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

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

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

vii
viii Foreword I

considerable experience in the care of these children. The book contains 27

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

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

In 1982, Prof. Alberto Pena made an outstanding contribution to the manage-

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

Dublin, Ireland Prem Puri

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

xi
xii Preface

a practical book, a reliable guide for all pediatric surgeons and pediatric
urologists.
A very important motivation for us to write this book is the fact that we
receive many patients who underwent technically deficient operations in
other parts of the world and suffered serious complications. Therefore, this
book puts particular emphasis on the surgical technique and attention to
details as well as the importance of observing meticulous delicate operations.
Since anorectal malformations represent a spectrum, it is difficult for a gen-
eral pediatric surgeon to be exposed to all the different anatomical variants of
these defects. There is not a single technique to repair all anorectal malforma-
tions; each type of defect represents a different anatomical variant, and the
surgeon must be prepared to deal with it. We also wanted to put emphasis on
clarity and simplicity.
All cases discussed in this book were operated by the senior author and by
the junior author during the last 5 years.
In order to make all the concepts more understandable and simple, dealing
with an anatomical area that is not well known by most surgeons, we intro-
duced another modality of teaching, represented by 27 animations, that we
hope will simplify the understanding of the surgical techniques and concepts
expressed in this book.
We are very proud to present an entire book with illustrations made by a
single person. All illustrations in this book were made by Ms. Lois Barnes
under the personal guidance of the senior author. Ms. Barnes is an excellent
medical illustrator and old friend with whom we have been working for
30 years. It took many years to establish a unique form of communication
between the medical illustrator and us. Due to the large number of illustra-
tions that she performed under our guidance, it is a great pleasure nowadays
to have a common language between a surgeon and an artist. We speak the
same language. Therefore, the illustrations made in the last few years repre-
sented much less effort from both surgeons and the illustrator.
The animations required, again, many hours of working together with
ingenious, intelligent experts in computer animations and establishing a com-
munication between a surgical mind and an engineer, computer expert type of
mind. We believe that in the future, teaching surgery will be highly simplified
with this kind of audiovisual material.
The reader will find that even though the book is related to the surgical
treatment of colorectal problems in children, we did not include inflamma-
tory bowel disease. The reason for that is simple: we do not have enough
experience as to be able to say something new and (or) different from what is
already written in the enormous literature on the subject.
The reader will also be surprised to find that even though this book was
finished in November 2014, it is not considered an “evidence-based surgical
book.” It is rather a book based on personal observations made through a
large experience in the management of these problems. We understand very
well that the future in surgery will be related very much to the concept of
“evidence-based” procedures. However, in dealing with anorectal malforma-
tions, some surgeons have tried to compare the results of the posterior sagittal
anorectoplasty with other techniques; they found a serious limitation: the
Preface xiii

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

Cincinnati, OH, USA Alberto Peña

Cincinnati, OH, USA Andrea Bischoff
Acknowledgments

We would like to sincerely thank innumerable individuals for contributing

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

Sincerely,
Alberto Peña and Andrea Bischoff

xv
Contents

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 Calcifications . . . . . 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

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xviii 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 xix

7.4 Patients with Hirschsprung’s Disease with

Enterocolitis After the Neonatal Period . . . . . . . . . . . . . . . . 104
7.5 Patients with Hirschsprung’s Disease Beyond
the Neonatal Period, Without Enterocolitis . . . . . . . . . . . . . 105
7.6 Colostomy Closures . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 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 Definition, 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 Definition 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
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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 Definition/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
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16 Cloaca, Posterior Cloaca and Absent Penis Spectrum . . . . . . . 225

16.1 Cloaca. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 225
16.1.1 Definition and Management . . . . . . . . . . . . . . . . . . . . 225
16.1.2 Urologic Concerns . . . . . . . . . . . . . . . . . . . . . . . . . . . 260
16.1.3 Gynecologic Concerns . . . . . . . . . . . . . . . . . . . . . . . . 261
16.1.4 Reoperations. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 262
16.1.5 Transpubic Approach . . . . . . . . . . . . . . . . . . . . . . . . . 268
16.2 Posterior Cloaca and Absent Penis Spectrum . . . . . . . . . . . . 270
16.2.1 Surgical Repair . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 275
16.2.2 Surgical Repair of the 2-Perineal-Orifice
Variant of the Posterior Cloacal Spectrum . . . . . . . . 276
16.2.3 Posterior Cloaca and Absent Penis . . . . . . . . . . . . . . 276
References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 279
17 Cloacal Exstrophy and Covered Cloacal Exstrophy . . . . . . . . . 285
17.1 Neonatal Approach. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 289
17.2 Pull-Through or “Permanent Stoma” . . . . . . . . . . . . . . . . . . . 291
17.3 Covered Cloacal Exstrophy . . . . . . . . . . . . . . . . . . . . . . . . . . 293
References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 295
18 General Principles for the Postoperative Management
of Patients with Anorectal Malformations . . . . . . . . . . . . . . . . . 299
18.1 General Care. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 299
18.2 Local Care . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 300
18.3 Anal Dilatations . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 300
18.4 Avoiding Constipation . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 303
18.5 Toilet Training . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 305
19 Postoperative Evaluation . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 307
References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 309
20 Bowel Management for the Treatment
of Fecal Incontinence . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 311
20.1 Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 311
20.2 Goals of the Bowel Management Program . . . . . . . . . . . . 314
20.3 Evaluation of the Patient for Bowel Management . . . . . . . 315
20.4 Individualization of the Management . . . . . . . . . . . . . . . . 316
20.5 Laxative Trial . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 320
20.6 About Our Program . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 321
20.7 Content of the Enema . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 323
20.8 Rationale to Change the Type of Enema . . . . . . . . . . . . . . 325
20.9 Bowel Management for the Treatment of Severe
Diaper Rash . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 329
20.10 Bowel Management Through a Stoma . . . . . . . . . . . . . . . . 330
References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 331
21 Operations for the Administration of Antegrade Enemas . . . . 333
21.1 Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 333
21.2 Our Preferred Technique . . . . . . . . . . . . . . . . . . . . . . . . . . . . 334
21.3 Surgical Technique: Continent Appendicostomy. . . . . . . . . . 336
21.4 Continent Neo-appendicostomy . . . . . . . . . . . . . . . . . . . . . . . 339
References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 345
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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
22.7 Presacral Masses . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 362
22.8 Reoperations in Female Patients . . . . . . . . . . . . . . . . . . . . . . 362
22.9 Prolapse . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 363
References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 366
23 Urologic Problems in Anorectal Malformations . . . . . . . . . . . . 371
23.1 Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 371
23.2 Neonatal Approach. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 372
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 Bifid 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
23.10 Ectopic Verumontanum . . . . . . . . . . . . . . . . . . . . . . . . . . . 385
23.11 Megalourethra . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 386
23.12 Ureterovesical and Ureteropelvic Obstruction . . . . . . . . . . 386
23.13 Neurogenic Bladder . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 386
23.14 Postoperative Problems . . . . . . . . . . . . . . . . . . . . . . . . . . . 389
23.15 Posterior Urethral Diverticulum . . . . . . . . . . . . . . . . . . . . . 390
23.16 Sexual Problems . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 391
23.17 Tethered Cord . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 391
23.18 The Ultimate Concern, Kidney Function . . . . . . . . . . . . . . 392
References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 392
24 Hirschsprung’s Disease. . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 397
24.1 Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 397
24.2 Historical Review . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 398
24.3 Incidence, Inheritance, and Associated Anomalies . . . . . . 399
24.4 Pathogenesis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 400
24.5 Genetics . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 403
24.6 Clinical Manifestations and Differential Diagnosis . . . . . . 403
24.7 Histologic Diagnosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 405
24.8 Differential Diagnosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . 406
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24.9 Early Management . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 407

24.10 Surgical Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 407
24.10.1 The Authors’ Approach . . . . . . . . . . . . . . . . . . . 408
24.10.2 Other Surgical Techniques for the
Treatment of Hirschsprung’s Disease . . . . . . . . . 417
24.11 Total Colonic Aganglionosis . . . . . . . . . . . . . . . . . . . . . . . 422
24.12 Ultrashort-Segment Hirschsprung’s Disease . . . . . . . . . . . 425
24.13 Problems, Complication, and Sequela Secondary
to Operations for Hirschsprung’s Disease . . . . . . . . . . . . . 425
24.13.1 Preventable Complications (Catastrophes) . . . . . . 425
24.13.2 Non-preventable Complications . . . . . . . . . . . . . . 429
24.13.3 Partially Preventable Complications. . . . . . . . . . . 430
References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 430
25 Idiopathic Constipation and Other Motility Disorders . . . . . . . 435
25.1 Definition and Terminology . . . . . . . . . . . . . . . . . . . . . . . . 435
25.2 Incidence, Social Impact, and Relevance . . . . . . . . . . . . . . 435
25.3 Etiology . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 435
25.3.1 Ultrashort Segment Hirschsprung’s Disease . . . . 436
25.3.2 Rectal Manometry . . . . . . . . . . . . . . . . . . . . . . . . 437
25.3.3 Doubts and Questions About the Anatomy
of the Internal Sphincter . . . . . . . . . . . . . . . . . . . . 437
25.3.4 Questions About Myectomy Technique . . . . . . . . 438
25.3.5 Botulinum Toxin Injection . . . . . . . . . . . . . . . . . . 438
25.4 Pathogenesis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 440
25.5 Natural History and Clinical Manifestations . . . . . . . . . . . 442
25.6 Diagnosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 443
25.6.1 Colonic Transit Time . . . . . . . . . . . . . . . . . . . . . . 445
25.6.2 The Evaluation of Severity: Search
for Objective “Instruments” . . . . . . . . . . . . . . . . . 446
25.7 Management . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 447
25.7.1 Fecal Disimpaction Protocol . . . . . . . . . . . . . . . . 448
25.7.2 Determination of Laxative Requirements. . . . . . . 449
25.7.3 Electric Stimulation . . . . . . . . . . . . . . . . . . . . . . . 450
25.8 Surgical Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 450
25.8.1 Operations to Administer Antegrade Enemas
(ACE Procedures) . . . . . . . . . . . . . . . . . . . . . . . . . 450
25.8.2 Colonic Resection . . . . . . . . . . . . . . . . . . . . . . . . . 451
References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 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
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26.8 Acquired Rectovaginal Fistula . . . . . . . . . . . . . . . . . . . . . . 473

26.9 Rectal Tumors . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 475
26.10 Presacral Masses . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 479
26.11 Surgical Technique . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 480
26.12 Posterior Sagittal Approach, Its Application in Cases
with Hirschsprung’s Disease . . . . . . . . . . . . . . . . . . . . . . . 481
26.13 Vaginal Atresia with Normal Rectum . . . . . . . . . . . . . . . . 482
References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 484
27 Miscellaneous Conditions . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 487
27.1 Part I: Perianal Abscess and Fistula . . . . . . . . . . . . . . . . . . 487
27.1.1 Definition . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 487
27.1.2 Etiology . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 488
27.1.3 Natural History . . . . . . . . . . . . . . . . . . . . . . . . . . . 488
27.1.4 Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 489
27.1.5 Fistulotomy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 489
27.2 Part II: Perianal Fistula and Rectovestibular Fistula
with Normal Anus in Females . . . . . . . . . . . . . . . . . . . . . . 490
27.2.1 Surgical Treatment . . . . . . . . . . . . . . . . . . . . . . . . 490
27.3 Part III: Other Conditions. . . . . . . . . . . . . . . . . . . . . . . . . . 493
27.3.1 Anal Fissure . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 493
27.3.2 Hemorrhoids . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 493
27.3.3 Idiopathic Rectal Prolapse . . . . . . . . . . . . . . . . . . 493
References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 496
 History of the Treatment
of Anorectal Malformations
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-
tific 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
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
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 commit-
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
2 1
publications that we reviewed, one can find 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 specific
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 paying much
attention to our classifications. 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 first 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].
History of the Treatment of Anorectal Malformations
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 finger-
nail of the little finger, to dilate the anus of those
babies who did not pass meconium after birth [5].
Paul of Aegina (625–690) made the first descrip-
tion of an operation for imperforate anus: “If pos-
sible, the membrane that covers the anus must be
divided with the finger. 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 first illustration describing an
anorectal procedure in pediatrics was found in a
book entitled Cerrahiyei Ilhaniye, written in
1465 by Dr. Sharaphedin in Turkey [7].
In 1606, Guilhelmus Fabricius Hildanus
described a case of a recto-bladder fistula. 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 immortal-
ized in a famous painting showing the autopsy of
a baby. He described the spontaneous 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.”
1.2 The Early Times
In 1753, M. Louis from Paris described the
case of a little girl who had an orifice 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 finally 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 find 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
fistula,” and “vaginal fistula.” Benjamin Bell was
probably the first 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 first 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,
3
was probably the first 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
fibers 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
first surgeon who decided to suture the wall of the
rectum to the skin edges, which could be consid-
ered the first anoplasty. After 28 days, the baby
was doing very well, without complications [20].
It was also Amussat who classified the anorectal
malformations into five 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 “deficient” 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 difficult to find 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 finger. That idea was practiced in
1872 by Leiserink, and he described a “good
result” [22].
4 1
In 1860, Bodenhamer [23] proposed a classifica-
tion dividing these malformations 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 finding 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 first 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 finger, 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-sacral one. This
was described by Maitre [28].
In 1894, Paul Delageniere suggested perform-
ing a lateral laparotomy to find the rectum and to
reach the perineum through the abdominal cavity,
using his finger 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
History of the Treatment of Anorectal Malformations
pelvic structures. He suggested entering the pel-
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 mal-
formations can be repaired through a peri-
neal approach.” Interestingly, this conclusion
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
1.2 The Early Times
must be performed strictly in the midline.”
He was right!
9. “The old method of stab of the perineum
without a proctoplasty was not justified.” 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 first 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.
5
21. “If, for some reason, the surgeon decided to
open a colostomy first, 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 cor-
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 dem-
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
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 find 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 excel-
lent paper in Surgical Gynecology and
Obstetrics and described his experience with
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 fibers, bowel con-
trol must be obtained by an axial rotation of the
gut or using some muscle fibers from the
gluteal region. Even though Brenner’s
conclusions are not valid at the present time,
his work is very significant, because of the
number of cases and the meticulous descrip-
tion of them.
6 1
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 film 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 film.
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
Fig. 1.1 Photograph –
Dr. Peña with Dr. Jonathan
Rhoad
History of the Treatment of Anorectal Malformations
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 significant 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 colosto-
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 fistula to the urinary tract untouched
until the patient was older!!
In 1948, Rhoads et al. [37] (Fig. 1.1) pub-
lished their experience with the first survivor of a
primary abdominoperineal pull-through, without
a colostomy. After that publication, many sur-
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.
1.2 The Early Times
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 justified, but not always.
In 1953, Douglas Stephens published his first
landmark paper on the subject, in Australia [38].
Dr. Stephens has the unique distinction of being
the first 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 sling,” 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 recommendations
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
fistulas 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,
fistulas have no sphincter mechanism, which, as
we know now, is mostly inaccurate.
7
In 1954, Dr. William Potts [40] published a
paper related to the treatment of 22 “rectovaginal
fistulas,” 8 rectourethral fistulas, 9 recto-perineal
fistulas, and 12 rectovesical fistulas. The ano-
plasty that he proposed for the most common
type of malformation seen in females that we
now know by the name of rectovestibular fistula
is still known as the “Potts’ anoplasty” and con-
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
“rectovaginal fistulas” were actually cases of rec-
tovestibular fistulas, since now we recognize that
real rectovaginal fistulas are extremely unusual
malformations. Unless we postulate the theory
that the type of pathology changes through the
years, it is difficult to believe that surgeons had
many cases of rectovaginal fistulas, which we
find now to be a malformation that is almost
nonexistent.
In 1960, Scott, Swenson, and Fisher published
one of the first 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 fistulas, which is simi-
lar to what we report.
8 1
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 field 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
classification of anorectal malformations.” That
“international classification” 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 different thera-
peutic modalities as well as the results of the
treatment of anorectal malformations and sent
History of the Treatment of Anorectal Malformations
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 fistula in males was 72 % and in
females was 19 %, and 28.7 % of these fistulas
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 fistulas 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
influenced 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
1.2 The Early Times 9

Melbourne classification, 1970 Melbourne classification, 1970

Males Females

High deformities High deformities

1. Anorectal agenesis
1. Anorectal agenesis
a. Without fistula
a. Without fistula
b. With fistula
Ano-rectal agenesis (No fistula)
i. Recto-vesical fistula
b. With fistula
ii. Recto-cloacal fistula
i. Recto-vesical fistula
iii. Recto-vaginal fistula
ii. Recto-urethral fistula
2. Rectal atresia
2. Rectal atresia
Intermediate deformities
Intermediate deformities
1. Anal agenesis
1. Anal agenesis
a. Without fistula
a. Without fistula
Anal Agenesis (no fistula)
Anal Agenesis (no fistula)
b. With fistula
b. With fistula
i. Recto-Vaginal fistula
Recto-bulbar fistula
ii. Recto-vestibular fistula
2. Ano-rectal stenosis
2. Ano-rectal stenosis
Low deformities (translevator)
Low deformities (translevator)
1. At normal anal site 1. At normal anal site
i. Covered anus - complete i. Covered anus - complete
ii. Anal stenosis ii. Anal stenosis
Covered anal stenosis Covered anal stenosis
2. At perineal site 2. At perineal site
i. Anterior Perineal Anus i. Anterior Perineal Anus
ii. Ano-cutaneous fistula ii. Ano-cutaneous fistula
(Covered anus – incomplete) (Covered anus – incomplete)
3. At vulvar site
Miscellaneous deformities
i. Vulvar anus
i. Imperforate anal membrane
ii. Ano-vulvar fistula
ii. Anal membrane stenosis
iii. Ano-vestibular fistula
iii. Vesico-Intestinal Fissure
iv. Duplications of the anus, rectum and genitourinary tracts Miscellaneous deformities
v. Combination of 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 classification of anorectal malformation. Melbourne, Australia 1971

8 years, becoming longer and longer. In addition,
he decided to use an electrical stimulator with the
specific 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 specifically 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 Pacific 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 fistula 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 significant discus-
sion, particularly between the Australian mem-
bers of the audience. They specifically 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
10
“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 Pacific 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 first
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 Pacific 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 first 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 classification and to rediscuss the whole
subject of anorectal malformation in Wingspread,
1 History of the Treatment of Anorectal Malformations
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 classification was
created, called the “Wingspread classification.”
Fortunately, that new classification was more
simplified and therefore more useful and yet,
from our point of view, was still very deficient
(Fig. 1.4 Wingspread classification) [70].
In May of 2005, Dr. Holschneider promoted
another meeting in Krickenbeck, Germany
(Fig. 1.5), to rediscuss the subject and a new clas-
sification, known as Krickenbeck classification,
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
findings 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
1.2 The Early Times
Fig. 1.3 Picture of the group of pediatric surgeons who met in Wingspread
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 first 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
11
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 rec-
tum from the urogenital tract under direct vision
is a technically demanding maneuver; it is there-
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
oversimplified classifications 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
on, the wider the spectrum becomes.
Classifications that divide a spectrum of defects,
in categories such as “high” and “low” or even
“high,” “intermediate,” and “low,” represent
oversimplifications that misguide rather than
help. Now we know that in surgery, it is not
12
Fig. 1.4 Wingspread
classification of anorectal
malformation
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 findings
with the final 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.
1 History of the Treatment of Anorectal Malformations
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
1.2 The Early Times 13

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

Standards for diagnosis international classification (Krickenbeck) technology. Willital [72] published his attempt to
repair an anorectal malformation using this thera-
Major clinical groups Rare/regional variants
Perineal (cutaneous) fistula Pouch colon peutic modality. In the year 2000, Dr. Keith
Rectourethral fistula Rectal atresia/stenosis Georgeson published his experience with seven
Prostatic Rectovaginal fistula
Bulbar H fistula cases, in a detailed description of the operation
Rectovesical fistula Others [73]. Following Georgeson, many other surgeons
Vestibular fistula
Cloaca have been using minimally invasive techniques to
No fistula repair anorectal malformations (see Chap. 13).
Anal stenosis
In July 1985, the senior author moved to Long
Fig. 1.6 Krickenbeck classification of anorectal Island, New York, United States, and became
malformation Chief of Pediatric Surgery at Schneider Children’s
Hospital until June 30, 2005. During those
protects the innocent,” since that case from 20 years, we were able to accumulate a very large
Monterrey, seen in retrospect, after having oper- experience and moved forward in our attempt to
ated on over 531 patients with a cloaca, we now benefit more children. We were able to:
belongs to the “good side of the spectrum” of clo- • Find further applications for the posterior sag-
acas. Later on, we would be confronted with ittal approach [74, 75]
much more challenging cases. To repair those • Describe the transanorectal approach for the
complex cases would require a great deal of treatment of urogenital sinus with normal rec-
creativity, imagination, and dedication. tum and other conditions [76, 77].
A very important positive development occurred • Create a protocol of medical and surgical
in the field of colorectal problems of children and management of patients with idiopathic con-
that is the introduction of the minimally invasive stipation [78].
14
Fig. 1.7 Picture taken during the first posterior sagittal approach to repair a cloaca in Monterrey, Mexico
• 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 deficien-
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 benefit
from the advice of a pediatric gynecologist.
There was an obvious conclusion that these
1 History of the Treatment of Anorectal Malformations
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.
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57. Varma KK (1991) Long-term continence after surgery
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58. Chatterjee SK, Talukder BC (1969) Double termina-
tion of the alimentary tract in female infants. J Pediatr
Surg 4(2):237–243
59. Rintala RJ (1996) Anorectal malformation-
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60. Endo M, Hayashi A, Ishihara M, Maie M, Nagasaki
A, Nishi T, Saeki M (1999) Analysis of 1,992 patients
with anorectal malformations over the past two
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61. Schärli AF (1986) History of Colostomy in child-
hood. Prog Pediatr Surg 20:188–198
62. Ito Y, Yokoyama J, Hayashi A, Ihara N, Katsumata
K (1981) Reappraisal of endorectal pull-through pro-
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63. Brayton D, Norris WJ (1958) Further experiences
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64. Aluwihare AP (1989) Imperforate anus in male chil-
dren: a new operation of primary perineal rectoure-
throanoplasty. Ann R Coll Surg Engl 71(1):14–19
65. Banu T, Hannan MJ, Aziz MA, Hoque M, Laila K
(2006) Rectovestibular fistula with vaginal malforma-
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66. Santulli TV, Kiesewetter WB, Bill AH Jr (1970)
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 Basic Anatomy and Physiology
of Bowel Control
The reader may be surprised for not finding in
this chapter many of the traditional terms histori-
cally used to refer to the different portions of the
sphincter mechanism of the human being. We use
a different terminology that we believe is realis-
tic, useful, and with important practical and tech-
nical implications for the practicing surgeon.
This is a result of our observations of the differ-
ent anatomic variants, found in more than 2,032
surgical explorations of the pelvis and the ano-
rectum of patients suffering from anorectal mal-
formations, as well as many others operated to
resect tumors and to repair pelvic organs (ure-
thra, vagina, and rectum).
In the early times, when we performed the first
posterior sagittal approaches to repair anorectal
malformations, influenced by the traditional con-
cepts expressed in the available textbooks on the
subject [1, 2], we were looking for the “puborec-
talis sling,” the “pubococcygeal muscle,” the
“pubourethralis muscle,” the “iliococcygeal mus-
cle,” the “superficial portion of the external
sphincter,” the “deep portion of the external
sphincter,” and the “internal sphincter,” and we
were rather frustrated for not seeing what was
described in the textbooks. Or at least, what we
were seeing was very different to what was
described.
Electronic supplementary material Supplementary
material is available in the online version of this chapter at
10.1007/978-3-319-14989-9_2.
A. Peña, A. Bischoff, Surgical Treatment of Colorectal Problems in Children,
DOI 10.1007/978-3-319-14989-9_2, © Springer International Publishing Switzerland 2015
2
In addition, through the years we found that
what we observed as part of the sphincter mecha-
nism in one patient was never the same as the one
that we saw in another one. After a large experi-
ence with the surgical treatment of anorectal mal-
formations, we are now certain to believe that we
are dealing with a spectrum of anatomic
variations.
We are aware of the fact that human beings
(and surgeons are not exceptions) prefer to deal
with artificial, man-made, classifications to refer
and to discuss biological phenomenon. Yet, we
like to say that Mother Nature does not like our
classifications and continues producing humans,
animals, and biological specimens following a
spectrum type of pattern. We recognize that it is
not easy from the clinical point of view to talk
about spectrums, but we could not ignore reality.
Figure 2.1 shows what we think is the best
photograph ever taken of the pelvic anatomy of a
male cadaver. The authors managed to show a
perfect sagittal section. We feel admiration and
respect for such achievement. This magnificent
photograph was reproduced with permission
from the excellent “Colon Atlas of Human
Anatomy” by R.M.H. McMinn Emeritus
Professor of Anatomy, Royal College of
Surgeons of England and University of London
and R.T. Hutchings, photographer, formerly
Chief Medical Laboratory Scientific Officer,
Royal College of Surgeons of England. Year
Book Medical Publishers, Inc., Chicago 1977,
page 248.
17
18
Fig. 2.1 Photograph of a sagittal section of a human
cadaver. 1 Rectus abdominis; 2 Extraperitoneal fat;
3 Sigmoid colon; 4 Promontory of sacrum; 5 Rectum;
6 Coccyx; 7 Anococcygeal body; 8 External anal sphinc-
ter; 9 Anal canal with anal columns of mucous membrane;
10 Perineal body; 11 Ductus deferens; 12 Epididymis;
13 Testicle; 14 Spongy part of urethra and corpus
spongiosum; 15 Corpus cavernosum; 16 Bulbospongiosus;
17 Perineal membrane; 18 Sphincter urethrae;
19 Membranous part of urethra; 20 Pubic symphysis;
21 Prostate gland; 22 Prostatic part of urethra; 23 Seminal
colliculus; 24 Bristle in ejaculatory duct; 25 Internal ure-
thral orifice; 26 Bladder; 27 Bristle passing up into right
ureteral orifice; 28 Rectovesical pouch
Figure 2.2 shows an MR image of the sagittal
section of the pelvis of a normal child. It is really
remarkable what modern imaging technology has
achieved. We invite the readers to look in a very
detailed way these two pictures (Figs. 2.1 and
2.2). The same muscle structures shown in the
cadaver are present in the MRI picture. A non-
biased, objective view of these two figures does
not allow us to identify separately the puborecta-
lis, ischiococcygeal, pubourethralis, and iliococ-
cygeal muscles and deep external sphincter,
superficial external sphincter, and internal
sphincter.
Rather, one can see a muscle structure that
comes from the anterior aspect of the lowest
2 Basic Anatomy and Physiology of Bowel Control
part of the sacrum and coccyx and runs all the
way down to the skin attached to the posterior
rectal wall. It actually runs in continuum and
one cannot see any hint of separation of differ-
ent structures. In our observations of normal
human beings (not only in cases of anorectal
malformations) operated posterior sagittally for
other reasons (tumors, trauma), and using an
electrical stimulator, we have always been able
to identify this strong funnel-like muscle struc-
ture. If one touches that muscle structure in the
upper portion, one can elicit a contraction that
pushes the rectum forward as described for the
levator muscle. If one touches the lowest part of
that muscle, one can see an elevation of the
anus, and perhaps that is why originally this
structure was called levator muscle. That por-
tion of this sphincter mechanism made mainly
of vertical fibers running parallel to the rectum
we call it “muscle complex,” to differentiate it
from the upper portion (levator) that is made out
of horizontal fibers that compress the rectum
from behind. However, these different portions
of the sphincter mechanism do not contract sep-
arately; in real life, they contract in a massive
unified way (Animations 2.1, 2.2, 2.3, 2.4, and
2.5). The muscle fibers running longitudinally
(parallel to the rectum) elevate the anus when
they contract, whereas those fibers that surround
the rectum are the ones that produce the com-
pression of the rectum posteriorly. Perhaps the
point of maximal contraction of this funnel-like
muscle structure is what has been considered
the puborectalis sling, but we must emphasize
that there is no real anatomic separation of these
structures. Near the skin, the funnel-like muscle
joins other types of muscle that run superficial
under the skin and divide into two portions, one
on each side of the anus, and that is why we call
those “parasagittal fibers.” The contraction of
these muscles may produce the impression of
closing the anus in a circular fashion; yet, actu-
ally these are parasagittal fibers that join one
side with the other, posterior and anterior to the
anal opening. The parasagittal fibers run actu-
ally perpendicular to the muscle complex. We
arbitrarily use the term “levator” to refer to the
upper portion of the funnel-like muscle mecha-
2 Basic Anatomy and Physiology of Bowel Control
a b
Fig. 2.2 MRI of a sagittal section of the normal pelvis. (a) Relaxed sphincter mechanism. (b) Contracted sphincter
mechanism
nism and use the term “muscle complex” to its
lower portion. The point where the muscle com-
plex and parasagittal fibers cross represents the
limits of the sphincter.
When the anatomy of the anorectal sphincters
is presented in this rather simplistic way, it is
easy to teach and to learn.
Traditional anatomy concepts have been
repeated from one generation to another, full of
details or concepts without clinical relevance.
The medical students are frequently obligated to
learn some of those concepts.
We have been impressed by publications
related with the anatomy of the sphincters; the
authors frequently show real photographs of the
anatomy, yet they over-impose arrows to show
inexistent, imaginary structures [2–5]. In other
words, photographs show the real anatomy,
whereas the diagrams or the arrows show what
the authors wanted to see.
More recently, advances in the technology of
imaging show images (Fig. 2.2) of the real anat-
omy; however, again the authors fabricate other-
19
wise unnecessary diagrams showing their
preconceptions and biases [6–13].
It is even more impressive to see how rather
bizarre anatomic concepts, like the idea of a “tri-
ple loop” without any evidence to support its
existence, are accepted, published, and repeated
between surgeons [14, 15].
In a medical community where everybody
seem to be able to see clearly structures such as
the puborectalis muscle and all the other portions
of the sphincter mechanism, very few dare to
express skepticism and disagree [16, 17]. During
our literature review, we found an excellent hon-
est paper written by Dr. Arthur F. Dalley II, PhD,
[17]. After a very thoughtful discussion, he con-
cludes saying: “I recommend that the three-part
external anal sphincter be removed from gross
anatomy texts, dissectors and atlases and be rel-
egated to the junkyard of anatomic trivia where it
may languish for the sake of the historical anato-
mist or the rare individual who spends time carv-
ing out the most meticulous of dissections.” This
is a paper that all colorectal surgeons must read.
20 2 Basic Anatomy and Physiology of Bowel Control

2.1 Internal Sphincter 2.2 General Anatomic Principles

in Anorectal Malformations
This elusive structure has been described as a
thickening of the circular layer of smooth muscle Figures 2.3, 2.4, 2.5, and 2.6 show the anatomic
of the bowel in the area of the anorectum. The variations that form part of the spectrum seen in
literature related to this structure, in general, has cases of anorectal malformations. Animation 2.1
the following characteristics: shows the sagittal view of the anatomy of the pel-
• There are no good quality photographs show-
ing the sphincter.
• There is no precise description of its size and
limits at different ages.
• Most papers discuss the “internal sphincter”
based on manometric findings.
• Some authors believe that the “internal sphinc-
ter” is very important for bowel control [18, 19].
• Others believe that its contribution for bowel
control is not significant [20].
• Many authors believe that the lack of relax-
ation of this structure is responsible for many
patients suffering from constipation and
megarectosigmoid (see Chap. 25).
• Our direct observations of the entire poste-
rior anorectal wall in normal individuals did
not allow us to see the thickening of the
smooth muscle that presents the “internal
sphincter.” Fig. 2.3 Diagram showing the most common anatomic
• As a consequence of all the importance that sphincter pattern seen in patients with perineal fistula
many doctors gave to the “internal sphincter,”
pediatric surgeons have been debating about
the possible existence of an “internal sphinc-
ter” in cases of anorectal malformations,
located at the most distal part of the bowel, the
portion attached to the urogenital tract (fis-
tula). Some surgeons believe that it is very
important to preserve the most distal portion
of the bowel in order to guarantee bowel con-
trol [21–23], whereas others believe that it
must be resected to avoid constipation [24].
Our results in terms of bowel control show
that fecal continence and constipation are
unrelated to the preservation or resection of
that portion of the bowel (see Chap. 15).
All these descriptions of our literature find-
ings related with the “internal sphincter,” plus our
concepts discussed in Chap. 25, explain our skep-
ticism related with the existence, function, and Fig. 2.4 Diagram showing the most common anatomic
relevance of this structure. sphincter pattern seen in patients with bulbar fistula
2.2 General Anatomic Principles in Anorectal Malformations
Fig. 2.5 Diagram showing the most common anatomic
sphincter pattern seen in patients with prostatic fistula
Fig. 2.6 Diagram showing the most common anatomic
sphincter pattern seen in patients with bladder neck
fistula
vis of a normal human being. Figure 2.3 shows
the most benign of all defects which is the peri-
neal fistula. The sphincter mechanism in these
types of cases is almost normal. The rectum, on
the other hand, deviates in its lowest portion to
21
open in the perineal body, anterior to the center of
the sphincter. The rectum is dilated.
Figure 2.4 shows the anatomy of a patient
born with a rectourethral bulbar fistula. Most of
these patients have a sphincter mechanism rea-
sonably good, perhaps not as strong and good as
the sphincter of a normal person or a patient with
a perineal fistula. The rectum connects to the
lowest portion of the posterior urethra which we
call bulbar urethra.
Figure 2.5 shows the anatomy on a patient
with a rectoprostatic fistula. One can see that the
sphincter mechanism is much more primitive and
weak. In addition, the distance between the
sacrum and the pubis is significantly shorter. The
available space for a pull-through is getting
smaller, the sphincter mechanism weaker, and
obviously the prognosis is not as good as in the
previous defects.
Figure 2.6 shows the anatomy of a patient
with a recto-bladder neck fistula. The rectum
opens at the bladder neck, the sphincter mecha-
nism is very tenuous, sometimes almost nonexis-
tent, and the distance between the sacrum and the
pubis is very short. Sometimes it makes it almost
impossible to pull the rectum down. As one can
see in this series of diagrams, in the case of the
rectourethral bulbar fistula, once we separate the
rectum from the urethra, it is conceivable that
the rectum can be placed within the limits of the
sphincter with minimal mobilization and will be
completely covered by the sphincter mechanism.
Whereas in Fig. 2.5, sometimes we find a rectum
that does not fit into the tenuous, delicate sphinc-
ter mechanism, and one has the feeling that the
reconstruction was not ideal. And finally, in some
of the recto-bladder neck fistulas, it becomes
very obvious that the sphincter is very weak and
that the patient most likely will have fecal incon-
tinence. In fact, as we will discuss in our results,
patients with recto-perineal fistulas have a 100 %
chance of bowel control provided they have a
good operation. Patients with bulbar fistula have
85 % chances, prostatic fistula 60 % chances, and
bladder neck fistula only 20 % chances. Similarly,
characteristically, the sacrum usually is more and
more primitive, as we go into higher and higher
22
locations of the rectum, as well as the
characteristics of the sphincter and the space
available between the sacrum and pubis. The
chance of suffering from tethered cord also
increases in higher malformations. The exception
is represented by some patients with perineal fis-
tulas. This particular group of patients has more
tendencies to suffer from presacral masses and
tethered cord. The anatomic differences shown in
Figs. 2.3, 2.4, 2.5, and 2.6 and Animations 2.2,
2.3, 2.4, 2.5, and 2.6 are seen in real life. However,
as it is well known by all surgeons, in medicine
and surgery, there are no “nevers” and there are
no “always.” In other words, it is possible to see,
although very unusual, a perineal fistula with
very poor sphincter mechanism and also to see a
recto-bladder neck fistula with a rather good-
looking sphincter mechanism, but those are
exceptions. Most of the time, as the rectum is
located higher connecting to the urogenital tract,
the sacrum tends to be shorter, the distance
between the sacrum and pubis decreases, and the
characteristics of the sphincter become more and
more rudimentary and weak.
2.3 Nerves
We surgeons frequently refer to the “lack of
nerves” when we deal with patients with anorec-
tal malformations with bad functional prognoses.
Yet, we actually never see the nerves when oper-
ating on these patients. There are no precise sci-
entific studies that give evidence of the presence
or absence, as well as characteristics and precise
location, of the pelvic nerves in cases of anorectal
malformations. We work, always assuming, that
the higher the location of the rectum and its con-
nection to the urogenital tract, as well as the more
deficient sacrum is, the more deficient the nerves
are. In addition, the fact that we have to mobilize
the rectum from higher distances most likely
means that we have to sacrifice more nerves. We
also assume that in a patient with absent sacrum,
all the nerves that normally come out of the seg-
ments of the sacrum and innervate the pelvic
organs are absent, or deficient, and that may
explain why patients with absent sacrum have
2 Basic Anatomy and Physiology of Bowel Control
zero possibility of having bowel and urinary con-
trol. Patients with anorectal malformations are
represented by a spectrum in terms of sacrum
deficiency that goes from patients with normal
sacrum to patients with completely absent
sacrum.
We know that under normal circumstances,
the nerves that innervate the bladder neck urinary
tract and corpora come from the orifices of the
sacrum and run lateral to the rectum in order to
reach the corpora, the bladder neck, and the rec-
tum itself. Therefore, from the early times, Dr.
Douglas Stephens [25] recommended (and is still
valid) to try to remain exactly in the midline as
much as possible during our surgical explora-
tions. Once the surgeon reaches the rectum, all
the dissection of the rectum must be performed,
staying as close as possible to the rectal wall to
avoid the damage of nerves that supposedly run
lateral to the rectum. In other words, the further
away from the rectum, the more chances to injure
nerves.
Concerning the anatomy of the pelvic auto-
nomic nerves, the reader is referred to anatomy
books and an excellent paper [26]. From the read-
ing of that material, we reinforce our belief that it
is essential to remain in the midline while
approaching the pelvis and to dissect the rectum
remaining as close as possible to the bowel wall,
in order to minimize the possibilities to injure
autonomic nerves and avoid neurogenic bladder
and impotence.
We have evidence from patients that had pre-
vious failed attempted repairs and that were born
with a “good” malformation and yet they suf-
fered from fecal and sometimes urinary inconti-
nence. Reading the operative reports of those
patients, one finds that the surgeon actually got
lost and certainly went out of the midline, which
may explain the nerve damage.
2.4 Blood Supply
Unfortunately, there are no scientific detailed
anatomic studies of the blood supply of the pelvic
organs of patients with anorectal malformations.
Again, what we have learned from our surgical
2.5 Basic Physiology Principles of Bowel Control
explorations is that it seems like the rectum has
an excellent intramural blood supply, as evi-
denced by the fact that in every case of anorectal
malformation that we operate on, we separate the
rectum from the urogenital tract, and then we
have to mobilize the rectum enough to reach the
perineum. In order to do that, we performed a cir-
cumferential dissection, dividing all the extrinsic
vessels and bands that hold the rectum up in the
pelvis. In other words, we are basically devascu-
larizing the rectum. We sacrifice its entire extrin-
sic blood supply. Yet, provided the wall of the
rectum remains intact and the inferior mesenteric
vessels are preserved, the intramural blood sup-
ply of the proximal part provides enough to
maintain alive the distal part of the rectum. This
finding should not be extrapolated to other parts
of the colon. The blood supply of the colon in
patients with anorectal malformations is basi-
cally the same as in normal individuals, except in
patients with cloacal exstrophies and patients
with a malformation called “rectal pouch” in
which the entire colon is represented by a single
saccular piece of colon with a very abnormal
bizarre type of blood supply (picture).
2.5 Basic Physiology Principles
of Bowel Control
We take care of many babies born with severe
anatomic deficiencies; we can certainly repair
their anatomy, but we cannot restore their normal
function; however, we try to help them to have a
normal social life, with the implementation of
our “bowel management program.” Unfortunately,
there is another large group of patients who suffer
from fecal incontinence as a consequence of a
technically deficient operation. That is obviously
something highly regrettable, but most important
is the fact that it is preventable. For that, it is
imperative for the surgeon to know a few basic
but extremely important anatomic and physio-
logic principles.
In order to have bowel control, it is necessary
to have three very important elements:
A. Sensation
B. Sphincter
23
C. Rectosigmoid motility and reservoir function
A. Sensation – this is the first indispensable ele-
ment for bowel control. The anal canal is per-
haps the most sensitive part of the human
body. There, we are capable of discriminating
gas, from liquid and from solid fecal matter
[27]. The anal canal remains collapsed by the
effect of the muscle tone of the sphincter
mechanism that surrounds it. When the fecal
matter (liquid, solid, or gas) reaches the anal
canal due to active rectal peristalsis, we per-
ceive it and, depending on the surrounding
circumstances, decide to use our voluntary
sphincter mechanism, to occlude the lumen
of the anal canal and avoid a bowel move-
ment, until the circumstances are appropriate
to have a bowel movement.
Above the anal canal, in the rectum, we do
not have the exquisite sensation described for
the anal canal. However, a distention of the
rectum with a balloon causes a vague sensa-
tion of fullness that is known as propriocep-
tion [28].
The implications of these facts for us sur-
geons are obvious. We must try to preserve
intact the anal canal during our operations in
patients with normal anal canal, such as
patients suffering from Hirschsprung’s dis-
ease, severe constipation, inflammatory
bowel disease, and familial polyposis.
The overwhelming majority of patients
with anorectal malformations are born with-
out an anal canal, except for a rare malforma-
tion called rectal atresia. Patients with
perineal fistula have a rather primitive anal
canal. This means that patients with anorectal
malformations, under the best circumstances,
do not have “perfect” bowel control. Many
patients born with a malformation with good
functional prognosis behave like normal chil-
dren, yet when they have a severe episode of
diarrhea, it becomes evident that they are less
than perfect in terms of bowel control.
B. Sphincter – the voluntary sphincter mecha-
nism maintains a certain tone constantly.
However, there is a common misconception
consisting on believing that a sudden relax-
ation of the sphincter will produce escape of
24
feces. That is simply not true. In fact, a human
being can only have a bowel movement when
the rectosigmoid has a peristaltic wave that
pushes the stool out. Human beings actually
use the voluntary sphincter occasionally, to
prevent the passing of gas or fecal matter in
inadequate circumstances.
C. Rectosigmoid motility and reservoir func-
tion – after many years of working with
patients suffering from bowel control prob-
lems, we came to realize that rectosigmoid
motility and its reservoir function are the
most important element for bowel control.
The rectosigmoid remains relaxed most of the
time, acting as a reservoir of fecal matter.
That is an extremely important function,
since it allows human beings to have a social
life and only use the toilet every 24–48 h.
The implications of this, for us surgeons, are
also very obvious. The removal of the rectosig-
moid and connection of a more proximal portion
of the colon, to the anal canal, means elimination
of the reservoir function which results in an
almost constant attempt of the colon to empty. If
the anal canal is maintained intact, the resection
of the rectosigmoid will result in many bowel
movements during the day and a constant effort
to avoid them. We all have seen how a colostomy
works. It passes stool almost constantly. An
ascending colostomy will be passing more often
liquid stool, and as we move distally, the stool
becomes more solid and the peristalsis less active.
That means that connecting a piece of colon
directly to the anal canal (without reservoir) will
produce very frequent bowel movements, and it
will require a well-preserved anal canal to main-
tain continence.
We can easily imagine what happens when we
remove the natural reservoir (rectosigmoid) and
damage or resect the anal canal. The result will
be permanent fecal incontinence.
Another scenario is the case of an absent anal
canal and sphincter; we have seen that in cases of
trauma. The motility of the rectosigmoid and its
reservoir function is preserved, and therefore, it
is possible that a particular patient behaves like if
he/she was fecally continent, provided he/she
2 Basic Anatomy and Physiology of Bowel Control
does not have episodes of diarrhea or multiple,
irregular, unpredictable bowel movements.
Using our imagination, we conceive the pos-
sibility of manipulating the rectosigmoid motil-
ity, using pharmacologic agents, in order to
paralyze the rectosigmoid when required and to
provoke a peristaltic wave to empty the rectosig-
moid when the surrounding circumstances are
appropriate. In fact, we consider that kind of
treatment more likely to be successful rather than
trying to reconstruct the sphincters or use artifi-
cial sphincters, without taking into consideration
the two more important elements that are sensa-
tion and motility.
References
1. Stephens D, Smith D (1972) Ano-rectal malformation
in children. Year Book Medical Publisher, Inc,
Chicago, pp 14–32
2. Oh C, Kark AE (1972) Anatomy of the external anal
sphincter. Br J Surg 59(9):717–723
3. Lawson JO (1974) Pelvic anatomy. I. Pelvic floor
muscles. Ann R Coll Surg Engl 54(5):244–252
4. Fucini C, Elbetti C, Messerini L (1999) Anatomic
plane of separation between external anal sphincter
and puborectalis muscle: clinical implications. Dis
Colon Rectum 42(3):374–379
5. Gil-Vernet JM, Torán N, Sanchís LF, Marhuenda C
(1988) Consideraciones a la anatomía del esfínter
externo anal en la atresia anorrectal alta. [Anatomy of
the external anal sphincter in high anorectal atresia].
Cir Pediatr 1(2):62–65
6. Ikawa H, Yokoyama J, Sanbonmatsu T, Hagane K,
Endo M, Katsumata K, Kohda E (1985) The use of
computerized tomography to evaluate anorectal
anomalies. J Pediatr Surg 20(6):640–644
7. Aronson MP, Lee RA, Berquist TH (1990) Anatomy
of anal sphincters and related structures in continent
women studied with magnetic resonance imaging.
Obstet Gynecol 76(5 Pt 1):846–851
8. Hussain SM, Stoker J, Zwamborn AW, Den Hollander
JC, Kuiper JW, Entius CA, Laméris JS (1996)
Endoanal MRI of the anal sphincter complex: correla-
tion with cross-sectional anatomy and histology. J
Anat 189(Pt 3):677–682
9. Guo M, Li D (2007) Pelvic floor images: anatomy of
the levator ani muscle. Dis Colon Rectum
50(10):1647–1655
10. Li D, Guo M (2007) Morphology of the levator ani
muscle. Dis Colon Rectum 50(11):1831–1839
11. Tang ST, Cao GQ, Mao YZ, Wang Y, Li SW, Yang Y,
Tong QS (2009) Clinical value of pelvic 3-dimensional
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magnetic resonance image reconstruction in anorectal
malformations. J Pediatr Surg 44(12):2369–2374.
doi:10.1016/j.jpedsurg.2009.07.074
12. Guo M, Gao C, Li D, Guo W, Shafik AA, Zbar AP,
Pescatori M (2010) MRI anatomy of the anal region.
Dis Colon Rectum 53(11):1542–1548. doi:10.1007/
DCR.0b013e3181f05256
13. Watanabe Y, Takasu H, Sumida W, Mori K (2013)
Wide variation in anal sphincter muscles in cases of
high- and intermediate-type male anorectal malfor-
mation. Pediatr Surg Int 29(4):369–373. doi:10.1007/
s00383-012-3250-z
14. Shafik A (1980) A new concept of the anatomy of the
anal sphincter mechanism and the physiology of
defecation. X. Anorectal sinus and band: anatomic
nature and surgical significance. Dis Colon Rectum
23(3):170–179
15. Zbar AP, Guo M, Pescatori M (2008) Anorectal mor-
phology and function: analysis of the Shafik legacy.
Tech Coloproctol 12(3):191–200. doi:10.1007/
s10151-008-0417-7
16. Konerding MA, Dzemali O, Gaumann A, Malkusch
W, Eckardt VF (1999) Correlation of endoanal sonog-
raphy with cross-sectional anatomy of the anal
sphincter. Gastrointest Endosc 50(6):804–810
17. Dalley AF 2nd (1987) The riddle of the sphincters.
The morphophysiology of the anorectal mechanism
reviewed. Am Surg 53(5):298–306
18. Frenckner B, Euler CV (1975) Influence of pudendal
block on the function of the anal sphincters. Gut
16(6):482–489
19. Penninckx F, Lestar B, Kerremans R (1992) The inter-
nal anal sphincter: mechanisms of control and its role
25
in maintaining anal continence. Baillieres Clin
Gastroenterol 6(1):193–214
20. Varma KK, Stephens D (1972) Neuromuscular
reflexes of rectal continence. Aust N Z J Surg
41(3):263–272
21. Holschneider AM, Ure BM, Pfrommer W, Meier-
Ruge W (1996) Innervation patterns of the rectal
pouch and fistula in anorectal malformations: a pre-
liminary report. J Pediatr Surg 31(3):357–362
22. Holschneider AM, Pfrommer W, Gerresheim B
(1994) Results in the treatment of anorectal malfor-
mations with special regard to the histology of the
rectal pouch. Eur J Pediatr Surg 4(5):303–309
23. Lambrecht W, Lierse W (1987) The internal sphincter in
anorectal malformations: morphologic investigations in
neonatal pigs. J Pediatr Surg 22(12):1160–1168
24. Rintala R, Lindahl H, Marttinen E, Sariola H (1993)
Constipation is a major functional complication after
internal sphincter-saving posterior sagittal anorecto-
plasty for high and intermediate anorectal malforma-
tions. J Pediatr Surg 28(8):1054–1058
25. Stephens D, Smith D (1972) Ano-rectal malformation
in children. Year Book Medical Publisher, Inc,
Chicago, pp. 212–273
26. Pearl RK, Monsen H, Abcarian H (1986) Surgical
anatomy of the pelvic autonomic nerves. A practical
approach. Am Surg 52(5):236–237
27. Duthie HL, Gairns FW (1960) Sensory nerve-endings
and sensation in the anal region of man. Br J Surg 47:
585–595
28. Turell R, Krakauer JS, De Maynard AL (1953)
Colonic and anorectal function and disease. Surg
Gynecol Obstet 96(4):313–339; contd
 Prenatal Diagnosis
Significant advances had been achieved in the
field of prenatal diagnosis in general, as a conse-
quence of amazing developments in the imaging
technology. The use of ultrasound to visualize the
fetus in utero already meant a great step in the
diagnosis of gross malformations in utero [1–8].
Subsequently, the MRI amplified the possibilities
of making much more accurate diagnosis of mul-
tiple congenital defects [9–11].
At the present time, we cannot claim that we
can make accurate prenatal diagnoses of anorec-
tal malformations. More specifically, we cannot
determine the precise type of defect that the fetus
has. Yet, with the current technology, we can
make gross diagnoses that allow us to make
important decisions and formulate meaningful
recommendations [12–19].
The benefits of the prenatal diagnosis in ano-
rectal malformations, as well as in all defects,
include the possibility of giving the parents a
fairly accurate idea of the functional prognosis
of the future baby, which will influence his/her
quality of life. This, beyond ethical and moral
issues, will help the parents to make important
decisions related to the possibility of terminat-
ing the pregnancy. In addition, depending on the
specific type of defect, as well as its complexity,
we can advise the parents as to the best possible
place where the baby should be delivered, in
order to receive optimal, comprehensive, and
high-quality care. In some of the most serious
anorectal and urogenital malformations, the
therapeutic decisions taken during the first few
A. Peña, A. Bischoff, Surgical Treatment of Colorectal Problems in Children,
DOI 10.1007/978-3-319-14989-9_3, © Springer International Publishing Switzerland 2015
3
hours or days of life are crucial for the future of
the baby. Some mistakes that occur in the early
management of these babies may have impor-
tant repercussions for the future quality of life
of the baby. That is the reason why in cases of
complex defects that affect the colorectal area
as well as the urogenital tract, we must advise to
deliver the baby in a specialized center, where a
multidisciplinary team of experts, with the nec-
essary experience in that field, takes care of
him/her.
3.1 Male Fetuses
A frequent in utero finding in babies with anorec-
tal malformations is the presence of a dilated
bowel (Fig. 3.1). This is a nonspecific finding that
can be present in other conditions such as
Hirschsprung’s disease.
The presence of intraluminal calcifications
makes the dilated bowel sign more significant,
since we know that the mixing of urine with
meconium frequently produces calcifications
(Fig. 3.2). We must keep in mind that over 80 %
of the patients with anorectal malformations have
a rectal urinary fistula, and meconium may go
into the urinary tract as well as urine into the
rectum.
There are several signs that can be clearly seen
by ultrasound and particularly with an MRI study
that will make the diagnosis of anorectal malfor-
mation more likely. These signs include:
27
28 3 Prenatal Diagnosis

Fig. 3.1 In utero MRI showing a dilated bowel in a fetus Fig. 3.3 In utero image of an abnormal sacrum. Arrow
with anorectal malformation. Arrow shows the dilated shows a very short sacrum
bowel

3.1.1 Abnormal Sacrum (Fig. 3.3)

Thirty percent of the patients with anorectal mal-

formations have an abnormal sacrum (see images
in Chap. 6). These abnormalities may include a
simply short sacrum, absent vertebra, as well as
hemivertebra. Some patients may have a hemisa-
crum which is associated with a presacral mass.

3.1.2 Tethered Cord

This spinal cord defect is present in about 25 %

of patients with anorectal malformations (see
Chap. 23). A prenatal diagnosis is feasible as
demonstrated in this figure (Fig. 3.4). This defect,
as well as the abnormal sacrum, has a definite
influence in the future prognosis for the urinary
function and bowel function.

3.1.3 Absent Kidney (Fig. 3.5)

It is the most common urologic malformation

associated with anorectal defects (see Chap. 23).
Fig. 3.2 Intraluminal calcifications. In utero image. This
is a consequence of the mixing of urine with meconium. It is more commonly present in serious anorectal
Arrow shows calcifications defects. The prenatal diagnosis is very reliable.
3.1 Male Fetuses
Fig. 3.4 Tethered cord diagnosed in utero. Arrow shows
the low lying location of the conus
Fig. 3.5 Absent kidney in a fetus with anorectal
malformation
29
Fig. 3.6 Fetal hydronephrosis. Arrow shows the
hydronephrosis
3.1.4 Vertebral Anomalies
A significant number of patients with anorectal
malformations have an abnormal vertebra,
mainly hemivertebra. This defect can be diag-
nosed in utero.
3.1.5 Hydronephrosis (Fig. 3.6)
This is the second most common anatomic abnor-
mality of the urinary tract seen in patients with
anorectal malformations (see Chap. 23). Bilateral
hydronephrosis denotes that both kidneys had
been suffering in utero; therefore, we can antici-
pate a significant degree of kidney damage. This
is extremely important because the patients will
require special care to protect those kidneys
already significantly affected.
30 3 Prenatal Diagnosis

3.2 Female Fetuses a

3.2.1 Dilated Bowel
and Intraluminal
Calcifications

The presence of a dilated bowel is also seen in

female patients. Intraluminal calcifications, on
the other hand, are seen less often, since the
majority of female cases do not have a communi-
cation between the rectum and urinary tract.

3.2.2 Pelvic Cystic Mass

The presence of a cystic pelvic mass unilateral or

bilateral, located behind the bladder, is highly sug-
gestive of a hydrocolpos in a female fetus suffer-
ing from a cloaca (Fig. 3.7). The importance of the
diagnosis of a hydrocolpos cannot be overempha-
sized. A tense hydrocolpos compresses the blad-
der and more specifically the trigone. This may
result in an extrinsic ureterovesical obstruction
that may provoke megaureter and hydronephrosis.
At birth, these babies need a permanent drainage
of the hydrocolpos to avoid kidney damage. a
In a large number of patients with cloacas seen
at our center, we requested the mother to allow us
to see the ultrasound studies done when the baby
was in the uterus. Many of the hydrocolpos that
these babies were born with were already present
in utero, yet the radiologists who saw those stud-
ies made a wrong diagnosis such as “double blad-
der,” “ureterocele,” and “bladder diverticulum.”
This indicates that in general, the index of suspi-
cion for the diagnosis of hydrocolpos is very low
between radiologists; they detected the abnormal
image but interpreted it in the wrong way.
The presence of hydronephrosis, abnormal Fig. 3.7 Bilateral hydrocolpos, diagnosed in utero. (a)
sacrum, abnormal vertebra, absent kidney, and Dilated bowel entering in the middle of two hemivaginas
tethered cord in female fetuses, as well as in with fluid (hydrocolpos) (b) bilateral hydrocolpos. a asci-
tes, b bladder, v vagina, h hydronephrosis
males, gives support to the possibility of the
patient having an anorectal malformation.
Chap. 17). These patients have an omphalocele, a
prominent prolapsed small bowel known as “ele-
3.2.3 Cloacal Exstrophy phant trunk,” located between two hemibladders,
separated pubic bones, and are frequently associ-
This malformation is the most serious one in the ated with a meningocele and abnormalities in the
spectrum of anorectal and urogenital defects (see lower limbs. In addition, they have an exstrophic
References
Fig. 3.8 Omphalocele, in utero diagnosis. O omphalo-
cele, M meningocele
Fig. 3.9 “Elephant trunk” (intussuscepted prolapsed
ileum) emerging in between two hemibladders. Arrow
shows uterine wall
bladder which is described or diagnosed prena-
tally as “absent bladder.”
The omphalocele (Fig. 3.8) can be easily seen
in utero. The elephant trunk can also be seen
(Fig. 3.9), as well as the separated pubic bones
and the meningocele (Fig. 3.8).
The prenatal diagnosis is particularly impor-
tant in this extremely serious malformation con-
sidering the very poor quality of life that these
patients will have during their future life, particu-
larly when they have a severe spinal deficiency
that may translate into incapacity to walk, for
life. We believe that it is extremely important for
pediatric surgeons, pediatric urologists, as well
31
as neonatologists to give the parents the most
accurate possible description of the functional
limitations that these patients will suffer from, in
terms of bowel function, urinary function, sexual
function, and other limitations that these patients
will have, so the parents can make a reasonable
decision concerning the possibilities of interrupt-
ing the pregnancy, wait for full term, mode of
delivery, and the place where the baby should be
delivered.
References
1. Baronciani D, Scaglia C, Corchia C, Torcetta F,
Mastroiacovo P (1995) Ultrasonography in pregnancy
and fetal abnormalities: screening or diagnostic test?
IPIMC 1986–1990 register data. Indagine Policentrica
Italiana sulle Malformazioni Congenite. Prenat Diagn
5(12):1101–1108
2. Brantberg A, Blaas HG, Haugen SE, Isaksen CV, Eik-
Nes SH (2006) Imperforate anus: a relatively com-
mon anomaly rarely diagnosed prenatally. Ultrasound
Obstet Gynecol 28(7):904–910. doi:10.1002/
uog.3862
3. Shono T, Taguchi T, Suita S, Nakanami N, Nakano
H (2007) Prenatal ultrasonographic and magnetic
resonance imaging findings of congenital cloa-
cal anomalies associated with meconium peritoni-
tis. J Pediatr Surg 42(4):681–684. doi:10.1016/j.
jpedsurg.2006.12.060
4. Shalev E, Feldman E, Weiner E, Zuckerman H (1986)
Prenatal sonographic appearance of persistent cloaca.
Acta Obstet Gynecol Scand 65(5):517–518
5. Petrikovsky BM, Walzak MP Jr, D’Addario PF (1988)
Fetal cloacal anomalies: prenatal sonographic find-
ings and differential diagnosis. Obstet Gynecol 72(3
Pt 2):464–469
6. Lande IM, Hamilton EF (1986) The antenatal sono-
graphic visualization of cloacal dysgenesis. J
Ultrasound Med 5(5):275–278
7. Odibo AO, Turner GW, Borgida AF, Rodis JF,
Campbell WA (1997) Late prenatal ultrasound fea-
tures of hydrometrocolpos secondary to cloacal
anomaly: case reports and review of the literature.
Ultrasound Obstet Gynecol 9(6):419–421.
doi:10.1046/j.1469-0705.1997.09060419.x
8. Ohno Y, Koyama N, Tsuda M, Arii Y (2000) Antenatal
ultrasonographic appearance of a cloacal anomaly.
Obstet Gynecol 95(6 Pt 2):1013–1015
9. Hung YH, Tsai CC, Ou CY, Cheng BH, Yu PC, Hsu
TY (2008) Late prenatal diagnosis of hydrometrocol-
pos secondary to a cloacal anomaly by abdominal
ultrasonography with complementary magnetic reso-
nance imaging. Taiwan J Obstet Gynecol 47(1):79–
83. doi:10.1016/s1028-4559(08)60059-5
10. Hayashi S, Sago H, Kashima K, Kitano Y, Kuroda T,
Honna T, Natori M (2005) Prenatal diagnosis of fetal
32
hydrometrocolpos secondary to a cloacal anomaly by
magnetic resonance imaging. Ultrasound Obstet
Gynecol 26(5):577–579. doi:10.1002/uog.2584
11. Picone O, Laperelle J, Sonigo P, Levaillant JM,
Frydman R, Senat MV (2007) Fetal magnetic reso-
nance imaging in the antenatal diagnosis and man-
agement of hydrocolpos. Ultrasound Obstet Gynecol
30(1):105–109
12. Mori M, Matsubara K, Abe E, Matsubara Y,
Katayama T, Fujioka T, Ito M (2007) Prenatal diag-
nosis of persistent cloaca associated with VATER
(vertebral defects, anal atresia, tracheo-esophageal
fistula, and renal dysplasia). Tohoku J Exp Med
213(4):291–295
13. Baier SR, Tank ES, Watson PT (2001) Persistent
cloaca: prenatal diagnosis of hydrometro-
colpos. J Diagn Med Sonog 17(4):220–224.
doi:10.1177/87564790122250471
14. Suzumori N, Obayashi S, Hattori Y, Kaneko S, Suzuki
Y, Sugiura-Ogasawara M (2009) Prenatal diagnosis of
3 Prenatal Diagnosis
persistent cloaca. Congenit Anom (Kyoto) 49(3):116–
117. doi:10.1111/j.1741-4520.2009.00236.x
15. Warne S, Chitty LS, Wilcox DT (2002) Prenatal diag-
nosis of cloacal anomalies. BJU Int 89(1):78–81
16. Cilento BG Jr, Benacerraf BR, Mandell J (1994)
Prenatal diagnosis of cloacal malformation. Urology
43(3):386–388
17. Cacciaguerra S, Lo Presti L, Di Leo L, Grasso S,
Gangarossa S, Di Benedetto V, Di Benedetto A (1998)
Prenatal diagnosis of cloacal anomaly. Scand J Urol
Nephrol 32(1):77–80
18. Morikawa M, Yamada T, Cho K, Yamada H,
Minakami H (2006) Prenatal diagnosis and therapy of
persistent cloaca: a case report. Fetal Diagn Ther
21(4):343–347. doi:10.1159/000092463
19. Bischoff A, Calvo-Garcia MA, Baregamian N, Levitt
MA, Lim FY, Hall J, Pena A (2012) Prenatal counsel-
ing for cloaca and cloacal exstrophy-challenges faced
by pediatric surgeons. Pediatr Surg Int 28(8):781–
788. doi:10.1007/s00383-012-3133-3
 Neonatal Management
4.1 Introduction
In dealing with most congenital anomalies, errors
that occur during the first few hours or days of
life may have serious consequences and sequelae.
Early accurate diagnoses, as well as adequate
therapeutic decisions, require a high index of sus-
picion from pediatricians, neonatologists, pediat-
ric surgeons, pediatric urologists, nurses, and
other physicians and surgeons who take care of
neonatal babies with congenital anomalies.
Our eyes only see what our mind suspects.
It is hard to believe that in this twenty-first cen-
tury in developed countries, we still hear of
patients born with imperforate anus that were sent
home as “normal babies.” Subsequently, it was the
mother who made the diagnosis or the babies suf-
fer from bowel perforation and some of them die
[1–9]. We cannot overemphasize the importance
of the anorectal examination during the first physi-
cal examination of a neonate. In addition, it is also
true what is written in the very old textbooks of
pediatric surgery; it is not enough by looking at the
external appearance of the anus, but it is rather
necessary to introduce a little catheter or thermom-
eter to be sure that the anus is patent. There is one
specific type of defect in which the babies have a
normal-looking anus externally, a normal anal
canal, approximately 1–2 cm deep, and then an
atresia. That type of defect is the one that can only
be diagnosed by trying to pass a thermometer,
catheter, or an instrument through the anus. That
A. Peña, A. Bischoff, Surgical Treatment of Colorectal Problems in Children,
DOI 10.1007/978-3-319-14989-9_4, © Springer International Publishing Switzerland 2015
4
particular malformation only occurs in 1 % of all
patients born with anorectal malformations [10].
4.2 Most Common Scenario
As pediatric surgeons, we are called to see a baby
that is just born and has no anus. There are two
very important questions to be answered.
1. Does the baby have a serious associated defect
that may kill him/her within the next few
hours or days of life?
2. Does the baby need some sort of primary anal
repair or a colostomy?
These questions should be answered in the
same order that they are presented here. In other
words, the surgeon must refrain from jumping
into trying to find the answer of the second ques-
tion but rather concentrate during the first 24 h of
life in trying to answer the first question.
4.3 Answering the Two Most
Important Questions
At this point, the neonatologist and the surgeon
should remember that about 30 % of all babies with
anorectal malformation have some sort of cardio-
vascular condition [11]. However, only one third of
that 30 % have serious hemodynamic repercus-
sions that mandate to implement some sort of med-
ical or surgical treatment [11]. The most common
cardiac congenital anomalies seen in these cases
33
34 4 Neonatal Management

are patent ductus arteriosus, atrial septal defect,

ventricular septal defect, and tetralogy of Fallot,
and then other more serious conditions are more
rarely seen. The presence of cyanosis or respiratory
distress should alert the clinician to investigate for
the presence of these conditions. At the present
time, most neonatal centers have a pediatric cardi-
ologist and an echocardiogram machine which
allows ruling out most of these conditions.
Eight percent of babies with anorectal malfor-
mations are born with esophageal atresia [12].
Part of the physical examination of the neonate
includes the passing of a nasogastric no. 8 feed-
ing tube through the nostril to confirm that the
esophagus is patent. When the clinician feels
resistance in the passing of the tube about
8–10 cm from the nostril, it is possible that the
baby has an esophageal atresia (Fig. 4.1). In addi-
tion, babies with esophageal atresia cannot swal-
low their saliva; this represents a risk of aspiration.
The saliva accumulates in their mouth as foam. Fig. 4.1 Radiologic image of a baby with esophageal
Approximately 3 % of babies with anorectal atresia. Arrow shows the blind end of the esophagus
malformations suffer from duodenal atresia [12].
A simple abdominal film shows a classic “double
bubble” image (Fig. 4.2). The “double bubble”
represents the stomach and the duodenum full of
gas: in addition, there is a conspicuous absence
of gas in the rest of the abdomen. Approximately
50 % of all patients with anorectal malformations
are born with an associated urologic condition
[13]. The most serious of these are hydronephro-
sis, vesicoureteral reflux, absent kidney, and
megaureters. One specific group of patients has a
very high risk of having kidney damage; those
are patients born with a single kidney, hydrone-
phrosis, vesicoureteral reflux, and megaureter. In
those cases, we must remember that the single
kidney has been suffering in utero, and therefore,
we must expect a serious functional limitation.
Every effort should be done to protect the dam-
aged kidney. For this, it is mandatory to do a kid-
ney and bladder ultrasound in all babies with
anorectal malformation. If the ultrasound shows
abnormalities such as hydronephrosis, then the
baby will need a full urologic evaluation includ-
ing, of course, a voiding cystourethrogram. If the
kidney ultrasound is normal and the patient is Fig. 4.2 Characteristic radiologic image known as “dou-
passing urine without difficulty, we must assume ble bubble” in a baby with duodenal atresia
4.4 Physical Examination
a b
Fig. 4.3 X-ray films of a normal sacrum in a baby with an anorectal malformation. (a) AP view. (b) Lateral view
that most likely his urinary tract is otherwise
healthy.
Taking a baby to the operating room to repair
an anorectal malformation or to open a colostomy
only to find that the baby gets very sick during
the operation because he has a serious cardiac,
esophageal, or kidney problem is an undesirable
and preventable experience.
All babies with anorectal malformations must
have an abdominal x-ray film that shows the degree
of bowel dilatation, the characteristics of the spine,
and the characteristics of the sacrum. The presence
of intraluminal calcifications in the rectosigmoid
most likely is due to the passing of urine to the
bowel. Urine mixed with meconium may produce
calcifications [14, 15]. The sacral films must
include AP and lateral views (Figs. 4.3 and 4.4).
The characteristics of the sacrum are extremely
important in order to determine the future func-
tional prognosis for bowel control, urinary control,
and sexual function. Traditionally, we evaluated
the sacrum by counting the number of vertebrae.
We found this to be a rather limited nonuseful way,
and therefore, we created what we call the sacral
ratio (Figs. 4.5 and 4.6) (see Chap. 6).
Taking x-ray films of the sacrum is mandatory
also because we must rule out the presence of a
35
hemisacrum, which means that the patient has a
presacral mass; this finding has important thera-
peutic and prognostic implications.
An ultrasound of the lower sacrolumbar spine
is extremely useful and must be done during the
first hours of life to determine whether or not the
baby has tethered cord (Fig. 4.7). This is also
important to determine the functional prognosis
for bowel and urinary control.
All these studies can be done during the first
24 h of life. Babies with anorectal malformations
are usually not born with a distended abdomen. It
takes a few hours for the abdomen to start becom-
ing distended. It is after 24 h of life that the
abdominal distention becomes critical, and a
management decision must be taken.
4.4 Physical Examination
4.4.1 Male Patients
During our first contact with the baby with ano-
rectal malformation, we should dedicate a special
time for a meticulous detailed examination of
the baby’s perineum. Babies with anorectal
36
a b
Fig. 4.4 X-ray films of an abnormal sacrum in a baby with an anorectal malformation. (a) AP view. (b) Lateral view
malformations have different external appear-
ances of their perineum, and they have very
important clinical significance.
The presence of a flat bottom, meaning
absence of the normal midline groove that all
human beings have between both buttocks, is
usually associated with malformations with bad
prognosis and very highly located rectum
(Fig. 4.8). In addition, most patients with anorec-
tal malformations have an anal dimple that repre-
sents the point in the perineum where the patients
have the largest concentration of sphincter fibers
(Fig. 4.9). The more prominent the anal dimple,
the better the quality of the sphincter and there-
fore the prognosis. The absence of an anal dimple
is a very bad sign, usually present in poor prog-
nosis type of defects. The location of the anal
dimple varies from patient to patient. The closer
the anal dimple to the scrotum, the worse the
prognosis (Fig. 4.10). The longer the distance
4 Neonatal Management
between the tip of the coccyx and the anal dim-
ple, the poorer the prognosis, either because the
anal dimple is located too anteriorly or because
the sacrum is very short or both. We call it a
“good-looking perineum” when the baby has a
well-formed midline groove and a well-located
anal dimple (Fig. 4.9a). Even by touching that
area, one can see the contraction of the sphincter
of the anal dimple.
The most “benign” of all anorectal malforma-
tions is called “perineal fistula.” The rectum
opens into the perineum, anterior to the sphincter
in a rather narrow orifice. This malformation is
also known as a “low defect.”
A perineal fistula in a male patient may have
different external manifestations. A common one
is the presence of a malformation called “bucket
handle” (Fig. 4.11) which is a prominent skin
band under which we can pass a mosquito clamp.
Another external manifestation of a perineal
4.4 Physical Examination
a Anterior-Posterior
A B
B
C
Normal Ratio: BC =.74
AB
b Anterior-Posterior
Fig. 4.5 Normal sacral ratio. (a) Diagram. (b) Radiograph
fistula can be a subepithelial fistula (Fig. 4.12).
The fistula tract may be full of meconium, giving
the appearance of a “black ribbon.” Other times,
it may be full of white mucous material
(Fig. 4.13). The black or white subepithelial tract
may extend toward the scrotum in the midline or
even to the base of the penis.
A perineal fistula can be repaired with an ano-
plasty during the neonatal period without a colos-
tomy and has an excellent functional prognosis.
37
Lateral
A
C
BC
= 0.77
AB
Lateral
The exception would be the group of patients
with perineal fistulas associated to an abnormal
sacrum and a presacral mass. Ironically, the pres-
ence of a presacral mass and hemisacrum seems
to be more frequently associated to perineal fistu-
las than to other anorectal defects.
Occasionally, one may see a patient with a
subepithelial fistula or a “bucket handle” malfor-
mation; we try to repair the defect only to find
that the patient has actually a very long narrow
38 4 Neonatal Management

a b

Fig. 4.6 Abnormal sacral ratio. (a) AP view. (b) Lateral view

Fig. 4.8 “Flat bottom” in a baby with a recto-bladder

Fig. 4.7 Spinal ultrasound of a newborn baby. (a)
Normal. (b) Tethered cord
fistula and the rectal pouch is located high in the
pelvis. That is a real exceptional situation
(Fig. 4.14). Under those circumstances, the sur-
geon has to decide to continue the operation to
mobilize the rectum down or to abort the proce-
neck fistula
dure and open a colostomy. That would depend
on the degree of experience of the operator.
The presence of bifid scrotum (Fig. 4.15) is
usually associated to a rather complex defect;
most likely the rectum is located high in the
pelvis, connecting to the urinary tract very high
(bladder neck or prostatic fistula), although there
4.5 Female Babies
a b
Fig. 4.9 Photograph of the perineum of a newborn baby with (a) good anal dimple. (b) Absent anal dimple. Arrow
shows the anal dimple
are exceptions. The bifid scrotum is something
that we usually repair at the same time that we
repair the anorectal malformation (see Chap. 23,
Sect. 23.5).
4.5 Female Babies
In female patients, the surgeon must be particu-
larly careful in the examination of the baby’s
perineum and genitalia. One should put the baby
in a convenient lithotomy position with some-
body holding the baby’s legs. We should have
good illumination and magnifying glasses if nec-
essary and take the time to clean the genitalia and
to separate the labia to see if there is meconium
and precisely where it comes from. Some fistulas
are very narrow, and it takes several hours, some-
times up to 24 h, for the babies to pass meco-
nium. The most common defect seen in babies is
a malformation called vestibular fistula. As we
separate the labia, we can see the urethral open-
ing, the hymen, and the vaginal orifice, and
immediately behind that, but still within the
introitus of the baby, one can see another little
orifice that we call vestibular fistula (Fig. 4.16).
The second most common defect that we see
is what we call perineal fistula; the orifice is
located somewhere between the normal location
of the anus (anal dimple) and the vestibule of the
genitalia (Fig. 4.17). Frequently, that orifice, ves-
tibular or perineal, is too narrow to function
39
adequately as an anus. One can identify the anal
dimple very clearly located posterior to the peri-
neal or the vestibular fistula.
Although most patients have a well-defined
vestibular or perineal fistula, some babies are
born with an orifice located right in between, in
what the French authors call the “fourchette” of
the genitalia (Fig. 4.18).
When the female baby has no anus and the
external genitalia look rather small (Fig. 4.19),
the surgeon should suspect that the baby has a
cloaca. A cloaca is defined as a malformation in
which the baby is born with a single perineal ori-
fice. Early diagnosis by inspection of this defect
is extremely important. The surgeon must
remember that about 90 % of the patients with
cloacas may have serious urologic problems. The
surgeon must take the time and be meticulous
enough to separate the little labia of those small-
looking female genitalia and will be able to see a
single perineal orifice, and by doing that, he/she
already made a diagnosis of a cloaca. Some
babies are born with genitalia that induce the
doctor to make a diagnosis of intersex or a disor-
der of sexual development. That is because the
patients have a structure that looks like a phallus
(Fig. 4.20). They have a single perineal orifice
and no testicles, and therefore, the doctors are
incapable of saying whether the baby is male or
female. In fact, about 60 of our patients with clo-
aca born in other institutions have been sent to us
with a label or misdiagnosis of “intersex.” These
families were told that the baby had an
40
Fig. 4.10 Location of the anal dimple. (a) Normal dis-
tance between the scrotum and the anal dimple (malfor-
mation with good functional prognosis). (b) Anal dimple
next to the scrotum (malformation with less than optimal
functional prognosis). Arrow shows the anal dimple
undetermined gender and therefore needs a full
evaluation by a geneticist, endocrinologist, and
urologist. A series of tests were run, only to find
out that the baby actually is XX and is otherwise
a normal female, except for the cloaca
4 Neonatal Management
Fig. 4.11 “Buckle handle” malformation in a baby with
a perineal fistula
Fig. 4.12 “Black ribbon” appearance of a subepithelial
fistula, external manifestation of a perineal fistula
malformation. In fact, we have never seen a baby
with a cloaca with sexual developmental disor-
der. The key for the diagnosis in this case is to
palpate that prominent structure that looks like a
phallus. In a real phallic hypertrophy like in cases
of adrenal hyperplasia, one can palpate the cor-
pora inside that structure, whereas in babies with
4.6 Neonatal Management
Fig. 4.13 “White ribbon” appearance of a subepithelial
fistula, external manifestation of a perineal fistula
cloacas, the palpation reveals that there is only
folded prominent skin with no corpora and that
makes the diagnosis of a cloaca with no need to
rule out an intersex.
A baby with Down syndrome and absent anal
orifice has over 90 % chances to have an imperfo-
rate anus with no fistula (see Chap. 12).
4.6 Neonatal Management
When we see the baby for the first time, we must
make a series of management suggestions to our
colleagues, neonatologists, or pediatricians.
These include to start the administration of intra-
venous fluids, to maintain the baby with nothing
by mouth, and to introduce a nasogastric tube to
avoid vomiting. The nasogastric tube does not
interfere with the development of abdominal dis-
tention that the baby will have in the following
24 h, but will avoid the risk of vomiting and
41
Fig. 4.14 Intraoperative aspect of a long narrow perineal
fistula communicating with a very high rectum
aspiration. A urinalysis is ordered particularly in
male babies, looking for the presence of meco-
nium in the urine. We also prescribe intravenous
antibiotics. If it is a female baby and has a fistula
(vestibular or perineal), we might pass a little
metallic dilator to facilitate the passing of meco-
nium to determine whether or not the fistula is
competent to decompress the abdomen and avoid
abdominal distention. We must explain to the
neonatologist that during the following 20–24 h,
the baby should have the diagnostic studies that
we already mentioned, including a chest film, an
abdominal film, an echocardiogram, an ultra-
sound of the lumbosacral spine, an ultrasound of
the kidneys, and an ultrasound of the pelvis. In
babies with cloacas, we emphasize the need to do
an ultrasound of the kidneys and also an ultra-
sound of the pelvis, looking specifically for the
42 4 Neonatal Management

presence of a hydrocolpos. We know, from our

experience with cloacas, that approximately
60 % of them have an associated hydrocolpos
that may be unilateral or bilateral. We also know
that the hydrocolpos may compress the trigone of
the bladder provoking an extrinsic ureterovesical
obstruction with megaureter and hydronephrosis.
It is extremely important to make this diagnosis
prior to any kind of intervention to be done in the
baby. The hydrocolpos must be drained as early
as possible, during the first surgical intervention
of the baby.
Based on this evaluation and on the experi-
ence gained with the long-term follow-up of our
patients, we can establish, fairly accurately, the
future functional prognosis of the baby and have
a long conversation with the parents. We usually
tell the parents that when a baby is born with an
anorectal malformation, the main concern of the
parents as well as the clinicians is to determine
what is going to be the quality of life of the baby
for the following 80 years. More specifically, is
Fig. 4.15 Bifid scrotum, a defect frequently associated to
a highly located rectum
the patient going to have bowel control? Is the

a b

Fig. 4.16 Vestibular fistula in a newborn baby. (a) Without separating the labia. (b) Separating the labia
4.6 Neonatal Management 43

baby going to have urinary control? Is the patient

going to have sexual function and will be able to
reproduce? All these with the specific purpose to
avoid what we call the saga of parents of children
with anorectal malformations.

Fig. 4.18 “Fourchette fistula” – fistula located between

Fig. 4.17 Perineal fistula – arrow shows the fistula the vestibule and the perineum

a b

Fig. 4.19 Single perineal orifice. (a) External view. (b) Separating the labia
44
Fig. 4.20 “Phallus-like” prominent skin in a patient with
a cloaca. Frequently confused with “intersex”
Many parents of patients born with anorectal
malformations describe their unfortunate experi-
ence of having a newborn baby with an anorectal
malformation. They mention that they were a
young happy couple with great expectations and
hopes about having a baby. After the delivery of
the baby, a doctor appeared in the mother’s room
and said that the baby had “no anus.” Most par-
ents never heard of this malformation because it
is not the type of malformation that people like to
talk about. As a consequence, most parents never
heard of the existence of this malformation. Then
the doctors tell the family that the baby is going
to have an operation, and subsequently, they
come back to say that the operation was “suc-
cessful.” The parents feel happy and take the
baby home, but most of the time nobody dis-
cusses the future of the baby. At that time, the
baby is either on diapers or has a colostomy.
Eventually, the baby undergoes the main repair of
the malformation followed by a colostomy clo-
sure. Since the baby is usually still wearing
4 Neonatal Management
diapers, the parents do not perceive the difference
of their baby’s bowel habits when compared
with babies without anorectal malformations.
However, when the baby reaches the age of bowel
control, the parents start making observations,
comparing the bowel habits of their baby with
other normal children and start worrying. It is
then when they start going from doctor to doctor
only to find out, sometimes years later, that the
baby actually was born with malformation and
with a bad functional prognosis and therefore
will never have bowel control. We believe that
even if this is extremely painful and difficult for
us, we are morally obligated to try to establish the
functional prognosis as early as possible to adjust
the parents’ expectations and avoid future painful
revelations. As can be seen in Chap. 20, when
dealing with families of children born with poor
functional prognosis type of defects, we offer
them a comprehensive bowel management pro-
gram to be started at age 3, and we commit our-
selves to keep these patients artificially clean in
the underwear, in order to be socially accepted,
attend school, and avoid psychological sequelae.
On the other hand, if the baby was born with a
good prognosis type of defect, we have the plea-
sure to tell the parents the good news.
Once we have all of the information that we
already described and we are certain that the
baby does not have a serious associated malfor-
mation that requires urgent care, we are ready to
answer the second question, related to the possi-
bility of opening a colostomy or doing some sort
of primary operation to create an anal opening.
Most of the time, with the meticulous examina-
tion of the perineum, the result of the urinalysis,
or the obvious presence of meconium in the
urine, as well as the results of the ultrasound and
the radiology studies, with a good index of suspi-
cion, we have enough information to make a
decision. Occasionally, after 24 h, in spite of all
the studies and examinations, we still do not have
a clear idea of what we are going to do (primary
repair or colostomy). In the old times, it was cus-
tomary to take an upside-down film [16] in lateral
position and with a marker in the anal dimple,
with the specific purpose of measuring the dis-
tance between the skin of the perineum and the
4.6 Neonatal Management
blind end of the rectum full of gas. At some point,
it became obvious that the same image obtained
with the upside-down film could be obtained with
the baby in a prone position, with the pelvis
elevated [17] (Fig. 4.21). In addition, it was also
risky to put the baby upside down, for the risk of
vomiting and aspiration.
The cross-table lateral film renders a reliable
image when it is taken 24 h post birth. When the
rectal bubble is located well below the coccyx,
the surgeon knows where to expect to find the
rectum (Fig. 4.21).
Babies with a flat bottom, poor sacrum, and
tethered cord need a colostomy most of the time.
Also, in babies that are passing meconium with
the urine, we suggest to open a colostomy.
Patients with “bucket handle” malformations,
subepithelial fistula, or an obvious perineal fis-
tula opening can be repaired primarily during the
newborn period.
There is one particular physiologic event
worth discussing, because of its diagnostic and
therapeutic implications. At birth, most babies
with anorectal malformations do not have a dis-
tended bowel and abdomen. It takes 18/24 h for
a b
Fig. 4.21 Cross-table lateral film (rarely used study). (a) Baby’s position. (b) Image of a reachable rectum. (c) Image
of a non-reachable rectum
45
the rectosigmoid to become distended. In addi-
tion, the rectum is surrounded by a striated
funnel-like sphincter mechanism (see Chap. 2)
with a significant tone. The muscle tone keeps the
most distal part of the rectum collapsed, until the
intraluminal pressure is high enough to overcome
the muscle tone. This occurs usually after 24 h;
therefore, all imaging diagnostic tests aimed to
detect the location of the blind rectum are inac-
curate when performed before 24 h of life. This
important fact is rarely mentioned. This is the
reason why we emphasize to spend the first 24 h
of the baby’s life in trying to rule out serious
associated conditions and try to determine the
location of the rectum to decide the surgical
approach after a 24 h period.
In order to determine the position of the rectal
pouch, different authors suggest using a perineal
ultrasound [18–21]. Others are enthusiastic about
the perineal injection of contrast material [22–
25]. More advance and sophisticated imaging
technology has been used, including CT scan
[26] and MRI [27–29].
None of the publications related with the opti-
mal imaging diagnostic studies to determine the
46 4 Neonatal Management

position of the rectal pouch mentioned the most most distal part of the rectum, as well as the loca-
important key factor which is the timing of those tion of the recto-urinary fistula; all of this repre-
studies. It does not matter how sophisticated the sents crucial information that allows us to make a
imaging technology employed is, if one does not well-defined, precise surgical plan. Primary
take into consideration the fact that the distal rec- repairs during the newborn period frequently
tum is surrounded by a striated muscle. Studies become authentic misadventures that expose the
done before the rectum becomes distended will baby to serious consequences and sequelae.
fail to make an accurate diagnosis. When the decision is reached to open a colos-
In general, all over the world, the pediatric tomy, the patient is taken to the operating room,
surgical community is moving toward the pri- and the surgeon should follow the principles
marily repair of anorectal malformations, in an described in Chap. 5. The anoplasty that we use
effort to avoid the significant morbidity of two for the treatment of perineal fistulas is described
important operations: colostomy opening and in Chap. 8.
colostomy closure (see Chap. 5) [30–37]. We
agree that we must try to move in that direction;
however, we must keep a very critical attitude to 4.7 Cloacal Exstrophy
be able to balance the desire of notoriety with the
benefit of the patient. We should always ask our- There is a specific chapter dedicated to this defect
selves what we would do if the patient was our (Chap. 17); here, we will only mention the spe-
son or daughter. cial neonatal care that these patients require. This
When making these kinds of decisions, the is the most serious of all congenital anorectal and
surgeon must take into consideration his specific urogenital malformations. These babies are born
surrounding circumstances, as well as his experi- with an omphalocele, an exstrophic bladder with
ence. One example could be the case of a new- two hemibladders and in the middle of both of
born baby that has a perineal fistula but is them, a portion of intestine protruding in what is
extremely sick either because he/she is prema- called an “elephant trunk” (Fig. 4.22). The pubic
ture, has respiratory distress, and may have a car- bones are widely separated. There is no anus, and
diac condition or other aggravating factors. In
such cases, we can simply dilate the fistula. If
that is not enough, we can make a cutback proce-
dure on temporary basis, in order to decompress
the abdomen and help the baby to recovery.
In general, we consider contraindicated per-
forming abdominal perineal, pull-throughs open
or laparoscopic, as well as posterior sagittal ano-
rectoplasties in neonatal babies. As can be seen
in the chapter of reoperations (Chap. 22), we
have seen multiple cases of patients approached
during the neonatal period, without the necessary
anatomic information, trying to repair an anorec-
tal malformation; many of those patients suffered
very serious damage of important pelvic
structures.
In addition, in newborn babies, we cannot do
the most valuable radiologic study in the man-
agement of anorectal malformation which is
called high-pressure distal colostogram (Chap.
6), which shows us the precise location of the Fig. 4.22 Cloacal exstrophy
References
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“elephant trunk.” It is very common to see an
associated meningocele and serious spinal
defects that may affect the motion of the lower
extremities.
The management of these patients requires a
multidisciplinary team of experts that include a
pediatric surgeon, pediatric urologist, orthopedic
surgeon, neurosurgeon, and of course neonatolo-
gists. From the time of delivery, nurses and doc-
tors must try to protect the pelvic structures. The
mucosa of the bladder and bowel are both
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care. The omphalocele is a delicate structure that
must be handled with special care to avoid its
rupture. These structures must be protected with
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baby will also receive antibiotics and a nasogas-
tric tube and will be on NPO. Twenty-four hours
later (sometimes more), the patient is usually
taken to the operating room. The surgical man-
agement is described in Chap. 17.
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neonatal males. J Pediatr Surg 42(11):1877–1881
 Colostomy
5.1 Introduction
Colostomy is a procedure designed to divert the
fecal stream from the normal passage to the rec-
tum, creating an opening between the colon and
the abdominal wall. This procedure was created
to relieve the obstruction of the colon produced
by acquired or congenital conditions. Another
indication is to avoid the passing of stool through
an operated area, trying to prevent complications,
such as infection and/or dehiscence [1–6].
Historically, it is considered that the first
colostomy in pediatrics was performed in 1783
by Antoine Dubois in a 3-day-old infant with an
imperforate anus [7].
A colostomy can be permanent, when it is
considered that there is no way to reconstruct the
colon distal to the stoma or there is no way to
establish bowel control, and it is considered that
the quality of life is better with a stoma as com-
pared without stoma.
Temporary colostomies are those created for a
period of time until the anatomic or functional
circumstances of the patients allow the reestab-
lishment of the colonic transit. Colostomies can
be divided into two groups: totally diverting and
partially diverting.
Totally diverting colostomies are those that
divert the entire fecal stream and do not allow the
spillage or passing of stool into the bowel distal to
the stoma. In order to achieve total diversion of
the stool, it is necessary to separate the proximal
and distal bowel after the colon is divided and to
A. Peña, A. Bischoff, Surgical Treatment of Colorectal Problems in Children,
DOI 10.1007/978-3-319-14989-9_5, © Springer International Publishing Switzerland 2015
5
separate both stomas enough, as to allow the
proximal (functional) stoma to be covered by a
stoma bag without including the distal stoma
(Fig. 5.1). Another way to achieve a totally divert-
ing procedure is by closing the distal end, a
maneuver that is known as a “Hartmann pouch.”
The closure of the distal stoma leaves the patients
with a completely closed blind loop distal bowel;
this, in the absence of a fistula, creates a mucocele
(accumulation of mucus) which will represent a
serious problem for the patient, as most muco-
celes eventually become infected. Mucoceles may
also occur in cases in which the fistula that con-
nects the colon with the urogenital tract is very
narrow and does not allow the passing of mucus.
In addition, patients with a Hartmann pouch can-
not have contrast studies done through the distal
stoma to evaluate their anatomy prior to recon-
struction because there is no access to it. This is a
serious deficiency, because we firmly believe the
most valuable diagnostic test in patients with ano-
rectal malformation consists in the injection of
contrast material through the distal stoma (high-
pressure distal colostogram) (see Chap. 6).
A partially diverting stoma, by definition,
allows most of the stool to come out of the body,
but some of the stool still may go into the distal
colon. This happens when both stomas are
together and covered by a single stoma bag, and
the surgeons open a colostomy known as “loop
colostomy” (Fig. 5.2).
The indication to divert the fecal stream totally
or partially depends on the specific problem of
49
50 5 Colostomy

a b

Fig. 5.1 Both stomas separated enough to cover only the proximal one with the stoma bag. (a) Diagram. (b) Picture

a b

Fig. 5.2 Inadequate colostomy. (a) Both stomas located too close. (b) Loop colostomy

the patient. In cases of anorectal malformations,
the surgeon should keep in mind that over 85 %
of all the patients have a connection between the
distal colon and the urogenital tract. In addition,
5 % of the total group of anorectal malformations
has a completely blind distal bowel. This is very
important to remember, because a partially
diverting colostomy (loop or both stomas located
too close one to the other) exposes the patient to
the passing of stool into the urinary tract and/or
to a distal impaction of stool that cannot pass
through a narrow fistula. Loop colostomies are
very appealing for most surgeons. We think the
reason for this is that it is an easy operation that
5.2 Stoma Locations 51

a b

Fig. 5.3 Loop colostomy with distal fecal impaction. (a) Diagram. (b) Picture

can be done fast and also can be closed in a fast

and easy way. However, we consider that loop
colostomies are formally contraindicated in
patients with anorectal malformations due to the
following reasons:
• High chances of producing direct fecal con-
tamination of the urinary tract
• High chances of producing distal fecal impac-
tion, megarectum, and consecutive severe
constipation after colostomy is closed
(Fig. 5.3)
• Higher incidence of prolapse

5.2 Stoma Locations

The stoma can be opened in the abdomen in dif-

ferent locations. The most common locations are:
• Right transverse colostomy (right upper quad-
rant): Some surgeons prefer this type of stoma
consisting in dividing the right portion of the
transverse colon and diverting it through the
right upper quadrant of the abdomen (Fig. 5.4).
• Left transverse colostomy: The left portion of
the transverse colon is divided, and the Fig. 5.4 Photograph of a right transverse colostomy
52
Fig. 5.5 Diagram of a left transverse colostomy
Fig. 5.6 Diagram showing a descending colostomy with
separated stoma (preferred by the authors)
stoma(s) is opened in the left upper quadrant
of the abdomen (Fig. 5.5).
• Descending colostomy: The bowel is divided
immediately distal to the descending colon, in
the first mobile portion of the sigmoid, and the
stoma is usually opened in the left lower quad-
5 Colostomy
Fig. 5.7 Sigmoid colostomy, leaving a too short distal
bowel that interferes with the pull-through
rant of the abdomen (Fig. 5.6). This is the type
of colostomy that we recommend.
• Sigmoid colostomy: The sigmoid colon is
divided, and the stomas are usually opened
somewhere in the lower abdomen. The prob-
lem with this kind of colostomy is that there is
a possibility of creating the stoma too distal in
the colon, leaving a very short piece of bowel
available for the pull-through (Fig. 5.7).
5.3 Ileostomies
Ileostomies are frequently performed in patients
with colorectal problems. These are indicated
when, for some specific reason, we cannot use the
colon to perform the diversion. In other words, the
entire colon does not function, such as in cases of
total colonic aganglionosis or when the patient was
born with no colon or has lost the entire colon. An
ileostomy is sometimes used when a patient had a
pull-through of the ascending colon, and therefore
the patient needs a diversion proximal to this.
5.4 To Divert or Not to Divert, That Is the Question
5.4 To Divert or Not to Divert,
That Is the Question
In general, pediatric surgeons and general sur-
geons are looking for safe ways to perform
colorectal procedures without a protective colos-
tomy. By doing this, the patients are prevented
from having two extra serious operations with
significant morbidity (colostomy opening and
colostomy closure). That is the reason why to
perform colorectal surgery without a colostomy
is always an attractive idea (see Chap. 4). Taking
advantage of modern surgical technology, effi-
cient methods to clean the colon, the possibility
of keeping the patient with nothing by mouth for
a period of time receiving parenteral nutrition,
the use of sophisticated surgical techniques, and
the availability of powerful antibiotics, allow,
nowadays, to perform successful operations on
the colorectal tract without a protective colos-
tomy. Yet, catastrophic complications still hap-
pen [8]. It is true, the incidence of these
complications is much less than in the past, but
unfortunately they still occur. That is why the
question whether “to divert or not to divert” is
still unanswered and remains a matter of
controversy.
In general, pediatric surgeons keep moving in
the direction of doing more and more primary
procedures without a protective colostomy. We
believe that it is good to move in that direction, to
save our patients from the potential morbidity
associated with stomas that are still high [3,
9–30].
It is also very important to remember that in
dealing with the treatment of anorectal malfor-
mations, a postoperative wound infection has
consequences much more serious than in cases of
other surgical conditions. A wound infection due
to the repair of an anorectal malformation means
not only that the patient will suffer the inconve-
niences and risks related with the infection itself,
but, in addition, the final functional prognosis
(bowel and urinary control) may be jeopardized.
We are against universal indications for a pro-
cedure. In other words, we believe that a colos-
tomy is indicated under certain specific
circumstances; a patient with a specific malfor-
53
mation may need a colostomy, and yet another
patient with exactly the same type of defect, but
under different surrounding circumstances, may
not require a colostomy. This includes how sick
the patient is, how severe are their associated
defects, how advanced is the technology avail-
able for the patient, how much experience the
surgeons have in the performance of primary pro-
cedures done without a colostomy, and how
sophisticated is the infrastructure that surrounds
the patient, including laboratory, intensive care,
surgical technology, availability of central venous
access, hyperalimentation, and a clean
environment.
A colostomy definitely still has a recognized
protective value in the postoperative course of
most colorectal and anorectal operations. In other
words, not opening a protective colostomy has a
definite increased risk for the patient. Admittedly,
many colorectal procedures can be done success-
fully without a protective colostomy, but cannot
be done without the acceptance of a certain
degree of risk.
Finally, we believe that when a surgeon is con-
fronted with the difficult decision of whether to
open or not to open a colostomy in a specific
patient, he or she should always try to imagine
what he would do if he was dealing with his own
son or daughter.
Most of the patients, who come to us after the
neonatal period, already have a colostomy, and
therefore we do not have to deal with this
dilemma.
In a full-term, newborn baby without severe
associated defects, we do not open a colostomy if
the baby has one of the following malformations:
perineal fistula, vestibular fistula, imperforate
anus with no fistula, and rectourethral bulbar fis-
tula. In the case of the last two malformations
(imperforate anus without fistula and rectoure-
thral bulbar fistula), we expect to see the distal
end of the rectum full of gas, located below the
coccyx in a cross-table lateral film. Based on our
experience, we can confidently operate primarily
on these types of cases without a colostomy with
good results. All other patients with anorectal
malformations, at our institution, receive a colos-
tomy, not only to protect the patient from the
54
operation to repair the malformation, but also for
other reasons, including the fact that we need a
stoma in order to do a high-pressure distal colos-
togram which we consider the most valuable
diagnostic test in patients with anorectal malfor-
mations. Other sophisticated, state-of-the-art
imaging modalities still cannot compete with the
accuracy of the anatomic information that we
obtain with a high-pressure distal colostogram.
Most of the serious catastrophes we have seen
occurred in cases that were surgically explored at
other institutions without a high-pressure distal
colostogram [8]. In addition, higher anorectal
malformations have a higher incidence of serious
associated defects, mainly urologic, cardiac, and
gastrointestinal, which means higher-risk patients.
5.5 Recommended Types
of Colostomies
5.5.1 Newborn Babies
with Anorectal Malformations
In newborn babies with anorectal malformations,
in whom we consider that a colostomy is indi-
cated, we prefer to open a descending colostomy,
with widely separated stomas, located in the left
lower quadrant of the abdomen (Fig. 5.6) [1, 30].
In our series of 2,032 patients, only 75 of them
had a colostomy done at our institution. Over 200
cases underwent a primary repair without a colos-
tomy; most of those suffered from perineal or ves-
tibular fistula. All of the others came to our
institution with a colostomy already open. As the
reader can imagine, that means that we have seen
almost all kinds of colostomies and have learned the
advantages and disadvantages of each type. That
gave us an illuminating experience related to colos-
tomies [30]. Based on that experience, we con-
cluded that a descending colostomy with separated
stomas is the best one, for the following reasons:
(a) It effectively diverts the entire fecal stream.
(b) It significantly decreases the chances of uri-
nary tract infection.
(c) It avoids the formation of megarectosigmoid
because it allows the irrigation and cleaning of
the distal bowel and avoids distal fecal spillage.
5 Colostomy
(d) It virtually eliminates the chances of hyper-
chloremic acidosis from resorption of urine
[31, 32].
(e) It does not interfere with the pull-through.
(f) It will not prolapse when done properly.
Transverse colostomies are not recommended
in anorectal malformations for several reasons:
(a) It is impossible to irrigate the distal colon
that remains full of meconium for the weeks
or months after the colostomy is created.
(b) It has a tendency to provoke a severe megar-
ectosigmoid, as a consequence of the pres-
ence of meconium that was never removed,
plus the accumulation of mucus produced by
the entire defunctionalized colon and des-
quamation of mucosa cells.
In fact, the longer the period of time
between the colostomy opening and the final
repair, the greater the megarectosigmoid
(Fig. 5.8). This will translate eventually into
severe constipation, difficult to manage, after
the repair of the malformation.
(c) Patients with recto-urinary fistulas not only
have a tendency to pass meconium from the
colon into the urinary tract, but also they tend
to pass urine into the colon, which is
absorbed, producing metabolic hyperchlore-
mic acidosis [33, 34]. The long defunctional-
ized segment allows this to occur.
(d) The incidence of urinary tract infection is
higher than in cases with descending
colostomies.
(e) The high-pressure distal colostogram (the
most valuable diagnostic study in anorectal
malformations) is difficult to do, may not be
accurate, and is risky. It is not accurate,
because it is very difficult to exert enough
hydrostatic pressure, when the injection of
contrast material is done from the transverse
colon to fill up and to demonstrate the fistula
site, located all the way down to the rectum.
In an attempt to demonstrate the location of
the fistula, the colon may perforate. We have
had two cases with such a complication. This
incident has never happened in patients with
descending colostomies.
The opening of a loop colostomy in the trans-
verse colon is perhaps the worst type of colostomy
5.8 Creation of a Colostomy
Fig. 5.8 Colostogram in a patient with transverse colos-
tomy, showing the characteristic narrow (non-used) distal
colon, with a megarectosigmoid
that we have seen, because in addition to all of the
problems that we have seen with transverse colos-
tomies, the patients pass stool into the distal bowel
which increases the chance of urinary tract infec-
tion and frequently produces fecal impaction in
the distal stoma. Figure 5.8 shows the characteris-
tic situation of a patient that had a bad loop trans-
verse colostomy with fecal impaction in the distal
bowel. Those fecal impactions cannot be relieved
by washing the colon. It is sometimes necessary to
perform a laparotomy to remove the hard stool
from the distal bowel prior to the main repair.
Loop colostomies, in general, also have a greater
tendency to prolapse (Fig. 5.9).
5.6 Left Transverse Colostomy
The complications that we mentioned about right
transverse colostomies are similar in a left trans-
verse colostomy.
55
Fig. 5.9 Prolapsed transverse colostomy
5.7 Cecostomies
Opening of colostomies in the cecum, in general,
has no indication in anorectal malformations. All
of the problems mentioned when discussing right
transverse colostomies are more serious in cases
of cecostomies.
In the type of descending colostomy that we
recommend, the proximal stoma will not pro-
lapse due to the fact that it belongs to the fixed
portion of the descending colon. On the other
hand, the distal stoma belongs to the mobile
portion of the sigmoid, and therefore it has a
higher risk of prolapse. To avoid that, we specifi-
cally recommend creating a very small (about
3 mm diameter) distal stoma (mucous fistula)
(Fig. 5.6).
5.8 Creation of a Colostomy
5.8.1 Surgical Technique
In a newborn baby with anorectal malformation,
we try to perform a colostomy not before 24 h
after the baby is born. In the chapter dedicated to
the management of a newborn with an anorectal
malformation, we explain in detail the reasons
56
Fig. 5.10 Diagram showing an oblique preferred inci-
sion for a neonatal colostomy
why we want to wait 24 h. However, we do not
want to wait much more than that time, because
perforations of the bowel have been reported to
occur in patients after 24 h of life. It is true that
occasionally, patients can live many days passing
meconium through the recto-urinary fistula with-
out perforation, but that is an exception. There
are reports of catastrophes that occurred in
patients who suffered colon perforation and died
because a colostomy was not done on time (see
Chap. 4). The patient is taken to the operating
room, and under general anesthesia, the abdomi-
nal wall is washed, prepped, and draped in the
usual manner. The incision that we recommend is
an oblique one, running from the left flank down
to the left lower quadrant of the abdomen
(Fig. 5.10). The upper part of the incision is
located at the same site where we expect to create
the proximal stoma. This point is located at equal
distance between the umbilicus, the ribs, and the
anterior superior iliac crest. We intentionally
want the proximal, functional stoma to be sur-
rounded by normal skin and to be located as far
away as possible from a prominence, irregularity,
or structure that may interfere with the placement
of a stoma bag. These structures are the umbili-
cus, the rib cage, and the iliac bone. When the
stomas are open too close to one of these struc-
tures, the stoma therapist, the nurses, and the
mothers struggle trying to place a stoma bag that
lays flat, avoiding leakage of stool and resultant
skin irritation.
5 Colostomy
Fig. 5.11 Trans-operatory picture, a dilated sigmoid
colon, full of meconium, is seen
The lower and medial end of the incision rep-
resents the location of the mucous fistula site,
which we create intentionally very small
(Fig. 5.10) to avoid prolapse. Both stomas must
be separated enough so as to be able to use a
stoma bag only on the proximal stoma, away
from the mucous fistula.
The abdomen is opened, and it becomes very
obvious that there is a big, dark loop of bowel
(Fig. 5.11) that represents a very dilated sigmoid
full of meconium. One should not try to manipu-
late this very tense, dilated sigmoid loop of
bowel, because this may result in injuries to the
seromuscular layer. Also, one should not try to
create the stoma in the most dilated part of this
colon, because that would make a huge stoma
difficult to manage and more prone to suffer pro-
lapse. We rather should look for the less-dilated
descending colon, normally fixed to the left retro-
peritoneum. The descending colon detaches from
the left parieto-colic space and becomes mobile
(Fig. 5.12). At that particular point, we select a
portion of the bowel, long and mobile enough, to
comfortably reach the anterior abdominal wall.
Before we divide the bowel at the selected loca-
tion, we specifically suggest putting a purse-
string suture with a 5-0 suture. In the center of the
purse-string suture, we make an opening in the
bowel wall and introduce a 12 Foley catheter into
the lumen of the very dilated colon with meco-
nium. The purse-string suture is tied to avoid
5.8 Creation of a Colostomy
Fig. 5.12 The proximal stoma must be created using the
first mobile portion of the descending colon
Fig. 5.13 A purse-string suture is placed on the anterior
wall of the selected loop of the colon, where the stoma
will be located
leakage of meconium in the operative field
(Fig. 5.13). The dilated colon is then irrigated
with warm saline solution for a period of about
10–15 min until the entire sigmoid is completely
decompressed and free of meconium. This
maneuver is extremely valuable for several rea-
sons. First, because it will allow the surgeon to
manipulate a collapsed, well-perfused bowel
(Fig. 5.14) and to perform a neat operation pre-
serving the bowel integrity. In addition, the
patient will have a clean, collapsed colon for the
rest of the weeks or months before the pull-
through or main repair is done. We have evidence
to believe that this helps to avoid the formation of
57
Fig. 5.14 A catheter is introduced through the center of
the purse string to irrigate and remove all the meconium.
The bowel collapses and is well perfused
a megarectosigmoid and therefore decreases the
incidence of severe constipation in these patients.
In the same place where the purse-string suture
was placed, we apply two Baby Allen clamps to
divide the bowel (Fig. 5.15). Special care and
attention must be given to the preservation of the
colonic vascular arcade during the division of the
bowel. The preservation of the arcade allows
manipulating and mobilizing the distal bowel, at
the time of the main repair, preserving a good
blood supply. The proximal bowel will be exteri-
orized as a functional stoma at the left upper cor-
ner of our incision and the mucous fistula in the
lower and medial end of the incision. The last 2
or 3 cm of the distal bowel is tapered, creating a
little stoma (mucous fistula) of approximately
3–4 mm diameter (Fig. 5.15). The mucous fistula
is necessary to do irrigations of the distal bowel if
indicated and also to allow access to the distal
stoma to perform a high-pressure distal colosto-
gram. For this, we do not need a large stoma that
may bleed and interfere with the quality of life of
the patient. The tiny lumen also helps to avoid
prolapse.
The proximal stoma must be meticulously
constructed. The bowel is sutured to the fascia
and peritoneum, being sure not to produce a stric-
ture and/or ischemia and being sure that it is per-
fectly open and patent. There are no concerns
about prolapse because this stoma is placed at the
first mobile portion of the sigmoid after the
58 5 Colostomy

a b

Fig. 5.15 The colon is divided at the same location of the showing the tapering of the distal bowel. (c) Intraoperative
purse-string suture, and the distal bowel is tapered. (a) view. Arrow showing tapered distal bowel
Diagram showing the division of the colon. (b) Diagram

descending colon, which is normally fixed. The

peritoneum and aponeurosis between both sto-
mas are sutured together with long-term, absorb-
able sutures (5-0 Vicryl) (Fig. 5.16). The anterior
aponeurosis is closed with the same suture mate-
rial, as well as the subcutaneous tissue and Scarpa
fascia. Both stomas, proximal and distal, are
matured with 6-0 long-term absorbable sutures
taking the skin edge, the bowel wall, and the
bowel edge (Fig. 5.17).
The skin in between both stomas is closed with
subcuticular 5-0, absorbable monofilament. We
try to leave a smooth surface between both stomas Fig. 5.16 Fixing the proximal stoma to the peritoneum
to facilitate the use of a stoma bag (Fig. 5.17). and fascia. Closing the wound in between both stomas
Many surgeons do not like this kind of colos-
tomy. They insist in saying that these patients This may be true in other hands; yet, we are
have a tendency to suffer from infection and very proud of our results, and we believe that the
dehiscence of the wound between both stomas. key for success depends on the observation of a
5.9 Colostomy in Cases of Cloaca with Hydrocolpos
Fig. 5.17 Both stomas are meticulously “matured.” The
operation is finished
Fig. 5.18 Colostomy aspect weeks after operation
meticulous technique, delicate care of the tissues,
and irrigation of every layer of the wound closure.
The final result is cosmetically adequate, and the
stomas are easy to manage by the mothers. Our
incidence of prolapse in this kind of colostomy is
zero (Fig. 5.18).
When we open a technically correct type of
colostomy and place a stoma bag, usually there is
no need to change the bag for the following 3
days. The first removal of the bag must be done
very gently. When it is difficult to apply a bag, or
there are frequent episodes of leakage of stool,
this probably means that the colostomy was not
done correctly. The mucous fistula must be pro-
tected from contact with the diaper with a little
piece of Vaseline gauze.
59
Fig. 5.19 Creation of a window in the vaginal septum of
a case with bilateral hydrocolpos
5.9 Colostomy in Cases of Cloaca
with Hydrocolpos
When a baby is born with a cloaca and we have
evidence of the presence of hydrocolpos, which
happens in about 29 % of our patients (see Chap.
16), the surgeon must be prepared not only to
open a colostomy but also to drain the hydrocol-
pos. This represents an interesting technical chal-
lenge. If the hydrocolpos is large enough, the
surgeon may consider the possibility of connect-
ing the vaginal wall directly to the abdominal
wall, like in the case of a colostomy. However,
the surgeon must keep in mind that many patients
with hydrocolpos have two hemivaginas; in other
words, the hydrocolpos is bilateral. In fact, about
60 % of all patients with a cloaca have two hemi-
vaginas (see Chap. 16). A tube placed into one
vagina does not necessarily drain the other one.
Therefore, the recommendation in cases of two
hemivaginas is to be ready to open one of the
dilated vaginas and create a window in the vagi-
nal septum to be sure that both large hemivaginas
(hydrocolpos) are drained through a simple stoma
or with a single catheter (Fig. 5.19).
When the hydrocolpos is not large enough to
reach the anterior abdominal wall, then it must be
drained with a catheter. We like to use a Pezzer or a
pigtail catheter that is exteriorized through the
abdominal wall (Fig. 5.20). The pigtail is helpful
because over the next several weeks, all the swell-
ing of the vagina decreases, the vagina then tends to
move away from the abdominal wall, and the cath-
eters frequently come out. The “pigtail” catheter
60
Fig. 5.20 Catheter drainage of a newborn with
hydrocolpos
Fig. 5.21 Subumbilical midline incision used in cases of
large bilateral hydrocolpos. The stomas are separated, and
there is no incision in between them
will remain in place. In order to drain the hydrocol-
pos at the same time of the colostomy opening, it is
necessary to open the abdomen with an incision
that allows us to do both things (colostomy opening
and drainage of a hydrocolpos). In these cases, we
recommend a midline subumbilical incision
(Fig. 5.21). This incision provides an excellent
exposure to the pelvic anatomy and allows the sur-
geon to perform the diversion of the hydrocolpos.
5 Colostomy
For the colostomy, we recommend creating two
separate orifices, one for each stoma, in the abdom-
inal wall, one in the left flank and the other one
located lower and medial; both orifices are sepa-
rated enough, to be able to adapt a stoma bag
covering only the proximal stoma. In this way,
there will be no incision in between both stomas,
but rather healthy, normal skin (Fig. 5.21). For this
kind of colostomy, the descending colon has to be
divided through the midline subumbilical incision.
5.10 Other Types of Colostomies
Sometimes the surgeons feel that the patient does
not need a totally diverting stoma. It may be the
case of a reoperation, during which the surgeon
feels very confident about the successful healing
of the sutured tissues; the colostomy in such case
is simply an extra precaution. We feel that in gen-
eral, in colorectal surgery, we must work feeling
free to open a colostomy when we think it is to the
benefit of the patient. That is one of the reasons
why, in general, we recommend the use of mid-
line abdominal incisions in pediatric patients with
colorectal problems. These types of incisions
allow preserving both upper and lower quadrants
of the abdomen in case the patient needs a stoma.
In cases in which the surgeon feels that the colos-
tomy does not necessarily need to be totally
diverting, we recommend opening an orifice in
the selected quadrant, resecting skin, subcutane-
ous tissue aponeurosis muscle, and peritoneum.
Through that orifice, we exteriorize the selected
piece of colon. We divide the bowel outside the
skin and taper the distal end to create a little,
3-mm-diameter stoma, mucous fistula attached
next to the proximal stoma (Fig. 5.22). This is a
kind of a loop colostomy but with a reduced size
mucous fistula lumen, which reduces the chances
of stool spillage into the distal part.
5.11 Colostomy Care
The postoperative care of the colostomy is easy
when the colostomy was made technically cor-
rect. Colostomies done in a technically incorrect
manner represent a challenge and a nightmare for
5.12 Colostomy Closure
a b
Fig. 5.22 Stoma with a very small mucous fistula, placed together. (a) Diagram. (b) Photograph
Fig. 5.23 Defective colostomy. Unable to adapt a stoma
bag
stoma therapists, nurses, and mothers. It is almost
impossible to adapt a stoma bag in a case of
stoma that has irregularities in the surrounding
skin (Fig. 5.23). When the stoma is surrounded
by normal skin, we usually use benzoin to cover
the skin that will be in contact with the appliance.
The orifice in the stoma bag is tailored according
to the size of the patient’s stoma. All this is done
by us in the operating room. We believe the sur-
geon should pay a lot of attention to the feedback
provided by mothers, nurses, and stoma thera-
pists, concerning the quality of stomas. It is our
impression that surgeons do not pay enough
attention to these details.
61
5.12 Colostomy Closure
As soon as the patient recovers from the main
repair and the parents are passing a dilator of a
size adequate for the patient’s age, the presence of
the colostomy is no longer necessary, and there-
fore, it can be closed. Leaving the colostomy open
longer than necessary exposes the patient to the
formation of a microcolon distal to the colostomy,
as a consequence of the lack of use. That makes
the colostomy closure technically more demand-
ing. We have seen two extreme cases, in which the
colostomy closure was delayed about 10 years.
The distal microcolon never really grew after sev-
eral attempts at closing the colostomy. Most of the
times, however, the microcolon grows back to a
normal size, after the colostomy is closed.
In cases of extremely severe, grotesque size dis-
crepancy between both ends (proximal and distal)
of the colostomy, we recommend a couple of good
technical maneuvers that proved to be very useful:
A. End-to-side anastomosis
B. Lateral window diversion
(a) End-to-side anastomosis
In general, we recognize and recom-
mend an end-to-end anastomosis as an
ideal way to close a colostomy. However,
in cases of severe size discrepancy, an
end-to-side anastomosis has demon-
strated to be equally useful and safer
(Fig. 5.24).
(b) Lateral window diversion
In cases of extreme size discrepancy
(Fig. 5.24), in which the surgeons feel
62 5 Colostomy

