Srb's Manual of Surgery [6 ed.] 9352709071, 9789352709076

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SRB’s

Manual of

Surgery

6

th Edition

SRB’s

Manual of

Surgery Sriram Bhat M ms (General Surgery)

Professor and Head Department of Surgery Kasturba Medical College Mangalore Mangaluru, Karnataka, India Honorary Surgeon Government Wenlock District Hospital Mangaluru, Dakshina Kannada, Karnataka, India e-mail: [email protected] Forewords

M Venkatraya Prabhu Thangam Verghese Joshua

JAYPEE BROTHERS Medical Publishers The Health Sciences Publisher New Delhi | London | Panama

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Jaypee Brothers Medical Publishers (P) Ltd Bhotahity, Kathmandu, Nepal Phone: +977-9741283608 Email: [email protected] Website: www.jaypeebrothers.com Website: www.jaypeedigital.com © 2019, Jaypee Brothers Medical Publishers The views and opinions expressed in this book are solely those of the original contributor(s)/author(s) and do not necessarily represent those of editor(s) of the book. All rights reserved. No part of this publication may be reproduced, stored or transmitted in any form or by any means, electronic, mechanical, photocopying, recording or otherwise, without the prior permission in writing of the publishers. All brand names and product names used in this book are trade names, service marks, trademarks or registered trademarks of their respective owners. the publisher is not associated with any product or vendor mentioned in this book. Medical knowledge and practice change constantly. This book is designed to provide accurate, authoritative information about the subject matter in question. However, readers are advised to check the most current information available on procedures included and check information from the manufacturer of each product to be administered, to verify the recommended dose, formula, method and duration of administration, adverse effects and contraindications. It is the responsibility of the practitioner to take all appropriate safety precautions. Neither the publisher nor the author(s)/editor(s) assume any liability for any injury and/or damage to persons or property arising from or related to use of material in this book. This book is sold on the understanding that the publisher is not engaged in providing professional medical services. If such advice or services are required, the services of a competent medical professional should be sought. Every effort has been made where necessary to contact holders of copyright to obtain permission to reproduce copyright material. If any have been inadvertently overlooked, the publisher will be pleased to make the necessary arrangements at the first opportunity. the CD/DVD-ROM (if any) provided in the sealed envelope with this book is complimentary and free of cost. Not meant for sale. Inquiries for bulk sales may be solicited at: [email protected]

SRB's Manual of Surgery First Edition Second Edition Third Edition Fourth Edition Fifth Edition Sixth Edition

: 2004 : 2007 : 2009; Reprint  2010 : 2013; Reprint 2013 : 2016; Reprint 2017 : 2019

ISBN 978-93-5270-907-6

God could not be everywhere all the time, so, He created Mothers

This book is in the memory of my mother Late Mrs Devaki Krishna Bhat who continues to inspire and guide me

This edition is dedicated to my beloved Professor

Late Professor Kodial Prakash Rao; Mangaluru

Foreword I am very proud to write a foreword to this book SRB’s Manual of Surgery (6th edition) written by Dr Sriram Bhat M, Professor and Head, Department of Surgery, Kasturba Medical College Mangalore, Mangaluru, Karnataka, India. Dr Sriram Bhat was a hard working undergraduate student with me in 1980s and shaped up as a gifted surgeon and our excellent teacher for undergraduates and postgraduates. in addition, he has proven to be a good teacher having organized many continuing medical educations (CMEs) and workshops. This book is meant for training the students for general surgery especially to face the university examination. I hope that this book will be different from other books. It will be student-centric rather author-centric. I am supremely confident that this book will serve as a constant guide to all surgical issues.

M Venkatraya Prabhu MD (General Medicine) Dean and Professor Department of Medicine Kasturba Medical College Mangalore Mangaluru, Karnataka, India

Foreword ‘To study the phenomenon of disease without books is to sail an uncharted sea; while to study books without patients is not to go to sea at all’. —Sir William Osler (1849–1919) Professor of Medicine, Oxford, UK

SRB’s Manual of Surgery has helped innumerable young doctors in the making, to start their voyage on a sea of surgical knowledge, well equipped with a sense of direction. This book is in its sixth edition, which speaks volumes about its popularity among the medical students. the first edition came out in 2004. It soon gained nationwide recognition as a comprehensible source of information. the text and the style of writing is easy to understand and to remember, the accompanying diagrams and photographs are perceivable and unforgettable—a combination which is so very important to a student facing theory and practical examinations. With each new edition, this manual has been updated and upgraded with a lot of dedication by the author. the newer concepts of treatment and staging of malignancies have been documented. Surgical anatomy has been included wherever the author deemed it essential. the book contains all the important chapters of general surgery, the common conditions have been dealt with in detail. the reader is also introduced to the various surgical specialities and is imparted with a basic yet-practical knowledge, which is an essential armament to both—the student of surgery as well as to the young practicing surgeon. I consider it a great privilege to be given the honour of writing a foreword to this manual, which has been authored by Dr Sriram Bhat M, a favourite student of mine. He is a great teacher, a skillful surgeon, renowned author of several surgical books, but above all a good human being. in spite of all the fame that rests lightly on his shoulders, he remains humble; in spite of being knowledgeable, he remains eager to learn, and in spite of being busy, he gives quality time to his family and friends. I wish Dr Sriram Bhat M, the best in his endeavours of inspiring generations of competent surgeons. I also wish the students who read this book to be greatly benefited, and perhaps one-day surpass their teachers.

Thangam Verghese Joshua MS MCh Professor and Ex-Head Department of Surgery Kasturba Medical College Mangalore Mangaluru, Karnataka, India

Preface to the Sixth Edition It is my pleasure to release the sixth edition, three years after the release of fifth edition of SRB’s Manual of Surgery. As my earlier editions were well accepted by the students, I intend to bring out the sixth edition in the intention of updating the treatment strategies of the needed surgical conditions. Extensive corrections, elaboration of a few topics, and updating of staging and grading of malignant conditions as per new standards have been done in this edition. AJCC 2018, 8th edition staging system is added. Style, formatting and fonts are rearranged as per students’ need. Newer concepts have added. Few subtopics like stings and bites are removed. I have referred many books, journals and took the help of my colleagues in surgery and other departments. I have retained all chapters with some rearrangements of a few chapters and topics. I sincerely thank our Dean, Professor M Venkatraya Prabhu for agreeing to give Foreword to this edition. I dedicate this edition also to my beloved Professor Kodial Prakash Rao who passed away recently. For better understanding of the students, three sizes of fonts are used namely—10pt, 9pt, and 8.5pt. Important subjects are kept in font size 10pt; remember, boxes, tables and other subjects are in 9pt; and notes, are in 8.5pt. This will be useful especially for undergraduates to understand which topics have got priorities. I sincerely express my thanks to everybody who have helped me and also to the publishers who are the backbone of this upgraded edition. I hope this edition will be well accepted by the teachers, surgeons, undergraduates and postgraduates of surgery department. I sincerely welcome all criticisms.

Sriram Bhat M

[email protected]

Preface to the First Edition This book is born out of a desire to provide a complete, authoritative, current textbook on surgery. for the past fifteen years of my teaching profession, watching students hurriedly jotting down notes during classes, an urge was born within me to put my thoughts in print. I am fortunate to have been guided by my teachers and supported by my students towards this goal. An attempt has been made to make this book simple and reader-friendly while not compromising on the aspect of providing students with necessary information and a better clinical understanding of surgery. Recent advances in surgery till date have been included to the best of my knowledge. Text has been presented in a simple and lucid language so as to help students understand and recapitulate the subject better. Since a picture is worth than a thousand words, over 500 photographs, X-rays and illustrations have been incorporated in this work to make surgery more interesting and understandable. Inspirational quotes have been interspersed to motivate students to go the extra mile. Discussion on Instruments and Operative Procedures has been dealt with in necessary detail separately. A chapter containing a list of interesting new developments in surgery and uniquely new topics like Fascinating Signs, Misnomers, Triads and other interesting chapters have been included. While this book has been compiled with undergraduates in mind, I am confident that it will serve as a useful reference for postgraduates and practitioners. In compiling this book I have consulted many authoritative books and publications and I sincerely express my appreciation and gratitude to all of them. Suggestions and constructive criticisms towards improving this book in subsequent editions are always welcome.

Sriram Bhat M

[email protected]

Acknowledgements I am happy to bring out one more edition (sixth edition) of the book SRB’s Manual of Surgery. This is due to constant help and support of many. ŠŠ I thank our Chancellor Dr Ramdas M Pai, Pro-Chancellor Dr HS Ballal, Vice-Chancellor of Manipal Academy of Higher Education (MAHE); Dr Vinod Bhat, Pro-Vice-Chancellor, Dr Poornima Baliga, and Dr Surendra Shetty. ŠŠ I immensely thank our beloved Dean, Prof Venkatraya Prabhu, for his constant support and affection and also I sincerely thank him for writing foreword to this edition. ŠŠ I thank our Additional Dean, Dr Alfred Augustine, Vice-Deans Dr Unnikrishnan and Dr Nuthan Kamath, for their affectionate help. ŠŠ I always remember my senior teachers, Prof CR Ballal, Prof Suresh Kamath, Late Prof K Prakash Rao, Late Prof Subramanya Bhat, Prof Bhaskar Shetty and Prof Ramachandra Pai, for their constant help. ŠŠ Late Prof Prakash Rao, is a guide and encourager to everybody, will be remembered as a special person by me always. ŠŠ My special and affectionate thanks to Prof Thangam Verghese Joshua, for agreeing to write a foreword. She is my teacher, constant guide in all my endevours. ŠŠ My special thanks to Prof Dr Jayaprakash Rao for his affection and support. ŠŠ Surgical unit heads in our college—Dr Jayaram Shenoy, Dr Thangam Verghese Joshua, Dr Harish Rao, Dr Shivaprasad Rai, Dr Yogishkumar, Dr Alfred Augustine and Dr Shivananda Prabhu, are always supportive for my work and are worth to be remembered always. ŠŠ I am grateful to all my teachers and colleagues in surgery department, who directly or indirectly helped me to bring out this sixth edition. ŠŠ I appreciate District Medical Officer and Resident Medical Officer of Government Wenlock District Hospital, Mangaluru, Dakshina Kannada, Karnataka, for their kind help. ŠŠ I will never forget my close associates—Dr Ganapathy, Mangala Hospital, Kadri Mangalore and Dr Ashok Pandith, Urologist, for their affectionate help and encouragement in all my endeavours. They always stood with me in my difficulties. ŠŠ I thank very much to the faculty, Department of Surgery and Paediatric Surgery, JJM Medical College (JJMMC), Davangere, Karnataka, for providing the needed photographs. ŠŠ My special thanks to Prof Laxman Prabhu, Department of Urology; Prof Sribatsa Kumar Mohapathra, Veer Surendra Sai Medical College (VSSMC), Burla, Sambalpur, Odisha, India; Prof Ramlingam, Kamineni Institute of Medical Sciences (KIMS), Narketpally, Telangana; Dr Arun Kumar, Surgical Gastroenterologist, Thiruvananthapuram, Kerala, India, Prof Sureshchandra, and Prof Narayanaswamy Srinivasan, Bengaluru for their contribution in this title. ŠŠ I sincerely thank Prof Navinchandra Shetty, Radiologist, Mangaluru, for his help in providing and guiding me in X-rays, CT scans and imaging methods. ŠŠ I acknowledge Prof Kishore Chandra Prasad, Ex-Head, Department of ENT, for his help, guidance and encouragement in bringing out this book. ŠŠ I thank Dr Manohar Pai, Dr Ashfaque Mohammed, Dr Shibu Mohan, Dr Poornachandra, Dr Keshava Prasad, Dr Sunil Kumar Shetty, Dr Rahul Bhat, Dr Ranjith Rao, Dr Kalpana Sridhar, Dr K Akbar, Dr Achaleshwar Dayal, Dr Raghav Pandey, Dr Rupen Shah, Dr Ashwini Mallya, Dr Praveen, Dr Irshad, Dr Amar, Dr Rajesh, Dr Suyog, Dr Chandini, Dr Adithya, Dr Ashok Hegde, Dr Rajesh Ballal, Dr Devidas Shetty, Dr Venkatesh Sanjeeva, Dr Shanbogh, Dr Harish Nayak, Dr Subraya Kamath, Dr Venkatesh Shanbogh, Dr Tantri, Dr Sandeep Gopal, Dr Ganesh, Dr Suresh Shenoy, Dr Sampath, Dr Shabin, Dr Nigil, Dr Nidhin, Dr Shijith, Dr Ramneik, Dr Lokesh, Dr Ayaz, and Dr Bichu, for their help in various aspects. ŠŠ I sincerely appreciate Dr Raghavendra Bhat and Dr Ravichandra, Consultants, Department of Radiology, Yenepoya Medical College, Mangaluru, for their contribution and affectionate help.

SRB's Manual of Surgery

xvi ŠŠ I thank Dr Ganesh Pai (Paediatric Surgeon), Dr Suresh Pai, Dr Ashok Shetty (Cardiothoracic Surgeon), Dr Muralidhar Pai (Neurosurgeon), Late Dr Vivekanand Prabhu (Paediatric Surgeon), Dr Sadashiva Rao (Paediatric Surgeon), Dr Narayana Bhat (Paediatric Surgeon), Dr Prashanth Mallya (Anaesthetist), for their help and contributions. ŠŠ I thank my friend Dr Jagadish, for his contributions to X-ray and opinion on dental and faciomaxillary topics. ŠŠ My wife Dr Meera Karanth, helped me day and night in revising this edition and without her help this could not have been possible. My beloved daughter Ananya helped me in drawing new diagrams artistically. I enjoy her love and affection towards me. ŠŠ I remember my students Dr Ravi CR (Chitradurga), Dr Ashwini Polnaya (Mangaluru), Dr Ishwara Keerthi, Dr Sudesh, Dr Narasimha Murthy, Dr Nithin, Dr Ashish Singh, and Dr Aromal, for their special contributions. ŠŠ I thank all my students especially postgraduates, Department of Surgery, who were helping regularly in bringing out this edition. ŠŠ Words are not sufficient to remember all my patients who are the main material for the book. I pray for their good health always. ŠŠ I appreciate Shri Jitendar P Vij (Group Chairman), Mr Ankit Vij (Managing Director), Mr MS Mani (Group President), Dr Madhu Choudhary (Publishing Head–Education), Ms Pooja Bhandari (Production Head) and all staff of M/s Jaypee Brothers Medical Publishers (P) Ltd, New Delhi, India, for doing appreciable work in their respective field of printing and publishing. Also, my appreciation to working team in Jaypee Brothers Medical Publishers (Bengaluru and Mangaluru branches), for their timely help. ŠŠ My sincere thanks to editing team members—Ms Sunita Katla (Executive Assistant to Group Chairman and Publishing Manager); Ms Seema Dogra (Cover Visualiser); Mr Rajesh Sharma (Production Coordinator); Mr Laxmidhar Padhiary, Mr Binay Kumar and Mr Vakil Khan (Proofreaders); Mr Kapil Dev Sharma, Mr Kulwant Singh, Kuldeep, Akshay Thakur, Ajeet Rathor (DTP Operators), and Mr Gopal Singh Kirola (Graphic Designer) of M/s Jaypee Brothers Medical Publishers (P) Ltd, New Delhi, India, for their dedicated and affectionate work in bringing out the sixth edition of the title.

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/Ischaemic 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  27; Bazin’s Disease  27; Tropical Ulcer  27; 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

D. 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 ;

Hyponatraemia  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;

SRB's Manual of Surgery

Disseminated Intravascular 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; Duodenal Injury  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 Ischaemic 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 Ischaemia  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;

Raynaud’s Phenomenon  176;

Thromboangiitis Obliterans  172;

Temporal Arteritis  177;

Takayasu’s Pulseless Arthritis  175;

Treatment of Arterial Diseases  177;

Subclavian Steal

Syndrome  183; Acute Arterial Occlusion  183; Traumatic Acute Arterial Occlusion  184; Embolism  184; Reperfusion 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 Ischaemia  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;

Thrombosis  216;

Venous Ulcer  232;

Varicose Veins  219;

Deep Vein

Compression Therapy for Varicose Veins  234;

Thrombophlebitis  235; Klippel-Trenaunay Syndrome  235; Anticoagulants  235; Oral Anticoagulants  236; Pulmonary Embolism  237

O. Lymphatics  239 Surgical Anatomy  239; Lymphadenitis  241;

Lymphangiography  240;

Lymphoedema  241;

Isotope Lymphoscintigraphy  241;

Lymphomas  248;

Acute Lymphangitis and

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; Brachial 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 Contents

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  270; Management Strategy for Cancers  274

R. Skin Tumours  276 Anatomy  276 ;

Clasification of Skin Tumours  277 ;

Dermatofibrosarcoma Protuberans  279;

Skin Adnexal Tumours  277 ;

Keratocanthoma  279;

Rhinophyma  279;

Dermatofibroma  278 ;

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; Immunosuppressive 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;

392

Saliva  395;

Sialography  395;

Salivary Calculus and Sialadenitis  396;

Parotid Abscess  398;

Parotid Fistula  399; Recurrent Childhood Parotitis  399; Sjögren’s Syndrome  400; Sialosis  400; Sialectasis  400; Salivary Neoplasms  400; Adenoma  404;

Pleomorphic Adenoma  401;

Mucoepidermoid Tumour  404;

Adenolymphoma  403;

Oncocytoma  404;

Adenoid Cystic Carcinoma  405;

Basal Cell

Acinic Cell Tumour  405;

xx

Malignant Mixed Tumour  405; Glands  406;

Adenocarcinoma of Salivary Glands  406;

Submandibular Salivary Gland Tumours  406;

Squamous Cell Carcinoma of Salivary

Minor Salivary Gland Tumours  409;

Parotid

SRB's Manual of Surgery

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

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; Sternomastoid Tumour  430; Tuberculous Lymphadenitis  431; Cold Abscess  434; Secondaries in Neck Lymph 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 Incidentaloma  479; Thyroidectomy  479; Emil Theodor Kocher  485; Kocher’s Test  485; Hypothyroidism  485; Recurrent Laryngeal Nerve Palsy  486

7. Parathyroids and Adrenals Anatomy  489;

Calcium  489;

Syndrome)  496; Tumours  498;

489

Hyperparathyroidism  490;

Apudomas  496;

Parathyroidectomy  493;

Hypoparathyroidism  497;

Adrenocortical Carcinoma  499;

Tetany  498;

Cushing’s Syndrome  500;

Men Syndrome (Mea

Adrenals  498;

Adrenal Cortical

Conn’s Syndrome  500;

Virilising

Syndrome or Adrenogenital Syndrome  500; Neuroblastoma  500; Phaeochromocytoma  502

8. Breast anatomy  504;

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; Disease  517;

Galactocele  514;

Tuberculosis of the Breast  518;

Mastitis  515;

Antibioma  517;

Breast Cysts  518;

Duct Ectasia  517;

Galactorrhoea  519;

Mondor’s

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 Anatomy  557;

Physiology  557;

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

Abdominal Tuberculosis  573; Ileocaecal Tuberculosis  574; Ileal 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

xxi

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

12. Gallbladder

623 Oral Cholecystogram  625;

Cholangiopancreatography  625;

Intravenous Cholangiogram  625;

Percutaneous Transhepatic Cholangiography  625;

Contents

Surgical Anatomy  623;

Endoscopic Retrograde Magnetic Resonance

Cholangiopancreatography  626; Radioisotope Scan Study  626; Peroperative Cholangiogram  626; Postoperative T-tube Cholangiogram  627;

Congenital Anomalies of Gallbladder  627;

Choledochal Cysts  628;

Caroli’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 Ileus  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;

Single Incision Laparoscopic Surgery in Cholecystectomy  657;

Laparoscopic Cholecystectomy  656;

Bile Duct Injuries  658;

Post-cholecystectomy

Syndrome  658; Biliary Dyskinesia  659

13. Spleen

660

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 ;

Thrombocytopaenic Purpura  668;

Idiopathic Thrombocytopaenic Purpura  667 ;

Splenectomy  668;

Thrombotic

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; Insulinomas  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  707; 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; Incisional

739

xxii

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

SRB's Manual of Surgery

Surgery  774; Parastomal Hernia  775

19. Oesophagus Anatomy  776;

776 Lower Oesophageal Sphincter  778;

Oesophagoscopy  780;

Dysphagia  778;

Oesophageal Endosonography  781;

Contrast Study of Oesophagus  780;

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; Tracheo-oesophageal Fistula  793; Oesophageal Diverticulum  794; Carcinoma Oesophagus  795; Benign Tumours of the Oesophagus  802; Oesophageal Perforation  802

20. Stomach Anatomy  804;

804 Gastric Physiology  806;

Gastric Function Tests  807;

Gastrin  807;

Barium Meal Study  808;

Gastroscopy  808; Congenital Hypertrophic Pyloric Stenosis  811; Gastritis  812; Acute Peptic Ulcer  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 Surgery  828; Trichobezoar  831; Chronic Duodenal Ileus  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 Intussusception  850; Vagotomy  851

21. Small Intestine Anatomy  852;

852

Meckel’s Diverticulum  853;

Regional Enteritis  855;

Surgical Complications of Typhoid  858;

Pneumatosis Cystoides Intestinalis  861; Mesenteric Vessel Ischaemia  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; Ischaemic 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 Ileus  918; Intussusception  919; Volvulus  922; Sigmoid Volvulus  922; Paralytic Ileus  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

942

Surgical Anatomy of Rectum  942; Surgical Anatomy of Anal Canal  943; Per-rectal Examination of the Rectum  945; Proctoscopy (Kelly’s)  945; Syndrome  952;

Sigmoidoscopy  945;

Rectal Prolapse  952;

Colonoscopy  945;

Carcinoma Rectum  946;

Anorectal Malformations  957;

Solitary Ulcer

Pilonidal Sinus/Disease  958;

Piles/

Haemorrhoids  961; Anal Fissure  967; Anorectal Abscess  969; Fistula-In-Ano  971; Anorectal Strictures  976; Condyloma Acuminata  976; Anal Intraepithelial Neoplasia  976; Malignant Tumours of Anal Area  976; Sacrococcygeal

xxiii

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

26. Urology

984 Contents

A. Kidney  984 Anatomy of Kidney and Ureter  984; Retrograde Pyelography  987 ;

Plan X-ray—Kidney, Ureter and Bladder  985;

Renal Angiogram  987 ;

Intravenous Urogram  986;

Micturating Cystourethrography  987 ;

Ascending

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  1000; Calculus  1005;

Pyonephrosis  1004;

Ureteric Calculi  1009;

Carbuncle of Kidney  1004;

Staghorn Calculus  1011;

Perinephric Abscess  1005;

Benign Tumours of Kidney  1012;

Renal Wilms’

Tumour  1012; 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-Löwenstein 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; Intracranial Abscess  1086 ;

Intracranial Aneurysms  1087 ;

Intracranial Tumours  1087 ;

Pituitary Tumours  1090 ;

Craniopharyngiomas  1091; Spinal Dysraphism  1091; Meningocele  1092; Spina Bifida  1092; Intervertebral 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; Intercostal Tube Drainage  1105; Shock Lung  1107; Pulmonary Embolism  1107; Surgical Emphysema  1108; Lung Cysts  1109; Mediastinal Tumours  1109; Thymomas  1111; Lung Cancers  1111; Pancoast Tumours  1112; Chest Wall Tumours  1112; Pericarditis  1113; Pericardial Tap  1113;

1097

xxiv

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

SRB's Manual of Surgery

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

Patent Ductus Arteriosus  1120;

Coarctation of Aorta  1121;

Atrial Septal

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

29. Adjuvant Therapy

1126

Radiotherapy  1126; Chemotherapy  1128; Cell Cycle  1128; Antimalignancy Drugs  1129; Hormone Therapy in Cancer  1129; Immunosuppression  1129; Immunotherapy  1131; Hybridoma  1131; Gene Therapy  1131

30. Anaesthesia

1132

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

32. Operative Surgery

1144

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

B. Operative Procedure  1156 Abdominal Incisions  1156;

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

1 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 shouldst draw it together for him with two strips (of plaster), and thou shouldst treat it with grease and honey every day until he recovers. —[Anonymous], circa 2500 BC

C hapter Outline ·· ·· ·· ·· ·· ··

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

·· ·· ·· ·· ··

¾¾ ¾¾

Degloving Injuries Scar Keloid Hypertrophic Scar Problems with Wound Healing

b. Untidy wounds ¾¾ ¾¾

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. ¾¾ ¾¾

WoundS

It is incised, clean, healthy wound without any tissue loss. Usually primary suturing is done. Healing is by primary intention.

Liberal excision of devitalised tissue and allowing to heal by secondary intention is the management. Secondary suturing, skin graft or flap may be needed.

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 lining—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 ¾¾

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

SRB's Manual of Surgery

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.

Fig. 1.1B: Untidy wound.

A

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

B

Figs. 1.3A and B: Lacerated wounds.

A

B

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

A

B

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

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 avulsion 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. Ischaemia, 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 brachial 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

3

CHAPTER 1A    General Surgery: Wounds and Wound Healing

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.

SRB's Manual of Surgery

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 injuries 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. 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).

III. 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

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.

™™ Simple wounds are one involving only one organ or tissue. ™™ Combined/complex wounds are one involving mixed tissues.

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 are— Appendicectomy, gastrojejunostomy, pancreatic and biliary surgeries.

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Fig. 1.14: Contaminated wound—burst appendicitis.

Fig. 1.12: Clean wound of thyroidectomy surgery.

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

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Wound classifications

• Simple wounds: Only skin is involved

• Tidy wounds

• Complex wounds: Vessels, nerves, tendons or bones are involved

• Untidy wounds

• Closed wounds:

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

–– Contusion –– Abrasion –– Haematoma

B

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

• Open wounds: –– –– –– ––

Incised wounds Lacerated wounds Crush injuries Penetrating wounds

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.

CHAPTER 1A    General Surgery: Wounds and Wound Healing

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%.

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WOUND HEALING A scab is a beautiful thing—a coin the body has minted, with an 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

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 → inflammation → proliferation → matrix synthesis (collagen and proteoglycan ground substance) → maturation → remodelling → epithelialisation → wound contraction (by myofibroblasts).

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 granulation tissue which is ready for skin grafting.

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xx xx xx

Stages Of Wound Healing

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

Phases of wound healing Inflammatory phase (lag or substrate or exudative phase) ™™ It begins immediately after formation and lasts for 72 hours. ™™ There is initial arteriolar vasoconstriction, thrombus forma-

tion, 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 inflammation—rubor, calor, tumour, dolor and loss of function.

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.

Note: Coagulation begins at wound haematoma → formation of platelet fibrin thrombus → release of cytokines, PDGF (platelet-derived growth factor), epidermal growth factor (EGF), transforming growth factor-β (TGF-β), 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-α (tumour necrosis factor-α) TGF-β 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—polymorphonuclear cells— neutrophills) 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) ™™ It begins from 3rd day and lasts for 3–6 weeks. There will

Note: (1) Initial angiogenesis (growth of new blood vessels) occurs following release of vascular endothelial cell growth factor (VEGF) by keratinocytes; by release of TNF-α, TGF-β, PDGF, FGF by macrophages. (2) Eventual fibroplasia develops by fibroblast activity with formation of collagen and ground substance/glycosaminoglycans. Type III 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 realign-

ment 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 III collagen is replaced by type I collagen causing maturation of the collagen. Ratio of type I collagen to type III collagen becomes 4:1. ™™ Early extracellular matrix contains fibronectin and collagen type III; 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 III) collagen; granulation tissue contains mainly type III collagen; scar contains both type I and III 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 α 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.

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xx

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-α, interleukin-1, fibroblast growth factor (FGF), proteinases (MMPs—matrix metallo proteinases). Contd...

Factors Affecting Wound Healing

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Local factors

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Infection

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Presence of necrotic tissue and foreign body

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Poor blood supply

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Venous or lymph stasis

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Tissue tension

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Haematoma

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Large defect or poor apposition

xx

Recurrent trauma

xx

X-ray irradiated area

xx

Site of wound, e.g. wound over the joints and back has poor healing

xx

Underlying diseases like osteomyelitis and malig­nancy

xx

Mechanism and type of wound—incised/lacerated/crush/avulsion

xx

Tissue hypoxia locally reduces macrophage and fibroblast activity

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General factors

xx Age,

obesity, smoking, alcohol, stress zinc, copper, manganese xx Vitamin deficiency (Vit C, Vit A) xx Anaemia, hypoxia xx Malignancy xx Uraemia xx Jaundice xx Diabetes, metabolic diseases xx HIV and immunosuppressive diseases xx Steroids and cytotoxic drugs xx Neuropathies of different causes xx Malnutrition,

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

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CHAPTER 1A    General Surgery: Wounds and Wound Healing

be formation of granulation tissue and repair of the wound. Granulation tissue contains fibroblasts, neocapillaries, collagen, fibronectin and hyaluronic acid.

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

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Nutrition: Adequate vitamin, trace elements, fatty acids and proteins are essential for wound healing. Vitamin A deficiency affects monocyte activation, inflammatory phase, collagen synthesis and growth factor actions. Vitamin K deficiency affects synthesis of prothrombin (II), factors VII, IX and X. Vitamin E, being an antioxidant stabilizes the cell membrane. Vitamin C deficiency impairs collagen synthesis, fibroblast proliferation and angiogenesis; increases the capillary fragility and susceptibility for infection. Zinc is an essential cofactor for RNA and DNA polymerase; magnesium is a co-factor for synthesis of proteins and collagen; copper is a required co-factor for cytochrome oxidase, for cytosolic antioxidant superoxide dismutase, and for the optimal cross-linking of collagen; Iron is required for the hydroxylation of proline and lysine. Glutamine is the most abundant amino acid in plasma; is a major source of energy for rapidly proliferating cells such as fibroblasts, lymphocytes, epithelial cells, and macrophages. The serum concentration of glutamine is reduced after major surgery, trauma, and sepsis, and supplementation of this amino acid improves nitrogen balance and diminishes immunosuppression. Glutamine stimulates the inflammatory immune response in early wound healing. Oral glutamine supplementation improves wound breaking strength and of mature collagen. Arginine is a semi-essential amino acid that is required in growth, severe stress, and injury. Arginine modulates immune function, wound healing, hormone secretion, vascular tone, and endothelial function. Arginine is a precursor to proline; supports collagen deposition, angiogenesis, and wound contraction. Under psychological stress situations, the metabolic demand of arginine increases, and its supplementation hastens the wound healing. Serum albumin level less than 2 g/dl causes prolonged inflammatory phase, decreased fibroplasia, neovascularisation and cell synthesis and wound remodeling and hence decreased wound healing. Wounds in patients who remain in catabolic state will not heal. Collagen is the major protein component of connective tissue; it contains mainly glycine, proline, and hydroxyproline. Collagen synthesis requires hydroxylation of lysine and proline, and co-factors such as ferrous iron and vitamin C. Polyunsaturated fatty acids which cannot be synthesized de novo by mammals, consist mainly of omega-6 (found in soybean oil) and omega-3 [(found in fish oil—fatty acids such as eicosapentaenoic acid (EPA) and docosahexaenoic acid (DHA)]. They affect pro-inflammatory cytokine production, cell metabolism, gene expression and angiogenesis; improve the systemic immune function of the host, thus reducing infectious complications. Wound infection: Infection prolongs inflammatory phase, releases toxins and utilizes vital nutrients thereby prevents wound epithelialisation. The β-haemolytic streptococci more than 105 per gram of tissue prevent wounds healing. Formation of biofilms on the wound surface by microorganisms prevents wound healing. Anaemia: Haemoglobin less than 8 g% causes poor oxygenation of tissues preventing healing of the wounds. Hypoxia: Hypoxia prevents fibroblast proliferation and collagen synthesis; it also promotes bacterial invasion into the wound.

Causes of hypoxia are—arterial diseases, cardiac failure, respiratory causes, hypotension, smoking, tobacco, infection, diabetes mellitus and radiation. Radiotherapy: Both external radiotherapy or ionizing radiation cause endarteritis, fibrosis and delay in wound healing. Radiation may itself cause local tissue necrosis, sepsis and hypoxia. Systemic and metabolic causes: Diabetes mellitus affects all stages of wound healing. Cardiac, renal, hepatic, respiratory diseases prevent wound healing. Tissue oedema impairs wound healing. HIV and immunosuppression of varying causes, malignancy leads into poor wound healing. Jaundice interferes with wound healing. Obesity causes hypoperfusion, reduced microcirculation, increased wound tension and hence prevents wound healing. Drugs: Steroids interfere with activation of macrophages, fibroblasts and angiogenesis in the early phase of healing (proliferative). Nonsteroidal anti-inflammatory drugs (NSAIDs) decrease collagen production. Chemotherapeutic agents used in oncology inhibit cellular proliferation, protein synthesis. Alcohol consumption decreases the phagocyte response and pro-inflammatory cytokine release; diminishes host response and thus increasing the infection rate.

Management of wounds Assessment of the wound Wound should be assessed accordingly as minor or major; acute or chronic; number and extent; superficial or deep; major organ injury present or not also to be assessed and anatomical location of the wound(s). Wound is measured either in two dimensions (length and width) or in three dimensions (length, with and depth). Assessment includes detailed history in relation to mode of injury and severity of pain/bleeding; examination in relation to—loss of function, extent of injury, involvement of deeper structures like nerves/vessels/bones/ organs; level of contamination, presence of foreign body in the wound, presence of swelling, viability of the tissues in and around the wound(s).

Wound cleaning It optimizes the wound healing environment and ‘wound bed preparation’. Ideally it should be done with all aseptic precautions; wound is irrigated/washed with warm sterile isotonic normal saline (37°C which is optimum temperature to support cellular activity) using sterile syringe. Wash should be gentle so that already existing wound healing process will not be interfered. Ideal cleaning agents should be of neutral pH and nontoxic. Delipidising agents, antiseptics and alkaline soaps should be avoided. ‘TIME’ (Tissue; Inflammation; Moisture imbalance; Edge of the wound) acronym by Schultz et al, 2003 (European Wound Management Association): It is widely used now as ‘Wound Bed Preparation’. Tissue: assessment and debridement of non-viable or foreign material (including host necrotic tissue, adherent

Note:

Biofilm: A biofilm is a complex microbial community, consisting of bacteria embedded in a protective matrix of sugars and proteins (glycocalyx). Biofilms provide a protective effect for the microorganisms embedded within them, improving their tolerance to the host’s immune system, antimicrobials and environmental stresses. Biofilm communities interact with host tissue resulting in stable attachment, sustainable nutrition and a parasitic relationship. Initial reversible biofilm eventually forms mature biofilm which shed biofilm fragments, bacteria which leads into local and distant invasive spreading infection. Biofilm is addressed by sharp debridement and cleaning. Its reform which is common should be prevented by rational dressings and topical antimicrobials.

Other management Antibiotics, fluids, blood transfusion; electrolyte management; tetanus toxoid injection (to deltoid muscle intramuscular); antitetanus globulin (ATG—250–500 units); critical care—are essential things in major injuries. Wound debridement/wound excision/wound toilet: Liberal excision of devitalised tissues until healthy, bleeding, vascular, tidy wound is created. It is done at regular intervals as staged procedures. Often it requires blood transfusions also. It may be—surgical, sharp, autolytic, enzymatic, larval and mechanical.

B xx xx

xx

Specific treatment

Types of wound suturing

Primary suturing means suturing the wound immedia­tely within 6 hours. It is done in clean incised wounds. Delayed primary suturing means suturing the wound in 48 hours to 10 days. It is done in lacerated wounds. This time is allowed for the oedema to subside. Secondary suturing means suturing the wound in 10–14 days or later. It is done in infected wounds. After the control of infection, once healthy granulation tissue appears, secondary suturing is done.

Minor wounds: Wound is cleaned with saline thoroughly; then non-stick dressing is placed. An incised wound is treated by primary suturing. In lacerated wound, wound edge is excised and then apposed by primary suturing without tension. Haematoma: Ice packs wrapped in cloth is applied and kept for 15 minutes in every 2 hours for 24 hours; compression bandage; elevation of the part; ultrasound of the part and guided aspiration if persists; occasionally haematoma is evacuated. In a crushed or devitalised wound, there will be oedema and tension; all devitalised tissue is removed (wound excision/debridement); oedema is allowed to subside for 2–6 days; then delayed primary suturing is done. If it is a deep devitalised wound, after wound debridement it is allowed to granulate completely. Later if wound edges are closer, secondary suturing is done usually after 10 days using monofilament non-absorbable suture. If the wound is wider, wound is covered with split skin grafting (SSG). Major wounds: Airway should be maintained; Bleeding should be controlled; intravenous fluids should be started. They need proper management in operation theatre under general anaesthesia after initial assessment of the patient and wound. In a wound with tension, fasciotomy is done to prevent development of compartment syndrome. Major vessels are sutured using 6-0 nonabsorbable polypropylene sutures (round body, usually continuous sutures). Nerve with clean cut ends is sutured primarily using fine (6-0 or 7-0) polypropylene suture; if nerve is crushed or cut ends are away or if there is difficulty in identifying cut ends, then marker stitches are placed [different coloured (silk) sutures] at the site and later secondary suturing is done. Internal injuries are managed accordingly—laparotomy/ craniotomy/intercostal tube drainage, etc. Fracture bones are identified and managed accordingly.

A

B

Figs. 1.19A and B: Delayed primary suturing is done once oedema over the wound subsides. It is done as single layer interrupted deep sutures using monofilament polypropylene or polyethylene.

A

B

Figs. 1.20A and B: Secondary suturing is done once wound is healthy after control of wound infection.

When you smile world smiles with you. When you cry, it will be your alone.

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CHAPTER 1A    General Surgery: Wounds and Wound Healing

dressing material, multiple organism related biofilm or slough, exudate and debris) on the surface of the wound. Infection/ inflammation: assessment of the aetiology of each wound, need for topical antiseptic and/or systemic antibiotic use to control infection and management of inappropriate inflammation unrelated to infection. Moisture imbalance: assessment of the aetiology and management of wound exudate. Edge of wound: assessment of non-advancing or undermined wound edges (and state of the surrounding skin).

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SRB's Manual of Surgery

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xx xx xx xx xx xx xx xx xx

Primary suturing should not be done if there is oedema/infection/ devitalised tissues/haematoma Always associated injuries to deeper structures like vessels/nerves or tendons should be looked for before closure of the wound Wound should be widened by extending the incision whenever needed to have proper evaluation of the deeper structures— proper exploration Proper cleaning, asepsis, wound excision/debridement Any foreign body in the wound should be removed Skin closure if it is possible to be done without tension Skin cover by graft/flap—immediate or delayed Untidy wound should be made tidy and clean before suturing Proper aseptic precautions should be undertaken Antibiotics/analgesics are needed Sutured wound should be inspected in 48 hours Sutures are removed after 7 days

B xx xx

xx xx

Principles of wound suturing

hours. Progressive, persistent severe pain which is aggravated by passive muscle stretching is the diagnostic sign. Tense tender regional lymph node is typical. Normal pulse will be felt usually in compartment syndrome; but may become absent if there is associated arterial injury. A catheter is placed in the muscle compartment connected to a pressure monitor and compartment pressure is measured; if it is more than 30 mm Hg, fasciotomy is indicated. Fasciotomy includes incising skin, fat and fascia covering the muscle longitudinally with adequate length to allow the muscle to bulge out. Leg is decompressed by two longitudinal incisions lateral to the subcutaneous margin of the tibia allowing the decompression of posterior, peroneal and anterior compartments.

Remember

Wound toilet is washing the wound thoroughly using normal saline—ideal Wound debridement (french-letting loose) is allowing content to come out by release incisions or facio­tomies. But commonly debridement is used for wound excision Wound excision is actually correct terminology for excision of devitalised tissues once or serially Radical wound excision is (pseudotumour approach) is excising entire devitalised tissues leaving tissues with visible bleeding from all layers

Fig. 1.21: Necrotizing fasciitis with extensive skin involvement which requires adequate wound excision and eventual skin coverage.

COMPARTMENT SYNDROME Compartment syndrome is a special entity; common in leg, forearm, thigh and arm; is a syndrome due to increased intracompartmental pressure within a limited space area. Causes are: narrowed space due to tight dressings/plaster cast, lying on one limb in comatous patient; increased content within the compartment due to trauma like fractures, oedema, ischaemic injury, haematoma, positioning after trauma, burn injury, etc.; high pressure injection injuries like gun injury, oilbased material injury, extravasation of chemotherapeutic drugs; snake bite. Features are: It compromises circulation and function mainly of muscles and nerves. It often maintains the normal colour and temperature of the fingers and distal pulses may not be obliterated in spite of severe muscle ischaemia. Muscle ischaemia more than 4 hours causes muscle death and myoglobinuria. Irreversible nerve damage develops if ischaemia persists for 8

Fig. 1.22: Fasciotomy for compartment syndrome should be longitudinal, deep and lengthy and should decompress the compartment to expose the underlying muscle. It should be done early.

It is common in calf and forearm. Closed injuries cause haematoma leading to increased pressure. It is often associated with fracture of the underlying bone which in turn compresses the major vessel further aggravating the ischaemia causing pallor, pulseless­ness, pain, paraesthesia, diffuse swelling and cold limb. If allowed to progress it may eventually lead to gangrene or chronic ischaemic contracture with deformed, disabled limb. Muscle necrosis releases myoglobulin which is excreted in the urine, damages the kidneys leading into renal failure.

B xx xx xx xx xx

Problems with the compartment syndrome

Infection, septicaemia and abscess formation Renal failure Gangrene of the limb Chronic ischaemic contracture Disabled limb, Volkmann's ischaemic contracture

Note: Affected muscle when passively stretched worsens the pain—the most reliable clinical sign.

Treatment ™™ Compartment pressure will be persistently more than 30 mm

Hg. It can be measured by placing a fine catheter in the compartment and using a pressure monitor. This is an indication for fasciotomy. Adequate lengthy incision involving skin, fat and deep fascia should be done until underneath muscle bulges out properly. Multiple incisions should be made if needed. Separate incision in each compartment should be done.

Fasciotomy done in forearm anterior compartment is a specific method. Carpal tunnel should be released by cutting flexor retinaculum. Incision begins at the junction of the thenar and hypothenar area; extends proximally initially transverse across flexion crease of the wrist at the ulnar border; then across forearm towards radial side of forearm; then in proximal forearm towards medial side creating convex flap towards lateral side. In the elbow it crosses along the medial border to reach the arm where it runs in arm along the medial part of the anterior arm. Injury to major nerves, palmar cutaneous branch of median nerve should be avoided while placing the incision. Incision should be deepened by cutting the deep fascia along the entire length of the incision. Dorsal fasciotomy should be added by placing longitudinal lengthy incision in the midline. Two longitudinal incisions on the dorsum of the hand also should be made.

CRUSH SYNDROME ™™ It is due to crushing of muscles causing extravasation of

blood and release of myohaemoglobin into the circulation leading to acute tubular necrosis and acute renal failure. ™™ Causes: Earthquakes, road traffic accidents; Mining and industrial accidents; Air crash; Tourniquet. ™™ Initially tension increases in the muscle compart­m ent commonly in the limb, which itself impedes the circulation and increases the ischaemic damage. In 3 days, urine becomes discoloured and scanty, patient becomes restless, apathy and delirious with onset of uraemia. Crush syndrome is often lifethreatening. Injury is much worser than initial look.

B xx xx xx xx xx

Effects of crush syndrome

Renal failure Toxaemia Septicaemia Disability with extensive tissue loss Gas gangrene

Treatment ™™ Tension in the muscle compartment is relieved by placing

Fig. 1.23: Incision for fasciotomy in upper limb begins at flexor retinaculum extending into the forearm with a convex flap towards radial side eventually leading towards medial epicondyle of the elbow joint.

CRUSH INJURY Crush injury is one where a part of the body is being squeezed/ compressed between two high force or pressure systems. It causes extensive lacerations, bruising, compartment syndrome, crush syndrome, fractures, haemorrhage, etc. with extensive tissue destruction and devitalisation. Renal failure, hypovolaemic shock and sepsis are the most dreaded problems in crush injuries.

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multiple parallel deep incisions in the limb so as to prevent further damage—Fasciotomy. ™™ Rheomacrodex, or mannitol is given to improve the urine output by improving the renal function. ™™ Alkalisation of urine is done by giving sodium citrate or sodium bicarbonate. It increases the solubility of acid haematin in the urine and so promotes its excretion. Urinary pH should be above 6.5 until urine does not show any myoglobin. Mannitolalkaline diuresis should be 8 litre/day. ™™ Initial aggressive volume load using saline about 1-1.5 litres/ hour is ideal in these patients. ™™ Haemodialysis is done sometimes as a life-saving procedure. ™™ Other measures: Catheterization; Oxygen therapy; Antibiotics; Blood transfusion; Correction of severe hyperkalaemia.

B

Figs. 1.24A and B: Traumatic crush injury pelvis exposing testis and iliac vessels. Patient underwent hemipelvectomy.

Success is getting what you want. Happiness is liking what you get.

CHAPTER 1A    General Surgery: Wounds and Wound Healing

™™ Antibiotics. ™™ Catheterisation. ™™ Mannitol or diuretics to cause diuresis, so as to flush the kidney. ™™ Fresh blood transfusion. ™™ Hyperbaric oxygen.

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Note: Doing fasciotomy several days after crush injury may not be safe as it may lead to sudden release of myoglobulin causing myoglobulinuria and renal failure.

DEGLOVING INJURIES ™™ It occurs due to shearing force between tissue planes as

traction—avulsion injury. It usually occurs between subcutaneous tissue and deep fascia or between muscle and bone. It can be localised or circumferential. ™™ Avulsion injury strips off the superficial tissues exposing the neurovascular bundle, muscles, tendons, and bone. Degloving injury can be open or closed.

B

Types of scar

A mature scar is paler, acellular, softer, flat, with reduced blood vessels and fibroblasts, without itching (diminishes). xx An atrophic scar is pale, flat and stretched. xx A hypertrophic scar is excess scar but will not extend beyond the margin of the scar of the original wound; there is prolonged inflammatory phase of wound healing. It develops in 1 to 3 months after trauma. It improves spontaneously. xx Keloid is persistent excessive growth of the scar beyond its margin into the adjacent skin; occurs in a triangular area between two shoulder points and xiphisternum. It develops 3 months to years after the trauma; progressive. Presternal area is the commonest site. Note: xx A linear scar is supple, thin, soft occurs after healing by primary intention. xx A wide, irregular firm scar occurs after healing by secondary intention or when there is infection. xx

Fig. 1.25: Degloving injury—finger; note the avulsed skin. ™™ It can be in one plane or multiple planes. ™™ It is commonly observed in machinery injuries or major road

traffic accidents. It is much more extensive than of on initial presentation. ™™ Under anaesthesia fluoroscein is injected intravenously and viable skin is visible as fluorescent yellowish—green colour under ultraviolet light. As injection of fluoroscein is not fully safer, serial excision is better to look for dermal punctate bleeding. ™™ It needs examination under general anaesthesia, wound excision/radical excision, flap coverage, microflap surgeries, skin grafting, with proper asepsis, and blood transfusion as there is significant blood loss in these injuries.

SCAR Scar is defined as formation of final relaxed, randomly arrayed collagen bundles (Type I) with formation of matured scar. Initially immature scar is formed during remodeling phase; this scar is disorganized and contains type III collagen. Such scar is raised, itchy, hard and pink in colour. Over the span of 12 months scar gets matured fully wherein disorganised collagen gets aligned along the stress lines and there is formation of more type I collagen. This matured scar is soft, supple, pale and flat without any itch. Hypertrophic scar and keloid persists to have more type III collagen than type I collagen unlike the matured scar.

Fig. 1.26: Diagrammatic representation of linear, hypertrophic and keloid scar.

KELOID: ‘Like a claw’ Keloid is the excessive abnormally stretched (type III thick) collagen tissue bundles arranged with aligning in the same plane as the epidermis but extends beyond the original scar margin which continues to grow for long period. ™™ Keloid is common in blacks. Common in females. ™™ Genetically predisposed. Often familial. Very rare in Caucasians. ™™ There is defect in maturation and stabilization of collagen fibrils. Normal collagen bundles are absent. ™™ Keloid continues to grow even after 6 months, may be for many years. It extends into adjacent normal skin. It is brownish black/ pinkish black (due to vascularity) in colour, painful, tender and sometimes hyperaesthetic; spreads and causes itching. ™™ Keloid may be associated with Ehlers-Danlos syndrome or scleroderma. ™™ When keloid occurs following an unnoticed trauma without scar formation is called as spontaneous keloid, commonly seen in dark skinned people.

13

™™ Some keloids occasionally become non-progressive after

initial growth.

™™ Pathologically keloid contains proliferating immature fibro-

Note: Excision and primary suturing has got high recurrence rate; hence it is not usually practiced.

Recurrence rate is very high—more than 50%.

B

A

Figs. 1.27A and B: Keloid over the sternum (butterfly shaped; commonest site) and upper part of the arm near shoulder—common sites of occurrence.

Fig. 1.29: Keloid at laparoscopic port (cholecystectomy) sites.

HYPERTROPHIC SCAR Hypertrophic scar is excessive formation of abnormal scar tissue containing type III collagen (thin) which is raised, often vascular but confined within the margin of the original wound; usually its growth stops in 6 months and often regresses spontaneously. ™™ Occurs anywhere in the body. ™™ Not genetically predisposed. Not familial. ™™ Growth usually limits up to 6 months. ™™ It is limited to the scar tissue only. It will not extend to normal skin. ™™ Spontaneous improvement with time occurs commonly. ™™ It is pale brown in colour, not painful, nontender. ™™ Often self-limiting also. It responds very well for steroid injection. ™™ Recurrence is uncommon. ™™ It is common in wounds crossing tension lines, deep dermal burns, wounds healed by secondary intention.

Complications ™™ Often this scar breaks repeatedly and causes infection, pain. ™™ After repeated breakdown it may turn into Marjolin’s ulcer.

Treatment ™™ It is controlled by pressure garments or often revision exci-

sion of scar and closure, if required with skin graft.

™™ Triamcinolone injection is the 2nd line of therapy for hyper-

trophic scar.

A

B

Figs. 1.28A and B: Keloid in the ear lobule and chest wall near shoulder.

A

Fig. 1.30A

It is not how much we have, but how we enjoy, that makes us happy.

CHAPTER 1A    General Surgery: Wounds and Wound Healing

blasts, proliferating immature blood vessels and type III thick collagen stroma. Site: Common over the sternum. Other sites are upper arm, chest wall, lower neck in front. Differential diagnosis: Hypertrophic scar. Treatment: Controversial. ™™ Steroid injection—intrakeloidal triamcinolone, is injected at regular intervals, may be once in 7–10 days, of 6–8 injections. Triamcinolone reduces the fibroblast proliferation and collagen synthesis; it is first line of therapy for keloid. ™™ Steroid injection—excision—steroid injection. ™™ Methotrexate, vitamin A and C therapy into the keloid. ™™ Silicone gel sheeting; topical retinoids. ™™ Laser therapy—Nd-YAG laser. ™™ Vitamin E/palm oil massage. ™™ Intralesional excision retaining the scar margin may prevent recurrence. It is ideal and better than just excision. ™™ Excision and irradiation or irradiation alone. ™™ Excision and skin grafting may be done.

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14

T

Difference Wloid and hypertrophic scar Keloid

Hypertrophic scar

a.

Genetic predisposition

Yes

No

b.

Site of occurrence

Chest wall, upper arm, lower neck, ear

Anywhere in the body, common in flexor surfaces

c.

Growth

Continues to grow without time limit

Growth limits for 6 months

Extends to normal skin

Limited to scar tissue only

d.

Treatment

Poor response

Good response to steroids

e.

Recurrence

Very high

Is uncommon

f.

Collagen synthesis

20 times more than normal skin (Type III thick)

3-6 times more than normal skin (Type III fine collagen)

g.

Relation of size of injury No relation. Small healed scar can form large and lesion keloid

Related to size of injury and duration of healing

h.

Age

Adolescents, middle age

Children

i.

Sex

Common in females

Equal in both

j.

Race

More in blacks (15 times)

No racial relation

k.

Structure

Thick collagen with increased epidermal hyaluronic acid

Fine collagen with increased alpha actin

l.

Features

Vascular, tender, itching

Not vascular, nontender, no itching

m.

Natural history

Progressive

Shows regression

n.

Problems

Hyperaesthesia, ulceration

Not much

B

Fig. 1.30B Figs. 1.30A and B: Different contractures in hypertrophic scars over forearm, finger and neck—in old burns patients.

Fig. 1.31: Hypertrophied scar in the abdominal healed wound.

PROBLE MS WITH WOUND HEALING ™™ Wound infection is common in devitalized deep difficult

wounds. Diabetes, immunosuppression, cytotoxic drugs,

Fig. 1.32: ‘Z’ plasty is very useful method to release small contractures.

anaemia, malnutrition, malignancy increases the chances of wound infection. ™™ Wound dehiscence is common in all above said adverse factors. Wound suddenly gives away with pain causing copious serosanguineous discharge. After laparotomy when done specially as an emergency in trauma, acute abdomen and also in malignancy, abdominal closed wound may burst in 5–7 days. Usually all layers of abdomen give away causing discharge, occasionally bowel will also extrude out. It needs emergency closure of the abdominal wound using specialized sutures or retention sutures. ™™ Hypertrophic scar or keloid formation due to altered collagen synthesis in the wound healing process. Collagen synthesis is increased by 3–6 times in hypertrophic scar and 20 times in keloid. ™™ Deeper wounds will cause specified problems like paraesthesia, ischaemia, paralysis, etc.

B. Ulcer

C hapter Outline ·· Ulcer ·· Granulation Tissue ·· Investigations for an Ulcer ·· Management of an Ulcer ·· Traumatic Ulcer ·· Trophic Ulcer ·· Ulcer due to Chilblains ·· Ulcer due to Frostbite ·· Martorell’s Ulcer ·· Arterial/ischaemic Ulcer ·· Bairnsdale Ulcer ·· Carcinomatous Ulcer ·· Rodent Ulcer

·· ·· ·· ·· ·· ·· ·· ·· ·· ··

Melanotic Ulcer Diabetic Ulcer Meleney’s Ulcer Lupus Vulgaris Tuberculous Ulcer Bazin’s disease Tropical Ulcer Venous Ulcer Syphilitic Ulcer Soft Chancre/Soft Sore/ Ducrey’s Ulcer/ Chancroid/Bubo ·· Climatic bubo/Tropical bubo

Sloping edge. It is seen in a healing ulcer.  Its inner part is red because of red, healthy granulation tissue.   Its outer part is white due to scar/fibrous tissue.   Its middle part is blue due to epithelial prolife­ration. Undermined edge is seen in a tuberculous ulcer. Disease process advances in deeper plane (in subcutaneous tissue) whereas (skin) epidermis proliferates inwards. Punched out edge is seen in a gummatous (syphilitic) ulcer and trophic ulcer. It is due to endarteritis. Raised and beaded edge (pearly white) is seen in a rodent ulcer (BCC). Beads are due to proliferating active cells. Everted edge (rolled out edge): It is seen in a carcino­matous ulcer due to spill of the proliferating malignant tissues over the normal skin. c. Floor: It is the one which is seen. Floor may contain discharge, granulation tissue or slough. d. Base: Base is the one on which ulcer rests. It may be bone or soft tissue.

Ulcer Definition An ulcer is a break in the continuity of the covering epithelium, either skin or mucous membrane due to molecular death.

Fig. 1.34: Parts of an ulcer.

A

B

Figs. 1.33A and B: (A) Nonhealing ulcer foot; (B) Malignant (SCC) ulcer leg.

Parts of an Ulcer a. Margin: It may be regular or irregular. It may be rounded or oval. b. Edge: Edge is the one which connects floor of the ulcer to the margin. Different edges are:

Fig. 1.35: Ulcer edges.

Induration of an Ulcer Induration is a clinical palpatory sign which means there is a specific type of hardness in the diseased tissue. It is obvious in well-differentiated carcinomas. It is better felt in squamous cell

Happiness is never found until we have the grace to stop looking for it.

SRB's Manual of Surgery

16

carcinoma. It is also observed in long standing ulcer with underlying fibrosis. It is absent or less in poorly differentiated carcinomas and malignant melanoma. Less indurated carcinoma is more aggressive. Specific types of indurations are observed in venous diseases and chronic deep venous thrombosis. Brawny induration is a feature of an abscess. Induration is felt at edge, base and surrounding area of an ulcer. Induration at surrounding area signifies the extent of disease (tumour). Outermost part of the indurated area is taken as the point from where clearance of wide excision is planned.

3. Non-healing ulcer: It may be a chronic ulcer depending on the cause of the ulcer; here edge will be depending on the cause—punched out (trophic), undermined (tuberculous), rolled out (carcinomatous ulcer), beaded (rodent ulcer); floor contains unhealthy granulation tissue and slough, and serosanguineous/purulent/bloody discharge; regional draining lymph nodes may be enlarged but non-tender.

Classifications Classification I (clinical) 1. Spreading ulcer: Here edge is inflamed, irregular and oedematous. It is an acute painful ulcer; floor does not contain healthy granulation tissue (or granulation tissue is absent) but with profuse purulent discharge and slough; surrounding area is red and edematous. Regional (draining) lymph nodes are enlarged and tender due to inflammation. There will be associated fever, pain, impairment of functions with local tissue destruction and with little evidence of regeneration.

Fig. 1.36: Spreading ulcer copious purulent discharge with slough.

2. Healing ulcer: Edge is sloping with healthy pink/red healthy granulation tissue with scanty/minimal serous discharge in the floor; slough is absent; regional lymph nodes may or may not be enlarged but when enlarged always non-tender. Surrounding area does not show any signs of inflammation or induration; base is not indurated. Three zones are observed in healing ulcer. Innermost red zone of healthy granulation tissue; middle bluish zone of growing epithelium; outer whitish zone of fibrosis and scar formation.

Fig. 1.38: Non-healing ulcer with pale unhealthy granulation tissue with slough.

4. Callous (stationary) ulcer: It is callousness towards healing; word “callous” means—insensitive and cruel; and also it means—hard skinned. It is also a chronic non-healing ulcer; floor contains pale unhealthy, flabby, whitish yellow granulation tissue and thin scanty serous discharge or rarely copious serosanguinous discharge, with indurated nontender edge; base is indurated, nontender and often fixed. Ulcer does not show any tendency to heal. It lasts for many months to years. Tissue destruction is more with absence of or only minimal regeneration. Induration and pigmentation may be seen in the surrounding area. There is no/less discharge. Regional lymph nodes may be enlarged; are firm/hard and nontender.

Fig. 1.39: Callous ulcer without any sign of healing and, without any granulation tissue.

Classification II (Based on duration) 1. Acute ulcer—duration is less than 2 weeks. 2. Chronic ulcer—duration is more than 2 weeks (long).

Classification III (Pathological) Fig. 1.37: Healing ulcer with healthy granulation tissue in the floor.

1. Specific ulcers: tuberculous; syphillitic; actinomycosis and Meleney’s ulcer.

17

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

B

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

A

Figs. 1.44A and B: (A) Malignant proliferative ulcer; (B) Melanotic 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: ¾¾ ¾¾ ¾¾ ¾¾ ¾¾

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.

B

¾¾ ¾¾ ¾¾ ¾¾ ¾¾ ¾¾

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.

CHAPTER 1B    General Surgery: Ulcer

A

SRB's Manual of Surgery

18

¾¾

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

Wagner’s Grading/Classification of Ulcer

Fig. 1.45: Ischaemic ulcer foot. Middle three toes are already amputated because of gangrene.

Grade 0  –  Preulcerative lesion/healed ulcer Grade 1  –  Superficial ulcer Grade 2  – Ulcer deeper to subcutaneous tissue exposing soft tissues or bone Grade 3  –  Abscess formation underneath/osteomyelitis Grade 4  –  Gangrene of part of the tissues/limb/foot Grade 5  –  Gangrene of entire one area/foot

B

Stages of ulcer healing

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 occurs. Different discharges in an ulcer (as well as from a sinus) 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

B

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.

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.

Types ™™ Healthy granulation tissue: It occurs in a healing ulcer. It

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

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

19

™™ Study of discharge: Culture and sensitivity, AFB study,

cytology.

™™ 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 Volkmann’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.

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

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 g% and serum albumin is 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: ¾¾ Loss of tendon reflexes (most common). ¾¾ Neuromuscular depression; Flaccid quadriplegia. ¾¾ Respiratory paralysis; Somnolence; Hypotension.

You cannot harm a bandaged wound.— Croatian Proverb

89

CHAPTER 1F    General Surgery: Electrolyte and Nutrition

oedema. ™™ Investigation: Serum electrolytes, plasma and urine osmolality, renal function tests, haematocrit. ™™ Management: ¾¾ Restriction of saline and sodium. Treatment of pulmonary oedema. ¾¾ 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.

90

HYPOMAGNESAEMIA

B

SRB's Manual of Surgery

™™ Serum magnesium than crystalloids). They improve cardiac output but not oxygen carrying capacity. Colloids should maintain oncotic pressure equal to that of plasma; should be stable and inert; non-toxic, non-antigenic, non-pyrogenic; should be easily sterilisable; should not interfere with blood grouping.

Colloids commonly used Colloid

content

Indications

Problems

Albumin (Heat treated human albumin) It maintains 75% of plasma oncotic pressure. It is the vehicle to transport many low molecular substances including drugs

• 5% human albumin (50 grams/L) has colloid osmotic pressure of 20 mm of Hg (that of plasma pressure); its effects last for 18 hours. It increases the volume 1:1 • 25% human albumin (250 grams/L) has got colloid osmotic pressure of 70 mm Hg; expands the intravascular volume 5 times more by shifting the fluid from extravascular (interstitial) space to intravascular space; should not be used in hypovolaemia • Dextran was first discovered by Louis Pasteur in wine • It is used to expand intravascular volume • Dextran 70 has got molecular weight of 70,000. Its excretion through kidney is poorer and so remains in the blood for weeks. It is used as 6% solution

• It is used—when rapid plasma volume expansion is needed like burns in correcting hypovolaemia sometimes; in plasmapheresis as exchange fluid • 500 ml of 5% albumin is infused at a rate of 2 ml per minute

• It is contraindicated in cardiac failure, anaemia • It can cause allergic reactions, nausea, febrile reactions

• It is used as volume expander in hypovolaemia. It increases the blood sugar also • It is also used as antithrombotic agent. Dextran is a plasminogen activator; inhibits erythrocyte aggregation and platelet adhesiveness

• It causes electrolyte disturbances and hyponatraemia • It causes acute renal failure by direct toxicity to glomerulus and tubules and by intraluminal hyperviscosity

Dextran Bacteria (Leuconostoc mesenteroides and streptococcus mutans) incubated in sucrose media produces complex branched glucan polymers as dextrans

Contd...

95

Contd... • It improves microcirculation and blood flow • Dextran 40 is commonly used

Haemaccel 500 ml bottle with active ingredient polygeline

• 1000 ml of haemaccel contains polypeptides of degraded gelatin (origin—bovine bone; cross linked via urea bridges), 35 grams (equivalent of 6.3 gram of nitrogen) • It also contains sodium and chloride of 145 mmol (0.85 gram); calcium 6.25 mmol (0.25 gram); potassium 5.1 mmol (0.20 gram); traces of phosphate, sulphate and anionic polypeptides. Its molecular weight is 30,000 with 1.7 viscosity • Haemaccel should be stored in 2–25°C • Haemaccel does not induce antibody formation

Hetastarch (hydroxyethyl starch) It is nonionic starch derivative, which is ethoxylated amylopectins which is a plasma volume expander

• It is a synthetic colloid available as 6% solution in 0.9% normal saline (30 gram hetastarch in 500 ml of isotonic saline). It is administered IV only using infusion pump; 20 ml/kg/hour; it is stored in room temperature. It is enzymatically degraded and causes rise in serum amylase level • Total dose per day should be 1500 ml only

• It is used for rapid volume replacement in circulatory collapse like shock, burns and trauma. It is infused rapidly at a rate of 125 drops/minute. H2 blocker like cimetidine or ranitidine should be given along with haemaccel as it releases histamine • It improves the plasma volume rapidly and remains like that for 5 hours • It will not interfere with coagulation, blood grouping and cross matching • It is used also in heart lung machine • Haemaccel can be mixed with other IV fluids and drugs • Haemaccel can be infused up to 2000 ml • It is used in hypovolaemia—shock, burns, trauma. • It is also used in leukapheresis • It is nonantigenic; does not interfere with blood grouping • It shows greater plasma volume expansion for longer period compared to 5% albumin

Pentastarch It is a subgroup of hetastarch with five hydroxyethyl groups with 50% esterification. It is a low molecular weight derivative; it is more effective plasma volume expander

It is available as 3%, 6% and 10% in normal saline

• It is used in hypovolaemia • It is useful in cardiac surgeries

Life will give you back everything you have given to it.

• It should be used carefully in diabetic patients • It can cause pulmonary and or cerebral oedema due to hyperosmolarity • It can cause anaphylaxis (being a potent antigenic) • It can interfere with blood grouping and cross matching • It prolongs the bleeding time and so may precipitate bleeding Rapid histamine release and anaphylactoid reaction

• Anaphylactoid reactions can occur • Renal impairment should be observed • It has no O2 carrying capacity; so should not allow haematocrit to fall below 30% • Impairment of coagulation is possible

CHAPTER 1F    General Surgery: Electrolyte and Nutrition

• Dextran 40 has got molecular weight of 40, 000. It is used as 10% solution; its action is faster but shorter than dextan 70 due to faster excretion through kidney compared to dextran 70

96

Methods of Enteral Feeding

NUTRITION

SRB's Manual of Surgery

Principles of Nutrition ™™ 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. ™™ Immunomodulators 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. ¾¾ By mouth: Requires—common sense, cleanliness, compassion. ¾¾ 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 ml/hours. A time gap of 5 hours in the night is given to allow gastric pH to return to normal. Note: Problems with tube feeding are: Blockage; nausea and vomiting, aspiration; hyperosmolarity; diarrhoea; tube discomfort; Cholestasis ¾¾ 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.

Note: • 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 30–40%; Protein 10–15%. • Caloric values: Carbohydrate 4 kcal/g; Protein 4 kcal/g; Fat 9 kcal/g.

Indications for Nutritional Support ™™ Preoperative nutritional depletion. ™™ Postoperative complications: Sepsis, ileus, fistula. ™™ Intestinal fistula: High type wherein output is more than 500

ml/day. 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.

B

Assessment

xx

Body weight

xx

Mid-arm circumference

xx

Triceps skin fold thickness

xx

Serum albumin

xx

Lymphocyte count

Nutritional requirements: 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 xx xx xx xx

Complications of enteral feeding

Aspiration, wound infection and leak Diarrhoea due to rapid feeding or hyperosmolarity Hyperglycaemia, hypokalaemia Refeeding syndrome due to severe hypokalaemia and hypophosphataemia

™™ Advantages of enteral nutrition:

Enteral nutrition preserves mucosal protein, digestive enzymes, IgA secretion; prevents mucosal atrophy and bacterial translocation. ¾¾ 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. ¾¾ It has got less serious complications. It is cost-effective. ¾¾ It supplies glutamine and short chained fatty acids to gut. ™™ Contraindications of enteral nutrition ¾¾ Intestinal obstruction, GI bleed, paralytic ileus, severe diarrhoea, high output fistula. ¾¾ Low cardiac output, haemodynamically unstable patient. ¾¾

¾¾ ¾¾

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

™™ 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. A

B

C

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.

4. Janeway’s mucus lined permanent gastrostomy by creating mucosal entire thickness tunnel in the stomach wall. ™™ Problems in gastrostomy tube: ¾¾ Leak from gastrostomy site—gastric fistula. ¾¾ Trauma to other organs like colon, spleen. ¾¾ Infection, aspiration and pneumonia. ¾¾ Diarrhoea is common (30%); bloating, abdominal cramps. ¾¾ Displacement, blockage of the tube.

JEJUNOSTOMY A

B

™™ Jejunostomy for enteral nutrition is becoming more popular

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

because of—its comfort, easy to do, can be kept for long time, lesser complication than gastrostomy.

™™ Types ¾¾ ¾¾ ¾¾

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 tube (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.

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CHAPTER 1F    General Surgery: Electrolyte and Nutrition

GASTROSTOMY

2. Kader-Senn temporary gastrostomy: It is serosal lined temporary type similar to Stamm’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. 1.198A and B).

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A

B

Figs. 1.195A and B: Percutaneous endoscopic gastrostomy (PEG). Guidewire passed through a trocar in the abdominal wall into the stomach is pulled out across the oesophagus and mouth under visualisation using gastroscope. Gastrostomy catheter (tube) is passed along the guidewire and pushed downwards across mouth and oesophagus to reach the stomach. It is further pushed along the abdominal wall through the trocar. Gastrostomy tube is pulled out through abdominal wall and fixed. Trocar is removed. Placement of tip of gastrostomy tube can be confirmed by doing gastroscope. ™™ Indications are same as gastrostomy.

Contd...

™™ Types ¾¾ ¾¾

Witzel jejunostomy: Site of placing jejunostomy is 30 cm from duodenojejunal junction. Needle jejunostomy using catheter of small gauge.

xx xx xx xx xx xx

TOTAL PARENTERAL NUTRITION (TPN) ™™ All nutritional requirements are given only through intra-

venous route, not through gastrointestinal tract. It can be through a central catheter through the subclavian/internal jugular vein where the tip of venous catheter is at distal part of superior vena cava. ™™ It can also be a peripheral (Peripheral parenteral nutrition/ PPN) through a peripherally inserted central venous catheter (PICC) or through a formal peripheral venous line.

B xx xx xx

Indications

Failure or contraindication for any enteral nutri­tion for 7–10 days High output abdominal fistulas, duodenal, biliary, pancreatic fistulas Major abdominal surgeries of liver, pancreas, biliary, colonic Contd...

Septicaemia; multiple trauma, short bowel syndrome Severe pancreatitis, bowel ischaemia, peritonitis, ileus Massive GI bleeding, unstable haemodynamically High risk of aspiration Hyperemesis gravidarum Multiorgan failure, head injury, severe burns

™™ About 5% of hospital admissions require TPN.

Technique ™™ Using a needle and guide wire a Subclavian vein catheter is

passed just below the clavicle and fixed securely to the skin.

™™ TPN is given through central vein and not through a periph-

eral vein.

™™ Peripherally inserted central catheter (PICC) is also com­monly

used (PPN).

Goals, Factors and Assessment in TPN ™™ To decrease adverse effects of catabolism; to increase protein

synthesis, to reduce protein breakdown, to prevent weight loss.

™™ To support ongoing metabolism. ™™ To improve immune function, cardiac and respiratory func-

tion.

muscles.

™™ To maintain acid, base and electrolyte metabolism. ™™ Age, premorbid state, muscle mass, weight, serum albumin

should be assessed.

™™ Underlying disease, its severity, therapies for the disease, GI

function should be assessed. ™™ Fluid requirement is assessed by—1500 ml for 20 kg weight + 20 ml/kg for additional weight. ™™ Energy needed is calculated by calculating resting energy expenditure (REE). ¾¾ By simple calculation: REE in kcal/day = 25 × weight in kg. ¾¾ Harris Benedict equation: REE in men = 66 + (13.7 × weight in kg) + (5 × height in cm) – (6.7 × age in years). In women = 655 + (9.6 × weight) + (1.8 × height) – (4.7 × age). Activity/disease/thermal factors are also added. ¾¾ Indirect calorimetry: It is more accurate method done using special instrument. REE: = (3.9 × VO2) + (1.1 × VCO2) – 61.

Components used in TPN/PPN ™™ Carbohydrates: Dextrose is less costly (1 gram dextrose

3.4 kcal); can be used in 50–70% concentration during PN. It supplies calories, stimulates insulin release and glucose oxidation, prevents muscle protein breakdown, has got nitrogen sparing ability. Problems of carbohydrate/dextrose are—low calorie value compared to fat, requires large fluid volume to infuse, hyperglycaemia, causes more CO2 production, because of high osmolality it causes thrombophlebitis in 10% or above concentration. Rate of administration of dextrose is 5 mg/kg/min. ™™ Fat: Fat gives high calorie (1 gram—9 kcal), essential fatty acids. It is given as emulsion containing long chain triglycerides. It contains soyabean/sunflower oil with egg yolk phospholipids (emulsifying factor), glycerin (isotonic). Fat has got low osmolality (260 mosm/L); it is available as 10%, 20%, 30% emulsions. Advantages of fat in PN are—high calorie, prevents hyperglycaemia, glucose and nitrogen sparing, less CO2 production, less insulin production; it prevents essential fatty acid deficiency (for this purpose 3 days a week dose is given), reduces thrombophlebitis. Problems of lipids in PN are—hypertriglyceridaemia, sepsis, fat embolism, fat overload, hepatic dysfunction, pancreatitis, delayed gastric emptying. Lipid emulsions are avoided in hyperlipidaemia, anaemia, acidosis, obesity. Lipid emulsion is a good culture media for bacteria and fungi; so care should be taken to prevent sepsis. Triglyceride level should be monitored weekly; if it is more than 400 mg%, infusion is discontinued. Mixture of long and medium chain fatty acids is better tolerated and efficient. ™™ Amino acids: They are source of proteins. Calorie value of amino acid is 4 kcal/gram. 6.25 gram protein has 1 gram

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Monitoring the patient

By body weight, fluid balance, blood glucose, electrolytes, blood urea, LFT, serum calcium, magnesium, phosphate should be done at regular intervals. A weight gain more than one kg/day signifies fluid overload.

Complications ™™ Technical: Air embolism; Pneumothorax; Bleeding; Catheter

displacement, sepsis, blockage; Infection, thrombosis. ™™ Biochemical

Electrolyte imbalance: Hyponatraemia, hypokalaemia, hypophosphataemia. ¾¾ Hyp­­­erosmolarity; Hyperglycaemia—common. ¾¾ Dehydration; Azotaemia ¾¾ Altered immunological and reticuloendothelial function. ™™ Others: Dermatitis; Anaemia and increased capillary permeability; Cholestatic jaundice (It is common); Severe hepatic steatosis; Metabolic acidosis; Candida infection (candidiasis), staphylococcal infection (10–15%). ™™ Contraindications: Cardiac failure; Blood dyscrasias; Altered fat metabolism. ¾¾

Note: Anabolic steroid durabolin 25 mg IM weekly is given to improve nitrogen balance.

B xx xx xx

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Home parenteral nutrition

It is becoming popular It is commonly used in western countries It is indicated in short bowel syndrome or any other conditions wherein enteral feeding is not possible but patient can be sent home with provision for home parenteral nutrition Patient himself uses the total parenteral nutrition (TPN) fluids as advised at home. He will be with TPN catheter Patient should attend TPN clinic weekly for follow-up or immediately whenever complications arise Patient will be comfortable psychologically and often can attend his job also

The secret of happiness is to admire without desiring.

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CHAPTER 1F    General Surgery: Electrolyte and Nutrition

™™ To maintain glycogen reserve in cardiac and respiratory

nitrogen. In PN 20% of energy comes from amino acids; rest from dextrose and fat. Daily protein need is 0.8–1.5 gram/kg. Protein supplement should be less in patients with CRF and hepatic encephalopathy. Its need is more in burns, trauma, enteropathy, sepsis. Protein supplement should not exceed 1.7 gram/kg/day; if so will cause raised urea production. Uses of amino acids in PN—in protein anabolism; prevents catabolism. Proper monitoring by doing BUN or ammonia level is essential during amino acid therapy. ™™ Vitamins, electrolytes, trace elements and minerals: Electrolytes like sodium, potassium, magnesium, phosphate, calcium; fat-soluble vitamins like A, D, E, K; water-soluble vitamins; trace elements like chromium, copper, iodine, iron, manganese, selenium, zinc are all used in PN.

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Refeeding Syndrome

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™™ Refeeding syndrome is occurrence of severe fluid and elec-

trolyte imbalance in severely malnourished individual while starting the proper feeding enteral or parenteral nutrition. It is more common in TPN. ™™ It causes hypomagnesaemia, hypocalcaemia and hypophosphataemia leading into myocardial dysfunction, respiratory changes, altered liver functions, altered level of consciousness, convulsions and often death. ™™ Gradual feeding and correction of magnesium, phosphate and calcium and other electrolytes is important. ™™ Condition is common in chronic starvation, severe anorexia and alcoholic patients.

OBESITY AND MORBID OBESITY ™™ Obesity is a condition where weight is more than 20% above

the normal. Body mass index (BMI) is weight in kilogram divided by height in metres squared [wt in kg/(Ht in meters)2]. ™™ Morbid obesity is a condition wherein BMI is more than 40 kg/m2. It is often also defined as weight 100 lbs or more; or 100% over ideal body weight. ™™ Weight more than double the expected weight to that age and height of the individual is also often called as morbid obesity. Body weight exceeding BMI 50 kg/m2 is called as superobesity.

Causes of obesity ™™ Familial, hyperinsulinism, hyperadrenocorticism, hyogonadism. ™™ Abnormal eating behaviour: Hormones which control eating

are—ghrelin from stomach; insulin from pancreas; leptin from fat; PYY 3–36 from colon. Hypothalamus is the center in CNS which controls eating.

Nutritional status

BMI (kg/m2)

• Underweight

• 30

–– Class I

–– 30.0–34.9

–– Class II (Moderate)

–– 35.0–39.9

–– Class III (Severe/Morbid)

–– 40.0

• Superobesity

• >50

• Super superobesity

• >60

Obesity is more common in young women. Child of a normal weight parents has 10% chance to become obese. If both parents are obese then child has got 80% chances to develop obesity.

Problems with Morbid Obesity ™™ Obstructive sleep apnoea, degenerative joint disease, back

pain, hypertension, GERD, gallstones, type II diabetes, hyperlipidaemia, arrhythmias, venous diseases, DVT, skin diseases, urine incontinence, infertility, ventral hernias, obesity hypoventilation syndrome, polycystic ovarian disease, hirsutism, gynaecomastia, steatohepatosis, malignancies—

are common problems. All these conditions are called as comorbidities. ™™ Infertility is common in married morbid obese women due to improper ovulation, polycystic ovary disease. Urinary incontinence is common in morbid obese women (stress incontinence and detrusor instability). Pregnancy in morbid obese patient is considered as high-risk pregnancy. Gestational diabetes, hypertension, are common. It is better these women to undergo bariatric surgery prior to pregnancy which definitely reduces the complications during pregnancy. Such individual after bariatric surgery needs more iron and vitamin supplements. ™™ Evaluation of the patient—cardiac and respiratory assessment; lipid profile; blood glucose; renal and liver status assessment; anaesthesia risk assessment.

Medical Management ™™ General: Diet, life style changes, exercise. ™™ Drugs: (1) Orlistat is a selective inhibitor of gastric and

pancreatic lipases that reduces the absorption of lipids from intestine. (2) Sibutramine is a noradrenaline and 5 HT reuptake inhibitor which act as appetite suppressor.

Surgical Treatment (Bariatric Surgery) Bariatric surgery causes long-term durable weight loss. Patient’s eating behaviour is reduced to slow ingestion of small boluses of food and or also reducing the absorptive surfaces.

Types of Bariatric Surgery ™™ Restrictive wherein caloric intake is reduced. Purely gastric

restrictive procedures are vertical banded gastroplasty (VBG) and laparoscopic adjustable gastric banding (LAGB). ™™ Malabsorptive wherein absorption of calories and nutrients from food is reduced. Biliopancreatic diversion (BPD) and biliopancreatic diversion with duodenal switch (BPD-DS) are malabsorptive procedures. ™™ Combined wherein both methods are used. Roux-en-Y gastric bypass (RYGB) is both restrictive and malabsorptive types. Gastric bypass reduces GI polypeptide ghrelin level secreted from fundus of stomach and duodenum. Ghrelin acts on specific receptor in CNS—hypothalamus to initiate appetite. This stimulation is reduced to decrease appetite. In restrictive only procedure ghrelin level raises and so appetite stimulation is not controlled.

Indications for Bariatric Surgery Body mass index (BMI) more than 40 kg/m2 or BMI more than 35 kg/m2 with comorbidity is indication for bariatric surgery. American Association of Bariatric Surgery (ASBS) published indications and approved surgeries.

Contraindications Patients who are unfit for general anaesthesia (cardiac/renal/ respiratory/hepatic causes) or who are unable to adjust post-

operative life styles or psychiatric patients are contraindications for bariatric surgery.

Gallstone formation is common after bariatric surgery (50%). It is due to rapid weight loss. If gallstones are present at the time of bariatric surgery it is essential to do laparoscopic cholecystectomy during bariatric procedure. It is controversial about doing prophylactic cholecystectomy during bariatric surgery even though it is practiced in many places. Advantages are—it prevents future gallstone formation at the time of bariatric surgery (loss of access); it prevents difficulties in approaching CBD in case needed endoscopically due to surgery. Disadvantage is cholecystectomy adds additional 1 hour time for bariatric surgery increasing the risk of immediate complications. Patients who are not having gallstones at the time of bariatric surgery, should receive ursodeoxycholic acid 300 mg twice daily for 6–12 months of post-bariatric surgery period. Often stomach is anchored to abdominal wall as access part to biliary system for future need.

Preoperative Preparations and Evaluation ™™ Complete cardiac, respiratory/renal/hepatic evaluation. ™™ Lipid profile and blood glucose assessment. ™™ Obstructive sleep apnoea in obese patient should be assessed

using polysomnography and be treated.

™™ Risk assessment for DVT should be done. ™™ If GERD symptoms are present gastroscopy should be done. ™™ USG abdomen to identify gallstones should be done; if gall-

stones present it is of usual practice to do cholecystectomy along with bariatric procedure. ™™ Nutritional evaluation and dietician advice for preoperative and postoperative diet management. ™™ Psychological screening is needed to all patients to counsel their postoperative care and diet.

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Equipments should be long and flexible. In laparoscopic surgery, special ports and instruments are needed.

DIFFERENT SURGERIES (Refer Table Below)

Note: Differnet Procedures are Disscussed Below Vertical Banded Gastroplasty (VBG); Mason (1982) • It is a purely restrictive type with creation of a calibrated stoma in the lesser curvature which is reinforced by an encircled mesh with a proximal gastric pouch. After laparotomy Ewald’s stomach tube is passed per orally to place against lesser curve. A 2.5 cm circular window is created in the body of stomach near lesser curvature 8 cm below the angle of His. After this, four lines of linear vertical stapling are done from circular opening towards angle of His. This staple line ideally should be divided using another cutting linear stapler to reduce chances of dehiscence. This creates a 50 ml proximal gastric pouch. A 1.5 × 7 cm polypropylene mesh is placed around the lesser curve through circular opening and sutured to create a 5 cm collar stoma. It is not used at present; it is only of historical importance. • VBG causes only medium term weight loss; its efficacy is less compared to other procedures. Complications of VBG are—stricture at stoma (20%), vomiting (30%), reflux (20%), staple line dehiscence (40%), conversion into other procedures. Mortality is 0.3%. • VBG is technically easier to do; it has got very less chances of long-term metabolic and nutritional deficiencies.

Laparoscopic Adjustable Gastric Banding (LAGB) • It is also a restrictive type (1992, Guy Bernard) to create a narrow stoma just below the OG junction. It is used in adolescents and elderly. It is contraindicated in hiatal and paraoesophageal hernias. It is done using laparoscopy. • Under general anaesthesia, with patient in reverse Trendelenburg position six laparoscopic ports are placed. Using pars flaccida method, retrogastric tunnel is created; a silicone band is passed through the tunnel to encircle the cardia just below the OG junction; tail of the band is buckled and locked. Stoma diameter is determined by inserting a calibration tube. Stomach over the band is imbricated using interrupted sutures except the buckle area. Silicone tube end is brought out through the abdominal wall to connect access port. It is used for band volume adjustment by injecting or withdrawing the saline.

Complications of obesity

Obesity and surgery

Treatment for obesity

• General: Difficulty in work, fatigue, depression, back pain, arthritis and gout • Cardiovascular: Hypertension, stroke, thrombophlebitis, pulmonary embolism • Pulmonary: Hypoventilation, poor respiratory effort • GIT: Hiatus hernia with reflux, changes in liver, pancreatitis, gallstones • Endocrine: Diabetes mellitus Note: Many of these patients after bariatric surgery require plastic surgery for abdominal contour (panniculectomy, abdominoplasty) after weight reduction.

• Hernia and gallstones are more common in obese individual • Burst abdomen, incisional hernia are more common in obesity • Delay in recovering from anaesthesia • Infertility is more common

• General: Dieting, exercise • Drugs: Orlistat, Sibutramine • Surgeries: –– Restrictive ŠŠ Vertical banded gastroplasty ŠŠ Laparoscopic adjustable gastric banding (LAGB) ŠŠ Jaw wiring –– Malabsorptive ŠŠ Biliopancreatic diversion (BPD) ŠŠ Biliopancreatic diversion with duodenal switch (BPD-DS) ŠŠ Jejunoileal bypass –– Combined –– Roux-en-Y gastric bypass (RYGB) open or laparoscopic

The fool is never satisfied while the wise man finds wealth in contentment

CHAPTER 1F    General Surgery: Electrolyte and Nutrition

Cholecystolithiasis After Bariatric Surgery

™™ Separate theatre table is needed for morbid obese patient.

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Fig. 1.196: Vertical banded gastroplasty (VBG). • Proximal jejunum is divided and proximal cut end is anastomosed to distal ileum just proximal to ileocaecal valve to reduce the absorptive surface area of small bowel. • Complication rates were higher and so procedure is not practiced. Colon in these patients absorbs high level of oxalate causing nephrocalcinosis. Bypassed bowel promotes bacterial growth causing endotoxic-induced liver injury, cirrhosis, liver failure. Complications like protein, vtamin K, vtamin B12 deficiencies; gallstone formation; enteritis and diarrhoea; arthritis, osteoporosis are common.

Roux-en-Y Gastric Bypass

Fig. 1.197: Laparoscopic adjustable gastric banding (LAGB). • Gastrograffin study is done on 1st postoperative day to assess band position and lumen patency. Patient is advised to have liquid diet for one month. Band adjustment is done under fluoroscopic guidance in 2 months. Adjustment is done to achieve weight loss at a rate of 2 kg/ week. Efficacy of LAGB is 55%. • Complications of LAGB are—spleen/stomach injury; bleeding; band slippage (10%); band erosion (7.5%); tube related complications; vomiting; pouch dilatation; reflux. Conversion rate is 3%; mortality is less than 0.5%.

• It is commonly done combined procedure. It can be done by open or laparoscopic method. Proximal stomach is dissected between 1st and 2nd branches of left gastric branches. Vagii nerves and nerves of Latarjet are retained carefully. Stomach is transected at this proximal site to create a proximal gastric pouch (15 ml if BMI is >50; 30 ml if BMI is 40–50). It is usually carried out through linear stapler. Jejunum is transected 45 cm from ligament of Treitz. A side to side jejunojejunal anastomosis is done using stapler 75 cm distal to the distal cut end. Proximal Roux part of the distal jejunal cut part (75–150 cm, based on patient’s preoperative weight) is brought out through the transverse mesocolon towards the created proximal gastric pouch and gastrojejunostomy is done to this proximal gastric pouch. Mesenteric defect is closed. Stomal integrity is checked on table by air distension and methylene blue infusion. Gastrograffin study is done in 24 hours to assess pouch size, stomal patency and distal obstruction. Oral food is started in 24 hours and patient is discharged in 4 days.

Jejunoileal Bypass • It was the first malabsorptive procedure done for obesity. Now this technique is not done due to high incidences of complications.

Fig. 1.199: Roux-en-Y gastric bypass procedure (RYBG). • RYGB is more useful in weight loss compared to purely restrictive types. 5 years weight loss is 60–75%. It also prevents progression of noninsulin dependent diabetes mellitus, controls hypertension, sleep apnoea, hyperlipidaemia, asthma, arthritis, GERD.

Fig. 1.198: Jejunoileal bypass procedure (JIB).

• Complications are—Roux obstruction, anastomotic leak, acute distal gastric dilatation, stomal stenosis, marginal ulcer, dumping syndrome, internal hernias, vitamin B12 deficiency, iron deficiency anaemia. Distal

103

gastric dilatation needs emergency intervention which is usually due to jejunojejunal obstruction.

Laparoscopic RYGB (1994, Wittgrove, Clark, Trembly)

• Now technique is modified to antegastric, antecolic one which has become popular (Gagner).

Fig. 1.201: Biliopancreatic diversion with duodenal switch procedure (BPD-DS).

Biliopancreatic Diversion (BPD) (Nicola Scopinaro, Italy) • It is done in patients who had failed restrictive procedure or who are superobese.

to create gastric reservoir (200 ml) along lesser curve. Duodenum just distal to first part is divided using stapler; proximal cut end is sutured to proximal upward pulled end of the distal ileal segment of earlier transected ileum, 250 cm from ileocaecal valve. Biliopancreaticoduodenal with proximal jejunoileal segment is later stapled to distal ileum 50 cm proximal to ileocaecal valve. Duodenal switch reduces the rate of marginal ulcer and dumping syndrome. • Results of BPD/BPD-DS are—excellent for weight reduction compared to restrictive procedures. But they need lifelong supplement of vitamins, fat-soluble vitamins, calcium, and iron. Technically BPD is easier to do when compared to BPD-DS. • Complications of BPD/BPD-DS are—anemia (30%); protein deficiency (20%); dumping syndrome; marginal ulcer (in BPD 10%; in BPD-DS it is 1%); osteoporosis; night blindness; biliopancreatic limb obstruction; staple line leak; staple line bleed; DVT; subphrenic abscess. Vitamin B12 deficiency is specific.

Ileal Interposition with Sleeve Gastrectomy Fig. 1.200: Biliopancreatic diversion procedure (BPD). • Distal subtotal gastrectomy is done with formation of proximal gastric pouch (of 400 ml in BMI 40–50; 200 ml in BMI >50). Ileum is transected 250 cm proximal to ileocaecal valve; distal ileal segment is brought up to anastomose into the proximal gastric pouch. Proximal biliopancreatic jejunoileal limb is anastomosed into distal ileal segment 50 cm proximal to ileocaecal valve as end to side stoma. Additionally cholecystectomy should be done. • Modification of BPD with duodenal switch (BPD-DS) has become more popular. Here sleeve gastrectomy along the greater curvature is done

It is done mainly in type II diabetes. Often they are associated with obesity, dyslipidaemia, hypertension, nephropathy and neuropathy. Two types of ileal interposition with sleeve gastrectomy are done. Type 1: Sleeve gastrectomy is done. 170 cm of ileum with mesentery is isolated 30 cm from ileocaecal junction. Jejunum is transected 50 cm from duodenojejunal flexure. Isolated 170 cm ileal segment is interposed 50 cm distal to DJ junction with end-to-end anastomosis on both ends. Type 2: After doing sleeve gastrectomy, gastroduodenal junction is transected; cut proximal end of duodenum is closed. 170 cm ileal segment with mesentry is isolated and interposed between cut end of stomach and side of jejunum 50 cm distal to DJ flexure.

The lazy man is always occupied with his laziness.

CHAPTER 1F    General Surgery: Electrolyte and Nutrition

• Technique is similar to open RYGB. Anastomoses are done using endoscopic stapler. GJ between gastric pouch and Roux jejunum is done either using linear stapler through laparoscopic port after making a gastrotomy in the pouch which is later sutured after staple firing; or using circular stapler anastomosis is done wherein anvil is initially passed transorally often under endoscopic guidance across the pouch into the Roux jejunum; or using hand sewing with absorbable sutures. Omentum is released from the colon and is covered over the GJ. Mesenteric defect and Patterson Brown defect are closed. A Bronlin antiobstructive stitch is placed between Roux and biliopancreatic limbs. Integrity of anastomosis is checked using insufflation of air, methylene blue. Complications are similar to open RYBG. Conversion rate is 9%. Advantages of LRYBG to open RYBG are—faster recovery, less postoperative pain, less wound related complications, less morbid one. Disadvantage of LRYBG is availability of facility, technical expertise, and steep learning curve.

G. Shock Contd...

C hapter Outline ·· ·· ·· ··

Shock Stages of Shock Effects of Shock Clinical Features of Shock (Hypovolaemic Shock) ·· Assessment, Investigations and Monitoring ·· Central Venous Pressure

·· Pulmonary Capillary Wedge Pressure ·· Systemic Inflammatory Response Syndrome ·· Multiple Organ Dysfunction Syndrome ·· Oxygen Therapy ·· Cardiac Arrest

Shock ™™ Shock is defined as a state of cellular and tissue hypoxia with

either reduced oxygen delivery or poor oxygen utilisation or increased oxygen consumption with circulatory failure (collapse) and poor perfusion. ™™ Normal aerobic metabolism is not maintained due to hypoperfusion. Shock is meant by ‘inadequate perfusion’ to maintain normal organ function. It may be initially reversible but becomes irreversible eventually if not treated leading multiorgan failure and death. Shock can be hypovolaemic (16%), cardiogenic (16%), distributive(septic (62%), neurogenic/anaphylactic (4%), acute adrenal insufficiency) or obstructive (2%) or endocrine (hypothyroidism, thyrotoxic crisis, adrenal insufficiency). ™™ At cellular level hypoxia causes change of normal aerobic to anaerobic metabolism causing lactic acidosis. Intracellular potassium is released into circulation. Lysosomes from cells get released into blood causing cell lysis. Hypoxia and acidosis through complements release free oxygen radicals and cytokines which damage capillary endothelium. Eventually cardiovascular, respiratory, renal, endocrine and GIT will be affected presenting as systemic features.

