Ebook Surgical treatment of colorectal problems in children: Part 1

Part 1 book “Surgical treatment of colorectal problems in children” has contents: History of the treatment of anorectal malformations, basic anatomy and physiology of bowel control, prenatal diagnosis, neonatal management, bowel preparation in pediatric colorectal surgery,… and other contents.

Surgical Treatment

of Colorectal Problems

in Children

Alberto Peña Andrea Bischoff

123

Surgical Treatment of Colorectal Problems in Children

Alberto Peña • Andrea Bischoff

Surgical Treatment

of Colorectal Problems

in Children

ISBN 978-3-319-14988-2 ISBN 978-3-319-14989-9 (eBook)

DOI 10.1007/978-3-319-14989-9

Library of Congress Control Number: 2015937190

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, specifi cally the rights of translation, reprinting, reuse of illustrations, recitation, broadcasting, reproduction on microfi lms 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 specifi c 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 )

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

To our parents

To our children

To all children born with congenital anomalies

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-

fi cult to reach a consensus on classifi cation, 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 fi stula and girls with a rectoforchette (ves-tibular) fi stula or those without a fi stula; however, in those defects where the rectal atresia ended with a recto-urethral or bladder fi stula and in instances of cloacal anomalies results were often poor Interest in these cases peaked when Peter de Vries and Alberto Peña fi rst 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 ify the old Wingspread and Peña Classifi cations 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 fi eld 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

refl ects the vast personal experience of the authors As noted by the authors

in their forwarding remarks, this is not a data-driven, evidenced-based book, but rather an observational personalized approach based on their

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 (fi stula-in-ano, fi ssure, 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 infl

amma-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 I

In 1982, Prof Alberto Pena made an outstanding contribution to the ment of anorectal malformations by introducing the procedure of posterior sagittal anorectoplasty which in subsequent years has become the classic

manage-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 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 documented text ever written on the surgical treatment of colorectal problems

well-in children This book provides an authoritative and complete account of ous colorectal problems in children I hope that the trainees as well as the established pediatric surgeons, pediatric urologists and pediatricians will fi nd this textbook useful as a guide when dealing with colorectal problems

Foreword II

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 fi rst description of the posterior sagittal 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 fi nd in this book a guide to repair anorectal malformations and other disorders such

anorecto-as Hirschsprung’s diseanorecto-ase and idiopathic constipation We hope that it will benefi t many children all over the world

From the beginning of our experience, we realized that we were ing a very unusual kind of diffi culty, and that is the fact that we were working

confront-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 fi rst time, directly, the anatomy of these defects, and soon, we learned that we were dealing with a spectrum of malformations and that each specifi c 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 fi nd 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

Pref ace

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 defi cient 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 diffi cult 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 simplifi ed

with this kind of audiovisual material

The reader will fi nd that even though the book is related to the surgical

treatment of colorectal problems in children, we did not include infl

amma-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 fi nd that even though this book was

fi nished 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

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 fi nd the problem that the numbers are 10 or 100 times greater

in our series In addition, most of the publications on anorectal 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, specifi c 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 eration of pediatric surgeons our passion for delicate, meticulous, fi ne surgi-cal technique, which is the essence of our specialty There is no other medical

gen-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 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 defi cient surgical proce-dure, and provoke serious, permanent sequelae as a consequence of our bad operation

prom-to operate on their little patients They made it possible for us prom-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

