Colorectal Surgery

Mark Killingback, AM, MS(Hon), FACS(Hon),FRACS, FRCS, FRCSEd

Colorectal SurgeryLiving Pathology in the Operating Room

Mark Killingback, AM, MS(Hon), FACS(Hon), FRACS, FRCS, FRCSEd18/1 Lauderdale AvenueFairlight 2094Australia

Library of Congress Control Number: 2006921548

ISBN-10: 0-387-29081-8ISBN-13: 978-0387-29081-2

Printed on acid-free paper.

© 2006 Springer Science+Business Media, Inc.All rights reserved. This work may not be translated or copied in whole or in part without the writtenpermission of the publisher (Springer Science+Business Media, Inc., 233 Spring Street, New York, NY10013, USA), except for brief excerpts in connection with reviews or scholarly analysis. Use in connection with any form of information storage and retrieval, electronic adaptation, computer soft-ware, or by similar or dissimilar methodology now known or hereafter developed is forbidden.The use in this publication of trade names, trademarks, service marks, and similar terms, even if theyare not identified as such, is not to be taken as an expression of opinion as to whether or not they aresubject to proprietary rights.While the advice and information in this book are believed to be true and accurate at the date of goingto press, neither the authors nor the editors nor the publisher can accept any legal responsibility for anyerrors or omissions that may be made. The publisher makes no warranty, express or implied, with respectto the material contained herein.

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To Bobbie, my wife of more than 50 years, who has made manysacrifices as the wife of a surgeon and without whom this workwould not have been completed.

To Sir Ian Todd, who supported my appointment as a ResidentSurgical Officer to St Mark’s Hospital in 1960, which determinedmy career path in surgery.

To my mentors, the late Edward Wilson and the late Sir Edward(Bill) Hughes, who were pioneers in colorectal surgery, master sur-geons, prolific authors, innovators, and valued friends.

Books addressing the issues of colorectal surgery tend to take a familiarformat. Frequently multiauthored, especially for comprehensive presen-tations on current status of the specialty, there are few single authoredtexts available. As for this book by Mark Killingback, one is not awareof any comparable treatises devoted to colorectal surgery. So what makesthis so unique? And what makes the acquisition and reading of this book so desirable? First, a certain amount of historical perspective. Untilthis time—and one hopes for sometime yet to come—descriptions offindings at operation, and what was done to correct them, have been considerably augmented—and clarified—by schematic diagrams. (Thereference to “sometime to come” is based on the emergence of the e-chart and e-operative note which promises to make such documentsentirely paperless).

Dr. Killingback throughout his distinguished and prolific career haspracticed the habit of schematically representing his operations—afterthe intervention—usually with captions. It is a practice he taught manyof us. This exemplifies the phrase “a picture is worth a thousand words.”However in the course of time, he acquired the skills of an artist and soconverted basic line drawings into an art form.

Well, that is nice, you might say. But what does this offer over andabove a good photograph of the specimen or of the operative field? Thisis the distinguishing point. Note how difficult it is to convey the spec-trum of the disease or the extent of the difficulty of an operation or showmanifestations of a particular syndrome in a photograph—or even a con-ventional line drawing! How does one adequately convey to the reader,the tapestry, the protean manifestations of Crohn’s disease, for example,in a single drawing? In Dr. Killingback’s imagery, all the features of thick-ened, strictured, obstructive, perforative, fistulizing, and ulceratedintestines are shown in one masterful piece of art. Photographic attemptsfor similar documentation are fortunate to provide two or three such features.

The experienced surgeon will appreciate this book by recognizing thedetails and exquisitely rendered images that call to mind similar casesencountered. For the surgeon or trainee relatively new to the specialty ofcolorectal surgery, the graphic presentation of the surgical pathology,with the accompanying succinct and informative text will make theacquisition of this book a valuable one.

Victor W. Fazio, MDCleveland, OH

Stanley M. Goldberg, MDMinneapolis, MN

Foreword

vii

This book makes no claims to be a textbook of colorectal surgery, asmany aspects of this specialty are not included. It is rather a collectionof cases illustrating surgical pathology as encountered by a surgeon per-forming operations for colorectal disease. The surgeon is the first, in whatmay be a succession of medical practitioners, to confront the pathologyof the disease “face to face.” It is a unique opportunity to see the pathol-ogy in vivo in its undisturbed state and the interpretation of this mor-phology is usually vital to the operative technique to follow. In 1907Moynihan of Leeds General Infirmary (UK) wrote on one of his favoritethemes “The Pathology of the Living.”1 He stressed the value of obser-vations of pathology during abdominal surgery and how this influenceddiagnosis and treatment. The title of this book is related to this philos-ophy of surgery proposed by Moynihan. The aim of this work is princi-pally to present illustrations of surgical pathology with artistic merit forsurgeons to include in their reference library as a “coffee table book” butthe author hopes the art and case history texts will have a significanteducational role. Perhaps its main value will be for the younger surgeonwho is commencing the journey into unchartered waters of surgicalpathology. The author certainly would have valued a forewarning ofmany of the cases presented in this publication.

Drawing was selected for the illustrations as an art form rather thanphotography. Illustrative art has the facility to probe into inaccessibleareas of the abdomen, to manipulate perspective to include importantdetails, and to emphasise or delete various parts of the subject. Illustra-tion can also combine the internal and external views of a viscus, etc.,in the one diagram.

The author has enjoyed a long standing interest in drawing andusually included this aspect in operation report records. The contribu-tion of the medical artist to surgical education was emphasized to theauthor in 1958–1959 while working as a surgical registrar at the CentralMiddlesex Hospital London. Ms. Mary Barber was a full-time medicalartist employed by the hospital working in a very small cottage in thehospital grounds. With watercolor painting, the artist produced beautifulillustrations of surgical specimens. Most of her work was generated bythe senior surgeon, T.G.I. James, who himself had a great interest inrecording surgical pathology. The quality of Ms. Barber’s work can beseen in her illustration of bowel affected by necrotising colitis2 (Figure1). Although this type of artwork has been somewhat overshadowed bycolor photography, perhaps this book will demonstrate that there is stillvalue in illustrative artwork. The evolution of the illustrations has beenpresented in three stages. On completion of an operation the author’spractice was to open the specimen and pin the bowel to a corkboard forthe pathologist. A rough sketch was made to record details. This sketchformed the basis for an improved diagram for the patient’s record (Figure2). Such diagrams have then facilitated third illustrations prepared forthis book. The author practiced colorectal surgery as a specialty for 26 ofthe 39 years of operating experience. Patients described in this book were

Preface

ix

managed by the author, who performed the surgery on the pathologydepicted in all cases, with the exception of: Case 21, lipomatosis-referredafter retirement; Case 49, composite diagram; Case 78, desmoid tumour-no operation and Case 79, pneumatosis-no operation. The observationsare therefore personal and prospective. The author has maintained hisown detailed records of all patients treated, and this has restricted aminimum need for retrospective searching of patient details in hospitalrecords. Follow-up cases were routine in patients with neoplastic disease,but in many cases not requiring follow-up for management. The patientshave been located by the author and follow-up details were establishedby phone. A number of patients underwent related operations by othersurgeons either prior to the author’s involvement or subsequently. Thestated age of the patient is that at the time of the initial referral.

Many surgeons have an interest in recording operation details by dia-grams which can become invaluable in the management of the patient.Victor Fazio attributes his interest in this method of recording operationdetails, to his mentor the late Rupert B. Turnbull Jr. who was an enthu-siastic sketcher of what he observed in the operating room. There are afew publications, however, that feature medical artwork by surgeons. SirCharles Bell (1774–1842), of London, was a surgeon-anatomist and a tal-ented artist who illustrated many texts with neuroanatomical drawings.His famous paintings of war wounds from the Napoleonic wars are nowwith the Royal College of Surgeons of Edinburgh.3 Bateman in his bookBerkeley Moynihan Surgeon relates that in the early part of the 1900s

x Preface

Figure 1: Necrotizing colitis. (Painting by M. Barber, 1959)

this doyen of British surgery was an enthusiastic sketcher of his findingsat operation.4

At the end of each operation he would draw with coloured crayons upon a thinwhite sheet of cardboard an exact picture of the abnormalities he had seen whileoperating. This he would accompany with illustrations and descriptive matterexplaining the curative methods he had adopted. He had a swift, light touchthat made his drawings very clear in an incisive way they told more than thecopious written notes could do. These little sketches were bound in the volumesof his case records.

The location of these records is unfortunately unknown at thepresent time. During the preparation of this book one other similar pub-lication has appeared describing operative details of 100 personal casesof interest with accompanying diagrams by the surgeon-author M. Tredeof Germany.5 This book contains black/white and color drawings, withaccompanying text, that devotes much attention to operative technique.It covers a wide spectrum of surgery including cardiac, pulmonary, vas-cular and abdominal surgery, the latter concentrating on a unique expe-rience of pancreatic disease. As one reads the book the impact of thepersonal contribution of the surgeon is obvious.

Colorectal Surgery: Living Pathology in the Operating Room restrictsitself to the specialty but should be of interest to those who practice

Preface xi

A

B

Figure 2: Contemporary diagram (1998) used for patients’ records, later used toproduce artwork. (Case 23)

general surgery. There is minimal inclusion of operative technique,which has been well covered by many quality textbooks, but lessons inpatient management have been included wherever appropriate in thecomment section of each case. The text describes some successes of sur-gical treatment but errors of judgement and disappointing results areemphasized. All surgeons are aware of the importance of understandingpathology and its relationship to appropriate surgical treatment. Thereare many prestigious textbooks of pathology to which surgeons may refer,but such publications written by pathologists cannot be expected to linkthe clinical and operative management to pathology in the one book.This aspect has been a motivation for this publication. The referencesare not as extensive as might accompany a case report in a journal or atextbook. They have been restricted to suit the needs of the case histo-ries, which are supplementary to the illustrations. An effort has beenmade to include current references but in relation to some of the uncom-mon conditions, publications are few and have appeared many years previously.

Philip H. Gordon, a colorectal surgeon from Montreal has written apaper on the problems of producing a medical book.6 In this he quotesApley:7 “. . . writing is like having a baby: the gestation period is long andthe labor painful, but in the end you have something to show for it.” Ihope what this book has to show will be of interest to my fellow sur-geons. The labor of producing the illustrations was not painful but a pleasurable exercise, which has taught me more about the surgicalpathology of colorectal disease than I knew previously. I hope the resultsdo the same for the reader.

Mark Killingback, AM, MS(Hon), FACS(Hon), FRACS, FRCS, FRCSEd

References

1. Moynihan BGA. An address on the pathology of the living. Br. Med. J.1907;2:1381–5.

2. Killingback M, Lloyd-Williams K. Necrotising colitis. Br. J. Surg.1961;49:175–85.

3. Crumplin MKH, Starling P. A surgical artist at war. The paintings andsketches of Sir Charles Bell 1809–1815. Edinburgh, The Royal College ofSurgeons of Edinburgh, 2005.

4. Bateman D. Berkeley Moynihan Surgeon London, McMillan and Co,1940.

5. Trede M. The art of surgery: Exceptional cases—unique solutions 100case studies. Thieme Verlag, Stuttgart, Germany, 1999.

6. Gordon PH. So you want to write a textbook? J. R. Soc. Med.2000;93:150–1.

7. Apley AG. So you want to get published. J. R. Soc. Med. 1993;86:6–8.

xii Preface

My surgical colleagues have encouraged me to publish artwork and Ithank them for that support. Drs. Victor Fazio and Stanley Goldberg fromthe United States have been most helpful in supporting the publicationof this book and reviewing its contents. My colorectal surgeon col-leagues, Drs. P. Chapuis, M. McNamara, and the late W. Hughes assistedat the majority of the operations and their operative skills and coun-selling while operating was invaluable. I am indebted to pathologists,Drs. Suzanne Danieletto, Stan McCarthy, and Ron Newland for thepreparation of the photomicrographs and their advice on many aspects ofthe pathology. It is important to acknowledge the assistance I had formany years with record keeping and follow-up of patients. Nurse JennySearle was responsible for initiating this aspect of my practice, and PrueBarron continued this with meticulous care. Diana Murray has typed themany drafts and final copy of the manuscript. She has done this withconsiderable expertise and unfailing interest in the project. I am gratefulto my art teacher Gwen Kowalski, who has been most encouraging eventhough some of the sketches unnerved the rest of the art class. I owe adebt of gratitude to Beth Campbell of Springer Science+Business Media,who has been enthusiastic about the book, supported its publication, andassisted greatly in liaising with the publisher. A text cannot be completewithout references and I should acknowledge the most helpful assistanceI have received over a prolonged period from Ilona Harsanyi, Ann Gilbert,and Eric Gaymer of the Charles Winston Library in Sydney Hospital.

Mark Killingback, AM, MS(Hon), FACS(Hon), FRACS, FRCS, FRCSEd

Acknowledgments

xiii

Foreword by Victor W. Fazio and Stanley M. Goldberg . . . . . . . . viiPreface . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . ixAcknowledgments . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . xiii

PART I SMALL BOWEL

1. Lipoma: Terminal Ileum . . . . . . . . . . . . . . . . . . . . . . . . . . . 22. The Intruding Carcinoid . . . . . . . . . . . . . . . . . . . . . . . . . . . 43. Carcinoidosis of the Ileum . . . . . . . . . . . . . . . . . . . . . . . . . 64. GIST Tumor of Ileum . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 85. Adenocarcinoma of the Jejunum . . . . . . . . . . . . . . . . . . . . . 106. Blind Pouch Syndrome After Bowel Resection . . . . . . . . . . . 127. Blind Pouch Syndrome After Ileorectal Anastomosis . . . . . . 14

PART II APPENDIX

8. Acute Appendicitis: Diagnosis at Colonoscopy . . . . . . . . . . 189. Mucocele of the Appendix . . . . . . . . . . . . . . . . . . . . . . . . . . 20

10. Cystadenoma: Appendix . . . . . . . . . . . . . . . . . . . . . . . . . . . 2211. Carcinoma of the Appendix . . . . . . . . . . . . . . . . . . . . . . . . . 24

PART III POLYPS-POLYPOSIS

12. A Mega Polyp Associated with a Micro Cancer . . . . . . . . . . 2813. Extensive “Benign” Polyp of the Rectum and

Sigmoid Colon . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 3014. A Bad Result from a Successful Operation for a Polyp

in the Sigmoid Colon . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 3215. One Operation for Double Pathology . . . . . . . . . . . . . . . . . . 3416. Juvenile Polyposis and Rectal Prolapse . . . . . . . . . . . . . . . . 3617. Juvenile Polyposis in an Adult . . . . . . . . . . . . . . . . . . . . . . 3818. Chronic Intussusception of the Colon Due to

Peutz-Jeghers Syndrome . . . . . . . . . . . . . . . . . . . . . . . . . . . 4019. Carcinoma of the Rectum: FAP and Rectovaginal

Fistula . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 4220. Ileorectal Anastomosis for FAP: Rectal Cancer . . . . . . . . . . 4421. Large Bowel Lipomatosis . . . . . . . . . . . . . . . . . . . . . . . . . . . 4622. A Polypoid Lesion in the Sigmoid Colon . . . . . . . . . . . . . . . 48

PART IV CANCER OF THE COLON AND RECTUM

23. Synchronous Colon Carcinoma and Malignant Carcinoid . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 52

24. Coexistent Cancer and Diverticulitis . . . . . . . . . . . . . . . . . 5425. Sigmoid Carcinoma and Serosal Cysts . . . . . . . . . . . . . . . . . 5626. Cavitating Cancer of the Transverse Colon . . . . . . . . . . . . . 5827. The Wagging Tongue of a Sigmoid Cancer . . . . . . . . . . . . . . 6028. Protracted Recurrence of Mucoid Cancer . . . . . . . . . . . . . . . 62

Contents

xv

29. Anaplastic Colon Cancer . . . . . . . . . . . . . . . . . . . . . . . . . . . 6430. Linitis Plastica of the Colon and Rectum . . . . . . . . . . . . . . 6631. Curative Resection of Rectal Cancer Despite Liver

Metastases . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 6832. Small Sigmoid Cancer: “Mega” Lymph Node

Metastasis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 7033. Rectal Cancer Infiltrating the Buttock Via an Anal

Fistula . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 7234. Lucky Local Recurrence . . . . . . . . . . . . . . . . . . . . . . . . . . . 7435. Thoraco-Abdominal Approach to Carcinoma of the

Splenic Flexure . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 76

PART V DIVERTICULAR DISEASE

36. Was It Diverticulitis? . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 8037. Large Pseudopolyp of the Sigmoid Colon . . . . . . . . . . . . . . . 8238. Which Operation for Acute Diverticulitis with

Peritonitis? . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 8439. Waiting to Die . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 8640. Distal Abscesses and Diverticular Disease . . . . . . . . . . . . . . 8841. Coloperineal Fistula . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 9042. Diverticulitis: Extensive Abscess in the Mesorectum . . . . . 9243. Diverticulitis: Colovesical Fistula . . . . . . . . . . . . . . . . . . . . 9444. Dissecting Diverticulitis . . . . . . . . . . . . . . . . . . . . . . . . . . . 9645. Annular Extramural Dissecting Diverticulitis . . . . . . . . . . . 9846. Giant Diverticulum . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 10047. Giant Diverticulum . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 10248. Diverticulitis: Large Bowel Obstruction . . . . . . . . . . . . . . . 104

PART VI INFLAMMATORY BOWEL DISEASE

49. Ulceration in Crohn’s Disease of the Small Bowel . . . . . . . . 10850. Recurrent Crohn’s Disease . . . . . . . . . . . . . . . . . . . . . . . . . 11051. Crohn’s Disease: Strictures of Ascending Colon and

Duodenum . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 11252. The Appendix, Fistulae, and Pseudopolyps in Crohn’s

Disease . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 11453. A “Shamrock” Deformity Due to Crohn’s Disease . . . . . . . 11654. A Short “Hose Pipe” Colon: Crohn’s Disease . . . . . . . . . . . 11855. Recurrent Crohn’s Disease: Pseudopolyposis . . . . . . . . . . . . 12056. Presentation of Crohn’s Ileitis as an Abdominal

Malignancy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 12257. Crohn’s Disease 19 Years After Initial Resection . . . . . . . . . 12458. Large Bowel Obstruction: Crohn’s Disease . . . . . . . . . . . . . 12659. Subacute Toxic Megacolon Due to Ulcerative Colitis . . . . . 12860. Colitis and Pseudopolyposis . . . . . . . . . . . . . . . . . . . . . . . . 13061. Ileorectal Anastomosis for Chronic Ulcerative Colitis:

Early Diagnosis of Carcinoma: Late Diagnosis of Large Polypoid Lesion . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 132

62. Childhood Ulcerative Colitis: Rectal Cancer . . . . . . . . . . . . 13463. Obstructive Colitis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 13664. Pseudomembranous Colitis and Toxic Megacolon . . . . . . . . 13865. Ileocecal Tuberculosis Mimicking Crohn’s Disease or

Vice Versa? . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 140

xvi Contents

PART VII LYMPHOMA

66. Burkitt’s Lymphoma (Ileum) with Intussusception . . . . . . . . 14467. Ileocecal Lymphoma . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 14668. Multiple Lymphoma and Ulcerative Colitis . . . . . . . . . . . . . 14869. Lymphoma of the Rectum . . . . . . . . . . . . . . . . . . . . . . . . . . 150

PART VII ANORECTAL DISEASE

70. An Intrasphincteric Anal Tumor . . . . . . . . . . . . . . . . . . . . . 15471. Aggressive Pelvic Angiomyxoma of the Pelvis . . . . . . . . . . . 15672. Implantation Metastasis into an Anal Fistula . . . . . . . . . . . 15873. Local Excision of a Rectal Carcinoma Can Be an Easy

Operation . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 16074. Proctitis Cystica Profunda . . . . . . . . . . . . . . . . . . . . . . . . . . 16275. Rectopexy for a Rectal Stricture-Ulcer . . . . . . . . . . . . . . . . . 16476. Intersphincteric Anal Fistula with Proximal Perirectal

Extension . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 16677. Necrotizing Infection After Removal of “Benign”

Rectal Polyp . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 168

PART IX VARIOUS PATHOLOGY

78. Intra-Abdominal Desmoid Tumor Unassociated with Familial Adenomatous Polyposis . . . . . . . . . . . . . . . . . . . . . 172

79. Pneumatosis Coli . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 17480. Stercoral Ulceration: Sigmoid Perforation . . . . . . . . . . . . . . 17681. Nongangrenous Ischemic Colitis . . . . . . . . . . . . . . . . . . . . . 17882. Infarction of the Omentum . . . . . . . . . . . . . . . . . . . . . . . . . 18083. Metastatic Linitis Plastica of the Colon . . . . . . . . . . . . . . . 18284. Lipoma Transverse Colon . . . . . . . . . . . . . . . . . . . . . . . . . . 18485. Intestinal Endometriosis . . . . . . . . . . . . . . . . . . . . . . . . . . . 18686. Hirschsprung’s Disease . . . . . . . . . . . . . . . . . . . . . . . . . . . . 18887. Gallstone Obstruction: Sigmoid Colon . . . . . . . . . . . . . . . . 19088. Intussusception of the Colon . . . . . . . . . . . . . . . . . . . . . . . . 192

PART X COMPLICATIONS OF INVESTIGATION ANDTREATMENT

89. Barium Perforation of the Rectum . . . . . . . . . . . . . . . . . . . . 19690. Colonoscopy Injury to the Colon . . . . . . . . . . . . . . . . . . . . . 19891. Mesenteric Thrombosis After Colon Resection . . . . . . . . . . 20092. Postoperative Abdominal Apoplexy . . . . . . . . . . . . . . . . . . . 20293. Local Excision of Rectal Cancer and Radiotherapy . . . . . . . 20494. Residual Diverticulitis After Resection Causing an

Elongated Abscess with Prolongated Solution . . . . . . . . . . . 20695. Perforated Diverticulitis and Its Consequences . . . . . . . . . . 20896. Anastomotic Dehiscence After Anterior Resection . . . . . . . 21097. Postoperative Necrosis of the Left Colon . . . . . . . . . . . . . . . 21298. Ileostomy Closure: An Impasse Due to Adhesions . . . . . . . . 21499. Perforation of the Sigmoid Colon Due to Radiation

Injury . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 216100. Radiation Rectovaginal Fistula . . . . . . . . . . . . . . . . . . . . . . 218

References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 221Appendix . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 233Index . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 255

Contents xvii

P A R T

I Small Bowel

HistoryDark red rectal bleeding and melena occurred overseveral days, 4 weeks prior to the patient’s referral.Chest pain occurred during this period diagnosed asangina. Colonoscopy revealed diverticular disease ofthe sigmoid colon and a lobulated polyp protrudingthrough the ileocecal valve. The polyp intermit-tently retracted from view, and examination beyondthe ileocecal valve confirmed its attachment to theterminal ileum by a broad pedicle. Biopsy showednonspecific inflammatory changes. A small bowelseries confirmed the polyp in the terminal ileumand suggested this was a solitary lesion.

Operation (12.22.95)The lesion in the terminal ileum was soft andrubbery on palpation with a broad attachment to thewall of the bowel. There were no enlarged lymphnodes in the mesentery. Eleven cm of terminalileum was resected and an end-to-end anastomosisperformed with a single layer of interrupted poly-glactin 910 (vicryl) sutures.

C A S E

PathologyThe polypoid lesion was pale yellow in color withsmooth mucosa covering a lobulated surface. It measured 32 × 28 × 28mm. There was a vascularulcer on the distal aspect interpreted as the site ofbleeding. The diagnosis of lipoma was confirmedhistologically.

CommentTumors of the small bowel are uncommon, andMinardi et al. report the incidence of lipomas in thesmall bowel to be 4.5%.1 They are usually submu-cosal but may be subserosal. When symptomatic,the most common presentation is abdominal paindue to intussusception. Bleeding which occurred inthis patient is much less common and was probablydue to venous congestion and ulceration on the tipof the polyp. Barium enema or CT may demonstratethe lesion.2 Newer endoscopy techniques and thesmall intestine camera (“pill cam”),3 should signifi-cantly improve the opportunity for preoperativediagnosis.

1 Lipoma: Terminal IleumMale, 81 Years

2

Diagram 1 3

HistoryThe patient was examined by colonoscopy as aroutine follow up procedure in view of a past historyof three small benign polyps in the ascending colon.There were no gastrointestinal symptoms. Threehyperplastic polyps (3mm) were removed from thesigmoid (1) ascending colon (2). A polypoid lesionwas noted in the partially open ileocecal valve,which was red and smooth. Attempts to biopsy thiswere unsuccessful. Endoscopy of 10–12cm of ter-minal ileum proximal to the polypoid lesion showedno abnormality of the mucosa.

Operation (11.22.93)A firm mass (30 × 30mm) was present in the ileo-cecal angle, attached to the ileum. It appeared tohave expanded within the mesentery and was con-tinuous with an intraluminal component within the terminal ileum. The operative diagnosis wasleiomyoma. The remainder of the small bowel wasnormal. A right hemicolectomy was performedwhich included 90mm of ileum.

PathologyThe lesion within the lumen of the ileum was a firm “sausage” shaped polypoid tumor, which had

C A S E

extended through the ileocecal valve into thececum. It was continuous with the extramural massand on section had a slightly yellowish color. Theluminal component was covered with normalmucosa. Histological examination confirmed thediagnosis of carcinoid tumor (Figure 2.1). There weresix lymph nodes found in the adjacent small bowelmesentery, the largest of which contained metasta-tic carcinoid.

Follow-Up (2004)The patient’s progress has been monitored withregular clinical examination, abdominal CT,colonoscopy, and urinary assay for 5-hydroxy-indole-acetic-acid excretion. No abnormalities havebeen detected. The patient remains in good health11 years since operation.

CommentThis patient’s carcinoid tumor was diagnosed bychance during a follow up examination for previouslarge bowel polyps. Diagnosis by colonoscopy mustbe very unusual. Incidental diagnosis, usually atlaparotomy, has been reported to occur in up to 60%of cases.1 At laparotomy, the “dumbbell” morphol-ogy of the luminal and mesenteric elements sug-gested the tumor was a leiomyoma. Carcinoidsoccur mostly in the lower third of the ileum, com-prising up to 34% of all small intestinal neoplasmsand up to 46% of malignant neoplasms.2 Most car-cinomas of the ileum produce serotonin and sub-stance “P,” which is common in the presence ofhepatic metastases. It is not unusual for carcinoidtumors to be multiple, and there is a significantassociation with other types of synchronousprimary malignancy, usually in the gastrointestinaltract.3 The presence of nodal or other metastases isrelated to the size of the primary tumor. In a litera-ture review Memon et al. found the size:metastasisrelationship to be: <1cm: 20–30%, 1–2cm: 60–80%,>2cm: >80%.3

2 The Intruding CarcinoidFemale, 62 Years

4

Figure 2.1: Section shows sheets of small bland cellstypical of carcinoid.

Diagram 2 5

HistoryThe patient presented with a family history of colorectal cancer (mother) and recent increase inrectal bleeding. At colonoscopy, seven polyps in thedescending and sigmoid colon were removed bydiathermy snare. Six polyps were ≤5mm in size(benign). The largest polyp was situated in the distalsigmoid colon on a short broad pedicle and mea-sured 18mm. This polyp was a villous adenoma containing infiltrating, moderately differentiatedcarcinoma. After a detailed discussion with thepatient, colon resection was recommended.

Operation (7.24.89)Laparotomy revealed no obvious pathology in thecolon or metastases related to the malignant polyp.On examination of the small bowel, 11 small, firmlesions were palpable over 60cm of the terminalileum. The largest “nodule” was associated withpuckering on the serosal surface and slightlyenlarged hard lymph nodes in the adjacent mesen-tery. The abnormal area of ileum and mesenterywere resected with anastomosis. The site of themalignant polyp was managed by a high anteriorresection.

PathologyThe resected colon contained no residual adenocar-cinoma. Examination of the mucosal surface ofresected ileum revealed an additional 11 nodulespreviously undetected by palpation during opera-tion. The 22 lesions ranged in size from 2mm to 12mm. Histological examination confirmed thediagnosis of multiple carcinoids. Twenty-one of thetumors were confined to the mucosa or submucosa.The largest tumor showed deep extension into themuscularis propria. Three of 5 mesenteric lymphnodes contained metastatic carcinoid tumor.

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Operation (3.16.90)A “second look” laparotomy was performed 8months after the bowel resections to detect carci-noid tumors that may have been missed at that oper-ation. None were found. There was no evidence ofmetastatic disease. Appendectomy was performed.

Follow-Up (2004)Clinical and biochemical assay of urine 5-hydroxy-indole-acetic-acid assessment has shown no evi-dence of recurrent carcinoid tumor now 14 years, 10months after resection. Colonoscopy surveillancehas continued with the occasional removal of smallbenign polyps. In October 1997, carcinoma of theleft breast was treated by mastectomy and postop-erative chemotherapy.

CommentThere have been very few reported cases of this largenumber of small bowel carcinoids in associationsynchronously with colorectal cancer (CRCa).1 Thediagnosis of carcinoid tumors of the small bowel isfrequently made incidentally during a laparotomyfor other abdominal pathology. Early diagnosis isotherwise unusual. There could be some debateabout the need for colon resection performed for thispatient’s adenomatous sigmoid polyp containing afocus of cancer. It certainly facilitated earlier diag-nosis of the malignant carcinoid. While multiplecarcinoids of the ileum are not unusual, 22 syn-chronous tumors is a rarity. In Thompson’s reviewfrom the Mayo Clinic, the largest number of multi-ple carcinoids in the ileum was 24.2 The “secondlook” laparotomy was useful and reassuring, but theuse of the intraluminal small bowel camera (capsulevideo endoscopy) at the present time would be pre-ferred to a “second look” laparotomy.3

3 Carcinoidosis of the IleumFemale, 56 years

6

Diagram 3 7

7.24.89

7.11.89

HistoryFor a few months, the patient had noticed intermit-tent pain in the right iliac fossa. There were no gastrointestinal symptoms. On referral to a gyne-cologist, a mobile firm swelling was palpable in theabdomen. The diagnosis of an ovarian tumor wasmade and operation advised.

Operation (5.22.95)Laparotomy revealed a soft lobulated tumorattached to the lower ileum over a moderatelylimited area of the surface of the bowel so that thetumor “flopped” about on manipulation of theileum. There were no enlarged lymph nodes in the adjacent mesentery or evidence of metastaticdisease. Examination of the rest of the small boweland large bowel revealed no abnormality. The uterusand ovaries appeared normal. At this stage thepatient was referred. Resection of 12cm of ileumand related mesentery was performed. An end-to-end anastomosis was constructed with a single,interrupted layer of polyglactin 910 (vicryl) suture.

PathologyThe tumor measured 60 × 60 × 60mm. No commentwas made on the appearance of the cut surface. Histologically, the lesion appeared to be arising

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from the muscularis propria of the bowel wall. Itwas composed of spindle cells with no evidence of atypia (Figure 4.1). The mitotic rate in some areas was 2 mitotic figures per 10 high-power fields.There was no evidence of tumor necrosis, but therewere areas of hemorrhage. The report stated thetumor was a “smooth muscle tumor of uncertainmalignant potential, but in view of the frequency of mitotic figures, the lesion is best regarded asmalignant.” Subsequent immunohistochemicalstaining with CD 117 was positive, therefore clas-sifying the lesion as a gastrointestinal stromaltumor (GIST).

Follow-Up (2004)The patient has remained well without any gas-trointestinal symptoms 9 years and 5 months sinceoperation. The patient is not having follow-up inves-tigations as a routine.

CommentThe GIST is the most common mesenchymal tumoroccurring in the small bowel.1 The diagnosis is madeon immunohistochemical investigation with CD117 proto-oncogenic receptor positive in 100% ofcases and CD 34 antigen positive reactivity in70–80%.2 The diagnosis can also be made on ultra-structural study.3 The surgical removal of thispatient’s lesion proved to be without difficulty as itwas not adherent to any other abdominal structure.There were no macroscopic signs of malignancy, butthis was inferred on histological examination on the basis of mitoses per high power field. Diagnosisof malignancy in the GIST lesion is difficult andWolber and Scudamore have suggested that two ormore of the following features may confirm malig-nancy: large size; tumor necrosis; spontaneous coag-ulation; infiltrative margins; high mitotic count;and nuclear pleomorphism.4 Clary et al. from theMemorial Sloan-Kettering Cancer Center reviewed215 patients with stromal tumors of the gastroin-testinal tract in which the incidence of malignantbehavior was high.5 They reported a local recurrencerate of 36% and a five year specific survival rate of28%5 and emphasize the importance of completeexcision of the GIST lesion.

4 GIST Tumor of IleumFemale, 67 Years

8

Figure 4.1: Section shows GIST spindle cells within acollagen stroma.

Diagram 4 9

HistoryThe patient was referred for investigation of an irondeficiency anemia which bone marrow studies sug-gested was due to chronic blood loss. Over a periodof 6 months the patient had suffered episodicabdominal pain of a colicky type and noticed theonset of fatigue and exertional dyspnea. Panen-doscopy and colonoscopy soon after the onset ofsymptoms revealed no abnormality. These endo-scopies were repeated 6 months later and againfailed to find a cause for bleeding. A small bowelenema x-ray revealed a stricture of the upperjejunum (Figure 5.1).

Operation (3.22.91)Laparotomy revealed a neoplastic mass in the upperjejunum with possible involvement of the serosalsurface. The associated small bowel mesentery con-tained several enlarged lymph nodes (the largestmeasured 20mm in diameter). There were no otherabnormalities on examination of the abdominalviscera or peritoneum. Twenty-five cm of jejunumwas resected, with a deep resection of the adjacentmesentery. An end-to-end anastomosis was per-formed with a single layer of interrupted vicrylsutures and the mesentery closed.

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PathologyExamination of the mucosal surface revealed anannular tumor with proliferative edges and centralulceration. The appearances were similar to that ofcolon cancer. The cut surface of the tumor was palewith yellowish foci. Histologically, the tumor was ahigh grade adenocarcinoma (Figure 5.2). The tumordeeply invaded the muscularis propria but did notinvolve the peritoneal surface. Nine lymph nodeswere examined, none of which contained metastases(Dukes A, T2 N0 M0).

Follow-Up (2005)There has been no evidence of recurrent diseaseduring the 14 years of follow-up.

CommentThe diagnosis of this patient’s small bowel malig-nancy was delayed for 6 months despite the atten-tion of two gastroenterologists. Such delay is notunusual.1 Although adenocarcinoma of the smallbowel accounts for less than 2% of gastrointestinalmalignancies, it is the more common cancer affect-ing the small bowel.1 Kusumoto et al. report that thesmall bowel enema x-ray achieved 83% accuracy indiagnosis.2 Abdominal computerized tomography

5 Adenocarcinoma of the JejunumFemale, 68 Years

10

Figure 5.1: Small bowel enema x-ray demonstrates thejejunal carcinoma (“apple core”). See arrow.

Figure 5.2: This section shows sheets of high gradeadenocarcinoma deep to the muscularis mucosae.

(CT) is not proven to be this accurate but may giveadditional information on the extent of the tumor.Capsule endoscopy may in the future surpass radiological techniques. These lesions, treated bysegmental resection, do not usually present a dif-ficult technical challenge. The role of adjuvantchemotherapy is not established at the present time.Howe et al. report from the National Cancer Data

Diagram 5 11

Base (USA) the results of 1528 cases of adenocarci-noma of the small intestine from 1985–1995. Therewere 880 cancers of the jejunum and 648 cancers ofthe ileum, a ratio of 1.4 :1.0. The cancer-specific 5-year survival rate was 37.8%, confirming that theprognosis is less favorable than that of colorectalcarcinoma.3

HistoryAt the age of 10 years, appendectomy was performedfor intermittent abdominal pain. At 33 years of age,laparotomy was performed for acute bowel obstruc-tion that had been preceded by some years of colickyabdominal pain, and during this period he was foundto be anemic. At operation, a fibrous stricture, at thebase of a Meckel’s diverticulum, was found to becausing the obstruction. This was resected with aside-to-side anastomosis, as the proximal bowel wasgrossly distended. In 1992, at the age of 55 years, thepatient presented with a 9-month history of colickycentral abdominal pain and distention. Hematologi-cal investigation revealed an iron deficiency anemia.Clinical examination, panendoscopy, and colono-scopy did not reveal significant pathology. A smallbowel barium series demonstrated an area of narrow-ing and dilatation in the lower ileum.

Operation (2.7.92)Laparotomy identified the side-to-side anastomosisin the terminal ileum. There were long extensionsof the closed ends of the afferent and efferent limbsof the anastomosis, with the former showing signif-icant dilatation. The ileum proximal to the anasto-mosis was dilated with a thickened wall indicatingchronic obstruction. The anastomosis, with 18cmof afferent and 25cm of efferent ileum, was resectedand an end-to-end anastomosis performed with asingle layer of interrupted polyglactin 910 (vicryl)sutures.

PathologyOpening the resected specimen revealed 2 shallowulcers involving the anastomosis. The mucosa at theedge of each ulcer was hyperemic and the basefibrotic. The mucosa in the remainder of the speci-men was normal except for the junction of the distalpart of the anastomosis with the efferent limb. Themucosa here was atrophic and fibrotic and associ-ated with a tight fibrous stricture. Histologicalexamination revealed full thickness ulceration ofthe mucosa. The base of the ulcers was florid inflammatory granulation tissue with chronicinflammation and fibrosis in the submucosa and

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deeper layers of the bowel wall. The features werenonspecific.

Follow-Up (2005)No further significant gastrointestinal problemshave been reported.

CommentBlind loop syndrome should be distinguished fromthe blind pouch (or “pocket”) syndrome in that theformer is most commonly associated with abypassed segment of bowel without resection. Itmay also occur in extensive jejunal diverticulosisand scleroderma. The blind pouch syndrome occursafter resection and side-to-side anastomosesbetween small bowel or between small and largebowel. An elongated and distended segment ofbowel develops which is usually the closed end ofthe proximal bowel but may also affect the distalclosed end if it is jejunum or ileum. Obstructivesymptoms are common even in the presence of awidely patent anastomosis,1 but anastomotic steno-sis may occur. Ulceration at the anastomosis or inthe pouch2 may occur and be responsible for micro-cytic iron deficiency anemia. These symptomstherefore contrast with the blind loop syndrome,which is characterized by diarrhea, steatorrhea,nutritional deficiencies, and a macrocytic anemia,usually in the absence of ulceration. The formationof the distended pouch is thought to be due toaltered intraluminal pressure or stagnation of con-tents in the closed end of the bowel. Walfish andFrankel (1979), at operation, observed preferentialflow of small bowel content into the pouch despitea patent anastomosis.1 It is most likely that the elon-gated pouch develops from a short closed end ofbowel and is not an excessive length of bowel leftinadvertently by the surgeon. The incidence of thiscomplication is difficult to estimate. Currentlymost reports are in the European literature. Frank et al. (1990) report 3 personal cases and review 76from the literature.3 Eighty one percent of thepatients required operation. The surgery was performed for bleeding (45%), obstruction (40%),

6 Blind Pouch Syndrome After Bowel Resection

Male, 55 Years

12

and peritonitis (15%). Since the 1980s, with theintroduction of the linear stapler, side-to-side anas-tomosis has been more widely practiced. Is there alegacy to follow? Although the blind pouch may

Diagram 6 13

develop and cause symptoms within months,4 itusually manifests after a long period, as in thepatient described in this case report. The necessarytreatment is resection with end-to-end anastomosis.

HistoryBetween 1959 and 1974, the patient had suffered 4episodes of profuse rectal bleeding requiring trans-fusion. Barium enema examination (1974) revealeddiverticulosis throughout the colon. The patientresided some distance from sophisticated surgicalservices and this influenced the decision to operate.

Operation (11.4.1974)An elective operation was performed. Many diver-ticula were present, particularly in the transverseand left colon. There was a focus of induration inthe transverse colon in relation to a diverticulum. Acolectomy and a high ileorectal anastomosis (IRA)was performed (side ileum-to-end rectum). Micro-scopic examination of the diverticulum in the trans-verse colon (Figure 7.1) showed conspicuous dilatedvessels.

Follow-UpSigmoidoscopy 2 years and 5 months later in 1977demonstrated a healthy IRA at 13cm. In 1981,profuse rectal bleeding occurred, and at this timesigmoidoscopy revealed ulceration of the ileum adjacent to the IRA. In 1984, further acute bleeding

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occurred requiring transfusion. Sigmoidoscopy nowrevealed a stricture of the IRA in addition to ulcer-ation. A barium enema demonstrated a blind elon-gated pouch in relation to the IRA (Figure 7.2).

Operation (7.20.1984*)A large length of ileum (15cm) was found projectingfrom the right side of the IRA. It measured 6cm inwidth. The IRA and 5cm of proximal ileum wereresected and a further IRA established with an end-to-end anastomosis.

PathologyThere was ulceration and stricture formation of the IRA and adjacent ileum. The mucosa at the apex of the blind loop was intensely hyperemic. Histological examination revealed nonspecificinflammation.

7 Blind Pouch Syndrome AfterIleorectal Anastomosis

Female, 68* Years

14

Figure 6.1: Diverticulum in the transverse colon causingbleeding (1974).

BP

RIL

Figure 6.2: A barium enema demonstration of the blindpouch. (1984). BP: blind pouch; IL: ileum; R: rectum.

Postoperative CourseThe patient’s gastrointestinal recovery from theoperation was slow but satisfactory. She was,however, suffering from persistent angina. Investi-gation revealed severe, inoperable coronary arterydisease and poor left ventricular function. Thepatient suffered a fatal myocardial infarct 29 daysafter the operation.

CommentThis patient is another example of the blind pouchsyndrome which, in contrast to Case 6, was a com-plication of a side-to-end anastomosis between

Diagram 7 15

ileum and large bowel. It has more frequently beenreported as a complication of ileo-colic anastomosisafter right hemicolectomy.1,2,3 Although there wasanastomotic stenosis, the symptoms were due solelyto bleeding from the ulceration, which commenced7 years after the ileorectal anastomosis. At this firstoperation (IRA), the closure of the terminal end ofthe anastomosis was adjacent to the anastomosis,proving the large blind pouch developed subse-quently. The ulceration in such a case can beresolved only by surgery. It is unfortunate that operation was not performed 3 years earlier (1981),which may have avoided the fatal consequences ofher cardiovascular disease.

7.20.84

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II Appendix

HistoryThe patient suffered recent acute pain in the rightiliac fossa. Admission to hospital was necessary, and a plain x-ray of the abdomen revealed loops ofdilated small bowel. A gastrograffin enema indicateddeformity of the ileocecal region. The patient’s con-dition settled and he was discharged from hospitaland transferred for further investigations.

ColonoscopyColonoscopy revealed a “beehive” shaped polypoidlesion at the base of the appendix (Figure 8.1). Itappeared to be inflammatory rather than neoplastic,and appendicitis was suspected.

Operation (8.7.98)At operation, recent acute appendicitis was con-firmed with retrograde intussusception induced bya tense and distended appendix. The distal cecumwith the appendix was resected.

CommentThis is a rare mode of acute appendicitis diagnosis.The smooth surface, the inflammatory changes, andthe site of the lesion suggested the diagnosis.

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8 Acute Appendicitis: Diagnosis atColonoscopy

Male, 59 Years

18

Figure 8.1: The inverted inflamed appendix seen atcolonoscopy.

Diagram 8 19

HistoryThe patient was examined by colonoscopy in viewof a family history of colorectal cancer (mother).There were no previous or current gastrointestinalsymptoms. In the base of the cecum there was asmooth hemispherical swelling covered by normalmucosa (Figure 9.1). This was diagnosed as a mucocele of the appendix by the colonoscopist. A computerized tomography (CT) examinationdemonstrated that the lesion was continuous withthe appendix, which was dilated (Figure 9.2).

Operation (7.28.97)The findings at operation confirmed the diagnosis.The proximal appendix was significantly dilated andcontinuous with a palpable intracecal swelling. Thedistal half of the appendix was pale, reduced incaliber, and firm in consistency, suggesting fibrosis.There was no evidence of malignancy or other intra-abdominal abnormality. A limited right hemi-colectomy was performed in preference to a localexcision, since an occult cystadenocarcinoma couldnot be excluded.

PathologyThe appendix was 60mm in length. Distally itscaliber was 6mm, whereas proximally it was dilated

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to 20mm. The appendix ostium was obstructed. Thelumen contained clear mucoid material. Histologi-cally there were areas of both mucosal hyperplasiaand atrophy. There was extravasation of mucin intothe wall of the appendix (Figure 9.3). This extrava-sation was devoid of cells.

9 Mucocele of the AppendixFemale, 51 Years

20

Figure 9.1: The endoscopic view of the mucocele.

Figure 9.2: The CT with contrast clearly shows the lesionin the cecum.

Figure 9.3: Pools of mucus are present within the wall ofthe appendix.

CommentRokitansky is credited with first description of thisentity in 1842.1 Woodruff and McDonald reported146 mucoceles in over 43000 appendectomy speci-mens examined at the Mayo Clinic.2 The pathologyis frequently not diagnosed until laparotomy. The

Diagram 9 21

first report of a colonoscopic diagnosis was byPonsky in 1976.3 Surgical treatment is necessary toconfirm the diagnosis and to prevent the complica-tion of pseudomyxoma peritonei which may followperforation of a mucocele if the primary pathologyis a cystadenoma or cystadenocarcinoma.

HistoryFollowing an “influenza type illness,” the patientcomplained of pain in the right sacral region. Awhite cell count of 19,000 returned to normal afterantibiotics. A computerized tomography (CT) exam-ination of the pelvis revealed a 4.0 × 6.5cm cysticmass, thick walled and partly calcified. The masswas intimately related to the right side of thesigmoid colon and contained multiple septations(Figure 10.1). A calculus was demonstrated in theright ureter. Examination under anesthetic revealeda mobile soft mass in the pelvis. Three small hyper-plastic polyps at 20cm were the only abnormalitiesseen on colonoscopy. The indirect hemagglutinationtest (IHA) for hydatid disease was negative.

Operation (11.4.96)A ruptured mucocele of the appendix was diagnosed,revealing a collection of green mucoid material inthe pelvis. Appendectomy was performed. Theureteric calculus was removed from the lower partof the right ureter by a urological colleague.

PathologyThere was focal calcification in the wall of the cysticmass. Foci of atypical mucinous epithelium (Figure10.2) and occasional papillary configuration werepresent. The appearance was consistent with amucinous cystadenoma.