Fig. 5.24 End-to-side colocolic anastomosis in cases of

severe size discrepancy. Lateral window diversion, located
proximal to the anastomosis, useful in cases of extreme
size discrepancy

insecure about the functional capacity of

the anastomosis and the distal bowel, we
have created a “lateral window” type of
vent located on the very dilated proximal
colon (proximal to the anastomosis)
(Fig. 5.24). In the following days and
weeks postoperatively, one can monitor
the amount of stool coming out through
the window and through the rectum. The
window can also be used as a communi-
cation to inject contrast material and
evaluate the function of the anastomosis
and growth of the distal bowel.
The preoperative preparation for colostomy
closure includes only irrigation of the proximal
stoma with saline solution. The distal stoma does
not have to be irrigated because that has been
clean from the time of the main procedure. In
cases of loop stomas, both ends need to be irri-
gated. Prior to the definitive colorectal reconstruc-
tion, only the distal stoma needs to be irrigated.
The patients are admitted to the hospital the
day before surgery and receive a normal break-
fast followed by clear fluids until midnight. The
proximal stoma is irrigated as many times as nec-
essary until the nurses believe that the fluid that
comes back is clear. We do not give GoLYTELY®1
to these patients because we do not need or expect
them to have a completely clean colon.
1
GoLYTELY® PEG 3350 236 g, sodium sulfate 22.74 g,
sodium bicarbonate 6.74 g, sodium chloride 5.86 g, and
potassium chloride 2.97 g (4,000 mL) [regular and pine-
apple flavor].
Fig. 5.25 Colostomy closure. Packing of the proximal
stoma
5.13 Surgical Technique
The patient is taken to the operating room. Under
general anesthesia, the abdominal wall is
washed, prepped, and draped in the usual fash-
ion. A packing gauze impregnated with Betadine
is placed in the proximal stoma (Fig. 5.25).
Multiple 5-0 silk sutures are placed at the muco-
cutaneous junction, including both stomas
(Fig. 5.26). These multiple silk sutures are used
to apply uniform traction to both stomas to facil-
itate the dissection. Usually, when the stomas are
not too distant from one another, we use a wedge
resection of both stomas with a piece of skin in
between (Fig. 5.27). A needle-tip cautery is used
to perform this elliptical incision. The incision is
done while applying uniform traction to both
stomas, and it goes through the skin, subcutane-
ous tissue, aponeurosis muscle, and peritoneum,
staying in our dissection, as close as possible to
the bowel wall, but without touching the bowel
wall itself (Fig. 5.28).
5.13 Surgical Technique
Once both stomas have been completely sepa-
rated from the abdominal wall, the packing gauze
is removed from the proximal one. We use Baby
Fig. 5.26 Multiple silk sutures are placed at the mucocu-
taneous junction of both stomas. Traction is applied
a b
Fig. 5.27 Wedge incision. (a) Diagram. (b, c) Intraoperative pictures
63
Allen clamps to resect the part of the bowel that
used to be attached to the abdominal wall
(Fig. 5.29).
By doing this, we use fresh portions of the
proximal and distal colon to perform an end-to-
end anastomosis with two layers of long-term
absorbable 6-0 sutures (Figs. 5.30 and 5.31). The
mesenteric defect is meticulously closed, also
with 6-0 long-term absorbable sutures. The peri-
toneal cavity is irrigated with saline solution. The
peritoneum and posterior fascia are closed
together, with a running, locked 4-0 long-term
absorbable suture. The anterior fascia of the
abdominal wall is closed with interrupted 5-0
long-term absorbable sutures. The same suture
material is used to close the subcutaneous tissue
and Scarpa fascia. The skin is closed with a sub-
cuticular 5-0 monofilament absorbable suture.
The wound is finally covered with flexible collo-
dion (Fig. 5.31).
64
At the beginning of the operation, the patient
receives intravenous metronidazole and a broad-
spectrum antibiotic. These medications will be
administered for 48 h postoperatively. At the end
of the operation, we do not insert a nasogastric
tube in the majority of our patients, but we keep
them fasting. The following day after surgery, if
the patient had no nausea or vomiting and the
abdomen is not distended, we start oral feedings.
The patient usually stays in the hospital 3–4 days.
Fig. 5.28 Both stomas are meticulously dissected and
separated from the abdominal wall
Fig. 5.29 Stomas are
resected to use a fresh
portion of the bowel on
each side to perform an
anastomosis. (a) Diagram.
(b) Picture
5 Colostomy
Occasionally, the patient has abdominal disten-
tion or vomits after the surgery; under those cir-
cumstances, we may insert a nasogastric tube and
keep the patient fasting until the ileus resolves.
Colostomy closures must be done using a deli-
cate and meticulous technique. This is an opera-
tion with serious potential complications [31, 32,
33–37].
We are very proud of our results in colostomy
closures [38]. We believe that a meticulous, deli-
cate technique explains our good results. We have
closed over 1,000 colostomies, and we have only
had one case of a dehiscence of the anastomosis.
That particular patient had a colostomy closed with
a single-layer anastomosis. The colostomy had to
be reopened on an emergency basis and closed a
month later with no problems. Another patient
came back to the hospital a week later, with a
colonic perforation located about 1 cm proximal to
the anastomosis. We do not have an explanation for
this complication; we are not sure if it may have
been a cautery burn done inadvertently. All of these
patients have been operated on without any drains
from the peritoneum or the subcutaneous tissue.
We put special emphasis in a meticulous hemosta-
5.14 Errors and Complications in Colostomies
Fig. 5.30 A two-layer
a
anastomosis with separated
stitches is performed, using
very fine (6-0) long-term
absorbable sutures. The
mesenteric defect is closed.
(a) Diagram. (b) Operative
picture
sis, closing each one of the layers of the abdominal
wall, leaving no dead spaces and irrigating each
plane. We never had a case of a wound infection,
despite all wounds being closed primarily.
5.14 Errors and Complications
in Colostomies
Over 1,500 patients came to us with a colostomy
created at another institution. As can be imagined,
we have seen literally all kinds of colostomies.
From that experience, we learned about the poten-
tial advantages and disadvantages, as well as
complications of each type [30]. The most com-
mon error seen by us in patients with anorectal
malformations, who underwent a colostomy
opening at another institution, consists in having
the stoma created too distal into the sigmoid colon
65
(Fig. 5.32) leaving a very short piece of bowel
between the distal stoma (mucous fistula) and the
end of the rectum (blind end or fistula site). This
is a serious mistake because it interferes with the
mobilization and pull-through of the rectum to
create a new anus. This is another reason why the
distal colostogram is so important. The first piece
of information that the surgeon must obtain from
this study is related to the length of bowel avail-
able for pull-through, distal to the mucous fistula
(Fig. 5.33). A colostomy located too distal must
be ruled out before embarking in a misadventur-
ous, failed attempted repair. The surgical alterna-
tives when confronted with that problem are:
A. Colostomy revision
Closing the colostomy and reopening a
more proximal one, exteriorized through the
same abdominal orifice, and doing the main
repair at least 3 months later (Fig. 5.34).
66 5 Colostomy

b c

Fig. 5.31 The bowel anastomosis finished and the wound is closed. (a) Diagram. (b) Operative field. (c) Closed wound

B. Repair the malformation, detaching the
bowel (mucous fistula) from the abdominal
wall, to allow its mobilization and leaving
the distal bowel closed as a Hartmann pouch
(Fig. 5.35).
C. Close the colostomy and perform the pull-
through at the same time.
• Leaving the patient without a protective
colostomy or
• Opening a new more proximal stoma
D. Resect the short piece of bowel located
between the mucous fistula and blind end of
the fistula, pulling through the colostomy
site bowel, leaving the patient without a
colostomy or opening a new one more
proximal.
Alternative A: Colostomy revision is probably
the safest one, although it represents an extra
operation for the patient.
Alternative B: Repair the malformation, leaving
the patient with a Hartmann pouch (Fig. 5.35),
may represent a future technical challenge,
depending on how low the pouch is located. It
is a technically demanding operation to close
a colostomy performing a bowel anastomosis
behind the bladder. In fact, if the upper end of
5.14 Errors and Complications in Colostomies
Fig. 5.32 Colostomy created too distal
Fig. 5.33 Distal colostogram showing a very short piece
of bowel distal to the stoma
the Hartmann pouch is located behind the pos-
terior urethra, the operation may be almost
impossible to perform, not to mention the risk
involved.
67
Fig. 5.34 Closure of a colostomy and opening of a more
proximal one, prior to the main repair, in a patient who
previously underwent a defective (too distal) colostomy
Fig. 5.35 Diagram showing a pull-through of a short dis-
tal colon. The mucous fistula had to be separated from the
abdominal wall. The distal stoma is closed (Hartmann
pouch). Alternatively, sometimes the distal stoma can be
created in a lower part of the abdomen
68
Alternative C: Closing the colostomy and doing
the pull-through, leaving the patient without a
colostomy, is feasible but involves a certain
degree of risk. We advise in such a case to
leave the patient fasting for 10 days, receiving
parenteral nutrition. In addition, to do this
kind of operation requires a total bowel
preoperative preparation.
Opening a new, more proximal colostomy
is of course safer but requires one more major
operation (colostomy closure).
Alternative D: (Resect the little, distal piece of
bowel) We consider this alternative formally
contraindicated. The most distal piece of
bowel represents the future rectum for the
patient. We have learned that the preservation
of this part of the intestine is extremely impor-
tant. Our observations in multiple patients
lead us to believe that the colon’s motility is
slower in its most distal part. In fact, the nor-
mal rectosigmoid acts mainly as a reservoir of
stool, except at the time of defecation, when
the rectosigmoid has a very active, massive
peristaltic wave that allows the emptying of its
entire contents that usually represents the
stool formed over a period of 12–48 h. In
between episodes of defecation, the rectum
remains virtually paralyzed (acting as a reser-
voir), receiving and storing stool. This is an
extremely important function that allows us,
human beings, to function socially, without
using the toilet constantly. The observation of
the way the different types of colostomies
pass stool represents a clear demonstration of
this. The more distal the colostomy, the longer
the periods without passing stool.
Elimination of the rectum from the fecal
stream results in an almost constant passing of
stool. This may be managed relatively well by an
otherwise normal individual in whom the anal
canal and sphincter mechanism are intact. Yet, in
patients with anorectal malformations, this is not
tolerated at all and may well represent the differ-
ence between bowel control and fecal inconti-
nence. In other words, it is necessary to have an
intact anal canal (sensation and sphincter mecha-
nism) in order to maintain bowel control with an
absent rectosigmoid.
5 Colostomy
In patients with typical Hirschsprung’s dis-
ease, we resect the aganglionic rectosigmoid and
anastomose the descending normoganglionic
colon to the anal canal, and patients have bowel
control, provided the anal canal is preserved
intact. Patients with anorectal malformations are
born without an anal canal, and their sphincter
mechanism is represented by a spectrum that
includes cases with almost normal sphincter (in
one extreme of the spectrum) to patients with
absent sphincter (in the other extreme of the
spectrum). Many patients operated from an ano-
rectal malformation behave as if they were fecally
continent; yet, they cannot tolerate sudden
changes in the consistency of the stool or sudden
peristaltic waves. For this reason, we insist that it
is extremely important to try to preserve to the
best of our capacity as much bowel as possible.
We know that the rectosigmoid in patients
with anorectal malformations suffers from hypo-
motility, which is reflected in a marked tendency
to constipation. Resection of the rectum may
decrease the severity of the constipation problem,
but may also provoke tendency to diarrhea,
which, as we mentioned, will turn into inconti-
nent a patient with borderline bowel control.
We have a large experience with patients that
have come to our clinic to receive bowel manage-
ment for the treatment of fecal incontinence. In
some of them, the surgeons found it easier to sim-
ply remove the distal short rectum and pull the
colostomy down. Those patients always become
incontinent even in cases born with a good func-
tional prognosis type of defect.
In addition, as mentioned in the chapter of
bowel management, that group of patients
(hypermotility, tendency to diarrhea) is much
more difficult to manage.
5.15 The Case of Upper
Sigmoidostomy
An interesting error occurs when the surgeon
tries to open a transverse colostomy (either right
or left sided) and actually creates what we have
called an “upper sigmoidostomy” (Fig. 5.36).
This occurs because the surgeon (frequently in a
5.15 The Case of Upper Sigmoidostomy 69

a b

Fig. 5.36 Upper sigmoidostomy. The surgeon thought that he was doing a transverse colostomy, but actually he cre-
ated a sigmoidostomy in the upper abdomen, which will interfere with the pull-through. (a) Diagram. (b) Colostogram

hurry) creates a right or left upper quadrant inci-
sion and grabs the first visible segment of the
colon, erroneously assuming that it is either a
right or left transverse colon. One must always
keep in mind that in cases of anorectal malforma-
tions, the sigmoid colon is very dilated and
redundant, reaching the upper abdomen. The sur-
geon must take the time to observe carefully the
characteristics of the piece of colon that he
selected, to be sure that that is the correct portion
of the colon.
The negative implications and inconveniences
of this type of colostomy (upper sigmoidostomy)
are obvious. The attached (tethered) sigmoid to
the abdominal wall will interfere with the
pull-through.
Again, we cannot overemphasize the impor-
tance of the distal colostogram in the planning of
the main repair of an anorectal malformation.
The location of the stoma in the abdominal wall
does not necessarily correspond to the portion of
the colon employed. In other words, a right upper
quadrant stoma does not necessarily mean that
the portion of the colon employed is the right
transverse. Only with a distal colostogram one
can objectively determine the characteristics of
the colostomy.
Complications in colostomies are divided into
immediate and late. Immediate complications
include dehiscence of the stoma, retraction, and
infection. These three complications usually
occur together. This represents a catastrophe
usually related to a poor technique, a colostomy
opened in a very sick patient, or both. A tense
anastomosis between the bowel and the
abdominal wall, plus a devascularization of the
bowel, may explain the retraction and dehis-
cence. A poor surgical technique with severe
contamination may explain the infection [30].
Late complications include
Parastomal hernia: This is also a technical prob-
lem that is avoidable by using a meticulous surgi-
cal technique.
70 5 Colostomy

5.16 Prolapse (Fig. 5.38). A sigmoid colostomy will have a high

tendency to prolapse, unless it is done in the way
Prolapse is one of the most common complications that we recommend, in which the proximal stoma
that we have seen in colostomies done at other is opened in the descending fixed portion of the
institutions [30]. There are some publications with colon and the mucous fistula is tapered to create a
recommendations to prevent prolapse from hap- very small stoma (Fig. 5.39).
pening [39–41]. There is a merit on those recom-
mendations. However, we think that we found the
most important factor that contributes to the occur-
rence of prolapse. It took us several years to under- Left transverse
stand the mechanism of prolapse. By observing all
No prolapse
of the patients that had prolapse, we finally con-
Likely to
cluded that prolapse occurs every time the colos-
prolapse
tomy is opened in a mobile portion of the colon. In
retrospect, this sounds like an extremely simplistic,
yet very valuable conclusion. In a case of a two

Fixed
stoma type of colostomy opened into a mobile por-
tion of the colon, we would expect both stomas to
Mobile
prolapse. Otherwise, if the proximal stoma was
opened in a fixed portion of the colon, like in the
right transverse colostomy, we would expect the
prolapse to occur in the distal stoma (Fig. 5.37).
The proximal will not prolapse because it is opened
into a fixed portion of the colon (hepatic flexure).
In a case with a left transverse colostomy, the prox-
imal one is expected to prolapse and not the distal

Fig. 5.38 Left transverse colostomy

Right transverse
Descending
Likely to
No prolapse prolapse
Fixed
Fixed

Mobile
Mobile
No
prolapse

Likely to
prolapse

Fig. 5.37 Understanding the etiology of prolapse. Right

transverse colostomy Fig. 5.39 Descending colostomy
5.17 Surgical Treatment for Prolapse 71

Fig. 5.40 Severe stoma prolapse Fig. 5.41 Packing gauze inserted in the prolapsing
stoma, reducing the prolapse
In general, if a surgeon has to open a colostomy
and has no choice but to open it in a mobile portion
of the colon, we recommend affixing that piece of
bowel to the anterior abdominal wall for approxi-
mately 8 cm, proximal to the stoma with nonab-
sorbable sutures. Some patients that came to us
with a severe prolapse were supposed to have a
repair of the anorectal malformation, but rather
than doing that, we decided to take care of the pro-
lapse. Severe prolapse (Fig. 5.40) frequently pro-
duces ischemia of the most distal part of the
prolapsed bowel with serious consequences and
must be avoided. We have seen patients that suf-
fered from prolapse, the parents took the baby to a
hospital, and the surgeons decided simply to Fig. 5.42 The prolapse is reduced, taking its natural
amputate the prolapsed part of the colon. This has position in the abdomen
very serious consequences for the patient, because
the absence of the colon or the presence of a short
colon, in a patient with an anorectal malformation, ment that we propose for the management of pro-
may result in incapacity to form solid stool which lapse is illustrated in diagrams 41–44. Under
will produce fecal incontinence, even in cases of general anesthesia, the prolapsed stoma is packed
patients born with a good functional prognosis with packing gauze impregnated with Betadine
type of anorectal malformation. In addition, as (Fig. 5.41). By doing this, we reduce the pro-
previously mentioned, the management of fecally lapsed bowel and let the bowel take its natural,
incontinent patients with tendency to diarrhea is comfortable position inside the abdomen
more difficult, and the results of the implementa- (Fig. 5.42). Once we finish packing the stoma, we
tion of our bowel management program are not as palpate the abdomen around the stoma. It is very
good as the ones in constipated patients. easy to feel a sausage-like mass, situated some-
where around the stoma (Fig. 5.43). We then
make a 4–5-cm incision, away from the stoma, in
5.17 Surgical Treatment the area where the “sausage” is palpated. The
for Prolapse incision must be located far enough from the
stoma, as to be sure that after the operation the
Several authors published ingenious procedures stoma bag can be placed on a smooth piece of
to treat colostomy prolapse [42–45]. We do not skin and not on top of the incision (Fig. 5.44).
have experience with those methods. The treat- Once we open the abdominal wall, we can easily
72
Fig. 5.43 A “sausage-
like” mass is easily
palpable, which represents
the reduced prolapse
Fig. 5.44 Incision made on the area of the palpable
“sausage-like” mass and away from the stoma. The abdo-
men is entered and the prolapsed bowel is easily identi-
fied. While closing the peritoneum and fascia, the stitches
take the bowel wall
see the dilated colon that used to be prolapsed
and now is full of the packing gauze (sausage)
(Fig. 5.44). We start by closing the peritoneum
and posterior fascia, including in our stitches a
bite of the colonic wall (“sausage”) that used to
be prolapsed. We finish by closing that incision
and removing the packing gauze. The bowel will
not prolapse again. We have seen one case of
recurrence, but we have done at least 25 of these
5 Colostomy
cases this way, with no recurrence. We have seen
no case of a cutaneous fistula related to this
suturing.
5.18 Malposition of the Stomas
We have seen stomas incorrectly located in
places near the umbilicus, near the ribs, near
the iliac bone, or near the pubis. Every time the
patient moves, the stoma bag detaches, and
there is stool leakage that is embarrassing and
causes a lot of skin problem. This is why we
emphasize that the functional stoma should be
opened in a location surrounded by normal
skin, and that is why we also emphasize the use
of midline incisions in patients with potential
colorectal problems.
The stomas located too close, one to another
(Fig. 5.2a), represent a problem because the nurses
and mothers cannot use a stoma bag to include
only the proximal stoma. They have to include
both stomas into the same bag, which means
potential passage of stool into the distal bowel that
may provoke urinary tract infections and/or fecal
impaction distally. Stomas that are located too far
one from the other (Fig. 5.45) represent a problem
because at the time of colostomy closure, the
patient will need a very long incision in order to
bring both stomas together.
5.18 Malposition of the Stomas 73

Inverted stomas represent a lack of care and

attention from the surgeon at the time of the oper-
ation. He or she thought that he or she was deal-
ing with a proximal stoma that actually was distal
and vice versa, and the bowel was twisted
(Fig. 5.46).
Stricture usually occurs secondary to isch-
emia. That means that the bowel was perhaps
squeezed when the surgeon closed the abdominal
wall. Sometimes, the bowel was not properly
mobilized, or the surgeon damaged the blood
supply, provoking a stricture that requires a revi-
sion, or the fascial opening was made too small.
A stricture may happen in the proximal stoma or
may also happen in the mucous fistula. Closure
of a mucous fistula (Hartmann pouch) represents
a risk of mucocele and has to be reopened. In
addition, we cannot do a high-pressure distal
colostogram, and that is another reason why it
should be reopened.

Fig. 5.45 Stomas located too far apart

a b

Fig. 5.46 Inverted stomas. (a) Diagram. (b) Picture

74 5 Colostomy

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AC, Kale N (2011) Ostomy complications in patients
Colostomies in infancy and childhood. Arch Dis
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27(10):1075–1078. doi:10.1007/s00383-011-2955-8
11. Cain WS, Kiesewetter WB (1965) Infant colostomy:
28. van den Hondel D, Sloots C, Meeussen C, Wijnen R
its role and complications. Arch Surg 91(2):314–320.
(2014) To split or not to split: colostomy complications
doi:10.1001/archsurg.1965.01320140104014
for anorectal malformations or Hirschsprung disease:
12. Hartford CE, Soper RT (1967) Complications of
a single center experience and a systematic review
colostomy in infants and young children. J Iowa Med
of the literature. Eur J Pediatr Surg 24(1):61–69.
Soc 57(3):229–232
doi:10.1055/s-0033-1351663
13. Yajko RD, Norton LW, Bloemendal L, Eiseman B
29. Oda O, Davies D, Colapinto K, Gerstle JT (2014)
(1976) Morbidity of colostomy closure. Am J Surg
Loop versus divided colostomy for the management
132(3):304–306
of anorectal malformations. J Pediatr Surg 49(1):87–
14. Fekete CN (1979) Les colostomies chez l’enfant. Med
90. doi:10.1016/j.jpedsurg.2013.09.032
Chir Dig 8:711–712
30. Peña A, Migotto-Krieger M, Levitt MA (2006)
15. Mollitt DL, Malangoni MA, Ballantine TV, Grosfeld
Colostomy in anorectal malformations: a procedure
JL (1980) Colostomy complications in children. An
with serious but preventable complications. J Pediatr
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16. Rees BI, Thomas DF, Negam M (1982) Colostomies
31. Tank ES, Watts H (1968) Hyperchloremic acido-
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17. Lister J, Webster PJ, Mirza S (1983) Colostomy com-
Surgery 63(5):837–839
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32. Iwai N, Ogita S, Shirasaka S, Yamamoto M, Majima
18. Perdzyński W, Zarzycka E, Zmijewski Z, Kalicki B
S (1978) Hyperchloremic acidosis in an infant with
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34. Miyano G, Okawada M, Yanai T, Okazaki T, Lane GJ,
Yamataka A (2009) Outcome of stoma closure in chil-
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ventional open techniques. J Laparoendosc Adv Surg
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35. Finch DR (1976) The results of colostomy closure. Br
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36. Rickwood AM, Hemalatha V, Brooman P (1979)
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37. Yadav PS, Choudhury SR, Grover JK, Gupta A,
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38. Bischoff A, Levitt MA, Lawal TA, Peña A (2010)
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39. Lau JT (1982) Proximal end transverse colostomy
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40. Ein S (1984) Divided loop colostomy that does not
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s00383-009-2500-1
 Imaging
6

6.1 Introduction 6.3 Neonatal Imaging

Advances in imaging technology have been
extremely beneficial for the management of
patients with anorectal malformations. Every
day, we learn about technologic innovations that
frequently surpass our imagination. Concurrently
with these, the surgeons have to speculate less
and less, like in the past [1], and have the privi-
lege of making very precise anatomic diagnosis
that allows the planning of a complex reconstruc-
tion in a very accurate manner.
In dealing with the spectrum of anorectal mal-
formations, we depend very much on the images
obtained by traditional x-rays, ultrasound, CAT
scan, and magnetic resonance imaging (MRI).
We use all of these images not only in the early
stages of our management but actually through
the entire life of the patient, since each stage of
life of the patient brings new therapeutic chal-
lenges that require an accurate diagnosis.
The first 24 h of life, before making the decision
to open a colostomy or to perform a primary
repair, represents a window of opportunity to
diagnose potential associated defects. The chest
film taken during the first 24 h of life also allows
us to see the integrity of the thoracic vertebra and
ribs. In addition, it helps us in the diagnosis of
esophageal atresia and potential cardiac malfor-
mations (Fig. 6.1).
The abdominal x-ray film allows us to see
and rule out the possibility of hemivertebra
(Fig. 6.2). The early detection of these types of

6.2 Prenatal Diagnosis

Every year, more and more, the pediatric surgeon

is asked to participate in the diagnostic discus-
sions of babies in utero that have congenital mal-
formations. The reader is invited to read Chap. 3.

Electronic supplementary material Supplementary Fig. 6.1 Chest film of a child with anorectal malforma-
material is available in the online version of this chapter at tion associated to thoracic hemivertebrae and esophageal
10.1007/978-3-319-14989-9_6. atresia

A. Peña, A. Bischoff, Surgical Treatment of Colorectal Problems in Children, 77

DOI 10.1007/978-3-319-14989-9_6, © Springer International Publishing Switzerland 2015
78
Fig. 6.2 Abdominal film showing a hemivertebrae
malformations is relevant to establish the func-
tional prognosis in these babies. The abdomi-
nal film must include an AP view of the sacrum
(Fig. 6.3). It is also important to take a lateral
abdominal film that allows a more accurate mea-
surement of the sacral ratio (Figs. 6.3 and 6.4).
Traditionally, the number of sacral vertebrae is
counted to evaluate the quality of the sacrum. Most
pediatric surgeons agree that when a patient has
less than three sacral vertebrae, the prognosis for
bowel and urinary control is not good. Many others
while discussing the subject of the sacrum use
rather nondescriptive terms such as “dysplastic” or
“hypoplastic.” There is no question that the pres-
ence of the sacrum as well as its integrity is crucial
to determine the functional prognosis of the patient.
We found the terms “dysplastic” or “hypo-
plastic” very inaccurate. In addition, we found
that there are cases with five sacral vertebrae, yet
very abnormal ones, which results in an extremely
short sacrum associated to fecal and sometimes
urinary incontinence. We thought that it was nec-
essary to create a more objective way to evaluate
6 Imaging
the sacrum, in an effort to determine the functional
prognosis of the baby. The result was the creation
of the “sacral ratio” (Fig. 4.5 in Chap. 4), [2, 3].
The sacral ratio results from comparing the
vertical length of the sacrum with the size of the
pelvis of the same patient.
A. A line is drawn between the most upper por-
tions of the iliac bone in an AP film of the
sacrum.
B. Another line is drawn between both the inferior
and posterior iliac spines (Fig. 4.5 in Chap. 4).
C. A third line is drawn, parallel to the first two
lines, touching the lowest radiologically visi-
ble point of the sacrum or coccyx.
The distance between lines A and B is
measured, as well as the distance between lines
B and C. A ratio is created: AB/BC.
We measured this ratio in 100 normal children
who had abdominal films taken for other reasons
and found that the average ratio in anterior-poste-
rior films was 0.76 and 0.77 in lateral films [2].
Patients with anorectal malformations frequently
suffer from different degrees and types of sacral
abnormalities. The sacral abnormalities present
like a spectrum with ratios similar to normal chil-
dren, in what we call the good side of the spec-
trum. However, in the “bad” extreme of the
spectrum, we see patients with a sacral ratio of 0.
We found that it is extremely unusual for a
patient with anorectal malformation and with a
ratio lower than 0.4 to have bowel control. To have
a normal sacral ratio is a good prognostic sign, but
it does not mean that the patient will necessary
have bowel control, since there are other factors
that influence the final functional results.
One of the relatively common associated
defects in children with anorectal malformations
are defects of the radial bone (Fig. 6.5).
Sometimes, the defect in the forearms and the
hands is very obvious (Fig. 6.5a). Other times,
one can see only a slight radial deviation of the
hand that would make us suspect this defect.
An ultrasound study is also part of the group of
imaging studies that must be done during the first
24 h of life before the baby suffers from abdomi-
nal distention. The kidney ultrasound is perhaps
the most important part of the evaluation of this
baby since about 50 % of them, globally, have
6.3 Neonatal Imaging 79

a b

Fig. 6.3 AP film of sacrum in a child with anorectal malformation. (a) Normal sacrum. (b) Short sacrum. (c) Severely
deficient. (d) Caudal regression. (e) Hemisacrum
80
some sort of urologic-associated condition. We
specifically look for hydronephrosis (Fig. 6.6).
One of the most common urologic anatomic
Fig. 6.4 Lateral film of sacrum
a b
Fig. 6.5 Absent radial bone – a frequently associated defect. (a) External appearance. (b) Radiologic appearance
6 Imaging
defects is absent or multicystic kidney (Fig. 6.7).
The ultrasound must include the rest of the abdo-
men looking for the presence of megaureters and
the bladder. This is particularly useful and impor-
tant in female babies with a single perineal orifice
(cloaca). In these babies, we will specifically look
for the presence of a cystic structure located
behind the bladder (hydrocolpos). Frequently, this
is a double cystic structure since about 30 % of
the babies with cloaca have two hemivaginas
(Fig. 6.8).
During these first hours of life, it is extremely
useful to take an ultrasound of the lumbosacral
spine trying to see the conus medullaris to rule out
the presence of tethered cord (Fig. 6.9). The pres-
ence of a tethered cord represents a negative factor
in terms of prognosis for urinary control and to
some degree, although not clear, for bowel control.
It is a well-known fact that if the ultrasound of the
spine is not done during the first 3 months of life,
after that time, it is no longer a reliable study for
the diagnosis of tethered cord, due to the ossifica-
tion of the spine, and at that point, the diagnosis of
tethered cord can only be done reliably with an
6.3 Neonatal Imaging 81

a b

Fig. 6.6 Neonatal ultrasound. (a) Normal. (b) Hydronephrosis

a b

Fig. 6.7 Ultrasound. (a) Absent kidney. (b) Multicystic kidney

MRI study which requires heavy sedation or gen-
eral anesthesia in babies (Fig. 6.9c).
Some surgeons routinely perform a voiding cys-
tourethrogram in male babies with anorectal mal-
formations. We do not believe this routine is
necessary. When the baby has normal kidneys by
ultrasound, no evidence of megaureters, and is
passing urine normally, we do not see the relevance
of the voiding cystourethrogram. Over 80 % of the
male patients with anorectal malformations have a
connection between the rectum and the urinary
tract (fistula), and at the location of the fistula,
sometimes there is a kink of the urethra that inter-
feres with the passing of a catheter. Rough manipu-
lations of this baby’s urethra in the radiology
department might have negative consequences;
sometimes they fall into urinary retention as a con-
sequence of injuries provoked by a failed attempt
to pass a catheter. When the baby has hydronephro-
sis and megaureter, that is when we consider an
indication for a voiding cystourethrogram. Other
surgeons believe that the voiding cystourethrogram
would allow them to determine the size and loca-
tion of the rectourethral fistula. We considered a
voiding cystourethrogram a non-reliable study for
the diagnosis of the fistula location. Most of the
time that study does not show the fistula.
Occasionally, one can see a kink of the urethra that
“suggests” where the fistula is located but certainly
is not considered a reliable study (see Fig. 6.10).
82 6 Imaging

a b

V V R

V
K V
K

c d

Fig. 6.8 Neonatal hydrocolpos in a newborn baby with hydronephrosis. (a) Ultrasound. V vagina, K kidney. (b) Abdominal
film. V vagina, R rectum. (c) MRI – transverse section. (d) Abdominal film with contrast. K kidney, U ureter

6.4 Determination of the Fistula
Location Prior
to the Colostomy
6.4.1 Anatomic Facts and Timing
To understand the rationality of the imaging during
the neonatal period to determine the location of the
rectum and the fistula, it is extremely important for
the clinician and the radiologist to understand the
anatomy of the pelvis of babies with anorectal mal-
formations, this is illustrated in Animation 6.1.
The sphincter mechanism in babies with
anorectal malformations is represented by a
wide spectrum that goes from almost normal
striated sphincter mechanism to almost absent
sphincters.
The sphincter mechanism in normal individu-
als is represented by a funnel-like voluntary mus-
cle structure, the upper limits of that funnel being
the pubococcygeal line (Fig. 6.11). That funnel-
like muscle mechanism is a continuum of a stri-
ated muscle that runs all the way down to the skin
of the perineum. The upper part of the funnel-like
mechanism inserts in the pubic bone and sur-
rounds the rectum. The contraction of those fibers
compresses the rectum from behind. During sur-
gical explorations, there is no way to identify
6.4 Determination of the Fistula Location Prior to the Colostomy 83

a c

b d

Fig. 6.9 Spinal ultrasound. (a) Normal location of the conus. (b) Tethered cord, ultrasound image. (c) Normal, MR
image. (d) Tethered cord, MRI image

separated portions of that muscle that has been

Fig. 6.10 VCUG showing a kink in the urethra suggest-
ing the fistula location
referred to as “levator mechanism,” “puborectalis
muscle,” “ischiococcygeal muscle,” “puboure-
thralis muscle”; one rather sees only a continuum
of musculature.
In cases of anorectal malformations, the rec-
tum is passing through this funnel-like muscle
mechanism and stops at different heights. In
cases of perineal fistulas, for example, most of
the rectum is passing through this muscle mecha-
nism and is only anteriorly deviated in the lowest
portion (Fig. 6.12). In rectourethral fistulas, most
of the rectum also passes through this funnel and
ends into the upper part of the posterior urethra
(prostatic fistula) or into the lowest portion of the
posterior urethra (rectourethral bulbar fistula)
84
a b
Fig. 6.11 Funnel-like normal sphincter mechanism. (a) Relaxed. (b) Contracted
Fig. 6.12 Diagram of a perineal fistula. Most of the rec-
tum is surrounded by the funnel-like sphincter
mechanism
(Fig. 6.13). In cases of recto-bladder neck fistula
which represents the highest of all defects in
male patients, the rectum opens in the bladder
neck and is not surrounded by this sphincter
6 Imaging
mechanism (Fig. 6.14). Strictly speaking and
using the old terminology, this particular defect is
the only one that we can call “supralevator
malformation.”
The funnel muscle mechanism has, as
expected, a muscle tone that keeps the rectum
collapsed. This muscle only relaxes in normal
individuals during the evacuation of feces. If
one takes an abdominal x-ray film of a normal
newborn with no anorectal malformation, it
would be easy to see that the gas in the rectum
stops at the level of the pubococcygeal line
(which is the upper limit of the funnel-like
sphincter mechanism). From there down to the
skin, the rectum remains collapsed due to the
tone of the muscle that surrounds it. In cases of
anorectal malformations, if one takes an
abdominal x-ray film during the first few hours
of life, we will never find the gas of the rectum
located below the pubococcygeal line and cer-
tainly that does not mean that the baby has a
“very high malformation” since most likely
(90 % chance), the rectum is located below the
pubococcygeal line but is compressed by the
sphincter mechanism. Interestingly, in our lit-
erature review, we only found one author [4]
6.4 Determination of the Fistula Location Prior to the Colostomy
a b
Fig. 6.13 Diagram showing a rectourethral fistula. (a) Prostatic. (b) Bulbar
Fig. 6.14 Diagram showing recto-bladder neck fistula.
The bowel is not surrounded by sphincter muscle
who suggested that the contraction of the
“puborectalis muscle” must be taken into con-
sideration to interpret radiologic studies in the
newborn.
85
We are convinced that:
Diagnostic Imaging Studies performed during the
first few hours of life are not reliable to determine
the real location of the rectum
The diagnostic challenge during the newborn
stage, in patients with anorectal malformations
prior to the opening of a colostomy, is not related
so much to the quality and sophistication of the
imaging technology used, but rather to the knowl-
edge of the anatomy and physiology of the rec-
tum and the surrounding sphincter in patients
with anorectal malformations during the first few
hours of life. That is the reason why we recom-
mend not doing diagnostic studies trying to
determine the location of the rectum during the
first 24 h of life. We have learned that babies with
anorectal malformations usually are not born
with abdominal distention. They rather become
distended after 20 or 24 h of life. This abdominal
distention represents, as expected, an increase of
the intraluminal pressure of the bowel, and at
some point, that pressure overcomes the muscle
tone of the funnel mechanism that surrounds the
rectum, and then one can see the real location of
the gas inside the rectum (Fig. 6.15).
We have been exposed to patients that are
referred to us after failed attempted repairs. Some
86
a
b
Fig. 6.15 Cross-table lateral film in a newborn baby
with imperforate anus. (a) Six hours old. (b) Twenty-four
hours old
of those babies were subjected to diagnostic imag-
ing studies during the first few hours of life that
led the surgeons to erroneously conclude that the
baby had a “high imperforate anus.” As a conse-
quence, the surgeons made one of two decisions,
either to open a colostomy (that was not indicated)
or even worse, to perform an abdominoperineal
procedure in a baby that had, for instance, an
unnoticed perineal fistula. Some of those patients
that had a non-indicated colostomy subsequently
have received a distal colostogram without enough
hydrostatic pressure, which induced the surgeons
“to confirm” the diagnosis of “high imperforate
anus.” Again, the distal colostogram was done
6 Imaging
without the necessary pressure to overcome the
muscle tone of the funnel mechanism, and the sur-
geon made a wrong diagnosis.
6.5 The Old Invertogram
The famous prominent professor of surgery Dr.
Wangesteen and Dr. Rice, a radiologist, pub-
lished a seminal paper [5] that represents the
beginning of the era of the radiologic evaluation
of patients with anorectal malformations. The
rationale behind that study was to put the new-
born baby upside-down, to wait for a few min-
utes, and to assume that by gravity, the gas inside
the bowel would reach the most distal part of the
rectum. The gas would then act like a contrast, a
simple lateral film of the pelvis with an anal
marker was taken and the distance from the anal
marker to the bubble of gas would allow the sur-
geon to classify the malformation into a “low
malformation” (when the distance was shorter
than 1 cm) or “high malformation” (when it was
longer than one centimeter). Traditionally, the
“low malformations” were surgically approached
through the perineum and the “high malforma-
tions” were operated abdominoperineally. Now
we know that when that kind of film is taken after
24 h, it certainly may show an image considered
representative of the location of the rectum, but
when the study is performed too early in life, it is
not reliable.
Later on, we learned about the “inherent errors
and disadvantages of the invertogram” [6, 7]. In
addition, we have learned that one can obtain
exactly the same image of the invertogram by
placing the patient in prone position with the pel-
vis elevated (Fig. 6.15) [8].
We were able to compare the two images
obtained with the invertogram and with this
cross-table lateral film and found that it is exactly
the same.
The cross-table lateral film has the great
advantage of avoiding the positioning of the baby
upside-down with the risk of vomiting and
aspiration.
Furthermore, we have learned that with a good
index of suspicion and looking at the perineum of
6.6 High-Pressure Distal Colostogram 87

the baby carefully, we actually need this kind of

film (cross-table lateral film) only in less than
5 % of our patients. Most of the times, we obtain
enough clinical information to make a good ther-
apeutic decision without this study (see Chap. 4).
Some authors are very enthusiastic about the
use of perineal ultrasound in neonates to deter-
mine the location of the rectum [9–14]. Others
use to recommend the injection of contrast mate-
rial through the perineum [15, 16] or through the
perineal fistula [17]. We feel that these studies are
very much dependent on the degree of experience
of the radiologist; the images are not easy to
interpret for us surgeons.
The CT scan has also been used to determine
the location of the rectum, in order to plan the
best possible surgical approach [18–21].
Unfortunately, those studies show only trans-
verse section images, and the sagittal reconstruc- Fig. 6.16 Long narrow fistula. Contrast injected through
tions show poor-quality images. a perineal fistula. Gives the false impression of a “high”
The MRI technology obviously represents a anorectal malformation
great advancement that contributes enormously to
the anatomic diagnosis of multiple conditions. We
use these kinds of studies to evaluate the anatomy consideration the anatomic facts already dis-
in patients already operated on. Some authors sug- cussed. Otherwise, those studies are not consid-
gest doing MRI studies in newborns with anorec- ered reliable.
tal malformations [22, 23]. Even when the images When the baby has a tiny orifice in the
are very good, we consider the study logistically perineum (perineal fistula), some surgeons pass a
demanding, expensive, sometimes risky for the fine catheter through the fistula and inject con-
baby (anesthesia), and not indispensable. trast material. They find frequently a long narrow
More important is the fact that none of the tract with a dilated rectum located up in the pel-
authors that we reviewed mentioned what we vis; based on that, they may think that the baby
consider is the most important aspect of the neo- has a “high” malformation (Fig. 6.16). However,
natal diagnosis, which is the timing of the studies that is not a reliable study because what they con-
and its relationship with the anatomy and physi- sider a long narrow fistula may be just a conse-
ology of the rectum and surrounding sphincters quence of the compression of a normal-caliber
in the newborn. rectum given by the surrounding muscle mecha-
There are multiple papers that recommend nism, and the rectum is actually located very low
other imaging studies (before the colostomy’s in the pelvis (Fig. 6.16).
opening) to try to determine the location of the
rectum. None of them discuss the anatomic facts
presented here. Some authors claim that an MRI 6.6 High-Pressure Distal
is good enough [3]; others believe it is the CT Colostogram
scan [3]. Finally, some authors propose perineal
ultrasound [5], and others prefer the injection of After the colostomy has been opened, the sur-
contrast material through the perineum [6]. If a geon must plan the best surgical strategy to repair
specific doctor or hospital uses this kind of tech- the anorectal malformation. We have found
nology for this diagnosis, they should take in through the years that this study is by far the most
88 6 Imaging

important, valuable, and accurate diagnostic test

that we can do in cases of anorectal malformation
(3D Animations 6.2, 6.3, and 6.4 illustrate this
study). Unfortunately, there are not many publi-
cations advocating this study [24–28], and many
radiologists are not familiar with this study and
the way to do it. We believe that we cannot over-
emphasize the value of this diagnostic test and
the technical details of its performance.
Some authors still believe that a voiding cys-
tourethrogram is good enough to demonstrate the
location of the rectourethral fistula [29]. We do
not agree because we have seen many false nega-
tives. Attempts have been made to combine distal
colostogram with an MRI [30, 31]; there is no
question that the images are good, but the study is
expensive and logistically difficult.

6.7 Technique

This study is only feasible in patients that already

have a colostomy. The baby is taken to the radiol-
ogy suite and a no. 8 Foley catheter is introduced
through the mucous fistula of the colostomy.
Many babies, unfortunately, are subjected to a
loop type of colostomy, and this makes it diffi-
cult, sometimes for the radiologist, to find in
which direction the catheter should be introduced
to be sure that the contrast material is injected in
the distal bowel. The mother usually knows
which one is the orifice that produces stool and
the one that is just a mucous fistula. The catheter
is inserted about 5 cm, and the balloon is inflated
with 2 mL of air or water. Traction is applied on
the catheter to be sure that the balloon impacts on
the stoma serving as a plug that will prevent the
contrast from leaking out, even after we apply
hydrostatic pressure during the injection. We
only use water-soluble contrast material. The
injection of the contrast is done using a 60 mL
syringe with a catheter tip connected to the Foley
catheter and is done by hand. Under fluoroscopic
control, with the baby in supine position, the
injection starts and continues until the contrast
seems to reach the most distal part of the bowel.
At this point, we obtain very valuable informa-
tion related with the length of bowel that the baby
Fig. 6.17 Distal colostogram showing a very short piece
of bowel distal to the colostomy pull-through is not
feasible
has available for the pull-through, from the colos-
tomy site to the most distal end or fistula site.
This is extremely important, since the most com-
mon error that we have seen in the location of the
colostomies is a too-distal colostomy, meaning
that the surgeon left a really short piece of bowel
beyond the colostomy site, which will certainly
interfere with the pull-through. Trying to repair
an anorectal malformation without this crucial
information frequently ends in a disaster.
Figure 6.17 shows a very short piece of bowel
distal to a colostomy. Figure 6.18 shows a colos-
tomy done in the descending colon leaving a loop
of the sigmoid colon long enough to perform a
comfortable pull-through. Once we obtained that
information, the patient is then turned into metic-
ulous lateral position. A lead marker is placed in
the anal dimple. During the fluoroscopic study in
lateral position, the images must include the
sacrum, the coccyx, the lead marker of the anal
dimple, and the entire lower pelvis. The radiolo-
gist must know that we are planning on a potential
6.7 Technique
Fig. 6.18 Distal colostogram showing a redundant piece
of bowel distal to the colostomy pull-through is feasible
posterior sagittal approach, and therefore, we
want to know the position of the rectum and fis-
tula as related to the coccyx and the perineal skin
(anal dimple). A common error from a radiologist
is to show only the fistula location without the
other points already mentioned that would orient
the surgeon in terms of the location of the fistula.
The injection continues, applying hydrostatic
pressure on the syringe and pulling on the Foley
catheter to avoid leakage of the contrast
(Animation 6.2). The contrast material runs
through the distal bowel and stops in a horizontal
line that corresponds to the pubococcygeal line
(Fig. 6.19). The fact that this line is horizontal
and the fact that it is located in the pubococcygeal
level indicate that it is not the end of the rectum.
That image is given by the contraction of the
“funnel-like” muscle mechanism. At that point,
89
the radiologist must exert enough hydrostatic
pressure to overcome the muscle tone of the fun-
nel mechanism to be able to distend the rectum
and see the real location of the end of the bowel
and the fistula site (Fig. 6.19). If the baby hap-
pens to have a recto-bladder neck fistula, the con-
trast goes into the bladder directly in a rather easy
way with minimal hydrostatic pressure. On the
other hand, if the rectum is connected to the pros-
tatic urethra and even more in cases of rectoure-
thral bulbar fistula, it requires a significant
hydrostatic pressure in order to force the contrast
material through the rectum surrounded by mus-
cle tone and through a tiny orifice communicat-
ing with the urethra. Figure 6.20 shows
characteristic images of a prostatic fistula and a
rectourethral bulbar fistula.
Interestingly, in cases of rectal urethral fistula,
the contrast material gets into the urethra and
usually goes up toward the bladder rather than
toward the penile urethra. We interpret this as a
manifestation of an increased muscle tone of the
so-called external urinary sphincter (Animation
6.2). The overwhelming majority of rectal uri-
nary fistulas are located above this external
sphincter, and we believe that is the reason why
the contrast goes rather toward the bladder. The
injection of contrast material must continue until
the bladder is full. By doing this, we now have a
cystogram, and if the baby has reflux, it will
become then evident. The injection continues
until the baby is forced to void. During the void-
ing, we have the best possible images of the anat-
omy of the rectum and the urinary tract
(Fig. 6.20). Babies with suspected urinary tract
problems, such as hydronephrosis and evidence
of reflux, must receive prophylactic antibiotics
prior to this study because we must keep in mind
that we are pushing the contrast passing through
a contaminated distal rectum.
The high-pressure distal colostogram, as most
studies, has some potential risks. In over 1,000
male patients we have operated, we have evi-
dence of two perforations of the bowel that
occurred during the injection of contrast material.
One of them had a transverse colostomy. We do
not advocate the use of transverse colostomies in
anorectal malformations, and one of the reasons
90
a b
Fig. 6.19 Distal colostogram showing (a) the contrast
material ending in a horizontal line at the level of the
pubococcygeal line, giving the wrong impression of a
is because it is very difficult to generate enough
hydrostatic pressure through a transverse colos-
tomy to be able to achieve our goals in trying to
determine the anatomy of the recto-urinary fis-
tula. The colostomy in cases with transverse
colostomies requires much higher pressure and
perhaps that is the reason why that patient had a
rupture of the colon during the injection of con-
trast material. Since we were dealing with a
defunctionalized portion of the bowel, we thought
that what all the baby would require would be
intravenous fluids, antibiotics, and observation.
However, to our surprise and alarm, that baby
went into severe hypovolemic shock that required
immediate resuscitation followed by a laparot-
omy to clean the peritoneal cavity and close the
perforation of the colon. We attribute this severe
reaction to the fact that the contrast is very hyper-
osmolar. The second case was a patient that had a
6 Imaging
“high malformation.” (b) Same study after applying more
hydrostatic pressure
very abnormal type of bowel with very abnormal
blood supply, and we believe that could be a pre-
disposing factor to explain the perforation,
although we do not have evidence of that.
6.8 Most Common Errors
Some patients come to us with a distal colosto-
gram that has been done in another institution.
We have seen many errors including passing the
Foley catheter too far (Fig. 6.21). This may give
the false impression that the patient has a very
short piece of bowel distal to the colostomy. That
is why we emphasized that the catheter should be
introduced only 5 cm and then pulled back to
ensure that the balloon is impacted against the
abdominal wall.
6.9 Not Showing the Coccyx and the Sacrum During the Fluoroscopy Studies 91

a b

Fig. 6.20 Distal colostogram showing a (a) recto-bladder neck fistula, (b) rectoprostatic fistula, (c) rectourethral bul-
bar fistula

By far, the most common error that we have 6.9 Not Showing the Coccyx
seen is the lack of hydrostatic pressure that and the Sacrum During
induced the surgeon to believe that the patient the Fluoroscopy Studies
had a “high malformation” when actually he
was looking at a characteristic image of the The radiologist must remember that this study is
contrast material stopping at the level of done with the specific purpose to determine how
the pubococcygeal line. reachable the rectum is through a posterior
92 6 Imaging

Fig. 6.21 Inadequate distal colostogram. The Foley catheter was introduced too deep, giving a false impression of a
short bowel distal to the colostomy

sagittal incision. Therefore, our main point of ref-

erence is the coccyx. Sometimes the radiologist,
trying to avoid unnecessary radiation to the baby,
uses diaphragms or cones that only show the fis-
tula site, but do not show the points of reference
that are the pubis, coccyx, and sacrum as well as
the lead marker in the anal dimple. Another com-
mon mistake is not to take the film on perfect lat-
eral position. Some radiologists were trained
mainly in adults; in those cases, to study the ure-
thra, they recommend an oblique view. This is
highly inconvenient in patients with anorectal
malformation because of the reasons already
explained.
When the babies were subjected to a trans-
verse colostomy and did not have the main repair
for a long period of time, the distal colostogram
shows a very characteristic image of a non-
used distal colon (microcolon) followed by an
extremely dilated rectosigmoid (Fig. 6.22). That Fig. 6.22 Distal colostogram done through a transverse
colostomy. It shows a characteristic image of a narrow,
is another reason why we do not like transverse nonused colon, followed distally by a very dilated rectum
colostomies. After the transverse colostomy has
been opened, the colon continues having peri-
stalsis, passing mucus and desquamation cells, colostomy does not allow cleaning the distal
and pushing all that material distally. All these colon during the newborn period, and the meco-
produce a characteristic megacolon as the one nium is left there. We have evidence that the
seen in Fig. 6.22. In addition, the transverse severity of megarectosigmoid correlates with
6.11 Distal Colostogram in Cloacas
Fig. 6.23 Distal colostogram in a patient that previously
had an attempted failed repair
the degree of constipation that the patient will
suffer later in life.
Some patients come to us after a failed
attempted repair. They suffered from catastro-
phes and misadventures during an attempted
operation. They still have the colostomy, and we
had to do a distal colostogram without knowing
what we are going to find. We may find that the
rectum is completely atretic (Fig. 6.23). We may
find also that the patient has also persistence or
recurrent rectourethral fistula (Fig. 6.24).
6.10 Distal Colostogram
in Female Patients
In the past, we used distal colostogram also in
female patients with vestibular fistulas and some-
times with perineal fistulas. We do not recom-
mend doing that kind of study because we
consider that it is not useful for the diagnosis and
treatment of the patient. The injection of contrast
material through the distal stoma in that kind of
patient would show an image similar to a long
narrow fistula, and even if we apply more hydro-
static pressure, we never are able to distend the
93
Fig. 6.24 Voiding cystogram showing a recurrent recto-
urethral fistula
distal rectum because the contrast material
escapes through the fistula site and one cannot
generate enough pressure to show the real size of
the rectum (Fig. 6.25). These may give a false
impression of a long narrow fistula when actually
the rectum is distended all the way down to reach
a point a few millimeters from the skin. A real
long narrow fistula is an extremely unusual
condition.
6.11 Distal Colostogram
in Cloacas
In patients born with cloacas, the distal colosto-
gram is still a very valuable study, but this is true
particularly if it is combined with a three-
dimensional rotational scan.
Depending on the specific anatomy of the clo-
aca, the distal colostogram may show only the
rectum followed by the common channel
(Fig. 6.26) or may show also the one vagina or
two vaginas (Fig. 6.27). It is extremely unusual to
be able to fill up the urethra and bladder through
a distal colostogram. The study in cloacas can be
94
Fig. 6.25 Distal colostogram in a patient with rectoves-
tibular fistula, giving a false impression of a long narrow
fistula
Fig. 6.26 Distal colostogram in a patient with a cloaca
showing only the rectum
6 Imaging
Fig. 6.27 Distal colostogram in a patient with a cloaca
showing the rectum and the vaginas
done in combination with the passing of a cathe-
ter through the common channel, which may go
into the urethra and bladder and have an image of
those three structures (bladder, vagina, and rec-
tum) (Fig. 6.28).
During the last few years, with the very valu-
able collaboration of our interventional radiolo-
gists, we have been using a 3D rotational scan for
the evaluation of cloacas, particularly those that
have a complex anatomy. This study is done
under anesthesia, and we take advantage of that
in order to do a vaginoscopy and cystoscopy. This
study is usually done 1–2 days before the main
repair. During the vaginoscopy and cystoscopy,
we can also insert catheters in a selective manner
into the urethra or vaginas to give the radiologist
more alternatives for contrast injection. Some of
those patients come, already, with vesicostomies
or suprapubic cystostomy tubes, vaginotomies,
and colostomies. We insert catheters in every
single orifice that we can see in the pelvis, and
6.12 Monitoring Constipation
Fig. 6.28 Distal colostogram combined with injection from
below showing the rectum, urethra, bladder, and vagina
then the contrast material is injected sequentially
through each one of those catheters, and the scan
is done rotationally which gives extraordinary
useful images (see videos 6.1, 6.2 and 6.3 show-
ing the rotational studies). This imaging repre-
sents the ultimate technology. Cloacas can be
repaired without the help of this study, but cer-
tainly this type of study allows us to be more pre-
cise in our preoperative diagnosis and to plan our
procedures in a more accurate way.
6.12 Monitoring Constipation
The most common sequelae in terms of bowel
function in patients with anorectal malformation
is constipation. Constipation produces dilatation
of the rectosigmoid and sometimes the entire
colon. When constipation is not treated properly,
it produces fecal impaction, and that interferes
with bowel control in those patients that are born
with potential for fecal continence. Those patients
may behave like the child with severe idiopathic
95
constipation and encopresis. To prevent this, we
try very actively to monitor the dilatation of the
bowel and the capacity to empty every day. This
is with the specific purpose to prevent irreversible
dilatation of the colon that would make the man-
agement of these patients much more difficult.
After the colostomy is closed, the babies have
usually a period of frequent bowel movements
and tendency to diarrhea. We believe this is a
consequence of passing stool through a colon
that has never been used. In addition, the use of
strong antibiotics administered during the colos-
tomy closure we believe may contribute to this
phenomenon.
However, the clinician must be aware of the
fact that after a few days or sometimes weeks,
the bowel movement pattern changes and must
be prepared to diagnose and treat aggressively
enough the problem of constipation. We have
learned through the years that the fact that the
baby passes stool every day does not mean that
it is not constipated since the main problem is
not the number of bowel movements but the
capacity to empty completely the rectosigmoid
during the bowel movement. In fact, some of
the babies that have multiple bowel movements
in a day are the most severely constipated, and
eventually those tiny bowel movements become
episodes of soiling. After years of speculating
and guessing as to whether or not the baby was
constipated, we learned that the only objective
way to know if the baby is emptying the colon
is radiologically.
After the colostomy is closed, we recom-
mend taking routine abdominal x-ray films 2
weeks after surgery, then 1, 3, and 6 months and
a year. In addition, every time the mother sus-
pects the child is constipated, we suggest taking
an abdominal x-ray film. Every patient needs a
different amount of laxative, and to determine
whether or not we are using the right amount,
we used abdominal x-ray films. In other words,
by trial and error, we prescribe a specific amount
of laxative and then, after a few days, we take an
abdominal film to be sure that the amount of
laxative that we are using is the correct amount.
Many patients come to us after having an ano-
rectal malformation repair either by us or by
96
a b
Fig. 6.29 Contrast enema done to evaluate colonic motility. (a) Hypomotility. (b) Hypermotility
others at other institutions, and they were not
subjected to any kind of monitoring through
years. We suspect clinically that the patients are
constipated, and to evaluate the degree of con-
stipation that they suffer from, we order a
contrast enema with water-soluble material and
without colon preparation. The contrast material
must fill up the entire colon, and we request to
show us post-evacuation films which give us an
idea of the degree of hypomotility that the
patient suffers from (Fig. 6.30).
6.13 Radiology During the Bowel
Management Program
In Chap. 20, the reader may find a description of
our bowel management program, created to help
patients who suffer from fecal incontinence.
Basically, over a period of 1 week, we determine
the enema that is capable of emptying at least all
of the left side of the colon, to keep the patient
6 Imaging
completely clean in the underwear for 24 h. To do
this, we take an abdominal x-ray film every day
over a period of 1 week. In addition, also daily
we hear the clinical information from the parents
and adjust the type of enema accordingly.
6.14 Monitoring the Urinary Tract
As previously mentioned, 50 % of the patients
with anorectal malformations have an associated
urologic condition. The most common anatomic
problem is absent kidney followed by hydrone-
phrosis, and the most common functional prob-
lem is vesicoureteral reflux. Many times the
babies are born with significant kidney damage
that occurred in utero. All patients require a close
monitoring of the urinary tract, since we have
seen many patients who have a tendency to dete-
riorate with time. Even under normal circum-
stances in babies with good anorectal
malformations, we always like to follow them
through life. In a patient that has normal kidneys
6.14 Monitoring the Urinary Tract 97

by ultrasound, normal functional bladder, no uri-
nary tract infections, and urinary control, we still
like to take a kidney ultrasound at 3 months, 6
months, 1 year, 3 years, and 5 years later. If the
patient has no urologic symptoms and the ultra-
sounds remain normal, we believe that the kidney
ultrasound is enough as a monitoring of the uri-
nary tract. On the other hand, if the baby has
problems with anatomic urologic defects previ-
ously diagnosed, he/she may require a much
closer and sophisticated monitoring of the uri-
nary tract that may include kidney ultrasound,

a b

c d

Fig. 6.30 MRI (Peña/Patel technique). (a) Well-located rectum. (b) Mislocated rectum. (c) Posterior urethral diver-
ticulum. (d) Giant posterior urethral diverticulum
98
cystograms, and urodynamic studies, as well as a
frequent nephrologic monitoring. Patients with
neurologic deficits including abnormal sacrum,
tethered cord, or complex cloacas are, by defini-
tion, urologic patients that require very close
follow-up monitoring of the urinary function.
Many of the complex cloacas eventually will
require urinary reconstructions including bladder
augmentations and Mitrofanoff.
For patients that have been operated on at other
institutions and come to us, for instance, for bowel
management due to fecal and/or urinary inconti-
nence, we evaluate the entire colorectal anatomy,
pelvic anatomy, and urinary tract. We specifically
order a magnetic resonance imaging (MRI) study
with a specific technique that includes the placing
of a big Foley catheter in the rectum (Fig. 6.30a
and b). This will allow us to see the location of the
rectum as related to the sphincter mechanism. In
addition, it is an excellent study to make a diagno-
sis of a posterior urethral diverticulum which is a
complication that we have seen in the past in
patients that were operated abdominoperineally,
having a rectourethral bulbar fistula; the surgeons
found it difficult to reach the lower end of the
bowel, amputated the bowel, and left a piece of
rectum attached to the urethra (Figs. 6.30c and d).
That piece of rectum becomes a diverticulum that
produces mucus, forms stones, and produces
pseudourinary incontinence, and in one specific
case, the patient developed an adenocarcinoma.
With the advent of laparoscopic procedures, we
are seeing again this problem of posterior urethral
diverticulum, when the surgeons try to approach
laparoscopically a rectourethral bulbar fistula,
finding difficult to reach the lower end of the rec-
tum Animation 5 (Posterior urethral Diverticulum
in a patient with a recto-urethral bulbar fistula
approached laparoscopically). The voiding cysto-
urethrogram may or may not show the posterior
urethral diverticulum and therefore is not the ideal
method of diagnosis. The diagnosis of this entity
is confirmed cystoscopically, and the repair is
described in Chap. 22.
When the babies come to us for the first time
with anorectal malformations and did not have
spinal ultrasound during the first 3 months of life,
the only way to know the location of the conus
6 Imaging
terminalis is by an MRI study that is the most
accurate way to determine whether or not the
patient has tethered cord (Fig. 6.9c).
Imaging plays a very important role in the
diagnosis, follow-up, and management of patients
already operated (see Chap. 19).
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 Bowel Preparation in Pediatric
Colorectal Surgery
Colorectal operations are considered “contami-
nated procedures” and represent, by definition,
cases with a higher risk of infection. Therefore,
patients must be subjected to some forms of bowel
preparation prior to the operation. In addition, the
use of prophylactic antibiotics must be considered.
Through the last 30 years, we have been fol-
lowing the evolution of the concepts related with
the different modalities of bowel preparation, as
well as the use of different antibiotics by mouth,
intravenously, and pre- and postoperatively.
Most of the literature is related with adults.
There are an enormous number of publications
related to adult conditions. We selected only a
few papers representative of the tendency to
avoid mechanical bowel preparation [1–4] and
antibiotics. Some surgeons found no evidence to
support the use of mechanical bowel prepara-
tions. Even more, some publications present data
indicating that the bowel preparations seem to
result in a higher incidence of infections and
dehiscence! The incidence of wound infections
in the adult literature varies from 4 to 19 % with
minor variations in favor of those patients who
did not receive preoperative colonic preparation.
The question for us, pediatric surgeons, is if
we should change our routines, based on the
information obtained from adult data. Our per-
sonal answer is no. The incidence of wound
infections reported in adult patients with and
without bowel preparations is still much higher
than in our cases [5]. If we closed 649 consecu-
tive colostomies without a single case of infec-
A. Peña, A. Bischoff, Surgical Treatment of Colorectal Problems in Children,
DOI 10.1007/978-3-319-14989-9_7, © Springer International Publishing Switzerland 2015
7
tion, following preoperative management, we
find no convincing reason to change. We under-
stand that perhaps, we could have the same good
results without bowel preparation; however, it is
not easy to change a routine that resulted in zero
infections. We also suspect that a meticulous sur-
gical technique is the key to avoid complications,
rather than the bowel preparation or the antibiot-
ics. Interestingly, during the last 30 years, we
have changed the type of antibiotics used pre-
and postoperatively according to the recommen-
dations of the epidemiology departments of the
institutions in which we worked, yet the results
were the same: no infections.
According to Breckler et al. [6], the over-
whelming majority of pediatric surgeons in the
United States still use mechanical bowel prepara-
tion and preoperative intravenous antibiotics.
Leys et al. [7] found slightly higher number of
anastomotic leaks and infections in pediatric
patients who underwent a mechanical bowel prep-
aration. Breckler et al. reported 14 % of wound
infections in colostomy closures and no differ-
ences between those who received antibiotics and
those patients who did not [8]. Serrurier et al.,
also in pediatric cases [9], found significantly
higher number of wound infections (14 %) in a
series of 272 colostomy closures, of patients who
received mechanical bowel preparation, whereas
only 5 % of those without preparation suffered
from this complication. Similar results were
reported by others [10, 11]. Pennington et al. [12]
in a retrospective analysis of the series of 42
101
102
American Children’s Hospitals, (5,473 patients)
not surprising, found that only 22.9 % of all
patients received an “evidence-based prepara-
tion.” However, the “evidence-based guidelines”
that they allude to are related with adult patients.
The question comes again: Should we change our
routines based on the adult literature? The ques-
tion is even more difficult to answer when our
results are excellent. In summary, we are not con-
vinced that following adult “evidence-based” rou-
tines will benefit our patients.
We are very proud of our excellent record of
zero wound infections in colostomy closures. We
close all wounds primarily and leave no drains.
The patients received irrigations of the proximal
stoma, with saline solution and intravenous anti-
biotics during anesthesia and 48 h postoperatively.
On the other hand, we had cases of infections or
minor dehiscent anoplasties in cases subjected to
primary pull-throughs without a colostomy. Those
patients received GoLYTELY® (total bowel prep-
aration). The dehiscences that we have seen
occurred usually between the 5 and 8 days post-
operatively while the patients are still fasting. We
take those patients to the operating room, resu-
tured the dehiscent areas, and prolong the period
of fasting for 2–3 more days. A complete dehis-
cence will receive a colostomy, which is a very
unusual event. A question to be answered is if a
dehiscence, occurring in a case with bowel prepa-
ration, has less serious consequences than in cases
without bowel preparation. We believe it does.
The type of colonic preparation that we recom-
mend varies, depending on the type of surgical
procedure that the patient is going to receive, as
well as the specific circumstances of the patient.
Thus, the bowel preparation required for a
newborn baby that is going to be subjected to a
primary, major, colorectal procedure without a
colostomy is different than the one required for
an older patient.
There is plenty of evidence related with the
safety of using GoLYTELY®1 (polyethylene gly-
1
GoLYTELY® PEG 3350 236 g, sodium sulfate 22.74 g,
sodium bicarbonate 6.74 g, sodium chloride 5.86 g, and
potassium chloride 2.97 g (4,000 mL) [regular and pine-
apple flavor].
7 Bowel Preparation in Pediatric Colorectal Surgery
col electrolyte solution) to clean the entire gastro-
intestinal tract in pediatric patients [13–18].
7.1 Major Procedures
Major procedures include primary or secondary
pull-throughs for anorectal malformations, for
Hirschsprung’s disease, or for idiopathic consti-
pation, performed without a protective colostomy.
Anoplasties and resection of rectal prolapse are
also included in this category. These patients must
be admitted, most of the time, 24 h before the
operation. Severely constipated or fecally
impacted patients, sometimes, are admitted 2 days
before surgery to be subjected to our protocol of
disimpaction before the cleaning of the gastroin-
testinal tract (See Chap. 25, Sect. 25.7.1).
The morning of admission the patient is only
allowed to take clear fluids by mouth. Usually
around noon time, the administration of
GoLYTELY®2 is started, at the rate of 25 mL/
kg/h until clear. Most of the time, this is adminis-
tered through a nasogastric tube. Occasionally,
some adolescents volunteer to drink GoLYTELY®
at the right speed, in order to try to avoid a naso-
gastric tube. Many of them try, yet, usually the
attempt is unsuccessful because they do not drink
fast enough or they have nausea, and therefore,
they need the nasogastric tube. It usually takes
about 4 h for the colon to be completely cleaned.
Some patients suffer from nausea and vomiting
during the administration of GoLYTELY®. When
that happens, the patient benefits from the admin-
istration of an antiemetic medication, but we con-
tinue the administration of the GoLYTELY®, at a
slightly slower rate, because we have seen that
most of the GoLYTELY® still goes through the
pylorus and through the bowel. During the
administration of GoLYTELY®, the patient usu-
ally suffers from a certain degree of abdominal
distention and cramps. Soon enough, they start
passing stool through the rectum. Later on, this
becomes liquid stool that becomes more and
more clear, until it is free of stool. Patients with
Hirschsprung’s disease frequently require rectal
2
See footnote 1.
7.2 Primary Procedures for the Treatment of Anorectal Malformation During the Newborn Period
irrigation to help relieve this distention during the
bowel prep. After 4 h of the administration of
GoLYTELY®, the nurse or the resident must see
what is coming out through the rectum of the
patient. A yellow or greenish color represents
bile that is excreted in the bowel and is consid-
ered acceptable. On the other hand, the presence
of particles of fecal matter is considered unac-
ceptable, and therefore, the administration of
GoLYTELY® should continue. Subsequently, the
resident or the nurse should check what is com-
ing out of the rectum every hour as the prep con-
tinues to run, until the goal of a clean colon is
achieved. At that point, the nasogastric tube can
be pulled out, and the patient is allowed to drink
clear fluids. (Clear fluids by mouth are not
allowed during the administration of the
GoLYTELY® because we were told by the com-
pany that produces GoLYTELY® that the glucose
contents allow for inflow of fluid into the colon
lumen, leading to dehydration.)
Most patients pass clear liquid through the
rectum after 4 h, but others require a longer
period of time. Occasionally, we see patients that
continue having GoLYTELY® through the night,
and then come to the operating room, only for us
to find out that they are still not clean.
It has been our experience that many patients
who received GoLYTELY® the day before an
operation come to the operating room suffering
from some degree of dehydration and a mild
degree of metabolic acidosis. This seems to be
more significant in babies. Therefore, it is our
routine to administer intravenous fluids during
the entire process to patients younger than 2 years
of age. In patients older than 2 years of age, intra-
venous fluids are desirable, but not vital.
In patients with a significant degree of mega-
colon and chronic fecal impaction, it is very
important to go through the process of fecal
disimpaction prior to the administration of
GoLYTELY®. The administration of
GoLYTELY® through a nasogastric tube in
patients with fecal impaction and severe megar-
ectosigmoid sometimes makes them feel very
uncomfortable. Their abdomen becomes very
distended, they complain of severe cramps, and
they feel very sick. The protocol of fecal disim-
103
paction consists of the administration of three
enemas per day and daily radiologic monitoring,
to be sure that the colon is free of fecal impaction
(see chapter on Idiopathic constipation). When
this is achieved, the patient can be admitted to
follow the protocol of administration of
GoLYTELY®.
Once the patient is in the operating room,
prior to the operation, we routinely pass a large
Foley catheter through the rectum, to evacuate
the remaining bowel prep fluid that is in the
colon.
Otherwise, this fluid becomes an inconvenience
during the surgical procedure. Also, we pass that
tube, in order to do a last irrigation before we start
the operation. Surprisingly, sometimes, even when
it was reported that the patient was clean, we find
stool in the colon. Occasionally, we have to cancel
a case because we were not successful in cleaning
the colon even in the operating room. We are very
proud of our postoperative results.
7.2 Primary Procedures
for the Treatment
of Anorectal Malformation
During the Newborn Period
There are some malformations that we repair pri-
marily without a colostomy during the newborn
period. It has been our experience that these
patients behave better from the point of view of
the possibility of infection, dehiscence, and
retraction, as compared to older patients. We
speculate that, perhaps, due to the fact that the
meconium has not been colonized during the first
few days of life, the chances of a wound infection
may be less.
Full-term, stable newborns, who are other-
wise healthy, without major associated defects,
born at our institution or nearby and having what
we call a “benign anorectal malformation,”
receive a primary procedure during the newborn
period without a full bowel preparation. These
cases include rectovestibular fistulas, recto-peri-
neal fistulas in males and females, anorectal mal-
formation without fistula in both male or female
patients, or rectourethral bulbar fistulas. In the
104
last two types of malformations, we expect to
see the distal rectal end, full of gas, located
below the coccyx, on the cross table lateral film
(See Chap. 25, Sect. 25.7.1), meaning that we
will be able to reach the rectum comfortably
through a posterior sagittal incision without tak-
ing unnecessary risks of injuring the urinary
tract. These patients are operated on at our insti-
tution usually within the first 48 h of life without
bowel preparation.
7.3 Primary Pull-Through
in Newborn Patients
with Hirschsprung’s Disease
When a baby is born at our institution, or is
brought during the first days of life, with the
diagnosis of Hirschsprung’s disease, we first con-
firm the diagnosis, and then, we introduce a naso-
gastric tube for gastric decompression and start a
program of rectal irrigations, performed as often
as necessary to decompress the colon. It is very
important for doctors and nurses to understand
the difference between a rectal irrigation and an
enema. A patient with Hirschsprung’s disease has
a congenital, intrinsic incapacity to empty the
colon due to a severe motility disorder. Therefore,
in general, enemas are to be avoided, as they may
simply be retained in the colon and can worsen
the distention. Irrigation, on the other hand, con-
sists of passing a large-lumen tube, through the
rectum and irrigating with small amounts
(10 mL) of saline solution at a time. The saline
solution is injected with a catheter-tip syringe
through the tube just to clear its lumen, to allow
decompression of the colonic contents.
Characteristically, the patient passes gas and liq-
uid stool in an explosive manner through the
lumen of the tube. A dramatic clinical improve-
ment can be observed in these babies after every
irrigation. Within a couple of days, the abdomen
is usually flat, and what we obtain with the irriga-
tions is only bile. At that point, and provided the
diagnosis has been histologically confirmed, the
patient is ready to undergo a primary pull-
through. We do not administer GoLYTELY® in
these kinds of patients. Most of these operations
7 Bowel Preparation in Pediatric Colorectal Surgery
are performed without a protective colostomy;
yet, we request consent for a colostomy in case it
is necessary.
7.4 Patients with Hirschsprung’s
Disease with Enterocolitis
After the Neonatal Period
A baby with enterocolitis is an extremely delicate,
sick patient. Therefore, he or she must be managed
in an expeditious way. The management includes
recto-colonic irrigations, adequate aggressive
hydration, and administration of metronidazole
(intravenously at the beginning, eventually by
mouth and later through rectal irrigations).
Occasionally we add broad-spectrum antibiotics.
The irrigations are performed as often as nec-
essary to decompress the colon and alleviate the
abdominal distention. It is important to keep in
mind what is happening in the colon. Stasis
occurs, leading to bacterial overgrowth, secretory
diarrhea, and dehydration. It is this cycle that
must be broken by the irrigations. During the
acute stages of enterocolitis, the irrigations will
produce a characteristic type of stool that is par-
ticularly fetid and frequently with abnormal bac-
teria, such as Clostridium difficile. We keep these
patients fasting, receiving parenteral nutrition
and with the irrigations per rectum until they
become asymptomatic. Fairly soon, the patient
has a flat abdomen, no vomiting, and no diarrhea.
In addition, the fetid stools disappear and are
replaced by clear fluid with bile. At this point, we
consider that the patient is free of enterocolitis
and therefore ready to be operated on. If we feed
these patients, they may develop enterocolitis
again. Therefore, we prefer to do the operation at
this point. These patients require longer periods
of rectal irrigations and fasting than neonates
before they are ready for an operation. In fact,
some of these patients receive a diverting colos-
tomy when we feel that the patient is not improv-
ing with the medical treatment described.
Sometimes it takes 1 or 2 weeks for the patient to
be ready for the operation to be sure that there is
no evidence of enterocolitis. We do not adminis-
ter GoLYTELY® in these kinds of patients.
References
7.5 Patients with Hirschsprung’s
Disease Beyond
the Neonatal Period,
Without Enterocolitis
Occasionally, we see patients at school age, or
even adolescence, who come to us suffering
from Hirschsprung’s disease. They usually have
a very severe megacolon and yet no history of
episodes of enterocolitis. We believe that this
type of patient suffers from a “benign type” of
Hirschsprung’s disease. For reasons that we do
not know, these patients never had an episode
of enterocolitis and behave in a more benign
way than the rest of the patients with
Hirschsprung’s disease. The colon preparation
in these patients is the same as the one described
for major pull-throughs. In other words, if the
patient is fecally impacted, we first disimpact
them, and then we bring them to the hospital to
administer GoLYTELY®, 1 or 2 days before the
operation.
7.6 Colostomy Closures
The preparation of the bowel in these patients is
much more simple. The patient has a normal
breakfast the day prior to surgery and comes to
the hospital thereafter. The rest of the day, the
patient is maintained on clear fluids by mouth
until 6 h before surgery. During that day, the
nurses irrigate the proximal stoma with saline
solution. There is no need to irrigate the distal
stoma, which is, by definition, clean and free of
stool. The patient receives irrigations through a
no. 10 Foley catheter with injections of 10–20 mL
of saline solution, allowing the saline to come
back out through the stoma. This maneuver is
repeated as many times as necessary, until the
saline solution recovered is clear. We do not use
GoLYTELY® for this preparation.
When the patient has two stomas that are
located too close one to the other, and both sto-
mas are included under the same stoma bag, it is
our routine to irrigate both stomas the day before
surgery. The same routine is applied for patients
who have a loop colostomy.
105
We have done over 700 colostomy closures, and
we are very proud of the fact that none of our
patients suffered from a wound infection [5]. None
of the patients had any kind of Penrose drainage
left in the wound or in the abdomen, and all wounds
were closed primarily including a subcuticular
stitch. Only two patients had postoperative compli-
cations: one of the patients had an anastomosis
dehiscence, and the other patient had a colonic per-
foration proximal to the anastomosis. We do not
have an explanation for that complication.
7.7 Patients with a Colostomy
Who Will Have a Repair
of an Anorectal
Malformation
When the colostomy is totally diverting (com-
pletely separated stomas, with the bag covering
only the proximal stoma), and the surgeon is sure
that the malformation can be repaired from below
(perineally or posterior sagittally), it is not neces-
sary to irrigate the proximal stoma. It is only nec-
essary to irrigate the distal one.
Sometimes, even when the rectum is consid-
ered reachable from below, the length of the
colon available between the distal stoma and the
most distal end of the rectum is so short that it is
fair to assume that this distance will interfere
with the pull-through, making it necessary to
mobilize the proximal stoma. In such cases, it is
necessary to clean the entire gastrointestinal tract
with GoLYTELY® as previously described. In
addition, in certain cases that may require a vagi-
nal replacement, a clean colon allows the surgeon
to consider the bowel from the proximal stoma as
an option for the neovagina.
References
1. Stellato TA, Danziger LH, Gordon N, Hau T, Hull
CC, Zollinger RM Jr, Shuck JM (1990) Antibiotics in
elective colon surgery. A randomized trial of oral, sys-
temic, and oral/systemic antibiotics for prophylaxis.
Am Surg 56(4):251–254
2. van Geldere D, Fa-Si-Oen P, Noach LA, Rietra PJ,
Peterse JL, Boom RP (2002) Complications after
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colorectal surgery without mechanical bowel prepara-
tion. J Am Coll Surg 194(1):40–47
3. Wille-Jørgensen P, Guenaga KF, Matos D, Castro AA
(2005) Pre-operative mechanical bowel cleansing or
not? An updated meta-analysis. Colorectal Dis
7(4):304–310
4. Dahabreh IJ, Steele DW, Shah N, Trikalinos TA
(2014) Oral mechanical bowel preparation for
colorectal surgery [Internet], Report No.
14-EHC018-EF. Rockville: Agency for Healthcare
Research and Quality (US)
5. Bischoff A, Levitt MA, Lawal TA, Peña A (2010)
Colostomy closure: how to avoid complications.
Pediatr Surg Int 26(11):1087–1092. doi:10.1007/
s00383-010-2690-6
6. Breckler FD, Fuchs JR, Rescorla FJ (2007) Survey of
pediatric surgeons on current practices of bowel prep-
aration for elective colorectal surgery in children. Am
J Surg 193(3):315–318
7. Leys CM, Austin MT, Pietsch JB, Lovvorn HN 3rd,
Pietsch JB (2005) Elective intestinal operations in
infants and children without mechanical bowel prepa-
ration: a pilot study. J Pediatr Surg 40(6):978–981
8. Breckler FD, Rescorla FJ, Billmire DF (2010) Wound
infection after colostomy closure for imperforate anus
in children: utility of preoperative oral antibiotics. J
Pediatr Surg 45(7):1509–1513. doi:10.1016/j.
jpedsurg.2009.10.054
9. Serrurier K, Liu J, Breckler F, Khozeimeh N, Billmire
D, Gingalewski C, Gollin G (2012) A multicenter
evaluation of the role of mechanical bowel prepara-
tion in pediatric colostomy takedown. J Pediatr Surg
47(1):190–193. doi:10.1016/j.jpedsurg.2011.10.044
10. Víctor D, Burek C, Corbetta JP, Sentagne A, Sager C,
Weller S, Paz E, Bortagaray JI, Lopez JC (2012)
Augmentation cystoplasty in children without preop-
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erative mechanical bowel preparation. J Pediatr Urol
8(2):201–204. doi:10.1016/j.jpurol.2011.01.015
11. Leal AJ, Tannuri AC, Tannuri U (2013) Mechanical
bowel preparation for esophagocoloplasty in children:
is it really necessary? Dis Esophagus 26(5):475–478.
doi:10.1111/j.1442-2050.2012.01378.x
12. Pennington EC, Feng C, St Peter SD, Islam S, Goldin
AB, Abdullah F, Rangel SJ (2014) Use of mechanical
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doi:10.1016/j.jpedsurg.2014.01.048
13. Tuggle DW, Hoelzer DJ, Tunell WP, Smith EI (1987)
The safety and cost-effectiveness of polyethylene gly-
col electrolyte solution bowel preparation in infants
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14. Ingebo KB, Heyman MB (1988) Polyethylene glycol-
electrolyte solution for intestinal clearance in children
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doi:10.4103/0189-6725.120889
 Recto-perineal Fistula
8

8.1 Definition, Frequency, These facts make us believe that this is not a
and Prognosis real anus. For similar reasons, we believe that
in order to call a malformation “anterior anus,”
Perineal fistula is an anal malformation in which it would be necessary for the patient to have a
the anal opening is located anterior to the center non-stenotic orifice, with normal anal canal,
of the sphincter. In females, the anal opening is and surrounded 360° by a sphincter mechanism
located somewhere between the location of the (as electrically demonstrated). If we accept
normal sphincter and the female genitalia in the this, as the definition of an anterior anus, then
area known as the perineum or perineal body. we must say that we have never seen that spe-
The anterior mislocation of the orifice could be cific type of defect. The fact that we have not
minimal (a few millimeters) (Fig. 8.1) or severe, seen such defect does not mean that it does not
becoming borderline with a malformation called exist, but it certainly means that it must be
vestibular fistula (Fig. 8.2). When the anal orifice
is located at the junction of both labia majora, the
malformation sometimes receives the French
name “fourchette malformation” (Fig. 8.3), which
is considered a defect intermediate between the
vestibular and perineal area. Yet, most anorectal
malformations in females can be clearly differen-
tiated between perineal and vestibular. This is,
perhaps, the anorectal defect subjected to more
controversies, both in semantics and treatments.
In female patients, some surgeons use different
terms to refer to this condition, including “ectopic
anus” and “anterior displacement of the anus”
[1–13]. We prefer the term perineal fistula for the
following reasons:
• The anal opening is most frequently strictured
or stenotic.
• There is no anal canal.
• The orifice is not surrounded 360° by a sphinc- Fig. 8.1 Picture of a perineal fistula with minimal
ter mechanism. anterior mislocation of the opening

A. Peña, A. Bischoff, Surgical Treatment of Colorectal Problems in Children, 107

DOI 10.1007/978-3-319-14989-9_8, © Springer International Publishing Switzerland 2015
108
a b
Fig. 8.2 Perineal fistula with a significant anterior mislocation of the anal opening. (a) Picture. (b) Diagram
Fig. 8.3 “Fourchette” type of fistula
extremely rare. In addition, we believe that
in the event of seeing a case suffering from
such condition, we would be very hesitant to
operate.
8 Recto-perineal Fistula
Perineal fistula is one of the most common
anorectal defects. Our series includes 90 males
and 84 females, occupying the second place in
frequency in males after the rectourethral fistulas
(bulbar and prostatic) together and the third place
in females after vestibular fistula and cloacas.
However, we have reasons to believe that the
perineal fistula is perhaps one of the two most
frequent defects in females. Most likely, the
majority of perineal fistula cases are not referred
to us, probably because they are considered easy
to repair. Ours is a referral center, and we receive
mainly complex and (or) complicated patients
from other institutions. That may be the reason to
explain the elevated number of cloacas that we
have, as well as the rather low number of cases
with perineal fistula. We conclude that the relative
frequency of presentation of these malformations
in our series is not representative of the frequency
in the general population.
Perineal fistulas are the most benign of all
anorectal defects in terms of functional prognosis
for bowel control. In fact, in our experience,
100 % of the patients operated by us have bowel
control provided the sacrum is normal. The
majority of patients have a good sacrum, but
there are exceptions, as we will be discussing
below.
8.2 Associated Defects
The chances for these patients to have bowel
control are 100 % provided they receive a good
operation. Ironically, the incidence of constipa-
tion in these patients is the highest of the entire
spectrum of anorectal malformations!! In fact,
we observed that the higher the malformation,
the poorer the prognosis for bowel control, but
the better the prognosis for constipation, or the
other way around, the lower and more benign the
malformation, the highest chances of bowel con-
trol, but also the highest possibilities of suffering
severe constipation. The reason for this is
unknown. Interestingly, higher malformations
require much more perirectal dissection in order
to bring the rectum down to the perineum. This
dissection means that we divide the vessels and
the nerves that surround the rectum in order to
mobilize it down. In other words, higher rectums
require more denervation, yet they have less con-
stipation!! Patients with perineal fistulas require
a minimal degree of mobilization and therefore
denervation, and yet they have the worst degree
of constipation.
Another important characteristic of this par-
ticular defect is that there is a group of patients
with perineal fistulas that run in families. It is
well documented in the literature that about 1 %
of all patients with anorectal malformations
have a sibling with an anorectal malformation
[14]. In other words, traditionally, when the par-
ents ask about the risk of having a second child
with an anorectal malformation, the standard
answer from geneticists, pediatricians, and us is
1 %. Yet, a deeper and more thoughtful analysis
of the question reveals that, actually, we have
never seen a family with two cloacas. In con-
trast, we have several families that have two or
more siblings with perineal fistula frequently
associated to a presacral mass and sacral defect.
The familiar characteristic of this condition has
been published many times [15–31]. Some other
members of the family may have the sacral
defect and the presacral mass and not the peri-
neal fistula. For this reason, in a patient with
perineal fistula, it is mandatory to document the
integrity of the sacrum with an AP x-ray film of
the sacrum to rule out a sacral defect because of
the high incidence of sacral defects and presa-
cral masses associated to perineal fistulas. Once
109
we document a case of perineal fistula with a
presacral mass, we have to screen the other
members of the family for the same defect. This
is extremely important because we have seen
patients that were diagnosed as having a peri-
neal fistula received an operation focused only
in the anal defect, and the patients were left with
a presacral mass and sometimes with very nega-
tive consequences. In addition, having diag-
nosed a sacral defect with a presacral mass
changes completely the functional prognosis for
these patients. This, we believe, it is extremely
important to diagnose, to discuss with the par-
ents, and to adjust their expectations in terms of
future functional prognosis. In addition, as will
be described later, this complex triad requires a
specific therapeutic strategy.
The association of anorectal malformation,
sacral defect, and presacral mass is well known
[32–61] and is frequently called Currarino
triad [38].
Unfortunately, there is a group of patients who
were born with perineal fistula, as previously men-
tioned, a defect with an excellent functional prog-
nosis, and yet, they end up suffering from fecal
incontinence after several therapeutic misadven-
tures and catastrophic events. Those are totally
preventable problems consecutive to the lack of
knowledge of the management of these patients.
8.2 Associated Defects
Following the same pattern already observed in the
entire spectrum of anorectal malformations,
perineal fistulas have the lowest incidence of
urologic-associated defects or functional disorders.
According to our database, 18 % of female patients
and 27 % male patients with perineal fistulas suffer
from urologic problems. Absent kidney occurs in
2–4 % of the cases, vesicoureteral reflux in 3–5 %,
hypospadias in 1 %, ectopic ureters in less than
1 %, vaginal septum in 2 %, hyposadias in 1 %,
undescended testicle in 2 %, bifid scrotum in 8 %,
and hydronephrosis in 6 %.
Vertebral anomalies also are less common in
these defects as compared to the others.
Hemivertebra occurs in less than 2–4 % of the
cases, and sacral defects are very unusual. The
110
sacra in these patients are usually normal except
in those patients who are born with presacral
mass and hemisacrum.
Patent ductus arteriosus occurs in less than
3 %, atrial septal defect in 2 %, ventricular septal
defect in 1 %, tetralogy of Fallot in 1 %, and
esophageal atresia in 1 %.
Tethered cord is present in 29 % of male
patients and 13 % of female patients. This rela-
tively high frequency of tethered cord is explained
by the fact that patients with perineal fistula have
a high frequency of association with sacral
defects and presacral masses.
8.3 Diagnosis
8.3.1 Female Patients
The diagnosis of a perineal fistula in a female
patient is a straightforward one. It is enough to
see the perineum of the baby to understand that
the anal orifice is abnormally located anterior to
the center of the sphincter (Figs. 8.1 and 8.2).
As previously mentioned, the orifice may be
located very close to the female genitalia or very
close to the center of the sphincter, and so the
degree of mislocation varies from patient to
patient. There is also a specific defect called by
Stephens [62] “perineal groove” which is a strip
of mucosa that runs between the female genitalia
and the anus. This defect is frequently associated
to perineal fistulas (Fig. 8.4). When this is left
Fig. 8.4 Picture of a perineal groove
8 Recto-perineal Fistula
untouched, most of the times, that strip of mucosa
suffers from metaplasia and changes into some-
thing that looks very much like skin. Yet, we have
seen patients of 5–7 years old in which that
mucosa continued producing some degree of
wetness, which prompted us to operate. The
operation of the perineal groove requires only
removal of a very thin layer of mucosa and sutur-
ing together in the midline the skin of the peri-
neal body. This operation is usually done in
conjunction with the mobilization of the anal
opening back to the center of the sphincter.
Sometimes, the anus looks like it is surrounded
by a normal sphincter mechanism. Yet, with an
electrical stimulator, under anesthesia, it is
extremely easy to demonstrate that it is just an
optical illusion. The bulk of the sphincter mecha-
nism is located behind the anal opening and
extends like a horseshoe on both sides of the anus,
but there is no sphincter whatsoever between the
female genitalia and the anal opening.
8.3.2 Male Patients
The diagnosis in the male patient may be a little
bit trickier. The baby may have a very obvious
orifice in the perineum through which one can
see meconium coming out. In such case, the diag-
nosis is easy (Fig. 8.5). The fistula is always
located anterior to the center of the sphincter. We
have never seen a case in which the anal opening
is located posterior to the center of the sphincter.
The orifice, however, could vary in size from
patient to patient being always stenotic.
Sometimes there is a tiny, almost invisible orifice
(Fig. 8.6). The fact that it is a small orifice usu-
ally delays the passing of meconium, which is
another reason why we believe that in the man-
agement of the newborn baby with anorectal mal-
formation, decisions concerning the opening of a
colostomy or anorectal repair should not be taken
before 24 h. In the first 24 h, it is very unlikely for
the patient to pass meconium through a tiny ori-
fice. When the pediatric surgeon is called to see a
baby with imperforate anus, it is extremely
important to do a meticulous inspection of
the perineum. One should not hesitate to use
8.3 Diagnosis
Fig. 8.5 Perineal fistula in a male. The anal orifice is
very obvious
Fig. 8.6 Very small perineal fistula
magnifying glasses if necessary to look carefully
for an orifice that sometimes is inconspicuous.
Figures 8.7, 8.8, 8.9, and 8.10 show different
appearances of the same malformation (perineal
fistula).
We intentionally eliminated confusing terms
used in the past to refer to this defect. These terms
include: “anal membrane,” “membranous steno-
sis,” “covered anus,” “anocutaneous fistula,” and
“translevator defect” [63–68].
111
Fig. 8.7 Arrow shows fistula opening
The orifice may go undetected. This may have
important therapeutic implications. We have seen
17 patients born with this defect in which the sur-
geon was unaware of the presence of a perineal
orifice, believed that the patient had a “high ano-
rectal malformation” opened a non-indicated
colostomy. Subsequently, the patient either did
not have a high-pressure distal colostogram or
had a colostogram without high pressure, and in
both circumstances the surgeon “confirmed” his
erroneous belief that he was dealing with a case
of “high” anorectal malformation. Because of
that, he went ahead with an abdominoperineal
operation designed for the treatment of more
complex malformations, an operation that is not
indicated in these cases, and that leaves the
patients fecally incontinent. This is something
tragic that should never occur if one follows spe-
cific steps in the diagnosis of these defects.
The perineum of these patients may have dif-
ferent external appearances. Sometimes they
112
Fig. 8.8 The discoloration of the skin, posterior to the
fistula opening represents the location of the sphincter
Fig. 8.9 Arrow shows fistula opening a little out of the
midline
8 Recto-perineal Fistula
Fig. 8.10 Arrow shows fistula opening at the base of the
scrotum
have what is called “bucket handle” malforma-
tion. Figure 8.11 shows three different variants
of a “bucket handle” malformation. Below the
strip of the skin (bucket handle), one can find the
fistula orifice. Sometimes, the orifice is located
at the base of the penis (Fig. 8.12). In other cases
the fistula runs subcuticular in the midline raphe,
forming a black ribbon type of structure, which
represents the presence of meconium running in
a subepithelial tunnel (Fig. 8.13). Other times,
we do not see a black ribbon type of structure
but rather a white ribbon which represents
mucous in the subepithelial fistula (Fig. 8.14).
The treatment in these patients consists in
unroofing that tract until we reach the real fistula
and then to continue with the technique described
in this chapter.
One must be aware of the fact that sometimes,
some patients are born with a fistula located at the
base of the penis or in the penis itself, and one
believes that it runs at a subcuticular level until it
reaches the rectum, and therefore, it can be treated
8.3 Diagnosis
a b
Fig. 8.11 Bucket handle malformations. Three different variants (a–c)
like a regular perineal fistula. Yet, surprisingly,
one may find that the fistula runs rather parallel to
the urethra, deeper and deeper in to the perineum,
and through the corpora (Fig. 8.15). As a conse-
quence, trying to follow that structure, the sur-
geon may provoke significant bleeding. Actually,
these specific variants are exceptions to the rule.
These malformations are considered rather com-
plex because they require more mobilization of
the rectum as well as to be separated completely
113
from the urethra. Fortunately, in most of the cases,
one can say that if one is able to see a perineal
orifice passing meconium, it means that we are
authorized to operate without a colostomy in the
way that it was described.
A sacral defect seen in an anterior/posterior
view of an x-ray film (Fig. 8.16) is always associ-
ated to a presacral mass [38], and the defect can
be very small (Fig. 8.17) or giant (Fig. 8.18). It
can be located laterally, giving the impression of a
114
Fig. 8.12 Perineal fistula located at the base of the penis
Fig. 8.13 “Black ribbon” malformation. Variant of a
perineal fistula (subepithelial with meconium)
8 Recto-perineal Fistula
Fig. 8.14 “White ribbon” malformation (subepithelial
with mucus) – variant of a perineal fistula
Fig. 8.15 Operative picture of a long narrow perineal
fistula. Very rare variant
“hemisacrum” or “scimitar.” It can also be located
in the midline (Fig. 8.19) giving an image of a
“bifid” sacrum. The size and location of the sacral
defect usually equals the location and size of the
mass. An MRI study will provide more accurate
information about the characteristics of the mass,
8.3 Diagnosis
Fig. 8.16 Hemisacrum
Fig. 8.17 Small sacral defect
Fig. 8.18 Giant sacral defect. The size of the presacral
mass corresponds to the size of the sacral defect
as well as the possible communication with the
dural space (anterior meningocele) (Fig. 8.20).
The most common types of presacral masses are,
115
Fig. 8.19 Bifid sacrum, the mass is located in the
midline
Fig. 8.20 MR image of a presacral mass with an anterior
meningocele
by far, teratoma/dermoid, lipoma, meningocele,
or a combination of all of these. Presacral masses
associated to perineal fistulas or to “rectal steno-
sis” represent an excellent example of the impor-
tance of having a good index of suspicion. The
presence of the mass may dramatically change the
functional prognosis for bowel and urinary func-
tion, particularly if the sacral defect is large.
116 8 Recto-perineal Fistula

In other words, a perineal fistula with normal
sacrum has an excellent functional prognosis, yet
the presence of a mass is a rather devastating find-
ing. The surgical strategy also changes, since it
must include the resection of the mass at the same
time of the repair of the anorectal malformation.
This resection can be a rather easy procedure or
can also be a formidable operation that requires
the participation of a neurosurgeon.
Some patients are born with a normally
located anus and a presacral mass producing a
rectal stenosis (Fig. 8.21). The perineal appear-
ance of these patients deserves a special com-
ment. This type of perineum is known as “funnel
anus” (Fig. 8.22). It certainly looks like a funnel.
The pectinate line is not visible outside because it
seems to be located higher, by the pushing effect
produced by the mass. This external appearance
must prompt us to order an AP x-ray film of the
sacrum and an MRI of the pelvis, in order to con-
firm the diagnosis of the presacral mass and rec-
tal stenosis.

a b

Fig. 8.21 Presacral mass producing a rectal stenosis. (a) Diagram showing the rectum compressed by a presacral mass,
which pulls up the anal canal. (b) Diagram of a perineal fistula associated to a presacral mass (pre- and postoperative)

Fig. 8.22 Picture of the perineum of a patient with a “funnel anus”

8.6 Cutback Operation
8.4 Management
Since these defects represent the simplest of all
anorectal malformations, the operation designed
to repair these defects is also a limited, rather
simple, yet meticulous operation. Operations to
repair these defects are performed without a pro-
tective colostomy. The exceptions to this rule
include cases of misdiagnosis. More specifically,
some male patients are born with no anal open-
ing; they rather have a tiny perineal orifice that
goes unrecognized, and the surgeon erroneously
believed that the patient had a “high” malforma-
tion and opens a non-indicated colostomy.
When the patients are born full term at our
institution, with no serious associated defects, we
operate on them without bowel preparation within
the first 72 h of life. Unfortunately, however,
patients frequently come to us weeks or months
after they were born, and in that type of case, we
feel it is safer to prepare the gastrointestinal tract
with GoLYTELY as described in the chapter
related to colonic preparation. In the case of a
newborn operated in the first hours of life, we feed
the patient usually 3–5 days after the operation.
We believe that those babies operated early, with-
out bowel preparation, have a better postoperative
course and less chance of infection, perhaps due
to the fact that the bacterial proliferation in the
bowel is nil or very limited. However, in older
patients, we are stricter in the protocol of manage-
ment. We use GoLYTELY for total bowel prepa-
ration (see Chap. 7), insert a central line, and keep
the patients 10 days with nothing by mouth.
During the newborn period, there are several
therapeutic options for these patients. The first
one is simple dilatation of the perineal orifice.
8.5 Dilatations
Dilatations usually help the patient to eliminate the
stool and avoid abdominal distention. This treat-
ment modality is preferred by many doctors all
over the world, based on the fact that these patients
will have bowel control with and without an opera-
tion. In fact, misadventure and catastrophic events
in these patients occur when somebody performed
117
the wrong type of operation or technically incor-
rect procedure that ended up in a series of compli-
cations that will be described later. Therefore, we
can say that it is preferable for the patient to receive
anal dilatations rather than a poor operation. The
fact is that these patients will have bowel control
with and without an operation. We explain that, by
the fact that the mislocation of the anal orifice is
only present in the lowest part of the rectum
(Fig. 8.2), most of the rectum, however, is well
located traversing within the funnel-like muscle
structure and is only deviated in the lowest part. It
is this type of case that exemplifies the fact that a
perfect location of the anus, within the sphincter
mechanism, is not a precondition to have bowel
control. Bowel control then seems to depend on
many other factors besides anal location.
Anal dilatations, from our point of view, are then
indicated under specific circumstances. One of
those could be a patient that has multiple serious
associated defects and abdominal distention.
Premature babies with severe cardiac conditions
that are not in good condition to be taken to the
operating room are also good candidates to receive
anal dilatations. The protocol of dilatation should be
as the one described here (Chap. 18). Another rea-
son to do dilatations is the case in which the surgeon
has no experience with performing a formal poste-
rior sagittal anoplasty for these patients. In that case,
the chances of hurting the patient are much less with
anal dilatations than with an operation. The opera-
tion also could be done later. Anal dilatations in
cases of perineal fistula are sometimes rather diffi-
cult and painful. The reason is that one has to dilate
a congenital stricture type of anomaly. This is not
the same as in cases of postoperative dilatations
after a technically correct operation; in those cases
usually the dilatations are uncomfortable, but not
painful. Very painful dilatation usually means a
congenital stricture or ischemia that occurred during
the performance of an anoplasty.
8.6 Cutback Operation
The cutback operation is another therapeutic
alternative for these patients. The indications are
similar to the ones of the anal dilatation. In fact,
118
it is a procedure that can be done with local
anesthesia. Again, we do not think that it should
be the final ideal operation for these patients, but
it is certainly an alternative for patients who are
extremely sick or in situations in which the sur-
geon does not have the necessary training to
perform a formal posterior sagittal anoplasty. The
cutback procedure consists of making an incision
in the posterior rim of the anal opening and sutur-
ing the skin to the mucosa. In other words, it is a
kind of Heineke-Mikulicz type of procedure. The
cosmetic appearance after the cutback is less than
optimal, yet this operation does not really hurt
the sphincter mechanism or the innervation of the
bowel, and therefore it is considered a good con-
temporizing preliminary operation, designed to
facilitate the passing of stool and alleviate the
abdominal distention.
8.7 Minimal Posterior Sagittal
Anoplasty
We believe that the ideal procedure to repair a peri-
neal fistula is what we call “minimal posterior sag-
ittal anoplasty.” Our experience with this operation
includes 174 cases with excellent results from the
functional and cosmetic point of view (Fig. 8.23).
We recognize, however, that even when it is a
small procedure, it is technically demanding. This
is the kind of operation that requires experience,
meticulous technique, familiarity with the delicate
Fig. 8.23 External appearance of an excellent operative
result
8 Recto-perineal Fistula
handling of tissues, and avoiding excessive burn-
ing. There is a significant difference in the surgical
technique for perineal fistulas in male and female
patients.
Obviously, when dealing with patients with
perineal fistulas associated to a presacral mass, it
is necessary to use a full posterior sagittal inci-
sion [47, 69–71].
8.7.1 Male Patients
A few authors referred to this malformation
using the terminology that we used, calling this
defect “recto-perineal fistula” [72–75]. The real
challenge in male patients with perineal fistula is
the separation of the rectum from the urethra
(Fig. 8.24). In fact, the most common and feared
intraoperative accident of this procedure is the
urethral injury. This happens when the surgeon
underestimates the complexity of this operation
and takes the baby to the operating room without
a Foley catheter in the bladder. Many surgeons
operate on these babies in the lithotomy posi-
tion; we do it in prone position. One must keep
in mind that the urethra of an infant or a newborn
male baby is an extremely delicate thin structure
and can be divided inadvertently. That is why we
cannot overemphasize the need to use a Foley
catheter in all of these cases. In addition, during
the repair, one must keep thinking very specifi-
cally in not injuring the urethra. Figure 8.25 shows
a suprapubic cystogram and retrograde urethro-
gram in a patient that underwent an attempted
failed repair of a perineal fistula and suffered a
complete division of the urethra. The patient was
passing urine through the perineum, and no urine
was coming out through the penis. The percuta-
neous cystogram showed that the urethra had
been divided. We had reoperated on several
patients like this; we were able to separate the
urethra from the rectum and put together both
ends of the urethra successfully. This complica-
tion is a serious one, and that is why we insist
that if the surgeon does not have experience with
this kind of operation, it is better to subject the
patient to anal dilatations and/or cutback type of
procedure.
8.7 Minimal Posterior Sagittal Anoplasty
Fig. 8.24 Diagram of a perineal fistula in a male patient, pre- and postoperative
Fig. 8.25 Suprapubic cystogram and anterior urethro-
gram in a patient who suffered from an accidental com-
plete division of the urethra, during the repair of a perineal
fistula. (a) Blind end of the distal urethra. (b) Distal ure-
thra draining next to the anus
119
8.7.2 Surgical Technique
Usually, a no. 8 Foley catheter goes well for a full-
term newborn baby, but if the baby is smaller than
that, we use a no. 6 Foley catheter. The baby is then
turned into the prone position with the pelvis ele-
vated. The skin of the perineum is washed, prepped,
and draped in the usual manner. Multiple 6-0 silk
stitches are placed in a circumferential manner tak-
ing the edges of the fistula site (Fig. 8.26). These
multiple stitches are used in order to apply uniform
traction on the rectum to facilitate the separation of
the rectum from the peripheral tissues, particularly
from the urethra. We recommend making a small
posterior sagittal incision that includes the entire
sphincter mechanism (Fig. 8.27). We have been
learning from all these cases that the sphincter
mechanism can be easily seen, particularly in white
babies because it is represented by a characteristic,
dark discoloration of the skin. In a full-term baby,
the anterior posterior diameter of the sphincter
mechanism is approximately 2–3 cm. The incision
is continued deeper through the entire sphincter
mechanism. The surgeon must keep in mind the
120
Fig. 8.26 Surgical repair of a perineal fistula in a male
patient – multiple silk stitches on the fistula site
Fig. 8.27 Posterior sagittal incision, dividing the sphinc-
ter mechanism
fact that near the fistula site (most distal rectum),
the rectal wall is almost in intimate contact with the
skin; as we become more proximal in the bowel,
the distance between the skin and the bowel is
greater. The entire sphincter mechanism is divided
until we are able to identify the characteristic white
8 Recto-perineal Fistula
Fig. 8.28 Picture showing the characteristic “white
fascia” covering the posterior rectal wall
fascia that covers the posterior rectal wall. This
becomes more evident when we apply traction on
the rectum (Fig. 8.28). The fascia is removed from
the posterior rectal wall in order to create a plane of
dissection in intimate contact with the rectal wall.
The dissection of the rectal wall is continued in
both lateral sides and from there; the dissection is
extended down to the skin. At this point, the next
step is the most delicate part of the operation which
is the mobilization and dissection of the anterior
rectal wall, with special emphasis in not injuring
the urethra. The surgeon must keep in mind that
while applying traction to the rectum, we are also
putting traction on the urethra that may be kinked
in an acute angle and to be injured, particularly if
the patient does not have a urethral catheter. One
important sign that indicates that the surgeon is
dangerously getting close to the urethra is to find
the kind of bleeding that is seen when working in
spongiosum/cavernosum type of tissue of the
penis. This indicates that we are dissecting very
close to the urethra (Fig. 8.29). If one finds that
8.7 Minimal Posterior Sagittal Anoplasty 121

kind of bleeding that is difficult to stop with the good position. Excessive, damaged tissue from
cautery, that means the dissection is being done too the rectum is resected, and a circumferential ano-
close to the urethra and far away from the rectum. plasty is performed with 16 stitches of 6-0 Vicryl
The dissection must be carried out closer to the rec- sutures (Fig. 8.30). As we mentioned before,
tum and away from the urethra. The bleeding origi- these patients usually have no pain after this kind
nated in the spongiosum tissue is better controlled of operation (Fig. 8.31). When this operation is
by suturing the spongiosum capsule with fine 6-0 performed in a newborn baby passing meconium
absorbable sutures. The dissection of the rectal and no real stool, the babies can be fed 2 or 3 days
wall and separation from the urethra is indispens- after the procedure. On the other hand, when the
able if one wants to really mobilize the rectum to operation is performed, without a protective
move it back to be placed within the limits of the colostomy weeks or months after the baby is
sphincter without tension. Fear to injure the urethra born, we believe that it is safer to insert a central
may provoke that the surgeon does not mobilize the line, keep the baby fasting, and administer paren-
rectum enough, leaving a tension anoplasty with teral nutrition for 10 days. These babies receive
high chances to suffer from dehiscence. intravenous antibiotics for 48 h.
Once the rectum has been completely sepa-
rated from the urethra and mobilized, the limits
of the sphincter are electrically determined and
marked with temporary silk stitches. The perineal
body then is reconstructed with interrupted 5-0
long-term absorbable sutures. The skin of the
perineum (where the fistula used to be located) is
sutured with interrupted 6-0 long-term absorb-
able sutures bringing together the anterior limits
of the sphincter. Usually the levator muscle is not
touched. Yet, the posterior edge of the muscle
complex in both sides of the midline is sutured
together with 5-0 long-term absorbable sutures
taking with the same sutures a bite of the poste-
rior rectal wall in order to anchor the rectum in a

Fig. 8.30 Anoplasty

Fig. 8.29 Picture showing the dissection of the anterior

rectal wall. The spongiosum tissue of the urethra can be
clearly seen Fig. 8.31 Finished operation
122 8 Recto-perineal Fistula

