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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


© Springer International Publishing Switzerland 2015
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(www.springer.com)
To our parents
To our children
To all children born with congenital anomalies
Foreword I

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

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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

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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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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
xx Contents

11.3 Diagnosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 165


11.4 Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 166
11.4.1 Colostomy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 166
11.4.2 Main Repair . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 166
11.4.3 Laparotomy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 168
11.4.4 Laparoscopy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 178
11.5 Special Problems . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 179
11.5.1 Dealing with Inadequate Colostomies
(Too Distal) . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 179
11.6 Functional Results . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 179
11.6.1 Fecal Control . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 179
11.6.2 Urinary Control . . . . . . . . . . . . . . . . . . . . . . . . . . . 180
References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 181
12 Imperforate Anus Without Fistula in Males and Females . . . . 183
12.1 Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 183
12.2 Anatomic Characteristics . . . . . . . . . . . . . . . . . . . . . . . . . . . 184
12.3 Main Repair . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 185
12.4 Function and Results . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 187
References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 187
13 Minimally Invasive Approach to Anorectal Malformations . . . 189
13.1 Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 189
13.2 Males . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 192
13.3 Females . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 194
References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 196
14 Rectal Atresia . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 201
14.1 Treatment . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 202
14.2 Surgical Repair . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 202
References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 204
15 Rectovestibular Fistula . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 205
15.1 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
Contents xxi

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
Contents xxiii

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

1.1 Introduction existence of illustrations in history books, going


back hundreds of years in different cultures and
So the conservative who resists change is as valu- civilizations, related to the treatment of surgical
able as the radical who proposes it. It is good that conditions of the anus.
new ideas should be heard, for the sake of the few One’s goal in the study of history should not
that can be used; but it is also good that new ideas be to try to memorize names and dates, but rather
should be compelled to go through the mill of
objection, opposition, and contumely; this is the to take advantage of the unique opportunity to
trial heat which innovations must survive before look back and have a wide perspective of the evo-
being allowed to enter the human race. It is good lution of our knowledge. Contemplation of the
that the old should resist the young, and that the historical facts, hopefully without prejudices,
young should prod the old; out of this tension, as
out of strife of the sexes and the classes, comes a allows us to recognize patterns of human behav-
creative tensile strength, a stimulated develop- ior. Some of those patterns are creative and posi-
ment, a secret and basic unity and movement of the tive and should be imitated, and some others are
whole. By Will and Ariel Durant [1] to be abandoned. It allows us to see repetitive
behaviors that disclose our limitations as human
The history of the surgical treatment of ano- beings as well as the creativity when dealing with
rectal malformations is a representative sample unknown facts. One can learn, for instance, that
of the history and evolution of medicine. some of the “new discoveries” are not really new.
Centuries ago, medicine was related to religion Other times, an old concept is brought back, but
and mysticism; the treatment of the different dis- with a different vision, and even when it is not
eases and surgical conditions was performed by essentially new, represents an advantage when
witches, barbers, or those who showed some compared to previous procedures. The dilemma
“wisdom” in the community. It took many centu- of those who study history is always the commit-
ries for medicine and surgery to become scien- ment to “the truth.” We are limited by the litera-
tific disciplines. Even in current days, the practice ture that is available that may or may not be
of medicine and surgery has a great element absolutely truthful.
of art. Finally, we, the authors of this book, must
Because of its nature, an anorectal malforma- confess that we are biased when describing the
tion is a particular defect that has been well history of the surgical treatment of anorectal
known for many centuries. The explanation is malformations. We are biased and impressed by
very obvious; one does not have to be a doctor to the fact that the real, intrinsic anatomy of the
make the diagnosis of an absent anal opening. anorectal malformations was really not known
That is perhaps one of the explanations for the until 1980. Looking into the many historical

