar hump. It is important cause of bleeding in cholecystectomy There may be double cystic duct or cystic duct may insert high nto the common hepatic duct or right hepatic duct

~

After choledochotomy, Kehr's T-tube is placed in CBD for 14 days and then water-soluble dye is injected into the tube and X-ray is taken. Complete free flow of dye into the duodenum indicates that there is no blockage. T-tube can then be removed safely. Block indicates residual CBD stones.

C"')

:::c

>

"'C

--t

m :::c

...... N

C') Ql

er Ql

Cl. Cl.

....

(II

I

11

r..:.,

~~,~~~~%hepa~ t·c duct (duct of [3 Luschka)-10%

Figs. 12.10A and 8: (A) Cystic duct is absent with wide communication

into CBD. There is higher chance of CBD injury here; (B) Accessory cholecystohepatic duct (Duct of Luschka-10%). It may cause bile leak postoperatively if it is not ligated during cholecystectomy.

Figs. 12.11A and 8: (A) Double cystic ducts from the gallbladder Fig. 12.9: T-tube cholangiogram.

ining the CBD; (B) Long cystic duct joining the CBD very low.

Give someone a fish and you feed him for a day. Teach someone to fish and you teed him for a lifetime. -Lao Tzu

628

CHOLEDOCHALCYSTS

c:-

... Q)

en

:::I Cl)

en

cc

cc

Cl)

Figs. 12.12A and B: (A) Cystic duct joining the right hepatic duct-a variation; (B) Phrygian (cap) gallbladder.

It is defined as isolated/focal or combined/diffuse congenital dilatation of extra or intrahepatic biliary tree. It is congenital cyst with partial or complete weakness of the CBD biliary wall. It may be single/multiple or extrahepatic or intrahepatic. It is more common in Asia. It is common in Japan. Its incidence is 1 in 1000 hospital admissions. It is often associated with pancreaticobiliary malju nction. There is long common channel more than 2 cm. It causes reflux of pancreatic juice into the bile duct, enzymatic destruction of the bile duct wall, ductal wall weakening and dilatation-Babbitt theory. During embryogenesis, there is abnormal early canalisation of bile duct with distal obstruction causing increased proximal pressure, weakening and ductal dilatation. Reduced postganglionic autonomous neurons in the distal portion of the cyst causing its poor function distally. Chronic inflammation, sparse mucin glands and metaplasia are the histological features. Histologically, it is either glandular with normal cuboidal epithelium with cavities in the mucosa. Or fibrotic type with fibrous cyst wall with thickened bile duct.

Figs. 12.13A and B: (A) Gallbladder diverticulum; (B) Cystic artery originates from right hepatic artery which isanterior tocommon bile duct.

Pancreatic duct

' - - - - - Pancreaticobiliary maljunction

Figs. 12.14A and B: Double gallbladder. One may be intrahepatic. It

may have separate cystic ducts joining CBD or two cystic ducts join to form single duct to join the CBD.

·, -, r , ,,, >=.

····~ .1~ ' .. . ," ~,.

•

CBD more proximally than normal causing reflux of pancreatic enzymes into the CBD. It leads into weakening, dilatation and choledochal cyst.

Types of Choledochal Cysts (Todani Modifi-cation of Alonso-Lei Classification

.

·• " '-·~Wi

Fig. 12.16: Pancreaticobiliary maljunction. Here pancreatic duct joins

,..

-

Fig. 12.15: Moynihan's caterpillar turn/hump is bend of right hepatic artery in front of/close to cystic duct. It may get injured during cholecystectomy causing torrential haemorrhage.

Type I: Dilatation of extrahepatic biliary tree (60%). ,. Type la-cystic. ,. Type lb-focal segmental (saccular). ,. Type le-fusiform. Type II: Diverticulum of extrahepatic biliary tree (5%). Type Ill: Choledochocele-cystic dilatation of intraduodenal part of CBD (5%). Type IV: Dilatation of extra- and intrahepatic or multiple parts of extrahepatic biliary tree (30%). ,. Type IVa: Dilatation of extrahepatic and intrahepatic biliary tree. It is 2nd most common. ,.. Type IVb: Dilatation of multiple sections of the extrahepatic bile duct.

Type V: Dilatation of the only intrahepatic biliary tree (Caro/i's l~C :. .o.:_m -lp:.. l_ .:ic_a_ti_o_ns_____________ disease). Pancreatitis, mainly in type Ill. Suppurative cholangitis. Gallstone and CBDstone formation. Biliary cirrhosis-secondary; portal hypertension. Rupture of cyst and peritonitis. Cholangiocarcinoma in CBD (30% of cases). Incidence of malignancy increases with age and is more common in type I and V. Reflux, stasis, superinfection , pancreaticobiliary maljunction are the causes for malignant transformation. Malignancy can develop even in residual cyst or at anastomotic site. Malignancy is common in posterior wall.

I Investigations US abdomen-unilocular cyst mainly in infants. CT scan- mainly to see intrahepatic biliary system. Hepatobiliary nuclide scanning. ERCP, Cholangiography-to see ductal anatomy. MRCP-to see status of pancreatic and biliary system and pancreaticobiliary maljunction-idea/. Liver function tests. PTC to see intrahepatic biliary tree.

I Treatment Fig. 12.17: Different types of choledochal cysts-(1) Fusiform type, (2) Saccular type, (3) Choledochocele, (4) Fusiform+ lntrahepatic type, (5) lntrahepatic type.

I Presentations Common in females 4:1. Common in Japan. Present in infants, children and even in adults. Obstructive jaundice (80%), pain in right hypochondrium, cholangitis. Mass per abdomen- mass is to the right and above the umbilicus, smooth , soft, not moving with respiration, not mobile and resonant (30%). Failure to thrive.

B

TRIAD OF CHOLEDOCHAL CYST (SEEN IN 10% OF CASES)

• Right upper quadrant pain • Palpable abdominal mass

B

SWELLINGS WHICH APPEAR AND DISAPPEAR I

I

t

• I

• t

I

• Pseudopancreatic cyst • Choledochal cyst

Resection of extrahepatic biliary tree with removal of choledochal cyst along with cholecystectomy and Roux-en-Y hepaticojejunostomy is the ideal treatment for choledochal cyst especially types I, 11 and IVb. In type I-excision of cyst with its mucosa and reconstruction by Roux-en-Y hepaticojejunostomy. In type II-excision of the diverticulum and suturing of the CBD wall, can be done. In type Ill-endoscopic sphincterotomy is done. Excision is also often needed. lntrahepatic dilatation is difficult to treat. If it is localised, hepatectomy is sufficient, but if it is diffuse, liver transplantation may be required. In type IV, if cyst is adherent to portal vein posteriorly, that part of the cyst wall over the portal vein is left behind. But mucosa of the part should be removed (Lily's operation). Cholecystectomy is a must in all these types. Incidence of gallbladder carcinoma is also high in these patients.

If cyst has already turned into malignancy-adenocarcinoma, then radical surgery and chemothe rapy is given. Liver transplantation is the choice in type IV and V cases.

Gangrenous cholecystitis is associated with thrombosis of cystic artery.

629

C"')

:c

>

m

....

:x, N

C,

I»

er I»

C. C.

.....

(II

630

CAROLI'$ DISEASE It is congenital, nonfamilial, multiple, irregular, dilatations of the intrahepatic ducts with stenotic segments in between. It is associated with congenital hepatic fibrosis and medul/ary sponge kidney. It can also be included under type V cho ledochal cyst. Presentations: It occurs in you ng age group; equal in both sexes; fever, cholangitis, hepatic failure, sepsis, end stage liver disease. Investigations: Haematocrit; CT scan; ERCP; LFT; MRCP. Complications: Recurrent cholangitis; stones in biliary tree; portal hypertension ; cholangiocarcinoma (7%). Treatment: Antibiotics for cholangitis; endoscopic stenting as initial measure only; hepatectomy. Liver transplantation is final need in most of the patients. Condition carries high mortality.

...en a.,

::I

en 0

BILIARY ATRESIA It may be either due to viral infection or defective embryogenesis resulting in fibrosis of extra- and intrahepatic biliary tree. Incidence is 1 in 10,000; equal in both sexes. It is obstructive cholangiopathy with inflammation, destruction and obliteration predominantly involving the extrahepatic biliary tree. There is bile duct proliferation, cellular and canalicular bile stasis, bile plugs in portal tract bile system, periportal inflammation and fibrosis.

Figs. 12.18A and B: CT picture of choledochal cyst (Courtesy: Dr Yogish Kumar, MS, Professor, KMC, Mangaluru).

• • • •

lnfections- cytomegalovirus, reoviru s, rotavirus and human papilloma virus Immune and autoimmune diseases Abnormal development of the biliary system Toxins Vascular defects of the hepatic artery

Classification: Correctable-10%; Noncorrectable- 90%.

B Fig. 12.19: Choledochal cyst on table look.

• Type I: Atretic CBD-1 0% • Type II: Atretic CBD and common hepatic duct • Type Ill: Atretic CBD, common hepatic duct and right and left hepatic ducts-88%

Type II

Fig. 12.20: Types of biliary atresia.

Biliary atresia can be: Perinatal acquired (70%), not associated with congenital anomalies. Fetal/embryonic (30%) is associated with congenital anomalies. It may be associated with other congenital anomalies (20%) of the GIT- (malrotation, annular pancreas duodenal atresia) and congenital heart diseases, polysplenia, situs inversus, absent vena cava, preduodenal portal vein.

I Features Progressive jaundice in a newborn. Steatorrhoea (Pale stool is common). Hepatomegaly. Splenomegaly with portal hypertension. Osteomalacia; Biliary rickets. Severe pruritus; Clubbing, skin xanthomas. Differential diagnosis , Neonatal hepatitis; Choledochal cyst. , Cholestatic jaundice; Sclerosing cholangitis. , Metabolic diseases; Storage disorders. ,, Alagille syndrome; Hypoplastic biliary ducts. Diagnosis , Liver function test-conjugated hyperbilirubinaemia. , US abdomen shows triangular cord, tubular echogenicity extending above the bifurcation of the portal vein which is more than 4 mm thick-diagnostic. , MRCP- 100% accuracy. , Radioisotope study (HIDA or PIPIDA scan). , On table cholangiogram. , Liver biopsy (Percutaneous)-is usual ly first test obtained. ,, Rose Bengal 1131 study, if shows 10%, it is more likely to be hepatitis.

I Treatment In correctable cases: Roux-en-Y jejuna! anastomosis. In noncorrectable cases, hepaticoportojejunostomy (Kasai's operation). Liver transplantation is becoming more popular in biliary atresia-ideal . Presently Kasai ( 1974) porto-enterostomy is done (in 8 weeks) as a preliminary procedure followed by liver transplantation eventually. After opening the abdomen, gallbladder is cannulated and on table cholangiogram is done to confirm atretic segment. Liver biopsy should also be done at the same time. If biliary patency is found, proced ure is abandoned. In atretic biliary tree, gallbladder is dissected off the liver and by applying traction to it, fibrotic atretic biliary tree is felt and dissected

upto intrahepatic segment (up to the segmental branches of portal vein). Portal plate is anastomosed to Roux-en-Y part of the small bowel. Postoperatively, antibiotics, intravenous corticosteroids initially, later orally (for 6-12 weeks), ursodeoxycholic acid (for 1 year), nutrition, medium chain and essential fatty acids, essential elements supplementation are given. Success of procedure depends on age of the patient; presence of cholangitis; ductule size (more than 200 µm is better); bridging fibrosis; type of atresia; bilirubin level. Eventually cholangitis, portal hypertension, variceal bleeding will develop.

631

(."')

::c > -a -t

m

:a -4

N

C) t:I)

C-

GALLSTONES Thus with stone obstruction of the common duct, dilatation of the gallbladder is rarely observed; the organ has already undergone contraction; with obstruction from other causes, dilatation is to be expected. -Ludwig Courvoisier, 1890

Fig. 12.21: Multiple gallstones removed along with gallbladder.

I Types 1. Cholesterol stones (Cholesterol solitaire-radiating crystalline appearance) are 6% common, often solitary. 2. Mixed stones are 90% common. It contains cholesterol, calcium salts of phosphate carbonate, palmitate, proteins, and are multiple faceted. 3. Pigment stones are small, black or greenish black, multiple. Often they can be sludge like. Common in "Fat, Fertile, Forty, Flatulent, Female". Common in western countries and in north India.

I Pathogenesis I. Metabolic:

, Cholesterol is synthesised in liver. Its solubility is determined by relative concentration of cholesterol, bile salts

Porcelain gaffbladder is radiopaque gaffbladder, due to calcification of the gaffbladder waff foffowing chronic cholecystitis which has got high malignant potential.

i»" Q. Q. CD

...

632

and lecithin. Altered levels of cholesterol, lecithin, and bile salts in bile reduces the micelle concentration in the bile leading to precipitation of insoluble cholesterol, hence, the stone formation (Lithogenic bile). Altered GB function • Stasis • Poor emptying • Poor absorption • Infection Supersaturated bile • Female • Fertile • Fat • Forty • High calorie

Cholesterol nucleation factors

Altered enterohepatic circulation • lleal resection • lleal diseases • Altered bowel transit time • Altered bowel flora • Cholestyramine • Deoxycholate

Fig . 12.22: Diagram showing different causes

of gallstone formation.

Fig. 12.23: Pigment stones showing faceted look.

, Normal ratio of bile salt and lecithin to cholesterol is 25:1. Ratio below 13:1 leads to precipitation of cholesterol. Insoluble cholesterol is within the soluble micelle which is formed by lecithin and bile salts. If cholesterol component increases, bile gets supersaturated and inadequate micelle makes insoluble cholesterol to undergo crystallisation and cholesterol monohydrate stone formation (Admiron's triangular hypothesis). , Some cholesterol remains as bilayered lipid vesicles which are soluble. A specific heat labile glycoprotein in

bile induces cholesterol monohydrate crystal formation in the vesicle and causes their aggregation. It is called as nucleation. .,,. Eventual precipitation and stone formation occurs by infection/infestation; pancreatic flu id reflux into CBD causing conversion of toxic lecithin to lysolecithin which is also toxic (causes supersaturated bile); bile stasis or altered enterohepatic circulation. , Any condition which either increases the cholesterol secretion in the bile or reduces the bile salt concentra· tion causes cholesterol stone formation. Old age; OCP; obesity; clofibrate may increase cholesterol secretion. Oestrogen, ileal resection and cholestyramine reduce the bile salt concentration . .,,. Chenodeoxycholic acid and ursodeoxycholic acid prevent cholesterol stone formation by maintaining bile acid pool; reducing cholesterol synthesis and secretion; converting supersaturated bile into normal bile.

B Obesity, Mutation (MDR3, GYP?, A1) • Drugs - Oral contraceptive pills, octreotide - Clofibrate; Cholestyramine lleal disease; lleal resection Altered enterohepatic circulation

-------------'

11 . Infections and Infestations: ,, Bacteria like E. coli, Salmonella , Parasites like Clonorchis sinensis and Ascaris lumbricoides are often associated. , Moynihan 's aphorism: "A gallstone is a tomb stone erected to the memory of the organism within it." Ill. Bile stasis: ,, Occurs due to estrogen therapy, pregnancy, vagotomy and in patients who are on long-term intravenous fluids or TPN. IV. Increased bilirubin production , Due to any of the causes of haemolysis as in hereditary spherocytosis, sickle cell anaemia, thalassaemia, malaria, cirrhosis. Here pigment stones are common.

Rarely centre of the stone contains radiolucent gas which is either triradiate (Mercedes Benz sign) or biradiate (Seagull sign). Sometimes gallbladder may be filled by 'toothpaste like' material which is a mixture of calcium carbonate and phosphate, which on plain X-ray looks like an opacified gallbladder, so called as Limey gallbladder.

633

Only 10% of gallstones are radio-opaque, 90% are radiolucent.

Figs. 12.26A and B: Gallbladder with stone removed from Hartmann's pouch.

I Effects of the Gallstones

Fig. 12.24: Multiple gallstones collected during cholecystectomy. Note:

• Black pigment stones are common in gallbladder. It is usually calcium bilirubinate, calcium phosphate and bicarbonate stone with a matrix. It is common in haemolytic disorders. They are usually multiple, small black and hard in consistency. Mucin Aand mucin C5 secreted by biliary glands may be the aetiology. Cholesterol component here is less than 30%. It is often seen in cirrhosis. They almost always form in gallbladder. They are common in Asia and Japan. • Brown pigment stones are formed in biliary tree as primary biliary stones. It is commonly due to infection like Escherichia coli and bacteroides (98%) with bacterial nidus at the centre (often Ascaris lumbricoides or Clonorchis sinensis infestation or foreign body or stents). They secrete 13 glucuronidase to cause hydrolysis of soluble conjugated bilirubin to insoluble calcium bilirubinate. It also contains calcium palmitate, calcium stearate and cholesterol. They are brownish yellow, soft and mushy. • 15% of population develops gallstones; 80% are asymptomatic; 2% of asymptomatic develop symptoms yearly.

Figs. 12.25A and B: (A) Gallstones removed, on cutting showing central nidus; (B) Thickened gallbladder due to chronic cholecystitis with single cholesterol stone.

In the gallbladder ,., Silent asymptomatic stones occur in 10% of males and 20% of females. , Biliary colic with periodicity, severe within hours after meal (commonest presentation). Biliary colic is spasmodic pain often severe, in right upper quadrant and epigastrium radiating to chest, upper back and shoulder. It is self-limiting, recurs unpredictably, often precipitated by a fatty/heavy meal. Fever and increased WBC count may be observed. , Acute cholecystitis; Chronic cholecystitis.

Figs. 12.27A to D: Different types of gallstones. Note the faceted stones; black stones; brown stones; green stones. Thickening of gallbladder is seen. Hartmann's pouch is obvious in one of the photos (with cholesteroses).

It is safer to look and see than to wait and see. -Sidney Cuthbert Wallace, 1907

C') C,)

C" C,)

C. C.

... CD

,, Empyema gallbladder. , Perforation causing biliary peritonitis or pericholecystitic abscess. ,. Mucocele of gallbladder. ,, Limey gallbladder. , Carcinoma gallbladder. In the CBD ,, Secondary CBD stones (occurs in 10% of gallstones). , Cholangitis. , Pancreatitis. , Mirizzi syndrome (compression of CHD/CBD by stone from cystic duct or cholecysto-choledochal fistula).

I Management of Gallstones Ultrasound abdomen (gallstones are seen with posterior acoustic shadowing); plain X-ray abdomen; LFT; total WBC count. CT scan abdomen to rule out presence of CBC stones Laparoscopic cholecystectomy ideal. Open cholecystectomy is done through right subcostal Kocher's incision. Open approach is used if patient is not fit for laparoscopic surgery (anaesthesia) ; in suspected CBD stones; Mirizzi syndrome, suspected carcinoma gallbladder.

Perforation (Hartmann's)

.r..- - - Mirizzi syndrome Carcinoma

~ =-- - CBD stone

AbscessPerforation _ / (fund us)

Fig. 12.28: Diagram showing complications of gallstones.

In the intestine , Cholecystoduodenal fistula causing gallstone ileus and so intestinal obstruction.

12.29A and B: Multiple gallstones in a plain X-ray. Only 10% gallstones are radio-opaque. Often they are faceted because of compactness and equal pressu re exerted by each stone. Centre of the gallstone is often found radiolucent and is called as Mercedes Benz sign/Seagull sign.

I Flatulent Dyspepsia It is discomfort in the abdomen, belching, heartburn, fat intolerance, sensation of fullness in the abdomen usually observed in fatty, fertile, flatulent female.

I Gallstone Colic It is sudden, severe colicky abdominal pain in right upper quadrant which radiates to back and shoulder. This pain is due to sudden spasm of gallbladder wall when gallstone moves towards the neck of the gallbladder or cystic duct and gets impacted. Tachycardia and restlessness are common. Right hypochondrium is tender. It is precipitated by supine position while sleeping at night. It lasts for few hours and is episodic. It may precipitate acute cholecystitis or empyema gallbladder. There is reflex pylorospasm causing vomiting.

• Asymptomatic stone in the gallbladder • Usually it is cholesterol stone, often single • It is accidentally discovered by U/S • It need not be treated unless: Patient is diabetic/immunosuppressed - High chances of developing gallbladder carcinoma Stone more than 2.5 cm/multiple stones If gallbladder wall is thickened

Fig. 12.30: Lateral X-ray of spine showing radio-opaque shadow in front of the vertebrae-could be radio-opaque gallstone. Note also the fused intervertebral disc spaces.

During laparoscopic cholecystectomy, if there is on table difficulty in dissection at Calot's triangle, then conversion into open approach may be required. Dissolution therapy for asymptomatic cholesterol stones using ursodeoxycholic acid can be tried. It is not very successful.

Portal vein. Through bile after filtering in the liver via portal circulation.

I Pathogenesis of Acute Cholecystitis

Fig. 12.31 : Ultrasound gallbladder showing echogenic lesion. US should be done with change of position to find out movement of the lesion with posterior acoustic shadow to say it as gallstone. Otherwise it will be gallbladder polyp or sludge ball.

• Kidney stone Calcified 12th rib tip Phlebolith Pancreatic stone Radio-opaque foreign body • Faecolith • Calcified lymph node • Calcified renal tuberculosis

I Pathology of Acute Cholecystitis Gallbladder will be distended with oedematous friable wall. Wall contains dilated vessels. Areas of necrosis and patchy gangrene may occur in severe cases. Mucosa shows ulceration and necrosis. Lumen contains infected fluid/infected bile or frank pus. Histology shows features of acute inflammation with neutrophils, oedema, and areas of necrosis and cell death.

• Renal cell carcinoma-

calcifications

• Gallstone • • • •

Stone causes obstruction at Hartmann's pouch or in cystic duct. Obstruction causes stasis, oedema of the wall, bacterial infection, acute cholecystitis and its effects. Impacted stone also causes mucosal erosion allowing bile salts to act over the submucosal tissues as bile is toxic to these tissues. It leads into necrosis, further infection and often perforation of the gallbladder usually at Hartmann's pouch.

Adrenal tumourcalcification • Teratomatous dermoid • Calcification of atheroma in aorta • Calcified lesion in liveramoebic liver abscess/ calcified hydatid cyst

ACUTE CHOLECYSTITIS Commonly, it occurs in a patient with pre-existing chronic cholecystitis but often also can occur as a first presentation. Usual cause is impacted gallstone in the Hartmann's pouch, obstructing cystic duct.

m

CAUSATIVE BACTERIA ARE:

J

• E. coli-most common • Salmonella • Klebsiella, Pseudomonas, Proteus • C/ostridium welchii • Strep. taecalis

I Classification

I Complications of Acute Cholecystitis Acute cholecystitis can lead to: Perforation-5-10% which usually occurs in the fund us or in the neck (Hartmann's). It can cause cholecystoduodenal, cholecystointestinal or cholecystobiliary fistula, Mirrri zi's syndrome. Peritonitis; Pericholecystitic abscess. Cholangitis and septicaemia. Empyema gallbladder; gangrenous gallbladder.

B J •

1·

1. Acute calculous cholecystitis. 2. Acute acalculous cholecystitis (10%).

• • • •

I Mode of Infection Haematogenous through hepatic artery-cystic artery.

Seen in elderly male patients Common in diabetic and immunosuppressed individuals Clostridium welchii, Coliform, streptococci are common organisms. USG and CT scan are diagnostic. Gas is seen in the gallbladder Results in life-threatening septicaemia It causes severe fulminant cholecystitis Gangrene, perforation and peritonitis are common Emergency cholecystectomy is needed

• Absence of stones- observed in more than 50% of cases.

Life is endless until it ends.

635

C")

:::c

>

"'CJ -4

m

:Jl

..... N

C') I» CT I» CL. CL.

...

C1I

636

LFT is important. Increased serum bilirubin often signifies cholangitis or stone in the CBD. Plain X-ray abdomen is not very relevant but is often important to rule out duodenal ulcer perforation , peritonitis. Only 10% of gallstones are radio-opaque. In emphysematous cholecystitis gas shadow may be seen in the region of gallbladder. Porcelain gallbladder may be seen as opacified area in gallbladder region.

... Q)

en :s

en 0

Fig. 12.32: On table look of gallbladder with acute cholecystitis.

Fig. 12.33: Gallbladder with acute cholecystitis-laparoscopic view.

I Clinical Features Sudden onset of pain in the right hypochondrium, with tenderness, guardi ng, and rigidity. Palpable, tender, smooth, soft gallbladder. Area of hyperaesthesia between 9th and 11th ribs posteriorly on the right side (Boas's sign). Jaundice may be present. Fever, nausea, palpable tender mass in GB region (25%). Tachycardia and toxic features. Murphy's sign may be positive (mid-inspiratory arrest).

I Investigations Ultrasound abdomen-very useful, reveals presence or absence of gallstones; and thickening of gallbladder wall. Sonographic Murphy's sign may be positive. Plain X-ray abdomen- 10% of gallstones are radio-opaque; also rules out other causes of acute pain abdomen. Gas is seen in emphysematous GB. Total count shows neutrophilia. HIDA/PIPIDA radioisotope study- very useful. Non-visualisation of gallbladder is diagnostic.

Fig. 12.35: CT scan showing stone in the neck of the gallbladder.

CT scan is useful in identifying the perforation, impacted stone, gallbladder wall thickness and oedema.

B Duodenal ulcer perforation Acute pancreatitis Acute appendicitis

• Acute pyelonephritis • Lobar pneumonia, myocardial infarction • Ruptured ectopic pregnancy _:__J

I Treatment Advised hospitalisation. Initially (nonoperative) conservative treatment (95%): ,.. Nasogastric aspiration. , IV fluids. , Analgesics and antispasmodics. , Broad spectrum antibiotics (cefoperazone, ceftazidime, ceftriaxone, cefotaxime + amikacin, tobramycin + metronidazole {antimicrobial}).

,. Observation. ,. Follow-up UIS scan. Later after 3-6 weeks, elective interval cholecystectomy, either by open method through right subcostal (Kocher's) incision or through laparoscopy is done.

treatment. Usually open surgery, either cholecystectomy (may be partial) or cholecystostomy is done.

Cholecystostomy is done immediately if patient is having: Empyema gallbladder; Persisting symptoms; Progressing symptoms. Here the gallbladder is opened and all stones and pus are removed. Either a Foley's or Malecot's catheter is placed in the gallbladder and is exteriorised. US-guided percutaneous cholecystostomyalso can be used. After 3 weeks, elective cholecystectomy is done.

B

•• I•

l

INDICATIONS FOR CHOLECYSTOSTOMY OR EMERGENCY CHOLECYSTECTOMY (5%)

Empyema GB Persisting symptoms/failure of medication Emphysematous cholecystitis Perforation/peritonitis; elderly

Fig. 12.37: On table photo of distended gallbladder. Note the Calot's triangle, liver and CBD.

ACUTE ACALCULOUS CHOLECYSTITIS (20%) It is not an uncommon entity, but can be commonly missed. It is common in patients who have undergone major surgeries, trauma, burns, or any other stress or in cases of cholecystoses.

Urosac bag

Fig. 12.36: A Foley's or Malecot's catheter is placed in severely

ill-patient with acute cholecystitis to drain pus initially, and later elective cholecystectomy is done. Procedure is done in elderly, severely ill, empyema GB, emphysematous GB, perforation of GB (cholecystostomy). Note:

• Cholecystostomy is better option than emergency cholecystectomy as chances of injuring adjacent structures are higher in emergency cholecystectomy. Other option is partial/subtotal cholecystectomy with removal of part of GB distal to the Calot's triangle. • There is a changing trend now towards early cholecystectomy in acute cholecystitis. Laparoscopic cholecystectomy can be done in 48-72 hours in these patients. But conversion chances are high-5-6 times more than usual. Complications also may be higher due to difficulty in dissecting the Calot's triangle. It can be done in good set up with surgeons having adequate experience. • When patient's condition is deteriorating, and acute cholecystitis is progressing, suspicious about forming empyema or necrosis and perforation; surgery should be done by abandoning conservative

Fig. 12.38: Acalculous cholecystitis. Note the necrotic

gallbladder wall. Common in ICU patients- in critically-ill patients. It is due to bile stasis and ischaemia. Common in AIDs, elderly males, diabetics, patients who have undergone major non biliary surgery, patients who are on TPN, atherosclerotics. It may also be seen in malignancy (biliary or other), as primary bacterial infection by E. coli, Salmonella, Clostridia, in cystic artery occlusion. Exact cause is not known. Gallbladder distension, release of activation factor VI I may be the causes. Pathology is oedema and necrosis of the gallbladder wall with features of acute inflammation. Presentation is usually acute. Gangrene, perforation, empyema are ve,y common (70%). Investigations: Isotope study (HIDA), US abdomen. Treatment: Cholecystectomy.

Surge,y is to be done, not to have.

637

638

... Q)

en

::I Cl)

0

Percutaneous US-guided/CT-guided or open cholecystostomy initially, later cholecystectomy is the treatment of choice.

MIRIZZI SYNDROME (Pablo Luis Mirizzi , Surgeon, Argentina, 1948) Mirizzi syndrome is seen in 1% of all cholecystectomies. In Mirizzi syndrome, gallstone impacts in the gallbladder wall and compresses it causing pressure necrosis which further gets adherent to CHD/CBD wall. It eventually causes compression and later occasionally leads into cholecystocholedochal fistula. It occurs either from Hartmann's pouch into CHD/CBD (common) or from fundus of gallbladder into the CBD. Presentations are-fever, abdominal pain, obstructive jaundice, upper abdominal tenderness on right side. Soft, enlarged liver may be palpable. Investigations- USG, CT scan, ERCP/MRCP to delineate duct anatomy, dilatation of intrahepatic biliary system with block at CHO is found. HIDA scan is useful.

,, Mirizzi syndrome is suspected on CT scan, but usually identified on table. It needs cholecystectomy; on table cholangiogram; and exploration of CBD. It often needs Roux-en Y hepaticojejunostomy. , Subtotal/partial cholecystectomy is done with ERCP stenting is done in type I. , Partial/subtotal cholecystectomy with primary closure of CHD/CBD is done with a T-tube insertion through a separate choledochotomy in type 11. ,, Choledochoplasty using retained gallbladder flap after subtotal/ partial cholecystectomy is often used successfully in many centers. ,, Partial/subtotal cholecystectomy with closure of gallbladder flaps is done with T-tube insertion through a separate choledochotomy in type Ill. Cholecystectomy with duodenal/jejuna! anastomosis (hepaticojejunostomy) is done in type 4 and type V and when difficulty arises in type I, II, Ill also. ,, Postoperative surgical mortality is 8-10% in Mirizzi syndrome.

Mlrizi's types Features (Cssndss classification, modifisd In 2008)

Type 1-10% Extrinsic compression of the CBD/CHD by a large impacted stone in Hartmann's pouch; IA is presence of cystic duct; IBis obliterated cystic duct-ERCP- tenting-subtotal/partial cholecystectomy Type IIThe stone has eroded the CBD/CHD less than 113rd 40- 50% circumference of the CBD/CHD forming a fistulasubtotal cholecystectomy with CBD exploration Type 111Involving up to 213rd of the circumference of 20- 30% CBD/ CHO-subtotal cholecystectomy with CBD exploration Type IVCholecystocholedochal fistula (>2/3rd) with 2-5% involving entire circumference of the CBD/CHD needs hepaticojejunostomy TypeVCholecystocholedochal fistula with cholecystoen15--25% teric fistula without gallstone ileus (VA) or with gallstone ileus (VB) Note: Incidence of gallbladder cancer association is more with Miriui syndrome.

f.:.1

Mirizzi syndrome type I (compression)

IT)

It is a type of acute cholecystitis wherein the gallbladder is filled with pus. In 30% cases pus may be sterile. It also can occur in a pre-existing mucocele of the gallbladder where it gets infected. It is commonly observed in impacted stone, diabetic individual, immunosuppressed people like HIV, long-time steroid therapy. It can perforate; can form abscess or can cause peritonitisbiliary and bacterial. Condition has got high mortality.

I Features Fever, toxicity; pain and tenderness in right hypochondrium. Tender, smooth, globular, gallbladder is palpable in right hypochondrium to the right of the right rectus muscle.

Mirizzi syndrome type IV (fistula)

Figs. 12.39A and B: Mirizzi syndrome type I and IV.

Treatment:

,

EMPYEMA GALLBLADDER

Open approach is ideal for Mirizzi even though laparoscopic approach is done in few centres.

Sphincter of Oddi

Fig. 12.40: Gallstone obstructing the neck of the gallbladder in Hartmann's pouch, may lead into empyema or mucocele of the GB.

any infection or inflammation in the gallbladder. This causes absorption of all bile and secretion of mucous into the gallbladder allowing gallbladder to distend causing mucocele of the gallbladder (Hydrops of the gallbladder). Content is usually sterile. Features: Dyspepsia with painless swelling in the right hypochondrium; nontender, smooth, soft, globular, palpable gallbladder which often reaches down up to pelvis. Differential diagnoses are-choledochal cyst; mesenteric cyst, hydatid cyst, pseudocyst of pancreas. Complications: If infected, can cause empyema gallbladder; perforation, biliary peritonitis can occur. Rupture can cause pseudomyxoma peritonei (but rare). Fig. 12.41 : Empyema gallbladder. Note the pus and stone.

Complications: Septicaemia; rupture and peritonitis. Investigations: Ultrasound abdomen; Radioisotope scan. , Total count is raised; Haematocrit, LFT, PT-INR.

I Treatment

Investigations: US abdomen (anteroposterior diameter is more than 5 cm); Liver function tests; CT scan; MRCP. Treatment: Cholecystectomy, either laparoscopic or open method. Occasionally needs initial percutaneous-guided needle decompression or cholecystostomy prior to definitive cholecystectomy.

Antibiotics: Cefotaxime, quinolones, ceftriaxone. Cholecystectomy-an emergency procedure. Often initially cholecystostomy is done, with either Foley's or Malecot's catheter kept in situ. Later after 3- 6 weeks, cholecystectomy is done. Laparoscopic cholecystectomy can be tried.

Fig. 12.43: Mucocele of gallbladder.

CHRONIC CHOLECYSTITIS Fig. 12.42: Gangrenous gallbladder.

MUCOCELE OF THE GALLBLADDER (HYDROPS GALLBLADDER) It is overdistension of the gallbladder containing mucoid or clear fluid; it is due to noninflammatory distension results from cystic duct obstruction usually due to an impacted stone but occasionally can be due to other cause like polyp, carcinoma, extrinsic compression, congenital narrowing of the cystic duct, parasitic block (ascaris) or prolonged TPN. It can occur in children also due to varying causes like Kawasaki syndrome, nephritic syndrome, typhoid, etc. It is due to obstruction of the cystic duct by a stone in the duct or in the neck (Hartmann's pouch) of the gallbladder, without

It is chronically inflamed, thickened gallbladder, which is nonfunctioning and nondistending. Causes: Gallstones; cholecystoses; chronic acalculous cholecystitis. Organisms: Klebsiella; streptococci; Salmonella.

I Pathology Gallbladder is shrunken, contracted, small, non-functioning and fibrotic with thickened gallbladder wall. Mucosa proliferates into the lumen creating deep clefts in the wall projecting into the muscular wall of the gallbladder being lined by epithelium. It is called as Rokitansky-Ashchoff's sinuses. Muscular wall is atrophied and is often replaced by fibrous tissue. Histologically, it shows dense chronic inflammation with fibrous tissue.

Success is the ability to go from failure to failure without losing enthusiasm.

639

640

MURPHY'S SIGN

0

C'O ::I C: C'O

:E: _VI

00

a: en Fig. 12.44: Specimen of gallbladder showing thickened gallbladder wall with multiple gallstones. Faceting of stones is due to equal pressure.

I Features Pain in right hypochondrium, may be colicky, or persistent. Positive Murphy's sign, wherein sitting position during deep inspiration, while palpating in right hypochondrium, patient winces with pain at the summit of the inspiration. Same sign elicited in lying down position is called as Moynihan 's sign. It may also be elicited in acute-on chronic cholecystitis. Flatulent dyspepsia; intolerance to fatty meals; biliary dyspepsia. Complications: CBD stone; cholangitis; pancreatitis; Mirizzi's syndrome; carcinoma of gallbladder. Differential Diagnosis: Peptic ulcer; pancreatitis; hiatus hernia, gastrointestinal malignancy, carcinoma biliary system. Investigations: Ultrasound abdomen may show stone with posterior acoustic shadowing (very useful) with thickened gallbladder. Isotope study (HIDA) may help to confirm the infection. LFT, total count may be alteEred if there is recurrent infection. ERCP, CT scan abdomen is required often. Treatment: Cholecystectomy usually laparoscopic; in case of difficulties open approach is used.

It is a sign of cholecystitis-acute or acute on chronic cholecystitis. John Benjamin Murphy (1857-1916) American surgeon, identified significance of his sign. His name is associated with many things-signs like Murphy's punch, Murphy's triad, Murphy's sign test for metacarpals and Murphy's sign for gallbladder and instruments like Murphy's intestinal anastomosis buttons, Murphy-Lane bone skid and Murphy drip. Murphy's sign of gallbladder was described in 1903 as hypersensitivity elicited by deep palpation in the subcostal area when a patient with presumed gallbladder disease takes a deep breath. This sign is also called Naunyn's Sign after the name of Bernard Naunyn (1839-1925, Berne) who described a similar sign 13 years before Murphy. Patient in uprighl/sitting position, the right hand of the examiner curls up under the right costal margin (or extended fingers with moderate pressure) of the patient at the tip of the ninth rib and patient is requested to take a deep breath. If the gallbladder is inflamed, the patient will experience pain (winces with pain) and catches the breath as the gallbladder descends and comes in contact with the palpating hand. Patients with cholecystitis often experience distress with this maneuver and may have a sudden cessation of inspiration when the inflamed gallbladder reaches the examining fingers. This is termed inspiratory arrest and has been described as a "shutting off" of the inspiration. Moynihan's modification of eliciting Murphy's sign: The left hand of the examiner is placed on the patient's lowermost aspect of right anterior rib cage; index finger is resting on the most inferior rib. The extended and abducted left thum b is placed in right subcostal region without pressing deep; the patient is requested to take a deep breath. When the inflamed gallbladder presses/touches against the thumb the patient will experience pain or tenderness enough to halt the inspiration. Moynihan's sign is elicited in lying down supine position. The presence of Murphy's sign is both sensitive (97%) and highly predictive (93%) . Negative Murphy's sign is less sensitive (35%). Bree RL found that this sign as unreliable in separating acute from chronic cholecystitis. Often in acute cholecystitis as such there is severe tenderness, guarding and rigidity in right hypochondrium and so test cannot be elicited. So it is the sign used for cholecystitis without any specific identification as acute or chronic. Sonographic Murphy's sign is eliciting same sign using sonographic transducer. It is more sensitive and more accurate.

GALLSTONE ILEUS

Figs. 12.45A to C: (A and B) Thickened GB due to chronic cholecystitis with multiple stones; (C) Thick GB with single large stone.

It is a type of acute intestinal obstruction, often seen in elderly and is due to blockage by a bolus or mass of gallstones which commonly enter the intestine through cholecystoduodenal fistula (75%) or rarely through cholecystointestinal or gastric fistulas.

,, Cholecystectomy, correction of fistula with T-tu be drainage can be done in same sitting if patient's general condition is good. Otherwise it is done after 12 weeks. If cholecystectomy and definitive corrective surgery is not done, recurrent gallstone ileus is likely to occur.

B

CAUSES FOR GAS IN THE BILIARY TREE

Cholecystoduodenal fistula • Transduodenal sphinctero- 1 Choledochoduodenostomy plasty Choledochojejunostomy • Emphysematous cholecysti~ Note: •

Fig. 12.46: Gallstone ileus.

Gallstones in the gallbladder (stone> 2.5 cm) Cholecystitis .J,

Suppuration and adhesion over the duodenal wall .J,

Communication of gallbladder into the duodenum (Spontaneous bilioenteric fistula) .J,

Gallstones pass into the duodenum forms a bolus ('Rolling stone gather mass')

Blocks narrow part in the ileum. .J, Gallstone ileus

I Features Pain abdomen and features of intestinal obstruction. Stones may perforate the ileum to cause peritonitis. It is 1% of all intestinal obstruction overall; 25% of obstruction in elderly. Recurrent episodic obstruction due to moving stone bolus is typical-tumbling obstruction. Investigations ,, Plain X-ray abdomen in erect posture shows air in the biliary tract (branching gas pattern , pneumobilia) and multiple air fluid levels. , U/S abdomen. ,, CT is diagnostic. Treatment ,, Laparotomy, enterotomy, removal of gallstones and closure of enterotomy is done. Enterotomy is done not at the site of obstruction but more proximal to the site of obstruction and stones are milked towards the enterotomy site. If bowel is found ischaemic at the impacted area, resection and anastomosis is done. ,, Laparotomy and crushing of stones with fingers to relieve the obstruction is only occasionally useful.

Bouveret's syndrome: A rare entity with cholecystoduodenal fistula causing gallstones from gallbladder to pass into the duodenum leading to duodenal obstruction.

CHOLECYSTOSES Cholecystoses (By Colesson and Jutras) a group of noninflammatory, nonlithiasic benign disease of the gallbladder wall. It can be-Hyperplastic or accumulating. The hyperplastic type is characterized by normal growth with hyperplasia of the cellular wall components which can be focal/segmental/extensive; accumulating type is an overload of the wall with organic substances or minerals such as lipids or calcium salts. However often it is associated with inflammation and stone formation. Cholecystoses are chronic, eventual inflammatory conditions of gallbladder with cholesterol deposits. It is due to defective transport of the absorbed cholesterol which accumulates in mucosa. There is also increased absorption of cholesterol by gallbladder epithelium. Cholecystoses gallbladder is more prone for infection. It is a premalignant condition.

Figs. 12.47A and B: (A) Types of cholecystoses. (I) Cholesterosis,

(II) Cholesterol polyposis, (Ill) Cholecystitis glandularis proliferans, (IV) Diverticulosis of gallbladder, (V) Fistula; (B) Cholesterosis of gallbladder. It is a premalignant condition.

I Types I. Aggregations of cholesterol crystals in the mucosa or submucosa- cholesterosis (Strawberry gal/bladdef). Lipoid

Ability may get you to the top, but it takes character to keep you there.

641

("')

:c

>

""C -4

m

::JJ

..... N

642

contents are present in large foamy cells which has phagocytosed cholesterol. Here cystic duct is normal. Disease occurs only in gallbladder. It is a premalignant condition. II. Cholesterol laden polypoid projections in the mucosacholesterol polyposis (Gallbladder polyp). Ill. Granulomatous thickening and hyperplasia of the gallbladder-cholecystit is glandularis proliferans. IV. Diverticula formation in the wall of the gallbladder- diverticulosis of gallbladder. V. Gallbladder wall fistula. Features: Features of acalculous cholecystitis like dyspepsia, positive Murphy's sign; recurrent pain abdomen. Investigations: US abdomen; oral cholecystography (OGG); Isotope study; MRCP (shows pearl necklace or diamond ring sign); LFT. Treatment: Cholecystectomy-always should be done whether it is symptomatic or not as it is potentially malignant.

1. Primary-Rare-brown pigment stones. 2. Secondary-Common-black pigment stones/cholesterol stones. It is seen in 15% of gallstone disease; 75% are cholesterol stones, 15% are pigment stones. Primary stones They are formed in CBD and biliary tree itself, and are multiple, often sludge like, commonly pigment or mixed type, extends into hepatic ducts (Brown pigment stones). Causes: Defective pathophysiology of biliary tree causing stasis, biliary dyskinesia, benign biliary stricture, sclerosing cholangitis, biliary dilatation, choledochal cyst. Congenital conditions like Caroli's disease, choledochal cyst. Infections and infestations like clonorchiasis, ascariasis. Others: Low-protein diet, malnutrition, obesity, females, old age. Note:

DISSOLUTION THERAPY FOR GALLSTONES

Stasis and bactibilia are the main causes of primary CBD biliary tree stones.

I Indications

Secondary biliary stones They are from gallbladder (gallstones) pass through cystic duct to CBD. Here CBD and biliary tree are otherwise normal. Secondary stones are better and easier to manage than primary stones. Commonly gallstones get impacted in supraduodenal portion of CBD.

Functioning gallbladder with cholesterol stone. Single stone less than 1.5 cm. Radiolucent stone. Old age. Patients who are not fit for surgery.

• Nonfunctioning gallbladder • Stone more than 1.5 cm

I Classification

I

• Radio-opaque stone • Multiple stones __J

Drugs used , Chenodeoxycholic acid (for 2 years). , Ursodeoxycholic acid (15 mg/kg/day). They inhibit absorption of cholesterol from the gut and synthesis of cholesterol in the liver. They inhibit HMG CoAa rate limiting step in cholesterol synthesis. Ursodeoxycholic acid also inhibits absorption of cholesterol in GIT.

B • Citrate; Monoterpenes • Percutaneous infusion of methyl-tertiary butyl ether (MTBE) into the gallbladder using a catheter • Extracorporeal shock wave lithotripsy (ESWL)-not popular

Problems with dissolution therapy , Drugs should be given for a long time. , Results are not good; Expensive. , Causes side effects like diarrhoea, pruritus. , Hepatic dysfunction. Overall results are not good by dissolution therapy.

CHOLEDOCHOLITHIASIS It is stones in the CBD and biliary tree.

Fig. 12.48: Multiple stones removed from common bile duct and hepatic ducts. They cause cholangitis. After stone removal, patient needs choledochoduodenostomy/sphincteroplasty/choledochojejunostomy. Note:

• Black pigment stones are common in-haemolytic diseases, cirrhosis, ileal resection, fasting for long time, TPN for long time (home TPN). • lntrahepatic stones are common- biliary sepsis, biliary stasis, parasitic infection, malnutrition. • Retained stones mean-if stones are found in CBD within 2 years of cholecystectomy. • Recurrent stones mean-if stones are found in CBD 2 years after cholecystectomy. • 5% of CBD stones can be asymptomatic

I Clinical Features Incidental CBD stones along with jaundice/without jaundice. Pain: It may be biliary colic; nonspecific abdominal pain; pain of ascending cholangitis, pain of pancreatitis. Jaundice-most common clinical manifestation. Fever with chills and rigors. Pain and tenderness in epigastrium and right hypochondrium. Steatorrhoea and darkening of urine; pruritus.

• Intermittent pain (may be colicky); Intermittent fever • Intermittent jaundice

Stone moves proximally and floats, obstruction is relieved and symptoms subside (Intermittent features). Stone in CBD due to impaction

Causes obstruction

Stasis

I Jaundice

Pain

Infection, bacteraemia and fever

Reynold'spentad ofacute obstructive cholangitis (suppurative cholangitis-5%). Here biliary tree contains pus.

t

m

SOMETIMES OBSTRUCTION PERSISTS CAUSING

Persistent pain; persistent fever; persistent jaundice; shock (toxicity); altered mental status

CT scan is the most sensitive investigation for CBD stones; it shows stones, location, ductal stricture or block, ductal dilatation, intrahepatic biliary changes and stones. Helical CT cholangiography is also useful but bilirubin level should be normal which is the limitation. MRCP is noninvasive investigation which delineates biliary tree anatomy and pathology clearly; but it is not therapeutic. Blood: Total count may be raised; LFT deranged with raised bilirubin (direct) and alkaline phosphatase. PT INR and platelet count should be done. Serum amylase and lipase should be done to rule out associated pancreatitis. EUS (endosonography) is useful and accurate but it is invasive. ERCP identifies pathology, site of block, stones, etc. (95% sensitivity). It is therapeutic also for extraction of biliary stones and stenting. PTC is done only when indicated like in those with previous gastrectomy, failed ERCP. PTC is not routinely needed. PTC can be therapeutic also in extracting the stones and stenting.

I Treatment Injection Vit. K 1Omg IM once a day for 5 days or FFP infusion to correct the prothrombin time. IV antibiotics (cefoperazone, cefotaxime). Correction of dehydration. IV mannitol daily 200 ml BO to prevent hepatorenal syndrome. ERCP-Therapeutic, i.e. endoscopic papillotomy (sphincterotomy) and stone extraction through Dormia basket or balloon catheter; or fragmenting the stone and extraction; or removal through baby endoscope. CBD stent is placed in situ.

I Complications Liver dysfunction and biliary cirrhosis. White bile formation and liver failure. Suppurative cholangitis; Liver abscess; Septicaemia. Pancreatitis if CBD stone is near sphincter of Oddi blocking drainage of bile and pancreatic duct. DIFFERENTIAL DIAGNOSIS

Obstructive jaundice due to other causes: • Carcinoma of head of pancreas • Carcinoma of periampullary region • Carcinoma of biliary tree; biliary stricture; viral hepatitis

I Investigations USabdomen may show gallstones, dilated CBD >8 mm which suggests biliary obstruction. Sensitivity for CBD stones is only 65%.

Fig . 12.49: Plain X-ray showing stents in CBD and pancreatic ducts. It was placed in a patient who presented with recurrent pancreatitis with block/stricture in both terminal CBD and pancreatic duct. Later patient underwent choledochojejunostomy and pancreaticojejunostomy.

Human mind is the most powerful weapon in the world. Great monuments and Great wars have always started in a human mind. -Typhoid Mary

643

C')

I»

CT

= = I»

...

CD

644

CBD stone/sand if ERCP was not done prior to cholecystectomy. If secondary stone is retrieved by this method, open exploration of CBD can be avoided. lntraoperative ultrasound (IOUS) or intraoperative choledochoscope is very useful to confirm that all stones are removed .

...=

Q)

Cl Cl)

0

Tube drain in GB bed

c,:i

= C

c,:i

:ii:: _v, CCI

cc

Cl)

Figs. 12.50A and B: ERCP showing CBD stones. Endoscopic view of stenting is also seen. Complications of sphincterotomy: Bleeding; sepsis; perforation; pancreatitis Once CBD stones are extracted through ERCP, laparoscopic cholecystectomy is done. In open cholecystectomy , After the removal of gallbladder, on table cholangiogram is done through cystic duct using water-soluble iodine dye to see any stones in CBD. Using stay sutures choledochotomy is done (opened longitudinally) to remove stones in CBD.

,

False results/failure of intraoperative cholangiogram (IOC)

Fig. 12.51: Diagram showing placement of T-tube in CBD after choledocholithotomy. Note the separate drain placed in the cholecystectomy bed to drain fluid, blood, bile.

is possible if air is trapped , if there is contrast leakage, if quick flow of contrast into the duodenum, if there is spasm of sphincter of Oddi.

Laparoscopic CBD exploration is becoming popular with availability of expertise and high technology imaging and instruments.

B (After open cholecystectomy) • U/S shows stone in CBD; palpable stone in CBD CBD diameter >8-10 mm • Recent history of jaundice, and with raised serum alkaline phosphatase level On table cholangiogram shows stone (done using C-arm image machine) • Failure of stone extraction by ERCP prior to cholecystectomy. • When in doubt ,, After choledochotomy, stones are removed using Des Jardin's choledocholithotomy forceps. Bake's CBD dilator is used to confirm the CBD patency. ,, Hube (Kehr's) is then placed in the CBD and kept for 14 days. ,, After 14 days a postoperative Hube cholangiogram is done to see for free flow of dye into the duodenum, so that Hube can be removed. , If Hube cholangiogram shows persistent stone, it can be extracted after 6 weeks, through a basket (Dormia) or catheter (Fogarty) through the track or through a choledochoscope. Retained stones can also be removed through ERCP. After open cholecystectomy, on table ERCP can be tried if there are indications for CBD intervention case of secondary

B • Clamp the T-tube, after 10- 14 days and observe 48 hours for development of pain, jaundice and fever Confirm free flow of dye in T-tube cholangiogram

B • Small stones may spontaneously pass down. Heparinised saline or bile acid flushing through the T-tube. (Wash 250 ml of normal saline with 25,000 IV heparin for 5 days) • Burhenne technique (Ganada)-after 6 weeks once T-tube track gets matured, track if needed, is dilated using graduated dilators. Either using Dormia basket or Fogarty catheter or choledochoscope, stone is removed through T-tube track under fluoroscopic guidance (C-arm) • ERGPand stone removal in 3 weeks Reoperation if everything fails, either transduodenal sphincteroplasty or choledochojejunostomy ESWL with endoscopic sphincterotomy/extraction/lavage/stenting • Through percutaneous transhepatic route, cholangioscope is passed and CBD is visualised, stone is identified and removed using Dormia basket or Fogarty catheter Laparoscopic choledocholithotomy • Open choledocholithotomy often with choledochojejunostomy

J

645

Contd...

Figs. 12.52A and B: (A) T-tube cholangiogram showing retained stone in the CBD (Courtesy: Dr Navin Chandra Shetty, HOD, Department of Radiodiagnosis, KMC, Mangaluru); (B) Hube cholangiogram showing dye in the duodenum and visualisation of normal biliary tree without any obstruction.

Treatment of Primary CBD Stones Difficult and is associated with more complications. Preferred procedures:

Transduodenal sphincteroplasty (open method), or Open choledochoduodenostomy, side-to-side, or Open choledochojejunostomy-Roux-en-Y method. ,. These procedures attain complete drainage of bile. ,. Postoperatively they may often require long-term antibiotics.

• • • • • • • •

INDICATIONS FOR CHOLEDOCHODUODENOSTOMYI CHOLEDOCHOJEJUNOSTOMY: Multiple CBD calculi with distal narrowing (Funnel syndrome). Papillary stenosis; impacted calculi. Biliary sludge-symptomatic; residual stones. Sphincter of Oddi dysfunction/stenosis. Primary CBD stones; previous choledochotomy. Marked CBD dilatation.

Prerequisite for choledochoduodenostomy: CBD should be more than 1.4 cm and stoma should be 2 cm.

Advantages of choledochoduodenostomy are: Bile leak is minimal/not there. Beneficial as a permanent remedy in multiple stones/sludge/ stenosis/strictu res/intrahepatic stones.

Problem with choledochoduodenostomy: Sump syndrome. Note: Roux-en-Y choledochojejunostomy is equally good as choledochoduodenostomy.

B • Gallstones are commonly radiolucent (90%). • Multiple stones are usually faceted because of exertion of equal pressure in a compact gallbladder. • Plain X-ray shows radio-opaque lesion to the right side of the vertebra below rib cage. It should be differentiated from kidney stones. In lateral view X-ray, gallstone will be in front of the vertebra whereas kidney stone overlaps the vertebra. Often gallstone has got central radiolucent area-seagull sign/Mercedes Benz sign. • Silent/asymptomatic gallstone is onewhich is identified on routine investigation where there are no specific relevant symptoms related to gallstones. Chances of developing symptoms in a silent llstone is 5% in 5 years and 20% in 15 years. ---Contd...

Presently ultrasound is ideal investigation for gallstones. To see gallbladder function or confirm cholecystitis radioisotope HIDA/PIPIDA scan is ideal. Cholecystitis can cause jaundice due to cholangitis. But other causes of jaundice should be ruled stones/Mirizzi syndrome. Limey gallbladder is gallbladder filled with toothpaste like mixture of calcium carbonate and calcium phosphate. Plain X-ray shows dense radio-opaque gallbladder shadow (opacified gallbladdet,. Porcelain gallbladder is one where gallbladder wall is calcified because of chronic cholecystitis. It is potentially malignant. It should be removed either by open or laparoscopic method. Cholesterol stone occurs when there is alteration in levels of cholesterol, lecithin and bile salts. This altered bile has got more cholesterol than adequate micelleand is called as lithogenic bile. Here bile is in supramicellar zone. Cholesterol stone is radiolucent but causes acoustic shadow in ultrasound I Mixed stones are most common-90%. 'Gallstone is a tomb stone erected to the memory of the organism within if---Moynihan's aphorism. • Saint's triad: Gallstones-colonic diverticulosis-hiatus hernia. Complications of gallstones: Acute cholecystitis, chron ic cholecystitis, empyema gallbladder, mucocele of gallbladder, perforation and peritonitis, secondary CBD stones, cholangitis, pancreatitis, Mirizzi syndrome, gallstone ileus, pericholecystic abscess and carcinoma of gallbladder. Black pigment stones are more common in gallbladder; brown pigment stones are common in CBD. Cholesterol stones are common in Western countries; pigment/ mixed stones are common in Asian countries. Acute acalculous cholecystitis is 5% common. It occurs after stress, major surgeries or in cholecystoses. Xanthogranulomatous cholecystitis is a rare pathological condi- I tion. Gallbladder is thickened with chronic inflammation. It is yellow xathogranulomatous in nature. It often extends to adjacent organs. It is due to reaction to penetrated bile. • ResiduaVretained bile duct stones are one which is present in CBD within 2 years of initial surgery-cholecystectomy. They are usually missed secondary bile duct stones. / Recurrent bile duct stones are one which is present 2 years after the initial surgery-cholecystectomy and CBD exploration. They are primary biliary stones. Salmonella cholecystitis (Typhoid Mary-a cook in New York transmitted typhoid through her infected faeces and urine) causes typhoid gallbladder. It can cause acute or chronic cholecystitis. Salmonella itself may predispose stone formation. Patient may be silent typhoid carrier.

I

SUMP SYNDROME It is commonly observed after choledochoduodenostomy rarely after choledochojejunostomy. CBD distal to the choledochoduodenostomy acts as a reservoir with stasis of food particles, bile, stones and sludge. Often it causes cholangitis and narrowing of the stoma of choledochoduodenostomy. Conversion to end-toend Roux-en-Y choledochojejunostomy is required.

Commitment is what transforms a promise into reality.

(")

::c

)':I,

m ::c

...... N

C, Q,)

C" Q,)

C. C.

CD .....

646

Exceptions to the rule are: Double impacted stone-one in CBD and one in cystic duct, with mucocele of gallbladder. Large stone in Hartmann's pouch. Empyema gallbladder with CBD stone.

SURGICAL JAUNDICE (Obstructive Jaundice)

I Definition Fig. 12.53: Technique of choledochoduodenostomy for CBD stones. Note the site of possible occurrence of Sump syndrome.

COURVOISIER'S LAW (SIGN) 'In a patient with jaundice if there is palpable gallbladder, it is not due to stones'.

Fig. 12.54: Courvoisier's law- "ln a patient with jaundice if there is palpable gallbladder it is not due to stones". In stone disease gallbladder is contracted and fibrotic and so nondistensible.

In obstruction due to CBD stone, gallbladder does not distend because it is chronically inflamed, thickened, fibrotic, contracted and nondistensible. In malignancy, like carcinoma of head of the pancreas or periam pullary carcinoma, gallbladder will be distended and palpable to the right of rectus muscle in the right hypochondrium, as non-tender, globular, smooth, soft, dull mass which moves with respiration and with horizontal mobility.

It is the jaundice that develops dueto biliary obstruction, partial or complete or intermittent. It causes conjugated hyperbilirubinaemia. Normal serum bilirubin level is 0.2-0.8 mg%. Sciera! icterus is visible when serum bilirubin level exceeds 2.5 mg%.

I Pathophysiology in Obstructive Jaundice • Aged red cells get lysed in the reticuloendothelial cells and breakdown into haem and globin. Haem is divided into globin and bilirubin. Bilirubin is combined with albumin and transported to liver. In the liver bilirubin get separated from albumin and conjugated to bilirubin glucuronide by glucuronyl transferase. This conjugated bilirubin glucuronide is water soluble and can be excreted in kidney (So in obstructive and hepatic jaundice bile pigment bilirubin is seen in the urine). • This conjugated bilirubin is excreted through biliary canaliculi reaching intestine. In the intestine, it is converted into stercobilinogen and urobilinogen by intestinal bacteria. 70% of this is absorbed in the colon and brought back to liver as enterohepatic circulation. Unabsorbed stercobilinogen colours faeces brown. Circulating urobilinogen is taken up by kidneys for excretion. • If direct bilirubin in the serum is more than 0.4 mg%, then bilirubin is seen in urine. Normal urinary urob ilinogen is 100-200 mg/day. It is absent in obstructive jaundice. Normal faecal stercobilinogen is 300 mg/day. It is also absent in obstructive jaundice. In obstructive jaundice urine contains bilirubin and bile salts but urobilinogen is absent; stool is devoid of stercobilinogen.

Fig. 12.55: Sciera of the patient with obstructive jaundice is greenish yellow in colour.

Obstructive jaundice, often called as surgical jaundice cirrhosis. Acute cholangitis is another complication assooccurs due to intrahepatic or extrahepatic biliary outflow ciated with obstruction of the biliary tract and is the most obstruction. It leads into cholestasis which means conjucommonly seen in stricture, most often at the level of the gated hyperbili rubi naemia; probab ly due to impaired CBD. Bile here is usually sterile. Bileflow stasis bile flow and impaired bile formation . Bile acid secretion colonization and multiplication of concomitant into the gut is impaired causing defective absorption of increased intraductal pressure reflux of biliary contents fat and fat-soluble vitamins like vitamin K; this, in turn bacteremia, septicaemia septic shock. If recurrence of causes poor synthesis of prothrombin and conversion of biliary colic after cholecystectomy occurs, one should think prothrombin to thrombin causing widened PT and PT INR. of possible choledocholithiasis. Factors II, VII, IX, and X are vitamin K-dependent clotting factors. PT indicates the extrinsic pathway of coagulation; Effects of Obstructive Jaundice whereas partial thromboplastin time represents the intrinsic In liver. Enlarged green bile stained liver (hydrohepatosis) pathway. Bile acid stasis causes hepatocytes injury. Fat shows dilated intrahepatic biliary radicles. Once intraductal malabsorption causes steatorrhoea and also deficiencies of CBD pressure increases bile secretion from liver is reduced vitamins A (visual problem, thick skin), D (osteomalacia), E causing formation of 'white bile' in CBD. Biliary cirrhosis (peripheral neuropathy, cerebellar ataxia, posterior column may develop later. dysfunction), K (bleeding tendencies). Cholestyramine and In the biliary tree: Recurrent inflammation-cholangitiscolestipol, used to treat pruritus, bind to bile salts and can fibrosis can occur. exacerbatethese vitamin deficiencies. Persistent cholestasis In bowet. Absence of bile from bowel impairs digestion, may be associated with deposits of cholesterol in the skin reduces fat absorption making faeces bulky and fatty. Vitamin (cutaneous xanthomatosis), occasionally in bones and K absorption is reduced causing fall in prothrombin level peripheral nerves. raising PT-INR. Bilirubinostasis (bile plugs) cause hepatocytes degeneration, Retention of bile salts and bile pigments in blood and body small duct and ductular obstruction, pericholangitis, oedema, bile leak, liver infarction and biliary cirrhosis. fluids occurs. Obstruction may be due to benign conditions like biliary tree Altered coagulation profile; hepatorenal syndrome and renal stones (most common cause) or strictures or sclerosing failure; sepsis. cholangitis; or due to malignant conditions like carcinoma Classification of Causes of of pancreas or cholangiocarcinoma. Extrahepatic obstruction may be luminal (stones, infestations [ascariasis, clonorchis Obstructive Jaundice sinensis and schistosomiasisl) or mucosal/wall (growth, 1. Congenital: Biliary atresia, choledochal cyst. stricture [post-inflammatory, postsurgical or post-RT], 2. Inflammatory: Ascending cholangitis, sclerosing cholangitis. primary sclerosing cholangitis) or external compression 3. Obstructive: CBD stones, biliary stricture, parasitic infestation. (pancreatitis, pancreatic tumou r, compression by nodal 4. Neoplastic: Carcinoma of head or periampullary region of mass). pancreas, cholangiocarcinomas, Klatskin tumour. lntrahepatic cholestasis generally occurs at the level of 5. Extrinsic compression of CBD by lymph nodes or tumours. the hepatocyte or biliary canalicular membrane. Causes include hepatocellular disease (e.g. viral he patitis, druginduced hepatitis/cholestasis (thiazides, chlorpromazine), biliary cirrhosis. In hepatocellular disease, interference in the 3 major steps of bilirubin metabolism- uptake, conjugation and excretion usually occu r. Excretion is the __,..+.- -1- Choledochal rate-limiting step and is usually impaired to the greatest cyst extent. As a result, conjugated bilirubin predominates in ;,+.....;..- -- -- I - Roundworm the serum. in CBD The lack of bilirubin in the intestinal tract is responsible for the pale stools in biliary obstruction. The cause of itching Carcinoma head Pancreatic of pancreas stone (pruritus) associated with biliary obstruction is not clear; may Periampullary be related to the accumulation of bile acids in the skin; or may carcinoma be related to the release of endogenous opioids. Chronic pancreatitis Severe biliary obstruction causes cell damage usually in Fig. 12.56: Different causes of obstructive jaundice (surgical jaundice). 1 month and, if unrelieved , may lead to secondary biliary

I

You are the sole master of your thought processes.

647

C")

::c l>

m

::J:J

..... N

648

• BENJAMIN'S (1983) CLASSIFICATION OF BILIARY • OBSTRUCTION Type 1: Complete obstruction • Tumours-pancreatic, cholangiocarcinoma CBD ligation-iatrogenic • Primary/secondary liver tumours

•

IType 2: Intermittent obstruction

• Choledocholithiasis; Periampullary tumour • Choledochal cyst; Bile duct papilloma • Hemobilia; Duodenal diverticula Type 3: Chronic complete obstruction • Bile duct stricture; Congenital • Traumatic; Post-radiotherapy • Chronic pancreatitis; Cystic fibrosis Type 4: Segmental obstruction • Traumatic; Sclerosing cholangitis • Cholangiocarcinoma, intrahepatic biliary stones (hepatolithiasis)

Clinical features Severe jaundice; Pruritus, more on the back and forearms. r Fever, may or may not be present. r Loss of weight; Loss of appetite. r Pain in right hypochondrium, palpable gallbladder, hydrohepatotic palpable, smooth, soft, non-tender liver are other features. r Courvoisier's law may suggest inflammatory/neoplastic cause. r Charcot's triad/Reynold's pentad as presentation in cholangitis. r Steatorrhoea (more fatty stool) due to improper absorption of fat soluble vitamins.

PTC to decompress, assess proximal dilated obstructed biliary system if ERCP fails; dine polythene catheter can be kept in situ to have biliary drainage; PTC-stenting across the obstruction can be done under image (C-arm) guidance. MRCP-Noninvasive diagnostic tool. It shows 96% sensitivity; 99% specificity. CT scan in case of tumours to assess operability.

r

I Investigations for Obstructive Jaundice Serum bilirubin: Normal value is less than 1.0 mg%. Both direct and indirect bilirubin are assessed. Direct is increased in obstructive jaundice, i.e. conjugated hyperbilirubinaemia. van den Bergh's test is done. Serum albumin, globulin and A:G ratio. Normal S. albumin is more than 3.5 gm%. Prothrombin time: Normal value is 12-16 seconds. It is significant if it is more than 4 from the control or more than one and half times the control. It is corrected by injection vitamin K, 10 mg IM OD for 5 days or by FFP-5-10 units. Serum alkaline phosphatase, SG PT, SGOT, 5' nucleotidase. Total count may be raised with neutrophilia in inflammatory conditions. Serum alkaline phosphatase (ALP) andy gtutamyt transpeptidase (GCT) are relevant enzymes in biliary obstruction ; especially ALP/GCT ratio is more relevant in differentiating between obstructive jaundice and hepatitis. Ultrasound abdomen. ERCP to visualise the site of obstruction, brush biopsy, bile sample for analysis.

Fig. 12.57: CT scan showing hydrohepatosis due to obstruction below, in a patient with obstructive jaundice.

•·· Tumour markers: CA 19/9 is useful for carcinoma pancreas (more than 70 units/L) with 70% sensitivity and 90% specificity. But it may also increase in other causes of biliary obstruction and cystadenoma. Endoscopic US (EUS): It is done through endoscope. It is more accurate in assessing pancreatic mass, staging of the disease, to identify involvement of portal venous system, CBD stones. It is also useful in EUS-guided FNAC, celiac axis neurolysis, EUS-guided immunotherapy. lntraductal US (/DUS): It is very useful in assessing tumour stage, tumour margin in bile duct cancer. It is also used in assessing pancreatic duct to differentiate pancreatic cancer and chronic pancreatitis. CT/MR angiogram or venogram to assess vascularity and portal venous system in malignancy. Urine tests: Fouchet's test for bile pigments, Hay's test for bile salts and test for urobilinogen in urine. Fouchet's test: 10 ml of urine+ 5 ml of BaCl2 + pinch of MgSO4 causes formation of BaSO4 which is filtered over a filter paper and few drops of Fouchet's reagent is added. Green or blue colour signifies presence of bile pigments in the urine. Hay's test for bile salt Sprinkle sulphur to 2 ml of urine. In presence of bile salts sulphur sinks to the bottom. Ehrlich's test 5 ml of freshly voided urine + 1 ml of Ehrlich reagent (p-dimethyl amino benzaldehyde) and wait for 5 minutes. Formation of red colour signifies presence of urobilinogen in urine. Normally it is present in traces; in obstructive jau ndice, it is absent; and in haemolytic jaundice, it is in excess.

IB___ • Proper diagnosis and assessment • Injection vitamin K IM 10 mg for 5 days • Fresh Frozen plasma-often requires 6 bottles or more • Adequate hydration is most important 5/10% dextrose Blood transfusion in case of anaemia Oral neomycin, lactulose Mannitol 100-200 ml BO IV to prevent hepatorenal syndrome Repeated monitoring by doing prothrombin time, electrolytes Antibiotics like third generation cephalosporins. • Calcium supplements as calcium chloride IV • Preoperative decompression is indicated if bilirubin is> 12 mg%, sepsis, hepatorenal syndrome, severe malnutrition or cardiopulmonary disease. • Correction of coagulopathy, prevention of renal failure, infection, J hepatic encephalopathy and electrolyte imbalance (correction of hypoglycaemia and dilutional hyponatraemia due to water retention; avoiding isotonic saline infusion).

••

I•• I

I Treatment of Obstructive Jaundice CBD stones-ERCP stone removal, choledocholithotomy, transduodenal sphincteroplasty, choledochojejunostomy or choledochoduodenostomy. Carcinoma periampullary or head of pancreas-Whipple's operation or triple bypass or ERCP stenting. Biliary stricture-stenting, choledochojejunostomy, Rouxen-Y hepaticojejunostomy. Klatskin tumour- radical resection or palliative stenting. Biliary atresia-Kasai's operation or liver transplantation. Choledochal cyst-excision, hepaticojejunostomy, mucosa! resection. Management of pruritus: Pruritus may be due to retention of bile salts which activates the release of histamine in skin, central mechanism or by release of endogenous opioids. It is often difficu lt to treat. Once cause is treated and obstruction is relieved, pruritus will regress. Drugs and therapies used are-cholestyramine (ion exchange resin binds bile salts in intestine inhibiting their absorption), rifampin , ondansetron, gabapentin, sertraline, ursodeoxycholic acid, antioxidants, phototherapy, plasmapheresis.

CBD STRICTURES (BILIARY STRICTURES)

I Causes

1. Postoperative (80% common) ,.. After cholecystectomy [open or laparoscopic, more common following laparoscopi c (0.8%) than open method (0.35%)]. ,, Biliary surgery; Gastrectomy; Liver surgery. ,, Duodenal and pancreatic surgery. 2. Inflammatory: Stricture following recurrent attacks of cholangitis due to: , CBD stones. , Parasites-Ascaris lumbricoids, Clonorchis sinensis. , Primary sclerosing cholangitis. 3. Malignant: Due to cholangiocarcinoma. 4. Traumatic.

IsI. LowfUMMiiH•iiiiii!MM!111 INMiiiMi!Mii~ CBD stricture with stump (proximal to stricture) > 2 cm II. Middle CBD stricture with proximal stump < 2 cm Ill. Hilar. Confluence of right and left hepatic ducts is intact IV. Separated right and left hepatic ducts V. Stricture involving intrahepatic ducts

I Postoperative Management Monitoring with prothrombin time, bilirubin, albumin, creatinine, electrolyte estimation. FFP or blood transfusion; Antibiotics. Observation for septicaemia, haemorrhage, pneumonia, pleural effusion, bile leak. Care of Hube and drains. T-tube cholangiogram in 10-14 days. TPN, CVP line, nasogastric tube, urinary catheter.

Figs. 12.58A to E: Bismuth classification of stricture CBD.

If there is no struggle, there is no progress. -Frederick Douglas. Try and fail, but don't fail to try. -Stephen Kaggwa

649

Features ,,. Obstructive jaundice; Pain abdomen. ,,. Features of ascending cholangitis. ,,. Profuse persistent bile leak.

SCLEROSING CHOLANGITIS It is fibrous thickening of the CBD and biliary ductular wall, associated with multiple strictures with dilatation in between the strictures. Both extra- and intrahepatic ducts are involved. They have increased risk of developing cholangiocarcinoma.

B Too much traction to the GB during' Fundus first cholecystectomy' Blind application of artery forceps in Calot's to control bleeding Anomalies of biliary tree Inflammatory adhesions at Calot's triangle Injury during other surgery like gastrectomy -- - - - - - - - ~ -

J

Note: Hogarth Pringle's manoeuvre is ideal to control bleeding on table by applying pressure using thumb and fingers over the Foramen Winslow instead of using instruments to prevent stricture.

Investigations ,, Ultrasound abdomen; liver function tests; ERCP. On table cholangiography; MRCP. Treatment: ERCP stenting; Choledochoduodenostomy or jejunostomy; Roux-en-Y hepaticojejunostomy- ideal.

I Types 1. Primary sclerosing cholangitis is one wherein no cause is found and is associated with ulcerative colitis, Sjiigren's syndrome, Crohn's disease, Graves disease. It eventually leads to biliary cirrhosis. Primary sclerosing cholangitis (PSC) is an idiopathic, progressive, chronic, cholestatic pathology with diffuse inflammation, sclerosis, and obliteration of intra- and extrahepatic biliary systems. PSC is associated with hypergammaglobulinaemia and elevated smooth muscle antibodies and antinuclear factor. There will be multi ple areas of strictures and dilatations (1%/year) . It has high risk for cholangiocarcinoma. It could be an autoimmune disease. It is common in HLA/88/DR3 halotype. 2. Secondary sclerosing cholangitis is due to stones, trauma, congenital lesions. AIDS, chemotherapy (5FU) , transplantation, collagen diseases, sarcoidosis, histiocytosis.

I Features Common in young men (70%); Intermittent jaundice. Abnormal liver functions; Weight loss, pain, fever, pruritus Features of ulcerative colitis in case of PSC (70%). PSC may be associated with retroperitoneal fibrosis, mediastinal fibrosis, Riedel's thyroiditis, orbital pseudo-tumour. Hepatic duct confluence is most severely strictured segment in PSC. Investigation: ERCPshows beaded appearance of biliary tree; LFT is altered; Liver biopsy is needed to identify the severity of hepatic fibrosis. Treatment: ,, Stenting; T-tube drainage. , Large dose steroids; lmmunosuppression therapymethotrexate, azathioprine, tacrolimus, cyclosporine; Ursodeoxycholic acid is beneficial , Liver transplantation; Median survival in PSC after diagnosis is 10 years.

GALLBLADDER POLYP

Figs. 12.60A and B: CBD stent and imaging after placement.

Its incidence is 5% in routine US abdomen; 10% in cholecystectomy tissue specimen. It is usually less than 10 mm in size, pedunculated appearance. Sessile polyps are often more than 10 mm in size. 30% are multiple. Gallbladder polyp more than 10 mm in size, associated with gallstones, age above 60 years, symptomatic, sessile polyp and multiple polyps-are indications for surgical intervention.

CT scan should be done if malignancy is suspected on US. It is treated by laparoscopic cholecystectomy. Histology is a must to confirm benign nature and to rule out carcinoma.

BENIGN BILIARY PAPILLOMA It is the most common benign biliary tumour. 50% occurs close to am pulla presenting as obstructive jaundice. Benign adenoma (soft), benign inflammatory pseudotumour and cholangiocarcinoma-are differential diagnosis. ERCP, CT scan, EUS, LFT- are the investigations. Treatment-papillotomy, wide local excision.

CARCINOMA GALLBLADDER It is more common in India (Patna) and Asian countries. It is also common in Chile. It is common in females and elderly. Male:Female = 1:3.

I Aetiologies for Carcinoma of Gallbladder 3% of gallstones with cholecystitis will develop carcinoma of gallbladder. 90% of carcinoma of gallbladder is associated with gallstones. Risk of developing carcinoma in gallstone disease is 7-10 times more than general population. Relative risk is less if stone size is less than 2 cm; if stone size is 2- 3 cm in size, it is 2.5; it is 10 or more if stone size is more than 3 cm. Choledochal cyst, anomalous pancreaticobiliary duct junction (20%), cholesteroses of gallbladder, gallbladder polyp more than 1 cm in size or more than 3 in number or adenomatous polyp, PSC. Chronic typhoid carriers, carcinogens, inflammatory bowel disease, hepatitis B and hepatitis C virus infection. Porcelain gallbladder is more prone for malignant transformation (25%) and 90% of them are inoperable tumours. Nitrosamines. Polypoid lesions (GB polyp), xanthogranulomatous cholecystitis.

I Spread of Carcinoma Gallbladder Direct spread to liver (segment IV and V), bile duct, duodenum, colon and kidney. Lymphatic-lymph node of Lund, periportal nodes, peripancreatic and periduodenal nodes. Blood spread-to liver, lungs and bones. Perineural spread is also known to occur.

I Features of Carcinoma of Gallbladder Pain in right hypochondrium, mass in right upper abdomen which is hard and nontender (gallbladder mass). Jaundice is common. Significant weight loss in short duration, anorexia Acute presentation of cholecystitis. Palpable nodular liver secondaries, ascites. It is common in places where there is more prevalence of gallstone disease-Patna, Bihar It is common in females. Incidentally confirmed by histological report as carcinoma gallbladder after cholecystectomy for chronic cholecystitis. Three clinical presentations: (1) Clinically obvious type with pain, obstructive jaundice, mass. (2) Early GB cancer mimics GB stone disease. (3) Atypical as unusual features.

I Gross Types of Carcinoma Gallbladder Polypoid/papillary- better prognosis. Scirrhous/nodular. Proliferative/infiltrative.

I Microscopy Commonly it is adenocarcinoma (90%); occasionally squamous cell carcinoma, adenosquamous or carcinoid tumour can occur. 25% show only localised disease; 35% have lymph node spread; 40% have distant spread at thetime of first diagnosis. It is very aggressive tumour.

651

Figs . 12.61A to C: Specimen of gallbladder after extended cholecystectomy. Note the liver margin cleared of carcinoma, stone in the gallbladder and lymph nodes cleared.

It is not enough to have a good mind; the main thing is to use it well... ! -Rene Descartes

C, I» CT I»

a. a.

.... Cl)

...

Cl)

en

::I

Cl)

c

Investigations , Ultrasound abdomen. , CT abdomen to see operability. , US-guided FNAC; Liver function tests; MRCP; Laparoscopy. , CA 19-9 is elevated in 80% of cases.

T

TNM staging for gallbladder cancer (AJCC, 8th edition, 2018)

Tumour(T)

Metastases (M)

TX Primary tumor cannot be assessed MO - No distant spread. TO No evidence of primary tumor M1 Distant spread. Tis Carcinoma in situ T1 Tumor invades lamina propria or muscular layer T1a Tumour invades lamina propria 11 b Tumour invades muscular layer T2a Tumour invades the perimuscular connective tissue on the peritoneal side without involving the serosa. T2b Tumour invades the perimuscular connective tissue on the peritoneal side without involving the liver. T3 Tumour pertorates the serosa (visceral peritoneum) and/or directly invades the liver and/or one other adjacent organ or structure, such as the stomach, duodenum, colon, pancreas, omentum, or extrahepatic bile ducts T4 Tumour invades main portal vein or hepatic artery or invades at least two extrahepatic organs or structures Nodes (N)

Staging

Nx Regional lymph nodes cannot be assessed NORegional lymph node metastasis N1 Metastases to 1-3 lymph nodes N2 Metastases to ~4 lymph nodes

Stage 0-Tis, NO, MO Stage I - T1 , NO,MO Stage IIA - T2a, NO, MO; 11B - T2b, NO, MO Stage Ill A- T3, NO, MO Stage Ill B - T1 to T3, N1 , MO Stage IV A- T4, NO or N1 , MO Stage IV B-Any T, N2, MO; Any T, any N, M1 Note: Stage I is Localised; Stages II to IV Bare unresectable.

IB

should be done to identify for the existence of microscopic tumour. If present, CBD resection and hepaticojejunostomy is done. Open approach rather than laparoscopic is ideal for carcinoma gallbladder. Hemihepatectomy with cholecystectomy with nodal clearance. During laparoscopic cholecystectomy, any suspicious of GB cancer, procedure should be converted into open cholecystectomy.

Fig. 12.62: Extended cholecystectomy technique.

If patient has undergone laparoscopic cholecystectomy and histology confirmed carcinoma, then staging should be done. All port areas should be re-excised to prevent port site recurrence. Often extended resection of segment IV and lymph nodes may be needed. Spillage of bile during laparoscopic cholecystectomy is common (30% in non-malignant GB, 50% in carcinoma GB). Chemotherapy either systemic or intra-arterial, and adjuvant radiotherapy but with poor success rate.

I Prognosis Overall prognosis for carcinoma gallbladder is poor due to early spread and aggressive nature of the tumour. 5-year survival is only 5%. Muscle invasion, nodal and distant spread carry poor prognosis. In stage T1 simple and extended cholecystectomy will not make difference in prognosis. In T2 , stage extended cholecystectomy is very much beneficial which gives 60% 5-year survival rate. T3 and T4 carry poor prognosis.

iiiiWiii§@i5'ii¼iihiM~§&ilf:i@+ili-

Stage I - Intramural Stage IV- Spread to cys= Stage 11 - Spread to muscularis node of Lund propria Stage V - Direct spread to adjaStage Ill - Spread to serosa cent organs/metastases -- --- --- - - ---------'

I

I Treatment Cholecystectomy with resection of liver segments IV and Vextended cholecystectomy with perihepatic nodal clearance. At least 2 cm margin in the liver from the gallbladder bed should be cleared. All pericholedochal lymph nodes should be removed. Frozen section biopsy from cystic duct stump

Figs. 12.63A and B: {A) Gallbladder showingproliferative carcinomatous

lesion; (B) Gallbladder showing scirrhous carcinoma with pre-existing gallstones and cholesteroses.

CHOLANGIOCARCINOMA (Bile Duct Carcinoma) It is associated with sclerosing cholangitis, clonorchiasis infestation, Caroli's disease or choledochal cyst. It is an aggressive adenocarcinoma which presents as obstructive jaundice. It commonly occurs at the hepatic duct confluence.

I Risk Factors for Cholangiocarcinoma Primary sclerosing cholangitis (PSC): Here commonly it is extrahepatic, usually at the confluence. Choledochal cyst; Hepatolithiasis. Hepatitis B and Care risk factors for intrahepatic cholangiocarcinoma. Lynch syndrome II. Multiple biliary papillomatosis. Previous biliary enteric anastomosis. Clonorchis sinensis infestation. Thorotrast, nitrosamines, dioxin.

T

I

Different classifications

Anatomical: 1. lntrahepatic-10%. (2) Perihilar-65%. (3) Distal-25%. Pathological: 1. Sclerosing (80%). (2) Nodular. (3) Papillary. Based on extent: 1. Upper third- from porta to cystic duct-50%. 2. Middle third- from cystic duct to upper margin of first part of the duodenum- 25%. 3. Lower third-from upper margin of first part of duodenum to ampulla- 20%. 4. Diffuse- 5%. Classification of perihilar cho/angiocarcinoma (Bismuth Corlette): Type I: Below the confluence of RHO and LHD Type II: Reaching the confluence of RHO and LHD Type Ill: Involving CHOwith RHO (IIIA) or LHD (I11B) Type IV: Involving confluence and both RHOand LHD or multicentric tumour

I Features Main presentation is painless obstructive jaundice of short duration. Palpable liver either smooth and soft (hydrohepatotic) or hard nodular (secondaries). It is common in men (1.5:1). Weight loss and anorexia is typical and significant. Investigations: , ERCP and choledochoscope; Liver function tests; PTC. ;.. Ultrasound abdomen, CT scan. MR scan-Best investigation. MRCPto see duct, MRI to see nodes; MR angiogram to see vascularity.

Assessment should be done to check the operability of tumour by-assessing the sp read in bilateral hepatic duct up to secondary radicles, both hepatic arterial spread, portal vein encasement, hepatic lobe atrophy, distant spread.

653

C"'l

:::c American Joint Cancer Committee/Union for International Cancer Control 8th edition , TNM staging for perihilar cholangiocarcinoma Primary tumor (T) Regional lymph nodes (NJ

T

Tx Primary tumor cannot be assessed TO No evidence of primary tumor Tis Carcinoma in situ T1 Tumor confined to the bile duct, with extension up to the muscle layer or fibrous tissue T2A Tumor invades beyond the wall of the bile duct to surrounding adipose tissue T2B Tumor invades adjacent hepatic parenchyma T3 Tumor invades unilateral branches of the portal vein or hepatic artery T4 Tumor invades main portal vein or its branches bilaterally; or the common hepatic artery; or the second-order biliary radical bilaterally; or unilateral second order biliary radicals with contralateral portal vein or hepatic artery involvement Note: TNM staging defers for intrahepatic, perihilar and distal cholangiocarcinoma.

NX Regional lymph nodes cannot be assessed NO No regional lymph node metastases N1 Regional 1- 3 lymph nodes metastases present (including nodes along the cystic duct, common bile duct, hepatic artery, and portal vein) N2 Metastases to nodes of N1 but 2!:4 nodes Distant metastases (M) MO No distant metastases M1 Distant metastases present Staging Stage OTis NO MO Stage I T1 NO MO Stage II T2A-B NO MO Stage IIIA T3 NO MO Stage 11IB T4 NO MO Stage III CAny T N1 MO Stage IVA Any T N2 Mo Stage IVB Any Any N M1

I Treatment When operable, portal region clearance with hemihepatectomy can be done. lntrahepatic type is treated with hemihepatectomy. Perihilar type is treated with hemihepatectomy or extensive bile duct resection, nodal clearance, caudate lobe removal , cholecystectomy. Distal tumour is treated with Whipple's pancreaticoduodenectomy. Most often it is inoperable. Stenting can be done to relieve jaundice, through PTC or ERCP or on table. Chemotherapy-5 FU ; Gemcitabine, cis platin. Chemotherapy, with external beam radiotherapy (ERST).

KLATSKIN TUMOUR It is cholangiocarcinoma at the confluence of the hepatic ducts and common hepatic duct above the level of the cystic duct (20% of cholangiocarcinomas).

You are the sole master of your thought processes.

>

""C

-I

m

:x,

...... N

C, QI

Cl" QI

a. a.

...

CV

654

...

a, c,,

::::s

(I)

Klatskin tumour is classified as 4 types-I: Just at or below the confluence; II: At the confluence; Ill: At the confluence extending along the RHO; IV: At the confluence extending along the LHD. It causes obstructive jaundice with hydrohepatosis without enlargement of gallbladder. Management is like cholangiocarcinoma. Hilar resection with hepaticojejunostomy. Segment Ill left hepaticojejunostomy-Blumgart's. Longmire's left hepaticojejunostomy after left liver lobe resection. Cattell's hepaticojejunostomy. Smith's mucosa! graft hepaticojejunostomy. Palliative PTC with stenting/ERCP stenting, intraoperative stenting. Metal stents show long period of patency (12 months) than polyethylene stents (4 months).

BILIARY FISTULAS

I Types

a. External: Usually occurs as a complication of surgery following: Gastrectomy. Cholecystectomy open or laparoscopic. CBD surgery; Pancreatic surgery. b. Internal: Cholecystoduodenal fistula causing gallstone ileus. Cholecystoenteric fistula. Cholecystocholedochal fistula. External biliary fistulas are difficult to manage, often dangerous, but many a times resolve spontaneously. Investigations: Fistulogram; ERCP; electrolyte estimation; liver function tests. Treatment Total parenteral nutrition (TPN). Antibiotics; Blood transfusion; Electrolyte management. Care of the fistula wound with regular dressing, using zinc oxide cream has to be done to protect the skin. Later continuity of biliary system has to be restored by open surgery or through ERCP and stenting.

HEMOBILIA

Figs. 12.64A and B: (A) Resected specimen of Klatskin tumour

(Coultesy: Dr Arunkumar, MCh); (B) Klatskin tumour.

m

CHOLANGIOCARCINOMA

• It is an uncommon tumour of elderly. 1 in 100,000 is the incidence • Primary sclerosing cholangitis is the commonest association. 20-fold increased risk • Choledochal cyst, Caroli's disease, pyogenic cholangiohepatitis, Clonorchis sinensis, opisthorchis viverrini infestation, hepatolithiasis, hepatitis C, Lynch syndrome II, bile duct adenoma and papillomatosis are other causes • Chemical carcinogens like thorium, nitrosamines, diosmin also can cause lntrahepatic cholangiocarcinoma is 10% • 60% cases are proximal to cystic duct CBD junction • Features are jaundice, pain, hepatomegaly, cachexia, early satiety and weight loss • It is adenocarcinoma type • Lymph node spread is common • Resection along with liver/resection along with pancreas are surgical options • But surgical resection is possible only in 5% of cases • Stenting/bypass are palliative options • Doxorubicin, cis platin and 1131 anti-CEA antibodies are adjuvant therapies • Condition has got 90% mortality in one year

It is bleeding commonly from the liver or occasionally from the gallbladder into the biliary tract. There is abnormal communication between a blood vessel and a bile duct or any part of the biliary tree.

I Causes Accidental trauma, iatrogenic trauma (50%). In accidents hemobilia is more commonly caused by blunt trauma than by penetrating one. Percutaneous diagnostic and therapeutic procedures. Vascular diseases of the hepatic artery-10%. Malignant liver diseases > common (5%) than benign. Portal hypertension. Parasitic liver diseases like hydatid disease. Gallstones rarely erode into hepatic artery causing lifethreatening hemobilia. Arterial hemobilia is more common. Portal venous hemobilia is rare. Hepatic artery aneurysm is common cause.

I Features Pain which is colicky in nature; Obstructive jaundice. Haematemesis and melaena. Blood and necrotic material drains into the biliary tree causing gastrointestinal bleeding. As bile interferes with coagulation a fatal haemorrhage can occur.

655

Early rapid presentation can occur; but delayed, after weeks/ months/years, presentations are known to occur which is often difficult to suspect and diagnose. These recurrent clots can cause pancreatitis, cholangitis, anaemia, cholecystitis.

'B

!

!II :

1. Jaund;ce-60%;

2. Pa;n-70%

3. Upper GI bleed with melaena (90%), haematemesis (60%) All three features of triad are seen only in 20% of cases.

Investigations ,, LFT; US abdomen. ,. Selective arteriography-test of choice in detecting bleeding site in 90% of cases. CT angiogram is useful. , Upper GI scopy-useful only in 10% of cases. Note: ERCP is usually not indicated.

Treatment: Aim: To stop bleeding and to relieve biliary obstruction. , Antibiotics; Blood transfusions. , Selective arterial embolisation. Transarterial embolisation (TAE) shows 90% success rate. It is the choice therapy with least morbidity. Coils and glue are used. , Surgical intervention is only rarely indicated in failed TAE. Laparotomy, ligation of bleeding vessel or hepatic artery, excision of aneurysm, hepatic resection-are the procedures done. Hemobilia is often a fatal condition. Note: Bilhemia: It is different very rare entity wherein bile flows into the

hepatic or portal venous system due to raise in intrabiliary pressure more than that of portal or hepatic venous pressure. It may be due to trauma, iatrogenic or gallstone eroding into the venous system. Large quantity of bile enters the venous system and later to lung becoming fatal. If flow is low which is common, subsides spontaneously. ERCP is diagnostic. Treatment is stenting. Septicaemia and mortality is common.

Fig . 12.65: Demonstration of white bile on table. It is not white but opalescent. It is not bile but it is mucus.

....

--

Cl)

-

~--

• Diabetic patients; Congenital haemolytic anaemia Patients who has underwent bariatric surgery

I Indications Gallstones-symptomatic. Cholecystitis-acute, chronic; Acalculous. Empyema; Mucocele gallbladder.

I Approach Open .,, Right subcostal incision (Kocher's). ,. Right paramedian; midine incision. ,. Horizontal incision. ,, Mayo-Robson incision. Laparoscopic approach. Kocher's incision (open method)

.j '°"

I

~5 mm,/port , • l :;

If

~7~;r•~wp, Rob~o~

It is double misnomer. It is neither white nor bile. It is opalescent. It is mucous secreted by the lining of biliary tree. It signifies severe obstruction due to stone (impacted in the CBD), or carcinoma head of pancreas or periampullary region. It is on table finding during surgery. It means liver is unable to secrete bile due to raised intraductal pressure, and so can anticipate hepatic failure. Indicates a poor prognosis.

Cl.. Cl..

PROPHYLACTIC CHOLECYSTECTOMY IS DONE IN

Mayo-1

WHITE BILE

Q)

It is the surgical removal of gallbladder.

B

:'

inc1s1on ;; Midline ---------t

Q)

er

CHOLECYSTECTOMY -

C')

5 mm port

10 mm port

and B: (A) Incisions for open cholecystectomy and laparoscopic cholecystectomy; (B) Kocher's incision used for open cholecystectomy. Figs . 12.66A

Love all, trust a few, do wrong to none. -William Shakespeare It is the quality of our work that will please god and not the quantity. -Mohandas Gandhi

656

OPEN APPROACH CHOLECYSTECTOMY

I Technique After opening the abdomen, colon is pushed downwards and stomach medially. Duct-first method: Here Calot's triangle is dissected. Cystic artery is identified and ligated. Cystic duct is ligated close to the gallbladder. Gallbladder is separated from gallbladder fossa and removed. Haemostasis is maintained. Fundus-first method: It is done in difficult gallbladder due to dense adhesions. Fundus is separated from the liver bed. Dissection is carried proximally until cystic duct and cystic artery are identified, which are then ligated.

Drain is placed, which is removed after 72 hours. On table cholangiogram is a must after cholecystectomy.

IComplications which can occur in both:

Infection and subphrenic abscess • Bleeding from cystic artery, and from liver bed Injury to CBD or hepatic duct Bile leak and biliary fistula formation Biliary stricture formation • Injuries to colon, duodenum, mesentery

LAPAROSCOPIC CHOLECYSTECTOMY It is the most popular method to remove gallbladder. It is the gold standard treatment for gallstone. Position: Supine head up and right up; General anaesthesia. Nasogastric tube and Foley's catheter is placed.

I Ports 10 mm port in umbilicus to pass 10 mm telescope. 10 mm port in mid line epigastrium as working channel. Two 5 mm ports at midclavicular and anterior axillary line in subcostal region.

I Procedure

Fig. 12.67: Ligation of cystic duct and cystic artery while doing cholecystectomy.

I Complications of Cholecystectomy Complications can occur either in open method or in laparoscopic method. Open method is done through either right paramedian incision or Kocher's incision (right subcostal).

Fig. 12.68: Diagram showing the partial cholecystectomy wherein GB is removed at Hartmann's pouch, distal to Calot's triangle. It is done when there is dense adhesions.with difficult surgical plane.

After creation of pneumoperitoneum with 12- 14 mm pressure, 1O mm umbilical port is inserted. Telescope is passed. Under vision remaining ports are passed. With lateral 5 mm port, gallbladder grasper forceps is passed and fund us of gallbladder is held and pushed up towards the diaphragm. With middle 5 mm port grasper is passed to hold Hartman n's pouch. With 10 mm port dissector is passed using reducer. Calot's triangle is dissected. Cystic duct is identified. Adhesions are released. First posterior dissection is completed. Cystic artery is above and deep to cystic duct. Cystic duct is clipped or ligated. Cystic artery is also clipped. Gallbladder is dissected off the liver bed using cautery (hook/ spatula)/harmonic scalpel. Gallbladder is removed through 10 mm working port with reducer or using a sterile bag. Any bleeding points are coagulated. If needed, saline wash is given to the bed. If infected, if gallbladder is opened, if there are adhesions, if there is oozing from gallbladder bed, a tube drain is placed through lateral 5 mm port. All ports are removed. Umbilical port is sutured in layers. Other ports are sutured. Patient is asked to take oral food in 24 hours and can be discharged in 24-48 hours.

Fig. 12.69: Laparoscopic cholecystectomy. Applying clip to cystic duct is shown.