fi nish this work; please forgive us for the omission

1 History of the Treatment of Anorectal Malformations 1

1.1 Introduction 1

1.2 The Early Times 2

References 14

2 Basic Anatomy and Physiology of Bowel Control 17

2.1 Internal Sphincter 20

2.2 General Anatomic Principles in Anorectal Malformations 20

2.3 Nerves 22

2.4 Blood Supply 22

2.5 Basic Physiology Principles of Bowel Control 23

References 24

3 Prenatal Diagnosis 27

3.1 Male Fetuses 27

3.1.1 Abnormal Sacrum 28

3.1.2 Tethered Cord 28

3.1.3 Absent Kidney 28

3.1.4 Vertebral Anomalies 29

3.1.5 Hydronephrosis 29

3.2 Female Fetuses 30

3.2.1 Dilated Bowel and Intraluminal Calcifi cations 30

3.2.2 Pelvic Cystic Mass 30

3.2.3 Cloacal Exstrophy 30

References 31

4 Neonatal Management 33

4.1 Introduction 33

4.2 Most Common Scenario 33

4.3 Answering the Two Most Important Questions 33

4.4 Physical Examination 35

4.4.1 Male Patients 35

4.5 Female Babies 39

4.6 Neonatal Management 41

4.7 Cloacal Exstrophy 46

References 47

Contents

5 Colostomy 49

5.1 Introduction 49

5.2 Stoma Locations 51

5.3 Ileostomies 52

5.4 To Divert or Not to Divert, That Is the Question 53

5.5 Recommended Types of Colostomies 54

5.5.1 Newborn Babies with Anorectal Malformations 54

5.6 Left Transverse Colostomy 55

5.7 Cecostomies 55

5.8 Creation of a Colostomy 55

5.8.1 Surgical Technique 55

5.9 Colostomy in Cases of Cloaca with Hydrocolpos 59

5.10 Other Types of Colostomies 60

5.11 Colostomy Care 60

5.12 Colostomy Closure 61

5.13 Surgical Technique 62

5.14 Errors and Complications in Colostomies 65

5.15 The Case of Upper Sigmoidostomy 68

5.16 Prolapse 70

5.17 Surgical Treatment for Prolapse 71

5.18 Malposition of the Stomas 72

References 74

6 Imaging 77

6.1 Introduction 77

6.2 Prenatal Diagnosis 77

6.3 Neonatal Imaging 77

6.4 Determination of the Fistula Location Prior to the Colostomy 82

6.4.1 Anatomic Facts and Timing 82

6.5 The Old Invertogram 86

6.6 High-Pressure Distal Colostogram 87

6.7 Technique 88

6.8 Most Common Errors 90

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 Cloacas 93

6.12 Monitoring Constipation 95

6.13 Radiology During the Bowel Management Program 96

6.14 Monitoring the Urinary Tract 96

References 98

7 Bowel Preparation in Pediatric Colorectal Surgery 101

7.1 Major Procedures 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

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 105

7.7 Patients with a Colostomy Who Will Have a Repair of an Anorectal Malformation 105

References 105

8 Recto-perineal Fistula 107

8.1 Defi nition, Frequency, and Prognosis 107

8.2 Associated Defects 109

8.3 Diagnosis 110

8.3.1 Female Patients 110

8.3.2 Male Patients 110

8.4 Management 117

8.5 Dilatations 117

8.6 Cutback Operation 117

8.7 Minimal Posterior Sagittal Anoplasty 118

8.7.1 Male Patients 118

8.7.2 Surgical Technique 119

8.7.3 Female Patients 122

8.8 Postoperative Care 123

References 125

9 Rectourethral Bulbar Fistula 129

9.1 Introduction 129

9.2 Associated Defects 129

9.3 Posterior Sagittal Anorectoplasty 132

9.4 Surgical Technique 132

9.5 Functional Results 148

References 148

10 Rectourethral Prostatic Fistula 151

10.1 Introduction 151

10.2 Associated Defects 151

10.3 Surgical Repair 153

10.4 Posterior Sagittal Anorectoplasty 153

10.5 Postoperative Care and Functional Results 161

References 161

11 Recto-bladder Neck Fistula 163

11.1 Defi nition and Frequency 163

11.2 Associated Defects 164

11.2.1 Sacral Defects 164

11.2.2 Spinal-Associated Defects 164

11.2.3 Urologic-Associated Defects 164

11.2.4 Gastrointestinal-Associated Defects 165

11.2.5 Neurosurgical-Associated Defects 165

11.2.6 Cardiovascular-Associated Defects 165

11.2.7 Other Associated Defects 165

Contents

11.3 Diagnosis 165

11.4 Treatment 166

11.4.1 Colostomy 166

11.4.2 Main Repair 166

11.4.3 Laparotomy 168

11.4.4 Laparoscopy 178

11.5 Special Problems 179

11.5.1 Dealing with Inadequate Colostomies (Too Distal) 179

11.6 Functional Results 179

11.6.1 Fecal Control 179

11.6.2 Urinary Control 180

References 181

12 Imperforate Anus Without Fistula in Males and Females 183

12.1 Introduction 183

12.2 Anatomic Characteristics 184

12.3 Main Repair 185

12.4 Function and Results 187

References 187

13 Minimally Invasive Approach to Anorectal Malformations 189

13.1 Introduction 189

13.2 Males 192

13.3 Females 194

References 196

14 Rectal Atresia 201

14.1 Treatment 202

14.2 Surgical Repair 202

References 204

15 Rectovestibular Fistula 205

15.1 Defi nition/Frequency 205

15.2 Associated Defects 207

15.2.1 Sacral 208

15.2.2 Spinal 208

15.2.3 Urologic 208

15.2.4 Gynecologic 208

15.2.5 Gastrointestinal 209

15.2.6 Tethered Cord 209

15.2.7 Cardiovascular 209

15.3 Diagnosis 211

15.4 Treatment 212

15.4.1 Colostomy or No Colostomy 212

15.5 Main Repair 213

15.6 Complications 219

15.7 Functional Results 219

15.8 Reoperations in Patients with Vestibular Fistula 220

15.9 Surgical Technique 222

15.10 Rectovestibular Fistula with Normal Anus 223

References 223

Contents

16 Cloaca, Posterior Cloaca and Absent Penis Spectrum 225

16.1 Cloaca 22516.1.1 Defi nition and Management 22516.1.2 Urologic Concerns 26016.1.3 Gynecologic Concerns 26116.1.4 Reoperations 26216.1.5 Transpubic Approach 26816.2 Posterior Cloaca and Absent Penis Spectrum 27016.2.1 Surgical Repair 27516.2.2 Surgical Repair of the 2-Perineal-Orifi ce

Variant of the Posterior Cloacal Spectrum 27616.2.3 Posterior Cloaca and Absent Penis 276References 279

17 Cloacal Exstrophy and Covered Cloacal Exstrophy 285

17.1 Neonatal Approach 28917.2 Pull-Through or “Permanent Stoma” 29117.3 Covered Cloacal Exstrophy 293References 295

18 General Principles for the Postoperative Management

of Patients with Anorectal Malformations 299

18.1 General Care 29918.2 Local Care 30018.3 Anal Dilatations 30018.4 Avoiding Constipation 30318.5 Toilet Training 305

Diaper Rash 32920.10 Bowel Management Through a Stoma 330References 331

21 Operations for the Administration of Antegrade Enemas 333

21.1 Introduction 33321.2 Our Preferred Technique 33421.3 Surgical Technique: Continent Appendicostomy 33621.4 Continent Neo-appendicostomy 339References 345

Contents

22 Reoperations 349

22.1 Introduction 349

22.2 Reoperations to Improve Bowel Control 350

22.3 Reoperations Performed After Failed Attempted

Repair (Catastrophes) Males 356

22.4 Reoperations for Postoperative Recto- urinary Fistula 357

22.4.1 Recurrent Fistula (17 Cases) 357

22.4.2 Persistent Rectourethral Fistula (24 Cases) 359

22.4.3 Acquired Fistula (9 Cases) 360

22.5 Posterior Urethral Diverticulum (32 Cases) 361

22.6 Acquired Rectal Atresia or Stenosis (83 Cases) 361

23.3 The Importance of the Colostomy Type from

the Urologic Point of View 373

23.4 Most Common Urologic Abnormalities in Male

Patients with Anorectal Malformations 373

23.4.1 Absent Kidney 373

23.4.2 Urethral Problems 376

23.5 Bifi d Scrotum 377

23.6 Hypospadias 380

23.7 Ectopic Ureters in Males 382

23.8 Ectopic Ureters in Females 382

23.9 Ectopic Vas Deferens 384

24.9 Early Management 40724.10 Surgical Treatment 40724.10.1 The Authors’ Approach 40824.10.2 Other Surgical Techniques for the

Treatment of Hirschsprung’s Disease 41724.11 Total Colonic Aganglionosis 42224.12 Ultrashort-Segment Hirschsprung’s Disease 42524.13 Problems, Complication, and Sequela Secondary

to Operations for Hirschsprung’s Disease 42524.13.1 Preventable Complications (Catastrophes) 42524.13.2 Non-preventable Complications 42924.13.3 Partially Preventable Complications 430References 430

25 Idiopathic Constipation and Other Motility Disorders 435

25.1 Defi nition and Terminology 435 25.2 Incidence, Social Impact, and Relevance 435 25.3 Etiology 43525.3.1 Ultrashort Segment Hirschsprung’s Disease 436 25.3.2 Rectal Manometry 43725.3.3 Doubts and Questions About the Anatomy

of the Internal Sphincter 437 25.3.4 Questions About Myectomy Technique 43825.3.5 Botulinum Toxin Injection 438 25.4 Pathogenesis 440 25.5 Natural History and Clinical Manifestations 442 25.6 Diagnosis 44325.6.1 Colonic Transit Time 44525.6.2 The Evaluation of Severity: Search

for Objective “Instruments” 446 25.7 Management 44725.7.1 Fecal Disimpaction Protocol 44825.7.2 Determination of Laxative Requirements 449 25.7.3 Electric Stimulation 450 25.8 Surgical Treatment 45025.8.1 Operations to Administer Antegrade Enemas

(ACE Procedures) 450 25.8.2 Colonic Resection 451References 453

26 Posterior Sagittal Approach for the Treatment

of Other Conditions 457

26.1 The Kraske Operation 457 26.2 Urogenital Sinus with Normal Rectum 458 26.3 Urogenital Sinus with Normal Rectum

and Adrenal Hyperplasia 467 26.4 Acquired Urethral Atresia 467 26.5 Acquired Rectourethral Fistula 471 26.6 Giant Seminal Vesicle 472 26.7 Urethral Tumors 473

Contents

26.12 Posterior Sagittal Approach, Its Application in Cases

with Hirschsprung’s Disease 481

26.13 Vaginal Atresia with Normal Rectum 482

27.2 Part II: Perianal Fistula and Rectovestibular Fistula

with Normal Anus in Females 490

A Peña, A Bischoff, Surgical Treatment of Colorectal Problems in Children,

DOI 10.1007/978-3-319-14989-9_1, © Springer International Publishing Switzerland 2015

1.1 Introduction

So the conservative who resists change is as

valu-able as the radical who proposes it It is good that

new ideas should be heard, for the sake of the few

that can be used; but it is also good that new ideas

should be compelled to go through the mill of

objection, opposition, and contumely; this is the

trial heat which innovations must survive before

being allowed to enter the human race It is good

that the old should resist the young, and that the

young should prod the old; out of this tension, as

out of strife of the sexes and the classes, comes a

creative tensile strength, a stimulated

develop-ment, a secret and basic unity and movement of the

whole By Will and Ariel Durant [ 1 ]

The history of the surgical treatment of

ano-rectal malformations is a representative sample

of the history and evolution of medicine

Centuries ago, medicine was related to religion

and mysticism; the treatment of the different

dis-eases and surgical conditions was performed by

witches, barbers, or those who showed some

“wisdom” in the community It took many

centu-ries for medicine and surgery to become

scien-tifi c disciplines Even in current days, the practice

of medicine and surgery has a great element

of art

Because of its nature, an anorectal

malforma-tion is a particular defect that has been well

known for many centuries The explanation is

very obvious; one does not have to be a doctor to

make the diagnosis of an absent anal opening

That is perhaps one of the explanations for the

existence of illustrations in history books, going back hundreds of years in different cultures and civilizations, related to the treatment of surgical conditions of the anus