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CommentMucus producing pathology of the appendix is rare,and the diagnosis is not usually made prior to oper-ation.1 In this patient a second radiological opinionprior to operation suggested the diagnosis was amucocele of the appendix, in view of the calcifica-tion in the wall of the cystic mass.2 Unfortunatelythe lesion had already ruptured at the time of laparo-tomy, thereby increasing the risk of peritoneal dis-semination. Clinical and CT examination have beennormal 8 years since operation. Mucocele of theappendix is divided into 4 distinct pathological entities: (i) nonneoplastic mucocele due to luminalobstruction; (ii) mucosal hyperplasia; (iii) mucinouscystadenoma with villous adenomatous change; and(iv) malignant mucinous cystadenoma.3 Histologi-cally there can be difficulty in distinguishingbetween categories (iii) and (iv). These lesions canproduce a refractory mucinous ascites, which hasbeen referred to as pseudomyxoma peritonei.Ronnet et al., in a review of 109 cases, have sug-gested subdividing this entity into disseminatedperitoneal adenomucinosis (DPAM) for benigndisease and peritoneal mucinous carcinomatosis(PMCA) for malignant mucinous ascites.4

10 Cystadenoma: AppendixMale, 70 Years

22

Figure 10.1: The CT examination demonstrates the pelvicmass.

Figure 10.2: Atypical stratified columnar epitheliumconsistent with the diagnosis of a cystadenoma. Mucuspresent in lumen of the appendix (left).

Diagram 10 23

HistoryFor 6 months the patient had noted an increase inthe frequency of bowel function. For this reason thepatient took part in a community bowel screeningprogram (Hemoccult II) for bowel cancer. The stools were positive for occult blood. Colonoscopyrevealed a “convoluted bowel”. In “the mid ascend-ing colon” there was a 1.5cm ulcerated lesion,which on biopsy revealed moderately different-iated adenocarcinoma. A computerized tomography (CT) scan showed a mass related to the right colon, extending posteriorly into the psoas muscle(Figure 11.1). No metastatic disease was detected in the liver. The patient was referred for surgicaltreatment.

Operation (10.9.95)Through the anterior wall of the cecum a mass waspalpable, fixed to the retroperitoneal tissues. Theappendix formed part of the mass, but only its proximal 2–3cm was recognizable. There were noenlarged mesenteric lymph nodes or any metastasesidentified. The tumor mass was “dumbbell” shaped,as illustrated on the CT (Figure 11.1), with the pos-terior extension deeply penetrating the psoasmuscle. Adequate access to this area was not possi-ble until the colon distally and the ileum proximallyhad been transected. Despite this improved access,

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the thin “isthmus” of the “dumbbell” fracturedduring dissection so that the deeper part of thetumor was excised separately from the main speci-men. The tumor bed in the psoas muscle was exten-sively treated with diathermy. An ileo-transversecolon anastomosis was completed with a circularstapler.

PathologyBelow the ileocecal valve there was a malignantulcer surrounded by a thin rim of friable tumorwhich merged with the appendix to form the tumormass. Histologically the lesion was a moderately dif-ferentiated mucinous adenocarcinoma. The tumorwas seen lining the appendiceal lumen and extend-ing through the wall. “The lumen of the appendixhas been completely blocked: the sections confirmthat it is the primary site” (Figure 11.2). The marginof tumor clearance of the deep part of the tumor(within the psoas muscle) was satisfactory. Therewere no metastases in 11 mesenteric lymph nodesexamined (Dukes B, T3 N0 M0).

Follow-Up (2004)In view of the risk of local recurrence, the patientwas treated with adjuvant chemotherapy (5Flourouracil and Folinic acid) for 6 months. The

11 Carcinoma of the AppendixFemale, 53 Years

24

Figure 11.1: The CT demonstrates the cecal mass withposterior extramural extension.

Figure 11.2: Adenocarcinoma is shown filling the lumenof the appendix.

patient remains free of recurrent or metachronousdisease 9 years after operation.

CommentAlthough the pathology was thought to be a carci-noma of the cecum at operation, the pathology evi-dence favors a malignancy developing in a retrocecalappendix and subsequently ulcerating into thececum. Appendiceal carcinoma is rare and it is notunusual for the diagnosis to be made first by thepathologist. Nitecki et al studied 94 patients treated

Diagram 11 25

at the Mayo Clinic and found that a correct intra-operative diagnosis was made in only 32% ofpatients.1 The mucinous variety of appendiceal car-cinoma occurs in approximately half the appen-diceal malignancies (if carcinoids are excluded). Itsspread is to the peritoneum (pseudomyxoma peri-tonei) rather than to lymph nodes or liver. Increas-ing depth of local invasion has an adverse effect onprognosis.1,2 Dukes B, T3 N0 M0 tumors have beenreported to have a 67%, 5-year survival.1 Righthemicolectomy is the recommended treatment.1,2

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III Polyps-Polyposis

HistoryA barium enema was performed in this patient toinvestigate the recent onset of constipation, abdom-inal distention, and weight loss. The x-ray demon-strated a large irregularity of the sigmoid colon andno other colonic pathology (Figure 12.1). On refer-ral, examination of the abdomen revealed a largeasymptomatic distention of the urinary bladder wallabove the umbilicus. Flexible sigmoidoscopyrevealed a large soft polypoid tumor at 22cm. Uro-logical investigations indicated the need for pros-tatectomy, which was performed prior to thecolorectal surgery.

Operation (3.2.98)At laparotomy the only abnormality was the largesoft polyp in the mid sigmoid colon. There was no

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obvious enlargement of mesenteric lymph nodes inrelation to the sigmoid colon. The appendix wasmarkedly kinked by congenital adhesions. Thelower two thirds of the sigmoid colon was resectedwith a sutured end-to-end anastomosis. Appendec-tomy was performed.

PathologyThe polyp extended over 80mm of the sigmoidcolon and “carpeted” the entire circumference of thelumen, forming a “tube” of polyp tissue. Most of thelesion was flat, the proximal edge of which mergedwith normal colon mucosa. There were raised poly-poid areas within the polyp, but, on careful palpa-tion, no firmness in consistency was detected. Thehistological examination of the polyp showed atubulovillous adenoma with areas of severe dyspla-sia but no evidence of invasive malignancy. Fivelymph nodes were examined, 1 of which containeda deposit of metastatic adenocarcinoma, (Figure12.2) Dukes C, pTNM stage 31 (assuming the nodalmetastasis is related to the sigmoid tumor).

12 A Mega Polyp Associated with aMicro Cancer

Male, 61 Years

28

Figure 12.1: The barium enema shows the moreproliferative bulk of the sigmoid polyp.

Figure 12.2: Deposit of adenocarcinoma in onemesenteric lymph node.

Follow-Up (2004)In the past 6 years 2 small benign polyps have beenremoved by colonoscopy. There is no evidence ofrecurrence of the colon cancer, but the patient devel-oped non-Hodgkin’s lymphoma in 2003, whichappears to have responded to chemotherapy.

CommentThis tumor is an example of “large polyp occult carcinoma” that showed no evidence of the primaryfocus of malignancy on operative, macroscopic, or

Diagram 12 29

microscopic examination. The malignancy was notidentified in the polyp despite the reexamination of a further 27 blocks of tissue. The incidence ofinvasive carcinoma in a polyp is known to bedirectly related to size,2 and, therefore, giant polypssuch as this case can be associated with a high incidence of carcinoma. Sakamoto et al reportedrecurrent malignancy after excision of a “benign”villous adenoma.3 Where possible, these lesions arebest treated by bowel resection as this and Cases 13and 14 demonstrate.

HistoryThe patient complained of diarrhea for 10 years. Sig-moidoscopy revealed an extensive flat polyp withpolypoid areas which extended from the anal canalto the lower sigmoid colon, reaching a level of 20cm. There were no obvious features of malig-nancy. A barium enema showed diverticulosis proximal to the lesion.

Operation (2.4.77)Laparotomy and loop transverse colostomy.

Operation (2.18.77)Via a posterior transsphincteric approach, submu-cosal dissection removed the tumor from the rectalmuscle. The rectum and sphincter were repaired.

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PathologyExamination of the specimen (23.0 × 18.0cm)revealed a villous adenoma with no evidence ofmalignancy.

Follow-UpThe colostomy was closed 3 months after the excision of the polyp. A rectal stricture resolvedspontaneously (Figure 13.1). Bowel function was satisfactory. No abnormality was detected onregular follow up until March 1986. A presacralcystic swelling, 11.0 × 7.0cm, was apparent on com-puterized tomography (CT) examination of thepelvis. Transrectal exploration of the cyst produceda copious discharge of mucus, which on histologicalexamination contained clusters of mucoid adeno-carcinoma cells. Palliative chemotherapy or radio-therapy was decided against. The patient’s healthsteadily deteriorated until he died (1.8.87), 10 yearsafter removal of the “benign” polyp.

CommentThe extensive lesion would be best classified as agiant polyp. The surgical options for such a polypshould be considered differently to those for smaller

13 Extensive “Benign” Polyp of theRectum and Sigmoid Colon

Male, 69 Years

30

Figure 13.1: Complete resolution of postoperativestricture.

Figure 13.2: Free polyp fragments in washings from theoperative field of another patient, also treated withposterior transsphincteric surgery.

flat polyps occurring in the rectum. The treatmentshould be based on the probability of invasive car-cinoma rather than the technical feasibility of localexcision. The focus of invasive carcinoma in suchpolyps may remain undetected despite multiple sec-tions. Galandiuk et al, in reviewing 1049 patientswith villous and tubulo-villous polyps, clearlydemonstrated an increasing incidence of invasivecarcinoma with larger lesions.1 Polyps larger than 4cm had a 32% incidence of invasive carcinoma.1

One can only speculate the risk of carcinoma inpolyps 10–20cm in size. A number of technicallyclever transanal operations are available for local

Diagram 13 31

excision.2,3 The posterior transsphincteric operation,first performed by Harrison Cripps in 1880,4 wasrevived by York Mason in the 1970s, but a review ofthis operation has highlighted the unacceptablecomplication rate.5 The mode of the local recurrencemay have been cell implantation, since the presacralspace was exposed throughout the operation. Figure13.2 shows the tissue debris in the wound irrigationfluid from another patient undergoing the sameoperation for a giant “benign” polyp. This patientalso succumbed to recurrent cancer in the presacralspace. Both patients were fit enough for resectionwith coloanal anastomosis.

2.18.77

HistoryThe patient presented with a 3-year history of rectalbleeding and “explosive” diarrhea. Flexible sigmoi-doscopy revealed a large polyp at 22cm and 4 smallpolyps at lower levels. Colonoscopy identified 4 small polyps between the large lesion and thesplenic flexure.

Operation (3.19.90)With an operating sigmoidoscope and snare, thepolyp was removed “piecemeal” until a “clean”mucosal defect was obtained with no visible resid-ual polyp.

PathologyThe polyp fragments were soft, and placed togetherthey measured 70 × 70mm. Histological examina-tion showed a villous adenoma with no evidence ofmalignancy.

Follow UpThe smaller polyps were removed at subsequentcolonoscopy (12.12.91) when the diathermy scar ofthe large polyp site was identified. The patientdelayed his next colonoscopy for almost 3 years. Atthis time he was asymptomatic. Colonoscopyrevealed an ulcer adjacent to the scar at 22cmwhich, on biopsy, showed adenocarcinoma.

Operation (9.16.94)At laparotomy a large mass of lymph nodes of thesigmoid mesentery was identified adjacent to thelower third of the sigmoid colon. There was a 15mmnode at the origin of the inferior mesenteric artery.

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Four small metastases (confirmed on biopsy) werepresent in the liver. A high anterior resection withanastomosis was performed.

PathologyExamination of the lumen of the colon revealed amalignant ulcer 23mm in diameter at the level ofthe lymph node mass and continuous with it. It wasadjacent to but not continuous with the diathermyscar. The extracolic mass had formed a “domeshaped” deformity beneath the malignant ulcer.Histological examination confirmed the diagnosis ofadenocarcinoma (moderately differentiated) withextensive lymph node involvement.

Follow-UpThe patient was treated with 5 flourouracil andfolinic acid but failed to show any response. He diedof metastatic disease 18 months after the palliativeresection.

CommentThe morphology of the resected lesion was consis-tent with the lymph node mass having “erupted”into the lumen of the colon to produce a malignantulcer. If so, the apparently benign polyp must havecontained an undiagnosed focus of carcinoma. Ametachronous primary carcinoma at the same siteseems less likely but cannot be excluded. Althoughthe challenging diathermy snare of a large polyp at 22cm was a technical success, in hindsight this patient would have been better managed byresection.

14 A Bad Result from a SuccessfulOperation for a Polyp in the Sigmoid Colon

Male, 54 Years

32

Diagram 14 33

3.19.90

9.16.94

HistoryThe patient complained of diarrhea and mucous dis-charge for 13 years. There had been intermittentrectal bleeding. Prolapse of the rectum had been notedfor a period of 12 months. There had been recentweight loss and general weakness. On digital rectalexamination, a soft polyp encircling the rectum waseasily palpable at the 7cm level. Complete rectal pro-lapse was present on straining. There was laxity of theanal sphincter. Sigmoidoscopy revealed an extensiverectal polyp with a combination of flat and polypoidcontours. The upper limit of the polyp was 20cmfrom the anal verge. There were no macroscopic features to suggest malignancy. There were abnor-malities of biochemistry on blood examination: Na,125mmol/L (N-134–143); K, 3.8mmol/L (N-3.5–5.0);Urea, 28.9mmol/L (N-2.0–7.0); and creatinine, 0.21mmol/L (N-0.05–0.11).

Operation (1.16.87)A Delorme operation was performed in view of thepatient’s poor general health. The excised mucosameasured 15 × 13cm.

PathologyThe histological examination showed both villousand tubulovillous adenoma with several submu-cosal “lakes” of mucin which were interpreted asearly proctitis cystica profunda.

Follow-UpThe postoperative assessment was satisfactory 2months after operation. The patient did not returnfor further examination.

CommentIn this frail patient the surgical approach was satis-factory treatment for the coexisting giant rectalpolyp and the rectal prolapse. The submucosal dis-section of the polyp by the Delorme techniqueallowed very precise separation of the polyp fromthe muscular layer of the rectum. This was greatly

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facilitated by infiltrating the submucosal plane withthe vasoconstrictor (ornipressin). The alternativeoperation in this patient was a low anterior resec-tion, decided against, as the patient was a poor riskfor abdominal surgery. In this group of patients theDelorme operation for rectal prolapse is well toler-ated, but the results are inferior to abdominal surgi-cal repair. Oliver et al found a recurrence rate of 22%in 41 operations in 40 poor risk patients and attrib-uted the failures to weak or absent sphincter tone.1

Tobin et al regarded the operation as most suitablefor the medically unfit patient.2 In 43 of 49 poor riskpatients who were reviewed, the recurrence rate was26%. There was an improvement in anal continencein 50% of the 40 patients documented with preop-erative sphincter disability.2

15 One Operation for Double PathologyFemale, 78 Years

34

Figure 15.1: Edmond Delorme (1847–1929). (Courtesyof Prof. M. Corman.)

Diagram 15 35

HistoryThe male patient aged 13 years had noticed rectalbleeding for 15 months. A prolapsed rectal polyp hadbeen observed. A barium enema showed no evidenceof other polyps.

Operation (initial procedure) (3.24.78)Examination of the rectum under anasthesiarevealed multiple polyps in the lower rectum. Sig-moidoscopy noted the upper level of involvementwas 9cm. The polyps were deeply congested,smooth with a glistening surface. They were fragileand fractured readily. The pedicles were vascularand varied in size. There were approximately 20polyps of moderate size (5–10mm) removed bydiathermy snare. There were many small polyps(1–8mm), which were fulgurated.

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PathologyHistological examination showed typical appear-ance of juvenile polyp (Figure 16.1).

Follow-UpTwenty months after the initial removal of rectalpolyps, 40 polyps (5–20mm) were removed bydiathermy snare and fulguration. Twelve monthslater a further 83 polyps were removed, and at thistime rectal prolapse was diagnosed while the patientwas anesthetized. Over the next 20 years furtherremoval of polyps was undertaken on an additional14 occasions. The polyps remained typical in appear-ance and consistency and, subsequent to 1993, werenever larger than 6mm. Colonoscopy examinationrevealed no polyps proximal to those described. Arectopexy (Ripstein) was performed for the rectalprolapse in 1995 at the age of 30 years. In excess of 400 polyps had been removed by July 2001.

CommentThe patient’s juvenile polyposis (JP) was confined tothe lower rectum over a period of 33 years. The his-tology of the polyps remained typical of juvenilepolyps. The incidental occurrence of rectal prolapsewith juvenile polyposis is unusual. McColl et alfrom St Mark’s Hospital, London reported that 11 of131 patients (8.4%) with polyposis of the colon hadJP, the rectum being the commonest site of thepolyps in the large bowel.1 Smilow et al were thefirst to report the familial nature of the disease,2

which can also be sporadic. The potential for upper or lower gastrointestinal malignancy has beenreported since 1975 and may be a cumulative riskas high as 50% in familial JP.3 Because of the poten-tial distribution of the polyps, screening of patientsshould include the gastrointestinal tract (GIT) fromstomach to rectum. Family members can now bescreened for mutations in the SMAD4 gene.3

16 Juvenile Polyposis and Rectal ProlapseMale, 13 Years

36

Figure 16.1: Within the vascular stroma there are dilatedglands lined by normal epithelium and containing mucus.

Diagram 16 37

11.28.80

HistoryIn 1981, approximately 40 polyps were identified inthe colon and rectum by colonoscopy. At least 30 ofthe polyps were situated in the right colon. Initially6 excised polyps were thought to be the Peutz Jeghervariety, but subsequently the diagnosis of juvenilepolyposis (JP) was established.

Operation (3.8.81)At laparotomy soft polyps were palpable in the rightand transverse colon, the largest of which was situ-ated in the cecum. A right hemicolectomy was per-formed with the distal level of resection in the midtransverse colon. An end-to-end anastomosis wascompleted with a single layer of interrupted sutures.

PathologyThe resected colon contained 30 polyps that werecharacterized by long pedicles of up to 30mm inlength and frequently branched, giving a “tree-like”appearance. The polyps were soft, the largest beinga lobulated mass 48mm in diameter: histologicalsections of all polyps showed the typical features ofJP. There was no evidence of malignancy.

Follow-UpSince operation a further 29 polyps have beenremoved during surveillance colonoscopies; most ofthese have been JP and a few have been adenomas.A family history emerged in 1986 when the patient’sdaughter, aged 9 years, was found to have JP of the

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colon treated by colonoscopic excision and righthemicolectomy.

CommentThe multiple polyps in juvenile polyposis were firstdescribed by Verse,1 but it was not until the 1980sthat the increased risk of colorectal carcinoma inthese patients was well established.2 Jass et al3 havereported 18 patients with colorectal carcinomasoccurring in 87 patients with JP, and they suggestedthe cumulative risk could be as high as 50%. Thecolon polyposis may be associated with polyps inthe stomach and small bowel as well as extracolonicabnormalities.3 The polyps may be typical (81%)with dilated cystic glands and inflammatory infiltrate in the lamina propria, atypical (16%) oradenomatous (2%).4 The disease may be sporadic orfamilial, and the latter is inherited as an autosomaldominant. An abnormality predisposing one to JPhas been identified on chromosome 18q21.1.5 Treat-ment is varied according to the number and distribution of the polyps and therefore may beendoscopic, resection, or a combination of thesemethods. When polyps are too numerous or difficultto control endoscopically, colectomy and ileorectalanastomosis (IRA) or restorative proctocolectomyare the operations of choice.6 Long term follow upby endoscopy is necessary in these patients subse-quent to initial treatment. It is also recommendedthat first degree family members undergo screeningfor colorectal carcinoma in addition to screening forpolyps.

17 Juvenile Polyposis in an AdultFemale, 31 Years

38

Diagram 17 39

Mother3.8.81

Daugher12.6.93

HistoryThe patient presented in May 1965 with an estab-lished family history (mother and sister) of thePeutz–Jeghers syndrome (PJS). He had suffered epi-gastric lower abdominal colic for 2 years, then dailyfor 2 months. The pain was accompanied by loudborborygmi, and black stools appeared after themore severe attacks. The only abnormality on phys-ical examination was the presence of pigmentedspots, typical of PJS, on the eyelids, lips, buccal sur-faces, and fingers (Figure 18.3). A barium mealfollow-through demonstrated a round filling defectin the first part of duodenum, the “size of a marble;”the small bowel appeared normal.

Operation (7.8.65)At laparotomy chronic colocolic intussusception ofthe transverse colon was found, fixed by adhesions.The intussusception had reached the lower descend-ing colon but was easily reduced after division ofadhesions. A large mass of confluent polyps in themid transverse colon was exposed by colotomy andremoved by ligature transection of a broad pedicle.The colon between this site and the rectum wasexamined with a rigid sigmoidoscope to exclude thepresence of other polyps. Feces in the proximalcolon precluded intraoperative enteroscopy, but apolyp detected in the cecum by palpation wasremoved via a colotomy. A small, branched polyp inthe terminal ileum and a spherical polyp in the duo-denum, adjacent to the pylorus, were removed viaenterotomies. A normal appendix was removed.

PathologyThe polyps were soft in consistency with prominentlobulation. Both the polyp mass in the transversecolon (45mm) and the ileal polyp (14mm) exhibiteda characteristic branched morphology. Histologi-cally the polyps were typical of PJS (Figure 18.4).

Follow-UpDuring the subsequent 23 years the patient requiredlaparotomy on 3 occasions: small bowel obstructionrequiring division of adhesions (1966), resection of

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20cm of ileum containing a benign 34mm polyp(1978), and resection of 50cm of ileum for a gan-grenous intussusception caused by a benign 30mmpolyp (1988). In 1990 he was diagnosed with lungcancer and died later that year aged 49 years. (It isnot known if the lung cancer was primary ormetastatic.)

CommentThis rare autosomal dominant disease has been esti-mated to occur in 1 :120000 births.1 Hamartoma-

18 Chronic Intussusception of the ColonDue to Peutz-Jeghers Syndrome

Male, 24 Years

40

Figure 18.1: J.L.A. Peutz (1886–1957).

Figure 18.2: H.J. Jeghers (1904–1990).

tous and adenomatous polyps may occur fromstomach to rectum. The predisposition to malig-nancy in the gastrointestinal tract (GIT) and extra-GIT sites was debated for some years, but evidencefor this now exists.2 Treatment should avoid bowelresection where possible, as surgery for furtherpolyps is often necessary. Asymptomatic polyps arebest removed before the complications of bleedingand intussusception occur. Spigelman et al recom-mend the removal of such polyps over 1.5cm in sizeby endoscopy or operation.2 St Mark’s Hospital andthe Cleveland Clinic recommend that during alaparotomy for PJS, all polyps, irrespective of size,should be removed (endoscopically or by entero-tomy) to effect a “clean sweep”3 and thus reduce the

Diagram 18 41

need for subsequent surgery.2,3 Follow-up for life isimportant for these patients in order to detectmetachronous polyps and cancers. It is likely thatsmall bowel surveillance will be best achieved bythe use of video capsule endoscopy (VCE) as itbecomes more widely available. Two recent studieshave shown VCE to be superior to radiographicinvestigation with impact on clinical manage-ment.4,5 The 2 family members of the patient whowere affected by PJS died of the disease. His sister,aged 8 years, died of small bowel intussusception,and the mother, aged 54, years succumbed to pan-creatic cancer. Primary lung cancer complicatingPJS is rare, and to 2005 only 7 cases have been published.6

7.8.65

Figure 18.3: Buccal pigmentation.

Figure 18.4: Polyp showing muscle strands betweenglands, characteristic of PJS.

For a full-page image of this figure see the appendix.

HistoryThe patient, who suffered from morbid obesity, anddenying any bowel symptoms presented with a 7-week history of a fecal vaginal discharge. There wasno family history of bowel cancer. Examinationrevealed a large fixed carcinoma of the rectum witha lower edge at 5cm. There was a small malignantulcer on the posterior wall of the vagina, discharg-ing flatus and fecal liquid. The narrow lumen of thetumor prevented endoscopic examination beyondthe lower edge. A barium enema was contraindi-cated due to the possibility of initiating bowelobstruction. Biopsy showed a well differentiatedadenocarcinoma. Computerized tomography (CT)examination showed perirectal extension of thetumor with adherence to the uterus and vagina.There was no pelvic lymphadenopathy or evidenceof extra pelvic metastases. The patient was treatedwith a 5-week course of radiotherapy (40Gy), andafter 4 weeks CT examination showed “an overallreduction in size” of the tumor.

Operation (12.9.85)Laparotomy revealed the surprise finding of multi-ple, palpable polyps throughout the colon. Therectal tumor was adherent to the uterus with exten-sion to the deep aspects of the pelvis on the rightside. Proctocolectomy with en bloc radical hys-terectomy and excision of the posterior wall of thevagina was performed.

PathologyThe mucosa of the colon and rectum contained hun-dreds of sessile and pedunculated polyps, the largestmeasuring 40mm in diameter. Polyps examined his-tologically were tubulovillous. The rectal carcinomawas annular, deeply excavated, 48mm in axialdimension, and showing malignant infiltration ofthe uterus as well as vagina. Histological examina-tion of the right aspect of the tumor confirmed

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that despite the concern at operation, the margin of excision was satisfactory. The tumor was a muci-nous adenocarcinoma. There were “nodules” in theperirectal fat containing mucus and epithelial cells,regarded as metastatic nodes, attenuated by radio-therapy (Dukes C, T3 N2 M0).

Follow-UpIn 1992, panendoscopy revealed a 20mm adenoma-tous polyp in the second part of the duodenum. In1994 cholecystectomy and transduodenal polypec-tomy was performed by open abdominal surgery. No abdominal metastases were identified. In 1998 acerebral tumor was diagnosed but considered to beinoperable. Biopsy was not performed. There was noresponse to chemotherapy or radiotherapy. Thepatient died in August 2000.

CommentThe patient and 1 of 2 children were confirmed assuffering from familial adenomatous polyposis (FAP)with positive gene testing. They appear to be thefirst FAP patients in the family line. Rectovaginalfistula is uncommon in patients with rectal cancer.It is difficult to believe this extensive tumor was notaccompanied by bowel symptoms. Restorative proc-tocolectomy was not regarded as a safe option in thispatient. The recommended operation for a low rectalcarcinoma and rectovaginal fistula is the one per-formed on this patient as illustrated by Keighley andWilliams.1 The patient’s survival for nearly 15 yearswas not expected. The cerebral tumor may havebeen coincidental, a metastasis, or another extracolonic manifestation of FAP syndrome. Kropilak et al have reported 13 patients with brain tumorsand a family history of FAP. Six of the patients haddocumented FAP. Although the brain tumorsappeared at an early age (mean 15 years) the agerange was 11/2 to 59 years.2

19 Carcinoma of the Rectum: FAP and Rectovaginal Fistula

Female, 46 Years

42

Diagram 19 43

Posterior aspect

HistoryIn July 1970 the patient underwent colectomy and ileorectal anastomosis for the treatment of familialadenomatous polyposis (FAP). At follow up, villousadenomata had been removed by diathermy snare on10 occasions. The surgeon supervising the follow upreferred her in 1994 for a restorative proctocolectomy,since he was concerned about future malignantchange in the rectum. Flexible sigmoidoscopy identi-fied the ileorectal anastomosis (IRA) at 20cm andmultiple flat polyps in the rectum. No obvious carci-noma was identified on endoscopy or digital exami-nation. Operation was advised to anticipate the onsetof carcinoma.

Operation (3.27.95)Laparotomy revealed adhesions involving the small bowel, particularly in the region of the side ileum:end rectum anastomosis. Projecting tothe left of the anastomosis was a blind pouch 10cmin length. There was no evidence of metastaticdisease within the abdomen. The ileum was dis-connected from the anastomosis and the rectumexcised to the level of the pelvic floor. The opera-tion was performed with coagulating diathermy dissection, laterally to the endopelvic pelvic fascia,anteriorly posterior to the Waldeyer’s fascia and posteriorly via a thin fascial presacral anatomicalplane. A J-pouch and a proximal loop ileostomy wasconstructed.

PathologyExamination of the rectum revealed multiple sessilepolyps and an extensive soft sessile polyp up to 12cm in length. This polyp was predominantly flatwith polypoid areas. Its shape was patchy withislands of normal mucosa within it, suggesting thatcoalescing of multiple polyps had occurred. Situatedin its center was an ulcerated tumor 25 × 25mm. Inthe upper part of the rectum there was a smallerulcerated lesion (8 × 8mm). Histological examina-tion of the polyps showed a tubulovillous pattern.The larger ulcer was a moderately differentiated ade-nocarcinoma associated with villous adenoma. Thistumor extended through the bowel wall to perirec-tal fat (T3). The smaller ulcer was also a moderately

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differentiated adenocarcinoma with invasionlimited to the submucosa (T1). Seventeen perirectallymph nodes were examined, 2 of which containedmetastatic carcinoma. The tumors were probablyDukes C, T3 N1 M0 and Dukes A, T1 N0 M0.

Operation (5.22.95)Closure of loop ileostomy.

Follow-Up (2004)Postoperative radiotherapy was not administered, toavoid the risk of post irradiation pouch dysfunction.The patient was treated with adjuvant chemother-apy (flourouracil and folinic acid) for 12 months. Sheremained well until May 1998 when computerized(CT) examination demonstrated a large solitary massin the right lobe of the liver. A right hemihepatec-tomy was performed. Progress was satisfactory until May 2004 when pulmonary metastases weredetected. At last follow-up the patient’s well beingdid not appear to be affected by this latest spread ofthe disease. There has been no evidence of localpelvic recurrence.

CommentIn FAP patients the risk of rectal cancer is known toincrease with length of follow up after IRA. Thispatient’s carcinomas were diagnosed 25 years afterthe IRA was performed. In patients treated entirelyor predominantly before the ileal pouch surgery com-menced, the risk of rectal cancer has been reportedas: 3.6% (St Mark’s Hospital), 1 12.9% (ClevelandClinic),2 13.1% (Leeds Castle Polyposis Group),3 and32% (Mayo Clinic),4 Church et al have highlighted achange in indication for IRA since the advent ofpouch surgery and suggest that IRA is a satisfactoryoperation if the rectum is not severely affected by polyposis.2 Church et al report that since 1983,135 patients treated at the Cleveland Clinic with IRA with a median follow up of 135 months haveremained free of rectal cancer.2 The patient reportedhere had a longer rectal segment remaining than therecommended 12cm level of anastomosis. A lowerIRA would not have prevented the development ofthe cancer in the mid rectum. It is of interest that thiscarcinoma was not diagnosed prior to operation.

20 Ileorectal Anastomosis for FAP:Rectal Cancer

Female 35, Years

44

Diagram 20 45

HistoryIn 1990 colonoscopy revealed multiple lipomas inthe left colon and rectum. There was also a centrallower abdominal mass reaching the level of theumbilicus. Laparotomy revealed a deep abdominalwall tumor (12 × 9cm) attached to the sigmoidcolon, bladder, and a tube-ovarian mass containinga cystadenoma (14 × 14cm). These lesions wereremoved. An ulcerated polypoid lipoma (6 × 2.5cm)was removed from the splenic flexure by colotomy.The histology of the abdominal wall tumor revealed a fibroblastic pattern (no mitoses) and chronicinflammatory cells. Diagnosis: inflammatorypseudotumor

Follow-Up Investigations (10.8.98)In 1998 colonoscopy and barium enema revealedlipomatous polyps in the rectum and colon as far asthe mid descending colon (Figures 21.1 and 21.2).They varied in size from 3mm to 65mm.

Operation (1.18.99)The colon was obscured by extensive “fat wrap-ping” and large appendices epiploicae. Resection-anastomosis was performed. The small lesions werelipomas, tubular adenomas, and hyperplastic polyps.The larger polyps were lipomas.

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Follow-Up (2005)Colonoscopy has subsequently revealed smalllipomas in the transverse and ascending colon. Someof the small rectal polyps removed are “stromalpolyps” showing increased smooth muscle fibers inthe submucosa.

CommentThe true nature of the “inflammatory pseudotu-mor” removed in 1990 remains unclear and may besimilar to the case reported by Kunakemakon et al.1

Its relationship to the lipomatosis of the colon is notestablished. While lipomas are the second com-monest benign “tumor” of the colon, lipomatosis israre. The age range at presentation in the few reportsavailable is 2–58 years. The condition may beasymptomatic or be the cause of abdominal pain,diarrhea, or rectal bleeding. Surgical treatment isnot obligatory and will depend on the extent of thecolon disease and the clinical significance. It doesnot appear to be a familial disease. The lipomas areusually submucosal but may be subserosal as well,forming “dumb-bell” lesions.2 Marked fatty infiltra-tion of the appendices epiploicae may be associatedwith the condition.3,4,5

21 Large Bowel LipomatosisFemale, 53 Years

46

Figure 21.1: Lipomas seen on colonoscopy. Figure 21.2: Lipoma in sigmoid colon.

Diagram 21 47

10.8.98

18.1.99

HistoryThe patient had a family history of colorectal cancer(father). He presented with a 10-day history ofcentral abdominal pain, anorexia, and fever. A rapidloss of weight had occurred during this period. Hewas admitted to a hospital where colonoscopyrevealed a polypoid mass at 30cm. This was diag-nosed as a carcinoma and the patient was referredfor operation. A further colonoscopy to the cecumwas performed. The lesion was of an intense redcolor, lobulated, and with a smooth surface (Figure22.1). Immediately proximal to it there was a lessprominent but similar change in the mucosa. Diver-ticula were present in the sigmoid and descendingcolon. There was no other mucosal abnormality.Biopsy revealed mucosal inflammation. On rectalexamination, there was a fixed left-sided pelvicmass. A preoperative diagnosis of diverticulitis wasmade.

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Operation (7.7.97)Laparotomy revealed a pelvic abscess contained byan inflamed segment of sigmoid colon, the left sideof the upper rectum, and the side wall of the pelvis.There was marked diverticulosis proximal to theinflammatory mass, which affected most of thedescending colon. The upper rectum, sigmoid, anddescending colon were resected. An extraperitonealanastomosis was performed with a circular stapler(later measured at 9cm). Irrigation suction drainswere placed in the “bed” of the previous abscess.Postoperative recovery was satisfactory.

PathologyThe diagnosis of diverticulitis was confirmed andthere was no evidence of malignancy. The colon wasgrossly thickened by inflammation. The polypoidmucosal lesions were due to marked edema. Anintramural abscess was anticipated to be the causeof the unusual mucosal mass but no such pathologywas found.

CommentWhile the differential diagnosis of sigmoid diver-ticulitis and carcinoma can be very difficult on the basis of clinical and radiological findings, ifcolonoscopy can negotiate the lesion, the diagnosticproblem is usually resolved. The prominent poly-poidal mass was an unusual manifestation of diver-ticulitis (the author has seen only one previoussimilar case). The smooth shiny surface of the lesionwas the principal endoscopic finding that distin-guished it from carcinoma. Schnyder et al report asimilar but much larger polypoidal mass caused bymucosal edema associated with divcerticulitis.1 Thedistal level of resection was extraperitoneal toobtain a healthy rectal wall for anastomosis. Theproximal level of resection was determined by theextent of the diverticulosis in a relatively youngpatient.

22 A Polypoid Lesion in the Sigmoid Colon

Male, 46 Years

48

Figure 22.1: Colonoscopy reveals a lobulated polypoidlesion at 30 cm, the surface of which is intensely red,smooth, and shiny.

Diagram 22 49

P A R T

IV Cancer of the Colon and Rectum

HistoryThe patient was found to be anemic (hemoglobin 8.0g/L) when investigated for an episode of syncope.Fecal occult blood test was positive. Colonoscopyidentified an annular carcinoma in the ascendingcolon and a large pedunculated polyp at the 15cmlevel in the rectum.

Operation (8.7.98)The carcinoma in the mid ascending colon was con-firmed with enlargement of adjacent lymph nodes.There was also a large hard lymph node related tothe superior mesenteric vessels, which was thoughtto be related to the colon pathology although itsanatomical position seemed unusual. There was noevidence of metastases beyond the suspicious lymphnodes in the mesentery. The right colon with 70cmof terminal ileum was resected. An end-to-end anas-tomosis was performed with a circular stapler.

PathologyThe colon cancer was deeply ulcerating andinvolved the pericolic tissues. Histological exami-nation revealed a poorly differentiated signet ringcarcinoma (Figure 23.1). The largest adjacent“lymph node” was a metastatic nodule composed of

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signet ring carcinoma with no identifiable lymphnode tissue (Dukes C, T3 N1 M0). On the luminalaspect of the ileum, there were 3 yellow submucosalnodules. Histologically they were carcinoids. Thelargest of these measured 11mm in diameter andextended through the muscularis propria. The large“ectopic” node and 2 smaller nodes related to thesuperior mesenteric vessels were yellow on the cutsurface and contained metastatic carcinoid tumor.

Operation (10.8.98)The snare removal of the rectal polyp (20mm) and2 smaller polyps nearby was performed subsequentto the resection to minimize the risk of implanta-tion from the proximal carcinoma. Histologicalexamination of the largest polyp showed severe dys-plasia in a tubular adenoma.

Follow-Up (2004)No adjuvant chemotherapy was administered inview of the patient’s age. In 2003 a left hemicolec-tomy was performed for a metachronous colon car-cinoma in the sigmoid colon (Dukes A, T2 N0 M0).The patient has no evidence of recurrent malig-

23 Synchronous Colon Carcinoma andMalignant Carcinoid

Female, 80 Years

52

Figure 23.1: Poorly differentiated adenocarcinoma inascending colon.

Figure 23.2: Typical carcinoid in the ileum.

nancy 5 years and 8 months since the first bowelresection.

CommentIt is not uncommon for carcinoids of the ileum tobe multiple.1 Carcinoid tumor may be associatedwith other malignancies of the gastrointestinal tract(29%–53%).2 The combination of colorectal carci-noma and multiple carcinoids of the small bowel israre.3 The largest lymph node in the small bowelmesentery in this patient was misinterpreted as acolorectal cancer (CRC) metastasis and, to includeit in the resection, 70cm of ileum was removed. Thecarcinoid tumors were thus resected fortuitously.

Diagram 23 53

The small bowel was not assessed adequately atoperation since the carcinoids should have beendetected by palpation. Three mesenteric lymphnodes contained carcinoid metastases and are pre-sumed to relate to the largest primary in the ileum,11mm in diameter. Thompson et al report an 18%incidence of lymph node metastasis in carcinoids ofthe ileum less than 10mm in size.1 This patient’slong term survival from a poorly differentiatedsignet ring carcinoma was not expected. There wassome reluctance to subject her to routine colono-scopic surveillance in view of her age. Neverthelessthe patient’s metachronous cancer was diagnosed atthe Dukes A, T2 N0 M0 stage.

HistoryThe patient’s complaint was an aggravation of pro-lapsed “hemorrhoids” for 2 months. She had notedoccasional constipation but no other gastrointesti-nal symptoms. Physical examination revealed cir-cumferential mucosal prolapse and a large mass,readily palpable in the pelvis. Sigmoidoscopy iden-tified the proliferative edge of a rectal carcinoma at16cm, confirmed by biopsy. Further examination ofthe bowel proximal to the tumor was not possible.Computerized tomography (CT) examination iden-tified an abnormality at the rectosigmoid area withextensive thickening of the bowel wall and diver-ticular disease. There was dilatation of the rightureter. The liver showed no evidence of metastases.

Operation (10.22.93)At laparotomy there was a large fixed mass in thepelvis formed by the upper third of the rectum anddistal sigmoid colon, the latter showing evidence ofchronic inflammation. The mass was hard, appearedto infiltrate the posterior surface of the uterus, andinvolve the right ureter which was dilated. Therewas no evidence of extra pelvic metastases. Proxi-mal to the mass, there was obvious diverticulosisthat reached the lower descending colon. It wasthought the cancer was solely responsible for thepelvic mass, and therefore the resection included aradical hysterectomy en bloc. It was thoughtprudent not to resect the ureter in this 78-year-oldfrail patient, and the ureter was “peeled” away fromthe mass. A low anterior resection was performedwith a proximal loop ileostomy.

PathologyThe annular carcinoma was ulcerating with a pro-liferative edge, causing significant constriction ofthe lumen and invasion of the perirectal tissues. Theuterus was adherent to the mid sigmoid colon bybenign adhesions that were related to a diverticularabscess confined to the pericolic fat. There wereother foci of inflammation in diverticula in this partof the sigmoid colon. There was narrowing of thelumen with redundant mucosa typical of chronicdiverticular disease. The excision margin of the

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resected cancer was negative for malignancy andexamined lymph nodes did not contain any meta-stases (Dukes B, T3 N0 M0).

Postoperative CourseInitially the patient’s recovery was satisfactory untilthe second week, when an unexplained episode oftachycardia and peripheral cyanosis occurred. Con-trast radiological assessment of the anastomosisshowed no evidence of impaired healing. CT exam-ination of the abdomen demonstrated thrombosis ofthe splenic vein and a major infarct of the spleen,which was managed conservatively. Routine pro-phylactic heparin therapy had been administeredduring the postoperative period. A follow up CTexamination 1 week later revealed infarcts of theliver and kidney, and within a few days the patientsuffered a stroke with a right hemiparesis. CT of thebrain confirmed an infarct in the postero-inferiorpart of the left occipital lobe. Despite increasingreturn of motor function, the patient remainedaphasic, depressed, and refused all attempts at oralfeeding. At the request of the family, total parentralnutrition (TPN) was eventually discontinued. Thepatient died 11 weeks after operation.

CommentThe patient suffered from synchronous majorpathologies of the large bowel and yet was virtuallywithout related symptoms. “Silent cancer” is wellrecognized as a clinical entity, but not so with diver-ticulitis of this severity. The diverticulitis was notdiagnosed until the resected specimen was exam-ined. At operation, difficult decisions were requiredin relation to the attachment of the pelvic mass tothe right ureter and uterus. If the benign nature ofthe attachment to the uterus had been appreciated,then hysterectomy would have been avoided. Thisis the only case of postoperative multiple visceralinfarcts encountered by the author. Its etiologyremained obscure. In a series of 208 elective resec-tions for diverticular disease, the incidence of coex-istent carcinoma of the sigmoid colon or upperrectum was 2 (1.0%).1

24 Coexistent Cancer and DiverticulitisFemale, 78 Years

54

Diagram 24 55

HistoryDuring a 2-month period, the patient had noticed aloss of weight, abdominal “wind” pain, and diar-rhea. Rectal examination revealed a mobile mass inthe pelvis, and sigmoidoscopy was normal to 19cm.A barium enema showed complete retrogradeobstruction in the lower sigmoid colon consistentwith a neoplasm. Flexible endoscopy was not performed.

Operation (5.30.79)A large sigmoid carcinoma, 6cm in diameter wasresected, with an anastomosis in the intraperitonealrectum. There were no obvious metastases.

PathologyOver the peritoneal surface of the bulky tumor,there were 3 thin-walled sacs, 2–3cm in size, con-taining a clear straw colored fluid. They were relatedto pale areas overlying transmural infiltration by thecancer. The tumor was a fungating ulcer 6.5cm inlength. The depth of invasion was limited to themuscle layer. Twenty-six lymph nodes were exam-ined and no metastases were found (Dukes A, T2 N0

M0). There was an inflammatory reaction in themuscle of the bowel wall, which was more acute inthe outer layers with small abscesses. There were nohistological features to suggest the tumor was

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anaplastic. The 3 cystic structures appeared to arisefrom the serosal tissue and were lined by squamousepithelium.

Follow-UpThe patient remained well for a year. The onset ofdysphagia was investigated with a barium swallowthat showed a mediastinal mass deforming themiddle third of the esophagus. Esophagoscopyrevealed an area of inflammation but no ulceration.Thoracotomy revealed a large tumor mass arising in the muscle layer of the esophagus. It was notresectable. A biopsy confirmed the diagnosis ofmetastatic undifferentiated carcinoma consistentwith a primary colon tumor. The patient did notreturn for further visits and subsequently suc-cumbed to metastatic disease.

CommentThe serous cysts attached to the tumor were mostunusual. No reference to such lesions has beenfound in the literature. The intramural metastasisof the esophagus is also a very uncommonhematogenous spread from an extrathoracic site.Primary malignancies that have metastasized to theesophagus in this way are breast, stomach, pancreas,kidney, testes, prostate, and uterus.1,2

25 Sigmoid Carcinoma and Serosal CystsFemale, 54 Years

56

Diagram 25 57

8.7.80

HistoryThis patient presented with symptoms due to irondeficiency anemia (Hb: 59g/L) made worse by hischronic bronchitis and emphysema, which requiredoxygen administration at home. A barium enemashowed a large carcinoma of the transverse colon.Short colonoscopy to the distal extent of the tumorrevealed 3 small polyps in the left colon and sigmoidcolon diverticulosis.

Operation (5.18.95)At laparotomy there was a large spherical mass (13× 13cm) involving the mid transverse colon. Theserosal surface was lobulated and gray in color.Enlarged lymph nodes were apparent along thecourse of the middle colic vessels. There was no evidence of metastatic disease beyond the intendedperimeter of surgical excision. An extended righthemicolectomy was performed. An end-to-end anastomosis was made with a circular stapler via acolotomy.

PathologyOn sectioning the specimen, the tumor was seen tooccupy the entire circumference of the lumen withmalignant stenosis at its proximal and distal limits.The tumor had formed a huge malignant cavity contained within a “shell” of tumor. Several poly-

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poid elevations projected from the surface. Histo-logical examination revealed a moderately to poorlydifferentiated mucinous adenocarcinoma, whichextended beyond the muscularis propria to involvethe serosa. There were no metastases identified inthe lymph nodes (Dukes B, T3 N0 M0). Seven smallpolyps in the resected colon (3–10mm) were benigntubular adenomata.

Postoperative Follow-UpWith intensive care support of the patient’s respira-tory problems, his postoperative course was uncom-plicated. The patient survived the malignancybeyond 5 years and died of respiratory failure.

CommentThe morphology of this type of tumor, which formsa large cavity within an outer “shell” of neoplasm,is a definite entity, although not often encounteredby the surgeon. It is possible that these are rapidlygrowing neoplasms which are accompanied by significant tumor necrosis, thus producing a largehollow mass. No description of this morphologicalvariety of colorectal cancer (CRC) has been found inthe literature. While the morphology appearedominous in this patient, the cancer survival wasbetter than expected.

26 Cavitating Cancer of the Transverse Colon

Male, 67 Years

58

Diagram 26 59

HistoryThe patient’s prior medical history included hyper-tension, a myocardial infarct, and diabetes. For 1year, frequent episodes of diarrhea had been present.For 3 months, she complained of lower abdominalpain, worse after meals, abdominal distention,audible bowel sounds, anorexia, and loss of weight.A barium enema examination demonstrated a largelesion of the distal sigmoid colon. On examination,there was abdominal distention and an irregular,immobile pelvic mass. Sigmoidoscopy revealed apolypoid tumor of the rectum at 10cm. Biopsy con-firmed the lesion to be a moderately well differen-tiated adenocarcinoma.