SINGLE INCISION LAPAROSCOPIC SURGERY (SILS) IN CHOLECYSTECTOMY

Fig. 12.70: Rouvier sulcus on the undersuface of liver is a guideline

during laparoscopic cholecystectomy wherein dissection in Calot's should be always kept in front of it to avoid CBD/hepatic duct injury. Note:

• Cystic artery should be clipped first. • When clipping of the cystic duct is difficult due its wide nature or due to fibrosis, then intracorporeal or extracorporeal knots should be placed using vicryl. Care should be taken not to injure the CBD. • On table cholangiogram should be done after cannulating the CBD through cystic duct when in doubt (both in open or laparoscopic method) to confirm that CBD is normal (without any stones). ERCP may be indicated if there are CBD stones. • When problem arises one should not be hesitant to do conversion into open cholecystectomy. Indications for conversion are-uncontrolled bleeding; dense adhesions; indistinct anatomy; suspected anomalies; suspected CBD injury. Conversion rate is 2-20%. Problems ;.. Difficult Calot's triangle; Dense adhesions. ;.. Bleeding; Anomalies of cystic duct, cystic artery. Relative contraindications: End stage cirrhosis, ascites or portal hypertension; Cholangitis-Cholecystectomy should be done after the control of cholangitis; CBD stones-Here, initially ERCP and stone extraction is done. Complications , Bile duct injury-0.8%; Bleeding; Bile leak. ,.. Infection, cholangitis, Septicaemia; subphrenic abscess formation. > Injury to colon, duodenum, mesentery.

SJLS is an advanced minimally invasive surgical procedure wherein surgeon operates exclusively through a single umbilical entry port. It is also called as single port access surgery (SPA), one port umbilical surgery (OPUS), and single port incision less conventional equipment-utilising surgery (SPICES), natural orifice transumbilical surgery (NOTUS). It needs general anaesthesia, specialised umbilical large trocars which accommodates working instruments along with flexible laparoscope, rotatable instruments, articulating handles, harmonic scalpel. Here through a large 2.5 cm umbilical vertical incision dissection is done by open method to reach peritoneal cavity. Specialised port in which one can pass 1O mm telescope and two 5 mm instruments for work is used. Instruments are angled and flexible to meet the ergonomic principles to certain extent. Dissection of gallbladder is done in similar fashion like four- port technique. Specimen is easily retrieved through umbilical port as it is wide enough. If difficulty arises any time, one can add additional ports as required.

Figs. 12.72A and 8: Single incision laparoscopic surgery

(SILS) for cholecystectomy.

Fig. 12.71: Post-cholecystectomy bile leak with fistula. It is common

and problematic complication after cholecystectomy.

Advantages: There is no visible scar like atraditional multiport; Faster recovery time, early return to work; Cosmetically better. Disadvantages: Expensive trocars and instruments-cost factor; Skilled work, learning curve; Dissection against normal surgical ergonomics. Complications: Umbilical wound pain, infection; Umbilical hernia; Because of limited visibility time consuming; During learning curve complications of cholecystectomy and conversion rate may be more.

Every day is little life. Live it to its fullest.

657 n

:::c

>

m

:a ...... N

C') I»

C" I» C. C. CD

...

658

Note: Waltman-Walter syndrome-. It is compression of IVG due to collection

of fluid in subphrenic space during post-cholecystectomy period. It mimics coronary thrombosis.

BILE DUCT INJURIES

I Causes

Cholecystectomy-open or laparoscopic. Trauma; lnstrumentation-ERCP, baby endoscope, choledochoscope. Other surgeries-pancreatic, duodenal, gastric. Anomalous biliary system increases the risk of injury.

I Classifications STRASBERG CLASSIFICATION

• Class A. Bile leak from the cystic duct or an accessory duct showing continuity with the common bile duct Class B: Section/injury of an accessory duct/sectorial ducts aberrant right hepatic duct with no continuity with the common bile duct with occlusion Class C: Leak/open drainage from a sectorial duct/aberrant right hepatic duct with no continuity with the common bile duct Class D: Partial section/lateral injury of an extrahepatric duct bile duct with no complete loss of continuity with the rest of the bile duct system Class E: Complete section/circumferential injury of the bile duct with subtypes according to the length of the stump at various levels - E1- Stricture/injury at more than 2 cm distal to bifurcation. E2 - Stricture/injury less than 2 cm distal to bifurcation. E3- Stricture/injury at bifurcation E4 - Stricture/injury involving right and left hepatic ducts ES - Complete obstruction of entire bile duct Note: Only right and left partial injuries are not included in this classification Note:

Other classifications used are:

• Stewart-Way classification: Class I: Incomplete. Class II: Lateral with

bile leak and stenosis. Class Ill: Complete transection of common bile duct (commonest 60%). Class IV: It is duct branch injuries with injury of right hepatic artery. • Hannover classification: Type A: Cystic duct or gallbladder bed leak. Type B: Incomplete or complete stenosis. Type C: Lateral tangential injuries. Type D: Complete bile duct injury with vascular injuries. Type E: Late bile duct stenosis. • Bismuth classification is discussed under biliary stricture earlier.

Presentations , Fever, pain, features of bile stasis like cholangitis, jaundice. ,, 10% of laparoscopic bile duct injuries are identified on table; 30% with bile leak; 25% with obstructive jaundice; 30% with cholangitis. Patients with bile leak present early; with formed biliary stricture presents with jaundice and cholangitis months or years later. Complications ,. Biliary fistula with electrolyte imbalance. ,. Biliary peritonitis, septicaemia.

,, Subphrenic abscess formation. ,. Biloma formation. :.- Severe malnutrition, deficiencies. , Late-biliary cirrhosis, portal hypertension. Investigations , LFT, coagulation profile. ,. US abdomen, fistulogram. ,, Dynamic CT scan will delineate the anatomy of injury. , ERCP is useful if injury is only partial where stenting can also be done. , PTC is the investigation of choice to identify site, nature, extent of stricture. It also facilitates drainage and stenting. ,, Hepatobiliary scintigraphy may be useful. , MRCP may be useful to identify ductal anatomy. ,. CT arteriography is useful to identity associated hepatic artery or portal vein injury which is 20% in association with CBD injury.

I Management General: antibiotics, nutrition, TPN. Conservative: Small partial injury to CBD may resolve spontaneously or with the help of ERCP, sphincterotomy and stenting is done. Management of biliary duct injury on table (during laparoscopic cholecystectomy/open): Conversion into open surgery with a lengthy subcostal/bucket handle incision. lntraoperative cholangiogram should be done. Partial injury of CBD less than 30% of circumference is treated with primary repair with a T-tube in place. Extensive injury more than 30% of the circumference or cautery injury or complete CBD transection should be treated with Roux-en-Y choledochojejunostomy. Isolated hepatic duct injury less than 3 mm in size should be ligated. More than 3 mm in size should be reimplanted or Roux-en-Y hepaticojejunostomy should be done. If facility for surgical repair is available, patient should be sent to a higher centre after placing a drain with abdomen closure. Management of bile duct injury identified at a later period: In such a situation it is better to wait for 6 weeks for the inflammation to subside which facilitates easier identification of proximal CBD. After proper assessment of injury, Roux-en-Y choledochojejunostomy or Roux-en-Y hepaticojejunostomy is done. Hepp-Couinaud approach: Here hilar plate is meticulously dissected; left hepatic duct is anastomosed to Roux jejunal loop to create hepaticojejunostomy often with creation of proximal 'access loop' for future endoscopic approach. Complications are-recurrent cholangitis, bile leak from stoma, hemobilia, stenosis of biliary enteric anastomotic site (10%, occurs often after many years).

POST-CHOLECYSTECTOMY SYNDROME (15%) Recurrent, new or persistent symptoms after cholecystectomy in patients who have no demonstrable abnormality is called as post-cholecystectomy syndrome.

It may be due to loss of reserving function of the gallbladder or continuous bile flow into the duodenum may be causing oesophagitis/gastritis or diarrhoea and colicky pain. 20% causes are other than due to hepatopancreatic biliary problems. However importance lies in ruling out the other causes of the symptoms like peptic ulcer, hiatus hernia, pancreatic diseases, residual CBD stone, cystic duct remnant, papillary stenosis. True post-cholecystectomy syndrome is treated with proper counseling, psychiatric evaluation and drug therapy. It should be evaluated thoroughly.

T

Causes ol post-cholecystectomy syndrome

Gallbladder

Residual or reformed gallbladder Stump stones in cystic duct Neuroma in the stump Slipped stones into the peritoneum Clips applied to cystic duct

Biliary tree

Residual stone in CBD Biliary dyskinesia Stricture Dilatation without obstruction Fistula Cholangitis Malignancy

Liver

Fatty liver, hepatitis, cirrhosis Sclerosing cholangitis

Pancreas

Pancreatitis, stones Carcinoma pancreas Sphincter ol Oddi dyskinesia

GIT

Oesophagitis / hiatus hernia/ achalasia cardia Duodenal diverticula IBS, constipation, incisional hernia Mesenteric ischaemia

Cardiac

Coronary heart disease

Others

Adrenal tumour Thyrotoxicosis lntercostal neuralgia Arthritis Psychiatric diseases

BILIARY DYSKINESIA Biliary dyskinesia is motility disorder of either gallbladder or sphincter of Oddi. Patient presents with features of biliary colic without any evidence of gallstone disease.

It can be: Gallbladder dyskinesia: ;.. Here CT scan, gastroscopy, ERCP are normal. CCK is injected intravenously after filling gallbladder with radio• nuclide labeled Tc99m, gallbladder ejection fraction is assessed after 20 minutes. If it is less than 35%, then it is called as gallbladder dyskinesia. It is treated with laparoscopic cholecystectomy. Sphincter of Oddi dysfunction: It is also known as biliary sphincter dyskinesia or pancreatic sphincter dyskinesia. It is benign acalculous obstruction to bile and/or pancreatic flow across sphincter of Oddi causing cholestasis, pancreatitis and pain. ,. It may be due to trauma, congenital anomalies. ,, In occurs in 1% of cholecystectomy patients. Condition is common in females. ,. Normally CCK relaxes sphincter by decreasing its pressure. After IV injection of CCK, if CBD diameter becomes more than 12 mm or if there is increase in CBD diameter in response to CCK on US evaluation it suggests dysfunction of sphincter of Oddi. If basal sphincter pressure, on study, increases more than 40 mmHg it also suggests the same. , Milwaukee's classification: Type I: Any one of the following features-unexplained biliary pain for more than 6 months after cholecystectomy, ERCP showing CBD >12 mm, delayed bile drainage >45 minutes, altered liver enzymes. Type It. Unexplained biliary pain for more than 6 months after cholecystectomy + with any one of the other criterra. Type Ill: Only unexplained biliary pain for more than 6 months after cholecystectomy, without any of other criteria. ,. ERCP/MRCP/sphincter of Oddi manometry, IV cholangiography are the investigations. ,. Treatment Endoscopic sphincterotomy, botulinum toxin injection into the sphincter, nifedipine, transcutaneous electric nerve stimulation to raise serum vasoactive intestinal peptide (VIP) level which decreases the sphincter pressure.

When a man works for an ideal, he becomes irresistible. Your purpose is more powerful than anyone's opinion.

659

C, I» CT I» Cl. Cl. CD

....

Chapter

Spleen

....

..

I Blood Supply

( iHAPTE R OUTL INE

Splenic artery is the branch of coeliac artery but may arise from aorta or superior mesenteric artery, blood flow is 300 mUmt. Splenic vein joins the superior mesenteric vein at right angle behind the neck of pancreas to form the portal vein. Splenic parenchyma contains white pulp and red pulp. White pulp lies in centre surrounding the central artery, which is a branch of trabecular artery. It is made up of lymphatic nodules with germinal centres and periarterial lymphatic sheaths with a network containing lymphocytes and macrophages. White pulp is surrounded by marginal zone which contains end arteries from central and peripheral peniciliary arteries. Marginal zone contains marginal sinus which filters the materials from the white pulp. lmmunog lobulins secreted by white pulp enter marginal zone and into main bloodstream. Red pulp is located outer to marginal zone. Red pulp contains cords and sinuses. Central artery gives reticular SURGICAL ANATOMY branches which open into these sinuses and cords wherein particles are phagocytosed. Commonly central artery eventuIt is a wedge-shaped organ lying mainly in left hypochonally ends in these cords and sinuses. Few end branches of the drium, along the long axis of 10th rib. central artery directly enters the pulp vein. Cords and sinuses Hilum of spleen transmits splenic vessels and nerves. The visceral surface is related to stomach, splenic flexure of eventually drain into pulp vein. Blood circulating through these cords and sinuses are called as 'open' circulation (90%). Blood colon, kidney. Spleen in English means " ill temper'. Galen called it as organ passing through white pulp but not entering these cords and sinuses are called as ' closed' circulation (10%). of mystery. Spleen is palpated under left costal margin during inspiration. It weighs 100-250 gram; it is 10 x 7 x 3 cm in dimension. It has to enlarge 2-3 times its normal size to become palpable.

..• •

.... .. ..•

Su rgical Anatomy Functions of the Spleen Splenunculi Splenic Injury Atraumatic Rupture of Spleen Splenomegaly Hereditary Spherocytosis Immune Haemolytic Anaemia Thalassaemia Sickle Cell Disease

..

..• •.. .. •

Idiopathic Th rombocytopaenic Purpura Thrombotic Th rom bocytopaen ic Purpura Splenectomy Overwhelming Postsplenectomy Infection Splenic Artery Aneurysm Splenic Abscess Hypersplenism Splenic Cyst

I Ligaments of Spleen

Spleen is suspended by two ligaments (a) lienorenal ligament, (b) gastrosplenic ligament. Lienorenal ligament transmits blood vessels to spleen. The tail of pancreas lies in this ligament, which can be damaged during splenectomy. The gastrosplenic ligament contains short gastric vessels which supply the left half of greater curvature of stomach. Phrenicocolic ligament comes in contact with lower pole of spleen, which may be damaged during mobilization of splenic flexure of colon.

FUNCTIONS OF THE SPLEEN Spleen has got two groups of functions: 1. Cellular function. Removal of non-deformable intracellular substances from deformable cells is called as "pitting''. Heinz bodies/ Howe/1Jolly bodies, Pappenheimer siderotic bodies are removed by this method from red blood cells (RBCs). Post-splenectomy patients will show these bodies in the RBCs in peripheral smear. In splenunculi, these bodies will be absent even after splenectomy.

Removal of aged/abnormal red cells is done by a process of "culling". RBCs which loose osmotic balance and membrane integrity is called as non-deformable and they are removed by this method.

SPLENUNCULI (30%)

661

m

These are single or multiple accessory spleens. SITES

Hilum of spleen (50%); Near splenic vessels Tail of the pancreas (30%) Splenic ligaments-gastrosplenic/splenorenal Mesocolon; Greater omentum.

Red pulp Trabecular Marginal zone Cord

Cl)

-cs

Cl) Cl)

Pulp vein

:::I

Sinus

Cord

Red pulp

Fig. 13.1: Architecture of spleen showing red pulp (peripheral); white

pulp (central); open and closed circulation; marginal zone and sinuses and trabecular artery and vein. Half life of platelets is 2-4 days. Life span is 8 days. In splenomegaly 80% of platelets may be sequestered in spleen causing thrombocytopenia. Normally spleen is reservoir for platelets. 113rd of total platelet mass is present in spleen. In pathological status like immune diseases phagocytosis of platelets in spleen is accelerated by many folds. Splenomegaly and hypersplenism can cause neutropenia. Neutrophil half-life is 6 hours. Bacterial clearance also occurs in spleen by phagocytosis. After splenectomy, patients are more prone for overwhelming post-splenectomy infection (OPSI). 2. Immunologic functions like synthesis of antibody lgM; formation of lymphocytes; production of tuftsin, opsonins, properdin and interferons.

Response to antigenic challenge-by secreting antibodies like lgM, tuftin, opsonins, properdin, interferons. These agents make bacteria and other organisms more vulnerable to phagocytosis. j All bacteria including the capsulated types, virus and fungi are destroyed efficiently. • Destruction or correction of abnormal cells like old RBCs, target cells, siderocytes, spherocytes or removal of inclusion bodies or parasites from the RBCs by culling and pitting. Phagocytosis of foreign substances. As platelet reservoir. • Erythrocyte production: In fetal life, it is an important site of RBC production till 5th month of gestation. In adults erythropoiesis in spleen occurs only if marrow production is inadequate, e.g. in myelofibrosis. Iron reutilisation.

I I

----------------~

Fig. 13.2: On table photo of splenunculi with splenomegaly. Splenunculi should be removed otherwise disease will recur.

After splenectomy , they undergo hyperplasia and lead to recurrence of the disord er for which spleen was removed. Blood peripheral smear, in these patients with splenunculi will not show Howell-Jolly bodies. Pappenheimer siderotic bodies. Accessory spleen can also be occasionally present adjacent to left ovary or adjacent to left testis. All accessory spleens should be removed along with splenectomy while doing for idiopathic thrombocytopenic purpura (ITP) or haemolytic disorders.

SPLENIC INJURY (RUPTURE SPLEEN) The spleen can rupture as-Trauma to a normal spleen OR to a diseased spleen; Atraumatic rupture of a diseased spleen [pathologic (occult) rupture) and Spontaneous rupture of a normal spleen [spontaneous (idiopathic) rupture).

I Causes Splenic injury occurs commonly following road traffic accidents, other blunt injury or penetrating/stab injuries. Most often associated with fracture of left lower ribs, haemothorax, injury of liver (left lobe commonly, occasionally both lobes), bowel, tail of pancreas, left kidney. Injury is more common and severe in enlarged spleen, i.e. in malaria, tropical splenomegaly, infectious mononucleosis. Spontaneous rupture of spleen can occur in malaria and infectious mononucleosis. Larang was used to kill by the murderers in far east where malaria was endemic leading to splenomegaly, which ruptured more easily. Spleen is the most common solid organ injured in blunt abdominal trauma.

Success isn't owned, it's leased and the rent is due every day. Success is not a destination, it's a journey

662 1. Splenic subcapsular haematoma: After initial injury patient remains asymptomatic for a short period. But this haematoma ruptures later, may be after few days causing torrential haemorrhage. 2. Clean incised wound over the surface: This can be treated by splenorrhaphy. 3. Lacerated wound. 4. Splenic hilar injury causes torrential haemorrhage, may even cause death. So immediate surgical intervention and splenectomy is done. 5. Splenic injury associated with other injuries (left kidney, left colon, small bowel, pancreas, diaphragm, left lung).

m

ASSOCIATED INJURIES

Left lobe liver injury; Tail of pancreas injury Left kidney; left colonic injury Small bowel injury; Diaphragm and left lung injury Fracture lower ribs-left sided (30%) Left sided haemothorax

Figs. 13.4A to D: Types of splenic injury. (A) Subcapsular haematoma; (B) Incised wound; (C) Lacerated wound; (D) Hilar injury.

leading to torrential bleeding. Blood clot temporarily seals off the bleeding which later gets dislodged causing severe bleeding. This time period in between is called 'latent period of Bandel'. Pseudoaneurysm and traumatic splenic arteriovenous fistula Presentation formation can also occasionally cause delayed life-threatening Hilar injury presents with rapid development of shock and detehaemorrhage. riorates fast. Even death can occur sometimes. Here emergency Features of other abdominal organ injuries may be present. surgery and splenectomy is mandatory. In other types, features of shock (pallor, tachycardia, restlessness, hypotension), pain, tenderness and abdominal rigidity • Kehr's sign-pain in left shoulder 15 minutes after foot end in left upper quadrant is seen. elevation Later there will be abdominal distension due to haemoperiBallance's sign-left-sided abdominal dullness which will not shift Saegesser's tender point between left sternomastoid and scalenus toneum. medius Dullness in the left flank which does not shift, as the collected Latent period of Bandel-refer above blood gets clotted. Dullness without shifting-Ba/lance'ssign.

I

B

L

Figs. 13.5A and B: Splenic injury with splenic haematoma. Fig. 13.3: Large subcapsular splenic haematoma.

Note: Sp/enosis-. Autotransplantation of fragments of splenic tissue may occur

within the peritoneal cavity following rupture of spleen.

Clot collected under the left side of the diaphragm irritates it and the phrenic nerve causing referred pain in the left shoulder-Kehr's sign. There may be left-sided haemothorax with fracture of ribs. Delayed presentation is also possible due to formation of subcapsular haematoma which later gives way. Initially gets temporarily localized by greater omentum, later giving way

I Investigations Ultrasound abdomen is the investigation of choice, as it is quicker, cheaper and noninvasive (FAST-Focused Abdominal Sonar for Trauma). Hb%, packed cell volume (PCV), blood grouping and cross matching.

Adequate amount of blood must be kept ready for transfusion. CT scan wil l show type of splenic injury and its class. CT scoring system is used based on capsu lar and parenchymal injuries and fluid in the abdomen and pelvis. Score 5 cm or parenchymal laceration >3 cm depth involving trabecular vessels

Diagnostic peritoneal lavage (DPL): By subumbilical incision the peritoneal lavage catheter is introduced into the peritoneal cavity. One litre of crystalloid (normal saline) is introduced into the cavity. Patient is turned to both left and right side and fluid is collected back. It is sent for cytology, culture, microscopy and biochemical analysis. It is significant when the aspirated fluid contains: , Gross blood of 10 ml. , >1,00,000/mm3 of RSC. , >500/mm3 of WBC. ,. Bile, bacteria or food fibres. , Amylase > 175 units/dl.

Grade IV

Laceration involving segmental or hilar vessels with >25% devascularization

Grade V

Shattered or avulsed spleen; hilar devascularization with entire spleen separation

I Treatment Initial Management (Resuscitation) ABCD, resusciltation and primary assessment of acute critical care. Central venous line for perfusion and monitoring; urinary catheter; nasogastric tube aspiration; ICU care. Fluid resuscitation with crystalloids using Ringer's lactate or normal saline using two wide bore cannula. Blood transfusion either whole blood or packed cell, FFP, platelet in 1:1:1 ratio. Antibiotics coverage; vaccine for pneumococcal/meningococcal and Haemophillus bacteria should be given. Assess the patient for other injuries (head to toe examination) by FAST, CT, X-ray, haematocrit, renal and liver functions.

Non-operative Management Figs. 13.6A and B: Lacerated injury over the spleen. Patient underwent splenectomy.

Angiogram, for diagnosis and often for angiographic embolisation to reduce the bleeding and to manage splenic injury non-operatively is also becoming routine in many centres.

I Complications of Splenic Rupture/Trauma Blood loss; Disseminated intravascular coagulation (DIC); Sepsis. Splenic artery pseudoaneurysm . Splenic arteriovenous fistula. Problems of associated injuries like of pancreas.

T

Splenic organ injury scale (1994 )

Grade I

Non-expanding subcapsular haematoma 3 times of upper normal limit. 3. Characteristic finding in CECT. Types 1. Acute interstitial oedematous pancreatitis; it is common with mortality only less than 1%, Mild-80%. 2. Acute necrolizing pancreatitis (10%)-it may be sterile necrosis or infected necrosis (occurs after first week). Sterile necrosis has got 48 hours), can be single or multiple organ failure

I I

of acute pancreatitis I1.Phases Early phase usually lasts for first week commonly with initial

systemic response to AP 2. Late phase has got a protracted course of many weeks to months I with local complications and organ failure Systemic inflammatory response syndrome {SIRS)

Core body temperature 38°C; heart rate >90/minute; Irespiratory rate >20/minute or PaC0 48 hours the patient is likely to have severe pancreatitis

Fluid , Metabolic, Haematologic and Biochemical Changes

B

Hypovolemia due to diffuse capillary leak and vomiting causing raised haematocrit, blood urea, serum creatinine levels. Hypoalbuminaemia which is more revealed after fluid correction. Hypocalcaemia is either due to decreased albumin level or speci1ic loss of ionized calcium. Hypocalcaemia due to reduced ionised calcium carries poor prognosis. Response of calcium reserve in bone to PTH is also reduced. Total count is raised with neutrophilia. Thrombocytopaenia, raised FOP, decreased fibrinogen, prolonged partial thromboplastin lime and PT-are common. DIC can develop later. Hypochloraemic metabolic alkalosis is common due to repeated vomiting. Reduced insulin secretion, increased glucagon and catecholamine secretion causes hyperglycaemia, more so in diabetic patients. Hyperbilirubinaemia may be due to biliary stone/obstruction or cholangitis or non-obstructive cholectasis. Hypertriglyceridaemia is common especially in hyperlipidaemic patients.

I

.___

Based on: 1. Local determinant is peripancreatic or pancreatic necrosis which 1 is non-viable tissue located in pancreas alone or in peripancreatic tissue alone or both. It can be solid or semisolid and without a radiologically defined wall; should be detected as areas of nonenhancement on CECT. 2. Systemic determinants as organ failure-cardiovascular, respiratory, renal; is assessed based on SOFA scoring as transient or persistent. 1

I

Type

I

back. Pain is severe, agonizing and refractory. Pain may be relieved or reduced by leaning forward. Vomiting and high fever, tachypnoea with cyanosis. Tenderness, rebound tenderness, guarding, rigidity and abdominal distension, severe illness. Often mild jaundice (due to cholangitis). Jaundice may also be due to bile duct disease/obstruction or cholestasis. Features of shock and dehydration. Oliguria, hypoxia and acidosis. Grey-Turner's sign, Cullen's sign, Fox sign. Haematemesis/malaena due to duodenal necrosis, gastric erosions, decreased coagulability/DIC. Hiccough when present is refractory. Ascites may be present. Paralytic ileus is common. Pleural effusion (20%), pulmonary oedema, consolidation, features of rapid onset ARDS is often observed. Neurological derangements due to toxaemia, fat embolism, hypoxia, respiratory distress can occur. It may be mild psychosis to coma. Occasionally haematemesis or melaena can occur.

(Peri) pancreatic necrosis Mild AP Absent Moderate AP Sterile Severe AP Infected Critical AP Infected

Organ failure And Absent And/Or Transient (48 hours) And Persistent

Working together is essential for success; even freckles would make a nice tan if they would get together.

677

678

Methemalbuminemia, when it occurs in acute pancreatitis indicates poor prognosis.

B

...= Cl)

en

• Perforated duodenal ulcer

v.,

c

DIFFERENTIAL DIAGNOSIS

Cholecystitis

Ectopic pregnancy Salpingitis

• Mesenteric ischaemia

Intestinal obstruction

• Ruptured aortic aneurysm • Diabetic ketoacidosis -- ------ ------

--

en

cc cc v.,

I Investigations Serum amylase is very high (> 1000 Somogyi units) or shows rising titre. Amylase creatinine clearance ratio is increased. It is urine amylase/serum amylase x serum creatinine/urinary creatinine x 100. Normal value is 1-4%. More than 6% signifies acute pancreatitis. Serum lipase more specific than amylase. Serum lipase level after rise persists for longer period than amylase. Pancreas is the only source unlike amylase, hence more specific.

T

CTSI Balthazar. 1990 (Emil J Balthazar)

Grade and appearance CT Grade: A- Normal pancreas B - Oedematous pancreatitis C- B+ mild extrapancreatic changes D-Severe extrapancreatic changes with one fluid collection E- Extensive/ multiple extra pancreatic collections or gas bubbles in or adjacent to pancreas

CTSI Morie le el al. 2004

Scar,

Grad, and appearance

0

A- Normal pancreas B- Intrinsic pancreatic abnormalities with or without inflammatory changes in peripancreatic fat C- Pancreatic/ peripancreatic fluid collection or peripancreatic fat necrosis

Score

CT Grade:

1 2 3 4

0 2

0 2 4

0 2 4

2

Necrosis score

None 30%

One or more extrapancreatic complications

Necrosis score None 150 mg/L) is also useful. Phospholipase A2, LOH levels are also often assessed. Liver function tests. Serum bilirubin, albumin, prothrombin time, alkaline phosphatase. Blood urea, serum creatinine. Blood glucose (hyperglycaemia is seen). Serum calcium level (hypocalcaemia occurs). Arterial P02 and PC02 level to assess pulmonary insufficiency (or ARDS). Urinary lipase estimation. Total count, haematocrit, platelet count, coagulation profile. Peritoneal tap fluid shows high amylase and protein level (very useful method). Lipase level in ascitic fluid is also useful. Plain X-ray shows , 'Sentinel loop ' of dilated proximal small bowel. , Distension of transverse colon with collapse of descending colon (colon cut off sign). ,. Air-fluid level in the duodenum. ,. Renal halo sign. ,. Obliteration of psoas shadow. ,. Localized ground glass appearance. US abdomen. Spiral CT (CECT-contrast enhanced CT) is better-gold standard. It is done after 72 hours. To look for oedema, altered fat and fascial planes, fluid collections, necrosis (non-enhancement area >30% or 3 cm), bowel distension, mesenteric oedema and haemorrhage. CT-guided aspiration (fluid should be sent for Gram's stain and bacterial culture); pigtail catheter insertion can also be done. If fluid culture shows bacterial growth with CT showing necrosis, it means it is infected necrosis and needs early pancreatic necrosectomy. CT-guided aspiration and Gram's staining may need to be repeated. CECT gives diagnosis of AP; assesses the severity, detects local complications. But CECT should be done ideally after first week not in initial period unless diagnosis is in doubt or any associated pathology is suspected. Non-enhancement finding is typical of pancreatic necrosis (WON). MRI, MRCP-should be done at a later date. ERCP is usually not done in acute phase. Chest X-ray for effusion and ARDS. EUS- to see necrosis, calcifications and to assess CBD.

679 n

:c

>

""C

-t

m

:ti

.....

Fig. 14.6: CT picture showing features of acute pancreatitis.

Fig. 14.7: CECT showing pancreatitis with stones in the duct.

Ranson's prognostic criteria In gallstone pancr,atitls SCOTB >3 suggests sev,re AP On admission: • Age >70 years • TC >18,000/cu mm • Blood sugar >220 mg% • LOH >400 IU/L • AST >250 IU/100 ml

Ranson 's prognostic crit,ria Glasgow-Imrie prognostic in non-gallstone pancreatitis criteria

Acute Physiology and Chronic H,alth Evaluation (APACHE II} score >8 points predicts 11 to 18% mortallty

On admission: • Age >55 years • TC >16,000/cu mm • Blood sugar >200 mg% • LOH >350 IU/L • AST >250 IU/100 ml

On admission: • Age >55 years • TC> 15,000/cu mm • Pa02 16 mmol/L (No response to IV fluids) • Blood sugar >200 mg% (no H/0 diabetes)

• Equation includes the following factors: age, rectal temperature, mean arterial pressure, heart rate, Pa02, arterial pH, serum potassium, serum sodium, serum creatinine, haematocrit, white blood cell count, Glasgow coma scale score.

Within 48 hours:

Within 48 hours:

Within 48 hours:

• • • • •

• • • • • •

• • • •

Haematocrit drop >10% BUN rise >2 mg% Serum calcium 5 mEq/L Fluid sequestration >4 L

Haematocrit drop>10% BUN rise >5 mg% Pa02 6 L

Serum calcium 200U/L

APACHE II modified (1996) LFT is added in biliary pancreatitis APACHE- 0 (Toh 1996) Obesity is added

Note: • Decrease serum calcium level is worst prognostic indicator of pancreatitis. • These scoring systems differ for non-gallstone pancreatitis and gallstone pancreatitis. • Other scoring systems which areoften used are APACHE II (Acute Physiology And Chronic Health Evaluation 11) and SAP (simplified acute physiology) scoring systems. Twelve variables are used to assess in APACHE II scoring. • C- Reactive Protein (CRP) >210 mg/ L in first 4 days and >120 mg/L in one week; phospholipase A2. assay; pancreas related protein; interleukin-6; polymorphonuclear elastase > 120 µg/L; serum ribonuclease assay-are other parameters used to assess the severity.

B • Conservative, 70-90% • Surgical treatment when indicated, 10-30% • Management of complications like acute pseudocyst, abscess, fistula , haemorrhage; systemic complications like ARDS, renal failure, MODS

Conservative Treatment Assessment of haemodynamic status and early resuscitation • Aggressive early hydration in first 24 hours using 400 ml/hour crystalloids (Ringer lactate, normal saline) to achieve rapid .____r_,_ ep_letion of the severe volume deplet~ Contd...

--

-

::1

Contd. .. Risk assessment stratification Patient with organ failure or SIRS should have ICU care in anticipation of ventilator and organ support (haemodialysis) Prevention or treatment of abdominal compartment syndrome (ACS) which carries high mortality; ACS is intra-abdominal pressure (IAP) more than 12 mmHg. It can occur during fluid therapy also; it requires often decompression by percutaneous catheter insertion or laparotomy Infection control, electrolyte management Risk assessment stratification • Patient features: Age >55 years; obesity (BMI >30); altered mental status; comorbid conditions SIRS Laboratory findings: BUN >20 mg/di with rising BUN; altered haematocrit; elevated creatinine; CRP > 210/L initially • Radiological features (X-ray initially; CECT later): Pleural effusion, ARDS, multiple or extrapancreatic fluid collections Scoring system: APACHE11, Ranson's, Glasgow, Modified Marshall, etc. ---

I

___J

Each case has its lesson-a lesson that may be, but is not always, learnt for clinical wisdom is not the equivalent of experience.-William Osler

680

...en= a,

Cl)

0 C'O

=

C

C'O

== _rn CCI

er:

Cl)

Rehydration is essential (25D-400 mUhour) as there is lot of fluid sequestration and 3rd space fluid loss. It is done by using ringer lactate, normal saline, dextrose saline, plasma and fresh blood transfusion/packed cells. Pain relief by pethidine and other analgesics. Morphine is not used as it causes spasm of sphincter of Oddi. In severe haemorrhagic episodes, fresh frozen plasma and platelet concentrate may be required in anticipation of DIC and haemorrhage. Nasogastric aspiration, urinary catheterisation to maintain and monitor urine output 50 ml hourly. Nasojejunal tube placement for feeding is very useful. Antibiotics like third generation cephalosporins, imipenem, meropenem, cefuroxime are used even though its role is not clear but it is commonly used to reduce the anticipated sepsis.

Other measures: Continuous monitoring, observation, maintaining good urine output 30 mUhr. The required investigations are repeated to find out the response to treatment like serum calcium, total count, blood urea, creatinine, Pa02, haematocrit, ECG.

B 1. (P)

2. (A) 3. (N) 4. (C)

Indications for antibiotics

In severe infected necrosis with/without proved culture. Prophylactic antibiotic therapy, in severe pancreatitis. In biliary pancreatitis with biliary stones and cholangitis. • Clinically disease is rapidly progressing with deterioration. No role of antibiotics in early pancrealitis. lmipenem, cilastatin, cefuroxime are used. Calcium gluconate 10 ml 10% IV 8th hourly is given as patient will be hypocalcaemic. CVP line is essential to monitor, for rapid fluid therapy and for Total Parenteral Nutrition (TPN) using carbohydrate, amino acids, vitamins, essential elements. IV ranitidine 50 mg 6th hourly or IV omeprazole 40 mg BD or IV pantoprazole 80 mg BD to prevent stress ulcers and erosive bleeding. Proper electrolyte management with monitoring is needed. It is always better to manage the patient in an intensive care set up so that when needed endotracheal intubation, ventilatory support, tracheostomy can be done as an emergency basis. In case of renal failure haemodialysis is required. Somatostatin/octreotide is often used to reduce pancreatic secretion. Anticholinergics, protease inhibitors and calcitonin are other agents used. Their efficacy is not sure. Steroid injection in initial phase of shock is beneficial. It is also useful in respiratory distress and ARDS. Nebulisation, bronchodilators are also tried . Nasojejunal tube placement and feeding should be started as early as possible once ileus subsides so that it reduces the infection rate by transmucosal migration of bacteria and it also improves nutritional status. During recovery period nasogastric feeding or jejunostomy feeding can be undertaken. Repeated calcium estimation, US examination to see the response is useful. Dopamine or low molecular weight dextran (to improve renal perfusion), somatostatin/octreotide (to reduce pancreatic secretion) are also often used. Management of complications like acute lung injury, atelectasis, renal failure, GI bleeding, metabolic encephalopathy, electrolyte deficiency as and when needed by repeated observation and evaluation.

5. (R)

6. (E)

7. (A) 8. (S)

: Pain relief [pethidine, meperidine (no morphine)]

Protease inhibitors (aprotinin, antisnake venom, EACA etc.), Plasma Antibiotics (ceftazidime, cefoperazone , cefotaxime) imipenem Anticholinergics (to reduce sphincter pressure) Nasogastric aspiration, Nasal 02 Nutritional support (TPN) Calcium gluconate 10 ml 10% 8th hourly Calcitonin CVPline Rehydration by IV fluids, plasma, blood Ranitidine IV 50 mg 8th hourly Respiratory support (ventilator) Resuscitation when required Endotracheal intubation Electrolyte management Antacids Swan-Ganz catheter for CVP and TPN Somatostatin and its analogue (Octreotide)

• Pancreatic surgeries • Variceal bleeding

• Dumping syndrome Carcinoid tumour, acromegaly + Endocrine pancreatic tumours • Acute pancreatitis • GI fistulas, pancreatic fistulas Dose:

50 µg as loading dose IV 50 µg 1 hour in 5% dextrose as maintenance dose.

Surgery Indications for Surgical Intervention (10% cases) 1. If condition of patient deteriorates in spite of good conservative treatment. 2. If there is pancreatic infected necrosis. 3. In severe necrotising pancreatitis as a trial to save the life of the patient which has got very high mortality.

Surgical management of acute pancreatitis Surgery removes intra- and extra-pancreatic necrotic materials, pancreatic fluid , and toxins. It permits preservation of viable pancreatic tissue. Open surgery is the gold standard for infected pancreatic necrosis. After opening the abdomen, all necrotic tissue, pus, infected fluid and toxins are removed . 10-12 litres of normal saline wash is given. Drainage tubes are placed liberally. Abdomen is closed in layers-Conventional closed method (necrosectomy, wide debridement, adequate drainage, cholecystectomy, closure). Re-laparotomy is done only on demand later.

Laparotomy-necrosectomy-wide debridement-wash-wide packing. Wound is left open. Repeated wash and packings are done until healthy granulation develops-open method. Laparotomy-necrosectomy and closu re with drain and re-laparotomy later-semi-open method. Zip technique can be used to give repeated wash to remove toxins and necrotic tissues until healthy granulation tissue develops in the pancreatic bed-Bradley's repeated Japarotomies and wash. Continuous closed peritoneal lavage of the pancreatic bed and lesser sac is done with 10-12 litres of normal saline or hyperosmolar, potassium free dialysate fluid 2 litres/hour using multiple tubes to remove toxic material in the peritoneal cavity/retroperitoneal area until return fluid becomes clear-Beger's lavage. Procedure is done after initial surgical debridement. Extra peritoneal lavage through bilateral flank incisions is also used. But being a blind procedure it is technically difficult and one may not be sure about the adequacy of the procedure. Under laparoscopic visualisation, necrosectomy, wash and drainage can be done. But often it is difficult to create pneumoperitoneum in acutely ill patient. But it is increasingly becoming popular. A jejunostomy is often done along with these procedures to have early enteral nutrition . Endoscopic necrosectomy is often done in some centres. Early endoscopic intervention (within 48 hours) with ERCP, biliary stone removal and stenting in biliary pancreatitis is done and favored in many centres. Further management is important to prevent recurrence. Gallstones should be dealt by laparoscopic cholecystectomy in 2 weeks after acute attack during same admission period. Endoscopic sphincterotomy (ERCP) and often stenting may be needed if there are CBD stones. Saline drip

Note: • GI fistula either enteric or pancreatic and incisional hernia are the late complications of these surgeries. • Localized collection of purulent material without significant necrotic material (considered as pancreatic abscess) is an extremely uncommon entity and so term 'pancreatic abscess' is not used now as per revised Atlanta classification 2012. Earlier it was thought to be 5% common; after an attack of severe acute pancreatitis or infection in pseudocyst; said to be seen in alcoholics; with a mortality of 25%. CECT confirmation, CT-guided aspiration and culture, CT-guided catheter drainage or open drainage is needed. Septicaemia, bleeding due to erosion and sloughing of vessel wall can occur. • Purtscher's retinopathy is a rare condition that is associated with complement-activating systemic diseases such as acute pancreatitis. After pancreatic injury or inflammation, trypsin activate the complement system and can cause coagulation and leukoembolisation of retinal precapillary arterioles. Emboli are small enough to pass through larger arteries; large enough to remain lodged in precapillary arterioles causing optic nerve oedema, impaired visual acuity, visual field loss, as well as retinal findings such as cotton-wool spots, retinal haemorrhage, artery attenuation, venous dilation, and Purtscher flecken.

Figs. 14.9A and B: On table photos of acute pancreatitis. Note the typical saponification in the omentum and mesentery.

COMPLICATIONS OF ACUTE PANCREATITIS Shock-hypovolemic and septic Respiratory failure and ARDS-Common in 7 days Septicaemia-common after 7 days Hypocalcaemia Disseminated intravascular coagulation (DIC) Acute renal failure Pancreatic pleural effusion (left sided 20%) • Pancreatic pseudoaneurysm Pancreatic ascites Colonic stricture Pseudocyst of pancreas Chronic pancreatitis Splenic vein thrombosis Abdominal compartment syndrome (ACS) Pancreatic endocrine (15%) and exocrine (20%) insufficiency as late sequelae can occur.

Acute fluid collection

, Lavage tube

Duodenum

Lavage tube

Fig. 14.8: Beger's lavage to pancreatic bed. Tubes are placed postoperatively. Continuous saline irrigation through the tube is done until irrigation fluid becomes clear.

,

It is collection of fluid in or near the pancreas during an attack of acute pancreatitis with an ill-defined or lacking fibrin wall or granulation tissue. It is 40% common; it usually occurs at peripancreatic area, occasionally intrapancreatic.

''.4ny tool can know. The point is to understand. '!..,_Albert Einstein

682

C'0

:::, C: C'0

::E: en

cc

a:

en

,

More than 50% of acute fluid collection regress spontaneously, remaining may form pseudocyst of pancreatic necrosis. , CT-guided aspiration at one puncture site is often necessary to confirm that it has not infected. Acute pseudocyst , It is collection of fluid with pancreatic juice in or near the pancreas localised by thin fibrin wall or granulation tissue. , Occurs in 2 weeks, usually resolves spontaneously. ,. Fluid can be removed percutaneously under guidance or through an endoscope. Pancreatic pseudocyst , It is collection of fluid in a false cavity which commonly contains brownish pancreatic enzyme rich fluid, lined by granulation tissue but not true epithelium with an organised thick fibrous covering. , It is commonly located in peripancreatic region, in lesser sac. It can be often intrapancreatic or in other places in the peritoneal cavity. It can be multiple also. ,. It usually forms 4 weeks after an attack of pancreatitis-a chronic entity. , It is usually sterile but can get infected. , Pseudocyst may rupture to form pancreatic ascites. , It may cause pancreaticopleural fistula when once it erodes into left side pleural cavity or it may erode into splenic vessels causing life-threatening haemorrhage. Pancreatic necrosis infected. (Walled of necrosis/WON) , It is focal/diffuse area of non-viable pancreatic parenchyma with peripancreatic fat necrosis. , It is initially sterile but eventually gets infected. ,. It contains paste/putty like material. , It may form pseudocyst, abscess or may be replaced by fibrous tissue during healing. , It can be sterile or infected necrosis. , It is confirmed by CT-scan and CT-guided aspiration usually at one site; collected fluid should be sent for culture. , Infection of necrotic area usually occurs within 4 weeks. It is ideally conventionally treated with laparotomy, debridement is done by removing all necrotic tissue with adequate drainage for remaining viable exocrine function. Often repeat laparotomies are done once in 3 days until all necrotic materials are cleared and jejunostomy is done in final approach. , laparotomy and debridement done once with continuous lavage; antibiotics with percutaneous drainage/only surgical drainage without debridemenUdebridement using minimally invasive methods-are unconventional approaches. , It may slough off the pancreatic/splenic vessel wall to cause torrential haemorrhage. Walled of necrosis (WON) may be single or multiple (60%). It is commonly in head/ body or tail. But often entire gland may be involved (25%). , Walled of necrosis (WON) may rupture into viscera or extend into other part of the abdomen. Features of sepsis, tender palpable mass in the epigastrium with leukocytosis are observed .

Respiratory complications , Thay are often severe and life-threatening. It is due to distension of abdomen, diaphragmatic elevation, pleural effusion, reduced surfactant (lecithin) activity in alveoli due to lecithinase, severe pain, pleural effusion (left), intravascular coagulation in lu ngs and ARDS. Arterial blood gas analysis should be done. Often it needs ventilator support. Pancreatic pseudoaneurysm , It is due to enzymatic digestion (elastase) of the wall and weakening and aneurysmal dilatation of the splenic (50%) or gastroduodenal vessels (15%) or inferior and superior pancreaticoduodenal arteries (10%). , It may rupture and cause life-threatening haemorrhage or may rupture into stomach or duodenum to cause massive upper GI bleed or may rupture into pancreatic duct causing haemosuccus pancreatitis. , It is diagnosed by CT angiogram. , It is treated by critical care, blood transfusion, emergency angiographic embolisation or by open surgery and ligation of the involved vessel. It carries high mortality. Pancreatic fistula , It can occur due to ductal wall disruption and necrosis or after surgical intervention for acute pancreatitis (necrosectomy). , It may be internal into bowel or external to skin. , Fistula may be low (200 ml) out put. , It can be straight or curved. , It is confirmed by biochemical analysis, ERCP, CT fistulogram. , If fistula persists for 6 months then sphincterotomy, resection of fistula with pancreatic resection, pancreaticojejunostomy is done. Emphysematous pancreatitis , It is gas in pancreatic parenchyma, a dangerous type and can be diagnosed by CT scan.

ia -~f-~ I I

. . .

CanHac Hypotension: pulse , 130/minute: arrhythmias, ECG ,• changes. Pulmonary. PaO2 >60 mm Hg; ARDS. Renal: Urine output 5000 units/ml).

683

B III Ill IV VVI VII -

Normal duct; no duct communication into pseudocyst. Normal duct with communication. Normal duct with stricture without communication. Normal duct with stricture with communication. Normal duct with abrupt duct termination. Chronic pancreatitis without duct cyst communication. Chronic pancreatitis with duct cyst communication.

Note:

• It is not a true cyst as there is no epithelial lining. • 75% of cystic lesions of pancreas are pseudocysts; Others

are-cystadenomas, IPMNs, cystic necrosis of adenocarcinoma, congenital polycystic disease.

I Clinical Features A swelling in the epigastric region which is hemispherical, smooth, soft, not moving with respiration, not mobile, upper margin is diffuse but lower margin well defined, resonant or impaired resonant on percussion, with transmitted pulsation confirmed by knee-elbow position. If it is infected, it will be tender mass and patient will be toxic with fever and chills. Because stomach is stretched towards the abdominal wall, Ryle's tube passed will be fe lt per abdominally (Baid test).

I Differential Diagnosis

Fig. 14.10: Pseudocyst of pancreas.

I Types Depending on whether it communicates with pancreatic duct or not it is classified as: 1. Communicating pseudocyst. 2. Noncommunicating pseudocyst. It can be Acute pseudocyst. Chronic pseudocyst. -

-

-

~-

- -~

-

--

---

--

D'EGIOIO CLASSIFICATION OF PSEUDDCYST

Type I: After an attack of acute pancreatitis. Normal duct anatomy; no fistula/communication. • Type JI: After an attack of acute on chronic pancreatitis. Abnormal duct anatomy without stricture; 50% chances of fistula. Type Ill: After an attack of chronic pancreatitis, abnormal anatomy with stricture; always communicating. It appears like retention cyst.

B Necrotic pseudocyst in acute pancreatitis. Retention cyst in chronic pancreatitis. lntrapancreatic retention due to pancreatic duct dilatation. Extrapancreatic retention cyst due to rupture into peripancreatic tissues.

I

Aortic aneurysm; Retroperitoneal cyst or tumour. Cystadenocarcinoma of pancreas. Cyst of the liver; Mesenteric cyst; Hydatid cyst.

I Investigations US abdomen (commonly done procedure), US reveals the size and thickness of the pseudocyst. Size less than 6 cm indicates that one can wait for spontaneous resolution. Endosonography (EUS) is very useful. CT scan is ideal and study of choice. It is two times more sensitive than US. It demonstrates size, shape, number, wall thickness, contents, pancreatic duct size, and extent of necrosis in pancreas, calcification and atrophy in chronic pancreatitis, regional vessels, pseudoaneurysm, splenic/ portal vein thrombosis. MRCP delineates the ductal anatomy and its abnormality. ERCP is done to find out the communication. Barium meal (lateral view) shows widened vertebrogastric angle with displaced stomach (Not usually done now). LFT, serum amylase platelete count, PT INR. £US-guided aspiration and analysis of fluid for amylase and CEA. Amylase will be high with normal CEA in pseudocyst; amylase will be normal/low with high CEA >400 ng/ml in mucinous neoplasm.

Doing the surgery may be easier, but it is managing the patient which counts finally.

684 V E

R T E B

R A •'

..-·-·--·······

,,

Normal

Fig. 14.11: Vertebrogastric angle.

2. lnterventionaf: Needs ERCP to delineate ductal anatomy, pancreatic duct stones, mainly communication into the pseudocyst. It is Guided-Endoscopic/EUS/US-guided drainage ,,. Transpapillary. Through side viewing endoscopy, pancreatic sphincterotomy is done; guidewire is passed into the pancreatic duct; stones if present are removed from the duct; 5 French pancreatic stent is placed which is removed in 6- 8 weeks. Confi rmation of resolution of pseudocyst is done by follow-up CT. This procedure is suitable only in communicating pseudocyst, it is safer and effective. , Transmural endoscopic or EUS-guidedstent placement to the pseudocyst; guided needle aspiration is done initially on the visible bulge; then endoscopic stent is placed to allow internal drainage; stent is removed after 8 weeks endoscopically. Recurrence rate is high; necrosectomy is not possible. , Percutaneous drainage under USG guidance: It is technically easier; catheter drainage is done after placing the catheter under guidance in large pseudocyst; high recurrence, infection, displacement and blockage of catheter are common. ,. Simple US-guided aspiration has got high recurrence and failure rate; so it is not practiced routinely. 3. Surgical drainage-. It is either open or laparoscopic method. INDICATIONS FOR SURGERY/INTERVENTION

Fig. 14.12: CT scan showing typical pseudocyst of pancreas.

Size more than 6 cm; Thick-walled pseudocyst Formed pseudocyst; Infected pseudocyst Cyst persisting after 6 weeks/progressive cyst Multiple cysts/cyst due to trauma Communicating cysts/cyst with severe pain

Cystogastrostomy: On laparotomy, anterior wall and posterior wall of the stomach is opened. Brownish fluid is aspirated. The thick capsule of pseudocyst is opened. All fluid with necrotic material are sucked. Fluid should be sent for cytology, culture and sensitivity and amylase estimation. Cyst wall always should be sent for biopsy. Cyst cavity should be washed with normal saline after breaking septae. Pseudocapsule is anastomosed to posterior wall of the stomach-Jurasz operation. Rupture-3%-into bowel or peritoneum Infection, commonest- 20%, abscess Bleeding from the splenic vessels-?% Cholangitis Duodenal obstruction Portal/splenic vein thrombosis and segmental portal hypertension Cholestasis due to CBD block

I Treatment 1. Conservative: Observation, follow-up using repeat USG at regular intervals. 50% of pseudocysts show spontaneous resolution.

Figs. 14.14A and B: Pseudocyst of pancreas aspiration of table.

Note the brownish-black colour of the fl uid aspirated.

Figs. 14.15A and B: (A) Cystogastrostomy for pseudocyst of the

pancreas; (8) Cystogastrostomy procedure. Anterior layer of the stomach is opened to visualize the bulge in the posterior wall. Aspiration of it shows brownish black coloured fluid, typical of pseudocyst of the pancreas.

• Small cyst can be drained through endoscopy. But debridement is not possible. Bleeding and cyst leakage are other problems. • CT-guided percutaneous catheter placement is done in critically ill patients, infected pseudocyst, unfit patients, cyst in pelvis/mediastinum. Improper drainage, recurrence and fistula formation are the problems. • External drainage is done when cyst is infected, haemorrhagic or ruptured. Problem with external drainage is formation of fistula (20%). • Therapy is decided based on thickness of the wall of pseudocyst; location of pseudocyst; contents of pseudocyst and ductal status. • Pseudocysts following trauma and chronic pancreatitis have got lesser chances of spontaneous resolution. • Pseudocyst following biliary pancreatitis has got 4 times more mortality than that of alcoholic pancreatitis. • Spontaneous resolution rate is 40% for pseudocyst less than 6 weeks; 8% for 6-12 weeks; less than 1% for cyst more than 12 weeks. • Presence of significant debris is a contraindication for endoscopic or image-guided drainage.

CHRONIC PANCREATITIS It is persistent progressive irreversible damage of the pancreas due to chronic inflammation. It can be

Chronic relapsing pancreatitis Chronic non-calcifying pancreatitis

Chronic pancreatitis (persistent) Calcifying pancreatitis

Fig . 14.16: Roux-en-Y cystojejunostomy for

pseudocyst of the pancreas.

Other procedures Cystoduodenostomy. Cystojejunostomy is done in large cyst, recurrent cyst. If infected, cystogastrostomy with external drainage is done using Malecot's catheter (Smith operation). Laparoscopic cystogastrostomy is becoming popular, effective and less invasive. Along with cystojejunostomy, pancreaticojejunostomy should be done if there is ductal stricture and dilatation and communication with pseudocyst. Distal pancreatectomy with pseudocyst removal if cyst is in distal part.

In the duct In the parenchyma (Stone in the pancreatic duct) Chronic pancreatitis is more common in males, common in Kerala (induced by diet, rich in Tapioca).

Note:

• Roux-en-Y cystojejunostomy is better with lesser recurrence rate than cystogastrostomy. • Pseudopseudopancreatic cyst: It is a mass in epigastric region due to pancreatitis formed by bowel, omentum which clinically mimics a pseudocyst of pancreas. But fluid collection is absent.

Fig. 14.17: Calcifications in the parenchyma of the pancreas.

A goal is a dream with a deadline.

685

686

... Q)

Cl

::::i Cl)

0

Fig . 14.18: Pancreatic ductal stone removed-specimen. Patient

underwent pancreaticojejunostomy. AETIOLOGY

Alcohol-80% main cause Stones in biliary tree-rare cause Malnutrition, diet Hyperparathyroidism Hereditary (familial hereditary pancreatitis)-Autosomal dominant ldiopathic-20%-as mutation Trauma; Congenital anomaly (Pancreatic divisum) Cystic fibrosis Autoimmune pancreatitis Hyperlipidaemia

I

TIGAR-O RISK FACTOR CLASSIFICATION 2001 T - Toxic-alcohol/tobacco/dietary/drug.

Metabolic-hypercalcaemia/lipidemia/lipoprotein lipase deficiency. I - Idiopathic-early/late onset/tropical -30% G - Genetic mutations-CFTR/SPINK 1. A-Autoimmune primary/with Sjogren/Crohn's disease. R - Recurrent and severe acute/ischemic. O - Obstructive-pancreas divisum/annular pancreas/stenotic papilla/duodenal obstruction/trauma/pancreatic ductal stones/ choledochocele. __ __ _ _

I I

_J

Alcohol reduces pancreatic blood flow, alters cell viability, releases the free radicals, creates pancreatic ischaemia, and activates the pancreatic stellate cells which produce abundant extracellular matrix and collagen. Genetic predisposition may be the cause of idiopathic pancreatitis. Mutation in pancreatic secretory trypsin inhibitor causes activation of trypsin causing pancreatitis. Hereditary pancreatitis is an autosomal disorder with mutation in trypsinogen gene in chromosome 7. It causes recurrent painful episodes of acute pancreatitis in childhood, leading to chronic pancreatitis and pancreatic cancer in adulthood.

Theories and Concepts in Pathogenesis of Chronic Pancreatitis Oxidative stress hypothesis-Reactive by-products of hepatic mixed function oxidase activity damage the pancreas through chronic reflux of bile into the pancreatic duct. The toxic-metabolic theory: Alcohol is directly toxic to the acinar cell where it brings changes in intracellular metabolism

causing pancreatic lipid accumulation, fatty degeneration, cellular necrosis, and eventual widespread fibrosis. Stone and duct obstruction theory: Alcohol increases the lithogenicity of pancreatic juice, leading to stone formation. Chronic contact of stones with duct epithelial cells produces ulceration, scarring, atrophy, fibrosis and chronic obstruction of the acini. The necrosis-fibrosis theory: Acute and chronic pancreatitis represents a spectrum of disease. Inflammation from acute pancreatitis leads to scarring, extrinsic compression of the pancreatic ductules, obstruction, stasis, atrophy and stone formation. The genetic defect of hereditary pancreatitis produces recurrent acute pancreatitis in early childhood , leading to chronic pancreatitis in early adulthood. Cellular mechanisms of pancreatic fibrogenesis is due to pancreatic stellate cells which are stimulated and activated by alcohol, oxidative stress, cytokines of acute pancreatitis; activated stellate cells migrate to the periacinar areas to deposit collagen and fibronectin. Transforming growth factor beta 1 is an important mediator of pancreatic fibrosis. The sentinel acute pancreatitis event (SAPE) hypothesis: An episode of acute pancreatitis, the sentinel event, sets the stage for the attraction of collagen-secreting stellate cells. Heavy, prolonged alcohol use is the most common cause of chronic pancreatitis. Alcohol-related chronic pancreatitis is associated with more severe pain, more extensive calcification and ductal changes, and more rapid progression to endocrine and exocrine insufficiency. Often recurrent episodes of acute pancreatitis for several years are seen in these patients. Some cofactors amplify the effect of alcohol in these patients. Prevalence of some genetic mutations linked with pancreatitis like cystic fibrosis transmembrane regulator (CFTR), serine protease inhibitor Kazal type-1 (SPINK1) has been noted in alcoholic pancreatitis. A high-fat diet and smoking affect pancreatic bicarbonate and water secretion adversely, inducing oxidative stress and increases the rate of pancreatic calcification. Tropical pancreatitis is endemic in some regions of India (Kerala), Africa, and South America. Episodic abdom inal pain begins in childhood and is followed by rapid progression to endocrine and exocrine insufficiency with pancreatic calculi in non-alcoholic individuals. Dietary toxins (cyanogens in the cassava plant, tapioca) and micronutrients like zinc, copper, and selenium, vitamin A deficiencies, genetic factors, ductal abnormalities are the probable causes of tropical pancreatitis. Linnamarin and its methyl derivative, in acid pH of stomach releases hydrocyanic acid which is cytotoxic in presence of rhodanase releases thiocyanates causing depletion of methionine, damaging pancreas pancreatitis. Gross look is small, firm fibrotic/adipose type. Early-onset idiopathic chronic pancreatitis manifests with severe abdominal pain in childhood, with relatively few structural and functional changes. Late-onset idiopathic chronic pancreatitis manifests in middle and late adulthood, often with minimal pain and pronounced exocrine insufficiency.

I Pathology It shows atrophy of acini, hyperplasia of duct epithelium, interlobular fibrosis, calcifications, ductal dilatation, with strictures in the duct, focal necrosis. There is loss of exocrine function initially and endocrine functions eventually. Ductular metaplasia and acinar atrophy along with fibrosis and cyst formation develops.

Focal necrosis Segmental or diffuse fibrosis Parenchymal calcification or ductal stones Stricture and/or dilatation of the duct

I Spectrum of Chronic Pancreatitis Early-pancreatic oedema-chronic inflammation-normal secretory function. Moderat~early fibrosis; only few acinar cells- exocrine dysfunction. Late-fibrosis- loss of secretory function-diabetes mellitus. Complications develop secondary to healing and fibrosis; deposition of inspissated proteinaceous material in the duct; over expression of CTGFand TGF-81 that stimulate extracellular matrix. IPain-h-yp-ot-he-sis -inc-lud-e:- - - - - - - - Acute and chronic inflammation of the pancreas. Increased pressure within the pancreatic ductal system and parenchyma, ductal dilatation, stasis. lschaemia of the parenchyma secondary to increased interstitial pressure. Over expression of a particular protein in pancreatic nerve fibres. Pancreatic nerve growth factor released by degenerating acinar cells and its high affinity receptor that promotes nerve growth and repair. Pain may be due to perineural sheath destruction by toxins, ,__ischaemia and also due to tissue acidosis. _I

I Classification of Chronic Pancreatitis I: Based on main duct dilated or not: Large duct disease-main pancreatic duct is dilated . Small duct disease-main pancreatic duct, is normal or smaller in size. II: Staging/classification of chronic pancreatitis (Stages A, B, C) A new classification of chronic pancreatitis, based on combination of clinical signs, morphology and function, is presented (2009 Buchler et al). Specific definition of chronic pancreatitis stage A Stage A chronic pancreatitis It is the early stage of chronic pancreatitis where complications have not yet appeared and the clinical exocrine and endo-

crine function is preserved. Subclinical signs (impaired glucose tolerance, reduced exocrine function but without steatorrhoea) might already be apparent. Stage A is accepted under the following conditions: Pain of any type and degree and/or attacks of acute pancreatitis, no complications, no steatorrhoea, and no insulin-dependant diabetes mellitus. Specific definition of chronic pancreatitis stage B Stage B chronic pancreatitis It is the intermediate stage where chronic pancreatitis has led to complications but clinical exocrine and endocrine function is still preserved. The type of complication is specified (e.g. stage B, bile duct). Stage B is accepted under the following conditions: Patients with complications but without steatorrhoea or diabetes mellitus. Specific definition of chronic pancreatitis stage C Stage C chronic pancreatitis Stage C is the end stage of chronic pancreatitis, where pancreatic fibrosis has led to loss of clinical exocrine and/or endocrine pancreatic function (steatorrhoea and/or diabetes mellitus). Complications of chronic pancreatitis might or might not be present. The type of exocrine and/or endocrine pancreatic function loss is specified (e.g. stage C, steatorrhoea). Stage Ccan be sub-classified into three categories: C1: Patients with endocrine function impairment. C2: Patients with exocrine function impairment. C3: Patients with exocrine/endocrine function impairment and/ or complications-as they are defined.

Stage Cis accepted under the following conditions-Patients with clinical manifestation of end-stage functional impairment with or without complications.

I Clinical Features Pain in epigastric region (80%) , It is persistent and severe, which radiates to back. , This pain is due to irritation of retropancreatic nerves, or due to ductal dilatation and stasis, or due to chronic inflammation itself. , Two patterns of pain have been described (Ammann and Muellhaupt). Type A pain is short relapsing episodes lasting days to weeks, with pain-free intervals. Type 8 pain is prolonged, severe, unrelenting pain. , Pain exacerbations need not be always associated with rise in amylase and lipase levels. , There is often a gradual diminish in pain over years due to "pancreatic burnout· by extensive calcifications, exocrine and endocrine insufficiency.

Carcinoma pancreas should be suspected when an elderly person develops diabetes and in spite of adequate treatment, continues to loose weight. - Robert D Lawrence

687

688 INVESTIGATIONS

Fig . 14.19: Patient will occupy leaning forward position to relieve

severe pain of pancreatitis.

Exocrine dysfunction: Diarrhoea, asthenia, loss of weight and appetite, steatorrhoea (signifies severe pancreatic insufficiency) (90%), malabsorption. Endocrine dysfunction: Diabetes mellitus. Pancreatic diabetes may often be typically brittle because of concomitant glucagon deficiency and requires insulin. Mild jaundice is due to narrowing of retropancreatic bile duct and cholangitis. Mass per abdomen, just above the umbilicus, tender, nodular, hard, felt on deep palpation, not moving with respiration, not mobile, resonant on percussion.

\

I

Serum amylase-not very useful Blood sugar Liver function tests and prothrombin time US abdomen to see duct dilatation, stones, parenchyma, liver status, CBD (normally, diameter of pancreatic duct is < 3 mm) Endosonography CT scan-CT-guided FNAC ERCP-Chain of lake appearance. Duct is dilated MRCP Plain X-ray shows calcification in 65% of patients Pancreatic secretory juice analysis Pancreolauryl test-pancreatic esterase cleaves fluorescein dilaurate after oral intake. Fluorescein is absorbed and quantified in urine after 2 days. Oral glucose tolerance test Faecal chymotrypsin and elastase analysis Bentiromide test

L I

- - - - - - - - - - - - - - --------' Investigations presently used are: CT scan abdomen: It is 95% reliable; to see pseudocyst, calcification, ductal stones, duct stricture and di latation, vasculature, fibrosis, surrounding structures, CBD status. It shows 90% sensitivity; 95% specificity.

(1) Pancreatic calcification; (2) Steatorrhoea; (3) Diabetes mellitus Triad is found in less than one-third of patients with GP ____.

Mallet-Guys sign: In right knee-chest position, if left hypochondrium is palpated tenderness can be evoked in case of chronic-relapsing pancreatitis. In this position , bowel loops are being shifted to right so as to have a direct palpation of pancreas. Note:

• Chronic pancreatitis can lead to carcinoma pancreas.

m

CLINICAL PRESENTATIONS

Stage A: 85%, recurrent/acute episodic pain with weight loss. Stage 8: Severe prolonged progressive pain with impaired pancreatic function with cholestasis, pseudocyst, sinistral portal hypertension. Stage C: Severe exocrine/endocrine deficiency, less severe pain, complications like pseudocyst and obstruction.

I Differential Diagnosis Carcinoma of head of the pancreas. Retroperitoneal tumour. COMPLICATIONS OF CHRONIC PANCREATITIS

Pseudocyst of pancreas; Pancreatic ascites; Carcinoma pancreas CBD stricture due to oedema or inflammation Duodenal stenosis Portal thrombosis- segmental portal hypertension Peptic ulcer Pancreatic pleural effusion, pancreatic ascites; Pancreatic fistula Splenic vein thrombosis; Pancreatics enteric fistula L_____

--- -

-

-

-- -- --

Figs. 14.20A and B: CT scan abdomen showing features of chronic

pancreatitis-calcification, ductal changes, and cyst formation.

ERCP is useful in chronic pancreatitis to see dilatations, strictures and altered ductal anatomy. It is mainly to assess structural pathology of the pancreas.

T

Cambridge grading of CP on ERCP

Grade

Main pancr,atic duct

Side branches

I: Normal

Normal

Normal

II: Equivocal

Normal

3 Abnormal

IV: Moderate Abnormal

>3 Abnormal

V: Severe

>3 Abnormal

Abnormal with at least one of the following: • Large cavity (>10 mm) • Obstruction • Filling defects • Severe dilatation or irregularity

Fig. 14.21 : ERCP showing pancreatic duct.

•·· MRCP is non-invasive method to see ductal anatomy. Endosonography (EUS) to see possible malignant site and to take FNAC. Site, duct status, stricture, stones, parenchyma, pseudocyst, CBD status, nodes are identified in EUS. Positive five parameters suggest chronic pancreatitis. It also helps in assessing operability. EUS criteria in chronic pancreatitis-parenchymal

EUS criteria in chronic pancreatitis-ductal

• Gland atrophy

• Narrowing, dilation, irregularity

• Hyperechoic foci

• Calculi

• Hyperechoic stranding

• Side branch dilation

• Cysts, lobularity

• Hyperechoic walls

Secretin cholecystokinin test is the gold standard for assessing pancreatic function. After over night fasting , double lumen tube is placed into the duodenum at the level of ligament of Treitz under C ARM guidance. Gastric and duodenal juices are aspirated. Continuous IV secretin 1 u/ kg/hour and CCK are infused in 90 minutes. Sampling of duodenal juice is done in every 10 minutes for one hour for analysis of volume, HC03, amylase, lipase and proteases. It is to assess functional deficiency. In chronic pancreatitis volume is normal- >2 mUkg but less bicarbonate content - 3 cm; thick wall cyst; nodules/papillary lesions; main duct> 1cm; raised CEA (120 ng/mL) in pancreatic juice/cyst fluid. Treatment: Main duct IPMN should be resected always. Branch duct IPMN 4 cm T4 - Extension to SMA, celiac axis, common hepatic artery Nodes-N

Nx NO N1 N2 -

Regional nodes cannot be assessed No nodal spread Nodal spread-1-3 nodes Nodal spread 4 or more spread

Metastases-M

MO - No distant spread cM1 - Distant spread present; pM1 - Distant spread microscopically confirmed Staging Stage 0: Tis NOMD. Stage IA - T1 NO MO. Stage 18 - T2 NO MD. Stage IIA - T3 NO M-. Stage 118 - T1 ,2,3 N1 MO. Stage Ill: T1 ,2,3 N2 MO; T4 any N MO. Stage IV: Any T Any N M1.

Preoperative Preparation in Carcinoma Pancreas Adequate hydration is important in prevention of dehydration postoperatively. Dehydration is common in obstructive jaundice. Glycogen reserve in liver will be inadequate which should be replenished by giving preoperative glucose orally or intravenously.

699

ERCPsigns

Important investigations

• Abrupt block of pancreatic duct with irregular stricture • Pancreatic duct encasement • Double duct sign-cut off of both pancreatic and bile duct • Parenchymal filling • Scrambled egg appearance

• Spiral CT/30 CT is ideal- to detect operability, portal vein invasion, size, extent, pancreatic and biliary tree and nodal status • ERCP to take biopsy/brush cytology, stenting, to see the luminal extent of the tumour • MRCP is non-invasive-entire biliary tree is visualised properly • Endosonography (EUS)-to assess size of primary properly; to take endosonographic FNAC • PTC often with if ERCP fails

Barium meal X-ray

Investigations to stage the disease

• Is rarely done in periampullary carcinoma • Rose thorn appearance in hypotonic duodenography • Reverse 3 sign in periampullary carcinoma • Pad sign-widened Cloop of duodenum in carcinoma head of pancreas • Features of Gastric outlet obstruction

• • • •

Patient is prone to develop hepatorenal syndrome postoperatively, leading into renal failure due to sludging of the bile salts and also due to toxins and sepsis; and so mannitol should be given intravenously for 3 days prior to surgery to flush the kidney (200 ml IV twice a day). Injection vitamin K 10 mg IM for 5 days. ERCP stenting is done only if serum bilirubin is >30 mg%, severe cholangitis or severe albumin deficit or in inoperable cases to relieve obstruction. Routine ERCP is not advisable as it may contaminate biliary system. Percutaneous transbiliary drainage (PTBD) or cholecystostomy are other options. Whipple's operation or resections are done 3 weeks later. Antibiotics one day prior to surgery-cephalosporins/ aminoglycosides. Often TPN may be required preoperatively which is also continued postoperatively. Pulmonary function study and respiratory physiotherapy to have adequate post-operative pulmonary function.

30/spiral CT scan EUS Laparoscopy ERCP

-

---

CRITERIA FOR RESECTION + Tumour size less than 3 cm +

Periampullary tumours

+ Growth not adherent to portal system

In operable cases: Whipple's operation is done by removing tumour with head and neck of pancreas, C loop of duodenum, 40% distal stomach, 10 cm proximal jejunum, lower end of the common bile duct, gallbladder, peripancreatic, pericholedochal, paraduodenal and perihepatic nodes. Continuity is maintained by choledochojejunostomy, pancreaticojejunostomy and gastrojejunostomy. Few advocate pancreaticogastrostomy into posterior wall of the stomach. Mortality in Whipple's operation is 2-8%. Original Whipple's operation (1935) was two staged procedure- initial bypass and a second stage resection with closure of pancreatic stump. In 1941 , Trimble performed one stage pancreaticojejunostomy. Complications Note: • In obstructive jaundice, serum bilirubin level will not go beyond 40 are delayed gastric emptying (19%); pancreatic fistula (1 4%); mg%. At this point raised intrabiliary pressure (Normal is 12-22 infection (intra-abdominal abscess, wound infection, cholcm of H20) to 25 cm of H20, blocks the formation of bilirubin and angitis, pancreatiti~, pneumonia) ; bile leak. Mortality is 3%. secretion of cholesterol and bile acids completely. Maximum producTraverso-Longmire pylorus preserving pancreaticotion of bilirubin per day possible is 250 mg%. Normal maximum excretion of bilirubin by kidney per day is 21 Omg%. So maximum duodenectomy (1978): Here duodenum is cut 2 cm distal to possible raise of serum bilirubin is 40 mg% in obstructive jaundice. the pylorus and continuity is r:r,aintained by anastomosing • Cholangitis in malignant obstruction is rare; but when it occurs it with jejunum. is rather severe form. Fortner's regional pancreatectomy (extended Whipple's) • Preoperative albumin therapy is usually not done. includes resection like Whipple's along with removal of Treatment segment of superior mesenteric vein and clearance of all regional nodes; and portal vein continuity is maintained by Only 10-15% of pancreatic carcinomas (head) are operable. a synthetic vascu lar graft. Even though technically it gives 40-50% are locally advanced. Another 40-50% will have distant adequate clearance, results are only equivocal. spread to liver or lungs.

I

"The only true wisdom is in knowing you know nothing."-Socrates

(")

::c

>

m

:a

....

pancreatic duct, morbidity by pancreatic fistula or pancreatitis after Whipple's operation is not seen here. Mortality in total pancreatectomy is higher 10- 20%. Severe, resistant brittle diabetes mellitus may be seen after total pancreatectomy.

...

a., Cl

=

en ci

= C

Gallbladder Cholecysto• jejunostomy

Gastrojejunostomy

Duodenum

==en

Jejunum

a:l

cc en

Jejuno• jejunostomy

Fig . 14.43: Whipple's operation .

Fig. 14.46: Specimen of total pancreatectomy with C loop, head, body and tail of pancreas with spleen. Total pancreatectomy is done in refractory severe chronic pancreatitis and carcinoma pancreas.

Distal pancreatectomy or central pancreatectomy or total pancreatectomy for cystadenocarcinoma of pancreas depending on the extent and size of the tumour.

Figs. 14.44A and B: (A) Specimen of pancreaticoduodenectomy with tumour in the head of pancreas; (B) Resected specimen (after Whipple's operation) of periampullary carcinoma of pancreas. Fig. 14.47: Photograph showing specimen of distal pancreatectomy.

In inoperable cases: Here mainly to palliate obstructive jaundice, duodenal obstruc· tion and pain . Roux-en· Y choledochojejunostomy is ideal palliative procedure along with gastrojejun ostomy after doing cholecystectomy. Choledochojeju nostomy is better than cholecystojejunostomy as CCJ may get blocked either by tumour infiltration or by bile sludge or by inflammation. 30% of periampulla ry carcinoma/carcinoma of head of pancreas

Fig . 14.45: On table finding in periampullary tumour showing dilated gallbladder and CBD.

Total pancreatectomy is presently said to be better. It is done along with nodal clearance. Reasons are-possibility of multi· centric nature of the disease, higher chance of recurrence after Whipple's operation, malignant cells may be present in

eventually develop gastric (duodenal) outlet obstruction and so gastrojejunostomy is undertaken . ERCP and stenting is done to drain bile. Problems here are recurrent cholang itis, stent blockage and displacement, requi rement of repeated stenting procedu re. Duodenal stenting for duodenal obstruction is tried. Chemotherapy; neoadjuvant chemotherapy. Steatorrhoea is treated by enzymes. Control of diabetes is important.

Adjuvant Therapy Liver

Pancreas

Fig. 14.48: Choledochojejunostomy (CDJ) with gastrojejunostomy

(GJ) and jejunojejunostomy (JJ). It is a Roux-en-Y anastomosis. CDJ should be 70 cm from the JJ.

Gallbladder Cholecysto- _ jejunostomy

..,..,,,

.>,,f"l--,.,_..J..__

~ --==--==+-½=-'"--Jejune- _

Gastrojejunostomy

__,,_

Jejunum

__

jejunostomy

Adjuvant chemotherapy using gemcitabine-better but costly; dose is 1000 mg/m2 su rface area; 5-fluorouracil; mitomycin; vincristine, cisplatin, docetaxel, leucoverin, abraxane (paclitaxel protein bound, oxaliplatin are used along with gemcitabine. , Different regimes are--fOLFIRINOX (oxaliplatin (Eloxatin), irinotecan (Camptosar), leucovorin, fluorouracil); Gemzar (gemcitabine) + Abraxane (albumin bou nd paclitaxel); Gemzar + erlotanib (Tarceva, EGFR drug). Radioactive iodine seeds 1125 to the field are on trial. External radiotherapy 4000 cGy units to relieve pain and to reduce the tumour size. lmmunotherapy-specific type to increase the effectiveness of chemotherapy and to improve the cure rate (allogeneic tumour cell vaccine).

Neoadjuvant Chemoradiotherapy Only 20% of pancreatic carcinomas are amenable for surgical resection. Even in these patients in spite of resection, overall outcome is poor. So neoadjuvant chemoradiotherapy is becoming popular [(50 Gy dose of radiotherapy for 5-6 weeks (25- 30 fractions) with infusion chemotherapy, then surgical resection) ) and under trial to improve the resectability and survival. It increases the RO resection status and reduces the node positive rate. There is less chance of tumour hypoxia (which is more after surgery) by which increases the susceptibility of tumour to chemoradiotherapy; less chance of mucosal anastomotic toxicity. It reduces the tumour burden at surgery; decreases the local or regional recurrence; decreases the chances of pancreatic fistula. But there is no survival benefit.

Postoperative Management in Carcinoma Pancreas Fig. 14.49: Cholecystojejunostomy-is not a preferred method.

Maintenance of proper fluid and electrolyte balance. Observation for bleeding and its control by transfusion of blood, fresh frozen plasma (FFP), and prevention of DIC at initial period. Injection vitamin K 10 mg IM for 5 days. Respiratory care-ideally post-operative ICU care is better. Often ventilator is needed for 24 hours. Maintaining adequate urine output-mannitol shou ld be continued. Injection octreotide infusion for 5 days to suppress pancreatic secretion so as to prevent leak. Antibiotics, nasogastric aspiration.

Fig. 14.50: White bile. It is neither white nor bile. It is opalescent; and

is mucous. It suggests complete obstruction. It is usually observed in carcinoma pancreas. It is peroperative finding.

Continuous monitoring the patient with pulse/blood pressure/ oxygen saturation/hourly urine outpuVinspection of drain site/ abdomen distension/by doing HB¾, LFT, serum creatinine, bilirubin, arterial blood gas analysis if needed, platelet count, prothrombin time.

Silence is a source of great strength. Silence is the best speech.

701

702

I Pain Control in Carcinoma Pancreas CT-guided 50% of 20 ml ethanol injection into coeliac ganglion. Epidural anaesthesia. Opioids administration. Transthoracic splanchnicectomy-greater splanchnic nerve. Palliative radiotherapy- 4000 cGy units.

0

n, ::::s n,

_en

ca a:

Cl)

Abdominal discomfort, trembling, sweating, hunger, dizziness, diplopia, hallucinations. Later epilepsy and unconsciousness. They are usually over weight. Weight gain is common due to overeating. Permanent neurological deficits can occur with behavioural changes.

B

C

::E

I Clinical Features

• Mean survival rate 6-9 months • Growth more than 3 cm • Nodal involvement Resection status-RO/R1/R2 Portal vein infiltration • Liver/lung secondaries • Ascites/Trousseau's sign/left supraclavicular lymph nodal spread • White bile on table carries poor prognosis • Associated problems like pancreatitis, diabetes mellitus I • Liver dysfunction

• I

ENDOCRINE PANCREATIC TUMOURS Most common is insulinoma. Others are gastrinoma, glucagonoma, vipoma, pancreatic polypeptidoma. They belong to group of tumours called "APUDOMAS". Endocrine tumours are associated with MEN syndrome (Type I (Wermer's syndrome). They are associated with: , Parathyroid adenoma. , Pituitary adenoma. , Peptic ulcer. They are usually multiple, small, 35-50% are malignant and presents with features of endocrine lesion. Commonly occurs in body and tail (except gastrinomas). Requires special method to identify. MRI, angiogram, hormone assay are the diagnostic methods required. Normal islet cells are < 2% of pancreatic mass. They contain a , p, 8, G, 02 and PP cells (pancreatic polypeptide). Incidence is 5 per 100000 per year. 20% are non-functional. lnsulinomas are commonly benign; glucagonomas are almost always malignant.

• An attack of hypoglycaemia in fasting state Blood sugar below 45 mg% during the attack • Symptoms relieved by glucose

I Diagnosis Insulin radioimmunoassay. An insulin level >7 µU/ml ; insulin/ glucose ratio >0.3 signifies insulinoma. Proinsulin leve,I more than 24% of total insulin signifies insulinoma. Proinsulin level more than 40% of total insulin signifies malignant insulinoma. C peptide level >1.2 µg/ml with glucose level 20% • Increased circulating C-peptide insulin to glucose ratio >0.3 is diagnostic

I Differential Diagnosis Nesidioblastosis. Non-insulinoma pancreatogenous hypoglycaemia.

I Treatment

INSULINOMAS lnsulinomas are commonest endocrine pancreatic tumour (60%) arising from B cells (P cells) of pancreas. They are commonly solitary but 10% can be multicentric and secrete insulin in excess causing profound hypoglycaemic features. Equal in both sexes. 90% of insulinomas are 120 mg/litre; interleukin level; elastase level; urinary TAP assessment.

Contd...

f I

707

__ __

Combined pancreatico duodenal injury .. ·--- ···"" I Type I Class I injury to both organs/class I iniury of one organ with class II injury to other Type II Class II injuries of both organs Type Ill Class Ill injury to one organ with less severe injury to other Type IV Class Ill injury to both organs _ _ _ _ _ _J

I

B Grade I - Minor contusion/laceration without duct injury Grade 11 - Major contusion/laceration without duct injury Grade Ill - Distal transection or injury with duct involvement I Grade IV - Proximal transection or injury with involving ampulla I Grade V - Massive disruption of pancreatic head __J

Fig. 14.54: CT showing pancreatic trauma.

Treatment ,, Antibiotics-imipenem, cefuroxime, ofloxacin, meropenem. ,.. Percutaneous drainage. ,, Laparotomy, necrosectomy, debridement, saline wash, closure with tube irrigations/lavage. Complications , Septicaemia, ARDS, renal failure, MODS. ,, Pancreatic fistula after surgery; Intra-abdominal abscess. ,, Abdominal dehiscence, incisional hernia after surgery.

PANCREATIC TRAUMA It is rare because of its anatomical location-retroperitoneum. Its injury is usually associated with injuries to liver/duodenum/ spleen/portal system/biliary system/ kidney. Deep force in epigastrium may cause crushing of body of pancreas against vertebra-closed injury. Penetrating injury may cause direct sharp injury of pancreas. Types ,, Parenchymal contusion/laceration without duct disruption. ,,. Parenchymal injury with duct disruption. ,, Complete transection of pancreas. ,, Massive destruction of pancreatic head. I Capsular damage; minor parenchymal injury I IClass Class 1/Transection of duct in body or tail-partial or complete Class /II Major duct injury in pancreatic head or intrapancreatic CBD Iinjury Duodenal injury I Contusion, haematoma, partial thickness injury IClass I Class II Full thickness duodenal injury Full thickness duodenal injury with I1.ClassMoreIllthan I 75% circumference injury Pancreatic injury

1 2. Extrapancreatic CBD injury

_J

Contd...

Features: Pain in epigastrium; Features of associated injuries; Features of shock; Rise in serum amylase level is common. Investigations: CT scan is diagnostic; ERCP to confirm duct disruption; Assessment of blood loss and other injuries. Treatment: Commonly conservative with fluid management; blood transfusions; antibiotics; pain relief; Surgery is needed when there is: ,, Major ductal disruption; Vascular injury; Extensive injury to head; Other organ injury.

Surgeries are Proper exploration and haemostasis. In neck transection, distal pancreatectomy with splenectomy. In injury to head, haemostasis and external drainage is done. Pancreaticoduodenectomy is done if there is major injury. Choledochojejunostomy is done if there is CBD injury. Different pancreatic drainage procedures are done to prevent sepsis and fistula. Portal system injury carries poor prognosis. It needs repair or intervening graft placement to control and maintain the continuity. Prognosis: Depends on type and class of injury and other associated injuries.

B Haemorrhage Pancreatic fistula Septicaemia Pancreatic abscess Pancreatitis-acute/recurrent Pancreatic duct stricture at I Pseudocyst formation the site of duct injury as a delayed complication. __ j _ _ ____ ______

CYSTIC LESIONS OF PANCREAS 1. True pancreatic cysts: Congenital; Not premalignant; Asymptomatic. ,, May be associated with cystic diseases of liver, kidney. ,, Multiple true pancreatic cysts (polycystic disease of pancreas) are common than solitary true cyst. They are lined with cuboidal epithelium and are associated with

It is better to aim at good things and miss it, than to aim at a bad thing and hit it.

"tJ Q) ::,

....

c-,

C'D

Q)

c,,

708

...::s

Q)

Cl

Cl)

C

«I

::s C «I

:E

von Hippe/-Lindau disease in 50% of patients. They do not require any treatment. 2. Pseudocyst of pancreas. 3. Cystic tumours of pancreas. a. Serous cystadenoma. b. Mucinous cystadenoma. c. Cystadenocarcinoma. d. Angioma. e. Cystic teratomas. 4. Hydatid cyst of the pancreas.

fluid is continuously collected with segregations as 15 minutes as 4 groups; in pancreatic insufficiency HCO3 level will be < 80 mEq/L in all four groups; total HCO3 output and volume also analysed. Secretin: CK test is more reliable and accurate than secretin test alone. Both hormones are administered as bolus injection continuously either simultaneously or sequentially; similar assessments are done. Normally duodenal fluid contains more than 80 mEq/L of HCO3 and HCO3 greater than 15 mEq/30 minutes.

_vi

CIC

a: Cl)

PANCREATIC EXOCRINE INSUFFICIENCY (EXOCRINE PANCREATIC DISEASE)

Indirect Methods

These methods are measure of effects of pancreatic insufficiency. Causes Serum trypsinogen and trypsin level estimation; trypsinogen Chronic pancreatitis, cystic fibrosis, pancreatic resections (total level less than 20 ng/ml suggest advanced pancreatic or partial), pancreatic duct obstruction by haemochromatosis or exocrine insufficiency. a1 antitrypsin deficiency, short gut syndrome, gastric resection, Faecal fat estimation after collecting 72 hours faecal fat and Shwachman Diamond syndrome (congenital entity with pancreestimation is done for daily fecal fat using Sudan stain. In a atic exocrine insufficiency, cytopenias, skeletal abnormalities). person ingesting 100 g of faVday, if faecal excretion of fat is more than 7 g/day, it signifies pancreatic insufficiency. It is the most sensitive and specific test. (Presence of neutral Features fat suggests pancreatic disease whereas split fat suggests Maldigestion of fat and proteins causing steatorrhoea and small bowel disease). weight loss. Pancreolauryl test Fluorescein dilaurate is ingested along Metabolic bone disease, impaired night vision, fat soluble with breakfast which is a substrate for pancreatic enzyme vitamin deficiencies can occur. cholesterol esterase; depending on the activity of the cholesVitamin 812 deficiency can occur as decreased intestinal pH terol esterase this substrate is cleaved to release proporprevents transfer of vitamin 812 to intrinsic factor from R tionate fluorescein; released fluorescein rapidly absorbed factor; it shows positive dual label Schilling test. from the gut and excreted in the urine. Serum fluorescein and 24-hour urine fluorescein assay gives the quantitative Evaluation estimation of the pancreatic exocrine function. It is accurate and acceptable test for pancreatic exocrine insufficiency. Direct Methods Trio/in breath test: Triolin labelled 13C or 14C is administered It is done by directly collecting the fluid from duodenum using as test meal; it is hydrolyzed in the gut lumen depending on double lumen collecting tubes, one is duodenum to collect the quantity of pancreatic lipase activity; hydrolysed products pancreatic fluid and other being in stomach to aspirate gastric are absorbed, metabolised and released through pulmonary content so that to avoid it mixing with pancreatic fluid. endothelium as 13CO2/14CO2 which is measured using Lundh test (Goran Lundh): Liquid test meal containing mass spectrometry or infrared. It is mainly used to quantify protein , fat and carbohydrate (300 ml) is given orally; steatorrhoea. duodenal fluid is collected for 2 hours; assessed for enzymes. Normally trypsin level is> 8 mEq/L. It is very low in pancreatic Treatment of Pancreatic Exocrine Insufficiency insufficiency. Test is obsolete now. Secretin test Initial test dose of secretin is given 0.2 ug; then Low fat diet; pancreatic enteric coated tablets during meals, full dose of 0.2 ug/kg as a bolus IV dose is given; duodenal treatment of cause.

I

I

I

I

Chapter

I

Retroperitoneal Space --i-IAPTER OUTLIN E • • •

Anatomy of Retroperitoneum Retroperitoneal Fibrosis Retroperitoneal Swellings

• •

Retroperitoneal Tumours Psoas Abscess (Retro peritoneal Abscess)

12th dorsal nerve lliohypogastric nerve , --...,.........~ moinguinal nerve Lateral femoral cutaneous nerve

ANATOMY OF RETROPERITONEUM This is the space between the peritoneal cavity and posterior abdominal wall. It is bounded anteriorly by posterior parietal peritoneum; posteriorly by vertebral column, psoas muscles, quad ratus lumborum muscle and tendinous portion of transve rsus abdominis muscles; superiorly by diaphragm; inferiorly by pelvic levator muscles (by Ackerman). It is real potential space. DISEASES OF RETROPERITONEAL SPACES

• • • • • •

Retropentoneal f1bros1s Retroperitoneal cysts Retroperitoneal tumours Retroperitoneal lymphomas Retroperitoneal vascular diseases, e.g. aneurysms Retroperitoneal trauma and haematoma Retroperitoneal infection, e.g. psoas abscess, pyogenic absce~

I

Diseases of the specific retroperitoneal organs, e.g. adrenals, kidney.

RETROPERITONEAL FIBROSIS Idiopathic type (70%) is called as Ormond's disease which is associated with mediastinal fibrosis, Dupuytren's contracture, plantar fasciitis, Peyronie's disease, Riedel's thyroiditis, sclerosing cholangitis, mesenteric panniculitis, pseudotumour of

Fig . 15.1: Anatomy of retroperitoneal space.

the orbit and other fibromatosis. It is nonspecific inflammation of fibrofatty tissue in the retroperitoneum. Patient presents with severe and persistent back pain. Diffuse fibrosis in retroperitoneum can compress both ureters leading to bilateral hydronephrosis and renal failure. Extravasation of urine, ascending lymphangitis, and autoimmune cause are the aetiologies. In 30% cases it is bilateral. Hypertension, lower limb oedema {lymphatic or venous block}, feeble lower limb arterial pulses are common. It is like woody fibrous tissue which narrows ureters, vessels and nerves. Ureteric obstruction commonly in lower 113rd causing dysuria, frequency, oliguria, renal failure. Venous and lymphatic oedema of limbs can occur. It may be localised or generalised. Generalised type (15%) may extend into duodenum, CBD and pancreas. It is not encapsulated but margin is well-demarcated.

The lesser the indication, the greater the complication.

710

It can also cause vascular compression, both venous and arterial. Disease is progressive one. Investigation: ,. Ultrasound , !VU-diagnostic:

B Deviation of middle 113rd of ureter medially Hydroureteronephrosis Extrinsic ureteral compression

,. Blood urea and serum creatin ine, raised ESR and CRP ,. CT scan. Treatment ,. Drug therapy. Methylprednisolone, azathioprine, penicillamine, tamoxifen. ,. Cystoscopic stenting of the ureters to prevent renal failure. ,. Symptomatic treatment: If stentin g fails, bilateral nephrostomy is done. , Ureterolysis. , Lateral repositioning of ureters. ,. Omental wrap after ureterolysis. , Vascular bypass graft for vascular encasement.

Note: • Tumours of retroperitoneal organs like kidneys, ureters, pancreas, and adrenals are conventionally not included in retroperitoneal tumours. • Paragangliomas are of neural crest originthat arises from paraganglionic tissues distributed along the major vasculatures (abdominal aorta in abdomen) or sympathetic chain, may be functioning or nonfunctioning. 20% secrete catecholamines. They may be multiple. Only 0.5% of cases present with hypertension. It can be familial, often associated with van Hippel-Lindau disease and RET proto-oncogene. It may arise from type I cells as chromogranin A positive-NSE positive; or type II cells as S 100 positive with good prognosis. • Costello syndrome is mental retardation, benign papilloma; develop embryonal rhabdomyosarcoma. • Retroperitoneal cysts are-cysts arising from Wolffian remnants or urogenital tract; RP mesenteric cyst; teratomatous dermoid cysts; RP lymphatic cyst; parasitic cysts.

12th dorsal nerve

Kidney--,~-1--C Retroperitoneal tumour Laterally ~ deviated ureter

lliohypogastric nerve ....,,...___ llioing uinal ~-....

nerve Lateral femoral cutaneous nerve

B • Drugs-methysergide and beta-blocker Urine leak and collection in the space Haematoma, blood collection Advanced malignancy, radiation Ergot's alkaloids, dopamine agonists Retroperitoneal surgeries, haemorrhage Trauma, infection

RETROPERITONEAL SWELLINGS

IB IIBJ.ilii,iMhiiiMi@iiiMMMifi1 ilHII Benign-20% of RP tumours Retroperitoneal lipoma Retroperitoneal neurofibroma, neurilemmoma Retroperitoneal leiomyoma Extra-adrenal chromatfinomas Paraganglioma Mucinous cystadenoma Haemangiopericytoma

Malignant-BO% of RP tumours Retroperitoneal liposarcoma, leiomyosarcoma-50% Retroperitoneal lymphoma (commonly NHL) Malignant tumours from specific organs Germ cell tumours, chordomas L__iPLN secondaries with hard nodules _ _ _ _ __

I I

Fig. 15.2: Retroperitoneal tumour can attain very large size often. It causes deviation of ureter laterally often may invade the ureter (In retroperitoneal fibrosis ureter is deviated medially). It may invade or encase the IVG also causing IVG obstruction with features of bilateral oedema feet, dilated lateral abdominal wall vein with upward direction flow of blood.

I Features of the Retroperitoneal Mass It is usually large; Not moving with respiration; Nonmobile. Does not fall forward (confirmed by knee-elbow position). Deeply placed. Resonant on percussion (because of the bowel in front). ,. In retroperitoneal cyst and sarcoma the swelling is smooth. ,. In lymphoma it is smooth and firm. ,. In aneurysm it is pulsatile (expansile pu lsation) which will persist even in knee-elbow position. ,. Confirmation is by US, CT scan, MRI. Most common retroperitoneal benign tumour is lipoma. ,. Sarcoma (commonest is liposarcoma 40%) which attains a very large size, goes for myxomatous degeneration very early and is aggressive.

711

C"')

:::c

>

m :c ...i.

UI

::c

-....

(II

0

"Cl

.... ::;: (II

0

Fig. 15.3: Typical look of retroperitoneal tumour.

RETROPERITONEAL TUMOURS Retroperitoneal tumour can be benign or malignant (refer box above); solid or cystic. Solid tumours are commonly malignant (85%); cystic tumours are usually benign.

Fig. 15.5: Retroperitoneal leiomyosarcoma, CT picture.

Malignant retroperitoneal tumour may be mesodermal (75%), neuroectodermal (24%) or embryonic remnant (1 %) in origin. Rarely it can be urologic or metastatic also. Sarcoma is the most common malignancy; in adult--liposarcoma is the most common retroperitoneal sarcoma (50%); leiomyosarcoma is 30%; malignant nerve sheath tumour (5%); fibrosarcoma (5%); malignant fibrous histiocytoma (MFH-10%) can also occur. 20% show neurological deficit; 40% show distant blood spread. In children rhabdomyosarcoma is the most common one. 15% of all sarcomas are of retroperitoneal.

Fig. 15.6: Retroperitoneal tumour, on table finding. It was large and inoperable.

I Aetiology Retroperitoneal tumors may be associated with radiation exposures to chemicals like vinyl chloride/thorium dioxide, Figs. 15.4A and B: Retroperitoneal tumour-benign lipoma was excised with a loin retroperitoneal approach.

familial Gardner's synd rome, familial retinoblastoma or familial neurofibromatosis, Li Fraumeni syndrome, germline mutations of p53 in chromosome 17.

Time neither subtracts nor divides, but adds to such a pace it seems like multiplication.

==

(II Q)

en

"Cl Q)

n

(II

712

I Features Tumor arises from fat, areolar tissue, vessels, nerves, skeletal and smooth muscle, fascia, lymph node and lymphatic systems and tissues of embryologic origin. Sarcomas can be commonly solid, cystic or mixed. Usually tumour develops a pseudocapsule. Liposarcoma often originates from (>30%) perinephric fat. Liposarcoma can be well-differentiated (60%) or und ifferentiated (dedifferentiated). It spreads along the fascial planes enveloping the organs. Infiltration of organs occurs only at late stage. It shows whorl-like pattern in histology with myofibroblastic or osteoblastic differentiation. Insulin like production by tumour or rapid utilisation of glucose by active tumour causes intermittent hypoglycaemia (paraneoplastic). Spread of retroperitoneal sarcoma mainly occurs through blood to lungs and often to liver. Nodal spread occurs rarely (5%). Malignant tumours of the retroperitoneum are 4 times common than benign one. Retroperitoneum is the 2nd most common site of malignant mesenchymal tumour first site is being lower extremities.