8.7.3 Female Patients bility of urethral injury and therefore no need of

The operation in female patients is usually sim-
pler than in the male patients because there is no
risk of injuring the urethra. The equivalent to the
urethra would be the vagina, yet it is extremely
unusual to see cases of vaginal injuries during the
repair of a perineal fistula. This is due to the fact
that the vagina and rectum are usually signifi-
cantly separated although one should not under-
estimate the possibility of injuring the vagina.
The technique is the same as described for male
patients except for the fact that there is no possi-
a Foley catheter (Fig. 8.32).
We have been impressed by the fact that one
of the anorectal malformations for which the
parents have more problems in making a deci-
sion about to operate or not to operate is pre-
cisely a perineal fistula in female patients. We
feel morally obligated to tell the parents that
babies with perineal fistulas will have bowel
control with and without an operation. Another
very important fact in these cases is that the
patients will suffer from severe constipation
with and without an operation. In other words,

a b

Fig. 8.32 Repair of a perineal fistula in a female patient. (a) Traction sutures. Relocation of anal opening. (b) Anoplasty.
Finished operation. (c) Separation of the rectum from the vagina
8.8 Postoperative Care
we should not let the parents develop wrong
expectations thinking that the operation will
prevent these patients from suffering constipa-
tion. In fact, we are not sure if the constipation
is going to get worse after an operation and the
unpleasant experience of anal dilatations. It is a
fact also that not relieving a stenosis (when
present) will make the constipation much more
severe. Therefore, if the patient has a perineal
fistula with stenosis (like most cases have), then
something must be done to avoid the exacerba-
tion of the constipation. When one tells the
family that the patient will have bowel control
with and without an operation and constipation
with and without an operation, then they rea-
sonably ask why to operate? There is a reason
why we believe that these female patients will
benefit from this operation, and that is the fact
that leaving the patient with an anteriorly
located anal orifice means that they have a very
short perineal body. We have seen teenage
patients that when they discover the character-
istics of their perineal anatomy, they express
their dissatisfaction, and they demand a repair
later in life, which is a more uncomfortable
experience than when it is done in the newborn
period. Also, a vaginal delivery of a baby may
have higher chances of a rectal injury in a
mother with a short perineum. Because of this,
we believe that it is indicated to do the opera-
tion in female patients provided the surgeon is
familiarized with the surgical technique and is
delicate enough to do it. In male cases, we can-
not argue that the operation will be good for
those kinds of reasons, and therefore we simply
tell the parents that the advantage of the opera-
tion is a better cosmetic effect as compared
with cutback or simple dilatations. We respect
the parents’ decision.
Cases with perineal fistulas associated to a pre-
sacral mass usually have a very long narrow fistula
with a dilated rectum above the location of the
mass (Fig. 8.33). The operation is a sophisticated
one because it requires resection of the mass. Most
presacral masses do not have an anterior meningo-
cele component. However, many have and there-
fore if the pediatric surgeon has no experience in
dealing with anterior meningoceles must go to the
123
Fig. 8.33 Contrast study showing a dilated rectum and a
narrow lower portion compressed by a presacral mass
operating room accompanied by a neurosurgeon
because it is a rather unpleasant surprise to find
that the dura has been open and the patient runs the
risk of suffering meningitis and (or) cerebrospinal
fluid fistula. After the mass has been resected, the
anterior meningocele is resected and the dura is
closed with nonabsorbable sutures. Sometimes it
is necessary to develop muscle and cartilage
patches to be sure that the dura space is well
sealed. The mass must always be resected because
we have seen patients later in life that suffer from
an infection of the mass, and also there is a certain
degree of risk of malignancy [41].
Once the mass is resected, we can mobilize the
rectum down. We should not try to dilate the long
narrow fistula that is the result of the compression
of the presacral mass. Usually that portion of rec-
tum is non-dilatable and therefore must be resected.
Attempts to dilate this kind of rectum have failed in
several cases and were referred to us after many
attempts of doing that. One must go and find the
upper rectum and pull it down to the perineum.
8.8 Postoperative Care
As we previously described, these babies, when
operated in the newborn period with meconium
in the bowel, can be fed after 3–5 days, and they
do not need parenteral nutrition. When the babies
124
are operated weeks or months later with real stool
in their colons, we keep them 10 days with noth-
ing by mouth, receiving parenteral nutrition.
We must anticipate a high degree of constipa-
tion in these patients. We cannot overemphasize
the importance of assuming that the patient is
going to be severely constipated and assuming
that he will need an amount of laxatives much
larger than what other patients need. The magni-
tude of constipation is worse when the defect is
associated with a presacral mass.
Constipation must be treated aggressively to
prevent severe consequences. We must keep in
mind that these babies are born with an abnormal
rectum that does not have the normal peristalsis,
and therefore these patients need help to empty
their rectum. We must also remember that every
patient has a different laxative requirement that is
not what it says in traditional books for the man-
agement of constipation. These patients need 2, 3,
4, 5, and 10 times larger dosages of laxatives than
other patients need. Constipation means incapacity
to empty the rectum which results in accumulation
of stool that leads to formation of a fecal impaction
that produces megacolon. All pediatric surgeons
are familiar with the fact that hollow viscus sub-
jected to an abnormal dilatation loses its peristaltic
efficiency. This phenomenon has been observed in
cases of colonic, duodenal, and small bowel
obstruction, as well as in megaureters. A dilated
bowel loses it peristalsis, and therefore, constipa-
tion produces retention, retention produces dilata-
tion, dilatation produces lack of peristalsis, lack of
peristalsis produces more constipation, creating a
vicious cycle that ends up with patients that behave
like being incontinent when actually they suffer
from overflow pseudoincontinence (see Chap. 18).
This is highly inconvenient because we must keep
in mind that we are dealing with patients that have
an excellent prognosis for bowel control. We have
seen 25 patients that came to our clinic suffering
from “fecal incontinence” and severe constipation.
When evaluated for the purpose of providing
bowel management, we found that they were born
with one of these malformations, they received a
technically correct operation, and yet they suffered
from “fecal incontinence.” A contrast enema
revealed that they had a huge rectosigmoid with
8 Recto-perineal Fistula
fecal impaction. We went ahead with our protocol
of disimpaction (see Chap. 25, Sect. 25.7.1).
Following that, we determined, by trial and error
and radiologic monitoring, the laxative requirement
of the patient. We usually found that it was a very
large amount of laxative, what the patient required
to allow the emptying of the rectosigmoid as radio-
logically demonstrated. Once we reached that
amount of laxative, we found that the patient was
actually fecally continent and all the patient
required from the beginning was the administra-
tion of the right amount of laxatives. Those are
very rewarding experiences in dealing with that
kind of patients.
Unfortunately, we also have been exposed to
about 35 patients that had the same symptomatol-
ogy just described, but unfortunately a surgeon sus-
pected that the patient could have Hirschsprung’s
disease. We strongly believe that Hirschsprung’s
disease is not more common in patients with ano-
rectal malformations than in the general popula-
tion. Yet, the incidence of constipation in patients
with anorectal malformation is extremely high, and
most of us pediatric surgeons were trained to sus-
pect Hirschsprung’s disease whenever we deal with
a child with constipation. Consequently, those
patients received a rectal biopsy, and occasionally,
those rectal biopsies show no ganglion cells which,
from our point of view, do not necessarily make the
diagnosis of Hirschsprung’s.
In a patient with Hirschsprung’s disease, we
expect to see not only absent ganglion cells, but we
also request an accurate description of the site
where the biopsy was taken from, and also we
expect from the pathology department to tell us
whether or not there was an increase in the activity
of acetylcholinesterase as well as the presence of
hypertrophic nerves. If we do not find that kind of
abnormalities and in the absence of symptoms of
enterocolitis, we simply do not believe that these
patients have Hirschsprung’s. Some of these
patients, as we said, had a biopsy done at other
institutions that showed unfortunately absent gan-
glion cells as a single finding with a dilated rectum,
which the surgeons considered enough evidence
for the diagnosis of Hirschsprung’s and went ahead
with an abdominoperineal resection. An abdomi-
noperineal resection in a constipated patient with
References
perineal fistula will certainly cure the problem of
constipation, but will also make the patient totally
fecally incontinent for life!! A patient with
Hirschsprung’s disease has a normal sphincter and
normal anal canal and therefore is capable of pre-
serving bowel control after the resection of the rec-
tosigmoid provided the operation is performed in a
technically correct manner, preserving the anal
canal. Yet, patients with anorectal malformations
do not have anal canal or have a very abnormal one
and therefore cannot tolerate that kind of operation.
In summary, one must be very careful before mak-
ing the diagnosis of Hirschsprung’s disease in
patients with anorectal malformations.
Another scenario includes patients born with a
perineal fistula, suffering from severe mistreated
constipation, megacolon, and overflow pseudoin-
continence. Some patients are seen by enthusias-
tic adult colorectal surgeons who offered them a
creation of an artificial sphincter or the creation
of a sphincter using gracilis muscle or gluteus
muscle. Those operations were performed with-
out a good preoperative selection of the patient,
and they actually make the patients worse.
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 Rectourethral Bulbar Fistula
9.1 Introduction
We define this malformation as a defect in a male
in which the rectum is abnormally communicat-
ing with the lowest portion of the posterior ure-
thra (Fig. 9.1). Figure 9.2 shows the characteristic
image of a distal colostogram performed in a
patient with this malformation.
This is the most common anorectal malforma-
tion defect in males in our series. This, of course,
should not be interpreted as an accurate reflection
of the incidence of this defect in the general pop-
ulation, since ours is a referral center, which
means that in general, we tend to receive more
complex defects and less of the benign and lower
type of malformations.
A literature review revealed very few publica-
tions under the name of “rectourethral bulbar fis-
tula” [1–4] in spite of the fact that it is a very
common defect. The reason for this, we believe,
is that most authors unfortunately are still using
the terminology of “high,” “intermediate,” and
“low”. Some authors consider this an “intermedi-
ate” malformation [1, 5].
Consistent with the concept that dictates that
these malformations occur in a spectrum fashion,
it is relatively common to see cases in which the
fistula is connected even more anteriorly (penile
Electronic supplementary material Supplementary
material is available in the online version of this chapter at
10.1007/978-3-319-14989-9_9.
A. Peña, A. Bischoff, Surgical Treatment of Colorectal Problems in Children,
DOI 10.1007/978-3-319-14989-9_9, © Springer International Publishing Switzerland 2015
9
urethra) (Fig. 9.3) [1, 2, 6]. Some authors use the
term “ano-penile-urethral fistula” [6].
Until the moment of writing this manuscript,
we have had experience with the treatment of 231
cases of rectourethral bulbar fistula. Two hundred
and nine of them were primary operations and 22
were reoperations.
As we have mentioned several times, every
case represents an anatomic variant. Therefore,
one can expect to see cases of rectourethral bulbar
fistula in which the rectum opens into the urethra a
little higher than the bulbar area. In other words,
the rectum connects with the urethra anywhere
between the prostatic urethra (middle portion of
the posterior urethra) and the bulbar portion of the
urethra (the lowest part of the posterior urethra).
9.2 Associated Defects
A significant number of our patients with recto-
urethral bulbar fistula have some sort of associ-
ated defect, mainly urologic. Hypospadias occur
in 10 % of the cases, vesicoureteral reflux in
about 13 % of the cases, and absent kidney in
10 % of the cases.
The sacrum, in this type of malformation, is
usually normal. The average AP sacral ratio in rec-
tourethral bulbar fistula is 0.61. The lateral one is
0.75; 2 % of the cases have sacral hemivertebrae.
Forty-six patients with this malformation were
evaluated for the presence of the tethered cord;
seven of them had that defect (15 %).
129
130 9 Rectourethral Bulbar Fistula

Fig. 9.1 Diagram showing a rectourethral bulbar fistula, preoperatively and postoperatively

Fig. 9.3 Distal colostogram showing a fistula located at

the penile urethra

Spinal abnormalities, mainly hemivertebra

Fig. 9.2 Distal colostogram showing a rectourethral
bulbar fistula
and butterfly vertebra, have been seen in approxi-
mately 7 % of the cases with a rectourethral bul-
bar fistula.
Esophageal atresia occurs in 4 % of these
patients; duodenal atresia in 1 %.
Patent ductus arteriosus occurs in 8 % of the
cases, but only one third of them required some
9.2 Associated Defects 131

sort of therapeutic intervention due to hemody-

namic problems. Atrial septum defects occurred
in 7 % of the cases, but only one third of them
required intervention. Ventricular septum defect
occurred in 5 % of the cases, and ¼ of the cases
required therapeutic intervention. Tetralogy of
Fallot occurred in 1 % of these cases.
There is a small group of male patients born
with an anorectal malformation that had commu-
nication between the rectum and the urethra in a
location more anterior to the bulbar site, meaning
somewhere in the spongiosum portion of the
penile urethra. In those cases, the rectum follows
a narrow fistula tract that runs parallel to the
penile urethra and opens at different levels of the
urethra (Fig. 9.3).
Those cases are not considered in the classifi-
cation presented in this book, due to the fact that
they are very unusual, but must be kept in mind
by the surgeons.
There are also many cases of rectourethral
bulbar fistula that, when seen during the newborn
period, the rectal pouch seems to be bulging
down and located very close to the perineal skin
(Fig. 9.4). Using the traditional, old parameters
to study the newborn babies, some of those cases Fig. 9.4 Distal colostogram showing the rectal pouch
were erroneously considered “low” malforma- located lower than the fistula
tions and consequently approached through the
perineum. The surgeons found the rectal pouch,
pulled it down successfully, and left the patient
with a persistent, untouched rectourethral fistula.
Soon enough, these patients passed urine through
the rectum and stool through the urethra [7]. That
is another reason why we do not favor the classi-
fication of these malformations into the tradi-
tional, old categories of “high,” “intermediate,”
and “low.”
During the neonatal period, these babies are,
in general, healthy, due to the fact that the inci-
dence of associated defects is rather low. The
perineum in these patients is usually “good look- Fig. 9.5 Perineum of a patient with a rectourethral bulbar
ing” (Fig. 9.5), meaning that they have good mid- fistula. There is a prominent midline groove and a clear
line groove and a well-defined anal dimple. Yet, anal dimple
there is no evidence of any kind of perineal fistula
or any meconium present in the perineum. During presence of the fistula, and we interpret that as a
the first 20 h of life, usually they do not pass manifestation of a very narrow fistula tract that
meconium through the urine, in spite of the requires a very significant intraluminal bowel
132
pressure in order to force the meconium through
the fistula into the urethra. Therefore, one should
not expect meconium in the urine in the first 20 h
of life (see Chap. 4 of this book).
If the baby is born in an institution where the
surgeons have a demonstrated interest and expe-
rience in the surgical management of neonatal
patients, it is conceivable that these patients could
be treated posterior sagittally during the newborn
period without a colostomy. The fundamental
requirement to perform that kind of treatment is
that the surgeon could see the image of the rec-
tum located well below the coccyx as delineated
by the presence of gas. By seeing that image in a
cross-table lateral film, one can be sure that by
opening posterior sagittally, the rectum will be
found, and therefore, one can avoid damage to
the urinary tract.
Most of the patients operated on by us come to
our institution with a colostomy already opened
at another hospital.
If the surgeon has no experience in the man-
agement of neonates with anorectal malforma-
tions, it is much safer to open a colostomy
(Chap. 5).
A laparoscopic approach is justified and conve-
nient in cases of anorectal malformations that
would require a laparotomy; yet, it has been done
in these kinds of cases [8–20]. We are definitely
opposed to that approach in this particular malfor-
mation. A posterior sagittal approach done in these
patients is an operation that takes approximately
2 h. The rectum is minimally dissected, just enough
to reach the perineum, which is very close. The
abdomen is not entered; the results are very good.
The patient has mild pain, can eat the same day of
the operation, and can be discharged the following
day. We do not understand the rationale of attempt-
ing the repair of these defects through the abdo-
men. A consequence of laparoscopic attempts to
repair bulbar fistulas is the posterior urethral diver-
ticula (pieces of rectum left attached to the urethra),
due to the fact that the surgeon was unable to reach
the end of the bowel (which resides well below the
peritoneal reflection) through the abdomen, laparo-
scopically [21] (Animation 9.1). Also, a much
more extensive circumferential dissection is done
laparoscopically which is unnecessary.
9 Rectourethral Bulbar Fistula
9.3 Posterior Sagittal
Anorectoplasty
We perform this operation as early in life as pos-
sible, since we try to avoid unpleasant memories
related to the operations and rectal dilations in
the patient. When the patients are born at our
institution, they receive a colostomy at birth,
and 2–4 weeks after that operation, assuming
that the baby has recovered well, is growing,
and developing normally, the baby is ready to
undergo this operation. Many times, the patients
are referred to us later in life, and that is why not
all of our patients have been operated early
in life.
9.4 Surgical Technique
Under good general anesthesia and endotracheal
intubation, the patient is placed first in lithotomy
position. We use a baby cystoscope routinely in
all of our cases to confirm the diagnosis of recto-
urethral bulbar fistula. We take advantage of this
procedure to learn more about the anatomy of the
posterior urethra. We have been learning impor-
tant anatomic facts from the cystoscopies in male
patients with anorectal malformations, including
abnormalities in the verumontanum, abnormal
locations of the prostatic utricle, and ectopic ure-
teral openings in the bladder neck and posterior
urethra. We also look at the bladder neck as well
as the ureteral orifices and the bladder in general.
At the end of this procedure, we pass a Foley
catheter into the bladder. Sometimes the recto-
urethral bulbar fistula is a large one, and due to its
location, the catheter tends to go into the rectum.
This happens in approximately 20 % of the cases.
If this happens, we can try to introduce a special
catheter with a curved tip, called “coude cathe-
ter.” We must try to direct the curve of the cathe-
ter anteriorly and cephalad in order to avoid the
catheter going into the rectum. This may or may
not work. If it does not work, then we can take a
regular Foley catheter and introduce a lacrimal
probe in its tip (Fig. 9.6) (photograph). By doing
that, we can direct the tip of the catheter conve-
9.4 Surgical Technique
niently into the posterior urethra. If this maneu-
ver does not work, we can pass a wire through a
cystoscope, followed by the catheter. Most of the
time, we are successful in placing the catheter in
the bladder using one of the two described
maneuvers. Occasionally, it is impossible to do
this and we have to leave the catheter in the rec-
tum. During the operation, when we open the rec-
tum, we have the opportunity to redirect the
catheter toward the bladder under direct vision.
With the Foley catheter in place, the patient is
placed in prone position with the pelvis elevated.
The positioning of the patient is the responsibil-
ity of the surgeon. One must be absolutely sure
that all of the pressure areas are well cushioned
Fig. 9.6 Lacrimal probe introduced in the tip of a Foley
catheter in order to direct it into the bladder and avoid
getting through the fistula into the rectum
Fig. 9.7 Prone position
with adequate cushioning
133
to avoid any potential injuries. The chest should
be slightly elevated from the table, with rubber
foam (Fig. 9.7) to be sure that the shoulders fall
forward to avoid stretching of the brachial
plexus. The tips of the toes should not rest on the
table. The penis should be free from the rubber
foam that is placed in the groin to elevate the
pelvis.
The perineum and perineal area are washed,
prepped, and draped in the usual manner. The
sterile towels are sutured to the skin to avoid their
displacement during the operation (Fig. 9.8).
Because of the position of the patient, the towels
that surround the operative field tend to fall away,
even if taped, and therefore, we have learned to
stitch them to the skin.
Animations 9.2 and 9.3 illustrate the basic
principles and technical maneuvers required to
repair this malformation.
An electrical stimulator is used to determine
the limits of the sphincter and also as a guide to
be sure that we make the incision dividing the
sphincter exactly in the midline, leaving equal
amounts of muscle on both sides. A special very
fine needle-tip cautery is used to avoid excessive
tissue burning. A very sharp needle allows the
surgeon to decrease the intensity of the electric
134 9 Rectourethral Bulbar Fistula

Fig. 9.8 Sterile towels sutured to the skin to avoid

contamination

current used during the operation by concentrat-

ing the energy in the tip of the needle. Not being
familiar with the use of this specific type of nee-
dle may provoke serious tissue burning. The nee-
dle must be used with quick movements touching
the tissues only with the very tip of the needle
and not with the lateral parts. The field should
always be dry, as wetness will diffuse the energy,
making the needle ineffective, which may induce
the surgeon to increase the intensity, producing
tissue burning.
Fig. 9.9 Parasagittal muscle fibers on both sides of the
The skin is opened with an incision running wound
from the inferior portion of the sacrum to the anal
dimple. When we open the skin, we continue
opening the subcutaneous tissue and we usually
find a group of fibers that run parallel to the skin
and on each side of the midline; therefore, we call
them parasagittal fibers (Fig. 9.9). These fibers
are divided exactly in the midline. We try to sepa-
rate rather than to cut them. A Weitlaner retractor
is used, trying to place the retractor as superfi-
cially as possible to not injure the muscle fibers.
Below the parasagittal fibers, we find a fatty tis-
sue that we call ischiorectal fossa (Fig. 9.10).
There, it is rather difficult to maintain the midline
plane. There is a very thin fascia that can be iden-
tified by careful observation of the tissues; that
thin fascia allows us to separate one side of the
ischiorectal fossa from the other and to avoid the
inconvenient herniation of fat into the operative
field that may interfere with the exposure. Below
the ischiorectal fossa, we find the levator muscle
(Fig. 9.11). The levator muscle is divided in the
exact midline with needle-tip cautery (Fig. 9.12).
In the area of the anal dimple, we find another
group of fibers that run perpendicular to the para- Fig. 9.10 Ischiorectal fossa located deeper than the para-
sagittal fibers, from the skin toward the urethra. sagittal fibers. Arrows – ischiorectal fossa
9.4 Surgical Technique 135

Fig. 9.12 The levator muscle is divided in the midline

Fig. 9.11 Levator muscles, found deeper than the ischio-

rectal fossa. L levator, I ischiorectal fossa

These we call the muscle complex (Fig. 9.13).

The muscle complex joins in continuity with the
levator muscle, forming an angle usually of about
90° (Fig. 9.14). The muscle complex fibers must
also be divided exactly in the midline. After we
divide the entire sphincter mechanism, deeper,
we identify a characteristic white fascia that cov-
ers the rectum posteriorly (Fig. 9.15). The white
fascia is divided to create a plane of dissection as
close as possible to the rectal wall. The dissection
of the rectum must be performed by staying in Fig. 9.13 Muscle complex. (a) Anterior limit of the
intimate contact with the rectal wall itself. sphincter. (b) Posterior limit of the sphincter
These patients should never be approached
without a good, preoperative distal colostogram injure the urinary tract since the rectum is the first
(Fig. 9.16 and Animation 9.4). This study per- structure that we identify because it actually
formed prior to this operation shows that the rec- bulges when we open the white fascia.
tum is located below the coccyx and opens in the Two 5-0 silk stitches are placed taking the
lowest part of the posterior urethra; therefore, posterior rectal wall, one on each side of the mid-
when we approach these patients posterior sagit- line (Fig. 9.17). The needle-tip cautery is used
tally, there is no way to miss the rectum or to again to divide the posterior rectal wall in
136
Fig. 9.14 Junction of levator and muscle complex (the
rectum in this case was already separated from the ure-
thra). Arrow shows the point of junction
between these two silk stitches. As we identify
the lumen of the rectum, we place 5-0 silk sutures,
taking the edges of the rectum in each side of the
incision (Fig. 9.18).
The incision then is extended distally, yet
remaining exactly in the midline. As we open
with the needle-tip cautery gradually, we keep
placing 5-0 silk stitches, taking the edges of the
rectal wall. The use of retractors placed on the
rectal mucosa must be avoided, because it trau-
matizes the tissues, producing edema and bleed-
ing that may interfere with the visualization of
the fistula. The fistula will become evident just by
continuing the incision in the midline. A last 5-0
silk stitch is placed, taking the most distal part of
our incision, including the edge of the fistula site
(Figs. 9.19 and 9.20). At this point, we are just
about to start the most delicate part of the
operation, which is the separation of the rectum
from the urinary tract.
9 Rectourethral Bulbar Fistula
Fig. 9.15 Deeper than the levator, a characteristic white
fascia is found, which covers the rectum from behind. The
arrow shows the white fascia
Most of the catastrophic events that have
occurred in cases that were referred to us from
other institutions occurred at this particular stage:
trying to separate the rectum from the urinary
tract without following the basic principles. The
first principle is to have a distal colostogram good
enough to know that what we are dealing with is
indeed the rectum, and we are not blindly explor-
ing the pelvis of a baby. The most common
source of mistakes in these cases is the lack of a
distal colostogram. The surgeons entered looking
for a rectum that was not there; they actually did
not know the location of the rectum and suffered
terrible misadventures in looking for it. In the
process of searching for the rectum, they rather
found the urethra, prostate, seminal vesicles, vas
deferens, and nerves that must be preserved to
avoid neurogenic bladder and impotence. Trying
to pass a big right-angle clamp around the rectum
is a dreadful old, traditional maneuver that must
9.4 Surgical Technique
Fig. 9.16 Distal colostogram showing a rectourethral
bulbar fistula
Fig. 9.17 Two silk stitches are placed, taking the poste-
rior rectal wall, one on each side of the midline
137
Fig. 9.18 Diagram showing stitches in both rectal edges
be avoided. Old, simplistic diagrams showing the
rectal fistula connected to the urethra in a “T”
fashion (Fig. 9.21) may explain why some sur-
geons think that they can go around the rectum
with a right-angle clamp and simply “ligate” the
fistula. This is also one of our concerns related to
the laparoscopic approach of this malformation.
One must keep in mind that the lower the mal-
formation, the longer the common wall between
the rectum and the urinary tract. In other words,
patients that have a recto-bladder neck fistula
basically have no common wall between the
anterior rectal wall and the posterior wall of the
urinary tract. Therefore, those cases are ideal to
be treated laparoscopically. Trying to make two
walls out of one in a rectourethral bulbar fistula
deep down in the pelvis with laparoscopic instru-
ments is a risky business that frequently produces
serious injuries (Animation 9.1).
138
The next step is to separate the rectum from
the urinary tract, being sure that we do not injure
any of the important elements of the urinary tract
in that area. In order to do that, we place multiple
Fig. 9.19 Operative view of the open rectum
a b
Fig. 9.20 The fistula is found always in the midline at
the most distal part of the rectal opening. (a) Arrow show-
ing the fistula. (b) Last stitch is placed in the midline, tak-
9 Rectourethral Bulbar Fistula
6-0 silk stitches taking the rectal mucosa about 2
or 3 mm above the fistula site, forming a hemi-
circumference cephalad to the fistula orifice. The
rectal mucosa of the anterior rectal wall in this
area has many folds that probably represent the
original pectinate line. Those multiple folds make
the separation of the rectum from the urethra
more difficult. The multiple silk stitches allow us
to have control of all of those folds and separate
the rectum from the urethra, maintaining both
structures intact (Fig. 9.22). Once those stitches
have been placed, we then use the needle-tip cau-
tery to make an incision in the rectal mucosa dis-
tal to the 6-0 silk sutures and cephalad to the
fistula site. The incision is only 1 mm deep
(Fig. 9.23). At this point, we specifically recom-
mend not to continue trying to work between the
rectum and the urethra, but rather to work on the
lateral walls of the rectum. The remaining white
fascia, fat, and extrinsic blood supply are removed
from the rectal wall and cauterized, establishing a
very clean lateral plane of dissection that will
make the separation of the rectum from the uri-
nary tract easier (Fig. 9.24). The lateral plane
shows the path toward the anterior plane. A sub-
mucosal plane of dissection is then created
between the rectum and the urinary tract. We
are not supposed to see the vas deferens or the
c
ing the lower edge of the fistula, lacrimal probe introduced
in the fistula. (c) Diagram
9.4 Surgical Technique 139

Fig. 9.21 Old simplistic misguiding diagram showing the fis-

tula connecting the rectum to the urinary tract in a “T” fashion

Fig. 9.23 Incision on the rectal mucosa, one millimeter

deep, immediately cephalad to the fistula and distal to the
silk sutures. (a) Diagram. (b) Operative

seminal vesicles. When we see those elements, it

means that we are too deep in our dissection.
Staying in the submucosal plane for about 5 mm
Fig. 9.22 Multiple fine silk sutures are placed in a hemi- and then going to full thickness is what allows us
circumference, cephalad to the fistula to respect all the important urologic structures.
140 9 Rectourethral Bulbar Fistula

a b

Fig. 9.24 Dissection of the lateral walls of the rectum, removing all the white fascia, fat tissue, and extrinsic vessels.
(a) Before removing the white fascia. (b) After removing the white fascia

The dissection continues, intermittently The perirectal dissection continues until we

switching from the lateral to the ventral dissec-
tion, until the rectum is completely separated
from the urinary tract (Fig. 9.25). At that point,
we estimate how much length is necessary in
order to bring the rectum down to suture it to the
skin with no tension. Rectourethral bulbar fistula
cases require a minimal dissection on the rectum
to reach the skin, due to the fact that the rectum is
located rather low in the pelvis. This dissection is
performed in a circumferential manner.
Uniform traction is applied on all the multi-
ple silk stitches, including the 5-0 silk stitches
that we placed originally when we opened the
posterior rectal wall and the multiple 6-0 silk
stitches. We like to say that “traction creates
a plane,” and by doing that, we can identify
bands that represent the vessels and nerves that
hold the rectum up in the pelvis (Animation
9.2). These tension bands are identified as
grooves. The bands must be separated from the
rectal wall, burned, and divided. These vessels
must be burned carefully because they have a
tendency to bleed when they retract into the
pelvis (Fig. 9.26).
feel that we have gained enough length of rectum
to bring it down to the perineum (Fig. 9.27). It is
rather unusual to have to open the peritoneum
when dealing with rectourethral bulbar fistulas.
Opening the peritoneum is frequently necessary
in cases of rectoprostatic fistulas but only rarely
in this defect.
Once we gain enough rectal length, we then
evaluate whether or not the patient needs tapering
of the rectum. This is a maneuver that we have
used very often in the past and we are using less
and less now. We believe that this is a manifesta-
tion of the fact that now patients are receiving
better colostomies (descending colon). An ade-
quate colostomy decreases the frequency of
megarectum that we frequently saw in patients
that had transverse colostomies and were left
many months without a repair. Those patients
developed a megarectum that later translated into
severe constipation. We believe that patients are
now operated on earlier in life, and they have bet-
ter colostomies (not transverse). Irrigating and
cleaning the distal bowel during the opening of
the colostomy result in a collapsed rectum (no
9.4 Surgical Technique
a b
Fig. 9.25 Submucosal dissection of the anterior rectal wall to protect the urinary tract. (a) Diagram. (b) Operative
megarectum); therefore, it is rather unusual now
to have to do tapering of these rectums. It is
almost impossible to irrigate and clean a distal
rectosigmoid through a transverse colostomy.
When a tapering is necessary, we should taper
the rectum to the size of the available space,
within the limits of the sphincter mechanism. The
posterior aspect of the rectum is resected accord-
ingly (Fig. 9.28). One should not taper the ante-
rior rectal wall as this would leave a suture line
located against the urethral fistula closure suture
line, which is a predisposing factor for a fistula
formation. The tapering may include 30–60 % of
the rectal wall. The posterior rectal wall is
repaired with two layers of interrupted 5-0 Vicryl
sutures (Fig. 9.29).
141
The fistula is closed with three or four inter-
rupted stitches of 6-0 Vicryl sutures. Those
stitches take the white fascia that used to cover
the rectum and urethra. No special effort is made
to suture mucosa to mucosa at the fistula orifice
(Fig. 9.30). In over thousand cases of male
patients with anorectal malformations operated
by us, we have never had a recurrence of a recto-
urethral fistula.
The rectum is then placed within the limits of
the sphincter. The levator muscle is sutured
together behind the rectum in the midline with
interrupted 5-0 Vicryl sutures. This can be done
with the rectum located up and away from its
future location to facilitate the visualization of
the levator muscle (Fig. 9.31) or can be done with
142
a b
Fig. 9.26 Dividing and burning extrinsic vessels and bands of the rectum while applying traction, in order to pull it
down. (a) Diagram. (b) Operative
the rectum already in place, particularly if the
rectum is not very bulky. One can see the edge of
the levator muscle, or one can use the electrical
stimulator to be able to see it better.
We identify the junction between the levator
and the muscle complex at the place where they
create a 90° angle (Fig. 9.32). Actually, both
structures (levator and muscle complex) are part
of a continuum of muscle. This angle is less
noticeable in cases with a poor sphincter
mechanism.
Some patients with very poor sphincter mech-
anism (mainly rectoprostatic or recto-bladder
neck fistula) simply have no available space
between the levator and the urethra. There is no
way to reconstruct the levator behind the rectum.
Also, in patients with a very flat bottom, and
therefore very poor sphincter mechanisms, it is
9 Rectourethral Bulbar Fistula
very difficult to identify these sphincter struc-
tures. Fortunately, in cases of rectourethral bulbar
fistula, this sphincter mechanism is easy to iden-
tify. The posterior edges of the muscle complex
from each side are sutured together in the midline
with interrupted 5-0 Vicryl sutures (Fig. 9.33).
These stitches take a bite of the posterior rectal
wall in order to anchor the rectum in a good posi-
tion, and we think that that helps to prevent pro-
lapse. The limits of the sphincters are electrically
determined and marked with temporary 5-0 silk
stitches (Fig. 9.34). The perineal body is recon-
structed, bringing together the anterior limits of
the muscle complex and the anterior limits of the
sphincter in those cases in which our posterior
sagittal incision was extended beyond the ante-
rior limits of the sphincter (Fig. 9.35). The ischio-
rectal fossa is obliterated suturing the fat with
9.4 Surgical Technique 143

interrupted 5-0 Vicryl sutures. The same suture

material is used superficially to bring together the
parasagittal fibers and subcutaneous tissue, put-
ting a special emphasis in not taking the fibers of
the parasagittal fibers themselves with our
sutures, but rather bringing them together. U-type
of stitches placed parallel to the muscle fibers
facilitates the maneuver (Fig. 9.36). The ano-
plasty is performed with 16 circumferential
stitches under slight tension.
The silk sutures used to pull the rectum are
separated into two, right and left sutures. An inci-
sion is made in the anterior rectal wall in the mid-
line; a first stitch of 6-0 Vicryl is placed, taking
skin, bowel, and skin again as a U-type of suture.
The same maneuver is done in the posterior cor-
ner of the anoplasty. The edges of the rectum are
resected (Fig. 9.36), and stitches are placed in a
radial (circumferential) fashion. The keys to
avoid dehiscence of the anoplasty are to avoid
excessive tension or devascularization and to be
sure that all the stitches are placed under the same
Fig. 9.27 Rectal dissection finished, the rectum reaches
tension. Also, we want to be sure that the stitches
the perineum comfortably, without tension

a b

Fig. 9.28 Rectal tapering in a case of megarectum. Approximately 40 % of the posterior aspect of the rectum is
resected. (a) Diagram. (b) Operative
144
a b
Fig. 9.29 The posterior rectal wall is repaired with two layers of interrupted sutures. (a) Before suturing. (b) After
suturing
Fig. 9.30 Fine absorbable sutures are used to close the
urethral side of the fistula. Arrow shows the fistula site
take full-thickness bowel, since sutures placed
taking only the mucosa do not hold well to the
skin. The sutures should be tied but not to the
point of cutting the tissue with excessive force.
9 Rectourethral Bulbar Fistula
Through the years, we have learned that the
blood supply of the rectum is well preserved pro-
vided the rectal wall integrity is respected and
remains intact. An intact rectal wall guarantees a
good intramural blood supply. Dissection of the
rectum should be performed, staying as close as
possible to the bowel wall, yet without injuring it.
That is particularly crucial in patients with recto-
prostatic fistulas or higher defects. In patients
with rectourethral bulbar fistula, this usually does
not represent a problem because these patients
have a rectum that requires minimal circumferen-
tial dissection to reach the skin of the anal
dimple.
The skin incision is usually closed with subcu-
ticular 5-0 Monocryl (Fig. 9.37). We use an anti-
biotic ointment for the anoplasty and the posterior
sagittal incision to be used for 5 days postopera-
tively. A Foley catheter is left in place for 1 week.
A “double diaper” technique is used for the Foley
catheter. The first diaper has an orifice through
which the catheter is exteriorized; a second diaper
is used to receive the urine. In this way, we avoid
the use of a bulky Foley bag or “urinometer.” In
addition, the skin of the patient’s perineum is bet-
ter protected. We have never seen a case of an
“ascending urinary tract infection” attributable to
this technique.
We try to leave the catheter in place for one
entire week. Sometimes, however, 3 or 5 days after
9.4 Surgical Technique 145

b c

Fig. 9.31 Sutures are lying down taking both edges of (b) Operative. (c) The rectum was pulled down deeper
the levator muscle. The rectum is pulled up in order to than the levator
have a more clear view of the levator muscle. (a) Diagram.
146
Fig. 9.32 The limits between the levator muscle and the
muscle complex are arbitrarily determined at the place
where both structures form an angle. a angle at the junc-
tion of levator muscle complex, L levator, M muscle
complex
a b
Fig. 9.33 The posterior edges of the muscle complex are sutured together, taking with the same suture a bite of the
posterior rectal wall. (a) Diagram. (b) Operative
9 Rectourethral Bulbar Fistula
the operation, the parents describe that the babies
suffer from severe cramps and simultaneously the
parents can see urine coming out around the Foley
catheter. That is a characteristic manifestation of
bladder spasms. One can give them oxybutinin for
the treatment of these spasms, but it usually does
not work, and it is better to simply remove the
Foley catheter; by doing that, even after only 48 h
from the operation, we have never seen a problem.
The fact that the baby is voiding around the cathe-
ter means that the catheter is no longer needed.
Most patients, however, can tolerate the catheter
for one entire week. A week after surgery, the baby
comes to our clinic and we pull the catheter in the
morning to be sure that the patient can urinate well.
Not being able to urinate for a baby with a
good sacrum and a rectourethral bulbar fistula
after the Foley is removed is a bad sign and usu-
ally is the result of a poor surgical technique. If
the baby was urinary continent, had a normal ure-
thra, and cannot void after a posterior sagittal
operation, it means that there must have been an
intraoperative nerve injury. This is prevented by
following the basic principles of the operation,
mainly staying exactly in the midline during the
dissection of the rectum and being careful in the
9.4 Surgical Technique
a b
Fig. 9.34 The limits of the sphincter at the skin level
have been electrically determined and marked with
temporary silk stitches, prior to the levator reconstruction.
Fig. 9.35 The perineal body was reconstructed, bringing
together the anterior limits of the sphincter. This was done
prior to the pull-through of the rectum
147
(a) Electrical stimulation to determine the limits. (b) The
limits of the sphincter marked with temporary silk stitches
Fig. 9.36 Anoplasty performed with 16 circumferential
stitches
area of dissection anteriorly between the rectum
and urinary tract. Also, a baby who had erections
prior to the operation and the parents describe
that they cannot see erections after the operation
can be interpreted in the same way.
148
Fig. 9.37 Subcutaneous tissue, dermis, and skin are
closed
Sometimes, those symptoms (urinary reten-
tion and absence of erections) are temporary and
patients recover, but it is still a very worrisome
sign. In patients with very abnormal sacra or
myelomeningocele, one can expect those kinds
of problems, but in patients with a normal sacrum,
they are considered iatrogenic and therefore
unacceptable.
Two weeks after surgery, the baby comes to the
clinic, and we show the parents how to perform
anal dilatations. We give them a copy of our pro-
tocol (See Chap. 5) that they are supposed to fol-
low religiously. Dilatation should not be painful;
they are uncomfortable but not painful if the oper-
ation was done correctly. Usually, about 2 months
after these operations, the colostomy is closed.
9.5 Functional Results
Ninety-seven patients were older than 3 years
and in contact with us, and 75 of them (78 %) had
voluntary bowel movements. Half of them occa-
sionally had marks (soiling) in the underwear.
Ninety-two percent of patients are urinary conti-
nent. The soiling is usually a consequence of a
problem with constipation that has not been
treated well. The use of laxatives frequently takes
care of this problem.
9 Rectourethral Bulbar Fistula
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comparison of laparoscopic-assisted (LAARP) and
posterior sagittal (PSARP) anorectoplasty in the out-
come of intermediate and high anorectal malforma-
tions. S Afr J Surg 49(1):39–43
17. England RJ, Warren SL, Bezuidenhout L, Numanoglu
A, Millar AJ (2012) Laparoscopic repair of anorectal
malformations at the Red Cross War Memorial
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18. Jung SM, Lee SK, Seo JM (2013) Experience with
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149
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doi:10.1016/j.jpedsurg.2012.08.001
19. Liem NT, Quynh TA (2013) Combined laparoscopic
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48(6):1450–1453. doi:10.1016/j.jpedsurg.2013.03.007
20. Yamataka A, Yoshida R, Kobayashi H, Lane GJ,
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21. Koga H, Okazaki T, Yamataka A, Kobayashi H, Yanai
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58–60
 Rectourethral Prostatic Fistula
10

10.1 Introduction As we previously mentioned, Mother Nature

does not respect the classifications that we create
This malformation is defined as a defect in in order to communicate between ourselves.
which the rectum is abnormally communicating Thus, a rectourethral prostatic fistula sometimes
with the middle portion of the posterior urethra, is located a little close to the bulbar urethra or
also known as the prostatic urethra, and there is sometimes is located close to the bladder neck.
no anal opening (Fig. 10.1). This is the second
most common anorectal malformation defect in
males seen by us [1]. Until the moment of writ- 10.2 Associated Defects
ing this manuscript, our experience included 227
patients operated by us with prostatic fistula: The general frequency of association of other
193 of them were primary and 34 were reopera- defects is higher than in cases of rectourethral
tions. Rectoprostatic fistula represents a defect bulbar cases. Absent kidney occurs in 10 % of
considered intermediate in terms of complexity cases, hydronephrosis in 6 % of cases, vesicoure-
between a rectourethral bulbar fistula and a
recto-bladder neck fistula. Many authors [2, 3]
do not make a distinction between rectourethral
bulbar fistula and rectourethral prostatic fistula,
but rather consider both groups together and use
a single generic name, which is “rectourethral
fistula.” We believe that it is important to differ-
entiate these two groups (prostatic and bulbar)
because they have different therapeutic implica-
tions and different functional prognoses. In
addition, the frequency of association of other
defects is significantly different between these
three groups (bulbar, prostatic, and bladder
neck).

Electronic supplementary material Supplementary

material is available in the online version of this chapter at
10.1007/978-3-319-14989-9_10. Fig. 10.1 Diagram showing a prostatic fistula

A. Peña, A. Bischoff, Surgical Treatment of Colorectal Problems in Children, 151

DOI 10.1007/978-3-319-14989-9_10, © Springer International Publishing Switzerland 2015
152
teral reflux in 26 % of cases, and hypospadias in
7.3 % of cases. Undescended testicles were pres-
ent in 8 % of these cases. Ten percent of the
patients had a bifid scrotum. As can be seen, the
high frequency of associated urologic defects
puts this malformation into a category of serious
defect. The average AP sacral ratio for a patient
with rectoprostatic fistula is 0.55 for patients with
voluntary bowel movements and 0.47 for those
who are fecally incontinent. The lateral average
ratio is 0.64 for continent patients and 0.58 for
incontinent patients, which is significantly lower
than the sacral ratio of patients with rectourethral
bulbar fistula and significantly higher than the
ratio for cases with for recto-bladder neck fistu-
las. Twenty-two percent of rectoprostatic fistula
patients suffer from tethered cord which, again, is
a higher incidence than in cases of rectourethral
bulbar fistula. Hemivertebrae occur in 8 % of
cases, and they occur mainly in the lumbar spine.
Esophageal atresia occurs in 14 % of cases and
duodenal atresia in 2 % of cases. Patent ductus
arteriosus occurs in 7 % of cases, but only one
fourth of them require some sort of intervention
due to hemodynamic problems. Atrial septum
defects occur in 8 % of cases but did not require
any type of treatment. Ventricular septum defects
occur in 6 % of cases, but only one third of them
required therapeutic intervention. Tetralogy of
Fallot occurred in 2 % of cases.
As previously suggested, the common wall
located between the rectum and the urethra above
the fistula site is shorter in cases of rectoprostatic
fistula when compared to those of rectourethral
bulbar fistula (Fig. 10.1). This fact makes the
separation of the rectum from the urethra techni-
cally easier. Yet, once the rectum has been sepa-
rated from the urinary tract, the mobilization
required in order to pull the rectum down is a
more complex and technically demanding
maneuver.
Some of the rectoprostatic fistulas are located
close the bladder neck but not quite into the
bladder and therefore can be approached by
both a posterior sagittal incision and laparo-
scopically through the abdomen. This particular
type of defect represents a matter of controversy
in terms of which approach is better. We believe
10 Rectourethral Prostatic Fistula
that a surgeon that has experience with the pos-
terior sagittal approach can easily and safely
find the rectum posterior sagittally, separate it
from the urinary tract, and mobilize the rectum
down safely, provided the colostomy is well
located (not too distal). We also believe that a
well-trained laparoscopic surgeon can relatively
easily separate the rectum from the urinary tract
via laparoscopy. In other words, we believe that
to decide how to approach these patients is
something that should be done based on the spe-
cific circumstances of the surgeon and the
patient. Some of the serious catastrophes and
failed attempted repairs that we have seen hap-
pening at other institutions precisely occurred in
these types of high prostatic fistulas. In retro-
spect, the surgeon either did not have a good
distal colostogram (Animation 10.1) or simply
operated on the patient without a distal colosto-
gram, entered posterior sagittally looking for a
rectum that was located much higher than what
he thought, could not find the rectum, but rather
found structures that he was not looking for,
such as seminal vesicles, vas deferens, urethra,
or during the search, damaged important nerves
of the urogenital tract, resulting in neurogenic
bladder, a complication considered totally pre-
ventable [4].
The perineum of patients with prostatic fistula
may show signs of what we call bad prognosis.
The midline groove may not be so prominent,
and the anal dimple may not be represented by a
real fossette but rather by a group of fibers in the
midline raphe (Fig. 10.2).
We believe that all patients born with recto-
prostatic fistula benefit from a diverting colos-
tomy at birth and the malformation must be
repaired in a second operation. One of the main
arguments in favor of this approach is the fact
that the colostomy, in addition to decompressing
the gastrointestinal tract and saving the baby’s
life, allows us to perform a good high-pressure
distal colostogram, which is the only and best
way to provide information about the precise
location of the rectum and the fistula (Fig. 10.3).
It is this study that allows us to follow a specific
strategy during the repair of this malformation
and to avoid catastrophic complications.
10.4 Posterior Sagittal Anorectoplasty
a b
Fig. 10.2 Photograph showing the perineum of two patients with prostatic fistula. (a) prone position. (b) supine position
Fig. 10.3 Colostogram (BU and PR) comparing the images
of a bulbar fistula with a prostatic. P = Prostatic, B = Bulbar
10.3 Surgical Repair
Two to four weeks after the colostomy has been
opened, provided the patient is growing and devel-
oping normally, the main repair can be performed.
It is not an urgent procedure; if the surgeon is not
familiarized with the anatomy of little babies, he
can wait until the baby is bigger or reaches the size
that the surgeon is accustomed to operate on.
153
10.4 Posterior Sagittal
Anorectoplasty (Animation
10.2)
It is our routine to perform a cystoscopy in all of
these patients, and that is how we have been learn-
ing important anatomic details of the posterior ure-
thra. We have found that there is a spectrum of
defects in the posterior urethra, including the pres-
ence of ectopic ureters and abnormalities in the
verumontanum. Once we finish the cystoscopy, a
no. 8 Foley catheter is introduced through the ure-
thra and into the bladder. In general, the Foley
catheter is passed without difficulty; it does not go
into the rectum. Occasionally, however, there is a
kink of the urethra at the location of the fistula,
which may interfere with the passing of the Foley
catheter. Sometimes, in addition to the kink, there
is a real congenital stenosis. The posterior sagittal
approach is ideal to repair abnormalities of the
posterior urethra at the same time than the repair of
the anorectal malformation.
The higher the location of the fistula, the more
frequently we may see ectopic ureters. When
these abnormal ureters are ectopically connected
to the posterior urethra, they must be dealt with,
during the posterior sagittal approach.
The patient is positioned prone as previously
described for the posterior sagittal approach. The
posterior sagittal incision runs from the middle
portion of the sacrum to the anal dimple. We put
154
special emphasis in making the incision exactly
midline using the electrical stimulator to try to
leave equal amounts of muscle in both sides of
midline. We go through the skin, subcutaneous
tissue, parasagittal fibers, ischiorectal fossa, and
levator mechanism (see Chap. 9, Sect. 9.4). Once
we divide the levator mechanism, we have to
keep in mind the image of the distal colostogram
to determine where to exactly look for the rec-
tum. Figure 10.4 shows a diagram of rectobulbar
and rectoprostatic fistula. In general, in patients
with rectoprostatic fistula, the surgeon must look
for the rectum immediately below the coccyx. In
those particular cases where the rectum seems to
be located a little higher, close to the bladder
neck, we have to look for the rectum above the
coccyx. This is extremely important because the
possibility of producing extra damage to the uro-
genital tract increases in cases of rectoprostatic
Fig. 10.4 Diagrams showing a bulbar and a prostatic fistula
10 Rectourethral Prostatic Fistula
fistula with a high rectum. The rectum in patients
with rectoprostatic fistula is found to be much
smaller than in cases with bulbar fistula.
Unfortunately, the appearance of the white fascia
after we have divided the levator muscle does not
allow us to determine or predict where the rectum
is going to be found. The distal colostogram is the
main guideline that we should follow. Two silk
stitches are placed as high as possible on the pos-
terior aspect of what we think is the rectum in
front of the coccyx or above the coccyx assuming
that we are holding on the rectum (Fig. 10.5).
Using traction on these silk sutures, we can divide
the white fascia that covers the rectum, as well as
the perirectal fat, bands, and vessels located
deeper than the white fascia. By doing this, we
notice that the rectal wall gives up, and we can
mobilize it lower (Fig. 10.6). We continue the
dissection, staying as close as possible to the
10.4 Posterior Sagittal Anorectoplasty
Fig. 10.5 Finding the rectum immediately under the
coccyx
Fig. 10.6 Pulling the rectum down
155
Fig. 10.7 The rectum is open and the fistula identified
bowel wall, dividing bands and vessels until we
feel safe that we are actually dealing with the rec-
tum. At that point, we make an incision on the
posterior rectal wall in between the two stitches
to find the rectal lumen (Fig. 10.7). The incision
in the posterior rectal wall is extended caudally,
placing silk sutures on the edges of the rectum
until we find the fistula site, and the last 5-0 silk
stitch is placed taking the lower edge of the fistula
site (Fig. 10.8). Multiple 6-0 silk stitches are
placed taking the mucosa of the anterior rectal
wall in the upper hemi-circumference of the fis-
tula. Those multiple stitches are included into a
single clamp to apply uniform traction to facili-
tate the separation of the rectum from the urethra
(Fig. 10.9). Needle-tip cautery is used to make an
incision in the rectal mucosa between the multi-
ple 5-0 silk stitches and the fistula site. This inci-
sion is barely 1 mm deep (see Chap. 9). At that
point, we put together into a single mosquito
clamp the silk stitches that were previously
placed on one of the rectal edges; by applying
uniform traction on the mosquito, we can clearly
see the white fascia and the extrinsic blood sup-
156
Fig. 10.8 Further rectal mobilization to see the lumen
and the fistula better. Arrow showing the fistula
Fig. 10.9 Sutures placed in the upper circumference of
the fistula
10 Rectourethral Prostatic Fistula
Fig. 10.10 The rectum is already separated from the
urethra
ply of the rectum. The white fascia, fat, and ves-
sels are resected, exposing a clean bowel wall
which is the plane of dissection of the rectum.
The same steps are repeated on the opposite side
(see Chap. 9). With both lateral rectal walls clean,
the dissection between the rectum and the urinary
tract is started, having as a reference the lateral
plane previously established. Fortunately, as we
said before, the common wall between the rectum
and urethra in cases of rectoprostatic fistulas is
relatively short, usually about 5 mm in length.
Very soon, we find that the rectum and urinary
tract are completely separated structures
(Fig. 10.10). At that point, a circumferential dis-
section is performed, aimed to gain length of the
rectum. For that, we put all of the silk stitches that
we originally placed in the edges of the rectal
wall and those that we placed in the rectal mucosa
into a single clamp, again, to apply uniform trac-
tion. Small malleable retractors are used, in order
to identify the bands and vessels that hold the rec-
tum up in the pelvis. The dissection is performed
in a systematic circumferential manner dividing
10.4 Posterior Sagittal Anorectoplasty
Fig. 10.11 The rectum fully mobilized
those bands. Dividing bands and vessels allows
gaining length, which allows us to see new bands
and vessels previously unrecognized. Sometimes,
all what we can see is a groove, which represents
a tense band. We grab those vessels, separate
them from the bowel wall, and burn them, putting
special emphasis on not burning the bowel wall.
Very soon, the peritoneal reflection is identified
and opened, which allows mobilizing the rectum
even more. The dissection continues until the rec-
tum is mobilized enough to be anastomosed to
the anal dimple with no tension (Fig. 10.11).
Another very important piece of information
that we obtain from the distal colostogram is the
amount of bowel available between the distal
stoma and the end of the bowel. In other words,
we want to be sure that we are not dealing with a
patient who has a defective colostomy with a
very short piece of rectum attached to the abdom-
inal wall that interferes with the pull-through.
We have been happily surprised in dealing
with patients with rectoprostatic fistulas; we dis-
sected and divided all the extramural blood sup-
ply of a very impressive length of bowel in order
157
Fig. 10.12 Electrical stimulation to determine the limits
of the sphincter. Stimulator touching the posterior limit of
the sphincter in one side
to gain length, and yet the rectum survives.
Sometimes we see some duskiness of the rectum,
but provided the bowel wall was not damaged,
we know that we did not interfere with the intra-
mural blood supply, and therefore the rectal
blood supply will be good, and the rectum will
survive.
The limits of the sphincter are determined
with an electrical stimulator and are temporarily
marked with 5-0 silk stitches (Figs. 10.12, 10.13,
10.14, and 10.15). The perineal body, in those
cases in which it was opened, is reconstructed,
bringing together the anterior limits of the
sphincter (Fig. 10.16). The rectum is placed in
front of the levator and within the limits of the
muscle complex. Figure 10.17 shows a fully
reconstructed perineal body.
In this type of malformation, we began to see
a significant degree of what we call “caudal
regression.” We see, for instance, that the levator
muscle is located much deeper in our incisions
than in cases of rectourethral bulbar fistula. This
158
Fig. 10.13 Stimulator touching the anterior limit of the
opposite side
Fig. 10.14 Limits of the sphincter were determined and
marked with temporary stitches
10 Rectourethral Prostatic Fistula
Fig. 10.15 The rectum will be located between the four
sutures
Fig. 10.16 Reconstructing the perineal body. Bringing
together the anterior limits of the sphincter
is due to the fact that the rectum was always
located up in the pelvis, and therefore the levator
is located much deeper in direct contact with the
urinary tract. Therefore, once we separate the
rectum and dissect it enough for the pull-through,
we may find that sometimes it is impossible to
suture the levator muscle behind the rectum
because there is no available space between the
10.4 Posterior Sagittal Anorectoplasty 159

Fig. 10.17 Perineal body reconstructed Fig. 10.18 The levator has been sutured. Sutures placed
at the muscle complex
levator muscle and the urinary tract due to a lack
of development of the entire pelvis. In such cir-
cumstances we repair only the upper part of the
levator muscle behind the rectum with 5-0 long-
term absorbable suture.
The posterior edge of the muscle complex on
each side is sutured together with interrupted 5-0
long-term absorbable sutures, taking with the
same sutures a bite of the posterior rectal wall to
anchor the rectum (Fig. 10.18). In this particular
malformation (rectoprostatic fistula), we can see
different degrees of poor sphincter development.
In rectourethral bulbar fistula cases, the over-
whelming majority of cases have good sphinc-
ters. In cases with recto-bladder neck fistulas, it is
very common to find very poor sphincters, and in
prostatic fistula, we may find variable develop-
mental deficiencies of the sphincter mechanism.
Figure 10.19 is an operative picture showing that
the muscle complex sutures have been tied.
The anoplasty is performed within the limits
Fig. 10.19 Sutures of the muscle complex have been tied
of the sphincter, usually with 16 circumferential
stitches of 6-0 Vicryl sutures (Figs. 10.20, 10.21,
10.22, and 10.23). mechanism around it (Fig. 9.28, Chap. 9). If
Occasionally, we find that the rectum is too that is the case, we resect part of the posterior
large and bulky and requires tapering in order wall of the rectum and repair the rectal wall
to facilitate the reconstruction of the sphincter with two layers of interrupted 5-0 long-term
160
Fig. 10.20 Beginning the anoplasty
Fig. 10.21 Trimmed off one side of the extra rectum
absorbable sutures (Fig. 9.29, Chap. 9). As we
previously mentioned, this scenario is rather
unusual at present time. It was very common to
see that in the old times. We firmly believe that
that was a consequence of two facts: first, a
colostomy that was opened too proximal, it
tends to produce a megarectum. We believe that
is consecutive to the desquamation of mucosal
cells as well as mucus production and persistent
peristalsis; all this produces an accumulation in
10 Rectourethral Prostatic Fistula
Fig. 10.22 Anoplasty finished. Holding sutures in place.
Open anus
Fig. 10.23 Anoplasty finished. No holding sutures. Anus
closed
the distal rectum and dilatation of it. In addi-
tion, when a proximal (ascending or transverse)
colostomy is created, the surgeon cannot
remove the meconium accumulated in the distal
colon. The other fact that contributes to the for-
mation of a megarectum is the long period of
time elapsed between the opening of the colos-
References
tomy and the main repair. Nowadays, fortu-
nately surgeons are opening better colostomies
(descending, with separate stomas) that allow
them to clean the distal bowel from day 1, and
they are also performing earlier and earlier
repairs that avoid the great number of cases of
megarectum that we have seen in the past.
The operations to repair rectoprostatic fistulas
take more time than rectourethral bulbar fistula
cases because of the mobilization of the rectum.
On the other hand, we must keep in mind that in
cases of rectourethral bulbar fistula, the common
wall between both structures (rectal and urethral)
is longer, and therefore it requires more time to
separate the rectum from the urethra.
10.5 Postoperative Care and
Functional Results
These patients usually eat the same day of sur-
gery even when we open the peritoneum. Again,
we have been impressed by the fact that these
operations are not particularly painful and the
patients do not need morphine postoperatively.
We like to administer intravenous antibiotics for
48 h and keep the Foley catheter for one entire
week. The patient remains in the hospital 48 h
after the operation. One week after surgery, the
patient comes to our clinic in the early morning
to have the Foley catheter removed and then
remains in the clinic a couple of hours so that we
can be sure the patient is voiding with no diffi-
culty before going home. Two weeks after sur-
gery we start the process of anal dilatations
following our specific protocol (see Chap. 18).
161
Long-term follow-up of these patients, in our
hands, indicates that roughly 66 % of the patients
after 3 years of age have voluntary bowel move-
ments provided they had a good sacrum. However,
80 % of them suffer from occasional soiling of
the underwear. This means that we are dealing
with a much more serious condition as compared
with bulbar fistula. Ninety-two percent of these
patients had urinary control.
The sacral ratio seems to have a definite influ-
ence on the functional prognosis for bowel con-
trol, since 65 % of the patients with a ratio higher
than 0.7 had voluntary bowel movements,
whereas only 35 % of them had voluntary bowel
movements when their sacral ratio was lower
than 0.4. All patients with sacral ratio higher
than 0.7 were urinary continent, whereas only
35 % of them were continent when their ratio
was lower than 0.4.
References
1. Peña A (1988) Posterior sagittal anorectoplasty:
results in the management of 332 cases of anorectal,
malformations. Pediatr Surg Int 3:94–104
2. Endo M, Hayashi A, Ishihara M, Maie M, Nagasaki
A, Nishi T, Saeki M (1999) Analysis of 1,992 patients
with anorectal malformations over the past two
decades in Japan. Steering Committee of Japanese
Study Group of Anorectal Anomalies. J Pediatr Surg
34(3):435–441
3. Rintala RJ (1996) Anorectal malformations—man-
agement and outcome. Semin Neonatol 1(3):219–230.
doi:10.1016/S1084-2756(96)80040-6
4. Hong AR, Acuña MF, Peña A, Chaves L, Rodriguez
G (2002) Urologic injuries associated with repair of
anorectal malformations in male patients. J Pediatr
Surg 37(3):339–344. doi:10.1053/jpsu.2002.30810
 Recto-bladder Neck Fistula
11

11.1 Definition and Frequency surgeon tried to reach the rectum posterior
sagittally; he obviously could not find it, but in
Imperforate anus with a recto-bladder neck fis- the process, he damaged the vas deferens, semi-
tula is the highest of all anorectal malformations nal vesicles, and/or prostate. In some cases, the
seen in male patients. The rectum connects to the surgeons divided the entire urethra or the bladder
bladder neck (Fig. 11.1). It is relatively common neck and pulled down a megaureter or even the
to see that these patients have a rather narrow pel- entire bladder thinking that they were dealing
vic space. We interpret this like a manifestation with the rectum. These catastrophic events
of a significant degree of caudal regression occurred only in patients that were operated on
(Animation 11.1). The sacrum may be normal, without a preoperative high-pressure distal
but frequently, it is very abnormal or even absent. colostogram (Animation 11.2).
The frequency of associated defects is much
higher than in all the other malformations.
Fortunately, in our experience, this defect only
occurs in approximately 10 % of all anorectal
malformation patients in males [1]. Unfortunately,
this defect runs with the worst functional progno-
sis for bowel control and occasionally for urinary
control. In our experience, this particular defect
is the only one that requires a laparotomy or lapa-
roscopy in order to be repaired. In other words,
the rectum is located so high in the pelvis and
connected to the bladder neck (very high in the
urinary tract) that it is not possible to be reached
posterior sagittally.
In fact, some of the worst unfortunate
catastrophes that we have seen occurred in
babies that were born with this defect and a

Electronic supplementary material Supplementary

material is available in the online version of this chapter at
10.1007/978-3-319-14989-9_11. Fig. 11.1 Diagram showing a bladder neck fistula

A. Peña, A. Bischoff, Surgical Treatment of Colorectal Problems in Children, 163

DOI 10.1007/978-3-319-14989-9_11, © Springer International Publishing Switzerland 2015
164 11 Recto-bladder Neck Fistula

11.2 Associated Defects Table 11.1 List of urologic abnormalities in patients

with bladder neck fistula
11.2.1 Sacral Defects Anomaly No. (%)
VUR 36
The average sacral ratio in patients with recto- Absent kidney 33
bladder neck fistula is AP 0.51 and lateral 0.6 (nor- Hydronephrosis 29
mal AP is 0.74 and lateral 0.76). It is not uncommon Undescended testis 26
Bifid scrotum 18
to find these patients to have, in addition, sacral
Hypospadias 18
hemivertebrae in approximately 35 % of the cases.
Intravesical verumontanum 45
Urethral stenosis 13
Neurogenic bladder (congenital) 11
11.2.2 Spinal-Associated Defects Megaureter 9
Other 24
Fifteen percent of these patients have suffered
from hemivertebra, which produces scoliosis of
different degrees in magnitude. The most frequent
location of the hemivertebra is the lumbar spine,
followed in frequency by the thoracic spine.

11.2.3 Urologic-Associated Defects

As expected, the incidence of associated urologic

defects is the highest in these particular types of
patients. In a recent evaluation of 110 patients
operated on by us with recto-bladder neck fistula,
99 (89 %) of them suffered from some sort of
urologic malformation or vesicoureteral reflux.
The frequency of absent kidney in these patients
is 37/111 (33 %). Vesicoureteral reflux was pres-
ent in 40 patients (36 %). Eighteen percent of
them suffer from hypospadias. Thirty patients
(26 %) suffered from an undescended testicle.
Eighteen percent of them have a bifid scrotum.
Two patients have penile-scrotal transposition.
Thirty-three patients (29 %) were born with
hydronephrosis. In addition, there is a specific
group of patients born with recto-bladder neck Fig. 11.2 Diagram showing a case of bladder neck fistu-
las with an ectopic ureter
fistula that have a single kidney with hydrone-
phrosis, megaureter, and massive reflux. This is a
very bad situation because usually they have poor Table 11.1 shows the list of urologic
sacrum and therefore poor prognosis for bowel abnormalities.
and urinary control. In addition, the fact that the Ectopic ureters were present in ten cases
patients are born with a single kidney with hydro- (9 %). The ectopia occurs most commonly toward
nephrosis indicates that they already have a sig- the bladder neck and occasionally in the posterior
nificant degree of kidney damage and there is a urethra. This last group of ureters connected to
high chance that these patients will end up with a the posterior urethra usually originates from
kidney transplant when they grow up. completely damaged kidneys (Fig. 11.2).
11.3 Diagnosis
Fig. 11.3 Cystoscopic aspect of a verumontanum located
at the trigone
A serious, not previously known anomaly
found in 45 % (15 cases) of those patients in
whom we performed a cystoscopy was an ecto-
pic verumontanum. Six of them were located
in the trigone (Fig. 11.3), five in the bladder
neck, and four immediately below the bladder
neck. Based on this experience, we now con-
sider it mandatory to do a cystoscopy on these
patients to avoid unpleasant future surprises
for the patient and the family. When these
patients reach adolescence, they have erections
and orgasms but they ejaculate into the blad-
der. Theoretically for these patients to have
children, it will require special maneuvers to
retrieve the sperm from the urine followed by
artificial insemination.
When the clinician makes the diagnosis of the
recto-bladder neck fistula type of malformation,
he/she should be aware of the fact that he/she is
dealing with a patient with a potential serious
urologic condition.
11.2.4 Gastrointestinal-Associated
Defects
The incidence of esophageal atresia is 15 %,
Meckel’s diverticulum 2 %, and abdominal wall
defects 4 %.
165
11.2.5 Neurosurgical-Associated
Defects
The incidence of tethered cord in these patients is
32 %. In addition, 5 % of the patients suffer from
other neurosurgical conditions, including a
lipoma and blunted conus.
11.2.6 Cardiovascular-Associated
Defects
Fifteen percent of these patients are born with an
atrial septal defect and 10 % with a patent ductus
arteriosus, 5 % with tetralogy of Fallot, 2 % with
tricuspid atresia, and 2 % with pentalogy of
Fallot.
11.2.7 Other Associated Defects
Five percent of these patients suffer from hand
abnormalities and 5 % from lower extremity
abnormalities (equinovarus).
11.3 Diagnosis
We must suspect the presence of these very
complex malformations when we see a new-
born baby with imperforate anus and flat
bottom. The midline groove in between the
buttocks that we see in normal children is not
present. In addition, we frequently see in these
patients a sphincter mechanism located right at
the base of the scrotum (Fig. 11.4). The
presence of a bifid scrotum (Fig. 11.5) also
suggests that the malformation that the baby
has is rather complex, most likely very high
(recto-bladder neck fistula). The diagnosis is
confirmed with a distal colostogram and
subsequently by cystoscopy that is performed
at the time of the main repair. The distal colos-
togram can be performed after the colostomy is
opened (Fig. 11.6).
166 11 Recto-bladder Neck Fistula

Fig. 11.6 Distal colostogram showing a bladder neck

fistula

Fig. 11.4 Sphincter located next to the scrotum, fre-

quently seen in cases of bladder neck fistula. Arrow show-
11.4 Treatment
ing the center of the sphincter
11.4.1 Colostomy

The type of colostomy that we recommend for

patients with recto-bladder neck fistula is the
same one recommended for the other types of
anorectal malformations. However, emphasis
must be placed on being sure that enough length
of distal colon is left beyond the mucous fistula
(distal stoma) (Fig. 11.7), in order to have enough
length of bowel for the pull-through without inter-
ference by the colostomy (Fig. 11.8). The fact that
these patients have the highest of all defects
means that the surgeon will need more length of
bowel for the pull-through. Unfortunately, in this
particular type of defect is where we have seen
more often the most common type of error in
making a colostomy (making the stoma too distal
in the bowel), leaving a very short piece of bowel
for the pull-through (Fig. 11.8).

11.4.2 Main Repair

We perform these operations as soon as we see

that the baby is growing and developing nor-
Fig. 11.5 Bifid scrotum, frequently seen in bladder neck mally. If the baby happened to be full term, had a
fistula good colostomy, and did not have important
11.4 Treatment
associated defects that interfered with his growth
and development, then the patient can be oper-
ated, at our institution, within a month after the
Fig. 11.7 Distal colostogram showing a good length of
bowel left distal to the colostomy
a b
Fig. 11.8 Distal colostogram showing a very short piece of bowel distal to the colostomy (insufficient length for a
pull-through). (a) Diagram. (b) Image
167
baby is born. However, as we previously men-
tioned, many of these patients come to our insti-
tution when they are much older, and that is why
we have experience with the main repair at differ-
ent ages. These patients should never be
approached surgically without a good-quality,
high-pressure distal colostogram that shows how
much bowel is available distal to the stoma as
well as the exact location of the fistula.
If we are dealing with a patient that has a very
short piece of bowel distal to the stoma, there is a
reason to believe that we will not have enough
bowel for the pull-through and that we may have
to mobilize the proximal stoma. This is extremely
important because knowing this in advance will
allow us to plan an adequate procedure. More
specifically, we have to prepare the entire gastro-
intestinal tract (administration of GoLYTELY;
see Chap. 7). On the other hand, if the distal
colostogram shows that we have enough distal
bowel from the mucous fistula, then we are cer-
tain that we will not be disturbing the proximal
stoma, and all that the patient needs is irrigation
of the distal stoma in preparation for the main
repair.
168
The fact that the patient has a recto-bladder
neck fistula means that we have to go into the
abdomen either by laparotomy, laparoscopy, or
both, in addition to the posterior sagittal approach.
We start the operation by putting the patient in
the lithotomy position and performing a cystos-
copy. The cystoscopy is extremely valuable. We
have been learning a great deal about the anat-
omy of the male urethra, bladder neck, and tri-
gone in these patients. As previously mentioned,
it is not uncommon to find that these patients
have no verumontanum located in the posterior
urethra. Rather than that, we find the verumonta-
num located in the trigone. In retrospect, now we
have an explanation for the adult patients that
were born with these kinds of defects and have no
ejaculation. Further studies demonstrate that they
actually ejaculate in the bladder. This must be
differentiated from the concept of retrograde
ejaculation. We are referring to a patient that has
the verumontanum located in the trigone and
ejaculates directly into the bladder. This is dem-
onstrated later in life, by finding sperm in the
urine after an ejaculation, as well as the cystos-
copy (Fig. 11.3). It is not unusual in this type of
patients to find, also, ectopic ureters. We have
learned that the higher the malformation, the
more chances of the patient to have ectopic ure-
ters. The ectopia in this type of defect usually
means that the ureters are located closer to the
bladder neck or even below the bladder neck into
the posterior urethra. Sometimes, the ureters
a b
Fig. 11.9 Total body preparation – diagram. (a) Supine.
(b) Legs up. (c) Sequence of photographs of bowel prepa-
ration. (a) Holding legs up. (b) Cautery plate up in the
back. (c) Packing gauze in proximal stoma. (d) Wash and
11 Recto-bladder Neck Fistula
open in the bladder neck and provoke either vesi-
coureteral reflux, ureterovesical obstruction, or
urinary incontinence. When the ureter is ectopic
and located into the posterior urethra, usually it is
associated with a severe stricture, megaureter,
and severe renal damage (Fig. 11.2). It is com-
mon for these patients to end up with a nephrec-
tomy. The location of the fistula is frequently
visualized at the bladder neck with the cysto-
scope. A Foley catheter is placed in the bladder.
11.4.3 Laparotomy
A total body preparation is performed on these
patients (Fig. 11.9). This means to wash, prep,
and drape both lower extremities, the perineum,
buttocks, perianal area, lower abdomen, and lum-
bar portion; in other words, the entire lower body
is included in the sterile field. The cautery plate is
placed in the back of the patient and is protected
with a plastic drape. The arms of the patient are
placed in the upward position because they belong
to the nonsterile part of the field. Both legs of the
patient are covered with stockinettes or with an
elastic bandage to avoid loss of temperature. The
proximal stoma of the colostomy is packed with
packing gauze impregnated with an antiseptic
solution to avoid contamination. The skin of the
abdominal wall is covered with a plastic drape.
We can start the operation either from below
or through the abdomen. We more often now
prep the entire body below the chest. (e) Wash and prep
the back. (f–h) Sterile sheets on table. (i, j) Covering sto-
mas (k) Foley catheter inserted
11.4 Treatment 169

c
ca cb

cd

cc

cf

ce

ch

cg

Fig. 11.9 (continued)

170
ci
ck
Fig. 11.9 (continued)
approach the abdomen first which can be done by
laparotomy or laparoscopy. We consider this par-
ticular defect to be a good indication for a laparo-
scopic approach. More information related with
the indications for laparoscopy in anorectal mal-
formations can be found in Chap. 13. The abdo-
men is entered through a midline incision running
from the umbilicus down to the pubis. A needle-
tip cautery is used changing from cutting to coag-
ulation to provide meticulous hemostasis. The
peritoneal cavity is entered. The urachal remnant
and obliterated umbilical arteries are identified
and divided. A clamp is placed on the urachal
remnant of the bladder to apply caudal traction.
The lateral avascular attachments of the bladder
to the abdominal wall are divided with cautery to
have easy access to the lower pelvis. By pulling
on the bladder out of the abdomen and toward the
pubis, caudally, we can see the posterior wall of
the bladder as well as the peritoneal floor, sig-
moid, both vas deferens, and ureters (Fig. 11.10).
11 Recto-bladder Neck Fistula
cj
Both vas deferens seen behind the bladder run
distally toward the bladder neck; the ureters are
seen retroperitoneally, and they also seem to be
running toward the bladder neck. Our specific
recommendation is to place a 4-0 silk stitch on
the anterior wall of the sigmoid to apply traction.
About 1 or 2 cm from the peritoneal floor, the
serosa of the anterior wall of the sigmoid is
divided in order to create a plane of dissection as
close as possible to the bowel wall, but without
damaging it. This plane of dissection is followed
all around the bowel, separating the mesenteric
fat from the sigmoid. Once we have created a
plane all around the bowel, a Silastic vessel loop
is passed around the rectum in order to have a
more effective handle for traction (Fig. 11.11a).
Applying traction on the vessel loop, it is very
easy to continue a circumferential dissection of
the bowel distally. Very soon, within a centimeter
or two from our initial dissection, one can appre-
ciate that the bowel decreases in size and becomes
11.4 Treatment 171

a b

Fig. 11.10 View of the peritoneal floor in a bladder neck fistula. (a) Diagram – b bladder, r rectum, u ureter, v vas
deferens. (b) Photograph – c colon, b bladder

a b

Fig. 11.11 Distal rectum dissected down to the fistula. (a) Vessel loop surrounding the fistula. (b) Sutures to close to
the fistula

very narrow, indicating that it is reaching the the end of the fistula and the beginning of the uri-
bladder neck. One does not have to be very pre- nary tract. Once the rectum starts being narrow, it
cise in trying to determine exactly the location of reaches a point where the diameter is about
172 11 Recto-bladder Neck Fistula

3–4 mm, obviously not useful for the reconstruc-

tion of an anus, and, therefore, that means that we
can divide the rectum right there. We must keep
in mind that because we are applying traction on
the bowel, there is a possibility that we are kink-
ing the bladder neck or the upper posterior ure-
thra, and therefore, when we divide the fistula,
actually, we will be dividing the bladder neck or
the posterior urethra. Therefore, the traction must
be gentle. Two 5-0 Vicryl stitches are placed in
both sides of the fistula site in order to avoid
retraction, and the fistula is divided. The distal
end of the rectum is also sutured with a running
5-0 Vicryl to avoid contamination from mucus or
meconium previously left in the bowel. The fis-
tula is closed with three to five 5-0 Vicryl sutures
(Animation 11.3) (Fig. 11.11b).
Once the rectum has been separated, the next
step is to divide the avascular attachments of the
distal rectum to evaluate and determine the loca-
tion of the mesenteric vessels (Fig. 11.12). At this
stage, it is very easy to appreciate that the main
limitation for the pull-through of the rectum is its
blood supply provided by the branches of the
inferior mesenteric vessels.
Traditionally, we surgeons learn that we can
mobilize different parts of the colon (up to the
neck or down to the perineum) provided we are
familiar with the blood supply of the colon in Fig. 11.12 Photograph of rectum separated from the uri-
nary tract, very high, does not reach the perineum
normal individuals, which is represented by
three main sources: (1) the ileocecal vessels, (2)
the mesocolic vessels, and (3) the left colic ves- sels. We can do the same with the ileocolic on
sels (Fig. 11.13). Once the rectum goes below the right side. This is a general notion. However,
the peritoneal reflection, its blood supply is pro- we must warn surgeons about the limitations that
vided by the hemorrhoidal vessels, which are this concept has in patients with anorectal mal-
branches of the internal iliacs. We also know that formations. We must keep in mind that we are
the three main vessels that irrigate the colon are dealing with patients whom already had a colos-
intercommunicated by a vascular arcade. As a tomy. Most of the time, the opening of a descend-
consequence, we can easily divide, let’s say, the ing or sigmoid colostomy included the ligation
middle colic vessels without interrupting the of the colonic vascular arcade. This means that
blood supply of the rest of the colon, provided the most distal portion of the rectosigmoid
we preserve intact the other two sources of blood receives all of its blood supply from the inferior
supply (ileocolic and left colic) and the vascular mesenteric vessels. The obvious recommenda-
arcade that intercommunicates the three sys- tion is do not ligate the inferior mesenteric ves-
tems. We can equally divide the left colonic and sels and to bring the rectosigmoid down because
inferior mesenteric vessels, preserving the doing that may represent the loss of that bowel
arcade; the most distal part of the colon will sur- (Fig. 11.14). We have learned that fortunately,
vive receiving blood from the middle colic ves- the rectum has an excellent intramural blood
11.4 Treatment
c
a
Fig. 11.13 Diagram showing the normal blood supply of
the colon. (a) ileocecal vessels, (b) Middle colic vessels,
(c) Left colic vessels. A vascular arcade, joins the three
systems
supply, which allows sacrificing all of its extrin-
sic vessels without compromising its vascularity
provided the bowel wall is maintained intact and
the inferior mesenteric vessels are not ligated. In
other words, we can ligate several peripheral
branches of the inferior mesenteric vessels,
being sure to preserve at least one or two proxi-
mal branches (Fig. 11.15 and Animation 11.3).
All this, provided we maintain intact the integ-
rity of the rectal wall. Damaging the rectal wall
interferes with the intramural blood supply, and
the distal blood supply suffers. Every time we
ligate one of the peripheral branches of the infe-
rior mesenteric vessels, we do it very close to the
rectal wall, and that allows us to gain length. We
must be sure to visualize that at least one good
branch from the inferior mesenteric vessels
remains intact, reaching the bowel wall and that
the bowel wall remains intact; by doing that, the
bowel blood supply is going to be good.
Following those recommendations, we have
been able to pull down all of these rectums, even
173
Fig. 11.14 Diagram showing the blood supply of a rec-
tosigmoid in a case with a colostomy and ligated vascular
colonic arcade
when they were located very high (Figs. 11.15
and 11.16).
When we separate the bowel from the bladder
neck, as previously mentioned, we divide the
avascular attachments of the bowel in order to
identify the mesenteric vessels. It is very easy to
pull on the bowel and identify exactly what is
limiting the pull-through. At the beginning, one
can see that what is limiting us are the vessels,
and we can selectively ligate the peripheral
branches of the mesenteric vessels as close as
possible to the bowel and see how we gain more
and more length until we have the necessary
length for the pull-through. However, occasion-
ally in the process, we find that we are no longer
limited by the vessels but rather limited by the
colostomy itself. Under those circumstances, we
must take the colostomy down. To do that, mul-
tiple 5-0 silk stitches are placed at the mucocuta-
neous junction of the mucous fistula. If the patient
has a loop colostomy (which we consider
contraindicated in anorectal malformations),
then, unfortunately, we had to take down the
entire colostomy, which makes the procedure
more complex. If, on the other hand, the patient
174 11 Recto-bladder Neck Fistula

a b

Fig. 11.15 Intraoperative photographs showing how to evaluate the blood supply of a very high rectum. (a) Before
dividing vessel. (b) Dividing vessels, preserving the arcade. (c) Gained length

has separated stomas, we only have to take down
the mucous fistula. By doing that, we may have
enough length of bowel to reach the perineum.
We can go ahead with the pull-through and decide
whether to leave the upper part of the pulled-
through bowel closed, as what is called
“Hartmann pouch,” or to close the colostomy and
do the pull-through, leaving the patient without a
protective colostomy and a colonic anastomosis
in the pelvis (Fig. 11.19). If one decides to leave
it as a “Hartmann pouch,” we want to be sure that
the length of the distal bowel is enough for the
blind upper end of the bowel to be found above
the peritoneal reflection at the time of the colos-
tomy closure; otherwise, it may become a techni-
cally demanding type of procedure.
In order for us to learn whether or not there is
enough length of colon for the pull-through, we
can open from below and see exactly if we have
enough length passing the rectum behind the pos-
terior urethra. If the perineum has not been
opened yet, we can guess whether or not there is
enough length by pulling the bowel outside of the
abdomen caudally toward the genitalia. We have
learned that we have enough distal bowel to reach
the perineum if the distal end of the bowel reaches
about 4 cm below the lower edge of the pubic
bone (Fig. 11.16). If we do not have this kind of
length, that means that we have to work more on
the blood supply or to take down the colostomy
in order for the bowel to reach.
The perineal approach can be done in two
ways. One is simply lifting the legs up, putting a
bulky roll below the pelvis of the patient. By
doing that, the perineum of the patient is well
exposed, horizontally, and we can work comfort-
ably (Fig. 11.17c). The incision that we make in
these patients does not have to be a full-length
posterior sagittal one. An incision that runs from
the base of the scrotum and about 5 or 6 cm
posteriorly usually provides plenty of exposure
to create a safe abdominal perineal path. Our
11.4 Treatment
a b
Fig. 11.16 How to gain length in a case of a very high
rectum. (a) Diagram – divide peripheral branches of the
inferior mesenteric vessels. Maintain intact rectal wall.
incision goes through the skin, parasagittal
fibers, muscle complex, and levator mechanism.
However, these patients often have very poor
sphincter mechanism, and sometimes it is very
difficult to identify each one of the components
of the sphincter mechanism. In addition, we find
different degrees of “caudal regression.” This
means that the pelvis may be extremely narrow,
175
Blood supply of the rectum is provided by intramural ves-
sels. (b, c) Intraoperative picture of the same maneuver.
(d) Bowel reaches the perineum
making a very difficult task to accommodate a
rectum through it. The entire procedure is done
with a Foley catheter in place. In the process of
opening the perineum, we frequently stop to pal-
pate the catheter in the urethra located in the
deepest portion of the “V” formed by the pubic
bones. As we progress deeper through the poste-
rior sagittal incision, after we have divided the
176 11 Recto-bladder Neck Fistula

b c

Fig. 11.17 Diagram showing the pull-through. (a) Pulling the rectum. (b) Rectum pulled down. (c) Photograph show-
ing the approach to the perineum. Legs up. (d) Posterior sagittal incision. (e) Rectum pulled down. (f) Anoplasty
11.4 Treatment
d e
Fig. 11.17 (continued)
entire striated sphincter mechanism, we find a
whitish fascia which represents the entrance to
the abdominal cavity. A safety path is obtained
remaining as much as possible in the midline.
Once we enter into the abdomen, we pass a
clamp to grasp the distal end of the bowel when
dealing with a laparoscopic approach or to grasp
the sutures holding the distal rectum when the
abdomen was opened (Fig. 11.17). Looking
from the abdominal side, we must remain away
from the ureters and the vas deferens. This space
has to be wide enough to avoid compression of
the rectum. The rectum then is pulled under
direct vision, and the anoplasty is performed
177
within the limits of the sphincter as previously
demonstrated in the other chapters. Sometimes,
in these operations, we find a minimal amount of
a sphincter mechanism, and therefore, the loca-
tion of the anus is determined in a rather arbi-
trary way. Many of these patients will have a
poor prognosis anyway, due to the lack of a
sphincter, poor sacrum, tethered cord, and other
spinal abnormalities.
Prior to the abdominal closure, we must close
the defect created between the mesentery of the
pulled bowel and the posterior abdominal wall
(Fig. 11.18). We had experience with two cases in
whom that space was left open and the patients
178
Fig. 11.18 Closing the mesenteric defect
suffered from intestinal obstruction within the
first 5 days postoperatively. They required an
emergency laparotomy to reduce multiple loops
of small bowel which were trapped in that defect.
Also, before we pull the colon through, we
evaluate the degree of dilatation of the rectum; if
it is considered to be too bulky, it must be tapered
resecting a portion of the posterior wall of the
rectum and closing with two layers of interrupted
sutures.
The posterior wall of the rectum must be
anchored to the neighbor tissue with 5-0 long-
term absorbable sutures. If the patient gets a good
muscle complex, the rectum is anchored to the
muscle complex as demonstrated in other malfor-
mations. The anoplasty is performed as previ-
ously described for other malformations.
Another way to do the posterior sagittal por-
tion of the operation consists in packing the
abdominal wound, covering it with a plastic
drape, turning the patient into prone position, and
opening posterior sagittally as previously
described. However, more and more, we do not
11 Recto-bladder Neck Fistula
have to do this, but rather to lift the legs up in the
way we have already described.
The abdominal wall is closed, and the patient
usually starts eating as soon as the colostomy is
working. The patient stays in the hospital 2 or
3 days. If, on the other hand, the patient was left
with no colostomy, he will remain 7–10 days with
nothing by mouth receiving parenteral nutrition.
11.4.4 Laparoscopy
The laparoscopic approach of anorectal malfor-
mation was first proposed by Willital [2]. Then he
was followed and popularized by Georgeson
et al. [3] and many other surgeons who are per-
forming the laparoscopy approach for the treat-
ment of anorectal malformations [4–31]. The
classic and indisputable indication of a laparos-
copy is an operation that requires an abdominal
approach. In other words, the laparoscopy serves
the purpose of minimizing the trauma and the
pain produced by the incision in the abdomen.
Because of this, the laparoscopic approach is
indicated to treat this particular malformation.
The exposure and view of the peritoneal floor
obtained laparoscopically is excellent (see Chap.
13). The dissection of the distal rectum is easily
done, as previously described, until the rectum
becomes narrow. At that point, unfortunately, the
division of the fistula cannot be done as accu-
rately as when it is done with a laparotomy. Yet,
we have not seen complications from the laparo-
scopic ligation of the fistula. It is important to
keep in mind that in this particular type of ano-
rectal malformation (recto-bladder neck fistula),
the rectum reaches the bladder neck in a “T”
fashion. In other words, there is no common wall
between the rectum and the urinary tract located
above the location of the fistula, like it happens in
the cases of prostatic fistula and even more in
cases of rectal urethrobulbar fistula. Our observa-
tions in 1,113 surgical repairs of anorectal mal-
formations in male patients allowed us to learn
that the lower the malformation, the longer the
common wall between the rectum and the urinary
tract. Therefore, in the highest of all defects
(recto-bladder neck fistula), the dissection of the
11.6 Functional Results
rectum is easier, and it does not include the risk
of injuring the urinary tract. In lower malforma-
tions such as rectoprostatic and particularly in
bulbar fistula, the common wall between the rec-
tum and urinary tract is much longer, and there-
fore, it is not that easy simply to ligate the fistula
like in the laparoscopic approach in cases of
recto-bladder neck fistula. That is one of the rea-
sons why we consider the laparoscopic approach
formally contraindicated in patients with recto-
urethral bulbar fistulas.
The separation of the rectum from the bladder
neck and the ligation of the fistula are easy maneu-
vers. The mobilization of the rectum and the cau-
terizing of the vessels to allow the rectum to reach
the perineum without undue tension, on the other
hand, may not be so easy. The burning of selected
mesenteric vessels may not be as accurate as when
it is done with an open abdomen; accidental burn-
ing of important neighbor vessels may occur. In
addition, it is not uncommon to find that the rec-
tum is too bulky to be placed within the limits of
the sphincter. A tapering of the rectum is required
in such cases. It is at this point when sometimes
we have decided not to continue the laparoscopic
approach and go into to a formal laparotomy. In
some cases, we have concluded the entire proce-
dure laparoscopically successfully.
11.5 Special Problems
11.5.1 Dealing with Inadequate
Colostomies (Too Distal)
When the colostomy is located too distal in the
colon, it is technically demanding to bring the dis-
tal rectum down to the perineum preserving its
blood supply. We have learned how to do it, but
sometimes the upper part of the rectum has to be
detached from the abdominal wall in order to be
pulled down. Once the pull-through is completed,
it may occur that the upper end of the rectum ends
up being located in the area of the posterior ure-
thra. If we leave it there, it would become an
impossible task to close the colostomy (Fig. 11.19).
At that point, we have to make a decision. One
possibility would be to resect the distal piece of
179
bowel and take the proximal stoma down as a pull-
through. In general, we do not like to do this
because that means the patient will lose its natural
bowel reservoir which will give him a tendency to
have diarrhea, making the bowel management to
keep him artificially clean more difficult. Another
possibility would be to take the proximal stoma,
separate it from the abdominal wall, close the
colostomy, and pull together down to the perineum,
the distal bowel with what used to be the proximal
stoma attached and anastomosed. If we do some-
thing like that, we have to make a decision to (a)
keep the patient postoperatively without a colos-
tomy, with parenteral nutrition, and nothing by
mouth for 10 days or (b) open a more proximal
colostomy (Fig. 11.20). The decision is a clinical
one and will depend on how secure the surgeon
feels about the blood supply of the distal rectum
and the surgical technique observed in general. We
have only removed one rectum in cases like this,
because the patient had only a 4 cm portion of
bowel, and if we anastomosed it to the proximal
stoma, the anastomosis would be located too low.
We felt that it was an unnecessary risk to do that
and preferred to pull down the colostomy itself.
When we did that, we had to keep the patient
10 days with nothing by mouth or to open a more
proximal colostomy. We almost never open a
proximal colostomy. We have, rather, kept the
patient with nothing by mouth for 10 days.
Another reason to open the abdomen even in
cases of prostatic or bulbar fistulas is when the
colostomy interferes with the pull-through of the
rectum because this has been created too distally.
We have seen this happening very often.
11.6 Functional Results
Our experience includes 110 patients. The func-
tional evaluation is only done in patients older than
3 years of age and that have been in touch with us.
11.6.1 Fecal Control
Forty-seven patients were evaluated after the age
of 3, and we found that 12 of them (25 %) had
180
Fig. 11.19 Diagram showing the pull-through of a very short piece of rectosigmoid, which will make the colostomy
closure a very difficult operation
voluntary bowel movements. Ninety percent of
these patients soiled occasionally in the underwear.
Only 10 % were totally continent. All patients that
received bowel management were kept totally
clean in the underwear. Among 21 patients with
sacral ratio of 0.7 and up, 7 (33.3 %) had voluntary
bowel movements. Patients with sacral ratio of
0.41–0.69, (20 %) had voluntary bowel move-
ments. None of the patients with a sacral ratio
lower than 0.4 had voluntary bowel movements.
11.6.2 Urinary Control
Forty-nine patients were available to evaluate uri-
nary control. Thirty-nine of them (78 %) had uri-
nary control. Among 19 patients with sacral ratio
higher than 0.7, 13 (68.4 %) had urinary control.
Twenty-two patients had a sacral ratio of
11 Recto-bladder Neck Fistula
0.4–0.69, and 18 of them (81.8 %) had urinary
control. When the sacral ratio was less than 0.4,
three out of 13 patients (23 %) had urinary con-
trol. The fact that a significant number of patients
have urinary control does not mean that the uri-
nary tract is working properly. Some patients
have urinary control but cannot empty the blad-
der well. In addition, a significant number of
cases with this malformation suffer from vesico-
ureteral reflux. That explains why a significant
number of patients 17/54 (31 %) are treated with
clean intermittent catheterization.
We consider it extremely important to alert
and to warn the parents about the future of these
babies as soon as we make the diagnosis of recto-
bladder neck fistula. This is extremely important
in order to adjust the expectations of the parents
concerning the future of the baby and to avoid
further frustration.
References
Fig. 11.20 Diagram showing a pulled-through short
piece of rectum with a colostomy closure and opening of
a more proximal colostomy
Once we make the diagnosis of this malfor-
mation, we tell the parents what we know about
the future bowel function of the baby, and at the
same time, we tell them that we will always be
there to help them. We offer them our bowel
movement program to be started when the
patient is 3 years old in order for the patient to
go to school like a normal child with normal
underwear and to be adapted and accepted into
the society.
When the patient has vesicoureteral reflux,
we leave a suprapubic cystostomy tube at the
time of the main repair. Prior to the colostomy
closure, we perform a suprapubic cystogram to
determine the presence and magnitude of
the reflux. Also, a urodynamic evaluation will
help to determine the best urologic future
management.
181
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4. Yamataka A, Segawa O, Yoshida R (2001)
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182
17. Vick LR, Gosche JR, Boulanger SC (2007) Primary
laparoscopic repair of high imperforate anus in neona-
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5/s-2007-965507
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11 Recto-bladder Neck Fistula
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J Pediatr Surg 45:1500–1504
 Imperforate Anus Without Fistula
in Males and Females 12

12.1 Introduction tion with other neurologic defects such as absent

corpus callosum.
Imperforate anus without fistula is a very unique Approximately 1.1–2.2 % of patients with
kind of defect (Fig. 12.1). Most likely, it has a Down syndrome have an anorectal malformation
very different embryologic origin, considering its [2–4], and the overwhelming majority has no
uniqueness, type of associated defects, and intrin- fistula.
sic anatomy. In the spectrum of anorectal malfor- The association of Down syndrome and ano-
mations, one can see that the frequency of rectal malformation without fistula may occur in
associated defects is very much related with the identical twins [5].
height of the location of the fistula. Thus, a peri- The literature, prior to 1980, reported a high
neal fistula type of malformation usually is rarely incidence of this defect [6]. Stephens reviewed
associated to other defects, whereas a recto-blad- his own series and those of eight authors and
der neck fistula seems to have the highest inci- found that approximately 10 % of all cases of
dence of associations with other malformations ARM suffered from this condition. We believe
mainly in the urinary tract. From that point of
view, an imperforate anus with no fistula repre-
sents an exception because it is extremely unusual
for these patients to have other associated defects
such as tethered cord and abnormal sacrum.
The most important feature of this particular
defect is the fact that it represents approximately
4 % of all anorectal malformations, and half of
the cases are Down syndrome [1]. In addition,
from all patients with Down syndrome suffering
from anorectal malformations, over 95 % of them
have this specific type of defect. This fact repre-
sents a fascinating and unique opportunity to
speculate and investigate anorectal malforma-
tions from the genetic point of view. It is also
interesting to know that, as mentioned before,
half of the anorectal malformations without fis-
tula suffer from Down syndrome and the other Fig. 12.1 Diagram showing the intrinsic anatomy of
half seem to have a higher incidence of associa- imperforate anus without a fistula

A. Peña, A. Bischoff, Surgical Treatment of Colorectal Problems in Children, 183

DOI 10.1007/978-3-319-14989-9_12, © Springer International Publishing Switzerland 2015
184 12
a b
Fig. 12.2 Distal colostogram done in a patient without a
fistula. (a) The lack of hydrostatic pressure gives the false
impression that the rectum ends at the pubococcygeal line.
that those series included many misdiagnosed
cases, in whom a fistula was not demonstrated
due to a lack of adequate imaging technology.
Even at present time, we see cases that come to
our center with a diagnosis of anorectal malfor-
mation “without fistula,” based on a distal colos-
togram done with an inadequate technique (lack
of hydrostatic pressure). When we repeat the dis-
tal colostogram, applying enough hydrostatic
pressure, a fistula is demonstrated (Fig. 12.2).
12.2 Anatomic Characteristics
Figure 12.1 shows a diagram of an imperforate
anus with no fistula. Interestingly, the blind end
of the rectum is located at the same level of the
bulbar urethra in the overwhelming majority of
cases. In the past, the literature referred to imper-
Imperforate Anus Without Fistula in Males and Females
(b) Increased hydrostatic pressure allows us to see the real
location of the blind end of the bowel
forate anus with no fistula, dividing the cases into
“high” and “low” location of the rectum [6].
In our series of 83 cases, 76 of them had the
rectum located at the level of the bulbar urethra.
Five cases had the blind rectum located at the
level of the prostatic urethra, and we have only
seen two blind rectums, floating into the
peritoneum [7]. That is an extremely unusual
defect, and we believe it belongs to another cate-
gory. This is a debatable issue [8].
Some authors suggest that the absence of fis-
tula may contribute for these patients to suffer
from colon perforation earlier than those with fis-
tula [9]. The diagram in Fig. 12.1 shows that the
rectum and the posterior urethra share a long
common wall. This means that the repair of this
malformation is not necessarily easier than the
one of a rectourethral fistula since the surgeon
has to work in that common wall and separate the
12.3 Main Repair
Fig. 12.3 The white fascia. Deeper to the levator mecha-
nism, a white fascia is seen which covers the posterior
rectal wall. Arrow shows the white fascia
rectum from the urinary tract preserving intact
the last one.
12.3 Main Repair
The posterior sagittal approach represents the
ideal way to repair this malformation [1, 7, 10].
A Foley catheter is inserted in the bladder. The
patient is turned into the prone position, with the
pelvis elevated. The skin of the perineum and
both buttocks are washed, prepped, and draped
in the usual manner. A midline incision is done
with the needle-tip cautery running from the
middle portion of the sacrum down to the base of
the scrotum. The incision divides the skin, sub-
cutaneous tissue, parasagittal fibers, and muscle
complex. After the parasagittal fibers have been
divided, we go through the ischiorectal fossa to
find the levator mechanism. When the levator
muscle is divided, a white fascia appears very
clearly (Fig. 12.3). This white fascia must be
divided and separated from the real bowel wall.
185
Fig. 12.4 Rectal lumen exposed in a patient with no
fistula
There is no way to injure the urinary tract in
these patients because the rectum bulges right
through the wound. Two 5-0 silk stitches are
placed taking the posterior rectal wall, and the
wall is opened with the needle-tip cautery. We
continue opening the rectum distally to reach
its blind end, to confirm that there is no fistula.
As we open the posterior rectal wall, 5-0 silk
stitches are placed taking the edges of the rec-
tum to expose the inside lumen of the rectum.
Figure 12.4 shows the lumen of the rectum in
a patient with imperforate anus with no fistula.
The surgeon must remember at this point that the
anterior rectal wall is intimately attached to the
posterior urethra and will require a meticulous
dissection in order to separate the rectum with-
out injuring the urinary tract. For that, multiple
5-0 silk stitches are placed taking the edge of the
rectum in its lowest portion, and the dissection is
done using uniform traction. Applying uniform
traction with multiple silk stitches, a circumfer-
ential dissection is performed dividing bands
and vessels that held the rectum up in the pel-
vis (Fig. 12.5). Since the rectum is located sig-
nificantly low, once it is completely separated,
usually it requires a rather minor dissection to
gain enough length to achieve a low-tension ano-
plasty. In this particular malformation, it is not
uncommon to find a very dilated rectum since the
patient had no fistula. Therefore, the chances for
this patient to require tapering of the rectum are
186 12 Imperforate Anus Without Fistula in Males and Females

a b

Fig. 12.5 Circumferential dissection to gain length. (a) Pulling down. (b) Pulling up

a b

Fig. 12.6 Rectal tapering. (a) Open rectum after resection of its posterior aspect. (b) Reconstructed, tapered rectum

higher than in other cases of anorectal malforma-
tions. This is particularly true in those patients
who have spent a long time, from the time of
colostomy until the time of the main repair. If
the rectum is too bulky, it must be tapered, and
for that, we resect the posterior aspect of the
rectal wall and closed the rectum with two lay-
ers of interrupted long-term absorbable sutures
(Fig. 12.6). The rectum is then placed in front
of the levator mechanism, which is closed with
5-0 interrupted long-term absorbable sutures.
The posterior edges of the muscle complex on
each side of the midline are reapproximated in
the midline and sutured with interrupted 5-0
long-term absorbable sutures, taking a bite of the
posterior rectal wall to anchor the rectum in a
good position. The anoplasty is performed in the
center of the sphincter with 16 circumferential
stitches of 6-0 Vicryl sutures. The ischiorectal
fossa is obliterated with the same suture mate-
rial as well as the subcutaneous tissue, and the
skin is closed with a subcuticular monofilament
absorbable suture. These patients require mini-
mal medication for pain. They can eat and drink
the same day as surgery. The Foley catheter can
be removed the next day. Dilatations are started
2 weeks after surgery according to our protocol.
Montalvo et al. designed an ingenious tech-
nique called “posterior flap anorectoplasty”
[11–13] to repair anorectal malformations
References
through a posterior sagittal approach, but without
separating the rectum from the urinary tract. The
bulging posterior wall of the rectum is used to
create a flap that is tubularized and switched
down to create the anus. We have no experience
with the procedure; however, we believe that it
can be used in patients who had no fistula and
rectum dilated enough to allow this maneuver.
We do not believe that this approach should be
used in patients with fistula, due to the risk of a
recurrent fistula.
12.4 Function and Results
In terms of bowel control, the results are, as
expected, different in patients with Down syn-
drome and patients without Down syndrome. In
our series, patients without Down syndrome have
an 85 % chance of having voluntary bowel move-
ments by the age of 3. This means that this mal-
formation has an excellent functional prognosis.
The incidence of constipation, however, seems to
be higher than in other types of defects, since we
know now that a megarectum relates directly
with the chances of constipation. Surprisingly,
60 % of the patients with Down syndrome have
voluntary bowel movements by the age of three.
Of course, we must remember that Down syn-
drome babies have different degrees of develop-
mental delay, and therefore, we very much
depend on that, to try to predict the chances of
bowel control.
Patients with Down syndrome have a well-
known tendency to be constipated. Patients with
anorectal malformations also have a tendency to
be constipated, provided their original rectum is
not resected and patients with anorectal malfor-
mation with no fistula have a higher incidence of
constipation. Therefore, we must expect these
patients to suffer from severe constipation. After
the colostomy closure, the surgeon should be
very aggressive and proactive in giving the right
amount of laxatives to avoid constipation. When
not treated adequately, this problem may produce
chronic fecal impaction and overflow pseudoin-
continence (see Chap. 25).
We have been surprised to see patients born
with Down syndrome and anorectal malformation
187
without fistula coming to our center with a colos-
tomy that is meant to remain on permanent basis.
Their doctors advised the parents to keep the
colostomy for life, because the patients with
Down syndrome are all incontinent. Our experi-
ence, as presented here, is quite different.
References
1. Torres R, Levitt MA, Tovilla JM, Rodriguez G, Peña
A (1998) Anorectal malformations and Down’s syn-
drome. J Pediatr Surg 33(2):194–197
2. Torfs CP, Bateson TF, Curry CJ (1992) Anorectal and
esophageal anomalies with Down syndrome. Am J
Med Genet 44(6):847; author reply 848–850
3. Zlotogora J, Abu-Dalu K, Lernau O, Sagi M, Voss R,
Cohen T (1989) Anorectal malformations and Down
syndrome. Am J Med Genet 34(3):330–331
4. Heinen F, Bailez M, Solana J (1994) Imperforate anus
and Down syndrome. [El ano imperforado en el
Sindrome de Down]. Rev Cir Infant 4(2):72–76
5. de Buys Roessingh AS, Mueller C, Wiesenauer C,
Bensoussan AL, Beaunoyer M (2009) Anorectal mal-
formation and Down’s syndrome in monozygotic
twins. J Pediatr Surg 44(2):e13–e16. doi:10.1016/j.
jpedsurg.2008.10.116
6. Stephens FD, Smith ED (1971) Chapter 7: Incidence,
frequency of types etiology. In: Ano-rectal malforma-
tions in children. Year Book Medical Publishers, Inc,
Chicago, pp 160–171
7. Bischoff A, Frischer J, Dickie BH, Peña A (2014)
Anorectal malformation without fistula: a defect with
unique characteristics. Pediatr Surg Int 30(8):763–
766. doi:10.1007/s00383-014-3527-5
8. Black CT, Sherman JO (1989) The association of low
imperforate anus and Down’s syndrome. J Pediatr
Surg 24(1):92–94
9. Chan KW, Lee KH, Tsui SY, Wong YS, Pang KY,
Mou JW, Tam YH (2014) Bowel perforation in new-
born with anorectal malformation and no fistula at
presentation. J Pediatr Surg 49(3):390–394. doi:00
10. Fanjul M, Molina E, Cerdá J, Parente A, Laín A,
Cañizo A, Carrera N (2009) Characteristics of the
anorectal atresia without fistula. Based on 12 cases.
Cir Pediatr 22(1):45–48
11. Montalvo-Marín A, Victoria-Morales G (1993)
Anorectoplasty with a distal tubular flap via a poste-
rior sagittal approach. Bol Med Hosp Infant Mex
50(7):499–502. Spanish
12. Zea-Salazar LE, Cabrera-Johnson M (1994)
Anorrectolpastia Sagital posterior y colgajo rectal
[Posterior sagittal anorectoplasty and rectal flap]. Rev
Cir Infant 3:112–114
13. Montalvo-Marin A, del Campo NM, Alvarez-Solís
RM, Diaz-Lira MA (1998) Continence in patients
who undergo posterior rectal flap anorectoplasty. J
Pediatr Surg 33(12):1760–1764
 Minimally Invasive Approach
to Anorectal Malformations
13.1 Introduction
The minimally invasive approach to the repair of
congenital malformation is here to stay. All of us,
pediatric surgeons, are concerned about inflicting
pain and/or being too invasive to our patients.
Every effort aimed to reduce the suffering of our
patients, as well as the length of stay in the hospi-
tal and subsequently the cost of the operations, is
welcomed. We embrace ideas that have, as the
end result, a less traumatic operation without
compromising the standard of care or provoking
more sequelae in our patients. That explains why
the minimally invasive approach to repair malfor-
mations had so much impact in pediatric surgery
and the surgical field in general.
Since the introduction of this new technology,
there are now operations in which the laparo-
scopic or thoracoscopic approach represents the
gold standard. There are others in which these
new approaches have not been applied, and there
is another group in which the approach is contro-
versial. Anorectal malformations have not been
an exception, and many pediatric surgeons have
used and continue trying to use more and more
the laparoscopic approach to the repair of anorec-
tal malformations, many of them with the correct
indication, others in malformations that can be
Electronic supplementary material Supplementary
material is available in the online version of this chapter at
10.1007/978-3-319-14989-9_13.
A. Peña, A. Bischoff, Surgical Treatment of Colorectal Problems in Children,
DOI 10.1007/978-3-319-14989-9_13, © Springer International Publishing Switzerland 2015
13
repaired either way laparoscopically or posterior
sagittally, and others in cases in whom we believe
that laparoscopy is formally contraindicated.
Our basic contention and belief is that laparos-
copy is primarily indicated to replace a laparot-
omy. In other words, a classic indication is a
procedure that is usually done opening the abdo-
men and now can be done equally well, through
the small orifices of the ports necessary for mini-
mally invasive operation. In male patients with
anorectal malformations, we have to open the
abdomen 10 % of the times, specifically in those
cases that have a recto-bladder neck fistula. We
consider that group a good potential indication
for laparoscopy. We use the word “potential”
because even in those particular types of defects,
laparoscopy has certain limitations, as will be
shown later.
Approximately 30 % of patients with cloacas
require a laparotomy. Yet, all of them require a
posterior approach in order to repair the urogeni-
tal component of the malformation. Conceivably,
a laparoscopic approach could be useful to sepa-
rate the rectum from the bladder neck in those
unusual cases that have the rectum connected to
the bladder neck. Yet, those patients require
rather sophisticated and technically demanding
maneuvers to repair the urogenital component of
the malformation (see Chap. 16).
We decided to review the literature related with
the laparoscopic approach of anorectal malforma-
tions. We found 52 papers published between 1998
and 2014. Forty-one of them [1–41] presented
189
Table 13.1 List of authors and cases reported
190

Bladder No Rectal Tethered

Year Authors Cases Comments N/A Urethral neck PR Bulbar fistula atresia Vaginal Vest Perineal Cloaca High Inter Low Sacrum cord
1. 1998 Willital 2 2
2. 2000 Georgeson 10 7 1 1 1 1
3. 2001 Ettayebi 1 1
4. 2001 Yamataka 3 3
5. 2002 Yamataka 6 3 3 Normal in 0
all
6. 2003 Lin 9 5 1 3
7. 2003 Iwanaka 12 1 2 5 1 2 1
8. 2003 Raghupathy 11 8 3
9. 2003 Tei 5 1 3 1
10. 2005 Kudou 13 7 2 1 2 1
11. 2005 Koga 1 1
12. 2005 Kubota 5 2 2 1
13. 2006 Lima 7 6 1 Abnormal
in 1
14. 2006 Hakgüder 4 1 3 Abnormal
in 1
15. 2007 Vick 6 3 3
16. 2007 Liem 2 2
17. 2008 Ichijo 15 5 4 1 1 2 2 Normal in Tethered
all cases in few
cases
18. 2009 El-Debeiky 15 15
19. 2009 Hay 12 12
20. 2009 Bischoff 6 6
21. 2009 Lopez 1 1 Normal
22. 2009 Yang 11 2 3 3 3
23. 2009 Uchida 24 2 15 3 2 2
24. 2009 Yamataka 1 1
25. 2009 Podevin 34 3 20 10 1
26. 2009 Rollins 5 No clips or ties 2 3
used
13 Minimally Invasive Approach to Anorectal Malformations
 13.1

27. 2010 Raschbaum 3 MRI guided 3

28. 2010 Bailez 5 5 1 sacral 1
agenesis
29. 2010 Kimura 13 1 10 1 1
30. 2010 Koga 5 3 2 Normal None
Introduction

