Ebook Surgical treatment of colorectal problems in children: Part 1

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


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

(eBook)

Library of Congress Control Number: 2015937190
Springer Cham Heidelberg New York Dordrecht London
© Springer International Publishing Switzerland 2015
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Printed on acid-free paper
Springer International Publishing AG Switzerland is part of Springer Science+Business Media
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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 anatomic 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 difficult 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 (vestibular) 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 honest 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 modify 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 textbook, but rather an observational personalized approach based on their

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Foreword I

viii

considerable experience in the care of these children. The book contains 27
chapters that cover the broad spectrum of colorectal conditions from the complex 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 management. To his credit Dr. Peña has been a strong advocate of establishing multidisciplinary 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 inflammatory 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 techniques 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 thorough 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 experience 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 management 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 providing 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 thorough 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 techniques. 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 welldocumented text ever written on the surgical treatment of colorectal problems
in children. This book provides an authoritative and complete account of various 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

ix



Preface

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 problems in children.
From the time of the first description of the posterior sagittal anorectoplasty for the treatment of anorectal malformations in 1982 until the publication 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 confronting 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 anatomy of these defects. After 1982, the descriptions of our surgical technique
were not like descriptions of any other surgical technique, in which the surgeons already know the anatomy, for instance, the mediastinum or the intraabdominal 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 technique with better, rather impeccable, audiovisual material in order to be successful 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 illustrations and photographs all that we have learned. We want this to be essentially
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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 general pediatric surgeon to be exposed to all the different anatomical variants of
these defects. There is not a single technique to repair all anorectal malformations; 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 introduced 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 illustrations 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 represented 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 communication 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 inflammatory 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 malformations, some surgeons have tried to compare the results of the posterior sagittal
anorectoplasty with other techniques; they found a serious limitation: the

Preface


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 malformations, even in the year 2014, unfortunately are still presented following the
old nomenclature discussing “high,” “intermediate,” and “low” malformations. 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 anomalies 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 generation of pediatric surgeons our passion for delicate, meticulous, fine surgical 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 serious 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 malformations 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 procedure, and provoke serious, permanent sequelae as a consequence of our bad
operation.
Cincinnati, OH, USA
Cincinnati, OH, USA

Alberto Peña
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 prominent daily participation in our task. We want to express our gratitude to hundreds 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

xv



Contents

1

History of the Treatment of Anorectal Malformations . . . . . . .
1.1 Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
1.2 The Early Times . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .

1
1
2
14

2

Basic Anatomy and Physiology of Bowel Control . . . . . . . . . . .
2.1 Internal Sphincter . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
2.2 General Anatomic Principles in Anorectal
Malformations . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
2.3 Nerves . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .

2.4 Blood Supply . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
2.5 Basic Physiology Principles of Bowel Control . . . . . . . . . . .
References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .

17
20

3

Prenatal Diagnosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
3.1 Male Fetuses. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
3.1.1 Abnormal Sacrum . . . . . . . . . . . . . . . . . . . . . . . . . . .
3.1.2 Tethered Cord . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
3.1.3 Absent Kidney . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
3.1.4 Vertebral Anomalies . . . . . . . . . . . . . . . . . . . . . . . . .
3.1.5 Hydronephrosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
3.2 Female Fetuses . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
3.2.1 Dilated Bowel and Intraluminal Calcifications . . . . .
3.2.2 Pelvic Cystic Mass . . . . . . . . . . . . . . . . . . . . . . . . . . .
3.2.3 Cloacal Exstrophy . . . . . . . . . . . . . . . . . . . . . . . . . . .
References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .

27
27
28
28
28
29
29
30

30
30
30
31

4

Neonatal Management . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
4.1 Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
4.2 Most Common Scenario. . . . . . . . . . . . . . . . . . . . . . . . . . . . .
4.3 Answering the Two Most Important Questions . . . . . . . . . . .
4.4 Physical Examination . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
4.4.1 Male Patients . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
4.5 Female Babies . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
4.6 Neonatal Management . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
4.7 Cloacal Exstrophy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .

33
33
33
33
35
35
39
41
46
47

20

22
22
23
24

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5

Colostomy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
5.1 Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
5.2 Stoma Locations . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
5.3 Ileostomies . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
5.4 To Divert or Not to Divert, That Is the Question . . . . . . . . .
5.5 Recommended Types of Colostomies . . . . . . . . . . . . . . . . .
5.5.1 Newborn Babies with Anorectal Malformations . . .
5.6 Left Transverse Colostomy . . . . . . . . . . . . . . . . . . . . . . . . .
5.7 Cecostomies . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
5.8 Creation of a Colostomy . . . . . . . . . . . . . . . . . . . . . . . . . . .
5.8.1 Surgical Technique . . . . . . . . . . . . . . . . . . . . . . . . .
5.9 Colostomy in Cases of Cloaca with Hydrocolpos . . . . . . . .
5.10 Other Types of Colostomies . . . . . . . . . . . . . . . . . . . . . . . . .
5.11 Colostomy Care . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
5.12 Colostomy Closure . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
5.13 Surgical Technique . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .

5.14 Errors and Complications in Colostomies . . . . . . . . . . . . . .
5.15 The Case of Upper Sigmoidostomy . . . . . . . . . . . . . . . . . . .
5.16 Prolapse . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
5.17 Surgical Treatment for Prolapse . . . . . . . . . . . . . . . . . . . . . .
5.18 Malposition of the Stomas . . . . . . . . . . . . . . . . . . . . . . . . . .
References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .

49
49
51
52
53
54
54
55
55
55
55
59
60
60
61
62
65
68
70
71
72
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6

Imaging . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
6.1 Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
6.2 Prenatal Diagnosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
6.3 Neonatal Imaging . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
6.4 Determination of the Fistula Location Prior
to the Colostomy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
6.4.1 Anatomic Facts and Timing . . . . . . . . . . . . . . . . . . .
6.5 The Old Invertogram . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
6.6 High-Pressure Distal Colostogram. . . . . . . . . . . . . . . . . . . .
6.7 Technique . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
6.8 Most Common Errors. . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
6.9 Not Showing the Coccyx and the Sacrum During
the Fluoroscopy Studies . . . . . . . . . . . . . . . . . . . . . . . . . . . .
6.10 Distal Colostogram in Female Patients . . . . . . . . . . . . . . . .
6.11 Distal Colostogram in Cloacas . . . . . . . . . . . . . . . . . . . . . . .
6.12 Monitoring Constipation . . . . . . . . . . . . . . . . . . . . . . . . . . .
6.13 Radiology During the Bowel Management Program . . . . . .
6.14 Monitoring the Urinary Tract . . . . . . . . . . . . . . . . . . . . . . . .
References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .

77
77
77
77

7

Bowel Preparation in Pediatric Colorectal Surgery . . . . . . . . .

7.1 Major Procedures . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
7.2 Primary Procedures for the Treatment of Anorectal
Malformation During the Newborn Period . . . . . . . . . . . . .
7.3 Primary Pull-Through in Newborn Patients
with Hirschsprung’s Disease . . . . . . . . . . . . . . . . . . . . . . . .

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90
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7.4

Patients with Hirschsprung’s Disease with
Enterocolitis After the Neonatal Period . . . . . . . . . . . . . . . .
7.5 Patients with Hirschsprung’s Disease Beyond
the Neonatal Period, Without Enterocolitis . . . . . . . . . . . . .
7.6 Colostomy Closures . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
7.7 Patients with a Colostomy Who Will Have
a Repair of an Anorectal Malformation . . . . . . . . . . . . . . . .
References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .

105
105

8

Recto-perineal Fistula. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
8.1 Definition, Frequency, and Prognosis . . . . . . . . . . . . . . . . .
8.2 Associated Defects . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
8.3 Diagnosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
8.3.1 Female Patients . . . . . . . . . . . . . . . . . . . . . . . . . . . .
8.3.2 Male Patients . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
8.4 Management . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
8.5 Dilatations . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
8.6 Cutback Operation . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
8.7 Minimal Posterior Sagittal Anoplasty . . . . . . . . . . . . . . . . .
8.7.1 Male Patients . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
8.7.2 Surgical Technique . . . . . . . . . . . . . . . . . . . . . . . . .
8.7.3 Female Patients . . . . . . . . . . . . . . . . . . . . . . . . . . .
8.8 Postoperative Care . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .

107
107
109
110
110
110
117
117
117
118
118
119
122
123
125

9

Rectourethral Bulbar Fistula . . . . . . . . . . . . . . . . . . . . . . . . . . . .
9.1 Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
9.2 Associated Defects . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
9.3 Posterior Sagittal Anorectoplasty. . . . . . . . . . . . . . . . . . . . .
9.4 Surgical Technique . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
9.5 Functional Results . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .

129
129
129

132
132
148
148

10

Rectourethral Prostatic Fistula . . . . . . . . . . . . . . . . . . . . . . . . . .
10.1 Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
10.2 Associated Defects . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
10.3 Surgical Repair . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
10.4 Posterior Sagittal Anorectoplasty. . . . . . . . . . . . . . . . . . . . .
10.5 Postoperative Care and Functional Results . . . . . . . . . . . . .
References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .

151
151
151
153
153
161
161

11

Recto-bladder Neck Fistula . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
11.1 Definition and Frequency . . . . . . . . . . . . . . . . . . . . . . . . . . .
11.2 Associated Defects . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
11.2.1 Sacral Defects. . . . . . . . . . . . . . . . . . . . . . . . . . . . .
11.2.2 Spinal-Associated Defects . . . . . . . . . . . . . . . . . . .

11.2.3 Urologic-Associated Defects . . . . . . . . . . . . . . . . .
11.2.4 Gastrointestinal-Associated Defects . . . . . . . . . . .
11.2.5 Neurosurgical-Associated Defects . . . . . . . . . . . . .
11.2.6 Cardiovascular-Associated Defects . . . . . . . . . . . .
11.2.7 Other Associated Defects . . . . . . . . . . . . . . . . . . . .

163
163
164
164
164
164
165
165
165
165

104

105
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11.3 Diagnosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
11.4 Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
11.4.1 Colostomy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .

11.4.2 Main Repair . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
11.4.3 Laparotomy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
11.4.4 Laparoscopy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
11.5 Special Problems . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
11.5.1 Dealing with Inadequate Colostomies
(Too Distal) . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
11.6 Functional Results . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
11.6.1 Fecal Control . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
11.6.2 Urinary Control . . . . . . . . . . . . . . . . . . . . . . . . . . .
References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .

165
166
166
166
168
178
179

12

Imperforate Anus Without Fistula in Males and Females . . . .
12.1 Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
12.2 Anatomic Characteristics . . . . . . . . . . . . . . . . . . . . . . . . . . .
12.3 Main Repair . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
12.4 Function and Results . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .

183
183

184
185
187
187

13

Minimally Invasive Approach to Anorectal Malformations . . .
13.1 Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
13.2 Males . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
13.3 Females . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .

189
189
192
194
196

14

Rectal Atresia . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
14.1 Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
14.2 Surgical Repair . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .

201
202
202
204

15

Rectovestibular Fistula . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
15.1 Definition/Frequency . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
15.2 Associated Defects . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
15.2.1 Sacral . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
15.2.2 Spinal . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
15.2.3 Urologic . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
15.2.4 Gynecologic . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
15.2.5 Gastrointestinal . . . . . . . . . . . . . . . . . . . . . . . . . . .
15.2.6 Tethered Cord . . . . . . . . . . . . . . . . . . . . . . . . . . . .
15.2.7 Cardiovascular . . . . . . . . . . . . . . . . . . . . . . . . . . .
15.3 Diagnosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
15.4 Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
15.4.1 Colostomy or No Colostomy . . . . . . . . . . . . . . . .
15.5 Main Repair . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
15.6 Complications . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
15.7 Functional Results . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
15.8 Reoperations in Patients with Vestibular Fistula . . . . . . . .
15.9 Surgical Technique . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
15.10 Rectovestibular Fistula with Normal Anus . . . . . . . . . . . .
References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .

205
205
207
208
208
208

208
209
209
209
211
212
212
213
219
219
220
222
223
223

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16

Cloaca, Posterior Cloaca and Absent Penis Spectrum . . . . . . .
16.1 Cloaca. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .

16.1.1 Definition and Management . . . . . . . . . . . . . . . . . . . .
16.1.2 Urologic Concerns . . . . . . . . . . . . . . . . . . . . . . . . . . .
16.1.3 Gynecologic Concerns . . . . . . . . . . . . . . . . . . . . . . . .
16.1.4 Reoperations. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
16.1.5 Transpubic Approach . . . . . . . . . . . . . . . . . . . . . . . . .
16.2 Posterior Cloaca and Absent Penis Spectrum . . . . . . . . . . . .
16.2.1 Surgical Repair . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
16.2.2 Surgical Repair of the 2-Perineal-Orifice
Variant of the Posterior Cloacal Spectrum . . . . . . . .
16.2.3 Posterior Cloaca and Absent Penis . . . . . . . . . . . . . .
References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .

225
225
225
260
261
262
268
270
275

17

Cloacal Exstrophy and Covered Cloacal Exstrophy . . . . . . . . .
17.1 Neonatal Approach. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
17.2 Pull-Through or “Permanent Stoma” . . . . . . . . . . . . . . . . . . .
17.3 Covered Cloacal Exstrophy . . . . . . . . . . . . . . . . . . . . . . . . . .
References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .

285
289
291
293
295

18

General Principles for the Postoperative Management
of Patients with Anorectal Malformations . . . . . . . . . . . . . . . . .
18.1 General Care. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
18.2 Local Care . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
18.3 Anal Dilatations . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
18.4 Avoiding Constipation . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
18.5 Toilet Training . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .

299
299
300
300
303
305

276
276
279

19

Postoperative Evaluation . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 307

References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 309

20

Bowel Management for the Treatment
of Fecal Incontinence . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
20.1 Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
20.2 Goals of the Bowel Management Program . . . . . . . . . . . .
20.3 Evaluation of the Patient for Bowel Management . . . . . . .
20.4 Individualization of the Management . . . . . . . . . . . . . . . .
20.5 Laxative Trial . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
20.6 About Our Program . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
20.7 Content of the Enema . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
20.8 Rationale to Change the Type of Enema . . . . . . . . . . . . . .
20.9 Bowel Management for the Treatment of Severe
Diaper Rash . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
20.10 Bowel Management Through a Stoma . . . . . . . . . . . . . . . .
References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .

21

Operations for the Administration of Antegrade Enemas . . . .
21.1 Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
21.2 Our Preferred Technique . . . . . . . . . . . . . . . . . . . . . . . . . . . .
21.3 Surgical Technique: Continent Appendicostomy. . . . . . . . . .
21.4 Continent Neo-appendicostomy . . . . . . . . . . . . . . . . . . . . . . .
References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .

311
311

314
315
316
320
321
323
325
329
330
331
333
333
334
336
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22

23

24

Reoperations . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
22.1 Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .

22.2 Reoperations to Improve Bowel Control . . . . . . . . . . . . . . . .
22.3 Reoperations Performed After Failed Attempted
Repair (Catastrophes) Males . . . . . . . . . . . . . . . . . . . . . . . . .
22.4 Reoperations for Postoperative Recto-urinary Fistula . . . . . .
22.4.1 Recurrent Fistula (17 Cases) . . . . . . . . . . . . . . . . . . .
22.4.2 Persistent Rectourethral Fistula (24 Cases). . . . . . . .
22.4.3 Acquired Fistula (9 Cases) . . . . . . . . . . . . . . . . . . . .
22.5 Posterior Urethral Diverticulum (32 Cases) . . . . . . . . . . . . . .
22.6 Acquired Rectal Atresia or Stenosis (83 Cases) . . . . . . . . . .
22.7 Presacral Masses . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
22.8 Reoperations in Female Patients . . . . . . . . . . . . . . . . . . . . . .
22.9 Prolapse . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .

349
349
350
356
357
357
359
360
361
361
362
362
363
366

Urologic Problems in Anorectal Malformations . . . . . . . . . . . .

23.1 Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
23.2 Neonatal Approach. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
23.3 The Importance of the Colostomy Type from
the Urologic Point of View. . . . . . . . . . . . . . . . . . . . . . . . .
23.4 Most Common Urologic Abnormalities in Male
Patients with Anorectal Malformations . . . . . . . . . . . . . . .
23.4.1 Absent Kidney . . . . . . . . . . . . . . . . . . . . . . . . . . .
23.4.2 Urethral Problems . . . . . . . . . . . . . . . . . . . . . . . . .
23.5 Bifid Scrotum . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
23.6 Hypospadias . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
23.7 Ectopic Ureters in Males . . . . . . . . . . . . . . . . . . . . . . . . . .
23.8 Ectopic Ureters in Females . . . . . . . . . . . . . . . . . . . . . . . .
23.9 Ectopic Vas Deferens . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
23.10 Ectopic Verumontanum . . . . . . . . . . . . . . . . . . . . . . . . . . .
23.11 Megalourethra . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
23.12 Ureterovesical and Ureteropelvic Obstruction . . . . . . . . . .
23.13 Neurogenic Bladder . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
23.14 Postoperative Problems . . . . . . . . . . . . . . . . . . . . . . . . . . .
23.15 Posterior Urethral Diverticulum . . . . . . . . . . . . . . . . . . . . .
23.16 Sexual Problems . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
23.17 Tethered Cord . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
23.18 The Ultimate Concern, Kidney Function . . . . . . . . . . . . . .
References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .

371
371
372

373
373

376
377
380
382
382
384
385
386
386
386
389
390
391
391
392
392

Hirschsprung’s Disease. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
24.1 Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
24.2 Historical Review . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
24.3 Incidence, Inheritance, and Associated Anomalies . . . . . .
24.4 Pathogenesis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
24.5 Genetics . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
24.6 Clinical Manifestations and Differential Diagnosis . . . . . .
24.7 Histologic Diagnosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
24.8 Differential Diagnosis . . . . . . . . . . . . . . . . . . . . . . . . . . . .

397
397
398

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400
403
403
405
406

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25

26

24.9 Early Management . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
24.10 Surgical Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
24.10.1 The Authors’ Approach . . . . . . . . . . . . . . . . . . .
24.10.2 Other Surgical Techniques for the
Treatment of Hirschsprung’s Disease . . . . . . . . .
24.11 Total Colonic Aganglionosis . . . . . . . . . . . . . . . . . . . . . . .
24.12 Ultrashort-Segment Hirschsprung’s Disease . . . . . . . . . . .
24.13 Problems, Complication, and Sequela Secondary
to Operations for Hirschsprung’s Disease . . . . . . . . . . . . .
24.13.1 Preventable Complications (Catastrophes) . . . . . .
24.13.2 Non-preventable Complications . . . . . . . . . . . . . .
24.13.3 Partially Preventable Complications. . . . . . . . . . .

References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .

407
407
408

Idiopathic Constipation and Other Motility Disorders . . . . . . .
25.1 Definition and Terminology . . . . . . . . . . . . . . . . . . . . . . . .
25.2 Incidence, Social Impact, and Relevance . . . . . . . . . . . . . .
25.3 Etiology . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
25.3.1 Ultrashort Segment Hirschsprung’s Disease . . . .
25.3.2 Rectal Manometry . . . . . . . . . . . . . . . . . . . . . . . .
25.3.3 Doubts and Questions About the Anatomy
of the Internal Sphincter . . . . . . . . . . . . . . . . . . . .
25.3.4 Questions About Myectomy Technique . . . . . . . .
25.3.5 Botulinum Toxin Injection . . . . . . . . . . . . . . . . . .
25.4 Pathogenesis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
25.5 Natural History and Clinical Manifestations . . . . . . . . . . .
25.6 Diagnosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
25.6.1 Colonic Transit Time . . . . . . . . . . . . . . . . . . . . . .
25.6.2 The Evaluation of Severity: Search
for Objective “Instruments” . . . . . . . . . . . . . . . . .
25.7 Management . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
25.7.1 Fecal Disimpaction Protocol . . . . . . . . . . . . . . . .
25.7.2 Determination of Laxative Requirements. . . . . . .
25.7.3 Electric Stimulation . . . . . . . . . . . . . . . . . . . . . . .
25.8 Surgical Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
25.8.1 Operations to Administer Antegrade Enemas
(ACE Procedures) . . . . . . . . . . . . . . . . . . . . . . . . .
25.8.2 Colonic Resection . . . . . . . . . . . . . . . . . . . . . . . . .

References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .

435
435
435
435
436
437

Posterior Sagittal Approach for the Treatment
of Other Conditions . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
26.1 The Kraske Operation . . . . . . . . . . . . . . . . . . . . . . . . . . . .
26.2 Urogenital Sinus with Normal Rectum . . . . . . . . . . . . . . .
26.3 Urogenital Sinus with Normal Rectum
and Adrenal Hyperplasia . . . . . . . . . . . . . . . . . . . . . . . . . .
26.4 Acquired Urethral Atresia . . . . . . . . . . . . . . . . . . . . . . . . .
26.5 Acquired Rectourethral Fistula . . . . . . . . . . . . . . . . . . . . .
26.6 Giant Seminal Vesicle . . . . . . . . . . . . . . . . . . . . . . . . . . . .
26.7 Urethral Tumors . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .

417
422
425
425
425
429
430
430

437

438
438
440
442
443
445
446
447
448
449
450
450
450
451
453
457
457
458
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26.8

26.9
26.10
26.11
26.12

27

Acquired Rectovaginal Fistula . . . . . . . . . . . . . . . . . . . . . .
Rectal Tumors . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
Presacral Masses . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
Surgical Technique . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
Posterior Sagittal Approach, Its Application in Cases
with Hirschsprung’s Disease . . . . . . . . . . . . . . . . . . . . . . .
26.13 Vaginal Atresia with Normal Rectum . . . . . . . . . . . . . . . .
References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .

473
475
479
480

Miscellaneous Conditions . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
27.1 Part I: Perianal Abscess and Fistula . . . . . . . . . . . . . . . . . .
27.1.1 Definition . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
27.1.2 Etiology . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
27.1.3 Natural History . . . . . . . . . . . . . . . . . . . . . . . . . . .
27.1.4 Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
27.1.5 Fistulotomy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
27.2 Part II: Perianal Fistula and Rectovestibular Fistula
with Normal Anus in Females . . . . . . . . . . . . . . . . . . . . . .

27.2.1 Surgical Treatment . . . . . . . . . . . . . . . . . . . . . . . .
27.3 Part III: Other Conditions. . . . . . . . . . . . . . . . . . . . . . . . . .
27.3.1 Anal Fissure . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
27.3.2 Hemorrhoids . . . . . . . . . . . . . . . . . . . . . . . . . . . . .
27.3.3 Idiopathic Rectal Prolapse . . . . . . . . . . . . . . . . . .
References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .

487
487
487
488
488
489
489

481
482
484

490
490
493
493
493
493
496