B

Causes of shock

1. Hypovolaemic shock—due to reduction in total blood volume. It may be due to: a. Haemorrhage –  External from wounds, open fractures – Internal from injury to spleen, liver, mesentery or pelvis b. Severe burns, which results in loss of plasma c.  Peritonitis, intestinal obstruction d.  Vomiting and diarrhoea of any cause 2. Cardiac causes a.  Acute myocardial infarction, acute carditis b. Acute pulmonary embolism wherein embolus blocks the pulmonary artery at bifurcation or one of the major branches

Contd...



c.  Drug induced d.  Toxaemia of any causes e. Cardiac surgical conditions like valvular diseases, congenital heart diseases f.  Cardiac compression causes i. Cardiac tamponade due to collection of blood, pus, fluid in the pericardial space which prevents the heart to expand leading to shock. ii.  Trauma to heart 3. Septic shock—is due to bacterial infections which release toxins leading to shock xx 4. Neurogenic shock—due to sudden anxious or painful stimuli causing severe splanchnic vessel vasodilatation. Here, patient either goes for cardiac arrest and dies or recovers fully spon­taneously—spinal cord injury/anaesthesia can cause neurogenic shock 5. Anaphylactic shock—is due to Type 1 hyper­sensitivity reaction 6. Respiratory causes a.  Atelectasis (collapse) of lung b.  Thoracic injuries c.  Tension pneumothorax d.  Anaesthetic complications 7. Other causes a.  Acute adrenal insufficiency (Addison‘s disease) b. Myxoedema

Pathophysiology of Shock Any cause of shock ↓ Low cardiac output ↓ Vasoconstriction occurs as a compensation to perfuse vital organs like brain, heart, kidneys, liever ↓ Because of vasoconstriction and tachycardia ↓ Dynamic circulation increases ↓ Tachypnoea occurs to increase the oxygen saturation ↓ Peripheral veins (capacitance vessels) constrict diverting blood from splanchnic system towards essential vital organs ↓ Decreased renal blood flow reduces the GFR and thereby the urine output ↓ Renin angiotensin mechanism gets activated causing further vasoconstriction and aldosterone release ↓ Causes salt and water retention ↓

ADH is released ↓ Further concentration of urine occurs

Hypoxia ↓ Anaerobic metabolism ↓ Lactic acidosis ↓ Cell wall damage ↓ Sodium and calcium enter the cell ↓ Potassium leaks out of the cell ↓ Causes hyperkalaemia, hyponatraemia and hypocalcaemia ↓ Intracellular lysosomes break down releasing powerful enzymes which destroy own cell ↓ SICK CELL SYNDROME Platelets are activated forming small clots in many places ↓ Disseminated intravascular coagulation (DIC) (Consumption coagulopathy) ↓ Further bleeding.

STAGES OF SHOCK Factors like infection, trauma, burns, haemorrhage, hypovolaemia ↓ Hypoxia and its effects. ↓ SIRS (Systemic inflammatory response syndrome) is due to vasodilatation, increased endothelial per­meability, thrombosis, leucocyte migration and activation. ↓ All these lead to altered cytokines level, abnormal NO (nitric oxide) synthesis, abnormal arachidonic acid metabolism, neutrophil activation,

B xx

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xx

xx

Stages of shock

Stage of hypoperfusion and hypoxia: aerobic metabolism changes to anaerobic leading to lactic acidosis (metabolic acidosis). Stage of compensatory shock: It is neuroendocrine response to maintain the perfusion of vital organs like brain, lungs and heart. Noradrenaline, renin-angiotensin and antidiuretic hormone (ADH) gets activated causing vasoconstriction of organs like gastrointestinal, kidney to divert the blood to heart, lungs and brain. Stage of decompensatory (progressive) shock: Here compensatory mechanism fails; cell perfusion decreases causing raised intracellular sodium but low intracellular potassium. Microcirculation fails beginning the failure of kidneys, liver and lungs. Stage of irreversible (refractory) shock: Here cellular ATP metabolism is lost completely leading into MODS and MOF (multiorgan failure).

Note: • Distributive shock is one in which there is vasodilatation, decreased vascular resistance, hypotension, altered microvascular perfusion with arteriovenous shunting, altered cellular oxygen metabolism. It is seen in septic shock, spinal trauma, adrenal crisis and anaphylaxis. • Obstructive shock occurs due to mechanical impediment of circulation due to pulmonary embolism, tension pneumothorax or cardiac tamponade, aortic stenosis.

Effects of Shock Heart: Low perfusion → low venous return → decreased cardiac output → hypotension → tachycardia. Persistent shock causes hypoxia and release of myocardial depressants leading to further cardiac damage. Lung: Interstitial oedema → decreased gaseous exchange → pulmonary arteriovenous shunting → tachypnoea → Adult/Acute respiratory distress syndrome (ARDS) and pulmonary oedema. Metabolic: Shock leads to hypoxia, which activates anaerobic metabolism leading to lactic acidosis. Antidiuretic hormone (ADH) is released which increases the reabsorption of water from renal tubules. Other hormones released are ACTH, prostaglandins, histamine, bradykinin, and serotonin to compensate the effects of shock to increase the perfusion of vital organs like heart, brain and lungs. Cellular changes occur in persistent shock due to release of lysosomal enzymes, which alters the cell membrane permeability causing cell death—sick cell syndrome. Sympathetic overactivity alters the microcirculation leading to capillary dysfunction.

In time of test, family is best.

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CHAPTER 1G    General Surgery: Shock

When shock persists cardiac output falls further ↓ Hypotension and tachycardia occurs leading to poor perfusion of coronaries ↓ Hypoxia—metabolic acidosis ↓ Release of cardiac depressants ↓ Cardiac (pump) failure

free radical production, altered complement activation, failure to have a localisation of inflammation. It is severe type of reversible shock. ↓ Which will lead to established microvascular occlusion, cellular dysfunction, sick cell syndrome, DIC and PUMP failure. ↓ MODS (Multiorgan dysfunction syndrome) (Irrever­sible shock)—of lungs, kidneys, liver, clotting system and brain.

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Brain perfusion, when decreases the patient becomes drowsy. Brain is the last organ to get underperfused in shock.

4. Cardiogenic 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.

ished forward flow leading into tissue hypoxia in the setting of adequate intravascular volume with systolic blood pressure 50%. ¾¾ Cardiogenic shock develops within 24 hours of MI. it occurs when 50% of left ventricular wall is damaged by infarction. ¾¾ It leads to pulmonary oedema and severe hypoxia. Ischaemic 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.

Blood: Alteration in cellular components including platelets leads to Disseminated intravascular coagulation (DIC). It causes bleeding from all organs. Gastrointestinal tract: Mucosal ischaemia develops causing bleeding from GIT with haematemesis and malaena. It is aggravated by DIC. Hepatic ischaemia leads into increased enzyme levels.

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. ¾¾ 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 (α 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

Types of Hypovolaemia

a. Covert compensated hypovolaemia (Mild 40%): 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.

™™ Cardiogenic shock is defined as circulatory failure causing dimin-

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 (β1 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. b 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 ™™ It is probably due to pericardial 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 vasculature leads 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 protozoal origin. 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 ↓ Inflammation, cellular activation of macrophages, neutrophils, monocytes ↓ Release of cytokines, free radicals ↓ Chemotaxis of cells, endothelial injury, altered coagulation cascade—SIRS ↓ Reversible hyperdynamic warm stage of septic shock with fever, tachycardia, tachypnoea ↓ Severe circulatory failure with MODS (failure of lungs, kidneys, liver, heart) with DIC

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—GCS38°C) or hypothermia (90/minute); tachypnoea (>20/minute); total white cell count >12,000/cu mm, or count 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. ™™ Integra 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,

B

Reconstruction territories in neck in burn contracture based on functional benefits are—central above; central below; central above and below; lateral.

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.

Treatment for Contracture ™™ Release of contracture surgically and use of skin graft or “Z”

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

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); fasciocutaneous/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.

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°; E1 95–110°; E2 is 85–95°; E3 is 30% Burns should be assessed whether it is accidental or homicidal Relatives should be informed about the duration of stay, problems, repeated surgeries In patients with severe burns who is likely to die, when suspected, dying declaration should be arranged Cost of therapy, long duration of stay and cosmetic problems should be informed to the relatives

J. Trauma

C hapter Outline

B

·· Triage ·· Concepts in Trauma Management ·· Spinal Injury ·· Neck Injuries ·· Bullet Injuries ·· Blast Injuries ·· Penetrating Injuries ·· Abdominal Trauma

i. ii. iii. iv.

·· Blunt Trauma of Abdomen ·· Duodenal Injury ·· Small Bowel Injury ·· Colonic Injury ·· Abdominal Compartment Syndrome ·· Seat-belt Injuries

Trauma is a major public health problem in all countries. It causes death disability and both. 50% die immediately at the time of accident. 25% die in golden hour of trauma, i.e. first 4–6 hours of trauma. 25% may die late during treatment period due to sepsis and complications. Injuries may be penetrating, blunt, blast, chemical, electrical and other injuries.

B

Assessment of four components Physiologic response Anatomical injury Biomechanical injury Comorbid factors Triage algorithm

Step One (Assess physiological impact) Measure vital signs and level of consciousness xx By Glasgow coma scale xx Systolic blood pressure xx Respiratory rate xx Revised trauma score. It is based on airway, laryngeal injury, spine injury, maxillofacial injury Step Two (Assess anatomical impact) All penetrating injuries to head, neck, thorax, major burns, fracture bones, pelvic fractures, paralysis Step Three (Assess mechanism) Automobile accidents, crash or blast injuries, high energy injuries, fall from more than 20 feet. Bullet injury Step Four (Assess history) xx xx xx xx xx

Fig. 1.235: Trauma causing large tissue defect exposing the bone.

Patient’s age below 5 years or age more than 55 years Cardiac diseases, respiratory and metabolic diseases Pregnancy Patients with bleeding disorders Immunosuppressed individuals

BASED ON THESE STEPS CONSIDER TO SHIFT THE PATIENT TO TRAUMA CENTER and TRAUMA TEAM SHOULD BE KEPT ALERT. It is important in multiple and mass casualities (fire, blasts, automobile accidents, train accidents).

Management

TRIAGE

™™ Initial evaluation of the patient.

Triage means “To sort” in French. Triage is a system to attend trauma patients, formu­lated by Committee of Trauma of the American College of Surgeons. Advanced trauma life support (ATLS) is essential for first hour care of an injured patient. Pre-hospital trauma life support (PHTLS) is to prevent deaths while injured patients are transported to the hospital.

Types of Triage System ™™ Multiple casualties: Staff and facilities are sufficient but

priority is given to life-threatening injuries.

™™ Mass casualties: Staff and facilities are not sufficient to

manage. Here those who are likely to have highest chance of survival are given priority.

™™ Physiologic stabilisation. ™™ Control of haemorrhage. ™™ Management of thoracic and abdominal injury. ™™ Management of cranial injury.

I.  Primary Management ™™ Airway management (blocked by food, vomitus, clot, fallen

tongue). ™™ Breathing. ™™ Circulation. ™™ Disability and level of consciousness assessment by Glasgow coma scale. ™™ Exposure of the patient from head to toe for final assessment. ™™ Fingers and tubes: Finger evaluation, Foley’s catheterisation.

There is no right way to do something wrong.

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Goals

III.  Secondary Survey

™™ Identify life-threatening conditions. ™™ Decide and implement appropriate treatment to the area of

Re-evaluate the patient completely again.

™™ First think to salvage the life, then think to salvage the limb. ™™ Rapid assessment, rapid resuscitation, rapid stabilisation. ™™ Optimum, complete care. ™™ Transport efficiently to higher trauma centre.

(All discussed under individual topics.)

trauma.

B

I: II: III: IV:

IV.  Definitive Care

Mechanism of Trauma ™™ Blunt trauma—direct or indirect blunt injury can occur. Seat-

belt reduces the blunt injury in vehicles.

Categorise the patient

™™ Penetrating injury—severity depends on the extent of deeper

Deceased Walking wounded Immobile wounded Trapped wounded

™™ Blast injury. ™™ Crush injury—earthquake, industrial accidents, and train acci-

Tag the patient accordingly Red colour : Immediate treatment is required : Urgent treatment is required Yellow colour : Delayed treatment is required Green colour : Expectant treatment is required Blue colour : Deceased Black colour

injury.

dents—causes crush syndrome; compartment syndrome.

™™ Burn injury. ™™ Injury in alcohol patients.

BREATHING AIRWAY Chin lift 100% oxygen, 15 litres/minute Assess bilateral chest raise Jaw thrust Nasal airway Assess breath sounds Oral airway Use pulse oximetry Endotracheal intubation Treat flail chest, pneumothorax Tracheostomy Intercostal tube drainage (assess airway patency) CIRCULATION DISABILITY EVALUATION Monitor vitals Neurological examination Heart sounds Glasgow coma scale ECG Pupillary reaction IV fluids blood transfusion Treatment of shock Control of external bleed Use two IV lines—14G/16G

Fig. 1.236: Crush injury leg due to road traffic accident.

EXPOSE THE PATIENT FINGERS and TUBES FULLY Examine all orifices like P/R, P/V, etc. Undress the patient Use required tubes like catheter, Hypothermia assessment Ryle’s tube Assess injuries Examine joints, bones, abdomen, other systems Look for identification marks

II. Investigations ™™ X-ray spine, chest, pelvis, extremities. ™™ CT scan—whole body CT scan is useful as it identifies all injuries. But still its concept in trauma to achieve effective survival is under debate. ™™ Blood group and cross-matching. ™™ Arterial blood gas analysis. ™™ Serum electrolytes. ™™ Ultrasoud abdomen.

Fig. 1.237: Ankle injury with open wound.

135

Abbreviated injury scale (AIS)

Injury

1

2

3

4

5

6

AIS score

Minor Moderate Serious Severe Critical Unsurvivable

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.

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 and colostomy was done to promote healing of perineal wound and prevent contamination.

Other scoring systems New injury severity score (NISS): It is the sum of the squares of the top three scores regardless of the body region. The NISS is said to be better than ISS score. Trauma and Injury Severity Score (TRISS) system is done using anatomical and physiological scores; it indicates the probability of survival.

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

CRAMS score (Circulation; Respiration; Abdomen; Motor; Speech) is used often; score 29

3

6–8

50–75

6–9

2

4–5

1–49

1–5

1

3

0

0

0

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).

CHAPTER 1J    General Surgery: Trauma

It is an anatomical scoring system. Injuries are ranked on a scale of 1 to 6, with 1 being minor, 5 severe and 6 a nonsurvivable injury. This assesses the 'threat to life' of an injury and is not a measure of severity.

136

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

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injury.

B xx xx xx

xx

xx

Concepts

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 35

≥25

Abdominal compartment syndrome (ACS) Causes • Multiple trauma and ICU patients— common • Postoperative ileus • Acute abdomen • Acute gastric dilatation • Laparoscopic procedures • Intestinal obstruction • Major burns

Features • Hypoxia, hypercarbia • Decreased urine output – anuric renal failure in severe cases • Tense abdomen— distended • Decreased venous return • Refractory hypoxaemia in severe cases • Bowel ischaemia • Cardiac arrest

Management • Bladder pressure assessment • Ryle’s tube aspiration • Hypotension • Resuscitation • ICU care • Surgical decompression

Treatment ™™ Abdominal decompression is the only ideal treatment for

ACS; technique and timings are decided based on the clinical situation. ™™ Temperature and coagulation profile should be made as possible as normal prior to decompression. ™™ Silastic sheet created chimneys sutured to fascia around is often used. Pressure free abdominal closure should be the target. Bogota bag, first described by Londoni, chief

SEAT-BELT INJURIES ™™ In an individual with seat-belt, during impact, violent decel-

eration of human body occurs. Seat-belt impinges heavily on its point of contact with trunk and viscera continue to move forward. It leads into severe contusion of abdominal contents; detachment of bowel from its mesentery due to free forward rapid mobility of the bowel over a relatively fixed mesentery. Solid organ injury occurs only occasionally. ™™ Two point anchorages causes’ solid organ injuries like of liver/spleen. Lap-belt causes contusion and bowel injury commonly. ™™ It is often difficult to identify the injuries due to presence of more obvious other injuries. CT chest and abdomen, diagnostic peritoneal lavage (DPL) are very useful. ™™ Petechiae around iliac crest or costal margin are signs wherein one can suspect seat-belt injuries. ™™ Distraction fracture of lumbar spine (chance fracture) with hyperaesthesia of T12 and L1 level is often associated. 10% of such fractures are associated with intra-abdominal injuries. ™™ Treatment is immediate laparotomy and proceed—bowel suturing/resection/suturing of the organ injuries/splenorrhaphy/splenectomy.

What we need is cup of understanding, barrel of love and an ocean of patience.

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CHAPTER 1J    General Surgery: Trauma

tocrit and serum creatinine estimation, USG abdomen should be done. ™™ Mortality for ACS is 40%.

resident in Bogota, Columbia is cost effective; here irrigation bags are sutured to each other as necessary to get a proper size and is sutured to fascia around using 1-zero nylon suture. Several litres of serosanguineous or ascitic fluid are let out through a plastic stoma bag attached to a closed drainage system. Once patient is haemodynamically stable, definitive closure is performed after 48 hours. Abdominal fascia is closed using nonabsorbable sutures; subcutaneous tissue and skin are closed at a later period. Fluid and electrolyte management, antibiotics, adequate blood or blood product transfusions should be used as needed. ™™ Other methods of closure: Towel clips, temporary mesh placement; PTFE mesh repair; vacuum assisted closure are all temporary closure methods. Definitive closure methods— primary closure of fascia; closure using synthetic mesh if no sepsis in the wound; biological mesh closure; component separation technique; closure using skin graft or flaps.

K. Hand and Foot

C hapter Outline ·· ·· ·· ·· ·· ·· ·· ·· ·· ·· ··

Hand Hand Infections Acute Paronychia Chronic Paronychia Apical Subungual Infection Terminal Pulp Space Infection (Felon) Infection of Web Spaces Deep Palmar Space Infection Space of Parona Infection Acute Suppurative Tenosynovitis Compound Palmar Ganglion

·· Hand Injuries ·· Dupuytren’s Contracture ·· Volkmann’s Ischaemic Contracture ·· Syndactyly ·· Foot ·· Callosity ·· Corn ·· Plantar Fasciitis (Policeman’s Heel) ·· Ingrowing Toe Nail (Onychocryptosis) ·· Athlete's Foot ·· Hallux Valgus

HAND Surgical Anatomy of the Hand Flexor Retinaculum ™™ It extends medially from pisiform and hook of hamate, later-

ally to scaphoid tubercle and trapezium crest as a strong fibrous band so as to bridge carpus to create a carpal tunnel. ™™ Ulnar nerve and vessels, palmar cutaneous branches of median and ulnar nerves, palmaris longus muscle are superficial to the carpal tunnel. ™™ Median nerve, tendons of flexor digitorum superficialis, profundus and pollicis longus, radial and ulnar bursa are deep to flexor retinaculum.

™™ Deep palmar arch is formed by radial artery and is completed

by deep branch of ulnar artery. It gives three palmar metacarpal arteries which communicate with superficial palmar arch. It also gives communicating, perforating branches to dorsal metacarpal arteries.

Muscles of the Hand ™™ Thenar muscles: Abductor pollicis brevis, flexor pollicis

brevis, opponens pollicis and adductor pollicis.

™™ Hypothenar muscles: Palmaris brevis, abductor digiti minimi,

flexor digiti minimi and opponens digiti minimi.

™™ Lumbricals are four in number—named from lateral to

medial.

™™ Four palmar interossei. ™™ Four dorsal interossei.

Nerve Supply ™™ Abductor pollicis brevis, flexor pollicis brevis, opponens

pollicis and 1st and 2nd lumbricals are supplied by median nerve (5 muscles). ™™ Rest of the muscles in hand are supplied by ulnar nerve (15 muscles).

Modified Verdan Zone System in the Hand (Tendon zones) ™™ Zone I: From the fingertip up to the attachment of flexor

digitorum superficialis (middle of middle phalanx). It contains tendon of flexor digitorum profundus. ™™ Zone II: It begins proximal to metacarpophalangeal joint at distal palmar crease and extends up to the attachment of

Palmar Aponeurosis It is a thickened, modified deep fascia in the palm with its apex pointing proximally (as continuation of palmaris longus) and base distally which in turn gets divided into four parts. They extend over deep transverse ligament into lumbrical tunnel.

Blood Supply of the Hand ™™ Superficial palmar arch is mainly formed by ulnar artery and

completed by superficial palmar branch of radial artery. It gives four digital branches to medial three fingers.

A

B

Figs. 1.256A and B: (A) Anatomy of flexor retinaculum and palmar aponeurosis; (B) Modified Verdan zone system in the hand.

HAND INFECTIONS ™™ Hand is a compact actively functioning unit. It contains neuro-

vascular bundles, muscles, bones and ligaments.

™™ Infection may be due to minor injuries or blood borne.

B

Different types of hand infections

Acute paronychia Chronic paronychia xx Terminal pulp space infection (felon) xx Subungual infection xx Web space infection xx Mid-palmar space infection xx Thenar space infection xx Deep palmar abscess xx Acute suppurative tenosynovitis xx Chronic tenosynovitis of flexor tendon sheath of palm and forearm—compound palmar ganglion xx Lymphangitis of the hand xx Arthritis of hand joints xx Subcuticular abscess Hand infection can be superficial or deep; it can be localised or spreading. xx xx

General Principles of Managing Hand Infections ™™ Antibiotic therapy. ™™ Position of rest with wrist slightly abducted and extended,

Fig. 1.257: Infection of finger at middle phalanx level. Note the oedema and position of the finger. ™™ Precipitating causes: Diabetes; Immunosuppression;

Trauma; HIV infection; Steroid therapy; Vascular diseases.

B xx xx xx xx

Common organisms

Staphylococcus aureus—most common—90% Streptococcus Gram-negative organisms like E. coli, Klebsiella, Pseudomonas Occasionally fungal infection causing chronic paronychia, Madura hand due to Nocardia group of fungi, viral infection like orf can occur

General Features of Hand Infection ™™ Infection spreads faster in all areas. ™™ Causes oedema over the dorsum of hand due to lax skin and

more lymphatic network even though infection per se is more over the volar aspect. It looks like frog hand. ™™ Restricted movements of fingers and hand. The hand functions like hook, pinch, grip, grasp are lost. ™™ Severe pain and tenderness, with fever. ™™ Tender palpable axillary lymph nodes are often present. ™™ Investigations: Pus for culture and sensitivity; Blood sugar; Urine sugar and ketone bodies; X-ray of the part; Arterial Doppler of the hand if needed.

147

thumb and index fingers away (glass-holding position). Position of function is in which thumb and index fingers are pinching firmly with wrist extension. ™™ Elevation of hand reduces the oedema, increases perfusion, promotes healing. ™™ Early recognition of localised pus. Once localised, Incision and Drainage is done ideally under general anaesthesia or regional block (not local anaesthe­sia). Draining incision should not cross the palmar crease. Incision should have adequate length and adequate depth (deep to palmar fascia, otherwise evacuation of pus is inadequate). Care should be taken not to injure neurovascular bundles. Pus should be sent for culture and sensitivity. Slough, if present should be excised thoroughly. Gauze drain is placed. Regular dressings are done with conti­nuation of antibiotics. Communi­cations into other areas of hand should also be drained. ™™ Bloodless field (using tourniquet) is better to drain pus from hand. ™™ Proper measures must be taken after treatment. Initial rest, elevation of hand and later proper physiotherapy and regular exercise of hand and fingers are encouraged to restore normal function.

B xx xx xx xx xx xx

Complications of hand infections

Stiffness of digits and hand (ankylosis) Deformity and disability Bacteraemia and septicaemia Osteomyelitis of bones depending on the location of abscess like metacarpal bones, terminal phalanx Suppurative arthritis of joints Paralysis of median nerve

An ounce of action is worth a ton of theory.

CHAPTER 1K    General Surgery: Hand and Foot

flexor digitorum superficialis at the middle of the middle phalanx. It is called as “No-Man’s-Land.” Here flexors are tightly enclosed within a fibro-osseous tunnel. It is the most dangerous zone in hand injuries (critical zone). ™™ Zone III: It begins at the distal end of flexor retinaculum (base of the palm) and ends at the transverse crease of the palm. It contains lumbricals attached to flexor digitorum profundus. ™™ Zone IV: It begins at the proximal end of the flexor retinaculum and ends at its distal end. ™™ Zone V: It extends from the proximal end of flexor retinaculum up to distal third of the forearm.

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A

C

B

D

E

F

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 xx xx

xx

Contd...

Remember

Hand should be flexible 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...

xx

xx

Nerve conduction studies; electrophysiology; MRI 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 surgical drainage of infective area; regional anaesthesia; usage of tourniquet; incisions when done across the flexor creases, should be at 45° angle

149

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.

A

™™ 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.

Features and Management ™™ Severe throbbing pain and tenderness (dependent throbbing) B

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

Fig. 1.261: Hand infection. Infection of ring finger extending into the palm.

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 using xylocaine 2% plain (without adrenaline as end artery supply to digits can develop arterospasm) is given as anaesthesia.

A

B

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.

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CHAPTER 1K    General Surgery: Hand and Foot

Fig. 1.263: Hypertrophic scar and keloid in hand and forearm after burn contracture.

Fig. 1.259: Hand positions.

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™™ Drainage with ‘V’ incision over the summit is the treatment

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along with antibiotics. ™™ Osteomyelitis is not common.

A

Fig. 1.266: Apical subungual infection and pus formation. Note the ‘V’ shaped incision to drain the same.

TERMINAL PULP SPACE INFECTION (FELON) ™™ It is the second most common hand infection (25%). B

Figs. 1.265A and B: Paronychia showing pointing pus in one picture and sloughed area granulating in another picture. ™™ If there is a floating nail, then the nail is dead and it has to

be removed. ™™ Recovery is fast.

™™ Index and thumb are commonly affected. ™™ Usually by a minor injury like finger prick.

B xx xx

CHRONIC PARONYCHIA It is commonly due to fungal infection—due to candida infection commonly.

xx

Features and Management ™™ It is common in females. ™™ Nail is diseased with ridges and pigmentation. ™™ Itching in the nail bed; Recurrent pain, discharge ™™ Secondary bacterial infection may supervene. ™™ Investigation: Culture of scrapings for fungus and other

causative agents. ™™ Treatment: ¾¾ Long-term antifungal therapy—local and systemic. ¾¾ Antibiotics for secondary infection. ¾¾ In severe cases removal of nail is required.

APICAL SUBUNGUAL INFECTION ™™ It is infection of the space between subungual epithelium

and the periosteum. ™™ It occurs after minor trauma or rarely after formation of subungual haematoma. ™™ Beneath the free edge of the nail, pus comes to the surface. ™™ Excruciating tenderness with small visible pus under the tip (summit) of the nail is the feature.

Surgical anatomy

Terminal pulp space contains fat and is partitioned by septae which is attached from periosteum of terminal phalanx to skin Proximally deep fascia is attached to the periosteum distal to the base of terminal phalanx, i.e. distal to the attachment of flexor tendon So, terminal space is a closed compartment, as the result of which pressure increases when there is infection, compressing terminal artery leading to thrombosis, resulting in osteomyelitis of terminal phalanx

™™ Bacteria: Staphylococcus—most common; Streptococcus,

Gram-negative organisms.

Features ™™ Pain, tenderness, swelling in the terminal phalanx. ™™ Fever, tender axillary lymph nodes.

Often suppuration is severe, forming collar stud abscess which eventually may burst.

B xx xx xx

Causes of collar-stud abscess

Tuberculous cold abscess Terminal pulp space infection (Felon) Deep palmar space infection

™™ Investigations: X-ray of the part is required often to rule

out osteomyelitis of terminal phalanx; Pus for culture and sensitivity to be sent. ™™ Treatment: ¾¾ Antibiotics and analgesics are started.

A

B

C

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: ¾¾ Osteomyelitis of the terminal phalanx. ¾¾ Pyogenic arthritis of distal interphalangeal joint and tenosynovitis of flexor sheath. ¾¾ Septicaemia—in immunosuppressed individuals. ¾¾

¾¾

INFECTION OF WEB SPACES Surgical Anatomy 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. xx Infection of proximal volar space of finger. xx Infection of proximal spaces. xx Spread from other palmar spaces and from flexor sheaths through lumbrical canal. xx

™™ Bacteria: Staphylococcus, Streptococcus, Gram-negative

organisms.

™™ Features and management: ¾¾ ¾¾ ¾¾ ¾¾ ¾¾ ¾¾ ¾¾ ¾¾

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.

Fig. 1.268: Incision for web space drainage.

DEEP PALMAR SPACE INFECTION Surgical Anatomy Two deep palmar spaces are present 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 finger. It contains flexor tendons, neurovas­ cular 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.

midpalmar space infection ™™ Causes

Trauma. Spread from infection of finger spaces and web spaces. ¾¾ Haematogenous spread. ¾¾ Spread from tenosynovitis. ™™ Features ¾¾ Pain and tenderness in the palm. ¾¾ Oedema of dorsum of hand (frog hand). ¾¾ Loss of concavity of palm. ¾¾ Painful movement of metacarpophalangeal joint (but interphalangeal joint movements are normal and painfree). ¾¾ Fever. ¾¾ Palpable tender axillary lymph nodes. ¾¾ Eventually pus may come out of palmar aponeurosis forming collar stud abscess and later sinus formation. ¾¾ X-ray of the part is required. ™™ Treatment: ¾¾ Elevation of the affected limb. ¾¾ ¾¾

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Pus is drained and sent for culture and sensitivity. If other web spaces are involved they should be drained through a separate incision. Edges of the wound are cut to leave a diamond shaped opening in front. Often counter-incision over dorsal skin of web is needed.

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A

Fig. 1.270: Incisions to drain midpalmar space infection.

B xx xx xx

B

xx

Complications

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

Thenar space infection 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 longus 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 midpalmar space infection. C

Figs. 1.269A to C: Anatomy of palmar 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. ¾¾ ¾¾

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. Palmar 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.

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

™™ It is drained similarly by placing incision on the lateral aspect

SPACE OF PARONA INFECTION 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 midpalmar 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.

ACUTE SUPPURATIVE TENOSYNOVITIS

™™ 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.

B xx xx xx xx

Kanavel signs

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 palmar creases

™™ In infection of radial bursa thumb is swollen with pain and

tenderness over the sheath of the flexor pollicis longus and there is inextensibility of interphalangeal joint. ™™ Swelling just above the flexor retinaculum is common.

It is the bacterial infection of flexor tendon sheaths.

Surgical Anatomy xx xx

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.

A

Fig. 1.272: Bursae hand. xx xx

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

™™ Common bacteria: Staphylococcus aureus, Strepto­coccus

pyogenes.

B

Figs. 1.273A and B: Suppurative tenosynovitis is drained through incision at proximal part and another at digital sheath. Often by placing fine polythene catheter into the sheath, saline wash is given into the area.

When a hand is seriously inflamed it takes up the position of greatest ease, which is, in fact, the position of rest.—Frederic Wood Jone

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of the palm or through the first web space incision is done along the first lumbrical canal on the radial side of the index finger. Often 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.

Features

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™™ Treatment: ¾¾ ¾¾

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¾¾ ¾¾

Elevation of the affected limb. 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.

B xx xx xx xx xx xx

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 a purulent-appearing papule 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’S NODES/NODULES (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

B xx

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 rheumatoid 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: ¾¾ ESR, chest X-ray, MRI hand. ¾¾ FNAC of axillary lymph node and swelling itself. ™™ Treatment: ¾¾ Start antituberculous drugs: INH, rifampicin, ethambutol and pyrazinamide for 9 months. ¾¾ Excision of flexor tendon sheath is done along with scraping of caseating material, tubercles, melon seed bodies. ¾¾ Care should be taken not to injure median and ulnar nerves. Note: ORF: It is a rare, benign, self-limiting exanthematous disease, also known as contagious pustular dermatitis or infectious labial dermatitis or ecthyma contagiosum or thistle disease or scabby mouth caused by a parapox

xx xx xx xx

Classification

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. Degloving injuries Indetermined injuries which could not be assessed.

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

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

globulin).

A

B

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

™™ Skin grafting or flaps for skin loss.

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™™ Tendon suturing or tendon graft for tendon injuries. ™™ Rest and elevation of the affected parts. ™™ 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 only when inevitable.

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.

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.

B xx xx xx

Complications and morbidity of hand injuries

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

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.— Ivo Andrick

CHAPTER 1K    General Surgery: Hand and Foot

™™ Management of fractures by splint, wiring.

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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

DUPUYTREN’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 palmar 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).

B xx xx xx xx xx

A

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: ¾¾ ¾¾ ¾¾ ¾¾ ¾¾ ¾¾

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.

B

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.

B xx xx

Galezia triad

Dupuytren’s contracture Retroperitoneal fibrosis

xx

Peyronie’s disease of penis

™™ Complications: ¾¾

rule out the onset of impending contracture).

™™ Paresis.

Late phase: Deformity Deformity (due to injury to median nerve): Wrist 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.

xx xx xx

™™ Treatment:

In acute phase: ¾¾ Removal of plastic cast applied after fracture reduction. ¾¾ Correction of fracture. ¾¾ Exposure of brachial artery and application of 2.5% papaverine sulphate to relieve the spasm if any. ¾¾ Suture of arterial tear if present, often with placement of arterial graft. ¾¾ Lateral incision over the deep fascia of forearm is placed to decompress the oedema. In late phase (once deformity occurs): ¾¾ Physiotherapy, Dynamic splints. ¾¾ Max-Page operation: Release of flexor muscles (forearm muscles) from their origins from the bone and allowing it slide down until full extension. ¾¾ Excision of fibrous tissue and damaged muscles along with tendon transfer; Arthrodesis.

Fig. 1.281: Z plasty done for Dupuytren‘s contracture.

VOLKMANN’S ISCHAEMIC CONTRACTURE It is a vascular injury leading to muscular infarction and subsequent contracture. ™™ Causes: ¾¾ Supracondylar fracture of the humerus. ¾¾ IV chemotherapy; Burns. ¾¾ Closed forearm crush injuries. ¾¾ Tight plaster after reduction of fracture. ™™ Pathogenesis:

Injury of brachial artery (tear, contusion, spasm, compression)

Results in infarction Injury to median nerve of forearm flexor (mainly) and ulnar nerve muscle both by ischaemia and ↓ infarction Aseptic muscle necrosis ↓ Fibrosis of flexor muscle of forearm ↓ Contracture ™™ Features: Acute phase: ¾¾ Pain (persistent pain in forearm, hand, fingers—ominous symptom). ¾¾ Pallor; Puffiness (due to oedema).

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SYNDACTYLY ™™ It is webbing or fusion of fingers. ™™ Causes: Congenital and hereditary—common; Traumatic

like burns.

B xx xx xx xx xx xx xx

Types

Cutaneous—simple. Fibrous. Bony—complex. It can be unilateral or bilateral. Often all four 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: ¾¾ ¾¾ ¾¾

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: • mallet 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.—Napoleon

CHAPTER 1K    General Surgery: Hand and Foot

Restriction of hand function and so disability. Arthritis of metacarpophalangeal (MCP) and proximal interphalangeal (IP) joints. ™™ Treatment: ¾¾ Fasciotomy of palmar aponeurosis and later physio­ therapy, Z plasty. ¾¾ In severe cases fasciectomy partial or complete. ¾¾ Treatment of the cause. ¾¾ Recurrence can occur in 5–50% cases. ¾¾

™™ Pulseless (absence of radial pulse; but its presence does not

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• Heberden’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”.

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.

Fig. 1.282: Syndactyly. Fig. 1.286: Callosity in the foot. ™™ A callosity protrudes outwards from the skin. ™™ It is greyish-brown, raised, protruded outwards, thickened,

Fig. 1.283: Syndactyly and polydactyly of both hands and both feet.

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.

Fig. 1.284: Arthritis of joints of hands.

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 foot 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

™™ Both medial and lateral sides of the toe can be involved.

™™ It is localised area of thickening over a bony projec­tions like

Soft Corn It usually occurs between 4th and 5th toes due to friction of bases of adjacent proximal phalanges.

™™ Recurrent attacks of acute and subacute paronychia

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: ¾¾ Regular dressing and packing. ¾¾ Antibiotics. Discharge is sent for culture and sensitivity. ¾¾ Nails should be cut concavely or straight without leaving lateral spikes towards soft tissues. ¾¾ Partial or complete matrixectomy: 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 inser-

tion of the plantar fascia which is common in people who stand or walk for long-time. ™™ Treatment: Analgesics, rest, steroid injections to the site.

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.

Fig. 1.289: Zadik‘s or Fowler‘s operation.

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CHAPTER 1K    General Surgery: Hand and Foot

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 smaller lesion 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: ¾¾ Excision. ¾¾ Local application of salicylic acid preparations or mixture of salicylic acid/lactic acid/collodion may be helpful. Skin softening agents are also tried. ¾¾ Eliminating the pressure is very important to prevent recurrence. ¾¾ Avoid excision of corn unnecessarily in diabetic (especially with neuropathy) and in ischaemic foot.

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A

Fig. 1.291: Hallux valgus deformity.

B

C

Figs. 1.290A to C: (a and B) Ingrowing toe nail. Note the granuloma caused by repeated infection and inflammation; (C) Incision for 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 Horn Nail); 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 the toes—Tinea pedis. ™™ Fungi enter through 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 bunion and splaying of forefoot. ™™ X-ray shows deviation with often osteoarthritis of the metatarsophalangeal joint. ™™ Treatment ¾¾ Keller’s operation: Proximal 1/3rd 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. ¾¾ 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. ¾¾ Simmond’s procedure: Varus osteotomy at the base of 1st metatarsal bone with reinsertion of adductor hallucis tendon is done. ¾¾ 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. ¾¾ Arthrodesis of metatarsophalangeal joint is done to relieve pain. ¾¾ Excision of bunion, deformity correction, osteotomy, muscle transfers are also done as a combined approach.

L. Arterial Diseases

C hapter Outline ·· S  urgical Anatomy of Thoracic Outlet ·· Arteries of Upper Limb ·· Arteries of Lower Limb ·· Arterial Diseases ·· Intermittent Claudication ·· Rest Pain ·· Limb Ischaemia ·· 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 Ischaemia 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 superior aperture of thorax subclavian vessels, brachial plexus traverse the cervicoaxillary canal to reach the upper limb. Cervicoaxillary canal is divided into proximal Costoclavicular space and distal axilla (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 brachial 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 brachial 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 passing 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.

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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 compart­ment 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. ¾¾ ¾¾

INTERMITTENT CLAUDICATION Claudio means “I limp” a Latin word. It is a crampy pain in the muscle seen in the limbs. 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 P and metabolites. During exercise increased perfusion and increased opening of collaterals wash the metabolites.

B xx

xx xx

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 III: Patient has to take rest to relieve the pain

B xx xx

A

B

Figs. 1.293A and B: CT angiogram of aortoiliac segment showing aortoiliac block due to atherosclerosis. Collaterals are also welldeveloped.

ARTERIAL DISEASES ™™ Stenosis due to trauma, atherosclerosis, emboli. It may be: ¾¾ ¾¾

In the brain causing transient ischaemic attacks. In the limbs causing claudication and rest pain.

xx

Boyd’s classification of claudication

Claudication

Arterial—typically develops after walking for certain distance and resolves rapidly within 5 minutes once walking is stopped Neurogenic—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

Note: Beta blockers may aggravate claudication. Claudication is not that common in upper limb but can occur during writing or any upper limb exercise.

• •

•

•

B xx xx xx xx xx xx xx

Rest Pain ™™ It is continuous aching in calf or feet and toes or in the region

even at rest depending on site of obstruc­tion. ™™ It is ‘cry of dying nerves’ due to ischaemia of the somatic

nerves. It signifies severe decompensated ischaemia. Pain gets aggravated by elevation and is relieved in dependent position of the limb. ™™ Pain is more in the distal part like toes and feet. It gets aggra­ vated with movements and pressure. ™™ Hyperaesthesia is common association with rest pain. ™™ Rest pain is increased in lying down and elevation of foot; it may be reduced on hanging the foot down. ™™ Rest pain is worst at night and so patient is sleepless at night. During sleeping at night heart rate and blood pressure diminishes which further aggravates the hypoperfusion and rest pain. ™™ Rest pain is apparently reduced by holding the foot with hand, probably due to suppression of transmission of pain sensation. ™™ Anaerobic muscle metabolism occurs even at rest mainly affecting the foot and leg.

Features of arterial stenosis/block in limbs

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

Limb Ischaemia Causes ™™ Atherosclerosis. ™™ Embolism (acute). ™™ Arteriopathies—Buerger’s disease, Raynaud’s disease,

Takayasu’s disease. ™™ Diabetes. ™™ Scleroderma. ™™ Physical agents—trauma, tourniquet, radiation injury.

Classification of Limb Ischaemia

B xx xx

xx xx

Fontaine classification of limb ischaemia

Stage 1: No clinical symptoms Stage 2: Intermittent claudication –– 2a: Well-compensated—can walk >200 metres –– 2b: Poorly compensated—walk only 2 weeks or ulceration or gangrene of the foot or toes with an ankle systolic pressure 200 mg%; high LDL (>100 mg%); low HDL ( 70% narrowing (severe) causes reduced blood flow. Ischaemia, 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 zones → Complicated plaque with calcification, ulceration, narrowing

Management ™™ Risk factor modification: Avoid smoking; control of hyperten-

sion, 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: ¾¾ Thrombectomy, endarterectomy, profundaplasty. ¾¾ Reverse/saphenous vein graft. ¾¾ By pass grafts—iliofemoral, aortofemoral, iliopopliteal, femorofemoral grafts. ¾¾ 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. Omental vessels as such are often poorly perfused in atherosclerotic patients due to involvement of coeliac trunk.

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.

Types of Aortoiliac Occlusive Disease ¾¾

¾¾

Infrainguinal Arterial Occlusive Disease It is either part of type III 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. Fig. 1.309: Types of aortoiliac occlusive disease.

Management ™™ If the popliteal artery below knee is patent femoropopliteal

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.

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. ™™ Profundaplasty may be done to improve the collateral circulation through profunda femoris (deep femoral).

™™ Atheromatous plaque may dislodge and may cause embolus

causing acute presentation.

™™ Aortic angiogram is diagnostic.

Management ™™ Treatment for diabetes, hyperlipidaemia, etc. ™™ Surgical treatment is the mainstay. ¾¾

Direct anatomical reconstruction –– Aortoiliac endarterectomy is reboring/disobliteration procedure useful for type I disease. Diseased intima, plaque with 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

A

B

Figs. 1.310A and B: Aortofemoral bypass graft.

Character is like a tree; reputation is like its shadow. The shadow is the one we think of but actually tree is the real thing.

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Type I: Disease localised to distal abdominal aorta and common iliac arteries. Type II: Wide spread aortic and iliac disease. Type III: 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.

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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 a disease 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 III 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.

Pathogenesis Smoke contains carbon monoxide and nicotinic acid                                         ↓ ← Carboxyhaemoglobin Causes initially vasospasm and hyperplasia of intima ↓ Thrombosis and so obliteration of vessels occur, commonly medium-sized vessels are involved. ↓ Panarteritis is common Usually involvement is segmental ↓

Eventually artery, vein and nerve are together involved ↓ Nerve involvement causes rest pain ↓ 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 ↓ It is called as compensatory peripheral vascular disease ↓ 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 hyperplasia → thrombosis → 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 Number of cigarettes smoked per day of smoking SI >300 is a risk factor Pack Years Index (PYI) =    Number of years Number of packets of    of smoking cigarettes per day PYI >40 is a risk factor Shianoya’s criteria for Buerger’s disease xx Tobacco use. Only in males xx Disease starts before 45 years xx Distal extremity involved first without embolic or atherosclerotic features xx Absence of diabetes mellitus or hyperlipidaemia xx With or without thrombophlebitis

Classification of TAO Type I: Upper limb TAO—rare. Type II: Involving leg/s and feet—crural/infrapopliteal. Type III: Femoropopliteal. Type IV: Aortoiliofemoral. Type V: Generalised.

Clinical Features ™™ Common in male smokers between the 20–40 years of age

group. It is a smoker’s disease.

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™™ Intermittent claudication in foot and calf pro­gressing to rest

Fig. 1.312B Figs. 1.312A and B: Bilateral lower limb TAO causing gangrene of both feet. Patient needs amputation on both sides.

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.

Investigations ™™ Hb%. 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 off 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. 1.313: Gangrene foot. ™™ Transbrachial angiogram: If femorals are not felt, then trans-

brachial angiogram (through left side brachial artery—left subclavian artery—and so to descending aorta) should be done. ™™ Ultrasound abdomen to see abdominal aorta for block/ aneurysm. ™™ Vein, artery, nerve biopsy.

A Fig. 1.312A

Fig. 1.314: Gangrene of all toes in a foot in TAO patient.

The art of seeing invisible things is called as ‘clinical vision’.

CHAPTER 1L    General Surgery: Arterial Diseases

B Fig. 1.311: Dry gangrene of leg. Patient needed above knee amputation.

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Fig. 1.315: Ischaemic 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: Ischaemic features in upper and lower limbs (four limbs).

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 ™™ Low dose of aspirin 75 mg once a day—antithrombin activity. ™™ Prostacyclins, ticlopidine, praxilene, carnitine. ™™ Clopidogrel 75 mg; atorvastatin 10 mg; parvostatin 40 mg;

Fig. 1.318: CT angiogram of lower limb (leg area) showing segmental block.

cilostazole 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: Intramuscular injection of vascular endothelial

Note: Vasodilators and anticoagulants are of no use in TAO. Drugs like pentoxiphylline increases the flexibility of RBC’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).

• •

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 circulation.

B

Buerger’s exercise

Patient is in supine position, legs elevated to 45 degree. Time taken for blanching 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 sequence 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 should 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 vasodilatation 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 IVC/aorta, spinal cord ischaemia.

Surgery

Fig. 1.321: Bilateral TAO. Patient has undergone right-sided above knee amputation and left-sided lumbar sympathectomy. Ischemic 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. ™™ Ilzarov 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—Ophthalmologist, Japan) ™™ 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.

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.

™™ Omentoplasty to 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 sympathectomy to increase the cutaneous perfusion so as to promote ulcer healing. But it may divert blood from muscles towards skin causing muscle more ischaemic.

™™ 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.

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growth factor (VEGF) which is an endothelial cell mitogen that promotes angiogenesis.

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™™ Treatment: ¾¾

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To suppress immunity prednisolone 50 mg/day and cyclophosphamide daily is given. Vascular reconstruction.

RAYNAUD’S PHENOMENON Under the influence of a very moderate cold, and even at the height of summer, she (case 1) sees her fingers become ex-sanguine, completely 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. —Maurice Raynaud, 1862 (French Surgeon)

“Raynaud’s phenomenon” is an episodic, localized, vasospasm (vasoconstriction) of small vessels (arteriolar vasospasm) of hands and feet that leads into temperature and colour changes with sequence of clinical features called as ‘Raynaud’s syndrome’ as initial pallor (blanching; vasospasm); then blue/ cyanosis (venous congestion); lastly red (congestion due to rapid blood flow, reactive hyperaemia).

B

Raynaud’s syndrome

Sequence of clinical features due to arteriolar spasm. xx Local syncope: It is due to vasospasm, causing white cold palm and digits along with tingling and numbness xx 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) xx Local recovery: It is due to relief of spasm in the arteriole, leading to return of blood to the circulation causing flushing and pain in digits and palm (pain is due to increased tissue tension) xx 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”.

Causes for Raynaud’s Phenomenon ™™ Raynaud’s disease:

It is seen in females, usually bilateral. It occurs in upper limb with normal peripheral pulses. ¾¾ 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. ¾¾ 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. ¾¾ ¾¾

Fig. 1.322: Vasculitis can cause arterial insufficiency. ™™ Collagen vascular diseases: Like scleroderma, rheumatoid

diseases causing vasculitis (all autoimmune diseases). ™™ Other causes: Cervical rib, Buerger’s disease, Scalene syndrome. It is often associated with CREST syndrome (Calcinosis cutis, Raynaud’s phenomenon, Esopha­geal defects, Sclerodactyly, Telangiectasia).

B �

Types of Raynaud’s phenomenon

Vasospastic

�  Obliterative

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 are–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.

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

Investigations ™™ Type is identified by angiogram of hand (DSA/MR angio-

gram), 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 capillary microscopy; laser Doppler flux to assess microvascular perfusion of finger skin—are special methods of evaluations. ™™ Nailfold capilloscopy (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 ™™ Treat the cause. ™™ Avoid precipitating factors—protect from cold/proper dress/

hand warmer electrical or chemical/hand gloves. Avoid smoking even though it is not direct etiological cause (other than upper limb TAO), but it may possibly aggravate the disease. Avoid vibrating tools. ™™ Vasodilators/pentoxiphylline/low dose aspirin (75–100 mg per/day). Calcium antagonist (nifedipine 20 mg) is useful. Steroids may be useful in case of secondary Raynaud’s. ™™ ACE inhibitors, nitrates, endothelin inhibitors (bosentan), epoprostenol—prostaglandin a potent vasodilator (Iloprost, a prostaglandin analogue) and antiplatelet drug (continuous intravenous infusion can be given), iloprost—prostacycline analogue, PG E1, misoprostol (oral PG E 1)—are all tried at different stages of the disease. 1% topical glycerine trinitrate is useful. ™™ Cervical sympathectomy—is used for nonhealing digital ulceration. Not very beneficial to Raynaud’s syndrome. ™™ Localized digital sympathectomy by stripping off the adventitia of digital vessels often with that of radial and ulnar arteries with resection of nerve of Henle (a branch of ulnar nerve in the forearm—is sympathetic innervation of the ulnar artery) is said to be effective. Procedure is done using operative microscope. Note: Avoid oral contraceptives, beta blockers and ergot preparations in Raynaud’s disease.

TEMPORAL ARTERITIS

177

™™ 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, tender, 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.

TREATMENT OF ARTERIAL DISEASES 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.

Drugs ™™ Nifedipine, praxilene, pentoxiphylline, low dose aspirin,

prostacycline, dipyridamole, ticlopidine. ™™ Clopidogrel (75 mg). ™™ Cilostazol (type III phosphodiesterase inhibitor) 100 mg

BD—inhibits platelet aggregation. ™™ Oral anticoagulants are used only if there is history of embolism or atrial fibrillation. ™™ Prostaglandins, growth factors, vascular endothelial growth factor (VEGF), E2Fdecoy (blocks intimal and smooth muscle cell proliferation), mesoglycan (breaks blood clot), testosterone, herbals like garlic (reduces viscosity of blood) are other newer drugs under use and trial. ™™ B vitamins and folic acid reduces homocysteine level (which is a risk factor). ™™ Inositol, L-carnitine (1500 mg), magnesium 500 mg (not in renal failure or with diarrhoea), vitamin E and C are other agents often used to improve walking distance. ™™ Heparin is used only in acute phase or embolism.

b. Surgery ™™ Percutaneous transluminal balloon angioplasty (PTA):

Through trans­femoral Seldinger approach, initially angiogram is done. Then under guidance (fluoroscopic) stenosed area is approached. First guidewire is introduced through which

Not just ‘Go’ through the life, but better ‘Grow’ through the life.

CHAPTER 1L    General Surgery: Arterial Diseases

presentation. Occasionally, if vasospasm becomes longer, gangrene or ischaemic ulceration supervenes along the tips of the fingers. ™™ Peripheral pulses (radial/ulnar) are normally felt. These pulses will be absent in upper limb TAO. ™™ Repeated attacks are common.

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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 ¾¾ Conventional: Here balloon is inflated along the lumen to break the plaque circumferentially. ¾¾ Subintimal: Here balloon is inflated after passing subintimal plane to break the plaque.

Fig. 1.324: Percutaneous transluminal balloon angioplasty (PTA). Note the inflated balloons on both side iliac arteries.

Fig. 1.323: Conventional and subintimal types of PTA.

Complications ¾¾ ¾¾ ¾¾

Thrombosis, bleeding, sepsis. Embolism, dissection, retroperitoneal haematoma. Pseuodoaneurysm formation.

Advantages ¾¾ It is done under local anaesthesia. ¾¾ Procedure can be repeated if needed. ¾¾ Stent can be placed at a later stage if needed. ¾¾ It is done when stenosis is less than 5 cm. In ideal indications its efficacy is equal to surgery.

A

B

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. ¾¾ 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: ¾¾ It is removal of thrombus along with diseased intima through an arteriotomy. Endothelium of the vessel is removed, hence the name. ¾¾

A

B

Figs. 1.326A and B: DSA showing superior mesenteric artery stenosis. It is corrected by PTA.

179

Fig. 1.329: Profundaplasty for deep femoral block.

™™ Profundaplasty: ¾¾

Fig. 1.328: Endarterectomy technique. Both thrombus and diseased intima are removed through an arteriotomy. Often ring stripper is used to remove the atheromatous plaque. ¾¾

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 segment; endarterectomy is done by removing thrombus, diseased intima with plaque along the plane of media. Arteriotomy is closed using 5-zero polypropylene suture and patient is heparinised. (2) Semiclosed method—Here two arteriotomies 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 closed. (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 everted out to extract the diseased intima like a tube; 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 for localised stenosis.

It is done when there is localised block in opening of profunda 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.) ¾¾ 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 femoropopliteal region to bypass the blood across. Venous valves are removed through valvulotomy instrument so as to allow the blood to pass. Here nutrient supply 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

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Fig. 1.327: Thrombectomy.

180

™™ Arterial/venous grafts:

Synthetic: Dacron woven graft xx Dacron knitted graft xx Polytetrafluoroethylene (PTFE) graft Natural: xx Internal mammary artery (ideal one) xx Long saphenous vein either reverse or in situ xx Umbilical vein graft (cryopreserved)—3 mm vein is the minimum diameter required Grafts of different length and size are available

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xx

¾¾

¾¾

Different procedures: Aortofemoral bypass graft (end to side)—5% mortality; Ileofemoral 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 sympathetic 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 Horner’s syndrome. For hyperhidrosis of axillary area, along with stellate ganglion upper four thoracic ganglia has to be removed.

Note: Angioscope is used to visualise the valves in saphenous vein or to visualise the completion of the by pass grafts like femorodistal graft.

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)

Fig. 1.333: On table picture of aortofemoral arterial graft.

B xx xx xx xx xx

Fig. 1.331: Femoropopliteal bypass graft.

xx xx

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

B xx xx xx xx xx xx

Complications

Bleeding Injury to subclavian artery and nerves Pneumothorax and haemopneumothorax Horner’s syndrome with ptosis, miosis, anhydrosis, enophthalmos Chylous fistula, chylocele Post-sympathetic neuralgia

Transthoracic/Axillary 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. ¾¾ Thoracoscopic sympathectomy is the choice, and popular approach at present. Advantages are better visibility with magni­fication, less trauma of access (wound), faster recovery, and precise. ¾¾

™™ Lumbar sympathectomy:

Indications ¾¾ Peripheral vascular disease like TAO. ¾¾ To promote healing of cutaneous ulcers. ¾¾ To change level of amputation and to make flaps to heal better after amputation. ¾¾ Causalgia of lower limb (it is common in upper limb). ¾¾ Hyperhidrosis of lower limb is rare. Principle ¾¾ It increases the cutaneous blood supply 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. ¾¾ 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 healing; 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. Inferior 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 xx xx xx xx xx xx xx

Injury to IVC 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 ¾¾ ¾¾

¾¾

Fig. 1.334: Cervical sympathectomy scar in the neck and patient has developed Horner’s syndrome. Healed ulcer over tip of index finger on left side is seen. Patient is asymptomatic after sympathectomy.

Complications

Its effects are only temporary (3–4 weeks). Long-term results are doubtful. 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.

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

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Approaches ¾¾ Supraclavicular approach: Through a supraclavicular 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.

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C 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. ™™ Chemical sympathectomy: ¾¾

¾¾

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 xx xx xx

Indications

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

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.

Fig. 1.337: Incisions for omentoplasty in the abdomen (upper midline) and in lower limb.

B xx xx xx

A

B

Figs. 1.336A and B

Complications of omentoplasty

Abdominal sepsis Incisional hernia, where omental pedicle is tunneled into the limb from the abdomen Adhesions and intestinal obstruction

Procedure: Under general anaesthesia, abdomen is opened 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 rectus muscle. Later this pedicle is mobilised in the subcutaneous tunnel across the leg, burried in the deep fascia. ™™ Other treatment methods: ¾¾ Amputations are done at different levels depending on extent of gangrene, site of block, amount of collaterals.

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, paraesthesia and fatigue in the arm which is aggravated by exercise (arm claudication often with exercise). Sudden turning of the neck to the affected

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.

Causes ™™ 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.

Pathophysiology 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. Ischaemia 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.

Features Fig. 1.339: Subclavian steal syndrome. (CCA: common carotid artery)

™™ 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

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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.

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; Endarterectomy or Surgery— bypass graft (Common carotid—subclavian graft).

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™™ Pulselessness—sudden loss of earlier palpable pulse.

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™™ 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.

TRAUMATIC ACUTE ARTERIAL OCCLUSION ™™ Causes: Thrombus due to trauma; Subintimal haematoma; Acute

compartment syndrome; During femoral or brachial 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: ¾¾ 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. ¾¾ Proper antibiotics and heparin are required to prevent thrombosis of the vessel. Later patient is advised to take oral warfarin for maintenance. ¾¾ 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. ¾¾ 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 emboli are 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.

B xx

xx

xx xx

xx

Sources

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 investigations source is not found

Effects of Arterial Embolism xx xx xx xx xx xx

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, paraesthesia, paresis, ulceration, gangrene Most common site of arterial emboli is common femoral artery

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%).

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 Embolism

Thrombosis

Source

Present

Not present

Pulse

Proximal and contralateral pulses normal Severely cold

Ipsilateral and opposite side pulses may be absent

Angiography

Sharp cut off sign

Diffuse tapered disease

Collaterals

Very few—not well-developed

Well-developed

Temperature

Cold or normal

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 brachial.

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 IIa: Marginally threatened—no pain; numbness/paraesthesia; no audible Doppler signal. Venous flow present. Class IIb: Immediately threatened—persistent pain; sensory and motor loss; no Doppler signal. Venous flow present. Class III: Irreversible—paralysis and anaesthesia. No venous flow.   Ischaemia up to class IIb with normal venous flow is called as Early; ischaemia which is class III, 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 tried.

Treatment Treatment of Embolism and Thrombosis of Acute Limb Ischaemia Immediate infusion of 5000–10,000 units of IV heparin and relief of pain are needed first.

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™™ 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 for acute thrombosis (Standard is thrombolysis, Dotter and co, 1974). Endovascular therapy ™™ Intrarterial 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 ¾¾ It is done as early as possible as an emergency operation. ¾¾ 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. ¾¾ Postoperatively initially heparin and later oral anticoagulants are used. Procedure is done under general anaesthesi a or local anaesthesia. ¾¾ 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: ¾¾ 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. ¾¾ 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 infusion 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. ¾¾ Tissue plasminogen activator (TPA): Alteplase, Reteplase—here lysis occurs in 24 hours. TPA is better

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Features

Surgical

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and ideal; 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 History suggestive of or confirmed active duodenal/gastric ulcers Uncontrolled hypertension or coagulation disorders

T

Advantages and disadvantages of thrombolysis

Advantages

Disadvantages

• Gentle angiographic clot removal

• Useful only for class I and IIa acute ischaemia

• Survival and limb salvage is equal

• Bleeding at the site and elsewhere is possible

• It is mainly useful for acute thrombus

• 25% rate of failure

• For embolus it is used often as an adjunct along with embolectomy • It avoids surgery

Anticoagulant therapy ™™ It is to prevent recurrent emboli formation. Immediate infu-

sion 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 Ischaemic Limb ™™ Reperfusion injury ™™ ‘No reflow’ 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 venules 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 common 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 mmHg 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: ¾¾ Mannitol to prevent renal failure; fluid therapy. ¾¾ 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. ¾¾ Antibiotics and supportive therapy.

SADDLE EMBOLUS ™™ It is an embolus blocking at bifurcation of aorta. ™™ Causes: Mural thrombus after myocardial infarction; Mitral

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.

Fig. 1.341: Saddle embolus blocking the bifurcation of abdominal aorta. It causes severe, rapid, dramatic symptoms. (EIA: external iliac artery; CIA: common iliac artery; IIA: internal iliac artery)

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™™ The embolus which blocks at aortic bifurcation is usually large. ™™ Features:

Features of sudden, rapidly progressive ischaemia in both lower limbs. ¾¾ Gangrene of both lower limbs. ¾¾ Features of associated infection. ™™ Investigations: Arterial Doppler, aortic angiogram; Ultrasound abdomen. ™™ Treatment ¾¾ Initially, heparin is injected intravenously—10,000 units and later 5,000 units subcutaneously 8th hourly. ¾¾ Embolectomy can be done using Fogarty’s catheter. ¾¾ Open arteriotomy and embolectomy can also be tried. ¾¾ Antibiotic prophylaxis is given to prevent infection. ¾¾

EMBOLECTOMY ™™ Indications ¾¾ ¾¾

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.

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.

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: ¾¾ Interventional method is usually employed using Fogarty’s catheter. Good back bleed signifies com­plete­ness of embolectomy. ¾¾ 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. ¾¾

Note: Intraoperative arteriogram is a must to confirm the adequacy of blood flow and completion. Intraoperative thrombolysis as an adjunct to save the limb using urokinase 2,50,000 IU for minutes into distal artery may be beneficial. Prophylactic fasciotomy 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.

• • • • •

FAT EMBOLISM (Ernst Von Bergmann, in 1873) ™™ 90% of major trauma especially with fractures develop fat

Fig. 1.342: Embolectomy technique.

A

Fig. 1.343A:

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 fat 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.

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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.

B

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Features

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™™ 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 >110/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

AIR/GAS EMBOLISM 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.

Features It causes respiratory distress, haemoptysis, convulsions, unconsciousness, visual and hearing disturbances, fatigue and numbness, paralysis, haemodynamic instability and coma. xx

xx

xx xx

™™ Differential diagnosis: Pulmonary embolism; Thrombotic

thrombocytopenic purpura.

™™ Investigations:

Presence of fat lobules in the blood obtained by pulmonary capillary wedging is diagnostic. ¾¾ Haemoglobin estimation, platelet count and total WBC count. ¾¾ Chest X-ray shows snow storm appearance. ¾¾ CT chest is useful; CT head is done to rule out causes of intracranial injuries. ¾¾ Transesophageal echocardiography (TEE) may be of use in evaluating the intraoperative release of marrow contents into the bloodstream during intramedullary reaming and nailing. ¾¾ Bronchoalveolar lavage with staining for fat will show lipid inclusions. ™™ Treatment: ¾¾ Adequate oxygenation with ventilator support (ICU care). ¾¾ Hydration, nutrition, achieving haemodynamic stability, prevention of DVT, avoiding volume overload with proper fluid therapy. ¾¾ Methylprednisolone may be useful; but use of heparin, low molecular dextran and other steroids are controversial even though commonly used. ¾¾ Albumin transfusion may be helpful as it binds with fatty acids to reduce the lung injury.

Early fixation of the fractures; placement of IVC filters will prevent the chances of fat embolism or emboli reaching into the lungs.

xx

¾¾

xx

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 of air at a rate more than 100 ml/second is fatal. When the air enters the right atrium, it gets churned up forming 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 septal 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.

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.

¾¾

B xx xx xx xx xx

B xx

™™ Haemangiomas; AV fistulas. ™™ Malignancies like renal cell carcinoma, hepatoma. ™™ Cerebrovascular problems. ™™ To arrest haemorrhage from GIT, urinary and res­piratory tract. ¾¾

of the sac and it usually occurs after trauma.

xx

Indications

xx

Types

Fusiform—uniform dilatation of entire circumference of arterial wall Saccular—dilatation of part of circumference of the arterial wall Dissecting—through a tear in the intima blood dissects between inner and outer part of tunica media of the artery

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, secon­ daries in liver (mainly due to carcinoids), hepatoma. Materials used for therapeutic embolisation

Blood clot Gel foam Balloons Quick setting plastics Stainless steel coils

xx xx xx xx

Human dura Plastic microspheres Ethyl alcohol Wool

Fig. 1.344: True and false aneurysms. In true type, all layers are intact. In false type all layers breached with haematoma having a false capsule.

CAISSON’S DISEASE or DECOMPRESSION DISEASE (BEND’S DISEASE) 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 TypeI/(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: ¾¾ Oxygen therapy; Recompression and gradual decompression in special chamber.

Fig. 1.345: Fusiform and saccular types of aneurysms.

ANEURYSM There is no disease more conducive to clinical humility than aneurysm of the aorta. —William Osler, Circa 1900 ™™ 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.

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Fig. 1.346: Thoracic aortic aneurysm.

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THERAPEUTIC EMBOLISATION

™™ False aneurysm contains single layer of fibrous tissue as wall

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™™ 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. ¾¾ Congenital: –– Berry aneurysm; cirsoid aneurysm; 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 circle of Willis.

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™™ 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.

Effects and complications of aneurysm

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

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.

A

Fig. 1.347: Chest X-ray showing aortic aneurysm.

Differential Diagnosis ™™ 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.

B

C 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.

Investigations ™™ Doppler study, duplex scan, CT angiogram, DSA. ™™ Tests relevant for the cause, like blood sugar, lipid profile,

MYCOTIC ANEURYSM (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

Fig. 1.349: Right renal artery aneurysm on CT angiogram.

Fig. 1.350: Cerebral CT angiogram showing (intracranial) Berry’s aneurysm.

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—Anel’s; ligation proximally proximal to an arterial branch—Hunter’s; ligation just distal to aneurysmal sac—Brasdor’s; ligation distally distal to an arterial

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 intima of the artery (femoral). ™™ Common vessels involved are aorta, visceral, head and neck and intracranial. ™™ 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 (EVAR) often with covered endovascular reconstruction of aortic bifurcation (CERAB) is becoming more commonly used nowadays as less invasive effective therapy. Note: Microbial arteritis with aneurysm is a different entity is due to bacteraemia occurring in an athero­sclerotic vessel due to Salmonella infection.

ABDOMINAL ANEURYSM ™™ Abdominal aortic aneurysm is the most common aortic aneu-

rysm. 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.

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echocardiography.

branch—Wardrop’s; ligature one proximal and another distal to aneurysmal sac—Antylus’).

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ABDOMINAL AORTIC ANEURYSM (AAA) AAA is a localised, progressive and permanent dilatation usually larger than 3 cm in diameter of the abdominal aortic wall.

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 III collagen, α1 antitrypsin and lysyl oxidase. Marfan’s, Ehler Danlos syndromes are related genetically. ™™ Others: Syphilis, dissection, trauma, collagen diseases, infection, arteritis, cystic medial necrosis, association with Chlamydia pneumoniae (55%). xx

xx

Classification I –– Infrarenal—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 supine as 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 retroperitoneal 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 (GI) 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 kidney which is anterior to aorta is difficult to manage. Left retroperitoneal approach is needed. Endovascular aneurysm repair (EVAR) is not possible.

Investigations ™™ Blood urea, serum creatinine. ™™ US (most widely used noninvasive test; but neck of the aneu-

rysm, 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 more reliable and precise investigation of choice—gives better information regarding extent on sides/ neck, size, dimensions, size and 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 xx xx xx xx

Complications of abdominal aortic aneurysm

Rupture, infection Thrombosis, embolism, distal ischaemia/gangrene Aortocaval fistula, aortoenteric fistula formation Erosion of vertebra, spinal cord ischaemia when thrombosis develops

Surgical Treatment

A

™™ Indications for surgery ¾¾ ¾¾ ¾¾ ¾¾

T

Asymptomatic aneurysm more than 5.5 cm. Growth rate more than 0.5 cm/year. Painful, tender aneurysm. Thrombosed aneurysm, aneurysm with distal emboli.

Low-risk, intermediate-risk and high-risk of abdominal aortic aneurysm

Low-risk abdominal aortic aneurysm—age below 70 years; active physically without cardiac, respiratory, renal impairment and noninflammatory aneurysm. Here surgical mortality is 3 mg%; liver failure status. Here surgical mortality is >10%. ™™ Open surgical repair ¾¾

¾¾

Fig. 1.352: Abdominal aortic aneurysm showing widened aortic wall with thrombosis in the periphery in CT angiogram.

Differential Diagnosis ™™ Retroperitoneal mass, pseudocyst of pancreas, retroperito-

neal cyst mimic abdominal aortic aneurysm especially when it is thrombosed. ™™ Mesenteric ischaemia, acute pancreatitis, perforated duodenal ulcer may mimic ruptured aneurysm. ™™ Other conditions causing back pain like disc prolapse, sciatica.

Treatment Conservative/medical Treatment ™™ It is done in low-risk abdominal aortic aneurysm (age below

70 years; active physically without cardiac, respiratory, renal

¾¾

It is called as endo-aneurysmorrhaphy with intraluminal graft placement (Crawford, 1960). It is done under GA with epidural support. Major challenges during anaesthesia are—blood loss, haemodynamic control, problems during clamping and declamping of aorta, temperature control, renal hypoperfusion, left ventricular strain. Incision is commonly lengthy midline transperitoneal or supraumbilical transverse. Retroperitoneal approach is used in horseshoe kidney, abdominal wall stoma, inflammatory aneurysm, suprarenal extension, peritoneal dialysis, hostile abdomen. Retroperitoneal approach favors rapid control of proximal aorta but prevents visualisation of abdomen. After laparotomy, duodenum and small bowel are retracted laterally and above; left renal vein which is in front of aorta is dissected and retracted; occasionally it may require to be ligated and it is safer provided left gonadal and left suprarenal veins are intact. Distal arterial clamps are applied first along common or external and internal iliac arteries on both sides. Proximal aortic clamp is applied at infrarenal level. Aorta is opened longitudinally midline towards right to avoid injury to orifice of inferior mesenteric artery. Atheroma, thrombus is removed until

A positive thought is a seed of positive result.

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impairment and noninflammatory aneurysm); if aneurysm size is 20 mmHg xx Proximal thrill or bruit xx Mass in the neck, thrill and bruit in the neck in supraclavicular region xx Adson test, hyperabduction (Halsted) test, Roos test, Allen’s tests are important xx

a. Acute type Causes: ™™ Embolism—common: 30% of the peripheral emboli lodge in upper extremity. the most common site is at the bifurcation of brachial artery (40%); next common is at axillary artery (12%). Embolism is due to: ¾¾ Cardiac origin (70%)—valvular lesions (atrial fibrillation, endocarditis), IHD, paradoxical. ¾¾ Others—aneurysms, thoracic outlet syndrome, plaque. ™™ Trauma—most common: Brachial artery injury is seen in 30% of civilian trauma with arterial injuries, blunt injuries, fractures and dislocations, penetrating injuries. ™™ Iatrogenic.

B xx

Symptoms of acute ischaemia

Pain, pallor, poikilothermia, paraesthesia, paralysis.

Fig. 1.369: Gangrene of left index finger. Patient has undergone cervical sympathectomy.

Investigations in Upper Limb Ischaemia ™™ Laboratory tests for vasculitis, hypercoagulable states, and

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.

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.

Bypass Surgeries in Upper Limb Ischaemia ™™ Conventional bypass

Fig. 1.370: Upper limb angiogram showing blocks in subclavian artery.

Management of Upper Limb Ischaemia ™™ Treatment of the cause.

Treatment of Embolus ™™ Time since the first symptom is very important. ™™ Clinical assessment of extent of ischaemia, immediate antico-

Aorto-subclavian/axillary bypass. Subclavian-Axillary/brachial bypass. ¾¾ Brachiodistal bypass. ™™ Extra-anatomical bypass ¾¾ Carotid: Subclavian/Axillary bypass. ¾¾ Subclavian: Subclavian bypass. ¾¾ Axillary: axillary bypass. ™™ Subclavian: Carotid transposition. ¾¾ ¾¾

agulation with heparin, Doppler study and angiogram of the arterial system, evaluation for the source of embolus—are the protocols. ™™ Embolectomy ¾¾ Brachial 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.

¾¾ ¾¾ ¾¾ ¾¾

B

Figs. 1.371A and B: (A) Carotid subclavian bypass; (B) Subclavian carotid transposition.

Treatment of Raynaud’s Phenomenon ™™ Avoiding triggering agents. ™™ Drugs (vasodilators)—calcium channel blockers, angiotensin

Treatment of chronic Ischaemia ™™ Medical management

A

Risk modification—diabetes, hypertension, dyslipidemia, smoking, homocystinaemia, exercise training. Antiplatelets—aspirin/ticlopidine/clopidogrel. Anticoagulants—heparin/warfarin. Xanthines/pentoxiphylline/cilostazol.

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.

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CHAPTER 1L    General Surgery: Arterial Diseases

Cilostazol—suppresses cAMP phosphodiesterase III rise in cAMP levels with antiplatelet, antithrombotic effects; induces vasodilatation; increases plasma HDL cholesterol; decreases plasma triglycerides. ™™ Catheter-based interventions ¾¾ Atherectomy. ¾¾ Angioplasty + stenting by conventional or subintimal approach. ¾¾ Stent grafts. ¾¾ Cryoplasty. ™™ Surgery ¾¾ Endarterectomy; Bypass surgery; Sympathectomy, extraperiosteal resection of the cervical rib.

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xx xx xx xx xx xx xx xx xx xx

Upper limb ischaemia

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

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

Classification of Arterial Substitutes ™™ 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 ¾¾ 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. ¾¾ Non-textile semi-inert polymer graft: –– ePTFE graft—expanded polytetrafluoroethylene graft. Preclotting 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.

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

C hapter Outline ·· Vascular Anomalies ·· Haemangioma ·· Vascular Malformations

·· 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 culture 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 feature. 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 imaging— coloured 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.

You may be disappointed if you fail, you are doomed if you don’t try.

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™™ 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.

B xx xx

Haemangioma classification (old)

Capillary –– Strawberry haemangioma Cavernous haemangioma

¾¾

¾¾

¾¾

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 Sturge-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 haemangioma’ is restricted to strawberry type only; salmon patch and port-wine stain are actually classified under vascular malformations.

Note: Salmon patch and port-wine stain are actually capillary vascular malformations even though they were earlier classified under capillary haemangioma.

Capillary Vascular Malformations ™™ Salmon patch (stork bite): ¾¾

¾¾

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).

Capillary Haemangioma ™™ Strawberry haemangioma: ¾¾

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 intravascular thrombosis, fibrosis and mast cell infiltration.

Fig. 1.372: Salmon patch. ™™ Port-wine stain (Naevus flammeus): ¾¾

¾¾

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 nodular 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.

¾¾ ¾¾ ¾¾ ¾¾ ¾¾ ¾¾ ¾¾ ¾¾ xx

xx

xx xx xx xx

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

B xx xx

xx xx

xx xx xx

™™ They are treated by wait and watch policy commonly—

allowed 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/CO2 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 steroid therapy. ™™ 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.



Associated syndromes

Klippel-Trenaunay-Weber syndrome: Naevus flammeus + osteo­ hypertrophy 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 + dyschondro­ plasia 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)

Cavernous Haemangioma

Treatment

™™ 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).

A

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Uncontrolled growth; Accidental haemorrhage Functional impairment like vision or hearing

B xx

Indications for surgery or intervention

B

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

CHAPTER 1M    General Surgery: Vascular Lesions

¾¾

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.

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™™ Features

It is smooth, soft, well-localised, warm, fluctuant, compressible, nonpulsatile swelling with bluish surface occurring in skin and subcutaneous tissue (often in mucosa like oral cavity) without any transillumination. ¾¾ 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. ¾¾ It is usually nontender unless it gets infected or undergoes thrombosis or causes haemorrhage. ™™ Differential Diagnosis ¾¾ Lymphangioma: It is brilliantly transilluminant unless it is infected or fibrosed. ¾¾ Lipoma, cold abscess, lymph cyst—clinically it is easier to differentiate.

SRB’s Manual of Surgery

¾¾

Fig. 1.377: Laparoscopic view of cavernous haemangioma of liver. It is the most common benign tumour of the liver.

™™ Complications: Haemorrhage; DIC; Thrombosis; Infection,

A

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 ¾¾ 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. ¾¾ Ligation of feeding artery and often at later stage excision is done once haemangioma shrinks. ¾¾ Therapeutic embolisation. ¾¾ If small and located in accessible area, excision is the initial therapy. ¾¾ Laser ablation—diode pulsed laser is becoming popular because of good control of bleeding. CO2/Nd:YAG laser is also equally effective.

VASCULAR MALFORMATIONS ™™ 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.

B

Figs. 1.376A and B: Cavernous haemangioma in the cheek near angle of the mouth and in the tip of the tongue. Haemangioma tongue is one of the causes of macroglossia.

™™ 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.

209

™™ Present at birth and grows in proportion to child’s growth.

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 / 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. ™™ Commonly seen in superficial temporal artery and its branches.

™™ 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 uncon-

trollable haemorrhage.

Fig. 1.378: Typical cirsoid aneurysm involving superficial temporal artery region; it is commonly congenital.

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 ¾¾ Ligation of feeding artery and excision of lesion, often requires preliminary ligation of external carotid artery. ¾¾ Intracranial extension requires formal neuro­s urgical approach. ¾¾ Endovascular therapy is also useful. ¾¾ 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.

T

Types of AVF

Congenital: AV malformation Acquired: traumatic

High flow: >800 ml/minute Normal flow: 400-800 ml/minute Low flow: 50 years). ™™ Painless progressive enlargement of lymph nodes. They are

smooth, firm, usually discrete (without matting), nontender, typically with India rubber consistency. ™™ Pain after alcohol consumption even though is very uncommon (3%) with low sensitivity but when present has got high specificity and is pathognomonic of HL. Pain appears within 3 minutes after alcohol intake in the vicinity of the involved node. Site: Cervical lymph nodes most common—82% (lower deep cervical group and in posterior triangle). Others include axillary, mediastinal, inguinal, abdominal. Axial lymphatics are commonly involved.

Specific Features ™™ Nodular sclerosis is most common type. ™™ Consecutive group of lymph nodes are involved. ™™ Splenomegaly is very common (45%). ™™ Hepatomegaly with jaundice (5%)—jaundice is due to

haemolysis or due to diffuse liver involvement. ™™ Para-aortic lymph nodes may be enlarged and often palpable as vertically placed mass at or just left of the midline, which does not move with respiration, does not fall forward, nonmobile, resonant, smooth, firm mass often with transmitted pulsation from aorta (secondaries are hard, nodular usually primary from GI, melanoma, testis). Ascites is not a common feature. ™™ Pruritus—25%. It may be the only presenting symptom. It is usually seen in nodular sclerosis type. ™™ Constitutional symptoms like fever, night sweats, weight loss may be present which signifies stage “B”, which has got poor prognosis. Stage “A” is absence of these symptoms which signifies better prognosis.

B xx xx xx

Stage ‘B’ symptoms

Weight loss, more than 10% in 6 months. Fever, (earlier called as Pel Ebstein fever is cyclical high fever, actually is due to brucellosis). Night sweats.

™™ Mediastinal lymph node involvement may cause compres-

sion features like SVC obstruction. Mediastinal lymphoma is the most common mediastinal malignancy which usually occurs in anterior mediastinum. Occasionally presentation may be difficulty in breathing, chest pain, dysphagia and SVC obstruction (Pemberton’s sign may be positive). It may be asymptomatic also. If ratio between maximum transverse diameter of mediastinal mass to maximum transverse intrathoracic diameter (MMR) is more than 0.33 in chest

The difference between ordinary and extraordinary is that little extra.—Zig Ziglar

CHAPTER 1O    General Surgery: Lymphatics

WHO modified REAL classification (Revised European American Lymphoma) of lymphoma: xx B-cell neoplasms –– Precursor B cell neoplasm—ALL, LBL. –– Peripheral B cell neoplasm—it includes all B cell related nonHodgkin’s lymphomas xx T-cell and putative NK cell neoplasms –– Precursor T cell neoplasms—ALL and LBL T cell related –– Peripheral T cell and NK cell neoplasm—it includes all T cell related non-Hodgkin’s lymphomas xx Hodgkin’s lymphoma –– Predominant HL—nodular lymphocyte type –– Classical HL -- Nodular sclerosis -- Lymphocyte rich -- Mixed cellularity -- Lymphocyte depletion xx Precursor lymphoid neoplasms. xx Immunodeficiency-associated lymphoproliferative disorders. Note: ALL is acute lymphoblastic leukaemia. LBL is lymphoblastic lymphoma.

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X-ray or more than 0.35 in CT chest, then it carries worst prognosis. ™™ Occasionally bone like vertebrae may get involved. ™™ Anaemia, pancytopenia, fatigue, bone pain, petechiae with red coloured skin patches, nephrotic syndrome (minimal change disease). ™™ Differential diagnosis: Tuberculosis; NHL; HIV; Chronic

lymphatic leukaemia; Nonspecific lymphadenitis; Sarcoidosis; Secondaries in lymph nodes.

B

Ann-Arbor clinical staging

Stage 1: Confined to one group of lymph node Stage 2: More than one group of lymph nodes on one side of the diaphragm Stage 3: Nodes involved on both sides of the diaphragm Stage 4: Extranodal involvement like liver, bone marrow Suffix ‘S’—Spleen involved Suffix ‘B’—Presence of constitutional symptoms Suffix ‘A’—Absence of constitutional symptoms

A

B

Figs. 1.452A and B: Lymphoma in a boy and in an adolescent with neck nodes. Note the diffuse nature. Nodes are usually India rubber consistency.

Note: In modification, following additions are there: xx Single extralymphoid site is I E xx An extralymphoid site with one or more lymph nodes same side of diaphragm is II E xx An extralymphoid site with lymph nodes on both sides of diaphragm III E. xx An extralymphoid site with spleen and lymph nodes on both sides of diaphragm III SE. xx Spleen with lymph nodes on both sides of diaphragm is III S. N—Nodes  H—Liver  S—Spleen  L—Lung M—Marrow  P—Pleura   O—Bone    D—Skin Stage III (1) is nodes above renal vein level and Stage III (2) is below it. In Cotswolds revision of Ann Arbor classification: Stage 3: Involvement of regions or structures on both sides of the diaphragm. Stage 4: Involvement of extranodal sites beyond E sites. Here E means—involvement of single extranodal site contiguous or proximal to known nodal site. X means: Bulky disease which is defined as >1/3rd widening of mediastinum at T5–T6 or >10 cm maximum dimension of nodal mass.

Fig. 1.453: Hodgkin’s lymphoma in neck region.

A

B

Figs. 1.454A and B: Fungating lymphoma in both axilla of a patient.

Fig. 1.455: Secondaries in neck lymph nodes. It is hard, large probably fixed and nonmobile making it advanced and inoperable.

251

Investigations ™™ Blood: Hb%, ESR, peripheral smear, blood urea, serum creati-

nine. Serum alkaline phosphatase and calcium may elevated. to rule out secondaries or tuberculosis.

™™ Excision biopsy of lymph nodes. Entire lymph node is excised

to retain the architecture of the lymph node. It is important to grade the tumour. It is better to have immunohistochemistry of tumour tissue. As cell mediated immunity is decreased, CD4/ CD 8 ratio will be decreased. CD 15, 30, 45 are very useful. ™™ Chest X-ray—to look for mediastinal lymph nodes, pleural effusion. ™™ Ultrasound abdomen—to look for the involvement of liver, spleen, abdominal lymph nodes. ™™ CT scan of mediastinum/chest, abdomen and pelvis is better, ideal and essential. CT is used ideally to stage the disease.

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. Laparoscopy and biopsy of different abdominal lymph nodes is also a good option. Lower limb lymphangiography to 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. xx 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 I/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. xx

A

B Figs. 1.456A and B: Mediastinal nodes involved in lymphoma as seen in chest X-ray and chest CT scan. ™™ MRI and PET scan are 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 xx xx xx xx xx xx xx

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 Immunosuppression 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

CHAPTER 1O    General Surgery: Lymphatics

™™ FNAC of lymph nodes. Not very sensitive; but initially done

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SRB’s Manual of Surgery

™™ Stage I and II:

Differences between HL and NHL

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

Common Not common

Any group can be involved Not common Common

Treatment for Relapses Mainly radiotherapy Chemotherapy (ABVD regime) Better

Mainly chemotherapy

xx xx xx

Poor

1. 2. 3. 4. 5.

Prognosis

Treatment strategy for HL

Unfavourable signs in early HL

Large mediastinal mass 6. Anaemia 2 mm, poorly differentiated, perineural invasion.

B

Broder’s classification

B Fig. 1.519: Typical epithelial pearls seen in squamous cell carcinoma. ™™ Differential diagnosis: BCC; Melanoma; Keratoacanthoma;

Skin adnexal tumours; Actinic keratosis; Pyogenic granuloma.

™™ Investigations ¾¾ ¾¾ ¾¾

Wedge biopsy from the edge. FNAC from lymph node. CT scan to identify nodal disease; MRI to identify local extension is useful.

xx xx xx xx xx xx

Dry, exophytic, warty growth, HPV association No lymph node spread No blood spread, well differentiated Surgery is the treatment—wide excision No radiotherapy Examples: –– Verrucous carcinoma of genitalia (Buschke-Lowenstein disease) –– Oral florid verrucous carcinoma –– Verrucous carcinoma of foot (plantar aspect)—carcinoma cuniculatum—Ackerman tumour

B xx xx xx xx xx xx xx xx

Fig. 1.520: Squamous cell carcinoma in the foot. It is proliferative cauliflower like lesion. Wedge biopsy has to be done.

Treatment

Prognostic factors in SCC

Tumour size > 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

Marjolin’s ulcer (Rene Marjolin, Paris—1828) ™™ It is well-differentiated squamous cell carcinoma which

™™ Radiotherapy using radiation needles, moulds, etc. is given. ™™ Wide excision, 2 cm clearance followed by skin grafting or



Verrucous carcinoma

flaps. (Presently for tumour less than 2 cm, 4 mm clearance and for tumour more than 2 cm, 1 cm clearance is sufficient). Wide

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.

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I: Well differentiated: 75% or more keratin pearls II: Moderately differentiated: 50–75% keratin pearls III: Poorly differentiated: 25–50% keratin pearls IV: Undifferentiated/anaplastic: 2 cm Near the eye/nose/ear Ill-defined margins

¾¾

B

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.

¾¾

MOHS (Microscopically Oriented Histographic Surgery) (Federic E Mohs, American Surgeon) is useful to get a clearance margin and in conditions like BCC 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 quadrants 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 BCC 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 peplus.

Your mind will give back exactly, what you put into it.

CHAPTER 1R    General Surgery: Skin Tumours

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.

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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). Multiple 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-fluctuant, non-compressible 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.

1. Hairy mole is a mole with a hair growing on its surface.

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. Micro­s co­p ically 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.

Types Fig. 1.529: Junctional naevus.

5. Compound naevus. It is combination of intradermal and junctional naevus. Intradermal part is inactive but junctional part is potentially malignant.

Fig. 1.530: Compound naevus.

Fig. 1.527: Different types of naevi.

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-

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Fig. 1.531: Intradermal 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 spilus: 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. Dysplastic 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).

Treatment Excision. Always should be sent for histopathology.

MELANOMA ™™ 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. xx xx xx xx xx xx xx xx

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.

DOPA reaction: Tyrosine

DOPA

Melanin

                     Tyrosinase Oxidase

Fig. 1.532: Congenital naevus.

Note:

• 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 / intracranial 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 freckle/ephilis.

™™ 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.

Sites

B xx xx xx

Sites of melanoma

Head and neck—25% Trunk—25% Other sites—14%

Don’t sit back and take what comes; go after what you want.

xx xx

Lower limb—25% Upper limb—11%

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cytes. Halo naevus is often seen along with vitiligo. Similar halo may develop around a malignant melanoma lesion. 9. Intradermal naevus: Cluster of dermal melanocytes is seen without junctional component. Common in face.

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™™ 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), muco­c utaneous junction (anorectal region, genitalia), head and neck (meninges, oropharynx, nasopharynx, paranasal sinuses).

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—RR 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 from mother to foetus Melanoma xx Lymphosarcoma

B xx

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 III: 1.51 mm to 4 mm IV: More than 4 mm

Relation of tumour thickness to nodal spread—based on AJCC classification Lesion Tumour thickness Nodal spread Thin 1 mm thick lesion)

ABCDE of melanoma (Ugly duckling rule)

Asymmetry Border irregularity – coast of Marine sign Colour variation Diameter > 6 mm Evolving (changing progressively)

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.

B

Blood spread in melanoma

Brain—convulsions, localising features and raised intracranial pressure xx Lung—cannon ball secondaries, pleural effusion-haemoptysis, chest pain and cough xx Liver (massive liver), ascites xx Skin—cutaneous nodules often pigmented xx Bones—bone pain, pathological fracture. Paraplegia/neurological deficits in spine metastasis Extensive visceral involvement causes melanuria xx

™™ 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.

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. ™™ Induration 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):

A

B

Figs. 1.543A and B: Primary melanoma with lymph node secondaries in two different individuals. Note the pigmented ulcerated secondaries in one.

B xx xx xx xx

B xx xx xx

A

xx

B

Figs. 1.544A and B: (A) ‘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.

xx

Note:

xx

xx

xx

• Satellite nodule occurs within 2 cm of the primary melanoma whereas in – transit nodules occur in between primary and lymph node secondaries.

xx

• Infiltration into deep fascia by melanoma is rare in initial stages as deep fascia acts as a strong barrier.

xx

• Melanoma can also occur in places other than skin like tongue, mucous membrane or genitalia. xx xx

Melanoma

5% of all skin cancers—incidence 20 times more common in whites than blacks Mucosal 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

Investigations ™™ No incision biopsy. It can cause early blood spread. Only in

Fig. 1.545: Melanoma in tongue.

A

B

Figs. 1.546A and B: Melanoma in vagina—in mucocutaneous junction. On table and excised specimen of melanoma.

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—LDH; Melan – 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.

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Occult melanomas (primary unknown) are common in Anus xx External auditory canal Genitalia xx Adrenal medulla Scalp xx Nail bed Eye

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B

TNM staging (AJCC, 8th edition, 2018)

T (Tumour) T0 - 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; T1b - < 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 N0: No regional nods involved. N1: N1a – Clinically occult One node detected by SLNB: N1b – Clinically detected one node; N1c – 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 M0 – No distant spread M1: M1a – Skin, soft tissue, non regional node spread without (M1a (0)) or with LDH elevation (M1a (1)). M1b – Distant spread to lungs with or without M1a without raise in LDH (M1b (0)) or with raised LDH (M1b (1)). M1c – Distant spread to Non CNS viscera with or without M1a or M1b without raise in LDH (M1c (0)) or with raise in LDH (M1c (1)). M1d – Distant spread to CNS with or without M1a,b,c without raise in LDH (M1c (0)) or with raise in LDH (M1c (1)). Staging Stage 0: Tis, N0, M0. Stage IA: T1a, T1b, N0 M0. Stage IB: T2a, N0 M0. Stage IIA: T2b, T3a, N0, M0. Stage IIB: T3b, T4a, N0, M0. Stage IIC: T4b, N0, M0. Stage IIIA: T1a/b, T2a; N1a or N2a; M0. Stage IIIB: T1a/b, T2a; N1 b/c, N2b; M0. T2b, T3a, N1a, N2b, M0; T0, N1b/c, M0. Stage IIIC: T1a to T3a, N2c or N3a/b/c, M0; T3b/ T4a, any N ≥ N1, M0; T4b, N1a to N2c, M0; T0, N2b, N2c, N3b / N3c, M0. Stage IIID: T4b, N3a/b/c, M0. Stage IV: Any T, Any N, M1.

B xx xx

Tumour markers for melanoma

MELAN-A HMB 45 (Human Melanoma Black 45)

xx xx

S 100 LDH

Treatment Surgery is the main treatment.

For primary: a. Handley’s wide local excision (WLE) is wide excision with clearance of margin as well as depth. Clearance margin used in olden days is 3–5 cm. Tumour thickness in mm

Wide Local Excision (WLE) clearance margin in cm

In situ and 4 mm

2.0 cm

  Present recommendation is—in situ melanoma needs 0.5 cm clearance; melanoma < 1 mm thickness needs 1.0 cm

clearance; 1–2 mm thickness needs 1–2 (1.5) cm clearance; 2 cm/3 cm clearance is sufficient for >2.0 mm thickness. Procedure can be done under regional or local anaesthesia. Evidence says that more than 2 cm clearance will not show any additional advantage in treating primary tumour.   Primary closure or SSG or local flaps are used to cover the defect. b. If primary area is wide and deep, then amputation with one joint above is done. c. In fingers and toes, disarticulation is required. d. Melanoma in anal canal may require abdominoperineal resection. e. Enucleation in case of melanoma in eye. f. Melanoma in pregnancy is treated with termination of pregnancy and specific therapy for melanoma. Pregnancy should be postponed for 2 years.

For lymph node secondaries: 1. In a clinically palpable lymph node, FNAC of lymph node is done. In case of spread, then regional block dissection, i.e. ilioinguinal or axillary or neck is done. Once FNAC shows positive lymph node 5-year survival rate reduces to 50%. 2. In a fixed lymph node, only chemotherapy is the treatment because it is inoperable. 3. Lymphatic mapping and sentinel node biopsy (Dr Donald

293

A

B

Figs. 1.547A and B: Widely excised melanoma specimen from heel. Cut section of widely excised specimen of melanoma from heel shows the depth of the tumour.

A

B

Figs. 1.550A and B: Melanoma secondaries in groin nodes after block dissection. Black lesions are observed in cut section.

B

Figs. 1.548A and B: Wide excision and skin grafting done for melanoma; (B) Disarticulation of lateral two toes for interdigital melanoma.

Figs. 1.551: Secondaries in inguinal lymph nodes from primary melanoma sole-on table finding. Pigmented nodes are observed.

Fig. 1.549: Wide excision of melanoma from the heel. Note the clearance margin and depth of dissection.

Morton, 1970): Radioactive colloid is injected around primary site and lymphoscintigraphy is done using hand held gamma camera to visualise the mi rometastasis in the nodal field. If there are micrometastasis, then regional block dissection (therapeutic LND) is done.   SLN (sentinel lymph node) can be identified in 95% or more of groin and axillary nodes and in 85% cases of head and neck melanomas. Often both blue dye and technetium sulfur colloid is used together to identify the SLN. SLNB is useful for melanoma with thickness more than 1 mm depth. Less than 1 mm thickness is considered as low-risk for metastases; between 1-4 mm thickness is considered as intermediate-risk for metastases mainly of nodal spread; more than 4 mm thickness will be considered as high-risk for both nodal as well as blood spread. SLNB is the investigation of choice for staging in intermediate thickness melanoma.

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A

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B

Technique of SLNB

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 a direct 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, immunohistochemistry—S-100 and HMB-45. Interval lymph nodes like popliteal or epitrochlear when involved should be cleared surgically.

B

Sentinel node biopsy

a. Carcinoma breast c. Carcinoma penis

b.  Melanoma

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. ¾¾ 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/l perfusion solution in leg (13 mg/l 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. ¾¾ 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/l, actinomycin D 75 µg/l in 400 ml saline (10 mg and 100 µg/l 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 GIT to 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. ¾¾ Chemotherapy and immunotherapy is the main treatment. ¾¾ Isolated lung or liver metastasis can be resected. ¾¾ 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.