I Presentations Most patients have initial asymptomatic course with eventual presentation as mass abdomen (90%). Pelvic mass (20%), vague abdominal pain and discomfort (70%), anorexia, fatigue, vomiting, weight loss, fullness in abdomen, back pain due to compression over lumbar and pelvic nerves, leg pain, recurrent episodes of fever-are other features. Other presentations are-GI bleeding; intestinal/urinary obstruction (10%); neurological manifestations (20%) due to compression or invasion of neurological systems by tumor; compression of iliac vein can cause lower limb varicosities, varicocele, and dilated abdominal veins with venous flow from below upwards, and oedema.

I Investigations CT abdomen-helical contrast CT (commonly used) and pelvis and CT chest (to see metastases) are essential investigations. CT-guided core biopsy, laparoscopic biopsy are needed. FNAC has got limited role.

Fig. 15.8: Retroperitoneal tumour showing encasement of major vessels. It becomes inoperable. Ureter, duodenum, intestines, kidney are other structures which may get infiltrated by tumour.

MRI is better than CT with near 100% accuracy. It identifies extent, desmoplastic reaction. Liposarcoma shows decreased signal in T1 and increased signal in T2. MFH shows heterogenous signals. Retroperitoneoscopy may be ideal to take biopsy. IVU shows laterally deviated ureter. Plain X-ray may show soft tissue shadow, obliterated psoas shadow, and often calcification. AFP, HCG and other tumour markers are often done. LFT, blood urea and serum creatinine is done. Histochemistry is essential in many suspicious types.

T

TNM staging (AJCC 8th edition, 2018)

Tumour {T) Metastasis (M) Tx: Cannot be assessed cMO: No distant metastases TO: No tumour cM1: Distant spread present T1 : 5 cm or less pM1: Microscopic distant spread T2: 5-10 cm T3: 10-15 cm T4:>15cm Nodal (NJ NO: No regional nodes N1 : Nodes present

Figs. 15.7A to C: Retroperitoneal tumour presenting as large mass abdomen with all features. CT pictures of the same patient.

FNCLCC histological grading based on (A) Tumour differentiation (score--1, 2, 3); (B) Mitotic count (score-1: 9/10 HPF; 2: 10-19; 3: >20); (C) Necrosis (score 0: no necrosis; 1: 50%). Gx: Grade cannot be assessed. G1 : Total score 2 or 3; G2: Total score 4 or 5; G3: Total score 6,7 or 8.

Staging Stage IA:T1 NO MO G1 /GX; Stage IB:T2, 3, 4 NO MO G1/GX; Stage II: T1 NO MO G2/G3; Stage II/A: T2 NO MO G2/G3: Stage 1118: T3, 4 NO Mo G2/G3; any T N1 MO any G; Stage IV: Any T any N M1 any G.

I Treatment

713

Surgery is the main modality of therapy with wide excisioncomplete enblock excision (possible only in 30% of cases) is the ideal. Limited resection; debulking (controversial) are other options. Often it needs extensive surgical dissection; resection of adjacent bowel. Wide exposure is the key for successful surgery (lengthy midline/thoracoabdominal incision). Limiting factors are encasement of major vessels; invasion of ureters; extension into duodenum or pancreas; extensive involvement of mesentery and spread. Proper bowel preparation, colostomy, urinary diversion, adequate blood to replace the lost blood, hypotensive general anaesthesia are the requirements during surgery. Posterior dissection along the IVG, aorta and near spinal canal are the difficult areas of dissection. Complications are-injury to major structures, bleeding, sepsis, and leak. Postoperative chemotherapy and radiotherapy is used as adjuvant method. Radiotherapy may be external beam RT (5000 cGy); or !ORT (intraoperative RT during surgical resection) which reduces local recurrence and radiation enteritis. Problems with RT are radiation injury to kidney, bowel, liver and spinal cord. Preoperative intensity modulated RT (IMRT) is tried. Recurrent disease needs re-exploration but often difficult to do complete clearance. Mesna, Adriamycin (main drug), ifosfamide and dacarbazine (MAID) are the chemotherapeutic agents used. Germ cell tumour, lymphoma (NHL) are treated accordingly.

::::c

... 0

"Cl (D

Figs . 15.9A and B: Retroperiton eal lymph node mass. It could be tuberculosis/lymphoma/secondaries/autoimmune lymph node enlargement.

0 :::s (D Q)

en

"Cl Q)

B

n

(D

FEATURES OF RETROPERITONEAL SARCOMAS

Mass per abdomen Pain in the back and compressive features GIT obstruction especially by leiomyosarcoma Compression over the ureter causing hydronephrosis Compression over the major veins causing varicose veins Arterial compression features are not common Liver and lung secondaries can occur CT-guided biopsy is ideal method of investigations

I Prognosis Prognosis depends on: ,. Tumour grade- low or high; ,. Resectability; Invasion into organs, veins, nerves; ,. Size of tumour 5 cm or> 1Ocm; ,. Deep location; Nonliposarcoma in histology-poor prognosis; , Recurrent disease; ,. Distant spread. ' Tumour grade and completeness of resection without distant spread are important prognostic factors. ,. RP tumours has got overall 40% recurrence rate. Overall survival is 1½ to 2 years. ,. 5-year surrival for well-differentiated tumour is 75%;

ITI

:::z:,

..... c.n •!• ;a

-... CD

0

...

-c

CD

;:::;: 0

::, CD

0)

Fig. 15.12: Psoas abscess.

Differential diagnosis: Iliac artery aneurysm; Ovarian cyst in females; Soft tissue tumours and cysts in the iliac region; Hernia. Investigations: X-ray spine and chest, CT scan; Mantoux test, ESR, peripheral smear; Ultrasound abdomen.

Figs. 15.13A and B: (A) CT picture of large psoas abscess;

(B) On table finding of psoas abscess.

Figs. 15.14A to C: Bilateral large psoas abscess-CT picture. Drainage and placement of Malecot catheters for the same. Note the location of incisions. It is obliquely placed incision above the lateral aspect of groin with extraperitoneal approach.

It is what a man thinks of himself that really determines his fate.

Chapter

Differential Diagnosis of Mass Abdomen ( ;HAPTER OUTLI NE

•

• •

• •

Mass in the Right Hypochondrium Mass in the Epigastrium Mass in the Left Hypochondrium Mass in the Lumbar Region Mass in the Umbilical Region

• •

• •

Mass in the Right Iliac Fossa Mass in the Left Iliac Fossa Mass in the Hypogastri um Distal Rectal Examination for Prostate and Other Conditions

In a patient presenting with mass abdomen, generally following clinical features should be assessed carefully. Pain: Site, nature, aggravating or relieving factors, duration of pain, referred pain. Vomiting-. Type, content, haematemesis, relation to food, frequency. Jaundice-. It is an important factor in relation to liver, gallbladder or pancreatic masses.

Fig. 16.1: Obstructive jaundice in a patient with carcinoma head of pancreas. Note the sclera for discolouration. Severe itching is common in these patients.

Bowel habits: Constipation, diarrhoea, bloody diarrhoea, furious diarrhoea, tenesmus. Decreased appetite and weight. Inspection of the mass: Anatomical location, margin, surface, movement with respiration. Palpation of the mass: Site, extent, surface, tenderness, consistency, movement with respiration, mobility, borders, plane of the swelling (by leg raising test), presence of other masses.

Fig. 16.2: Abdomen should be inspected by exposing from nipple level to knee level. Inspection is done from foot end and from right side.

Often mass needs to be examined for change of positiol}-in sitting, in standing, in side position, after a brisk walk, in knee elbow position for retroperitoneal mass and for puddle sign (but difficult to keep patient in this position). Percussion is an important aspect of examination in case of an abdominal mass. Percussion over the mass is important to predict the anatomical location of the mass. If mass is dull, then it is in the anterior abdominal wall or in front of the bowel intra-abdominally like liver, spleen, gallbladder. If the mass is with a impaired resonant note, then the mass is arising from the bowel like stomach, colon, small bowel. If the mass is resonant on percussion, then the mass is probably in the retroperitoneal region. Other than this, liver dullness, free fluid in the abdomen should be elicited during percussion. Per rectal examination: It is done to look for any secondaries in rectovesical pouch, primary tumour or relation of lower abdomen masses (pelvic masses). Pervaginal examination is done to assess pelvic masses.

717

C

Fig. 16.5: Abdomen mass should be palpated clinically properly.

Fig. 16.3: Superior vena caval obstruction causing dilated veins in the neck, chest wall and shoulder. Note the neck swelling extending into the mediastinum.

3:: I»

en en

>

= 3 CT Cl Cl)

Fig. 16.6: Percussion over the mass is absolute need to find outthe plane of the mass. Anterior masses are dull on percussion; posterior masses are resonant and mass from bowel has impaired resonance.

Fig. 16.7: Digital examination of rectum is a must in patients with mass abdomen to look for secondaries, possible primary and often to assess the mass itself.

Figs. 16.4A and B: Inferior vena caval obstruction causing dilated veins over the lateral aspect of the flank with flow of blood upwards.

Fig. 16.8: Neck should be examined for left supraclavicular lymph node enlargement in all abdominal masses.

Diagnosis of diseases is often easy; often difficult and often impossible.-William Harvey

::I

718

-

i!'

a, Cl

....

Right upper

::I

-

Left upper

Cl)

L:::

0

Cl:I

::I C Cl:I

_en

~!:r lo~

I

7

8

9

Pancreatic mass

Aortic aneurysm

Kidney mass

C0

a: Cl)

Figs. 16.9A and B: (A) Different quadrants in the abdomen. They are four in number formed by two lines-one is vertical midline through the umbilicus; another is horizontal line passing through the umbilicus. Quadrants are-right upper, right lower, left upper and left lower, (B) Regions in the abdomen. lntussusception

1. 2. 3. 4

Right hypochondrium Epigastric region Left hypochondrium R'ght lumbar region mbilical region

Mass in RIF

Pelvic mass

Left lumbar region Right iliac Iossa Hypogastrium Left iliac Iossa

-------

Abdomen is divided into nine regions by tour lines. 1. Upper horizontal or transpyloric line is mid-way between the umbilicus and xiphisternum. 2. Lower horizontal line is transtubercular line at the level of two tubercles on the iliac crest. 3. Right vertical line is the line through the midpoint of right anterosuperior iliac spine and pubic symphysis. 4. Left vertical line is the line through the midpoint of left anterosuperior iliac spine and pubic symphysis.

MASS IN THE RIGHT HYPOCHONDRIUM

I Liver Palpable as Mass in Right Hypochondrium It is horizontally placed. It usually moves with respiration. Upper border is not felt. It is dull on percussion (This dullness continues over liver dullness above). Fingers can not be insinuated under right costal margin. Conditions where liver gets enlarged: 1. Soft, smooth, nontender liver:

,,. Hydrohepatosis. It is due to obstruction of CBD causing dilatation of intrahepatic biliary radicles. , Congestive cardiac failure. , Hydatid cyst of the liver: Here mass is well-localised in the liver with typical hydatid thrill. Three finger test: Three fingers are placed over the mass widely. When central finger is tapped fluid movement is elicited in lateral two fingers.

Left and right lobe liver mass

Gallbladder mass

• Pyloric mass

Pseudocyst of pancreas

Fig. 16.10: Mass abdomen.

1. Stomach mass. 2. Pancreas body mass.

3. Colonic mass. 4 . Mesenteric cyst.

5. Omental mass. 6. Carcinoma caecum. 7. Appendicular mass. 8. lleocaecal tuberculosis

9. Pelvic mass. 10. Gallbladder. 11 . Umbilicus. 12. Liver.

Fig. 16.11: Different causes of mass abdomen.

2. Soft, smooth, tender liver: ,. Amoebic liver abscess: Here liver often gets adherent to the anterior abdominal wall and will not move with respiration. lntercostal tenderness, right-sided pleural effusion are common. 3. Hard, smooth liver: ,. Hepatoma (HCC): Here a large, single, hard nodule is palpable in the liver. But occasionally there can be multiple nodules when it is multicentric. Rapidly growing tumour can be soft also. Hepatoma often can also be tender due to tumour necrosis or stretching of the liver capsule. Vascular bruit may be heard over the liver during auscultation. It mimics amoebic liver abscess in every respect. ,,. Solitary secondary in liver.

I Other Masses in the Right Hypochondrium Pericholecystic inflammatory mass: It is tender, smooth, firm or soft, non mobile, intra-abdominal mass often with guarding. Kidney mass arising from upper pole of the kidney. It may be due to renal cell carcinoma or hydronephrosis.

719

C"')

:c

>

"'C

-I m ::z, -"

0,

-... C

(D

Secondary

C "'__j

(D

:::,

= Q)

C

m

Figs. 16.13A and B: Multiple secondaries in liver with umbilication. It is due to central necrosis. Secondaries are the most common malignant tumour of the liver. It could be from GIT or extra-gastrointestinal sites like from breast, lungs, melanoma, thyroid, prostate, kidneys, etc. Patient with liver secondaries has got poor general condition. It should be differentiated from multicentric hepatoma. It is usually treated by palliative chemotherapy. Solitary secondary from carcinoma colon can be removed by segmentectomy.

i»'

IC :::, 0 Cl)

;;·

0

3:

Q) Cl) Cl)

:z:,, O" C. 0

3 (D

:::,

MASS IN THE EPIGASTRIUM

Figs. 16.12A and B: (A) Hepatocellular carcinoma/hepatoma. It is common in right lobe, unicentric, attains large size; (8) Large upper

abdomen mass-could beliver mass, pancreatic mass, retroperitoneal mass. 4. Hard, multinodular liver: ,. Multiple secondaries in liver: Here hard nodules show umbilication which is due to central necrosis. ,. Macronodular cirrhotic liver.

I Palpable Gallbladder in Right Hypochondrium It is smooth and soft (except in carcinoma gallbladder). It is mobile horizontally (side-to-side). It moves with respiration. It is located right of the right rectus muscle, below the right costal margin or below the lower margin of the palpable liver. It is dull on percussion.

Conditions where gallbladder is palpable: 1. Soft, nontender gallbladder. ,. Mucocele of the gallbladder. ,. Enlarged gallbladder in obstructive jaundice due to carcinoma head of the pancreas or periampullary carcinoma or growth in the CBD.

2. Hard gallbladder: ,. Carcinoma gallbladder. 3. Tender gallbladder-empyema GB.

I Palpable Left Lobe of the Liver It is in the epigastric region. Its upper border cannot be felt. It moves with respiration. It extends towards left hypochondrium. It is dull on percussion.

B

CONDITIONS WHERE LEFT LOBE OF THE LIVER IS PALPABLE

Hepatoma Amoebic liver abscess in left lobe

• Left lobe secondaries Hydatid cyst of the left lobe

I Features of Stomach Mass It is located in the epigastric region. It moves with respiration. It is intra-abdominal. It is resonant or impaired resonant on percussion. Mass may be better felt on standing or on walking. Mass is often mobile, unless it gets adherent posteriorly. In pylorus mass, all margins are well felt which is mobile with features of gastric outlet obstruction. Mass from the body of the stomach is horizontally placed without any features of obstruction. Mass from the upper part of the stomach near the OG junction causes dysphagia.

As beds and couches vary in height, the examiner to palpate the abdomen, if need be, must sit on a suitable chair or even kneel upon the floor, no matter how undignified this may appear. - Charles P

720

...

a., Cl

Mass from the fundus of the stomach is in the upper part of the epigastric region towards left side. Carcinoma stomach is nodular and hard. It is the most common cause for stomach mass. Leiomyoma of stomach is smooth and firm.

Lower border is well felt. Upper border is not clear. It is resonant on percussion. Baid test As the stomach is pushed in front, Ryle's tubewhen passed, can be felt per abdomen on palpation.

::s

Cl)

0 «I

::s C «I

==

"' iio

a: Cl)

•

Dilated stomach

I

umour Umbilicus

m Umbilication

Figs. 16.16A and B: (A) Pseudocyst of pancreas-CT scan picture; (B) Pseudocyst of pancreas presenting as epigastric mass.

I Cystadenocarcinoma of the Pancreas Mass is smooth, firm, does not move with respiration, nonmobile, resonant on percussion. Patient complaints of back pain. Carcinqma stomacn Secondary (primarh nodules

@

----~

§

Figs. 16.14A to E: Carcinoma pylorus causes gastric outlet obstruction with palpable mass above the umbilicus. Carcinoma body of stomach mainly presents as loss of appetite and decreased weight with horizontally placed stomach mass. Carcinoma from fundus of the stomach presents as mass abdomen with loss of appetite and weight. Carcinoma oesophagogastric (OG) junction presents as dysphagia. Carcinoma stomach is one of the common causes of secondaries in liver.

I Colonic Mass It is due to carcinoma of transverse colon. It is mobile, horizontally placed, nodular, hard mass which does not move with respiration. Caecum will be dilated and palpable. It is resonant or impaired resonant on percussion. Patient will be having bowel symptoms, loss of appetite and decreased weight.

I Para-aortic Lymph Node Mass Mass in the epigastric region which is deeply placed, nonmobile, not moving with respiration. It is vertically placed, above the level of the umbilicus and resonant on percussion. Causes for enlargement are: Secondaries, lymphomas or tuberculosis.

I Aortic Aneurysm It is smooth, soft, pulsatile (expansile pulsation which is confirmed by placing the patient in knee-elbow position). It is vertically placed above the level of the umbilicus, nonmobile, not moving with respiration and resonant on percussion. Fig. 16.15: Epigastric mass arising from carcinoma stomach.

I Pseudocyst of the Pancreas Mass in the epigastric region. It is smooth, soft. It can be tender if it is infected. It does not move with respiration. It is not mobile. It has got transmitted pulsation. It is confirmed by placing the patient in knee-elbow position.

MASS IN THE LEFT HYPOCHONDRIUM

I Enlarged Spleen

Spleen has to enlarge three times to be palpable clinically. It enlarges towards the right iliac fossa from left costal margin. It moves with respiration, mobile, obliquely placed, smooth, soft or firm , with a notch on the anterior edge which is directed downwards and inwards. Fingers cannot be insinuated over the upper border.

721

" Hook sign" is positive, i.e. one cannot insinuate the fingers under the left costal margin. It is dull on percussion.

C"">

:c

>

m :c

..... C,

r.J Figs. 16.19A and B: Renal (Kidney) punch. Renal angle should be palpated for tenderness. Renal angle is junction of 12th rib and erector spinae. On percussion it is normally resonant as it is occupied by colon. It is replaced by kidney in kidney enlargement. Renal angle should be inspected, palpated and percussed in sitting position from behind.

C

= .... CD

CD ::::I

= I»

Figs. 16.17Aand 8: (A) Hepatosplenomegaly is the common condition (clinical entity). It is due to macronodular cirrhosis with portal hypertension, lymphoma, autoimmune diseases, congestive cardiac failure, hepatoma with portal hypertension, haemolytic diseases, etc. There may be ascites, supraclavicular palpable lymph node and pleural effusion (right-sided); (B) Method of palpating spleen.

OLeft-sided Colonic Mass It is mobile, nodular, resonant. It does not move with respiration. It is commonly due to carcinoma colon.

i Left Renal Mass from Upper Pole of-any Cause -It has got features of renal mass.

I Left-sided Adrenal Mass It does not move with respiration. It is not mobile. It is deeply placed mass. Often it crosses the midline. It is resonant on percussion. It mimics kidney mass.

I Mass Arising from the Tail of the Pancreas

Clinical features are same as other pancreatic masses. Causes are pseudocyst in tail of the pancreas and cystadenomas.

MASS IN THE LUMBAR REGION

I Palpable Kidney Mass There is fullness in the loin which is better observed in sitting position. Mass moves with respiration. It is vertically placed. It is bimanually palpable. It is ballotable. Renal angle is dull on percussion (normally it is resonant due to colon). There is a band of resonance in front due to reflected colon. It does not cross the midline.

Conditions Where Kidney Gets Enlarged Hydronephrosis:

It is smooth, soft, lobulated, nontender mass, nonmobile. Pyonephrosis:

History of throbbing pain in the loin, pyuria and fever with chills. It is smooth, soft and tender kidney mass, nonmobile. Po/ycystic kidney:

History of loin pain and haematuria. Hypertension, anaemia and features of renal failure. Usually bilateral. But one side can present early than on t he other side. Lobulated smooth surface. Renal cell carcinoma:

History of mass in the loin, haematuria, fever and dull pain. Mass is nodular and hard. It does not cross the midline. Initially mobile; eventually it infiltrates gets fixed and becomes non mobile.

Mass from the Ascending Colon on Right Side or Descending Colon on Left Side Fig. 16.18: Kidney should be palpated bimanually with two hands. Ballotability also should be checked.

History of altered bowel habits with decreased appetite and weight.

After every end is a new beginning and every beginning has an end.

C

= I» ::::I 0

en

in"

s.

3:

I»

en en

>

er

C. 0

3

CD ::::I

722

Mass is nodular, hard which does not move with respiration and is not ballotable. It is resonant or there is impaired resonance on percussion. Renal angle is resonant. Proximal dilated bowel may be palpable.

I Adrenal Mass It is nodular and hard. It does not move with respiration. It is not mobile and often crosses the midline. It is felt on deep palpation. It is resonant in front. It is not ballotable.

I Retroperitoneal Tumours They are not mobile, resonant and do not fall forward in knee-elbow position. They are deeply placed mass which are usually smooth and hard. They may be retroperitoneal sarcomas or teratomas or lymph node mass.

Fig. 16.20: Palpation of mass in knee-elbow position. Mobility and

falling forward should be confirmed in knee-elbow position.

I___Retro__,peritoneal Cysts _ __ _ _.__ _ _ _ _ _ _ _ _ __ They are smooth and soft with the same features as retro peritoneal tumours.

B Mucocele/empyema of gallbladder Pseudocyst of pancreas Hydatid cyst of liver Congenital nonparasitic cyst of liver Hydronephrosis Mesenteric cyst

Ovarian cyst Omental cyst Aneurysm Retroperitoneal cyst Cystadenocarcinoma of ovary Loculated ascites

MASS IN THE UMBILICAL REGION

B

USUAL MASSES ARE:

Mesentenc cyst Omental cyst Ovarian cyst (pedunculated) Small bowel tumours Extension of masses from other region Transverse colon mass

Mass 1n the body of pancreas Mesentery mass Lymph node masssecondaries (primary from GIT, testis, ovary, melanoma)/ lymphoma/tuberculosis Retroperitoneal tumour

Mesenteric cyst , Ti/faux triad: 1. Soft intra-abdominal umbilical mass. 2. Mobile in the direction perpendicular to the attachment of the mesentery. 3. Resonant mass. , May precipitate intestinal obstruction, volvulus. Omental cyst: It is smooth, soft and nontender; It moves with respiration. It is mobile in all directions; It is dull on percussion. Small bowel swellings: Small bowel lym phomas; Small bowel carcinomas; lntussusception.

lntussusception

Fig. 16.21: Retroperitoneal tumour.

Mass in umbilical region usually towards left and above the umbilicus. Occasionally towards right side. Mass is intra-abdominal which is sausage shaped, with concavity towards umbilicus, well-defined, smooth , firm and mobile. Mass does not move with respiration. Mass contracts under palpating fingers. Often mass disappears and reappears. Mass is resonant or there is impaired resonance on percussion. "Red currant ge/ly" stool with features of intestinal obstruction may be present.

MASS IN THE RIGHT ILIAC FOSSA

Fig. 16.22: Mass in right iliac fossa. All possible differential diagnosis should be considered and clinically analysed. Common masses are appendicular mass; carcinoma caecum; ileocaecal tuberculosis; lymph node mass; ameboma.

• Appendicular mass or abscess • Carcinoma caecum lleocaecal tuberculosis Amoeboma • Psoas abscess • Lymph node mass either mesenteric or external Iliac lymph nodes Bony swellings

I•

Ectopic kidney Undescended testis (Abdominal) Actinomycosis Crohn's disease Iliac artery aneurysm Ovarian swelling-ovarian cyst Tubo-ovarian mass Uterine mass like pedunculated fibroid

Appendicular mass: It is smooth, firm, tender mass in the right iliac fossa; It is not mobile. It does not move with respiration; It is resonant on percussion. It is well-localised mass with distinct borders. Appendicular abscess: It is smooth, soft, tender and dull mass in the right iliac fossa with indistinct borders. Carcinoma caecum: It is nodular, hard, mass in the right iliac fossa; It does not move with respiration; It is mobile but mobility may be restricted once it gets adherent to psoas muscle; Mass is resonant or there is impaired resonance on percussion; Often features of intestinal obstruction may be present. 1/eocaecal tuberculosis: Mass in the right iliac fossa which is smooth, hard, resonant and nontender; It does not move with respiration and has restricted mobility; Caecum may be pulled up to lumbar region due to fibrosis. Amoeboma: History of dysentery with pain in the right iliac fossa; Smooth, hard, well-defined mass in the right iliac fossa which is nonmobile; It may or may not be tender. Psoas abscess: It is localised, smooth, soft, non mobile mass in the right iliac fossa; Psoas spasm (flexion of the hip joint) is typical; Spine may show gibbus, tenderness, paraspinal spasm. Spinal movements will be restricted.

MASS IN THE LEFT ILIAC FOSSA • Carcinoma sigmoid or descending colon • Bony masses • Ovarian/uterine masses

Psoas abscess Ectopic kidney Lymph node mass Undescended testis

MASS IN THE HYPOGASTRIUM Bladder mass: It is in the midline. It is dull on percussion. Lower border is not felt; It can be mobile in horizontal direction. Mass reduces in size after emptying the bladder. It can be felt on per-rectal examination; It is either carcinoma bladder (common) or leiomyoma or sarcoma bladder. Uterine mass: It is midline mass which is smooth, hard; Lower border is not felt which extends into the pelvis; It is felt on pervaginal examination. Ovarian mass ,, Pelvic soft tissue mass. ,, In all lower abdomen masses PIR and/or PN is a must. ,. In all regions parietal masses can occur: Benign and malignant soft tissue tumours; Common, is lipoma; Fatty hernia of linea alba; Desmoid tumour; Parietal wall abscess. Blaxland ruler test (Athelstan J Blaxland): A flat ruler placed on the lower abdomen just above the anterosuperior iliac spines and pressed firmly backwards. In ovarian cyst, aortic pulsation is transmitted to fingers through ruler; it is not so in ascites.

B The patient, in lying down position, is asked to lift both legs off the bed with knee extended. This puts the abdominal muscles into contraction. Intra-abdominal mass becomes less prominent, parietal mass persists as same, but becomes less mobile.

Fig. 16.23: Looking for minimal ascites in knee-elbow positionPuddle sign.

I Investigations for Mass Abdomen Haematocrit, liver function tests, renal function tests, stool/ urine examination. Ultrasound abdomen. Endoscopies-gastroscopy-colonoscopy-ERCP-MRCP. Barium studies-Barium meal-Barium enema-Barium meal follow through.

CT scan-MRI. Endosonography. Ascitic tap. Diagnostic laparoscopy. Ultrasound-guided/CT-guided biopsy.

Smiles are free-don 't save them. The world always looks brighter from behind a smile.

723

C I» ICI

= 0 en

en· s. 3::

I»

en en

>

CT

Cl. 0

3

(I)

=

724

IVU/RGP/Cystoscopy/lsotope renogram. Exploratory laparotomy.

B

B

I

Hard mass in the abdomen is commonly malignant Firm mass may be tuberculous, lymphoma or many benign conditions Soft masses are hydronephrosis, pseudocyst, mesenteric cyst, omental cyst, and loculated ascites In tuberculosis abdomen may be doughy due to thickened parietal peritoneum or omentum It is difficult to elicit fluctuation in the mass abdomen as mass cannot be fixed properly Plane of the swelling should be checked by leg raising/head raising I tesWalsalva manoeuvre/knee elbow test Bimanual palpation, ballotability, renal angle inspection, palpation and percussion should be done in case of renal mass Intrinsic mobility should be checked. Different mobilities/movements are-gallbladder shows side to side; stomach lateral; ovarian mass-all over; mesenteric cyst- right angle to line of mesentery; transverse colon mass- vertical; small bowel mass all over; appendicular mass/pancreatic mass/nodal mass (para-aortic)/retroperitoneal mass do not show any mobility; cystadenocarcinoma of pancreas may show false mobility (tree top mobili(Y'J Percussion is very important method of examination to find out the anatomical plane of the mass. Mass in front of the bowel like liver/spleen/gallbladder/parietal mass are dull on percussion. Mass from the bowel is resonant on percussion like from stomach, small bowel and colon. Retroperitoneal masses like cyst, sarcoma, nodal mass, aneurysm, pancreatic mass are resonant on percussion Succussion splash and auscultopercussion tests are done for gastric outlet obstruction in pyloric stenosis Digital examination of rectum (PR) and left supraclavicular examination for nodes is a must. PR is done to see Blumer shelf secondaries in rectovesical pouch. Pervaginal examination should be done in pelvic mass in females Bladder should be emptied while examining pelvic mass Bimanual examination is done often under general anaesthesia in case of pelvic mass I Auscultation for bruit depends on condition and location of mass-over epigastrium, over liver Other relevant systemic examination is amust in examination of mass abdomen-respiratory system, skeletal and neurological systems

Pseudocyst of pancreas (communicating) Hydronephrosis (intermittent)

Choledochal cyst lntussusception

I Srinivasan Costal Sig._n_ _ _ _ _ _ __ Srinivasan costal sign is an inspectory fin ding to assess the acute distended abdomen secondary to surgical or post-operative status or medical causes under treatment. The bilateral costal margin is visualised tangentially on the either side of the abdomen in supine position to appreciate this costal sign.

• Visible costal margin

I I

Figs. 16.25A to C: Srinivasan costal sign: This sign can be appreciated under inspection, the costal margin which is not visible or poorly visible in case of distended abdomen. Once the acute abdomen or distended abdomen secondary to medical cause start settling, the costal margins become visible and prominent, this holds good even in case of tatty protuberant abdomen.

"Prominence of costal margins will be lost in distended abdomen. During therapy, once distension reduces, prominence of costar margins will be visible (during recovery)".

'---

Acknowledgements With kind permission, from Professor Narayanaswamy Srinivasan, MS, FRCS, who was earlier worked as HOD, General Surgery, Bangalore Medical College; Director of Sanjay Gandhi Accident Hospital and Research Institute, Byrasandra, Bengaluru; Dean and Director of Bangalore Medical College and Research Institute, Bengaluru; Joint Director of Medical Education , Government of Karnataka, India.

DIGITAL RECTAL EXAMINATION FOR PROSTATE AND OTHER CONDITIONS Fig. 16.24: Abdominal wall tumour (lipoma).

(By kind permission from Professor Laxman Prabhu, MS, MCh, Urologist, Kasturba Medical College, Mangaluru)

i

Rectum is the Dictum

- Anonymous

The prostate by virtue of its placement just anterior to the rectum easily tends itself to digital examination. This makes Digital rectal examination (DRE) of the prostate not only easy but an effective method of clinical assessment of prostatic diseases. The prefix 'digital' to rectal examination of the prostate became a necessity with the advent of transrectal ultrasound of the prostate (Watanabe et al 1968, 1974) when the two methods of examining the prostate, namely digital (clinician's forefinger) and sono/ogical per rectum re established as reliable clinical tools. ~ ---------

I

I

B· -

I1. Grad;ngotb8~

2. Local staging of prostatic cancer and in the performance of digitally guided biopsy. 3. In the evaluation of inflammatory disorders of the prostate. 4. To assess the integrity of prostatomembranous urethra in suspected urethral injuries. ~ n the evaluation of ejaculatory dysfunctions.

I

Note: • Few generalizations in DRE are essential. The need for a rectal examination is explained to the patient and his consent is taken. The patient is always requested to empty the bladder. During this, urinary samples of initial stream and midstream are collected for culture in sterile widemouthed receptacles. Abdominal examination to rule out a distended bladder should always precede examination of the prostate. Findings on DRE of the prostate are unreliable in the presence of a lull bladder. Posterior surface of the distended bladder can be erroneously labeled as a large prostate. Urollowmetry can be performed during voiding to empty the bladder whenever appropriate or if such facility is accessible. • It is absolutely essential to ensure privacy. Chaperoning (as insisted while examining a female patient) is not necessary but a caretaker may be allowed to stand next to the patient, especially when the patient is very old and dependent on assistance; presence of a female nurse is better. • DRE can be uncomfortable in those patients who are apprehensive and this may persuade the inexperienced clinician to conclude that the patient has a tender prostate. • It is recommended that DRE is done only after collecting blood for prostatic specific antigen (PSA) assay as digital manipulation can falsely raise the PSA value (

,,, "'C --4

:a

..... en C

C

iii'

IC ::I 0

~en

s. s: D,I

en en

l>

CT

C. 0

3

Cl)

::I

726

...

Q)

Cl :::, Cl)

ci

I DRE in Benign Enlargement of the Prostate The prostate feels typically elastic in benign enlargements implying the gland yields to the pressure exerted by the examining digit only to recoil. When fibrous elements predominate, it feels firm. When the lateral lobes enlarge, they cause convex bulges into the rectal lumen which is invariably apparent to the examining finger. The enlargement of lateral lobes could be asymmetric. It is difficult to make out a purely median lobe enlargement. A median lobe can be felt against a bougie, introduced just before DRE. Rarely, a large median lobe can be felt bimanually in a thin subject. The rectal mucosa can be moved over a benign enlargement, exception being a massive enlargement stretching the anterior rectal wall. The surface of a benign enlargement is usually smooth. A benign prostate retains its mobility which is checked by gently pushing the gland forwards and upwards. A bougie or cystoscope sheath can be used to depress the prostatic urethra to demonstrate this mobility. It is common practice among urologists to grade prostatic size by DRE. The various grades (Normal , Grades 1 to 4) have been published in the endoscopy volume of Encyclopedia Urologica (Roger W Barnes, 1959): Normal: Encroaches 0-1 cm into the rectal lumen Grade 1: Encroaches 1-2 cm into the rectal lumen Grade 2: Encroaches 2- 3 cm into the rectal lumen Grade 3: Encroaches 3-4 cm into the rectal lumen Grade 4: Encroaches more than 4 cm into the rectal lumen Note: The above grading system takes into account only that part

I

of the gland which is bulging into the rectal lumen and hence does not give an accurate estimate of prostatic volume. However, studies have suggested that DRE provides a fairly accurate estimate of the gland size when the prostate measures 50 cm3 or less. The clinical method of grading is as follows: The examining finger is run over the gland from one side to the other and above downwards to determine the summit of thebulge. The finger is then slide towards the lateral sulcus, mentally counting the number of pulp breadths. Breadth of the digital pulp is roughly taken as one centimeter. Total number of pulp breadths gives the measurement of the encroachment into the rectal lumen, which is recorded as a particular grade.

l

I DRE in Prostate Cancer Most cancers of the prostate commence per primam in the peripheral zone (McNeal's Zones) of the prostate which is directly accessible to the examining finger. However small and early cancers (Jewett-Whitmore A or T1 of TNM staging) remain impalpable for a long time. Digital rectal examination therefore can pick up only higher stages of the disease (Jewett-Whitmore B and C or T2 and T3 of TNM staging). Stage T2 cancers are felt as indurated or hard areas within the confines of the gland, involving it to varying extent (half of one lobe-T2a; more than half of one lobe-T2b and both the lobes-T2c). Contoural abnormalities (asymmetry, loss of median sulcus and irregularity) suggest extraprostatic spread (T3a). The areas of hardness may tail into one or both the seminal vesicles (T3b). The rectal mucosa may be tethered in most areas as the cancer spreads. The mobility of the gland is also lost in higher stages of the disease. When

the disease involves the lateral pelvic wall (T4), the situation is called 'frozen pelvis' implying there is total loss of normalcy (vide supra-normal DRE) In the past, clinicians have used a bougie or a Cystoscope as an aid to clinical methods to evaluate prostatic cancer. A bougie or a cystoscope passed into the bladder is gripped by the prostate as though by a rigid ring and a finger in the rectum feels the instrument with great difficulty where it passes through the gland (Gerachty's sign). Benign enlargements can exist pari passu with cancers. It is therefore prudent look for discrete induration within a firm enlargement. Only 50% of abnormal DRE's suggestive of a tumor turn out to be cancers. The fact that an abnormal DRE in the presence of a 'normal' PSA ( -a -I m ::::r::,

..... .....

>

CT

C. 0

3

:::I

I»

a

rn

Fig. 17.3: Umbilical infection causing abdominal wall abscess.

Figs. 17.2A and B: (A) Omphalitis (umbilical infection); (B) Umbilical sepsis with profuse discharge.

I»

UMBILICAL GRANULOMA

Causes: Poor asepsis and umbilical hygiene during delivery and after birth. Bacteria: Staphylococci, streptococci; Gram-negative organisms; Clostridium tetani causing neonatal tetanus. Features: ,. Infection may spread along the hypogastric vessels into the abdominal wall causing abdominal wall abscess. ,. Infection may spread into the peritoneum causing peritonitis. ,. In severe cases, septicaemia can occur. ,. Umbilical granuloma can occur once infection gets localised. ,. Purulent discharge with red, swollen umbilicus. ,. Investigations: Culture study of discharge; USG abdomen.

It is due to chronic infection of the umbilical cicatrix, causing the granulation tissue to pout, leading to the formation of umbilical granuloma. It occurs in any age group, but common in infants and children. Presents as umbilical discharge with tender, red, swelling protruding from the umbilicus which bleeds on touch. It has to be differentiated from the anomalies of vitellointestinal duct. It also mimics umbilical adenoma.

I Treatme:...n_t _ _ _ _ _ _ _ _ _ _ __ Antibiotics. Drainage of the abscess if present. Aseptic precautions while ligating the cord will prevent the chances of umbilical sepsis. Excessive granulation tissue must be removed surgically or by cautery (commonly) using trichloroacetic acid or silver nitrate or copper sulphate in small quantity.

Abdominal wall abscess Extensive abdominal wall ulceration and skin and subcutaneous gangrene formation Septicaemia via umbilical vein Peritonitis carries poor prognosis Umbilical granuloma, umbilical hernia Neonatal tetanus Portal vein thrombosis and portal hypertension Neonatal jaundice due to cholangitis-6 weeks after childbirth due to spread of infection into liver across umbilical vein

a

rn Figs. 17.4A and B: Umbilical granuloma due to sepsis.

•·· Treatment: ,. Antibiotics, application of silver nitrate and dry dressings. ,. Occasionally might require excision of granuloma or umbilectomy (rarely).

ANOMALIES OF VITELLOINTESTINAL DUCT

1

During development of fetus, midgut communicates with yolk sac through vitellointestinal or omphalomesenteric duct. This duct slowly narrows during the development of abdominal wall to lie within the cord, later this communication obliterates to free intestine from yolk sac. Any defect in obliteration causes different anomalies.

Umbilical black eye is Cullen's sign in acute pancreatitis and ruptured ectopic pregnancy.

:::I

C.

c:: 3

CT

n C: en

730

... = Cl)

Cl

-

Cl)

0

cu

= C

cu ::::E

en

a:i a: Cl)

Anomalies are: 1. May remain completely patent, forming an intestinal fistula. 2. Only a small portion near the umbilicus may remain patent forming discharging umbilical sinus. Often the mucosa of this retained portion (epithelial lining) protrudes or everts to form umbilical adenoma. 3. Duct is closed on either side, but the intervening portion may remain as an intra-abdominal cyst. 4. Vitellointestinal duct which is obliterated can retain as a band which may be a seat for intestinal obstruction, volvulus, internal herniation. 5. Intestinal end may remain patent forming Meckel's diverticulum, which may be attached to umbilicus with a fibrous band . Meckel's diverticulum itself can cause diverticulitis, obstruction.

I!]

Figs. 17.5A to E: Anomalies of vitellointestinal duct. {A) Intestinal fistula; (B) Umbilical sinus; (C) Intra-abdominal cyst; {D) Band; {E) Meckel's diverticulum with band.

m

ANOMALIES OF VITELLOINTESTINAL DUCT

Intestinal fistula Umbilical sinus Umbilical adenoma

Intra-abdominal cyst Meckel's diverticulum Intra-abdominal band

Investigations ,.. Fistulogram; Ultrasound abdomen; Radioisotope study. , If obstruction is present, plain X-ray abdomen in erect posture is useful to visualise the multiple air-fluid levels. Treatment: Excision of vitellointestinal duct is done, along with resection of bowel segment containing Meckel's diverticulum.

UMBILICAL SINUS Causes ,. Persistent vitellointestinal tract partially towards umbilical side; Persistent urachus. ;.. Tuberculosis; Umbilical infection. Umbolith-desquamated epithelium of umbilicus gets collected and inspissated in the umbilical recess causing black/brown coloured stone (umbilical stone). It causes recurrent umbilical infection and sinus. , Pilonidal sinus of the umbilicus. , Malignancy in the urachus. Clinical features ,.. Pain, swelling, discharge and tenderness in the umbilicus. ,,. Features of the specific causes. Investigations ,.. Study of the discharge culture and sensitivity, cytology for malignant cells, AFB study. , Sinusogram, Chest X-ray, ESR. Ultrasound abdomen, CT abdomen. Treatment ,.. Treat the cause; Antibiotics :,.. Antitubercular drugs in case of tuberculosis ,, Umbilectomy.

UMBILICAL ADENOMA (RASPBERRY TUMOUR)

•

Fig. 17.6: Patent vitellointestinal duct.

Fig. 17.7: Umbilical adenoma (Raspberry tumour).

It is commonly seen in infants. It is due to partially obliterated vitellointestinal duct towards umbilical end, causing prolapse of the mucosa giving rise to umbilical adenoma also called as Raspberry tumour. It protrudes out as a red swelling , which is moist with mucus and tends to bleed on touch. It often gets infected, discharging pus through the umbilicus. Histologically, it consists of columnar epithelium rich in goblet cells. Differential diagnosis: Umbilical granuloma. Treatment , If the tumour is pedunculated, a firm ligature is tied round it, so that tumour will fall off in few days. If it reappears, umbilectomy is done. , Sometimes there may be a patent vitellointestinal duct or a Meckel's diverticulum, which may require resection along with the bowel segment.

Urachal cyst if only middle portion of the urachus is patent with lining and fluid content. Urachal diverticulum when bladder side of the urachus is patent.

731

C")

:::c

I Features

>

"'CJ -4

Persistent discharge from the umbilicus often stained with urine, if it is fistula. Recurrent infection and bleeding. Pain in the umbilicus and below. Recurrent urinary infection.

l'TI

::J:I

..... ...., >

c:r

Q.

0

3

::I

QI

QI

::I

Q.

c:: 3

Note: Umbilical adenoma does not disappear after silver nitrate application.

n

UMBILICAL FISTULA Causes: Patent vitellointestinal duct; Patent urachus; Postsurgical; Tuberculosis. Clinical features ,. Faecal discharge or urinary discharge, mucoid discharge. Recurrent infection. Pain, tenderness and excoriation in and around the umbilicus. Investigations ,. Fistulogram, CT fistulogram. Discharge study, CS, AFB and cytology. Ultrasound abdomen. Treatment , Fistulectomy, along with resection of bowel segment, patent vitellointestinal tract, and anastomosis of the bowel. , Fistulectomy and excision of vitelline duct up to the antimesentric surface of the ileum. Opening in the ileum is closed transversely using vicryl or silk sutures.

Urachus fistula (patent urachus)

Urachus sinus

Urachus cyst

Urachus diverticula

Fig. 17.8: Different types of urachal anomalies.

PATENT URACHUS Allantoic duct/stalk which is remnant of cranial part of ventral urogenital sinus forms urachus. It gets fibrosed and forms median umbilical ligament.

When urachus is patent it can form: Patent urachus (Urachal fistula) between umbilicus and dome of the urinary bladder (It is persistent median umbilical ligament). Urachal sinus if only umbilical side of the urachus is patent.

Fig. 17.9: Tuberculosis of urachus which is excised.

Everytime something good happens to you, make something good happen to someone else.

C

rn

732

...= Q)

en

Cf.)

Investigations: Fistulogram to see the extent; US abdomen; Discharge analysis and culture; Urine analysis. Treatment: Surgical excision of the tract; Often umbilical excision may be required.

l_ginical Features (2%) A sudden feeling of giving way from the wound-on 5th to 8th postoperative day often precipitated by bouts of severe cough.

Note: Malignancy (adenocarcinoma) or tuberculosis can occur in patent urachus.

BURST ABDOMEN (Abdominal Wound Dehiscence) (ACUTE WOUND FAILURE) It is disruption of a laparotomy wound, occurring usually between 5th and 8th postoperative day. Usually sutures opposing the deep layers, i.e. peritoneum and rectus sheath tear through, causing burst abdomen-Acute wound failure. It is a mechanical wound failure due to various factors causing separation of the closed abdominal wound often with evisceration of the contents. There is postoperative separation of the abdominal musculoaponeurotic layers. It is 2% common. Common in males. Mortality is more than 30%. Other than technical errors, deep wound infection causes localised separation of the wound which in addition to raised intra-abdominal pressure leads to wound dehiscence. Vertical incision is more prone for dehiscence than transverse as vertical incision cuts across the aponeurosis and when abdominal wall contracts, it creates laterally directed tension on vertical closure. Mass closure is better than layer- by-layer closure. Suturing of the peritoneum is not vital in wound failure; it is the fascial closure which decides the strength. Nonabsorbable monofilament suture material is used for the closure even though evidence says that there is no difference between synthetic absorbable like polyglactic acid and nonabsorbable monofilament suture. Nonabsorbable suture causes prolonged wound pain but is preferred in risk category patients. Suture bite interval should be 1 cm but not more; suture length and wound length ratio should be 4:1 or more but not less.

Fig. 17.10: Abdominal wound dehiscence in a transverse incision with evisceration of bowel; it is rare to have dehiscence of transverse incision.

Figs. 17.11A and B: Burst abdomen wound exposing intestines outside. It is covered with a mesh later.

Fig. 17.12: Ulcer over the postoperative wound.

Choice of suture materials used Method of closure; Tension free suturing is most important. Upper midline and vertical wounds are more likely to disrupt than transverse Surgical wounds of peritonitis, acute abdomen, major surgeries like pancreatic, hepatic, gastric, surgeries for malignancies have a high incidence of disruption Severe cough, vomiting and distension in postoperative period Poor general condition of the patient-anaemia, jaundice, hypoproteinaemia, obesity, uraemia and diabetes mellitus, old age, steroids, radiation, malignancy, chemotherapy. Ascites, previous laparotomies, duration of surgery> 150 minutes are other risk factors.

Pinkish serosanguineous discharge (salmon-coloured large quantity of fluid) from the wound. Often omentum or coils of intestine are forced out of the wound. Clinically burst abdomen can be diagnosed without fail.

Probing of the wound using gloved finger appreciates dehiscence of musculoaponeurotic layer.

Wound usually heals well without much second dehiscence. Late problem, may be development of incisional hernia. ,:. Biological dressings, wound vacuum systems are newer modalities used.

a

733

>

CT

a.

Figs. 17.13A and B: lncisional hernia which was repaired earlier has

0

3

gaped exposing the mesh; (8) Horizontal tension sutures.

::I

Q)

I Treatm_e_n_t __ Nasogastric aspiration, IV fluid s. Emergency surgery, i.e. under anaesthesia, wound is opened up properly. ,. Coils of intestines are replaced into the abdominal cavity. Thorough wash is given. Wound is closed by all layer sutures, passing a nonabsorbable suture material through the red rubber or plastic collar-tension sutures (which is kept for 14 days). ,,. Modified Smead-Jones closure mainly used to prevent burst abdomen-it is interrupted specialised suture used in the closure of abdomen as single layer excluding the skin. Linea alba is held with Allis' forceps. Number one polyethylene or PDS suture material is used. First bite on one side taken 3 cm away (width) from the margin from outside to inside; it is then passed through the corresponding opposite edge with 3 cm width from inside to outside; later again one small loop of 5 mm width from the edges of each side of the wound from first bite site to second bite site is taken; suture is knotted on the free edge of the first bite side. Full thickness bite holds the suture and maintains the tension in the wound. Smaller loop keeps the linea alba in apposed place. Large curved Ferguson needle is better to place these sutures. Each suture is placed at 2 cm interval. This is the type of suturing used at present in acute abdominal conditions instead of the retention sutures. Here also it is better to place all sutures under proper vision and knotting is done at the end. At least four knots should be placed. Excessive tension should be avoided. In upper abdomen peritoneum need not be included in the bite; but in lower abdomen as linea alba is indistinct, peritoneum is included in this. ,,. Subcutaneous drain with loose skin sutures are preferred. In severe infection, skin wound is left open and closed as secondary suturing after 14 days. ,,. Problem with immediate closure with forced attempt may cause abdominal compartment syndrome. Antibiotics and IV fluids are continued.

:e

Q) Q)

::I

a.

rn Figs. 17.14A and B: Vertical tension sutures.

C:

3

CT C")

C

Newer present methods of managing the burst abdomen If the fascial edges are necrotic, proper debridement of the wound edges is done. One should not attempt forcible wound closure as it will increase intra-abdominal pressure further and redehiscence occurs (When fascia is strong and intact primary closure can be done). Wound and contents are covered with absorbable mesh or biological prosthesis like decellularised porcine submucosa and dermis or human cadaveric dermis. They prevent bowel desiccation, bacterial infection. Wound vacuum system using-open cell foam, semiocclusive drape over the foam and suction apparatus. It provides immediate coverage, minimises heat loss, by negative pressure it clears interstitial fluid, reduces the bowel oedema and contamination, increases wound blood flow and promotes wound healing. Once wound granulates wound is closed with primary closure or with skin graft or flaps depending on the size of the wound. Component separation technique may allow primary fascia! closure.

Primary deep fascial closure-deep tension/retention sutures Delayed closure with initial mesh wrap or pack/opposite or Bogota bag Topical negative (vacuum) pressure closure Delayed skin closure or skin graft over the dehiscence wound once it granulates well. Component separation technique with primary fascial closure Placing drain in subcutaneous plane Suitable antibiotic therapy; fluid management Prevention of abdominal compartment syndrome, haematoma or intra-abdominal abscess formation or sepsis Blood or blood product transfusion; nutrition supplemental~

Truth is what is actually should be. Reality is what is happening. Every reality need not be truthfully right.

Cl>

734

ABDOMINAL WALL TUMOURS

J:w~~ ~ ,,.,..w

a

0Jml)@11m1•lii@1~

~ - ~•!&1Jltiml ~~waruru:._,..., m

lmiiiJC!f:D!!l•totii€i~

Figs. 17.15A and B: (A) Abdominal wall secondaries from carcinoma stomach; (B) Sister Mary Joseph (Nee Julia Dempsey, Mayo clinic USA) metastatic umbilical nodule from carcinoma stomach.

They are not uncommon but often present late as they are usually asymptomatic. Common tumours are lipoma, fibroma, neurofibroma, and fibromatosis. Malignant tumours occasionally when occurs, are either from skin or soft tissues. They may be desmoid tumour, soft tissue sarcoma like fibrosarcoma, dermatofibrosarcoma, liposarcoma, umbilical secondaries (Sister Joseph Mary tumour). Presents usually as painless progressive swelling. Otten ulceration can occur. Attaining large size is also known. It is dull to percuss. On contracting the abdominal wall muscles, swelling becomes prominent and less mobile. Differential diagnoses are-abdominal wall abscess, haematoma, intra-abdominal tumours (adherent to abdominal wall). US abdomen, CT abdomen is diagnostic. Biopsy is essential. Treatment is wide excision with adequate clearance with removal of adjacent skin and musculoaponeurotic layer. Defect is covered with a large mesh.

Fig. 17.168

Figs. 17.16A and B: Abdominal wall proliferative ulcer (A) and large tumour, may be fibromatosis of abdominal wall also (B).

DESMOID TUMOUR ( 'Desmo' Means Band/Tendon in Greek) It is a tumour arising from the musculoaponeurotic layer of abdomen, below the level of the umbilicus. It is unencapsulated, hard, fibroma, presently classified under aggressive tibromatosis. 80% of cases occur in women, commonly after deliveries. It is common over old abdominal operation scars (lower abdomen), may be due to old haematomas. Oral contraceptive (OCP) use is more commonly associated with desmoid. It is often associated with the familial polyposis colon (FAP) , osteomas, odontomes epidermal cysts-Gardner's syndrome. Desmoid tumour is 1000 times more common in FAP. It is a slow growing tumour involving muscle and soft tissue of the abdominal wall, locally spreading, often undergoes myxomatous changes. Unlike fibromas, it never turns into sarcoma. It is often classified as superficial and deep. It is also classified as abdominal (common in females); extra-abdominal (common in males, common in back, head, scars, chest wall, neck); and peripheral. Histologically it contains multinucleated plasmodial giant cells.

I Management Fig. 17.16A

The possible association of Gardner's syndrome is looked for and Barium enema, X-ray, USabdomen are done. MRI is useful. Biopsy is done to confirm the diagnosis.

Wide excision of the tumour with a margin of 2.5 cm is done along with placement of a mesh to the abdominal defect. It is moderately radiosensitive. Drugs like sulindac and tamoxifen are also used. Chemotherapeutic agents like doxorubicin, actinomycin D, dacarbazine, carboplatin are used but with significant toxicity. Recurrence rate is high-20-40%.

Vitellointestinal anomaly, diaphragmatic hernia, malrotation can co-exist, exomphalos, macroglossia, gigantism (EMG) can co-exist in 2/3 of such babies-is called as BeckwithWeidemann syndrome. Chromosomal trisom ies-13, 15, 18 and 21 , bladder extrophy, imperforate anus, sacral vertebral anomaly, meningomyelocele. Abdominal wall defect can be confirmed in utero by US. Amniocentesis and chromosomal analysis can be done. This will allow deciding the possible need for termination of pregnancy, or management plan immediately after delivery. Abdominal wall muscle is normally developed but peritoneal layer is hypoplastic.

735

>

er

=0 3

::::, DI

DI

::,

=c: 3

er

n C

c,,

Fig. 17.17: Recurrent abdominal wall tumour. It may be fibromatosis or recurrent dermatofibroma.

EXOMPHALOS (OMPHALOCELE) It is the fai lure of all or a part of the gut to return to the coelomic cavity during early foetal life, as coelomic cavity has not developed properly. Sac covering the content is very thin, consists of three layers-outer amniotic membrane, middle Wharton's jelly and inner peritoneal layer. Sac may get ruptured during birth. Omphalocele is often associated with congenital anomalies of cardiac and genitourinary system-70%.

Fig. 17.19: Exomphalos major. Note the liver, small and large bowel.

Dillerences between exomphalos minor and exomphalos major

Exomphalos major

Fig. 17.18: Exomphalos.

Small sac

Large sac

Defect < 5 cm

Defect > 5 cm

Content is small bowel only

Liver, small and large bowel

Primary closure is possible

Primary closure is not possible

Good prognosis

Poor prognosis

a. Exomphalos minor: Here the sac is small and umbilical cord is attached to the summit, with small bowel as the content. Treatment for exomphalos minor is relatively easier. The sac has to be twisted so as to reduce the content to the peritoneal cavity

Difficulties come not to obstruct, but to instruct.

736

through the umbilical defect and the abdomen is strapped. Later the defect is closed (Defect is 5 cm) is present with contents lying completely outside. Umbilical cord is attached to the inferior aspect of the sac. Contents being small bowel, large bowel and liver. Often the sac ruptures during delivery, which in turn leads to severe infection and high mortality. Here immediate surgery (within hours) is the only hope to save the child.

GASTROSCHISIS (BELLY CLEFT) It is a defect of the anterior abdominal wall just lateral to the umbilicus. It is common in premature babies. It is associated with defect in the involution of 2nd umbilical vein. It is common in mothers younger than 20 years, those who take aspirin, ibuprofen, pseudoephedrine during 1st trimester and who regularly smoke and take alcohol. Umbilicus is normal. The defect is almost always to the right of an intact umbilical cord. Evisceration of the bowel occurs through the defect during intrauterine life. There is no peritoneal sac and the irritating effect of amniotic fluid causes chemical peritonitis with formation of a thick, oedematous membrane. Nonrotation and intestinal atresia are common associations (15%). Cardiac anomaly is not common as in omphalocele. After delivery, these infants are more prone for fluid loss, hypothermia, hypovolaemia, sepsis, metabolic acidosis. Necrotising enterocolitis is also common in such infants (20%). They are also more prone for paralytic ileus.

Liver

E. minor

E. major

Fig. 17.20: Diagram showing differences between exomphalos minor

and exomphalos major. As there is hard ly any properly developed abdominal cavity, it is not possible to reduce the contents to the peritoneal cavity. So initially, relaxing incisions are placed over the lateral abdominal wall and also the subcutaneous layer has to be undermined so as to accommodate, whatever possible contents in the cavity. The contents are not to be forced into the cavity, which in turn may cause intestinal obstruction, respiratory distress, venous engorgement. Often a sterile polythene bag (Silastic silo) can be wrapped over the content carefu lly. Gradual twisting of the bag over the summit at regular intervals over two to three weeks wil l stimulate the peritoneal cavity to increase in capacity and eventually the contents get reduced . Later the defect is closed properly. Antibiotics, IV fluids, nutritional support are required during this period. Mortality is higher in spite of all these-due to sepsis, dehydration, hypothermia and respiratory failure. Late ventral hernia should be corrected at later period.

Vitamin K injection, TPN Sepsis control with antibiotics Evaluation for other anomalies In exomphalos major when sac is intact application of 0.5% mercurochrome with 65% alcohol to promote granulation tissue formation Prevention of aspiration Prevention of hypothermia Wrapping the content with sterile bag/wrapper Correction of dehydration Definitive surgical procedures like release incisions and closure

•· Figs. 17.21A and B: Gastroschisis.

I Treatment

~

Fluid management TPN biotics

R-T aspiration Calorie supplement.

Specific: Later under GA, intestines are pushed into the abdomen through the defect and defect is closed with interrupted nonabsorbable sutures. Often when bowel is not accommodating in the abdominal cavity, bowel is initially placed in sterile silastic silo bag. In later period, it is pushed into the abdomen gradually. Often a part of the bowel may not be viable, then resection and anastomosis has to be done. Exomphalos

Gastroschisis

1. Defect through umbilicus 2. Covered by sac which has three layers 3. Associated with anomalies

1. Defect lateral to umbilicus 2. No peritoneal sac 3. Not associated (except malrotation)

With proper surgery, nutrition, resuscitation, survival rate is 90% which is better than omphalocele. Prolonged postoperative ileus is the common problem in these patients.

B Infected haematoma Umbilical sepsis spreading into the abdominal layers causing the abscess Blood spread from distant focus.

- - - - - - -- -

RECTUS SHEATH HAEMATOMA Rectus abdominis muscle is supplied by superior and inferior epigastric arteries. Injury to one of these vessels will cause bleeding and haematoma in rectus sheath. Commonly it is due to bleeding from inferior epigastric artery in the lower abdomen.

B Trauma; Surgery Spontaneous haematoma, typhoid fever • Blood dyscrasias, haemophila, anticoagulant therapy Severe straining and exercises Tetanus and other convulsions Patients on anticoagulants

737

J

I Features Tender, soft/firm swelling which is well-localised, adherent to skin and abdominal muscles underneath and not mobile. Aspiration will show pus. Should be ruled out from intra-abdominal mass, cold abscess, parietal hernia. Ultrasound and needle aspiration is confirmative. ,:. Treatment: It is antibiotics and drainage under general anaesthesia.

>

go C.

C

3 ::I

al

al ::I C.

c:: 3 go

Puerperium.

n

------------

C

(I)

I Features Common in females. Sudden onset of swelling in lower abdomen, which is tender, warm, firm on one side of the abdomen. Swelling does not cross the midline. Blu ish discoloration over the swelling. US and aspiration confirms the diagnosis. Should be differentiated from other masses and parietal hernias. Coagulation profile is a must. CT scan abdomen is often needed. Treatment ;.. Usually conservative with analgesics and antibiotics. ,. Angiographic embolisation of inferior epigastric artery. Occasionally requires drainage of haematoma and ligation of inferior epigastric artery.

rn

r.J

Figs. 17.23A and B: (A) Abdominal wall abscess; (B) Postoperative

abdominal wall abscess.

MELENEY'S PROGRESSIVE SYNERGISTIC BACTERIAL GANGRENE OF ABDOMINAL WALL ,:. It is due to infection by microaerophilic streptococci, staphylococci and other anaerobes of the postoperative abdominal or thoracic wounds.

ABDOMINAL WALL ABSCESS

Figs. 17.22A and B: (A) Abdominal wall abscess in a child;

(B) Abdominal wall infection with a wound.

Fig. 17.24: Severe abdominal wall sepsis in a postoperative patient.

A deaf husband and a blind wife are always a happy couple.

738

0

co

::i C

co

==

"'

CCII

a: Ct)

It is common in HIV, diabetic and immunosuppressed people. Sudden pain, redness, blackening and gangrene of the skin of the abdomen with abdominal wall necrosis. Toxicity, septicaemia, renal failure can occur. Treatment: Antibiotics like penicillins and metronidazole; Excision of necrotic and gangrenous tissue until it bleeds. Blood transfusion, nutrition supplement. Maintaining adequate urine output. Management of toxaemia, hyperbaric oxygen and critical care. Skin grafting and coverage, when it granulates well.

DIVARICATION OF RECTI (DIASTASIS RECTI) It is thinning of linea alba in midline in epigastrium. Abdominal wall protrudes in midline with prominent divaricated edges of both recti. Transversalis fascia remains intact and hence hernia will not be present; so impulse on coughing will be absent.

Fig. 17.25: Divarication of the recti with visible mass in epigastrium.

Diastasis will become prominent on lifting the head from the bed. It does not require any treatment.

Chapter

Hernia APTER O UTLI NE

• • •

•

• •

Aetiology Parts of Hernia Classification of Hernia Inguinal Hernia • Surgical Anatomy of Inguinal Canal • Classification of Inguinal Hernia Indirect (Oblique) Inguinal Hernia Direct Inguinal Hernia Recurrent Hernia Hernioplasty • Incarcerated Hernia Strangulated Hernia Sliding Groin/Inguinal Hernia

• •

• • • • • • • • •

• •

•

Pantaloon Hernia Femoral Hernia Ventral Hernia lncisional Hernia Umbilical Hernia Paraumbilical Hernia Epigastric Hernia Spigelian Hernia Obturator Hernia Richter's Hernia Lumbar Hernia Sciatic Hernia Complications of Hernia Surgery Parastomal Hernia

Hernia is also defined as an abnormal protrusion of a viscous or a part of a viscous throu gh an opening, artificial or natural with a sac, covering it. Inguinal hernia is the most common hernia (73%) because the muscular anatomy in the inguinal region is weak and also due to the presence of natural weakness like deep ring and cord structures. Indirect is more common than direct. Femoral is 7%; umbilical is 8.5%; others are 1.5% (Excluding incisional hernia) . In general, incisional hernia is next to ingu inal hernia in occurrence-15%. Groin hernia is 25 times more common in men than women. Indirect ingu inal hernia is commonest hernia in men and women. Femoral hernia s more common in females (10:1); umbi lical and incisional hernias are also common in females (2:1). 4

INTRODUCTION Hernia means-'To bud' or 'to protrude', 'off shoot' (Greek) 'rupture' (Latin). A hernia is defined as an area of weakness or disruption of the fibromuscular tissues of the body wall. Often hernia is also defined as an actual anatomical weakness or defect. 75- 85% of abdominal wall hernias are groin hernias. 15% of males and 5% of females will develop groin hernia. Presently all hernias in groin are grouped as groin hernias. But in this chapter discussion of the indirecVdirecVfemoral hernias are given, in detail as it is still practiced and followed in most of the centres and still it is important when surgical technical aspects are considered.

7cL Common hernias: 1. Inguinal 2. lnclslonal 3. Femoral 4. Eplgastric 5. Umbilical

Rare hernias: 6. Obturator 7. Spigelian 8. Lumbar 9. Gluteal

Fig. 18.1: Common and rare sites of hernias.

In order to achieve a radical cure of inguinal hernia it is absolutely essential to restore those conditions in the area of hernial orifice which exist under normal circumstances. -Edoardo Bassini

740

Figs. 18.2A to C: Note the clinical look of indirect inguinal (commonest), direct inguinal and femoral hernias.

Fig. 18.3: Large ventral hernia in the lower abdomen. Fig . 18.5: Epigastric hernia.

AETIOLOGY

Fig. 18.4: lncisional hernia; common in lower abdomen.

Straining. Lifting of heavy weight. Chronic cough (tuberculosis, chronic bronchitis, bronchial asthma, emphysema). Chronic constipation (habitual, rectal stricture). Urinary causes ,.. Old age-BPH, carcinoma prostate. ,.. Young age-stricture urethra. :.- Very young age-phimosis, meatal stenosis. Obesity.

Pregnancy and pelvic anatomy (especially in femoral hernia in females). Smoking. Ascites. Appendicectomy through McBurney's incision may injure the ilioinguinal nerve causing right sided direct inguinal hernia.

direct sac. Body of the sac is thin in infants, children and in indirect sac, but is thick in direct and long-standing hernia.

741

Inferior epigastric artery

n :::c

>

m

:x, Indirect sac

Direct sac

Fig. 18.8: Diagram showing the differences between indirect and direct sacs.

,

Fig. 18.6: Old appendicectomy scar with direct inguinal hernia. It is due to injury to ilioinguinal nerve during appendicectomy.

An indirect inguinal hernia occurs in a congenital, preformed sac, i.e. the remains of processus vaginalis. Chances of presence of bilateral preformed sac is 60%. Familial collagen disorder- Prune Belly syndrome. Acquired herniation is also probably due to collagen deficiency called as metastatic emphysema of Read.

PARTS OF HERNIA Hernia comprises of: Covering; Sac; Content.

Neck

-

-1------

- - - -- - -- ---+-

Body

Inferior epigastric artery

Fundus - t - - - - - ;~~,

Fig. 18.7: Parts of hernia. Note the fundus, body and neck of the hernia.

Sac is a diverticulum of peritoneum with mouth, neck, body and fund us. Neck is narrow in indirect sac but wide in

Hernia without neck: Those hernias with larger mouth lack neck, e.g. direct hernia, incisional hernia. , Hernia without sac: Epigastric hernia-it is protrusion of extraperitoneal pad of fat. Coverings of the sac are the layers of the abdominal wall through which the sac passes. Contents of Sac , 0mentum-Omentocele (Epiplocele). Difficult to reduce the sac later, initially it can be reduced easily. , lntestine-Enterocele-commonly small bowel, but sometimes even large bowel. , Difficult to reduce the sac initially. , Richter's hernia: A portion of circumference of bowel is the content. , Urinary bladder may be the content or part of the posterior wall of the sac-cystocete. , Ovary, often with fallopian tube. , Meckel's diverticulum-Littre's hernia. , Appendix in inguinal hernial sac which is often incarcerated-Amyand's hernia. , Fluid: Fluid is secreted from congested bowel or omentum. It may be an infected fluid or ascitic fluid or blood from the strangulated sac. Note: • The Groin even though anatomically not a well-defined area, it includes inguinal and femoral regions; both are separated by medial part of the inguinal ligament. Clinically when hip is completely flexed inguinal and femoral regions touch each other and that area is recognised as groin. Sebaceous cyst, lipoma, neurofibroma are swellings arising from integuments; inguinal and femoral hernias; lymphadenopathy; saphena varix and aneurysms are vascular swellings; ectopic testis, lipoma or hydrocele of the cord, hydrocele of the canal of Nuck (in females) are swellings related to inguinal canal; psoas abscess which is deeper- are groin swellings. Common

groin swellings are inguinal and femoral hernias, inguinal lymphadenopathy and saphena varix. Groin pain may be due to hernia, funiculitis, varicocele, lymph node diseases or post surgery pain. • Hemiography {by Gu/Imo): Injection of contrast into the peritoneal cavity and taking films in supine and prone positions to diagnose

Anything the mind of man can conceive and believe, it can achieve.

00

742

position", in which the patient is supine with knees bent and heels together. It is probably due to torn external oblique aponeurosis or conjoint tendon or transversalis fascia or internal oblique muscle or entrapment of ilioinguinal nerve. MRI may be useful. Sleeping in a prone position with the hip on theaffected side flexed and externally rotated can be a cure in some individuals. Many eventually require a definitive treatment consisting of surgical repair followed by a structured rehabilitation.

CLASSIFICATION OF HERNIA

I Classification I (Clinical) 1. Reducible Hernia Figs. 18.9A and B: Hernial sac containing small bowel (enterocele) and omentum (omentocele) in two different patients.

Hernia gets reduced on its own or by the patient or by the su rgeon. Intestine reduces with gurgling sound and it is difficult to reduce the first portion. Omentum is doughy, and it is difficult to reduce the last portion. Expansile impulse on coughing present.

2. Irreducible Hernia Here contents cannot be returned to the abdomen due to narrow neck, adhesions, overcrowding. Irreducibility predisposes to strangulation.

3. Obstructed Hernia It is an irreducible hernia with obstruction, but blood supply to the bowel is not interfered. It eventually leads to strangulation.

Fig. 18.10: Amyand hernia-is appendix in inguinal hernial sac. Ileum

Fig. 18.12: Inguinal hernia presenting with featu res of intestinal obstruction.

Fig. 18.11: Littre's hernia with Meckel's diverticulum as content. small protrusions of peritoneal sac is called as herniography. It was earlier also used for diagnosing undescended testis. • Gilmore's groin or hockey groin or Athletic pubalgia or hockey/ sports hernia, or groin disruption is a condition of the pubic joint in athletes; presents with chronic groin pain and a dilated superficial inguinal ring. Football and ice hockey players are most frequently affected. Present with pain during movements of sports, particularly hip extension, twisting and turning which radiates to the adductor region and even the testicles. Symptoms can often be reproduced by performing sit-ups or crunches or with the patient in "frog

Note: • Garrey's stricture: Constriction that occurs due to ischaemic narrowing of small bowel which has reduced from an obstructed hernia.

4. Inflamed Hernia It is due to inflammation of the contents of the sac, e.g . appendicitis, salpingitis. Here hernia is tender but not tense; overlying skin is red and oedematous.

5. Strangulated Hernia It is an irreducible hernia with obstruction to blood flow. The swelling is tense, tender, with no impulse on coughing and with features of intestinal obstruction.

I Classification Ill: According to the Contents Omentocele-omentum. Enterocele-intestine. Cystocele- urinary bladder. Littre's hernia-Meckel's diverticulum. Note: Littre described Meckel's diverticulum in a hernial sac 81 years before Meckel was born. Maydl's hernia. Sliding hernia. Richter's hernia-part of the bowel wall.

I Classification IV: Based on Sites Inguinal hernia Femoral hernia Obtu rator hernia Diaphragmatic hernia Fig. 18.13: Garrey's stricture on table. Due to ischaemia, a stricture is formed in the bowel at the constriction ring after reduction.

Features of intestinal obstruction may be absent in case of omentocele, Richter's hernia, Littre's hernia. Strangulation is the most serious complication of hernia; most common strangulated hernia is indirect inguinal hernia. Highest rate of strangulation is seen in femoral hernia (ratio of strangulated femoral hernia to total number of femoral hernia).

6. Occult (Inguinal) Hernia Hernia swelling is clinically not detectable but presents with groin pain; there may not be any expansile impulse on coughing.

I Classification II Congenital-Common It occurs in a preformed sac/defect. Clinically may present at a later period due to any of the precipitating causes like in indirect inguinal hernia.

Acquired j 11 is secondary to any causes which raise the intra-abdominal pres~ ure leading into weakening of the area like in direct inguinal hernia.

Lumbar hernia Spigelian hernia Umbilical hernia Epigastric hernia

INGUINAL HERNIA

I SURGICAL ANATOMY OF INGUINAL CANAL Superficial inguinal ring is a triangular opening in the external oblique aponeurosis and is 1.25 cm above the pubic tubercle. The ring is bounded by a superomedial and inferolateral crus. Normally, the ring does not or just admit the tip of little finger. Deep inguinal ring is a U-shaped condensation of the transversalis fascia, lies 1.25 cm above the inguinal ligament midway between the symphysis pubis and the anterosuperior iliac spine. Inguinal (Poupart's) ligament It is formed by the lower border of the external oblique aponeurosis which is thickened and folded backwards on itself, extending from anterosuperior iliac spine to pubic tubercle. Inguinal canal: It is an oblique passage in lower part of abdominal wall, 4 cm long, situated above the medial ½ of inguinal ligament, extending from deep inguinal ring to superficial inguinal ring. In infants both superficial and deep rings are superimposed without any obliquity of the inguinal canal. Inguinal canal in female is called as 'canal of Nuck'. CONTENTS OF INGUINAL CANAL

Spermatic cord in males Round ligament in females

Fig. 18.14: Inguinal hernia on right side in a child. It needs only herniotomy.

_J

llioinguinal nerve

Vas deferens Artery to vas Testicular and cremasteric artery Genital branch of genitofemoral nerve Pampiniform plexus of veins Remains of processus vaginalis Sympathetic plexus around the artery to vas

LJ

When spermatic cord is rolled transversely beneath the gentle pressure of index finger. Thickening of the cord denotes presence of a hernia. - William E Ladd

743

C")

:::c

>

""ti

m

::r,

..... 00

744

Internal oblique muscle

Internal spermatic fascia from fascia transversalis. Cremasteric fascia. External spermatic fascia from external oblique aponeurosis is seen below the external ring in the scrotum. Pampiniform plexus of veins

llioinguinal nerve

I

lliohypogastric nerve

Inferior epigastric ----:i~---3;a.,ii vessels

llioinguinal nerve

Inguinal ligament

co

:::s

:::i:

Artery to cremaster

en

a:, a: Cl)

Cremasteric fascia with muscle

Genital branch of genitofemoral nerve

Artery of ductus deferens

Lymph vessels Vas deferens

Fig. 18.15: Structures related to cord in the inguinal canal.

Superficial - -.\-- - - -___:_:~ ~ = ~ ;i,-,. inguinal ring Pubic tubercle

Fig. 18.16: Surgical anatomy of inguinal canal.

I Boundaries In front External oblique aponeurosis and conjoined muscle laterally. Behind: Inferior epigastric artery, fascia transversalis and conjoined tendon medially. Above: Conjoined muscle (Arched fibres of internal oblique). Below. Inguinal ligament.

B Obliquity of inguinal canal Arching of conjoint tendon 'Shutter mechanism' of internal oblique ' Ball valve mechanism' due to contraction of cremaster muscle which plugs to superficial ring When external oblique muscle contracts, intercrural fibres of superficial ring appose causing 'slit valve mechanism' Hormones

Figs. 18.17A to C: Nerves in inguinal canal: 1/iohypogastric nerve (T12, L1) runs between transversus abdominis and internal oblique divides into lateral and anterior branches; anterior branch pierces internal oblique 2 cm medial to anterosuperior iliac spine and later pierces the external oblique 3 cm above the superficial inguinal ring to supply abdominal skin above the pubis. 1/ioinguinal nerve (L 1) pierces the transversus abdominis near anterosuperior iliac spine, pierces the internal oblique just above the internal ring, and enters the inguinal canal within cremasteric fascia outside the cord supplies medial thigh, base of penis and proximal scrotum. Genital branch of genitofemoral nerve (L 1, L2) enters the cord through internal ring supplying the cremaster and scrotum. FRUCHAUD 'S MYOPECTINEAL ORIFICE

It is an osseo-myo-aponeurotic tunnel. It is through this tunnel all groin hernias occur. It is bounded: Medially by lateral border of rectus sheath. Above by the arched fibres of internal oblique and transversus abdominis muscle. Laterally by the iliopsoas muscle. Below by the pectin pubis and fascia covering it.

rectus muscle, laterally by inferior epigastric artery, below by inguinal ligament). Sac is medial to the inferior epigastric artery.

Rectus muscle

Anterosuperior iliac spine

,, Direct----.'""""-..,------hernia site

-----+--

Supravesical external hernia site

~ 1-?C,.....-L_ Internal ring (Cord )

Indirect

Femo ral vessels

Femoral hernia site Myopectineal orifice (MPO) (Fruchaud's)

Inguinal__,,___.__ ligament

__c

Femoral ~ hernia

:::c

...

/

CD

::I Col

Fig. 18.18: Fruchaud's myopectineal orifice is bound-by lateral border of rectus, iliopsoas, conjoint tendon, pectin pubis.

Pubic tubercle

Fig. 18.20: Location of direct and indirect inguinal hernia and femoral hernia.

Classification According to the Extent Incomplete: , Bubonocele: Here sac is confined to the inguinal canal. , Funicular: Here sac crosses the superficial inguinal ring, but does not reach the bottom of the scrotum. Complete: Here sac descends to the bottom of the scrotum. Saddle-bag or pantaloon hernial sac has got both medial and lateral component. Fig. 18.19: Laparoscopic view of groin anatomy with hernia.

I CLASSIFICATION OF INGUINAL HERNIA (EARLIER)

Note:

Inguinal hernia is above and medial to the pubic tubercle. Femoral hernia is below and lateral to pubic tubercle.

Anatomical Classification (in Inguinal Hernia) Indirect hernia It comes out through internal ring along with the cord. It is lateral to the inferior epigastric artery. Direct hernia It occurs through the posterior wall of the inguinal canal through 'Hesselbach 's triangle' (bounded medially by lateral border of

NEWER CLASSIFICATIONS OF INGUINIAL HERNIA I. Gilbert Classification Type t. Hernia has got snug internal ring through which a peritoneal sac passes out as indirect sac. Type It. Hernia has a moderately enlarged internal ring which admits one finger but lesser than two finger breadth. Once reduced it s during coughing or straining.

I

Contd...

Figs. 18.21A to C: {A) Bubonocele; (8) Irreducible left side inguinal hernia. It is above the upper part of the testis (funicular). Taxis is the method used to reduce it; (C) Bilateral complete inguinal hernia. Here hernia descends up to the bottom of the scrotum.

Great minds have purposes, others have wishes.

746

Contd... Type Ill: Hernia has got large internal ring with defect more than two finger breadth. Hernia descends into the scrotum or with sliding hernia. Once reduced it immediately protrudes out without any straining. Type IV: It is direct hernia with large full blow out of the posterior wall of the inguinal canal. The internal ring is intact. Type V: It is a direct hernia protruding out through punched out hole/ defect in the transversalis fascia. The internal ring is intact. Type VI: Pantaloon/double hernia. Type VII: Femoral hernia. Type VI and VII are Robbin's modifications.

Smoking; Obesity Respiratory causes like bronchial asthma, tuberculosis, bronchitis I Ascites Previous surgery like appendicectomy which can cause direct inguinal hernia Chronic constipation due to anorectal strictures. Rectal stricture may be due to chronic proctitis (amoebic), tubercul osis of anorectum, previous anorectal surgery, rectal carcinoma or stricture due to lymphogranuloma venereum Urinary problems like benign prostatic hyperplasia (BPH), urethral stricture Straining; Multiple pregnancies

II. NYHUS Classification Type I: Indirect hernia with normal deep ring. Type II: Indirect hernia with dilated deep ring. Type Ill: Posterior wall defect. a. Direct. b. Pantaloon hernia. c. Femoral hernia. Type IV: Recurrent hernia.

Ill. BENDAVID Classification [Type, Staging, Diameter (TSO) Classification) Type I: Anterolateral defect (indirect). Type II: Anteromedial (direct). Type Ill: Posteromedial (femoral). Type IV: Posterior prevascular hernia. Type V: Anteroposterior defect: lnguino-femoral hernia. European Hernia Society (EHS) classification (Aachen) P = primary hernia. R = recurrent hernia. o = no hernia detectable. 1 = c.,

752

repair is done. (Different mesh procedures are discussed at a later part of this chapter). Note: Other methods of mesh repair are:

c,:, :::I C:

c,:,

::!::

en

CCI

a: Cl)

• Nyhus pre-peritoneal mesh repair (sublay). • Gilbert mesh repair (patch and plug). Gilbert's PHS (Prolene Hernia System) repair (onlay and sublay). • Stoppa's (Rene SStoppa) giant prosthesis reinforcement of visceral sac (GPRVS- sublay). • Kugel (Rober DKugel) groin hernia mesh repair (sublay).

Tissue repairs are: • Modified Bassini's Herniorrhaphy Conjoined tendon and inguinal ligament are approximated using interrupted nonabsorbable monofilament sutures (polypropylene); medial most stitch is taken from the periosteum of pubic tubercle (called as key or Bassini's stitch); external oblique is closed and other layers are closed. Bassini originally used silk suture for the repair (1887). He also used triple layer of upper leaf which contains transversalis fascia, transversus abdominis and internal oblique muscles in his repair. Care should be taken not to injure iliac vessels (mainly vein) while taking bite from inguinal ligament. It is often practiced to take bites in inguinal ligament at different levels to prevent tearing of the fibres of inguinal ligament. Laterally repair is done beyond the line of internal ring. Few advocate horizontal mattress sutures starting from conjoined tendon to inguinal ligament and from inguinal ligament to conjoined tendon.

Internal ring Conjoint tendon Inguinal ligament External oblique ~ aponeurosis

d

Sutures - + - - -~ (interrupted) Testes

Fig . 18.40: Modified Bassini's repair: It is approximation of inguinal ligament to conjoined tendon using interrupted non-absorbablesutures.

• Desarda no mesh tension free tissue repair(Professor Mohan Desarda, Pune, India): It is live external oblique tissue flap reconstruction of the posterior wal l of the inguinal canal. He proposed that strength of the inguinal canal depends on the aponeurotic extensions from the transverse abdominis aponeurotic arch normally which is physiologically dynamic and strong and his repair supports this effectively with least complications and recurrence. • Shouldice repair: Even though transversalis fascia is thin, it is a tough layer and so double breasting of this fascia using continuous sutures (with non-absorbable material) strengthens the posterior wall of the inguinal canal. It is a multilayered repair. Continuous sutures provide even

distribution of tension throughout the repair. It was originated at Shouldice hernia clinic in Toronto by Shouldice (EE Shouldice 1930) where it was usually done under local anaesthesia. Often cremasteric resection is done in Shouldice in order to have proper revelation of the posterior inguinal wall. Cremasteric vessels (located at lateral part of the cord along with genital branch of genitofemoral nerve) need ligation during cremasterectomy (it causes hanging testis/clapper in bel1. After doing herniotomy as in any other inguinal hernia, transversalis fascia is incised along the line of the wound from deep ring to pubic tubercle. Upper medial flap is elevated without elevating lower lateral flap. First suture line-Lower latera l flap of fascia is sutured to posterior deep part of the elevated upper flap using continuous sutures from pubic tubercle to internal ring which is tied at deep ring without cutting/ending . Second suture line-Using same suture which is not cut, free margin of upper flap is sutured to the shelving edge of the inguinal ligament from deep ring towards pubic tubercle. It causes double-breasting of the transversalis fascia. Knot is placed at the end over the pubic tubercle. Fist and second line sutures are done using a single suture material. Third suture line-The conjoined tendon and anterior to the shelving part of the inguinal ligament is sutured from internal ring to pubic tubercle continuously and tied without cutting. Fourth suture line-Same uncut suture is again sutured back continuously between conjoined tendon and anterior fibres of the inguinal ligament to reach internal ring. External oblique aponeurosis is sutured in two layers (5th and 6th suture lines; double-breasting) in front of the cord in original Shouldice repair. Hence, this original Shouldice repair is 6-layered procedure. Suture material used here is 34 gauge fine steel wire (in original Shouldice repair) or polypropylene or polyethylene. Recurrence rate in Shouldice repair is 1%. Berliner modified Shouldice repair-It involves double-breasting of the transversalis fascia like in Shouldice repair and single layer closure of the external oblique aponeurosis without any additional two-layered repair of conjoined tendon to inguinal ligament. • Lytle ·s repair (William James Lytle): Often internal ring is narrowed by placing interrupted sutures over the medial side of the ring to the transversalis fascia using either silk or polypropylene (to narrow the ring and push the cord laterally). Lytle's repair

Internal ring

Testes

Fig. 18.41 : Lytle's repair is narrowing of the widened internal ring.

• Halsted (USA)-Tanner (London) slide operation: To reduce the tension in the repair area, relaxing incision is placed over the lower rectus sheath so that conjoined tendon is allowed to slide downward. • Abrahamson nylon darning-. Continuous intervening network of non-absorbable sutures are placed between conjoined tendon and inguinal ligament to give good support to posterior wall inguinal wall. • Koontz 'operation (Koontz AR, New York, 1963): In old people after taking consent, orchidectomy is done along with removal of full cord, testis and total closure of posterior inguinal wall by repair so as to reduce the recurrence.

•

Tanner's slide

I~ Bassini's repair

•

External oblique

Fig. 18.42: Tanner slide operation.

•

Darning repair

•

External- - 4 - - -.,.___ oblique Conjoint - - - - - - ' tendon

Fig . 18.43: Darning in inguinal hernia- Abrahamson's nylon

darning. • Removal of cord at inguinal region (Hamilton Bailey): Cord is removed from the inguinal canal by ligating both at external and internal ring . But testis is retained [for psychological reason) and closure of inguinal canal by repair is done. • McVay operation, 1940 (Cooper's ligament repair): It is repair by placing interrupted sutures between edge of the transversus abdominis to Copper's ligament starting from pubic tubercle medially towards femoral sheath and later continued as suture repair between transversus abdominis

and iliopubic tract laterally up to the entrance of cord. It is a pure tissue repair. It requires relaxing vertical/curvilinear oblique incision at the lateral border of the anterior rectus sheath from pubic tubercle to a point superiorly for 4 cm. It covers all three groin defects (Myopectineal orifice)-indirect, direct and femoral. Nyhus (original) iliopubic repair. Through suprainguinal approach, a transverse incision is made two fingerbreadths above the pubic symphysis to expose the rectus sheath. Left index finger is passed into the external ring; level of internal ring is confirmed. Anterior rectus sheath is incised horizontally above the level of the deep ring; lateral part of lower end of the rectus is retracted. Posterior rectus sheath is opened. External oblique, internal oblique and transversus abdominis muscles are cut along the length of the incision. Transversalis fascia is cut to reach the preperitoneal space. If it is direct sac, it is dissected without opening and isolated. Sac is inverted using absorbable purse string suture. If it is indirect sac, it is dissected proximally high up, opened and ligated. Transaponeurotic arch (transverse abdominis muscle and transversalis fascia) is sutu red below to Cooper's ligament and iliopubic tract. Nyhus mesh repair is done similarly through this approach by placing mesh and suturing to Cooper's ligament. Andrew's operation: It is overlapping of the external oblique aponeurosis. Condon procedure: It is anterior iliopubic tract repair by suturing the transversus abdominis to iliopubic tract from pubic tubercle to deep ring; then extending lateral to the cord. In direct sac relaxing incision is made on the lower rectus before tying all placed sutures. Wilkinson method: Transversus abdominis and internal oblique are sutured to inguinal ligament with continuous monofilament sutures- 1st layer; lower free edge of the external oblique is passed above, behind the cord and is sutured to internal oblique surface-2nd layer; upper free edge of the external oblique is brought down in front of the cord and is sutured to lower visible surface of the external o0blique-3rd layer. It has less recurrence rate.

Complications of open hernia repair are: Haemorrhage, haematoma, seroma, haematocele. Infection, osteitis pubis. Post tierniorrhaphy hydrocele, lymhocele. Hyperaesthesia over the medial side of inguinal canal due to injury to iliohypogastric nerve-neuralgia (15%). Recurrence-10-15% in modified Bassini; 1-5% only in other types. Injury to urinary bladder, bowel, ileus. Testicular atrophy due to thrombosis of pampiniform plexus of veins, penile oedema-rare.

Language is the dress of thought.

753

::c

....

CD

::I

iii'

754

c=Q)

...:::s

en

v., 0

:::s

C:

:!:: _v,

CCI

a: v.,

Fig . 18.44: Left sided inguinal hernia repair wound is infected; right side is clean.

Around 50 ml of xylocaine 0.5% is used. Plain xylocaine 0.5% or xylocaine 0.5% with adrenaline can be used. Plain xylocaine- dose is 2 mg/kg body weight. Xylocaine with adrenaline-is 7 mg/kg body weight. Two methods are used: 1. Nerve block method (Point block): 1Oml of xylocaine is infiltrated 2 cm above and medial to anterior superior iliac spine to block the iliohypogastric nerve. Midinguinal point is infiltrated with 10 ml xylocaine. Pubic tubercle place is infiltrated with 10 ml xylocaine. 10 ml of xylocaine is infiltrated just below the inguinal ligament lateral to femoral artery to block the genital branch of genitofemoral artery. Line of skin incision is infiltrated with 10 ml of xylocaine. Later neck of the hernial sac is infiltrated with 1Oml of xylocaine. 2. Field block method (Shouldice method): Skin of around 4 cm wide area is infiltrated including the subcutaneous plane as first layer from anterior superior iliac spine to pubic symphysis. Skin, subcutaneous and two layers of superficial fascia (Camper and Scarpa's) are incised. Area deep to external oblique aponeurosis is infiltrated with 1Oml of xylocaine. External oblique aponeurosis is incised. Exposed inguinal canal and hernial sac is infiltrated with 1Oml of xylocaine to continue with the dissection.

B Taxis: Patient is placed in supine position with hip and knee flexed and hip internally rotated. Contents are pushed with one hand directing with other hand Use of Truss: Rat-tailed sprung truss is used. Measurement is taken from the tip of greater trochanter to third piece of sacrum circumferentially - Complications are discomfort, ulceration, strangulation, inflammation, testicular atrophy, ilioinguinal neuritis, femoral neuritis - It may be used in elderly people, who are not fit for anaesthesia and surgery - Conservative treatment should be avoided in hernia as much as possible - Truss is absolutely contraindicated in femoral and sliding hernia - Truss increases the surgical bleeding, postoperative oedema, testicular pain

Figs. 18.45A and B: Hernia truss. It is used only when patient is not fit for surgery. It may precipitate strangulation. Before placing truss, contents of the hernia should be reduced completely.

I DIRECT INGUINAL HERNIA 10-15% of the hernias are direct. 50% of direct hernias occur bilateral. 35% of inguinal hernias are direct. It is uncommon in females and children. It is always acquired, due to weakening of posterior wall of inguinal canal. Hernia is medial to the inferior epigastric artery with wide neck. Sac is thick and often the medial wall or content may be bladder. Direct hernia occurs through Hesselbach's triangle which is bounded by inferior epigastric artery laterally, lateral border of rectus medially, inguinal ligament below. It is divided into medial and lateral halves by obliterated umbilical artery (medial umbilical ligament). So, direct hernia is classified as medial or lateral depending on which part of the Hesselbach's triangle, it is arising from.

Fig. 18.46: Surgical anatomy of direct inguinal hernia.

Anterior superior ilia c spine

Inferior epigastric artery

I

Rectus muscle

Malgaigne bu/gings are often seen in these patients on examination, more often than in ind irect hernia. They are protrusion of abdominal wall muscle during leg raising test as weak, soft, supple, swellings which signifies poor abdominal muscle tone. It is common in old age, obesity; it indicates that hernia requires mesh repair. Direct hernia rarely descends into the scrotum and strangulation is not as common as in indirect hernia. But in longstanding cases, it can descend down to the scrotum and strangulation can occur.

I Treatment Fig. 18.47: Surgical anatomy of Hesselbach's triangle.

Chronic cough, smoking Straining Heavy work

Constipation Previous appendicectomy

Surgery

Usually direct sac is not opened. Care should be taken on the medial aspect due to the presence of bladder (bladder should be emptied before surgery). •·· Ideally herniop/asty (mesh repair) is done. In case of bilateral hernia, mesh repair can be done on both sides together. Laparoscopic approach (TEP) or suprapubic approach may be better in bilateral cases.

B It is also called as Ogi/vie's hernia. It is a type of direct hernia which is prone for strangulation. It is a narrow necked hernia with prevesical fat and a portion of bladder, and or intestine that herniates through a small defect in the medial part of the conjoined tendon just above the pubic tubercle. It occurs in elderly males.

I

--------------'

Fig. 18.48: Bilateral direct hernia. Note the medial location of the direct hernias.

Hernia reaches below the mid-level of thigh when patient stands.

Figs. 18.50A and B: Giant indirect and direct inguinal hernias. (Courtesy: Professor Ramlingam, KIMS, Narkatpally, Telangana, India).

I RECURRENT HERNIA {Inguinal) Fig. 18.49: Large bilateral direct hernia. Occasionally direct hernia descends down and becomes completeand then may cause obstruction. Descent is not as common as indirect hernia.

Incidence is 10%; If recurrence is within 3 years it is called as early, if after 3 years it is late.

Femoral hernia is never congenital.

755

("')

::c:

>

m ::c

..... 0)

756

Old scar of hernia surgery

!

Old scar of hernia surgery

l

..

Recurrent hernia

Fig. 18.53: Large recurrent inguinal hernia.

I Clinical Features Fig. 18.51: Recurrent inguinal hernia-left sided.

Note the scar earlier surgery.

Same as for inguinal hernia. Defect is usually narrow and so more likely to go in for strangulation. It can be medial recurrence or lateral recurrence depending on the location of the sac. Medial recurrence is common as tension in suture line is greatest near the pubic tubercle.

I Treatment After thorough investigations, the cause of recurrence has to be treated and later hernioplasty is done. Laparoscopic (TEP/TAPP) approach is better for recurrent hernia. In open repair, preperitoneal mesh repair is ideal. In elderly people, Koontz orchidectomy or cord excision at inguinal canal may be added after proper prior consent. Fig. 18.52: Recurrent inguinal hernia is 10% common. It is

commonly medial recurrence. It is treated by mesh repair.

B

PREDISPOSING FACTORS

Preoperative

Smoking; Chronic cough; Constipation Old age; Anaemia; Hypoproteinaemia; Straining Increased intra-abdominal pressure of any cause (BPH, carcinoma prostate, stricture urethra); Ascites Operative

Tension in the sutures Weak anterior abdominal wall Postoperative Infection (50%); Haematoma formation during earlier surgery

Retained sac in pantaloon hernia; Straining

l..!!!:Eurrence Rate Bassini's repair-10%; Shouldice repair-1 %. Hernioplasty-1 to 3%; Other methods-1 to 5%.

True or false recurrences are the types of recu rrence. If hernia occurs in inguinal region after inguinal hernia repair it is called as true recurrence. If other groin hernia occurs after inguinal hernia repair like femoral hernia or obturator or other rare types, it is called as false recurrence. But presently hernia is classified grossly as groin hernias and so all recurrences are true recurrences.

1% common. Causes: Infection, earlier mesh extrusion, failure of treating the

precipitating causes. Treatment The cause is treated and mesh repair is done; GPRVS (Giant prosthesis reinforcement of the visceral sac) or TEP through laparoscopy is ideal.

I HERNIOPLASTY It is strengthening of posterior inguinal wall in case of indirect hern ia or in any large hernia with weak abdominal wall using a supportive material. This allows and supports good fibroblast proliferation which in turn strengthens the weak posterior wall of inguinal canal or abdominal wall.

B

MATERIAL USED

Synthetic: Prolene mesh (white in colour) Dacron mesh, Morlex mesh, Mersilene sheath. Biological:Tensor fascia lata, temporal fascia and skin. (presently biological materials are not well-accepted as infection is common and its efficacy is not proved). Prolene mesh is commonly used at present. Non-absorbable interspersed absorbed mesh (Vipro/Ultrapro) are also used nowadays.

Indications: Direct hernia; Recurrent hernia; Re-recurrent hernia; lncisional hernia; Old age; Hernia with weak abdominal muscle tone; Sliding hernia.

Complications: Infection, Mesh extrusion, Foreign body reaction; Mesh inguinodynia causing hyperaesthesia and pain along the distribution of ilioinguinal or iliohypogastric nerves; Mesh erosion into bladder, bowel or vessels can occur occasionally (rare).

•

•

Fig. 18.54: Infected mesh after open hernioplasty; it needs removal of the mesh.

•·· Principle: ,. Size of the mesh should be bigger than the size of the defect; ,. Mesh should be fixed above and below to the conjoint tendon and inguinal ligament or abdominal wall using interrupted, non-absorbable sutures; ,. Absolute haemostasis and control (prevention) of infection is important. Hernioplasty is becoming the prime treatment for inguinal hernia.