A. Peña, A. Bischoff, Surgical Treatment of Colorectal Problems in Children, 1


DOI 10.1007/978-3-319-14989-9_1, © Springer International Publishing Switzerland 2015
2 1 History of the Treatment of Anorectal Malformations

publications that we reviewed, one can find dia- Soranus de Ephesus was considered the father
grams that only show the imagination of the of obstetrics in ancient Rome. He wrote the book
authors and the medical illustrators, but not the On the Care Of the Newborn. In that book, one
real anatomy. Those diagrams were followed by can read that he instructed the women in charge
interpretations and erroneous conclusions about of delivering babies how to trim off their finger-
what should and should not be done in the treat- nail of the little finger, to dilate the anus of those
ment of these malformations. There are very few babies who did not pass meconium after birth [5].
photographs showing the real anatomy, for Paul of Aegina (625–690) made the first descrip-
instance, of the connection between the gastroin- tion of an operation for imperforate anus: “If pos-
testinal tract and the urogenital tract. Some of sible, the membrane that covers the anus must be
the few real pictures of the intrinsic anatomy of divided with the finger. If this is not successful,
these defects prior to 1980 belong to the publica- then an incision must be done.” To avoid or to pre-
tions of Dr. Douglas Stephens [2]. Yet, they are vent the scarring or stricture of the new anus, he
not representative of the whole spectrum of ano- recommended a form of bougienage consisting of
rectal malformations. the local application of wine and balsam [6].
The retrospective analysis of the history of Perhaps the first illustration describing an
anorectal malformations shows a very common anorectal procedure in pediatrics was found in a
human tendency to classify biological phenom- book entitled Cerrahiyei Ilhaniye, written in
ena into types, groups, and categories. It is under- 1465 by Dr. Sharaphedin in Turkey [7].
standable that this is usually done for the specific In 1606, Guilhelmus Fabricius Hildanus
purpose of communicating among ourselves and described a case of a recto-bladder fistula. For
comparing our results. Yet, Mother Nature con- that case, many doctors were consulted; they all
tinues producing biological phenomena follow- saw meconium coming out of the urethra, and
ing a pattern of a spectrum without paying much nobody wanted to do anything. The baby died on
attention to our classifications. Anorectal malfor- the 17th day of life [8].
mations are not an exception. In other words, Littre, in 1710, proposed (but did not perform)
anorectal malformations do not occur in artifi- the opening of a colostomy in cases of anorectal
cially created groups, traditionally described as malformation [9].
“high,” “intermediate,” and “low.” They occur as Frederik Ruysch (1683–1731) was immortal-
most biological phenomena, following a spec- ized in a famous painting showing the autopsy of
trum type of pattern. Over time and with careful a baby. He described the spontaneous rupture of
analyses of presentation and results, it has an anal membrane after 5 days of life. The baby
become more and more clear that there are no died soon thereafter [10].
“nevers” and no “always” when describing the The practice of a perineal incision followed by
variety of anorectal malformations. dilatations, in babies born with “imperforate
anus,” was a method of choice until the later part
of the nineteenth century. During that time, there
1.2 The Early Times were many anecdotal descriptions of babies with
anorectal malformations that were treated that
The first reference of an anorectal malformation way, but the overwhelming majority of them died
was found in Babylon, about 650 years B.C. It [11]. Some surgeons disagreed with the way of
was written in stone, “When a woman gives birth treating those patients, such as Dr. Bigelow,
to a baby with a closed anus the entire Earth will Professor of Surgery at the Massachusetts
suffer from disease” [3]. General Hospital in Boston 1857 [12]. He men-
Geracao and Aristotle wrote a book on the tioned, “Based on the analysis of the results of
Generation of Animals; there, they described a those procedures, I believe that considering the
cow that was born without an anus and defecated state of the art in surgery for those anorectal
through the urethra [4]. defects, it is better to let those babies die.”
1.2 The Early Times 3