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Immunotherapy/biological therapy ™™ 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 combination of CVD with interferon α and interleukin 2. ™™ Interferon α is a cytokine 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. ™™ Imiquimod is applied over the tumour as cream. Talimogene laherparepvec (T-VEC), BCG, IL-2, IF alpha 2b, BCG are used as liquid injected into the tumour. ™™ GM2 ganglioside based vaccine (stimulates production of IgM

antibodies), Melacine (contains melanoma cell lysates) and cancerVax are three vaccines under trial at present.

™™ Ipilimumab 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 I131 or P32 with ultrafluid lipiodol along with lymphangiography.

Targeted therapy ™™ Signal transduction inhibitor therapy using—(1) BRAF

inhibitors—Vemurafenib, dabrafenib; (2) MEK inhibitors— Trametinib 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:

Fig. 1.552: Melanoma involving face extensively with destruction. Note the maggots over the surface of tumour. Melanoma is most aggressive cutaneous malignancy.

Prognosis for Melanoma ™™ 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.

Prognostic factors—overall poor

Staging as prognostic factor

• 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 • Mitotic activity

Stage I: >90% prognosis Stage II: 70% Stage III: 35% Stage IV: 5 cm should be biopsied in suspicious of sarcoma

™™ Commonest soft tissue sarcoma is liposarcoma / malignant

fibrous histiocytoma (MFH – 25%) overall; in the extremities both MFH and liposarcoma; in the retroperitoneum it is liposarcoma. ™™ Commonest visceral (GIT) sarcoma is leiomyosarcoma. ™™ In genitourinary system leiomyosarcoma is commonest in adults and rhabdomyosarcoma in paediatric age group; in uterus leiomyosarcoma; in myocardium angiosarcoma; in hand and foot synovial sarcoma; in skin Kaposi’s sarcoma; in head and neck region angiosarcoma.

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Incidence of STS

B xx

Fig. 1.554: Osteosarcoma of the upper end of humerus in an adolescent boy.

xx xx xx xx xx xx xx xx

Aetiology

Genetic –– von Recklinghausen disease –– Gardner’s syndrome –– Tuberous sclerosis –– Basal cell naevus syndrome –– Li-Fraumeni syndrome Chemicals—PVC, tetrachlorodibenzodioxin, arsenic Viral—HIV in Kaposi’s sarcoma, cytomegalovirus Ionising radiation—malignant fibrous histiocytoma (p53) Lymphangiosarcoma in post-mastectomy lymphoedema— Stewart—Treves syndrome. Osteogenic sarcoma in Paget’s disease of bone/exposure to radium Retinoblastoma associated sarcoma Gorlin’s syndrome Thorotrast, vinyl chloride, arsenic, pesticides

Clinical Features of Soft Tissue Sarcoma ™™ Painless swelling of short duration with progressive increase

Fig. 1.555: Osteosarcoma upper tibia. Note the size of the tumour and ulceration. It is very vascular.

A

in size—soft tissue mass. Thirty percent of patients may present as pain during first evaluation. ™™ Compression of adjacent structures ™™ Smooth, firm/hard, warm and vascular ™™ Features of secondaries in lung—cough, haemoptysis and chest pain. Lung is the commonest site of secondary. ™™ Secondaries in liver as a principal site especially in visceral STS. ™™ There are no reliable findings to distinguish benign from malignant swellings. ™™ One has to maintain a high index of suspicion in any soft tissue mass deep-to-deep fascia, any soft tissue mass >5 cm, any new enlarging or symptomatic soft tissue mass.

B Figs. 1.556A and B: X-ray pictures of osteosarcoma in tibia and femur.

Note:

• Many liposarcomas arise at sites devoid of adipose tissue. Most rhabdomyosarcomas arise in locations that lack voluntary muscle. • 40% of soft tissue sarcomas are more than 10 cm in size at the time of presentation; 30% are 5-10 cm and 30% are less than 5 cm.

A

B

Figs. 1.557A and B: Soft tissue sarcoma upper chest wall. Note the vascularity and extent.

All things are difficult before they are easy.

CHAPTER 1S    General Surgery: Sarcomas

Fig. 1.553: Soft tissue sarcoma foot. Note the vascularity.

In adults ™™ MFH and liposarcoma—35–45%. ™™ Rhabdomyosarcoma—10%. ™™ Leiomyosarcoma—9–15%. ™™ Synovial sarcoma—7%. ™™ Malignant peripheral nerve sheath tumour (MPNST)—6%. ™™ Fibrosarcoma—5%. In children ™™ Rhabdomyosarcoma, neuroblastoma are common tumours.

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Investigations

SRB’s Manual of Surgery

B xx xx xx xx

Preoperative evaluation of STS is done:

To obtain a tissue diagnosis To determine the “exact extent” of the tumour To evaluate metastatic disease Other investigations

Tissue diagnosis ™™ Incision biopsy is the most reliable method of diagnosis. It

provides adequate tissue sample.

B xx xx

xx xx xx

xx xx xx xx xx xx xx

xx xx

Incision biopsy for soft tissue sarcoma

It is the ideal tool to conclude sarcoma histologically Incision should be placed in such a way that it can be included in wide tumor excision at a later period—as biopsy track is always contaminated One should achieve absolute/adequate haemostasis to avoid haematomas as tumours are vascular Proper site of incision biopsy should be decided One should use shortest possible route to tumour while taking incision biopsy; should not violate more than one compartment; should avoid neurovascular bundle. (Injury to vessels and nerves should be avoided) Smallest longitudinal incision is used to provide adequate specimen (Incision should be longitudinal in limbs) Transverse incision is contraindicated in the limbs except over the flexures Minimal tissue disturbance and avoiding raising of flaps are crucial (Flaps should not be undermined) It is better to use cold knife One should avoid crushing/distorting the specimen Frozen section/Imprint specimen can be used to avoid sampling error. Drains are not used routinely in incision biopsy. If used exit near/close to the wound and not away/distant from the incision biopsy wound. Excise the biopsy tract and drain site enbloc during the definitive procedure. Immunohistochemistry and cytogenetics are possible

Fig. 1.558: Incision for biopsy in STS should be longitudinal so that it can be included in future surgical wide excision. Never place transverse incision for biopsy.

™™ Trucut biopsy/core needle biopsy is an acceptable first

diagnostic step as it is technically easier, not costly, with fewer complications. But it is not useful in visceral STS. Again if it is inadequate one should not be hesitant to go for incision biopsy at the earliest. It is done using 14 gauge needle; often US/CT guide is used. ™™ Excision biopsy is done only if the tumor size is 100 mmHg, and central venous pressure >5 cm/H2O); an acceptable PaO2 and haemoglobin level

• Enough liver to donate

• No severe abdominal trauma, systemic infection, or cancer

• Enough residual liver

• Normal kidneys—diuresis greater than 50 ml/h and normal creatinine;and finally, a dopamine requirement less than 10 μg/kg/min

• Suitable anatomy of liver

• Simple technically

• Technically complex procedure

• Recipient complication is low (of bile duct)

• Recipient complication is higher

• Graft quality is average

• Graft quality is good

• Donor’s complication—nil

• Donor’s complication—25% morbidity; 0.4% mortality

Donor Operation

Graft should be:

It is by a midline incision from suprasternal notch to pubic symphysis. Initially dissection is done with a beating heart; but later with a cold preservation into aorta and portal vein and local ice application. UW solution extends cold ischaemic time for 24 hours but usually within 10 hours transplantation is done. IVC segment is removed with hepatic veins. Portal vein is transected. Celiac artery with branches along with hepatic artery is dissected and transected at celiac artery origin. Bile duct is transected. Note:

• 40% of the estimated standard liver volume. • 0.8 to 1% of the body weight of the recipient is necessary for the recipient recovery.

• Remnant liver—>30% of the original liver volume with complete venous drainage is safe for donor survival.

Recipient operation Bowel preparation, prophylactic antibiotics, administrative immunosuppressive agents are needed before surgery. Bilateral subcostal incision is used with midline upward extension. Falciform ligament, left lateral ligaments, hepatogastric and hepatoduodenal ligaments are divided. Right and left branches of hepatic arteries are ligated. CBD and cystic ducts are divided with ligatures. Portal vein is dissected. Infra and suprahepatic vena cava are exposed. Portal vein and suprahepatic vena cava are clamped. Venovenous bypass between portal and femoral veins and internal jugular vein is created with a flow of blood through it more than 2.5 ml/minute. Warming circuits and ultrafiltration is needed. Suprahepatic vena caval cuff is created at the opening of the right, middle and left hepatic veins. Donor IVC is sutured to recipient IVC first above and then below as end-to-end. Recipient IVC can be preserved entirely and donor IVC is sutured to recipient IVC as end-to-side. Preservative solution is passed. Portal vein is sutured. Reperfusion injury can occur at this stage with hyperkalaemia and acidosis. Arterial reconstruction is done between donor celiac artery to recipient gastroduodenal artery. Bile ducts are sutured as endto-end with or without T tube. Often choledochojejunostomy is also done.

Segmental/lobar/split/living donor liver transplantation It is newer beginning but becoming preferred one. It is based on segmental anatomy and liver regeneration capacity. Minimum liver mass required is more than 1% of graft to body weight ratio. Right lobe is better. It is basically used in children; but useful in adults also. MELD score in donor should be less than 20 for living donor transplantation. Regeneration occurs very significantly in recipients in first 2 weeks reaching standard in 1 month. Regeneration is slow in living donor liver in 1 year. Graft to standard liver ratio should be more than 40%. Function of liver in living donor should be optimum. Proper imaging of the donor for entire anatomy including vasculature is important. Individual branches of portal vein, hepatic artery and bile duct are dissected and isolated. Liver is carefully separated from IVC; small branches of hepatic vein are ligated. Main hepatic vein is isolated; liver parenchyma is dissected with finger/Kelly fracture technique. After completion of parenchymal division, vessels are transected; dissected liver is perfused with cold preservative solution. Diseased liver of the recipient is removed completely. Graft is anastomosed in usual manner hepatic vein, portal vein, hepatic artery, bile duct. Living donor transplantation has got higher chances of morbidity, complications compared to cadaveric transplantation.

Postoperative management ™™ Antirejection drugs to be given. ™™ Liver shows low immunogenicity and high regeneration

capacity causing long-term outcome. Initially cyclosporine or tacrolimus with MMF or azathioprine with prednisolone is given. ICU care is needed.

™™ Electrolyte and fluid management, sepsis management,

prevention of encephalopathy, observation for complications are important.

™™ Bleeding on table and postoperatively can occur especially when

recipient liver is cirrhotic. FFP, platelet and blood are needed. Re-exploration is needed if bleeding persists postoperatively. ™™ Hepatic artery thrombosis is the common vascular complication leading into graft failure, CBD necrosis and anastomotic dehiscence. ™™ Immediate graft failure as primary nonfunctional graft can occur (5% of liver grafts). ™™ Bile leak due to CBD ischaemia which can be identified by HIDA scan, ERCP, revision anastomosis is needed. ™™ Infection is common especially enterococci, staphylococci, gram-negative organisms, Candida, Aspergillus, etc. Antibiotic and antifungal therapy is needed. ™™ Acute rejection (30%) of T cell mediated is seen within 10 days commonly but can occur up to 6 months. Liver biopsy confirms the rejection. Higher steroid therapy, polyclonal anti-T cell antibodies are used. ™™ Chronic rejection is seen after 6 months; appears gradually with liver cell dysfunction and hyperbilirubinaemia. Liver biopsy shows very few numbered biliary radicles— vanishing bile duct syndrome. It is due to humoral immunity. It is difficult to control; eventually needs retransplantation. ™™ Recurrence of the earlier disease to the transplanted liver like hepatitis, biliary sclerosis, sclerosing cholangitis. Hepatitis can be controlled by lamivudine (antiviral DNA polymerase for HBV), interferon α, ribavirin for HCV. ™™ Complications of immunosuppressive drugs like hypertension, hyperglycaemia, hyperlipidaemia, osteoporosis, malignancy, infection, bone marrow suppression are often difficult to manage.

Survival ™™ 5-year survival is 70% in adults and 80% in children. ™™ It depends on age, general condition, earlier disease, associ-

ated problems, MELD scoring.

PANCREATIC TRANSPLANTATION ™™ It is used in diabetic patients, taken from cadaver donor to

replace insulin.

™™ It can be combined with kidney transplantation to patients

who have diabetes with end stage kidney disease.

™™ Donor criteria are individuals without pancreatitis. ™™ Entire pancreas with duodenum (Lillehei) or part of the

pancreas (body and tail of the pancreas) can be transplanted. ™™ It is placed in the right iliac fossa with anastomosis done between portal vein and iliac vein, duodenum fixed to bladder. ™™ Graft take up is 60–70%. ™™ Complications: Graft pancreatitis, pancreatic leak, bleeding, urinary infections like cystitis, failure.

Isolated Pancreatic Islet Transplantation Islets of Langerhans are obtained by mechanical disruption of pancreas by injecting collagenase into the pancreatic duct.

Fig. 1.636: Pancreatic transplantation with duodenum and small bowel to achieve adequate exocrine function. Exocrine function is achieved immediately; endocrine (insulin) function is achieved after few days.

™™ The risk of rejection is highest (40%) during the first 3–6

months after transplantation and decreases significantly thereafter.

BONE MARROW TRANSPLANTATION ™™ Indications: Leukaemias; Aplastic anaemias; Immune defi-

ciencies, etc.

Recipients are initially treated with total body irra­dia­tion. As bone marrow is an active immune system, proper tissue typing is essential. Infant bone marrow is better marrow as a donor. Marrow aspirated from donor’s bone is transplanted by intravenous injection to the recipient.

Fig. 1.637: Pancreatic transplantation.

Everytime something good happens to you, make something good happen to someone else.

333

CHAPTER 1V    General Surgery: Transplantation

Complications and problems of liver transplantation

Immunosuppression with cyclosporin-A is always needed. It will take few weeks to show the response. ™™ Problems: Graft rejection; Graft-versus-host disease (GVH) is more dangerous.

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Tissue disrupted is collected and purified by density gradient centrifugation. These islet cells are injected into the liver through portal vein. Islet cell rejection is prevented by covering them with semipermeable membrane which prevents antibodies reaching islet cells but allowing insulin to get secreted. By this method animal pancreatic islet cells transplantation is also under trial.

SMALL BOWEL TRANSPLANTATION ™™ Indication is short bowel syndrome following massive resec-

tion, atresia, necrotising enteritis, Crohn’s disease. ™™ Bowel anastomosis with a stoma (ileostomy) is usual method.

Fig. 1.638: Patient with CRF undergoing haemodialysis using a dialysis unit.

™™ As small bowel is rich in lymphoid tissue, graft versus host

reaction is a major problem. So graft take up is poor. ™™ It is an immunological challenge even though technically

easier.

DIALYSIS ™™ It is technique for the removal of waste product of metabolism,

normalisation of plasma electrolytes and removal of plasma water. ™™ Types: (a) Peritoneal dialysis; (b) Haemodialysis.

a.  Peritoneal Dialysis ™™ Indications

Acute renal failure until renal function recovers. Chronic renal failure until long-term dialysis is instituted. ™™ Contraindications: Abdominal surgery; Hypercatabolic states; Infection; Pre-existing malignancy. ™™ Insertion of catheter: A rigid plastic catheter is inserted through the abdominal wall into the peritoneal cavity using a trocar through a small cut made in the skin under L/A. Catheter is bound to abdomen. Dialysis is done using sterile dialysate solution instilled into and drained out of abdomen. ™™ Complications: Leakage; Pain—short lasting, mild; Peritonitis; Membrane failure; Hernia. ¾¾ ¾¾

Hypotension; Dyspnoea; Disequilibrium syndrome. Bleeding (due to dysfunctional platelets due to uraemia and due to heparin use). ™™ Even though repeated dialysis (in CRF, at least twice a week is needed) helps, patient needs repeated blood transfusions, which will lead into haemochromatosis/iron overload. Patient should be given erythropoietin injection 3,000 units twice weekly to prevent repeated blood transfusions. But it is expensive. Transplantation is the best answer eventually, in all these individuals. ¾¾ ¾¾

CIMINO FISTULA (Cimino-Brescia) It is an arteriovenous fistula created for haemodialysis. Usually at wrist, radial artery is anastomosed to cephalic vein side-to-side and a created good fistula shows continuous thrill and bruit, with increased venous engorgement along with hyperdynamic circulation. At the ankle, often fistula is created between posterior tibial artery and saphenous vein; in the thigh between femoral artery and long saphenous vein. Distal gangrene is not common. ™™ Complications: Infection, bleeding, hyperdynamic circulation.

Continuous ambulatory peritoneal dialysis (CAPD) is the preferred method of dialysis in some centre.

b.  Haemodialysis ™™ Indications

Acute renal failure; Chronic renal failure. ¾¾ Acidosis; Electrolyte disturbance; Intoxication. ¾¾ Uraemia (pericarditis and polyneuropathy due to uraemia are absolute indications). ™™ Haemodialysis requires access to circulation which is achieved by creating a fistula between the radial artery and the cephalic vein at the wrist (Cimino fistula). Here dialysis occurs in a dialysing machine across a semipermeable membrane (usually cellulose membrane). ™™ Complications ¾¾ Access site—arterial and venous stenosis, throm­bosis, infection. ¾¾

A

B

Figs 1.639A and B: Arteriovenous fistula between radial artery and cephalic vein at wrist is done to attain increased venous engorgement and arterialisation so as to achieve rapid blood flow which is necessary for haemodialysis. Patient with chronic renal failure who requires regular haemodialysis needs this.

W. Pain ™™ Obstruction like intestinal obstruction. ™™ Colicky pain like ureteric, biliary, intestinal. ™™ Compression over nerve roots like in inter vertebral disc

C hapter Outline ·· Gate Control Theory

prolapse.

™™ Advanced malignancies cause severe distressing pain, which

Pain is a subjective one and is difficult to assess and quantify. Pain perception varies from person-to-person and from timeto-time. xx xx xx xx xx

It can be physical or mental It can be localised or diffused It can be acute or chronic It can be intermittent or persistent It can be mild or severe

Pain receptors in the skin ↓ Neurotransmitters of pain like substance P or peptides are activated ↓ Sensory nerves ↓ Posterior horn of spinal cord ↓ Spinothalamic tract ↓ Thalamus ↓ Cerebrum ↓ Pain perception

GATE CONTROL THEORY Gate control system is located at the junction of first and second neuron. Large diameter ‘A’ fibre is stimulated by temperature and touch. Fine ‘C’ fibre is stimulated by pain. If ‘A’ fibre once gets stimulated, blocks the gate mechanism, then pain from ‘C’ fibre cannot pass through the gate to reach the brain for perception. Pain modulators like endorphins and opioid peptides in brain and spinal cord inhibit the release of substance ‘P’.

Causes of Pain ™™ Inflammatory causes due to any infection or infestations. ™™ Hypoxia due to poor blood supply like in myocardial infarc-

tion, peripheral vascular disease.

other causes.

Clinical Assessment of Pain ™™ Its severity, nature, cause should be assessed. ™™ Cause should be thoroughly analysed by doing all investiga-

Pain Pathway

™™ Trauma.

requires proper pain control.

™™ Ulcers, perforation, peritonitis, abscess formation are all

tions like haematocrit, sinology, CT scan, culture of the fluid like pus, blood. Pain is a most common symptom which patient complains to a clinician. Latin word ‘poena’ means penalty/punishment. Pain is the one patient feels; tenderness (sign) is one surgeon/ clinician elicits.

Specific points in history in relation to pain to be asked are: ™™ Original site of pain is very important. In acute appendicitis

original site of pain is in umbilicus; but later it is referred to right iliac fossa. Shift of pain towards other site. ™™ Time and mode of onset of pain—it is sudden onset and rapidly progressive in acute appendicitis; it is of insidious onset and of long duration with episodic nature in chronic peptic ulcer. ™™ Type/nature of pain—superficial/deep; dull ache or sharp severe/pricking/bursting/vague aching (continuous mild pain), throbbing, scalding (burning sensation particularly felt during urination in cystitis, pyelonephritis, urethritis), pins and needles pricking sensation in peripheral nerve injury or irritation, shooting pain (seen in intervertebral disc prolapse and sciatica—pain shoots along the course of nerve), stabbing (sudden, severe, sharp, episodic—seen in perforated duodenal ulcer), distension pain (a feeling of restricted or distended like in paralytic ileus or intestinal obstruction), colicky pain is due to muscular contraction in a hollow tube in an attempt to obviate the obstruction by forcing the content out—gripping, episodic pain with vomiting and sweating (seen in intestinal colic, ureteric colic of stone, biliary colic of stone), twisting pain of bowel volvulus/twisted ovarian cyst/torsion testis, constricting pain around the chest by angina, etc. ™™ Severity of the pain: In acute conditions like peritonitis and abscess pain will be severe compared to chronic one.

Success is dependent upon the glands—sweat glands.

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336

™™ Progression of pain: It may be persistent and progressive;

or initially mild gradually increases, later gradually subsides; fluctuation in intensity whether increases and decreases in intensity at regular intervals; quickly reaches maximum and remains like that. ™™ Duration of pain ™™ Periodicity of pain: Pain appears, persists for few weeks and then disappears for few weeks; again reappear. Such periodicity is often observed in chronic peptic ulcer; trigeminal neuralgia. ™™ Precipitating/aggravating factors: Abdominal pain may get worsened by taking food like in gastric ulcer. Pain due to appendicitis, ureteric stone aggravates by change of position, walking, jolting. Pain of urinary bladder stone aggravates in standing position. In reflux oesophagitis pain increases while scooping. Pain in pancreatitis increases while lying down. Pain in intervertebral disc prolapse aggravates by lifting the weight. ™™ Relieving factors of pain: Pain reduce by certain methods and patient uses that method to relieve the pain. Hunger pain of early morning in duodenal ulcer is relieved by taking food. Pain of pancreatitis is relieved in sitting and bending forward. Propped up position relieves pain of reflux oesophagitis. In acute peritonitis, pain reduces temporarily by lying still. ™™ Associated symptoms: Acute pain may be associated with pallor, sweating and vomiting. Migraine pain with vomiting and visual disturbances; intestinal/ureteric colic with sweating, vomiting and cold periphery; acute pyelonephritis and urinary infections with chills/rigors and fever; ureteric colic with haematuria; biliary colic with jaundice and pale stool are other examples of such association. ™™ Time of occurrence of pain is often important in diagnosing the condition. In duodenal ulcer, hunger pain occurring in early morning or later evening is typical. Migraine occurs in early morning; frontal sinusitis induced headache occurs a few hours after getting up. ™™ Pain may move from one place to other: Radiation of pain It is extension of pain from original site to another site with persisting of pain at original site. This radiating pain is of same character of original site. Penetration of duodenal ulcer posteriorly causes pain both in epigastrium and back—is an example. Pain of pancreatitis radiates to back. Referred pain: Pain is not felt at the site of the disease but felt at distant site. Diaphragmatic irritation causes referred pain at the tip of shoulder through same segmental supply of diaphragm (phrenic nerve C4, C5) and shoulder (cutaneous supply C4, C5). Hip joint pathology may cause referred pain in knee joint—through articular branches of femoral, obturator and sciatic nerves. Other examples—referred ear pain from carcinoma tongue through lingual and auriculotemporal nerve; referred pain in the epigastrium from the heart; referred pain in the abdomen from pleura; referred pain over the testis from the ureter. Shifting/migration of pain: Origin of pain is one site; later pain shifts to another site and pain at original site disappears. Pain when begins in viscera, it is felt at the same somatic segmental area in the body; but once parietal

layer is involved by inflammation/pathology pain is felt at the anatomical site. Example is pain of acute appendicitis where original visceral pain is at the umbilicus (T9 and T10 segments supply both umbilicus and appendix) shifts later to right iliac fossa when once the parietal peritoneum of that area is inflamed. Types of pain: ™™ Superficial pain: It is sharp usually localised pain, due to irritation of peripheral nerve endings in superficial tissue by chemical/mechanical/thermal/electrical injury. Segmental pain: It occurs due to irritation of particular nerve trunk/root; located in particular dermatome of the body supplied by the sensory nerve trunk or root. ™™ Deep pain: It is due to irritation of deeper structures like muscles/tendons/bones/joints/viscera. It is vague and diffuse when compared to superficial pain. It is often referred to common segmental areas of representation. Often spasm of skeletal muscle of same spinal cord segment can occur. ™™ Psychogenic pain: It may be functional/emotional/hysterical. ™™ Other pain: like due to thalamic/spinothalamic diseases/ causalgia (intense burning pain along the distribution of the partially. Grading of pain: It is done using pain scale. It is compared to a 10 cm line numbered 0 to 10. This is called as visual analogue scale (VAS). Minimum is 0 means no pain. 10 is the worst excruciating pain. 2 is mild; 4 is discomforting; 6 is distressing; 8 is intense. Reasons to control postoperative pain/acute pain Uncontrolled pain causes tachycardia, hypertension and vasoconstriction Abdominal (upper abdominal mainly) and thoracic wound pain restricts the respiration causing tachypnoea, altered respiration, coughing, chest infection, pneumonia Persisting pain causes restricted movements, deep venous thrombosis and its problems, bed sores Pain delays the recovery and also causes psychological trauma to the patient

B xx xx

xx xx

Management of Pain ™™ Correct the cause like removal of renal stone, cholecystec-

tomy for gallstones.

™™ Analgesics. ™™ Surgical removal of tumour. ™™ Injection of phenol or alcohol. ™™ Electric stimulation, massaging, infrared therapy, wax bath. ™™ Proper physiotherapy. ™™ Hormone therapy. ™™ Injection to ganglion like in trigeminal neuralgia. ™™ Chemotherapy for malignancies. ™™ Radiotherapy. ™™ Sympathectomy for vascular diseases, causalgia. ™™ Cordotomy for severe pain in case of advanced tumours. ™™ Mental relaxation. ™™ Attending pain clinic.

Acute pain

Drugs/opioids/epidural anaesthesia/drug infusion

Chronic pain due to malignancy

First level—simple analgesics (aspirin, paracetamol, NSAIDs, tricyclic antidepressants)

337

Second level—intermediate opioids codeine, tramadol, dextropropoxyphene

Chronic pain in benign disease

Local anaesthetics/steroid injections/nerve stimulation like acupuncture/nerve decompression/ pain due to sympathetic overactivity is corrected by—IV pentolamine (alpha adrenergic block), stellate ganglion block, block using guanethidine, percutaneous chemical lumbar sympathectomy using phenol. Other methods for chronic pain are—paracetamol/NSAIDs/antidepressants/opioids/ carbamazepine/ neuroablative surgeries

™™ Continuous epidural anaesthesia/analgesia using opioids. ™™ Patient controlled analgesia (PCA) is injecting opioids through

epidural route or intravenous route by patient himself after training him. ™™ Intravenous infusions of the analgesia. Pain may be: xx Acute pain. xx Chronic pain due to malignancy. xx Chronic pain due to benign disease.

Drugs for Pain Drugs can be given orally, intramuscularly, few intravenously, intrathecally, per rectally as suppositories, sublingually.

Narcotic Analgesics ™™ Morphine 10–15 mg. Very useful in intractable pain. It can

cause nausea, constipation, and respiratory depression. Its action is neutralised by naloxone. ™™ Pethidine 50–100 mg IM. ™™ Diamorphine 5–10 mg. It is used only in intractable pain. ™™ Pentazocine 30–60 mg. ™™ Dihydrocodeine.

™™ Codeine phosphate is commonly used after intracranial

surgery. It should not be given intravenously as it may precipitate severe hypotension. Codeine also causes constipation. ™™ Bupremorphine.

Non-narcotic Drugs ™™ Aspirin. ™™ Ibuprofen. ™™ Naproxen. ™™ All these NSAIDs cause gastric irritation and may risk the

gastric bleeding. NSAIDs are nonspecific cyclo-oxygenase inhibitors. So gastric protection and platelet function are lost. Rectal diclofenac as suppository is good analgesic. ™™ Paracetamol is effective analgesic and antipyretic agent. It is relatively less common to cause gastric bleeding. Note: Specific COX2 inhibitors are contraindicated in ischaemic heart disease.

Other Drugs ™™ Anticonvulsants. ™™ Antidepressants, anxiolytics. ™™ Carbamazepine for neuralgia. 

We seldom think of what we have, but always of what we lack.

CHAPTER 1W    General Surgery: Pain

Third level—strong opioids like oral morphine, intravenous morphine, subcutaneous diamorphine, epidural diamorphine. Analgesic infusion is also useful. Neurolytics are used whenever there is limited life-expectancy. Other methods are—Subcostal phenol injection for rib secondaries/celiac plexus block using alcohol/intrathecal hyperbaric phenol/percutaneous anterolateral cordotomy/pituitary ablation/hormone ablation/palliative radiotherapy/steroids/flecainide therapy

chapter

2

Faciomaxillary Diseases C hapter Outline

™™ Maxillary tumour extending into the palate.

·· ·· ·· ·· ··

™™ Gummatous perforation in the middle of the palate seen in

Diseases of the Palate Orthopantomogram Cleft Lip and Cleft Palate Maxillofacial Injuries Primary Care (Early Care) in Maxillofacial Injuries ·· Fracture Middle Third Area ·· Zygomatic Complex Fracture ·· Fracture of the Mandible

™™ Squamous cell carcinoma of the palate.

·· Dislocation of the Mandible ·· Jaw Tumours ·· Epulis ·· Ameloblastoma ·· Dentigerous Cyst ·· Dental Cyst ·· Osteomyelitis of Jaw ·· Alveolar Abscess ·· Fibrous Dysplasia of Bone/Jaw ·· Cherubism

congenital syphilis. ™™ Perforation of the palate anywhere in carcinoma palate.

Orthopantomogram (OPG) ™™ It is a plain X-ray of the mandible which shows the entire

mandible in a single plane. It is better than X-ray mandible lateral view as it highlights proper dentition, inner and outer plates of mandible and joints. It is like a rotational tomogram. ™™ Indications ¾¾ Jaw tumours—Adamantinoma, dental cyst, denti­gerous cyst, osteoclastoma. ¾¾ Osteomyelitis of the mandible; Fracture mandible. ¾¾ Carcinoma oral cavity to check infiltration into the mandible.

DISEASES OF THE PALATE

CLEFT LIP AND CLEFT PALATE

™™ Cleft palate. ™™ Torus palatinus—a bony hard swelling in the centre of the

hard palate. ™™ Nasopalatine cysts. ™™ Epstein‘s pearls—at the junction of soft and hard palates, in the midline, in infants due to retained developmental cell rests. ™™ Apical cyst or abscess. ™™ Minor salivary gland tumour—commonest site is palate.

A

Development of Face Face develops from median nasal process, lateral nasal process, maxillary process, mandibular arch, globular arch, olfactory pit and eye. Any change in the development or fusion of these arches leads to formation of different types of cleft lip or cleft palate.

B

Figs. 2.1A and B: Orthopantomogram is being taken and also picture showing OPG X-ray.

B xx xx xx xx

‘L’ for lip, ‘A’ for alveolus, ‘H’ for hard palate, ‘S’ for soft palate Capital ‘LAHS’ for ‘complete type’ Small letters ‘lahs’ for ‘incomplete type’ Asterisks ‘lahs’ for microclefts ‘LAHSHAL’ for bilateral clefts

Incidence Fig. 2.2: OPG showing jaw tumour probably adamantinoma.

™™ In 75% of cases it is unilateral. Commonly occurs on the left

Aetiology ™™ Familial—more common in cleft lip or combined cleft lip and

palate (Risk is 1:25 live births).

™™ Protein and vitamin deficiency. ™™ Rubella infection. ™™ Radiation. ™™ Chromosomal abnormalities. ™™ Maternal epilepsy and drug intake during pregnancy (steroids/

eptoin/diazepam).

Classification

Defect is often associated with other congenital anomalies of cardiac, gastrointestinal, neurological system, Pierre-Robin syndrome (most commonly associated syndrome with features of isolated cleft palate, retrognathia, posteriorly displaced tongue), Klippel-Feil syndrome, Stickler’s syndrome (eye, skeletal, muscular, cleft disorder), Shprintzen’s syndrome (cardiac and cleft disorder), Down’s syndrome, Treacher-Collin’s syndrome, Apert’s syndrome and trisomy.

xx xx xx xx xx xx

side (60%). ™™ In 50% of cases, it is combined cleft lip and palate. Incidence

is 1:600 live births. Common in boys. ™™ In 15–25% of cases, it is cleft lip alone. ™™ In 25–40% of cases it is cleft palate alone. Incidence is 1:1000 live births. More common in girls.

Problems in Cleft Disorders ™™ Difficulty in sucking and swallowing. This is more commonly

I. Cleft lip alone: Unilateral; Bilateral; Median. II. Cleft of primary palate (in front of incisive foramen) only: a. Complete—means absence of pre-maxilla. b. Incomplete—means rudimentary pre-maxilla. i. Unilateral; (ii)  Bilateral; (iii)  Median. III. Cleft of secondary palate (behind the incisive foramen) only: a. Complete—nasal septum and vomer are separated from palatine process. b. Incomplete. c. Submucous. It can be: –– Cleft with soft palate involvement. –– Cleft without soft palate involvement. IV. Cleft of both primary and secondary palates. V. Cleft lip and cleft palate together.

B

™™ Common in Caucasians.

observed in cleft palate than in cleft lip. ™™ Speech is defective especially in cleft palate, mainly to

phonate B, D, K, P, T and G. ™™ Altered dentition or supernumerary teeth. ™™ Recurrent upper respiratory tract infection. ™™ Respiratory obstruction (in Pierre-Robin syndrome) ™™ Chronic otitis media, middle ear problems. ™™ Cosmetic problems; Hypoplasia of the maxilla. ™™ Problems due to other associated disorders.

Fig. 2.3: Central cleft lip (Hare lip, Type I cleft lip—It is rare).

Cleft lip

Central—rare. In upper lip. Between two median nasal processes. (Hare lip) Lateral—maxillary and median nasal process, commonest; can be unilateral or bilateral Incomplete cleft lip does not extend into nose Complete cleft lip extends into nasal floor Simple cleft lip is only cleft in the lip Compound cleft lip is cleft lip with cleft of alveolus

Treatment for Cleft Lip Millard criteria are used to undertake surgery for cleft lip.

B

Millard criteria (Rule of ‘10’)

10 pound in weight; 10 weeks old; 10 g% haemoglobin

Surgery is a science as well as an art.

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xx

LAHS classification of cleft disorders

340

™™ Millard cleft lip repair by rotating the local nasolabial flaps.

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™™ 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-Randall 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.

A

B

Figs. 2.5A and B: (A) Bilateral cleft lip. (B) Cleft lip with cleft lip.

A

B

Figs. 2.6A and B: Cleft lip and cleft palate in an adult.

A

B

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.

B xx xx xx xx xx xx xx xx xx xx xx xx

A

B

Figs. 2.7A and B: Cleft palate only. Lip is normal. Pre-maxilla is not involved.

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 III 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 Continuity of white line should be maintained Vermilion notching should not be there

Fig. 2.8: Steps of cleft lip repair.

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).

A

B

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.

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™™ Incomplete fusion of these three components can cause

Fig. 2.12: Bilateral congenital craniofacial cleft (Courtesy: Dr Sathish Bhat, Plastic surgeon, Mangaluru). ™™ Cleft palate is usually repaired in 12–18 months. Early repair

A

B

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).

A

B

Figs. 2.11A and B: (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.

B

™™ Cosmetic problems can occur.

Treatment for Cleft Palate

B xx xx

xx xx xx

Criteria for surgery

10 kg weight 10 months of age (10–18 months)

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. ™™ Wardill- Kilner push back operation—by raising mucoperiosteum flaps based on greater palatine vessels. ™™ Secondary management: ¾¾ Hearing support is given using hearing aids if defect is present; control of otitis media. ¾¾ Speech problems occur due to velopharyngeal incompetence; articulation problems also can occur—speech therapy is given. It is corrected by pharyngoplasty, veloplasty, speech devices. ¾¾ Dental problems like uneruption, unalignments are common. They should be corrected by proper dentist opinion, and reconstructive surgery. ¾¾ Orthodontic management with alveolar bone graft, maxillary osteotomy—done in 8–11 years of age. ¾¾ Veloplasty, dental implants, rhinoplasty, orthognathic surgeries, etc.

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10 g % haemoglobin

xx

Principles of palatoplasty

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 palate 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

CHAPTER 2    Faciomaxillary Diseases

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.

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Fig. 2.13: V-Y palatoplasty.

MAXILLOFACIAL INJURIES It may be due to road traffic accidents, assaults, bullet injuries or sport injuries.

Classification Fracture in maxillofacial region can be grouped as: ™™ Fracture lower third that comprises mandible.

Fig. 2.15: Posterior gagging of occlusion due to backward displacement of fracture segment in middle third fracture.

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

nose. ™™ Fracture upper third of the face involving part of the orbit, frontal bones.

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.

Maxillofacial fracture also can be grouped as:

Injuries to the Facial Bones

™™ Fracture middle third that comprises maxilla, zygoma and

™™ Fractures of the face which do not involve the dental occlu-

sion—fractures of zygoma and nose. ™™ Fracture which involves the dental occlusion—fracture mandible and maxilla.

Fig. 2.14: Relation between middle third and cranium in 45° plane.

™™ 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. ™™ Infraorbital ecchymosis of the orbit is called Panda sign. ™™ Features ¾¾ Localised swelling due to haematoma. ¾¾ Facial oedema; Bleeding with open wounds. ¾¾ Asymmetry which is clinically confirmed by observing supraorbital ridges, nasal bridge. ¾¾ Localised tenderness; Step deformity; Trismus; Diplopia. ¾¾ Features of associated injuries like intracranial, abdominal or thoracic injuries. ™™ Investigations: X-ray face; CT scan of head and jaw.

B xx xx xx

Suturing of soft tissues Airway maintenance Control of bleeding Pain relief

xx xx

Control of infection Treating the individual fractures

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.

Respiratory Obstruction Causes

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.

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. Haemorrhage may be due to: ™™ Soft tissue bleeding. ™™ Bleeding from inferior alveolar artery, palatine vessels. ™™ Nasal bleeding.

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 facilitating the easy airway and breathing.

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 Lateral • Fractures involving zygomatic bone, arch and maxilla excluding the dentoalveolar component

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 III—high level • Craniofacial disjunction— unilateral or bilateral

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

Features

Le Fort I (Guerin’s fracturelow level) (floating fracture, horizontal fracture of maxilla)

• 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

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General treatment for faciomaxillary injuries

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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

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, zygoma and nasal bones.

Features

Le Fort III (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 mal-alignment with zygomatic arch Guerin sign: Haematoma at greater palatine foramen

A

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.

™™ Oedema face, subconjunctival haemorrhage, ocular ecchy-

mosis. ™™ 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.

B

Fig. 2.17: Fixation of the splint to skull. It is often used with gunning splints to fix it to skull. C

D

Figs. 2.16A to D: Le Fort classification—different types and also dentoalveolar fracture (Refer table for details).

™™ Treatment ¾¾ ¾¾

It should be managed in a center for maxillofacial injuries. Antibiotics.

¾¾ ¾¾ ¾¾

¾¾ ¾¾ ¾¾

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Features ™™ 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. ™™ Infraorbital ecchymosis of the orbit is called as Panda sign. ™™ Investigations ¾¾ 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.

Fig. 2.18: Photograph showing bilateral maxillary fracture.

ZYGOMATIC COMPLEX FRACTURE Classification ™™ Simple fracture which is stable and undisplaced— here frac-

ture 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. Infraorbital 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.

A

B

C

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

CHAPTER 2    Faciomaxillary Diseases

¾¾

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.

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Orbital floor line for fracture. Opacity in the antrum due to blood. ¾¾ CT scan is done to see orbital depression and herniation of orbital fat. ™™ Treatment ¾¾ Every patient with zygoma fracture need not require surgical correction. ¾¾ Need for surgery is decided based on clinical features. ––

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B xx xx xx xx

A

Indications for surgery are:

Infraorbital anaesthesia, trismus Diplopia, enophthalmos Flattening of the cheek Undisplaced fracture with infraorbital anaesthesia

Surgical Approaches 1. C losed reduction of the zygomatic arch through Gillies temporal approach:

B

Figs. 2.21A and B: Disimpaction of maxilla using Rowe’s disimpaction forceps by downward leverage action.

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 ¾¾ When fracture is unstable or ¾¾ Comminuted or ¾¾ 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 ¾¾ Comminuted fracture in orbital floor. ¾¾ Orbital fat herniation. ¾¾ 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.

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CHAPTER 2    Faciomaxillary Diseases

Fig. 2.23: Types of mandibular fractures.

III. 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.

Other Classifications

T

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 stick fracture in children

• Fracture angle of the mandible

Fig. 2.24: Muscle actions in mandible fracture causing different displacements.

• 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

Fig. 2.25: Unreduced and reduced fracture mandible.

Dentoalveolar fracture

Features

Management

• Horizontal fracture below the alveolar margin

• 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 horizontally reduction and placing jaws in central occlusion position • Derangement in occlusion and alignment

• Stabilisation using interdental wires or arch bars

• Gingival laceration • Bleeding

• Liquid diet for 3–4 weeks

• Infection and late osteomyelitis of mandible

Fig. 2.26: Different sites of fracture mandible.

Clinical Features ™™ Pain and tenderness in the lower jaw with bruising over the

surface.

The first two letters of goals are go.....

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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. ¾¾ Open fixation of the fracture segments using silver wires for 4–6 weeks. ¾¾ Fixation by: Interdental 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.

Fig. 2.28: Circummandibular wiring used in gunning splints.

¾¾

™™ 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.

Fig. 2.29: Interdental 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 fracture mandible, it should be dealt with accordingly.

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.

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B xx xx xx xx xx xx

™™ Swelling arising from the dental epithelium and ectomes-

Treacher-collins syndrome

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 tumour 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 (Epulis): Congenital epulis, Fibrous epulis, Pregnancy epulis, Giant cell epulis, Myelomatous epulis, Sarcomatous epulis, Carcinomatous epulis.

enchyme (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 palatinus 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. (B) 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 cemento-ossifying fibroma. (B) Nonneoplastic bone lesions like fibrous dysplasia, cemento-ossifying dysplasia. (C) Cemento-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.

CHAPTER 2    Faciomaxillary Diseases

Fig. 2.31: Compression plating of a mandibular fracture. Note the different methods.

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A

B

C

Figs. 2.33A to C: Lower jaw tumour with images.

A

B

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 tumour. 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.

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/pedunculated. 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

B xx xx xx xx

Fig. 2.36: Large jaw tumour—could be adamantinoma/ osteoclastoma.

Epulis

Congenital Fibrous—commonest Granulomatous Pregnancy

B

351

�  Carcinomatous �  Myelomatous �  Fibrosarcomatous

Fig. 2.37: Orthopantomogram (OPG) X-ray showing unilocular odontogenic tumour in lower jaw.

Odontogenic tumours

Epithelial tumours xx Ameloblastoma xx Calcifying odontogenic tumour xx Odontogenic adenomatoid tumour xx Composite odontoma, which may be either complex or compound. It is odontogenic hamartoma contains all 4 layers, dentin, enamel, cementum and pulp Mesodermal tumours xx Odontogenic fibroma, myxoma xx Cementoma, dentinoma Malignant odontogenic tumours xx Malignant ameloblastoma xx Fibrosarcoma

A

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

B

Figs. 2.38A and B: X-ray (two different X-rays) showing typical honeycomb/multiloculated features of adamantinoma (Courtesy: Dr Veena Jagadish, MDS).

Rudeness is a weak imitation of strength.

CHAPTER 2    Faciomaxillary Diseases

from the gum. Investigations—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 due to inflammatory gingivitis. Usually, it occurs during 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 cell carcinoma of the alveolus and gum presenting as localised, hard, indurated swelling with ulceration. ™™ Fibrosarcomatous Epulis: Fibrosarcoma arising from fibrous tissue of the gum.

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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.

Features ™™ Swelling in the jaw usually in the mandible near the angle

which attains a large size, extending to vertical ramus— Eggshell 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 ¾¾ Osteoclastoma of the mandible: Here inner table is not intact. ¾¾ Dentigerous cyst; Dental abscess. ¾¾ 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: ¾¾ Orthopantomogram (OPG) shows multiloculated lesion— Honeycomb appearance. CT scan of the region in ideal. ¾¾ Biopsy from the swelling. ™™ Treatment: Segmental resection of the mandible. OR Hemimandibulectomy with reconstruction of the mandible.

A

B

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 ¾¾ ¾¾ ¾¾

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. Fig. 2.40: Dental cyst with orthopantomogram x-ray.

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: Painless swelling in the jaw which is smooth and hard. ™™ Differential diagnosis: Adamantinoma; Dental cyst; Osteoclastoma

™™ 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.

B xx xx xx

Curable malignancies

Adamantinoma Basal cell carcinoma Verrucous carcinoma

�  Papillary carcinoma thyroid �  Marjolin‘s ulcer �  Carcinoma colon

T

Differences between dental cyst and denti­gerous cyst Dentigerous cyst

a. Site of occurrence

Erupted tooth under the root

Over the crown of an unerupted tooth

b. Infection

Common

Not common

c. Complication

Osteomyelitis

Adamantinoma

d. Treatment

Excision and extraction of tooth

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.

¾¾ ¾¾ ¾¾

¾¾

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.

Actinomycosis of Jaw (Refer Chapter 1D) Faciocervical is the commonest type; lower jaw is commonly involved; infection begins at carious tooth; indurated gums → nodules → abscess → multiple sinuses → discharging sulphur granules with normal X-ray (Ray fungus). Actinomycosis israelii is the causative agent. It is treated by penicillins.

ALVEOLAR ABSCESS (Dental abscess)

™™ Causes: ¾¾

¾¾

Alveolar abscess leading into osteomyelitis. Recurrent dental infection; Trauma. After dental extraction; surgeries of the jaw. Postradiotherapy osteomyelitis (osteoradionecrosis).

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.

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; common 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 cheek → 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.

Features ™™ 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

Fig. 2.41: Osteomyelitis of the mandible. Discharging sinus is obvious.

spontaneously leading to sinus formation.

Open confession is good for the soul.

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CHAPTER 2    Faciomaxillary Diseases

Dental cyst

™™ Management

354

™™ Oedema, pain and tenderness in the floor of the mouth. ™™ Trismus and dysphagia. Fever and features of toxaemia.

SRB's Manual of Surgery

™™ Tender palpable lymph nodes in the neck. ™™ Investigations: X-ray of the mandible or maxilla; Discharging

pus for culture study; CT mandible.

B xx xx

xx

xx

Complications

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 benign 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.

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 dysplasia (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 femur 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 (Café

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). xx xx xx xx xx xx

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 Monostotic ceases with cessation of growth Surgery should never be done during growing period

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 ¾¾

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.

A

Fibrous dysplasia mandible

B xx

B

xx xx

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 femur is the commonest site.

xx xx xx xx

Pierre-Robin syndrome

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 facts.

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CHAPTER 2    Faciomaxillary Diseases

¾¾

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. ¾¾ Parathormone assay, serum calcium estimation in suspected parathyroid pathology. ™™ Treatment: It should 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. ¾¾

chapter

3 Oral Cavity

C hapter 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.

A

B

Oral cavity includes lips, buccal mucosa, alveolar margins (gingiva), retromolar trigone, hard palate, floor of the mouth, anterior 2/3rd of the tongue (oral/mobile tongue).

RANULA (Rana = Frog, Ranula looks like belly of frog, hence the name— Latin). 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

C Figs. 3.1A to C: Ranula. Note the bluish discolouration, brilliantly transilluminant and the location.

357

Features ™™ Presents as a bluish smooth, soft, fluctuant, brilliantly transil-

luminant swelling in the lateral aspect of the floor of the mouth.

ular 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.

Treatment

A

B

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.

Types

• •

1. Median sublingual dermoid: ¾¾ 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 midline swelling which is smooth, soft, cystic, nontender, nontransilluminant. ¾¾ Treatment is excision through oral approach. ¾¾ Complication is abscess formation. 2. Lateral sublingual dermoid: ¾¾ 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 the mouth. ¾¾ Treatment: Small one is removed per orally. Larger one, through submandibular incision.

•

STOMATITIS

™™ Marsupialisation (unroofing) can be done initially, and

later once the wall of the ranula 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 and swallowing. 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 epithe-

lium 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 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 painful 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 B and C, local applications of anaesthetics (xylocaine)/choline salicylate/benzalkonium chloride.

If you think you are right all the time, you learn nothing from life. —Sir Henry T Buutlin

CHAPTER 3    Oral Cavity

™™ Plunging ranula: Ranula often extends into the submandib-

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358

™™ Candida stomatitis (Monilial thrush) is due to fungal infec-

tion, Candida albicans which is seen in diabetics, individuals on steroid therapy, long-term antibiotics, patients who are bedridden, on prolonged ICU care, in infants, and debilitated patients. Initially multiple red spots which are painful appear in the tongue and buccal area which later turn into curdy white patches. Often it extends into pharynx and oesophagus causing dysphagia. It is treated with antifungal drugs like clotrimazole or fluconazole. Rhagades occur at corners of mouth in congenital syphilis leaving radiating scar and furrow. ™™ Vincent’s ulcerative stomatitis (Vincent’s angina/trench mouth) is due to infection by Gram –ve anaerobic bacteria Borrelia vincentii and Fusiformis. It is common in adolescents and young adults below the age of 35 years. Presents with fever, excessive salivation, red swollen gums with painful ulcers covered with yellow slough (pseudomembrane) which can be removed like membrane—ulcerative gingivitis. From the gums it spreads to cheek, palate, and pharynx. Tongue involvement is uncommon. Tender neck lymph nodes are palpable. Musty foetor oris is typical. Edentulous patients will not develop this infection. Infection in tonsillar crypts is called as Vincent’s angina. It is confirmed by swab culture. It is treated by antibiotics (penicillin group); peeling of membrane, mouth wash, supportive measures, vitamin B and C. ™™ Nutritional stomatitis is due to—(1) vitamin B deficiency like nicotinic acid (pellagra), riboflavin deficiency. It is common in tongue presenting as red area with atrophy of papillae. (2) Vitamin C deficiency is commonly seen as bleeding gums and loosening of teeth. (3) Iron deficiency anaemia causes superficial glossitis mainly in females. ™™ Angular stomatitis is superficial lengthy red brown fissures/ulcers in and around the angle of the mouth with cracks. Candida and streptococci infections are common. It is often called as cheilosis/perleche. It is treated with vitamin B, C, iron and protein supplements with adequate oral hygiene. Perleche is seen in children who suck their finger.

CANCRUM ORIS (NOMA) ™™ ‘Noma’ means—‘to devour’ in Greek—‘eat greedily’ or

‘consume destructively’. ™™ It is a rapidly spreading, progressive, mutilating, gangrenous devastating stomatitis, beginning in the mucous membrane of the corner of the mouth or cheek, progressing rapidly to involve the entire thickness of the lips or cheek or both, with necrosis and sloughing of the entire tissue; usually observed in poorly nourished children and debilitated adults caused by polymicrobial, opportunistic infection caused by components of the normal oral flora that become pathogenic during periods of compromised immune status. It is also common in HIV patients. Noma pudendi involves genitals.

™™ It is an infective gangrene, a severe form of Vincent’s acute

ulcerative gingivitis and stomatitis. ™™ Seen in poorly nourished, ill-child due to Borrelia vincentii

and Fusiformis bacteria. ™™ It starts in lips later extends to gums, spreads into cheek,

bone, soft tissues and skin causing extensive tissue loss with severe toxaemia. ™™ Extensive necrosis of the mucus membrane of the oral cavity with destruction of deeper soft tissue and often bone. ™™ In children it may follow after an attack of measles, gastroenteritis, typhoid, bronchopneumonia. Malnutrition is a predisposing factor. ™™ Excessive salivation, fetid odour with destruction, discharge and toxic features. ™™ Borrelia vincentii can be cultured. ™™ X-ray part shows bone destruction. ™™ Condition has got high mortality. ™™ Secondary infections may also coexist.

Fig. 3.3: Severe infection of lip. Note: Phagedena is a destructive ulceration with gangrene seen in cancrum oris, chancroid. (Phagedena is destruction without proliferation but malignancy is destruction with proliferation).

Treatment ™™ Systemic antibiotics, high dose penicillins, metro­nidazole. ™™ High protein and vitamin rich diet, through naso­gastric tube. ™™ Wound irrigation and liberal excision of the dead tissue. ™™ Blood transfusion, TPN. ™™ Later patient requires flaps to cover the defect.

SYPHILITIC LESIONS OF ORAL CAVITY ™™ Chancre in lip: It is highly contagious primary chancre,

presents as painless macule later forming painful superficial ulcer. Ulcer eventually heals with a scar. It can be on both upper and lower lip—primary syphilis.

™™ Mucous patches which are greyish white contagious patches

LEUKOPLAKIA be characterised clinically or patho­lo­gi­cally to any other disease. It is a premalignant condition. ™™ It is hyperkeratotic, irreversible patch which is most common premalignant lesion of the oral cavity. It is persistent and adherent patch.

Types 1. Homogenous—uniform white patches with less malignant potential; most common type; prevalent in buccal mucosa. 2. Nodular—fine nodules on the surface; more malignant potential. 3. Speckled leukoplakia—white flecks with fine nodules with an atrophic erythematosus base; much higher potentiality for malignancy. But it is less common. ¾¾ Proliferative verrucous leukoplakia is rare but aggressive with 85% malignant potential; is common in elderly women; not associated with tobacco use.

xx xx xx

Smoking Spirit

xx

Sepsis

xx

Superficial glossitis

xx

Syphilis Spices Sharp tooth

xx xx



Susceptibility, vitamin deficiencies chewing using areca, tobacco, slaked lime   C hronic hypertrophic candidiasis (long-standing candida infection)   Human papilloma virus, a possible cause   Pan

™™ Incidence of leukoplakia in those who smoke or chew pan is

™™ It is a white patch in the mucosa of the oral cavity that cannot

B

xx

xx

359

Common causes

20%, whereas incidence in non-smokers is 1%. ™™ Incidence of its turning into malignancy is 4–10%. It increases with age, duration of the pan chewing, smoking. ™™ Buccal mucosa and oral commissures are most common sites. Common in males (3:1). ™™ Leukoplakia of long duration; leukoplakia in elderly; leukoplakia in younger females; leukoplakia in floor of the mouth and tongue; leukoplakia with induration, cracks and fissures are more likely to turn into malignancy. ™™ Biopsy confirms the diagnosis as well as rules out the carcinoma. Tissue study for p53 mutation, inappropriate expression of oncogenes, DNA aneuploidy, reduction in epithelial cadherin adhesive protein (reduction indicates higher malignant potential). ™™ Histology: Parakeratosis with widening of rete pegs.

B xx

xx

Features of higher risk potential in leukoplakia

Speckled type Nodular type Erosions, ulcerations

 

xx

Histological staging

Acanthosis—elongation of rete pegs—smooth, white, dry patch Parakeratosis Widening of rete pegs

xx

xx xx

Dyskeratosis—keratin cell layer formation deep to epidermis Dysplasia Carcinoma in situ

Hard and indurated type Presence of epithelial dysplasia

Features ™™ White or greyish, well-localized patch which is adherent,

firm or hard or indurated. It is often multifocal, bilateral or occurs in many areas like cheek, tongue, gums. Ulcerations are often seen. ™™ It is usually painless and non-tender. ™™ 80% occur after the age of 40 years. ™™ Hairy leukoplakia is dense pigmented friable lesion seen commonly in the tongue; it is common in AIDS patients; there are no hairs (misnomer). Surface looks like hairy due to thickening, corrugation by severe hyperkeratosis and acanthosis. Epstein Barr Virus (EBV) is the comon cause.

Fig. 3.4: Leukoplakia cheek. Note: Leukoplakia tongue looks as though it has been covered with white paint that had hardened, dried and cracked—Sir Henry T Buutlin.

Treatment ™™ Pan chewing and smoking has to be stopped. ™™ Excision, if required skin grafting has to be done.

Do not try hard to learn all things in life; life itself teaches you everythings at right time.

CHAPTER 3    Oral Cavity

seen on lip, cheek and fauces. Mucous patches fuse together to form linear snail track ulcers in fauces, pillars—secondary syphilis. Hutchinson’s contagious condyloma in midline tongue can occur. ™™ Gummatous painless ulcer is seen in anterior 2/3rd of tongue, palate and nasal septum (causes perforation and collapse of nasal bridge). ™™ Syphilitic chronic glossitis is seen in tertiary syphilis which is a precancerous condition.

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™™ Regular follow-up is necessary. ™™ Isotretinoin is helpful. Beta-carotene, tocoferol are also used. ™™ CO2 laser excision.

ERYTHROPLAKIA ™™ It is red velvety appearance of the mucosa which cannot

characterise any recognised condition.

™™ It is 17–20 times more potentially malignant than leukoplakia. ™™ Malignant transformation occurs in more than 50% of cases. ™™ Histologically parakeratosis with severe epithelial dysplasia

is the typical feature. ™™ Red colour is due to decreased keratin causing shining and prominence of submucosal red vascularised connective tissue. ™™ It is equal in both sexes. ™™ It is common in lower alveolar mucosa, gingivobuccal sulcus, floor of the mouth, tongue, vestibule and soft palate. ™™ It can be homogenous/speckled/granular or erythroplakia interspersed with leukoplakia. ™™ Diagnosis is done by biopsy. ™™ Treatment: Biopsy and surgical excision.

cosa epithelial atrophy and dysplasia. Arecoline stimulates collagen synthesis; tannin stabilizes the collagen fibrils and makes them resistant for collagen degradation by collagenase. ™™ 4.5–7.6% of oral submucosal fibrosis turns into malignancy.

Treatment ™™ Precipitating factors should be avoided—spices, chilli,

alcohol, tobacco. ™™ Maintaining oral hygiene. ™™ Local injection of dexamethasone (4 mg) with hyalase (1500

units) biweekly for 10 weeks; vitamin and iron supplements. ™™ Chewable hydrocortisone tablets. ™™ Submucosal injection of human chorionic gonadotrophin; IFN gamma, colchicine tablets (0.5 mg); lycopene 16 mg a day. ™™ Surgical excision of the lesion with coverage of raw area using skin graft or tongue flap. ™™ Multiple release incisions with ‘Z’ plasties; tongue flap coverage can be done. ™™ Stem cell therapy.

Fig. 3.6: Submucosal fibrosis of right cheek. Note the stiff fibrotic bands and scarring.

B xx

Fig. 3.5: Erythroplakia of cheek.

ORAL SUBMUCOSAL FIBROSIS (In 1952, J Schwartz coined the term atrophica idiopathica mucosa oris to describe an oral fibrosing disease which he discovered in five Indian women from Kenya. S.G. Joshi subsequently coined the termed oral submucous fibrosis in 1953). ™™ It is a progressive fibrosis deep to the mucosa of the oral cavity which causes trismus and ankylo­glossia. ™™ The mucosa of cheek, gingivae, palate and tongue shows a mottled/marbled pallor. ™™ It is common among Asians and Indians. ™™ Aetiology: Hypersensitivity to chilli, betelnut, tobacco and vitamin deficiencies probably alter the collagen metabolism leading to juxtaepithelial progressive inflammatory fibrosis of the submu-

xx xx xx xx xx

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Submucosal fibrosis

Due to: –– Prolonged local irritation by chilies, tobacco (pan/quid), areca due to arecoline –– Dietary causes—deficiencies of vitamin A, B complex (riboflavin) and iron –– Localised collagen disorder Racial: It is common among Indians/Asians and people of Indian origin Prevalence in India is 5 per 1000 Incidence is 4–7% Common in middle age; equal in both sexes Soreness and burning in mouth which is more during meals; vesicular eruptions; trismus; difficulty in protruding the tongue. Initial red area turns into superficial ulcers which later forms stiff, fibrotic bands and scarring Common in soft palate, faucial pillars; buccal mucosa Disease is progressive, even after cessation of causative factor like areca use/smoking It shows epithelial atrophy, hyperplasia, dysplasia and fibrosis

Premalignant Conditions of Oral Cavity

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Premalignant conditions of oral cavity

Leukoplakia Erythroplakia Chronic hyperplastic candidiasis Oral submucosal fibrosis Syphilitic glossitis

xx

Sideropenic dysphagia Oral lichen planus Discoid lupus erythematosus

xx

Dyskeratosis congenita

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ORAL AND UPPER AERODIGESTIVE CANCERS ™™ It is one of the commonest cancers in Asian countries and

India (40%).

™™ All ‘S’ mentioned probably are the causative agents. Smoking,

quid of chewing pan are important causes. Tobacco, betel nut, alcohol, human papilloma virus (present in 80% of oral cancers; present in 40% normal individuals), EB virus, vitamin A deficiency, Plummer-Vinson syndrome, bad dental hygiene, denture irritation—are etiologies. Risk is 8 times in tobacco chewers; 10 times with quid users; 30 times with night quid users.

Fig. 3.7: Extensive leukoplakia on the dorsum of the tongue with carcinoma on the lateral margin. Leukoplakia is high risk premalignant.

Medium risks—premalignant but not associated with higher incidence of carcinoma ™™ Oral submucosal fibrosis. ™™ Syphilitic glossitis. ™™ Sideropenic dysphagia (Sideropenia is iron deficiency without

anaemia); or Plummer-Vinson syndrome. Sideropenia is common in Scandinavian females. It causes atrophy of epithelium and becomes potentially malignant. Proper iron therapy controls the disease and reduces the risk. Equivocal risk lesions ™™ Oral lichen planus. ™™ Dyskeratosis congenital—reticular atrophy, nail dystrophy, leukoplakia in oral cavity. ™™ Discoid lupus erythematosus. Note: Oropharyngeal cancer is one of the most common cancers —40% in Indian subcontinent. In western countries, it accounts for 4% only. Risk factors—tobacco and related products; alcohol; areca nut; human papilloma virus; Epstein Barr virus; PatersonKelly syndrome; nutritional deficiency. Patient may develop a second primary (15%) in the oropharynx in different site at same time or within 6 months of the existing primary (synchronous—4% prevalence; 20% of second primaries) or after 6 months of first primary (metachronous—80% of second primaries). Metachronous second primary is more common than synchronous second primary and it usually occurs in 2 years.

• • •

Known risk factors

Possible risk factors

Tobacco, alcohol, betel quid and areca nut Human papilloma virus Precancerous conditions Previous cancer (oral or other) Family history of SCC Sun exposure Diet low in vegetables and fruits Weakened immunity Graft versus host reaction Lichen planus

Poor oral and dental health Poor fitting dentures Secondary smoking (second hand smoke)

™™ Alcohol increases the solubility of carcinogens and suppresses

the DNA repair.

™™ Incidence of oral cancer in India is 28 per 1, 00,000 popula-

tion. Commonest oral cancer in India is of buccal mucosa (more than 70%). ™™ In oral cavity, in West, it is common in tongue (50%), buccal mucosa (25%), floor (15%), gums and others (10%). ™™ Leukoplakia (commonest), erythroplakia, chronic hyperplastic candidiasis are precancerous lesions; submucosal fibrosis, syphilitic glossitis, sideropenic dysphagia are precancerous conditions. Oral lichen planus, discoid lupus, dyskeratosis congenita are doubtful associated lesions. Precancerous lesion is one where cancer is more likely to occur; precancerous condition is one where there is increased risk of cancer. ™™ Upper aerodigestive cancers include that of oral cavity, larynx and pharynx. Depending on anatomical location they present with different features other than common features—trismus, ear pain, hoarseness of voice, dysphagia, ankyloglossia. ™™ Usually they are locoregional disease with high affinity to involve regional lymph nodes. Distant spread is rare except in nasopharyngeal carcinoma. Tongue has highest incidence of

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High risks—lesions with definite risk of malignant change ™™ Leukoplakia. ™™ Erythroplakia. ™™ Chronic hyperplastic candidiasis: It is common in commissures of the mouth and tongue. Dense plaque of leukoplakia is common with curdy white patches due to Candida albicans infection. It often may not respond to drugs, surgery or laser. Immunodeficiency is often associated with this. It is treated by topical or systemic antifungal drugs/surgical excision or laser therapy.

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B

C

Figs. 3.8A to C: Nasolabial flap–used to cover the defect in the lower alveolus. nodal spread, then floor of the mouth, lower alveolus, cheek, upper alveolus, palate. ™™ Multiple synchronous (at same time, 10%) de novo sites and or metachronous (at different periods, 15%) multiple sites. ™™ Cancers in posterior third of tongue and floor of the mouth is often missed on clinical examination—coffin corner or sump area. ™™ Primary may be very small to be detected clinically in places like fossa of Rosenmuller, pyriform fossa, nasopharynx, posterior third tongue but present clinically as hard lymph node secondaries in neck called as secondaries with unknown primary. Hard secondaries in neck confirmed by FNAC but all investigations including blind biopsies, CT head and neck region and endoscopies could not identify primary lesion creates a situation called as secondaries in neck nodes with an occult primary (30%). ™™ Trismus (pterygoid muscle involvement), ear pain due to auriculotemporal nerve involvement, eye pain in nasopharyngeal carcinoma, dysphagia due to tongue involvement mainly the posterior third, hearing loss due to spread to Eustachian tube can occur. ™™ Bronchopneumonia, aspiration are common problems. ™™ Biopsy, endoscopy, CT neck, MRI, chest X-ray are different investigations needed depending on anatomical location of lesion. ™™ Staging will help to plan the treatment and predict prognosis. ™™ Surgical wide excision and radiotherapy are main modalities of treatment. Chemotherapy is used as an adjuvant. Curative treatment in early growth with preservation of functions like swallowing, speech, cosmesis; but with adequate oncological clearance is the principle of surgical approach. Radiotherapy is also used as curative therapy. ™™ Palliative chemotherapy, radiotherapy and surgery can be done depending on location of the lesion. ™™ Involvement of mandible, neck nodes—number, size and fixity alters the prognosis and treatment schedule. ™™ Outcome also depends on the anatomical location of the malignancy. Lip carries better prognosis; tongue has poor prognosis.

™™ General principles used in approaching oral cancers are as

follows (however it depends on grading and staging of the tumour): ¾¾ If only primary is present which is mucosal with size less than 2 cm without nodal spread, then wide local excision with supraomohyoid block dissection of same side is done (N0); primary may also be treated with curative brachytherapy or external beam teletherapy. If nodes are histologically positive then radical neck dissection is done. ¾¾ Larger mucosal primary with similar features are also treated similarly; but post-operative RT or/and chemotherapy is added depending on grading of the tumour. ¾¾ In all these types of lesions, if there are positive mobile neck nodes which is confirmed by FNAC, then radical neck dissection should be done. ¾¾ If primary lesion extends into adjacent soft tissue with mandibular involvement then mandibular resection (marginal mandibular/segmental/partial/hemimandibulectomy) is needed. Part is reconstructed using plates or bone graft taken from iliac crest or opposite 11th rib. 2.4 mm reconstruction plate with PMMF or non-vascularised bone graft (iliac crest cancellous chips) or vascularised bone graft from fibula/iliac crest/scapula are the present recommendations. Skin covering is done by split skin graft inside to mucosa or by appropriate flaps depending on the need and feasibility of donor area (PMMF/DP flap/forehead flap). Neck is addressed similarly. Post-operative EBRT and chemotherapy is needed either concurrent or sequential. ¾¾ If primary is advanced then chemotherapy with EBRT is used. If lesion reduces in size and becomes operable it is then operated accordingly. ¾¾ In fixed primary or secondary, RT with chemotherapy is used for palliation to relieve pain, fungation, sepsis. ™™ In advanced stage terminal events may be severe malnutrition, bleeding, sepsis, and bronchopneumonia. ™™ Posterior lesions has got poor prognosis than anterior lesions. Lip carries best. Prognosis depends on anatomical

location, grading, lymph node status, soft tissue involvement and response of therapy.

™™ It is cancer (SCC) involving buccal mucosa and gingiva etiology

of which is keeping tobacco quid in gingivobuccal sulcus.

™™ It is often called as Indian oral cancer as it is most com­­monly

seen in India.

™™ Buccal mucosa extends from upper to lower alveolus; from

commissure in front to retromolar region behind. ™™ Features, management are same. Marginal mandibulectomy/ segmental resection is commonly needed. ™™ Adjuvant RT and chemotherapy is useful.

™™ Retromolar trigone (coffin corner) is the attached mucosa on

the anterior surface of the ascending ramus of the mandible posterior to the last lower molar tooth extending superiorly to the maxillary tuberosity. It is triangular in shape with base is superior behind the 3rd upper molar tooth and apex inferiorly behind the lower 3rd molar tooth. ™™ Carcinoma in this area commonly invades the ascending ramus of the mandible and upward spread involves the pterygomandibular space. Tonsillar fossa, soft palate may also get involved. Lymphatics from this area may communicate into pharyngeal lymphatics. ™™ SCC in this area can be treated with high voltage radiotherapy or surgery. Lip split incision is required with mandibulotomy. Marginal mandibulectomy is not possible; mandibulectomy with reconstruction is required.

CARCINOMA CHEEK/Buccal Mucosa Squamous cell carcinoma is the most common type of carcinoma of the cheek.

Fig. 3.9: Carcinoma alveolus and gingiva—wide excision and marginal mandibulectomy is done.

CHEEK Anatomy of Cheek ™™ They are fleshy flaps on either side of the face. The demarca-

tion between the lips and cheek is nasolabial fold.

™™ It is composed of skin, superficial fascia with parotid duct,

buccinator muscle, submucosa with buccal glands and mucous membrane. ™™ Lymphatics: Submandibular and preauricular nodes.

Fig. 3.11: Carcinoma buccal mucosa with extensive necrosis. It is the most common oral cancer in India. ™™ Occasionally it can be adenocarcinoma arising from the

minor salivary glands or mucous glands. Rarely it can also be melanoma.

B xx xx xx xx xx xx

Squamous cell carcinoma—commonest Minor salivary gland tumours Melanomas Adenocarcinomas—rare Sarcomas—rare (Sarcomatoid) Basaloid SCC

B

Fig. 3.10: Haemangioma cheek.

Malignancies of the oral cavity

Sites of carcinoma in oral cavity in order

In India xx Cheek—commonest xx Tongue xx Floor of the mouth xx Palate xx Lips

All surgeries are major; there is no minor surgery.

In Western countries xx Tongue xx Floor of the mouth xx Lip xx Cheek

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Carcinoma of Gingivobuccal Complex

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Retromolar Trigone

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C

Figs. 3.12A to C: Carcinoma cheek in different patients. Note the usage of tongue depressor for proper visualisation of the lesion.

Precipitating Factors ™™ All ‘S’—Smoking, spirit, syphilis, sharp tooth, sepsis, spices. ™™ Incidence of oral cancer is six times more in smokers than

non-smokers.

B xx xx xx

Premalignant lesions and conditions

Leukoplakia Erythroplakia Chronic hyperplastic candidiasis

� � �

Oral submucosal fibrosis Sideropenic dysphagia Syphilitic glossitis

™™ Betel nut chewing (Pan, with pan quid kept in cheek pouch for a

long time) is an important causative factor of carcinoma cheek.

™™ Betel/areca nut, betel leaf, slaked lime, and tobacco (often

with catechu and condiments) wrapped in betel leaf is repeatedly chewed after putting into the mouth; and the quid formed is kept for long duration in gingivolabial sulcus; which is said to be highly carcinogenic. Tobacco is the main carcinogenic component, followed by arecoline (stimulate collagen synthesis and fibroblast proliferation) and tannin (stabilizes collagen) alkaloids of areca.

B Fig. 3.13B

Figs. 3.13A and B: Betels commonly used to prepare quid which is kept in gingivoalveolar groove. It is common cause for carcinoma oral cavity.

A

Fig. 3.14: Verrucous carcinoma of the cheek extending into the lip. Fig. 3.13A

B

Types

Verrucous Carcinoma ™™ It occurs as a superficial proliferative exophytic lesion with

minimal deep invasion, often multiple.

™™ Lesion has white, dry, velvety or warty, keratinised surface.

It is common in females. ™™ It is of low grade, very well-differentiated squa­mous cell carcinoma, which is locally malignant without any lymphatic spread. ™™ It is a curable malignancy. ™™ After biopsy treatment is wide excision. Radiotherapy is not given as it may lead to poorly differentiated carcinoma.

Biological Behaviour of Carcinoma Cheek ™™ Carcinoma is common in posterior half of cheek than anterior. ™™ It spreads into the deeper plane to involve buccinator, ptery-

goids; into the retromolar trigone, base of the skull, pharynx. ™™ It spreads outwards to involve the skin causing fungation, ulceration, orocutaneous fistula formation. ™™ Mandible is commonly involved either by direct extension or through subperiosteal lymphatic plexus which communicates freely with oral lymphatics. ™™ Lymph nodes commonly involved are submental, submandibular, deep cervical and often lateral pharyngeal groups. Nodal spread is seen in 50% of cases. ™™ Infection of the tumour area and soft tissues around is common, causing fever, foul smelling ulcer, halitosis. ™™ Respiratory infection is common in these patients. ™™ Once tumour extends into the retromolar region, soft palate and pharynx, dysphagia will occur. ™™ Lesion will later spread to involve alveolus.

Clinical Features

Fig. 3.15: Advanced carcinoma cheek presenting as swelling outside. There is an ulcer inside over the mucosa.

Fig. 3.16: Trismus in an advanced carcinoma of cheek. ™™ Occasionally it may extend into the upper alveolus and to the

™™ Ulcer (painless to begin with) in the cheek which gradually

increases in size in a patient with history of chewing pan and smoking is the commonest presentation and initially it is painless. ™™ Pain occurs when it involves the skin, bone or if secondarily infected. Referred pain to the ear signifies involvement of lingual nerve. Lingual and auriculotemporal nerves arise from mandibular division of trigeminal nerve. ™™ Halitosis which is bad odour breath is common in many oral cancers. It is due to necrosis of tumour, release of mercaptan, butyric acid and ammonia. ™™ Involvement of retromolar trigone indicates that it is an advanced disease, as the lymphatics here com­municate freely with the pharyngeal lymphatics. ™™ Everted edge, induration are the typical features of the ulcer. ™™ Mandible is examined bidigitally, for thickening, tenderness, irregularity and sites of fracture. Mandible may get

maxilla causing swelling, pain and tenderness. ™™ Once involvement of soft tissue occurs, it may come out through skin as fungating lesion often with orocutaneous fistulas with saliva dribbling through fistula. ™™ Submandibular lymph nodes and upper deep cervical lymph nodes are involved which are hard and nodular; initially mobile and later get fixed to each other and then to deeper structure. ¾¾ Once lymph nodes get fixed it may infiltrate into hypoglossal nerve (tongue will deviate towards the same side), spinal accessory nerve (defective shrugging of shoulder) and cervical sympathetic chain (Horner’s syndrome). ¾¾ Compression over external carotid artery leads to absence of superficial temporal artery pulsation. Eventually it causes fungation and bleeding from major vessels—carotid blow out. Note: Node involvement may be due to infection. So often trial antibiotic is given initially.

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(1) Ulcerative; (2) Proliferative (exophytic)—commonest; (3) Verrucous

involved by direct extension, through mandibular canal, or through periodontal membrane. Loss of central part of mandible due to destruction by tumour will cause pouting of lower lip with drooling of saliva—Andy Gump deformity. Mandibular canal is close to occlusive alveolar surface in elderly and edentulous patients to cause early mandibular spread in carcinoma. ™™ Trismus and dysphagia signify involvement of pterygoids or posterior extension.

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TNM staging for oral cavity cancers (AJCC, 8th edition, 2018)

T – Primary tumour Tx – Primary tumour cannot be assessed. Tis – Carcinoma in situ. T1 – Tumour ≤2 cm with DOI (depth of invasion) ≤5 mm. T2 – Tumour 5 mm OR tumour 2–4 cm in size with DOI ≤10 mm. T3 – Tumour 2-4 cm with SOI >10 mm OT tumour >4 cm with DOI ≤10 mm. T4a – Moderately advanced local disease: Tumour >4 cm with DOI >10 mm OT tumour invading adjacent structures only like cortical bone of mandible or maxilla or involves the maxillary sinus or skin of the face. Note: Superficial erosion of bone/tooth socket by a gingival primary is not sufficient to classify as T4. T4b – Very advanced local disease: Tumour invades masticator space, pterygoid plates or skull base and/or encasing the internal carotid artery. Note: DOI is Depth of Invasion NOT tumour thickness.

A

B

Figs. 3.17A and B: Carcinoma cheek fungating outside extensively. Note the pigmentation in one of the pictures.

Fig. 3.18: Advanced carcinoma cheek with fungation and orocutaneous fistula.

N – Nodal spread Nx – Nodes cannot be assessed. N0 – No regional lymph node spread. N1 – Regional single node 60 m/second ™™ Surgical—if nerve velocity is 50%) can be asymptomatic or symptomatic (15–20%).

Fig. 5.4: Cervical rib types. Note: 7 types of cervical ribs are identified. Type III is the commonest which is a band extending from C7 vertebra to scalene tubercle of 1st rib elevating the neurovascular bundle upwards causing compression.

Pathology Cervical rib narrows the scalene triangle (bounded by scalenus anterior, scalenus medius and first thoracic rib below). Compression of subclavian artery; C8 and T1 nerve roots due to cervical rib. ↓ Angulation of subclavian artery occurs. ↓ Causes constriction of artery at the site where artery crosses the cervical rib ↓ ‘Eddie’s current’ created in the blood flow causes sudden release of pressure distal to the narrowing. ↓ Poststenotic dilatation → Venturi phenomenon (due to vessel wall ischaemia) ↓ Stasis of blood occur ↓ Thrombosis → Embolus ↓ ↓ Features of ischaemia in the hand and forearm. Later digital gangrene occurs. ™™ Neurological (95%)—Compression of C8 and T1 causes

tingling and numbness along its distribution, i.e. in the little finger, medial side of hand and forearm. ™™ Venous—(rare—4%): Paget–Schroetter (‘Effort’ axillary— subclavian vein thrombosis) syndrome is subclavian vein compression by cervical rib in costoclavicular space. It is rare.

™™ Arterial compression is very rare (2%) but when it occurs

A

B

C

Figs. 5.5A to C: Anatomy of cervical rib and its relation to subclavian artery and vein and brachial plexus. Note the Eddie’s current, poststenotic dilatation due to arterial compres­sion by cervical rib. X-ray shows left-sided complete cervical rib.

Clinical Features Majority of patients are asymptomatic—(80%). 1. Neurological features is due to compression of T1 and C8 causing tingling and numbness in the little finger, medial side of hand and forearm. ¾¾ Pain, on the medial side, weakness on the medial side of hand and anaesthesia may be evident. ¾¾ Card test for interossei muscle weakness, Froment’s sign/ test to detect weakness in adductor pollicis are positive. Wasting may be due to neurological cause also.

Fig. 5.6: Adson’s test—after palpating radial pulse, elbow with stretched hand is slightly raised; neck is turned to same side; deep breath is taken; feeble or pulse becoming absent may be obvious in positive Adson's. ¾¾ ¾¾

Modified Adson’s test is same as Adson’s, but neck is turned towards the opposite side. Costoclavicular compression manoeuvre (Falconer test): While palpating the radial pulse of the patient, he is asked to move his shoulder backwards and downwards (exaggerated military position) which may cause absence/ feeble radial pulse and a bruit may be heard while auscultating the supraclavicular region—military attitude test.

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more dangerous. Rarely thrombus may extend proximally into the subclavian artery causing vertebrobasilar insufficiency. ™™ Double crush syndrome – TOS with carpal tunnel syndrome (CTS).

2. Vascular manifestations: ¾¾ Pain is due to ischaemia in the muscle. It is more during work, exercise, and is relieved by rest. ¾¾ Upper limb claudication usually is observed in forearm and arm more obvious after usage of limb. Pain (dull pain) in posterior triangle of neck may be due to presence rib mass. Ischaemic pain in the digits and hand may be present. ¾¾ Vasomotor changes with cyanosis, cold fingers, excessive sweating may be observed. ¾¾ Roos test: The patient raises his arms at 90° of abduction at shoulder with the arms fully externally rotated and the elbows at 90° of flexion. The hands are kept in this position for up to 3 minutes. The test is positive if the patient is unable to hold the arms up for 3 minutes, or if the patient feels pain, heaviness or paraesthesia in the shoulder, arm or hand. ¾¾ Elevated arm stress test (EAST): Both shoulders (arms) are abducted to 90° with arms fully externally rotated and the elbows braced backwards. Patient will open and close (clench and unclench) the hands rapidly for 5 minutes. Normal individual can do this without any discomfort and pain. Patient with thoracic outlet syndrome develops pain, fatigue, paraesthesia of forearm with tingling and numbness of fingers which gradually progresses. Patient will not be able to continue the test for 5 minutes. This test can also differentiate thoracic outlet syndrome from cervical disc prolapse disease. ¾¾ Adson’s test: The hand is raised above after feeling the radial pulse. The patient is asked to take a deep breath and turn the head to the same side. Any change in pulse, i.e. either becoming feeble or absent, is noted.

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¾¾

¾¾

This is due to compression of subclavian artery between clavicle and first rib. Similary Halstead manoeuvre is done by 45° abduction and extension of arm with downward pushing of the shoulder with neck turned opposite side to cause radial pulse feebly palpable. Hyperabduction manoeuvre (Wright’s test): While palpating the radial pulse, arm on the diseased side is passively hyperabducted causing feeble or absence of radial pulse. This is due to compression of artery by pectoralis minor tendon (pectoralis minor syndrome). An axillary bruit may be heard on auscultation. Allen’s test: It is used in hand to find out the patency of radial and ulnar arteries. Both radial and ulnar arteries of the patient is felt and pressed firmly at the wrist. Patient clinches his hand firmly (often repeated clinching) and holds it tightly. After 1 minute clinch is released to open the palm of the hand which looks pale. Pressure on radial artery in the wrist is released to see area of distribution of the radial artery. Normally, it becomes flushed with pink color. If there is block in radial artery, the area will remain white. Test is repeated again. This time pressure on the ulnar artery is released to check the patency of ulnar artery. Area will be pale and blanched after releasing in case of ulnar artery block. Otherwise it becomes pink after release in normal individual.

Fig. 5.8: Upper limb ischaemia showing ischaemic ulcers and gangrene of finger tips. Note: Most common presentation is neurological. Most problematic presentation is vascular which requires surgery.

• •

B xx xx xx xx xx xx

Differential Diagnosis

Cervical spondylosis—to differentiate, X-ray neck—lateral view should be taken Carpal tunnel syndrome Tumours or swellings compressing over the vessel or nerves in the neck Other causes of digital gangrene like atherosclerosis, Raynaud’s syndrome, collagen diseases, diabetes mellitus, and embolism Syringomyelia, motor neuron disease Pancoast tumour.

Investigations ™™ Chest X-ray PA view and lateral view including neck—only

Fig. 5.7: Allen’s test done upper limb ischaemia to look for perfusion of hand. ¾¾ ¾¾ ¾¾

Wasting of thenar, hypothenar and forearm muscles. Often digital gangrene, ischaemic ulcers in digits, oedema of fingers and hand are observed. Limb is colder and paler than the opposite side.

3. Features in the neck: ¾¾ Hard, fixed, bony mass in the supraclavicular region. ¾¾ Palpable thrill above the clavicle in the subcla­vian artery. ¾¾ Bruit on auscultation.

(radio-opaque) bony rib can be identified. ™™ Nerve conduction studies to confirm neurological compression and also to rule out carpal tunnel syndrome or cervical spondylosis. ™™ Arterial Doppler of subclavian artery and of the upper limb. ™™ CT scan neck and thorax and CT angiogram of subclavian artery are ideal investigations. MRI of neck is useful. ™™ Other relevant investigations like blood sugar, lipid profile, cardiac assessment. � MR neurography is newer method used now. � Scalene muscle block by injecting 5 ml of bupivacaine with 1 mL of betamethasone into the scalenus muscle to observe for relief of symptoms.

Treatment Conservative ™™ It is aimed at increasing the space and reducing the compres-

sion. ™™ Proper posturing; correction of wrong posture; muscle strengthening exercises; strengthening the shoulder girdles;

Indications: Failure of conservative treatment beyond 2 months; subclavian arterial compression; progressive neurological deficit; nerve conduction velocity 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.

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. Intrathoracic goitre itself. Neck is normal. Common in men.

Features ™™ Dyspnoea at night during lying down or neck extended. ™™ Cough and stridor (stridor is harsh sound on inspiration). ™™ Dysphagia.

A

B

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.

C

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

Investigations ™™ Radioactive iodine study is diagnostic (I123 or technetium).

SRB's Manual of Surgery

™™ 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.

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

• • • •

B xx xx xx xx

Breathing difficulties in thyroid swelling

Retrosternal goitre—positive Pemberton’s sign Multinodular goitre of long duration—positive Kocher’s test— compressive 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 itself— viz., enlargement of the thyroid gland…. A lady, aged twenty, became affected with some symptoms which were supposed to be hysterical…. It 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)

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.

Types 1. Diffuse toxic goitre—(Graves’ disease, Basedow’s disease. Primary thyrotoxicosis)–60%. 2. Toxic multinodular goitre (Secondary thyrotoxi­c osis) (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 β 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 (TSI)/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 (TSIs) 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.

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T3 Toxicosis xx Here T3 alone is raised; TSH is decreased; T4 is normal. xx Free T3 estimation is important.

Struma Ovarii Ovarian teratoma with thyroid differentiation will secrete T3 and T4 and suppress TSH. xx Function of normal thyroid in neck is suppressed. xx Radioisotope scan shows uptake in pelvis with no or less uptake in neck. xx

A

B

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 stimulating 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 commonly shows higher T3 levels than T4. ™™ TSH receptor or G protein genes show somatic mutation. ™™ US neck, T3, T4, TSH and radioisotope scan (shows hot nodule)—are the relevant investigations. ™™ Treatment: ¾¾ 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. ¾¾ 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.

•

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. xx Mild toxic features develop during initial stage of hyperplasia. xx Already formed thyroid hormones are released by inflamed gland causing toxicity. It eventually leads into euthyroid and later hypothyroidism in Hashimoto’s disease. xx

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. xx It is associated with HLA DR3 and HLA DR5. xx

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.

xx

Trophoblastic Thyrotoxicosis HCG secreted from vesicular mole, choriocarcinoma or metastatic embryonal carcinoma in females, acts like TSAb causing toxicity.

xx

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.

xx

Apathetic Hyperthyroidism It lacks all usual clinical features of toxicity. xx It is commonly observed in old people. xx Thyroid gland is not enlarged. xx

Men take only their needs into consideration, never their abilities.

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CHAPTER 6    Thyroid

Subclinical Hyperthyroidism Subclinical hyperthyroidism is defined as a state of decreased TSH level but not undetectable with T3, T4, free T3 and free T4 are within the normal range without any clinical symptoms. xx Its incidence is 1% of hyperthyroidism. xx It is one of the causes of infertility in females (both subclinical hyper or hypothyroidism can cause infertility). xx It may present as cardiomyopathy or arrhythmias. xx Hormone assay, radioisotope scan and US neck, ECG are used for evaluation. xx

460

Contd...

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xx xx xx

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.

xx

Clinical Features of Thyrotoxicosis ™™ It is eight times more common in females.

Fig. 6.35: Primary thyrotoxicosis. Note the exophthalmos.

™™ 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-

A

B

Figs. 6.36A and B: Note the dilated veins in an enlarged vascular thyroid gland. Auscultation is important to hear a bruit.

Fig. 6.34: Diffuse toxic goitre (primary) involves both lobes with hyperfunctioning acini.

T

ioural 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 thyrotoxicosis

Secondary thyrotoxicosis

1. Symptoms appear first, then thyroid swelling

1. Thyroid swelling appears first

2. Goitre is diffuse, smooth, firm or soft, both lobes are involved with thrill and bruit

2. Swelling is large nodular, obvious

3. Features are much more severe compared to that of secondary toxicosis of short duration

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 hepato­ splenomegaly

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. Internodular 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

T

Wayne’s diagnostic indices (clinical) of thyrotoxicosis Symptoms

Present

1.

Dyspnoea on effort

+1

2.

Palpitation

+2

3.

Tiredness

+2

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

Absent

­–5

Absent

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. Dalrymple’s sign: Upper eyelid retraction, so visibility of upper sclera. 4. Stellwag’s sign: Absence of normal blinking—so staring look. First 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.

–2

Palpable thyroid 19 points— toxic

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.

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CHAPTER 6    Thyroid

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.

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15. Kocher’s sign: When clinician places his hands on patient’s eyes and lifts it higher, patient’s upper lid springs up more quickly than eyebrows.

B xx xx

Order of appearance of signs

Stellwag’s sign—mild; first sign to appear von Graefe’s sign—mild

xx xx

Joffroy’s sign—moderate Moebius sign—severe

EXOPHTHALMOS ™™ It is proptosis of the eye, caused by infiltration of the retrob-

ulbar tissues with fluid and round cells, with lid spasm of upper eyelid (Lid spasm is spasm of levator palpebrae superioris muscle which is partly innervated by sympathetic fibres). ™™ Exophthalmos is visible sclera first below (lower part) the lower edge of the iris and later eventually upper part of sclera will be visible. It is due to pushing of the eyeball forwards due to fat, oedema fluid, cells like macrophages in retrobulbar space ™™ Proptosis can be measured by exophthalmometer. ™™ Exophthalmos is of ten self-limiting, but not always. Sleeping in propped up position and lateral tarsorrhaphy will help to protect the eye. ™™ Exophthalmos even though usually bilateral it can be unilateral or frequently unequal in both eyes.

Treatment of severe exophthalmos—emergency ™™ Steroids intravenously; IV antibiotics; Diuretics ™™ Guanethidine, steroid, antibiotic drops ™™ Lateral tarsorrhaphy; Orbital decompression by surgical removal of lateral wall or roof of orbit ™™ Dark spectacles, protective eye patches ™™ Eyelid surgeries, extraocular muscle surgeries ™™ Local radiation therapy to orbital space ™™ 1% methylcellulose eye drops to prevent corneal ulceration ™™ Sleeping with head end elevation

Causes of exophthalmos ™™ Endocrinal: Thyrotoxicosis—common; Cushing’s syndrome,

acromegaly—rare

™™ Congenital deformities of skull: Craniostenosis, oxycephaly,

hypertelorism ™™ Primary tumours: Periorbital meningioma; Optic nerve glioma; Orbital haemangioma; Lymphoma; Osteoma; Pseudotumour—granuloma ™™ Secondary tumours: Antral carcinoma, neuroblastoma; ™™ Inflammatory: Orbital cellulitis, frontal sinusitis; ™™ Vascular causes: Cavernous sinus thrombosis/A-V fistula; Ophthalmic artery aneurysm ™™ Other eye causes: Severe myopia; Severe glaucoma— buphthalmos Note: Antithyroid drugs may worsen exophthalmos and the patient should be observed once antithyroid drugs are started as steroid supplementation may be required. Ophthalmopathy may worsen by thyroidectomy or radioiodine therapy also. Visible lower sclera—sign of exophthalmos Moebius sign—most important—early sign of ophthalmoplegia Thyroid ophthalmopathy in Graves disease-Werner’s abridged classification of ocular changes with van Dyke’s modification—here 0 –6 grades with specified eye signs are used. Grading of exophthalmos: (1) Mild: Widening of palpebral fissure due to lid retraction; (2) Moderate: Orbital deposition of fat causing bulging with positive Joffroy’s sign; (3) Severe: Congestion with intraorbital oedema, raised intraocular pressure, diplopia and ophthalmoplegia; (4) Progressive: In spite of proper treatment progression of eye signs is seen with chemosis, corneal ulceration and ophthalmoplegia Causes of pulsating exophthalmos: Carotid-cavernous sinus A-V fistula; Cavernous sinus thrombosis; Orbital vascular neoplasm; Orbital haemangioma; Ophthalmic artery aneurysm

• • • • • Fig. 6.37: Diagram showing normal relation of eyelid and pupil in lid retraction and exophthalmos.

Severe exophthalmos ™™ Eyelid oedema, chemosis, conjunctival injection. ™™ Diplopia, ophthalmoplegia (complete weakness of all extraoc-

ular muscles and so no movements possible).

™™ Corneal ulceration. ™™ Papilloedema soon develops. ™™ Finally it may also cause loss of vision.

It is called as malignant exophthalmos (It is misnomer even ­though it is not malignant nor related to any malignancy).

•

•

2. Cardiac manifestations ™™ Tachycardia is common.



As per Crile’s grading; Sleeping pulse rate is usually checked for three consecutive nights and average is taken as the value.

B

Pulse rate (Crile’s grading)

Grade I: < 90/min  Grade II: 90-110/min  Grade III: >110/min ™™ Others: Ectopic; Pulsus paradoxus; Wide pulse pressure;

Fig. 6.38: Malignant exophthalmos.

Multiple extrasystoles; Paroxysmal atrial tachycardia; Paroxysmal atrial fibrillation.; Persistent atrial fibrillation (not responsive to digoxin).

3. Myopathy ™™ Weakness of proximal muscles occurs, i.e. the front thigh

4. Pretibial myxoedema It is a misnomer. Pretibial myxoedema is often a feature of primary thyrotoxicosis: ™™ Is usually bilateral, symmetrical, shiny, red thickened dry skin with coarse hair in the feet and ankles. ™™ In severe cases skin of entire leg below the knee with involvement of foot and ankle can occur. ™™ It is due to deposition of myxomatous tissues (mucin-like deposits) in skin and subcutaneous plane. Glycosaminoglycans (hyaluronic acid) deposition occurs. ™™ It might or might not regress completely after treatment for toxicity. ™™ It is associated with exophthalmos with high levels of thyroid stimulating antibodies. ™™ Skin becomes cyanotic when cold. Skin changes in toxicosis are called as thyroid dermopathy. They include—pretibial myxoedema, pruritus, palmar erythema, hair thinning, Dupuytren’s contracture (fascial).

5. Thyroid Acropachy Thyroid acropachy is clubbing of fingers and toes in primary thyrotoxicosis. Hypertrophic pulmonary osteoarthropathy can develop.

6. Others ™™ Thrill is felt in the upper pole of the thyroid and also bruit

on auscultation. It is because in upper pole, superior thyroid artery enters the gland superficially and so thrill and bruit can easily be felt. In lower pole inferior thyroid artery enters the gland from deeper plane and so thrill cannot be felt. ™™ Hepatosplenomegaly.