One’s goal in the study of history should not

be to try to memorize names and dates, but rather

to take advantage of the unique opportunity to look back and have a wide perspective of the evo-lution of our knowledge Contemplation of the historical facts, hopefully without prejudices, allows us to recognize patterns of human behav-ior Some of those patterns are creative and posi-tive and should be imitated, and some others are

to be abandoned It allows us to see repetitive behaviors that disclose our limitations as human beings as well as the creativity when dealing with unknown facts One can learn, for instance, that some of the “new discoveries” are not really new Other times, an old concept is brought back, but with a different vision, and even when it is not essentially new, represents an advantage when compared to previous procedures The dilemma

of those who study history is always the ment to “the truth.” We are limited by the litera-ture that is available that may or may not be absolutely truthful

Finally, we, the authors of this book, must confess that we are biased when describing the history of the surgical treatment of anorectal malformations We are biased and impressed by the fact that the real, intrinsic anatomy of the anorectal malformations was really not known until 1980 Looking into the many historical

1 History of the Treatment

of Anorectal Malformations

publications that we reviewed, one can fi nd

dia-grams that only show the imagination of the

authors and the medical illustrators, but not the

real anatomy Those diagrams were followed by

interpretations and erroneous conclusions about

what should and should not be done in the

treat-ment of these malformations There are very few

photographs showing the real anatomy, for

instance, of the connection between the

gastroin-testinal tract and the urogenital tract Some of

the few real pictures of the intrinsic anatomy of

these defects prior to 1980 belong to the

publica-tions of Dr Douglas Stephens [ 2 ] Yet, they are

not representative of the whole spectrum of

ano-rectal malformations

The retrospective analysis of the history of

anorectal malformations shows a very common

human tendency to classify biological

phenom-ena into types, groups, and categories It is

under-standable that this is usually done for the specifi c

purpose of communicating among ourselves and

comparing our results Yet, Mother Nature

con-tinues producing biological phenomena

follow-ing a pattern of a spectrum without payfollow-ing much

attention to our classifi cations Anorectal

malfor-mations are not an exception In other words,

anorectal malformations do not occur in artifi

-cially created groups, traditionally described as

“high,” “intermediate,” and “low.” They occur as

most biological phenomena, following a

spec-trum type of pattern Over time and with careful

analyses of presentation and results, it has

become more and more clear that there are no

“nevers” and no “always” when describing the

variety of anorectal malformations

1.2 The Early Times

The fi rst reference of an anorectal malformation

was found in Babylon, about 650 years B.C It

was written in stone, “When a woman gives birth

to a baby with a closed anus the entire Earth will

suffer from disease” [ 3 ]

Geracao and Aristotle wrote a book on the

Generation of Animals; there, they described a

cow that was born without an anus and defecated

through the urethra [ 4 ]

Soranus de Ephesus was considered the father

of obstetrics in ancient Rome He wrote the book

On the Care Of the Newborn In that book, one

can read that he instructed the women in charge

of delivering babies how to trim off their fi nail of the little fi nger, to dilate the anus of those babies who did not pass meconium after birth [ 5 ] Paul of Aegina (625–690) made the fi rst descrip-tion of an operation for imperforate anus: “If pos-sible, the membrane that covers the anus must be divided with the fi nger If this is not successful, then an incision must be done.” To avoid or to pre-vent the scarring or stricture of the new anus, he recommended a form of bougienage consisting of the local application of wine and balsam [ 6 ] Perhaps the fi rst illustration describing an anorectal procedure in pediatrics was found in a book entitled Cerrahiyei Ilhaniye , written in

nger-1465 by Dr Sharaphedin in Turkey [ 7 ]

In 1606, Guilhelmus Fabricius Hildanus described a case of a recto-bladder fi stula For that case, many doctors were consulted; they all saw meconium coming out of the urethra, and nobody wanted to do anything The baby died on the 17th day of life [ 8 ]

Littre, in 1710, proposed (but did not perform) the opening of a colostomy in cases of anorectal malformation [ 9 ]

Frederik Ruysch (1683–1731) was ized in a famous painting showing the autopsy of

immortal-a bimmortal-aby He described the spontimmortal-aneous rupture of

an anal membrane after 5 days of life The baby died soon thereafter [ 10 ]

The practice of a perineal incision followed by dilatations, in babies born with “imperforate anus,” was a method of choice until the later part

of the nineteenth century During that time, there were many anecdotal descriptions of babies with anorectal malformations that were treated that way, but the overwhelming majority of them died [ 11 ] Some surgeons disagreed with the way of treating those patients, such as Dr Bigelow, Professor of Surgery at the Massachusetts General Hospital in Boston 1857 [ 12 ] He men-tioned, “Based on the analysis of the results of those procedures, I believe that considering the state of the art in surgery for those anorectal defects, it is better to let those babies die.”

1 History of the Treatment of Anorectal Malformations

In 1753, M Louis from Paris described the

case of a little girl who had an orifi ce that was

considered a cloacal malformation [ 13 ] She

was menstruating through the anus! That patient

got married and told her secret to her husband

He convinced her to have sex with him, and she

became pregnant The lady had a “normal”

delivery and was described as producing a

“minor laceration” of the anal sphincter The

presentation of that case was considered in the

Parisian courts, and it was decided by

theolo-gists and modernists that Dr M Louis somehow

had acted in an illegal manner The father of the

baby was called, M Louis was fi nally declared

innocent, and the court allowed M Louis to

publish the case

In 1771, Bertin [ 14 ] described a case of a baby

that was passing feces through the urethra He

was convinced that the baby would die unless he

had an operation He approached the patient

through the perineum and could not fi nd the

rec-tum The baby died and Bertin concluded that the

operation of choice for that particular case should

have been a cystostomy

In 1787, Benjamin Bell (1749–1806) from

Edinburgh [ 15 ] described two successful

opera-tions in which the rectum was found to be located

“high” in the pelvis The procedure that he

described consisted in the introduction of a sharp

instrument in a blind fashion at the location

where the anus was supposed to be located This

procedure was followed frequently by

complica-tions that included bladder perforation and

open-ing of the cul-de-sac of Douglas, and in some

cases, the rectum was never found In his book

entitled A System of Surgery , Bell described

dif-ferent types of anorectal malformations including

“anal agenesis,” “anorectal agenesis,” “vesical

fi stula,” and “vaginal fi stula.” Benjamin Bell was

probably the fi rst one to emphasize the need and

importance of decreasing the pain during these

procedures that were generally done using

homeopathic techniques

It was Antoine Dubois, in 1783, who

appar-ently performed the fi rst inguinal colostomy on

the left side in a 1-day-old baby with imperforate

anus The patient died 10 days later [ 16 ] In 1793,

Duret, following the suggestion of Littre in 1710,

was probably the fi rst one to perform an inguinal colostomy in the sigmoid colon in a baby boy with imperforate anus; a week later, the patient was still alive [ 17 ]

In 1832, almost 100 years later, Martin decided to follow the suggestion of Bertin and to perform a cystostomy in a patient who was pass-ing stool through the urethra Unfortunately, the patient died [ 18 ]

Roux de Brignoles, in 1834, suggested that the

fi bers of the sphincter mechanism should be meticulously preserved during the perineal dis-section [ 19 ]

Amussat, a prominent young surgeon, also in

1835, in Paris, described the case of a 2-day-old girl who was not passing meconium He operated

on the patient on the dining room table of the patient’s house, assisted by his collaborators He found the blind rectum, and he is considered the

fi rst surgeon who decided to suture the wall of the rectum to the skin edges, which could be consid-ered the fi rst anoplasty After 28 days, the baby was doing very well, without complications [ 20 ]