31. 2010 Lopez 1 1 No

32. 2011 Wong 18 Does not 18
specify type
or number.
Only “high/
intermediate”
and no numbers
33. 2011 Bailez 17 8 9 50 % in
both groups
had SR
<0.6
34. 2011 De Vos 20 3 13 3 1
35. 2011 Tong 33 1 22 6 4
36. 2012 Miglani 3 2 1
37. 2012 England 24 3 7 9 3 2
38. 2013 Jung 25 6 16 3
39. 2013 Liem 19 15 4
40. 2013 Bischoff 15 13 2 < 0.4 in 2, Tethered
0.4–0.7 in in 5, No
6, > 0.7 in T/C in 8,
6 Unknown
in 2
41. 2014 Ming 24 11 13 Average
0.64 in
prostatic,
and 0.54 in
recto-
bladder
neck
Totals 446 0 21 16 93 185 63 17 2 29 8 2 8 2 0 0
191
192
Table 13.2 Summarized results of a literature review on minimally invasive approach to anorectal malformation
357
males
446 45
cases females
44
gender not
specified
series of cases (Table 13.1). Ten papers [42–52]
were rather informative, written by experts but
without discussing specific cases.
The total number of cases reported in the lit-
erature according to our review is 446. These
included 357 males, 45 females, and 44 in whom
the gender was not specified (Table 13.2).
The group of male cases included 185 recto-
prostatic fistulas, 93 bladder neck, 63 bulbar, and
16 labeled as “urethral” without specifying the
location of the fistulas.
The female group included 29 “vaginal fis-
tula,” 8 vestibular, and 8 cloacas. The group of
patients without gender specification included 21
cases, 17 without a fistula, 2 recto-perineal, 2
“rectal atresia,” and 2 cases labeled simply as
“high” (Table 13.2).
13.2 Males
Anorectal malformations in males are divided
into the following categories:
• Recto-bladder neck fistula
• Rectourethral prostatic fistula
13 Minimally Invasive Approach to Anorectal Malformations
185 prostatic fistula
93 Bladder neck
63 Bulbar
16 “Urethral”
29 “vaginal”
8 Vestibular
8 cloaca
17 without fistula
21 not specified
2 perineal
2 rectal atresia
2 “High”
• Rectourethral bulbar fistula
• Anorectal malformation with no fistula
• Perineal fistula
We believe that mainly those patients with
recto-bladder neck fistulas may benefit from the
laparoscopic approach. In our own experience,
the laparoscopic approach provides an excellent
exposure of the peritoneal floor and the distal part
of the bowel as it approached the bladder neck
(Animation 13.1). The dissection of the rectum,
as well as the ligation of the fistula, is an easy
maneuver. The main reason for this is that in ano-
rectal malformations, the higher the malforma-
tion, the shorter the common wall existing
between the rectum and the urinary tract, and
therefore the rectum ends in the bladder neck in a
T fashion, which makes the ligation of the fistula
an easy task, without any risk of damaging neigh-
boring structures. To repair this malformation,
the next step after the ligation of the fistula is the
mobilization of the rectum. We found that some-
times this is feasible, and the entire procedure
can be completed after making a small incision in
the perineum. Yet, sometimes the mobilization of
the rectum (as explained in Chap. 11) requires a
13.2 Males
delicate and selective ligation of mesenteric ves-
sels, preserving the necessary arcades in order to
preserve the blood supply of the distal rectum.
Laparoscopically, this maneuver is done using
cautery, which is a less than optimal way to
divide vessels, because the extension of the burn
is not controlled accurately and may damage col-
lateral vessels necessary to preserve the blood
supply of the distal rectum. In addition, some of
these patients have a very dilated rectum that
requires tapering. We found difficult to perform
the tapering of the bowel laparoscopically, and
therefore we have to open the abdomen. It is
important to remember that unfortunately, these
groups of patients (recto-bladder neck fistula)
have a very poor functional prognosis. Only
20–25 % of our patients in this category have vol-
untary bowel movements, and about half of those
suffer from soiling. These patients are born with
serious congenital deficiencies in their sphinc-
teric mechanism and frequently are associated to
very poor sacra.
Ninety-three cases of our literature review
belong to this category of recto-bladder neck fis-
tula. We believe that all pediatric surgeons agree
that this malformation represents an excellent
indication for a laparoscopic approach.
Perineal fistulas are malformations that can
be repaired with an operation that takes less
than 45 min with 100 % chance of bowel con-
trol. Therefore, we do not see the reason to
attempt a less invasive procedure than the one
that we do at present time. Yet, we found two
publications that included perineal fistula
repaired laparoscopically [2, 12].
Imperforate anus with no fistula is a malfor-
mation in which the rectum is blind; it has no
communication with the urinary tract. Half of
these patients have Down syndrome. Our experi-
ence shows that this is a benign condition if we
consider the good functional prognosis. Ninety
percent of our patients without Down syndrome
have bowel control, and 70 % of our Down
syndrome patients also have voluntary bowel
movements.
We found that 17 patients (from our literature
review) with this defect underwent a laparoscopic
repair [2, 7, 10, 17, 22, 23, 25, 34, 37]. We believe
193
that patients with anorectal malformation without
fistula should not be approached laparoscopi-
cally. The rectum is located very close to the
perineum, and therefore it requires a minimal dis-
section to be mobilized. A laparoscopic approach
implies in these cases a full dissection of the rec-
tum, which is actually unnecessary. In our series,
the operation takes 2 h, the patients have minimal
pain, the blood loss is negligible, and the patient
can be discharged 24 or 48 h after the operation.
Rectourethral bulbar fistula is fortunately
the most common defect that we see in male
patients. The rectum is located approximately
2 cm above the skin and connects to the bulbar
urethra at the same level of those cases of imper-
forate anus with no fistula. Eighty-five percent
of our patients with normal sacrum have bowel
control, and 94 % of them have urinary control.
The operation takes approximately 2½ h, and the
patients eat the same day, have minimal pain, and
can be discharged the next day. We find it very
difficult to have a less invasive procedure than
the one that we perform. Again, trying to repair
these malformations laparoscopically represents
a maximally invasive approach since the entire
rectum has to be dissected from above. When
these malformations are repaired posterior sagit-
tally, we only dissect the distal part of the rectum
as much as necessary to bring the rectum down;
we do not have to go all the way to the perito-
neum. As a consequence of attempting the repair
of this malformation laparoscopically, a signifi-
cant number of patients suffered from recurrent
rectourethral fistula or a large persistent posterior
urethral diverticulum which represents a piece
of rectum left attached to the urethra [53, 54]
(Animation 13.2) (Fig. 13.1). We believe that
laparoscopically the surgeons found it impos-
sible to reach the bulbar fistula site and ampu-
tated the rectum leaving a piece of rectum that
later gave significant symptoms. We believe that
the laparoscopic approach of this malformation
is contraindicated. Unfortunately, many surgeons
keep trying [5, 6, 11–13, 17, 24–26, 30, 34, 35,
37–39].
Rectourethral prostatic fistula is the second
most common malformation that we have seen in
male patients. For us, this malformation
194 13 Minimally Invasive Approach to Anorectal Malformations
a b
Fig. 13.1 Posterior urethral diverticulum. (a) Diagram. (b) MRI study showing a giant posterior urethral
diverticulum
represents a source of controversy concerning the
best way to approach it. The posterior sagittal
approach in these malformations renders a 66 %
chance of bowel control by the age of three.
Ninety-two percent of the patients have urinary
control. In most of these patients, the posterior
sagittal dissection of the rectum requires to open
the peritoneum. In other words, the dissection is
a complete one, due to the fact that the rectum is
located high in the patient’s pelvis. If a surgeon is
particularly well trained laparoscopically and
prefer to approach this type of malformation lap-
aroscopically, rather than posterior sagittally, we
do not see a real difference between going one
way or the other. In addition, some of the recto-
prostatic fistulas are located a little higher than
others closer to the bladder neck; in those cases,
we have seen complications when inexperienced
surgeons try to repair this malformation via pos-
terior sagittal. Perhaps, if they have good training
in laparoscopic techniques, it will be better for
them to approach those malformations that way.
We were happy to find in our literature review
that 185 out of 357 male cases operated laparo-
scopically had this kind of defect.
Unfortunately, we found in our literature
review that many surgeons still use the old termi-
nology that we consider inadequate and mislead-
ing. They still refer to “high, intermediate, and
low” malformations. Sixteen of the published
cases received the nonspecific diagnosis of
“rectourethral fistula” [7, 10, 13, 22]. Operating a
male baby with an anorectal malformation
without knowing the detailed anatomy represents
a potential misadventure with serious conse-
quences for the patient.
13.3 Females
Anorectal malformations in females are divided
into the following categories:
• Perineal fistula
• Vestibular fistula
• Cloacas
• Complex malformations
Perineal fistulas in females are repaired with a
minimal posterior sagittal operation that takes
less than an hour; the patients have minimal pain,
100 % of them have bowel control, and therefore
we do not see a justification to do a maximally
invasive operation dissecting the entire rectum
through the abdomen to repair these malforma-
tions. Yet, as previously mentioned, some cases
are occasionally done laparoscopically.
Vestibular fistulas are by far the most common
defects in female patients with anorectal malfor-
mations. In our series, 95 % of these patients with
a normal sacrum have bowel control. The opera-
tion to repair this defect takes us approximately
2½ h. The patients can eat the same day when
operated with a colostomy and can be discharged
13.3 Females
the next day after surgery. The most important
anatomic feature in these patients is the common
wall existing between the rectum and the vagina
(see Chap. 15). The real challenge in the repair of
these malformations is to make two walls out of
one, preserving intact the posterior vaginal wall
and the anterior rectal wall. It is difficult for us to
imagine why somebody wants to dissect the
entire rectum through the abdomen, in order to
repair these malformations and still claim that it
is less invasive [9, 17, 36, 37]. In some of those
patients in whom the surgeons attempted a lapa-
roscopic repair, actually, they left a piece of rec-
tum attached to the vestibule and mobilized more
colon from above, which is against the basic prin-
ciples of the repair of anorectal malformations.
Cloacal malformations are the second most
common anorectal malformations seen by us in
females. The repair of a cloaca requires separa-
tion of the rectum from the urinary tract followed
by the mobilization and repair of the urethra and
vagina. For that, most of the time we use a maneu-
ver called total urogenital mobilization. There are
reports of laparoscopic attempted repairs of clo-
acas [7, 9, 10, 17, 23, 29]. The authors actually
only repaired the rectal component of the malfor-
mation, and then they performed a posterior sagit-
tal approach from below in order to repair the
urogenital component of the malformation. In
other cases the surgeons repaired the rectal com-
ponent and left the urogenital tract intact! In other
words, we cannot say that the laparoscopic
approach has been used to repair cloacas, but,
rather, we should say that it has been used to
repair the rectal component of cloacal malforma-
tions. Yet, in our experience, in over 500 cloacas,
the rectum can be reached posterior sagittally in
the majority of cases; therefore, we do not see a
reason to try to go through the peritoneal cavity to
repair a malformation that can be repaired from
below. Again, in theory, laparoscopy could be
helpful to mobilize a rectum that can only be
mobilized through the abdomen. Yet, the patient
still will require a posterior sagittal approach any-
way to repair the urogenital sinus.
Laparoscopy can also be useful instead of a lapa-
rotomy for the examination of the intra-abdominal
anatomy in complex anorectal malformations.
195
We are obviously biased against the laparo-
scopic approach of cases with rectourethral bul-
bar fistula because of the cases that we see at our
center, suffering from a complication consecutive
to the laparoscopic approach, including urethral
injuries and posterior urethral diverticula
[53, 54].
We are aware of the fact that these complica-
tions are not necessarily a consequence of the
laparoscopy, since we have seen similar compli-
cations occurring during the repair of anorectal
malformations with other techniques. However,
these complications occurred mainly in patients
suffering from rectourethral bulbar fistulas.
In our literature review, we found that 29
patients were operated laparoscopically with the
diagnosis of rectovaginal fistula [2, 3, 6–10, 17,
23, 28, 29, 34–36].
We consider that real vaginal fistula is an
almost nonexistent malformation. In our series
of over 1,000 female patients, we have seen only
seven real rectovaginal fistula cases (see Chaps.
15 and 16). Most of the cases that came to us
with the diagnosis of “rectovaginal fistula,” in
retrospect, were misdiagnosed cloacas or rec-
tovestibular fistula. If these authors found 29
vaginal fistulas out of 45 female cases, we have
reason to suspect that they were not really vagi-
nal fistulas.
Some authors [10, 17, 35] try to evaluate the
clinical results using an old score that includes
“number of bowel movements” as indicative of
results; “many bowel movements” were consid-
ered as bad and “few bowel movements” were
considered “good”!! The number of bowel move-
ments relates to many factors (diet, length of the
colon, inflammatory factors, degree of dilatation
of the rectosigmoid); none of them related to the
type of repair (laparoscopic or posterior
sagittal).
We were negatively impressed by the fact that
only eight of the papers that we reviewed men-
tioned the characteristics of the sacrum of the
patients [5, 13, 14, 17, 21, 28, 30]. We think that
it is impossible to discuss functional results in the
treatment of anorectal malformations without
specifying the characteristics of the sacrum of the
patients. The presence or absence of tethered
196 13 Minimally Invasive Approach to Anorectal Malformations
cords was only mentioned by five authors [2, 4,
17, 28, 40]. The presence of this anomaly is rec-
ognized as a negative prognostic factor, mainly
for the urinary function.
One of the rationales to justify the laparo-
scopic approach in patients with anorectal mal-
formations is the short length of stay. We were
very surprised to find that the length of stay was
only mentioned in three papers [22, 35, 41], and
the authors reported 5–11 days’ range of length
of stay. In our series, 90 % of our male cases did
not require a laparotomy, and they remained in
the hospital 48 h postoperatively. In females,
with the exception of complex cloacas that
required a laparotomy, the length of stay was
similar to the males.
Ten publications [6, 10, 17, 22, 32–35, 37, 41]
compared the functional results obtained with
laparoscopy and the traditional PSARP (posterior
sagittal anorectoplasty). Most of the authors con-
cluded that the differences found were not sig-
nificant. Others suggested that the results
obtained with the use of laparoscopy are better
[17, 41].
Four of the papers [6, 10, 22, 35] evaluated
their results using rectal manometry, which we
consider a non-reliable method (see Chap. 25).
Some authors mentioned the “potential nega-
tive effect of the scarring produced by the poste-
rior sagittal approach.” That is a reasonable
theoretical concern; however, we strongly sug-
gest for those authors and the readers to read our
publication [55] describing our experimental
experience (in dogs), comparing different types
of rectal operations and their effect on bowel con-
trol. The posterior sagittal incision did not affect
bowel control. In addition, from the clinical point
of view [56], we have operated multiple cases
with normal rectum that required a complete
division of the rectum and anus, including the
posterior and anterior sphincter mechanism (what
we call trans-anorectal approach), and bowel
control was not affected. All patients have nor-
mal bowel control.
We have not found conclusive evidence that
the laparoscopy is less invasive than the posterior
sagittal approach for the repair of anorectal mal-
formations in patients that do not require a
laparotomy. There is no current evidence of bet-
ter clinical results. There are a significant number
of cases in which laparoscopy has been used with
no real justification.
We are happy to see that many authors recog-
nize the risk of urethral damage and leaving a
posterior urethral diverticulum and are showing a
high degree of creativity, using sophisticated
technology to avoid these complications [12, 24,
30]. Others are already limiting the use of lapa-
roscopy to cases with bladder neck or prostatic
fistula [37].
We strongly suggest to all surgeons interested
in performing laparoscopic repair of anorectal
malformations to follow the following
guidelines:
• Use an adequate terminology; try to be accu-
rate in the type of malformation treated.
• Refer to the characteristics of the sacrum as
well as the presence or absence of tethered
cord, in order to discuss clinical results.
• Avoid confusing terms such as “high,” “inter-
mediate,” and “low.”
• When discussing cloacal approach laparo-
scopically, be specific as to “repair of a clo-
aca” or repair of the “rectal component of the
cloaca.” Be specific in saying exactly what
was done with the urogenital component,
which represents the real technical challenge.
We firmly believe that minimally invasive
techniques will play an increasing positive role in
the management of many congenital anomalies
including of course anorectal malformations.
However, we must be cautious to avoid negative
results as a consequence of excessive enthusiasm
or the desire of notoriety.
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treatment of anorectal malformations. Pediatr Med
Chir 28(4–6):79–82
44. Georgeson K (2007) Laparoscopic-assisted anorectal
pull-through. Semin Pediatr Surg 16(4):266–269
45. Albanese CT (2000) Literature review and comment.
Alight at the end of the tunnel? Pediatr Endosurg
Innov Tech 4:251
46. Srimurthy KR, Ramesh S, Shankar G, Narenda BM
(2008) Technical modifications of laparoscopically
assisted anorectal pull-through for anorectal malfor-
mations. J Laparoendosc Adv Surg Tech A 18(2):340–
343. doi:10.1089/lap.2006.0247
47. Grapin-Dagorno C, Fayad F (2008) Surgical treat-
ment of high-type imperforate anus: role of laparos-
copy. Bull Acad Natl Med 192(5):913–918; discussion
918–919
48. Al-Hozaim O, Al-Maary J, AlQahtani A, Zamakhshary M
(2010) Laparoscopic-assisted anorectal pull-through for
anorectal malformations: a systematic review and the need
for standardization of outcome reporting. J Pediatr Surg
45(7):1500–1504. doi:10.1016/j.jpedsurg.2009.12.001
49. Yamataka A (2011) Correspondence reply to letter to the
editor by Hamrick et al. J Pediatr Surg 46:1018–1019
50. Japanese Multicenter Study Group on Male High
Imperforate Anus (2013) Multicenter retrospective
comparative study of laparoscopically assisted and
conventional anorectoplasty for male infants with rec-
toprostatic urethral fistula. J Pediatr Surg 48(12):2383–
2388. doi:10.1016/j.jpedsurg.2013.08.010
51. Bischoff A, Levitt MA, Peña A (2011) Laparoscopy and its
use in the repair of anorectal malformations. J Pediatr Surg
46(8):1609–1617. doi:10.1016/j.jpedsurg.2011.03.068
52. Shawyer AC, Livingston MH, Cook DJ, Braga LH
(2015) Laparoscopic versus open repair of recto-
bladderneck and recto-prostatic anorectal malforma-
tions: a systematic review and meta-analysis. Pediatr
Surg Int 31(1):17–30
References
53. Peña A, Hong AR, Midulla P, Levitt M (2003)
Reoperative surgery for anorectal anomalies. Semin
Pediatr Surg 12(2):118–123
54. Alam S, Lawal TA, Peña A, Sheldon C, Levitt MA
(2011) Acquired posterior urethral diverticulum
following surgery for anorectal malformations.
J Pediatr Surg 46(6):1231–1235. doi:10.1016/j.
jpedsurg.2011.03.061
199
55. Peña A, Amroch D, Baeza C, Csury L, Rodriguez G
(1993) The effects of the posterior sagittal approach
on rectal function (experimental study). J Pediatr Surg
28(6):773–778
56. Peña A, Filmer B, Bonilla E, Mendez M, Stolar C
(1992) Transanorectal approach for the treatment of
urogenital sinus: preliminary report. J Pediatr Surg
27(6):681–685
 Rectal Atresia
14

Rectal atresia is a very unique malformation that
deserves a special description. It happens in our
experience, in about 1 % of all cases of anorec-
tal malformations. In this defect, the anus seems
to be completely normal, including the quality of
the sphincter and the location of the anal orifice.
However, deep inside the anus, just at the junction of
the anal canal with the rectum, there is an atresia or
narrowing (stenosis) (Fig. 14.1). Occasionally, we
see atresias or stenosis located at a different level.
The space that separates the dilated blind rectum,
from the anal canal, is represented by a septum that
sometimes is extremely thin and can be perforated,
and other times it is very thick. In some unusual
cases, there is a significant separation between the
blind upper rectum and the lower anal canal.
Interestingly, the sphincter mechanism is excel-
lent in most cases. There is one particular malfor-
mation similar to this one that is represented by a
stricture or by atresia of the rectum, associated to a
presacral mass and a sacral defect (see Chap. 8,
Sect. 8.2), which is a completely different type of
defect. The only thing they have in common is the
fact that the rectum is narrow or atretic.
We believe that rectal atresia with normal
sacrum and no presacral mass is unique, because
the sphincter mechanism is normal and also
because these patients do not have the typical
association with all the defects that we see in
other anorectal malformations. As a conse-
quence, the prognosis for these patients is excel-
lent, in terms of bowel control. They have a

a b

Fig. 14.1 Rectal Atresia. (a) Diagram. (b) External appearance

A. Peña, A. Bischoff, Surgical Treatment of Colorectal Problems in Children, 201

DOI 10.1007/978-3-319-14989-9_14, © Springer International Publishing Switzerland 2015
202
significant tendency to suffer from severe consti-
pation because they are born with a blind, very
dilated rectum. These malformations have been
previously described in the literature [1–5].
Rectal atresia has been traditionally described
in the old textbooks. The baby is born with a
normal-looking anus, and the nurse or the pedia-
trician tries to pass a thermometer through the
anus and finds an obstruction. In fact, part of a
routine examination of every “normal” newborn
is to check the patency of the anus, unless the
baby is already passing meconium.
14.1 Treatment
If one could think in an ideal indication for a pos-
terior sagittal approach, this would be the malfor-
mation which seems to be more indicated. The
defect is easily repaired through a posterior sagit-
tal incision. In our initial cases, we simply remove
the septum that separates the upper rectum from
the anal canal and created an end-to-end anasto-
mosis (Figs. 14.2 and 14.3). Subsequently, we
found some cases in which the size discrepancy
between the upper blind rectum and the small
anal canal was very severe, and in order to expand
the size of the anal canal, we introduced a techni-
Fig. 14.2 Repair of a Rectal Atresia. (a) Incision, exposed defect, open upper rectum, and anal canal. (b) Anastomosis
of the upper rectum to anal canal
14 Rectal Atresia
cal modification maneuver [5] (Fig. 14.4). Most
of the patients that we operated on came to us
already with a colostomy in place. Since the
patient has a colostomy, one can perform a distal
colostogram and simultaneously introduce a
metallic dilator in the anal canal to have a lateral
image of the atresia and estimate the distance
between the upper pouch and the anal canal. If
we could make the diagnosis early in an other-
wise healthy newborn baby, we would recom-
mend to do the operation without a colostomy.
14.2 Surgical Repair
The patient is placed in the prone position and we
approach the malformation posterior sagittally.
We go through the skin, subcutaneous tissue,
parasagittal fibers, ischiorectal fossa, and the
entire sphincter mechanism to expose and open
completely the anal canal and the upper blind rec-
tum. One can see in most cases the pectinate line,
at the same location as the atresia (Fig. 14.2a).
Unfortunately, we still see some of these
patients, previously operated in whom the sur-
geon considered that the little anal canal was use-
less and therefore decided to resect it and pulled
down the dilated piece of rectum. That is rather
a b
14.2 Surgical Repair
a b
Fig. 14.3 Diagram showing the repair of rectal atresia. (a) Rectum repaired, (b) Sagittal view of the finished
operation
regrettable, because the anal canal, as we know,
represents the area of sensation that will provide
bowel control to these patients. It is, therefore,
very important to preserve that little anal canal.
Sometimes the size of the anal canal is too small.
For that, we introduced a technical modification
[5], consisting in mobilizing the posterior rectal
wall, down to the skin of the anus (Fig. 14.4),
enlarging the circumference of the anus. We real-
ize that by doing that, the posterior aspect of the
anus will no longer be a real anal canal, but rather
a rectal wall. However we manage to preserve
most of the circumference of the original anal
canal, which will provide enough sensation to
have bowel control. We must keep in mind that
after we finish this procedure, the anastomosis
that we created between the upper dilated rectum
203
and the anal canal is going to be permanently col-
lapsed by the effect of the sphincter mechanism
that keeps the anal canal closed all the time,
except during defecation; therefore, these babies
must be subjected to the same protocol of anal
dilatations that we already described.
Some surgeons [4] went as far as to perform a
“laparoscopic transanal approach” to repair this
malformation. To demonstrate that something
can be done does not mean that it must be done.
We cannot justify to change a limited, painless,
bloodless, quick, minimally invasive, non-
laparoscopic procedure for a laparoscopic inva-
sive operation that includes an unnecessary total
rectal dissection.
Our experience includes 11 cases and has
been previously published [5].
204 14 Rectal Atresia

a b c

d e f

Fig. 14.4 Technical variant to expand the size of a very small anal canal. (a) Incision. (b) Open rectum. Arrows show
the portion of the rectum to be mobilized. (c) Sutures on one side of anal canal and rectum. (d) Sutures tied down.
(e) Same maneuver, opposite side. (f) Finished operation

Child Health 41(12):691–693. doi:10.1111/j.1440-

References
1. Dias RG, Santiago Ade P, Ferreira MC (1982) Rectal
atresia: treatment through a single sacral approach.
J Pediatr Surg 17(4):424–425
2. Upadhyaya P (1990) Rectal atresia: transanal, end-to-end,
rectorectal anastomosis: a simplified, rational approach to
management. J Pediatr Surg 25(5):535–537
3. Kisra M, Alkadi H, Zerhoni H, Ettayebi F,
Benhammou M (2005) Rectal atresia. J Paediatr
1754.2005.00763.x
4. Nguyen TL, Pham DH (2007) Laparoscopic and
transanal approach for rectal atresia: a novel alterna-
tive. J Pediatr Surg 42(11):E25–E27. doi:10.1016/j.
jpedsurg.2007.08.049
5. Hamrick M, Eradi B, Bischoff A, Louden E, Pena A,
Levitt MA (2012) Rectal atresia and stenosis: unique
anorectal malformations. J Pediatr Surg 47(6):1280–
1284. doi:10.1016/j.jpedsurg.2012.03.036
 Rectovestibular Fistula
15.1 Definition/Frequency
Rectovestibular fistula is the most important
anorectal malformation in females. This is due
to the fact that, by far, it is the most common
defect seen in females. Two hundred and ninety
of our 1,123 female patients were born with this
malformation. Two hundred and seventeen were
operated primarily by us, and 73 were reopera-
tions due to a previous failed attempted repair.
Interestingly, our series include 531 patients
with a cloaca. However, we are convinced that
this high number of cloacas in our series is
because ours is a referral center. We believe that
vestibular fistula is much more common in the
general population. Most of the vestibular fis-
tula cases are operated at the place where the
babies are born, whereas many cloacas are
referred to us due to the complexity of the repair.
When this malformation is repaired with a
meticulous surgical technique, the recovery of
the patients is excellent, and the functional
results are also very good. Unfortunately,
another characteristic of this malformation is
the fact that it is frequently mismanaged. We
compared the results obtained in a group of
patients that were repaired primarily by us with
those of the group that had a secondary proce-
dure due to the fact that the patient underwent a
Electronic supplementary material Supplementary
material is available in the online version of this chapter at
10.1007/978-3-319-14989-9_15.
A. Peña, A. Bischoff, Surgical Treatment of Colorectal Problems in Children,
DOI 10.1007/978-3-319-14989-9_15, © Springer International Publishing Switzerland 2015
15
previous failed attempted repair. The difference
in terms of bowel control is significantly differ-
ent. Therefore, we can repeat what other pediat-
ric surgeons through history have said, and that
is that “these patients have a single opportunity
to have a good repair.”
In the old literature [1] one can find that this
malformation received different names, includ-
ing “anovestibular fistula.” The authors believed
that this was a more benign variant of defect and
that these patients had a very short fistula and a
very low-lying rectum. They also believed that
malformation owed to be distinguished from a
“rectovestibular fistula,” which has a long, nar-
row fistula and a rectum located higher in the pel-
vis, and, therefore, they believed that the
prognosis was not as good as the one observed in
cases of “anovestibular fistula” [1, 2]. We also
found the term “vestibular anus”; the authors
believed that some patients were born with an
otherwise normal anus located in the vestibule of
the female genitalia [2]. We have never seen this
type of defect. We do not use those three terms
mentioned here, because we found that all our
patients with an anal opening located in the vesti-
bule can be repaired with the same surgical pro-
cedure and have the same functional prognosis;
therefore, we consider the old terminology
impractical and misleading. It is true that some
patients have a longer fistula than others; those
cases may require more dissection to bring the
rectum down. However, our results are uniformly
good, regardless the type of fistula.
205
206 15 Rectovestibular Fistula

a b

Fig. 15.1 Diagram of vestibular fistula. (a) Sagittal view. (b) Perineum

Rectovestibular fistula is a defect in which

the rectum opens in the vestibule of the female
genitalia. This should not be confused with a
rectovaginal fistula. In order for us to call a mal-
formation “rectovaginal fistula,” one must see the
anal opening located inside the vagina, deeper
to the hymen. Vestibular fistula patients have a
normal hymen, and the anal orifice is located
posterior to the hymen (Figs. 15.1 and 15.2). The
anal opening is visible most of the time, provided
the clinician separates the labia of the baby’s
genitalia. The newborn female frequently has a
significant degree of edema and swelling of that
area, considered to be a consequence of the effect
of maternal hormones. Therefore, in the new-
born baby, it may be a little bit more difficult to
see the precise location of the vestibular fistula
(Fig. 15.3).
Some cases of vestibular fistula have the anal
opening located rather deep and are almost
impossible to see it without general anesthesia. In
fact, some patients have a rather small-looking
genitalia (vulva) similar to what we see in cases
of cloaca. The anal orifice is located very deep in
the vestibule, and the urethra is also located
deeper than normal, which is what urologists call
“female hypospadias.” This particular variant, we
call “cloaca type I,” one could also use the term
“deep vestibular fistula with a female hypospa- Fig. 15.2 Picture of a vestibular fistula
15.2 Associated Defects 207

Fig. 15.4 Deep rectovestibular fistula with female hypo-

spadias – observe small vulva

Fig. 15.3 Vestibular fistula in a newborn baby. Arrow

shows the fistula site

dias” (Fig. 15.4). We consider this particular type

of malformation a transition in between a cloaca
and a vestibular fistula.
Some patients are born with the anal orifice
located just in between the perineal body (skin
lined) and the vestibule, wet tissue (Fig. 15.5).
This type of defect is considered intermediate
between the vestibular fistula and perineal fistula.
The management of these patients is not different
from any other type of vestibular fistula. This
defect is also known as “fourchette fistula.”

15.2 Associated Defects

A retrospective review of 290 patients with ves-

tibular fistulas operated by us (217 primary and
73 secondary) showed a significant number of
associated defects. Since vestibular fistula is con-
sidered a malformation representative of the
“good side” of the spectrum of anorectal defects Fig. 15.5 Fourchette fistula
208
in general, the frequency of association of all the
defects is rather low. Yet, it is significant enough
to be searched for.
15.2.1 Sacral
We were able to measure the sacral ratio in 113
of our cases and found that the average AP ratio
was 0.57 and lateral was 0.7. Six percent of these
cases had a ratio lower than 0.4. This is consis-
tent with the fact that we consider this malforma-
tion a “benign” one, with good functional
prognosis. Fourteen cases had a hemisacrum and
a presacral mass, and as previously mentioned,
presacral masses occur more frequently in lower
defects.
15.2.2 Spinal
Approximately, 9 % of our patients had some
form of spinal defect, mainly hemivertebra.
15.2.3 Urologic
Ten percent of vestibular fistula cases had a sin-
gle kidney, which as we know is the most com-
mon anatomic abnormality associated to all
anorectal malformations, and 13 % of patients
had vesicoureteral reflux, which is consistent
with the fact that this disorder is the most com-
mon functional urologic abnormality seen in ano-
rectal malformation cases. Hydronephrosis was
present in 6 % of the cases.
15.2.4 Gynecologic
There are not many reports in the literature,
related to this very important assoc [3, 4]. A ret-
rospective review of our patients with vestibular
fistula showed that 17 % of them had associated
genital anomalies [5]. Eight percent had absent
vaginas or vaginal atresia. Figure 15.6 shows the
different types of absent vaginas or vaginal atre-
sias encountered.
15 Rectovestibular Fistula
Figure 15.6a shows the perineum of one of
these patients, and there is no vaginal opening.
Figure 15.6b shows a diagram of a sagittal view
and the type of repair that we used, consisting in
leaving the rectum attached to the urethra, to
function as a neovagina and pulling the upper
rectum down to the perineum.
Eighty percent of the patients with vestibular fis-
tula and absent vagina are born with agenesis of the
internal genitalia (uterus and fallopian tubes). In
such cases the vagina is replaced with a piece of
colon; this is done only for the patient to have sexual
function. Twenty percent of the patients have a
uterus and a blind ending of vagina, usually located
very high in the pelvis (Fig. 15.6c). In that type of
case, the lower vagina is replaced with a piece of
colon with dual purpose (sexual and reproductive).
Some cases of vestibular fistula with absent
vagina can be repaired without vaginal replacement,
but rather pulling down their native vagina. That can
only be done in cases with a large blind vagina.
Five percent suffered from some sort of septa-
tion disorder of the Müllerian structures. These
included a vaginal septum, always associated
with the presence of two hemicervices and two
hemiuteri (Fig. 15.7). Three patients had a unilat-
eral streak ovary; the rest had two normal ovaries.
Two patients had a perineal lipoma, and one
patient had a labial hemangioma.
We were able to see patients born with a ves-
tibular fistula that came to us as adolescents; they
had a repair in the past, but the surgeons missed
the diagnosis of a vaginal septum. These vaginal
septa can only be detected when the surgeon sus-
pects their existence. Based on these findings, it
is our routine and our recommendation to perform
a vaginoscopy with a pediatric cystoscope in all
patients with vestibular fistula. The presence of a
vaginal septum may, in some cases, interfere with
tampon placement and sexual intercourse when
the patient grows up. But more important than
that is the fact that the presence of a vaginal sep-
tum means, by definition, that the patient has two
hemiuteri, representing a partial or total septation
disorder. Hemiuteri have important gynecologic
and obstetric implications. We know that patients
with hemiuteri may have a higher degree of infer-
tility, and those patients who become pregnant
15.2 Associated Defects 209

have a higher incidence of miscarriages and pre- 15.2.6 Tethered Cord

mature labor. Therefore, it is extremely important
to make the diagnosis as early as possible in order
to provide these patients with special gyneco-
logic and obstetric care later in life.
It is our routine to do a vaginoscopy in every
case of vestibular fistula. We perform that study
with a baby cystoscope, during the same anesthe-
sia given for the repair. Figure 15.8 shows the
aspect of a normal infant cervix.
Fifty-seven patients were evaluated with an
ultrasound (first 3 months of life) or with an
MRI, looking for spinal cord anomalies; twenty
of them had tethered cord (35 %). These figures
are higher than the average of all anorectal mal-
formations, and we believe that this is explained
by the fact that the incidence of presacral masses
is also high in this malformation. Tethered cord
is very common in cases with presacral mass.

15.2.5 Gastrointestinal
Six percent of our patients with vestibular fistula
had an associated esophageal atresia, one patient
without a fistula, and all the others with a tra-
cheoesophageal fistula; 1 % had a form of duode-
nal obstruction (atresia or stenosis).
15.2.7 Cardiovascular
Twenty-seven patients (9 %) had an atrial septal
defect. Twenty-two (8 %) had a ventricular sep-
tum defect. Fourteen (5 %) had a patent ductus
arteriosus, and four (1 %) suffered from tetralogy

a b

Fig. 15.6 Vestibular

fistula with absent or
partially absent vagina.
(a) Photograph.
(b) Diagram of a sagittal
view and one type of
repair, using the rectum to
replace the vagina.
(c) Diagram showing the
internal genitalia of a
patient with a high vaginal
atresia, with a piece of
colon. (d) Diagram
showing an absent vagina
as well as the uterus, the
vagina totally replaced
with colon. (e) Two types
of vaginal atresia.
Prepuberty and
postpuberty
210 15 Rectovestibular Fistula

Fig. 15.6 (continued)

15.3 Diagnosis
Fig. 15.7 Pocket of the original vestibular fistula in a
patient previously operated with the erroneous diagnosis
of a “rectovaginal fistula.” (R) rectum, V original fistula
Fig. 15.8 Vestibular fistula with a vaginal septum. Arrow
shows the fistula site
of Fallot. Most of these defects (approx. 80 %)
did not require treatment, since the patients were
hemodynamically stable.
211
Fig. 15.9 Aspect of a normal cervix in a baby with a ves-
tibular fistula
15.3 Diagnosis
The diagnosis of vestibular fistula is a simple
one. It only requires a meticulous inspection of
the genitalia of the baby. Yet, amazingly, many
patients are not diagnosed or are misdiagnosed as
having “rectal vaginal fistula.” From our series of
1,123 female patients, we have only seen seven
cases of documented real rectovaginal fistula.
During the same period of time (over 30 years),
we have operated on 290 cases of vestibular fis-
tula. Fifteen of them come to our center with a
previous diagnosis of “rectovaginal fistula.”
Actually, they were born with a vestibular fistula
as evidenced by the presence of a little pocket
where the vestibular fistula used to be located
(Fig. 15.9). Forty-five female patients also came
to us after a failed attempted repair of a
malformation diagnosed as “rectovaginal fistula.”
A careful examination revealed that those patients
actually had a persistent urogenital sinus, which
means that they were actually born with a cloaca
and the surgeons only repaired the rectal compo-
nent of the malformation, because they were
thinking that the patient only had a rectovaginal
fistula (see Chap. 16, Sect. 16.1.4).
Prior to 1980, the literature [2, 6–13] reported
an elevated number of cases of “rectovaginal fis-
212
tula” in female patients. In contrast, those authors
reported very few vestibular fistula cases and
very rare cloaca cases. A few publications after
1980 persist reporting “rectovaginal fistula
cases.” Interestingly, looking at the diagrams of
most of those publications, they actually show
vestibular fistulas, although they call them “vagi-
nal fistula.” The term “vestibular fistula” has been
used correctly by some authors with large experi-
ence in the management of these defects
[14–21].
We believe that this is not a simple semantic
problem, but rather has important clinical
implications [22]. We have seen patients born
with vestibular fistula that were previously
misdiagnosed as “vaginal fistula” and under-
went a type of repair designed to repair “high”
malformations, namely, a contraindicated
abdominoperineal (open or laparoscopic) pro-
cedure that resulted in fecal incontinence. We
also have seen that at least 30 patients born
with a cloaca received the wrong diagnosis of
“rectovaginal fistula” and underwent a repair
only of the rectal component of the
malformation, leaving the patient with a uro-
genital sinus [22].
15.4 Treatment
15.4.1 Colostomy or No Colostomy
This is a frequently debated subject. Many sur-
geons claim that they routinely repair vestibular
fistulas without a colostomy and they have “good
results” [12, 23–27]. Many others prefer to open
a colostomy in all cases with a vestibular fistula.
In the meantime, we see many patients that
underwent a repair of a vestibular fistula without
a colostomy and suffered from serious complica-
tions, including dehiscence and retraction of the
rectum as well as reopening of the fistula.
However, this recurrent fistula is frequently an
acquired rectovaginal fistula, due to the fact that
during the attempted repair, the posterior wall of
the vagina was damaged.
As discussed in the Chap. 5, we believe that
the answer for this question of colostomy or no
15 Rectovestibular Fistula
colostomy is different for every surgeon and his/
her different surrounding circumstances. It very
much depends on the experience of the surgeon,
the clinical condition of the patient, and the infra-
structure of the hospital where the patient is
treated.
In general, at our institution, if a baby is born
with a vestibular fistula, we operate on her within
the first 5 days of life without a colostomy, pro-
vided the baby is in good clinical condition, is
full term, and does not have severe associated
defects.
Consider the case of a premature baby with a
cardiac condition and vestibular fistula. Under
those circumstances, dilatations of the fistula
may prove to be useful for the patient to be able
to pass stool, eat, and grow. That would allow the
surgeon to postpone the decision of colostomy or
primary repair. On the other hand, a full-term
baby in good clinical condition without associ-
ated defects in an institution with a good infra-
structure and a pediatric surgeon with experience
in the management of this defect, the patient can
be operated before starting her feedings, at a time
when the patient is still passing meconium,
because when it is done in that way, the patient
actually does not need any kind of bowel
preparation.
Most of our patients come to us after the new-
born period and with a colostomy already opened
at another institution, sometimes in another coun-
try. Many other patients come to us after several
months of passing stool with difficulty through
the non-operated vestibule, with severe constipa-
tion and megacolon. Those patients are also
treated without a colostomy at our institution, but
our routine includes the admission of the patient
1 or 2 days prior to the main operation, insertion
of a nasogastric feeding tube, and administration
of GoLYTELY1 at a rate of 25 mL/kg/h until the
colon is completely clean. The patient receives a
PICC line and parenteral nutrition for a period of
7–10 days postoperatively.
When the patient has a colostomy, the opera-
tion can be done without following this routine,
1
GoLYTELY… (Polyethylene glycol/electrolytes.) Braintree
Laboratories, Braintree, MA, USA
15.5 Main Repair
but rather irrigating only the distal stoma of the
colostomy the day before surgery. In that case,
the baby can eat the same day of the operation;
she will stay for 48 h in the hospital receiving
intravenous antibiotics. Our experience is that the
pain that these patients experience postopera-
tively is rather minimal. We have operated on pri-
marily without a colostomy in approximately
50 % of our cases.
We use a posterior sagittal approach to repair
these malformations. Other approaches do exist,
and the most traditional and popular was
described by Dr. Potts and is called a fistula trans-
plant [28–32]. Some surgeons describe an opera-
tion called “anterior sagittal approach” [33–37].
We found that the word “anterior” in those publi-
cations was actually not referring to the incision,
but rather to the position of the patient; in other
words, the patient is positioned in lithotomy posi-
tion, rather than prone, but the incision is always
posterior to the fistula, because there is no way to
make an incision anterior to the fistula site. In
other words, the so-called “anterior sagittal
approach” is actually a posterior sagittal approach
performed in lithotomy position.
Interestingly, Professor Francesco Rizzoli
from Bologna, Italy, published in 1869 [38] the
technique now referred as “anterior sagittal
approach”; his publication includes magnificent
illustrations.
The essential components of the posterior sag-
ittal approach described below avoid the flaws
observed in those other techniques, and the most
common problems seen in our reoperations were
retraction of the anoplasty and an inadequate
perineal body (anteriorly located anal orifice).
15.5 Main Repair (Animation 15.1)
The patient is brought to the operating room, and
we start the procedure with the patient in the
lithotomy position in order to perform a
vaginoscopy using a baby cystoscope. We do this
with the specific purpose to rule out vaginal
malformations. Although a vaginal septum can
be simply seen by separating the labia without
the use of a cystoscope, we prefer to use a cysto-
213
scope, because sometimes one can see a vaginal
septum that is only present in the lower part of
the vagina, and the upper part has a single cervix
(Fig. 15.8). Most of the times, however, the sep-
tum is complete, and one can see two cervices at
the end of the vagina.
The patient is then turned into the prone posi-
tion with the pelvis elevated, and the perineum,
as well as the genitalia and perianal area, is
washed, prepped, and draped in the usual man-
ner. Most of the times, one can see the anal ori-
fice in the vestibule, and in such case multiple
5-0 silk stitches are placed at the mucocutane-
ous junction of the anal opening (Fig. 15.10).
These stitches serve the purpose of applying
uniform traction to facilitate the separation of
the rectum from the vagina. Occasionally, the
fistula is located so deep that it is impossible to
do this; in such case, we first make the incision
and go deep enough to be able to see the edges
of the fistula and apply multiple 5-0 silk stitches
(Fig. 15.11). We use the electrical stimulation to
determine the limits of the sphincter and to
guide ourselves to try to stay as much as possi-
ble exactly in the midline, dividing the entire
sphincter mechanism leaving equal portions of
the sphincter in both sides of the midline. For
this, we use a needle-tip cautery, changing from
cutting to coagulation. The size of the incision
usually is shorter than the regular posterior sag-
ittal anorectoplasty. The incision usually runs
from the lowest part of the sacrum and coccyx
down to the fistula orifice, passing through the
sphincter mechanism. We divide the entire
sphincter, including the parasagittal fibers, the
muscle complex, and the levator mechanism.
Deeper to the levator mechanism, one can iden-
tify the characteristic white fascia that covers
the posterior wall of the rectum (Fig. 15.12).
Figure 15.13 shows the aspect of the rectum
with traction sutures. Traction creates the plane.
The white fascia is removed from the posterior
rectal wall, including the extrinsic blood supply of
the rectum. We do this to identify the real rectal
wall completely clean. The dissection is then
extended to the lateral walls of the rectum
(Fig. 15.13). The next step consists of extending
the dissection of the lateral walls of the rectum all
214 15 Rectovestibular Fistula

a b

Fig. 15.10 Multiple stitches placed at the anal orifice located in the vestibule. (a) Diagram. (b) Photograph

a b

Fig. 15.11 (a) Incision – when the fistula is located too deep in the vestibule, we must open first and place the sutures later.
(b) Multiple stitches in a case of a deep fistula

the way down to the skin. It is important to mately 1 cm proximal in the rectum, one can
remember that at the level of the skin, there is no clearly identify the plane that separates the rectum
real plane of dissection between the rectal wall from the surrounding tissues, and therefore the
and the surrounding tissues. Whereas approxi- recommendation is to follow the steps mentioned
15.5 Main Repair
Fig. 15.12 “White fascia” after dividing the entire
sphincter mechanism, the rectum is identified covered by
the white fascia
in this description, meaning to identify the poste-
rior rectal wall; continue the dissection to the lat-
eral walls of the rectum and then from there,
applying uniform traction on the multiple 5-0 silk
sutures; and continue the dissection from the lat-
eral walls of the rectum down to the skin. One
must expect to find important vessels that provide
the blood supply of the lower rectum while
dissecting the lateral walls of the rectum.
A Weitlaner retractor is used to achieve adequate,
optimal exposure. At this point, we are ready to
initiate the most important part of the operation,
which is the separation of the rectum from the
vagina.
One must keep in mind that the rectum and
vagina share a common wall with a variable
215
Fig. 15.13 Diagram showing the dissection of rectum
length from 1 to 3 cm and that there is no real
plane of dissection between both structures. In
other words, one must make two walls out of one,
and very often, this common wall is extremely
thin. This happens to be the most important ana-
tomic feature of this malformation. Surgeons
must keep in mind that the main challenge in the
repair of these defects is the separation of the rec-
tum from the vagina and should take it as a per-
sonal challenge. The separation of the rectum
from the vagina requires a very meticulous, deli-
cate surgical technique. It cannot be done by
blunt dissection. We like to perform this separa-
tion using the needle-tip cautery while applying
traction on the rectal wall and checking with a
lacrimal probe the thickness of the anterior rectal
wall and the posterior vaginal wall very fre-
quently, to be sure that we are not getting too
close to one or the other (Fig. 15.14). As we
progress in this meticulous dissection, the wall of
the rectum, as well as the wall of the vagina,
216
Fig. 15.14 Different stages of the separation of the rectum from the vagina. Posterior vaginal wall and anterior rectal
wall intact
starts getting thicker, which indicates that we are
getting close to the point where both are expected
to be completely separated and have a full thick-
ness. At this time, the surgeon should not be
overconfident, because in that point he could
injure either the rectum or the vagina (Fig. 15.14).
The dissection must continue until the rectum has
been completely separated from the vagina
(Fig. 15.14).
It is extremely common for surgeons to ask
what happens and what to do in the event of acci-
dentally opening either the vagina or the rectum.
Our routine answer is as follows: if it happens that
we opened the vaginal wall, but maintained intact
the rectal wall, one can actually leave the vaginal
orifice of the injury open, provided the rectum is
intact, and the anoplasty is not under tension, and
the patient is going to do alright. Something simi-
lar can be said when the orifice is created in the
rectum, but the vaginal wall is intact. What is con-
sidered nonacceptable is to have an injury of the
rectal wall in front of an injury to the vaginal wall,
leaving sutures in front of sutures, since that is
considered an obvious predisposing factor for the
formation of a rectovaginal fistula. Under such
circumstances (vaginal injury and a rectal injury),
one must continue the dissection of the rectum
15 Rectovestibular Fistula
until we can leave a normal rectal wall in front of
the vaginal orifice or suture.
We must always remember that in dealing with
anorectal malformations, the real challenge in the
surgical repair is represented by the separation of
the structures, namely, the rectum from vagina,
the rectum from urethra, and the vagina from ure-
thra, because all those structures share a common
wall without a plane of dissection. Most of the
complications that we have seen in patients who
underwent failed attempted repairs of anorectal
malformations occur during the separation of
these structures.
Sometimes when the rectum has been fully
separated from the vagina, we find that we have
enough rectal length to do an anoplasty without
tension and with good blood supply. However,
many other times, the rectum needs further mobi-
lization. To do this, one must continue applying
uniform traction on the multiple silk stitches. By
doing this, it becomes evident that there are some
bands and vessels holding the rectum up in the
pelvis. These must be separated from the rectum,
independently burned and divided in a circumfer-
ential manner, continuing until we have enough
rectal length to create an anastomosis without
tension.
15.5 Main Repair
a b
Fig. 15.15 Perineal body reconstructed. (a) Diagram. (b) Intraoperative diagram
The incision required to repair rectovestibu-
lar fistulas includes the opening of the muscle
complex and part of the levator mechanism.
Sometimes, it is not necessary to open com-
pletely the levator mechanism, and therefore we
call this a limited posterior sagittal anorecto-
plasty. However, we are convinced that the size
of incision does not affect, in any way, the future
functional prognosis, provided all of the other
important surgical steps are done correctly.
Once the rectum has been separated from the
vagina and mobilized, in preparation for the
reconstruction, the limits of the sphincter are elec-
trically determined and marked with temporary
silk stitches. The goal at this stage is to bring
together the anterior limits of the sphincter and by
doing that to reconstruct the perineal body of the
patient (Fig. 15.15). This is the space that sepa-
rates the vagina from the rectum. It is extremely
important to use strong sutures (5-0 or 4-0 long-
term absorbable sutures depending on the patient’s
age) to approximate both sides of the perineal
body. There, we usually find a fibrous tissue that
surrounded the original vestibular fistula. We use
this tissue to anchor our stitches. These deep
217
stitches must relieve most of the tension of the
perineal body to be sure that the skin edges in the
perineal body come together with no tension. We
close the skin of the perineal body with 6-0 Vicryl
sutures, only to be sure that the edges of the skin
have come together, but those sutures hold no ten-
sion. Figure 15.15 shows the repaired perineal
body. The rectum then is located within the limits
of the sphincter immediately behind the perineal
body. The posterior edges of the muscle complex
and levator are sutured together in the midline
using 5-0 long-term absorbable sutures, including
a bite to the posterior rectal wall to anchor it in
normal location (Fig. 15.16). These stitches are
aimed to avoid retraction and prolapse. The
ischiorectal fossa, as well as the subcutaneous tis-
sue, is obliterated using 5-0 long-term absorbable
sutures, and the skin is closed either with subcu-
ticular 5-0 monofilament, absorbable, or inter-
rupted 6-0 long-term absorbable sutures.
The anoplasty is done as previously described,
using two layers of interrupted 5-0 or 6-0 long-
term absorbable sutures (Fig. 15.17). We try to
trim off as little as possible rectal tissue, but we
do not hesitate to remove all of the tissue that is
218
Fig. 15.16 Diagram showing sutures taking the posterior
edges of the muscle complex, including a bite to the pos-
terior rectal wall to anchor it
a b
Fig. 15.17 Anoplasty and wound closed. (a) Diagram. (b) Intraoperative picture
15 Rectovestibular Fistula
considered damaged, to be sure that we have
healthy rectal tissue with good blood supply to
create a healthy anoplasty (Fig. 15.18).
Patients operated without a colostomy are
kept on parenteral nutrition with nothing by
mouth for a period not shorter than 7 days.
After 7 days, we look into the external aspect of
the perineum, and if it looks that it has healed
nicely, we allow the patient to eat and discharge
her. Anal dilatations start 2 weeks after surgery
following our protocol (see Chap. 18).
Occasionally, when one examines the patient’s
perineum 1 week after the operation, one may
find that there is an area of partial dehiscence of
the perineal body or the posterior sagittal inci-
sion, and this is a good opportunity to take the
patient to the operating room and resuture that,
taking advantage of the fact that the patient has
been with nothing by mouth. Under those cir-
cumstances we keep the patient 2 or 3 more
days fasting, receiving parenteral nutrition.
At the time of colostomy closure (in those
patients with a colostomy) 2 or 3 months after the
operation, the external aspect of the anus and the
vagina, as well as the perineum in these patients,
is remarkably normal looking (Fig. 15.19).
15.7 Functional Results 219

15.6 Complications

Five of our patients suffered from a dehiscence

requiring a reoperation.
We have seen patients born with vestibular fis-
tula that underwent a “laparoscopic repair.” It
must be very difficult for the surgeon to work in
this common wall through the abdomen with a
laparoscope. What they rather have done is to
amputate the rectum at a “convenient” location,
leaving the distal piece of the rectum attached to
the vagina. We consider this an inappropriate
way of management.

15.7 Functional Results

Ninety percent of our patients have voluntary

Fig. 15.18 Photograph of a finished operation
bowel movements by the age of three. This is,
provided they have a normal sacrum, not tethered
cord, and they had received a good operation.
Over 50 % of the patients suffer from significant
constipation that deserves special attention. Not
taking good care of the constipation will
provoke chronic fecal impaction and overflow

Fig. 15.19 External

appearance 2–3 months
postoperatively
220
pseudoincontinence. We usually have a long con-
versation with the parents and explain in detail the
importance of taking care of the constipation. We
emphasize the fact that the constipation that these
patients suffer from is much more severe than the
common idiopathic constipation of the general
pediatric population. The amount of laxatives that
these patients need sometimes is two, three, four,
or five times higher than in other types of patients.
We try to make the parents paranoid against the
problem of constipation. We also emphasize the
fact that the amount of laxatives that these patients
require to empty the colon every day cannot be
predicted. We determine the amount of laxatives
by trial and error over a period of several days,
taking abdominal x-ray films to be sure that the
patient empties the colon. If the patients are
receiving breast feedings at the time of our opera-
tion, most likely they will not need laxatives until
they start decreasing the amount of breast milk
and receiving another type of formula.
Sexual life in these patients is normal, and as
we have seen, many of our patients are becoming
adults and are getting married. They also can
deliver babies vaginally, since we did not actually
injure the vagina which preserves a normal elas-
ticity in most of its circumference, since we only
dissected the posterior vaginal wall.
In the past, some surgeons [2] claimed that
these patients could have a normal life without an
operation or simply doing a “cutback” type of
procedure to enlarge the anal opening. We have
seen that this is not true. First of all, the bowel
control under those circumstances is rather poor.
In addition, when these patients grow up, they
feel very unhappy about the fact that they have
the anus located immediately behind the vagina
with no perineal body. This gives them insecurity
and psychological problems, and in addition, a
vaginal delivery is contraindicated, because it
will produce severe rectal damage.
15.8 Reoperations in Patients
with Vestibular Fistula
From all anorectal defects treated by us, it is the
vestibular fistula type of case that most frequently
came to us after a failed attempted repair at
15 Rectovestibular Fistula
another institution. In fact, from our total series
of 290 patients with vestibular fistula, 73 of them
are reoperations. We believe that this is a reflec-
tion of the fact that surgeons in general probably
underestimate the complexity of the repair of this
defect. As previously mentioned, vestibular fis-
tula is by far the most common anorectal malfor-
mation seen in females. The functional prognosis
in girls when they are born with a good sacrum,
have no tethered cord, and receive a good opera-
tion is excellent. Unfortunately, patients who
underwent a failed attempted repair followed by
a reoperation do not have the same good func-
tional prognosis. Eighty percent of them have
voluntary bowel movements as compared to
90 % for those operated primarily.
Probably, the surgeons find it relatively easy to
imagine that the orifice of the rectum located in
the vestibule could easily be moved back to the
normal location of the anus. In reality, the repair
of this malformation is a delicate and technically
demanding procedure.
The most common scenario in dealing with
reoperations for vestibular fistulas is a patient
that was operated without a protective colostomy
and soon after suffered from dehiscence and
retraction of the rectum, followed by opening of
the rectum into the posterior vaginal wall. In
other words, the original malformation was a ves-
tibular fistula, but the patient comes with a real
acquired rectovaginal fistula secondary to a poor
initial operation. During the re-exploration, our
most common finding in this specific type of
problem has been an intact common wall between
the rectum and vagina. In other words, the sur-
geons try to repair the malformation but failed to
separate the rectum completely from the vaginal
wall. They still tried to pull the rectum down
which was left, we think, under tension, because
it was still attached to the vaginal wall. As a con-
sequence, the rectum retracted. We assume that
during the attempt to separate the rectum from
the vagina, the lower part of the vaginal wall was
injured, and therefore when the rectum retracted,
it reopened into the posterior vaginal wall creat-
ing an acquired vaginal fistula.
Another common scenario in reoperations for
vestibular fistula is a group of patients that
underwent a previous operation called cutback
15.8 Reoperations in Patients with Vestibular Fistula 221

Fig. 15.20 Pictures of

two patients born with a
vestibular fistula and
underwent a cutback
procedure prior to coming
to our center

Fig. 15.21 External

aspect of perinea of two
patients born with a
vestibular fistula and two
hemivaginas. They
underwent a poor
attempted repair and were
left with no perineal body
and two hemivaginas

procedure at another institution [39, 40]; these
consisted in making a posterior slit in the poste-
rior edge of the anal opening in the vestibule
and suturing it horizontally like a Heineke-
Mikulicz type of procedure. That procedure
only enlarges the anal opening and leaves the
rectum attached to the vaginal wall with no peri-
neal body (Fig. 15.20). We believe that, perhaps,
in cases of perineal fistula, the cutback proce-
dure could be considered an acceptable thera-
peutic alternative, but we strongly believe that
this type of operation is contraindicated in
patients with vestibular fistula. There was an old
belief that went from generation to generation
that by leaving the rectum attached to the vagina,
as time went by, the perineal body would grow,
which is definitely not true.
Another finding that is interesting to mention
is the fact that in some of these patients, we found
that they had two hemivaginas, and such malfor-
mation was never mentioned in the operative
reports of the previous surgeons (Fig. 15.21).
Again, we like to say that “our eyes see only what
our mind suspects.”
222
Fig. 15.22 External appearance of the perineum of different patients referred to us, after failed attempted repairs
15.9 Surgical Technique
Reoperations for recurrent or dehiscent,
retracted vestibular fistulas are currently done by
us without a protective colostomy. Figure 15.22
shows examples of cases that came to us after
a failed attempted repair of their malformation.
However, we follow the precautions already
mentioned in the chapter related to bowel prepa-
ration. We take the baby to the operating room
with the bowel completely clean. As part of
our routine, we perform vaginoscopy and cys-
toscopy to rule out the presence of associated
defects (mainly vaginal septum). We place the
patient in prone position with the pelvis elevated
and make a posterior sagittal incision following
the specifications already described. Multiple
5-0 silk stitches are placed at the mucocutane-
ous junction of the rectovaginal fistula or the
rectal opening in order to apply uniform trac-
tion. Through the posterior sagittal incision, all
structures are divided in the midline until the
posterior rectal wall is identified and then the
dissection of the rectum proceeds, first on the
lateral walls and eventually in the common wall
between the rectum and the vagina. As previ-
ously mentioned, we have been impressed by
15 Rectovestibular Fistula
the fact that most of these patients have an intact
common wall between the rectum and vagina,
which reflects the fact that the surgeons did an
incomplete mobilization of the rectum. We go
ahead and make two walls out of one. In other
words, we separate the rectum from the vagina
as previously described in the primary proce-
dure. We must suture the defect of the posterior
vaginal wall. Once the rectum has been com-
pletely separated, we then mobilize the rectum
enough to guarantee that an intact anterior rectal
wall is left in front of the vaginal sutures. We
are convinced that the vaginal defect can even
be left unsutured, and it will heal normally pro-
vided the rectal wall left behind is intact. We
dissect the rectum enough to guarantee that the
rectal wall in front of the vagina is completely
normal and also to be sure that the anastomo-
sis between the rectum and the skin of the anal
dimple is performed without tension. Before we
do the anoplasty, we repair the posterior vaginal
wall with long-term absorbable sutures, deter-
mine the limits of the sphincter, and continue
the operation as described for primary cases,
reconstructing the perineal body and doing the
anoplasty. The patients remain 10 days fasting
and receiving parenteral nutrition.
References
We were impressed by the fact that many
patients had a failed operation early in their life,
remained incontinent during childhood, and
searched for help only when they became teen-
agers and had decided to become sexually
active. We believe that they had become aware
of their defective anatomy and felt very upset
about the fact that their rectum and vagina were
located one next to the other, with no perineal
body. In other words, they felt embarrassed at
considering sexual life with that kind of
anatomy.
15.10 Rectovestibular Fistula
with Normal Anus
See Chap. 27, Sect. 27.2.
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15 Rectovestibular Fistula
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36. Wakhlu A, Kureel SN, Tandon RK, Wakhlu AK
(2009) Long-term results of anterior sagittal ano-
rectoplasty for the treatment of vestibular fistula.
J Pediatr Surg 44(10):1913–1919. doi:10.1016/j.
jpedsurg.2009.02.072
37. Shehata SM (2009) Prospective long-term functional
and cosmetic results of ASARP versus PASRP in
treatment of intermediate anorectal malformations in
girls. Pediatr Surg Int 25(10):863–868. doi:10.1007/
s00383-009-2434-7
38. Rizzoli F (1869) Atresia Congenita. Collezione delle
memorie. Chirurgiche ed Ostetriche 2:321–357
39. Mariño Espuelas JM, Martinez Utrilla MJ, Gonzalez
Utrilla Y (1988) Consideraciones A La Fistula
Vestibular. Cir Pediátr Hosp Infat 1(2):88–90
40. Matley PJ, Cywes S, Berg A, Ferreira M (1990) A 20-year
follow-up study of children born with vestibular anus.
Pediatr Surg Int 5(1):37–40. doi:10.1007/BF00179636
 Cloaca, Posterior Cloaca
and Absent Penis Spectrum
16.1 Cloaca
16.1.1 Definition and Management
A cloaca is a malformation that affects the rec-
tum and urogenital tract in females. These girls
are born with a single perineal orifice. The vagina,
urethra, and rectum are fused together inside the
pelvis, creating a single common channel that
opens into a single orifice in the location where
the urethra normally opens (Fig. 16.1). The
length of the common channel varies from case
to case, from 1 to about 10 cm with an average of
approximately 3 cm.
Thirty percent of these patients suffer in addi-
tion from a very dilated vagina full of fluid and/or
mucus, called hydrocolpos [1] (Fig. 16.2). The
reason why these very dilated vaginas retain fluid
remains a mystery, since they are never really
atretic. We speculate that there must be some sort
of valve mechanism that interferes with the emp-
tying of the fluid. Most of the patients with
hydrocolpos, in addition, have duplicate
Müllerian systems (Fig. 16.3).
The hydrocolpos may produce two important
complications:
Electronic supplementary material Supplementary
material is available in the online version of this chapter at
10.1007/978-3-319-14989-9_16.
A. Peña, A. Bischoff, Surgical Treatment of Colorectal Problems in Children,
DOI 10.1007/978-3-319-14989-9_16, © Springer International Publishing Switzerland 2015
16
(a) The first is the possibility of compressing the
trigone of the bladder, producing an extrinsic
ureterovesical obstruction, megaureter, and
hydronephrosis.
(b) The second possibility is that the hydrocol-
pos, left undrained, may become infected;
creating a pyocolpos that eventually may
perforate, which is a catastrophic event with
risk of death. In addition, the resulting
inflammation may scar the vagina and impact
the future reconstruction.
Approximately 60 % of the patients with clo-
acas also have a double Müllerian system con-
sisting of the presence of two hemiuteri and two
hemivaginas [1]. This septation disorder may be
partial or total. In addition, it can be symmetric or
asymmetric. In the asymmetric types, the double
Müllerian system phenomenon is frequently
associated with a unilateral atresia of the
Müllerian structure. When this goes unrecog-
nized, it may produce an accumulation of men-
strual blood at the age of puberty, as well as
retrograde menstruation into the peritoneal cavity
(Fig. 16.4) which produces rather dramatic signs
of an acute abdomen and requires an emergency
laparotomy. The presence of double Müllerian
systems also has important potential obstetric
implications that will be discussed later in this
chapter.
Cloacas represent a very wide spectrum of
defects, but the common denominator is the pres-
ence of a single perineal orifice. On the very bad
side of the spectrum, one may find patients with a
225
226 16 Cloaca, Posterior Cloaca and Absent Penis Spectrum

a b

Fig. 16.1 Diagram of a cloaca. (a) Short common channel. (b) Long common channel

Fig. 16.3 Diagram showing a cloaca with two

Fig. 16.2 Diagram of a cloaca with hydrocolpos hemivaginas

common channel as long as 10 cm; in such cases,
usually two little hemivaginas, as well as the rec-
tum, connect to the urinary tract at the bladder neck
or above the bladder neck (at the trigone) (Fig. 16.5).
We are far from knowing the genetic causes of
this condition [2]. Yet, we have never seen two
cases of cloaca in the same family.
The knowledge of the intrinsic anatomic
characteristics of this malformation is relatively
new. We were able to detect and read old publica-
tions that we think described patients suffering
from cloacas, although were not recognized as
such [3–6].
We were also very impressed by the fact that
most publications prior to 1982 reported high
numbers of rectovaginal fistula cases and very
few cloacas [7]. In retrospect, we are convinced
that the authors were reporting patients suffering
16.1 Cloaca 227

a b

Fig. 16.4 Accumulation of menstrual blood in a patient with obstruction of the Müllerian structures. (a) Diagram.
(b) MRI

Fig. 16.5 Diagram

showing a cloaca with a
very long common channel

Bladder Rectum

Ureter

(R) Hemivagina Long common channel

from cloacas as “vaginal fistulas.” We believe
that because of the large number of cases of clo-
acas that we have seen, coming with a history of
suffering from a “rectovaginal fistula,” yet, when
we examined them, we found an untouched
persistent urogenital sinus and a pulled-down
rectum; in the medical records of those cases, the
word cloaca is not present.
Most publications prior to 1982 reported
very few cases of cloacas; many of them were
228
autopsy findings. The cases that underwent an
attempted repair suffered from a high mortality.
The treatments used include a colostomy at
birth, followed by a rectal pull-through, leaving
the patient with a urogenital sinus to be repaired
“later” [8–23].
The terminology used in those years was also
confusing. The authors frequently published
series that included cloacas and other different
conditions such as “adrenal hyperplasia,” “vagi-
nal atresia,” and “high anorectal malformation.”
One particular publication from 1973 [17] is the
most prominent one because it proposes the full
repair of the vagina. However, looking at the dia-
grams, it becomes clear that the technique used
by Dr. Raffensperger, the author, may be appli-
cable only in cases with a relatively large vagina,
located very low.
All pediatric surgeons as well as hundreds of
patients are in debt with Dr. Hardy Hendren for
his contributions in the field of pediatric surgery
and pediatric urology. His seminal work on the
surgical management of cloacas is the most
important one that we found in our literature
review [24–32]. In his initial publications, Dr.
Hendren referred to this malformation as “uro-
genital sinus and anorectal malformation” [25].
Dr. Hendren’s contribution was particularly
important in dealing with complex reoperations
and repairing the challenging urologic-associated
defects of these patients.
Some authors refer to cloacas with a clear
embryologic bias, and therefore, they used rather
confusing terms such as “urorectal septum mal-
formation sequence” [33] or “urorectal septal
defects” [34] including variants such as adrenal
hyperplasia, as well as male cases [35]. Others
include the cloacas as part of “Müllerian duct
anomalies” [36].
16.1.1.1 Associated Defects
Twenty percent of those patients with a common
channel longer than 3 cm had an absent kidney.
When the common channel was shorter than
3 cm, 17 % of the patients suffered from this
anomaly. Hydronephrosis occurred in 45 and
22 %, respectively, in patients with a common
channel longer or shorter than 3 cm.
16 Cloaca, Posterior Cloaca and Absent Penis Spectrum
Table 16.1 Correlation between sacral ratio and length
common channel
Sacral ratio
Length of Anterior/
common channel Lateral film posterior film Average
Less than 3 cm 0.68 0.55 0.615
More than 3 cm 0.6 0.53 0.56
It is important to notice that in all other ano-
rectal malformations, absent kidney is the most
common anatomic-associated anomaly. The high
incidence of hydronephrosis in this malformation
is consistent with the fact that the most serious
problems that these patients will suffer from
(including death) are urologic.
Vesicoureteral reflux occurs in 40 and 21 %,
respectively, in patients with common channel
longer and shorter than 3 cm.
Most patients with hydronephrosis suffered
from vesicoureteral reflux. At birth, however,
some patients with hydronephrosis and megaure-
ter seemed to suffer from a ureterovesical
obstruction. In reality, the obstruction was an
extrinsic one, caused by a tense hydrocolpos.
When the hydrocolpos was drained, the vesico-
ureteral reflux becomes obvious.
Hemivertebra occurs in 13 % of cases (lum-
bar, thoracic, cervical, and sacral). The average
sacral ratio in patients with cloacas is 0.52 AP
and 0.64 lateral. Table 16.1 shows the correlation
between sacral ratio and the length of the com-
mon channel.
Cardiovascular anomalies occur in 20 % of
cases in cloacas. Patent ductus arteriosus occurs
in 8 % of cases, atrial septum defect in 19 % of
cases, ventricular septum defect in 5 % of cases,
and tetralogy of Fallot in 2 % of cases. Tethered
cord occurs in 36 % of the patients with cloacas.
Esophageal atresia was present in 11 % of our
cloacas and duodenal atresia in 3 %.
16.1.1.2 Goals of Treatment
The treatment of cloacas represents a significant
technical challenge. The final goals of treatment
must result in a patient with urinary control,
bowel control, sexual function, and capacity to
procreate. These goals, of course, are sometimes
achieved, sometimes partially achieved, and
16.1 Cloaca 229

a b

Fig. 16.6 Perineum of a patient with a cloaca. (a) Without separating the labia. (b) Separating the labia

sometimes not achieved at all. For the worst sce-

nario, we are firm in our philosophy that all
patients with anorectal and urogenital malforma-
tions should be clean of stool and dry of urine in
the underwear after the age of three, either
because they were born with a benign malforma-
tion that was adequately reconstructed or because
even when they were born with a malformation
with bad functional prognosis, the patient is
maintained artificially clean of stool (subjected to
a successful bowel management program) [see
Chap. 20] and dry of urine (subjected to intermit-
tent catheterization) through the native urethra or
through a neourethra (continent diversion). Fig. 16.7 “Good-looking perineum.” Obvious midline
groove and prominent anal dimple
Since we are dealing with a spectrum of
defects, one should expect a spectrum of results
after the treatment. perineum includes a single perineal orifice but, in
addition, a completely “flat bottom” with no
16.1.1.3 Neonatal Management traces of sphincter mechanism (Fig. 16.8) and
The diagnosis of a cloaca is a clinical one. It is most likely, a poor functional prognosis. In
enough to look at the patient’s perineum and between those extremes of the spectrum, one can
make the correct diagnosis (Fig. 16.6). These find a variety of external appearances.
patients have a single orifice, yet, the perineum Occasionally, one can find a very large single
has other important characteristics that help to perineal orifice, leaking urine, and with evi-
predict the internal anatomy and the final func- dence of a mild separation of the pubic bones
tional prognosis. A “good-looking” perineum (Fig. 16.9). Those external signs correspond to
consists of the presence of a well-formed midline a patient who has separated pubic bones and a
groove and a well-located and clear anal dimple, malformation called covered cloacal exstrophy
indicating that the patient has a good sphincter [37]. These patients have no bladder neck; their
(Fig. 16.7). On the other hand, a “bad-looking” bladder is very small because it has never been
230
Fig. 16.8 “Bad-looking perineum.” “Flat bottom,”
absence of midline groove, no evidence of anal dimple
Fig. 16.9 Perineum of a patient with a large single peri-
neal orifice. The pubic bones are separated
full. Eventually, these girls will need a total uri-
nary reconstruction. In addition, in these
patients (covered exstrophies), it is very com-
mon to find inside the abdomen the same kind
of anatomic abnormalities seen in cloacal
16 Cloaca, Posterior Cloaca and Absent Penis Spectrum
Fig. 16.10 Grotesque abnormal colon frequently seen in
cases of cloacal exstrophies or in covered cloacal
exstrophies
Fig. 16.11 External appearance of a patient with covered
cloacal exstrophy. The umbilicus is frequently located
lower than normal. Observe low implantation of the umbi-
licus and hemangiomas
exstrophies, mainly the presence of a very short
colon with a very abnormal blood supply
(Fig. 16.10). Yet, the abdominal wall is intact,
which makes a difference with cloacal exstro-
phies (Fig. 16.11).
16.1 Cloaca 231

Fig. 16.12 Pictures of patients with a cloaca and a pseudophallus. Palpation of this structure allows to feel only folded
skin and no real corpora

It is not unusual to find hypertrophic folds of

skin in the area of the single perineal orifice,
which gives a false impression of a phallus
(Fig. 16.12) [38], and that is why 65 cases in our
series came to our institution with the misdiagno-
sis of intersex made at other hospitals. In our
experience of over 531 cases, we only had one
case of gonadal dysplasia associated with a ves-
tibular fistula, but never with a cloaca.
The patients that came to our institution with
a cloaca and with a history of a suspected diag-
nosis of “intersex” described the unpleasant
experience of being told that their baby had an Fig. 16.13 Picture of a cloaca. The single perineal orifice
is very small and is located at the tip of a pseudophallus.
undetermined gender. It usually took a couple This kind of external anatomy is usually associated with
of weeks, with consultation to urology, genet- severe urologic defects and a long common channel
ics, and many laboratory tests, to conclude that
the patients actually were females suffering
from a cloaca. and finally “penis-like clitorises with megaloure-
The key for the diagnosis of those cases with thra in non-virilized female fetus” [43]. Looking
a pseudophallus resides in the palpation of that at the pictures of all those cases presented, it
structure. One can feel that it is actually folded was obvious that all those patients suffered
skin with no palpable corpora. Retrospectively, we from cloacas with normal female gonads and
found five publications referring to this condition chromosomes.
as “pseudohermaphroditism” [39]. Some authors Figure 16.13 shows a single, very small, very
used the term “caudal anomalies” [40], “ambigu- narrow perineal orifice located in the tip of a
ous genitalia with VATER” [41], or “caudal pseudophallus, which usually means that the
developmental field defect with female pseudo- patient has other important associated urologic
hermaphroditism and VACTERL anomalies” [42] malformations and a long common channel.
232
Fig. 16.14 Lipomas in the perineum of patients with cloaca
Fig. 16.15 Preoperative and postoperative appearance after a cloaca repair, including the resection of lipomas
Figure 16.14 shows the perineum of patients
with cloaca and lipomas. Lipomas are relatively
common in the perineum of patients with cloacas
and do not necessarily mean that they require a
more complicated type of treatment. At the time
of the main repair, the lipoma can be easily
excised (Fig. 16.15).
As previously shown, a patient with a cloaca
has a very high likelihood of suffering from a
urologic condition. The first 24 h of life, like in
all other babies with an anorectal malformation,
should be used to rule out the presence of associated
16 Cloaca, Posterior Cloaca and Absent Penis Spectrum
problems that may represent a risk for life. The
most important one, in patients with a cloaca, of
course, is urinary tract obstruction. The baby
must have a kidney ultrasound to rule out the
presence of hydronephrosis and also a pelvic
ultrasound to rule out the presence of hydrocol-
pos and megaureter (Fig. 16.16). A plain abdomi-
nal film in a baby with a single perineal orifice
may show an image of a pelvic mass, as shown in
Fig. 16.17. This represents, most likely, hydro-
colpos that must be drained soon. An ultrasound
that shows hydronephrosis, megaureter, and a
16.1 Cloaca
Fig. 16.16 Pelvic ultrasound of a patient with bilateral
hydrocolpos
Fig. 16.17 Abdominal film of a newborn baby with a
cloaca and a large hydrocolpos
pelvic cystic mass most likely represents a hydro-
colpos that is compressing the trigone and is the
cause of the bilateral megaureters and the
hydronephrosis.
Hydrocolpos as a cause of megaureters and
hydronephrosis has been poorly recognized in
the cases that we have received from other
233
institutions. Many patients with hydronephrosis
and megaureters were subjected to unnecessary,
non-indicated ureterostomies, vesicostomies,
and/or nephrostomies (Fig. 16.18). Most of the
time, the simple drainage of the hydrocolpos
takes care of the problem of megaureter and
hydronephrosis, except in those patients who
have, in addition, vesicoureteral reflux and diffi-
culty emptying their bladder.
Vesicostomies are occasionally indicated in
these babies when we demonstrate that the common
channel is too narrow and interferes with the empty-
ing of the bladder. Also, we have seen an indication
in babies with massive reflux and megaureters.
However, if the patient has a hydrocolpos, the first
step should be the drainage of the hydrocolpos,
prior to making decisions concerning other proce-
dures. Almost always, drainage of the hydrocolpos
is all that is needed to decompress the ureters.
Also, the baby must have an echocardiogram
to rule out cardiac conditions. Esophageal atresia
must be ruled out in the usual manner. A spinal
ultrasound is indicated to evaluate for the pres-
ence of tethered cord, and an x-ray film of the
abdomen will show the characteristics of the
lumbar and thoracic spine, as well as the sacrum,
and a sacral ratio can be calculated.
Between 18 and 24 h after the baby is born, a
decision must be made concerning the surgical
treatment. These babies need a colostomy. The
primary treatment of a cloaca without a colos-
tomy has not been attempted, as far as we know.
In Chap. 5 in this book, we recommended the
opening of a descending colostomy with a
mucous fistula, completely separated from the
proximal stoma and reduced in size to avoid pro-
lapse, since we only need that orifice to perform
irrigations and diagnostic tests (high-pressure
distal colostogram). In patients with cloaca, we
must put more emphasis in being sure that the
patient is left with a piece of colon, distal to the
mucous fistula, long enough to guarantee that the
pull-through will be possible in the future.
If the patient has evidence of hydrocolpos, the
surgeon must be prepared to drain the hydrocolpos
at the same time. We specifically recommend a
midline subumbilical incision that will give the
surgeons access to the entire lower abdomen.
234 16 Cloaca, Posterior Cloaca and Absent Penis Spectrum

a b

Fig. 16.18 (a) Picture of a baby with a cloaca, who underwent a colostomy and a non-indicated vesicostomy. The
vesicostomy is prolapsed because it was not done correctly and the hydrocolpos remained tense and undrained. (b) MRI
showing a sagittal view, the vesicostomy and the undrained hydrocolpos

Through this incision, the surgeon can identify the
junction between the descending and sigmoid
colon. There, the colon is divided and the stomas
are created, separated enough to be able to use a
stoma bag without including the mucous fistula.
The proximal stoma is created in the left flank and
the mucous fistula in the left lower quadrant. At
the same time, through this incision, the surgeon
will be able to drain the hydrocolpos. We prefer to
drain it with a tube. We have used different types
of catheters for this drainage. We specifically rec-
ommend the use of a pigtail catheter that can be
exteriorized through one of the lower quadrants.
We like a curled catheter because it is less likely to
fall out during the initial several months of life as
the inflammation recedes and the vagina moves
away from the abdominal wall. Since most of the
patients with hydrocolpos have both hemivaginas
distended, what we have done in such cases is to
create a window in the septum between both hemi-
vaginas in order to use a single tube to drain both
hydrocolpos. Figures 16.19 and 16.20 show the
aspect of the abdomen of one of these babies with
a cloaca after the colostomy has been opened and
the hydrocolpos has been drained.
Figure 16.21 shows the kidney ultrasound of a
baby with a cloaca born with hydronephrosis and
hydrocolpos: (a) before the drainage of the
hydrocolpos and (b) shows the same patient’s
ultrasound after the hydrocolpos has been
drained. We cannot overemphasize the impor-
tance of draining the hydrocolpos. We have
received a series of patients that had a colostomy,
vesicostomy, nephrostomy, or ureterostomy, but
not drainage of the hydrocolpos. Those patients
had multiple problems, including vesicostomy
prolapse. One of the patients had a pyocolpos,
and another one had a perforation of the infected
vagina with severe peritonitis. Several presented
with failure to thrive, acidosis, and urinary tract
infections, all of which resolved once the hydro-
colpos was drained.
16.1 Cloaca 235

a b

Fig. 16.19 Intraoperative appearance of a case with two large vaginas (bilateral hydrocolpos). (a) Before drainage. (b)
Creation of a “window” in the septum between both hemivaginas

If the bladder cannot empty due to the presence

of a quasi-atresia of the common channel, then a
vesicostomy would be indicated. Also, in the event
of a patient who has a drained hydrocolpos, well-
decompressed urinary tract but severe reflux,
megaureter as demonstrated on a cystogram, and
urinary tract infections, a vesicostomy could be
indicated, with a plan for urologic reconstruction
in the future. Early ureteral reimplantation of
megaureters in a little baby with a bladder that
most likely will have some degree of malfunction
and kidneys with significant congenital damage is
not recommended. We therefore prefer the open-
ing of a temporary vesicostomy, which represents
the best way to protect the kidneys.
Some of the hydrocolpos are giant and may
even interfere with the respiratory function. Also,
some babies with cloacas are extremely sick at
birth; they have ascites, hydronephrosis, high
creatinine, and severe renal damage.
The presence of calcified meconium in the
abdominal film of a newborn baby with a cloaca
[44] may represent a serious sign. Sometimes the Fig. 16.20 Abdomen of a patient with a cloaca showing
the separated stomas and the vaginostomy tube
meconium passes through the fallopian tubes into
the peritoneal cavity producing severe peritonitis.
Once the colostomy and urogenital tract decom- A female baby born with a cloaca that has a
pression has been done, the surgical emergency has colostomy and is not doing well postoperatively
been solved. Patients usually recover very well usually has an undrained obstructed urinary tract
from this operation, and they start eating, growing, with or without hydrocolpos. Therefore, the first
and developing normally. The exceptions, of study in such patients should be an ultrasound to
course, are patients that are born with severe kidney rule out the presence of hydronephrosis,
damage and renal failure, requiring hemodialysis megaureter, large bladder, or hydrocolpos and act
and consideration for a kidney transplant. accordingly.
236
Fig. 16.21 Kidney
ultrasound of a patient a b
born with a cloaca and
hydronephrosis. (a) Before
drainage of hydrocolpos.
(b) After drainage
Another reason why these patients have sepsis
and do not grow well sometimes is because the
colostomy is inadequate. We are strongly opposed
to the opening of loop colostomies in these
babies, because that type of stoma frequently
allows the passing of stool into distal bowel with
direct fecal contamination of the urinary tract.
When the patients are well treated, their colos-
tomy is adequate, and their urinary tract and
hydrocolpos are well drained, they usually recover
very rapidly and can go home. Within several
months, they will be ready for the main repair.
16.1.1.4 Main Repair
In June 1982, for the first time, we had the oppor-
tunity to use the posterior sagittal approach to
repair a cloaca under direct vision. Fortunately,
that first cloaca was what we now consider a
“benign type” of malformation, meaning that the
common channel was relatively short (less than
3 cm), and therefore, we were able to repair the
malformation successfully. That particular
patient today has urinary control, bowel control,
sexual function, and already has successfully
delivered a baby by cesarean section.
During the first few years after 1982, our
approach for the repair of cloacas consisted of
separating the rectum from the urogenital tract
like in all other malformations, followed by the
16 Cloaca, Posterior Cloaca and Absent Penis Spectrum
separation of the vagina from the urethra and
bladder, reconstruction of what used to be the
common channel as a neourethra, mobilization
and dissection of the vagina to be able to pull it
down to be placed posterior to the urethra, and
performing a pull-through of the rectum to be
placed within the limits of the sphincter [45]
(Fig. 16.22). Soon enough, we learned that that
approach was highly successful in a certain type
of malformations that now we call “benign,” but
was not successful in other more complex types.
The main lesson learned during the last 32 years
is that we are dealing with a wide spectrum of
defects [1, 46]. It has been an eye-opening, con-
stant learning experience. The more experience
we develop, the more we understand that the
spectrum seems to be wider and wider. As will be
shown in this chapter, the learning process
allowed us to design surgical maneuvers appli-
cable to different anatomic variants of these
defects. The posterior sagittal anorectovagino-
urethroplasty is the name that we gave to the
repair when it was done in the way that was
already described, meaning separation and mobi-
lization of the three structures (rectum, vagina,
and urethra), done posterior sagittally.
In many patients, the posterior approach was not
enough to repair the malformation, and it was nec-
essary to open the abdomen to complete the repair.
16.1 Cloaca 237

a b

c d

Fig. 16.22 Diagrams and pictures showing the technique vagina being separated from the urethra. The old common
originally used by us, before the advent of the total urogenital channel is reconstructed as a neourethra. (d) Intraoperative
mobilization. (a) Opening. (b) Diagram showing the separa- picture showing rectum and vagina separated. (e) Diagram
tion of the rectum from the vagina. (c) Diagram showing the showing the reconstruction being completed
238
Fig. 16.23 Total urogenital mobilization (diagrams showing the basic concept). (a) Separation of the rectum. (b) Total
urogenital mobilization
In 1996, for the first time, we used an innovative
surgical maneuver that we called “total urogenital
mobilization,” which allows us to reduce the opera-
tive time about 70 %, significantly reduces the
blood loss, makes the operation more reproducible,
and renders better cosmetic and functional results
in the management of cloacas [47] (Fig. 16.23)
(Animations 16.1 and 16.2). Subsequently, we
found that the total urogenital mobilization was not
enough to repair more complex types of defects,
and, therefore, we designed the “transabdominal
extended total urogenital mobilization,” which
allows us to repair cloacas with common channels
16 Cloaca, Posterior Cloaca and Absent Penis Spectrum
between 3 and 5 cm. Yet, even with the use of an
“extended transabdominal” approach, some clo-
acas required further technically demanding
maneuvers, including the complete separation of
bladder and urethra from the genital tract
(Animation 16.3). In order to do that, we had to
open the bladder and pass feeding tubes through
the ureters to avoid their injury. In addition, in some
patients, we perform a maneuver called “carving
the pubic cartilage,” in order to create a shorter tra-
jectory for the urethra and vagina to be pulled down
behind the pubis and to be sutured next to the clito-
ris. In some specific type of cases, we apply a
16.1 Cloaca
(3–5 cm) PSARVUP Total Urogenital Mobilization
Does not reach:
open abdomen -
extended total
urogenital
mobilization
Does not reach:
Separation of vaginas
from urinary tract
(catheters on ureters)
Does not reach:
Big vagina → vaginal switch
Carve
pubic bone
Fig. 16.24 Decision-making algorithm to repair cloacas with a common channel longer than 3 cm
maneuver called “vaginal switch” that will be
described below [48]. In another group of cases, we
have to replace the vagina totally or partially, and
we perform that with the rectum, colon, or small
bowel. Finally, there is a group of cloacas with an
extremely long common channel (more than 5 cm),
in which we leave intact the common channel to be
used eventually as a conduit for intermittent cathe-
terization, and we go directly through the abdomen
to separate the vagina(s), and the rectum, from the
trigone or the bladder neck.
As we learned more about the complexity of
cloacal malformations, we developed a serious
concern about the reproducibility of some of the
techniques used to repair complex cloacas.
Fortunately, more than 50 % of the cloacas have a
common channel shorter than 3 cm. This means
that they can be repaired posterior sagittally, with-
out opening the abdomen and using the maneuver
called “total urogenital mobilization.” We believe
239
Reaches perineum
>5 cm laparotomy
Does not reach:
Small vagina → Vaginal replacement
1 - Rectum
2 - Sigmoid
3 - Small bowel
that the total urogenital mobilization is highly
reproducible, and we believe that most pediatric
surgeons can learn to do it well. On the other hand,
we believe that those cases of cloacas with a com-
mon channel longer than 3 cm must be repaired by
those surgeons specially dedicated and experi-
enced in dealing with these malformations.
Figure 16.24 shows the different steps of the
decision-making algorithm in the repair of clo-
acas. We will describe each one of them.
Cloacas with a Common Channel
of Less Than 1 cm
Figure 16.25 shows a cloaca with a short com-
mon channel. In these cases, we recommend a
relatively simple procedure that we call a
posterior sagittal anorectovaginoplasty. The ure-
thra is left untouched. Basically, what we do in
these cases is to separate the rectum from the
vagina the same way that we do in cases of
240 16
a b
Fig. 16.25 Intraoperative picture of a cloaca with 1 cm
common channel. (a) Exposure – multiple silk sutures in
the rectum. Observe the vaginal septum. (b) Sutures
placed in lateral vaginal walls. The rectum has been
vestibular fistulas (see Chap. 15). Next to that,
rather than separating the vagina from the urinary
tract or performing a total urogenital mobiliza-
tion, we mobilize only the lateral and posterior
walls of the vagina, enough as to be able to suture
the edges of the vagina to the skin of the neolabia
(Fig. 16.25b). By doing that, we do not disturb
the urethra or the common wall between the
vagina and urethra, which is a high-morbidity
type of maneuver. The cosmetic effect of this
operation is excellent. The patients look and
behave basically like a patient operated on for a
rectovestibular fistula. We call this type of cloaca
“cloaca type 1.” The results in terms of bowel and
urinary control are not different from those of
patients with rectovestibular fistulas when they
have a normal sacrum. Figure 16.25c shows the
final result after one of these introitoplasties.
These patients may have mild female hypospa-
dias, which is irrelevant because they do not need
intermittent catheterization and because the ure-
thral meatus is readily visible.
Cloacas with a 1–3 cm Common Channel
Fortunately, 66 % of our patients with cloacas
belong to this type. These patients have, in gen-
eral, a good prognosis. Twenty-eight percent of
them will require intermittent catheterization
after the reconstructive operation, and the bowel
Cloaca, Posterior Cloaca and Absent Penis Spectrum
c
already separated, and the vaginal septum has been
resected. (c) Repaired introitus – the lateral walls of the
vagina are sutured to the labia. The introitus has been
enlarged
function depends very much on the quality of the
sacrum and spine [1, 46].
The procedure to repair these malformations
was performed by us any time from 1 to
12 months of age. If a baby happens to be born in
our institution and is growing and developing
normally, we do it between 1 and 3 months of
life. Most of our patients, however, come from
other institutions, and, therefore, we have experi-
ence doing this procedure at different ages.
We start the operation by performing vaginos-
copy and cystoscopy. We strongly recommend for
the general pediatric surgeon to do the vaginoscopy
and cystoscopy as a separate setting. By doing that,
he or she will be able to measure the length of the
common channel and based on that to:
• Determine whether or not he is capable of
doing that operation or if the patient should
rather be referred to another center.
• Determine whether or not it will be necessary
to open the abdomen for the reconstruction.
This represents important information for the
anesthesiologist as well as the entire operating
team. It helps with equipment needs, predict-
ing operating time, etc.
• Determine the final functional prognosis.
• Determine whether or not the patient needs a
total bowel preparation, in case some form of
vaginal replacement with bowel is necessary.
16.1 Cloaca 241

Fig. 16.26 Diagram of a

posterior sagittal incision
to repair a cloaca

When the vaginoscopy and cystoscopy shows

that the patient has a common channel of 1–3 cm,
we can be confident that we can repair that malfor-
mation using only the posterior sagittal approach
and total urogenital mobilization, without opening
the abdomen. The operation will take us approxi-
mately 3 h. The cosmetic result is excellent, and
the function, in general, is very good.
The patient is placed in prone position with
the pelvis elevated and is washed, prepped, and
draped in the usual manner. A posterior sagittal
incision is used, running from the middle portion
of the sacrum down to the single perineal orifice.
Fig. 16.27 Picture showing the anatomy of the most
We divide the skin, subcutaneous tissue, parasag- common type of cloaca. R rectum, V vagina, U urethra
ittal fibers, and the entire sphincter mechanism
precisely in the midline (Fig. 16.26). The
common channel is opened exactly in the mid-
line, including the vagina and the rectum (when it
is found), exposing the internal anatomy of the
malformation (Fig. 16.27). This step is facilitated
by placing a mosquito clamp in the single peri-
neal orifice, to help guide the midline incision of
the posterior aspect of the common channel.
The first step, as in all cloacas, consists of
separating the rectum from the vagina. When
the patient has two Müllerian systems, the rec-
tum is found in the middle of both hemivaginas.
Usually, it opens in a little orifice located in the
posterior aspect of the vaginal septum. Multiple
5-0 silk stitches are placed around the rectal
Fig. 16.28 Multiple fine sutures are placed on the edges
opening in order to apply uniform traction of the rectal wall, the lateral walls of the vagina, and the
(Fig. 16.28). Special emphasis is placed on common channel
242
a b
Fig. 16.29 Rectal dissection. (a) The beginning of the separation of the rectum from the vagina. (b) Rectum fully
separated
creating a plane of dissection in the common
wall existing between the rectum and the
vagina(s). The use of uniform traction is highly
recommended in order to achieve this. We must
keep in mind that these structures (rectum and
vagina(s)) share a common wall without a natu-
ral plane of dissection. Once the rectum and
vagina(s) are fully separated, a circumferential
dissection is performed, applying uniform trac-
tion on the rectum, dividing the bands and ves-
sels that hold the rectum in the pelvis. As we
progress with this dissection, we keep gaining
length until we have enough rectum to comfort-
ably reach the perineum within the limits of the
sphincter (Animation 16.2)
After we finish that part, in the past (before
1996) [1, 45, 46], we used to separate the vagina
from the urinary tract, which was a technically
demanding maneuver that we do not do anymore
in this type of malformation. It took many hours to
do this, and over 10 % of our patients suffered
from vaginal strictures and/or urethrovaginal fistu-
las as a consequence of that separation [47].
Because of that, in 1996, we switched to the total
urogenital mobilization. For this we place multiple
5-0 silk sutures in the edges of the common chan-
nel and the lateral walls of the vagina to apply a
uniform traction (Figs. 16.28 and 16.29). Another
set of sutures is placed in a horizontal, transverse
fashion, about 5 mm from the clitoris (Fig. 16.30).
The common channel is divided distal to the trans-
verse line of sutures between the clitoris and the
16 Cloaca, Posterior Cloaca and Absent Penis Spectrum
sutures using the needle-tip cautery. The incision
includes the full thickness of the common channel.
A plane of dissection exists between the pubis and
the common channel (Animation 16.2). The sepa-
ration of the common channel from the posterior
aspect of the pubis is a very easy maneuver because
there is an obvious plane, and within a couple of
minutes, we can reach the upper part of the pubis.
Once there, it is relatively easy to identify white,
avascular bands that represent the suspensory
mechanism of the bladder, vagina, and urethra
(Fig. 16.31). These are divided with the cautery as
well as their lateral attachments on both sides of
the vagina. When we divide these suspensory liga-
ments of the vagina and urethra, one can see a
characteristic fat herniating through the fascia.
This is a characteristic retropubic fat pad that indi-
cates that we are in the right plane (Fig. 16.32).
The suspensory ligaments of urethra and vagina
extend onto both lateral walls of the vagina and
must be divided, trying to preserve the blood sup-
ply of the vagina. By doing this division of the sus-
pensory ligaments, we gain approximately 2 cm of
length in the common channel. We then go to the
dorsal part of the vagina(s) and divide the bands,
holding them posteriorly and laterally. By doing
that, we usually gain another centimeter. As a con-
sequence, in most instances, the total urogenital
mobilization allows the mobilization of the vagina
and urethra with a common channel of 3 cm com-
fortably (Fig. 16.33). Occasionally, we were able
to totally repair cloacas with up to a 4.5-cm
16.1 Cloaca 243

a b

Fig. 16.30 Picture showing another set of sutures places horizontally, approximately 5 mm proximal to the clitoris.
(a) Sutures in place. (b) The urogenital sinus is divided between the clitoris and the sutures

common channel only posterior sagittally, using

this maneuver. Other times, we do not know why,
we can only gain 2 cm of length, due to lack of
elasticity and an inflammatory process that we find
in some of these patients.
Once we mobilize the urogenital sinus, we
then split in the midline of what used to be the
common channel, into two lateral flaps
(Fig. 16.34). By doing that, we can suture the
urethral meatus to the tissue behind the clitoris
with interrupted 6-0 Vicryl sutures (Fig. 16.35).
The two flaps that we develop from what used to
be the common channel now become part of the
neolabia that is sutured to the skin with inter-
Fig. 16.31 Picture showing the lateral dissection of the
rupted 6-0 Vicryl sutures (Animation 16.2). The common channel and vagina
lateral walls of the vagina(s) are sutured to the
neolabia until we create a nice-looking introitus
(Fig. 16.36). The electrical stimulator is then front of the levator mechanism. The posterior
used to determine the limits of the anal sphincter, edge of the levator muscle is sutured with inter-
which are marked with temporary silk stitches. rupted 5-0 Vicryl sutures in the same way that it
The perineal body is reconstructed between the is done in all other malformations. The posterior
posterior limit of the vagina and the anterior limit edge of the muscle complex on each side is
of the sphincter. We use 4-0 Vicryl or 5-0 Vicryl sutured together in the midline, taking with the
sutures to bring together the tissue of the perineal same stitches a bite of the posterior rectal wall.
body. These stitches are very important because The ischiorectal fossa and the subcutaneous tis-
they represent the main supporting mechanism to sue are both closed with interrupted 5-0 Vicryl
avoid dehiscence of the perineum. The skin of the sutures, and the skin is closed with subcuticular
perineal body is sutured usually with interrupted 5-0 monofilament, absorbable sutures. The ano-
6-0 Vicryl or 5-0 Vicryl sutures. By doing this, plasty is performed with 16 circumferential
we bring together the anterior limits of the anal stitches of 6-0 long-term absorbable sutures after
sphincter (Animation 16.2). The rectum then is we resect the excessive rectal tissue, usually
placed within the limits of the sphincter and in between 5 and 10 mm (Fig. 16.37).
244 16 Cloaca, Posterior Cloaca and Absent Penis Spectrum

a b

Fig. 16.32 The suspensory ligaments of urethra and vagina. (a) Exposure – observe whitish fascia. (b) Divided sus-
pensory ligaments (observe retropubic fat)

Fig. 16.33 Picture showing a fully mobilized urogenital Fig. 16.34 The urogenital sinus (original common
sinus channel) is divided in the midline

These patients do very well and can eat the
same day of surgery. The patients stay in the hos-
pital approximately 48 h. A Foley catheter
remains in place for approximately 2 or 3 weeks.
We must keep in mind that about 20 % of these
patients may eventually require intermittent cath-
eterization, and, therefore, we leave the Foley
catheter until the postoperative inflammatory
process allows us to see where the urethral
meatus is located, in case the patient needs
intermittent catheterization, before we pull the
catheter out. Two weeks after surgery, the parents
come to the clinic, we teach them how to dilate
the anal orifice, and they do it following our pro-
tocol of anal dilatations as previously described.
Prior to the colostomy closure and under the
same anesthesia, a vaginoscopy and cystoscopy are
performed to confirm that the urethra and vagina are
patent and healthy. In the event of finding problems
with these, they have to be taken care of, prior to the
16.1 Cloaca 245

a b

c d

Fig. 16.35 Urethral opening repositioning and resection immediately behind the clitoris. (b) Sutures are tied.
of the vaginal septum. (a) Fine long-term absorbable (c) Resecting the vaginal septum. (d) Vaginal septum
sutures are used to anastomose the urethral opening resected

Fig. 16.36 Suturing vaginal walls to the neolabia Fig. 16.37 Final external aspect of a repaired cloaca
246 16 Cloaca, Posterior Cloaca and Absent Penis Spectrum

a b c

Fig. 16.38 External vaginoplasty to enlarge a strictured vaginal orifice. (a) Narrow vaginal orifice R rectum, U urethra,
V vagina. (b) Longitudinal incision of the posterior aspect of the anal opening. (c) Horizontal suture

colostomy closure. If, for instance, at the time of

colostomy closure, we find a narrow ring-like, vagi-
nal opening with a wide, deep compliant vagina, we
may do nothing at this age. On the other hand, if the
orifice is too narrow and we believe it is at risk of
closing completely, then we perform an external
vaginoplasty to make the orifice larger and post-
pone the colostomy closure for a month (Figs. 16.38
and 16.39). On the other hand, if the patient has a
long, narrow vaginal stricture, she may need a com-
plete reoperation. We frequently saw this kind of
complication prior to the advent of the total urogen-
ital mobilization. The total urogenital mobilization
prevents this complication from happening most of
the time since it preserves a very good blood supply
for the vagina and the urethra.
After the total urogenital mobilization, some
patients leak urine for a period of several weeks.
A voiding cystourethrogram at this point may
show an image consistent with an absent bladder
neck, due to the pulling of the whole urogenital
sinus from below. This happens mainly when the
repair required a significant traction of the uro-
genital tract. However, most of these patients
recover normal urinary function after a few
weeks. The colostomy can be closed following
the same principles that we mentioned in the Fig. 16.39 Enlarged vaginal orifice. Large Hegar dilator
chapter related to colostomy closure. inserted
16.1 Cloaca 247

Cloacas with a 3- to 5-cm Common

Channel (Animation 16.3)
When the endoscopy allows us to determine that
the patient has a common channel length of
3–5 cm, we perform a total body preparation as
described in Chap. 11 because we know that most
likely it will be necessary to open the abdomen,
in addition to the posterior sagittal approach, to
repair the malformation. We tell the anesthesiolo-
gist about these findings and the possibility that
the operation will go for a longer period of time
than one that could be done posterior sagittally
only. After we perform a total body preparation,
Fig. 16.40 Intraoperative picture showing the bladder
we put the patient in the prone position with the pulled caudally to have a better access to the pelvic floor.
pelvis elevated and perform the same posterior B bladder, Ut uterus, Ur ureter
sagittal incision as previously described. The
internal anatomy of the malformation is exposed;
the rectum is separated from the urogenital tract
in the same way as previously described. A total
urogenital mobilization is performed as previ-
ously described. Occasionally, we are happily
surprised to find out that it is possible to recon-
struct the urethra, vagina, and rectum without
opening the abdomen. However, most of the
time, the total mobilization is not enough to
repair the malformation. In such a case, the
patient is turned to the supine position and the
abdomen is opened with a midline infraumbilical
incision.
The next step is to perform what we call
“extended transabdominal urogenital mobili- Fig. 16.41 Intraoperative picture showing bladder and
urogenital sinus out of the pelvis. Us urogenital sinus, B
zation.” Traction is applied to the dome of the bladder
bladder; the lateral attachments of the bladder
are divided to obtain a better exposure
(Fig. 16.40). The midline incision is extended urogenital complex and repair the urethra and
all the way down to the pubis. Between the vagina as previously described. If that is not
bladder and posterior aspect of the pubis, one enough to achieve a tension-free anastomosis
can see the space that we created from below between the urethra and clitoris and vagina and
with the total urogenital mobilization. The uro- neolabia, a maneuver called “carving of the
genital sinus is then brought up through this pubic cartilage” is indicated.
space between the bladder and pubis
(Fig. 16.41). At this point, we divide all the Carving of the Pubic Cartilage Maneuver
pelvic avascular attachments of the bladder Under normal circumstances, the urogenital
and urethra. Through the laparotomy, these sinus is located behind the pubic cartilage; it
attachments are easily seen and divided. runs below the cartilage and up, anterior to the
Usually this maneuver allows us to gain extra cartilage to connect to the clitoris. Resecting
length on the urogenital mobilization. If that is approximately 50 % of the lower portion of the
enough to complete our repair, we then go pubic cartilage does not compromise the pelvis
ahead and pull through back down the stability, and yet, it allows a straighter trajectory
248 16 Cloaca, Posterior Cloaca and Absent Penis Spectrum

a b

Fig. 16.42 Carving the lower part of the pubic cartilage to create a shorter trajectory of urethra and vagina. (a) Before
carving. (b) After carving. (c) Urethra sutured – arrow in urethral opening

of the urogenital sinus. The resection of the car-
tilage can be done easily, with the needle-tip
cautery on “cutting” mode in babies. In older
patients, this can be done with a “rongeur” type
of instrument. This maneuver may allow for a
tension-free anastomosis between the urethra
and vagina with the clitoris and neolabia
(Fig. 16.42). This maneuver may work in cases
that need 0.5–1 cm of extra urethra and vaginal
length to achieve a tension-free anastomosis. If
that is not enough, then the next step must be
the separation of the vagina from the urinary
tract.
Separations of Vagina(s) from the Urinary
Tract (Animation 16.3)
This is the most technically demanding maneu-
ver of the entire repair of cloacas. This procedure,
in the past, before the total urogenital mobilization,
was attempted from below, but it was very
difficult to do. Now that we do the total urogeni-
tal mobilization, we do not have to separate the
vagina from the urinary tract in cases with com-
mon channel shorter than 3 cm. Yet, in cases with
longer common channel, we must separate both
structures through the abdomen. The separation
is done through a laparotomy but with bladder
and vagina(s) fully mobilized and out of the
abdomen. The bladder is opened in the midline,
and feeding tubes are introduced through each
one of the ureters (Fig. 16.43a). We must keep in
mind that in cloaca patients, both ureters pass
through the common wall between the vagina
and the bladder. The separation of these two
structures may include the skeletonizing and dis-
section of both ureters. If the patient suffers from
reflux, this is a golden opportunity to perform a
ureteral reimplantation or, if appropriate, a cuta-
neous ureterostomy; otherwise, to do it later
would represent a technically more demanding
16.1 Cloaca
a b
Fig. 16.43 Intraoperative pictures taken during the separa-
tion of vagina from the urinary tract. (a) Bladder open and
catheters placed in the ureters. (b) The assistant puts fingers
procedure. The assistant puts two fingers inside
the bladder and applies traction caudally into the
bladder (Fig. 16.43b). Vicryl sutures are used to
pull the uterus or hemiuterus in the opposite
direction. A plane is created in the middle of the
wide common wall that exists between the
vagina(s) and the urinary tract. This common
wall extends from the urethra and includes the
bladder neck, trigone, and part of the bladder. In
general, the most technically demanding steps of
the operations designed to repair anorectal mal-
formations is actually the separation of the
structures (rectum, genitalia, and urinary tract).
We are supposed to separate them without dam-
aging them. This is difficult to achieve for several
reasons:
(a) Those structures are congenitally fused with-
out a plane of separation.
(b) The common wall has a very rich blood
supply.
(c) The exposure is difficult because these struc-
tures are located in a place difficult to reach
from below or from above.
(d) The ureters run through this common wall.
The separation of structures usually takes
about 70 % of the total operative time. To
achieve a good repair, it is necessary to achieve
a good separation of these structures with mini-
mal or no damage. The previously described
“extended transabdominal total urogenital mobi-
lization” allowed us to perform the separation of
249
into the bladder and thumb outside the bladder, pulling it
caudally. Traction sutures are placed in the uterus to apply
traction and facilitate the dissection. B bladder, Ut uterus
structures basically outside the abdomen. The
surgeon works from above between the bladder
and the vagina(s). We perform the entire dissec-
tion with a fine needle-tip cautery. During the
dissection, the surgeon must stop frequently to
verify that the thicknesses of the vaginal wall, as
well as that of the bladder, are equal. In other
words, the surgeon does this to be sure that nei-
ther of those walls are becoming too thin.
Intermittently, this dissection is interrupted to
palpate the location of the ureters (previously
catheterized). Also, it is convenient to perform
part of the dissection backwards, meaning from
caudal to cephalad, since the common channel is
exposed and brought up through the incision.
We keep dissecting a little bit from below and
then from above, until both dissection planes
meet. At that point, the only structures that join
the genitourinary structures to the patient’s body
are the ureters, the ovarian vessels, and the inter-
nal iliac vessels. These vessels must be kept in
mind and carefully respected.
Figure 16.44 shows the posterior aspect of the
bladder and trigone. Both ureters can be seen
intact.
Once the separation has been achieved, the
surgeon can plan the type of reconstruction that is
best for the patient’s specific anatomic variant.
The first possibility is that after the separation,
one becomes happily surprised to find that the
vagina(s) actually reaches the perineum. This is
250
Fig. 16.44 Picture taken after the bladder and vagina
have been separated. The ureters can be seen intact.
Arrows on ureters. Black retractor pushing down uterus
and vagina
the ideal time to remove the vaginal septum that
separates two hemivaginas (if present) to trim off
damaged vaginal tissue and tubularize the avail-
able vaginal tissue in preparation for the
pull-through.
The total separation of vagina(s) and urinary
tract runs with the implicit risk of devasculariza-
tion of the distal urethra. It is necessary to be sure
that the urethra that is sutured immediately
behind the clitoris has a good blood supply. At
least ten of our patients suffered from an
ischemic-acquired urethral atresia after one of
these operations performed by us.
If the urethra’s blood supply is severely defi-
cient, the surgeon must make a decision about the
possibility of permanently closing the bladder
neck. In that case, the patient needs a vesicostomy;
subsequently, an artificial conduit for bladder
catheterization will be done (Mitrofanoff prin-
ciple) [49]. Later in the patient’s life (3–4 years),
the functional and anatomic characteristics of the
bladder can be studied to determine its capacity,
detrusor activity, compliance, as well as the pres-
ence of vesicoureteral reflux, and a final recon-
struction can be planned, which may include a
bladder augmentation and a Mitrofanoff type of
procedure.
Planning the reconstruction of the vagina, ure-
thra, and rectum, after the total separation of the
structures, the surgeon will face one of several
scenarios, and based on those, he/she will make a
16 Cloaca, Posterior Cloaca and Absent Penis Spectrum
Fig. 16.45 Anatomic characteristics of a case that will
benefit from a vaginal switch maneuver
decision. The first possibility will be that the
patient has anatomic characteristics that make
her suitable for a surgical maneuver called “vagi-
nal switch.”
Vaginal Switch
This maneuver is applicable only when the
patient has a specific type of anatomy
(Fig. 16.45). These patients have two hemivagi-
nas very separated, as well as the hemiuteri with
a vaginal septum and originally two large hydro-
colpi. If we can estimate that the distance
between one hemiuterus and the other is longer
than the vertical length of both hemivaginas,
then the patient may be a candidate for the vagi-
nal switch maneuver [48]. As can be seen in
Fig. 16.46, the maneuver consists in sacrificing
one of the hemiuteri, being careful enough to
preserve the ovary and its blood supply. The
vaginal septum is removed. Both dilated hemi-
vaginas are tubularized into a single vagina, and
what used to be the dome of one side, the place
where we resected the hemiuterus, becomes the
lowest part of the new switched-down vagina
(Fig. 16.46) (Animation 16.4). This maneuver
works, and we have several patients menstruat-
ing through this type of repair, but it is only
applicable if the patient has the specific type of
anatomy already described. If, early in the oper-
ation, one estimates that the anatomy of the
patient belongs to this category, one has to sepa-
rate completely only one hemivagina from the
urinary tract and try to preserve the blood sup-
ply of the opposite side. In this type of maneu-
ver, the blood supply of the entire switched
16.1 Cloaca 251

R.tube
R. hemiuterus Branches to tube

Divide Vaginal septum

R.OVARY Lo Ovarian a.
rin
e
ar
te
ry
R. giant Va
gin
hemivagina al
art
eri
hydrocolpos es

Communication with
urinary tract
and/or rectum

a Perineum

b Branches to tube

Preserved
L. hemiuterus
Preserved
ovary

Ovarian a.
( Right hemihisterectomy ) in
e
ar
te
Resected Va ry
vaginal septum gina
la
r te
rie
s

R. hemivagina
switched down

Fig. 16.46 Schematic representation of the basic princi- other hemivagina and ovaries. The vaginal septum is
ples of a vaginal switch maneuver. (a) One hemiuterus resected and both large hemivaginas are tubularized
will be amputated. The blood supply of that hemivagina is together. (b) What used to be the dome of one hemivagina
sacrificed being careful to preserve the blood supply of the is pulled down to create the introitus
252
a b
Fig. 16.47 Vaginal replacement with part of a dilated rectum. (a) Diagram. (b) Intraoperative picture. V vagina,
R rectum
vagina will depend on the preservation of the
blood supply of the opposite hemivagina. If one
can see that the distance between both hemiuteri
is not long enough, then we have to separate
both hemivaginas completely from the trigone
and urinary tract as previously discussed.
The next possible scenario could be the case
of a patient in whom we have gone through all
the steps previously described, and her anatomy
does not make her suitable to be repaired using a
vaginal switch maneuver. The vagina(s) is too
short and/or located too high in the pelvis. Under
those circumstances, the patient will need a vagi-
nal replacement.
Vaginal Replacement
Vaginal replacement has been done by many
authors through many years. Most authors includ-
ing us prefer to use the colon [50–59]. Others
used local tissues expanded with different meth-
ods [60–62].The amniotic membrane has also
been used [63].
We have developed a significant experience in
130 cases suffering from cloaca that required
vaginal replacement. Our order of preference in
terms of tissue to be used for the replacements
are rectum 50 cases, colon 44 cases, and finally,
small bowel 36 cases.
16 Cloaca, Posterior Cloaca and Absent Penis Spectrum
Vaginal Replacement with Rectum
There are several ways to replace the vagina with
rectum. It all depends on the anatomic character-
istics of the patient. If the patient has a very
dilated rectum, conceivably, we can divide the
rectum longitudinally, preserving the blood sup-
ply of both portions, the one that is going to be
the neovagina and the other one that will remain
as rectum (Fig. 16.47a). The vagina and the rec-
tum are both tubularized. Both structures are
rotated 90° in opposite directions to avoid the
overlap of two suture lines, which is an important
predisposing factor for the formation of a fistula
(Fig. 16.47b). If the patient has no internal
genitalia (rarely occurs), then the vagina is cre-
ated only for sexual purposes and is not anasto-
mosed to any internal structures. On the other
hand, if the patient has an internal genitalia, then
we have to perform an anastomosis between the
upper portion of the neovagina (old rectum) and
the original native short vagina.
The longitudinal incision of the rectum to cre-
ate two separated tubular structures (vagina and
rectum), preserving a good blood supply of both
of them, is an interesting technical maneuver
worth describing in detail.
Through all these years, we have learned that
the rectum has an excellent intramural blood
16.1 Cloaca
Fig. 16.48 Finished vaginal replacement
supply. It is perhaps the only hollow viscus of the
human body that can be deprived, to a limit, of
its extrinsic blood supply and still survives due
to an excellent intramural blood supply, provided
it maintains its continuity with a piece of colon
with good blood supply. This is only possible if
the surgeon manages to maintain intact the bowel
wall. In other words, we can dissect and mobi-
lize a significant length of rectum, burning and
dividing the vessels that represent its extrinsic
blood supply and still have a good blood supply,
provided we perform the dissection as close as
possible to the bowel wall but without injuring
it and provided the upper rectum receives a good
blood supply from inferior mesenteric vessels.
Figure 16.47 shows the longitudinal division of
the rectum and the tubularization. As can be seen,
the blood supply of the rectum after this maneu-
ver will depend entirely on the intramural blood
supply. Obviously, the piece of rectum designated
253
to replace the vagina must receive its blood sup-
ply from at least one or two of the inferior mesen-
teric vessels. The lower anastomosis is performed
between the neovagina (rectum) and the neolabia.
The perineal body is reconstructed in the usual
manner as well as the rectum (Fig. 16.48).
In the case of a patient with a non-dilated rec-
tum but with plenty of available length, we can
plan on using the most distal part of the rectum as
a neovagina and mobilize the upper rectum or sig-
moid as a neorectum (Fig. 16.49a). In order to do
that, it is imperative to learn to preserve the blood
supply of the distal rectum. One must keep in mind
that the mesentery of the rectum is completely dif-
ferent from the mesentery of the rest of the gastro-
intestinal tract. In the small bowel and colon, the
mesentery reaches the bowel on the so-called mes-
enteric side only. It is very obvious that the rest of
the bowel has no mesentery and no fat, just the
seromuscular layer. The rectum, on the other hand,
is surrounded by fatty tissue with vessels, which
has received the name of mesorectum, and sur-
rounds basically the entire circumference of the
rectum. In order to preserve the blood supply of
the distal rectum, it is necessary to create a plane
of separation between the bowel wall itself and the
fat tissue with vessels (Fig. 16.49b). The dissec-
tion between the mesorectum and bowel wall is
carried out all around the rectal wall at the place
where we previously planned that the rectum
would be divided, leaving the distal part as the
neovagina and the upper part as the neorectum.
Prior to making the decision to divide the rectum,
we must be absolutely sure that we have enough
bowel length proximally to reach the perineum
with no tension. We must keep in mind that occa-
sionally the presence of a sigmoid colostomy cre-
ated too distal may interfere with our plan.
Like previously mentioned, if the patient has
no internal genitalia, the neovagina (original rec-
tum) is closed blind on its upper end and will be
used only for sexual purposes.
There are other ways to replace the vagina
with rectum using techniques of combined longi-
tudinal section with the use of the distal bowel
(Fig. 16.50), depending on the specific anatomic
circumstances.
254
a b
Fig. 16.49 Vaginal replacement with the distal part of
rectum, in patients who have plenty of rectosigmoid
length. (a) Diagram. (b) Intraoperative picture. Dissecting
Fig. 16.50 Vaginal replacement with rectum. Other tech-
nical alternative
If the rectum is not adequate to be used for
vaginal replacement, then our next choice is
the colon.
16 Cloaca, Posterior Cloaca and Absent Penis Spectrum
the mesorectum from the rectal wall. D distal rectum, M
mesorectum, P proximal rectum
Vaginal Replacement with Colon
In the past, we have typically used the sigmoid
colon when available (Fig. 16.51). Frequently,
the colostomy interferes with this, and that is
why, in patients with cloacas who may require
vaginal replacement, a higher type of colostomy
has been recommended by Hendren [31]. Lately,
however, in spite of the patients having a colos-
tomy located in the left lower quadrant, we have
found that the left transverse colon and the
descending colon have a nice vascular arcade of
vessels that make that part of the colon ideal for
vaginal replacement (Fig. 16.52) (Animation
16.5). Therefore, we have chosen to take the
colostomy down and use that part that used to be
the colostomy to replace the vagina. The pedicle
of the left colon graft often reaches the perineum
more easily than the sigmoid would.
Vaginal Replacement with Small Bowel
This is our last choice, because it is our impres-
sion that the blood supply of the small bowel is
more delicate and more prone to suffer from
occlusion with a mild twist of the pedicle of the
bowel that has been pulled down. When we
decide to use the small bowel, we prefer to use
16.1 Cloaca
Fig. 16.51 Diagram of vaginal replacement with sig-
moid colon. Dashed lines show the limits of bowel resec-
tion and vessel ligation
terminal ileum. We made an interesting obser-
vation; the length of the mesentery at the cecum
represents the length of the superior mesenteric
axis (Fig. 16.53). If one takes the terminal
ileum about 10 cm proximal to the ileocecal
valve, it is easy to see that the mesentery is lon-
ger than at the cecum, and if we go even more
proximal, the mesentery is even longer. The
longest mesentery of the small bowel seems to
be located about 15 cm proximal to the ileoce-
cal valve, and because of that, we thought that
this part of the bowel would be ideal to be used
for vaginal replacement. Accordingly, we
observe the blood supply to decide which ves-
sels divide and which ones to preserve to be
sure that the bowel reaches the perineum
(Fig. 16.54). Again, one must be extremely
meticulous in the way we pull the bowel,
because a little twist will produce ischemia and
we may lose the graft. If the patient has internal
genitalia, then, as previously mentioned, the
small bowel (neovagina) is to be anastomosed
to the internal genitalia and to the new labia. On
the other hand, if the patient has no internal
255
genitalia, the bowel in the upper part must be
closed blind, and we have only to do an anasto-
mosis to the neolabia (Fig. 16.55).
Cloacas with Extremely Long Common
Channels
When the endoscopy allows us to see that the
patient has an extremely long common channel
(more than 5 cm), it is conceivable that the separa-
tion of the structures (rectum, vagina, and urinary
tract) could be done easier through a laparotomy
rather than through the perineum or posterior sag-
ittally (Animation 16.5). In these cases, the anat-
omy is like the one illustrated in Fig. 16.5. The
rectum opens either in the bladder neck or in the
trigone, and two little hemivaginas open also in
the trigone or in the bladder neck in a perpendicu-
lar fashion (with no common wall). The ureters
also open in that particular area. These patients
therefore have about five important structures
opening in the same area of the urinary tract. The
separation of these structures usually leaves the
patient with no bladder neck or with a damaged
one. Good clinical judgment and experience are
required to make a decision about reconstructing
the bladder neck or closing it on a permanent
basis. In the last circumstance, the patient will
need a vesicostomy, and eventually, she will
require a bladder augmentation and a Mitrofanoff
type of procedure. On the other hand, frequently
we separate these structures carefully and we are
left with a bladder neck that looks like it can be
reconstructed successfully, and we have cases in
which this reconstruction has been successful.
The rationale that supports the idea of
approaching these patients through a laparot-
omy first is that we can leave intact what used to
be the common channel as a conduit for inter-
mittent catheterization; that will work beauti-
fully, provided the bladder neck is reconstructed
adequately.
It has been our impression that, in general,
patients with cloacas have a good bladder neck. If
this bladder neck is preserved or reconstructed
adequately, these patients have no problems hold-
ing urine in the bladder. The main type of bladder
malfunction that we see in these patients is inca-
pacity to empty. Most cloaca patients operated on
256 16 Cloaca, Posterior Cloaca and Absent Penis Spectrum

a b

Fig. 16.52 Intraoperative pictures of a vaginal replacement with descending colon. (a) Studying and selecting the ves-
sels that must be preserved. (b) Measuring the length of colon. (c) Creation of the introitus
16.1 Cloaca 257

have a smooth, large, floppy bladder with a good

Fig. 16.53 Vaginal replacement with small bowel. The
mesentery of the small bowel is longest in the terminal
ileum, about 15 cm proximal to the ileocecal valve.
Arrows show the direction of the traction
bladder neck. They cannot empty the bladder;
therefore, during the period of filling up of the
bladder, the patient may remain completely dry
in the underwear. Once the bladder is completely
full, they start dribbling urine as an overflow type
of phenomenon. This makes these patients ideal
candidates for intermittent catheterization.
However, in most types of cloacas, the common
channel is surgically manipulated and, therefore,
sometimes is not regular and smooth enough to
allow a successful and easy catheterization.
In these extremely high types of cloacas, with
a very long common channel, we insist on leav-
ing the common channel untouched and to
approach the patient initially directly through the
abdomen, preserving intact the common channel,
to be used as a conduit for catheterization. The

a b

Fig. 16.54 Intraoperative picture. Vaginal replacement with small bowel. Selecting the vessels to be preserved. (a)
Selecting the vessels. (b) Dividing the vessels preserving the arcade. (c) Dividing the bowel
258
Fig. 16.55 New introitus made with small bowel
higher the malformation, the shorter the common
wall between the bladder and vaginas, which is
why it is relatively easy to separate vaginas and
urinary tract through a laparotomy.
Occasionally, the laparotomy is indicated
because of a very high rectum and not so much
because of the urogenital tract. Sometimes, the rec-
tum opens very high in between both hemivaginas
and cannot be reached posterior sagittally, even
after the total urogenital mobilization has been per-
formed, and, therefore, one has to go into the abdo-
men just to look for the rectum and pull it down.
The posterior sagittal approach, as well as the
total urogenital mobilization, has been adopted
by many pediatric surgeons, with variable results
[64–73]. Other types of repair have been also
advocated, although with very few cases [74–80].
An interesting report from China was recently
published [81]; the authors claimed that they
were able to use serial mechanical dilatations of
the common channel and eventually they were
16 Cloaca, Posterior Cloaca and Absent Penis Spectrum
able to create a urethra and vagina from the
dilated common channel!!
At the end of the cloaca repair, we must decide
to leave our patient with a Foley catheter through
her native urethra, with a suprapubic cystostomy
or with a vesicostomy. In general, we make that
decision based on the following principles:
• Foley catheter: Patients with a common chan-
nel shorter than 3 cm, repaired via posterior
sagittal approach, with a good sacrum, and no
tethered cord. We assume that after 2 weeks of
having the catheter, the patient will be able to
void satisfactorily.
• Suprapubic cystostomy: Patients who required a
laparotomy and we assumed that will need the
catheter for a period of time shorter than 3 months.
It is very useful because it allows us to evaluate
the bladder function and make decisions, during
the following 3 months post operation.
• Vesicostomy: Patients with poor renal func-
tion and or tethered cord, poor sacrum, vesico-
ureteral reflux, megaureter, hydronephrosis,
and single kidney.
16.1.1.5 Postoperative Care
Since we are dealing with a spectrum of defects,
the postoperative care represents also a wide spec-
trum. A patient who has a cloaca type 1 can eat the
same day and go home the next day after surgery.
A patient who has a common channel shorter than
3 cm also can eat the same day of surgery and stay
in the hospital 48 h. A patient who requires an
8–12-h operation may remain intubated overnight
in the intensive care unit. We have found that little
babies subjected to long surgical procedures
(more than 6 h) may need to remain intubated and
recover in the intensive care unit. It is our experi-
ence that these patients retain a lot of fluid in their
bodies and may have respiratory problems. It
takes several days for them to get rid of the exces-
sive third-space sequestered fluid.
Little babies subjected to long operations
should not be treated in institutions without an
adequate, third-level intensive care unit, with
experts in the management of these types of criti-
cally ill patients.
Some of these patients already have significant
kidney damage and elevated creatinine at birth that
16.1 Cloaca
Fig. 16.56 Picture showing a significant rectal prolapse
in a case with a repaired cloaca
requires special care by an expert team of nephrol-
ogists. We have seen that after these operations,
the creatinine becomes elevated even without any
evidence of urinary tract obstruction.
Sometimes, we repair these malformations and
pull the colostomy down at the same time. In those
cases, the patients remain with nothing by mouth,
receiving parenteral nutrition for 7–10 days. The
Foley catheter remains in for 2–3 weeks until the
urethral orifice is visible, and a catheter could be
reinserted if necessary.
Two weeks after surgery, the protocol of anal
dilatations is started like we do in all the other types
of malformations. At the time of colostomy closure,
the patients need a vaginoscopy and cystoscopy to
be sure that the repair was successful. In the event of
finding any problems with the repair, the colostomy
closure should be canceled, and we should focus on
repairing the vagina or urethra as necessary.
Sometimes at the time of colostomy closure,
we find that the patient has a significant rectal
prolapse (Fig. 16.56). If that is the case, we repair
the prolapse and postpone the colostomy closure
259
Fig. 16.57 Prolapse of neovagina created with bowel
at least for 1 month. Occasionally, when the
vagina has been replaced with colon, rectum, or
small bowel, one may also find some degree of
bowel mucosa prolapse as part of the neovagina
(Fig. 16.57). Again, this should be treated before
the colostomy is closed because it can be done in
a single-day admission and because the perineum
is completely clean. A long, narrow stricture of
the vagina requires a full reoperation. Fortunately,
that is unusual in our cases.
Sometimes, we find that the urethra is difficult
to catheterize. In such a case, it might be appropri-
ate to open a vesicostomy and delay the need for
intermittent catheterization until the baby is older.
At the time when we were doing repairs with-
out the total urogenital mobilization, 10 % of our
patients suffered from urethrovaginal fistulas.
260
Usually, these fistulas were located near the
bladder neck and produced urinary incontinence,
and therefore, the patient required a total reop-
eration. The advent of the total urogenital mobi-
lization allowed us to have no more of these
complications [47].
16.1.2 Urologic Concerns
The main long-term problems of patients with
cloacas are usually urologic. These patients do
not die from the cloaca malformation, but rather
from major cardiac anomalies early in life or
from kidney failure later on. The urologic con-
cerns of patients with cloaca have been recog-
nized for a long time [82]. There are four
wonderful publications related with the long-
term urologic and renal outcome of patients with
cloacas [83–86]. The authors presented series of
12–64 cases that allowed them to reach valid con-
clusions. From this experience, as well as ours, it
becomes clear that treatment of cloacas must be
done by experienced pediatric surgeons and pedi-
atric urologists. Protecting the kidneys must be
the top priority from the first day of life. In addi-
tion, it is clear that these patients must be fol-
lowed to be sure that the bladder function does
not deteriorate and affect the kidneys.
In addition, two publications alerted us about
the possibility that the total urogenital mobiliza-
tion could provoke nerve damage that could
result in poor bladder function [87, 88]. Based on
our experience, we believe that patients with nor-
mal sacrum, no tethered cord, and with a com-
mon channel shorter than 3 cm should not have
serious urinary problems, provided the total uro-
genital mobilization has been performed in a
meticulous way. A similar experience was pub-
lished by others [89–93].
We have learned that when a patient is born
with hydronephrosis, we must anticipate that the
patient already has a significant degree of kidney
damage. Even if they have a normal creatinine,
one must expect that at some point later in life,
the patient may develop kidney failure. This is
particularly true when the patient has a single
kidney and hydronephrosis at birth. Therefore,
16 Cloaca, Posterior Cloaca and Absent Penis Spectrum
one must be rather obsessive about the protection
of the kidneys, particularly if it is a single one.
We must take care of the reflux, obstruction, and/
or infection. Frequently, we are confronted with
the problem of a little baby with megaureters, an
“inadequate bladder,” and severe vesicoureteral
reflux. For that kind of patient, we prefer to open
a vesicostomy and wait until the ureters decrease
in size, the bladder becomes more compliant, and
at that point, the reimplantation of the ureter may
have more chances of success. The reimplanta-
tion should be done after the bladder has been
urodynamically studied to determine its func-
tional characteristics. We also must determine the
mechanism of emptying of the bladder, either
because the bladder empties spontaneously or
needs intermittent catheterization.
Real ureterovesical obstructions without
hydrocolpos rarely occur in patients with cloacas.
In the presence of a true ureterovesical obstruc-
tion, during the repair of a complex cloaca in a
baby, with megaureter and a bladder with ques-
tionable function, we take care of the obstruction,
creating a wide refluxing ureterovesical anasto-
mosis and a vesicostomy. That represents the best
protection for the kidney. We then wait until the
ureter decreases in size, the patient grows up, and
the bladder becomes more compliant. An evalua-
tion of the bladder function then will allow one to
determine the best course of action for the patient.
We do not see a reason to do bladder augmenta-
tions and Mitrofanoff procedures in babies before
3 years of age, particularly, if the patients already
have a certain degree of kidney damage. So far, 46
patients already underwent a bladder augmenta-
tion and a Mitrofanoff type of operation. Twenty-
seven of those were done by the senior author and
19 by our pediatric urology colleagues.
These are patients for life and require a team
of urologists, nephrologists, gynecologists, and
colorectal pediatric surgeons to follow them.
The patient’s bowel function will depend very
much on the characteristic of the sacrum and spine.
At the age of three, if the patient has no bowel con-
trol, the patient is offered our bowel management
program (see Chap. 20). We insist that all patients
after 3 years of age should be clean of stool in the
underwear, as well as dry of urine.
16.1 Cloaca 261