Investigations for Thyrotoxicosis ™™ Thyroid function tests

Serum T3 and T4 levels are very high. TSH is very low or undetectable. Sometimes, only T3 level is increased and is called as T3 toxicosis. Here in T3 toxicosis, free T3 estimation is important. Free T3, free T4 estimation is done as total T3 and total T4 levels will vary depending on the amount of thyroid binding globulin (TBG). TBG will be raised in pregnancy, cirrhosis, hyperestrogenism. It decreases in conditions with high androgen level, hypoproteinaemia, acromegaly. Free T3 and free T4 are measured using radioimmunoassay. Normal free T3 is 3.0–9.0 pmol/L; free T4 is 8–26 nmol/L.

T4

T3

TSH

Conventional hyperthyroidism

Increased

Increased

Undetectable

T3 hyperthyroidism

→

Increased

Undetectable

Subclinical hyperthyroidism

→

→

Undetectable

™™ Radioisotope study

Radioisotope study by I131 (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. ¾¾ I131 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 I123 is better as it has got short half-life (13 hours). ¾¾ 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 could 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 agranulocytosis). ™™ Thyroid antibodies estimation—antithyroglobulin antibody, TSH receptor antibody, antithyroid peroxidase (anti-TPO) antibody. ¾¾

Treatment for Thyrotoxicosis

B xx

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.

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CHAPTER 6    Thyroid

muscles, arm muscles. ™™ Weakness is more when muscle contracts isomet­rically either while getting down steps, or lifting a full bucket. ™™ Often when it is severe it resembles myasthenia gravis. Once hyperthyroidism is controlled, recovery occurs.

463

Type of disease

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464

Contd... xx Antithyroid pharmacotherapy. It prevents the: –– Release of hormones: Lugol’s iodine (potassium iodide and iodine) 10 drops—3 times day –– Production of thyroxine : Methimazole: long-acting, 20–40 mg OD; more potent; but not used in pregnancy; Tapazole 5 or 10 mg tablets. Carbimazole 20 mg three times a day up to 120 mg. Propyl thiouracil (PTU) 100 mg three times a day, used in pregnancy; 50 mg tablets –– Action of thyroxine on end organs: Propranolol—40 mg tid up to 120–160 mg; PTU –– Production of antibodies: Steroids, methimazole, carbimazole; Levels of TSH R Ab fall and permanent cure may occur in 50% of patients in Grave’s Radioactive iodine therapy Thyroidectomy

1. ANTITHYROID DRUGS Indications for antithyroid drugs ™™ Toxicity in pregnant women—Propylthiouracil is preferred. ™™ Toxicity in children and young adults. ™™ Before thyroidectomy, to make the patient euthyroid. ™™ Soon after starting radioactive I131 therapy for 6 to 12 weeks (Effects of radiotherapy start only in 6 to 12 weeks). A. Thionamides Thionamides are most commonly used antithyroid drugs. It can be imidazole (carbimazole or methimazole) or thiouracil (propylthiouracil) derivatives. They inhibit iodine organification, iodide oxidation by peroxidase and iodotyrosine coupling. PTU also blocks the type 1 deiodinase and so conversion of T4 to T3 peripherally (liver and other organs). Carbimazole: It is the common drug used. Dose is 5–10 mg, exactly 8th hourly (as T1/2 of carbimazole is 8 hours). Each tablet is 5 mg; usually given for 12–18 months; peak plasma level should be maintained in optimum concentration to have a proper benefit; Often tri-iodothyronine 20 microgram 4 times daily or Thyroxine 0.1 mg daily are given in combination with antithyroid drugs, to prevent iatrogenic thyroid insufficiency or to prevent the increase in size of goitre (Block and Replacement therapy). Carbimazole also suppresses the autoimmune process in thyroid in Grave’s disease. Carbimazole causes fever, rashes, arthralgia, myalgia, neuritis, lymph node enlargement, liver cell dysfunction, psychosis, agranulocytosis. Methimazole: It is more potent and longer-acting than propylthiouracil. It is given as once a day dose; 20–40 mg. It is not used in 1st trimester pregnancy as it may cause cloacal and scalp abnormalities. Propylthiouracil: It acts by blocking thyroid hormone synthesis as well as by blocking peripheral conversion of T4 to T3. It also decreases the thyroid autoantibody levels. It can be given for hyperthyroidism in children and in pregnancy, lactation. Dose is 200 mg 4th to 8th hourly. It is short acting. Propylthiouracil is reserved for use in thyroid storm, first trimester of pregnancy and methimazole allergy or intolerance. PTU causes dose unrelated hepatoxicity; agranulocytosis; antineutrophilic cytoplasmic antibody in 20% of patients after long-term usage.

Note: Dose of antithyroid drug are titrated every 4 weeks until thyroid functions (TSH and FT4) normalize. Toxic multinodular goitre and toxic adenoma will not go into remission. Reduction of thyroid hormones occurs in 2–8 weeks. In Graves, once patient is rendered euthyroid options include use of ATD for 12–18 weeks or definitive treatment with RAI or surgery. After cessation of therapy, close follow up for 3–6 months is required to detect relapse. 40% experience recurrence in 1 year in Grave’s; in such situation RAI or surgery has to be considered. Antithyroid drugs are continued during and after surgery, for 7–10 days. It has to be continued even after starting radioactive iodine therapy for 6 weeks to 12 weeks. Response to treatment and possibility of relapse in primary thyrotoxicosis can be assessed by studying HLA status and TsAb level.

• • • • • • • •

B. Beta adrenergic blocking drugs Propranolol: Dose is 40 mg tid. It reduces the cardiac problems and also blocks the peripheral conversion of T4 to T3, as it is the T3 which is the principle active agent in periphery. Contraindications are bronchial asthma, heart block, cardiac failure. It is very useful in toxicity induced due to thyroiditis which is self limiting. 2 mg IV propranolol is used in thyroid storm. Longacting nadolol 160 mg OD can also be used. C. Lugol’s iodine (5% iodine + 10% potassium iodide) It decreases the vascularity of the gland and makes it more firm and easier to handle during surgery. Dose is 10–30 drops/day (minims) for 10 days prior to surgery. Potassium iodide tablets 60 mg tid also can be given instead of Lugol’s iodine. But its use at present is disqualified. (One minim = one drop. One ml = 16 drops). Lugol’s iodine prevents the release of hormone from the gland–thyroid constipation. After 2 weeks, effect of Lugol’s iodine is lost causing thyroid escape from iodine control. Its action is similar like Wolf Chaikoff effect.

Advantages of Antithyroid Drugs It avoids surgery and its complications and also avoids radioiodine therapy its problems. Clinical improvement occurs in 2 weeks. Biochemical improvement occurs in 6 weeks. Remission is confirmed by TsAb assessment, which will be low. But permanent remission rate is very less in adults and is 20% in children.

Disadvantages of antithyroid drugs ™™ Prolonged course of treatment for 18 months, and in spite

of this, cannot predict the remission or relapse. Relapse rate is 40%. Large gland/severe disease/abnormal TSH receptor antibodies (TSH-RAbs) are likely to lead into high recurrence. ™™ Size of swelling may not regress. ™™ It may lead to agranulocytosis and thrombocytopenia, liver damage, hair loss (except propranolol). Sore throat is the earliest presentation of agranulocytosis. If it is so, drug is stopped, total count is done. If it is less, agranulocytosis is confirmed. High doses of injection benzyl penicillin 10–20 lac, 6th hourly, IV is started to prevent infection. If required, blood transfusion is done. Patient usually recovers by this. To control toxicity, Tab. Propranolol 40 mg tid is started. Rarely, they need bone marrow transplantation.

Complications of antithyroid drugs

Note: They are dose-related in methimazole but not so in PTU. Patients with agranulocytosis usually present with fever and pharyngitis. After the drug is stopped, granulocyte counts usually start to rise within several days but may not normalize for 10–14 days. Granulocyte colony-stimulating factor (G-CSF) appears to accelerate recovery in patients with a bone marrow aspiration showing a granulocyte-to-erythrocyte ratio of 1:2 or greater than 0.5.

• • • •

Other drugs which are effective (but not commonly used) Potassium perchlorate inhibits iodide transport by direct inhibition of the sodium iodine symporter. It can be combined with thionamides. Dose is 500 mg bd. It is used in amiodarone induced type I thyrotoxicosis. Iopanoic acid 1 g/day is used in severe unresponsive cases. It inhibits peripheral conversion of T4 to T3. Lithium carbonate 300 mg 6th hourly. It inhibits the coupling of the iodotyrosines and prevents thyroid hormone release. It is useful in when thionamide is contraindicated. Lithium level should be maintained below 1 mEq/L. Guanethidine 40 mg orally 6th hourly. Reserpine 5 mg IM. Dexamethasone 2 mg orally 6th hourly. It inhibits peripheral conversion of T4 to T3. It is used in thyroid storm. Cholestyramine—decreases reabsorption of thyroid hormones from the enterohepatic circulation; used orally at the rate of 4 g four times daily, in combination with methimazole or PTU. High dose of glucocorticoids: It impairs peripheral conversion of T4 to T3 and also lowers serum TSH level; hence can be used in severe resistant/refractory cases.

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2. SURGERY

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entire lateral lobe with whole of isthmus is removed.

Advantages ™™ Rapid and high cure rate. ™™ problems of radioiodine therapy are avoided. ™™ Surgery provides tissue for biopsy, removes the occult

malignant foci. ™™ Surgery is better option for ophthalmopathy due to thyro-

toxicosis. ™™ It is the option for women planning for child. ™™ Coexisting parathyroid carcinoma can be removed. ™™ For intrathoracic retrosternal toxic thyroid, surgery is the choice. Antithyroid drugs and also radioactive iodine may increase the goitre size. Patient should be made euthyroid before doing sur­gery. (It should be confirmed by repeated estima­tion of serum T3, T4 and TSH levels).

Disadvantages ™™ Recurrent thyrotoxicosis (5%). ™™ Thyroid insufficiency (20–45%). It is revealed in 6 months to

2 years and is confirmed by estimating T3, T4 and TSH levels. Hypothyroidism is better than recurrent thyrotoxicosis. It is treated by tab. L-thyroxine 0.1 mg daily (OD) for life-long— usually given in morning. ™™ Complications of thyroid surgery itself.

3. RADIOIODINE THERAPY

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Indications

Failure of drug treatment in primary thyrotoxicosis in young patients Autonomous toxic nodule; Nodular toxic goitre When malignancy cannot be ruled out Graves disease in children, Graves with nodules Need for antithyroid drugs for more than 2 years Large goitre, substernal/intrathoracic goitre Pressure symptoms, Graves ophthalmopathy Amiodarone-induced thyrotoxicosis.

™™ It is now observed that total thyroidectomy may be a better

option in Graves disease to achieve lowest relapse rate and successful stabilisation of thyroid ophthalmopathy as it clears the antigenic focus in thyroid completely. ™™ Surgery done is subtotal thyroidectomy—Both lobes with isthmus are removed and a tissue equivalent to pulp of finger is retained at lower pole of the gland on both sides (5–8 grams).

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Indications

Primary thyrotoxicosis In recurrent thyrotoxicosis

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In autonomous toxic nodule

™™ Radioiodine destroys the cells and causes the complete abla-

tion of thyroid gland. ™™ Usual dose is 5 to 10 millicurie, or 160 microcurie/gm of thyroid. ™™ Patient is made euthyroid using antithyroid drugs; drug is discontinued for 5 days; I131 300–600 MBq is given orally; antithyroid drugs are started after 7 days and are continued for 8 weeks. In 30% of patients, additional 2 or 3 doses may be required. ™™ It takes 3 months to get full response and so until then, the patient has to take antithyroid drugs. Often additional one or two doses of radioiodine are required to have complete ablation. Eventually they go for hypothyroidism and so require main­tenance dose of L-thyroxine 0.1 mg daily. ™™ To give therapeutic dose, patient should be admitted and isolated for 7 days (Half-life) to prevent irradiation. It is given orally soon after getting from the manufacturer without much delay to have optimal efficacy.

You don’t drown by falling in the water; you drown by staying there. —Edwin Louis Cole

CHAPTER 6    Thyroid

Allergic reactions; agranulocytosis (0.2 to 0.5%); severe hepatitis (0.2%) more often with PTU; polyarthritis; Lupus vasculitis; abnormal taste.

™™ In autonomous nodule, hemithyroidectomy is done. Here

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Radioactive iodine therapy (RAIT)

Preferred therapy for hyperthyroidism as it avoids surgery, prolonged drug therapy with >90% cure rate; but proper follow up is needed and facilities should be available (not much centers are having) Administered orally as a single dose in capsule or liquid form 131I (Sodium iodide-131) Causes fibrosis and destruction of the thyroid over weeks to many months causing hypothyroidism It is effective, safe, and does not require hospitalization Given orally as a single dose in a capsule or liquid form - I131 is given (6 to 8 milliCuries) Very few adverse effects as no other tissue absorbs RAI But the effect is less rapid than drugs or thyroidectomy Goal is to make the patient hypothyroid Not recommended with patients of severe ophthalmopathy Not advisable in chronic smokers Pregnancy, breastfeeding, and recent lactation are contraindications A gland-specific dosage based on the estimated weight of the gland and the 24-hour uptake is used. The dose of I131 administered is 75–200 µCi/g of estimated thyroid tissue divided by the percent of 123I uptake in 24 hours No evidence indicates that radioactive iodine therapy for hyperthyroidism causes the development of thyroid carcinoma or results in increased mortality for any other form of cancer, including leukemia Radioactive iodine should be avoided in children younger than 5 years. In children 5 to 10 years old, 131 I therapy is acceptable if the calculated activity of administered131 I is less than 10 mCi. In children older than 10 years of age, radioactive iodine therapy is acceptable if the activity is greater than 150 µCi/g of thyroid tissue Radioactive iodine should never be administered to pregnant women, because it can cross the placenta and ablate the fetal thyroid, resulting in hypothyroidism Risk of hyperparathyroidism may be there after RAI therapy Similarly, breastfeeding is a contraindication, as the radioisotope is secreted in breast milk. Women will continue to receive increased radiation to the breast from radioactive iodine for few months after ceasing lactation It is standard practice to check for pregnancy before starting radioactive iodine therapy and to recommend the patient not to become pregnant for at least 12 months after the treatment. No excess fetal malformations or increased miscarriage rates have been found in women previously treated with radioactive iodine for hyperthyroidism.

RADIOACTIVE IODINE It is used both as a diagnostic as well as a therapeutic agent. 1. I131—is used for radioactive iodine therapy (β-rays are used). 2. I123—is used for diagnostic studies (γ-rays are used).

Diagnosic uses

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Indications for diagnostic radioactive iodine study

Doubtful toxicity; Autonomous toxic nodule Ectopic thyroid; Retrosternal thyroid After total thyroidectomy, to look for secondaries in follicular carcinoma thyroid

For diagnostic purpose I123 is given orally in empty stomach on previous day (dose—5 micro curie; T1/2 (half-life) of I123 is 13 hours and so it is suitable for diagnostic purpose). Patient should not take L-thyroxine for 6 weeks prior to radioisotope study. ™™ Thyroid treats this I123 similar to inorganic I127. This I123 enters the thyroid from the circulation and gets incorporated into T3, T4 and later released into circulation as protein bound iodide (PBI). Normal value of PBI is 8 mgm%. ™™ Using Gieger Muller’s gamma ray counter, scanning of thyroid gland is done to visualize the gland. ™™ Hot area suggests more uptake, ™™ Warm area suggests normal uptake, ™™ Cold area suggests no uptake. I123 radioisotope can be safely used in children and pregnancy for diagnostic purpose only (5 micro­curie) as the dose is low. Note: If the patient is on T3 (60 µg/day) medication, the drug is stopped 10 days before radioisotope scan. Injection TRH, if given, radioisotope scan can be done in 24 hours. Presently I131 is also becoming popular as a diagnostic tool. For treating malignancy β-rays are more used than γ-rays. Technetium 99 scan is used for diagnostic purpose at present as it is effective and faster (in 20 minutes). IV administration is used. Radioisotope study is done to look for secondaries by doing whole body scanning (total body scinti­graphy).

• • • • •

Therapeutic Uses ™™ In primary thyrotoxicosis: It is the main therapy for Grave’s

disease after the age of 10 years except in pregnancy, lactation. ™™ In autonomous toxic nodule, it is useful as remaining gland still will function adequately after radiotherapy (As during radiotherapy radioisotope will not be taken up by this retained normal gland as it is suppressed in the presence of toxic nodule which will function later adequately). ™™ In follicular carcinoma of thyroid, after total thyroidectomy, if there are secondaries elsewhere in the body, as in bones or lungs, then radioiodine therapy is given. I131 is given as its half-life is 8 days. It is given orally in a dose of 5 millicuries (160 microcurie/g of thyroid). It ablates the residual disease in the thyroid bed; facilitates the identification of the metastases and therapy; makes the thyroglobulin estimation as a marker of recurrence at a later period. Precautions in RAI therapy: Sleep alone for 7 days; avoid hugging, kissing (children) for 7 days; keep away from people for 6 feet for 3 days; plenty of water to drink to flush the isotope in urine and prevent radiation cystitis; laxatives to void constipation to prevent radiation enteritis; one should not share utensils, bed, clothes with others for 3 days; wash clothes and utensils separately; wash hands frequently; avoid public places or transports for 5 days; take low iodine diet for 7 days. Contraindications for RAI Therapy: Pregnancy/females desiring to have pregnancy within 1 year/ lactating mothers and children. Problems in RAI Therapy: Permanent thyroid failure with hypothyroidism; effects will be seen only after 3 months;

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Dose of radioactive iodine

Diagnostic • For thyroid-5-50 microcurie • For whole body iodine scan 5–10 millicurie in 72 hours

Therapeutic • Residual thyroid ablation—50 mCi • Bone secondaries from FCT—200–240 mCi • Lung secondaries from FCT—180 mCi

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Radioactive isotopes used in thyroid

Isotopes

Route of administration

Half-life

Type of rays

I123

γ rays

Oral

13 hr

I

124

PET scan in thyroid cancer

4 days

I

125

Oral

60 days

γ rays

I

131

Oral

8 days

β, γ rays

I

132

Oral

2.3 hr

IV

6 hr

Tc 99 scan*

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remember

High dose of retinoic acid will make I131 to concen­trate in tumour cells (70 mg/daily for 2 weeks) Fertility should be avoided for 1 year after I131 therapy Avoid contrast CT in thyroid diseases as much as possible because I131 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 carefully. 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. Subtotal/total thyroidectomy can be done in second trimester.

•

• • • •

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 and then 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 of proptosis: Early thyroidectomy has to be avoided, 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.

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 cell/oxyphil or oncocytic (has potential for microinvasion); hyalinising trabecular adenoma. Note: All adenomas are invariably follicular. Colloid adenoma is the commonest. The existence of papillary adenoma is doubtful; it is invariably a lowgrade papillary carcinoma.

• • •

B. Malignant (Dunhill classification).

*U  sed 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.

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•

a. Differentiated—80% 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.

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.

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.

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ophthalmopathy and dermopathy will be worsened; it may induce hyperparathyroidism; sialadenitis with salivary enlargement and dry mouth and loss of taste or metallic taste. In men, it decreases the sperm count temporarily for 2 months; sperm banking may be better prior to start of RAI therapy. Acute radiation sickness; radiation thyroiditis; pain, bleeding, swelling at the metastatic site are other problems. ™™ Advantages: It is safe; does not require longer hospitalization (if dose is >30 mCi); administration is oral (solution/capsule [one capsule = 50 mCi]; solution is better to take and easier to adjust the dose). RAI dose: (Thyroid mass [gram] X 80-200 uCi)/% uptake.

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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 NHL/papillary 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, C erb, C fos, Ras are associated thyroid neoplasms.

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Differential diagnosis for carcinoma thyroid

Multinodular goitre Riedel’s thyroiditis

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PAPILLARY CARCINOMA of thyroid (PCT) ™™ It is 70–80% common. Common in females and younger

age group.

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.

Fig. 6.40: Papillary carcinoma of thyroid with nodal infiltration— diagrammatic representation.

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Woolner Classification

Types i. Occult primary (50% of MTC 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) should be done in suspected pheochromocytoma. ™™ Serum, calcium and parathormone (PTH) estimation for hyperparathyroidism. ™™ 111-Indium 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.

results. treated surgically by adrenalectomy first and later only total thyroidectomy is done. ™™ All family members of the patient should be evaluated for 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 II 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; Imatinib mesylate tyrosine kinase inhibitor; Chemotherapy–less success– adriamycin, capacetabine, 5 FU, irinotecan; newer targeted therapies.

Prognosis ™™ Sporadic MCT and MCT with MEN syndrome II are aggres-

sive. ™™ Familial MCT not associated with MEN II syndrome has got

better prognosis. ™™ Presence of nodal disease carries poor prognosis. ™™ Survival is overall good—10 years—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.

Follow-up

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.

™™ 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.

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

CHAPTER 6    Thyroid

Note: Consider MCT in thyroid swelling with significant diarrhoea and hypertension. 50% of MCT spread to neck nodes. 15% have distant spread to liver, lung and bone.

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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 (undetectable). ™™ 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 thyroid-

itis (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 autoimmune thyroiditis—common in women (15

times more common). ™™ There is hyperplasia initially, then fibrosis, even­tually infiltra-

tion 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: ¾¾ ¾¾ ¾¾

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, stridor, with positive Berry’s sign (absence/impalpable carotid pulsation); small goitre; common in males. ™™ Differential diagnosis: Anaplastic carcinoma of thyroid. ™™ Investigations: ¾¾ T3, T4 may be low due to hypothyroidism. ¾¾ Radioisotope scan will not show any uptake. ¾¾ FNAC to rule out carcinoma. ™™ Treatment: ¾¾ Isthmectomy is done to relieve compression on the airway. They require L-thyroxine replacement later, as hypothyroidism is common. ¾¾ High dose of steroid often used. ¾¾ Thyroidectomy is not necessary. Recent rapid increase in thyroid swelling is due to: Previous MNG undergoing malignant transformation Haemorrhage into a nodule Anaplastic carcinoma of thyroid

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Carcinoma thyroid infiltrating recurrent laryngeal nerve/trachea Large, long-standing goitre causing tracheomalacia Retrosternal goitre Congestive cardiac failure in thyrotoxicosis

B xx xx

Scabbard trachea in longstanding MNG Retrosternal goitre

B xx xx xx xx

xx

xx xx xx xx

xx xx

Narrowing of trachea is seen in: xx xx

Carcinoma of thyroid Riedel’s thyroiditis

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 I123 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 Occasions wherein thyroid swelling may not move upwards with deglutition Anaplastic carcinoma thyroid—often Carcinoma thyroid with extensive local infiltration into soft tissues, trachea/larynx and posterior muscles Intrathoracic retrosternal extension with infiltration/impaction Riedel’s thyroiditis with encasement of trachea Massive thyroid wherein upward movement is difficult to observe and appreciate

B xx xx xx xx xx

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 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 thyroidectomy commonly 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 multinodular 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, medullary carcinoma of thyroid. 6. Hartley Dunhill operation is removal of one entire lateral lobe with isthmus and partial/subtotal removal of opposite lateral lobe. It is done in non-toxic multinodular goitre. 4 grams of tissue is left behind only on one side.

B xx xx xx

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

xx xx xx xx xx

Preoperative preparation

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.

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Causes of dyspnoea/stridor in thyroid diseases

480

Procedure ™™ 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: ¾¾ 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: ¾¾ Skin and platysma are incised (Subplatysmal plane)— upper flap raised up to thyroid cartilage, lower flap up to sterno­clavicular joint. Deep fascia is opened vertically in the midline. ¾¾ 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 zero vicryl.

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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.

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.

Fig. 6.54: Hemithyroidectomy: Entire one lateral lobe and entire isthmus are removed retaining entire opposite lateral lobe. It is done in solitary nodule/toxic or nontoxic adenoma in one lobe.

Fig. 6.57: Near total thyroidectomy is done in papillary carcinoma of thyroid. Here most of the gland except small tissue of 1 g on lower aspect of one side usually is retained to safeguard recurrent laryngeal nerve and parathyroid gland.

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Fig. 6.58: Total thyroidectomy is done for follicular carcinoma and medullary carcinoma of thyroid.

Fig. 6.59: Hartley Dunhill procedure: Here one entire lateral lobe, isthmus, and most part of the opposite lateral lobe except small quantity of tissue in the lower pole/tracheo-oesophageal groove—subtotal/ partial/one gram is retained.

Fig. 6.60: Skin flaps raised in thyroidectomy. Thyroid covered with pretracheal fascia is seen.

Fig. 6.61: Total thyroidectomy specimen done for follicular carcinoma of thyroid.

Fig. 6.62: Typical thyroidectomy postoperative scar.

Fig. 6.63: Note the relation of recurrent laryngeal nerve.

Have, goals, they give direction, purpose and meaning to life.

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¾¾

¾¾

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Pretracheal fascia is opened vertically to expose thyroid gland. Short stout middle thyroid vein which enters the IJV is ligated immediately (first vessel to ligate; it is vein without accompanying artery) using vicryl or silk and divided. Avulsion of this vein from its junction to IJV will cause bleeding. Gland is mobilised medially using peanut dissection and bipolar cautery. Superior pedicle is dissected; artery and vein are individually ligated and divided. In olden days mass ligation close to gland at superior pole was the practice. Chances of injuring external laryngeal nerve and AV fistula may happen in mass ligation. It is also always better to identify external laryngeal nerve entering the cricothyroid. Dissection is done in an avascular plane between cricothyroid and gland. Parathyroids both superior and inferior are identified. They are 6 × 4 × 2 mm in size weighing 50 mg with yellowish brown/orange brown colour. Superior (parathyroid IV) and inferior (parathyroid III) glands are identified and dissected. Both glands receive their blood supply from inferior thyroid artery and through an anastomotic branch. Superior parathyroid is above and behind the junction of RLN and inferior thyroid artery; inferior parathyroid is below and in front of this junction. Recurrent laryngeal nerve should be identified with careful dissection through its entire course. Riddle’s triangle is between inferior thyroid artery above, carotid artery laterally trachea medially. From this area nerve runs upwards to enter the larynx at greater cornu of thyroid cartilage. Many branches of nerve and the variations should be remembered while dissecting here. One should not use monopolar cautery here; only bipolar cautery should be used carefully. Nerve usually crosses the inferior thyroid artery from deeper aspect; but variations are common. Posterior extension of lateral thyroid lobes close to Berry’s ligament is called as Zuckerkandl tubercle which is seen in 40% of cases. Nerve runs upwards in a fissure between Zuckerkandl tubercle and trachea or main thyroid gland. Nonrecurrent laryngeal nerve (0.5%) is occasionally seen in right side due to failure of development of 4th aortic arch; it directly arises from vagus enters the larynx

Fig. 6.64: Position of the patient for thyroid surgery. Head end elevated, sand bag under the shoulder.

Fig. 6.65: Patient under anaesthesia with proper position for thyroidectomy surgery.

Fig. 6.66: Position of neck in thyroid surgery.

Fig. 6.67: Kocher’s thyroid incision for thyroidectomy.

Fig. 6.68: Suction drain kept after thyroidectomy.

¾¾

Note: Thyroid steal: In thyrotoxicosis, patient is taken to operation theatre daily for few days before doing surgery, so as to reduce the anxiety of the patient. MIVAI—Minimally Invasive Video-Assisted Thyroidec­tomy is becoming popular for small nodules and gland without thyroiditis. But it is costly. Robotic thyroidectomy; thyroidectomy through axillary incision; thyroidectomy through intraoral approach are all newer approaches for thyroidectomy. Vocal cord monitoring through specialized electrodes placed along the endotracheal tube and also to vagus to assess recurrent laryngeal nerve during surgery is accepted method currently; but facilities are available in most of the centers.

• • •

to laryngeal oedema and so endotracheal intubation is also needed immediately; in difficult situation if it is impossible to intubate, a needle tracheostomy using 12-gauge needle (2.3 mm diameter) is done temporarily. 3. Recurrent laryngeal nerve palsy: It can be transient or permanent. Transient is 3% common. They usually recover in 3 weeks to 3 months. Often they require steroid supplement and speech therapy. Permanent paralysis is rare. It presents with hoarseness of voice, aphonia, aspiration, ineffective cough. Unilateral injury can be well compensated. It is advisable to do routine postoperative ILS on 5th postoperative day. Later ILS is done in 4 weeks and 12 weeks. 4. Hypoparathyroidism: It is rare 0.5% common. Mostly it is temporary due to vascular spasm of para­thyroid glands, occurs in 2nd-5th postoperative day. Present with weakness, +ve Chvostek’s sign, carpopedal spasm, convulsions. Serum calcium estimation is done and then 10 ml of 10% calcium gluconate is given IV 8th hourly. Later supple­mented by oral calcium carbonate 500 mg 8th hourly. After 3–6 weeks, patient is admitted, drug is stopped and serum calcium level is repeated.

•

Complications of Thyroidectomy 1. Haemorrhage: May be due to slipping of ligatures either of superior thyroid artery or other pedicles or small veins. It causes tachycardia, hypotension, breathless­ness and compression over the trachea may cause severe stridor, respiratory obstruction due to tension haematoma under strap muscles. As a first aid, immediate release of sutures including that of deep fascia has to be done and pressure over the trachea is released. Then patient is shifted to operation theatre and under general anaesthesia exploration is done and bleeders are ligated. Blood transfusion may be required. 2. Respiratory obstruction: It may be due to haematoma (if it is so, the haematoma has to be evacuated), or due to laryngeal oedema, or due to tracheomalacia or bilateral RLN palsy (emergency endotracheal intubation is done along with steroid injections). Often emergency tracheostomy may be required as a life-saving procedure. Laryngeal oedema is the commonest cause may be due to haematoma with tension along with intubation injury and surgical trauma. In such situation even though haematoma should be evacuated immediately to avoid further compression, airway obstruction will not be relieved due

Fig. 6.69: Note the location of parathyroid glands.   Note:    Earliest symptom of hypocalcaemia is muscle weakness.

5. Thyroid crisis/thyroid storm/thyrotoxic crisis It is a rare but severe life-threatening complication of hyperthyroidism with acute hypermetabolic state induced by release of excessive thyroid hormones. Crisis can be due to surgical or medical causes.

Causes ™™ It occurs in a thyrotoxic patient inadequately prepared for

thyroidectomy or thyrotoxic patient presents with crisis following an unrelated operation or stress.

Do not waste one moment in regret, for to think feelingly of mistakes of the past is to re-infect yourself.

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at the level of inferior horn of thyroid cartilage. Recurrent laryngeal nerve is in close contact with suspensory ligament of Berry. Inferior thyroid artery which is a branch of thyrocervical trunk ascends upwards reaching gland at its lower pole after turning towards midline behind the carotid artery. Here ligation is done at capsular level by identifying every small branch entering the gland (capsular ligation of inferior thyroid artery). This retains the blood supply of parathyroids which is very important. In olden days, ligation of inferior thyroid artery was done away from the gland often in continuation using absorbable suture material, is now no longer in practice. Mobilized gland is removed. Critical points of recurrent laryngeal nerve injury are—at the entry of inferior thyroid artery and crossing the nerve, at suspensory ligament of Berry, at lower pole of the gland.

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Complications of thyroidectomy

Metabolic

Nerve injuries

Other complications

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

• External laryngeal nerve injury – pitch of the voice is lost • Recurrent laryngeal nerve injury Vascular complications • Haemorrhage – primary and reactionary • Haematoma formation • Compromised tracheo-oesophageal blood supply

• • • •

Intraoperative

Immediate postoperative

Late postoperative

• Haemorrhage • Nerve injuries

• • • • •

• • • • •

Haematoma Laryngeal oedema Respiratory obstruction – stridor Hypoparathyroidism RLN injury and its problems

Respiratory obstruction Skin flap necrosis – rare Infection Discharge and sinus formation • Wound granuloma • Keloid formation

Hypothyroidism Recurrent nodule Recurrent toxicity Wound infection Granuloma/keloid

™™ Other causes are—infection, trauma, pre-eclampsia, diabetic

™™ Antiadrenergic drugs: Propranolol 80 mg orally or through

ketoacidosis, emergency surgery, stress, drugs like anticholinergics or antiadrenergic or NSAIDs or chemotherapy, diabetes mellitus.

nasogastric tube 6th hourly, or IV propranolol 1 mg IV in 10 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 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; score 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.

6. Injury to external laryngeal nerve It causes weakness of cricothyroid muscle leading to alteration in pitch of voice, voice fatigue, breathy voice, frequent throat clearing. ILS reveals short hyperaemic vocal cords, lower level affected vocal cod, oblique glottis chink due to rotation of the posterior commissure to the paralyzed one.

7. Hypothyroidism.

10. Recurrent thyrotoxicosis is 5% common. It is due to retaining of more thyroid tissue during thyroidectomy for toxic thyroid. It is difficult to manage. It is treated with antithyroid drugs, radioiodine therapy or re-excision surgically which is technically demanding (so often avoided).

B xx xx xx xx xx xx

xx

xx

xx

xx

Remember in thyroidectomy

Middle thyroid vein is present only in 30% of cases Bipolar cautery or harmonic scalpel (harmonic wave) are better to control bleeding and to prevent injury to RLN RLN should be identified in both sides. Variations should be remembered Parathyroids should be retained and also their blood supply Parathyroid autotransplantation may be needed if their blood supply is compromised Rapid influx of serum calcium into bones in immediate postoperative period may cause severe hypocalcaemia— hungry bone syndrome which is initially corrected by IV calcium gluconate—10 ml of 10%. It is more commonly observed in patients with beta blockers. It is due to sudden drop in PTH level after surgery Tension haematoma under strap muscles is very dangerous and should be relieved by removing sutures from the skin and strap muscles Permanent hypoparathyroidism is rare (0.5–1%) whereas temporary hypoparathyroidism and hypocalcaemia is common (25%) which is corrected well with calcium for certain period of 3–6 months. External laryngeal nerve injury is more common than of RLN. There is loss of tension of vocal cords causing reduced power and range in voice (pitch) Thyroid insufficiency develops in 25–45% cases. It is confirmed by doing serum T3, T4 and TSH after 6 months

KOCHER’S TEST In case of a huge or long-standing thyroid enlargement, both lateral lobes of thyroid are pushed posteromedially with fingers. The test is positive, if it produces stridor. This signifies weakened tracheal (rings) cartilage because of constant pressure by the thyroid gland. But then trachea is kept patent by the thyroid itself. Once the gland is removed there is no support to trachea and so it collapses causing stridor (scabbard trachea). Such patient requires temporary tracheostomy for 2–3 weeks. By then trachea regains its strength back.

HYPOTHYROIDISM

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After thyroidectomy, common cause xx Agenesis or dysgenesis; Enzyme deficiency xx Iodine deficiency; Hashimoto’s thyroiditis; Antithyroid drugs xx Radioiodine xx Drugs: Lithium, amiodarone

Primary—is due to thyroid diseases or removal of thyroid. Secondary—is due to hypopituitarism. Tertiary—is due to hypothalamic diseases.

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EMIL THEODOR KOCHER

xx

We have arrived at the point where we generally recommend the radical operation for goitre as the surest and simplest method of treatment…. Surgeons have simply assumed that the thyroid gland has no function whatever…. Patients with total excision…all show more or less severe disturbances in their general condition… We cannot fail to recognise (the) relation to idiocy and cretinism… We see no objection for the time being, to the use of the name cachexia strumipriva. —Emil Theodor Kocher, 1883 He is the first surgeon to get Nobel prize. He did extensive work on thyroid surgeries and designed present technique of thyroid surgeries.

Causes

xx xx xx xx

Cretinism

It is foetal/infantile hypothyroidism due to inadequate thyroid hormone production during fetal and neonatal period. It may be due to agenesis, inborn error of thyroid metabolism, dyshormonogenesis or dietary deficiency (endemic). Typical hoarse cry, macroglossia, umbilical hernia, thickened skin are the features. TSH will be raised; T3 and T4 will be low. Incidence is 1:4000 live births. It is treated with L thyroxine once a day morning orally.

Features ™™ General: Tiredeness, weight gain, cold intolerance, goitre,

hyperlipidaemia. ™™ Cardiovascular: Bradycardia, angina, cardiac failure, peri-

cardial effusion.

Everyone should be quick to listen and slow to speak.

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Revealed clinically after 6 months. 8. Wound infection, stitch granuloma formation. 9. Keloid formation.

He was from Switzerland. He was the founder of: ™™ Kocher’s vein. ™™ Kocher’s forceps (has got tooth in the tip). ™™ Kocherisation (Duodenal mobilisation). ™™ Kocher’s incision (Right subcostal for cholecys­tectomy). ™™ Kocher’s thyroid incision. ™™ Kocher’s test. Kocher’s eye sign. ™™ Kocher’s method for reduction of shoulder dislo­cation. He died in 1917. He is the father of thyroid surgery. Other surgeons who got Nobel prizes are—Alexis Carrell, for his work on vascular anastomosis; Christian Bernard, for heart transplantation, Charles Huggins, urologist, for management of carcinoma prostate.

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™™ Haematological: Anaemia. ™™ Dermatological: Dry skin, vitiligo, alopecia, erythema.

SRB's Manual of Surgery

™™ Reproductive: Infertility, menorrhagia, galactorrhoea. ™™ Gastrointestinal: Constipation, ileus.

™™ Investigations: T3, T4 estimation; TSH level estimation which

is higher.

™™ Treatment: ¾¾

™™ Developmental: Growth and mental retardation, delayed

puberty. ™™ Other features: Carpal tunnel syndrome, myalgia, hoarse-

ness, deafness, ataxia, depression, psychosis (Myxoedema madness).

¾¾

Replacement with L-thyroxine 100 to 150 µg/day. In old patients with ischaemic heart disease initial therapy is with 25–50 µg/day and then gradually increased up to the required dose. Initial rapid response can be achieved by giving L iodothyronine 20 µg tid.

RECURRENT LARYNGEAL NERVE PALSY Anatomy and Relations of Nerves with Thyroid Gland ™™ External laryngeal nerve is a branch of superior laryngeal

A

B

Figs. 6.70A and B: Typical myxoedema patient.

T

Myxoedema coma

Characteristic features

Precipitated Treatment by

Hypothermia

Cold

Drug of choice—T3 , (Levothyronine) 10 µg given IV 6th hourly

Hypotension

Infection trauma

Levothyroxine—300 µg IV/1000 µg orally (large dose)

Hyponatraemia

Glucocorticoids

Hypoventilation

Antibiotics

Hypoglycaemia

Slow warming

Bradycardia

Electrolyte management

Skin is deadly cold like of a todd Coma Note: Myxoedema is severe thyroid failure.

nerve. Vagus gives superior laryngeal nerve branch at inferior ganglion of vagus which is at the level of greater cornu of hyoid bone, which in turn, divides into internal laryngeal and external laryngeal nerves. Internal laryngeal nerve pierces the thyrohyoid membrane and gives sensory supply to larynx above the vocal cord and hypopharynx. External laryngeal nerve runs close to superior thyroid vessels to supply cricothyroid muscle which is tensor of vocal cord. It may get injured while ligating superior pedicle causing defective pitch of the voice. So upper pedicle of thyroid should be ligated close to gland. ™™ Recurrent laryngeal nerve is a branch of vagus which hooks around ligamentum arteriosum with arch of aorta on the left and right subclavian artery on the right side. It runs in the tracheo-oesophageal groove near the posteromedial surface close to thyroid gland. Nerve lies in between branches of inferior thyroid artery. So inferior thyroid artery should be ligated away from the gland. Anomalies of the nerve should be remembered. In 5% of cases nerve passes through the gland. Nerve may be much more away from the gland. Nerve may be closely adherent to gland posteriorly. Nerve may lie within the ligament of Berry (25%). Non-recurrent laryngeal nerve may be present in 1 in 1,000 cases with a horizontal course. RLN supplies all muscles (abductors, adductors) of the larynx except cricothyroid and also gives sensory supply of larynx below the vocal cords.

Muscles of the Larynx ™™ Cricothyroid: It is the only muscle which is located on the

external aspect of the larynx. It is supplied by external laryngeal nerve. It is tensor and mild adductor of the vocal cord. ™™ Abductors of the vocal cord—posterior cricoarytenoids. ™™ Adductors of the vocal cord—lateral cricoarytenoids, transverse arytenoid, thyroarytenoids and cricothyroids. ™™ Relaxant of vocal cords—thyroarytenoids and vocalis. ™™ Muscles which close the laryngeal inlet—oblique arytenoids and aryepiglottic. ™™ Muscles which open the laryngeal inlet—thyroepiglotticus.

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Positions of Vocal Cord

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Median; paramedian (1.5 mm); normal; cadaveric/neutral (3.5 mm); gentle abduction (7 mm); completely abducted (9.5 mm).

Fig. 6.72: On table identification of recurrent laryngeal nerve is a must during thyroidectomy. 

B xx xx xx xx xx

A

Assessment of voice change

Pitch of the voice—whether raised/lowered or pitch locked Breath support during speaking is adequate or not Ability to alter the rapidity of speech—slow/fast/ medium Altered laryngeal and neck muscle tension Indirect laryngoscopy—with tongue pulled out using gauze, warmed ILS is placed into the oral cavity to see vocal cords. Patient is asked to say ‘e’ to see the vocal cord movements

Types 1. Unilateral recurrent laryngeal nerve palsy. 2. Bilateral recurrent laryngeal nerver palsy. 3. Unilateral, combined recurrent laryngeal and superior laryngeal nerve palsy. 4. Bilateral, combined recurrent laryngeal and superior laryngeal nerve palsy.

1. Unilateral recurrent laryngeal nerve palsy All intrinsic muscles of the larynx except cricothyroid are paralysed. Vocal cord becomes median or paramedian position. Reasons are: a. Only retained cricothyroid is a weak adductor of vocal cord causing median or paramedian position of vocal cords (Wagner-Grossman hypothesis). Causes for unilateral recurrent laryngeal nerve paralysis Thyroidectomy Infiltration from carcinoma thyroid Infiltration from carcinoma oesophagus Infiltration from carcinoma bronchus (left-sided) Mediastinal tumours (left-sided) Aortic aneurysms Trauma Idiopathic Neck or mediastinal lymph node mass

B xx xx xx xx

B

xx

Figs. 6.71A and B: Anatomy of superior and recurrent laryngeal nerve. Superior laryngeal nerve divides into internal and external laryngeal nerves. Right recurrent winds round subclavian artery and left winds around arch of aorta.

xx xx xx xx

The same beam of light can illuminate two objects and produce two different effects.

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b. Abductor fibres which are phylogenetically newer (to posterior cricoarytenoid) of the recurrent laryngeal nerve is more susceptible and paralysed than adductor fibres (Semon’s law). ™™ Features: Asymptomatic in 33% cases. Some change in

voice, which gradually becomes normal with speech therapy. Aspiration and airway obstruction never occurs. ™™ Treatment: No specific treatment is required. But steroid should be started. Prednisolone 20 mg tid for 10 days orally after food is given with gradual tapering in another 10 days.

2. Bilateral Recurrent Laryngeal Nerve Palsy ™™ Both side intrinsic muscles are paralysed. So both vocal

cords lie in median or paramedian position due to unopposed actions of the both side cricothyroid. ™™ Features: Change in voice; severe dyspnoea and stridor (more during exertion) leading to airway block and respiratory arrest. ™™ Treatment: Emergency tracheostomy is needed. Lateralisation of the cord is done by: Arytenoidectomy by open surgery or through an endoscope; or by vocal cord lateralisation through endoscope. Often excision of vocal cord through an endoscope or laser cordectomy is required. Implantation of sternohyoid, and thyroplasty are other procedures used. .

3. Unilateral, combined recurrent laryngeal nerve and superior laryngeal nerve palsy ™™ All the muscles on one side are paralysed. Vocal cord is in

cadaveric position, 3.5 mm from the midline. ™™ Features: Hoarseness of voice; aspiration through ineffective glottis; ineffective cough. ™™ Treatment: Speech therapy (Commonly healthy opposite cord moves to opposite side of the midline so as to compensate for the paralysis of the opposite side); injection of Teflon to the paralysed cord; muscle or cartilage implant to the paralysed cord; arthrodesis of cricoarytenoid joint.

4. Bilateral, combined recurrent laryngeal nerve and superior laryngeal nerve palsy ™™ Causes: All (total) paralysis of intrinsic muscles of larynx;

total laryngeal anaesthesia.

™™ Features: Aphonia (no voice); aspiration due to severe glottis

incompetence and laryngeal anaesthesia; absence of cough; retention of secretions in the chest; respiratory arrest. ™™ Treatment: Emergency tracheostomy; fixing epiglottis over the arytenoids to prevent aspiration; plication of vocal cords to prevent aspiration; often needs total laryngectomy. xx xx xx xx

Fig. 6.73: Vocal cord in recurrent laryngeal nerve palsies.

Unilateral recurrent laryngeal nerve palsy Bilateral recurrent laryngeal nerve palsy Unilateral combined palsy Bilateral combined palsy

– Not dangerous – Most dangerous – Not dangerous – Dangerous

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Parathyroids and Adrenals About three years ago I found on the thyroid gland…a small organ, hardly as big as a hemp seed, which was enclosed in the same connective tissue capsule as the thyroid, but could be distinguished therefrom by a lighter color. A superficial examination revealed an organ of a totally different structure from that of the thyroid, and with a very rich vascularity…. (I) suggest the use of the name Glandulae parathyreoideae; a name in which the characteristic of being bye-glands to the thyroid is expressed. —Ivar Victor Sandström, 1879

C hapter Outline ·· ·· ·· ·· ·· ·· ·· ·· ··

Anatomy Calcium Hyperparathyroidism Parathyroidectomy Men Syndrome (MEA Syndrome) Apudomas Hypoparathyroidism Tetany Adrenals

·· Adrenal Cortical Tumours ·· Adrenocortical Carcinoma ·· Cushing’s Syndrome ·· Conn’s Syndrome ·· Virilising Syndrome or Adrenogenital Syndrome ·· Neuroblastoma ·· Phaeochromocytoma

is usually adjacent to the anastomosis between superior and inferior thyroid arteries posteriorly. ™™ Glands (chief cells) secrete parathormone (PTH) which controls the calcium metabolism. ™™ Variations are common in inferior parathyroids. It can be located in thyrothymic ligament, superior pole of thyroid, tracheo-oesophageal groove, behind oesophagus, carotid sheath. There may be more than two glands on one side called as supernumerary gland (2.5–22%). ™™ Both superior and inferior glands receive their blood supply from inferior thyroid artery and its anastomotic branch. Blood supply is through an end arterial branch. ™™ Gland contains chief and oxyphil (water clear) cells.

B

ANATOMY ™™ Parathyroids are endocrine glands situated behind the thyroid

gland. They are four in number two on each side.

™™ Two upper glands are constant in position. Superior is behind

recurrent laryngeal nerve. They develop from 4th pharyngeal pouch hence called as parathyroid IV. ™™ Two lower glands are variable in position. They develop from endoderm of 3rd pharyngeal pouch hence called as parathyroid III. It is usually in front of the recurrent laryngeal nerve, lower part. ™™ Inferior parathyroids are larger than superior; adenoma is common in inferior parathyroids. Inferior parathyroid and thymus develop from 3rd pharyngeal arch. ™™ Each gland weighs 40–50 mg. It is brownish (khaki coloured) firm gland, which sinks in the fluid unlike fat which floats. It

xx xx xx xx xx xx xx

Parathormone—PTH

PTH is a peptide of 84 amino acids. PTH secretion is not dependent on pituitary gland. It converts vitamin D into 1, 25-dihydrocholecalciferol in the kidney, an active principle. Increases absorption of the calcium from the gut Mobilizes calcium from the bone Increases the calcium reabsorption from the renal tubules Half life of PTH is 4 minutes.

CALCIUM Total calcium in plasma in ionised and nonionised (unbound and bound) form is 8.5–10.2 mg/dl (2.2–2.5 mmol/L). 55% is bound; 45% is unbound ionised free active part (4.5–5.0 mg/dl). Commonest protein part of bound calcium is albumin (80%);

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remaining is beta globulin, non-protein molecules. Ionised calcium is inversely related to pH of blood; raise in pH by 1 will reduce the ionized calcium by 0.36 mmol/L. Levels of calcium is controlled by PTH, calcitonin and vitamin D acting on bone, kidney and GIT. PTH (84 amino acids) is secreted by chief cells whenever serum calcium level falls. PTH stimulates osteoclasts for bone resorption; kidney to promote calcium reabsorption and production of 1,25-dihydroxy vitamin D; GIT to promote absorption of calcium and phosphorous. Vitamin D hydroxylation at carbon 25 occurs in liver, and later at carbon 1 occurs in kidney. It is important for blood coagulation, neuromuscular activity, cellular activity, bone integrity.

Fig. 7.1: Anatomical location of parathyroids.

HYPERPARATHYROIDISM (HPT) Types: 1. Primary. 2. Secondary. 3. Tertiary. Primary HPT is unstimulated inappropriate high PTH secretion due to hyperplasia or adenoma causing hypercalcaemia. It is hypercalcaemia with unsuppressed and so elevated PTH level. Primary hyperparathyroidism may be due to adenoma (75–90%), hyperplasia (20–24%) or carcinoma, rare (1%). Its prevalence is 0.5%. Mostly present with vague symptoms with bone pain. Sporadic cases are common. Familial association with type 1 or 2A can occur. Secondary HPT is due to chronic renal failure or due to mala­ bsorption, vitamin D deficiency rickets, i.e. due to chronic hypocalcaemia. There is always parathyroid hyperplasia. ™™ Chronic kidney disease causes hyperphosphataemia and reduced conversion of 25 DHCC to 1, 25 DHCC. So absorption of calcium from GIT is reduced causing chronic hypocalcaemia which stimulates PTH secretion and parathyroid hyperplasia. In secondary hyperparathyroidism, cause is not in the parathyroids but away from that; all four glands get affected here. ™™ Anemia which is resistant to erythropoietin stimulation; bone, joint, muscle symptoms; progressive ectopic calcification;

dilated cardiomyopathy with cardiac failure; features of CKD—are the features. ™™ Glands often become nodular causing it refractory to medical therapy. Serum phosphate level is higher (>6.g/dL); with low serum vitamin D. Serum calcium is normal or low; persistently high intact PTH >500 pg/mL; estimated volume of the largest gland is >500 mm3 or long axis is >1 cm; metastatic calcification is common; vertebral body shows ‘Rugger Jersey pattern’ (simulates transverse band of rugby jersey). Bone densimetry shows features of osteoporosis. Osteitis fibrosa cystica can also occur. ™™ Calciphylaxis can occur with disseminated vascular and cutaneous calcifications (skin necrosis) is due to calcific uremic arteriolopathy (tunica media). Sepsis and gangrene is common. Calciphylaxis is due to reduction in a 2 Heremans Schmid glycoprotein, a calcification inhibitory protein. Calciphylaxis carries high mortality. Treatment is dialysis, wound care, hyperbaric oxygen, cincacalcet, sodium thiosulphate and emergency parathyroidectomy, renal transplantation. ™™ Treatment: Calcimimetic drugs, vitamin D, subtotal parathyroidectomy, renal transplantation. Tertiary HPT is due to autonomous reactive hyperplasia of parathyroids causing hyperparathyroidism and hypercalcaemia seen after renal transplantation. Serum calcium and PTH will be high but serum phosphate will be low. It requires subtotal/total parathyroidectomy with autotransplantation. Carcinoma of parathyroid: It is rare—1%. Prevalence is 0.005%. ™™ Risk factors are—earlier neck irradiation, chronic kidney disease and genetic (mutation of HRPT2 gene), HPT-JT syndrome (hyperparathyroidism—jaw tumour). ™™ It is slow growing but 95% of them are functioning tumours with severe hypercalcaemia (serum calcium >14 mg/dL). ™™ It presents as nodule (50%), palpable neck lymph nodes along with HPT. It is invasive to soft tissues, thyroid, perineural spaces. Often blood spread can occur. It clinically mimics thyroid nodule. ™™ Histologically, it shows thick fibrous bands (90%); mitotic Activity (80%); capsular invasion (65%) and vascular invasion (15%). It is equal in both sexes. ™™ Hypercalcaemia is common. US neck, CT/MRI neck is useful. Immunohistochemistry for parafibromin and protein gene product 9.5 (PGP 9.5) is very useful. Raised levels of serum immunoreactive PTH (iPTH) is diagnostic. Tech99 labelled sestamibi isotope scan is useful. ™™ Treatment: It needs wide en bloc tumour excision, hemithyroidectomy, central or lateral neck node dissection and later radiotherapy to neck and mediastinum. Control of hypercalcaemia is very important [hydration, bisphosphonates (pamidronate, zoledronate), cinacalcet (90 mg four times a day) and denosumab (120 mg/month)]. Chemotherapy and radiotherapy has got limited benefits even though tried. With therapy, 5-year survival rate is 85%.

Primary Hyperparathyroidism

Clinical Features ™™ Clinical vignette of hyperparathyroidism: “Bones, stones,

abdominal groans and psychiatric moans”. As palpable neck swelling is uncommon, disease is suspected by various clinical features and so to confirm by biochemical evaluation (PTH assay). ™™ It is common in middle-aged women (3:1). ™™ Incidence is 1:1000 patients. ™™ Presentation may be asymptomatic >50% cases—(Asymptomatic hypercalcaemia). ™™ Bones: Raised PTH causes increased osteoclastic activity leading into extensive decalcification of the bone resulting in bone pain, subperiosteal erosions on the radial aspect of the middle phalanges and clavicle. There will be osteitis fibrosa cystica (von Recklinghausen disease of hyperparathyroidism/brown tumour of hyperparathyroidism—15% cases of PTH) which shows single or multiple cysts or pseudotumours in the jaw, skull or phalanges. There will be osteopenia, osteoporosis and pathological bone fractures. Acquired trachyonychia (racquet nails) suggests bone resorption. Lamina dura of tooth is the first bone to show changes. Brown tumour is seen in long bones. ™™ Stones: In the kidney due to hypercalcaemia, renal stones (25%) develop which are usually multiple. 3% of all renal patient stones is due to primary PTH. Recurrent stones are commonly observed in PTH. Calculi are usually calcium phosphate or oxalate types. Metastatic calcification of the renal parenchyma can occur resulting in nephrocalcinosis resulting into renal failure. Calcification occurs in the renal vessels leading into renal hypertension. ™™ Abdominal groans: Hypercalcaemia stimulates gastrin release which causes peptic ulceration. Hypercalcaemia can precipitate acute pancreatitis. Gallstone disease is increased due to hypercalcaemia; calcium bilirubinate type is the one usually observed. ™™ Psychic moans: Behavioural and neurotic problems with depression, anxiety; present as nonspecific symptoms.

ulceration, band keratopathy (identified through slit-lamp examination), pseudogout, myalgia, joint pain, back ache, polyuria, polydypsia, constipation, glycosuria and hypertension. Proximal myopathy and muscle wasting can occur. ™™ MEN syndrome (I and IIA) can co exists. Primary HPT is the earliest and commonest manifestation of MEN I syndrome and develops in more than 80% of these patients. ™™ Calcinosis of PTH can present as renal stones, gallstones. Metastatic calcifications occur in kidney (nephrocalcinosis), chondrocalcinosis, calcifications in skin, vessels, cardiac valves and soft tissues. ™™ On examination of the neck, it is not common to reveal a palpable swelling of parathyroid enlargement. xx xx xx xx xx xx

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Primary HPT is commonly sporadic than familial Histological difference between adenoma and hyperplasia is difficult to assess On table gross look is preferred method Adenoma is usually single. Multiple adenomas occur in elderly (5%) Hyperplasia involves all four parathyroids Parathyroid cyst may be developmental/secondary to degeneration of nodule or adenoma. Cyst aspiration shows clear fluid with high PTH level in the content fluid Preoperative IV methylene blue 5 mg/kg in 500 mL dextrose saline which stains parathyroids is used often to localise glands 50 percentage of one gland is sufficient to maintain function Persistent HPT is one in which HPT persists immediately even after initial surgery Recurrent HPT (initially after first surgery HPT is corrected but recurs 12 months after surgery) is due to parathyromatosis, development of new adenoma, and hyperplasia of transplanted parathyroid. Parathyromatosis is due to rupture and spillage of parathyroid tissue in neck, mediastinum forming functioning nodules Re-exploration and removal in persistent/recurrent HPT needs on table PTH assay. On table PTH level drop more than 50% of preoperative value indicates the success of surgical removal CT, MRI, PET scan and selective angiography are usually done only for recurrent diseases

Acute Hyperparathyroidism (Crisis) Causes ™™ Sudden increase in PTH level due to rupture of parathyroid

cyst or bleeding in the parathyroid tumour. ™™ Severe dehydration precipitates crisis. ™™ Secondaries in bone (primary may be from breast). Note: Acute hyperparathyroidism is rare but dangerous pre­sentation (crisis) wherein patient presents with abdominal pain, vomiting, dehydration, oliguria, muscular weakness and death. Serum Ca++ is very high >12 mg% (3.5 mmol/L). Serum calcium >4.5 mmol/L (>14 mg/dL) causes coma and death.

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Primary HPT is 3rd most common endocrine disease after diabetes and thyroid disease. Increased PTH causes hypercalcaemia. Adenoma is the most common cause; adenoma is common in inferior parathyroid. Hyperplasia, carcinomas are less common causes. Exact etiology is unknown; commonly it is sporadic. Familial or genetic causes are known to occur. MEN I syndrome, hyperparathyroidism–jaw tumour syndrome (HPT–JT syndrome) are often associated. Therapeutic ionizing radiation (like RAIT) can cause hyperparathyroidism. Lithium can cause parathyroid hyperplasia and HPT but without causing bone or renal problems.

™™ Other features: They are prone for skin necrosis, corneal

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Treatment of crisis ™™ Forced diuresis using 3–5 litres of saline with frusemide.

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™™ Rehydration using normal saline 300 mL/hour. ™™ Steroids, inhibits effects of vitamin D. Dose is 400 mg/day

IV for 5 days.

™™ Clodranate Na+, Pamidronate (90 mg IV slowly in 4 hours,

is a biphosphonate inhibits mobilization of the calcium from the bone). Zoledronic acid 4 mg IV initially and later 8 mg is also used. ™™ Drugs to reduce Ca++ level, i.e. mithramycin, calcitonin, prednisolone, biphosphonates. Mithramycin rapidly controls the hypercalcaemia of malignant disease but temporarily. Cinacalcet is calcium receptor agonist which reduces the serum calcium level. Gallium nitrate inhibits osteoclast resorption of calcium at a dose of 200 mg/m2/day for 5 days. ™™ Condition has high mortality rate. Patient develops hyperchloraemic metabolic acidosis.

Fig. 7.2: Plain X-ray skull. Note the characteristic salt-pepper appearance of the skull bone.

Investigations for HPT ™™ High serum calcium level more than 10 mg%. Serum albumin

level also should be assessed to identify accurate ionic calcium level. Parameters

Primary HPT

Secondary HPT

Parathormone

High

High

Calcium

High

Normal

Phosphate

Decreased

Increased

™™ Increased serum PTH level is specific and diagnostic—

>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 xx xx xx

X-ray features in hyperparathyroidism

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.

A

B

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 diag-

nostic 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.

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).

B xx xx

Treatment

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Indications for parathyroidectomy

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 neck – sternomastoid (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, transcervical 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

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Problems in parathyroidectomy

Permanent hypoparathyroidism Persistent hyperparathyroidism—5% Recurrent hyperparathyroidism—hypercal­c aemia 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 absorption of calcium by bones—hungry bone syndrome

PARATHYROIDECTOMY Indications in Primary HPT ™™ Symptomatic HPT ™™ Asymptomatic HPT with—Criteria (2002) for surgical inter-

vention are: ¾¾ Raise in serum calcium level more than 1 mg/dL of upper limit of the normal calcium range; ¾¾ 24 hour urinary calcium if more than 400 mg; creatinine clearance when reduced more than 30%; ¾¾ Bone density greater than 2.5 standard deviations below peak bone mass in lumbar spine/hip/lower end of radius; ¾¾ Age below 50 years; ¾¾ When medical therapy is not possible.

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.

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 TID 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.

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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.

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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).

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 parathyroidectomy is done for parathyroid hyperplasia by removing all four 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.

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. 10 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 ¾¾ 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. ¾¾ 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. ¾¾ 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. ¾¾ Bone-specific alkaline phosphatase (ALP) continues to rise in the first few weeks indicating increased bone reconstruction. ¾¾ Supplementation of vitamin D and elemental calcium is needed during discharge for 6 months. ¾¾ Postoperatively they need calcitriol (gradually increased to 16 mg in 1 month, then gradually reduced) with 2 g calcium supplement. ¾¾ Monitoring is done by evaluating serum calcium, albumin, magnesium, phosphorus and bone specific alkaline phosphatase. ™™ Injury to recurrent laryngeal nerve, oesophagus can occur.

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%. Intraoperative 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.

Surgical Approaches

Endoscopic Parathyroidectomy

Classic approach (Traditional approach)

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.

Remedial Parathyroidectomy

Fig. 7.4: On table look of parathyroid adenoma of inferior parathyroid gland (left sided).

Minimally Invasive Parathyroidectomy (MIP) (Focused Parathyroidectomy) It is done in case of single adenoma of parathyroid under regional cervical block anaesthesia usually in ambulatory set up. It is actually unilateral parathyroidectomy with removal of adenoma and involved entire that particular one parathyroid gland using limited neck exploration (small incision of 2–4 cm). Procedure is not useful in multiple adenomas or hyperplasia. Preoperative localisation with sestamibi combined with SPECT is a must. Intraoperative PTH assay is a must to confirm drop in PTH level to required level. Cervical block is given by infiltrating the xylocaine (1%) with adrenaline along posterior and deep to sternocleidomastoid muscle and also along the neck incision. Direct field block along the anterior border of the sternocleidomastoid and over the incision is also used. Postoperative serum calcium and PTH level should be assessed until 7th day. It can be video assisted or radio guided parathyroidectomies also.

Video-assisted Parathyroidectomy (Paolo Miccoli) It is done in localised single adenoma using multiple ports on one side with intermittent CO2 insufflation and suction irrigation. 1.5 cm incision 1 cm above the sternal notch helps in tactile assessment and dissection. Strap muscles are retracted laterally and thyroid is retracted medially. Conversion rate is 11%; RLN palsy is 2%.

It is done for persistent HPT (serum calcium is not normalised immediately after surgery leading to total failure) or recurrent HPT (serum calcium after surgery becomes normal but in 6–12 months, it again increases). It is done through lateral approach (Feind) between anterior margin of sternocleidomastoid and strap muscles so that scar tissues are avoided from initial dissection. On table localisation methods, on table PTH assay are needed. Medial sternotomy often may be needed to explore the mediastinum for complete removal of the gland. Parathyroids in such situations may be embedded in the tissue of thyroid or thymus. So removal of these glands also may be a need in such situations.

Subtotal Parathyroidectomy It is indicated in hyperplasia or secondary HPT wherein 3½ glands are removed, retaining ½ of one gland.

Total Parathyroidectomy with Parathyroid Autotransplantation It is done alternatively in hyperplasia wherein all four glands are removed; 1/3 or ½ of one gland is transplanted into sternocleidomastoid or brachioradialis muscle with a marker. Disease control will not occur, if more part of the gland is transplanted. It is removing all four glands and ½ or 1/3 of one gland is autotransplanted into the forearm muscle (brachioradialis) or sternocleidomastoid muscle with marker stitch. Transplanting gland is sliced into 1 mm pieces and around 18 pieces are embedded in decided muscle with a marker stitch or clip. If postoperatively patient still presents with features of primary HPT; transplanted area is re-explored and further reduction in parathyroid tissue is done. Migration or inability to identify the

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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 surgeon. Many place trocars on one side only; but few prefer to place working trocars on opposite side.

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transplanted parathyroid is often a problem in autotransplantation of parathyroid.

MEN SYNDROME (MEA SYNDROME) MEN—Multiple Endocrine Neoplasia; MEA—Multiple Endocrine Adenomatosis. It is the endocrine syndrome complex usually inherited as an autosomal dominant (MEN type 1); could be due to inactivation of the MEN type 1 tumour suppressor gene 11q13; but occasionally it can be sporadic. It is mutation of RET (REarranged during Transfection) protooncogene in MEN type 2 in chromosome 10. Types: MEN type 1; MEN type 2a; MEN type 2b. MEN Type 1: 3 PS (Parathyroid; Pancreas; Pituitary); it is also called as Wermer’s syndrome (1954). Here the defect is in chromosome 11q13 (Menin related). At least two of the above target endocrines involvement or in familial MEN type I one target endocrine tumour in a first degree relative. ™™ Hyperparathyroidism (90%) due to parathyroid hyperplasia or adenomas is the most common manifestation. Unlike other primary HPT, in MEN type 1 HPT occurs in younger age group; multiple glands are involved; recurrence is high after parathyroidectomy. It occurs in young age group (20–25 years). Serum calcium, PTH, Tech99 labelled sestamibi isotope scan, imaging of neck are done. ™™ Pancreatic islet (endocrine/neuroendocrine) tumours are 2nd common (80%). Gastrinomas are the commonest type (55%); insulinomas are 2nd common (30%); glucaganomas, VIPOMAs are other tumours which can occur in MEN type 1. Usually they are multiple microadenomas (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: ¾¾ High amine content. ¾¾ Capacity of amine precursor uptake. ¾¾ Property of decarboxylation of these precursors to form amines. ™™ Initially it is thought that APUD 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: ¾¾ Orthoendocrine: (1) Tumors secreting normal polypeptides of their cells of origin, e.g. Insulinoma, astrinoma, pancreatic glucagonoma, calcitoninoma. (2) Tumors secreting normal amines of their cells of origin, e.g. Phaeoechromocytoma, neuroblastoma and ganglioneuroma (adrenal medulla) and carcinoid tumor. ¾¾ 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. Insulinoma, 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.

Features of Hypoparathyroidism

™™ Hypoparathyroidism is defined as a PTH level 90% of Cushing’s syndrome. ™™ Low dose dexamethasone suppression test: Dexamethasone is a potent glucocorticoid that suppresses adrenal production of corticosteroids in normal persons but not in persons with Cushing’s syndrome. 1 mg of dexamethasone is injected; normal— 10 mg/dL. ™™ Late night salivary cortisol—Cortisol concentration in saliva correlates with free plasma cortisol; it is independent of salivary flow rate; it shows high sensitivity and specificity. ™™ Negative urinary free cortisol and Low dose dexamethasone suppression test rules out Cushing’s syndrome. ™™ Measurement of corticotropin (ACTH): if low—corticotropin independent Cushing’s syndrome; if high—corticotropin dependent Cushing’s disease. ™™ Localising methods/imaging: Adrenal—CT scan/MRI abdomen; Iodocholesterol scan. Pituitary—MRI pituitary. It can be incidentaloma in pituitary. Inferior petrosal sinus sampling of blood for ACTH shows central to peripheral ratio of ACTH >3.

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 (Aldosteronoma)—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 (PRA); PAC: PRA >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. ¾¾ 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. ¾¾

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%).

Types

™™ Hypertension, fever, weight loss, anaemia, flushing (due to

Pathology

catecholamine release) and sweating.

™™ Diarrhoea, hypokalaemia due to release of Vasoactive Intes-

tinal Polypeptide (VIP). Other hormones like ACTH are also released.

B xx

™™ 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.

Clinical Features

xx xx

xx

xx xx

Staging of neuroblastoma

Stage I: Localised tumour 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 III: Unresectable unilateral tumour infiltrating across midline/localised tumour with opposite side node spread/bilateral nodal spread/midline tumour with bilateral infiltration Stage IV: Any primary tumour with spread to distant nodes, bone, bone marrow, liver, skin and other organs Stage IV S: Localised primary tumour (stage I, IIA, IIB) with spread to skin, liver or bone marrow—limited to infants younger than one year

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 III without N myc amplifica-

tion; Stage III with favourable Shimada’s histology.

™™ Child presents as a huge mass per abdomen, in the loin which

™™ High-risk groups—all patients with N myc amplification;

stage IV neuroblastoma.

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.

Wilm’s tumour, which is mobile, with smooth surface, moves with respiration, does not cross the midline.

Fig. 7.6: Left-sided adrenal neuroblastoma with secondaries in orbit. Note scar of left-sided adrenalectomy.

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.

Differential Diagnosis

A

B

One only gets to the top rung of the ladder by steadily climbing up one at a time.

CHAPTER 7    Parathyroids and Adrenals

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.

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Investigation ™™ Ultrasound, CT to assess the mass and secondaries in liver.

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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% cases. It is taken from the iliac bones both sides under general anaesthesia ™™ PET scan is done if MIBG is not beneficial. ™™ Elevated ferritin, LDH and NSE (neuron specific enolase) is observed

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 (GD2), radionuclide targeted therapy, angiogenesis/ tyrosine kinase/aurora kinase inhibitorsare on trial. Bone marrow or stem cell transplantation are newer methods used.

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; N myc 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 Zuckerkandl). 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 intes-

tinal 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, SDHD. ™™ 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 lack the enzyme PNMT. ™™ Commonly benign (90%).

Tumour is: 10% malignant xx 10% extra-adrenal xx 10% bilateral xx 10% familial xx

xx xx xx xx

10% childhood 10% multiple 10% not associated with hypertension 10% calcified

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-IIa or MEN-IIb 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, phaeochromo­ cytoma), 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)

™™ 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. I131 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.

Treatment

• • • •

B xx xx xx xx

xx xx

Adrenalectomy ™™ Before surgery, BP is controlled initially by α-blocking agent,

phenoxybenzamine or doxazosin; then by β-blocking agent, propranolol. Then during surgery, sodium nitroprusside IV infusion is used. ™™ Adequate fluid therapy to have volume expansion is essential. ™™ Careful anaesthetic management with a good postoperative care is very essential. In case of bilateral presentation, opposite side can be operated in later period. ™™ Intraoperative hypovolemia and postoperative hypotension is a demanding situation for anaesthetist to manage. ™™ Handling of adrenal tumour on table must be careful and gentle. ™™ Adrenal vein should be ligated first. CVP, arterial lines should be present for monitoring. ™™ Rupture and spillage of tumour should be prevented.

xx xx xx xx xx xx

Remember

Pheochromocytoma is rarely malignant in MEN II Pheochromocytoma under 40 years of age suspect MEN 2A, VHL Beta-blocker is given only after patient is fully alpha blocked with phenoxybenzamine (20–60 mg/day) or doxazocin Alpha-blocker is given 4 weeks prior to surgery to control hypertension and beta-blocker is given one week before surgery to control tachycardia and arrhythmias Tumours, those secrete dopamine exclusively has got high malignant chances 5-year survival for malignant pheochromocytoma is 50%. It needs additional chemotherapy using vincristine, dacarbazine and cyclophosphamide. Pheochromocytoma in pregnancy has got 50% maternal mortality Vaginal delivery is contraindicated in pregnancy with pheochromocytoma Adrenal vein should be ligated first Avoid breach in the capsule of tumour during surgery Careful handling and haemodynamic monitoring is a must Sodium nitroprusside may be required on table to control the hypertension—10 µg/kg/minute

Malignant pheochromocytoma is 10% common. It is more common in extra-adrenal site. It commonly spreads to lymph nodes, bone and liver. Adrenalectomy in early tumour and debulking in advanced cases with α-blockers, mitotane and I 131MIBG therapy and combination chemotherapy are the therapeutic choices. Overall prognosis is not good with 5-year survival being 5 cm in size)—common in Africa.

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CHAPTER 8    Breast

Microscopy: 1. Intracanalicular—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.

Treatment

Fig. 8.8: Fibroadenoma, on table look—well-capsulated neoplasm. It is part of ANDI.

Excision through a circumareolar incision (Webster’s) or submammary incision (Gaillard Thomas incision) is done under general anaesthesia.

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.

A

Fig. 8.9: Large fibroadenoma left breast in a 14-year-old female.

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.

B

Figs. 8.11A and B: Multiple fibroadenomas.

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

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Fibroadenoma which is small (2 cm Size of giant gastric ulcer is >3 cm Size of giant fibroadenoma is >5 cm Diameter of transverse colon in toxic megacolon is >6 cm Size of giant naevus is >20 cm

Fig. 8.13: Diagram showing circumareolar and submammary (Gaillard Thomas) incisions.

FIBROCYSTADENOSIS (Fibrocystic disease of the breast/Mammary dysplasia/ CYCLICAL MASTALGIA WITH NODULARITY) ™™ It is due to Aberration of Normal Development and Involution

(ANDI) of breast causing changes in the breast.

™™ It is presently called as cyclical mastalgia with nodularity.

B xx xx xx xx xx

Microscopic changes

Stromal fibrosis Microcyst formation Glandular proliferation (Adenosis) Hyperplasia (Epitheliosis)—in ducts and acini Papillomatosis—within the ducts, often with apocrine metaplasia

™™ It is an estrogen dependent condition. One of the cysts may

A

get enlarged to become a clinically palpable, well-localised swelling—bluedome cyst of Bloodgood. It is fluctuant, transilluminant, nontender, often tensely cystic swelling (macrocyst) with thin bluish capsule. It should be aspirated initially. Surgical excision is done if it persists or recurs even after two aspirations; if it is blood stained; if there is residual lump after aspiration. ™™ When diffuse small, multiple cysts are the main component, it is called as Schimmelbusch’s disease. ™™ It is the most common breast disease. ™™ It is common in upper and outer quadrant. ™™ It is an exaggerated response of breast stroma and epithelium to hormones and growth factors. ™™ It is rare in nulliparous/ovulating/OCP taking women.

Classification ™™ Nonproliferative—moderate hyperplasia of ductal luminal

cells—no life time risk for cancer.

B

Figs. 8.12A and B: Subareolar and submammary incision to excise benign lesions of the breast.

™™ Proliferative without atypia (severe hyperplasia). ™™ Proliferative with atypia (Atypical ductal/lobular hyper-

plasia)—is risk factor for breast cancer—often mimics in situ carcinoma. RR is 4.5.

Stages I—Stromal proliferation or hyperplasia. II—Adenosis (increased glands). III—Cyst formation.

B xx xx xx xx xx xx xx xx xx

xx xx

Pathophysiology of fibrocystadenosis

Oestrogen predominance over progesterone is considered causative Serum levels of oestrogen is more Luteal phase is shortened Progesterone level decreased to 1/3rd normal Corpus luteum deficiency/anovulation in 70% Patients with premenstrual tension syndrome more likely to develop fibrocystadenosis Prolactin levels are increased in 1/3rd of women with fibrocystadenosis. It is probably due to oestrogen dominance on pituitary Thyroid in suboptimal levels sensitize mammary epithelium to prolactin stimulation Methylexanthiones derived from increased intake of coffee, tea, cold drinks, chocolate is associated with development of fibrocystadenosis Oestrogens stimulate proliferation of connective and epithelial tissues Fibrocystadenosis entails simultaneous progressive and regressive change

Clinical Features ™™ Presentation is during menstruating age group as a bilateral,

painful, diffuse, granular, tender, swelling which is better felt with palpating fingers (poorly felt with palm). ™™ Common in upper outer quadrant. ™™ Pain and tenderness are more just prior to menstrua­­tion (cyclical mastalgia). ™™ It subsides during pregnancy, lactation and after menopause. ™™ Discharge from the nipple when present will be serous or occasionally greenish. ™™ Occasionally shotty enlargement of axillary lymph nodes can occur (20%). ™™ Not fixed to skin, muscle or chest wall.

511

Investigations ™™ FNAC (Epitheliosis, when florid is undoubtedly premalignant). ™™ Ultrasound. ™™ Mammography.

Treatment 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: Oil of evening primrose used in moderate pain—drug of choice. It contains gamolenic acid which reverses saturated to unsaturated fatty acids. 1000–3000 mg/day for 4–6 months—but costly. It also contains 7% of linolenic acid and 72% of linoleic acid. ¾¾ Gamolenic acid—120 mg/day. ¾¾ Danazol—interferes with FSH and LH (gonadotrophin releasing hormone inhibitor); most effective drug; but second drug of choice; used in severe cases; 200 mg/day; very effective but causes acne, hirsutism, weight gain and amenorrhoea. It is teratogenic and so cannot be used if patient is planning for pregnancy. ¾¾ Bromocriptine—lowers prolactin—2.5 mg/day for 3 months. ¾¾ Tamoxifen—10 mg BD is an antiestrogenic drug. ¾¾ LHRH agonist (Goserelin) is reserved for refractory cases. It shows 96–99% success. But it causes reversible postmenopausal symptoms. ¾¾ Vitamin E and B are tried. 6 ¾¾ NSAIDs—oral and topical. ¾¾ Diuretics even though used by many—not effective. II. Surgery: ™™ Subcutaneous mastectomy with prosthesis place­ment—only in severe, persistent disease. ™™ Excision of the cyst or localised excision of the diseased tissue. ¾¾

B

Indications for surgery

xx

Intractable pain

xx

xx

Florid epitheliosis— on FNAC

xx

Persistent bloody discharge Bloodgood cyst

Clothe yourselves with compassion, kindness, humility, meekness and patience.

xx

Psychological reason

CHAPTER 8    Breast

Usual hyperplasia is presence of 3 or more layers of proliferating epithelial cells without atypia above the basement membrane in a lobular or ductal unit. It can be mild, moderate or florid. It is seen in 20% of biopsies. Risk of developing cancer is 1.5 times. Observation of such patient is the advice. Atypical ductal hyperplasia (ADH) is defined as a lesion that has got some but not all features of DCIS; a lesion that has all features of DCIS but less than 2 mm in size; lesion with all features of DCIS but involving less than 2 duct spaces. Its incidence is 30% of all mammographies and 5% of benign breast disease biopsies. Risk of developing cancer is 5 times the normal women of that age. Atypical lobular hyperplasia (ALH) is LCIS with half of the acini of lobular unit is involved. Developing invasive carcinoma in 10 years is 20%.

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Note: Subcutaneous mastectomy is removal of entire 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 nonepithe-

lial 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. ™™ Multiple, small, firm, nodules with fibrous tissue and tiny cysts are common pathology. ™™ Recurring pain alike cyclical mastalgia and often breast mass (20% cases) is the presentation. ™™ Tender breast often with palpable tender firm mass may be felt with granular surface. ™™ It contains proliferative terminal ductules and acini with proliferation of stroma often with deposition of calcium. Number of acini per terminal duct is increased more than double the number of normal lobule. There is lobular enlargement, fibrous stromal proliferation and distortion. ™™ Complex type is with papilloma and epithelial hyperplasia often with fibroadenoma. Radial scar is a variant of this. ™™ Condition mimics carcinoma clinically, radiologically and histologically. ™™ 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.

Fig. 8.14: Cystosarcoma phylloides of right breast. Note the dilated veins. Tumour occupies the entire breast.

Microscopy: ™™ It contains cystic spaces with leaf like projections, hence the

name (Phylloides—Greek—leaf-like).

™™ Cells show hypercellularity and pleomorphism. ™™ It may be a variant of intracanalicular fibroade­noma of breast

(Giant type).

Features ™™ They occur in middle aged or elderly. ™™ It is usually unilateral, grows rapidly to attain a large size with

bosselated surface.

™™ Swelling is smooth, nontender, soft, fluctuant with necrosis

of skin over the summit due to pressure.

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.

™™ Investigations: ¾¾ ¾¾ ¾¾

sarcoma, breast abscess and carcinoma breast.

Ultrasound; Mammography. FNAC, core biopsy Chest X-ray CT chest in malignancy to see secondaries.

Treatment ™™ Wide excision with 1 cm margin or subcutaneous mastec-

tomy to avoid recurrence. ™™ If malignant (sarcoma) total mastectomy is indicated with

adjuvant chemotherapy; but carries poor prognosis. Note: Enucleation should not be done. Benign type is 70% common; 3 mitoses/10 HPF; modest cellularity with uniform stroma; low Ki 67 index; negative IMP3; 10% recurrence. Borderline type is 15%; 4–9 mitoses/10 HPF; modest cellularity with heterogenous stroma; intermediate Ki 67 index; negative IMP3; 20% recurrence. Malignant type is 15%; 10 or more mitoses/10 HPF; cellularitry, stromal overgrowth and nuclear pleomorphism is marked; infiltrative margin with heterogenous elements; high Ki 67 index; positive IMP3; 50% recurrence; metastases can occur. AMOS criteria for predicting recurrence: Atypia; Mitoses; Overgrowth 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.

Types Cyclical—65%; Noncyclical—30%; Chest wall pain—5%.

Cyclical ™™ Pain related to menstrual cycles.

A

™™ 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”.

B

Figs. 8.17A and B: Recurrent cystosarcoma phylloides.

Treatment: ™™ Diclofenac gel—is very useful as local application. ™™ Evening primrose oil 325 mg BD. ™™ Gamolenic acid 120 mg bd. ™™ Danazol (100–200 mg BD)—antigonadotrophin agent. ™™ Bromocriptine (2.5 mg bd)—prolactin inhibitor. ™™ Tamoxifen (20 mg daily). ™™ GnRH analogue 3.6 mg injection depot-monthly. ™™ Testosterone undecanoate 40 mg bd. ™™ Vitamin B6, B12. ™™ Analgesics.

Gratitude, joy, love, passion, excitement, enthusiasm, hope, goal, satisfaction, humility, contentment – are different needs of happy life but difficult to acquire.

CHAPTER 8    Breast

™™ Recurrence is common. ™™ Differential diagnoses: Juvenile/giant fibroadenoma, angio-

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Noncyclical

SRB's Manual of Surgery

™™ 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. 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.

A

TRAUMATIC FAT NECROSIS It may be due to either direct or indirect trauma (trauma may not be noticed many times).

Pathogenesis Capillary ooze causes triglyceride in the fat to dissociate into fatty acids. It combines with calcium from the blood resulting in saponification which causes inflammatory reaction and later presents as a non­p rogressive swelling in the breast.

Features ™™ Painless swelling in the breast which is smooth, hard,

B

Figs. 8.18A and B: Traumatic fat necrosis.

nontender and adherent to breast tissue. ™™ It is nonprogressive, nonregressive. ™™ Investigations: ¾¾ FNAC shows chalky fluid with fat globules. ¾¾ Mammography to rule out malignancy. ™™ Differential diagnosis: Carcinoma breast. ™™ Treatment: Excision.

GALACTOCELE ™™ Seen in lactating women. ™™ Occurs during cessation of lactation. Often up to 10 months

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.

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Features ™™ Lump in the lower quadrant of the breast which is usually

unilateral, large, soft, fluctuant, with smooth surface.

CHAPTER 8    Breast

™™ 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 Types

Fig. 8.21: Subareolar abscess.

1. Subareolar.     2. Intramammary. 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.

Intramammary 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 xx xx xx xx xx

Precipitating factors

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 be 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: ™™ Continuous throbbing pain in the breast and high grade fever. ™™ Diffuse redness, tenderness, warmness and brawny indura-

tion in the breast. ™™ Purulent discharge from the nipple. ™™ Entire breast may get involved eventually. ™™ Occasionally tender fluctuant swelling (10%) may be felt;

Fig. 8.20: Diagram showing subareolar, intramammary and retromammary abscess.

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

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Often takes very long time to heal after surgery causing distress to patient and surgeon as well. Indications for drainage in mastitis/breast abscess xx Mastitis not resolving with antibiotics in 48 hours xx Persistent fever and progression of mastitis xx Brawny induration Do not wait for abscess to form (fluctuation to develop)

SRB's Manual of Surgery

B

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: ¾¾ 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 BD for 2 weeks) or stilbestrol.

• • • •

A

B

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: ¾¾ It commonly occurs in duct ectasia and periareolar infections. Common organisms are bacteroides, anaerobic streptococci, enterococci and Gram-negative organisms. It is commonly recurrent with tender swelling under the areola. ¾¾ It is common in diabetes mellitus and immunosuppression. ™™ Treatment: Antibiotics; Repeated aspirations; Drainage and later cone excision of the duct is done.

Retromammary Mastitis ™™ It is commonly due to tuberculosis of the intercostal lymph

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. ¾¾ Drainage under general anaesthesia, a counter incision may be needed. It is not advisable to wait till the formation of abscess. ¾¾

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 nonmobile. ™™ 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.

• • • • • •

absorption of secretions and desquamated cells causing obstruction are the probable other causes and features.

Features ™™ Greenish discharge or creamy/paste like from the nipple. ™™ Indurated 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. ™™ Common in multiple pregnancies, perimenopausal age,

ANTIBIOMA

hyperprolactin status.

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.

™™ May present as mastalgia. ™™ Axillary nodes may be palpable as nonspecific. ™™ Secondary bacterial infection (anaerobic) is common. ™™ Differential diagnosis: Carcinoma breast. ™™ Investigations:

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 carci-

noma breast). ™™ Investigations: FNAC; mammography; US breast. ™™ Treatment: Excision (Submammary incision). Later antibiotics are given. It should be sent for histology.

Discharge study, FNAC. Mammography. ™™ Treatment ¾¾ It is important to stop smoking. ¾¾ Cone excision of involved major ducts (Adair-Hadfield operation). ¾¾ Antibiotics. ¾¾ Melhem Novel modified breast ductal system excision. ¾¾ ¾¾

MONDOR’S DISEASE (Henri Mondor—Paris, 1939) ™™ Mondor’s disease is spontaneous thrombophlebitis of the

super­ficial veins of the breast and anterior chest wall.

DUCT ECTASIA ™™ It is dilatation of lactiferous ducts due to muscular relaxation

(myoepithelial relaxation) of duct wall with periductal mastitis.

™™ 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

Fig. 8.25: Duct ectasia—ductography. ™™ It is also called ‘plasma cell mastitis’ as periductal inflamma-

tion contains plasma cells. ™™ Commonly many ducts are involved.

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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 the lymphatic permeation of carcinoma breast. ™™ Restriction of arm movements, brassiere support and anti-inflammatory 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

CHAPTER 8    Breast

Note: Mastitis of infancy (Witch’s milk) 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.

™™ Hormonally induced myoepithelial relaxation with poor ductal

518

Contd...

TUBERCULOSIS OF THE BREAST

SRB's Manual of Surgery

™™ 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 sinus are the features. ™™ Presents as a swelling in the breast with cold abscess, sinuses and a typical bluish appearance of surrounding skin with matted lymph nodes in the ipsilateral axilla. ™™ Differential diagnosis: Carcinoma breast. ™™ Investigations: FNAC; Frozen section biopsy is useful to differentiate from carcinoma; Excision biopsy. ™™ Treatment: Antituberculous drugs—INH, rifampicin, ethambutol, pyrazinamide; Drainage of cold abscess. Note: Mastectomy is not done.

•

xx xx xx xx

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; negative 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 recurrent/refractory 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. GRANULOMATOUS MASTITIS (Intralobular granulomatous mastitis/ILGM) 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. Indurated discharging sinuses with tenderness; positive lymph nodes in axilla—often seen.

B xx

xx

xx xx xx xx

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.

™™ 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.

B

Other cystic swellings of the breast

xx

Bloodgood cyst

xx

Breast abscess

xx

Haematoma in breast

xx

Hydatid cyst

xx

Galactocele

xx

Lymph cyst

xx

Serocystic disease of Brodie

xx

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 pituitary 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.

A

Management ™™ Estimation of serum prolactin, T3, T4, TSH, CT/MRI head. ™™ Bromocriptine therapy. ™™ Treatment of cause. Causative drug should be stopped and

different drugs should be used for the needed condition. Note:

B

Figs. 8.28A and B: Ultrasound look of breast cysts—simple and complex type. 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.

• • • • •

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 child 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

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CHAPTER 8    Breast

Treatment

•

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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.

¾¾

B xx xx xx xx xx xx xx xx xx xx xx xx

Fig. 8.29: Gynaecomastia—right side breast (diffuse type). Compare with opposite side to note the difference in size.

Inhibition of testosterone synthesis—cimetidine, phenytoin, spironolactone. Causes

Idiopathic—25% Teratoma testis—3% Ectopic hormonal production in bronchial carci­noma Anorchism, 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%)

B

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 xx Senescent in old is due to fall circulating testosterone causing relative hyperestrinism xx Prepubertal—may also be seen in girl child It can be: xx Puffy nipples xx Pure glandular xx Adolescent hereditary xx Adult—most common xx Androgenic Simon’s classification of gynaecomastia: Group 1—Minor visible type without skin redundancy Group 2 A—Moderate without skin redundancy Group 2 B—Moderate with minor skin redundancy Group 3—Gross pendulous breast xx

™™ It can be diffuse—involving all quadrants or small well local-

ised hard subareolar nodule.

xx

Features Fig. 8.30: Right-sided gynaecomastia (well-localised—puffy nipple).

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—aging, Klinefelter’s/Kallmann syndromes, eunuch, ACTH deficiency. ™™ Secondary testicular failure—cryptorchidism, orchitis, trauma, CRF, etc. ™™ Drugs (25%): ¾¾ 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. ™™ Differential diagnosis: ¾¾ Pseudogynaecomastia—adipose tissue deposition as a part of obesity; needs liposuction. ¾¾ Pectoral muscle hypertrophy. ¾¾ Lipoma, dermoid, haematoma. ™™ Presentations: ¾¾ Diffuse enlargement of breast occupying all quadrants. ¾¾ 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: ¾¾

¾¾ ¾¾

¾¾ ¾¾ ¾¾ ¾¾ ¾¾

A

B

Figs. 8.31A and B: Papilloma breast which is large. Usually it is small intraductal.

Note: 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 DCIS unlike 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 Biopsy and proceed is the preferred method.

T

Van Nuy‘s prognostic index for DCIS Score (VNPI score)

VNPI Score

1

2

3

Size in mm

≤15 mm

16–40 mm

>40 mm

Clearance margin in mm

≥10 mm

1–9 mm

50 mm in greatest dimension T4 – Any size with direct extension to the chest wall or skin or both. T4a – Tumour of any size extending into the chest wall, not including only 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.

Regional Lymph Nodes (N) Nx – Regional nodes cannot be assessed N0 – No regional nodes involved N1 – Metastases to mobile ipsilateral level 1 and 2 axillary nodes N2 – N2a – 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 N3 — N3a – Metastases to ipsilateral infraclavicular lymph nodes (level III axillary) 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. Note: 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.

•

•

Distant Metastases (M) M0—No clinical or radiological evidence of distant metastases cM0(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 for a recurrent tumor in an individual that had some period of time free from the disease.

• • • • •

Stage Groups Stage 0: Tis N0 M0 Stage IA: T1/T1 mi N0 M0 Stage IB: T0 N1 mi M0; T1/T1 mi N1mi M0 Stage IIA: T0 N1 M0; 1 N1 M0; T2 N0 M0 Stage IIB: T2 N1 M0; T3 N0 M0 Stage IIIA: T0 N2 M0; T1 N2 M0; T2 N2 M0; T3 N1 M0; T3 N2 M0 Stage IIIB: T4 N0 M0; T4 N1 M0; T4 N2 M0 Stage IIIC: Any T N3 M0 Stage IV: Any T any N M1

• • • • • •

•

B xx

xx

xx xx

xx xx

xx xx

Molecular subtypes of carcinoma breast

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. Laptinib 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–/AR+: recently identified androgen responsive subtype which may respond to antihormonal treatment with bicalutamide. ERBB-2/Her-2+: has amplified Her-2/Neu. 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.

B

533

Contd... Oncotype Dx: 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 DCIS will benefit from radiation after surgery. Oncotype DX 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 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 pectoralis 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 xx xx xx xx xx

Differential diagnosis for carcinoma breast

Fibroadenosis Traumatic fat necrosis Tuberculosis of breast Bloodgood cyst Filariasis breast

xx xx xx xx xx

Mastitis Antibioma Galactocele Mondor’s disease Cystosarcoma phyllodes

Investigations in Carcinoma Breast ™™ Mammography.

Genomic profiles and scores–DNA microarray gene analysis (genomic tests)

Genomic tests analyze a sample of a cancer tumor to see how active 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 10 years after diagnosis. Endopredict is a gene expression numerical scores from 0 to 15. Up to 5 is low risk; >5 is high risk. MammaPrint: 70 genes are assessed to predict breast cancer recurrence. 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.

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SRB's Manual of Surgery

xx xx

Findings

Size and location of mass lesion Microcalcifications signify malignancy

xx xx

Soft tissue shadow is irregular Spiculations

™™ 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: ¾¾

B xx

xx

xx

xx xx xx xx xx xx

To find out whether the lesion is solid or cystic. Ultrasound of breast

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 (lateral/horizontal) dimensions more than 1 are 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 H2O 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.

™™ It is difficult to differentiate between in situ and invasive breast

cancer by FNAC.

Advantages ¾¾

Note: FNNAC is Fine Needle Non-Aspirating Cytology.

Fig. 8.61: FNAC of breast lump.

T

Fig. 8.62: Trucut biopsy from carcinoma breast. It is the ideal investigation for confirmation.

Reliability of FNAC and mammography FNAC

Mammography

Sensitivity (true positivity)

90–98%

90%

Specificity (without false-positive)

98–100%

90%

False-negative

2–10%

10%

False-positive

Near 1–5% 10%

B

FNAC scoring

C0 : No epithelial cells C3 : Atypical cells C1 : Scanty epithelial cells, benign C4 : Suspicious cells C2 : Benign cells C5 : Malignant cells ™™ Corecut/Trucut biopsy: ¾¾

¾¾ ¾¾

¾¾ ¾¾

It is done under local anaesthe­sia. It gives clear histological evidence and also confirms DCIS (FNAC cannot confirm DCIS). This allows proper neoadjuvant/primary chemotherapy, receptor status of the tumour. Wide bore needle biopsy with vacuum is also used. 14–18 gauge spring loaded needle is used. Multiple punctures are needed. US guided biopsy is also done. Large core biopsy is done using 6–14 gauge needle; single 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 guidance. Multiple core biopsies are

B

Image guided biopsies

It is done when lump is not clearly palpable. xx US guided core needle biopsy. xx Stereotactic mammographic core needle biopsy. xx Mammography guided wire localisation using needle sheath over the tumour and through an incision under local anaesthesia hook is reached and biopsy is done. It is used if core needle biopsy fails in localising nonpalpable lesion. xx 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: ¾¾ 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: ¾¾ Done only when there is ulceration and fungation. 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: ¾¾ 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 where you’re going, you may miss it when you get there.

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CHAPTER 8    Breast

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).

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.

