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Diseases of theGallbladder and BileDucts To our mentors, to whom we are profoundly indebted for their inspired teaching, long-standing support, and advice during our careers (PAC): Felix Harder, Adrien Rohner, Martin Allgöwer Bernie Langer, Steve Strasberg and David Sabiston (JB): Jack Vennes, Steve Silvis, Peter Cotton and Dick Kozarek The Editors and Publisher have made every effort to contact all copyright holders to obtain their permission to reproduce copyright material. How- ever, if any have been inadvertently overlooked, the Publisher will be pleased to make the necessary arrangements at the first opportunity. Diseases of the Gallbladder and Bile Ducts Diagnosis and Treatment EDITED BY Pierre-Alain Clavien, MD, PhD, FACS, FRCS Professor and Chairman Swiss Hepato-Pancreato-Biliary Center Department of Visceral and Transplant Surgery University Hospital Zurich Zurich, Switzerland John Baillie, MB, ChB, FRCP, FACG Professor of Medicine Director of Hepatobiliary and Pancreatic Disorder Service Wake Forest University Health Sciences Center Winston-Salem, North Carolina, USA ASSOCIATE EDITORS Michael A.Morse, MD Duke UniversityMedical Center Markus Selzner, MD University Hospital Zurich SECOND EDITION © 2006 by Blackwell Publishing Ltd Blackwell Publishing, Inc., 350 Main Street, Malden, Massachusetts 02148-5020, USA Blackwell Publishing Ltd, 9600 Garsington Road, Oxford OX4 2DQ, UK Blackwell Publishing Asia Pty Ltd, 550 Swanston Street, Carlton, Victoria 3053, Australia The right of the Author to be identified as the Author of this Work has been asserted in accordance with the Copyright, Designs and Patents Act 1988. All rights reserved. No part of this publication may be reproduced, stored in a retrieval system, or transmitted, in any form or by any means, electronic, mechanical, photocopying, recording or otherwise, except as permitted by the UK Copyright, Designs and Patents Act 1988, without the prior permission of the publisher. First published 2001 Second edition 2006 1 2006 Library of Congress Cataloging-in-Publication Data Diseasesofthegallbladder and bile ducts : diagnosis and treatment / editedbyPierre-Alain Clavien, John Baillie ; associate editors, Michael A. Morse, Markus Selzner.– 2nd ed. p. ; cm. Includes bibliographical referencesand index. ISBN-13: 978-1-4051-2740-0 ISBN-10: 1-4051-2740-6 1. Gallbladder–Diseases. 2. Bile ducts–Diseases. I. Clavien, Pierre- Alain. II. Baillie, John, FRCP (Glasg.) [DNLM: 1. Gallbladder Diseases–diagnosis. 2. Bile Duct Diseases – diagnosis. 3. Bile Duct Diseases–therapy. 4. Gallbladder Diseases –therapy. WI 750 D611 2006] RC845.C55 2006 616.3′65–dc22 2006000956 ISBN-13: 978-1-4051-2740-0 ISBN-10:1-4051-2740-6 A catalogue record for this title is available from the British Library Set in 9 on 12 pt Meridien by SNP Best-set Typesetter Ltd., Hong Kong Printed and bound in India by Replika Press Commissioning Editor: Alison Brown Editorial Assistant: Jennifer Seward Development Editor: Elisabeth Dodds Production Controller: Kate Charman For further information on Blackwell Publishing, visit our website: http://www.blackwellpublishing.com The publisher’s policy is to use permanent paper from mills that operate a sustainable forestry policy, and which has been manufactured from pulp processed using acid-free and elementary chlorine-free practices. Furthermore, the publisher ensures that the text paper and cover board used have met acceptable environmental accreditation standards. Blackwell Publishing makes no representation, express or implied, that the drug dosages in this book are correct. Readers must therefore always check that any product mentioned in this publication is used in accordance with the prescribing information prepared by the manufacturers. The author and the publishers do not accept responsibility or legal liability for any errors in the text or for the misuse or misapplication of material in this book. Contents