In 1753, M. Louis from Paris described the was probably the first one to perform an inguinal
case of a little girl who had an orifice that was colostomy in the sigmoid colon in a baby boy
considered a cloacal malformation [13]. She with imperforate anus; a week later, the patient
was menstruating through the anus! That patient was still alive [17].
got married and told her secret to her husband. In 1832, almost 100 years later, Martin
He convinced her to have sex with him, and she decided to follow the suggestion of Bertin and to
became pregnant. The lady had a “normal” perform a cystostomy in a patient who was pass-
delivery and was described as producing a ing stool through the urethra. Unfortunately, the
“minor laceration” of the anal sphincter. The patient died [18].
presentation of that case was considered in the Roux de Brignoles, in 1834, suggested that the
Parisian courts, and it was decided by theolo- fibers of the sphincter mechanism should be
gists and modernists that Dr. M. Louis somehow meticulously preserved during the perineal dis-
had acted in an illegal manner. The father of the section [19].
baby was called, M. Louis was finally declared Amussat, a prominent young surgeon, also in
innocent, and the court allowed M. Louis to 1835, in Paris, described the case of a 2-day-old
publish the case. girl who was not passing meconium. He operated
In 1771, Bertin [14] described a case of a baby on the patient on the dining room table of the
that was passing feces through the urethra. He patient’s house, assisted by his collaborators. He
was convinced that the baby would die unless he found the blind rectum, and he is considered the
had an operation. He approached the patient first surgeon who decided to suture the wall of the
through the perineum and could not find the rec- rectum to the skin edges, which could be consid-
tum. The baby died and Bertin concluded that the ered the first anoplasty. After 28 days, the baby
operation of choice for that particular case should was doing very well, without complications [20].
have been a cystostomy. It was also Amussat who classified the anorectal
In 1787, Benjamin Bell (1749–1806) from malformations into five types: type 1, anal steno-
Edinburgh [15] described two successful opera- sis; type 2, anal membrane; type three, a blind
tions in which the rectum was found to be located rectum at a variable distance from the anal skin;
“high” in the pelvis. The procedure that he type 4, a blind but also very “deficient” rectum;
described consisted in the introduction of a sharp and type 5, the rectum communicated with other
instrument in a blind fashion at the location organs, such as the bladder, urethra, or vagina.
where the anus was supposed to be located. This He recommended dilatation for type 1, incision
procedure was followed frequently by complica- and excision of the membrane followed by dilata-
tions that included bladder perforation and open- tions in type 2, and suture of the rectum to the
ing of the cul-de-sac of Douglas, and in some skin in type 3. In types 4 and 5, he recommended
cases, the rectum was never found. In his book mobilization of the posterior part of the rectum
entitled A System of Surgery, Bell described dif- and pulling it down to the perineum. In cases in
ferent types of anorectal malformations including which it was difficult to find the rectum through
“anal agenesis,” “anorectal agenesis,” “vesical the perineal incision, he recommended making
fistula,” and “vaginal fistula.” Benjamin Bell was the incision larger and to totally or partially
probably the first one to emphasize the need and remove the coccyx.
importance of decreasing the pain during these In 1844, Stromeyer [21] suggested that in
procedures that were generally done using cases in which the rectum could not be found
homeopathic techniques. through the perineal dissection, the peritoneal
It was Antoine Dubois, in 1783, who appar- cavity should be opened through the perineum,
ently performed the first inguinal colostomy on and the surgeon should look for the blind rec-
the left side in a 1-day-old baby with imperforate tum with a finger. That idea was practiced in
anus. The patient died 10 days later [16]. In 1793, 1872 by Leiserink, and he described a “good
Duret, following the suggestion of Littre in 1710, result” [22].
4 1 History of the Treatment of Anorectal Malformations