Operation (4.13.89)There was a large mass involving the distal sigmoidcolon and mesentery. A loop of terminal ileum wasadherent to the mass. The liver appeared normal. Nointraabdominal metastases beyond surgical excisionwere identified. A low anterior resection was per-formed including 30cm of ileum in continuity. Thewide excision compromised the blood supply of theovaries, which were therefore removed.

PathologyThe primary lesion was an ulcer measuring 6cm inlength. There was extensive spread locally to form

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a large mass in the mesentery that had also spreaddistally to then breach the wall of the upper third ofthe rectum and “re-enter” the lumen. This mass ofneoplasm measured 11cm in length, its distal partprotruding like a “tongue,” freely movable withinthe lumen of the rectum. The mesentery of theresected ileum was invaded by carcinoma. None ofthe 12 lymph nodes showed metastases. The tumorwas moderately well differentiated adenocarcinoma(Dukes B, T3 M0 N0).

Follow-UpThe patient lived for 7 years, 5 months without evi-dence of recurrent disease. Death was the result ofcardiac disease.

CommentIt is of interest that adenocarcinoma of the largebowel can develop locally to form an extensiveprimary mass without metastasizing to lymphnodes. It is unusual to find extraluminal spread“bursting” back into the bowel. This may have beenrelated to the large extra rectal component beingimpacted in the pelvis. The “eruption” of the lesionto reenter the rectum suggested incorrectly that twoprimary cancers were present.

27 The Wagging Tongue of a Sigmoid Cancer

Female, 56 Years

60

Diagram 27 61

HistoryIn 1981 the patient (aged 37 years) underwent colec-tomy and ileorectal anastomosis for chronic ulcera-tive colitis complicated by mucoid carcinomas (2) of the ascending colon that had penetrated beyondthe muscularis propria (Dukes B, T3 N0 M0). Heremained well until 1995, when he presented witha 3-month history of abdominal pain and localizeddistention on the left side of the abdomen. Exami-nation revealed a firm mass in the left iliac fossaaccompanied by distended loops of small bowel. Thefindings were confirmed by computerized tomogra-phy (CT) examination.

Operation (7.17.95)Laparotomy revealed a mass in the posterior aspectof the left abdomen that was intimately involvedwith the mesentery of the small bowel. Mucoidmaterial was erupting from the surface of the mass,and similar deposits were in the left “paracolic”gutter and pelvis. The liver appeared normal. Exten-sive adhesions to the small bowel were not com-pletely dissected. The mass was dissected free of theleft ureter and removed in continuity with 20cm ofthe small bowel. All visible mucoid deposits wereremoved.

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PathologyThe mass (7cm in diameter) contained numerouscysts filled with “semisolid mucinous material.”Histologically the wall of the cysts was lined withcolumnar epithelium with loss of polarity andhyperchromatic nuclei (Figure 28.1). The low-gradedysplasia and mucus formation suggested mucinouscarcinoma and pseudomyxoma peritonei. Some ofthe isolated deposits showed mucin devoid ofepithelial cells.

Operation (4.12.97)Obstructive symptoms occurred 16 months after theoperation, requiring further surgery. A metastaticmass of mucinous carcinoma, densely adherent tothe left ureter, was removed. The liver was normal.No other deposits were present. Histologically,signet ring cells were identified within the tumor.

Operation (7.3.99)A further recurrence in the left lower abdomen, 12cm in size, was resected in continuity with 20cmof ileum, the left ureter, and left kidney.

Operation (8.25.01)Laparotomy was performed for a symptomatic recur-rence, most of which was excised in a “debulking”procedure. This required excision of psoas muscle, aloop of ileum, and the ileorectal anastomosis. A newileorectal anastomosis (IRA) was constructed.

Follow-UpIn March 2004, the patient was admitted to the hos-pital, suffering a massive rectal hemorrhage due to avascular fistula between the external iliac artery andthe small bowel. This was successfully controlled by the insertion of a vascular stent by interventionalradiology. The patient succumbed to metastaticdisease some months after this complication.

CommentOkuno et al report that the incidence of mucoid col-orectal cancer was 6.4% in a series of 540 patients.1

The incidence of mucoid cancers arising in associa-

28 Protracted Recurrence of Mucoid Cancer

Male, 51 Years

62

Figure 28.1: Histological features of recurrent tumorremoved 7.17.95. Pools of mucus adjacent to flattenedlow-grade adenocarcinoma.

tion with ulcerative colitis is higher: Mayer et alreport an incidence of 37% (mucinous 25%, signetring cancer 12%).2 Umpleby et al3 found suchtumors were more common in the proximal colon.Compared to nonmucinous colorectal cancer, themucinous type exhibit an increased risk of localspread, lymph node metastasis, and peritoneal dis-semination.1,3,4 The mucinous tumor may cause awidespread malignant mucinous ascites, which has been included in the classification of pseudo-

Diagram 28 63

myxoma peritonei. Ronnett et al prefer the termperitoneal mucinous carcinomatosis (PMCA).5 Inthe case described here, the recurrent mucoidtumors were less generalized. Debulking surgery, asadvocated by Sugarbaker, et al4 did achieve pallia-tion. It is of interest that the first noted recurrenceof the colitis cancer was 14 years after the colec-tomy. Over the ensuing 9 years, abdominal surgerywas performed on 4 occasions, usually followed byrelief of symptoms for approximately 2 years.

7.17.95

HistoryThe patient presented with a 2-year history of diar-rhea and intermittent rectal bleeding. Colonoscopywas limited to 30cm by a large polypoid massthought to be carcinoma. Biopsies were inconclu-sive. There were 6 polyps in the rectum 10mm–20mm size. A barium enema confirmed these findings, suggesting there was infiltration by thelarge sigmoid lesion as well as additional smallpolyps in the descending colon (10) splenic flexure(1) and hepatic flexure (1).

Operation (8.22.85)A large carcinoma of the mid distal sigmoid colonwas present. It was 11cm in length, with multiplenodules over its serosal aspect, and palpable mesen-teric lymph nodes. There were no other metastases.The bowel was resected from proximal sigmoid tomid rectum.

PathologyThere was a malignant ulcer present, measuring 6cm × 4cm, associated with submucosal permea-tion of carcinoma producing thickened “folds” onthe mucosal surface and extending over a distanceof 11cm. Small polyps (6) were also included in the resected specimen. Histological examinationrevealed undifferentiated carcinoma extendingwidely by permeation of submucosal lymphatics as well as deep penetration of the bowel wall,extending into the pericolic fat. Vascular invasion bycarcinoma was a prominent feature histologically.

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There was heavy infiltration of the tumor by neu-trophils. The serosal nodules were confirmed as carcinoma as well as metastases in a large mass oflymph nodes within the mesentery. (Dukes C:T3 N3

M0). The presence of significant numbers of malignant serosal nodules made the staging of thepathology more difficult.

Follow-Up (2003)Further polyps (60–70) were removed bycolonoscopy over the subsequent 12 years. A largepolyp (benign) in the ascending colon was removedby right hemicolectomy (9.10.97). At the last follow-up visit there was no evidence of metastaticdisease.

CommentThe tumor exhibited features of both the scirrhousand lymphangiosis forms of linitis plastica, a classi-fication proposed by Shirouzu et al.1 The lymphaticpermeations of the cancer in the submucosal layerof the colon produced remodelling of the mucosaaround the malignant ulcer to form prominentmucosal folds characteristic of one of the morpho-logical types of linitis plastica. The histologicalappearance can be anticipated by the naked eye find-ings. The resection in this patient was regarded asprobably noncurative, in the presence of serosalnodules of carcinoma, and yet the patient remainedfree of recurrence for more than 18 years after operation.

29 Anaplastic Colon CancerMale, 36 Years

64

Diagram 29 65

HistoryThe patient complained of rectal bleeding, diarrhea,and lower abdominal discomfort for 2 months.There had been a weight loss of 35lbs in 12 months.A nonmobile mass was present in the left lowerabdomen. On rectal examination and sigmoi-doscopy, abnormal mucosa was present that was nottypical of adenocarcinoma. Crohn’s Disease wasconsidered prior to referral. A barium enema demon-strated a long stricture with “scalloped” marginsinvolving rectum and sigmoid. Biopsy of “inflamedand edematous mucosa” at 10cm revealed anaplas-tic carcinoma. Biopsy of an enlarged lymph node inthe neck (Figure 30.1) showed anaplastic carcinomawith signet ring cells present. Other investigationsrevealed the patient was suffering from thyrotoxi-cosis, and therapy with carbimazole was com-menced. Despite the ominous prognosis, operationwas performed since the patient had constant rectalsymptoms and requested that at least an exploratorylaparotomy be undertaken.

Operation (12.23.69)At operation, the rectum and distal sigmoid wereconverted into a firm “hosepipe-like” structure by

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diffuse spread of the tumor. There was extensivelymph node involvement that extended into themesentery of the left colon. The distal limit of the submucosal spread appeared to be 5cm from theanal verge. There was a 1.5cm metastasis in the left lobe of the liver. Resection with a straightcoloanal anastomosis and proximal colostomy wasperformed.

PathologyExamination of the resected specimen indicated thatthe poorly differentiated tumor (Figure 30.2) arose in the rectum with diffuse submucosal, intramural,and extramural spread to produce the “tubular”morphology. Signet ring cells were prominent. Thedistal margin of the specimen was positive for carcinoma. Extensive lymph node metastases wereconfirmed.

Postoperative CoursePostoperative recovery was slow. Digital examina-tion revealed a small subclinical defect in the anas-tomosis. The patient continued to lose weight andsteadily deteriorated until he died 7 weeks afteroperation.

30 Linitis Plastica of the Colon and Rectum

Male, 32 Years

66

Figure 30.1: Cervical lymph node metastasis.Figure 30.2: Colon: Anaplastic adenocarcinoma,including signet ring cells.

CommentSignet ring colorectal cancer is rare and accountedfor 0.7% of colorectal cancer reported by Nissan etal.1 Linitis plastica is a morphological classification,and in these colorectal cancers, the incidence ofsignet ring histology is 16.6%.2 The poor survival of patients with colorectal linitis plastica is wellknown.1,3,4 This case was the most aggressive

Diagram 30 67

example encountered by the author. The operationdid achieve relief from the distressing rectal symp-toms, but resection with and end stoma or a pal-liative stoma alone would have been a moreappropriate procedure. The patient’s limited sur-vival was never going to facilitate satisfactory func-tion of a straight coloanal anastomosis after closureof the proximal colostomy.

For a full-page image of this figure see the appendix.

HistoryIn January 1989 the patient was investigated forheavy rectal bleeding. A fungating carcinoma of therectum was diagnosed 10cm from the anal verge.The lumen at this level only just permitted exami-nation by the colonoscope. Computerized tomo-graphy (CT) examination showed no evidence ofperirectal spread, but both lobes of the liver appearedto contain numerous small metastases. The patientwas informed that her life expectancy was approxi-mately 12 months. A course of “palliative” radio-therapy (45Gy) was administered to the rectaltumour followed by a further treatment (6Gy) 1month later. Sigmoidoscopy 3 months after treat-ment revealed no visible tumor, but subsequentlyrecurrence occurred. Further radiotherapy (8Gy) andlocal excision with a urological resectoscope failedto control the lesion, and the patient was referred inJuly 1990.

Sigmoidoscopy revealed a stricture of therectum at 10cm, which would not permit thepassage of the sigmoidoscope (17mm). The tumor

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edge was visible. A further CT examination of theliver was associated with ultrasound (US) examina-tion and fine needle aspiration. The liver lesionswere now regarded as cysts (Figure 31.1). This diagnosis was supported by the lack of any increasein size since the initial CT (18 months previously).

Operation (8.13.90)No metastatic disease was identified in theabdomen. The liver contained multiple benigncysts. The carcinoma was located in the intraperi-toneal rectum, and there was no evidence of perirec-tal spread. An extended low anterior resection witha proximal loop ileostomy was performed. The anastomosis was subsequently measured at 7cm.Recovery was uneventful.

PathologyThe tumor showed macroscopic evidence of tumorattenuation by radiotherapy. It was 30mm in diam-eter with a thin, rolled margin and a flat contour.

31 Curative Resection of Rectal CancerDespite Liver Metastases

Female, 55 Years

68

Figure 31.1: The review CT scan (7.30.90) shows the lowdensity foci in the liver that were diagnosed as liver cystsat this examination.

Figure 31.2: In this section, there are no obviousmicroscopic features of a radiotherapy effect on thecarcinoma. Vessels elsewhere displayed endarteritisobliterans.

The base was covered by a yellow slough. There wasthickening and edema around the lesion but noexcessive fibrosis. There was narrowing of thelumen at the level of the tumor. Histologically thelesion was well differentiated and did not penetratebeyond the muscle layer of the rectum (Dukes A, T2

N0 M0). There was minimal evidence of previousradiotherapy (Figure 31.2).

Operation (10.15.90)Closure of loop ileostomy.

Follow-Up (2004)The patient has remained free of recurrence ormetachronous colorectal tumors for 13 years, 7months.

Diagram 31 69

CommentThe error in diagnosis was devastating for thepatient, who already had a history of endogenousdepression and lived with “palliative” care for 18months. At the present time, by virtue of improvedtechniques, this radiological misdiagnosis is lesslikely. At operation, a generous distal margin (6cm)for resection was selected to enable the anastomo-sis to be made in the lower third of the rectum,beyond the area of maximum radiation effect. Theindication for the proximal loop ileostomy was thetreatment with 59Gy prior to operation. The highdose of radiotherapy, which became preoperativerather than palliative, may have significantly con-tributed to the local and systemic control of themalignancy.

HistoryThe patient presented with a 4-month history ofurinary symptoms (frequency and dysuria) associ-ated with noncolicky abdominal pain. There wereno bowel symptoms. On abdominal examination,there was a large, smooth suprapubic mass thatreached the level of the umbilicus. A urologist madea diagnosis of urinary retention, but investigationswere normal. A barium enema showed a distortedsigmoid colon “stretched” over the abdominal mass.Rigid sigmoidoscopy revealed 2 small polyps in therectum.

Operation (11.29.66)The mass was found to be a large spherical cysticmass within the sigmoid mesocolon, approximately12cm in diameter. It was intimately associated withthe sigmoid colon and a loop of terminal ileum. Twolesions (1 soft, 1 firm) were palpable in the midsigmoid colon. There was no evidence of otherintraabdominal pathology. Resection of the sigmoidcolon, mass, and 30cm of terminal ileum was per-formed with 2 anastomoses.

PathologyThe lesions in the sigmoid colon were a peduncu-lated benign polyp and a small plaque-shaped ade-nocarcinoma 15mm × 15mm. The latter was not incontinuity with the intramesenteric mass. The mass

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contained obvious tumor tissue which was friableand soft in consistency. There were areas of necro-sis and liquefaction within the mass. Adenocarci-noma was confirmed histologically in the colon andmesenteric mass. (The extent of any infiltration ofthe resected ileum is not known).

Follow-UpOne year after the operation (11.14.67), the patientunderwent laparotomy to investigate abdominalpain and anemia. Laparotomy revealed a largepreaortic mass of metastatic nodes and a furtherlarge mass in the mesocolon. No resection wasundertaken. The patient died of metastatic diseaseduring the following year.

CommentThe pathology in this patient was of interest becausethe primary tumor was small, whereas the lymphnode involvement was huge and associated withnecrosis. The author has seen this type of “mega”lymph node pathology only twice in treating morethan 2000 patients with colorectal cancer. Cur-rently, the patient would be treated with postopera-tive adjuvant chemotherapy. The prognosis of apatient undergoing a curative operation for colorec-tal cancer may be directly related to the size of thelargest metastatic lymph node.1

32 Small Sigmoid Cancer: “Mega” Lymph Node Metastasis

Male, 69 Years

70

Diagram 32 71

On admission

HistoryFor 2 months the patient had noticed an “abscess”in the right buttock associated with rectal bleeding,constipation, and loss of weight. There was a largerectal cancer found at the 7cm level, and biopsy ofulcerating nodules on the surface of the buttock alsorevealed adenocarcinoma. The buttock mass mea-sured 8cm × 15cm (Figure 33.1). Laparotomy andcolostomy were performed. The surgeon consideredthe tumor to be nonresectable. The patient wasreferred for further opinion and management. Exam-ination under anesthesia revealed a subepithelialcord of hard tissue extending from the distal edge ofthe rectal cancer into the buttock mass. The rectallesion appeared fixed. Investigations revealed no evi-dence of other metastases. The patient was treatedwith two courses of 5 fluorouracil and Lomustineadministered by regional perfusion via the rightcommon iliac artery. Preoperative radiotherapy wasconsidered but not given.

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Operation (10.4.77)Abdominoperineal excision (APE) of the rectum,including a wide excision of the buttock mass incontinuity, was performed.

PathologyExamination of the specimen revealed a large ulcerated and polypoid tumor encircling the rectum6cm above the dentate line. The “core” of malig-nant tissue connecting the primary tumor to thebuttock mass did so via an anal fistula. The tumorin both areas was well differentiated adenocar-cinoma. There were no metastases in the lymphnodes. The tumor had extended into the mesorec-tum. The margins of the pelvic dissection were notinvolved.

Follow-UpAn enlarged lymph node was noted in the rightinguinal region 5 months after the APE. Block dissection was performed, revealing metastatic ade-nocarcinoma in 1 node. The patient remained freeof metastases until he died of “natural causes” atage 82, 11 years and 9 months after the resection ofthe rectal cancer.

CommentThe initial presentation of this patient suggested a devastating malignancy with a poor prognosis.There was a reduction in the size of the buttockmass after regional chemotherapy. The combinedtreatment and the biology of the tumor were pro-bably responsible for the long period of cancer-free survival.

33 Rectal Cancer Infiltrating the ButtockVia an Anal Fistula

Male, 70 Years

72

Figure 33.1: Appearance of the right buttock prior tointra-arterial perfusion with chemotherapy.

Diagram 33 73

HistoryIn February 1976 the patient underwent a curativeresection of a moderately well differentiated adeno-carcinoma of the lower third of the sigmoid colon.The vascular ligation was immediately below theupper left colic artery. The distal margin of thetumor clearance was 5cm. A 2-layer anastomosiswas performed which was later measured at 13cmfrom the anus. At operation, the uterus was notedto be enlarged and retroverted with a large fibroid onthe upper part of the posterior surface. The carci-noma involved 2/3 of the lumen for a distance of 45mm and “partly infiltrated” the muscularispropria. One mesenteric lymph node 2cm from thecolon contained adenocarcinoma, 12 other lymphnodes were negative (Dukes C, T2 N1 M0). One yearafter operation, clinical examination and examina-tion under anesthetic revealed a mass in the “pouchof Douglas” (PD), attached to the uterus and thewall of the bowel at the level of the anastomosis.Investigations for metastatic disease elsewhere were negative. [Computerized tomography (CT) and transrectal ultrasound (TRUS) assessment of thepelvis were not available for this patient in 1976].

Operation (3.14.77)Laparotomy revealed many adhesions in the pelvisbut no metastases in the remainder of the abdomen.The uterine fibroid and the left ovary obscured therecurrent tumor in the PD. An en bloc excision ofthe conglomerate mass included the uterus, bothovaries, and 18cm of large bowel with a distalmargin clearance of 4cm of rectum. A colorectalanastomosis was performed with a single inter-rupted layer of dexon sutures.

PathologyThe recurrent tumor was 17mm in diameter andwas adjacent to the anterior aspect of the anasto-mosis. It was infiltrating the bowel wall immedi-ately above the anastomosis where ulceration of themucosa (10mm in diameter) had occurred. Therewas malignant invasion of the left ovary. The uteruswas attached to the recurrence by benign adhesions.

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The solitary mass of tumor was moderately well differentiated adenocarcinoma.

Follow-Up (2005)The patient has attended regular follow-up investi-gations. There has been no recurrence of the carci-noma since the re-resection more than 28 yearspreviously.

CommentIn a review by Abulafi and Williams, the local recur-rence (LR) rate from colon cancer was found to be between 4.5% and 17.8%.1 From the UnitedKingdom Large Bowel Cancer project, Phillips et alreported an LR rate from left colon cancer of 12%.2

Local recurrence rates of 3.1% and 3.8% have beenreported from specialist colorectal units.3,4 Thispatient was fortunate that the LR was detected onrectal examination by an experienced colorectalsurgeon during a routine follow-up visit. Interpreta-tion of the clinical findings was made more difficultby the presence of the uterine fibroids. Carcinogenicembryonic antigen estimations were not performedroutinely for this patient during 1976–1977. At thepresent time, endoluminal ultrasound would proba-bly be the investigation of choice to assess the typeof lesion described in this case. Its use in staging T3

and T4 primary rectal cancer is credited with a highdegree of accuracy.5 Robinson et al. concluded thatmagnetic resonance imaging (MRI) is an accuratetechnique for assessing locally recurrent disease.6

This accuracy may be increased with the use of anendorectal probe with MRI examination. The prog-nosis may be adversely affected by the surgical tech-nique employed in the original resection.4 Read etal. suggest a wide excision of the mesentery shouldbe performed, and, for left colon resections, a highvascular ligation is preferred.4 The outcome hasbeen related with significance to the number oflymph nodes identified in the mesentery of theresected specimen.7,8 Re-resection with curativeintent for local recurrence subsequent to coloncancer is possible in few patients.9

34 Lucky Local RecurrenceFemale, 43 Years

74

Diagram 34 75

HistoryThe patient developed ulcerative colitis (UC) at theage of 14 years (1948) and was referred for a surgicalopinion in 1966 with a history of UC for 17 years.He had recently noticed intermittent pain in the left lumbar region and pain under the left costalmargin on deep inspiration. Sigmoidoscopy showedminimal active colitis. A barium enema demon-strated total colitis, a large polypoid lesion of thesplenic flexure, and a stricture of the upper descend-ing colon 9cm in length regarded as malignant(Figure 35.1).

Operation (6.10.66)Laparotomy revealed a large mass at the splenicflexure that was adherent to the diaphragm, thelower ribs, and spleen. There were no obviousintraabdominal metastases. Biopsy of an enlargednode at the origin of the inferior mesentericartery (IMA) was negative for carcinoma. A thoraco-abdominal approach was then employed to enablefurther assessment and dissection of the mass.Resection was performed with en bloc removal of

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colon, spleen, tail of pancreas, portion of 3 ribs(11th, 10th, 12th), and the lateral aspect of the leftdiaphragm, as malignant invasion of these struc-tures appeared to have occurred. The resectedmargin of the diaphragm was sutured to the chestwall at a higher level (Figure 35.2). The right colonresection was deferred, in view of the duration andcomplexity of the left colon resection. The posto-perative recovery was satisfactory.

PathologyThe macroscopic appearance of the mucosa (fea-tureless, fragile, and hyperemic) and the histologicalfeatures were typical of chronic ulcerative colitis.The proximal carcinoma was 40 × 40mm, localizedto the colon wall (Dukes A, T2 No Mo). The distaltumor was annular, with marked narrowing of thelumen, and extended 55mm along the colon. This

35 Thoraco-Abdominal Approach toCarcinoma of the Splenic Flexure

Male, 31 Years

76

Figure 35.1: Two carcinomas at the splenic flexuredemonstrated by barium enema.

Figure 35.2: Chest x-ray 2 years after operation showsthe position of the transposed left hemidiaphragm.

larger tumor had penetrated well beyond the musclewall of the colon but was attached to the diaphragmand ribs by a prominent layer of fibrous tissue(Dukes B, T3 N0 M0). Both tumors were well differ-entiated, with obvious mucoid formation in thedistal lesion. There were 36 lymph nodes in theresected mesentery, none of which contained metas-tases on histological examination.

Operation (11.16.66)The right colon and colostomy were resected. Anend-to-end ileorectal anastomosis was performed,leaving 15cm of rectum. Postoperative recovery wassatisfactory.

Follow-Up (2005)The patient has remained well for 39 years with norecurrence or further carcinoma of the large bowel.Regular sigmoidoscopy examinations show minimalinflammatory changes of the mucosa of the rectumwithout dysplasia. Bowel frequency is 5/24 hrs.

CommentIn 1964, 15 months prior to the diagnosis of multi-ple carcinomas, a follow-up contrast enema hadshown no evidence of any lesion in the vicinity ofthe splenic flexure. He had known high risk factorsfor supervening malignancy: the colitis commenc-ing at an early age and symptom duration of 17years. Lennard-Jones has assessed the risk as 12% in patients with a history of 10–25 years.1 Mayer,reporting on 39 patients with ulcerative colitiscancer, found 18% of the patients had a history ofless than 8 years.2 The nature of the patient’s symp-toms and the size of the larger tumor on x-ray sug-gested that local extension in this “remote” regionof the abdomen could be significant. This was con-firmed at laparotomy via an upper left paramedianincision. The thoracoabdominal incision throughthe bed of the seventh rib provided optimal accessto the most difficult part of the operation. There islittle doubt that without this exposure the pathol-ogy would have been regarded as nonresectable. Inthe author’s series of 2093 patients with colorectalcancer, there were 47 splenic flexure lesions. Thethoracoabdominal approach was employed in 4patients. Landman et al. report from the ClevelandClinic that, in 5 multivisceral resections of splenicflexure cancers, some part of the diaphragm wasincluded in the en bloc excision.3 Attachment toother viscera is not always due to malignancy asthere is frequently an inflammatory reaction aroundthe tumor. Contiguous malignant infiltration hasbeen reported in 44%,4 48%,3 and 52%5 of multi-

Diagram 35 77

visceral resections. At operation it can be impossi-ble for the surgeon to define a safe plane of dissec-tion to avoid violation of the tumor clearance,which results in increased local recurrence6 anddecreased survival.7 Currently, preoperative com-puter tomography (CT) and/or magnetic resonanceimaging (MRI) examinations may be of assistance,although the distinction between fibrous tissue andmalignant infiltration can still be difficult. Thepatient’s long term survival has been associatedwith regular follow up examinations of the residual13cm of rectum. Although patients who developulcerative colitis cancer tend to have a higher inci-dence of Dukes C and D tumors, Johnson et al.found there was not a significant difference in the 5year cancer-specific survival of curative operationscompared with noncolitic colorectal cancer.8 Theonly other reference found describing the thora-coabdominal approach for splenic flexure cancer wasthat of Walfisch and Stern.9

For a full-page image of this figure see the appendix.

P A R T

V Diverticular Disease

HistoryIn August 1994 the patient was admitted to the hos-pital with severe pain and tenderness in the left iliacfossa accompanied by fever and vomiting. A pelvicultrasound suggested a possible left-sided pelvicmass. Laparoscopy by a gynecologist revealednormal ovaries and “extensive diverticular disease.”Antibiotics were administered and the symptomssettled. A subsequent barium enema confirmed the diagnosis of sigmoid diverticular disease. Ninemonths later, a series of similar episodes occurredover a period of 12 weeks, resulting in a furtheradmission to hospital for 7 days. Elective operationwas advised for recurring diverticulitis.

Operation (8.11.95)There was marked diverticular disease involving thesigmoid colon but no focus of diverticulitis wasidentified. The remainder of the colon and smallbowel were normal. Both ovaries were normal. Thelarge bowel was resected between the sigmoiddescending junction and the upper third of therectum. The anastomosis was completed with a cir-cular stapler. Two diverticula in the mid descendingcolon were inverted.

PathologyNo evidence of active inflammation was found onexamination. Marked diverticulosis was presentwith associated thickening of the muscularispropria. The mucosal folds were prominent and dis-torted, consistent with long standing diverticulardisease.

Follow-Up (2004)One year after operation, examination revealed astenosis of the anastomosis (at 10cm). There were

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no associated symptoms, and a 17mm diameter sig-moidoscope passed beyond the stenosis without dif-ficulty. This degree of stenosis was not treated. Thepatient has been free of gastrointestinal symptomsfor 9 years.

CommentMorson reported that, in a series of 173 patientswith symptomatic diverticular disease treated byresection at St Mark’s Hospital, no evidence ofinflammation was found in 32.4% of the speci-mens.1 The patients had been treated during a periodwhen various radiological criteria of inflammation,since proven incorrect, were accepted. The inci-dence of noninflammatory diverticular diseasefound after resection is infrequently reported. In theauthor’s series of 206 patients managed by electiveopen resection, the incidence was 12.1%.2 In a seriesof 162 laparoscopic resections reported by Le Moineet al., the incidence was 56.2%.3 The selection cri-teria for operation will greatly affect this incidence.These patients present with no evidence of compli-cations on clinical examination or investigation buthave a convincing history characterized by chronicleft iliac fossa pain or “attacks of diverticulitis.”The possible explanations for the absence of inflam-mation on pathological examination are: (i) resolu-tion of an inflammatory focus beyond detection; (ii) the symptoms are due to dysfunction of the colon affected by the diverticular disease; and (iii)the patient’s symptoms are due to irritable bowelsyndrome in the presence of incidental diverticulardisease.

36 Was It Diverticulitis?Female, 63 Years

80

Diagram 36 81

HistoryThe patient was referred after an urgent laparotomyduring which a transverse colostomy was performedfor an inflammatory mass in the lower sigmoid andrectum. Subsequent colonoscopy was limited to thedistal sigmoid, where a 10-mm polyp was removed(villous adenoma). Anterograde colonoscopy via thecolostomy revealed marked narrowing of thesigmoid colon due to diverticular disease and a largepolyp with a broad pedicle which could not besnared with safety.

Operation (2.23.1995)High anterior resection.

PathologyChronic inflammation, muscle thickening, andredundant mucosa were present in the sigmoidcolon due to diverticular disease. Several areas ofredundant mucosa showed intense hyperemia. In

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the center of the specimen, there was a large “polyp”40mm in width, described by the pathologist as a“semicircular flap of mucosa.” Microscopic exami-nation of the polyp revealed numerous thick-walledblood vessels in the mucosa and submucosa with noevidence of adenoma.

Operation (6.9.1995)The transverse colostomy was closed.

Follow-Up (2001)At 5 years and 9 months there were no further gastrointestinal symptoms, and colonoscopy wasnormal.

CommentThe interest in this patient is the unusual largepseudopolyp that had formed from the redundantmucosa within the sigmoid diverticular disease.This entity has been described as “polypoid prolapsing mucosal folds”1 and “crescentic folddisease,”2 the latter representing the early stage ofthis pathology. The histological appearances areidentical to those seen in mucosal prolapse of therectum.1 The changes are presumably due to trac-tion and subsequent prolapse of the mucosa, whichinitially appears as hyperemic patches. Kelly foundthis abnormality in 7% of resected specimens.1

Figures 37.1 and 37.2 (from a different patient) showtwo forms of the pathology.

37 Large Pseudopolyp of the Sigmoid Colon

Male, 59 Years

82

Figure 37.1: Hyperemic patches in chronic diverticulardisease (“crescentic” fold disease) from a different patient.

Figure 37.2: Example of a further stage of the pathology(pseudopolyp) from same patient shown in Figure 37.1.

Diagram 37 83

HistoryOne year prior to emergency admission to the hos-pital, the patient had an attack of abdominal painthat was diagnosed as diverticulitis. At the time ofthis urgent admission, he complained of left iliacfossa pain, vomiting, and diarrhea. There was lowerabdominal tenderness maximal on the left. A com-puterized tomography (CT) examination showeddiffuse induration surrounding the sigmoid colonwith bubbles of gas beyond the lumen. Some freefluid was present but no loculated collection suit-able for catheter drainage. Over a period of 4 days,there was no significant improvement and operationwas advised.

Operation (5.25.98)There was a focus of acute diverticulitis in thesigmoid colon which had perforated into thesigmoid mesentery forming an abscess which burstas soon as the sigmoid colon was handled. There wasminor contamination of the operative field by theleaking purulent fluid. There were adhesions andcollections of yellow gelatinous fibrin near the per-foration, between loops of small bowel, and in theright subphrenic spaces. Most of the sigmoid colonwas resected without anastomosis. The peritonealcavity was copiously irrigated with saline, andsuction drains were placed in the right subphrenicspaces.

PathologyExamination confirmed the diagnosis of diverticuli-tis with extensive acute inflammation and the pres-ence of a mesenteric abscess.

PostoperativeRecovery from operation was uncomplicated. Sig-moidoscopy and colonoscopy were performed and

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found to be normal, and the patient was scheduledfor Stage 2 surgery.

Operation (11.23.98)Reversal of the Hartmann operation was performedwith a stapled anastomosis in the mid rectum. Theimmediate postoperative recovery was satisfactory,but readmission was required for a bleeding duo-denal ulcer. This was managed by laparotomy andsuture of the bleeding vessel. Since then the patienthas remained free of gastrointestinal symptoms(2005).

CommentThis patient’s acute diverticulitis, present forapproximately a week prior to laparotomy, caused a fibrinous peritonitis. Free perforation (via themesenteric abscess) was imminent as evidenced bythe disruption of the abscess as soon as mobilizationof the colon was commenced. This event persuadedthe surgeon to perform a Hartmann procedureinstead of resection and immediate anastomosis,which in retrospect was probably overly conserva-tive. The patient was otherwise healthy with nocomorbidity, and there was no fecal peritonitis.There has been increasing evidence that anasto-motic integrity is not jeopardized by the presence of peritonitis.1,2 The literature currently supportsthat immediate anastomosis is safe if the patient’sgeneral state will allow the longer procedure.3,4 TheHartmann reversal operation in this patient wascomplicated by upper gastrointestinal bleedingrequiring major surgery, underlining the fact thatthis operation has the potential for significant com-plications. Despite this criticism, the Hartmannoperation may still be the safest option in an elderlypatient with comorbidity and gross peritonealsepsis.

38 Which Operation for AcuteDiverticulitis with Peritonitis?

Male, 56 Years

84

Diagram 38 85

HistoryIn 1977 the patient underwent laparotomy for a sus-pected carcinoma of the sigmoid colon. The surgeonfound a large fixed mass firmly adherent to thepelvic structures, which he considered to be inoper-able. A left-sided loop colostomy was performed.The patient was subsequently informed that herdisease was incurable cancer. After a 5-year period,by which time no deterioration in her health had occurred, the patient was referred for a sec-ond opinion. Clinical examination revealed a pelvicmass. Flexible sigmoidoscopy was possible only to25cm, at which level no malignancy was identified.Review of the barium enema films performed priorto the laparotomy in 1977 showed a stricture of thesigmoid with mucosal continuity throughout(Figure 39.1). The diagnosis was now considered tobe diverticulitis.

Operation (11.5.82)Laparotomy revealed a large hard mass in thesigmoid colon, which itself was kinked and adher-ent to the uterus, left fallopian tube, and left ovary.

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On the basis of the morphology of the mass, it wasdifficult to exclude carcinoma as the primarypathology. There was no evidence of intraabdominalmetastases. The surgical approach, however, wasguided by the interpretation of the barium enema of1977. A low anterior resection was performed withremoval of the colostomy. The uterus was separatedfrom the mass, but the left adnexal structures,involved in tough adhesions, were removed “enbloc” with the sigmoid colon. To avoid damage tovital structures on the left brim of the pelvis, intra-muscular dissection of the colon at that point left a“patch” of colon musculature protecting the leftureter and iliac vessels from dissection trauma.1

Cholecystectomy was performed for a gallstone.

PathologyThe serosal surface of the colon showed chronicinflammatory changes with an increase in the pericolic adipose tissue. Muscular thickening waspresent in the strictured segment associated with achronic inflammatory infiltration. The wall of thecolon was 11 mm in thickness. There was no focusof suppuration within the specimen. The mucosawas intact but compressed and redundant within thestricture. The findings were consistent with thediagnosis of phlegmonous diverticulitis.2

Follow-Up (2004)The patient’s recovery was delayed due to a severewound infection that appeared after discharge fromthe hospital. In the immediate and early postoper-ative period, the patient was euphoric having been“delivered” from a terminal malignancy. This wasfollowed by a longer but temporary period of pro-found depression. The patient has experienced nofurther significant bowel trouble and, at the age of89 years, was in reasonable health.

CommentAlthough the barium enema examination is notusually the strategic investigation to differentiatediverticular disease and carcinoma of the sigmoidcolon, it can be very helpful. If the stricture showsa mucosal pattern within it, then malignant diseaseis unlikely. Other features (less reliable), whichsupport diverticular disease, are a tapered transitionat the proximal and distal limits of the stricture and

39 Waiting to DieFemale, 67 Years

86

Figure 39.1: The barium enema demonstrates typicalmucosal continuity within the diverticular stricture.

a lengthy disease segment.3 If the diseased segmentof sigmoid colon can be negotiated with the colono-scope, then this is the preferred investigation. Thedegree of stricturing and the axial distortion of thecolon may obstruct endoscopy. In these circum-stances, a small caliber endoscope may be success-ful. Computerized tomography (CT) is regarded as

Diagram 39 87

an important investigation if acute diverticulitis issuspected, but its role in the assessment of long-standing disease is less clear. This patient’s bariumenema was a classic example of diverticulitis incor-rectly interpreted and incompletely investigated,which caused her the anguish of waiting to die for5 years.

HistoryThe patient presented with a 1-month history oflower abdominal pain and narrow frequent stools.Previous, less severe attacks had been diagnosed asdiverticulitis, which had responded to antibiotictreatment. There was abdominal tenderness in thesuprapubic region. Rectal examination revealed afixed, hard mass involving the anterior pelvis. Flexi-ble sigmoidoscopy was not possible beyond 20cmdue to marked narrowing of the lumen. A bariumenema demonstrated a stricture of the sigmoid colonconsistent with diverticular disease. There was nosignificant concern regarding the possibility of car-cinoma.

Operation (8.3.89)The mid third of the sigmoid colon was the site ofan inflammatory mass adherent to the bladder withan intervening chronic abscess. As the sigmoidcolon was mobilized from the pelvis, a furtherabscess was apparent between the rectum and theseminal vesicles. To reach healthy bowel wall foranastomosis, further distal dissection was required,revealing a third abscess between the rectum andthe prostate. There was no abscess track connectingthese 3 abscesses. The chronic inflammatory tissuebetween the rectum and the anterior structures wasdense and fibrous. The bowel was resected from thelower descending colon to the lower third of therectum. The rectal wall was friable and denuded ofmuscle anteriorly and, therefore, not ideal for anas-tomosis. The colon was lavaged between the site fora transverse colostomy and the divided descendingcolon. An end-to-end anastomosis was performedwith a double stapling technique. The anterior partof the “doughnut” was thin. The anastomosis, ashort distance above the pelvic floor, was too low toinsert supporting sutures. A transverse colostomywas performed to protect a precarious anastomosis.

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Suction drains were placed in the anterior pelvis todiminish the possibility of continuing sepsis fromthe “inflammatory nests” on the bladder, seminalvesicles, and prostate.

PathologyThe resected bowel measured 35cm in length. Therewas a 9cm segment of chronically inflamed colon inthe middle of the specimen. The wall of the colonin this area was markedly thickened with muscularhypertrophy and fibrosis, causing stricture forma-tion. The serosal surface was inflamed and puck-ered. Within the inflamed segment, a diverticulumcontaining pus was identified as the primary focusof the diverticulitis. The mucosa showed no specificfeatures.

Postoperative CourseThis was satisfactory, and contrast enema examina-tion of the anastomosis prior to the patient’s discharge from the hospital showed no evidence of anastomotic leak.

Operation (10.17.89)The transverse colostomy was closed.

CommentThe pericolic and perirectal abscesses were not incontinuity, and while multiple abscesses can com-plicate chronic diverticulitis, such discontinuous,deep, and anterior extraperitoneal abscesses areunusual. An ultralow anterior resection was neces-sary to obtain distal bowel for anastomosis that wasunaffected by secondary contiguous inflammation.Even so, the rectal wall was less than satisfactory.The proximal stoma combined with colon lavagewas an essential precaution.

40 Distal Abscesses and Diverticular Disease

Male, 56 Years

88

Diagram 40 89

HistoryThe patient complained of attacks of lower abdom-inal pain for 7 years. An intermittently dischargingabscess in the right ischiorectal fossa had beenpresent for 1 year (Figure 41.1). The referring doctorhad been able to pass a probe into the abscess for adepth of 12cm. A sinogram demonstrated a connec-tion with the sigmoid colon in which there wasextensive diverticular disease. Rectal examinationrevealed marked induration anteriorly and laterallyaround the rectum at the level of the prostate. Rigidsigmoidoscopy revealed no pathology to 16cm. Abarium enema confirmed the findings of the sino-gram (Figure 41.2).

Operation (5.29.75)The sigmoid colon was indurated and fixed to thepelvic floor by marked fibrosis. A proximal loopileostomy was performed. Over the next 7 months,the patient stated he “hadn’t felt so well in years.” There was some diminution in the perirectalinduration.

Operation (2.17.76)The sigmoid colon was dissected free from the rectovesical fossa. The fistula was demonstrated passingdistally between seminal vesicles, prostate, and

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rectum. This area was not dissected. There was noabscess associated with the fistula. Following resec-tion, the proximal colon was anastomosed to theintraperitoneal rectum by hand suture. Postopera-tive contrast studies demonstrated a radiologicalanastomotic leak (AL).

Operation (2.15.77)Closure of the ileostomy was delayed by the pres-ence of the AL and persistent sequestrated bariumin the presacral space related to contrast studies ofthe anastomosis.

Follow-Up (1980)The patient’s last documented follow up was 4years, 10 months after ileostomy closure. Therewere no bowel symptoms.

41 Coloperineal FistulaMale, 53 Years

90

Figure 41.1: External opening of fistula onto rightbuttock.

Figure 41.2: Fistula track and diverticular disease seen onbarium enema.

CommentIn the author’s series of fistulae due to diverticuli-tis, this is the only coloperineal fistula encountered.A report from the Cleveland Clinic by Fazio et al.included 93 patients with colocutaneous fistulae.1

Only 5 developed spontaneously, and no col-operineal fistula was reported. Parks and Gordonreported 4 cases of sigmoid perineal fistula due to

Diagram 41 91

diverticulitis, stating that no local perianal treat-ment is required, since the fistulae heal once thesource of the infection is resected.2 The operationprobably could have been managed in 2 stages ratherthan 3. There was, however, improvement with thepatient’s general health and some diminution of the pelvic reaction as a result of the preliminaryileostomy.

HistoryLower abdominal pain had been present for 2months, with an increased frequency of bowelaction. At times the stools were loose. During thisperiod there had been a fever treated with anti-biotics. There was tenderness in the left iliac fossa.No pelvic mass was apparent on rectal examination.Limited colonoscopy revealed diverticular disease of the sigmoid colon with marked narrowing of thelumen, which prevented further advancement of theinstrument.

Operation (12.23.91)There was a large inflammatory mass involving therectosigmoid and upper third of the rectum. Carci-noma could not be excluded. The left colon showedevidence of chronic obstruction. The proximal colon was distended, in particular, the cecum wasmarkedly affected with dehiscence of the seromus-cular layer. A low anterior resection (a circularstapler anastomosis) and a loop ileostomy were performed. Postoperative recovery was slow butuneventful.

Pathology ExaminationThe opened specimen demonstrated diverticulardisease with a stricture and redundant “polypoidal”

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mucosa. A diverticulum was identified communi-cating with a complex serpiginous abscess in amarkedly thickened mesorectum. Histologicalexamination showed nonspecific inflammatorychanges consistent with diverticulitis.

Operation (2.3.92)Closure of loop ileostomy.

Follow-Up (1996)Subsequent colonoscopy revealed no mucosal abnor-mality of the colon. The patient was known to be ingood health 5 years after the bowel resection.

CommentThe clinical features underestimated the extent ofthe pathology found at operation. The abscess in themesentery of the sigmoid and mesorectum wascomplex and would have tracked further if resectionhad been avoided. An abscess in the mesorectum asdistal as in this example is unusual. Although com-plicated, the disease was still localized to the largebowel and its mesentery, which greatly reduced therisk of postoperative morbidity.

42 Diverticulitis: Extensive Abscess inthe Mesorectum

Male, 66 Years

92

Diagram 42 93

HistoryThe patient presented with a 5-week history ofurinary frequency, hematuria (clots), and probablepneumaturia. Constipation had been present overthe same period. An intravenous pyelogram revealedan extravesical mass on the upper left aspect of the bladder. A colonoscopy confirmed diverticulardisease of the sigmoid colon where the lumen wasnarrowed and the mucosa hyperemic and edema-tous. A cystoscopy examination confirmed a massbulging into the upper left wall of the bladder andcovered with hyperemic mucosa. A fistula openingwas not identified. Operation was advised, mindfulof the fact that the patient’s husband had died someyears previously after a resection for diverticulardisease.

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Operation (7.28.97)Catheters were placed in both ureters. Laparotomyrevealed a mass in the mid proximal third of thesigmoid colon adherent to the left side of thebladder, which was indurated at that site. Digitaldissection separated the colon and bladder to reveala small chronic perforation in the colon that hadbeen in communication with an abscess in the wallof the bladder. The sigmoid colon and upper rectumwere resected and an anastomosis performed with acircular stapler. The abscess cavity in the wall of thebladder was curetted and suction irrigation drainsplaced into the defect. No communication betweenthe abscess and the lumen of the bladder was identified.

43 Diverticulitis: Colovesical FistulaFemale, 85 Years

94

A

Figure 43.1: Barium enema shows significantextravasation of barium into an abscess cavity (A).

A

B

Figure 43.2: Sinogram (day 11) showing significantcontraction of abscess cavity (A) and communication withbladder lumen (B).

PathologyThe resected colon showed changes of chronic diver-ticular disease. In addition to the site of the chronicperforation, there were several diverticula showingevidence of focal inflammatory changes.

Postoperative CourseOn the 10th postoperative day, a limited gastrograf-fin enema showed no evidence of a leak from theanastomosis. On the 11th postoperative day, a sino-gram showed contraction of the bladder wall abscessand a communication with the lumen of the bladder(Figure 43.2). On the 16th postoperative day, a sino-gram showed resolution of the abscess space and nocommunication with the bladder. The drain wasremoved.

Follow-Up (2005)The patient developed dementia within a year of theoperation and, at the age of 92, requires full-timecare. There has been no recurrence of gastrointe-stinal symptoms.

CommentThe diverticular abscess was clearly demonstratedby the barium enema (Figure 43.1) but not by the colonoscopy which, however, was essential toexclude carcinoma. In the assessment of colovesicalfistula (CVF), a computerized tomography (CT)examination (not performed in this case) is creditedwith an accuracy of more than 90%.1 At operation,the track of the fistula into the lumen of the bladderwas not demonstrable. It can be identified in 2/3 ofpatients with a CVF due to diverticular disease.2 Theone-stage resection, even in the presence of anabscess, is currently recommended for elective oper-ation for CVF.1,3 The prolonged suction drainage of

Diagram 43 95

the abscess site was instigated to reduce the risk of delayed pelvic sepsis.4 The communication withthe bladder was demonstrated on the postoperativesinogram (Figure 43.2).

For a full-page image of this figure see theappendix.