Contributors, vii Preface, ix Abbreviations, xi Section 1. Anatomy, pathophysiology, and epidemiology of the biliary system 1. Anatomy and physiology of the biliary tree and gallbladder, 3 James Toouli and Mayank Bhandari 2. Pathology of the intrahepatic and extrahepatic bile ducts and gallbladder, 21 Kay Washington 3. Epidemiology of diseases of the bile ducts and gallbladder, 58 Markus H. Heim Section 2. Diagnostic and therapeutic approachesfor the biliary tree and gallbladder 4. Noninvasive imaging of the biliary system, 71 Elmar M. Merkle, Rendon C. Nelson and Henrik Petrowsky 5. Endoscopic diagnosis and treatment of disorders of the biliary tree and gallbladder, 97 Kevin McGrath and John Baillie 6. Percutaneous biliary imaging and intervention, 120 Paul V. Suhocki 7. Radiation therapy for disease of the biliary tree and gallbladder, 147 Brian G. Czito and MitchellS. Anscher 8. Surgery ofthe biliary system, 163 Lucas McCormack,Markus Selzner and Pierre-Alain Clavien 9. Laparoscopic treatment for diseases of the gallbladder and biliary tree, 174 Stefan Wildi, Sarah K. Thompson, John G. Hunter and Markus Weber 10. Laparoscopic biliary injuries, 182 Steven M. Strasberg 11. Medical and innovative therapies for biliary malignancies, 205 Michael A. Morse and Bernhard Pestalozzi Section 3. Specific conditions Section 3.1. The gallbladder 12. Natural history and pathogenesis of gallstones, 219 Beat Müllhaupt v vi Contents 13. Acute and chronic cholecystitis, 229 Stefan Breitenstein, Armin Kraus and Pierre-Alain Clavien 14. Biliary fistula, gallstone ileus, and Mirizzi’s syndrome, 239 Henrik Petrowsky and Pierre-Alain Clavien 15. Benign and malignant gallbladder tumors, 252 John T. Mullen, Christopher H. Crane and Jean-Nicolas Vauthey Section 3.2. The intrahepaticand extrahepaticbile ducts 16. Acute cholangitis, 265 Suyi Chang and Joseph Leung 17. Cystic diseases of the biliary system, 277 Robert J. Porte and Pierre-Alain Clavien 18. Biliary complications of liver transplantation, 289 Mary T. Austin and C. Wright Pinson 19. Primary sclerosingcholangitis, 306 Robert Enns 20. Cholangiocarcinoma, 332 Markus Selzner and Pierre-Alain Clavien 21. Primary biliary cirrhosis, 341 Piotr Milkiewicz and Jenny Heathcote Section 3.3. Intrahepaticcholestasis 22. Intrahepatic cholestasis, 355 Andrew Stolz and Neil Kaplowitz Section 3.4. Pediatric population 23. Biliary disease in infants and children, 377 Riccardo Superina Answers, 411 Index, 415 Color plate section appears after page 84 Contributors Mitchell S. Anscher, MD Robert Enns, MD, FRCP Department of Radiation Oncology Division of Gastroenterology Duke UniversityMedical Center Department of Medicine Durham, North Carolina, USA St. Paul’sHospital University of British Columbia Mary T. Austin, MD, MPH Vancouver, British Columbia, Canada Department of Surgery Jenny Heathcote,MD, FRCP, FRCP Division of Hepatobiliary Surgery and Liver Transplantation Vanderbilt University Medical Center University of Toronto Nashville, Tennessee, USA Toronto Western Hospital Toronto, Ontario, Canada John Baillie, MB, ChB,FRCP, FACG Markus H. Heim, MD Professor of Medicine Division of Gastroenterology and Hepatology Director of Hepatobiliary and Pancreatic Disorder Service University Hospital Basel Wake Forest University Health Sciences Center Basel, Switzerland Winston-Salem, North Carolina, USA John G. Hunter, MD Mayank Bhandari, MBBS, MS Department of Surgery Department of General and Digestive Surgery Oregon Health and Science University Flinders Medical Centre Portland, Oregon, USA Flinders University Bedford Park, Adelaide, SA, Australia Neil Kaplowitz, MD USC Research Centerfor Liver Diseases Stefan Breitenstein, MD Division of Gastrointestinal and Liver Diseases Swiss Hepato-Pancreato-Biliary Center Keck School of Medicine Department of Visceral and Transplantation Surgery University of Southern California University Hospital Zurich Los Angeles, California, USA Zurich, Switzerland Armin Kraus, MD Suyi