16.1.3 Gynecologic Concerns

The association of anorectal malformations with

defects of the internal and external genitalia has
been described before [6, 94–96].
The negative implications of the hydrocolpos
as well as the serious consequences of not drain-
ing it have been reported [97–103]. We cannot
overemphasize the importance of suspecting,
diagnosing, and draining a hydrocolpos as early
as possible.
For the first few years of life (until puberty),
the patients usually have no gynecologic prob-
Fig. 16.58 Picture showing the irrigation of fallopian
lems. However, when they reach puberty, one tube to confirm its patency
must be very careful in following these patients.
If the patient develops pubic and axillary hair and
the breasts start to develop but no menstruation,
one must suspect that there is some sort of
obstruction or absence of one or both of the
Müllerian structures. This is particularly true
when the patient has no menstruation and epi-
sodes of abdominal pain with monthly exacerba-
tions that reflect the presence of trapped menstrual
blood. At that point, the patient needs an ultra-
sound of the pelvis and/or an MRI.
From the first 27 patients with cloacas from
our series, who reached puberty, 7 of them
required an emergency laparotomy, due to
trapped menstrual blood in the peritoneum which
formed pseudocysts [104]. After that experience,
our routine now is to check the patency of the
Müllerian structures early in life, whenever we
have the opportunity to be in the abdomen of
these patients. We do this by putting a no. 3 feed-
ing tube through the fimbriae of the Fallopian
tube and injecting a saline solution to confirm
that it comes out through the vagina (Fig. 16.58).
If one of the sides is obstructed and the other is Fig. 16.59 Picture showing a strictured vaginal orifice –
arrow on the vaginal orifice
patent, we prefer to resect the obstructed part,
since the patient will do well with only one
Müllerian structure. When both are obstructed, an examination under anesthesia. The purpose of
we decide to preserve both of them but tell the this is to determine whether or not the introitus is
parents about this and follow the patient closely, large enough for the patient to have normal sexual
particularly when they reach puberty. activity. Sometimes we find a fibrous ring in the
Assuming that the patient starts menstruating area of our anastomosis of the vagina to the neola-
normally in puberty, the next step would be to think bia (Fig. 16.59). If the vagina has not been injured
about sexual function. When the patient is ready to and does not have a long, narrow stricture, usually
develop sexual activity, it is important to perform the repair of this ring is an easy, ambulatory
262 16 Cloaca, Posterior Cloaca and Absent Penis Spectrum

operation that should be done prior to sexual activ-

ity to avoid unpleasant experiences.
Two hundred and fourteen of our cloaca
patients are older than 18 years. From those, 48
patients are all having normal sexual activity; 45
of them claim to have orgasms; another 3 patients
tried sexual activity and have been unsuccessful;
and 7 patients became pregnant and delivered
babies by cesarean section.
The long-term follow-up of all cases suffering
from congenital anomalies is extremely valuable. A
new chapter on the gynecologic problems of patients
born with cloacas is in the process of being inte-
grated. Although, with small numbers, several
important papers have been published concerning
the long-term psychosexual outcome of patients
born with cloacas and urogenital sinus [105–110].
Other unexpected problems may appear later in life
in these patients, including tubal pregnancy [111]
and tubo-ovarian abscess [112]. We have no experi- Fig. 16.60 Diagram of a persistent urogenital sinus. The
ence with tubal pregnancy, but we have seen several patient was born with a cloaca and underwent a repair of
the rectal component of the malformation. The urogenital
cases of adolescents born with cloacas, repaired by sinus was left untouched
us, who suffered from recurrent tubo-ovarian abscess
that eventually resulted in a resection of the tube.
Vaginal septum with two hemiuterus occurred from all over the country and from other coun-
in 2 % of our cases with perineal fistula, 6 % of tries as well. From our experience of 531 cases of
cases with vestibular fistula, and 60 % of our clo- cloacas, 97 of them are actually secondary proce-
acas. It is extremely important to alert the parents dures. This high frequency of complicated cases
of these babies about the future obstetric implica- previously operated also represents the fact that
tions of those anomalies including premature labor the surgical maneuvers described here to repair
and miscarriage [113–115]. Patients born with a cloacas, perhaps, are not highly reproducible.
cloaca must be followed for life by gynecologists Cloacas are not an exception when we affirm that
with special interest and experience in this type of children with congenital malformations “have a
problem. The pregnancies in these patients are by single opportunity” to be repaired, meaning that
definition “high risk.” if that first attempt is not successful, the func-
Finally, malignancy of a cloacal remnant [116] tional prognosis changes negatively after a sec-
has been reported, as well as originating from a ondary procedure.
neovagina [117]. In addition, the occurrence of The most common reoperation that we per-
ulcerative colitis in the neovagina has been reported form in patients with cloacas consists in repair-
[118, 119]. In our series, we have seen two cases of ing persistent urogenital sinuses (Fig. 16.60).
cancer and two of inflammatory bowel disease, at These patients were born with a cloaca and
the colon used to replace the vagina. underwent an operation at another institution
to repair only the rectal component of the mal-
formation. The patients were then left with a
16.1.4 Reoperations persistent urogenital sinus. Interestingly, from
all the persistent urogenital sinuses operated
Secondary procedures in cloacas are very com- by us, only one had come to us with a previ-
mon in our experience. This is perhaps a reflec- ous diagnosis of a cloaca. In other words, the
tion of the nature of our center, receiving patients surgeons were unaware of the fact that the
16.1 Cloaca
Fig. 16.61 Pictures of four patients with persistent urogenital sinus
patient had a cloaca; they tried to repair the
malformation, but they only ended up working
with the rectum and left the urogenital sinus
untouched. On the other hand, all other cases
came to us with the diagnosis of “rectovagi-
nal fistula.” In other words, the surgeons did
not make the diagnosis of a cloaca and oper-
ated on the patient thinking that the patient
had a rectovaginal fistula. The word cloaca
was never mentioned in the medical records
of the patients, and consequently, the surgeons
repaired only the rectal component of the mal-
formation. They did not perform a vaginoscopy
or cystoscopy, pulled the rectum down, and left
the urogenital sinus untouched. Amazingly,
these patients continued their life, recovered
from those operations, and become aware of
their persistent urogenital sinus later in their
lives. We actually had several adult patients
with a persistent urogenital sinus. One of them
had been married for 2 years. She came to us
because of incapacity to perform sex satisfac-
torily. Our examination disclosed a very nar-
row persistent urogenital sinus (Fig. 16.61).
The cases of a persistent urogenital sinus
labeled as “rectovaginal” fistula were more
common at the beginning of our practice,
which reflected (from our point of view) the
263
fact that surgeons were thinking of “rectovagi-
nal” fistulas and not about a cloaca. Looking at
the literature, one can find a very obvious shift
in the apparent frequency of “rectovaginal” fis-
tulas and cloacas. Prior to 1982, the literature
had many reports of “rectovaginal” fistulas and
almost no mention of cloacas. In retrospect, we
know that most of those “rectovaginal” fistu-
las operated and reported in the literature in
the past were actually cloacas or rectovestibu-
lar fistulas. We know that for sure because of
the number of patients that we received with
persistent urogenital sinus and also because in
many of those patients with “rectovaginal” fis-
tulas, when we examined them, we found the
original pocket, or sinus, remnant of the origi-
nal vestibular fistula (Fig. 16.62).
As time went by, the number of persistent
urogenital sinus that we saw decreased, for-
tunately, and we interpreted that as a mani-
festation of the surgeons becoming more
knowledgeable about the existence of this con-
dition. In other words, their index of suspicion
that they were dealing with a cloaca increased
through time.
Now we know that real, congenital, rectovagi-
nal fistulas are extremely rare; we have seen only
7 cases in over 1,000 female patients with
264
Fig. 16.62 Picture of the genitalia of a patient born with
a vestibular fistula who underwent an abdominoperineal
operation under the misdiagnosis of “rectovaginal fistula.”
The original location of the fistula in the vestibule is
clearly seen. F fistula, R rectum
anorectal malformations. In cases of rectovaginal
fistulas, the rectal orifice must be located above
the hymen (Fig. 16.63).
16.1.4.1 Persistent Urogenital Sinus
Figure 16.60 shows a diagram of a persistent uro-
genital sinus. The rectum has been pulled down;
frequently it is mislocated as shown in the figure,
and the urogenital sinus persists untouched. As
the patient reaches adolescence, her first mani-
festation is menstruation “through the urethra.”
Sometimes, the surgeons, during the repair of
the rectal component of the malformation, dam-
age the vagina, producing an acquired vaginal
atresia. In that case, when the patient reaches
puberty, she will accumulate menstrual blood
(hemato-metrocolpos) and suffer from severe
abdominal pain with monthly exacerbations.
Figure 16.61a–c show the external appearance of
the perineum of several patients with persistent
urogenital sinuses.
16 Cloaca, Posterior Cloaca and Absent Penis Spectrum
Fig. 16.63 Picture of a case with a real congenital recto-
vaginal fistula. The rectal orifice is located inside the
vagina (above the hymen)
The diagnosis is a simple one and is done by
inspection. Vaginoscopy and cystoscopy will
allow us to recognize the length of the common
channel and the characteristics of the vagina(s),
which, as previously mentioned, will allow us to
plan on the magnitude of the operation and the
possibilities of requiring a vaginal replacement.
We have been repairing these malformations
without a protective colostomy. However, as
mentioned in Chap. 7 about bowel preparation,
we are very strict in the cleaning of the entire gas-
trointestinal tract with GoLYTELY.
The patient is taken to the operating room
with a central line already inserted and with the
bowel completely clean. Most of the time, the
persistent urogenital sinus can be approached
posterior sagittally first, because of the length of
the common channel. The patient is placed in the
prone position with the pelvis elevated, and the
skin of the genitalia, perianal area, and perineum
is washed, prepped, and draped in the usual
16.1 Cloaca
manor. Multiple 5-0 silk stitches are placed at the
mucocutaneous junction of the anal orifice.
Frequently, this is strictured and/or mislocated.
Applying uniform traction on those multiple 5-0
silk stitches, a posterior sagittal incision is cre-
ated, running from the middle portion of the
sacrum to the anal verge. The incision continues
as a “hockey stick type” of incision around the
anus, using the needle-tip cautery, changing from
cutting to coagulation to provide meticulous
hemostasis. The posterior sagittal incision con-
tinues deeper, going through the skin, subcutane-
ous tissue, parasagittal fibers, muscle complex,
and the levator muscle. Applying uniform trac-
tion to the multiple silk sutures allows us to rec-
ognize the posterior rectal wall easily. The lateral
walls of the rectum are also dissected applying
uniform traction; all this is done with the needle-
tip cautery. Finally, the anterior rectal wall is also
dissected. Depending on the type of procedure
that the patient previously had, one may find real
rectum or colon. It is not uncommon to find that
the previous surgeons resected the original rec-
tum of the patient and pulled colon from inside
the abdomen, down to the perineum. The reason
for doing that is either because they used to per-
form an endorectal type of operation (Soave)
which, by definition, sacrifices the rectosigmoid
and then they pulled the colon from inside the
abdomen (through the seromuscular cuff of the
rectosigmoid left in situ) down to the perineum.
Other times, perhaps more frequently, the sur-
geons open a colostomy too distally, and when
they tried to repair the malformation, they found
that the available length of rectosigmoid from the
mucous fistula to the end of the rectum, where it
connects to the urogenital tract, was too short. We
have learned to preserve that piece of rectum, but
many surgeons simply resect that part of the rec-
tum and take the colostomy down, to save time
and to make the procedure easier.
One can recognize whether or not we are deal-
ing with colon or rectum. In the case of colon,
one can identify the mesentery surrounding the
colon. We must be careful in preserving the mes-
entery to avoid devascularization of the bowel,
because the preservation of the blood supply is
very important, particularly if we decide to use
265
colon for vaginal replacement, when indicated.
Other times, one finds the original rectum char-
acterized by the absence of mesentery. As pre-
viously described, the rectum is surrounded by
fat with vessels, which is the so-called mesorec-
tum that does not have the characteristics of the
mesentery of the colon. Most of the time, the dis-
section of the rectum in a reoperation is simpler
than what we have anticipated. It may become
very difficult in those patients who previously
suffered from catastrophic complications such
as abscesses, dehiscences, or retractions. In such
cases, the amount of fibrosis surrounding the rec-
tum can be extraordinary. When those complica-
tions did not occur in previous operations, the
dissection is straightforward and relatively sim-
ple. We cannot overemphasize the importance
of applying uniform traction while dissecting
delicate structures, particularly in a reoperation.
The dissection of the rectum must continue all
the way up to the supralevator space. Frequently,
we open the peritoneum during this dissection.
Once the rectum has been fully dissected, we
are ready to continue our incision more anteri-
orly. The perineal body is divided with needle-
tip cautery, and the incision goes deeper until we
identify the posterior wall of the vagina. Most of
the patients that we have repaired with persistent
urogenital sinus have a reachable vagina through
the posterior sagittal approach. Once the rectum
has been dissected and moved out of the way, the
posterior vaginal wall is perfectly visible. Two
5-0 silk stitches are placed, taking the posterior
vaginal wall. An incision is created in between
both sutures to open the vagina and to see the
characteristics of the urogenital sinus. The inci-
sion done at the posterior wall of the vagina is
extended distally through the common channel
to expose the entire anatomy. Depending on the
characteristics of the urogenital sinus, mainly the
length of the common channel, we will follow the
strategies already mentioned during the repair of
cloacas. Most of the time, the urogenital sinus is
untouched and therefore, the surgical manipula-
tion of the structure is not more difficult than in
primary cases.
As previously described, if the common chan-
nel is shorter than 3 cm, we will proceed with the
266
total urogenital mobilization exactly the way we
do in a primary case. Most of the times, that
maneuver has been enough to repair these mal-
formations. Dealing with adult patients or teen-
agers, the operation, of course, involves bigger
structures, which represents a technical advan-
tage. The vessels are bigger, and therefore, some-
times the needle-tip cautery used in coagulation
mode is not enough to maintain good hemostasis
and it is necessary to use suture ligatures. The
venous plexus, located behind the pubis, must be
preserved as much as possible. However, some-
times it is impossible; we hit those veins and, in
teenagers or adults, it is necessary to use suture
ligatures because the cautery is not enough to
obtain good hemostasis. The decision-making
algorithm mentioned in the treatment of the uro-
genital sinus of cloacas in primary procedures is
applied in these cases the same as previously
described.
Vaginal replacement with rectum, dividing the
rectum longitudinally as previously described, is
more commonly done in these cases because
these patients have been using the rectum for a
long time and are frequently suffering from con-
stipation, and therefore, they have a dilated rec-
tum. The fact that they have a clear mesentery, if
they have colon in the pelvis, makes the preserva-
tion of the blood supply even easier.
Postoperatively, the patients remain 10 days
with nothing by mouth, receiving parenteral
nutrition. The rectum is reconstructed following
the same principles described in the primary
repair of a cloaca. If the rectum happens to be
mislocated, now is the opportunity to put it in the
right place.
16.1.4.2 Acquired Vaginal Atresia
or Stricture
Another common reason why patients are
referred to us after an attempted failed repair of a
cloaca is because they suffer from an acquired
vaginal atresia or stricture. In these cases, the sur-
geon usually knew that he was dealing with a clo-
aca and actually tried to repair the vaginal
component of the malformation. Unfortunately,
for different reasons, the vagina was not mobi-
lized properly and suffered from ischemia and
16 Cloaca, Posterior Cloaca and Absent Penis Spectrum
acquired atresia or stricture (Fig. 16.64). As a
consequence, the patient comes to us with a pat-
ent urethra and rectum, but without a vaginal ori-
fice. The vagina may or may not have a
communication with the urethra, but the distal
end that the surgeon originally attached and
sutured to the perineum disappeared. The dis-
tance between the blind end of the vagina and the
perineum varies from patient to patient and,
depending on that length, the magnitude and type
of operation changes. In order to repair these
complex cases, it is necessary, again, to mobilize
the rectum. The mobilization of the rectum, in the
way described in the paragraph related to persis-
tent urogenital sinus, is the same in these cases.
We have to mobilize the rectum in order to have
access to the vagina. The exception could be a
case in which the blind vagina is located so high
that we estimate with a CT scan and/or MRI
study that the blind vagina will be easier to reach
through the abdomen rather than from below.
Most cases, however, can be reached from below,
at least to initiate the dissection. The rectum is
dissected and mobilized in the same way as pre-
viously described. If the patient already has
bowel control, it is our experience that a reopera-
tion done correctly does not change the func-
tional prognosis that the patient had from the
fecal continence point of view.
Once the rectum has been mobilized, we find
the posterior vaginal wall, open it, identify the
blind distal end, and use multiple stitches to initi-
ate our dissection. If the patient never underwent
a total urogenital mobilization, we do it as a first
step. On the other hand, sometimes the previous
operation done by another surgeon included an
attempt to separate the vagina from the urethra
and they left the urethra intact. If that is the case,
we can try to mobilize the vagina again. The
decision-making algorithm described in the pri-
mary repair of a cloaca is applied here. In other
words, if the total urogenital mobilization is not
enough to make the vagina reach the perineum,
we have to go into the abdomen to continue with
the extended transabdominal urogenital mobili-
zation. If that is not enough, we have to continue
with the separation of the vagina from the urinary
tract, and if that is not enough, we have to evaluate
16.1 Cloaca
Fig. 16.64 Picture of the perineum of several patients who underwent a previous failed attempted repair of a cloaca
the possibility of a vaginal switch and eventually
a vaginal replacement.
16.1.4.3 Acquired Urethral Atresia
or Stricture
Very occasionally, one may find a patient who
comes after a failed attempted repair of cloaca;
the patient developed an acquired atresia or
severe stricture of the urethra. Depending on the
location of this, we may follow a different strat-
egy. Certainly, one alternative to repair this is the
transpubic approach that will be described in
another chapter. If the urethral stricture is located
low enough, we may try to repair that posterior
sagittally. First, we have to go through the mobi-
lization of the rectum, as previously described,
then the vagina, and eventually the urethra.
Sometimes, the acquired atresia of the urethra is
so severe and the length of separation of both
267
healthy portions of the urethra is so great that the
decision is made not to repair the urethra but
actually to perform a Mitrofanoff type of proce-
dure, creating a conduit for the patient to empty
her bladder with intermittent catheterization
through an orifice created in the abdomen.
16.1.4.4 Sequelae from Catastrophic
Complications
Some patients underwent an attempted repair and
almost everything went wrong; the patients had
dehiscence, infection, retraction, and fistula for-
mation. The operative reports are frequently con-
fusing and only illustrate the disorientation
suffering and anxiety of the surgeon exploring
the pelvis of a patient with an anatomy that seems
to be completely unknown to him or her. As a
consequence, we do not know exactly what hap-
pened, but we may see, for instance, that the
268
patient has a blind perineum. In other words,
there is no urethra, no vagina, and no rectum. Or,
they may have one or two orifices. A study with
contrast material injected through the different
orifices of the patient (colostomy, cystostomy,
vaginostomy, vesicostomy, or rectum) helps us to
make the anatomic diagnosis. More recently, at
our institution, the Interventional Radiology
Department has helped, performing a study
called three-dimensional, rotational scan, inject-
ing contrast material simultaneously, through the
stomas of orifices of the patient’s perineum.
Videos 16.1, 16.2 and 16.3 illustrates the beauti-
ful images provided by this kind of study. We
often use this modality in primary complex clo-
aca cases as well. These help us to understand the
sequelae in these patients. It is not unusual for us
to find that the patient has what we call a “frozen”
or “cement type of pelvis.” These patients are
extremely difficult to approach and based on the
diagnostic tests, already described, the surgeon
has to make a decision as to how to approach this
kind of patient. The first possibility would be to
repair these posterior sagittally. However, some-
times posterior sagittally, the operative findings
are very frustrating, and it is very difficult to
mobilize the structures. The next possibility is an
abdominal midline incision. Yet, through the
abdomen, sometimes it is also very difficult to
find the plane of dissection in the pelvis. At that
point, the last alternative and more aggressive
approach that provides the best exposure is the
transpubic route.
16.1.5 Transpubic Approach
The transpubic approach is part of our armamen-
tarium to approach complex pelvic problems
[120]. If the surgeon considers that the lesion, fis-
tula, mass, or structure that he or she wants to
reach is surrounded by excessive fibrosis and is
located in a place impossible to reach from below
or from above, then another alternative is to con-
sider the transpubic approach. This type of
approach provides excellent exposure. It is par-
ticularly indicated in a patient who has a func-
tional rectum but a significant number of problems
16 Cloaca, Posterior Cloaca and Absent Penis Spectrum
in the urogenital tract, in other words, urethrovag-
inal and bladder vaginal fistula (Fig. 16.65a–c).
A midline incision running from the umbili-
cus down toward the genitalia is performed, all
the way down to the urethra. The clitoris is
divided exactly in the midline with needle-tip
cautery. The pubis is exposed and dissected, in
order to identify what is bone and what is carti-
lage, to be sure that the cartilage is divided
exactly in the midline (Fig. 16.65). The needle-
tip cautery divides the pubic cartilage very eas-
ily. We must keep in mind that behind the pubis
are venous plexuses that may bleed significantly,
particularly in teenage or adult patients. In those
cases, we should use sutures to stop the bleed-
ing. Once we divide the pubic cartilage, the
pubic bones spontaneously separate, giving us
usually enough space to work. If that is not the
case, we can use a retractor to separate the pubic
bones more. With this approach, we achieve a
great exposure to the urogenital tract
(Fig. 16.65b); we can work comfortably to deal
with the specific complications that the patient
has. We have operated on 20 patients with this
transpubic approach.
At the end of the procedure, the pubic carti-
lage is reapproximated using 0, 00, or 1 Vicryl
sutures. We keep the patient in bed for a week
before we allow her to walk making sure to avoid
external rotation of the hips. The main inconve-
nience of the transpubic approach is the fact that
it produces significant pain postoperatively. The
patients usually refuse to walk for a week or 2
because of the pain. One patient suffered from an
acute dehiscence of the pubic cartilage that had to
be resutured on an emergency basis. Surprisingly,
this occurred in a little baby and not in an ambu-
latory patient. Another patient suffered from high
fever; a bone scan suggested that the patient was
suffering from an infection in that area. The
patient received antibiotics for 6 weeks to treat
osteomyelitis and healed normally. Other than
that, all the patients have done very well. We do
not recommend the transpubic approach except
under the circumstances already mentioned.
The functional results in reoperations for clo-
acas vary from case to case and depend very
much on the type of complication and the origi-
16.1 Cloaca 269

a b

Fig. 16.65 Transpubic approach. (a) Dividing the pubis. (b) Absent bladder neck. Wide communication between
vagina and bladder. B bladder, V vagina. (c) Ectopic ureters opening below the bladder neck

nal complexity of the malformation. In a patient
with a normal sacrum, common channel shorter
than 3 cm, and an intact persistent urogenital
sinus, we can expect an excellent result, includ-
ing normal urinary control. Of course, when the
previous surgeon was too invasive, performed a
lot of dissection, damaged important nerves and
structures, and produced more fibrosis, the func-
tional prognosis cannot be predicted. This has to
be informed to the parents.
As part of the long-term follow-up, we explain
to the parents of our patients that they must pay
special attention when their daughter shows signs
of puberty, has no menstruation, and complains
of abdominal pain with monthly exacerbations;
when these are observed, they must contact us. In
such case, we order a pelvic ultrasound and an
MRI to rule out the presence of menstrual blood
in the peritoneal cavity. To alleviate the symp-
toms, sometimes it is beneficial to administer
270
medication to suppress the menstruation, which
gives us time to plan the surgical treatment.
The next time to be concern is when the patient
is expected to have sexual intercourse. We
encourage them to have an examination under
anesthesia. We want to be sure that the vagina has
the adequate characteristics (size and elasticity)
to avoid unpleasant experiences.
Sometimes, we find a narrow external orifice
with a large, elastic vagina. That problem is
solved with an external vaginoplasty (see
Fig. 16.38). Other times, we find a more serious
problem, a patent vagina, but very inelastic due
to scarring. For that problem, we designed an
operation consisting in patching the posterior
wall of the vagina with the most distal piece of
rectum. Figure 16.66a–g shows the surgical
technique that we use to patch the vagina with
rectum.
This procedure is only recommended when
the patient has no bowel control. We must keep in
mind that the resection of the distal rectum in a
patient with bowel control may provoke fecal
incontinence in a patient that had bowel control.
If the patient is fecally incontinent, the removal
of the most distal part of the rectum has no func-
a b
Fig. 16.66 Surgical technique to patch a narrow vagina
with rectum. (a) Diagram showing the creation of a rectal
patch, preserving the blood supply. (b) Operative view.
The rectum has been mobilized and is pulled up. A Hegar
dilator is introduced in the vagina. R rectum, V vagina. (c)
Operative view. The rectum has been divided, preserving
the blood supply of the distal segment. D distal, P proximal.
16 Cloaca, Posterior Cloaca and Absent Penis Spectrum
tional repercussions. We have done this operation
in 11 of our patients.
16.2 Posterior Cloaca and Absent
Penis Spectrum
“The case report of M. Louis from Paris, has
become famous. In his thesis in 1753, he
described a girl with an anus cloacal who men-
struated per anus. Happily married to an impetu-
ous young man, she finally confided her secret to
him. At the height of passion, the latter persuaded
his wife to engage in coitus. She agreed to it and
became pregnant. The birth of the infant occurred
at term and caused a tear of the anal sphincter.”
“The presentation of this thesis before the sur-
geons, led to legal prosecution of M. Louis in
Paris. During the legal proceedings, it was decided
that moral theologians should resolve the issue of
whether the conduct of this woman and that of
M. Louis was illegal. At last, the Pope himself was
called upon to decide. He, however, more far-
sighted than the parliament of Paris and the physi-
cians of the Sorbonne, granted M. Louis absolution,
and the thesis was finally published in 1754.”
c
(d) The distal segment is open to tailor the patch. P patch.
(e) The rectal patch is being sutured to the narrow vagina.
The cervix is clearly seen in the vagina. V vagina, P patch.
(f) Diagram showing the finished operation. (g) Diagram
showing a technical modification to increase also the
length of the vagina
16.2 Posterior Cloaca and Absent Penis Spectrum
d e
f g
Fig. 16.66 (continued)
These two paragraphs were taken from Scharli
[121], from a publication entitled “Malformations
of the Anus and Rectum and Their Treatment in
Medical History,” published in Progress in Pediatric
Surgery, volume 11, 1978. Dr. Scharli took this
information from a publication of M. Louis himself
[122] who was also quoted by Bushe [123].
Two-hundred and thirty-two years later, in
1986, the senior author of this book was invited
271
by Dr. Fred Leditschke to Brisbane, Australia, to
operate on a little girl who was born with “an
unusual anorectal and urinary malformation”
[124]. That particular girl had a very unusual
history. She was born and considered normal;
therefore, she was discharged and went home.
The mother noticed that she was passing many,
very frequent, liquid bowel movements and went
through a series of consultations with different
272 16 Cloaca, Posterior Cloaca and Absent Penis Spectrum

pediatric specialties who could not find the rea-

son why the girl suffered from this “chronic diar-
rhea.” Subsequently, the patient was referred to
Dr. Fred Leditschke (pediatric surgeon) who per-
formed a more thorough examination and also an
endoscopy; by doing that, he was able to find that
the patient had no vaginal opening and no urethral
opening (Figs. 16.67 and 16.68). She was passing
stool and urine through a single orifice located in
the same location of a normal anus! In addition,
the endoscopy disclosed the presence of an ori-
fice in the anterior rectal wall, and introducing
the scope through that orifice, he was able to find
a urethra and vagina; in other words, it was a
urogenital sinus, posteriorly deviated and con-
nected to the anterior rectal wall (Fig. 16.69).
Interestingly enough, the little girl went through
18 months of her life suffering from this “pseudo-
diarrhea” and nobody had seen directly the exter-
nal genitalia. This is very important because

Fig. 16.68 External appearance of a case with a poste-

rior cloaca, separating the labia majora

Fig. 16.67 External genitalia of a patient with a posterior

cloaca Fig. 16.69 Diagram of a posterior cloaca
16.2 Posterior Cloaca and Absent Penis Spectrum
many of these patients, when examined exter-
nally, they look normal; it takes a special interest
to separate the labia of the genitalia in order to
see the anomaly (Figs. 16.67 and 16.68).
The senior author, together with Dr. Leditschke,
repaired that very unusual malformation success-
fully. We are happy to say that that little girl is
now a beautiful young lady, happily married, and
also delivered a baby by cesarean section.
Based on the external and internal findings of
this particular malformation, we decided to call this
“posterior cloaca.” These patients have a single per-
ineal orifice, but what makes this defect unique is
the fact that the single orifice is located in the same
location as a normal anus. A typical cloaca has a
single orifice located in the same location of a nor-
mal urethra. As a consequence of the repair of this
malformation, we suspected that perhaps there
would be other similar cases; in retrospect, we
found that there were more cases than what we orig-
inally thought, except that they had not been named
and had been included in the category of cloaca.
As a consequence of the repair of that initial
defect, two ideas came into our minds. Number
one is the idea of dividing the entire rectum, what
we now call “trans-anorectal approach.” In other
words, the fact that the patient has a normal anus
with normal pectinate line and therefore normal
anal canal makes that patient fecally continent by
definition. The surgical technical implication of
this is that we should not mobilize the anus and
the rectum. We can divide the entire rectum in the
midline, including both posterior and anterior
walls, repair the urogenital sinus anteriorly with
an excellent exposure, and reconstruct the rec-
tum. We had evidence that this does not harm
fecal control (see Chap. 26). The second idea that
came to our minds as a consequence of dealing
with this malformation is the “total urogenital
mobilization.” In order to move the urogenital
sinus from its posterior mislocation, seen in pos-
terior cloacas, it is necessary to mobilize it and
switch it forward to be able to place the urethra in
a normal location and the vagina behind the ure-
thra. That maneuver has demonstrated to be
extremely useful in the repair of cloacas with a
common channel shorter than 3 cm and also in
273
urogenital sinus cases with normal rectum (see
Sect. 16.1 and Chap. 26).
Very soon, in the management of these cases,
we realized that there was another very conspicu-
ous anomaly present in these cases, and that was
the fact that the pubis is extremely thick. In other
words, the urogenital sinus is posteriorly devi-
ated, and we do not know if that is because of the
presence of an extremely prominent pubis or is
just coincidental. The fact is that it is necessary to
carve the pubis, removing part of it, to be able to
create the necessary space to move the urogenital
sinus forward and create urethral and vaginal ori-
fices in a normal location.
As we gained experience in the management in
these cases, we found another variant of these mal-
formations. We were able to see girls that are born
with the anus normally located or slightly anteri-
orly mislocated and a second orifice (urogenital
sinus) located immediately anterior to the anus
(Fig. 16.70). The fact that they have two perineal
orifices would prevent us to call that a “cloaca”;
yet, the urogenital sinus is posteriorly deviated and
mislocated; therefore, we believe that we should
consider that malformation as a part of the spec-
trum of posterior cloaca [125]. In addition, we
found cases that had a posterior cloaca, but in
addition, they have an accessory micro-urethra
that runs from the bladder toward the tip of a pseu-
dophallus or clitoris (Fig. 16.71). We also include
that in the spectrum of posterior cloaca because of
the common denominator that is a normally
located anus or slightly anteriorly mislocated with
a posterior location of the urogenital sinus.
We could not find in the literature a report
using the term posterior cloaca. However, look-
ing at the very few publications on the treatment
of cloaca, prior to 1982, we found that some-
times the authors of those papers referred to clo-
aca patients using the term “urogenital sinus and
imperforate anus.” Looking at the specific pic-
tures that the authors presented in the publica-
tions, we were able to see that some of those
cases were actually posterior cloacas, but that
term was not mentioned and the specific poste-
rior deviation of the urogenital sinus was not
described. We found one specific paper in 1981
274 16 Cloaca, Posterior Cloaca and Absent Penis Spectrum

a b

Fig. 16.70 Posterior cloaca variant. The urogenital sinus is located immediately anterior to the anus, but far away from
the clitoris. (a) Diagram of sagittal aspect, (b) external appearance of the perineum, (c) demonstration of large pubis

by John Duckett and Ballinger. They entitled
their paper “Accessory Phallic Urethra in the
Female Patient,” and actually they described
three cases that are posterior cloacas according
to the pictures that we saw in the paper [126]. In
1994, Chatterjee published a case described as
clitoromegaly, duplex urethra, and dysplastic
vagina, but the photograph of the patient shows
the 2-orifice variant of the posterior cloaca spec-
trum [127].
More recently, the term posterior cloaca seemed
to be becoming more popular and we were able to
16.2 Posterior Cloaca and Absent Penis Spectrum 275

a b

Fig. 16.71 Diagrams showing a sagittal view of three different variants of posterior cloaca. (a) With hydrocolpos,
(b) with double vagina and (c) with an accessory quasi-atretic urethra opening at the tip of a pseudophallus (clitoris)

find publications referring to this specific defect as megaureters and hydronephrosis. That should
using the term posterior cloaca [128–131]. be treated at birth.

16.2.1.1 Typical Cloaca with a Single

16.2.1 Surgical Repair
Most of these patients came to us with a colos-
tomy performed at another institution during the
newborn period. Patients born with a posterior
cloaca also may have hydrocolpos with all of the
negative consequences related to this defect such
Perineal Orifice in the Anal
Location
The patient is placed in the prone position with
the pelvis elevated. A posterior sagittal trans-
anorectal incision is performed. As described in
Chap. 26, the incision includes the skin, subcuta-
neous tissue, parasagittal fibers, muscle com-
276
plex, posterior rectal wall, anterior rectal wall,
and anterior sphincter mechanism. With the use
of self-retractors, we have beautiful exposure
(Fig. 16.72). The urogenital sinus that is
connected to the anterior rectal wall, usually
about 1 or 2 cm from the anal verge, can be seen
as soon as we open the posterior rectal wall.
Multiple 5-0 silk sutures are placed in the cir-
cumference of the single orifice of the urogenital
sinus, in order to be able to apply uniform trac-
tion to facilitate its dissection. The urogenital
sinus is separated from the anterior rectal wall.
The perineum anterior to the anus is also divided
in the midline all the way up to the clitoris. At
this point, we can evaluate and feel if the pubis is
interfering and does not allow us to find space to
place the urethra immediately behind the clitoris
and the vagina behind. If that is the case, in
babies, we can resect more than 50 % of that
very heavy thick cartilage of pubis with needle-
tip cautery, with minimal bleeding. By doing
that, we create enough space to switch forward
and mobilize the urethra and vagina together
(total urogenital mobilization). As previously
described in the Sect. 16.1, if the common
channel is shorter than 3 cm, the overwhelming
majority of cases can be repaired without open-
ing the abdomen, placing the urethra immedi-
ately behind the clitoris, with the vagina behind,
without separating the vagina from the urethra.
Figure 16.72 shows pictures and diagrams of this
procedure.
The urethral opening is sutured with 6-0 long-
term absorbable sutures to the tissue immediately
behind the clitoris as described in the repair of
cloacas. The vaginal walls are sutured with inter-
rupted 5-0 long-term absorbable sutures, to the
skin of what is going to be the neolabia. The
divided rectum is already perfectly visible
(Fig. 16.72). A meticulous reconstruction is per-
formed, bringing together corresponding por-
tions of the sphincter mechanism anterior to the
anus. The anterior rectal wall and the anal wall
are sutured with two layers of interrupted long-
term absorbable sutures. The posterior rectal wall
is reconstructed in the same manner followed by
a meticulous reconstruction of a normal sphincter
mechanism located behind the rectum and anus.
16 Cloaca, Posterior Cloaca and Absent Penis Spectrum
16.2.2 Surgical Repair
of the 2-Perineal-Orifice
Variant of the Posterior
Cloacal Spectrum
When patients are born with two orifices, one is
the anal opening located in the normal location or
slightly anteriorly and the second one located
anterior to the anus, but far away from the clitoris,
it represents the urogenital sinus (Fig. 16.70a, b).
In that type of malformation, provided the com-
mon channel is not too long, it is conceivable, as
many authors like to say, that we could repair the
urogenital sinus without necessarily dividing the
anorectum. Of course, we agree that if that is fea-
sible, it should be done that way, but also we
believe that we should not compromise the quality
of the repair trying to avoid the opening of the
healthy rectal walls (See Chap. 26, Fig. 26.13a, b).
The urogenital sinus is mobilized as described in
the case of cloacas. When the pubic bone and car-
tilage represent an obstacle, it should be resected,
part of it, to create the necessary space, and the
reconstruction is done following the same steps
already described.
Our experience with the repair of posterior
cloaca and its variants includes 30 patients [132].
The results in terms of urinary function, bowel
control, and sexual concerns are very similar to
those found in the repair of cloacas and depend,
like in cloacas, on the length of the common
channel that influences mainly the urinary func-
tion and the presence, absence, or abnormalities
of the sacrum.
16.2.3 Posterior Cloaca
and Absent Penis
The reader must be surprised that we are discuss-
ing here a malformation called absent penis in a
chapter dedicated to female patients with poste-
rior cloacal malformations. The reason for this is
that we found interesting striking similarities
between both malformations: posterior cloaca in
females and absent penis in males.
Absent penis through history is a very well-
known malformation. We believe that the defect
16.2 Posterior Cloaca and Absent Penis Spectrum 277

a b

c d

Fig. 16.72 Operative pictures of the posterior approach opened. “V” is vagina and “U” is urethra. (c) The urethral
of a posterior cloaca. (a) The rectum has been divided in opening is sutured a few millimeters behind the clitoris.
the midline, and the urogenital sinus is already mobilized. (d) The lateral walls of the vagina are sutured to the
(b) The posterior wall of the urogenital sinus has been neolabia
278
has been recognized for many years, due to the
fact that it is a very obvious, conspicuous defect. It
is not the purpose of this presentation to review the
historical information related with this defect; we
found references even from the last two centuries.
We included in this review a few references
that we consider important related with this
defect [133–138]. These babies are chromosom-
ally males, have testicles, and are born without a
phallus. The posterior urethra is posteriorly devi-
ated and opens into the anterior rectal wall or
immediately anterior to the anus (Fig. 16.73).
Looking carefully to this diagram, one can see
the remarkable similarity with the anatomy of a
posterior cloaca in female patients.
Historically, and occasionally even now, these
babies are raised as females. In other words, they
are subjected to bilateral orchiectomies, the ure-
thra is mobilized to be placed similar to a female
patient, and a neovagina is created [139–150].
There is another very interesting malforma-
tion, seen in male patients, that is known with
different names such as “urethral atresia with ure-
throrectal communication,” “duplication of the
urethra,” “dystopic urethral orifice into the anus,”
and “Y-duplication of the male urethra.” All these
names refer to a malformation very similar to the
absent penis, except that the penis is present. In
other words, the posterior urethra is posteriorly
deviated and opens into the anterior rectal wall or
Fig. 16.73 Diagram showing a sagittal view of an absent
penis. The posterior urethra is connected to the anterior
rectal wall
16 Cloaca, Posterior Cloaca and Absent Penis Spectrum
immediately anterior to it; the patient has a very
hypotrophic, usually useless, tiny penile urethra
that opens in the tip of the penis, and that is the
reason why a lot of people call this “double ure-
thra” or “Y urethra” (Fig. 16.74). Again, one can
see the striking similarity between this defect and
absent penis and also with the posterior cloaca in
females [151–154].
We have no experience with the treatment of
absent penis. However, we have experience in the
treatment of the variation that we just described,
namely, the posterior urethra deviated to the ante-
rior rectal wall and a quasi-atretic penile urethra
(Fig. 16.74). This malformation is repaired by
stages. The first procedure is done with the
patient in prone position, mobilizing the urethra
from the anterior rectal wall or from the very
anterior perineum, forward, to put it in a more
convenient perineal location away from the anus
(Fig. 16.75a). This operation can be done divid-
ing the anterior rectal wall and the posterior rec-
tal wall or in more simple cases without touching
the rectum. After the operation, basically, the
Fig. 16.74 Diagram showing a sagittal view of a malfor-
mation considered a variant of the absent penis. The pos-
terior urethra is connected to the anterior rectal wall and
the penile urethra is very structured
References
a b
Fig. 16.75 Diagrams showing the repair of the “absent
penis variant.” (a) The posterior urethra has been discon-
nected from the anterior rectal wall and switched forward
patient would have the equivalent to a severe per-
ineal hypospadias. The penile urethra is usually
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penile urethra is reconstructed; the posterior ure-
thra connected to the perineum is switched for-
ward and is connected to the reconstructed penile
urethra (Fig. 16.75b). This part of the procedure
(urethral reconstruction) must be done by skilled,
dedicated pediatric urologists.
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s00383-006-1676-x
 Cloacal Exstrophy and Covered
Cloacal Exstrophy
Cloacal exstrophy is the most complex, severe,
and devastating congenital defect that affects the
gastrointestinal tract, the genitourinary tract, the
spine and cord, and therefore also potentially the
motion of the lower extremities.
From our literature review, we found that prior
to 1960 all patients born with this constellation of
defects died [1–3]. Peter Rickham in 1960 pub-
lished a report of four cases, with one survivor
[4]. After Rickham, for several years we found
multiple isolated reports, small series with over-
whelming mortality [5–18].
Until 1964, there had been 52 cases reported
in the literature [5]. Until 1991, there were 190
cases reported [6]. The early reports estimated
that it affected 1 in 200,000–400,000 pregnancies
[5, 19]. More recent reports indicate that it seems
to be more common than previously thought
[20], most likely affecting 1:100,000–1:50,000
pregnancies.
The list of associated defects is very extensive
[21–26] and includes diverse gastrointestinal,
genital, vertebral, and urogenital malformations,
tethered cord, and other forms of dysraphism and
myelomeningocele and intracranial defects.
Regardless of the efficiency of the available
treatment modalities and technical advances of
the major medical institutions in the world, the
final quality of life of these unfortunate patients
is still very poor.
Until recently, the quality of life of the patients
who survived provoked controversies and serious
ethical and unanswered questions [27, 28].
A. Peña, A. Bischoff, Surgical Treatment of Colorectal Problems in Children,
DOI 10.1007/978-3-319-14989-9_17, © Springer International Publishing Switzerland 2015
17
Fortunately, important advances in prenatal
diagnoses allow us to detect this defect earlier
and earlier in utero, which gives the parents
options, in terms of continuation or interruption
of pregnancies [28–30].
In more recent years, we saw emerging presti-
gious centers, with special interest in urogenital
malformations. Those centers and their distin-
guished surgical leaders were able to collect
larger series of cases, from which we have learned
[31–43]. The contributions of Gearhart et al. [35,
41, 43] have been particularly important.
There is no question in our minds that com-
plex congenital malformation, particularly those
affecting different areas of the human body, must
be treated in specialized centers, with experts
subspecialized in the specific problem. We pre-
dict that we will be seeing more and more sub-
specialized medical centers that will benefit
many children.
Cloacal exstrophy affects different anatomic
territories that must be discussed and treated
separately. From a urinary point of view, these
patients have a bladder completely open
(extrophic) (Fig. 17.1). What is different about
these malformations, when compared to the clas-
sic bladder exstrophy, is the fact that these
patients actually have two extrophic hemiblad-
ders, as can be seen in Fig. 17.1. In between the
hemibladders, there is a piece of bowel that can
be a colon or small bowel, which can be also pro-
lapsed, creating an appearance that has been
called “elephant trunk.” These patients may have,
285
286 17 Cloacal Exstrophy and Covered Cloacal Exstrophy

a b

Fig. 17.1 Cloacal exstrophy. Three examples of the external appearance at birth. (a, b) External appearance.
H hemibladders, O omphalocele, B bowel. (c) Cloacal exstrophy. Observe the defective lower extremities

in addition, other urinary problems, such as
absent kidney, hydronephrosis, or different kinds
of obstruction in the urinary tract.
The abdominal wall in these patients is defec-
tive, since they have an omphalocele that could
be minor or very serious. The pelvis is widely
open. The pubic bones are completely separated.
The degree of separation and severity of the pel-
vic malformation is much greater than in classic
bladder exstrophies, and therefore, the idea of
bringing together the pubic bone as early as pos-
sible that is frequently done in bladder exstro-
phies is not always possible in cases of cloacal
exstrophy (Fig. 17.2).
The bowel is also severely affected. These
patients are born with no anus. They also have
colonic malformations represented by a spectrum
that goes from patients that have normal colonic
length to patients that have basically absent colon
or patients who have a bizarre-looking pouch, as
the only representative of the colonic tissue that
they have. The pouch may have different sizes and
very bizarre and abnormal blood supply (Fig. 17.3).
Sometimes the patients have only two little pieces
of cecum, with two appendices and a prolapsed
ileum. To recognize and identify the length of
colon that these patients have is extremely impor-
tant, because, as we will be discussing later, the
possibility of a colonic pull-through will depend
very much on the total length of colon that they
have. It has been our experience that even when
we see sometimes that these patients have a very
short piece of colon, the surgeon should not under-
estimate the potential for growth that those pieces
of colon have, and therefore, every single piece of
bowel should be preserved.
In spite of the fact that these patients would
have a less than optimal quality of life in the future,
17 Cloacal Exstrophy and Covered Cloacal Exstrophy
Fig. 17.2 X-ray film showing the wide separation of the
pubic bones in a patient with cloacal exstrophy
Fig. 17.3 Intraoperative appearance of the bizarre
colonic anatomy, frequently seen in cases of cloacal
exstrophy at birth. CO colon, V ileocecal valve, I ileum,
B blind end, Ce Cecum
it has been our impression that most of them grow
up to become extremely charismatic and intelli-
gent little children. We have been following these
patients for many years and have been deeply
impressed by their personality and their charisma
as well as what they achieve through life.
Female patients are born with two completely
separated hemivaginas that may have external
287
orifices located immediately below the hemiblad-
ders (Fig. 17.4). Each one of those hemivaginas is
directed in opposite directions toward the lateral
part of the pelvis. Sometimes, the patients have
atresias of the Müllerian structures. They have
two widely separated hemiuteri. Usually they
have normal ovaries. The two vaginal orifices
sometimes are located together at the midline, and
sometimes they are widely separated and are
located at a very short distance from the location
of the ureteral orifices (Fig. 17.4a). Male patients
are born with two hemiphalli that are located each
one on top of the completely separated pubic
prominences (Fig. 17.4b).
Our series includes 32 cases. This number is
obviously not representative of the frequency in
the general population, since we work at a refer-
ral care center for these kinds of defects. Cloacal
exstrophies are another wide spectrum of defects
that go from, what we call, covered cloacal
exstrophy to a full cloacal exstrophy like those
shown in Figs. 17.1 and 17.2.
Fourteen of our cases were classic cloacal
exstrophies, 15 were covered cloacal exstrophies,
and three were variants of cloacal exstrophies.
From the orthopedic point of view, these
patients represent a challenge, because of the
wide separation of the pubic bones, more severe
than in the bladder exstrophies. In addition, they
frequently have spinal problems and luxation of
the hips that represent a real challenge for the
orthopedic surgeons. It is not unusual to see that
these patients have myelomeningocele or severe
sacral defects that affect the prognosis of the
patient, particularly for the motion of the lower
extremities.
An interesting historical fact is related with
the designation of gender in male patients born
with cloacal exstrophy. For many years, the
pediatric surgical as well as the pediatric uro-
logic community considered that it was basi-
cally impossible to reconstruct a functional
phallus, and therefore, the general agreement
was to perform a bilateral orchiectomy, as well
as sometimes partial or total resection of the
hemiphallus of these patients, and raise them as
females, in spite of the fact that they were chro-
mosomally males. Later in life, a vagina was
288
a b
Fig. 17.4 Genitalia in patients with cloacal exstrophy. (a) Female. (b) Males
created with the bowel; the patients received a
female name, were educated as females, and
were expected to have reasonable sexual func-
tion as females [31–39, 41].
The long-term follow-up of these patients
demonstrated that kind of management was less
than optimal. Many of these patients, when they
learned that they were actually chromosomally
males, became extremely upset because some-
body made a critical decision on their behalf,
ignoring their own desires. They considered that
being a male is much more than just having a
phallus to perform sexually, because in addition,
if they had gonads, actually they could fertilize
and have children, and they had all the other char-
acteristics of a male individual. Also, the long-
term observation of the behavior of these patients
(chromosomally males raised as females) fre-
quently showed that even when they received a
female name and were raised and educated as
females, they behaved very much like male indi-
viduals. Because of this, our attitude toward these
kinds of problems radically changed [44–47].
Nowadays, males are raised as males and females
17 Cloacal Exstrophy and Covered Cloacal Exstrophy
as females. Ambitious urologists are trying to
reconstruct the phallus in these patients, to try to
make them sexually active in an efficient way.
Therefore, the orchiectomies are no longer per-
formed on male patients.
Many patients are born in institutions where
different specialty surgical departments (orthope-
dics, general pediatric surgery, pediatric urology,
gynecology) work without a unified, specific plan
for the management of these patients. We con-
sider that this is less than desirable. We have cre-
ated, what we call, a “unified approach” [42, 48].
It is important for all the participants in the man-
agement of these patients to previously discuss
and create a common philosophy and protocol of
management for the benefit of these patients. An
example of the lack of coordination and second-
ary effects that this may have on these patients is
a baby that is born with cloacal exstrophy in a
hospital where the first contact with the patient is
a pediatric urologist, who decides independently
to use intestinal tissue to reconstruct the urinary
tract. By doing that, sometimes the patient is con-
demned to a permanent stoma that could have
17.1 Neonatal Approach
been avoided if more active pediatric surgeons
had been present from day one, in the manage-
ment of these patients. Since the possibility of
pull-through or not pull-through in these patients
depends very much on the length of bowel that
they have and therefore the capacity to form solid
stool, it is imperative and extremely important to
preserve every single piece of gastrointestinal
tract, as part of the gastrointestinal tract, since we
have evidence that the bowel grows with time,
and even if it looks insignificant in length at the
beginning, it may grow more than expected and
become crucial, for the patient to be a candidate
for colonic pull-through and a successful bowel
management in the future.
Another example of the negative conse-
quences of a lack of collaboration could be the
reverse, namely, the baby that is seen and treated
first by a pediatric surgeon who focuses on the
gastrointestinal issues, without paying attention
to the extremely important urologic concerns of
the patient.
17.1 Neonatal Approach
When the pediatric surgeon is called to see a
newborn baby with a cloacal exstrophy, the
patient is frequently taken to the operating room,
and the pediatric surgeon would be in charge of
the closure of the omphalocele and the diversion
of the fecal stream.
Some surgeons are very much in favor of try-
ing to approximate the pubic bones as early as
possible in life. We agree with the idea; however,
the approximation of the pubic bones is more fea-
sible in patients with bladder exstrophy, but not
as easy in patients with cloacal exstrophy, in
whom the separation of the pubic bones is more
severe. In our particular institution, the orthope-
dic surgeons participate in trying to approximate
the pubis, but usually they do not do it in the first
few days of life. Therefore, more often the sur-
geons are called to deal with the omphalocele and
the bladder without approximation of the pelvis.
This means that the omphalocele can usually be
closed, but sometimes it is so large that we can
only afford to close it partially (the upper part and
289
not the lowest part of the defect). The bladder is
managed by the urology team, and their role con-
sists in trying to close the bladder, to bring
together the two hemibladders trying to protect
the bladder mucosa, but not with the specific goal
of making this patient urinary continent from the
beginning. Both the urologist and pediatric sur-
geon must agree about the main goal which is to
separate the gastrointestinal tract from the uro-
thelium, bring together the hemibladders, and
close the bladder anteriorly.
The role of the pediatric surgeon is crucial, to
be sure that no gastrointestinal tissue is left
attached to the urinary tract. The most common
error that we have observed, in the neonatal man-
agement of these patients, from the pediatric sur-
gical point of view, is for the pediatric surgeon to
open a proximal ileostomy and leave the distal
bowel (hindgut) attached to the urinary tract.
Some pediatric urologists may consider this
advantageous, because that creates a reservoir
that they plan to use for a future bladder augmen-
tation. However, that bowel absorbs urine, and
the babies develop hyperchloremic acidosis that
interferes with their growth and development
[49]. In addition, the bowel left defunctionalized,
attached to the urinary tract, does not grow, as
when the bowel is connected to the fecal stream.
Every effort should be made by the pediatric sur-
geon to disconnect every single piece of gastroin-
testinal tract. Sometimes the patients have two
ceca, and those should be placed in continuity,
one to the other, in order to try to create a real end
colostomy, with no mucous fistula. When the
vaginas are opening near one to the other during
the same procedure, we try to create a single vag-
inal orifice by bringing together both openings,
but no attempt is made to bring together the entire
length of both long hemivaginas.
Many babies born with cloacal exstrophies are
referred to us suffering from severe hyperchlore-
mic acidosis and hyponatremia after they under-
went the opening of an ileostomy [49]. For them,
we designed a procedure that we call “rescue oper-
ation,” (Fig. 17.5a, b) consisting in opening the
abdomen, closing the ileostomy, separating the
gastrointestinal tissue from the urinary tract, rein-
corporating it into the fecal stream, and opening an
290
a b
Fig. 17.5 Rescue operations. (a) Diagram showing an
ileostomy and the hindgut have left attached to the urinary
tract. (b) Diagram showing the anatomy after the opera-
end colostomy in the most convenient part of the
abdomen, being sure that the bowel opens in an
area where it is surrounded by normal skin at 360°.
Sometimes, as previously mentioned, the patients
are born with two separate portions of colon that
look rather insignificant. We must look carefully
into the blood supply of these portions of the
colon, try to identify which part is proximal and
which part is distal and to incorporate them into
the fecal stream and again, open an end colostomy.
Figure 17.5 shows an example of a rescue opera-
tion. We have done twelve of these operations in a
patient that received an ileostomy at another insti-
tution. The hyperchloremic acidosis improved in
24 h, and the patients eat, grow, and develop very
soon after this procedure.
Some patients, as previously mentioned, only
have a pouch type of colon, which is almost a cys-
tic, very dilated piece of colon, with a very abnor-
mal blood supply (Fig. 17.6). In such cases, we
have to observe carefully the blood supply, to be
sure that we do not produce ischemia, because
every pouch has a different, rather bizarre, unpre-
dictable blood supply. There is always a tempta-
tion to resect this pouch, assuming that it will not
work, due to a very poor peristalsis and very
abnormal anatomy. Yet, we emphasize the impor-
tance of preserving every single piece of bowel in
17 Cloacal Exstrophy and Covered Cloacal Exstrophy
tion. The ileostomy was closed, the colon (hindgut) was
disconnected from the urinary tract, and an end colostomy
was created
these patients, because sometimes the colon is
used to create a vagina or to augment the size of
the bladder. However, the decision to use gastroin-
testinal tissue to increase the size of the bladder or
to create the vagina should be taken years later at
the very end, after the pediatric surgeon has
decided whether or not the patient is a candidate
for pull-through or a permanent stoma. Even when
the patients improve significantly with this end
colostomy, sometimes the motility of the piece of
colon that the patient has is extremely poor and
behaves almost like an aganglionic piece of colon;
the patient develops proximal dilatation of the
bowel in spite of the fact that there is no stricture.
The stasis of stool produces bacterial proliferation
and the patients develop secretory diarrhea. For
that, the management that we offer to those patients
is to teach the mother to do irrigations like we do
with Hirschsprung’s disease and give metronida-
zole by mouth to prevent bacterial overgrowth.
In the past, we read in many publications that
the authors performed permanent ileostomies, or
sometimes the paper described the urinary recon-
struction, using gastrointestinal tissue, without a
mention of what was done in terms of colorectal
pull-through [40, 50–55]. Fortunately, we perceive
a tendency to change for the good and avoid ileos-
tomies [41–43, 48, 56].
17.2 Pull-Through or “Permanent Stoma”
a b
Fig. 17.6 Bowel management through the stoma to determine if the patient is a candidate for a pull-through. (a) Passing
a catheter. (b) Contrast in pouch
17.2 Pull-Through or
“Permanent Stoma”
Some patients obviously have a normal length of
colon, and because of that they are candidates for
pull-through, since they have the capacity to form
solid stool. Even when most of these patients
have a very abnormal sacrum and therefore poor
functional prognosis, we believe that they are
candidates for pull-through, because the quality
of life that we offer them, with the implementa-
tion of our bowel management program, is much
better than the quality of life of patients with an
end colostomy. This is something that the patients
tell us. Therefore, the only contraindication for a
pull-through that we recognize at the present
time, in anorectal malformations, is the incapac-
ity to form solid stool. Since this depends very
much on the length of the colon, each patient in
this spectrum of defects has a different chance to
have a pull-through. If the patient has no colon
and therefore would never be able to have solid
stool, we can anticipate that the patient will
remain with an ileostomy for life. On the other
291
hand, if the patient has half or one third of the
normal length of colon, we are not sure if the
patient will be a candidate for a pull-through.
Under those circumstances, we open the end
colostomy and watch the patient in terms of
growth and development. As mentioned before,
small pieces of colon sometimes grow much
more than what we expected, provided they are
included into the fecal stream. Therefore, every
6 months or every year, the patients come back to
our clinic, and we inject water-soluble contrast
material through the stoma and monitor the size
of the piece of colon. In that way, we can
document its growth and development. At the age
when the patient is expected to be clean and dry
in the underwear (usually 3 years old) and the
family and the patient are unhappy about having
a stoma, if we are not sure about how good is the
water absorption capacity of the colon and
whether or not the patient will have a successful
bowel management, we offer the family our
“bowel management through the stoma”
(Fig. 17.6). This means that we teach the mother
how to give enemas through the stoma itself. The
292
goal of the management is to have the patient
with an empty colostomy bag for 24 h after the
enema. If we achieve that, it means that the same
result can be achieved in the event of taking that
stoma down as a neo-anus. Sometimes those
patients require not only the enema, but in addi-
tion, they need a constipating diet and the admin-
istration of loperamide. If we are successful with
this bowel management, the patient and the par-
ents then have an idea of the amount of effort that
will be required, in the event of a pull-through,
for the patient to stay completely clean in the
underwear. Sometimes, the parents find that even
when the bowel management through the stoma
is successful, the effort to keep the patient clean
or the stoma bag clean is too much for the patient,
and they prefer not to go for the pull-through.
However, the enema, given through the stoma,
keeps the stoma bag empty, and parents decide to
continue giving the enema through the stoma,
because at the age when the children are more
active, playing sports is very advantageous for
them to have an empty stoma bag, rather than a
bag full of stool, with the high risk of leaking
during the school activities. Other parents decide
to go for the pull-through operation. Figure 17.7
shows an intraoperative view of a pouch colon. It
must be tubularized in order to pull it through.
Once the decision has been made to do the
pull-through, we can tell the urologist about the
decision; he may want to do some sort of urinary
reconstruction (usually bladder augmentation
Fig. 17.7 Intraoperative picture. Pouch colon must be
tailored to pull it down
17 Cloacal Exstrophy and Covered Cloacal Exstrophy
and Mitrofanoff), and it would be ideal to do it at
the same time. The colon is pulled down first,
since it will be placed posterior in the pelvis, and
the bladder reconstruction must be done after the
pull-through. These are long, technically
demanding procedures. It is very important not to
allow the patient to have a urinary reconstruction
and Mitrofanoff prior to the pull-through; other-
wise, the pull-through will become technically
much more difficult. Since these patients usually
have one or sometimes two appendices, we do a
Malone procedure in order for the patient to
receive an enema after the pull-through. The type
of enema has been previously determined during
a bowel management week through the stoma.
If the bowel management fails, in other words,
we are unable to keep the stoma bag clean, we do
not offer the family a pull-through, and they are
rather invited to come back 1 year later, since we
have evidence that the water absorption capacity
of the colon improves and the colon grows as time
goes by. At some point, if the colon is extremely
short, the patient is growing, and it is becoming
more and more important to keep the patient dry of
urine, and still the bowel management is not suc-
cessful, the family may decide, and we respect that
opinion, to go for the urinary reconstruction and
keep the patient with a permanent stoma for life. It
is at that point that the urologist must feel free to
use the colon and/or small bowel for the urinary
reconstruction. Table 17.1 shows our results in
terms of bowel, and Table 17.2 shows our results
in terms of urinary function.
Due to the advances of sciences and surgical
techniques, we have the opportunity to learn
about the long-term concerns of these patients.
We cannot overemphasize the importance of fol-
lowing our patients as long as possible. Patients
Table 17.1 Urinary control
Results 32 cases
Dry with intermittent catheterization 18
Awaiting for reconstruction 8
Incontinent 2
Voiding spontaneously, continent 1
Too young to assess 3
Total 32
17.3 Covered Cloacal Exstrophy 293

Table 17.2 Bowel function

Results 32 cases
Primarily continent 3
Continent with occasional soiling 3
Clean with bowel management 15
Incontinent 2
Too young to assess 5
Permanent ileostomy 4
Total 32

born with cloacal exstrophy are patients for life.

They must be followed by specialized members
of a dedicated team. We are learning about the
gynecologic concerns, including the possibility
of high-risk pregnancies. The urologic concerns
of these patients are multiple and very serious.
Our advances and progress, ironically, are cre-
ating new future challenges. The transition of
care, from pediatrics to adults, in patients with
complex malformations is taking by surprise, and
we are morally responsible for the well-being of
Fig. 17.8 Picture of the abdomen of a patient with a cov-
our patients. ered cloacal exstrophy. Observe the low implantation of
the umbilical cord

17.3 Covered Cloacal Exstrophy

We want to describe this subgroup of patients
that are born with most of the anatomic defects
seen in those patients born with cloacal exstro-
phy. They do have separated pubic bones, not as
severe as in cases of cloacal exstrophy, but sig-
nificantly separated. They also have an open
bladder neck, with a very small bladder that usu-
ally will require a major urinary reconstruction
(usually bladder augmentation and Mitrofanoff),
and frequently they also suffer from the spectrum
of anatomic abnormalities of the colon seen in
cases of cloacal exstrophy. This means that they
may have a normal-sized colon or a very short
colon, and in some cases they have the character-
istic pouch type of colon that patients with cloa-
cal exstrophy have. They have imperforate anus
with a fistula to the genitalia and also suffer from
spinal and sacral abnormalities. The only differ-
ence between this subgroup of patients and those
with cloacal exstrophy is the fact that the skin
that covers the abdomen is intact. Sometimes the
patients have low implantation of the umbilical
cord (Fig. 17.8). Other times they have an area of
very thin abdominal wall with separated rectus
muscle, which represents a “forme fruste” of an
omphalocele. The perineum is characterized by
the presence of a very large single orifice
(Fig. 17.9a). Because of this, this is considered a
cloaca; yet, the size of the orifice is extremely
large, compared with the characteristic typical
case of a cloaca. If one looks carefully, one can
see the urine constantly coming out through that
orifice (Fig. 17.9a). Some cases show four ori-
fices in the perineum (Fig. 17.9b), all within the
female genitalia.
This malformation has been described by sev-
eral authors before [57–63]. Nowadays it can be
diagnosed in utero fairly accurately [64, 65]. It has
also been reported in omphalopagus twins [66].
This is the kind of malformation that requires
a high index of suspicion in order to be diag-
nosed. The key for the diagnosis is to observe
carefully the abdominal wall, to see the low
294
a b
Fig. 17.9 Picture of the perineum of a patient with
covered cloacal exstrophy. (a) Observe the large size of
the single perineal orifice and the two separated pubic
implantation of the umbilical cord, and one can
see two prominences in both sides of the midline
in the area of the pubis, and by palpation, one can
feel that there is a fibrous band connecting both
separated pubic bones under the skin. The
perineum, as was described before, shows a sin-
gle orifice, rather large and leaking urine
(Fig. 17.8) [67].
It is extremely important for the surgeon to
make the diagnosis before embarking in the treat-
ment of these patients. We believe that it is
important for the family to know what they
should expect. We do not like to create false
expectations in the families. The surgeon is con-
fronting a case with a very similar dismal func-
tional prognosis and sequela than the typical
cloacal exstrophy. Obviously, a pediatric urolo-
gist might be involved in the management of
these patients. At birth, the treatment in these
patients should include a colostomy. Again, we
17 Cloacal Exstrophy and Covered Cloacal Exstrophy
prominences. (b) Another variant. Observe the urethra
(U), two hemivaginas (V), and rectum (R) opening in the
vulva
emphasized the importance of not leaving a piece
of colon attached to the urinary tract, particularly
if the patient has a short colon. If the patient has
an intact normal size of colon, conceivably the
surgeon can do a colostomy with separated sto-
mas and, like in any other type of anorectal mal-
formation, particularly if the rectum is implanted
low, near the perineum and is reachable from
below. Most of the times, these patients do not
have urinary tract obstruction, and therefore the
kidneys are well preserved. The patient may con-
tinue leaking urine until the age of urinary con-
trol (usually 3 years of age), and then important
decisions have to be made concerning the urinary
incontinence, and at that point, the patient will
need a major reconstruction. At that age, both
pediatric urologists and pediatric surgeons should
know how much bowel the patient has, and based
on that, what type of gastrointestinal tract is more
convenient to use for the augmentation of the
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more liberal to use a piece of bowel that he wants
for the bladder augmentation.
Concerning the surgical management of the
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the management of cloacas and cloacal exstro-
phies (see Chap. 16).
The functional prognosis of these patients
seems to be similar or better than in cases of cloa-
cal exstrophy. Therefore, we put together the
results of covered cloacal exstrophy and cloaca
exstrophy in Tables 17.1 and 17.2 of this chapter.
Our experience includes 28 cases and was
reported in 2013 [67].
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 General Principles
for the Postoperative
Management of Patients
with Anorectal Malformations
18.1 General Care
As discussed in each one of the specific anorectal
malformations, patients operated with a protec-
tive colostomy that underwent a posterior sagittal
repair, without opening the abdomen, are usually
fed the same day of the operation. The pain that
these patients suffer from is minimal. We try to
stay away from strong pain medications, such as
morphine. The patients feel comfortable once
they feel the presence of the parents. We keep
them in the hospital for 48 h in order to adminis-
ter intravenous antibiotics. We do not have cases
of infections after a posterior sagittal anorecto-
plasty, performed with a protective colostomy.
When the patient is discharged home, we ask
them to come back to the clinic 2 weeks after the
operation, to start the process of anal dilatations.
Occasionally, patients suffer from vomiting after
the operation; we believe that it is related with the
anesthesia, since we did not perform any abdomi-
nal surgery.
When we perform a posterior sagittal anorecto-
plasty without a colostomy as described in the
chapter on bowel preparation, the patients receive
GoLYTELY®1; in other words, we clean the entire
gastrointestinal tract. They also receive a PICC
line or central venous catheter in order for them to
receive parenteral nutrition. We tell the family that
the patient will remain with nothing by mouth for
1
(Polyethylene glycol/electrolytes) Braintree Laboratories,
Braintree, MA., USA
A. Peña, A. Bischoff, Surgical Treatment of Colorectal Problems in Children,
DOI 10.1007/978-3-319-14989-9_18, © Springer International Publishing Switzerland 2015
18
a period of 7–10 days after the operation; we
explain that he or she will not be hungry because
we will be providing parenteral nutrition. After
7 days from the procedure, we look at the perineum
of the patient. If everything looks well and it seems
to be healing nicely, we let them eat. On the other
hand, if we have any doubts or we see dehiscences
of 1, 2, 3, or 4 sutures, either in the anoplasty, in
the perineum, or in the posterior sagittal incision,
we continue the period of fasting for 2 or 3 more
days, take the patient to the operating room, and
resuture the dehiscent areas. Very rarely, we have
patients that suffer from a complete dehiscence of
the anoplasty without a colostomy. In those cases,
we perform a colostomy and wait until the wound
is healed, not less than 3 months, in order to
reoperate.
Patients who underwent a posterior sagittal
anorectoplasty in addition to a laparotomy usu-
ally start having po feeds 2–4 days after the oper-
ation. That period of time depends very much on
the degree of ileus that the patient suffers from,
which is related with the manipulation of the
bowel during the laparotomy. Sometimes, we
operate on patients who had previous multiple
operations, and we spend long periods of time in
the operating room taking down all the peritoneal
adhesions. The longest operation that we per-
formed so far, nonstop, has been 18 h in a patient
with a cloacal exstrophy in which we were able to
repair the rectal component, the vagina, and the
urinary tract. Those patients obviously would
remain with nothing by mouth for longer periods
299
300 18 General Principles for the Postoperative Management of Patients with Anorectal Malformations
of time because they have a prolonged ileus. We
followed the general guidelines that most sur-
geons follow-up after complicated laparotomies.
Babies subjected to long operations (longer
than 6 h) always go to the intensive care unit. We
have seen a tendency for them to suffer from
respiratory arrest postoperatively.
Occasionally, we operate on patients that
came to us with a loop colostomy. As we previ-
ously discussed in the chapter of colostomies,
some loop colostomies are totally diverting, and
we see that the entire stool goes into the stoma
bag. However, when those loop colostomies
retract a little bit, they pass stool into the distal
bowel. When that happens, after the operation, to
avoid contamination, there are several maneuvers
that may prevent fecal contamination. One can be
the introduction of a Foley catheter in the distal
colonic limb, with the balloon inflated for several
days, to avoid the passing of stool. Another one is
to place a purse string suture in the distal stoma
with a heavy, long-term, absorbable suture to pre-
vent the spillage of stool into the distal limb.
18.2 Local Care
We do not restrain the movements of our patients.
We do not tie them to the bed. We do not keep the
legs together. We let them walk around and move
in any way they want. However, we encourage
the mother to use a double diaper, to serve as a
cushion for the bottom. Also, we prevent the
patients from jumping and sitting in a rough way
after the operation. Because the posterior
approach is basically painless, the patients start
jumping around, and sometimes they injure
themselves. We keep the double diaper for
1 month. We like to use antibiotic ointment on the
posterior sagittal incision, the anoplasty, and the
area of the genitalia, in cases of female malfor-
mations. However, we use this ointment only for
5 days, because we have seen that after that, the
patient has a tendency to suffer from fungus
infection in the perineum.
We do not believe that the urine contributes to
producing dehiscences. In female babies, with a
rectovestibular fistula or rectovaginal fistula, we
do not leave a Foley catheter. In cloaca patients,
the type of urinary diversion that we use postop-
eratively depends on the type of cloaca that the
patient has (see Chap. 16). We leave a Foley cath-
eter for 2 weeks in a case of a posterior sagittal
anorectovaginourethroplasty and total urogenital
mobilization with a short common channel.
When the patient has a common channel longer
than 3 cm and we enter into the abdomen, we pre-
fer to leave a suprapubic tube particularly if we
believe that the patient will be able to urinate nor-
mally, before 3 months after the operation. When
the patient has severe kidney damage, hydrone-
phrosis, megaureters, and complex malforma-
tions and we believe that it is going to take longer
than 3 months for them to pass urine normally,
we prefer to leave a vesicostomy.
In male patients with rectourinary fistulas, we
leave a Foley catheter for 1 week. After a week,
the patient comes to the clinic, our nurses pull the
catheter out early in the morning and ask the par-
ents to take the baby to walk around and drink a
lot of fluids, to be sure that the patient is passing
urine normally before sending him home.
Occasionally, the patient shows signs consistent
with burning of the urethra during the voiding
episodes; we encourage them to take more fluids
and eventually the symptom disappears. It is
extremely unusual to see patients in urinary
retention after a technically correct posterior sag-
ittal operation. However, if the patient has a poor
sacrum, tethered cord, and a very high malforma-
tion, we can anticipate that, occasionally, they
may have urinary retention. Under those circum-
stances, we leave the Foley catheter for a few
more days and remove it again. If we already
know that the patient has neurogenic bladder,
then sometimes we leave a suprapubic tube, in
order for our colleague urologists to evaluate the
bladder postoperatively with a urodynamic study.
18.3 Anal Dilatations
Prior to the repair of anorectal malformations, the
parents of our children receive a piece of paper
describing our protocol of anal dilatations
(Fig. 18.1).
18.3 Anal Dilatations 301

Fig. 18.1 Anal dilatation. a Anal Dilatation Protocol

(a) Protocol. (b) Size of
dilators according to age • 14 days post-operative the surgeon will determine the initial size of the
Hegar dilator. He will demonstrate how to pass the dilator.

• The parents must perform the dilatation twice a day, passing the dilator
and leaving it inside for 30 seconds.

• Dilatations must be performed before meals.

• The baby must be restrained, keeping the knees against the chest.

• The size of the dilator must be increased every week, until reaching the
desired size, according to the patient’s age.

• Once final size has been reached, dilatations must continue twice a day
until the dilator passes easily. At that point, the parents must start tapering
the frequency of dilatations, guided by the following sequence:

– Once a day for one month

– Every other day for one month

– Every third day for one month

– Two times a week for one month

– Once a week for one month

– Once a month for three months

b Size of Dilators According to Age

Age Hegar #

0 – 4 months #12

4 – 8 months #13

8 – 12 months #14

1 – 3 years #15

3 – 12 years #16

> 12 years #17

(Hegar numbers represent millimeters in diameter)

We encourage the parents of our patients to
read the protocol and memorize it, before coming
to the clinic 2 weeks after our procedure.
We have been in national and international
meetings, in which some surgeons question the
need to perform anal dilatations in patients with
anorectal malformations. In fact, some surgeons
have expressed a negative impression about the
use of dilatations, because of the potential psy-
chological trauma produced in the children, due
to the pain that we provoke with these
dilatations.
As an answer for criticisms related with our
protocol of anal dilatations, we always explain
that patients subjected to pull-throughs follow-
ing old, rather rude, grotesque surgical tech-
niques end up having an anoplasty that actually
looks like a colostomy (Fig. 18.2). We agree
302 18 General Principles for the Postoperative Management of Patients with Anorectal Malformations
Fig. 18.2 Grotesque, prolapsed anoplasty. Does not
require dilatations
that in the type of repair, the patients do not
need dilatations. However, in patients that are
subjected to technically correct surgical tech-
niques to repair anorectal malformations, par-
ticularly if the patient has good sphincter
mechanism and the rectum is placed within the
limits of the sphincter, at the end of the proce-
dure, as can be seen in the pictures that we
show after the surgical repair of each malfor-
mation, the anus looks closed (Fig. 18.3). This
is consecutive to the effect of the sphincter.
This is seen in good anorectal malformations
subjected to good repairs. If the patient is left
like that with a colostomy, for several months,
the anus is going to heal that way and is going
to suffer from a stricture. We believe that what
we do are not really dilatations, but we rather
want the anus of the patient to heal with the
rectum open, in the way we left it at the end of
the procedure. When we do the anoplasty in the
way that can be seen in diagrams and pictures
in the chapters of this book, one can see that at
Fig. 18.3 Postoperative appearance of a technically cor-
rect anoplasty, in a patient with good sphincter mecha-
nism. The anus is closed. The patient needs anal
dilatation
the end of the procedure, the anus has the size
of a normal anus for the age of the patient.
However, when we remove the retention
sutures that are holding the anus open, the anus
closes by the effect of the sphincter mechanism
that surrounds it. If we leave it that way, it is
going to heal that way. That is the reason why
we believe that it is necessary to follow our
protocol of anal dilatations. We emphasized
the importance of starting the dilatations
2 weeks after surgery, not earlier, not later.
Earlier than that, we would be concerned about
damaging the sutures; later than that, we would
be concerned about finding already fibrosis
that would make the dilatation process most
difficult.
Difficult dilatations that produce a lot of pain
and bleeding occur when the rectum that was
pulled down suffered from ischemia, and in spite
of the efforts of the doctors, those cases usually
end up having a severe anal stricture.
We definitely oppose the routine followed by
some surgeons, consisting of trying to avoid pain
18.4 Avoiding Constipation
to the patient and booking the anal dilatations
under anesthesia once a week. The doctors do
that to calm the anxiety of the parents. Under
anesthesia, the patient does not complain, and as
a consequence, the doctors tend to over-dilate the
anus, producing injuries to the suture line. In the
following days, the anus is not dilated; the inju-
ries tend to heal, producing fibrosis. This is fol-
lowed by another traumatic dilatation every
week, with more fibrosis. In those cases, we see
patients coming to our clinic with severe fibrosis
that is impossible to dilate. Because of that, we
are very strict about our protocol. In the clinic
2 weeks after surgery, we show the parents how
to dilate the anus and we passed a dilator that the
baby does not even feel. It is usually a no. 6 or no.
8 Hegar. We do that in order for the mother to
gain confidence performing dilatations. We show
the father how to hold the baby with the knees
against the chest, to really immobilize him/her in
order to avoid showing the mother a “moving tar-
get,” which would make the mother more anx-
ious. With the baby completely immobilized, the
mother passes the dilator well lubricated, leaves
the dilator in place for 30 s, and repeats the
maneuver twice in the morning and twice at
night. We encourage the mother to do the dilata-
tion before meals, to avoid the possibility of
vomiting and aspiration. We specifically tell the
parents to increase the size of the dilator by one
size per week (1 mm per week) and continue
increasing the size until they reach the size that is
adequate for the age of the patient. Based on our
previous experience, we believe that a normal
anal size for a full-term newborn is a no. 12
Hegar dilator, for a 4-month-old baby a no. 13,
for an 8-month-old baby a no. 14, and for 1-year-
old baby a no. 15 Hegar. Older than that, usually
we recommend to go up to no. 16 Hegar dilator
(Fig. 18.1b).
Once the parents reach the adequate size for
the age of the patient, we encourage them to con-
tinue doing the dilatations twice a day until the
dilator goes easily and painlessly. At that point,
the parents are supposed to start tapering the fre-
quency of dilatations. In other words, they move
from the routine of doing it twice a day to once a
day, for a month, and then every other day for a
303
month, twice a week for a month, once every
2 weeks for a month, and then stop the dilatations.
We are very emphatic when we explain to the par-
ents about the importance of dilatations, and we
explain that not following the routine of dilata-
tions may provoke anal stenosis that would be
impossible to dilate, and the patient may require
an operation. When the anoplasty has been done
correctly, when the blood supply of the distal rec-
tum is good, and when the technique has been
meticulous, the dilatations are not really painful.
Most of our patients subjected to primary
repairs of anorectal malformations were only a
few months old, and it is our experience that
those patients really do not have pain during the
anal dilatations.
When we reoperate patients that are older than
2 years old, dilatations become a problem,
because the patient comes to us already trauma-
tized from previous procedures and painful dila-
tations. Under those circumstances, particularly
when we know that the patients have a bad prog-
nosis type of malformation and we are dealing
with a reoperation, we actually tried to make the
anus a little larger than normal in diameter, being
aware that during the healing process, it will
decrease a little bit in diameter but still will be
adequate to pass stool. This facilitates the dilata-
tions and avoids any pain. Older patients are very
difficult to restrain in order to do dilatations, and
it is a more traumatic experience.
18.4 Avoiding Constipation
Constipation is by far the most common sequela
seen in patients born with anorectal malforma-
tions and subjected to surgical procedures that
include the preservation of the rectum. The resec-
tion of the rectum in patients with anorectal mal-
formations followed by a pull-through of colon
certainly may avoid the problem of constipation,
but for sure it will produce fecal incontinence.
We keep insisting that the rectum represents the
natural reservoir of the human being and there-
fore must be preserved as much as possible.
Patients with anorectal malformations suffer
from a hypomotility disorder of the rectosigmoid,
304 18 General Principles for the Postoperative Management of Patients with Anorectal Malformations
which translates in constipation. The constipa-
tion is more severe in patients with lower defects
(recto-perineal fistula, rectovestibular fistula, and
imperforate anus with no fistula); these patients
suffer from more constipation than patients with
recto-bladder neck fistulas. We do not know the
reason for that. It is important to remember and to
be more proactive and aggressive in preventing
and managing the constipation of those patients
that are at higher risk. When the patients have
been with a colostomy, for long periods of time
particularly if it is a transverse colostomy, with-
out repair of the malformation, they come to us
with a severe megarectum and sometimes fecally
impacted, they suffer from more severe constipa-
tion, and it is important to anticipate that to avoid
aggravation of the problem.
Avoiding constipation must start when we
open a colostomy; we insist on the importance of
cleaning the distal colon at the time of the open-
ing of the colostomy and to keep the colon col-
lapsed from day one. In addition, we try to avoid
transverse colostomies and try to repair the mal-
formations as early as possible to avoid dilatation
of the distal end.
We, as well as the parents, must be ready to
deal with the problem of constipation after the
colostomy is closed (in patients with colostomies)
or after the main repair, when the repair is done
without a colostomy. We have long conversations
with the parents of our patients and try to make
them paranoid against constipation. We tell them
that constipation produces fecal incontinence. We
know that it is actually overflow pseudoinconti-
nence, but we try to make the parents aware of the
importance of taking care of the constipation. In
the chapter of bowel management, we described a
group of patients that had a good repair, of a good
prognosis type of malformation, but did not
receive care for constipation; as a consequence,
they suffer from severe fecal impaction, megarec-
tum, and fecal overflow pseudoincontinence. We
try to avoid that in our patients.
The parents take home a little glycerin enema
to be used only if necessary. The golden rule is to
be sure that the patient passes stool every day. In
addition, the parents must develop the sense that
the patient is really emptying the colon. Many
persons do not know that passing stool does not
necessarily mean that the patient is not consti-
pated. Sometimes patients can pass many bowel
movements, but they do not empty, and therefore,
they suffer from severe constipation. If the patient
goes one entire day without a bowel movement,
before the baby goes to sleep, the parents must
give the small glycerin enema and must increase
the amount of laxatives or will start the adminis-
tration of laxatives if they have not given laxa-
tives yet. We prefer to use senna derivative type
of laxatives from day one, because it has a better
and more controlled effect. Pediatricians in gen-
eral prefer to use a different type of laxatives,
such as lactulose. The problem with those laxa-
tives is that they have a tendency to produce liq-
uid stools, which is one of the enemies of patients
with anorectal malformations. We do not know
the amount of laxatives that every patient needs;
we know that every patient needs a different
amount, and we determine the amount by trial
and error with each patient. As a routine, we
order an abdominal x-ray film 1 week after the
colostomy closure or after the pull-through; sub-
sequently, we order films to be taken after
2 weeks and 1, 3, and 6 months. Even when the
parents tell us that the patient is doing very well
and they think that he is not constipated, by fol-
lowing this routine, we detect many times severe
constipation that the parents were unaware of and
we can give laxatives to avoid that problem. Once
we start with laxatives, we explain to the parents
that that must be given on a permanent basis.
We explain to the parents that from the time
the colostomy is closed and until the age of bowel
control (2 1/2 to 3 years), their work and respon-
sibility consists of:
(a) Avoiding constipation
(b) Trying to achieve regularity in bowel
movements
Patients with anorectal malformations suffer
from a colonic (mainly rectosigmoid) hypomotil-
ity disorder, and if we leave them alone, they tend
to pass a small amount of stool through the day,
not really emptying. That is why they need help to
empty the rectosigmoid completely. In addition,
many of them do not have the excellent regularity
of the motility of the rectosigmoid of normal
18.5 Toilet Training
human beings, who usually empty the rectosig-
moid all at once, every day. After 1 year of age,
we encourage the parents to give the patients only
three meals per day and no snacks in between. By
doing that, we try to produce a gastrocolic reflex
only three times a day and not seven times, like
many children that have snacks in between. It is
much easier to toilet train a child that has two or
three bowel movements in a day at a predictable
time. The opposite, a patient that suffers from epi-
sodes of diarrhea followed by episodes of consti-
pation is very difficult to toilet train.
We explain to the parents that the big enemies
of our patients are two:
A. Diarrhea
B. Constipation
We try to keep the patients right in between.
We want them to empty the rectosigmoid every
day and have soft but formed stool. We do all that
trial and error, and we educate the parents to
achieve our goal.
18.5 Toilet Training
It is very important to determine the future func-
tional prognosis, as early as possible in patients
with anorectal malformations. When the patients
belong to the “poor prognosis” group (poor
sacrum, tethered cord, bladder neck fistula), we
do not encourage them to try to toilet train their
children. We try not to create false expectations.
The toilet training in our patients with a good
or reasonably good functional prognosis usually
starts by the age of 2. If we achieve our goal of
having our patients free of constipation with the
use or without the use of laxatives and also if we
achieve our goal of producing one to three bowel
movements, at regular times, during the day at
predictable times, then it is relatively easy to toi-
let train the patients.
In cases with poor functional prognosis, we
encourage the family to start our bowel manage-
ment program by the age of 3. We arbitrarily
selected the age of 3 because many children, at
least in the United States, are in diapers even
between 2 and 3 years of age. In addition, many
parents send the children to school by the age of
305
3, and we believe that the child must go to school
either toilet trained, like all other children, or
completely clean in the underwear, because of
the bowel management that we provide. We are
very much against the idea of sending a child to
school knowing that everybody is using normal
underwear except our patient. We believe that
that is not good from the psychological point of
view.
For the patients that have malformations with
a functional prognosis that is considered in the
middle of the spectrum, in other words, patients
that have about 50 or 60 % chance to have vol-
untary bowel movements by the age of 3, such
as cases with rectoprostatic fistula, if the patient
is not toilet trained by the time that the parents
want to send him to school, we offer our bowel
management program and explain to the parents
that it will not be necessarily for life. Every
year, the patient should come to our clinic, and
we will try to stop the enemas (bowel manage-
ment) and see how much bowel control the
patient has, when subjected to a regime that
includes giving three meals per day, laxatives,
and fiber with the purpose of making the stool
bulky. If the patient does not gain bowel control,
we encourage the parents to go back to the
bowel management with enemas, for another
year, and come back later, because every year
we have more chances to toilet train the patient.
Every year, the patient will be more cooperative
and more interested in being clean with the
management plan.
Concerning the urinary control, we can very
much predict what kind of urinary function the
patient will have, based on the original anatomy
and the associated defects of the sacrum and
tethered cord. A normal baby, at that age of
1 year old, most likely is not toilet trained for
urine; however, it is very easy to observe that the
baby passes urine in episodes, about every 3 or
4 h, with a good stream and then remains com-
pletely dry in between voiding episodes. That
patient has good functional prognosis, and we do
not need a urodynamic study to know that he will
have urinary control. On the other hand, if we
see a 1-year-old baby dribbling urine constantly,
obviously the patient will have problems with
306 18 General Principles for the Postoperative Management of Patients with Anorectal Malformations