HistoryThe patient, whose health problems includedischemic heart disease and atrial fibrillation, presented with an episode of rectal bleeding that occurred over a 2-day period. A barium enemashowed “extensive diverticular disease involvingthe sigmoid colon, where there is a parallel sinustrack 7cm in length, inferior to the narrowedsigmoid lumen” (Figure 44.1). Colonoscopy wasonly possible to 33cm, where a stricture preventedfurther access to the colon. There were no endo-scopic features at that level to suggest malignancy.Surgical treatment was advised for the complicateddiverticular disease and the possibility of an occultcolon cancer.

Operation (3.14.88)The sigmoid colon showed features of well estab-lished inflammation with induration of the bowel

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wall, hyperemic serosa, fatty infiltration of the peri-colic tissue, and adhesions to the adjacent struc-tures, in particular, the bladder. There was nopathology in the colon proximal to the sigmoid. Thebowel was resected from the sigmoid descendingjunction to the upper third of the rectum with anastomosis.

PathologyChronic diverticulitis was confirmed. The stricture,9cm in length, was due to muscle thickening, fibro-sis and a chronic abscess. The mucosa within thestricture was edematous and redundant due to theaxial shortening of the bowel. The long abscess wasimmediately external to the muscularis propria buthad not penetrated the peritoneum covering thecolon. The site of a perforated diverticulum withinthe abscess was clearly identified.

Further ProgressPostoperative retention of urine required a trans-urethral prostatectomy. Histological examination ofthe prostatic tissue revealed carcinoma. In view ofthe patient’s age, no therapy was recommended. Thepatient was last examined 7 months after the bowelresection. The anastomosis at 13cm was satisfac-tory There were no bowel symptoms.

CommentThe presentation with a brief episode of minor rectalbleeding was not typical of chronic inflammatorydiverticular disease. The inflammatory process, welladvanced, was “silent” clinically, and, in the pres-ence of an impassable stricture, raised the possibi-lity of carcinoma. The abscess track was extramuraland subperitoneal along the axis of the bowel so thatthe infection remained localized within the peritoneal compartment surrounding the colon. The term “dissecting diverticulitis” is suggested asa suitable description for this unusual manifestationof diverticulitis.

44 Dissecting DiverticulitisMale, 83 Years

96

Figure 44.1: The barium enema demonstrates thelongitudinal abscess parallel to the bowel (arrow).

Diagram 44 97

HistoryThe patient had undergone laparotomy for a “diver-ticular abscess” 16 years previously, but details werenot available. The present illness commenced withpain in the left iliac fossa 6 weeks previously andwas accompanied by diarrhea and abdominal distention. A tender mass was present in the left iliac fossa (LIF) that was also palpable on rectalexamination. The pelvic floor was subtle on pal-pation, indicating it was not involved. Colonoscopywas limited by a stricture in the sigmoid colon.There was no endoscopic evidence of malignancy. A limited barium enema demonstrated a stricture of the mid sigmoid colon with obstruction pro-ximal to it (Figure 45.1). There was mucosal continuity within the stricture, suggesting it wasinflammatory.

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Operation (4.2.84)A large inflammatory mass was present in the midthird of the sigmoid colon “prolapsed” into thepelvis and adherent to adjacent structures. Therewas no pericolic abscess present. The large bowelwas dilated due to the obstruction. There weremany diverticula in the left colon and splenicflexure with areas of induration in the latter. Thebowel was resected from mid rectum to distal trans-verse colon, and, after retrograde irrigation of thecolon from the proximal level of resection, the anas-tomosis was constructed with a circular stapler.Although the circulation in the marginal vesselsappeared adequate, much of the colon exhibited acyanotic discoloration. This was thought to be amanifestation of the colon obstruction accompaniedby significant edema of the bowel wall. A loopileostomy was performed.

Postoperative CourseA cautious sigmoidoscopy 24 hours after operationrevealed that the colon at the anastomosis (8cm)was a good color although markedly edematous. Alimited contrast enema prior to the patient’s dis-charge from hospital demonstrated the anastomosisto be intact.

PathologyThe stricture was due to a severe focus of divertic-ulitis that had formed an extramural encirclingabscess at the level of the perforated diverticulum.There was marked fibrosis associated with theabscess as well as muscular thickening and redun-dancy of the mucosa. In the vicinity of the splenicflexure, 4 foci of localized diverticulitis were identified.

Operation (6.15.84)The loop ileostomy was closed 7 weeks after theresection.

Follow-Up (2004)No further bowel trouble has occurred in a 20-yearfollow up period.

45 Annular Extramural DissectingDiverticulitis

Female, 67 Years

98

Figure 45.1: The barium enema demonstrates thestricture, with mucosal continuity.

CommentThe onset of diverticulitis of the colon may be rel-atively silent and more like malignant disease. Theestablished pathology in this patient suggested theinflammatory process had been present for longerthan the history of 6 weeks. The annular abscess haddissected around the colon in the extramural plane.This configuration has been noted in 1% of theauthor’s series of elective resections for diverticular

Diagram 45 99

disease.1 Multiple foci have been present in 16.6%of 208 primary (no previous resection) operationsand 5 foci in 0.5%.1 The poor circulation of the colonwas attributed to the acute on chronic obstructivepathology in the bowel wall. If misinterpreted, itcould persuade the surgeon to resect an excessivelength of colon. The postoperative sigmoido-scopy on the first postoperative day was a reassur-ing investigation.

HistoryThe patient had noticed left-sided abdominal dis-comfort, night sweats, and a discharge of pale greenmucus from the rectum for 2 months. Pelvic exami-nation revealed a soft cystic mass in the pelvis. Onflexible sigmoidoscopy, there was purulent materialin the sigmoid colon and rectum and diverticulardisease was noted. A barium enema examinationreported diverticular disease with narrowing in the sigmoid colon most likely due to benign disease (Figure 46.1). Examination under anesthesiarevealed a soft fluctuant mass in the pelvis and leftiliac fossa that appeared to soften during examina-tion. A diagnosis was made of pelvic abscess due todiverticulitis.

Operation (5.19.75)There was a large cystic swelling attached to themid sigmoid colon with chronic inflammatorychanges on the surface. The swelling extended intothe pelvis. Diverticular disease was present in thesigmoid colon. Resection of the sigmoid colon withanastomosis was performed.

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PathologyA cystic swelling 10cm in diameter was attached tothe colon. On section, its wall was 0.4cm in depth.There was a communication with the lumen of thecolon (Figure 46.2). There was purulent materialwithin the “cyst,” the lining of which showedchanges of chronic inflammation. Histologicalexamination of the wall of the cyst revealed densevascular collagen tissue with 1 focus of colonicepithelium.

CommentThe giant diverticulum presented as a pelvic abscess.The lesion had formed a “sac of pus” within thepelvis. There was no radiological evidence of thelesion, which contrasts with the large gas-filled cyston abdominal x-ray that can be present.1,2,3,4 In theimmediate preoperative period, the patient’s nightsweats ceased, undoubtedly due to the spontaneousdischarge of pus into the lumen of the colon. Choonget al. reports 4 patients treated by diverticulectomy(2), initial diverticulectomy and subsequent sigmoidresection (1), and sigmoid resection (1) with suc-cessful outcome.4 In reviewing the literature, theysuggest that patients with few normal elements ofbowel wall in the diverticulum be classified as TypeI, whereas those with all layers of the bowel wall beclassified as Type II and are related to colonic dupli-cation. Patients with the thin-walled diverticulum,as occurred in this patient, are presumably the typethat might be treated by diverticulectomy.

46 Giant DiverticulumFemale, 71 Years

100

Figure 46.1: The barium enema failed to demonstratethe giant diverticulum.

Figure 46.2: The communication between the colon andthe thin-walled diverticulum.

Diagram 46 101

HistoryThe patient complained of pain and tenderness inthe left iliac fossa for 10 days. There had been rigorsduring 1 night in this period. There was no dis-turbance of bowel function. Clinical examinationwas normal. A barium enema showed well markeddiverticular disease in the sigmoid colon and anassociated “giant cyst” containing fecal residue(Figure 47.1). Colonoscopy to the hepatic flexurerevealed no stricture of the sigmoid colon ormucosal pathology.

Operation (6.10.82)There was a large cystic swelling within the mesen-tery of the sigmoid colon which was attached to thebladder by an intervening small, chronic abscess.Changes of chronic diverticular disease were appar-ent. The sigmoid colon and upper rectum wereresected with anastomosis.

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PathologyThe 20cm length of colon contained extensivediverticulosis with a large diverticulum that had athick (1cm) fibrous wall. The cavity of the lesionmeasured 4.5cm in diameter and was packed with“ribbon like” material, presumably vegetable foodresidue that was not examined histologically. It waslined by chronic inflammatory tissue, and colonicmucosa was present in some areas. Communicationwith the lumen of the sigmoid colon was identified.

CommentA giant diverticulum may be apparent on an abdom-inal x-ray as a large, gas-filled cyst (Figure 47.2).Choong et al report that this is present in mostpatients,1 although it was not so in this patient orin Case 46. The incidence of giant diverticulum inthe author’s series of elective resection for divertic-ular disease was 2/208 (1%).2 The condition requires

47 Giant DiverticulumMale, 71 Years

102

Figure 47.1: The barium enema demonstrates thediverticulum (arrow).

Figure 47.2: A giant diverticulum may present as a gas-filled “cyst” on plain x-ray of the abdomen (courtesy ofProf. E.L. Bokey). Different patient.

surgical treatment, because of the potential compli-cations of infection, torsion, and perforation.3 Thelatter two complications would seem unlikely inthis case with a very thick-walled diverticulum and

Diagram 47 103

a broad attachment to the wall of the sigmoid colon. Diverticulectomy was not considered for thispatient.

HistoryRecurrent diverticulitis had been diagnosed 15 yearspreviously, and mild episodes, always responding to antibiotic therapy, continued during this period.The patient presented with a 4-week history of lower abdominal colicky pain, constipation, andrectal bleeding on 1 occasion. A firm pelvic mass waspresent on rectal examination. A short colonoscopywas performed with the small caliber panendoscope,and a tight sigmoid stricture was negotiated, estab-lishing the diagnosis of sigmoid diverticulitis. A cautious contrast enema showed dilatation of thecolon above the stricture and no pathology in theproximal colon (Figure 48.1). Within a few weeks,the patient developed further severe pain, nausea,and vomiting. Operation was expedited.

Operation (6.24.94)Laparotomy revealed marked dilatation of the colonabove the pathology in the mid sigmoid colon. Thewall of the colon was thickened, indicative of long-standing obstruction. The colon was deflated with a

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cannula and irrigated clear of feces. The bowel wasresected from upper descending colon to lower thirdof the rectum, and a hand sutured anastomosis wasperformed. The operation was completed with aloop ileostomy.

PathologyExamination confirmed the diagnosis of chronicdiverticulitis with stricture formation at whichpoint the thickness of the bowel wall measured 15mm. There was an abscess within the stricturedarea, and the mucosa immediately proximal to thestricture was cyanotic, edematous, and ulcerated.Histologically these ulcers revealed no specific features; several diverticula in the sigmoid colonshowed early abscess formation.

Operation (8.15.94)The postoperative recovery was satisfactory. Theileostomy was closed 7 weeks after the resection.

Follow-UpNo further bowel problems occurred. Six monthsafter operation, the patient was found to have a highgrade transitional cell carcinoma of the bladder.Radical surgery was performed, but the patient diedof metastatic disease 2 years later.

CommentSurprisingly, chronic diverticular disease does notoften present with the degree of acute obstructionseen in this case. The abscess (extramural) in thestrictured segment may have precipitated the presenting obstruction. The multiple sites of early abscesses in diverticula is also uncommon.The mucosal ulceration may have been stercoral inorigin. This mucosal pathology is sometimes seenin the vicinity of an obstructing left-sided carci-noma. It may be related to an alteration in theintegrity of the mucosal defense system. The tech-nical difficulties and potential risks caused by thepresence of the dilated and thickened colon with itsfecal content were overcome by intraoperativecolonic irrigation.

48 Diverticulitis: Large Bowel Obstruction

Female, 66 Years

104

Figure 48.1: A barium enema demonstrates thediverticular stricture (arrow) and dilated colon.

Diagram 48 105

P A R T

VI Inflammatory Bowel Disease

The composite diagram illustrates some of the mor-phological types of ulceration that may be seen insmall bowel Crohn’s disease. Although a number ofthese ulcer types may appear in a patient in a con-tiguous segment or in skip lesions, one would notexpect to see the full spectrum of ulceration in anyone patient.

1. Small aphthous ulcers are the initial ulcerativelesion. They may be the only lesions or be adja-cent to more severely affected bowel.

2. Slit-like fissure ulcers in otherwise normalmucosa.

3. Edema accentuates the “cobblestone” effectcaused by transverse extensions of the linearulcers. Some of the ulcers are covered with a fibrinous exudate.

4. Deep parallel ulcers adjacent to near normalmucosa. The linear ulceration is usually locatedon the mesenteric side of the lumen.

5. Extensive linear ulceration with more loss ofmucosa isolating mucosal islands. This chronic

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disease is associated with thickening of all layersof the bowel wall and, as fibrosis predominates,a long stricture will form.

6. Recent ulceration that is solitary, deep, and situ-ated in normal mucosa. Such a lesion can be theonly focus of Crohn’s disease present in theintestinal tract.

7. Healed ulceration that is covered with atrophicmucosa and is associated with loss of mucosalfeatures and fibrosis.

8. Acute foci of ulceration surrounded by normalmucosa. The histological diagnosis of Crohn’sdisease may be difficult in this stage of thedisease.

9. Acute severe and extensive ulceration usuallyaffects only the terminal ileum in associationwith the fulminant form of acute Crohn’s colitis. The mucosa is “shredded” by the severeinflammatory process, exposing the underlyingmuscle.

49 Ulceration in Crohn’s Disease of theSmall Bowel

108

Diagram 49 109

9

8

7

6

5

4

3

2

1

History1971 At 25 years of age resection of Crohn’s

disease of the ileum. Severe diarrheacontinued despite medical treatment.

1978 Resection of terminal ileum (40cm) forrecurrent Crohn’s disease (Figure 50.1).Severe diarrhea continued (14–15/day,3–4 night). Figure 50.1 shows theinvolvement of the terminal ileum.

1981 Resection of right colon (25cm) andileum (40cm) for recurrent Crohn’sdisease associated with large inflamma-tory mass. Diarrhea continued (8/day,0/night).

1996 X-ray and colonoscopy evidence ofrecurrent disease with marked strictureformation (Figure 50.2).

1997 The patient remained debilitated withdiarrhea (12–15/day), abdominal pain,and fever. Resection of ileum (26cm)and ascending colon (6cm) was per-

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formed for advanced Crohn’s diseasewith stricture formation causingchronic obstruction. Remaining smallbowel measured 115cm.

1998 Pyelotomy for renal calculus and infection.

2000 Diarrhea (12/day), anovaginal fistula (June) with minor symptoms, managed

conservatively.2000 Patient described her health as “good” (October) with minimal symptoms from the

anovaginal fistula. No further follow-upis available.

CommentThe case illustrates the relentless natural history ofCrohn’s disease over a 30-year period. The fourresections failed to relieve the most troublesomesymptom (diarrhea). Medical therapy was nothelpful. The patient could not tolerate steroidswhich caused debilitating candidiasis. Until the lastexamination in October 2000, the patient preferredto manage without anti-inflammatory drug therapy.

50 Recurrent Crohn’s DiseaseFemale, 51 Years

110

Figure 50.1: Recurrence in terminal ileum in 1978 afterfirst resection.

Figure 50.2: Colonoscopy (1996) showing recurrentCrohn’s disease and anastomotic stricture.

Diagram 50 111

6.30.97

HistoryCrohn’s disease of the ascending colon was diag-nosed in 1984. The symptoms of abdominal pain and diarrhea were relieved by treatment with pred-nisolone and sulfasalazine. Five years later, thepatient was suffering from episodes of severe abdom-inal pain, and investigations revealed a long stric-ture of the ileum and ascending colon (Figure 51.1)associated with a large right-sided abdominal mass.

Operation (5.25.89)A large mass involving the right colon was firmlyadherent to the anterior abdominal wall and 15cmof the adjacent terminal ileum, which was alsoaffected by the inflammatory process. There were no

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51 Crohn’s Disease: Strictures ofAscending Colon and Doudenum

Female, 34 Years

112

Figure 51.1: Demonstrating stricture and spasm of theterminal ileum and ascending colon prior to righthemicolectomy in 1989.

Figure 51.2: Recurrent disease with stenosis of theileocolic anastomosis indicated by the arrow: March 2000.

Figure 51.3: The stricture D2 persists with a furthernarrowed segment in D3: May 2004.

apparent “skip” areas affected in the remainder ofthe gastrointestinal tract. A right hemicolectomywith 20cm of ileum was performed.

PathologyThe mass measured 10 × 11cm and was mainly dueto gross thickening of polypoid mucosa, musclewall, and pericolic fat in the ascending colon. Themucosal surface within the stricture was atrophic,with scars due to previous ulceration. The appendixwas markedly distended due to proximal obstruc-tion of its lumen. There were 4 ulcers in the ileum,the largest of which extended for 9cm along themucosa. Prominent enlarged mesenteric nodes werepresent. The histological changes were consistentwith Crohn’s disease. Two typical granulomas werefound in a lymph node.

Follow-Up (2004)At colonoscopy (11.30.89), a dysplastic villousadenoma (0.9cm) of the sigmoid colon was removed.Further colonoscopies were normal until 10 yearsand 6 months after resection when a stenosis of theanastomosis was diagnosed (Figure 51.2). A smallbowel x-ray series demonstrated a stricture of thesecond part of the duodenum (0.6cm in diameter).This was confirmed on panendoscopy, whichrevealed associated inflammation and ulceration.

Diagram 51 113

Biopsies showed inflammation not diagnostic of Crohn’s disease although this diagnosis wasaccepted. The patient was treated with prednisolonefor 5 months. Azathioprine and mesalazine werecommenced in 2000 and are current therapy. Thestricture in the second part of the duodenum hasbeen treated at intervals with balloon dilatation.Now 15 years since operation, x-rays show persis-tence of the stricture in the second part of the duo-denum and a moderate narrowing in the third part of the duodenum (Figure 51.3). Colonoscopydemonstrates persistence of the ileocolic anasto-motic stricture. The patient has infrequent bouts ofdistention and reflux sometimes accompanied byvomiting.

CommentThe right hemicolectomy specimen showed activeCrohn’s disease in addition to resolved inflamma-tion, which caused a mass, stricture, andpseudopolyps. The duodenal disease has persistedwith the development of a second stricture. Thepatient’s symptoms at present are not troublesomeenough to justify surgical intervention. The inci-dence of duodenal involvement in patients withCrohn’s disease is uncommon. Yamamoto et al havereported an incidence of 5% in a series of patientsfrom Birmingham UK.1

5.25.98

For a full-page image of this figure see the appendix.

HistoryIn 1982 at the age of 9 years, the patient underwentappendectomy for persistent pain in the right iliacfossa. The distal two-thirds of the appendix wasdescribed as abnormal with slight enlargement anda vascular reaction on the serosal surface. The ter-minal 10cm of the ileum was thickened and hyper-emic with some fibrin on its surface. The cecumappeared normal. The ileocecal lymph nodes wereenlarged. Histological examination of the appendixshowed mild nonspecific inflammation. In 1985, thepatient was referred with an enterocutaneous fistulathat had recently appeared in the appendectomyscar. There was a mass in the right iliac fossa andradiological investigation demonstrated a strictureof the terminal ileum.

Operation (10.25.85)A phlegmonous mass involved the ileum and cecumand was attached to the anterior abdominal wall atthe site of an ileocutaneous fistula. The terminalileum was thickened and the ileum immediatelyproximal to it dilated, indicating chronic obstruc-tion. There were enlarged mesenteric lymph nodes

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in the ileocecal region. The wall of the cecum andascending colon was thickened and a palpable masswas within the cecum. No other pathology wasdetected. A right hemicolectomy was performed,which resected 20cm of ileum and 25cm of colon.The anastomosis was performed with a single inter-rupted layer of polyglactin 910 (vicryl) sutures.

PathologyThe terminal 6–7cm of ileum was markedly thick-ened, forming a tight stricture at the ileocecal junction. The mucosa was chronically inflamed,atrophic in appearance, and there were several smallsuperficial ulcers present. There were 2 mucosal“bridges” present in the terminal ileum. Themucosa of the cecum and ascending colon alsoshowed evidence of previous inflammation, nowquiescent, with an atrophic appearance and healedulceration. The more remarkable finding was a largemass of pseudopolyps in the cecum originating fromthe ileocecal junction. Immediately inferior to thepolyps was an ileocecal fistula, which was distal to the site of origin of the ileocutaneous fistula.

52 The Appendix, Fistulae, andPseudopolyps in Crohn’s Disease

Female, 12 Years

114

Figure 52.1: The ileum shows marked ulceration, fissure,and transmural inflammation.

Figure 52.2: A pseudopolyp from the ileocecal junctionshowing a prominent lymphoid infiltrate. The architectureof the submucosa shows a traction effect.

Histologically, the mucosal changes in thepseudopolyps and ulcers showed nonspecific inflam-mation and fibrosis. The histological features in thebowel wall were consistent with Crohn’s disease(Figure 52.1).

Follow-Up (2005)Recovery from operation was satisfactory. There hasbeen no further recurrence of the disease, 20 yearssince the ileocolic resection.

CommentIn the presence of Crohn’s disease of the terminalileum, involvement of the appendix is common.Keighley and Williams report an incidence of 24%.1

They also state acute appendicitis in Crohn’s diseaseas “exceedingly uncommon.”1 If operating for symp-toms of acute appendicitis, many surgeons wouldperform appendectomy, in the presence of terminal

Diagram 52 115

ileitis, if the cecum were normal. Enterocutaneousand internal fistulae are recognized complicationsafter appendectomy in the presence of Crohn’sileitis, the site of origin of the fistula being the terminal ileum1,2 and not the appendiceal stump, as illustrated in this case report. Simonowitz et al.reviewed 20 patients who required bowel resectionafter incidental appendectomy in the presence ofCrohn’s ileitis. If the history was less than 7 days,minimal problems occurred during follow up. If the history exceeded 7 days duration, there was a28% incidence of enterocutaneous fistula and a 44%incidence of cutaneous sinus.2 The pseudopolyps inthis patient were unusually large, and this may havebeen due to chronic intussusception of the polyps atthe ileocecal junction (Figure 52.2). Although theileocecal pseudopolyps appeared multiple, the lesionmay qualify as an example of a giant inflammatorypolyp in Crohn’s disease.3

For a full-page image of this figure see the appendix.

HistoryThe patient was referred for possible surgical treat-ment in August 1988 with a 12-year history ofCrohn’s disease. He had been unwell for 12 months,with an increase in chronic diarrhea, particularly atnight (×5). A barium enema showed a “disorganized”colon with shortening, sacculation, and strictures.A colonoscopy was possible only to 30cm (stric-ture). An anal stricture was present, the mucosa inthe mid upper rectum appeared normal, and proxi-mal to this pseudopolyp formation was present. Atthis time, the patient was unwilling to undergooperation. In March 1992, clinical features ofchronic large bowel obstruction were obvious.Investigations of the bowel lumen were limited byan impassible stricture at 30cm, and a superveningcarcinoma could not be excluded (Figure 53.1).

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Operation (2.18.93)The transverse, descending, and sigmoid colon weregrossly abnormal with shortening, thickened bowelwall, fat wrapping, and serosal inflammation. Thesplenic flexure was a conglomerate mass of saccula-tion, stricture, and colocolic fistula. The lowersigmoid colon appeared normal. There was no evi-dence of small bowel disease. Abdominal colectomyand ileosigmoid anastomosis were performed (end-to-end with single layer interrupted suture). Threecentimeters of ileum was included in the resection.

PathologyThe opened colon revealed a bizarre distortion at the splenic flexure with 3 sacculations forming a“shamrock” deformity, two strictures, and a colo-colic fistula. In the transverse colon, there were 2areas of grouped filiform strands forming bridgesacross the lumen. The proximal colon showedchanges of atrophic, chronically inflamed mucosa.Distal to the strictures, the inflammation of the mucosa was more marked with ulceration and“cobblestone” mucosa. Histological examinationshowed chronic transmural inflammation with mul-tiple granulomata typical of Crohn’s disease. Theattached ileum was normal. There was no evidenceof carcinoma in the strictures.

Follow-Up (2004)The patient remains asymptomatic at 68 years ofage, 11 years and 4 months following his resection.Bowel frequency is: 2–3 per day; 0 at night. Currentmedication is sulphasalazine 2g daily (prescribed byhis family practitioner).

CommentThe long duration of colitis was responsible for thesevere malformation at the splenic flexure. The con-traction of fibrosis related to extended linear ulcer-ation, causing less-affected bowel to “pouch” orsacculate. In the past, there must have been a severe

53 A “Shamrock” Deformity Due toCrohn’s Disease

Male, 52 Years

116

Figure 53.1: The barium enema (1992) shows the degreeof bowel deformity at the splenic flexure.

exacerbation of colitis to form the pseudopolyp fili-form “bridges” in the transverse colon. This casedemonstrates the difficulty of excluding carcinomain a patient with Crohn’s disease in the presence of

Diagram 53 117

an impassable stricture. The colon stricture in longstanding Crohn’s disease was the indication for sur-gical intervention in this patient, whose symptomswere minimal.

HistoryCrohn’s colitis was diagnosed in 1979 at the age of14 years. It manifested clinically with chronic diar-rhea and multiple perineal fistulae, which respondedfor some years to maintenance therapy with pred-nisolone. In 1982, a contrast barium enema showeda remarkably shortened colon with sacculation ofthe transverse colon, “cobblestone” mucosa, incom-petent ileocecal valve, and a long stricture of the leftcolon (Figure 54.1). In 1987, the patient presentedwith a mass and enterocutaneous fistula in the rightiliac fossa. She was markedly small for her age (17years) with no evidence of sexual development. Theperineal disease was quiescent, an anal stricture was present, and the perineum deeply scarred fromprevious active fistulae. Colonoscopy revealed ashortened colon, (ileocecal valve at 50cm) with acontracted lumen, areas of pseudopolyps, and

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typical ulceration of Crohn’s disease. X-rays of thesmall bowel demonstrated a severely distorted ter-minal ileum, with “cobblestone” mucosa. After afurther period of ill health supervened with fevers,weakness, and weight loss, the patient and familyagreed to major surgical treatment.

Operation (12.14.87)The terminal ileum showed changes typical ofCrohn’s disease on its peritoneal aspect, wasattached to the anterior abdominal wall, and associ-ated with a subcutaneous abscess and 3 externalopenings of a fistula. There were no skip areas ofCrohn’s disease affecting the small bowel. The colonwas shortened and thickened with pouch-likedilatation of the transverse colon. Proctocolectomywas performed. A pedicle of greater omentum wastransposed to the pelvis.

PathologyThe bowel was thickened, rigid, and contractedthroughout its length. There was a stricture in the distal transverse colon. The “sacculation” of thetransverse colon was a prominent feature of theopened specimen in which were active ulcers. The activity of the disease varied with more acuteinflammatory disease, affecting the terminal ileum,transverse colon, splenic flexure, and rectum. Else-where, linear scars indicated the areas of previousulceration. There was a long, narrow, healed ulcerscar present in the left colon and rectum. There wasno normal mucosa present. The ileocecal junctionwas difficult to identify, and in this area there wereseveral large smooth pseudopolyps. The anal canalwas distorted by scar tissue and stricture. Histolog-ically, the bowel wall was affected by transmuralacute and chronic inflammation consistent withCrohn’s disease but without granuloma formation.

Follow-UpWithin 5 weeks, the patient reported she was a “newperson” enjoying good health. Four weeks later, pyoderma gangrenosum appeared adjacent to theileostomy. This healed in 4 months with local treat-ment. For further follow up, see Case 55.

54 A Short “Hose Pipe” Colon: Crohn’s Disease

Female, 22 Years (Part 1)

118

Figure 54.1: The barium enema demonstrates themarked shortening of the colon (1982).

CommentThis patient’s normal growth and sexual develop-ment was retarded by the onset of Crohn’s diseaseat an early age and the continuous steroid therapy.Three years after the diagnosis was established, abarium enema demonstrated the “pouch” deformity

Diagram 54 119

of the transverse colon. After a clinical duration of8 years, the chronic inflammatory process had pro-duced marked deformity throughout the colon,which was a rigid and significantly shortened “hosepipe.”

HistoryOne year after the proctocolectomy for chronicCrohn’s disease of the terminal ileum and colon (seeCase 54), the patient was experiencing episodicabdominal pain, fever, and lethargy. A small bowelx-ray showed nodularity of the mucosa throughoutits length. A blood count revealed iron deficiencyanemia and a raised erythrocyte sedimentation rate(ESR) (85). Steroid therapy was recommenced andcontinued for 4 months. A remission for 3 years wasobtained with this therapy, albeit with occasionalabdominal pain and fever. In May 1993, the patientwas admitted to hospital with severe abdominalpain, diarrhea, and fever with a palpable mass in theleft iliac fossa (LIF). Radiological investigationdemonstrated an intraabdominal abscess.

Operation (5.31.93)Laparotomy revealed extensive adhesions whichwere most dense in association with a length of mid small bowel affected by an acute, bordering on chronic, inflammation. There was an interloopabscess present which fixed the inflammatory massto the left retroperitoneal area. Enlarged lymphnodes were present in the related small bowel

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mesentery. Proximal to the pathology, the bowelwas thickened and dilated due to chronic intestinalobstruction. The abscess was evacuated and 70cmof small intestine was resected with an end-to-endanastomosis performed with a single layer of inter-rupted sutures. Long-term suction drains wereplaced in the region of the abscess.

PathologyExamination of the bowel lumen revealed 3 stric-tures and an obvious “polyposis” of the inflamedmucosa. There were multiple linear ulcers typical ofCrohn’s disease. The strictures involved all layers of the bowel wall and were due to chronic inflam-mation, edema, and fibrosis. The inflammatorychanges were transmural, and, although no granulo-mas were identified, the histological appearanceswere consistent with Crohn’s disease. The histolog-ical examination of the polyps identified them as“evolving inflammatory polyps” with marked sub-mucosal edema (Figure 55.1).

Follow-UpIrrigation and drainage of the abscess cavity wasnecessary for 4 weeks. By 8 weeks, the patient hadresumed her favorite sport of horse riding. Sincethen the patient has remained stabilized on azoth-iaprine therapy and is without clinical evidence ofrecurrent disease. She continues to accumulateprizes for her horse jumping in major competitions.

CommentRecurrence involving the small bowel rapidly super-vened within 1 year. The surgical treatment in thispatient was resection rather than stricturoplasty, inview of the “clustering” of the 3 strictures. Thepolypoid mucosa was due to foci of submucosaledema beneath an intact lamina propria and proba-bly represents some of the earliest morphologicalchanges of Crohn’s disease. Pseudopolyps in Crohn’sdisease can be due to a combination of submucosaledema and fissure ulcers producing the “cobble-stone” appearance, or caused by severe ulcerationleaving islands of polypoid granulation or intactmucosa.1,2

55 Recurrent Crohn’s Disease:Pseudopolyposis

Female, 28 Years (Part 2)

120

Figure 55.1: Edema has significantly increased the depthof the submucosa, which is responsible for the smallpseudopolyps.

Diagram 55 121

HistoryThe patient presented with an 18-month history ofepigastric and back pain, anorexia, and loss ofweight. This was associated with postprandialabdominal distention. His bowel habit was normal.Physical examination revealed a tense palpablececum. A barium enema showed an unusual extrin-sic deformity in the mid transverse colon associatedwith angulation and narrowing of the colon (Figure56.1). Carcinoma of the pancreas was considered asa possible diagnosis.

Operation (3.17.78)Laparotomy revealed a large complex area ofCrohn’s disease involving the distal ileum, whichwas attached to the mid transverse colon. At thissite, there was an ileocolic fistula. Distal to thiswere 2 further sites of ileal Crohn’s disease, themore distal of which was associated with an ileo-ileal fistula. There was marked dilatation of the

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ileum (the circumference was 15cm) immediatelyproximal to the ileocolic fistula, and proximal tothis there were further short skip segments ofCrohn’s ileitis. Ten centimeters of transverse colonwas resected en bloc with 120cm of ileum. Resec-tion of the small bowel mesentery was difficult, dueto the marked lymphadenopathy, and this resultedin extension of the length of small bowel removeddue to impaired circulation. The small bowelresected was affected by 4 segments of Crohn’sdisease. Two minimally affected areas in the proxi-mal ileum were not resected. The two anastomoseswere completed with a single layer of interruptedsutures.

PathologyThe lumen aspects of the affected ileum showedtypical morphology of Crohn’s disease. There waslinear ulceration, “cobblestone” mucosa, thickenedbowel wall, and marked stricture formation. At thesite of the ileocolic fistula, the lumen of the ileumpermitted only the passage of a probe along the stric-ture. The fistula opening in the transverse colon was6–8mm in diameter, and immediately adjacent to itthere were polypoid changes in the mucosa. The restof the colon mucosa was normal. At this level, therewas stenosis of the transverse colon, which wasattached to the adjacent ileum by dense, tough adhe-sions. Histological examination confirmed the diag-nosis of Crohn’s disease.

Follow-UpRecovery from operation was uneventful and bowelfrequency was × 1 per 24 hours until 2 years afterthe bowel resection, when diarrhea and loss ofweight occurred. He was found to be thyrotoxic.These symptoms partially abated with radioactiveiodine therapy. For further follow-up, see Case 57.

CommentThis patient’s presentation with epigastric and backpain associated with a very unusual deformity of thetransverse colon led to a preoperative diagnosis ofcarcinoma of the body of the pancreas. Computer-ized tomography (CT) examination was not avail-

56 Presentation of Crohn’s Ileitis as anAbdominal Malignancy

Male, 54 Years (Part 1)

122

Figure 56.1: The deformity in the mid transverse colon(barium enema 3.3.78).

able, but colonoscopy would have been an appropri-ate investigation. It is surprising that, in the pres-ence of an ileocolic fistula, the patient had nodisturbance of bowel function. His pain must havebeen due to the marked degree of bowel obstruction.

Diagram 56 123

At the present time, such pathology would betreated with a combination of resection and stric-tureplasty. The role of strictureplasty and its resultshave been well defined from the experience at theCleveland Clinic.1,2

HistoryFour years after a bowel resection for Crohn’sdisease (Case 56), this patient was admitted to hos-pital with a 2 week history of lower abdominal painand alternating diarrhea and constipation. A tendermass was present in the right iliac fossa. A smallbowel contrast series showed fixed loops of dilatedsmall bowel indicative of “mild obstruction” (Figure57.1). Recurrent Crohn’s of the small bowel wasdiagnosed and treatment with prednisolone initi-ated. The patient’s reasonable progress over the next 15 years was interrupted by 3 significantepisodes of small bowel obstruction. Radiologicalinvestigations demonstrated 2 or 3 small bowelstrictures with evidence of chronic obstruction(Figure 57.1). Colonoscopy was normal up to andincluding the ileocecal valve. The patient nowagreed to surgical treatment.

Operation (9.5.97)There were 2 short strictures a short distance prox-imal to the previous anastomosis in the ileum. Large

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“saccular” dilatations had formed in relation tothese strictures and this area was affected by adhe-sions. Proximally, there was an area of induration inthe ileum, indicating minimal stricture formation.Excluding this focus of disease, 29cm of ileum wasexcised, removing 2 strictures. Care was taken toensure an adequate blood supply to the terminalileum in view of the previous resection in this area.The anastomosis was completed with a single layerof interrupted sutures. The fragile mesentery wasclosed. The residual small bowel measured 150cm.

PathologyThe lumen of the resected bowel revealed longitu-dinal scars of healed ulceration, more marked in the region of the strictures. Two ileo-ileal fistulaewere present with adjacent areas of inflammation.Histological examination confirmed the diagnosis ofCrohn’s disease. In the distal part of the resectedsmall bowel there was a long pedunculated polyp 15mm, which was inflammatory.

Follow-Up (2004)There has been no further recurrence of Crohn’sdisease in 7 years. The patient’s bowel frequency is1–2 per 24 hours. His principal health problem hasbeen the onset of Parkinson’s disease.

CommentRecurrence is a well known feature of Crohn’sdisease, and it is likely that there is a long period ofasymptomatic recurrence before the diagnosis ismade. Within 6 months of resection, there is a highincidence of recurrence on endoscopic examina-tion.1 Reoperation in this patient was performed 19years after the initial surgery, but the first episodeof bowel obstruction due to recurrent stenosisoccurred at 4 years postresection. There was no evi-dence of Crohn’s disease at the resection margins in1978, when the disease-free margins were generousin length. The Cleveland Clinic study has demon-strated that recurrence is not related to the lengthof normal bowel clearance or disease-positivemargins.1 Borley et al found that there was a signif-

57 Crohn’s Disease 19 Years After Initial Resection

Male, 73 years (Part 2)

124

Figure 57.1: Small bowel series (1993) showing evidenceof chronic obstruction and at least 2 strictures.

icantly higher recurrence rate for ileal disease thanfor ileocolic or colic Crohn’s disease.3 In a study of51 patients with intestinal fistulae due to Crohn’sdisease (including 9 entero-enteric fistulae), Poritz etal. found the fistula was usually at the site of activedisease and recommended surgery as the preferred

Diagram 57 125

treatment.4 The pathology found at this patient’sreoperation was an inflammatory conglomerate,which did not appear suitable for strictureplasty.Despite the patient’s 81 years and shortened smallintestine, his bowel function is within normallimits.

Anastomosis3.17.78

HistoryIn 1976, the patient presented with a 9-monthhistory of diarrhea and anal discharge. Sigmoi-doscopy to 20cm revealed an anterior anal fissureand a mild patchy proctitis. A barium enemademonstrated a long stricture of the descendingcolon with almost complete obstruction “due toadvanced Crohn’s disease” (Figure 58.1). Rectal biop-sies were consistent with this diagnosis. Over thenext 9 months, the patient was treated with oralprednisolone, resulting in intermittent improve-ment. In April 1977, a cautious barium enemashowed persistence of the stricture and gross dila-tion of the transverse colon (Figure 58.2). Electiveoperation was arranged but was superceded byurgent admission due to acute bowel obstructionand signs of peritonitis.

Operation (5.24.77)The colon was markedly distended (12cm in diam-eter) proximal to the chronic inflammatory process

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in the descending and sigmoid colon. This part ofthe colon was thickened, contracted, deeply con-gested, and adherent to the left paracolic gutter withdense adhesions. In the anterior wall of the trans-verse colon, there was a 4-mm perforation withsome minimal fecal spill and localized peritonitis.The small bowel was normal in appearance withoutdistention. Colectomy and ileo-rectosigmoid anas-tomosis were performed, associated with a proximalloop ileostomy.

PathologyThe bowel wall proximal to the stricture was thick-ened, indicating chronic obstruction. The perfora-tion in the transverse colon showed nonspecificnecrotic changes and was not the site of the Crohn’sdisease. The stricture was due to marked thickeningin the wall of the colon due to long standing fibro-sis. In addition, prominent polypoidal change in the

58 Large Bowel Obstruction: Crohn’s Disease

Male, 46 Years

126

Figure 58.1: The x-ray demonstrates a long, tightstricture of the descending colon (7.6.76).

Figure 58.2: Large bowel obstruction is apparent onlimited contrast enema 3 weeks prior to emergencyoperation. The transverse colon diameter measures 13cm.

mucosal surface had further compromised thelumen of the bowel. There was ulceration present,partly obscured by the polypoid mucosa. Two linearulcers measured 3cm and 5cm in length. Histolog-ical examination confirmed the diagnosis of Crohn’sdisease.

Postoperative CourseThis was complicated by a profuse hemorrhage from a gastric ulcer, adrenal hypofunction, and pro-longed ileus requiring total parenteral nutrition(TPN). Two months after operation, abdominalsurgery was required to drain subphrenic abscesses(right subhepatic and left perisplenic).

Follow-UpThe proctitis in the lower rectum persisted withexacerbation, causing stool frequency of up to 12 ×per day during the worst periods. Medical treatmentwas continued with maintenance azathioprine, sulphasalazine, and local steroids. In 1994, refluxesophagitis and stricture (biopsies: benign) werediagnosed. In 1998, the patient was found to be suffering from carcinoma of the stomach antrum,which was inoperable and caused the patient’s deathin 1999.

CommentThe management of this patient was less than idealin that elective surgery should have been performedinstead of an emergency procedure precipitated by a life threatening complication. While the shorthistory of 9 months and the patient’s obesity(weight: 321lbs) were relative contraindications, thepresence of a tight stricture, obstructive symptoms,and a constant requirement of 30mgm of pred-nisolone daily were indications that elective opera-tion was appropriate. The perforation of thetransverse colon appeared to be caused by theextreme dilatation of the obstructed colon. Perfora-tion of the colon in Crohn’s colitis has been consid-ered to be a rare complication.1 This is probably dueto the fact that the presence of a stricture in thecolon is an indication for elective surgical treat-ment. Strictures of the colon in Crohn’s diseaseshould always raise the possibility of superveningcarcinoma. Even if examined by colonoscopy biopsy,

Diagram 58 127

the diagnosis may not be apparent until a resectedspecimen is examined histologically. Although it isnow recognized that there is an increased risk of colorectal cancer in Crohn’s colitis, the number ofcases reported are few: St Marks Hospital, UK: 15 in52 years;2 Queen Elizabeth Hospital, Birmingham,UK: 8 in 30 years;3 and Mount Sinai Hospital, NewYork: 30 in 29 years.4 These reports indicate the riskof colorectal cancer is increased in long standingdisease, particularly when the disease commencesat a young age.3,4 Patients with extensive colitiswere found to have an 18-fold increased risk of colo-rectal cancer.3

For a full-page image of this figure see theappendix.

HistoryUlcerative colitis had been diagnosed more than 2years previously. A severe attack of colitis super-vened in April 1992, necessitating 2 admissions tothe hospital with clinical signs of septicemia anddilatation of the colon. With conservative treatment(steroids) he made very slow progress over a periodof 3 months, but was readmitted with abdominalpain and constipation. The patient looked unwell,groaning with pain. There was generalized abdomi-nal distention and tenderness most marked in theright iliac fossa. A plain abdominal x-ray showeddilatation of the colon and a large collection of fecesin the right colon. On referral, laparotomy wasadvised.

Operation (7.31.92)At operation the colon was grossly dilated withinflammatory changes on its serosal surface. Thelumen was loaded with soft feces and the wall of thebowel was thickened and friable. The colon wasadherent in 3 sites, over a broad attachment to theanterior abdominal wall (1), liver–stomach (2), and aloop of jejunum (3). To minimize fecal spill, thecolon was gently irrigated, via an ileotomy, with 22liters of saline solution (evacuated per rectum) untilclear of feces. Separation of the colon from the adhe-sions revealed 3 massive perforations at least 40mmin length where the colon wall had disintegrated,forming extensive ulcers, the base of which was theadherent structure. The large bowel was resected tothe level of the mid sigmoid. An end ileostomy andsigmoid mucous fistula completed the procedure.Peritoneal contamination was minimal.

PathologyThe lumen aspect of the bowel showed extensiveulceration that had left a few “islands” of inflamedmucosa on the surface of the denuded muscle. Areasin the cecum showed macroscopic and microscopicchanges consistent with acute-on-chronic ulcerativecolitis.

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Further ProgressRecovery from operation was slow but uneventful.

Operation (7.19.93)The distal sigmoid and rectum were excised and a restorative proctocolectomy and loop ileostomyperformed.

Operation (11.1.93)Closure of the loop ileostomy.

CommentAt the time of this patient’s first 2 admissions, hesuffered acute toxic megacolon and should havebeen assessed by a surgeon. The importance of com-bined physician and surgeon management had beenignored.1 The patient was fortunate that free perfo-ration of the colon and fecal peritonitis did notoccur. Turnbull et al. were the first to emphasize thepathology of large penetrating ulcers in acute toxicmegacolon sealed off by the omentum, viscera, orparietes.2 This patient’s incomplete recovery fromthe acute phase of the illness left the colon chroni-cally dilated and malfunctioning as an adynamicobstruction. At the time of his elective operation,he was fit enough for an abdominal colectomy to beperformed. The Turnbull “blowhole” ileostomy–colostomy procedure was not considered. Prelimi-nary colon irrigation via an ileotomy substantiallyreduced the risk of fecal contamination when thelarge defects in the bowel wall were exposed bymobilization. The technique of irrigation wassimilar to that advised by Khoo et al, who proposethe method as a technique to facilitate resection fortoxic megacolon.3 The Cleveland Clinic experienceof the “blowhole” operation has been significantlyreduced. In the 18 year period, 1983–2001, it hasbeen employed in only 6/328 (1.9%) of patients withtoxic colitis due to inflammatory bowel disease(IBD).4

59 Subacute Toxic Megacolon Due toUlcerative Colitis

Male, 29 Years

128

Diagram 59 129

1

3

2

HistoryThis patient underwent urgent laparotomy (Febru-ary 1989) for toxic megacolon that was initiallyinterpreted as mechanical large bowel obstruction.The surgeon performed a loop colostomy in thetransverse colon. The true nature of the disease was revealed with a subsequent colonoscopy, whichdemonstrated severe colitis from the rectosigmoidto the cecum. The patient made a satisfactory re-covery and was referred for further management 6months after the urgent operation. Colonoscopyrevealed an active colitis commencing at the rec-tosigmoid, with extensive polyposis extending tothe transverse colon. The colon proximal to thecolostomy was diffusely inflamed without obviousulceration or polypoid lesions. The mucosa of a largeprolapsed transverse colostomy was relativelynormal.

Operation (10.16.89)Adhesions involving an otherwise normal smallbowel were dissected. There were vascular changeson the serosal surface of the colon consistent withan underlying inflammatory process. The colon wasresected from the cecum to the rectosigmoid junc-tion, and an end-to-end anastomosis between ter-minal ileum and rectosigmoid was completed witha single-layer interrupted suture of 3/0 polyglactin910 (vicryl).

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PathologyThe mucosa proximal to the colostomy was activelyinflamed, showing hyperemia, edema, and small fociof bleeding. The mucosa of the colon between thecolostomy and the splenic flexure showed a cobble-stone effect with some linear ulceration, indicatingan active chronic colitis. The colon distal to this was contracted, with its lumen covered with smallsessile pseudopolyps between which the surface ofthe lumen was scarred without active ulceration.The pathology report stated that the mucosa of thedistal 5cm of the specimen was “almost normal” inappearance. The histological features of the bowelwall confirmed the diagnosis of chronic ulcerativecolitis with pseudopolyp formation (Figure 60.1).