Chang, MD, PhD Swiss Hepato-Pancreato-Biliary Center Division of Gastroenterology Department of Visceral and Transplant Surgery Davis Medical Center University Hospital Zurich University of California Zurich, Switzerland Davis, California, USA Joseph Leung, MD, FRCP, FACP, FACG Davis School of Medicine Pierre-Alain Clavien,MD, PhD, FACS, FRCS University of California Swiss Hepato-Pancreato-Biliary Center Section of Gastroenterology Department of Visceral and Transplant Surgery VA Northern California HealthCare System University Hospital Zurich Sacramento, California, USA Zurich, Switzerland Lucas McCormack, MD Christopher H. Crane, MD Swiss Hepato-Pancreato-Biliary Center Department of Radiation Oncology Department of Visceral and Transplant Surgery University of Texas University Hospital Zurich M. D. Anderson Cancer Center Zurich, Switzerland Houston, Texas, USA Kevin McGrath, MD Brian G. Czito, MD Division of Gastroenterology Department of Radiation Oncology Department of Medicine Duke UniversityMedical Center Duke UniversityMedical Center Durham, North Carolina, USA Durham, North Carolina, USA vii viii Contributors Elmar M. Merkle, MD Andrew Stolz, MD Department of Radiology USC Research Centerfor Liver Diseases Duke UniversityMedical Center Division of Gastrointestinal and Liver Diseases Durham, North Carolina, USA Keck School of Medicine University of Southern California Piotr Milkiewicz, MD, MRCP Los Angeles, California, USA University of Toronto Toronto Western Hospital Steven M. Strasberg, MD Toronto, Ontario, Canada Section of HPB/GI Surgery and Department of Gastroenterology Washington University in Saint Louis Pomeranian Medical School Saint Louis, Missouri, USA Szczecin, Poland Paul V. Suhocki, MD Michael A. Morse, MD Division of Interventional Radiology Division of Medical Oncology Department of Radiology Duke UniversityMedical Center Duke UniversityMedical Center Durham, North Carolina, USA Durham, North Carolina, USA John T. Mullen, MD Riccardo Superina, MD Department of Surgical Oncology Feinberg Schoolof Medicine University of Texas Northwestern University M. D. Anderson Cancer Center Chief of Pediatric Transplant Surgery Houston, Texas, USA Children’s Memorial Hospital Chicago,Illinois, USA Beat Müllhaupt, MD Swiss Hepato-Pancreato-Biliary Center Sarah K. Thompson, MD Division of Gastroenterology and Hepatology University Hospital Zurich Department of Surgery Zurich, Switzerland Oregon Health and Science University Portland, Oregon, USA Rendon C. Nelson, MD Department of Radiology James Toouli, MBBS, B(Med)Sci, PhD, FRACS Duke UniversityMedical Center Department of General and Digestive Surgery Durham, North Carolina, USA Flinders Medical Centre Flinders University Bernhard Pestalozzi, MD Bedford Park, Adelaide, SA, Australia Swiss Hepato-Pancreato-Biliary Center Department of Oncology Jean-Nicolas Vauthey, MD, FACS University Hospital Zurich Department of Surgical Oncology Zurich, Switzerland University of Texas M. D. Anderson Cancer Center Henrik Petrowsky, MD Houston, Texas, USA Swiss Hepato-Pancreato-Biliary Center Department of Visceral and Transplant Surgery Kay Washington, MD, PhD University Hospital Zurich Department of Pathology Zurich, Switzerland Vanderbilt University Medical Center Nashville, Tennessee, USA C. Wright Pinson, MD, MBA Department of Surgery Markus Weber, MD Division of Hepatobiliary Surgery and Liver Transplantation Swiss Hepato-Pancreato-Biliary Center Vanderbilt University Medical Center Department of Visceral and Transplant Surgery Nashville, Tennessee, USA University Hospital Zurich Zurich, Switzerland Robert J. Porte, MD, PhD Department of Surgery Stefan Wildi, MD Division of Hepatobiliary Surgery and Liver Transplantation University Medical Center Groningen Swiss Hepato-Pancreato-Biliary Center Groningen, The Netherlands Department of Visceral and Transplant Surgery University Hospital Zurich Markus Selzner, MD Zurich, Switzerland Swiss Hepato-Pancreato-Biliary Center Department