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Tissue for receptor study is sent at low tempe­rature in ice flasks. It is assessed by quantitative analysis (Frozen –70°). ¾¾ If value is more than 10 units (f/mols) per n gram of tissue it is called as ER +ve status. ER positivity is common in postmenopausal women (60%) compared to premenopausal women (30%). If value is less than 10 units per gram of tissue it is called as ER –ve status. Currently IHC is used to measure ER/PR expression. Staining of 1% or more is considered as positive. In ER +ve status –– Prognosis is good. –– Hormone therapy including tamoxifen is more beneficial. –– Response to treatment is better. In ER –ve status –– Prognosis is poor. –– Hormone therapy is not very beneficial (but used) as compared to ER +ve patients. –– Response to treatment is not good. ™™ Progesterone receptor (PR status) study or Her-2/Neu receptor status or cErb B2 (growth factor receptor study) are other studies done at present to plan the therapy and assess the prognosis. ™™ Her-2/Neu receptor (Human epidermal growth factor receptor-2 Neu oncogene [Neuropilin 2]) is a tyrosine kinase receptor and is associated with ER negative, high grade, tumours. It carries poor prognosis. Over expression of Her-2/ Neu shows good response to adriamycin. Her-2/neu is also called as c Erb B2 (cell surface Erythroblastic oncogene B2). ™™ Study of discharge from the nipple. ¾¾ Nipple discharge is usually unilateral in carcinoma breast. Ductal lavage may be useful in some patients. Microcatheter of 1 cm length is introduced gently into the ductal opening. 10 mL saline is infused through the catheter. Fluid is withdrawn into the syringe and cytological analysis is done. ™™ Sentinel lymph node biopsy (SLNB): ¾¾ The first axillary node draining the breast (by direct drainage) is designated as the sentinel lymph node (SLN). SLN is first node involved by tumour cells and presence or absence of its histological involvement, when assessed will give a predictive idea about the further spread of tumour to other nodes. The incidence of involvement of other nodes without SLN is less than 3% and so if SLNB is negative nodal dissection can be avoided but regular follow-up is needed. SLNB is done in all cases of early breast cancers, T1 and T2 without clinically palpable node. ¾¾ It is not done in clinically palpable axillary node as there is already distortion of lymphatic flow due to tumour. It is also not done in multifocal and multicentric tumours, as there is involvement of many lymphatic trunks from different places of breast, and chances of false-negative is high. ¾¾ Sentinel node is localised by preoperative (within 12 hours prior) or perioperative injection of patent blue (Isosulfan vital blue dye 2.5–7.5 mL) or 99m TC ¾¾

¾¾

¾¾

¾¾ ¾¾

¾¾

¾¾

radioisotope labelled albumin (one mCi on previous day)/sulphur colloid (6 hours before) near the tumour (peritumour area) or into subdermal plexus around the nipple. Marker will pass through the sentinel node which can be visually detected as blue staining or with a hand held gamma camera; and is biopsied with a small incision made directly over it. Frozen section biopsy or touch imprint cytology is done for presence of malignant cells. If there is no involvement of sentinel node by tumour then further axillary dissection is not required as skip lesions (skipping sentinel node) occur only in less than 3% cases. Detection rate of sentinel node for blue dye and radioisotope is 90% and 98%, respectively. Subdermal/subareolar injection of radioisotope has got better sentinel node localisation than peritumour injection. But better imaging is obtained by peritumour injection and so peritumour injection is usually practiced. Radioisotope tracer injection done in the early morning of the day of surgery into peritumour area and perioperative injection of patent blue dye in subareolar region—as a combined method is often used in many centres. After injection of patent blue, breast is massaged continuously to enhance the uptake. Incision is made after 5–7 minutes between pectoralis major and latissimus dorsi to identify blue stained lymphatics which are traced to 2–3 blue lymph nodes. Hand-held radioprobe is used to identify the sentinel node which is later excised. Often 2–3 nodes are removed. Paraffin section histology is better than frozen section to identify positive sentinel lymph node. If report comes negative immunohistochemistry test is done to confirm that lymph node is negative for tumour. Sentinel lymph node biopsy should be done before wide local excision of the primary tumour. Wide local excision of the primary tumour is done after SLNB in the same sitting. SLNB is less invasive than axillary dissection. It is ideal in early invasive carcinoma. Positive SLNB is again classified as macrometastasis (>2 mm) or micrometastasis (1 cm, +ve axillary nodes, high nuclear grade, vascular/lymphatic invasion, ER/Pr –ve with over expression Her-2/Neu, then adjuvant chemotherapy

Stage III

Neoadjuvant chemotherapy + MRM + RT + CT + Hormone therapy

Stage IV

Hormone therapy (Trastuzumab/Lapatinib) with chemotherapy using taxanes or capacetabine + palliative mastectomy (toilet mastectomy if needed when fungation is present)

Breast cancer Features group

Prognosis—5-year survival

Stage

5 years survival

>90%

90%

Very low-risk primary

DCIS; tubular

I II

70% 40%

Node negative; favourable microscopy

70–90%

III

Low-risk primary

IV

20%

High-risk primary

Node positive; unfavourable 2.5 cm has poor prognosis More than 4 nodes/level III (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. ErbB2— Her-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 (30,000/mm3.

Treatment ™™ Diagnostic tapping, tube peritoneal drainage and laparoscopic

drainage and wash are useful methods.

™™ Laparotomy and peritoneal toilet. ™™ Broad spectrum antibiotics including combination of amino-

glycosides, cephalosporins and metro­nidazole.

™™ Local instillation of antibiotics, into the peritoneal cavity to

achieve quick and effective results. Mortality is high.

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 the commonest causes of secondary peritonitis.

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 intra-

abdominal infection after an adequate treatment for primary or secondary peritonitis—usually after 48 hours.

™™ It is common in immunosuppressed individual with inef-

™™ 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 termi-

nation of pregnancy.

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, β-haemolytic streptococci, mycobacterium are few other bacteria which can cause peritonitis. 1. Primary Monomicrobial, extraperitoneal source, blood spread 2. Secondary Most common, polymicrobial, intra-peritoneal source 3. Tertiary Due to superadded infection peritonitis 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 during abscess formation is B. fragilis. Mortality for diffuse peritonitis is 10%.

Fig. 9.1: On table finding in a case of peritonitis, where the peritoneal cavity is filled with pus.

559

Fig. 9.3: Jejunal perforation due to trauma. Trauma is the common cause for jejunal perforation. It is commonly blunt abdominal injury due to shearing force (traction injury) causing jejunal tear near duodenojejunal junction.

Attack the problem, not the person.

CHAPTER 9    Peritoneum

fective peritoneal host defenses against microbes. Infection due to E. 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.

Mode of Infection

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SRB's Manual of Surgery

™™ Bacterial load (2 × 108 CFU/mL) and virulence overwhelm the

A

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 peritonitis 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%.

Clinical Features B

Figs. 9.4A and B: Ileal perforation with severe peritonitis. Note the closure done horizontally. It could be typhoid perforation. Factors affecting the spread of the infection in peritonitis 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) Immunosuppression—HIV, steroids Anatomical nature of the peritoneal cavity Age, associated diseases like malignancy, mal­nutrition, anaemia

B xx xx xx xx xx xx xx

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 gastric 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.

Investigations ™™ Chest X-ray in standing position with abdomen (erect) will

show ground glass appearance of the abdomen with gas

fluid will be low in bacterial peritonitis but will show raised protein and LDH (greater than serum LDH). ™™ Diagnostic laparoscopy. It can also be used as therapeutic for 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.

Differential Diagnosis ™™ Pancreatitis: In pancreatitis back pain is common. But it is

Fig. 9.6: Plain X-ray abdomen showing gas under diaphragm. 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. DPL with more than 500 WBCs/mL suggests peritonitis; fluid should be assessed for pH, sugar, protein, LDH, 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 (3 cm in depth.

IV – Laceration

Parenchymal disruption 25–75% hepatic lobe; 1–3 Couinaud segments.

V – Laceration – Vascular

Parenchymal disruption more than 75% or more than 3 Couinaud segments. Major hepatic veins or retrohepatic venacaval injuries.

VI – Vascular

Hepatic avulsion.

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. ™™ Hb%, 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.

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 VII—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 (I-III) 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.

587

Note: Mervis et al. proposed to add thickness of subcapsular hematoma in grading 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 I-III 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 cholecystectomy. 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

Damage control surgery initially

Phase I: Laparotomy control of bleeding by various methods, closure with pack. Phase II: Resuscitation, managing metabolic insult (hypothermia with temperature 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.

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.

B xx xx

Indications for surgical resection

Rupture of adenomas—50% If there is possibility of turning into malignancy (large adenomas)

Note: Kupffer 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.

599

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 bile ducts that fail to involute with cystic dilatation. It mimics metastatic liver disease. MRI is essential to diagnose.

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.

HEPATOCELLULAR CARCINOMA (HEPATOMA) ™™ Hepatocellular carcinoma (HCC) is a primary 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.

CHAPTER 11    Liver

™™ It is commonly solitary; but 20% of cases can be multiple;

™™ It also should be differentiated from HCC, hepatic adenoma,

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Aetiology

SRB's Manual of Surgery

™™ Aflatoxin B1, a product of fungus aspergillus. It is powerful

carcinogen. ™™ Hepatitis B and hepatitis C virus 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, α1 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.

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.

B

Other macroscopic classifications of HCC

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) xx Multifocal—synchronous xx Indeterminate —different features in different parts of the liver xx Diffuse Okuda classification—Okuda staging is different xx Multifocal; Indeterminate; Spreading; Expanding NCCN classification xx Nodular; Massive; Diffuse xx

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 haemobilia and obstructive jaundice. Lymphatic spread occurs to hilar nodes, celiac nodes, pericardial 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. Variants of HCC are—fibrolamellar (good prognosis); mixed hepatocellular cholangiocellular (poor); clear cell variant (better); giant cell variant; (bad) sarcomatoid variant (carcinosarcoma).

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

B12 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.

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 system– currently used

Very early stage 0

Early stage A

Intermediate Advanced stage B stage C

Terminal stage D

Child Pugh A

Child Pugh A-B

Child Pugh A-B

Child Pugh C

Child Pugh A-B

Single nodule 1-3 nodule 2 cm without vascular invasion. T2 – Solitary tumour >2 cm with vascular invasion/multiple tumours equal or less than 5 cm. T3 – Multiple tumours, more than 5 cm. T4 – Single or multiple tumours invading major branches of portal or hepatic veins or tumour with direct invasion of adjacent organs other than gallbladder or to visceral peritoneum. Nx – Regional lymph nodes not involved. N0 – No regional nodes are involved. N1 – Regional nodes are involved. cM0 – No distant spread; cM1 – Distant spread is present; pM1 – microscopically confirmed distant spread. Staging: IA: T1aN0M0. IB: T1bN0M0. II: T2N0M0. IIIA: T3N0M0. IIIB: T4N0M0. IVA: AnyTN1M0. IVB: Any T, any N M1.

B

Presentations

Asymptomatic —incidentally identified Symptomatic —abdominal pain (capsule stretching, tumour necrosis), weight and appetite loss, jaundice, mass abdomen Symptoms due to complications —bleeding HCC can cause life threatening spontaneous haemoperitoneum after an attempt of biopsy; jaundice (in 10% of HCC) due to obstruction by tumour of main intrahepatic ducts or CHD at porta hepatis or by tumour infiltration of biliary system or by haemobilia or due to underlying cirrhosis; oesophageal varices causing haematemesis with portal hypertension Symptomatic due to metastasis —from pulmonary metastases, bone metastases (10%) Paraneoplastic syndrome (1%)

Spread of Tumour ™™ Lymphatic spread: It can spread to other part of liver through

lymphatics within the liver, to the lymph nodes in the porta hepatis and other abdominal lymph nodes later. Often spread occurs directly to cisterna chyli. ™™ Blood spread: To lungs, bones and adrenals often can occur. ™™ Direct infiltration: To diaphragm and neighbouring structures.

Clinical Features ™™ Painless mass in right hypochondriac region with loss of

appetite and weight. Liver is hard, smooth/irregular and often massively enlarged. Cirrhotic liver may be nodular. ™™ Acute presentation is not uncommon, when the tumour undergoes necrosis and haemorrhage. Both types of presentations mimic amoebic liver abscess (Haemoperitoneum is also known). ™™ Jaundice when present is commonly due to hepatic dysfunction, but occasionally due to compression of bile ducts. ™™ Ascites (40%) often it is massive, splenomegaly and features of portal hypertension may be present. ™™ Hepatic thrill and bruit—25%. ™™ Fever (10–20%) may be present due to tumour necrosis. ™™ Dull aching pain in right upper quadrant is common. ™™ Features of chronic liver disease—jaundice, dilated veins, palmar erythema, gynaecomastia, testicular atrophy, etc. ™™ Liver failure sets in once tumour replaces the functioning liver parenchyma or portal vein gets occluded by tumour thrombus. ™™ Gastrointestinal bleeding may be the presentation in 10% of cases due to portal hypertension or portal vein invasion by tumour.

A

B

Figs. 11.22A and B: CT pictures of hepatoma. In right lobe and left lobe.

B xx xx xx xx

Differential diagnoses for HCC

Secondaries in liver; Cholangiocarcinoma Liver abscess—amoebic Polycystic liver disease, hydatid cyst Benign tumours of liver; Hepatosteatoses

Investigations ™™ Ultrasound abdomen—very useful method. It shows

hyperechoic mass; mosaic pattern with thin halo and lateral shadows. Extent, tumour thrombi extension can be made out. ™™ CT scan abdomen (CECT) more reliable and ideal (hypodense, mosaic, vascular lesion with irregular margin). ¾¾ Reveals the size, location and extent, vascularity, portal vein invasion, nodal status, portal vein thrombosis. ¾¾ Helps to assess operability.

Adversity is better teacher than prosperity. —James Thurber If aim is ended most be ended in this life. —Rabindranath Tagore

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Less than 400 More than 400 Portal vein No thrombosis Yes Total scores 0–6 Early disease with potential long-term survival 0–3 Advanced stage 4–6

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™™ Tumour markers—α fetoprotein (AFP). AFP will be raised

more than 100 IU; as high as 1000 IU is possible in HCC. PIVKA II is des γ carboxy prothrombin protein induced by vitamin K abnormality/antagonists 2. It is increased in 80% of HCC patients. AFP is usually more than 400 IU in 70% of HCC patients. AFP –L3 (serum lens culinaris agglutinin reactive fraction of AFP, DCP (des γ carboxy prothrombin) and glypican-3 are other tumour markers used. AFP begins to rise once vascular invasion by the tumour starts, hence it becomes insensitive in detecting early tumour. ™™ Celiac angiography/CT angiography—it shows the vascularity of the tumour, tumour blush, arterial pattern of the liver and tumour; venous phase can show portal vein invasion or thrombosis and invasion/spread to IVC. Angiogram is essential while planning for hepatic resection. ™™ Liver function tests like serum bilirubin, albumin, enzymes (alkaline phosphatase, transaminase, 5’ nucleotidase) including prothrombin time. Note: Preoperative liver biopsy is not recommended now. Biopsy is relatively contraindicated in any candidate for resection, as there is a significant risk of haemorrhage and peritoneal implantation of tumour cells. In case of potentially resectable tumour one should proceed to surgical exploration without tumour biopsy. Biopsy is done only in inoperable advanced cases wherein palliative therapies are undertaken to establish the diagnosis as in a typical images with normal AFP. Liver biopsy is done after controlling prothrombin time if it is elevated (by Inj vitamin K IM or IV 10 mg for 5 days; FFP transfusion). US/CT guided core liver biopsy is useful in non-surgical therapies and mandatory before clinical trials. It is better than FNAC as it reveals tissue architecture. Problems with the core liver biopsy are spillage of tumour and bleeding (due to hypervascularity, associated thrombocytopenia and reduced liver dependent clotting factors like prothrombin). Such problems may be minimal in FNAC. Patients with high suspicious of HCC by clinical and imaging methods and who are appropriate for surgery may be taken up for surgery without a preoperative biopsy. In case of portal vein infiltration by tumour a portal vein biopsy may be done to exclude the patient for surgery or transplantation. PIVKA II: It is protein induced vitamin K absence II - is also known as des γ carboxy prothrombin is an abnormal prothrombin molecule produced due to acquired defect in the carboxylation of the prothrombin precursor in malignant cells. It is more sensitive than AFP in differentiating HCC from cirrhosis and chronic hepatitis. LI-RADS (Liver imaging reporting and data system) are used currently on CT images – LI RADS I is benign to LI RADS V definitely malignant. CEUS: Contrast enhanced ultrasound is also used now. A lesion >1 cm in size in ultrasound and AFP >20 ng/mL should trigger to evaluation for HCC. Tumour markers like GPC3, HSP70 and GS are helpful to identify HCC from high grade dysplasia.

™™ MRI—T2 weighted studies are useful for small HCC. MR

angiography is also done to see tumour thrombus in portal vein, hepatic vein and IVC. ™™ 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. ™™ FDG-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.

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.

•

•

A

B

•

• • • •

™™ 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.

C

D

Figs. 11.23A to D: Hepatocellular carcinoma—CT and resected specimen. ™™ In cirrhotic patients with HCC, total hepatectomy with ortho-

topic 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 xx xx xx xx

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

Note: 30% of liver tissue or at least two segments should be retained in liver 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 anatomical location of the tumour, hepatic reserve, liver function (normal bilirubin, HVPG 1,00,000/, sufficient functional liver remnant (FLR). MELD (Model for End Stage Liver Disease) score is used for resectability using creatinine, bilirubin, PT- INR. Milan’s criteria are tumour less than 5 cm or 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 Adriamycin/ cisplatin/mitomycin through gastroduodenal artery. It is often given along with lipiodol to make drugs 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 exclusively gets its blood supply 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 I131 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

Neoadjuvant therapy in HCC

B

Treatment strategies for HCC

Large primary tumour—downstaging/downsizing may make resection possible. xx Transarterial chemoembolisation (TACE) xx Chemotherapy and radiotherapy (SBRT—stereotactic body RT) xx Hepatic artery infusion of chemotherapy (HAI) xx Radioimmunotherapy xx Fractioned radiotherapy 90 xx Transarterial Y microspheres (TARE—transarterial radioembolisation)

xx xx xx xx xx xx xx xx xx xx xx

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/I131 lipiodol Hepatic artery ligation Adjuvant systemic chemotherapy Immunotherapy/hormone therapy/growth factors

The best bridge between despair and hope is a good night’s sleep.—E Joseph Cossman

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Indications for transplantation in HCC

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Hepatoma/hepatocellular carcinoma/HCC

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 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 liver disease LFT, CT scan, raised AFP, liver biopsy are the investigations 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/chemoembolisation/percutaneous ethanol or acetic acid injection/ radiofrequency ablation/chemotherapy using adriamycin, carboplatin, gemcitabine—are palliative procedures

™™ PET scan is done to identify occult extrahepatic metastases.

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.

Fig. 11.24: Secondaries in liver—solitary type from colonic primary.

SECONDARIES IN THE LIVER ™™ It is the commonest malignant tumour in liver. The incidence

of primary: secondary : : 1 : 20.

B

Secondaries are common malignant tumours In bone     In liver     In brain

Causes Abdominal: Carcinoma in stomach, colon, pancreas, small bowel, kidney, abdominal oesophagus, rectum, and carcinoids. Extra-abdominal: ™™ Melanoma. ™™ Carcinoma breast, lung, thoracic oesophagus, bladder, 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).

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 second-

aries 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.

Other classifications ™™ Precocious: Secondaries are presented/identified before

primary is suspected—carcinoid, colorectal cancer.

™™ Synchronous: both primary and secondaries are identified at

the same time—carcinoma stomach.

™™ Differential diagnosis: Multicentric hepatoma; Macronodular

cirrhosis; Polycystic liver disease, hydatid cyst of liver.

™™ Metachronous: secondaries develop much later may be long

Investigations ™™ Primary is identified by—gastroscopy, colono­scopy, contrast

X-rays, CT scan.

Route of Spread a. Direct spread: Stomach, colon, gallbladder, bile ducts. b. Hepatic artery: Melanoma. c. Portal vein: Carcinoid tumours, other GIT malignan­cies. d. Lymphatic spread: Breast, lung.

Clinical Features ™™ They are multiple, hard, nodular, with nodule showing umbili-

cation due to central necrosis.

™™ Patient may have jaundice depending on the extent of liver

involvement.

A

B

™™ Loss of appetite and weight. ™™ Ascites. ™™ Pain in liver secondaries is due to stretching of liver capsule/

tumour necrosis. Often pain may be of primary tumour.

™™ Rectovesical secondaries—‘Blumer shelf’. ™™ Supraclavicular lymph node enlargement—Virchow’s node

(Troisier’s sign). ™™ Clinical features of primary tumour, e.g. stomach, colon— ¾¾ Stomach: Vomiting; visible gastric peristalsis; palpable stomach mass.

D

C

Figs. 11.26A to D: Secondaries in liver are multiple and often with umbilication. CT picture shows multiple secondaries with ascites. On table photo of multiple liver secondaries with umbilication and also laparoscopic view. ™™ Liver biopsy is done, only if primary is not identified by any

of these methods.

™™ Liver function tests. ™™ Ultrasound abdomen—multiple hypo- or hyperechoic lesions;

site of possible primary; nodal status; ascites.

™™ Tumour marker: CEA. ™™ MRI/CT scan whenever resection is indicated. ™™ Laparoscopy often with laparoscopic US is very useful to

detect small secondaries.

Fig. 11.25: Secondaries in liver. Note the multiple lesions (nodules) in both lobes of the liver. ¾¾ ¾¾ ¾¾ ¾¾

B xx xx xx

Colon: Constipation/diarrhoea; palpable mass; features of obstruction. Pancreas: Jaundice; steatorrhoea; itching; palpable gallbladder. History of earlier surgery: Like mastectomy/wide excision of the melanoma. Testis: Fullness; hard mass; loss of testicular sensation. Clinical features of secondaries in the liver

Poor general condition Palpable liver which is hard, multinodular with umbilication Clinical features of primary; Ascites ±; Jaundice ±

Treatment ™™ Chemotherapy is the treatment of choice. ™™ Surgery has a definitive role if primary is from the colon, kidney. ™™ If the primary growth is in the colon, then a palliative hemi-

colectomy is done. Along with that if a solitary secondary in the liver is present, it can be resected. If secondaries are in single lobe, hemihepatectomy is done.

Criteria for selection for liver resection in secondaries in liver, when primary is in colon are: xx Stage of the primary xx Number of secondaries xx Type of primary resection xx Segments involved xx Size of the largest liver xx Associated liver disease like secondary cirrhosis

A superior man is modest in his speech, but exceeds in his actions.

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time after the treatment for primary—melanoma, breast carcinoma.

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™™ Palliative procedures for other primaries like—for carcinoma

stomach—palliative partial gastrectomy/anterior gastrojejunostomy; for carcinoma pancreas—triple anastomosis/ ERCP stenting. ™™ Depending on the site of primary, drugs vary. ¾¾ For carcinoma stomach, mitomycin and 5FU are given. ¾¾ For carcinoma colon, 5-FU, oxaloplatinum/VEGF or EGFR antibodies used with folinic acid. ™™ Hepatic artery ligation or therapeutic embolization with clot/ gel foam can be tried to relieve pain. ™™ Intra-arterial chemotherapy is tried with little success. Methods other than resection available to ablate the tumour locally in secondaries in liver. xx Microwave therapy xx Laser hyperthermia xx Radiofrequency ablation xx Ultrasound or electrolyte therapy

Overall outcome is poor in liver secondaries. They die of malignant cachexia, infection, liver failure.

B xx xx xx

Causes of massive enlargement of liver

Hepatoma Secondaries in liver when primary is from melanoma, carcinoid tumour Syphilitic enlargement of liver (hepar lobatum)

LIVER CYSTS

™™ Aspiration confirms the diagnosis. Aspiration will not cure

the cyst as it recurs 100%. ™™ Treatment is—aspiration with sclerosant therapy where the

recurrence rate is 20%. Deroofing with partial excision of cyst wall, by laparoscopic or open method has recurrence rate of 10%. Complete excision has very less recurrence rate but surgical mortality of 2% is present.

Polycystic liver disease ™™ It is autosomal dominant condition associated with polycystic

kidney disease in 40% of cases. ™™ It arises from biliary epithelium but does not communicate

with biliary system. ™™ Usually they are asymptomatic. ™™ Hepatomegaly, pain abdomen, jaundice and portal hypertension are the presentations. ™™ Haemorrhage, biliary obstruction, Budd-Chiari syndrome, rarely malignant transformation—are the complications. ™™ Liver function is well preserved and so liver failure will not occur. ™™ US, CT, MRI are investigations done. ™™ Treatment: Percutaneous aspiration with sclerosant injection, deroofing of all cysts, resection, liver transplantation.

NEOPLASTIC LIVER CYSTS ™™ They are acquired cysts.

Congenital Liver Cysts

™™ They are common in females. ™™ They attain large size; multiloculated with papillary projec-

tions.

Simple liver cyst ™™ It is 3% common. It is common in males (2:1). ™™ Solitary simple cyst is congenital; developing from abnormal

intrahepatic bile duct in utero. ™™ 1/4th of simple cysts are symptomatic only at 4th or 5th decade. ™™ It is thin bluish walled cyst in one or other lobe containing clear fluid. Simple cyst will never communicate with biliary system. If communication is found, it should be cystadenoma.

™™ 10% of neoplastic cysts are malignant. ™™ Cystadenoma is benign containing mucinous or bilious fluid. ™™ Cystadenocarcinoma contains haemorrhagic fluid. ™™ AFP, CEA are normal; CA 19/9 is elevated.

Hepatobiliary (hepatic) cystadenoma ™™ Incidence is 5% of biliary intrahepatic cysts; 85% occur in

females. ™™ 50% are in right lobe; 30% are in left lobe; 15% bilateral. ™™ Cystadenoma without mesenchymal stroma is equal in both

A

B

Figs. 11.27A and B: Simple liver cysts—CT pictures in two different patients. ™™ 50% present with abdominal pain. Hepatomegaly is common.

Jaundice can be present. ™™ Differential diagnoses are—hydatid cyst, cystic neoplasms

and solitary secondary with tumour necrosis. ™™ US shows anechoic lesion with smooth margin. ™™ CT scan shows nonenhancing thin lesion. MRI shows hypoin-

tense T1 image and hyperintense T2 image.

sexes and occurs in 5th decade. It is not associated with cystadenocarcinoma. ™™ Cystadenoma with mesenchymal stroma is common in young females and is often associated with cystadeno­carcinoma. ™™ It is septated, multiloculated, whereas if cavity contains debris it suggests malignancy. It is surrounded by a smooth and thick fibrous capsule. It contains internal septations and intraluminal papillary projections and lined by cuboidal or columnar biliary epithelium. ™™ It originates from—congenital aberrant bile duct/embryonic foregut cells/peribiliary endocrine cells. ™™ Presentations —palpable, smooth, firm liver often large, with occasional pain, tenderness, jaundice and ascites. ™™ Differential diagnoses —simple liver cyst, hepatic adenoma, focal nodular hyperplasia, hydatid cyst, cystadenocarcinoma.

B xx xx xx

PORTAL HYPERTENSION A

B

Figs. 11.28A and B: Hepatic cystadenoma of right lobe of liver—CT picture and on table look (Courtesy: Dr Ashfaque Mohammed, DNB, KMC, Mangaluru). ™™ Complications are —rupture, bleeding, malignant transfor-

mation, obstructive jaundice, IVC obstruction and infection.

™™ MRI is diagnostic. US/CT scan, LFT, tumour markers (CA

19/9, CEA) is also useful. ™™ ERCP is done to identify communication with biliary system; usually to proximal left hepatic duct. ™™ Treatment—enucleation of entire cyst with surrounding rim of normal liver tissue. Hepatic resection is also an option. ™™ Note: Aspiration/deroofing, partial excision, sclerotherapy should not be done.

(Gilbert and Villaret first coined the term portal hypertension in 1906).

Definition It is sustained elevation of the portal venous pressure more than 10 mm of Hg. Normal portal venous pressure is 5–10 mmHg. It is also defined as elevation of the hepatic venous pressure gradient (HVPG) >5 mmHg. HVPG is the difference between the wedged hepatic venous pressure (WHVP) and free hepatic venous pressure (FHPV). When HVPG is more than 10 mmHg, porta systemic shunting develops; when it becomes more than 12 mmHg, risk for variceal bleeding increases; often it will be as high as 20 mmHg in bleeding oesophageal varices. Portal venous pressure more than 5 mm Hg greater than the inferior vena cava pressure is defined as portal hypertension.

Hepatic cystadenocarcinoma ™™ It presents as large palpable smooth liver with abdominal pain. ™™ It carries better prognosis than HCC or cholangiocarcinoma.

It can be: ¾¾ Cystadenocarcinoma arising from pre-existing cystadenoma with mesenchymal stroma, which occurs only in young females, carries good prognosis. ¾¾ Cystadenocarcinoma without mesenchymal stroma not associated with cystadenoma, which is common in males and is more malignant. ¾¾ Cystadenocarcinoma without mesenchymal stroma occurring in women. ™™ Resection is the treatment. It carries good prognosis.

TRAUMATIC LIVER CYST ™™ It is an acquired cyst of liver after liver injury in a blunt

abdominal trauma. Biliary duct which is injured will cause bile leak into the existing intrahepatic haematoma causing mixture of bile and blood. Cyst is pseudocyst in liver without an epithelial lining. ™™ It may resolve spontaneously or may cause abdominal pain, jaundice. It may present months or years later. ™™ CT scan, old history of liver trauma may be helpful. MRI is diagnostic. ™™ Treatment is conservative. Often deroofing or cyst excision with ligation of biliary ductule may be needed but technically demanding.

Fig. 11.29: Anatomy of the portal system.

Causes There is increased portal resistance and altered portal blood flow. (Increased resistance may be presinusoidal, sinusoidal, postsinusoidal). ™™ Prehepatic [normal wedged hepatic venous pressure (WHVP) and free hepatic venous pressure (FHVP) with normal hepatic venous pressure gradient (HVPG)]. ¾¾ Portal/Splenic vein thrombosis; Congestive splenomegaly (Banti’s syndrome; Arteriovenous fistula.

My life as a surgeon-scientist, combining humanity and science, has been fantastically rewarding. In our daily patients we witness human nature in the raw–fear, despair, courage, understanding, hope, resignation, heroism. If alert, we can detect new problems to solve, new paths to investigate. —Joseph E Murray

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™™ Hepatic (increased WHVP, normal FHVP, and increased

HVPG). ¾¾ Presinusoidal: Schistosomiasis; Congenital hepatic fibrosis; Primary biliary cirrhosis. ¾¾ Sinusoidal: Cirrhosis; Alcoholic hepatitis; Nodular regenerative hyperplasia; Polycystic liver disease; haemochromatosis, Wilson’s disease, non-alcoholic steatohepatitis (NASH) ¾¾ Postsinusoidal: Sinusoidal obstructive syndrome; BuddChiari syndrome; veno-occlusive disease. ™™ Posthepatic (increased WHVP and FHVP and normal HVPG) ¾¾ Inferior vena cava webs/thrombosis; Cardiac causes (restrictive cardiomyopathy, constrictive pericarditis, and congestive heart failure); Pulmonary hypertension. Note: Left sided portal hypertension (sinistral) can be caused by isolated splenic vein thrombosis, which is often caused by adjacent pancreatitis. A rise in portal pressure stimulates portasystemic circulation. Portal vein carries 75% of blood flow to liver with all nutrients to maintain its integrity and gives 50% oxygen supply to liver. 25% hepatic arterial blood flow gives remaining 50% of oxygen supply to liver. Portal hypertension causes compensatory portosystemic venous collateral formation, altered intrahepatic circulation and increased splanchnic blood flow. High pressure portal blood is diverted via coronary (left gastric vein), short gastric and oesophageal veins into azygos venous system. There is dysregulation of compensatory increased hepatic arterial flow response in relation to decreased portal vein flow. 30% of varices patients will bleed; 30% of them will die of bleed; 30% of patients with cirrhosis will develop portal hypertension; 30% of them have variceal bleed in 2 years; 70% of patients who had bleeding once, will rebleed later. Variceal bleed accounts for 7% of upper GI bleed. In western country, more than 90% cases cirrhosis is the cause. In India, extrahepatic portal vein obstruction (33%) and extrahepatic periportal fibrosis (33%) is becoming more common. As liver is normal in these two conditions, patients get better cure rate by shunt surgery. Small varix is 5 mm.

Sites of Portosystemic Collateralisation 1. Lower end of oesophagus, between left gastric and short gastric veins with azygos vein resulting in oesophageal varices—commonest.

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Causes of portal hypertension

Prehepatic

Hepatic (80%)

Posthepatic

Portal vein or splenic vein Portal pyaemia thrombosis

Budd-Chiari syndrome

Hypercoagulable status

Alcoholic cirrhosis

Periportal inflammation

Idiopathic portal hypertension Primary biliary cirrhosis Schistosomiasis

Constrictive pericarditis Congestive cardiac failure Veno-occlusive disease

Trauma

Extrinsic compression from pancreas, stomach Neonatal umbilicus sepsis Hepatitis Nodular regenerative hyperplasia Wilson’s disease Haemochromatosis Congenital hepatic fibrosis Note: Commonest prehepatic cause of portal hypertension is portal vein thrombosis.

Fig. 11.30: Portosystemic collaterals.

2. Umbilicus, between paraumbilical vein and anterior abdominal vein resulting in caput medusae. 3. Lower end of rectum, between superior haemor­rhoidal vein and inferior, middle haemorrhoidal vein resulting in piles. 4. Retroperitoneum (vein of Retzius). 5. Bare area of the liver.

Presentations xx

Triad of portal hypertension – Oesophageal varices. – Splenomegaly. – Ascites.

xx xx xx xx xx

Jaundice. Features of encephalopathy. Recurrent infection. Coagulopathy. Hepatorenal syndrome.

™™ General—weakness, tiredness, anorexia, weight loss,

abdominal pain, jaundice, oedema, pruritus, spontaneous bleeding and bruises, impotence, muscle cramps, palor, cyanosis, hypotension. ™™ Features of variceal bleeding—anaemia, haematemesis, melaena, shock. Bleeding may be from gastric/oesophageal varices or portal gastropathy. ™™ Features of encephalopathy—memory loss, altered behaviour and mental status, asterixis (flapping tremor/liver flap), unconsciousness, foetor hepaticus. ™™ Features of liver cell failure—gynaecomastia, palmar erythema, leuconychia, testicular atrophy, spider angioma, parotid enlargement, cyanosis, and tachypnoea. ™™ Features of porta systemic shunting—see above; venous hum. Venous hum which is heard louder on inspiration in epigastrium in portal HT is called as Kenawy’s sign.

™™ Ascites and generalised anasarca with oedema scrotum

A

™™ Blood – Hb%—anaemia is due to bleeding, hypersplenism,

nutritional, bone marrow suppression. Blood picture shows pancytopenia leucopenia, thrombocytopenia. Counts may be serially repeated during monitoring period. Blood grouping should be done as patient often needs packed cell or FFP transfusions. ™™ Liver function tests—usually altered with raised bilirubin. Prothrombin time (INR), activated partial thromboplastin time (APTT) and other coagulation profile should be done as coagulopathy is not uncommon.

B

Figs. 11.31A and B: (A) Ascites with umbilical hernia; (B) Caput medusae in liver disorder. Fig. 11.33: Laparoscopic view of cirrhosis with nodules on the surface.

A

Fig. 11.32: Patient with portal hypertension showing massive ascites with oedema of limb and scrotum—end-stage liver disease. ™™ Others—Features of spontaneous bacterial peritonitis (SBP);

haemorrhoids, paraumbilical hernia.

Note: Cruvielhier-Baumgarten syndrome is loud venous hum heard at umbilicus in portal hypertension due to shunting of blood through congenital patent umbilical vein to portal vein. Hepatopulmonary syndrome: It is liver disease, pulmonary vascular dilatation, intrapulmonary arteriovenous shunting and poor oxygenation. There will be platypnoea (dyspneoa in upright position) with orthodeoxia (decreased arterial oxygen tension from supine to standing). Portopulmonary hypertension (PAH >25 mmHg); hepatic hydrothorax; hepatic cardiomyopathy; hepatic osteodystrophy are other problems in portal hypertension.

• •

•

609

B

Figs. 11.34A and B: CT scan pictures showing ascites, splenomegaly.

“Reality continues to ruin my life.” —Bill Watterson

CHAPTER 11    Liver

and abdominal wall and limbs. Ascites is a sign of hepatic decompensation. ™™ Decreased urine output features of renal failure—hepatorenal syndrome. ™™ Splenomegaly causing hypersplenism; hypersplenism occurs in 30% of cases with features of leucopaenia (WBC 2000 mL. It causes marked abdominal distension. Fluid thrill is elicited. Note: Refractory ascites are those that do not respond to sodium restriction and high doses of diuretics or develop diuretic-induced side effects that preclude their use.

B

Classification of ascites

Transudate (Protein 1.1); hypoproteinaemia; anaemia; beri-beri; nephrotic syndrome; portal hypertension; polyserositis. Exudate (Protein >2.5 g/dL) (SAAG 1.5 mg/dL (3) The absence of other apparent cause. (4) Lack of improvement in renal function after volume expansion with intravenous albumin (1g/kg of body weight per day up to 100 g/day) for at least two days and withdrawal of diuretics (5) Absence of parenchymal kidney disease as indicated by proteinuria >500 mg/day, microhematuria (>50 red blood cells per high power field) or ultrasonographic evidence of obstructive uropathy or renal parenchymal disease. ™™ Types: ¾¾ Type 1 HRS: It is rapidly developing renal failure with serum creatinine >2.5 mg%, with 50% decrease in creatinine clearance in less than 2 weeks; shows high mortality. ¾¾ Type 2 HRS: It is moderate stable renal failure slowly progressive often have longer stable course but with diuretic resistant ascites. ™™ Patient develops oliguria, azotaemia and hyponatraemia. ™™ Causes: Common in liver diseases (severe cirrhosis) and obstructive jaundice due to—Bile salt sludging in the tubules; absorption of toxins; increased ADH release; hypoperfusion and renal ischaemia; may be precipitated by surgery, stress. ™™ Treatment: Mannitol infusion; Diuretics; The cause is treated; Dialysis; Liver transplantation.

HEPATIC Resection Anatomy and Types of Resections segmental arteries. Left hepatic artery divides into medial and lateral segmental arteries. ™™ Portal vein divides at porta hepatis as right and left branches and these branches enter the respective lobes of the liver. ™™ Hepatic veins originate as central vein of the liver which is formed by liver sinusoids. Middle hepatic vein is located in main lobar fissure. Left hepatic vein is in left segmental fissure—upper portion; right hepatic vein is in right segmental fissure. Inferior part of middle and anterior segments are drained by middle hepatic vein. In 60% of cases middle and left hepatic veins join to form single trunk to enter IVC. ™™ Main portal fissure divides liver into two lobes by Cantlie’s line that passes from anteroinferior part of gallbladder fossa to left of the IVC with 75° angle with horizontal plane. It divides into right/left paramedian sectors/segments. ™™ Liver is divided into 8 segments (Couinaud segments). Right lobe is formed by segments V, VI, VII, VIII from front to behind. Left lobe formed by segments II, III and IV. Segment I (Spigel lobe) is caudate lobe which has got independent blood supply. ™™ Resection along the main portal fissure (Cantlie’s line) and removal on right side is right hemihepatectomy and removal on left side is left hemihepatectomy. ™™ Resection on right of the ligamentum teres is called as right trisegmentectomy (older—right lobectomy); and left of the ligamentum teres is called as left lateral segmentectomysegment II and III (older left lobectomy). ™™ Removal of single segment is called as unisegmentectomy. ™™ Plurisegmentectomy is removal of two or more segments. Segments IV and V are removed in carcinoma of gallbladder. ™™ Removal of small portion of liver either within the segment or traversing segments is called as wedge resection of the liver.

T

Couinaud— 1957

Goldsmith and Woodburne—1957

V-VIII

Right Right hemihepatectomy hepatectomy

Right hepatic lobectomy

IV-VIII

Right trisectiontectomy

Extended right hepatic lobectomy

II-IV

Left Left hemihepatectomy hepatectomy

Left hepatic lobectomy

II, III

Left lateral sectionectomy

Left lateral segmentectomy

II, III, IV, V, VIII

Left Extended left Extended left trisegmentectomy hepatectomy lobectomy

Right lobectomy

Left lobectomy

Types of hepatectomy techniques ™™ Preliminary vascular section (Lortat-Jacob). ™™ Primary parenchymal transection (Ton That Tung in 1965). ™™ Selective clamping. ™™ Total vascular exclusion. ™™ Pedicular clamping (Pringles). ™™ Suprahilar clamping (Glissonian approach). ™™ Intrahepatic portal control.

Procedure

Nomenclature for liver resection

Segments Brisbane—2000

Fig. 11.53: Reverse J incision for hepatectomy; other incisions are—Mercedes Benz, bucket handle, thoracoabdominal.

Technical principles Inflow (portal vein, hepatic artery, bile duct) control; outflow (hepatic veins) control; parenchymal transection; preservation of

™™ Proper preoperative preparation is essential. LFT, prothrombin

time is done. Adequate blood, FFP, along with central line is kept ready. ™™ Subcostal or thoracoabdominal incision is needed. ™™ Proper mobilisation, identification and dissection of portal areas is essential; dissection of hepatic veins, usage of vascular clamp secured ligation of branches of portal vessels and major hepatic veins are essential steps. ™™ Liver is dissected by finger dissection, back of scalpel, Cavitron ultrasonic surgical aspirator (CUSA), laser ligation of biliary radicles and vascular branches individually using non-absorbable sutures are other methods. ™™ Control of bleeding is done by on table Pringle manoeuvre, gel foam, cautery, laser and vessel ligation. ™™ Proper antibiotic coverage is needed to prevent sepsis. ™™ Postoperative ICU care is needed.

Understanding is part of learning and needed before teaching.

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CHAPTER 11    Liver

™™ Right hepatic artery divides into anterior and posterior

adequate sized liver remnant with intact inflow, biliary drainage and venous outflow; mobilisation of left and right triangular ligament; ligation of branches of portal vein and hepatic artery and resection of hepatic duct branch of the side; ligation of hepatic vein at a later period; proper haemostasis and ligation of individual biliary ductules. Retrohepatic caval dissection includes—dissection of right adrenal vein, inferior hepatic veins and IVC ligament.

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Contd...

Indications

Contraindications

• Primary/Secondary malignancy from gallbladder or colorectum or carcinoids

• Acute hepatitis—viral/ alcoholic

• Benign tumours of liver

• Severe chronic hepatitis

• Parasitic/non-parasitic cysts— hydatid cysts/simple liver cysts

• Poor liver reserve

• Refractory abscess/Caroli disease/RPC

• Severe portal hypertension

• LDLT

• Severe coagulopathy

• Klatskin’s tumour

• Severe thrombocytopenia—platelet count less than 30,000/-

• Trauma to liver

How much to cut: • Remnant liver—≥30% of the original liver volume with complete venous drainage is safe After completion of resection Complications • Check for bile leak by injecting • Bleeding, infection, dye through cystic duct septicaemia • Check for absolute haemostasis • Subphrenic abscess formation • Cholangiogram to confirm • Bile leak, haemobilia contralateral biliary patency • Fix the left lobe • Liver failure • Pleural effusion, empyema

PORTAL BILIOPATHY ™™ In patients with portal hypertension, particularly with extra-

B

A

Figs. 11.54A and B: Liver resection. Suprahepatic outflow control and parenchymal marking is shown. (Courtesy: Dr Ravishankar, Mch, Gastroenterologist and liver transplantation surgeon, Bengaluru). Techniques of parenchyma dissection • Kelly clamp and bipolar forceps • Water jet dissection • Ultrasonic dissection • Ultrasound cutting

• Dissecting sealer

Intraoperative ultrasound is done to: • Identifying tumour • Recognising the anatomy • Unexpected contralateral tumour • Intrahepatic selective vascular control using a balloon catheter • Guided biopsy Contd...

A

B

hepatic portal vein obstruction, portal biliopathy producing biliary ductal and gallbladder wall abnormalities are common. Portal cavernoma formation, choledochal varices and ischemic injury of the bile duct have been implicated as causes of these morphological alterations. ™™ Gallbladder varices, bile duct varices are typical. Often it may cause torrential haemorrhage per se or on table if patient is undergoing choledocholithotomy. ™™ While a majority of the patients are asymptomatic, some present with a raised alkaline phosphatase level, abdominal pain, fever and cholangitis. ™™ Choledocholithiasis may develop as a complication and manifest as obstructive jaundice with or without cholangitis. ™™ Endoscopic sphincterotomy and stone extraction can effectively treat cholangitis when jaundice is associated with common bile duct stones. ™™ Definitive decompressive shunt surgery is sometimes required when biliary obstruction is recurrent and progressive. ™™ Portal biliopathy is a specific entity which may cause severe technical difficulties especially bleeding during ERCP sphincterotomy or open biliary surgeries.

C

Figs. 11.55A to C: Liver resection of left lobe tumour. CT picture, incision scar and specimen are also shown.

12

chapter

Gallbladder C hapter Outline ·· Surgical Anatomy ·· Oral Cholecystogram ·· Intravenous Cholangiogram ·· Endoscopic Retrograde Cholangiopancreatography ·· Percutaneous Transhepatic Cholangiography ·· Magnetic Resonance Cholangiopancreato­ graphy ·· Radioisotope Scan Study ·· Peroperative Cholangiogram ·· Postoperative T-tube Cholangiogram ·· Congenital Anomalies of Gallbladder ·· Choledochal Cysts ·· Caroli’s Disease ·· Biliary Atresia ·· Gallstones ·· Acute Cholecystitis ·· Acute Acalculous Cholecystitis ·· Mirizzi Syndrome ·· Empyema Gallbladder ·· Mucocele of the Gallbladder ·· Chronic Cholecystitis

·· ·· ·· ·· ·· ·· ·· ·· ·· ·· ·· ·· ·· ·· ·· ·· ·· ·· ·· ·· ·· ··

·· ·· ··

SURGICAL ANATOMY Murphy's Sign Gallstone Ileus Cholecystoses Dissolution Therapy for Gallstones Choledocholithiasis Sump Syndrome Courvoisier’s Law Surgical Jaundice CBD Strictures Sclerosing Cholangitis Gallbladder Polyp Benign Biliary Papilloma Carcinoma Gallbladder Cholangiocarcinoma Klatskin Tumour Biliary Fistulas Hemobilia White Bile Cholecystectomy Open Approach Cholecystectomy Laparoscopic Cholecystectomy Single Incision Laparoscopic Surgery in Cholecystectomy Bile Duct Injuries Post-cholecystectomy Syndrome Biliary Dyskinesia

Gallbladder It is a pear-shaped (size is 5–12 cm) reservoir, located in a fossa on the inferior surface of the liver. It is under segment IVB and V of the liver. Cystic plate, a fibroareolar condensed tissue is more evident under the body of the gallbladder. Ducts of Luschka may drain directly from the liver to the gallbladder through cystic plate across the gallbladder bed. Submucosa and muscularis mucosa are absent in gallbladder. Gallbladder is supplied by cystic artery, which is a branch of right hepatic artery. Venous drainage is through direct veins entering into the segment IV and V through gallbladder bed and also through cystic veins into portal vein.

Parts ™™ Fundus, body, infundibulum and neck. Hartmann‘s pouch is

pathological one located in the infundibular region created by gallstones. ™™ Gallbladder drains through cystic duct into common hepatic duct to form common bile duct. Cystic duct is 3 cm in length having 1–3 mm diameter lumen. Duct is covered by sphincter of Lutkens; lumen contains spiral valves of Heister. ™™ It is supplied by cystic artery, a branch of right hepatic artery. ™™ Calot’s triangle (Cystohepatic/Cystobiliary—JR Calot, France, 1891) is formed by common hepatic duct to the left, cystic duct below, and inferior surface of liver/cystic artery above. Cystic artery originating from right hepatic artery passes behind the common hepatic duct, enters the Calot’s triangle to reach the gallbladder. It contains lymph node of ‘Lund’ (Fred Bates Lund)—Mascagni's node. ™™ Often cystic artery, hepatic artery, cystic duct have anomalous positions and anomalous origins.

Your mind will give back exactly, what you put into it.

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A

Fig. 12.1: Anatomy of gallbladder.

B

Fig. 12.2: Cystic duct and cystic artery in Calot’s triangle.

Figs. 12.3A and B: Calot’s triangle in (A): Open cholecystectomy method. (B) Laparoscopic method. Note the Rouvier sulcus in laparoscopic view which is an important landmark during dissection.

Extrahepatic Biliary Tree ™™ The left hepatic duct is formed by the ducts draining II, III,

IV segments of the liver. The right hepatic duct is formed by the ducts draining V, VI ,VII, VIII segments of the liver. Both join to form the common hepatic duct, which joins with the cystic duct to form the common bile duct. ™™ Common bile duct is normally 10–12 cm in length and 6–8 mm in diameter. It joins the major pancreatic duct in the wall of the 2nd part of duodenum to form the ampulla of Vater. Intraduodenal part of common bile duct (CBD) is surrounded by smooth muscle fibres called as sphincter of Oddi. Blood supply: From gastroduodenal, retroduodenal, posterosuperior pancreaticoduodenal arteries.

Bile ™™ Daily up to 1000 ml of bile is secreted from the liver which

contains water (98%), bile salts, bile pigments, fatty acids, lecithin, cholesterol, and electrolytes (sodium, potassium, chloride, bicarbonate, calcium, magne­sium) with a pH more than 7.0. ™™ Main function of gallbladder is to concentrate and store the bile. Capacity of gallbladder is 40–50 mL.

Fig. 12.4: CBD parts. ™™ Bile salts form micelle which makes cholesterol soluble. Bile

salts (i.e. salts of cholic and chenodeoxy­cholic acids) with formed deoxycholic acid (in the bowel) enters the enterohepatic circulation to get resecreted in the bile. ™™ Bilirubin conjugated in the liver is secreted into the bile, which in the bowel is converted into urobilinogen by bacteria. Urobilinogen gets absorbed in the bowel, enters the liver for

ORAL CHOLECYSTOGRAM (OCG; GRAHAM-COLE TEST) ™™ 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 OCG, plain X-ray abdomen in erect posture is taken to visualise the gallbladder.

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 Iopanoic acid is 500 mg.

•

Intravenous CHOLANGIOGRAM

™™ It is to visualise bile ducts and biliary tree, by injecting IV

Meglumine ioglycamate (Biligram) and taking X-ray abdomen. ™™ It can be combined with OCG. ™™ Problems with this method are poor visualisation, drug reac-

tion. It is not very useful if serum bilirubin is >3 mg%.

ENDOSCOPIC RETROGRADE CHOLANGIOPANCREATOGRAPHY (ERCP) 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) ¾¾ Malignancy—irregular filling defect. ¾¾ Chronic pancreatitis—‘chain-of-lakes’ appearance. ¾¾ Congenital anomalies—Stones. ¾¾ Stricture of biliary tree—Choledochal cyst. ¾¾ For sampling of biliary and pancreatic juices for analysis and cytology. ¾¾ Brush biopsy from tumour site.

B xx xx xx xx xx

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

™™ Complications

Pancreatitis; Cholangitis Duodenal injury, perforation; Sphincter stenosis ¾¾ Bleeding from pancreaticoduodenal artery. ™™ Relative contraindications: Acute pancreatitis; Previous gastrectomy. ¾¾ ¾¾

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.

625

xx xx xx

Indications

Failure of ERCP; high biliary strictures Klatskin tumour; stenting in high tumours High blocks when external and internal catheter drainage is needed

To be a successful surgeon, you need to have eye of a hawk, heart of a lion and hands of a lady.

CHAPTER 12    Gallbladder

resecretion again and part of it is excreted in the urine. Absence of urobilinogen in the urine signifies obstructive jaundice. ™™ In the absence of gallstones or any other disease, bile is sterile. Symptomatic gallstone disease shows positive culture for bacteria, commonest being E. coli and Klebsiella.

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A

B

C

D

E

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 CHD), in Klatskin tumour, in catheter drainage (external) in high blocks, in stenting high tumours. ™™ Prerequisites for PTC ¾¾ Should have normal prothrombin time (or should be made normal by injection of vitamin K or FFP). ¾¾ Should be a last and final inevitable method for evaluation or therapy. ¾¾ 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 ™™ I131 Rose Bengal and Tc99 labelled iminodiacetic acid (HIDA,

PIPIDA) are very useful in diagnosing acute cholecystitis and other biliary disorders, like biliary atresia. Note: HIDA is Hippuran Iminodiacetic 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.

Congenital anomalies of gallbladder xx xx

xx

xx

xx xx

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

xx xx

xx

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.

B xx xx

Residual CBD stones are removed by:

Dormia basket Fogarty’s catheter

xx xx

Choledochoscope ERCP A

B

Figs. 12.10A and B: (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.

A

Fig. 12.9: T-tube cholangiogram.

B

Figs. 12.11A and B: (A) Double cystic ducts from the gallbladder 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 feed him for a lifetime. —Lao Tzu

CHAPTER 12    Gallbladder

xx

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/floating/mesenteric 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 infundibulum 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 into the common hepatic duct or right hepatic duct

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CHOLEDOCHAL CYSTS ™™ It is defined as isolated/focal or combined/diffuse congenital

SRB's Manual of Surgery

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 inci-

dence is 1 in 1000 hospital admissions.

™™ It is often associated with pancreaticobiliary maljunction. A

B

Figs. 12.12A and B: (A) Cystic duct joining the right hepatic duct—a variation; (B) Phrygian (cap) gallbladder.

A

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.

B

Figs. 12.13A and B: (A) Gallbladder diverticulum; (B) Cystic artery originates from right hepatic artery which is anterior to common bile duct.

A

B

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.

Fig. 12.16: Pancreaticobiliary maljunction. Here pancreatic duct joins 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 modification of Alonso-Lej classification) ™™ Type I: Dilatation of extrahepatic biliary tree (60%).

Type Ia—cystic. Type Ib—focal segmental (saccular). ¾¾ Type Ic—fusiform. ™™ Type II: Diverticulum of extrahepatic biliary tree (5%). ™™ Type III: Choledochocele—cystic dilatation of intraduodenal part of CBD (5%). ™™ Type IV: Dilatation of extra- and intrahepatic or multiple parts of extrahepatic biliary tree (30%). ¾¾ Type IVa: Dilatation of extrahepatic and intrahepatic biliary tree. It is 2nd most common. ¾¾ Type IVb: Dilatation of multiple sections of the extrahepatic bile duct. ¾¾ ¾¾

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 V: Dilatation of the only intrahepatic biliary tree (Caroli’s

disease).

Complications

629

™™ Pancreatitis, mainly in type III. ™™ Suppurative cholangitis. ™™ 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.

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–ideal. ™™ Liver function tests. ™™ PTC to see intrahepatic biliary tree.

Treatment ™™ Resection of extrahepatic biliary tree with removal of chole-

Fig. 12.17: Different types of choledochal cysts—(1) Fusiform type, (2) Saccular type, (3) Choledochocele, (4) Fusiform + Intrahepatic type, (5) Intrahepatic type.

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 xx xx

Right upper quadrant pain Palpable abdominal mass

B xx xx

Triad of choledochal cyst (seen in 10% of cases) xx

Jaundice

Swellings which appear and disappear

Hydronephrosis Intussusception

xx xx

Pseudopancreatic cyst Choledochal cyst

dochal cyst along with cholecystectomy and Roux-en-Y hepaticojejunostomy is the ideal treatment for choledochal cyst especially types I, II 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 III—endoscopic sphincterotomy is done. Excision is also often needed. ™™ Intrahepatic 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 chemotherapy is given. ™™ Liver transplantation is the choice in type IV and V cases.

Gangrenous cholecystitis is associated with thrombosis of cystic artery.

CHAPTER 12    Gallbladder

™™ Gallstone and CBD stone formation.

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CAROLI’S DISEASE ™™ It is congenital, nonfamilial, multiple, irregular, dilatations

SRB's Manual of Surgery

of the intrahepatic ducts with stenotic segments in between. It is associated with congenital hepatic fibrosis and medullary sponge kidney. It can also be included under type V choledochal cyst. ™™ Presentations: It occurs in young 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.

A

BILIARY ATRESIA ™™ It may be either due to viral infection or defective embryogen-

esis 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, destruc-

B

tion 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).

B xx xx xx xx xx

Aetiology of biliary atresia

Infections—cytomegalovirus, reovirus, 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 xx xx xx

Fig. 12.19: Choledochal cyst on table look.

Biliary atresia

Type I: Atretic CBD—10% Type II: Atretic CBD and common hepatic duct Type III: Atretic CBD, common hepatic duct and right and left hepatic ducts—88%

Fig. 12.20: Types of biliary atresia.

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. ¾¾ Metabolic diseases; storage disorders. ¾¾ Alagille syndrome; hypoplastic biliary ducts. ™™ Diagnosis ¾¾ Liver function test—conjugated hyperbilirubinaemia. ¾¾ US abdomen shows triangular cord, tubular echogenicity extending above the bifurcation of the portal vein which is more than 4 mm thick—diagnostic. ¾¾ MRCP—100% accuracy. ¾¾ Radioisotope study (HIDA or PIPIDA scan). ¾¾ On table cholangiogram. ¾¾ Liver biopsy (Percutaneous)—is usually first test obtained. ¾¾ Rose Bengal I131 study, if shows 10%, it is more likely to be hepatitis.

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.

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

¾¾ ¾¾

Treatment ™™ In correctable cases: Roux-en-Y jejunal 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, procedure 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

Fig. 12.21: Multiple gallstones removed along with gallbladder.

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.

Pathogenesis I. Metabolic: ¾¾ Cholesterol is synthesised in liver. Its solubility is determined by relative concentration of cholesterol, bile salts

Porcelain gallbladder is radiopaque gallbladder, due to calcification of the gallbladder wall following chronic cholecystitis which has got high malignant potential.

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CHAPTER 12    Gallbladder

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.

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SRB's Manual of Surgery

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).

¾¾

¾¾

¾¾

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 fluid 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 concentration 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.

Factors altering the cholesterol to bile salt ratio Obesity, Mutation (MDR3, CYP7, A1) Drugs –– Oral contraceptive pills, octreotide –– Clofibrate; cholestyramine Ileal disease; ileal resection Altered enterohepatic circulation

B xx xx

Fig. 12.22: Diagram showing different causes of gallstone formation.

Fig. 12.23: Pigment stones showing faceted look. ¾¾

¾¾

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

xx xx

II. Infections and Infestations: ¾¾ Bacteria like E. coli, Salmonella ¾¾ Parasites like Clonorchis sinensis and Ascaris lumbricoides are often associated. ¾¾ Moynihan’s aphorism: “A gallstone is a tomb stone erected to the memory of the organism within it.” III. Bile stasis: ¾¾ Occurs due to estrogen therapy, pregnancy, vagotomy and in patients who are on long-term intravenous fluids or TPN. IV. Increased bilirubin production ¾¾ Due to any of the causes of haemolysis as in hereditary spherocyto­sis, sickle cell anaemia, thalassaemia, malaria, cirrhosis. Here pigment stones are common.

B

Saint’s triad

Gallstones; diverticulosis of the colon; hiatus hernia ™™ 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 radio-

lucent.

Figs. 12.26A and B: Gallbladder with stone removed from Hartmann’s pouch.

Effects of the Gallstones ™™ In the gallbladder ¾¾ ¾¾

Fig. 12.24: Multiple gallstones collected during cholecystectomy. Note:

•

•

•

A

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 A and 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 β 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.

B

Figs. 12.25A and B: (A) Gallstones removed, on cutting showing central nidus; (B) Thickened gallbladder due to chronic cholecystitis with single cholesterol stone.

¾¾

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.

A

B

C

D

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

CHAPTER 12    Gallbladder

B

A

634

Empyema gallbladder. Perforation causing biliary peritonitis or pericholecystitic abscess. ¾¾ Mucocele of gallbladder. ¾¾ Limey gallbladder. ¾¾ Carcinoma gallbladder. ™™ In the CBD ¾¾ Secondary CBD stones (occurs in 10% of gallstones). ¾¾ Cholangitis. ¾¾ Pancreatitis. ¾¾ Mirizzi syndrome (compression of CHD/CBD by stone from cystic duct or cholecysto-choledochal fistula). ¾¾

SRB's Manual of Surgery

Management of Gallstones

¾¾

™™ 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.

A

Fig. 12.28: Diagram showing complications of gallstones. ™™ In the intestine ¾¾

Cholecystoduodenal fistula causing gallstone ileus and so intestinal obstruction.

B

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 pressure exerted by each stone. Centre of the gallstone is often found radiolucent and is called as Mercedes Benz sign/Seagull sign.

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.

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.

B xx xx xx xx

Silent gallstone

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.

635

™™ Through bile after filtering in the liver via portal circulation.

™™ Stone causes obstruction at Hartmann’s pouch or in cystic

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.

B xx xx xx xx xx xx xx xx xx

Differential diagnosis of radio-opaque shadow in X-ray Kidney stone Gallstone Calcified 12th rib tip Phlebolith Pancreatic stone Radio-opaque foreign body Faecolith Calcified lymph node Calcified renal tuberculosis

xx xx xx xx xx

Renal cell carcinoma— calcifications Adrenal tumour— calcification Teratomatous dermoid Calcification of atheroma in aorta Calcified lesion in liver— amoebic 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.

B xx xx xx

Causative bacteria are: E. coli—most common Klebsiella, Pseudomonas, Proteus Strep. faecalis

xx xx

Salmonella Clostridium welchii

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.

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.

Complications of Acute Cholecystitis Acute cholecystitis can lead to: ™™ Perforation—5–10% which usually occurs in the fundus or in the neck (Hartmann’s). It can cause cholecystoduodenal, cholecystointestinal or cholecystobiliary fistula, Mirrrizi's syndrome. ™™ Peritonitis; Pericholecystitic abscess. ™™ Cholangitis and septicaemia. ™™ Empyema gallbladder; gangrenous gallbladder.

B xx xx xx xx

Classification

xx

1. Acute calculous cholecystitis. 2. Acute acalculous cholecystitis (10%).

xx xx xx

Mode of Infection

xx

™™ Haematogenous through hepatic artery—cystic artery.

xx

Emphysematous cholecystitis

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.

CHAPTER 12    Gallbladder

Pathogenesis of Acute Cholecystitis

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 impor-

SRB's Manual of Surgery

tant 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.

Fig. 12.32: On table look of gallbladder with acute cholecystitis. Fig. 12.34: Ultrasound showing roundworm in gallbladder.

Fig. 12.33: Gallbladder with acute cholecystitis—laparoscopic view.

Clinical Features ™™ Sudden onset of pain in the right hypochondrium, with

tenderness, guarding, 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).

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 xx xx xx

Differential diagnosis Duodenal ulcer perforation Acute pancreatitis Acute appendicitis

xx xx xx

Acute pyelonephritis Lobar pneumonia, myocardial infarction Ruptured ectopic pregnancy

Treatment ™™ Advised hospitalisation. ™™ Initially (nonoperative) conservative treatment (95%): ¾¾ ¾¾ ¾¾ ¾¾

Nasogastric aspiration. IV fluids. Analgesics and antispasmodics. Broad spectrum antibiotics (cefoperazone, ceftazidime, ceftria­xone, cefotaxime + amikacin, tobramycin + metronidazole {antimicrobial}).

¾¾

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 cholecystostomy also can be used. After 3 weeks, elective cholecystectomy is done. Indications for cholecystostomy or emergency cholecys­tectomy (5%) Empyema GB Persisting symptoms/failure of medication Emphysematous cholecystitis Perforation/peritonitis; elderly

B xx xx xx xx

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.

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 VII 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 very common (70%). ™™ Investigations: Isotope study (HIDA), US abdomen. ™™ Treatment: Cholecystectomy.

Surgery is to be done, not to have.

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CHAPTER 12    Gallbladder

Observation. Follow-up U/S scan. ™™ Later after 3–6 weeks, elective interval cholecystectomy, either by open method through right subcostal (Kocher’s) incision or through laparoscopy is done. ¾¾

SRB's Manual of Surgery

638

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 CHD is found. HIDA scan is useful. Mirizi’s types Features (Csendes classification, modified in 2008) Type I—10% Extrinsic compression of the CBD/CHD by a large impacted stone in Hartmann’s pouch; IA is presence of cystic duct; IB is obliterated cystic duct—ERCP—tenting—subtotal/partial cholecystectomy Type II— 40– 50%

The stone has eroded the CBD/CHD less than 1/3rd circumference of the CBD/CHD forming a fistula— subtotal cholecystectomy with CBD exploration

Type III— 20–30%

Involving up to 2/3rd of the circumference of CBD/ CHD—subtotal cholecystectomy with CBD exploration

Type IV— 2–5%

Cholecystocholedochal fistula (>2/3rd) with involving entire circumference of the CBD/CHD – needs hepaticojejunostomy

Type V— 15–25%

Cholecystocholedochal fistula with cholecystoenteric fistula without gallstone ileus (VA) or with gallstone ileus (VB)

Note: Incidence of gallbladder cancer association is more with Mirizzi syndrome.

A

¾¾

¾¾ ¾¾

¾¾

¾¾

¾¾

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 II. 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 III. Cholecystectomy with duodenal/jejunal anastomosis (hepaticojejunostomy) is done in type 4 and type V and when difficulty arises in type I, II, III also. Postoperative surgical mortality is 8–10% in Mirizzi syndrome.

EMPYEMA GALLBLADDER ™™ 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 peritonitis— biliary and bacterial. ™™ Condition has got high mortality.

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.

B

Figs. 12.39A and B: Mirizzi syndrome type I and IV. ™™ Treatment: ¾¾

Open approach is ideal for Mirizzi even though laparoscopic approach is done in few centres.

Fig. 12.40: Gallstone obstructing the neck of the gallbladder in Hartmann’s pouch, may lead into empyema or mucocele of the GB.

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. ¾¾

Total count is raised; Haematocrit, LFT, Pt-INR.

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.

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.

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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.

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MURPHY’S SIGN ™™ It is a sign of cholecystitis—acute or acute on chronic chol-

ecystitis.

SRB's Manual of Surgery

™™ John Benjamin Murphy (1857–1916) American surgeon,

Fig. 12.44: Specimen of gallbladder showing thickened gallbladder wall with multiple gallstones. Faceting of stones is due to equal pressure.

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.

A

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 upright/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 thumb 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 B

C

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.

B

Causes for gas in the biliary tree Cholecystoduodenal fistula Choledochoduodenostomy Choledochojejunostomy

xx xx xx

xx xx

Transduodenal sphincteroplasty Emphysematous cholecystitis

Note:

• Fig. 12.46: Gallstone ileus. Gallstones in the gallbladder (stone > 2.5 cm) ↓ Cholecystitis ↓ Suppuration and adhesion over the duodenal wall ↓ Communication of gallbladder into the duodenum (Spontaneous bilioenteric fistula) ↓ Gallstones pass into the duodenum forms a bolus (‘Rolling stone gather mass’) ↓ Blocks narrow part in the ileum. ↓ Gallstone ileus

Features ™™ Pain abdomen and features of intestinal obs­truction. ™™ Stones may perforate the ileum to cause peritonitis.

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.

™™ It is 1% of all intestinal obstruction overall; 25% of obstruc-

tion 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. ¾¾ U/S abdomen. ¾¾ CT is diagnostic. ™™ Treatment ¾¾ 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. ¾¾ Laparotomy and crushing of stones with fingers to relieve the obstruction is only occasionally useful. ¾¾

A

B

Figs. 12.47A and B: (A) Types of cholecystoses. (I) Cholesterosis, (II) Cholesterol polyposis, (III) Cholecystitis glandularis proliferans, (IV) Diverticulosis of gallbladder, (V) Fistula; (B) Cholesterosis of gallbladder. It is a premalignant condition.

Types I. Aggregations of cholesterol crystals in the mucosa or submucosa—cholesterosis (Strawberry gallbladder). Lipoid

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Cholecystectomy, correction of fistula with T-tube 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.

¾¾

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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 mucosa— cholesterol polyposis (Gallbladder polyp). III. 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 (OCG); 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.

DISSOLUTION THERAPY FOR GALLSTONES Indications ™™ Functioning gallbladder with cholesterol stone. ™™ Single stone less than 1.5 cm. ™™ Radiolucent stone. ™™ Old age. ™™ Patients who are not fit for surgery.

B

Contraindications

Nonfunctioning gallbladder Stone more than 1.5 cm

xx xx

xx xx

Classification 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: Stasis and bactibilia are the main causes of primary CBD biliary tree stones.

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.

Radio-opaque stone Multiple stones

™™ 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 CoA— a rate limiting step in cholesterol synthesis. Ursodeoxycholic acid also inhibits absorption of cholesterol in GIT. ¾¾ ¾¾

B xx xx xx

Other methods used

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. ¾¾ ¾¾ ¾¾ ¾¾

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). • Intrahepatic 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

Clinical Features

™™ CT scan is the most sensitive investigation for CBD stones;

™™ Incidental CBD stones along with jaundice/without jaundice. ™™ Pain: It may be biliary colic; nonspecific abdominal pain; pain

™™ Fever with chills and rigors. ™™ Pain and tenderness in epigastrium and right hypochondrium. ™™ Steatorrhoea and darkening of urine; pruritus.

B xx xx

Charcot’s triad of ascending cholangitis

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

Jaundice

Pain

Stasis

Infection, bacteraemia and fever

™™ Reynold’s pentad of acute obstructive cholangitis (suppura-

tive cholangitis—5%). Here biliary tree contains pus. →

B

Sometimes obstruction persists causing

Persistent pain; persistent fever; persistent jaundice; shock (toxicity); altered mental status

Treatment ™™ Injection Vit. K 10 mg 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 BD to prevent hepatorenal syndrome. ™™ ERCP—Therapeutic, i.e. endoscopic papillotomy (sphincter-

otomy) 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.

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.

B

Differential diagnosis

Obstructive jaundice due to other causes: xx Carcinoma of head of pancreas xx Carcinoma of periampullary region xx Carcinoma of biliary tree; biliary stricture; viral hepatitis

Investigations ™™ US abdomen 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

CHAPTER 12    Gallbladder

of ascending cholangitis, pain of pancreatitis. ™™ Jaundice—most common clinical manifestation.

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.

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CBD stone/s and if ERCP was not done prior to cholecystectomy. If secondary stone is retrieved by this method, open exploration of CBD can be avoided. ™™ Intraoperative ultrasound (IOUS) or intraoperative choledochoscope is very useful to confirm that all stones are removed.

A

B

Figs. 12.50A and B: ERCP showing CBD stones. Endoscopic view of stenting is also seen. ™™ Complications of sphincterotomy: Bleeding; sepsis; perfora-

tion; pancreatitis Once CBD stones are extracted through ERCP, laparoscopic cholecystectomy is done. ™™ In open cholecystectomy ¾¾ 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. ¾¾

B

False results/failure of intraoperative cholangiogram (IOC) 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. Indications for choledochotomy

(After open cholecystectomy) xx U/S shows stone in CBD; palpable stone in CBD xx CBD diameter >8–10 mm xx Recent history of jaundice, and with raised serum alkaline phosphatase level xx On table cholangiogram shows stone (done using C-arm image machine) xx Failure of stone extraction by ERCP prior to cholecystectomy. xx 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. ¾¾ T-tube (Kehr’s) is then placed in the CBD and kept for 14 days. ¾¾ After 14 days a postoperative T-tube cholangiogram is done to see for free flow of dye into the duodenum, so that T-tube can be removed. ¾¾ If T-tube 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 ¾¾

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. ™™ Laparoscopic CBD exploration is becoming popular with

availability of expertise and high technology imaging and instruments.

B xx xx

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 xx xx xx

xx xx xx xx

xx xx

Methods to confirm the removal of T-tube

Management of retained CBD stones

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 (Canada)—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) ERCP and 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

Contd... xx xx

xx

B

A

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) T-tube 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. ¾¾ These procedures attain complete drainage of bile. ¾¾ Postoperatively they may often require long-term antibiotics. Indications for choledochoduodenostomy/ 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.

xx

xx

xx xx xx xx

B xx xx xx xx xx xx

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/strictures/intrahepatic stones. Problem with choledochoduodenostomy: ™™ Sump syndrome. Note: Roux-en-Y choledochojejunostomy is equally good as choledochoduodenostomy.

B xx xx xx

xx

Remember

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 one which is identified on routine investigation where there are no specific relevant symptoms related to gallstones. Chances of developing symptoms in a silent gallstone is 5% in 5 years and 20% in 15 years. Contd...

xx xx xx xx

xx

xx

xx

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.

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xx

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 out—CBD 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 gallbladder). 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 micelle and is called as lithogenic bile. Here bile is in supramicellar zone. Cholesterol stone is radiolucent but causes acoustic shadow in ultrasound Mixed stones are most common—90%. ‘Gallstone is a tomb stone erected to the memory of the organism within it’—Moynihan’s aphorism. Saint’s triad: Gallstones—colonic diverticulosis—hiatus hernia. Complications of gallstones: Acute cholecystitis, chronic 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 condition. 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. Residual/retained 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.

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Rule may not be useful in

Absence of gallbladder Intrahepatic gallbladder

xx

Previous cholecystectomy

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) 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’.

It is the jaundice that develops due to biliary obstruction, partial or complete or intermittent. It causes conjugated hyper­bilirubinaemia. Normal serum bilirubin level is 0.2–0.8 mg%. Scleral icterus is visible when serum bilirubin level exceeds 2.5 mg%.

Pathophysiology in Obstructive Jaundice

xx

xx

xx

xx

Fig. 12.54: Courvoisier’s law—“In 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.

Bilirubin metabolism and enterohepatic circulation 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 urobilinogen 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.

B

xx

™™ 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 periampullary 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.

Fig. 12.55: Sclera of the patient with obstructive jaundice is greenish yellow in colour.

™™ Obstructive jaundice, often called as surgical jaundice

Effects of obstructive jaundice ™™ In liver: Enlarged green bile stained liver (hydrohepatosis)

shows dilated intrahepatic biliary radicles. Once intraductal CBD pressure increases bile secretion from liver is reduced causing formation of ‘white bile’ in CBD. Biliary cirrhosis may develop later. ™™ In the biliary tree: Recurrent inflammation—cholangitis— fibrosis can occur. ™™ In bowel: Absence of bile from bowel impairs digestion, reduces fat absorption making faeces bulky and fatty. Vitamin K absorption is reduced causing fall in prothrombin level raising PT-INR. ™™ Retention of bile salts and bile pigments in blood and body fluids occurs. ™™ Altered coagulation profile; hepatorenal syndrome and renal failure; sepsis.

Classification of Causes of Obstructive Jaundice 1. Congenital: Biliary atresia, choledochal cyst. 2. Inflammatory: Ascending cholangitis, sclerosing cholangitis. 3. Obstructive: CBD stones, biliary stricture, parasitic infestation. 4. Neoplastic: Carcinoma of head or periampullary region of pancreas, cholangiocarcinomas, Klatskin tumour. 5. Extrinsic compression of CBD by lymph nodes or tumours.

Fig. 12.56: Different causes of obstructive jaundice (surgical jaundice).

You are the sole master of your thought processes.

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occurs due to intrahepatic or extrahepatic biliary outflow obstruction. It leads into cholestasis which means conjugated hyperbilirubinaemia; probably due to impaired bile flow and impaired bile formation. Bile acid secretion into the gut is impaired causing defective absorption of fat and fat-soluble vitamins like vitamin K; this, in turn causes poor synthesis of prothrombin and conversion of prothrombin to thrombin causing widened PT and PT INR. Factors II, VII, IX, and X are vitamin K-dependent clotting factors. PT indicates the extrinsic pathway of coagulation; whereas partial thromboplastin time represents the intrinsic pathway. Bile acid stasis causes hepatocytes injury. Fat malabsorption causes steatorrhoea and also deficiencies of vitamins A (visual problem, thick skin), D (osteomalacia), E (peripheral neuropathy, cerebellar ataxia, posterior column dysfunction), K (bleeding tendencies). Cholestyramine and colestipol, used to treat pruritus, bind to bile salts and can exacerbate these vitamin deficiencies. Persistent cholestasis may be associated with deposits of cholesterol in the skin (cutaneous xanthomatosis), occasionally in bones and peripheral nerves. ™™ Bilirubinostasis (bile plugs) cause hepatocytes degeneration, small duct and ductular obstruction, pericholangitis, oedema, bile leak, liver infarction and biliary cirrhosis. ™™ Obstruction may be due to benign conditions like biliary tree stones (most common cause) or strictures or sclerosing cholangitis; or due to malignant conditions like carcinoma of pancreas or cholangiocarcinoma. Extrahepatic obstruction may be luminal (stones, infestations [ascariasis, clonorchis sinensis and schistosomiasis]) or mucosal/wall (growth, stricture [post-inflammatory, postsurgical or post-RT], primary sclerosing cholangitis) or external compression (pancreatitis, pancreatic tumour, compression by nodal mass). ™™ Intrahepatic cholestasis generally occurs at the level of the hepatocyte or biliary canalicular membrane. Causes include hepatocellular disease (e.g. viral hepatitis, druginduced hepatitis/cholestasis (thiazides, chlorpromazine), biliary cirrhosis. In hepatocellular disease, interference in the 3 major steps of bilirubin metabolism—uptake, conjugation and excretion usually occur. Excretion is the rate-limiting step and is usually impaired to the greatest extent. As a result, conjugated bilirubin predominates in the serum. ™™ The lack of bilirubin in the intestinal tract is responsible for the pale stools in biliary obstruction. The cause of itching (pruritus) associated with biliary obstruction is not clear; may be related to the accumulation of bile acids in the skin; or may be related to the release of endogenous opioids. ™™ Severe biliary obstruction causes cell damage usually in 1 month and, if unrelieved, may lead to secondary biliary

cirrhosis. Acute cholangitis is another complication associated with obstruction of the biliary tract and is the most commonly seen in stricture, most often at the level of the CBD. Bile here is usually sterile. Bile flow obstruction → stasis → colonization and multiplication of bacteria → concomitant increased intraductal pressure → reflux of biliary contents → bacteremia, septicaemia → septic shock. If recurrence of biliary colic after cholecystectomy occurs, one should think of possible choledocholithiasis.

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Benjamin’s (1983) classification of biliary obstruction Type 1: Complete obstruction xx Tumours—pancreatic, cholangiocarcinoma xx CBD ligation—iatrogenic xx Primary/secondary liver tumours

B

Type 2: Intermittent obstruction Choledocholithiasis; Periampullary tumour xx Choledochal cyst; Bile duct papilloma xx Hemobilia; Duodenal diverticula xx

™™ 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.

Type 3: Chronic complete obstruction Bile duct stricture; Congenital xx Traumatic; Post-radiotherapy xx Chronic pancreatitis; Cystic fibrosis xx

Type 4: Segmental obstruction Traumatic; Sclerosing cholangitis xx Cholangiocarcinoma, intrahepatic biliary stones (hepatolithiasis) xx

™™ Clinical features ¾¾ ¾¾ ¾¾ ¾¾

¾¾ ¾¾ ¾¾

Severe jaundice; Pruritus, more on the back and forearms. Fever, may or may not be present. Loss of weight; Loss of appetite. Pain in right hypochondrium, palpable gallbladder, hydrohepatotic palpable, smooth, soft, non-tender liver are other features. Courvoisier’s law may suggest inflammatory/neoplastic cause. Charcot’s triad/Reynold’s pentad as presentation in cholangitis. Steatorrhoea (more fatty stool) due to improper absorption of fat soluble vitamins.

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, SGPT, SGOT, 5’ nucleotidase. ™™ Total count may be raised with neutrophilia in inflammatory conditions. Serum alkaline phosphatase (ALP) and γ glutamyl 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. ™™ Intraductal US (IDUS): 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 jaundice, it is absent; and in haemolytic jaundice, it is in excess.

B xx xx xx xx xx xx xx xx xx xx xx

xx

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, mucosal 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 difficult 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) Causes 1. Postoperative (80% common) ¾¾ After cholecystectomy [open or laparoscopic, more common following laparoscopic (0.8%) than open method (0.35%)]. ¾¾ Biliary surgery; gastrectomy; liver surgery. ¾¾ Duodenal and pancreatic surgery. 2. Inflammatory: Stricture following recurrent attacks of cholangitis due to: ¾¾ CBD stones. ¾¾ Parasites—Ascaris lumbricoids, Clonorchis sinensis. ¾¾ Primary sclerosing cholangitis. 3. Malignant: Due to cholangiocarcinoma. 4. Traumatic.

B

Bismuth classification of biliary stricture

I. Low CBD stricture with stump (proximal to stricture) > 2 cm II. Middle CBD stricture with proximal stump < 2 cm III. Hilar. Confluence of right and left hepatic ducts is intact IV. Separated right and left hepatic ducts V. Stricture involving intrahepatic ducts

A

B

C

D

Postoperative Management ™™ Monitoring with prothrombin time, bilirubin, albumin, creati-

nine, electrolyte estimation. ™™ FFP or blood transfusion; antibiotics. ™™ Observation for septicaemia, haemorrhage, pneu­monia, pleural effusion, bile leak. ™™ Care of T-tube and drains. ™™ T-tube cholangiogram in 10–14 days. ™™ TPN, CVP line, nasogastric tube, urinary catheter.

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E

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

CHAPTER 12    Gallbladder

Preoperative preparation of patient with obstructive jaundice 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 BD 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, hepatic encephalopathy and electrolyte imbalance (correction of hypoglycaemia and dilutional hyponatraemia due to water retention; avoiding isotonic saline infusion).

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™™ Features ¾¾

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B xx xx xx xx xx

Obstructive jaundice; Pain abdomen. Features of ascending cholangitis. Profuse persistent bile leak. Causes of postoperative stricture

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

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. ¾¾

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.

Types 1. Primary sclerosing cholangitis is one wherein no cause is found and is associated with ulcerative colitis, Sjögren’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 multiple areas of strictures and dilatations (1%/year). It has high risk for cholangiocarcinoma. It could be an autoimmune disease. It is common in HLA/B8/DR3 halotype. 2. Secondary sclerosing cholangitis is due to stones, trauma, congenital lesions. AIDS, chemotherapy (5FU), transplantation, collagen diseases, sarcoidosis, histiocytosis.

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, medi-

astinal fibrosis, Riedel’s thyroiditis, orbital pseudo-tumour. ™™ Hepatic duct confluence is most severely strictured segment

in PSC. A

B

Figs. 12.59A and B: ERCP picture showing stricture and passing stent into CBD.

™™ Investigation: ERCP shows beaded appearance of biliary tree;

LFT is altered; liver biopsy is needed to identify the severity of hepatic fibrosis. ™™ Treatment: ¾¾ Stenting; T-tube drainage. ¾¾ Large dose steroids; immunosuppression therapy— methotrexate, azathioprine, tacrolimus, cyclosporine; Ursodeoxycholic acid is beneficial ¾¾ liver transplantation; Median survival in PSC after diagnosis is 10 years.

GALLBLADDER POLYP ™™ Its incidence is 5% in routine US abdomen; 10% in cholecys-

tectomy 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 A

B

Figs. 12.60A and B: CBD stent and imaging after placement.

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.

™™ It is the most common benign biliary tumour. ™™ 50% occurs close to ampulla 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.

Aetiologies for Carcinoma of Gallbladder ™™ 3% of gallstones with cholecystitis will develop carcinoma

of gallbladder. ™™ 90% of carcinoma of gallbladder is associated with gall-

stones. 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.

™™ 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.

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.

A

B

Gross Types of Carcinoma Gallbladder ™™ Polypoid/papillary—better prognosis. ™™ Scirrhous/nodular. ™™ Proliferative/infiltrative.

Microscopy ™™ Commonly it is adenocarcinoma (90%); occasionally squa-

mous cell carcinoma, adenosquamous or carcinoid tumour can occur. ™™ 25% show only localised disease; 35% have lymph node spread; 40% have distant spread at the time of first diagnosis. ™™ It is very aggressive tumour.

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C

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

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BENIGN BILIARY PAPILLOMA

Spread of Carcinoma Gallbladder

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™™ Investigations ¾¾

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T

Ultrasound abdomen. CT abdomen to see operability. US-guided FNAC; liver function tests; MRCP; laparoscopy. CA 19-9 is elevated in 80% of cases.

TNM staging for gallbladder cancer (AJCC, 8th edition, 2018)

Tumour (T)

Metastases (M)

TX Primary tumor cannot be assessed M0 – No distant spread. M1 Distant spread. T0 No evidence of primary tumor Tis Carcinoma in situ T1 Tumor invades lamina propria or muscular layer T1a Tumour invades lamina propria T1b 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 perforates 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) Nx Regional lymph nodes cannot be assessed N0 Regional lymph node metastasis N1 Metastases to 1–3 lymph nodes N2 Metastases to ≥4 lymph nodes

B

Staging Stage 0–Tis, N0, M0 Stage I – T1, N0,M0 Stage IIA – T2a, N0, M0; IIB – T2b, N0, M0 Stage III A – T3, N0, M0 Stage III B – T1 to T3, N1, M0 Stage IV A – T4, N0 or N1, M0 Stage IV B – Any T, N2, M0; Any T, any N, M1 Note: Stage I is Localised; Stages II to IV B are unresectable.

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.

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.

Nevin’s Staging of carcinoma gallbladder

Stage I – Intramural Stage II – Spread to muscularis propria Stage III – Spread to serosa

Stage IV – Spread to cystic lymph node of Lund Stage V – Direct spread to adjacent organs/metastases

Treatment ™™ Cholecystectomy with resection of liver segments IV and V—

extended 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

A

B

Figs. 12.63A and B: (A) Gallbladder showing proliferative carcinomatous lesion; (B) Gallbladder showing scirrhous carcinoma with pre-existing gallstones and cholesteroses.

CHOLANGIOCARCINOMA (Bile Duct Carcinoma) 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.

Risk Factors for Cholangiocarcinoma ™™ Primary sclerosing cholangitis (PSC): Here commonly it is

extrahepatic, usually at the confluence. ™™ Choledochal cyst; Hepatolithiasis. ™™ Hepatitis B and C are risk factors for intrahepatic cholangiocarcinoma. ™™ Lynch syndrome II. ™™ Multiple biliary papillomatosis. ™™ Previous biliary enteric anastomosis. ™™ Clonorchis sinensis infestation. ™™ Thorotrast, nitrosamines, dioxin.

T

Different classifications

Anatomical: 1. Intrahepatic—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 cholangiocarcinoma (Bismuth Corlette): Type I: Below the confluence of RHD and LHD Type II: Reaching the confluence of RHD and LHD Type III: Involving CHD with RHD (IIIA) or LHD (IIIB) Type IV: Involving confluence and both RHD and LHD or multicentric tumour

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: ¾¾ ERCP and choledochoscope; liver function tests; PTC. ¾¾ Ultrasound abdomen, CT scan. ¾¾ MR scan—Best investigation. MRCP to see duct; MRI to see nodes; MR angiogram to see vascularity.

T

American Joint Cancer Committee/Union for International Cancer Control 8th edition, TNM staging for perihilar cholangiocarcinoma

Primary tumor (T)

Regional lymph nodes (N)

Tx Primary tumor cannot be assessed T0 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

NX Regional lymph nodes cannot be assessed N0 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 ≥4 nodes

Note: TNM staging defers for intrahepatic, perihilar and distal cholangiocarcinoma.

Distant metastases (M) M0 No distant metastases M1 Distant metastases present Staging Stage 0 Tis N0 M0 Stage I T1 N0 M0 Stage II T2A-B N0 M0 Stage IIIA T3 N0 M0 Stage IIIB T4 N0 M0 Stage IIIC Any T N1 M0 Stage IVA Any T N2 Mo Stage IVB Any Any N M1

Treatment ™™ When operable, portal region clearance with hemihepatec-

tomy can be done. Intrahepatic 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 (ERBT).

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).

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™™ It is associated with sclerosing cholangitis, clonor­chiasis

Assessment should be done to check the operability of tumour by—assessing the spread in bilateral hepatic duct up to secondary radicles, both hepatic arterial spread, portal vein encasement, hepatic lobe atrophy, distant spread.

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™™ Klatskin tumour is classified as 4 types—I: Just at or below

the confluence; II: At the confluence; III: At the confluence extending along the RHD; IV: At the confluence extending along the LHD. ™™ It causes obstructive jaundice with hydrohepatosis without enlargement of gallbladder. ™™ Management is like cholangiocarcinoma. ¾¾ Hilar resection with hepaticojejunostomy. ¾¾ Segment III left hepaticojejunostomy—Blumgart’s. ¾¾ Longmire’s left hepaticojejunostomy after left liver lobe resection. ¾¾ Cattell’s hepaticojejunostomy. ¾¾ Smith’s mucosal graft hepaticojejunostomy. ¾¾ Palliative PTC with stenting/ERCP stenting, intraoperative stenting. Metal stents show long period of patency (12 months) than polyethylene stents (4 months).

BILIARY FISTULAS Types a. External: Usually occurs as a complication of surgery following: ¾¾ Gastrectomy. ¾¾ Cholecystectomy open or laparoscopic. ¾¾ CBD surgery; pancreatic surgery. b. Internal: ¾¾ Cholecystoduodenal fistula causing gallstone ileus. ¾¾ Cholecystoenteric fistula. ¾¾ 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 ¾¾ Total parenteral nutrition (TPN). ¾¾ Antibiotics; blood transfusion; electrolyte management. ¾¾ Care of the fistula wound with regular dressing, using zinc oxide cream has to be done to protect the skin. ¾¾ Later continuity of biliary system has to be restored by open surgery or through ERCP and stenting.

HEMOBILIA ™™ It is bleeding commonly from the liver or occasionally from A

B

Figs. 12.64A and B: (A) Resected specimen of Klatskin tumour (Courtesy: Dr Arunkumar, MCh); (B) Klatskin tumour.

B xx xx xx

xx xx xx xx xx xx xx xx xx xx xx

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 Intrahepatic 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 I131 anti-CEA antibodies are adjuvant therapies Condition has got 90% mortality in one year

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.

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.

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.

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™™ Early rapid presentation can occur; but delayed, after weeks/

B

Triad of Sandblom/Quincke’s in hemobilia

1. Jaundice—60%; 2.  Pain—70% 3. Upper GI bleed with melaena (90%), haematemesis (60%) All three features of triad are seen only in 20% of cases. ™™ Investigations ¾¾ ¾¾ ¾¾

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. ¾¾ Antibiotics; blood transfusions. ¾¾ Selective arterial embolisation. Transarterial embolisation (TAE) shows 90% success rate. It is the choice therapy with least morbidity. Coils and glue are used. ¾¾ 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.

CHOLECYSTECTOMY It is the surgical removal of gallbladder.

B xx xx

Prophylactic cholecystectomy is done in

Diabetic patients; congenital haemolytic anaemia Patients who has underwent bariatric surgery

Indications ™™ Gallstones—symptomatic. ™™ Cholecystitis—acute, chronic; acalculous. ™™ Empyema; mucocele gallbladder.

Approach ™™ Open

Right subcostal incision (Kocher’s). Right paramedian; midine incision. ¾¾ Horizontal incision. ¾¾ Mayo-Robson incision. ™™ Laparoscopic approach. ¾¾ ¾¾

WHITE BILE ™™ 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.

A

B

Figs. 12.66A and B: (A) Incisions for open cholecystectomy and laparoscopic cholecystectomy; (B) Kocher’s incision used for open cholecystectomy.

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

CHAPTER 12    Gallbladder

months/years, presentations are known to occur which is often difficult to suspect and diagnose. ™™ These recurrent clots can cause pancreatitis, cholangitis, anaemia, cholecystitis.

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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.

Complications which can occur in both: Infection and subphrenic abscess xx Bleeding from cystic artery, and from liver bed xx Injury to CBD or hepatic duct xx Bile leak and biliary fistula formation xx Biliary stricture formation xx Injuries to colon, duodenum, mesentery xx

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.

Ports ™™ 10 mm port in umbilicus to pass 10 mm telescope. ™™ 10 mm port in midline epigastrium as working channel. ™™ Two 5 mm ports at midclavicular and anterior axillary line in

subcostal region.

Procedure

Fig. 12.67: Ligation of cystic duct and cystic artery while doing cholecystectomy.

Complications of Cholecystectomy ™™ Complications can occur either in open method or in lapa-

roscopic 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, 10 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 fundus of gallbladder is held and pushed up towards the diaphragm. With middle 5 mm port grasper is passed to hold Hartmann’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

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 ¾¾ Bile duct injury—0.8%; bleeding; bile leak. ¾¾ Infection, cholangitis, septicaemia; subphrenic abscess formation. ¾¾ Injury to colon, duodenum, mesentery. ¾¾ ¾¾

A

B

Figs. 12.72A and B: Single incision laparoscopic surgery (SILS) for cholecystectomy. ™™ Advantages: There is no visible scar like a traditional multiport;

faster recovery time, early return to work; cosmetically better.

™™ Disadvantages: Expensive trocars and instruments—cost

Fig. 12.71: Post-cholecystectomy bile leak with fistula. It is common and problematic complication after cholecystectomy.

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.

CHAPTER 12    Gallbladder

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.

SILS 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 10 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.

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Note: Waltman-Walter syndrome: It is compression of IVC due to collection of fluid in subphrenic space during post-cholecystectomy period. It mimics coronary thrombosis.

BILE DUCT INJURIES Causes ™™ Cholecystectomy—open or laparoscopic. ™™ Trauma; instrumentation—ERCP, baby endoscope, chole­

do­choscope. ™™ Other surgeries—pancreatic, duodenal, gastric. ™™ Anomalous biliary system increases the risk of injury.