•

•

the iliopectineal ligament of Cooper and sutured to it using two or three interrupted non-absorbable sutures. It is sutured to transverse abdominis above and transversalis fascia from deep. Modified Rives preperitoneal mesh repair (Read-Rives) is preperitoneal mesh repair through transinguinal approach. Direct sac, is inverted with sutures. For indirect sac, a high ligation is done. Here mesh is placed in preperitoneal space, folded and sutured below to iliopectineal ligament, above to the transverse abdominis in deeper plane. Often transversalis fascia opened earlier is sutured back using nonabsorbable suture material in front of the placed mesh. Stoppa's giant prosthesis reinforcement of visceral sac (GPRVS): It is done in large hernias, hernias in elderly, bilateral hernias, recurrent and re-recurrent hernias, hernia with very lax abdomen, and hernia with collagen diseases like Marian's syndrome. Horizontal length (size) of the mesh is 2 cm less than distance between two anterosuperior iliac spines and vertical length (size) is distance between the umbilicus and pubic symphysis. Large mesh is placed between peritoneum and lateral, inferior, anterior abdominal wall which stretches in the lower abdomen and pelvis. It is done through lower midline or Pfannenstiel incision. Usually, such large mesh is placed without any anchorage. Gilbert mesh repair (patch and plug): After herniotomy, internal ring is plugged by cone-shaped (umbrella plug) piece of prolene mesh. Later on lay/inlay mesh repair of posterior wall of the inguinal canal is done. Gilbert's PHS (Prolene Hernia System) repair (onlay and sublaysandwich technique): It is an open transinguinal approach to place a specially devised mesh in both preperitoneal as inlay and in front as onlay mesh repair. This mesh has got a rounded deeper part which is placed in preperitoneal space and a modified quadrangular part which is placed as onlay. Both parts are connected through a connecting stiff rounded part in between which steadies the mesh in place preventing its displacement.

B • Onlay repair • Lichtenstein mesh repair-a type of onlay Inlay repair- mesh placed at myopectineal level-bridging repair • Underlay repair-mesh placed at deeper plane usually in preperitoneal space Gilbert patch and plug repair/Gilbert's PHS repair (onlay + sublay) • Nyhus preperitoneal mesh repair • Kugel groin hernia mesh repair Modified Ri ves preperitoneal mesh repair through inguinal approach Stoppa GPRVS repair • TEP mesh repair • TAPP mesh repair Note: • Onlay mesh repair by placing mesh in front. It is sutured above to conjoint tendon and below to inguinal ligament using monofilament non absorbable suture material. • Lichtenstein tension free onlay mesh repair (1993) where the cord is encircled with mesh which is often done under local anaesthesia. Suturing of mesh is done similar to onlay mesh repair. It has got less recurrence rate. • Nyhus preperitoneal mesh repair: It is done through suprainguinahlorizontal incision above the pubic symphysis and internal ring. Preperitoneum is approached through lateral border of the lower part of rectus muscle by making an opening in the posterior rectus sheath. Mesh is placed in the preperitoneal space deep to the cord, conjoint tendon, and transversalis fascia. Below, it is folded deep to

Fig . 18.55: Gibert's PHS mesh.

• Kugel (Rober D Kugel) groin hernia mesh repair (sublay): It is done through posterior preperitoneal approach with tension free less invasive sutureless repair. Kugel patch contains two overlapping layers of knitted polypropylene mesh which are attached to each other ultrasonically. Near the outer edge of the mesh it has got a

To attain knowledge, add things every day. To attain wisdom, remove things every day.-Lao Tzu

757

c.-,

:c

>

""C -4

m

:l:I .J.

0C)

::z: (D

.... :::,

Q)

758

c:-

...

Q)

en

::I

Cl)

0

ca

::I

C:

ca

:I!:: _V>

= a:

Cl)

polyester spring which stiffens the mesh to unfold. 1 cm of mesh extends outer to the spring which also has radial slits to have easy maneuverability. Anterior layer of the mesh has got single transverse slit to facilitate the insertion. Multiple 3 mm holes through both layers of the mesh are present that allow tissue contact in better way to prevent mesh displacement. Small V shaped triangular cuts on the anterior layer adjacent to these multiple holes act as sutureless anchors. Incision is above the deep ring and lateral to inferior epigastric vessels. Skin, subcutaneous tissue, external oblique aponeurosis is cut; internal oblique and transversus abdominis muscles are split to reach deeper space. Transversalis fascia split vertically to avoid injury to inferior epigastric vessels which are retracted medially. Preperitoneal space is created; indirect sac is dissected off the deep ring from above; proximal part being ligated using absorbable 3 zero vicryl. Direct sac is dissected off the defect without opening; its pseudosac which is modified transversalis fascia is dissected off entirely fro m the peritoneum behind. Kugel's mesh patch is placed properly without allowing any tissue bands folding it. Transversalis fascia is sutured with one absorbable suture. Transversus abdominis and internal oblique muscles are not sutured. External oblique aponeurosis is sutured.

Transabdominal preperitoneal /aparoscopic mesh repair (TAPP repair): It is mainly used in irreducible and large hernias.

Totally extraperitonea/ laparoscopic mesh repair (TEP)becoming popular.

Transabdominal Preperitoneal Mesh Repair Using Laparoscope This is used in large indirect hernia or irreducible inguinal hernia. 1O mm umbilical port is used for laparoscope. 5 mm ports are placed one on each side on pararectal point at the or above the level of umbilicus so as to achieve adequate triangulation. Contents of the hernia are reduced. Hernial sac is dissected in preperitoneal plane after making horizontal incision at the upper part of the sac opening. Vas, gonadal vessels, pubic bone, inferior epigastric vessels are identified. Once sac is dissected and excised , a prolene/vipro/ultrapro mesh of 15 x 1Ocm sized or smaller is placed in pre peritoneal space. It is fixed to pubic bone using tacks. Peritoneum is closed with continuous prolene sutures.

Fig. 18.56: View of indirect hernia laparoscopically during TAPP.

Fig. 18.57: Mesh placement in TAPP procedure.

Totally Extraperitoneal Repair Using Laparoscope This technique is gaining more popularity than TAPP. Through subu mbilical incision (1 0 mm) extraperitoneal space is reached. After CO 2 insufflation, another 5 mm port is inserted 4 cm below the first port in the midline. Third 5 mm port is inserted in the same line 4 cm below or in the right iliac fossa. Dissection is carried out downwards carefully, then medially up to the pubic tubercle, iliopectineal ligament, laterally to iliac vessels, and inferior epigastric vessels. Once adequate space is dissected, 15 x 15 cm mesh is placed and spread. Care should be taken not to have any folding in the mesh. Mesh may be sutured to iliopectineal ligament. Displacement of mesh is not common. Other side also can be done together.

Preperitoneal space is a potential space in front of the peritoneum, behind the transversalis fascia and anterior rectus muscle. Below in front of the urinary bladder, it is called as space of Retzius (med ially), laterally it is called as space of Bogros. Median umbilical fold is formed by urachus in the midline. Medial umbilical ligament is formed by obliterated umbilical arteries, lateral umbilical fold by inferior epigastric vessels. Three fossae are lying in relation to these folds- supravesical and medial fossae are medial to lateral umbilical fold which are sites of direct hernia whereas lateral Iossa is lateral to lateral umbilical fold and is site of indirect hernia. In 1956 Fruchaud described his myopectineal orifice bounded medially by the lateral border of rectus abdominis, laterally by iliopsoas, superiorly by conjoint tendon and inferiorly by pectin pubis. This area is the site of groin hernia which should be covered by mesh of adequate size to strengthen the defect and to prevent the recu rrence. lliopubic tract is analogue of the inguinal ligament, extends from Cooper's ligament to anterosuperior iliac spine which divides endoscopic view of preperitoneal space into superior compartment (contains inferior epigastric artery, Hesselbach's triangle, cord structures and is site of inguinal hernia) and inferior compartment (contains femoral canal, iliac vessels, iliopsoas muscle, genitofemoral nerve, lateral femoral cutaneous nerve). External iliac vessels lie in a triangle formed by gonadal vessels laterally, vas deferens medially and peritoneal reflection inferiorly (triangle of doom).

759

c-,

(TAPP) o 10 mm

rrt

::c > -a --i m

5 m~ (TAPP)

:x:,

.....

5 mm (TAPP)

l

00

l -10mm(TEP)

I :c

t-5 mm (TEP)

----

Fig. 18.61 : TEP after dissection prior to placement of mesh. Direct

t -5 mm (T~

defect, inferior epigastric artery, pubic symphysis and Cooper's ligament are clearly seen.

Fig. 18.58: Ports used for TEP and TAPP. Rectus muscle

,.. /

-

skin

-- -- ~

:

•

' - ••• • ••-

Ill

-

Normal anatomy of abdominal wall

Abdominal muscles (external/internal/transverse) Preperitoneal space Transversalis fascia Peritoneum Intestines

Preperitoneal approach (for TEP)

Transabdominal preperitoneal approach (TAPP)

Fig . 18.59: Diagrammatic representations of TEP and TAPP.

Fig. 18.63: TEP mesh placement in preperitoneal space.

Fig. 18.60: Port incisions in TEP.

Aberrant obturator artery is an occasional branch of infe, 1vr epigastric artery replacing its pubic branch travels across Cooper's ligament, which during fixation of mesh can cause torrential haemorrhage- circle of death. Triangle of pain is formed by gonadal vessels medially, iliopubic tract laterally and peritoneal reflection below. Genitofemoral nerve and lateral cutaneous nerve of thigh traverse this triangle. Injury to these nerves either by dissection or by tacks cause postoperative pain. Tacks/staplers should not be placed in this triangle.

Smile is the curve which puts everything straight.

...

C'D :::I

Q)

760

...

Q)

CJI

:::s

Cl)

Indications for TEP

Contraindications for TEP

• Recurrent hernia • Bilateral hernia • Indirect/direct/femoral hernia

• Obstructed/strangulated inguinal hernias • Ascites • Bleeding disorders

Landmarks to be identified Principles in TEP in TEP

0

• • • • • •

Pubic bone midline Inferior epigastric artery Cooper's ligament lliopubic tract Cord and vas deferens Psoas muscle and nerves in relation

B

• Head-down supine position • Surgeon standing on opposite side of hernia • Camera person placed on opposite side of hernia • Monitor at foot end • Catheterise/empty the bladder properly prior to TEP • Adequate wide space creation • Careful dissection of cord and sac • Ligate indirect sac • Mesh should not be fixed laterally • Size of mesh is 15" 15 cm • Two point fixation-one at pubic bone other at Cooper's ligament by tacks/staplers

Obstruction .J, Initially venous return is impaired .J, Congestion of the bowel .J, Further dilatation of the bowel which becomes purple coloured .J, Fluid collects in the sac .J, Eventually arterial blood supply is impaired .J, Bowel becomes dark, brownish black coloured with flabby and friable wall .J, Bacteria migrate transerosally and multiply in fluid of the sac .J, Perforation occurs at the site of constriction ring .J, Peritonitis occurs. Strangulation commonly occurs in the small bowel and also in large bowel. Occasionally strangulated omentocele also can occur without any intestinal obstruction. Strangulation can occur in inguinal, femoral, obturator, umbilical or any hernias. Indirect inguinal hernia is more prone for strangulation than direct inguinal hernia. It is due to narrow neck, adhesions, narrow external ring in children. Part of circumference of the bowel when strangulated, is called as Richter's hernia wherein the patient presents with diarrhoea, toxicity mimicking gastroenteritis. Richter's hernia is more common with femoral, obturator hernias.

DIFFICULTIES ANO COMPLICATIONS IN TEP REPAIR

Difficulty in dissecting indirect sac. Cord/vas injury Inadvertent opening of the sac/peritoneum and creation of pneumoperitoneum. Injuries to major structures like iliac vessels--0.5-1 .0% Displacement of mesh or erosion into structures like urinary bladder-rarely may occur Nerve injury; Formation of seroma/haematoma Infection; Recurrence ADVANTAGES OF TEP REPAIR

~

Approach is totally eXlraperitoneal Small incision; Proper placement of mesh in right space that is preperitoneal space itoneal cavity is intact and not o_p_en_e_d_ _ _ _ _ __.,

I INCARCERATED HERNIA

Abdominal -

wall Ring blocking the content Toxic fluid

Inguinal canal Inguinal ligament

Here the lumen of the portion of colon occupying a hernial sac is blocked with faeces. Here scybalous content of the bowel should be capable of being indented with the finger, like putty. In incarcerated hernia, sac and contents are densely adherent to each other (contents are fixed to sac). It is always irreducible; often obstructed but may not be strangulated.

Thigh

Sac of hernia

Fig. 18.64: Picture showing strangulated hernia with

toxic fluid and site of constriction.

STRANGULATED HERNIA

I Pathology

It occurs when blood supply of the contents of hernia is seriously impaired leading to formation of gangrene. Common bacteria in strangulated hernia: E. colt, Anaerobic streptococci; Anaerobic bacteria; Klebsiella.

rl Figs. 18.65A and B: (A) Strangulated inguinal hernia will be tender and

irreducible without impulse on coughing; (B) Hernia with gangrenous omentum. Note the colour of the gangrenous omentum.

761

Causes of strangulation: Narrow neck; Adhesions; Irreducibility; Long-time, large hernia with adhesions Maydl 's hernia (Hernia-in-W): Here a loop of bowel in the form of 'W' lies in the hernial sac and the centre portion of the 'W' loop is strangulated and lies within the abdominal cavity. Thus local tenderness over the hernia is not marked and hernia gets reduced with the strangulated loop in the centre of the "W". Strangulation in this case is often missed during surgery and may lead to peritonitis due to retained gangrenous loop. ,.___ _ _ _ _ Strangulated segment

n :c

>

'"ti

-t

m

::i::,

..... 0)

:c

Fig. 18.67: In an obstructed hernia, if reduction is forced it may get

reduced with the sac and ring with the obstructed/strangulated bowelReduction-en-masse.

In strangulated omentum features of obstruction are not present (i.e. vomiting , constipation). Omentum becomes congested, oedematous and black in colour which secretes toxic fluid with secondary bacterial infection. But here, initially the sepsis is slower than that of strangulated intestinal obstruction. Eventually the infection spreads causing diffuse peritonitis. Downward spread of infection can cause scrotal abscess. Fig. 18.66: Maydl's hernia (Hernia-in-W).

I Clinical Features of Strangulated Hernia Sudden severe pain, initially over a pre-existing hernia which later becomes generalised over the abdomen; 3% in incidence. Persistent vomiting, constipation and distension of the abdomen. Hernia is tense, severely tender, irreducible and without any expansile impulse on coughing. Rebound tenderness is diagnostic. Features of toxicity and dehydration; Electrolyte imbalance; Abdominal distension with guarding and rigidity; Oliguria.

B • Incidence is 4%. Femaleto male ratio is 5:1 • In female infants, the content may be ovary with or without Fallopian tube

I Investigations Plain X-ray abdomen in erect posture shows multiple air-fluid levels. Serum electrolytes; Blood urea and serum creatinine; Total count is increased; US abdomen.

•·· Treatment of Strangulated Hernia , The patient is admitted; , Ryle's tube aspiration; , Intravenous fluids to correct dehydration and electrolyte imbalance; Antibiotics; , Catheterisation to maintain adequate urine output; , Emergency surgery.

I Taxis Often in irreducible hernia, reduction of hernia is tried by elevation, sedation and taxis (i.e. with flexion and medial rotation of the hip, reduction of hernia is tried). In obstructed hernia, taxis may be dangerous as during taxis, contusion and rupture of the intestinal wall can occur. Reduction-en-masse may mask the gangrenous bowel existing in the sac. Inner gangrenous loop of Maydl's hernia may be missed. Rupture of the sac extraperitoneally is also a possibility. Taxis has no role in femoral hernia and strangulated hernia. If tried, contusion, reduction-en-masse and rupture of the sac can occur.

....::::I

(I)

• Incision - - -"'-· Hernia sac - - - - - - '\ Content -

- -___c_,_;

Fig. 18.68: Incision for strangulated hernia is obliquely placed extending to the scrotum.

The most important thing that you wear is the expression on your face.

Q)

762

...

Groin incision is made with incision extending into the most prominent area of the swelling. ,!, Sac is exposed.

Q)

en

Constriction ring and superficial ring is released (cut).

'E

Sac is opened carefully without allowing the spillage of fluid (Usually spillage occurs extraperitoneally) ,!, Fluid is sucked with a suction apparatus. ,!, The bowel is held with fingers so as to prevent it from getting reduced. ,!, The viability of the bowel is checked by colour, peristalsis, pulsation, bleeding. ,!, When gangrenous, resection and anastomosis is done and drain is placed.

= en

SLIDING GROIN/INGUINAL HERNIA (HERNIA-EN-GLISSADE) Here posterior wall of the sac is not only formed by the parietal peritoneum, but also by sigmoid colon with its mesentery on left side; caecum on right side and often with portion of the bladder (Both sides) (1:2000). Rarely small bowel sliding hernia or sacless sliding hernia can occur (1 :8000). Content of the sac is usually small bowel or omentum. Sliding hernia occurs exclusively in males. Mainly on the left side (85%). It commonly occurs in indirect sac even though femoral and direct sliding hernias are known to occur. Bilaterality is extremely rare. It is seen commonly in adults and elderly. Huge hernia can occur which extends into the scrotum and do not get reduced totally. Inferior epigastric artery

Bassini's repair is done by placi ng interrupted nonabsorbable sutures. Antibiotics, IV fluids are continued. Drain is removed in 4- 5 days. Once the bowel movement begins, oral diet is started (in 5 days). r-:,,.....--

- --

-----

--+- Urinary bladder

-,- Sliding hernia

Sac

Fig. 18.70: Sliding hernia with urinary bladder as posterior wall of the sac.

I Clinical Features (2%) Large globular swelling in the inguinal region descending into the scrotum, often irreducible. Sliding inguinal hernias are almost always discovered intraoperatively. But clinically it can be suspected. Small bowel as the content of sliding hernia can lead to strangulation. Posterior wall of sac

Figs. 18.69A to E: Strangulated hernia-left side. It is tense, tender, without any impulse on coughing. Note the incision, discoloured sac, content (gangrenous bowel) and proximal viable bowel.

Note: During surgery for strangulated hernia mesh is usually not used, only repair is done. Biological mesh can be used.

Postoperative problems: Infection , leak with fistula, septicaemia.

Figs. 18.71A and B: Sliding hernia (Hernia-en-Glissade).

I Treatment

During surgery, indirect sac may be missed and so leads to recurrent hernia through retained (or unidentified) indirect sac. Here both medial and lateral sacs straddle the inferior epigastric artery. It is one of the causes for recurrent hernia.

Always Surgery Posterior wafl of the sac should not be separated from large bowel or bladder. If tried, injury may result to these organs leading to faecal or urinary fistulas. Partially excised sac is pushed into the peritoneal cavity with posterior wall and repair is done usually using prolene mesh (Hernioplasty). In LaRoque repair deep ring is incised above and below; reefing of the sigmoid colon mesentery is done which forms the posterior wall of the inguinal canal so that entire sigmoid is replaced into the peritoneal cavity. Posterior wall of the sac should not be separated from large bowel or bladder. If tried it may lead into faecal or urinary fistulas. Often sac can be excised partially; this partially excised sac can be pushed into peritoneal cavity. Right sided sliding hernia will have caecum and appendix in its posterior wall; caecum should not be separated from posterior wall of the sac which may otherwise create faecal fistula. Appendix should not be removed as it may precipitate sepsis. Appendices epiploicae from sigmoid colon should not be removed as there are chances that they may contain small colonic diverticula which may get opened to contaminate the field. Bladder will be present on medial side of the sac and sac should not be separated; if bladder injury occurs it should be sutured in two layers with vicryl. Inside out purse string suture on the opened sac is applied and the sac with its posterior wall is pushed into the abdominal cavity. Urinary catheterisation is a must. Modified Bevan technique for repair of sliding inguinal hernias is often used successfully. Here sac is excised partially and is sutured over the sliding component as "reperitonealisation". Then only mesh repair (Lichtenstein) is done. Truss should not be used in sliding hernia.

B

--t

Clinically difficult to diagnose. Often it is diagnosed on table. • Processus vaginalis is closed at internal ring and hernial sac either invaginates processus vaginalis as "inverted umbrella" or comes behind processus vaginalis.

I

I

I Surgical Anatomy of Femoral Canal It is the medial, most compartment of the femoral sheath, which extends from femoral ring above to saphenous opening below. It contains fat, lymphatics, lymph node of Cloquet. It is 1.25 cm long and 1.25 cm wide at the base. Below it isclosed by cribriformfascia. Femoral ring is bounded anteriorly by inguinal ligament; posteriorly by iliopectineal ligament of Cooper, pubic bone and fascia covering the pectineus muscle; medially by concave, sharp lacunar (Gimbernat's) ligament; laterally by athin septum separating from femoral vein. /

Anterior superior iliac spine Inguinal ligament

Femoral vein

L Femoral hernia

Pubic bone

Fig. 18.73: Surgical anatomy of femoral hernia. Anterior superior iliac spine Femoral artery Femoral vein Adductor longus Lateral cutaneous nerve of thigh Profunda femoris artery

it-li--1.,_.,,

Sartorius -

...-..,.:..

Indirect sac

Fig. 18.74: Femoral triangle.

It takes 26 muscles to smile, and 62 muscles to frown.

::ti

FEMORAL HERNIA

Femoral nerve

inferior epigastric artery having both direct and indirect sacs.

>

m

Inferior epigastric artery

Fig. 18.72: Saddle/Pantaloon hernia. It is on either side of the

n ::c ""Cl

PANTALOON HERNIA (DOUBLE HERNIA, SADDLE HERNIA, ROMBERG HERNIA) Here both direct and indirect inguinal sacs are present and clinically present as direct hernia.

763

00

764 Femoral hernia

Inguinal hernia

I

Figs. 18.75A and B: Hernia; inguinal hernia is above and medial to tubercle, and femoral hernia is below and lateral.

•·· Aetiology: Wide femoral canal; Multiple pregnancies. ... Pathology in femoral hernia: Through femoral canal, hernial sac descends down vertically up to saphenous opening and then escapes out into the loose areolar tissue to expand out like a retort. Because of its irregular pathway and narrow neck, it is more pronefor obstruction and strangulation. During surgery, precaution should be taken about the femoral vein and pubic branch of obturator artery (or accessory obturator artery) which often may get injured leading to torrential haemorrhage.

I Features •·· Common in females (2:1 ratio), common in multipara. Rare before puberty. 20% occurs bilateral, however, more common on right side. Presents as a swelling in the groin below and lateral to the pubic tubercle (Inguinal hernia is above and medial to the pubic tubercle). Swelling, impulse on coughing, reducibility, gurgling sound during reduction, dragging pain, are the usual features. When obstruction and strangulation occurs which is more common, presents with features of intestinal obstruction-painful, tender, inflamed, irreducible swelling without any impulse. They also present with abdominal distension, vomiting and features of toxicity. Often femoral hernia can be associated with inguinal hernia also. 40% of femoral hernias present as emergency hernia with obstruction/strangulation. Currently CT scan is used.

B Inguinal hernia • An enlarged Cloquet lym ph node of any cause Psoas abscess-psoas spasm with flexed hip but difficulty in extension Lipoma Femoral aneurysm Distended psoas bursa (disappears on hip flexion) Saphena varix-it is enlarged terminal portion of long saphenous vein. It is soft, disappears on lying down, fluid thrill, impulse on coughing and venous hum on auscultation are present. There is associated varicose veins on leg • Haematoma in the region Note: • Gaur's sign: In femoral hernia, distension of superficial epigastric and/or circumflex iliac veins occurs due to the pressure by the hernial sac. • Hydrocele of femoral hernia occurs when adherent omentum which is the content secretes fluid into the sac. • Herniation through a gap in the lacunar ligament (medial) is always strangulated and is called as Laugier's femoral hernia (L for L). • In congenital dislocation of hip, femoral hernia occurs behindthe femoral vessels-Narath 's femoral hernia. • If sac lies under the pectineal fascia, is called as Cloquet's hernia. • Strangulation and Richter's hernia are common in femoral hernia. • Otten on medial side, a portion of bladder forms the wall of the femoral hernial sac- sliding-femoral hernia. • Femoral hernia-occurs medial to femoral vein • Narath's hernia-occurs behind femoral artery, in congenital dislocation of hip • Hesselbach's hernia-occurs lateral to femoral artery • Serofini's hernia-occurs behind femoral vessels • Laugier's hernia-through lacunar ligament • Teale's hernia-in front femoral vessels • Callison-Cloquet hernia-through pecti neal fascia • Cooper's hernia-femoral hernia with two sacs • The de Garengeot's hernia-incarcerated (acute appendicitis) appendix in femoral hernial sac

Fig. 18.76: Femoral hernia on right side. Note the location below the inguinal ligament. Femoral hernia is common on right side. It is common in females.

I Treatment

765

Approaches 1. Lockwood-low operation: Here sac is approached below the inguinal ligament through groin crease incision (or over the swelling) so that fundus of sac is dissected by direct vision and repair is done from below. Here inguinal ligament is sutured to Cooper's ligament. standard and ideal (Cooper's ligament repair). 2. Mc'Evedy-high operation: A incision is made over the femoral canal extending vertically above the inguinal ligament. Sac is dissected from below, neck from above and repair is done from above. It gives a very good exposure of both neck, fundus of sac and repair is also easier. It is done in strangulated femoral hernia. 3. Lotheissen's operation: It is through inguinal canal approach (like for inguinal hernia). Transversalis fascia is opened and neck of the sac is identified in the femoral ring. Sac is dissected from above, neck is ligated and repair is done.

("')

:c

>

"'ti

--t m :::,:, -,I,

Cl0

Mc'Evedy-high approach

Figs . 18.79Ato F: Strangulated femoral hernia. Note: clinical look,

Richter's gangrenous bowel, resection and Lotheissen's repair.

i

Lockwood-low ...../, approach [

I 2

15

1. Femoral hernia 3. Femoral artery 5. Lacunar ligament

2 . Femoral vein 4. Femoral nerve 6. Cooper's

Fig. 18.77: Different surgical approaches for femoral hernia.

Lotheissen's repair After herniotomy, conjoined tendon is sutured to iliopectineal line (ligament) by interrupted sutures (2 or 3), using nonabsorbable monofilament sutures. Care should be taken to avoid injury to femoral vein, pubic branch of obturator artery, bladder. It is not as strong as Cooper's ligament repair. Complications: Bleeding haematoma, abscess formation. 4. AK Henry's approach: Repair of bilateral femoral hernia through lower abdominal incision. 5. Polypropylene mesh can be buttressed over the femoral canal to close the defect. 6. Laparoscopic mesh repair-TEP/TAPP.

VENTRAL HERNIA Any protrusion through abdominal wall with the exception of hernia through the inguinofemoral region is defined as ventral hernia. lncisional hernia (80%) and primary defects in abdominal fascia which can cause umbilical hernia, epigastric hernia, paraumbilical hernia or Spigelian hernia are grouped under ventral hernia.

B Fig. 18.78: Femoral hernia repair. Inguinal ligament is approximated to

Cooper's (lliopectineal line) ligament. In Lotheissen's repair conjoined tendon is sutured to iliopectineal line.

Reducible; Irreducible; Obstructed; Strangulated; Single; Multiple small defects (Swiss cheese hernia). Causes: Congenital defect; Obesity; Smoking; Chronic cough.

Love means sharing the same road, wherever it leads.

766

It is common in old age and obese individuals. It occurs in 10% of abdominal surgeries; 70% occurs in fi rst 5 years; 30% occurs in 5-1 0 years.

Figs. 18.80A and B: Large ventral hernias. It needs proper mesh repair.

Fig. 18.83: lncisional hernia adherent to skin. Note the ulceration over the summit. Patient underwent resection and anastomosis with removal of the ulcerated skin with mesh placement.

Additional History to be Collected in lncisional Hernia Figs. 18.81A and B: (A) CT scan showing ventral hernia which is irreducible; (B) Ventral hernias with Swiss cheese multiple defects. Palmar point (camera port) 10mmor5mm

~ent~al __['\

* ern1a

\)

_:Jm

Working po~s5

Figs. 18.82A and B: (A) Port placement for laparoscopic ventral hernia repair. Left subcostal point is Pa/mar point, (B) Laparoscopic view of

ventral hernia with omentum as irreducible content. Content is released and duval or four-layered mesh is fixed with tackers or sutures.

Preoperative preparation: Proper skin hygiene; Respiratory care; Control of obesity; Antibiotics.

Management: ,. Relevant investigations; ,. Open mesh repair-inlay; onlay; sublay-retrorectus; underlay; ,.. Laparoscopic mesh placement-underlay (directly in front of the contents) using dual mesh or four layered mesh.

INCISIONAL HERNIA It is herniation through a weak abdominal scar (scar of previous su rgery).

Details of surgery that patient has undergone earl ier. Duration after how long incisional hernia has occurred is important. History of wound infection, wound dehiscence, whether surgery done was an emergency or elective, and ten sion sutures placed or not. History of pain, irreducibility and details of precipitating factors to be asked. Other precipitating factors are similar to inguinal hernia like smoking, urinary/respiratory/abdominal symptoms. FACTORS RESPONSIBLE FOR DEVELOPMENT OF INCISIONAL HERNIA Vertical incision has got higher chances of incisional hernia than horizontal incision • Layered closu re of the abdomen has got higher chance than single layer Continuous closure has got higher chances than interrupted closure Using absorbablesuture material has got higher chances of hernia than nonabsorbable sutures Emergency surgical wound has higher chances than elective surgical wound Laparotomy for peritonitis, acute abdomen, and trauma can commonly cause incisional hernia Drainage through the main laparotomy wound may precipitate formation of incisional hernia Chronic cough, smoking, obstructive uropathy, constipation can precipitate incisional hernia Causes which increases the intra-abdominal pressure (BPH , straining, stricture urethra or rectum, ascites). Diabetes, old age, malnutrition, malignancy , anaemia, hypoproteinaemia, jaundice, ascites, liver disease, uraemia, steroid therapy, immu nosuppressive diseases are other precipitating factors

-- ------

I Features Swelling in the scar region. Pain; Impulse on coughing; Gurgling sound. Often bowel peristalsis may be visible under the skin. Eventually features of irreducibility, obstruction , strangulation is seen.

Scar, its extent and location , whether healed primarily or secondarily, skin over the scar and swelling is noted. Details of the swelling with expansile impulse on coughing and examination both in lying down and standing are done. Gap cannot be assessed in an irreducible hernia.

Small defect 2 cm Massive/diffuse Multiple defects-Swiss cheese pattern - - - - - - -- ----

U

--

Note:

• Size of the defect is important to decide the type of surgical closure in incisional hernia. • Midline hernia expels the content more outwards due to contraction of rectus muscles on both sides. Investigations: Always the precipitating factors must be looked for: Chest X-ray; US abdomen; Tests relevant for causes. Complications of incisional hernia: Irreducibility, obstructio:J , strangulation, incarceration

- - --- - - - - - - - -

Preoperative Preparations for lncisional Hernia Surgery Reduction in weight and control of obesity. Nutrition, control of anaemia. Treatment for diabetes, hypertension , cardiac diseases, respiratory problems. Treating the precipitating causes. Chest X-ray, U/ S abdomen to be done. Massive incisional hernia after reduction may cause IVG compression, paralytic ileus and diaphragmatic elevation with respiratory embarrassment (abdominal compartment syndrome). This can be prevented by prior increasing the capacity of peritoneal cavity by creating pneumoperitoneum using CO2 so as to increase the peritoneal cavity pressure by 12-15 cm of H20, daily for 3-6 weeks. Later definitive su rgery is done.

Figs. 18.84A and B: lncisional hernia in a patient during straining and during relaxing.

Fig. 18.85: lncisional hernia which is irreducible with visible bowel beneath.

B Defect is felt and assessed during head rising, by placing fingers over the scar horizontally; Size of the defect is very important

--

----

Hernia is common in lower abdomen. It may be small or large; huge or massive (diffuse).

1

Fig. 18.86: CT scan abdomen showing incisional hernia.

So many languages in the world and a smile speak them all. A warm smile is the universal language of kindness.

767

768

I Treatment Strategy for lncisional Hernia Mesh repair of the incisional hernia defect is always better and ideal with less chances of recurrence-retrorectus or sublay is ideal (mesh onlay is not accepted). , Adequate sized mesh is placed either outer to peritoneum (sublay), or occasionally combined sublay and onlay mesh placement, both deep and outer to musculoaponeurotic layer is used Onlay/overlay mesh is placement of mesh outer to the abdominal musculoaponeurotic layer which is not advisable in ventral/incisional hernias. Rive's Stoppa's mesh placement for incisional hernia is placing mesh between posterior rectus sheath and rectus muscle-retrorectus mesh placement is easier for dissection and mesh placement. , Commonly polypropylene mesh is used. Other materials used are Dacron, polytetrafluoroethylene (PTFE) mesh, polyglycolic mesh (vicryl mesh) or combined polypropylene and polyglycolic acid mesh (vipro mesh). Drain (suction drain) must be placed after surgery. ,, Mesh repair is ideal approach for incisional hernia. Prevention of infection/haemostasis and drainage are important. Laparoscopic mesh repair is done for incisional hernia by placing a mesh under the defect laparoscopically in intra peritoneal plane. The only problem of this underlay placement is chances of adhesion and GI fistula formation, but still it is found to be safer. Laparoscopic preperitoneal mesh placement is also done for smaller defects. Now dual mesh (PTFE) or four layered mesh are available. Here mesh is placed under the peritoneum deep to the defect after reducing the contents. Mesh is fixed with sutures and tacks. In four-layered mesh, deepest 1st layer is absorbable cellulose which allows new peritoneum to creep underneath. 2nd layer is PDS/PTFE mesh 3rd layer is polypropylene mesh and the last 4th layer is again PDS/PTFE mesh. It is ideal but costly.

Note: • Cattell's operation: When the defect is less than 3 cm, and if the patient is having adequate abdominal muscle tone, layer by layer anatomical repair is done using monofilament nonabsorbable suture material like polypropylene/polyethylene, ideally with interrupted sutures. Sac should be dissected, ligated and excised prior to repair. Peritoneum and posterior rectus sheath is apposed as first layer and anterior rectus sheath as second layer. • Double breasting of the rectus sheath using interrupted nonabsorbable sutures using monofilament suture material. It is overlapping the rectus sheath in two layers with two rows of sutures. • Keel operation is done in large defect. Scar is excised and is dissected beyond the margin of the defect. Sac is never opened unless there is obstruction of the content. Sac in inverted using continuous/interrupted inverting non-absorbable sutures, layer by layer until the defect margins are apposed together which is then again sutured with interrupted sutures. Keel is inverted beam of the ship. • Nuttall's operation is done for lower midline incisional hernia. Recti attachments are detached from the pubic bones and are crossed over to fix to opposite pubic bones so as to create a firm abdominal wall support by crossed recti muscles.

Figs. 18.88A and B: (A) Keel operation, and (B) Nuttall's operation.

Different Types of Mesh Repair for lncisional Hernia

Figs. 18.87A to C: On table incisional hernia defect also showing mesh placement in retrorectus plane.

Outer to peritoneum is ideal method (sublay): Large sized mesh is placed in preperitoneum. It need not be fixed as abdominal pressure keeps it in position (according to Pascal's law). Under the peritoneum, directly over the content (underlay): Now it is accepted but there are chances of adhesions/fistula formation. It is used in laparoscopic repair. Dual mesh/fourlayered mesh is used. Overlay mesh placed outer to musculoaponeurotic layer. Here mesh is placed under subcutaneous tissue; it carries high recurrence rate (30%). So it is not recommended. Combined inlay and overlay with two layers of mesh. Rive's Stoppa's method of placing mesh between posterior rectus sheath and rectus muscle widely. Dissection is done at least 1O cm beyond the defect under rectus muscle in

front of the posterior rectus sheath; mesh should cover 5 cm beyond the defect all around. Recurrence rate with this is less than 10%. Components separation technique is better method in large defects. Here skin and subcutaneous fat are dissected off the anterior rectus sheath external oblique is incised 2 cm lateral to rectus abdominis external oblique is separated from internal oblique until posterior axillary additional relaxing incisions are made over rectus sheath, internal oblique, transverse abdominis it is repeated on opposite side 20 cm mobilisation is achieved also reinforced with a large mesh. Component separation technique causes often lateral bulges on both sides. If it is done without mesh support recurrence rate is high. Advantage is defect up to 20 cm can be easily brought together. Technique is also called as Autologenous repair by vascularised innervated muscle flaps) (Ramirez, 1990). Bridging at myoaponeurotic level outer to peritoneum (inlay): Adequate sized mesh is placed in the defect level bridging the gap to cover preperitoneum. It is better by principle but still shows high recurrence.

I Postoperative Care Antibiotics; Analgesics; Nasogastric aspiration. Abdominal binder for support. Prevention of paralytic ileus. Control of obesity and other precipitating factors. Stop smoking and treat other associated causes. Early ambulation; Fluid management, catheterisation. Drain should be kept until drainage becomes minimal. Abdominal binder is used to support abdominal wall during recovery period.

B • Wound infection, seroma formation Paralytic ileus, abdominal compartment syndrome in large hernias; Wound sinus, enterocutaneous fistula Infection of the mesh, recurrence

Often might need resection of the adherent bowel segment. Large mesh placement is required. Note:

• It is now universally accepted that prosthetic repair is gold standard for all incisional hernia. Prostheses used are polypropylene mesh, e-PTFE, Dacron, dual intraperitoneal mesh, biologic grafts. • Non prosthetic repair has got only historical value as recurrence rate is very high (as high as 55%).

UMBILICAL HERNIA

I Anatomy of Umbilical Region Umbilical ring is a complex structure which is related to linea alba, falciform ligament, obliterated urachus (median umbilical ligament) and umbilical fascia (Richet's fascia). It is located at the level of L4 and L5 vertebral disc. It is at lower position in infants. It is water shed area for venous and lymphatic drainage-above umbilicus it drains to axillary vein or lymphatics; below to inguinal area. Umbilical skin is supplied by T10 spinal cord. It is the meeting point of four folds of embryonic plate and three systems-GI (vitellointestinal), urinary (urachus), and vascular (umbilical vessels). Umbilical hern ia develops due to either absence of umbilical fascia or incomplete closure of umbilical defect. Weakest part in the umbilical cicatrix is upper part where hernia begins.

I Features Umbilical hernia can be congenital in newborn and infants (common in males) or acquired in adults (common in females). Congenital umbilical hernia is common in Africa or in African origin people (8 times). Acquired is like any ventral hernia. It is herniation through a weak umbilical scar (cicatrix). It is common in infants and children, occurs commonly due to neonatal sepsis. Male:female :: 2:1 . It is seen in 20% of newborn infants. Umbilical hernia is common in Down's syndrome, BeckwithWeidman syndrome, ascites.

I Additional Problems in Large lncisional Hernia Wh ile reducing the bulky contents like bowel and omentum, inadequate intra-abdominal capacity leads to increased intra-abdominal pressure causing IVG compression, mesenteric oedema followed by stasis of splanchnic bed, paralytic ileus, diaphragmatic elevation and respiratory distress (abdominal compartment syndrome) , urinary and bowel disturbances. Abdominal capacity can be raised by creating regular pneumoperitoneum over the period of 3-6 weeks. Lordosis and back pain may be presenting feature. Sac and contents may get adherent to the thin skin over the summit of the hernia leading to skin ulceration and occasionally fistula formation.

Figs. 18.89A and B: Umbilical hernia in a male child and adult.

Often it can attain a large size.

Luck is when opportunity knocks and you answer.

769

770

Presents with a swelling in the umbilical region within first few months after birth, the size increases during crying. It is hemispherical in shape. Defect can be felt with finger during crying. Occasionally it can go for irreducibility and obstruction which presents with pain, distension, vomiting.

I Treatment Initially conservative. In 93 to 95% of cases, it disappear spontaneously in few months after birth (masterly inactivity). It can be hastened by adhesive strapping across the abdomen.

Umbilectomy: Unhealthy thin skin over the large umbilical hernia is a real problem. It is better to do umbilectomy in these patients (exci· sion of umbilical cicatrix). It is done only in adult with large umbilical hernia with thinning of umbilical skin. Prior consent and eventual creation of umbilicus is needed. Open dual PTFE and polypropylene mesh placement: Umbilical hernia is dissected similarly through subumbilical incision. Redundant sac is excised . Peritoneum is not closed. A special composite mesh containing wider PTFE on the inner side with little smaller polypropylene mesh on the outer aspect is used. It has also got two additional straps of the mesh attached to the outer part of the main composite mesh which are used to hold and later to fix into the defect anteriorly. This has got excellent results with low recurrence rate. Laparoscopic umbilical hernia repair:

Inferior -••---"'...,. I . crease mc1s1on

(7 Jmbi1;~1) -.-e--

It is similar like any ventral hernia repair through laparoscopy which is done under general anaesthesia. It is useful only in large umbilical hernia.

Fig. 18.90: Incision for umbilical hernia repair (infraumbilical)-

inferior crease incision.

Fig. 18.91 : Umbilical hernia dual mesh to place both intraperitoneally

If persists even after the age of two years. If the defect is more than 2 cm in size. Acquired/adult umbilical hernia.

Different surgeries are: Primary closure of the defect: An infraumbilical incision is made encircling its lower half. Sac is dissected circumferentially and is released off from the umbilicus and subcutaneous tissue. Sac is opened; contents are reduced; excess part is excised up to the umbilical ring. Defect is closed with interrupted nonabsorbable polypropylene sutures. Mayo's operation: Here horizontally opened rectus sheath is approximated as double breasting with upper flap overlapping lower flap in front. It is rarely done today. Sublay mesh repair: In a large umbilical hernia (>3 cm size defect) with a degenerated skin on its surface it is often difficult to retain the umbilicus. When umbilicus is tried to be saved, infraumbilical incision should extend laterally about 2 cm on each side at 3 and 9 o'clock positions. Sac is dissected similarly. Sac is excised after excision of redundant sac. Presently it is standard to use polypropylene mesh as sublay or in retrorectus position and then rectus sheath is closed.

and retrorectus plane with straps to fix.

PARAUMBILICAL HERNIA (Supra- and lnfraumbilical Hernia) It occurs commonly in adults. It is a protrusion or herniation through linea alba, just above or below the umbilicus. It enlarges ovally, often attains a large size and sags downwards. Neck of the sac is relatively narrow. Contents are usually omentum, small bowel, sometimes large bowel. It has got tendency to go for adhesion, irreducibility and obstruction.

Figs. 18.92A and B: Large ventral hernia front and side views. It needs preperitoneal mesh repair.

Predisposing factors: Obesity; Multiple pregnancies; Flabby abdominal wall.

I Clinical Features Common in females (5:1 ratio). It presents as a swelling which has smooth surface, distinct edges, soft, resonant with dragging pain and impulse on coughing. Large hernias can present with intestinal colic due to subacute intestinal obstruction. Eventually strangulation can occur.

If the defect is less than 1.5 cm, lateral margin of the defect is formed by only anterior and posterior lamina of the rectus sheath; if the defect is> 1.5 cm, then lateral margin is also formed by rectus muscle. Content of true epigastric hernia is usually omentum, sometimes it may be small bowel. Common in muscular men; manual labourers.

771

n >

:c m

:a co :::c

...

(D

I Treatment

:::,

Is always surgery Dissection of hernial sac and placement of mesh in retrorectus plane and under the umbilicus is the ideal treatment. Often umbilectomy is required and also mesh placement is beneficial (when defect is> 4 cm in size). If there is strangulation, resection of bowel segment and anastomosis is done followed by repair of the hernia. Mayo's operation: Through a transverse elliptical incision, sac is identified and dissected. Herniotomy is done. Double breasting of the defect in the rectus is done by interrupted nonabsorbable sutures. Additional lipectomy (panniculectomy) may be done in case of pendulous abdomen.

0 0

I»

j Swiss cheese defects

Fig. 18.93: Epigastric hernia. Often multiple Swiss cheese defects are seen.

B

~

It is done for umbilical and paraumbilical hernia Once lower flap or umbilicus is raised above, sac is identified, dissected and opened After reducing the contents sac is transfixed using vicryl Rectus sheath is repaired with double breasting using nonabsorbable sutures between superior and inferior fascial margins as 'vest on pant' imbrications Skin flap is closed with a drain. Infection, recurrences (30%) are known complications t is not commonly used now

Fig. 18.94: Epigastric hernia showing features of strangulation.

Postoperative weight reduction, use of abdominal binder is required for these patients.

EPIGASTRIC HERNIA (Fatty Hernia of Linea Alba) It is 10% common; common in males. 20% of epigastric hernias are multiple-Swiss cheese like. It occurs usually through a defect in the decussation of the fibres of linea alba, any where between xiphoid process and umbilicus. Extraperitoneal fat protrudes through the defect as fatty hernia of the linea alba presenting likea swelling in the upper midline with an impulse on coughing. It is sacless hernia. Later protrusion enlarges and drags a pouch of peritoneum, presenting as a true epigastric hernia.

Often asymptomatic. Swelling in the epigastric region which is tender. Pain in epigastric region. It is often associated with peptic ulcer and so pain may be due to peptic ulcer. So gastroscopy is done to rule out acid peptic disease. Impulse on coughing; defect in the epigastric region are also fuu~. Irreducibility, obstruction, strangulation as seen in any other hernia can also occur in epigastric hernia. _ _ __

I

J

I Treatment Through a vertical incision, sac is dealt with. Defect is closed with nonabsorbable interrupted sutures. Large defect is supported with preperitoneal mesh.

Those who can't hear the music think the dancer's mad.

772 ,-----;:;

------

-----~

Commonly it is deep to external oblique aponeurosis Types: - Preperitoneal-between peritoneum and transversus abdominis muscle-20% - lnterparietal/intermuscular-between external oblique and internal oblique; most common-60%. It is commonly associated with inguinal hernia - Extraparietal (inguinosuperficial)-herniates through external 1 oblique aponeurosis into subcutaneous plane-20% U/S; X-ray abdomen; often CT confirms the diagnosis Through a transverse incision, surgical ligation of sac, repair or mesh placement is the treatm~ __ __ __ _J

c=Q)

...

Cl

=

Cl)

0

I

SPIGELIAN HERNIA It is a type of interparietal hernia occurring at the level of arcuate line through spigelian point. Hernial sac lies either deep to the internal oblique or between external and internal oblique muscles. It is lateral ventral hernia through Spigelian fascia at any point along its line. Semilunar line of Spigel is a line from pubic tubercle to tip of 9th costaI cartilage. It marks the lateral margin of the rectus sheath. Semicircular arcuate line (fold) of Douglas is lower end of posterior lamina of rectus sheath below the umbilicus and above the pubis. Spigelian fascia is area between lateral border of the rectus muscle and external and internal oblique and transversus abdominis muscle. Spigelian hernia can occur above (10%) or below (90%) the umbilicus. Below the umbilicus it occurs at the junction of linea semilunaris and linea semicircularis (wider and weaker point). In Spigelian hernia, defect is formed by internal oblique and transversus abdominis muscle. External oblique is outer to the hernial sac.

I Features Figs. 18.95A to D: Epigastric hernia-incision, sac and defect.

Complete reconstruction of linea alba is needed from xiphisternum to umbilicus especially in Swiss cheese type using different methods like-interrupted primary closure using polypropylene sutures; modified shoelace technique is used after removing strip of medial margins of the linea alba; dou ble breasting of the linea alba.

Presents as a soft, reducible mass lateral to the rectus muscle and below the umbilicus, with impulse on coughing. Strangulation is common in spigelian hernia.

B

L

Herniation through parietal peritoneum into various layers of the abdominal wall Common in Down's syndrome, Prune Belly syndrome Often it can attain large size May mimic abdominal wall lipoma; haematoma As neck of the sac is often narrow, can present with irreducibility or obstruction

Spigelian hernia - - - iµ_ - ~

I

Contd...

Linea semicircularis~ 4 - - - - - - ' (Douglas)

Fig. 18.96: Spigelian hernia.

Presence of precipitating factors like obesity, chronic cough, old age, multiple pregnancies. Common in females after 50 years of age. Investigation: Ultrasound abdomen. Differential diagnosis: Abdominal wall lipoma; Soft tissue sarcoma; Abdominal wall haematoma.

I Treatment Through a lengthy transverse incision herniotomy and later closure of the defect layer by layer using nonabsorbable interrupted sutures. But ideally mesh is required to cover the defect properly. Laparoscopic dual mesh placement is also useful.

Referred pain in knee joint through geniculate branch of obturator nerve signifies not only obturator hernia but also strangulation-Howship-Romberg sign (50%). On per vaginal examination, tender swelling is felt over the obtu rator foramen. Here strangulation is usually of Richter's type. Treatment: , Laparotomy is done and the sac is identified. It is dissected and ligated. If strangulation is present (common), resection and anastomosis is done. Broad ligament is stitched over the opening to prevent recurrence; , Mesh placement is the ideal way of repairing the obturator defect; ,. In nonobstructive type, if diagnosed clinically and by CT scan imaging, either TEP (laparoscopic) or lower abdominal mid line extraperitoneal approach may be the method to manage the obturator hernia; , In strangulated hernia, rarely additional exposure in thigh often may be required to reduce the content through a vertical medial pectineus muscle split incision.

RICHTER 'S HERNIA Figs. 18.97A and B: Spigelian hernia. On tablefinding and

placement of mesh in same patient.

OBTURATOR HERNIA

Intestinal lumen

It is hernia occurring through obturator canal between superior ramus of pubis and obturator membrane. It is a rare entity, seen in elderly females (6::1 ratio female to male).

_ __.m

I Features Usually presents with features of intestinal obstruction (85%) and more often confirmed only on laparotomy. Rarely seen as a swelling in Scarpa's triangle (20%) deep to the pectineus muscle, with limb in flexed and abducted position. Movement of limb is painful. Inguinal ligament Lacunar ligament Conjoint tendon

Inguinal hernia

Figs. 18.99A and B: (A) Strangulated femoral hernia, Richter's type

with perforation. Patient underwent resection and anastomosis; (B) Richter's hernia. It is a hernia in which the sac contains only a portion of the circumference of the intestine (small bowel). It is usually seen in femoral and obturator hernia. It mimics gastroenteritis with pain abdomen, diarrhoea, toxicity, vomiting. There are no features of intestinal obstruction. Constipation does not occur in Richter's hernia. Gangrene (strangulation) of a part of bowel occurs, eventually leading to peritonitis. Treatment: , Resection and anastomosis is done; ,. The type of hernia is treated; ,. Mortality increases with delay in surgical intervention.

LUMBAR HERNIA Fig . 18.98: Obturator hernia through obturator canal in obturator

foramen. Note also the locations of inguinal and femoral hernias.

It is herniation either through superior or inferior lumbar triangle.

We do not remember days, we remember moments.

773

C")

:c

>

-c m

:::r, 00

774

0

('Cl

:::I

C

('Cl

_rn

Superior lumbar triangle (Grynfelt's/Lesgaft's triangle) is bounded by sacrospinalis, 12th rib and posterior border of internal oblique. Inferior lumbar triang le is bounded by latissimus dorsi, external oblique and iliac crest (triangle of Petit). Lumbar hernia is more common through superior lumbar triangle. It can be: , Primary. ,.. Secondary, which is due to previous renal surgery, more common.

CCI

cc Cl!)

B Classified based on their relationship to the pyriformis muscle and ischial spine. 1. Suprapyriformis. 2. lnfrapyriformis. 3. Subpyriformis. Sac lies deep to gluteus maximus. Large hernias protrude below the buttock crease.

Diagnosis: Usually on laparotomy for intestinal obstruction. Treatment: Defect is covered by fascia mobilised from pyriformis muscle after reducing the sac contents.

COMPLICATIONS OF HERNIA SURGERY

f

Lower lumbar triangle

Upper lumbar triangle (common site)

Sites of lumbar hernia

Figs . 18.100A and B: (A) Lumbar triangles; (8) Lumbar incisional hernia-large. Previous lumbar incision scar is visible.

Differential diagnosis: Lipoma; Cold abscess; Lumbar phantom hernia. Treatment: Repair using fascial flaps or mesh.

Proper idea of complications of hernia surgery are important as often complications may become more problematic than hernia itself. 20 years back where tissue repair was popular recurrence was the most worried complication. Now since recurrence rates have come down due to standard usage of mesh, mesh related complications have become more common than recurrence. lnguinodynia, mesh extrusion, mesh infection, mesh erosion are the worried problems. • Complications of open hernia surgery

• Early

• Complications of TEP/TAPP

• Delayed

• Complications of other hernia surgeryventral/femoral

• Late • Life-threatening

I lnguinodynia It is chronic inguinal pain seen in post-hernia surgery patients (30%)-whether tissue or mesh repair. Causes are-traction, cautery, transection, entrapment. Even though it can occur in both tissue and mesh repair, it is more observed in mesh repair especially in onlay mesh repair. It is due to entrapment of nerve in the suture or in the mesh itself or nerve gets adherent to the mesh during fibrosis (perineural fibrosis) (Mesh inguinodynia). Complications of open hsmia surgery Complications of TEP/TAPP Fig. 18.101: Recurrent lumbar hernia. Note: • Phantom hernia: It is a muscular bulge as a result of local muscular paralysis due to interference with nerve supply of the affected muscles,

like poliomyelitis. It is common in lumbar region. It is often seen in lower abdomen. • Phantom limb is feeling of pain in amputated toe or limb • Phantom tumour is tumour like lesion in lung like interlobar pleural effusion

SCIATIC HERNIA It is a rare hernia. It is the protrusion of the peritoneal sac through the greater or lesser sciatic foramen.

• Infection • Groin pain; ostetitis pubis • lschaemic orchitis-due thrombosis of pampiniform venous plexus (0.5%) • Injury to vas • Injury to viscera • Recurrence • Hydrocele formation • Seroma, haematoma • lnguinodynia:-neural • Dysejaculation-painful, burning sensation just before/during/after ejaculation due to cremaster dysfunction or vas stricture (0.25%)

• SC emphysema • Pneumothorax, hypercarbia • Vascular • Neural • Visceral • Infection, ileus • Conversion • Recurrence

Immediate

Late/delayed

• Vascular-injury to iliac vessels/IE vessels • Visceral injury-bowel/ bladder • Vas injury • Anaesthetic complications

• • • • • • •

Seroma/haematoma Neural complications Intestinal obstruction Bowel adhesion/fistula Testicular atrophy Mesh related complications Recurrence

It may be transient or persistent. Nerves involved are-iliohypogastric, ilioinguinal, genital branch of genitofemoral nerve, paravasal nerves. Features are-distressing pain in the groin which often radiates to thigh, scrotum and loin. Arch and twist mobility of pelvis reproduce the pain . Bupivacaine injection relieves the pain. Imaging/nerve conduction studies are of no use. Open method has higher (32-38%) incidence of inguinodynia than TAPP/TEP. In open hernia surgery inguinodynia has replaced recu rrence as a primary compl ication. It is distressing discomfort to both patient and surgeon. Incidence is less in posterior or laparoscopic approach. It is treated with analgesics/nerve block (Injection of steroid, local anaesthetic agents, phenol, and alcohol}/transcutaneous stimulation/cryotherapy/radiofrequency therapy/neurectomy. Neurectomy is done via groin or suprainguinal or laparoscopic or laparotomy approach. Commonly groin approach is used. llioinguinal, iliohypogastric and genitofemoral nerves are carefully dissected. Nerve which is causing the problem is transected at a point where it comes out of the internal oblique muscle and proximal end should be ligated, otherwise end neuroma may form to cause recurrence of the pain. This ligated stump is buried in the internal oblique muscle. Just neurolysis may be suffi cient but chances of re-adhesion are higher with recurrence of symptoms. In difficult cases nerve may be transected through laparotomy/laparoscopy approach.

PARASTOMAL HERNIA It is herniation of intestine on the side of bowel stoma-ileostomy/colostomy/other stomas. Incidence is 8%. Classification (Devlin 's): (1) Subcutaneous-intestine herniates along the side of stoma to reach subcutaneous plane-most common. (2) Interstitial-along the side of stoma intestine herniates into intermuscular plane. (3) lntras-

tomal-bowel herniates between emerging and everted parts of the stoma. (4) Perstomal-herniation occurs between the layers of the prolapsed stoma. Features: Pain, features of obstruction, stomal malfunction, swelling which is tender, toxicity. Diagnosis: X-ray; CT abdomen. Treatment Surgery is indicated in recurrent obstruction, narrow neck, strangulation, interference with stoma. , Stoma is dissected, hernia content is reduced and defect is repaired. But it shows high recurrence. , Subcutaneous mesh repair as onlay placement after reduction of hernia. , Extraperitoneal mesh repair around the stoma. , lntraperitoneal mesh repair deep to peritoneum surrounding the stoma and defect.

B 1. 2. 3. 4. 5. 6. 7. 8. 9. 10. 11 . 12. 13. 14. 15. 16. 17. 18. 19. 20. 21. 22. 23. 24. 25.

DIFFERENT TYPES OF HERNIA

Gibbon's hernia-It is hernia with hydrocoele Berger's hernia- Hernia in pouch of Douglas Romberg hernia-Saddle hernia Obturator hernia-Hernia through obturator foramen (canal) Grynfelt's hernia-Upper lumbar triangle hernia Petit's hernia-Lower lumbar triangle hernia Femoral hernia-Hernia medial to femoral vein Cloquet's hernia-Hernia through pectineal fascia Narath's hernia-Behind femoral artery, in congenital dislocation of hip Hesselbach's hernia-Lateral to femoral artery Serofini's hernia- Behind femoral vessels Laugier's hernia-Through lacunar ligament Teale's hernia-In front of femoral vessels Richter's hernia-Part of circumference of bowel wall is gangrenous Littre's hernia-Hernia with Meckel's diverticulum as the content Sliding hernia- Posterior wall of the sac is formed by colon or bladder Maydl's hernia-'W' hernia Phantom hernia-Localised muscle bulge following muscular I paralysis Spigelian hernia-Hernia through spigelian fascia Mery's hernia- Perinea! hernia Sciatic hernia-Hernia through greater or lesser sciatic foramen Beclard's hernia- Femoral hernia through the saphenous 1 opening Barth's hernia- Hernia between abdominal wall and persistent vitellointestinal duct Holthouse's hernia-Inguinal hernia that has turned outwards / into the groin De Garengeot's hernia is incarcerated appendix in femoral hernia

The wind of anger blows out the lamp of intelligence.

J

775

...

:::z::: CD

=· r»

Chapter

Oesophagus ....

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

B

()HAPT E R OUTLIN E

.... ....

... ...

....

....

Anatomy Lower Oesophageal Sphincter Dysphagia Contrast Study of Oesophagus Oesophagoscopy Oesophageal Endosonography Third Space Endoscopy Gastro-oesophageal Reflux Disease Hiatus Hernia Rolling Hernia Reflux Oesophagitis Barrett's Oesophagus Barrett's Uleer Oesophageal Motility Disorders

..

Achalasia Cardia Plummer-Vinson Syndrome Corrosive Stricture of Oesophagus Schatzki's Rings Boerhaave's Syndrome Mallory-Weiss Syndrome Trachea-oesophageal Fistula Oesophageal Diverticulum Carcinoma Oesophagus Benign Tumours of the Oesophagus Oesophageal Perforation

ANATOMY Oesophagus is a hollow muscular tube which begins at the lower edge of the cricoid cartilage (C6 vertebra) and ends at oesophagogastric junction (T12 vertebra). It is 25 cm in length. Upper end is closed by cricopharyngeus muscle which is 18 cm from upper incisors. Lower end is 40 cm from the upper incisors (upper jaw is fixed and so is used as the landmark to measure, but not the lower jaw which is mobile). It lies anterior to vertebral column and posterior to the trachea. It lacks serosal layer but is surrounded by a layer of loose fibroareolar adventitia. It is lined by squamous epithelium throughout the length except the last 3 cm (OG junction) which is lined by columnar epithelium. Submucosa of the oesophagus is thick and strongest layer.

.

Lacks serosa (other structure without serosa is rectum). Contains 2 different types of muscles (striated and smooth at proximal 1/3 and distal 2/3 respectively) Contains 2 different types of epithelium. I Segmental blood supply. Only part of GIT which shows ve,y thinly scattered Meissner's plexus. Longitudinal arrangement of veins and lymphatics.

I

-

-----

---

I Parts 1. Cervical oesophagus: It extends from cricopharyngeus which is the horizontal part of inferior constrictor muscle. Upper oblique part is called as thyropharyngeus. Gap between the two is called as Killian's dehiscencewhich is a site of occurrence of pharyngeal pouch. Cervical oesophagus is related to trachea and recurrent laryngeal nerve. 2. Thoracic oesophagus: Lies initially towards the right side. In lower third, it deviates towards the left and continues as abdominal oesophagus. It is related to azygos vein, thoracic duct (which crosses the oesophagus posteriorly from right to left), aorta, pleura and pericardium. 3. Abdominal oesophagus is 2.5 cm long and grooves behind the left lobe of the liver.

Cervical constriction-occurs at the level of cricopharyngeal sphincter-narrowest point of G/T-15 cm from upper incisorsite of F/8 impaction Bronchoaortic constriction-located at the level of T4 25 cm from upper incisor-site of endoscopic perforation \ Diaphragmatic constriction-occurs whereoesophagus traverses the diaphragm (Level of T10)-40 cm from upper incisor

-- -- ---- ---- ------ --

J

Gap between upper oblique thyropharyngeus muscle and lower horizontal cricopharyngeus muscle (parts of inferior constrictor muscle) is called as "Killian's dehiscence" which is the site of occurrence of pharyngeal pouch. Laimer's/Lamier's) triangle is formed by cricopharyngeus above as base and on either side

divergent longitudinal oesophageal muscles. Laimer's triangle is in the posterior part of the oesophagus midline. Arterial supply of oesophagus By inferior thyroid artery, oesophageal branches of the aorta, gastric arteries and inferior phrenic arteries. Venous drainage of oesophagus By inferior thyroid vein, brachiocephalic vein, hemiazygos vein, azygos vein, coronary vein, splenic vein and inferior phrenic vein. Veins are longitudinal and they lie in submucosal plane in lower third and in muscular plane above.

Thoracic oesophagus drains into paraoesophageal and tracheobronchial nodes in thorax. Abdominal oesophagus drains into coeliac nodes.

777 n

Descending artery

:::c

Clavicle

SVC

>

m :::c

......

Azygos vein

ca:,

0

C'D

Horizontal artery

c:,)

0

-c ::r

I» IC

C

c:,)

Splenic vein Ascending artery

Left pulmonary artery

m

Figs. 19.2A and B: (A) Arterial supply and (B) Venous drainage of oesophagus.

1-11 are posterior intercostal veins

~ -,.iC-""-.....-;.,-- Accessory azygos vein " ' -__::::!!..,;'.--

Hemiazygos vein

Fig. 19.1: Anatomical relations of the oesophagus.

Lymphatic drainage Lymphatic arrangement in oesophagus is longitudinal and so spread of carcinoma to distant lymph nodes occurs early. Longitudinal lymphatics are 6 times more than transverse one There are more lymph vessels in submucosa than blood vessels. Lymph nodes are: Paraoesophageal groups located in the wall of the oesophagu s and are cervical, thoracic, paraoesophageal and paracardial nodes. Perioesophageal groups located immediately adjacent to oesophageal wall. They are deep cervical, scalene, paratracheal, mediastinal, diaphragmatic, gastric and coeliac lymph nodes. Lateral oesophageal groups receive lymph from para and perioesophageal lymph nodes. Upper oesophagus drains into supraclavicular nodes.

azygos vein Right ascending lumbar vein Right renal vein

Left ascending lumbar vein .___

_ Left renal vein

Fig. 19.3: Venous drainage of the oesophagus.

However, due to longitudinal arrangement of lymphatic plexus in the wall of oesophagus, any group of (neck/thorax/coeliac) lymph nodes can get involved in diseases at any level.

I Nerve Supply Oesophagus is innervated by vagus. It has got both sympathetic and parasympathetic innervation. It has got mainly Auerbach's plexus between longitudinal and circular muscle layers.

Don 't go through life, but grow through life.

DYSPHAGIA

----

...

a.,

5

1. Cervical group j 2. Lateral cervical group 3. Deep cervical group 4. Upper thoracic group 5. Paratracheal group 6. Subcarinal group 7. Hilar group 8. Middle thoracic group 9. Lower thoracic group 10. Posterior mediastinal (para-aortic) group 11. Diaphragmatic group 12. Paracardial group 13. Lesser curvature group 14. Coeliac left gastric group 15. Common hepatic group 16. Greater curvature group _J 17. Suprapyloric group

CJ'I

:::I Cl)

'E «I

:::I

C: n:,

:E _111

CICI

a: Cl)

I

6 8

9

12 14 15 17

Fig. 19.4: Lymphatic drainage of the oesophagus.

Leugart's pouch is prolapse of the wall of the oesophagus like a diverticulum over a band (Leugart's band) between the left bronchus and adjacent vertebra.

I Physiology Three types of contractions in the oesophagus: 1. Primary: Progressive, triggered by swallowing. 2. Secondary: Progressive, generated by distension or irritation. 3. Tertiary: Non prog ressive. Presbyoesophagus is less efficient oesophageal peristalsis. Note: Laimer's triangle is formed with base above formed by cricopharyngeus and on either side divergent longitudinal oesophageal muscles. Laimer's triangle is in the posterior aspect of the oesophagus in midline. • Collar of Helvetius is the site where oesophageal circular muscle fiber becomes oblique muscle of the stomach. •

LOWER OESOPHAGEAL SPHINCTER Lower oesophageal sphincter (LOS) is a high physiological pressure zone located in the lower end of the oesophagus in terminal 4 cm, with a resting pressure of 10-25 mm Hg. LOS prevents reflux of gastric and duodenal contents. It is influenced by food, gastric distension, gastric pathology, smoking, GI hormones, alcohol. Normally there is a transient relaxation period wherein reflux (physiologica~ occurs but then immediately gets cleared by oesophageal clearance mechanism. So pathological reflux or GORD can occur due to decreased LOS tone, altered relaxation time, reduced oesophageal clearance mechanism, or other altered mechanical factors. Oesophageal clearance mechanism is due to primary oesophageal peristalsis which carries saliva with high bicarbonate content which neutralizes and clears the transient physiological reflux. Manometry with special microtransducers are used to measure the LOS pressure.

Dysphagia is difficulty in swallowing. Painful swallowing is odynophagia. It can be acute---- '"Cl

m ::::a::J ...I,

(0

0 (D en 0

"Cl

I Treatment of Uncomplicated GORD

:::r I» cc

B

C

Operation should restore the LES pressllre twice the resting gastric pressure The adequate length of the intra-abdominal oesophagus should be maintained Apposition of diaphragmatic crurae, reduction of hiatus hernia when present Repaired OG junction should relax during swallowing adequately Tension free fundoplication should be done

A. General measure-Lifestyle changes , Control of obesity. Stop smoking and alcohol. Avoid tea, coffee and chocolate. Propped up position. Small frequent meals.

B. Drugs

H2 antagonists; antacids Proton pump inhibitors (PPls)-very effective - Omeprazole 20 mg-BD for 3-6 months. - Lansoprazole 30 mg. - Pantoprazole 40 mg. Esomeprazole 20 mg. - Rabeprazole 20 mg, llaprazole 10 mg. Prokinetic drugs - Metoclopramide, Domperidone 30 mg. - Cisapride, Mosapride. - ltopride 50 mg-does not cause cardiac arrhythmias. - Defoaming semethicone, alginic acid along with antacids. - Anticholinergic drugs-pirenzipine. - Mucosal protector drugs-sucralfate, colloid bismuth. C. Endoluminal therapies for GORD Endoluminal suturing (Wilson-Cook) Plexiglass microspheres (PMMA). Microsphere suspended in gelatin is injected through endoscopic needle. Gelatin gets absorbed and spheres cause a tissue bulking. Gatekeeper reflux repair system. Endoscopic delivery of preformed radiopaque hydrogel into submucosa. Stretta catheter. Flexible, soft, 6 mm sized, 65 cm length tube with balloon/basket and 5.5 mm NITI electrode, irrigation and suction fnte,yx injection technique (estrinyl vinyl alcohol) Endocinch technique Endoscopic full thickness plication. D. Surgery Indications for surgical treatment - Failure of drug treatment - Sliding hernia

I Fundoplication Nissen's posterior total fundop/ication: Here after narrowing the crus of diaphragm, mobilised posterior part of the fundus of the stomach is wrapped totally 360° around the area of OG junction. Diaphragm

Sutured fundus

-

'Y--'i---=

-

Partial plication

Figs. 19.9A and B: (A) Nissen's fundoplication; (B) Toupet's partial

fundoplication. Rudolph Nissen (1959) first did total fundoplication. Opening the peritoneum over the oesophagus to mobilise the lower end of oesophagus. Dissection of the diaphragmatic crura of the oesophagus. Mobilisation of entire fundus by ligating short gastric vessels. Vagi are preserved. In Nissen's, 60 French bougie wrap is ideal; after complete mobilisation, only posterior part of the tundus is wrapped

Attitude determines altitude.

en

784

s:-

... G)

Cl

:::s

Cl)

0

around after crural repair (otherwise fundoplication may is brought around oesophagus to suture into anterior part of displace into thorax causing paraoesophageal hernia); fundus. Here short gastric vessels are not ligated and much intra-abdominal length of 2 cm oesophagus is created less fundus is needed to be mobilised. (not more); fundus of the stomach is known to relax in Dor anterior tundoplication: Here right margin of the fund us is concert with oesophageal sphincter so only fund us should sutured to left margin of the oesophagus; front part of fund us be used to buttress the sphincter; wrapping should be is sutured to right margin of the oesophagus; 2nd row is also done only around sphincter oesophagus not around body sutured to right crus. of stomach; fundoplication should be kept in abdomen Watson's anterolateral fundoplication: 5 cm intraabdominal without under tension and without displacing into thorax. oesophagus is created with blunt transhiatal dissection. Nonabsorbable polypropylene sutu res are used. 120° anterolateral fundoplication augments the LOS function Crural repair is done using interrupted polypropylene during stomach distension. Bougie insertion, division of short gastric vessels are not needed here. sutures. Lind both anterior and posterior fundoplication of 300° with Fundoplication should prevent sphincter shortening/ unfolding during gastric distension, should preserve 60° anteriorly uncovered area is not routinely practiced now. Partial fundoplication with mesh wrap around is also used. But normal swallowing ability, allow proper belching and allow vomiting whenever needed. complications of mesh like erosion, stricture and adhesions are problems. Bougie; on table and postoperative manometry to assess the pressure of new LOS; on table and postoperative FUNDOPLICATIONS gastroscopy-are commonly used in fundoplication. Mortality of procedure is very less; other parts of the Nissen's-total 360° posterior fundoplication Toupet's-partial 180° posterior fundal/posterolateral abdomen can be assessed and addressed like gallstones. Rosetti Hell-total anterior fundal Complications are-gas bloat syndrome (inability to • Dor- anterior partial belch); dysphagia; inability to vomit; slippage, proximal • Watson's-,mterolateral 120° partial migration; paraoesophageal hernia, splenic injury. Floppy Lin~posterior and anterior Nissen's fundoplication reduces the incidence of gas bloat syndrome. Note: Other Procedures Laparoscopic Nissen's fundoplication is ideal and equally • Belsey Mark IV operation (1967): It is plication of oesophagus to the diaphragm through many interrupted mattress sutures so as to push successful. the oesophagus downwards to make it intra-abdominal with adequate Transthoracic approach is used for Nissen's fundoplicalength. It is done through left transthoracic approach. 240° fundoplication in patients who had hiatus hernia repair earlier; who tion, creation of intra-abdominal oesophagus, crural sling repair are the has short oesophagus; in patients who is having sliding techniques. It corrects GERO and also maintains normal eructation and hiatal hernia that does not reduce below the diaphragm, physiological reflux. But long hospital stay and respiratory complications associated pulmonary disease, in obese, in patients who are distressing problems. are having narrow subcostal angle or barrel-shaped chest. Toupet's partial 180° posterior fundoplicatiorr. It is similar to Nissen's posterior fundoplication; it controls reflux alike Nissen's but gas bloat is very less. It is commonly done procedure now. Short gastric vessels are divided completely or partially.

B

I row

II row

Anterior 213rd of fundal wrap

First row behind

Second row front, also to right crus

Fig . 19.10: Dor anterior fundoplication. Right margin of the fundus is sutured to left margin of the oesophagus. Front aspect of the fund us is sutured to right margin of the oesophagus. Second row is also sutured to right crus.

Rosetti Hell anterior fundoplication: Here anterior part of tundus of stomach is used. Superomedial part of the fund us

Fig. 19.11: Belsey mark 4 operation for sliding hiatus hernia. It is done through thoracic approach. Here angle of oesophagus is restored with sutures and partial anterior fundal wrap is done. Then oesophagus is pushed down by oesophago-diaphragmatic plication sutures.

• Hill's operation: Intra-abdominal fixation of OG junction (cardia) into median arcuate ligament to augment the effect of LOS, to enable the effective oesophageal peristalsis. Procedure is done by transabdominal approach which is assessed by intraoperative manometry. But it is technically difficult, may damage celiac plexus. • Placement of Angelchik prosthesis. Annular silicone gel filled angelchik prosthesis with a tape on either end is used. After insertion around OG junction ends are tied around. • Collis' vertical gastroplasty is done using fundus of stomach. Avertical cut along the line of oesophagus is made on thefundus which is sutured to create extra length of oesophagus and new lesser curvature. It is done whenever oesophagus cannot be mobilised due to oesophageal shortening by a stricture developed as a complication of GORD. Partial fundoplication is added to this usually.

• Boerema operation: It is fixing OG junction in front into anterior abdominal wall. It is not practiced now. • Oesophagogastrectomyis required whenever there is failure of antireflux procedure/metaplasia is present/ nondilatable stricture is present. Lower end of oesophagus and proximal stomach is resected with oesophagogastric anastomosis is done through thoracoabdominal approach. • Transhiatal oesophagectomy with anastomosis between cervical oesophagus and stomach in the neck is an alternate procedure which can be used in severe extensive disease and stricture. • Allison's procedure: It is transthoracic repair of GE junction to replace the GE junction in its normal intra-abdominal location is one of the earliest procedures done with symptomatic relier but 80% recurrence rate; hence it is not done. • Stricture oesophagus due to GERO which is dilatable can be treated by regular oesophageal dilatation and long-term PPI therapy.

Diaphragm t----,,.......""""--:::,.... Gastroplasty

incision

Oesophageal/gastric perforation; Haemorrhage. Pneumothorax/pyothorax. Vagus nerve injury; Cardiac arrhythmias. Sepsis-mediastinitis or septicaemia. Disruption/failure of fundoplication. Gas bloat syndrome.

It is the most common type of diaphragmatic hernia.

•

That's patch procedure. It is done tor localised nondilatable stricture oesophagus. Stricture is incised full thickness longitudinally. Mobilised rundus or stomach is placed and sutured as a serosal patch technique over the defect.

B Type/ hiatus hernia: It is the cephalad displacement of the gastrooesophageal junction through the hiatus into the mediastinum. It is usually small, asymptomatic and reducible. It is commonest type Type II hiatus hernia: It is superior migration of the fu ndus of the stomach along side the GE junction and oesophagus into the mediastinum with GEjunction in normal intra-abdominal location. It is rolling hernia Type Ill hiatus hernia: It is combination of type I and type II Type /Vhiatus hernia: It is the hernia containing other abdominal viscera as content like transverse colon and omentum '----

I Types Sliding hernia (85%); Rolling hernia (10-12%); Combined.

Fig. 19.13: Thal's patch done for oesophageal stricture due to reflux oesophagitis. • Narbona's ligamentum teres cardiopexy (commonly done in Spain): Ligamentum teres is mobilised from falciform ligament with its blood supply from left lobe of liver; it is detached from umbilicus and brought around the mobilised lower oesophagus with traction until LOS pressure becomes 15-20 mmHg with manometry; flap end is sutured left of OG junction and anterior wall of stomach. It is more physiological and division of short gastric vessels is not required.

Sliding

Rolling

Combined

Fig. 19.14: Different types of hiatus hernia.

Kindness is a language which the dumb can speak, the deaf can understand.

C'D

Cl)

0

-0

:::r I»

Cl)

HIATUS HERNIA

Fig. 19.12: Vertical gastroplasty is done to lengthen the intraabdominal oesophagus.

0

IC C

I Complications of Surgery

Oesophagus

785

j

786

B Gangrene of stomach; Gastric volvulus. Perforation into the mediastinum. Perforation into the peritoneum. !schaemic longitudinal ulcer in the herniated stomach in rolling ~ ia is called as Cameron ulcer.

I

-

1

0

«I

:::I

C «I

:E: _V>

m

fJ

Figs. 19.15A and B: Endoscopic view of oesophageal hiatus hernia.

ca

cc

en

Sliding hernia is commonly associated with GORD (should be discussed like GORD). Here the cardia migrates back and forth between the posterior mediastinum and peritoneal cavity.

B

T

Investigations: Plain X-ray-lateral and PA erect view showing retrocardiac air-fluid level; Barium meal study very useful; ECG; 30 CT scan is useful. Treatment: Treatment is always surgical. Excision of sac and repair of the defect. If it is gangrenous, gastrectomy is required. Either abdominal or thoracic or laparoscopic approach can be used in treating rolling hernia surgically. Mesh reinforcement to hiatus to close the defect may be needed.

REFLUX OESOPHAGITIS Grading of hiatus hernia Grade

Type

I-HO II-H1 III-H2 IV·H3 V-H4

Normal Sliding Rolling Mixed Massive herniation

I Types 1. Acute: Following burns, trauma, infection, peptic ulcer. 2. Chronic-. Reflux of acid in sliding hernia, after gastric surgery.

Reflux is quite common in pregnancy. Site is always in lower oesophagus.

I Pathology There is bleeding granulation tissue in lower oesophageal mucosa with spasm of longitudinal muscle which pulls the adjacent gastric area upwards into the oesophagus causing sliding hernia.

B Sliding

hernia

Rolling

Mucosa! erythema Mucosa! erythema + superficial ulceration Mucosa! erythema + superficial ulceration + submucosal fibrosis Mucosa! erythema + extensive ulceration + paramural fibrosis

hernia

Fig . 19.16: Types of hiatus hernia.

ROLLING HERNIA (PARAOESOPHAGEAL HERNIA) It is herniation of stomach fundus or rarely other abdominal contents colon/spleen through a hiatus, usually towards left side.

I Features Common in elderly. Abdominal pain and chest pain; Hiccough, early satiety. Regurgitation, postprandial bloating; Dysphagia, dyspnoea. Cardiac abnormality (arrhythmia). 40% presents as acute features with perforation/gangrene/ bleeding.

B

SAVARY-MILLER CLASSIFICATION OF REFLUX OESOPHAGITIS-GRADING

1. Single or isolated erosive lesion(s), oval or linear but affecting only one longitudinal folds 2. Multiple erosive lesions, noncircumferential affecting more than one longitudinal fold with or without confluence 3. Circumferential erosive lesions 4. Chronic lesion: Ulcers, strictures and or short oesophagus alone or in association with grade 1-3 lesions Columnar epithelium in continuity with the Z-line, non-circular, star shaped or circumferential, alone or in association with grade 1-4 lesions

t

A. B. C. D.

LOS ANGELES CLASSIFICATION OF REFLUX OESOPHAGITIS- GRADING One or more mucosal break no longer than 5 mm that does not extend between the tops of two mucosal folds One or more mucosal break more than 5 mm long that does not extend between the tops of two mucosal folds One or more mucosal break that continues between the tops of two or more mucosal folds but which involves less than 75% of the circumference One or more mucosal break, which involves at least 75% of the oesophageal circumference

I Features It is a part of GORD. Pain and burning sensation in retrosternal area often referred to shoulder, neck, arm. Heart burn is common; Dysphagia; Anaemia. Diagnosis: Barium meal X-ray; Gastroscopy and biopsy. Barrett's ulcer is an ulcer with gastric (col umnar) metaplasia in lower oesophagus. Treatment: Antacids; H2 blockers: Ranitidine, famotidine. Proton pump inhibitors-Main method and more effective. - Omeprazole 20 mg BO one hour before food (Morning) for 6 months; Lansoprazole 30 mg; Pantoprazole 40 mg; Esomeprazole 20 mg; Rabeprazole 20 mg (can be given with food). Prokinetic drugs like metochlopramide, domperidone, cisapride, mosapride. Treating GORD and associated causes. By fundoplication and other surgeries. Resection in severe cases. Note:

Erythromycin is a prokinetic drug (motilin agonist), which acts by binding with motilin receptor on GI smooth muscle cells.

BARRETT'S OESOPHAGUS (Norman Barrett, British, 1950) It is the metaplastic changes in the mucosa of the oesophagus as the result of GORD. Squamous epithelium of lower end of the oesophagus is replaced by diseased columnar epithelium (columnar metaplasia). There is macroscopic visible length of columnar mucosa with microscopic features of intestinal metaplasia. It commonly affects lower oesophagus but often middle oesophagus also. There is proximally displaced squamocolumnar Junction on endoscopy with acid- mucin containing goblet cells that is specialized intestinal metaplasia. There is presence of columnar epithelium lined oesophagus (GELO) with intestinal metaplasia on histology.

••• Columnar epithelium should be visible endoscopically (minimum of 1cm) above the GEJ to diagnose as Barett's oesophgus. Upper end of gastric folds is more su itable landmark. In the Australian guidelines, a diagnosis of Barrett's oesophagus requires two components- (1) Endoscopic evidence of a salmon-pink coloured columnar epithelium extending above the GE jn, partially replacing the normal tubular oesophageal squamous epithelium; (2) Biopsies from the oesophageal columnar epithelium will show the evidence of intestinal metaplasia, with the presence of mucin containing goblet cells.

I Types (Based on Length) a. If the length of metaplasia is more than 3 cm, it is called as long segment Barrett's oesophagus- classic Barrett type. b. If the length is less than 3 cm, it is called as short segment Barrett's oesophagus.

I Histological Types a. Gastric type: Contains chief and parietal cells. b. Intestinal type: Contains goblet cells. c. Junctional type: Contains mucous glands alike of gastric cardia. Cardia metaplasia is metaplasia at OG junction without any macroscopic change in gastroscopy. This diseased columnar epithelium is more prone for malignant transformation, i.e. when there is intestinal metaplasia, risk of malignant transformation increases (40 times more than general population). More the amount of dysplasia, more is the risk of malignant transformation. Dysplasia may be indefinite; low grade or high grade.

I Features Features of GERO, haematemesis, dysphagia. Common in men; common in whites. PRAGUE endoscopic criteria is used to diagnose based on proximal circumferential extent of columnar metaplasia (C), maximum extent of columnar metaplasia (M) and gastrooesohageal junction length (GE jn). Targeted biopsies of visible lesions then random biopsies proximally at 4 quadrants are done. Barrett's oesophagus >3 cm needs repeat endoscopies regularly. Complications of Barrett's oesophagus: Ulcerations and stricture; Dysphagia; Bleeding; Perforation; Adenocarcinoma of 0-G junction (25 times more common)

I Management Regular endoscopic biopsy and surveillance for low-grade dysplasia. Ablation of Barrett's oesophagus by laser/radiofrequency. Photodynamic therapy- through endoscopy.

Never forget that only dead fish swims with the stream.

787

C")

::c

>

"tJ

-i

m

:::D ...i.

co 0

Cl)

"' 0 -0

-:::r-

1»

IC C

"'

c:-

... QJ

Cl :::,

-

Argon beam coagulation. Proton pump inhibitors-high dose for 3-6 months. Antireflux treatment by surgery. Resection-Always better choic~for high-grade dysplasia. Transhiatal oesophagectomy is preferred. Endoscopic mucosal resection is also good.

Cl)

0

:::, C

:::

en

r:a

cc

Cl)

BARRETT'S ULCER It is an ulcer in columnar epithelium lined Barrett's oesophagus at or just above the squamocolumnar junction. It is more prone for: Bleeding; Perforation; Adenocarcinoma of oesophagus-very high >40 times. Treatment for Barrett's ulcer is endoscopic biopsy and resection.

OESOPHAGEAL MOTILITY DISORDERS

I Primary

Achalasia, vigorous achalasia. Diffuse and segmental oesophageal spasm: It is 5 times less common than achalasia, mainly primary spasm of the oesophageal body, predominantly presenting only as chest pain and less severe dysphagia. There is hypertrophy of oesophageal wall muscle with degeneration of vagal fibers; Simu ltaneous multipeak waveforms of high amplitude and long duration with 20 or more simultaneous waveforms out of 1Owet swallows is diagnostic. Often it can be segmental or involve distal 213rd of oesophagus. LES resting pressure is normal with normal deglutition relaxation time. Barium swallow shows corkscrew pattern . Treated with nitrates, calcium channel blockers, bougie dilatation, long oesophageal myotomy either video assisted or thoracotomy approach. Nutcracker oesophagus (supersqueeze oesophagus): Common hypermotility disorder with high am plitude peristalsis, equal in both sexes. Severe chest pain (noncardiac), dysphagia without regurgitation, odynophagia are the features. Waves of hypertensive amplitude pressure >180 mm Hg often can become very high >400 mmHg; long duration contraction waves >6 seconds with normal LES pressure and LES relaxation. It is treated with nifedipine, nitrates, antispasmodics, occasionally long myotomy. Hypertensive LES: LES pressure is above normal (>26 mm Hg). LES relaxation, amplitude pressure, contraction waves,

Nonspecific oesophageal motility disorders: It shows slow transit incomplete emptying, with normal or hypertensive LES pressure, LES shows incomplete relaxation, decreased amplitude pressure 20% nontransmitted , prolonged waves of >6 secs with abnormal peristalsis. Dysphagia, chest pain reflux and regurgitations are the features .

I Secondary Neurological-stroke, bulbar palsy, motor neuron disease, multiple sclerosis, Parkinson's disease, poliomyelitis. Muscular-myasthenia gravis, muscular dystrophy, dermatomyositis. Autoimmune disorders- systemic sclerosis, polymyositis, SLE, CREST syndrome, scleroderma. Eosinophilic allergic oesophagitis, alcoholic neuropathy. Endocrine and metastatic diseases.

ACHALASIA CARDIA (Cardiospasm) The symptom-complex in cardiospasm (the term by which oesophageal achalasia was formerly known) is as a rule almost pathognomonic. It may be divided into the three stages-first, cardiospasm without food regurgitation; second, cardiospasm with immediate food regurgitation; third, cardiospasm with dilated oesophagus, the retention of food in the dilated portion and its regurgitation at irregular intervals after taking. - Henry Stanley Plummer, 1908

I

It is failure of relaxation of cardia (oesophagogastric junction) due to disorganised oesophageal peristalsis, as a result of failure of integration of parasympathetic impulses causing functional obstruction (Achalasia means failure to relax-Greek). It is first identified by Thomas Willis in 1672.

Normal

oesophageal body peristalsis are normal. Botulinum, balloon

dilatation, modified Heller's myotomy are the treatment. Ineffective oesophageal motility disorders: It is irreversible contraction abnormality of distal oesophagus in association with GORD. It is often due to secondary inflammation of oesophageal body following more exposure to gastric contents and poor oesophageal acid clearance. Reflux, dysphagia, heartburn, chest pain, are the features. Manometry shows-sum of total number of low amplitude contractions of less than 30 mmHg and nontransmitted contractions exceeds 30% of wet swallows.

Fig. 19.17: Diagrammatic representation of achalasia cardia.

I Aetiology There is absence or less numbered ganglions in myenteric plexus. Stress; Vitamin B1 deficiency. Chaga's disease. It is caused by Trypanosoma cruzi, which is common in South America called as sleeping sickness.

Diffuse oesophageal spasm (Corkscrew oesophagus). Most commonly it is idiopathic. There is degeneration of Auerbach's myenteric plexus along the entire length of oesophagus more so in LOS. There is pencil-shaped narrowing of cardia (0-G junction) with enormous dilatation of proximal oesophagus, which contains foul smelling fluid and is more prone for aspiration pneumonia. Achalasia cardia is a precancerous condition-seven times chances of getting squamous cell carcinoma (8%, after 15 years).

Note: • Clinical scoring of achalasia cardia are: O No weight loss or dysphagia or retrosternal pain or regurgitation. 1 Weight loss< 5 kg; occasional dysphagia, retrosternal pain, regurgita· lion. 2 Weight loss 5 -10 kg; daily dysphagia, retrosternal pain, regurgitation. 3 Weight loss >1Okg; dysphagia and regurgitation during each meal; retrosternal pain several times a day. • Achalasia can be classic or vigorous.

I Investigations

,..

I Pathology

Barium swallow is diagnostio-shows.

Thickening of circular muscle fibres in distal oesophagus. Myenteric inflammation; depletion of ganglion cells; neural fibrosis, reduced nitric oxide and VIP (mediators of LES relaxation). Absence of peristalsis; raised LES pressure; failure of relaxation with functional obstruction of OG junction. Dilatation of proximal oesophagus with atony.

----,-------

I Features Common in females between 20 and 40 years age group. Incidence is 6 per 1, 00,000 population. Chest pain occurs in early stage. Achalasia with diffuse oesophageal spasm is called as ' vigorous achalasia' (Achalasia with high amplitude contractions). Presents with progressive dysphagia, which is more for liquid than to solid food. Regurgitation and recurrent pneumonia are common (60%). Walking while eating, chin thrusting, neck and shoulder extension, Valsalva manoeuvre facilitates emptying of food from the oesophagus. Heartburn (50%) is common. Chest pain during meal is common. Malnutrition and general ill health. Lung abscess formation. Odynophagia and weight loss. Recurrent chest infection with nocturnal cough, laryngospasm. Excess stale food in the oesophagus, oesophageal candidiasis and halitosis is common.

Figs. 19.18A and B: (A) Barium swallow X-ray showing features of achalasia cardia; (B) Endoscopic view of achalasia.

I

I

Pencil-like smooth narrowing of lower oesophagus-Bird beak appearance Dilatation of proximal oesophagus-cucumber oesophagus Absence of fundic gas bubble Sigmoid oesophagus or megaoesophagus

TRIAD Dysphagia; Regurgitation; Weight loss

B

Fig. 19.19: Sigmoid oesophagus in achalasia cardia-Barium X-ray and endoscopic view.

Chest X-ray shows patches of pneumonia. Double mediastinaf strip of dilated oesophagus is typical with air fluid level in posterior mediastinum on lateral view. Self-respect - that a cornerstone of all virtue.

("')

::c

>

'"'C --4

m

::x, (,Cl

790

Oesophageal manometry shows unrelaxed lower oesophageal sphincter with high resting pressure-very useful and gold standard. It shows failure of LES to relax completely during swallowing and complete absence of peristalsis. LES pressure is >35 mmHg. Baseline oesophageal pressure will be high without progressive oesophageal peristalsis with low amplitude muscular tone. lntraoesophageal pressure in relation to intragastric pressu re is elevated. Oesophagoscopy is done to confirm the diagnosis and to rule out carcinoma oesophagus. It shows totally closed LES (rosette like) with atonic, dilated proximal oesophagus. Biopsy of mucosa at LES should be done.

I Differential Diagnosis Carcinoma oesophagus. Stricture oesophagus. Scleroderma.

Either through thoracic or through abdominal approach, thickened circular muscle fibres are cut longitudinally for about 8-10 cm, 2 cm proximal to the thickened muscle to 1 cm distal to OG junction. Care should be taken not to open the mucosa (anterior myotomy is done now. Original Heller's is both anterior and posterior myotomies). Nissen's or Toupet's or Dar's fundoplication is done along with the above procedure to prevent reflux. >- Laparoscopic/thoracoscopic cardiomyotomy-ideal. Robotic-assisted laparoscopic cardiomyotomyimproves the dexterity and three-dimensional vision. Resection is done when failure of myotomy occurs or when megaoesophagus or metaplasia is present. Transhiatal total oesophagectomy with gastric pull up and oesophagogastric anastomosis in the neck is a good option in such patients.

I Treatment B

-

TREATMENT FOR ACHALASIA CAROIA

Surgery: Modified Heller's cardiomyotomy with Toupet's / Dor's fundoplication _ Open abdominal/thoracic (rare) approach - Laparoscopic approach- ideal _ Robotic-assisted laparoscopic approach Resection of OG junction/transhiatal total oesophagectomy in severe cases-megaoesophagus/metaplasia POEM (Per Oral Endoscopic Myotomy) Forcible dilatation: Plummer's pneumatic dilatation Negus hydrostatic balloon dilatation Drugs: Botulinum toxin Nitroglycerin, nifedipine

Surgery Modified Heller's operation (Heller-German , 1913): Oesophagocardiomyotomy. Success rate is 85%.

Figs. 19.21A and B: Laparoscopic port positions and view of Helller's cardiomyotomy for achalasia cardia (Courtesy: Dr Roshan, MS DNB, Laparoscopic Surgeon, City Hospital, Mangaluru) .

POEM (Per Oral Endoscopic Myotomy) Here using endoscope, methylene blue diluted saline is injected into the submucosa of the oesophagus 15 cm proximal to OG junction ; mucosa! incision of 2 cm is made proximally to enter submucosal space; adequate tunnel in the space is created ; small bleeders are controlled by specialized cautery device; LES myotomy is done using special electric endoknife. Mucosa is closed using clips. It is a novel seemingly effective technique.

Forcible Dilatation

Figs. 19.20A and B: (A) Heller's cardiomyotomy for achalasia cardia. Only circular muscle layer is cut longitudinally in OG junction until mucosa protrudes out without perforating the mucosa; (B) Heller's cardiomyotomy-on table look with partial fundoplication (Courtesy: Dr Yogishkumar, Professor of Surgery, KMC, Mangaluru).

It stretches spasmodic segment. Gradual repeated dilatations are required. Dilatation up to 54 French bougie can be done. Two types of dilatations are used- pneumatic and hydrostatic. Plummer's pneumatic dilatation is done using balloons of 30-40 mm diameters. It is inserted over a guidewire. Risk of perforation (5%), need for repeated dilatations are the problems. Negus hydrostatic dilatation is done to dilate O-G junction. It is not very well-accepted method as chances of perforation is high. Success rate is 65%. 30 mm diameter balloon is inflated for 3 minutes. Self expanding metal stents (SEMS) are also tried.

Oesophagealnegus - --t-tube

Achalasia segment - ---

Fig. 19.22: Negus hydrostatic dilator used for achalasia cardia. It has got only 65% success rate.

Drugs

a. Endoscopic injection of botulinum toxin to sphincter-high recurrence rate. b. Calcium channel blocker, nitroglycerin sublingually; Sildenafil Note:

• Pseudoachatasia shows features like achalasia cardia with dysphagia and weight loss, seen in an elderly due to carcinoma. Amylnitrate inhalation causes sphincter relaxation in Achalasia cardia but not in pseudoachalasia. • Botulinum toxin is neurotoxic protein derived from Clostridium botulinum is highly toxic poisonous substance. Very small dose is used for therapeutic purpose. Seven types of toxins are found. A (A1 /A2 /A3) type is used for therapy. It blocks the cholinergic nerve ends reducing the cholinergic acetylcholine release causi ng flaccid paralysis of muscles. It is used in cosmetic facial line, strabismus, focal dystonia, tremor, tics, muscle spasms, achalasia, smooth muscle hyperactivity, Frey syndrome, and hyperhidrosis.

PLUMMER-VINSON SYNDROME (Paterson-Kelly Syndrome)

,:. Here oesophageal webs are seen in uppermost portion of the oesophagus with spasm of circular muscle fibres. Common in patients with long-standing iron deficiency anaemia. Common in females. Superficial glossitis, cheilitis, koilonychia commonly seen. ,:. Splenomegaly may be present. ,:. In oesophageal webs, mucosa is hyperkeratotic, friable, desquamated. It is a premalignant condition and presents with severe dysphagia. Oesophagoscopy and biopsy is required to rule out malignancy. Treatment: Oral iron-ferrous sulphate 300 mg TDS with vitamins. Blood transfusion is given when there is severe anaemia (Transfusion of packed cells). IV or IM iron therapy. Once anaemia comes under control, webs will clear and patient can swallow. Follow-up endoscopy is a must. Dilatation of web may be required.

CORROSIVE STRICTURE OF OESOPHAGUS

B

I

!

:

I

Acute/immediate -

Severe pain, shock, laryngeal oedema Mediastinitis, septicaemia, haemorrhage, perforation

Late/chronic - Dysphagia; Stricture-SO%; Malignant changes - Severe malnutrition; Recurrent respiratory infection - Oesophageal shortening - Tracheo-oesophageal fistula formation

I

Corrosive strictures can be multiple. Damage is more in lower 113rd

Iof oesophagus

Corrosives are commonest cause of oesophageal stricture. Mainly due to ingestion of alkali (Lye strictur~Lye is strong alkali sodium hydroxide) sodium hydroxide, occasionally due to acid (sulphuric acid, nitric acid). Acid commonly damages the stomach. It causes extensive inflammation of the mucosa with perioesophagitis which, if not treated, leads to multiple strictures in oesophagus. Sometimes it causes severe life-threatening necrotising lesion which requires immediate surgical intervention. Acute phase lasts for 3 weeks. Damage is more in lower 113rd of oesophagus. Alkali is odourless and tasteless and so large volume is ingested. Alkali causes liquefaction, saponification and throm-

Fig. 19.23: Oesophageal web, endoscopic view.

bosis of vessels and later leading to fibrosis and stricture. Acid causes intense pylorospasm, antral pooling of acid, coagulation necrosis and eschar formation. Severity depends on type of agent, its concentration and volume.

Happiness comes more from loving than being loved. Happiness is not a destination; it is a method of life and is a way of travel.