urinary control. Patients who have a large blad-
der, rather atonic, difficult to empty, benefit from
intermittent catheterization and, due to the fact
that the bladder is hypotonic, they remain dry in
between catheterizations. Patients that have a
hypertonic bladder and a poor bladder neck are
more difficult to manage. Our urologist col-
leagues would indicate intermittent catheteriza-
tion not only to keep the patient dry but also for
reasons such as to protect the upper urinary tract
and kidneys, particularly when the patient has
neurogenic bladder and reflux.
 Postoperative Evaluation
It is very obvious that every day we feel the need
and the pressure to evaluate the results of our
operations in the most objective possible way.
One of the biggest problems of medicine and
surgery through history is the difficulty to mea-
sure, if possible with numbers, the quality of our
results.
In an effort to be objective in the measure-
ment of the bowel control, different surgeons
created scores. Unfortunately, all of these scores
that have been published until now are basically
deficient, because they include very subjective
issues. Some of them [1] try to give points to
subjective and abstract concepts such as “extra
underpants for liners needed,” “social problems,”
“activity restrictions,” and “rashes.” Obviously,
those concepts do not reflect the real result of an
operation. Others [2] include concepts such as
“influence in lifestyle,” “need to wear pad,” “tak-
ing constipation medicine,” and “lack of activity”
to “defer defecation for 15 min.” Other scores
[3] are extremely extensive, sophisticated, and
complicated and therefore cannot be used on an
everyday basis; in addition, they are full of sub-
jective concepts not necessarily relevant to bowel
control such as “in the last week has your child
experienced constipation?” “In the last week,
has your child experience pain in the abdomen?”
Even when some of these scores have been “vali-
dated,” we considered them highly inadequate.
Other scores [4] include a very controversial con-
cept such as “frequency of defecation” that is not
necessarily related to bowel control. We can say
A. Peña, A. Bischoff, Surgical Treatment of Colorectal Problems in Children,
DOI 10.1007/978-3-319-14989-9_19, © Springer International Publishing Switzerland 2015
19
the same about “stool consistency,” “stool odor,”
“constipation,” and “modifications of the diet.”
Other highly controversial concepts included in
these scores are, for instance [5]: “Does stool
leak so that you have to change your underwear?”
“Does bowel or stool leakage cause you to alter
your lifestyle?” One of the first scores created
to try to measure bowel control in an anorectal
malformation was designed by Kelly [6] but,
unfortunately, includes very subjective concepts,
such as “strength of puborectalis muscle action
on digital examination.”
Another very serious problem that we found
in all of our literature review is that when dis-
cussing results, in terms of bowel control, unfor-
tunately, most of the times, the surgeons continue
using the old, archaic, misleading classifica-
tion that divides the malformations into “high,”
“intermediate,” and “low.” In addition, most of
those papers do not include a description of the
characteristics of the sacrum and/or the presence
or absence of tethered cord. Those big deficien-
cies obviously disqualify many papers.
We propose that in the future, when we dis-
cuss results in terms of bowel control, we describe
malformation per malformation, in other words,
bladder neck fistula patients, prostatic fistula,
bulbar fistula, etc., since each one of those has a
different prognosis. In addition, we must subdi-
vide them into those with normal sacrum and
those with a very deficient one. A very deficient
sacrum is one with a sacral ratio lower than 0.4.
In addition, we must indicate if the patient has
307
308
tethered cord or not. We will be comparing rectal
prostatic fistula with rectal prostatic fistula, bul-
bar with bulbar, perineal with perineal, etc.
Frequency of bowel movements is not neces-
sarily related to the potential for bowel control
or the quality of the procedure that was used to
repair a malformation. The frequency of bowel
movements depends on the length of the colon
and/or the possibility of the patient’s suffering
from some sort of irritation or inflammation of
the colon. Likewise, the presence or absence of
constipation does not necessarily relate to the
quality of the operation or the capacity of the
patient to have bowel control. Constipation rep-
resents rather a hypomotility disorder that, as we
discussed in this textbook, is aggravated by many
other factors. In other words, there are patients
with severe constipation but continent; and there
are other patients that have no constipation and
they are incontinent. We should discuss sepa-
rately the results in terms of constipation and the
results in terms of bowel control.
In summary, we do not use any scores in the
evaluation of our patients. The optimal best result
in the repair of an anorectal malformation is to
have a patient that behaves like a normal individ-
ual, in other words, a person that has voluntary
bowel movements and does not soil or smear the
underwear. That is what we call totally continent
patients. Another category of patients are those
who have voluntary bowel movements. In other
words, the patient tells the parents that he/she
wants to use the toilet, voluntarily; he/she goes
there and has a bowel movement; occasionally,
the mother sees the underwear with smears or
soiling. The third category includes patients that
are totally incontinent; because they do not have
voluntary bowel movements, they simply pass
stool in the underwear. We realized that we are
still far away from being able to quantify with
numbers our results, but we believe that this is
a better way to evaluate patients with anorectal
malformations.
Efforts have been made in the past to evaluate
bowel control, using radiology. Justin Kelly pub-
lished some of the first studies on defecograms in
anorectal malformations [7]. He emphasized the
importance of the rectal angle, as a manifestation
19 Postoperative Evaluation
of the action of the “puborectalis muscle.” Yet,
the studies were difficult to interpret, basically
because in those years, anorectal malformations
were still classified as “high,” “intermediate,”
and “low.” The findings of those studies demon-
strated that the presence of that angle, in general,
correlates with good clinic results but not 100 %.
The concept was reintroduced by Yagi, doing
what he calls “postoperative fecal flow metric
analysis” in patients with anorectal malforma-
tions [8]. It is a very sophisticated study, but it
does not tell us anything new and does not help
us in the management of our patients. The same
concept of the anorectal angle was again used
to compare the results between posterior sagit-
tal anorectoplasty and the laparoscopic approach,
but unfortunately, the authors still refer to the
malformations in terms of “high” and “interme-
diate” [9].
Early in our practice, we performed defeco-
grams in all of our patients, and in general, a
good angle was more frequently associated with
patients with bowel control, but not necessarily in
all cases. Very soon we learned that bowel con-
trol was something much more complex to
evaluate.
Rectal manometry has been a very popular
study. We exposed our methodological doubts
about the validity of that study (see Chap. 25,
Sect. 25.3.2). Again, the authors keep referring to
the anorectal malformations as “high” and “low”;
they even claim that they can manometrically
evaluate the quality of the “internal sphincter”
[10–16]. The quality of the sacrum as well as the
presence or absence of tethered cord is not men-
tioned in those papers.
CT scan evaluation of anorectal malformations
has frequently been used. Obviously, the images
have better definition than the previous radio-
logic studies [17]. Unfortunately, CT scans show
only transverse sections of the pelvis; therefore,
when the rectum is completely anterior or poste-
riorly mislocated, the study is not good enough.
On the other hand, when the rectum is placed into
the ischiorectal fossa, the study is valid and may
guide us to make a decision about repositioning
of the rectum within the limits of the sphincter
(see Chap. 22). Unfortunately, sagittal views are
References
not seen in this kind of study, and we consider
that an important deficiency. We were ecstatic and
happily surprised to read in one of the papers that
the authors no longer refer to the puborectalis and
internal or external sphincter, but rather mention
the “muscle complex” [18]. We were also very
happy to see that some of the publications related
with computed tomography in anorectal malfor-
mations were very honest papers, in which the
authors mentioned that the clinical application of
those imaging findings were not applicable in the
everyday practice [19–21].
Electromyography has also been used to eval-
uate the sphincter mechanism in patients with
anorectal malformations [22–24]. The informa-
tion obtained with electromyography does not
help us to make any decision preoperative or
postoperative. We believe that there is no need to
use an electromyographic study to see a sphincter
that we already saw directly during an operation.
We have the feeling that sometimes the doctors
who perform sophisticated studies do it with a spe-
cific goal in mind, to find the preconceived struc-
tures that they learned, such as “external sphincter,”
“puborectalis,” and “internal sphincter.”
We are extremely enthusiastic about the MRI
technology to evaluate the anatomy of the pelvis,
particularly in anorectal malformations. We
believe that the MRI studies provide the best
images that reflect the real anatomy of the patient.
In fact, we feel that the MRI studies confirm our
operative findings and our concepts, related with
the anatomy of the sphincter mechanism in a nor-
mal individual and in patients with anorectal mal-
formations (see Chap. 2). We use MRI studies
when we deal with complex malformations and
particularly in patients that already had a previ-
ous operation and suffer from fecal incontinence.
The MRI study allows us to determine exactly the
position of the rectum as related with the sphinc-
ter mechanism. This study provides beautiful
sagittal images as well as transverse sections. We
perform the study introducing a large rubber tube
into the rectum to be able to see exactly what is
rectum and what is sphincter mechanism [25].
We have been very impressed by the fact that in
spite of the beautiful, realistic images, provided
by the MRI study, some authors insist in being
309
able to see a distinct “puborectalis muscle,” as
well as an “internal sphincter” with this study;
and yet, when we look at the images that they
provide, we only see a continuum of muscle and
no separation between those structures [26–29].
Some authors claim not only to be able to see the
“internal sphincter” in the MRI study, but they
evaluate the sphincter, creating what they call an
“internal sphincter score” [30]. The MRI study
has been used by others to “compare differences
between patients with constipation and fecal
incontinence” [31]. We do not believe that this
excellent imaging study is necessary to differen-
tiate those two conditions.
In summary, we only use MRI studies in
patients that were already operated on in the past,
and we want to know the specific location of the
rectum as related with the sphincter mechanism.
Preoperatively, we find difficult to do the MRI,
logistically speaking, in little babies, because
they need heavy sedation or anesthesia.
Endosonography has been used mainly in
adults to evaluate the sphincter mechanism pre-
operatively and postoperatively. We feel that the
images provided by that study are not nearly as
accurate as an MRI study. In addition, there is no
study that tells us exactly at what levels of the
anorectum are the images taken from [32].
The reading about the literature related with
imaging technology confirms the old saying:
“our eyes see only what we want to see.”
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 Bowel Management
for the Treatment of Fecal
Incontinence
20.1 Introduction
We use the term “bowel management for the
treatment of fecal incontinence” to refer to a pro-
gram implemented at our institution which is
designed to keep patients who suffer from fecal
incontinence artificially clean in the underwear
[1–3]. The management basically consists of the
administration of an individually designed enema
that is given once a day, which allows the patient
to remain completely clean in the underwear for
24 h. In a number of cases, the program includes
the medical manipulation of the colonic motility
with a specific diet and/or medication such as
loperamide.1
Enemas have been used for the management
of fecal incontinence for many years with vari-
able results [4–12]. When the patients come to
our clinic and their parents learn about what our
bowel management program is all about, it is not
unusual for us to perceive their disappointment.
They frequently explain to us that they expected a
more sophisticated management of fecal inconti-
nence; in fact, they frequently say that their child
1
Loperamide HCl 2 mg simethicone 125 mg (in each
caplet) slows the rate at which the stomach and intestines
move. It also increases the density of stools and reduces
the amount of fluid in the stool.
Electronic supplementary material Supplementary
material is available in the online version of this chapter at
10.1007/978-3-319-14989-9_20.
A. Peña, A. Bischoff, Surgical Treatment of Colorectal Problems in Children,
DOI 10.1007/978-3-319-14989-9_20, © Springer International Publishing Switzerland 2015
20
previously received enemas that resulted in no
improvement. Some even say that enemas actu-
ally make their son or daughter worse in terms
of bowel control. That parent’s reaction is under-
standable. The bowel management program that
we implement in our clinic includes therapeutic
elements (enemas, constipating diet, and medica-
tions to slow down the colonic motility) that have
been widely used in the past in the management
of fecal incontinence. However, we like to say
that we use the same therapeutic elements, but
with a specific, different rationale that allows us
to have a high degree of success. We explain this
to the parents and ask them for patience and tol-
erance, so we can demonstrate that the same ther-
apeutic elements (enemas, diet, and medication)
when used following a systematic rationale may
have much better results.
The basic principles of our program are:
• Every patient needs a different type of enema
because each one has a different type of colon
(size and motility).
• The only way to monitor the efficiency and
effectiveness of an enema is by taking an
abdominal x-ray film to determine the amount
and distribution of stool in the colon
before and after the enema. Every day (during
1 week), we readjust the volume, concentration,
311
312 20
and content of the enema, according to the
specific patient’s reaction and the radiologic
image of his or her abdomen.
• It is extremely important, as a first step, to
determine the patient’s specific type of colonic
motility in order to be successful. We infer
this from a contrast enema.
The bowel management program at our insti-
tution was created by trial and error, out of our
desperation, provoked by the follow-up of many
patients who suffered from this devastating prob-
lem (fecal incontinence). During this long pro-
cess (30 years), we learned many lessons, and we
believe that we now have reached an important
degree of expertise that allows us to have very
good results, and with that, we have improved the
quality of life of many children [1–3].
In dealing with anorectal malformations,
clearly, a perfect initial anatomic reconstruction
is only part of the job. After the operation, we
are obligated to continue following our patients
in order to manage the expected functional
sequelae; the ultimate goal being a child with
clean and dry underwear.
It is unacceptable to operate on a patient and
let other professionals, not knowledgeable about
our procedures, take care of the functional
sequelae related to our operations.
In retrospect, from our own experience in the
surgical treatment of anorectal malformations,
we have learned that roughly 75 % of our patients
have voluntary bowel movements [13]. This
means the child is capable of verbalizing his or
her desire to use the toilet voluntarily and suc-
cessfully. Unfortunately, half of that 75 % group
still soils the underwear occasionally. Usually
those episodes of soiling are a manifestation of a
degree of fecal impaction as a consequence of a
mistreated problem of constipation. Once we
readjust the amount of laxatives, the patient usu-
ally responds very well, and the soiling disap-
pears. That leaves us with approximately 25 % of
patients who suffer from total fecal incontinence.
It is this group of patients for whom we feel mor-
ally obligated to get them clean and from whom
we got the motivation to create, step by step, a
series of principles and maneuvers that we now
call the “bowel management program.” Soon
Bowel Management for the Treatment of Fecal Incontinence
enough, we started seeing patients not operated
on by us, suffering from fecal incontinence. In
fact, nowadays, most of the patients that we take
care of in our bowel management clinic are
patients operated on at other institutions. What
we convey here is the result of an accumulated
experience with the management of approxi-
mately 800 patients suffering from different types
of fecal incontinence.
The bowel management program is only a
medical and not a surgical treatment. Yet, most
pediatricians and gastroenterologists are not
familiar with this kind of management, and sur-
geons are usually “too busy” to perform medical
treatments. As a consequence, many fecally
incontinent patients remain rather abandoned,
looking for centers where they can receive the
benefit of a well-integrated, comprehensive,
bowel management program.
We have found that bowel management pro-
grams are not popular. There are several reasons
that may explain this. Hospitals like to advertise
what they do, but usually they advertise “elegant”
conditions and procedures. The public relations
departments of hospitals like to advertise, for
example, when the institution performs the first
cardiac transplant, when they inaugurate a depart-
ment of fetal surgery, or when they make an
advance in the management of cancer. In addi-
tion, generally speaking, the media does not like
to talk about fecal incontinence, stool, urine, and
sexual problems. To advertise the opening of a
department for bowel management is “not ele-
gant.” In fact, we found that many doctors in the
United States or in other countries perform an
operation to repair an anorectal malformation,
and then, when the patient goes back to their
clinic suffering from fecal incontinence, the sur-
geons refer the patient to us, for the bowel man-
agement, and sometimes they use derogatory
terms such as “go to that clinic to learn how to
give enemas.” It is a very common misconception
to believe that bowel management is equivalent
to giving enemas. In this chapter, we try to show
that the bowel management program is much
more than giving an enema.
We also believe that the bowel management
program is not popular because it does not pay
20.1 Introduction
well. Most insurance companies do not even
know what a bowel management program is. As
the reader will be able to learn from this chap-
ter, it takes a significant amount of time and
effort from surgeons and nurses to implement
the bowel management in a single child, and
insurance companies do not compensate for all
this. It is rather ironic that, at the present time,
a surgeon can charge about ten times more
money for a 30-min operation than he can for a
1-week treatment that requires a lot of dedication
and work with a child with fecal incontinence.
Yet, the bowel management program allowed us
to improve the quality of life, more than with any
operation that we have done and in many more
children.
In addition, there is certain reluctance by par-
ents to accept the bowel management program.
This is based on certain misconceptions. These
include the idea that the enemas may produce
malnutrition in children because some parents
think that the enemas wash out nutrients from the
bowel. We have to go through a long explana-
tion, including showing diagrams, to explain that
enemas only remove the waste material from the
colon and not the nutrients from the small bowel.
In addition, we have never seen a patient suffer-
ing from malnutrition related to the administra-
tion of enemas.
Another frequent misconception of many par-
ents is the idea that once they start the bowel
management, it is going to be for life. This con-
cept is partly true. Many patients, of course, were
born with severe anatomic defects that allow us
to predict that most likely they will never have
bowel control, and therefore we may reasonably
believe that the bowel management will be neces-
sary for life, unless a new scientific advance
allows us to offer them something better.
However, there are many other patients that have
a borderline kind of bowel control, who may ben-
efit from a temporary bowel management and
who later in life develop bowel control.
Another misconception is the idea that the fre-
quent administration of enemas will make a male
patient a homosexual. There is no evidence that
this could happen. Homosexuality is not more
common among patients who received bowel
313
management, and the overwhelming majority of
homosexuals have never received bowel
management.
Finally, many parents believe that subject-
ing their child to a bowel management program
may interfere with the natural toilet training pro-
cess. This is false. In fact, we are convinced that
the bowel management may help the patient to
become toilet trained. A temporary bowel man-
agement in a patient that has borderline bowel
control allows the patient to gain self-confidence
by feeling clean, not smelly, attend school, and
play with other children without being worried
about having “accidents” in the underwear. If
the patient has some potential for bowel control,
the bowel management is considered temporary
and gives the patient the opportunity of being
absolutely clean. The toilet training process can
subsequently be attempted during the summer
vacations, having more chance of success, partic-
ularly when the child already experienced being
clean and odorless. It will be easy for a child that
has been clean for several months to perceive
when he is soiled with stool. A child that grows
up with diapers and stool in the underwear all the
time becomes accustomed to that and sometimes
is more difficult to train.
Once we are successful with the bowel man-
agement regimen and keep the patients com-
pletely clean, provided the patients are old enough
to understand what an operation is all about, we
discuss with the parents and the patients the pos-
sibility of performing an operation that will allow
the patient to receive enemas in an antegrade man-
ner. This is through a small orifice or an artificial
device, located in the abdominal wall, connected
with the colon of the patient, frequently through
the cecal appendix (see Chap. 21). This has been
called Malone [14] or ACE procedure (antegrade
continent enema). There are many techniques and
different ways to do it. There is no question that
these antegrade enema procedures, or techniques,
are beneficial and contribute to improve the qual-
ity of life of many patients. However, we firmly
believe that these procedures are only indicated
when the surgeon has demonstrated that the bowel
management is successful. We have seen a signifi-
cant number of patients that were operated on at
314 20
other institutions, undergoing different types of
antegrade enema procedures; in whom the proce-
dures were successful, but the patients were still
dirty with stool in the underwear, simply because
the surgeon never implemented a good bowel
management program. We consider it highly inad-
equate to offer an antegrade enema operation to a
patient in whom the surgeon never proved or dem-
onstrated that the bowel management worked. If
enemas given through the rectum fail to keep the
patient clean, most likely they will be equally inef-
ficient when given in an antegrade fashion.
20.2 Goals of the Bowel
Management Program
The bowel management program was designed to
take care of patients who suffer from fecal inconti-
nence, from different origins, not only anorectal
malformations. Our goal is to keep the patient arti-
ficially completely clean 24 h per day, so the patient
can be socially accepted, attend school, play, and
become psychologically adjusted to society.
The majority of fecally incontinent patients
that we treat are patients that were born with
anorectal malformations, some of them operated
on by us, but the majority of them were repaired
in other centers. Another group of fecally incon-
tinent patients were born with Hirschsprung’s
disease; they were operated on and subse-
quently suffer from fecal incontinence. This is
very unfortunate because theoretically patients
with Hirschsprung’s disease who are born with
an intact continence mechanism, which receive
a technically correct operation, should not suf-
fer from fecal incontinence; yet, we have treated
many such patients (see Chap. 24). Part of our
routine evaluation of patients with Hirschsprung’s
disease, who suffer from fecal incontinence,
includes an examination under anesthesia to
determine the integrity of the anal canal. In a
technically correct operation for Hirschsprung’s,
the patient’s anal canal and the dentate line should
have been preserved intact. Having an intact anal
canal means that the sensation (indispensable
to have bowel control) most likely is preserved
and also that the voluntary sphincter mechanism
Bowel Management for the Treatment of Fecal Incontinence
Fig. 20.1 Destroyed anal canal
is most likely preserved. Unfortunately, we
see many patients who had an operation that
destroyed the anal canal (Fig. 20.1); the surgeon
resected it during the dissection and anastomosed
the normoganglionic bowel to the perianal skin,
leaving no trace of anal canal, which most likely
will make that patient fecally incontinent for life.
Another group of patients that suffer from
fecal incontinence are those who are born with
myelomeningocele and spina bifida. This group
represents a population of patients much larger
than the population of anorectal malformations
and Hirschsprung’s disease [15]. We have not
been actively advertising our program in that
population, because we do not have the logistic
capacity to take care of so many patients, particu-
larly with the limitations that were already men-
tioned, in terms of reimbursement, time, and
personnel. However, we have treated a significant
number of these patients who definitely benefited
from our bowel management program.
Other patients were born with sacrococcygeal
teratomas or other kinds of tumors in the pelvis.
The tumors or the resection of those tumors dam-
aged the structures that are important for bowel
control and led the patient to suffer from fecal
incontinence. Finally, patients who suffered from
severe pelvic trauma that damaged the mecha-
nism of continence may also benefit from this
program. Occasionally, we take care of patients
born with sacral agenesis without an anorectal
malformation.
20.3 Evaluation of the Patient for Bowel Management
20.3 Evaluation of the Patient
for Bowel Management
Characteristically, we receive letters, phone calls,
or e-mails of families of patients who hear about
us, from pediatricians or pediatric surgeons, or
they learn about our center through the Internet.
They send a letter or an e-mail asking for help.
We ask them to send us copies of the operative
reports of their child and request several studies
that can be done at home and sent to us, or alter-
natively, the patient and the family may come to
our center and have the studies done here. These
studies include:
• X-ray films of the sacrum and lumbar spine in
AP and lateral positions to evaluate for scolio-
sis and spinal hemivertebrae and to assess the
development of the sacrum from which we
can partially infer the functional prognosis of
the specific malformation
• Kidney ultrasound and voiding cystourethro-
gram to evaluate for associated urologic
problems
• Contrast enema with water-soluble contrast
material and without bowel preparation
• MRI of the pelvis (Peña/Patel protocol)2 –
mainly in patients born with complex
malformations
• MRI of the spine – to rule out the presence of
tethered cord or other associated spinal and
cord problems
• A voiding cystourethrogram in cases with an
abnormal kidney ultrasound or urinary
symptoms
The purposes of performing all these studies
in all patients that come to our clinic suffering
from fecal incontinence include:
• First, we want to find out the specific type of
malformation that they were born with and
their associated malformations (operative
reports, x-rays of the sacrum and lumbar
2
MRI Peña/Patel protocol: The purpose of this study is to
determine the position of the rectum in relationship to the
sphincteric mechanism. We try to see if the rectum is ante-
riorly, posteriorly, or laterally mislocated, in relation to
the sphincter mechanism. In addition, we look for other
abnormalities such as posterior urethral diverticulum fre-
quently found in these patients.
315
spine, and MRI of the spine to rule out tethered
cord).
Learning about these allows us to predict
whether or not the bowel management will be
given on a permanent basis or temporarily
(depends on the functional prognosis of the orig-
inal malformation and the associated problems).
Also, this will help us to detect a very special
and interesting group of patients that were born
with a “good prognosis” type of defect. They
underwent a technically correct operation, they
never received adequate treatment for their
constipation, and they suffer from overflow
pseudoincontinence. They only require laxa-
tives and no enemas!!
• Second, we want to learn about the type of
colonic motility that the patient has, which is
the key for success. (For that we use the con-
trast enema.)
• Third, we want to find out untreated or poorly
treated associated defects (mainly urologic).
This will be discussed separately due to its
importance. This is the reason to request a kid-
ney ultrasound, voiding cystourethrogram,
and MRI of the spine and pelvis. We also want
to know whether or not the rectum following
the pull-through is located within the limits
of the sphincter; the MRI done with a special
technique (Peña/Patel protocol)3 is the best
way to determine this.
We evaluate all those studies, elaborate a man-
agement plan, and then give the patient an
appointment to come to our clinic.
A few years ago, we decided to run our bowel
management program only during one specific
week every month rather than daily. During
that particular week, we see between 15 and 40
patients, all gathered for evaluation and manage-
ment of fecal incontinence. We start the first day
with a conference from one of us (surgeons) to
welcome the parents and to explain generalities
about the bowel management program. That is
followed by a lecture by one of our nurses to talk
about different types of enemas and techniques
of enema administration. Then, in our clinic we
see each one of the patients to discuss, on an
3
See footnote 2.
316 20 Bowel Management for the Treatment of Fecal Incontinence
individual basis, the kind of bowel management
that we will implement, which is different in
every patient, depending on the results of our
evaluation.
For the following days, the patient receives the
treatment (enema) we have chosen, plus the spe-
cific diet and/or medication when indicated. In
addition, the parents will bring the child to the
hospital every day and have an abdominal x-ray
film taken. The parents then call one of our nurses
daily and report to them the results of the enema.
The nurse asks the parents how the patient toler-
ated the enema, whether or not it was uncomfort-
able, the reaction of the patient including vomiting
and pallor (vagal reflex types of reaction), and an
estimate from the parents about the amount of
stool that came out with the enema. The nurses
will also ask what happened in the patient’s under-
wear during the 24 h after the enema and how
much time it took to implement the management
(from the beginning of the enema application
until the patient has finished evacuating his/her
colon). Sometime in the afternoon, the members
of our staff (surgeons and nurses) meet in a con-
ference room, see each one of the abdominal
films, and discuss the information obtained by the
nurses about each one of the patients. The abdom-
inal films show us the amount and location of the
stool in the colon which reflects the efficiency of
the enema. That information, plus the description
of the nurse about the patient’s reaction, allows us
to implement changes in the enema that may
include volume, concentration, or content (ingre-
dients) of the fluid that we use. We may also make
recommendations about diet and/or medication
when indicated. Our nurses then call the parents
of our patients and notify them of our decision.
This routine is carried out daily until we are suc-
cessful. Success is defined as a completely clean
and happy patient and parents. Usually, about a
week after this process, 95 % of our patients are
clean [1–3].
The rationales for the use of enemas, in patients
suffering from fecal incontinence, are based on
the idea of administering a volume of fluid in the
colon to provoke a peristaltic wave, followed by
a partial or total expulsion of stool (Animation
20.1). Under normal circumstances, and in the
majority or our patients, the colon moves slowly;
therefore, the new stool that reaches the colon
will travel through it in a period of time not
shorter than 24 h. During those 24 h, the patient
is expected to remain clean in the underwear. It
is therefore easy to understand that the success
of the program depends on the efficiency of the
enema to clean the colon and the motility of the
colon. In other words, if the patient passes stool
in the underwear, there are only two possible
explanations:
A. The enema did not clean the colon.
B. The enema cleaned the colon, but the colon is
moving too fast, and the new stool reaches the
anus before 24 h (Animation 20.2).
The only way to know which one of these two
circumstances is occurring is by taking an
abdominal x-ray film. The presence of significant
amount of stool in the colon, shortly after the
administration of the enema, means that the
enema is not cleaning the colon, and therefore it
must be upgraded. On the other hand, a rather
clean colon in a patient that is passing stool in the
underwear means that the colon is moving too
fast (liquid stool is not easily seen in a regular
abdominal film).
20.4 Individualization
of the Management
Based on the studies already mentioned, we
learn several very important facts that allow us
to design and individualize the management plan.
It took us several years to realize that within the
population of children suffering from fecal incon-
tinence, there are two main groups of patients.
The contrast enema is the most valuable study to
identify these two groups:
• Group A: Incontinent patients that suffer from
megarectosigmoid and constipation (hypomo-
tility of the colon) (Fig. 20.2). The majority of
patients belong to this group.
• Group B: Incontinent patients with a non-
dilated, spastic, or short colon that suffer from
a tendency to diarrhea (hypermotility of the
colon). This includes patients who underwent
different types of resection of the colon for a
20.4 Individualization of the Management
Fig. 20.2 Contrast enema of a patient with megarecto-
sigmoid and constipation (hypomotility)
variety of reasons (Fig. 20.3). This group also
includes patients who suffer from different
types of enteritis or colitis (inflammatory
bowel disease, food allergy, acquired phos-
phate enema-induced colitis, or idiopathic
hypermotility).
The separation of patients into these two
groups represents an essential element of our
program as well as a key to success. We have not
found in any of the previous publications [4–12]
a description of this classification of patients. The
management of patients in each group is radi-
cally different as will be described.
In Group A, patients that suffer from hypomo-
tility (constipation) and megarectosigmoid, the
management will put emphasis on trying to find
the enema (large and concentrated enough) to be
capable of cleaning a large floppy colon. Once
we find that enema, the patient will most likely
stay clean in the underwear for 24 or even some-
times 48 or 72 h due to the fact that the colon
suffers from hypomotility. No special diet or
medication is necessary to keep these patients
clean.
317
Patients who belong to Group B have a
hyperactive, non-dilated, spastic, or short colon
(hypermotility). Such a colon is very easy to clean
with a small enema. These patients need a rather
small volume, not a concentrated type of enema
(usually plain, normal saline solution is adequate).
However, the main challenge in this group of
patients is to keep the colon quiet or to reduce
its peristalsis enough to avoid bowel movements
in between enemas. In other words, the enema is
capable of cleaning the colon very well and very
easily, but because the patients have increased
motility of the colon or a short colon, new stool
will come out through the anus only a few hours
after the administration of the enema. This can
only be treated with medication to slow down the
colon such as loperamide4 (Animation 20.3), pec-
tin (a water-soluble fiber that helps bind the stool
and make it bulkier), and/or a constipating diet
(Fig. 20.4), depending on the severity of the hyper-
motility. In addition, we must try to identify and
eliminate irritating factors that may be responsible
for the increased colonic motility (inflammatory
disease, food allergy, phosphate enemas).
Not recognizing these two categories of
patients represents the main reason for unsuc-
cessful attempts to keep the patients clean.
We never prescribe enemas and laxatives to
the same patient, since that would make no sense.
The enema is meant to clean the colon; after that,
we hope to make the colon move very slowly to
allow the patient to remain clean for 24 h. Giving
laxatives will make the colon move fast, which
will result in “accidents” (passing stool in the
underwear) before the next scheduled enema.
There is a small subgroup of patients that
emerges from the clinical evaluation and the
studies that we have already mentioned. These
patients were born with what we call “benign
malformations” (malformations that are associ-
ated with an excellent functional prognosis in
our experience), underwent a technically correct
operation, have a good sacrum, have no teth-
ered cord, and suffer from severe constipation,
and yet, they behave like fecally incontinent
patients. The contrast enema shows a severe
4
See footnote 1.
318 20
a b
Fig. 20.3 Contrast enema of a patient with no rectosigmoid (previously resected), hyperactive and tendency to diar-
rhea. (a) Diagram. (b) Contrast enema
megarectosigmoid (Fig. 20.2). Interestingly,
even when they were born with a “good” prog-
nosis type of malformation and everything indi-
cates that they should be continent, they come to
our clinic complaining of “fecal incontinence.”
We have learned that those patients have a great
chance of suffering from what we call “overflow
pseudoincontinence.” In this group, we follow
a completely different strategy. The treatment
consists in the administration of enemas, until
the colon is completely clean, as radiologi-
cally demonstrated (disimpaction) (see Chap.
25; Animation 20.4). Once we have evidence
that the colon is completely clean, we start the
process of determining (by trial and error) the
laxative requirements of the specific patient. We
have learned that such requirements are differ-
ent in each patient and much higher than what
the books recommend. It is extremely important
to recognize the fact that every patient will need
a different amount of laxative, which is not easy
to predict. The dosage of laxatives that a patient
Bowel Management for the Treatment of Fecal Incontinence
needs is defined as the amount of laxative capa-
ble of emptying the colon completely, as dem-
onstrated radiologically (see Chap. 25). We have
the patient come to the clinic every day, take an
abdominal x-ray film, and increase daily the
amount of laxatives, until we see a clean colon.
At that point, we know the amount of laxative
that the patient needs. We then ask the parents
whether the bowel movements are occurring in
the diaper or in the toilet. If it is in the toilet,
it means that the patient is fecally continent; in
fact, he/she never suffered from fecal inconti-
nence, but actually was always “overflow pseu-
doincontinent.” All that he/she needed was the
administration of the right amount of laxatives
that had never been previously determined!!
Once we determine the required amount of laxa-
tive, it is up to the parents if they are willing to
give their child that amount of laxative for life
in order to keep the patient clean. Alternatively,
we offer them an operation designed to reduce
the laxative requirement that consists in the
20.4 Individualization of the Management
Fig. 20.4 List of Constipating Foods
constipating and laxative
types of food
Apple Sauce
Apple Without Skin
Rice
White bread
Bagels
Boiled, broiled, baked meat, chicken or fish
Soft drinks
Banana
Pasta
Pretzels
Tea
Potato
Jelly (no jam)
resection of the most dilated part of the colon,
preserving the rectum (Fig. 20.5), a subject
that will be discussed later. This third group of
pseudoincontinent patients unfortunately repre-
sents only about 5 % of our series. The diag-
nosis and management of this group has not
been mentioned in the available literature. It is
extremely rewarding to treat patients like these,
because with a little effort we really dramati-
cally change their quality of life. On occasion
we see a patient with potential for bowel control
and with hypermotility. In those rare patients,
slowing down the colon, so the patient has one
or two well-formed stools per day, allows them
to perceive rectal fullness and thus succeed in
having voluntary bowel movements.
The evaluation of our patients also allows us
to determine whether or not the bowel manage-
ment is going to be implemented on a perma-
nent basis or it is going to be a temporary one.
That will depend on the functional prognosis
of the malformation that the patient was born
with, including of course the characteristics of
the sacrum and the presence or absence of teth-
ered cord. In our experience, patients with blad-
der neck fistulas only have a 20 % chance of
having voluntary bowel movements by the age
of three [13], rectoprostatic fistulas 60 %, recto-
urethral bulbar fistulas 85 %, and rectoperineal
319
Laxative Foods
Milk or milk products
Fats
Fried foods
Fruits
Vegetables
Spices
Fruit juices
French Fries
Chocolate
fistulas 100 %. In females, perineal 100 % and
vestibular fistulas have 95 % chance. Cloaca
patients with a common channel shorter than
3 cm and a normal sacrum have about 70 %
chance of having voluntary bowel movements
[13]. The characteristic of the sacrum is very
important as it has been recognized over many
years. Patients with a sacral ratio less than 0.4
most likely will need bowel management for
life. (For more information about the way to
estimate the sacral ratio, go to Chap. 6.) We
have not seen continent patients who have a
sacral ratio shorter than that. For patients that
are in the middle of the spectrum such as rec-
toprostatic fistula (60 % chance of voluntary
bowel movements) or sacral ratio around 0.5,
we explain to the parents that it will not be easy
for them to have bowel control, yet their chil-
dren have “some potential” for bowel control
that must not be underestimated. We, therefore,
offer these patients the implementation of our
bowel management program when they reach
the age of three, as an expeditious and effec-
tive way to keep them clean and get them into
normal underwear. We explain that every 6 or
12 months, preferably during the summer vaca-
tions, we will give the child the opportunity to
become toilet trained and see if we can stop the
enemas. We call that a “laxative trial.”
320 20
Fig. 20.5 Sigmoid resection with preservation of the rectum
20.5 Laxative Trial
We first implement the bowel management, and
the patient remains completely clean and is sent
home to enjoy the fact that he is clean for the first
time in his/her life, attending school like a nor-
mal child. In the summer vacation we suggest
that they come back for one entire week for the
“laxative trial.” During that week, we encourage
the parents to stop the enemas and find out
together with us the amount of laxative that is
capable of producing 1, 2, or 3 soft but formed
bowel movements every day, emptying the colon
as radiologically demonstrated. We encourage
them to have only three meals per day, hopefully
with the same kinds of food. We do that with the
specific purpose to try to condition the colon to
produce a bowel movement at the same time
every day, hopefully at a predictable time, which
makes the process of toilet training much easier.
Bowel Management for the Treatment of Fecal Incontinence
We also give the patients a special type of
fiber called pectin (used to make jams) that does
not have a laxative effect, but rather has a bulk-
ing effect on the stool. A patient with borderline
fecal control may not be able to feel a liquid
stool; yet he or she may be able to feel a bulky
type of stool that is produced by this kind of
fiber. Working by trial and error, manipulating
the amount of the laxative as well as the amount
of fiber and giving three meals per day, we find
out over a period of 1 or 2 weeks whether or not
the patient is capable of having voluntary bowel
movements, remaining completely clean in the
underwear without receiving enemas. If we are
not successful, then we encourage the family to
go back to the bowel management (enemas) for
another year. Every year, we will deal with a
patient that is usually more cooperative and
more mature and therefore will have a better
chance of success.
20.6 About Our Program
Patients with borderline fecal control do have
some sensation, but it is not the exquisite sensa-
tion of a normal person. That is why a great deal
of interest and cooperation is required from the
patient in order to perceive when the stool is
approaching the anus. A child that is too young
and/or hyperactive or suffers from attention defi-
cit disorder most likely will not respond to that
faint sensation. Something similar can be said
about the sphincter. Most patients with anorectal
malformations have some kind of sphincter
mechanism, never as good as in a normal child,
but they can certainly try to use it. Some patients
are even capable of having bowel control with
diarrhea; yet, most of them cannot cope with an
episode of diarrhea. Therefore, during a laxative
trial, we try to keep the patients right in between
diarrhea and constipation. We want them to have
soft but formed stools and hopefully a minimal
number of times (1–3 times per day).
It is not easy to switch from enemas to laxa-
tives. We emphasize this to the parents to avoid
false expectations. Sometimes, we are successful,
but the patients prefer to continue with enemas,
because it requires less effort and discipline.
20.6 About Our Program
On the first day of the program, parents fre-
quently express skepticism when hearing about
the results of our treatment. Many of them say
that they already gave enemas to the child in the
past with no success. This is certainly under-
standable. Giving an inadequate enema to a
child that is fecally impacted and not being able
to clean the colon completely would make the
patient feel worse. An enema that is not good
enough to clean the entire colon just softens the
hard stool, increasing the leakage (Fig. 20.6).
A fecally impacted incontinent patient soils day
and night, but does not leak stool, and they rather
soil and stain the underwear. Therefore, an enema
that does not clean the colon, but rather softens
the hard stool, may produce more leakage and
will make the patient more uncomfortable due to
a more obvious problem of fecal incontinence.
We have to explain to the parents with special
321
diagrams the reason why they believe that an
enema makes the child feel worse; we ask them
to give us a chance to demonstrate that finding
the right type of enema will eventually keep him
or her clean.
Also, many of the parents express their dissatis-
faction or skepticism because their previous expe-
rience indicates that an enema was something
painful or uncomfortable. Again, we have to
explain to them that giving the right type of enema,
at the right time, in the right way, and using the
right concentration, should not hurt. If an enema
hurts, something has not been done well. We ask
the parents to be patient with us and allow us to
demonstrate, by trial and error, and over a period
of 1 week, that an enema does not hurt.
Many times, patients with fecal incontinence
suffer from severe, chronic diaper rash equiva-
lent to a second-degree burn of the perianal area.
It is therefore not surprising that they refuse to
any kind of rectal maneuver. If somebody tries to
give them an enema with a tube in a careless way,
touching the ulcerated skin around the anus, the
patient will confirm that this is a painful maneu-
ver. Actually, we have to explain to the parents
that inside the bowel there is no sensation, and we
have to convince the patient to allow us to pass a
tube that he will not feel. Interestingly, a success-
ful bowel management will cure the worst diaper
rash in about 48 h. At that point, the patient and
the family get the positive feedback that encour-
ages them to cooperate more.
During the administration of the enema, some
patients experience symptoms suggestive of a
vagal reflex. That is understandable and means
that most likely the enema was either given too
fast, has an excessively large volume, is too con-
centrated, or all of the above. The quick adminis-
tration of a high volume of fluid inside the bowel
provokes stretching of the bowel wall, followed
by a smooth muscle spasm which is manifested
in cramp or colic, sometimes including vomiting.
The treatment of this problem is very obvious.
We have to advise the parents to give the enema
slowly (over a period of 5–10 min). Sometimes
we also advise them to warm the fluid of the
enema to body temperature. We also suggest that
they start giving the enema slowly, watching the
322 20 Bowel Management for the Treatment of Fecal Incontinence

a b

Fig. 20.6 Inefficient enema in an impacted colon. (a) Enema fluid did not go in. (b) Enema given against gravity. (c)
Kinked enema tube
20.7 Content of the Enema
child’s reaction, who should be doing something
that he likes and that keeps him busy, like watch-
ing TV or reading a book. The moment the child
starts complaining of any discomfort in the
abdomen, the parents should reduce the speed of
administration of the enema, until those symp-
toms disappear. If in spite of all this the patient
complains of having cramps, it means that per-
haps the patient’s colon is hypersensitive to the
type of enema that we are trying to administer.
Some colons respond in a very violent way
to certain kinds of enemas, and others do not
respond, even to very concentrated enemas. We
do not have an explanation for this. A patient that
has these kinds of vagal symptoms usually needs
a lower volume or lower concentration of the
substances that we use in the enemas (glycerin,
phosphate, and/or soap.)
20.7 Content of the Enema
The enemas that we use in our clinic may contain
one or several of the following components:
• Normal saline solution
• Glycerin
• Castile soap5
• Phosphate (Fleet)6
• Others (Dulcolax,7 polyethylene glycol)
Early in our experience, we frequently allowed
the parents to prepare the saline solution by mix-
ing salt from the kitchen with tap water (normal
0.9 % saline = 960 cc water plus 1.5 teaspoons
salt). Most parents are very diligent and careful to
do this, but we had two scary experiences, with
patients who came unconscious to the emergency
room. When we measured the amount of sodium
in their blood, it was extremely high. In retro-
spect, when we asked the mothers of those
patients exactly how much salt they put in the
water, they could not give an accurate, reliable
answer, which means that most likely they were
5
Castile soap – 0.30 fluid oz. packets (1 packet = 9 ml);
mild, gentle soap ideal for soft soap enemas
6
Fleet – monobasic sodium phosphate 19 g, dibasic
sodium phosphate 7 g
7
Dulcolax ® bisacodyl
323
rather liberal in the administration of salt and
were not paying very much attention during the
preparation of the solution. Because of that, we
now advise to use only normal saline solution
from the pharmacy. Of course this is more expen-
sive, but we prefer to take the safe route.
During the first consultation we determine,
rather arbitrarily, the volume of saline solution to
be used. We do that estimate based on our experi-
ence and the image of the contrast enema. The
volumes that we used vary from 200 to 1,500 ml,
depending on the patient’s age and degree colonic
dilatation.
For glycerin, we usually use about 20 mL for
every 500 mL of saline solution. We increase or
decrease the amount of glycerin depending on
the patient’s reaction.
For Castile8 soap, we usually use one package
(9 cc) for every 500 mL of saline solution. Yet,
we modify this amount according to the patient’s
response.
The phosphate is added in the commercial
form known as Fleet9 enema. We use a pediatric
Fleet9 (60 mL) in patients up to 10 years of age.
In older patients, we use an adult Fleet9 (120 mL).
We never give more than one Fleet9 per day.
Giving more than that exposes the patients to suf-
fer from hyperphosphatemia, hypocalcemia, and
tetany [16–29]. We had only one patient who
experienced those problems after receiving three
Fleet9 enemas in a day.
We try to be as conservative as possible in the
use of these different solutions. This means that
if we can manage a patient with plain, normal
saline solution and keep him completely clean,
then that is all we use. We try not to use any
other ingredient. However, many patients receiv-
ing a saline enema only do not respond; in other
words, 2 or 3 h go by, after the administration
of the enema, and nothing comes out through
the rectum, indicating that the enema that we are
using is not concentrated enough. In that case, the
next option for us is to add glycerin, which fre-
quently makes the patient respond. Some patients
do not respond to the administration of glycerin,
8
See footnote 5.
9
See footnote 6.
324 20 Bowel Management for the Treatment of Fecal Incontinence
Fig. 20.7 Colon with significant amount of stool. This is
an indication to increase the volume and the concentration
of the enema
or they respond, but the colon does not empty
completely. The patient continues passing stool
during the day. An x-ray film of the abdomen will
show that the colon still has a significant amount
of stool (Fig. 20.7). Under those circumstances
we add phosphate (Fleet10) to the enema solution.
The phosphate is an extremely powerful ingredi-
ent and usually makes the patient respond well.
Unfortunately, we have seen a number of patients
who received Fleet11 enemas for a long period of
time and behave like patients with tendency to
diarrhea (hypermotility). In such cases, a contrast
enema shows a spastic, narrow, hyperactive left
colon (Fig. 20.8). A colonoscopy shows signs of
colitis, due to the chronic irritation produced by
the phosphate solution on the colonic mucosa.
The patient may have signs and symptoms of a
severe colitis, including the presence of blood and
10
See footnote 5.
11
See footnote 6.
Fig. 20.8 Spastic left colon, secondary to the prolonged
use of phosphate enemas
mucus in the stool. We have seen patients who
came to us originally suffering from constipation
and megasigmoid, received phosphate enemas,
and gradually changed, developing a tendency to
diarrhea and a spastic colon. It is rather impres-
sive to see the change in symptoms and radio-
logic image, related to the use of phosphate. In
these cases the administration of phosphate is
immediately suspended. It takes several weeks or
months for the symptoms to disappear. Because
of this, nowadays we try to stay away from the
use of phosphate and leave it as a last resource.
We try to manage our patients with saline, plus
glycerine and soap if necessary.
The administration of an enema may require a
significant amount of teaching from the nurses,
many times “hands-on” type of teaching. It is
wrong to prescribe an enema to a child and
assume that the father or the mother knows what
an enema is and how to give it.
We have learned that many parents think they
are giving an enema, and actually the enema fluid
leaks all over the place and the floor; the fluid is
not really going inside the patient.
The position of the patient during the admin-
istration of an enema is very important. Parents
20.8 Rationale to Change the Type of Enema
Fig. 20.9 Patients’ positions to receive an enema, according to age
sometimes try to give an enema to a child stand-
ing up, and obviously the fluid will leak on the
floor. The position of the patient must facilitate
the effect of gravity for the enema fluid to go
into the colon (Fig. 20.9). The position of the
patient also depends on his/her age, as can be
seen in Fig. 20.9.
If the enema fluid keeps leaking in spite of an
adequate patient’s position, then we advise the
parents to use a Silastic tube (14–18 F) and pass
it through the rectum as high as possible in the
colon, to be sure that the fluid really goes inside
the colon and does not leak during the enema
administration (Fig. 20.10). If that is not enough,
then we advise the use of a Foley catheter. The
catheter is introduced (5–10 cm), the balloon is
inflated (10 ml), and then the mother or father
must pull on the catheter in order for the balloon
to act as a plug and prevent leakage (Fig. 20.11).
Every patient has a different caliber anus and a
different-sized rectum; therefore, a different-
sized balloon is needed for each patient. We start
by testing a 10-mL balloon. The balloon is
inflated and traction is applied. If the balloon
comes out through the anus, this means that the
patient needs a larger balloon (20 ml), and if that
comes out, then we try a 30-mL balloon. The
325
patients that are more prone to leak during the
administration of the enema are patients suffer-
ing from myelomeningocele. Patients with ano-
rectal malformations respond better because they
have a certain degree of fibrosis in the anal verge
that usually allows them to hold the balloon
inside during the administration of the enema.
20.8 Rationale to Change
the Type of Enema
If we see that the abdominal x-ray film shows a
significant amount of stool in the colon (Fig. 20.7)
and the patient is still passing stool in the under-
wear, we have to increase the volume of the
enema. On the other hand, if the parents say that
it took a long time for the patient to pass stool
after the administration of an enema that means
we must also increase the concentration of the
enema. We typically start by increasing the con-
centration of glycerin. Some patients have a neg-
ative reaction to glycerin (severe cramps, nausea);
in such cases we try Castile12 soap. In some
patients we go as high as 30 mL of glycerin, and
12
See footnote 5.
326 20
a b
Fig. 20.10 Efficient (adequate) enema. (a) High colonic enema. (b) Enema given taking advantage of gravity
the patient still does not respond (meaning it
takes a long time to pass stool or does not empty),
and then we add soap in addition to the glycerin.
On the other hand, if the nurses give us infor-
mation provided by the parents, indicating that
the child reacted with vagal types of symptoms,
we try to reduce the amount of Fleet or the
amount of glycerin; we try only saline solution
and see how the patient responds.
There are patients that show an x-ray image of a
completely clean colon (Fig. 20.12) from day one,
and yet they still pass stool in the underwear in
between enemas. That happens in patients who suf-
fer from colon hypermotility. The liquid stool that
runs fast through the colon is not seen on a plain
abdominal x-ray film. These patients are the most
difficult to manage. In fact, most of the 5 % group
of patients that did not respond to our bowel man-
agement are patients who suffer from severe hyper-
motility. The colon was very clean from day one,
after the administration of a small saline enema, yet
there was no way to slow down the colon.
Bowel Management for the Treatment of Fecal Incontinence
To slow down the colon in hypermotility
patients, we have the following therapeutic
elements:
(a) Constipating diet (Fig. 20.4)
(b) Loperamide13
(c) Pectin
(d) Eliminating the irritating factor when known
(e.g., lactose intolerance, food allergy, phos-
phate enema, inflammatory bowel disease)
(see Animation 20.3).
The constipating diet we use is a very radical
one. Every patient has a different susceptibility or
idiosyncrasy to different types of foods. In other
words, some patients have severe diarrhea when
they ingest prunes, and in some other patients,
prunes have no effect whatsoever. Since we are
limited by time (1 week), we prescribe for our
patients with colonic hypermotility a diet that
has proved to be extremely constipating in most
patients. Once we are successful with that diet,
13
See footnote 1.
20.8 Rationale to Change the Type of Enema
Fig. 20.11 Foley balloon technique
we advise the parents to try to individualize and
liberalize the diet, trying to find out in the patients’
diet the really offending types of food that pro-
duce diarrhea. Our constipating diet is shown in
Fig. 20.4; it basically consists of the elimination
of milk and milk products, elimination of fats, as
well as all kinds of fried foods, all fruits except
apple and banana, and all cereals except rice. The
patients can eat all kinds of meat that is broiled
or boiled but never fried. They are allowed to eat
white bread, and they can drink artificially sweet-
ened soft drinks, but no fruit juices. It has been
our experience that this diet is constipating for
the majority of the patients that we see. Once the
patient comes to our clinic smiling because he/she
has been completely clean in the underwear for
24 h, we know that with that specific constipat-
ing diet and that specific enema, he/she is com-
pletely clean, and that is our baseline or point of
327
Fig. 20.12 Abdominal x-ray film showing a clean colon
reference. Then we tell the parents that during the
following weeks, they will be able to try to slowly
liberalize the diet. Typically, we ask the patient
what type of food he or she misses the most. The
answer is usually ice cream, pizza, or French fries,
so we tell the parent to give one of those types of
food for three consecutive days, in addition to the
constipating diet. If the patient still remains clean,
this means that the type of food that he/she loves
will now be a part of his regular diet. We ask the
patient again what is the next type of food that he
or she wants, and the answer may be ice cream.
Now we instruct the mother to give ice cream,
in addition to the same constipating diet, and see
what the effect is. If the patient is clean, that is
good news for him/her. Now he/she will be able to
eat the constipating diet plus ice cream. Let’s say
that then he/she says chocolate; we give him/her
chocolate, and he/she has “accidents” (meaning
passing stool in the underwear); now we know
that chocolate is a real “offender” and should go
to the list of forbidden types of food, most likely
on a permanent basis. Following a similar routine,
for the following weeks and months, the parent
328 20 Bowel Management for the Treatment of Fecal Incontinence
will be able to tailor a specific diet that is the
best for his/her daughter or son that will be most
tolerable.
We do something similar with the administra-
tion of the loperamide.14 For instance, we may
know that with a full dosage of loperamide14
and the diet, the child remains completely clean.
Yet we want to find out the minimum amount
of loperamide14 capable of keeping the patient
completely clean. If the medication had being
administered three times per day, the first step
would be to decrease the administration to twice
per day for one entire week and see the patient’s
reaction. If the patient is still clean, that means
that he or she only needs the administration of
loperamide 14 twice a day. Subsequently, we
decrease to once a day. We keep decreasing the
dosage to determine the minimal required amount
to keep the patient clean. Some patients may stay
completely clean without administration of lop-
eramide14. Some others need a minimal amount.
We like to say that fecal incontinence is not a
psychological problem but rather a physiologic
one. Many of our patients have already seen psy-
chiatrists and psychologists before coming to our
clinic. Certainly, fecal incontinence produces
secondary psychological problems. Most of the
times, however, those problems disappear when
the patient is clean in the underwear.
Many patients have also been on biofeedback,
which we believe is useless, particularly if the
patient was born with what we call a “bad mal-
formation” and does not have anatomic elements
for bowel control.
During the last visit to our clinic (the last day
of the week), we explain to the parents that a suc-
cessful bowel management is something adjust-
able. At that point, we know that that particular
enema with or without diet and/or loperamide15
works for the patient at that particular time.
However, we explain that the same enema may
not work next week. We have patients that have
been on the same regime for 10 years with no
changes, and they remain clean. Other times, par-
ents will call us after a month because, even when
14
See footnote 1.
15
See footnote 1.
they are giving the same enema that we prescribed,
the patient is passing stool in the underwear dur-
ing the day, in between enemas. The advice we
give to these parents is very specific: take a plain
x-ray abdominal film, and send it to us by e-mail.
We take a look at the image, and most of the times
we can say what is happening. If we see a lot of
stool in the colon (Fig. 20.7), it means that either
the enema that used to work no longer works or
the parents are not giving the enema correctly. We
then go over the technique of administration of an
enema with the parents to be sure that the enema
is really being given and not leaking. If the enema
has been given correctly, we conclude that the
enema that used to clean the colon now does not
work for unknown reasons. We can then increase
the volume and/or the concentration of the ingre-
dients. On the other hand, if the x-ray film that the
parents sent us shows a completely clean colon
(Fig. 20.12) and the patient is having “accidents,”
that means that for some reason the patient is hav-
ing hypermotility of the colon. It is either because
he/she suffers from diarrhea, he/she has a virus,
he/she is eating something that upsets his/her
colon, or the phosphate enema is producing a
spastic type of colon. The recommendation is
then to discontinue the phosphate if they have
been using it or to reduce the amount of glycerin
if they are using it. If there is evidence of a viral
diarrhea episode in the family, we simply tell
them to stop the enema until the patient goes 24 h
without a bowel movement and then restart the
bowel management again.
The parents should know that during an epi-
sode of diarrhea (viral or bacterial), the bowel
management must be suspended. The patient
must remain at home and should not go to school
because there is no way to avoid the leakage of
stool.
Once we are successful with the bowel
management, we discuss with the parents the
possibility of performing a Malone procedure
(continent appendicostomy or continent neo-
appendicostomy) or any other kind of ACE
type of operation. In Chap. 21, we will discuss
our technique and the type of operation that
we perform for the administration of antegrade
enemas.
20.9 Bowel Management for the Treatment of Severe Diaper Rash
We like to perform that operation when the
patient is capable of understanding its advantages.
Some parents want us to do a Malone procedure
too early (on a 3-year-old child). We believe that
is not appropriate, because perhaps they want the
operation to make things easier for them, but not
necessarily for the child. We like the participation
of the children. The patients that benefit the most
from antegrade enemas are patients who are usu-
ally older than 8 years of age and want to become
more independent. They want to give the enemas
themselves because they feel embarrassed when
the mother or the father is giving the enema. They
want to be able to go on camping overnights and
be more independent in general. Teenagers are
the population who benefits the most from this
kind of operation.
20.9 Bowel Management
for the Treatment of Severe
Diaper Rash
It has been our experience that patients who had
an anorectal malformation already repaired, fol-
lowed by a colostomy closure, suffer from severe
diaper rash as a consequence of the constant
passing of stool. Sometimes this occurs in other
colorectal conditions as well. Fortunately, this
is not a permanent condition. As time goes by,
the number of bowel movements decreases and
mothers are able to manage the severity of the
diaper rash. During the acute stage, the passing
of stool is so frequent that it makes it impossible
to change the diapers frequently enough. Also,
the use of different types of creams to avoid the
contact of stool with the skin fails when patients
pass stool constantly.
Under these circumstances, we give the par-
ents a long explanation, so they can understand
that the goal of the treatment is to avoid the con-
tact of stool with the skin. The different types of
ointments and creams that we prescribe only rep-
resent a potential barrier between the skin and the
stool. Our team of nurses tries different strategies
to avoid contact by stool with the skin, including
when possible keeping the baby with no diapers
and near a fan to keep the skin dry, to be able to
329
see when the baby passes stool and be able to
clean them with soap and water without rubbing
the skin.
In spite of all of this, the diaper rash some-
times is very difficult to treat. This led us to the
new idea of implementing a sort of bowel man-
agement, consisting in the administration of a
small glycerin enema, repeated twice per day.
Even when the patient recently had the colos-
tomy closed, and therefore has a suture line in
the colon, we have found that the administra-
tion of a 15-mL glycerin type of enema, twice
per day, does not represent a risk for the suture
line of the colostomy. Yet, that small enema pro-
duces a large bowel movement that will allow
the baby to remain several hours without passing
stool, which is very important for the treatment of
the diaper rash. After a few days or weeks, most
patients with anorectal malformation with an
intact colon eventually will develop constipation,
which means a lack of bowel movements, which
will help in the management of the diaper rash.
Yet, frequently, constipation is manifested by the
frequent passing of small amounts of soft stool
in the diaper, which exacerbates the diaper rash.
Arbitrarily, we decided that once the patient
is over 1 month post-colostomy closure, the
patient may be a candidate for the implementa-
tion of a full bowel management, even when he/
she is only a baby (before 3 years of age). The
bowel management in these cases is not imple-
mented for the treatment of fecal incontinence,
but for the treatment of diaper rash. We follow
the specific protocol of bowel management as
described in this chapter, but because most of
these patients are babies, we start usually with
an enema of 150 mL of saline solution or larger
volumes depending very much on the age of the
patient. The bowel management has a spectacu-
lar effect in curing the diaper rash, and the par-
ents like it very much. Even when a normal baby
has no bowel control and uses diapers, there is
a big difference between him and a baby who
was born with a poor prognosis type of anorectal
malformation. These babies have a tendency to
pass stool constantly, which is a reflection of a
motility disorder of the rectosigmoid. The con-
stant presence of stool in the diaper makes the
330 20 Bowel Management for the Treatment of Fecal Incontinence
rash extremely difficult to manage. This is seri-
ously exacerbated in cases with urinary inconti-
nence. The bowel management implemented in
babies under these circumstances has been very
successful.
20.10 Bowel Management
Through a Stoma
Many patients have come to our center with a
colostomy created at another institution, a colos-
tomy that was considered a permanent one.
Usually this is because the patients were born
with a poor prognosis type of defect (recto-
bladder neck fistula, absent sacrum, poor sacrum,
tethered cord, myelomeningocele), which under-
standably makes the surgeons believe that the
patient will never have bowel control. Based on
that assumption, the surgeons open an end colos-
tomy and explain to the parents that this is going
to be for life. Many of these parents look for us
and come for consultation with the hope and
expectation that we offer an operation that will
allow them to get rid of the stoma. Some of these
patients belong to the group of cloacal exstro-
phies or covered exstrophies. As is well known,
patients with cloacal exstrophy or covered exstro-
phy frequently have a very abnormal colon. It is a
spectrum that goes from a normal length colon to
an almost absent colon. In between, we see
patients with a very short and bizarre-looking
colon, with a very abnormal blood supply, and
we are not sure whether or not that amount of
colon is enough to absorb enough water, so as to
be able to form solid stool, in order to respond to
a bowel management program consisting of a
daily enema. Most of the patients with cloacal
exstrophy or covered exstrophy have a poor
innervation of the pelvic organs, due to a very
defective sacrum, defective spine, or meningo-
cele. We call covered exstrophy to a specific
defect consisting in separated pubic bones (see
Chap. 17), absent bladder neck, and abnormal
colon; yet, these patients do not have omphalo-
cele or bladder exstrophy; in other words, the
lower abdominal wall is intact. Inside their abdo-
men, however, they have a very similar anatomy
to the one seen in classic cloacal exstrophies,
meaning very abnormal colonic blood supply and
different degrees of short colon. For this group of
patients, most surgeons will open a colostomy
and assume that it is going to be a permanent one.
We look at the quality of the stool that comes
through their stoma; if they have solid stool, there
is a strong possibility that they will respond to
the bowel management (enema) administered
through the stoma. If they respond to the bowel
management through the stoma (meaning that we
are capable of keeping the patient completely
free of stool coming out through the stoma for
24 h), it means that we must consider the possi-
bility of a pull-through of the stoma down to the
perineum. The parents understand that the same
enema that was demonstrated to be efficient
given through the stoma must be given through
the rectum or through an antegrade continence
mechanism after the pull-through, in order for the
patient to remain clean. Sometimes, the patients
have extremely short colons, and we are not sure
whether or not they have enough water absorp-
tion capacity to respond to the bowel manage-
ment. In that particular group of patients, we are
afraid of offering the family a pull-through of the
stoma, which will be equivalent to a perineal,
incontinent stoma that will make their life miser-
able. The bowel management through the stoma
(Fig. 20.13) consists in following the same sys-
tematic protocol of administration of enemas
through the stoma, monitoring with abdominal
films the amount of stool in the colonic pouch
and following the principles already described.
Often we must add a constipating diet, loper-
amide, and pectin to help bulk the stool. We have
tried bowel management through the stoma in 13
patients. In four of them, we were unsuccessful
due to the fact that the patients had a very short
colon and therefore incapacity to form solid
stool. Our management was successful in nine
patients; seven of them had a pull-through proce-
dure even when we knew that the patient had a
bad prognosis for bowel control. These patients
had an ACE procedure at the same time of the
pull-through. The patients expressed their satis-
faction, and they seemed to believe that it is much
better, in terms of quality of life, to have no stoma
References
Fig. 20.13 Bowel management through a stoma
and receive bowel management than to have a
permanent stoma for life. Two patients were hesi-
tant about the pull-through; yet, they decided to
continue giving themselves the bowel manage-
ment through the stoma, because that, itself, rep-
resents an enormous advantage in their quality of
life, since they have now a completely empty
stoma bag for 24 h, which allows them to be
much more active, play sports, without risking a
leakage of stool from the stoma bag, which repre-
sents a potentially embarrassing experience.
These two indications (bowel management for
severe diaper rash and bowel management
through the stoma) have never been reported by
others as far as we know. We are very happy with
the use of bowel management for these kinds of
problems.
Biofeedback
We are aware of the enthusiasm of some authors
that related with the use of this modality of treat-
ment [30–32]. Our experience with this form of
treatment was never published. The patients who
received this kind of management for several
331
months were able to improve their manometric
parameters. However, such improvement did not
translate into a real, clinical, significant, positive
change for the patient.
Medication
The use of medication such as loperamide [33],
cholinergic drugs [34], or cholestyramine [35]
has been used. We believe that medication alone,
used in patients suffering from fecal incontinence
due to anorectal malformation, has very few pos-
sibilities of being successful. However, as we
mentioned previously, medications can be used
as an adjunct to our bowel management program.
Patients suffering from borderline fecal inconti-
nence may benefit from the use of drugs to regu-
late the colonic motility. In cases of overflow
pseudoincontinence, the laxatives used as indi-
cated can make the difference between inconti-
nence and bowel control.
Electrical Stimulation
This kind of treatment, posterior tibial or sacral
[35–37], is receiving a lot of attention, mainly
between adult colorectal surgeons. We have no
experience with this type of treatment. However,
since there is no rational explanation of the mech-
anism of action, we remain skeptical about it.
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M, Peña A (2009) Treatment of fecal incontinence
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 Operations for the Administration
of Antegrade Enemas
21.1 Introduction
As discussed in our previous chapter, the bowel
management program, consisting mainly in the
administration of enemas, plus sometimes treat-
ment with specific medication and a specific diet,
greatly changes the quality of life of many chil-
dren. Giving an enema to a small child is a rela-
tively easy task. It is easy for the parents to
position the child on their lap for the enema, and
usually the child tolerates this procedure very
well. As times goes by, as the patients grow, par-
ticularly in those patients that require an enema
for long periods of time or on a permanent basis,
it becomes logistically more difficult, simply
because of the size of the patient. If one tries to
imagine how to give an enema in a teenager, tak-
ing advantage of gravity, it is easy to understand
that is difficult. Sometimes the patients’ squat on
the tub to give themselves the enema, and the
whole procedure becomes very messy. We have
been trying to use different methods to teach
teenagers to administer their own enemas while
sitting on the toilet, which is not easy, because
the enema is then given against gravity. We teach
them to pass a Foley catheter into the rectum, to
inflate the balloon inside the rectum, and while
they are applying traction on the catheter, to
administer the enema. This is easy to say but not
easy to do. As a consequence, most teenagers
need some help from another person to receive
the enema, which is an embarrassing event.
Imagine a 13-year-old girl receiving enemas
A. Peña, A. Bischoff, Surgical Treatment of Colorectal Problems in Children,
DOI 10.1007/978-3-319-14989-9_21, © Springer International Publishing Switzerland 2015
21
every day with the assistance of her father or the
other way around, a 14-year-old boy assisted by
his mother to have an enema.
Malone, Ransley, and Kiely had a great idea
[1] of creating a mechanism for the administra-
tion of an enema in an antegrade fashion. They
thought that an enema could be given through an
orifice created in the abdominal wall, connected
to the appendix, which allowed the passage of a
small catheter directly into the colon to give the
enema while the patient is sitting on the toilet
[2–7]. This was a very creative concept that
gained popularity quickly. The original idea of
Malone, Ransley, and Kiely included a technique
consisting of dividing the base of the appendix,
rotating the appendix 180°, and reimplanting it in
the wall of the cecum, in a submucosal fashion, to
avoid leakage of stool through the appendix. In
retrospect, this represents a complex maneuver
that we now believe is unnecessary. The basic
idea of Malone and Kiely is still extremely valu-
able, but now we know that we do not have to go
through all the surgical maneuvers they described.
They also advocated the opening of the orifice in
the right lower quadrant, which is something that
we do not do at the present time; we prefer the
umbilicus [8–10].
Fairly soon, after Malone and Kiely popular-
ized their idea, many others [11–16] came up
with different ways to achieve the same goal.
Other variants included a “noninvasive” or “min-
imally invasive” procedure to introduce a Silastic
button into the cecum. That technique became
333
334 21 Operations for the Administration of Antegrade Enemas

very attractive because it is minimally invasive, it

a
does not require a big operation, and it is techni-
cally simple. It can be done under fluoroscopy,
with endoscopy, or with laparoscopy. However,
we have been exposed to several patients who
had a button cecostomy in the past; they came to
our clinic very unhappy about the multiple long-
term complications and sequelae of that procedure
that included malfunction of the button, leakage
of stool around the device, prolapse of the colonic
mucosa, and granulation tissue around the but-
ton. These problems have been seen by others
[17, 18]. Many patients also dislike the visible b
foreign body on the abdomen and are self-con-
scious about it, avoiding swimming and other
activities. Those patients required a revision,
removal of the button, and creation of a continent
appendicostomy. It is because of that experience
that we do not favor the insertion of button
cecostomies.
Several authors [19–24] had the idea of per-
forming these types of procedures laparoscopi-
cally. This, of course, has advantages but also
some limitations as will be seen in this chapter.
Fig. 21.1 External aspect of the abdomen of a child who
underwent a continent appendicostomy operation (Malone
procedure). (a) Without a feeding tube. (b) With a feeding
21.2 Our Preferred Technique tube

We believe that the essential purpose of this type

of procedure is to improve the quality of life of
the patient. Therefore, our goal is to perform an
operation that will allow the patient to have an
active life, play sports, swim, and not be embar-
rassed of an orifice that rather looks like a colos-
tomy or to have to hide a plastic device protruding
from the abdomen. We believe that these patients
benefit the most by the creation of a little hidden,
umbilical orifice that should be as inconspicuous
as possible (Fig. 21.1). We create that little orifice
in the deepest portion of the umbilicus, making it
invisible. Yet, making a tiny orifice and avoiding
stricture requires a technically challenging and
meticulous surgical technique.
We think that we should use the appendix as
much as possible. Yet, we are limited by the fact
that about 30 % of the patients in whom we plan
to do this operation have no appendix because it
had been removed in the past for other reasons.
The old surgical tradition of doing an appendec-
tomy as part of a laparotomy luckily seems to be
disappearing as surgeons become more cognizant
of the potential use of the appendix for the care of
the incontinent child. In addition, nowadays, urol-
ogists are using the appendix to create a conduit
that goes from the abdominal wall to the bladder
for intermittent catheterization which is known
as a Mitrofanoff procedure (see Chap. 23, Sect.
23.13). That is another reason why the appendix
is not always available for us for this operation.
Because of that, we had to learn to make appen-
dices out of the colonic or small bowel tissue.
We call that a “continent neo-appendicostomy”
[10]. We make the appendix out of a flap of the
colonic wall that is tubularized around a feed-
ing tube to make a new appendix. We have been
doing this, mainly from the ascending colon, but
we have cases in which, because of anatomic
21.2 Our Preferred Technique
circumstances, we were obligated to make the
neo-appendix from the transverse or the sigmoid
colon. We have done this, in cases with multi-
ple peritoneal adhesions or “frozen abdomens.”
In such cases, we have no choice but to use that
part of the colon that we have access to. Ideally,
we prefer to create the new appendix from the
ascending colon near the cecum but away from
the ileocecal valve.
We have done approximately 200 of these
operations (appendicostomies and neo-appendi-
costomies). On two occasions, we opened the
abdomen and for a couple of hours tried to divide
the multiple peritoneal adhesions between the
loops of bowel and decided to abort the proce-
dure, because we had the feeling that we would
do more harm than benefit to the patient.
A patient that has his or her native appen-
dix does not need colonic preparation to do this
operation. The operation in a patient with mini-
mal peritoneal adhesions, a thin abdominal wall,
a normal appendix, and a mobile cecum becomes
minimally invasive, although non-laparoscopic,
because it only requires a small incision (about
4 cm long) running from the umbilicus down.
The whole procedure can be done through that
incision in about 1 hour; the patient can be fed the
same day and discharged the following day from
the hospital. On the other hand, when the patient
has the appendix located in a rather bizarre posi-
tion (behind the duodenum or behind the liver)
or has multiple peritoneal adhesions, in addition,
suffers from obesity, and has no appendix, the
procedure becomes technically very demanding,
taking an unpredictable number of hours. In simi-
lar cases, provided the peritoneal adhesions are
not too dense, the laparoscopy may be helpful to
avoid a laparotomy in mobilizing the cecum.
It is very important, prior to the planning of
these operations, to know whether or not the
patient has an appendix. Some radiologists are
very optimistic and believe that an ultrasound is a
reliable study to determine whether or not the
patient has an appendix, but we all know that
such a study is not 100 % accurate in visualizing
a normal appendix. A contrast enema that shows
an appendix gives us 100 % evidence that the
patient has one, but if the contrast enema does not
335
show an appendix, it does not mean necessarily
that the patient does not have an appendix. A CT
scan seems to be a better study.
The importance of learning about the presence
or absence of an appendix prior to the operation
resides in the fact that creation of a neo-appendix
requires full bowel preparation with administration
of GoLYTELY. It is not advisable to make a new
appendix with a colon full of stool which would
increase the chances of infection. We are very proud
of the fact that we have not had any cases of infec-
tion. In case of doubt, therefore, we go ahead with a
total bowel preparation the day before surgery with
GoLYTELY as described in the corresponding
chapter in this book (see Chap. 7).
Continent appendicostomy and neo-appendi-
costomy, in our experience, have two main late
sequelae or complications: one is stricture (or
acquired atresia) of the little stoma that we create
between the tip of the appendix or neo-appendix
and the skin of the umbilicus. The other is the
problem of leakage of stool.
In our series, the frequency of stricture or
acquired atresia was 18 %. In trying to avoid this
problem, we have been practicing a technique
that takes time to learn and to master, but we
believe will eventually decrease the frequency
of stricture. This technique consists of creat-
ing a triangle of skin from the umbilicus that
will be accommodated in a spatulated appendix
(Fig. 21.3) [9].
Concerning the problem of leakage of stool
through the appendix, it is very common to hear
that some authors believe that the appendix is
naturally continent. This we found to be true, half
of the time. The leakage of stool through the
Malone orifice occurred in 2.9 % of our cases
who underwent a plication of the cecum around
the appendix, and in contrast, it occurred in 29 %
of those cases without plication. In addition, dur-
ing the performance of many of our continent
appendicostomy procedures, prior to making a
decision about plication or no plication of the
cecum around the appendix, we tested the natural
continence of the appendix by finger clamping
the ascending colon and the terminal ileum and
injecting saline solution through a feeding tube
introduced in the cecum through the tip of the
336 21
appendix. Once the cecum was full with saline
solution, the feeding tube was removed, and we
then observed if the saline solution leaked
through the tip of the appendix. Interestingly, we
found that about half of the appendices that we
tested leaked. In addition, some patients who had
no leakage during this test turned out to leak stool
after the operation. Because of that, we recom-
mend to create an anti-leakage mechanism by
plicating the wall of the cecum around the appen-
dix or the neo-appendix. This plication does not
guarantee that the patient will not leak, since
about 2.9 % of our cases still leak, despite the
plication. But we believe that we should give the
patient the maximum possibility of avoiding
leakage, which is a very uncomfortable compli-
cation and requires a full reoperation to tighten
the cecal plication.
The laparoscopic approach to perform opera-
tions for the administration of antegrade enemas,
of course, is very appealing [19–24]. For those
who believe that these patients do not need plica-
tion of the colon around the appendix, the laparo-
scopic approach becomes the ideal way to do this
operation, since all the surgeons have to do is to
find the appendix, grab it, and exteriorize it and
to perform the anastomosis to the deepest portion
of the umbilicus. This becomes an extremely
easy and fast procedure. However, some of those
patients eventually may leak (29 % in our experi-
ence) stool through the Malone orifice. The pli-
cation of the cecum around the appendix is
usually not done laparoscopically; it must be
done through an infraumbilical incision of the
same size of the incision that we use. Laparoscopy
is essentially used to mobilize the cecum. Also, if
the patient has no appendix, one has to make one
from a flap of the colon. That has not been done
laparoscopically as far as we know.
One way to approach this problem in a patient
in whom we do not know whether he/she has an
appendix, and we do not know whether the
patient has many peritoneal adhesions and also
do not know where the appendix is located, could
be to start the operation laparoscopically. Using
one or two ports to examine the peritoneal cavity,
if it turns out that the patient has a mobile appen-
dix and cecum, a 4-cm subumbilical incision is
Operations for the Administration of Antegrade Enemas
created through which the operation (including
the cecal plication) is performed. If the patient
has a fixed cecum and appendix or a retrocecal or
mislocated appendix in a difficult location, the
laparoscopic part of the procedure is extremely
beneficial to mobilize the cecum and appendix,
avoiding a big laparotomy.
Pediatric urologists have been using the
appendix, implanted in the bladder, as a conduit
to perform intermittent catheterization, a proce-
dure that is known as “Mitrofanoff operation” in
recognition to the doctor who designed the
operation (see Chap. 23) [25]. They frequently
must perform this procedure in patients who
suffer from both urinary and fecal incontinence.
As a consequence, many pediatric urologists are
doing the Malone procedure or one of its vari-
ants [26–35].
There is no question that the Malone proce-
dure benefits many patients, and that explains
why this operation is so popular [36–48].
Some surgeons and gastroenterologists advo-
cate the Malone procedure in cases of constipa-
tion “resistant to medical management” [49–52].
We are skeptical about this indication and have
the feeling that it represents an overuse of the
procedure, a “quick way” to take care of a rather
serious problem (see Chap. 25). The doctors who
perform a Malone procedure in patients suffering
from “non-manageable constipation” do not
define what “non-manageable” means, and we
feel that many of the operated patients could be
managed medically following our protocol.
A large number of patients with spina bifida and
myelomeningocele benefit from a Malone proce-
dure or one of its multiple variants [53–55]. Also,
adult colorectal surgeons are becoming familiar
with the antegrade enema concept [56–59].
21.3 Surgical Technique:
Continent Appendicostomy
We recommend not performing this operation in an
extremely obese patient, because the abdominal
wall may be so thick that the entire length of the
appendix is not enough to reach the skin
comfortably. We encourage these types of patients
21.3 Surgical Technique: Continent Appendicostomy
to lose weight prior to these operations. The risk of
infection also increases significantly in obese
patients.
If we have evidence that the patient has no
appendix, or we are not sure, we perform a full
bowel preparation with GoLYTELY (see Chap.
7). If we have evidence that the patient has an
appendix, then the patient does not need bowel
preparation.
The patient is taken to the operating room and
the skin of the abdomen is prepared, with special
emphasis on the umbilicus. We open with an
incision in the midline, running from the deepest
portion of the umbilicus down in the midline for
approximately 4 cm, which is enough for us to
introduce two fingers into the peritoneal cavity.
We explore the right lower quadrant.
Frequently, we find a very mobile cecum
with a very healthy appendix which we can
exteriorize through the little incision below the
umbilicus. When laparoscopy is used, the
cecum is mobilized and then the small infraum-
bilical incision is made. Occasionally the sur-
geon may find that the patient has a very short
appendix. Fig. 21.6 illustrates a surgical tech-
nique designed to elongate the appendix.
The appendix and cecum are exteriorized.
Then, we create windows between the appendi-
ceal vessels (Fig. 21.2a, b). We use these win-
dows to pass the wall of the cecum through them,
to be able to plicate the cecum around the appen-
dix, without producing vascular compression that
may produce appendiceal ischemia. We use 5-0
silk stitches to plicate the cecum around the
appendix (Fig. 21.2c). Prior to the plication, we
place two 6-0 silk stitches at the tip of the appen-
dix. The tip of the appendix is then opened with a
needle-tip cautery in between both stitches. A no.
8 feeding tube is passed through the tip of the
appendix into the cecum. The plication of the
cecum should be done on the lateral wall of the
cecum and not into the medial portion. If we
make it into the medial portion of the cecum,
there is a possibility that we produce an obstruc-
tion of the ileocecal valve (Fig. 21.2d, e). The
stitches of the plication take a full thickness bite
of the cecum and a small bite of the seromuscular
layer of the appendix. Every time we tie the
337
stitches, we have to be sure that we are not com-
pressing the blood supply of the appendix, and
we make sure we are not narrowing the channel,
checking by passing the catheter through it. We
look at the blood supply of the tip of the appendix
to be sure that it is viable. The cecum is then
sutured to the anterior abdominal wall around the
umbilicus with interrupted 5-0 long-term absorb-
able sutures. The ventral part of the tip of the
appendix then is spatulated by making an inci-
sion (Fig. 21.3a–c). Special care must be taken to
cauterize the distal end of the appendiceal artery,
and we do not hesitate to ligate it with a 5-0
suture. A triangle of skin at the umbilicus is cre-
ated (Fig. 21.3a–c). The tip of the triangle is
sutured to the angle of the spatulated appendix
(Fig. 21.3d); the edges of the triangular skin flap
are sutured to the edges of the spatulated appen-
dix with interrupted 6-0 long-term absorbable
sutures (Fig. 21.3d–g) creating a Y-to-V anasto-
mosis. The midline aponeurosis of the abdomen
is closed with long-term absorbable sutures, as
well as the subcutaneous tissue taking care to rec-
reate the interior umbilical fold. The skin is
closed with subcuticular 5-0 long-term absorb-
able monofilament suture. The no. 8 feeding tube
is left in place fixed to the skin with a silk stitch
(Fig. 21.3h). The cosmetic effect of this operation
is excellent. This operation takes approximately
45 min in a patient who is thin, has minimal peri-
toneal adhesions, and has a mobile cecum and
appendix. As I previously mentioned, the opera-
tive time may get much longer in an obese patient
with peritoneal adhesions, a fixed cecum, a mis-
located appendix, or an absent appendix.
Sometimes, we open the abdomen with the
incision that we described, only to find that the
cecum is not located in the right lower quadrant,
and we cannot feel it with our fingers. At that
point, the alternative is to extend the incision all of
the way to the lower abdomen and the upper abdo-
men and look for the cecum that sometimes is
located in the area of the duodenum or located ret-
rocecally, with the tip of the appendix located all
of the way up into the area of the liver. Alternatively,
we can use the laparoscope to look for the cecum
and the appendix to mobilize and bring them
through the small incision previously described.
338 21 Operations for the Administration of Antegrade Enemas

b c

d e

Fig. 21.2 Continent appendicostomy. (a) Diagram. (b) vessels. (d) Appendix laid down lateral to the cecum,
Creation of windows between the appendiceal vessels. (c) away from ileocecal valve. (e) Plication finished
Placing sutures for the cecal plication, avoiding the
21.4 Continent Neo-appendicostomy
21.4 Continent
Neo-appendicostomy
The patient receives a total bowel preparation.
The skin is prepared in the way described for a
continent appendicostomy. The midline abdomi-
nal incision is made, long enough to mobilize the
cecum and most of the ascending colon.
Usually, this type of procedure requires divid-
ing of the normal attachments of the cecum and
ascending colon from the right gutter. We have to
take down those attachments in order to bring the
cecum and part of the ascending colon through
the wound with no tension.
a b
c d
Fig. 21.3 Anastomosis of appendix to the skin of the
umbilicus. (a) Intraoperative picture. (b) Diagram. (c) The
tip of the appendix is spatulated (diagram). (d) The trian-
gular skin is accommodated into spatulated space, created
339
We have learned through the performance of
over 50 cases of continent neo-appendicostomy
to create a narrow and long vascularized flap of
the colonic bowel and yet to preserve an excel-
lent blood supply to the tip of the flap. This is
achieved by creating the flap following the
direction of the mesenteric vessels that run in the
colon (Fig. 21.4a). Looking at the normal blood
supply of the colon, one can see that the
mesenteric vessels of the ascending, transverse,
and descending colon run perpendicular to the
main axis of the colon. Therefore, the flaps that
we develop in the colon should be created
perpendicular to the main axis of the colon
at the tip of the appendix. Many 6-0 Vicryl sutures are
used (diagram). (e–g) Intraoperative aspect of the recon-
struction. (h) Final aspect of the maneuver
340 21
e f
g h
Fig. 21.3 (continued)
(Fig. 24.1a, b). That way, one can make a very
long, narrow flap, narrow enough to be tubular-
ized around a no. 8 feeding tube (Fig. 21.4d)
with an excellent blood supply running through
the length of the flap. When making this flap in
the ascending colon, the surgeon must keep in
mind that the right ascending colon is going to
be moved toward the midline in order for the tip
of the neo-appendix to be exteriorized through
the umbilicus. Therefore, we specifically recom-
mend making the base of the flap in the lateral
part of the ascending colon away from the ileo-
cecal valve. The flap must be created identifying
two good mesenteric vessels at the center of the
base of the flap (Fig. 21.4a, b). Those vessels run
in the main direction of the longitudinal axis of
the flap. The flap is raised (Fig. 21.4c). The ante-
rior wall of the colon is closed with two layers of
sutures; the first one is a running locked suture of
5-0 long-term absorbable sutures, and the second
one is done with interrupted 5-0 long-term
absorbable sutures. The flap is tubularized and
Operations for the Administration of Antegrade Enemas
sutured around a no. 8 feeding tube with multi-
ple interrupted 6-0 long-term absorbable sutures.
The area of the taenia of the colon is not elastic,
and therefore, we intentionally leave more tissue
in that part of the flap in order to be able to sur-
round the no. 8 feeding tube. We have been hap-
pily surprised by the good blood supply of these
long narrow neo-appendices, provided we pre-
serve the mesenteric vessels. The neo-appendix
is then laid down on the anterior wall of the
colon somewhat askew so the suture line of the
neo-appendix does not lie on the suture line of
the colonic wall, which is plicated using 5-0 silk
stitches in the way that was described before,
when we discussed the continent appendicos-
tomy (Fig. 21.2c, d).
The entire ascending colon is moved toward
the midline in order to attach the anterior wall of
the colon to the anterior abdominal wall around
the umbilicus with 5-0 long-term absorbable
sutures. The tip of the neo-appendix is spatulated
in the same way that we described for the
21.4 Continent Neo-appendicostomy
continent appendicostomy procedure and anasto-
mosed to the umbilicus using the same triangular
skin flap type of technique.
In a patient with a continent appendicostomy,
in whom we use the native appendix, the enemas
can be administered if necessary the day after
surgery through the catheter (which is left in
place). We usually start giving enemas through
that catheter 24 h after the patient is started on a
normal diet.
In Chap. 20, we emphasized the importance of
not performing this kind of operation without
having previously demonstrated that the patient
responded successfully to the enema program. In
other words, when we perform this operation, we
already know what kind of enema works in that
specific patient. Therefore, as soon as possible,
we start using the same type of enema that the
patient already knows that works very well.
In patients with a native appendix, this can be
done the day after surgery, if necessary. In
patients with a continent neo-appendicostomy,
we do not like to give high-pressure, large-
volume enemas before 1 month postoperatively,
because we are afraid of producing a perforation
of the colon since it has multiple sutures. In those
patients with a neo-appendicostomy, we encour-
age them to receive two small enemas through
the rectum, one in the morning and one at night.
These are small-volume, low-pressure enemas
that hopefully will not compromise the healing of
the ascending colon sutures. There is no guaran-
tee that those two small enemas will keep the
patient completely clean, but they will certainly
help. We follow that routine for one entire month
to give enough time for the patient’s colon to heal
safely. After 1 month, the enemas previously
designed, during the bowel management week,
can be safely given through the umbilicus.
The no. 8 feeding tubes are left in place
through the umbilicus in both kinds of operations
(appendicostomy and neo-appendicostomy). In
the case of a native appendix, the patient comes
to the clinic 2 weeks later; we remove the feeding
tube and show the patient and the family how to
pass a feeding tube every night. The great advan-
tage of this operation is that the appendix has no
sensation, and therefore, the patient is happily
341
surprised to see that the entire maneuver is totally
painless. In patients with a neo-appendicostomy,
we do this maneuver after 4 weeks. The patient is
usually very happy to see how easy and gratify-
ing it is to use this new orifice in the umbilicus, to
receive enemas (Fig. 21.4).
We believe that it is extremely important to
make this orifice as invisible as possible.
However, sometimes, in spite of our attempts,
one can see a little bit of mucosa visible in the
umbilicus (Fig. 21.5). We have had a couple of
patients who required a trimming off of that
mucosa as a secondary operation, and one of
them developed a stricture after that revision.
There is a dilemma between leaving too much
mucosa prolapse (similar to a colostomy) (by
doing that, no strictures will occur) and being
very ambitious and try to make it as invisible as
possible, which has a higher risk of developing a
stricture. We believe that the triangular skin flap
technique helps to decrease the incidence of
stricture and also keeps the stoma hidden on the
umbilical fold.
Sometimes, several weeks later, the parents
report to us that they are having difficulty passing
the catheter, which usually means that the orifice
is getting strictured. When that happens, we tell
them to pass the catheter and leave it in place for
a couple of weeks, to continue giving the enema
every day with the catheter in place, and then
after 2 weeks, to try again and see if they have the
same difficulty. If they have problems after doing
that, we ask them to come to our clinic, and we
try dilatations of the stoma with fine Hegar dila-
tors. If that does not work, then we offer them a
revision to make the orifice larger. This happened
in 15 % of our cases. The revision of a stricture is
a 30-min procedure performed on an ambulatory
basis. The stricture occurs at the skin level and is
only a ring-like fibrous band. More recently, we
have been recommending the use of a little
Silastic device called “stopper”1 [60, 61].
When the patients report to us that they are
leaking stool through the umbilicus, we ask them
if it is a daily problem, if it happens all of the
1
ACE Stopper (ACE Stoma Device) – Marian Medical,
Inc.
342 21 Operations for the Administration of Antegrade Enemas

Fig. 21.4 Continent neo-appendicostomy. (a) Diagram. flap and closure of the colon. (e) Lying down the appendix
(b) Design of a colonic flap, based on a mesenteric vessel. on the colon, away from the colonic suture, prior to the
V vessels. (c) Extending flap. Observe excellent blood plication
supply at the tip of the appendix. (d) Tubularization of the
21.4 Continent Neo-appendicostomy 343

Fig. 21.4 (continued)

344
Fig. 21.5 Prolapse mucosa through the Malone orifice
time, or only when the patient is having diarrhea.
If the leakage occurs only occasionally and/or
when the patient has diarrhea, we tell the parents
that the patient should try to avoid laxative type
of food. On the other hand, if the problem occurs
every day, we offer the family a reoperation to
re-plicate the cecum around the appendix.
Fortunately, there are some good publications
related with the long-term outcomes of these
procedures, including a significant number of
complications and reoperations [62–65]. The fre-
quency of stricture of the little stoma varies from
6 to 40 %, the leak rate from 10 to 32 %, and
mucosal prolapse 4 %. Many patients abandoned
the antegrade enema (8–11 %) because they found
that it was not helpful.
Unfortunately, in most series, there is no real
discussion related with the type of enema that
they used. As a consequence, many patients are
subjected to this type of procedure, only to
remain fecally incontinent. We like to say that
what failed in those cases was not the operation,
but rather the bowel management that, for us, is a
prerequisite for the operation. We only offer the
21 Operations for the Administration of Antegrade Enemas
procedure to those patients in whom the bowel
management has been 100 % successful. We
believe that the Malone procedure or any of its
variants only represents another route of admin-
istration of an enema. In addition, there is no evi-
dence to believe that an enema given in an
antegrade fashion is more efficient than when it is
given through the rectum. The operation is the
easy part; the hard work and time-consuming part
is the bowel management.
When we reoperate on these patients, we have
been amazed to see that the 5-0 silk sutures that
we placed when plicating the cecum around the
appendix simply are floating in the peritoneum
and are no longer holding the cecum.
We had two cases of catastrophic complica-
tions in which the neo-appendix necrosed at its
tip and the patient leaked stool into the peritoneal
cavity, requiring an emergency reoperation. We
therefore emphasize the importance of watching
the blood supply at the tip of the neo-appendix
during the performance of this operation.
A couple of patients came to the clinic com-
plaining of the fact that they had some difficulty
passing the feeding tube through the orifice in
the umbilicus, but the apparent obstruction was
not located at the skin level in the umbilicus but
rather 3 or 4 cm deeper. We learned that a nice
way to overcome this problem is to pass a baby
cystoscope under anesthesia in the operating
room. By doing that, we found that there was
not a real obstruction but that there was a kink
of the appendix interfering with the passing of
the catheter. Once we identify the direction of
the kink with the cystoscope, we teach the fam-
ily to pass a coudé type of catheter directing the
curve of the tip of the catheter in the right direc-
tion in order to access the colon through the ori-
fice. This has been highly successful. This
troubleshooting maneuver can also be done with
the help of fluoroscopy in an interventional radi-
ology suite (brief comment on page 3).
We emphasize to the family the importance
of lubricating the feeding tube as much as
possible prior to the introduction through the
umbilicus. A school-aged child, who wants to go
on playing, may be in a hurry to pass the catheter,
and the fact the he/she has no sensation makes
References
Fig. 21.6 Diagram
showing the technique
used to elongate a short
appendix
him/her pass the catheter too quickly and rather
roughly. Every time he/she passes the catheter
that way, he/she is injuring the mucosa of the
appendix and the healing of that injury eventually
may create a stricture. Occasionally the surgeon
may find that the patient has a very short appen-
dix. Figure 21.6 illustrates a surgical technique
designed to elongate the appendix.
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3. Roberts JP, Moon S, Malone PS (1995) Treatment of
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 Reoperations
22.1 Introduction
Anorectal malformations are represented by a
wide spectrum of defects. On the good side of
the spectrum, that includes, malformations that
can be repaired with a relatively easy technique
and can obtain excellent functional results, mak-
ing the patient basically a normal individual that
can enjoy a normal life. On the other hand, on the
“bad side” of the spectrum, one can find complex
defects, associated to very significant anatomic
deficiencies that make the functional progno-
sis rather somber. In these serious and complex
defects, it is almost impossible to restore nor-
mal bowel function, urinary function, or sex-
ual function. Many of these patients are born
without the necessary nerves and muscles that
represent the mechanism of bowel and urinary
control. In the middle of these two extremes of
the spectrum, there are many types of malforma-
tion with variable anatomic setups and different
prognoses. We look forward to the design and/
or discovery of therapeutic methods that allow
obtaining better functional results for patients
that are currently born in the “bad side” of the
spectrum.
However, it must be considered unacceptable
to see a patient born with a malformation consid-
ered benign, in other words, in the “good side” of
the spectrum to receive a technically deficient
surgical procedure that destroys important struc-
tures and mechanisms of bowel and urinary con-
trol and ends up with a patient that belongs to the
A. Peña, A. Bischoff, Surgical Treatment of Colorectal Problems in Children,
DOI 10.1007/978-3-319-14989-9_22, © Springer International Publishing Switzerland 2015
22
“bad side” of the spectrum. This is preventable
and totally unacceptable. Unfortunately, it is
something that happens more often than desired.
We do not know if there is a problem related
with our perception, the fact that we feel that
more morbidity occurs during the attempt to
repair anorectal malformations than the one that
occurs in other congenital defects. The fact is that
at our center, from a total of 2,032 cases of ano-
rectal malformations operated by the authors over
the previous 30 years, 478 are reoperations. One
hundred and fifty-three of those were done in an
attempt to regain bowel control. Three hundred
and twenty-five were done in cases that underwent
an attempted failed repair at another hospital and
suffered serious complications. We are aware of
the fact that we are a referral center, and therefore
the proportion of cases that we see, that had failed
attempted repairs, is not representative of the pro-
portion seen in the general population. Yet, we
still consider this number extremely high. From
a total of 909 male cases we had operated, 223
are reoperations, 93 of them were performed in an
attempt to improve bowel control, and 130 were
done to repair sequelae from injuries provoked by
previous surgical misadventures. Between 1,123
female cases, 60 were reoperated in an attempt to
regain bowel control and 195 for other reasons.
This takes us into a controversial issue of
“reproducible” versus “non-reproducible” opera-
tions in surgery. We are convinced that some
operations are highly reproducible, such as pylo-
romyotomy, hernia repair, cholecystectomy, and
349
350
other similar procedures. Those operations have
demonstrated the efficiency and efficacy through
many years, performed by all kinds of surgeons.
Unfortunately, we believe that the operations
designed to repair anorectal malformations are
not very reproducible. This means that to repair
anorectal malformations successfully, the sur-
geon must be familiar with an anatomic territory
that was not well known until recently. For that,
he must be open-minded enough to forget many
of the traditional anatomic concepts and become
familiar with the anatomic spectrum seen in this
complex part of the body, in patients with anorec-
tal malformations. In addition, the surgeon in
charge of repairing these defects must be very
meticulous, careful, and delicate. There are, we
are convinced, many surgeons who are very good
to repair certain types of defects, but not for oth-
ers. Many surgeons have a tendency to be in a
hurry and do not have tolerance or patience to
slow down when dealing with delicate tissues
and complex anatomic arrangements. Anorectal
malformation patients are born with a rectum,
sometimes a vagina, and sometimes a urethra
located in rather unusual places. These three
structures are frequently abnormally attached one
to another, and they share common walls without
a plane of dissection. The separation of these
structures is a mandatory step, in order to recon-
struct the anatomy of these unfortunate patients.
The separation of those structures represents a
technical challenge. Until now, the only way to
do it is observing a very meticulous, delicate dis-
section. This mandatory step (the separation of
the structures) has not been facilitated by new
technologic advances, such as laparoscopic
approach or robotic approach. In fact, we have
seen more complications when such separation
has been attempted through a laparoscope. We
are sure that new advances in the technology will
result in finer, most likely digital instruments that
will allow to perform complex reconstructions
with minimally invasive type of procedures.
Professors of pediatric surgery, all over the
world, are responsible for the surgical training of
the young generation of pediatric surgeons that
will be operating on thousands of babies born
with these defects. We look forward to the time
22 Reoperations
when all patients born with “benign” malforma-
tions undergo impeccable operations to repair the
defect and enjoy a normal life.
Perhaps, part of the problem to explain why
these operations are not as reproducible as others
is the fact that the wide spectrum of anatomic
variations found in these patients is not well
known by the majority of pediatric surgeons. In
addition, during the adult general surgical train-
ing of most surgeons, they learn the traditional
anatomic concepts from adult general surgical
textbooks, which are not representative of the
anatomic variations seen in patients with anorec-
tal malformations.
22.2 Reoperations to Improve
Bowel Control
During our early experience with the posterior
sagittal approach [1], we were extremely opti-
mistic and believed that we would be able to
restore the anatomy of many patients that have
been operated with old techniques and suffered
from fecal incontinence. We assumed that since
most of the operations used before 1980 were
performed, at least in part, blindly, one would
expect that the surgeons may have positioned the
rectum in the wrong location and not in the center
of the sphincter mechanism. Based on that, we
thought that by repositioning the rectum within
the limits of the sphincter, the patient may gain
bowel control. Consequently, we accepted to sur-
gically reexplore all patients who underwent a
previous repair and suffered from fecal inconti-
nence. Our initial experience included eight
patients [1]. In these, we found that the most
common mislocation of the rectum was an ante-
rior one (Fig. 22.1). These mislocations could be
total or partial. The second most common
mislocation of the rectum was a posterior one
(Fig. 22.2). In general, the cases of posterior mis-
located rectum were the patients operated at a
time before the Stephens contribution, when sur-
geons believed that the rectum should be pulled
down as close as possible to the sacrum to avoid
damage to the urinary tract. The cases in whom
the rectum was located too anterior, in general,
22.2 Reoperations to Improve Bowel Control 351

a b

Fig. 22.1 Anterior mislocation of the rectum. (a) Diagram. (b) Perineum. (c) MRI. S sphincter, A anal opening

belonged to the era when Dr. Stephens suggested
that the rectum should be pulled down, as close
as possible to the rectum, in order to preserve the
“puborectalis sling” and give the patient the pos-
sibility of bowel control.
A third type of rectal mislocation was a lateral
one that happened to be very unusual (Fig. 22.3).
The operation consists in opening posterior
sagittally (Fig. 22.4). Multiple silk stitches are
placed at the mucocutaneous junction of the anal
opening in order to apply uniform traction to
facilitate this redissection of the rectum. The
incision is performed exactly in the midline,
dividing all sphincter mechanisms found, poste-
rior to the rectal wall. The incision continues
until we identify the posterior rectal wall. Many
times, what we really find is the colon. In other
words, the surgeons who performed the first
352 22 Reoperations

a b

Fig. 22.2 Posterior mislocation of the rectum. (a) Diagram. (b) Perineum. S sphincter, A anal opening. (c) MRI

operation resected the rectum and pulled down
colon from inside the abdomen. We must be pre-
pared to be able to identify whether the patient
has a colon or rectum.
Originally, we were concerned and thought
that this type of reoperation could be
extremely difficult. Actually, it turned out that
the reoperation was easier to perform than the
primary procedures. Since most of these patients
did not suffer from infections, retractions, and
abscesses (catastrophes), the surgical planes and
the anatomic features were easily recognized. In
addition, it was not necessary to separate the
rectum from the urogenital structures, which is,
22.2 Reoperations to Improve Bowel Control 353

a b

Fig. 22.3 Lateral mislocation of the rectum. (a) Diagram. (b) Perineum

Fig. 22.4 Opening

a b
incision. (a) Diagram. (b)
Operative

as we know, the most important challenge in the
treatment of anorectal malformations. Also, there
was no fistula, and finally, there was not a prob-
lem of bowel length, because the bowel was
already connected to the perineum, so it turned
out to be a rather quick and easy procedure.
Once we identify the posterior bowel wall, the
dissection must be extended to the lateral walls of
the bowel and eventually to the distal end at the
skin. The posterior sagittal incision is continued
in a circumferential manner, peripheral to the silk
stitches to mobilize the entire rectum (Fig. 22.5).
Once the rectum has been mobilized, the lim-
its of the sphincter are electrically determined
(Fig. 22.6). Our findings in this type of operation
include patients who had the rectum completely
mislocated and an intact sphincter mechanism. In
those procedures, it was extremely satisfactory
to mobilize the rectum and place it within the
limits of the sphincter. We finished the operation
354
Fig. 22.5 Diagram showing the dissection of the rectum
with the impression that we really benefited the
patient. Other times, to our dismay, we found
either that the patient was born with no sphinc-
ters and the rectum was surrounded by fat tissue
or we found that the sphincter had been destroyed
during the previous operation and the bowel was
surrounded by scar tissue only. In those cases, we
finished the procedure, feeling that we did not
help the child.
The rectum then is relocated and placed within
the limits of the sphincter mechanism. The previ-
ous location of the rectum is obliterated and
repaired with long-term absorbable sutures. The
rectum is anchored to the sphincter mechanism
as in the primary procedures, and the anoplasty is
done with circumferential multiple, fine, long-
term, absorbable sutures (Fig. 22.7).
The first retrospective review of our experience
with this type of operations showed us that five of
our eight cases had a significant improvement in
22 Reoperations
terms of bowel control [1]. In other words, they
recovered voluntary bowel movements. We then
analyzed the characteristics of the group of
patients that improved with the operation and
compared with the group that did not improve and
found that, as expected, those patients who
improved were the ones that had a completely
mislocated rectum, a preserved rectum, and a
good sacrum and were born with a malformation
that belongs to the “good side” of the spectrum.
Based on that experience, we changed the indica-
tions for this type of procedure, and ever since
that time, we only reoperate, with the expectation
to improve bowel control, on those patients that
have a good sacrum (sacral ratio between 0.6 and
0.8), no evidence of tethered cord, and a com-
pletely mislocated rectum with an intact sphinc-
ter; they still have their original rectum (was not
resected), and they were born with a malforma-
tion that we consider a “benign” type. The results
of those reoperations with the new indications
were not as good as we expected; only about 2/3
of our 77 patients improved significantly in terms
of bowel control [2, 3]. Yet, 85 % of them still soil
the underwear significantly.
The review of the history of the reoperations
designed to improve bowel control, in patients
suffering from fecal incontinence, is a demon-
stration of the ingenuity, imagination, and cre-
ativity of surgeons in general. However, the
results have been in general less than optimal.
Those surgeons who believed in the existence
of the puborectalis, soon enough, designed an
operation to recover the “missed puborectalis”
[4, 5]. Others [6–10] believed that they could
improve bowel control by plicating, tightening,
or releasing the levator mechanism from its pos-
terior attachments. These types of procedures
were known as “levatorplasties.”
A few surgeons embraced the idea of using the
posterior sagittal approach to perform a relocation
of the rectum or to perform a “levatorplasty” [11–
16], most of them with encouraging results and
others with bad results [12]. One author described
an “anterior sagittal approach” to reoperate incon-
tinent patients [17]. A careful reading of the article
showed that the procedure was actually a posterior
approach performed in lithotomy position.
22.2 Reoperations to Improve Bowel Control 355

a b

Fig. 22.6 Limits of the sphincter. (a) Diagram. L limits of the sphincter. (b) Operative view of one side of the incision,
showing the limits of the sphincter and the original anterior mislocation of the anus

a b

Fig. 22.7 Anoplasty. (a) Diagram. (b) Perineum

356
Following the original idea of Pickrell [18],
many surgeons tried the “gracilis sling” opera-
tion, with and without electrical stimulation, with
questionable results [19–36]. The gluteus muscle
was also used to create a voluntary sphincter [37–
41] with variable results.
The possibility of using an artificial sphincter,
capable of giving bowel control, has always been
in the mind of surgeons [42–52]. It has been tried
only in adult patients. The morbidity of this pro-
cedure is high, including infection, rectal stric-
ture, and mechanical failure of the device. In
1988, we implanted in pigs Silastic, hydraulic
devices normally used in the urinary tract (unpub-
lished data). We were able to avoid bowel move-
ments when the cuffs were inflated, but we were
not able to produce bowel movements when the
device was deactivated. That experience contrib-
utes to make us believe that we will not be able to
produce bowel control, unless we find the way to
manipulate the rectosigmoid motility.
In 1975, Hakelius from Sweden suggested
that we could produce bowel control with free
autogenous muscle transplantation [53, 54].
Several surgeons followed his idea, with variable
results [55–59].
Surgeons who believed in the existence of the
“internal sphincter” and its importance for bowel
control devised ingenious procedures to create a
structure similar to the “internal sphincter” [60–
63]. The lack of long-term results makes us to
suspect that the results have not been good.
Based on the idea that the contraction of the
sphincter mechanism is a response to electric-like
stimuli, transmitted by a nerve, some surgeons
have been trying different modalities of electrical,
magnetic, or temperature-controlled radiofre-
quency stimulation to produce bowel control. The
reports describe mainly adult patients, and the
results are rather controversial [64–74].
The extreme example of the rather simplistic
and naïve idea of the mechanisms of bowel control
is the use of a tampon-like device [75] or by the
injection of bulking agents in the anus [76, 77].
In summary, we believe that a rational
approach to the problem of fecal incontinence
in children must consist in the regulation of
colonic motility in patients with borderline fecal
22 Reoperations
incontinence. In other words, patients with mild
degree of incontinence may benefit by the use of
medication and/or diet to slow down the colon,
in cases with tendency to diarrhea and the use
of laxatives in those patients who suffer from
constipation.
In some cases with total fecal incontinence
consecutive to a previously repaired anorectal
malformation, we offer a reoperation to those
patients that have the following characteristics:
A. Completely mislocated rectum
B. Have normal sacrum
C. No evidence of tethered cord
D. Were born with good prognosis type of ano-
rectal malformation (perineal fistula, vestibu-
lar fistula, bulbar fistula, and absent fistula)
In all other cases, or those who did not respond
to our procedure, we offer them our bowel man-
agement program (see Chap. 20).
22.3 Reoperations Performed
After Failed Attempted
Repair (Catastrophes) Males
We have done approximately 130 cases of male
patients born with an anorectal malformation,
who underwent an attempted failed repair. These
patients suffered from postoperative acquired
rectal atresia, anorectal stenosis, dehiscence,
retractions, abscess, infections, and persistent,
recurrent, or acquired rectourethral fistulas.
Interestingly, in all these cases, we found that
the common denominator to explain the failed
operation was the lack of a preoperative high-
pressure distal colostogram or a technically
deficient one. The surgeons did not have accu-
rate anatomic information, which resulted in a
deficient surgical technique.
The lack of a high-pressure distal colostogram
frequently induced the surgeons to look for the
rectum in the wrong place, damaging other
important structures, including the urethra, vas
deferens, and seminal vesicles, or provoking
nerve damage that resulted in neurogenic bladder
and/or lack of erections (impotence). The most
common scenario was a patient with anorectal
malformation with a recto-bladder neck fistula or
22.4 Reoperations for Postoperative Recto-urinary Fistula
a very high rectal prostatic fistula that was
approached posterior sagittally without a distal
colostogram. The surgeon went straight deep
through the incision, looking for the rectum that
was not there, and in the process of searching for
the rectum, he injured the structures that we
already mentioned.
Another scenario was a surgeon who entered
posterior sagittally, without a distal colostogram,
found the rectum, separated it from the urinary
tract, and tried to mobilize it down unsuccess-
fully. The surgeon was unaware of the fact that
the patient had a very short piece of bowel from
the mucous fistula of the colostomy to the distal
end or fistula of the rectum (see Chap. 5). The
fact that the patient did not have a distal colosto-
gram did not allow the surgeon to predict that he
would find such anatomic limitation. As a conse-
quence, after persistent, unsuccessful attempts to
mobilize the rectum, the result usually was devas-
cularization of the bowel. The surgeons had to go
into the abdomen, resect that damaged piece of
bowel, and take the proximal colostomy down.
This had important negative repercussions for the
patient in terms of bowel control, because we
have evidence that when the original rectum is
resected, the chances for the patient to have
bowel control are significantly decreased.
Other cases had an unnecessarily extensive
operation for a rather benign defect. This happened
when a patient with a benign malformation (such
as perineal fistula) underwent a non-indicated
colostomy and subsequently a technically defi-
cient distal colostogram. The study was done
without applying the necessary hydrostatic pres-
sure in order to distend the most distal part of the
bowel, which we know is surrounded by voluntary
sphincter mechanism (see Chap. 6, Sect. 6.6). The
technically deficient distal colostogram showed
an image that gave the wrong impression; the sur-
geon and the radiologists believed that the patient
was born with “high imperforate anus.” Actually
the patient had a rectum located much lower and
could be easily reached with an incision from
below (posterior sagittally). Rather than that, the
surgeon went ahead and performed a laparotomy
or laparoscopy, which was not indicated. Trying to
separate the rectum from the urinary tract, through
357
the abdomen, with a laparotomy or laparoscopy
in a case of a very low-lying rectum is a very dif-
ficult task, and occasionally the patients suffered
a urethral damage or were left with a piece of
rectum attached to the urethra (posterior urethral
diverticulum).
22.4 Reoperations
for Postoperative Recto-
urinary Fistula
Fifty-two cases came to us, with a fistula that
connected the rectum to the urinary tract, with a
history of a previous failed attempted repair. This
condition has been reported in the past [78–87].
We classified those fistulas into three categories:
22.4.1 Recurrent Fistula (17 Cases)
In these cases, the surgeon recognized the
presence of the fistula, separated the rectum
from the urinary tract, closed the fistula during
the operation, and then repaired the anorectal
malformation, and yet the patient developed
a recurrent fistula (Fig. 22.8a, b). A careful
and detailed reading of the operative report
of those cases, as well as our findings in the
reoperations, leads us to believe that the main
problem in these type of cases was the fact
that during the separation of the rectum from
the urinary tract, the anterior rectal wall was
damaged more than necessary, as well as the
posterior wall of the urethra next to the fistula
site. In addition, the rectum was not mobilized
enough, and most likely an anterior rectal wall,
already damaged, was left located in front of a
sutured-damaged urethra. As we know, leaving
sutures, in front of sutures, represent the ideal
situation for the recurrence of a fistula.
Our reoperation consisted of a posterior sagit-
tal approach, separation of the rectum from the
urinary tract, which was more difficult than the
original procedure and then performed enough
mobilization of the rectum, so as to be sure that
we left a normal rectal wall in front of a urethral
suture.
358 22 Reoperations

1 2

Fig. 22.8 Recurrent fistula. (a) Diagram. (1) of urethral sutures. (3) Recurrent fistula. (b) Intraoperative
Preoperative. (2) Postoperative. The rectum was not picture showing the recurrent fistula
mobilized enough; sutures of the rectum were left in front
22.4 Reoperations for Postoperative Recto-urinary Fistula 359

1
2

Fig. 22.9 Persistent fistula. (a) Diagram. (1) Preoperative. (2) Postoperative – the rectum was pulled down, and the
fistula was left intact. (b) Intraoperative – picture taken during the posterior sagittal approach. R rectum, F fistula

22.4.2 Persistent Rectourethral
Fistula (24 Cases)
This occurs when the patient was born with a rec-
tourethral fistula, most of the time located in the
lowest part of the posterior urethra (bulbar)
(Fig. 22.9a, b). In these types of cases, the lowest
part of the rectum bulges down and is seen in the
radiologic diagnostic studies as “low” malforma-
tion. However, the radiologist did not apply
enough hydrostatic pressure to demonstrate the
fistula. As a consequence, the surgeon believes
that he is dealing with a low-lying rectum with-
out a fistula that he can approach from below.
360
a b
Fig. 22.10 Acquired fistula. (a) Foley catheter passing
through the penis and coming out through the anus. (b)
Cystogram of a patient born with a perineal fistula, oper-
Consequently, he enters through a posterior sagit-
tal or perineal approach, finds the rectum rela-
tively easily, mobilizes only the necessary part of
the rectum in order to create an anus, and finishes
the operation leaving the rectourethral fistula
untouched and intact (Fig. 22.9).
The reoperation in this type of case is straight-
forward. First, we go posterior sagittally, open
the rectum like we do in a primary procedure, and
deal with the fistula exactly in the same way as
we do in a primary procedure. Again, we must be
certain to leave a completely normal rectal wall
in front of the urethral fistula site.
22.4.3 Acquired Fistula (9 Cases)
In these cases, the patients were born with an
anorectal malformation, without a fistula,
and underwent an operation, and after the
22 Reoperations
ated without a Foley catheter. The urethra was completely
transected. b bladder, u urethral blind end, p proximal ure-
thral end pulled down together with the rectum
procedure, they passed urine through the
rectum and stool through the urethra!
Obviously, the fistula was created by the sur-
geon. The most common scenario is the one
of a patient who was born with a perineal fis-
tula and underwent an operation; the surgeon
did not use a Foley catheter in the urethra and
damaged or divided inadvertently the urethra
during the mobilization of the anterior rectal
wall (Fig. 22.10).
The operation in these cases consists of sep-
arating the rectum from the perineum and
approach the urethra to close the fistula. Then
mobilize the rectum again to be sure to leave a
normal rectal wall in front of the urethra. These
types of patients frequently have a complete
section of the urethra and require a resection of
the stricture and reanastomosis, which can be
done comfortably posterior sagittally with
good results.
22.6 Acquired Rectal Atresia or Stenosis (83 Cases)
Fig. 22.11 Posterior urethral diverticulum – operative
view of a posterior sagittal approach. R rectum, D diver-
ticulum, F lacrimal probe introduced in the fistula
22.5 Posterior Urethral
Diverticulum (32 Cases)
We use this term to refer to patients who under-
went a repair of an anorectal malformation and
were left with a piece of rectum attached to the
urethra (Fig. 22.11) [83–88]. That piece of rec-
tum behaves like a diverticulum (see Chap. 23).
It has negative implications, including urinary
pseudoincontinence, formation of stones, uri-
nary tract infections, and orchiepididymitis, and
in one particular case, the patient developed an
adenocarcinoma at the junction of the piece of
the rectum with the urethra.
The great majority of patients, who presented
to us with this condition, originally were born
with a rectourethral bulbar fistula. Most of them
were adolescents who underwent abdominoperi-
neal operations for the repair of a rectourethral
bulbar fistula. It is easy to understand how diffi-
cult it could be for a surgeon working through the
abdomen to reach the lowest end of the rectum in a
case of rectourethral bulbar fistula. Consequently,
the surgeons tried to avoid damage to the uri-
nary tract and decided to amputate the rectum
at a “convenient” location, leaving a significant
piece of bowel still attached to the urethra and
then continue with the pull-through (Fig. 22.11).
This happened very often in the old times, when
surgeons divided anorectal malformations into
“high” and “low.” For those malformations that
361
they considered high, routinely they performed
an abdominoperineal operation. In those years,
the distinction between the bulbar, prostatic, and
bladder neck was not recognized. An abdomino-
perineal operation for a recto-bladder neck fistula
turned out to be a relatively easy procedure, and
the separation of the rectum from the bladder
neck was easy, whereas a case with a rectourethral
bulbar fistula could represent a serious challenge,
and that is when these types of sequelae occurred.
In the recent years, we have been seeing again
more cases of posterior urethral diverticula con-
secutive to a laparoscopic approach of a recto-
urethral bulbar fistula (Animation 13.2) [85–88].
We consider contraindicated to approach laparo-
scopically a case with a rectourethral bulbar fis-
tula. One of the reasons for that is to avoid these
undesirable sequelae.
The surgical repair of this condition consists
of performing a posterior sagittal approach,
mobilizing the rectum, identifying the posterior
urethral diverticulum to open it posteriorly, iden-
tifying the fistula, and dealing with it exactly in
the same way as described for a primary case of
rectourethral bulbar fistula.
22.6 Acquired Rectal Atresia
or Stenosis (83 Cases)
These are cases with a history of a previous
attempted repair, and subsequently disappear-
ance of the anal opening or developing a very
severe stricture. This means that the patient
actually suffered from a complete dehiscence and
retraction of the distal rectum.
Reading the operative reports, in these types
of cases, we found that most likely the surgeon
did not mobilize the rectum enough to perform
a non-tense bowel-skin anastomosis. In other
cases, during the mobilization of the rectum, the
surgeon damaged the wall of the bowel, interfer-
ing with its intramural blood supply, which pro-
duced ischemia or duskiness of the distal rectum.
The combination of poor blood supply with a
tense anastomosis explains most of these com-
plications. In other cases, the surgeons obviously
tried to dissect the rectum in the wrong plane.
362
When the rectum is dissected and mobilized, we
must be sure that we remove all the fat tissue that
surrounds it, including the extrinsic blood sup-
ply, but being sure not to injure the bowel wall
itself, in order to preserve the intramural blood
supply. Another important aspect of the success-
ful creation of an anus is to be sure that all of
the sutures of the anoplasty are performed under
equal tension. The anastomosis should not be
under tension, yet cannot be too loose, because
then the patient will be more prone to suffer
from prolapse. The sutures in the anoplasty must
include full thickness of the bowel and not only
the mucosa. Another reason why patients may
have retraction and dehiscence is because of a
limitation done by the colostomy that was origi-
nally created too distally. Again, the surgeon did
not have a good preoperative distal colostogram
and did not recognize this anatomic limitation
prior to the operation.
The reoperation in these patients includes a
posterior sagittal approach to find the rectum and
to mobilize it adequately down to the perineum.
Sometimes this is feasible; sometimes it is not.
Depending on the type of complication that
patients suffered from postoperatively, the sur-
geon who reoperates may find different degrees
of technical difficulties. The patient who suffered
from dehiscence, infection, retraction, abscesses,
and fistula generates an enormous amount of
fibrous tissue, creating what we surgeons call
“frozen pelvis” or “cement type” of pelvis. Under
those circumstances, the surgeon would have to
struggle in an “ocean” of fibrosis to find the rec-
tum and to mobilize it adequately.
22.7 Presacral Masses
There is a specific group of patients born with a
perineal fistula who underwent an anoplasty fol-
lowed by simple anal dilatations at another insti-
tution and came to us, suffering from severe
stricture, megacolon, constipation, and fecal
impaction. A meticulous study of the patient
includes an anterior-posterior view of the sacrum
that shows a sacral defect. This indicates that the
patient was born with a presacral mass that was
22 Reoperations
not detected by the previous surgeons. In those
cases, the narrow portion of the rectum cannot be
dilated using a conventional anal dilatation pro-
gram. The adequate treatment includes the resec-
tion of the mass and mobilization of the
normal-looking rectum above the narrow area.
Pediatric surgeons must remember that the
association of anorectal malformation and presa-
cral mass occurs more commonly in patients with
perineal fistulas (see Chap. 8). The key for the
diagnosis is the AP film of the sacrum. That may
show a specific defect that is always associated
with a presacral mass. These patients, in addition,
need an MRI of the lower spine, to rule out the
presence of tethered cord and/or an anterior
meningocele. If the surgeon has no experience
with this kind of case, he/she must work together
with a pediatric neurosurgeon familiar with this
condition. He/she must be prepared to find a
communication with the dural space and leakage
of cerebrospinal fluid.
When we reoperate patients with anorectal
malformations even in those cases that were born
with what we called “benign malformations,” we
try to be very clear with the parents and explain
that we cannot guarantee the recovery of bowel
function and bowel control. There are many fac-
tors and events that occurred during the original
operation that does not allow us to predict the
functional prognosis of the patient. However, the
results of reoperations in patients with failed
attempted repairs, catastrophes, are in general
very rewarding [3].
22.8 Reoperations in Female
Patients
We have done 255 reoperations in female
patients. Sixty of those were performed to try to
improve bowel control. The results were not dif-
ferent from those obtained in male patients. One
hundred and ninety-five patients underwent reop-
erations to correct sequelae from failed attempted
repairs done at other institutions; ninety-seven of
them were cloacas (see Chap. 16). Sixty-four of
them were rectovestibular malformations (see
Chap. 15).
22.9 Prolapse
Fig. 22.12 Postoperative rectal prolapse
22.9 Prolapse
Rectal prolapse occurs frequently after the
repair of anorectal malformations [89–93]. We
believe it is the result of a lack of fixation of
the rectum to the sphincter mechanism, as well
as an absent or poor sphincter mechanism. It is
more frequently seen during the last 10 years
in cases repaired laparoscopically (see Chap.
13). A potential disadvantage of the laparo-
scopic approach is the fact that the rectum is
not anchored to the neighbor sphincter
mechanism.
The prolapse is exacerbated when the patients
suffer from constipation and spend long periods
of time, pushing, in the toilet.
We reoperate those cases with a prolapse that
we feel interferes with the quality of life of the
patient, for example, when the prolapse is so
prominent (Fig. 22.12) that it bleeds if the patient
rides his bicycle. It also produces mucus that
becomes evident through the dress, provoking
embarrassing moments to the patient. We do not
repair minor prolapses (Fig. 22.13).
363
Fig. 22.13 Minor rectal prolapse
When we repair prolapses, we explain to the
parents that we cannot guarantee that the pro-
lapse will not come back since we cannot cure
the origin of the prolapse. We have seen recur-
rences in our cases, but never as severe as the
original prolapse.
When the prolapse is detected at the time of
the colostomy closure, we offer the family to
cancel the colostomy closure and rather take care
of the prolapse. Under those circumstances, the
operation does not require bowel preparation
and is painless, and the patient is discharged 24
or 48 h after the operation. When we decide to
repair the prolapse after the colostomy has been
closed, it is necessary to admit the patient the day
before and administer GoLYTELY (see Chap. 7).
The procedure takes approximately 1 h, but we
keep the patient fasting for 5–7 days, receiving
parental nutrition.
The repair is performed in the prone position.
Figure 22.14a–f shows the different stages of the
364
Fig. 22.14 Surgical repair of prolapse. (a) Diagram
showing the multiple silk sutures placed at the mucocuta-
neous junction and the beginning of the circumferential
incision. (b) Operative view of the rectum fully dissected.
(c) Diagram showing a fully dissected rectum and the
placement of the deep layer of sutures and dividing the
22 Reoperations
prolapsed rectum in the midline. (d) Operative view of the
same stage of operation. (e) Diagram showing the resec-
tion of the rectum and the superficial layer of sutures of
the anoplasty. (f) Operative view of the same stage of
procedure
22.9 Prolapse 365

Fig. 22.14 (continued)

366
operation. Multiple silk stitches are placed at the
mucocutaneous junction of the anus, in order to
apply uniform traction (Fig. 22.14a). A circum-
ferential incision is done with the needle-tip cau-
tery, peripheral to the silk sutures (Fig. 22.14b)
The dissection proceeds around the rectal wall
until a reasonable length of rectum has been
dissected (Fig. 22.14c). The final goal is to resect
the portion of the rectum that used to prolapse
and reconnect the proximal rectum to the skin,
under mild tension, in order to avoid prolapse and
if possible to invert the suture line (Fig. 22.14d).
The bowel-skin anastomosis is done with two
layers of interrupted, fine, long-term absorbable
sutures (Fig. 22.14e).
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 Urologic Problems in Anorectal
Malformations
23.1 Introduction
Urogenital problems in patients with anorectal
malformation represent a very important source
of morbidity; in fact, it is more likely for a child
with an anorectal malformation to die from a uro-
logic problem, rather than from gastrointestinal
problems or any other associated defect. Pediatric
surgeons must keep in mind that the patient with
an anorectal malformation represents a potential
life-threatening problem originated in the uro-
genital tract, mainly kidney damage and eventu-
ally kidney failure.
The association of urogenital defects with
anorectal malformations has been recognized for
a long time. The incidence has been estimated to
vary from 25 to 85 % [1–17]. The majority of
reports inform of an incidence around 30–50 %.
We believe that the difference found between dif-
ferent authors is a consequence of the thorough-
ness of the search. A high index of suspicion, in
an institution with high technologic support,
mostly likely will find more urologic problems.
Table 23.1 shows the frequency of associ-
ated urologic defects for each type of anorectal
malformation. The anorectal defects are listed
according to the degree of complexity, being the
simplest the perineal fistulas and in the opposite
Electronic supplementary material Supplementary
material is available in the online version of this chapter at
10.1007/978-3-319-14989-9_23.
A. Peña, A. Bischoff, Surgical Treatment of Colorectal Problems in Children,
DOI 10.1007/978-3-319-14989-9_23, © Springer International Publishing Switzerland 2015
23
extreme the cloacal exstrophy and other complex
malformations. It can be very easily seen that the
frequency of association increases directly pro-
portional to the height of the defect and the com-
plexity of the malformation. This information is
very valuable to increase the index of suspicion
of the clinician and allow an early detection of
the urologic problems, particularly those that
may produce renal damage and eventually kid-
ney failure.
It is a relatively common experience to see a
patient that was born with an anorectal malfor-
mation that was repaired early in life, and the
parents were not alerted about the fact that the
patient had a urologic abnormality, and years
later, the parents have the very unpleasant expe-
rience of learning that the child needs a kidney
transplant. In some cases, the patient is born with
the kidneys already severely damaged, and the
role of the physician should be directed to avoid
worsening of that problem. On the other hand,
some patients are born with normal kidneys, but
the lack of care of a problem such as vesicoure-
teral reflux and a poor functional bladder even-
tually may damage the kidneys, and the patient
may require a kidney transplant or even die. This
last situation is mostly preventable and should
not occur. All this means that it is very impor-
tant to suspect, diagnose, and treat adequately
all those urologic problems associated with ano-
rectal malformations. In addition, patients with
anorectal malformations should be followed on a
long-term basis, monitoring their kidney function
371
372 23 Urologic Problems in Anorectal Malformations

Table 23.1 Frequency of association of urologic defects for each type of anorectal malformation
Females
Absent kidney (%) Vesicoureteral reflux (%) Hydronephrosis (%)
Cloaca CC >3 cm 26 40 45
Cloacal exstrophy 26 21 9
Cloaca CC <3 cm 17 21 22
Vestibular 10 13 6
Perineal 4 5 5
Males
Absent kidney Vesicoureteral
(%) reflux (%) Hydronephrosis (%) Hypospadias (%) UDT (%)
Bladder neck 31 29 24 15 18
fistula
Prostatic 16 6 6 6 7
Bulbar 11 13 9 10 5
No fistula 7 7 4 3 10
Perineal 2 3 6 1 2

and the anatomy of the urinary tract, because we
know that many of them have anatomic or func-
tional problems with a tendency to deteriorate.
23.2 Neonatal Approach
If a pediatric surgeon has no experience in the
diagnosis and management of urologic problems,
he should work together with a pediatric urolo-
gist in the initial evaluation of a patient with ano-
rectal malformation.
All newborn babies with anorectal malforma-
tions should have a kidney ultrasound, within the
first 24 h of life and before any surgical interven-
tion. Female babies, particularly those with clo-
aca (single perineal orifice), must have, in
addition, a pelvic ultrasound to rule out the pres-
ence of a hydrocolpos.
We believe that a male baby with a normal
kidney ultrasound, that is passing urine satis-
factorily, has a normal sacrum, and no evidence
of tethered cord can be operated on without any
further urologic tests. On the other hand, if the
baby has hydronephrosis, he will require further
urologic studies, including a voiding cystoure-
throgram. We do not believe that the voiding
cystourethrogram should be done routinely, in
all male babies with anorectal malformations,
because in our experience, it is not a good study
to diagnose the location of the recto-urinary fis-
tula. In addition, the urethral catheterization in
babies with rectourethral fistulas sometimes is
difficult, because the patients frequently have
kinks or narrowing of the urethra, particularly at
the junction between the rectum and the urethra.
If the baby has tethered cord and/or a very
abnormal sacrum (sacral ratio less than 0.4), he is
at risk of suffering from deterioration of the blad-
der function, which eventually may affect the
kidneys and therefore is considered a high-risk
patient from the urologic point of view, even if
the initial kidney ultrasound is normal.
The female baby with a cloaca represents a
particularly difficult challenge (see Chap. 16).
Thirty percent of patients born with a cloaca have
a hydrocolpos that may produce an extrinsic
compression of the ureterovesical junction,
which results in megaureter and hydronephrosis.
As we mentioned in the Chap. 16, before consid-
ering drainage of the urinary tract with a nephros-
tomy or ureterostomy, the surgeon must drain the
hydrocolpos and reevaluate the megaureter and
hydronephrosis. After the hydrocolpos has been
drained, the megaureter will improve or disap-
pear, as well as the hydronephrosis. If the hydro-
nephrosis or the megaureter persists, the baby
must be subjected to further evaluation to con-
sider the possibility of making a ureterostomy,
nephrostomy, or vesicostomy. We have seen
23.4 Most Common Urologic Abnormalities in Male Patients with Anorectal Malformations
many babies with cloacas, hydronephrosis, and
megaureter, subjected to unnecessary ureterosto-
mies, nephrostomies, and/or vesicostomies, in a
futile attempt to improve the hydronephrosis,
without draining the hydrocolpos (see Chap. 16).
There is a small group of patients with a cloaca
that are born with a very narrow (almost atresia) com-
mon channel which interferes with the emptying of
the bladder. After draining the hydrocolpos, an ultra-
sound may show that the bladder is still full and does
not empty well. An attempt to catheterize the bladder
may be extremely difficult; in those cases it is justi-
fied to do a cystostomy or a vesicostomy.
Another possible indication for a vesicostomy
is the case of a baby with demonstrated massive
vesicoureteral reflux, megaureter, hydronephro-
sis, and important risk factors for poor bladder
function, such as poor sacrum and tethered cord.
The newborn baby should not be taken to the
operating room until the surgeon has ruled out
and treated important associated urologic prob-
lems. After this, the baby is taken to the operating
room to perform either a colostomy or a primary
repair of the anorectal malformation.
23.3 The Importance
of the Colostomy Type
from the Urologic Point
of View
The location and type of colostomy may have
important urologic implications. It is important
for the neonatologist as well as the urologist to
remember this (see Chap. 5).
The colostomy that we recommend is one cre-
ated at the end of the descending colon and proxi-
mal to the sigmoid loop. The purpose of that
specific location is dual, first, to avoid prolapse of
the proximal stoma, since the descending colon is
fixed, and, second, to leave enough distal bowel
(loop of the sigmoid) to allow a successful pull-
through. In addition, we specifically recommend to
make the distal stoma (mucous fistula) very narrow
in order to avoid prolapse, but still, we believe that
is important to keep it open to be able to irrigate the
distal bowel and to perform radiologic studies,
most specifically, a high-pressure distal colosto-
373
gram. We strongly recommend separating both
stomas in order to be able to put a colostomy bag,
including only the proximal stoma and not the dis-
tal one. When both stomas are too close together,
the mother is forced to use a single bag to cover
both stomas, which may produce fecal contamina-
tion of the distal bowel and consequently of the
urinary tract. Loop colostomies, therefore, from
our point of view, are formally contraindicated in
anorectal malformations, because they represent a
source of fecal contamination of the urinary tract.
During the creation of the colostomy, we spe-
cifically recommend the surgeons to wash the
bowel distal to the mucous fistula until it is com-
pletely collapsed and cleaned of meconium. If a
baby receives a colostomy and after the procedure
is not doing well and suffers from frequent urinary
tract infections and from episodes of acidosis, it is
extremely important for the physician to look at the
type of colostomy that the baby has, looking for an
explanation for that behavior. A loop colostomy
producing frequent urinary tract infections would
require an operation to separate the stomas. If the
surgeon did not wash the distal bowel, sometimes
the accumulation of meconium, plus mucus pass-
ing through the fistula, produces urinary tract infec-
tions. Finally, when the colostomy was opened in
the transverse colon (which we consider contrain-
dicated), the meconium may pass into the urinary
tract, but also urine passes from the urinary tract
into the colon where it is absorbed, producing
hyperchloremic acidosis that may interfere with the
growth and development of the newborn baby.
When a pediatric urologist is called to see a baby
with this kind of problem, he should look at the
type of colostomy that the baby has.
23.4 Most Common Urologic
Abnormalities in Male
Patients with Anorectal
Malformations
23.4.1 Absent Kidney
The most common congenital urologic defect
associated to anorectal malformations is an
absent, dysplastic, multicystic, or nonfunctional
374 23 Urologic Problems in Anorectal Malformations

a b

Fig. 23.1 Absent kidney (multicystic, dysplastic). (a) Ultrasound showing an image of a multicystic kidney. (b) Renal
scan showing an absent kidney. (c) MRI showing a horseshoe kidney

kidney. In our series, 106 out of our 909 male
patients and 165 out of 1,123 females were born
with this problem.
Figure 23.1 shows the images of a patient with
an absent kidney. Figure 23.1a shows the ultrasound
and Fig. 23.1b shows a renal scan. This is by far the
most common type of anatomic abnormality seen in
anorectal malformations. The kidney may be totally
absent or rather represented by a nonfunctional
multicystic and/or dysplastic kidney remnant.
Figure 23.2a shows the images of an ultra-
sound in a patient with hydronephrosis, and
Fig. 23.2b shows a case with hydronephrosis
and megaureter in a patient with anorectal mal-
formation. This problem was present in 198 out
of 1,123 female patients and 70 out of 909 male
patients. The presence of a megaureter in patients
with anorectal malformations, most of the time,
represents the existence of severe vesicoureteral
reflux, since the cases of ureterovesical obstruc-
tion are very unusual. Of course, we must remem-
ber that cloaca patients may have an extrinsic
compression of the ureterovesical junction that is
released when a dilated vagina is drained.
Figure 23.3 shows the images of a void-
ing cystourethrogram of a baby suffering from
vesicoureteral reflux (Fig. 23.3a unilateral and
Fig. 23.3b bilateral). In our series, this problem
was present in 207 out of 1,123 females and 133
out of 909 males. The incidence of reflux in cases
of anorectal malformations has been estimated to
vary from 30 to 60 % [18–21]. In our series it
occurred between 14 and 18 % of the cases.
The presence of severe megaureter and vesi-
coureteral reflux in a newborn baby is an omi-
nous sign. That baby will require special care to
protect the kidneys, particularly in cases with real
or potential neurogenic bladder, such as patients
with severe sacral deformity and/or tethered
cord. The best way to protect the kidneys in those
babies is with a temporary vesicostomy, since
trying to perform a ureteral reimplantation, with
ureteral tapering, in an infant with a well-known
poor functional bladder most likely will not suc-
ceed and may put the marginal renal function of
the patient at risk.
There is one particular group of male
patients born with a recto-bladder neck fistula.
23.4 Most Common Urologic Abnormalities in Male Patients with Anorectal Malformations 375