It was also Amussat who classifi ed the anorectal malformations into fi ve types: type 1, anal steno-sis; type 2, anal membrane; type three, a blind rectum at a variable distance from the anal skin; type 4, a blind but also very “defi cient” rectum; and type 5, the rectum communicated with other organs, such as the bladder, urethra, or vagina

He recommended dilatation for type 1, incision and excision of the membrane followed by dilata-tions in type 2, and suture of the rectum to the skin in type 3 In types 4 and 5, he recommended mobilization of the posterior part of the rectum and pulling it down to the perineum In cases in which it was diffi cult to fi nd the rectum through the perineal incision, he recommended making the incision larger and to totally or partially remove the coccyx

In 1844, Stromeyer [ 21 ] suggested that in cases in which the rectum could not be found through the perineal dissection, the peritoneal cavity should be opened through the perineum, and the surgeon should look for the blind rec-tum with a fi nger That idea was practiced in

1872 by Leiserink, and he described a “good result” [ 22 ]

1.2 The Early Times

In 1860, Bodenhamer [ 23 ] proposed a classifi

ca-tion dividing these malformaca-tions into four types:

Type 1: Incomplete rupture of the “inner

mem-brane” or anal stenosis

Type 2: Imperforate anus due to a persistence of

the “anal membrane”

Type 3: Imperforate anus with blind rectum

sepa-rated from the “anal membrane”

Type 4: The presence of a blind rectum separated

from the anal canal

In 1866, Chassaignac [ 24 ] decided to follow

the idea suggested by Martin de Lyon of opening

a colostomy in order to introduce some sort of

guide through the intestinal lumen of the

colos-tomy, to facilitate fi nding of the blind rectal end

The perineum was then opened where the surgeon

could feel the bulging of the guide Chassaignac

operated on a 7-month-old baby who had a

previ-ous colostomy and was able to create an opening

in a “satisfactory” manner using that technique

Delens, in 1874 [ 25], described a case in

which he achieved good exposure in the perineum

area by removing or mobilizing back the coccyx

without resecting it The next year, Polaillon

described splitting of the coccyx in the midline,

obtaining better exposure to be able to dissect the

rectum in a deeper area [ 26 ]

In 1880, Neil McLeod was the fi rst to suggest a

combined abdominoperineal approach He chose

to start the operation through the perineum, and if

the rectum was not found, to open the abdomen

through a midline incision With a fi nger, as a

guide from inside the abdomen, the perineal

inci-sion should be created to reach the peritoneal

cav-ity and the rectum pulled through [ 27 ]

In 1887 Vincent of Lyons performed a

parasa-cral incision instead of a mid-saparasa-cral one This

was described by Maitre [ 28 ]

In 1894, Paul Delageniere suggested

perform-ing a lateral laparotomy to fi nd the rectum and to

reach the perineum through the abdominal cavity,

using his fi nger as a guide and then pulling

through the rectum [ 29 ]

In 1897, Rudolph Matas [ 30 ], a brilliant

sur-geon in New Orleans, mentioned that cutting,

dividing, or destroying the sacrum had a negative

effect because it damaged the muscle insertions

as well as the innervation and blood supply of the

pelvic structures He suggested entering the vis through the third sacral foramen He sup-ported the idea of opening a colostomy He also believed that the rectal ampulla could move down spontaneously; therefore, he proposed to open a colostomy and wait In 1897, Matas wrote 22 conclusions related to the management of ano-rectal malformations Some of which are still valid:

1 “The most common types of anorectal formations can be repaired through a peri-neal approach.” Interestingly, this conclusion

mal-is quite accurate

2 “There are no external signs to determine the internal anatomic malformations.” This con-clusion is partially valid since now we know that we can learn a lot just by careful inspec-tion of the perineum

3 “One should not depend on the introduction of guides through the vagina or the urinary tract to determine the presence or absence of intestine The use of a needle to aspirate meconium is also dangerous because of the risk of peritoneal contamination.” This is still true

4 “The operation should be done as early as possible to avoid death consecutive to the passing of stool to the blood, peritonitis, intestinal obstruction, absorption of toxins, and migration of bacteria from the intes-tines.” Although now we are aware of many new, sophisticated pathophysiologic mecha-nisms, this concept is still valid

5 “The tolerance of the baby to the trauma is inversely proportional to the age in days after birth And in addition, the baby without sep-sis is as tolerant to trauma as the adult.” Again, he was right

6 “The ideal result in this kind of operation is the restoration of the passage of stool, creat-ing an anus in a normal position with bowel control.” This, of course, is still valid

7 “The only way to obtain this kind of result is performing a proctoplasty as proposed by Amussat.” Obviously, this is mostly wrong

8 “In order to obtain the best possible results from the functional point of view, the opera-tor must avoid the unnecessary injury of the sphincter mechanism, for that, the incision

1 History of the Treatment of Anorectal Malformations

must be performed strictly in the midline.”

He was right!

9 “The old method of stab of the perineum

without a proctoplasty was not justifi ed.” He

was right

10 “The initial peritoneal exploration of the

pel-vis through a perineal-sacral aperture was

one of the greatest advances in the treatment

of these conditions.” Of course, that is no

longer true

11 “The peritoneal exploration through the

perineum must be attempted systematically

when the rectum is not found through the

perineum.” This is no longer valid

12 “Those techniques that use a sacral resection

or excision or osteoplasty to increase the

exposure and to reach the peritoneum

look-ing for the rectum are valid.” Obviously, we

do not use that anymore

13 “The best approach is a midline incision

through the coccyx and sacrum.” This is

mostly true

14 “A predisposition to suffer prolapse must be

expected in cases of resection of the sacrum.”

Obviously, we do not touch the sacrum

anymore

15 “A primary exploratory laparotomy is not

indicated as a rule.” This is true

16 “The great majority of imperforate anus can

be treated successfully through the

perineum.” That is true

17 “The perineal anus can be created pulling the

colon and connecting it to the perineum But,

in cases of emergency, one can connect the

small bowel to the perineum.” Obviously, we

do not do that

18 “The mortality from a colostomy is greater

than the anoplasty and perineal-sacral

approach.” This is obviously wrong by

mod-ern standards

19 “Primary colostomy in the groin, as a

pri-mary procedure, is only indicated when the

baby is extremely sick Under all of the other

circumstances, the perineal incision must be

the fi rst one.” This statement is partially true

20 “An exploratory laparotomy is only

per-formed after the rectum was not found

through the perineum.” This statement is

also partially true

21 “If, for some reason, the surgeon decided to open a colostomy fi rst, he should always make every effort in a second procedure to open the anus in the perineum.” This is mostly true

22 “The perineal-sacral anus, when it is rectly done, is almost certain to have bowel control as time goes by.” That is, of course, mostly not true

In 1899 and published in 1908, Mastin onstrated that a permanent colostomy was com-patible with growth and development [ 31 ] He operated on a newborn baby and created a colostomy, and when he offered the family the opening of an anus, the family refused to have that operation done because the patient was doing very well and has adapted to the presence

dem-of the stoma, playing sports and growing and developing normally In 1903, Mastin was called to take care of another case He per-formed a perineal midline incision He was able

to fi nd the bowel and perform an anoplasty that

he sutured to the skin with catgut He described that 4 years later, the patient had bowel control

In 1915, Brenner [ 32 ] published an lent paper in Surgical Gynecology and Obstetrics and described his experience with

excel-61 cases He described different degrees of development of the external sphincter He sug-gested that the operations to repair imperforate anus should “last no more than 5–8 min!!” He performed a posterior incision, and he sug-gested opening a colostomy if the perineal approach was unsuccessful He described that

in males, the rectum opens more often into the bladder rather than the urethra, which is not true, since now we have well- documented evi-dence that the connection between the rectum and the bladder only occurs in 10 % of the male cases He suggested that if the patient did not have external sphincter fi bers, bowel con-trol must be obtained by an axial rotation of the gut or using some muscle fi bers from the gluteal region Even though Brenner’s conclusions are not valid at the present time, his work is very signifi cant, because of the number of cases and the meticulous descrip-tion of them