In 1860, Bodenhamer [23] proposed a classifica- pelvic structures. He suggested entering the pel-
tion dividing these malformations into four types: vis through the third sacral foramen. He sup-
Type 1: Incomplete rupture of the “inner mem- ported the idea of opening a colostomy. He also
brane” or anal stenosis believed that the rectal ampulla could move down
Type 2: Imperforate anus due to a persistence of spontaneously; therefore, he proposed to open a
the “anal membrane” colostomy and wait. In 1897, Matas wrote 22
Type 3: Imperforate anus with blind rectum sepa- conclusions related to the management of ano-
rated from the “anal membrane” rectal malformations. Some of which are still
Type 4: The presence of a blind rectum separated valid:
from the anal canal 1. “The most common types of anorectal mal-
In 1866, Chassaignac [24] decided to follow formations can be repaired through a peri-
the idea suggested by Martin de Lyon of opening neal approach.” Interestingly, this conclusion
a colostomy in order to introduce some sort of is quite accurate.
guide through the intestinal lumen of the colos- 2. “There are no external signs to determine the
tomy, to facilitate finding of the blind rectal end. internal anatomic malformations.” This con-
The perineum was then opened where the surgeon clusion is partially valid since now we know
could feel the bulging of the guide. Chassaignac that we can learn a lot just by careful inspec-
operated on a 7-month-old baby who had a previ- tion of the perineum.
ous colostomy and was able to create an opening 3. “One should not depend on the introduction of
in a “satisfactory” manner using that technique. guides through the vagina or the urinary tract to
Delens, in 1874 [25], described a case in determine the presence or absence of intestine.
which he achieved good exposure in the perineum The use of a needle to aspirate meconium is
area by removing or mobilizing back the coccyx also dangerous because of the risk of peritoneal
without resecting it. The next year, Polaillon contamination.” This is still true.
described splitting of the coccyx in the midline, 4. “The operation should be done as early as
obtaining better exposure to be able to dissect the possible to avoid death consecutive to the
rectum in a deeper area [26]. passing of stool to the blood, peritonitis,
In 1880, Neil McLeod was the first to suggest a intestinal obstruction, absorption of toxins,
combined abdominoperineal approach. He chose and migration of bacteria from the intes-
to start the operation through the perineum, and if tines.” Although now we are aware of many
the rectum was not found, to open the abdomen new, sophisticated pathophysiologic mecha-
through a midline incision. With a finger, as a nisms, this concept is still valid.
guide from inside the abdomen, the perineal inci- 5. “The tolerance of the baby to the trauma is
sion should be created to reach the peritoneal cav- inversely proportional to the age in days after
ity and the rectum pulled through [27]. birth. And in addition, the baby without sep-
In 1887 Vincent of Lyons performed a parasa- sis is as tolerant to trauma as the adult.”
cral incision instead of a mid-sacral one. This Again, he was right.
was described by Maitre [28]. 6. “The ideal result in this kind of operation is
In 1894, Paul Delageniere suggested perform- the restoration of the passage of stool, creat-
ing a lateral laparotomy to find the rectum and to ing an anus in a normal position with bowel
reach the perineum through the abdominal cavity, control.” This, of course, is still valid.
using his finger as a guide and then pulling 7. “The only way to obtain this kind of result is
through the rectum [29]. performing a proctoplasty as proposed by
In 1897, Rudolph Matas [30], a brilliant sur- Amussat.” Obviously, this is mostly wrong.
geon in New Orleans, mentioned that cutting, 8. “In order to obtain the best possible results
dividing, or destroying the sacrum had a negative from the functional point of view, the opera-
effect because it damaged the muscle insertions tor must avoid the unnecessary injury of the
as well as the innervation and blood supply of the sphincter mechanism, for that, the incision
1.2 The Early Times 5

must be performed strictly in the midline.” 21. “If, for some reason, the surgeon decided to
He was right! open a colostomy first, he should always
9. “The old method of stab of the perineum make every effort in a second procedure to
without a proctoplasty was not justified.” He open the anus in the perineum.” This is
was right. mostly true.
10. “The initial peritoneal exploration of the pel- 22. “The perineal-sacral anus, when it is cor-
vis through a perineal-sacral aperture was rectly done, is almost certain to have bowel
one of the greatest advances in the treatment control as time goes by.” That is, of course,
of these conditions.” Of course, that is no mostly not true.
longer true. In 1899 and published in 1908, Mastin dem-
11. “The peritoneal exploration through the onstrated that a permanent colostomy was com-
perineum must be attempted systematically patible with growth and development [31]. He
when the rectum is not found through the operated on a newborn baby and created a
perineum.” This is no longer valid. colostomy, and when he offered the family the
12. “Those techniques that use a sacral resection opening of an anus, the family refused to have
or excision or osteoplasty to increase the that operation done because the patient was
exposure and to reach the peritoneum look- doing very well and has adapted to the presence
ing for the rectum are valid.” Obviously, we of the stoma, playing sports and growing and
do not use that anymore. developing normally. In 1903, Mastin was
13. “The best approach is a midline incision called to take care of another case. He per-
through the coccyx and sacrum.” This is formed a perineal midline incision. He was able
mostly true. to find the bowel and perform an anoplasty that
14. “A predisposition to suffer prolapse must be he sutured to the skin with catgut. He described
expected in cases of resection of the sacrum.” that 4 years later, the patient had bowel
Obviously, we do not touch the sacrum control.
anymore. In 1915, Brenner [32] published an excel-
15. “A primary exploratory laparotomy is not lent paper in Surgical Gynecology and
indicated as a rule.” This is true. Obstetrics and described his experience with
16. “The great majority of imperforate anus can 61 cases. He described different degrees of
be treated successfully through the development of the external sphincter. He sug-
perineum.” That is true. gested that the operations to repair imperforate
17. “The perineal anus can be created pulling the anus should “last no more than 5–8 min!!” He
colon and connecting it to the perineum. But, performed a posterior incision, and he sug-
in cases of emergency, one can connect the gested opening a colostomy if the perineal
small bowel to the perineum.” Obviously, we approach was unsuccessful. He described that
do not do that. in males, the rectum opens more often into the
18. “The mortality from a colostomy is greater bladder rather than the urethra, which is not
than the anoplasty and perineal-sacral true, since now we have well-documented evi-
approach.” This is obviously wrong by mod- dence that the connection between the rectum
ern standards. and the bladder only occurs in 10 % of the
19. “Primary colostomy in the groin, as a pri- male cases. He suggested that if the patient did
mary procedure, is only indicated when the not have external sphincter fibers, bowel con-
baby is extremely sick. Under all of the other trol must be obtained by an axial rotation of the
circumstances, the perineal incision must be gut or using some muscle fibers from the
the first one.” This statement is partially true. gluteal region. Even though Brenner’s
20. “An exploratory laparotomy is only per- conclusions are not valid at the present time,
formed after the rectum was not found his work is very significant, because of the
through the perineum.” This statement is number of cases and the meticulous descrip-
also partially true. tion of them.
6 1 History of the Treatment of Anorectal Malformations