Fig. 23.2 Hydronephrosis (ultrasound image). (a) Bilateral hydronephrosis. (b) Hydronephrosis and megaureter

a b

Fig. 23.3 Vesicoureteral reflux (VCUG). (a) Unilateral. (b) Bilateral

376
Fig. 23.4 VCUG in a patient with a single kidney with
hydronephrosis and reflux
Twenty-five patients of a group of 111 patients
born with a bladder neck fistula had an absent
kidney, megaureter, and hydronephrosis in the
opposite site (Fig. 23.4). These patients are at
high risk to end up with a kidney transplant.
The fact that a baby is born with hydrone-
phrosis means that those kidneys had been
suffering in utero and at birth already have a
significant degree of damage. We have seen
patients with hydronephrosis at birth and with
a normal creatinine; they maintain the normal
level of serum creatinine during the first years
of life, but when they have a growth spurt,
sometimes the creatinine suddenly elevates,
indicating that most likely the patient was born
with a limited kidney function, which became
obvious when the patient grows significantly.
Those cases with bladder neck fistula, single
kidney, megaureter, and hydronephrosis should
be considered high-risk patients.
The management of vesicoureteral reflux in
patients with anorectal malformations deserves a
special comment. The general principles of man-
agement of this disorder, in patients without ano-
rectal malformations, should not be extrapolated
and applied for cases with anorectal malformation
for the following reasons:
23 Urologic Problems in Anorectal Malformations
A. Patients with anorectal malformations
frequently suffer from a spectrum of sacral
abnormalities that have a demonstrated effect
on bladder function.
B. At least 25 % of patients with ARM suffer
from tethered cord, which also has a signifi-
cant negative effect on bladder function.
C. The repair of an anorectal malformation,
when done inappropriately, produces differ-
ent degrees of neurogenic bladder.
D. The repair of complex cloacas includes a sig-
nificant dissection between the vagina(s), the
bladder neck, the trigone, and the urethra. All
these have a very significant effect on the
bladder function. In addition, the scar pro-
duced by that dissection may represent a seri-
ous challenge to perform a reimplantation.
It is becoming obvious that the bladder function
is a fundamental factor to take into consideration
when making a decision about a ureteral reimplan-
tation. A ureteral reimplantation, done in a patient
with neurogenic bladder and incapacity to empty,
most likely will fail. The decision to reimplant a
ureter must not be taken without a previous urody-
namic evaluation. This is particularly true in patients
with megaureter and hydronephrosis. The ureteral
reimplantation must be performed being sure that
the patient is able to empty the bladder spontane-
ously or with intermittent catheterization through
the urethra [22] or through an appendicovesicos-
tomy tract (Mitrofanoff principle) [23, 24].
Because of all these reasons, in general, we try
to avoid a reimplantation in a newborn with reflux,
megaureter, and hydronephrosis. A vesicostomy is
a better option, under those circumstances; it pro-
tects the upper tracts and gives us time to reevalu-
ate the case and make an appropriate decision.
23.4.2 Urethral Problems
Figure 23.5 shows an image of male urethrogram
of a patient born with an anorectal malformation
and a recto-urinary fistula. The kink shown in the
study most likely corresponds to the location of the
fistula. However, we believe that the VCUG is not
the ideal study to determine the location of the fis-
tula. Some patients suffer from a urethral stricture
23.5 Bifid Scrotum 377

Fig. 23.5 Radiologic image of a kinked urethra. The nar-

rowing and/or the kink is located at the junction of the
rectum and the urethra (fistula site)

or a kink of the urethra at the same location of the

junction of the rectum (fistula) (Fig. 23.6). The
posterior sagittal approach represents an ideal way
to repair urethral strictures. This can be done at the
same time of the repair of the anorectal malforma-
tion. We have operated on our patients with this
kind of problem and found relatively simple the
urethral repair. The results have been good.
Fig. 23.6 Distal colostogram showing a urethral stricture
Figure 23.7 shows operative pictures of a repair of
at the junction of the rectum with the urethra
a urethral stricture located at the same location of
the fistula. A urethroplasty was performed using
a flap of rectal tissue (Figs. 23.7a–d). from a urethral stricture, then there is no way to
It is relatively common to see cases that have pass the catheter into the bladder. Under those
a very acute kink of the urethra at the same loca- circumstances we allow the catheter to go into
tion where the rectum joins the urinary tract. the rectum and approach the patient posterior
When the doctors, radiologists, or surgeons try to sagittally; when the rectum is opened, one can
pass a catheter, the catheter stops at that place or see the catheter inside, and then under direct
rather goes into the rectum. To try to overcome vision, one can redirect the catheter into the blad-
this obstacle, it is recommended to use a coude der. If the surgeon finds that there is a urethral
type of catheter (curved tip) to get access to narrowing, we have been using a little piece of
the bladder. Sometimes, however, even with the the local rectal tissue to create a small flap to
use of this catheter, it is not possible to catheter- enlarge the area of the stricture of the urethra
ize the bladder. In such cases it is necessary to with good results (Fig. 23.7).
pass the cystoscope using a wire in order to
catheterize the bladder. There is a maneuver that
proved to be useful to pass a Foley catheter under 23.5 Bifid Scrotum
those circumstances. A lacrimal probe is inserted
in the hole of the catheter, and the lacrimal probe In our series of 909 cases of male patients with
is curved at its tip and can be externally directed anorectal malformation, we found 102 patients
(see Chap. 9). If the patient, in addition, suffers suffering from a bifid scrotum (Fig. 23.8). Most
378
a b
d e
Fig. 23.7 Operative findings in a patient with urethral
stricture. The catheter was redirected into the bladder
under direct vision, and the urethral stricture was repaired.
(a) The rectum open, lacrimal probe into upper the ure-
thra, catheter emerging from the bulbar urethra. (b) The
of the cases of bifid scrotum occur in babies who
have anorectal malformations that belong to
the “bad” side of the spectrum, in other words,
patients that have bladder neck or high pros-
tatic fistulas. Therefore, a bifid scrotum should
be considered as a sign of a potential complex
malformation.
Through the years, we learned to repair the
bifid scrotum in a rather simple way, at the same
23 Urologic Problems in Anorectal Malformations
c
upper portion of the urethra has been enlarged with a
plasty. (c) The urethral catheter was passed toward the
bladder. (d) The area of stricture is expanded using rectal
tissue. (e) Reconstruction finished
time that we repair the anorectal malforma-
tion. It is important to recognize that in cases
of bifid scrotum, there is a midline portion of
nonelastic skin that separates both hemiscro-
tums (Fig. 23.9a–e). The skin of the scrotum is
recognized because it has rugae and is elastic.
The skin in between the hemiscrotum lacks that
kind of rugae, is rather smooth, and is nonelastic.
The technique that we use consists of resecting
23.5 Bifid Scrotum
Fig. 23.8 Different types of bifid scrotum
the smooth, nonelastic portion of the skin.
Figure 23.9a shows how we have marked the
limits that separate the smooth, nonelastic skin
from the normal rugae scrotal skin. Figure 23.9b
shows the incision that is made with the needle-
tip cautery. Figure 23.9c shows that the smooth
skin has been resected and the beginning of a
379
two layer suture of the skin edges (Fig. 23.9d).
Both layers sutured with multiple, interrupted,
6-0 long-term absorbable sutures. Figure 23.9e
shows the final aspect of the repair. Later in life,
it is very difficult to know that this patient had
a repair of a bifid scrotum and the parents have
expressed their satisfaction.
380 23 Urologic Problems in Anorectal Malformations

a b

c d

Fig. 23.9 Repair of a bifid scrotum. (a) Marking the limits of the abnormal skin. (b) Incision. (c) Resected non elastic
skin. (d) Two-layer suture. (e) Final aspect

23.6 Hypospadias ately anterior to the anal opening (Fig. 23.10c).

Sixty of our male patients suffered from different
types and severity of hypospadias. It may go from
a subcoronal type (Fig. 23.10a) to an extreme type
in which the urethral orifice is located immedi-
Extreme, severe types of hypospadias are fre-
quently associated to penoscrotal inversion.
The timing for the repair of hypospadias is
something rather controversial. Some pediat-
ric urologists like to repair the hypospadias
23.6 Hypospadias
a b
c d
Fig. 23.10 Hypospadias. (a) Subcoronal. (b) Mid-shaft.
(c) Perineal. (d) Repaired anorectal malformation with
severe hypospadias. The urethra was originally located
before the colostomy is closed, to avoid fecal
contamination during the postoperative period.
Some others prefer to wait until the patient is
older to be able to deal with better tissues and
have more chances of success. From the point
of view of the pediatric surgeon, it is important
to identify the most severe type of hypospadias
(Fig. 23.10c, d) in which the urethral orifice is
381
next to the anal opening. The urethral opening was moved
forward, and the anus was moved back
located immediately anterior to the anal open-
ing. The surgeon must keep in mind that the rec-
tum and urethra share a common wall, usually in
the area of the spongiosum tissue. Those babies
have a strictured anal opening located anterior
to the center of the sphincter, and therefore,
the operation will consist in moving the anus
back and simultaneously to correct the severe
382
chordee. In order to do that, the surgeons have to
work on the common wall between the rectum
and the urethra and move, if possible, the ure-
thra a little forward. The idea is to create a rea-
sonable space between the anal opening and the
urethral opening and move the penis forward,
away from the anal opening. After this repair,
the urologist will take care of the problem of
repairing the rest of the perineal urethra, as well
as the penile urethra. Figure 23.10d shows the
external aspect of a patient that underwent a
repair of an anorectal malformation and separa-
tion of the rectum from the urethra in a severe
perineal hypospadias.
23.7 Ectopic Ureters in Males
This malformation has been reported in cases
without ARM [25]. It seems to be relatively rare.
We believe that it occurs more often in patients
with anorectal malformations.
Twenty-one of our male patients suffer from
some form of ureteral ectopia. In patients with
anorectal malformations, we have seen that the
ureters have a tendency to open ectopically, in the
line that goes from the normal location of the ure-
ter in the trigone toward the bladder neck. It is
relatively common to see patients with a ureteral
orifice located closer to the bladder neck. There
are however cases in whom, definitely, we see the
ureter opening in the bladder neck (Fig. 23.11a).
The opening of the ureter in the bladder neck fre-
quently is associated with some degree of ure-
teral obstruction and megaureter. In addition, the
presence of the ureter in that abnormal location
may interfere with the closure of the bladder
neck, producing a certain degree of bladder neck
incapacity to hold the urine. We have relocated
the ureters out of the bladder neck with success,
recovering urinary control in patients that were
leaking urine.
The most severe type of ureteral ectopia in
males occurs when the ureters are connected to
the posterior urethra (Fig. 23.11b). In these
cases, the patient suffers from severe megaure-
ter, and they frequently have severe damage to
23 Urologic Problems in Anorectal Malformations
the kidney that usually ends up with a nephrec-
tomy. The surgeon should be alerted about the
fact that the megaureter connected to the poste-
rior urethra may give the false intraoperative
impression of the rectum. In one specific case,
the patient was operated in another city without
a distal colostogram, and the surgeon entered
posterior sagittally looking for the rectum,
found a “tubular structure,” separated it from the
posterior urethra, and pulled it down, believing
it was the rectum, and it turned out to be a mega-
ureter. The patient had actually a recto-bladder
neck fistula.
As expected, the frequency of ectopic ureter is
higher in patients with higher and more complex
malformations. If one already made the diagnosis
of an ectopic ureter located in the posterior ure-
thra, the posterior sagittal approach represents a
golden opportunity to identify the megaureter,
separate it from the posterior urethra, close the
posterior urethra, and push the megaureter up
into the pelvis, to be recovered through a small
incision in the groin, where it can be connected to
the skin as a ureterostomy. It will take several
months for us to determine the degree of renal
damage that the kidney has and whether or not
the patient will benefit from a nephrectomy or
a ureteral reimplantation.
23.8 Ectopic Ureters in Females
All that we said about the ectopic ureters in the
trigone and bladder neck is true for female
patients. The ureters also may be ectopically con-
nected to the urethra in a female. Figure 23.12
shows a picture taken during a transpubic
approach of a female patient with a complex mal-
formation. It would be very difficult to separate
the ureter with a different type of approach. We
use the transpubic approach in cases that we
believe would be very difficult to approach in a
different way.
In patients with cloaca, it is also relatively
common to find ureters connected ectopically to
the vagina. Again, most of the time these ureters
belong to severely damaged kidneys.
23.8 Ectopic Ureters in Females 383

B a b

Fig. 23.11 Diagram showing ectopic ureters. (A) Connected to the bladder neck. (B) Connected to the posterior ure-
thra. (a) Voiding cystourethrogram. (b) Diagram
384
Fig. 23.12 Ectopic ureters in the urethra of a female
patient with a complex malformation. Intraoperative pic-
ture taken during a transpubic approach
a b
Fig. 23.13 Ectopic vas deferens. (a) Operative picture V
vas deferens, U ureter, T testicle. (b) Vas deferens con-
nected to a giant seminal vesicle – picture taken during the
23 Urologic Problems in Anorectal Malformations
23.9 Ectopic Vas Deferens
This rare anomaly has been reported in patients
without anorectal malformation [26–28]. The
most common clinical manifestation has been
orchiepididymitis. However, we found more pub-
lications related to this malformation in cases of
anorectal malformations [29–38].
We have demonstrated the presence of ectopic
vas deferens in three cases of our population of
male patients with anorectal malformations. We
suspect that this defect occurs more often, in
patients with ARM, particularly in the group of
patients who suffer from orchiepididymitis. It is
not easy to demonstrate this problem. We were
able to make the diagnosis injecting contrast
material directly into the vas deferens with a
30-gauge needle. The vas deferens may be abnor-
mally connected to the bladder or to the ureter.
Figure 23.13a shows an operative picture of an
ectopic vas deferens, connected to the ureter. The
symptomatology in this patient consisted of
recurrent orchiepididymitis. The treatment for
trans-anorectal resection of the giant seminal vesicle. V
vas deferens, J junction of seminal vesicle to urethra, L
limits of the anus
23.10 Ectopic Verumontanum
this condition should be a vasectomy, to preserve
the endocrine function of the testicle that may be
destroyed after repeated episodes of infection.
Sometimes, the vas deferens is abnormally con-
nected to an extremely dilated giant seminal ves-
icle which is also a source of orchiepididymitis
(Fig. 23.13b).
Orchiepididymitis occurs relatively frequent
in patients with anorectal malformations. Many
patients who underwent a repair of an anorec-
tal malformation at our center were followed by
other colleagues in other cities. Some of those
patients suffered from an episode of “acute scro-
tum” and were surgically explored to rule out a
possible testicular torsion, only to find that the
babies actually suffered from orchiepididymitis.
In fact, we have never seen or heard of a case
of testicular torsion in a patient with ARM. Yet,
we have seen 17 cases of orchiepididymitis. The
etiology of this condition seems to be multifacto-
rial. Most patients have a urinary tract infection
and some degree of neurogenic bladder. Some of
them have an identifiable anatomic problem that
may explain the problem. These include urethral
obstructions and ectopic vas deferens. A patient
with acute scrotum and ARM must be considered
as orchiepididymitis until proven otherwise.
All patients with orchiepididymitis deserve a
full urologic evaluation after the acute episode. In
addition, they must receive prophylactic medica-
tion to try to avoid episodes of urinary tract infec-
tions. The anatomic problem must be treated when
found. In cases of frequent recurrences, a vasec-
tomy of that particular side is indicated, in order to
try to preserve the endocrine function of that tes-
ticles; otherwise, it may be totally destroyed.
23.10 Ectopic Verumontanum
An unreported, unusual, interesting malforma-
tion is the ectopically located verumontanum. We
have four cases suffering from this defect within
the group of patients born with recto-bladder
neck fistulas. We become aware of this condition
because one of our first patients operated from
a bladder neck fistula reached adolescence and
came for consultation, because even when he
385
had erection and orgasm, he did not ejaculate.
Urinalysis after ejaculation demonstrated the
presence of sperm in the urine. This was not
what is called retrograde ejaculation, because by
cystoscopy we documented the presence of the
verumontanum in the trigone (Fig. 23.14). After
that case, we intentionally started looking for the
location of the verumontanum in patients with
bladder neck fistula, and we were able to find
three more cases. This number of cases only rep-
resents 30 % of the patients who underwent an
endoscopy. Most of the patients operated on for
bladder neck fistula have not reached adolescence
yet, so we expect that in the coming years more
of these patients will come looking for help under
these circumstances.
We now follow the routine of performing
cystoscopy in all male patients with anorectal
malformation, during the same anesthesia that
we used for repair of the malformation. This is
particularly important in patients with a bladder
neck type of malformation in which this defect
occurs.
We have been told that these patients may be
able to fertilize their couple by retrieving the
sperm from their urine and artificially inseminate
the future mother. None of our patients have done
that so far.
Fig. 23.14 Ectopic verumontanum – cystoscopic
appearance
386
23.11 Megalourethra
This malformation has been reported in cases
without anorectal malformation [39, 40], as well
as associated to anorectal malformations [41–
47]. The patients that we have seen have a
normal-looking penis (Fig. 23.15a); it is neces-
sary to retract the foreskin to be able to see the
abnormality (Fig. 23.15b). This means that this is
the kind of defect that requires a good index of
suspicion in order to make the diagnosis.
These patients are born with a very large
urethral meatus, followed by a very dilated
penile urethra. From the base of the penis to the
glans, the urethra is extremely wide. However,
from the base of the penis to the bladder, the
urethra is otherwise normal. We have not seen
many of the severe types of megalourethra
with the absence of corpora and a large sac-
cular floppy penis, described in the literature.
Our cases were not associated with hypospa-
dias. The repair of this defect is much easier
than the one in hypospadias, the reason being
that we are dealing with very good tissue and
plenty of urothelium to repair the urethra. With
the foreskin completely retracted back, like in
a circumcision, we open the urethra ventrally
(Fig. 23.15c) and excise the extra tissue of the
megalourethra. The urethra is reconstructed over
a convenient-sized Foley catheter (Fig. 23.15d).
Figure 23.15e shows the final appearance of a
patient with this defect after repair. We have 15
cases, all of them repaired with the same tech-
nique prior to the closure of the colostomy, with
excellent results. These patients never devel-
oped any fistula, and they have normal voiding.
Figure 23.15f, g shows a case of a much larger,
floppy urethra.
23.12 Ureterovesical
and Ureteropelvic
Obstruction
We believe that most likely these two congenital
abnormalities occur in cases of anorectal
malformation with similar frequency as in the
general population. It is extremely unusual to
detect them in cases of anorectal malformations.
23 Urologic Problems in Anorectal Malformations
The treatments of these defects are well described
in the textbooks of urology.
23.13 Neurogenic Bladder
Neurogenic bladder associated to anorectal mal-
formations is receiving significant attention [48–
64]. The subject is not easy to study, considering
the following facts:
A. May be congenital
B. May be acquired
C. May be influenced by the complexity of the
anorectal malformation
D. Is influenced by the sacral defect
E. Is influenced by the tethered cord
Most of the publications include a rather small
number of cases of different malformations with
or without sacral defects or tethered cord. All this
makes the analysis and interpretation of those
publications a rather difficult task.
Most authors, like us, agree that it will be ideal
to do a urodynamic evaluation pre- and postop-
eratively in all cases, documenting the sacral
ratio and the presence or absence of tethered cord
in order to draw valid conclusions.
We believe that most pediatric surgeons and
pediatric urologists are now aware of the rele-
vance of the prevention, early diagnosis, and
management of neurogenic bladder in order to
protect the kidneys.
Intermittent catheterization and bladder aug-
mentation had a definitive positive impact to pro-
tect the kidneys in patients with hypertonic,
neurogenic bladder [65–70]. However, these are
patients who must be followed for life. The blad-
der augmentation has short- and long-term
significant morbidity that must be detected and
treated efficiently [71–78].
So far, in our series 58 patients underwent a
bladder augmentation, 32 of them are performed
by the senior author until 2005, and 26 since that
time are performed by pediatric urologists. Most of
these operations were done in girls with cloacas.
Some patients with anorectal malformations
have manifestations of a neurogenic bladder since
birth (primary). That is understandable when the
patients have a poor sacrum (sacral ratio less than
0.4) and the presence of tethered cord. However, a
23.13 Neurogenic Bladder 387

a b

c d

Fig. 23.15 Megalourethra. (a) External appearance – portion of the megalourethra is divided. (d) The excessive
observe that it looks remarkably normal. (b) External urethral tissue is resected, and the urethra is reconstructed
appearance with the foreskin retracted showing the giant around a Foley catheter. (e) Final appearance. (f) Giant,
meatus. (c) The foreskin divided and retracted. The ventral floppy megalourethra. (g) Open giant megalourethra
388 23 Urologic Problems in Anorectal Malformations

e f

Fig. 23.15 (continued)

23.14 Postoperative Problems
large group of patients are born without neurogenic
bladder, but they are subjected to a technically defi-
cient anorectal operation, during which denerva-
tion is produced, and they acquire this problem.
In some cases, the nerve damage is immediately
evident after the repair of the anorectal malforma-
tion. It is not uncommon to hear from the mother of
the patient to say that the baby had erections prior
to the operation and since the time of the operation
she has not seen one! In other cases, the baby was
passing urine satisfactorily, but suffered from uri-
nary retention after the procedure. The urinary
retention may be temporary; the Foley catheter is
reinserted and removed after a week or two, and
then the patient may start passing urine. Other
times the damage is permanent. However, the fact
that the baby passes urine does not mean that we
can rule out the presence of a neurogenic bladder.
The most common bladder disorder in babies
operated for anorectal malformations is the inca-
pacity to empty the bladder. As part of the follow-
up of all patients with anorectal malformations, we
always order a kidney ultrasound and bladder ultra-
sound (taken at different postoperative intervals,
for instance, 1, 3, 6, and 12 months). In patients in
which we see a persistently full bladder, one must
suspect that the baby is not emptying the bladder
well. A high residual volume of urine reflects an
incapacity to empty the bladder, all of which may
eventually produce vesicoureteral reflux and kidney
damage. We have also seen patients that do not have
vesicoureteral reflux, yet a persistently full bladder
may also produce megaureter and kidney damage.
By emptying the bladder with a catheter and repeat-
ing the ultrasound, one can see the improvement of
the megaureter and hydronephrosis which in the
absence of reflux means that the megaureter and
hydronephrosis are not provoked by a stenosis, but
rather by extrinsic ureteral compression provoked
by a distended bladder. These patients require a full
urinary evaluation, including a urodynamic study
and follow-up by a competent pediatric urologist
who must decide whether the baby is a candidate
for intermittent catheterization to help him empty
the bladder. When the intermittent catheterization
is not feasible or interferes with a good quality of
life, some urologists perform a Mitrofanoff type
of operation [23, 24] to facilitate the emptying of
389
the bladder and to protect the kidneys. In addition,
when the patient has a very limited bladder capac-
ity, high intravesical pressure, and/or poor compli-
ance, the patient may need a bladder augmentation.
Perhaps the most serious problem is the fact
that many of these functional disorders, such as
neurogenic bladder, may turn out to be progres-
sive. This may result in a very unpleasant sur-
prise later in life. Patients that behaved normally
from the urinary function point of view may
develop reflux and kidney damage. That is why
all patients must be followed meticulously with
emphasis in the preservation of the anatomy of
the kidney, as well as its function.
We routinely recommend to do a kidney ultra-
sound 1 month postoperatively and subsequently
3, 6, and 12 months later and every year later,
provided the patient has no urinary tract infec-
tions. When they have urinary tract infections,
the patients deserve a full urologic evaluation.
23.14 Postoperative Problems
Many babies suffer from urologic problems con-
secutive to a damage provoked by a technically
deficient operation performed to repair an anorec-
tal malformation [79–81]. The most conspicuous
problems that we have seen occurred in patients
that were operated without a high-pressure distal
colostogram (see Chap. 6, Sect. 6.6). We insist
that the high-pressure distal colostogram is the
most important diagnostic preoperative study in
anorectal malformations, which allows us to deter-
mine the precise location of the recto-urinary fis-
tula as well as the length of the bowel available
from the colostomy to the fistula site. This infor-
mation is vital for the surgeon to make a precise
surgical plan and to avoid damage to important
urologic structures. Approaching the patient pos-
terior sagittally, looking for a rectum that is not
there, frequently ends up in severe damage to other
important structures. We have seen patients that
suffer from urethral strictures (18 cases) and resec-
tions of the vas deferens, seminal vesicles, and
prostate as demonstrated by the fact that the
patients do not have ejaculation later in life. Also,
the search for the rectum through a posterior
390 23 Urologic Problems in Anorectal Malformations

sagittal incision, in a technically deficient manner,

frequently produces nerve damage that results in
impotence and neurogenic bladder that was not
present prior to the operation. The most important
rule to follow during the posterior sagittal approach
is for the surgeon to remain exactly in the midline.
Once the rectum is found, the dissection of the rec-
tum must be performed, remaining as close as pos-
sible to the rectal wall. Deviating from these rules
increases the chances of nerve damage.

23.15 Posterior Urethral

Diverticulum
(Animation 13.2)

In our series of male patients, 32 of them had the

anorectal repair done at another institution and
came to us complaining of “urinary incontinence,
urinary tract infections, and sometimes orchiepi-
didymitis,” and when we studied them, we found
that they had a piece of rectum left attached to the
urethra during the repair of the anorectal malfor-
mation. We called this “posterior urethral diverticu-
lum” (Fig. 23.16). The retrospective analysis of
these cases showed that most of these patients were
born with a rectourethral bulbar fistula, and the sur-
geons tried to repair the malformation approaching
it through the abdomen, following the old tradition
of performing an abdominoperineal pull-through
for those “high” imperforate anuses (we do not use
that terminology anymore). While trying to dissect
the rectum through the abdomen, trying to reach
the bulbar urethra was very difficult, and frequently
the surgeons decided to “play it safe” and ampu-
tated the rectum at a “convenient” level, leaving a
piece of rectum attached to the urethra.
More recently, we have seen more cases of pos-
terior urethral diverticulum in patients operated
laparoscopically [82–87]. The surgeons, again,
were trying to reach the lowest part of the rectum
and its attachment to the bulbar urethra; they found
that technically difficult and amputated the rectum
again, leaving a piece of rectum attached to the ure-
thra (Animation 23.1). Characteristically, these
patients complained of passing mucus through the
urethra in between voiding episodes. In addition, as
can be seen in Animation 23.1, during the process
Fig. 23.16 Voiding cystourethrogram in a case of poste-
rior urethral diverticulum, B Bladder, D Diverticulum
of voiding and emptying the bladder, part of the
urine goes out through the penis, but other signifi-
cant volume may go into the diverticulum. The
patient finishes emptying his bladder and goes play-
ing, and during that time, he leaks urine from the
diverticulum.
The diagnosis of this condition requires a good
index of suspicion. The history of being born with
a rectourethral bulbar fistula operated through the
abdomen (open or laparoscopically) is highly
suggestive. In addition, the passing of mucus
through the urethra and leaking urine in between
episodes of voluntary voiding strongly suggest
the presence of a posterior urethral diverticulum.
The diagnosis can be confirmed with a voiding
cystourethrogram (Fig. 13.1b) which may or may
not detect the diverticulum. An MRI is a better
study to see the diverticulum in a very accurate
way (Fig. 23.16). Finally, a cystoscopy will con-
firm the diagnosis (see Chaps. 9, 13 and 22).
The treatment is straightforward. The diver-
ticulum can be resected through a posterior
sagittal approach. The pull-through rectum is
23.17 Tethered Cord
mobilized; the diverticulum is open and is sepa-
rated from the urethra following the same steps
and principles described for the separation of the
rectum and the urethra in cases of rectourethral
bulbar fistula.
One particular patient, 30 years old, devel-
oped an adenocarcinoma at the junction between
the diverticulum and the urethra. Fortunately, we
detected the case early enough to resect it and
cure the patient. This is one of the reasons why
we believe that the laparoscopic approach of a
rectourethral bulbar fistula must be considered
contraindicated.
23.16 Sexual Problems
Most of the male patients born with anorectal
malformations operated by us that reached
adulthood are sexually active. They claim to
have satisfactory intercourse and many of them
have children. There is however a small group of
patients that suffer from impotence and others
from lack of ejaculation [88, 89]. It is not clear
to us why that happens. The lack of ejaculation
may happen in patients that underwent “diffi-
cult” operations, performed by surgeons who
were lost in the operative field, and we suspect
that most likely a “small piece of tissue” that
represents the prostate in babies was inadver-
tently resected. The majority of these adult
patients complaining of sexual problems were
operated with “blind techniques.” In other
words, the surgeons had a poor visualization or
exposure of the junction of the rectum to the
urethra. We believe that one of the great advan-
tages of the posterior sagittal approach is the
exposure of the intrinsic anatomy of these mal-
formations, which allows us to avoid this kind of
complications.
23.17 Tethered Cord
Approximately, 24 % of all of our anorectal mal-
formation patients have tethered cord [90, 91].
Others have found a higher prevalence (34 %)
[92]. The presence or absence of tethered cord
391
seems to be important to determine the future
prognosis in terms of urinary function [93]. The
effect of tethered cord on bowel control has not
been scientifically studied. The subject is still
very controversial. In addition, there is no unified
criterion related to the indications to release a
tethered cord. There is also lack of agreement
related with the precise diagnosis of tethered
cord. Many of our patients were seen by different
neurosurgeons, and some of them felt that the
patient had tethered cord, and others believed that
they did not have tethered cord. In addition, some
of our patients had an ultrasound or an MRI study
that showed no evidence of tethered cord, and yet
they were operated as if they had tethered cord,
because the radiologist and/or the neurosurgeons
believed that the conus was “abnormally thick,”
the patient had a “lipoma in the tip of the conus,”
or “the conus was not mobile.” Other anatomic
variations have been described to make the sub-
ject more confusing, including tethered cord with
transitional lipoma and “short spinal cord” [94].
Some neurosurgeons believe that detethering the
cords is important to prevent problems with the
motion of the lower extremities. This is not sup-
ported by others [95].
On the other hand, we have seen adult patients
with tethered cord, and they play soccer and have
urinary and bowel control! We have seen others,
whose mothers indicated that the child was hav-
ing some problems in walking and running, were
operated for tethered cord, and the patients expe-
rienced an improvement in the function and
motion of the lower extremities. Unfortunately,
we have also seen patients that have had release
of the tethered cord and suffered urinary reten-
tion immediately after the operation and subse-
quently developed severe signs of neurogenic
bladder that were not present preoperatively. A
couple of our patients become paraplegic after an
operation for tethered cord!
Most radiologists consider that the presence
of the conus medullaris below L2 plus a nonmo-
bile conus means that the patient suffers from
tethered cord (Fig. 23.17a, b). At present time,
we recommend to do a spinal ultrasound during
the first few days of life, in order to rule out the
presence of tethered cord. If the study shows no
392
a 23.18 The Ultimate Concern,
b stage renal disease [96]. Those patients represent
Fig. 23.17 Spinal Ultrasound. (a) Normal location of the
conus. (b) Tethered cord
evidence of tethered cord, the patient has a nor-
mal sacrum, and in anorectal malformation con-
sidered “benign” in terms functional prognosis,
we do not think that it is indicated to do an MRI
of the spine, provided the patient has bowel and
urinary control, as well as no lower extremity
motion problems. On the other hand, if the ultra-
sound showed evidence of tethered cord, or is
considered borderline or doubtful, we performed
an MRI to confirm or to rule out the diagnosis. If
the patient is asymptomatic and has a “good”
malformation, we keep waiting until the age
when the patient is willing to cooperate and
remain quiet inside the rather scary and noisy
MRI machine. We try to avoid the administration
of heavy sedation or anesthesia as much as
possible.
A good, scientifically oriented study is
required to determine the real significance of the
presence of tethered cord, as well as the indica-
tions for operation, benefits, and potential nega-
tive implications.
Tethered cord is frequently associated with
malformations with very bad prognosis.
Therefore, many of those patients have no bowel
control and no urinary control, but we do not
know how much is because of the tethered cord
and how much is because of the poor sacrum and
other negative anatomic features.
23 Urologic Problems in Anorectal Malformations
Kidney Function
Some patients with anorectal malformations are
born with severe kidney damage, including end-
one of the most serious therapeutic challenges.
They must be treated by a multidisciplinary team
in a sophisticated tertiary center, with experi-
enced pediatric nephrologist and pediatric trans-
plant surgeons [97]. Fortunately, the number of
this type of cases is limited.
There is however a much larger and important
group of patients who were born with relatively
“good kidneys,” but unfortunately they were not
followed and monitored closely. The urologic
problem (reflux, obstruction, infection, poor
bladder function) was not treated adequately
which resulted in severe kidney damage. This is
preventable and must not happen.
We cannot over-empathize the importance of
the frequent monitoring and long-term follow-up
of patients with anorectal malformations [98].
This is particularly true in patients considered at
risk of suffering kidney damage. These include
cloacas [99, 100], bladder neck fistula, deficient
sacrum, tethered cord, patients with hydronephro-
sis at birth, and patients with a single kidney.
In summary, urologic malformations are
extremely important in patients with anorectal
malformations. Pediatric surgeons should become
aware of the fact that most of these patients have
urinary problems and must become familiar with
the way to detect and manage these problems, as
well as to work together with a dedicated pediatric
urologist in studying these kinds of problems.
Also, we want to emphasize the importance of
follow-up of these patients for life, to avoid dam-
age and deterioration of the renal function.
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BJU Int 96(1):131–134
 Hirschsprung’s Disease
24.1 Introduction
Hirschsprung’s disease is a relatively common
condition, well known to all pediatric surgeons.
It is one of the most common causes of intesti-
nal obstruction in the newborn. From the time of
the original description by Harold Hirschsprung
[1] and the proposal of the first rational surgi-
cal treatment by Orvar Swenson [2–4], multiple
reports have been written in the international lit-
erature. The basic principles of the surgical treat-
ment established by Dr. Swenson are still timely
today. Yet, different modalities of treatment have
been described aimed at trying to avoid morbidity
related to the operation as well as trying to be as
least invasive as possible. Simultaneously, there
are a significant number of scientists looking into
the genetics and pathogenesis of this condition,
as well as the pathophysiology of the most feared
sequelae and complication which is enterocolitis.
The reader will find this a rather unusual
chapter. Since we are clinically active pediat-
ric surgeons dedicated to the field of colorec-
tal problems in children, we are privileged to
receive a very high number of cases. We are also
very impressed by the high number of patients
referred to us, who were operated at other institu-
Electronic supplementary material Supplementary
material is available in the online version of this
chapter at 10.1007/978-3-319-14989-9_24.
A. Peña, A. Bischoff, Surgical Treatment of Colorectal Problems in Children,
DOI 10.1007/978-3-319-14989-9_24, © Springer International Publishing Switzerland 2015
24
tions and suffer from preventable complications
consecutive to a poor surgical technique or lack
of good clinical judgment. Interestingly, perhaps
due to the nature of our center, we have treated, so
far, more secondary cases than primary ones. Our
database has information related with 328 cases;
102 cases were operated on by us primarily, 127
secondarily, and 99 were only medically man-
aged. This has given us a unique insight into the
problems affecting children who were born with
Hirschsprung’s disease, problems which some-
times are not mentioned in the literature. Medical
publications (articles and books) are frequently
triumphant reports of good results and success-
ful techniques. Generally speaking, the medical
and surgical community has a tendency to avoid
publications of failures, complications, and bad
sequelae. In addition, very few pediatric surgeons
follow their patients until adult life and, therefore,
miss important feedback, which is related to the
long-term sequelae and quality of life of those
patients who we operated on in the pediatric age.
Since we personally do not have anything to
contribute in terms of genetics and laboratory
research related to this condition, we decided
rather to share with the pediatric surgical com-
munity our clinical experience dealing with
sequelae and complications, particularly those
that we consider preventable, and the way to treat
those problems. Therefore, the first part of this
chapter will be a formal but rather short presenta-
tion of the main characteristics of the disease in
the traditional academic fashion, which means a
397
398
brief discussion about incidence, pathophysiol-
ogy, clinical manifestations, radiologic studies,
histologic findings, surgical treatment, and medi-
cal management of the sequelae. After that, we
will expand on the preventable complications and
the way to deal with those.
24.2 Historical Review
Harold Hirschsprung in 1886, at the Pediatric
Congress in Berlin [1], described an infant who
died with an enormously dilated colon. However,
the cause of this was unknown. In 1691, Frederik
Ruysch [5] also reported a case of a child who
died that may have had the same condition. It was
not until 1901 when Tittle for the first time men-
tioned the possibility that this condition could be
provoked by an absence of ganglion cells of the
distal rectosigmoid [6].
Frederick Treves in London in 1898 [7] actu-
ally performed the complete resection of a
descending colon, sigmoid rectum, and anus on a
6-year-old girl. The patient survived, and Dr.
Treves expressed his desire that she will recover
her bowel control. That case represents the first
case of cured Hirschsprung’s disease, although
the surgeon did not know the intrinsic abnormal-
ity of the distal resected bowel. In 1900, Fenwick
suggested that a possible cause of the disease was
a spastic contraction of the lower end [8]. In
1907, Hawkins suggests that the condition could
have a neuropathic origin [9].
Although not frequently mentioned, it was
actually Dr. Alberto Dalla Valle from Parma,
Italy, in 1920, who found that the distal narrow
portion of the colon had no ganglion cells [10].
This remarkable finding was not mentioned for
unknown reasons. For some years, a sympathec-
tomy and daily enemas were the only treatment
offered to these patients, with less than optimal
results [11, 12].
In 1934, Arthur Hurst firmly believed that anal
achalasia was the primary cause of this condition,
as well as an imbalance of the parasympathetic
and sympathetic innervations of the bowel. He
also mentioned that the name “Hirschsprung’s
disease should be discarded, as von Ammon gave
24 Hirschsprung’s Disease
a good description of two cases in 1842, 44 years
before Hirschsprung’s of Copenhagen” [13].
The abnormal or absent peristalsis of the distal
aganglionic bowel was described by Robertson
and Kernohan in 1938 [14] and Tiffin et al. in
1940 [15], and subsequently, Zuelzer and Wilson
in 1948 [16] correlated the functional disturbance
of the rectosigmoid with the lack of ganglion
cells. These findings were confirmed by
Whitehouse and Kernohan in a study of 11 cases
[17]. In 1948, Swenson in an experimental study
[2] proposed a resection of the rectosigmoid with
preservation of the sphincter for patients who
suffer from this condition. In 1949, Swenson and
Neuhauser (pediatric radiologist) published a
very important paper on the etiology, diagnosis,
and treatment of “congenital megacolon” [3].
After that, until the time of his retirement, Dr.
Swenson accumulated an enormous experience
in the management of this condition [4]. He also
trained many surgeons who continued perform-
ing his operation until the present time.
Many surgeons around the world tried to imi-
tate Dr. Swenson and reproduce his results in the
treatment of this condition and found that some-
times the patients suffered from complications
due to damage of pelvic structures or nerves.
Because of that, in an attempt to reduce this mor-
bidity, other surgical modalities were designed,
but all of them were based on the same principle
of resecting the aganglionic segment and pulling
the normoganglionic bowel down. Most notable
was the technique described by Duhamel in
1956 in France [18]; his technique will be
described later in this chapter.
In 1959, Rehbein, in Germany published his
experience in the treatment of Hirschsprung’s
disease with an operation very similar to an
anterior resection [19]. In other words, he left
in situ a significant piece of aganglionic rectum.
For that reason, the technique did not have many
followers; yet surprisingly, there are many sur-
geons practicing it and claiming reasonably good
results [20].
In Europe, Romualdi [21] in 1960, Ehrenpreis
in 1961 [22], and Pellerin 1962 [23] pub-
lished their experience with the resection of the
rectosigmoid.
24.3 Incidence, Inheritance, and Associated Anomalies
In 1964, Dr. Franco Soave from Italy [24] and
Dr. Scott Boley from the United States [25], inde-
pendently, submitted a paper to the pediatric sec-
tion of Surgery (editor Dr. M. Ravitch). The
technique that they both presented consisted in
dissecting the rectosigmoid, in a surgical plane
between the mucosa and the muscularis (endorec-
tal), in order to protect the neighbor pelvic struc-
tures. The difference between both techniques
was that Dr. Soave pulled the normoganglionic
bowel down and left it exteriorized through the
anus, for several days, after which he performed
the anastomosis to the anus. Boley, on the other
hand, was doing a primary anastomosis. For rea-
sons unknown to us, the paper of Soave was pub-
lished before the one of Boley. It is important to
recognize that Boley’s paper was not presenting a
modification to the Soave technique, but rather a
modification to the Swenson technique [26].
More recently, the concept of minimally
invasive surgery (laparoscopy) was applied.
In 1994, Smith et al. [27] reported on one case
who underwent a “laparoscopic Duhamel pull-
through procedure.” Subsequently, Georgeson,
in 1995, published his experience with 12 cases
who underwent a “primary laparoscopic pull-
through,” applying the endorectal dissection of
the rectosigmoid performed through the abdo-
men and transanally [28]. This kind of technique
was widely accepted.
In 1996, Saltzman et al. introduced a modifi-
cation to the Soave technique, consisting in per-
forming the entire mucosal dissection through a
transanal approach as the abdominal incision was
made. Their experience included 25 cases [29].
Dr. Luis de la Torre published his experimen-
tal work in rabbits, subjected to transanal,
endorectal rectosigmoid resection [30]. His clini-
cal experience with five cases, with Hirschsprung’s
disease operated transanally, without laparotomy
or laparoscopy, was published in 1998 [31].
Shortly after that, in 1999, Dr. Jacob Langer et al.
published his experience with the transanal, one-
stage Soave procedure [32].
When this operation is performed endorec-
tally, basically it is the same principle of
Soave, but done transanally and many times
without opening or invading the abdomen. This
399
revolutionary concept gained enormous popular-
ity, and it is perhaps the most common surgical
technique used at the present time. Yet, the basic
principles proposed by Swenson must still be
observed during this kind of operation. The fact
that 85 % of the patients with Hirschsprung’s dis-
ease have an aganglionic segment that extends to
the rectosigmoid, the need to open the abdomen
or to use laparoscopy is reduced to about 20 % of
the cases when using a transanal approach. The
transanal approach is painless and when it is done
correctly, has very good results [33].
24.3 Incidence, Inheritance,
and Associated Anomalies
The estimated incidence rate was found to be 1 in
4,417 live births in what we thought was the most
representative study of the subject [34]. It occurs
four times more frequently in males in the typical
form. In long-segment cases, this ratio is 1.5:1
(male/female). Familiar cases are three times
more frequent in females and occur in approxi-
mately 4 % of the cases [35].
Associated defects occur in approximately
29 % of cases [34], mainly cardiovascular and
gastrointestinal. Five percent of the cases may
have Down syndrome [36]. Approximately 5 %
of the cases may also suffer from a developmen-
tal disorder (Mowat-Wilson syndrome) [37].
Other associated problems include malrotation,
ileal and colonic atresia, Dandy-Walker syn-
drome [38], and Ondine’s curse [39]. Finally a
great interest developed recently about the “mul-
tiple endocrine neoplasia” (MEN) syndrome and
its association with Hirschsprung’s disease [40].
This is mainly due to the ability to predict the risk
for development of malignant tumors by genetic
RET proto-oncogene analysis. This resulted in
the recommendation to follow up these patients
and the possibility to do a prophylactic thyroidec-
tomy [40].
Many believe that Hirschsprung’s disease
occurs more frequently in anorectal malforma-
tions as compared with the general population
[41, 42], but we disagree with the concept. We
believe that Hirschsprung’s disease is frequently
400
erroneously diagnosed in patients who were born
with anorectal malformation due to the fact that
the most common sequela seen in anorectal mal-
formation is constipation. Surgeons are educated
and trained to suspect Hirschsprung’s disease
in patients with constipation. It is not unusual,
therefore, for them to overdiagnose this condi-
tion, and unfortunately, sometimes they treat it as
such. We believe that Hirschsprung’s disease in
anorectal malformations most likely occurs with
the same frequency as in the general population.
24.4 Pathogenesis
The etiology and potential explanation of the pos-
sible origin and mechanisms of this fascinating
condition has been the subject of an enormous
number of studies and publications. It is beyond
our area of expertise in the scope of this, mainly a
practically oriented book, to analyze and discuss
the multiple papers related with this subject. It is
worth mentioning, however, the monumental
work performed by Dr. Prem Puri [43–52].
Every year we read more new material coming
from the laboratory of prominent research-
oriented pediatric surgeons. The deeper they get
in the investigation of the origin and the intrinsic
mechanisms of this condition, the more complex
it seems to be [53–61].
In 1967, Okamoto and Ueda [62] published an
elegant paper, a study done with human embryos
that allowed him to present his theory of the
cranial-caudal migration of the neuroblast in the
gastrointestinal tract. As time goes by and more
research is performed, things seem to be more
complex than previously thought.
From a more practical point of view, we can
say that congenital megacolon (Hirschsprung’s
disease) is characterized by a functional colonic
obstruction usually manifested mostly during the
newborn period. These patients do not have a real
mechanical obstruction but rather have abnor-
mal or absent peristalsis of the colon (usually the
most distal portion). The part of the colon that
has no ganglion cells does not have the neces-
sary peristalsis to expel the stool, but rather is
a spastic portion of the bowel. The aganglionic
24 Hirschsprung’s Disease
segment of the bowel is always located distally,
and the length of the aganglionic segment varies
from patient to patient. Typically, the aganglionic
segment affects the rectosigmoid (Fig. 24.1).
This type is known as “typical Hirschsprung’s
disease” and represents about 67–82 % of the
total number of cases. In about 15–25 % of the
cases, the aganglionosis affects longer segments
up to the splenic flexure or even the transverse
or right colon (Fig. 24.1); this is called “long-
segment Hirschsprung’s disease.” About 3–8 %
of the patients suffer from aganglionosis of the
entire colon including segments of the terminal
ileum which is called “total colonic agangli-
onosis” [34]. Very rarely, patients are born with
what is called “universal aganglionosis” which
is mostly a lethal condition unless treated with
intestinal transplantation. A condition known as
“ultrashort-segment aganglionosis” (Fig. 24.1) is
highly debatable; a strict rational, objective, sci-
entific analysis of the available published mate-
rial generates many doubts and skepticism about
the existence of this condition. This will be dis-
cussed later.
The aganglionic segment in Hirschsprung’s
disease has been described radiologically as the
“narrow segment.” In reality, rather than narrow,
it is a nondistended or spastic part of the colon.
Proximal to the aganglionosis, the colon becomes
extremely dilated (Fig. 24.1). At birth, the dilata-
tion of the proximal colon may not be so severe
(Fig. 24.2). However, as time passes, the dilata-
tion gets worse, and the size discrepancy between
the proximal dilated normoganglionic and the
distal, non-dilated aganglionic segment becomes
more conspicuous, eventually showing the typi-
cal radiologic image. The surgical literature fre-
quently refers to the “transition zone” referring to
that part of the bowel located between the dilated
normal ganglionic and non-dilated aganglionic
segment. The histologic study of the distal non-
dilated bowel shows absence of superficial sub-
mucosa (Meissner), deep submucosal (Henle),
and intramuscular (Auerbach) ganglion cells. In
addition, there is a marked increase in size of
prominent nerve fibers. The enzyme acetylcho-
line esterase activity is markedly increased. The
ganglion cells act as a final common path for both
24.4 Pathogenesis
Fig. 24.1 Diagrams
showing different types of
Hirschsprung’s (a) Normal
colon, (b) Classic type, (c)
Long-segment, (d) Total
colonic aganglionosis,
(e) Ultra short type
b c
d
sympathetic and parasympathetic activities. The
aganglionic segment, therefore, suffers from
spasm, lack of propulsive peristalsis, and con-
traction. In addition, characteristically these
patients suffer from a lack of relaxation of the
muscle that surrounds the lower part of the rec-
tum described as an internal sphincter. This is a
manometric finding. Recently, it has been pro-
posed that nitric oxide, a neurotransmitter respon-
sible for the inhibitory action elicited by the
intrinsic enteric nerves, has a role in this condi-
tion. A lack of nitric oxide synthase (the enzyme
401
a
e
required for nitric oxide production) may play a
role in the pathophysiology [56–58].
Patients with Hirschsprung’s disease not only
have manifestations of colonic obstruction but
what makes this clinical picture different from
other etiologies of colonic obstruction is the
fact that proximal to the aganglionic segment,
these patients frequently have a tendency to
overgrow abnormal bacteria, specifically C. dif-
ficile, and they suffer from severe endotoxemia,
a condition well known by pediatric surgeons as
“Hirschsprung’s enterocolitis.” In other words,
402 24 Hirschsprung’s Disease

Fig. 24.2 Contrast enema showing the transition zone in a newborn. Contrast enema
24.6 Clinical Manifestations and Differential Diagnosis
these babies not only have the pathophysiology
and possible complications of lower intestinal
obstruction, but in addition, they may become
extremely sick and even die from the characteris-
tic endotoxemia produced by enterocolitis.
24.5 Genetics
Exciting developments are occurring in the field
of genetics in Hirschsprung’s disease [63–71].
We look forward to witness more of these scien-
tific advances that will result in the benefit of
many children.
24.6 Clinical Manifestations
and Differential Diagnosis
The first clinical manifestations of babies born
with Hirschsprung’s disease are abdominal disten-
tion, vomiting, and the lack of passage of meco-
nium in the first 24–48 h of life. Most neonatal
units recognize the lack of passage of meconium
in the first 24–48 h as a very suggestive sign of
Hirschsprung’s disease. Every baby who has not
normally passed meconium should be thoroughly
investigated for the possibility of having
Hirschsprung’s disease. There is a small group of
patients born with Hirschsprung’s disease that do
not have very obvious clinical manifestations at
birth; they grow up and are diagnosed later in life.
This group of patients most likely belongs to a cat-
egory of Hirschsprung’s disease that we consider
benign. In our experience, they rarely have epi-
sodes of enterocolitis, and when they are operated
on, the results are better than those obtained in
babies who have very early manifestations [72].
Characteristically, newborn babies with
Hirschsprung’s disease look lethargic and the
abdomen is distended. However, rectal stimula-
tion either with a digital exam or with a catheter
frequently results in an explosive and massively
deflating passage of liquid stool and gas, which
dramatically improves the baby’s condition. The
baby may then show signs of being hungry and
even eat, only to have the same symptoms return
hours later and to repeat the cycle when the baby
403
has a rectal examination. The liquid stool in these
babies is characteristically fetid. Early detection
of this dangerous stage of the disease is extremely
important. These babies need aggressive resusci-
tation with intravenous fluids, fasting to avoid
exacerbation of the symptoms, and a prompt and
efficient way to decompress the colon. The most
expedient and easy way to decompress the colon
is using a rather large (no. 18 or no. 20 French)
tube passed through the rectum and irrigate with
saline solution. The liquid stool then comes out
through the lumen of the tube, decompressing the
baby’s abdomen. This is repeated several times
during the day and allows the baby to improve his
symptomatology significantly. The administra-
tion of intravenous metronidazole is also recom-
mended to fight the bacterial overgrowth. When
the colon is not adequately decompressed, these
babies are at risk of dying from enterocolitis,
endotoxemia, and hypokalemia or they may suf-
fer perforation of the colon. The most common
site of perforation is the cecum or the appendix.
In addition, they may have pneumatosis intesti-
nalis or develop pericolic abscesses. When this
condition goes unrecognized, the mortality
ranges between 25 and 30 % [73]. Some authors
even report 50 % mortality for untreated patients
during the first year of life [74].
The diagnosis of Hirschsprung’s disease is
strongly suggested by the clinical symptomatol-
ogy. A contrast enema reinforces the clinical sus-
picion, and finally, the diagnosis is confirmed by
a rectal biopsy showing absent ganglion cells, the
presence of hypertrophic nerves, and increased
activity of acetyl cholinesterase. An abdomi-
nal plain x-ray film shows enormously dilated
loops of bowel (Fig. 24.3). Most pediatric radi-
ologists and experienced clinicians agree that it
is extremely difficult to differentiate in a plain
abdominal film in a newborn baby a distended
colon from a distended small bowel. That may
be possible in adults but is almost impossible
in newborn babies. However, very few neona-
tal conditions give an image of such an enor-
mous dilatation of loops of bowel. The next step
after we see the plain abdominal x-ray film is
to perform a contrast enema. Characteristically,
the study shows a non-dilated portion, usually
404 24 Hirschsprung’s Disease

Fig. 24.3 Abdominal x-ray film of a newborn baby with Hirschsprung’s disease
24.7 Histologic Diagnosis
a b
Fig. 24.4 Characteristic colonic changes in Hirschsprung’s. (a) Diagram. (b) Contrast enema
rectosigmoid, followed by a transition zone, and
more proximately a dilated segment of the colon.
These characteristic changes (Fig. 24.2) may
not be present or may not be very obvious in the
newborn period. As time goes by, the functional
obstruction produces more proximal dilatation,
and months or years later, the characteristic
radiologic picture of a giant proximal megaco-
lon with a narrow distal segment becomes very
clear (Fig. 24.4). It is important to remember
that in dealing with intestinal obstruction of the
newborn, the only condition that has symptoms
of intestinal obstruction and a dilated colon is
Hirschsprung’s disease. All the others have char-
acteristically a nonused colon (microcolon). An
exception could be observed in babies with nec-
rotizing enterocolitis, but the other symptoms
and signs make this easy to differentiate from
Hirschsprung’s disease.
Some newborns have a very obvious, radio-
logic image characteristic of Hirschsprung’s dis-
ease. In others, however, the transition zone and
the proximal dilated portion are not so clear, yet
together with the clinical manifestations allow us
to make a diagnosis of Hirschsprung’s disease
(Fig. 24.2). When the contrast study has been
done, another characteristic of these babies is
their incapacity to expel the contrast material
405
24 h after the study. This makes the diagnosis of
Hirschsprung’s more likely.
We, like others [75], use and recommend a
contrast enema as an initial diagnostic tool. We
believe that with the combination of a good index
of suspicion, clinical experience, contrast enema,
and rectal biopsy read by an experienced patholo-
gist, we can offer a safe management to these
patients. We do not remember, from our large
practice a false-positive or false-negative case.
We recognize that many times, the symptoms
can be rather equivocal; in addition, sometimes
the images obtained with the contrast enema
may not be characteristic. Also, we had cases in
which the suction rectal biopsies did not obtain
good specimens. In such cases, we take a full rec-
tal biopsy, which finalizes the diagnostic work.
We are aware of the fact that many surgeons
value the rectal manometry for the diagnosis of
Hirschsprung’s [76–82]. We have no experience
with that diagnostic modality.
24.7 Histologic Diagnosis
Very significant advances occur in the knowledge
of the histologic abnormalities present in this
disease [83–87]. Sometimes, we surgeons feel
406
overwhelmed by the variety of terms and abun-
dance of new findings. We keep trying to famil-
iarize with a variety of terms like VIP (vasoactive
intestinal peptide), adenylate cyclase-activating
polypeptide (PACAP), gastrin-releasing peptide
(GRP), calcitonin gene-related peptide (CGRP),
substance P (SP), enkephalins and galanin-
immunoreactive nerve fibers, neuropeptide Y
(NPY)-containing nerve fibers, and calretinin.
Despite all the advances, the fundamental
basis for the diagnosis of Hirschsprung’s disease
still depends on a skillful pathologic analysis and
the use of hematoxylin and eosin stains, to iden-
tify the presence or absence of ganglion cells and
hypertrophic nerve trunks. Acetylcholinesterase
(AchE) histochemistry on frozen sections and
immune histochemistry for ganglion cells are
useful ancillary techniques, but they do not
replace the H&E methodology. Adequate sam-
pling, extensive sectioning, and an experienced
pathologist are the most important aspects of a
good histologic diagnosis [88].
Different clinicians give a different value to the
diagnostic tools for Hirschsprung’s disease. Some
believe very much in the rectal mucosal suction
biopsy, and that is all they do to make the diagno-
sis. Others prefer a full-thickness rectal biopsy. In
certain institutions, there are experienced patholo-
gists dedicated to the diagnosis of Hirschsprung’s
disease by a suction rectal mucosal biopsy. At
other institutions, pathologists do not have experi-
ence with this; they insist that the tissue obtained
with suction rectal biopsy is not enough for a
definitive diagnosis, and they demand a full-thick-
ness specimen to make a reliable diagnosis. We
try not to be dogmatic in this chapter concerning
which diagnostic tool is more valuable than oth-
ers. We rather believe that it depends on the spe-
cific surrounding circumstances where the baby is
born and the experience of the surgeon, the neona-
tologist, the pathologist, and the radiologist. As a
matter of fact, in one of the institutions where we
worked in the past, there was only one pathologist
that had enough experience in Hirschsprung’s dis-
ease to make a reliable diagnosis. Therefore, we
only operated on Hirschsprung’s disease patients
when that particular pathologist was present. The
main message that we would like to give to the
24 Hirschsprung’s Disease
pediatric surgical community is that we should
not assume that every pathologist can make the
diagnosis of Hirschsprung’s disease. For instance,
the diagnosis based on frozen section is reliable
for certain specific experienced pathologists but
not for others. Therefore, the surgeon must be
ready to follow a different surgical strategy in the
management of these patients depending on his or
her surrounding circumstances. In some countries
that we have visited, there are no pathologists
available sometimes for several days, and there-
fore, that requires a different surgical strategy. In
some places, the surgeons tend to believe only in
the increase of the activity of acetylcholine ester-
ase rather than on the absence of ganglion cells.
Most pathologists all over the world agree, like
us, that the basis for the diagnosis should be the
absence of ganglion cells and the presence of
hypertrophic nerves.
24.8 Differential Diagnosis
The most common condition that must be dif-
ferentiated from Hirschsprung’s disease is the
meconium plug syndrome. The expulsion of the
meconium plug with disappearance of the symp-
toms allows Hirschsprung’s to essentially be
ruled out. Meconium ileus is an early manifesta-
tion of cystic fibrosis, and therefore, one expects
to find the other stigmata of cystic fibrosis includ-
ing intestinal obstruction, an image of “ground
glass” representing inspissated meconium in the
small bowel, respiratory symptoms, lack of air-
fluid levels, and response to the specific treatment
to try to liquefy the inspissated meconium.
One very unusual condition, called small left
colon syndrome, occurs when the narrow portion
of the colon on the contrast enema usually reaches
the splenic flexure. Symptoms usually disappear
after the contrast study has been done and resolve
spontaneously after several weeks. The mothers of
these babies frequently have maternal diabetes.
Several other conditions are associated to
hypomotility of the colon and may mimic
Hirschsprung’s including hypothyroidism and
effects from opiate or magnesium sulfate,
transmitted from the mother.
24.10 Surgical Treatment
24.9 Early Management
We recommend the administration of metronidazole
as soon as we suspect the diagnosis of Hirschsprung’s
disease; however, we believe that the most valuable
maneuver that actually saves many lives in babies
with Hirschsprung’s disease is colonic irrigation
with saline solution (Animation 24.1).
Unfortunately, in many institutions, the term
“colonic irrigation” is confused with an enema. An
enema consists of the introduction of a specific vol-
ume of a fluid into the colon in order to provoke a
bowel movement. By definition, patients with
Hirschsprung’s disease have poor peristalsis, inca-
pacity to expel fluids, and therefore, enemas are
contraindicated as they may be retained and aggra-
vate the symptomatology. On the other hand, irriga-
tion means the passing of a tube through the rectum,
large enough to allow the expulsion of liquid stool
through the lumen of the tube itself. The term “irri-
gation” comes from the idea of passing, through the
lumen of the tube, small volumes of saline solution,
10 mL at a time, in order to clear the lumen of the
tube to allow decompression of the colon. The tube
is well lubricated and is passed through the rectum,
which allows an explosive decompression of most
of the colon. After this first episode, the nurse or the
doctor passes small volumes of saline solution
through the tube and keeps moving the tube back
and forth and rotating it, trying to get into the pock-
ets of gas and liquid stool (See Animation 24.1
showing a “Colonic Irrigation”). The benefit of this
irrigation is very obvious from the moment it is
done. In fact, it may save the baby’s life. Even when
this explanation of the difference between an enema
and irrigation sounds obvious, we have been
impressed by the frequency with which these two
terms are confused even in well-reputed institutions
and how often irrigations are neglected in the initial
management altogether.
24.10 Surgical Treatment
The main goal in the treatment of patients with
Hirschsprung’s disease is to resect the agangli-
onic segment and pull down normoganglionic
bowel that must be anastomosed to the rectum
407
above the anal canal, preserving the part of the
bowel where the sensitivity resides and preserv-
ing the sphincter mechanism (anal canal). All
techniques, regardless of its specific maneuvers,
must observe these basic principles.
There is some controversy about how to treat
these babies. Should we perform this operation
primarily? Or should we open a colostomy first?
Rather than recommending a specific type of
approach to this condition, we believe that the
surgical approach depends on the specific circum-
stances that surround the patient as well as the
surgeon. A primary operation for Hirschsprung’s
disease done in the newborn requires experi-
ence, a sophisticated surgical environment, good
anesthesia, good intensive care, good pathology,
a sterile environment, as well as possibilities to
provide parenteral nutrition to the baby. This, we
take for granted in the United States; yet, it is not
available in many other countries, and therefore,
different strategies must be followed to allow
these babies to survive.
Traditionally, these patients received a colos-
tomy at birth, usually a right transverse colos-
tomy followed later in life by an abdominoperineal
resection of the aganglionic segment with pull-
through of the normoganglionic bowel and a
third operation consisting of a colostomy closure.
Subsequently, another way to approach these
patients consisted in opening what is called a
“leveling colostomy,” meaning to open the colos-
tomy in the transition zone; later on, in a second
operation, the aganglionic segment was resected
and the normoganglionic colostomy site was
pulled down to be connected to the rectum. This
was known as a “two-stage approach.” Finally in
1980, Dr. Henry So, in Long Island, New York,
published his experience with an endorectal pull-
through without colostomy in neonates with
Hirschsprung’s [89]; soon after, that modality of
treatment was adopted by many surgeons [90–
101]. Other modalities include a laparoscopic
primary approach and, more recently, the primary
transanal approach [30].
We believe that there are many ways to solve a
problem, and all of them can be equally good.
Regardless of the therapeutic approach used, the
fundamental goals of treatment should be to
408
resect the aganglionic segment and to pull down
a normoganglionic piece of colon without caus-
ing any harm to the pelvic organs, or their nerves,
and preserving intact the anal canal. This can be
done in a single, primary operation or with two or
even with three operations, depending on the spe-
cific circumstances of the patient, the surgeon’s
experience, and the surrounding environment.
The main goal can also be achieved using differ-
ent techniques, laparoscopically or open. It is
fashionable among surgeons to talk about the
“gold standard” when referring to the best way to
treat a specific condition. It is also very common
for pediatric surgeons to compete trying to per-
form these operations earlier and earlier in life
and using the least invasive technique, with a
minimal length of stay in the hospital and mini-
mal cost. Looking at our experience in dealing
with patients who came to our institution after a
failed attempted repair, or after suffering cata-
strophic preventable complications, lead us to
believe that it is not so important which treatment
modality was used. What is really important for
the patient is to be operated by a meticulous,
experienced surgeon, using the treatment modal-
ity with which he or she feels most comfortable
and with which the aforementioned goals can be
achieved.
Clearly, it is ideal to repair a defect as early as
possible, in the least invasive way, and in a single
operation, but far more important than that, to
avoid catastrophic, preventable complications
such as neurogenic bladder, fecal incontinence,
dehiscence, retraction, rectovaginal fistula, and
rectourethral fistulas.
24.10.1 The Authors’ Approach
If a baby is born nearby our institution or is
brought very early with symptoms consistent with
Hirschsprung’s disease, we perform a contrast
enema and a suction rectal biopsy. Once we estab-
lish the diagnosis, assuming it is a full-term baby
with no concerning associated defects and is clini-
cally stable, we insert a central line (PICC line)
and keep the patient with nothing by mouth
receiving parenteral nutrition. We have learned
24 Hirschsprung’s Disease
that these patients do not need a nasogastric tube
provided one keeps the colon decompressed. The
babies vomit when they have a distended colon
and have enterocolitis, but the decompression of
the colon stops the vomiting. A program of irriga-
tions three times per day and intravenous metroni-
dazole is started. The goal of the irrigations is to
avoid enterocolitis and to maintain the colon
decompressed and the abdomen flat. Gradually,
over the period of a few days, we can see that
what used to be fetid liquid stool becomes less
and less so more clear and eventually becomes
bilious and odorless. Occasionally (rare in our
institution), one can see a baby suffering from
severe enterocolitis, endotoxemia, and shock
coming to the hospital. Under those circum-
stances, we understand that may be justified to
open a colostomy to save the baby’s life. At least
in the environment where we work, this particular
situation occurs very rarely. Newborn babies
come to our hospital looking sick, yet, they
respond well to the management described and
can be operated primarily after a few days of med-
ical management (irrigations and metronidazole).
At this point, with no evidence of enterocoli-
tis, a nondistended colon, and the baby in good
condition, we perform a primary transanal repair.
Based on the fact that 85 % of the patients have
an aganglionic segment that extends only to the
rectosigmoid, we believe that it is necessary to
open the abdomen or to perform a laparoscopy in
approximately 15 % of the cases. Some surgeons
advocate the use of laparoscopy in all cases, to
take biopsies to determine the extent of the agan-
glionosis. We believe that it is not necessary to
follow that approach. Transanally, as we progress
in our dissection, we can take full-thickness biop-
sies every 5 cm until we reach the normogangli-
onic bowel. If the contrast study gives no clue as
to the location of the transition zone or if we are
suspicious of total colonic Hirschsprung’s, start-
ing with laparoscopy and biopsies may be
appropriate.
We perform a full-thickness (like Swenson)
transanal (de la Torre) dissection. Based on a
large experience in the treatment of anorectal
malformations, we have learned to dissect the
rectum, keeping our plane of dissection as close
24.10 Surgical Treatment
as possible to the bowel wall without injuring any
nerves and/or pelvic organs. In other words, we
do not feel the need to do this dissection using an
endorectal, submucosal technique. As we gain
length in the dissection, we keep taking biopsies
every 5 cm, sending them to pathology. Once we
have reached the normal ganglionic bowel, we
still go 5 cm or more, proximally. If the normal
ganglionic bowel is very dilated, we continue
pulling down more colon. In other words, we try
to resect not only the aganglionic segment but
also as much of the dilated normal ganglionic
colon as possible. After the resection, a two-layer
anastomosis is done 2 cm above the pectinate
line. If, in the process of dissecting the rectum,
we find that it is becoming technically more
demanding and difficult to continue the dissec-
tion because we already have passed the sigmoid
and reaching the descending colon becomes
extremely difficult, then we decide to go into the
abdomen either laparoscopically or opening the
abdomen to free the descending colon, the splenic
flexure, or even the transverse colon.
The transanal approach is a very impor-
tant significant contribution in the therapy of
Hirschsprung’s disease. We believe that both Drs.
de la Torre and Langer should be commended for
this contribution. Yet, we have serious concerns
that this technique may1 not be very reproducible.
In other words, when this technique is not done
correctly, serious damage may be provoked to the
sphincter mechanism and to the anal canal. We
have seen videos and photographs of transanal
operations presented in different meetings, show-
ing images with rather aggressive, less-than-
optimal maneuvers that we believe may provoke
fecal incontinence. Because of that, we want to
emphasize what we consider the crucial steps in
the surgical technique of the transanal resection
of the rectosigmoid (see attached DVD).
We use a Lone Star2 retractor to perform this
operation [102, 103]. This retractor provides an
excellent exposure combined with minimal inva-
siveness. The baby is placed in the prone position
1
Lone Star Retractor System, CooperSurgical Inc.,
Trumbull, CT, USA.
2
See footnote 1.
409
with the pelvis elevated. We believe that there is
no need to operate on these babies in lithotomy
position, which is a reminiscent of adult surgi-
cal techniques. With the patient in prone position,
the surgeon works more comfortably in a hori-
zontal field without losing instruments and can
perform the dissection easier, and this position is
much easier on the assistant and the scrub nurse.
The eight hooks of the Lone Star retractor are
placed in a symmetric and radial way at the anal
margin in order to be able to see the pectinate
line (Fig. 24.5). Subsequently, all the hooks are
replaced deeper, taking the rectal wall above the
pectinate line (Fig. 24.6) so that it is hidden. At
that point, we know that what is exposed is only
rectal mucosa. We are now sure that the entire
anal canal, plus at least 1 cm of rectal mucosa, has
been circumferentially folded, retracted, and pro-
tected. What we see deep in our field (Fig. 24.6)
is only rectal mucosa. This way we can be very
accurate in measuring the distance between the
pectinate line and the beginning line of our resec-
tion. Many authors talk about initiating the resec-
tion 5 or 10 mm above the pectinate line. We
prefer to go 2 cm more proximal for the reasons
that we will explain when we talk about compli-
cations from these procedures. Multiple 5-0 or
6-0 silk circumferential stitches are placed tak-
ing the rectal mucosa (Fig. 24.7). These stitches
will provide uniform traction. We use a delicate
needle-tip cautery and make a full-thickness cir-
cumferential incision of the rectal wall, periph-
eral to the multiple silk stitches (Fig. 24.8). We
use the cautery in cutting mode when cutting and
coagulation mode to fulgurate specific vessels.
Peripheral to the full-thickness wall of the rec-
tum, one finds a funnel-like skeletal muscle that
contracts every time we touch it with the cautery.
We should not violate that wall of skeletal mus-
cle. If we go through that, we find characteristic
ischiorectal fat that should alert us that we are too
far away from the correct plane and therefore at
risk of injuring other structures. The dissection of
the anterior rectal wall in male patients must be
performed very meticulously. It is perfectly valid
to go submucosally in this particular area to guar-
antee that we are not injuring the prostate and
the posterior urethra. Likewise in females, the
410 24 Hirschsprung’s Disease

a b

Fig. 24.5 Eight hooks in place. (a) Diagram. (b) Intraoperative picture

Fig. 24.6 Eight hooks placed deeper – only rectal

mucosa is exposed. The anal canal is protected (folded) by Fig. 24.7 Multiple silk stitches
the hooks of the retractor

posterior vaginal wall must be protected. If the
dissection is performed correctly, one finds that
dissecting the rectum in a full-thickness fashion
allows us to work in a bloodless field only burn-
ing the extrinsic blood vessels of the rectum. This
represents a much less bloody field than when
using a submucosal dissection. When one is
performing this dissection in the right plane, the
dissection is performed most of the time outside
the level of the retractor. In other words, we keep
dissecting and pulling the rectum mostly outside
the anus; we try not having to work in a deep field
24.10 Surgical Treatment 411

a b

Fig. 24.8 Circumferential incision, peripheral to the silk stitches. (a) Diagram. (b) Intraoperative picture

a b

Fig. 24.9 Biopsies taken every 5 cm (a) 15 cm dissected, (b) 20 cm dissected

unless we are dealing with a reoperation with a
lot of fibrosis in the pelvis. Every 5 cm of length
that we gain, we take a full-thickness biopsy that
is sent to pathology (Fig. 24.9). Obviously, this
approach is safe when one is working in an envi-
ronment with a pathologist who has experience
with frozen sections. In about 80 % of the cases,
we reach the normoganglionic bowel without the
need to open the abdomen or doing laparoscopy.
Yet, we go at least 5 cm above the site of the
biopsy that showed ganglion cells; we also resect
as much of the dilated normoganglionic bowel as
possible through the same transanal approach. A
two-layer anastomosis between the normogan-
glionic bowel and the rectum is performed, 2 cm
above the anal canal (Fig. 24.10). The first layer
of the anastomosis takes the external or serosal
part of the colon and sutures it to the peripheral
tissues above the edge of the resection of the rec-
tal mucosa. This first layer of sutures is performed
before the resection of the colon. Figure 24.11
shows the resection of the colon. In the second
412
Fig. 24.10 Diagram showing the deep layer of the
anastomosis
inner layer, more emphasis is placed in being
sure that the mucosal edges come together. This
suture is performed with 6-0 Vicryl (Fig. 24.12).
During the dissection, we assist ourselves with
the use of small pediatric malleable retractors
and a long, narrow Deaver retractor. During this
dissection, we work following the principle of
triangular exposure. We pull on the multiple silk
rectal stitches in one direction, use the suction
tube to improve the exposure, and 1 or 2 narrow
retractors are introduced in order to see exactly
what we are doing. We are very careful to not
excessively stretch the anal canal. We believe that
serious damage may be inflicted when the sur-
geon stretches the anal canal excessively in order
to have better exposure.
In about 20 % of patients, the dissection con-
tinues, but we cannot reach the normoganglionic
24 Hirschsprung’s Disease
Fig. 24.11 Resection of the colon
bowel. The dissection becomes more difficult and
risky because we are trying, through the anus, to
cauterize mesenteric intraperitoneal vessels that
may retract and bleed into the peritoneal cavity.
Under these circumstances, we go into the abdo-
men, either laparoscopically or by laparotomy.
This is easily done because we perform these
operations using what we call “total body prepa-
ration.” We use this type of preparation modal-
ity in all of our abdominoperineal procedures.
The reader can go to Chap. 7 to see the details
of a total body preparation. When necessary, the
baby is turned to supine position and laparotomy
or the laparoscopic approach is performed. In
order to avoid the leak of CO2 from the perito-
neal cavity, packing gauze can be used in the
anus. Laparoscopically, the descending colon is
detached from its normal (left gutter) attachments
as well as the splenic flexure. This maneuver is
usually enough for the normoganglionic bowel
to reach the anus and to perform a successful
tension-free anastomosis. If there is not enough
length, we have to detach the hepatic flexure to
gain extra colonic length. Sometimes the right
colon must be de-rotated for the hepatic flexure
to reach the anus. Rarely, one is confronted with
the case of a total colonic aganglionosis which
should have been suspected preoperatively. This
will be discussed later.
If the decision is made to go into the abdomen
through a laparotomy, we prefer a midline inci-
sion. In dealing with colorectal problems, we
favor the midline abdominal incisions in order to
24.10 Surgical Treatment
a b
Fig. 24.12 Superficial layer of stitches (inner layer). (a) Diagram. (b) Operative picture
preserve both sides of the abdominal wall in the
event that the patient requires a stoma. Transverse
incisions invade the area of the stomas, and the
patients may end up with a stoma located too
close to an incision, which is a serious inconve-
nience, since it may interfere with the placement
of the stoma appliance.
At the end of the procedure, most of the time,
the patient stays without a protective colostomy,
provided we feel comfortable about the blood
supply of the pulled-through bowel as well as
the tension of the anastomosis. We believe that
strictures that occur at the anastomosis site are
due to poor surgical technique and frequently
ischemia of the bowel or excessive tension. If it
happens that the patient requires a laparotomy or
laparoscopy, the surgeon must be very careful in
413
observing the blood supply of the colon in order
to be sure that they ligate or cauterize the right
vessels to avoid ischemia of the distal bowel. The
anastomosis should not be done under tension.
When there is any question about the viability
of the bowel as well as the tension, a protective
colostomy is indicated.
Postoperatively, these patients recover remark-
ably well. They have no pain unless they had
a laparotomy or laparoscopy. They frequently
show signs of being hungry. We give broad-
spectrum antibiotics for 48 h. Metronidazole is
administered on a long-term basis, which we will
explain. Seven to ten days after the operation, we
start feeding the patients.
Many patients with Hirschsprung’s disease
are, or eventually become, lactose intolerant, and
414 24 Hirschsprung’s Disease

a b

Fig. 24.13 Characteristic image postoperative for Hirschsprung’s showing gas and liquid in the descending colon and
rectum. Indication for irrigation. (a) Before irrigation. (b) After irrigation

therefore, as early as possible, we suggest feed-
ing them with a substitute for milk. In addition,
we have seen that the ingestion of milk tends to
produce more bowel gas and may show signs
of intolerance as well as increase the chance of
the patient to suffer from enterocolitis. We like
to feed the patients while still in the hospital, in
order to observe their reaction. We take an x-ray
film to be sure that there is a normal colonic gas
pattern. We watch carefully for abdominal dis-
tension and irrigate the colon, through the anus,
if necessary.
Nowadays surgeons and institutions compete
to try to decrease the length of stay. Unfortunately,
sometimes this has been mainly motivated by
economic reasons rather than for the benefit of
the patient. We have seen patients that have been
discharged very early from other institutions,
after a “successful” pull-through performed in
the newborn period, coming to our institution
suffering from severe enterocolitis.
The main concern for patients operated on for
Hirschsprung’s disease is the possibility of suf-
fering from postoperative enterocolitis, which is
dangerous. Because of that, we keep the patients
in the hospital when we start feeding them. We
look for signs of abdominal distention. If the
baby throws up or becomes distended, we take
abdominal films that usually show a characteris-
tic image of a dilated colon with gas and liquid
stool (Fig. 24.13). In that case, we (the surgeons)
personally perform the first colonic irrigation.
In a well-done anastomosis, the catheter should
go straight into the dilated bowel since there is
24.10 Surgical Treatment
no sigmoid. It is a “straight shot” that decom-
presses the baby’s colon. However, in general,
this should not be done by the nurses because the
surgeon must take the responsibility since he or
she is passing the catheter through a new, fresh
anastomosis. Metronidazole is administered
intravenously, and the irrigations are performed
three times a day which keeps the baby comfort-
able and stable. Eventually, the parents learn to
do this procedure. Once the baby is stable, not
vomiting and thriving, and the parents know how
to do the irrigations, we discharge the patient
home. The baby goes home taking metronidazole
by mouth and receiving three irrigations per day.
In addition, the mother is instructed to irrigate
more often if she feels that the baby needs it. We
have been very much influenced by Dale Johnson
et al. [104] concerning the prevention and early
management of postoperative enterocolitis, with
proactive rectal irrigations.
A month after surgery, the patients come to
the clinic and we take an abdominal x-ray film. If
the colon is not distended, the baby is eating and
thriving, and there are no signs of enterocolitis,
we decrease the number of irrigations from 3 to 2
and decrease the amount of metronidazole by
50 %. A month later, the baby comes back again
and we do exactly the same. If the baby is doing
well, we keep reducing the number of irrigations
and the amount of metronidazole. Most babies
will do well. It may be necessary to administer
laxatives at the same time that the number of irri-
gations is reduced. Gradually, we expect the par-
ents to report to us that the baby has spontaneous
bowel movements, in between irrigations, as well
as obtaining less liquid stool during the
irrigations. These are indications of the patient’s
colonic motility improvement. Occasionally, we
will find babies that cannot tolerate the decrease
of rectal irrigations or lower dosages of metroni-
dazole. Sometimes the treatment continues for
months. Under those circumstances, if a child is
“irrigation and metronidazole dependent,” we
consider a reoperation to resect more colon, even
knowing that it is a normoganglionic bowel.
Enterocolitis represents the greatest risk for
patients suffering from Hirschsprung’s disease
[105, 106]. It also represents the greatest research
415
challenge for the new generation of pediatric
surgeons. Several interesting papers have been
published shedding some light on the possible
etiology of the enterocolitis. Changes in the
mucin composition of the bowel, an important
mechanical and chemical factor of the mucosal
defense mechanism, have been advocated as a
possible cause [107, 108]. Also, cell activation
seems to play a role [109]. The role of bacterial
proliferation certainly contributes to the problem,
particularly Clostridium difficile [110].
We are aware of important predisposing fac-
tors [106] including Down syndrome, long-
segment aganglionosis, and infection. We believe,
like others [106], that fecal stasis is one of the
most important predisposing factors.
We do not know why babies suffer from entero-
colitis. This is what we call a non-preventable and
non-predictable complication. After following
many patients, we have had the opportunity to
make an interesting observation. Some patients
come to us from centers where they claim they
have zero incidence of enterocolitis. Interestingly,
most of those patients suffer from fecal inconti-
nence. We also know that patients with colosto-
mies done in normoganglionic bowel do not have
enterocolitis. We postulate that an operation that
damages the sphincter mechanism producing
fecal incontinence ironically protects the patient
against the risk of enterocolitis. The stool flows
out with no obstruction and thus no stasis (like a
colostomy). One can ask a tongue-in-cheek ques-
tion: what do we want: incontinence or enteroco-
litis? In our series, many patients (perhaps 70 %)
underwent the operation that we have already
described and did not suffer from enterocolitis.
We do not know why some do and some do not.
We believe that myectomies [111, 112] or
injections of botulinum toxin [113, 114] into the
area of the sphincter play only a temporary role
in the treatment of this problem. These kinds of
treatments may produce a temporary improve-
ment, because they decrease the efficiency of the
sphincter mechanism. Repeated myectomies and/
or Botox injections eventually may avoid entero-
colitis in a fecally incontinent patient! We prefer
to be proactive with irrigations and administra-
tion of metronidazole. The vast majorities of
416
a b
Pre-irrigation
Fig. 24.14 Irrigation in cases of enterocolitis. (a) Contrast enema of a patient with enterocolitis. (b) Diagram showing
a colon, pre- and post irrigation
patients eventually improve, do not require irri-
gations, and do not require metronidazole.
Figure 24.14a shows a contrast enema taken in
a patient with enterocolitis. Figure 24.14b shows
a diagram of a colon before and after irrigation.
We try to make the parents of our patients
paranoid about enterocolitis. A typical exceed-
ingly sad event in pediatric surgery is when a
child gets sick at home with enterocolitis and dies
while being transported to a hospital. We tell the
parents about this and they learn to recognize the
first symptoms of mild enterocolitis. We do not
wait until the patient has a full clinical picture of
enterocolitis to start irrigations; we rather make
the parents experts in the prophylactic use of
these irrigations. Often, this aggressive approach
with irrigations in response to abdominal disten-
sion keeps the patient from getting sick at all. We
look forward to hearing good news from scien-
tists who are doing studies about the origin of
the problem of enterocolitis. Unfortunately, the
problem of enterocolitis post-Hirschsprung’s
operation is frequently unknown to many pedia-
tricians or simply confused with gastroenteritis.
As a consequence, we have seen many babies
24 Hirschsprung’s Disease
Post-irrigation
suffering from this condition, seen and treated by
pediatricians, simply receiving intravenous fluids
and antibiotics, which may actually aggravate
the problem, particularly when the baby does
not receive the benefit of the most important life-
saving therapeutic maneuver, which is the rectal
irrigation.
A small group of patients, after having a suc-
cessful operation for Hirschsprung’s disease,
develop constipation without enterocolitis. We
believe that leaving a very dilated normogangli-
onic bowel, particularly in patients operated
using the Duhamel technique, may explain some
of the cases that suffer from constipation after an
operation for Hirschsprung’s disease.
The great majority of patients that had a success-
ful operation for Hirschsprung’s disease have a char-
acteristic defecation pattern consistent with the loss
of the rectosigmoid. Under normal circumstances,
the rectosigmoid represents our reservoir for stool
where a significant absorption of water occurs, mak-
ing solid stool. The colonic motility is more active
in the proximal (cecum, ascending) portion and less
active distally (descending colon and rectosigmoid).
In other words, the right colon moves faster than
24.10 Surgical Treatment
Fig. 24.15 Swenson operation (a) diagram showing lines of resection, (b) recto-sigmoid dissected and pulled through,
(c) finished operation
the transverse; the transverse moves faster than the
descending, which moves faster than the rectosig-
moid. Actually, the rectosigmoid remains quiet most
of the time. It just receives and stores the stool dur-
ing 24 or 48 h. In a normal individual, after 24–48 h,
the rectosigmoid starts having peristaltic activity,
giving signs of an imminent massive contraction to
expel the stool, after which it remains quiet again
for another 24 h. This is extremely important and
valuable for us as human beings, because it allows
us to have a social life and not to need the use of a
toilet frequently. Resecting the rectosigmoid makes
this function disappear. During a rectosigmoid resec-
tion (like the one performed for the treatment of
Hirschsprung’s disease), a more proximal portion of
the colon (usually descending) is anastomosed to the
upper portion of the anal canal. The colon that we
pull down does not behave like the rectosigmoid; in
other words, it does not act like a reservoir, but rather
tends to pass stool slowly but constantly, like a colos-
tomy. The more proximal the colostomy, the more
constant the passage of stool and the more liquid the
consistency of the stool. Therefore, the more colon
we resect, the more efficient anal canal (sensation and
417
sphincter) the patient must have in order to preserve
bowel control. When these operations are performed
in little babies and the proximal colon is connected to
the anal canal, the tendency of the baby after surgery
will be to have very frequent bowel movements that
will give him a diaper rash which is very difficult to
treat. We treat this condition by giving a constipating
diet, sometimes loperamide, and observing regular-
ity in meals trying to decrease the number of bowel
movements. We assume that less frequent meals will
result in less bowel movements, which will make the
management of the diaper rash easier.
24.10.2 Other Surgical Techniques
for the Treatment
of Hirschsprung’s Disease
The original operation of Swenson consisted
in the resection of the aganglionic portion of
the colon using an abdominoperineal approach
(Fig. 24.15). The aganglionic segment of the
bowel was mobilized transabdominally below
the peritoneal floor and a meticulous dissection
418
a
f
Fig. 24.16 Duhamel operation (a) Diagram showing
lines of resection, (b) megacolon resected, retrorectal pre-
sacral dissection, (c) normoganglionic bowel pulled
down, (d) incision above dentate line, (e) normogangli-
of the bowel was performed, staying as close
as possible to the rectal wall to avoid damage
to pelvic structures and important nerves. The
dissection of the rectum includes ligation of the
hemorrhoidal vessels and the extrinsic blood
supply of the rectum. Once the rectosigmoid has
24 Hirschsprung’s Disease
c
g
onic bowel pulled down through rectal incision, (f) creat-
ing a wide communication between rectum and colon
using a stapler, (g) finished operation
been dissected through the abdomen, it is everted
through the anus, and the resection is performed
under direct vision outside the anus. The rectum
is passed inside out, and an anastomosis between
the normoganglionic bowel is performed out-
side the anus, away from the pectinate line. Even
24.10 Surgical Treatment
when some authors claimed that this technique
may provoke damage to important pelvic nerves,
Swenson himself and his followers [115] claim
that when this technique is performed in a tech-
nically correct way, the results are good and
no damage is inflicted to the pelvic organs. We
believe this is true.
Duhamel from France [18] devised a very inge-
nious and interesting technique. The aim was to
avoid the potential complications of the Swenson
procedure. Via laparotomy, he divided the rectum
at the level of the peritoneal reflection, resecting
the aganglionic intraperitoneal portion including
the very dilated proximal colon (Fig. 24.16). He
then selected a normoganglionic piece of bowel
to be pulled down and closed the rectal agangli-
onic stump at the level of the peritoneal reflection.
A plane of dissection was created between the
sacrum and the posterior rectal wall. The poste-
rior anorectal wall was exposed through the anus
and an incision was made about the pectinate line,
through which he passed the normoganglionic
Fig. 24.17 Diagram showing the characteristic “Duhamel” pouch and the operation to resect it
419
bowel that was pulled down behind the rectum
and in front of the sacrum. The rectum was hand
anastomosed to the posterior rectal wall. The
follow-up of those patients demonstrated that the
aganglionic rectal stump suffered frequently from
fecal impactions and became extremely dilated
(Figs. 24.17 and 24.18). To try to avoid this com-
plication, other surgeons [116] modified the pro-
cedure by creating a very wide communication
between the anterior wall of the pull-through’s
normoganglionic bowel and the posterior wall of
the rectal stump. This is now usually done using
a stapler. They claim that by doing this, the typi-
cal dilatation of the rectal stump is avoided. We
believe that is half true, because we have seen
a significant number of cases who underwent a
Duhamel procedure, including the technical mod-
ification to avoid the creation of a pouch, and yet
they suffer from severe constipation and required
an operation [117, 118]. For those who like quick
and technically easy operations, the Duhamel
procedure seems to be ideal.
420
Fig. 24.18 Contrast enema showing the post-“Duhamel” pouch
There is an interesting question that must be
raised at this point: in Duhamel operations, if the
rectum left is really aganglionic, then why for
many times does it become so dilated when actu-
ally we know that aganglionic segments of bowel
never become dilated? Perhaps the answer is to
be found in the fact that many children without
Hirschsprung’s disease received an operation
designed to treat Hirschsprung’s.
Franco Soave, in Italy [24], and Scott Boley
in the United States [25] applied the endorec-
tal, submucosal dissection principle to treat
Hirschsprung’s disease. Through a laparotomy,
they resected the aganglionic intraperitoneal
portion of the bowel as well as the most dilated
part of the proximal colon. They also selected
the normoganglionic piece of bowel to be pulled
down (Fig. 24.19). An endorectal dissection of
the aganglionic segment was performed trans-
abdominally, from the peritoneal floor down to
the anal canal. The dissection is carried down
relatively easily and continues distally until the
surgeon “feels” that he is reaching the area of
the anal canal. At that point, the surgeon goes
24 Hirschsprung’s Disease
through the rectum and initiates a submuco-
sal dissection that is basically similar to the
transanal initial approach to meet the dissec-
tion that came from above. Once the resection
is completed, the normoganglionic bowel is
pulled down through the seromuscular cuff of
the aganglionic bowel and the anastomosis is
supposed to be performed above the pectinate
line. In the original Soave technique [24], the
normoganglionic bowel pulled down was left
hanging outside the anus. One week later, the
surgeon could evaluate the blood supply of the
pulled-down bowel and perform the resection
and anastomosis. Boley, however, performed a
primary anastomosis [25], proposing a resec-
tion and anastomosis done in a single opera-
tion. Again, Soave and Boley designed this
technique for the specific purpose of avoiding
potential damage to pelvic structures. They
considered that the submucosal anorectal plane
represented a safe plane to avoid the damage
and denervation of pelvic nerve organs that had
been observed with the Swenson approach. The
“Soave procedure” is more technically demand-
24.10 Surgical Treatment 421

Fig. 24.19 Soave technique (a) lines of resection, (b) endorectal disection, (c) finished endorectal disection, (d) nor-
moganlgionic colon anastomosed to rectum above the dentate line

ing than the Duhamel one. Complications with
this technique have been reported and consist
of leaving pieces of mucosa deep in the pelvis
producing mucus and then generating abscesses
and multiple fistulas that are very difficult to
treat [118]. We receive a number of cases oper-
ated on with the Soave technique that came to us
because they suffer from abscesses and fistulas.
We had to explore those patients posterior sagit-
tally or transanally and found islets of mucosa
or sometimes even complete pieces of bowel
left trapped between the seromuscular cuff and
the bowel wall of the normoganglionic pulled-
through bowel [118]. Some patients underwent
an attempted repair for Hirschsprung’s and suf-
fered from dehiscence and retraction. When we
422
Fig. 24.20 PSARP for frozen pelvis in Hirschsprung’s
studied them, we found that they have severe
fibrosis (“frozen pelvis”) and a blind rectum.
Those cases are approached posterior sagittally
(Fig. 24.20). We have seen many others with a
retained seromuscular cuff, partially obstructing
the normoganglionic pulled-through bowel. We
must also say that from all patients that came
to our clinic complaining of fecal incontinence
after having an operation for Hirschsprung’s
disease at another institution, those who under-
went a Soave operation had a higher frequency
of incontinence, whereas those who underwent
a Duhamel operation tend to suffer more from
constipation.
24.11 Total Colonic Aganglionosis
Total colonic aganglionosis is a very serious
form of Hirschsprung’s disease. Even when
these patients are treated in a technically cor-
rect way, they still suffer from very significant
sequelae [119, 120]. We believe that we are
not really curing this condition. As previously
mentioned, this condition is defined as the
absence of ganglion cells in the entire colon.
Sometimes the aganglionic segment includes
24 Hirschsprung’s Disease
part of the terminal ileum. These patients must
be subjected to a total colectomy and an ileo-
proctoanastomosis, with or without patches or
pouches. The consequences of these procedures
can be easily predicted. The patients suffer from
severe and constant diarrhea for life. In addi-
tion, they will be particularly prone to dehydra-
tion when suffering from any kind of condition
(viral gastroenteritis) that gives them diarrhea.
And, they have a higher tendency to suffer from
enterocolitis.
Adult colorectal surgeons as well as pediatric
surgeons, dealing with patients subjected to total
colectomies for different reasons, have been
trying to be very creative in dealing with the
problems of diarrhea and very frequent bowel
movements. Dr. Lester Martin [121] proposed an
interesting idea to deal with this problem. A
technique similar to the one used by Duhamel is
performed, except that the aganglionic segment
left includes the entire descending colon and
rectosigmoid. The normoganglionic terminal
ileum is pulled through, behind the rectum.
A very long longitudinal anastomosis between
the normoganglionic terminal ileum and
the aganglionic rectosigmoid is performed
(Fig. 24.21). The rationale of this operation was
24.11 Total Colonic Aganglionosis
Fig. 24.21 Lester Martin technique
that the water absorption capacity of the agangli-
onic rectosigmoid could be preserved, as well as
its reservoir function, therefore reducing the
number of bowel movements and creating formed
stool. At the same time, the hope was that the
patient would benefit from the peristalsis of the
normoganglionic terminal ileum.
Subsequently, Kimura et al. [122] proposed a
technical modification to Martin’s original idea,
creating a patch using the right ascending colon,
due to its great capacity to absorb water.
Kimura’s procedure was rather complex and
required at least two surgical stages. Consequently,
Boley modified the procedure to do it in a single
operation [123].
Dr. Orvar Swenson argued that the straight
ileoanal anastomosis was a good procedure and
presented two long-term survivors [124].
423
Subsequently, long-term follow-up studies
showed that the creation of colonic patches, in an
attempt to absorb water, promote formed, solid
stool, and decrease the number of bowel move-
ments, did not improve the clinical conditions of
these patients [125–127].
In adults, small bowel pouches have also
been used in cases of total colectomies per-
formed for the treatment of familial polyposis
and inflammatory bowel disease. Yet, patients
with Hirschsprung’s disease are different from
otherwise normal individuals who undergo total
colectomies. As usual, every time we humans try
to be very smart and cheat on Mother Nature, we
very soon learn that things are usually more com-
plicated than what we thought. Producing stasis
of stool in the small bowel, in general, is not well
tolerated, and this is particularly true in patients
with Hirschsprung’s disease. Stasis of stool in
the small bowel in patients with total colonic
Hirschsprung’s disease frequently produces pro-
liferation of bacteria (frequently Clostridium
difficile). This produces a severe inflammatory
process of the bowel, and rather than having the
expected absorption of water and forming solid
stool decreasing the number of bowel move-
ments, it frequently produces the opposite effect,
which is secretory diarrhea and a severe inflam-
matory process with ulceration, bacterial prolif-
eration, and endotoxemia.
We have been very disappointed by the use of
those patches and pouches, and therefore, we
prefer a direct end-to-end anastomosis between
the terminal ileum and the rectum, located 2 cm
above the anal canal. We are biased against
patches and pouches due to the fact that we have
taken care of patients suffering from complica-
tions consecutive to that kind of operations.
Several of those cases required chronic infusion
of IV fluids to compensate for the great intestinal
losses. Even if diverted with an ileostomy, we
have seen a defunctionalized severely inflamed
pouch, in a patient with severe secretory diarrhea
through the ileostomy, likely from some humoral
factor released by the inflamed patch. When the
pouch with chronic enterocolitis was removed,
the ileostomy output dropped dramatically.
424
Because of this experience, plus other reasons
that will be explained, our approach to the treat-
ment of total colonic aganglionosis is very differ-
ent to the approach proposed by most surgeons
[128]. If the baby is in good condition, we do not
hesitate to perform a transanal resection of the rec-
tosigmoid followed by a laparotomy to resect the
entire colon, pull through the normoganglionic
terminal ileum, and perform a two-layer anasto-
mosis to the rectum 2 cm above the anal canal (as
previously described here). In addition, we open a
protective ileostomy. The ileostomy is not closed
until the patient fulfills two main conditions:
A. He or she is toilet trained for urine and can sit
on a potty.
B. He or she allows the mother to pass a catheter
through the rectum to do rectal irrigations.
This means that we close these ileostomies
usually in patients that are 3–5 years old [128].
This is very different to the current trend of oper-
ating on patients with congenital malformations
as early as possible. The reason for the manage-
ment plan that we propose is that we have seen
many patients with total colonic aganglionosis
operated early in life. We surgeons are very proud
of the fact that we can perform major operations
in the newborn period. Yet, in dealing with total
colonic aganglionosis, when these operations are
done in very young babies, they suffer from con-
stant passing of stool in the underwear and
develop one of the worst diaper rashes that we
have seen (Fig. 24.22). The surgeons usually do
not become aware of this, since it is the mothers
and the nurses who desperately try to control
these kinds of problems. The babies, sometimes,
suffer for years from what seems to be almost
like a second-degree burn of the buttocks. On the
other hand, the presence of an ileostomy some-
times upsets the surgeons and the parents,
whereas the babies are happy. The baby grows up
without enterocolitis and does not care about
having an ileostomy. He/she becomes aware of
the ileostomy only when he/she goes to school.
Before that, in general, he/she is well adapted.
Care of a longstanding ileostomy requires careful
observation for dehydration and monitoring of
sodium losses, often necessitating oral sodium
supplementation.
24 Hirschsprung’s Disease
Fig. 24.22 Dramatic diaper rash
We like to wait until the baby is toilet trained
for urine because at that time, he/she knows how
to verbalize his/her desire to go to the toilet. He/
she has the capacity to hold the urine and to go to
void when necessary. In addition, he/she is accus-
tomed to being clean and dry in the underwear,
and therefore, he/she has a greater chance to
become toilet trained sooner after the ileostomy
is closed. We all know that otherwise normal
adult individuals, subjected to total colectomies
and ileoproctoanastomosis (even when the opera-
tion has been done in a technically correct man-
ner), sometimes have “accidents” (passing stool
accidentally in the underwear), particularly dur-
ing the night. The very active, constant peristalsis
(hypermotility) characteristic of the small bowel,
when connected to the rectum, even if it is done
in a technically correct way is not tolerated by
little babies and that is why they keep passing
stool constantly, producing a severe and non-
manageable diaper rash. On the other hand, a
3–5-year-old child that is toilet trained for urine
already knows what the toilet is all about and
therefore has more possibilities to become toilet
trained for stool very soon. The parents of our
patients treated this way express a high degree of
satisfaction. Yet, the patient still has to go through
a period of many bowel movements in the under-
wear. The number of bowel movements decreases
with time because the patient learns to hold the
stool. The terminal ileum that we anastomose to
the anal canal, as time goes by, becomes dilated
and populated by abnormal flora; therefore, they
24.13 Problems, Complication, and Sequela Secondary to Operations for Hirschsprung’s Disease
have a high incidence of enterocolitis. That is
why we created the second condition for a patient
to be eligible for an ileostomy closure: that the
child must tolerate rectal irrigations prior to the
ileostomy closure. Interestingly, in retrospect, we
found two authors that follow a similar strategy
concerning the timing of the ileostomy closure
[129, 130].
After the ileostomy has been closed, we watch
the patient very closely, as previously described,
every time the patient feels sick or suffers from
abdominal distention and/or vomiting, the care-
giver performs a rectal irrigation that helps the
child feel better and to recover the appetite. In a
characteristic patient with total colonic agangli-
onosis, the parents learn to hear the bowel noises
from far away. In addition, we monitor radiologi-
cally the degree of bowel dilatation, as well as the
mucosal irregularities as signs of imminent
enterocolitis and, when necessary, start irriga-
tions three times daily, as well as administer oral
metronidazole proactively.
One month after transanal operations, we like
to do a rectal exam in all our patients to be sure
that the anastomosis is not getting narrow.
Occasionally, we may feel a rather tight ring at
the location of the anastomosis that may require
anal dilatations. Anal dilatations in patients with
anorectal malformations are started 2 weeks after
surgery, whereas in patients with Hirschsprung’s,
we specifically recommend starting 1 month
after, because we are concerned about a potential
perforation done with the dilator at the suture line
that is located deep in the rectum. In anorectal
malformations, the suture line is located at the
level of the skin; that is the reason why we are
more liberal about early anal dilatations; we have
never seen an injury of the anoplasty provoked by
an anal dilatation.
24.12 Ultrashort-Segment
Hirschsprung’s Disease
As we mentioned at the beginning of the chapter,
we believe that the existence of this condition is
highly debatable. From the clinical and radiologi-
cal point of view, patients who have been labeled
425
with the diagnosis of “ultrashort Hirschsprung’s”
have manifestations that do not allow us to
differentiate them from those who suffer from
idiopathic constipation and “achalasia of the
internal sphincter.” For a more comprehensive
discussion on this subject, see Chap. 25.
24.13 Problems, Complication,
and Sequela Secondary
to Operations
for Hirschsprung’s Disease
We believe that complications that occur after
operations for Hirschsprung’s disease as well as
sequelae can be divided into three categories:
A. Preventable
B. Nonpreventable
C. Partially preventable
24.13.1 Preventable Complications
(Catastrophes)
These complications are consecutive to poor sur-
gical technique. Those of us who are responsible
for the training of young pediatric surgeons that
will go into practice and perform these proce-
dures should have as our goal to take the number
of preventable catastrophic complications down
to zero. In this category, we include patients who
have suffered from acquired rectourethral and
rectovaginal fistulas, neurogenic bladder, rectal
stricture, fecal incontinence, chronic abscess and
fistula, and severe fecal retention with a rectal
pouch in cases of Duhamel operations. Some of
the patients that we have seen have what we call
a frozen pelvis. They previously had multiple
operations, abscesses, fistulas, strictures, or
acquired atresias of the distal pull-through
bowel. For those cases, we sometimes use the
posterior sagittal approach but often can do the
redo procedure transanally with or without a
laparotomy. The posterior sagittal approach is
particularly useful when we believe that through
an abdominal operation, we would not be able to
reach the lowest part of the rectum. Also, it is
useful when a transanal approach is not possible
426
due to the fact that the patient has a “cement”
type of pelvis because of the severe fibrosis. It is
in those cases that we use a posterior sagittal
approach to obtain a direct view of the troubled
area (Fig. 24.20). We have resected remnants of
rectal mucosa or pieces of trapped bowel left in
the pelvis, after attempted, failed endorectal dis-
sections. With this approach, we can perform an
end-to-end anastomosis under direct vision. We
have been able to close acquired rectovaginal as
well as rectourethral fistulas under direct vision.
Also, it is possible to mobilize normal bowel to
be sure that we leave a normal bowel wall in
front of the vaginal or urethral repair. We
strongly recommend the use of a protective
colostomy if one is obligated to use a posterior
sagittal approach to repair these cases. Not open-
ing a colostomy exposes these patients to suffer
Fig. 24.23 Exposure of the posterior rectal wall during a posterior approach. Internal sphincter?
24 Hirschsprung’s Disease
from fistula formation in the location of the
anastomosis of the pull-through rectum and the
anal canal. We must keep in mind that when
using this approach, one is creating a bowel
anastomosis immediately above a normal
sphincter that remains contracted (closing the
anus) most of the time, which acts as a distal
obstruction. In addition, we leave a posterior
midline suture line overimposed on the bowel
anastomosis; all these are predisposing factors
for a fistula formation.
The posterior sagittal approach used for dif-
ferent types of problems gave us a unique oppor-
tunity to be exposed directly to the posterior
rectal wall and evaluate its characteristics
(Fig. 24.23). We have not been able to identify
the thickness of smooth muscle described as
“internal sphincter.”
24.13 Problems, Complication, and Sequela Secondary to Operations for Hirschsprung’s Disease 427

Soiling after Hirschsprung’s

operation

History and physical

contrast enema
exam under anesthesia*
(integrity of anal cana?)
Hypomotility Hypermotility
Constipation Tendency to loose stool

Laxatives Loperamide
Constipating diet
Bulking agents (pectin)
Three meals/day (no snacks)

Incontinence persisted Continent Incontinence persisted

True incontinence with hypomotility In retrospect patient had True incontinence with hypermotility
Pseudo-incontinence

Bowel management program Bowel management program

large enema Small enema, constipating diet,
Bulking agents (pectin), and loperamide

Fig. 24.24 Decision-making algorithm for patients suffering from fecal incontinence after a Hirschsprung’s
operation