Classifications

B

Strasberg classification

Class A: Bile leak from the cystic duct or an accessory duct showing continuity with the common bile duct xx Class B: Section/injury of an accessory duct/sectorial ducts aberrant right hepatic duct with no continuity with the common bile duct with occlusion xx Class C: Leak/open drainage from a sectorial duct/aberrant right hepatic duct with no continuity with the common bile duct xx 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 xx 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 –– E5 – Complete obstruction of entire bile duct Note: Only right and left partial injuries are not included in this classification xx

Note: Other classifications used are:

• • •

Stewart—Way classification: Class I: Incomplete. Class II: Lateral with bile leak and stenosis. Class III: 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. ¾¾ 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 ¾¾ Biliary fistula with electrolyte imbalance. ¾¾ Biliary peritonitis, septicaemia. ¾¾

Subphrenic abscess formation. Biloma formation. Severe malnutrition, deficiencies. Late—biliary cirrhosis, portal hypertension. ™™ Investigations ¾¾ LFT, coagulation profile. ¾¾ US abdomen, fistulogram. ¾¾ Dynamic CT scan will delineate the anatomy of injury. ¾¾ ERCP is useful if injury is only partial where stenting can also be done. ¾¾ PTC is the investigation of choice to identify site, nature, extent of stricture. It also facilitates drainage and stenting. ¾¾ Hepatobiliary scintigraphy may be useful. ¾¾ MRCP may be useful to identify ductal anatomy. ¾¾ CT arteriography is useful to identify associated hepatic artery or portal vein injury which is 20% in association with CBD injury. ¾¾ ¾¾ ¾¾ ¾¾

Management ™™ General: antibiotics, nutrition, TPN. ™™ Conservative: Small partial injury to CBD may resolve spon-

taneously 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. Intraoperative 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 cholecystec­

tomy 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

T

Causes of 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 of Oddi dyskinesia

GIT

Oesophagitis / hiatus hernia/ achalasia cardia Duodenal diverticula IBS, constipation, incisional hernia Mesenteric ischaemia

Cardiac

Coronary heart disease

Others

Adrenal tumour Thyrotoxicosis Intercostal 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.

Gallbladder dyskinesia: ¾¾ Here CT scan, gastroscopy, ERCP are normal. CCK is injected intravenously after filling gallbladder with radionuclide labeled Tc99m, gallbladder ejection fraction is assessed after 20 minutes. If it is less than 35%, then it is called as gallbladder dyskinesia. ¾¾ It is treated with laparoscopic cholecystectomy. ™™ Sphincter of Oddi dysfunction: ¾¾ 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. ¾¾ It may be due to trauma, congenital anomalies. ¾¾ In occurs in 1% of cholecystectomy patients. Condition is common in females. ¾¾ 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. ¾¾ 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 II: Unexplained biliary pain for more than 6 months after cholecystectomy + with any one of the other criteria. Type III: Only unexplained biliary pain for more than 6 months after cholecystectomy, without any of other criteria. ¾¾ ERCP/MRCP/sphincter of Oddi manometry, IV cholangiography are the investigations. ¾¾ 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.

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CHAPTER 12    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.

It can be:

13

chapter

Spleen C hapter Outline ·· ·· ·· ·· ·· ·· ·· ·· ·· ··

Surgical Anatomy Functions of the Spleen Splenunculi Splenic Injury Atraumatic Rupture of Spleen Splenomegaly Hereditary Spherocytosis Immune Haemolytic Anaemia Thalassaemia Sickle Cell Disease

·· Idiopathic Thrombocytopaenic Purpura ·· Thrombotic Thrombocytopaenic Purpura ·· Splenectomy ·· Overwhelming Postsplenectomy Infection ·· Splenic Artery Aneurysm ·· Splenic Abscess ·· Hypersplenism ·· Splenic Cyst

SURGICAL ANATOMY ™™ It is a wedge-shaped organ lying mainly in left hypochon-

drium, along the long axis of 10th rib. ™™ Hilum of spleen transmits splenic vessels and nerves. The

visceral surface is related to stomach, splenic flexure of colon, kidney. ™™ Spleen in English means “ill temper”. Galen called it as organ of mystery. ™™ It weighs 100–250 gram; it is 10 × 7 × 3 cm in dimension.

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.

Blood Supply Splenic artery is the branch of coeliac artery but may arise from aorta or superior mesenteric artery, blood flow is 300 ml/mt. 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. Immunoglobulins 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 branches which open into these sinuses and cords wherein particles are phagocytosed. Commonly central artery eventually ends in these cords and sinuses. Few end branches of the central artery directly enters the pulp vein. Cords and sinuses eventually drain into pulp vein. Blood circulating through these cords and sinuses are called as ‘open’ circulation (90%). Blood passing through white pulp but not entering these cords and sinuses are called as ‘closed’ circulation (10%). Spleen is palpated under left costal margin during inspiration. It has to enlarge 2–3 times its normal size to become palpable.

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/HowellJolly 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

™™ These are single or multiple accessory spleens.

B xx xx xx xx

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 sple-

Sites

Hilum of spleen (50%); Near splenic vessels Tail of the pancreas (30%) Splenic ligaments—gastrosplenic/splenorenal Mesocolon; Greater omentum.

Fig. 13.2: On table photo of splenunculi with splenomegaly. Splenunculi should be removed otherwise disease will recur.

nomegaly 80% of platelets may be sequestered in spleen causing thrombocytopenia. Normally spleen is reservoir for platelets. 1/3rd 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).

™™ After splenectomy, they undergo hyperplasia and lead to

2.  Immunologic functions like synthesis of antibody IgM; formation of lymphocytes; production of tuftsin, opsonins, properdin and interferons.

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].

B xx

xx

xx xx xx

xx

Functions of spleen

Response to antigenic challenge—by secreting antibodies like IgM, tuftin, opsonins, properdin, interferons. These agents make bacteria and other organisms more vulnerable to phagocytosis. 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.

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recurrence of the disorder 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 splenec-

tomy while doing for idiopathic thrombocytopenic purpura (ITP) or haemolytic disorders.

SPLENIC INJURY (Rupture spleen)

Causes ™™ Splenic injury occurs commonly following road traffic acci-

dents, other blunt injury or penetrating/stab injuries.

™™ Most often associated with fracture of left lower ribs, haemo-

thorax, 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 spleno­m egaly, 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

CHAPTER 13    Spleen

“culling”. RBCs which loose osmotic balance and membrane integrity is called as non-deformable and they are removed by this method.

SPLENUNCULI (30%)

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Types of Injury 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).

B xx xx xx xx xx

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

Presentation Hilar injury presents with rapid development of shock and deteriorates fast. Even death can occur some­times. Here emergency surgery and splenectomy is mandatory. ™™ In other types, features of shock (pallor, tachy­cardia, restlessness, hypotension), pain, tenderness and abdominal rigidity in left upper quadrant is seen. ™™ Later there will be abdominal distension due to haemoperitoneum. ™™ Dullness in the left flank which does not shift, as the collected blood gets clotted. Dullness without shifting—Ballance’s sign.

A

B

C

D

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 Bandet’. ™™ Pseudoaneurysm and traumatic splenic arteriovenous fistula formation can also occasionally cause delayed life-threatening haemorrhage. ™™ Features of other abdominal organ injuries may be present.

B xx xx xx xx

Signs in splenic rupture

Kehr’s sign—pain in left shoulder 15 minutes after foot end elevation Ballance’s sign—left-sided abdominal dullness which will not shift Saegesser’s tender point between left sternomastoid and scalenus medius Latent period of Bandet—refer above

A

B

Figs. 13.5A and B: Splenic injury with splenic haematoma. Fig. 13.3: Large subcapsular splenic haematoma. ™™ 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

Note: Splenosis: Autotransplantation of fragments of splenic tissue may occur within the peritoneal cavity following rupture of spleen.

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 will show type of splenic injury and its class. CT

Grade II

xx xx xx xx xx xx

Non-expanding subcapsular haematoma 10–50% surface area. Non-expanding intraparenchymal haematoma 50% surface area or ruptured bleeding subcapsular haematoma/ intraparenchymal haematoma. Intraparenchymal haematoma >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: ¾¾ Gross blood of 10 ml. ¾¾ >1,00,000/mm3 of RBC. ¾¾ >500/mm3 of WBC. ¾¾ Bile, bacteria or food fibres. ¾¾ 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

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 A

™™ When facilities are available, splenic injury can be managed

B

Figs. 13.6A and B: Lacerated injury over the spleen. Patient underwent splenectomy. ™™ Angiogram, for diagnosis and often for angiographic embo-

lisation to reduce the bleeding and to manage splenic injury non-operatively is also becoming routine in many centres.

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 130/minute; arrhythmias, ECG changes. Pulmonary: PaO2 >60 mm Hg; ARDS. Renal: Urine output 5000 units/mL).

Nealon and Walser classification

I – Normal duct; no duct communication into pseudocyst. II – Normal duct with communication. III – Normal duct with stricture without communication. IV – Normal duct with stricture with communication. V – Normal duct with abrupt duct termination. VI – Chronic pancreatitis without duct cyst communication. VII – 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.

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 felt per abdomi­n ally (Baid test). Fig. 14.10: Pseudocyst of pancreas.

Types

™™ Aortic aneurysm; Retroperitoneal cyst or tumour.

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.

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xx xx xx xx

D’Egidio classification of pseudocyst

Type I: After an attack of acute pancreatitis. Normal duct anatomy; no fistula/communication. Type II: After an attack of acute on chronic pancreatitis. Abnormal duct anatomy without stricture; 50% chances of fistula. Type III: After an attack of chronic pancreatitis, abnormal anatomy with stricture; always communicating. It appears like retention cyst.

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Differential Diagnosis

Sarle’s classification

Necrotic pseudocyst in acute pancreatitis. Retention cyst in chronic pancreatitis. Intrapancreatic retention due to pancreatic duct dilatation. Extrapancreatic retention cyst due to rupture into peripancreatic tissues.

™™ Cystadenocarcinoma of pancreas. ™™ Cyst of the liver; Mesenteric cyst; Hydatid cyst.

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. ™™ EUS-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.

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Fig. 14.11: Vertebrogastric angle.

2. Interventional: Needs ERCP to delineate ductal anatomy, pancreatic duct stones, mainly communication into the pseudocyst. It is Guided—Endoscopic/EUS/US-guided drainage ¾¾ 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. Confirmation of resolution of pseudocyst is done by follow-up CT. This procedure is suitable only in communicating pseudocyst; it is safer and effective. ¾¾ Transmural endoscopic or EUS-guided stent 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. ¾¾ 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. ¾¾ 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.

B xx xx

Fig. 14.12: CT scan showing typical pseudocyst of pancreas.

xx xx xx

Indications for surgery/intervention

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

Surgery ™™ Cystogastrostomy: On laparotomy, anterior wall and poste-

A

rior 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.

B

Figs. 14.13A and B: CT scan showing pseudocyst of pancreas both single and multiple. Note the cyst cavity and the thick cyst wall. Patient needs drainage procedure either cystogastrostomy or cystojejunostomy.

B xx xx xx xx xx xx xx

Complications of pseudocyst

Rupture—3%—into bowel or peritoneum Infection, commonest—20%, abscess Bleeding from the splenic vessels—7% Cholangitis Duodenal obstruction Portal/splenic vein thrombosis and segmental portal hypertension Cholestasis due to CBD block

Treatment 1. Conservative: observation, follow-up using repeat USG at regular intervals. 50% of pseudocysts show spontaneous resolution.

A

B

Figs. 14.14A and B: Pseudocyst of pancreas aspiration of table. Note the brownish-black colour of the fluid aspirated.

• •

A

•

B

Figs. 14.15A and B: (A) Cystogastrostomy for pseudocyst of the pancreas; (B) 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.

• • • •

CHRONIC PANCREATITIS ™™ It is persistent progressive irreversible damage of the

pancreas due to chronic inflammation. It can be   Chronic relapsing pancreatitis Chronic pancreatitis (persistent)   Chronic non-calcifying Calcifying pancreatitis 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.

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•

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.

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Fig. 14.18: Pancreatic ductal stone removed—specimen. Patient underwent pancreaticojejunostomy.

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Aetiology

Alcohol—80% main cause Stones in biliary tree—rare cause Malnutrition, diet Hyperparathyroidism Hereditary (familial hereditary pancreatitis)—Autosomal dominant Idiopathic—20%—as mutation Trauma; Congenital anomaly (Pancreatic divisum) Cystic fibrosis Autoimmune pancreatitis Hyperlipidaemia

B

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 Sjögren/Crohn’s disease. R – Recurrent and severe acute/ischemic. O – Obstructive—pancreas divisum/annular pancreas/stenotic papilla/duodenal obstruction/trauma/pancreatic ductal stones/ choledochocele. ™™ 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 abdominal 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.

Pathology ™™ It shows atrophy of acini, hyperplasia of duct epithelium,

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Pathology of chronic pancreatitis

Focal necrosis Segmental or diffuse fibrosis Parenchymal calcification or ductal stones Stricture and/or dilatation of the duct

Spectrum of Chronic Pancreatitis ™™ Early—pancreatic oedema—chronic inflammation—normal

secretory function. ™™ Moderate—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 CTGF and TGF-B1 that stimulate extracellular matrix. Pain hypothesis include: Acute and chronic inflammation of the pancreas. xx Increased pressure within the pancreatic ductal system and parenchyma, ductal dilatation, stasis. xx Ischaemia of the parenchyma secondary to increased interstitial pressure. xx Over expression of a particular protein in pancreatic nerve fibres. xx Pancreatic nerve growth factor released by degenerating acinar cells and its high affinity receptor that promotes nerve growth and repair. xx Pain may be due to perineural sheath destruction by toxins, ischaemia and also due to tissue acidosis. xx

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 Büchler 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-

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 C can 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 C is accepted under the following conditions—Patients with clinical manifestation of end-stage functional impairment with or without complications.

Clinical Features ™™ Pain in epigastric region (80%) ¾¾ ¾¾

¾¾

¾¾ ¾¾

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 B 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

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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.

crine function is preserved. Subclinical signs (impaired glucose tolerance, reduced exocrine function but without steatorrhoea) might already be apparent.

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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.

B

xx xx

xx xx xx

Investigations

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

Investigations presently used are: ™™ CT scan abdomen: It is 95% reliable; to see pseudocyst,

calcification, ductal stones, duct stricture and dilatation, vasculature, fibrosis, surrounding structures, CBD status. It shows 90% sensitivity; 95% specificity.

Triad of chronic pancreatitis

(1) Pancreatic calcification; (2) Steatorrhoea; (3) Diabetes mellitus Triad is found in less than one-third of patients with CP

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.

B xx xx

xx

Clinical presentations

A

Stage A: 85%, recurrent/acute episodic pain with weight loss. Stage B: 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.

Differential Diagnosis ™™ Carcinoma of head of the pancreas. ™™ Retroperitoneal tumour.

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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

B

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

™™ Secretin cholecystokinin test is the gold standard for

Cambridge grading of CP on ERCP Main pancreatic duct

Side branches

I: Normal

Normal

Normal

II: Equivocal

Normal

3 Abnormal

IV: Moderate

Abnormal

>3 Abnormal

V: Severe

Abnormal with at least one of the following:

>3 Abnormal

• 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

Rosemont EUS criteria Major: • A—Hyperechoic foci with shadowing and calculi in main pancreatic duct (PD) • B—Lobularity with honeycombing Minor: • Cysts; Dilated ducts ≥3.5 mm; Irregular PD contour; Dilated side branches ≥1 mm; Hyperechoic duct wall, strands; Non-shadowing hyperechoic foci; Lobularity with non-contiguous lobules

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, HCO3, amylase, lipase and proteases. It is to assess functional deficiency. In chronic pancreatitis volume is normal— >2 ml/kg but less bicarbonate content — 150 pg/mL Common in body and tail—solitary Distal pancreatectomy is the treatment of choice Prednisolone controls the diarrhoea Octreotide is useful

ZOLLINGER-ELLISON SYNDROME Type I: “G” cell hyperplasia with hypergastrinaemia and chronic peptic ulceration. Treatment is partial gastrectomy with removal of “G” cell area. TYPE II: Gastrinomas (see above). Note:

•

• •

Somatostatinoma arises from δ cells of pancreas. It is exceedingly rare. Usually larger than 2 cm and single. It is common in head of pancreas and duodenum. Its association with MEN II syndrome is rare. It is often associated with neurofibromatosis type I and phaeochromocytoma. Presentations are steatorrhoea; diabetes mellitus; gallstones; hypochlorhydria and occasional jaundice. Tumour is commonly malignant and commonly present with nodal and liver spread. Elevated fasting somatostatin level is diagnostic. Treatment is pancreaticoduodenectomy. 20% of pancreatic endocrine tumours are nonfunctional. Nonfunctional means these tumours do not produce hormones or produced hormones will not produce any symptoms. Pancreatic polypeptide tumour (PP tumour) is classified under the non-functioning endocrine tumour as it does not cause any symptoms even though there is high pancreatic polypeptide. It is commonly malignant, often with spread. PPoma occurs predominantly in head of pancreas. Pancreaticoduodenectomy is the treatment.

CYSTIC FIBROSIS It is inherited as an autosomal recessive disease. It is common in Caucasians. It is inherited as mutation in cystic fibrosis transmembrane conductance regulator gene in chromosome. It causes: 1. Severe exocrine dysfunction—due to blockage of pan­creatic ducts as a result of precipitation of pancreatic enzymes leading to duct ectasia. 2. Chronic pulmonary disease due to blockage of bronchi and bronchioles. 3. Elevated sodium and chloride—in the sweat more than 90 mmol/L.

Presentations ™™ Chronic pulmonary disease with emphysema, bronchiectasis. ™™ Steatorrhoea. ™™ Meconium ileus with intestinal obstruction. ™™ Salty sweat (while kissing the baby). ™™ Poor growth due to malabsorption. ™™ Cirrhosis of liver due to bile plugging. ™™ Pancreatitis is common. ™™ Sialadenitis, choroiditis can occur. ™™ Absence of vas deferens in men.

Diagnosis ™™ DNA study. ™™ Vomitus when put on an exposed X-ray film will not digest the

gelatin in half an hour (wherein in normal individual vomitus contains trypsin which will digest the gelatin of X-ray film and turns white). ™™ US abdomen. ™™ Sweat test for sodium and chloride. ™™ Pulmonary function tests.

Treatment ™™ Low fat and salt rich diet; Correction of malabsorption. ™™ Pancreatic enzyme supplements. ™™ Antibiotics; Respiratory physiotherapy. ™™ If meconium ileus is present, it might require surgery, i.e.

Bishop-Koop operation.

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Problems in cystic fibrosis

Meconium ileus Recurrent respiratory infection and complications Sodium deficit; Severe malnutrition Sialadenitis, choroiditis Cirrhosis of liver Usually die in childhood or early age group Prognosis is poor.

ANNULAR PANCREAS ™™ It is due to failure of complete rotation of ventral bud of

pancreas, so that ring of pancreatic tissue encircles the 2nd part of the duodenum causing obstruction. It contains a duct that joins the main pancreatic duct.

™™ Distension of upper abdomen; Bilious vomiting; Visible

™™ Chronic pancreatitis commonly due to dietary factors, familial,

hyperparathyroidism.

™™ Commonly they are multiple and associated with or due to

chronic pancreatitis.

™™ It is due to stricture of pancreatic duct and stasis of pancre-

atic secretion.

™™ Investigations ¾¾ ¾¾ ¾¾

Plain X-ray abdomen shows double-bubble appearance. ERCP and Radioisotope study. Barium meal shows obstruction at 2nd part of duo­denum.

™™ Treatment ¾¾ ¾¾

Duodenoduodenostomy—ideal. Duodenojejunostomy.

A

Note:

•

Do not resect the ring. Attempt at resection will lead to pancreatic fistula; G-J is not done.

ECTOPIC (ACCESSORY) PANCREATIC TISSUE

B xx xx xx

Sites

Stomach wall. Small intestine. Meckel’s diverticulum.

xx xx

Greater omentum. Splenic hilum.

™™ In the bowel, it may be in the submucous or intra­muscular

B

plane. It may precipitate intussusception. ™™ About 30–33% contain islet of Langerhan cells. Often endocrine

pancreatic tumour can arise from ectopic pancreas. ™™ It may cause upper GI bleed. C

PANCREATIC DIVISUM ™™ During development ventral pancreatic bud with distal bile

duct is towards right and dorsal pancreatic bud is towards left side. Ventral pancreatic bud forms uncinate process and inferior part of the head of the pancreas. Dorsal bud forms body and tail of the pancreas. Ventral bud rotates clockwise towards left from behind the duodenum to join dorsal bud to form pancreas. Ventral pancreatic bud duct forms main pancreatic duct of Wirsung; dorsal bud duct forms accessory pancreatic duct of Santorini. ™™ Pancreatic divisum is failure of fusion of ventral and dorsal pancreatic ducts. So that dorsal pancreas drains through duct of Santorini (proximal minor papilla); ventral one drains through duct of Wirsung into major papilla of Vater. ™™ Its incidence is 10%. ™™ Here major pancreatic secretion drains through minor papilla causing partial obstruction precipitating pancreatitis.

Figs. 14.53A to C: (A) X-ray showing multiple pancreatic ductal stones; (B and C) CT scan picture showing pancreatic ductal stones. Stones that are removed from the same patient are also shown. Patient underwent longitudinal pancreaticojejunostomy.

Types a. Calculi in the duct: ¾¾ Here parenchyma is still functioning and less severely affected. ¾¾ Duct is dilated significantly (more than 3 mm) with multiple large stones in the duct along with multiple strictures. ¾¾ Duct is having dilatation alternating with strictures— chain of lake appearance. –– Plain X-ray abdomen shows stones placed horizontally at L1-L2 level. –– US, ERCP, MRCP are other useful investi­gations. –– Blood sugar should be checked.

Those who wish to sing always finds a song.

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gastric peristalsis; It is often associated with the Down’s syndrome, and other congenital gut abnormalities. ™™ Types ¾¾ Neonatal type—produces symptoms of intestinal obstruction. ¾¾ Adult type—presents after the age of 20. Presents with features of duodenal ulcer and bilious vomiting. ¾¾ Differential Diagnosis; Duodenal atresia; Wilkie's syndrome.

PANCREATIC CALCULUS Causes

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Treatment The duct is opened longi­tudinally and after removing all stones, side-to-side pancreaticojejunostomy is done. b. Calculi in the Parenchyma ¾¾ Here multiple calcifications occur in the pan­creatic paren­ chyma with pre-existing chronic pancreatitis. Gland is fibrosed and severely affected with less functioning capacity. ¾¾ Plain X-ray, US, ERCP, MRCP are the diagnostic methods. ¾¾ It is more likely to turn into carcinoma pancreas. Treatment ¾¾ Total pancreatectomy.

PANCREATIC ASCITES ™™ It can occur in chronic pancreatitis or as a complication of

acute pancreatitis or in ruptured pseudocyst of pancreas or after pancreatic trauma (10%, in children trauma is the cause). ™™ It may be associated with pleural effusion which can be both sided but commonly on left side. Often pleural effusion and ascites may be massive. It is due to internal pancreatic fistula. ™™ Incidence is 1%. If not treated mortality is 30%. ™™ It is due to leakage from disrupted pancreatic duct or ruptured pseudocyst. Anterior disruption causes pancreatic ascites. Posterior disruption causes fluid to travel along oesophageal and aortic hiatus resulting in pleural effusion. ™™ Pancreatic ascites and fistula are common in pancreatitis due to alcohol intake. It is less common in pancreatitis due to biliary tract disease. ™™ US, very high amylase and protein levels in ascites fluid, ERCP to identify leak, CT scan are the essential investigations. ™™ Repeated ascitic tap; pleural tap; TPN; somatostatin or octreotide to reduce pancreatic secretion are tried as conservative therapy for 3 weeks. 50% respond to this therapy. ™™ If there is no improvement beyond 3 weeks, ERCP stenting of pancreatic duct is done initially. Then resection (better option) or drainage (jejunal loop Roux-en-Y anastomosis directly over disrupted duct) surgery is done. Pancreatic pleural effusion disappears once abdominal cause or ascites is corrected.

PANCREATIC FISTULAE Types: External and internal fistulae.

External Fistulae Types a. Low output fistulae 200 ml. (Note: For other GI fistulae it is 500 ml) Present ISGPF (International study group of pancreatic fistula) grading system is : Grade 0,A,B,C using various parameters like amylase level of fluid, clinical features, imaging etc. Causes Whipple’s operation; Pancreaticojejunostomy; Splenectomy; Colonic surgeries; Trauma; After external drainage of the infected

pseudocyst of pancreas; After percutaneous drainage of the pseudocyst.

B xx xx xx

Clinical features

Patient is in a catabolic state Skin excoriation Electrolyte imbalance

xx xx

Malnutrition Sepsis.

™™ Investigations CT scan; ERCP; Fistulogram; Amylase estimation of the discharge. ™™ Treatment Conservative treatment: Total parenteral nutrition; Zinc oxide cream for skin excoriation; Correction of electrolyte imbalance; Antibiotics; Octreotide. ™™ Surgery: Roux-en-Y anastomosis; Resection of fistula with pancreas; Endoscopic stenting of the pancreatic duct.

Internal Fistulae ™™ Communicating pseudocyst. ™™ Pancreatic ascites. ™™ Pancreatic pleural effusion. ™™ Pancreatic-enteric fistulae.

B xx xx xx

Complications of pancreatic fistulae

Septicaemia Malnutrition Bleeding due to erosion of vessels

xx xx

Electrolyte imbalance Severe skin excoriation

PANCREATIC NECROSIS It is diffuse or focal area of non-viable parenchyma of pancreas, occurs during an attack of severe pancreatitis that is associated with peripancreatic fat necrosis. It occurs in 20% of acute pancreatitis. ™™ Types ¾¾ Sterile necrosis is 60% of total pancreatic necrosis. Mortality here is 10%. ¾¾ Infected necrosis is 40% of total necrosis. Mortality here is 30–40%, very high. Risk of infection is 25% in one week of acute attack; 35% at the end of 2nd week; and 70% at the end of 3rd week. ¾¾ Necrosis extends to retroperitoneal fat, mesentery, retrocolic and perinephric areas. ™™ Pathogenesis: Premature activation of proteolytic enzymes; Intrapancreatic vessel thrombosis; Pancreatic microcirculation failure; Release of inflammatory mediators.

B xx xx xx

Mode of infection

Haematogenous; Reflux through ampulla Mucosal translocation of bacteria; From the biliary tree Lymphatic route; Transperitoneal spread.

™™ Bacteria ¾¾ ¾¾

65% are polymicrobial; E. coli is commonest. Others are—Proteus, Pseudomonas, Klebsiella, staphylococci, Streptococcus faecalis, enterococci, anaerobes, Clostridium welchii.

™™ Diagnosis ¾¾ ¾¾

Type I Class I injury to both organs/class I injury of one organ with class II injury to other Type II Class II injuries of both organs Type III Class III injury to one organ with less severe injury to other Type IV Class III injury to both organs

B

Grading of pancreatic injury

Grade I – Minor contusion/laceration without duct injury Grade II – Major contusion/laceration without duct injury Grade III – Distal transection or injury with duct involvement Grade IV – Proximal transection or injury with involving ampulla Grade V – Massive disruption of pancreatic head ™™ 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

Fig. 14.54: CT showing pancreatic trauma. ™™ Treatment

Antibiotics—imipenem, cefuroxime, ofloxacin, meropenem. Percutaneous drainage. ¾¾ Laparotomy, necrosectomy, debridement, saline wash, closure with tube irrigations/lavage. ™™ Complications ¾¾ Septicaemia, ARDS, renal failure, MODS. ¾¾ Pancreatic fistula after surgery; Intra-abdominal abscess. ¾¾ Abdominal dehiscence, incisional hernia after surgery. ¾¾

¾¾

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 ¾¾ ¾¾ ¾¾ ¾¾

B

Parenchymal contusion/laceration without duct disruption. Parenchymal injury with duct disruption. Complete transection of pancreas. Massive destruction of pancreatic head.

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: ¾¾ 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 xx xx xx xx

Classification

Pancreatic injury Class I Capsular damage; minor parenchymal injury Class II Transection of duct in body or tail—partial or complete Class III Major duct injury in pancreatic head or intrapancreatic CBD injury Duodenal injury Class I Contusion, haematoma, partial thickness injury Class II Full thickness duodenal injury Class III Full thickness duodenal injury with 1. More than 75% circumference injury 2. Extrapancreatic CBD injury Contd...

Complications

Haemorrhage Septicaemia Pancreatitis—acute/recurrent Pseudocyst formation

xx xx xx

Pancreatic fistula Pancreatic abscess Pancreatic duct stricture at the site of duct injury as a delayed complication.

CYSTIC LESIONS OF PANCREAS Types 1. True pancreatic cysts: Congenital; Not premalignant; Asymptomatic. ¾¾ May be associated with cystic diseases of liver, kidney. ¾¾ 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.

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CHAPTER 14    Pancreas

CT scan abdomen and CT-guided FNA for Gram’s staining and culture. CRP value— >120 mg/litre; interleukin level; elastase level; urinary TAP assessment.

Contd... Combined pancreatico duodenal injury

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von Hippel-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.

PANCREATIC EXOCRINE INSUFFICIENCY (EXOCRINE PANCREATIC DISEASE)

Indirect Methods

Causes Chronic pancreatitis, cystic fibrosis, pancreatic resections (total or partial), pancreatic duct obstruction by haemochromatosis or α1 antitrypsin deficiency, short gut syndrome, gastric resection, Shwachman Diamond syndrome (congenital entity with pancreatic exocrine insufficiency, cytopenias, skeletal abnormalities).

Features ™™ Maldigestion of fat and proteins causing steatorrhoea and

weight loss.

™™ Metabolic bone disease, impaired night vision, fat soluble

vitamin deficiencies can occur.

™™ Vitamin B12 deficiency can occur as decreased intestinal pH

prevents transfer of vitamin B12 to intrinsic factor from R factor; it shows positive dual label Schilling test.

Evaluation Direct Methods It is done by directly collecting the fluid from duodenum using double lumen collecting tubes, one is duodenum to collect pancreatic fluid and other being in stomach to aspirate gastric content so that to avoid it mixing with pancreatic fluid. ™™ Lundh test (Goran Lundh): Liquid test meal containing protein, fat and carbohydrate (300 ml) is given orally; duodenal fluid is collected for 2 hours; assessed for enzymes. Normally trypsin level is > 8 mEq/L. it is very low in pancreatic insufficiency. Test is obsolete now. ™™ Secretin test: Initial test dose of secretin is given 0.2 ug; then full dose of 0.2 ug/kg as a bolus IV dose is given; duodenal

These methods are measure of effects of pancreatic insufficiency. ™™ Serum trypsinogen and trypsin level estimation; trypsinogen level less than 20 ng/ml suggest advanced pancreatic exocrine insufficiency. ™™ Faecal fat estimation after collecting 72 hours faecal fat and estimation is done for daily fecal fat using Sudan stain. In a person ingesting 100 g of fat/day, 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 fat suggests pancreatic disease whereas split fat suggests small bowel disease). ™™ Pancreolauryl test: Fluorescein dilaurate is ingested along with breakfast which is a substrate for pancreatic enzyme cholesterol esterase; depending on the activity of the cholesterol esterase this substrate is cleaved to release proportionate fluorescein; released fluorescein rapidly absorbed from the gut and excreted in the urine. Serum fluorescein and 24-hour urine fluorescein assay gives the quantitative estimation of the pancreatic exocrine function. It is accurate and acceptable test for pancreatic exocrine insufficiency. ™™ Triolin breath test: Triolin labelled 13C or 14C is administered as test meal; it is hydrolyzed in the gut lumen depending on the quantity of pancreatic lipase activity; hydrolysed products are absorbed, metabolised and released through pulmonary endothelium as 13CO2/14CO2 which is measured using mass spectrometry or infrared. It is mainly used to quantify steatorrhoea.

Treatment of Pancreatic Exocrine Insufficiency Low fat diet; pancreatic enteric coated tablets during meals, treatment of cause.

15

chapter

Retroperitoneal Space C hapter Outline ·· Anatomy of Retroperitoneum ·· Retroperitoneal Fibrosis ·· Retroperitoneal Swellings

·· Retroperitoneal Tumours ·· Psoas Abscess (Retroperitoneal Abscess)

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, quadratus lumborum muscle and tendinous portion of transversus abdominis muscles; superiorly by diaphragm; inferiorly by pelvic levator muscles (by Ackerman). It is real potential space.

B xx xx xx xx xx xx xx

Diseases of retroperitoneal spaces

Retroperitoneal fibrosis Retroperitoneal cysts Retroperitoneal tumours Retroperitoneal lymphomas Retroperitoneal vascular diseases, e.g. aneurysms Retroperitoneal trauma and haematoma Retroperitoneal infection, e.g. psoas abscess, pyogenic abscess

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 1/3rd 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.

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™™ It can also cause vascular compression, both venous and

arterial. ™™ Disease is progressive one. ™™ Investigation: ¾¾ Ultrasound ¾¾ IVU—diagnostic:

B xx xx xx

Triad in IVU

Deviation of middle 1/3rd of ureter medially Hydroureteronephrosis Extrinsic ureteral compression

Blood urea and serum creatinine, raised ESR and CRP ¾¾ CT scan. ™™ Treatment ¾¾ Drug therapy: Methylprednisolone, azathioprine, penicillamine, tamoxifen. ¾¾ Cystoscopic stenting of the ureters to prevent renal failure. ¾¾ Symptomatic treatment: If stenting fails, bilateral nephrostomy is done. ¾¾ Ureterolysis. ¾¾ Lateral repositioning of ureters. ¾¾ Omental wrap after ureterolysis. ¾¾ Vascular bypass graft for vascular encasement. ¾¾

B xx xx xx xx xx xx xx

RETROPERITONEAL SWELLINGS Types of retroperitoneal (RP) tumours (0.3–3%)

Benign—20% of RP tumours xx Retroperitoneal lipoma xx Retroperitoneal neurofibroma, neurilemmoma xx Retroperitoneal leiomyoma xx Extra-adrenal chromaffinomas xx Paraganglioma xx Mucinous cystadenoma xx Haemangiopericytoma Malignant—80% of RP tumours Retroperitoneal liposarcoma, leiomyosarcoma—50% xx Retroperitoneal lymphoma (commonly NHL) xx Malignant tumours from specific organs xx Germ cell tumours, chordomas xx RPLN secondaries with hard nodules xx

• •

• •

Other causes for retroperitoneal fibrosis (30%)

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

B

Note: Tumours of retroperitoneal organs like kidneys, ureters, pancreas, and adrenals are conventionally not included in retroperitoneal tumours. Paragangliomas are of neural crest origin that 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 von 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.

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 IVC also causing IVC obstruction with features of bilateral oedema feet, dilated lateral abdominal wall vein with upward direction flow of blood.

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). ¾¾ ¾¾ ¾¾ ¾¾ ¾¾ ¾¾

In retroperitoneal cyst and sarcoma the swelling is smooth. In lymphoma it is smooth and firm. In aneurysm it is pulsatile (expansile pulsation) 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.

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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.

A

Fig. 15.6: Retroperitoneal tumour, on table finding. It was large and inoperable.

Aetiology ™™ Retroperitoneal tumors may be associated with radiation B

Figs. 15.4A and B: Retroperitoneal tumour—benign lipoma was excised with a loin retroperitoneal approach.

exposures to chemicals like vinyl chloride/thorium dioxide, familial Gardner’s syndrome, 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.

CHAPTER 15    Retroperitoneal Space

Fig. 15.3: Typical look of retroperitoneal tumour.

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Features

Investigations

SRB's Manual of Surgery

™™ Tumor arises from fat, areolar tissue, vessels, nerves, skel-

etal 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 undiff­ erentiated (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.

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.

A

B

C

Figs. 15.7A to C: Retroperitoneal tumour presenting as large mass abdomen with all features. CT pictures of the same patient.

™™ 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 hetero­ genous 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) Tx: Cannot be assessed T0: No tumour T1: 5 cm or less T2: 5–10 cm T3: 10–15 cm T4: >15 cm

Metastasis (M) cM0: No distant metastases cM1: Distant spread present pM1: Microscopic distant spread

Nodal (N) N0: No regional nodes N1: Nodes present

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 N0 M0 G1/GX; Stage IB: T2, 3, 4 N0 M0 G1/GX; Stage II: T1 N0 M0 G2/G3; Stage IIIA: T2 N0 M0 G2/G3: Stage IIIB: T3, 4 N0 Mo G2/G3; any T N1 M0 any G; Stage IV: Any T any N M1 any G.

713

Treatment ™™ Surgery is the main modality of therapy with wide excision—

Prognosis ¾¾ ¾¾ ¾¾ ¾¾ ¾¾ ¾¾ ¾¾ ¾¾ ¾¾

Fig. 15.9B

Figs. 15.9A and B: Retroperitoneal lymph node mass. It could be tuberculosis/lymphoma/secondaries/autoimmune lymph node enlargement.

B xx xx xx xx xx xx xx xx

Tumour grade—low or high; Resectability; Invasion into organs, veins, nerves; Size of tumour 5 cm or >10 cm; 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%; 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.

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CHAPTER 17    Abdominal Wall and Umbilicus

™™ Wide excision of the tumour with a margin of 2.5 cm is done

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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.

Fig. 17.20: Diagram showing differences between exomphalos minor and exomphalos major. ™™ As there is hardly 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 carefully. Gradual twisting of the bag over the summit at regular intervals over two to three weeks will 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.

B xx xx xx xx

xx xx xx xx xx

Management of exomphalos

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

A

B

Figs. 17.21A and B: Gastroschisis.

Treatment

B xx xx xx

General

Fluid management TPN Antibiotics

xx xx

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

1. Defect lateral to umbilicus

2. Covered by sac which has three layers

2. No peritoneal sac

3. Associated with anomalies

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

RECTUS SHEATH HAEMATOMA

xx xx xx

™™ 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 xx xx xx xx xx xx xx

737

Causes

Infected haematoma Umbilical sepsis spreading into the abdominal layers causing the abscess Blood spread from distant focus.

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

Causes

Trauma; Surgery Spontaneous haematoma, typhoid fever Blood dyscrasias, haemophila, anticoagulant therapy Severe straining and exercises Tetanus and other convulsions Patients on anticoagulants Puerperium.

abscess, parietal hernia. ™™ Ultrasound and needle aspiration is confirmative. ™™ Treatment: It is antibiotics and drainage under general

anaesthesia.

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. ™™ Bluish 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 ¾¾ ¾¾ ¾¾

Usually conservative with analgesics and antibiotics. Angiographic embolisation of inferior epigastric artery. Occasionally requires drainage of haematoma and ligation of inferior epigastric artery.

A

B

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, staphy-

lococci and other anaerobes of the postoperative abdominal or thoracic wounds.

ABDOMINAL WALL ABSCESS

A

B

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.

CHAPTER 17    Abdominal Wall and Umbilicus

these patients.

B

738

™™ It is common in HIV, diabetic and immunosuppressed people.

SRB's Manual of Surgery

™™ 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 divari-

cated 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

18 Hernia

C hapter Outline ·· ·· ·· ··

Aetiology Parts of Hernia Classification of Hernia Inguinal Hernia xx Surgical Anatomy of Inguinal Canal xx Classification of Inguinal Hernia xx Indirect (Oblique) Inguinal Hernia xx Direct Inguinal Hernia xx Recurrent Hernia xx Hernioplasty xx Incarcerated Hernia ·· Strangulated Hernia ·· Sliding Groin/Inguinal Hernia

·· ·· ·· ·· ·· ·· ·· ·· ·· ·· ·· ·· ··

Pantaloon Hernia Femoral Hernia Ventral Hernia Incisional 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 through 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 inguinal hernia in occurrence–15%. ™™ Groin hernia is 25 times more common in men than women. Indirect inguinal hernia is commonest hernia in men and women. Femoral hernia s more common in females (10:1); umbilical and incisional hernias are also common in females (2:1).

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 indirect/direct/femoral 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.

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

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A

B

C

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 ™™ 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. ¾¾ Very young age—phimosis, meatal stenosis. ™™ Obesity. ¾¾ ¾¾

Fig. 18.4: Incisional hernia; common in lower abdomen.

™™ Pregnancy and pelvic anatomy (especially in femoral hernia

in females).

direct sac. Body of the sac is thin in infants, children and in indirect sac, but is thick in direct and long-standing hernia.

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™™ Smoking. ™™ Appendicectomy through McBurney’s incision may injure the

ilioinguinal nerve causing right sided direct inguinal hernia.

Fig. 18.8: Diagram showing the differences between indirect and direct sacs.

Hernia without neck: Those hernias with larger mouth lack neck, e.g. direct hernia, incisional hernia. ¾¾ 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. ¾¾

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.

 

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 fundus. Neck is narrow in indirect sac but wide in

Contents of Sac ¾¾ Omentum—Omentocele (Epiplocele). Difficult to reduce the sac later, initially it can be reduced easily. ¾¾ Intestine—Enterocele—commonly small bowel, but sometimes even large bowel. ¾¾ Difficult to reduce the sac initially. ¾¾ Richter’s hernia: A portion of circumference of bowel is the content. ¾¾ Urinary bladder may be the content or part of the posterior wall of the sac—cystocele. ¾¾ Ovary, often with fallopian tube. ¾¾ Meckel’s diverticulum—Littre’s hernia. ¾¾ Appendix in inguinal hernial sac which is often incarcerated—Amyand’s hernia. ¾¾ 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. • Herniography (by Gullmo): 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.

CHAPTER 18    Hernia

™™ Ascites.

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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 the affected 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 Classification I (Clinical) A

B

Figs. 18.9A and B: Hernial sac containing small bowel (enterocele) and omentum (omentocele) in two different patients.

1.  Reducible hernia Hernia gets reduced on its own or by the patient or by the surgeon. 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.

Fig. 18.12: Inguinal hernia presenting with features 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.

Classification III: According to the Contents xx xx xx

xx xx xx

Classification IV: Based on Sites � � xx xx

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.

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 It is secondary to any causes which raise the intra-abdominal pressure leading into weakening of the area like in direct inguinal hernia.

Inguinal hernia Femoral hernia Obturator hernia Diaphragmatic hernia

Lumbar hernia Spigelian hernia �  Umbilical hernia �  Epigastric hernia � 

INGUINAL HERNIA

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’.

B xx xx

xx xx xx xx xx xx xx

Contents of inguinal canal

Spermatic cord in males Round ligament in females

B

Fig. 18.14: Inguinal hernia on right side in a child. It needs only herniotomy.

� 

xx

Ilioinguinal nerve

Contents of spermatic cord

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

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

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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.

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Coverings of Spermatic Cord ™™ Internal spermatic fascia from fascia transversalis.

SRB's Manual of Surgery

™™ Cremasteric fascia. ™™ External spermatic fascia from external oblique aponeurosis

is seen below the external ring in the scrotum.

A

Fig. 18.15: Structures related to cord in the inguinal canal.

B

Fig. 18.16: Surgical anatomy of inguinal canal.

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 xx xx xx xx xx xx

Defence mechanism of inguinal canal

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

C

Figs. 18.17A to C: Nerves in inguinal canal: Iliohypogastric 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. Ilioinguinal nerve (L1) 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 (L1, L2) enters the cord through internal ring supplying the cremaster and scrotum.

B

Fruchaud’s myopectineal orifice

It is an osseo-myo-aponeurotic tunnel. It is through this tunnel all groin hernias occur. It is bounded: xx Medially by lateral border of rectus sheath. xx Above by the arched fibres of internal oblique and transversus abdominis muscle. xx Laterally by the iliopsoas muscle. xx 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.

CHAPTER 18    Hernia

Fig. 18.18: Fruchaud’s myopectineal orifice is bound—by lateral border of rectus, iliopsoas, conjoint tendon, pectin pubis. Fig. 18.20: Location of direct and indirect inguinal hernia and femoral hernia.

Classification According to the Extent ™™ Incomplete: ¾¾ ¾¾

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.

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)

B

Indirect hernia It comes out through internal ring along with the cord. It is lateral to the inferior epigastric artery.

I. Gilbert classification

Direct hernia It occurs through the posterior wall of the inguinal canal through ‘Hesselbach’s triangle’ (bounded medially by lateral border of

A

B

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Newer classifications of inguinial hernia

Type I: Hernia has got snug internal ring through which a peritoneal sac passes out as indirect sac. Type II: Hernia has a moderately enlarged internal ring which admits one finger but lesser than two finger breadth. Once reduced it protrudes during coughing or straining. Contd...

C

Figs. 18.21A to C: (a) Bubonocele; (b) 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.

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Contd... Type III: 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. II. NYHUS classification

B

1. Extraperitoneal tissue 4.  External spermatic fascia 2.  Internal spermatic fascia 5.  Skin 3.  Cremasteric fascia

B xx xx xx xx xx

Type I: Indirect hernia with normal deep ring. Type II: Indirect hernia with dilated deep ring. Type III: Posterior wall defect. a. Direct. b. Pantaloon hernia. c. Femoral hernia. Type IV: Recurrent hernia. III. BENDAVID Classification [Type, Staging, Diameter (TSD) classification] Type I: Anterolateral defect (indirect). Type II: Anteromedial (direct). Type III: Posteromedial (femoral). Type IV: Posterior prevascular hernia. Type V: Anteroposterior defect: Inguino-femoral hernia. European Hernia Society (EHS) classification (Aachen) P = primary hernia. R = recurrent hernia. 0 = no hernia detectable. 1 = 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.

Fig. 18.91: Umbilical hernia dual mesh to place both intraperitoneally and retrorectus plane with straps to fix.

PARAUMBILICAL HERNIA (Supra- and Infraumbilical 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.

A

B

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.

™™ 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.

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.

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.

B xx xx xx xx

xx xx

Fig. 18.93: Epigastric hernia. Often multiple Swiss cheese defects are seen.

Mayo’s operation

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 It is not commonly used now

™™ Postoperative weight reduction, use of abdominal binder is

required for these patients.

Fig. 18.94: Epigastric hernia showing features of strangulation.

B xx

EPIGASTRIC HERNIA (Fatty Hernia of linea alba)

xx xx

™™ 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 like a 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.

xx xx

Clinical features

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 found. Irreducibility, obstruction, strangulation as seen in any other hernia can also occur in epigastric hernia.

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.

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Clinical Features

™™ If the defect is less than 1.5 cm, lateral margin of the defect

772

Contd... xx

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xx

A

xx

Commonly it is deep to external oblique aponeurosis Types: –– Preperitoneal—between peritoneum and transversus abdominis muscle—20% –– Interparietal/intermuscular—between external oblique and internal oblique; most common—60%. It is commonly associated with inguinal hernia –– Extraparietal (inguinosuperficial)—herniates through external 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 treatment

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 costal 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.

B

C

Features

D

Figs. 18.95A to D: Epigastric hernia—incision, sac and defect. ™™ Complete reconstruction of linea alba is needed from xiphi-

™™ 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.

sternum 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; double breasting of the linea alba.

B xx xx xx xx xx

Interparietal hernias/interstitial hernias

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 Contd...

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. ™™ Differential diagnosis: Abdominal wall lipoma; Soft tissue

sarcoma; Abdominal wall haematoma.

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.

obturator nerve signifies not only obturator hernia but also strangulation—Howship-Romberg sign (50%). ™™ On per vaginal examination, tender swelling is felt over the obturator foramen. ™™ Here strangulation is usually of Richter’s type. ™™ Treatment: ¾¾ 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; ¾¾ Mesh placement is the ideal way of repairing the obturator defect; ¾¾ In nonobstructive type, if diagnosed clinically and by CT scan imaging, either TEP (laparoscopic) or lower abdominal midline extraperitoneal approach may be the method to manage the obturator hernia; ¾¾ 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 A

B

Figs. 18.97A and B: Spigelian hernia. On table finding and placement of mesh in same patient.

OBTURATOR HERNIA ™™ 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).

Features

A

™™ 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.

B

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: ¾¾ Resection and anastomosis is done; ¾¾ The type of hernia is treated; ¾¾ 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.

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CHAPTER 18    Hernia

™™ Investigation: Ultrasound abdomen.

™™ Referred pain in knee joint through geniculate branch of

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™™ Superior lumbar triangle (Grynfelt’s/Lesgaft’s triangle) is

bounded by sacrospinalis, 12th rib and posterior border of internal oblique. ™™ Inferior lumbar triangle 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: ¾¾ Primary. ¾¾ Secondary, which is due to previous renal surgery, more common.

B

Types

Classified based on their relationship to the pyriformis muscle and ischial spine. 1. Suprapyriformis. 2. Infrapyriformis. 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 pyri-

formis muscle after reducing the sac contents.

COMPLICATIONS OF HERNIA SURGERY

B

A

Figs. 18.100A and B: (a) Lumbar triangles; (b) 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. Inguinodynia, 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 surgery— ventral/femoral

• Late • Life-threatening

Inguinodynia ™™ 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 hernia 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 • Ischaemic orchitis—due thrombosis of pampiniform venous plexus (0.5%) • Injury to vas • Injury to viscera • Recurrence • Hydrocele formation • Seroma, haematoma • Inguinodynia—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

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 recurrence as a primary complication. 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. Ilioinguinal, 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 sufficient 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—ileos-

tomy/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) Intras-

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. ¾¾ Stoma is dissected, hernia content is reduced and defect is repaired. But it shows high recurrence. ¾¾ Subcutaneous mesh repair as onlay placement after reduction of hernia. ¾¾ Extraperitoneal mesh repair around the stoma. ¾¾ Intraperitoneal mesh repair deep to peritoneum surrounding the stoma and defect.

B

Different types of hernia



1. Gibbon’s hernia—It is hernia with hydrocoele 2. Berger’s hernia—Hernia in pouch of Douglas 3. Romberg hernia—Saddle hernia 4. Obturator hernia—Hernia through obturator foramen (canal) 5. Grynfelt’s hernia—Upper lumbar triangle hernia 6. Petit’s hernia—Lower lumbar triangle hernia 7. Femoral hernia—Hernia medial to femoral vein 8. Cloquet’s hernia—Hernia through pectineal fascia 9. Narath’s hernia—Behind femoral artery, in congenital dislocation of hip 10. Hesselbach’s hernia—Lateral to femoral artery 11. Serofini’s hernia—Behind femoral vessels 12. Laugier’s hernia—Through lacunar ligament 13. Teale’s hernia—In front of femoral vessels 14. Richter’s hernia—Part of circumference of bowel wall is gangrenous 15. Littre’s hernia—Hernia with Meckel’s diver­ticulum as the content 16. Sliding hernia—Posterior wall of the sac is formed by colon or bladder 17. Maydl’s hernia—‘W’ hernia 18. Phantom hernia—Localised muscle bulge following muscular paralysis 19. Spigelian hernia—Hernia through spigelian fascia 20. Mery’s hernia—Perineal hernia 21. Sciatic hernia—Hernia through greater or lesser sciatic foramen 22. Beclard’s hernia—Femoral hernia through the saphenous opening 23. Barth’s hernia—Hernia between abdominal wall and persistent vitellointestinal duct 24. Holthouse’s hernia—Inguinal hernia that has turned outwards into the groin 25. De Garengeot’s hernia is incarcerated appendix in femoral hernia

The wind of anger blows out the lamp of intelligence.

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CHAPTER 18    Hernia

Immediate

chapter

19 Oesophagus

C hapter Outline

B

·· 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 Ulcer ·· Oesophageal Motility Disorders

xx

·· Achalasia Cardia ·· Plummer-Vinson Syndrome ·· Corrosive Stricture of Oesophagus ·· Schatzki’s Rings ·· Boerhaave’s Syndrome ·· Mallory-Weiss Syndrome ·· Tracheo-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.

xx xx xx xx xx

Anatomical specialities

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. Segmental blood supply. Only part of GIT which shows very thinly scattered Meissner’s plexus. Longitudinal arrangement of veins and lymphatics.

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 dehiscence which 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.

B xx

xx xx

Three areas of anatomic narrowing

Cervical constriction—occurs at the level of crico­pharyngeal sphincter—narrowest point of GIT—15 cm from upper incisor— site of F/B impaction Bronchoaortic constriction—located at the level of T 4 – 25 cm from upper incisor—site of endoscopic perforation Diaphragmatic constriction—occurs where oeso­phagus traverses the diaphragm (Level of T10)—40 cm from upper incisor

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.

tracheobronchial nodes in thorax. ™™ Abdominal oesophagus drains into coeliac nodes.

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.

A

B

Figs. 19.2A and B: (A) Arterial supply and (B) Venous drainage of oesophagus.

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 oeso­ phagus 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.

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CHAPTER 19    Oesophagus

Arterial supply of oesophagus By inferior thyroid artery, oesophageal branches of the aorta, gastric arteries and inferior phrenic arteries.

™™ Thoracic oesophagus drains into paraoesophageal and

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.

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.

778

DYSPHAGIA ™™ Dysphagia is difficulty in swallowing. Painful swallowing is

odynophagia.

SRB's Manual of Surgery

™™ It can be acute—due to foreign body impaction or acute infec-

tion or chronic due to causes like stricture or carcinoma, etc.

™™ Associated hoarseness of voice may be present in advanced

pharyngeal or post-cricoid carcinomas. At late stage laryngeal carcinoma also can cause dysphagia along with hoarseness of voice. ™™ Dysphagia can be oropharyngeal or oesophageal depending on the cause. ™™ Dysphagia may be due to pathology in voluntary/pharyngeal phase of the swallowing wherein patient also develops cough while swallowing. Dysphagia due to problem in oesophageal involuntary phase of swallowing is specified by food getting stuck in the pathway. But site of “food getting stuck” feeling is not relevant. ™™ Dysphagia can be progressive or intermittent. 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.

Physiology Three types of contractions in the oesophagus: 1. Primary: Progressive, triggered by swallowing. 2. Secondary: Progressive, generated by distension or irritation. 3. Tertiary: Nonprogressive. 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 pres-

sure zone located in the lower end of the oesophagus in terminal 4 cm, with a resting pressure of 10–25 mmHg. ™™ 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 (physiological) 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.

Causes of Dysphagia Common causes: ™™ Gastro-oesophageal reflux diseases (GORD/GERD/Hiatus hernia). ™™ Carcinoma oesophagus: Here dysphagia is of short duration and progressive. 2/3 of the lumen should be blocked by tumour to develop dysphagia. ™™ Foreign body in oesophagus: It may be coin/bone piece/ denture. It is common in children. It causes acute dysphagia. It may be often life-threatening. ™™ Carcinoma of pharynx or posterior 1/3rd of the tongue. ™™ Corrosive strictures: It is usually alkali stricture. Squamous mucosa is resistant to acid effect to certain extent. ™™ Oesophageal candidial infection: It is becoming common due to immunosuppression in association with HIV infection; steroid therapy; cancer chemotherapy; post-transplant period, etc. Presentation is dysphagia and odynophagia. Oral candidiasis (thrush) is obvious. Endoscopy shows whitish curd-like plaques in the oesophageal mucosa which cannot be moved (whereas food particles can be moved). Barium swallow shows mucosal ulceration and irregular areas. Biopsy confirms the diagnosis. Treatment is oral antifungal as well as topical antifungal therapy.

A

B

Figs. 19.5A and B: Endoscopic view of oesophageal candidiasis infection. Note the whitish curd-like plaques.

T

779

Causes of dysphagia Causes in the wall of oesophagus or other area

Causes in the lumen

• Mediastinal nodes— secondaries/lymphoma/ tuberculosis • Aortic aneurysm • Rolling hiatus hernia • Thyroid enlargement— malignant • Dysphagia lusoria • Congenital anomalies • Mediastinitis/mass

• Carcinoma oesophagus • Corrosive/tuberculous/inflammatory/congenital stricture oesophagus • GERD • Achalasia cardia • Plummer-Vinson syndrome • Oesophageal diverticulum • Carcinoma posterior 1/3rd of tongue/pharynx • Diffuse oesophageal spasm • Congenital anomalies • Retropharyngeal abscess/peritonsillar abscess (Quinsy)/acute tonsillitis/pharyngitis

• Foreign body in the oesophagus—coin/ dentures/fish or meat bone Other causes Cranial causes (neurological): • Bulbar palsy/infarction/hemiplegia • Vertebrobasilar insufficiency

™™ Plummer-Vinson syndrome.

™™ Boerhaave’s syndrome: It is vertical full thickness tear of

™™ Mediastinal swellings—primary tumours/nodal mass either

lower oesophagus due to vomiting with closed glottis. It is often life-threatening and emergency. ™™ Neurological causes like stroke, bulbar palsy, motor neuron disease, Parkinson’s disease, etc. ™™ Congenital anomalies of oesophagus. ™™ Drug-induced dysphagia: Drugs like KCl, quinine, NSAID can cause dysphagia. ™™ Mediastinal fibrosis.

lymphoma or secondaries or tuberculosis. Rare causes: ™™ Diffuse oesophageal spasm: They are incoordinated contrac-

tions of oesophagus causing chest pain or dysphagia. It is common in distal 2/3rd of the oesophagus. Hypertrophy of circular muscle fibres with very high persistent pressure of 400–500 mmHg is specific. Treatment is calcium channel blockers, vasodilators, endoscopic dilatation and extended oesophageal surgical myotomy up to the aortic arch (very useful especially for dysphagia; not much for chest pain). ™™ Oesophageal diverticula, Chaga’s disease. ™™ Dysphagia lusoria (Lusoria means in Latin “sport of nature”): It is a congenital vascular anomaly of aortic root. Aortic arch anomalies are—double arch (40%), right arch and left ligamentum arteriosum (25%), anomalous innominate or common carotid artery or aberrant right subclavian artery (10%). It is due to disappearance of proximal right 4th aortic arch instead of distal portion. All patients having this anomaly (dysphagia lusoria) have got an aberrant right subclavian artery in a transposed position arising from descending aorta that courses posterior to oesophagus. Often there will be a complete vascular ring around trachea and oesophagus. It is categorised based on their specific subclavian anomaly— depends on presence of aneurysm, occlusive disease and compression. Commonly they are asymptomatic. Presentations may be dysphagia, chest pain, stridor, wheeze, recurrent respiratory infection (usually presents after the age of 40). Investigations: CT chest, MRI, chest X-ray, barium swallow (T4 level diagonal impression) and endoscopy (shows pulsating extraluminal compressive mass). Treatment is reconstruction or ligation of aberrant right subclavian artery by sternotomy/by neck approach. ™™ Thyroid swelling: It is uncommon to develop dysphagia in a thyroid swelling. There will be always dyspnoea when dysphagia develops. Large malignant thyroid or anaplastic thyroid can cause dysphagia with dyspnoea or stridor.

Evaluation of a Patient with Dysphagia ™™ Proper history. ™™ Haematocrit. ™™ Chest X-ray often shows mediastinal mass lesion/foreign

body. ™™ Oesophagoscopy: Once lesion is detected, it is treated accordingly. Biopsy from lesions, endotherapy if needed should be carried out (like F/B removal; stricture dilatation; sclerotherapy). ™™ Barium swallow may show irregular filling defect or extrinsic compression. ™™ CT scan chest is very useful method to identify the anatomical location of the cause (nodes/tumour/aorta/cardiac cause/ congenital). Extent, spread, nodal status, size and operability of tumour also well-assessed. ™™ Oesophageal manometry in achalasia cardia/GERD. ™™ 24 hours pH monitoring is ideal and most accurate for GERD. Small pH probe (transnasal catheter) is passed into the distal oesophagus 5 cm proximal to upper margin of LOS under manometry guidance. Probe is connected to a digital recorder worn by the patient for 24 hours. Record is analysed using a computer. A pH less than 4 for more than 4% of total 24 hours period (more than near to one hour in toto in 24 hours) is pathological reflux. It is often assessed by scoring system. Radio-telemetry pH probes are used now without any nasal tube. It is passed and placed on the oesophageal wall using endoscope.

Instrumentation is the most common cause of oesophageal rupture.

CHAPTER 19    Oesophagus

Extraluminal causes

SRB's Manual of Surgery

780

™™ Endosonography is very useful in many conditions causing

dysphagia. It can assess site, layers of the oesophagus, nodes, spread, etc. properly. Different layers are seen as alternating hyperechoic and hypoechoic bands. ™™ Ultrasound abdomen to see abdominal nodes/liver/ascites. ™™ MRI study.

Treatment for Dysphagia Depends on cause—modified Heller’s myotomy; oesophageal resection; dilatation; F/B removal, etc.

CONTRAST STUDY OF OESOPHAGUS Types

™™ Oesophageal carcinoma—irregular stenosing lesion with

shouldering (‘Rat tail’ is a fluoroscopic finding). ™™ Pharyngeal pouch—demonstration of the pouch. ™™ External compression—indentation of barium column by

superior or posterior mediastinal mass, enlarged left atria as in mitral stenosis.

OESOPHAGOSCOPY

B

Diagnostic xx xx

1. Barium swallow using barium sulphate (thick paste). 2. Using water-soluble contrast like ‘Gastrografin’.

Indications

To identify the lesion and to take biopsy in carci­noma oesophagus. For diagnosing other oesophageal conditions.

Therapeutic To remove foreign body xx To dilate stricture xx To place endostents for inoperable carcinoma oeso­phagus xx To inject sclerosants for varices xx

B

A

Figs. 19.6A and B: Barium swallow X-rays showing narrowing and irregularity in carcinoma oesophagus.

Indications 1. Barium swallow ¾¾ Dysphagia due to motility disorder like acha­lasia cardia, diffuse oesophageal spasm. ¾¾ Dysphagia due to mechanical causes like carcinoma, benign strictures and neoplasms, external compression. ¾¾ Pharyngeal pouch and other diverticula. ¾¾ GORD. 2. Water-soluble contrast radiograph ¾¾ In suspected oesophageal perforation. ¾¾ Leaking oesophageal anastomosis.

Important Findings in Barium Swallow ™™ Achalasia cardia—‘Bird beak’ appearance, as the oesophagus

is grossly dilated above an apparent narrowing at the cardia. In long-standing cases—‘sigmoid oesophagus’. ™™ Diffuse oesophageal spasm—Corkscrew appearance. ™™ GORD—shows reflux when done in Trendelen­burg’s position.

Fig. 19.7: Oesophageal candidiasis—note the multiple curd-like lesions.

Types 1. Rigid oesophagoscope (Negus type) ¾¾ It is done under general anaesthesia. Head is extended and head end of the table is tilted upwards, scope is passed behind the epiglottis and cricoid through the crico­pharyngeal opening. This is the most diffi­cult part in oeso­phagoscopy. After that, negotiating through the oesophagus is easier. The lesion is identi­fied and biopsy is taken if required. ¾¾ Complications are perforation (most common site of perforation is at the level of cricopharyn­geus), bleeding. 2. Fibreoptic flexible oesophagoscope ¾¾ It can be done under local anaesthesia. Reflux and hiatus are well identified. Stomach also can be visualised. Easy to pass and perforation is unlikely. ¾¾ But tissue taken for biopsy is smaller and removal of foreign body is also difficult.

Oesophageal Endosonography ™™ It is useful method of finding and assessing invol­vement

THIRD SPACE ENDOSCOPY ™™ It is newer method wherein submucosal or intramural space

which is called as 3rd space (first being luminal space and 2nd peritoneal space) is approached for various therapeutic and diagnostic purposes. It is also designated as SuMO (SubMucosal Operation). ™™ Currently, it is used in upper gastrointestinal area like oesophagus and stomach. Procedures done are – POEM (Per oral endoscopic myotomy) for achalasia cardia, submucosal tunneling tumour biopsy, submucosal tunneling pyloromyotomy for gastroparesis after vagotomy (G-POEM), submucosal tunneling tumour excision (STER). It is said to be safer, effective and less invasive; procedure is fast developing.

GASTRO-OESOPHAGEAL REFLUX DISEASE (GORD/GERD) ™™ It is a pathological reflux from the stomach into the lower

oesophagus. ™™ It accounts for 75% of oesophageal pathology (most

common). ™™ Symptoms may be due to peptic oesophagitis, LOS dysfunc-

tion, aspiration due to reflux, due to late effects like stricture and obstruction. ™™ It is due to varied anatomical and physiological factors.

Anatomical Factors

™™ Reduced LOS pressure. Normal resting LOS pressure

is 10–25 mmHg. During swallowing, pressure drops to 1–3 mmHg for few seconds (10 seconds). Resting pressure is increased by gastrin, cholinergic, adrenergic, prokinetic drugs; decreased by secretin, cholecystokinin, glucagon, calcium channel blockers, deriphylline, coffee, fatty meal. ™™ Altered transient relaxation period in LOS. ™™ Reduced oesophageal clearance mechanism. Change in oesophageal clearance is due to altered oesophageal body pump (primary peristalsis with 30–80 mmHg amplitude propagative downwards waves; secondary by food bolus), altered salivary bicarbonate neutralizing mechanism of reflux acid, altered mucosal defense mechanism (mucous and surface active phospholipid). ™™ Delayed gastric emptying due to diabetes, neuromus­cular block, gastroparesis, medications. ™™ Increased gastric distension and gastric acid hyper­secretion. Note: GERD is not associated with H. pylori infection.

Other Factors ™™ Alcohol, smoking, stress, lifestyle. ™™ It is seen in infants and children and also in pregnant women.

Pathogenesis GERD begins from distended fundus due to variety of causes and other anatomical and physiological factors, repetitively unfolding the sphincter to the gastric juice leading to all problems. There is reduced and low LES resistance causing loss of barrier to reflux. It is probably due to gastric distension, delayed gastric emptying, and raised intragastric or intra-abdominal pressure. Gastric distension may be due to overeating or aerophagia. Fundic distension → stretching of fundus → LOS squamous epithelium exposed to acid gastric juice → oesophagitis →increased stimulus to swallow saliva to neutralise oesophagitis → further fundal distension → cycle repeats → sphincter is taken into stretched fundus → effects like erosion, ulceration, fibrosis, mucosal metaplasia.

B

™™ Obesity. ™™ Altered length of intra-abdominal oesophagus. Level of

acid exposure is inversely related to length of the intra­ abdominal oesophagus. More the length more the need of intra-abdominal pressure to cause the reflux and so less the reflux with more length. ™™ Alteration of phreno-oesophageal ligament. ™™ Altered obliquity of O-G junction (alteration in angle of ‘His’). ™™ Reduced pinching action (Pinch-Cock effect) of right crus of diaphragm. ™™ Alteration in normal mucosal rosette at O-G junction. ™™ Alteration in sling mechanism of gastric musculature.

781

Types

Classification I xx Symptomatic uncomplicated GORD (NERD—Nonerosive Reflux Disease) xx Symptomatic, complicated GORD Classification II xx Primary: Incompetent LOS xx Secondary: Due to surgery/disease Classification III xx GORD with sliding hernia xx GORD without sliding hernia Classification IV xx xx

Acute: After alcohol intake, stress, burns. Chronic: Hiatus hernia, postoesophageal surgery.

Reflux is precipitated by flexion of the trunk—Boot lacing sign.

CHAPTER 19    Oesophagus

or pathology of diffe­rent layers of oesophagus especially in carcinoma oesophagus. It shows all layers clearly and distinctly and so invasion can be better made out and operability can be decided. ™™ Endoscopic oesophageal staining using labelled iodine is used to identify early carcinoma in oeso­phagus. Normal mucosal cells contain glycogen which takes up iodine and so stains brown, whereas carcinoma cells will not take up iodine and so mucosa appears pale (not stained).

Physiological Factors

782

Clinical Features

Complications ™™ Reflux oesophagitis; Sliding hiatus hernia.

™™ Fatty dyspepsia.

SRB's Manual of Surgery

™™ Chest pain and heart-burn (pyrosis)—80%—main symptom.

Pain is more in lying down position and at night. It may mimic cardiac pain/angina. It often begins in the epigastrium and becomes substernal. ™™ Odynophagia (painful swallowing). ™™ Appearance of symptoms within seconds of ingestion of food is typical. ™™ Regurgitation—50%. It is return of oesophageal content. ™™ Laryngeal symptoms—hoarseness of voice. ™™ Dysphagia will occur once complications begin. ™™ Symptoms are more with change of position. ™™ Chronic cough, shortness of breath and hoarse­n ess— Nocturnal reflux—reflux is return of gastric content. ™™ Haematemesis. ™™ Post-prandial fullness, choking, wheezing, recurrent pneumonia are other features.

B xx xx

Triad

Heart burn Epigastric pain with dysphagia

xx

Regurgitation

Typical Symptoms Heart burn, regurgitation, dysphagia mainly; odynophagia, haematemesis often.

™™ Stricture lower-end oesophagus; Oesophageal shortening. ™™ Barrett’s oesophagus. ™™ Carcinoma (adeno) oesophagus (10% of GERD).

Oesophageal • • • • •

Extraoesophageal • Laryngitis • Recurrent pneumonia • Progressive pulmonary fibrosis

Oesophagitis Stricture Barrett’s oesophagus Oesophageal shortening Carcinoma (adeno)

Investigations ™™ Barium study in head down position. ™™ Endoscopy to exclude other disease and to assess any

mucosal injury—red inflamed mucosa often with ulceration. Sliding hernia can be identified through endoscopy. When patient retches, gastric mucosa will enter the OG junction and ascends upwards to variable distance. ™™ Mucosal biopsy to confirm metaplastic trans­formation. ™™ Oesophageal manometry to assess the function of LES. Length and pressure of LOS is important. Transient relaxation period which is increased is most important. ™™ 24 hours oesophageal pH monitoring—gold standard. PPI should be stopped for 3 weeks prior to pH monitoring.

Atypical Symptoms Cough, hoarseness, wheezing, sore throat, noncardiac chest pain and palatal/dental erosions. It will eventually lead into recurrent pneumonia and pulmonary fibrosis. If pH of the cervical oesophagus is below 4, for less than 1% of time, then respiratory symptoms are due to GERD.

T

De Meester’s scoring system

Grade

Features

A

B

C

D

Heart burn 0

None

1

Minimal—occasional episodes

2

Moderate—medical therapy visits

3

Severe—interferes daily activities Regurgitation

0

None

1

Minimal—occasional episodes

2

Moderate—on position and straining

3

Severe—features of aspiration Dysphagia

0

None

1

Minimal—occasional episodes

2

Needs fluid to clear

3

Causes food impaction

Figs. 19.8A to D: Gastro-oesophageal reflux disease (GORD) endoscopic views. ™™ Bernstein test: Instillation of 1:10 HCl into stomach of patient

with reflux will reproduce the symptoms; and pH in the lower end of the oesophagus decreases. Later pain disappears with saline infusion. ™™ Dual probe pH monitoring (one in distal oesophagus and one in proximal oesophagus/trachea) is used to confirm the respiratory complications of GORD.

™™ DeMeester scoring system is used to assess the severity of

B xx xx xx xx

Differential diagnosis

Achalasia cardia Carcinoma oesophagus Peptic ulcer Gallstones

xx xx xx

Pancreatic diseases Gastritis Cardiac angina

shortening, respiratory problems

Surgeries Antireflux surgery is the only effective long-term beneficial therapy ideally available and considered now. Laparoscopic fundoplication (most popular), Collis-Nissen vertical gastroplasty and fundoplication, resection of OG junction when metaplasia is present, Belsey Mark 4 operation (technically difficult) are the procedures used now.

B

Treatment of Uncomplicated GORD A. General measure—Lifestyle changes ¾¾ Control of obesity. ¾¾ Stop smoking and alcohol. ¾¾ Avoid tea, coffee and chocolate. ¾¾ Propped up position. ¾¾ Small frequent meals. B. Drugs ¾¾ H2 antagonists; antacids ¾¾ Proton pump inhibitors (PPIs)—very effective –– Omeprazole 20 mg—BD for 3–6 months. –– Lansoprazole 30 mg. –– Pantoprazole 40 mg. –– Esomeprazole 20 mg. –– Rabeprazole 20 mg, Ilaprazole 10 mg. ¾¾ Prokinetic drugs –– Metoclopramide, Domperidone 30 mg. –– Cisapride, Mosapride. –– Itopride 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 ¾¾ Endoluminal suturing (Wilson-Cook) ¾¾ Plexiglass microspheres (PMMA). Microsphere suspended in gelatin is injected through endo­scopic needle. Gelatin gets absorbed and spheres cause a tissue bulking. ¾¾ Gatekeeper reflux repair system. Endoscopic delivery of preformed radiopaque hydrogel into submucosa. ¾¾ Stretta catheter. Flexible, soft, 6 mm sized, 65 cm length tube with balloon/basket and 5.5 mm NITI electrode, irrigation and suction ¾¾ Enteryx injection technique (estrinyl vinyl alcohol) ¾¾ Endocinch technique ¾¾ Endoscopic full thickness plication. D. Surgery ¾¾ Indications for surgical treatment –– Failure of drug treatment –– Sliding hernia

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Principles of antireflux surgeries

Operation should restore the LES pressure 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

Fundoplication ™™ Nissen’s posterior total fundoplication: 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.

A

B

Figs. 19.9A and B: (A) Nissen’s fundoplication; (B) Toupet’s partial fundoplication. ¾¾ Rudolph Nissen (1959) first did total fundoplication. ¾¾ Opening the peritoneum over the oesophagus to mobilise ¾¾ ¾¾ ¾¾ ¾¾

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 fundus is wrapped

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the GORD. ™™ 99Tc sulphur colloid mixed with saline scan to study OG reflux and 99Tc HIDA scan to study and detect duodeno-gastric reflux are used.

–– Barrett’s ulcer; Severe pain –– Presence of complications like bleeding/stricture/

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around after crural repair (otherwise fundoplication may displace into thorax causing paraoesophageal hernia); intra-abdominal length of 2 cm oesophagus is created (not more); fundus of the stomach is known to relax in concert with oesophageal sphincter so only fundus should be used to buttress the sphincter; wrapping should be done only around sphincter oesophagus not around body of stomach; fundoplication should be kept in abdomen without under tension and without displacing into thorax. Nonabsorbable polypropylene sutures are used. ¾¾ Crural repair is done using interrupted polypropylene sutures. ¾¾ Fundoplication should prevent sphincter shortening/ unfolding during gastric distension, should preserve normal swallowing ability, allow proper belching and allow vomiting whenever needed. ¾¾ Bougie; on table and postoperative manometry to assess the pressure of new LOS; on table and postoperative gastroscopy—are commonly used in fundoplication. ¾¾ Mortality of procedure is very less; other parts of the abdomen can be assessed and addressed like gallstones. ¾¾ Complications are—gas bloat syndrome (inability to belch); dysphagia; inability to vomit; slippage, proximal migration; paraoesophageal hernia, splenic injury. Floppy Nissen’s fundoplication reduces the incidence of gas bloat syndrome. ¾¾ Laparoscopic Nissen’s fundoplication is ideal and equally successful. ¾¾ Transthoracic approach is used for Nissen’s fundoplication in patients who had hiatus hernia repair earlier; who has short oesophagus; in patients who is having sliding hiatal hernia that does not reduce below the diaphragm, associated pulmonary disease, in obese, in patients who are having narrow subcostal angle or barrel-shaped chest. ™™ Toupet’s partial 180° posterior fundoplication: 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.

Fig. 19.10: Dor anterior fundoplication. Right margin of the fundus is sutured to left margin of the oesophagus. Front aspect of the fundus is sutured to right margin of the oesophagus. Second row is also sutured to right crus. ™™ Rosetti Hell anterior fundoplication: Here anterior part of

fundus of stomach is used. Superomedial part of the fundus

is brought around oesophagus to suture into anterior part of fundus. Here short gastric vessels are not ligated and much less fundus is needed to be mobilised. ™™ Dor anterior fundoplication: Here right margin of the fundus is sutured to left margin of the oesophagus; front part of fundus is sutured to right margin of the oesophagus; 2nd row is also sutured to right crus. ™™ Watson’s anterolateral fundoplication: 5 cm intraabdominal oesophagus is created with blunt transhiatal dissection. 120° anterolateral fundoplication augments the LOS function during stomach distension. Bougie insertion, division of short gastric vessels are not needed here. ™™ Lind both anterior and posterior fundoplication of 300° with 60° anteriorly uncovered area is not routinely practiced now. ™™ Partial fundoplication with mesh wrap around is also used. But complications of mesh like erosion, stricture and adhesions are problems.

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Fundoplications

Nissen’s—total 360° posterior fundoplication Toupet’s—partial 180° posterior fundal/posterolateral Rosetti Hell—total anterior fundal Dor—anterior partial Watson’s—anterolateral 120° partial Lind—posterior and anterior

Note: Other procedures Belsey Mark IV operation (1967): It is plication of oesophagus to the diaphragm through many interrupted mattress sutures so as to push the oesophagus downwards to make it intra-abdominal with adequate length. It is done through left transthoracic approach. 240° fundoplication, creation of intra-abdominal oesophagus, crural sling repair are the techniques. It corrects GERD and also maintains normal eructation and physiological reflux. But long hospital stay and respiratory complications are distressing problems.

•

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.

•

•

•

Boerema operation: It is fixing OG junction in front into anterior abdominal wall. It is not practiced now. Oesophagogastrectomy is 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 relief but 80% recurrence rate; hence it is not done. Stricture oesophagus due to GERD which is dilatable can be treated by regular oesophageal dilatation and long-term PPI therapy.

• • • •

Complications of Surgery ™™ Oesophageal/gastric perforation; Haemorrhage. ™™ Pneumothorax/pyothorax. ™™ Vagus nerve injury; Cardiac arrhythmias. ™™ Sepsis—mediastinitis or septicaemia. ™™ Disruption/failure of fundoplication. ™™ Gas bloat syndrome.

HIATUS HERNIA It is the most common type of diaphragmatic hernia.

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B

Fig. 19.12: Vertical gastroplasty is done to lengthen the intraabdominal oesophagus.

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Thal’s patch procedure: It is done for localised nondilatable stricture oesophagus. Stricture is incised full thickness longitudinally. Mobilised fundus of stomach is placed and sutured as a serosal patch technique over the defect.

xx

xx xx

Types of hiatus hernia—classification

TypeI 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 fundus of the stomach along side the GE junction and oesophagus into the mediastinum with GE junction in normal intra-abdominal location. It is rolling hernia Type III hiatus hernia: It is combination of type I and type II Type IV hiatus hernia: It is the hernia containing other abdominal viscera as content like transverse colon and omentum

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.

Fig. 19.14: Different types of hiatus hernia.

Kindness is a language which the dumb can speak, the deaf can understand.

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•

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. A vertical cut along the line of oesophagus is made on the fundus 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.

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Complications

Gangrene of stomach; Gastric volvulus. Perforation into the mediastinum. Perforation into the peritoneum. Ischaemic longitudinal ulcer in the herniated stomach in rolling hernia is called as Cameron ulcer.

™™ Investigations: Plain X-ray—lateral and PA erect view A

B

Figs. 19.15A and B: Endoscopic view of oesophageal hiatus hernia.

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 xx

SAINT’S TRIAD

showing retrocardiac air-fluid level; Barium meal study very useful; ECG; 3D CT scan is useful. ™™ Treatment: Treatment is always surgical. ¾¾ Excision of sac and repair of the defect. ¾¾ If it is gangrenous, gastrectomy is required. ¾¾ Either abdominal or thoracic or laparoscopic approach can be used in treating rolling hernia surgically. ¾¾ Mesh reinforcement to hiatus to close the defect may be needed.

Hiatus hernia; Diverticulosis; Gallstones

REFLUX OESOPHAGITIS

T

Grading of hiatus hernia Grade

Type

I-H0 II-H1 III-H2 IV-H3 V-H4

Normal Sliding Rolling Mixed Massive herniation

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.

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 xx xx xx

Fig. 19.16: Types of hiatus hernia.

xx

Grading

Mucosal erythema Mucosal erythema + superficial ulceration Mucosal erythema + superficial ulceration + submucosal fibrosis Mucosal erythema + extensive ulceration + para­mural fibrosis

ROLLING HERNIA (PARAOESOPHAGEAL HERNIA) It is herniation of stomach fundus or rarely other abdominal contents colon/spleen through a hiatus, usually towards left side.

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.

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 5. Columnar epithelium in continuity with the Z-line, non-circular, star shaped or circumferential, alone or in association with grade 1–4 lesions

B

B

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 (columnar) metaplasia in lower oesophagus. ™™ Treatment: ¾¾ Antacids; H2 blockers: Ranitidine, famotidine. ¾¾ Proton pump inhibitors—Main method and more effective. –– Omeprazole 20 mg BD 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). ¾¾ Prokinetic drugs like metochlopramide, domperi­done, cisapride, mosapride. ¾¾ Treating GORD and associated causes. By fundopli­cation and other surgeries. ¾¾ 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 (CELO) 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 suitable 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.

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.

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.

Features ™™ Features of GERD, 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 O-G junction (25 times more common)

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.

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Los Angeles classification of reflux oesophagitis—grading A. One or more mucosal break no longer than 5 mm that does not extend between the tops of two mucosal folds B. One or more mucosal break more than 5 mm long that does not extend between the tops of two mucosal folds C. 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 D. One or more mucosal break, which involves at least 75% of the oesophageal circumference

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™™ Argon beam coagulation. ™™ Proton pump inhibitors—high dose for 3–6 months. ™™ Antireflux treatment by surgery. ™™ Resection—Always better choice—for high-grade dysplasia.

™™ Nonspecific oesophageal motility disorders: It shows slow

Transhiatal oesophagectomy is preferred. ™™ Endoscopic mucosal resection is also good.

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.

BARRETT’S ULCER

Secondary

™™ It is an ulcer in columnar epithelium lined Barrett’s oeso­

phagus 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 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; Simultaneous multipeak waveforms of high amplitude and long duration with 20 or more simultaneous waveforms out of 10 wet swallows is diagnostic. Often it can be segmental or involve distal 2/3rd 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 amplitude peristalsis, equal in both sexes. Severe chest pain (noncardiac), dysphagia without regurgitation, odynophagia are the features. Waves of hypertensive amplitude pressure >180 mmHg 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, 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.

™™ Neurological—stroke, bulbar palsy, motor neuron disease,

multiple sclerosis, Parkinson’s disease, poliomyelitis. ™™ Muscular—myasthenia gravis, muscular dystrophy, dermato-

myositis. ™™ 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

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.

Fig. 19.17: Diagrammatic representation of achalasia cardia.

Aetiology ™™ There is absence or less numbered ganglions in myenteric

plexus. ¾¾ Stress; Vitamin B1 deficiency. ¾¾ Chaga’s disease. It is caused by Trypanosoma cruzi, which is common in South America called as sleeping sickness.

¾¾ Diffuse oesophageal spasm (Corkscrew oeso­phagus). ¾¾ Most commonly it is idiopathic. There is degeneration of

•

Clinical scoring of achalasia cardia are: 0  No weight loss or dysphagia or retrosternal pain or regurgitation. 1 Weight loss < 5 kg; occasional dysphagia, retrosternal pain, regurgitation. 2  Weight loss 5 –10 kg; daily dysphagia, retrosternal pain, regurgitation. 3 Weight loss >10 kg; dysphagia and regurgitation during each meal; retrosternal pain several times a day. Achalasia can be classic or vigorous.



•

Investigations ™™ Barium swallow is diagnostic—shows.

Pathology ™™ 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.

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.

B

A

B

Figs. 19.18A and B: (A) Barium swallow X-ray showing features of achalasia cardia; (B) Endoscopic view of achalasia. xx xx xx xx

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. Staging/Grading

I: Proximal dilatation 6 cm IV: Sigmoid dilatation

™™ Chest X-ray shows patches of pneumonia. Double mediastinal

strip of dilated oesophagus is typical with air fluid level in posterior mediastinum on lateral view.

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CHAPTER 19    Oesophagus

Auerbach’s myenteric plexus along the entire length of oesophagus more so in LOS. ™™ There is pencil-shaped narrowing of cardia (O-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).

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™™ Oesophageal manometry shows unrelaxed lower oesopha-

geal 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. Intraoesophageal pressure in relation to intragastric pressure 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.

Differential Diagnosis ™™ Carcinoma oesophagus. ™™ Stricture oesophagus.

¾¾ 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). ¾¾ Nissen’s or Toupet’s or Dor’s fundoplication is done along with the above procedure to prevent reflux. ¾¾ Laparoscopic/thoracoscopic cardiomyotomy—ideal. ¾¾ Robotic-assisted laparoscopic cardiomyotomy improves 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.

™™ Scleroderma.

Treatment

B

Treatment for achalasia cardia

Surgery: xx Modified Heller’s cardiomyotomy with Toupet’s / Dor’s fundoplication –– Open abdominal/thoracic (rare) approach –– Laparoscopic approach—ideal –– Robotic-assisted laparoscopic approach xx Resection of OG junction/transhiatal total oesophagectomy in severe cases—megaoesophagus/metaplasia POEM (Per Oral Endoscopic Myotomy) Forcible dilatation: xx Plummer’s pneumotic dilatation xx Negus hydrostatic balloon dilatation Drugs: xx Botulinum toxin xx Nitroglycerin, nifedipine

Surgery ™™ Modified Heller’s operation (Heller—German, 1913):

Oesophagocardiomyotomy. Success rate is 85%.

A

B

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; mucosal 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 ™™ It stretches spasmodic segment. Gradual repeated dilata-

A

B

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).

tions are required. Dilatation up to 54 French bougie can be done. Two types of dilatations are used—pneumatic and hydrostatic. ¾¾ 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. ¾¾ 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. ¾¾ Self expanding metal stents (SEMS) are also tried.

™™ Here oesophageal webs are seen in uppermost portion of the

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oesophagus with spasm of circular muscle fibres. ™™ Common in patients with long-standing iron deficiency

™™ Superficial glossitis, cheilitis, koilonychia commonly seen. ™™ Splenomegaly may be present. ™™ In oesophageal webs, mucosa is hyperkeratotic, friable, desqua-

mated. ™™ It is a premalignant condition and presents with severe

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: Pseudoachalasia 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 causing 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)

Fig. 19.23: Oesophageal web, endoscopic view.

dysphagia. ™™ Oesophagoscopy and biopsy is required to rule out malignancy. ™™ Treatment: ¾¾ Oral iron—ferrous sulphate 300 mg TDS with vitamins. ¾¾ Blood transfusion is given when there is severe anaemia (Transfusion of packed cells). ¾¾ IV or IM iron therapy. ¾¾ Once anaemia comes under control, webs will clear and patient can swallow. Follow-up endoscopy is a must. ¾¾ Dilatation of web may be required.

CORROSIVE STRICTURE OF OESOPHAGUS

B

Features of oesophageal corrosive lesion

Acute/immediate –– Severe pain, shock, laryngeal oedema –– Mediastinitis, septicaemia, haemorrhage, perforation xx Late/chronic –– Dysphagia; Stricture—50%; Malignant changes –– Severe malnutrition; Recurrent respiratory infection –– Oesophageal shortening –– Tracheo-oesophageal fistula formation Corrosive strictures can be multiple. Damage is more in lower 1/3rd of oesophagus xx

™™ Corrosives are commonest cause of oesophageal stricture. ™™ Mainly due to ingestion of alkali (Lye stricture—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 1/3rd of oesophagus. ™™ Alkali is odourless and tasteless and so large volume is ingested. Alkali causes liquefaction, saponification and thrombosis 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.

CHAPTER 19    Oesophagus

anaemia. ™™ Common in females.

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Phases of tissue injury in corrosive ingestion

Degrees of burns after corrosive oesophageal and gastric burns

Phase 1: Acute necrosis—1–4 days

1st degree: Mucosal hyperaemia and oedema

Phase 2: Ulceration— granulation—4–12 days

2nd degree: Small bleeding, exudates, ulcers, pseudomembrane

Phase 3: Cicatrisation and scarring—3 weeks to 6 months

3rd degree: Mucosal slough, deep ulcers, massive bleed, complete obstruction, charring, perforation

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. Note: Malignancy can develop in corrosive strictures—5%. It is 1000- fold greater than general population.

B

A

Figs. 19.24A and B: Endoscopic view of corrosive injury. Endoscopy should be done very gently in corrosive injury.

Treatment ™™ Acute phase management: ¾¾ 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. ¾¾ Emetics and NaHCO3 are avoided as they may precipitate perforation. ¾¾ 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. ¾¾ 2nd and 3rd degree burns: They are treated with fluid therapy, antibiotics, nutrition, resuscitation, PPIs, aerosolised steroids, fiberoptic-guided airway 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

B xx xx xx xx xx xx xx

Causes of stricture oesophagus

Peptic stricture (oesophagitis induced) Corrosives—most common cause Foreign body Postsurgical, radiotherapy Congenital Infection like tuberculosis Drugs like tetracycline, vitamin C

B

A

Figs. 19.25A and B: (A) Endoscopic view of oesophageal stricture; (B) Oesophageal dilatation is being done endoscopically.

A

B

Figs. 19.26A and B: (A) Barium swallow showing stricture 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.

SCHATZKI’S RINGS

jejunostomy.

¾¾ Most often surgery with resection may be required (thora-

cotomy and repair)—left thoracotomy is better. ¾¾ When severe mediastinitis occurs, condition has high mortality.

Note:

A

Ring should not be excised.

B

Figs. 19.28A and B: Diagrams showing: (A) Mallory-Weiss syndrome and (B) Boerhaave’s syndrome.

MALLORY-WEISS SYNDROME ™™ It is seen in adults with severe prolonged vomiting, causing

A

B

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.

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.

Investigations ™™ Gastroscopy, Hb%, PCV, blood grouping. ™™ During gastroscopy, if stomach is not inflated properly, 50%

BOERHAAVE’S SYNDROME

cases may be missed.

™™ 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

Presentations

Differential diagnosis

B xx xx

Features of Boerhaave’s syndrome Complications

Sudden onset of Myocardial infarction Mediastinitis symptoms Severe chest pain Pancreatitis Septicaemia Pain abdomen Peritonitis Subcutaneous Emphysema emphysema Shock ‘Crunching effect in the chest’ is called as Hamman’s sign. Mackler’s triad: (1) vomiting, (2) chest pain, (3) subcutaneous emphysema. ™™ 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.

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Differential diagnosis

Bleeding peptic ulcer Oesophageal varices

xx xx

Erosive gastritis Carcinoma stomach

™™ Treatment ¾¾ Conservative, as it is only a mucosal tear. ¾¾ Blood transfusion; IV fluids; Sedation. ¾¾ Haemostatic agents like vasopressin. ¾¾ Endoscopic injection therapy is used if required. ¾¾ Surgery is rarely required.

TRACHEO-OESOPHAGEAL FISTULA (OESOPHAGEAL ATRESIA)

Types (Refer Figure 19.28) ™™ In 85% cases, it is a blind upper end with lower end commu-

nicating with trachea.

All are equal in their ignorance.

CHAPTER 19    Oesophagus

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.

™™ Treatment: ¾¾ Nil by mouth; Antibiotics, IV fluids, TPN; Feeding by

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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 anas-

tomotic site; Reflux; Dysphagia

OESOPHAGEAL DIVERTICULUM

Vertebral defects Anal atresia Cardiac defect (PDA/VSD) Tracheo-oesophageal fistula Radial hypoplasia and renal agenesis

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 commonly associated with maternal hydramnios (50%). ™™ Investigations ¾¾ Obstruction is revealed while passing nasogastric tube. ¾¾ Contrast study will reveal fistula and obstruction (Dionosil 1 mL). ¾¾ Other anomalies are looked for. ¾¾ Chest X-ray. ¾¾ Echocardiography. ™™ Treatment Surgery ¾¾ Child requires feeding gastrostomy commonly. ¾¾ Often the procedure is staged one. ¾¾ 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.

Types 1. Pulsion diverticulum: Pulsion diverticula are false type containing mucosa and submucosa only; is due to high abnormal intraluminal oesophageal pressure developed due to various motility disorders. a. Pharyngeal pouch through Zenker’s or Killian’s dehiscence (Refer chapter Neck). b. Epiphrenic pulsion 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. evaluation with EUS, manometry is a must. –– Treatment: Diverticulopexy/diverticulectomy (exci-

sion) + oesophageal myotomy (Heller’s) + repair of associated hiatus hernia/antireflux procedure. 2. Traction diverticulum: Occurs in mid-oesophagus or in parabronchial region, is due to mediastinal granulomatous disease like tuberculosis. ¾¾ Traction diverticulum is a true type containing all layers in its wall and is due to traction by the healing fibrosing mediastinal lymph nodes. ¾¾ It is seen commonly towards right side. It has got wide mouth and it rests on the spine. ¾¾ Presentation is dysphagia, chest pain and regurgitation. ¾¾ CT scan (chest), barium study, manometry, endoscopy to assess mucosa with EUS, blood test for tuberculosis (ESR, peripheral smear) are the investigations. ¾¾ 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.

CARCINOMA OESOPHAGUS

xx xx xx xx xx

795

Tylosis

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

Pathology ™™ Common in: ¾¾ Middle third—50%. ¾¾ Lower third—33%. ¾¾ Upper third—17%. ™™ 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.

A

™™ 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.

B

Figs. 19.31A and B: Carcinoma oesophagus middle third— pathology specimen, gross and cut section.

™™ 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.

Aetiology Diet, deficiencies (vit. 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% Tylosis* (Hovels-Evans syndrome) Nitrosamines *Tylosis is an inherited disease with thickening of skin of palm and sole

Note: In India 90% are squamous cell carcinomas. In western countries, adenocarcinoma is becoming more common. Adenocarcinoma arises from submucosal oesophageal glands/heterotrophic columnar epithelium/Barrett’s oesophagus.

• • •

H. pylori infection is associated with reduced risk of oesophageal adenocarcinoma (inversely related).

B xx xx xx

Gross types

Annular (15%) Ulcerative (20%) Fungating—cauliflower like (60%)

xx xx

Polypoid Varicoid—diffuse submucosal type

Spread ™™ Direct ¾¾ Lack of serosal layer in oesophagus favours local exten-

sion. In upper third it spreads through muscular layer

All things must change to something new and strange.

CHAPTER 19    Oesophagus

–– Barium study, CT chest are diagnostic; endoscopic

B

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and gets adherent to left main bronchus, trachea, and left recurrent laryngeal nerve (causes hoarseness), aorta or its branches (causes fatal haemorrhage, but rare). ¾¾ It may perforate and cause mediastinitis. ¾¾ It may get adherent to pleura also. ¾¾ Broncho-oesophageal, tracheo-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.

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/coeliac 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 T0 No primary tumour Tis High-grade dysplasia T1 Invasion into the lamina propria, muscularis mucosae (T1a) or submucosa (T1b) 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)

B

A

N status – Nodes

Nx Regional nodes cannot be assessed N0 No 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 cM0 No distant metastases cM1 Distant metastases present pM1 Distant metastases microscopically confirmed

D

C

Figs. 19.32A to D: Gross outer look and cut-section of proliferative and indurated lesions of carcinoma oesophagus. ™™ Lymphatic spread ¾¾ It spreads both by lymphatic permeation and lymphatic

embolisation. ¾¾ It can cause satellite nodules elsewhere in the oeso­

phagus, away from the main tumour. ¾¾ Above in the neck, it spreads to supraclavicular

lymph nodes. ¾¾ In thorax, it spreads to paraoesophageal, tracheobron-

chial lymph nodes to sub­diaphrag­matic lymph nodes. ¾¾ In abdomen, it spreads to coeliac lymph nodes. ™™ Blood spread occurs to liver, lungs, brain and bones.