These ideas, like in many other historic events, embryologic description. They also included a
illustrate how naive we tend to be. Therefore, we detailed table of associated malformations. This
like to say that every time we try to cheat on is extremely important since, as the reader will be
Mother Nature, she teaches us a lesson. able to see in this textbook, the frequency of the
In 1930, Owen Wangensteen and Carl Rice associated defects in cases of anorectal malfor-
published a paper describing a method of radio- mations is very significant and those associated
logically determining the height of the blind rectal defects have a vital role in the prognosis of these
end to select the best surgical approach for patients patients. Ladd and Gross’s publication is a beau-
with anorectal malformations [33]. The technique tiful one; it has very elegant drawings done per-
that they described is well known as an “inverto- sonally by Dr. Robert Gross, illustrating the
gram.” It consisted of putting the newborn baby development of female malformations. The mor-
upside down for several minutes and taking an tality in their series was 26 %.
x-ray film of the pelvis to determine the location of In 1936, Stone [35] published a paper entitled
the blind end of the rectum, as well as the distance “Imperforate Anus with a Rectovaginal Cloaca.”
from the blind end of the rectum to the anal skin. In 1938, J. K. Berman [36] published a paper
The blind end of the rectum can be seen because it on 23 cases of anorectal malformations with
is full of gas. That method still has some value. 47 % mortality. He opposed the use of colosto-
However, we use a variation of it in less than 5 % mies in his patients because of its high mortality
of all cases, in those in whom there is no clinical and proposed an incision running from the peri-
evidence of the location of the distal rectum. Yet, neal body to the coccyx in newborns, with local
we have learned through the years that the same anesthesia. He used 0 size chromic catgut. He
image that Wangensteen and Rice were able to described only the pull-through of the bowel,
obtain with the invertogram can be achieved by leaving the fistula to the urinary tract untouched
placing the patient in prone position with the pel- until the patient was older!!
vis elevated and taking a cross-table, lateral film. In 1948, Rhoads et al. [37] (Fig. 1.1) pub-
We have learned many lessons from the exter- lished their experience with the first survivor of a
nal examination of the perineum of the babies, as primary abdominoperineal pull-through, without
well as other more sophisticated imaging a colostomy. After that publication, many sur-
methodology. geons tried to perform that kind of operation,
In 1934, William Ladd and Robert E. Gross sometimes with success, but many other times
[34] published a very comprehensive series of with serious catastrophic results, and therefore,
cases. Their publication also included good years after that, this approach was reconsidered.