Postoperative Course/Follow-Up (2005)Recovery from operation was satisfactory. Twomonths after operation, his bowel frequency wasDay/Night: 4/1. On flexible sigmoidoscopy, themucosa of the rectum appeared normal, the anastomosis was at the 18cm level. These featuresremained unchanged throughout a follow up periodof 5 years which included regular sigmoidoscopyexaminations. The patient reports that he is well forhis 84 years, more than 15 years since the operation.

CommentThe clinical details preceding the patient’s emer-gency laparotomy are not available, but the surgeonrecorded that he undertook the operation for largebowel obstruction. Subsequent findings suggest theevent was acute toxic megacolon due to ulcerativecolitis (UC). The rectal sparing raised the possibilityof Crohn’s disease (CD) (excluded by the histologi-cal examination) and facilitated the ileo-rectosig-moid anastomosis. Interestingly, the patient’s colonexhibited 3 distinct morphological types of ulcera-tive colitis. The pseudopolyps were principally“mucosal islands” consistent with a previous severeepisode of colitis. Goligher reported that 20% of hispatients with total or substantial colitis were foundto have pseudopolyps.1 Pseudopolyps are morecommon in UC than CD, may be localized to asegment of colon, and do not usually involve thelower 10cm of rectum.1 They may be sessile, pedun-

60 Colitis and PseudopolyposisMale, 68 Years

130

Figure 60.1: Inflamed mucosa forms an inflammatorypolyp, which is one variety of pseudopolyp.

culated, or filiform, usually less than 15mm in size,but they can form large masses.2,3 Histologically,they may be islands of normal or inflammatorymucosa, excessively regenerative glands, or epethe-lialized foci of granulation tissue. The morphologyof the colon may persist with little change, and the

Diagram 60 131

inflammatory change may even become quiescent.The pseudopolyps have no specific malignant poten-tial.1,3,4,5 Their presence alone is not an indication for colectomy.1 Macroscopic Dysplasia AssociatedMass Lesion (DALM) may be differentiated by itsmorphology and histological appearance.

HistoryThe patient’s colitis had been diagnosed at 10 yearsof age, and after 18 years of medical treatment, herdiarrhea was still disabling (Day/Night = 8/1). Sig-moidoscopy revealed chronic changes of proctocoli-tis without obvious ulceration. A biopsy showedhistological changes consistent with chronic ulcer-ative colitis. Colonoscopy was not performed (1974).A barium enema had demonstrated a smooth stric-ture of the transverse colon.

Operation (4.23.74)Colectomy and ileorectal examination was per-formed. The distal level of resection was 2cm belowthe sacral promontory.

PathologyIn the distal half of the resected specimen, themuscle wall of the colon was thickened and themucosa showed multiple small areas of ulceration.

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The stricture in the transverse colon was lessobvious than on x-ray. Histological examinationconfirmed the diagnosis of chronic ulcerativecolitis. There was no evidence of carcinoma or dysplasia.

Follow-UpIn a few months, the patient’s bowel frequencybecame stable (4–5/24). She attended sigmoidoscopyassessment regularly. After 1981, no biopsies wereperformed for dysplasia (at the patient’s request) asrectal bleeding, requiring transfusion, had occurredafter 2 of these examinations. The endoscopicappearance of the chronic proctitis did not changeuntil 1990, when 4 small white ulcers were notedbelow the anastomosis at 14cm. The ulcersappeared to be inflammatory and biopsy was notperformed. Sigmoidoscopy on 12/19/97 revealed asmall (5mm) sessile polyp on the posterior wall ofthe rectum at 11cm. On palpation, it felt soft and its appearance was benign. Diathermy snareremoval was performed. Histological examinationshowed well differentiated adenocarcinoma arisingin a dysplastic villous adenoma.

Operation (1.5.98)The small bowel was dissected free of many adhesions. A restorative proctocolectomy and loopileostomy were performed.

PathologyExamination of the specimen revealed that the siteof the small carcinoma was related to a flat palepolypoid change in the mucosa 50mm in its largestdiameter. Histologically, this was regarded as Dysplasia Associated Mass Lesion (DALM) (Figure61.1), but the distinction between this and aninflamed villous adenoma can be difficult. Therewas no evidence of residual carcinoma. The ulcersnear the previous anastomosis were consistent with

61 Ileorectal Anastomosis for ChronicUlcerative Colitis: Early Diagnosis of Carcinoma: Late Diagnosis of Large Polypoid Lesion

Female, 51 Years

132

Figure 61.1: Intense inflammation in a macroscopiclesion interpreted as a Dysplasia Associated Mass Lesion(DALM).

ulcerative colitis. Six mesorectal lymph nodesshowed no metastases. Long standing anal fissuresshowed no specific features.

Operation (5.18.98)Closure of ileostomy.

CommentThe patient remains free of recurrent cancer 7 yearssince resection of the rectum. Although regularexamination with a rigid sigmoidoscope detected anearly carcinoma, it failed to diagnose an extensiveflat lesion. Flexible endoscopy with its magnifica-tion would probably have been more successful. Ifthe patient had permitted routine random biopsies,it may have detected the dysplastic polyp. The riskof supervening carcinoma after ileorectal anastomo-sis (IRA) has been well documented, and early diag-nosis, even with careful surveillance, is not alwayspossible. Johnston et al reviewed 155 patients withulcerative colitis treated by resection and IRA.1

During the follow up period (3 months through 40years), 11 patients developed carcinoma in the resid-ual rectum. The estimated probability of developingrectal cancer was 17.1% after 27 years. The 11tumors were advanced stage, high histologic grade,and the median cancer specific survival was 14months.

Diagram 61 133

12.9.97For a full-page image of this figure see the

appendix.

HistoryThe patient, aged 9 years, was first seen by theauthor within a few days of the first episode of diarrhea and rectal bleeding (1972). Within 1 week,a significant proctocolitis to 20cm was apparent.Treatment with intermittent courses of pred-nisolone and maintenance with sulphasalazine wassupervised by the attending gastroenterologist. In1982, colonoscopy revealed total chronic colitiswith pseudopolyp formation, and biopsies were con-sistent with ulcerative colitis. The patient’s familyrejected proctocolectomy at this time. An exacerba-tion of the colitis resulted in a short hospital stay in1988. Follow-up colonoscopies revealed slow pro-gressions of the colitis with pseudo polyps, contrac-tion and rigidity of the distal colon. No dysplasiawas identified in random biopsies. Colonoscopy(9.23.94) identified chronic active disease in theright colon and more acute inflammation in the left colon and rectum. The patient was advised toundergo restorative proctocolectomy but declined.Thirteen months later, cystoscopy was performed toinvestigate dysuria and hematuria. Inflammatorychanges were present in the bladder. Sigmoidoscopynow revealed an obstructing carcinoma in the upperthird of the rectum (Figure 62.1).

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Operation (12.4.95)A large malignant mass in the rectum was foundattached to the upper surface of the bladder. Therewere no metastases identified beyond this mass. Thecolon was dilated, indicating obstruction was wellestablished. There was a marked inflammatory reac-tion in the right anterior aspect of the pelvis. Proc-tocolectomy–end ileostomy was performed with enbloc partial cystectomy. The omentum was mobi-lized and used to fill the pelvic space.

PathologyThe carcinoma was annular, causing constriction of the lumen. Its length was 60mm. A perirectalabscess between the rectum and the bladder wasdraining pus into the lumen of the bladder, but nofistula from the rectal lumen was identified. Asegment of vas deferens was attached to the rightaspect of the specimen. Histologically, the tumorwas a well differentiated mucinous adenocarcinomawhich extended locally beyond the muscularispropria, but did not invade the bladder wall. Nometastases were detected in the examination of 12lymph nodes (Dukes C, T3 N0 M0). Chronic ulcera-tive colitis without dysplasia was present through-out the large bowel.

Postoperative Course/Follow-UpRecovery from operation was complicated by a deepvenous thrombosis and gastrointestinal bleedinginduced by heparin therapy. Sexual function in rela-tion to surgery had been a concern for the patient,but this proved not to be a problem, and his wife’spregnancy commenced 3 months after the procto-colectomy. Unfortunately, abdominal metastasesbecame apparent 18 months after operation. Therewas no response to chemotherapy and the patientdied 25 months after the resection.

CommentThe patient’s colitis-cancer was diagnosed after 23years of active disease. It was an unusual experiencefor a surgeon to follow the patient’s inflammatorybowel disease a few days after its commencement

62 Childhood Ulcerative Colitis: Rectal Cancer

Male, 34 Years

134

Figure 62.1: Carcinoma at 12–13cm seen onsigmoidoscopy (11.16.95).

until the patient’s death. It was regrettable that atleast twice the patient almost underwent electivesurgery. In 1982, a permanent ileostomy wasrejected, and, in 1994, the discussion on postopera-tive impotence proved to be a deterrent. Dysplasiawas never detected on repeated colonoscopic biop-sies. The extensive tumor was diagnosed 13 monthsafter a colorectal surgeon performed a colonoscopy.There had been no alteration in bowel symptoma-tology. The practice of regular cancer surveillance inthese patients unfortunately does not always yieldearly-stage disease,1 although Lennard-Jones et al

Diagram 62 135

from St Mark’s Hospital found that 70% of tumorsdiagnosed in patients attending a surveillanceprogram for total ulcerative colitis were eitherDukes A or B.2 They also agreed that surveillancefor mucosal dysplasia had limitations. The originalpaper by Morson and Pang described it as “a usefulbut imperfect marker” in colitis–cancer detection.3

Currently, the problems are that to be effective,extensive biopsy sampling is necessary, interpreta-tion by an expert pathologist can be difficult, and upto 25% of established colitis–cancers are not associ-ated with dysplasia.4

12.4.95

For a full-page image of this figure see the appendix.

HistoryThe patient had been aware of constipation for aperiod of 4 months and, for 6 weeks, had noticed afrequent and frustrated desire to defecate. Duringthis latter period, a small amount of bleeding andmucus appeared with the stools. On examination,distention of the left and right colon was palpable.Rectal examination revealed a large firm mass in thepelvis, and flexible sigmoidoscopy revealed edema ofthe mucosa at 15cm where the further lumen couldnot be visualized due to angulation of the rectum.A limited barium enema demonstrated a stenoticlesion in the rectosigmoid (Figure 63.1).

Operation (1.31.92)Laparotomy revealed a large malignant tumor of therectosigmoid with significant serosal involvementover the tumor that was contiguous with the adja-cent parietal peritoneum. No metastatic disease wasdetected on examination of the abdomen. The colon

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was markedly distended and loaded with feces. Thewall of the sigmoid and left colon was thickened andedematous. A low anterior resection was performed,including the abnormal left colon to include the

63 Obstructive ColitisFemale, 55 Years

136

Figure 63.1: Barium enema showing a malignantrectosigmoid stricture (arrow).

Figure 63.2: Clumps of adenocarcinoma surrounded bypools of mucin. Signet ring cells were identified elsewherein the tumor.

Figure 63.3: Typical changes of ischemia causinghemorrhagic infarction. Necrosis has occurred in thelamina propria.

splenic flexure. This procedure was combined withon-table lavage and a loop ileostomy.

PathologyExamination of the specimen revealed a stenotictumour 65mm in length. Commencing 50mm prox-imal to the lesion and reaching the splenic flexure,the mucosa was intensely inflamed with longitudi-nal and parallel ulceration. The lymph nodes notedin the mesentery were small. Histologically, thetumor was a poorly differentiated mucinous adeno-carcinoma (Figure 63.2) with a prominent signet ringcell population extending through the bowel wallinto the pericolic tissue. The ulcerated mucosashowed features of acute hemorrhagic infarctionextending into the submucosa without evidence ofpreexisting inflammatory bowel disease (Figure63.3). Six lymph nodes contained metastatic muci-nous adenocarcinoma. There was extensive lym-phatic and perineural invasion of the perinodaltissue (Dukes C, T3 N2 M0).

Follow-Up (2005)The ileostomy was closed 3 months after operation,by which time adjuvant chemotherapy had beencommenced with 5-Fluorouracil and Levamisole.This treatment was administered for 12 months.The patient has continued follow-up surveillancefor more than 13 years without evidence of recur-rent disease.

CommentDespite the ominous nature of the pathology of thetumor, long-term survival has occured. It wouldseem appropriate to attribute this success to adju-vant chemotherapy, as the results from surgeryalone are very poor for signet ring adenocarcinoma.The ulcerating colitis observed in this patient is dis-tinct from ulcerative and Crohn’s colitis and wasprobably first described in the early 1940s. Furtherreports have described the characteristic features ofthe disease.1,2,3 The colitis may be near total or seg-mental, and typically there is a zone of normalmucosa immediately proximal to the obstructivepathology which most frequently is a carcinoma.

Diagram 63 137

The ulceration may be longitudinal, transverse, orcircumferential, and pseudopolyps may be evident.3

Many of the morphological and microscopic featuresresemble those of ischemic colitis.3,4 Boley et al havedemonstrated experimentally that as intraluminalpressure rises, blood flow in the mucosal and musclelayers of the bowel wall diminishes.5 The ischemiceffect induced by obstruction is regarded as themechanism of the colitis. The possible role ofintestinal flora is unclear.3 Teasdale and Mortensenhave reported 6 cases with acute features requiringemergency surgery for necrotic changes in the wallof the bowel.6

For a full-page image of this figure see theappendix.

HistoryThe patient was admitted to hospital with a re-spiratory infection which had been treated with erythromycin. The patient also suffered frombronchiectasis and asthma. Prednisolone andtrimethoprim were administered in view of poorlung function. Ten days after admission, there hadbeen no bowel action for 3 days. At the time of theinitial surgical consultation, the abdomen was dis-tended with absent bowel sounds. The patient wasafebrile with a pulse rate of 110 and a white cellcount of 57,400. During the next 4 days, thepatient’s bowel function varied from diarrhea to nobowel action or flatus in a 24 hour period. Abdomi-nal distention persisted. Radiological investigationrevealed a grossly dilated colon (Figure 64.1) withoutevidence of mechanical obstruction. The diagnosisremained obscure. On the fifth day of admission, the

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patient complained of severe abdominal pain in theright upper quadrant. The abdomen was distendedwith localized tenderness. A computerized tomog-raphy (CT) examination revealed a small collectionof free gas adjacent to the transverse colon.

Operation (8.10.94)Laparotomy revealed distended colon with areas onthe right colon and mid transverse colon showingnecrosis and imminent perforation. The precise siteof the free gas leak was not identified. The intra-operative diagnosis was ischemic colitis. A subtotalcolectomy with anastomosis and proximalileostomy was performed.

PathologyThe macroscopic appearance of the mucosa wasclassical of pseudomembranous colitis (PMC). Therewas extensive loss of mucosa of the left colonleaving “islands” of intensely inflamed mucosacovered with small foci of yellow pseudomembrane(Figure 64.2). Histologically, the suspect areas in the colon revealed full thickness necrosis. Thepseudomembrane showed numerous neutrophilsand gram positive organisms consistent with a diag-

64 Pseudomembranous Colitis and Toxic Megacolon

Female, 67 Years

138

Figure 64.1: A plain x-ray demonstrates the dilatation ofthe colon.

Figure 64.2: The pseudomembrane is superficial to thesubmucosa. Complete loss of mucosa has occurred.

nosis of PMC. Clostridium difficile was not identi-fied on culture.

ProgressRecovery was complicated but satisfactory after 4weeks. The ileostomy was closed 7 months after theresection.

CommentIn a review of the surgical aspects of clostridium dif-ficile colitis, Bradbury and Barrett emphasize therehas been a dramatic increase in the diagnosis ofClostridium difficile infection amongst surgicalpatients.1 This has been due to increased awareness,better methods of diagnosis, more widespread use ofantibiotics, and the increasing numbers of elderlyand immunocompromised patients.1 A wide range of antibiotics has been associated with PMC. Ery-thromycin and trimethoprim used in this patient are

Diagram 64 139

less commonly associated than other antibiotics. Inthe patient described here, the diagnosis was notrealized until the resected specimen was examinedimmediately after operation. The intraoperativediagnosis was ischemic colitis. Stool cultures didnot reveal Clostridium difficile, and stool assay fortoxin A was not performed. Longo states that thisassay is the most reliable test to establish the diag-nosis.2 The very high white cell count is character-istic of PMC, It was not appreciated prior tooperation that PMC could cause toxic megacolon(TM). Gan and Beck stress that TM can be causedby a wide variety of inflammatory conditions of the colon, which include various infections andischemic colitis.3 Prendergast et al report the inci-dence of TM in PMC to be 2%.4 Surgical treatmentis required in very few patients and, if necessary, isassociated with a reported mortality of 38%.5 If TMdevelops, the mortality rate can reach 80%.4

For a full-page image of this figure see the appendix.

HistoryIn 1986, the patient was suffering chronic abdomi-nal pain and was investigated in the Department ofSurgery at the Aga Khan University Karachi, wherea diagnosis of ileocecal Crohn’s disease was made.The patient lived in isolation 1000km from Karachi,and her family in Australia preferred that furthertreatment occur in Sydney. Investigations indicatedthe need for operation, which was performed(12.22.86). Right hemicolectomy with 75cm ofileum was performed for an inflammatory mass ofthe cecum, cecoileal fistula, and two small bowelstrictures. The histology was nonspecific chronicinflammation. Tests for tuberculosis were negative.Recovery was satisfactory and the patient returnedto Pakistan until 1990 when she presented withcolicky abdominal pain, diarrhea, and marked lossof weight. Endoscopic and x-ray investigations nowdemonstrated an enteroduodenal fistula and aninflammatory stricture at the ileocolic anastomosis.Extensive tests for tuberculosis (TB), performed

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in Pakistan, were negative. The patient returned to Australia for further surgical treatment. TheMantoux test was positive and there was a calcifiedfocus in the mid zone of the right lung. A 10-dayperiod of total parenteral nutritional (TPN) therapywas administered prior to operation.

Operation (5.27.93)There were extensive adhesions associated with alarge inflammatory mass involving the ileocolicanastomosis, adjacent ileum, and duodenum. Theterminal ileum was dilated due to chronic obstruc-tion. Firm enlarged lymph nodes were present in thesmall bowel mesentery and “knobbly” hard nodeswere adjacent to the transverse colon. The ileocolicanastomosis and 27cm of ileum were resected withreanastomosis. The small fistula defect in the duo-denum was repaired.

PathologyThe opened specimen revealed a smooth thickenedstricture at the site of the previous anastomosis.Proximal to the stricture was a fistula communi-cating with a loop of ileum. This fistula was also incontinuity with the ileoduodenal fistula. Immedi-ately proximal and distal to the stricture, themucosa was inflamed, atrophic, and featureless withplaques of fibrin exudate obscuring superficial ulcer-ation. These appearances were not typical of Crohn’sdisease. The histological examination revealed “nonspecific inflammation” and the diagnosis remaineduncertain. Some years later further tissue blockswere examined and the slides reviewed by five pathologists. The bowel wall and mesenteric lymphnodes now showed chronic transmural inflamma-tion, with fibrous ulceration and granulomas withLanghans giant cells (Figure 65.1). Some granulomaswere suppurative and others were caseous. Specialstaining (Zeihl-Neelsen, auramine rhodamine) failedto demonstrate any mycobacteria. Examination oflymph nodes showed granulomas with extensivecaseation. Three pathologists regarded tuberculosisas the most likely diagnosis.

65 Ileocecal Tuberculosis MimickingCrohn’s Disease or Vice Versa?

Female, 62 Years

140

Figure 65.1: Typical tuberculous caseation and fibrosiswith Langhans’ giant cells. These granulomas were presentin the wall of the small bowel and mesenteric lymphnodes.

Postoperative CourseThe patient’s general condition remained poor withdifficulty in establishing adequate oral intake.Upper gastrointestinal (GIT) bleeding and systemiccandidiasis complicated the patient’s weakenedstate. She succumbed 4 weeks after operation. Noautopsy was performed.

CommentThe diagnosis of TB was not made until after thepatient’s death when the slides were examined for a further opinion in an academic department ofpathology. No acid fast bacilli (AFB) were seen orobtained by culture from the tissue removed at both

Diagram 65 141

resections. Confirmation of intestinal tuberculosisis known to be difficult. Findlay et al report the pos-itivity of tests as follows: Mantoux 31%, chest x-ray29%, histology 56%, and tissue culture 27%.1

Where doubt exists, a therapeutic trial of antituber-cular treatment has been recommended,2 and suchtreatment would have benefited this patient. Theoriginal description of regional ileitis (later knownas Crohn’s disease) was based on the review ofpathology specimens at Mount Sinai Hospital, NewYork. The authors referred to ileocecal tuberculosisin the differential diagnosis and stated, “In all of our first cases of regional ileitis, the diagnosis of ileocecal tuberculosis was the unvarying best possibility.”3

12.22.86

5.27.93

P A R T

VII Lymphoma

HistoryThe patient had suffered from recurrent abdominalpain for 1 year. The pain, central and in the rightiliac fossa, had been worse for 1 week, requiringdaily attention from the family doctor. On admis-sion to the hospital, clinical examination revealed along mass lying transversely in the upper abdomen.A contrast enema confirmed the diagnosis of in-tussusception (Figure 66.1). The apex reached thesplenic flexure.

Operation (1.11.96)Laparotomy confirmed the extent of the intussus-ception, which was easily reduced. There were 4 dis-crete firm lesions palpable in the terminal ileum,the distal one of which was the apex of the intus-susception. The related small bowel mesentery contained enlarged lymph nodes. The bowel wasobviously viable. There were several hard nodules (2mm) over the surface of the right colon. A righthemicolectomy, including 70cm of ileum, was performed.

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PathologyThere were a total of nine mucosal lesions of the ileum, 3 of which were polypoidal and firm (15mm–30mm). The distal lesion and 1 other wereulcerated. There were 5 soft plaque-like abnormali-ties along the ileum (10mm–20mm). Sections of thepolypoid lesions revealed a pale surface, and one ofthese lesions extended through the full thickness of the intestinal wall. Histologically, the lesionsshowed atypical lymphocytic infiltration consistentwith a diagnosis of Burkitt’s Lymphoma (Figure66.2). The nodules (nodes) on the surface of the rightcolon and the mesenteric lymph nodes showed nosignificant abnormalities.

Progress and InvestigationsPostoperative recovery was satisfactory. Investiga-tion to stage the patient’s disease showed no otherfoci of lymphoma. The non-Hodgkin’s lymphoma(NHL) classification was Stage III (Murphy classifi-cation: childhood NHL).

66 Burkitt’s Lymphoma (Ileum) with Intussusception

Female, 10 Years

144

Figure 66.1: The barium enema shows theintussusception has reached the splenic flexure.

Figure 66.2: The small bowel lesion shows a diffuseinfiltrate of lymphoid cells with the “starry sky”appearance.

ChemotherapyThe patient was treated with 6 courses ofchemotherapeutic agents, which includedcyclophosphamide, vincristine, methotrexate, dox-orubicin, and cytarabine.

Follow-Up (2005)The patient remains well without evidence of recur-rence, 9 years since the resection.

CommentIn Western countries, Burkitt’s Lymphoma is spo-radic and accounts for 2% of all lymphomas. They

Diagram 66 145

usually present as abdominal tumors, as in this case,and may affect the jaw and peripheral lymph nodes,particularly in the neck. It is associated with theEpstein-Barr virus (EBV) in 20% of cases (Westerndisease). The histology shows diffuse changes withsheets of monomorphic neoplastic lymphoid cellsevenly interspersed with histiocytes, producing thecharacteristic “starry sky” pattern.1 The prognosiswithout treatment is very poor, howeverchemotherapy has been effective in inducing dra-matic and complete tumor regression, as in thispatient.

HistoryThe patient had noted the recent onset of abdomi-nal distention with 2 episodes of acute lower abdom-inal pain within 2 months. These symptoms wereaccompanied by a loss of weight. The stools hadbecome black in color over a period of 7 days. Exam-ination revealed a large nontender spherical massoccupying the right lower abdomen. A bariumenema demonstrated dilatation of the terminalileum with distortion of the mucosal pattern. Acomputerized tomography (CT) examinationrevealed a mass in the lower abdomen 10 × 10 ×8cm extending into the cecum and ascending colon.A collection of contrast appeared within the mass,suggesting a bowel perforation was present.Colonoscopy revealed a large polypoid mass in themedial aspect of the proximal ascending colon andcecum, which obscured the ileocecal valve.

Operation (12.4.93)A large confluent mass was confirmed in the ileo-cecal region. Its bulky nature suggested the pos-sibility of a lymphoma. The mass appeared toinfiltrate the ascending colon, terminal ileum, andmesentery. Enlarged lymph nodes were presentalong the ileocolic vessels. There was no otherpathology detected within the abdomen. A right

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hemicolectomy was performed, including removalof 50cm of terminal ileum.

PathologyThe mass “encased both the terminal ileum and theproximal right colon.” On section, it was pale incolor with a central cavity that communicated withthe terminal ileum in two places. The mucosa of theileum was otherwise normal. Within the mesentery,seven enlarged, firm lymph nodes were present. His-tological examination confirmed the diagnosis of lymphoma (Figure 67.1). The tumor was a non-Hodgkin’s large B cell type with a diffuse pattern. It was consistent with the so-called MALT lym-phoma (arising from mucosal associated lymphoidtissue). The mesenteric lymph nodes were notinvolved with lymphoma. In the cecum, a flat palepolyp was present measuring 50 × 50mm, which on examination was a dysplastic tubulovillousadenoma.

Follow-UpSix weeks after operation, a right-sided abdominalmass was present and a gallium scan confirmed 4foci of lymphoma within the abdomen. Over thenext 7 months, 5 courses of chemotherapy wereadministered (cyclophosphamide, mitozantrone,vinblastine). The response to treatment was satis-factory and the patient remained in remission until1997, when the disease recurred. At the age of 87years, no further treatment was advised. The recur-rent lymphoma was not the cause of death 4 years9 months after operation, when he died unexpect-edly during sleep.

CommentThis patient’s clinical presentation and tumor morphology were typical of small bowel lymphoma.Such lesions represent 20% of small bowel tumors1

and may be associated with Coeliac or Crohn’sdisease as well as immunodeficiency states. Pre-operative diagnosis is unusual, but at operation apale bulky tumor will suggest the diagnosis is lym-phoma. In this patient, the MALT B cell tumor waslow grade, which is usually associated with a morefavorable prognosis. Adjuvant chemotherapy is fre-quently associated with resection of the disease, par-ticularly if regional lymph nodes are involved.2

67 Ileocecal LymphomaMale, 82 Years

146

Figure 67.1: The left of the field shows 2 lymphoidfollicles with germinal centers. The lymphoma is on theright. Small bowel villi are seen in transverse section.

Diagram 67 147

HistoryThe patient had noticed a few weeks of minimaldark red bleeding from the rectum and was exam-ined by colonoscopy. There was diffuse low gradecolitis (Figure 68.1) affecting the colon with a lossof vascular pattern and diminution of the haustralindentation. In the lower third of the sigmoid colon(at 20cm), there was nodularity of the mucosa with associated hyperemia and small submucosalhemorrhages. Biopsy of this area revealed non-Hodgkin’s lymphoma (NHL) of a diffuse type.Immunochemistry was positive for the B cellmarker L26. Other biopsies of the mucosa showednonspecific chronic inflammation. Staging investi-gation by gallium and computerized tomography(CT) scans revealed no other foci of lymphoma. Surgical treatment was advised in preference tochemotherapy.

Operation (11.14.94)The small bowel and colon were normal in appear-ance, with some thickening apparent in the lowerthird of the sigmoid colon on palpation. Small firm

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lymph nodes were palpable in the mesentery of the sigmoid and descending colon. The spleen wasenlarged 40–50% of normal size. The extent of theresection was related to distribution of the palpable

68 Multiple Lymphoma and Ulcerative Colitis

Female, 49 Years

148

Figure 68.1: Endoscopy shows chronic inflammatorychanges in the mucosa of the ascending colon (1998).

Figure 68.3: Evidence of Colitis: surface ulceration anddistorted mucosal glands.

Figure 68.2: Diffuse large B cell lymphoma.

lymph nodes. The proximal level was the upperdescending colon, and the distal level was midrectum. The anastomosis was performed with a circular stapler.

PathologyIn the mucosa of the distal sigmoid, there were 5pale, firm nodules 3–5mm in size with surroundingcongestion and areas of mucosal hemorrhage. His-tologically, the nodules were malignant lymphomaof large cell B type (Figure 68.2). There were diffuseas well as follicular areas of lymphoma in the bowelwall. There was no evidence of lymphoma in any of11 lymph nodes examined. Macroscopic evidence ofcolitis was minimal, but microscopic examinationconfirmed the presence of colitis (Figure 68.3).

Follow-Up (2005)No chemotherapy was administered, since the local-ized disease appeared to be adequately removed byoperation. Periodic examinations by gallium and CTscans have shown no evidence of recurrent lym-phoma. The patient remains well more than 10years since operation except for occasional episodesof diarrhea, which last for a few days. Surveillancecolonoscopies with random biopsies have continuedand reveal no change in the endoscopic or macro-scopic appearances, which is consistent with adiffuse chronic nonspecific colitis of moderatedegree (Figure 68.2).

CommentHopefully this patient’s prognosis is favorable due tominimal localized lymphoma with no evidence ofrecurrence in over 10 years. It is unusual for gas-trointestinal lymphoma to be a small lesion at thetime of diagnosis. The obvious and numerous lymphnodes in the sigmoid and left colon mesentery deter-mined the proximal level of the resection but provednegative for lymphoma. Chronic colitis coexistingwith malignant lymphoma is rare and was firstreported by Bargen in 1928.1 Baker reported an inci-dence of 5 lymphomas in 2500 patients with chronic

Diagram 68 149

colitis.2 It is not yet established whether the associ-ation of lymphoma and chronic ulcerative colitis(CUC) is a definite risk factor or coincidence, butsome of the literature does support the former.3

Multifocal involvement is not uncommon in gas-trointestinal lymphoma, and the small multiplenodules were of interest in this patient. Wagonfeldet al have described a patient with CUC and 22 lym-phomas in the colon.4 The severity and duration ofthe colitis is variable in patients with coexistentlymphoma, and the patient described here has beenasymptomatic most of the time. Surgery remainsthe mainstay for colorectal lymphoma. If the lymphnodes are not involved, chemotherapy is usuallyomitted.

For a full-page image of this figure see theappendix.

HistoryThe patient presented with a history of bright bloodon the stool for only a few days. He otherwise feltwell. On rectal examination, a large firm polypoidlesion was palpable on the left anterior aspect of thelower third of the rectum 5cm from the anal verge.It was moveable on palpation. Colonoscopy revealedno other pathology in the colon.

Operation (10.28.99)The lesion was attached to the wall of the rectumby a broad pedicle, which could be readily con-stricted by closure of the diathermy snare. Thepolyp was removed with coagulation current (Figure69.1) as a total specimen. The underlying rectalmuscle remained intact.

PathologyThe polyp measured 25 × 22 × 12mm. It was palepink in color, with a lobulated surface. Histologi-cally, the lesion was covered by colonic mucosawith some inflammatory reaction. Beneath themucosa, there was a nodular mass infiltrating thesubmucosa composed of a “monotonous popula-tion” of intermediate to large sized lymphoid cells.Numerous mitotic figures and apoptotic cells were

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scattered throughout. Immunoperoxidase stainsidentified the lesion as a malignant non-Hodgkin’slymphoma of diffuse large B cell type (Figure 69.2).

Further Treatment/Follow-Up (2004)Investigations revealed no other foci of lymphoma.The patient was treated with 4 cycles of chemother-apy (cyclophosphamide, doxorubacin, vincristine,and prednisolone). The site of the lymphoma healed readily, and regular clinical, endoscopic, andcomputerized tomography (CT) examinations haveshown no evidence of recurrent disease 4 years 6months after removal of the rectal lesion.

CommentGastrointestinal lymphoma accounts for 2%–5% ofgastrointestinal tract (GIT) malignancy1 and is themost common extranodal site.2 Au et al report theincidence in the GIT as: stomach, 57%: small bowel,22%; large bowel, 7%; gallbladder, 2%; and multi-ple sites, 11%.1 The prognosis for control of thispatient’s disease is estimated to be in the vicinity of70%. The technique of diathermy snare with strongcoagulation current was used in this patient onlybecause the lesion was in the extraperitonealrectum.

69 Lymphoma of the RectumMale 63, Years

150

Figure 69.1: Endoscopic view of the defect afterdiathermy snare removal. Blood clot in the proximal partof the defect.

Figure 69.2: The lymphoma is apparent beneath themucosa.

Diagram 69 151

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VIII Anorectal Disease

HistoryThe patient had, for some years, been diagnosed as suffering from an irritable bowel. For a “fewmonths,” she had noticed rectal pain on defecationwhich had become severe and was aggravated by herbowel frequency. She was not aware of any swelling,but there was a sense of blockage during a bowelaction. On examination, there was a smooth,rounded, nontender mass on the left side of the analcanal, extending just above the anorectal junctionand bulging into the lumen beneath intact mucosa.It extended into the ischiorectal fossa and wasreadily palpable in the perianal region. Flexible sig-moidoscopy was normal to 30cm. Transrectal ultra-sound (TRUS) revealed a poorly echogenic massdeep to the mucosa within the sphincter muscle. Atthis examination, fine needle aspiration biopsy wasperformed. Histologically, this revealed a “smoothmuscle tumor” with no evidence of malignancy.

Operation (9.2.91)Under spinal anaesthetic and with the patient in theprone jack-knife position, a hemicircumferentialincision was made over the palpable mass on the left

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side of the anal verge. Dissection lateral to the exter-nal sphincter, “stretched” over the mass, identifiedthe inferior hemorrhoidal nerve. Incision of a thinrim of sphincter muscle revealed the surface of thetumor that was then dissected free from its locationentirely within the external sphincter musclewithout loss of muscle tissue. The space within themuscle was closed over a small soft rubber drain.

PathologyThe egg shaped tumor, with a well defined capsule(Figure 70.1), measured 50 × 38 × 35mm, and thesurface was smooth with some shallow lobulation.On section, it was pale yellow in color and homo-geneous in texture without hemorrhage or necrosis.Histologically, it was uniformly cellular, being com-posed of spindle cells exhibiting mild atypia with 4mitoses per 50 high-power fields (Figure 70.2). Thetumor was regarded as being “most likely benign.”Since the original pathology examination, im-munohistochemical staining with C117 has beenperformed, confirming that the lesion is a gastroin-testinal stromal tumor (GIST).

70 An Intrasphincteric Anal TumorFemale, 59 Years

154

Figure 70.1: Section shows a circumscribed tumor with awell defined capsule.

Figure 70.2: Higher power magnification shows a densefield of plump spindle cells, typical of a GIST.

Follow-Up (2005)There has been no recurrence of the tumor sinceoperation more than 13 years ago. Anal sphincterfunction is normal without any degree of inconti-nence. The patient is examined by panendoscopyand colonoscopy on a regular basis for a hiatushernia, gastric polyps, and symptomatic diverticulardisease.

CommentTumors of the anal sphincter are rare and stromaltumors the more so.1 Topographical tumors, morecommon in other sites, have been reported involv-ing the anal canal: spindle cell lipoma;2 neurofi-brous;3 and granular cell tumor.4 Other lesions thatmay clinically suggest a tumor in this region arechronic intersphincteric abscess, oleogranuloma,and endometrioma.5 The cloacogenic carcinoma

Diagram 70 155

may present as a subepithelial mass in the analcanal. Tan et al have managed a similar tumor to theone described here. Their tumor was initially locallyexcised and found to have a mitotic count of 4 per50 high-power fields.1 Subsequently, abdominoper-ineal excision of the rectum was performed, whichrevealed residual stromal tumor with a mitoticcount of 2 per 10 high-power fields. This emphasizesthe difficulty of assessing the malignant potential ofthese tumors. Precise preoperative imaging is essen-tial in planning the surgical approach to a tumorinvolving the anal sphincter, and currently eitherendoanal ultrasound or magnetic resonance imaging(MRI) with endorectal coil appear to be equally accu-rate.6 Local excision to provide a “total biopsy”would appear to be the first step in management. Ifthere is then doubt concerning malignancy, the areacan be readily monitored by the available imagingtechniques.

HistoryThe patient had been aware of a swelling in the (left)ischiorectal region for 2 years, which altered inprominence with posture, i.e., it appeared to be lessobvious when laying on his right side. On examina-tion, a soft cystic swelling was apparent, filling theleft ischiorectal fossa. Computerized tomography(CT) examination revealed the ischiorectal lesionwas in continuity with a large pelvic mass, mainlyleft-sided with clearly defined margins. There wasdisplacement of pelvic structures. The maximumdiameter was 75mm. It did not appear to be con-nected with the small or large bowel. Needle biopsywas nondiagnostic.

Operation (6.4.90)The surgical approach was abdomino-ischiorectal.The tumor was closely associated with the rectum,which it displaced from the left side. The areolartissue around the tumor was dense and fibrous. Thetumor was dumbbell shaped with an isthmus where

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it passed through the levator ani. Large vessels werepresent over its surface. The margins of the levatorani defect were rigid and difficult to dilate in orderto extract the tumor via the pelvis. The tumor wasremoved intact. Profuse venous bleeding was con-trolled with packs.

PathologyThe tumor size was 17 × 11cm. There was a thincapsule of fibrous tissue. The consistency was“doughy.” The cut surface was pale gray and softwith evidence of some fibrous tissue. Histologicalexamination revealed spindle cells in a loose myxoidstroma (Figure 71.1) with scattered mast cells. Mod-erate numbers of blood vessels were present. Therewas no evidence of malignancy. Diagnosis: aggres-sive angiomyxoma.

Follow-Up (2004)The patient has been free of recurrence for 14 years.For some years prior to operation, he had been trou-bled by intractable nausea, which was immediatelyrelieved by removal of the tumor.

CommentThis rare mesenchymal tumor, more common infemales, can be troublesome with local recurrence,but there is no tendency to metastasize.1 The tumorusually involves the pelvic soft tissues and oftenattains a large size,1 as occurred in this patient.Wilson reported an identical case, although it waslarger, measuring 45cm in length.2 The need forradical operation does not appear to be mandatorybut will depend on the operative findings. Nyam andPemberton report an angiomyxoma in a similaranatomical location but with invasion of therectum.3 The patient was treated with preoperativeembolization, external radiotherapy, intraoperativeradiotherapy, and abdominoperineal excision of therectum.

71 Aggressive Pelvic Angiomyxoma of the Pelvis

Male, 64 Years

156

Figure 71.1: Histological appearance shows a myxoidstroma and thin-walled vascular spaces.

Diagram 71 157

HistoryThe patient had been diagnosed with an anal fistula8 years previously. During this period, intermittentdischarge occurred in the left perianal region. Hecomplained of a further swelling in the area, incre-asing in size over a period of 3 weeks. There wereno bowel symptoms. There were 2 firm nodules inthe left ischiorectal fossa surrounded by inflamma-tion. Adenocarcinoma was confirmed, “erupting”through the perianal skin. There were enlarged hardlymph nodes in the left inguinal region. Sigmoi-doscopy and biopsy identified an ulcerating carci-noma of the sigmoid colon at 20cm.

Operation (8.7.63)An abdominoperineal resection of the primarytumor with en bloc resection of the lesion in theischiorectal fossa was performed. The large defect inthe buttock was left open to heal by secondaryintention.

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PathologyThe primary tumor was a well differentiated adeno-carcinoma confined to the muscular wall of thecolon. There was an adjacent polyp containing afocus of adenocarcinoma. The internal opening ofthe anal fistula was identified on the dentate linewith a track leading to the 2 nodules of adenocarci-noma. Free cancer cells were identified in the fistulatrack (Figure 72.1). Both sites of cancer were histo-logically similar. There were no metastases in themesenteric lymph nodes or in multiple sections ofthe bowel between the primary tumor and the analcanal.

Follow-Up (1976)The perineal wound healed in 10 weeks. Theinguinal lymphadenopathy subsided completely andno biopsy was performed. The patient remained freeof recurrence for a known follow up period of 13years and 3 months.

CommentDistal implantation by a proximal adenocarcinomainto an anal fistula may have been first described by Guiss.1 Implantation into other anal lesions, in-cluding hemorrhoidectomy wounds, has also beenreported.2,3 From a pathological viewpoint, a highanterior resection and radical excision of theischiorectal anal canal lesions would have been fea-sible in the patient reported here, as there was nointervening focus of malignancy. Using immuno-histochemical stains, Hyman and Kida identified a sigmoid carcinoma and a metastasis in an analfistula as the same malignancy.4 Although thisinvestigation was not performed in the patientdescribed here, the perianal lesion is regarded as animplantation metastasis, rather than a synchronousprimary tumor. Adenocarcinoma arising in a long-standing fistula is most frequently a colloid carcinoma.5

72 Implantation Metastasis into an Anal Fistula

Male, 63 Years

158

Figure 72.1: Free cancer cells are present in the proximalpart of the fistula track.

Diagram 72 159

HistoryThe patient, who suffered from severe cerebraldementia, was under supervision in a nursing homefor the aged. The nursing staff noted rectal prolapseof at least 8cm in length. An “ulcerated area” on theapex of the prolapse was biopsied and the diagnosisof adenocarcinoma confirmed. The attending gas-troenterologist referred the patient. Sigmoidoscopyafter reduction of the prolapse located the tumor on the posterior wall of the rectum at 10cm fromthe anal verge (there is no available record of acolonoscopy). Abdominal computerized tomogra-phy (CT) showed no evidence of major perirectalspread or metastatic disease.

Operation (5.20.83)With the rectum prolapsed, a “disc” local excisionwas performed (coagulation diathermy), including a >5mm margin of normal tissue. The excisionexposed the mesorectal fat, but the peritoneal cavitywas not exposed. The defect in the wall of therectum was closed with interrupted sutures.

PathologyThe tumor was a flat plaque 4.5cm in diameter,deeply penetrating the muscle wall but not extend-

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ing beyond it. Mesorectal fat, 15mm in thickness,was attached to the deep surface. Histological exam-ination revealed a well differentiated adenocarci-noma. The excision margins were negative forcarcinoma.

Follow-Up (1987)Postoperative healing of the rectal wall was satis-factory. No surgery was performed for the rectal pro-lapse, which did not reappear with any significantfrequency. The patient was last examined 4 years, 5 months after the local excision, at the age of 91years, when sigmoidoscopy was normal to 25cmand there was no evidence of recurrent carcinoma.

CommentThe rectal prolapse greatly facilitated the local exci-sion by presenting the tumor at the apex of the pro-lapse. Rectosigmoidectomy (Altemeier operation) at the same time as the local excision was deferredin the presence of the exposed malignancy. Subse-quently, the prolapse was not causing sufficientproblems to justify any surgery in a dementedpatient. It is quite likely that the rectal carcinomawas initiating the more frequent prolapse prior tolocal excision.

73 Local Excision of a Rectal CarcinomaCan Be an Easy Operation

Female, 86 Years

160

Diagram 73 161

HistoryThe patient complained of symptoms for 8 years,which were: rectal discomfort (“a lump”), frequentbowel actions, diarrhea (mucus), intermittent bleed-ing, and rectal pain. There was evidence of a chronicanxiety state and addiction to analgesics. Sigmoi-doscopy and examination under anaesthesiarevealed 2 hard areas of leukoplakia, a fibrous stric-ture of the mid anal canal, and squamous metapla-sia immediately above the dentate line. The mucosaabove this area was hyperemic, redundant, and poly-poid to a level of 8cm, above which the mucosa wasnormal. Straining revealed marked internal rectalprolapse. Biopsy of the hyperemic mucosa showedhistological changes “consistent with Morson’s solitary rectal ulcer” (Figure 74.1).

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Operation (1.30.79)Ripstein rectopexy was performed.

Follow-Up (1995)The rectal symptoms were relieved and most of themacroscopic features of the rectal mucosa resolvedpermantly. The leukoplakia was unchanged. Twomonths after operation, bowel function was satis-factory, but 6 years later, the patient was complain-ing of prolonged periods of constipation, abdominalpain, and distention. Investigations revealed amarkedly redundant colon. Colon transit studieswere not performed. On 8/26/85, abdominal colec-tomy was performed for intractable constipation.During the period 1989–1991, several operationswere required to cure a complicated anal fistula. Thepatient continued to complain of chronic abdominalpain and remained addicted to analgesics.

CommentThe morphology and histology were consistent with the diagnosis of proctitis cystica profunda. Anassociation with rectal prolapse of 54% has beenreported by Stuart.1 In this patient, complete rectalprolapse may have been “held back” by the analcanal stricture. There was a satisfactory response ofthe patient’s ongoing symptoms to rectopexy, whichunfortunately was later complicated by severe con-stipation requiring further operation. The morpho-logical and histological appearances may causeconfusion with carcinoma of the rectum, particu-larly the presence of displaced, distorted mucoussecreting glands within the submucosa. Nagasako etal. report such a case treated by abdominoperinealexcision of the rectum.2 Valenzuela et al. report 2patients in whom computerized tomography (CT)and magnetic resonance imaging (MRI) demon-strated the disease limited in depth to the submu-cosal layer. Both patients were treated with a high fiber diet, bulk laxatives, and reeducation to avoid straining. Resolution of the pathologyoccurred in both patients with this conservativetreatment.3

74 Proctitis Cystica ProfundaFemale, 26 Years

162

Figure 74.1: Thick extensions of the muscularis mucosaare seen radiating between the mucosal glands towardsthe ulcerated surface.

Diagram 74 163

Examination under anaesthesia11.28.78

HistoryThe patient presented in 1985 with a long history ofa sense of blockage in the rectum that resulted inprolonged defecation. There had been minor rectalbleeding and mucous discharge for 1 year. There wasno awareness of any prolapse. Rectal examinationrevealed a firm stricture in the mid rectum, con-firmed at 8cm by sigmoidoscopy. The lumen at thispoint was approximately 13mm in diameter. Therewere leukoplakia-like projections on the “rim” ofthe stenosis and 5 areas of shallow ulceration at thislevel. The base of the ulcers was yellow-white, firm,and surrounded by erythematous mucosa. Abovethis level to 15cm, the mucosa appeared normal. A barium enema demonstrated the abnormality(Figure 75.1). The radiologist stated the appearanceswere not exclusive of a neoplasm.

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BiopsyBiopsy of the lesion showed distortion of themucosal glands, chronic inflammation, submucosalfibrosis, and squamous epithelium. These findingsalong with the sigmoidoscopic appearances wereconsistent with the diagnosis of solitary rectal ulcersyndrome (SRUS).

Operation (7.29.85)At laparotomy, there was no apparent abnormalityof the rectum or colon. There was no palpable thick-ening of the rectum at the level of the stenosis.There was a deep rectoprostatic “pouch of Douglas”and virtual absence of normal pararectal fascia(“lateral ligaments”). The rectum was mobilized tothe pelvic floor and anterior rectopexy performedusing a 4cm wide band of monofilament poly-propylene (Ripstein operation).