1.2 The Early Times

These ideas, like in many other historic events,

illustrate how naive we tend to be Therefore, we

like to say that every time we try to cheat on

Mother Nature, she teaches us a lesson

In 1930, Owen Wangensteen and Carl Rice

published a paper describing a method of

radio-logically determining the height of the blind rectal

end to select the best surgical approach for patients

with anorectal malformations [ 33 ] The technique

that they described is well known as an

“inverto-gram.” It consisted of putting the newborn baby

upside down for several minutes and taking an

x-ray fi lm of the pelvis to determine the location of

the blind end of the rectum, as well as the distance

from the blind end of the rectum to the anal skin

The blind end of the rectum can be seen because it

is full of gas That method still has some value

However, we use a variation of it in less than 5 %

of all cases, in those in whom there is no clinical

evidence of the location of the distal rectum Yet,

we have learned through the years that the same

image that Wangensteen and Rice were able to

obtain with the invertogram can be achieved by

placing the patient in prone position with the

pel-vis elevated and taking a cross- table, lateral fi lm

We have learned many lessons from the

exter-nal examination of the perineum of the babies, as

well as other more sophisticated imaging

methodology

In 1934, William Ladd and Robert E Gross

[ 34 ] published a very comprehensive series of

cases Their publication also included good

embryologic description They also included a detailed table of associated malformations This

is extremely important since, as the reader will be able to see in this textbook, the frequency of the associated defects in cases of anorectal malfor-mations is very signifi cant and those associated defects have a vital role in the prognosis of these patients Ladd and Gross’s publication is a beau-tiful one; it has very elegant drawings done per-sonally by Dr Robert Gross, illustrating the development of female malformations The mor-tality in their series was 26 %

In 1936, Stone [ 35 ] published a paper entitled

“Imperforate Anus with a Rectovaginal Cloaca.”

In 1938, J K Berman [ 36 ] published a paper

on 23 cases of anorectal malformations with

47 % mortality He opposed the use of mies in his patients because of its high mortality and proposed an incision running from the peri-neal body to the coccyx in newborns, with local anesthesia He used 0 size chromic catgut He described only the pull-through of the bowel, leaving the fi stula to the urinary tract untouched until the patient was older!!

In 1948, Rhoads et al [ 37 ] (Fig 1.1 ) lished their experience with the fi rst survivor of a primary abdominoperineal pull-through, without

pub-a colostomy After thpub-at publicpub-ation, mpub-any geons tried to perform that kind of operation, sometimes with success, but many other times with serious catastrophic results, and therefore, years after that, this approach was reconsidered

Lately, many others have been trying to approach

newborn babies primarily without a colostomy

As will be seen in this textbook, that approach is

sometimes justifi ed, but not always

In 1953, Douglas Stephens published his fi rst

landmark paper on the subject, in Australia [ 38 ]

Dr Stephens has the unique distinction of being

the fi rst person who studied the anatomy of the

pelvis in patients who died from an anorectal

mal-formation From his studies, he concluded that the

key part of the sphincter mechanism to achieve

bowel control in these cases was the “puborectalis

sling.” It took time for his concept to be learned

and accepted by the world community of pediatric

surgeons, but within a few years, most pediatric

surgeons recognized that was something to be

considered seriously, and therefore, the “era of the

puborectalis” began From that time, most

sur-geons tried to design operations aimed to

preserv-ing the “puborectalis slpreserv-ing,” which was considered

key for bowel control Unfortunately, it is not easy

to obtain cadavers of children born with anorectal

malformations because most children with

ano-rectal malformations survive, and therefore the

number of specimens studied by Dr Stephens was

very limited In retrospect, we believe that his

conclusions are not valid because his studies were

performed in a limited number of the most severe

cases, not representative of what we call the

spec-trum of anorectal malformations The cases

(cadavers) that he studied we think are not

repre-sentative of the most common types of

malforma-tions that we see Yet, one of his recommendamalforma-tions

is still valid: he recommended pulling the bowel

down, as close as possible to the urethra In

addi-tion, Dr Douglas Stephens published a book [ 2 ]

that represents the document with the largest

amount of information related to the subject of

anorectal malformations at that time

In 1955, Sir Denis Browne, a prominent

sur-geon from Great Ormond Street Hospital in

London, proposed that patients with rectovaginal

fi stulas had a normal sphincter located at the

vagina site [ 39 ] We now know that that never

happens He also suggested that female patients

with vaginal, as well as males with rectoprostatic,

fi stulas have no sphincter mechanism, which, as

we know now, is mostly inaccurate

In 1954, Dr William Potts [ 40 ] published a paper related to the treatment of 22 “rectovaginal

fi stulas,” 8 rectourethral fi stulas, 9 recto-perineal

fi stulas, and 12 rectovesical fi stulas The plasty that he proposed for the most common type of malformation seen in females that we now know by the name of rectovestibular fi stula

ano-is still known as the “Potts’ anoplasty” and sists of dissecting the rectum from the vestibule and passing it behind a bridge of skin, to be placed within the limits of the sphincter In retro-spect, now we believe that what he described as

con-“rectovaginal fi stulas” were actually cases of tovestibular fi stulas, since now we recognize that real rectovaginal fi stulas are extremely unusual malformations Unless we postulate the theory that the type of pathology changes through the years, it is diffi cult to believe that surgeons had many cases of rectovaginal fi stulas, which we

rec-fi nd now to be a malformation that is almost nonexistent

In 1960, Scott, Swenson, and Fisher published one of the fi rst papers on long-term follow-up results Their patients suffered from a mortality

of 12.7 %; 4.8 % was operative deaths In their study, they reported 68 % incontinence in patients with the so-called “high” malformations and

89 % good results in what were described as

“low” malformations [ 41 ]

In 1963, Kiesewetter et al [ 42 ] reported their experience with 146 patients followed over a period of 16 years These surgeons had a demonstrated special interest in the manage-ment of anorectal malformations Their mortal-ity was 19.2 %; 86 cases were followed on a long-term basis The authors divided their series into “high malformations,” where the rectum was located two centimeters above the anal skin, and “low malformations,” for those where the rectum was closer to the skin They obtained 72 % “good results” in “low malfor-mations” and 45 % “bad results” in “high mal-formations.” They mention that it was important

to preserve, as much as possible, the distal part

of the bowel, which is a concept that we support

at the present time They reported 24.4 % of the cases having vestibular fi stulas, which is simi-lar to what we report

1.2 The Early Times

In 1966, Dr Kiesewetter [ 43 ] supported the

idea proposed by Stephens that the puborectalis

muscle was the only available muscle useful to

achieve bowel control He adopted Stephens’

idea of a sacral incision to preserve the

puborec-talis sling Through that incision, a blind tunnel

was created, behind the urethra (in males) A

Penrose drain was then passed through the

tun-nel The rectum was then to be pulled down

through that tunnel He also adopted the principle

of a transabdominal endorectal dissection in

order to try to avoid damage to the innervation of

the pelvic organs This is the same principle that

Soave and Boley proposed for the treatment of

Hirschsprung’s disease Kiesewetter’s operation

was called a sacro-abdominoperineal endorectal

pull-through

In 1967, Dr Rehbein [ 44 ] also proposed an

abdominal sacroperineal procedure He presented

70 cases, 55 males and 15 females He

empha-sized the importance of preserving the

“puborec-talis muscle.”