Histologic grade Gx Grade cannot be assessed G1 Well differentiated G2 Moderately differentiated G3 Poorly differentiated; Undifferentiated

Clinical staging (AJCC 8th edition, 2018) Stage 0: Tis N0 Mo. Stage I: T1 N0-1 M0. Stage II: T2 N0-1 M0; T3 N0 M0. Stage III: T3 N1 M0; T1-3 N2 M0. Stage IVA: T4 N0-2 M0; Any T N3 M0. Stage IVB: 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 [W0,W1,W2]; N- Nodal spread; M- Metastases)

Investigations ™™ Barium swallow: Shouldering sign and irregular filling defect.

Rat tail lesion on fluoroscopy is typical.

™™ Oesophagoscopy—to see the lesion, extent and type.

797

B

A

Figs. 19.33A and B: Barium swallow showing irregular filling defect and shouldering sign in middle third oesophagus.

B

C

D

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. ™™ CT scan (95% accuracy)—to look for local extension, nodal

status, perioesophageal/diaphragmatic/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 1/3rd 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 (Lugol’s, methylene blue—get absorbed by specific cell membranes), contrast (indigocarmine—permeate into the mucosal 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.

CHAPTER 19    Oesophagus

A

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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 (FDG): 18 FDG is given to the patient. FDG enters highly active cells and gets phosphorylated to FDG 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—to stage oesophageal carcinoma and to identify the operability, nodal status. ™™ Endoscopic mucosal 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 1/3rd of tumour. Sufficient removal of contiguous structures may be needed in curative resection. ™™ If nodes are present, then multimodal approach should be used like—curative 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 1/3rd 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.

B

Palliative therapy (80% cases)

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 Horner’s syndrome Loss of weight >20% Metastatic disease

Pain Dysphagia Prevent bleeding Prevent aspiration

Treatment Note:

Gastrostomy should not be done as a palliative procedure.

Principles

B

Indications for curative treatment

1. Early growth when patient is fit. 2. When there is no involvement of adjacent perioesophageal structures, bronchus, liver or distant organs. ™™ 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).

799

Approaches for Different Level Tumours

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 tumour is mobilised. Partial oesophagectomy and oesophagogastric anastomosis is done in the thorax. Intercostal 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 should 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 oesoph-

agogastrectomy 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.

Fig. 19.38: Transhiatal blind oesophagectomy— on table surgical specimen.

Other Approaches ™™ Thoracoscopic-laparoscopic oesophagectomy and lymphad-

enectomy 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 SCC is upwards. In adenocarcinoma 2-field clearance is sufficient (abdominal— coeliac 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: ¾¾ 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. ¾¾ 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, fistula formation, delayed emptying, redundancy, peptic ulceration, stricture formation. Colonic polyp and diverticulosis are contraindications for colonic conduit. ¾¾ 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.

CHAPTER 19    Oesophagus

Post-cricoid tumour (Squamous cell carcinoma): ™™ Treated mainly by radiotherapy. Radical radiotherapy— 5000–6000 rads. ™™ Often pharyngolaryngectomy is done along with gastric or colonic transposition. But complications are more in this procedure. Free jejunal transfer is the other option.

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Transposition is done through posterior mediastinum (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 oesophagectomy with 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: ¾¾ Standard: Nodes removed are—paratracheal, parabronchial, carinal, paraoesophageal, posterior mediastinal, paracardial, left gastric, along lesser curve of stomach. ¾¾ Extended/three field/ultraradical: As like standard above, with bilateral cervical lymphadenectomy, removal of upper mediastinal, coeliac, retroperitoneal, subhepatic nodes.

B xx xx xx xx xx

Surgical approaches

Ivor-Lewis 2-phase oesophagectomy McKeown 3-phase oesophagectomy Left abdominothoracic approach for lower oesophagus—Sweet approach Thoracoscopic—laparoscopic Transhiatal blind approach—Orringer’s

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 jeju-

nostomy feeding for nutrition.

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 ¾¾ 3000 Rads. Severe mucositis, stricture and fistula forma-

tion are the complications.

™™ Intraluminal RT ¾¾ 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

B

Figs. 19.39A and B: (A) Stricture oesophagus after radiotherapy given for carcinoma oesophagus; (B) Oesophageal stent in situ—a look endoscopically. ™™ Chemotherapy ¾¾ Cisplatin; methotrexate; mitomycin C; 5 FU; palcitaxel,

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 tracheo-oesophageal fistula or external compression. 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: ¾¾ Atkinson tube. ¾¾ 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. ¾¾ 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. ¾¾ 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

only after 1 week; need to avoid direct sunlight exposure by the patient for one month is the drawback. ™™ Surgery ¾¾ Transhiatal Orringer’s blind oesophagectomy is a palliative surgical procedure. ¾¾ 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).

B xx xx xx xx xx xx

Palliative procedures

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

A

B

Figs. 19.40A and B: (A) Mousseau-Barbin tube placed as a palliative method for carcinoma oesophagus; (B) Mousseau-Barbin tube.

B xx xx xx xx xx xx xx xx xx xx xx

Complications of oesophagectomy

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 Colonic dysmotility causing partial obstruction in colon transfer

Terminal Events in Carcinoma Oesophagus ™™ Cancer cachexia. ™™ Sepsis, mediastinitis.

Truth, like surgery, may hurt, but it cures.

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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 ¾¾ 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—here tissues 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.  P roblems of stents are—aspiration, displacement, erosion, bleeding, tumour growth across or beyond mesh, food bolus obstruction, retrosternal pain, need for reinsertion (40%). Mortality is 1–2%. ¾¾ 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 formation—5%, costly, takes one week to relieve dysphagia ¾¾ Endoscopic bipolar diathermy using a olive tip; argon beam plasma coagulation; endoscopic alcohol injection into the tumour. ¾¾ 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 endoscopy. 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

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™™ Immunosuppression.

™™ Treatment—if tumour is more than 5 cm/symptomatic

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™™ Malignant tracheo-oesophageal fistula (causes severe respir-

tumour/intraluminal tumour/when diagnosis is doubtful 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 mucosal ulceration/if tumour is near OG junction. Thoracoscopic resection can be done. Leak, empyema, sepsis and stricture are the occasional complications.

atory infection and death. Here expansile (self-expandable) endoluminal stents are used at the site of fistula to have temporary benefit). ™™ Erosion into major blood vessel—bleeding.

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%.

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/leiomyoma (commonest benign tumour of oesophagus—65%)/neurofibroma/rhabdomyoma/lipoma. 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. ¾¾ Only when leiomyoma reaches 5 cm in size, it causes obstruction. ¾¾ Multiple localised leiomyomas can occur which can be enucleated independently. ¾¾ True diffuse leiomyomas 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. ¾¾ Benign leiomyoma of oesophagus rarely turns into leiomyosarcoma. ¾¾ 90% of oesophageal leiomyomas occur in lower third of the oesophagus. ¾¾ Leiomyomas are common in men in 5th decade. ¾¾ 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.

A

B

Figs. 19.41A and B: Oesophageal submucosal tumour— endoscopic view—leiomyoma. Note: Unlike in the stomach and 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 ¾¾ It is 2nd commonest benign tumour of oesophagus. ¾¾ 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. ¾¾ Acquired cyst is from obstruction of the excretory ducts of oesophageal glands. ¾¾ Treatment: Enucleation or resection. If fistula is present it should be ligated and divided.

OESOPHAGEAL PERFORATION ™™ Causes: ¾¾ Instrumental injuries—commonest cause, 75%

commonest site is just above the level of crico­pharyngeus. ¾¾ Foreign bodies; Alkali injuries; Carcinoma oesophagus 1%. ¾¾ Boerhaave’s syndrome 15%; Trauma 9%. ¾¾ Surgical trauma (Vagotomy, thyroidectomy, Heller’s operation, pneumonectomy, spine surgery). ™™ Features: Chest pain, vomiting, shock, subcutaneous emphysema. ™™ Investigations: Chest X-ray—shows pneumomediastinum; CT scan ™™ Complications: Mediastinitis; Septicaemia; Empyema, ARDS

B

Foreign body oesophagus

Features: Sudden dysphagia with chest pain and breathless­ness xx Later features of shock, sepsis, mediastinitis, empyema xx

Management: X-ray shows site and level of the foreign body (FB) xx Endoscopic removal can be tried. xx Impacted large FB should be removed by thoraco­tomy xx Antibiotics, jejunostomy, TPN, ICT are also required xx

Fig. 19.42: Oesophageal perforation with mediastinitis and emyema; perforation is after eating large chicken bone. ™™ Treatment: ¾¾ Conservative treatment: It is advocated in small perfora-

¾¾

¾¾ ¾¾ ¾¾

¾¾ ¾¾

tions due to instrument where there is minimal air leak and contamination of mediastinum 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 pedicled 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. Intraluminal stents/mediastinal irrigation and drainage. Resection of oesophagus with gastric pull up. As condition is an emergency situation and with sepsis it carries high mortality. Oesophageal exclusion with cervical oesophagostomy above and feeding 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.

CHAPTER 19    Oesophagus

Common foreign bodies: xx Coins, metals, plastics xx Dentures xx Pins, toothpicks, batteries xx Fish or meat bones—dangerous—40% xx Food (meat—common/vegetables) impaction—45% Sites of impaction in oesophagus: xx Cervical constriction—C6 xx Broncho-aortic constriction—T4 xx Diaphragmatic constriction—T10 xx Pre-existing malignancy or inflammatory stricture site

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chapter

20 Stomach

C hapter Outline ·· Anatomy xx Blood Supply of Stomach xx Nerve Supply of Stomach xx Histology xx Lymphatic Drainage of Stomach xx Duodenum ·· Gastric Physiology ·· Gastric Function Tests ·· Gastrin ·· Barium Meal Study ·· Gastroscopy xx Helicobacter pylori ·· Congenital Hypertrophic Pyloric Stenosis ·· Gastritis ·· Acute Peptic Ulcer ·· Gastric Ulcer ·· Duodenal Ulcer ·· Pyloric Stenosis due to Chronic Duodenal Ulcer xx Electrolyte Changes in Pyloric Stenosis ·· Perforated Peptic Ulcer xx Perforated Duodenal Ulcer xx Perforated Gastric Ulcer xx Perforated Stomal Ulcer xx Dry Perforation ·· Bleeding Peptic Ulcer xx Bleeding Duodenal Ulcer

ANATOMY Bleeding Gastric Ulcer Haematemesis Complications of Gastric Surgery xx Duodenal Blow-out xx Recurrent Ulcer xx Dumping Syndrome xx Roux Stasis Syndrome xx Gastrojejunocolic Fistula Trichobezoar Chronic Duodenal Ileus Dunbar’s Syndrome Acute Gastric Dilatation Gastric Volvulus Gastric Polyp Menetrier’s Disease Duodenal Diverticula Carcinoma Stomach Gastric Lymphoma xx Primary Gastric Lymphoma xx Secondary Gastric Lymphoma Gastric Sarcomas Gastrointestinal Stromal Tumours Pyloroplasty Gastrostomy Gastrectomy Gastrojejunostomy Retrograde Jejunogastric Intussusception Vagotomy xx

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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 ampulla of Vater and enter the 2nd part of duodenum. The 3rd part of duodenum is wedged between aorta and superior mesenteric artery.

Fig. 20.1: Parts of stomach.

BLOOD SUPPLY OF STOMACH 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.

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CHAPTER 20    Stomach

Fig. 20.2: Blood supply of the stomach.

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.

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 fundus 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 HCl secretion, which is an important concept in vagotomy that is done as a treatment in duodenal ulcer. ™™ Truncal vagotomy with 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 fibres entering the stomach are ligated close to the lesser curve to reduce the acid secretion. ™™ In selective vagotomy splanchnic branches are retained but it is presently not done. ™™ Gastroduodenal pain is sensed via sympathetic fibres (T5 –T10).

Histology ™™ The fundus 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.

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 R0, R1, R2, R3, or D1, D2 (dissection) based

Goals and actions must meet.

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on levels of lymph nodes in the abdomen in relation to the stomach. R0 is no residual disease; R1 is microscopic residual disease; R2 is macroscopic residual disease; R3 is inoperable.

™™ Lymphatic drainage: Drains mainly to pancreaticoduodenal

nodes present along the inside of the curve of duodenum.

GASTRIC PHYSIOLOGY ™™ Gastric function is regulated by hormonal and neural

Fig. 20.4: Lymphatic drainage of stomach.

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, superior 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.

methods. Hormonal mediators control function through endocrine (through release into blood), paracrine (diffusion across interstitial space) and neurocrine (diffusion across synopsed 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, G and enterochromaffin-like (ECL) 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 stomach → antral G cells → acid release though 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 HCl, 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 PPIs. 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

GASTRIC FUNCTION TESTS

B xx xx xx xx xx

Basal

Peak

Normal

5 mmols

25–27

Gastric ulcer

2

15

Duodenal ulcer

6

35–38

Z-E syndrome

Very high

>than 60

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.

Insulin Test (Hollander) ™™ This test is useful postoperatively to confirm the complete-

ness 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 hypothalamus and through vagus to cause inc­rea­sed acid secretion. Patient who had under­ gone 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

Pentagastrin test/Kay’s augmented histamine test Hollander’s insulin test Radioisotope labelled gastric emptying study 24 hours intragastric pH monitoring Gastrin level estimation

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.

™™ Gastrin is secreted by the G-cells from gastric antrum. ™™ Types of gastrin are—Big gastrin (G 34 ); little gastrin

(G 17)—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 mucosal defense, has trophic effects on parietal and ECL 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 radioimmunoassay. ™™ It increases to many 1000’s in gastrinomas. ™™ Hypergastrinaemia can occur in ulcerogenic conditions like—antral G cell hyperplasia, retained excluded antrum, Z-E syndrome, short gut syndrome, gastric outlet obstruction; nonulcerogenic conditions are—PPIs, 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.

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CHAPTER 20    Stomach

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. ™™ Mucosal defense factors are—mucous production, mucosal HCO3–, mucosal blood flow, growth factors, cell renewal, endogenous prostaglandins, epithelial barrier (hydrophobic phospholipids, restitution, NO, epidermal growth factor and microcirculation). Aggressive factors are—H. pylori, HCl secretion, pepsin, smoking, alcohol, duodenal bile reflux, ischaemia, NSAIDs.

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xx

xx xx

Gastrin Somatostatin—2 types, 14 and 28. In stomach, type 14 is common; from D cells of gastric mucosa of fundus and antrum. Antral acidification is the main stimulus; vagal acetylcholine inhibits its release. It has paracrine effect inhibiting acid secretion from parietal cell, gastrin release from G cell and histamine release from ECL 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 ECL and mast cells. Its release is stimulated by gastrin, acetylcholine and epinephrine Ghrelin (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.

B xx

xx

xx xx xx

xx xx

Gastric hormones

Mottled appearance of stomach because of retained food particles. ¾¾ 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, trichobezoar, 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. ¾¾

Procedure ™™ Barium sulphate solution is used [Barium is neurotoxic, but

Investigations for gastroduodenal diseases

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 FDG 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)

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 Indications 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: ¾¾ Enormous dilatation of stomach. ¾¾ Greater curvature below the level of iliac crest. ¾¾ Absence of duodenal cap. ¾¾ No filling of dye in 2nd part of duodenum.

A

B

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.

Uses 1. For diagnosing any pathology, e.g. ¾¾ Gastric ulcer; Duodenal ulcer; Gastritis; Stomal ulcer. ¾¾ Carcinoma stomach.

¾¾ ¾¾

B xx xx xx xx xx xx

Therapeutic procedures done are:

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)

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. Fundus 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 ¾¾ Videoendoscopy is used not only for diagnosis but also mainly for therapeutic procedures; Both end viewing and side viewing gastroscopes are available. ¾¾ For therapeutic 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 Sandeep Gopal, Gastroenterologists, KMC, Mangaluru).

The stomach you can hear, the stomach you can see, the stomach you can feel.—Sir James Walton

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CHAPTER 20    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 gastrod­uodeno­ scopy is used.

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Often sedation with midazolam is beneficial to have an easy passage. Note: Buscopan is used to relax stomach wall. Z-line signifies squamocolumnar junction. Multi-byte biopsy forceps is used.

• • •

Complications ™™ 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.

HELICOBACTER 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 xx xx xx xx

H. pylori

Duodenal ulcer—95%; Gastric ulcer—70% Gastritis—70–90% Gastric cancer—No. 1 carcinogen Gastric MALTOMA (mucosa-associated lymphoid tissue lymphoma)

™™ Even though normal duodenum cannot harbour the Helico-

bacter, duodenum with gastric meta­plasia can very well get infected by Helicobacter which 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 xx xx xx

B xx

™™ 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 mucosal 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.

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. pylori three biopsies are taken one each from antrum, body and fundus.

xx

Pathogenesis

Enzymes and toxins released by H. pylori

xx xx xx xx

Tests for H. pylori

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 IgG 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

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.

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™™ Constipation.

Control of H. pylori Infection

™™ Dehydration and loss of weight.

Clarithromycin 500 mg BD

Metronidazole 400 mg BD

Omeprazole 20 mg BD

OR

OR

OR

Amoxycillin 500–750 mg BD

Tinidazole 600 mg BD

Lansoprazole 30 mg BD

OR

OR

Tetracyclines, or Bismuth.

Pantoprazole 40 mg BD

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.

Clinical Features

™™ Electrolyte imbalance—hypokalaemic metabolicalkalosis.

B xx xx

Clinical features of congenital pyloric stenosis

Vomiting; VGP Palpable mass

xx xx

Constipation Dehydration

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. ¾¾ Pyloric muscle 4 mm or more in thickness. ¾¾ Length of pyloric canal >14 mm. ¾¾ 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.

Differential Diagnosis ™™ Duodenal atresia (Bilious vomiting is present).

™™ 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. ™™ Visible 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%).

™™ High intestinal obstruction (e.g. volvulus neonato­rum). ™™ Intracranial haemorrhage.

Treatment 1. Correction of dehydration and electrolyte imba­lance. 2. Surgery: ¾¾ 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. ¾¾ 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 xx xx

Complications of surgery

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.

•

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CHAPTER 20    Stomach

It is treated with antibiotics and other drugs with different combinations

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Reflux Gastritis ™™ Usually occurs after gastric surgeries.

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™™ Prokinetic drugs are useful—metochlopramide, domperi-

done, cisapride, mozapride.

Erosive Gastritis ™™ It occurs due to disturbed gastric mucosal barrier. It is

induced by NSAID/alcohol. ™™ The precursor of prostaglandins, arachidonic acid, is A

B

C

D

Figs. 20.11A to D: (A) Ramstedt operation for congenital hyper­ trophic pyloric stenosis. Note that here muscular layer is cut but not mucosal layer; (B to D) Ramstedt pyloromyotomy for congenital hypertrophic pyloric stenosis. Only muscular layer is cut to allow mucosa to bulge out.

GASTRITIS Types 1. Type A gastritis. 2. Type B gastritis. 3. Reflux gastritis. 4. Erosive gastritis. 5. Others: Stress gastritis, lymphocytic gastritis, granulomatous gastritis, phlegmonous gastritis.

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 through 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 ischaemia of 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 occurs 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 (ECL 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.

B xx

xx xx xx xx xx xx

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—GERD/acid peptic diseases/gallstones/ pancreatitis/carcinoma H. pylori eradication is not required and there is no surgical role

ACUTE PEPTIC ULCER (DUODENAL OR GASTRIC ULCER)

abnormalities in acid and pepsin secretion. Acid becomes ulcerogenic even to normal gastric mucosa. ™™ Smoking, alcohol, NSAIDs, steroids. ™™ Helicobacter pylori infection (70%). ™™ There is either normochlorhydria or hypochlor­hydria. ™™ Altered mucosal barrier mechanism. ™™ Lower socioeconomic group.

Factors Involved in Gastric Ulcer Formation ™™ Duodenogastric reflux—reflux containing bile salts and lysol-

ecithin break the mucosal barrier making it more vulnerable for injury, action of drugs and pepsin injury. ™™ Gastric stasis. ™™ Ischaemia of the gastric mucosa. ™™ Type II and III gastric ulcers show acid hypersecretion.

™™ 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. ¾¾ Vomiting with or without haematemesis. ¾¾ Often acute peptic ulcers can lead to perforations. ™™ Treatment ¾¾ Intravenous ranitidine 50 mg, 8th hourly. ¾¾ IV fluids. IV pantoprazole/rabeprazole/omeprazole. ¾¾ 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 antiulcer drugs for 4-6 weeks—ranitidine, omeprazole or lanso­prazole. ¾¾

Curling’s Ulcers

A

B

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.

Pathology ™™ Gastric ulcer is large in size, usually lies in the lesser curva-

™™ 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 Aetiology It occurs due to imbalance between protective and damaging factors of gastric mucosa.

ture, 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 mucosal folds converging towards the base of the ulcer.

Troubles waste the stomach like rust waste iron.—Croatian Proverb

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CHAPTER 20    Stomach

xx

™™ Atrophic gastritis, duodenogastric bile reflux, gastric stasis,

Nonulcer dyspepsia

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Fig. 20.14: Specimen of stomach (identified by the mucosal 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, fundus 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.

Note: Often in lesser curve, saddle-shaped ulcer can occur. Type V gastric ulcer is–ulcer anywhere in the stomach associated with NSAIDs.

• •

B xx xx xx

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 occurs. ™™ On deep palpation, tenderness is felt in epigastric region.

Hiatus hernia Cholecystitis Chronic pancreatitis

xx xx xx

Chronic gastritis Dyspepsia Carcinoma stomach.

™™ Barium meal X-ray to see niche and notch.

B xx

™™ 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

Differential diagnosis

Investigations

xx

Clinical Features

T

Fig. 20.15: Types of gastric ulcer (Daintree Johnson).

xx xx xx xx

Barium meal X-ray features of benign gastric ulcer

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 ulcer— projects inwards towards the ulcer—Hamptom’s line Converging mucosal folds towards the base of the ulcer Symmetrical normal gastric mucosal folds

™™ Gastroscopy is done to see the location, type of ulcer and

also to take biopsy (10 biopsies).

™™ Ultrasound abdomen mainly to rule out other diseases and

to confirm associated diseases.

Types of gastric ulcer (Daintree Johnson)

Type

Location

Incidence Acid level

Type I

In the antrum, near the lesser curve 55%

Normal

Type II Combined gastric ulcer (in the body) with duodenal ulcer

25%

High

Type III Prepyloric ulcer

15%

High

Type IV Gastric ulcer in the proximal stomach or cardia

5%

Normal Fig. 20.16: Multiple ulcers visualised on gastroscopy.

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CHAPTER 20    Stomach

A

B

Figs. 20.17A and B: (A) Gastric ulcer in the body of the stomach; (B) Gastric ulcer in prepyloric region.

Fig. 20.19: Diagram showing Pauchet’s, Kelling Madlener, Csendes operations for proximal Type IV benign gastric ulcer.

Complications of Gastric Ulcer A

B

Figs. 20.18A and B: Barium meal study showing niche and notch— gastric ulcer.

Treatment

1. Hour glass contracture: It, occurs exclusively in women, is due to cicatricial contracture of lesser curve ulcer. ¾¾ Here stomach is divided into two compartments. ¾¾ Clinical features: Loss of periodicity; Persistent pain; Vomiting; Loss of appetite and weight.

™™ 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.

Fig. 20.20: Hour glass contracture.

Diagnosis: Barium meal: It shows filling only in the proximal stomach or double pouched stomach; Gastroscopy. ¾¾ 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. ¾¾

Fig. 20.21: Tea-pot deformity.

It is consequences which are more important than rewards or punishments.

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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 II and III 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 area rugae are normal

Surrounding area shows nodules, ulcers and irregularities

Size and extent

Large and deep

Small, deep up to part of muscle layer

DUODENAL ULCER 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: Zollinger-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 chronic 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.

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 of gastric ulcer and duodenal ulcer

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.

Complications of Duodenal Ulcer ™™ Pyloric stenosis (10%): Due to scarring and cicatrisation of A

B

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.

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%).

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 cm is called as giant duodenal ulcer.

Investigations

™™ Specific measures:

™™ Barium meal X-ray shows deformed or absence of duodenal

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, fundus, 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 xx xx

Differential diagnosis

Carcinoma stomach (pylorus) Dyspepsia due to other causes: Hiatus hernia; Oesophagitis; Cholecystitis; Chronic pancreatitis

Treatment Aim of therapy: To relieve symptoms; to heal ulcer; to prevent recurrence. ™™ General measures: ¾¾ Avoid alcohol, NSAIDs, smoking, spicy foods. Have more frequent food.

Intragastric pH should be maintained above 5.

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 H 2 blocker. Dose is 20–40 mg/day. –– Tab roxatidine; Tab nizatidine. ™™ Proton-pump inhibitors: ¾¾ Inhibit parietal cell H+, 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, Ilaprazole 10 mg –– Pantoprazole 40 mg—IV preparation available. –– Rabeprazole 20 mg—IV preparation available. ™™ Antacids: ¾¾ Neutralises the HCl to form water and salt and also inhibits peptic activity. ¾¾ Aluminium hydro­x ide and magnesium trisilicate are com­monly used. ¾¾ Dose is 2 grams 2 hours after food. ¾¾ Aluminium hydroxide causes constipation, magnesium trisilicate causes diarrhoea. Osteomalacia, milk alkali syndrome, rebound ulcer due to gastrin release are other complications. ™™ Sucralfate: ¾¾ It is an aluminium salt of sulfated sucrose which provides a protective coat to ulcer crater thereby promotes healing. It inhibits peptic activity. ¾¾ 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. ¾¾ Dose is 1 g qid for 6 weeks (before food). It is an effective drug. ™™ Anti-Helicobacter pylori regime: ¾¾ It is very useful, given for 7–14 days—later the protonpump inhibitors are continued. ¾¾ 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. ¾¾ PG E1 (mesoprostol) and E2 increase mucus and bicarbonate secretion, improves mucosal blood flow, but reduces acid secretion. ¾¾

Every man is the master of his own fortune.

CHAPTER 20    Stomach

cap (because of spasm). Appearance of ‘trifoliate’ duodenum is due to secondary duodenal diverticula which occurs as a result of scarring of ulcer.

¾¾

817

818

T

Anti-Helicobacter regime (Triple regime)

SRB's Manual of Surgery

Clarithromycin 500 mg BD

Metronidazole 400 mg BD

Omeprazole 20 mg BD

OR

OR

OR

Amoxycillin 500–750 mg BD

Tinidazole 600 mg BD

Lansoprazole 30 mg BD

OR

OR

Tetracyclines, or Bismuth, etc.

Pantoprazole 40 mg BD

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 PPIs by creating alkaline media.

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—intractable 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. ¾¾ HSV was first described by Amdrup and Johnston in 1969. ¾¾ 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. ¾¾ Intraoperative test for completion of vagotomy should be done—Grassi test or insulin test. ¾¾ 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. ¾¾ Problems—lesser curve necrosis due to ischaemia; recurrent ulcer 10–15% in 10 years. ™™ Selective vagotomy with pyloroplasty (SV + P). ™™ Truncal vagotomy with gastrojejunostomy (TV + GJ). ™™ Posterior truncal vagotomy with anterior seromyo­tomy— Taylor’s operation. It can be done through laparoscopy. ™™ Vagotomy with antrectomy: Gastrin producing antrum, vagal cholinergic pathway from ulcer bearing area is removed with ¾¾

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 gastrectomy with 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 Pathology Chronic DU after many years undergoes scarring and cicatrisation causing total obstruction of the pylorus, leading to enormous dilatation of stomach.

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—half an hour after installation of

Investigations ™™ Barium meal study:

Absence of duodenal cap. Dilated stomach where greater curvature is below the level of iliac crest. ¾¾ Mottled stomach. ¾¾ 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. ¾¾ ¾¾

™™ Correction of dehydration and electrolytes by IV fluids—

normal saline or double strength saline, calcium, potassium, magnesium. ™™ 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 ¾¾ HSV with gastrojejunostomy is present recommendation even though it is technically difficult. HSV is better than TV as it maintains the nerve supply of the chronically obstructed antrum and so may eventually reduce the chronic emptying problems. ¾¾ Truncal vagotomy along with gastroje­juno­stomy of Mayo (posterior, vertical/oblique, short loop, retrocolic, isoperistalsis) is done—ideal, commonly advocated procedure. ¾¾ Vagotomy, antrectomy (acid secreting area) with Billorth I anastomosis along with feeding jejunostomy for nutrition is the other option.

Fig. 20.25: Pyloric stenosis with gastric dilatation.

Fig. 20.27: Gastric outlet obstruction showing dilated stomach on table.

A

B

Figs. 20.26A and B: Barium meal pictures showing gastric outlet obstruction.

Differential Diagnosis Carcinoma pylorus—here mass may be palpable.

B xx xx xx xx xx

Pyloric stenosis—causes

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

819

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 E Coates

CHAPTER 20    Stomach

750 mL of saline, if volume remained and if more than 250 mL, suggest obstruction.

Treatment

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.

SRB's Manual of Surgery

Features ™™ 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

Fig. 20.29: Gastrojejunostomy stoma view on endoscopy.

analysis; Serum calcium level estimation. ™™ Treatment ¾¾ 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. ¾¾ The cause is treated. 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.

PERFORATED PEPTIC ULCER ™™ It is the terminology used for perforation of duodenal ulcer

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 temporarily with high recurrence rate.

• • • • •

ELECTROLYTE CHANGES IN PYLORIC STENOSIS xx xx xx

Hyponatraemia Hypokalaemia Hypomagnesaemia

xx xx xx

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 excess bicarbo­nate (In alkalosis without hyponatraemia, bicarbonate is secreted along

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 (GDP) 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 approximately 41%. The number of perforated ulcers remains unchanged. Sustained incidence 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 - Iatrogenic – endoscopies; ERCP; foreign body; others – like ZE syndrome. Precipitating factors: Helicobacter pylori infection; Aspirin, other NSAID ingestion; smoking, alcohol. 65% occurs in duodenum; 25% occurs in pylorus; remaining are stomal, proximal gastric. Classification: (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,

•

• • • •

• •

mesenchymal stem cells to enhance wound healing have only been evaluated experimentally and are not yet in clinical testing. In traumatic perforation, management is individualised depending on the site and associated injuries. Morbidity factors: Pneumonia (11%); wound Infection (5%); wound dehiscence (5%); leak (5-20%). Mortality factors: Sepsis (5%); leak; respiratory Failure (2%); cardiac (2%). Re leak carries high mortality—60%. Factors are—Age >60 years; Pulse rate >110/minute; Blood pressure 6 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. xx Ileum maintains enterohepatic circulation of bile salts, absorption of vitamin B12 and vitamin D. Resection of middle part of small bowel is better tolerated. xx 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. xx 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 will increase in colon which produces large amount of D lactate which is absorbed causing D lactic acidosis presenting with confusion, ataxia and nystagmus. xx TPN was introduced by Dudrick. It is very useful in short gut syndrome.

SRB's Manual of Surgery

872

quency transmitter with an antenna. Capsule takes 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 tumours. ™™ This capsule 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. ™™ Capsule 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.

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. ™™ Intraoperative enteroscopes to assess lumen on table.

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 overtube into stomach,

duodenum, and jejunum. Proper evaluation of the mucosa is done during withdrawal. If there is a bleeder point, it is fulgurated.

Sonde 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 air 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. Intraoperative 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 xx xx xx xx

Complications and problems

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 imbalance, malnutrition, and sepsis. An enterocutaneous fistula is an abnormal communication between the mucosal epithelium of the intestine and skin surface.

Classifications Anatomical ™™ External: They are enterocutaneous fistulas which are discussed in detail here. ¾¾ 75% of enteocutaneous fistulas are of postoperative cause due to disruption of the anastomotic site. ¾¾ 25% are spontaneous due to malignancy, radiotherapy, inflammatory bowel disease (most common in spontaneous cause), diverticular disease and bowel ischaemia. ¾¾ 70% of external fistula will close spontaneously. ™™ Internal ¾¾ 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

Fig. 21.34: Fistula at gastrostomy site. This is becoming the common cause.

A

B

Figs. 21.33A and B: Enterocutaneous fistula in two different patients.

Colonic: It can be due to postappendicectomy, postcolonic surgeries. 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.

A

B

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.

Fistula may be due to:

Physiological—based on quantity of daily output:

Fistula may be:

• • • •

• High output—>500 mL/day—usually small bowel; 50% mortality; less chance of spontaneous closure. • Moderate output—200–500 mL/day—colonic and small bowel mixed. • Low output—2 cm—30–50% chances of developing carcinoma; 1–2 cm —10% chances of carcinoma; 3 cm diameter; proximal 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.

B

Triad of small bowel obstruction in plain X-ray

1. Dilated small bowel loops >3 cm 2. Multiple air fluid levels in erect X-ray 3. Paucity of air in the colon

Fig. 23.11: CT abdomen showing dilated bowel loop; CT is ideal investigation for intestinal obstruction.

B xx xx xx xx

Complications of intestinal obstruction

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.

CHAPTER 23    Intestinal Obstruction

Fig. 23.9: Plain X-ray abdomen showing multiple air fluid levels due to bowel 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, ischaemia, 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 done with 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.

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Treatment

¾¾

SRB's Manual of Surgery

™™ 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 care: 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: ¾¾ 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.

B xx xx xx xx xx

¾¾

To check for viability of bowel, look for:

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. ¾¾

¾¾ ¾¾

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. ¾¾

¾¾

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.

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.

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.

Fig. 23.14: colonic obstruction due to stricture/growth in the transverse colon; right hemicolectomy is done. ¾¾

In case of left-sided colonic obstruction, left hemicolec­ tomy (resection) and colo-colic anastomosis is done with a defunctioning colostomy (right-sided transverse) which is closed after 6 weeks.

¾¾

¾¾

¾¾

B xx xx xx

Postsurgery complications

Pelvic abscess Subphrenic abscess Biliary or faecal fistulas

xx xx xx

Burst abdomen Bands and adhesions Incisional 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.

Types

xx

xx xx

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. 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; incom-

plete 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 preampullary (nonbilious vomiting) or postampullary (bilious vomiting). Postampullary is common (80–90%).

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.

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).

Investigations ™™ Plain X-ray shows classic double-bubble sign with absence

of air in the distal part.

Fig. 23.15: Types of duodenal atresia—complete; fibrous cord; duodenal stenosis and windsock deformity.

Fig. 23.16: Double-bubble sign in duodenal atresia.

An unspoken word never does harm.

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CHAPTER 23    Intestinal Obstruction

¾¾

Obstruction 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.

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™™ In partial obstruction, air may be present in distal loop and so

™™ Proximal bowel wall is dilated with hypertrophy but villi are

contrast study has to be done. Risk of aspiration in newborn infant should be remembered. ™™ US will show distended stomach and proximal duodenum, rail road track duodenum and features of associated anomalies. Maternal and fetal ultrasound in pregnancy may identify the pathology and also maternal polyhydramnios.

normal. Distal bowel is collapsed but with hypertrophied villi. Narrow collapsed large intestine (microcolon) is seen in proximal atresia.

Treatment ™™ Proper preoperative preparation like correction of fluid and

electrolytes; gastric decompression; TPN; injection vitamin K; evaluation for associated anomalies. ™™ Duodenoduodenostomy is done. Associated malrotation should be corrected in these patients (Ladd’s operation). Side-side duodenoduodenostomy may cause dilated duodenum (megaduodenum—30%); anastomotic dysfunction; and delayed transit of the content. ™™ Kimura’s diamond-shaped anastomosis between transversely opened proximal pouch and longitudinally opened distal pouch reduces the problems of anastomosis. Presence of bile in the duodenum and proximal and distal patency should be confirmed by saline irrigation. ™™ Transanastomotic nasojejunal or gastrostomy tube for feeding purpose is needed as prolonged postoperative ileus is the usual problem.

Fig. 23.18: Typical intestinal atresia. Griesfield modification of martin’s classification of intestinal atresia Type I: Membranous/mucosal with normal mesentery— 20%. Type II: The lumen is atretic—fibrous cord between proximal and distal parts of the segment involved (only one atretic segment) but mesentery is normal—40%. Type III (a): Atresia with complete separation of proximal and distal ends and V-shaped defect of mesentery—35%. Type III (b): Atresia with Christmas tree-shaped defect in mesentery with distal bowel being supplied by single artery—right colic/ ileocolic/superior mesenteric—apple peel atresia. Type IV: Multiple atresias—5%.

B xx xx

xx xx

xx

Fig. 23.17: Duodenoduodenostomy is ideal in duodenal atresia. Kimura’s modification has got lesser chance of developing side effects like gastroparesis. Note: Duodenojejunostomy may cause blind loop problems. Gastrojejunostomy should not be done due to high incidence of marginal ulceration and bleeding.

•

SMALL INTESTINE ATRESIA (INTESTINAL ATRESIA) ™™ It is jejunoileal atresia. ™™ It is due to intrauterine mesenteric vascular accidents (occlu-

sion) of the segments affected. V-shaped mesentery; presence of bile pigments in the distal segments suggesting earlier period of patency—are supportive for the above theory. ™™ It is often associated with malrotation, gastroschisis, volvulus— 20%. ™™ Common site is proximal jejunum; next common is distal ileum. ™™ Maternal polyhydramnios occurs in 35% of jejunal atresia.

Fig. 23.19: Types of intestinal atresia.

™™ Features ¾¾

™™ Stage 1: Exomphalos major/minor or gastroschisis. ™™ Stage 2: Errors of rotation in this stage is important and is

usually considered under malrotation. ¾¾ Nonrotation: Causing small bowel in right side; colon in left side; caecum in midline as suspension. ¾¾ Incomplete rotation: It is the most common type of malrotation. Caecum is located in subhepatic right hypochondrium. Ladd’s peritoneal band connects from caecocolic loop to posterior abdominal wall compressing the 2nd part of the duodenum. Entire midgut is hanging down along with SMA with a narrow-based mesentery causing midgut volvulus.

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 ¾¾ ¾¾ ¾¾ ¾¾

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 xx

xx

xx

Stages of normal rotation of midgut

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.

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Reverse rotation: Final 180° rotation occurs clockwise bringing colon posterior to duodenum and SMA. ¾¾ Hyper-rotation: Rotation up to 360° or 450° causing caecum on left side of the abdomen. ¾¾ 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. ¾¾

B xx xx

Associated anomalies (20%)

Congenital diaphragmatic hernia of Bochdalek Prune belly syndrome; Duodenal atresia; Oesophageal atresia

Presentations ™™ Acute/recurrent/subacute 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 ¾¾ ¾¾

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 is 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

CHAPTER 23    Intestinal Obstruction

Equal in both sexes; Commonly infant is of low birth weight. Bilious vomiting with features of intestinal obstruction like distension. ¾¾ Features of associated anomalies. ¾¾ Respiratory distress; Jaundice. ™™ Investigations ¾¾ Plain X-ray abdomen shows—triple-bubble appearance in jejunal atresia; multiple air fluid levels in ileal atresia. ¾¾ Barium enema may show narrow microcolon. Calcification suggests antenatal fetal bowel perforation. ¾¾ US abdomen to confirm associated anomalies. ™™ Differential diagnosis: Other causes of neonatal intestinal obstruction like duodenal atresia, malrotation, volvulus of midgut. ¾¾

Different Errors of Rotation

SRB's Manual of Surgery

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US abdomen/CT abdomen, if needed. Haematocrit, serum electrolytes estimation. ™™ Treatment ¾¾ Resuscitation, antibiotics, fluid and electrolytes, blood transfusion. ¾¾

™™ There is hypertrophy and dilatation of the proximal ileum

¾¾

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.

Fig. 23.22: Ladd’s operation for incomplete gut rotation. Ladd’s band is released. Duodenum is straightened. Appendicectomy is done. ¾¾

¾¾

Laparotomy through horizontal incision is done. Clockwise rotated midgut 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 Cl– 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 contain 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 xx xx xx xx xx xx xx xx

Commonly associated with cystic disease of pancreas but not necessarily always Respiratory dysfunction Exocrine pancreatic insufficiency High salt in the sweat >90 mmol/L Ileal 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 xx xx

Meconium ileus

Complications of meconium ileus

Intestinal bolus obstruction; Perforation and peritonitis Gangrene, volvulus formation

Treatment

xx

xx xx

Management of meconium ileus

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.

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, Bishop-

Koop 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.

Fig. 23.25: Santulli operation done for meconium ileus wherein proximal ileum is brought out as ileostomy. Distal ileum is sutured to proximal.

B xx xx xx xx

Causes of neonatal intestinal obstruction

Hirschsprung’s disease; Duodenal atresia Intestinal atresia; Malrotation Midgut volvulus/volvulus neonatorum Meconium ileus; Anorectal malformation

INTUSSUSCEPTION (ISS) Definition It is telescoping or invagination of one portion (segment) of bowel into the adjacent segment.

Types 1. Antegrade: Most common. 2. Retrograde: Rare (jejunogastric in gastrojejunostomy stoma). In elderly intussusception: xx Colocolic is most common type  xx Apex is formed usually by growth xx No role of hydrostatic reduction ™™ It can be single or multiple (rare). ™™ It can be ileo-colic (most common type, 75%), colocolic,

ileoileocolic, colocolic.

™™ It is common in weaning period of a child (common in male),

Fig. 23.24: Bishop-Koop operation. Here distal ileum is brought as ileostomy and proximal part sutured to distal bowel.

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.

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CHAPTER 23    Intestinal Obstruction

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.

B

920

Pathology ™™ Apex is the one which advances;

™™ Intussuscipiens is the one which receives (outer sheath);

SRB's Manual of Surgery

™™ Intussusceptum are the tubes which advance (middle and

A

B

Figs. 23.26A and B: (A) Typical intussusception—on table look; (B) Parts of intussusception.

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 xx xx xx xx xx

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

inner sheath). ¾¾ Apex and inner tubes will have compromised blood supply which leads to gangrene. ¾¾ 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. ¾¾ Gangrene which sets in leads to perforation and peritonitis. ¾¾ Red currant jelly is not commonly observed in ISS in adult, but it can occur.

Clinical Features ™™ Common in males (3:2). ™™ Common in 6–9 months. But can also occur at later age-

grouped children.

™™ Common in spring and winter, coinciding with the gastroen-

teritis 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 disten-

sion of the abdomen, vomiting, with passage of “red-currantjelly” stool. It is usually not found in adult ISS. ™™ Often ISS is recurrent, when it gets reduced, child 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).

Fig. 23.28: Sausage-shaped mass of intussusception. ™™ On examination, a mass is felt either on the left or right of the

Fig. 23.27: Intussusception. Different parts and apex point is also clearly seen.

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

B xx

xx xx

Abdominal palpatory findings in ISS

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

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.

A

Fig. 23.29: A rare condition where colonic intussuscep­tion has occurred through rectum, coming out per anally. Part is already gangrenous.

B xx

Complications

Intestinal obstruction; perforation; peritonitis

Differential Diagnosis In children: Acute gastroenteritis. Purpura with intestinal symptoms. In adults: Carcinoma colon. Mesenteric mass.

B

Figs. 23.30A and B: Barium enema showing the typical ‘Claw sign’ of intussusception.

B

Investigations ™™ Barium enema shows typical claw sign or coiled spring sign

xx

™™ Ultrasound shows target sign or pseudokidney sign or bull’s

xx

(Pincer end).

eye sign, which is diagnostic.

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xx xx

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.

CHAPTER 23    Intestinal Obstruction

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.

™™ Doppler may show mass with doughnut sign and is useful to

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Surgery

SRB's Manual of Surgery

™™ Cope’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. ¾¾ Ileocolic resection is sufficient.

B xx xx xx

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: xx xx xx xx

Ogilvie’s syndrome Mentally-retarded individuals Chaga’s disease Hypothyroidism

xx xx xx xx

Anticholinergic drugs Multiple sclerosis Scleroderma Parkinson’s disease

Recurrence rate

In hydrostatic reduction—10% In open manual reduction—2% In resection—very less 6 mm AP diameter, hyperechoic thickened appendix wall >2 mm—target sign. Appendicolith. Interruption of submucosal continuity. Periappendicular fluid.

B xx xx xx

A

B

Figs. 24.14A and B: Ultrasound showing dilated noncompressible appendix in two different patients.

Score

Migrating pain

1

Anorexia

1

Nausea and vomiting

1

Tenderness in right iliac fossa

2

Rebound tenderness

1

Elevated temperature

1

Leucocytosis with count more than 10,000

2

Shift to left with neutrophilia in peripheral smear

1

Total score

10

Score less than 5: Not sure. Score between 5–6: Compatible. Score between 6–9: Probable. Score more than 9: Confirmed.

Kalam modified Alvarado scoring system (1994) where shift to left is removed. ¾¾ Tzanakis scoring system 2005—lower abdominal tenderness—4; rebound tenderness—3; total count >12,000/ cm—2; USG features—6. ¾¾ RIPASA scoring system (2010)—with 15 parameters. ¾¾ 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 sign—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 obstruction, ureteric stone. ¾¾

Appendices epiploicae are absent in rectum, appendix and caecum.

CHAPTER 24    Appendix

Fig. 24.13: Radio-opaque appendix in a plain X-ray. It could be calcified or have calcified content.

xx

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Different Scoring Systems Used

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Treatment

SRB's Manual of Surgery

Surgery-Appendicectomy:

Approaches 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 laterally). 3. Lanz crease incision centering at McBurney’s point— cosmetically 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.

Fig. 24.15: Approaches for appendicectomy.

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 appendicectomy can 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.

A

B

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.

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B xx xx xx xx xx xx xx xx xx

Complications after appendicectomy

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)—due to injury to ilioinguinal nerve Wound sepsis 10% Faecal fistula Respiratory problems and DVT

Fig. 24.20: Transfixation of the base of appendix using 2 zero vicryl is an important step after secured ligation of appendicular artery.

Fig. 24.19: Gangrenous appendix. It is gently ligated at the base using 2 zero vicryl. It is not buried as stump will be friable (burying is not necessary to any appendicectomy).

B xx xx xx xx

xx xx

Portal pyaemia

It is rare nowadays It is septic portal system thrombosis Commonly seen in immunosuppressed individuals Infection spreads to liver through portal vein causing rapid multiplication of virulent organisms leading into septicaemia (toxaemia with hypotension, tachycardia), jaundice, tender palpable liver. Patient will be drowsy Treatment—antibiotics like cefoperazone, amikacin, metronidazole, meropenem; fluid management; ventilator support It carries poor prognosis

Fig. 24.21: Faecal fistula after appendicectomy. Most of the time faecal fistula subsides by conservative treatment unless there is distal obstruction or specific causes like Crohn’s, tuberculosis or malignancy.

Perforated appendix rarely causes pneumoperitoneum.

CHAPTER 24    Appendix

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.

936

Contd... xx

SRB's Manual of Surgery

xx xx xx xx xx

Fig. 24.22: Wound infection after appendicectomy in patient with burst appendicitis.

Troubles in Appendicectomy

xx

™™ During surgery if appendix is found normal, other cause for

symptoms should always be looked for like Meckel‘s diverticulum, Crohn‘s disease, ovarian/pelvic causes in females, malignancy, etc. ™™ Appendicular tumour may be found. If it is in the tip, appendicectomy is sufficient. It could be carcinoid tumour. If it is in the base right hemicolectomy is done. ™™ Absence of appendix—a rare occasion can occur. Caecum and taeniae should be traced properly before finalising it. ™™ Appendicular abscess/pelvic abscess formation. ™™ Malignancy in the caecum is identified on table—right hemicolectomy should be done. ™™ If Crohn‘s disease is identified during surgery, appendicectomy can be done with care, if base of the appendix is normal. But in rare occasion where appendix is involved by Crohn‘s disease, appendicectomy should not be done but treated only with antibiotics and steroids, otherwise fistula can develop.

xx xx xx xx

xx xx

xx

xx

INCIDENTAL APPENDICECTOMY ™™ Here removal of normal appendix is done at laparotomy for

other conditions, e.g. hysterec­tomy.

™™ It is done in vague lower abdominal pain of doubtful severity. ™™ It is a useful procedure to tackle ‘Munchausen syndrome,

i.e. the patient is always worried of pain abdomen and gets relieved after the procedure (psychological benefit). Baron Hieronymus Munchausen (1797) was a German officer who fought with Russians against Turks and returned to tell tall stories. Patient presents with various stories of pain, bleeding, earlier medical or surgical therapies. ™™ It is done along with Ladd’s procedure for malrotation. ™™ It is also done during on table colonic lavage (Doodleys lavage).

B xx xx

xx

xx

xx xx xx

xx

Remember

Appendicitis is common in white races, young males and in those who are on westernised diet It most commonly affects individuals of age group 10-20 years. 2/3rd will develop perforation due to rapid progression and poor localisation Gangrene, perforation and peritonitis are rare in nonobstructive type; but recurrent appendicitis of nonobstructive origin can cause perforation Contd...

xx xx

xx

Pneumoperitoneum is not common in appendicular perforation Appendicular artery which is an end artery can undergo infective thrombosis and can cause gangrene and perforation In retrocaecal appendicitis rigidity is not common; psoas spasm is known to occur Pelvic and post-ileal appendicitis can cause diarrhoea. Post ileal appendicitis is difficult to diagnose It is difficult to remove subhepatic appendix through McBurney’s incision Pain will be above and lateral in appendicitis in pregnant women. Appendicitis is the most common acute abdominal condition in pregnancy (1:1500 pregnancies). Incidence of foetal loss is 5% without perforation and it becomes 20% if there is perforation. It is better to do laparotomy to remove the appendix in pregnancy In elderly atypical features are more common and so diagnosis is often missed. Gangrene and perforation are common in elderly. Often it mimics subacute obstruction In obese patients diagnosis is often difficult Appendicitis is rare before 2 years. But when it occurs perforation and peritonitis are common carrying poor prognosis Negative appendicectomy—incidence is 30% Reginald Fitz of Boston coined the term appendicitis. McBurney described clinical features; Claudius Amyand (1736) did first appendicectomy Appendix is found on the left side in situs inversus patient. Situs inversus may be both thoracic and abdominal or only abdominal Acute pancreatitis (straw/haemorrhagic chicken broth fluid), DU perforation (bile fluid), perforated Meckel’s diverticulum, twisted ovarian cyst/ectopic pregnancy (bloody fluid) are important lifethreatening conditions which may be missed for appendicitis and patient might undergo appendicectomy as a wrong procedure in these patients Simple appendicitis is one where the symptoms are of less than 48 hours duration with imaging studies showing appendicitis without abscess or phlegmon Chronic appendicitis’ earlier this term was not used, but is presently accepted terminology; few attacks of recurrent appendicitis will lead into chronic appendicitis. It presents with episodic often vague discomfort with colicky pain in RIF, anorexia, malaise, pain with movement and is often called as grumbling appendicitis. TC, US, CT scan may be normal in these patients Perforation rate in appendicitis is 25% in general; in children and elderly it becomes 45–50%. High fever more than 102°F, TC >18,000/- are suspected features of rupture Mortality rate of appendicectomy is 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... xx xx

CHAPTER 24    Appendix

xx

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

™™ It is not done in Crohn’s disease (during acute phase), post-

radiation, immunosuppression, aortoiliac grafts.

APPENDICULAR MASS (Periappendicular Phlegmon) ™™ It is the localisation of infection occurring 3 to 5 days after

an attack of acute appendicitis.

Fig. 24.24: Appendicular mass is formed by dilated ileum; greater omentum; inflamed appendix and caecum. It is resonant, smooth, firm, and tender with well defined borders which does not move with respiration and does not have mobility. ¾¾ ¾¾ ¾¾

Fig. 24.23: Appendicular mass—a well localised one. ™™ Inflamed appendix, greater omentum, oedematous caecum,

parietal peritoneum and dilated ileum (ileus) forms a mass in the right iliac fossa. ™™ This mass is tender, smooth, firm, well localised, not moving with respiration, not mobile, all borders well made out (well localised) and resonant on percussion. Patient may have fever and features of toxicity.

B xx xx xx xx

Differential diagnosis

Carcinoma caecum Crohn’s disease Ovarian disease Twisted ovarian cyst

xx xx xx xx

Actinomycosis Mesenteric lymphadenitis Ruptured ectopic pregnancy Ileocaecal tuberculosis

™™ Investigations: TC is increased; Ultrasound confirms the

mass.

™™ Treatment Conservative (Ochsner-Sherren Regimen), as nature has

already localised the infection, if now disturbed will cause faecal fistula. Includes observation: ¾¾ Temp, BP, pulse chart. ¾¾ Marking the mass to identify the progression/regression. ¾¾ Antibiotics (ampicillin, metronidazole, genta­micin, or other drugs given depending on severity and requirement).

IV fluids. Analgesics. Initial nasogastric aspiration. –– Patient usually shows response by 48 to 72 hours and mass reduces in size, temperature and pulse becomes normal. Appetite is regained. 90% of patients respond to conservative therapy. –– Patient is discharged and advised to come for interval appendicectomy after 6 weeks.

Contraindications for Ochsner-Sherren regimen 1. When diagnosis is in doubt. 2. In acute appendicitis in children and elderly. 3. In burst, gangrenous appendicitis. 4. In patients in whom diffuse peritonitis sets in. Criteria to discontinue Ochsner-Sherren regimen xx Patient becomes more toxic (tachycardia, high fever) xx Persistent vomiting xx Increase or spread of pain abdomen (means onset of diffuse peritonitis) xx Increased size of the mass xx Suppuration (abscess formation) in the mass In these patients the regimen is discontinued. The patient is taken for immediate surgery, either through laparotomy or through classic approaches

B

APPENDICULAR ABSCESS ™™ It occurs due to suppuration in an acute appendicitis or

suppuration in an already formed appendicular mass.

™™ Abscess commonly occurs in retrocaecal region but often

can occur in subcaecal, preileal lumbar or postileal regions.

™™ Pelvic abscess is also common after an attack of acute

appendicitis.

Poverty knows how extremely expensive it is to be poor.

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FAECAL FISTULA AFTER APPENDICECTOMY Causes

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™™ It can occur when appendicectomy is done in gangrenous/

perforated/friable base appendix. ™™ It can occur after drainage of appendicular abscess. ™™ It can occur if appendicectomy is done/attempted in appen-

dicular mass. ™™ If there is underlying additional pathology like Crohn‘s

disease/carcinoma/ileocaecal tuberculosis/actinomycosis during appendicectomy, fistula can occur.

Features ™™ Faeculent, foul smelling discharge from either main wound

Fig. 24.25: Appendicular abscess. Swelling with ill-defined lower border.

or drain site.

Features ™™ High fever, features of toxicity, tender, smooth, dull (to

percuss), soft swelling in right iliac fossa which lies towards right lateral and lower side with clear upper margin but indistinct lower margin. ™™ Ultrasound confirms the diagnosis. ™™ Treatment ¾¾ Antibiotics are started. ¾¾ CT-guided aspiration or catheter drainage is done often as initial therapy. ¾¾ Under G/A, incision is made in the lower lateral aspect of the swelling above the inguinal ligament. Skin, external oblique muscle is cut. Abscess cavity is opened and pus is drained extraperitoneally, which is sent for culture and sensitivity. Wound is closed. A drain is placed through a separate incision. Antibiotics are continued. ™™ Interval appendicectomy is done after 3 months. Pelvic abscess is drained per-rectally or through posterior colpotomy (in females).

Fig. 24.27: After appendicectomy fistula can occur. Note the faecal discharge. ™™ Features of infection; Skin excoriation; Features suggestive

of cause. ™™ Investigations

CT fistulogram to delineate the track. CT scan abdomen to find out the other pathology. ¾¾ Other relevant investigations, Hb%, albumin level, etc. ™™ Treatment ¾¾ Conservative—antibiotics, IV fluids, dressing, zinc oxide cream over the skin, observation. ¾¾ Most of the time fistula subsides provided there is no distal obstruction by adhesions or kinking or specific causes like carcinoma or tuberculosis. ¾¾ If persists even after 6 weeks, resection of ileocaecal segment and anastomosis is done. ¾¾ ¾¾

MUCOCELE OF APPENDIX ™™ It can be neoplastic or non-neoplastic. ™™ It occurs when proximal end of the lumen of appendix gets

Fig. 24.26: Different sites where abscess can occur after appendicitis retrocaecal, appendicular; pelvic; subphrenic; preileal and lumbar. Portal pyaemia can occur with multiple pyaemic abscesses in the liver.

slowly and completely occluded, usually by a fibrous stricture causing collection of sterile fluid (mucus) in the lumen. It is a retention cyst.

™™ Appendix is grossly enlarged with features of sub-acute

appendicitis.

™™ Mucocele can get infected leading to empyema of appendix. ™™ Rupture of mucocele can lead to pseudomyxoma peritonei.

Neoplastic type causes generalised pseudomyxoma peritonei; non-neoplastic type causes localised pseudomyxoma peritonei. (Other cause for pseudomyxoma peritonei is ruptured mucinous carcinoma of ovary). ™™ Often mucocele of appendix is also caused by a mucus secreting adenocarcinoma and if it is so right hemicolectomy is done. ™™ Clinical features: Colicky pain in right iliac fossa, Tenderness in the right iliac fossa. ™™ Investigations: Ultrasound abdomen. ™™ Treatment: Appendicectomy.

B xx xx xx xx xx xx xx xx

Pseudomyxoma peritonei

Jelly like mucoid yellowish-brown substance accumulates in peritoneal cavity Due to ruptured adenocarcinoma appendix/mucocele or mucinous carcinoma of ovary Common in females Painless progressive distension of abdomen with intestinal obstruction occurs eventually Shifting dullness is absent Surgical debulking, oophorectomy, appendicec­tomy, omentectomy are often done Chemotherapy is useful—cisplatin Carries poor prognosis

Note: Pseudomyxoma peritonei is presently consi­dered to be due to neoplastic adenocarcinoma of appendix with gelatinous fluid collection in the peritoneal cavity. It is also seen in cystadenocarcinoma of ovary. Treatment is surgery and chemotherapy.

NEOPLASMS OF THE APPENDIX ™™ It is rare; It is often postappendicectomy histological diagnosis. ™™ Cystic neoplasms of appendix: Simple cyst (non-neoplastic

mucocele); mucinous cystadenoma; mucinous cystadenocarcinoma (most common form of cystic neoplasms); pseudomyxoma peritonei. Simple cyst is non-neoplastic obstruction of the lumen and is less than 2 cm in size which contains mucin. Mucinous cystadenoma attains progressively large size of up to 8 cm with CT showing calcification of the wall. Laparoscopic appendicectomy is not used in mucinous cystadenoma. Hemicolectomy is done in mucinous cystadenocarcinoma and cystadenoma of large size and if base is involved. ™™ Carcinoid tumour is the most common type. It is less aggressive. It is often incidentally found. It is arising from Kulchitsky cells in crypts of Lieberkuhn (argentaffin tissue). It is ten times more common than other types (One in 400 appendices). Commonly its location is in the tip. 75% are less than 1 cm; 15% are 1–2 cm; 10% are >2 cm in size. It stains chromograninB immunohistochemically. Distant and

A

B

Figs. 24.28 and B: Carcinoid tumour in two different patients. If it is in the tip or away from the base, then appendicectomy is sufficient. If it is in the base or extending into the caecum then right hemicolectomy is needed. ™™ Primary adenocarcinoma of the appendix is rare. It can be

mucinous (common) or colonic (less common) type. Acute presentation as appendicitis is common in colonic type. It is staged as Duke’s staging A, B, C and D. 5-year survival rate for each is 100%; 65%; 50% and 5% respectively. Mucinous type has got better prognosis. 5-year survival for mucinous type is 70% and colonic type is 40%. Mucinous type can rupture into the peritoneal cavity and can cause pseudomyxoma peritonei.

B xx xx xx

Tumours of the appendix

Carcinoid tumour—most common site is appendix Primary adenocarcinoma of appendix Mucocele of appendix leading into pseudomyxoma peritonei

LAPAROSCOPIC APPENDICECTOMY This is newer, popular and ideal method of appendicectomy. It has become gold standard method of treatment.

Great people neither boast nor indulge in tall talk. Does diamond has to announce that it is worth a fortune?

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CHAPTER 24    Appendix

nodal spread occurs if tumour is more than 2 cm. Carcinoid of appendix may be goblet cell type or classical type histologically. Goblet cell has got more mortality than classic type. Treatment is appendicectomy. Right hemicolectomy is done if base is involved or size is more than 2 cm or nodes are involved. 5-year survival is 90%.

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Advantages ™™ Diagnosis is confirmed. ™™ Other parts of the abdomen are visualised. ™™ In females pelvic structures are assessed properly. ™™ Trauma of access is less. ™™ Faster recovery. ™™ Laparoscopic appendicectomy is definitely better when-

ever there is vague abdominal pain; atypical pain; situs inversus; in women; subhepatic appendix and as interval appendicectomy.

Disadvantages ™™ Technical difficulties especially in burst appendix. ™™ Cost factor and availability.

one on left side and another on the lower midline. One of the working ports can be 10 mm in difficult appendectomies. ™™ Pneumoperitoneum is created using CO2. ™™ Appendix is held with grasper or Babcock’s forceps. Mesoappendix is cauterised by bipolar or unipolar cautery. ™™ Appendix is dissected up to the base of the appendix. ™™ Base of the appendix is ligated with loop ligature. Intracorporeal ligature also can be placed using vicryl 2 zero suture material. Appendix is removed through 10 mm working port along with reducer. Often retrieval bag can be used to remove the appendix. ™™ Umbilical port is closed in two layers. Other ports are closed by skin sutures. If gangrenous or burst appendix drain can be placed through one of the ports. ™™ Oral food is started in 12 hours.

Note: Consent for conversion should be taken.

Technique ™™ Procedure is done under general anaesthesia. Head down

position with right tilt is needed. Surgeon and camera man stands on the left side. Scrub nurse on the right side. Monitor is kept on the foot end right side. ™™ 10 mm camera port is placed at the umbilicus. Working ports are two 5 mm, one on each side of lower abdomen or

Fig. 24.29: Different possible port placements for appendicectomy.

A

B

Figs. 24.30A and B: Laparoscopic appendicectomy. Note the mesoappendix is being cauterised. Ligation of the appendix is done using vicryl (Courtesy: Dr Keshava Prasad, MS, KMC, Mangaluru).

Complications ™™ Injury to bowel, vessels while passing the ports. ™™ Complications of pneumoperitoneum. ™™ Accidental cautery injury to bowel, vessels and other vital

structures.

™™ Bleeding; Bowel perforation, peritonitis.

Fig. 24.31: Laparoscopic assisted appendicectomy.

™™ Ligature slipping, leak, peritonitis, fistula formation.

An essential aspect of creativity is not being afraid to fail.

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CHAPTER 24    Appendix

Laparoscopic assisted appendicectomy can be done if caecum is mobile without much adhesions and mobilised appendix can be delivered through the 10 mm umbilical port. As in regular laparoscopic procedure, appendix is identified and mobilised. Appendix which is not friable and not turgid can be delivered through umbilical port gently. During this procedure gas flow should be stopped so that all gas in the cavity will be empty. Mesoappendix is ligated. Appendix is transfixed like in open method and removed. Stump is pushed back into the place. Telescope is passed again to confirm the position and security of the stump. Advantage is that it is faster.

chapter

25

Rectum and Anal Canal C hapter 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 xx External Piles ·· Anal Fissure xx Sentinel Pile ·· Anorectal Abscess ·· Fistula-in-Ano xx Low-Level Fistula xx High-Level Fistula

·· Anorectal Strictures ·· Condyloma Acuminata ·· Anal Intraepithelial Neoplasia ·· Malignant Tumours of Anal Area ·· Sacrococcygeal Teratoma ·· Anal Incontinence ·· Descending Perineal Syndrome ·· Proctitis ·· Proctalgia Fugax ·· Hidradenitis Suppurativa of Anal Region ·· Pruritus Ani ·· Gastrointestinal Haemorrhage xx Upper GI Bleed xx Lower GI Bleed xx 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, Denonvillier’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 ¾¾ Pelvic floor and muscles. ¾¾ Fascia of Waldeyer: It is the condensation of pelvic fascia behind the ectum, contains superior rectal vessels and lymphatics. ¾¾ Lateral ligaments of rectum: It is the condensation of pelvic fascia, attaches rectum to the posterolateral wall of lesser pelvis.

¾¾ Denonvillier’s fascia: It is the fascial condensation which

separates rectum from prostate in males. ¾¾ Perineal body: It is in front of the anorectum.

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 pudendal 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 rectum— nervi erigentes. Pudendal 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. ™™ Ischiorectal fossa—pyramidal shape; 5X2 cm sized; Alcock’s canal carries pudendal vessels and nerve; lunate fascia lines above. ™™ Parts ¾¾ Upper part—15 mm; anal columns of Morgagni, anal valves, anal crypts, anal glands, pectinate/dentate line. ¾¾ Middle part—15 mm—transition pectin, white line of Hilton; pale, thin, glossy, squamous epithelium without sweat glands. ¾¾ 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 pudendal nerve. The venous drainage above this line is by inferior mesenteric and portal circulation, whereas below to

Fig. 25.1: Interior of the anal canal and it's relations. ™™ Anal folds and columns; anorectal junction and ring; inter-

sphincteric space/plane; intersphincteric groove; Hilton’s line; mucosal suspensory ligament of Park’s—are important anatomical parts. ™™ Internal anal sphincter: Involuntary—smooth muscle—2–5 mm thickness; covers upper 3/4th of the anal canal—formed by downward extension of circular muscle of rectum, under control of autonomic nervous system. ™™ External anal sphincter: Deep, superficial, subcutaneous parts. Voluntary—supplied by pudendal nerve; it forms the collar around the mid and lower anal canal. It ssurrounds the internal and continuous with the levator ani muscle. External sphincter forms the triple loop of Shafik (puborectalis part + deep and superficial parts of external sphincter) which is very important in continence system.

Fig. 25.2: Sphincters of anal canal.

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

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™™ Arterial supply: Rectum is supplied by rich network of vessels

systemic venous circulation. Internal haemorrhoids develop above this line. ™™ Surgical anal canal is 4.5 cm extends more superior; anatomical anal canal is 3.0 cm. It is important in assessing malignancy extension and its dissection.

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™™ Blood supply: Arterial—from inferior rectal artery. Venous—

from 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’.

Fig. 25.3: Triple loop of Shafik: (A) Puborectalis; and (B) Deep part; (C) Superficial part; (D) Subcutaneous part of external anal sphincter. ™™ Levator ani: Conglomeration of three striated muscles—

puborectalis; pubococcygeus; iliococcygeus. Puborectalis— innermost; 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.

Fig. 25.6: Nerve supply of rectum and anal canal

Continence Mechanism

Fig. 25.4: Levator ani with puborectalis.

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—Holschneider. Anorectal continence is complex dynamic process involving the striated muscle complex (levator ani + 3 parts of external anal sphincter); internal sphincter (smooth muscle complex); perineal 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 mmHg of pressure and internal sphincter 10 mmHg. S2 component of the pudendal 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 abdominal muscles); completion (rectum contracts, internal and external sphincters relax).

PER-RECTAL EXAMINATION (Digital Examination of the Rectum—Refer Chapter 16)

Annual sigmoidoscopy for all, after their fortieth birthday: something to look forward to. — Henry George Miller, 1968

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.

B

Positions for Per-rectal Examination

Right lateral position xx Lithotomy position Left lateral position xx Knee-elbow position xx Dorsal position in ill-patients xx Picker position: Patient in standing position leans forward by grasping 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. Per-rectal examination is contraindicated in acute fissure-in-ano. xx xx

Fig. 25.8: Rigid sigmoidoscope with inflation balloon and biopsy forceps. ™™ It is used to visualize rectum and sigmoid colon, take biop-

PROCTOSCOPY (Kelly’s) ™™ Indications ¾¾ Diagnostic—piles, fissure-in-ano, polyps, stricture, etc. ¾¾ Therapeutic—injection therapy for partial prolapse or

piles, cryotherapy for piles, polypectomy, biopsy for carcinoma rectum or anorectum.

sies 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 procedure is done. Note: Precaution: Care should be taken in acutely inflamed sigmoid colon, because chance of perforation is high.

A

B

Figs. 25.7A and B: Types of proctoscopes: (A) Non-illuminating; (B) Illuminating.

™™ Types: Illuminating; Nonilluminating. ™™ 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 sigmoidos-

copy, 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. Often 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.

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CHAPTER 25    Rectum and Anal Canal

Note: No abdominal examination is complete without a per-rectal examination. ™™ It is done to palpate carcinoma rectum; Stricture rectum;

SIGMOIDOSCOPY

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negotiation of the colonoscope. Technique differs in patients after haemicolectomy or through colostomy. ™™ Indication ¾¾ Bleeding per rectum, resistant anaemia. ¾¾ To take biopsies from different parts of the bowel. ¾¾ To identify synchronous growths, ulcerative colitis. ¾¾ To remove polyps. ¾¾ When barium enema shows irregularity. ¾¾ For therapy—colonoscopic polypectomy, dilatation of stricture colon, fulgaration. xx xx xx xx

™™ 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. Fig. 25.10: Large rectal polyp which has come out of the anal canal. It is potentially malignant.

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 of any man that was cured…but I have known many that died of the foresaid 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.

Aetiology ™™ Red meat and saturated fatty acids increase the risk. ™™ High fibre diet reduces the risk.

A

B

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

A

B

Figs. 25.9A and B: Rectal polyp—it is premalignant condition.

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.

Histologically: It is adenocarcinoma which may be: xx xx xx

Well-differentiated—10% Moderately differentiated—65% Undifferentiated—25%

B

Colloid carcinoma of the rectum

It is 12% common in young people Types xx Primary and secondary xx 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.

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. 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

B

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: sm1—upper (inner) 1/3rd; sm2—middle 1/3rd; sm3—lower 1/3rd. 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. Perineural spread carries poor prognosis.

™™ Spurious diarrhoea: It occurs in early morning due to over-

night 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 ¾¾ Proctoscopy; Sigmoidoscopy. ¾¾ Biopsy using Yeoman’s forceps. ¾¾ Barium enema in case of FAP and synchronous growths. ¾¾ Colonoscopy is ideal to rule out presence of any synchronous growths proximally (5%) or polyps. ¾¾ 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. ¾¾ Ultrasound abdomen—to look for secondaries in liver, ascites.

• • • • • •

Features ™™ Bleeding per rectum/anum (may mimic haemor­rhoids)—

earliest symptom.

Fig. 25.12: Air-contrast CT of colon and rectum showing significant narrowing in rectosigmoid junction. ¾¾ 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

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Gross: It can be: xx Ulcerative; Papilliferous; Infiltrative xx Annular: It is common in rectosigmoid junction. xx Diffuse type: Often observed in patients with ulcerative colitis which carries poor prognosis

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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.

A

B

Figs. 25.15A and B: MRI showing carcinoma of rectum and adjacent structures. MRI is good method to evaluate the spread and staging. ¾¾ 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.

Fig. 25.13: CT picture of carcinoma rectum. CT is useful in evaluating the extent, nodal status and operability.

B xx xx xx

Differential diagnosis

Inflammatory stricture Amoebic granuloma Tuberculosis

xx xx

Carcinoid Solitary ulcer syndrome

Treatment Surgery

Fig. 25.14: CT scan showing carcinoma of rectum. ¾¾ Endorectal ultrasonography—very useful to assess

the local extent of the tumour. Transrectal ultrasound (TRUS)/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. ¾¾ Endorectal 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. ¾¾ Fluorine—18 fluorodeoxyflucose 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. Preoperative 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 fascial 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 perineal 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 tumour 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.

¾¾ It is a sharp dissection (not blunt) in avascular areolar

and bilateral oophorectomy may be needed in T4 lesions to achieve surgical resection. Removal of uterus and ovaries prevents patient from developing possible associated cancers of these organs. Carcinoma rectum also spreads to ovaries commonly which can be prevented by oophorectomy. ™™ Often resection of liver secondaries can be undertaken in selected patients when one lobe is involved or solitary secondaries are present. ™™ Laparoscopic APR/AR is becoming popular. Features are: ¾¾ Dissection will be more meticulous. ¾¾ Less blood loss, less postoperative pain. ¾¾ Early bowel function. ¾¾ Clearance is same as open method in relation to primary tumour and nodes. ¾¾ Short hospital stay, mortality and morbidity are similar to open method. ¾¾ Port site recurrence chances are 0.5–2% (Earlier it was higher; now it has reduced due to proper technique, careful handling of the specimen, specimen isolation prior to extraction, trocar site irrigation with cytotoxic agents and povidone iodine). ™™ For carcinoma rectum presenting with obstruction, an initial proximal colostomy is done. Neoadjuvant chemoradiation is given. Patient is reassessed for operability. Then APR is done with permanent colostomy. ™™ Incidence of local recurrent rectal cancer is 30%. ¾¾ Eighty per cent of local recurrence occurs within 2 years of surgery. ¾¾ Common site of recurrence is in the pelvic wall. It also can occur at distal anastomotic margin. ¾¾ Intractable pelvic pain, urinary symptoms, sepsis, bleeding, perineal sinus, swelling and induration, bowel disturbances are the features. ¾¾ It is evaluated by CEA, biopsy, CT abdomen, MRI of pelvis and PET scan. ¾¾ It is often difficult to manage. ¾¾ Incidence of recurrence will come down to 5% in proper TME. ¾¾ Palliative chemoradiation, end colostomy, ureteral stenting are the palliation. RT controls pain and bleeding. ¾¾ Extensive radical surgery like removal of tumour with pelvic structures can be undertaken with diversions but with a high mortality rate and failure rate. ™™ Proper preoperative bowel preparation is a must in all rectal cancers which reduces the postoperative problems like sepsis, leak and increases the early chance of recovery. ¾¾ Bowel preparation by polyethylene glycol; electrolytes orally; bowel wash per anally; bowel antiseptics like neomycin 1 g tid previous day/erythromycin 1 g tid/ metronidazole tid orally. ™™ Systemic antibiotics like cefazolin, metronidazole, gentamicin. ™™ Prophylactic heparin or low molecular weight heparin.

Never insult the vagina by examining the rectum first. —An old axiom

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plane between fascia of rectum which encroach the mesorectum and parietal pelvic wall fascia. ¾¾ Mesorectum should not be breached. ¾¾ Absolute haemostasis, preservation of autonomic nerves and dissection under vision are the essential principles. ¾¾ Both layers of membranous anterior Denonvillier’s fascia should be dissected off the prostate and seminal vesicles in male to have proper clearance. ¾¾ In TME for middle and lower rectum, entire mesorectum should be removed. ¾¾ For upper rectal tumours, TME is done 5–6 cm below the lower margin of the tumour. ¾¾ 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 bed with 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 5 cm. T4: Tumour of any size invading adjacent organ(s), such as vagina, urethra or bladder. Nx: Regional nodes cannot be assessed. N0: No regional nodes. N1a: Metastasis in inguinal, mesorectal or internal iliac or external iliac nodes. N1b: Metastasis in external iliac nodes. N1c: Metastasis in external iliac nodes with any of N1a nodes. Stage groups Stage 0: Tis, N0, M0. Stage I: T1, N0, M0. Stage IIA: T2, N0, M0. Stage IIB: T3, N0, M0. Stage IIIA: T1-2, N1, M0. Stage IIIB: T4, N0, M0. Stage IIIC: T3-4, N1, M0. Stage IV: Any RT, Any N, M1.

™™ Squamous cell carcinoma of anal canal, usually present as

a fungating or ulcerative growth, which spreads to inguinal lymph nodes. ¾¾ Biopsy and FNAC of lymph nodes are the essential investigations. ¾¾ 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.

Classification of tumours of anal area

It can be: Benign; Malignant It also can be: xx Tumour of the anal canal (proximal to dentate line)—SCC, adenocarcinoma, melanoma xx Anal margin tumour (distal to dentate line)—ANI, Bowen’s disease, Paget’s disease, BCC, anal margin SCC

Nigro Regime

Types 1. Squamous cell carcinoma is the commonest type. Predisposing causes: Papilloma, irradiation, derma­titis, long standing fistula-in-ano. 2. Basaloid carcinoma—it is rare, non-keratinising squamous cell carcinoma. Highly malignant. 3. Muco-epidermoid carcinoma—arises near squamocolumnar junction. 4. Basal cell carcinoma. 5. Melanoma—blue/black in colour mistaken for thrombosed pile—poor prognosis (5 years—10%). 6. Adenocarcinoma from the anal glands in a pre-existing fistulain-ano.

Nigro Regime for Anal Carcinoma (Norman Nigro, et al. 1974) Initial radiotherapy for 3 weeks 3000 rads (30 Gy total) to perineum and pelvis Then chemotherapy—5 FU, for 4–5 days; is a radiosensitizer, started on 1st day of RT as 1000 mg/m2 continuous infusion. Mitomycin C is 15 mg/m2 as single dose on 1st day of RT Later after 3 weeks abdominoperineal resection (APR)

B xx xx

xx

¾¾ Chemoradiation is becoming popular for carcinoma of

anal canal. ¾¾ Drugs used for chemotherapy are 5 FU, bleomycin, vincristine, adriamycin.

Two types of mankind are there; Hosts and Guests; on either way you are in trouble.

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CHAPTER 25    Rectum and Anal Canal

to the anal verge distally, and include the anal transition zone and the non-hair or gland-bearing squamous tissue comprising the anoderm. The anal margin is defined as the hair and gland-bearing skin lateral and up to 5 cm away from the anal verge. This distinction between anal canal and anal margin malignancies is important as anal canal lesions are more aggressive and are treated differently. The incidence of anal canal lesions is significantly higher (5 times) than that of anal margin lesions. ™™ Epidermoid carcinoma is the most common type of anal canal malignancy—85% of all lesions. This includes squamous cell carcinoma and its variants—cloacogenic, basaloid, and transitional carcinomas. ™™ Twenty to twenty five per cent cases metastases occur to inguinal lymph node. ™™ Anal canal tumours are common in females.

Features

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¾¾ If persistent or recurring disease presents following

combined modality therapy (CMT—chemoradiotherapy), APR with colostomy is indicated. ¾¾ Neoadjuvant chemotherapy using 5 FU, cisplatin and mitomycin C is also commonly used. ¾¾ In advanced growths radiotherapy is the only treatment. ™™ All other tumours: Abdominoperineal resection with permanent colostomy is done.

B xx xx

Paget’s

Paget’s disease of breast Paget’s disease of anal margin—of apocrine glands

xx xx xx

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 Kamath, 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 ™™ Continence of anal canal is maintained by two factors: ¾¾ Normal rectal and colonic pressure and activity. ¾¾ Normal pelvic floor function.

A

™™ Types ¾¾ Urge incontinence—here rectal and colonic pressure and

B

Figs. 25.85A and B: Sacrococcygeal teratoma, typical site (Courtesy: Professer Suresh Kamath, MS, Mangaluru). ™™ X-ray and CT scan are must. ™™ Treatment: Excision soon after birth.

Complications

Differential diagnosis

• • • •

• Sacral meningocele • Sacral chordoma • Postanal dermoid

Ulceration Infection Urinary obstruction Malignant changes

activity is increased but normal pelvic floor. ¾¾ True incontinence—here rectal and colonic pressure and activity is normal but defective pelvic floor function. ¾¾ Full incontinence—here rectal and colonic pressure and activity is reduced and also defective pelvic floor function. ¾¾ Temporary—treated by reassurance. Often seen after Lord’s dilatation. ¾¾ Permanent—needs definitive therapy. ™™ Causes

B xx xx xx xx xx xx xx

Causes of anal incontinence

Denervation—spinal injury, spina bifida Damage—childbirth, wounds, surgeries Descent—rectal prolapse, perineal descent Debility—old age, diseases Destruction—RT, malignancy Dementia—senility, psychosis Deficiency—congenital anomalies

¾¾ Irritable bowel syndrome, severe diarrhoea. ¾¾ Prolapsed piles, rectal prolapse. ¾¾ Old age, malnutrition, debilitating illness. ¾¾ Trauma, surgeries, injury during childbirth in females. ¾¾ Spina bifida, spinal tumours, spinal injuries and surgeries. ¾¾ Malignancy, postirradiation. ¾¾ Psychological causes.

B xx xx xx xx

Evaluation of the patient

For specific causes Anorectal manometry Per-rectal examination Sigmoidoscopy

xx xx xx

Electromyography Defaecography Perineometer to assess level and angle of anorectal junction

™™ Treatment ¾¾ Suturing of the torn sphincter. ¾¾ Repair of puborectalis muscle and plication of external

sphincter.

¾¾ Encircling operations around anal canal to give support

using gracilis sling or mersiline sutures. ¾¾ Electrical stimulation of the puborectalis. ¾¾ 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 pres-

sure 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, perineal descent can occur with obliteration of the normal concavity of the perineum. It is called as descending perineal 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. ™™ Defecography is 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. ™™ Total pelvic marlex mesh repair; transcoccygeal posterior hitching of the rectum (Kraske); correction of cystocele, rectocele and enterocele. ™™ Results are not very good as recurrence or residual problems may persist.

It is inflammation of rectal mucosa often with the inflammation of colon and anal canal. ™™ Types ¾¾ Acute or chronic. ¾¾ Nonspecific—common. ¾¾ Specific –– Bacillary dysentery. –– Amebic proctitis—common. –– Combined amoebic and bacillary. –– Gonococcal proctitis. –– Lymphogranuloma inguinale (LGV). –– Tuberculous proctitis. –– Bilharzial proctitis due to schistosoma haema­tobium. –– Enema induced proctitis especially of herbal enemas. ¾¾ Ulcerative proctocolitis as part of ulcerative colitis.

B xx xx xx xx

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 ¾¾ Sigmoidoscopy is more relevant than just proctoscopy. ¾¾ Stool study, stool culture; Mucosal biopsy; Serological

tests. ¾¾ Relevant investigations like ESR, blood smear, and chest

X-ray. ™™ Treatment ¾¾ Antibiotics, antiamoebic drugs like metronidazole. ¾¾ In severe cases, retention enema using metronidazole, prednisolone, salazopyrin. ¾¾ IV fluids, IV antibiotics and IV metronidazole are often required. ¾¾ Treating the specific causes like tuberculosis, gonococcal infection and bilharzial infection.

PROCTALGIA FUGAX ™™ It is sudden severe recurring pain in the rectum of unknown

cause with segmental pubococcygeal spasm.

Features ™™ It is common in young people may be due to stress, straining. ™™ Common at night, starts suddenly, lasts for few minutes and

then subsides spontaneously. ™™ 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.

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¾¾ Congenital anomalies.

PROCTITIS

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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 involvement → secondary infection (Staphylococcus aureus, streptococci) → skin oedema, multiple raised pustules → 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: ¾¾ Weight reduction; proper hygiene. ¾¾ Antibiotics; analgesics. ¾¾ Incision and drainage of abscess. ¾¾ Laying open of all communicating tracks and regular dressing. ¾¾ Radical local excision of entire apocrine bearing perineal skin with reconstruction using flap. ¾¾ Recurrence is known to occur.

PRURITUS ANI ™™ It is intractable itching in and around anal canal. ™™ Skin is reddened, hyperkeratotic, cracked and moist. ™™ Causes ¾¾ Poor hygiene. ¾¾ Anal discharge due to fissure/fistula/piles/warts/polyp. ¾¾ Trichomonas vaginalis infection of vagina in females. ¾¾ Parasites; Epidermophytosis; Allergic cause; Dermatitis/

psoriasis. ¾¾ Intertrigo/erythrasma (Corynebacterium minutissimum). ¾¾ 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) 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.

Fig. 25.88: Anatomy of ligament of Treitz.

Upper GI Bleed It is considered as: Variceal; Nonvariceal.

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—anticoagulants, clopidogrel, ecospirin.

B xx xx xx xx xx

Factors which aggravate the bleeding

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 occurs when erosion of gastroduodenal artery or left gastric artery or splenic artery occurs or when bleeding occurs from varices

¾¾ For peptic ulcer, saline wash with 1 : 2,00,000 adrenaline,

Features

™™ Chronic bleed ¾¾ Hypochromic microcytic anaemia, glossitis, koilony­chia,

congestive cardiac failure; Mortality in upper GI bleed is 10%. ™™ Investigations ¾¾ Gastroscopy to see the spurting vessel, oozing, clot in the ulcer, collected blood in the lumen of the stomach. ¾¾ CT angiography of coeliac trunk and SMA. ¾¾ Hb%, packed cell volume, CVP measurement, blood grouping and crossmatching. Ultrasound abdomen. ¾¾ LFT; prothrombin time; platelet count; blood urea and serum creatinine; serum electrolytes. ¾¾ Modified Forrest classification (refer Chapter 20— Stomach) and Rockall scoring system (better) is used. In Rockall scoring system parameters used are—age [60 (0), 60–79 (1), >79 (2) Years]; shock [none (0), pluse >100 (1), pluse >100 with hypotension (2)]; comorbidity [none (0), IHD (2), renal/liver failure/advanced maligancy (3)]; condition diagnosed [Mallory Weiss (0), all but mailigancy (1), upper GI maligancy (2)]; endoscopic finding [none (0), dark spot, blood/clot/visible vessel (2)]. Score: 8 high. ™™ Treatment

B xx xx xx

A

B

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. ¾¾ Prognosis ¾¾ Varices and gastric ulcer bleed has higher mortality. ¾¾ Bleeding duodenal ulcer has got better prognosis.

Lower GI Bleed ™™ Bleeding in the GIT below the level of the ligament of Treitz. ™™ Normal faecal blood loss is 1.2 mL/day. A loss more than

10 mL/day is significant.

Treatment of upper GI bleed

General Medical—PPI/tranexamic acid/octreotide Endoscopic

xx xx

CT angiography guided embolisation Surgical

™™ General: IV fluids, catheterisation, Ryle’s tube aspiration,

blood transfusion.

™™ Medical ¾¾ Injection ranitidine IV 50 mg 8th hourly, or famotidine IV,

omeprazole IV, pantoprazole IV. ¾¾ Antifibrinolytics like tranexamic acid, EACA. ¾¾ Somatostatin or octreotide, PPI infusion. ™™ Others ¾¾ Endoscopic therapy (tamponade, laser, haemoclip, banding, sclerotherapy, etc.) is the first line of therapy in all upper GI bleed. ¾¾ CT angiography guided transcatheter embolisation of artery (gastroduodenal) is very useful in bleeding duodenal ulcer if endoscopic therapy fails. ¾¾ Persistent recurrent bleeding needs surgical intervention. ¾¾ For varices, vasopressin, propranolol, isosorbide dinitrate, Sengstaken tube tamponade, sclero­therapy, Boerema-crile operation, Hasaab operation (devascula­ risation with splenectomy), oesophageal transection, Siguira-Futagawa operation or TIPSS.

Fig. 25.90: Causes of lower gastrointestinal bleed. ™™ Causes ¾¾ Angiodysplasia. ¾¾ 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; Intussusception. Tumours, either benign or malignant of colon or small bowel. ¾¾ Meckel’s diverticulum; Mesenteric artery occlusion. ¾¾ Ischaemic colitis; Stercoral ulcer; Infectious colitis. ¾¾ ¾¾ ¾¾ ¾¾ ¾¾

His heart cannot be pure whose tongue is not clean.

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Nd:YAG laser therapy, cautery coagu­lation, thrombin injection, Finney’s pyloroplasty, partial gastrectomy, ligation of gastroduodenal or left gastriartery, cytoprotectant mesoprostil injection. ¾¾ The cause is treated, once acute episode is under control.

™™ Acute bleed ¾¾ Features of shock; Haematemesis; Melaena.

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Occult bleed: >10 mL/day but not revealed Overt bleed: Bleeding which is revealed –– Overt acute –– Overt acute massive (bleed >1.5 litre/day) –– Overt chronic

B xx xx xx

xx

Lower GI bleed can be:

xx xx

Inflammatory bowel diseases Angiodysplasia

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. Bleeding more than 1.5 litres per day is called as acute massive GI bleed.

B xx xx xx xx

xx xx xx

Blood with mucus—colitis, 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

Investigations ™™ Hb%, packed cell volume, ESR.

Common causes

Internal piles Diverticular disease Neoplasia

xx

Acute bleed occurs in:

Mesenteric ischaemia: Angiodysplasia; Ischaemic colitis Meckel’s diverticulum; Intussusception Acute episodes of ulcerative colitis 80% of acute bleed regress spontaneously; 20% will become either massive or recurrent

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 which may reveal polyp, growth, ulcerations. ™™ Haematochezia. ™™ Mass palpable per abdomen in left or right iliac fossa or mass of intussusception.

™™ 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).

A

B

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.

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.

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,

B

Investigations for GI bleed

Blood xx Haematocrit, LFT, blood urea, serum creatinine xx Serum ferritin, iron, binding capacity xx Coagulation profile—platelet count, BT, CT, PT, APTT Occult stool blood test (1–2 mL/day) xx Benzidine test xx Guaiac test xx Haemoccult—guaiac impregnated electrophoresis paper xx Fecal test xx Immunological test Endoscopy xx Gastroduodenoscopy xx Proctosigmoidoscopy xx Colonoscopy—often difficult in acute bleed for clarity visualisation, 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 Contd...

Angiography It identifies when bleeding rate is 0.5 mL/min; useful in active bleed; in therapeutic embolisation, injection vasopressin can be done. Embolisation of small bowel vessel may cause bowel infarction which is dangerous and resection is needed in such situation. Visualisation in angiography can be improved by selective infusion of vasodilators like tolazoline and prostaglandins, using magnification films, using vasoconstrictor drugs xx Angiogragraphic criteria in angiodysplasia are—early and prolonged filling of draining veins; cluster of small arteries; visualisation of vascular tuft xx

Nuclear scintigraphy Identifies 0.1 mL/min of bleed ; Tc sulphur colloid scan is very 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 xx Advantages are—high-sensitivity even with active continued bleed; screening test prior to angiography xx Problems are—no specificity; therapy is not possible xx

Other methods CT angiography; MR angiography xx Aortography for aortoenteric fistula xx Intraoperative localisation—on table enteroscopy; on table Doppler; on table bowel lavage and colonoscopy xx

God gives every bird its food, but he does not throw it into the nest.

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CHAPTER 25    Rectum and Anal Canal

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.

Contd... xx 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

chapter

26 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

C hapter 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 xx Foley’s Catheter xx Malecot’s Catheter xx Red Rubber Catheter ·· Nephrostomy ·· Suprapubic Cystostomy ·· Haematuria ·· Horseshoe Kidney ·· Cystic Diseases of the Kidney

Anatomy of kidney and ureter xx

Polycystic Kidney Disease

xx

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 xx

Recurrence of Stones

·· Staghom Calculus ·· Benign Tumours of Kidney ·· Wilms’ Tumour ·· Renal Cell Carcinoma

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 hilum. 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 sacrospinalis 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.

Capsules of Kidney

Ureter—ANATOMY

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 (Brodel) 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. ¾¾ 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) ™™ Preparation of the patient: Enema/bowel wash/laxative is

Fig. 26.1: Segments of kidney. ™™ Nerve supply: T 10,11,12 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.

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.

CHAPTER 26A    Urology: Kidney

1. Proper capsule—fibrous membrane which can easily be stripped off from the organ. 2. Perirenal fat is in the space of Gerota. 3. Renal fascia of Gerota—has got anterior layer (Fascia of Toldt) and posterior layer (Fascia of Zuckerkandl). 4. Pararenal body of fat.

985

986

B xx

SRB's Manual of Surgery

xx xx xx xx xx

Psoas shadow is obliterated in:

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 IVU: 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.

A

B

Fig. 26.2: Plain X-ray KUB AP-view. Note the psoas shadow.

INTRAVENOUS UROGRAM (IVU) 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 mL/kg body weight, IV urograffin is given (40–50 mL).

C

D

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.

™™ X-ray is taken in 1–5 minutes, which shows the nephro-

graphic 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 mL/kg body weight to get a better film—Infusion IVU. Often diuretics are used in these patients to have better secretion. ™™ Lower abdominal compression is done for 10 minutes to have better definition of calyces, but not done in children and patients with abdominal aortic aneurysm.

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 fossa are nodal mass; carcinoma caecum; tuberculosis; psoas abscess; retroperitoneal 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

™™ Disadvantage: Anaesthesia is required and is laborious.

Intravenous Urogram (IVU) Indications

Findings Clubbing of calyces

CHAPTER 26A    Urology: Kidney

1. Hydronephrosis 2. Congenital anomalies a. Horseshoe kidney

Flower vase appearance

b. Duplex kidney and double ureter c. Ureterocele

Adder (cobra) head appearance

d. Polycystic kidney disease

Spider leg appearance

e. Retrocaval ureter

Reverse ‘J’ sign with hydronephrosis

3. Renal cell carcinoma

Irregular filling defect; spider leg appearance

4. To see the function of the kidneys in bilateral diseases

Bilateral stones, obstructive uropathy

5. After surgery for urinary diseases

To see the function of kidneys and outcome of the surgery

6. Renal injury

To see the function of other kidney (a very specific investigation)

RETROGRADE PYELOGRAPHY (RGP)

xx xx xx

Fig. 26.5: Left RGP—normal study.

RENAL ANGIOGRAM

Contraindications 1. Iodine sensitivity—may go for anaphylaxis. Hence, all precautions must be taken and essential drugs should be available while doing IVU 2. Multiple myeloma and hypergammaglobulinaemias (Acute renal failure may be precipitated due to dehydration) 3. Toxic thyroid

B

987

Indications

Failure of showing any secretions in an IVU as late as 72 hours film Urinary tuberculosis Urothelial tumours from the renal pelvis

™™ Procedure: Under G/A, cystoscope is passed. Ureteric

orifice is visualised; Ureteric catheter is passed. Dye, sodium diatrizoate is injected; Patient is put in 15° head down position to allow the dye to reach upper urinary system; X-ray is taken. ™™ Advantages ¾¾ Prior to dye injection selective urine sample can be taken from each ureter. ¾¾ Brush biopsy from suspected urothelial tumours of upper urinary tract can be taken. ¾¾ Better-delineation of anatomy (due to more concen­tration of dye).

B xx xx xx

Indications

Renal artery stenosis Renal artery atheroma Renal artery aneurysm

xx xx

Occasionally renal cell carcinoma Arterial anomalies

Retrograde Seldinger technique: ™™ Through femoral artery, using Seldinger’s needle selective

angiogram is done to visualise tumour vascularity, narrowing, ano­malies. Dye used is Hypaque. Dose: 6–7 mL. ™™ Therapeutic embolisation, transluminal balloon angioplasty for renal artery stenosis can also be done by this approach. ™™ Translumbar approach for angiogram (through aorto­gram) is also used. Hypaque used is 30 mL.

