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Surgical Treatment of Colorectal Problems in Children

Alberto Peña Andrea Bischo
Alberto Peña
Andrea Bischo
Surgical Treatment of Colorectal Problems in Children Alberto Peña Andrea Bischo 123
Surgical Treatment of Colorectal Problems in Children Alberto Peña Andrea Bischo 123
Surgical Treatment of Colorectal Problems in Children Alberto Peña Andrea Bischo 123

123

Surgical Treatment of Colorectal Problems in Children

Alberto Peña • Andrea Bischoff

Surgical Treatment of Colorectal Problems in Children

Alberto Peña • Andrea Bischoff Surgical Treatment of Colorectal Problems in Children

Alberto Peña Pediatric Surgery Colorectal Center for Children Cincinnati Children’s Hospital Cincinnati, OH USA

Andrea Bischoff Pediatric Surgery Colorectal Center for Children Cincinnati Children’s Hospital Cincinnati, OH USA

ISBN 978-3-319-14988-2 DOI 10.1007/978-3-319-14989-9

Library of Congress Control Number: 2015937190

ISBN 978-3-319-14989-9

(eBook)

Springer Cham Heidelberg New York Dordrecht London © Springer International Publishing Switzerland 2015 This work is subject to copyright. All rights are reserved by the Publisher, whether the whole or part of the material is concerned, specifically the rights of translation, reprinting, reuse of illustrations, recitation, broadcasting, reproduction on microfilms or in any other physical way, and transmission or information storage and retrieval, electronic adaptation, computer software, or by similar or dissimilar methodology now known or hereafter developed. The use of general descriptive names, registered names, trademarks, service marks, etc. in this publication does not imply, even in the absence of a specific statement, that such names are exempt from the relevant protective laws and regulations and therefore free for general use. The publisher, the authors and the editors are safe to assume that the advice and information in this book are believed to be true and accurate at the date of publication. Neither the publisher nor the authors or the editors give a warranty, express or implied, with respect to the material contained herein or for any errors or omissions that may have been made.

Printed on acid-free paper

Springer International Publishing AG Switzerland is part of Springer Science+Business Media (www.springer.com)

To our parents To our children To all children born with congenital anomalies

Foreword I

The care of infants and children with anorectal malformations and disorders has long been an important component of the pediatric surgeon’s practice. Information concerning the recognition and management of these relatively common conditions dates back hundreds of years. Progress during the past half-century due to advances in imaging, both prenatal and postnatal ana- tomic and embryologic studies, improved detection of associated anomalies, the availability of antibiotics, and improvements in overall care resulted in survival of most of the patients, but the functional outcomes following attempted surgical correction of these conditions were not optimal and remained a challenge. While children’s surgeons worldwide recognized the guarded outcomes particularly related to establishing continence, it was dif- ficult to reach a consensus on classification, type of procedure, and methods to assess outcomes and compare results. Following the early work of Douglas Stephens and Durham Smith, many surgeons were successful in achieving good outcomes for most infants with imperforate anus and a perineal fistula and girls with a rectoforchette (ves- tibular) fistula or those without a fistula; however, in those defects where the rectal atresia ended with a recto-urethral or bladder fistula and in instances of cloacal anomalies results were often poor. Interest in these cases peaked when Peter de Vries and Alberto Peña first described the posterior sagittal anorectoplasty (PSARP) procedure in 1980. While there remains some hon- est differences of opinion regarding the operative approach to some cases and the success rates, surgeons throughout the world have employed the PSARP in many patients and there has been a cooperative international effort to mod- ify the old Wingspread and Peña Classifications and adopt the Krickenbeck consensus to identify and classify the various anomalies and assess outcomes. Dr. Peña has focused his career on caring for infants and children with colorectal problems both here and abroad. He is clearly recognized as one of the leading experts in the field and has made major contributions to the care of these children. This textbook Surgical Treatment of Colorectal Problems in Children co-edited by Dr. Andrea Bischoff is truly a labor of love and reflects the vast personal experience of the authors. As noted by the authors in their forwarding remarks, this is not a data-driven, evidenced-based text- book, but rather an observational personalized approach based on their