We want to express our recognition to many

prominent surgeons from all over the world who

demonstrated special interest and dedication to

the fi eld of anorectal malformations We are

unable to mention all of them; therefore, we

selected the most prominent ones including:

• Santull [ 45 ], Hanley [ 46 ], Lynn [ 47 ], Partridge

[ 48], Trusler [ 49], Cozzi [ 50], Soave [ 51 ],

Louw [ 52], Knutrud [ 53], Nixon and Puri

[ 54 ], Smith [ 55 ], Holschneider [ 56 ], Varma

[ 57 ], Chatterjee [ 58 ], Rintala [ 59 ], Endo [ 60 ],

Scharli [ 61 ], Ito [ 62 ], Brayton [ 63 ], Aluwihare

[ 64 ], and Banu [ 65 ]

In 1970, in Melbourne, an international

com-mittee was created to design an “international

classifi cation of anorectal malformations.” That

“international classifi cation” was adopted by all

of the pediatric surgeons in the world, but

because of its complexity, it was not used in the

everyday practice by most pediatric surgeons

(Fig 1.2 ) [ 66 ]

In 1971 [ 67], the American Academy of

Pediatrics proposed a joint effort between

differ-ent institutions to learn about the differdiffer-ent

thera-peutic modalities as well as the results of the

treatment of anorectal malformations and sent

questionnaires to the members of the surgical section of the academy From the answers, they were able to put together 1,116 patients from 51 institutions, 58 % were males and 42 % females The incidence of fi stula in males was 72 % and in females was 19 %, and 28.7 % of these fi stulas were “rectovaginal.” This is something that we know was most likely a misnomer or lack of accurate examination of the genitalia because, as

we mentioned, congenital rectovaginal fi stulas in our experience are almost nonexistent; 81 % of the patients with a “high malformation” received

a colostomy, and 62 % of those colostomies were done in the transverse colon The mortality reported was 19 %

During the years of 1969–1970, the senior author had the privilege of meeting Dr Justin Kelly Dr Kelly, a fully trained pediatric surgeon from Melbourne, Australia, had been greatly infl uenced by Dr Douglas Stephens Dr Kelly went to Boston Children’s Hospital as a clinical fellow for two extra years There, he lectured and communicated the ideas of Dr Stephens to all members of the surgical staff Dr Peña (senior author) was at Boston Children’s Hospital as a research fellow in 1969 and as a resident in 1970 and 1971 During that time, he learned from Dr Kelly’s, Dr Stephen’s, and Dr Smith’s ideas about the management of anorectal malforma-tions Upon his return to Mexico City, Dr Peña became the Chief of Surgery at the New National Institute of Pediatrics

From 1972 to 1980, the senior author of this book operated, in Mexico City, on 56 cases of the so-called, at that time, “high” imperforate anus He followed the principles proposed by Stephens and learned from Dr Kelly (sacral approach) and laparotomy when necessary as proposed by Kiesewetter, and Rehbein (sacro- abdominoperineal pull-through) During those

8 years, he became aware of the fact that, in the earlier cases, he was opening the abdomen very often (80 % of the time) to repair these malfor-mations, but after 8 years of experience, he was opening the abdomen only 20 % of the time He attributed this to the fact that the sacral incision that Stephens originally proposed (very small) was being gradually enlarged throughout those

1 History of the Treatment of Anorectal Malformations

8 years, becoming longer and longer In addition,

he decided to use an electrical stimulator with the

specifi c purpose to identify the “puborectalis

sling.” Eventually, he felt the need to divide part

of the sphincter mechanism that was visible

dur-ing this operation He thought that this muscle

mechanism must have been the “puborectalis

sling.” He specifi cally proposed to divide part of

what he thought was the “puborectalis muscle” to

have better exposure and to facilitate the

separa-tion of the rectum from the urinary tract He

decided to present that experience of 56 cases at

the annual meeting of the Pacifi c Association of

Pediatric Surgeons in March 1980 in Colorado

Springs, Colorado, United States Basically, in

this presentation, he proposed to make a longer

midsagittal incision to have better exposure to

facilitate the separation of the rectum from the

urinary tract in male patients and to avoid the need of a laparotomy He also proposed the use of

an electrical stimulator to identify the sphincter mechanism and also to divide in the midline the muscle that he found, in order to facilitate, again, the dissection of the fi stula and proposed to resu-ture the muscle behind the rectum at the end of the procedure This was basically a step prior to the full posterior sagittal anorectoplasty proposed later The paper provoked a signifi cant discus-sion, particularly between the Australian mem-bers of the audience They specifi cally mentioned that the muscle that was shown in the movie in that presentation was not the “puborectalis sling” because that structure was only seen in Australia,

by Dr Stephens, in autopsies In other words, for years, the world’s pediatric surgical community had been talking about how to preserve the

Low deformities (translevator)

1 At normal anal site

i Covered anus - complete

ii Anal stenosis

Covered anal stenosis

2 At perineal site

i Anterior Perineal Anus

ii Ano-cutaneous fistula

(Covered anus – incomplete)

Miscellaneous deformities

i Imperforate anal membrane

ii Anal membrane stenosis

iii Vesico-Intestinal Fissure

iv Duplications of the anus, rectum and genitourinary tracts

v Combination of deformities

Melbourne classification, 1970 Females

Low deformities (translevator)

1 At normal anal site

i Covered anus - complete

ii Anal stenosis Covered anal stenosis

2 At perineal site

i Anterior Perineal Anus

ii Ano-cutaneous fistula (Covered anus – incomplete)

3 At vulvar site

i Vulvar anus

ii Ano-vulvar fistula iii Ano-vestibular fistula Miscellaneous deformities

i Imperforate anal membrane

ii Anal membrane stenosis iii Vesico-Intestinal Fissure iii Perineal groove

iv Perineal canal

v Vesico-intestinal fissure

vi Duplications of the anus, rectum and genitourinary tracts vii Combination of deformities

Fig 1.2 International classifi cation of anorectal malformation Melbourne, Australia 1971

1.2 The Early Times

“puborectalis sling” structure during an

opera-tion Yet, nobody has seen such structure, except

for Dr Stephens in his autopsy specimens!!

After that meeting, Dr Peña went back to

Mexico and decided to use a much longer,

poste-rior, midsagittal incision, running from the

mid-dle portion of the sacrum to the base of the

scrotum, trying to stay exactly in the midline and

using an electrical stimulator The purpose of that

incision was to clarify the controversy about the

characteristics and location of the “puborectalis

sling.” During the meeting at Colorado Springs,

he had conversations with different members of

the Pacifi c Association of Pediatric Surgeons,

including Dr Peter deVries Most surgeons were

very negative about the idea of “cutting the

sphincters.” Dr deVries, on the other hand, was

enthusiastic and showed a supportive attitude

about the idea On August 10, 1980, in Mexico

City at the National Institute of Pediatrics, Dr

Peña and Dr Miguel Vargas performed the fi rst

posterior sagittal anorectoplasty in a female

patient They were surprised by the fact that they

were unable to identify anything that looked like

a “puborectalis sling.” Dr Pieter deVries visited

Dr Peña and joined him to explore four more

patients in September of 1980 The results of

these initial operations were presented at a

round-table at the World Symposium of Pediatric

Surgery in Acapulco in September of 1980 Dr

Stephens was part of that roundtable

Subsequently, Dr deVries invited Dr Peña to go

to Sacramento, California, to operate on four

more patients The experience was presented at

the Pacifi c Association of Pediatric Surgeons in

Hawaii together by Dr Peña and Dr deVries

Subsequently, Dr Peña and Dr deVries

pre-sented their experience at the meeting of the

American Academy of Pediatrics in the fall of

1981 in New Orleans After that, unexpectedly,

Dr Pieter deVries published the fi rst paper on

posterior sagittal anorectoplasty [ 68 ] Following

that, Dr Peña submitted another paper 2 months

later on the same subject [ 69 ]