B xx xx xx

Complications

Paraplegia Embolism Dissecting aneurysm

xx xx

Bleeding Renal tubular necrosis

Renal pharmacoangiogram: ™™ Noradrenaline is injected along with the dye. Normal vessels will constrict in res­ponse to noradrenaline. But since tumour is auto­n omous, vessels in renal cell carcinoma do not respond to noradrenaline and so tumour blush is seen.

MICTURATING CYSTOURETHROGRAPHY (MCU) ™™ Indications: (a) Vesicoureteric reflux; (b) Posterior urethral

valve.

Men find it easier to flatter than praise.

SRB's Manual of Surgery

988

™™ Procedure: Catheter is passed into the bladder. Dilute iodine

dye is infused. X-ray is taken during micturition. Free reflux is looked for. Applying pressure over the suprapubic region, X-ray is taken. Pressure reflux is studied.

Vesicoureteric reflux is graded depending on the severity of the reflux—as:   I—Ureters seen II—Ureters and pelvis are seen III—Ureters, pelvis, calyces are seen IV—With grossly distended calyces V—Tortuous elongated serpentine ureters It can be unilateral or bilateral. Often it is associated with posterior urethral valve. It is often complicated by infection, pyonephrosis and renal failure Investigations: MCU, IVU, U/S, blood urea and serum creatinine Treatment: Tailoring of ureter with reimplantation

B Fig. 26.7B

Figs. 26.7A and B: Ascending urethrogram showing stricture urethra.

ISOTOPE RENOGRAPHY ™™ A measure of individual kidney function is obtained by this

method using a gamma camera. ™™ Radiolabelled Technetium 99m DMSA (Dimer­capto- succinic acid) or DTPA (Diethylenetriamine-penta acetic acid) is given intravenously. DTPA—renal function. DMSA—renal parenchymal changes.

Fig. 26.6: Micturating cystourethrogram showing concomi­tant existence of posterior urethral valve (causing dilatation of proximal urethra) and vesicoureteric reflux (left side).

ASCENDING URETHROGRAM ™™ It is the investigation of choice for stricture urethra. ™™ Red rubber catheter is passed into the external meatus. ™™ Water-soluble iodine dye is injected through the catheter. ™™ Oblique X-ray films are taken to visualise the urethra. ™™ Site, size, extent of stricture and extra­vasation can be found

out in urethrogram.

A

A Fig. 26.7A

Fig. 26.8A

989

Fig. 26.10: Isotope renogram showing kidney function. Fig. 26.8B

Figs. 26.8A and B: Isotope renogram showing reduced secretion on right side.

It shows: ™™ Early vascular phase. ™™ Then secretory phase. ™™ Later excretory phase. ¾¾ This allows the assessment of renal plasma flow to each kidney and the efficiency and effectiveness of pelvicalyceal excretion also. ¾¾ Secretion 5 RBC’s/HPF).

Early (initial) haematuria: Urethral origin, distal to external sphincter Terminal haematuria: Bladder neck or prostate origin Diffuse (total) haematuria: Source is in the bladder or upper urinary tract False haematuria: Discolouration of urine from pigments such as food colouring and myoglobin. Silent haematuria is due to tumours of kidney or bladder unless proved otherwise.

B

™™ Cause should be identified and treated. ™™ Blood transfusion. ™™ Antibiotics. ™™ Nephroureterectomy for RCC; removal of stone from kidney,

ureter, urinary bladder.

™™ Types ¾¾

Management

Causes

Renal injury Urinary stones Wilm’s tumour Tuberculosis Renal cell carcinoma Cystitis

xx xx xx xx xx xx

Bladder tumour Urinary bilharziasis BPH, carcinoma prostate Renal infarct Glomerulonephritis Blood dyscrasias

™™ Treatment of bladder tumour by cystoscopic resection;

intravesical chemotherapy using BCG; radiotherapy; systemic chemotherapy. ™™ Treatment of medical causes like glomerulonephritis. ™™ Correction of BPH. ™™ Correction of bleeding diathesis.

HORSESHOE KIDNEY ™™ It is a developmental anomaly where there is failure of

complete ascent of kidneys with the fusion of lower or upper poles. It is due to fusion of subdivisions of mesonephric duct, when the embryo is as early as 30–40 days old. ™™ This condition is common in males. ™™ Fusion of lower pole is common (rarely upper poles). ™™ Most common site is in front of 4th lumbar vertebrae. The part in front of the vertebra is called as isthmus. It has blood supply which freely communicates one kidney to other. Isthmus usually lies in front of aorta.

A

B

Fig. 26.21: Causes of haematuria.

Figs. 26.22A and B: (A) Horseshoe kidney and (B) Plain X-ray KUB showing horseshoe kidney with renal stones.

The skilful doctors know what is wrong by observing alone, the middling doctor by listening and the inferior doctor by feeling the pulse.—Chang Chung-Ching

CHAPTER 26A    Urology: Kidney

Fig. 26.20: X-ray showing radio-opaque Malecot’s catheter.

™™ Ultrasound to look for the stone, tumour in the urinary tract. ™™ Cystourethroscopy to look for bladder or urethral pathology. ™™ IVU look for function of the kidneys. ™™ Urinary cytology for diagnosing urothelial malignancy. ™™ Bleeding time; clotting time; prothrombin time; platelet count. ™™ CT abdomen. ™™ Renal function tests—blood urea, serum creatinine.

994

Features

™™ It is bilateral and presents in third decade. One side presents

little earlier than other side.

SRB's Manual of Surgery

™™ Presents as a fixed, nonmobile, firm mass in the midline at the

level of 4th lumbar vertebra which is resonant on percussion. ™™ It is more prone for infection, stone formation, hydronephrosis, tuberculosis. ™™ Investigations: ¾¾ IVU—medialisation of lower calyces and curving of ureter like a ‘flower vase’. ¾¾ Ultrasound abdomen.

A

Fig. 26.23: IVU showing flower vase appearance of horseshoe kidney. ™™ Urine analysis, blood urea and serum creatinine are

supportive investigations. ™™ CT abdomen, CT angiogram. ™™ Treatment ¾¾ Whatever the complication occurs in horseshoe kidney, it is treated accordingly. ¾¾ Per se separation of isthmus is not indicated, unless to approach aorta for aortic diseases.

CYSTIC DISEASES OF THE KIDNEY Types ™™ Genetic

Adult polycystic kidney disease (Autosomal dominant). ¾¾ 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. ¾¾

POLYCYSTIC KIDNEY DISEASE (PCKD) ™™ Adult PCKD is inherited as autosomal dominant disease. It

is common in females.

B

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). ¾¾ ¾¾

B xx xx xx

Clinical features

Bilateral palpable renal mass Loin pain Haematuria

xx xx xx

Infection Hypertension 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.

995

™™ Differential diagnosis: Renal cell carcinoma, Hydrone-

phrosis, Solitary renal cyst

™™ Investigations

Ultrasound confirms the presence of cysts. IVU—Spider leg pattern with an elongated com­pressed renal pelvis, narrowed and stretched calyces. ¾¾ Blood urea and serum creatinine. ¾¾ Urine shows low specific gravity. ™™ Treatment ¾¾ Wait and watch policy. ¾¾ If one of the cysts overdistends causing pain, haemorrhage, infection, then surgical intervention is required. ¾¾ Rovsing operation: The kidney is exposed. The cyst is opened. The fluid is evacuated. The cut edge is marsu­ pialised. ¾¾ Presently US-guided aspiration is done as a simpler approach. ¾¾ Laparoscopic/retroperitoneoscopic aspiration/de-roofing of the renal cyst. ¾¾ Once renal failure sets in, then initial haemodialysis followed by bilateral nephrectomy, is done and later renal transplantation should be planned for. ¾¾

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.

Fig. 26.26: Left side renal 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 ¾¾ IVU—diagnostic. US to look for complications. ¾¾ Cystoscopy shows double ureteric orifices on the same side. ¾¾ 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 ¾¾ 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. ¾¾ Occasionally if the cyst is in one of the poles, partial nephrectomy is done. ¾¾ Laparoscopic approach.

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

CHAPTER 26A    Urology: Kidney

¾¾

996

RETROCAVAL URETER ™™ It is due to developmental defect of IVC, as a result of which

SRB's Manual of Surgery

right ureter passes behind the IVC, causing right-sided hydronephrosis with upper third hydroureter.

A

B

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.

A

™™ Treatment ¾¾ ¾¾

Ureteric meatotomy is done if there is narrowing of the orifice. The co-existing complications are treated.

B

Figs. 26.30A and B: Retrocaval ureter—IVU 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 IVC. ™™ IVU shows hydronephrosis with ‘reverse J sign’. ™™ Treatment:Anderson Hynes’ operation.

URETEROCELE Fig. 26.29: IVU showing bilateral duplex kidney. ¾¾ ¾¾

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; Hydro-

nephrosis.

B

997

Stephen classification

Stenotic, sphincteric, sphinctero­stenotic

CHAPTER 26A    Urology: Kidney

Fig. 26.31: IVU reveals left-sided ureterocele with duplex kidney. Note the characteristic Cobra (Adder) head pattern of left ureter­ocele.

Fig. 26.33: Renal trauma causing extensive laceration. Patient underwent nephrectomy (Courtesy: Dr Ashfaque Mohammed, DNB; KMC, Mangaluru).

™™ Investigations ¾¾ ¾¾

IVU—shows Adder head appearance or cobra head appearance. Cystoscopy—shows translucent cyst which is thin walled surrounding the ureteric orifice.

Types 1. Small subcapsular. 2. Large subcapsular. 3. Cortical laceration. 4. Laceration with perinephric haematoma. 5. Medullary laceration with bleeding into the renal pelvis. 6. Corticomedullary complete rupture. 7. Hilar injury (most dangerous).

B

Grading of renal injury

1. Subcapsular nonexpanding haematoma without parenchymal laceration 2. Cortical laceration 1 cm depth; no urine extravasation 4. Parenchymal laceration extending through cortex and medulla with collecting system; with extravasation of urine; expanding subcapsular haematoma 5. Renal pedicle avulsion; shattered kidney Fig. 26.32: Ultrasound picture of ureterocele—Adder head appearance. ™™ Treatment ¾¾ ¾¾ ¾¾

Cystoscopic ureteric meatotomy with the removal of cyst wall. In addition to that co-existing complications should be treated. Often ureteric reimplantation is needed.

INJURIES TO KIDNEY ™™ Commonly, it is due to a blunt injury. ™™ Often it is associated with other abdominal injuries—of liver,

spleen, bowel, mesentery, etc. ™™ Per se renal injury is extraperitoneal.

Features ™™ Features of shock. ™™ Haematuria—may be mild to profuse depending on the type

of injury.

™™ Sudden delayed profuse haemorrhage causing haematuria

can occur between 3rd day to 3rd week after trauma.

™™ Clot colic. ™™ Bruising, swelling and tenderness in the loin. ™™ Paralytic ileus with abdominal distension occurs due to

retroperitoneal haematoma implicating splanchnic nerves.

™™ Investigations ¾¾

CT scan is the investigation of choice. It gives the grading of the renal injury; identifies the associated injuries; gives idea about the function of the kidney reasonably.

Good health is a serious business: like life itself, it has to be worked at and it takes on added meaning with effort —Normour Cousins

998

75% of patients respond to conservative mana­gement. While treating conservatively, regular moni­toring of blood urea and serum creatinine is a must. ¾¾ If the patient goes in for renal failure, haemo­dialysis should be done for 6–8 weeks. Mean­while, other kidney starts functioning again and patient recovers without any further problem. II. Indications for surgical intervention: ¾¾ When there are signs of progressive blood loss with the condition of the patient deteriorating. ¾¾ Formation of progressive perinephric haema­toma (pulsatile haematoma). ¾¾ When there are associated other injuries. ¾¾ Hilar injury with PUJ disruption. ¾¾

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A

B

C

D

E

F

Surgery (Only in 10–20% of patients) Options: G

Figs. 26.34A to G: (A) Small subcapsular; (B) Large subcapsular; (C) Cortical laceration; (D) Laceration with perinephric haematoma; (E) Medullary laceration with bleeding into the renal pelvis; (F) Corticomedullary complete rupture, (G) Hilar injury (most dangerous).

Ultrasound abdomen is also important initial tool to identify the site and type of injuries, haemoperitoneum and associated injuries. It is very useful tool to check the response for conservative therapy by repeating it at regular intervals. ¾¾ IVU is also useful to see the function of the injured kidney as well as opposite kidney; often it is possible that opposite renal artery goes for reflex spasm, temporarily ceasing the function of the opposite kidney. This may lead into uraemia with raised blood urea and serum creatinine and often needs haemodialysis for few times. Presently, IVU is not an essential investigation; but often done for documentation. ¾¾ Renal function tests at regular intervals; serum electrolyte estimation, haematocrit assessment, blood grouping— are other needed investigations. ¾¾ Chest x-ray, CT scan chest, plain x-ray abdomen often may require to be done. ™™ Treatment I. Initially always conservative: ¾¾ Catheterise and watch the urine colour and output. ¾¾ Blood transfusion. ¾¾ Strict observation with regular monitoring of the pulse, BP, temperature, U/S follow-up daily. ¾¾ Sedation, analgesic and antibiotics.

™™ Gentle suturing of the laceration. Often kidney is friable, this

is not possible. ™™ Then nephrostomy (Cabot’s) is done. ™™ When the injury is in the poles, partial nephre­ctomy is done. ™™ In hilar injury and severe laceration, nephrectomy is the only choice.

¾¾

Types

Treatment

I Only bruise/contusion

Conservative

II Breach in calyceal system rupture of one of the small branches of renal artery

Conservative/nephrectomy

III Rupture of pelvi-calyceal system/renal substance

Nephrectomy

B xx xx xx xx xx xx xx

Complications

Clot retention in the bladder and may go for renal failure Pararenal pseudohydronephrosis Infection Perinephric abscess Aneurysm of the renal artery Renal failure Hypertension occurs 3 months later

RENAL TUBERCULOSIS ™™ Commonly it is secondary. Primary may be in the lung. ™™ Tuberculous bacilluria occurs with an early lesion in the renal

cortex, and the disease spreads along the ureter causing tuberculous ureteritis and stricture ureter. ™™ Tuberculous kidney results in any of the following pathological types.

A

B

C

D

Figs. 26.35A to D: Specimen of kidney showing dilatation and caseous material as content. It is tuberculous pyonephrosis. Ureter is visualised in the specimen. Often there may be ureteric stricture due to tuberculosis.

Pathological Types Through blood, bacteria reach the glomeruli causing caseating granuloma with Langhan’s type of giant cells and epithelioid cells. These granulomas coalesce to form a papillary ulcer and other consecutive different forms. ™™ Tuberculous papillary ulcer. ™™ Cavernous form. ™™ Hydronephrosis. ™™ Pyonephrosis [due to (secondary) superadded infection by E. coli, Klebsiella]. ™™ Tuberculous perinephric abscess. ™™ Calcified tuberculous area (mimics calculi, hence called as pseudocalculi). ™™ Caseous kidney—often called as putty kidney or cement kidney (it goes for autonephrectomy). ™™ Miliary tuberculosis. ™™ Tuberculous bacilluria occurs from an early stage of the disease which causes tuberculous ureteritis and stricture ureter. Most common site is ureterovesical junction; second common site is pelviureteric junction. ¾¾ Tuberculous cystitis eventually results in golf hole ureter and thimble bladder (cystoscopic findings). This is due to fibrosis causing rigid withdrawn dilated ureteric orifice looking like golf hole. Entire urinary bladder gets fibrosed, stiff and unable to dilate and accommodate urine causing thimble systolic bladder. ¾¾ Tuberculous prostatitis, seminal vesiculitis (P/R— palpable seminal vesicle), tuberculous epididymitis and

Fig. 26.36: Mode of spread in urinary tuberculosis.

Clinical Features ™™ Common in males. ™™ Common on right side. ™™ Frequency—both day and night; Polyuria. ™™ Sterile pyuria: Urine is pale and opalescent with presence

of pus cells without organisms in an acid urine—abacterial aciduria (Other causes: Intersti­tial cystitis, chlamydia). ™™ Painful micturition with often haematuria. Haematuria may be overt or microscopic (50%). ™™ Renal pain and suprapubic pain. Suprapubic pain is more common due to cystitis. ™™ Tuberculous kidney is rarely palpable unless there is hydronephrosis or perinephric abscess. ™™ Enlarged prostate and seminal vesicle, thickened beaded vas, thickened epididymis, impotence, infertility are other features. ™™ Presentation like acute pyelonephritis. ™™ Features of urinary stones; recurrent urinary tract infection; renal failure if both kidneys are diseased; hypertension. ™™ Haemospermia; pelvic pain. ™™ Dyspareunia; menstrual dysfunction; vaginal discharge; infertility in females. ™™ Fever and weight loss. ™™ Often cough with expectoration and haemoptysis may be present.

The value of experience is not in seeing much but in seeing wisely—William Osler

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CHAPTER 26A    Urology: Kidney

funiculitis are other associations. Thickened epididymis with ulcer on the posterior aspect of the scrotum may often be found. Tuberculous funiculitis with beaded, thickened vas deferens.

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Investigations ™™ ↓Hb%, ↑ESR. Mantoux skin test is usually positive. ™™ Chest X-ray, U/S abdomen. ™™ Three consecutive early morning samples of urine (EMSU) are

collected and sent for microscopy (Ziehl-Neelsen staining), culture (L-J media) or guinea pig inoculation. ™™ Plain X-ray KUB—shows calcification. ™™ CT scan of abdomen and pelvis to see hydronephrosis, shrunken kidney, stricture, necrosis. ™™ IVU—hydrocalyx, narrowing of calyx, stricture ureter which are often multiple with dilatations in between. ™™ Often RGP is very useful, as better definition of ureter, pelvis, calyces and selective sampling of urine are possible.

™™ Voiding cystourethrography (MCU) to see ureteric stricture

and reflux.

Treatment ™™ Antitubercular therapy is started. INH, rifampicin, ethambutol

and pyrazinamide. Duration of treatment is one year.

™™ After 6–12 weeks of drug therapy, surgical treat­ment is

planned. Kidney is exposed. Pyocalyx is drained. Cut edge of the capsule is sutured—Hanley’s renal cavernostomy. ™™ Hydronephrosis—Anderson Hynes operation or nephrostomy or stenting (“J” stent) of ureter is done. ™™ Renal tuberculous abscess not resolving for 2 weeks should be drained. ™™ Ureteral stricture—stenting/reimplantation of the ureter into the bladder/psoas hitch/Boari’s flap/ileal conduit (Koch’s ileal conduit). ™™ Thimble bladder—hydraulic dilatation/ileocystoplasty/caecocystoplasty/sigmoid colocystoplasty is done. ™™ In unilateral lesion, with gross impairment of renal function—nephroureterectomy is done.

B xx xx xx xx xx

Indications for nephroureterectomy

Nonfunctioning kidney Disease extensively involving the kidney Disease causing hypertension and severe obstruction Tuberculous pyonephrosis Coexisting renal cell carcinoma

HYDRONEPHROSIS (HN) Fig. 26.37: Renal tuberculosis types.

It is an aseptic dilatation of pelvicalyceal system due to partial or intermittent obstruction to the outflow of urine.

Aetiology It can be unilateral or bilateral.

Unilateral

Fig. 26.38: Stricture of left ureter lower part—IVU picture. Common cause is tuberculosis of ureter. ™™ Cystoscopy reveals multiple tubercles, bladder spasm,

oedema of ureteric orifice eventually forming “golf hole ureter”, scarring, ulceration, bleeding, stone formation. ™™ Polymerase chain reaction (PCR) for tuberculosis. Radioisometric culture.

A. Extramural: 1. Aberrant renal vessels (vein or artery). It is common on left side. 2. Compression by growth (carcinoma cervix, carcinoma rectum). 3. Retroperitoneal fibrosis. 4. Retrocaval ureter. B. Intramural: 1. Congenital PUJ obstruction. 2. Ureterocele. 3. Neoplasm of ureter. 4. Narrow ureteric orifice. 5. Stricture ureter following removal of stone, pelvic surgeries or tuberculosis of ureter. C. Intraluminal: 1. Stone in the renal pelvis or ureter. 2. Sloughed papilla in papillary necrosis.

Treatment of aberrant renal vessels

A. Congenital: ¾¾ Congenital stricture of external urethral meatus, pin-hole meatus. ¾¾ Congenital posterior urethral valve. B. Acquired: ¾¾ BPH. ¾¾ Carcinoma prostate. ¾¾ Postoperative bladder neck scarring. ¾¾ Inflammatory/traumatic urethral stricture. ¾¾ Phimosis. ¾¾ Carcinoma cervix. ¾¾ Bladder carcinoma. ™™ Congenital PUJ is the most common cause of HN. ™™ Often it is bilateral and presentation on one side is earlier than the other side. ™™ Aberrant renal artery or vein in the lower pole of kidney can compress the PUJ causing HN. Renal angiogram confirms the diagnosis.

If it is a vein, it can be ligated safely. But if it is an artery, it exclusively supplies the lower pole of the kidney and so cannot be ligated. So kidney is mobi­lised; upper and lower poles are approximated together so that artery is made to slip away from the site of compression—Hamilton Stewart operation. ™™ In pregnancy dilatation of ureters and both pelvis occur due to atony of ureteric musculature by progesterone. It starts as early as in the first few weeks of pregnancy and lasts until few weeks after delivery. Involution occurs 2–12 weeks after delivery.

B

Classifications

Classification I xx Unilateral HN xx Bilateral HN without renal failure xx Bilateral HN with renal failure Classification II Intermittent HN: Obstruction occurs, swel­ling and pain appear in the loin. After sometime, patient passes large amount of urine following which swelling and pain disappear—Dietl’s crisis xx Persistent HN: It is due to persistent partial obstruction Classification III xx HN only xx HN with hydroureter Classification IV xx Extrarenal pelvic HN (80%) xx Intrarenal pelvic HN (20%). Destruction of kidney is earlier and severe here, as compared to extrarenal pelvic HN xx

Fig. 26.39: Aberrant renal vessels are one of the known causes of hydronephrosis.

Pathology ™™ Initially pressure burden is taken up by the pelvis; later

calyces and renal parenchyma. Gradually, parenchyma thins out due to destruction and it dilates. Eventually leading to compromised secretory function. Parenchymal thickness of less than 2 mm is unlikely to function. In bilateral cases such patients will go for renal failure.

A

B

Figs. 26.40A and B: Aberrant renal vessels causing obstruction and hydronephrosis. As this often supplies lower pole of kidney exclusively, it needs to be retained.

Fig. 26.41: Hydronephrosis due to congenital PUJ obstruction. It is the common cause of hydronephrosis.

Always make a total effort even when the odds are against you.

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CHAPTER 26A    Urology: Kidney

Bilateral

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A

B

Figs. 26.42A and B: Specimens showing hydronephrosis. A

Fig. 26.43: Stages of HN.

Features A. In unilateral cases: ¾¾ Congenital PUJ obstruction and calculus are the most common causes. ¾¾ M : F : : 2 : 1. –– Right side kidney is affected more commonly. –– Dull aching loin pain with dragging sensation or heaviness. –– Mass in the loin which is smooth, mobile, ballotable, moves with respiration with dullness in renal angle and a band of colonic resonance in front. –– Attacks of acute renal colic. –– Often patient may be having Dietl’s crisis—after an acute attack of renal colic, swelling in the loin is seen which disappears after sometime following passage of large volume of urine. –– Dysuria, haematuria, if infected fever and tenderness in renal angle. –– Occasionally hypertension.

B

Figs. 26.44A and B: Types of renal pelvis: (A) Intrarenal; (B) Extrarenal.

A

B. In bilateral cases: ¾¾ From lower urinary tract obstruction. xx xx

Loin pain, Features of bladder outlet obstruction—frequency, hesitancy, poor stream Kidneys are often not palpable if renal failure develops early ¾¾

xx xx

From bilateral upper urinary tract obstruction.

Loin pain, mass in the loin, attacks of renal colic In bilateral cases, when it is severe, features of renal failure like oliguria, oedema, hiccough may be present

B

C

Figs. 26.45A to C: CT scan showing hydronephrosis of left kidney with extrarenal pelvis. Patient underwent laparoscopic pyeloplasty (Courtesy: Dr Ashok Padit, MCh, urologist, Mangala hospital, Mangaluru).

1003

B

Fig. 26.48: Hydronephrosis as seen in CT scan.

Figs. 26.46A and B: (A) IVU showing bilateral hydronephrosis and hydroureter; (B) (IVP showing right hydronephrosis. Note the dilated right renal pelvis and delay in the excretion of the dye from the affected kidney.)

Complications ™™ Pyonephrosis. ™™ Perinephric abscess. ™™ Renal failure in bilateral cases.

Investigations ™™ Blood urea and serum creatinine. ™™ Urine for microscopy. ™™ Ultrasound abdomen: Investigation of choice.

B

A

Figs. 26.49A and B: (A) Retroperitoneal tumour causing ureteral obstruction with hydronephrosis; (B) CT scan showing bilateral hydronephrosis. One side large (left side); another side early hydronephrosis.

Type of pelvis, thickness of parenchyma, site of obstruction and cause of obstruction, e.g. stones, can be made out.

Fig. 26.47: Ultrasound picture of hydronephrosis showing dilated pelvis. ™™ IVU: To find out the function of diseased as well as opposite

kidney. Normal calyx is cup shaped. It gets flattened and later club shaped which even­tually becomes broadened in hydronephrosis. ™™ CT scan is diagnostic. ™™ Isotope renography is also useful to study the function of the kidney before and after the surgical treatment and also to see the efficacy of surgery as far as function is considered—DTPA scan.

Fig. 26.50: Isotope scan showing hydronephrosis on the left side. ™™ Whitaker test: A fine needle is passed into the renal pelvis

through loin. Pelvis is perfused with saline at a rate of 10 mL/minute. Normally, initially the pres­sure increases and later it will remain constant. Persistent increase in pressure suggests HN.

Treatment ™™ Always conservative surgeries which are aimed at con­­serving

the kidneys are done. Nephrectomy is not done unless indicated.

Variability is the law of life, and so no two faces are the same, so no two bodies are alike, and no two individuals react alike and behave alike under the abnormal conditions which we know as disease.—William Osler

CHAPTER 26A    Urology: Kidney

A

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1. The cause is treated: Stone, congenital anomaly, aberrant renal vessels, stricture urethra (dilatation, urethrotomy, urethroplasty); phimosis (circumcision); BPH (TURP); posterior urethral valve (cystoscopic fulguration of valve). Note: Please refer respective topics and highlight the individual treatment.

2. Anderson-Hyne’s operation (Dismembered pyelo­plasty): In congenital PUJ obstruction, the spasmodic segment and redundant pelvis are excised. A new pelvis is created and the cut end of pelvis is anastomosed to the ureter in the dependent position.

Features ™™ Usually unilateral.

B

Triad

Anaemia; Fever; Loin swelling ™™ Tender mass in the loin which is smooth, soft, not mobile,

not moving with respiration.

™™ Patient may also have cystitis, pyuria, burning micturition. ™™ Features of toxicity such as fever with chills and rigors. ™™ Investigations

Plain X-ray, KUB, may show renal calculus. IVU shows HN poor secretion. ¾¾ Cystoscopy reveals cystitis with efflux of purulent pus through the ureteric orifice. ¾¾ Ultrasound shows dilatation. ¾¾ DTPA scan later. ™™ Treatment ¾¾ After starting antibiotics, pus is immediately drained from the kidney through a loin incision and nephro­ stomy tube (Cabot’s nephrostomy) (Malecot’s catheter) is placed. ¾¾ If kidney is totally destroyed, subcapsular nephrec­tomy is done. This also prevents other kidney from getting infected through perirenal lymphatic connections. ¾¾ In bilateral pyonephrosis, bilateral nephrostomy is the only choice. ‘J’ stenting is done often to keep the ureters patent. ¾¾ ¾¾

Fig. 26.51: Anderson-Hyne’s operation.

3. Davis T-tube ureterostomy: Placement of T-tube in the ureter by making longitudinal incision. 4. Non-dismembered pyeloplasties: Here PUJ is not transected. Reconstruction is done without PUJ transection by different methods, e.g. Foley’s Y-V plasty. 5. In bilateral HN, without renal failure, kidney which is functioning better should be operated first. Three months later, otherside kidney is dealt with. 6. In bilateral HN with renal failure, bilateral nephrostomy and haemodialysis support is required initially. After 3–6 weeks IVU is done and the functions of both kidneys are looked for. If they function, then treated accordingly by Anderson-Hyne’s operation.  If kidneys are still not functioning (renal function 2 ng/mL/year [rate of change in PSA in one year]); PSA Doubling Time (50 years with PSA >3 ng/mL should undergo prostatic biopsy.

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™™ Haematuria.

SRB's Manual of Surgery

™™ Renal failure. ™™ Prostatism is a combination of symptoms like frequency both

at day and night, poor stream, delay in starting and difficulty in micturition. ™™ Tenderness in suprapubic region, with palpable enlarged bladder due to chronic retention. Hydronephrotic kidney may be palpable. ™™ Per rectal examination shows enlarged prostate. It should be done when bladder is empty. ™™ Features of urinary infection like fever, chills, burning micturition. ™™ International prostate symptom score is available now.

T

Lower urinary tract symptoms (LUTS)

Symptoms of voiding

Symptoms of storage

• • • • • •

• • • • •

Hesitancy Poor flow not improving by straining Dribbling even after micturition Intermittent stream—stops and starts Poor bladder emptying Episodes of near retention

Voiding pressure 80 is high. Cystoscopy. Transrectal ultrasound (TRUS) is useful to find out nodules/possibility of carcinoma prostate. It is not done routinely. Acid phosphatase. Prostate specific antigen. IVU—to see kidney function. Serum electrolytes. ––

Frequency Nocturia Urgency Urge incontinence Nocturnal incontinence

™™ Differential diagnosis

Stricture urethra. Bladder tumour, carcinoma prostate. ¾¾ Neurological causes of retention of urine like diabetes, tabes, disseminated sclerosis, Parkinson’s disease. ¾¾ Idiopathic detrusor activity. ¾¾ Bladder neck stenosis; bladder neck hypertrophy. ™™ Investigations ¾¾ ¾¾

¾¾ ¾¾

¾¾ ¾¾ ¾¾ ¾¾

Note: Normal peak urine flow rate is 20 mL/sec. In obstruction, it is less than 10 mL/sec. ™™ Management

Patient with acute retention of urine requires urethral catheterisation. ¾¾ If urethral catheterisation fails, then suprapubic cystostomy (SPC) is done. ¾¾ If patient presents with uraemia, then urethral catheterisation is a must. That allows the kidney to function adequately and further obstructive damage is prevented. ¾¾ Serum electrolytes should be corrected properly in these patients. ™™ Indications for Surgery ¾¾ Prostatism (frequency, dysuria, urgency). ¾¾ Acute retention of urine. ¾¾ Chronic retention of urine with residual urine more than 200 mL. ¾¾ Complications like hydroureter, hydronephrosis, stone formation, recurrent infection, bladder changes; Haematuria. ¾¾

Surgeries

Fig. 26.109: IVU showing bladder with enlarged median lobe of prostate. ¾¾ ¾¾ ¾¾ ¾¾

Urine for microscopy and C/S. Blood urea and serum creatinine. Ultrasound abdomen—look for presence of residual urine. Urodynamics. –– Urine flow rate >15 mL/sec is normal. 10–15 mL is equivocal; 35). Hypertension—not controlled Surgery requiring more than one hour Surgery with anticipation of major fluid/blood loss or needs postoperative critical care Preterm babies and infants less than 3 month’s age Patient living in far and not easily reachable or able transport easily Unstable psychiatric illness If proper caregiver is not available Uncontrolled diabetes, alcohol abuse, Chronic obstructive pulmonary disease (COPD), severe asthma, epilepsy Pregnancy

B

Levels of day Care Surgery

Three levels are used but minor procedures in outpatient clinic, accident cases are not included. • Minor ambulatory surgery. • Major ambulatory surgery. • Inpatient surgery—patient stays overnight and get discharged within a day.

Day care surgery unit (DSU) is present in many centres. It may be hospital integrated or hospital based or free standing or officer based. It should have a separate dedicated unit with reception, surgery team, theatre, recovery unit, anaesthetist. Patient selection is done prior to surgery including all evaluations. Many surgeries are done as day care surgery—hernia, haemorrhoidal procedures, laparoscopic surgeries, excisions, biopsies, laparoscopic cholecystectomy, appendicectomy, ovarian cystectomy, varicose vein surgery, all endoscopies, circumcision, orchidectomy, hydrocele surgery, vasectomy, renal stone procedures like ESWL, skin grafting, liposuction, fracture manipulation, arthroscopy, surgical decompression of

Genius is one percent inspiration 99% perspiration.

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carpal tunnel syndrome, most of the eye surgeries, tympanoplasties, myringoplasty, adenoidectomy, laryngoscopy, orthodontic surgeries, laparoscopic sterilization, etc. Any surgery which has got low-risk with less bleeding and early ambulation can be done as day care surgery with fulfilling the selection criteria. Advantages are—less infection, home food, less chance of DVT, early ambulation, reduced cost, reduced waiting list, early return to work, psychological benefit. Guidelines for safe discharge—stable vitals; proper orientation and recovery of the patient; tolerant for oral food adequately; ability to pass urine; able to move with or without support; no features of vomiting, nausea, severe pain, swelling or bleeding. Responsible relative to take care of the patient at home should be present. Problems: Postoperative nausea and vomiting (50%); postoperative pain and postoperative drowsiness/dizziness (50%) are the common complications in day care anaesthesia.

Precautions ™™ Patient should be assessed properly before sending to day

care surgery. ™™ The nurse should give proper instruction to the patient as

patient stays in the hospital for a short period. ™™ Patient should be warned about possible problems like

bleeding, vomiting, pain, discomfort, and sedation. ™™ Before discharging, patient should be seen by the doctor for

the fitness. ™™ All records should be carefully documented. ™™ Patient should be advised to rush to hospital if any problems

arise or to communicate immediately. ™™ Now hernia; small gynaecology procedures; ENT, cataract surgeries are done as day care procedures. Nurses hold an important role in day care surgery.

SURGICAL AUDIT ‘Clinical audit’ is a process used by clinicians who intend to improve the patient care. The process involves comparing various aspects of patient care that includes structure, process and outcome against explicit criteria.

Aspects of Patient Care ™™ Structure—includes what is there in that place—the people

in place, their training and knowledge, the equipments and facilities provided, the organization, management and their payment, etc. ™™ Process—includes what procedure is followed in that place in managing referred patients, what antibiotics used, what diagnostic tests done, use of ICU facilities, use of postoperative rehabilitation care, what procedure used for discharge of patients, etc. ™™ Outcome—includes the overall results that include the morbidity, mortality, readmission, improvement /deterioration of the patient’s condition.

Explicit Criteria Proposal for changes can be made in the care of the patient if it falls short of the criteria chosen which can be undertaken at one or more levels: ™™ Individual level—more training can be given to the doctors. ™™ Infrastructure—upgrading of the newer diagnostic tools. ™™ Team level—nurses getting more trained in handling the procedures along with the doctors. ™™ Institution—change in the treatment strategy, or antibiotic policy. ™™ Regional level—providing a good referral centre with all facilities and trained personnel. ™™ National level—introduction of screening programmes and health campaigns. Surgical audit is a systematic, critical analysis of the quality of surgical care that is reviewed by peers against explicit criteria or recognised standards, and then used to further inform and improve surgical practice with the ultimate goal of improving the quality of care for patients. In surgical practice, there will be definitely variations in the results of the surgery done by a trainee or an experienced surgeon, variations in outcome of the operation done in peripheral setup and in referral institutions, usage of modern equipments and technique used. Step 1 Determine scope It should be clearly defined, otherwise results in ineffective/ inappropriate data collection. It should also be relevant, easily measurable.

Fig. 32.56: Surgical audit cycle.

Step 2 Select standards Standards for the selected topic/practice area is decided based on relevant information obtained from: ™™ Evidence-based research and guidelines. ™™ Local guidelines for local relevance. ™™ New guidelines developed based on references from a library. The standard which was already existing or developed must be clearly described, measurable, specific, and realistic. Step 3 Collect data It is important aspect of the audit which has to be informative for the audit to be successful. The best quality data collected depends on by whom it is being collected; when—retrospective/prospective collected; how—on form/PDA/computer; at/ fter the time of surgery; follow-up data when collected; patient identification in a prospective/retrospective study. Collected data must be relevant to the objectives of the surgical audit. Sometimes, the standards may need to be expanded or reduced/or the data collection methods may need to be modified. Step 4 Present and interpret result with peer review Audit aims in continuous improvement by experience and by making changes which is ultimately rewarding. The outcome of the audit should be presented and discussed in a clinical meeting. It should undergo peer review. It involves viewing and analyzing one’s outcome by one’s own peers who are none other than other experienced trained surgeons. It should be conducted in an atmosphere of confidentiality, trust and teamwork, should not be an opportunity to blame or brag but exchange of frank, non-confrontational discussions between the colleagues. Mortality/morbidity meetings, grand rounds are one form of peer review. Step 5 Introduce changes and monitor progress Based on the conclusion of the audit and meetings certain changes to be made in respect to the patient care are decided and all the personnel involved in the process are informed or educated. The outcome due to changes made are monitored by follow-ups either by reauditing the whole process/ or only the part that has been changed.

SURGEON AND LAW ™™ It is important to a surgeon to know legal aspects in relation

to his profession. Consumer protection act and criminal

negligence are the two things surgeons are regularly worried about and face often problems. ™™ It is better to have a fair idea about consumer protection act in relation to patient treatment. ™™ Surgeon should keep all documents regarding the patient with him or in the hospital. ™™ Case sheet should be written in detail. Daily follow-up should be written with date and time of visit with progress about the patient. ™™ It is better to take detailed consent after proper explanation about the disease and treatment protocol to patient and his close attender/relative. It is better to get signature about discussion given from them with date and time. In many centres, it is practiced to record the explanation part to keep it as document. ™™ Surgical method, its problems, risks due to anaesthesia, high-risk if any, risk of bleeding, complications, duration of hospital stay should be discussed. ™™ One should make sure that anaesthetist will do preanaesthetic check up prior to surgery; he should also write his preoperative/operative/postoperative anaesthetic notes. ™™ Daily information sheet should be used wherein patient or party should be informed about the condition of the patient. Timing of this and signature of surgeon and party should be taken. ™™ After surgery detailed surgical procedure technique should be written in case sheet and should be informed to patient. Specimens should be shown to patient party and should be sent for histology. ™™ Approximate cost of the procedure and entire bill in the hospital should be informed. One should also inform that it may change depends on complications, number of days in ICU, critical care, need for higher antibiotics, etc. ™™ Negligence about retaining mops/instruments are legally not acceptable; it is better to take care of enough precautions about that. ™™ Surgeon has got vicarious liability about the mistakes done by ward boys, nurses, theatre nurses, etc. So it is better to train them for proper care in OT, postoperative wards and ICU. ™™ It is ideal to show all reports to patient party and discuss/ brief with them about the condition especially when patient is in ICU. ™™ If patient or party become arrogant or aggressive it is better to make a note of it in case sheet and inform police people about the same. ™™ It is better to make a professional indemnity insurance policy always to cover these problems in case if needed. ™™ It is again ideal to have an advocate to discuss these matters whenever needed. ™™ It is care which surgeon gives not cure always.

So long as enthusiasm lasts, so long is life still with us.

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Common areas in scope of an audit include—duration of hospital stay/unplanned admissions/readmissions/operative specific complications/30 days mortality /morbidity/investigations done/management strategy/patient satisfaction.

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33 Miscellaneous

A. Fascinating Signs in Surgery “If it is a question of doubt in diagnosis, you may often observe that one man solves the doubt when the others could not, and the way in which one man happened to solve it is this: he applied to the diagnosis of the case some method of examination which others had not applied.” —Charles Barrett Lockwood, 1856–1914 SIGN: Sign is an indication of existence of an objective evidence of a disease, i.e. such evidence as is perceptible to the examining physician, as opposed to the subjective sensation (symptoms) of the patient. PATHOGNOMONIC SIGN (patho = disease, gnoma = signature, pathognomonic = signature of the disease): Specially distinctive or characteristic sign of a disease or pathological condition on which a diagnosis can be made. ACCESSORY SIGN (Assident sign): Any nonpathognomonic sign of disease, which adds on to the surety of the diagnosis when present. ANTECEDENT SIGN: Any precursory indication of an attack of disease. These signs are to be identified at the earliest. 1. Aaron’s sign: A sensation of pain and/or distress in the epigastric or precordial region on pressure over McBurney’s point in appendicitis. 2. Abadie’s sign: Charles Abadie—Spasm of levator palpebrae superioris muscle—A sign of Graves’ disease. Jean Abadie—Insensibility of Achilles tendon to pressure, seen in tabes dorsalis. 3. Air cushion sign (Syn: Klemm’s sign): In the radiograph of chronic appendicitis, there is often an indication of tympanitis in the right lower quadrant. 4. Alder’s sign of shifting tenderness: This sign is useful to diagnose acute appendicitis in pregnancy. Locate the most tender spot and mark it on the skin. Now request the patient to turn on the left side and wait for a full minute. If the tenderness is of uterine origin it will shift with the uterus while the position remain constant in case of appendicular origin. 5. Angell’s sign: Helpful in diagnosing “Torsion testis” due to developmental anomaly—The presence of mesentery between the testis and the epididymis is invariably bilateral. The sign is usually obscured on the affected side and can be made out by examining the patient in the standing position wherein the opposite testis will be found to lie horizontally instead of in the normal vertical position. 6. Anghelescus’ sign: Seen in Pott’s disease of spine (TB spine) wherein the victim is unable to bend the spine while lying on the back so as to rest on the head and heel alone. 7. Argyll Robertson pupil sign: Typically described for neurosyphilis wherein light reflex is lost while accommodation reflex is retained. Such an eye responds poorly to mydriatics. This is due to destruction of fibres between pretectal nucleus and Edinger-Westphal nucleus. Other conditions where this sign may be seen—encephalitis, vascular and traumatic lesions, cerebral tumours, diabetes mellitus and chronic alcoholism. 8. Auenbrugger’s sign: Bulging of the epigastrium due to massive pericardial effusion. This sign highlights the importance of examining thorax in patients with abdominal symptoms. 9. Babinski’s sign (Not syn with Babinski’s reflex): a. Loss or weakening of the Achilles tendon reflex in sciatica. This sign helps distinguish true sciatica from hysterical sciatica. b. In hemiplegia the contraction of platysma muscle in the healthy side is more pronounced than on the affected side. It can be elicited by asking the patient to open the mouth, whistling, blowing, etc.



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c. When a hemiplegic patient is lying with arms crossed upon the chest and makes an effort to sit up, the thigh on the paralyzed side is flexed upon the pelvis and the heel lifted from the floor while limb on the healthier side does not move. d. When the paralyzed forearm is placed in supination it turns over to pronation. It is seen in organic paralysis and is also called ‘Pronation sign’. 10. Baid sign: Described for pseudocyst of pancreas and is well appreciated in thin individuals. When Ryle’s tube is passed into the stomach, it may be palpated over the swelling because the stomach is displaced anteriorly by the pseudocyst pancreas. 11. Ballances’ sign: Seen in about 25% of ruptured spleen. There is a dull note in both the flanks due to haemoperitoneum. The dullness on the right side can be made to shift, but that on the left side remains constant/fixed because the blood in the vicinity of the ruptured spleen gets coagulated soon. 12. Ballet’s sign: This sign is helpful in Graves’ disease and hysteria wherein there will be persistence of involuntary pupillary and reflex eye movements with loss of all voluntary eye movements (external ophthalmoplegia). 13. Bamberger’s sign: A sign described in pericardial effusion—Presence of signs of consolidation at the angle of scapula which disappears when the patient leans forwards. 14. Banana sign: An ultrasonographic sign described for Arnold-Chiari deformity—a cause for congenital hydrocephalus. Sonography of the fetal skull reveals flattened and curved (banana like) shape of the cerebellar hemisphere. 15. Bastede’s sign: A sign described in a case of appendicitis—Mentioned to be condemned—when colon is inflated with air through a rectal tube, pain and tenderness can be elicited in the right iliac fossa in a suspected case of appendicitis. Such manoeuvre carries risk of perforation and hence should not be entertained. 16. Battle’s sign: It is relevant in middle cranial fossa fracture—bruising/ecchymosis over the mastoid process in the line of posterior auricular artery is a tell-tale sign of underlying middle cranial fossa fracture (temporal bone fracture). 17. Bergman’s sign: A urologic radiographic sign: a. The ureter is dilated immediately below an obstructing neoplasm rather than collapsed as seen in cases of obstructing stones. b. The ureteral catheter passed in such cases tends to coil in this dilated portion of the ureter. 18. Berry’s sign: Indicated by the absence of carotid artery pulsation in a patient presenting with goitrous swelling, is an ominous sign of thyroid malignancy (due to carotid sheath infiltration by the malignant tissue). 19. Bezold’s sign: Described in a case of mastoiditis—An inflammatory swelling seen below the apex of the mastoid process. 20. Biederman’s sign: A dark colour instead of normal pink colour of the anterior pillar of the throat is seen in some patients with syphilis. 21. Bird’s sign: Described for hydatid disease of lung wherein a definite zone of dullness with absence of the respiratory sounds may be appreciated. 22. Biernacki’s sign: Analgesia of ulnar nerve in general paresis and tabes dorsalis. 23. Blatin’s sign (Syn: Hydatid thrill): A sign elicited in cases of hydatid cystic disease. It is due to displacement of daughter cysts in the fluid of the mother cyst. 24. Boas sign: An area of hyperaesthesia, posteriorly extending 2.5 cm lateral to the spinous process of vertebrae to the posterior axillary line and vertically from the level of the 11th dorsal to the 1st lumbar spine—A definitive sign of the presence of cholecystitis. 25. Blumberg sign (Syn: Rebound tenderness; Release sign): It is a sign of peritonitis due to presence of an inflamed organ underneath it. 26. Bonnet’s sign: Pain on thigh adduction in sciatica. 27. Boston’s sign (Syn: von Graefe’s sign): It constitutes the lid lag elicited in cases of thyrotoxicosis. 28. Bowler’s hat sign (Syn: Double ring sign): A radiological sign which describes the appearance of a gastric polyp seen on end-on position in a double contrast barium meal study. There will be a central lucency with two rims of barium around. May also be seen in cases of sessile intestinal polyp or a diverticulum. 29. Boyce’s sign: A gurgling sound heard on pressure by the hand on the side of neck, in cases of oesophageal diverticulum. 30. Bozzolo’s sign: A visible pulsation of the arteries in the nostrils. A sign believed to indicate the presence of aneurysm of the thoracic aorta. 31. Branham sign (Syn: Nicoladoni sign): This sign is elicited when arteriovenous fistula is suspected. A pressure on the artery proximal to the fistula will cause: a. Reduction in size of the swelling. b. Disappearance of bruit. c. Fall in pulse rate. d. Pulse pressure returns to normal. 32. Brodie’s sign: A black spot on the glans penis—a sign of gangrene due to urinary extravasation into the corpus spongiosum. 33. Bald fundus sign: A radiological sign described for atrophic gastritis. The fundus of the stomach looks like a small dome with absence of mucosal pattern indicated by a very thin smooth appearing gastric wall. 34. Bent inner tube sign: A radiological sign described for sigmoid volvulus—an important diagnostic sign. A plain X-ray abdomen taken in the supine position reveals a massively distended ahaustral sigmoid colon arising from the pelvic loop. 35. Bird of prey sign: A radiological (Barium enema) sign which helps in confirming the diagnosis in doubtful cases of sigmoid volvulus. Barium enema reveals a smooth tapered narrowing at the point of torsion of the colon. Mucosal folds show a screw pattern around the point of twist.

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36. Border sign: Describes the appearance of ventral hernia in barium study follow through done in suspected cases, in the postoperative period. Lateral and inferior border of the hernia are sharply outlined while the medial and upper border blends with the abdominal shadows. 37. Cardarelli’s sign: Transverse pulsations in the laryngotracheal tube in suspected cases of aneurysms and dilatation of the aortic arch. 38. Chvostek’s sign (Syn: Chvostek Weiss sign; Schultze’s sign): A clinical sign typically described for hypocalcaemic tetany. The sign is elicited by tapping over the muscles and/or superficial nerves to induce the muscle spasm. It may be: a.  Facial sign: A light tap over the facial nerve branches in front of the ear lobe causes muscular twitching over the whole of that side of the face. b.  Peroneal sign: Tapping the peroneal nerve near the fibular neck will cause dorsiflexion and abduction of the foot. 39. Cobra head sign (Adder head appearance): A radiological sign seen in cases of ureterocele—congenital dilation of lower end of ureter. Urography done in suspected cases produces a characteristic appearance which resembles a cobra head and hence the name. 40. Crescent sign: A radiological sign described in relation to two different conditions affecting the lungs and the kidneys: a. In plain chest X-ray taken in a patient suspected of hydatid disease or aspergilloma (fungus ball) of the lung reveals a crescent of air in the shadow of partially ruptured hydatid cyst or in the cavity containing fungus ball. b. On IVP, the nephrogram reveals a crescent sign in cases of congenital hydronephrosis. 41. Cullen’s sign (Syn: Cullen Hofstatter sign): A clinical sign which was typically and initially described for ruptured ectopic pregnancy wherein there is discolouration (ecchymosis) of the umbilicus and the surrounding skin (aptly referred to as umbilical black eye). It is due to haemoperitoneum and may be seen in conditions like ruptured ectopic pregnancy (a bluish tinge), acute haemorrhagic pancreatitis (a yellowish tinge). 42. Carman’s sign (Syn: Carman-Kirklin meniscus sign): A radiographic sign helpful in the analysis of gastric ulcers in barium meal study. It is a reliable indicator of malignancy. Nonprojecting ulcers lying intraluminal in all projections surrounded by an elevated rim of tumour produces a curval interface resembling a meniscus and is called Carman’s meniscus sign. Whether the meniscus is convex or concave towards the lumen is mainly dependent upon the site of ulcer in relation to incisura angularis. Meniscus is concave towards the lumen in cases of ulcer being proximal to the incisura while it is convex when the ulcer is distal to the incisura. 43. Carnett’s sign: A clinical sign which helps differentiate the plane of abdominal swelling. The abdominal wall muscle is made tense by asking the patient to raise both the legs with knee extended. If the lump is intraperitoneal it disappears or becomes less prominent, while it becomes prominent or persists when the lump arises from the abdominal wall. 44. Chilaiditi’s sign (Syn: Chilaiditi’s syndrome; Hepatoptosis): A radiological sign seen in plain X-ray abdomen which is helpful in the diagnosis of this syndrome wherein there is interposition of colon between liver and diaphragm. It needs to be differentiated from the conditions causing gas under the diaphragm. The presence of haustrations in the gas shadow in a plain X-ray abdomen tilts the favour in the diagnosis of Chilaiditi’s syndrome. 45. Coiled spring sign: A radiological sign (Barium enema) classically described for intussusception. The passage of barium beyond the apex of the intussusception into the intussuscipien gives a coiled spring appearance. The sign may also be seen in cases of: a. Post-traumatic haematoma of duodenum. b. Acute appendicitis. c. Mucocele of appendix. d. Endometriosis of appendix. e. Intestinal carcinomas. 46. Coles’ sign: A radiological sign. Barium meal follow through study reveals deformity of the duodenal contour in the presence of duodenal ulcer. 47. Colon cut off sign: A radiographic sign of appendicular perforation or colonic spasm. Absence of gas and feces in the right lower quadrant, reflex dilatation of transverse colon and sharp cut-off of gas at the hepatic flexure. This sign is also seen in acute pancreatitis 48. Coopernail’s sign: Ecchymosis on the perineum and scrotum/labia. A sign of fracture pelvis. 49. Cope’s sign (Syn: Psoas sign): Clinical signs which are relevant in cases of acute appendicitis. This has two tests: a.  Cope’s psoas test: In acute appendicitis there is psoas muscle spasm secondary to the inflamed organ and hence the patient keeps the thigh in a flexed position. This pain can be aggravated by passively hyper-extending the hip joint which stretches the psoas muscle. b.  Cope’s obturator test: Principles are same as above except the muscle involved is obturator internus which is passively stretched by internally rotating the right leg which is flexed at the hip and knee. 50. Cupola sign: A radiological sign helpful in the diagnosis of pneumoperitoneum. A plain X-ray abdomen (erect position) reveals gas under the diaphragm when there is relatively large amount of air in the peritoneal cavity—Cupola sign. 51. Crow foot sign (Syn: Mercedes Benz sign; Seagull’s sign): A radiological sign described in relation to cholelithiasis (Gallstones). Nearly 80–90% of the gallstones are radiolucent with only 10–20% being radio-opaque. However, rarely a non-opaque gallstone can be diagnosed in plain radiography by the presence of gas containing crevices within the stone. These radiolucent crevices give the appearance of the crow foot; Hence the name crow foot sign, Mercedes Benz sign or the Seagull’s sign. 52. Courvoisier’s sign (Syn; Courvoiser’s law): In a patient with obstructive jaundice, if the gallbladder is palpable it is not due to gallstones.

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53. Cowen’s sign: A clinical sign elicited in cases of Graves’ disease. In response to a light shone on one eye there is jerky constriction of the contralateral pupil. 54. Crowe’s sign: Refers to axillary freckling seen in neurofibromatosis. 55. Cruveilhier’s sign of the saphena varix: A clinical sign elicited in cases of varicose veins (Saphena varix). A thrill is felt over the saphena varix when the patient is asked to perform a Valsalva manoeuvre/cough in an erect position and is due to a jet of blood entering and filling the pouch. 56. Coleman’s sign: Helps in the clinical evaluation of fracture mandible. There is obvious swelling and bruising over the bony injury and a haematoma in the floor of the mouth if the body of the mandible is fractured. 57. (Meniscus) Claw sign: A radiological sign (Barium study) diagnostic of ileo-colic intussusception. The barium in the intussuscipien is seen as a claw around the negative shadow of the intussusceptum. 58. Coffee bud sign: It is a radiological sign seen in volvulus of sigmoid colon. 59. Dalrymple’s sign: It is one of the manifestations of Graves’ ophthalmopathy. It consists of retraction of the upper eyelid so that the palpebral opening is abnormally wide and upper sclera is visible. 60. Delbet’s sign: A prognostic indicator in cases of aneurysm of the main artery of the limb. If the nutrition of the part distal to the aneurysm is maintained then the collateral circulation is said to be sufficient even though the distal pulse is not felt. 61. de Musset’s sign: Rhythmical jerking movement of the head with each heart beat and is seen in cases of aortic insufficiency and aortic aneurysm. 62. Dance sign (Syn: Signe de Dance): A feeling of emptiness in the right iliac fossa—A sign of intussusception. 63. Demarouay’s sign: Fixation or lowering of larynx during phonation and deglutition. A sign of syphilis of trachea. 64. Dew’s sign: A clinical sign described in relation to the diaphragmatic hydatid abscess beneath the right cupola of the diaphragm. The area of resonance moves caudally with the patient in knee-elbow position. 65. Dixon Mann’s sign (Syn: Mann’s sign): In case of Graves’ orbitopathy the two eyes appear not to be on the same level. 66. Dorendorf’s sign: The sign identifies the fullness in the supraclavicular groove on one side in aneurysm of aortic arch. 67. Double bubble sign: A radiological sign described in cases of duodenal obstruction. A plain X-ray abdomen reveals two foci of gas, one in the stomach and the other in the duodenum—a sign of duodenal atresia. A similar feature is observed in the foetus in ultrasonography done in the antenatal period. 68. Drummond’s sign: A whiff sound heard over the open mouth during respiration in cases of aortic aneurysm. 69. DTP sign (Syn: Distal tingling on percussion; Tinel’s sign; Formication sign): A prognostic indicator which is helpful in the evaluation of nerve recovery following nerve injury. If percussion over the site of nerve injury causes tingling sensation in the distal end of the limb it suggests that the nerve injury was a partial one or it heralds the recovery from the nerve injury—a good prognostic sign. 70. Dubois sign: Shortness of the little finger in congenital syphilis. 71. Duchenne’s sign: This clinical sign identifies the sinking of the epigastrium during inspiration (a paradox). It is classically seen in cases of paralysis of the diaphragm and in certain cases of hydropericardium (Pericardial effusion). 72. Dupuytren’s sign: It is described in two conditions: a. A crackling sensation on pressure over a sarcomatous bone. b. In congenital hip dislocation, it refers to free up and down movement of the head of the femur. 73. Dott’s sign: It is helpful in differentiating pain due to acute appendicitis and basal pneumonia with pleuritis. Compression of lower thorax from side to side elicits obvious distress when the lesion is above the diaphragm whereas in appendicitis it has no effect. 74. Dock sign: A radiological sign described in relation to coarctation of aorta. A chest X-ray PA view reveals rib notching on the inferior margins of 3rd-9th ribs while sparing the first two ribs and is indicative of collateral circulation developed in coarctation of aorta. 75. De Weese sign: A clinical sign helpful in cases presenting with history suggestive of intermittent claudications with palpable peripheral pulses. The patient is asked to exercise by walking or running sufficiently to bring on the pain. If prompt re-examination reveals absence of peripheral pulses it suggests that the pain was truly due to intermittent claudication. 76. Echo sign: A percussion sound resembling an echo which is heard over a hydatid cyst. 77. Elliot’s sign: Refers to presence of indurated edge of a syphilitic skin lesion. 78. Enroth’s sign: Identifies the abnormal fullness of the eyelids—a manifestation of Graves’ orbitopathy. 79. Escherich’s sign (Syn: Escherich’s reflex): Described in relation to tetany, where percussion of the inner surface of the lips or tongue produces contraction of lips, tongue and masseter muscles. 80. Ewart’s sign: It is said to be positive in cases of pericardial effusion if there is bronchial breathing and dullness on percussion at the lower angle of the left scapula and is due to pressure and collapse of the lingular lobe due to enlarged pericardial sac. 81. E-sign (Syn: Reverse 3 sign): A radiological sign seen on barium swallow radiograph in patient suspected of coarctation of aorta and is due to indentation produced on the barium filled oesophagus by the aorta. 82. Figure of three sign: A radiological sign seen in the plain chest X-ray PA view in patient with coarctation of aorta. A pair of bulges is seen in the wall of the aortic arch one above and one below the aortic knuckle and is due to pre- and post-stenotic dilatation of the aorta. 83. Frostberg’s sign: A radiological sign seen in barium study in carcinoma of the head of pancreas involving the duodenum. In carcinoma of the head of the pancreas, there is widening of the ‘C’ loop of the duodenum and with the involvement of the ampulla of Vater, the expanded loop assumes a reversed 3 configuration—Frostberg’s sign. Once the carcinoma of the head affects the duodenum the surgical cure is remote.

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84. Fox sign: Discolouration near the inguinal ligament—seen in few cases of haemorrhagic pancreatitis. 85. Fuchsig’s sign (Syn: Crossed leg test): This sign is more relevant when popliteal artery is not clinically palpable along with absent distal pulses. The patient is made to sit with his legs crossed. Normally, oscillatory movements of the foot occur synchronously with the pulse if the popliteal artery is patent while a negative result is more suggestive of popliteal artery block in a patient with absent distal pulses. 86. Federici’s sign: An interesting sign described in relation to pneumoperitoneum (due to intestinal perforation) wherein on auscultation of the abdomen, the cardiac sounds can be heard. 87. Flush tank sign: A clinical sign described in relation to hydronephrosis. The patients classically present with features suggestive of Dietle’s crisis, i.e. passage of a large amount of urine with consequent disappearance of a lumbar swelling—Flush tank sign. 88. Flare sign: The precursor of varicose ulcer is a splay of fine venules that courses from the medial (sometime the lateral) malleolus, and spreads out to be lost beneath the thick skin of the heel and is known as the “Flare sign”. 89. Gifford’s sign: A clinical sign which helps in differentiating unilateral exophthalmos and proptosis due to intraorbital tumours. If the upper eyelids cannot be easily everted on the affected side, the exophthalmos is more likely to be due to thyrotoxicosis, whereas if the lid is easily everted it is more likely due to an intraorbital mass. 90. Grey Turner sign: Skin discolouration (bruising) in the left flank (left costovertebal angle) in cases of acute haemorrhagic pancreatitis. 91. Gilbert’s sign: Opsiuria indicative of hepatic cirrhosis (opsiuria means excretion of urine more rapidly during fasting than after a meal). 92. Glasgow’s sign: A clinical sign—presence of systolic murmur over the brachial artery is found in latent aortic aneurysm. 93. Griffith sign: It is one of the clinical signs of Graves’ orbitopathy wherein there is lower lid lag on upward gaze. 94. Guyon’s sign: Ballottement and palpation of a floating kidney. 95. Gaur sign: A clinical sign seen in femoral hernia. Distension of superficial epigastric and/or circumflex iliac veins on the affected side due to pressure on these vessels by the hernial sac. 96. Guerin’s sign: Haematoma at greater palatine foramen seen in maxillary fracture. 97. Homan’s sign: Pain on sudden dorsiflexion of the foot—a sign of deep vein thrombosis of calf veins. 98. Hamilton Bailey sign: A clinical sign described for intussusception. A sausage shaped mass with concavity towards the umbilicus which is felt to harden as a wave of peristalsis commences. 99. Hall’s sign: This sign identifies a tracheal diastolic shock felt in the aneurysm of aorta. 100. Hatchcock’s sign: Refers to the tenderness towards the angle of the jaw in mumps. 101. Haudek’s sign (Haudek’s niche): A radiological sign identified in barium meal X-ray done in patient suffering from gastric ulcer. It describes a projecting shadow (niche) in radiographs due to settlement of barium in pathological niches of stomach wall. 102. Hitzelberger’s sign: A clinical sign described in case of acoustic neuroma wherein there is anaesthesia of medial, posterior or superior areas of the external auditory canal because of the tumour tissue compressing on the facial nerve. 103. Hook sign: This clinical sign identifies flexion of fingers in a case of acute suppurative tenosynovitis. 104. Horn’s sign: This sign identifies the pain produced by traction on the right spermatic cord in acute appendicitis. 105. Howship Romberg sign: A sign described in the patients suffering from obturator hernia. The patient complains of pain passing down the inner side of the knee due to pressure on the obturator nerve by the hernial sac. 106. Harvey’s sign: Two index fingers are placed by the side on a vein. The fingers are now pressed firmly and the finger near the heart is moved proximally keeping the steady pressure on the vein so as to empty the short length of vein between the two fingers. The distal finger is now released. This will allow venous refilling to be observed which will be poor in ischemic limb and increased in arteriovenous fistula. 107. Hamman’s sign (Syn: Hamman’s mediastinal crunch or murmur): A clinical sign—precordial crunching, clicking or knocking sound synchronous with each heart-beat heard on auscultation in conditions such as: ¾¾ Acute mediastinitis ¾¾ Pneumomediastinitis ¾¾ Pneumothorax. 108. Inflammatory signs (Syn: Cardinal signs in inflammation): ¾¾ Rubor—Redness ¾¾ Calor—Temperature ¾¾ Dolor—Pain ¾¾ Tumour—Oedema/swelling ¾¾ Functio lesa—Loss of function 109. Jendrassik’s sign: Paralysis of extraocular muscles—A manifestation of Graves’ orbitopathy. 110. Jugular sign (Syn: Quekenstedt’s sign; Tobey Ayer test): On spinal canal block (e.g. spinal tumour) when pressure is applied over jugular veins, in the manometer connected to LP needle, either pressure will not raise or if raises falls slowly. 111. Joffroy’s sign: Absence of wrinkling of the forehead when the head is bent down and the patient is asked to look upwards—A sign of Graves’ ophthalmopathy. 112. Kernig’s sign: A sign which is positive in meningitis. With the hip flexed, the knee is extended, normally it can be done upto 175°. In meningitis it is restricted due to spasm of the hamstrings. 113. Kaposi Stemmer sign: Failure to pick up or to pinch a fold of skin at the base of the second toe. It is characteristic of lymph oedema.

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114. Kocher sign (Syn: Mean’s sign): A sign of Graves’ orbitopathy. The examiner places one hand on a level with the patient’s eye and then lifts it higher. The upper eyelid springs up more quickly than the eyeball. 115. Kanavel sign: This sign is useful in the diagnosis of ulnar bursitis. In case of ulnar bursitis the site of maximum tenderness is over that part of the ulnar bursa lying between the transverse palmar creases. 116. Kantor’s sign (String sign of Kantor): A radiological sign described in barium enema follow through in patients suffering from Crohn’s disease. A string like configuration of contrast material through a filling defect is seen in the radiography. 117. Kehr sign: This sign identifies the pain elicited in the left shoulder in patients with suspected splenic rupture. The pain (referred pain) experienced by the patient is due to blood in the peritoneal cavity irritating the diaphragm. 118. Klemm’s sign: It is the radiological sign found in chronic appendicitis where there is often an indication of tympanitis in the right lower quadrant. 119. Knie’s sign: Unequal dilatation of the pupils—a sign of Graves’ orbitopathy. 120. Krisowski’s sign: Cicatricial lines radiating from the mouth in congenital syphilis. 121. Kenawy’s sign: A clinical sign usually associated with bilharzial fibrosis of liver (Egyptian splenomegaly) but may be present in any type of portal hypertension. Auscultation over the xiphoid process reveals a venous hum (splenic vein engorgement) which becomes prominent on inspiration. 122. Klein’s sign (of shifting tenderness): A clinical sign useful in acute nonspecific mesenteric lymphadenitis. When patient is shifted to left lateral position, point of maximum tenderness also gets shifted to the left side in contrast to acute appendicitis. It may also be positive in Meckel’s diverticulitis. 123. Lemon sign: A radiological sign of Arnold-Chiari deformity. There is scalloping of the frontal bones giving the skull a lemon shaped configuration in sonography of the fetal skull during second trimester of pregnancy. 124. Lennhoff’s sign: A furrow appearing on deep inspiration below the lower rib and above an echinococcal cyst of liver. 125. Sign of Leser Trelat: Sudden appearance and rapid increase in size and number of seborrhoeic keratoses, a sign of internal malignancy of the GIT. 126. Lloyd’s sign: A clinical sign elicited in a patient with renal calculus. Pain elicited in the loin on deep percussion over the kidney even when there is no pain on applying deep pressure. 127. London’s sign: It is useful in ruptured intestine suspected in an accident case. The presence of pattern of bruising of the skin (i.e. an imprint of the clothing is noted on the skin) indicates that a crushing force has been applied sufficient to rupture the bowel against the vertebral column. This sign is a strong indication to carry a laparotomy. 128. Mallet Guy’s sign: A clinical sign identified in chronic relapsing pancreatitis. It is elicited by placing the patient in the right lateral position with the patient’s hip and knee flexed and deeply palpating the abdomen in the epigastric and the left subcostal region. This will evoke tenderness in chronic relapsing pancreatitis. 129. McBurney’s sign: Finger tip pressure is made over the McBurney point elicits severe tenderness in patients with appendicitis. 130. Murphy’s sign (Moynihan’s method): This clinical sign is classically described in patients suffering from cholecystitis. It is elicited by asking the patient to breath deeply while exerting moderate pressure with the left hand such that thumb lies over the fundus of the gallbladder. The patient catches his breath as the inflamed gallbladder which is pushed down by the diaphragm gets imposed against the thumb. 131. McEwen’s sign: A clinical sign elicited in children with hydrocephalus. On percussion of skull behind the junction of frontal, parietal and temporal bones and auscultation over opposite mastoid bone there is a more resonant note than normal, seen in internal hydrocephalus and cerebral abscess in children (syn: cracked pot sign, cranial cracked pot sound). 132. Meniscus sign: Refer No. 57. 133. Moebius sign: Inability to keep the eyeballs converged due to insufficiency of medial rectus muscle—A clinical sign of Graves’ ophthalmopathy. 134. Milian’s ear sign: A clinical sign which is useful to differentiate facial erysipelas from cellulitis. Erysipelas being a cuticular lymphangitis spreads from the face to the pinna while the cellulitis which is spreading inflammation of the subcutaneous tissue stops short of pinna because of close adherence of the skin to cartilage. 135. Mahler’s sign: A steady increase of pulse rate without corresponding increase of temperature seen in thrombosis. 136. Mann’s sign (Syn: Dixon Mann’s sign): In Graves’ disease the two eyes appear not to be on the same level. 137. Marie’s sign: Tremors of body extremities in Graves’ disease and other types of hyperthyroidism. 138. Mean’s sign (Syn: Kocher’s sign): Refer No. 114. 139. Medusa Lock sign: A radiological sign described in patients with intestinal obstruction due to roundworm infestation. A plain X-ray erect film of the abdomen reveals trapped intestinal gas within the worm mass giving a characteristic appearance of coiled locks of hair—Medusa Lock sign. 140. Meitzer’s sign: Loss of normal second heart sound on auscultation of the heart after swallowing. Seen in occlusion or contraction of lower part of oesophagus. 141. Mercedes Benz sign (Syn: Crow foot sign): Refer No. 51. 142. Mexican hat sign: A radiological sign described in barium enema (filling defect) done in patients with pedunculated polyp of the inferior wall of the colon. 143. Maulage sign: Waxy cast appearance of bowel segments—A radiographic sign of coeliac disease. 144. Mose’s sign: A clinical sign suggestive of deep vein thrombosis. It is elicited by squeezing the relaxed calf muscles from side to side which is painful in case of deep vein thrombosis.

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145. Niche sign (Syn: Haudek’s sign): Refer No. 101. 146. Nicoladoni’s sign (Syn: Branham’s sign): Refer No 31. 147. Oliver’s sign (Syn: Porter’s sign; Tracheal tug): Tracheal tug is seen in: ¾¾ Aneurysm of the aorta. ¾¾ Neoplasm which fixes bronchus to aorta. 148. Omega sign: A radiological sign observed in patient suspected of sigmoid volvulus on the X-ray. A plain X-ray abdomen taken in the supine position shows a massively distended sigmoid colon with haustrations arising from the pelvis resembling Greek letter ‘omega‘ (w). 149. Payr’s sign: Pressure over the sole of the foot elicits severe pain in cases of thrombophlebitis. 150. Peroneal sign: Tapping the peroneal nerve near the fibular neck causes dorsiflexion and abduction of foot in hypocalcaemic tetany. 151. Perez’s sign: A friction sound heard over the sternum when the patient raises and drops his arms. A sign of mediastinal tumour or aneurysm of arch of aorta. 152. Parrot’s sign (Syn: Parrot’s nodes, hot cross bun skull, Natiform skull): Bony nodes on the outer table of the skull of infants with congenital syphilis, so that it has a hot cross bun or buttock shape. 153. Pfuhl sign: A clinical sign which helps in differentiating between subphrenic abscess and pyopneumothorax. Inspiration increases the force of flow in paracentesis in subphrenic abscess, but lessens in case of pyopneumothorax. This distinction is however lost in cases where diaphragm is paralyzed. 154. Pitre’s sign: Hypoaesthesia of scrotum and testes in tabes dorsalis. 155. Plummer’s sign (Syn: Quadriceps sign): Inability to step up onto the chair or to walk up steps seen in Graves’ disease and other forms of hyperthyroidism. 156. Pool Schlesinger’s sign: In tetany if patients leg is held at the knee joint and flexed strongly at the hip joint, within a short time there will be an extensor spasm at the knee joint with extreme supination of foot. 157. Porter’s sign: Tracheal tugging in aneurysm of aortic arch and neoplasms which fix the left bronchus to aorta. 158. Potain’s sign: Extension of percussion dullness over the arch of the aorta from the manubrium to the third costal cartilage on the right side—seen in cases of dilatation of the aorta. 159. Prehn’s sign: Elevation and support of the scrotum will relieve the pain in epididymo-orchitis but not in torsion testis. 160. Psoas sign (Syn: Cope’s test): Refer No. 49. 161. Puddle sign: Describes a clinical method to detect small amount of ascites (150–500 mL). Patient lies prone for 5 minutes and then goes for knee-elbow position. In this position dullness is elicited in the umbilicus in case of minimal ascites. 162. Pemberton’s sign: This sign refers to symptoms of faintness with evidence of facial congestion and external jugular vein distension when the arms are raised above the head touching the ears. This manoeuvre reduces the thoracic inlet thereby hampering venous drainage of the face in the presence of retrosternal thyroid. 163. Pointing sign: Ask the patient to point to the site of maximum pain. If this proves to be the site of localized tenderness it is also certainly the site of diseased organ, e.g. appendicitis. 164. Patel’s sign: It is important in paralytic ileus. Apply the stethoscope firmly to the skin just below and right of the umbilicus for full 3 minutes. In paralytic ileus there will be omnious silence, broken by patient’s own heart sounds believed to be transmitted via the over distended coils of the intestine with added succussion splashes if the patient moves and very occasionally by faint tinkles. 165. Pad sign: A radiological sign identified in patients with carcinoma of the head of pancreas wherein the ‘C’ loop of barium filled duodenum may be widened. 166. Panda sign (Raccoon sign, Spectacle haematoma, Black eye): Haemorrhages in soft tissues around the eye and in the eyelids is known as Panda sign as it resembles panda eyes. It is caused by: 1. Direct trauma, such as punch to the eye. 2. Blunt impact to the forehead, the blood gravitating downwards over the supraorbital ridge. 3. Fracture of the floor of the anterior fossa of the skull. 167. Queckenstedt’s sign (Jugular sign; Tobey Ayer test): Refer No. 110. 168. Quenu Muret sign: A prognostic indicator. In aneurysm, when the main artery of the limb is compressed and a puncture is made at the periphery, if the blood flows, then the collateral circulation is probably established. 169. Rat-tail sign: A radiological sign described in relation to oesophageal carcinoma. 170. Ridge sign: A clinical sign of dehydration. In considerable dehydration if the skin is picked up in between the thumb and fingers and then released, instead of it springing back with normal elasticity, a ridge is formed that subsides slowly. 171. Rovsing sign: In acute appendicitis, when left iliac fossa is pressed; pain is felt in right iliac fossa. 172. Release sign (Syn: Rebound tenderness; Blumberg’s sign): Refer No. 25. 173. Reversed 3-sign: Refer No. 81. 174. Reisman sign (Syn: Snellen’s sign): The bruit heard over the closed eye in Graves’ disease. 175. Rocher’s sign: A clinical sign which helps in the differentiation of epididymitis from torsion of the testes. In torsion testes, the epididymis cannot be distinguished from the body the testes, whereas in epididymitis the body of the testes can be felt in the enlarged crescent of epididymis. 176. Rommelaere’s sign: An abnormally small proportion of normal phosphates and of sodium chloride in urine in cancerous cachexia. 177. Rotch sign: A clinical sign identified in case of pericardial effusion. Dullness is felt on percussion on the right 5th intercostal space. 178. Rovighi’s sign: A fremitus felt on percussion and palpation of the superficial hepatic hydatid cyst.

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CHAPTER 33    Miscellaneous

179. Racoon sign (Syn: Panda sign, Spectacle Haematoma, Black Eye): Refer No. 166. 180. Sign of splashing (Syn: Succussion splash): Seen in gastric outlet obstruction. 181. Saegesser’s sign: A clinical sign identified in patients with splenic rupture. An excruciating tenderness is elicited in the Saegesser’s point or splenic point (seen in the lower part of the posterior triangle of the neck between the left sternomastoid and scalenus medius muscle above the clavicle). 182. Sternomastoid sign (Syn: Trail’s sign): The sternal head of the sternomastoid muscle will become more prominent on the side to which trachea is deviated. 183. Saenger’s sign: Refers to the light reflex of the pupil that has ceased, returns after a short stay in the dark. Observed in cerebral syphilis but not in tabes dorsalis. 184. Sansom’s sign: Described in two separate context: ¾¾ Marked increase in the area of dullness in the second and third intercostal space – Due to pericardial effusion. ¾¾ A rhythmical murmur heard with a stethoscope applied to the lips in aneurysms of the thoracic aorta. 185. Schlesinger’s sign (Syn: Pool’s phenomenon): Refer No. 156. 186. Schultze sign (Syn: Schultze Chvostek sign): Refer No. 38. 187. Silex’s sign: Furrows radiating from the mouth in congenital syphilis. 188. Sisto’s sign: Constant crying in infancy—a sign of congenital syphilis. 189. Setting sun sign: A clinical sign described in patients suffering from raised intracranial tension. There is downwards deviation of the eyes so that each iris appears to set beneath the lower eyelid with white sclera exposed between it and the upper lid. This sign is observed in cases: ¾¾ Hydrocephalus ¾¾ Intracranial haemorrhage ¾¾ Brain tumours 190. Snellen’s sign (Syn: Reisman sign): Refer No. 174. 191. Sumner’s sign: This sign is said to be positive when there is increase in the tone of the abdominal muscle on gentle palpation of the right iliac fossa. It may indicate: ¾¾ Appendicitis ¾¾ Right ureteric or renal stones ¾¾ Torsion ovarian cyst. 192. Simon’s sign: Identifies absence of usual correlation between the movements of diaphragm and thorax, seen in early cases of meningitis. 193. Stierlin’s sign: A radiological sign observed in barium enema study of the colon where there is absence of normal shadow due to an indurating or ulcerative process such as tuberculosis of caecum or colon. 194. String sign (Syn: Kantor’s sign): Refer No. 116. 195. Suker’s sign: Deficient complementary fixation in lateral eye rotation—a manifestation of Graves’ orbitopathy. 196. String of beads sign: A series of round shadows resembling a string of beads or pearls, seen on a radiograph of small intestine, indicating of trapped gas surrounded by the fluid of obstructed and distended bowel. 197. Stellwag’s sign: Identifies the widening of palpebral fissures (staring look) due to retraction of upper eyelids, an early sign of Graves disease. 198. Stemmer sign (Kaposi Stemmer sign): Refer No. 113. 199. Slip sign: A clinical sign which helps in differentiating a solid swelling, e.g. lipoma, from a cystic swelling. Here, when the edge of the swelling is palpated, the margin of the solid swelling does not yield but slips away from it unlike a cystic swelling which yields to the pressure of the palpating finger and does not slip away. 200. Solius sign: A radiological finding observed in a chest X-ray lateral view in patient’s with enlarged thymus. An enlarged thymus being a firm swelling does not get flattened against sternum by the pressure of heart and great vessels. 201. Shrinkage sign: A radiological sign of thymus enlargement. In a chest X-ray there is a paradoxical alteration of the shape of the chest with respiration (Decrease in transverse diameter with deep inspiratory film than the expiratory film). 202. Seagull sign: Refer No. 51. 203. Suzmann’s sign: A clinical sign described in patient with coarctation of aorta. The collaterals which develop display visible and palpable pulsations together with thrills and murmurs which are most obvious in the inter-scapular and infra-scapular regions of the back. The Dock sign is the radiological counterpart of this sign. 204. Trail’s sign (Syn: Sternomastoid sign): Refer No. 182. 205. Tap sign (Syn: The percussion sign; Chevrier’s sign): If the valves are incompetent an impulse will be felt by the fingers overlying the long saphenous vein when the varicosities are percussed below. 206. Thornton’s sign: Refers to the severe pain complained by the patient in the region of the flanks in nephrolithiasis. 207. Tinel’s sign (Syn: DTP sign; Formication sign): Refer No. 69. 208. Tresilian sign: This sign identifies reddish appearance (congestion) in opening of Stensen’s duct in cases of mumps. 209. Trimadeau’s sign: A radiological sign identified in barium swallow X-ray done in patient’s with dysphagia. If the dilatation above an oesophageal stricture is conical, the stricture is fibrous; while it is cup shaped (shouldering) it is likely to be malignant. 210. Tanyol’s sign: In ascites umbilicus shifts downwards and in mass arising from pelvis it shifts upwards.

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211. Troisier’s sign: Identifies enlargement of left supraclavicular lymph node (Virchow’s node). Seen in: ¾¾ Ca stomach ¾¾ Ca testes ¾¾ Ca bronchus ¾¾ Malignancy of any other abdominal organ. 212. Trousseau’s sign: This sign is described under two different context: ¾¾ The blood pressure cuff is applied to the arm and inflated to pressure above systolic pressure for 3–5 minutes. This will elicit typical carpopedal spasm (obstetrician’s hand) in cases of hypoparathyroidism and other conditions associated with hypocalcaemia. ¾¾ Migrating superficial thrombophlebitis—a sign of visceral carcinomas especially of pancreas or the stomach. 213. Tracheal fluctuation sign: A unique sign elicited in patients suffering from achalasia cardia. 214. von Graefe’s sign (Syn: Graefe’s sign): Persistent lagging of upper lid behind the corneoscleral limbus when patient is asked to follow the finger moved up and down several times. Seen in Graves’ disease. 215. Vermooten’s sign: A clinical sign helpful in the intrapelvic rupture of urethra. On per rectal examination, the prostate cannot be felt but in its position a doughy swelling (blood and urine) is felt. If prostate is felt, it is displaced upwards. 216. Vas sign: This sign is helpful in differentiating testicular neoplasm and an inflammatory lesion of the testes. Inflammatory lesions causes vas deferens to become considerably thickened which remains normal in cases of neoplasm. 217. Vein sign: A bluish cord along the mid-axillary line formed by the swollen junction of the thoracic and superficial epigastric vein. Seen in: ¾¾ Tuberculosis involving the bronchial glands ¾¾ Superior vena cava obstruction. 218. Wegner’s sign: A postmortem finding—A broadened discoloured appearance of the epiphyseal line in infants who have died from congenital syphilis. 219. Wilder’s sign: An early clinical sign of Graves’ disease consisting of slight twitch of the eyeball when it changes its movement from adduction to abduction or vice versa. 220. Wimberger’s sign: Symmetrical erosions of the proximal tibia seen radiographically in infants with congenital syphilis. 221. Water lily sign (Syn: Lily pad sign): A radiological sign identified in cases of hydatid cyst of the lung. When the hydatid cyst ruptures, the daughter cyst floating within the cavity appear like a water lily hence the name water lily sign. 222. Smith’s sign: Murmur heard in cases of enlarged bronchial lymph nodes on auscultation over the manubrium with the patients head thrown backwards. 223. ‘H’ Bomb sign: A radiological sign seen in cases of atrophic gastritis. The gastric folds within fundus and the body of stomach are very thin and the thin walled fundus becomes distended with air (in erect posture) or with barium contrast.

B. Triads in Surgery 1. Saint’s triad: –– Diverticulosis of colon –– Gallstones –– Hiatus hernia.

14. Pancoast’s triad: Seen in Pancoast tumour. –– Excruciating pain in the arm –– Horner’s syndrome –– Erosion of ribs.

2. Whipples’ triad: Seen in insulinoma. –– Features of hypoglycaemia –– Blood sugar less than 45 mg% –– Symptoms are relieved by glucose.

15. Prune-Belly triad: Seen in Prune-Belly syndrome. –– Cryptorchidism –– Abdominal wall defects –– Genitourinary defects.

3. Charcot’s triad: Seen in ascending cholangitis. –– Intermittent fever –– Intermittent pain –– Intermittent jaundice. 4. Virchow’s triad: –– Change in the vessel wall –– Diminished rate of blood flow –– Increased blood coagulability. 5. Murphy’s triad: Seen in acute appendicitis. –– Pain in right iliac fossa –– Vomiting –– Temperature. 6. Hutchinson’s triad: Seen in late congenital syphilis –– Interstitial keratitis –– 8th nerve deafness –– Hutchinson’s teeth. 7. Trotter’s triad: Seen in nasopharyngeal carcinoma. –– Conductive deafness –– Elevation and immobility of same side soft palate –– Pain in the side of the head. 8. Tillaux’s triad: Seen in mesenteric cyst. –– Soft fluctuant swelling in the umbilical region –– Freely mobile in the direction perpendicular to mesentery –– Zone of resonance all around. 9. Triad of portal hypertension: –– Varices –– Splenomegaly –– Ascites. 10. Cushing’s triad: In intracranial hypertension. –– Increased blood pressure –– Decreased pulse rate –– Decreased respiratory rate. 11. Triad of renal cell carcinoma: –– Anaemia –– Haematuria –– Mass in the loin.

16. Mackler’s triad: Seen in Boerhaave’s syndrome. –– Vomiting –– Chest pain –– Subcutaneous emphysema. 17. Triad of Sandblom: Seen in haemobilia –– Jaundice –– Pain –– Melaena. 18. Galezia triad: –– Dupuytren’s contracture –– Retroperitoneal fibrosis –– Peyronie’s disease of penis. 19. Dieulafoy’s triad: Seen in appendicitis –– Hypersensitiveness of skin –– Reflex muscular contraction –– Mac Burney’s tenderness. 20. Triad of congenital diaphragmatic hernia –– Respiratory distress –– Apparent dextrocardia –– Scaphoid abdomen. 21. Carney’s triad –– Functioning adrenal para ganglioma—nonfamilial –– Gastric leiomyosarcoma—GIST –– Pulmonary chondroma. 22. Triad of Ohashi in IPMN in ERCP –– A bulging ampulla of Vater –– Mucin secretion –– Dilated main pancreatic duct. 23. Haimovici triad of revascularisation of an acutely ischaemic limb –– Muscle infarction –– Myoglobinuria –– Acute renal failure.

12. Borchardt’s triad: Seen in gastric volvulus –– Acute epigastric pain –– Violent vomiting –– Inability to pass nasogastric tube.

24. Triad of 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.

13. Beck’s triad: Seen in cardiac tamponade. –– Muffled heart sounds –– Distended neck veins –– Hypotension.

25. Gilroy Bevan triad of adhesive pain is –– Pain may get aggravated or relieved on change of posture –– Pain in the region of old abdominal scar –– Tenderness is elicited by pressure over the scar.

Reason of life is destiny of unknown; and desire of life

C. Misnomers in Surgery 1. White bile: It is neither white nor bile. It is opalescent. It contains mucous. It signifies severe obstructive jaundice due to which secretion of bile from liver is stopped. Mucous is derived from biliary tree lining. 2. Mycotic aneurysm: It is not due to fungal infection. It is due to bacterial infection. 3. Lateral aberrant thyroid: It is not an aberrant thyroid. It is secondaries in neck lymph node from occult primary in the thyroid, i.e. papillary carcinoma thyroid. 4. Adenolymphoma of parotid gland: It is not lymphoma. It is a benign tumour of the parotid. It never turns into malignancy. 5. Pretibial myxoedema: It is not seen in myxoedema. It is seen in thyrotoxicosis. 6. Sternomastoid tumour: It is not a tumour. It is due to birth trauma which causes organised haematoma in sternomastoid muscle. 7. Dissecting aneurysm: It is aortic dissection, not dissecting aneurysm. 8. Malignant hydatid: It is not malignant. It is due to Echinococcus alveolaris. It behaves like a malignant condition. 9. Malignant exophthalmos: It is due to primary thyrotoxicosis. It is not a malignant condition. 10. Spina ventosa: It is tuberculous dactylitis. It is not related to spine. 11. Mycosis fungoides: It is cutaneous “T” cell lymphoma. It is not due to fungal infection. 12. Hypernephroma: It is renal cell carcinoma. It is not above the kidney. 13. Hepatoma: It is hepatocellular carcinoma. It is not benign tumour. 14. Melanoma: It is melanocarcinoma. It is not benign. 15. Surgical ganglion: It is arising from the synovial sheath. It is not from nerve ganglion. 16. Brain fungus: It is not a fungal infection. It is seen in cranial injury wherein due to injury brain protrudes out of the wound as fungus. 17. Cock’s peculiar tumour: It is not a tumour. It is ulcerated sebaceous cyst. It mimics SCC of skin. 18. Pott’s puffy tumour: It is not a tumour. It is osteomyelitis of the frontal bone with cellulitis of the frontal region of the scalp. It may spread intracranially through emissary veins which endangers the life of the patient. 19. Tumour alba: It is tuberculosis of synovial sheath of knee joint. 20. Umbilical adenoma: It is not a tumour. It is prolapse of mucosa of cutaneous end of vitello-intestinal duct 21. Madelung’s deformity: It is not associated with any of the conditions in the lung. It is a congenital subluxation or dislocation of the lower end of the ulna, from malformation of the bones. 22. Ray fungus: Actinomycoses, a bacteria infection. 23. Malignant pustule: Anthrax infection. 24. Malignant oedema: Gas gangrene. 25. Juvenile melanoma: It is not a melanoma. It is benign SPITZ naevus. 26. Hydrocele of the neck: It is cystic hygroma 27. Kuttner tumour: It is chronic sclerosing sialadenitis of submandibular salivary gland. 28.  Aneurysmal bone cyst: It is not related to artery. It is expanding lesion in the bone containing blood mixed fluid of unknown aetiology. 29.  Hairy leucoplakia: It is hairy leucoplakia with white confluent patches of fluffy/hairy hyperkeratotic thickening almost always situated in the lateral border of the tongue. It is not hair in tongue; not related hair diseases; not structurally hair component.

D. Triangles in Surgery 1. Hesselbach’s triangle: The medial border of the triangle is formed by the rectus sheath, superolateral border by the inferior epigastric artery, inferior border by the inguinal ligament. Importance: Helps in differentiating direct from indirect hernias. 2. Bryant’s triangle: Patient lies in supine position, three lines are drawn, one from the anterior superior iliac spine vertically down to the bed; another from the tip of the greater tronchanter to join the first line at the right angles; third line from anterior superior iliac spine to the tip of the greater tronchanter—this forms a triangle. Importance: Diminution in the length of the second line indicates upward displacement of the greater trochanter. Third (oblique) helps determine the anteroposterior displacement of the greater tronchanter. 3. Triangle of Doom: Bounded by the ductus deferens medially, spermatic vessels laterally in the male and the apex of the triangle is at the level of internal inguinal ring. Importance: External iliac artery and vein, femoral nerve are located in the triangle and so stapling is avoided in this triangle while doing laparoscopic preperitoneal repair for hernia. 4. Calot’s triangle: Formed medially by the common hepatic artery, laterally by the cystic duct and the apex is formed by the junction of the cystic and the hepatic ducts. Base is formed by the cystic artery. Importance: Cystic lymph node of ’Lund’ is present in the fork created by the junction of the cystic and common hepatic artery. 5. Anal triangle and urogenital triangle: A transverse line joining the anterior parts of the ischial tuberosities and passing immediately anterior to the anus divides the perineum into two triangles, anal and urogenital triangle. Anal triangle contains the termination of the anal canal in the median plane and an ischiorectal fossa on each side. Importance: Both the perineal and ischiorectal spaces in the anal triangle are common sites of abscess. 6. Femoral triangle: It is triangular depression on the front of the upper one-third of the thigh below the inguinal ligament, bounded medially by the medial border of adductor longus, laterally by the medial border of sartorius, apex is formed by the meeting point of the medial and lateral boundaries. Importance: Femoral vein in this site is most suitable for intravenous injection in the infants; femoral artery pulsations are an important aid for clinicians (in diagnosing peripheral vascular disease). 7. Lumbar triangle: a. Inferior lumbar triangle of Petit—bounded by the crest of ilium below, external oblique laterally and medially by the latissimus dorsi. Importance: Most primary lumbar hernia occur through this triangle. b. Superior lumbar triangle of Grynfelt—bounded by the 12th rib above, medially by the sacrospinalis and laterally by the posterior border of the inferior oblique. Importance: Grynfelt hernia appears through superior lumbar triangle. 8. Triangle of auscultation: This is a small triangular interval bounded medially by the lateral border of the trapezius, laterally by the medial border of the scapula and inferiorly by the upper border of latissimus dorsi. Floor of the triangle is formed by the 7th rib, 6th and 7th intercostal spaces and rhomboids minor. Importance: This is the only part of the back which is not covered with muscles. Respiratory sounds are better heard with a stethoscope here than that heard elsewhere in the back. On the left side, the cardiac end of the stomach lies deep to this triangle and in days before X-rays were discovered, sounds of swallowed liquids were auscultated over this triangle. 9. Simon‘s triangle: Bounded anteriorly by the recurrent laryngeal nerve, posteriorly by the common carotid artery and base is formed by the inferior thyroid artery. Importance: Aids in identification of the recurrent laryngeal nerve. 10. Lumbosacral triangle of Marcille: It is a triangular interval on each side of the body of the 5th lumbar vertebra, bounded medially by the body of the 5th lumbar vertebra, laterally by the medial border of the psoas muscle, apex by the junction of the psoas major muscle and the body of the 5th lumbar vertebra. Base is formed by the upper surface of the ala of the sacrum and floor by the transverse process of the 5th lumbar vertebra and the iliolumbar ligament. Importance: Ureter crosses the common iliac vessels at the lateral angle of the triangle. 11. Retromolar trigone: Base overlies the ascending ramus of the mandible from the last molar, apex terminates at the maxillary tuberosity, laterally continuous with the buccal mucosa and medially blends with the anterior tonsillar pillar. Importance: Common site for oral malignancy. 12. Sherren’s triangle: Bounded by the umbilicus, symphysis pubis and the anterior superior iliac spine. Importance: Indicates the area of hyperaesthesia in an acute episode of appendicitis. 13. Scalene triangle: Bounded by scalenus anticus anteriorly, scalenus medius posteriorly and first rib inferiorly. Importance: Subclavian artery and trunks of the brachial plexus pass through the scalene triangle where they may be compressed causing thoracic outlet syndrome.

A quiet, vacant mind is distressed, depressed mind.

Further Reading 1. Bailey and Love’s Short Practice of Surgery, 27th edition. 2. Oxford Textbook of Surgery, 2nd edition. Reprint 2000. 3. Maingot’s Abdominal Operations, 12th edition. 4. Fischer’s Mastery of Surgery, 6th edition. 5. Sabiston Textbook of Surgery, 19th edition. 6. The Washington Manual of Surgery, 6th edition. 7. Farquharson’s Textbook of Operative General surgery, 10th edition. 8. Surgery of Anus, Rectum and Colon by John Goligher, 5th edition. 9. Surgery of the Anus, Rectum and Colon by Michael RB Keighley, Norman S Williams, 3rd edition.

Appendix Important Laboratory Values URINE Specific gravity: Normal: 1.010 to 1.025. Low: less than 1.010. High: more than 1.025. Fixed: 1.010 to 1.014. Reaction: Acidic with a pH of 6.0. Colour: Clear and amber coloured. Parameter

Values

Aldosterone Nitrogen Amylase Calcium Catecholamines Copper Creatinine Creatinine clearance Estrogens 17-hydroxy corticosteroids 5-HIAA 17-ketosteroids Magnesium Metanephrines Urine osmolarity Phosphorus Porphyrins   Coprophyrin   Uroporphyrin Potassium Protein Sodium Urobilinogen VMA

2–10 μg/day 0.4–1.0 g/day 30–250 Somogyi units/hour