24.13.1.1 Fecal Incontinence

For the group of patients that came to us suffering
from fecal incontinence after having an operation
for Hirschsprung’s disease, we have a specific pro-
tocol of management. Figure 24.24 shows our deci-
sion-making algorithm for those patients. We
perform a contrast enema and an examination under
anesthesia. The purpose of the examination under
anesthesia is to determine whether or not the patient
has an intact anal canal and/or to determine the
degree of damage that the anal canal suffered during
the previous operation. One hundred and three
patients come to our center suffering from fecal
incontinence consecutive to an operation for
Hirschsprung’s, and we found 54 cases in which the Fig. 24.25 Bowel-skin anastomosis – no anal canal
surgeons performed an anastomosis of the normo-
ganglionic bowel basically to the perianal skin this kind of anatomy during the examination under
(Fig. 24.25). In other words, the anal canal was anesthesia, we offer the family a bowel manage-
destroyed or removed during the original operation. ment program with a daily enema, most likely on a
Those patients most likely will suffer from fecal permanent basis (see the Chap. 20).
incontinence for life. Sometimes we see a very pat- The contrast enema allows us to determine
ulous anus indicative of overstretching the sphincter whether the patient belongs to the constipated
during the pull-through (Fig. 24.26). When we find (hypomotility), megacolon group (Fig. 24.27) or
428
Fig. 24.26 Patulous anus
Fig. 24.27 Abdominal film showing retention of stool
post-Hirschsprung’s operation
to the opposite (narrow, non-dilated) tendency to
diarrhea, hypermotility type (Fig. 24.28). As we
explained in the chapter about bowel manage-
ment, those groups require a completely different
regimen of bowel management in order to be suc-
cessful incontinence [131, 132].
24 Hirschsprung’s Disease
Fig. 24.28 Contrast enema – spastic, hyperactive colon
If the patient has an intact anal canal, we
believe that the patient has potential for bowel
control. Some patients (49/103) even with intact
anal canals suffer from fecal incontinence, and
we do not know exactly why. However, the con-
trast enema in those cases will tell us whether
the patient suffers from severe constipation and
megacolon (hypomotility) or non-dilated colon
and tendency to diarrhea (hypermotility). The
management of each group is different. We give
all these patients the opportunity to see how
much bowel control they have, helping them to
improve their specific motility problem. In other
words, if the patient has constipation and mega-
colon, we give them laxatives. If the patient has
tendency to diarrhea and a non-dilated colon,
then we administer a constipating diet, pectin,
and Imodium.3 In both groups, we radiologically
monitor the effects of our treatment. The patients
are managed on an ambulatory basis over a period
of 1 week (see Chap. 20). They are expected to
3
Imodium – active ingredients: loperamide HCl 2 mg and
simethicone 125 mg (in each caplet); slows the rate at
which the stomach and intestines move. It also increases
the density of stools and reduces the amount of fluid in the
stool.
24.13 Problems, Complication, and Sequela Secondary to Operations for Hirschsprung’s Disease
Table 24.1 Medical management in patients with fecal incontinence after Hirschsprung’s operation
Patients with constipation
Clean with Remain incontinent in Continent with the
enemas spite of the use of use of laxatives
laxatives
33.8 % 5.8 % 8.8 %
Fig. 24.29 Contrast enema in a patient with retained
aganglionic segment
come to the clinic every day. Abdominal x-ray
films are taken daily to monitor the amount of
stool in the colon, and with that information, we
determine if the amount of laxative that we are
giving is adequate. By doing that, many patients
who suffer from constipation and “incontinence”
now behave like continent patients which means
that perhaps they were actually suffering from
overflow pseudoincontinence.
In cases of patients with a non-dilated colon
with tendency to diarrhea, a decreased borderline
sensation and perhaps a partially damaged
sphincter cannot cope with a hyperactive bowel
that has constant peristalsis trying to pass stool.
Therefore, the use of specific fiber (pectin) that
makes the stool more bulky plus a constipating
diet and Imodium* and regular meals trying to
429
Patients with tendency to diarrhea
Continent with the Clean with the Remain
use of use of enemas incontinent in
constipating diet and constipating spite of enemas
and bulking diet and constipating
agents diet
8.8 % 33.8 % 8.8 %
decrease the number of bowel movements may
make the patients behave like fecally continent.
Table 24.1 shows our results with the manage-
ment of patients with fecal incontinence after
Hirschsprung’s operations. The contrast enema,
in addition, occasionally may show changes
consistent with retention of an aganglionic piece
of colon (Fig. 24.29); in such cases, during the
examination under anesthesia, a rectal biopsy is
done. If the results of the biopsy confirm the
absence of ganglion cells, a secondary resection
and pull-through is performed.
24.13.2 Non-preventable
Complications
We think that enterocolitis is a non-preventable
and non-predictable complication. For us, this
complication represents a mystery. Fortunately,
we know some of the predisposing factors that
contribute to generate this problem of enterocoli-
tis, but we still do not know its intrinsic
pathophysiology.
There is something characteristic about
Hirschsprung’s disease patients; in general, they
do not tolerate fecal stasis. A normal human
being who retains stool develops a clinical pic-
ture consistent with what we call constipation,
but they generally do not develop toxemia and
proliferation of bad bacteria. In Hirschsprung’s
disease, the patients usually behave differently,
as previously mentioned. They tend to suffer
from enterocolitis. So far, our management of
this problem that has been described here includes
only the use of frequent irrigations and metroni-
dazole. If that is not enough, we offer the patients
subsequent resections of bowel, even knowing
that the resection will include normoganglionic
bowel. It is expected that those resections may
430
decrease the chances of the patient to suffer from
enterocolitis. Again, the contrast enema may
show changes consistent with the possibility that
the patient still has a piece of aganglionic
segment.
24.13.3 Partially Preventable
Complications
In this category, we include the problem of con-
stipation. We believe that if we leave an extremely
dilated piece of bowel, even when it is normogan-
glionic, most likely the patients will suffer from
postoperative constipation. The old, demon-
strated principle that a hollow viscus (ureter,
colon, small bowel, and esophagus) that is
allowed to suffer from severe viscus dilatation
will lose its normal peristalsis is applicable here.
A colon that is extremely dilated, by definition,
will suffer from hypomotility, and therefore, it is
expected that the patient will have severe consti-
pation. Therefore, we emphasize the need to
resect not only the aganglionic segment but also
the dilated normoganglionic portion of the colon.
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DA (2009) Comprehensive review of procedures for
total colonic aganglionosis. J Pediatr Surg
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127. Shen C, Song Z, Zheng S, Xiao X (2009) A compari-
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doi:10.1016/j.jpedsurg.2009.07.056
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131. Levitt MA, Martin CA, Olesevich M, Bauer CL,
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353. doi:10.1053/j.sempedsurg.2012.07.009
 Idiopathic Constipation and Other
Motility Disorders
25.1 Definition and Terminology
The term idiopathic constipation refers to the
incapacity or difficulty to pass stool regularly and
efficiently. In addition, we believe that it also
means incapacity to empty the colon.
We use the term “idiopathic” because we do
not know the etiology of this condition. We are
aware of many proposed explanations to under-
stand the pathophysiology of this condition, but
we firmly believe that none of those explanations
have solid scientific basis. In agreement with
Benjamin Disraeli, we believe that “to be con-
scious that you are ignorant is a great step to
knowledge” [1].
We intentionally avoided other terms fre-
quently used in the literature, basically because
many of those names implied an accepted etiol-
ogy. Some of those names include: “puborectalis
spasm syndrome,” “descending perineum syn-
drome,” “chronic obstipation,” and “spastic pel-
vic floor syndrome.” In addition and most
importantly the treatments that we have to offer
to patients with constipation are not different,
regardless of the category or type of constipation
that the patient suffers from.
We also refer to idiopathic constipation as the
central subject of this chapter because, by far, the
greatest number of patients coming to our clinic
Electronic supplementary material Supplementary
material is available in the online version of this
chapter at 10.1007/978-3-319-14989-9_25.
A. Peña, A. Bischoff, Surgical Treatment of Colorectal Problems in Children,
DOI 10.1007/978-3-319-14989-9_25, © Springer International Publishing Switzerland 2015
25
with a colonic motility disorder, requesting a sur-
gical consultation, belongs to this particular
group.
25.2 Incidence, Social Impact,
and Relevance
Idiopathic constipation is by far the most com-
mon defecation disorder and colonic motility
disorder seen in children. It represents a common
cause for surgical consultation [2–4]. It affects
millions of Americans, as well as patients of
other countries, but perhaps most important is
the fact that it is an incapacitating condition
when it is not treated properly. In fact, it pro-
duces a form of fecal incontinence known as
encopresis or overflow pseudoincontinence that
makes the patient socially rejected and
discriminated.
25.3 Etiology
We are aware of multiple publications proposing
different possible causes for this condition.
However, most of those explanations have no sci-
entific basis, and therefore we do not embrace
them. We prefer to take a healthy and potentially
more productive attitude by declaring our igno-
rance about the origin of this condition.
Some authors believe that diet is very impor-
tant as an etiologic factor of constipation [5, 6].
435
436
There is no question that different types of food
have either a laxative or a constipating effect on
our bodies. In addition, we recognize the exis-
tence of personal idiosyncrasies that explain why
one type of food may act as a laxative for one
individual and have a constipating effect for
another one. Although we recognize that diet is
important to regulate colonic motility, we believe
that the therapeutic value of diet is negligible in
the most serious forms of constipation. We must
keep in mind that this chapter belongs to a book
of surgical treatments of colorectal problems in
children. The type of constipation that is manage-
able by diet belongs to the pediatric clinics.
Those patients do not come to our clinic because
they are treated either by a pediatrician or gastro-
enterologist. The patients that come for surgical
consultation are patients that have already
received all kinds of unsuccessful medical man-
agements previously.
There are also many articles [7–12] that try to
explain the problem of idiopathic constipation on
psychological basis. The psychodynamic mecha-
nisms proposed are interesting and sometimes
picturesque; including, strict demanding parents.
Strict demanding parents who impose rigid rules
on a child during the toilet training process, chil-
dren that supposedly retain the stool to manipu-
late the parents to achieve their own purposes. All
these mechanisms may have an element of truth,
but we do not believe that they can explain the
severe forms of constipation in patients with fecal
pseudoincontinence, giant megacolon, some-
times megabladder, serious nutritional and devel-
opmental disturbances, and sometimes death. We
think that it is certainly not easy to retain stool
voluntarily in an otherwise autonomous normal
rectosigmoid with normal peristalsis.
It is true that most patients suffering from
idiopathic constipation also have a psychological
disorder, but we do not think it is a primary one.
Any human being suffering from severe consti-
pation and soiling, ostracized and discriminated
understandably, must have a very significant sec-
ondary psychological problem.
Surgeons, on the other hand, have proposed
different potential mechanisms to explain this
problem. For instance, a rather simplistic expla-
25 Idiopathic Constipation and Other Motility Disorders
nation is that there is a lack of relaxation of the
“internal sphincter” also known as achalasia of
the internal sphincter [13–16]. This is, obviously,
a very attractive and popular idea. In other words,
a simplistic logic dictates that incontinence
means “lack of sphincter”; therefore, constipa-
tion most likely means “too much sphincter.”
However, as discussed in the chapter of ultrashort
Hirschsprung’s, we do not believe that the “acha-
lasia of the internal sphincter” is an entity that
can explain the symptoms of these patients.
Many patients suffering from idiopathic con-
stipation are subjected to rectal manometry.
Many of them have no relaxation reflex. The lack
of relaxation has been described as a diagnostic
of Hirschsprung’s disease, and therefore the next
step is usually to take a rectal biopsy. If the rec-
tum has no ganglion cells, the diagnosis of
Hirschsprung’s disease is confirmed. On the
other hand, if the rectal biopsy shows ganglion
cells, the patient then receives the diagnosis of
“achalasia of the internal sphincter.”
25.3.1 Ultrashort Segment
Hirschsprung’s Disease
Ultrashort Hirschsprung’s disease has been
defined as a condition in which a small length of
the distal bowel has no ganglion cells. The spe-
cific length of this aganglionic zone has not been
defined. Under normal circumstances, human
beings have an area of aganglionosis above the
pectinate line [43–45]. The length of this normal
aganglionic zone has not been well established at
different ages from premature life to the adult
size. Consequently, it is very difficult to know if
a biopsy that shows absent ganglion cells was
taken from this normal aganglionic area of the
rectum. At what point an aganglionic zone is con-
sidered normal and at what point is considered
typical Hirschsprung’s? Both are unanswered
questions.
Many authors believe that this is a common
cause of constipation [39–42]. We believe that
“ultrashort segment Hirschsprung’s disease” and
the so-called internal sphincter achalasia are
highly debatable conditions. From the clinical
25.3 Etiology
and radiologic point of view, patients who have
been labeled with these conditions cannot be dif-
ferentiated from those suffering from idiopathic
constipation clinically or from the radiologic
point of view. In other words, the three groups of
patients suffer from severe constipation, they
have a tendency to soil the underwear when the
constipation is severe, they have a very dilated
rectum, and most importantly, the three groups
respond to the use of laxatives. Those authors
who support the existence of these two condi-
tions (ultrashort segment Hirschsprung’s and
internal sphincter acatalasia) [13–16, 33–40]
claim that they can make the diagnosis based on
manometric studies [17–21] and confirmed by
sophisticated histochemical techniques not
always available to the clinician.
We have serious questions about the existence
of these conditions for several reasons:
25.3.2 Rectal Manometry
During a regular rectal manometry study, a bal-
loon is placed inside the lumen of the rectum, and
the pressure of the anal canal is recorded. Under
normal circumstances, the inflation of the balloon
in the rectum elicits a response from the patient
consisting in a drop of pressure in the anal canal,
which the manometrists interpret as “relaxation
of the internal sphincter.” The first question that
comes to our mind is how do they know that the
internal sphincter is the structure that is relaxing?
If we think about the real anatomy of normal
individuals (see Chap. 2), we find that the so-
called internal sphincter has been defined as a
thickening of the circular layer of smooth muscle
of the most distal portion of the bowel. However,
surrounding the entire rectum, there is an obvious
and powerful striated skeletal muscle structure or
voluntary sphincter mechanism. During very
early manometric studies, some investigators
used paralyzing agents in animals and even in
human volunteers in order to be able to discrimi-
nate the relaxation of the striated muscle, from
relaxation of the smooth muscle (internal sphinc-
ter), and they concluded that it was the smooth
muscle that was relaxing. In addition, they even
437
reached the conclusion that 85 % of the bowel
control depended on the “internal anal sphinc-
ter!” Yet, during all the clinical manometric eval-
uations of patients, muscle relaxants are not used,
and therefore, we do not know if the drop of pres-
sure in the anal canal is due to a relaxation of the
striated muscle complex or to the smooth
muscle.
Another very serious question that comes to
our mind when discussing rectal manometry is
the fact that most patients evaluated manometri-
cally suffer from constipation, and, by definition,
most of them have a very dilated rectum. In order
for the patient to have a relaxation reflex, it is
necessary to stretch the rectal wall with the
inflated balloon. That means that we are sup-
posed to use different volume balloons for differ-
ent patients depending on the size of the rectum.
We are unaware of this kind of methodology.
Most manometries use the same size balloon for
all patients, and certainly we have never seen 1 or
2 L balloons, yet we have seen many patients
with giant rectums. As a consequence, it is con-
ceivable that the inflation of a relatively small
balloon that does not stretch the rectum would
produce a negative reflex. In other words, the
pressure in the anal canal would not drop because
actually the rectum was never distended due to
the fact that they used a standard size balloon in a
megarectum. When we asked these questions to
different proponents of manometry, we do not
have a reasonable answer.
25.3.3 Doubts and Questions About
the Anatomy of the Internal
Sphincter
The “internal sphincter” has been defined as a
thickening of the circular layer of the smooth
muscle of the bowel in its most distal portion, the
anorectum. Yet, real photographs documenting
the presence of this structure are extremely
unusual to see (see Chap. 2). A description of the
specific thickness and upper and lower limits of
this elusive structure at different ages is inexis-
tent. There are plenty of diagrams but very few
photographs, and those photographs do not show
438
the limits of that structure and the way to separate
it from the striated sphincter mechanism.
Recently, endoanal sonography illustrates a series
of circular structures that are interpreted arbi-
trarily with an arrow as “internal sphincter.” In
addition, most of those endoanal study publica-
tions do not specify how much they introduce the
device and how the so-called internal sphincter
looks at different depths in the anal canal and the
rectum.
Our experience in the surgical exploration of
the normal rectum via posterior sagittal for the
repair of urethral problems or tumors did not
allow us to identify a structure that looked similar
to the descriptions of the so-called internal
sphincter (see Chap. 2). With a posterior sagittal
incision, we can identify and clean the entire pos-
terior rectal wall all the way down to the skin and
evaluate the thickness of the bowel wall. We have
been unable to see a thickening of the bowel wall
layer. With the use of an electrical stimulator, we
can clearly differentiate what is a striated muscle
from a smooth muscle, and, again, we have not
seen the so-called internal sphincter. Another
problem is that all anatomic structures have dif-
ferent sizes depending on the patient’s age, and
we are unaware of a study of the characteristics
and size of the anatomic structures such as the
so-called internal sphincter at different ages.
25.3.4 Questions About Myectomy
Technique
The treatment proposed for the treatment of
ultrashort Hirschsprung’s and internal sphincter
achalasia is an operation called myotomy or
“myectomy,” myotomy being just dissection of
the internal sphincter and myectomy a resection
of part of that [22–25]. The description of this
operation is rather vague; the surgeon must make
an incision at the posterior aspect of the mucocu-
taneous junction in the anus and create a plane of
dissection between the mucosa and the posterior
wall of the rectum. All the tissue that remains
between the mucosa and the posterior wall of the
rectum is smooth muscle, and once the surgeon
created those two planes, he is supposed to resect
25 Idiopathic Constipation and Other Motility Disorders
a strip of that tissue. The length of this strip has
not been defined for different ages. That speci-
men should be oriented and should be sent to
pathology. The pathologist is supposed to look
for ganglion cells in a well-oriented specimen,
and he is going to find absent ganglion cells in the
most distal part and present ganglion cells in the
proximal portion. However, again, the question is
how do we know what is a normal length of agan-
glionosis in a human being? We are unaware of
publications describing the specific technique
followed by different pathologists. The study of
the specimens must show that only included
smooth muscle. In addition, looking at the pic-
tures of the intraoperative operations as well as
the description of the surgical technique used by
different surgeons, one gets the feeling that every
surgeon is doing a different technique, and they
do not really know exactly what is included in the
specimen that they send to pathology.
Interestingly, for those who believe that the inter-
nal sphincter is the key for bowel control, it is
difficult to conciliate the concept with the idea of
doing an operation to cut that “important
structure.”
25.3.5 Botulinum Toxin Injection
Recently, the injection of botulinum toxin is
becoming popular to paralyze the “internal
sphincter” and by doing that to improve the
symptoms consecutive to the lack of relaxation of
this structure [102–104]. A detailed, meticulous
description of the injection has not been pub-
lished. The injection is performed rather blindly.
There are some reports on the endosonographic
control of the injection, but again, the fact that we
see an endosonographic image does not tell us
exactly where we are injecting the toxin. The
effect of this toxin, as we know, is to paralyze the
muscle, but it also paralyzes the striated muscle.
How do we know if we are actually injecting the
voluntary sphincter mechanism rather than the
internal sphincter? In addition, the effect of the
injection of botulinum toxin is transient and must
be repeated to continue the effect, and that is
another reason why we are so skeptical about it.
25.3 Etiology
As can be concluded from all this discussion,
until all these questions can be answered in a sat-
isfactory way, we will continue thinking that we
need more scientific and systematic studies to
clarify this rather confusing subject.
There are many publications of authors who
propose the intestinal neuronal dysplasia (IND)
as a potential explanation for some cases of con-
stipation [26–31]. Again, we are rather skeptical
about this. A critical, comprehensive evaluation
of the literature on neuronal intestinal dysplasia
was conducted by us [32]. The most obvious
impression that we obtained from this review was
that there is no basic agreement between patholo-
gists about this histologic diagnosis [33]. In addi-
tion, we are not aware of the existence of
topographic studies that describe the extension of
this histologic disorder in different patients. For a
surgeon to be able to offer a rational treatment for
this condition, we need to know the extent of the
affected bowel that will be resected. In theory
that will cure the patient. This has never been
done or has never been reported. In addition, the
symptoms described in patients with neuronal
intestinal dysplasia vary from patient to patient.
The treatments vary from laxatives to enemas and
to different types of resections, and finally, the
follow-up of the patients has not been consistent.
To complicate the problem even more, some
patients recover spontaneously. We believe that
“neuronal intestinal dysplasia” represents an
interesting histologic disorder that deserves fur-
ther scientific evaluation to try to establish a
truthful clinicopathologic correlation, but at the
present time, the concept has very little clinical
application.
“Hypoganglionosis” has also been invoked as
a potential explanation for patients with severe
constipation [34–38]. Again, we have raised sev-
eral serious questions about the existence of such
entity. We do not know if the number of ganglion
cells remains constant through our lifetime.
Assuming that the number of ganglion cells
remains the same in patients who develop consti-
pation and megacolon, it is conceivable that in a
specimen taken from the colon in a case with a
giant megasigmoid the pathologist will see rela-
tively less ganglion cells in every field and that
439
may give him the impression of dealing with a
case of hypoganglionosis. Yet, what the patholo-
gist really sees in that case is only the result of
stretching a normal ganglionic colon. We have
never heard a coherent answer to this question,
and therefore we believe that at the present time
we have no basis to make such diagnosis. In addi-
tion, the treatment for such condition, assuming
that exists, is the same that we offer for idiopathic
constipation.
As we discussed in the Chap. 24 and specifi-
cally in the paragraph dedicated to “ultrashort
Hirschsprung’s,” most of the patients that come
to our center for consultation for severe idio-
pathic constipation already had manometric stud-
ies of the colon and the rectum, as well as rectal
biopsies, with different inconsistent types of
results. The protocol of management that we pro-
pose for all these patients is the same. We believe
that there is no way clinically to differentiate
those patients with idiopathic constipation and
so-called ultrashort Hirschsprung’s, and we
believe that the treatment should be the same.
We try to follow closely the developments
related to newly described histologic disorders
that may explain the problem of constipation
including a deficiency of the substance P [46],
abnormalities found with the use of monoclonal
anti-neurofilament antibodies [47], and abnor-
malities in the cells of Cajal [48]; also we are
recently learning more about the increased
plasma level of pancreatic polypeptide and a
decreased plasma level of motilin in children
with encopresis [49]. All these deserve future
investigation but at the present time have no clini-
cal application.
We believe that patients with idiopathic con-
stipation are born with a colonic hypomotility,
not a well-characterized disorder, that affects
mainly the rectosigmoid, but also may extend to
the rest of the colon. We also strongly believe that
this is a spectrum type of condition that may
include patients who have very mild constipa-
tion, manageable with diet, as well as very severe
cases that overlap with a condition known as
“intestinal pseudo-obstruction” and may also kill
patients. We also believe that the motility disor-
der frequently affects also the urinary tract simul-
440
taneously. We do not believe that the urinary
problems seen in some of these patients are con-
secutive to the mechanical effect of the dilatation
of the colon, but rather that these patients suffer
from a similar idiopathic malfunction (hypotonic,
large) of the bladder associated to the hypomotil-
ity of the rectosigmoid.
The concept of spectrum of the disease cannot
be overemphasized. Most of the proposed treat-
ments for constipation [50–55] do not take this
concept into consideration. The authors rather
offer standard therapeutic protocols, like if all
patients suffered from the same degree of consti-
pation. In other words, the treatments proposed
are not individualized. Interestingly, the different
modalities of treatment proposed in the literature
[56–64] report percentages of success that varies
from 50 to 80 %, but there is always a group of
patients who do not respond. We believe that this
is another manifestation of a spectrum type of
condition. We also believe that we, pediatric sur-
geons, most likely will be dealing with the group
of patients in whom the traditional therapeutic
strategies failed.
25.4 Pathogenesis
Although we do not know the cause of idiopathic
constipation, we have learned a great deal about
its natural history due to the long-term follow-up
of our patients. Idiopathic constipation is a self-
perpetuating and self-aggravating incurable con-
dition, incurable but manageable. We assume that
the babies are born with a primary hypomotility
disorder that affects mainly the rectosigmoid but
may affect the entire colon. This motility disorder
incapacitates the patient to empty the rectum.
This produces accumulation of stool, which is
responsible for the dilatation of the rectosigmoid
or sometimes other portions of the colon.
We have learned that in patients that are born
with atresias of different hollow viscus, recon-
necting or reanastomosing an extremely dilated,
chronically obstructed hollow viscus to a tiny,
nonused microintestine produces poor results in
terms of function which is attributed to a lack of
peristalsis of the most dilated part of the bowel or
25 Idiopathic Constipation and Other Motility Disorders
hollow viscus. Therefore, the recommendation is
to resect the most dilated part of the bowel or to
taper it. This has been observed when dealing
with the small bowel, colon, esophagus, or ureter.
It seems like it is necessary for the hollow viscus
to have a specific diameter in order for the peri-
stalsis to be optimal and efficient. It seems to us
that the dilatation of the rectosigmoid has the
same effect, affecting the peristalsis. In other
words, retention of stool produces dilatation, and
dilatation produces poor peristalsis. Poor peri-
stalsis produces more retention, and more reten-
tion produces more inefficient peristalsis,
creating a vicious cycle (Fig. 25.1).
Eventually the passage of a large, hard piece
of stool through the anus may produce a lacera-
tion (fissure), understandably producing pain
during defecation. This explains the voluntary
attempt of the patient to retain the stool and avoid
bowel movements. In other words, the patient is
born with a certain degree of rectosigmoid mal-
function, but eventually, another factor is added
to the equation which is the voluntary intention to
hold the stool to avoid pain. This happens often in
these patients, but we do not believe that it is a
primary phenomenon (Fig. 25.2). We believe that
the patient retains the stool because he learned
that passing the stool is painful. Therefore, the
treatment of fissures in patients with constipation
consists in providing the parents with the neces-
sary information, for them to understand the
pathophysiology of this condition. In other
Vicious Cycle
Hypomotility
Megarectosigmoid Constipation
Fig. 25.1 Vicious cycle of idiopathic constipation. Poor
colonic motility produces fecal retention; fecal retention
produces dilatation; rectal and colonic dilatation produces
poor peristalsis which continues producing more severe
constipation
25.4 Pathogenesis
words, in order for the fissure to heal, we have to
guarantee that the patient does not pass a hard
piece of solid stool through the anus, which could
reopen the fissures contributing to the aggrava-
tion of the symptoms. Stool softeners, laxatives,
and time will make the fissure heal, provided the
patient does not have another episode of impac-
tion, passing hard stool which will reopen the
laceration of the anus (fissure).
The concept of incurability of this condition is
also fundamental for a successful management.
Not understanding and not accepting the idea that
this condition is incurable explains in part the
high recurrence rates reported in the literature
[56–64]. Treatments are provided frequently on a
temporary basis based on the rather naive
assumption that the condition is cured.
Subsequently, the treatments are tapered or inter-
rupted, assuming that the patient has been cured,
only to find that the patient suffers a recurrence.
This creates frustration for the patients and par-
ents which may explain why most of these
patients go from institution to institution looking
for an answer. Sometimes, colostomies or ene-
mas are performed, also on a temporary basis.
Opening a proximal colostomy or applying ene-
mas may produce a decrease in the size of the
Vicious Cycle
CONSTIPATION
Voluntary Hard
stool fecal
retaining matter
Anal
Pain fissure
Fig. 25.2 Vicious cycle of patients with idiopathic con-
stipation and painful bowel movements. Poor rectosig-
moid motility produces stool retention. This eventually
becomes fecal impaction, which is the presence of hard
fecal matter in the rectum. When this finally passes
through the anus, it produces a fissure. The fissure pro-
duces pain, and the pain induces the patient to try to hold
the stool and avoid bowel movements, which exacerbates
the stool retention
441
dilated viscera and give the impression that the
patient has been cured. In fact, the patients actu-
ally may show symptoms of improvement after
the colostomies are closed or when the enemas
are discontinued. However, if the patient does not
receive further treatment, symptoms most likely
will come back.
Figure 25.2 shows a cycle of constipation and
megarectum that we believe occurs in these
patients. Many publications support the idea that
the problem of constipation starts during the toi-
let training process [50–55]. We believe that the
toilet training stage of life is rather the time when
the symptoms become more evident; however,
we think the patients are born with this condition.
Babies who are breastfed may not show symp-
toms because of the well-known laxative effect of
the human breast milk. However, when the
breastfeeding is discontinued and the patient
receives formulas and other kinds of food, the
symptoms become obvious. Babies who have
symptoms of constipation while receiving breast
milk most likely suffer from a more severe type
of constipation. Many times, the parents tell us
that the problem started during the preschool
years. However, when we inquire specifically
about the bowel movement pattern since birth,
we frequently find evidence of constipation from
very early in life. Actually, the parents remember
most vividly the episode of the first fecal impac-
tion, and they may refer to that event as the initia-
tion of symptoms. Yet, we all know that a
symptomatic episode of fecal impaction repre-
sents the final step of a chain of events that started
a long time before.
Many pediatricians believe that normal
individuals can go 2 or 3 days without a bowel
movement through life without having any sig-
nificant implications. We believe that that is true
for many human beings; however, in dealing with
patients with idiopathic constipation, it is
extremely important to expect the patient to have
bowel movements every day as a manifestation
of the response to our treatment. Allowing the
patient to go one or several days without bowel
movements would generate again the vicious
cycle that we have been referring to (Figs. 25.1
and 25.2).
442
25.5 Natural History and Clinical
Manifestations
A meticulous, detailed clinical history may show
sometimes that babies did not pass meconium in
the first 24 h and started having symptoms of con-
stipation even when they were young babies and
were taking breast milk. As the patient grows, the
symptoms of constipation become more severe.
The parents describe vividly how the patient suf-
fered for the first time a painful bowel movement
with blood in the stool. After that, the patient
became a “stool retainer”; he goes to a corner of a
room and hides while passing stool, trying to avoid
the bowel movement. The patient refuses to sit on
the toilet because he knows that what follows is a
painful experience. Eventually, the patient has the
first episode of fecal impaction. This is a very
stressful event in the life of the patient and the par-
ents. We refer to fecal impaction as a situation in
which the patient has in the rectum a very large,
solid piece of stool that has been there for days or
weeks. When laxatives are prescribed to a patient
who has fecal impaction, the result is exacerbation
of severe, crampy abdominal pain and sometimes
vomiting. This may resemble the symptomatology
of colonic obstruction. For this reason, we con-
sider it contraindicated the use of laxatives in a
patient with fecal impaction. Occasionally, the
laxatives produce diarrhea, and the patient keeps
passing liquid stool around the impacted fecal
matter (this is known as “paradoxical diarrhea”).
This gives the parents the false impression that
they are overusing laxative and may induce them
to reduce the dosage, which of course, will exacer-
bate the problem.
Usually it is during the preschool or school
age, when the patient starts showing a very bad
prognostic sign which is soiling the underwear
also known as “encopresis.” This is a phenome-
non that we call overflow pseudoincontinence.
Sometimes, constipated patients have 1, 2, 3, 5,
or even 10 bowel movements every day, giving
the parents and the doctors the false impression
that the patient is not constipated. Actually, this
may be a manifestation of a serious constipation
problem. The patient passes a very small amount
of stool, but never empties the rectum. Eventually,
25 Idiopathic Constipation and Other Motility Disorders
he/she will suffer from chronic fecal impaction.
The impaction produces dilatation of the entire
rectum including the anal canal. The anal canal
represents the sensitive part of our bowel that
allows us to know when stool is coming down to
the anus and allows us to determine when to use
our voluntary sphincter. However, we believe that
perhaps the presence of a large mass of solid
stool stretching the rectum on a chronic basis,
eventually, makes the patient accustomed to the
presence of that mass and then starts soiling on a
chronic basis without the patient’s awareness.
This is an ominous sign. This means that the con-
dition has already advanced very significantly
and will require a very aggressive management.
The patient soils the underwear day and night and
basically does not have spontaneous bowel move-
ments. At this stage, the patient basically behaves
like a fecally incontinent patient, with all the
implications that come with this diagnosis. In
other words, the patient smells bad, and the fam-
ily starts fighting with him/her and rejects them.
In addition, the patient becomes accustomed to
his/her own smell and is not accepted at school,
and as a consequence, the patients develop seri-
ous psychological sequelae which we believe
again are not primary.
Eventually, the parents believe that the patient
is intentionally trying to upset them by sitting at
home in the living room, obviously smelling very
badly and not doing anything to solve the problem.
In fact, the patient does not perceive the bad odor.
These ideas in the parents are supported some-
times by the explanations given by psychiatrists in
the sense that they believe that the patient is trying
to manipulate the family by holding the stool
intentionally. The emotional interrelations in the
family are severely affected, and that is when the
psychological problems become worse. We do not
believe that the patients do this intentionally. We
believe that if an individual wants to manipulate
his/her parents, he could select many other ways to
do so. Nobody wants to have stool-stained under-
wear, to smell bad, and to be rejected by society.
The family may put a lot of emphasis on the
lack of cooperation from the patient and make the
patient feel guilty. By the time these patients
come for surgical consultation, they are with-
25.6 Diagnosis 443

drawn, shy, negative, and reluctant to be exam-
ined by the surgeon. They usually have been
subjected to many painful rectal examinations.
They have scars from previous fissures in the
anus. The family is usually in distress. These
patients have also been subjected to unsuccessful
therapeutic programs including biofeedback [65–
67], behavior modification [68–74], and psycho-
logical and sometimes psychiatric treatments
without positive results.
As we will see, a successful, efficient, ade-
quate management of these patients will make
the problem of encopresis disappear. Very occa-
sionally, we see patients that we treat efficiently;
in other words, we are sure that the patient no
longer carries large amounts of stool in the rec-
tum and yet they keep behaving like if they are
incontinent. Those patients deserve a more
meticulous study to rule out neurologic problems
such as tethered cord, spina bifida, tumors, or a
more severe psychological disorder. Fortunately,
this particular situation is rare.
A contrast enema performed with a hydrosol-
uble material (Fig. 25.3) is the most valuable
diagnostic study to confirm the diagnosis of idio-
pathic constipation. The characteristic image of a
contrast enema in a child with a megarectosig-
moid is shown in Fig. 25.4. Most of the times the
dilatation of the colon affects the rectosigmoid all
the way down to the level of the levator muscle
which is recognized because it coincides with the
pubococcygeal line (Fig. 25.4). The lack of dila-
tation of the rectum below the levator mechanism
(pubococcygeal line) should not be interpreted as
a transition zone or non-dilated rectosigmoid.
Unfortunately, we have seen many patients that
suffer from idiopathic constipation; somebody
misinterpreted the radiologic study and errone-
ously treated the patient like Hirschsprung’s
disease.
In cases of idiopathic constipation, the rectum,
above the anal canal, and the sigmoid are extremely
dilated. This provokes an image that has been
described many times in the literature as a “posterior

25.6 Diagnosis

The diagnosis of idiopathic constipation is a clin-

ical one, supported by a radiologic evaluation. A
patient that presents with the symptoms already
described most likely has a problem of idiopathic
constipation. Patients with Hirschsprung’s dis-
ease do not soil. In addition, when left unattended
without surgical treatment, patients with
Hirschsprung’s disease are at risk of dying. They
frequently suffer from severe enterocolitis. The
patients who survive and go undiagnosed with
Hirschsprung’s disease are frequently malnour-
ished and have a history of episodes of enteroco-
litis. Most patients with idiopathic constipation
are well nourished. It is extremely unusual to see
a clinical picture similar to enterocolitis in
patients with idiopathic constipations. We have
seen something similar in cases with extremely
severe idiopathic constipation.
Obviously, the patient must be examined with Fig. 25.3 Characteristic image of a contrast enema per-
formed in a patient with severe idiopathic constipation.
special emphasis in the characteristics of the
Typically the most dilated part of the colon is the rectosig-
anus, to be sure that there is no stricture and/or moid, and the descending and transverse colon seem to be
anterior mislocation of the anal orifice. normal in caliber
444
Fig. 25.4 Megarectosigmoid extending all the way down
to the pubococcygeal line. Below this line, the rectum is
not dilated because it is compressed by the funnel-like
sphincter mechanism. Arrow shows the rectum com-
pressed by the sphincter
shelf” (Fig. 25.4). This “posterior shelf” has been
interpreted by some authors as evidence of an ante-
riorly located anus [75–78]. Many surgeons adopt
this concept and treat these patients on the basis of
that idea. We believe that such diagnosis has no sci-
entific basis. We have never seen a real anteriorly
located anus, defined as a normal anus, nonstric-
tured, with normal anal canal, surrounded by the
sphincteric mechanism 360°. The contrast enema in
patients with idiopathic constipation shows different
degrees of dilatation of the rectosigmoid as expected
in this spectrum of disease (Fig. 25.5a, b). Most
interestingly, most of the times, there is a dramatic
size discrepancy between a normal size transverse
and descending colon and the very dilated megar-
ectosigmoid (Fig. 25.5a). These changes are actu-
ally the reverse from what we see in Hirschsprung’s
disease (Fig. 25.6). In Hirschsprung’s disease, the
aganglionic segment is the most distal part of the
rectosigmoid, and the dilatation is located in the
proximal colon (normoganglionic).
25 Idiopathic Constipation and Other Motility Disorders
We learned that patients with a more local-
ized rectosigmoid dilatation have a better prog-
nosis and respond better to the treatment. When
the patients have a dilatation of the entire colon,
we consider that a bad prognostic sign
(Fig. 25.5b). We formally contraindicate the use
of barium in these patients. The term “barium
enema” is widely used. In addition, adult radiol-
ogists like to use barium because the barium
lines the mucosa of the colon and allows an
accurate diagnosis of mucosal abnormalities
such as diverticula, ulcers, and/or polyps. In our
patients, on the other hand, we are not looking
for mucosal abnormalities; we rather want to see
the degree, location, and extension of the dilata-
tion of the colon, and also in addition, we want
to know how well or how bad the colon empties
after it has been filled up with a contrast mate-
rial. In other words, we want to see sequential
imaging of the filling up of the colon and then a
post-evacuation film.
For many years, we took rectal biopsies in
these patients as part of our routine evaluation.
Now, we have found that study unnecessary
when the clinical picture and the radiologic
images are characteristics. At the present time,
we only perform biopsies when there is a suspi-
cious radiologic image of Hirschsprung’s in the
contrast enema or when the patient clinically
behaves in a way similar to a patient with
Hirschsprung’s disease. As we previously dis-
cussed, we do not perform rectal manometries in
these patients because we believe that it has no
diagnostic or therapeutic value. Total colonic
manometry [79–82] is a promising study. We
would like very much to correlate the site,
degree, and location of the dilatation of the colon
with the colonic manometric abnormalities and
subsequently with the histologic abnormalities.
In the very few colonic manometric studies per-
formed in our patients, we did not find a correla-
tion between the colonic manometric results,
clinical behavior, radiologic findings, and histo-
logic changes. However, we look forward to
doing more studies and learning more from this
diagnostic modality. We look forward to the
improvement of the accuracy of these studies, so
we can use them as an aid for therapeutic pur-
poses, but at the present time we believe are not
25.6 Diagnosis 445

a b

Fig. 25.5 Two different types of colonic dilatation in patients with idiopathic constipation. (a) Dilated rectosigmoid
with normal caliber proximal colon. (b) Generalized colonic dilatation

reliable. We also look into the future to have sys-

tematic, thorough, reliable, histologic studies of
the colonic specimens from bowel resections,
including the rectal portion, the most dilated
part, and the non-dilated part of the colon, look-
ing for all those recently described types of his-
tologic abnormalities, including neuronal
intestinal dysplasia [26–31], hypoganglionosis
[34–38], and “desmosis” [83].

25.6.1 Colonic Transit Time

This is a promising way to evaluate the magni-

Fig. 25.6 Characteristic changes of the rectosigmoid in
a patient with the most common type of Hirschsprung’s
disease. Arrow shows the transition zone
tude and modality of constipation. It has been
done in the past using radio markers [84–87].
More recently, this type of measurement has been
improved, using nuclear scintigraphy [46, 88–
94]. By using this diagnostic modality, Hutson
has been able to identify three transit modalities,
namely, “normal transit,” “slow transit,” and
“functional fecal retention.” He believes that this
study helps to determine the type of treatment
446
that will benefit the patient; “slow transit” patients
may benefit more from an operation, whereas
“functional fecal retention” responds to medical
management. Perhaps even more interesting,
Hutson found that about 80 % of patients suffer-
ing from “slow transit constipation” had reduced
SP immune reactivity in the axons of the colonic
muscle and 6 % had heterotopic ganglion cells or
hypoplastic ganglia on routine histology [46, 89].
Hutson also found that patients suffering from
“slow transit” do not respond to “standard medi-
cal therapy.” All of this represents a very signifi-
cant piece of information and deserves to be
commended.
We have no experience with the scintigraphic
measurement of colonic transit time. However,
our evaluations with contrast enemas tend to
show two different types of images:
(a) Those with dilated rectosigmoid and normal
caliber proximal colon (Fig. 25.5a)
(b) Those with a generalized dilatation of the
colon (Fig. 25.5b)
In general, we observed that those with gener-
alized dilatation of the colon have less favorable
prognosis, and they suffer from a more severe
form of constipation. It is conceivable that this
group can suffer from “slow transit constipation,”
whereas those with dilatation of the rectosigmoid
and normal caliber proximal colon could suffer
from what Hutson calls “anorectal retention.”
Unfortunately Hutson does not describe what
“standard medical therapy” is.
Many of the patients that come to our clinic
have been “resistant to a standard medical ther-
apy.” The parents say that they tried “all kinds of
laxatives” and “nothing worked.” Yet, we try our
protocol of management in all patients and find
that most patients respond. However, the amount
of laxative necessary for them to empty the colon
is usually much higher than what “the book” says
or than “standard” dosages.
We look forward to hear more about the clini-
cal possible implication of the substance P
deficiency.
From our point of view, the treatments of
patients with idiopathic constipation are the
same, regardless what specific type or modality
of constipation they suffer from.
25 Idiopathic Constipation and Other Motility Disorders
25.6.2 The Evaluation of Severity:
Search for Objective
“Instruments”
One serious concern about the literature related
with constipation is the fact that we, the readers,
do not know the severity or magnitude of the prob-
lem that the authors are referring to. Consequently,
we cannot judge their results. Most publications
claim a percentage of success and/or failure with
different therapeutic modalities. Yet we do not
know if the authors were treating patients that
belong to the most benign side of the spectrum,
patients who respond to a change in diet.
We firmly believe that constipation presents in
the form of a spectrum. Benign forms of consti-
pation respond to all kinds of treatments, whereas
very severe forms overlap with “intestinal
pseudo-obstruction” and represent a therapeutic
challenge and a risk of death.
A great deal of enthusiasm has been provoked
by the “Rome III criteria” as a validated measure
instrument for constipation [95–98]. The efforts
of the authors are appreciated. However, we were
very disappointed to learn about the signs and
symptoms used to evaluate the severity of consti-
pation. They included extreme subjective signs
and symptoms such as:
1. “Two or three defecations in the toilet per
week” – the presence and frequency of bowel
movements does not necessarily reflect the
magnitude of the problem. In fact, many
patients have several bowel movements, but
they are fecally impacted.
2. “At least 1 episode of fecal incontinence per
week” – we agree that soiling is a sign of
severity; however, there are cases with severe
constipation without soiling.
3. “History of retentive posturing or excessive
volitional stool retention” – the history of
retention attitude does not reflect the magni-
tude of the problem.
4. “History of painful or hard bowel
movements.”
5. Presence of large-diameter stools that may
obstruct the toilet.
These last three criteria are so subjective that
they do not deserve any comment.
25.7 Management
We believe that the severity of the problem
can be measured by the amount of senna neces-
sary to produce a complete emptying of the
colon, radiologically demonstrated.
25.7 Management
The management plan that we offer to parents of
our patients is based on the following premises:
• Idiopathic constipation is mostly incurable but
manageable.
• It is represented by a spectrum of severity, and
therefore the treatment must be
individualized.
• Most patients considered non-manageable by
“standard methods” respond to an aggressive
management with laxatives.
• The most objective way to know the effect of
our management is with radiologic
monitoring.
• As many as 85 % of our patients have been
manageable. Fifteen percent are candidates
for surgical treatment.
In this chapter, we concentrate our attention
on the treatment of those patients with severe
forms of constipation. Drugs like cisapride sup-
posedly increased the motility of the colon but
in the type of constipation that we are discuss-
ing here, have no good results [99–101]. Some
surgeons use botulinum toxin injected in the
“anal sphincter” to produce relaxation of the
sphincter [102–104]. It is understandable to
believe that this type of treatment may facilitate
the passing of stool, but usually does not solve
the problem of the severe idiopathic constipa-
tion, because the relaxation of the sphincter is
temporary and the primary problem of the
patient has not been solved. In addition, again,
that modality of treatment appeals to those who
believe in the simplistic idea that constipation is
consecutive to the presence of “too much
sphincter.” The problem of constipation seems
to be less simplistic [105].
During the first visit, we give the parents a
specific manuscript designed for them. In that
manuscript, we emphasize the unknown nature
of this condition, the fact that it is incurable and
447
therefore the fact that the patients have to accept
treatment for life or the possibility of an opera-
tion. When the patients come to our clinic, the
parents sometimes feel frustrated by the fact
that we offer them a medical treatment that they
think is not different from previous unsuccess-
ful treatments. We try to convince them that
although we will be using the same medications
(laxative), we are going to use them with a spe-
cific rationale and following a different proto-
col. The first difference consists in that we
adapt the dosage to the patient’s response. The
overwhelming majority of patients with severe
forms of idiopathic constipation that come to
our clinic have been receiving insufficient
amount of laxatives. The parents usually tell us
that they were treated with “ALL KINDS OF
LAXATIVES and none of them worked.” That is
because they were prescribed following what
the book says. We have found that these patients
need 2, 3, 4, 5, and 10 times more laxatives than
what the books usually says, which is a mea-
sure of the magnitude of the severity of the con-
dition. Another very important feature, not
described in the literature, is the radiologic
monitoring of the patient’s response to our
treatment. We become tired of speculating
about the efficiency of our treatment. In other
words, we used to give laxatives to a patient;
the parents came back a few days later to say
how great the results were. In retrospect, those
patients were passing stool, but they were not
emptying their colon. Yet, soon enough, we
were very disappointed because the patient
came back with new symptoms and are fecally
impacted. The only reliable way to know how
much stool is in the colon of a patient is by tak-
ing abdominal x-ray films.
When the patients come to our clinic fecally
impacted (Fig. 25.7), the first step of our routine
is to apply our protocol of fecal disimpaction.
We explain to the parents that the disimpaction
process (Animation 25.1) is going to be cumber-
some and very uncomfortable for both the par-
ents and the patient. We also tell them that if
they follow our instructions, this will be the last
time that the patient will be fecally impacted in
his/her life.
448
Fig. 25.7 Severe fecal impaction in a patient with idiopathic constipation. (a) Abdominal film. (b) Sequence of events
during disimpaction
25.7.1 Fecal Disimpaction Protocol
(Animation 20.4)
Our routine includes the administration of three
enemas per day. The first enema is administered
in the morning and includes the use of saline
solution alone; the second enema (in the middle
of the day) includes saline solution plus phos-
phate, and the third enema (before bed) includes
saline solution plus glycerin. The volume of the
enema depends on the size of the patient and the
25 Idiopathic Constipation and Other Motility Disorders
degree of colonic dilatation and usually varies
from 250 to 1,000 mL of saline solution. The
phosphate (Fleet1 enema) solution amount varies
from half pediatric Fleet to 1 adult Fleet depend-
ing on the patient’s age, and the amount of glyc-
erin also varies from 5 to 30 mL of glycerin,
depending on the patient’s age. Every day the
1
Fleet – monobasic sodium phosphate 19 g, dibasic
sodium phosphate 7 g. C.B. Fleet Company, Inc.
Lynchburg, VA, USA.
25.7 Management 449

patient gets an x-ray film of the abdomen, and

most of the time, by the second day, the patient is
disimpacted. If the patient is not disimpacted
after 3 days of this treatment, then we offer to
bring the patient to the hospital, continue with the
same three enemas per day, and in addition intro-
duce a nasogastric tube and administer
GoLYTELY at a rate of 25 mL/kg per hour until
disimpaction. In the event in which the patient
stays 48 h receiving GoLYTELY and enemas and
is still impacted, we offer them disimpaction
under anesthesia. It is very unusual for us to do
fecal disimpactions under anesthesia because
most patients respond to our protocol. Thirty-two
percent of our patients required the implementa-
tion of this protocol of disimpaction.
Once the patient has been disimpacted as
demonstrated by an x-ray film of the abdomen,
we start the second part of the management which
is the determination of the laxative requirement.

25.7.2 Determination of Laxative

Requirements
We prescribe an arbitrary amount of laxative
(usually senna) that we think is going to work
based on our impression of the degree of dilata-
tion of the colon and patient’s age, and watch the
patient for the next 24 h. If the patient does not
have a bowel movement, it means that the amount
of laxative is not sufficient. The amount of laxa-
tive is then increased, but we also administer an
enema to remove the stool that was produced dur-
ing the previous 24 h. The basic rule is that the
stool, in these extremely constipated patients,
should never remain in the rectosigmoid more
than 24 h because if it stays there, it will become
hard and be more difficult to expel in the follow-
ing days. We continue the routine, increasing the
amount of laxatives and administering an enema
every night, until we achieve our goal, which is to
produce bowel movements and empty the colon
completely as radiologically demonstrated
(Fig. 25.8). At that point, the overwhelming
majority of the patients stop soiling. The patients
and the parents, for the first time, learn what the
real individual laxative dosage for the patient is,
Fig. 25.8 Abdominal x-ray film showing a completely
clean colon once the right amount of laxatives has been
reached
which, as we previously mentioned, represents a
measurement of the magnitude of the condition.
Because we are dealing with a spectrum type of
disease, we find patients with laxative require-
ments much larger than one might expect. In our
series, for school-age children, the average
amount of senna that allowed them to empty their
colon was 69 mg/day with a range of 15–180 mg.
The average dosage of senna that these patients
were receiving prior to coming to our center was
33 mg/day. The most popular medication pre-
scribed for our patients, prior to treatment, was
polyethylene glycol.
Occasionally, in the process of increasing the
amount of laxatives, we find patients who vomit
before reaching any positive effect. In these
patients we may try a different medication to see
whether it is better tolerated, and some patients
vomit all kinds of laxative, feel very sick, and
have severe cramps, and we never reach the
450
amount of laxative capable of producing a bowel
movement that empties the colon. Those patients
are considered medically intractable and there-
fore candidates for surgical intervention.
Approximately 85 % of the time, however, we
find the dosage that the patient needs to empty
the colon completely, as demonstrated radiologi-
cally. Once we have reached that amount, we
expect the patient to stop soiling.
Patients that soil the underwear after reaching
their laxative requirement, with a radiologically
demonstrated empty colon, are very rare. As we
previously mentioned, they deserve a more
detailed study to rule out unusual neurologic or
psychiatric conditions.
Sometimes, the required amount of senna
necessary to empty the colon produces diarrhea.
At that point, the parents know that they not only
reached the necessary amount of laxative to
empty the colon, but also perhaps they went a
little too far in the amount of laxative, and now
they are allowed to reduce a little bit of the
amount of laxative that they were giving. In
addition, once the patient is responding to the
administration of laxatives, it is no longer neces-
sary to give an enema. At this point, the parents,
as well as us, have an idea of the magnitude of
the problem. We usually use senna derivatives
because we found that it is a laxative that has a
more controlled effect. Once we have reached
the decided amount, the parents are given two
choices:
1. To continue with that amount of laxative for
an undetermined period of time, perhaps for
life, and most probably will still have to
increase the laxative as time goes by.
2. The other alternative is to consider the possi-
bility of an operation that may not cure the
condition, but at least will help to decrease
significantly the amount of laxative that the
patient needs.
Confronted with these alternatives, the par-
ents frequently express their concern about the
long-term, secondary effects of the administra-
tion of laxatives, particularly senna. We share
with the parents our concern about the use of this
kind of medication, but unfortunately, so far, we
do not have a better alternative. They may con-
25 Idiopathic Constipation and Other Motility Disorders
sider, rather than giving a single type of laxative,
to mix different types, trying to achieve the same
effect.
In 84 % of our patients, this protocol was suc-
cessful, which meant we reached the amount of
laxative necessary to avoid soiling, keep the
colon radiologically clean, and make the patients
and families happy. Sixteen percent of our
patients did not have a good result; they showed
signs of intolerance to the laxative, as previously
described; and we were unable to clean their
colon.
We also explain to the family that there is no
scientific evidence of senna derivatives produc-
ing cancer [106–110].
25.7.3 Electric Stimulation
Recently, a new modality of treatment is becom-
ing popular [94, 111]. This has been tried in
adults with encouraging results, and some enthu-
siastic doctors are trying it in children. We have
no experience with that methodology. Since the
mechanism of action has not been explained, we
remain skeptical about its use.
25.8 Surgical Treatment
The most common operations offered to these
patients are:
• Continent appendicostomy, cecostomy, or
other kinds of procedures for the antegrade
administration of enemas [89, 112–114]
• Colonic resections [115–118]
• Total proctocolectomy [119–121]
• Combination of resection plus an operation to
administer antegrade enemas [122]
25.8.1 Operations to Administer
Antegrade Enemas (ACE
Procedures)
This type of procedure is perhaps the most com-
mon operation performed nowadays for the treat-
ment of what the authors call unremitting or
25.8 Surgical Treatment
intractable constipation. It is done with different
modalities. The original operation, called Malone
procedure, consisted of using the cecal appendix
and connecting it to the skin of the abdominal
wall in order for the patient to administer enemas
in an antegrade fashion. A plication of the cecum
around the appendix is frequently done with the
goal to create a one-way valve mechanism to
allow the passing of a feeding tube and to prevent
fecal soiling through the orifice created in the
abdominal wall. This original technique has been
modified in an attempt to make it less invasive,
and nowadays many doctors preferred to use but-
ton cecostomies that are very attractive because
they are performed with a minimally invasive
technology; however, they have the inconve-
nience of leaving a foreign body (button) in
place. Many surgeons are extremely enthusiastic
about the use of this procedure [89, 114].
However, there are also reports of rather poor
results with the use of this type of procedures
[123, 124].
We believe that ACE procedures or opera-
tions designed for the administration of ante-
grade enemas only represent a different route of
administration of enemas. We have two con-
cerns about this type of operations when per-
formed in patients suffering from idiopathic
constipation. The first one is that authors say
that they perform those operations in cases of
“unremitting constipation or intractable consti-
pation,” but they do not define what exactly they
mean by that. This means that some of those
patients, conceivably, receive an operation, and
actually they could have been treated medically
and avoided the procedure. In addition, the logi-
cal way to proceed would be to try medical
management following the guidelines that we
expose, and if the patient does not tolerate that,
we should try enemas, and once we demonstrate
that the enemas are working, meaning that they
are capable of keeping the colon completely
clean every day as radiologically demonstrated,
it would be a choice for the patient or the family
to decide whether the enema should be given
from below or from one of these orifices in
the abdomen (see Chap. 21 on “Operations to
Administer Enemas”).
451
25.8.2 Colonic Resection
This type of procedure has been used through the
years and is still used at the present time [115–
118]. The results of these operations, as expected
in dealing with a spectrum type of condition, are
variable, some surgeons being very enthusiastic
and others rather skeptical. Our early experience
included 237 patients medically treated for con-
stipation following our guidelines; 70 of them
underwent a resection of the most dilated part of
the sigmoid (Fig. 25.9). Some of these patients
received the operation because they were really
nonresponsive to the medical treatment. In other
words, they became very sick in the process of
increasing the amount of laxatives, and they did
not pass stool, or in other cases, the parents
elected to have the procedure in an attempt to
decrease the laxative requirement. Approximately
10 % of the patients did not require any more
laxatives after the procedure and had bowel
movements every day with no soiling; 30 % of
the patients decreased the laxative requirement
by 80 %. The remaining 60 % of the patients
decreased the laxative requirement by 40 %. The
resection included the most dilated part of the
sigmoid but respected the entire rectum. In other
words, the colon was resected at the level of the
peritoneal reflection, and a piece of non-dilated
descending colon was anastomosed to the rectum
[118].
As previously mentioned, these operations do
not cure the patients but rather simply improve
them.
In an attempt to find a more radical cure for
this condition, total proctocolectomy has been
performed [119–121]. Obviously, this kind of
operation would have more chances to succeed in
eradicating the problem of constipation; how-
ever, the price that the patient has to pay for this
is very high because the patient will have the
problem of liquid stool for life and many bowel
movements.
Another more conservative approach is the
resection of the rectosigmoid including the most
dilated part of the colon (not a total colectomy),
performing the operation via transanal like in
Hirschsprung’s disease (Fig. 25.10). We have
452
Fig. 25.9 Diagram showing a sigmoid resection. The most
dilated part of the sigmoid is resected, and an anastomosis
is performed between the normal caliber descending colon
Fig. 25.10 Intraoperative picture of resection of a giant
megarectosigmoid
done this in more severe types of constipation.
However, the main concern in those cases is the
possibility of a patient suffering from a mild
degree of fecal incontinence. If this procedure is
done, it should be performed in a very meticulous
way, being sure to preserve the integrity of the
anal canal, performing the anastomosis 2 cm
25 Idiopathic Constipation and Other Motility Disorders
and the rectum at the level of the peritoneal reflection. The
(dashed lines) in the diagram on the left, show the limits of
the resection. The (arrow) shows the finished operation
above the pectinate line and being sure not to
stretch too much the anal opening during the
procedure.
In summary, patients suffering from idio-
pathic constipation are clinically diagnosed at
our center followed by a contrast enema; if both
are characteristic of idiopathic constipation, we
do not performed manometric studies, and we
do not feel the need to take unnecessary rectal
biopsies. The patients then are disimpacted
when necessary, and the next step consists of
finding the amount of laxative necessary to
empty the colon, radiologically demonstrated,
which represents a measure of the severity of
the problem. For those patients (10–15 %) that
demonstrate to be truly resistant to the medical
management, we offer them an alternative of
enemas given through the rectum or through a
Malone or a conservative resection of the most
dilated part of the colon. If this is not enough,
we may consider the possibility of a more radi-
cal transanal resection of the most dilated part
of the colon.
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 Posterior Sagittal Approach
for the Treatment of Other
Conditions
26.1 The Kraske Operation
Early in our experience with the posterior sagittal
approach, used for the repair of anorectal malfor-
mations (1980), we frequently heard colleagues/
surgeons saying that the approach that we were
using was not different from what a German sur-
geon by the name of Kraske had done many years
before during the nineteenth century. In reality,
when we started using the posterior sagittal
approach to repair anorectal malformations, we
had not heard of such a procedure, and we were
not familiar with the name Kraske. Driven by
curiosity, years later, we decided to go into the
old medical and surgical literature looking for the
original publication of Dr. Kraske [1, 2]. Our
findings in such a review were totally
unexpected.
We found multiple old papers referring to dif-
ferent types of “posterior approaches” used to
treat prostatic and rectal rumors. However, many
of them were not midline transsphincteric. In
other words, many surgeons made a parasacral
incision and dissected the rectum laterally to
have access to the urogenital tract.
Table 26.1 shows a summary of our findings.
In 1874, a doctor by the name of Verneuil pro-
posed a coccygectomy in order to resect rectal
tumors [3]. In 1875, Kocher supported the idea of
Verneuil [4]. Then, to our surprise, we found the
publication of Dr. Cripps in 1876, obviously the
first comprehensive paper on the resection of 36
malignant rectal tumors using a posterior sagittal
A. Peña, A. Bischoff, Surgical Treatment of Colorectal Problems in Children,
DOI 10.1007/978-3-319-14989-9_26, © Springer International Publishing Switzerland 2015
26
incision, dividing the entire sphincter mechanism
[5, 6]. This means that if we want to use an
eponym for the posterior sagittal approach, we
should rather use the name Cripps who deserves
the credit for the first midline transsphincteric
incision.
Subsequently, Allingham in 1879 proposed
the same incision, for the treatment of tumors,
plus the use of a colostomy [7]. Interestingly, the
name Kraske appeared much later in the litera-
ture in 1885 [1, 2], but actually even when he
supported the use of a posterior incision, he actu-
ally used a paramedian one, resecting part of the
sacrum and did not propose to divide the entire
sphincter mechanism; therefore, the Kraske
approach is not a real posterior sagittal trans-
sphincteric incision.
In 1917, Dr. Arthur Bevan from Chicago pro-
posed for the first time in the United States a
complete division of the sphincters in order to
resect rectal tumors [8, 9].
Table 26.2 shows a list of surgeons that used
the transsphincteric approach to the rectum for
the treatment of rectal tumors through history
[5–20]. The names Cripps, Bevan, Kilpatrick,
and Mason are underlined, because they pre-
sented the most comprehensive series of cases.
Kilpatrick apparently was a urologist, who
worked together with Dr. York Mason in England,
and they have the largest series of transsphinc-
teric approach used in adults.
It is important to mention that all of these
authors were adult surgeons that used the
457
458 26 Posterior Sagittal Approach for the Treatment of Other Conditions

Table 26.1 History of the posterior approach and different kinds of sequelae from trauma.
Verneuil [3] 1874 Coccygectomy What all those conditions had in common was
Kocher [4] 1875 Supported the idea the fact that the location of the defect or tumor
Cripps [5, 1876 Division sphincters (36 was difficult to access through the abdomen or
6] cases, CA) through the perineum. We thought that a possi-
Allingham [7] 1879 Same plus colostomy ble alternative could be a posterior sagittal,
Kraske [1, 1885 Same plus partial
transsphincteric, trans-anorectal approach that
2] removal of sacrum
Bevan, Arthur [8, 1917 United States, complete
will give us access to the urogenital tract. Prior
9] division of sphincters to trying this approach in human beings, we
performed an experimental study to be sure that
Table 26.2 Transsphincteric posterior approach to the this surgical approach will not affect the bowel
rectum control of the patients, since most of them had
Cripps [5, 6] 1876 England normal anorectum and normal bowel control
Bevan [8, 9] 1917 United States [21]. The study in animals demonstrated that
David [10] 1943 United States the trans-anorectal approach did not affect
Larkin [11] 1959 United States bowel control.
Kilpatrick [12] 1969 Urol. England
Beneventi [13] 1971 Urol. New York
Oh [14] 1972 New York 26.2 Urogenital Sinus
Mason [15] 1972 England with Normal Rectum
Dahl [16] 1974 Urol., Salt Lake
Henderson [17] 1981 Urol., Salt Lake This particular malformation occurs more often
Criado [9] 1981 Baltimore associated to adrenal hyperplasia. Our experience
Westbrook [18] 1982 Arkansas
has been mainly in cases without adrenal hyper-
Madsen [19] 1987 Denmark
plasia. This is a rather infrequent condition.
Jorge [20] 1987 Pennsylvania
Figure 26.1 shows a diagram of the sagittal view
of a patient suffering from this malformation.
transsphincteric approach for the treatment of The rectum is normal, is well located, and has a
rectal tumors. We could not find a publication normal sphincter mechanism. The vagina is con-
prior to ours, in 1982, indicating that this nected to the urethra, creating a urogenital sinus
approach has been used for children and/or for of different lengths. The malformation occurs in
the treatment of anorectal malformations. the form of a spectrum. In other words, we have
Apparently the posterior transsphincteric seen patients with a very short common channel
approach for the treatment of tumors is no longer (low implantation of the vagina) (Fig. 26.1a) as
popular, most likely due to the fact that those well as cases with a higher junction of the vagina
patients suffered from a significant morbidity, and urethra (Fig. 26.1b), including connections
and in addition, modern techniques allowed to directly into the bladder, or even more complex,
treat those conditions in a less invasive manner creating a single vesicovaginal chamber.
and with less morbidity. Figure 26.2a shows a radiologic study consisting
Because of our experience with the posterior of the injection of contrast material into the blad-
sagittal approach, some colleagues referred to der and into the vagina. Figure 26.2b, c shows
us patients suffering from different problems, and MRI study of a urogenital sinus. The length
unrelated to anorectal malformations. These of the common channel, as mentioned when clo-
included urogenital sinus with and without acas are discussed, can only be measured accu-
adrenal hyperplasia, vaginal or urethral fistula, rately endoscopically (cystoscopy).
26.2 Urogenital Sinus with Normal Rectum 459

There are many publications describing dif- made by very distinguished surgeons [22–30].
ferent ways to repair urogenital sinus. Most Those techniques are still useful, mainly to
publications are related to cases of adrenal repair urogenital sinus with a short common
hyperplasia. Significant contributions were channel (less than 3 cm).

a b

Fig. 26.1 Diagram shows a urogenital sinus with normal rectum. (a) Short common channel. (b) Long common
channel

a b

Fig. 26.2 Cystovaginogram of a patient with a urogenital a urogenital sinus with hydrocolpos, h hydrocolpos, v
sinus. (a) Short common channel. (b) MRI study of a uro- vesicostomy, cc common channel
genital sinus with long common channel. (c) MRI study of
460 26 Posterior Sagittal Approach for the Treatment of Other Conditions

Fig. 26.2 (continued)

Fig. 26.4 External appearance of the perineum of a

patient with a urogenital sinus

The trans-anorectal approach is an excellent

Fig. 26.3 Diagram showing the incision used for the
trans-anorectal approach of a urogenital sinus
way to expose areas of the pelvis that are oth-
erwise difficult to approach abdominally or
perineally [31–33]. Figure 26.3 shows the inci-
sion that we used for the trans-anorectal
approach of a urogenital sinus. In the figure,
the patient is placed in the prone position, and
the incision runs from the middle portion of the
sacrum, passing through the anus itself, and
continues all the way down to the single uro-
genital orifice. Figure 26.4 shows the external
appearance of the perineum of a patient with a
urogenital sinus in the prone position. A mid-
sagittal incision is performed using a needle-tip
cautery, changing from cutting to coagulation,
to provide meticulous hemostasis. The incision
continues through the skin, subcutaneous tis-
sue, parasagittal fibers, muscle complex, and
levator mechanism until we identify the poste-
rior rectal wall. The posterior rectal wall is
divided, placing silk temporary sutures, one in
26.2 Urogenital Sinus with Normal Rectum 461

Fig. 26.5 Diagram showing the trans-anorectal incision

(the arrows show the anterior and posterior limits of the Fig. 26.6 Intraoperative appearance of the trans-
sphincter) anorectal approach (the arrows show the anterior and pos-
terior limits of the sphincter), the arrows show the limits
of the sphincter
front of the other, in corresponding locations
of the rectum edges in order to identify them
its of the sphincter. The posterior wall of the
during the reconstruction of the rectum and the
urogenital sinus is divided exactly in the mid-
sphincter mechanism. The incision continues
line to expose the common channel, the ure-
dividing the anterior rectal wall and the sphinc-
thral orifice, and the vaginal orifice. A Foley
ter mechanism, anterior to the anal opening,
catheter is introduced into the bladder.
and runs all the way to the single orifice in the
Figure 26.7 shows a diagram of the anatomic
perineum until we identify the posterior wall of
findings. Figure 26.8 shows a photograph of
the urogenital sinus. Figure 26.5 shows a dia-
the intraoperative findings after the urogenital
gram of the incision, and the arrows show the
sinus has been opened.
posterior and anterior limits of the anal sphinc-
ter. Figure 26.6 shows an intraoperative view Prior to the use of the maneuver called “total
of the same stage of the operation, and again, urogenital mobilization,” the repair of this
the arrows show the anterior and posterior lim- defect consisted in separating the vagina from
462 26 Posterior Sagittal Approach for the Treatment of Other Conditions

Fig. 26.7 Diagram showing the anatomy of the urogenital

sinus, after the posterior wall of the sinus has been divided,
a Foley catheter is introduced through the urethra. Arrows
show the limits of the sphincter
Fig. 26.9 Diagram showing the separation of the vagina
from the urinary tract

the urinary tract (Figs. 26.9 and 26.10). Once the

vagina was separated and mobilized enough to
reach the labia with no tension, the urethra was
reconstructed, using what used to be the common
channel (Fig. 26.11). The vagina was pulled down
behind the urethra and sutured to the neolabia.
The anorectum and anterior and posterior sphinc-
ter mechanism are reconstructed (Fig. 26.12).
In 1997, we described a maneuver called
“total urogenital mobilization” [34], in an attempt
to simplify the repair of cloacas. This maneuver
turned out to be very useful to repair cloacas with
common channels shorter than 3 cm. As a natural
consequence of this, we decided to repair uro-
genital sinuses with a normal rectum, using a
transanal approach combined with the total uro-
genital mobilization (see Chap. 16, Sect. 16.1.1.4
on cloacas).
The total urogenital mobilization was well
received by many surgeons [35–39]. However, as

Fig. 26.8 Intraoperative view of the anatomy. Arrows

show the limits of the sphincter
26.2 Urogenital Sinus with Normal Rectum
a b
Fig. 26.10 Vagina completely separated from the urinary tract and mobilized enough to reach the perineum.
(a) Diagram. (b) Operative
expected, some surgeons, mainly urologists,
voiced their concerns about the trans-anorectal
approach. Consequently, some of them decided
to do the total urogenital mobilization, but not
perform the full trans-anorectal approach, but
rather to divide only the anterior portion of the
sphincter and anorectum [40, 41] or perform the
mobilization without touching the anus [42, 43].
Also, a limited urogenital mobilization was
described [44]. We agree with the basic idea of
being as less invasive as possible, but we also
believe that we should not hesitate to divide the
rectum and anus, in order to achieve our goal of
performing an adequate anatomic repair.
Figure 26.13 shows the operative field of a
urogenital sinus. We started that particular case
463
with an incision that included the sphincter
mechanism located anterior to the anus. We tried
to mobilize the urogenital sinus, as much as pos-
sible without opening the rectum (Fig. 26.13a).
We then found that exposure was not good enough
to achieve a satisfactory mobilization, and there-
fore we elected to divide the entire anorectum in
the midline. Figure 26.13b shows the improved
exposure achieved with the full trans-anorectal
approach. The total urogenital mobilization is
performed with a magnificent exposure. The sus-
pensory ligaments of the urethra and vagina are
divided. By doing that, we gained enough length
to bring the urethra and vagina down (Fig. 26.13c,
d). What used to be the common channel is
divided in the midline, creating two flaps that will
464
Fig. 26.11 Diagram showing the urethra reconstructed
using the original common channel
Fig. 26.12 Diagram
showing the reconstructed
vagina, perineal body,
sphincter mechanism
anterior to the rectum, and
anterior rectal wall. The
posterior rectal wall was
closed using two layers of
interrupted long-term
absorbable sutures. The
sphincter mechanism
posterior to the anus is
meticulously
re-approximated
26 Posterior Sagittal Approach for the Treatment of Other Conditions
be sutured to the neolabia. The urethral meatus is
sutured a few millimeters behind the clitoris
(Fig. 26.13e). Figure 26.14 shows the final exter-
nal appearance.
We must always keep in mind that we are deal-
ing with a spectrum of defects, and therefore, the
surgeon must be prepared to implement different
surgical maneuvers, adequate for each one of the
anatomic variants of the malformation that we
deal with. For very low vaginal-urethral conflu-
ence, the surgeon can use any technique, because
the repair is relatively easy. On the other hand, for
cases with extremely high implantations of the
vagina, such as vaginovesical communication, the
transabdominal approach represents the ideal way
to do it. In the middle of those extremes are mal-
formations shown in these diagrams with com-
mon channels that vary from 2 to 4 cm.
The size of the vagina is also another important
factor. Some patients with this type of urogenital
sinus also have hydrocolpos, like in the patients
with cloacas. The presence of hydrocolpos, of
course, must alert the surgeons about the possibility
of megaureters and hydronephrosis (see chapters on
cloacas). However, the presence of hydrocolpos
also means that the surgeon has more vaginal tissue
26.2 Urogenital Sinus with Normal Rectum 465

a b

c d

Fig. 26.13 Intraoperative view of the repair of a urogeni-
tal sinus. (a) An attempt is made to mobilize the urogeni-
tal sinus, with a limited incision, trying not to open the
rectum. The exposure is limited. (b) Improved exposure
after extending the incision through the anus and rectum.
R divided rectum, u urogenital sinus. (c) Operative picture
of a case with a short common channel. It was possible to
mobilize the urogenital sinus without dividing the rectum.
(d) Operative picture showing a total urogenital mobiliza-
tion. The urethra and vagina have been fully mobilized.
(e) Operative picture. The urethral meatus is sutured a few
millimeters behind the clitoris
466 26 Posterior Sagittal Approach for the Treatment of Other Conditions
e Subsequently, as we gained confidence in these
Fig. 26.13 (continued)
Fig. 26.14 Final external appearance
to work with and more chances to bring the vagina
down. Patients with very small vaginas and long
common channels represent a challenge and may
require a partial vaginal replacement.
The first trans-anorectal operations performed
by us included a protective colostomy.
kinds of techniques, we performed the same opera-
tions without a colostomy. We followed the same
strict rules of bowel preparation (see chapter on
bowel preparation). The patients remained
7–10 days with nothing by mouth, receiving paren-
teral nutrition; our patients did not have infections,
dehiscence, or other kinds of complications.
Our current way to approach these patients is
to determine the length of the common channel
endoscopically. For those that are extremely long
common channels (vaginovesical fistula), we
prepare the patient to be approached through the
perineum and also through the abdomen. In addi-
tion some of those patients may require a partial
vaginal replacement. If we believe that the patient
can be treated trans-anorectally without a lapa-
rotomy (common channel between 2 and 4 cm),
we prepared the bowel as described and take the
patient to the operating room. We start by making
an incision running from the anus to the urogeni-
tal sinus, trying not to open the rectum and see if
it is possible to do the total urogenital mobiliza-
tion without dividing the anterior rectal wall or
the posterior rectal wall (Fig. 26.13a). Sometimes
this is feasible, but if we feel uncomfortable
about the exposure, we do not hesitate to divide
first the sphincter mechanism located anterior to
the anus or even the posterior rectal wall and the
posterior sphincter mechanism (Fig. 26.13b).
The trans-anorectal approach consists in
dividing the anus and the rectum exactly in the
midline. By doing that, we obtain a magnificent
exposure to the urogenital tract. However, the
procedure requires a meticulous bowel prepara-
tion or a colostomy, followed by a delicate, scru-
pulous technique with special attention to details.
Our experience with the repair of a urogenital
sinus with normal rectum includes 16 cases. We
are extremely happy, because bowel control has
been normal in all of them. The patients also
showed evidence of urinary control. None of our
patients reached the age of sexual function, and
therefore, we cannot comment on that. However,
we expect them to have an examination under
anesthesia at the age when they expect to become
sexually active. We will not be surprised to find
that they may need an external introitoplasty like
we do in patients with cloaca, because they have
a ringlike fibrous band that may interfere with
26.4 Acquired Urethral Atresia
satisfactory sexual activity and may require a
small operation to take care of that.
26.3 Urogenital Sinus
with Normal Rectum
and Adrenal Hyperplasia
We have used the same approach in patients with
adrenal hyperplasia. However, we must admit that
these groups of patients (with virilization) are
more difficult to repair. First of all, they require a
clitoral recession. In spite of doing that procedure,
one deals with the presence of hypertrophic cor-
pora that makes the operation technically more
difficult. In addition, the vagina in those types of
patients is usually much smaller, both in length
and diameter. All of that makes the operation
technically more demanding. Our experience with
this group of patients is limited to three cases, and
therefore, we cannot claim that this is the ideal
way to repair this malformation.
26.4 Acquired Urethral Atresia
Male patients, who suffer from severe pelvic
trauma, frequently have severe urethral injuries
that result in an acquired atresia of the posterior
a b
Fig. 26.15 Disrupted posterior urethra, a mild penile urethral stricture and the trans-anorectal incision. (a) Diagram.
(b) Contrast study – injection of contrast done through a suprapubic tube and through the penis
467
urethra. These represent a surgical challenge, not
only to be able to reconnect both ends of the pos-
terior urethra, but also to try to obtain urinary
control after the repair.
Traditional approaches to this problem include
perineal operations [45–49] or transpubic
approach [50–56]. We have a modest but signifi-
cant experience with the trans-anorectal approach
that has also been used by others [57–60].
Six patients have been referred to us suffering
from this type of problem. These patients under-
went several previous attempts to repair, using a
perineal approach, and all of them were unsuc-
cessful. Figure 26.15a shows a cystogram and
urethrogram of one of these patients. The gap
between the blind ends measures over 3 cm. This
study was done injecting contrast material
through a suprapubic tube and simultaneously
through the urethral meatus. This was an adult
patient who suffered a very severe pelvic trauma
that destroyed the posterior urethra. The patient
was ejaculating through the suprapubic tube and
had three previous failed attempts to repair the
problem. Figure 26.15b shows a diagram trying
to show the anatomy of the same patient. In addi-
tion to the disruption of the posterior urethra, the
patient also had a mild stenosis of the penile ure-
thra. The subset of the figure shows the trans-
anorectal incision used to approach this urethra.
468 26 Posterior Sagittal Approach for the Treatment of Other Conditions

A posterior sagittal trans-anorectal incision is

done, dividing, as previously mentioned, the
sphincter mechanism posterior to the anus, as
well as the anterior one, dividing also the poste-
rior and anterior rectal walls. Figure 26.16 shows
a picture of the intraoperative aspect of the trans-
anorectal incision. Both posterior and anterior
rectal walls have been divided. Figure 26.17 is a
diagram showing both blind ends of the urethra
deep in the operative field. Multiple fine silk
sutures are placed in each end of the urethra in
order to apply uniform traction and mobilize
them to be able to create a non-tense end-to-end
anastomosis. Figure 26.18 shows both urethral
ends already open and with the silk sutures
placed. The gap between both ends is about 3 cm,
and therefore both sides require a significant Fig. 26.17 Diagram showing both urethral blind ends.
mobilization. Figure 26.19 shows after the mobi- Multiple fine silk sutures are placed in both ends to mobi-
lize them

Fig. 26.18 Operative field showing both urethral ends

Fig. 26.16 Intraoperative aspect of the trans-anorectal open. The distal one has a metallic sound
approach. Both posterior and anterior rectal walls have
been divided. The urethral ends will be found deep in the
incision
26.4 Acquired Urethral Atresia 469

lization both urethral ends come together, the

Fig. 26.19 Operative field. The anterior wall of the ure-
thra has been reconstructed. A Foley catheter is in place,
and the posterior wall is being sutured
anterior wall has been already sutured, a Foley
catheter is passed through the penis into the blad-
der, and the final step is the reconstruction of the
posterior urethral wall. Reconstructing the
perineal body as well as the rectum will complete
the operation (Fig. 26.20). Figure 26.21 shows
the reconstruction suturing of the anterior rectal
wall with two layers of fine, long-term absorb-
able sutures. The reconstruction of the anterior
rectal wall must be done simultaneously with the
reconstruction of the sphincter. Figure 26.22
shows the completion of the urethral suturing,
perineal body reconstruction, as well as the
sphincter mechanism and rectum. The perineal
body is already reconstructed, as well as the
sphincter mechanism anterior to the rectum and
the anterior rectal wall. The next step will be to
suture the posterior rectal wall and to approxi-
mate meticulously the sphincter mechanism pos-
terior to the anus and rectum. Figure 26.23 shows
a diagram of the completed operation. The patient
shown in this diagram has urinary control, as well
as sexual function. However, when dealing with
this kind of severe pelvic trauma, one cannot

Fig. 26.20 Diagram

showing the urethral
anastomosis and the rectal
reconstruction
470 26 Posterior Sagittal Approach for the Treatment of Other Conditions

Fig. 26.23 Diagram showing the completed operation

Fig. 26.21 Operative view of the closure of the incision,

suturing the anterior rectal wall

Fig. 26.24 Three-dimensional rotational scan of a

patient with an anorectal malformation with an acquired
urethral atresia

Fig. 26.22 Diagram showing the completion of the ure-

thral reconstruction and the closure of the perineal body,
sphincter mechanism, and rectum
26.5 Acquired Rectourethral Fistula
Fig. 26.25 Intraoperative view of the urethral anastomosis
Fig. 26.26 Diagram showing a giant rectourethral fistula
consecutive to the surgical treatment of prostatic cancer
guarantee good functional results in all patients
because each patient suffers from a trauma of a
different magnitude. Two of our six patients suf-
fered from postoperative urinary incontinence
and required another type of urinary reconstruc-
tion, including the closure of the bladder neck.
471
More recently, the anatomy of these complex
conditions can be elucidated better by the use of
a three-dimensional rotational scan, which pro-
vides excellent images, like one shown in
Fig. 26.24. That patient was also approached
trans-anorectally, and Fig. 26.25 shows the intra-
operative findings, as well as the reconstruction
of the urethra. The patient also has urinary
control.
26.5 Acquired Rectourethral
Fistula
Our colleagues, adult surgeons, and urologists
referred adult patients to us. Those patients were
suffering from acquired rectourethral fistula. This
type of problem represents a serious surgical
challenge and has been treated in different ways
[61–63]. Some authors used the transanal ante-
rior approach [64–67], and others adopted the
transanal posterior approach [68, 69].
We had the opportunity to operate on adult
patients who originally suffered from prostatic
cancer and were treated either surgically by
implantation of radioactive seeds or using cryo-
surgery. As sequelae of those treatments, the
patients developed a huge communication
between the rectum and the posterior urethra.
The original urology surgeons unsuccessfully
tried to repair the fistula through the perineum,
and the patients were referred to us. Figure 26.26
shows a diagram of one of these cases. The com-
munication between the posterior urethra and the
anterior rectal wall is extremely large, and some-
times it measures 4 or 5 cm in diameter, making
these kinds of conditions a real surgical chal-
lenge. The dotted lines in Fig. 26.27 show the
lines of resection that we do. The patient is
approached trans-anorectally. The portion of the
rectum located between the dotted lines is
resected, and the upper rectum is pulled down to
be anastomosed above the pectinate line in order
to preserve the bowel control. The goal of the
operation consists in leaving a completely nor-
mal rectal wall in front of the urethral suture, as
the only way to guarantee that the patient would
not suffer a recurrence. Figure 26.27 shows the
472 26 Posterior Sagittal Approach for the Treatment of Other Conditions
Fig. 26.27 Diagram showing the completed repair of the
urethra and a healthy rectal wall left next to the urethral
suture. The rectal anastomosis is performed above the
pectinate line to preserve bowel control
Fig. 26.28 Intraoperative picture. Trans-anorectal
approach, dissection of a giant seminal vesicle. Seminal
vesicle open
completed procedure, including the urethral
sutures and the anastomosis between the upper
rectum and the lower rectum above the pectinate
line.
Our experience includes six patients, two of
them actually had an operation for Hirschsprung’s
disease; the surgeons damaged the posterior ure-
thra, as well as the anterior rectal wall, and the
patients came with anatomic findings similar to
those shown in Fig. 26.28. The other four patients
suffered from prostatic cancer originally. The fis-
tulas were successfully closed, except in one of
the patients who had excessive local radiation
that, we believe, interfered with the healing pro-
cess of the local tissue.
26.6 Giant Seminal Vesicle
There is some confusion related with the termi-
nology and embryogenesis of cystic structures
communicating with the posterior urethra.
Different authors use terms such as “prostatic
utricle,” “Müllerian duct remnants,” and “giant
seminal vesicle.” A good attempt to simplify this
was done by Currarino [70]. In his paper he pres-
ents a series of radiologic images of these abnor-
mal structures.
The treatment of these conditions includes
transvesical approach [71], transurethral fulgura-
tion [72], and laparoscopic approach [73]. Kaplan
et al., following the original idea of Hunt, accu-
mulated significant experience with a posterior
approach without opening the rectum, but rather
pushing it laterally in order to have access to the
posterior urethra [74–77]. Other authors use the
same approach [78–81].
A few surgeons tried the trans-anorectal
approach to resect one of these utricles [82].
Four cases were referred to us. Two of them
suffered from orchiepididymitis of unknown
origin. The patients did not have any other asso-
ciated defects. They had a normal rectum and
apparently a normal urinary tract. The episodes
of orchiepididymitis were treated with antibiot-
ics, but they had several recurrences. A full
urologic evaluation disclosed the presence of a
giant seminal vesicle connected to the posterior
26.8 Acquired Rectovaginal Fistula
Fig. 26.29 Giant seminal vesicle has been resected, and
the urethra will be closed
Fig. 26.30 Perineal appearance at the end of the
operation
urethra. One of the patients had been previously
approached unsuccessfully, through the abdomen
and another one through the perineum. We
decided therefore to approach the problem via
trans-anorectal. Figure 26.30 shows the operative
field in one of these cases. The rectum has been
divided into two halves, and the giant diverticu-
lum is shown. The upper part of it is actually con-
nected to the vas deferens. Therefore, these
patients underwent a vasectomy in order to resect
the giant seminal vesicle, which was dissected
473
down to its neck that connected to the urethra. In
order to do that in a more precise way, we opened
the giant seminal vesicle, as shown in Fig. 26.28;
disconnected the vesicle from the urethra; and
sutured the posterior urethra as shown in
Fig. 26.29. Figure 26.30 shows the final aspect of
the perineum of the baby after the operation. Two
more patients were referred to us with a giant
seminal vesicle, but they also suffered from an
anorectal malformation and severe hypospadias.
In those patients, the approach was not real trans-
anorectal, but rather we approached the giant
seminal vesicle posterior sagittally, directly, at
the same time that we pulled the rectum down to
repair the anorectal malformation. All patients
had bowel control, urinary control, and no evi-
dence of orchiepididymitis after our operation.
26.7 Urethral Tumors
Two patients were referred to us because they
suffered from a rhabdomyosarcoma of the poste-
rior urethra; they both received chemotherapy,
until the oncologists considered they achieved
the maximum reduction in tumor size. The pedi-
atric oncology group requested a biopsy and if
possible a resection of the remnant of the tumor.
The patients were approached trans-anorectally,
and the tumor was resected. One case did not
require the division of the urethra, but the other
one case did. We performed a resection of part of
the urethra with the tumor and reanastomosed
both urethral ends. Both patients preserved bowel
control, but we do not have information concern-
ing the long-term follow-up from the oncologic
point of view.
26.8 Acquired Rectovaginal
Fistula
Two adult patients were referred to us because
they suffered from cervicouterine cancer and
received local radiation. As a consequence of
that, they developed a giant communication
between the rectum and the vagina. Basically,
both the rectum and vagina became a single
474 26 Posterior Sagittal Approach for the Treatment of Other Conditions
Fig. 26.31 Diagram showing a giant rectovaginal fistula
in a patient with cervical cancer subjected to local
radiation
Fig. 26.32 Diagram showing the repaired fistula
chamber, as can be seen in Fig. 26.31. The
patients were considered cured from the cancer,
but were referred to us to try to close that wide
communication. The approach used was similar
to the one described in male patients. The rectum
was separated from the vagina and was divided
above the pectinate line, and we mobilized the
upper rectum enough to be sure to leave a com-
pletely normal rectum in front of the vaginal
Fig. 26.33 Picture showing the perineum of a girl suffer-
ing from an acquired rectovaginal fistula as sequelae of an
operation for Hirschsprung’s disease. The picture was
taken during the repair. A Hegar dilator is passing through
the fistula
suture, as shown in Fig. 26.32. The fistulas were
successfully closed, and the patients preserved
bowel control.
Three little girls were referred to us, suffering
from acquired rectovaginal fistulas, consecutive
to an operation for Hirschsprung’s disease. The
patients had been previously subjected to a Soave
type of pull-through for Hirschsprung’s disease,
and soon after the operation, the parents noticed
that the patients were passing stool through the
vagina. Figure 26.33 shows the perineum of one
of these girls. The diaper rash was extremely
severe, because the stool came out constantly
through the vagina. The patient is in prone posi-
tion, the rectum has been opened posterior sagit-
tally, and a Hegar dilator has been passed through
the wide communication between the anterior
rectal wall and the posterior vaginal wall. The
perineal body was divided, and Fig. 26.34 shows
the urethra, the vagina, and the rectum. The
vagina was reconstructed (Fig. 26.35), and the
rectum was also sutured, being sure not to leave
over-imposed the vaginal suture and the rectal
suture. The patient recovered uneventfully, and
the fistula was successfully closed.
26.9 Rectal Tumors
Fig. 26.34 Operative picture. The perineal body was
opened. The picture shows the internal anatomy. V vagina,
R – rectum
Fig. 26.35 Operative picture. The rectum was mobi-
lized, and the posterior vaginal wall is being pulled down
475
26.9 Rectal Tumors
We have used the posterior sagittal approach to
resect a variety of tumors that affect the rectum
and sometimes the vagina. Figure 26.36 shows
the diagram of a sagittal view of the pelvis of an
adolescent girl who suffered from an “inflamma-
tory pseudotumor” that invaded the rectum and
vagina. This is considered a histologic benign
tumor but highly invasive. In an oncologic center,
she was offered a pelvic exenteration; yet, the
posterior sagittal approach proved to be very effi-
cient to resect the mass, including 75 % of her
rectum and one third of her vagina (Fig. 26.37).
We were able to preserve part of the pectinate
line of her rectum. The patient recovered very
successfully and preserved her bowel and urinary
control. Figure 26.38 shows the external appear-
ance of her perineum 6 months after the
operation.
We were invited to use the trans-anorectal
approach to resect rectal cancers, in adult
patients, localized to the rectal wall, without
local invasion, and located above the pectinate
line. In addition, we used the same approach to
Fig. 26.36 Diagram showing the sagittal view of the pel-
vis. “Inflammatory pseudotumor” invading most of the
rectum and part of the vagina
476 26 Posterior Sagittal Approach for the Treatment of Other Conditions
Fig. 26.37 Same case in which the tumor has been
resected. A portion of the rectum with pectinate line has
been preserved
Fig. 26.38 External appearance of the perineum of the
same patient, 6 months after the operation
resect villous adenomas of the rectal wall. We
like to emphasize the fact that this approach,
when used with this specific purpose, should be
done only if the tumor is localized and is located
above the pectinate line. The rationality of this
Fig. 26.39 Diagram showing a small tumor located
above the pectinate line. The dotted lines represent the
limits of the resection
approach is to be able to preserve the anal canal,
as well as the sphincter mechanism and by
doing that, to guarantee that the patient will
have bowel control, and by doing that, we avoid
the possibility of an abdominoperineal resec-
tion and a permanent colostomy. Figure 26.39
shows a diagram of a patient with a small tumor
located on the anterior rectal wall about the
pectinate line. The dotted line shows the line of
resection preserving the anal canal and the pec-
tinate line. Figure 26.40 shows a diagram illus-
trating the posterior sagittal incision and the
opening of the rectum. The dotted line repre-
sents the line of resection above the pectinate
line. Multiple silk sutures are placed, taking the
rectal wall above the pectinate line in order to
apply uniform traction to facilitate the dissec-
tion of the rectum with the tumor. Figure 26.41
shows the operative field of one of these cases.
The pectinate line is perfectly obvious, and the
line of silk sutures are placed 2 cm above the
pectinate line. Figure 26.42 shows the effect of
uniform traction to facilitate the dissection of
the rectum. The dissection continues, remain-
ing as close as possible to the rectal wall, until
a margin of about 5 cm above the location of
the tumor is reached. At that point, the rectum
is divided as shown in Fig. 26.43 and the upper
26.9 Rectal Tumors 477

Fig. 26.40 Diagram

showing the posterior
sagittal approach. The
entire sphincter mechanism
posterior to the rectum has
been divided, as well as the
posterior rectal wall. The
tumor can be seen. The
rectal wall is being divided
above the pectinate line

Fig. 26.42 Operative field. Applying uniform traction

Fig. 26.41 Picture of the operative field. The pectinate
line is clearly seen, and the traction sutures are placed
2 cm above the pectinate line
478 26 Posterior Sagittal Approach for the Treatment of Other Conditions

Fig. 26.43 Diagram

showing the rectum
dissected and pulled down
enough to resect 5 cm
above the tumor

Fig. 26.44 Picture of the operative field, showing the Fig. 26.45 Picture of the operative field. The rectum has
rectum pulled over been opened to show the tumor
26.10 Presacral Masses
rectum mobilized, sutured with two layers of
interrupted long-term absorbable sutures to the
lower rectum above the pectinate line.
Figure 26.44 shows a full mobilization of the
rectum, immediately prior to the resection.
Figure 26.45 shows the operative field; the rec-
tum has been opened to show the location of the
tumor and to be sure that we leave a 5-cm mar-
gin before we suture the upper rectum to the
lower rectum. Figure 26.46 shows the operation
completed. The rectum has been mobilized and
sutured to the lower rectum.
Fig. 26.46 Diagram showing the operation completed.
Anastomosis done 2 cm above the pectinate line
a b
Fig. 26.47 MRI of a patient with giant cystic mature teratomas, without a sacral defect. (a) Transverse section.
(b) Sagittal view
479
26.10 Presacral Masses
Presacral masses in association with anorectal
malformation were discussed in the chapter of
perineal fistula.
There are, however, cases of presacral masses
with or without sacral defects, without anorectal
malformation.
Our series of presacral masses includes over
50 cases, mainly dermoid, teratomas, lipomas,
and anterior meningocele, but also includes one
schwannoma and two malignant tumors. That is
another reason why all the presacral masses must
be resected. Patients with presacral masses and
no anorectal malformations have mainly cystic
teratomas. Figure 26.47 shows the MRI of the
pelvis of an adult patient with giant cystic presa-
cral teratomas without a sacral defect. The pres-
ence of presacral masses without sacral defect
has a much better functional prognosis in terms
of bowel and urinary function.
There are many other patients that are born
with presacral masses without anorectal malfor-
mation. These types of cases are more difficult to
detect. A typical example is a patient that suffers
from, what the doctors believed, an infected pilo-
nidal sinus. The patient is operated several times,
only to find that the abscess and drainage keep
recurring. Under such circumstances, it is
recommended to order an MRI of the pelvis,
480 26 Posterior Sagittal Approach for the Treatment of Other Conditions
Fig. 26.48 Infected presacral dermoid, misdiagnosed as
a recurrent pilonidal cyst. The patient underwent seven
previous failed operations
Fig. 26.49 Chronic draining sinus in the sacral area, the
patient had an infected presacral dermoid
Fig. 26.50 External aspect immediately after the
operation
which may disclose the presence of a teratoma or
dermoid that becomes infected and drains in the
subcoccygeal area. Figure 26.48 shows a 6-year-
old girl that had seven previous operations under
the misdiagnosis of a pilonidal sinus; actually she
had a very large presacral mass that had become
chronically infected.
Another one of our patients, a 19-year-old
girl, suffered from constipation of unknown ori-
gin for her entire life. She developed an abscess
in the area of the sacrum, as well as manifesta-
tions of meningitis and sepsis. She became
extremely ill and was admitted to the hospital.
The abscess was drained, and the sepsis and men-
ingitis were controlled; the patient developed as
sequelae a chronic draining sinus in the area of
the coccyx. Figure 26.49 shows the external
appearance of this patient. Figure 26.50 shows
the same area, immediately after the resection of
a very large presacral infected dermoid. The
x-ray film showed a hemisacrum. Patients who
suffer from idiopathic constipation should have,
as part of their evaluation, an x-ray film of the
sacrum, as well as an MRI of the spine and cord,
to rule out the presence of presacral masses.
Table III shows our series of presacral masses by
histologic diagnosis, with and without associa-
tion with anorectal malformation and with and
without association of sacral abnormality.
26.11 Surgical Technique
Most presacral masses are resectable using only
the posterior sagittal approach without an abdomi-
nal intervention. We are not including in this dis-
cussion the cases of neonatal sacrococcygeal
teratomas, which represent, we believe, a different
entity, with special characteristics and important
technical considerations. The posterior sagittal
incision is done, as described multiple times in this
textbook. It is very important for the surgeon to
have a very precise anatomic diagnosis of the
mass, with emphasis in the location, in terms of
distance between the coccyx and the mass. Using a
posterior sagittal approach, we can usually reach
up to the level of sacral vertebra 2 or 3; above that,
it would represent a serious technical challenge.
The incision involves exactly the midline and
divides the skin, the subcutaneous tissue, and the
entire sphincter mechanism. The MRI study
should alert us as to what we are going to find after
we divide the entire sphincter mechanism
below the coccyx. If the mass is located above the
26.12 Posterior Sagittal Approach, Its Application in Cases with Hirschsprung’s Disease
Fig. 26.51 Intraoperative picture of the resection of a
retrorectal mass
coccyx, then we will identify, right away, the pos-
terior rectal wall, and dissecting on top of the rectal
wall, we will be able to find the mass. On the other
hand, sometimes the mass is attached to the poste-
rior rectal wall below the coccyx, and therefore,
we should look and expect the mass to be found as
soon as we divide the sphincter mechanism.
Figure 26.51 shows the operative field of one of
these operations, where the mass can be seen in the
deepest portion of the incision. We usually place
several silk stitches in the mass in order to apply
uniform traction to facilitate the dissection. We
must be aware of the fact that the mass and the
posterior rectal wall are intimately attached; basi-
cally we have to shave the posterior rectal wall to
separate the mass. In those congenital cases, in
which the mass has been compressing the rectum
in utero, the resection of the mass is sometimes not
enough to deal with the problem, due to a stricture
of the rectum. That stricture is fibrotic and should
not be expected that it would regain a normal
caliber after the mass has been resected. Our
481
experience shows that it is necessary to resect the
narrow portion of the rectum and pull down the
healthy-looking bowel. Sometimes there is a very
giant dilated upper rectum that must be tapered in
order to pull it down. Several patients came to us
after they underwent the resection of the mass;
they had been subjected to multiple anorectal dila-
tations, with the expectation that the narrow por-
tion of the rectum would improve which actually
does not happen. Therefore, from day one, we sug-
gest to resect that narrow portion of the rectum.
In those cases, in which the surgeon is able to
make the diagnosis of anterior meningocele
component of the mass, the patient should be
seen by a pediatric neurosurgeon with experience
in these kinds of problems. When the anterior
meningocele is very small, sometimes we can
simply respect it, knowing that it is there, and
leave it in place. However, some neurosurgeons
prefer to approach the patient from behind,
through a laminectomy to close the communica-
tion of the dura with the presacral mass. In the
second stage, the surgeon may resect the poste-
rior rectal component, of the meningocele, with-
out being concerned about injuring the dura.
Other neurosurgeons prefer to be present during
the posterior sagittal resection of the mass and to
close directly the dural defect. Usually, in addi-
tion to the closure with nonabsorbable sutures, a
patch of fat, muscle, or sometimes cartilage is
used to reinforce the defect. We have never seen
a case of chronic drainage of cerebrospinal fluid
consecutive to one of these operations.
26.12 Posterior Sagittal Approach,
Its Application in Cases
with Hirschsprung’s Disease
In the chapter corresponding to Hirschsprung’s
disease, we briefly mentioned cases of reopera-
tions that may represent a very serious surgical
technical challenge, due to the fact that the patient
suffered from dehiscence, infection, abscesses,
and fistulas that provoked a very significant
amount of fibrosis in the pelvis. These patients are
particularly difficult to treat using a traditional
transanal or abdominal approach. When con-
fronted with that type of case, the surgeon must
482 26 Posterior Sagittal Approach for the Treatment of Other Conditions
a b
Fig. 26.52 Diagram showing a sagittal view of the pelvis
of a girl who underwent a failed attempted repair for
Hirschsprung’s. There was a dehiscence of the anastomo-
consider the possibility of approaching the patient
posterior sagittally. These patients are frequently
referred to us with a patent anus, but an atretic rec-
tum as a consequence of dehiscence of the pull-
through. The area of fibrosis that produced the
acquired atresia or stricture of the rectum is located
in a place difficult to access in the pelvis. Trying to
resect the lower rectum, preserving the anal canal,
as well as pulling normal bowel down through a
“cement type” of pelvis may prove to be extremely
difficult. Figure 26.52a shows a diagram with a
sagittal view of a female patient who suffered from
this kind of problem. There was a dehiscence of
the anastomosis, producing a local abscess, severe
scarring, and fibrosis. The reoperation for these
complications should be done not before 6 months
after the event. Figure 26.52b is a diagram of the
completed repair. A distal colostogram is per-
formed in order to see the precise location of the
normal-looking upper bowel, as well as injection
of contrast material from below, in order to mea-
sure the gap between the proximal and distal
bowel. A rectal examination allows the surgeon to
realize that there is a lot of scar and therefore very
difficult to attempt a traditional transanal or
abdominoperineal approach. Under those circum-
sis, leaving the patient with an acquired rectal atresia and
severe pelvic fibrosis. (a) Preoperative diagram. (b)
Postoperative diagram
stances, the posterior sagittal approach represents
a viable alternative. Something similar happens in
patients who underwent a Soave type of pull-
through, and, for unexplained reasons, the sur-
geons left pieces of bowel mucosa attached to the
muscular cuff; those islets of mucosa produce
mucus, abscess, and fistula formation and are
extremely difficult to find in a reoperation, particu-
larly when trying to do it with a traditional
approach. Again, the posterior sagittal approach
gives much better exposure and more chances to
find those remnants of mucosa. We have found
trapped pieces of bowel left in the pelvis produc-
ing mucous and multiple fistulas, sometimes
draining into the perineum, sometimes into the
vagina or through the urinary tract.
26.13 Vaginal Atresia with Normal
Rectum
Patients suffering from vaginal atresia have a
dilated, blind vagina located at different levels
sometimes compressing the trigone of the blad-
der. Depending on the specific distance between
the blind lower part of the vagina and the
26.13 Vaginal Atresia with Normal Rectum
perineum, the surgeon must select the best pos-
sible approach to mobilize the vagina down. If
the vagina is considered not reachable through
the perineum, one alternative that we have used is
exploring the space between the urethra and rec-
tum, trying to reach as high as possible, provided
we feel safe and confident in not producing injury
of the rectum or urethra. Once we have reached
from below the point in which we feel it is not
safe to continue, then the vagina can be mobi-
lized through the abdomen with a laparotomy or
laparoscopy. In the event of finding the blind
vagina during the exploration between the ure-
thra and rectum, but the mobilization becomes
technically difficult, in a patient that has been
subjected to a strict bowel preparation prior to the
procedure, we can expand the incision trans-
anorectally, as demonstrated in cases of urogeni-
tal sinus with normal rectum. Provided the
reconstruction of the rectum is performed metic-
ulously, as we previously mentioned, the patients
recover very well, even without the opening of a
a b
Fig. 26.53 Vaginal atresia, hematocolpos, and hemato-
metra. (a) Introitus showing only the urethral opening. (b)
MRI study showing the giant vagina. (c) Surgical explora-
tion between the urethra and the rectum. Silk sutures
483
colostomy. Figure 26.53a–f shows images related
with one specific patient who came to our clinic
with a very dilated blind vagina (hematocolpos
and hematometra). With the bowel completely
clean in order to have access to trans-anorectal
approach, if necessary, we approached the patient
through the perineum, and we were fortunate
enough to find the vagina and were able to mobi-
lize the vagina down enough to reach the labia,
without having to divide the rectum. However,
we can easily divide the rectum, if necessary, if
the procedure becomes technically demanding.
Figure 26.53a shows the preoperative aspect of
the introitus of this patient that only shows the
urethral opening. There is no vaginal opening.
Figure 26.53b shows the MRI of this patient.
Figure 26.53c, d shows the appearance of the
field during the surgical exploration, looking for
the vagina. Figure 26.53e shows that the vagina
has been mobilized, and Fig. 26.53f shows that
the vaginal opening has been successfully
created.
applied on the vagina. (d) Vagina open. Observe old blood
coming out. (e) Vaginal fully mobilized. (f) Finished
operation
484 26 Posterior Sagittal Approach for the Treatment of Other Conditions
c d
e f
Fig. 26.53 (continued)
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55. Khan AU, Furlow WL (1976) Transpubic urethro-
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 Miscellaneous Conditions
27.1 Part I: Perianal Abscess
and Fistula
27.1.1 Definition
Perianal fistula is a fairly common condition in
male infants. It consists in the presence of a tract
that runs from one of the anorectal crypts toward
the perianal skin (Figs. 27.1 and 27.2). This fis-
tula occurs as a consequence of an abscess previ-
ously formed in the perianal area. We do not
know precisely the incidence of this condition. It
is extremely unusual to see this condition in
female patients, as well as in older children. In
pediatrics, this condition has very significant
differences when compared with perianal and
Fig. 27.1 Perianal abscess
A. Peña, A. Bischoff, Surgical Treatment of Colorectal Problems in Children,
DOI 10.1007/978-3-319-14989-9_27, © Springer International Publishing Switzerland 2015
27
perirectal fistulas in adults [1–3]. It is important
to recognize these differences because of their
therapeutic implications.
Perianal and perirectal abscesses and fistula
are fairly common in the adult population, but it
occurs in forms and variants more severe and
serious than in pediatrics [1–3]. The recom-
mended treatments in adults also vary, depending
on the type of fistula (transsphincteric, transleva-
tor, intrasphincteric). In pediatrics, we only see a
benign form in babies (mainly males), and that is
the type that we will discuss here.
Fig. 27.2 Perianal fistula. Arrow shows the point of the
future fistula
487
488
We are aware of the existence of other types of
fistulas that are extremely rare in babies and are
consecutive to inflammatory bowel disease or
complications from Soave operations in cases of
Hirschsprung’s disease. Perianal fistulas in
patients with inflammatory bowel disease must
be treated following the principles established for
the primary condition.
Perianal fistula secondary to endorectal, Soave
type of procedure for the treatment of
Hirschsprung’s disease occurs as a consequence
of poor surgical technique; pieces of mucosa
were usually left trapped in the pelvis, producing
mucoceles that become infected and form a fis-
tula (see chapter on Hirschsprung’s disease).
Some authors published their experience, mix-
ing cases with the infant type, with other types of
fistulas seen in immunocompromised children,
with leukemia, HIV, neutropenia, or cases receiv-
ing chemotherapy, which makes the interpretation
of the results rather impossible [4–7].
27.1.2 Etiology
The etiology of this condition is basically unknown.
However, there is a great deal of speculation related
with its origin [8]. Traditionally, it is considered
that the anorectal crypts of the pectinate line, that
separate the rectum from the anal canal, have small
channels that become contaminated with feces and
create abscesses located lateral to the anorectal
line. These abscesses eventually drain through the
skin next to the anus. Once the drainage occurs,
then it may remain a patent tunnel (fistula) between
the crypt and the skin. This usually occurs or opens
about 1 cm lateral to the anus (Fig. 27.2). However,
there is no real demonstration that this is true. The
fact that it is possible to pass a lacrimal probe or a
wire from the orifice in the skin to the crypt makes
this theory very plausible.
27.1.3 Natural History
Characteristically, baby males, during the first
months of life, suddenly develop a redness and
tender area next to the anus. Most of the time, this
abscess is surrounded by mild cellulitis and gives
27 Miscellaneous Conditions
minimal symptoms such as irritability, low fever,
and fussiness. Occasionally, the patient may have
high fever and cry inconsolably, particularly dur-
ing the bowel movements. In a period of a few
days, this abscess, if left alone, eventually drains
through the skin, which produces alleviation of
the symptoms. We have never seen a baby who
suffered from sepsis or expanding cellulitis. The
use of antibiotics does not seem to modify the
natural course of this condition. When these
patients are seen by pediatricians and/or pediatric
surgeons at this stage, with the abscess, many
times the abscesses are drained in the doctor’s
office or in the operating room. The drainage of
the abscess (spontaneous or surgical) makes all
the symptoms disappear, giving the impression
that the patient is cured and leaving only a small
dimple-like scar. In fact, some of these babies
never have this problem again. We do not know
exactly how many of the perianal abscesses
become fistulas. Different authors propose spe-
cific figures, varying from 24 to 35 % [9, 10].
However, that data is based on their personal
observations and not necessarily representative
of what happens in the world’s population.
A few days or a couple of weeks after the
abscess has been drained, suddenly the mother
notices redness in the area of the dimple where
the abscess drained in the past. The redness this
time is not as significant as during the episode of
the primary abscess formation and does not pro-
duce any symptoms of fever, irritability, or cel-
lulitis. Within a matter of hours, the little crust
that covers the dimple falls, and one or two drops
of pus come out of the little hole. The orifice
again looks completely healed, leaving only the
little dimple, and about 10 or 14 days later, it
reopens again, passing again a drop or two of pus.
This becomes a chronic condition. Eventually, if
left alone the overwhelming majority of fistulas
disappear; they disappear before 1 year of age
and a few more before 18 months of age [11].
Baby patients with perianal abscesses and fistu-
las are seen by pediatricians and pediatric surgeons,
either when they have the acute abscess or when
they already have a fistula. The general tendency
between doctors and surgeons is to drain the abscess
during the acute stage and to do some sort of
operation to try to cure the fistula. Our experience is
27.1 Part I: Perianal Abscess and Fistula
very different from most of the literature. We do not
operate on these patients because we are convinced
that all fistulas disappear before 18 months of age,
without an operation [11].
27.1.4 Treatment
A great majority of pediatric surgeons believe
that a fistulotomy or fistulectomy is the best treat-
ment for this condition [12–24]. The recurrence
rate has been reported to be between 4 and 68 %
[15, 16, 20, 22, 24–26].
Perhaps influenced by the experience of our
adult colorectal surgeons, some pediatric sur-
geons have treated this condition using a “seton”
[25–28]. We consider a fistulotomy an exces-
sively aggressive rather unnecessary treatment
for this benign condition. A “seton,” we think, is
even more aggressive to be used in infants.
Some surgeons are rather hesitant about fistu-
lotomy versus observation [39].
A growing group of surgeons are becoming
more conservative about the management of this
condition and are advocating, like us, a nonoper-
ative approach [29–34].
Other conservative treatments have been pro-
posed in adults [35] and more recently in pediat-
rics [36], consisting in the use of adhesive
material to occlude the fistula. This last treatment
seems too simplistic, and therefore we remain
skeptical about it.
During the acute stage of the abscess forma-
tion, most doctors give antibiotics. It is our
impression that the use of antibiotics does not
change the natural history of this condition, and
therefore they are unnecessary. We have never
seen, heard, or read about sepsis, expanding cel-
lulitis, or more serious problems consecutive to a
perianal abscess and fistula in babies. This is
completely different to the experience seen in
adults and must always be kept in mind.
In babies, we have never seen other, more
complicated type of fistulas referred to in the
adult colorectal literature [2, 3]. It is extremely
unusual for us, pediatric surgeons, to see perianal
abscess and fistula in patients older than 1 year,
and when we see something like that, we are obli-
gated to rule out unusual conditions such as
inflammatory bowel disease (Crohn’s disease).
489
During the acute stage of the abscess, we have
drained a few in the clinic, with local anesthesia.
We do it to alleviate the symptoms and to calm the
mother’s concern. Most of the time, if the symp-
toms are not dramatic and the mother is not anx-
ious, we simply wait; usually, the next day the
abscess drains spontaneously, and the mother finds
pus in the diaper. After this, the most important
part of our treatment is to convince the parents that
the drainage of one or two drops of pus every 10 or
14 days in an otherwise happy, normal baby is
meaningless, and eventually that will disappear.
The parents sometimes tend to be anxious and
look for another opinion and usually find a sur-
geon who performs a fistulotomy. In 29 years of
experience and over 25 patients we have treated,
we have never seen a child in whom the fistula did
not disappear spontaneously within 18 months of
life. We do not know if some of these babies who
suffer from perianal fistula during this period of
life are the same patients who will develop more
complicated fistulas when they become adults.
27.1.5 Fistulotomy
As previously mentioned, most pediatric sur-
geons advocate a fistulotomy for the treatment of
this chronic fistula in babies [12–24]. We per-
formed fistulotomies prior to the moment when
we learned from a senior pediatric surgeon
(James Warden) what the natural history of this
condition is.
Under general anesthesia, the baby is placed in
lithotomy position or in prone position, and an
anal speculum can be used. A wire or a lacrimal
duct probe is passed through the orifice in the skin
and finds a path toward one of the crypts of the
anorectal junction. This tract is dilated slightly,
and then all the tissue, including the mucosa,
bowel wall, and perianal skin, is divided to leave
the tract of the fistula wide open. The wound is
left open to granulate, which scares the parents
and bothers the baby. When the pectinate line is
exposed with the anorectal speculum, another
way to find the crypt that is connected with the
fistula tract and to avoid the creation of false tracts
with the lacrimal duct probe is by injecting air
through the orifice in the skin and seeing bubbles
coming out through the specific crypt that is
490
responsible for this abscess. We had experience
with this operation in the past, and our results
were similar to those mentioned in the literature.
Our first publication related with the conser-
vative approach for the management of this con-
dition [11] was published in 2000. Yet, many
publications related to the surgical treatment of
this condition indicate that the majority of sur-
geons continue using an aggressive approach for
the treatment of this self-limited condition. We
learned that we surgeons, in general, do not like
presentations and publications advocating non-
operative treatments.
27.2 Part II: Perianal Fistula
and Rectovestibular Fistula
with Normal Anus in Females
Perianal fistula in baby girls is extremely rare.
However, we decided to discuss this entity
together with a condition that received different
names, including:
• N type of fistula
• Double termination of the intestinal tract
• Perineal canal
• Congenital H-type vestibular fistula
• Rectovestibular fistula with normal anus
We were very surprised to find similarities
between the condition that received five different
names in the literature and the acquired perianal
fistula.
We previously published our experience with
the surgical treatment of the “H type of rectoves-
tibular fistula” [37]. Like most authors [38–55],
we thought that all cases suffering from this con-
dition were congenital.
Subsequently, we had the opportunity to read
some papers in which the authors believed that
this was an acquired condition [56, 57]! One of
these reports [57] presents an overwhelming
experience in favor of the idea that this is an
acquired condition. From a series of 182 cases,
85 % of the cases had a history of a local inflam-
mation prior to the diagnosis of the fistula. Other
surgeons, even when they think that this is a con-
genital malformation, reported some spontane-
ous closures of the fistula [58], or they mentioned
the occurrence of local abscesses prior to the
diagnosis [59]. Finally, some authors believe that
27 Miscellaneous Conditions
some cases could be congenital and others could
be acquired [60–62]. As a consequence of this
controversy, we started looking at our cases with
a different suspicion in mind. We recently had
two little female infants suffering from a fistula
between the vestibule of the genitalia and a crypt
of the pectinate line of the anal canal. We sus-
pected that these babies could be suffering from
an acquired perianal fistula, like the one that we
discussed in male infants. Consistent with our
conservative approach that we advocate for male
infants, we offered the same approach to the par-
ents, and to our surprise, the fistulas closed spon-
taneously after a few months of observation.
We now believe that both types (congenital and
acquired) may occur in females babies. This con-
dition is particularly common in Asiatic countries;
therefore, our experience is a modest one [37].
Some of the cases described in the literature are
obviously congenital. The reason why we believe
that is because the patients have associated malfor-
mations, frequently seen in ARM. In addition,
some of these patients have anal stenosis, presacral
mass, or anterior mislocation of the anus. The con-
genital fistula has a wide tract with no inflamma-
tory changes, and the histologic study of the inside
lying shows bowel mucosa. The acquired type is
more narrow, like the ones seen in males.
In summary, our current therapeutic approach
consists in selecting the patients. A conservative
approach (observation) is offered to infants
(younger than 1 year), with a fistula behaving in the
way described in males, without any of the malfor-
mations frequently associated to other ARM.
We try to simply observe the patient until the
age of 18 months; if the fistula remains open, we
repair it surgically.
On the other hand we will offer a surgical
repair to a case that is older than 1 year, has a
large fistula, has an evidence of being present
since birth, has a strictured or mislocated anus,
and has an abnormal sacrum or urogenital mal-
formations frequently associated with ARM.
27.2.1 Surgical Treatment
Most patients come to us with a colostomy done
at another institution. If the patient does not
have a colostomy, we repair the defect without
27.2 Part II: Perianal Fistula and Rectovestibular Fistula with Normal Anus in Females
it, but with a strict bowel preparation and fasting
for a period of 7–10 days, receiving parenteral
nutrition.
The patient is placed in prone position. A lac-
rimal probe is passed through the fistula
(Fig. 27.3a – a, b). The perineal body is divided
between the vestibular opening of the fistula and
the opening in the anal canal (Fig. 27.3b). The
mucosa of the fistula is resected, and multiple fine
a Aa Ab
b c
Fig. 27.3 Surgical repair of an H type of fistula in a
female patient. (a) Fistula located in the vestibule. (a)
Preoperative. Arrow showing the fistula. (b) Intraoperative,
with lacrimal probe coming out through the rectum.
Lacrimal probe passing through the fistula. (b) Divide all
tissue of the perineal body. Fistula tract exposed. (c)
491
silk stitches are placed taking the anterior rectal
wall in order to apply uniform traction (Fig. 27.3c).
The anterior rectal wall is dissected away from the
vaginal wall (Fig. 27.3d). The rectal wall is mobi-
lized enough to reach the perineum comfortably
(Fig. 27.3e). The perineal body is reconstructed
(Fig. 27.3f). The anterior rectal wall is sutured to
the perineal skin (Fig. 27.3g). Figure 27.3h shows
the aspect of the operated area.
Resected fistula tract. Multiple stitches placed on the ante-
rior rectal wall to apply uniform traction. (d) Anterior rec-
tal wall is mobilized dissected away from the vaginal wall.
(e) Anterior rectal wall fully mobilized. (f) The perineal
body is reconstructed. (g) The anterior rectal wall is
sutured to the perianal skin. (h) Finished procedure
492 27 Miscellaneous Conditions

d e

f g

Fig. 27.3 (continued)

27.3 Part III: Other Conditions
27.3 Part III: Other Conditions
27.3.1 Anal Fissure
Anal fissure is a benign condition that we see in
pediatric patients suffering from severe constipa-
tion. The accumulation of stool in the rectum
produces a very large solid mass of fecal matter
that eventually comes out, producing a laceration
in the anal verge. While the laceration starts heal-
ing, another big mass of stool passes again one or
several days after, reopening the wound, and this
cycle continues, creating a chronic ulcer in the
anus with severe pain, fear to defecate, and
attempts of the patient to hold the stool, which
will exacerbate the problem of constipation. (See
chapter on idiopathic constipation.)
The literature on anal fissure in adults indicates
that most likely it is a completely different condition
than the one that we see in pediatrics. In adults, they
consider that it is related with the non-relaxing
internal sphincter, and they advocate as a treatment
dividing the internal sphincter or the injection of
Botox in order to facilitate the healing of the ulcer.
We cannot understand the relationship of the
contraction of the internal sphincter with the pres-
ence of a fissure. In the pediatric population, all the
patients that we have seen heal as soon as the vicious
cycle already described is discontinued. In other
words, the treatment for anal fissures in children is
the same as the treatment for constipation. In order
for the wound (fissure) to heal, it is necessary to
avoid the passing of large pieces of solid fecal mat-
ter, and it may take weeks for the fissure to heal.
Also, in order for the baby to forget about the pain-
ful experience of the bowel movements, it will take
several weeks or months. Therefore, the essential
component of the treatment is the parent’s as well as
pediatricians’ about what we are trying to achieve.
The fissure is a consequence of constipation, and
the treatment is the treatment of constipation.
In all the patients that we treated in this way,
the fissure healed, and the patients significantly
improve.
27.3.2 Hemorrhoids
In 30 years, we have never done a hemorrhoidec-
tomy in a pediatric patient. The type of hemor-
493
rhoids that we have seen is minimal and frequently
related again with constipation. We never saw a
thrombosis or a bleeding hemorrhoid in pediatrics.
We read in the very old literature the uncommon
event of an obstruction of the portal system that may
provoke as a symptom, the presence of hemor-
rhoids. We have never seen anything like that.
27.3.3 Idiopathic Rectal Prolapse
Idiopathic rectal prolapse is a serious problem
and is very difficult to treat. The main problem
consists in the fact that we do not know the origin
of that condition. We try not to operate on patients
with idiopathic prolapse because we believe that
the prolapse is a symptom and not a disease. It is
a symptom of a more serious condition, whose
nature is unknown to us. We see these patients
referred to us by pediatricians and gastroenterol-
ogists, and we try to postpone the operation and
rule out predisposing conditions such as consti-
pation, cystic fibrosis, inflammatory bowel dis-
ease, parasites, and food allergy. We know that an
inflammatory condition of the colon as well as
constipation may exacerbate the idiopathic pro-
lapse. Frequent trips to the toilet and spending
long periods of time sitting in the toilet also exac-
erbate an existing prolapse. We try to rule out all
those predisposing conditions, and if the prolapse
continues, we discuss with the family the possi-
bility of an operation. We explained that it is a
symptom and not a disease, and therefore we
cannot guarantee that it will not recur. We cannot
cure a disease that is unknown to us. We went
through different procedures through these last
30 years. We had original experience with “cer-
clage,” followed by perirectal injection of irritant
substances and a posterior sagittal approach to
suture the posterior rectal wall to the cartilage of
the coccyx and sacrum. All of those procedures
have some degree of success, but we were not
universally successful. Lately, we have been
doing a transanal resection of the prolapse, being
sure to respect and protect the anal canal
(Fig. 27.4a–g). In other words, we resect part of
the rectum, at least 2 cm above the anal canal,
and resect as much rectum as we estimate that
was prolapsing. We do not have any recurrences
with that kind of treatment. However, some of the
494 27 Miscellaneous Conditions

a b

c d

Fig. 27.4 Transanal resection of idiopathic rectal pro-
lapse. (a) Exposed anal canal. (b) Retracting hooks placed
deeper than the pectinate line (only rectal mucosa is
exposed). (c) Initial resection margin, marked 2 cm.
Above pectinate line. (d) Multiple silk sutures pulling up
the upper rectum. (e) Full-thickness rectal circumferential
dissection. (f) Estimated prolapsing portion of the rectum
mobilized. Two-layer anastomosis started. (g) Rectum
resected. Finished anastomosis
27.3 Part III: Other Conditions 495

e f

Fig. 27.4 (continued)

496
patients that we operated that way frequently
come back complaining of different gastroenter-
ologic symptoms such as abdominal pain, some-
times blood in the stool, episodes of diarrhea, and
constipation. They tend to behave almost like
what is described in adults as an irritable colon. It
remains to be seen what is the future of those
patients that had a successful operation for an
idiopathic prolapse and what kind of gastroen-
terologic symptoms they would have during their
adult lives.
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