75 Rectopexy for a Rectal Stricture-UlcerMale, 38 Years

164

Figure 75.1: The barium enema on 5/14/85(preoperative) shows an annular irregular stenosis of therectum with “shouldering.” Below this level, the rectumwas in spasm.

Figure 75.2: Barium enema on 8/19/86 (15 months afteroperation) shows a persistent area of narrowing. Therectum below this appears normal.

Follow-Up (1990)Four weeks after operation, the patient reported a significant improvement in bowel function. Thesense of obstruction was greatly diminished. Fre-quency of defecation was reduced, and the stoolswere now of normal diameter. Rectal examinationrevealed the stenosis had improved and was nowsoft and supple. The clinical improvement wasmaintained and, 13 months after operation, thestenosis was almost impalpable and not apparent onsigmoidoscopy. The ulceration had healed, leavingsome erythema and persistent leukoplakia. Abarium enema 15 months after operation showedpersistent narrowing, which was surprising in viewof the clinical and sigmoidoscopic findings (Figure75.2). When the patient was last examined 5 yearsafter the operation he was asymptomatic andwithout recurrence of the rectal ulceration.

CommentThe clinical, endoscopic, and pathological featuresof this condition were defined by the seminal pub-lication of Madigan and Morson in 1969.1 The ulcer-ation is more common in the incomplete (internalintussusception) variety of rectal prolapse.2 Thispatient’s rectal ulcers were classical SRUS in appear-ance, although circumferential ulceration and asso-ciated stricture are unusual. The stricture is relatedto the marked submucosal fibrosis that occurs inassociation with the ulceration. There was no rectalintussusception detected endoscopically (proctogra-phy was not performed). Rectopexy for SRUS hasbeen shown to have a low success rate when notaccompanied by complete rectal prolapse,1 althoughNicholls and Simson have reported symptomaticsuccess in 12 out of 14 patients.3 In the presence ofdisabling symptoms and no alternative operation,rectopexy in this patient was undertaken as a spec-ulative procedure. It was considered that rectalintussusception may have been an etiological factorbut obscured by the development of stenosis. Theresponse to rectopexy was successful and lasting.The persistence of the narrowing radiologically isinteresting in view of the near-normal appearanceon sigmoidoscopic examination. In the author’sseries of 216 Ripstein rectopexies (complete pro-

Diagram 75 165

lapse, 180; incomplete prolapse, 36), there was onlyone other patient with SRUS and stenosis. Thispatient also responded well to rectopexy. The ter-minology and classification of the rectal prolapsesyndrome, rectal intussusception, solitary rectalulcer syndrome, and proctitis cystica profunda needsan international consensus.

HistoryIn June 1977, an anal fistula was treated by opera-tion, and, although healing occurred, the rectumwas “abnormal”. The radiological demonstration ofa rectal stricture suggested the possibility of Crohn’sdisease (Figure 76.1). The patient was referred forfurther management. Examination under anaesthe-sia revealed marked rigidity of the anorectal junc-tion and the rectum with a long stricture of therectum, most marked between 10cm and 13cm. Aninternal opening was identified on the left lateralaspect of the dentate line with an ascending trackpassing anteriorly. A focus of granulation tissue at10cm in the midline anteriorly was identified as a probable secondary internal opening. The rectalmucosa was pale and edematous. Biopsies at variouslevels showed nonspecific inflammation.

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Operation (12.6.77)Peranal exploration enabled only part of the fistulatrack to be laid open into the lumen. Access to theupper limit of the track was prevented by the rectalstricture.

Operation (2.21.78)Laparotomy revealed an extraperitoneal mass on the left side of the rectum but no other intra-abdominal abnormality. A loop ileostomy was performed.

Operation (6.8.78)A posterior transsphincteric approach was used withincision of the posterior wall of the rectum to the12cm level. Access was still difficult due to intensefibrosis of the rectum, and strong retraction wasrequired to facilitate laying open the track into the lumen. Sutures approximated each edge of thetrack to the divided wall of the rectum to ensurehemostasis and prevent premature healing (“marsupialization”).

Operation (9.12.78)The ileostomy was closed after examinationrevealed healing of the fistula was complete.

Follow-Up (1980)Bowel function was satisfactory after 4 months.Examination under anaesthesia (28 months after thefistulotomy) revealed sound healing of the rectumwith resolution of the stricture.

CommentThis “upside down” fistula caused significant diffi-culty in diagnosis until both internal openings wereidentified. The Parks classification of anal fistulaeincludes the upward extension of the intersphinc-teric fistula,1 but the high level of the track and sec-ondary internal opening in this case was unusual.The transsphincteric operation provided the onlyaccess possible for complete fistulotomy. The reso-lution of the stricture and most of the perirectalinduration had occurred within 2 months of the fistulotomy.

76 Intersphincteric Anal Fistula withProximal Perirectal Extension

Male, 34 Years

166

Figure 76.1: The barium enema examination shows astricture of the lower rectum. Crohn’s disease wasconsidered as the likely diagnosis until the fistula openingswere identified.

Diagram 76 167

Anterior

Posterior aspect

1 Fistula track (epithelized)2 Perirectal fibrosis3 Rectal muscle4 Mucosa-submucosa

12

34

HistoryOn 5.1.90, endoscopic removal of a sessile rectalpolyp (15 × 15 × 3mm) was performed. It was situ-ated posteriorly in the lower third of the rectum.The histology report noted severe dysplasia (“atleast carcinoma in situ”) with no evidence of invasive carcinoma. Within a few days, an abscessappeared in the left ischiorectal fossa, which wasdrained. A fecal fistula was apparent subsequently.The patient was referred.

Operation (5.31.90)Examination revealed a large area of necrosis in theleft posterior wall of the rectum with underminingof the mucosa. This was in continuity with a largesupralevator fistula. The internal opening easilyadmitted an examining digit. Laparotomy, antero-grade colon irrigation, and loop ileostomy were performed.

ProgressThe fistula healed and the ileostomy was closed(11.12.90). Follow up examinations revealed satis-factory healing until 2 years after the polyp excision.Examination now revealed a small, firm polypoidlesion at the site of the previous internal opening of the fistula. Biopsy confirmed the diagnosis ofmoderately differentiated adenocarcinoma. Com-puterized tomography (CT) examination revealed noevidence of metastatic disease. The anal canal wallwas thickened.

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Operation (7.13.92)Abdominoperineal excision of the rectum was per-formed. There were no intraabdominal metastases.

PathologyThere was a raised ulcer at the site of the excisedpolyp that was in continuity with a small mass (8 ×8mm) in the left parasphincteric area. Histologicalexamination revealed the mass was adenocarcinomaextending into fatty tissue. The mesenteric lymphnodes showed no evidence of carcinoma.

Follow-Up (2004)The patient remained well until August 1996, whena 2cm lung metastasis in the right upper lobe (RUL)was detected upon routine chest x-ray. A right upperlobectomy was performed. No further recurrentdisease has been detected 11 years and 7 monthssince rectal excision was performed.

CommentHistological review of the initial polyp removed in1990 did not reveal evidence of invasive carcinoma,and yet this relatively small “benign” polyp wasultimately responsible for a rectal excision and aright lobectomy. Necrotizing infection after polypexcision is rare. The healing of the supralevatorfistula after a defunctioning ileostomy was encour-aging, but direct spread or implantation of carci-noma cells supervened. Local re-excision wasconsidered to be inappropriate.

77 Necrotizing Infection After Removalof “Benign” Rectal Polyp

Female, 64 Years

168

Diagram 77 169

5.31.90

8.28.96

7.13.92

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IX Various Pathology

HistoryDuring the first year of her life, this patient was treatedfor bilateral congenital dislocation of the hips. At 26years (1956), a caesarian section was performed, asthere was “a pelvic lump present the size of a tennisball.” In 1975, x-rays revealed a large soft-tissue massin the pelvis, causing a lytic lesion in the sacrum. Thiswas thought to be a chondroma. In 1977, a trans-abdominal biopsy was performed and the diagnosis ofdesmoid tumour confirmed. On clinical examination,a firm lobulated mass filled the lower half of theabdomen to the level of the umbilicus. Rectal andvaginal examination revealed a hard pelvic masscausing marked compression of the vagina and rectum.A computerized tomography (CT) scan demonstratedconsiderable destruction of the sacrum. The tumorsize was “12 × 12 × 15cm.” Sigmoidoscopy was notpossible. A barium enema revealed sigmoid diverticu-losis. At a colorectal conference (1981), the unanimousopinion was that the lesion was inoperable.

TreatmentOral delta testolactone was commenced in 1978 andcontinued for 32 months. Oral sulindac was admin-istered for 19 months (1984–1985). Intra-aterial per-fusion chemotherapy with doxorubicin, vincristine,

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78 Intra-Abdominal Desmoid TumorUnassociated with FamilialAdenomatous Polyposis

Female, 74 Years

172

Figure 78.1: CT scan shows the maximum transverse andantero-posterior dimension. (4.24.84).

Figure 78.2: CT scan demonstrates the erosion of sacrumand sacral canal. (4.24.84).

Figure 78.3: CT scan reveals no increase in size in 18years. Calcification and areas of hypodensity are apparent.(9.30.99).

5 Fluorouracil, and carmustine was performed in1986. None of these treatments appeared objectivelyto reduce the size of the tumor (Figures 78-1 and 78-2).

Follow-Up (2004)Clinical and CT examination have not demon-strated any further progression of the tumor since1981 (Figure 78-3). On CT examination, the tumordimensions are 12 × 13 × 15.5cm. Small areas of

calcification have appeared, as have stable areas of hypodensity interpreted as cystic or necroticchanges. The colon and bladder are markedly dis-placed cranially and to the right. The stretcheddome of the bladder is at the L4/5 disc level justabove the upper surface of the tumor. A left hipreplacement was performed in 2004. The patient’sgeneral health is good for her 74 years.

CommentThis tumor, present for 55 years is not an exampleof a familial adenomatous polyposis (FAP) relateddesmoid. It is better classified as pelvic fibromato-sis which occurs in young females (20–35 years)unrelated to pregnancy. Such slow growing, locallyaggressive tumors appear to arise from muscle oraponeurosis in the pelvis. The absence of smallbowel obstruction suggests it has not primarilyinvolved the bowel mesentery, so common indesmoids complicating FAP. The tumor growth hasnot progressed since 1981. This could be sponta-neous regression known to occur in 7% of desmoids2

or due to 1 of the 3 nonsurgical therapies adminis-tered. The adaptation of pelvic viscera to thismassive pelvic tumor is surprising, as there are nosignificant bladder or large bowel symptoms. Inaddition, the involvement of the sacral canal has not become symptomatic. Considering the risks ofattempted resection, [St Mark’s Hospital, London,reports an operative mortality of 8 out of 22(36.4%)3] the nonoperative treatment of this patientappears to have been validated.

Diagram 78 173

X: Bladder

X

For a full-page image of this figure see theappendix.

HistoryFor several years, the patient had been troubled withdiarrhea (6 bowel movements in 24 hours). Thestools often contained small flecks of red blood andmucus. Initially on sigmoidoscopy, the patient wasthought to have a polypoid carcinoma at the 20cmlevel. A further opinion confirmed the diagnosis ofpneumatosis coli. Sigmoidoscopy revealed tensesubmucosal cysts between 20cm and 25cm, mostly5mm in diameter, but also clusters of smaller lesions throughout the mucosa. Many of the cystswere “capped” with hyperemia. There were alsosmall patches of similar hyperemia without mucosalprojections. Biopsy was difficult, because the smoothsurface and firm consistency of the cysts hinderedclosure of the forceps. The histology was consistentwith the diagnosis. Colonoscopy was not performed.A barium enema revealed typical appearances ofpneumatosis in the descending and sigmoid colon(Figure 79.1).

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Treatment (9.19.75)Over a period of 4 days, the patient was given oxygen therapy via a mask at the rate of 14L perminute.

Follow-Up (1987)A barium enema examination 6 days after cessationof oxygen therapy showed no evidence of the previ-ous radiological abnormalities (Figure 79.2). Sigmoi-doscopy subsequently was normal to 25cm. Thepatient’s symptoms were improved within days ofcompleting the oxygen treatment, and his bowelhabit was no longer a social disability. The patientwas last examined 12 years after treatment. Hisgeneral health was good, bowel actions occurredtwice per day, varying in consistency. Flexible sig-moidoscopy to 70cm showed small pale areas cor-responding to the previously involved colon. Therewas no recurrence of the pneumatosis.

79 Pneumatosis ColiMale, 53 Years

174

Figure 79.1: Barium enema shows typical appearance ofpneumatosis coli in the sigmoid colon.

Figure 79.2: Barium enema 6 days after completion ofoxygen therapy demonstrates resolution of thepneumatosis.

CommentThe etiology of pneumatosis is unknown, but insome cases it is associated with respiratory, gas-trointestinal or psychiatric comorbidity.1 In thepatient described, the disease appeared to be idio-pathic, which is usually associated with involve-ment of the left colon.2 Oxygen therapy, first

Diagram 79 175

advocated by Forgacs et al,3 was successful long-term in this patient after 1 treatment. It is notunusual for oxygen therapy to be required for morethan a single treatment. Complications of pneu-matosis occur in 3% of patients (volvulus, necrotiz-ing enterocolitis, rupture of cysts) and may requiresurgical treatment.2

HistoryIn 1987, the patient, aged 52 years, was initiallyinvestigated for rectal bleeding and a family historyof colorectal cancer. Her general health status wasfrail, with chronic respiratory disease, severe kypho-sis, osteoporosis, recurring vertebral fractures,obesity, and a Cushinoid state induced by years ofsteroid therapy administered for severe generalizedeczema. There was a long history of constipation.Due to respiratory insufficiency and muscle weak-ness, the patient’s ambulatory capacity was limitedto a few steps. On January 24, 1997, the patient wasadmitted to the hospital with severe abdominal painand clinical signs of peritonitis.

Operation (1.24.97)Laparotomy revealed a colon grossly loaded withfeces that were “lumped” in firm-to-soft masses,rather than scybala, which was oozing into the peritoneal cavity through a 4mm perforation of theanterior wall of the colon at the sigmoid descendingcolon junction. This area of the colon showedprominent inflammatory changes of increased vas-cularity and fibrinous exudate. Fecal peritonitis waspresent in the lower abdomen. Immediately mobi-lization of the left colon was commenced, the colon

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ruptured, and significant fecal contaminationoccurred. When this difficult situation was con-trolled, a Hartmann resection was performed. Thedistal sigmoid was oversewn.

PathologyThere was a large ulcer involving the mucosalsurface of the resected colon, revealing the inflamedand partly necrotic muscle layer. The perforationwas in the distal area of the ulceration. The histo-logical changes were nonspecific, consistent withulceration and necrosis (Figure 80.1).

Postoperative CourseThe patient remained in the intensive care unituntil she died of respiratory failure 21 days afteroperation. She remained dependent on respiratoryventilation during this time, until the patient andfamily requested that this therapy (managed via atracheotomy) be withdrawn.

CommentStercoral perforation is rare, and, adopting strict criteria, Maurer et al have found 81 cases in the literature.1 These criteria are:

1. sharply demarcated round or ovoid perforation;2. larger than 1cm (85%);3. antimesenteric in position;4. associated fecalomas; and5. necrosis and/or ulceration associated with the

perforation (74%).

This patient’s morphology exhibited 3 of these fea-tures. The long history of constipation and the fecalloading found at operation is also consistent withthe diagnosis. Immune suppressed patients are vul-nerable to this complication.2 The tissues of thispatient, affected by high, long-term doses of pred-nisolone, were extremely fragile, and this undoubt-edly contributed to the complication. There was noevidence that the pathology was primarily ischemic.The histological features (Figure 80.1) are similar tothose described by Haddah et al.3 Colon irrigationprior to bowel mobilization may have prevented theoperative fecal contamination, but this maneuver

80 Stercoral Ulceration: Sigmoid Perforation

Female, 62 Years

176

Figure 80.1: The stercoral ulcer shows complete loss ofmucosa and nonspecific inflammation in the submucosa.

would have been difficult (long-dilated and fragilecolon, obesity, high flexures, and the patient notpostured in the lithotomy Trendelenberg position).Two senior anesthetists administered the anestheticbecause of the anticipated problem of respiratoryfunction. The choice of operation in this condi-tion is significantly influenced by the patient’scomorbidities, which are often multiple. They arefrequently frail, nursing home residents or institu-

Diagram 80 177

tionalized patients.3 This patient’s anesthetic statusfor any future elective surgery was regarded as pro-hibitive, and the Hartmann stoma would have beenpermanent if the patient had survived. Stercoral perforation is association with a high postoperativemortality. Serpell and Nicholls, in reviewing 64cases, recommended resection without anastomo-sis; even so, there was still a high postoperative mortality of 32%.2

Splenicflexure

Anterior wallulcer-perforation

HistoryTwo weeks prior to referral, the patient suffered asudden episode of colicky abdominal pain accompa-nied by diarrhea that lasted 3 days. On the third day,the patient passed a small amount of dark red blood.A barium enema 1 week after the onset of symptomsshowed an area of narrowing at the splenic flexure(Figure 81.1). The mucosal pattern was distorted,and small areas of “thumb printing” were justvisible. A colonoscopy subsequently revealedsigmoid diverticular disease and a marked stenosisat the splenic flexure associated with ulceration.Biopsy showed nonspecific inflammatory changes.A limited contrast enema was performed 5 weeksafter the onset of symptoms (Figure 81.2). The steno-sis measured 10cm in length, which the radiologistregarded as consistent with a constricting carci-noma. This diagnosis was not accepted. The patientremained asymptomatic, however a third contrastenema, now 6 months after the acute episode,

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81 Nongangrenous Ischemic ColitisFemale, 71 Years

178

Figure 81.1: Barium enema at 1 week shows earlymucosal changes and narrowed lumen at the splenicflexure.

Figure 81.2: Contrast enema at 5 weeks reveals a longstricture with no mucosal pattern.

Figure 81.3: Contrast enema at 6 months demonstratesa short persistent stricture.

showed further narrowing of the lumen, which radi-ologically measured 2mm in diameter (Figure 81.3).

Operation (9.15.94)At laparotomy, the short stricture of the splenicflexure was confirmed. The mesenteric vesselsappeared normal. Sigmoid diverticular disease,without inflammatory changes, was present. Resec-tion with anastomosis was performed.

PathologyInflammatory changes were present on the serosalaspect of the stricture. The muscle wall was thick-ened (8mm) and white due to fibrosis. The stricturewas associated with mucosal ulceration, which wasshallow and well defined. The adjacent mucosa wassmooth, slightly congested, and featureless. Histo-logically, nonspecific inflammatory changes werepresent in the floor of the ulcer and bowel wall.There was no evidence of vascular occlusion or vascular disease. The findings were consistent withischemic ulceration.

Follow-Up (1996)The patient’s progress was satisfactory with nofurther gastrointestinal symptoms. Colonoscopy 15months after operation showed no abnormalityother than sigmoid diverticulosis.

CommentNongangrenous ischemic colitis is the morecommon form of this vascular disease of the colon.1,2

Post ischemic stricturing frequently occurs and inthis patient was developing 1 week after the onsetof symptoms. At 5 weeks, the radiological opinionsuggested the diagnosis was carcinoma. This wasexcluded by colonoscopy and biopsy. The strictur-ing had persisted at 6 months, and operation wastherefore advised despite the patient’s lack of symp-toms. Brown, in a review of 17 patients, found thatthe stricture was often well tolerated and that therewas significant resolution in patients, thus avoiding

Diagram 81 179

operation.2 No histological evidence of vascularocclusion was found, which is not unusual in spec-imens resected some time after the acute event.Nonocclusive ischemia due to inadequate perfusionof the bowel wall was unlikely in this patient, asthere were no acute comorbid conditions presentusually associated with this form of ischemicdisease.3

For a full-page image of this figure see theappendix.

HistoryIn 1984, the patient underwent a high anterior resection for symptomatic diverticular disease. OnJuly 4, 1996, laparotomy was performed to exclude asigmoid carcinoma in view of an abnormality oncolonoscopy and computerized tomography (CT)examinations. No lesion was identified in thesigmoid colon, but extensive adhesions were foundinvolving the small bowel, omentum, and the ante-rior abdominal wall. Due to the trauma of a difficult dissection, a small length of ileum wasresected. Three weeks after the operation, a severebout of abdominal pain occurred followed by persis-tent pain in the left iliac fossa (LIF). A firm mass waspalpable in the LIF. A CT examination confirmed thepresence of the mass anteriorly with a “fatty centralcomponent” (Figure 82.1). The pain continued andbecame worse in January 1997, when it was associ-ated with a fever. The patient was treated in the hos-pital with intravenous antibiotics. When this acuteepisode subsided, the patient continued to suffer sig-nificant pain. On referral, laparotomy was advised.

Operation (3.3.97)An inflammatory, omental mass was found in theLIF, intimately fused with the small bowel, making

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dissection hazardous. (The adhesions are minimizedin the diagram on page 181 for clarity). Incision intothe mass revealed inflamed adipose tissue with acentral necrotic area, which was curetted anddrained. Excision of the mass was not undertaken.

PathologyThe necrotic material was identified as fat necrosis(Figure 82.2).

Follow-UpRespite from the abdominal pain was only tempo-rary, and within 3 months the patient required nar-cotics for pain relief. The abdominal mass persistedclinically and radiologically (CT).

Operation (7.14.97)Extensive dense adhesions were again encounteredbetween the fibrofatty mass, small bowel, and theleft lateral and anterior parietes. The mass, measur-ing 10.5 × 45 × 40cm, was excised. Anatomically, itwas isolated from the stomach and colon by loopsof small bowel. There were no significant bloodvessels attached to it.

82 Infarction of the OmentumFemale, 63 Years

180

Figure 82.1: CT examination (10.10.96) shows the left-sided abdominal mass (arrow) prior to laparotomy on3.3.97.

Figure 82.2: Tissue from the abdominal mass (3.3.97)showing fat necrosis. Fat vacuoles are surrounded byinflammation containing histiocytes and foreign body giantcells.

PathologyIncision into the resected mass revealed yellowadipose tissue with no residual abscess. Microscopicexamination revealed areas of foreign body reactionwith numerous foamy macrophages and intracellu-lar and extracellular fat vacuoles. The appearanceswere consistent with organizing fat necrosis.

Follow-Up (2004)The operation relieved the severe pain, but subse-quently, bouts of abdominal pain have recurredwithout a clear diagnosis. In 2004, relief is obtainedwith nonnarcotic analgesics and short courses ofantibiotics prescribed by her family physician.

Diagram 82 181

CommentAs a result of a difficult abdominal dissection(7.4.96) it seems most likely that acute omentalinfarction occurred 3 weeks later. This resulted inseparation and sequestration of omental tissue,which was sustained by the vascular inflam-matory tissue surrounding it, forming a virtual “parasitic” fibrofatty mass within the abdomen. Theexcision (7.14.97) did resolve the constant severeabdominal pain, but intermittent symptoms havecontinued. The patient has suffered various abdom-inal pains for 29 years.

Leftparietes

Dissectiontraumasmallbowel

Focus offat necrosis

3.3.97

HistoryIn October 1996, the patient was admitted to thehospital with acute abdominal pain in the epigas-trium and right iliac fossa. Investigations were nonspecific, until a barium enema demonstratednarrowing of the ascending and transverse colon.Colonoscopy was not performed. Six years previ-ously, the patient had a lobular carcinoma of theright breast treated by mastectomy.

Operation (10.18.96)Elective laparotomy was performed, which revealedextensive carcinomatosis of the large and smallbowel, pelvic organs, omentum, and parietal peri-toneum. The colon was thickened by infiltrationthat produced the typical “leather bottle” consis-tency. There were 2 predominantly affected areas inthe colon that corresponded to the strictured areasshown on the barium enema. The terminal ileumwas distorted by kinking due to malignant adhe-sions, with its serosa also markedly affected by the

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peritoneal nodules of carcinoma. A side:side anasto-mosis was performed between the ileum andsigmoid colon. The length of small bowel proximalto this anastomosis was 280cm.

PathologyBiopsies of peritoneal nodules revealed metastaticadenocarcinoma similar to that seen in the primarybreast carcinoma removed in 1990 (Figure 83.1).

Follow-Up (1997)Recovery from operation was uneventful. An oncologist who treated her with tamoxifen under-took her further management. The response to this treatment was minimal. The patient died of her disseminated malignancy the following year.

CommentMetastatic linitis plastica of the intestinal visceradue to breast carcinoma is rare. Graham et alreported 7 cases (9.4%) of 75 patients examined atlaparotomy or autopsy.1 The stomach or the colonwere the viscera most often affected. The onset ofmetastatic disease may be delayed for years and mayremain confined to the abdomen. It may be thatlobular carcinoma has a tendency to metastasize inthis fashion.2 The external appearance of the coloncan be confused with chronic inflammatory boweldisease. The intact mucosa, without ulceration, mayhave a “cobblestone” appearance. This diffusemalignancy affects all layers of the bowel wall. Biopsies of the mucosa or serosal tissue will usuallyidentify the origin of the metastatic disease. Dif-ferentiation between a primary bowel cancer and ametastasizing breast cancer can be achieved by theuse of immunohistochemical staining to identifyprimary gastrointestinal cancer (cytokeratin posi-tive) and lobular carcinoma of the breast (estrogen receptor positive).3

83 Metastatic Linitis Plastica of the Colon

Female, 78 Years

182

Figure 83.1: Histological appearance of metastatic colontumor showing cancer cells in “Indian file” typical oflobular breast cancer.

Diagram 83 183

HistoryThe patient had noticed colicky abdominal pain for3 weeks. There had been fresh blood on the stool on3 occasions. A barium enema demonstrated a largepolypoidal mass in the mid transverse colon (Figure84.1). Immediately proximal to the mass there wasmucosal irregularity and narrowing of the lumen.Computerized tomography (CT) examination con-firmed the lesion and excluded other pathology. Acolonoscopy was obstructed in the mid transversecolon by what appeared to be a fungating carcinoma.No biopsy was performed.

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Operation (1.16.98)A large firm mass was confirmed with no externalabnormality of the transverse colon. The lesion wasregarded as malignant. An extended right hemi-colectomy was performed with anastomosis.

PathologyThe lesion was recognizable as a submucosal lipomamostly covered by mucosa that had dehisced at theapex and was associated with ischemic necrosisimpregnated with barium. It measured 75 × 45mmwith a broad (20mm) pedicle. There was ulcerationand inflammation in the immediately adjacentmucosa.

CommentLipomas of the large bowel are usually asympto-matic until, over a long period, they become largeenough to cause obstruction and/or bleeding.1

The morphology of this lesion suggests that somedegree of intussusception was occurring, producingischemic necrosis at the apex. These changes wereinterpreted as carcinoma at colonoscopy examina-tion. Confusion with carcinoma is likely only whensecondary changes occur to alter the typical smoothsurface of a lipoma in the colon. Small lipomas areusually asymptomatic, but lipomas larger than 3.5cm may become symptomatic2 and may requireremoval—most likely by a segmental resection ofthe bowel or local excision via a colotomy. Colono-scopic removal of a lipoma 50mm in size, has beenreported.3

84 Lipoma Transverse ColonMale, 65 Years

184

Figure 84.1: The barium enema demonstrates a largelesion in the transverse colon (arrows).

Diagram 84 185

HistoryThe diagnosis of endometriosis had been confirmedsome years previously and been treated by ovariancystectomy and laparoscopic pelvic surgery. In 1987,to investigate chronic lower abdominal pain and con-stipation, flexible sigmoidoscopy to the splenic flex-ure revealed dome shaped swellings (Figure 85.1),most obvious in the sigmoid colon, covered withnormal mucosa, consistent with endometriomas. A gynecologist supervised a further 5 years of medi-cal treatment until the symptoms were sufficientlydisabling to require surgery. Repeat endoscopyshowed no extension of the colon lesions, which didnot show on a barium enema examination.

Operation (6.4.92)Laparotomy revealed extensive pelvic endometriosiswith widespread adhesions involving ovaries, anenlarged uterus, and upper rectum. There were hardspherical masses involving the sigmoid and lowerdescending colon causing rotation and constriction,and similar pathology was noted in a short segmentof terminal ileum. Hysterectomy with excision ofuterine adnexa was performed. The disease in theileum and colon was removed with a double resec-tion, and the 2 anastomoses were performed with asingle layer of interrupted sutures.

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PathologyThe left ovary was cystic, 45 × 45mm in size anddensely adherent to the uterus. The right ovary wasalso cystic, 60 × 40mm in size, and containing oldblood. Both ovaries had smaller cysts associatedwith the larger cystic lesion. There were fibroidswithin the body of the uterus and hemorrhagiccysts, 1–5mm in size within the myometrium. Themasses in the ileum and colon were firm andinvolved the full thickness of the bowel wall. Theyvaried in diameter from 7–30mm. The serosalsurface was indented and the luminal aspect wascovered with normal mucosa. The lesions, onsection, had a predominantly white appearance with areas of black pigment and small areas of hemorrhage. Histologically, endometriosis was con-firmed in the uterus, ovaries, ileum, and colon(Figure 85.2).

Postoperative CourseThe patient’s recovery in the hospital was satisfac-tory and uneventful. Unfortunately, 3 weeks afterthe operation, the patient suddenly collapsed athome and died. Autopsy revealed that a massive pul-monary embolus was the cause of death.

85 Intestinal EndometriosisFemale, 36 Years

186

Figure 85.1: The “dome” shaped intramural deposit iscovered with intact mucosa.

Figure 85.2: A focus of endometriosis is seen within themuscularis propria of the colon.

CommentThis patient illustrates a number of features ofintestinal endometriosis, which is reported to occurin 3–18% of patients with endometriosis.1 Thesigmoid and the rectum are most commonlyinvolved (72%).2 Endoscopy and barium studies mayfail to demonstrate endometriosis, even thoughdome shaped lesions protrude into the lumen. Inmore extensive disease, transmural fibrosis maycause sufficient deformity to simulate carcinoma.Diagnostic laparoscopy, endorectal ultrasound, com-puterized tomography (CT) and magnetic resonanceimaging (MRI) may more accurately confirm thediagnosis and document the extent of the disease.The long-term results of hormone treatment are dis-appointing, and, once chronic symptoms of infiltra-tive intestinal endometriosis are present, surgicaltreatment is the preferred option. There is a spec-trum of surgical treatment available which is deter-

Diagram 85 187

mined by the distribution of lesions, the depth,length, and circumferential extent of bowel involve-ment, and whether future childbearing is hoped for.Woods et al have reported disc excision of the ante-rior wall of the rectum using the circular stapler.3

Jatan et al have reviewed the surgical treatment of95 patients of whom 80% were managed by variouslaparoscopic procedures, which were: diathermyablation, shave excision, disc excision, and low ante-rior resection.4 Concomitant procedures on otherpelvic structures were necessary in 71%. Prior tooperation, pain on defecation during menses andprevious laparoscopy were predictors that moreextensive surgery would be required.4 The surgicaltreatment can be complex and is ideally managed bya combined colorectal and gynecological team. Thepostoperative death in the young patient withbenign disease, reported here, was a salutaryreminder of the risks of complex pelvic surgery.

For a full-page image of this figure see the appendix.

HistoryIn 1932, when the patient was a few months old,Hirschsprung’s disease was diagnosed. Details of themethod of diagnosis are unknown. As a child, con-stipation was a significant problem, and in 1942, atage 10, bilateral lumber sympathectomy was per-formed with some relief of symptoms. In adult life,the constipation continued to be a significant dis-ability despite treatment, which included regularself-administered enemas. Periods of absolute con-stipation for 2 weeks occurred frequently. A bariumenema (Figure 86.1) showed a small-caliber rectumwith marked dilatation of the sigmoid colon com-mencing at the recto sigmoid junction, consistentwith Hirschsprung’s disease. Clinical examinationand sigmoidoscopy (15cm) were normal.

Operation (8.8.80)Biopsy of the anorectal region revealed absence ofganglia in the myenteric plexus.

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Operation (11.25.80)Laparotomy revealed gross enlargement of thesigmoid colon, which was 15cm in diameter withmarked prominence of the longitudinal muscle

86 Hirschsprung’s DiseaseFemale, 47 Years

188

Figure 86.1: Barium enema demonstrates the dilatedcolon and contracted rectum.

Figure 86.2: In the intermuscular myenteric plexus (lowpower), nerves were identified, (arrows) but ganglion cellswere absent. (This section was close to distal level ofresection.)

Figure 86.3: Normal ganglion cells in the myentericplexus (arrows) (proximal resected bowel).

bundles. The dilatation was relatively abrupt at thelevel of the sacral promontory. The caliber of therectum was smaller than normal. The inferiormesenteric vessels were markedly enlarged. Bowelpreparation had successfully removed the fecalcontent of the colon. An extended (ultralow) ante-rior resection (LARx) was performed with a circularstapler and a loop colostomy made in the transversecolon. At the proximal level of resection in thelower descending colon, the presence of ganglia wasconfirmed on frozen section.

PathologyThe macroscopic features were a thickened bowelwall and less prominent mucosal folds in a megasig-moid colon. Histological examination revealed anabsence of ganglion cells in the distal end of thespecimen (Figure 86.2), whereas in the mid and prox-imal sigmoid colon, a normal population of ganglioncells was present (Figure 86.3).

Follow-Up (2005)One year after operation, bowel function was 1/24hrs. The anastomosis level was 5cm. The patientwas not seen for routine follow up examinationsafter this time. Currently, 25 years since operation,the patient reports that at, 73 years of age, her healthis satisfactory. While her bowel habit is irregularwith occasional constipation, the result of the oper-ation is satisfactory.

Diagram 86 189

CommentHirschsprung’s disease presenting in adult life israre, and such patients are usually young adults.McCready et al reported 50 patients over 10 years ofage treated by surgery at the Mayo Clinic over aperiod of 28 years.1 The barium enema in the patientreported here was diagnostic (Figure 86.1) but is notalways typical of the disease.2 The x-ray may indi-cate the likely proximal limit of the aganglionosis.3

Anal manometry was not available for this patient.The LARx operation, with a long period of followup, performed in this 47-year-old patient has beensuccessful. Undoubtedly, there remains a small cuffof aganglionic rectum, which has not affected theresult. Elliot and Todd reviewed the results of 39patients from St Mark’s Hospital, London, treated bythe Duhamel operation with excellent results.2

Gordon has suggested the Duhamel operation isfacilitated by the use of the circular stapler for the colorectal anastomosis and the linear stapler to divide the intervening spur.4 Wheatley et al.reviewed 199 operations for adult Hirschsprung’sdisease and concluded that low anterior resection,Duhamel–Martin and Soave operations were themost acceptable methods of treatment.5

Harald Hirschsprung, 1830–1916 (courtesy of Prof. O. Kronborg).

For a full-page image of this figure see theappendix.

HistoryThe patient was admitted to the hospital with ashort history of abdominal pain, distention andabsolute constipation for some days. Clinical andradiological examinations confirmed the diagnosisof acute large bowel obstruction.

Operation (6.2.86)The colon was markedly distended due to a hardmass impacted in the lower sigmoid colon. Therewas a seromuscular tear over this mass and inflam-matory changes in the wall of the colon. There wasa large inflammatory mass involving the hepaticflexure of the colon, gallbladder, liver, duodenum,and pancreas. Exploration revealed a thickened gall-bladder containing a solitary large gallstone with 1facet surface. There was a large perforation 3cm indiameter between the gallbladder and the lumen ofthe colon (Figure 87.1). The stone and the distal halfof the gallbladder were removed. Long-term suctiondrains were placed in its lumen. Abdominal colec-tomy and a high ileorectal anastomosis were per-formed, leaving 18cm of rectum.

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PathologyThe stone in the sigmoid colon was tightly wedgedin the lumen, causing complete obstruction (Figure87.2). There was longitudinal and transverse ulcera-tion of the mucosa of the distal colon for a distance of15cm. Histological examination of the ulceration,colon fistula, and gallbladder showed nonspecificinflammatory changes. The smooth, dark green gall-stones were similar in size and shape to a hen’s egg.

Follow-UpPostoperative recovery was satisfactory. Twomonths after operation, the drain site from the gall-bladder remnant had closed. Four months after oper-ation, bowel function was satisfactory (2/24hrs).The patient was referred to a hepatobiliary surgeon for follow up but failed to reattend after the initialvisit.

CommentThe triad of gallstone ileus : bowel obstruction, airin the biliary tree, and an unusually sited gallstonewere not observed in this patient. In the less urgent,

87 Gallstone Obstruction: Sigmoid Colon

Male, 64 Years

190

Figure 87.1: The defect in the hepatic flexure of thecolon (cholecystocolic fistula).

Figure 87.2: Incision in the resected specimen exposes alarge gallstone at the point of impaction in the sigmoidcolon.

clinical circumstance, barium enema and endo-scopic retrograde cholangiopancreatography havebeen successful radiological investigations.1 Thesigmoid colon is the usual site of impaction, partic-ularly in the presence of sigmoid diverticulardisease. The passage of the stone into the colon ismore commonly via a cholecystoduodenal fistula.2

Diagram 87 191

Colotomy, extraction of the obstructing calculus,and proximal stoma may be the optimal treatmentif the findings at laparotomy indicate this is appro-priate.3 This patient was treated by resection ratherthan colotomy–extraction of the stone. Safe repairof the colotomy in the grossly inflamed sigmoidcolon was doubtful.

For a full-page image of this figure see the appendix.

HistoryFor his age, the patient had been very active untilhe developed colicky pain in the right iliac fossaassociated with loose stools and minor bleeding.These symptoms had been present for 3 weeks. Hehad lost weight and was found to be anemic (irondeficiency). On examination of the abdomen, therewas no abnormality. Ultrasound examinationrevealed an abdominal mass in the mid abdomenclose to the anterior abdominal wall (no descriptionof the mass available). Colonoscopy revealed a largevascular polypoid tumor filling the lumen “in themid transverse colon” (biopsy showed benignvillous adenoma). At the time of the colonoscopy,under sedation, a mass was palpable in the right iliacfossa.

Operation (7.29.92)The colon, from cecum to the splenic flexure, wasthickened and edematous, with marked vascularreaction on its serosal surface. It was initiallythought to be Crohn’s disease until the diagnosiswas realized. There was a large soft polyp occupyingthe cecum, and the right colon was significantlymobile. There was marked diverticulosis of the leftcolon. Most of the small bowel was normal inappearance, but the terminal 10–15cm of the ileumalso had a significant vascularity on the serosalaspect. A subtotal colectomy was performed with anend-to-end anastomosis in the mid sigmoid colon.

PathologyThe flat polyp occupying the cecum measured 60 ×60mm and was deeply hyperemic and soft in con-sistency. The mucosa of the colon was otherwisenormal. Histologically, the polyp contained multi-ple foci of early invasive, moderately differentiatedadenocarcinoma arising in a dysplastic villousadenoma. There were no metastases in the lymph

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nodes (Dukes A, T1 N0 M0). There was a fibrous reac-tion on the serosa and in the subserosal tissues ofthe proximal colon.

Follow-UpRecovery from operation was satisfactory. In view ofthe patient’s age, routine follow-up visits were notadvised and no further clinical details are available.

CommentThis patient’s cecal tumor was responsible for inter-mittent or chronic intussusception, which is oftenthe case in adult intussusception.1 Its spontaneousreduction caused some initial diagnostic confusionat laparotomy. The diagnosis was supported by theidentification of the polyp in the transverse colon atthe time of the colonoscopy and the observation ofa transient abdominal mass. Adult colonic intus-susception is rare: Nagorney et al report 24 cases in23 years at the Mayo Clinic.2 The lead point in thecolon is likely to be a malignant tumor, and Azarand Berger report an incidence of 43%.3 Preoperativediagnosis is infrequent3 and can be best achievedwith abdominal computerized tomography (CT) orultrasound when a target or doughnut sign may beelicited in the transverse view, or a “sausage”-shaped mass or “pseudokidney” may be present inthe longitudinal view.1,3,4,5 If colonic intussusceptionis encountered at laparotomy, resection, includingadequate mesentery, is recommended rather thaninitial reduction, since malignancy can rarely beexcluded and peritoneal contamination with malig-nant cells could occur on reduction.1,3,4,5 If thesurgeon is unsure that recurrent intussusception hasbeen occurring, the pathologist may identify anumber of histological features in the resected spec-imen that confirm the diagnosis.6

88 Intussusception of the ColonMale, 90 Years

192

Diagram 88 193

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X Complications of Investigation and Treatment

HistoryA barium enema was performed to investigate rectalbleeding (10.2.86).

The barium was injected using a “hand pump”technique, continued despite the patient experi-encing severe and increasing pain in the rectum. The patient was unable to evacuate the barium. In hospital, sigmoidoscopy revealed marked edema ofthe lower half of the rectum. Urgent laparotomyrevealed no intraabdominal pathology. A left-sidedloop colostomy was performed. Two months later,after referral, sigmoidoscopy examination underanesthetic revealed an indurated strictured rectumwith intense inflammation of the mucosa and submucosal barium “blebs.” A traumatic ulcer waspresent immediately above the anal canal posteri-orly. Plain x-rays showed persistence of a large massof barium in the pelvis. These clinical and radiolog-ical findings showed no resolution over the next 17months of follow up (Figure 89.1).

Operation (3.31.88)Resection of the rectum, coloanal anastomosis, andloop ileostomy were performed. Total excision ofthe internal sphincter and barium deposits on the

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89 Barium Perforation of the RectumMale, 58 Years

196

Figure 89.1: Plain x-ray of the pelvis 17 months afterperforation shows persistent presence of barium.

Figure 89.2: Transverse section of the resected specimenshows extensive infiltration of tissues by barium.

Figure 89.3: An x-ray of resected rectum and fragmentsof perirectal tissue demonstrates the presence of thebarium 17 months after the barium enema.

levator ani were included in the resection. Intensefibrosis was present. There was barium infiltrationof all layers of the rectum and perirectal tissues(Figures 89.2 and 89.3).

Follow-Up (1996)The ileostomy was closed 3 months after resection.Subsequently, bowel function was attenuated butacceptable. There was no evidence of sexual orurinary dysfunction.

Diagram 89 197

CommentThe incidence of this complication has beenreported as high as 0.2%.1 The barium infiltrated alllayers of the lower rectum, including the analsphincter. Examination of the resected specimenillustrates why no resolution was possible after aperiod of conservative management. The mortalityof barium perforation of the rectum has beenreported as high as 60%2 and 100%.3 Survivingpatients frequently require a permanent stoma.

HistoryThe patient complained of altered bowel habit andrectal bleeding for 6 weeks. He suffered from signi-ficant ischemic heart disease. Clinical examinationrevealed a palpable carcinoma on the left side of the rectum at 5cm, occupying 50% of the lumen.There was some loss of mobility of the tumor.Colonoscopy was performed to the cecum. The find-ings were sigmoid diverticulitis and small hyper-plastic polyps. No specific difficulty with theexamination was recorded. The patient did not com-plain of any abdominal symptoms subsequent tocolonoscopy.

Operation (8.4.89)Elective laparotomy to resect the rectal cancer wasperformed 7 days after the colonoscopy. There wasan extensive hematoma of the anterior ascendingcolon, 9cm in length, disrupting the muscle layersof the bowel wall and associated with free blood

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(approximately 500ml) in the peritoneal cavity. Aright hemicolectomy was performed. Unusualfragility of the mesenteric blood vessels was noted.There were small metastases in the liver related tothe rectal tumor, which was removed by anextended (ultra low) anterior resection without aproximal stoma.

PostoperativeThe patient’s postoperative course was complicatedby several episodes of cardiac arrest that respondedpromptly to resuscitation management. On the 11thday of his recovery, a fecal fistula appeared and thiswas managed by laparotomy, colon irrigation, andloop ileostomy. Further episodes of cardiac arrestdue to his unstable cardiac condition kept him inthe hospital for 12 weeks. The patient suffered afurther cardiac arrest 5 weeks after discharge, fromwhich he did not recover.

CommentThis complication is very rare following diagnosticcolonoscopy, and only one other case has been foundin the literature.1 That patient, reported by Gallo et al, suffered the complication after a difficultcolonoscopy without any other procedures such asbiopsy. Computer tomography (CT) examinationwas helpful in diagnosis and in monitoring progress,which continued without the necessity of operation.Trauma to the colon induced by diagnostic colono-scopy is usually located in the sigmoid colon, andinjuries less than perforation consist of single ormultiple areas of muscle dehiscence (Figure 90.1).The colonoscopist reported that the examination ofthe patient described here, was not a difficult pro-cedure. The double resection and the anastomoticleak requiring reoperation undoubtedly contributedto the patient’s unstable cardiac state, which ulti-mately caused his death.

90 Colonoscopy Injury to the ColonMale, 67 Years

198

Figure 90.1: Post colonoscopy appearance of sigmoidcolon (different patient) showing multiple areas of muscledehiscence.

Diagram 90 199

HistoryThe patient was investigated for iron deficiencyanemia. Colonoscopy revealed a large rectal polypand a carcinoma of the transverse colon. The ascend-ing colon was not viewed due to the malignantstenosis. The patient’s comorbidity included dia-betes, asthma, chronic obstructive airways disease,and coronary artery disease. He was declared unsuit-able for surgical treatment of his coronary disease.

Operation (7.3.98)A right hemicolectomy was performed.

PathologyThe resected specimen contained an ulcerating car-cinoma of the transverse colon (Dukes B, T3 N0M0). In the ascending colon, there were 3 polyps.The largest, measuring 30 × 30mm was a benigntubulovillous adenoma. The polyp next to theappendix measured 15 × 15mm and contained afocus of invasive carcinoma (Dukes A, T1 N0 M0)in a tubulovillous adenoma.

ProgressThe patient’s recovery was satisfactory until the 9thpostoperative day, when he complained of severeabdominal pain associated with vomiting. Therewas generalized abdominal tenderness and a largemass, the size of a rugby football, palpable in thecentral abdomen.

Operation (7.12.98)Extensive mesenteric venous thrombosis waspresent which had caused infarction of the distalhalf of the small bowel. Resection of the ileum (230cm) and ileocolic anastomosis were performedwith an anastomosis of jejunum to the transversecolon. There was 215cm of small bowel remaining.

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The patient’s postoperative recovery was, at times,uncertain but eventually satisfactory.

Operation (1.14.99)Diathermy snare of the large rectal polyp. It was situated 14cm from the anal margin and filled the lumen at that level. Histological examinationrevealed a villous adenoma with no evidence ofmalignancy.

Follow-UpThe patient’s cardio-respiratory diseases intensifiedfrequently, requiring repeated admissions to hospi-tal. This caused his death 27 months after his righthemicolectomy.