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Foreword I

considerable experience in the care of these children. The book contains 27 chapters that cover the broad spectrum of colorectal conditions from the com- plex to the mundane (fistula-in-ano, fissure, hemorrhoids, bowel preparation) as well as an historical background, imaging, the role of minimally invasive surgery, motility disorders, and perhaps more importantly bowel manage- ment. To his credit Dr. Peña has been a strong advocate of establishing mul- tidisciplinary centers for colorectal disorders to aid children with motility disorders and those that require bowel management programs especially in the postoperative period. The textbook also includes chapters on Hirschsprung disease and reoperative surgery but excludes any information on inflamma- tory bowel disease (IBD). While this might be viewed as a weakness of the book, it is noted that the authors intentionally left out the IBD chapter as they did not feel their experience in this area was adequate. One of the strengths and more unique aspects of the textbook is its use of animation and careful attention to details that illustrate the surgical tech- niques employed in the operative care of the patients. The illustrations in the book are superior. Another important area of emphasis in the text is the thor- ough evaluation of the status of the sacrum and spinal cord in determining outcomes. The textbook by Peña and Bischoff is an extensive reference on colorectal disorders in children that will be useful to those both in training and practice and provides insights into these conditions based on their enormous experi- ence with these cases. It will be an excellent resource and valuable addition to a pediatric surgeon’s personal library.

Jay L. Grosfeld Indiana University School of Medicine Indianapolis , IN , USA

Foreword II

In 1982, Prof. Alberto Pena made an outstanding contribution to the manage- ment of anorectal malformations by introducing the procedure of posterior sagittal anorectoplasty which in subsequent years has become the classic approach for the treatment of anorectal malformations. The book Surgical Treatment of Colorectal Problems in Children represents over 30 years of Prof. Pena’s experience in dealing with colorectal disorders in children. The pediatric surgical community will greatly appreciate the efforts of Prof. Alberto Pena and Dr. Andrea Bischoff in putting together their vast experience in a valuable and easily readable book. The authors provide a comprehensive description of operative techniques for various colorectal malformations in children. The text is organised in a systematic manner pro- viding step-by-step detailed practical advice on operative approach on the management of these congenital malformations. The strength of the book is that it is based on the experience and best belief of the authors which goes on to show that the successful correction of colorectal anomalies requires a thor- ough understanding of the problem and the pathological anatomy as well as meticulous attention to surgical techniques. An interesting and unique feature of this book is the generous use of high quality colour illustrations to clarify and simplify various operating tech- niques. Another unique feature of the book which has not been used before in a pediatric surgical text is the use of sophisticated computer animation for the diagnostic accuracy required for the effective treatment of anorectal malformations. I congratulate the authors for producing the most comprehensive and well- documented text ever written on the surgical treatment of colorectal problems in children. This book provides an authoritative and complete account of vari- ous colorectal problems in children. I hope that the trainees as well as the established pediatric surgeons, pediatric urologists and pediatricians will find this textbook useful as a guide when dealing with colorectal problems.

Dublin, Ireland

Prem Puri

Pref ace

It is a great pleasure for us to present to the consideration of the pediatric surgical community this book on the surgical treatment of colorectal prob- lems in children. From the time of the first description of the posterior sagittal anorecto- plasty for the treatment of anorectal malformations in 1982 until the publica- tion of this book, we were able to accumulate a very large series of cases of anorectal malformations, with no similar precedent. The experience gained has been invaluable. We wanted to share our experience with all pediatric surgeons, particularly the young generation. We hope that they will find in this book a guide to repair anorectal malformations and other disorders such as Hirschsprung’s disease and idiopathic constipation. We hope that it will benefit many children all over the world. From the beginning of our experience, we realized that we were confront- ing a very unusual kind of difficulty, and that is the fact that we were working in an anatomical area that was not well known by most pediatric surgeons. Prior to the posterior sagittal approach, the patients were surgically explored either through the abdomen or through the perineum with preconceived ideas about the anatomical area and without a direct exposure to the intrinsic anat- omy of these defects. After 1982, the descriptions of our surgical technique were not like descriptions of any other surgical technique, in which the sur- geons already know the anatomy, for instance, the mediastinum or the intra- abdominal organs. In dealing with anorectal malformations, we were seeing for the first time, directly, the anatomy of these defects, and soon, we learned that we were dealing with a spectrum of malformations and that each specific type of defect would require a different surgical maneuver to be repaired. In 1982, we presented our “new approach” (posterior sagittal) to one of the master pediatric surgeons, Dr. Hardy Hendren. He encouraged us to continue using the approach but emphasized the importance of presenting our tech- nique with better, rather impeccable, audiovisual material in order to be suc- cessful in introducing this approach and to gain the acceptance of the pediatric surgical community. As a consequence, we have been making a great effort to document our presentations with high-quality photographic material including videos and animations. The reader, therefore, will find that this book is a very graphic one. We believe that it is extremely important to document with good illustra- tions and photographs all that we have learned. We want this to be essentially

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Preface

a practical book, a reliable guide for all pediatric surgeons and pediatric urologists.