791

0

CD

"' 0 "Cl

:::r 0,)

IC

C:

"'

792

co

= C co en

Pha11s of tissue injury In CO"OSillB ingestion

Degrees of bums affer corrosive oesophageal and gastric bums

Phase 1: Acute necrosis-1--4 days Phase 2. Ulcerationgranulation-4-12 days Phase 3'. Cicatrisalion and scarring-3 weeks to 6 months

1st degree-. Mucosa! hyperaemia and oedema 2nd degree: Small bleeding, exudates, ulcers, pseudomembrane 3rd degree-. Mucosa! slough, deep ulcers, massive bleed, complete obstruction, charring, perforation

,.

CICI

a:

Cl.)

stomach. Dilatation should be done up to minimum 16 mm diameter. Pneumatic or balloon dilatation is also practiced. Gum elastic dilators, mercury weighted dilators, Eder-Puestow dilators, Savary-Gilliard dilators, balloon dilators are other dilators used. Earlier, blind dilatation using oesophageal bougies of increased diameters was the practice, which is followed even now in many places, but chances of perforation is more. Oesophageal resection in corrosive strictures is technically difficult and may be hazardous. Oesophageal bypass is better and easier, and following later by regular endoscopic surveillance for malignant transformation (5%). Colon is used as replacing conduit as stomach itself may be diseased in corrosive pathology. In multiple strictures oesophageal resection and colonic transposition may be advocated if risk of malignancy is considered . Malignancy can develop in corrosive strictures- 5%. II is 1000- fold greater than general population.

b.. Figs. 19.24A and B: Endoscopic view of corrosive injury. Endoscopy

should be done very gently in corrosive injury.

I Treatment Acute phase management Neutralisation with vinegar or citrus food if it is alkali ingestion (If pH of the solution is less than 11.5, then damage is less); it is with antacids, milk, egg whites if it is acid ingestion. Early endoscopy is needed to assess the severity and extent. Emetics and NaHCO3 are avoided as they may precipitate perforation. In 1st degree burns: 48 hours observation; oral feeds are started once patient swallows saliva painlessly. Regular follow-up endoscopy at 1st, 2nd and 8th months. Stricture, if formed , can be identified by this time. 2nd and 3rd degree burns: They are treated with fluid therapy, antibiotics, nutrition, resuscitation, PPls, aerosolised steroids, fiberoptic-guided ai rway intubation if needed/tracheostomy; endoscopic oesophageal stenting, feeding jejunostomy, laparoscopy for evaluation. Unstable patients have high mortality. Laparotomy is done in such patients. If oesophagus and stomach shows full thickness necrosis, resection of these parts is done and end cervical oesophagostomy with jejunostomy is done. When in doubt, re-exploration for second look is done after 36-48 hours to assess the stomach. Careful early gentle repeated endoscopy is mandatory. Though advocated often for 2- 3 weeks, use of steroids is controversial and under debate. Antibiotics if there are chances of aspiration or perforation. Regular oesophageal dilatation is done for stricture. Stricture is dilated endoscopically using guidewire. Dilators are solid type with gradual increase in diameters. Often radiologic C-arm guidance is needed to pass the guidewire into the

Peptic stricture (oesophagitis induced) Corrosives-most common cause Foreign body Postsurgical, radiotherapy Congenital Infection liketuberculosis Drugs like tetracycline, vitamin C

Figs. 19.26A and B: (A) Barium swallow showing strictu re at lower oesophagus. It may be due to oesophagitis, congenital, tuberculosis, malignancy, drug induced, etc.; (B) Patient underwent total oesophagectomy with colonic transposition. Colon placed in subcutaneous plane.

793

Treatment:

SCHATZKl'S RINGS They are semicircular protrusion of lower oesophageal mucosa located at or just above the oesophagogastric junction (squamocolumnar junction). Its undersurface is lined by columnar gastric epithelium. They involve only the mucosa and submucosa of the oesophagus, not the muscle. They present with dysphagia and reflux. Episodic aphagia can occur causing food bolus or meat bone to get impacted which requires emergency rigid oesophagoscopy to remove the food. 5 ml of 2.5% oral papain every 30 minutes to digest food protein along with 50 mg meperidine IV to dislodge the impacted food bolus can be tried initially. Treatment: Intermittent oesophageal bougienage; Antireflux drugs. Note: Ring should not be excised.

Nil by mouth; Antibiotics, IV fluids, TPN; Feeding by jejunostomy. Most often surgery with resection may be required (thoracotomy and repair)-left thoracotomy is better. When severe mediastinitis occurs, condition has high mortality. Superficial tear with bleed 1n stomach near O-G junction

Deep tear with perforation in oesophagus lower 1/3rd - - - I.a

a, Cl.I 0

"Cl

:::r

Al IC

= Cl.I

rJ

m

Figs. 19.28A and B: Diagrams showing: (A) Mallory-Weiss syndrome and (8) Boerhaave's syndrome.

MALLORY-WEISS SYNDROME

Figs. 19.27A and B: (A) Barium swallow X-ray showing Schatzki ring, also shows features of hiatus hernia; (B) Schatzki's ring-endoscopic view.

BOERHAAVE'S SYNDROME It is a tear in the lower third of the oesophagus which occurs when a person vomits against a closed glottis, causing leak into the mediastinum, pleural cavity and peritoneum (Refer Table).

T

It is seen in adults with severe prolonged vomiting, causing longitudinal tear in the mucosa of stomach at and just below the cardia, leading to severe haematemesis. Violent vomiting often may be due to migraine or vertigo or following a bout of alcohol. Presents with severe vomiting and later haematemesis, with features of shock. It is common in 1 o'clock position. Only 10% of cases involve lower oesophageal mucosa.

I Investigations Gastroscopy, Hb%, PCV, blood grouping. During gastroscopy, if stomach is not inflated properly, 50% cases may be missed.

Features of Boerhaave's syndrome '

Presentations Diffsrential diagnosis Complications Sudden onset of Myocardial infarction Mediastinitis symptoms Severe chest paln Pancreatitis Septicaemia Pain abdomen Peritonitis Subcutaneous Emphysema emphysema Shock 'Crunching effect in the chest' is called as Hamman's sign. Mack/er's triad: (1) vomiting, (2) chest pain, (3) subcutaneousemphysema.

Site: 2-10 cm of posterolateral part of lower oesophagus. Investigations: Chest X-ray-shows pneumomediastinum ('V' sign of Naclerio); MRI/CT chest; Total count.

0

Treatment Conservative, as it is only a mucosa! tear. Blood transfusion; IV fluids; Sedation. Haemostatic agents like vasopressin. Endoscopic injection therapy is used if required. Surgery is rarely required.

TRACHEO-OESOPHAGEAL FISTULA (OESOPHAGEAL ATRESIA)

I Types (Refer Figure 19.28)

In 85% cases, it is a blind upper end with lower end communicating with trachea.

All are equal in their ignorance.

794

Type A

- 8.5%

Type B

- 1%

Type C

Type D

-85%

- 1.5%

Type E

- 4% Fig. 19.29: Gross classification of TOF- Type A: Atresia without fistula - 8.5%. Type B: Atresia with proximal TOF-15. Type C: Atresia with distal TOF- 85% (commonest). Type D: Atresia with both proximal and distal TOF -1 .5%. Type E: No atresia but 'H' type TOF - 4%.

It may be associated with VACTER anomalies. It is a spectrum of anomalies which shares a defect of the oesophageal continuity with or without a fistula to the trachea or bronchi. V A C TE R

-

Complications of surgery: Pneumonia; Leak from the anastomotic site; Reflux; Dysphagia

OESOPHAGEAL DIVERTICULUM

Vertebral defects Anal atresia Cardiac defect (POANSO) Tracheo-oesophageal fistula Radial hypoplasia and renal agenesis

1

I Features Incidence is 1 in 4,500 births; common in males (3:1 ). TOF should be recognised within 24 hours of birth. Newborn baby regurgitates all feeds and there is continuous pouring of saliva from the mouth which is a diagnostic feature. Cough and cyanosis. It is com monly associated with maternal hydramnios (50%). Investigations Obstruction is revealed while passing nasogastric tube. Contrast study will reveal fistula and obstruction (Dionosil 1 ml ). Other anomalies are looked for. Chest X-ray. Echocardiography. Treatment Surgery Child requires feeding gastrostomy commonly. Often the procedure is staged one. Through right-sided thoracotomy (opposite to the side of aortic arch), fistula is identified and resected. Lower segment is anastomosed to the blind upper segment. Occasionally if the length is inadequate or the atretic segment is long one, then colonic or gastric transposition is required.

Fig. 19.30: Endoscopic view of oesophageal diverticulum.

I Types 1. Pulsion diverUcu/um: Pulsion diverticula are false type containing mucosa and submucosa only; is due to high abnormal intraluminal oesophageal pressure developed due to various motility diso rders. a. Pharyngeal pouch through Zenker's or Killian's dehiscence (Refer chapter Neck) . b. Epiphrenic pu/sion diverticulum occurs in lower oesophagus, usually towards right side, due to obstruction in the distal oesophagus or due to incoordinated LOS relaxation. - Site is within 10 cm of OG junction. It is false type. - It is associated with nonspecific oesophageal motility disorders and often with achalasia and diffuse oesophageal spasm. Ehler Danlos syndrome and trauma are the other causes.

- It is common on right side with wide mouth. - Features are of motility disorders like dysphagia, regurgitation, cough, weight loss, chest pain. - Barium study, CT chest are diag nostic; endoscopic evaluation with EUS, manometry is a must. - Treatment: Diverticulopexy/diverticulectomy (excision) + oesophageal myotomy (Heller's) + repair of associated hiatus hernia/antireflux procedure. 2. Traction diverticu/um: Occurs in mid-oesophagus or in parabronchial region, is due to mediastinal granulomatous disease like tuberculosis. Traction diverticulum is a true type containing all layers in its wall and is due to traction by the healing fibrosing mediastinal lymph nodes. It is seen commonly towards right side. It has got wide mouth and it rests on the spine. Presentation is dysphagia, chest pain and regu rgitation. CT scan (chest), barium study, manometry, endoscopy to assess mucosa with EUS, blood test for tubercu losis (ESR, peripheral smear) are the investigations. Treatment: Treating the cause like tuberculosis, histoplasmosis. Diverticulum less than 2 cm is observed; progressive symptoms, size >2 cm needs surgery. Surgeries are- diverticulopexy, long oesophageal myotomy.

795

B

:j

Autosomal dominant condition seen from childhood Soles and palms are involved called as palmoplantar keratoderma Waxy, yellow lesions, which do not itch 60% of members of families develop carcinoma oesophagus after the age of 60 Systemic retinoids are the drugs used for tylosis '--

I Aetiology IDiet, deficiencies (vii. A, C, riboflavin)

5% common Mycotoxin Common after 45 years Alcohol and tobacco Common in men Fungal contamination of food Common in China-Henan province In India, common in Odisha and Karnataka Human papilloma virus (HPV 16,18) Geotrichum candidum fungi in China Achalasia cardia 30% Oesophageal webs 25% Barrett's oesophagus Plummer-Vinson's syndrome 15% Corrosive strictures 30% I Tylosis* (Hovels-Evans syndrome) Nitrosamines -rylosis is an inherited disease with thickening of skin of palm and sole

I Pathology Common in: Middle third-50%. Lower third-33%. }., Upper third-1 7%. Lower 3 cm of oesophagus is lined by columnar epithelium, and so adenocarcinoma is common here. Barrett's columnar metaplasia which occurs in lower third of oesophagus is also more prone for adenocarcinoma. Squamous cell carcinoma is commonest type in India and Asian countries.

L___

---

---

Figs. 19.31A and B: Carcinoma oesophagus middle thirdpathology specimen, gross and cut section. Note: • In India 90%are squamous cell carcinomas. • In western countries, adenocarcinoma is becoming more common. Adenocarcinomaarises from submucosal oesophageal glands/heterotrophlc columnar epithelium/Barrett's oesophagus. • H. pylori infection is associated with reduced risk of oesophageal adenocarcinoma (inversely related).

B Annular (15%) Ulcerative (20%) Fungating-caulillower like

Polypoid Varicoid-dilluse submucosal type

(60%)

I Spread Direct Lack of serosal layer in oesophagus favours local extension. In upper third it spreads through muscular layer

All things must change to something new and strange.

:c

>

m

....

:a

CD

CARCINOMA OESOPHAGUS Carcinoma oesophagus is common in China, South Africa and Asian countries. It is 6th most common cancer in the world . It is less than 1% of all cancers. It is 7% of all GI malignancies. It is less common in America and European countries. In India, it is common in Karnataka and Odisha. When patient presents with dysphagia, often it is fairly advanced and inoperable and only palliation is the possibility. But then surgery is the treatment of choice in early growths.

C"">

0

Cl)

en 0

"O

:::r

QI

IC C

en

796

...

Q)

Cl

::I

Cl)

0

and gets adherent to left main bronchus, trachea, and left recurrent laryngeal nerve (causes hoarseness), aorta or its branches (causes fatal haemorrhage, but rare). It may perforate and cause mediastinitis. It may get adherent to pleura also. Broncho-oesophageal, trachea-oesophageal, oesophagoaortic fistulas can occur in advanced cases; it may cause torrential haemorrhage, pulmonary infection. Barium study, contrast dynamic CT, bronchoscopy will help in identifying fistulas. Surgery and radiotherapy are not possible in such situation. It is the terminal stage of the disease. Endoscopic stenting is the better palliation method.

I Clinical Features Recent onset of dysphagia is the commonest feature. For the dysphagia to develop, two-third of the lumen should be occluded. •·· Regurgitation; Anorexia and loss of weight (severe), cachexia. Pain-substernal or in the abdomen. Liver secondaries, ascites. Bronchopneumonia, melaena. Features of broncho-oesophageal fistula in carcinoma of upper third oesophagus (30%). Left supraclavicular lymph nodes may be palpable. Hoarseness of voice due to involvement of recurrent laryngeal nerve. Hiccough, due to phrenic nerve involvement. Back pain-due to nodal spread (paraoesophageal/coe/iac nodes). Male to female ratio is 3:1. In adenocarcinoma, it is 15:1. TNM staging and histologic grading for carcinoma oesophagus and esophagogastric junction (AJCC staging 8th edition, 2018)

T status - Tumour Tx Tumour cannot be assessed TONo primary tumour Tis High-grade dysplasia T1 Invasion into the lamina propria, muscularis mucosae (T1 a) or submucosa (T1 b) T2 Invasion into muscularis propria T3 Invasion of paraoesophageal tissues without adjacent structure spread T4a Invades resectable adjacent structures (pleura, pericardium, azygos vein, diaphragm) T4b Invades unresectable adjacent structures (aorta, vertebral body, trachea) N status - Nodes

Nx Regional nodes cannot be assessed NONo regional lymph node metastases N1 1 to 2 positive regional lymph nodes N2 3 to 6 positive regional lymph nodes N3 7 or more positive regional lymph nodes M status - Metastasis cMONo distant metastases cM1 Distant metastases present pM1 Distant metastases microscopically confirmed Histologic grade Gx Grade cannot be assessed G1 Well differentiated G2 Moderately differentiated G3 Poorly differentiated; Undifferentiated

Figs. 19.32A to D: Gross outer look and cut-section of proliferative and indurated lesions of carcinoma oesophagus.

Lymphatic spread It spreads both by lymphatic permeation and lymphatic embolisation. It can cause satellite nodules elsewhere in the oesophagus, away from the main tumour. Above in the neck, it spreads to supraclavi cul ar lymph nodes. In thorax, it spreads to paraoesophageal, tracheobronchial lymph nodes to subdiaphragmatic lymph nodes. In abdomen, it spreads to coeliac lymph nodes. Blood spread occurs to liver, lungs, brain and bones.

Clinical staging (AJCC 8th edition, 2018) Stage O: Tis NO Mo. Stage I: T1 N0-1 MO. Stage II: T2 N0-1 MO; T3 NO MO. Stage Ill: T3 N1 MO; T1 -3 N2 MO. Stage /VA: T4 N0-2 MO; Any T N3 MO. Stage /VB: Any T any N M1. Note: Pathological stage groups are different for adenocarcinoma and squamous cell carcinoma; Grading (G) is used in P staging. Ellis WNM staging is the other staging system used (W-wall penetration [WO,W1 ,W2J; N- Nodal spread; M- Metastases)

I Investigations Barium swallow. Shouldering sign and irregular filling defect. Rat tail lesion on f/uoroscopy is typical. Oesophagoscopy-to see the lesion, extent and type.

797

C

CD

en 0

'C

-=

=en 0,)

C

Figs. 19.33A and B: Barium swallow showing irregular filling defect and shouldering sign in middle third oesophagus.

Figs. 19.34A to D: Barium swallow X-rays showing irregular filling defect at different levels-feature of carcinoma of oesophagus.

Biopsy-for histological type and confirmation. Chest X-ray-to look for aspiration pneumonia. CTscan (95% accuracy)-to look for local extension, nodal status, perioesophageal/d iaph ragmatic/pericardial (1%)/ vascular infiltration, obliteration of mediastinal fat and status of tracheobronchial tree in case of upper third growth. Bronchoscopy to see invasion in the upper 113rd carcinoma of oesophagus; laryngoscopy to identify vocal cord palsy. Chromoendoscopy, magnification endoscopies are newer methods. Local topical application of different stains will improve the tissue (tumour) localization, features and diag-

noses. Different stains that are used like absorptive (Lugo l's, methylene blue-get absorbed by specific cell membranes), contrast(indigocarmine-permeate into the mucosa! crevices showing irregularity and topography) or reactive (Congo red, phenol red-shows colour change due to reaction with cell chemicals). Biopsy is done in these selected areas. Oesophageal endosonography-to look for the involvement of layers of oesophagus, nodes, cardia and left lobe of the liver. Nodes smaller than 5 mm can be very well visualised by EUS which may be missed in CT scan. EUS guided transmucosal nodal needle aspiration cytology can also be done.

Fig. 19.35: Endoscopic views of oesophageal carcinomas-at different levels and different gross types.

Tolerance is another word for indifference.

798

...= Cl)

en

en 'E

Fig. 19.36: CT scan chest and abdomen is essential investigation in

carcinoma oesophagus to identify operability, nodal status. Ultrasound abdomen-to look for liver and lymph nodes status in abdomen. Endoscopic oesophageal staining with labelled iodine results in normal mucosa being stained brown, but remains pale in carcinoma (as mucosa in carcinoma will not take up iodine). Blood tests: Haematocrit; ESR ; Liver function tests. Laparoscopy It is useful to see peritoneal spread, liver spread and nodal spread. It is the only reliable method to detect peritoneal seedlings. Biopsy from different places can also be taken. It will prevent unnecessary laparotomy for anticipated surgical resection. PET scan using 18 F-fluorodeoxyglucose (FOG): 18 FOG is given to the patient. FOG enters highly active cells and gets phosphorylated to FOG 6 phosphate. It stays in the cell as end product and gets polarised there. PET with CT scan is used to see response for therapy. Video-assisted thoracoscopic approach-4.o stage oesophageal carcinoma and to identify the operability, nodal status. Endoscopic mucosa/ resection (EMR) is done using double channeled endoscope with tip having a soft plastic cap. Cap is firmly placed over the lesion and suction is created; a snare is brought over the lesion; biopsy specimen is snared off 1.5 cm in size containing mucosa and submucosa. It is basically a diagnostic biopsy tool; but can be therapeutic in early and premalignant lesion. Endoscopic submucosal dissection (ESD, Japan) is now devised using hook cautery and scissors to remove the lesion up to muscularis propria. Newer modalities of evaluation-flow cytometry, p53 immunohistochemistry, optical coherence tomography, spectroscopy, etc.

nodal spread, curative surgery is the main approach-radical oesophagectomy. Proximal extent of resection should be 10 cm above the macroscopic tumour and distal extent of resection is 5 cm from macroscopic distal end of tumour. Proximal stomach has to be removed commonly especially in lower 113rd of tumour. Sufficient removal of contiguous structures may be needed in curative resection. If nodes are present, then mu ltimodal approach shou ld be used like-cu rative resection; radiotherapy and chemotherapy. Outcome of surgery depends on location of tumour; number, location and size of nodes; tumour grading. Lymph node involvement more than 5 carries poor prognosis. If 5 or less nodes are involved then, it is called as curative resection. Neoadjuvant therapy by chemotherapy and/or radiotherapy prior to surgery may improve the survival. Aggressive chemoradiation also may be used as curative therapy in some patients especially upper 113rd growths and in patients who are unfit for surgery. In remaining patients (80%) palliation is the main modality of treatment. Palliation therapy is done if patient is not fit for major surgery; if there is blood spread; if there is spread to adjacent organ; if there is peritoneal/liver spread. It is to relieve pain and dysphagia and also to prevent aspiration and bleeding.

Indications where probably curative therapy is not possible

To palliate

Nodes >5 involvement Invasive, poorly differentiated grade Length of involvement >8 cm Abnormal oesophageal axis in barium study Homer's syndrome Loss of weight >20% Metastatic disease

Pain Dysphagia Prevent bleeding Prevent aspiration

I Treatment Note: Gastrostomy should not be done as a palliative procedure.

I Principles B 1. Early growth when patient is fit. 1

2. When there is no involvement of adjacent perioesophageal struc- j lures, bronchus, liver or distant organs. _J Only 20% of oesophageal cancers present early and becomes curable. In such early growths confirmed with absence of

Fig. 19.37: Carcinoma oesophagus (Endoscopic view).

I Approaches for Different Level Tumours

799

Post-cricoid tumour (Squamous cell carcinoma): Treated mainly by radiotherapy. Radical radiotherapy5000-6000 rads. Often pharyngolaryngectomy is done along with gastric or colon ic transposition. But complications are more in this procedure. Free jejunal transfer is the other option. Upper third growth (Squamous cell carcinoma): Treated mainly by radiotherapy. Commonly it is advanced with left recurrent laryngeal nerve palsy and bronchial invasion. If it is early and operable , McKeown three phased oesophagectomy and anastomosis is done in the neck. Initially laparotomy is done to mobilise the stomach. Then thoracotomy through right 5th space is done and oesophagus is mobilised. Through right side neck approach, oesophagus with growth is removed. Anastomosis between pharynx and stomach is done in the neck. Split sternum approach oesophagectomy is also practiced.

Middle third growth (Squamous cell carcinoma): Ivor Lewis operation (Lewis-Tanner two-phased oesophagectomy): After laparotomy stomach is mobilised. Pyloroplasty is done. Through right 5th space thoracotomy is done and growth with tumou r is mobilised. Partial oesophagectomy and oeso phagogastric anastomosis is done in the thorax. lntercostal tube drainage is placed during closure. Right gastroepiploic vessels should be retained safely (essential). Azygos vein should be ligated securely. Mediastinal nodes should be dissected. Thoracic duct shou ld be ligated if needed. Feeding jejunostomy is better to maintain nutrition. Complications of Ivor Lewis operation-Pulmonary (bronchopneumonia, empyema), anastomotic leak, mediastinitis, oesophagitis and sepsis. If the growth is inoperable, palliative radiotherapy is given.

Lower third growth (Squamous cell carcinoma + adenocarcinoma): Here through left thoracoabdominal approach, partial oesophagogastrectomy is done with oesophagogastric anastomosis. Often jejunal Roux-en-Y loop anastomosis is done. Orringer approach, i.e. transhiatal blind total oesophagectomy with anastomosis in the left side of the neck. Through laparotomy, stomach and lower part of the oesophagus are mobilised. Through left sided neck approach, upper part of

the oesophagus is mobilised using finger. Blind dissection is completed by meeting both fingers above and below in the thorax. Later oesophagus is pulled up out above through the neck wound and removed . Continuity is maintained in the neck. It is a palliative surgery.

0

ell

en

0 'C

:::r

Fig. 19.38: Transhiatal blind oesophagectomyon table surgical specimen.

I Other Approaches Thoracoscopic-laparoscopic oesophagectomyand lymphadenectomy is becoming popular, safer and effective. Radical oesophagectomy with 3-field clearance of abdominal/ thoracic and cervical nodes is also practiced in many centres. 3-Field clearance (coeliac, thoracic and neck) is done for mainly squamous cell carcinoma as spread in sec is upwards. In adenocarcinoma 2-field clearance is sufficient (abdominalcoeliac and thoracic) as spread is downwards. Note: When oesophagus is removed totally or subtotally an interposition is required between cervical oesophagus/pharynx and distal stomach. Different parts of GI is used for the same.

Oesophageal substitutes: Stomach: It is preferred one and is based on right gastric and right gastroepiploic arteries. It needs only one anastomosis and take up is well due to good vascularity. But it can cause postprandial fullness with bile or acid regurgitation (>50%). It also needs a pyloroplasty for gastric drainage as vagotomy is done. Entire stomach or tubed part or reverse tubed part can be transposed. Colon: It is better as there are less postprandial problems. But it needs 3 anastomoses. Left colon is used with ascending branch of left colic artery as isoperistaltic loop. Right colon is used with middle colic artery as base as antiperistaltic loop. Complications are leak, fi stula formation, delayed emptying , redundancy, peptic ulceration, stricture formation. Colonic polyp and diverticu losis are contraindications for colonic conduit. Jejunum: Isolated required length of pedicled jejunum is transposed. Jejunal free transfer is also tried; vessels are sutured to internal mammary vessels or to the vessels available the neck. Pedicled jejunum is also supercharged with microvascular anastomoses with internal mammary vessels. It is used as a last option only.

Life is half spent before we know what it is. Life is like a beautiful melody; only its lyrics are messed up.

f»

IC

C

en

800

Transposition is done through posterior med iastinum (shortest route), right pleural space (transpleural), retrosternal or subcutaneous route. Skinner en bloc resection used to be of practice in olden days in which tumour with oesophagus is resected along with thoracic duct, azygos vein, intercostal vessels crossing the vertebral bodies, pericardium and mediastinal pleura. There is no survival benefit with this procedure. Vagal sparing oesophagectomywith HSV is tried to preserve pylorus functioning with avoiding pyloroplasty to reduce the chances of postprandial fullness. But it is technically tedious. Lymphadenectomy in carcinoma oesophagus: Standard: Nodes removed are-paratracheal, parabronchial, carinal, paraoesophageal , posterior mediastinal, paracardial , left gastric, along lesser curve of stomach. Extended/three field/ultraradical: As like standard above, with bilateral cervical lymphadenectomy, removal of upper mediastinal, coeliac, retroperitoneal, subhepatic nodes.

Ivor-Lewis 2-phase oesophagectomy McKeown 3-phase oesophagectomy • Left abdominothoracic approach for lower oesophagus- Sweet approach • Thoracoscopic-laparoscopic Transhiatal blind approach-Orringer's

I Postoperative Management Fluid and electrolyte management. Antibiotics and proper analgesia. Respiratory care; ICT care; physiotherapy. Prevention of DVT- elevation, exercises, heparin. Monitoring for bleeding, sepsis, leak, oxygen saturation. TPN only during initial postoperative period and early jejunostomy feeding for nutrition.

I Palliative Treatment 80% of patients with carcinoma oesophagus, when present first, have fairly advanced tumour and so they are amenable for only palliative treatment. It is to relieve pain, dysphagia and to prevent aspiration and bleeding. Palliation therapy is done: If patient is not fit for major surgery. If there is blood spread. If there is adjacent organ spread. If there is peritoneal/liver spread.

Different methods are:

Palliative external radiotherapy 3000 Rads. Severe mucositis, stricture and fistula formation are the complications.

••• lntraluminal RT Brachytherapy (radiation intraluminally). Loading catheter is placed using endoscope and applicator is fixed to mouth to give 1500 cGy radiation with least systemic effects. •

a Figs. 19.39A and B: (A) Stricture oesophagus after radiotherapy given for carcinoma oesophagus; (B) Oesophageal stent in situ- a look endoscopically.

Chemotherapy Cisplatin; methotrexate; mitomycin C; 5 FU; pa/citaxel, etoposide, bleomycin. Platinum based chemotherapy is beneficial especially in advanced adenocarcinoma of oesophagus. Intubation Intubation was described by Symmonds in 1887. It is commonly used method. Guidewire is passed across the growth under X-ray screening or C-arm guidance; flexible introducer and prosthetic tube is pushed across the tumour along the guidewire. It carries 90% success rate. Problems are-tube intolerance, poor drainage, airway compression, reflux, aspiration, displacement, food blockage, tumour overgrowth beyond the prosthesis causing its failure. Intubation is used for trachea-oesophageal fistula or external comp ress ion. Prosthesis with a sponge-filled balloon is used for fistula closure. Standard tube wrapped with multilayered polyvinyl sponge is other option. It is less expensive, single time, rapid acting. They can be traction or pulsion tubes. Perforation chance is 10%. Different tubes used are: Atkinson tube. Celestin tube (armoured rubber tube with a long tail)ideal, commonly used tube. It is wider proximally. It can be passed through endoscopy or laparotomy. Souttar tube (coiled German silver wire}--block is first dilated with bougies; tube is passed over small sized bougie and pushed across the block. It is mainly useful for lower oesophagus. Mousseau-Barbin tube-cheaper but needs laparotomy. After laparotomy, anterior wall of the stomach is opened. MB tube is passed from mouth with the help of anesthetist by stitching its tip to nasogastric tube and pulled

down into the stomach . Tip is cut near the cone part. It is sutured to anterior wall of the stomach. Gastrotomy is closed. Endoscopic therapy Self-expanding metal stents (SEMS) are passed through endoscope under C-arm guidance. It is the ideal method of palliation. Stent is collapsed during insertion and released once it is placed in proper position. There is no need to dilate oesophagus more than 8 mm to pass this expanding stent and so chances of perforation is minimal. Uncovered SEMS-heretissues project through the mesh to have a better grip with less chances of migration. But stent occlusion is more. Plastic covered SEMS-it shows less stent occlusion and less friction. Stent migration is more. SEMS may be-stainless wall stent; knitted nitinol Strecker stent; stainless steel covered Gianturco-Rosch stent. Problems of stents are-aspiration , displacement, erosion, bleeding, tumou r growth across or beyond mesh, food bolus obstruction, retrosternal pain, need for reinsertion (40%). Mortality is 1- 2%. Endoscopic laser is used to core a channel through the tumour to improve dysphagia (Nd YAG laser; Diode laser). It causes thermal destruction of tumour. It improves dysphagia but needs repeated laser ablation. It may be used more effectively to remove tumour block in previously placed stents. Exophytic tumour less than 6 cm is suitable for laser. Noncontact high power Nd:YAG 50-100 W laser from distal to proximal end facilitates visualisation of lumen and also reduces the chances of perforation. Contact low power Nd:YAG 10-20 W laser is used for fully occluded tumour with less smoke formation and less perforation chance. Success rate of palliation is 85%. Problems are-fever, chest pain, 3% mortality, perforation (2%) and fistula tormation- 5%, costly, takes one week to relieve dysphagia Endoscopic bipolar diathermy using a olive tip; argon beam plasma coagulation; endoscopic alcohol injection into the tumour. Endoscopic photodynamic therapy (PDT) is used to destruct tumor and to relieve dysphagia. It is often used as a therapy in early cancer. Photosensitive haematoporphyrin agent is injected intravenously 48 hours before en doscopy. It is activated over tumour using laser. Visible infrared light also can be passed to tumour endoscopically to create tumour necrosis by released cytotoxic singlet oxygen through photosensitiser. Sunburn, fever, perforation, pleural effusions are complications. It is effective only to superficial cancers; effects will be seen

only after 1 week; need to avoid direct sunlight exposu re by the patient for one month is the drawback. Surgery Transhiataf Orringer's blind oesophagectomy is a palliative surgical procedure. Kirschner palliative gastric bypass done in advanced carcinoma oesophagus wherein mobilised stomach is brought to neck via retrosternal or subcutaneous route and anastomosed to divided cervical oesophagus. Lower cut end of oesophagus is anastomosed to a jejunal loop. Here oesophagus is not addressed (left alone).

801 n :c

>

""0

-I

m

:::D

...... u::,

0

CD

rn

0 'C :::r' I»

B External or intraluminal RT (Brachytherapy) Traction tubes like Celestin or MB tubes through open surgery • Pulsion tubes like self-expandable metal stents through endoscopes using C-arm Endoscopic laser Chemotherapy • Transhiatal oesophagectomy-Orringer

Tube in place

Figs. 19.40A and B: (A) Mousseau-Barbin tube placed as a palliative method for carcinoma oesophagus; (B) Mousseau-Barbin tube.

5-10% mortality • Haemorrhage • Respiratory infection, often severe Septicaemia Chylothorax, injury to thoracic duct • Anastomotic leak-thoracic leak is most dangerous (5- 10%) • Hoarseness due to recurrent laryngeal nerve palsy Stricture formation (40%) Gastro-oesophageal reflux in gastric transfer Conduit necrosis due to ischaemia either stomach or colon

J

Colonic dysmotilitycausing partial obstruct ion in colon transfer

I Terminal Events in Carcinoma Oesophagus Cancer cachexia. Sepsis, mediastinitis.

Truth, like surgery, may hurt, but it cures.

cc C rn

802

...= Q)

en

Cl)

0 11:1

= C 11:1

:!::

-CCI"' a:

Cl)

lmmunosuppression. Malignant tracheo-oesophageal fistula (causes severe respiratory infection and death. Here expansile (self-expandable) endofuminaf stents are used at the site of fistula to have temporary benefit). Erosion into major blood vessel-bleeding.

I Prognosis Not good because of early spread, longitudinal lymphatics, aggressiveness, difficult approach, late presentation. Nodal involvement carries bad prognosis. 5-year survival rate is only 10%.

,.

Treatment-if tumour is more than 5 cm/symptomatic tumour/intralu minal tumou r/when diagnosis is doubtfu l surgical enucleation is indicated. Enucleation is the therapy of choice. Ideally it should be done through right-sided thoracotomy. Occasionally oesophageal resection is needed if tumour is very large/tumour with mucosa! ulceration/if tumour is near OG junction. Thoracoscopic resection can be done. Leak, empyema, sepsis and stricture are the occasional complications.

BENIGN TUMOURS OF THE OESOPHAGUS Benign tumours of the oesophagus are rare (0.5-1 % of all oesophageal tumours). It grows slowly like a balloon by expansion, compressing surrounding structures. It never infiltrates or spreads. It can be solid, cystic, polypoid. It is usually in submucosal plane. It can cause obstruction/regurgitation/aspiration/ mediastinal compression. It can be squamous papilloma/polyp/inflammatory pseudo tumour/feiomyoma (commonest benign tumour of oesophagus-65%)/neurofibromal rhabdomyomallipoma. True adenoma in oesophagus is very rare. Features may be asymptomatic (85%-identified incidentally during contrast X-ray/endoscopy); dysphagia/airway obstruction/pneumonia/spluttering during swallowing; stridor/ regurgitation. Leiomyoma (commonest-65%) is smooth, sessile, lobulated, firm, with grey-white whorled appearance. Only when leiomyoma reaches 5 cm in size, it causes obstruction. Multiple localised leiomyomas can occur which can be enucleated independently. True diffuse feiomyomas can occur occasionally in females (4%) in lower oesophagus, as diffuse hyperplasia and thickening of both muscular layers; often as part of the Alport's syndrome which needs total oesophagectomy with gastric pull up, even though benign. Benign leiomyoma of oesophagus rarely turns into leiomyosarcoma. 90% of oesophageal leiomyomas occur in lower third of the oesophagus. Leiomyomas are common in men in 5th decade. Leiomyoma which expresses the c-kit oncogene (CD117) is considered as GIST. Investigations-barium swallow X-ray (smooth circular outline/eccentric filling defect)/oesophagoscopy/endosonography/CT scan.

Figs. 19.41A and B: Oesophageal submucosal tumour-

endoscopic view-leiomyoma. Note: Unlike in the stomach an d intestine (gastric leiomyoma more than 6 cm/intestinal leiomyoma more than 4 cm are potentially malignant) increased size of the oesophageal leiomyoma does not predispose the malignant transformation.

Oesophageal cysts It is 2nd commonest benign tumour of oesophagus. It can be congenital or acquired. Congenital is derived from foregut. It contains mucus. It is lined by ciliated columnar epithelium. In infants, it is common in upper third of oesophagus; often with a fistula into airway. It can cause obstruction. Acquired cyst is from obstruction of the excretory ducts of oesophageal glands. Treatment Enucleation or resection. If fistula is present it should be ligated and divided.

OESOPHAGEAL PERFORATION Causes: Instrumenta l injuries-commonest cause, 75 % commonest site is just above the level of cricopharyngeus. Foreign bodies; Alkali injuries; Carcinoma oesophagus 1%. Boerhaave's syndrome 15%; Trauma 9%. Surgical trauma (Vagotomy, thyroidectomy, Heller's operation, pneumonectomy, spine surgery). Features: Chest pain , vom iti ng, shock, subcutaneous emphysema. Investigations: Chest X-ray-shows pneumomediastinum; CT scan Complications: Mediastinitis; Septicaemia; Empyema, ARDS

803 Common foreign bodies: Coins, metals, plastics Dentures Pins, toothpicks, batteries Fish or meat bones-dangerous- 40% Food (meat- common/vegetables) impaction-45% Sites of impaction in oesophagus: Cervical constriction-C6 Broncho-aortic constriction-T4 Diaphragmatic constriction-T10 Pre-existing malignancy or inflammatory stricture site Features: Sudden dysphagia with chest pain and breathlessness Later features of shock, sepsis, mediastinitis, empyema

Fig. 19.42: Oesophageal perforation with mediastinitis and emyema; perforation is after eating largechicken bone.

Management: X-ray shows site and level of the foreign body (FB) Endoscopic removal can be tried. Impacted large FB should be removed by thoracotomy Antibiotics, jejunostomy, TPN, ICT are also required

Treatment: Conservative treatment It is advocated in small perforations due to instrument where there is minimal air leak and contami nation of med iastinum with less septic load. Crepitus should be absent; pleura should be clear and without any obstruction. Treatment is-antibiotics, nutrition (TPN/enteral through tube) , fluid management, proper observation and monitoring the patient by repeated blood counts, and imaging. Biodegradable removable self-expanding stents also can be used. It may also act as a bridge therapy for eventual major surgical exploration. Stents which are used for carcinomas cannot be used as they cannot be removed. Thoracotomy, proper saline wash to pleura, mediastinum and repair with buttressing the area using pedic/ed intercostal musculopleural flap is done. Nasogastric tube for long duration, jejunostomy tube for feeding, ICT for drainage is essential. Often in late cases decortication of lung is needed to achieve full expansion of the lung. Repair over T-tube so as to create a controlled fistula along with feeding jejunostomy and ICT on both sides. lntraluminal stents/mediastinal irrigation and drainage. Resection of oesophagus with gastric pull up. As cond ition is an emergency situation and with sepsis it carries high mortality. Oesophageal exclusion with cervical oesophagostomy above and feed ing jejunostomy below. Diversion surgeries using colon/stomach/jejunum.

Fig. 19.43: Foreign body in the oesophagus.

Fig. 19.44: Foreign body (COIN) in the lower oesophagus. Usually it can be removed by endoscope.

You can become strongest in your weakest place.

0

CD

en

0 "Cl ::I" 0,1

IC C

"'

Chapter

Stomach ( iHAPTER

•

• • • • • • • • • • •

•

•

OUTLINE ::::=========--

Anatomy • Blood Supply of Stomach • Nerve Supply of Stomach • Histology • Lymphatic Drainage of Stomach • Duodenum Gastric Physiology Gastric Function Tests Gastrin Barium Meal Study Gastroscopy • Helicobacter pylori Congenital Hypertrophic Pyloric Stenosis Gastritis Acute Peptic Ulcer Gastric Ulcer Duodenal Ulcer Pyloric Stenosis due to Chronic Duodenal Ulcer • Electrolyte Changes in Pyloric Stenosis Perforated Peptic Ulcer • Perforated Duodenal Ulcer • Perforated Gastric Ulcer • Perforated Stomal Ulcer • Dry Perforation Bleeding Peptic Ulcer • Bleeding Duodenal Ulcer

• •

• • • • • • • • • •

• • • • • • • •

• Bleeding Gastric Ulcer Haematemesis Complications of Gastric Surgery • Duodenal Blow-out • Recurrent Ulcer • Dumping Syndrome • Roux Stasis Syndrome • Gastrojejunocolic Fistula Trichobezoar Chronic Duodenal lieus Dunbar's Syndrome Acute Gastric Dilatation Gastric Volvulus Gastric Polyp Menetrier's Disease Duodenal Diverticula Carcinoma Stomach Gastric Lymphoma • Primary Gastric Lymphoma • Secondary Gastric Lymphoma Gastric Sarcomas Gastrointestinal Stromal Tumours Pyloroplasty Gastrostomy Gastrectomy Gastrojejunostomy Retrograde Jejunogastric lntussusception Vagotomy

ANATOMY The stomach contains four anatomic regions: 1. Fundus; 2. Cardia; 3. Body; 4. Pyloric part contains pyloric antrum, pyloric canal. The duodenum is 20-30 cm in length. It extends from pyloric sphincter to ligament of Treitz. It is divided into four parts. Ninty per cent of duodenal ulcer occurs in the 1st part of duodenum (duodenal bulb/cap). CBD and pancreatic duct merges to form ampu//a of Vater and enter the 2nd part of duodenum. The 3rd part of duodenum is wedged between aorta and superior mesenteric artery.

Duodenum

Body

Fig. 20.1: Parts of stomach.

I BLOOD SUPPLY OF STOMACH I Arterial Supply Left gastric artery, a branch of coeliac artery (Smallest branch of coeliac axis) . Right gastric artery, a branch of hepatic artery. Gastroduodenal artery, a (largest) branch of hepatic artery. Right gastroepiploic artery, a branch of gastroduodenal artery. Left gastroepiploic artery, a branch of splenic artery. Short gastric arteries, branches of splenic artery.

Posterior vagus nerve

.______

Right gastric artery

Criminal nerve of Grassi (posterior vagus)

Pylorus

.,,,,-r--...e~":ll

f:L~ == ===,

b-- Anterior and posterior nerves of Latarjet

C"')

:::c

>

""O

-t

m

:::J:J N

Cl

Cl)

0

3

I»

n ::r

Right gastroepiploic artery

Fig. 20.2: Blood supply of the stomach.

I Venous Drainage

Fig. 20.3: Anatomy of nerve supply of stomach.

Right and left gastric veins drain into portal vein (left gastric vein also called as coronary vein). Right gastroepiploic vein drains into superior mesenteric vein. Left gastroepiploic vein and short gastric veins drain into splenic vein. Prepyloric vein of Mayo distinguishes pyloric canal from the first part of duodenum.

I NERVE SUPPLY OF STOMACH Intrinsic innervation occurs through myenteric plexus of Auerbach and submucous plexus of Meissner. Right vagus is posterior and left vagus is anterior. Posterior vagus gives criminal nerve of Grassi which supplies lower oesophagus and fund us of stomach, which, if not cut properly during vagotomy, may lead to recurrent ulcer. Vagus also gives splanchnic branches (hepatic and coeliac branches), ends as nerve of Latarjet which supplies the antrum and maintains the antral pump. Parietal branches help in HCI secretion , which is an important concept in vagotomy that is done as a treatment in duodenal ulcer. Truncal vagotomywith posterior gastrojejunostomy is done for chronic duodenal ulcer with pyloric stenosis. Highly selective vagotomy (HSV) is done in case of uncomplicated chronic duodenal ulcer which is not responding to available medical line of treatment. In HSV, nerve of Latarjet is retained so as to retain antral pump and no drainage is required. Here only the fibre s entering the stomach are ligated close to the lesser curve to reduce the acid secretion. In selective vagotomysplanchnic branches are retained but it is presently not done. Gastroduodenal pain is sensed via sympathetic fibres (T5- T, 0).

I HISTOLOGY The fund us and body contains parietal and chief cells. Parietal cells secrete acid and intrinsic factor. Chief cells produce pepsinogen. There are two types of pepsinogen secreted by chief cells- I and II. Pepsinogen I is produced only in stomach. In gastric atrophy pepsinogen I is decreased. In the antrum, endocrine cells produce gastrin (G cells) and somatostatin (D cells). 12% of epithelial cells of stomach are parietal (oxyntic) cells; 45% chief (zymogenic cells); 40% mucous cells; 3% endocrine cells. Pyloric sphincter is a thick circumferential layer of smooth muscle. Submucosa is strongest, collagen rich layer of gastric mucosa. Gastric mucus is a mucosal barrier containing mucopolysaccharides which maintains the integrity of gastric mucosa.

I LYMPHATIC DRAINAGE OF STOMACH Lymphatics of proximal half of stomach drain into left gastric, splenic, and superior pancreatic lymph nodes. From antrum, it drains into right gastric, right gastroepiploic, and subpyloric lymph nodes. From pylorus, it drains into right gastric and subpyloric lymph nodes. Efferent lymphatics from suprapyloric region drain into paraaortic lymph nodes and so into left supraclavicular lymph nodes. Efferent lymphatics from subpyloric lymph nodes drain into superior mesenteric lymph nodes. Lymphatics near oesophagogastric (OG) junction communicate with oesophageal lymphatics. In carcinoma stomach if upper lymphatics are blocked, retrograde spread through lower lymphatics can occur. Presently in carcinoma stomach different resections are classified as RO, R1 , R2, R3, or D1 , D2 (dissection) based

Goals and actions must meet.

806

on levels of lymph nodes in the abdomen in relation to the stomach. RO is no residual disease; R1 is microscopic residual disease; R2 is macroscopic residual disease; R3 is inoperable.

1. Coeliac group 2. Splenic group 3. Gastroepiploic group 4. Subpyloric group 5. Omental group 6. Gastric group

__,._.......;..;;r-

5

Fig . 20.4: Lymphatic drainage of stomach.

I DUODENUM Arterial supply: It is mainly supplied by superior and inferior pancreaticoduodenal arteries. First part also gets supply from right gastric artery, supraduodenal artery, a branch of hepatic artery. Venous drainage: Drains into the splenic, su perior mesenteric and portal veins. Nerve supply: Sympathetic from spinal segments T8 and T10 ; Parasympathetic from vagus.

Fig. 20.5: After passing the Ryle's tube to the stomach, air from the

syringe should be infused through the tube. When auscultated gush of air into the stomach can be heard which confirms that tube tip is in the stomach.

Lymphatic drainage: Drains mainly to pancreaticoduodenal nodes present along the inside of the curve of duodenum.

GASTRIC PHYSIOLOGY Gastric function is regu lated by hormonal and neural methods. Hormonal mediators control function through endocrine (through release into blood), paracrine (diffusion across interstitial space) and neurocrine (diffusion across synapsed target cell and receptor binding). Gastric acid secretion is regulated by acetylcholine, histamine and gastrin. Acetylcholine is the principal mediator of acid release through vagal parasympathetic ganglion cells. Vagus innervates parietal, Gand enterochromaffin-like (EGL) cells. Basal acid secretion is 10% of maximal acid output (1-5 mmol/hour) . It is reduced by 90% after vagotomy or H2 receptor blockage. Phases of acid secretion are-(1) Cephalic phase-through central meditation (smell/sight/ taste vagus acetylcholine muscarinic receptors. (2) Gastric phase-food enters the antral G cells acid release th.ough gastrin (gastric distension causes direct acid release). Gastric phase lasts until stomach is empty and releases 70% of total acid release. (3) Intestinal phase-it is 10% of acid release and is mediated by chyme entering the small bowel-nongastrin related. Receptors of acid secretions are-(1) Gastrin-CCK receptors: Two types are present in parietal cell; Gastrin CCK type A receptor has high affinity for CCK but less to gastrin; Gastrin CCK type B receptor has got high affinity for both CCK and gastrin. (2) Muscarinic receptor (parietal cell)-its M3 type is mediator for acetylcholine. (3) Histamine receptor (parietal cell)-H2 subtype binds to histamine to cause effect. (4) Somatostatin receptor-these are 5 subtypes. Its inhibitory action is through parietal cell subtype. (5) Second messengers involved are intracellular cAMP and calcium. Luminal gastric pH is 2. The pH at surface epithelial cells is 7. These cells secrete bicarbonate continuously into the lining mucus gel to keep the pH of surface mucus at 5. Bicarbonate in mucus barrier reduces once luminal pH reduces below 1.4. Functions of gastric acid Conversion of pepsinogen to pepsin which in turn hydrolyses proteins into polypeptides; promotes release of duodenal secretin; prevents bacterial colonisation of upper GIT; formation of food chyme which contains food particles of 1 mm size or less. Gastric juice contains HCI, mucus, swallowed saliva, reflux content from duodenum. Parietal cells secrete electrolytes of 160 mmol/L. They also secrete intrinsic factor, a mucoprotein which is essential for absorption of vitamin B12 in ileum; its secretion is independent of acid secretion from parietal cell and is not influenced by PPls. Pepsinogen is a proteolytic proenzyme (42,000 mol wt). Type I is secreted from chief cells and mucous neck cells of only acid secreting part of the stomach (acid secreting mucosa). Type II is secreted

from surface epithelial cells of entire stomach and proximal duodenum. Mucous is secreted by surface muocus cells and mucous neck cells from acid secreting area of stomach and antrum. Gel like viscous mucous contains 85% water and 15% glycoproteins. It contains bicarbonate secreted from surface epithelial cells. Mucous is strong gastric barrier. Mucous secretion is inhibited by anticholinergics and NSAIDs. H. pylori break the mucin and so barrier. Gastric motility begins from pacemaker cell of Cajal located at proximal stomach. Special myoelectric migrating complex slow waves with electric spikes maintain gastric motility in three phases. Immediately after food intake resting tone of fundus and proximal stomach decrease causing receptive relaxation and gastric accommodation mediated by vagus. Vagotomy eliminates this causing early fullness, early satiety and rapid gastric emptying. Delayed gastric motility occurs in diabetes, H. pylori infection and after vagotomy. Many factors like stress, hormones increase the gastric motility. Prokinetics, erythromycin helps in gastroparesis. Mucosa/ defense factors are-mucous production, mucosal HC0 3- , mucosa! blood flow, growth factors, cell renewal, endogenous prostaglandins, epithelial barrier (hydrophobic phospholipids, restitution, NO, epidermal growth factor and microcirculation). Aggressive factors are-H. pylori, HCI secretion, pepsin , smoking, alcohol, duodenal bile reflux, ischaemia, NSAIDs.

GASTRIC FUNCTION TESTS

B

I ,~ormal Gastric ulcer Duodenal ulcer Z-E syndrome

Basal

Peak

5 mmols 2

25-27

6

Very high

15

35-38 >than 60

Pentagastrin test/Kay's augmented histamine test Hollander's insulin test Radioisotope labelled gastric emptying study 24 hours intragastric pH monitoring • Gastrin level estimation

J

Peak Acid Output Test (Pentagastrin/Augmented Histamine Test Initially stomach is emptied completely. Basal acid level of aspirated stomach content (aspiration is done for 1 hour on empty stomach) is analysed . Pentagastrin- 6 µg /kg or histamine 2 µg/kg is injected IV/SC/IM and 15 minutes samples for subsequent hours (usually 1 hour) are collected and analysed. Peak acid output level is calculated . Test is useful in Zollinger-Ellison (Z-E) syndrome, duodenal ulcer and gastric ulcer. Basal acid output (BAO) is 2- 3 mEq/h. Peak acid output (PAO) is highest rate of secretion obtained in any of the 15 minutes samples following stimulation . Maximal acid output (MAO) is obtained by averaging the output of two final 15 minutes samples. In duodenal ulcer and Z-E syndrome basal and stimulated acid is increased; in pernicious anaemia, gastric atrophy and gastric cancer both are decreased.

(")

:c

>

'"C

-I

Achlorhydria is defined as a condition in which stomach cannot produce secretions with a pH of less than 7.0 even after maximal stimulation. It is seen in 20% of carcinoma stomach. Note:

Achylia means-no pepsin in the gastric juice.

I Insulin Test (Hollander) This test is useful postoperatively to confirm the completeness of vagotomy. 0.2 units/kg body weight of insulin is injected intravenously to a fasting patient so as to create hypoglycaemia of below 35 mg%, which in turn stimulates the parietal cells through hypothalam us and through vagus to cause increased acid secretion. Patient who had undergone complete vagotomy does not show increase in acid secretion. Early response signifies incomplete vagotomy wherein there is increase in acid concentration in first hour of >20 mmol/L. Delayed response signifies an increase in acid concentration in between first and second hours, and is probably due to vagal gastrin release.

GASTRIN

GASTRIC FUNCTION TESTS

807

Gastrin is secreted by the G-cells from gastric antrum. Types of gastrin are-Big gastrin (G 34); little gastrin (G u)-most common 90%; mini gastrin (G 14). Pentapeptide at the C terminal end of G 17 is active part which is identical to CCK. Luminal peptides and amino acids are most potent stimulator of gastrin release. Luminal acid is the most potent inhibitor of gastrin release. Gastrin promotes the release of acid and also regulates it. It also maintains mucosa! defense, has trophic effects on parietal and EGL cells (enterochromaffin like cells). Number of G-cells are increased in duodenal ulcer, G-cell hyperplasia, but not in gastric ulcer. Normal plasma value is 50 ng/L of plasma (fasting). It is analysed by radioi mmunoassay. It increases to many 1OOO's in gastrinomas. Hypergastrinaemia can occur in ulcerogenic conditions like- antral Gcell hyperplasia, retained excluded antrum, Z-E syndrome, short gut syndrome, gastric outlet obstruction; nonulcerogenic conditions are- PPls, pernicious anaemia (-ve feedback) , vagotomy, atrophic gastritis (hypochlorhydria), H. pylori, chronic renal failure. Gastrin is raised very high in gastrinomas.

Excellence is rarely found, more rarely valued.

m :::c

N

C)

808

B Gastrin , Somatostatin-2 types, 14 and 28. In stomach, type 14 is common;

from Dcells of gastric mucosa of fundus and antrum. Antral acidification is the main stimulus; vagal acetylcholine inhibits its release. It has paracrine ettect inhibiting acid secretion from parietal cell, gastrin release from Gcell and histamine release from EGL cell. H. pylori decrease antral D cells and so somatostatin, leading into increased gastrin release Gastrin releasing polypeptide (GRP, Bombesin)-it stimulates gastrin and somatostatin release by binding to receptors on G and D cells. It is released from sympathetic nerve terminals of the gastric mucosa of body and antrum. Its half-life is 1½ minutes Histamine is stored in EGL and mast cells. Its release is stimulated by gastrin, acetylcholine and epinephrine Ghre/in (28 amino acids) is mainly secreted from endocrine cells of gastric mucosa. It has mainly got growth hormone releasing action; prolactin, ACTH, cortisol, aldosterone release is also promoted. Its action on islet cells reduces insulin release. Ghrelin stimulates appetite. Gastric bypass, gastrectomy reduces the appetite.

I I

B Gastroduodenoscopy is ideal and most common investigation used to visualise mucosa. If any lesions biopsy is taken for H. pylori and carcinoma. Also often used for endotherapy Endosonography (EUS) is very sensitive method to assess tumours, visualise stomach layers (90% accuracy), lymph nodes (80%), and to detect early liver metastasis which may not be identified by CT especially from left lobe CT scan is good imaging method to detect the stage, spread, nodal status, liver secondaries, and status of lungs CT and PET scan (together) using FOG is better to assess early spread Laparoscopy is very good investigating tool to identify peritoneal secondaries, and to stage the disease. Laparoscopic US is very sensitive to detect the liver secondaries Barium meal studies are used to detect hiatus hernia • Celiac angiography is useful in bleeding ulcers, both for diagnostic and therapeutic purpose (therapeutic embolisation)

,,. Mottled appearance of stomach because of retained food particles. ,, Evidence of gastritis. 4. Carcinoma stomach- irregular filling defect. 5. Pseudocyst of pancreas-widened vertebrogastric angle. 6. Stomal ulcer in previous gastrojejunostomy. 7. In chronic duodenal ileus ( Wilkie's syndrome)-shows dilatation of stomach, 1st and 2nd parts of duodenum , proximal portion of 3rd part of duodenum. 8. Others-gastric volvulus, duodenal diverticula, t richobezoar, gastric fistulas, diaphragmatic hernias when stomach is the content. 9. Carcinoma of head of pancreas shows "pad sign "; in periampullary carcinoma-reverse "3' sign. Frostberg's reverse '3' sign {inverted '3' sign). Rose thorn duodenum in carcinoma head of pancreas. 10. Hiatus hernia.

I Procedure Barium sulphate solution is used [Barium is neurotoxic, but in sulphate media it will not get absorbed and so barium sulphate is used (Barium phosphate is not used)]. About 300 ml solution is given to the patient to drink and its flow down to the stomach is observed under fluoroscopic guidance. Films are taken as required. Commonly oblique views are taken. Microcrystalised barium sulphate (Microbar solution) is better as it does not get precipitated.

---~-------

BARIUM MEAL STUDY

11ndi cations

1. Gastric ulcer- shows a niche which is the ulcer crater, a notch which is due to spasm of circular muscle on the greater curvature. 2. Chronic duodenal ulcer- shows absence or deformed duodenal cap (due to spasm of 1st part of duodenum, barium will not stay and so cap will not be formed). 3. Gastric outlet obstruction-the cause may be chronic duodenal ulcer with pyloric stenosis or carcinoma pylorus. Features are: ,, Enormous dilatation of stomach. ,, Greater curvature below the level of iliac crest. ,, Absence of duodenal cap. ,, No filling of dye in 2nd part of duodenum.

Figs. 20.6A and B: Barium meal X-rays showing normal picture in one and dilated stomach with features of gastric outlet obstruction in another.

Complication: It may precipitate intestinal obstruction.

GASTROSCOPY It is visualisation of interior of stomach, duodenum, oesophagus. It can be done as OP procedure.

I Uses 1. For diagnosing any pathology, e.g. ,. Gastric ulcer; Duodenal ulcer; Gastritis; Stomal ulcer. ,, Carcinoma stomach.

, ,

Oesophagitis; Varices. Biopsies from the suspected cases of malignancy or for Helicobacter pylori can be taken. - Endosonography can be done to assess the staging, operability of carcinoma stomach or oesophagus. - Presently fibreoptic, flexible, or video gastroduodenoscopy is used.

B Variceal injection or ligation or glueing or banding Stenting of pseudocyst of pancreas through gastroscopy Polyp removal Submucosal resection For ERCP diagnostic and therapeutic procedures Percutaneous gastrostomy (PEG)

I Procedure Gastroscopy is done following eight hours of fasting. After lignocaine spray into the oral cavity, gastroscope is passed gently down the oesophagus when the patient does the swallowing action. Once the scope is inside the stomach, air is inflated and different parts of the stomach is visualised. Fund us is visualised by retropulsion. Scope is passed through the pylorus to see the 1st and 2nd parts of duodenum and looked for any pathology. If required biopsy is taken.

Fig. 20.7: Technique of doing gastroscopy using videoendoscope.

2. Therapeutic ,

,

Videoendoscopy is used not only for diagnosis but also mainly for therapeutic procedures; Both end viewing and side viewing gastroscopes are available. For therapeuti c procedures and ERCP, side viewing gastroscope is required.

Fig. 20.9: Endosonography of stomach showing different layers with echogenicity.

Fig. 20.8: Gastroscopic views of different parts-vocal cords; pyriform fossa; oesophagus; oesophagogastric (OG) junction; body, fundus and pylorus of stomach, duodenum with clearly visible duodenal papilla (Courtesy: Dr Tantry and Dr San deep Gopal, Gastroenterologists, KMC, Mangaluru).

The stomach you can hear, the stomach you can see, the stomach you can teel.-Sir James Walton

C')

:::c

>

""CJ

-4

m

:::z:, N

Cl

Cl)

0

3 Cl) n

:::r

810

Often sedation with midazolam is beneficial to have an easy passage.

...

Note: • Buscopan is used to relax stomach wall. • Z-line signifies squamoco/umnar junction. • Multi-byte biopsy forceps is used.

c

I Complications

a.,

Cl

::I Cl)

Bleeding; Aspiration. Perforation (rarely). Perforation occurs mainly in therapeutic procedures like oesophageal dilatation, endoscopic mucosal resection (EMR). It is common in proximal and middle oesophagus but can occur anywhere. Patient presents with hypotension, tachycardia, sudden severe pain in chest, abdomen, neck and surgical emphysema.

I HEL/COBACTER PYLORI It is gram -ve spiral like flagellated organism , first studied by Warren and Marshall (both got Nobel prize), which is commonly present in stomach. It is involved in pathogenesis of duodenal ulcer, gastric ulcer, gastritis (type B) and gastric cancer.

B Duodenal ulcer-95%; Gastric ulcer-70% Gastritis-70-90% Gastric cancer- No. 1 carcinogen Gastric MALTOMA (mucosa-associated lymphoid tissue lymphoma)

I Pathogenesis It is the most common bacterial infection in the world. Rhesus monkey is the only natural reservoir. Its incidence increases with age. It releases enzymes like urease that hydrolyses urea so as to release ammonia which through negative feedback mechanism increases the gastrin release from G-cells. Infection occurs in stomach, i.e. body, fundus, antrum, disrupts the mucosal barrier, causes chronic inflammatory response leading to gastritis, gastric ulcer. Other than urease it also secretes dehydrogenase (converts alcohol to aldehyde which is toxic to mucosa), endopeptidase (disrupts mucosal barrier). Urease creates alkaline environment around it in mucus layer of gastric epithelium. It can survive only in gastric epithelium or gastric metaplasia in duodenum or Barrett's oesophagus or in heterotopic gastric mucosa in Meckel's diverticulum or rectum. It is because receptors for organisms to adhere into mucosa are available only in gastric mucosa. H. pylori impair mucosa! healing, cause degranulation of eosinophils. It releases various protease and lipases that break mucus and so strong protective mucus barrier. It also secretes cytotoxins (cagA and vacA) which may also be involved in inflammatory reaction or malignancy.

Even though normal duodenum cannot harbour the Helicobacter, duodenum with gastric metaplasia can very well get infected by Helicobacterwhich explains why Helicobacter is involved in duodenal ulcer. Helicobacter is normally not found in saliva. Infection is transmitted through faeco-oral route, with a infection rate of 80-90% in a population (common and high). It is more common in lower socioeconomic group. It is considered to be a carcinogen for stomach. It is not linked with carcinoma of OG junction.

B Urease Dehydrogenase Vacuolising cytotoxin-Vac A exotoxin CagA-cytotoxin-associated gene A

Endopeptidase Haemolysin

It is identified by. a. Rapid urease test (cod liver oil test/CLO test)-noninvasive. b. C13-C14 breath tests (labelled urea breath test)noninvasive. c. Biopsy from the different parts of the stomach and staining to identify the organism-invasive. For H. pylorithree biopsies are taken one each from antrum, body and fundus.

Rapid urease test-90% sensitivity, 98% specificity C13/C14 breath tests-95% sensitivity and specificity-'gold standard' - C13 requires spectrometry and costly - C14 uses radioactivity Serology to identify lgG antibody-ELISA test with 90% sensitivity and specificity Biopsy and culture-very costly Warthin's starry silver stain, acridine orange are special stains

used Newer methods-special fluorescent technique, PCR products

of H. pylori-urease gene, 165 rRNA identified using specialised probes when organisms are in less number

I Treatment Therapy consists of antimicrobials combined with suppression of gastrc acid . Various combinations like triple regimes are used

to control and eradicate the infection. Clarithromycin, amoxicillin, tetracyclines, bismuth and metronidazole/tinidazole with omeprazole/lanzoprazole/pantoprazole (proton pump inhibitors) combinations-are used. Triple regime is given for 7-14 days. After that PPI should be continued for 6-12 weeks. Follow-up confirmation of the eradication of the infection is needed often. Quadruple therapy using PPI twice daily, bismuth 2 tablets 4 times daily, metronidazole 250-400 mg three times daily, tetracycline 500 mg 4 times daily is used for 7-14 days.

T

Control of H. pylori Infection

It is treated with antibiotics and other drugs with different combinations

Constipation. Dehydration and loss of weight. Electrolyte imbalance-hypokalaemic metabolicalkalosis.

811

C"')

Clarithromycin 500 mg BO

Metronidazole 400 mg BO

Omeprazole 20 mg BO

OR

OR

OR

Amoxycillin 500-750 mg BO

Tinidazole 600 mg BO

Lansoprazole 30 mg BO

OR

OR

Tetracyclines, or Bismuth.

Pantoprazole 40 mg BO

The above regime is given for 7-14 days and then only proton pump inhibitors are continued.

CONGENITAL (INFANTILE) HYPERTROPHIC PYLORIC STENOSIS It is hypertrophy of musculature of pyloric antrum, especially the circular muscle fibres, causing primary failure of pylorus to relax. Duodenum is normal. There is increased risk of developing the condition if newborn gets erythromycin or azithromycin in first 14 days after birth.

I Clinical Features Common in first born males (4:1). Incidence is 4 in 1000 births; It is familial. It is seen between 3rd and 6th weeks of age of an infant, the time taken by the hypertrophied muscle to cause complete obstruction. Vomiting-forcible, projectile and non-bilious. Visi ble gastric peristalsis (VGP).

Fig. 20.10: Visible gastric peristalsis.

Palpable lump of hypertrophied pylorus which is better felt from left side, as a mobile, smooth , firm, olive like mass, with all borders well made out, moves with respiration , with impaired resonance on percussion. It is the most important clinical feature (95%).

:::c

>

"'ti

-4 m ::::z:, N

In premature infants: VGP and mass is better seen and felt. Vomiting is regurgitant. Anorexia is common. Diagnosis is established by: Clinical examination. Ultrasound abdomen (very useful)-Doughnut sign. ,, Pyloric muscle 4 mm or more in thickness. , Length of pyloric canal >14 mm. , Cervix sign on long axis, target sign on short axis. Barium meal shows obstruction. Contrast study is not commonly done; but when it is done, it shows 'string sign' or 'railroad track sign' or 'double track sign' with pyloric obstruction.

I Differential Diagnosis Duodenal atresia (Bilious vomiting is present). High intestinal obstruction (e.g. volvulus neonatorum). lntracranial haemorrhage.

I Treatment 1. Correction of dehydration and electrolyte imbalance. 2. Surgery. :.- Ramstedt's operation-After laparotomy, hypertrophied muscle is cut along the whole length adequately until the mucosa bulges out. Mucosa should not be opened (pyloromyotomy). If mucosa is injured, it should be sutured horizontally using interrupted vicryl or silk sutures. , Laparoscopic pyloromyotomy is becoming popular. 3. Endoscopic pyloromyotomy is also tried (alike POEM for achalasia cardia). 4. Balloon dilatation is tried but results are not optimum like surgery or myotomy.

B Postoperative pyrexia (Hyperthermia). Gastroenteritis; Electrolyte imbalance Note: • Medical treatment: Not advisable as cure is not guaranteed. Atropine methyl nitrate orally is tried to relax the pylorus muscle.

Pleasure in the job puts perfection in the work.

C)

Cl)

c 3 QI n

:::r

812

Reflux Gastritis Usually occurs after gastric surgeries. Prokinetic drugs are useful-metochlopramide, domperidone, cisapride, mozapride.

Erosive Gastritis

en C0

a:

en

Figs. 20.11 A to D: (A) Ramstedt operation for congenital hypertrophic pyloric stenosis. Note that here muscular layer is cut but not mucosal layer; (B to D) Ramstedt pyloromyotomylor congenital hypertrophic pyloric stenosis. Only muscular layer is cut to allow mucosa to bulge out.

GASTRITIS

I Types 1. 2. 3. 4. 5.

Type A gastritis. Type B gastritis. Reflux gastritis. Erosive gastritis. Others. Stress gastritis, lymphocytic gastritis, granulomatous gastritis, phlegmonous gastritis.

It occurs due to disturbed gastric mucosal barrier. It is induced by NSAID/alcohol. The precursor of prostaglandins, arachidonic acid , is catalysed by the two cyclo-oxygenase isoenzymes, cyclooxygenase-1 and cyclo-oxygenase-2 (COX 1 and COX 2). Cyclo-oxygenase-1 maintains the homeostasis of organs. Cyclo-oxygenase-2 is the inflammatory enzyme. NSAIDs can inhibit both cyclo-oxygenase 1 and 2 pathways. The antiinflammatory properties of NSAIDs are mediated th rough inhibition of cyclo-oxgenase-2 and adverse effects, such as erosive gastritis, gastric and duodenal ulceration, occur as a result of effects on the cyclo-oxygenase-1. Selective COX 2 mediated NSAIDs will not cause erosive gastritis. Prostaglandin is cytoprotective. Erosive gastritis is treated by-IV ranitidine; IV omeoprazole/ Pantoprazole or sucralfate. Mesoprostol is PG E1 analog which increases the mucosal resistance but it is costly and causes diarrhea. Note: • Stress gastritis: It is due to stress induced reduction in blood supply of superficial part of the gastric mucosa usually seen in ICU patients, in major illness or major injury. IV pantoprazole/ranitidine can prevent it. • Lymphocytic gastritis. It is associated with H. pylori infection; it is rare; there is T cell infiltration of gastric mucosa. • Granulomatous gastritis-. It is seen in Crohn's disease and tuberculosis; it is very rare. • Phlegmonous gastritis. It is due to severe bacterial infection of the stomach. It is rare but dangerous. It is seen hypochlorohydria, HIV infection. There is necrosis and ischaemiaof mucosa or deeper layers of the stomach leading into gangrene, necrosis and perforation of stomach. Condition carries high mortality.

Type A Gastritis Autoimmune disease. There is formation of antiparietal cell antibodies. Parietal cell dysfunction occu rs causing achlorhydria and vitamin B12 deficiency. Antrum is not affected. 'G' cell hyperplasia occurs with raised serum gastrin level. There is formation of microadenoma of enterochromaffin like cells (EGL cells) with predisposition to gastric carcinoma.

Type B Gastritis Occurs due to Helicobacter pylori infection. Antrum is affected. Peptic ulcer is common. Helicobacter related pangastritis commonly occurs which may turn into gastric cancer.

Fig. 20.12: Endoscopic view of erosive gastritis.

-

-

-

- ---

-

Atrophic gastritis, duodenogastric bile reflux, gastric stasis, abnormalities in acid and pepsin secretion. Acid becomes ulcerogenic even to normal gastric mucosa. Smoking, alcohol, NSAIDs, steroids. Helicobacter pyloriinfection (70%). There is either normochlorhydria or hypochlorhydria. Altered mucosal barrier mechanism. Lower socioeconomic group.

NONULCER DYSPEPSIA

• Symptom complex with pain and discomfort in the upper abdomen • It is intermittent upper abdomen pain in the absence of peptic ulceration • It occurs in 25% of population-large number • Anatomical or biochemical abnormalities are not discovered in this condition • H. pylori is not associated with this condition • Often it lasts for long time decreasing the quality of life • Differential diagnosis- GERO/acid peptic diseases/gallstones/ pancreatitis/carcinoma • H. pylori eradication is not required and there is no surgical role

813

I Factors Involved in Gastric Ulcer Formation 1

ACUTE PEPTIC ULCER (DUODENAL OR GASTRIC ULCER) They are usually multiple erosions due to disruption of the mucosal barrier. Causes: Stress, drugs like analgesics, steroids, surgeries. Features Sudden onset of acute pain and tenderness in epigastric region. Vomiting with or without haematemesis. .,. Often acute peptic ulcers can lead to perforations. Treatment :.- Intravenous ranitidine 50 mg, 8th hourly. IV fluids. IV pantoprazole/rabeprazole/omeprazole . .,. Blood transfusions if there is bleeding. Most of the time surgery is not required for acute ulcers. During follow-up patients are advised to take anti ulcer drugs for 4-6 weeks- ranitidine, omeprazole or lansoprazole.

Curling's Ulcers They are acute ulcers which develop after major burns, presenting as pain in epigastric region, vomiting or haematemesis. Treatment is conservative-IV ranitidine. IV pantoprazole 80 mg in 100 ml DNS-slow, later 40 mg IV maintenance. Curling's ulcer occurs when burn injury is more than 35%. It is observed in the body and fundus not in antrum and duodenum.

Cushing's Ulcers They are acute ulcers which develop after cerebral trauma or after neurosurgical operations. It is commonly single, deeper ulcer more frequently perforates. It can occur in oesophagus and duodenum also. Treatment is conservative by IV ranitidine.

GASTRIC ULCER

I Aetiology

It occurs due to imbalance between protective and damaging factors of gastric mucosa.

Duodenogastric reflux-reflux containing bile salts and lysolecithin break the mucosal barrier making it more vulnerable for injury, action of drugs and pepsin injury. Gastric stasis. lschaemia of the gastric mucosa. Type II and Ill gastric ulcers show acid hypersecretion.

Figs. 20.13A and B: (A) Barium meal showing Niche in the lesser curve as benign gastric ulcer; (B) Benign gastric ulcer endoscopic view. Biopsy is a must. Ideally 10 biopsies should be taken from the edge.

I Pathology Gastric ulcer is large in size, usually lies in the lesser curvature, its floor being formed by the muscular layer. Posteriorly it may penetrate into the pancreas; it may cause torrential bleeding by eroding left gastric (commonly) vessles or splenic vessels or vessels in the gastric ulcer wall. Anteriorly it may perforate or penetrate into the liver. It may lead into hour glass contracture, or tea-pot deformity. Microscopically, it shows ulcer crater with chronic inflammatory cells and granulation tissue, endarteritis obliterans and epithelial proliferation. (Ulcer to the right of the incisura is malignant unless proved otherwise). Gastric ulcer >3 cm is called as giant gastric ulcer. It has got 6-23% chances to turn into malignancy. Incidence of perforation and bleeding is also very high. Endoscopy and biopsy should be done to rule out malignancy. Follow-up endoscopy is a must if drug therapy is used for treatment to confirm the complete healing of the ulcer. If complete remission has not occurred then partial gastrectomy should be done. Grossly, margin of the benign gastric ulcer is clear; deep; near lesser curve; edge is not everted with gastric mucosa! folds converging towards the base of the ulcer.

Troubles waste the stomach like rust waste iron.- Croatian Proverb

Cl)

0 3

Q) C")

:::r

814

... Q)

en

::::,

Cl)

0

Fig. 20.14: Specimen of stomach (identified by the mucosa! pattern and rugae) showing deep ulcer near lesser curvature. Margin of the ulcer is clear, not everted with gastric mucosal folds converging towards the base of the ulcer. 95% of benign gastric ulcer occurs towards lesser curve. Benign gastric ulcer is more common in lesser curvature, as it takes more burden of passage of food and so more of wear and tear. Benign gastric ulcer is rare in greater curvature, fundus and cardia. Histologically it shows destruction of epithelial lining; proliferation of margin; destruction of the part of the muscle layer; granulation tissue in the floor; infiltration with chronic inflammatory cells; endarteritis and fibrosis in the base.

Ninty-five per cent of benign gastric ulcer occurs towards lesser curve, as it takes more burden of passage of food and so more of wear and tear. Benign gastric ulcer is rare in greater curvature, fund us and cardia. Acute ulcer: It is confined to mucosa and submucosa. It is commonly due to NSAIDs. Chronic ulcer: It penetrates muscularis layer of stomach.

I Clinical Features Equal in both sexes. It is becoming more common in females. Common after the age of 40 years. Pain in epigastric region after taking food, lasting up to two hours. Pain is uncommon during night. It is relieved by vomiting or by inducing vomiting. Periodicity Symptom free interval may be 2-6 months. Often with seasonal variation. Vomiting relieves pain and often it is induced by the patient for relief of pain. Haematemesis and melaena: Haematemesis is more common. Appetite is good but hesitant to eat, because eating induces pain and that results in loss of weight. But once complications occur, appetite decreases. Aversion to spicy, fried foods

Fig. 20.15: Types of gastric ulcer (Daintree Johnson). Note: • Often in lesser curve, saddle-shaped ulcer can occur. • Type V gastric ulcer is-ulcer anywhere in the stomach associated with NSAIDs.

B Hiatus hernia Cholecystitis Chronic pancreatitis

Chronic gastritis Dyspepsia Carcinoma stomach.

I Investigations Barium meal X-ray to see niche and notch.

Niche on the lesser curve with notch on the greater curvature Ulcer crater projects beyond the lumen of the ulcer Regular/round margin of the ulcer crater-stomach spoke wheel pattern Overhanging mucosa at the margins of a benign gastric ulcerprojects inwards towards the ulcer- Hamptom's line Converging mucosa! folds towards the base of the ulcer Symmetrical normal gastric mucosa! folds

Gastroscopy is done to see the location, type of ulcer and also to take biopsy (1 Obiopsies). Ultrasound abdomen mainly to rule out other diseases and to confirm associated diseases.

occurs.

On deep palpation, tenderness is felt in epigastric region.

T Type

'

Types of gastric ulcer (Dainlree Johnson)

Location

Type I In the antrum, near the lesser curve Type II Combined gastric ulcer (in the body) with duodenal ulcer Type Ill Prepyloric ulcer Type IV Gastric ulcer in the proximal stomach or cardia

Incidence Acid level 55% 25%

Normal High

15% 5%

High Normal Fig. 20.16: Multiple ulcers visualised on gastroscopy.

815

C")

::c > -a -t

m ::a N c::::,

Figs. 20.17A and B: (A) Gastric ulcer in the body of the stomach; (B) Gastric ulcer in prepyloric region.

-

en 0

3

I»

n

::r

Pauchet's procedure

Kelling madlener procedure

Csendes procedure

Fig. 20.19: Diagram showing Pauchet's, Kelling Madlener, Csendes operations for proximal Type IV benign gastric ulcer.

I Complications of Gastric Ulcer Figs. 20.18A and B: Barium meal study showing niche and notchgastric ulcer.

I Treatment Drugs like H2 blockers, proton-pump inhibitors, carbenoxolone {biogastrone, sucralfate, prostaglandins which coats the ulcer and so creates a mucosal barrier) helps in reducing or eliminating the symptoms. But asymptomatic ulcer may exist silently and may turn into malignancy. So surgery is the preferred line of treatment. Partial gastrectomy and Billroth I gastroduodenal anastomosis is done. Type IV proximal gastric ulcer is difficult to manage. It is treated by subtotal gastrectomy. Often distal gastrectomy with selective sleeve like extension cut along the lesser curve to remove the ulcer is done- Pauchet's procedure. Other surgical procedures: 1. de Miguel's antrectomy Distal antrectomy, pylorectomy with excision of ulcer along with gastroduodenal anastomosis is done. It preserves gastric reservoir function, shows less recurrence rate and less operative morbidity. 2. Maki's pylorus preserving gastrectomy: Hemigastrectomy with excision of pyloric ulcer but retaining 2 cm pre-pyloric stomach. It is only used in type I gastric ulcer. Even though it has got fewer incidences of postoperative diarrhoea and dumping, it has got high recurrence rate. 3. HSV with excision of ulcer. 4. Kelling Madlener procedure: It is antrectomy and excision of proximal gastric ulcer Type IV. 5. Csendes procedure: It is subtotal gastrectomy with sleeve extended resection along the lesser curve for type IV proximal gastric ulcer.

1. Hour glass contracture: It, occurs exclusively in women, is due to cicatricial contracture of lesser curve ulcer. ,, Here stomach is divided into two compartments. ,, Clinical features: Loss of periodicity; Persistent pain; Vomiting; Loss of appetite and weight.

Fig. 20.20: Hour glass contracture.

:.- Diagnosis: Barium meal: It shows filling only in the proximal stomach or double pouched stomach; Gastroscopy. ,, Treatment: Partial gastrectomy wherein gastric ulcer with lower compartment of the stomach is removed and Billroth I anastomosis is done. 2. Tea-pot deformity(Hand-bag stomach): It is due to cicatrisation and shortening of the lesser curvature; They present with features of pyloric stenosis; Treatment is partial gastrectomy with Billroth I anastomosis.

Fig. 20.21: Tea-pot deformity.

It is consequences which are more important than rewards or punishments.

816

3. Perforation-most frequent. 4. Bleeding by erosion into the left gastric and rarely splenic vessels or to vessels in the wall of ulcer-35%. It is common in type 11 and 111 gastric ulcers. 5. Penetration posteriorly into pancreas, anteriorly into liver. 6. Malignant transformation usually into adenocarcinoma of stomach (5-10%). Benign gastric ulcer

Malignant gastric ulcer

Mucosal folds

Converging mucosal folds Effacing mucosal folds up to the margin

Site

95% lesser curve

Greater curvature

Margin

Regular margin

Irregular margin

Floor

Granulation tissue in floor Necrotic slough in floor

Edge

Not everted; punched or sloping

Everted edge

Surrounding Surrounding area and rugae are normal area

Surrounding area shows nodules, ulcers and irregularities

Size and extent

Large and deep

Small, deep up to part of muscle layer

DUODENAL ULCER

I Aetiology

Common in people with blood group O +ve. Stress, anxiety-'hurry, worry, curry'. Helicobacter pylori infection is an important aetiology for duodenal ulcer (90%). NSAIDs, steroids. Endocrine causes: Zo llinger-Ellison syndrome , MEN syndrome, hyperparathyroidism. Other causes: Alcohol, smoking, vitamin deficiency. Dragstedt dictum: "No acid - No ulcer".

Eventually it shows cicatrisation causing pyloric stenosis. Serosa overlying the site of duodenal ulcer shows petechial haemorrhages with speckled red dots, appearing like sprinkled cayenne pepper. Microscopically, ulcer with chro nic inflammation with granulation tissue, gastric metaplasia of duodenal mucosa, endarteritis obliterans are visualised. Sometimes two opposing ulcers, i.e. over anterior and posterior surfaces of duodenum are present and are called as kissing ulcers. An anterior ulcer perforates commonly, posterior ulcer bleeds or penetrates commonly.

I Clinical Features In India, ratio of duodenal ulcer (DU) to gastric ulcer is 30: 1. A very high incidence. It is common in all socioeconomic group, more with stressed professionals (Type A personality). Pain is more before food, in early morning and decreases after taking food. It is classically called as hunger pain as it is relieved by taking food. Night pains are common. Common in males. Periodicity is more common than in chronic gastric ulcer with seasonal variation. Water-brash, heart burn, vomiting may be present. Melaena is more common, haematemesis also can occur.

T

Differences between clinical features ol gastric ulcer and duodenal uleer

Gastric ulcer

Duodenal ulcer

Pain after food intake

Pain before food intake

Periodicity less common

Periodicity more common

Haematemesis more common

Malaena more common

Weight loss occurs

Weight gain occurs

Equal in both sexes

Common in males

Appetite is good and there is gain in weight. It decreases once stenosis develops. Eats more frequently without any restriction. Chronic duodenal ulcer can be uncomplicated or complicated.

I Complications of Duodenal Ulcer Figs. 20.22A and B: (A) Anatomical location of chronic duodenal ulcer; (B) Serosal surface in a chronic duodenal ulcer. It appears like cayenne pepper.

I Pathology Ulcer occurs in the first part of duodenum, usually with in the first inch.involving the muscular layer. Sites: (a) In the bulb (bulbar)-95%; (b) Post-bulbar (5%).

Pyloric stenosis (10%): Due to scarring and cicatrisation of first part of the duodenum. Bleeding (10%). Perforation (5%). Both acute and chronic ulcers can perforate. Anterior ulcers perforate. Residual abscess. Penetration to·pancreas. Intractability: It is an ulcer that has not healed after 8 weeks of full-dose treatment with anti-ulcer drug(s). Note:

• Chronic duodenal ulcer will not turn into malignancy. • Ulcer which is more than 2 cmis called as giant duodenal ulcer.

I Investigations Barium meal X-ray shows deformed or absence of duodenal cap (because of spasm). Appearance of 'trifoliate'duodenum is due to secondary duodenal diverticula which occurs as a result of scarring of ulcer.

Fig . 20.23: Absence of duodenal cap-chronic duodenal ulcer.

Gastroscopy reveals the type, location of ulcer, narrowing if any. Biopsy also can be taken to look for the presence of Helicobacter pylori. Usually biopsies are taken from duodenum, pylorus, antrum, body, fund us, and confirmed by rapid urease test or C13 or C14 breath tests. Estimation of serum gastrin level, serum calcium level.

Fig. 20.24: Endoscopic view of duodenal ulcer.

B

DIFFERENTIAL DIAGNOSIS

• Carcinoma stomach (pylorus) • Dyspepsia due to other causes: Hiatus hernia; Oesophagitis; Cholecystitis; Chronic pancreatitis

I Treatment Aim of therapy: To relieve symptoms; to heal ulcer; to prevent recurrence. General measures: Avoid alcohol, NSAIDs, smoking, spicy foods. Have more frequent food.

Specific measures: ,, lntragastric pH should be maintained above 5.

I Drugs H2 Blockers: :.- Promotes ulcer healing in 4-8 weeks, by reducing acid secretion. - Tab cimetidine. - Tab ranitidine (300 mg HS or 150 mg BID), (IV preparation is available). - Tab famotidine (IV is available) Most potent H2 blocker. Dose is 20----40 mg/day. Tab roxatidine; Tab nizatidine. Proton-pump inhibitors: ,. Inhibit parietal cell W , K+ ATPase enzyme responsible for acid secretion. They are used for 6-12 weeks. They stop acid secretion completely. - Omeprazole 20 mg OD 1 hour before food-IV preparation is available. - Esomeprazole 40 mg. - Lansoprazole 30 mg, llaprazole 1Omg - Pantoprazole 40 mg-IV preparation available. - Rabeprazole 20 mg-IV preparation available. Antacids: , Neutralises the HCI to form water and salt and also inhibits peptic activity. Aluminium hydroxide and magnesiu m trisilicate are commonly used. Dose is 2 grams 2 hours after food. Aluminium hydroxide causes constipation, magnesium trisilicate causes diarrhoea. Osteomalacia, milk alkali syndrome, rebound ulcer due to gastrin release are other complications. Sucralfate: ,, It is an aluminium salt of sulfated sucrose which provides a protective coat to ulcer crater thereby promotes healing. It inhibits peptic activity. , It binds to ulcer bed and stays for 12 hours; prevents back diffusion of hydrogen ion; raises endogenous prostaglandin level in tissues; binds bile acid and pepsin; prevents colonisation of gastric mucosa by bacteria. ;., Dose is 1 g qid for 6 weeks (before food). It is an effective drug. Anti-Helicobacter pylori regime: ;., It is very useful, given for 7-14 days-later the protonpump inhibitors are continued. ;., Triple or quadruple (tetracycline, bismuth, tinidazole, pantoprazole) regimes are used. Colloid bismuth sulphate is a good drug for ulcer, but it stains the oral cavity and mucosa. Misoprostol (200 mg tid) is the only prostaglandin agonist accepted. ;., PG E1 (mesoprostol) and E2 increase mucus and bicarbonate secretion, improves mucosal blood flow, but reduces acid secretion.

Every man is the master of his own fortune.

817

c:-,

:c

>

"'tJ --4

m

:::D N

c:::,

en Cl

3

Q)

c:,

:-

818

T

I

Anli-Helicobacter regime (Triple regime)

Clarithromycin 500 mg BO

Metronidazole 400 mg BO

Omeprazole 20 mg BO

OR

OR

OR

Amoxycillin 500-750 mg BO

Tinidazole 600 mg BO

Lansoprazole 30 mg BO

OR

OR

Tetracyclines, or Bismuth, etc.

Pantoprazole 40 mg BO

Follow-up gastroscopy is a must, to confirm that ulcer has healed. Note: Antacids and H2 blockers should not be used along with PPI as these drugs will reduce the action of PPls by creating alkaline media.

I Surgery for Uncomplicated Duodenal Ulcer Indications for surgical intervention for chronic DU (Uncomplicated DU): 1. Uncomplicated DU, not responding to drug therapy of 8-12 weeks-intra ctable duodenal ulcer 2. Repeated recurrences Presently most of the uncomplicated DU does not require surgery

Highly selective vagotomy (HSV). , In HSV, only fibres supplying the parietal cells are ligated. Nerve of Latarjet which supplies the antrum pump is retained and so no drainage procedure is required in HSV. HSV is also called as parietal cell vagotomy or superselective vagotomy. Here nerve fibres in last 6 cm of stomach, just proximal to pylorus are preserved (Crow's foot). Vagotomy reduces acid secretion, hence ulcer heals. No acid, No ulcer. , HSV was first described by Amdrup and Johnston in 1969. , Distal 6 cm oesophageal nerve fiber clearance is essential. Fibers up to 6 cm proximal to pylorus in stomach are cleared; nerve of Latarjet is preserved; adequate distal greater curve clearance is essential. , lntraoperative test tor completion of vagotomyshould be done-Grassi test or insulin test. , It has got distinct advantages-low operative mortality (0.2%) and postoperative morbidity (0.5%); postvagotomy diarrhoea and dumping syndrome is very low; chances of developing anaemia, weight loss, osteoporosis, tuberculosis, and carcinoma are very less. , Problems-lesser curve necrosis due to ischaemia; recurrent ulcer 10- 15% in 1Oyears. Selective vagotomy with pyloroplasty (SV + P). •·· Truncal vagotomy with gastrojejunostomy (TV + GJ). Posterior truncal vagotomy with anterior seromyotomyTaylor's operation. It can be done through laparoscopy. Vagotomy with antrectomy. Gastrin producing antrum, vagal cholinergic pathway from ulcer bearing area is removed with

gastroduodenal anastomosis. Ulcer recurrence is very low but has got morbidity. Posterior truncal vagotomy with HSV without drainage procedure (Kim's) often through laparoscopy is also done. Linear gastrectomywith posterior truncal vagotomy through laparoscopy. Most of these procedures presently can be done through laparoscope. Note: • Presently, there is no role of gastrectomy or gastrojejunostomy (Just GJ) for uncomplicated DU. • A peptic ulcer is an excavated defect in the gastric or duodenal mucosa that extends through the muscularis mucosa into the deeper layers of the wall. • A refractory peptic ulcer is defined as an endoscopically proven ulcer greater than 5 mm in diameter that does not heal after 12 weeks of treatment with a proton-pump inhibitor. • A recurrent peptic ulcer is defined as an endoscopically proven ulcer greater than 5 mm in diameter that develops following complete ulcer healing.

PYLORIC STENOSIS DUE TO CHRONIC DUODENAL ULCER

I Pathology Chronic DU after many years undergoes scarring and cicatrisation causing total obstruction of the pylorus, leading to enormous dilatation of stomach.

I Clinical Features Pain is severe, persistent, in epigastric region, and also with feeling of fullness. Vomiting-large quantity, foul smelling and frothy, vomitus contains food consumed on previous day (partially digested or undigested food). Loss of periodicity. Loss of appetite and weight. Visible gastric peristalsis (VGP)- may be elicited by asking the patient to drink a cup of water. Positive succussion splash which is done with 4 hours empty stomach, by placing a stethoscope over the epigastric region and shaking the patient adequately. Auscultopercussion test shows dilated stomach. Test is done by placing a stethoscope over the epigastric region. Skin is scratched from left side downwards, at several points away from the epigastrium (towards left side) using finger and these points are joined. Normally greater curvature of stomach is above the level of umbilicus (midway between the umbilicus and epigastrium). In gastric outlet obstruction it lies below the level of the umbilicus (Stomach we see; stomach we feel; stomach we hear). Confused status because of alkalosis and electrolyte changes. Electrolyte changes. Because of vomiting, hypochloraemic, hyponatraemic, hypokalaemic, hypocalcaemic, hypomagnesaemic alkalosis occurs. It causes paradoxical aciduria.

Mass is never palpable. Goldstein saline load test- halt an hour after installation of 750 ml of saline, if volume remained and if more than 250 ml, suggest obstruction.

I Investigations Barium meal study: Absence of duodenal cap. , Dilated stomach where greater curvature is below the level of iliac crest. ,.. Mottled stomach. , Barium will not pass into duodenum. Gastroscopy to rule out carcinoma stomach and to visualise the stenosed area. Electrolyte study for correction of electrolyte imbalance. ECG to check for hypokalaemia.

I__Treatment __;_ _ _ _ _ _ _ _ _ _ _ _ _ _ _ _ __ Correction of dehydration and electrolytes by IV fluidsnormal saline or double strength saline, calcium, potassium, magnesiu m. Blood transfusion is given if there is anaemia. TPN support. Stomach wash to clean the stomach contents (using normal saline) is given using stomach tube like Eswald's. It also reduces the oedema of stomach wall and improves gastric emptying time by increasing the gastric muscle tone. Surgery , HSVwith gastrojejunostomyis present recommendation even though it is technically difficult. HSV is better than TV as it maintains the nerve supply of the chronical ly obstructed antrum and so may eventually reduce the chronic emptying problems. , Truncal vagotomy along with gastrojejunostomy of Mayo (posterior, vertical/oblique, short loop, retrocolic, isoperistalsis) is done-ideal, commonly advocated procedure. ,.. Vagotomy, antrectomy (acid secreting area) with Billorth I anastomosis along with feeding jejunostomy for nutrition is the other option.

Iliac crest

Fig. 20.25: Pyloric stenosis with gastric dilatation.

Fig. 20.27: Gastric outlet obstruction showing dilated stomach on table.

Figs . 20 .26A and B: Barium meal pictures showing gastric outlet obstruction.

I Differential Diagnosis Carcinoma pylorus- here mass may be palpable.

B •

Congenital

• Chronic DU-fibrosed/cicatrised • Carcinoma pylorus • Adult pyloric stenosis- it is treated by pyloroplasty (not by pyloromyotomy) • Pyloric mucosal diaphragm- it should be excised surgically or endoscopically

Fig. 20.28: Truncal vagotomy and gastrojejunostomy. It is posterior, vertical, short loop, retrocolic, isoperistaltic GJ of Mayo.

It is not sufficient if one becomes great leader or great follower; but more importantly one should be great thinker.- Albert ECoates

819 n

::::c

>

-a

-4

m

:a N

Q

en c :,

3

I»

"

::I"

820

with sodium). Here, due to hyponatraemia, body conserves sodium and so bicarbonate is secreted along with hydrogen ion. So urine becomes acidic. It is called as paradoxical aciduria.

I Features

Fig. 20.29: Gastrojejunostomy stoma view on endoscopy.

Note: Metabolic changes are not severe in carcinoma pylorus as it is seen in pyloric stenosis due to chronic duodenal ulcer because in carcinoma stomach often there is hypochlorhydria or achlorhydria.

Right and left vagi

Antrectomy (hemigastrectomy) Part retained

Fig. 20.30: Vagotomy and antrectomy. It is occasionally used for intractable resistant duodenal ulcer or chronic duodenal ulcer with obstruction. HSV is commonly used in intractable cases. GJ with vagotomy is commonly used in pyloric stenosis due to chronic duodenal ulcer. Note: • Often gastric emptying may be delayed for 2--4 weeks after surgery in pyloric stenosis. It usually recovers in 7 days. • After recovery eradication of H. pylori infection is routinely done even though infection may not be evident in many of patients with outlet obstruction. • No role of pyloroplasty or only HSV in a scarred duodenum-as it can cause disruption and bile leak. • Gastrectomy and other procedures are usually not necessary in pyloric stenosis. • Endoscopic balloon dilatation of the stenosed area benefits only tempo· rarily with high recurrence rate.

I ELECTRDL YTE CHANGES IN PYLORIC STENOSIS Hyponatraemia Hypokalaemia Hypomagnesaemia

•·· Irritability, confused status, dehydration. Often convulsions can occur. Features of alkalosis like rapid breathing-Cheyne-Stokes breathing and tetany. Investigations: Serum electrolytes; Arterial blood gas analysis; Serum calcium level estimation. Treatment Normal saline is infused; often double strength normal saline is used; slow correction is important. IV potassium slowly under ECG monitoring; IV magnesium, IV calcium is also used. ,.. The cause is treated.

Hypochloraemia Metabolic alkalosis Paradoxical aciduria.

Because of severe vomiting which contains acid as well as undigested retained food, sodium, chloride, potassium, magnesium levels drop, causing metabolic alkalosis. Alkalosis can lead to hypocalcaemia causing tetany. It is called as 'gastric tetany'. To control alkalosis, kidney secretes t:xcess bicarbonate (In alkalosis without hyponatraemia, bicarbonate is secreted along

PERFORATED PEPTIC ULCER It is the terminology used for perforation of duodenal ulcer or gastric ulcer or stomal ulcer. Otherwise all clinical features and management are similar. Perforation is common in duodenal ulcer (75% of perforated peptic ulcers). Mortality is more in gastric ulcer perforation and perforation in elderly. Note: Gastroduodenal perforation (GOP) is a different terminology.

• GDP is the most common perforation among GI perforations - 32%; 2nd being appendicular -24%. Incidence of GD perforation remained same in last 2 decades and increased in elderly. Mortality is 6· 14% in GDP patients with morbidity >17%. Advanced age (greater than 70 years) is associated with a higher mortality with rates of approxi· mately 41 %. • The number of perforated ulcers remains unchanged. Sustained inci· dence possibly due to increased NSAIDs in elderly. 80% of perforated duodenal ulcers are H. pylori positive. • Men are much more affected than women • Ratio is approximately 12 : 1 to 20 : 1. • Causes are- Nontraumatic: Gastric ulcer, duodenal ulcer (peptic ulcers - most common); obstruction; ischemia; malignancy. Traumatic• lalro· genie - endoscopies; ERCP; foreign body; others - like ZE syndrome. • Precipitating factors: Helicobacterpylori infection; Aspirin, other NSAID ingestion; smoking, alcohol.