CommentIntestinal infarction occurring after elective colonresection for cancer is rare.1 In a subset of theauthor’s series, the incidence was 3 in 1418 (0.3%),2

which is less than the 4.8% incidence reported aftercolectomy for inflammatory bowel disease (IBD).3 Inaddition to the surgery, the presence of cancer, dia-betes, and severe cardiovascular disease would havebeen etiological factors of significance. The patient’scoagulation profile was normal. The need for urgentreoperation was obvious, and an investigation suchas computerized tomography (CT) was not per-formed. The venous thrombosis was situated in thedistal and mid mesenteric veins. The superiormesenteric vein did not contain thrombus. Thepatient’s bowel function subsequently was 2–3times per day, which was surprising in view of thecomplete loss of the ileum. Mesenteric vascularocclusion in patients over 70 years of age carries ahigh mortality rate, and in the series of 74 patientsreported by Wadman et al, 28 out of 40 (70%) diedwithin 30 days of the event.1

91 Mesenteric Thrombosis After Colon Resection

Male, 73 Years

200

Diagram 91 201

7.12.987.3.98

7.12.98

HistoryInvestigation of lower abdominal pain and rectalbleeding revealed a carcinoma of the sigmoid colonat 30cm. Apart from well controlled hypertension,the patient was in reasonable health.

Operation (2.20.98)At laparotomy, the tumor appeared localized with no evidence of metastases. Significant diverticulardisease was present in the sigmoid and descendingcolon. A left hemicolectomy including upper rectumwas performed (Figure 92.1).

PathologyThe ulcerating carcinoma was 35 × 35mm in sizeoccupying two-thirds of the lumen. It was a well dif-ferentiated lesion that penetrated deeply into themuscularis propria. There were no metastases in the6 lymph nodes examined (Dukes A, T2 N0 M0).

Postoperative CourseThe patient’s initial recovery was satisfactory until48 hrs after operation, when he suddenly developeda tender, distended abdomen, pallor, and hypoten-sion. He was stabilized with resuscitation and trans-ferred to the operative theater.

Operation (2.22.98)Laparotomy revealed the presence of a profuse hemorrhage filling the abdomen with over 4000mlof blood. The site of bleeding was identified in theupper part of the lesser omentum, where a smallartery was still actively bleeding. The lesseromentum and anterior wall of the stomach (sub-peritoneal) were extensively suffused with blood.The site of the previous colorectal anastomosis wasunaffected. The bleeding vessel was ligated.

Postoperative Course/Follow-Up (2004)Further recovery was uneventful. The patientremains free of recurrent disease 6 years, 7 monthsafter the bowel resection.

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CommentThe incidence of intraabdominal bleeding requiringemergency return to the operative theatre is un-common. In a series of 1418 elective resection-anastomosis for cancer, 4 patients required reopera-tion for bleeding from the surgical site, and 1 patient(reported here) for bleeding remote from the opera-tion area.1 Abdominal apoplexy in older patients hasbeen associated with hypertension and atheroscle-rosis.2 The most common arteries to be the sourceof bleeding are the middle colic, left gastric, splenic,and superior mesenteric.2 The bleeding vessel is notalways identifiable, and in such circumstances, amorbidity of 56% has been reported3 Tan advisesthat the bleeding vessel is likely to be located at thesite of maximum hematoma.4

92 Postoperative Abdominal ApoplexyMale, 74 Years

202

Figure 92.1: Showing the extent of the resection.2.20.98.

Diagram 92 203

HistoryThe patient had noticed dark red rectal bleeding for 5months accompanied by frequent urge to defecate.Her previous health included mastectomy 51 yearspreviously and stable congestive cardiac failure asso-ciated with aortic incompetence. She had borne 12children and now had 54 grandchildren! She wassprightly physically, but early signs of dementia werepresent. Examination revealed a carcinoma on theposterior wall of the rectum 6cm from the anal vergeoccupying 40% of the lumen. Above the lesion, nopathology was detected on colonoscopy. A compu-terized tomography (CT) examination showed noevidence of perirectal extension or metastaticdisease. A sphincter-saving resection was planned,and the patient was treated with preoperative radio-therapy (37.5Gy in 15 fractions over 3 weeks).

Operation (10.7.93)Reexamination of the tumor 4 weeks after comple-tion of radiotherapy revealed increased mobility anda significant decrease in size of the lesion. Withunderlying concern about the impact of majorsurgery on the 87-year-old patient, transanal localdisc excision (LE) with diathermy was performedinstead of the planned resection. The operation wasperformed 4 weeks after radiotherapy treatment(RT) was completed.

PathologyThe tumor was a flat plaque, 35mm in diameter.Histologically, it was a moderately well differenti-ated adenocarcinoma. Its depth occupied the fullthickness of the muscular layer but did not extendbeyond it (T2). The excision margins were clear by3mm.

Postoperative Course/Follow-UpThe immediate postoperative recovery wasuneventful. After leaving the hospital, the patientsuffered constant rectal pain requiring readmission

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to hospital. Examination under anesthesia, 5 weeksafter the operation, revealed a large necrotic ulcer ofthe posterior wall of the rectum and upper anal canal70mm in length. There was florid granulation tissuepresent, partly covered by fibrin and slough. Biop-sies showed no evidence of carcinoma. There wereoccasional bizarre cells consistent with radiother-apy effect. The rectal pain continued over a periodof 11 months. Healing of the ulcer occurred withoutspecific treatment in 12 months. The referring gas-troenterologist in a distant rural area performedfurther supervision. The patient succumbed to livermetastases 18 months after the local excision. Therewas no clinical evidence of local recurrence.

CommentDue to a “last minute” change of treatment plan onthe morning of an intended sphincter-saving resec-tion, the patient’s sequence of treatment modalitieswas different from the author’s usual practice. Forthose patients in whom adjuvant radiotherapy hasbeen planned, it has been administered postopera-tively when the local excision site has healed. Theextensive rectal ulceration is a rare complication ofLE and adjuvant RT and may be related to thesequence of the treatment and the method of RTadministration. Recent reports of local excision ofrectal cancer following preoperative radiotherapy(with doses of RT up to 52.5Gy) do not documentthis complication.1,2 It is now established that, inview of the high risk of local recurrence, T2tumours3 are not suitable for local excision unlessthe operation is a compromise in a frail, high-riskpatient. In the case of this 87-year-old patient withearly dementia, it would have been better manage-ment to select local excision therapy originally.Although the radiotherapy followed by local surgerycontrolled the pelvic disease, its complicationcaused 11 of her remaining 18 months of life to beassociated with rectal pain.

93 Local Excision of Rectal Cancer and Radiotherapy

Female, 87 Years

204

Diagram 93 205

10.7.93

5 weekspost excision

HistoryThe patient suffered from asthma, an enormousgoiter, obesity (weight, 252lb), and severe kyphosisof the thoracic spine. In March 1993, a bariumenema demonstrated an “apple core” deformity thatcould not be reached by colonoscopy examination.

Operation (May 1993)Laparotomy revealed a complex mass involving thesigmoid colon and ovary. A limited resection andanastomosis of the proximal sigmoid and excisionof the left ovary was performed in the belief that theoperation was a palliative procedure for an ovariancancer. Pathology examination revealed diverticulardisease and no evidence of carcinoma of the colonor ovary. The patient remained unwell with signs ofsepsis.

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Operation (November 1993)Laparotomy was performed in November 1993 todrain a left subphrenic abscess. The colon wasdivided above a persistent sigmoid mass, and theproximal end of the colon was established as an end colostomy. A further contrast enema (February1994) demonstrated extensive diverticular diseaseand a leak from the colon entering a retroperitonealabscess, which reached the diaphragm. The patientwas referred for further management.

Operation (6.16.94)Total thyroidectomy was undertaken to obviate any airway difficulties during the anticipated majorabdominal surgery. The thyroid was benign andweighed 560g.

Operation (8.1.94)A ureteric catheter was inserted on the left side.Laparotomy revealed dense pelvic adhesions in rela-tion to a mass formed by pathology in the distalsigmoid colon, on the surface of which diverticulawere obvious. There was a chronic thick-walledabscess in relation to the mass, adherent to the left pelvic brim and related vessels. The abscessinvolved the intraperitoneal rectum, uterus, andbladder, and extended into the extraperitonealtissues adjacent to the lower rectum. By intubation,a long retroperitoneal extension of the abscess wasidentified posteromedial to the left kidney andextending as far as the diaphragm (Figure 94.1). Thesigmoid colon was resected to a level immediatelydistal to the pelvic brim. The abscess in the pelviswas debrided. The unopened cranial extension wasirrigated. The colostomy was taken down, the colonwas irrigated clear of fecal content via an ileotomy,and a colorectal anastomosis performed. Theileotomy site was converted to a loop ileostomy.Long-term suction drains were placed in the regionof the pelvic abscess and the long cranial extension.During the procedure, it was not possible to visual-ize the left ureter.

94 Residual Diverticulitis AfterResection Causing an ElongatedAbscess with Prolongated Resolution

Female, 63 Years

206

Figure 94.1: Sinogram showing the vertical extent of theretroperitoneal abscess.

PathologyExamination of the resected colon revealed diver-ticular disease with stricture formation. Within thestricture, 2 large perforations were present that hadpreviously been in continuity with the complexabscess. The lumen contained pus, the mucosa wasinflamed.

Operation (12.2.94)The ileostomy was closed 4 months after the resection.

Follow-UpThe long retroperitoneal abscess track healed veryslowly. Irrigation was complemented by antibiotic

Diagram 94 207

therapy. The drainage tube was in situ for a periodof 9 months.

CommentThis case highlights the difficulty that can confrontthe surgeon if preoperative colonoscopy cannotassess the pathology. The surgeon was misled toperform a “palliative debulking procedure” whichleft perforated the colon in situ. The case also illus-trates the tracking potential of a diverticularabscess. The long postoperative period of intubationirrigation was tedious but maintained the patient’shealth and prevented further surgical treatment.The preoperative preparation included thyroidec-tomy, which is somewhat unusual.

For a full-page image of this figure see the appendix.

HistoryIn November 1971, this patient suffered a sigmoidcolon perforation due to diverticulitis. His generalhealth was very poor due to respiratory insufficiency(heavy smoker), poor nutrition, and homelessness.At laparotomy, fecal peritonitis was present,managed by abdominal irrigation, drainage of theperforation site, and a proximal colostomy. Thepostoperative course was stormy. A colocutaneousfistula appeared, and radiological investigationsdiagnosed a colovesical fistula. The patient wasreferred.

Operation (4.20.72)At laparotomy, the site of origin of the colocuta-neous fistula was identified as the recent perforationin the upper third of the sigmoid colon. Thecolovesical fistula arose from the mid third of the sigmoid colon and entered the bladder via thetrigone. The margins of the defect in the bladder (10× 15mm) were densely fibrotic, indicating a long-standing fistula. Closure of this large defect was possible only with more than optimal tension on the sutures. The sigmoid and upper rectum wasresected, and a 2-layer anastomosis performed.There was insufficient omentum to place betweenthe bladder repair and the anastomosis.

Postoperative ProgressAlthough the patient’s clinical course appeared sat-isfactory, contrast radiological studies 21 days afteroperation demonstrated a subclinical anastomoticleak that was in continuity with the lumen of thebladder.

Operation (11.1.73)An extended low anterior resection was undertakento eradicate the persistent anastomotico-vesicalfistula (Figures 95.2 and 95.3). A chronic abscess was situated between the rectum and the bladder.The inflammatory changes in the bladder wall had obscured the fistula track, so no repair wasattempted. Prior to the anastomosis, the circulationof the small rectal segment was noted to be suspect.The proximal colostomy was maintained.

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Early Postoperative ProgressAfter a difficult operation, it was surprising thatrecovery appeared to be uncomplicated. Assessmentof the anastomosis, however (14 days after opera-tion), revealed complete separation of the colon andrectum. There was purulent discharge per rectum asthe only manifestation of this serious complication.In the absence of any clinical evidence of peritonealinfection, it was decided that no further surgicalintervention was indicated. It was anticipated thatthe transverse colostomy would be permanent.

Further ProgressDuring the next 20 months, the pelvic spacebetween the 2 ends of the bowel healed, and as thisoccurred, the colon and rectum became approxi-mated with an intervening stricture (Figures 95.1and 95.5). There appeared to be an adequate lumenfor bowel function. The transverse colostomy wasclosed 20 months after the resection.

95 Perforated Diverticulitis and Its Consequences

Male, 59 Years

208

Figure 95.1: The x-ray (6.20.75) demonstrates the bowelcontinuity, with a stricture, which formed spontaneously,subsequent to anastomotic dehiscence in 1973.

Follow-Up (1976)Bowel function was variable in frequency but pre-ferred to a stoma 12 months after colostomy closure.Further follow-up was not available as he returnedto his homeless existence.

CommentThe first series describing colovesical fistulae (CVF)was published by Harrison Cripps in 1888.1 Hool etal found an incidence of CVF in 5% of patientsrequiring surgery for diverticulitis.2 In the author’sseries of 206 elective resections for diverticulardisease, there were 23 (11.2%) patients with CVF.3

The trigone of the bladder is an unusual site for CVF,unless the fistula track is extraperitoneal.4 This loca-tion and size of the defect created technical difficul-ties, which lead to failure of the closure. Although inmany patients with a CVF repair of the bladder

Diagram 95 209

defect is not required,5 the size of the fistula in thebladder wall made attempted closure obligatory inthis patient. The second rectal anastomosis was afailure, almost certainly due to a vascular problemwith the short rectal stump. A coloanal anastomosiswould have been a better choice of operation. If aHartmann operation had been performed, it isunlikely it could have been reversed due to thepatient’s chronic comorbidity. Remarkably, in the presence of dehiscence the sepsis remained localized, draining per rectum, and without systemiceffects. The spontaneous restoration of bowel continuity (“auto-anastomosis”) has been seen bythe author in only 2 other patient. This unusualsequence permitted closure of the proximalcolostomy and acceptable bowel function in apatient whose general health would have contrain-dicated any further major reconstructive surgery.

Figure 95.2: Shows theanastomotico-vesical/fistula.

Figure 95.3: The extended lowanterior resection (11.1.73).

Figure 95.4: Anastomoticdehiscence.

Figure 95.5: Spontaneousauto-anastomosiswith stricture.

For a full-page image of this figure see the appendix.

HistoryThe patient presented with a 5-month history ofrectal bleeding and diarrhea. Colonoscopy revealeda carcinoma at 13cm and no other significant abnor-mality to the cecum. The patient’s psychologicalbackground included depression, aggression, and atendency towards litigation.

Operation (12.23.88)The carcinoma was situated in the intraperitonealrectum. There was no evidence of metastatic spread.The distal two-thirds of the sigmoid and the upperrectum were removed and the extraperitoneal anas-tomosis performed using an intraluminal stapler.The blood supply to the levels of resection of colonand rectum appeared satisfactory. No proximalstoma was performed. A drain with continuoussuction was placed in the presacral space for 48 h.

PathologyThe carcinoma was a polypoidal tumor (6 × 7cm) ofaverage grade penetrating the muscularis propria

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without lymph node metastases (Dukes B, T3 NoMo). It was continuous distally, with a soft, flattubular adenoma (8 × 7cm).

Postoperative CourseFor some days, the patient’s temperature remainedelevated, and he complained of suprapubic pain.There was minimal abdominal tenderness, andbowel function occurred without delay. He showedno significant signs of intraabdominal sepsis. Aroutine limited gastrograffin enema revealed a largespace in the pelvis with no identification of theanastomosis (Figure 96.1). Sigmoidoscopy revealedthat the ends of the bowel were separated by a largecavity (Figure 96.3). Conservative management waspursued with the patient because:

(i) he remained surprisingly free of septic signs;(ii) bowel function continued;

(iii) the patient was seriously disturbed at theprospect of a stoma; and

(iv) early reoperation would have been a Hartmannprocedure.

96 Anastomotic Dehiscence AfterAnterior Resection

Male, 54 Years

210

Figure 96.1: Contrast enema 27 days after operation. C:terminal colon; P: pelvic cavity; R: rectum.

Figure 96.2: Contrast could not pass the anastomoticstricture 9 months after failed anterior resection.

Over a period of 9 months, severe stricture forma-tion occurred that did not respond to dilatation(Figure 96.2).

Operation (9.28.89)Excision of the anastomotic stricture and distalcolon, reanastomosis of colon to rectum, and loopileostomy. The postoperative course was uneventful.

PathologyThere was a small chronic abscess posterior to thestricture in continuity with the lumen (Figure 96.4).The left colon showed evidence of chronic obstruc-tion. On histological examination, the distal colonadjacent to the persistent defect showed ulceration,and granulation, with submucosal fibrosis, consis-tent with ischemia.

Operation (12.4.89)Closure of loop ileostomy.

Diagram 96 211

CommentA mid rectal (extraperitoneal) anastomosis has alower incidence of anastomotic leak (1.7%) than anextended (ultra low) anterior resection (5.4%).1 Thisdifference has been noted in a number of series,including the results of 1014 patients with stapledrectal anastomosis treated at the Cleveland Clinic.2

Accordingly, complete dehiscence of the anasto-mosis was not expected in this patient, who wasthought to be “risk free.” Nevertheless, it isacknowledged that accurate prediction of AL isimpossible.3 There was a concern in this patient thata malfunctioning suction drain may have inducedexcessive suction pressure, causing damage to themarginal vessels of the terminal colon. In theabsence of clinical signs of sepsis, the complicationwas managed conservatively. Bowel continuity canbe gradually established spontaneously in such caseswith stricture formation that may allow reasonablebowel function, note Case 95, or require electivereconstructive surgery.

Figure 96.3Anastomotic dehiscence(December 1988)

Figure 96.4Anastomotic stricture(September 1989)

HistoryThe patient had noticed diarrhea, urgency, andminimal rectal bleeding for 8 months. Until thistime, the patient had been in excellent health.Examination revealed a mobile carcinoma of therectum at 7cm. It occupied one-third of the lumencircumference in the right lateral quadrant.Colonoscopy revealed no other pathology.

Operation (5.27.94)Laparotomy revealed no evidence of metastaticdisease. The transverse and sigmoid colon wasredundant. The bowel was resected from midsigmoid to lower third of the rectum (3cm above thepelvic floor). The upper left colic artery was pre-served to support the blood flow in the marginalartery, which was found to be excellent at the levelof resection of the sigmoid colon. An extended(ultra) low anterior resection (LARx) anastomosiswas performed with a single circular stapler. Thelength of the left colon–sigmoid was more than adequate, and splenic flexure mobilization was not

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undertaken. No blood transfusion was administeredand there were no circulatory abnormalities duringthe operation. No proximal stoma was performed.

PathologyThe tumor was a flat ulcer, 50 × 40mm withminimal penetration beyond the muscularis propria(Dukes B, T3 N0 M0). It was a moderately differenti-ated adenocarcinoma.

Postoperative ProgressInitially, the patient’s postoperative state was satis-factory, but by day 7 she was pyrexial with abdom-inal distention. A limited gastrograffin enema onday 9 (Figure 97.1) suggested ulceration in a narrowdistal colon with minimal extravasation of the con-trast into the pelvis. The patient was not generallyunwell, but remained distended without a bowelaction. A rectal examination on day 21 revealed

97 Postoperative Necrosis of the Left Colon

Female, 35 Years

212

Figure 97.1: Contrast enema on day 9 shows narrowingand ulceration of the colon due to ischemia.

Figure 97.2: Contrast enema on day 21 demonstratesextensive extravasation of contrast into the pelvis due toanastomotic dehiscence. The enema tube is in the pelviccavity.

complete separation of the anastomosis which wasalso demonstrated with a further limited contrastenema (Figure 97.2). There were no clinical signs ofperitonitis.

Operation (6.18.94)Laparotomy revealed dehiscence of the anastomosisdue to ischemic necrosis of the distal colon. Thepelvis was lined by granulation tissue without pusformation or fecal contamination and was effec-tively sealed off by adherent loops of small bowel.The feces in the dilated colon had not negotiated an ischemic stricture. The pelvic granulation tissuewas curetted. The remaining rectum transected atthe pelvic floor level and the colon resected to theupper descending colon. The colon was irrigated free of fecal content. After mucosectomy, a straightsleeve coloanal anastomosis was performed with peranal sutures. A loop ileostomy was constructed.

PathologyThe resected bowel showed mucosal ulceration,stricture formation, areas of full thickness necrosis,

Diagram 97 213

and thrombus within arteries consistent with anischemic process.

Follow-Up (2004)The patient’s recovery was uneventful. Theileostomy was closed three months after the reop-eration. Bowel function is sporadically frequent(“clustering”), but continence is normal. Thepatient’s colorectal cancer (CRCa) follow up hasbeen satisfactory for 10 years and 2 months.

CommentThis catastrophic complication was unexpected inan otherwise healthy 35-year-old patient. During theoperation, the marginal blood flow to the sigmoidcolon was seen to be satisfactory. Thrombosis in the postoperative period must have supervened. Theclinical presentation of complete separation of theanastomosis was that of obstruction and not peri-tonitis. The “clean” pelvis facilitated an immediatereconstruction to be performed with a coloanal anas-tomosis, which is an unusual opportunity in thepresence of this serious complication.

6.18.94

For a full-page image of this figure see the appendix.

HistoryIn 1975, cholecystectomy was performed for gall-stones. In 1982, appendectomy for gangrenousappendicitis was followed by several operations fora pelvic abscess. During 1996, episodes of acutesmall bowel obstruction necessitated 3 admissionsto the hospital. Laparotomy (9.19.96) revealed smallbowel obstruction due to extensive adhesions. Com-plete adhesiolysis of small bowel was performedwith repair to several sites of the bowel wall. Theobstruction failed to settle over the subsequent 17days and reoperation was necessary. Laparotomy(10.6.96), revealed a “mass of matted bowel withadhesions rock solid, like concrete.” Extensive dis-section resulted in ischemia to segments of smallbowel and the left colon. A “massive” resection ofileum was performed (leaving 100cm of smallbowel), and the left colon was resected as a Hart-mann operation. Subsequent to this operation, asmall bowel fistula presented in the lower part ofthe abdominal wound. It failed to heal with conser-vative treatment. The patient was referred forfurther management.

Operation (1.13.97)The small bowel fistula was attached to the lowerend of the incision. Dense fibrous adhesionsinvolved the total length of the small bowel. Tencentimeters of small bowel containing the fistulawas resected with anastomosis. The patient was dis-charged from the hospital 28 days after operation,when nutritional support was stabilized.

Operation (4.20.98)Reversal of Hartmann operation. The shortenedcolon was brought through the mesentery of thejejunum. Adhesions in the right iliac fossa were leftundisturbed, which meant the loop stoma was moreproximal than was ideal. (Figure 98.1).

Operation (5.4.98)Closure of the ileostomy was performed by resec-tion 14 days after the preceding surgery. This waswell prior to the usual 3-month delay for this operation. Phlegmonous adhesive changes were

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maximal, with dense fibrosis obscuring the smallbowel. The usual trephine wound required signifi-cant extension. The distal limb of the small bowelwas fixed by adhesions that could not be safely dis-sected. Fortunately, the proximal ileum could bemobilized for a satisfactory single layer anastomo-sis. Two denuded areas of the seromuscular layercould not be closed without tension due to the fix-ation of the ileum by adhesions.

Follow-Up (October 2004)The patient’s nutritional state requires continuedsupport. Investigations have demonstrated a degreeof malabsorption. Bowel function is: Day/Night: 2-5/0. There have been no further episodes of bowelobstruction.

CommentThe adhesion pathology encountered in theileostomy closure in this patient made it difficult toprogress or “back off” to another day. The toughreactive adhesions caused the dissection to danger-ously denude the exposed small bowel. The patient

98 Ileostomy Closure: An Impasse Due to Adhesions

Male, 69 Years

214

Figure 98.1: Reversal of Hartmann Operation(4.20.98).*:Indicates the site selected for loop ileostomy.

Diagram 98 215

was known to form difficult adhesions, as observedat previous surgery, and attempting ileostomyclosure at 14 days postoperatively almost certainlyincreased the operative difficulties. The author’spreference has been to close stomas after 12 weeksbut was persuaded otherwise in this case. Bakx et al,however, have reported early closure of ileostomies,during the same admission as the initial operation,and found this was associated with a low incidenceof morbidity.1 Trials with adhesion-preventing sub-stances have not as yet established their efficacy

beyond doubt. Tang et al, in a randomized trial,studied early closure of ileostomies in which anadhesion barrier membrane had been previouslywrapped around the limbs of the ileostomy. Theyconcluded that the technique reduced peristomaladhesions and facilitated early closure of theileostomies.2 Tjandra and Ng, in a randomized con-trolled trial, assessed the use of a spray gel when cre-ating an ileostomy and found there were feweradhesions at the closure of the ileostomy in thosecases where spray gel was used.3

HistoryIn April 1996, the patient was urgently admitted tothe hospital, with severe abdominal pain. Clinicalexamination revealed evidence of peritonitis, indi-cating the need for immediate operation. Her rele-vant past history was:1965 Removal of ovarian tumor and postoperative

radiotherapy (RT) to the pelvis (diagnosisand dose of RT not known).

1993 Stroke followed by full recovery.1994 Left femoropopliteal bypass.1995 External beam radiotherapy (60Gy) to the (Aug.) pelvis for a high-grade transitional cell car-

cinoma of the bladder.1996 Flexible sigmoidoscopy to investigate pelvic (Feb.) pain revealed stenosis at 20 cm and evidence

of irradiation proctocolitis.

Operation (4.26.96)Laparotomy revealed a purulent generalized peri-tonitis due to a perforation in the mid sigmoidcolon. The perforation was situated on the anteme-senteric border of the colon, 7–8mm in diameter

C A S E

with a thin necrotic edge. The sigmoid colon wasshortened in length, contracted in diameter withabsence of haustra. The bowel wall felt thickened.There were acute and chronic inflammatorychanges on the serosal surface. The small bowel wasadherent to the sigmoid colon, and dense adhesionshad “welded” together adjacent loops. There waspallor and an abnormal vascularity on the serosalaspect of the sigmoid colon and small bowel, typicalof an irradiation reaction. The left colon was dilatedand impacted with solid feces. There was a 2cmmetastasis in the anterior aspect of the right lobe ofthe liver. A Hartmann resection was performed afterthorough irrigation of the abdominal cavity.

PathologyThe mucosa of the opened specimen showed hem-orrhagic inflammation and ulceration. The perfora-tion had occurred in the base of a radionecroticulcer. The bowel wall was thickened and the lumenstenosed. Histologically, there were changes due tothe radiation (Figures 99.1 and 99.2).

99 Perforation of the Sigmoid Colon Dueto Radiation Injury

Female, 72 Years

216

Figure 99.1: Endarteritis typical of radiation effect(arrow).

Figure 99.2: Section shows complete loss of mucosa withedema and granulation in the submucosa.

Postoperative CourseOn day 2 after operation, an urgent left iliacendarterectomy was performed for acute arterialobstruction. Although bowel function returned sat-isfactorily, reestablishing oral feeding failed andtotal parenteral nutrition was commenced. Thepatient remained anorexic, lethargic, and depressed,and continued to decline until her death on day 14after operation. No autopsy was performed.

CommentRadiotherapy was selected for treatment of thebladder tumor, because the patient was regarded as

Diagram 99 217

unfit for cystectomy. The radiotherapist discussedwith the patient his concern about the potentialmorbidity of radical radiotherapy in relation to theunknown dose of pelvic radiotherapy 30 years pre-viously, her age, and general health. Schellhammeret al state that preexisting arteriosclerotic vasculardisease is important in determining the degree ofearly and late radiation injuries.1 The patient’sdemise occurred in the absence of any obviousabdominal complications. This mode of postopera-tive decline has been noted previously in the frailelderly patient undergoing surgery for the compli-cations of radiotherapy.

HistoryIn 1974, the patient, aged 28 years, was diagnosedwith chorionic carcinoma. Local spread had formed a large pelvic mass and lung metastases were present. The latter disappeared after treatment withmethotrexate. Laparotomy was performed in August1974 when extensive pelvic spread was foundbeyond surgical excision. This pelvic disease wassuccessfully treated with external beam radiother-apy (58.6 Gy) and actinomycin D. Eighteen monthsafter completing this treatment, the patient wasreferred for the surgical management of a stricture (6mm in diameter) of the rectum and a rectovaginalfistula discharging into the posterior fornix. Investi-gations revealed no evidence of recurrent tumor. Theskin of the perineum showed evidence of tissue reaction to radiotherapy (Figure 100.1).

Operation (9.7.76)Laparotomy–loop ileostomy. The rectovaginalfistula subsequently appeared to heal.

Operation (10.18.77)Laparotomy revealed no evidence of recurrenttumor. There was dense pelvic fibrosis, particularlyin the rectovaginal septum. The bowel was resectedfrom proximal sigmoid to mid rectum. After muco-sectomy of the rectum and anal canal, the colon was“sleeved” within the rectum and a coloanal anasto-mosis performed (Soavé operation).

PathologyThe site of the rectovaginal fistula was not identi-fied. There were 2 rectal strictures present. Therectal mucosa was pale and atrophic, with telang-iectases prominent on its surface. The rectal wallwas thickened due to fibrosis. Histologically, therewas mucosal ulceration, chronic inflammation, andfibrosis in the submucosa and the muscle wall(Figure 100.2). There were vascular changes consis-tent with a radiation effect. There was no evidenceof recurrent tumor.

Operation (2.7.78)Closure of the ileostomy.

C A S E

Follow-Up (2000)The coloanal anastomosis remained well healed and supple, but bowel function was unsatisfactorywith poor control of flatus, rectal discomfort, andprolonged efforts to evacuate stool. A permanentileostomy was established 5/26/81. The patient waslast assessed, 26 years after the diagnosis of chori-onic carcinoma. There was no evidence of recurrentdisease.

100 Radiation Rectovaginal FistulaFemale, 30 Years

218

Figure 100.1: The perineum shows permanenttelangiectasia due to radiotherapy.

Figure 100.2: Histology of the rectum showing fibrosis inthe muscularis propria and fibrous thickening of thesubmucosa.

CommentCurrently, the cure rate for chorionic carcinoma hasbeen reported to be 86.4%.1 This patient appears tohave been cured but at the cost of serious postradi-ation morbidity. The high dose of radiotherapyadministered in 1974 was 58.6Gy in 29 fractions,but severe complications are not always dose

Diagram 100 219

related.2 The operative technique in this patientavoided the difficult, if not hazardous, deep pelvicdissection. Unfortunately, the technical success wasa functional failure due to poor compliance causedby the rigid rectal remnant. Parks et al, however,demonstrated in 4 patients that the Soavé procedurehas a role in treating this difficult problem.3

For a full-page image of this figure see the appendix.

CASE 1

1. Minardi AJ Jr, Zibari GB, Aultman DF, McMillanRW, McDonald JC. Small bowel tumors. J. Am.Coll. Surg. 1998;186:664–668.

2. Pan Y, Kuo H, Lai H, Chuang S, Liu C. Solitary ileallipoma presenting with ileocolic intussusception:an unusual cause of enteritis cystica profunda. J.Formos. Med. Assoc. 1997;96:469–472.

3. Chong AK, Taylor AC, Miller AM, Desmond PV.Initial experience with capsule endoscopy at amajor referral hospital. Med. J. Aust. 2003;178:537–540.

Case 2

1. Thompson GB, van Heeden JA, Martin Jr JK,Schutt AJ, Ilstrup DM, Carney JA. Carcinoidtumors of the gastrointestinal tract: presentation,management and prognosis. Surgery 1985;98:1054–1063.

2. Vinik AI, McLeod MK, Fig LM, Shapiro B, LloydRV, Cho K. Clinical features, diagnosis and locali-zation of carcinoid tumors and their management.Gastroenterol. Clin. North Am. 1989;18:865–896.

3. Memon MA, Nelson H. Gastrointestinal carcinoidtumors. Dis. Colon Rectum 1997;40:1101–1118.

Case 3

1. Tse V, Lochhead A, Adams W, Tindal D. Concur-rent colonic adenocarcinoma and two ileal carci-noids in a 72-year-old male. Aust. N.Z. J. Surg.1997;67:739–741.

2. Thompson GB, van Heerden JA, Martin JK Jr.,Schutt AJ, Ilstrup DM, Carney JA. Carcinoidtumors of the gastrointestinal tract: presentation,management, and prognosis. Surgery 1985;98:1054–1063.

3. Chong AK, Taylor AC, Miller AM, Desmond PV.Initial experience with capsule endoscopy at amajor referral hospital. Med. J. Aust. 2003;178:537–540.

Case 4

1. Rangiah DS, Co M, Richardson M, Tompsett E,Crawford M. Small bowel tumors: a 10 year expe-rience in four Sydney teaching hospitals. Aust.N.Z. J. Surg. 2004;74:788–792.

2. Huilgol RL, Young CJ, Solomon MJ. The GIST ofit: case reports of a gastrointestinal stromal tumorand a leiomyoma of the anorectum. Aust. N.Z. J.Surg. 2003;73:167–169.

3. Skandalakis J. Smooth muscle tumors of the gastrointestinal tract. Introduction. World J. Surg.2000;24:389–390.

4. Wolber RA, Scudamore CH. The gastrointestinaltract. In: Banks PM, Kraybill WG, eds. Pathologyfor the Surgeon. Philadelphia: Saunders, 1996:176.

5. Clary BDE, Matteo R, Lewis J. Gastrointestinalstromal tumors and leiomyosarcomas of theabdomen and retro peritoneum: a clinical compar-ison. Ann. Surg. Oncol. 2001;8:290–299.

Case 5

1. Hutchins RR, Bani Hani A, Kojodjojo P, Ho R,Snooks SJ. Adenocarcinoma of the small bowel.Aust. N.Z. J. Surg. 2001;71:428–437.

2. Kusumoto H, Takahashi I, Yoshida M, Maehara Y,Watanabe A, Oshiro T. Primary malignant tumorsof the small intestine: analysis of 40 Japanesepatients. J. Surg. Oncol. 1992;50:139–143.

3. Howe JR, Karnell LH, Menck HR, Scott-Conner C. Adenocarcinoma of the small bowel. Rev. Nat. Cancer Data Base 1985–1995. Cancer1999;86:2693–2706.

Case 6

1. Walfish J, Frankel A. Chronic pseudo-obstructionsecondary to side-to-side intestinal anastomosis.Arch. Surg. 1979;114:1075–1078.

2. Whitaker Jr WG, Shepard D. Late complications ofside-to-side intestinal anastomosis: case reports.Ann. Surg. 1965;161:824–831.

3. Frank P, Batzenschlager A, Philippe E. Blind-pouchsyndrome after side-to-side intestinal anastomosis.Chirurgie. 1990;116:586–596.

4. Clawson DK. Side-to-side intestinal anastomosiscomplicated by ulceration, dilatation, and anemia:a physiologically unsound procedure. Review ofthe literature and presentation of a case. Surgery1953;34:254–257.

Case 7

1. Black BM, McEachern CG. Redundant blind segments of intestine following side-to-side

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3. Frank P, Batzenschlager A, Phillippe E. Blind-pouch syndrome after side-to-side intestinal anas-tomosis. Chirurgie. 1990:116:586–596.

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1. Kahn M, Friedman IH. Mucocele of the appendix:diagnosis and surgical management. Dis. ColonRectum 1979;22:267–269.

2. Woodruff R, McDonald JR. Benign and malignantcystic tumors of the appendix. Surg. Gynecol.Obstet. 1940;71:750–755.

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Case 11

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3. Sakamoto GD, MacKeigan JM, Senagore AJ.Transanal excision of large villous adenomas. Dis.Colon Rectum 1991;34:880–885.

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2. Nivatvongs S, Balcos EG, Schottler JL, GoldbergSM. Surgical management of large villous tumoursof the rectum. Dis. Colon Rectum 1973;16:508–514.

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2. Tobin SA, Scott IH. Delorme operation for rectalprolapse. Br. J. Surg. 1994; 81:1681–1684.

Case 16

1. McColl I, Bussey HJR, Veale AMO, Morson BC.Juvenile polyposis coli. Proc. R. Soc. Med. 1964;57:896–897.

2. Smilow PC, Pryor CA, Swinton NW. Juvenile poly-posis coli. Dis. Colon Rectum 1966;9:248–254.

3. Howe JR, Mitros FA, Summers RW. The risk of gastrointestinal carcinoma in familial juvenilepolyposis. Ann. Surg. Oncol. 1998;5:751–756.

Case 17

1. Platell C, Levitt S. Juvenile polyposis: a premalig-nant condition? Anz. J. Surg. 1990;60:481–482.

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3. Jass JR, Williams CB, Bussey HJR, Morson BC.Juvenile polyposis—a precancerous condition.Histopathology 1988;13:619–630.

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5. Howe JR, Ringold JC, Summers RW, Mitros FA,Nishimura DY, Stone EM. A gene for familial juve-nile polyposis maps to chromosome 18q21.1. Am.J. Hum. Genet. 1998;62:1129–1136.

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1. McGarrity TJ, Kulin HE, Zaino RJ. Peutz-Jegherssyndrome. Am. J. Gastroenterol. 2000;95:596–604.

2. Spigelman AD, Murday V, Phillips RKS. Cancerand the Peutz-Jeghers syndrome. Gut. 1989;30:1588–1590.

3. Oncel M, Remzi FH, Church JM, Connor JT, FazioVW. Benefits of “clean sweep” in Peutz-Jegherspatients. Colorectal Dis. 2004;6:332–335.

4. Parsi MA, Burke CA. Utility of capsule endoscopyin Peutz-Jeghers syndrome. Gastrointest. Endosc.Clin. N. Am. 2004:14:159–167.

5. Schulmann K, Hollerbach S, Kraus K, Willert J,Vogel T, Moslein G, Pox C, Reiser M, Reinacher-Schick A, Schmiegel W. Feasibility and diagnosticutility of video capsule endoscopy for the detectionof small bowel polyps in patients with hereditarypolyposis syndromes. Am. J. Gastroenterol. 2005:100:27–37.

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1. Keighley MRB, Williams NS. Intestinal fistulas.Surgery of the Anus, Rectum and Colon.London:WB Saunders, 1993;Ch 63, pp. 2013–2102.

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1. Bussey HJ, Eyers AA, Ritchie SM, Thomson JP. Therectum in adenomatous polyposis: the St Mark’spolicy. Br. J. Surg.1985;72:S29–S31.

2. Church J, Burke C, McGannon E, Pastean O, ClarkB. Risk of rectal cancer in patients after colectomyand ileorectal anastomosis for familial adenoma-tous polyposis. Dis. Colon Rectum 2003;46:1175–1181.

3. De Cosse JJ, Bülow S, Neale K, Järvinen H, AlmsT, Hultcrantz R, Moesgaard F, Costello C. Rectalrisk in patients treated for familial adenomatouspolyposis. Br. J. Surg. 1992;79:1372–1375.

4. Bess MA, Adson MA, Elveback LR, Moertel CG.Rectal cancer following colectomy for polyposis.Arch. Surg. 1980;115:460–467.

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1. Kunakemakon P, Ontai G, Balin H. Pelvic inflam-matory pseudotumor: a case report Am. J. Obstet.Gynecol. 1976;126:286–287.

2. Ramtrez JM, Ortego J, Deus J, Bustamante E,Lozano R, Dominguez M. Lipomatous polyposis ofthe colon. Br. J. Surg. 1993;80:349–350.

3. Swain VAJ, Young WF, Pringle EM. Hypertrophy ofthe appendices epiploicae and lipomatous poly-posis of the colon. Gut 1969;10:587–589.

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Case 22

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Case 23

1. Thompson GB, van Heerden JA, Martin JK Jr.,Schutt AJ, Ilstrup DM, Carney JA. Carcinoidtumors of the gastrointestinal tract: presentation,management, and prognosis. Surgery 1985;98:1054–1063.

2. Memon MA, Nelson H. Gastrointestinal carcinoidtumors. Dis. Colon Rectum 1997;40:1101–1118.

3. Lotlikar U, Fogler R, Novetsky AD, Yoon NY. Con-current colonic carcinoma and small bowel

carcinoid tumor: Case reports and review of the literature. Dis. Colon Rectum 1982;25:375–382.

Case 24

1. Killingback M, Barron PE, Dent OF. Electivesurgery for diverticular disease: an audit of surgicalpathology and treatment. ANZ J. Surg. 2004;74:530–536.

Case 25

1. Sasson L. Metastatic neoplasm of esophagus simulating primary carcinoma. JAMA 1960;174:2075–2076.

2. Segalin A, Bonavina L, Ruol A, Boccasanta P,Salamina G, Peracchia A. Secondary esophagealtumors: treatment and outcome in 115 consecu-tive patients. Dis. Esoph. 1994;7:118–122.

Case 28

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2. Mayer R, Wong WD, Rothenberger DA, GoldbergSM, Madoff RD. Colorectal cancer in inflamma-tory bowel disease. A continuing problem. Dis.Colon Rectum 1999;42:343–347.

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Case 29

1. Shirouzu K, Isomoto H, Morodomi T, Ogata Y,Akagi Y, Kakegawat T. Primary linitis plastica carcinoma of the colon and rectum. Cancer1994;74:1863–1868.

Case 30

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2. Nakahara H, Ishikawa T, Itabashi M, Hirota T. Dif-fusely infiltrating primary colorectal carcinoma oflinitis plastica and lymphangiosis types. Cancer1992;69:901–906.

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Case 32

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Case 34

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4. Read TE, Mutch MG, Chang BW, McNevin MS,Fleshman JW, Birnbaum EH, Fry RD, Caushaj PF,Kodner IJ. J. Am. Coll. Surg. 2002;195:33–40.

5. Rieger N, Tjandra J, Solomon M. Endoanal andendorectal ultrasound: applications in colorectalsurgery. ANZ J. Surg. 2004;74:671–675.

6. Robinson P, Carrington BM, Swindell R, ShanksJH, O’Dwyer ST. Recurrent or residual pelvicbowel cancer: accuracy of MRI local extent beforesalvage surgery. Clin. Radiol. 2002;57:514–522.

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Case 35

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2. Mayer R, Wong WD, Rothenberger DA, GoldbergSM, Madoff RD. Colorectal cancer in inflamma-tory bowel disease. A continuing problem. Dis.Colon Rectum 1999;42:343–347.

3. Landmann DD, Fazio VW, Lavery IC, Weakley FL,Jagelman DG. En Bloc resection for contiguousupper abdominal invasion by adenocarcinoma ofthe colon. Dis. Colon Rectum 1989;32:669–672.

4. Lehnert T, Methner M, Pollok A, Schaible A, HinzU, Herfath C. Multivisceral resection for locallyadvanced primary colon and rectal cancer: ananalysis of prognostic factors in 201 patients. Ann.Surg. 2002;235:217–225.

5. Nakafusa Y, Tanaka T, Tanaka M, Kitajima Y, SatoS, Miyazaki K. Comparison of multivisceral resec-tion and standard operation for locally advancedcolorectal cancer: analysis of prognostic factors forshort-term and long-term outcome. Dis. ColonRectum 2004;47:2055–2062.

6. Hunter JA, Ryan JA, Schultz P. En bloc resectionof colon cancer adherent to other organs. Am. J.Surg. 1987;145:67–71.

7. Gall FP, Tonak J, Altendorf A. Multivisceral resec-tions in colorectal cancer. Dis. Colon Rectum1987;30:337–341.

8. Johnson WR, McDermott FT, Hughes ESR, PihlEA, Milne BJ, Price AB. Carcinoma of the colonand rectum in inflammatory disease of the intes-tine. Surg. Gynecol Obstet. 1988;156:193–197.

9. Walfisch S, Stern H. Use of thoracoabdominal incision for cancer of the splenic flexure in theobese patient. Dis. Colon Rectum 1989;32:169–170.

Case 36

1. Morson BC. The muscle abnormality in diverti-cular disease of the colon. Proc. R. Soc. Med.1963a;56:798–800.

2. Killingback M, Barron PE, Dent OF. Electivesurgery for diverticular disease: an audit of sur-gical pathology and treatment. ANZ J. Surg.2004;74:530–536.

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Case 37

1. Kelly JK. Polypoid prolapsing mucosal folds indiverticular disease. Am. J. Surg. Pathology. 1991;15:871–878.

2. Gore S, Shepherd NA, Wilkinson SP. Endoscopiccrescentic fold disease of the sigmoid colon: theclinical and histopathological spectrum of a dis-tinct endoscopic appearance. Int. J. Colorectal Dis.1992;7:76–81.

Case 38

1. Gooszen AW, Tollenaar RA, Geelkerken RH,Smeets HJ, Bemelman WA, van Schaardenburgh P,Gooszen HG. Prospective study of primary anas-tomosis following sigmoid resection for suspectedacute complicated diverticular disease. Br. J. Surg.2001;88:693–697.

2. Ravo B, Metawally N, Castera P, Polansky PJ, GerR. The importance of intraluminal anastomoticfecal contact and peritonitis in colonic anasto-motic leakages. An experimental study. Dis. ColonRectum 1988;31:868–871.

3. Zorcolo L, Covotta L, Carlomagno N, BartoloDCC. Safety of primary anastomosis in emergencycolorectal surgery. Colorectal Dis 2003;5:262–269.

4. Salem L, Flum D. Primary anastomosis or Hart-mann’s procedure for patients with diverticularperitonitis? A systemic review. Dis. Colon Rectum2004;47:1953–1964.

Case 39

1. Killingback M, Barron PE, Dent OF. Electivesurgery for diverticular disease: an audit of sur-gical pathology and treatment. Aust. N. Z. J. Surg.2004;74:530–536.

2. Pheils MT, Duraiappah B, Newland RC. Chronicphlegmonous diverticulitis. Aust. N. Z. J. Surg.1973;42:337–341.

3. Whelan RL, Umana JP. Colon cancer versus diver-ticulitis. In: Welch JP, Cohen JL, Sardella WV,Vignati PV, eds. Diverticular Disease: Manage-ment of the Difficult Case. Baltimore: Williamsand Wilkins, 1998;Ch 4, pp. 55–66.

Case 41

1. Fazio VW, Church JM, Jagelman DG, Weakley FL,Lavery IC, Tarazi R, van Hillo M. Colocutaneousfistulas complicating diverticulitis. Dis. ColonRectum 1987;30:89–94.

2. Parks AG, Gordon PH. Perineal fistula of intra-abdominal or intrapelvic origin simulating fistulain ano:report of 7 cases. Dis. Colon Rectum1976;19:500–506.

Case 43

1. Del Pino A, Abcarian H. Colovesical fistula. In:Welch JP, Cohen JL, Sardella WV, Vignati PV, eds.Diverticular Disease: Management of the DifficultSurgical Case. Baltimore:Williams and Wilkins,1998;Ch 10, pp. 151–166.

2. Killingback M, Barron PE, Dent OF. Electivesurgery for diverticular disease: an audit of surgical pathology and treatment. ANZ J. Surg.2004;74:530–536.

3. Woods RJ. Diverticulitis and fistula. In: Fazio VW,Church JM, Delaney CP, eds. Current Therapy inColon and Rectal Surgery. 2nd ed. Philadelphia:Elsevier Mosby, 2005;Ch 52, pp. 297–300.