A very important motivation for us to write this book is the fact that we

receive many patients who underwent technically deficient operations in other parts of the world and suffered serious complications. Therefore, this book puts particular emphasis on the surgical technique and attention to details as well as the importance of observing meticulous delicate operations. Since anorectal malformations represent a spectrum, it is difficult for a gen-

eral pediatric surgeon to be exposed to all the different anatomical variants of these defects. There is not a single technique to repair all anorectal malforma- tions; each type of defect represents a different anatomical variant, and the surgeon must be prepared to deal with it. We also wanted to put emphasis on clarity and simplicity. All cases discussed in this book were operated by the senior author and by the junior author during the last 5 years.

In order to make all the concepts more understandable and simple, dealing

with an anatomical area that is not well known by most surgeons, we intro- duced another modality of teaching, represented by 27 animations, that we hope will simplify the understanding of the surgical techniques and concepts expressed in this book. We are very proud to present an entire book with illustrations made by a single person. All illustrations in this book were made by Ms. Lois Barnes under the personal guidance of the senior author. Ms. Barnes is an excellent medical illustrator and old friend with whom we have been working for 30 years. It took many years to establish a unique form of communication between the medical illustrator and us. Due to the large number of illustra- tions that she performed under our guidance, it is a great pleasure nowadays to have a common language between a surgeon and an artist. We speak the same language. Therefore, the illustrations made in the last few years repre- sented much less effort from both surgeons and the illustrator. The animations required, again, many hours of working together with ingenious, intelligent experts in computer animations and establishing a com- munication between a surgical mind and an engineer, computer expert type of mind. We believe that in the future, teaching surgery will be highly simplified with this kind of audiovisual material. The reader will find that even though the book is related to the surgical treatment of colorectal problems in children, we did not include inflamma- tory bowel disease. The reason for that is simple: we do not have enough experience as to be able to say something new and (or) different from what is already written in the enormous literature on the subject. The reader will also be surprised to find that even though this book was finished in November 2014, it is not considered an “evidence-based surgical book.” It is rather a book based on personal observations made through a large experience in the management of these problems. We understand very well that the future in surgery will be related very much to the concept of “evidence-based” procedures. However, in dealing with anorectal malforma- tions, some surgeons have tried to compare the results of the posterior sagittal anorectoplasty with other techniques; they found a serious limitation: the

Preface

xiii

series are not nearly comparable. The number of cases that we accumulated over the last 30 years has no precedent. If one tries to compare this technique with others, we find the problem that the numbers are 10 or 100 times greater in our series. In addition, most of the publications on anorectal malforma- tions, even in the year 2014, unfortunately are still presented following the old nomenclature discussing “high,” “intermediate,” and “low” malforma- tions. This makes it impossible to compare the results. Through the entire book, we emphasize the importance of recognizing the existence of different, specific types of defects, each one requiring different surgical maneuvers and each one with a different functional result. In addition, we keep emphasizing the importance of describing the characteristics of the sacrum and the anoma- lies of the spinal cord if we want to discuss results. Trying to compare our results with old techniques is an impossible task. We are more concerned with trying to be sure that all new generations of pediatric surgeons learn the real, true, intrinsic anatomy of anorectal malformations and learn to repair these malformations in an optimal way. Finally, we would like very much to be able to transmit to the young gen- eration of pediatric surgeons our passion for delicate, meticulous, fine surgi- cal technique, which is the essence of our specialty. There is no other medical or surgical specialty as curative as pediatric surgery. We become pediatric surgeons because we like the idea that we could repair with our hands a seri- ous congenital malformation and change the quality of life of a baby. It is extremely important for us to recognize that a mistake in the management of these patients will leave sequelae for life. This book also puts a special emphasis on the detailed repair of those mal- formations that are considered to have a good functional prognosis. One thing that we cannot afford is to take care of a child with a malformation with a good functional prognosis, perform a technically deficient surgical proce- dure, and provoke serious, permanent sequelae as a consequence of our bad operation.