of Visceral and Transplant Surgery University Hospital Zurich Zurich, Switzerland Preface Diseasesofthe gallbladder and bile ducts are common and added two new chapters, one on “Epidemiology of diseases of major focuses in gastroenterology, oncology, radiology, nu- the bile duct and gallbladder”by Dr Markus Heim from Basel clear medicine, and surgery. These past two decades have (Chapter 3), and one on “Noninvasive imaging of the biliary brought numerous new diagnostic and therapeutic modali- system” by Drs Elmar Merkle and Rendon C. Nelson from ties ranging from mini-invasive proceduressuch as complex Duke University and Dr HenrikPetrowsky fromthe Univer- endoscopic or laparoscopic procedures to new techniques of sity Hospital Zurich. A number of new authors have been in- liver transplantation. Major advances have also been made in vited based on their expertise and excellence in science the understanding of the pathogenesis of a variety of condi- writing. Finally, to enhance the didactic aspect of the book tions and the natural history of previously unclear entities. wehaveincluded objectives at the beginning of each chapter While this has led to better “evidence-based” treatments of and, at the end, a series of key questions covering the main patients, the proliferation of new diagnostic and therapeutic message of each respective chapter. tools has also led to confusion about which therapy to select This second edition is designed to serve the needs of all for particular situations. The modern treatment of biliary those involved in the management of patients with biliary diseases must be approached through a multidisciplinary diseases from medical students tospecialists in various areas. team having special knowledge in this field. In addition, a The first series of three chapters comprehensively covers number of innovative approaches are still experimental and anatomy, physiology and pathology, and imaging modalities often technically demanding, so that complex biliary prob- of the biliary tree. The next eight chapters present various lems should be managed in centers with experience in treat- therapeutic approaches involving medical, endoscopic, and ing these patients and a strong commitment to research. percutaneous treatments, as well as open and laparoscopic To this end, the first edition of Diseases of the Gallbladder and surgery. Then, four chapters are dedicated to the gallbladder Bile Ducts: Diagnosis and Treatmentpublished in 2001 has pro- followed by six chapters about a variety of common diseases, vided a comprehensive and critical approach to established such as acute cholangitis, and less common intra- and extra- and new diagnostic and therapeutic modalities. The book hepatic biliary diseases. Finally, a chapter is dedicated to the was written by a multidisciplinary panel of international ex- complex problem of intrahepatic cholestasis, and the last perts with extensive experience in this population of pa- chapter covers specific biliary disorders in the pediatric tients. Due to rapid developments in this field, we felt it was population (Chapter 23). necessary to prepare a second edition. This new edition was We hope thatthis new edition of Diseases of the Gallbladder done with the same spirit and format. To further secure qual- and Bile Ducts: Diagnosis and Treatmentwill provide timely in- ity, to achieve the comprehensive and balanced coverage of formation and guidelines for the management of this popula- each topic, to avoid redundancy among chapters, and to pro- tion of patients. vide appropriate cross-references, we included two Associate Editors, Dr Michael A. Morse from Duke University and Dr P.-A.C. Markus Selzner from the University Hospital Zurich. We also J.B. ix

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Prothrombin time. PTBD. Percutaneous transhepatic biliary drainage. PTC tokinin (CCK), the main gallbladder-contracting hormone,.
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