• 65% occurs in duodenum; 25% occurs in pylorus; remaining are stomal, proximal gastric. • Classiticatiorr. (1) Minor/small-3 cm. • Features are-shock, peritonitis, stages, SIRS, MODS; Nonspecific presentations; in mental illness and spinal cord injury patients difficult to elicit any typical features. • May mimic appendicitis, cholecystitis, bowel ischaemia and pancreatitis. • Boey scoring is used commonly- based on parameters: Concomitant severe medical illness (1 score); Preoperative shock (1 score); Duration of perforation >24 hours (1 score). Four distinct degrees are-0, 1, 2, 3. Increase in each degree increases the mortality and morbidity. • Evaluation: Only at later period blood parameters will alter. However all blood investigations like haematocrit, liver and renal function tests,

blood sugar should be done. Chest X-ray with abdomen or Left lateral decubitus is basic investigation; CT scan abdomen is idea~air (extraluminal) in the peritoneum; low attenuation cleft; air bubble density; dirty fat sign; oedematous wall are the findings. • Surgical treatment is the choice. Perforation more than 24 hours old; hemodynamic instability, features of shock, leukocytosis, patients above 70 years and failure of NOM. - Simple closure of the perforation using silk or vicryl interrupted sutures through the perforated edges. - Ce/Ian Jones (1929) technique-. Pedicled omental patch is placed (plugged) over the perforation and interrupted sutures are placed. It is ideal and commonly practiced. Karanjia technique: modified Cellan-Jones omental pedicle is secured to the tip of a NGT passed through the perforated duodenal ulcer. - Roscoe Graham's patch (1937): Perforation is plugged with free omental patch (not pedicled) and sutured over it. - Modified Graham's patch/operation: Primary closure with interrupted sutures and placement of pedicled omental patch over the sutures which is again fixed with loose sutures. - Ulcer excision with closure- in gastric ulcer; but here again closure may be sufficient after taking biopsy from the edge. Primary closure

Primary closure with omental patch modified Graham's

Cellan Jones repair (1929)

'- -'

Graham's patchplugging the free omental patch {1937)

Fig. 20.31: Different techniq ues used in gastroduodenal perforations. Non-operative management (NOM): Conservative treatment is known as the Taylor method (1946) and consists of nasogastric aspiration, antibiotics, intravenous fluids and nowadays H. pylori triple therapy. Indications are: The onset of symptoms of less than 24 hours, mild abdominal pain with minimal peritoneal irritation, hemodynamic stability and absence of systemic signs of sepsis in a patient under the age of 70. Close observation and decision for surgical intervention when needed should be done. • Definitive surgery: Definitive ulcer surgery is no longer required in the majority of patients, as recurrence rates have dropped dramatically with postoperative medical therapy. Formal gastric resection with reconstruction (Billroth I, Billroth II, roux-en-Y) with or without vagotomy is rarely required, and is used in less than 10% of cases. In patients with a recent (60 years: Pulse rate >110/minute; Blood pressure

l'TI

::c N

=

-

en C)

3

QI

c:,

::r

834

I Features Acute epigastric pain Violent ineffective vomiting-retching Inability to pass a nasogastric tube

Types: Acute; Chronic recurrent (It is common type). Complications: Perforation; Gangrene of the stomach; Bleeding. Investigations , Plain X-ray chest and abdomen in erect posture will show retrocardiac gas filled viscous with dilated bowel loop in the abdomen. , Barium meal X-ray. , CT scan abdomen. Treatment , Untwisting of the volvulus and gastropexy by fixing the anterior wall of the stomach to anterior abdominal wall. , Gastrojejunal fixation (gastrojejunostomy without a stoma). , Gastrectomy when stomach is gangrenous. , Treating hiatus hernia or eventration. ,. Displacing colon downwards by dividing gastrocolic omentum-Tanner's operation.

GASTRIC POLYP Gastric polyps are observed in 3% of total gastroscopies. 45% of them are found in fundus which does not show any malignant potential. These polyps are often associated with FAP or Gardener's syndrome and colorectal neoplasms; but these gastric polyps perse are non-neoplastic.

Adenomatous polyp is usually single. It is common in antrum. It can be sessile (common) or pedunculated. It is usually 2 cm or more (80%). It has got hyperchromatic nuclei arranged in a picker fence pattern. Size more than 2 cm has got high malignant potential. Inflammatory, hamartomatous and heterotrophic are other gastric polyps of negligible malignant potential. 50% are asymptomatic. Fundal polyps are associated with PPI therapy and familial polyposis. Gastric carcinoids arise from enterochromaffin like cells (EGL). They usually present as small polyps with pernicious anaemia. It may present with pain, haematemesis and gastric outlet obstruction. Gastroscopy is diagnostic.

I Management Gastroscopic biopsy is a must. Bariu m study shows soap bubble or paint brush look. It depends on the size, number, location, type and presence of symptoms. H. pylori eradication should be done in all hyperplastic polyps. Endoscopic resection is used in hyperplastic type more than 1 cm; pedunculated polyps. Snare polypectomy is done. Gastrotomy and resection is needed in endoscopically failed or difficult cases and polyps >5 cm-open or laparoscopic sleeve resection. Partial gastrectomy of multiple polyps bearing area is needed in multiple polyps. Small polyps can undergo surveillance at regular intervals through endoscopy. All gastric adenomatous polyps should be resected due to their high potential for malignancy.

MENETRIER'S DISEASE

Figs. 20.60A and B: (A) Gastric polyp in prepyloric region; (B) Polyp in the duodenum first part.

I Types 1. Hyperplastic polyp-75% common, minimal risk of malignancy (2%). Size usually is 2 cm is potentially malignant (25% chance).

I Features Hyperplastic polyp is randomly distributed throughout the stomach. They are usually less than 2 cm in size, often multiple. Spontaneous regression can occur. Often it is associated with H. pylori infection.

It is an acquired condition with giant gastric mucosal folds (hypertrophic gastropathy) in the fundus and body of the stomach-'cobblestone appearance'. Common in males. Histologically, it shows hyperplasia, mucosal thickening and gastric gland atrophy. Hypoalbuminaemia, anaemia and hypochlorhydria is common. Antrum is not involved. There is over expression of transform ing growth factor alpha (TGFct) peptide which binds to epidermal growth factor (EGF) . There is foveolar surface mucus cell hyperplasia with absence of parietal cells causing excessive mucus production, protein loss and achlor/hypochlorhyd ria. It is associated with CMV infection in children and H. pylori infection in adult.

I Features Epigastric pain, anaemia and weight loss, melaena.

Investigations ,, Serum total proteins, albumin. ,, Gastric function tests. ,. Barium meal studies, gastroscopy.

B Giant folds in fundus and body Gastric gland atrophy Hypochlorhydria

Complications of diverticula-diverticulitis, haemorrhage (most usual), perforation, abscess formation, Lemme/ syndrome (compression of the intrahepatic CBDby the diverticula causing intra- and extrahepatic biliary dilatation with no biliary calculi). Adenocarcinoma can occur within the diverticulum.

Hypoalbuminaemia It may turn into malignancy Antral sparing

:c

>

m N

C)

Cl)

0

3

Cl,) C")

-=

Primary diverticulum 2nd part

DUODENAL DIVERTICULA

Secondary diverticulum 1st part (trifoliate)

Fig . 20.61 : Primary and secondary diverticula of the duodenum.

It is outpouching/prolapse of the duodenal mucosa. It may be mucosa! outpouching of mucosa only through muscularis propria or of all layers. They are quiet common; 25% asymptomatic; only

m

:::0 N

Cl

en

0

3

n ::r

846 Palliative partial gastrectomy is the best palliation Palliative anterior gastrojejunostomy with jejuno-jejunostomy

Devine's exclusion procedure M-8 tube/Celestin tube insertion for proximal stomach growths Endoscopic stenting/dilatation • Laser recanalisation Palliative chemotherapy-FAM regime

en

When serosa is not involved 5-year survival is 50%. When serosa is involved it is 20%. Nodal spread is a bad prognostic factor. Involvement of more than 4 nodes carry poor prognosis. In Japan, prognosis is better due to early diagnosis and better technical and staging approach. Early gastric cancer has got better prognosis. Intestinal type has better prognosis than diffuse type. Size of the tumour (2 cm); tumour depth; and histological type and grading.

B

CCI

cc Cl)

-

- -

-

PROGNOSTIC FACTORS

Early or advanced; Histological grading • Staging; Gross types Lymph node status; Liver secondaries Serosal involvement- an important factor Intestinal type-got better prognosis than diffuse type Ascites; Response to treatment Note:

Fig. 20 .84: Anterior gastrojejunostomy is one of the palliative procedures used in case of inoperable carcinoma stomach. It is anterior, antecolic GJ.

• Recurrence rate is very high in first 3 years after therapy. • If primary tumour can be removed surgically, it is called as resectable tumour. Resectability can be for palliation or along with radical surgery for cure. • Operable tumour means radical gastrectomy with (amenable for) nodal clearance. • Inoperable tumour means not amenable for radical surgery. • Inoperable tumour can be resectable for palliation. • Ro is resection with complete clearance-no residual disease. R1 is resection but with microscopic residual tumour. R2 is resection with macroscopic residual tumour, R3 is unresectable. • D1 and D2 dissections are different for proximal and distal tumours. • In early gastric cancers, laparoscopic endoluminal transgastric mucosal resection of the lesion is under trial now. • Adjuvant chemoradiation (RT with 5-FUas radiosensitiser) is also under trial for proximal tumour, undifferentiated high-grade tumour. • Antrectomy is also called as hemigastrectomy. Removal of 60-75% stomach is called as partial gastrectomy. Removal of more than 80% of stomach is called as subtotal gastrectomy. • Perforation in gastric cancer has got high mortality. • Most common site of recurrence is stomach bed, then lymph node and anastomotic site. • Will Roger's stage migration phenomenon is thorough staging with stage for stage to improve the outcome.

GASTRIC LYMPHOMA Fig. 20.85: Oevine's exclusion procedure. Here inoperable pyloric tumour is left and excluded. Proximal stomach is transected and used for Billroth II like gastrojejunostomy.

I Prognosis In early gastric cancer which has undergone good surgical resection, 5-year survival rate is 70-90% in Japan. In India, 5-year survival rate is 20%. •·· In advanced gastric cancer it reduces to 20-25% in Japan. In other countries it is still worser. •·· Overall prognosis is worse in carcinoma stomach.

There are two types of gastric lymphomas. 1. Primary; 2 Secondary. Note:

• Gut lymphomas are 10% of all lymphomas. It is common in males (2:1 ). Coeliac disease, alpha chain disease, ulcerative colitis, HIV, transplantation-are the aetiology in general for gut lymphomas. H. pylori is relevant for gastric lymphoma. • It occurs in stomach (55%), small bowel (30%), colon (10%), ileocaecal region (5%).

I A. PRIMARY GASTRIC l YMPHOMA Primary gastric lym phoma is the most common one among GI primary lymphomas.

It is the second most commons malignant neoplasms of the stomach (5% of gastric malignancies). It is common in elderly men and common in the antrum of the stomach. ,:. It can be infiltrative, ulcerative, nodular, polypoid or combined. Gastric lymphomas are commonly of non-Hodgkin's type (NHL-B cell type 98%). ,:. It is arising from B cells-derived from mucosal-associated lymphoid tissue (MALT). So often called as MALTOMA Diffuse mucosal thickening which eventually ulcerates-is the pathology. Disease remains in the stomach for long time and later spreads to liver and other lymph nodes. Association of primary gastric lymphoma with H. pylori is well-established. Normal gastric mucosa does not contain lymphocytes but mucosa with H. pylori infection contains lymphocytes. Usually disease is localised to stomach without any other lymph nodes in the abdomen or mediastinum with normal liver, spleen, bone marrow and total count. It is basically a localised disease of stomach to begin with but later to have systemic spread. Main presentation is mass abdomen, pain, vomiting and loss of weight. Perforation, bleeding, obstruction, gastrocolic fistula are also not uncommon. Dawson's criteria for primary GI lymphoma (N HL): No palpable superficial lymph nodes; no thoracic lymph nodes in CT scan; normal WBC count and bone marrow; predominant bowel pathology; liver, spleen are normal. Ann Arbor staging: Stage IE-confined to GIT; IIE - GIT+ regional nodes; IIIE - GIT+ nodes away from and beyond regional; IVE - GIT+ other intra-abdominal organs or extraabdominal. It can be-B cell, T cell, low grade, high grade, pure centroblastic, unclassified. Lugano staging system is widely accepted one for MALTomaEarly. Single primary lesion or multiple noncontiguous primary lesions confined to GI tract with or without nodal involvement; Advanced Disseminated extranodal spread or supradiaphragmatic spread.

I Features Pain abdomen, melaena and mass abdomen which is smooth and firm. Loss of weight, loss of appetite.

B ,

Wiskott-Aldrich syndrome

Ataxia telangiectasia

Klinfelter syndrome HIV, H. pylori (75%)

SLE; MALT Chronic gastritis

Complications: Obstruction; Perforation; Bleeding; Spread to other lymph nodes and liver- only late event.

847

("')

:::z:::

:r:,,

m

::r, N 0

Cl)

0

3

I»

C,

=r

Fig. 20.86: Gastric lymphoma, endoscopic view. It can be primary or secondary. Secondary is common. Primary occurs first in stomach and later other lymphatic system are involved. Gastrectomy is the treatment with chemotherapy.

Investigations ; Gastroscopy and biopsy. ,. Endosonography. ;... Peripheral smear, bone marrow aspiration. ,. CT abdomen and CT chest and mediastinum. Treatment ,. Primary chemotherapy with anti-Helicobacter pylori regime, often with RTis the standard treatment. H. pylori regime with immunotherapy (rituximab with fludarabine) and chemotherapy (oral cyclophosphamide or oral chlorambucil; IV cladribine, bortezomib) is used. , Palliative gastrectomy in case of bleeding, perforation and obstruction is needed. ,. Radical gastrectomy is done in diseases which are confined to stomach only in selected individuals-when primary lymphoma is in antrum, radical subtotal gastrectomy is done. When it is in proximal stomach, radical total gastrectomy is done.

1 B. SECONDARY GASTRIC l YMPHOMA It is the most common type of lymphoma occu rring in stomach. It differs from primary in many aspects. Here mainly system ic lymph nodes are involved first and the disease begins in lymph nodes at different regions, later to occu r in stomach . Peripheral smear shows lymphocytosis; bone marrow shows changes; CT abdomen and chest shows multiple nodal enlargement.

It is basically a systemic disease extending to stomach.

Perforation and bleeding are the complications. Treatment is mainly chemotherapy-CHOP/ABVD/MOPP/ BACOP and other regimes. Surgery is not done unless any local complications occur.

Most intelligent animal is human being, but he happened to be the most cruel animal also.

848

' BGISTiiiiMiMHMMIMf UiiiiMMIIIMWM. . CD 117; CD 34; smooth muscle actin

l

Smooth muscle tumours-CD 34; smooth muscle actin; Desmin Neural tumours---S-100; CD 34

--

----

I

___,

GASTRIC SARCOMAS Stomach is the most common site of GI sarcoma. Leiomyosarcoma is the most common type of gastric sarcoma. Other type is malignant leiomyoblastoma. Leiomyosarcoma is common in proximal stomach. It attains enormous size with rubbery consistency. Mainly spreads through the blood to the liver. Pain, vomiting, anorexia; Mass in upper abdomen. Complication: GI bleeding. Investigations: Ultrasound abdomen and CT scan; Gastroscopy is useful in less than 50% of cases. Treatment: Subtotal gastrectomy; Chemotherapy. Leiomyosarcoma has got better prognosis compared to adenocarcinoma and lymphoma of stomach.

muscle neoplasms. 80% of GISTs are positive for CD34haematopoietic progenitor cell antigen. Mutations occur in KIT (c Kit) as exon 11 (70%), or exons 8,9, 13, 17; it can occur as mutation in PDGFRA (platelet- derived growth factor receptor alpha) in exons 12, 14 and 18. Familial GIST occurs as autosomal dominant with mutation of germline gene KIT or PDGFRA; multiple GISTs in small bowel, colon, stomach are common. It is associated with utricaria and hyperpigmentation. Abdominal pain; weight loss; GI bleed (most common presentation) and large mass abdomen are typical. Mass is extraluminal as it is of submucosal origin but expands and compresses the mucosa. 50% of GIST can present as metastatic disease to liver and peritoneum (ascites). GISTs almost never metastasise to regional lymph nodes. It can be spindle shaped (70%); epithelioid (25%); combination. Carney's triad-extra-adrenal paraganglioma; pulmonary chondroma; gastric GIST.

Fig. 20.87: Gastric sarcomas.

GASTROINTESTINAL STROMAL TUMOURS (GISTs) It is a rare tumour of GI tract-0.2% of all GI tumours. But it is the most common nonepithelial tumour of the gastrointestinal tract. Stomach is the most common site of all GISTs-50%; 25% of all GISTs are from small bowel; rectum-15%; colon-10%. Equal in both sexes and common in 50-70 years age group. GIST arises from interstitial cell of Cajal (pacemaker cell which intercalates between smooth muscle cells and intramural neurons). Mutation of tyrosine kinase and platelet derived growth factor alpha (PDGFa) are the newer pathogenetic theories. GIST is classified as very low-risk (2 cm); low-risk (2-5 cm); intermediate risk (5-10 cm) and high-risk (> 1O cm) based on tumour size and mitotic activity of cells. 95% of GISTs express c-kit-CD117 mutations-a specific molecular marker. Kit protein (CD117, stem cell factor receptor) is a transmembrane tyrosine kinase receptor which is detected by immunohistochemistry. It distinguishes from true smooth

Figs. 20.88A and B: Barium meal X-ray showing gastric teratoma presenting as a large mass lesion compressing gastric lumen. Inset shows CT picture also. Operated specimen is also shown.

Fig. 20.89: Gastric leiomyosarcoma near the fundus-endoscopic

view. It is now classified under GIST.

Investigation: Mainly CT scan. CT shows ulceration, calcification, necrosis, cystic areas, spread , metastases, ascites, etc. Tumour/molecular marker to differentiate it from sarcomas (immunohistochemistry). Endosonography guided biopsy/guided FNAC are important to get histological confirmation . 18 FOG PET scan is very useful adjunct to CT but reserved for difficu lt/equivocal cases. Treatment: Surgical resection of the tumour with part of the adjacent bowel. Resection should be - RO, with intact pseudocapsule, without rupture. lmatinib mesylate-a specific oral drug (year 2000) that inactivates tyrosine kinase kit and so prevents phosphoryla· tion of the receptor and proliferation of tumour is very much beneficial in advanced cases. Now it is also used if tumour size is more than 10 cm; in intraperitoneal rupture/spillage; haemorrhage in GIST; multifocal occurrence. Duration of imatinib is usually one year. Dose is 400 mg/day. lmatinib can also be used as neoadjuvant drug (6- 12 months) for advanced GIST to initial downsizing. Newer drug-SU11248 inh ibits tyrosine kinase receptor as well as blocks PDGFRA. Another newer derivative- suni· tinib and dasatinib are used in imatinib refractory cases. Regorafenib are used in imatinib/sunitinib resistance cases as 160 mg/day for 3 weeks with one week no treatment with repetition of such cycles for 3-6 months. Still more newer drugs are under trial but not approved yet. Doxorubicin ± ifosfamide is tried in metastatic disease but with only 5% response rate. Prognostic factors: Size of GIST more than 5 cm, male sex. High mitotic activity (> 15 mitoses per 30 high power field), mixed pathology (spindle + epithelioid). Liver spread. KIT exon 9 mutation which is more aggressive than KIT exon 11 mutations-carries poor prognosis. Ruptured GIST-nearly all ruptured GISTs show abdominal metastases.

I

GIST can be benign, intermediate/borderline, or malignant. Size and mitotic activity are main factors of malignancy; others arenecrosis, haemorrhage, hypercellularity. GIST is common in fundus of stomach. It can be submucosal (60%), subserosal (30%), intramural. Fundal GIST carries better prognosis than small bowel GIST. Small bowel GIST is common in jejunum (40%), ileum and duodenum. It can be submucosal or subserosal. It presents with GI bleed, abdominal pain, intestinal obstruction, mass abdomen, intussusception, perforation and cachexia. It often produces HCG causing hyperemesis. G/SS-is gastrointestinal stromal sarcoma. It is common in stomach (50%), small bowel. GISS accounts for 2% of soft tissue sarcomas. 70% of them are symptomatic at early stage. Surgical resection is the treatment. _ _ _ _ ____ __J

I

PYLOROPLASTY Indications Bleeding duodenal ulcer. ,, As a drainage procedure after vagotomy in uncompli· cated· DU which is not responding to medical line of treatment. ,, As a part of procedure like Ivor-Lewis operation and Siguira·Futagawa operation. Contraindications , Duodenal ulcer with cicatrisation and scarring. Types ,, Finney's pyloroplasty. ,, Heineke·Mikulicz pyloroplasty. Complications ,, Duodenal leak- very dangerous one. ,, Later reflux. r

•!•

Figs. 20.90A and B: Types of pyloroplasty. (A) Finney's

pyloroplasty; (B) Heineke·Mikulicz pyloroplasty.

GASTRECTOMY

GIST

• GIST is the most controversial topic. • Primary nonepithelial tumours are classified as: Group I-smooth muscle type expressing smooth muscle actin and desmin; Group II-gastrointestinal autonomic nerve tumours (GANs/ myenteric plexus tumour/plexosarcomas) express neuron specific enolase and s· 100 and are aggressive; Group III-dual differentiation; Group IV-uncommitted type.

I Types

1. Billroth I is done for benign condition. Here along with partial gastrectomy, gastroduodenostomy is done. 2. Billro th II is done for carcinoma stomach. After partial gastrectomy, gastrojejunostomy is done and duodenal stump is closed.

Goodness is the permanent investment.

849

C")

:c > -a -4 m :x, N

C)

Cl) C)

3

n =r

Q)

....

en ::I

en 0

3. Lower radical gastrectomyis done in early carcinoma pylorus. Here along with the growth and proximal 5 cm of stomach, omentum, lymph nodes, spleen with tail of pancreas are removed and Billroth 11 anastomosis is done. 4. In growth of upper part or OG junction, upper radical gastrectomy is done along with oesophagogastric anastomosis. 5. In some cases like linitis plastica, total gastrectomy along with oesophagojejunal anastomosis is done.

::I C

:::!E en

a::i

a: en

INDICATIONS

Chronic benign gastric ulcer Benign tumours of stomach (Leiomyoma) Carcinoma stomach Stomal ulcer; Bleeding ulcer Leimyosarcoma, gastric lymphoma; Menetrier's disease.

J

GASTROJEJUNOSTOMY (GJ) 1. Anterior GJ.

2. Posterior GJ. Anterior gastrojejunostomy is done as a palliative procedure in case of advanced inoperable (adherent posteriorly) carcinoma pylorus to palliate vomiting. It is anastomosis between jejunum and anterior surface of stomach, in front of transverse colon (antecolic). Posterior GJ is done along with truncal vagotomy, in pyloric stenosis due to chronic duodenal ulcer. It is posterior, vertical,

retrocolic, short loop, isoperistaltic (of Mayo). GJ is also done as part of the Billroth II gastrectomy.

Fig. 20.93: Gastrojejunostomy stoma view on endoscopy.

Figs. 20.91A and B: Types of gastrectomy. (A) Billroth I

Stomal obstruction; Reflux gastritis Afferent or efferent loop obstruction Dumping syndrome; Duodenal blow-out Retrograde intussusception It is a rare entity occurs after gastrojejunostomy It can occur immediately, in weeks or in months.

_J

anastomosis; (B) Billroth II anastomosis.

RETROGRADEJEJUNOGASTRIC INTUSSUSCEPTION Subtotal gastrectomy (80% or above removed)

Partial gastrectomy(65--75% removed)

Antrectomy (hemigastrectomy)

Fig. 20.94: Retrograde jejunogastric intussusception with Fig. 20.92: Different types of gastrectomy. Subtotal-more than

80% of stomach is removed; partial-60-75% removed.

B Bleeding Bile leak Duodenal blow-out

Gastric fistula Dumping syndrome Anaemia

gangrene-resected specimen.

Jejunogastric intussusception is rare but definitive complication which occurs in loop (omega) gastrojejunostomy only; not in Roux en Y type. 80% cases efferent limb intussusception occurs; 20% cases afferent; rarely both. It can present as acute with featu res of obstruction or strangulation or intermittent with repeated episodes of symptoms.

Presents with pain above and left of the umbilicus, haematemesis, firm, tender mass above and towards the left side. Patient becomes better in erect posture. Condition causes stomal obstruction. Barium meal shows coiled spring look within the stomach remnant. Gastroscopy is diagnostic. It is treated by open reduction of the intussusception with anchoring stitch to the bowel or enteroanastomosis. When gangrenous, resection and Roux-en-Y anastomosis is done.

VAGOTOMY In an inquiry which I had formerly instituted, respecting the functions

rof the stomach, I divided these nerves (the vagi) in the neck of a dog, for the purpose of ascertaining the influence which they possess on the secretion of the gastric juice.. .. We may conclude that the suppression of the secretions...sufficiently demonstrate, that the secretions of the stomach and intestines are very much under the control of the nervous system. -Benjamin Collins Brodie, 1814

T

Complications of vagotomy

lntraoperative

Late complications

Injury to spleen/ oesophagus/liver/ pancreas/thoracic duct stomach Bleeding from phrenic veins/gastric vessels/ perioesophageal vessels Pneumothorax Oesophagogastric disconnection Early postoperative Gastric atony Lesser curve necrosis in HSV Transient dysphagia

• Vagotomy diarrhoea- 20% - Most common complication of vagotomy - Gastric stasis - Hypoacidity with fermentation with bacterial over-growth causing enteritis - Alteration in intestinal villi and enzyme content, altered small bowel motility due denervation - Altered biliary and pancreatic exocrine function - Cholestyramine 4 gram tid/ reverse jejunal loop is the treatment for intractable case • Reflux oesophagitis • Oesophageal stricture • Gallstones-due to bile stasis and gastric atony-15%

I Types 1. Truncal vagotomy along with GJ as drainage procedure, is done in case of pyloric stenosis due to chronic DU. Nerve of Lala~et (spared)

Figs. 20.95A to C: Types of vagotomy. (A) Highly selective vagotomy; (B) Selective vagotomy with pyloroplasty; (C) Truncal vagotomy with gastrojejunostomy.

In uncomplicated DU, with failure of medical treatment and when HSV cannot be done, then truncal vagotomy with pyloroplasty is done. 2. HSV (Highly selective vagotomy) or Parietal cell vagotomy or Super selective vagotomy is done in case of uncomplicated DU where medical treatment fails. Here only fibres entering the stomach is divided both anteriorly as well as posteriorly. Nerve of Latarjet is retained to supply antrum and so no drainage procedure is required (Amdrup). It has got 10% recurrence rate. 3. Selective vagotomy is at present not done often. Here along with a drainage procedure, either GJ or pyloroplasty, vagotomy is done with retaining of coeliac and hepatic branches. It has got 10% recurrence rate.

Success is the better way of dealing with failures.

en 0

3

OI

n

=r

Chapter

Small Intestine

....

.. .. ....

( ;HAPTER OUTLINE

• •

.. •

.. • •

Anatomy Meckel's Diverticulum Regional Enteritis Surgical Complications of Typhoid Surgical Complications of Roundworm Pneumatosis Cystoides lntestinalis Mesenteric Vessel lschaemia Necrotising Enterocolitis Small Bowel Tumours

.... ..

..

Benign Tumours of Small Bowel Malignant Tumours of Small Bowel Carcinoid Tumour Short Bowel Syndrome Small Bowel Enema Capsule Endoscopy Small Bowel Enteroscopy Enteric/Gastrointestinal Fistula

ANATOMY The small intestine is 6 m in length. This includes upper fixed (duodenum, 25 cm) , lower mobile part (proximal 215th is jejunum and distal 315th is ileum).

Differences between the Features of Jejunum and Ileum Jejunum

Mesentery attaches the small intestine to the posterior abdominal wall, contains blood vessels, lymphatics, fat. Mesentery at its root is attached to L2 vertebra on the left side at duodenojejunal junction, running obliquely downwards and right towards right sacroiliac joint at ileocaecal junction. Mesentery is 25 cm in length at its root and spreads like a wide fan when it reaches to small bowel wall becoming 5-6 meters in length. It crosses the duodenum, aorta, IVG, right ureter, right psoas major muscle, right gonadal vein. Mesenteric cyst, mesenteric lymph nodes, mesenteric tear, mesenteric inflammations are of surgical importance. SMA, SMV and their branches and tributaries pass through it. During small bowel resection, mesentery should be ligated using surgical ligatures or energy sources and later anastomosis is done.

I Nerve Supply Sympathetic supply is from T9-T11 and parasympathetic supply is from vagus. Both pass through coeliac and superior mesenteric plexus. Bowel wall contains the myenteric plexus of Auerbach which lies between the circular and longitudinal muscle coats; and submucous plexus of Meissner. Sympathetic nerves are motor to sphincter, inhibitory to peristalsis; parasympathetic nerves are inhibitory to sphincter and stimulates peristalsis.

Ileum

Short vasa recta

1. Long and few vasa rectae Short and numerous vasa rectae 2. Long plicae 3. Thick wall, wider-4 cm 4. Mesentery transparent 5. Peyer's patches are scanty 6. Villi, leaf like and more abundant 7. Proximal 40% of small bowel-deep red

Small plicae Thin wall-3 cm Mesentery contains fat Peyer's patches are abundant, located in the antimesenteric border Villi, finger like and less abundant. Distal 60%- pale pink

Jejunum

Vascular arcades

m

Ileum

Figs. 21 .1A and B: Vascular pattern in (A) jejunum; (B) ileum.

Coeliac artery

853

MECKEL'S DIVERTICULUM Jtappears at a specific site in the ileum and the wall contains eacfl' of the several layers of the intestinal tract.. .. The proof that the diverticulum is a residuum of the communication between the intestinal canal and the umbilical stalk rests in the findings which / I have observed in three stillborn, full-term fetuses. I __ __ __ Johann Friedrich Meckel, 180~

I

It is a congenital divertic ulum arising from the terminal ileum and is part of the unobliterated proximal portion of the vitellointestinal duct.

--------i

It is: • 2% common. 2 feet from the ileocaecal valve. 2 inch in length. 2% of Meckel's diverticulum only will be symptomatic. 50% of symptomatic are below 2 years of age. 20% heterotopic epithelium. 2:1 female preponderance is seen.

B • Saliva-1000 ml Gastric-1500 ml • lntestinal-4000 ml

• Pancreas-1500 ml

_J

Cell type

Function

Goblet cells

Mucous

Paneth cells

lysozyme, tumour necrosis factor, cryptidins

Enterocytes

Absorption

Enteroendocrine cells

Different hormones

B • • • • • • • •

Meckel's diverticulum

INVESTIGATIONS FOR SMALL BOWEL OISEASES

Barium meal foll ow through Small bowel enema (Enteroclysis) Plain X-ray abdomen to see intestinal obstruction Selective splanchnic angiography to see vascular bleed or / malformation (DSA) Isotope scintigraphy to see Meckel's diverticulum Estimation of faecal fat in 24 hours-normal is less than 7 gram j Schilling test to find out the absorption of vitamin B12 from terminal ileum SeHCAT bile acid absorption test (selenium labelled) to find out the absorption of bile acids from the terminal ileum Many different breath tests are used to find out malabsorption, bacterial overgrowth and transit time Jejunal biopsy CT scans to see fistula, tumour, and spread Capsule endoscopy is very useful

I

I

• •

It is congenital, results from incomplete closure of vitellointestinal duct. It is the most common congenital anomaly of small intestine. Arises from the antimesenteric border of the ileum, containing all three layers of the bowel with independent blood supply. In 20% of cases mucosa contains heterotopic epithelium like gastric (commonest-50%), colonic and pancreatic tissues (5%). It may be connected to or communicated with the umbilicus through a band or fistula. It may be associated with oesophageal atresia, exomphalos, and anorectal malformations.

Figs. 21 .3A and B: (A) Anatomy of Meckel's diverticulum;

(B) Meckel's diverticulum.

I Presentations in Meckel 's Diverticulum Asymptomatic-in majority cases. Severe haemorrhage most common, seen in children aged 2 years or you nger (Maroon-coloured blood). Intestinal obstruction due to bands/adhesions/ intussusception. Perforation. lntussusception, volvulus of small bowel.

One cannot love what he cannot respect, whether it be himself or another.

C")

:::c

>

.....

""Cl

m

::c N

..I,

Ci)

3

I»

854

Peptic ulceration. Diverticulitis (20%)-features mimic acute appendicitis. Littre's hernia-it is presence of Meckel's diverticulum in hernial sac as content. It is observed in inguinal/femoral hernia. Silent Meckel's diverticulum found during laparotomy or laparoscopy or by radioisotope study. Carcinoid or GIST can occur in Meckel's diverticulum.

Surgery is done whenever the base is narrow, and in lengthy diverticulum. Presence of adhesions or band which may precipitate obstruction, intussusception or volvulus. Symptomatic patients or presence of complications. If it is found in children below 2 years.

Note:

"Meckel's diverticulum frequently suspected; often sought; seldom found"-Charles Mayo.

I Diagnosis Technetium (Tc 99) radioisotope scan is very useful (90-95% accuracy). 90% of heterotrophic gastric mucosa can be identified in Meckel's diverticulum by radioisotope study. It can detect Meckel's diverticulum with minimal bleeding also (0.1 mUminute). So it is very useful investigation in children presenting with bleeding. X-ray abdomen to see complications like obstruction, perforation . Laparoscopy is very useful. Enteroclysis/small bowel enema under fluoroscopy may show the Meckel's diverticulum. It is probably the most accurate investigation.

I Treatment Asymptomatic Meckel's diverticulum can be left alone when identified during laparotomy. Resection of a short segment of ileum containing Mecke/'s diverticulum and end-to-end anastomosis is done. Meckelian diverticulectomy with closure of enterotomy also can be done, but chances of retaining heterotopic tissues and stenosis are higher.

Meckel's diverticulum

Fig. 21 .4: Laparoscopic view of Meckel's diverticulum. Patient

presented with features of acute appendicitis. On laparoscopy, Meckel's diverticulum which was adherent to umbilicus by a cord was found. Appendix was identified separately. Appendicectomy and Meckelian diverticulectomy was done.

I

Resected Meckel's diverticulum Meckelian diverticulectomy linear stapler can be used

I

I

End-to-end Resected Meckel's diverticulum anastomosis with ileum Note:

Care of clearing heterotopic gastric epithelium should be taken. Should avoid stricture formation later. Fig . 21 .5: Meckel's diverticulum-surgical treatment. Meckelian diverticulectomy is done by obliquely clamping beyond the base of the Meckel's diverticulum. Care should be taken not to retain heterotopic gastric (or other) epithelium which can be felt-like indurated area. Enough precaution should be taken so that stricture will not form later. When heterotopic tissues extend beyond the Meckel's diverticulum into the ileum, then resection of ileum with Meckel's and anastomosis is done. Clamping distal to the base of the Meckel's diverticulum like appendicectomy should not be done. It will cause stricture, leak or retaining heterotopic tissues in the part.

• Duodenal diverticulum is the most common acquired diverticulum of small bowel. + Meckel's is the most common true congenital diverticulum of small bowel. Duodenal diverticulum: It is common in females; 65% occur within 2 cm of ampulla. Commonly they are asymptomatic;

-,:,

m

...

::z::, N

en

3

Cl

864

Erythema and oedema of anterior abdominal wall-late feature. Mortality is 20%.

...=

a, Cl

Cl)

0

Figs . 21 .20A and B: Necrotising enterocolitis. Skin necrotic lesions are typical. It is common in infants and children but can be seen in adolescents occasionally (Courtesy: Dr Rupen, MS, KMC, Mangaluru).

Fig. 21.21 : Jejuna! haemangioma. Surgical resection

cured the condition.

Investigations ,, Plain X-ray abdomen: Linear gas shadow in bowel wall; Free intraperitoneal air implies perforation. ,, Blood examination- leukocytosis, anaemia, hyperkalemia, metabolic acidosis.

B Perforation-peritonitis. Colonic stricture can develop lately after healing of ischaemic area. It is seen in 10% of cases.

I Management Medical therapy ,, Nasogastric aspiration. ,, Broad spectrum IV antibiotics. , TPN for 10-14 days. ,, Oxygen supplement is required. Surgery Indications: Pneumoperitoneum; An abdominal mass; Dilated intestinal loops. Procedures done Bowel necrosis and perforation-resection with stoma. Localised disease-resection and primary anastomosis. Complication: Extensive bowel resection-short bowel syndrome.

SMALL BOWEL TUMOURS They are rare neoplasms- 3% of all GI tumours (Even though 80% total length and 90% of mucosal surface area of the GIT is small bowel). Common in elderly men. Common in New Zealand and Hawaii. It can be benign or malignant. Early diagnosis is difficult. Presentations are vague initially.

Fig. 21 .22: Jejuna! tumour.

Reasons why malignancy is uncommon in the small bowel (even though it comprises 75% of length and 90% of GI mucosa): Rapid transit time, 30 minute to 2 hours. So exposure of mucosa to toxins and metabolites is less. Alkaline mucus rich luminal content is protective. Cells of small bowel produce the enzyme benzopyrene hydroxylase which detoxifies the carcinogen- benzopyrone. High levels luminal lgA provides immunity. Plenty of lymphoid tissue in the wall provides immunity. Healthy small bowel has got less bacterial load and so their toxic metabolites (as compared to colon).

Bile acids and their metabolites Post-cholecystectomy status Familial adenomatous polyposis (FAP) especially with duodenal adenomas has got very high chance of developing adenocarcinoma • Crohn's disease has got 100 times increase in incidence of adenocarcinoma • Celiac disease increases the risk of lymphoma Peutz-Jegher's syndrome has got increased risk of small bowel adenocarcinoma Contd...

- -

Contd...

I I

-

- -

- - -,

Patients with chronic immunosuppression have very high-risk of developing NHL (PTLO- post-transplant lymphoproliferative disorder, HIV) or sarcoma Patient with von Recklinghausen's disease of neurofibromatosis is prone to develop GI neurofibroma or neurofibrosarcoma Smoking, red meat, alcohol, salt food

GIST express CD117 (90%), the c-kit proto-oncogene protein membrane receptor for stem cell growth factor and CD34 (80%), a human progenitor cell antigen.

Adenomas

Figs. 21.23A and B: Jejuna! tumour coming out of serosa.

I Presentations Asymptomatic initially. Features of obstruction/intussusception/bleeding. Vague abdominal discomfort.

I Investigations Often it is very difficult to assess small bowel. It may be on table finding during surgery, while presenting as complication like obstruction. Small bowel enteroclysis. CT abdomen is better investigation to assess bowel/ nodes/ organs. CT enteroclysis shows 90% accu racy. Video capsule endoscopy enables to visualise small bowel mucosa properly. CT angiography is useful in identifying vascular tumours. Enteroscopes (push type or Sande pull enteroscopes) are technically difficult. lntraoperative enteroscopes are useful.

BENIGN TUMOURS OF SMALL BOWEL Benign tumours of small bowel are 50% of primary small bowel tumours. Adenoma can be potentially malignant.

I Types Leiomyoma or GIST It is the most common symptomatic benign tumour of small bowel. It arises from interstitial cell of Cajal. It can be spindle cell type (70%) or epithelioid type. Commonly they are benign in small bowel.

It is 15% of all benign small bowel tumours. 50% are in ileum; 30% in jejunum; 20% in duodenum. Commonly single but can be multiplewhen familial or associated with FAP. Commonly presents as bleeding and obstruction. Adenomas can be: Brunner gland adenoma ,. It occurs as ben ign hyperplastic tumour in proximal duodenal submucosa which secretes alkaline bicarbonate rich fluid. ,. Bleeding is the usual problem. ,. It is treated by endoscopic resection. ,. It never turns into malignancy. •·· Villous adenoma ,. It can occur anywhere in small bowel presenting as intestinal obstruction or haemorrhage. ,. But commonly seen in periampullary region presenting as obstructive jaundice, upper abdominal pain. ,. Size more than 3 cm can lead into adenocarcinoma with an adenoma-carcinoma sequence. ,.. EUS is ideal tool to assess size and depth. ,. ERCP is done to relieve obstruction, to biopsy and to remove if it is small in size. ,. Malignant potentiality is very high-50%. ,. Transduodenal excision/pancreaticoduodenectomy are the surgical options. Small bowel adenomas are treated by reception. True adenomas ,. It can be tubular or tubulovillous. ,. It is usually single. ,. Endoscopic excision is the treatment; resection/pancreaticoduodenectomy is done if there is invasive lesion. Familial adenomas :.- It is associated with FAP with 5% risk for adenocarcinoma in duodenum. ,. It is diffuse throughout the duodenum. ,. Spigelman classification is used for assessment of FAP related duodenal adenomas. Villous adenomas larger

A fault once denied is twice committed.

866

than 3 cm or suspicious lesions identified on endoscopy are biopsied. , Screening endoscopy to be done every year. , Endoscopic or open polypectomy; argon beam or photodynamic therapy is tried in benign lesions. ,. Pancreaticoduodenectomy is done in high grade, carcinoma in situ, Spigelman IV classification. Spigelman classification of duodenal adenomatosis Parameter about polyps

Point 1

Point 2

Point 3

Number

1-4

5-20

More than 20

Size

1-4 mm

5-10 mm

More than 10

Histology

Tubular

Tubulovillous

Villous

Dysplasia

Mild

Moderate

Severe

Stage 0 1 2 3 4

Points 0 1-4 5-6

7-8

9-1 2

Lipoma It mainly causes intussusception. It is common in elderly men. It is usually single intramural submucosal lesion. Lipoma does not have malignant potential.

I General Features They cause haemorrhage, colicky pain, and intussusception. Diagnosis is by suspicion. Small bowel enema, capsu le endoscopy, radioisotope study, CT scan or MRI may help in identifying the disease. Laparoscopy and proceed may be useful. Commonly it is on table finding during exploration for acute presentations. Adenomas are potentially malignant depending on the size (>3 cm).

I Treatment It depends on type, number, nature, size of the tumour. Resection and anastomosis cures the condition. Surgery is not done for Peutz-Jegher's syndrome unless it presents with complications.

MALIGNANT TUMOURS OF SMALL BOWEL 1. Adenocarcinoma Its incidence is 40% of small bowel tumours. It is most common primary malignant small bowel tumour.

Peutz-Jegher's Syndrome Autosomal dominant condition with melanotic pigment patches and GI polyps. Brown black pigments of 1-2 mm diameter in circumoral face, cheek, forearms, palms, soles, digits, perianal region. Hamartomas are located in entire jejunum and ileum; 50% of colorectum; 25% of stomach. lntussusception, bleeding, anaemia-presentations. Cancer of small intestine, stomach, pancreas, ovary, lung, uterus and breast can occur. Cure is not possible. Segment which is causing complication is resected.

Fig. 21.25: Resected small bowel specimen showing small bowel adenocarcinoma.

Haemangioma It is 4% of all small bowel benign tumours. They are multiple in 60% of patients. They are usually submucosal abnormal proliferative vessels. Jejunum is the commonest site. They are often seen in association with Osler Weber Rendu

disease, Turner's syndrome. It presents as small bowel bleed. It can be diagnosed by angiography, 99mTc RBC scanning or capsule endoscopy. Treatment Endoscopic sclerotherapy or angiographic embolisation or resection of bowel segment.

Fig. 21 .26: Carcinoma ileum near ileocaecal junction. It (adenocarcinoma of ileum) carries poor prognosis. It is often on table diagnosis.

In 80% cases it is in the duodenum and jejunum. FAP, adenoma, Crohn's, coeliac diseases are the causes. Adenocarcinoma in Crohn's disease occurs in younger age group and commonly in ileum (70%). Nonspecific featu res, anorexia, cram py pain abdomen, bleeding and diarrhoea, obstruction or features of metastases (liver). CT scan , capsule endoscopy are the investigations. TNM system is used for staging. Nodal spread is the important prognostic factor. Surgical resection is the choice of therapy. Pancreaticoduodenectomy is done for duodenal tumour; for ileal/jejunal site tumours radical resection with 1Ocm margin with adjacent mesenteric clearance is done. Adenocarcinoma of terminal ileum is treated with right hemicolectomy. RT and chemotherapy are less beneficial. It carries a poor prognosis.

Fig. 21 .27: Jejuna! adenocarcinoma presenting as stricture with features of proximal small bowel obstruction. 2. Non-Hodgkin's lymphoma (NHL)- 25% GI is the com monest extranodal site of NHL (20%), with small bowel (primary intestinal NHL) as second common site (30%) for extranodal site of NHL (First common site is stomach-60%). Lymphadenopathy/mediastinal lymph node enlargement are absent. Normal spleen, liver, blood peripheral smear are observed. 8 cell type is thecommonest type 75%. It is common in ileum. T cell type (25%) carries poor prognosis. Presentations are malabsorption, obstruction, perforation, haemorrhage or palpable mass. In child ren lymphomas are the most common intestinal neoplasm. 25% of patients develop perforation. Fever when present suggests systemic spread. CT scan with CT guided biopsy or laparoscopic biopsy is needed.

Surgical resecti on and chemotherapy are the treatment. Prognosis is poor.

867

3. Carcinoid tumour-30%. 4. GIST (gastrointestinal stromal tumour) It is rare but most comm on nonepithelial small bowel tumours. 25% of GIST occurs in small bowel (stomach-SOto 60%). It is 0.2% of all GI tumours. Cl)

3

I»

Fig. 21.28: Gross look of small bowel lymphoma. It is equal in both sexes. It arises from interstitial cell of Cajal. More than 95% show c-kit (transmembrane receptor tyrosine kinase) mutation. Many mesenchymal tumours of small bowel are now classified as GIST. GIST attains massive size when presenting clinically. Palpable mass, compression, haemorrhage are the features; has less affinity for lymphatics. CT is diagnostic. Histochemistry and tumour markers are needed.

Fig. 21 .29: GIST of small bowel (Courtesy: Dr Chinivalar, MS, Surgeon).

Man is a creation of desire; not a creation of need to this world.

868

...= Q)

CII

en 'E

GIST can be low risk or high-risk group based on tumour size and mitotic index. Surgical wide resection is the treatment. lmatinib mesylate a KI T kinase inhibitor is used successfully. SU11248 with similar effect is also used.

5. Liposarcoma and myxoliposarcoma. 6. Secondaries in small bowel They are very rare- if it is present, primary being commonly melanoma.

Adenocarcinoma is commonest-40%. Order of occurrenceduodenum, jejunum and ileum GIST is new entity comprising smooth muscle tumours also ' Carcinoid and lymphoma has got better prognosis Adenocarcinoma and GIST has got poor prognosis Presentations are pain, diarrhoea, weight loss, anaemia, melaena, palpable mass, intestinal obstruction and metastatic featu res Bleeding and perforation are common in lymphoma and sarcoma Enteroclysis/cap'"le endoscopy/CT scan/histochemisl,y a6 gram/ day); increases bile lithogenicity; causes deficiency of vitamins A, D, E, K; colonic bacteria converts unabsorbed fatty acids and conjugated bile salts into hydroxy fatty acids and deconjugated bile salts. Ileum maintains enterohepatic circulation of bile salts, absorption of vitamin B12 and vitamin D. Resection of middle part of small bowel is better tolerated. Removal of ileocaecal valve reduces the intestinal transit time; reduces absorption of vitamin B12, calcium, magnesium, zinc; increases diarrhoea and contamination of shortened small bowel by bacteria. In massive resection, colonic bacteria degrade fatty acids into lactate and short chain fatty acids; lactate reduces colonic pH which inhibits bacteroides; due to this acid resistant anaerobes I will increase in colon which produces large amount of D lactate which is absorbed causing D lactic acidosis presenting with confusion, ataxia and nystagmus. TPN was introduced by Dudrick. It is very useful in short gut j syndrome.

I

,. Mucosal stem cell transplantation using enterocytes without lymphoid tissue. ,. Seeded mucosa! autografts in a prosthetic tubes.

C")

SMALL BOWEL ENEMA (ENTEROCLYSIS)

:c

It is the contrast study of the small bowel by infusing contrast agent either high density microbar solution with methyl cellulose or water soluble iodine dye through Nolan intestinal tube.

I

---

I Treatment Early phase Total parenteral nutrition. Fat and fibre free but protein rich liquid diet with essential fatty acids. Diarrhoea is controlled by loperamide/codeine phosphate. Oral cholestyramine to bind bile salts is needed in massive resection which includes ileum. Parenteral vitamin B12 injection regularly. H2 antagonists/PPls/somatostatin (to reduce secretions from stomach, liver and pancreas). Octreotide reduces the secretion and reduces GI motility. Fluid and electrolyte management. Control of diarrhoea. Late phase Enteral nutrition to stimulate intestinal adaptation. TPN supplement-often permanently required. Home parenteral nutrition is universally used method in western countries. But in Asian countries it is yet not practicable. Hormones and glutamine administration are under trial. Surgical techniques to delay the intestinal transit time or small bowel transplantation are under trial but with less success. , Reversal of 10 cm intestinal segmentto delay transit time. Intestinal lengthening to delay transit time and increase the absorption surface. ,. Small bowel transplantation, ideal but graft rejection and failure is the problem. Antiperistaltic colonic interposition into the small bowel segment. , Regeneration of intestinal mucosa over denuded serosa. ,. Longitudinal splitting of the intestine and closing of these as separate tubes which are anastomosed to each other to achieve lengthening-Binachi's surgery.

Fig. 21.31: Enteroclysis X-ray film showing entire small bowel. It is still commonly used investigation to assess small bowel pathology.

Indications: Small bowel tumours; Stricture small bowel; Congenital anomalies; Crohn's disease, intestinal tuberculosis. Findings to look for: Narrowing; Filling defects, irregular or regular; Mass lesions Problems: Poor patient acceptance; Technically difficult

CAPSULE ENDOSCOPY Radiofrequency tag with polyimide coating

26mm X

4 grams

11 mm

Lactose body with 10% barium

Fig. 21 .32: Capsule endoscopy-diagrammatic look.

It is a type of endoscopy wherein highly sensitive miniature video-camera which after activation is swallowed by the patient. It is 26 mm x 11 mm in size. It weighs 4 grams. It contains single use video-camera, with 6 light emitting diodes, a lens, a colour camera chip, two batteries, radiofre-

Our senses don't deceive us; our judgement does.

871

>

ITI

::z:, N

872

quency transmitter with an antenna. Capsuletakes 2 pictures per second which is transmitted to a worn recording device through radiofrequency. From the recording device data is downloaded to special computer. It is swallowed with 12 hours fasting. After activation it functions for 8 hours. It is mainly used to study small bowel diseases like vascular malformations, narrowing, tuberculoses, ulcers and tumou rs. This capsu le camera sends signals and endopictures at regular intervals to the receiver (digital recorder) tied over the patient's waist. This receiver is later attached to specialised computer software to get different level pictures for study. Capsule gets deactivated in 8 hours and is passed out in the stool. It is available only at few centers and is costly. Capsu le retention is the complication in 5% cases. It may not give proper evaluation in obstructive pathology and motility disorders of small bowel.

SMALL BOWEL ENTEROSCOPY It is a difficult technique done to visualise the small bowel.

I Indications Occult or obscure GI bleed. 5% of GI bleed is not diagnosed by any methods like gastroduodenoscopy, colonoscopy, contrast imaging. Angiodysplasia and ectasias are common causes for such type. Small bowel tumours; Crohn's disease; Celiac disease; Refractory sprue. HIV related small bowel diseases. lntraoperative enteroscopes to assess lumen on table.

I Technique Push Enteroscopy It is easier and faster. It reaches 60 cm beyond the ligament of Treitz. Flexible enteroscopes or paediatric colonoscopes are used. Simethicone is given prior to enteroscopy. BT, CT, LFT and prothrombin time should be checked. Aspirin should be stopped 5 days prior if patient is taking it. Oesophageal overtube is used to pass the entero-scopes through upper GI. Enteroscopes is passed through overtu be into stomach, duodenum, and jejunum. Proper evaluation of the mucosa is done during withdrawal. If there is a bleeder point, it is fulgurated.

Sande Enteroscopy It is a 5 mm diameter, 275 cm enteroscope passed through nose by a piggyback technique with a paediatric colonoscope.

Its two internal channels are for ai r inflation and for balloon inflation. Biopsy or working channel is not there. Scope is passed through pylorus and further it is moved with subsequent inflation of balloon and distal progression by peristalsis. Distal position is confirmed by fluoroscopy. It takes 6 hours to pass through the entire small bowel. Once it is in the terminal part of the small bowel, small bowel is inflated with air and mucosa is inspected. lntraoperative enteroscopy Abdomen is opened. By a small enterotomy scope is passed into the lumen. It is guided manually by compression and release to visualise entire mucosa of small bowel.

B Epistaxis due to nasal irritation. Perforation; Pancreatitis Failure to pass enteroscopes as needed. Time consuming and not acceptable by the patient.

ENTERIC/GASTROINTESTINAL FISTULA Enteric fistula is a challenging problem due to its high mortality of 30%, management of electrolyte im balance, malnutrition, and sepsis. An enterocutaneous fistula is an abnormal communication between the mucosal epithelium of the intestine and skin surface.

I Classifications Anatomical External: They are enterocutaneous fistulas which are discussed in detail here. :.- 75% of enteocutaneous fistu las are of postoperative cause due to disruption of the anastomotic site. , 25% are spontaneous due to malignancy, radiotherapy, inflammatory bowel disease (most common in spontaneous cause), diverticular disease and bowel ischaemia. , 70% of external fistula will close spontaneously. Internal , Most of the internal fistula need surgical correction, if they are symptomatic or causing complications. Aetiological Anastomotic disruption in postperative period. Trauma to normal bowel-inadvertent enterotomy/injury. Disease of the bowel extending into the adjacent structures. Disease outside the bowel extending into the normal bowel. Depending on the site Oropharyngeal Oesopharyngeal Gastric: Commonest cause is the site of gastrostomy tube, either with tube in situ or after tube removal. Other causes are- postgastrectomy and anastomotic leak after surgery for carcinoma stomach, surgeries for benign gastric diseases, reflux diseases, and obesity.

873

Duodenal: It occurs after surgery for pancreas, biliary system, duodenal stump leak, gastroduodenal anastomosis, renal, aortic, colonic surgeries. Spontaneous fistula occurs in Crohn's, ulcers, malignancy and trauma. It can be duodenal stump fistula which often closes spontaneously; lateral duodenal fistula which less commonly closes spontaneously. Small bowel: It is the commonest site of GI fistula. More than 75% are postoperative. Crohn's disease is the most common cause of spontaneous small bowel fistula in which 50% each are internal and external.

en

3 0,)

::I ctl

Fig. 21 .34: Fistula at gastrostomy site. This is becoming the common cause.

Figs. 21.33A and B: Enterocutaneous fistula in two different patients.

Colonic: It can be due to postappendicectomy, postcolonic su rgeries. It is common after emergency surgeries, and surgeries in unprepared bowel. Appendicectomy in acute phase of Crohn's may cause fistula due to adhesion of inflamed terminal ileum to abdominal wall wound, not from appendicular stump. Spontaneous fistula even though rare, occurs in diverticulitis, malignancy, ulcerative colitis, radiation and pancreatitis. Fistula may be du, to: • • • •

Figs. 21 .35A and B: (A) Complete disruption of the anastomotic site causes fistula which is unlikely to close spontaneously; (B) Partial disruption also is equally problematic.

Phys/o/og/eal-bas,d on quantity of daily output:

Complete disruption of anastomotic site • High output->500 ml/day-usually Partial disruption small bowel; 50% mortality; less chance of Lateral fistula with distal obstruction spontaneous closure. Fistula in stricture bowel • Moderate output-200-500 ml/day-colonic and small bowel mixed. • Low output-

""C

m

::J:I N N

r-

1» ....

cc

CII

::I CII

Fig. 22.1 : Arterial supply and lymphatic drainage of colon.

en

=

I Lymphatic Drainage

::I CII

Mucosa contains no lymph channels, so mucosa! cancers rarely metastasize. Nodes are epicolic (located in the colonic wall), paracolic (located along the inner margin), intermediate (located near mesenteric vessels), principal (located near main mesenteric vessels).

I Nerve Supply

Ultrashort segmentcongenital megacolon

Short segmentcongenital megacolon

Fig. 22 .2: Types of Hirschsprung's disease.

Colonic motility is under control of autonomic nervous system; parasympathetic via vagi and pelvic nerves, sympathetic via superior and inferior mesenteric ganglia. The effect of meal on colonic activity is termed as gastrocolic reflex.

HIRSCHSPRUNG'S DISEASE (CONGENITAL MEGACOLON) My first specimen is a colon, but a colon of such a size that itwitt

rno doubt surprise you to learn that it comes from a child only 11 months old when it died.... Only (the) rectum was not dilated, nor indeed subject to any obstruction. -Harald Hirschsprung, 1888

l

877

I

a

a a. Dilated segment (Normal ganglionic area) b. Cone c. Spasmodic segment Fig . 22.3: Zones in Hirschsprung's disease.

Ill Dilated proximal colon-normal ganglia

---

It is a congenital, familial condition, occurring in newborn due to the absence of ganglion cells- Auerbach's and Meissner's plexus in anorectum, which may extend proximally either a part or full length of the colon. It always involves the anus, internal sphincter and rectum (partly or entirely). There is narrow, spasmodic, non-relaxing pathological segment. Transitional zone proximal to it contains only few ganglion

I Spasm segmentno nerve ganglia

cells with formation of cone.

Still proximal to it, colon is dilated enormously with hyperaemia, multiple ulcersand hypertrophied circular musclefibres. It is one of the causes of neonatal intestinal obstruction. Severe enterocolitis can occur which may be fatal. Perforation, peritonitis and septicaemia can occur.

Fig. 22.4: Congenital megacolon showing spasmodic aganglionic segment, coning (transi tional zone). and proximal dilated normal ganglionic segment.

Onceyou learn to quit, it becomes a habit.

0

It has got three zones: I. Distal immobile spastic segment, i.e. aganglionic zone. 11. Proximal, middle transitional zone of about 1-5 cm length with less, sparse number of ganglions (cone). Ill. A still more proximal, hypertrophied dilated segment is actually the normal ganglionic area.

I Clinical Features

Cl:! :::, C

Cl:!

::E: Cl)

co

cc Cl)

Fig. 22.6: Newborn with Hirschsprung's disease with intestinal obstruction (Courtesy: Dr Vivek Prabhu, MCh, Mangaluru). Fig. 22.5: Child suffering from Hirschpurung's disease undergone

initial sigmoid colostomy.

Presentations: Acute, recurrent, chronic. It is common in males (80%). Its incidence is 1 in 5000 live births. It is common in infants and children , occasionally it occurs in adults also. Often it is associated with Down's syndrome (10%). {Commonest association). In 90% of cases, symptoms appear in early neonatal period , i.e within three days of birth. The child fails to pass meconium. After introducing finger into the rectum, child passes toothpaste like stool, with evidence of straining. Distension of the abdomen with features of intestinal obstruction is seen. In children, there is passage of goat pellet like stools, malnutrition, abdominal distention-chronic type. Constipation, with history of passing stools once in 3-4 days with straining is seen throughout the childhood and also in adolescent period. Occasionally, condition can cause intestinal obstruction. 10% familial. Gene mutation can occur in chromosome no. 1Ocommonly; occasionally in chromosome no. 13. Rectal examination shows tight sphincter with empty rectum. Child passes lot of gas and meconium.

Fig. 22.7: Barium enema X-ray showing parts of congenital megacolon-spasmodic area, cone, proximal dilated segment.

Barium enema is done to look for the extent of disease and three zones. Foley's catheter should not be used while doing barium enema in case of Hirschsprung's disease. Anorectal manometry-shows the absence of rectoanal reflex in Hirscllsprung's disease, which is diagnostic. Acetylcholine esterase staining shows hypertrophied nerve bundles.

I Diagnosis History of failure of passing meconium. Plain X-ray abdomen- shows intestinal obstruction. Useful in case of perforation. Biopsyfromall three zones to study the gang/ions and hypertrophic nerve terminals in spasmodic segment. Starting from 2 cm above the dentate line, a full thickness rectal biopsy is ideal.

Fig. 22.8: Barium enema X-ray showing features of Hirschsprung's

disease in a newborn.

879

B

L

Proximal colon

Proximal colon

Colitis (lntramucosal gas in plain X-ray). Enterocolitis may be fulminant and fatal. Intestinal obstruction. Perforation, peritonitis, septicaemia. Growth retardation; Constipation.

n

:c :I> -g -4

m

:x, N

B

N

Spur crushed

Total neuronal dysplasia cquired megacolon-rectum is loaded with stool Anorectal malformations (ARM) Hypothyroidism; Meconium plug syndrome

U

----

Fig. 22.9: Modified Duhamel operation. Normal colon is brought behind the aganglionic rectum and anastomosed just above the dentate line.

J

I Treatment Initially, colostomy is done either transverse or transitional, so to have normal bowel function. Nutritional supplementation. Once the child attains 10 kg of weight, definitive procedure is done, i.e. a. Excision of aganglionic segment (spasmodic segment). b. Maintenance of continuity by doing coloanal anastomosis. c. Closure of colostomy later. Common procedures done are: Modified Duhamel operation-rnsection of upper part of the rectum and a part of colon; anastomosis of colon to posterior part of the lower rectum and crushing the spurs to create the rectal pouch. It is technically easier and a retro-rectal pull through. New pouch is created by anterior part of the aganglionic rectum and by ganglionic proximal pulled down colon. Biopsy should be taken from proximal pulled down colon to look for evidence of ganglions. Pulled down proximal colon is sutured to full thickness posterior anal canal just above the dentate line. Spur between these two segments is crushed by Kocher's forceps or specialized instrument to create a single pouch. Soave's mucosectomy and pull through operation. Coloanal anastomosis after proctocolectomy. Total proctocolectomy with ileo-anal anastomosis in case of total colonic HD. Swenson's operatiotr-through abdom ino-anal approach, aganglionic segment is resected and colo-anal anastomosis is done. Anorectal myectomyis found to be very useful for ultrashort segment and short-segment Hirschsprung's disease. Mucosa in incised horizontally 1 cm proximal to mucocutaneous junction. A strip of muscularis with part of internal sphincter is excised with both muscle layers of the rectum for about 6-1 0 cm length. Mucosa is sutu red back. Complications are abscess formation and failure.

Complications of surgery: Severe colitis; Faecal fistula; Stenosis; Stunted growth.

No contracted segment in rectum Seen in children with faulty toilet training Rectum and sigmoid colon are dilated Normal ganglions in all levels Improper bowel habit causing chronic bowel dilatation Repeated enemas, manual evacuation, toilet training, educating the parents are required Should be differentiated from Hirschsprung's disease

J

DIVERTICULAR DISEASE OF THE COLON They are acquired herniations of colonic mucosa through circular muscles at the points where blood vessels penetrate (points of least resistance). It is more commonly localised to sigmoid colon (90%) but occasionally seen in full length of the colon. Rectum is not affected.

B Diverticulitis, hiatus hernia, gallstones It is rare in Asian and African countries because of the high fibre diet. It is common in western countries. Colonic diverticulosis is usually of false type with only mucosal herniation.

Fig. 22.10: Diverticular disease of colon.

Much wisdom often goes with fewer words.

880

0

n,

=

C:

n,

==

"'

CCI

a:

Cl)

I Aetiology Diet-it is the main factor. Low fibre diet increases the stool transit time, reduces the stool weight, reduces the bulkiness of stool which increases the intraluminal pressure and muscle hypertrophy. High fibre diet prevents this. Disease is more common in females. It is more common in aged. It is more common in non-vegetarian than in vegetarian. NSAID intake by inhibiting prostaglandin synthesis may cause diverticular disease. It is more common in individuals with steroid therapy or immunocompromised people. Smoking and alcohol. Long-standing constipation increases the stool transit time and causes diverticulosis.

cated; if there is pericolic abscess or fistula or intraperitoneal perforation with peritonitis it is called as complicated. Uncomplicated type (75%) is-actually peridiverticulitis with extraluminal pericolic infection caused by luminal perforation into pericolic area without formation of fistula ore abscess or intraperitoneal perforation. Presentations are-pain in the left iliac fossa and left groin persistent or recurrent, fever, loose stool, tender palpable thickened colon, urinary urgency, radiating pain towards back and suprapubic region. Complicated type (25%) shows- abscess or fistula into urinary bladder (commonly) or small bowel (occasionally), intraluminal perforation and peritonitis, haemorrhage (it is common due to close proximity of diverticula to perforating arterioles).

Diverticula-associated Colitis (DAG) Serosa

Acquired diverticula -Common -Sigmoid colon -Only mucosal protrusion

Muscle

layer Mucosa.....£...-

-'-::.,,,,

I Pathology

Diverticula

Mucosa Muscle~ layer Serosa

L

It is distinct entity herein diverticula is associated with colitis. It presents tenesmus, diarrhoea, haematochezia. There is segmental colitis.

Congenital diverticula -All layers with muscle layer -Caecum/ascending colon -Rare

Fig. 22.11: Diagram showing the formation of diverticula. Note the

difference between congenital and acquired diverticula.

I Types Diverticulosis These false diverticula occur at the site where arteriole perfo· rates the muscular wall; so they occur on the mesenteric side of the antimesenteric taenia; they do not occur on the anti· mesenteric border. Diverticula are common in sigmoid colon (50%), descending colon (40%), rarely in the other parts of the colon ; muscular wall thickening with hypertrophy limits to sigmoid colon only. Sigmoid gets narrowed due to spasm with mucosal false diverticula adjacent to lateral taenia with raise in intraluminal pressure 90 mmHg or more. There will be muscular incoordination, segmentation, causing episodic spasmodic left iliac fossa pain- painful diverticular disease. Here luminal perforation with peridiverticulitis is not observed.

Peridiverticulitis (Diverticulitis) Diverticulitis is a misnomer; actually it is perforation through the diverticula with peridiverticulitis; if extraluminal extraperitoneal perforation with only pericolic infection it is called as uncompli·

There is hypertrophy and thickening of the muscle layer with progressive colonic narrowing and segmentation with raised intraluminal pressure causing pulsion diverticula of only mucosa adjacent to taenia in antimesenteric region. Pathology is common in sigmoid colon.

I Features of Oiverticular Disease In western countries, 50% risk to develop diverticular disease for an individual is at the age of 60 years. Only 15% of patients with diverticulosis develop diverticulitis. 75% of patients with diverticulitis have uncomplicated course with features of only diverticulitis. 25% of patients with diverticulitis develop complications like abscess, stenosis and fistula. Abscess can be commonly pericolic and pelvic, rarely in buttock and ischiorectal fossa. Features of diverticulosis-fullness of abdomen, bloating, flatulence, vague discomfort. Features of diverticulitis-pain in left iliac fossa which is constant radiates to back and groin, tenderness, bloody stool, often massive haemorrhage, fever, rigidity and mass in left iliac fossa. Mass is usually tender, firm, resonant, non-mobile. Features of fistula-colovesical is the commonest type of fistula. It causes passage of gas in the urine (pneumaturia) commonly and occasionally faeces. COMPLICATIONS OF DIVERTICULITIS

• Perforation with pericolic abscess or peritonitis • Progressive stenosis and intestinal obstruction Profuse colonic haemorrhage (17-20%) Fistula formation (5%)- vesicocolic, vaginocolic, enterocolic, colocutaneous

L

Sigmoid colon

ticula by barium with stercolith inside-seen in sigmoid diverticula. Sigmoidoscopy is useful but should not be done in acute stage. Once acute stage subsides, barium enema, sigmoidoscopy, colonoscopy can be done (To rule out associated malignancy). CT scan in acute phase to see pericolic abscess.

881 n

::J::

>

""0

m

:tJ N N

Fig. 22.12: Sigmoid diverticula causing pericolic abscess as a known complication.

Fig. 22 .14: Colonoscopic view of sigmoid diverticula.

,

CT scan shows thickening of muscle layer, abscess, perforation, fistula, involvement of organs like urinary bladder and associated pathology. CT scan is the ideal investigation. Cystoscopy and colonoscopy in case of fistu la. Ureteric stenting is needed to make eventual surgery easier.

m

DIFFERENTIAL DIAGNOSIS

Carcinoma sigmoid colon; Tuberculosis Amoebic colitis, ulcerative colitis, ischaemic colitis and Crohn's disease Coexistence of carcinoma and diverticulitis can occur in 12% I of cases J

Vesico-colic ___..,._ ____ fistula

Urethra

Fig. 22.13: Diverticular disease causing colovesical fistula. Patient will have urine stained with faecal matter and severe urinary infection. Contrast dye study, CT scan is required. Initial colostomy as diversion is required before definitive procedure. Note: Diverticulitis is not a precancerous condition.

It may coexist with malignancy, irritable bowel syndrome, Crohn's disease. HINCHEY'S CLASSIFICATION OF DIVERTICULITIS

Stage I: Pericolie or mesenteric abscess • Stage II: Walled off pelvic abscess Stage Ill: Generalised purulent peritonitis Stage IV: Generalised faecal peritonitis

I Investigations Barium enema (best method to diagnose) shows 'saw-teeth' appearance. Champagne glass sign-partial filling of diver-

I Treatment MEDICAL TREATMENT

High fibre diet; Antibiotics; Bulk purgatives; Avoid constipation Regular follow-up of the progress of disease and onset of complications Abscess can be drained by CT guided aspiration or percutaneous drainage tube

In acute stages, conservative treatment like bowel rest, antispasmodics, antibiotics are advised. Guided aspiration of the abscess is sufficient if abscess is small. Proper antibiotics are needed.

Later, surgery is required. Resection of sigmoid colon and anastomosis (colorectal) is done. Indications for surgery: Recurrent diverticulitis; Diverticulitis with complications.

The eyes see only what the mind is prepared to comprehend.

882

Resection

i.. .4Ullr - t End colostomy =-~ -+---,,- Resection and primary anastomosis

IQ I

Irr:

Mucous fistula

IPart,,_,,

Fig. 22.17: Resection and formation of end colostomy and mucous

fistu la for acute sigmoid diverticulitis.

Part rn~t,d

Fig. 22.15: Resection and primary anastomosis can be done in

sigmoid diverticula after proper bowel preparation electively.

If colon is loaded with faecal matter, initially a transverse colostomy is done. Then resection and anastomosis; later colostomy closure is done as a staged procedure.

Occasionally, Hartmann 's procedure (combination of sigmoidectomy, end colostomy and closu re of rectal stump) is better and life saving. Fistulas are treated by resection of the diseased bowel and closure of the fistula along with diversion procedures like colostomy, cystostomy, ureterostomy. In certain cases of diverticulosis, a longitudinal incision through the taenia and muscular layer without opening the mucosa is sufficient (like Heller's/Ramstedt's myotomy)Rei//y's sigmoid myotomy.

Resection Diversion loop -+-f:~ colostomy

•

'::9=F:--F.--i- Primary

Irr: I

anastomosis

Sigmoidectomy end colostomy

Part,,_,,

Fig. 22.16: Resection of sigmoid colon for sigmoid diverticula with

primary anastomosis and defunctioning diversion colostomy.

In case of perforation, proximal colostomy and exteriorisation of the affected bowel with later resection and anastomosis is done as it has high mortality.

Fig . 22 .18: Hartmann's operation for acute diverticulitis with

sepsis/perforation/peritonitis. Here after resection proximal colon is exteriorised as end colostomy. Distal rectal stump is closed.

883

Note:

• It is basically a benigncondition, therefore theprognosis is good. High fibre diet is advised. • Complications like perforations, fistulas are dangerous and life threat•

ening. Right-sided diverticulum in the caecum and ascending colon is usually solitary and congenital.

B Heller's myotomy-achalasia cardia Ramstedt's myotomy-congenital pyloric stenosis • Reilly's myotomy-diverticulosis

_J

ULCERATIVE COLITIS It is an inflammatory condition of rectum and colon of unknown aetiology perhaps related to stress, westernized diet, autoimmune factor, familial tendency, allergic factor. Disease commonly starts in the rectum, spreads proximally to the colon and often into the ileum as back wash ileitis (5%).

I Aetiological Factors Westernized diet, red meat; less common in vegetarians. Defective mucin prod uction in the colonic mucosa and mucosal immunological reaction. Au toimmune factors-cytot oxic T lymphocytes against colonic epithelial cells and presence of anticolon antibodies. Association with HLA DR2 is observed in ulcerative colitis. DR 1501 is associated with less severe type' DR 1502 is associated with more severe form. Appendicectomy and smoking protects ulcerative colitis especially from extraintestinal features and from postoperative complications. Familial in nature. Allergy to milk (cow mi lk) and other dietary factors. Excess reactive oxidative metabolism in ulcerative colitis. Psychological aspects, stress, lifestyle, personality disorders.

I Pathology To begin with, multiple minute ulcers (pin point ulcers) occur with proctitis and colitis .!, These ulcers extend into the deeper layer J, Spasm of the bowel J, Stricture of the colon J, Permanently contracted colon (pipe stem colon) J, In between ulcers, epithelial thickening occurs which appears like polyps J, Pseudopolyposis.

Fig. 22.19: Operated specimen showing colon with features of

ulcerative colitis. It is a disease confined to mucosa and submucosa. There is no bowel wall thickening and no granuloma formation. There are no skip lesions. Rectum is always involved. Distal bowel is involved to begin with, then spreads proximally. Distal involvement is more severe. Entire colon including caecum and appendix may be involved. Only rectum involvement, as proctitis occurs in 25% cases. Such patients will have 5% risk of developing rectal cancer. In 15% cases, it is left sided ulcerative colitis presenting with severe recurrent diarrhoea. In 25% patients, total proctocolitis is the presentation. Bloody diarrhoea, malnutrition, complications like toxic megacolon, perforation (steroid may mask the features) and carcinoma are common here. Pseudopolyps are of inflammatory in nature. Absence of normal mucosa between these pseudopolyps is important to differentiate it from neoplastic polyps. Intense inflammation in the mucosa and submucosa with typical feature like: Multiple crypt abscesses ,. Sparing of the deeper layers of the colonic wall ,,. Inflammatory pseudopolyps ,. Multiple pinpoint ulcers ,. Increase in substance p containing nerve fibres ,, Lymphoid hyperplasia in mucosa and submucosa (25%) ,. Presence of anti neutrophil cytoplasmic antibodies with a perinuclear staining pattern (86%) ,. Decreased goblet cell mucin Only in toxic megacolon (1.5-2.5%) there is acute inflammation extending to entire thickness of the colon ic wall including the serosa. It is not the colon that is toxic but it is the patient who is toxic, hence the name. It is precipitated by non specific causes, during barium enema study, due to drugs like opiates, antidiarrhoeal drugs and anticholinergics. Toxic megacolon commonly affects the transverse colon which will be more than 6 cm in diameter. Left colon or entire colon also may be involved. Caecum when rarely involved; becomes more than

The best doctors in the world are-Doctor Diet, Doctor Quiet and Doctor Merryman.~onathan Swift

884

0

('a

::I C

10 cm in diameter. Colon will be like wet blotting paper. It is prone for perforation, peritonitis which carries high mortality (25-50%). C-reactive protein will be increased. It needs emergency laparotomy with total colectomy/proctocolectomy with ileostomy. Occasionally toxic megacolon can occur in pseudomembranous colitis, amoebic colitis or typhoid colitis. Carcinoma in ulcerative colitis is more prevalent than in Crohn's disease. Factors involved are:

('a

::ii:

"'

CCI

cc

, ,

Cl)

, ,. ,,. ,,

,. ,

Extent of involvement (more in total colonic). Duration of the disease; continuous active disease than intermittent disease. Incidence is 5% when duration is 15 years; 25% in 25 years; 35% in 30 years; 45% in 35 years; 65% in 40 years. Incidence of developing cancer in left sided colitis is 10 years later than universal ulcerative colitis. Incidence of carcinoma developing in ulcerative colitis is equal in both sexes. Carcinoma in ulcerative colitis is commonly aggressive and poorly differentiated, multicentric, synchronous, infiltrative and scirrhous; half the patients will have colloid carcinoma (signet ring), more advanced at the time of presentation, dysplasia developing into cancer is common. Ulcerative colitis with primary sclerosing cholangitis has still increased risk of developing cancer. In ulcerative colitis, dysplasia is very important factor to transform into carcinoma. It may be mild, moderate or marked. It is often called as dysplasia associated lesion or mass (DALM).

Fig. 22.20: Multiple pseudopolyposis involving entire colon in ulcerative colitis. Disease involves only mucosa and submucosa.

I Clinical Features Disease usually beg ins in rectum as proctitis later becomes left sided colitis and eventually causes severe total proctocolitis. More common in females (2:1 ), begins in 3rd decade. Watery diarrhoea, mucus or blood stained discharge per rectum.

Colicky pain, spasms. Decreased appetite and loss of weight. Relapses and remissions at regular intervals.

T

Clinical grading of ulcerative colitis Mild

Stool frequency Pulse

Moderate

Severe

6

100 10%

Temperature

Normal

99-100

>100

ESR

30

Albumin

Normal

3- 3.5

6 cm . It has high mortality and requires emergency surgery, i.e. either colostomy or resection with ileostomy and later ilea-anal anastomosis. Here colon is like wet blotting paper. b. Chronic type (95%): , Lasts for months to years with diarrhoea, blood loss, anaemia, invalidism, abdominal discomfort and pain. ,. Severe mal nutrition and hypoproteinaemia. Note: • Prevalence is 40-100/1,00,000. • It is common before 30 years. • Smoking increases Crohn's but protects ulcerative colitis. • Incidence of colonic stricture is 10% in ulcerative colitis and is due to muscular hypertrophy. Strictures are usually benign. But 60% stricture appear after 20 years; 80% of stricture occur proximal to splenic flexure; stricture causing obstruction are likely to be malignant. • Perinuclear staining antinuclear cytoplasmic antibodies (pANCA) are seen in 85% of patients with ulcerative colitis and is diagnostic test to differentiate from Crohn's. • Arthritis (20%), ankylosing spondylitis (5%), erythema nodosum (15%), pyoderma gangrenosum are extraintestinal features which resolve after total colectomy. • Primary sclerosing cholangitis (PSC, 5%) is common in ulcerative colitis before 40 years of age; common in males; related to HLA B8 and HLA DR3 (10 times). Malignant transformation in colon is 5 times more in these patients compared with ulcerative colitis without PSC. It causes pain abdomen, obstructive jaundice, later liver cirrhosis and failure. Total colectomy will not resolve PSC.

•

885

Surveillance colonoscopy to identify transformation to carcinoma in

ulcerative colitis is done as follows - Every year from 8 years after the onset of pancolitis and 15 years after theonset of left sided colitis. 10-30 random biopsies should be done. No dysplasia (less chance of carcinoma)/low grade dysplasia (10% chance)/high grade dysplasia (40% chance)/DALM (50% chance). All dysplasia types need proctocolectomy. Flow cytometry of biopsy specimens to study DNA aneuploidy or polyploidy is needed for further confirmation. 30% of high grade dysplasias show invasive carcinomas. • Sacroiliitis and ankylosing spondylitis are 20 times more common in patients with ulcerative colitis and is associated with HLA B27.

T

Figs. 22.21A and B: Colonoscopic view of ulcerative colitis and multiple ulcers.

r-

..,

Q)

Differences between Crohn's disease and ulcerative colitis

Crohn's dis,ase

Ulcerative colitis

• It affects ileum and often colon but can involve any part of GIT- rectal sparing is common

• It affects rectum and colon from distal to proximal

• Full thickness-transmural disease

• It affects mucosa and submucosa-not deeper

• Skip lesions are typical

• Skip lesions are not observed

• Granulomatous lesion on histology-deep ulcers; pseudopolyps are not found

• Not a granulomatous lesion-superficial ulcers, pseudopolyps are present

• Stricture and fistula are common

• Narrowing can occur but not very common

• Anal fissure and perianal abscess are more common

• Fissure and perianal disease can occur but not as common as in Crohn's disease

• May mimic appendicitis

• Will not mimic appendicitis

• Bleeding is not common

• Bleeding is common

• Fever is common

• Fever is uncommon

• Mass in RIF is common

• Mass is not a common feature

• Anal pathology very common

• Anal pathology is rare

• Discontinuous segmental asymmetrical colitis

• Continuous mucosal disease-colitis

• Toxic megacolon is rare

• Toxic megacolon is common

• Normal vascular pattern

• Distorted vascular pattern

• Fistula is very common

• Fistula is uncommon

• Recurrence is common after resection

• Total proctocolectomy cures the disease

I Investigations Barium enema-shows loss of haustrations, narrow con tracte d colon (hose pipe colon}, mucosal changes, pseudo-polyps. It is avoided in tulminant cases. Sigmoidoscopy and biopsy. Colonoscopy is also required. Due to very high incidence of malignant transformation in ulcerative colitis (10-20%}, multiple biopsies should be taken from suspected areas of the colon. Risk increases with age of the patient and duration of the disease (20%).

B 0-Normal mucosa 1-Loss of vascular pattern 2-Granular, non-friable mucosa

IC CD

3-Friability on rubbing 4-Spontaneous bleeding, ulcerations

••• Plain X-ray abdomen is useful in obstruction, toxic megacolon, perforation. C-reactive protein will be very high in acute phase.

B Crohn's disease lschaemic colitis Irritable bowel syndrome Amoebic colitis

Bacillary dysentery Carcinoma colon Collageous colitis in females Infectious colitis by Clostridium

difficile, Campy/obacter jejuni

B GIT Pseudopolyposis Turning into malignancy Stricture formation, commonly in recto sigmoid and anal canal-10%; Fistula in ano-20% Toxic megacolon in transverse colon Massive haemorrhage-1 %; Perforation-10- 20% Extraintestinal Severe malnutrition; Liver cirrhosis (50%) Skin lesions- pyoderma, erythema nodosum Arthritis, iritis, ankylosing spondylitis- common Sclerosing cholangitis, carcinoma of bile duct

lJ

------

I Treatment General Correction of anaemia Fluid and electrolyte supplimentation Nutrition (high protein, carbohydrate, vitamin, but low fat diet), TPN Sedatives and tranquillisers Psychological counselling

Drugs In active disease, drugs are used to induce remission. Later drugs also should be given for maintenance of remission and to prevent relapses.

Hard work doesn't guarentee success, but improves its chances.

-

::I CD ::I CD

886

0

RI :::I C RI

:ii:: en

ca cc en

Salazopyrine/sulfasalazine splits into 5 amino-salicylic acid and sulphapyridine in colon. It is used as first line therapy. It is more than 6 stools per day, with cramping pain, toxicity, Its dose is 2-4 gm/day. It is mainly used to induce remission. fever, raised ESR, anaemia, tachycardia, more than 10% weight It suppresses PG E1 and PG E2 to reduce the inflammation, loss, hypoalbuminaemia less than 3.5 gm% inhibits proteolytic enzymes and also causes immunosupIt always needs hospitalisation IV corticosteroids [hydrocortisone) 300 mg/day/oral prednisopression. 5 ASA is the most active ingredient. Side effects are lone/5 ASA orally or as enema skin rashes, bone marrow suppression, folic acid deficiency, IV cyclosporine with azathioprine or 6 mercaptopurine haemolysis in glucose 6 phosphate dehydrogenase deficiency • Fluid and electrolyte management patients, temporary fertility problems in men. TPN/enteral nutrition Proper monitoring of the patient for complications 5 ASA (Mesa/amine) is also used in active disease as first line Surgery therapy. It is used with an azo bond to prevent its absorp- - ~- tion in small bowel. Its induction of remission is same as sulfasalazine. Idiosyncrasy and temporary infertility is not present in mesalamine. Its oral dose is 2-4 gm/day. It is also 1.5% incidence used as retention enema which is better than steroid enema Common in transverse colon Colon is more than 6 cm in diameter in left sided ulcerative colitis/proctitis. But mesalamine enema Colon is like wet blotting paper (4 gm in 100 ml saline) is costly. Enema is combined with Commonly seen in ulcerative colitis oral mesalamine or oral steroid in acute cases. Mesalamine Can occur in bacterial colitis, pseudomembranous colitis, fulminant amoebic colitis 500 mg suppository is also used in BID doses. Daily oral (1 .6 May be precipitated by antidiarrhoeal drugs gm) and topical 4 gm enema, twice weekly is often used for IV fluids, blood transfusion, antibiotics, steroids are also needed maintenance therapy. Headache, dyspepsia and myocarditis • Can be life threatening are the complications. Plain X-ray is very useful Emergency surgery, colectomy, colostomy/ileostomy may be Steroids are used in cases where salazopyrine fail to induce required remission. It is a drug for refractory cases. Oral prednisoIn olden days blowhole procedure was advocated for toxic megalone 60 mg/day tapering in 4 weeks is the dose. Intravenous colon, may be still useful in severe cases. Skin level transverse hydrocortisone 100 mg 8th hourly is used in acute cases. colostomy, sigmoid colostomy and a loop ileostomy is done to decompress entire small and large bowel. After many months 100 mg hydrocortisone enema/40 mg methylprednisolone (6- 8 months) once patient recovers from acute illness, total enema (steroid retention enema) are used for 2 weeks in proctocolectomy with ilea! pouch anal anastomosis is done acute active diseases. Most patients with moderate to severe disease need steroid therapy. Budesonide is a newer steroid Indications for Surgery-30% Cases hydrocortisone analogue is also equally effective with lesser ~ tractability-commonest indication side effects and less adrenal suppression. Toxic dilatation; Perforation lmmunomodulators are used often to induce remission and Haemorrhage for maintenance. Azathioprine and 6-mercaptopurine are Risk of malignant transformation, dysplasia (DALM) used. They act at DNA level (purine ribonucleotide) and inhibit • Onset at early age; Chronic invalidism Progressive disease with stricture, abscess, fistulae lymphocyte function (of T cells). But this action is slow. Steroid dependency, persistent active disease Cyctosporin is used in refractory, fulminant severe ulcerative Malignancy; Severe extraintestinal manifestations colitis as a reserve drug. Dose is 4 mg/kg/day IV. Growth retardation in children Mebeverine HG/ and Tegaserod (6 mg) are the other drugs used in ulcerative colitis. Other measures are using sucral- Surgeries fate, short chain fatty acids, probiotics, antidiarrhoeal drugs Total proctoco/ectomy with ilea-anal anastomosis with (diphenoxylate, loperamide, codeine) , avoiding milk products, pouches as reservoir("J', 'S', or "W' pouches). It is called as fibre, fruits. restorative proctoco/ectomy with ilea/ pouch anal anastomosis Antitumour necrosis factor alpha-lnfliximab is also used (!PAA). It is ideal curative procedure for ulcerative colitis. Anal selectively (in some studies). It is given intravenously at sphincter complex is preserved. 30 cm ileal pouch is created interval of 6 weeks. It shows 70% remission in ulcerative either hand sewn or with stapler. Created ileal pouch reservoir colitis. is anastomosed just above the anal canal using end to end Newer drugs with a targeted delivery into the colon is stapler. Diameter of the pouch should be twice the diameter of the ileum. Obstruction (25%), diarrhoea, pouchitis (25%), used nowadays, e.g. Olsalazine (azobond); Balsalazine (4 leak, sepsis, incontinence are the complications. aminobenzoyl carrier).

B

L

I

I

Total proctocolectomy with ileostomy (permanent, continent Kock's ileostomy, with one way valve is done). It is used at present only in revision surgery when needed after restorative proctocolectomy. Total proctocolectomy with end non continent ileostomy was the earliest operation done for ulcerative colitis. It is now reserved only for ulcerative colitis complicated with carcinoma of rectum. lleostomy site should be marked in standing and sitting position. Stoma should be within the right rectus abdominis muscle at the summit of infraumbilical fat, away from midline incision, bony prominences and umbilicus.

l

I Complications of Surgery for Ulcerative Colitis Pouchitis(20%) with pain, diarrhoea, fever, bleeding, toxicity, pouch-vaginal fistula, fecal incontinence {5%). Pouchitis disease activity scoring index is at present used which is based on clinical, endoscopic and histological inflammatory features. Stenosis, pelvic abscess formation. Leak, fistula formation. Problems with i/eostomy-psychological trauma, skin excoriation, retraction, stenosis {25%), prolapse, bleed ing, enteritis, ileal necrosis, ileal volvulus, paraileostomy hernia, paraileostomy abscess. Sexual dysfunction following proctocolectomy by nerve injury. Persistent perinea/ sinus after total proctocolectomy. Risk of cancer persists if only total colectomy is done.

ISCHAEMIC COLITIS

J

-

W

H

Fig. 22.22: Different pouches are used after restorative proctocolectomy

and during ileoanal anastomosis. Pouch acts as reservoir and reduces the frequency of stool. It avoids ileostomy with natural stool passage with continence. Pouchitis can occur with diarrhoea, blood in stool, perianal pain. Occasionally, redoing or conversion may be required. J type is commonly used.

Total colectomy with ileorectal anastomosis. Proper foll owup at regu lar intervals by regular sigmoidoscopy evaluation should be done as rectum is also diseased and vulnerable for complications. It is not commonly practiced now. Total colectomy with rectal mucosectomy and anastomosis above the dentate line on posterior aspect is also occasionally used.

B It is usually end ileostomy. But often loop ileostomy is also done Indications:

After total proctocolectomy for ulcerative colitis, total colonic Hirschsprung's diseases, Crohn's disease (occasionally), and total colectomy for carcinomas Loop ileostomy is done in critically ill patient with acute ileal conditions like multiple ileal perforations, ileal gangrene or distal fistulas or sepsis. It is temporary ileostomy • lleostomy is placed in right iliac fossa through the right rectus muscle. • lleostomy may be temporary (loop) or permanent (end) Brooke's classic end non continent ileostomy or Kock's continent ileostomy {with continent intra-abdominal pouch) are used Ileum should project as a spout at least 4 cm above the skin surface, which facilitates the effluent to pass directly into the bag lleostomy usually acts in 48 hours lleostomy bag, ileostomy care, nutrition and electrolyte management are important Complications like prolapse, stenosis, haemorrhage, retraction can occur

It occurs in splenic flexure where blood supply is precarious. Splenic flexure is the water shed area of colon, receiving blood supply from terminal branches of superior and inferior mesenteric arteries {Griffith's critical point). lschaemic colitis is common in females; common in aged. It is related to atherosclerosis, emboli, vasculitis, diabetes, chronic renal failure, autoimmune diseases, polycythaemia, haemodialysis, etc. Water shed point- Griffith's point in artery of Riolan gets poor perfusion by arterial disease/low perfusion pressure/ altered viscosity causing ischaemic colitis.

B 1. Gangrenous type-ischaemia of full thickness colon causing peritonitis. 2. Stricture type- ischaemia of muscu/aris layer causing scarring. 3. Transient type-most vulnerable layer, mucosa/ involvement usually recovers completely. ------Features , Pain in left iliac fossa and left hypochondrium . .,. Vomiting, diarrhoea; Passing blood in the stool. Differential diagnosis: Carcinoma colon; Ulcerative colitis; Crohn's disease; Tuberculosis.

Fig. 22.23: lschaemic colitis in splenic flexure.

God gives every bird its food, but he does not throw it into the nest.

887

888

Investigation ,. Plain X-ray reveals 'thumb printing sign' due to mucosal oedema and submucosal haemorrhage. Perforation is also diagnosed. ,. CT scan shows colonic wall thickening with posterior fat shadowing. Angiography is not helpful. ,. In acute stage, barium enema or contrast study or sigmoidoscopy or colonoscopy is avoided due to risk of perforation. ,. In chronic stage, colonoscopy and contrast study is a must. Treatment ,.. 80% of patients will recover from conservative treatment- bowel rest, fluids, antibiotics, adequate perfusion. ,. Surgery is indicated in gangrene and peritonitis, stricture (15%), segmental ischaemia (20%). ,. Laparotomy, resection and anastomosis (splenic flexure) is done. ,, In acute phase with peritonitis (covering), diversion colostomy is needed.

B

SURGICAL COMPLICATIONS OF INTESTINAL AMOEBIASIS Trophozoites of Entamoeba histolytica by digesting mucosa, submucosa of rectosigmoid (75%) region commonly or ileacaecal region of the colon, causes retort shaped amoebic ulcers which exudes blood, pus and necrotic material. Cyst is the infective agent. It enters through faeco oral route. It forms trophozoite which multiplies and causes inflammation and flask shaped ulcers in the rectosigmoid, caecal and often in the terminal ileal region. It causes blood with mucus diarrhoea, toxaemia, secondary bacterial infection, fulminant colitis, perforation and peritonitis, rarely toxic megacolon, extraintestinal amoebiasis. Amoebic liver abscess is the commonest form of extra- intestinal amoebiasis. Others are cutaneous amoebiasis, amoebic empyema, and very rarely amoebic brain abscess, amoebic pericard itis. Chronic amoebiasis causes vague abdominal pain, decreased appetite, intestinal colic and psychological trauma to the patient.

Colon is the commonest site of intestinal ischaemia Most of colonic ischaemia results from small vessel occlusion or low flow ischaemia. Vascular diseases, vasculitis, diabetes, hypotension, aortic surgery with ligation of inferior mesenteric artery, aortic atherosclerosis blocking opening of IMA, OCP intake, cocaine abuse, coagulopathies, CMV, E. coli infections, long distance runningare the causes Splenic flexure is the most common site; but any segment like sigmoid colon can be involved Rectum is spared due to rich collaterals

PSEUDOMEMBRANOUS COLITIS It is an acute diarrhoea due to toxins produced by the overgrowth of Clostridium difficile after antibiotic therapy (usually after clindamycin). Clostridium difficile is a Gram positive, anaerobic, spore forming bacillus. It produces toxin A (enterotoxin) and toxin B (cytotoxin, more potent). It is also often seen in immunocompromised patients and patients who are on cancer chemotherapy. Incidence is 2% . Mortality is 30%. Stool cytotoxin assay is highly sensitive and specific. ELISA test for toxins is also useful. Colonoscopy is ideal as right side involvement is more common. It can occur up to 6 weeks after stopping the drug. Diarrhoea, toxaemia, perforation, haemorrhage also can occur. Treatment: IV vancomycin 500 mg 8th hourly; IV metronidazole 8th hourly.

.-"f'~>2 cm-30-50% chances of developing carcinoma; 1-2 cm - 10% chances of carcinoma;

1. Temporary: Is done in conditions wherein diversion is required to facilitate healing distally in the rectum or distal colon. And this is closed once the purpose is over. Site of temporary colostomy is usually right hypochondrium and left iliac fossa. It can be loop colostomy or Devine's double-barrel colostomy (wherein there is a gap between the two openings of colostomy which prevents spillage into the distal loop). 2. Permanent colostomy is always end colostomy placed in left iliac fossa, 6 cm above and medial to the anterior superior iliac spine.

T

Indications

Temporary

Permanent

Congenital megacolon Anorectal malformations Sigmoid volvulus Perforation of left sided colon Left sided colonic growth High anal fistula Trauma to left sided growth

AP-Resection. Carcinoma anal canal. After Hartmann's operation

Colostomy can be: Loop, end, double barrel. It can be: Diversion colostomy It is done when there is breach in bowel wall, trauma, destruction, sphincter injuries, Crohn's, carcinoma rectosigmoid. It is usually brought out as an end colostomy with a mucus fistula or with a Hartmann's resection. It can be at sigmoid, descending and transverse colon . Decompression colostomy It is done for obstructive lesions in rectosigmoid , toxic megacolon. Types-(1) Blow hole procedure is done as a single or multiple small stomas onto the skin to decompress colon in acutely ill patients with massively dilated or impending perforation of colon. Problems are-remaining possible ischaemic parts of the colon is not identified still risking perforation; peristomal skin complications and mucosal prolapse is common. (2) Tube caecostomy-lt is technically easier without much complications; but tube getting blocked limits its use regularly. (3) Loop transverse colostomy-It serves as decompressing and diverting long stoma without getting blocked. It is done only in mobile colon. Irrigation colostomy It avoids need for appliance wear; reduces the passage of uncontrolled gas with less leak of stools. But it is time consuming with chance of water intoxication due to excess water absorption during irrigation.

Art is long, lite short, judgement difficult, opportunity transient.

c-,

::c m

:::0 N N

,...

...

Q)

IQ (D

:::s

cii' :::s (D

I Colostomy Care

902

, ,

i::' Q)

...