Cincinnati , OH , USA

Alberto Peña

Cincinnati , OH , USA

Andrea Bischoff

Acknowledgments

We would like to sincerely thank innumerable individuals for contributing ideas and encouragement and facilitating in different ways to make this book a reality. The following names came to our minds because of their very prom- inent daily participation in our task. We want to express our gratitude to hun- dreds of surgeons, not mentioned here, from all over the world who trusted us to operate on their little patients. They made it possible for us to accumulate the experience that we want to share with the new generation. We are sure that we have missed many important names of individuals that contributed to finish this work; please forgive us for the omission. Richard Azizkhan Daniel von Allmen Michael Fischer Prem Puri Lois Barnes Jeffrey Cimprich Ken Tegtmeyer Ren Wilkey Mikeisha Isome Kennethia Banks Borden Jennifer Hall Alicia Vincent Teri Martini Bruno Martinez Jason Frischer Belinda Dickie Nurses and administrative assistants of the Colorectal Center Elizabeth Stautberg George Rodriguez John Cardone Michael Rose Walter Dibbins

Sincerely, Alberto Peña and Andrea Bischoff

Contents

1 History of the Treatment of Anorectal Malformations

 

1

1.1 Introduction

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1.2 The

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2 Basic Anatomy and Physiology of Bowel Control

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2.1 Internal

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2.2 General Anatomic Principles in Anorectal

 

Malformations

 

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2.3 Nerves

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2.4 Blood Supply

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2.5

Basic Physiology Principles of Bowel Control

 

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3 Prenatal Diagnosis

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3.1 Male

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3.1.1 Abnormal Sacrum

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3.1.2 Tethered Cord

 

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3.1.3 Absent

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3.1.4 Vertebral Anomalies

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3.1.5 Hydronephrosis

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3.2 Female

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3.2.1 Dilated Bowel and Intraluminal Calcifications

 

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3.2.2 Pelvic

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3.2.3 Cloacal Exstrophy

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References.

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4 Neonatal Management

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4.1 Introduction

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4.2 Most Common

 

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4.3 Answering the Two Most Important Questions

 

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4.4 Physical

 

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4.4.1

Male

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4.5 Female Babies

 

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4.6 Neonatal Management

 

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4.7 Cloacal Exstrophy

 

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References.

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xviii

Contents

5 Colostomy

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5.1 Introduction

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5.2 Stoma Locations.

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5.3 Ileostomies .

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5.4 To Divert or Not to Divert, That Is the Question

 

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5.5 Recommended Types of Colostomies

 

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5.5.1

Newborn Babies with Anorectal Malformations

 

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5.6

Left Transverse Colostomy

 

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5.7

Cecostomies .

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5.8

Creation of a Colostomy

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5.8.1

Surgical Technique

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5.9

Colostomy in Cases of Cloaca with Hydrocolpos

 

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5.10 Other Types of

 

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5.11 .

Colostomy Care

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5.12 Colostomy

Closure.

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5.13 Surgical

Technique.

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5.14 Errors and Complications in Colostomies

 

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5.15 The Case of Upper Sigmoidostomy

 

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5.16

Prolapse

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5.17 Surgical Treatment for

 

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5.18 Malposition of the Stomas

 

72

References.

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74

6 Imaging

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77

6.1 Introduction

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77

6.2 Prenatal Diagnosis

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77

6.3 Neonatal

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77

6.4

Determination of the Fistula Location Prior

 

to the Colostomy

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6.4.1

Anatomic Facts and

 

82

6.5 The Old Invertogram

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86

6.6 High-Pressure Distal

 

87

6.7 Technique .

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6.8 Most

Common

Errors.

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6.9

Not Showing the Coccyx and the Sacrum During the Fluoroscopy Studies

 

91

6.10

Distal Colostogram in Female Patients

 

93

6.11

Distal Colostogram in

 

93

6.12

Monitoring Constipation

 

95

6.13

Radiology During the Bowel Management

 

96

6.14

Monitoring the Urinary

 

96

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98

7 Bowel Preparation in Pediatric Colorectal Surgery

 

101

7.1 Major

Procedures .

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102

7.2 Primary Procedures for the Treatment of Anorectal Malformation During the Newborn Period

 

103

7.3 Primary Pull-Through in Newborn Patients with Hirschsprung’s Disease

 

104

Contents

xix

 

7.4 Patients with Hirschsprung’s Disease with Enterocolitis After the Neonatal Period

 

104

7.5 Patients with Hirschsprung’s Disease Beyond the Neonatal Period, Without Enterocolitis

 

105

7.6

Colostomy

Closures .

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