In 1984, Dr Stephens promoted a meeting to

discuss the possibility of creating a new, more

practical classifi cation and to rediscuss the whole

subject of anorectal malformation in Wingspread,

near Wisconsin He invited pediatric surgeons well known for their interest and experience in the surgical treatment of anorectal malformations from all over the world These surgeons included

Dr Peter deVries, Dr Subir Chatterjee (India),

Dr Durham Smith (Australia), Dr Nicolas Martin del Campo (Mexico City), Dr Alberto Peña, Dr Stephen Dolgin, Dr Sid Cywes (South Africa), Dr Morihiro Saeki (Japan), Dr Jotaro Yokorama (Japan), Dr Donnellan (Chicago), Dr Dale Johnson (Salt Lake City), and Dr Alex Holschneider from Germany (Fig 1.3) As a result of that meeting, a new classifi cation was created, called the “Wingspread classifi cation.” Fortunately, that new classifi cation was more simplifi ed and therefore more useful and yet, from our point of view, was still very defi cient (Fig 1.4 Wingspread classifi cation) [ 70 ]

In May of 2005, Dr Holschneider promoted another meeting in Krickenbeck, Germany (Fig 1.5 ), to rediscuss the subject and a new clas-sifi cation, known as Krickenbeck classifi cation, was created (Fig 1.6 ) This Krickenbeck classifi -cation we feel is much better than the two previ-ous ones [ 71 ]

From August 10, 1980, until the day of sending this manuscript to be printed, the authors have operated on over 2,032 cases, documenting their

fi ndings and recording them into a database Efforts have been made to try to follow all these cases on a long-term basis The senior author of this book traveled to many countries, invited by generous pediatric surgeons who helped contrib-ute to accumulate the largest series of cases of this condition, operated mostly by a single person, fol-lowing as consistently as possible similar princi-ples and techniques Here is a list of the surgeons with whom the authors are deeply in debt for their generosity in inviting the senior author and shar-ing their patients to be operated upon

What started as a controversy related to the anatomy of the sphincter mechanism in patients with anorectal malformations represented the main catalyst and motivation to study, learn, and collect

an enormous amount of information related with anorectal malformations and associated defects

In 1980, we were not aware of the fact that we were “opening a Pandora’s box.” The controversy

1 History of the Treatment of Anorectal Malformations

over the anatomy of the sphincter mechanism,

seen in retrospect, appears today of little signifi

-cance, compared to the fascinating observations

that emerged from the surgical exploration,

eval-uation, and long-term follow-up of so many

patients

The road has been long, exciting, wonderful,

and illuminating We have been learning many

important lessons, and now we are certain that

the more we learn about the subject, the more

intriguing questions we must answer The dictum

that “It’s not the unanswered questions, but rather

the unquestioned answers” crystallizes well the

evaluation of this problem in surgery This has

been a lifetime, very enjoyable, fascinating,

hum-bling, and extraordinary experience

Opening the pelvis posterior-sagittally

allowed us, for the fi rst time, to be directly

exposed to the peculiar, complex, intrinsic

anat-omy of these defects Much beyond the anatomic

characteristics of the sphincter mechanism, we

learned about the detailed anatomy of the

junc-tion between the rectum and the urogenital tract

With that knowledge came the awareness of the

potential damage that we could provoke while

trying to separate on these structures blindly

Suddenly, we had an explanation for the many

older patients who had come to our clinic who were born with an anorectal malformation and were subjected to blind or semi-blind operations that resulted in a urethral stricture and/or acquired urethral atresia, neurogenic bladder, impotence, retrograde ejaculation, painful ejaculation, and many other problems

Now we know that the separation of the tum from the urogenital tract under direct vision

rec-is a technically demanding maneuver; it rec-is fore easy to understand how much damage we could provoke doing the operation blindly, and sadly that is what we were doing prior to 1980 Being directly exposed to the intrinsic anat-omy of these malformations only made more obvious our naivety when trying to create oversimplifi ed classifi cations of a spectrum of defects, without really knowing the true anatomy The most conspicuous lesson learned through all these years is that we have been dealing with a spectrum of defects The more cases we operate

there-on, the wider the spectrum becomes Classifi cations that divide a spectrum of defects,

in categories such as “high” and “low” or even

“high,” “intermediate,” and “low,” represent oversimplifi cations that misguide rather than help Now we know that in surgery, it is not

Fig 1.3 Picture of the group of pediatric surgeons who met in Wingspread

1.2 The Early Times

advisable to use radical terms such as “always”

or “never.” Being exposed directly to the anatomy

of these malformations also gave us a unique

opportunity to correlate the anatomic fi ndings

with the fi nal functional results and the potential

devastating functional sequelae

Confronted with an anatomy never described

before, we were obligated, by common sense, to

describe technical maneuvers never described

before Many remarkable experiences were

wait-ing ahead of us

In 1982, Dr Maricela Zarate, an ex-resident of

Dr Peña in Monterrey, N.L., Mexico, had a patient with a cloaca The available literature at that time related to the surgical treatment of clo-aca was very scant (see Chap 16 )

In 1982 in Monterrey, Mexico, Dr Peña, Dr Maricela Zarate, and Dr Marshall Schwartz (vis-iting from the United States) operated via poste-rior sagittal on a girl with a cloaca (Fig 1.7 ) That girl is now an adult, has bowel and urinary con-trol, and has a baby We like to say that “God

protects the innocent,” since that case from

Monterrey, seen in retrospect, after having

oper-ated on over 531 patients with a cloaca, we now

belongs to the “good side of the spectrum” of

clo-acas Later on, we would be confronted with

much more challenging cases To repair those

complex cases would require a great deal of

creativity, imagination, and dedication

A very important positive development occurred

in the fi eld of colorectal problems of children and

that is the introduction of the minimally invasive

technology Willital [ 72 ] published his attempt to repair an anorectal malformation using this thera-peutic modality In the year 2000, Dr Keith Georgeson published his experience with seven cases, in a detailed description of the operation [ 73 ] Following Georgeson, many other surgeons have been using minimally invasive techniques to repair anorectal malformations (see Chap 13 )

In July 1985, the senior author moved to Long Island, New York, United States, and became Chief of Pediatric Surgery at Schneider Children’s Hospital until June 30, 2005 During those

20 years, we were able to accumulate a very large experience and moved forward in our attempt to benefi t more children We were able to:

• Find further applications for the posterior ittal approach [ 74 , 75 ]

sag-• Describe the transanorectal approach for the treatment of urogenital sinus with normal rec-tum and other conditions [ 76 , 77 ]

• Create a protocol of medical and surgical management of patients with idiopathic con-stipation [ 78 ]

Fig 1.5 Picture of the group of pediatric surgeons who attended the Krickenbeck meeting

Standards for diagnosis international classification (Krickenbeck)

Major clinical groups

Perineal (cutaneous) fistula

H fistula Others

Fig 1.6 Krickenbeck classifi cation of anorectal

malformation

1.2 The Early Times

• Describe the maneuver known as total

uro-genital mobilization to facilitate the repair of

cloacas [ 79 ]

• Describe the precise anatomy of a

malforma-tion called posterior cloaca [ 80 ]

• Create and implement a bowel management

program aimed to keep clean patients

suffer-ing from fecal incontinence [ 81 – 84 ]