, , ,

Cl

::I (I)

0

Similar to stoma care. Initially stoma bag should be transparent as content is liquid stool but later it can be opaque. Regular consultation with stoma therapist. Care of the skin. Training for managing colostomy, its care to prevent leak, odour, and discomfort.

I Closure of Colostomy m

[!I Figs. 22.47A to 0 : Types of colostomy. (A) Temporary colostomy;

(B) Loop colostomy; (C) Devine's doublebarrel colostomy (D) Permanent colostomy.

Loop stoma

Double barrel! stoma

Terminal stoma

Fig. 22.48: Different types of stomas used in colostomy.

Criteria for temporary colostomy closure-. Integrity of distal colon should be normal and adequate. Anorectal sphincter should be normal. Cause for construction of colostomy is cured completely without any suspicious of recurrence of same disease distally. When temporary colostomy is done, it is closed usually after 3 months. Two types of closure are presenF--extraperitoneal and intraperitoneal type. Now intraperitoneal closure is done. Earlier extraperitoneal closure was done in loop colostomy by placing a spur in between and closing the antimesentric part of the colon. It prevents the peritoneal contamination. But inadequate closure, leak, adhesions are the problems. lntraperitoneal closure-commonly advocated technique now, is done by placing a circumferential incision over the margin with skin edge. Incision is deepened to enter the peritoneum and pull out the colostomy stoma. Part adjacent to the skin is resected and anastomosed using silk/vicryl. Sutured bowel is placed into the peritoneal cavity. Drain is placed into the peritoneal cavity. Abdomen is closed in layers.

Fig . 22.49: Diagram of colostomy in a patient with colostomy bag.

B Prolapse of mucosa (prolapse of distal loop mucosa is common)-commonest complication Retraction; Necrosis; Stenosis; Herniation Bleeding; Diarrhoea; Enteritis; Skin excoriation Educating the patient regarding the proper usage of colostomy bags and proper care of the colostomy is very essential.

Fig. 22.50: Patient with colostomy bag. He has undergone

abdominoperineal resection (APR) for carcinoma rectum. Closure of colostomy is done after proper bowel preparation, under general or spinal anaesthesia. Proper postoperative care is important.

Patient should perform anal sphincter exercises to prevent sphincter atrophy and to maintain sphincter tone.

structures inside, like bowel or ureter. It is done for diversion of urine or faecal matter in case of malignancy, trauma, and sepsis or after surgery.

I Types lleostomy-terminal 5 cm ileum is projected out, on to the skin of abdominal wall to drain semi-liquid, faecal matter. Colostomy-colon at different levels, can be brought out to the skin as required as colostomy, to divert faecal matter. Cutaneous ureterostomy-cut ends of one or both ureters are apposed to the skin of abdominal wall. Ilea/ urinary conduit-segment of isolated ileum can be used to drain urine from the ureter as urinary ileal conduit. Ureters are anastomosed to a closed ileal conduit. lleal stoma is brought out as stoma. Different types of continent ileostomies are in use to prevent leak, soakage and discomfort. Vesicostomy-it is done in children. Here anterior wall of bladder is brought out and bladder mucosa is sutured to the skin of abdominal wall. Stoma created may be round (commonly) or square in shape.

Preparation and Counselling of the Patient for Stoma Figs . 22.51A and B: Prolapse of colostomy is acommon complication.

Caecostomy tube

Fig. 22.52: Caecostomy is often done in acute colonic conditions as

diversion procedure after surgery distally like perforation closure and resection. But it is not as popular as colostomy because it may not function adequately, may get displaced or blocked. But it is technically easier and usually done by doing appendicectomyand passing Malecot's tube through appendicular stump. Removal is easier without any surgical intervention.

STOMA CARE

I Definition of Stoma Stoma is an artificial opening or 'mouth like' to the exterior, the abdominal wall so as to drain the content from the tubu lar

To certain extent stoma of any type causes psychological and physical trauma to the patient, as it is nonphysiological, distressing and socially not acceptable. Patient should be explained about the procedure and should be convinced and consoled about the stoma. Detailed meaning, explanation and after care of the stoma should be discussed. Indication for the stoma and consent for the same should be taken. Reassurance about the stoma, its care, and its position should be diagrammatically explained to the patient and his close relative. In case of obstructive disease, stoma is done as an inevitable procedure to relieve the obstruction, often it may be temporary. Proper bowel preparation by bowel wash, gut irrigation is required before surgery. The surgeon selects the site of the stoma. ,. Stoma is usually sited midway between anterior superior iliac spine and umbilicus. ,. It should be away from the belt line. ,. It should be away from the scar, creases, and bony points. ,. Patient should be assessed for proper size, adequacy for stoma in lying down, sitting, and standing positions. ,.. Proper stoma appliances should be decided after thorough check-up and discussion with patient and patient's relative. ,. Stoma site should be marked properly before surgery. ,.. lleostomy is usually sited in the right iliac Iossa, colostomy in left iliac Iossa. ,.. Allergy for the particular appliances should be checked for. ;.. The patient should consult stoma therapist.

The most vital test of a man's character is not how he behaves after success, but how he sustains defeat.

903

C")

:::c

>

rn

::l:I N N

904

c=a,

...

Cl

:::I Cl)

0

en

cc

a: Cl)

I Postoperative Care for the Stoma Stitches are removed in 6-1 Odays. Dressing should be done first over the stoma and after placement of appliance, laparotomy wound is dressed otherwise stoma appliance will not sit properly. Patient should be observed for any complications. Once wound has healed patient can take bath by removing the appliances. After bath skin is dried up and stoma appliances can be fit again. Patient should be taught about the stoma care and its appliances. Care and prevention of skin excoriation due to leak is also looked into. Psychotherapy is given for the patient. Skin should be absolutely dry prior to placing the stoma appliances.

Stenosis and block. Infection either bacterial or candidial. Diarrhoea due to irritation. Leak due to improper fitting of the appliances, scar, irregularity of stoma, prolapse. Bleeding from the stoma edge. Herniation of the abdominal contents adjacent to stoma. Skin Excoriation

It is a major problem in stoma patients. It is basically due to leak adjacent to appliances.

Causes for Excoriation Leak due to improper appliances. Wet skin before placing the appliance. Inadequate stoma hole. Improper and inadequate adhesive sheet usage. Allergy. Infection like of bacteria and candida. Altered weight of the patient. Stoma bag is overfilled or kinked or air in the stoma bag.

Treatment of Excoriation Control of infection by antibiotics and control of moniliasis. Allergy has to be confirmed, and if it is the cause, the agent is found out and treated as required. Zinc oxide cream application. Change of the type of appliance. Refashioning of the stoma.

STOMA APPLIANCES Fig. 22.53 : Right sided temporary loop colostomy. Note the tube

kept in between.

I Complications of Stoma Skin excoriation. Mucosal prolapse-common complication.

Stoma appliances are devices, which are used to collect and dispose the effluent materials which come out of the stoma.

B Leak proof; Easier to use Should not damage the stoma and surrounding skin Should prevent odour; Should be available

I Types of Appliances It can be-.

Closed type is discarded when full and is used in patients with well-formed stool. Drainable type is used in patients with loose liquid stool. It can be emptied and retained and reused. Immediately after colostomy, drainable appliance is used. Later, it can be changed over to closed type. It can also be: Fig. 22.54: Colostomy prolapse is the common complication .

One-piece stoma appliance as a bag with adhesive system attached which adheres to skin around the stoma.

Two-piece stoma appliance has got a flange with adhesive system and abag over it, which can be removed and replaced with a new one without disturbing the flange underneath. Bag can be: Transparent, in which fluid can be visualised. It is used in initial period of the stoma. Opaque, in which fluid cannot be visualised. It is used eventually later.

905

B High output >500 ml/24 hr Distal bowel obstruction Sepsis, inflammatory bowel disease Tuberculosis

Cancer Radiation enteritis Foreign body in fistula tract Epithelialisation of tract

FAECAL FISTULA

, • , • • • •

F: R: I: E: N: D: S:

Foreign body in fistula tract Radiation enteritis Inflammatory bowel disease Epithelialisation of tract Neoplasm Distal obstruction Sepsis

)>

m N N

,-

...

Al

cc

CD

::I

cii ::I CD

fig. 22.55: Multiple faecal fistulous openings in the abdominal wall. Patient has undergone laparotomy for acute peritonitis.

B Fistulogram using gastrograffin CT scan and CT fistulogram Barium enema study

This is a dangerous entity. It commonly occurs after appendicectomy (gangrenous), ileal resection, colonic surgery, malignancy, ileocaecal TB, actinomycosis and Crohn's disease. It may be from the ileum or the colon. It may be single or multiple openings. More proximal the fistula, more is the severity in fluid and electrolyte imbalance. It may be from the main incision wound or from the drain wound. If there is no distal obstruction, the fistula will heal spontaneously, but may take a longer time. Fistulogram often delineates the track. CT fistulogram with CT scan abdomen is essential. Often gastrograffin study is also useful to study the fistulous track in detail. FACTORS PREVENTING CLOSURE (FRIENDS)

::c ::::r:,

I General Care and Advice to Patients with Stoma Patient can have normal diet. Diet, which regulates the bowel action, is better. Plenty of water is advisable. Patient can go for normal work, exercise like sports, swimming, tennis. Stoma appliances suitable for these works are available. Antidepressants, anticholinergics might cause constipation. So these drugs should be taken carefully. Using irritant solutions near stoma should be avoided. It may lead to dangerous complications. Patient can have normal sexual activity. Patient should have additional stoma bags in hand so as to use if required urgently. Patient should be aware of different appliances available and should be well-versed with its use. He can take the help of the stoma societies.

(")

Fig. 22.56: Gastrointestinal faecal fistula after an emergency surgery for ilea I perforation with severe peritonitis. Patient recovered after longterm stay in the hospital.

I Treatment TPN and blood transfusion may be required to improve the nutritional status of the patient. Sepsis is controlled with proper antibiotics. Better alone than in bad company.

906 c:-

...::s

(1)

en

-

Cl)

0

ta

::s

C:

ta

:!!:: _vi

CCI

a: Cl)

Management of fluid and electrolyte loss. Skin is protected from excoriation by using zinc oxide cream. If it persists, later exploration and resection is done. The cause is treated. Bypass may be required. (Most of the time, the decision is, taken on table during surgery) .

PREPARATION OF LARGE BOWEL FOR SURGERY

I Principle Behind Bowel Preparation Colon contains large amount of bacteria up to 10 ml of faeces. Most common anaerobe is bacteroides; commonest aerobe is Escherichia coli; Pseudomonas, Enterococcus, Proteus, Klebsiella, Streptococcus are other organisms. Bowel preparation is done to clear this bacterial load to reduce postoperative complications. 91

I Methods Used 1. Mechanical bowel preparation , Polyethylene glycol (PEG) is a non-absorbed sodium sulphate solution, 2-3 litres of which is asked to drink by the patient along with plenty of additional fluids orally. It cleans the bowel by passing loose stool for 10- 15 times in 12 hours. It acts by its hygroscopic action. Side effects are-nausea, vomiting, and abdominal cramps. Antiemetics are often needed. It is ideal in renal failure, ascites, cirrhosis, CCF. , Sodium phosphate is an alternative to PEG as smaller volume is sufficient to take. But it causes electrolyte imbalance. Its efficacy is similar to PEG. But patient compliance is better with sodium phosphate. It can cause hyponatraemia, hypocalaemia, hypocalcaemia and hypophosphataemia in case of renal dysfunction. In such patients it is contraindicated. , Other methods-(1) Total gut irrigation daily using 200 ml of oral mannitolor through nasogastric tube for 3 days prior to surgery. (2) Bowel wash daily for 3 days prior to surgery using 2 litres of normal saline (not water as it will cause water intoxication); it cleans entire large bowel. (3) Senna, castor oil, bisacodyl. (4) Repeated enemas. PEG or sodium phosphate has taken over all this methods.

Contraindications for mechanical bowel preparation: Complete bowel obstruction and perforation. 2. Antibiotics-parenteral and as bowel antiseptics , Oral neomycin (gentamycin, streptomycin were used in olden days) 1 gram, erythromycin 1 gram, is used 3 days prior to surgery. Alternatively ciprofloxacillin and metronidazole are used. IV fluids should be given in addition to these patients to maintain adequate hydration. , IV antibiotics 4 hours before making incision, reduces the incidence of sepsis. Usually cephalosporins are given.

I Controversies There is doubt about the advantages of preoperative mechanical bowel preparation even though it is practiced universally. Oral antibiotics to reduce the bacterial load in the colon is also controversial. However, preoperative single dose parenteral antibiotic improves the result and reduces the sepsis.

I Indications for Large Bowel Preparation Carcinoma colon (especially left sided). Anorectal malformations; Megacolon; Carcinoma rectum. Surgery for ulcerative colitis. FAP; Diverticulitis; High pelvirectal fistulas; Before colonoscopy.

SURGICAL POUCHES Pouches are created as reservoir whenever needed as a replacement for the existing reservoir like stomach, rectum, and urinary bladder.

I Types Jejuna/ pouches: It is used for stomach. Hunt Lawrence pouch, omega loop, Roux en loop are the examples. It is commonly used after total gastrectomy. Hunt Lawrence pouch is 15 cm in length. Ilea/ pouch: It is used as rectal reservoir, urinary reservoir, ileostomy reservoir. Examples are-J, S, W, H pouches after proctocolectomy with ileo anal anastomosis; Koch's pouch for ileostomy; lleal pouch for bladder replacement or cutaneous ureterostomy. Colonic. Sigmoid reservoir or caecal/ileocaecal reservoir is often used to replace urinary bladder. Pathological pouch,s

Experimental Natural pouches physiological pouch,s

• Zenker's • Heidenhein pouctr. diverticulum causing To study gastric pharyngeal pouch physiology done in • Physick's pouch in stomach the rectal wall in • Pavlov pouch: between rectal valves Gastric pouch • Hartmann's pouch created with is protruded retaining vagii pathological to study gastric gallbladder physiology infundibulum

• Rathke's pouch in pituitary • Seesse/'s pouch in pharynx • Morrison's hepatorenal pouch • Rectouterine pouch of Douglas

I Principles Blood supply should be preserved. Mesentery should extend adequately. Bowel is opened on the antimesenteric border and sutured using vicryl posterior and anteriorly. Created pouch is anastomosed to the required area from its summit or from open end.

I Complications

I Procedure

Obstruction (25%) is the common complication. It may be due to stomal stricture, internal hernia, volvulus and adhesions. Pouch disruption and leak can occur which is usually treated conservatively. Pelvic abscess formation in ileoanal pouch is confirmed by clinical features of fever, back pain, raised total count, CT scan. It is treated with antibiotics, drainage per abdomen or reservoir excision and ileostomy. Pouchitis: It is the common problem (20%). Tenesmus, bloody diarrhoea, spasm, back pain, fever, cramps, dehydration are the presentations. It is treated conservatively with antibiotics, hydration, probiotics. Vaginal fistula formation. Anal canal stricture formation. Faecal incontinence. In urinary conduits, sepsis, acidosis, electrolyte imbalance, stone formation, urine leak are the complications.

Laxative is given previous evening. Enema is given on same day before doing the procedure. About one litre of barium sulphate solution is infused into the colorectum per anally through enema tube. In children 12F Foley's catheter is used. Once complete filling occurs, X-ray is taken. Patient is asked to evacuate the barium and postevacuation film is taken. After that, air is inflated into the colon which gives a better contrast to visualise thin mucosa of the colon (air-contrast barium enema). Contraindications: Acute colonic conditions.

>

m

:::1:1 N N

...rQ)

CV

::I

CV

::I CV

Note:

BARIUM ENEMA It is the contrast X-ray done to visualise large bowel. Therapeutic barium enema is done in intussusception in children. Barium enema

Indications Carcinoma colon lleocaecal tuberculosis

Finding Irregular filling defect Pulled up caecum, obtuse ileocaecal angle Filling defect, incompetent ileocaecal valve Ulcerative colitis Loss of haustrations, lead pipe appearance Colonic polyps Smooth, regular filling defect Congenital mega colon Narrow zone, zone of cone, dilated proximal segment Diaphragmatic hernia Colonic shadow in the left thoracic cavity Gastrojejunocolic fistula Leak into the stomach from colon

Figs. 22.58A to C: Barium enema X-ray in different indications should Fig. 22.57: Barium enema showing normal peristaltic movement.

n :c

cc

Pouch is contraindicated in Crohn's disease.

T

907

be taken with complete filling, postevacuation and after air-contrast.

Liberty means responsibility; that is why most men dread it.

Chapter

Intestinal Obstruction Classification II: Depending on Type of Obstruction

( iHAPTE R O UTLINE

•

• • •

• •

Intestinal Obstruction: Types Dynamic Obstruction Duodenal Atresia Small Intestine Atresia Mal rotation Meconium lieus

• •

• • • •

lntussusception Volvulus Sigmoid Volvulus Paralytic lieus Adhesions and Bands Internal Hernias

1. Acute: Common in small bowel.

2. Chronic. 3. Acute on chronic: Common in large bowel. 4. Closed loop obstruction .

Classification Ill: Depending on Site of Obstruction

INTESTINAL OBSTRUCTION: TYPES

nClassification I: Depending on Aetiopathology A. Dynamic. B. Adynamic. Dynamic

Proximal small bowel Site of Duodenum obstruction and jejunum

In the wall In the lumen

Hernia-25% Adhesions-40% Volvulus lntussusception

Tuberculous stricture Crohn's disease Malignancy

Gallstones Roundworm lnspissated faeces Meconium ileus

Cessation of peristalsis Postoperative period Electrolyte imbalance Spinal injuries Uraemia Diabetes mellitus Retroperitonealhaematomas and surgeries Renal surgeries Mesenteric ischaemia Pseudo-obstruction

Laro, bow,1

Ileum

Anywhere in large intestine

Causes:

Congenital Lipomas Leiomyomas Malignancy Bands and adhesions

Tuberculosis strictures Malignancy Crohn's Gallstones Herniascommon cause Roundworm Congenital

Malignancy Tuberculosis stricture Anorectal malformation Volvulus Congenital megacolon Bands

Clinical features:

Severe vomiting, dehydration, no or less distension, colicky pain

Central distension, vomiting, dehydration Central abdominal pain

Constipation, distensionearly; Late vomiting less pain

Plain X-ray

Valvulae conniventes

Characterless Central fluid level

Dilatation and haustratio n

Adynamic

Outside the wall

Distal small bowel

I Classification IV Congenital

Acquired

Anorectal malformations

Hernia (commonest)

Congenital megacolon

Postoperative

Adhesions

lntussusception

Duodenal atresia

Roundworm

Intestinal atresia (ilea!)

Gallstones

Bands and adhesions

Tuberculosis

Mal rotation

Malignancy

Volvulus neonatorum

Internal hernias

common. In developing countries both adhesions and hernia are the common causes of intestinal obstruction. Adhesions commonly cause small bowel obstruction than large bowel. Eighty per cent of intestinal obstruction occurs in small bowel; 20% in colon. 70% of colonic obstruction is due to malignancy. Other 30% is due to volvulus; diverticulitis, inflammatory cause like tuberculosis, etc. Mortality is 3% in obstruction without strangulation; 30% in obstruction with strangulation. Recurrent obstruction is more common in adhesions.

909

I Pathology and Pathogenesis Changes proximal to the bowel obstruction:

0

er

-"'.... C

• • Trichobezoars + Phytobezoars

Meconium pellets Foreign body • Food bolus Stercoliths

0

::::,

Intestinal obstruction still is a challenging, commonly acute condition in surgical practice. Adhesions are becoming the more common cause of intestinal obstruction than hernia nowadays. Common causes for adhesions are gynaecologic surgeries, appendicectomy, colonic and pelvic surgeries. 20% cases of intestinal obstruction are due to malignancy or its peritoneal carcinomatosis spread. More than 50% of intestinal obstruction in Western countries are due to adhesions; 20% due to malignancies; 10-15% due to hernias. Crohn's disease is also becoming a common cause of obstruction. Fig. 23 .1: Step ladder peristalsis with visible dilated bowel loop in

Note:

• Adhesions and hernias are most common causes of intestinal obstruction • Depending on degree of block intestinal obstruction can be complete, partial or partial circumferential. Subacute obstruction implies incomplete obstruction. • Depending on the vascular compromise it can be-simple obstruction with intact blood supply or strangulated with compromised blood supply. • 80% of intestinal obstruction is in small bowel; 80% of small bowel obstructions are due to benign cause. ?D-80%of largeintestine obstruction is due to malignancy, others being volvutus and tuberculosis.

intestinal obstruction due to adhesions - post-laparotomy patient. Intestinal obstruction -IIncreased peristalsis -IBecomes vigorous ,j,

Obstruction not relieved -IPeristalsis ceases

DYNAMIC OBSTRUCTION

,j,

It is mechanical blockage of normal propulsion and passage of intestinal contents. Obstruction may be external/internal; partial (incomplete or subacute)/complete; acute/acute on chronic/or chronic; si mpie/closed loop/strangulation; congenital/acquired ; proximal/distal. Earlier, hernia was the commonest cause of intestinal obstruction. Now adhesions (40%) are the commonest cause especially in developed countries and hernia being 15-25%

Flaccid, paralysed , dilated bowel ,

Fluid collects just proximal to the obstruction which is derived from saliva, stomach, pancreas and intestine. Because of oedema and inflammation absorption decreases, sequestration of fluid from the circulation into the lumen occurs and bacteria (E. coli, Klebsiella, anaerobes, bacteroides and other organisms) multiply, toxins are released-toxaemia occurs. This leads to severe dehydration, electrolyte imbalance.

Today's friction is tomorrow's destruction.

910

,, Intestinal wall hypoxia is also the cause for dilatation. ,, In first 12 hours of obstruction, there is only decreased absorption which causes accumulation of fluid and electrolytes in the lumen. ,, After 12 hours, there is also increased intestinal secretion causing further accumulation of the fluid . ,. Accumulation of bacterial toxins, bile salts, prostaglandins, and mucosa-derived free radicals, VIP-all increase the luminal secretion of fluid in obstructed bowel. ,, Dilatation of bowel wall increases intraluminal pressure which exceeds the bowel wall venous pressure causing ischaemia which causes further dilatation and ischaemic injury. This leads into eventual blockage of arterial perfusion causing bowel wall necrosis/gangrene. ,, Increased bacterial colony in the bowel (Normal flora is less than 106 colonies/ml in jejunum and 108 colonies /ml in the ileum) due to altered luminal content and environment multiplication toxins further mucosa damage disrupted mucosa! defense/barrier/integrity translocation of bacteria across mucosa into submucosa and also absorption of bacterial and other toxins into the bacteraemia/toxaemia/septicaemia/ SIRS/MODS.

i!"

...

Q)

en :::i

en 0

l'0 :::i C: l'0

en a:i

cc: en

Fig. 23.3: Postoperative internal hernia with obstruction.

,. Proximal to the collected fluid, air accumulates (derived from swallowed air(70%), diffusion from blood into the lumen (20%), from digested product and bacterial action (10%)), in which, main component is nitrogen (90%) and also hydrogen sulphide. During vigorous peristalsis, air enters the distal fluid , results in churning, is the reason to cause multiple air-fluid levels in plain X-ray abdomen. ,. Defective absorption, decreased fluid intake, loss of fluid by vomiting, sequestration of fluid into the bowel

lumen-leads into severe dehydration, fluid and electrolyte imbalance. ,. Inflammatory response in the bowel wall (intramural inflammation) causes accumulation of activated neutrophils and macrophages in the muscle wall which release reactive enzymes and cytokines. These substances damage secretory and motor process of muscle leading into dilatation of the bowel. Increased release of nitric oxide in muscle wall and production of intramural reactive oxygen metabolites alter gut motility and permeability.

Fig . 23.4: Multiple strictures in the small bowel with dilated bowel intestinal obstruction probably due to tuberculosis.

B • • • • •

Dilatation of the bowel Decreased absorption across mucosa Increased secretion into the lumen Intramural inflammation and hypoxia Increased intraluminal pressure Venous congestion and increased venous pressure Disrupted mucosa! bacterial translocation

_______,

Changes at the site of the obstruction: Initially venous return is impaired

Congestion, oedema of bowel wall occurs which turns purple

J.

Later this jeopardizes the arterial supply

J.

Growth

Loss of shineness, blackish discolou ration, loss of peristalsis J, Gangrene J, Perforation occurs

(Closed loop) Dilated loop

911

C"')

::z::

>

-g

-t

Bacteria and toxins migrate into the peritoneum J, Peritonitis

m

:::0 N

c,.)

Obstructed gangrenous bowel ring

Proximal dilated bowel

Distal collapsed bowel

Fig. 23.5: Gangrenous bowel with proximal dilated and distal collapsed bowel segments.

Note: • Causes for strangulations are-external like hernia (by constriction ring of the sac), adhesions, bands; compression in the wall causing mechanical block and compromised blood supply like in volvulus, intussusception; increased intraluminal pressure like closed loop obstruction; mesenteric ischaemia. • Morbidity, poor outcome and mortality are more common in intraperitoneal strangulation than in strangulation of hernia as it is localised with less fluid loss. • Outcome also depends on age of the patient, extent of the disease and time at which patient reaches for therapy. • Massive 3rd space fluid loss, dehydration, hypovolaemia, hypotension, hypochloraemia, hypokalaemia, metabolic alkalosis, oliguria, azotemia. haemoconcentration (after fluid therapy haematocrit falls indicating the need for blood transfusion). • Translocation of bacteria across oedematous small bowel wall causes septicaemia.

Closed loop obstruction: ,. When there is obstruction in th e large bowel , with ileocaecal valve competence (40%), pressure increases in the caecum J, Stercoral ulcer in the caecum J, Gangrene J, Perforation J, Peritonitis (Faecal) ,. Perforation also can occur at the site of obstruction due to the malignant growth.

f.J

Proximal Distal bowel bowel loop loop

Stercoral a IT) ulcer

Figs. 23.6A and B: (A) Closed loop obstruction. Here loop of the bowel is obstructed at its point of entry and exit creating closed loop; (B) Closed loop obstruction of ilea-caecal region.

,. Closed loop obstruction also can occur when bowel get obstructed at both proximal and distal parts of the loop of the bowel. It can occur in external or internal hernias, volvulus, etc. Necrosis and perforation are both common at obstructed site and over the convex summit of the bowel content. Bowel distal to the obstruction is inactive and collapsed.

B Proximal to obstruction Distension of bowet. - By gas due to swallowed air(70%), from blood in the lumen (20%), from bacterial action and digestion (10%); mainly N2 and H2S. - Due to fluid mainly of digestive juices which normally get absorbed, but in obstruction absorption ceases and accumulated fluid causes bowel distension (1500 ml saliva, 2000 ml gastric juice, 1000 ml bile, 1500 ml pancreatic juice and 3000 ml from small intestine [succus entericusl); often oedematous bowel wall further secretes more fluid into the lumen aggravating the distension. This fluid is toxic with high bacterial load. Peristalsis initially increases (borborygm1) to overcome obstruction; but later fatigue develops and peristalsis ceases. Distal to the obstruction-bowel initially empties the content and become collapsed and contracted remaining silent. At the level obstruction depends on cause strangulation, perforation, peritonitis can occur. Systemic features-Toxaemia, septicaemia, renal failure, ARDS, SIRS, MODS can occur.

I Clinical Features •·· Abdominal pain: ,. Initially colicky and intermittent: later continuous and severe.

The coexistence of i/1testinal colic and borborygmi, establishes the diagnosis of obstruction of the small intestine in more than 9 out of 10 cases. -Clarence Dennis

0

er

... C

0

:::,

912

,

Pain is the first symptom to develop which is sudden and severe. Initial colicky pain suggests obstruction and eventual diffuse persistent pain suggests strangulation. Pain begins usually around umbilicus in small bowel obstruction.

... = CIJ

CII

Cl)

ci

Fig. 23.7: Step ladder peristalsis seen in small bowel obstruction. Note the previous laparotomy scar signifies obstruction is due to adhesions. Laparoscopy and proceed-is the needed management.

,. In small bowel obstruction, it is crampy, recurrent paroxysms occurring as short crescendo/decrescendo episodes (of 30 seconds). ,,. In large bowel obstruction, it is of longer episodes of minutes (In paralytic/adynamic ileus, pain is diffuse and mild). Vomiting: ,, In jejuna/ obstruction, it is early and persistent. , In ilea/obstruction, it is recurrent occurring at an interval; initially bilious later faeculent. ,, In large bowel obstruction, vomiting is a late feature. Distension: , It is absent or minimal in case of jejuna/ obstruction , Obvious with visible intestinal peristalsis (VIP) and borborygmi sounds in case of ilea/ obstruction-Step ladder peristalsis. ,. It is enormous in case of large bowel obstruction. Constipation: ,,. It is absolute, i.e. neither faeces nor flatus is passed.

Richter's hernia obstruction Gallstone obstruction Mesenteric vascular occlusion Intestinal obstruction with a pelvic abscess

Dehydration: , Leads to oliguria renal failure. Features of toxaemia and septicaemia: , Tachycardia, tachypnoea, fever, sunken eyes, cold periphery. Abdominal tenderness: ,,. It is initially localised but later becomes diffuse-is a feature of intestinal obstruction. Rebound tenderness and guarding will not be present in simple obstructions which are features of strangulation. Features of strangulation: , Continuous severe pain, shock, tenderness, rebound tenderness (Blumberg's sign).

,, Guarding and rigidity, absence of bowel sounds. ,, In case of strangulated hernia, a swelling which is tense, tender, rigid, irreducible, no expansile impulse on coughing and history of recent increase in size is seen. Temperature: , Fever signifies inflammation in the bowel wall/ ischaemia/ perforation. ,, Hypothermia can occur when septicaemia develops due to lack of pyrogenic response. It suggests poor prognosis. Bowel sounds: They are increased-high-pitched metallic (rushes and groans) sounds followed by metallic tinkling sounds of dilated bowel. Eventually once fatigue occurs or gangrene develops, bowel sounds are not heard-silent abdomen of peritonitis develops (in paralytic ileus, there are only continuous metallic sounds of dilated bowel). Per-rectal examination: , Shows empty, dilated rectum, often with tenderness. If rectal growth is the cause for obstruction, it may be palpable. CARDINAL FEATURES OF INTESTINAL OBSTRUCTION

Colicky abdominal pain; vomiting; distension; constipation later. In high jejuna/ obstruction -no distension (scaphoid abdomen), severe bilious vomiting, no peristalsis, without constipation initially. In ilea/ obstruction -central moderate distension, bilious and faeculent vomiting, intermittent crescendo colicky pain, step ladder peristalsis, constipation at a later period. In lo w/colonic obstruction-variable colicky pain, right to left peristalsis, marked enormous distension, constipation to begin with, late feculent vomiting.

I

I Investigations Plain X-ray abdomen: (initially supine abdominal X-ray is taken; later if needed, X-ray in erect posture is tr1.ken if perforation is suspected). , Multiple air-fluid levels. ,, Proximal the obstruction Lesser the air fluid level. ,, Distal the More the air fluid level.

Fig. 23.8: Plain X-ray abdomen in erect posture showing dilated

bowel and colon- a feature of intestinal obstruction.

CT scan is very reliable investigation for intestinal obstruction. It has got 93% sensitivity; 94% accuracy and 100% specificity. In CT scan, small bowel loop >2.5 cm suggests dilatation. It can show dilated loop, transition zone and collapsed part which are definitive features of intestinal obstruction. It can also give idea of changes in the bowel wall, ischaem ia, strangulation, mesenteric oedema and thickening. It also shows bowel wall gas, portal venous gas and mass lesion. Barium (micro bar solution) enema or gastrografin contrast enema X-ray is useful in intussusception. [Barium meal is usually contraindicated in acute intestinal obstruction. However dilute (micro bar) barium meal/gastrografin meal follow through X-ray may be donewith caution in suspected subacute/ partial intestinal obstruction under fluoroscopy, otherwise it may precipitate complete obstruction or may cause perforation and barium peritonitis which is very dangerous]. Haematocrit, blood urea and serum creatinine; arterial blood gas analysis (acidosis is common), LFT, platelet count (in severe sepsis, there will be altered LFT with thrombocytopenia). Serum electrolytes estimation. Hypokalaemia is common. Total count is increased. But can be significantly low in severe stage of sepsis. Estimation of serum D lactate, CPK-BB isoenzyme, intestinal fatty acid binding proteins are different investigations may be useful to predict bowel ischaemia/gangrene. US abdomen is useful to see dilated bowel and fluid in the peritoneal cavity. It is better than X-ray but not as good as CT scan. It has got 95% sensitivity; 80% specificity; 80% accuracy. Doppler US is useful in detecting strangulation. Basic electrical rhythm of small bowel will be changed in ischaemia. It can be determined by noninvasive method using superconducting quantum interference device (SQUID). Differential diagnosis: Paralytic obstruction ; pseudoobstruction; ascites.

Fig . 23 .9: Plain X-ray abdomen showing multiple air fluid levels due to bowel obstruction.

Fig. 23.10: Plain X-ray showing valvulae conniventes with intestinal obstruction.

Normally, three fluid levels can be seen in plain X-ray film- at fund us of stomach, at duodenum and often at caecum. ,.. Jejunum shows concertina effect du e to val vulae conniventes (Herring bone pattern}- by the valves of Kerckring. , Ileum is smooth and characterless (by Wangensteen). ,.. Large bowel shows haustration. , Pneumobilia (gas in biliary tree) may be due to gall- stone ileus. ,. Distended caecum is shown as round gas shadow in the right iliac fossa. Dilated caecum signifies large bowel obstruction. ,.. Small bowel >3 cm diameter; proxi mal large bowel >9 cm; transverse colon >5.5 cm; sigmoid colon >5 cm are suggestive of intestinal dilatation. But this increased diameter need not suggest intestinal obstruction everytime.

m

TRIAD DF SMALL BOWEL OBSTRUCTION IN PLAIN X·RAY

Dilated small bowel loops >3 cm Multiple air fluid levels in erect X-ray Paucity of air in the colon

_

J

Fig. 23.11: CT abdomen showing dilated bowel loop; CT is ideal investigation for intestinal obstruction.

B •M:1iiii!ti•11i ~Ji,1,11•H·ii@··i+i·i!iiiii·ii•

Peritonitis; Moribund status • Hypovolaemic and septic shock Renal failure; ARDS Intra-abdominal abscess formation

Never insult an alligator unless and until you have crossed the river.

913

C")

:::c

>

"'lJ

-I

m

::::c N

c.)

0

=en

-... C

2

0 :::I

914

Treatment Nasogastric aspiration: To reduce toxic effects, to reduce bowel distension which indirectly improves pulmonary ventilation and to reduce possibility of aspiration pneumonia. Replacement of fluid and electrolytes. Antibiotics: Ampicillin, gentamicin, metronidazole, cephalosporins. Blood transfusion: FFP or platelet transfusions are often needed in critical patient. ICU critical carf!. Systemic management of complications like ARDS, DIC, SIRS are important. If there is hypotension, dopamine/dobutamine are also needed. CVP for fluid and monitoring. PCWP (pulmonary capillary wedge pressure) monitoring are often needed in haemodynamically unstable patient. Surgery. , Immediate laparotomy is done and the site {by finding the junction of dilated proximal and collapsed distal bowel) and cause of the obstruction is identified. The obstruction is relieved.

,. Fluorescein fluorescence study may be helpful on table to check the viability. 1000 mg of fluorescein is injected into the peripheral vein and bowel is inspected under Wood's UV light, nonviable loops are identified which requires resection and anastomosis. , If bowel is not viable, resection and anastomosis is done. A good peritoneal wash is given and the abdominal cavity is drained.

IB

Peristalsis; Pulsations Bleeding in mesentery and bowel wall Friability-friable, flabby muscle is seen in ischaemia Colour (black/pink)-dull and lusterless serosa is seen in ischaemia Serosal shining

Fig. 23.13: Gangrenous bowel-resected specimen in a case of mesenteric ischaemia.

,

, ,

Proximal dilated bowe1---~

l

J

Abdomen is closed in layers using nonabsorbable sutures (polyethylene, polypropylene, nylon). Often tension sutures are required. Small bowel can be decompressed using Savage 's decompressor. In case of right-sided colonic obstruction, right hemicolectomy with ileocolic anastomosis is done.

Reseciioa

Leagthy midlioe iocis~,

Anastomosis

Fig. 23.12: Bowel resection and anastomosis for gangrene in case of intestinal obstruction. Cheatle's cut on the antimesenteric margin of the collapsed distal segment is often needed to avoid discrepancy in luminal width. Single layer interrupted (silk/vicryl) or two layered continuous sutures can be used. Single layer is better in acute conditions.

,

,

Warm-saline soaked mop is placed over the doubtful area with 100% oxygen inhalation for 20 minutes; if colour becomes normal with peristalsis then bowel is viable. On table Doppler study may be useful.

Fig. 23.14: Colonic obstruction due to stricture/growth in the transverse colon; right hemicolectomy is done.

,

In case of left-sided colonic obstruction, left hemicolectomy(resection) and colo-colicanastomosis is done with a defunctioning colostomy(right-sided transverse) which is closed after 6 weeks.

Obstru·ction due to rectosigmoid growth with patient being severely ill-Hartmann's operation can be done to save the life of the patient wherein distal stump after removal of the growth is closed, proximal colon is brought out as end colostomy. ;.. Second look operation may be needed in doubtful cases or multiple segment obstructions in 24-48 hours to confirm viability. ,. Laparoscopic approach may be useful in partial obstruction, proximal obstruction, obstruction due to band . Conversion when needed, should be done without hesitation. ,. Acute postoperative obstruction is difficult to identify and manage. CT is very useful. Initially it is treated conservatively (90% ), but suspected ischaemic cases or persistent obstruction becomes an indication for surgery. Resection with exteriorization may be the choice.

B • Pelvic abscess • Subphrenic abscess • Biliary or faecal fistulas

Bands and adhesions lncisional hernias

DUODENAL ATRESIA It is the commonest site of intestinal atresia. It is usually a complete stenosis of the second part of duodenum at the level of ampulla of Vater. It is defective fusion of foregut and midgut with failure of recanalisation. Incidence is 1 in 10,000 live births.

I Types

Type 1: Duodenal complete atresia: It is the commonest type (50%). It is usually complete separation with intact wall. In 25% cases, complete separation with wall also occurs. I Type 2: Fibrous cord. Type 3: Incomplete or partial obstruction. It can be stenosis or web with an aperture. Duodenal diaphragm/web can present as complete; incomplete with a fenestra; incomplete diaphragm with central aperture which causes 'windsock' deformity due to proximal dilatation. Here actual diaphragm will be proximal to the site of obstruction. Duodenal atresia may be preampullaty(nonbilious vomiting) or postampullary (bilious vomiting). Postampullary is common (80-90%).

I Associations Duodenal stenosis is often associated with annular pancreas. It can be isolated duodenal atresia or in association with Down's syndrome (30%)/incomplete rotation of gut (20%)/ congenital heart diseases (30%)/trisomy (30%)/anorectal malformations (10%), etc. It is commonly associated with maternal polyhydramnios (50%). Antenatal US can confirm it. 50% infants are premature.

I Features Jaundice. Bilious/nonbilious vomiting immediately after birth. Features of gastric outlet obstruction. Dehydration. Electrolyte changes are common. Growth retardation of newborn due to deprived nutrition (by swallowed amniotic fluid in fetus).

I Investigations Plain X-ray shows classic double-bubble sign with absence of air in the distal part.

Proximal dilated duodenum Collapsed part Complete atresia with separation

Fibrous cord atresfa

Duodenal stenosls

Windsock deformity

Fig. 23.15: Types of duodenal atresia-

""ti -4

m

:::J:I N

c.)

0

CT Cl)

C:

0 ::I

Fig. 23.21: Diagram showing incomplete gut rotation and small bowel volvulus. Fig. 23.20: X-ray abdomen of a patient with intestinal obstruction in a newborn as neonatal intestinal obstruction. •·· Treatment , Resection and anastomosis is the choice of therapy. ,. Treatment of associated anomalies. , Tapering proximal jejunoplasty is done if extensive length of jejunum is involved. , Prognosis depends on length of atretic bowel; multiple atresias; associated anomalies.

MALROTATION It is interference in the process of normal rotation of midgut in fetus and its mesenteric fixation.

B • Stage 1: In 4th-8th week of intrauterine period, midgut supplied

by superior mesenteric artery (SMA) grows rapidly. As coelomic cavity cannot accommodate growing midgut during that period, it protrudes into the umbilical cord as physiological hernia. Stage 2. In 10th-12th week, midgut migrates into coelomic cavity. First, small bowel returns towards the left side of the abdomen. Then caecocolic loop returns to left lower abdomen. It rapidly rotates 270° counterclockwise to reach right iliac fossa. Then duodenojejunal segment rotates 270° counterclockwise to reach left of SMA and behind the colon. Stage 3: Fusion of different parts of mesentery and posterior peritoneum.

Reverse rotation: Final 180° rotation occurs clockwise bringing colon posterior to duodenum and SMA. , Hyper-rotation: Rotation up to 360° or 450° causing caecum on left side of the abdomen. , Encapsulated small bowel occurs while fetal midgut returns into coelomic cavity. Stage 3: Final defect in fixation causes mobile caecum and ascending colon leading into caecal volvulus.

,

B

ASSOCIATED ANOMALIES (20%)

Congenital diaphragmatic hernia of Bochdalek • Prune belly syndrome; Duodenal atresia; Oesophageal atresia

Acute/recurrenVsubacute intestinal obstruction. Midgut volvulus (30%) (usually clockwise rotation) with features of strangulation, perforation, peritonitis. Shock, septicaemia, passage of dark blood per rectum, oedema and erythema of anterior abdominal wall. In children- failure to thrive, recurrent abdominal pain, cyclical vomiting, constipation and diarrhoea. Investigations ,. Plain X-ray abdomen shows air fluid levels. , Barium meal (dilute/microbarium) and follow through X-ray is the investigation of choice.

If the hand ,s kept flat upon the abdomen the underlying coil may be felt to harden and soften alternately much like in a pregnant uterus, in a case of intestinal obstruction. - Arthur H Burgess

,. US abdomen/CT abdomen, if needed. ,. Haematocrit, serum electrolytes estimation. •·· Treatment ,. Resuscitation, antibiotics, fluid and electrolytes, blood transfusion.

There is hypertrophy and dilatation of the proximal ileum containing thick, viscid, tenacious dark green meconium. Distal ileum and colon are narrow and contracted having grayish meconium pellets. Meconium gets calcified very rapidly. Gangrene, perforation, volvulus can occur in 50% of cases. Intrauterine perforation causes fetal meconium peritonitis which leads to dense adhesions and calcification in peritoneum. Fetal meconium peritonitis is sterile.

Colon ~ ~ ~ ~Append~ cectomy Small bowel Incomplete rotation

,_____._ Proximal dilated ileum Narrow collapsed colon

Ladd's operation

Fig. 23.22: Ladd's operation for incomplete gut rotation. Ladd's band

is released. Duodenum is straightened. Appendicectomy is done.

,

Laparotomythrough horizontal incision is done. Clockwise rotated mid gut which is congested and cyanotic is identified. Untwisting of the midgut in counterclockwise direction is done. Viability of bowel is confirmed (colour, vessels in mesentery, peristalsis, on table Doppler). Ladd's band is divided large bowel is repositioned in left side. The entire duodenum is Kocherised and the ligament of Treitz is divided so that duodenum becomes straight towards right iliac fossa. This achieves wide root of the mesentery and places the small bowel in the right side of the abdomen thus preventing further volvulus. A complementary appendicectomy is done-Ladd's operation. ,.. After laparotomy, if bowel is gangrenous, it is resected and remaining parts of the bowel are exteriorised as enterostomies. A second look operation is done to look for viability of remaining bowel and also to maintain the continuity. Often it will be extensive bowel resection leading into poor prognosis.

MECONIUM ILEUS It is neonatal manifestation of fibrocystic disease of the pancreas wherein thick meconium, which is viscid and pastelike, gets collected in the terminal ileum. Because of inspissation it forms a firm bolus leading to obstruction of the ileum. Neonates present with features of ileal obstruction as well as respiratory dysfunction, exocrine pancreatic insufficiency, and high salt in the sweat (Na+ and c1- more than 90 mmol/L). Meconium ileus occurs in 15% of patients with cystic fibrosis. Cystic fibrosis is an autosomal recessive disease involving bronchioles, exocrine pancreas and sweat glands. Condition is common in Caucasians. Exocrine pancreatic insufficiency and malabsorption is seen in 90% of patients with cystic fibrosis.

Fig. 23.23: Pathology of meconium ileus. Note the meconium pellets

in the distal ileum and colon; thick viscid meconium in proximal ileum. Plain X-ray shows calcified meconium pellets with multiple air fluid levels which appear as 'soap- bubbles' (Neuhauser sign). Vomitus of the patient which does not conta in trypsin, when poured on the exposed X-ray film, will not digest the gelatin of the film whereas the vomitus of individual with normal pancreas will digest the gelatin of X-ray film-very useful test. Pilocarpine, a cholinergic drug is injected into skin to stimulate the sweating and collected sweat (100 µg sweat) is analysed for sodium and chloride. Elevated albumin level in meconium, sodium level assay in nail clipping and serum immune active trypsin assay are other investigations.

B Commonly associated with cystic disease of pancreas but not necessarily always Respiratory dysfunction Exocrine pancreatic insufficiency

• High salt in the sweat >90 mmol/L lleal obstruction Soap-bubble appearance in X-ray • Gelatin in X-ray film will not get digested by patient's vomitus Bishop-Koop operation, ileostomy, dissolving ileal meconium pellets are required

B Intestinal bolus obstruction; Perforation and peritonitis Gangrene, volvulus formation

I Treatment Nonoperative measures Dissolution through enema can be tried using gastrografin which is hyperosmolar and contains Tween 80 as dissolving agent. Gastrografin is diatrizoate meglamine with Tween 80 (polysorbate 80) as dissolving agent. N acetylcysteine 10% also can be used for irrigation per-anally. N acetylcysteine 10% wash through nasogastric tube also can be used-5-10 ml 6th hourly. Treatment for cystic fibrosis.

919

B Nonoperative wash per rectally using acetyl-cysteine or gastrografin through Foley's catheter passed per anally. Acetyl cysteine can also be passed for irrigation per orally through a Ryle's tube as 10 ml 6th hourly. Bishop-Koop and Santulli operations done in very sick neonates. Resection and anastomosis is ideal when child is adequately fit.

lleostomy

0 ::I

Fig. 23.25: Santulli operation done for meconium ileus wherein proximal

ileum is brought out as ileostomy. Distal ileum is sutured to proximal.

B Hirschsprung's disease; Duodenal atresia Intestinal atresia; Malrotation Midgut volvulus/volvulus neonatorum Meconium ileus; Anorectal malformation L..__

- - - - - - - - - '

INTUSSUSCEPTION (ISS)

I Definition

It is telescoping or invagination of one portion (segment) of bowel into the adjacent segment.

1. Antegrade: Most common. 2. Retrograde: Rare (jejunogastric in gastrojejunostomy stoma).

IIn elderly intussusception:

Colocolic is most common type is formed usually by growth ole of hydrostatic reduction

--------

Fig . 23 .24: Bishop-Koop operation. Here distal ileum is brought as

ileostomy and proximal part sutured to distal bowel.

-I

m

:J:I CA)

I Types Colon

> "'ti N

lleostomy

Operative Measures Present standard surgical approach-Enterotomy with irrigation using warm normal saline or 4% N acetylcysteine and complete evacuation of all meconium pellets (or can be pushed down into large bowel manually) closure of the enterotomy is present standard method. N acetylcysteine, by breaking the disulphide bonds of the meconium, separates it from intestinal mucosa allowing its retrieval. A T tube can be placed in the small bowel through which N acetylcysteine wash can be given repeatedly if further meconium needs dissolution. Other Methods When patient's condition is critical with obstruction, BishopKoop operation is done. Proximal dilated segment is resected and resected end is anastomosed to the side of the distal collapsed ileum. End of the distal ileum is brought out as ileostomy. Through the ileostomy gastrografin or acetyl cystine wash is given regularly to dissolve meconium pellets. This ileostomy can be kept for long time. Continuity is maintained at later period. Santulli operation is done where proximal ileum is brought as ileostomy for irrigation using a fine tube and distal ileum is sutured to proximal ileum as end to side anastomosis. Resection and anastomosis is ideal if patient is fit and if proximal bowel is suitable for anastomosis.

n

:c

It can be single or multiple (rare). It can be ilea-colic (most common type, 75%), colocolic, ileoileocolic, colocolic. It is common in weaning period of a child (common in male), between the period of 6-9 months. It is the commonest cause of intestinal obstruction in children of 6- 18 months age.

The remedy for injuries is not to remember them.

I Pathology

920

Apex

:·"'r pt,m Figs. 23.26A and B: (A) Typical intussusception-on table look;

(B) Parts of intussusception.

I Aetiology Idiopathic ISS is common in children, occurs in terminal 50 cm of ileum. During weaning, change in diet causes inflammation and oedema of Peyer's patches-may stimulate ISS. Upper respiratory tract viral infection which causes oedema of Peyer's patches is also thought as an aetiology for intussusception in children. Other causes in adolescents and adults are submucous lipoma, leiomyoma, polyps in jejunum (Peutz-Jegher syndrome), other polyps and carcinomas with papillary projections.

B

CAUSES

Change in diet during weaning Upper respiratory tract viral infection Intestinal polyps; Submucous lipoma Leiomyoma of intestine; Meckel's diverticulum Carcinoma; Purpuric submucosal haemorrhages

Apex is the one which advances; lntussuscipiens is the one which receives (outer sheath); lntussusceptum are the tubes which advance (middle and inner sheath). ,. Apex and inner tubes will have compromised blood supply which leads to gangrene. , Because of ischaemia, apex sloughs off and bleeds, which mixes with the mucus to produce the classic red-currant Jelly that is passed per anum. ,. Gangrene which sets in leads to perforation and peritonitis. ,. Red currant jelly is not commonly observed in ISS in adult, but it can occur.

I Clinical Features Common in males (3:2). Common in 6-9 months. But can also occur at later agegrouped children. Common in spring and winter, coinciding with the gastroenteritis and respiratory infections in respective periods. Commonest cause of intestinal obstruction in infancy. Initial colicky abdominal pain (75%) which eventually becomes severe and persistent. Sudden onset of pain in a male child, with progressive distension of the abdomen, vomiting, with passage of " red-currantJel/y" stool. It is usually not found in adult ISS. Often ISS is recu rrent, when it gets reduced, ch ild automatically becomes asymptomatic (Mother often complains "Bachha rotha he, Bachha sotha he". It means child cries during an episode and sleeps peacefully once it gets reduced).

Sausage-shaped mass

Fig. 23.28: Sausage-shaped mass of intussusception.

Fig. 23.27: lntussusception. Different parts and apex point is also clearly seen.

••• On examination, a mass is felt either on the left or right of the umbilicus which is sausage shaped with concavity towards umbilicus, smooth, firm, resonant, not moving with respiration, mobile, contracts under the palpating fingers. Often mass appears and disappears.

Right iliac fossa is empty (Sign of Dance). After 24-48 hours, abdominal distension appears and increases progressively with features of intestinal obstruction. Features of intestinal obstruction with step-ladder peristalsis. Blood-stained stool is often obvious on digital examination of the rectum. Occasionally ISS can be seen per anally and felt with a long mesentery. Eventually, gangrene and perforation occurs with features of the peritonitis.

• Palpable mass (85%) - Sausage-shaped smooth, firm mass - Mass does not move with respiration - Mobile in all directions Resonant - Mass contracts under the palpating fingers - Mass appears and disappears • Empty right iliac fossa • Features of intestinal obstruction/peritonitis-later

Doppler may show mass with doughnut sign and is useful to check blood supply of bowel. Plain X-ray abdomen shows multiple air fluid levels. CT abdomen is needed . Treatment Initial management: Ryle's tube aspiration; IV fluids; Antibiotics; Catheterisation.

Later management Nonoperative management Reduction by hydrostatic pressure using either warm saline or microbarium sulphate solution or air (popular in China). Barium or saline is infused into the rectum through a catheter (Foley's catheter). Under fluoroscopy, reduction can be observed. Child will pass large quantity of air and faeces; distension reduces; child shows recovery and stops crying. Air or contrast enters the proximal bowel freely. Palpable abdominal mass disappears. Rare complication is perforation of colon. It is done in early stage within 24 hours of presentation. 70% cases of ISS will respond to nonoperative method. It is contraindicated in complete obstruction; perforation and peritonitis.

Fig. 23.29: Arare condition where colonic intussusception has occurred through rectum , coming out per anally. Part is already gangrenous.

B

I Differential Diagnosis In children: In adults:

Acute gastroenteritis. Purpura with intestinal symptoms. Carcinoma colon. Mesenteric mass.

I Investigations

Figs. 23.30A and B: Barium enema showing the typical 'Claw sign' of intussusception.

B

Barium enema shows typical claw sign or coiled spring sign (Pincer end). Ultrasound shows target sign or pseudokidney sign or bull's eye sign, which is diagnostic.

I

INDICATIONS FOR SURGERY IN ISS

ISS more than 48 hours • Features of perforation, strangulation, peritonitis Recurrent ISS In adult commonly, resection is required

In the field of observation, chance favours only the prepared mind.

921

::I Q)

0

CT

....

= C')

= 0 ::I

Surgery

... Cl)

Cl

::s

Cl)

0

Cape's method: If reduction does not occur, laparotomy is done under G/A. By gently milking out the intussusception with warm packs, it is reduced. After reduction, viability of the bowel is checked carefully. If manual reduction is not possible, it is understood that the bowel is likely to be gangrenous which requires resection and anastomosis. In case of viable bowel, often terminal ileum is anchored to the ascending colon and Jackson veil band is cut. Patient also requires nasogastric tube aspiration, IV fluids, antibiotics. Appendicectomy should be done after reduction of the intussusception. Laparoscopic approach may be used to reduce the intussusception. ,:. If intussusception persists for more than 48 hours or intussusception in adult requires resection. , lleocolic resection is sufficient.

B

SIGMOID VOLVULUS (Volvulus of Pelvic Colon) It is common in Asia, common in India (7% of intestinal obstruction) and especially South India because of high fibre diet. ,:. It is very common cause of large bowel obstruction in Peru and Bolivia due to high altitude. ,:. More common in males and old age. It is common in patients with chronic constipation with laxative abuse. It is common in: f

Ogilvie's syndrome Mentally-retarded individuals Chaga's disease Hypothyroidism

Anticholinergic drugs Multiple sclerosis Scleroderma Parkinson's disease

RECURRENCE RATE

In hydrostatic reduction- 10% In open manual reduction-2% In resection-very less "'Cl

....m :x, N

w

0

-

"' .C.. &. 0

CT

=

Chapter

Appendix ( )HAPTER OUTLIN E

•

• • • • •

Surgical Anatomy Acute Appendicitis Incidental Appendicectomy Appendicular Mass Appendicular Abscess Faecal Fistula after Appendicectomy

• •

•

Mucocele of Appendix Neoplasms of the Appendix Laparoscopic Appendicectomy

SURGICALANATOMY It is located at the terminal end of the caecum where three taeniae join, about 2 cm below the ileocaecal orifice. Usually, around 5- 10 cm in size but can be variable. Size of its lumen is that of matchstick. Diameter of appendix is 3- 8 mm; diameter of lumen is 1-3 mm (matchstick). Mesoappendix is extension of the mesentery contains appendicular artery, a branch of ileocolic artery. Often an accessory appendicu lar artery (of Seshachalam) may be present. Thrombosis of these vessels leads to gangrenous appendicitis.

Fig. 24.2: Different anatomical positions of the appendix.

B Type A: Partial duplication in a single caecum. Type B: Two separate appendices in a single caecum. Type C Double caecum with each one having one appendix.

Most common position is retrocaecal (75%). Next common is pelvic (21%).

I Other sites are: Preileal (anterior)-rare (1%) Postileal (splenic)-rarest Paracaecal

Fig. 24.1: On table demonstration of mesoappendix with appendicular artery. Note the appendix (A); mesoappendix (B); caecum (C); ileum (D); taenia (E).

Subcaecal -2% Subhepatic

Often it may be in subserosal plane leading to difficult dissection during appendicectomy. In situs inversus appendix is on the left side. Mucosa of appendix is lined by columnar cells with crypts. Submucosa contains numerous lymphatic aggregations (follicles)(abdominal tonsil). Opening of the appendix into caecum is guarded by 'valve of Geralch'.

Single caecum double appendix

Type C

Viral infection may cause mucosal oedema and inflammation which later gets infected by bacteria causing appendicitis. Family history may be relevant in 30% of appendicitis in children with appendicitis occurring in first degree relatives. Obstruction of the lumen of appendix causing obstructive appendicitis. ,. Blockage occurs due to-faecoliths, stricture, foreign body, round worm or threadworm. ,. Adhesions and kinking-carcinoma caecum near the base, ileocaecal Crohn 's disease. Distal colonic obstruction. Abuse of purgatives. Faecolith is the most common cause. Organisms:

Fig. 24.3: Duplication of appendix.

E. coli (85%), enterococci, (30%), streptococci , Anaerobic streptococci, Cl. welchii, bacteroides. Pseudoappendicitis is appendicitis due to acute ileitis following Yersinia infection. It is often due to Crohn's disease.

Fig. 24.5: Removed appendix with its mesoappendix.

I Pathogenesis Fig. 24.4: Laparoscopic view of subhepatic appendix.

ACUTE APPENDICITIS The partial inflammation of the peritoneum, in the liac fossa, is sometimes set up by disease in the Appendix caeci... . The appendix having been perforated by ulcerations, occasioned by the lodgement of the faecal concretions in its cavity, extravasation takes place, and inflammation of a more severe and serious kind is originated.... Nature sometimes succeeds in limiting the inflammation to a part of the right side; but it is at other times diffused over the whole abdomen .. .and quickly proves fatal. -Thomas Hodgkin, 1836

Acute inflammation of the mucus membrane with secondary infection without obstruction causes acute nonobstructive appendicitis. It may lead into resolution, fibrosis, recurrent appendicitis or eventual obstructive appendicitis. Luminal obstruction by faecolith, lymphoid hyperplasia, pinworm (oxyuris vermicularis), other worms, foreign body, carcinoma/Crohn's mucus and inflammatory fluid collects inside the lumen increases intralum inal pressure leads to blockage of lymphatic and venous drainage resulting in increased oedema of mucosa and causes

I Aetiology It is common in young males. It is common in white races. •·· Fibre rich diet prevents appendicitis. Less fibre diet increases chance of appendicitis. It is common in May and August-seasonal variation- often called as epidemic appendicitis.

Fig. 24.6: Severely infected appendix with faecolith inside. Faecolith

is one of the causes of obstructive appendicitis.

Never explain- your friends do not need it and your enemies will not believe it anyway. Our friends show us what we can do; our enemies teach us what we must do.

929

... Q)

Cl

:::s

-

Cl)

C

n,

= C n,

mucosal ulceration and ischaemia bacterial translocation bacterial spread through submucosa and muscularis propria acute obstructive thrombosis of appendicular artery ischaemic necrosis of full thickness of the wall of the appendix gangrene of the appendix perforation at the tip or at the peritonitis . After perforation localisation by greater omentum and dilated ileum occurs with suppuration and pus inside forming appendicular abscess.

:E en

ao cc

en Fig. 24.9: Gangrenous obstructive appendicitis-on table.

2. Acute obstructive appendicitis: Here pus collects in the blocked lumen of appendix which is blackish, gangrenous, oedematous and rapidly progresses leading to perforation either at the tip or at the base of appendix. This leads to peritonitis, formation of appendicular abscess or pelvic abscess. Most often, there will be thrombosis of the appendicular artery. 3. Recurrent appendicitis: Repeated attacks of nonobstructive appendicitis leads to fibrosis, adhesions causing recurrent appendicitis. 4. Subacute appendicitis is milder form of acute appendicitis. 5. Stump appendicitis is retained long stump of appendix after commonly laparoscopic appendicitomy.

Fig. 24.7: Laparotomy showing perforated appendix with peritonitis.

I Clinical Features

Fig. 24.8: Appendicolith with appendicitis.

In severe acute appendicitis localisation can occur by omentum and dilated ileum without pus inside forming appendicular mass. Acute appendicitis with blockage at the opening of the lumen inflammation rarely subsides mucus collects inside the lumen of the appendix resulting in its enlargement Mucocele of the appendix.

Umbilicus

_.0

-

-i13

--- - McBurney's point

i\nterosuperior·...,___-~ iliac spine

I Types

McBurney's incision (Gridiron/McArthur)

1. Acute nonobstructive appendicitis (catarrhal) (mucosa/ appendicitis):

Inflammation of mucous membrane occurs with redness, oedema and haemorrhages which may go for following courses: • Resolution • Ulceration Fibrosis Suppuration

Spino-umbilical line

Recurrent appendicitis Gangrene-rare initially in nonobstructive type but later can occur Peritonitis.

1

Fig . 24.10: McBurney's point is junction of lateral 113rd and medial 213rd of spinoumbilical line. McBurney's/Gridiron's/McArthur's incision is perpendicular to this line in this point.

It is rare before the age of two, common in children and other age groups. Pain: It is the earliest symptom. Visceral pain starts around the umbilicus due to distension of appendix, later after few hours, somatic pain occurs in right iliac fossa due to irritation of parietal peritoneum due to inflamed appendix. Pain

eventual ly becomes severe and diffuse which signifies spread of infection into the general peritoneal cavity. Vomiting: Due to reflex pylorospasm.

suppuration occurs in the localised area resulting in appendicular abscess.

931

C")

::c

>

-0 -4

m

:::J:I N

.,:,.

>

Sherren's triangl e -- - ~

Umbilicus

Pubic symphysis

Fig. 24.12: Sherren's triangle. It is area of hyperaesthesia found in acute appendicitis between anterosuperior iliac spine; umbilicus and pubic symphysis.

B B

MURPHY'S TRIAD

• Pain- first; Vomiting- next; Temperature- last '--

Constipation is the usual feature but diarrhoea can occur if appendix is in postileal or pelvic positions. ,:. Fever, tachycardia, foetor oris are other features. Urinary frequency: Inflamed appendix may come in contact with bladder and can cause bladder irritation. Tenderness and rebound tenderness at McBurney's point in right iliac fossa (release sign-8/umberg's sign) are typical. Rovsing's sign: On pressing left iliac fossa, pain occurs in right iliac fossa which is due to shift of bowel loops which irritates the parietal peritoneum. Hyperextension (in case of retrocaecal appendix-Cape's psoas tes~ or internal rotation (in case of pelvic appendixobturator test) of right hip causes pain in right iliac fossa due to irritation of psoas muscle and obturator internus muscle respectively. Baldwing's test is positive in retrocaecal appendix-when legs are lifted off the bed with knee extended, the patient complains of pain while pressing over the flanks.

P/R examination shows tenderness in right side of the rectum. Hyperaesthesia in 'Sherren's triangle'. This triangle is formed by anterosuperior iliac spine, umbilicus, pubic symphysis. Often infection gets localised by omentum, dilated ileum and parietal peritoneum leading to appendicular mass. Often

Rovsing's sign Blum berg's sign (Release sign) • Cape's psoas test • Obtu rator test Baldwing's test Bastede sign

Oumphy's cough tenderness sign (Refer fascinating signs for detail) Bapat bed shaking test Heel Drop test

Acute appendicitis in infancy: ,. Even though it is rare, when it occurs, it has got 80% chances of perforation with high mortality (50%). Acute appendicitis in children: ,. Here localisation is not present, and so peritonitis occurs early. ,. It requires early surgery. Dehydration, septicaemia are common. In elderly: ,. Gangrene and perforation are common. Because of lax abdominal wall, localisation is poor and so peritonitis sets in early. In pregnancy: ,. Incidence is 1 in 2,000 pregnancies. It is more common in 1st and 2nd trimesters. ,.. Appendix shifts to upper abdomen. So pain is higher and more lateral. ,. Rebound tenderness and guarding may not be evident. ,

co :::, a. >
12,000/ cm-2; USG features-6. ,, RIPASA scoring system (2010)-with 15 parameters. , Anderson scoring system-8 parameters. Contrast CT scan is very much useful when diagnosis is difficult especially in old people. Dilated appendix; dilated lumen; thickened wall; nonfilling of the lumen by contrast or air; periappendicular fluid collection; presence of mass/abscess/ associated pathology like carcinoma can be identified. It has 95% sensitivity and specificity with 95% accuracy. Dirty fat thickened mesoappendix, appendicular phlegmon, appendicular faecolith and thickened caecum funneling contrast into the orifice of the appendix as arrowhead sig1r-are all relevant features in CT scan. C-reactive protein, even though nonspecific increases in acute phase. 99mTc HMPAO labeled leukocyte imaging may give guidance in deciding the management. MRI is very useful tool in pregnancy. Plain X-ray may show lumbar scoliosis towards right due to psoas spasm which is not uncommon; faecolith on the right side; obliteration of preperitoneal fat line due to retrocaecal appendicitis; segmental ileus in caecum and terminal ileum; speckled extraluminal gas in right iliac fossa, gas in appendix, pneumoperitoneum (very rare); intestinal obstruction (occasionally only); soft tissue mass in mass or abscess of appendix-all these features are very much nonspecific. X-ray is useful to rule out DU perforation, intestinal obstnuction, ureteric stone.

Appendices epiploicae are absent in rectum, appendix and caecum.

l>

"Cl 'C

CL >C

934

I Treatment Surgery-Appendicectomy: Approaches

C

«I

=

C:

«I

:E:

_en

m en

a:

1. Gridiron incision: Incision is placed perpendicular to the right spinoumbilical line at the McBurney's point (i.e. at the junction of lateral one-third and medial two-third of spinoumbilical line). (Gridiron is a frame of cross beams to support a ship during repairs. This incision was first described by McArthur). 2. Rutherford Morison's muscle cutting incision (Muscles are cut upwards and lateralljl). 3. Lanz crease incision centering at McBurney's pointcosmetically better. 4. Right lower paramedian incision/lower midline incision-when in doubt or when there is diffuse peritonitis. 5. Laparoscopic approach: Becoming popular and better. 6. Fowler-Weir approach by cutting muscle medially over the rectus.

Fig. 24.16: Appendix, on table during appendicectomy.

• Gridiron_,._._____.., . t--- - - - t - -. - Right ....',:: paramedian Lanz

Fig. 24.15: Approaches for appendicectomy.

I Procedure

Under general anaesthesia, skin is incised. Two layers of superficial fascia are cut. External oblique aponeurosis is opened in the line of incision. Internal oblique and transverse muscles are split in the line of fibres. Peritoneum is opened in the line of incision. Caecum is identified by taeniae, and ileocaecal junction. Omentum when adherent is separated. Appendix is held with Babcock's forceps. Mesoappendix with appendicular artery is ligated. Using thread or silk, a purse-string suture is placed around the base of the appendix. Base of the appendix is crushed with artery forceps and transfixed using vicryl (absorbable). Appendix is cut distal to the suture ligature and removed. Stump is cleaned with antiseptics. Purse string suture is tightened so as to bury the stump. In difficult cases-Retrograde appendicectomycan be done. In presence of pus or burst appendix, the peritoneal cavity is drained. Postoperatively, IV fluids, antibiotics are given. Once bowel sounds are heard, oral diet is started.

Figs. 24.17A and B: Burst appendicitis showing pus. Tip of appendix is burst causing collection of pus adjacent. Usually proximal site of appendix just distal to the site obstruction will burst.

935

:r:,,

AP.e_endix stump

Fig. 24.18: Steps in open appendicectomy. Note the base of appendix, mesoappendix; its ligation; transfixation of the base using 2 zero vicryl; cutting of appendix and closure of the wound. Burying is not essential; it is optional. Burying is done using silk/vicryl using purse string suture.

B Paralytic ileus Reactionary haemorrhage due to slipping of ligature of the appendicular artery Residual abscess (pelvic, paracolic, local, subdiaphragmatic) Pylephlebitis (portal pyaemia) Adhesions, kinking and intestinal obstruction Right inguinal hernia (direct)- 18,000/- are suspected features of rupture Mortality rate of appendicectomyis less than 1%. Morbidity and complications are more after surgery for perforated appendicitis Surgical site infection is 5% in uncomplicated appendicitis; 20% in perforated appendix after surgery Small bowel obstruction postoperatively is 1% with simple appendicitis; 3-5% in perforated appendicitis after surgery. More than 50% of obstruction occurs in first year of postoperative period • In children with appendicitis, there is poor localisation and so peritonitis is common. So conservative therapy should be avoided. Surgery is the only choice of treatment otherwise early peritonitis is the danger. Appendicular mass is initially treated with Ochsner Sherren regime. After 6 weeks, interval appendicectomy is done. Children, old age, faecolith, laxative abuse, diabetes mellitus, immunosuppression and pelvic appendix are high-risk factors for perforation in appendicitis In pelvic and retrocaecal appendicitis, adjacent ureteral inflammation can occur in which urine on analysis shows blood cells and pus cells Contd...

937

Contd...

Incidence of removal of normal appendix is 30% Stump appendicitis is inflammation and infection in the remaining portion of the appendix in the stump after appendicectomy. It is a rare entity On table during surgery, normal appendix if found, it is called as 'Lily white appendix'. Then other pathology like Meckel's, ileal/ mesenteric lymph node/ovarian disease has to be looked for

("')

::c

>

""D -4

m

:a N

It is not done in Crohn's disease (during acute phase), postradiation, immunosuppression, aortoiliac grafts.

Appendicular mass

l>

APPENDICULAR MASS (Periappendicular Phlegmon) It is the localisation of infection occurring 3 to 5 days after an attack of acute appendicitis.

•

Inflamed appendix

(D

:::I

a. >
2 cm in size. It stains chromograninB immunohistochemically. Distant and

:c > -a -I

m N

.,::..

>

"Cl "Cl (D

::I

CL

>

"Cl "Cl CD

::I

Q. )(

Chapter

Rectum and Anal Canal ( jHAPTER OUTLINE

• • •

•

• • •

• • • •

• • • •

Surgical Anatomy of Rectum Surgical Anatomy of Anal Canal Per-rectal Examination (Digital Examination of the Rectum) Proctoscopy (Kelly's) Sigmoidoscopy Colonoscopy Carcinoma Rectum Solitary Ulcer Syndrome Rectal Prolapse Anorectal Malformations Pilonidal Sinus/Disease Piles/Haemorrhoids • External Piles Anal Fissure • Sentinel Pile Anorectal Abscess Fistula-In-Ano • Low-Level Fistula • High-Level Fistula

• •

• • • •

• • • • • •

Anorectal Strictures Condyloma Acuminata Anal lntraepithelial Neoplasia Malignant Tumours of Anal Area Sacrococcygeal Teratoma Anal Incontinence Descending Perinea! Syndrome Proctitis Proctalgia Fugax Hidradenitis Suppurativa of Anal Region Pruritus Ani Gastrointestinal Haemorrhage • Upper GI Bleed • Lower GI Bleed • Obscure GI Bleed

SURGICAL ANATOMY OF RECTUM It is the distal portion of the large gut in the posterior pelvis, placed between the sigmoid colon above and anal canal below; in front of last three pieces of sacrum and coccyx (from S3). Rectosigmoid junction is 15 cm from the anal verge. The three cardinal features of large intestine (sacculation/haustration, appendices epiploicae and taeniae) are absent.

The upper third of rectum is covered by peritoneum on front and sides, mid third only on the front, lower third is infraperitoneal. Upper third is related in front to intraperitoneal structures and sacrum behind; middle third is related in male to urinary bladder, Oenonvillier's fascia in front and Waldeyer's fascia behind; in females, cervix and vagina in front. Lower third in front related to prostate, seminal vesicles, behind to pelvic floor; in females, behind to vagina. The rectum is pulled forward by the puborectalis muscle forming the anorectal sling which is primarily responsible for rectal continence. Rectosigmoid junction implies a segment of bowel comprising the last seven centimetre of sigmoid colon and upper five centimetre of rectum. On sigmoidoscopic examination it is taken as a point 15 cm from the anal verge. Rectum has got three lateral flexions left, right and left from below upwards (Valves of Houston are present in the mucosal part). Holy avascular plane of Heald (RJ Heald, 1987) posteriorly is important in creating the plane; this is the ideal avascular plane of dissection Neurovascular bundle of Walsh in Denonvillier's fascia-at 2 and 10 o'clock positions is important to retain both in rectal and urinary bladder dissections. Superior hypogastric plexus and two hypogastric nerves posteriorly behind Waldeyer's fascia should be retained after identification during posterior dissection of the rectum. Supports of rectum ,. Pelvic floor and muscles. ,. Fascia of Waldeyer. It is the condensation of pelvic fascia behind the ectum, contains superior rectal vessels and lymphatics. ,. Lateral ligaments of rectum: It is the condensation of pelvic fascia, attaches rectum to the posterolateral wall of lesser pelvis.

,

Denonvillier's fascia: It is the fascial condensation which separates rectum from prostate in males. ,. Perinea/ body It is in front of the anorectum. Arterial supply: Rectum is supplied by rich network of vessels that originates from superior (from inferior mesenteric artery, IMA), middle and inferior rectal arteries (from internal iliac arteries) and median sacral artery (from abdominal aorta). Venous drainage: Submucosal rectal venous plexus surrounds rectum, communicates vesical plexus in males and uterovaginal plexus in females. Internal plexus joins superior rectal vein; External plexus- upper one joins superior rectal vein; middle to middle rectal vein and lower to inferior rectal vein. Superior rectal vein-formed by internal venous plexus (6 main tributaries)-drains inferior nmesenteric vein (IMV); middle rectal vein-by middle part of the external venous plexus-drains to anterior division of internal iliac vein; Inferior rectal vein-by inferior part of the external venous plexus drains internal pudenda! vein. Lymphatics: Lymphatic drainage from upper half of rectum is to inferior mesenteric nodes; from lower half to internal iliac and sacral nodes. Nerve supply: Sympathetic-L1 L2; inferior hypogastric plexus- motor to internal sphincter; inhibitory to rectal muscle. Parasympathetic-S2 S3 S4-pelvic splanchnic nerves- inhibitory to internal sphincter; motor to rectumnervi erigentes. Pudenda/ nerve (S2,3,4) supplies external anal sphincter. Sensation of distension is carried through parasympathetic; pain sensation is carried by both.

SURGICAL ANATOMY OF ANAL CANAL It is 4 cm long, extends from levator ani muscle to anal verge. On either side ischiorectal fossae are present. Rectum pierces pelvic diaphragm at anorectal junction to continue below as anal canal. lschiorectal fossa-pyramidal shape; 5X2 cm sized; A/cock's canal carries pudenda! vessels and nerve; lunate fascia lines above. Parts ,. Upper part-15 mm; anal columns of Morgagni, anal valves, anal crypts, anal glands, pectinate/dentate line. ,. Middle part-15 mm-transition pectin, white line of Hilton; pale, thin, glossy, squamous epithelium without sweat glands. , Lower part-8 mm-true skin with sebaceous and sweat glands. The dentate line. It represents the former site of the embryonic anal membrane. The lining of the canal above this line is columnar epithelium and below is skin. The mucosa above this line has an autonomic nerve supply, below is by pudenda! nerve. The venous drainage above this line is by inferior mesenteric and portal circulation, whereas below to

systemic venous circulation. Internal haemorrhoids develop above this line. Surgical anal canal is 4.5 cm exte nd s more superior; anatomical anal canal is 3.0 cm. It is important in assessing malignancy extension and its dissection.

943

C"')

:c

l>

....m ::::c N

(11

,w/-J.li.l~,..:1---Anal valve

AV.MM:JW~

-.H~::::-- Pectinate line

~ ....-~ ~ = ./- Middle part15 mm

'- = Al

944

Blood supply Arterial-from inferior rectal artery. Venousfrom internal rectal venous plexus lies in the submucosa of the anal canal. It drains mainly into the superior rectal vein but communicates freely with external plexus . It is an important site of portosystemic communication. They are situated in anal column at 3,7,11 o'clock. Their saccular dilatation forms 'primary internal piles'.

C

n,

= C n,

:ii:: _II)

CCII

a:

Fig. 25.3: Triple loop of Shafik: (A) Puborectalis; and (8) Deep part; (C) Superficial part; (D) Subcutaneous part of external anal sphincter.

L1 L2 . . .

v.,

Levator ani: Conglomeration of three striated musclespuborectalis; pu bococcygeus; iliococcygeus. Puborectalisinnermost; causes 120° angulation with anal canal; closes the lumen by closing the posterior and side walls of the rectum; other two close the urogenital diaphragm and so elevate, steady, straighten and suspend the rectum.

L3

L4 _ . _ _ _ _ Superior hypogastric plexus (presacral nerve)

LS Hypogastric nerve

-+-- - Sacral flexure Perinea! flexure Pubic bone

I

Sacral bone

Inferior hypogastric plexuses (right and left) Fig. 25.6: Nerve supply of rectum and anal canal

Puborectal --',._"""..._ sling of levator ani

..._-=-..,.

I Continence Mechanism

Fig. 25.4: Levator ani with puborectalis.

Superior rectal artery Internal iliac artery

Middle rectal artery

Fig. 25.5: Blood supply of rectum and anal canal.

Continence is the ability to retain intestinal contents voluntarily or involuntarily until an evacuation is desired-Ho/schneider. Anorectal continence is complex dynamic process involving the striated muscle com plex (levator ani + 3 parts of external anal sphincter); internal sph incter (smooth muscle complex); perinea! body, anorectal ring, rectal ampulla, rectal valves; motor, sensory and autonomic systems. Continence during sleep and rest is by continuous contraction of internal sphincter; continence during raised intra-abdominal pressure is by levator ani and external anal sphincter. Continence is present in normal intrarectal pressure (30 mmHg); levator ani external anal sphincter (triple loop of Shafik) creates 20 mm Hg of pressure and internal sphincter 10 mm Hg. S2 component of the pudenda! nerve (S2) originates from the spinal cord Onuf nucleus which controls the continence. Continence is a dynamic process involving sensory and motor reflux with muscles and neural systems. Defecation is also a compkex dynamic process having three stages-holding (contraction of puborectalis, external and internal sphincters); initiation (relaxation of puborectalis and external sphincter, contraction of levator ani, diaphragm and abdom inal muscles); completion (rectum contracts, internal and external sphincters relax).

945

SIGMOIDOSCOPY

PER-RECTAL EXAMINATION (Digital Examination of the Rectum-Refer Chapter 16) Note: No abdominal examination is completewithout aper-rectal examina-

tion. It is done to palpate carcinoma rectum; Stricture rectum; Polyps; Thrombosed piles; BPH and carcinoma prostate; secondaries in the rectovesical pouch (Blumer shelf); sphincter tone; pelvic abscess (is felt as boggy swelling). To feel the internal opening of anal fistulas. In bimanual palpation of the bladder or pelvic tumours. In acute abdominal conditions-it reveals dilated empty rectum with tenderness.

Annual sigmoidoscopy for all, after their fortieth birthday: something to look forward to. - Henry George Miller, 1968

j

---

C"')

::2:

:z:,,

"'0

m

::::D N UI

:::c n,

C')

C:

3

c» ::s

C.

> ::s c»

• Lithotomy position Knee-elbow position

Right lateral position • Left lateral position Dorsal position in ill-patients Picker position: Patient in standing position leans forward by rasping a chair or stool. This method is used to palpate seminal vesicles which is involved by tuberculous seminal vesiculitis (as craggy feeling) or in trichomonas vaginalis infestation of seminal vesicle.

U

-rectal examination is contraindicated in acute fissure-in-ano.

C")

c» ::s c»

Fig. 25.8: Rigid sigmoidoscope with inflation J

PROCTOSCOPY (KELLY'S) Indications ,. Diagnostic-piles, fissure-in-ano, polyps, stricture, etc. ,. Therapeutic-injection therapy for partial prolapse or piles, cryotherapy for piles, polypectomy, biopsy for carcinoma rectum or anorectum.

Obturator

balloon and biopsy forceps. It is used to visualize rectum and sigmoid colon, take biopsies from suspected lesions and do therapeutic procedures (polypectomy, control of bleeding, etc.). There are two types: 1. Rigid- 25 cm long, with illumination. 2. Flexible--60 cm long. In lateral position as in P/R examination or proctoscopy, sigmoidoscope with obturator is passed into the rectum and obturator is removed. Rectosigmoid is inflated with air and scope is negotiated into the sigmoid through Alpha (a) manoeuvre. Looked for any disease, biopsies are taken and also any required procedu re is done. Note: Precaution: Care should be taken in acutely inflamed sigmoid colon,

because chance of perforation is high.

m

Figs. 25.7A and 8: Types of proctoscopes:

(A) Non-illuminating; (B) Illuminating. Types: Illuminating; Non illuminating. Parts (10 cm): Proctoscope is conical shape, with proximal diameter more than the distal, so as to illuminate the light at the required site properly. Obturator is the inner part which allows the easy insertion of the proctoscope. Technique of proctoscopy: After doing digital examination, proctoscope with the obturator is introduced inside, through the anal canal in the direction towards the umbilicus. The obturator is removed. Proctoscope is withdrawn and during the course of withdrawal, any pathology has to be looked for. Note: Acute anal fissure is contraindication for proctoscopy.

COLONOSCOPY It is 160 cm long, flexible; Technique is same as sigmoidoscopy, but is passed up to the caecum. Technique: It is often done under GA using propofol or with laryngeal mask airway (LMA). It can be also done under high sedation. But patient finds difficult to tolerate pain and distension. Passage by elongation; looping with a manoeuvre; dither-torquing (clockwise-anticlockwise rotations) methods are used. Difficulty is encountered while passing through sigmoid colon, splenic flexure, and hepatic flexures. Continuous air inflation is important. It is better to visualise the lumen and then pass the colonoscope. Otten it can also be negotiated into the terminal ileum. Changing position, abdominal pressure is required for better

Use your imagination not to scare yourself to death, but to inspire yourself to life.

948

C)

ca

=

C

ca _rA

m

a: (I)

negotiation of the colonoscope. Technique differs in patients after haemicolectomy or through colostomy. Indication Bleeding per rectum, resistant anaemia. To take biopsies from different parts of the bowel. To identify synchronous growths, ulcerative colitis. To remove polyps. When barium enema shows irregularity. For therapy-colonoscopic polypectomy, dilatation of stricture colon, fulgaration.

Alcohol and smoking increases the risk. FAP and adenomas are more prone to carcinomas.

Contraindication: Acute ulcerative colitis. Advantage: It helps to visualise full length of the colon. Disadvantage: Takes a long time and requires expertise to do

the same. Hazards: Perforation of bowel, splenic flexure is the commonest site; Trauma to anorectum; Sepsis; Haemorrhage; Problems of incomplete therapeutic procedures.

CARCINOMA RECTUM Bubo is an apostem breeding within the anus in the rectum with great hardness but little aching. This I say, before it ulcerates, is nothing else than a hidden cancer.... Out of bubo (cancer) goes hard excretions and sometime they may not pass, because of the constriction caused by the bubo, and they are retained firmly within the rectum .... I never saw nor heard ofany man that was cured... but I have known many that died of the toresaid sickness. -John of Arderne, 1414

It is common in females. In 3% of cases, it occurs in multiple sites (synchronous). •·· Usually originates from a pre-existing adenoma or papilloma (tubular polyp). Any tumour within 15 cm proximal to the anal margin is called as rectal tumour/cancer. More than 95% are adenocarcinoma. It is characterized by chromosomal instability. Microsatellite instability (MSI) is rare in rectal cancer.

I Aetiology Red meat and saturated fatty acids increase the risk. High fibre diet reduces the risk.

Figs. 25.9A and B: Rectal polyp- it is premalignant condition.

Figs. 25.11A and B: Pathological specimens of anal canal, rectum and sigmoid colon after abdominoperineal resection for low rectal carcinoma and midrectal carcinoma.

Villous adenoma has 40% chance of turning malignancy, size more than 2 cm is at high-risk. Ulcerative colitis; Crohn's disease; HNPCC carries higher incidence of carcinoma of rectum. Family history of rectal cancer-any first degree relative of a person with rectal cancer will show two times increased risk of carcinoma rectum. Risk of developing other cancers like of endometrium (40%); stomach (20%); biliary tree (20%); ovary (10%) in the same patient also increases. 'Adenoma-carcinoma sequence' like in carcinoma of colon is known common method of occurrence.

Gross: It can be: Ulcerative; Papilliferous; Infiltrative Annular. It is common in rectosigmoid junction. Diffuse type: Often observed in patients with ulcerative colitis

which carries poor prognosis Histologically: It is adenocarcinoma which may be:

I

Well-differentiated-10% Moderately differentiated-BS% Undifferentiated-25% COLLOID CARCINOMA OF THE RECTUM

It is 12% common in young people Types

Primary and secondary Secondary colloid carcinoma is common type and is due to mucoid degeneration of adenocarcinoma itself. Primary is mucus within the cell with displaced nucleus (signet ring). Primary type has got poorer prognosis compared to secondary.

I Spread Local spread: Initially, it spreads, locally circumferentially (takes 12-18 months to complete the circumference of the bowel). Later spreads out to the muscular coat and peri-rectal tissue. Then to prostate, bladder, seminal vesicles in males, and uterus and vagina in females. Posteriorly into the sacrum and sacral plexus, laterally into the ureters.

m

HAGGITT'S INVASION OF MALIGNANT POLYP (SIMILAR TO CARCINOMA COLON) In pedunculated polyp

Level 0-noninvasive carcinoma over the summit Level 1-invasion to head of pedunculated polyp Level 2- invasion to neck of the pedunculated polyp Level 3-invasion to stalk of the pedunculated polyp Level 4-invasion to base of pedunculated polyp In sessile polyp-all lesions are level 4

Spurious diarrhoea: It occurs in early morning due to overnight mucus accumulation in the rectum causing urgency for defecation, but results in spurious diarrhoea with incomplete evacuation. Tenesmus: It is painful incomplete defecation with bleeding. Bloody slime-. Mucus with blood in stool. Sense of incomplete evacuation, constipation. Presenting as piles due to proximal venous congestion by tumour or as fistula in perianal region (which itself is tumour extension into the anal canal). Anaemia, malnutrition, loss of appetite and weight. Altered bowel habits. Urinary symptoms are due to infiltration of bladder or prostate. Back pain, due to invasion of sacral plexus. Ascites, liver secondaries, urinary symptoms. Ninety per cent of rectal growths can be felt by per-rectal examination. Depth of tumour penetration can be assessed through digital examination as superficial tumours are mobile; deep penetrating tumours are not mobile. Investigations ,, Proctoscopy; Sigmoidoscopy. ;.,- Biopsy usi ng Yeoman 's forceps. ,. Barium enema in case of FAP and synchronous growths. ,, Colonoscopy is ideal to rule out presence of any synchronous growths proximally (5%) or polyps. ;... Even though colonoscopy is done rigid proctosigmoidoscopy is a must to identify the precise location of the tumour and to measure the tumour distance from anal sphincter accurately. ,, Ultrasound abdomen-to look for secondaries in liver, ascites.

Note:

• For Dukes, modified Duke's, Astler-Coller's and TNM (8th edition, 2018) refer Chapter 22-Large Intestine (refer Page 895). • Primary rectal cancers are divided into 4 groups-very early; early; intermediate; locally advanced. • For sessile adenoma, Kikuchi sm (submucosal invasion) classification is used (in T1) depends on depth of submucosal invasion: sml-upper (inner) 113rd; sm2-middle 113rd; sm3-lower 113rd. • Lymphatic spread: Above the peritoneal reflection, spread occurs upwards along the colonic lymph nodes. In mid-rectum, into the pararectal and mid-rectal lymph nodes. Downward spread is rare occurs when growth is close to the anal canal into the inguinal lymph nodes. Obturator nodes may be involved in 8% of lower rectal growths. • Venous spread occurs to the liver 35%, lungs 20%, adrenals 10%and other areas. • Perineura/ spread carries poor prognosis.

I Features Bleeding pe r rectum/anu m (may mimic haemorrhoids)earliest symptom.

Fig. 25.12: Air-contrast CT of colon and rectum showing significant

narrowing in rectosigmoid junction. ,. CT scan to see operability, local extension, size, nodal status, ureteral involvement, presence of perforation or fistula. CT is very useful to assess nodal status. Local extension is better assessed by TRUS. Any mesorectal node detected in CT is considered as malignant spread.

In the case of acute abdomen, it is more important to insert the finger into the lower end than to put the thermometer into the upper end of the alimentary tract. -Sir Zachary Cope

947

C")

::c :r> '"Cl

-4

m

::x, N CJ1

:D (ti C')

c 3 c»

::I CL

>

::I

c»

n

c» c»

::I

948

Liver secondaries are well-identified in CT. Ureteral involvement in CT scan signifies requirement of stenting prior to surgery. Chest CT is essential to look for secondaries.

. ,. --..._,,-v. .

r

. ,.

'-

~-

tfC,.,.: •.

_·

--

Figs. 25.15A and B: MRI showing carcinoma of rectum and adjacent

structures. MRI is good method to evaluate the spread and staging. ,. Blood tests like haematocrit; CEA; blood urea and serum creatinine; serum electrolytes and proteins for management purpose. CEA estimation-it is raised in metastatic disease. It is important during follow-up after treatment. :

DIFFERENTIAL DIAGNOSIS

Inflammatory stricture Amoebic granuloma Tuberculosis

Surgery

Fig. 25.14: CT scan showing carcinoma of rectum.

;... Endorectal ultrasonography-very useful to assess the local extent of the tumour. Transrectal ultrasound (TRUSJ/endorectal ultrasound gives more accurate picture of primary tumour, layers, perirectal tissues and nodes. TRUS is superior in T staging of rectal cancers. Its accuracy is 95% compared to MRI (85%); and CT (75%). Endorectal US based T staging and N staging used now depends on layers involved and presence of nodes. TRUS detects nodes more or equal to 5 mm size. ;... Endorecta/ coil MRI (EC MRI) is very useful as it gives larger field of view compared to TRUS; extent, adjacent organ spread are better assessed by MRI. Recurrent tumour is better assessed by MRI. ,. F/uorine-18 fluorodeoxytlucose PET scan is useful to detect recurrent local tumours; metastatic disease; to detect pathologic response in preoperative chemoradiation. PET is not accurate for nodal spread .

Principles Surgery is the main method of treatment. Preope rative chemoradiotherapy is often used if growth is invading into adjacent tissues (T4). Adjuvant chemotherapy and radiotherapy is a must. Genetic, morphologic, biologic features of rectal cancers are similar to colonic cancers. But anatomical factors make it more complex than colonic cancers, like its location deep in the pelvis, relation to important structures like ureters, bladder, genital, autonomic nerves and anal sphincters. So surgical approach is very difficult. Avascular endoplelvic fascia/ plane is important during dissection to avoid injury laterally to autonomic nerves (will cause impotence in men and bladder dysfunction in both sexes); more medial dissection leads into incomplete clearance and high local recurrence. Abdomino perinea/ resection (APR) is the gold standard. But if tumour is well-differentiated and if there is adequate margin above the anal canal, a sphincter saving anterior resection (AR) can be done. Low anterior resection (LAR) is possible if EEA stapler is used for anastomosis. But anterior resection should not be done by compromising the adequacy of tumou r clearance. Tumour clearance is still the priority in rectal cancer as it decides the eventual outcome. Total mesorectal excision (TME) should be the goal in all procedures as mesorectum contains nodes and lymphatics, clearance of which gives better result.

,, It is a sharp dissection (not blunt) in avascular areolar plane between fascia of rectum which encroach the mesorectum and parietal pelvic wall fascia. ,, Mesorectum should not be breached. ,, Absolute haemostasis, preservation of autonomic nerves and dissection under vision are the essential principles. ,, Both layers of membranous anterior Denonvillier's fascia should be dissected off the prostate and seminal vesicles in male to have proper clearance. ,, In TME for middle and lower rectum, entire mesorectum should be removed. ,, For upper rectal tumours, TME is done 5-6 cm below the lower margin of the tumour. ,, TME improves the quality of life in relation to impotence, retrograde ejaculation and urinary incontinence. These complications are around 50% or more in APR whereas in TME it is less than 20%. Recommended distal rectal margin clearance is 5 cm, however 2 cm distal margin is an acceptable clearance. Circumferential resected margin-CRM (radial margin, >2 mm) is more important than proximal and distal margin. A 5 cm clearance of mesorectum from the primary tumour is essential as tumour implants can occur only for up to 4 cm from primary tumour margin. Principles to be followed-adequate lymphatic and vascular clearance; en bloc resection of primary tumour; no/less touch technique; avoiding spillage; adequate radical surgery. At least 12 lymph nodes' examination is recommended. Ultra-low anterior resection or intersphincteric resection (Turnball- Cutait) can be considered in low rectal tumours. After resection (on table) irrigation of rectal bedwith cetrimide or hypertonic solution like distilled water is often practiced as they are tumoricidal. Selection of the procedure AR or APR is decided by proper staging using TRUS and MRI. Neoadjuvant chemotherapy or neoadjuvant chemoradiotherapy is often used in T3 lesions which also may avoid APR; and AR may be sufficient. Local wide excision approaches are often used when tumour is

"'ti -4

Q.

Treatment for complete prolapse Aim of treatment

To control the prolapse; to restore continence; to prevent constipation. In young males, abdominal repair should be avoided as it injures pelvic nerves leading to sexual impotency. So perinea! approach is better. Rectopexy is fixing the rectum to sacrum by sutures or mesh after complete mobilisation of the rectum. Laparoscopic rectopexy using polypropylene mesh and sutures gives good result and has become very popular.

:c

QI ::II

I Surgery-Types

B

C")

(") QI ::II QI

956

Many advocate laparoscopic mobilisation and fixation of mobilised rectum to sacral promontory using polypropylene sutures without mesh. Laparoscopic sigmoid resection and rectopexy(Laparoscopic resection rectopexy, LRR) is done when there is rectal prolapse with constipation, with excess redundant sigmoid colon with kinking. Well's operation Polyvinyl alcohol sponge is wrapped around the mobilised rectum and is fixed to sacrum. Infection, fistula formation is high. Polypropylene mesh is used as a modification now instead of polyvinyl sponge; wrapping is only partially done to reduce the incidence of constipation. Ripstein operation After mobilisation of the rectum , 5 cm width Teflon mesh sling is passed around the rectum to fix it behind to fascia 5 cm below and in front of the sacral promontory. Sling is also fixed in front and laterally to rectum. Lahaut's operation Rectosigmoid is mobilised fully; mobilised loop of rectosigmoid is passed in front through posterior rectus sheath behind the rectus muscle; extraperitonealisation is done to pull the rectus forward to prevent descent. Other abdominal procedures Goligher's operation: Rectum is entirely mobilised up to anorectal ring and its posterior muscular layer is fixed to presacral fascia using interrupted polypropylene sutures. Devadhar rectal plication: Through abdominal approach, junction between thicker lower part and thinner upper part of the intussusception is identified. A purse string suture using silk is placed in front and laterally; further 3-4 interrupted submucosal Lambert sutures are placed to create reverse intussusception. Rosoe Graham operation: After mobilisation of the rectum, levator muscles are exposed and sutured in front of the rectum along with removal of pouch of Douglas. In Dumphy operation, it is done through combined abdominoperineal approach with perinea! rectosigmoidectomy. Both procedures control prolapse well but not incontinence. Muir low anterior resection: In a redundant rectosigmoid with prolapse, rectosigmoid resection is done; excision of redundant pouch of Douglas is done; rectum is fixed to sacrum behind. Perinea/ Procedures Delrome's operation (Mucosal sleeve resection and plication) After prior bowel preparation, under spinal anaesthesia, in lithotomy position, completely prolapsed rectum is held with

Babcock's forceps. 1 in 2,00,000 adrenaline solution is injected into the submucosal plane of the rectum to cause haemostasis. By making longitudinal incision, with sharp scissor an d cautery dissection, mucosa is stripped off from the deeper muscular layer from 1 cm below the anal margin to the apex of the prolapsed rectum. Muscular layer of rectum is plicated using absorbable vicryl 2-zero interrupted sutures all around; approximately 12-15 plication stitches are needed. These sutures are tied after finishing the passage of all sutures. Cut end of the mucosa of the apex is sutured to anal margin using interrupted vicryl sutures. It is technically easier. But incontinence may persist and recurrence chance is high. Other perinea/ procedures Perinea/ posterior fixation of the rectum of LockhardtMummery: Retrorectal space is dissected through perinea! approach and posterior rectal wall is sutured to sacrum and coccyx, additionally placing a retrorectal gauze pack to stimulate adhesions. Wyatt operation: Through perinea! post-anal approach dissected retrorectal space is placed with a Marlex/mersilene mesh which is sutured high to the sacral promontory and rectal wall laterally. Mickulicz Miles perinea/ transanal rectosigmoidectomy/ amputation of prolapse: Prolapsed rectum is excised and sigmoid is sutured to the anal margin. Aftemeier's rectosigmoidectomy: After rectosigmoidectomy, colonic anastomosis and pelvic floor is supported by suturing puborectalis muscle in front of the rectum using nonabsorbable sutures. Anal encircling :.- In 1891 , Thiersch did anal encircling using silver wire to provoke inflammatory fibrosis as well as to give mechanical support to the anal orifice-Thiersch wiring. It is done under local anaesthesia. Two small incisions are made at lateral parts of the anal canal. Silver wire is passed around deeper to these incisions and tied after placing the index finger into the anal canal. Wire can be removed after 12 months. Polypropylene, nylon are the other materials that can be used. Complications. Pain due to wire erosion, infection, faecal impaction, incarceration, high recurrence of 50% or more. In chi ldren with rectal prolapse, temporary wiring along with Goodsall's ligature or injection sclerotherapy using thick catgut are often advocated. ,, Supralevator high encirclement ofanal orifice (of Notaras) is done above the level of levator muscles by placing Teflon or nylon ribbons through anterior and posterior incisions. It gives better support and will not cut through. Thoralsksen modified this by placing plastic/mersilene tape around the bowel high up.