4. Killingback M. Elective surgery for sigmoid diver-ticular disease. In: Fielding LP, Goldberg SM, eds.Rob and Smiths Operative Surgery. Surgery of the Colon Rectum and Anus. 5th ed. Oxford: Butterworth-Heinemann, 1993:369–386.

Case 45

1. Killingback M, Barron PE, Dent OF. Electivesurgery for diverticular disease: an audit of sur-gical pathology and treatment. ANZ J. Surg.2004;74:530–536.

Case 46

1. Heimann T, Aufses AH Jr. Giant sigmoid diver-ticula. Dis. Colon Rectum 1981;24:468–470.

2. Ellerbroek CJ, Lu CC. Unusual manifestations ofgiant colonic diverticulum. Dis. Colon Rectum1984;27:545–547.

3. Altaf N, Geary S, Ahmed I. Giant colonic diver-ticulum. J. R. Soc. Med. 2005;98:169–170.

4. Choong CK, Frizelle FA, Giant colonic diver-ticulum. Report of 4 cases and review of the liter-ature. Dis. Colon Rectum 1998;41:1178–1186.

Case 47

1. Choong CK, Frizelle FA. Giant colonic diverticu-lum. Report of 4 cases and review of the literature.Dis. Colon Rectum 1998;41:1178–1186.

2. Killingback M, Barron PE, Dent OF. Electivesurgery for diverticular disease: an audit of surgi-cal pathology and treatment. ANZ J. Surg.2004;74:530–536.

3. Gallagher JJ, Welch JP. Giant diverticula of thesigmoid colon. Arch. Surg. 1979;114:1079–1083.

Case 49 Recommended Reading List

Morson BC, Dawson IMP, Day DW, Jass JR, Price AB,Williams GT. Inflammatory disorders. In: MorsonBC, Dawson IMP, eds. Morson and Dawson’s GastroIntestinal Pathology. 3rd ed. London: Blackwell Scientific Publications, 1990;Ch 37, pp. 477–549.

Keighley MRB, Williams NS. Crohn’s disease: pathol-ogy, diagnosis and differential diagnosis. Surgery ofthe Anus, Rectum, and Colon. London:WB Saun-ders, 1993;Ch 50, pp. 1631–1659.

Kleer CG, Appelman HD. Surgical pathology ofCrohn’s disease. Surg. Clin. North Am. 2001;81:13–30.

Case 51

1. Yamamoto T, Allan RN, Keighley MR. An audit ofgastroduodenal Crohn’s disease: clinicopathologicfeatures and management. Scand. Gastroenterol.1999;34:1019–1024.

Case 52

1. Keighley MRB, Williams NS. Surgical treatment of small bowel Crohn’s disease. Surgery of theAnus, Rectum, and Colon. 1st ed. London: W.B.Saunders, 1993;Ch 55, pp. 1710–1756.

2. Simonowitz DA, Rusch VW, Stevenson JK. Naturalhistory of incidental appendectomy in patientswith Crohn’s disease who required subsequentbowel resection. Am. J. Surg. 1982;143:171–173.

3. Nakano H, Miyachi I, Kitagawa Y, Saito H,Yamauchi M, Horiguchi Y, Nakajima S, Itoh M,Miyagawa S, Iwase K, et al. Crohn’s disease associated with giant inflammatory polyposis.Endoscopy. 1987;9:246–248.

Case 55

1. Morson BC, Dawson IMP, Day DW, Jass JR, Price AB, Williams GT. Inflammatory disorders. In: Morson BC, Dawson IMP, eds. Morson andDawson’s Gastrointestinal Pathology. 3rd ed.London:Blackwell Scientific Publications, 1990;Ch 22, pp. 240–302.

2. Keighley MRB, Williams NS. Crohn’s disease:pathology, dignosis and differential diagnosis.Surgery of the Anus, Rectum and Colon. London:WB Saunders, 1993;Ch 50, pp. 1631–1659.

Case 56

1. Dietz DW, Lauretti S, Strong SA, Hull TL, ChurchJ, Remi FH, Lavery IC, Fazio VW. Safety and longterm efficacy of strictureplasty in 314 patientswith obstructing small bowel Crohn’s disease. J.Am. Coll. Surg. 2001;192:330–337.

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Case 57

1. van Hogezand RA, Bemelman WA. Management ofrecurrent Crohn’s disease. Neth. J. Med.1998;53:S32–S38.

2. Fazio VW, Marchetti F, Church M, Goldblum JR,Lavery IC, Hull TL, Milsom JW, Strong SA, OakleyJR, Secic M. Effect of resection margins on therecurrence of Crohn’s disease in the small bowel.A randomized controlled trial. Ann. Surg. 1996;224:563–573.

3. Borley NR, Mortensen NJ, Chaudry MA,Mohammed S, Warren BF, George BD, Clark T,Jewell DP, Kettlewell MG. Recurrence afterabdominal surgery for Crohn’s disease: relation-ship to disease site and surgical procedure. Dis.Colon Rectum 2002;45:377–383.

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Case 58

1. Fisher J, Mantz F, Calkins WG. Colonic perforationin Crohn’s disease. Gastroenterol. 1976;71:385–388.

2. Connell WR, Sheffield JP, Kamm MA, Ritchie KJ,Hawley PR, Lennard-Jones JE. Lower gastrointesti-nal malignancy in Crohn’s disease. Gut1994;35:347–352.

3. Gillen CD, Andrews HA, Prior P, Allan RN.Crohn’s disease and colorectal cancer. Gut1994;35:651–655.

4. Ribeiro MB, Greenstein AJ, Sachar DB, Barth J, Bal-asubramanian S, Harpaz N, Heimann TM, AufsesAH Jr. Colorectal adenocarcinoma in Crohn’sdisease. Ann. Surg. 1996;223:186–193.

Case 59

1. Gan IS, Beck PL. A new look at toxic megacolon:an update and review of incidence, etiology, patho-genesis and management. Am. J. Gastroenterol.2003;98:2363–2371.

2. Turnbull RB Jr., Hawk WA, Weakley FL. Surgicaltreatment of toxic megacolon. Ileostomy andcolostomy to prepare patients for colectomy. Am.J. Surg. 1971;122:325–331.

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Case 60

1. Goligher JC. Ulcerative colitis. Surgery of theAnus, Rectum and Colon. 4th ed. London: BaillièreTindall, 1980;Ch 21, pp. 689–826.

2. Joffe N. Localised giant pseudopolyps secondary toulcerative colitis or granulomatous colitis. Clin.Radiol. 1977;28:609–616,

3. Fenoglio-Preiser CM, Noffsinger AE, Stem-mermann GN, Lantz PE, Listrom MB, Rilke FO.Inflammatory bowel disease. GastrointestinalPathology. An Atlas and Text. 2nd ed. Philadelphia:Lipincott-Raven, 1999;Ch 16, pp. 631–716.

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5. Keighley MRB, Williams NS. Pathology and diag-nosis of ulcerative colitis. Surgery of the anus,rectum and colon. London: WB Saunders, 1993;Ch39, pp. 1274–1303.

Case 61

1. Johnson WR, Hughes ESR, McDermott FT,Katrivessis H. The outcome of patients with ulcer-ative colitis managed by subtotal colectomy. Surg.Gynecol. Obstet.1986:162:421–425.

Case 62

1. Mayer R, Wong WD, Rothenberger DA, GoldbergSM, Madoff RD. Colorectal cancer in inflam-matory bowel disease. A continuing problem. Dis.Colon Rectum 1999;42:343–347.

2. Lennard-Jones JE, Melville DM, Morson BC,Ritchie JK, Williams CB. Precancer and cancer inextensive ulceration colitis: findings among 401patients over 22 years. Gut 1990;31:800–806.

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Case 63

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2. Feldman PS. Ulcerative disease of the colon prox-imal to partially obstructive lesions: report of twocases and review of the literature. Dis. ColonRectum 1975:18:601–612.

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4. Morson BC, Dawson IMP, Day DW, Jass JR, PriceAB, Williams GT. Vascular disorders. In: MorsonBC, Dawson, IMP. Morson and Dawson’s Gas-trointestinal Pathology. 3rd ed. London: BlackwellScientific Publications, 1993;Ch 38, pp. 550–562.

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Case 64

1. Bradbury AW, Barrett S. Surgical aspects of clostrid-ium difficile colitis. Brit. J. Surg. 1997;84:150–159.

2. Longo WE, Mazuski JE, Virgo KS, Lee P, Bahadurs-ingh AN, Johnson FE. Outcome after colectomy forclostridium difficile colitis. Dis. Colon Rectum2004;47:1620–1626.

3. Gan SI, Beck PL. A new look at toxic megacolon:an update and review of incidence, megacolon eitiology, pathogenesis and management. Am. J.Gastroenterol. 2003;98:2363–2371.

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Case 65

1. Findlay JA, Addison NV, Stevenson BK, Mirza ZA.Tuberculosis of the gastrointestinal tract in Brad-ford 1967–1977. J. R. Soc. Med. 1979;72:587–590.

2. Keighley MRB, Williams NS. Tropical coloproc-tology. Surgery of the Anus, Rectum and Colon.London: WB Saunders Company Ltd., 1993;Ch 68,pp. 2223–2261.

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Case 66

1. Ioachim HL, Ratech H. Burkitt lymphoma.Ioachim’s Lymph Node Pathology. 3rd ed.,

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Case 67

1. Morson BC, Dawson IMP, Day DW, Jass JR, PriceAB, Williams GT. Non-epithelial tumours. In:Morson BC, Dawson IMP, eds. Morson andDawson’s Gastrointestinal Pathology. 3rd ed.London: Blackwell Scientific Publications,1990;Ch 27, pp. 372–387.

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Case 68

1. Bargen JA. Chronic ulcerative colitis associatedwith malignant disease. Arch. Surg. 1928;17:561–576.

2. Baker D, Chiprut RO, Rimer D, Lewin KJ, Rose-berg MZ. Colonic lymphoma in ulcerative colitis.J. Clin. Gastroenterol. 1985;7:379–386.

3. Fan CW, Changchien CR, Wang JY, Chen JS, HsuKC, Tang R, Chiang JM. Primary colorectal lym-phoma. Dis. Colon Rectum 2000;43:1277–1282.

4. Wagonfeld JB, Platz CE, Fishman FL, Sibley RK,Kirsner JB. Multicentric colonic lymphoma complicating ulcerative colitis. Am. J. Dig. Dis.1977;22:502–508.

Case 69

1. Au E, Ang PT, Tan P, Sng I, Fong CM, Chua EJ, OngYW. Gastrointestinal lymphoma–a review of 54patients in Singapore. Ann. Acad. Med. Singap.1997;26:758–761.

2. Crump M, Gospodarowicz M, Shepherd FA. Lymphoma of the gastrointestinal tract. Semin.Oncol. 1999;26:324–337.

Case 70

1. Tan GY, Chong CK, Eu KW, Tan PH. Gastroin-testinal stromal tumor of the anus. Tech. Colo-proctol. 2003;7:169–172.

2. Robb JA, Jones RA. Spindle cell lipoma in a peri-anal location. Hum. Pathol. 1982;13:1052.

3. Frick EJ Jr, Lapos L, Vargas HD. Solitary neurofi-broma of the anal canal: report of two cases. Dis.Colon Rectum 2000;43:109–112.

4. Cohen MG, Greenwald ML. Garbus JE, Zager JS.Granular cell tumor—a unique neoplasm of theinternal anal sphincter: report of a case. Dis. ColonRectum 2000;43:1444–1447.

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Case 71

1. Tsang WY, Chan JK, Lee KC, Fisher C, FletcherCD. Aggressive angiomyxoma. A report of fourcases occurring in men. Am. J. Surg. Pathol.1992;11:1059–1065.

2. Wilson E. Ischiorectal tumor. Med. J. Aust.1969;2:402–403.

3. Nyam DCNK, Pemberton JH. Large aggressiveangiomyxoma of the perineum and pelvis: an alter-native approach. Dis. Colon Rectum 1998;41:514–516.

Case 72

1. Guiss RL. The implantation of cancer cells with afistula-in-ano. Surgery 1954;36:136–139.

2. Keynes WM. Implantation from the bowel lumenin cancer of the large intestine. Ann. Surg.1961;153:357–364.

3. Killingback M, Wilson TE, Hughes ESR. Analmetastases from carcinoma of the rectum andcolon. ANZ J. Surg. 1965;34:178–187.

4. Hyman N, Kida M. Adenocarcinoma of thesigmoid colon seeding a chronic anal fistula. Dis.Colon Rectum 2003;46:835–836.

5. Kline RJ, Spencer RJ, Harrison Jr. EG. Carcinomaassociated with fistula-in-ano. Arch. Surg.1964;89:989–994.

Case 74

1. Stuart M. Proctitis cystica profunda. Dis. ColonRectum 1984;27:153–156.

2. Nagasako K, Nakee Y, Kitao Y, Aoki G. Colitiscystica profunda: report of a case in which differ-entiation from rectal cancer was difficult. Dis.Colon Rectum 1977;20:618–624.

3. Valenzuela M, Martin-Ruiz JL, Alvarez-CienfuegosE, Caballero AM, Gallego F, Carmona I, Rodriguez-Tellez M. Colitis cystica profunda: imaging dia-gnosis and conservative treatment: report of twocases. Dis. Colon Rectum 1996;39:587–590.

Case 75

1. Madigan MR, Morson BC. Solitary ulcer of therectum. Gut 1969;10:871–881.

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3. Nicholls RJ, Simson JNL. Anterior posterior rectopexy in the treatment of the solitary ulcersyndrome without overt prolapse. Br. J. Surg.1986;73:222–224.

Case 76

1. Parks AG, Gordon PH, Hardcastle JC. A classifica-tion of fistula in ano. Br. J. Surg. 1976;63:1–12.

Case 78

1. Weiss S, Goldblum J. Fibromatoses. Enzinger andWeiss’s Soft Tissue Tumors. 4th ed. St Louis:Mosby Inc., 2001;Ch 10, pp. 309–346.

2. Church JM. Desmoid tumors. In Fazio VW, Church JM, Delaney CP, eds. Current Therapy inColon and Rectal Surgery. 2nd ed. Philadelphia:Elsevier Mosby, 2005;Ch 61 pp. 355–358.

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Case 79

1. Gagliardi G, Thompson IW, Herhsman MJ, ForbesA, Hawley PR, Talbot IC. Pneumatosis coli: a proposed pathogenesis based on study of 25 casesand review of the literature. Int. J. Colorectal. Dis.1996;11:111–118.

2. Galandiuk S and Fazio VW. Pneumatosis cystoidesintestinalis. A review of the literature. Dis. ColonRectum 1986;29:358–363.

3. Forgacs P, Wright PH, Wyatt AP. Treatment ofintestinal gas by oxygen breathing. Lancet 1973;1:579.

Case 80

1. Maurer CA, Renzulli P, Mazzucchelli L, Egger B,Seiler CA, Büchler MW. Use of accurate diagnosticcriteria may increase the incidence of stercoral perforation of the colon. Dis. Colon Rectum2000;43:991–998.

2. Serpell JW, Nicholls RJ. Stercoral perforation of thecolon. Br. J. Surg. 1990;77:1325–1329.

3. Haddad R, Bursle G, Piper B. Stercoral perforation ofthe sigmoid colon. ANZ J. Surg. 2005;75:244–246.

Case 81

1. Marston A, Pheils MT, Thomas ML, Morson BC.Ischemic colitis. Gut 1966;7:1–15.

2. Brown AR. Non-gangrenous ischaemic colitis: areview of 17 cases. Br. J. Surg 1972;59:463–473.

3. Longo WE, Ballantyne GH, Gusberg RJ. Ischemiccolitis: patterns and prognosis. Dis. Colon Rectum1992;35:726–730.

Case 83

1. Graham WP, Goldman L. Gastrointestinal metas-tases from carcinoma of the breast. Ann. Surg.1964;159:477–480.

2. Rabau MY, Alon RJ, Werbin N, Yossipov Y.Colonic metastases from lobular carcinoma of thebreast. Dis. Colon Rectum 1988;31:401–402

3. Tot T. The role of cytokeratins 20 and 7 and estro-gen receptor analysis in separation of metastaticlobular carcinoma of the breast and metastaticsignet ring cell carcinoma of the gastrointestinaltract. Acta Pathol. Microbiol. Scand. 2000;108:467–472.

Case 84

1. Taylor BA, Wolff BG. Colonic lipomas. Dis. ColonRectum 1987;30:888–893.

2. Chung YFA, Ho Y-H, Nyam DCNK, Leong AFPK,Seow-Choen F. Management of colonic lipomas.ANZ J. Surg. 1998;68:133–135.

3. Church JM. Experience in the endoscopic man-agement of large colonic polyps. ANZ J. Surg.2003;73:988–995.

Case 85

1. Tran KTC, Kuijpers HC, Willemsen WNP, BultenH. Surgical treatment of symptomatic recto-sigmoid endometriosis. Eur. J. Surg. 1996;162:139–141.

2. Macafee CHG, Greer HLH. Intestinal endome-triosis: a report of 29 cases and a survey of the literature. J. Obstet. Gynaecol Br. Commonw.1960;67:539–555.

3. Woods RJ, Herior AG, Chen FC. Anterior wall exci-sion for endometriosis using circular stapler. ANZJ. Surg. 2003;73:647–648.

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Case 86

1. McCready RA, Beart RW. Adult Hirschsprungdisease. Results of surgical treatment in the MayoClinic. Dis. Colon Rectum 1980;23:401–407.

2. Elliot MS, Todd IP. Adult Hirschsprung’s disease:results of the Duhamel procedure. Br. J. Surg.1985;72:884–885.

3. Keighley MRB, Williams NS. Adult Hirschsprung’sdisease, megacolon and megarectum. Surgery ofthe Anus, Rectum and Colon. London: WB Saunders, 1993;Ch 23, pp. 639–674.

4. Gordon PH. An improved technique for theDuhamel operation using the EEA stapler. Dis.Colon Rectum 1983;26:690–692.

5. Wheatley MJ, Wesley JR, Coran AG, Polley TZ Jr.Hirschsprung’s disease in adolescents and adults.Dis. Colon Rectum 1990;33:622–629.

Case 87

1. Hession PR, Rawlinson J, Hall JR, Keating JP,Guyer PB. The clinical and radiological features ofcholecystocolic fistulae. Brit. J. Radiol. 1996;69:804–809.

2. Milson JW, MacKeighan JM. Gallstone/obstructionof the colon. Report of two cases and review ofmanagement. Dis. Colon Rectum 1985;28:367–370.

3. Anseline P. Colonic gallstone ileus. Postgrad. Med.J. 1981;57:62–65.

Case 88

1. Begos DG, Sandor A, Modlin IM. The diagnosis andmanagement of adult intussusception. Am. J. Surg.1997;173:88–94.

2. Nargoney DM, Sarr MG, McIlrath DC. Surgicalmanagement of intussusception in the adult. Ann.Surg. 1981;193:230–236.

3. Azar T, Berger DL. Adult intussusception. Ann.Surg. 1997;226:134–138.

4. Takeuchi K, Tsuzuki Y, And T, Sekihara M, Har T,Takayuki K, Kuwano H. The diagnosis and treat-ment of adult intussusception. J. Clin. Gastroen-terol. 2003;36:18–21.

5. Tan KY, Tan SM, Tan AG, Chen CY, Chng HC,Hoe MN. Adult intussusception: experience inSingapore. ANZ J. Surg. 2003;73:1044–1047.

6. Fenoglio-Preiser CM, Noffsinger AE, Stemmer-mann GN, Lantz PE, Listrom MB, Rilke FO. Nonneoplastic lesions of the colon. Gastrointe-stinal Pathology. An Atlas and Text. 2nd ed.Philadelphia: Lippencott-Raven, 1999;Ch 19, pp.763–908.

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1. Fry RD, Shemesh EI, Kodner IJ, Fleshman JW,Timmcke AE. Perforation of the rectum andsigmoid colon during barium enema examination.Dis. Colon Rectum 1986;32:759–764.

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1. Gallo D, Tebrock C, Rivera D. Intramural cecalhematoma: an unusual complication of colono-scopy. Gastrointest. Endosc. 2003;57:254–257.

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1. Wadman M, Syk I, Elmståhl S. Survival after operation for ischaemic bowel disease. Eur. J. Surg.2000;166:872–877.

2. Killingback M, Barron P, Dent O. Elective resec-tion and anastomosis for colorectal cancer: aprospective audit of mortality and morbidity1976–1998. ANZ J. Surg. 2002;72:689–698.

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1. Killingback M, Barron P, Dent O. Elective resec-tion and anastomosis for colorectal cancer: aprospective audit of mortality and morbidity1976–1998. ANZ. J. Surg. 2002;72:689–698.

2. Stevenson IM, Mansfield AO, Temple JG. Abdom-inal apoplexy. Br. J. Surg. 1978;65:318–320.

3. Kleinsasser IJ. Abdominal apoplexy. Am. J. Surg.1970;120:623–628.

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1. Kim CJ, Yeatman TJ, Coppola D, Trotti A,Williams B, Barthel JS, Dinwoodie W, Karl RC,Marcet J. Local excision of T2 and T3 rectalcancers after downstaging chemoradiation. Ann.Surg. 2001;234:352–358; discussion 358–359.

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1. Cripps WH, The Passage of Air and Faeces fromUrethra. London: JA Churchill Ltd., 1888.

2. Hool GJ, Bokey EL, Pheils MT. Diverticularcoloenteric fistulae. Aust. N. Z. J. Surg. 1981;51:358–359.

3. Killingback M, Barron PE, Dent OF. Electivesurgery for diverticular disease: an audit of surgi-cal pathology and treatment. ANZ J. Surg.2004;74:530–536.

4. Del Pino A, Abcarian H. Colovesical fistulas. In:Welch JP, Cohen JL, Sardella WV, Vignati PV, eds.Diverticular disease. Management of the DifficultCase. Baltimore: Williams and Wilkins, 1998;Ch10, pp. 151–166.

5. Woods RJ, Lavery IC, Fazio VW, Jagelman DG,Weakley FL. Internal fistulas in diverticulardisease. Dis. Colon Rectum 1988;31:591–596.

Case 96

1. Killingback M, Barron P, Dent O. Elective resec-tion and anastomosis for colorectal cancer: aprospective audit of mortality and morbidity1976–1998. ANZ J. Surg. 2002;72:689–698.

2. Vignali A, Fazio VW, Lavery IC, Milsom JW,Church JM, Hull TL, Strong SA, Oakley JR. Factorsassociated with the occurrence of leaks in stapledrectal anastomoses: a review of 1014 patients. J. Am. Coll. Surg 1997,185:105–113.

3. Alberts JCJ, Parvaiz A, Moran BJ. Predicting riskand diminishing the consequences of anastomoticdehiscence following rectal resection. ColorectalDis. 2003;5:478–482.

Case 98

1. Bakx R, Busch OR, van Geldere D, Bemelman WA,Slors JF, Lanschot JJ. Feasibility of early closure ofloop ileostomies: a pilot study. Dis. Colon Rectum2003;46:1680–1684.

2. Tang CL, Seow-Choen F, Fook-Chong S, Eu KW.Bioresorbable adhesion barrier facilitates earlyclosure of the defunctioning ileostomy after rectalexcision: a prospective randomized trial. Dis.Colon Rectum 2003;46:1200–1207.

Case 100

1. Rodabaugh KJ, Bernstein MR, Goldstein DP,Berkowitz RS. Natural history of postterm chorio-carcinoma. J. Reproductive Med. 1998;43:75–80.

2. Anseline PF, Lavery IC, Fazio VW, Jagelman DG,Weakley FL. Radiation injury to the rectum. Ann.Surg. 1981;194:716–724.

3. Parks AG, Allen CLO, Frank JD, McPartlin JF. Amethod of treating post-irradiation rectovaginalfistulas. Br. J. Surg. 1978;65:417–421.

232 References

3. Tjandra JJ, Ng KH. A sprayable hydrogel adhesionbarrier facilitates closure of defunctioning loopileostomy: a randomized trial. Dis. Colon Rectum2004;47:640.

Case 99

1. Schellhammer PF, Jordan GH, El-Mahdi AM.Pelvic complications after interstitial and externalbeam irradiation of urologic and gynecologicmalignancy. World J. Surg. 1986;10:259–268.

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234 Appendix

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Case 18 (pp. 40–41)

Appendix 235

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Case 30 (pp. 66–67)

236 Appendix

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Case 35 (pp. 76–77)

Appendix 237

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Case 43 (pp. 94–95)

238 Appendix

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Case 51 (pp. 112–113)

Appendix 239

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Case 52 (pp. 114–115)

240 Appendix

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Case 58 (pp. 126–127)

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Case 61 (pp. 132–133)

242 Appendix

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Case 62 (pp. 134–135)

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Case 63 (pp. 136–137)

244 Appendix

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Case 64 (pp. 138–139)

Appendix 245

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Case 68 (pp. 148–149)

246 Appendix

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Case 78 (pp. 172–173)

Appendix 247

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Case 81 (pp. 178–179)

248 Appendix

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Case 85 (pp. 186–187)

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Case 86 (pp. 188–189)

250 Appendix

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Case 87 (pp. 190–191)

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Case 94 (pp. 206–207)

252 Appendix

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Figure 95.5: Spontaneousauto-anastomosis with stricture.

Figure 95.4: Anastomoticdehiscence.

Figure 95.2: Shows theanastomotico-vesical/fistula.

Figure 95.3: The extended lowanterior resection (11.1.73).

Case 95 (pp. 208–209)

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Case 97 (pp. 212–213)

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Case 100 (pp. 218–219)

AAbscesses

of the bladder, 94, 95chronic intersphincteric, 155diverticular, 96–97

annular extramural, 98–99chronic diverticulitis-related,

104–105giant diverticulum-related,

100–103retroperitoneal, 206–207

intra-abdominal, Crohn’s disease-related, 120–121

pelvic, giant diverticulumpresenting as, 100–101

pericolic and perirectal, 88–89in right ischiorectal fossa, 90serpiginous mesorectal, 92–93subphrenic, 127

Adenocarcinomaappendiceal, 24–25cavitating, of the transverse colon,

58–59cecal, 192–193colonic, 6–7

with malignant carcinoid tumors,52–53

metastatic breast cancer-related,182–183

colorectal, protracted recurrence of,62–63

jejunal, 10–11metastatic into anal fistula,

158–159rectal, 168–169

metastatic, 72–73, 158–159post-ileorectal anastomosis,

132–133rectosigmoid, as obstructive colitis

cause, 136–137of the sigmoid colon

in apparently benign polyps,32–33

with “eruption” into the rectum,60–61

metastatic, 28recurrence of, 74–75

Adenoma, tubulovillous, 200–201Adhesion-preventing substances,

214

Adhesionseffect on ileostomy closure,

214–215omental, 180–181

Aganglionosis, Hirschsprung’sdisease-related, 188–189

Altemeier operation(rectosigmoidectomy),160–161

Anal canal, spindle cell lipomas of,155

Anal sphincter, gastrointestinalstromal tumor of, 154–155

Anaplastic colon/colorectal cancer,64–65, 66–67

Anastomosiscoloanal, 212–213for diverticulitis with peritonitis,

84–85ileorectal

as blind pouch syndrome cause,14–15, 14–151, 221

for chronic ulcerative colitis,132–133

for familial adenomatouspolyposis, 44–45

for juvenile polyposis, 38rectal cancer development after,

133Anastomotic dehiscence,

postoperativeafter rectal anterior resection,

208–209of the left colon, 212–213

Anastomotic leak, radiological, 90Anemia, iron deficiency, 10, 58, 120,

200Aneurysm, postoperative rupture of,

202Angiomyxoma, aggressive, of the

pelvis, 156–157Anus. See also Anal canal

fistulas ofadenocarcinoma implantation

into, 158–159intersphincteric, 166–167proctitis cystica profunda related,

162–163Apoplexy, postoperative abdominal,

202–203

Appendicitis, acuteCrohn’s disease-related, 115diagnosis at colonoscopy, 18–19

Appendixin Crohn’s disease, 114–115cystadenoma of, 21, 22–23mucocele of, 20–23mucus-producing pathology of,

22–23, 24–25retrocecal, adenocarcinoma of,

24–25Ascending colon

Crohn’s disease of, 114resection of, 110as stricture cause, 110–111,

112–113MALT lymphoma of, 146–147

Ascites, malignant mucinous, 63Atherosclerosis, as spontaneous

abdominal apoplexy riskfactor, 202

Auto-anastomosis, 208–209

BBarium enemas, rectal perforation

during, 196–197Biliary tree, air in, 190–191Bladder

abscess of, 94, 95colovesical fistula of, 208–209desmoid tumor-related

displacement of, 173Blind loop syndrome, 12Blind pouch syndrome

after bowel resection, 12–13ileorectal anastomosis-related,

14–15Bowel resection, as blind pouch

syndrome cause, 12–13Brain tumors, familial adenomatous

polyposis-related, 42Breast cancer, metastatic

differentiated from primary bowelcancers, 182

as linitis plastica cause, 182–183Buttocks

coloperineal fistula opening onto,90

rectal cancer infiltration of, 72–73

Index

255

CCalcification, of desmoid tumors,

172, 173Candidiasis, steroids-related, 110Carcinoid tumors

colon cancer-associated, 52–53colorectal cancer-associated, 6–7ileal, 4–6, 221

colorectal cancer-associated, 6–7intruding, 4–6

CecumMALT lymphoma of, 146–147tumors of, as colonic

intussusception cause,192–193

in ulcerative colitis, 128–129Chemotherapy

for Burkitt’s lymphoma, 145for intra-abdominal desmoid tumor,

172–173Chorionic carcinoma, radiotherapy

for, 218–219Clostridium difficile, as colitis cause,

139Colitis

nongangrenous ischemic, 178–179pseudomembranous, 138–139pseudopolyps associated with,

130–131as splenic flexure deformity cause,

116–117ulcerative

childhood, with rectal cancer,134–135

chronic, ileo-rectal anastomosisfor, 132–133

mucoid cancers associated with,62–63

multiple lymphoma-asociated,148–149

obstructive, 136–137splenic flexure carcinoma

associated with, 76–77toxic megacolon associated with,

128–129, 130–131Colloid carcinoma, 158Colon. See also Ascending colon;

Descending colon; Leftcolon; Right colon; Sigmoidcolon; Transverse colon

diverticulitis-related obstruction of,104–105

intussusception ofcecal tumor-related, 192–193Peutz-Jeghers syndrome-related,

40–41juvenile polyposis of, 36shortened (“hose pipe”), Crohn’s

disease-related, 118–119

ulcerative colitis-relatedperforation of, 128–129

Colon cancer. See also Colorectalcancer

anaplastic, 64–65cavitating, 58–59local recurrence rate of, 74with malignant carcinoid tumors,

52–53Colonoscopy

acute appendicitis diagnosis during,18–19

for ileum carcinoid diagnosis, 4preoperative, failure to assess

pathology in, 206–207as sigmoid colon injury cause,

198–199Colon resection, as mesenteric

thrombosis cause, 200–201Colorectal cancer

Crohn’s disease-related, 127juvenile polyposis-related, 38mucoid, protracted recurrence of,

62–63signet ring, 66–67small bowel carcinoids associated

with, 6–7Constipation, intractable, 162Crescentic fold disease, of the

sigmoid colon, 82–83Crohn’s disease

appendix in, 114–115of the ascending colon, 110–111,

112–113colic, recurrence rate of, 124–125,

227as colon (“hose pipe”) shortening

cause, 118–119as colorectal cancer cause, 127as duodenal stricture cause,

112–113as ileal stricture cause, 110–111ileocecal, as intestinal tuberculosis

mimic, 140–141ileocolic, recurrence rate of,

124–125ileocolic fistulas associated with,

122–123, 124–125ileoileal fistulas associated with,

122–123, 124–125as large intestine obstruction

cause, 126–127presenting as abdominal cancer,

122–123pseudopolyps associated with,

113, 114–115, 120–121, 130

ileocecal, 114–115, 118–119recurrent, 110–111, 124–125

256 Index

pseudopolyposis associated with,120–121

relationship to resection margins,124–125

as “shamrock” deformity cause,116–117

of the terminal ileum, 114–115ulceration morphology in, 108–109

Cystadenocarcinoma, of theappendix, mucoceleassociated with, 21

Cystadenoma, of the appendix,mucocele associated with, 21

Cystsgiant diverticulum presenting as,

102–103hemorrhagic, endometriosis-

related, 186hepatic, 68–69pneumatosis coli-related, 174–175serosal, coexistent with sigmoid

colon cancer, 56–57

DDelorme, Edmond, 34Delorme operation, 34–35Descending colon

Crohn’s disease-related obstructionof, 126–127

lymphoma of, 148–149Desmoid tumors, intra-abdominal,

172–173Disseminated peritoneal

adenomucinosis (DPAM), 22Diverticulitis

chronic, 104–105with colocutaneous fistulas,

208–209with colonic obstruction, 104–105with coloperineal fistulas, 90–91with colovesical fistulas, 208–209dissecting, 96–97

annular extramural, 98–99with mesorectal abscess, 92–93misdiagnosed as sigmoid colon

cancer, 86–87with pericolic and perirectal

abscesses, 88–89with peritonitis, 84–85phlegmonous, 86–87with rectosigmoid cancer, 54–55recurrent, 104–105residual, after colon resection,

206–207of the sigmoid colon, 48–49,

198annular extramural dissecting,

98, 99chronic, 96–97

with colovesical fistula, 94–95,208–209

following resection, 206–207as giant diverticulum, 100–101misdiagnosis of, 86–87as perforation cause, 208–209with peritonitis, 84–85

without inflammation, 80–81Diverticulosis

cecal tumor-related, 192giant diverticulum-related, 102

Diverticulumcolonic, 14–15giant, 100–101, 102–103, 226

Duodenum, Crohn’s disease-relatedstrictures of, 112–113

Dysplasia-associated mass lesion(DALM), 131, 132–133

differentiated from inflamed villousadenoma, 132

EEndometrioma, of the anal canal, 155Endometriosis, intestinal, 186–187Endoscopy, video capsule (VCE), 41Enterocolitis, necrotizing,

pneumatosis coli-related, 175Epstein-Barr virus, 145Erythromycin: pseudomembranous

colitis, 139

FFamilial adenomatous polyposis

(FAP)rectal cancer associated with

ileorectal anastomosis for, 44–45with rectovaginal fistula, 42–43

with unassociated desmoid tumor,172–173

Fat necrosis, omental infarction-related, 180–181

Fecal contamination, operative,176–177

Fibrosis, barium infiltration-related,196–197

Fistulasanal, 72–73

adenocarcinoma implantationinto, 158–159

intersphincteric, 166–167proctitis cystic profunda-related,

162–163cholecystocolic, 190–191cholecystoduodenal, 192colocolic, Crohn’s disease-related,

116colocutaneous

colon perforation-related,208–209

Index 257

diverticulitis-related, 208–209coloperineal, 90–91colovesical, 94–95

of the bladder, 208–209colon perforation-related,

208–209diverticulitis-related, 208–209

enterocutaneous, Crohn’s disease-related, 118–119

fecal, 168–169, 198ileocecal, Crohn’s disease-related,

114–115ileocolic, Crohn’s disease-related,

122–123ileocutaneous, Crohn’s disease-

related, 114–115ileoduodenal, ileocecal

tuberculosis-related, 140–141ileoileal, Crohn’s disease-related,

122–123, 124–125rectovaginal, 42–43

radiotherapy-related, 218–219

GGallbladder, lymphoma of, 150Gallstones, as sigmoid colon

obstruction cause, 190–191Ganglion cells, Hirschsprung’s

disease-related absence of,188–189

Gastrointestinal stromal tumors(GIST), ileal, 8–9

Gastrointestinal tract (GIT) cancer,lymphoma as, 150

GIST (gastrointestinal stromaltumors) ileal, 8–9

Granular cell tumors, of the analcanal, 155

Granulomas, tuberculous, 140–141

HHartmann operation, reversal of,

214–215Hemorrhage, postoperative intra-

abdominal, 202–203Hirschsprung, Harald, 189Hirschsprung’s disease, 188–189Hypertension, as spontaneous

abdominal apoplexy riskfactor, 202

IIleitis, Crohn’s, 115

presenting as abdominal cancer,122–123

presenting as ileocecal tuberculosis,140–141

Ileocecal angle, carcinoid tumor of,4–6

Ileocecal junction, Crohn’s disease-related pseudopolyps of,114–115

Ileorectal anastomosis. SeeAnastomosis, ileorectal

Ileostomy closure, effect of adhesionpathology on, 214–215

Ileumcarcinoid tumors of, 4–7gastrointestinal stromal tumors

(GIST) of, 8–9obstruction of, Crohn’s disease-

related, 122–123terminal

in Crohn’s disease, 110–111,114–115, 118–119

lipoma of, 2–3MALT lymphoma of, 146–147in metastatic linitis plastica,

182–183resection of, in recurrent Crohn’s

disease, 110–111Infarction

intestinal, thrombosis-related,200–201

omental, 180–181Inflammatory bowel disease. See also

Colitis, ulcerative; Crohn’sdisease

as toxic colitis cause, 128Intussusception

Burkitt’s lymphoma-related,144–145

coloniccecal tumor-related, 192–193chronic, 40–41

Pseudokidney sign on x-ray, 192

rectal, 165transverse colon lipoma-related,

184–185, 230Ischemia, colitis-related, 178–179Ischiorectal fossa

adenocarcinoma metastases into,158–159

angiomyxoma of, 156–157

JJejunum, adenocarcinoma of, 10–11

LLanghans’ giant cells, 140Large intestine. See also Ascending

colon; Cecum; Sigmoidcolon; Splenic flexure;Transverse colon

Crohn’s disease-related obstructionof, 126–127

lymphoma, 150

“Large polyp occult carcinoma,”28–29

Left gastric artery, as postoperativehemorrhage source, 202

Left iliac fossa, omental mass in,180–181

Linitis plasticacolorectal, 66–67metastatic, 182–183of the sigmoid colon, 64–65

Lipomasrectal, 46–47of the sigmoid colon, 46–47spindle cell, of the anal canal, 155of the terminal ileum, 2–3of the transverse colon, 184–185

Liver metastases, rectal cancer-related, 68–69

Lung cancer, Peutz-Jeghers syndrome-related, 41

LymphomaBurkitt’s, 144–145diffuse large B cell, 148–149ileocecal, 146–147multiple, 148–149rectal, 150–151

MMacroscopic dysplasia. See Dysplasia-

associated mass lesion(DALM)

MALT B cell tumors, 146–147Meckel’s diverticulum, 12Megacolon, toxic

pseudomembranous colitis-associated, 138–139

ulcerative colitis-related, 128–129,130–131

Mesenchymal tumors, 156–157Mesorectum, serpiginous abscess of,

92–93Metastases

of breast cancerdifferentiated from primary

bowel cancers, 182as linitis plastica cause, 182–183

carcinoid tumor-related, 4hepatic, rectal cancer-related,

68–69implantation into anal fistula,

158–159“mega” lymph node, sigmoid colon

cancer-related, 70–71of rectal adenocarcinoma, 72–73,

158–159rectal cancer-related, 198–199

Middle colic artery, as postoperativehemorrhage source, 202

Morson’s solitary rectal ulcer,162–163

Mucoceles, of the appendix, 20–21ruptured, 22–23

Mucoid cancer, colorectal, protractedrecurrence of, 62–63

Mucus-producing pathology, of theappendix, 22–23, 24–25

Muscle dehiscence, post-colonoscopy,198–199

Myenteric plexus, in Hirschsprung’sdisease, 188–189

NNecrosis

fat, omental infarction-related,180–181

ischemicas anastomosis dehiscence cause,

212–213submucosal lipoma-related,

184–185rectal, 168–169

Neurofibrous tumors, of the analcanal, 155

OOleogranuloma, of the anal canal,

155Omentum, infarction of, 180–181Ovaries, cystic, 186Oxygen therapy, for pneumatosis coli,

174–175

PPalliative debulking procedure,

206–207Pelvis

abscess of, giant diverticulumpresenting as, 100–101

aggressive angiomyxoma of,156–157

Perineum, radiotherapy-relatedtelangiectasia of, 218

Perirectal tissues, barium perforationof, 196–197

Peritoneal mucinous carcinomatosis(PMCA), 22, 63

Peritonitisdiverticulitis-related, 84–85fecal, 176–177radiotherapy injury-related,

216–217Peutz-Jeghers syndrome, as chronic

colonic intussusceptioncause, 40–41

Pigmentation, in Peutz-Jegherssyndrome patients, 40, 41

258 Index

Pneumatosis coli, 174–175Polypoid prolapsing mucosal folds,

82–83Polyposis

familial adenomatous (FAP)rectal cancer associated with

ileorectal anastomosis for,44–45

with rectovaginal fistula, 42–43

with unassociated desmoidtumor, 173

juvenile, 36–37in adult, 38–39

Polyps. See also Polyposis;Pseudopolyps

adenomatous sigmoid, 6–7“benign”

giant, of the rectum and sigmoidcolon, 30–31

of the sigmoid colon, 30–31,32–33

cecal, 192–193inflammatory, 130–131“large polyp occult carcinoma,”

28–29Peutz-Jeghers syndrome-related,

40–41rectal

juvenile polyposis-related, 36–37,38–49

as necrotizing infection cause,168–169

with rectal prolapse, 34–35of the terminal ileum, 2–3

Proctitis cystica profunda, 34–35,162–163, 165

Pseudomyxoma peritonei, 21, 25, 62,63

Pseudopolypscolitis-related, 130–131Crohn’s disease-related, 113,

114–115, 120–121, 130ileocecal, 114–115, 118–119

large, of the sigmoid colon, 82–83

RRadiotherapy

pelvic, as sigmoid colon perforationcause, 216–217

preoperative, for rectal cancer,204–205

as rectovaginal fistula cause,218–219

Rectal canceranterior resection of, as

anastomotic dehiscencecause, 208–209, 212–213

childhood ulcerative colitis-associated, 134–135

differentiated from proctitis cysticprofunda, 162–163

diverticulitis associated with,54–55

excision of, with rectal prolapse,160–161

familial adenomatous polyposis-associated

ileorectal anastomosis for, 44–45

with rectovaginal fistula, 42–43following polyp removal, 168–169with liver metastases, 68–69local excision of, after radiotherapy,

204–205lymphoma, 150–151metastatic, 198–199

to the buttock, 72–73post-ileorectal anastomosis, 133

Rectal prolapseDelorme’s operation for, 34–35with juvenile polyposis, 36–37proctitis cystica profunda-related,

162–163rectal carcinoma local excision,

160–161Rectal prolapse syndrome, 165Rectopexy, for solitary rectal ulcer

syndrome, 164–165Rectosigmoid, adenocarcinoma of,

136–137Rectosigmoidectomy (Altemeier

operation), 160–161Rectum. See also Anal canal; Anus

adenocarcinoma of, 136–137barium perforation of, 196–197“benign” polyps of, 30–31desmoid tumor-related compression

of, 172involvement in intestinal

endometriosis, 186–187juvenile polyposis of, 36–37

in an adult, 38–39lipomas in, 46–47polyps of, with necrotizing

infection, 168–169sigmoid cancer “eruption” into,

60–61strictures of, rectopexy treatment

of, 164–165Renal calculus, recurrent Crohn’s

disease-associated, 110Right colon

lymphoma of, 146–147resection of, in recurrent Crohn’s

disease, 110

Index 259

SSacrum, desmoid tumor of, 172–173Sexual development, Crohn’s disease-

related inhibition of,118–119

“Shamrock” deformity, Crohn’sdisease-related, 116–117

Sigmoid colon“benign” polyps of, 30–31, 32–33,

222cancer of

adenocarcinoma, 6, 28, 32–33,60–61, 74–75

anaplastic, 64–65in apparently benign polyp,

32–33with coexistent diverticulitis,

54–55with coexistent serosal cysts,

56–57with “eruption” into the rectum,

60–61local recurrence of, 74–75with “mega” lymph node

metastasis, 70–71metastatic into anal fistula,

158multiple lymphoma, 148–149as rectal bleeding cause,

202–203colonoscopy-related injury to,

198–199crescentic fold disease of, 82–83diverticular disease of, 80–81,

82–83diverticulitis of, 48–49, 198

annular extramural dissecting,98, 99

chronic, 96–97with colovesical fistula, 94–95,

208–209following resection, 206–207as giant diverticulum, 100–101misdiagnosis of, 86–87as perforation cause, 208–209with peritonitis, 84–85

gallstone-related obstruction of,190–191

in Hirschsprung’s disease, 188–189in intestinal endometriosis,

186–187“large occult polyp carcinoma” of,

28–29large pseudopolyp of, 82–83lipomas of, 46–47perforation of

diverticulitis-related, 208–209radiation injury-related, 216–217

stercoral ulceration-related,176–177

pneumatosis coli of, 174–175Signet ring cells, 136Signet ring colorectal cancer, 66–67“Silent cancer,” rectosigmoid, 54–55SMAD4 gene mutations, 36Small intestine. See also Duodenum;

Ileum; JejunumCrohn’s disease-related obstruction

of, 124–125lymphoma of, 150thrombosis-related infarction of,

200–201tumors of, 2–3

Solitary rectal ulcer syndrome(SRUS), 164–165

Splenic artery, as postoperativehemorrhage source, 202

Splenic flexureCrohn’s disease-related “shamrock”

deformity of, 116–117in ischemic colitis, 178

Splenic flexure carcinoma, thoraco-abdominal approach to, 76–77

SRUS (solitary rectal ulcersyndrome), 164–165

Stomach cancer, 150Strictureplasty, in small bowel-

obstructive Crohn’s disease,122

Superior mesenteric artery, aspostoperative hemorrhagesource, 202

TTelangiectasia, radiotherapy-related,

218Thoraco-abdominal approach, to

splenic flexure carcinoma,76–77

Thrombosis, mesenteric venous, aftercolon resection, 200–201

Thyroidectomy, preoperative,206–207

Transverse coloncancer of

cavitating, 58–59ulcerating, 200–201

Crohn’s disease-related deformityof, 122–123

Crohn’s disease-related perforationof, 126–127

lipomas of, 184–185“pouch” deformity of, 119

Trimethoprim, pseudomembranouscolitis, 139

Tuberculosis, ileocecal, 140–141

UUlceration. See also Colitis,

ulcerativeCrohn’s disease-related,

108–109diverticulitis-related, 104–105ileorectal anastomosis-related,

14–15

ischemic, 178–179of the left colon, 212–213Morson’s solitary rectal,

162–163necrotic, anorectal, 204–205radiotherapy injury-related,

216–217rectal, 164–165

260 Index

stercoral, 176–177of transverse colon cancer, 200–201

VVagina, desmoid tumor-related

compression of, 172Volvulus, pneumatosis coli-

associated, 175