It was there, in Long Island, New York, where

it became clear to us that children suffering from

anorectal malformation required and deserved

better care Yes, we were very proud because we

were able to repair the anatomy of the

malforma-tion, but we learned that at least 25 % of all our

cases suffered from fecal incontinence because

they were born with severe anatomic defi

cien-cies We also learned that many of the 75 %

group of patients had a “borderline” bowel

con-trol and require supervision and help for life We

understood that 25 % of our patients required

neurosurgical services, 30 % of them had

ortho-pedic problems, most of them need the help of a

gastroenterologist, at least 50 % of them need a

pediatric urologist, and most girls will benefi t

from the advice of a pediatric gynecologist

There was an obvious conclusion that these

patients must be treated in specialized centers where they will be treated by a multidisciplinary team With that in mind, we presented the idea to some leaders of prominent children’s hospitals

in the United States Dr Richard Azizkhan had the vision and courage to adopt the plan, and the Colorectal Center for Children was created at the Cincinnati Children’s Hospital Medical Center

in July 2005

References

1 Durant W, Durant A (1968) The lessons of history Simon and Schuster, New York

2 Stephens D, Smith D (1971) Anorectal malformation

in children Year Book Medical Publishes, Chicago

3 Ballantyne JW (1894) The teratological records of Chaldea J Antenatal Pathol Teratol 1–2:127–142

4 Aristotle (1953) Generation of animals, (trans: Peck AL) Harvard University Press, Cambridge, book IV, chap IV, pp 444–447

5 Soranos of ephesus: Gynecology, based on Ilberg edition, (trans: Temkin O) Johns Hopkins Press, Baltimore (1956), books II, VI, pp 79–80

6 Adams F (1847) The seven books of Paulus Aegineta: translated from the Greek, with a commentary embrac- ing a complete view of the knowledge possessed by

Fig 1.7 Picture taken during the fi rst posterior sagittal approach to repair a cloaca in Monterrey, Mexico

1 History of the Treatment of Anorectal Malformations

the Greeks, Romans, and Arabians on all subjects

connected with medicine and surgery Sydenham

Society- publisher, London 2:405–406

7 Numanoglu I (1973) Cerrahiyei Ilhaniye: the earliest

known book containing pediatric surgical procedures

J Pediatr Surg 8(4):547–548

8 Fabricius G, Hildanus WF (1606) Observationum et

curationum chirurgicarum centuria Centur I obs 73

und 75 Basil 1606 Quoted by Scharli

9 Littré M (1709) Memoire de l’Academic Royal de

Sciences de Paris p 9

10 Ruysch AP (1680) Adversaria Anatomica, Decad II

10, p 43 (um 1680)

11 Ferdinand K Dic Chirurgischen Krankheiten des

kindesalters 1894 verlag von Ferdinand Enke Stuttgart

12 Bigelow HJ (1857) Extracts from the Records of the

Boston Society for Medical Improvement Boston

Med Surg J 57(12):240

13 Louis (1754) Theses Anatomicae chirurgicae, Paris,

zit bei Bushe (57)

14 Bertin EJ (1771) Mémoire sur les enfans qui naissent

sans un véritable anus Mem Acad Roy Sci Paris

15 Bell B (1787) A system of surgery, 3rd edn Bell and

Bradfute, Edinburgh, p 375

16 Allan (1797) Rapport sur les observations et réfl

ex-ions de Dumas, relatives aux imperforatex-ions de

1’anus [Reports on the observations and refl ections

of Dumas, relative to imperforate anus] Recueil

Périodique de la Société de Médecine de Paris, No

XIII, pp 123–131

17 Duret C (1798) Observation sur un enfant né sans

anus, et auquel it a été fait une overture pour y

sup-pléer [Observation of a child born without anus, in

whom an overture was made to compensate it]

Recueil Périodique de la Société de Médecine de

Paris 1798, No XIX, Tome IV, pp 45–50

18 Martin: Annales de Societe Natur Bd 12, 1832 Cited

by Schärli AF (1978) Malformations of the anus and

rectum and their treatment in medical history Prog

Pediatr Surg 11:141–472

19 Roux de Brignoles JN (1834) De l’imperforation de

l’anus chez les nouveaux Rapport et discussion sur

l’operation a tenter dans ces cas Gazette Medicale de

Paris 2:411–412

20 Amussat JZ (1835) Histoire d’ume operation d’anus

practique avec success par un nouvean procede Gaz

Med Paris 3:753–758

21 Stromeyer GFL (1844) Handbuch der Chirurgie

Freiburg, Herder

22 Leisrink H (1872) Atresia Ani, fehlen des rectum:

operation nach der methode van stromeyer Dtsch Z

Chir 1:494

23 Bodenhamer WH (1860) A practical treatise on the

aetiology, pathology and treatment of the congenital

malformations of the congenital malformations of the

rectum and anus Wood, New York

24 Chassaignac M (1856) Presentation de Malades

Bulletin de la Societe de chirurgie de Paris, p 410

25 Delens M (1875) Observations: II Imperforation

Anale enterotomia perineale avec resection du coccyx-

Guerison avec prolapse Bull Med Soc Chir 1:217

26 Polaillon H (1875) Bull Soc Chir, Seancedu 14 Juliet

1875, pp 613–619; Gaz Hebd Med Chir 31:400, cited

by Mastin W (1908) A resume of the surgical ment of ano-rectal imperforation in the newborn Surg Gynecol Obstet 7:317

27 McLead NC (1880) Case of imperforate rectum with

a suggestion for a new method of treatment Br Med J 2:653–658

28 Maitre J (1887) Contribution à l’étude des rations ano-rectales, exposé d’un nouveau procédé opératoire appliqué à leur traitement Doctoral disser- tation, Th èse Médecine, Lyon

29 Delageniere P (1894) Du traitement de L’imperforation

de l’anus Arch Prov De chir 1(3):405

30 Matas RD (1897) The surgical treatment of congenital ano-rectal imperforation considered in the light of mod- ern operative procedures Trans Am Surg Ass 15:87–95

31 Mastin W (1908) A resume of the surgical treatment

of ano-rectal imperforation in the newborn Surg Gynecol Obstet 7:316–323

32 Brenner EC (1915) Congenital defects of the anus and rectum Surg Gynecol Obstet 20:579–588

33 Wangensteen OH, Rice CO (1930) Imperforate anus:

a method of determining the surgical approach Ann Surg 92(1):77–81

34 Ladd WE, Gross RE (1934) Congenital tions of anus and rectum: report of 162 cases Am J Surg 23(1):167–183

35 Stone HB (1936) Imperforate anus with rectovaginal cloaca Ann Surg 104(4):651–661

36 Berman JK (1938) Congenital anomalies of the tum and anus Surg Gynecol Obstet 66:11–22

37 Rhoads JE, Pipes RL, Randall JP (1948) A neous abdominal and perineal approach in operations for imperforate anus with atresia of the rectum and rectosigmoid Ann Surg 127(3):552–556

38 Stephens FD (1953) Congenital imperforated rectum, recto-urethral and recto-vaginal fi stulae Aust N Z J Surg 22(3):161–172

39 Browne D (1955) Congenital deformities of the anus and the rectum Arch Dis Child 30(149):42–45

40 Potts WJ, Riker WL, DeBoer A (1954) Imperforate anus with recto-vesical, -urethral-vaginal and -peri- neal fi stula Ann Surg 140(3):381–395

41 Scott JE, Swenson O, Fisher JH (1960) Some ments on the surgical treatment of imperforate anus

com-Am J Surg 99:137–143

42 Kiesewetter WB, Turner CR, Steber WK (1964) Imperforate anus Review of a sixteen year experience with 146 patients Am J Surg 107:412–421

43 Kiesewetter W (1967) Imperforate anus the rationale and technic of the sacrabdominoperineal operation J Pediatr Surg 2(2):106–110

44 Rehbein F (1967) Imperforate anus: experiences with abdomino-perineal and abdomino-sacro-perineal pull-through procedures J Pediatr Surg 2(2):99–105

http://dx.doi.org/10.1016/S0022-3468(67)80291-4

45 Santulli TV (1952) The treatment of imperforate anus and associated fi stulas Surg Gynecol Obstet 95(5):601–614

46 Hanley PH, Hines MO, Ray JE (1958) Management

of imperforate anus Dis Colon Rectum 2(3):284–290 References