T

11Features

Procedures tor repair ol rectal prolapse

Perinea/ operations

Abdominal operations: Open/1.aparoscopic

• Mucosal sleeve resection (Delrome's)

• Suture rectopexyGoligher, Lahaut, Devadhar, Rosoe Graham

• Perinea! rectosigmoidectomy (Altemeier's)

• Prosthetic or mesh rectopexy-anterior/ posterior- Well's, Ripstein

• Posterior fixation of the rectum of Lockhardt-Mummery

• Resection rectopexyMuir's

• Wyatt operation

• Anterior resection

• Mickulicz Miles perinea! transanal rectosigmoidectomy/ amputation of prolapse

• Lahaut's operation

957

C'")

:::c

>

"'ti

-I

m

:a N

c:.n ::z, Cl)

n

c 3

Fig. 25. 29: Anal stenosis.

Q)

:::I

CL

Newborn presents with inability to pass meconium, abdominal distension, features of intestinal obstruction, improper anal dimple, sometimes with complaints of passing meconium per urethra.

• Anal encircling (Thiersch)

I Complications of Surgery Injury to hypogastric nerve causing impotence. Bladder dysfunction. Bleeding from sacral venous plexus. Injury to rectum and colon causing faecal fistula. Constipation after rectopexy is a known complication. Recurrence of prolapse. Improper correction of continence occurs in 50% cases. lnfection-proctitis/pelvic abscess, etc.

ANORECTAL MALFORMATIONS (ARM) It is due to imperfect fusion of the post-allantoic gut with the proctodaeum. Incidence is one in 4500 newborns. Wingspread classification (Refer table on next page)

Figs. 25.28A and B: Anorectal malformation with fistula in (A) Females; (B) Males. Note: Present classification of ARM: Nonsyndromic ARM

• Nonsyndromic ARM with fistula - Rectoperineal - Rectourinary (with bladder neck, bulbar or prostatic urethra) in males - Rectovestibular, rectovaginal, cloaca! in females. • Nonsyndromic ARM without fistu/a--imperforate anus • Nonsyndromic complex fistula---cloaca with short common channel 3 cm; Hshaped rectovaginal fistula; rectal duplication. Syndromic ARM-associated with different syndromes.

Fig. 25.30: Anorectal malformation, Cloaca type.

It can be associated with-cardiac anomaly, trachea-oesophageal fistula, renal anomalies, spinal anomalies. VATER/ VACTERL anomaly. Most common ARM in boys is rectobulbar fistula with fistula beginning distal to puborectalis from distal rectum to bulbar urethra. Most common ARM in females is rectovestibular fistula. Investigations ,. Wangenstein's invertogram: Usually done 6-12 hours after birth, so as to allow air to reach the rectal pouch. A metal coin (marker) is strapped at the presumed site of anus and X-ray is taken. Length between the rectal pouch and anal dimple marker is more than 2.5 cm in high anal fistula. - In low fistula, rectal pouch is distal to the Stephen's line (Pubococcygeal line). - In intermediate, pouch is at the level of ischial spine (Kelly's point). - In high fistula, rectal pouch is proximal to the Stephen's line.

He who is angry is seldom at ease.

>

:::I Q)

(") Q)

:::I Q)

958

... Cl)

Cl

::I

Cl)

0

Wingspread classification of anorectal malformations ('Wingspread'-name of the place where the conference was held) Low It is below the level of pelvic floor, (Puborectalis). Easy to diagnose and treat with good outcome. It may be: • Covered anus • Anovestibular fistula • Anal stenosis • Anal membrane

lnt,rm,diate

High

It occurs at the level of puborectalis, with or without fistula

It can be with or without a fistula into the bladder urethra, uterus, vagina It may be: • Anorectal agenesis • Rectal atresia • Cloaca (only in females, with confluence of rectum, vagina, bladder and urogenital sinus)

Murugassu's technique: Through visible anal dimple, meconium is aspirated by passing a needle into the rectal pouch in sitting propped up position. Watersoluble iodine dye is injected. Lateral X-ray is taken to study the level through Stephen line and Kelly's point. US abdomen; Evaluation of cardiac fu nction is also important; MRI spine.

I Treatment 1. In low fistula, single stage reconstruction is done under GA with very good results. i. Anoplasty. ii. Anovestibuloplasty. iii. Anal dilatation. iv. Incision of anal membrane.

PILONIDAL SINUS/DISEASE (JEEP BOTTOM; DRIVER'S BOTTOM) Pi/us-hair; Nidus-nest It is epithelium lined tract, situated short distance behind the anus, containing hairs and unhealthy diseased granulation tissue. It is due to penetration of hairs through the skin into subcutaneous tissue. It forms granuloma/unhealthy granulation tissue in the deeper plane. It is of infective origin and occurs in sacral region between the buttocks, umbilicus, axilla. It is common in hair dressers (seen in interdigital clefts), jeep drivers. It is common in 20-30 years of age. It is common in males and mostly affects hairy men. ()...-···t··••... , ""0-- Secondary,sinus

II

I

II

• • Pubic bone ~ "

I

I

/

i I

/ I

II \ \ I I \\ II I\ ' ....,_..,; / // / I \ I

· \

,

\

'

.,,,

,/

/

o... . . - ~ Primary sinus

I I ff I 1 I

j

'

t

Anal canal

·:f Ii

1

•

Coccyx

' ....__ ...., /Pubococcygeal

Fig. 25.32: Site and types of pilonidal sinus .

(Stephen's) line

Fig. 25.31: Stephen's line.

2. In high fistula , initial colostomy is done. Later definitive proced ure, i.e. Pull through operation through puborectalis and anastomosis of rectal pouch to create the anal canal is done. Closure of colostomy is done later. Posterior sagittal anorectoplasty is commonly done procedure. Note:

Level of rectal pouchand normal/abnormal sacrum are the deciding factors for good results.

m

COMPLICATIONS

Infection Faecal fistula Stenosis

Colitis Malnutrition Faecal incontinence

Fig. 25.33: Typical site of pilonidal sinus in the sacral region. Note

the primary and secondary sinuses.

and stay there to get buried deep into pilonidal sinus. Depth, narrowness, friction movements in the natal cleft; soft/macerated skin with erosions, splits, wide skin pores, wounds, presence of moisture and sweat are other factors.

B

Fig. 25.34: Recurrent pilonidal sinus. 25-40% of pilonidal sinuses can recur.

Commonest site: lnterbuttock sacral region.

I Pathology

Congenital theory is no longer considered; it is an acquired entity Hair follicles have never been demonstrated in the wall of the sinus (only hairs have found) Number, sharpness, nature, shape of hairs; depth and narrowness of the natal cleft; friction movements; nature of the skin, moisture and sweating are the factors predisposing pilonidal sinus Hair need not be local, tract always traverses cephalad Male preponderance- 74%, male sex hormone effect, hairy body, more sweat and maceration Occurs in young-20-29 years, who are having active pilosebaceous glands Dark haired-stiff hairs, rare in Negroes Obese and overweight-deep natal cleft Prolonged sitting Many procedures for treatment are available with each one having their own advantages and disadvantages

I Features Discharge-either serosanguinous or purulent. Pain-throbbing and persistent type. A tender swelling seen just above the coccyx in the midline (primary sinus); and on either sides of the mid line (secondary sinus). Figs. 25.35A and B: Specimen of typical excised pilonidal sinus. Note the tuft of hair.

Hair penetrates the skin ,J, Dermatitis ,J, Infection ,J, Pustule formation ,J, Sinus formation ,J, Hair gets sucked into the sinus by negative pressure in the area ,J, Further irritation and granulation tissue formation ,J, Pus forms ,J, Multiple discharging sinus

Primary sinus occurs in the midline. Secondary sinus occurs laterally (paramedian). Note:

• Theories like Preen gland, medullary canal vestige, traction dermoid, inclusion dermoid are no longer accepted. Now it is considered as •

acquired condition. Types of hair (H), force of hair insertion into subcutaneous tissue (F), vulnerability of the skin (V) are the three factors that cause pilonidal

sinus. Number of hairs collected, acuteness of root end of hairs, type of hair- tough/silky, shape of hair- straighVcurled, scaliness of hair are the deciding features of hair. Cut hairs from above descend into cleft

Fig. 25.36: Pilonidal sinus-primary and secondary sinuses are clearly seen.

Tuft of hairs may be seen in the opening of the sinus. Presentation may be as an acute exacerbation (abscess), or as a chronic one. It causes recurrent infection , abscess formation which bursts open forming recu rrent sinus with pain, discharge and discomfort.

Complications ,. Chronic pilonidal sinus can cause occasionally sacral osteomyelitis, necrotising fasciitis and rarely meningitis. It is not a life-threatening condition but often it can be a morbid disease because of high recurrence rate.

Patience is the companion of the wisdom.

959

n > "'C

::c m

:D N

U'I

:a

CD

n

c 3

DJ ::I

Q.

>

::I DJ (') DJ ::I DJ

960

I Treatment In acute phase initially-drainage of the abscess and antibiotics; later definitive treatment is undertaken.

Excision with Z plasty-good result. Excision with multiple Z plasty.

...cu

en

::::,

Cl)

0

Defect

-

'Z' plasty

Fig. 25.38: 'Z' plasty is done for pilonidal sinus. It gives good result.

Multiple Z plasties are also used.

Figs. 25.37A and B: Pilonidal sinus-operative jack knife position.

Definitive treatment: In prone position Uack knife position, i.e. prone with buttocks elevated) excision and primary closure is done under general anaesthesia or local anaesthesia. All sinus tracks, unhealthy granulation tissues with hairs are removed completely. Methylene blue is injected to demonstrate the multiple tracks properly. Excision and skin grafting-has got high recurrence rate.

Karydakis excision through a sem ilateral incision and lateralised suturing of the wound away from the midline gives good result. Excision with closure using Limberg (Rhomboid) buttock flap (single or double rhomboid flaps)-good resu lt. V-Y gluteal advancement flap. Bascom technique of excision through lateral approach is a good method. Through small lateral incision or multiple small lateral incisions 2-4 mm sized sinus is approached and pus is drained; hairs are removed with only minimal excision of sinus done. Cavity walls are not excised. Lateral small wounds are either sutured or left open for spontaneous healing. Lahey and Cattel/'s relaxing skin incisions on one buttock to relieve tension on main wound sutures with later closure of secondary wounds by sutures or advancement. Davies and Starr buttock skin flap rotation into the defect and secondary defect is closed at a later period. After excision of entire sinus completely, wound is left open to granulate and heal by epithelialisation with regular dressings. Buie's marsupialisation of the sinus track-after making incision on the sinus track, edge of the laid opened area is

Figs. 25.39A to G: Rhomboid-Limberg flap for pilonidal sinus. It gives good result.

sutured to the skin edge all round using silk or vicryl. This reduces healing time and promotes healing. Lord and Mi/Jar's limited excision of primary track for 0.5 cm depth with removal of tuft of hairs, debris and unhealthy granulation tissue using tiny brush and nylon bristle. Injection of phenol to the track destroys the epithelium after removal of the tufted hairs. Phenol is allowed to be in contact with epithelium for 3 minutes to create a blanch in the track orifice. Laser pilonidoplasty using diode laser-1470. Nonoperative treatment/prevention of recurrence after surgery: Regular shaving of natal cleft to have meticulous hair control. Laser, depilatory cream, electrolysis are other methods used. Proper perinea! hygiene.

Anal canal

961

External piles

Figs. 25.40A and B: Anatomical locations of internal and external piles.

::,::,

-

C1I

n C

3

c»

::I Cl.

>

::I

c»

B

("')

Entry of new tuft of hairs Overlooking of existing diverticulum Breakage of scar Note: Condition has got high recurrence rate (20%)

c» c»

::I

Fig. 25.41 : Parts of piles-plum coloured internal part and black cutaneous external component.

I Classification I

PILES/HAEMORRHOIDS ff bile or phlegm be determined to the veins in the rectum, it heats the blood in the veins: and these veins becoming heated attract blood from the nearest veins, and being gorged the inside of the gut swells outwardly, and the heads of the veins are raised up, and being at the same time bruised by the faeces passing out, and injured by the blood collected in them, they squirt blood, most frequently along with the faeces, but sometimes without faeces.

Primary haemorrhoids-. Located at 3, 7, 11 o'clock positions, related to the branches of the superior haemorrhoidal vessel which divides on the right side into two; left side it continues as one. Secondary haemorrhoids: One which occurs between the primary sites.

1

---Hippocrates (460-375 BC)

Piles = a ball or mass, Haemorrhoids = blood to ooze, Figs = a fruit (Anjoora). The word 'Haemorrhoids' is derived from Greek word Haima (bleed) + Rhoos (flowering), means bleeding. The pile is derived from the Latin word 'Pila' means Ball. It is downward sliding of anal cushions abnormally due to straining or other causes. Anal cushions (Thomson, 1975) are aggregation of blood vessels (arterioles, venu les), smooth muscles and elastic connectivetissue in the submucosa that normally reside in left lateral, right posterolateral and right anterolateral anal canal. Piles can be mucosa! or vascular (Graham Stewart, 1963). Vascular type is seen in young; mucosa! is seen in old. Present concept is weakening of Park's ligament which is the lower end of the external sphincter.

I Types Internal-above the dentate line, covered with mucous membrane. External-below the dentate line, covered with skin. lnterno-externa/---together occurs.

Right anterior

11

7

I r.J Right posterior

Left lateral :

Figs. 25.42A and B: Positions of haemorrhoids.

B First degree haemorrhoids Piles within that may bleed but does not come out Second degree haemorrhoids Piles that prolapse during defaecation, but returns back spontaneously Third degree haemorrhoids Piles prolapsed during defaecation, can be replaced back only by manual help Fourth degree haemorrhoids Piles that are permanently prolapsed

----

Piles begin as pedicle and it is located at the origin of the internal pile, i.e. at the level of anorectum.

We make a living by what we get; we make a life by what we give.

962

0

«I ::I C «I

:!:

Cl)

m cc

"'

1°

11°

Ill°+ IV

0

Fig. 25.43: Degrees of haemorrhoids.

I Aetiology Hereditary. Morphological-weight of the blood column without valves causes high pressure. Veins in the lower rectum are in loose submucosal plane, but the veins above enter the muscular layer, which on contraction increases the venous congestion below (more prevalent in patients with constipation). Superior rectal veins have no valves (as they are tributaries of portal vein) and so more congestion. Other causes are straining, diarrhoea, constipation, hard stool, low fibre diet, ove rpurgation, carcinoma rectum, pregnancy, portal hypertension (rare cause). During pregnancy factors causing haemorrhoids-raised progesterone relaxes the venous wall and reduces its tone, enlarged uterus compresses the pelvic vein, and constipation is common problem.

of arterial pressure; pressure in rectal ampullary pump (Wannas) during straining raises the portal as well as systemic pressure causing obstruction to venous outflow causing haemorrhoids. Disruption of suspensory tissues which hold plexus in position (sliding lining theory'/; raised basal anal pressure; unsupported superior haemorrhoidal vein in the loose submucosal connective tissue in the anorectum when passes through the muscular coat gets constricting effect leading into congestion of haemorrhoidal plexus-are the other theories of haemorrhoid formation. Idiopathic cause: It is very difficult to pinpoint the cause for production of piles.

Figs. 25.45A and B: (A) Thrombosed prolapsed pile. Note the colour

of the thrombosed pile. It is very painful and tender; (8) Proctoscopic view of the internal pile (grooved proctoscope). Note: • An arterial pile is haemangiomatous condition of superior rectal artery entering the pedicle of internal haemorrhoid which will bleed profusely.

I Features

Figs. 25.44A to D: Different types of prolapsed piles.

Bulging of haemorrhoidal plexus (anal corpus cavernosum, by Stelzner) occurs due to raised luminal pressure and transmission

The prevalence rate of piles is 4.4% in the world, in about 10 million people. It may occur at any age but mostly seen in the age between 30 to 65 years. Incidence is equal in both the sexes. Bleeding-1st symptom-'Splash in the pan'- 'bright red and fresh'-occurs during defecation. Mass per anum; Anaemia- secondary. Discharge-a mucoid discharge; Pruritus. Pain-may be due to prolapse, infection or spasm. On inspection, prolapsed piles will be visualized. On P/R examination, only thrombosed piles can be felt. Through proctoscopy, exact position can be made out as a bulge into the proctoscope. Points to be noted during proctoscopy: ,. The numbers, degrees and size. , The surface and appearance of piles. ,. Features, chronicity of the prolapse. , One should look for other rectal lesion such as external tags, anal papillae and fissure, proctitis.

,.. Any gynaecological, genitourinary or abdominal conditions like-carcinoma of rectum, polyps, tumours, features of ulcerative colitis should be identified. ,, Presence of other discharge like blood, pus, mucous. Sigmoidoscopy or colonoscopy or barium enema should be done if there is any suspicion of associated malignancy. Differential diagnosis: Carcinoma; Rectal prolapse; Perianal warts. Investigations: Haematocrit; Colonoscopy to evaluate proximally for any cause; Barium enema X-ray.

Pylephlebitis (Portal pyaemia) is rare, but can occur in 3rd degree piles after surgery.

963

I Treatment Preventive

Therapeutic/curative

Diet-more Medical -local applications; sitz bath, diet, laxatives, fibre/liquid drugs-analgesics Parasurgical-Sclerotherapy; Banding; Cryotherapy; Laxatives Infrared coagulation (IRC); Laser therapy; Dopplerguided haemorrhoidal artery ligation (DGHAL) Surgical

• • • •

Open haemorrhoidectomy Closed haemorrhoidectomy Stapled haemorrhoidopexy Anal stretching-Recamier, Lord's

:J:J

C1I

n C

3

Q)

::I

a.

>

::I

Figs. 25.46A to B: Different types of piles and parts.

T

Causes for bleeding per anum

Piles Fissure-in-ano Polyps Ulcerative colitis Amoebic colitis Fistula-in-ano

Carcinoma rectum Carcinoma colon Diverticulitis lntussusception Vascular anomaly of the colorectum Mesenteric ischaemia

I Complications Profuse haemorrhage which may require blood transfusion. Strangulation- piles is being gripped by anal sphincter.

1. Nonoperative: Sitz bath-means the patient has to sit in warm water with the anal region dipped in water for 20 minutes, 2-3 times a day. This reduces the oedema, pain and promotes healing. Local applications to reduce pain, itching and oedema can be used. Antibiotics, laxatives, anti-inflammatory drugs are beneficial. Fibre diet 35 g/day, plenty of water. Fibre alternatives (bulkforming agents, e.g. ispaghula husk, sterculia, methylcellulose) can be used to supplement a high-fibre diet; squatting position may reduce the incidence of piles; plenty of liquid intake. Laxatives such as lactu lose solution which soften bowel motions and relieve the constipation. Bulk laxatives such as psyllium mucilloid, Konsyl or polycarbophil may be necessary. 2. In case of inflamed, permanently prolapsed, oedematous piles, initially, manual stretching of the anal canal sphincter is tried. This prevents congestion of anal cushions and relaxes the anal sphincter, as a result of which the prolapsed piles gets reduced-Lord's dilatation (8 fingers). Once oedema subsides, in 1-2 weeks, formal procedure is done. • •

COMPLICATIONS OF ANAL DILATATION (3-4 FINGERS DILATATION)

• Incontinence-rectalusually temporary Infection

Figs. 25.47A and B: Prolapsed, strangulated piles.

Thrombosis-piles appear dark purple/black, feels solid and tender. Ulceration; Gang rene; Fibrosis; Stenosis. Suppuration, leads to perianal or submucosal abscess.

3. lnjection-Sclerosant therapy. It is done in 1st degree and early 2nd degree piles (internal)outpatient procedure. Using proctoscope and Gabriel syringe, 3-5 ml of 5% phenol in almond oil is injected into the submucosal plane just above the anorectal ring to the pedicle. All three piles can be injected separately-3- 5 ml to each site in single sitting. Technique can be repeated after 6 weeks. This technique is not done in the presence of sepsis or prolapse. The drug causes fibrosis in the submucosal region (sclerosis leading to mucosal fixation on to deeper planes and occlusion

Trust your hopes, not your fear.

Q)

C") Q)

::I Q)

0

«I

::II

C «I

:ii: _v,

CICI

a:

en

of lakes) and thereby fixation of the anal cushions which do not prolapse, causes strengthening of the vessel wall and obliteration of the vessel lumen. ,:. It is quick and painless; gives 95% cure rate in 1st degree piles; done on OP basis. Contraindications are---thrombosed/prolapsed piles, presence of proctitis/fissure/fistula-in-ano, pregnancy and diabetes mellitus. Sclerotherapy has not gained popularity, oily solution is difficult to handle and inject. Complications-recurrence (15%), hypochondriac pain due to entry of drug into the portal system , tenesmus, mucosa! sloughing/ulceration, submucosal abscess, anal canal pain, anal stricture. Inadvertent deep injection can precipitate pelvic abscess, prostatitis, impotence, rectovaginal fistula.

Fig. 25.48: Gabriel syringe-It is used for injection sclerotherapy fo_r internal piles using 5% phenol in almond/olive oil (~lmond/v~getabl~ 011 acts as a vehicle which holds phenol for long period of action). It_Is a stainless steel syringe with two metallic finger brims near t~e proximal end. One more metallic finger brim is present on the proximal end of the piston to place thumb while injecting.

4. Barron's banding: It is done for 2nd degree piles. It causes ischaemic necrosis and piles fall off. ,:. At one time only two piles can be banded . Repeat banding can be done only after 3 weeks. ,:. Band should be placed 2 cm above the dentate line. ,:. Usually 2 bands are used for pile mass to take care of breakage. Tissue sloughs off in 1-2 weeks leaving an ulcer which heals by scarring. Equipment is inexpensive, simple to perform; done without anaesthesia on OP basis; results are consistent, stops bleeding and tackles the prolapsing anal cushion.

Note: Suction banding is used presently. Suction is used to suck the internal pile into the banding gun.

5. Cryosurgery: ,:. Using nitrous oxide (-98°) or liquid nitrogen (-196°), extreme cold temperature is used to coagulate and cause necrosis of piles which gets separated and falls off subsequently. It is relatively painless and can be done on OP basis. All masses can be tackled at one sitting. This is carried out with the help of a cryoprobe. Nitrous oxide is preferred since it produces adequate freezing. Nitrous oxide delivery system/cryoprobe does not require special rewarming circuit. Liquid nitrogen produces quick destruction and damage of sphincter muscle; also needs special rewarming circuit for its release and is costlier.

Fig. 25.50: Cryosurgery instrument set up for cryohaemo rrh oidectomy.

The patient is put in lithotomy position; cryoprobe is applied in the longitudinal axis of internal pile above the dentate line; the pressure must be maintained above 700 lb continuously; the rapid adhesion with freezing (white area formation on piles) occurs; traction and slight rotation in both directions is done to draw entire pile mass to come in contact with the probe; when entire tissue is frozen in 20-30 seconds, the probe is detached after warming (defrosting/thawing). Procedure is repeated on other pile masses.

CRYO SURGICAL SYSTEM

u-. N,_

Ozide (N,0)

Fig. 25.49: Barron's band instrument for 1st and early 2nd degree piles.

It is contraindicated in fissu re/fistula/proctitis. Complications: If applied low into skin it causes severe pain; discomfort; secondary haemorrhage, ulceration.

Fig. 25.51: Cryoinstrument for cryosurgery.

965

C"')

::c

>

m

:x, N

c.n : \.

:::c

Figs. 25.52A and B: Cryosurgery for piles. Note the probe, grooved

CD

n

proctoscope, freezing the internal pile mass. Advantages: It is reasonably painless, simple, safe, can be done on OP basis, with less bleeding. Disadvantages: It causes profuse watery discharge, itching, pain if skin is frozen; marked oedema of adjacent skin, incontinence occasionally. 6. Infrared coagulation: Here heat is used to burn the piles so as to allow it to fall off. Pulses of infrared radiation are applied through a handheld applicator. The specific infrared wavelengths produce chemical changes that cause blood coagulation within the haemorrhoid itself, which causes the haemorrhoid to seal, shrivel, shrink or slough off. Source of infrared rays of 14 volt Wolfram halogen lamp with a gold plated reflector rays are transmitted from libero-optic cable which terminates in a probe or pistol for coagulation. In left lateral position, the probe is applied at the base of pedicle above the dentate line and bursts are given in clover leaf fashion. Timer is set at 2 seconds giving a depth of 2 mm; total time taken 2-5 minutes. It is done in 1st, 2nd , and 3rd degree piles. It produces a discrete area of necrosis (coagulates tissue proteins and evaporates water from the cells) which heals to form a scar; reduces or eliminates blood flow through the haemorrhoid thereby shrinking it and mucosa becomes fixed to the underlying tissue. Often 3 or 4 sittings are needed at 1 month intervals. It does not cause noncontact coagulation; does not cause interference with electro magnetic devices such as pacemakers. It is contraindicated in external pile, proctitis. Longterm results are not good. Equipment is expensive; multiple sessions are needed. 7. Laser therapy for piles-{or 3rd degree piles. Nd-YAG, diode and carbon dioxide lasers can be used but are expensive and tedious. The intense beam of light interacts with tissue and can be used to cut, coagulate or ablate the tissue, sealing off nerves and tiny blood vessels can be done by laser beam. By sealing superficial nerve endings patients have minimum postoperative discomfort. Laser is used for dissecting and excising pile masses. It is done for internal haemorrhoids.

C:

3

Cl)

:::, C.

>

:::, Cl)

(") Cl)

:::,

Cl)

Figs. 25.53A to C: Laser surgery for piles-CO2 laser and

diode laser.

Advantages-less operative time; less intraoperative and postoperative bleed and pain; rapid healing; quick recovery; done under LNSA; less complications; minimal pain, constipation and urinary retention. Disadvantages--needs skill; sphincter shou ld be taken care of; non-contact burning can occur; secondary haemorrhage can occur due to heat tissue destruction; and also injury to sphincter can occur.

To different minds, the same world is a hell, and a heaven.

8. Doppler guided haemorrhoidal artery ligation (DGHAL) DGHAL is an advanced instrument that works under Doppler guided ultrasound. It is painless, 20-minute procedure that cures all degrees of haemorrhoids. It causes choking and blocking of the blood supply of piles. It is done using proctoscope with an incorporated Doppler probe. This proctoscope is inserted and used to locate the haemorrhoidal arteries by an audible signal. Once located, a needle holder is inserted into the lumen of the proctoscope and the artery ligated with a 'figure of eight' absorbable suture into the submucosa. The procedure is repeated until no more Doppler signals are identified. Advantages: Anaesthesia is not needed; blood loss, pain, residual problems are minimal; done as day care surgery; early return for work; may be safe in diabetic, cardiac, old age patients, and in pregnancy. It is under trial and too early to confirm the efficacy. 9. Stapled haemorrhoidopexy (Antonio Longo) It is circumferential excision of the mucosa and sub-mucosa 4 cm above the dentate line using ci rcular haemorrhoidal stapler passed per anally (Ml PH-minimally invasive procedure for haemorrhoids). Advantages are-it is less painful; less blood loss; faster recovery; short hospital stay and equally efficacious. It is done only for prolapsed piles.

,- This procedure avoids wou nd in the sensitive perianal skin thereby reducing the postoperative pain. Using stapling gun, a unique circular stapler which reduces the degree of prolapsed piles by excising a circumferential strip of mucosa from the proximal anal canal. The strip of mucosa and submucosa is excised circumferentially above the dentate line. The veins leading to the haemorrhoids are thus incorporated in this excision. Activation of the gun also simultaneously recovers the cut mucosa and sub mucosa by stapling the edges together. ;... Disadvantages-need for experience in advanced surgical skill; costlier; may cause a full-thickness excision of the rectal wall; may injure theanal sphincter. Improper purse string can cause incomplete doughnut leading to severe haemorrhage. ,. Contraindication: Associated anorectal disease like fi ssure, fistula-in-ano. Note:

• Doughnut should be sent for histology for muscular layer; proper doughnut should not contain muscular layer. 10. Open operative methods: Still gold standard.

B ~

3rd degree piles lure of nonoperative methods

Haemorrhoidectomy is the best treatment for haemorrhoids. The haemorrhoidectomy is performed using an open or closed technique. The open technique is commonly used in UK and is known as Milligan-Morgan operation. The closed technique (Hill Ferguson) is more popular in USA. Both involve ligation and excision of the haemorrhoid, but in the open technique the anal mucosa and skin are left open to heal by second intention, and in the close technique the wound is sutured . Efficacy of surgery is 95% with 2- 5% recurrence rate.

Figs. 25.54Aan d B: Stapler haemorrhoidopexy. It is minimally invasive procedurefor haemorrhoids. It is high mucosal rim excision including vascular pedicles using circular (single use) staplers. It is done for prolapsed piles.

Methods are: Ligation and excision of pi/es-commonly done procedures (Milligan-Morgan) (Open method). ,- Under anaesthesia, in lithotomy position, initially the sphincter should be dilated to reduce the postoperative pain. Later skin is held with Allis forceps, internal pile is held with artery forceps. Skin is cut in "V' shaped manner and internal sphincter is separated and pushed up. Pedicle is transfixed with vicryl or catgut and distal part is excised. All the three piles can be dealt in a single sitting. , Postoperatively, sitz bath, antibiotics, laxatives, analgesics, local applications are given. Often few finger dilatation of the anal canal is required to prevent stenosis. Submucosal haemorrhoidectomy of 'Parks!...-approach is above the skin through submucosal plane. Hill-Ferguson closed method. Here patient in prone position, under GA/caudal anaesthesia, retraction is done using

Hill-Ferguson retractor. Incision is made around pile mass, pedicle is dissected to its proximal base; it is ligated with transfixation using 2-zero vicryl or silk; mucosa and anal skin is sutured using 3-zero vicryl/dexon after proper haemostasis using cautery.

I

967

Fig. 25.56: Typical external pile. It causes

haematoma, abscess, pain, itching.

ANAL FISSURE (FISSURE-IN-ANO) Fig. 25.55: During haemorrhoidectomy, skin part is held with Allis

forceps; internal pedicle is held with artery forceps. A 'V' cut is placed over the outer skin up to mucocutaneous junction. Dissection is deepened to visualise the internal sphincter. Once pedicle is dissected, it is transfixed using vicryl suture material. Distal tissue is excised. Technique is repeated on other sites also.

B Pain-due to spasm, nerve irritation, muscle injury Retention of urine-commonest-50% Reactionary or secondary haemorrhage Anal stricture; Anal fissure; Recurrence Anal discharge for sometime Incontinence for faeces or gas Ectropion (Whitehead deformity) 11. Management of strangu/ated/thrombosed/gangrenous piles-. Here initially conservative treatment is done using warm water sitz bath; antibiotics; elevation; bed rest; saline compression dressing; analgesics. This reduces the oedema and piles shrink. Later in 4-5 days haemorrhoidectomy is done. Doing haemorrhoidectomy immediately may precipitate portal pyaemia and also increases risk of developing anal stricture. 12. Newer methods-. Using ultrasound or controlled electric energy (Harmonic scalpel or ligasure), haemorrhoidectomy can be done with less postoperative pain. But tissue charring may precipitate secondary haemorrhage.

I EXTERNAL PILES Causes: As a part of internal piles, Sentinel pile associated with anal fissure, Anal ski n tags. Treatment: The cause is treated, Sitz bath, Excision. Problems: Pruritus ani, Perianal haematoma , Perianal abscess formation.

It is an ulcer in the longitudinal axis of the lower anal canal. Commonly it occurs in the midline, posteriorly (more common in males), but can also occur in the midline anteriorly (more common in females). Ninety-five per cent of anal fissures in men are posterior; 5% are anterior. Eighty per cent of anal fissures in females are posterior; 20% are anterior. Anterior anal fissure is common in females. It is superficial, small but distressing lesion. Fissure ends above at the dentate line.

Fig. 25 .57: Chronic fissure-in-ano with ulcer and sentinel (pile) skin tag.

Internal Sphincter The internal sphincter is formed from a thickening of the smooth muscle of circu lar coat of upper end of anal canal. Circular muscle is the continuation of the inner coat of the rectum. This involuntary muscle commences where the rectum passes through the pelvic diaphragm and ends just within the anal orifice, where its lower border can be felt. The internal anal sphincter is 2.5 cm long and 2 to 4 mm thick. The internal sphincter is closed by a sheath of striped muscle.

We do not see things as they are, we see them as we are.

968

Spasm and contracture of this muscle play a major part in fissure and several other anal infections.

External Sphincter

... Q)

en

::I Cl)

0

::I C

:E

·"' cc a: Cl)

It can be divided into three parts-deep, superficial and subcutaneous portion. It is considered to be one muscle. Deep part encircles the upper end of anal canal and has no bony attachment. Superficial part is attached posteriorly to the coccyx, anteriorly inserted into the mid-perinea! point in the male, in female it fuses with the sphincter vagina. Subcutaneous part encircles the lower end of the anal canal and has no bony attachment.

I Causes Because of the curvature of the sacrum and rectum, hard faecal matter while passing down causes a tear in the anal valve leading to posterior anal fissure. Anterior anal fissure is common in females due to lack of support to pelvic floor. Hard stool ; diarrh oea; increased sphincter tone; local ischaemia; trauma; sexually transmitted diseases. Other causes- haemorrhoidectomy, Crohn's disease, venereal disease, ulcerative colitis, tuberculosis.

I Types Anal fissure can be acute or chronic.

Acute Anal Fissure It is a deep tear in the lower anal skin with severe sphincter spasm without oedema or inflammation. It presents with severe pain and constipation.

Chronic Anal Fissure It has got inflamed, indurated margin with scar tissue. Ulcer at its inferior margin is having a skin tag which is oedematous, acts like a guard- sentinel pile. Proximally hypertrophied anal papilla is observed. It can cause repeated infection-fibrosis- abscess formation-fistula formation. Chronic fissure is less painful than acute one. Multiple fissu res are seen in inflammatory bowel disease, homosexuals and venereal diseases. Chronic fissure can cause complications like-abscess, fistula formation.

Sentinel pile

Fissure-in-ano

Fig . 25.58: Parts of chronic fissure-in-ano. Ulcer in the lower anal

canal; sentinel pile below and hypertrophied papilla above.

Fig. 25.59: Lateral fissure-in-ano.

I Features Common in middle aged women, not in elderly. Pain is severe in nature in acute type, whereas less severe in chronic. Constipation, bleeding and discharge. P/R examination and proctoscopy is not possible in acute fissure-in-ano. General anaesthesia is required for examination. In chronic fissure, ulcer is felt with button like depression, induration and often sentinel pile. Differential diagnosis: Carcinoma anal canal; Inflammatory bowel disease; Venereal diseases; Anal chancre (painful); Tuberculous ulcer; Proctalgia fugax.

.ILT_r_e_at_m _e_n _t______________

B Adequate fluid intake (6-8 glasses of liquids) Fibre rich diet (vegetables, fruits, brown rice) Bulk forming agents (psyllium husk, bran) Stool softeners (lactulose) Local anaesthetic agents (lignocaine 5%) Sitz bath Avoid constipation ~ ce recovers, regular anal dilatation In an acute case Stretching of the anal sphincter (Recamier, 1829) using two fingers of each hand (4 fingers) under anaesthesia is also an alternative one. It is better than Lord's dilatation as complications are less. Lord's dilatation is done under G/A to relax the sphincter. It is the manual dilatation (Lord, 1969) of the anus under general anaesthesia with relaxation using four fingers of each hand (8 fingers) to cause vigorous stretching of the anal canal to break the circular constricting band in the wall of the anorectum. Later, use of laxatives, xylocaine surface anaesthetic application, and anal dilatation with finger can be carried out for certain period . Bed rest; 2% nifedepine ointment. For chronic fissure Dorsal fissurectomy with sphincterotomy is done under anaesthesia. Specimen should be sent for biopsy to rule

out carcinoma, tuberculosis, etc. Here transverse fibres of internal sphincter is divided in the floor of the fissure. Lateral anal sphincterotomy ;... Here internal sphincter is divided partially away from the fissure either in right or left lateral positions (also gives a good result). , Here closed or open methods (Notaras) are used. Sphincterotomy is done below the dentate line. In closed method no. 11 blade is inserted into the intersphincteric groove to pass upwards. Blade is moved medially to cut lower 1/3 or 1/2 of the internal sphincter. In open method skin is incised laterally, external to anal verge. Hypertrophied band of lower part of internal sphincter is dissected and divided. Wound is left open. ,. Haematoma, perianal abscess, bruising, fistula, incontinence are the complications of lateral sphincterotomy. Topical nitroglycerine 0.2% is also used to relax the sphincter. It causes severe headache. Botulinum toxin 25 units injected into the internal sphincter. It causes temporary denervation of the internal sphincter; reducing its tone, improving the blood supply and control of ischaemia. It causes temporary incontinence for flatus (10%). Diltiazem (2%), L arginine are the other agents used. Regular anal dilatation is also often important to prevent recurrence. Anal advancement flap is more effective especially in females.

I SENTINEL PILE ('sentinel' means guard}

969

It is commonly associated with fissure-in-ano of chron ic type wherein, in the lower part of fissure, skin enlarges and appears like guarding the fissure.

m

N U1

:ll CD n

c 3

I»

::::, C.

Fig. 25.62: Perianal haematoma-it should be drained to remove clot. Small haematoma may resolve on its own. Larger one if not drained will form an abscess.

It can cause perianal haematoma, abscess formation, discomfort. The chronic fissure is treated along with excision of the sentinel pile. There may be low grade infection and lymphatic oedema. Haematoma/abscess can develop in it.

ANORECTAL ABSCESS Most common causative organism is E. coli (60%). Others are Staphylococcus, Bacteroides, Streptococcus, 8. proteus. Commonly occurs due to infection of anal gland in perianal region. 95% of anorectal abscesses are due to infection of anal glands in relation to crypts-cryptoglandulardisease. Common in diabetics and immunosuppressed.

l

Fig. 25.60: Lithotomy position used for all perinea! surgeries like for fissure, piles, fistu la, and APR. lschiorectal abscess

Perianal abscess

Fig. 25.63: Anatomy of anorectal abscess and sites

'

·-----·-··

,;~'

,'..,

: Fig. 25.61 : Sitz bath is used after perinea! surgeries like for piles, fissure, and fistula. Patient sits in warm water bath with perineum dipped in the water. Often small quantity of antiseptics or KMn04 is added. \.

-

>

::::, I»

n

I»

::::,

Sobm=~I , ,,~~

,' \

::c

>

::0

Pelvirectal abscess

'

(")

Other causes: ;.. Injury to anorectum. , Cutaneous infection (e.g. Boil). , Blood born infections. ,. Many anorectal abscesses are associated with anal fistulas. - Fissure-in-ano; Perianal haematoma. - Post-anorectal surgery; Crohn's disease; Tuberculosis.

Life is ten percent, to make it and ninety percent, how you take it.

I»

970

n:I

::i C:

n:I

:ii: _v,

cc

cc en

Differential diagnosis of anorectal abscess: Periurethral abscess; Bartholin abscess; Tuberculous abscess. Investigations ,. MRI is the investigation of choice for anorectal abscess. ,., Perinea/ and anal US is also very useful. :;... Investigations relevant to specific cause may be done. Proctosigmoidoscopy is needed to identify secondary cause in anorectum.

B Perianal lschiorectal Sub mucous

• Pelvirectal Fissure abscess (in relation to fissure-in-ano).

I lschiorectal Abscess (30%) Surgical Anatomy (Refer Fig. 25.1) lschiorectal fossa (pyramidal shape 5 cm depth and 2 cm width) lies between anal skin and levator an i. Right and left communicates with each other. Laterally, it is related to fascia covering obturator internus; medially to levator ani and external sphincter; posteriorly sacrotubercous ligament and gluteus maximus; anteriorly urogenital diaphragm; below, the floor by skin. Above it is related to lunate fascia and pudenda! neurovascular bundle in pudenda! canal (A/cock's cana~.

I Perianal Abscess (60%) This usually results due to suppuration of anal gland or su ppuration of thrombosed external pile or any infected perianal condition. It lies in the region of subcutaneous portion of external sphincter.

Fig. 25.66: lschiorectal abscess-left side.

Causes

Fig. 25.64: Perianal abscess. Note the swelling and inflammation.

Features Severe pain in perianal region with difficulty to sit. Tender, smooth, soft swelling in the region. Treatment ,. Sitz bath, antibiotics, analgesics, local application of anaesthetic agents and laxatives. ,. Drainage under G/A.

Commonly, it is due to extension of low intermuscular anal abscess, laterally through external sphincter. But often it can be blood or lymphatic born. Fat in the fossa is more prone for infection because it is least vascularised. Fossa communicates with that of opposite side through postsphincteric space and so horse-shoe like abscess can occur. It presents with tender, indurated, brawny swelling in the skin over the ischiorectal fossa with high fever. Swelling is not we/I-localised and fluctuation is absent in ischiorectal abscess.

Treatment

Cruciate incision

Fig. 25.65: Perianal abscess showing pus oozing out.

lschiorectal abscess

Fig. 25.67: Cruciate incision is used for drainage of ischiorectal abscess.

971

n

:c l>

m :c N U'I

Figs. 25.68A to C: lschiorectal abscess drainage done under general anaesthesia. Note the cruciate incision placed and pus gushing out. Under G/A in lithotomy position, through a cruciate incision a portion of skin is excised (de roofing) and pus is drained. Pus is sent for C/S and presence of any internal opening to rectum should be looked for (for possibility of an existing fistula).

I Submucous Abscess (5%) It occurs above the dentate line, which can be drained with sinus forceps, through a proctoscope. Aching pain in the anorectum with significant perinea! discomfort. On digital examination (P/R), tender, soft, smooth swelling in the lower rectum and anal canal. It may be missed clinically as there is no obvious swelling externally. Treatment is proper antibiotics; incision and drainage under general anaesthesia.

I Pelvirectal Abscess It is situated between the upper surface of levator ani and pelvic peritoneum. It is almost like a pelvic abscess, occurs secondary to appendicitis, salpingitis, diverticulitis, Crohn's. US abdomen is done to rule out the above factors. Treated accordingly, after thorough investigations for diabetes, Crohn's and other conditions. Problems with anorectal abscess: Recurrent abscess formation; Fistula formation.

FISTULA-IN-ANO It is a track lined by granulation tissue which connects perianal skin superficially to anal canal; anorectum or rectum deeply. It usually occurs in a pre-existing anorectal abscess which burst spontaneously. Fistula-in-ano can be: (1) Cryptoglandular- 90%; (2) Noncryptoglandular (other causes)-10%.

Cryptog/andular Hypothesis The intersphincteric space is the surgical plane between the internal and external sphincters and is found between the

longitudinal muscle and external sphincter, where it exists as a sheet of fat containing loose areolar tissue. The fat-filled ischioanal fossa lies lateral to the sphincter complex and is traversed by a network of fibroelastic connective tissue. Proximal half of the anal canal is characterized by longitudinal mucosal folds, the anal columns of Morgagni. The distal aspect of each column is linked to its neighbour by a small semilu nar fold (the anal valves), which in turn forms small pockets (the anal sinuses, or crypts of Morgagn,). The distal undulating limit of these valves is the dentate (pectinate) line, which also marks the most distal aspect of the anal transitional zone, a histologic junction between anal squamous and rectal columnar epithelium. The dentate line lies 2 cm proximal to the anal verge and is important landmark in fistula-in-ano because the anal glands empty into the crypts that lie proximal to the valves. These glands secrete mucus to lubricate anus, and are the source of infection. These glands present in the subepithelium, internal sphincter, and twothirds of these glands are located within the intersphincteric space. It is the infection of these intersphincteric glands that initiates the fistula-in-ano, known as the "cryptoglandular hypothesis". These glandular infection leads into an intersphincteric abscess due to blockage of the draining duct by infected debris. This abscess may resolve by spontaneous drainage into the anal canal or may progress to an acute anorectal abscess. Treatment of this abscess is incision and drainage; but source of infection in the intersphincteric space persists, leading into development of a fistula-i n-ano. Acute anorectal abscess and fistula in ano are believed to be acute and chronic manifestations, respectively of the same disease. While most fistulas start as a simple single primary tract, recurrent infection eventually causes formation of extensions (secondary tracts). Extensions may be intersphincteric, ischioanal, or supralevator (pararectal). The ischioanal fossa is the commonest site for an extension. Extensions also occur in the horizontal plane known as "horseshoe" if there is ramification on each side of the internal opening.

One cannot love what he cannot respect, whether it be himself or another.

:::c

Cl)

n

c 3

t:11

= a. >

= t:11 C")

t:11

= t:11

972

Longitudinal muscle

i!" Cl)

...

Cl

t:5

Levator ani

0 Puborectalis Internal ana I~ J..--1-1~ sphincter

- I

External anal sphincter Dentate line

Fig. 25.69: Anatomy of anal glands (Cryptoglands of Morgagni).

1. Subcutaneous fistula 2. Submucosal fistula 3. Low-anal fistula

4 . High-anal fistula 5 . Pelvirectal fistula

Fig. 25.71: Classification of fistula-in-ano (Standard classification).

OTHER CAUSES ARE (NON-CRYPTOGLANDULAR)

Tuberculosis Carcinoma Crohn's disease Ulcerative colitis

Lymphogranuloma venereum Hydradenitis suppurativa Traumatic

Plenty of lymphoid aggregates surround the anal glands, which explain the high incidence of anal fistula in Crohn's disease. lntersphincteric fistula

Supralevator fistula

Fig . 25.70: Anatomy of fistula·in-ano.

Extrasphincteric fis tula

It can be:

Standard (Milligan Morgan, 1934; Go/igher 1975)

Park's classification (1976)

• Subcutaneous commonest • Low anal-common

• lntersphincteric-commonest

• Submucous

@

Figs. 25 .72A to D: Park's classification of fist ula-in-ano:

(A) lntersphincteric fistula; (B) Transphincteric fistula; (C) Supralevator fistula; (D) Extrasphincteric fistula.

I Classifications

• High anal • Pelvi rectal

@ Transphincteric fistula

70%

• Transphincteric 25% • Supralevator/suprasphincteric 4%

• Extrasphincteric 1%

It can be: 1. Low level fistulas-these open into the anal canal below the internal ring. 2. High level fistulas-these open into the anal canal at or above the internal ring.

•·· Simple fistula without extensions. Complex fistula with extensions. It can be with:

••• Single external opening. Multiple external openings which are often seen in tuberculosis, ulcerative colitis, Crohn's disease, lymphogranuloma venereum (LGV), hidradenitis suppurativa, actinomycosis. The term complex fistula means- the track crosses >50% of the external sphincter, anterior in females, mu ltiple tracks, recurrent, or the patient has preexisting incontinence, local irradiation, or Crohn's disease.

973

B Fistulas with an external opening in relation to the anterior half of the anus is of direct type. Fistulas with external openings in relation to posterior half of the anus, has a curved track may be of horse-shoe type, opens in the midline posteriorly and may present with multiple external opening all connected to a single internal opening. Anterior

:::c

C1I

n

C

3

Q)

:::, Cl.

Fig. 25 .73: Fistula-in-ano both anterior and posterior (multiple).

>

:::, Q)

n Q)

:::, Q)

Posterior

Figs. 25 .76A and B: (A) Goodsall's rule. Anterior fistulas are having straight track. Posterior fistulas are having curved track with internal opening in the posterior midline; (B) Complex fistula-in-ano.

Fig. 25.74: Complex fistula-in-ano both sides of long duration.

I LOW-LEVEL FISTULAS I Clinical Features

It has a prevalence of 0.01% and is common in young adult males (2:1, male to female). It presents with seropurulent discharge (65%), along with skin irritation and one or more external opening may be present with in duration of the surrounding skin. Often it may heal superficially but pus may collect beneath forming an abscess which again discharges through same or new opening. lschiorectal fossa on each side, most often commu nicates with each other behind the anus causing horseshoe fistula.

Figs. 25.75Aand B: Fistula-in-ano (simple type)-typical look and site.

P/R examination shows indurated internal opening usually in the midline posteriorly. Most of the fistulas are on posterior half of anus. Probing in the ward and fistulogram in the ward before surgery using Lipiodol is not advisable as it may cause recrudescence of inflammation. It can be done with adequate precaution. Probing is done under general anaesthesia gently with care without creating extensions.

Fig. 25.77: Anterior fistula-in-ano with probe in place. Anterior low fistula has got straight track. Both internal and external openings are seen.

Success is never final and failure is never fatal.

974

i!' Q)

...=

en

Cl.)

0 ('1:1

= C ('1:1

:ii:

en

CCI

cc

Cl.)

Fig . 25.78 : Multiple fistulas-in-ano. It may be Crohn's disease; carcinoma or HIV. Biopsy should be done prior to formal therapy. MRI of perineum is of great help in such patients to anatomically evaluate the fistula-in-ano.

Tuberculous fistulas do not have induration, will have pale granulation tissue with watery discharge and they are most often multiple. Here, the infection occurs in lymphoid tissue over the lower part of anal canal, arou nd anal gland opening.

I Investigations Chest X-ray, ESR and barium enema X-ray. If required fistulogram is done only under anaesthesia. MRI/MRI fistulogram ideal. Endorectal ultrasound (US perineum) is useful to assess deeper plane. Discharge study, methylene blue dye study, biopsy. Colonoscopy often when ulcerative colitis/Crohn 's is suspected . Specific blood test.

Fig. 25.80: MR fistulogram.

• Urethral fistula in male Chronically infected Bartholin's gland Pilonidal sinus '---

- - - ---- - - - -

I Treatment Fistulectomy ;... Under G/A or spinal anaesthesia, probe is passed through external opening up to the internal opening which is felt as an induration. Fistula is opened along the probe using a knife. Fibrous track along with unhealthy granulation tissue and additional external openi ngs are excised. Specimen is always sent for histopathology. Principles In management • Identify the cause-cryptoglandular or other • Delineate exactly the fistula anatomyMRI/EUS Identity relation of fistula to anal sphincter • Drain all sites of infection • Eradicate track and secondary extensions • Preserve anal continence function The primary objectives are to eradicate the tract and drain all associated sites of infection while simultaneously preserving anal continence

Fig. 25.79: Fistulogram X-ray showing track.

• Hidradenitis suppurativa Carcinoma Crohn's tuberculosis, ulcerative colitis

Procedures • Laying open the fistulafistulotomy with cu retting • Fistulectomy • Gluing of fistula-not much useful • Mucosal flap procedure • Fistulectomy with primary repair • Fistulectomy with primary repair with episiorectopexy • Fistulectomy with secondary repair • Anal fistula plug repair (AFP) • LIFT technique (Ligation of intersphincteric fistula track) • Seton technique (Latin-seta-a bristle) • Colostomy-lay open, fistulectomy, later closure of colostomy-for high type • VAAFT procedure (Video assisted anal fistula track ligation)

I HIGH-LEVEL FISTULAS

Fig. 25.81 : Methylene blue injection to delineate track on

table in fistula-in-ano.

975

Its upper opening is at or above the anorectal ring. It is difficult to treat. Common causes are: Crohn's disease; Ulcerative colitis; Trauma; Carcinomas; Foreign body Incontinence may follow after lay opening of these fistulas. Investigations: Barium enema X-ray, colonoscopy, chest X-ray, biopsy. Treatment ,. Requires staged procedure- initial colostomy is done followed by definitive procedure. This prevents sepsis and promotes faster healing. , Later closure of colostomy is done.

C')

:::c

>

"'ti

-4

m

:J:J N

UI

:a

-

CD

n

C

3

c» ::I a.

>

::I

;.., Postoperatively-sitz bath, antibiotics, analgesics, laxatives are given. Fistulectomy for low level fistulas do not cause rectal incontinence. , Proper curetting of the infected anal gland area is essential. Fistulotomy It is done in low anal fistula. It is technically easier. After passing the probe through the entire fistulous track, incision is made over the probe to cut and lay open the fistulous track. It is allowed to granulate and heal from the floor/surface. Technique is safer, easier and can be done on outpatient basis. Advancement flaps are used occasionally to get better result-mucosa! flap procedure. Gluing of the fistula track is tried but success rate is not good. Fibrin glue is a mu lticomponent system containing mainly human plasma fibrinogen and thrombin. Once prepared it is injected into the fistula track which hardens in few minutes and fills the entire track. Success rate is 70%. Anal fistula plug (AFP) repair. Surgisis anal fistula plug (porcine small intestine submucosa, SIS) is used with 85% success rate in simple fistula. It contains naturally derived extracellular matrix which acts as scaffolding, ingrowth of tissue, remodeling. LIFT technique (Ligation of intersphincteric fistula track) : Under anaesthesia in lithotomy position, intersphincteric space is reached through a transverse incision. Fistula running across is identified and ligated using vicryl on either side. Part is excised; outer part is curetted through external opening. VAAFT procedure (Video assisted anal fistula track ligation): Fine specialized endoscope is passed through the outer opening into the fistula track; with continuous irrigation fistula track is cleaned and wall is cauterized. Inner opening is ligated through vicryl from luminal side. Fistula clip closur~closure of the internal fistula opening with a superelastic clip made of nitinol (over-the-scope clipOTSC)-by German surgeon Ruediger Prosst.

c»

Seton Technique A silk or linen ligature is passed across the fistula and left in place with a tie. Striated muscle superficial to fistula track is encircled with Seton material and tied securely and left in situ to create ischaemic necrosis, dividing the muscle slowly without allowing it to spring apart avoiding gutter deformity. Even though internal sphincter is divided in this, causing adequate laying open of entire fistula track, it will well-preserve the sphincter function and pressure. This allows the fistula to granulate and heal from above and to close completely. Usually takes longer duration to heal. It is done for intermediate and intersphincteric fistula. It is used prior to definitive procedures like fistulectomy or advancement flap. It is staged fistulotomy. Seton can be kept for 3 months. It can be regularly replaced by new silk or any material by rail road technique without anaesthesia.

Fistula-in-a no Seton material

Fig. 25.82: Seton technique tor intersphincteric fistula-in-ano. It is kept for 3 months. It can be tight or loose seton depending on the indications. It promotes formation of granulation tissue, healing by fibrosis and track recedes downwards.

Our senses don't deceive us; our judgement does.

976

Two types of setons are present.

Large wart may block the anal canal orifice. Whitening occurs on applying acetic acid on it. Biopsy confirms the diagnosis. Treatment is local application of 25% podophyllin cream; surgical excision of the wart; intralesional injection of interferon a 2b. Malignancy should be ruled out by histology.

1. Loose setons are used mainly to drain for long period in recu rrent/postoperative fistulas and due to specific causes like Crohn's. There is no tension in seton. 2. Cutting setons are used when enclosed muscle is needed to cut (cheese wiring through ice effect). It is placed tight.

ANORECTAL STRICTURES

Congenital Fibrotic anal fissure Irradiation Senility LGV (in females)

---

Note: • Buschke-Lowenstein disease (1925, giant condyloma acuminata) is a

Ulcerative colitis Crohn's disease Carcinomas Postoperative (surgery for coloanal anastomosi~

I Features Progressive constipation. On P/R examination, stricture can be felt as a tight ring. Featu res relevant of specific cause.

• •

• •

slow-growing, locally destructive verrucous plaque but seldom metastasizes that typically appears on the penis but may occur elsewhere in the anogenital region. It is commonly considered to be aregional variant of verrucous carcinoma. It is common in thepenis but can also occur in anal region, vaginaand scrotum. It is common in males. HPV 6, 11 and HIV infections may commonly coexist Biopsy and MRI confirms the diagnosis. Giant condyloma acuminatum is differentiated histologically from ordinary condyloma acuminata by its thicker stratum corneum and the presence of an endophytic downgrowth, along with a tendency to invade deeper. Treatment-surgical wide excision, topical podophyllin, 5 FU, interferon, cidofevir gel, bleomycin are different treatment modalities. HPV vaccine (Gardasil) may prevent the disease. A combination of radiofrequency surgical dissection and oral acitretin is used in perianal Buschke-Lowenstein tumor with good success. The use of acitretin may eliminate the residual disease that was not removed by surgery.

ANAL INTRAEPITHELIAL NEOPLASIA (AIN) It is dysplasia of anal or perianal epidermis. It is seen in individuals with HIV infection; HPV infection (16, 18); individual who do anorectal intercourse.

Classification: Figs. 25.83A and B: Anal dilator. Many proctology surgeries require

regular anal dilatation postoperatively. Note how to hold the dilator to pass into the anus after applying lubricant.

Investigations: Barium enema X-ray, biopsy, colonoscopy. Treatment , The cause is treated. , Dilatation of the anal canal under general anaesthesia. ,. Resection in severe recurrent cases. ,. Anal reconstructio1r-anoplasty is technically demanding with preservation of continence along with prevention of restenosis is important.

CONDYLOMA ACUMINATA It is most common sexually transmitted anal disease. It is common in homosexual men. Penile warts or female genital warts may be present. It is caused by human papilloma virus (HPV). Pruritus, discharge, pain and bleeding are the features.

AIN I -absence of keratocyte maturation and cellular atypia observed in outer 113rd of epithelium-low grade. AIN II-cellular atypia observed in middle 1/3rd-low grade squamous intraepithelial neoplasia. AIN Ill-cellular atypia full thickness-high grade squamous intraepithelial neoplasia. Thirty per cent of anal warts will show AIN. It is raised scaly white/pigmented/cracked lesion. Biopsy confirms the disease. Treatment: Excision; topical imiquimod, 80% tricholoroacetic acid and oral retinoids .

MALIGNANT TUMOURS OF ANAL AREA Anal malignant tumours are 2 cm but $5 cm. T3: Tumour >5 cm. T4: Tumour of any size invading adjacent organ(s), such as vagina, urethra or bladder. Nx: Regional nodes cannot be assessed. NO: No regional nodes. N1a: Metastasis in inguinal, mesorectal or internal iliac or external iliac nodes. N1 b: Metastasis in external iliac nodes. N1 c: Metastasis in external iliac nodes with any of N1 a nodes. Stage groups Stage 0: Tis, NO, MO. Stage I: T1 , NO, MO. Stage IIA: T2, NO, MO. Stage IIB: T3, NO, MO. Stage IIIA: T1-2, N1 , MO. Stage IIIB: T4, NO, MO. Stage IIIC: T3-4, N1 , MO. Stage IV: Any RT, Any N, M1 .

Squamous cell carcinoma of anal canal, usually present as a fungating or ulcerative growth, wh ich spreads to inguinal lymph nodes. ,. Biopsy and FNAC of lymph nodes are the essential investigations. ;.. Treatment: Wide excision of the lesion with 3 to 5 cm clearance and ilioinguinal block dissection for lymph nodes are done. Follow-up radiotherapy is also often given-as per Nigro regime.

I Nigro Regime

1. Squamous cell carcinoma is the commonest type. NIGRO REGIME FOR ANAL CARCINOMA Predisposing causes: Papilloma, irradiation, dermatitis, long (Norman Nigro, et al. 1974) standing fistula-in-ano. Initial radiotherapy for 3 weeks 3000 rads (30 Gy total) to perineum and pelvis 2. Basaloid carcinoma- it is rare, non-keratinising squamous Then chemotherapy-5 FU, for 4-5 days; is a radiosensitizer, cell carcinoma. Highly malignant. started on 1st day of RT as 1000 mg/m2 continuous infusion. 3. Muco-epidermoid carcinoma-arises near squamocolumnar I ~itomycin C is 15 mg/m2 as single dose on 1st day of RT junction. L_Later after 3 weeks abdominoperineal resection (APR) 4. Basal cell carcinoma. > Chemoradiation is becoming popu lar for carcinoma of 5. Melanoma-blue/black in colour mistaken for thrombosed anal canal. pile-poor prognosis (5 years-10%). Drugs used for chemotherapy are 5 FU, bleomycin , 6. Adenocarcinoma from the anal glands in a pre-existing fistulavincristine, adriamycin. in-ano. Two types of mankind are there; Hosts and Guests; on either way you are in trouble.

977

C"')

::c

>

"'C

m

:::z:, N U1

:c

CD

n C

3

I»

:::,

a.

>

::I I» (")

I»

:::,

I»

978

,... If persistent or recurring disease presents following combined modality therapy (CMT-chemoradiotherapt,, APR with colostomy is indicated. ,,.. Neoadjuvant chemotherapy using 5 FU, cisplatin and mitomycin Cis also commonly used. , In advanced growths radiotherapy is the only treatment. All other tumours-. Abdominoperineal resection with permanent colostomy is done.

m

PAGffS

Paget's disease of breast • Paget's disease of anal margin-of apocrine glands

Paget's disease of penis • Paget's test • Paget's disease of bone

SACROCOCCYGEAL TERATOMA

Fig. 25.86: X-ray of a patient with sacrococcygeal tumour (Courtesy: Professor Suresh Karnath, MS, Mangaluru).

It is an uncommon tumour, but most common of the large tumours in first 3 months of life. More common in females. Retention of large amount of primitive toti-potential cell in this region may be the reason for this tumour. It occurs between coccyx and rectum It is attached to coccyx, extends commonly downwards as a huge mass, occasionally upwards into the pelvis. It is a congenital condition arising from totipotent cells.

Fig. 25.87: Recurrent chordoma of sacrum.

ANAL INCONTINENCE

Figs. 25.85A and B: Sacrococcygeal teratoma, typical site (Courtesy: Professer Suresh Karnath, MS, Mangaluru).

Continence of anal canal is maintained by two factors: ;... Normal rectal and colonic pressure and activity. ,. Normal pelvic floor function. Types ,. Urge incontinenc~here rectal and colonic pressure and activity is increased but normal pelvic floor. ;.. True incontinenc~here rectal and colonic pressure and activity is normal but defective pelvic floor function. , Full incontinenc~here rectal and colonic pressure and activity is reduced and also defective pelvic floor function. ;.. Temporary-treated by reassurance. Often seen after Lord's dilatatiJn. , Permanent-needs definitive therapy. Causes

X-ray and CT scan are must. Treatment: Excision soon after birth. Complications Ulceration Infection Urinary obstruction Malignant changes

• • • •

Differential diagnosis • Sacral meningocele • Sacral chordoma • Postanal dermoid

L

Denervation-spinal injury, spina bifida Damage-childbirth, wounds, surgeries Descent- rectal prolapse, perinea! descent Debility-old age, diseases Destruction-RT, malignancy Dementia-senility, psychosis Deficiency-congenital anomalies

,, Irritable bowel syndrome, severe diarrhoea. Prolapsed piles, rectal prolapse. ;..- Old age, malnutrition, debilitating illness. ;.,. Congenital anomalies. Trauma, surgeries, injury during childbirth in females. ,. Spina bifida, spinal tumours, spinal injuries and surgeries. ;.. Malignancy, postirradiation. Psychological causes.

B For specific causes Anorectal manometry Per-rectal examination Sigmoidoscopy ---

Electromyography Defaecography Perineometer to assess level and angle of anorectal junction

1

Treatment ;..- Suturing of the torn sphincter. ,. Repair of puborectalis muscle and plication of external sphincter. ,. Encircling operations around anal canal to give support using gracilis sling or mersiline sutures. Electrical stimulation of the puborectalis. Secca therapy. Temperature controlled radiofrequency energy in the anal canal and distal rectum to create scarring and fibrosis of internal sphincter and adjacent tissues.

DESCENDING PERINEAL SYNDROME When a healthy person increases the intra-abdominal pressure and relaxes the pelvic floor muscles, there will not be any changes in the concavity of the perineum. In chronic ill-patients, malnourished, and people with preprolapse, perinea! descent can occur with obliteration of the normal concavity of the perineum. It is called as descending perinea/ syndrome. Levators got injured directly or indirectly causing weakening of pelvic floor. Anal canal is situated many centimeters below the pubococcygeal line. Usually 3-4 cm low during straining. Defecographyis ideal to evaluate such patients. Perineometer is also used. Presentations are tenesmus; incomplete evacuation; incontinence. Treatment is diet, laxatives, avoiding straining, and suppositories. Restoration of pelvic floor by various surgical methods may be needed often with rectal resection and suspension.

PROCTITIS It is inflammation of rectal mucosa often with the inflammation of colon and anal canal. Types ,. Acute or chronic. ,. Nonspecific-common. ,, Specific - Bacillary dysentery. - Amebic proctitis-common. - Combined amoebic and bacillary. - Gonococcal proctitis. - Lymphogranuloma inguinale (LGV). - Tuberculous proctitis. - Bilharzia! proctitis due to schistosoma haematobium. Enema induced proctitis especially of herbal enemas. ;.,. Ulcerative proctocolitis as part of ulcerative colitis.

979 n

:c

>

-a m

::::D N

UI

:J:J CD

n

c 3

Q)

::I CL

>

::I Q)

C") Q)

::I

Q)

CLINICAL FEATURES

Pain per rectum and anum; Tenesmus Passage of mucus and blood; Frequent urge to pass stool Fever, loss of appetite; Pain and tenderness in left lower abdomen P/R is tender

Investigations Sigmoidoscopy is more relevant than just proctoscopy. Stool study, stool culture; Mucosa! biopsy; Serological tests. ,. Relevant investigations like ESR, blood smear, and chest X-ray. Treatment :;.. Antibiotics, antiamoebic drugs like metronidazole. ,. In severe cases, retention enema using metronidazole, prednisolone, salazopyrin. ;.,. IV fluids, IV antibiotics and IV metronidazole are often required . ,. Treating the specific causes like tuberculosis, gonococcal infection and bilharzia! infection.

PROCTALGIA FUGAX It is sudden severe recurring pain in the rectum of unknown cause with segmental pubococcygeal spasm.

I Features It is common in young people may be due to stress, straining. Common at night, starts suddenly, lasts for few min utes and

Total pelvic marlex mesh repair; transcoccygeal posterior

then subsides spontaneously.

hitching of the rectum (Kraske); correction of cystocele, rectocele and enterocele. Results are not very good as recurrence or residual problems may persist.

Pain is unbearable and severe with often constipation. Gradually subsides on its own. Occasionally, only cutting of puborectalis muscle is required but with danger of developing incontinence.

You never get the second chance to make the first impression.

980

c=Q)

...=

en

en

0 «I

=

C: «I

:E _VI

cc

a:

en

HIDRADENITIS SUPPURATIVA OF ANAL REGION It is a chronic suppurativa condition of apocrine glands of the skin in axilla/perineum/mons pubis/ thighs/scrotum, etc. Apocrine gland duct obstruction bacterial infection multiple glands secondary infection (Staphylococcus aureus, streptococci) skin oedema, multiple raised multiple communicating fistulae formation. Disease does not extend above dentate line or into sphincter. It is common in young obese females. Sinus; scarred areas ; discharge; skin changes; pain and tenderness; foul smelling fluid are the presentations. Differential diagnosis are-Crohn's disease; fistula-in-ano; pilonidal sinus; tuberculosis; actinomycosis; LGV. Treatment ,, Weight reduction; proper hygiene. ,, Antibiotics; analgesics. ;... Incision and drainage of abscess. ,, Laying open of all communicating tracks and regu lar dressing. ,, Radical local excision of entire apocrine bearing perinea! skin with reconstruction using flap. ;., Recurrence is known to occur.

PRURITUS ANI It is intractable itching in and around anal canal. Skin is reddened, hyperkeratotic, cracked and moist. Causes ,, Poor hygiene. ;., Anal discharge due to fissure/fistula/piles/warts/polyp. ,, Trichomonas vaginalis infection of vagina in females. ,, Parasites; Epidermophytosis; Allergic cause; Dermatitis/ psoriasis. ;, lntertrigo/erythrasma (Corynebacterium minutissimum). ,.. Diabetes mellitus; psychological cause. Treatment: Proper cause should be assessed and treated. Good hygiene; local steroid application; topical xylocaine; strapping of the buttocks are needed.

GASTROINTESTINAL HAEMORRHAGE (GI BLEED)

Fig. 25.88 : Anatomy of ligament of Treitz.

I UPPER GI BLEED It is considered as: Variceal; Nonvariceal.

I Causes Peptic ulcer 55%. Ulcer bleeding is precipitated by NSAIDs, steroids, alcohol. Ulcer bleeding is overall common in men. But NSAID induced ulcer bleeding is common in females. Duodenal ulcer (35%) more commonly bleeds than gastric ulcer(20%) Gastroduodenal erosions; Oesophageal varices; Oesophagitis and erosions. Carcinoma stomach- 5% Mallory-Weiss syndrome--5-15%. Vascular anomalies-Dieulafoy's syndrome (AV malformation in the fundus of the stomach), Osler-Weber-Rendau syndrome, Ehlers-Danlos syndrome. Aortoduodenal fistula. Bleeding disorders. History of drug intake-an ticoagulants, clopidogrel, ecospirin.

Gastrointestinal bleed is classified as upper GI and lower GI bleed. Upper GI bleed is bleeding above the level of ligament of

Treitz. Lower GI bleed is bleeding below the level of ligament of Treitz. Ligament of Treitz is a fibromuscular band, which extends from right crus of diaphragm to duodenojejunal flexure with upper part made up of striped muscle fibres, lower part smooth muscle fibres and middle part with elastic fibres.

Gastric acid which inhibits the platelet aggregation Pepsin , by its proteolytic action causes erosion of the ulcer into the vessel. It also digests the clot, so as to aggravate the bleeding Mucosal blood supply pattern Gastric motility Alcohol, drugs

Major haemorrhage occ,rs wheo eros;oo of gaSfrodoodeoal artery or left gastric artery or splenic artery occurs or when bleeding occurs from varices ---

I

I Features

,

Acute bleed ;, Features of shock; Haematemesis; Melaena. Chronic bleed ,,. Hypochromic microcytic anaemia, glossitis, koilonychia, congestive cardiac failure; Mortality in upper GI bleed is 10%. Investigations ,... Gastroscopy to see the spurting vessel, oozing , clot in the ulcer, collected blood in the lumen of the stomach. ;, CT angiography of coeliac trunk and SMA. , Hb%, packed cell volume, CVP measurement, blood grouping and crossmatching. Ultrasound abdomen. ;.. LFT; prothrombin time; platelet count; blood urea and serum creatinine; serum electrolytes. :;.. Modified Forrest classification (refer Chapter 20Stomach) and Rocka/1 scoring system (better) is used. In Rocka/1 scoring system parameters used are-age (60 (0), 60-79 (1), >79 (2) Years]; shock [none (0), pluse >100 (1 ), pl use> 100 with hypotension (2)1; comorbidity [none (0), IHD (2), renal/liver failure/advanced maligancy (3)]; condition diagnosed [Mallory Weiss (0), all but mailigancy (1 ), upper GI maligancy (2)1 ; endoscopic finding [none (0), dark spot, blood/cloVvisible vessel (2)]. Score: 8 high. Treatment

acid/octreotide

CT angiography guided embolisation Surgical

• _ Endoscopic .__ _ _ _ _ _ _ _ _ _ _ _ _ _ _ _ _J

General: IV fluids, catheterisation, Ryle's tube aspiration, blood transfusion. Medical ,. Injection ranitidine IV 50 mg 8th hourly, or famotidine IV, omeprazole IV, pantoprazole IV. , Antifibrinolytics like tranexamic acid, EACA. , Somatostatin or octreotide, PP/ infusion. Others , Endoscopic therapy (tamponade, laser, haemoclip, banding, sclerotherapy, etc.) is the first line of therapy in all upper GI bleed. ,. CT angiography guided transcatheter embolisation of artery (gastroduodenal) is very useful in bleeding duodenal ulcer if endoscopic therapy fails. , Persistent recurrent bleeding needs surgical intervention. , For varices, vasopressin, propranolol, isosorbide dinitrate, Sengstaken tube tamponade, sclerotherapy, Boerema-Crile operation, Hasaab operation (devascularisation with splenectomy), oesophageal transection, Siguira-Futagawa operation or TIPSS.

,

For peptic ulcer, saline wash with 1 : 2,00,000 adrenaline, Nd:YAG laser therapy, cautery coagulation, th rombin injection, Finney's pyloroplasty, partial gastrectomy, ligation of gastroduodenal or left gastriartery, cytoprotectant mesoprostil injection. The cause is treated, once acute episode is under control.

981

C")

:c -a -4 m :::c N U'I

:::D (0

C')

c 3

Q)

= > ::I ::I Q)

Figs. 25.89A and B: Bleeding peptic ulcer (duodenal ulcer) is the commonest cause of upper GI bleed. Oesophageal varix causes dangerous severe life-threatening bleed.

,. Prognosis , Varices and gastric ulcer bleed has higher mortality. ,, Bleeding duodenal ulcer has got better prognosis.

I LOWER GI BLEED Bleeding in the GIT below the level of the ligament of Treitz. Normal faecal blood loss is 1.2 mUday. A loss more than 10 mUday is significant. Superior mesenteric artery thrombosis Superior mesenteric artery embolism Angiodysplasia

--

Small bowel tumour Carcinoma colon-I-~ ~..!" lntussusception

, __._..

Meckel's diverticulum

--+- Ulcerative colitis

_(T:=:/"-f'----:::i:=-- Diverticulum

-

~ -- -,-- Polyps - - - - --Carcinoma rectum

Piles ---..::::::::===sit==--

- - - F i s sure

Fig. 25.90: Causes of lower gastrointestinal bleed.

Causes ,. Angiodysplasia. ,.. Diverticular disease-commonest cause in Western countries. , Tumours of colon or small bowel. ;., Anorectal diseases- haemorrhoids, fissure-in-ano. , Ulcerative colitis, Crohn's disease. , Colorectal polyps; rectal carcinomas; lntussusception. , Tumours, either benign or malignant of colon or small bowel. , Meckel's diverticulum; Mesenteric artery occlusion. :.- lschaemic colitis; Stercoral ulcer; Infectious colitis.

His heart cannot be pure whose tongue is not clean.

(") Q)

::I Q)

982

Cl)

en .... ::I

-

Cl)

m

LOWER GI BLEED CAN BE:

Occult bleed: >10 mUday but not revealed Overt bleed: Bleeding which is revealed Overt acute Overt acute massive (bleed >1.5 litre/day) Overt chronic

C

::I C

:E

-"' = a:

COMMON CAUSES

Internal piles Diverticular disease Neoplasia

Inflammatory bowel diseases Angiodysplasia

Cl)

I Classification I. Bleeding may be: Small bowel bleed: Polyp', Meckel's diverticulum, mesenteric ischaemia, intussusception; small bowel tumor. Large bowel bleed: Angiodysplasia, carcinoma, colitis, diverticulitis, carcinoma. Anorectal diseases: Piles, fissure-in-ano, carcinomas.

II. Bleeding may be: Congenital: Polyp's, Meckel's diverticulum. Inflammatory: Ulcerative colitis, infective, amoebic, Crohn's disease. Neoplastic: Adenomas, carcinomas, polyps. Vascular: Angiodysplasia, mesenteric artery ischaemia, colitis. Others: Piles, fissure-in-ano. Angiodysplasia is common in caecum and ascending colon. Bleed ing more than 1.5 litres per day is called as acute massive GI bleed. ACUTE BLEED OCCURS IN:

• Mesenteric ischaemia: Angiodysplasia; lschaemic colitis Meckel's diverticulum; lntussusception Acute episodes of ulcerative colitis 80% of acute bleed regress spontaneously; 20% will become either massive or recurrent

I Presentations Acute bleeding presents with features of shock. Chronic blood loss occurs in piles, fissures, colitis. Presents with hypochromic, microcytic anaemia. Tenesmus, subacute obstruction, loss of appetite, decreased weight, bloody diarrhoea is seen in carcinoma distal, large bowel. Per-rectal examination is a must wh ich may reveal polyp , growth, ulcerations. Haematochezia. Mass palpable per abdomen in left or right iliac fossa or mass of intussusception.

Blood with mucus-col itis, carcinoma Fresh blood as splashes in the pan-piles Maroon coloured stool-Meckel's diverticulum • Red currant jelly in stool-intussusception Bright red blood in stool-polyps

I Investigations Hb¾, packed cell volume, ESR. Bleeding time; clotting time; prothrombin time; platelet count; blood urea and serum electrolytes. Occult blood in the stool-positive faecal blood test using Guaiac reagent. Barium enema. Proctoscopy for piles and sigmoidoscopy for rectosigmoid diseases. Colonoscopy for colitis, carcinomas, polyps. Small bowel enema (enteroclysis).

Figs. 25.91A and B: Sigmoid diverticula on colonoscopy. It is the

common cause of lower GI bleed in Western countries. Ultrasound abdomen. Mesenteric angiogram is very useful investigation in acute bleed , especially in angiodysplasia. Technetium scan for Meckel's diverticulum. Capsule endoscopy.

I Treatment Endoscopic fulguration or therapeutic embolisation or right hemicolectomy (for angiodysplasia). Endoscopic polypectomy for polyps. Treatment for ulcerative colitis with mesacol enema or drugs or total proctocolectomy with ileoanal anastomosis. Surgical resection of colonic carcinoma. Massive resection of small bowel in mesenteric ischaemia. Sigmoid colectomy in sigmoid diverticula. Cause is treated. Proper exploration through a lengthy midline incision is essential.

I OBSCURE GI BLEED It is intermittent GI bleed for which no source has been found endoscopically/radiologically. It is 5% common.

Commonly, it is due to either missed common cause or angiodysplasia. If it is angiodysplasia, angiography, nuclear scintigraphy, capsule endoscopy; then angiographic embolisation or resection of the part of the bowel is done. Enteroscopy, upper and lower GI scopies are needed in other conditions. All other conditions are treated accordingly.

B Blood • Haematocrit, LFT, blood urea, serum creatinine • Serum ferritin, iron, binding capacity • Coagulation profile-platelet count, BT, CT, PT, APTT Occult stool blood test (1-2 ml/day) • Benzidine test • Guaiac test • Haemoccult-guaiac impregnated electrophoresis paper • Fecal test • Immunological test Endoscopy I • Gastroduodenoscopy Proctosigmoidoscopy Colonoscopy-often difficult in acute bleed for clarity visualisa- J lion, if bleeding point is seen it can be controlled by cautery, laser, haemoclips, injection sclerotherapy, heater probe, etc. But still it becomes the test of choice once bleeding has controlled/ stopped temporarily

I

• I I I

I

L -- ---- ------ --------

Contd. ..

Contd... I • Small bowel endoscopy-push type (160 cm); Sonde enteroscope (275 cm, 5 mm diameter with balloon tip) are used. Entire small intestine is visualised while withdrawing but therapy or biopsy is not possible Angiography It identifies when b!eeding r~te !s 0.~ mU_ min; useful in _active I bleed; in therapeutic embolisat1on, in1ect1on vasopress1n can be done. Embolisation of small bowel vessel may cause bowel infarction which is dangerous and resection _ is needed in such si!uation. Visualisation in angiography can be improved by selective 1 infusion of vasodilators like tolazoline and prostaglandins, using / magnification films, using vasoconstrictor drugs Angiogragraphic criteria in angiodysplasia are-early and prolonged filling of draining veins; cluster of small arteries; visualisation of vascular tuft

I

I

I

I

• I J

I

Nuclear scinti graphy / • Identifies 0.1 ml/min of bleed ; Tc sulphur colloid scan is ve~ sensitive and is completed in 1 hour but increased uptake in spleen and liver obscures bleeding point and needs repeated / administration due to rapid clearance; Tc labeled RBC recirulates / and so effective for 1 day with better localisation • Advantages are-high-sensitivity even with active continued I bleed; screening test prior to angiography Problems are-no specificity; therapy is not possible

• IOther methods

• CTangiography; MR angiography • Aortography for aortoenteric fistula • lntraoperative localisation-on table enteroscopy; on table Doppler; on table bowel lavage and colonoscopy -- -- -- ------ - - - __,

God gives every bird its food, but he does not throw it into the nest.

____

983

C")

:c ):,,

'"1l

--i

m ::c N c.n :::c C")

i: 3

I» :::I

C.

>

:::I

I» (")

I»

:::::,

I»

Chapter

Urology A. Kidney It is no exaggeration to say that the composition of the blood is determined not by what the mouth ingests but by what the kidneys keep; they are the master chemists of our internal environment, which, so to speak, they synthesize in reverse. -Homer William Smith, 1939

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

ANATOMYOF KIDNEYAND URETER

( iHAPTER OUTLINE

.... ..

....

Anatomy of Kidney and Ureter Plain X-ray-Kidney, Ureter and Bladder Intravenous Urogram Retrograde Pyelography Renal Angiogram Micturating Cystourethrography Ascending Urethrogram Isotope Renography Cystoscopy Catheters • Foley's Catheter • Malecot's Catheter • Red Rubber Catheter Nephrostomy Suprapubic Cystostomy Haematuria Horseshoe Kidney Cystic Diseases of the Kidney

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

• Polycystic Kidney Disease • Solitary Renal Cyst Duplication of Renal Pelvis and Ureter Retrocaval Ureter Uretrocele Injuries to Kindney Renal Tuberculosis Hydronephrosis Pyonephrosis Carbuncle of Kidney Perinephric Abscess Renal Calculus Ureteric Calculi • Recurrence of Stones Staghom Calculus Benign Tumours of Kidney Wilms' Tumour Renal Cell Carcinoma

I KIDNEY-ANATOMY

They are a pair of excretory organs situated retroperitoneally, on the posterior abdominal wall; one on each side of vertebral column. Vertically it extends from the upper border of T12 vertebra to the centre of the body of L3 vertebra. The right kidney is slightly lower than the left, left kidney is slightly nearer to the median plane. Each kidney is about 11 cm long, 6 cm broad, 3 cm thick. Lateral border is convex. Medial border is concave with a hi lum. Structures seen in the hilum are (from anterior to posterior)- (1) renal vein , (2) renal artery, (3) renal pelvis. Upper pole is related to suprarenal gland; lower pole lies 1 inch above the iliac crest. Capacity of renal pelvis is 10 mL. The angle between the 12th rib and the outer border of the sacrosp inalis is the kidney angle. Kidney pain is usually referred here and pressure over this point elicits pain in kidney lesions. Hepatorenal pouch is a peritoneum lined deep recess, related to the upper pole of kidney. It is the lowest site when the body is in horizontal position, excluding the pelvis. Collection of extravasated fluid is likely to occur in this pouch following surgeries to liver and biliary tract.

I URETER-ANATOMY

I Capsules of Kidney 1. Proper capsule-fibrous membrane which can easily be

stripped off from the organ. 2. Perirenal fat is in the space of Ge rota. 3. Renal fascia of Gerota-has got anterior layer (Fascia of Toldt) and posterior layer (Fascia of Zuckerkandl). 4. Pararenal body of fat.

I Structure of Kidney The kidney is divided into anatomic segments based on blood supply. In the hilum, the main artery divides into anterior and posterior divisions. The anterior division supplies the apical, upper, middle and lower segments; posterior segment is supplied by the posterior division. The knowledge of this is important in the operation of anatrophic nephrolithotomy, where a functionally avascular plane (Brode~ between the posterior segment and the upper and middle segments exist on the posterior half of the kidney, about two-thirds of the way from the hilum to the lateral margin of the kidney. Gross-. Coronal section shows outer brownish cortex, inner pale medulla, renal sinus. Renal sinus is a space that extends into the kidney from hilus. It contains (1) branches of renal artery, (2) branches of renal vein, (3) renal pelvis. The pelvis divides into 2-3 major calyces which, in turn divides into 7-13 minor calyces. Histology: Each kidney is composed of 1-3 millions of uriniferous tubules ; each of which has a collecting part (collecting tubules) and secretory part (nephron). Blood Supply: It is from renal artery, which arises from the aorta at right angles, at the level of intervertebral discs between L1 and L2 vertebrae. About 95% of abdominal aortic aneurysm arises below the level of origin of renal artery.

Each ureter is 25-30 cm long. It begins within the renal sinus as a funnel-shaped dilatation called renal pelvis. It enters the bladder wall obliquely to open at the lateral angle of the trigone. It lies in the retroperitoneal space not attached to any fixed structures, so can be displaced or obstructed by retroperitoneal masses like tumours or aneurysms. Abdominal portion of ureter lies on the medial portion of psoas major muscle near the tips of transverse processes of lumbar vertebra (which is very well seen in IVP). The ureter enters the pelvis crossing the end of common iliac or beginning of external iliac artery. In females, uterine artery crosses the ureter from lateral to medial side, about 2 cm lateral to the cervix (this has to be remembered while ligating the uterine arteries in hysterectomy). Normal sites of constrictions-. (1) PUJ, (2) At the brim of lesser pelvis, (3) Along its passage through the bladder wall

Arterial supply: Upper segment of ureter receives blood supply from branches of renal and adrenal arteries; the middle portion from the branches of arteries of posterior abdominal wall; the pelvic portion from the branches of internal iliac arteries. ,. Vessels reach the abdominal portion of ureter on the medial side; whereas the pelvic portion receives on the lateral side (This has to be remembered while mobilising the ureter). The vessels form an anastomotic plexus in the adventitia of the ureter, which is found to be deficient in 10-15% of individuals leading to necrosis of cut ends of ureter following extensive mobilisation.

PLAIN X-RAY-KIDNEY, URETER AND BLADDER (KUB)

Fig. 26.1: Segments of kidney.

Nerve Supply: T10.11 .12 through the lesser and lower splanchnic nerves. Lymphatics: They are drained into para-aortic nodes. Perinephric fat is removed during nephrectomy (for malignancies), as intrarenal lymphatics freely communicate with the plexus in the perinephric fat.

Preparation of the patient: Enema/bowel wash/laxative is given on the previous day and the patient is asked to fast in order to reduce the bowel gas shadows in X-ray. High penetration X-ray is taken in supine position which covers pubic symphysis and lower two ribs. Interpreting the film: a. First bony parts are looked for, i.e. the hip, pelvis, lumbar vertebrae for fractures, scoliosis, spina bifida, secondaries in the spine. b. Kidney shadow. Kidney shadows are visualised in plain X-ray KUB due to difference in the density between kidney (high vascularity) and perinephric fat (low vascularity). Findings noted are size, location, calcification and stones. In children, perinephric fat is absent and so kidney shadows are not visualised. c. Psoas shadow. It is visualised well in normal KUB.

The remedy for injuries is not to remember them.

985

986

B In enlarged kidney In scoliosis due to inflammatory or infiltrative causes In malignancy Tuberculous spine with cold abscess (psoas abscess) Splenic injury-in left-sided shadow Retroperitoneal tumours

Minute /VU: In case of renal artery stenosis, within first minute many films are taken to see nephrographic shadow (where a small , concentrated kidney is seen). Nonvisualization of kidney: No contrast is seen in the film even after 12 hours.

d. Ureteric line: It is looked for any radio-opaque shadow (ureteric stone). It runs along the tips of the transverse processes of the lumbar vertebrae, crosses the sacroiliac joints and reaches up to a point medial to the ischial spine. e. Bladder, prostate and urethral areas are visualised for any lesion.

Fig. 26.2: Plain X-ray KUB AP-view. Note the psoas shadow.

INTRAVENOUS UROGRAM (IVU)

I Procedure

Renal function must be normal. Overnight fasting for 8 hours is advised. Laxatives are given to reduce bowel shadow and get a good quality film. First, a plain X-ray KUB is taken (IVU should not be read without doing KUB). Then 1 ml test dose of sodium diatrizoate ( Urograffin) or meglumine iothalamate is injected IV and waited for 5- 10 minutes for any reaction. If no adverse reaction occurs, then full dose 1 mUkg body weight, IV urogratfin is given (40-50 ml). ,:. X-ray is taken in 1-5 minutes, which shows the nephrographic and secretory function of the kidneys. Later 15 minutes and then 20- 30 minutes films are taken. Further films are taken depending on the need. Film can be taken as late as 72 hours. Late films show bladder pathology as well as residual urine. In case of renal failure with high blood urea, dose of dye is increased to 2 mUkg body weight to get a better film-Infusion /VU. Often diuretics are used in these patients to have better secretion. Lower abdominal compression is done for 1O minutes to have better definition of calyces, but not done in children and patients with abdominal aortic aneurysm.

Figs. 26.3A to D: IVU showing hydronephrosis with clubbing of calyces and dilatation. Delayed post-lasix film often should be taken whenever there is poor secretion in initial films. Film can be taken as late as 72 hours. This delayed film shows dilatation. IVU showing hydronephrosis on right side; normal secretion on left side; with bladder filling.

Fig. 26.4: Ectopic kidney right sided IVU picture. It is un-ascended kidney. It often presents as mass in the right iliac fossa. CT scan is diagnostic. Differential diagnoses for masses in right iliac Iossa are nodal mass; carcinoma caecum; tuberculosis; psoas abscess; retro peritoneal tumour. Sepsis; stones; tumour; can occur in ectopic kidney. Management of whatever problems arise is done through open method. Bilateral ectopic kidney may lead into eventual renal failure. Isotope scan is diagnostic.

T

Indications 1. Hydronephrosis

987

Disadvantage: Anaesthesia is required and is laborious.

Intravenous Urogram (IVU)

Findings C")

Clubbing of calyces

:c

>

2. Congenital anomalies a. Horseshoe kidney

rr,

::c N en

Flower vase appearance

>

b. Duplex kidney and double ureter

...

c::

c. Ureterocele

Adder (cobra) head appearance

d. Polycystic kidney disease

Spider leg appearance

0

0

e. Retrocaval ureter

IC




994

I Features Presents as a fixed, non mobile, firm mass in the mid line at the level of 4th lumbar vertebra which is resonant on percussion. It is more prone for infection, stone formation, hydronephrosis, tuberculosis. Investigations: ,,. IVU-medialisation of lower calyces and curving of ureter like a 'flower vase'. :.- Ultrasound abdomen .

Fig. 26.23: IVU showing flower vase appearance of horseshoe kidney.

Urine analysis, blood urea and serum creatinine are su pportive investigations. CT abdomen, CT angiogram. Treatment :.- Whatever the complication occurs in horseshoe kidney, it is treated accordingly. ,,. Per se separation of isthmus is not indicated, unless to approach aorta for aortic diseases.

CYSTIC DISEASES OF THE KIDNEY

I Types Genetic , Adult polycystic kidney disease (Autosomal dominant). :.- Infantile polycystic kidney disease (Autosomal recessive)-fatal. Nongenetic: Simple cyst, multicystic kidney, medullary sponge kidney. Acquired renal cystic disease may develop in patient on long-term dialysis.

I POLYCYSTIC KIDNEY DISEASE (PCKD) Adult PCKD is inherited as autosomal dominant disease. It is common in females.

It is bilateral and presents in third decade. One side presents little earlier than other side.

Figs. 26.24A and B: Polycystic kidney disease, picture and CT scan. It is bilateral, multiple.

Associations ,,. Polycystic diseases of liver (18%), pancreas and lungs. , Berry aneurysm in the circle of Willis. Cyst formation occurs at the junction of the distal tubule and the collecting duct. Grossly it contains multiple cysts with a clear or brownish fluid (due to haemorrhage) .

B • Bilateral palpable renal mass • Infection • Loin pain • Hypertension • Haematuria • Uraemia

Renal mass, which is lobular, firm, mobile, moves with respiration, ballotable, with dull renal angle and resonant band in front. Pain, which is due to stretch of renal capsule or haemorrhage into a cyst. Haematuria (25%), due to overdistended cyst rupturing into the renal pelvis. Infection is due to stasis. Hypertension (75%). Uraemia occurs in late stage due to renal failure.

Differential diagnosis: Renal cell carcinoma, Hydronephrosis, Solitary renal cyst Investigations , Ultrasound confirms the presence of cysts. ,. IVU-Spider leg pattern with an elongated compressed renal pelvis, narrowed and stretched calyces. ;... Blood urea and serum creatinine. , Urine shows low specific gravity. Treatment , Wait and watch policy. , If one of the cysts overdistends causing pain, haemorrhage, infection, then surgical intervention is required. ,. Rovsing operation: The kidney is exposed. The cyst is opened. The fluid is evacuated. The cut edge is marsupialised. ,. Presently US-guided aspiration is done as a simpler approach. , Laparoscopic/retroperitoneoscopic aspiration/de-roofing of the renal cyst. :- Once renal failure sets in, then initial haemodialysis followed by bilateral nephrectomy, is done and later renal transplantation should be planned for.

I SOLITARY RENAL CYST Solitary renal cyst is never congenital. It is due to an earlier trauma or infection resulting in blockage of tubule, leading to cyst formation. It is usually unilateral, presents as a renal mass which is smooth, often tender if infected or haemorrhagic. Differential diagnosis: Renal neoplasm, Hydronephrosis, Polycystic disease, Hydatid cyst. Solitary cyst Kidney

C"')

::c

>

"'0

-t

m

:::0 N a,

>

Fig. 26.26: Left siderenal cyst-CT picture. It could be solitary renal cyst. It can be aspirated or de-roofed by laparoscopy/retrope-ritoneoscopy.

DUPLICATION OF RENAL PELVIS AND URETER It is most common congenital anomaly of the upper urinary tract (4%). Usually unilateral. Common on the left side. In 3% of cases, it is associated with duplication of ureter. Upper renal pelvis is small, drains the upper calyces. Lower renal pelvis is larger, drains the middle and lower calyces. Double ureter when associated, may be partial where two ureters join in lower third or complete where upper ureter opens into the bladder at a lower level and lower ureter opens into the bladder at the upper, normal ureteric orifice. This is called as " Weigert Meyer Law". In partial duplex, there is reno-renal reflux resulting in infection, stone formation and hydronephrosis. Investigations , !VU-diagnostic. US to look for complications. , Cystoscopy shows double ureteric orifices on the same side. , DTPA scan to see the function.

Fig. 26.25: Solitary renal cyst. It is acquired, unilateral.

Investigation: Ultrasound and IVU confirms the diagnosis, CT scan. Treatment , Kidney is exposed. The cyst is aspirated and a portion of the cyst wall is removed (Kirwin 's operation) and cavity is filled with perinephric fat. :..- Occasionally if the cyst is in one of the poles, partial nephrectomy is done. , Laparoscopic approach.

Partial

Fig. 26.27: Duplex renal system .

When the patient dies the kidneys may go to the pathologist, but while he lives the urine is ours. It can provide us a serial story of the major events going on within the kidney-Thomas Addis

Complete

996

RETROCAVAL URETER It is due to developmental defect of IVC, as a result of which right ureter passes behind the IVC, causing right-sided hydronephrosis with upper third hyd roureter.

...=

en

Kidney

(I)

IVC

'E C'O

=

C C'O

en CCI

a: (I)

Figs. 26.28A and B: Bilateral complete duplex kidney on table look and X-ray postoperatively. Both duplex ureters are reimplanted into the urinary bladder.

Treatment , Ureteric meatotomy is done if there is narrowing of the orifice. , The co-existing complications are treated.

Figs. 26.30A and B: Retrocaval ureter-lVU picture showing reverse J sign. On table finding of retrocaval ureter. It is treated by Anderson's Hynes operation. It causes hydronephrosis. It is due to anomalous development of IVG.

IVU shows hydronephrosis with 'reverse J sign'. Treatment.Anderson Hynes' operation.

URETEROCELE Fig. 26.29: IVU showing bilateral duplex kidney.

, ,

Often heminephrectomy including removal of corresponding ureter may be essential as treatment. In females with complete duplication, lower ureteric orifice is ectopic, causing urinary incontinence which needs partial nephrectomy or ureteric reimplantation.

It is a cystic enlargement of the intramural portion of ureter due to congenital atresia of the ureteric orifice. Its wall contains mucous membrane only. It is common in females, often bilateral (10%). Complications: Stone formation; Recurrent infection; Hydronephrosis.

997 n

::::c

>

"'CJ

--f

m :::c N

0)

>

...

C: 0

0

ICI

'


...c::

C C IC




"'lJ

-I

rn

::::i::,

N a,

>

C: .... C C ICI




""C

-I IT! ::D N

en

Ureteric orifice

Diverticula (only mucosa)

=

...c:: 0 0

IC

-:-;

c::

~-

Fig. 26.101: Micturating cystourethrogram (MCU) showing gross vesicoureteric reflux on both sides.

=

I»

50 years with PSA

m :c N en

n

...

C: 0 0 IC