Fig. 1.1 Photograph –


Dr. Peña with Dr. Jonathan
Rhoad
1.2 The Early Times 7

Lately, many others have been trying to approach In 1954, Dr. William Potts [40] published a
newborn babies primarily without a colostomy. paper related to the treatment of 22 “rectovaginal
As will be seen in this textbook, that approach is fistulas,” 8 rectourethral fistulas, 9 recto-perineal
sometimes justified, but not always. fistulas, and 12 rectovesical fistulas. The ano-
In 1953, Douglas Stephens published his first plasty that he proposed for the most common
landmark paper on the subject, in Australia [38]. type of malformation seen in females that we
Dr. Stephens has the unique distinction of being now know by the name of rectovestibular fistula
the first person who studied the anatomy of the is still known as the “Potts’ anoplasty” and con-
pelvis in patients who died from an anorectal mal- sists of dissecting the rectum from the vestibule
formation. From his studies, he concluded that the and passing it behind a bridge of skin, to be
key part of the sphincter mechanism to achieve placed within the limits of the sphincter. In retro-
bowel control in these cases was the “puborectalis spect, now we believe that what he described as
sling.” It took time for his concept to be learned “rectovaginal fistulas” were actually cases of rec-
and accepted by the world community of pediatric tovestibular fistulas, since now we recognize that
surgeons, but within a few years, most pediatric real rectovaginal fistulas are extremely unusual
surgeons recognized that was something to be malformations. Unless we postulate the theory
considered seriously, and therefore, the “era of the that the type of pathology changes through the
puborectalis” began. From that time, most sur- years, it is difficult to believe that surgeons had
geons tried to design operations aimed to preserv- many cases of rectovaginal fistulas, which we
ing the “puborectalis sling,” which was considered find now to be a malformation that is almost
key for bowel control. Unfortunately, it is not easy nonexistent.
to obtain cadavers of children born with anorectal In 1960, Scott, Swenson, and Fisher published
malformations because most children with ano- one of the first papers on long-term follow-up
rectal malformations survive, and therefore the results. Their patients suffered from a mortality
number of specimens studied by Dr. Stephens was of 12.7 %; 4.8 % was operative deaths. In their
very limited. In retrospect, we believe that his study, they reported 68 % incontinence in patients
conclusions are not valid because his studies were with the so-called “high” malformations and
performed in a limited number of the most severe 89 % good results in what were described as
cases, not representative of what we call the spec- “low” malformations [41].
trum of anorectal malformations. The cases In 1963, Kiesewetter et al. [42] reported
(cadavers) that he studied we think are not repre- their experience with 146 patients followed
sentative of the most common types of malforma- over a period of 16 years. These surgeons had a
tions that we see. Yet, one of his recommendations demonstrated special interest in the manage-
is still valid: he recommended pulling the bowel ment of anorectal malformations. Their mortal-
down, as close as possible to the urethra. In addi- ity was 19.2 %; 86 cases were followed on a
tion, Dr. Douglas Stephens published a book [2] long-term basis. The authors divided their
that represents the document with the largest series into “high malformations,” where the
amount of information related to the subject of rectum was located two centimeters above the
anorectal malformations at that time. anal skin, and “low malformations,” for those
In 1955, Sir Denis Browne, a prominent sur- where the rectum was closer to the skin. They
geon from Great Ormond Street Hospital in obtained 72 % “good results” in “low malfor-
London, proposed that patients with rectovaginal mations” and 45 % “bad results” in “high mal-
fistulas had a normal sphincter located at the formations.” They mention that it was important
vagina site [39]. We now know that that never to preserve, as much as possible, the distal part
happens. He also suggested that female patients of the bowel, which is a concept that we support
with vaginal, as well as males with rectoprostatic, at the present time. They reported 24.4 % of the
fistulas have no sphincter mechanism, which, as cases having vestibular fistulas, which is simi-
we know now, is mostly inaccurate. lar to what we report.
8 1 History of the Treatment of Anorectal Malformations

In 1966, Dr. Kiesewetter [43] supported the questionnaires to the members of the surgical
idea proposed by Stephens that the puborectalis section of the academy. From the answers, they
muscle was the only available muscle useful to were able to put together 1,116 patients from 51
achieve bowel control. He adopted Stephens’ institutions, 58 % were males and 42 % females.
idea of a sacral incision to preserve the puborec- The incidence of fistula in males was 72 % and in
talis sling. Through that incision, a blind tunnel females was 19 %, and 28.7 % of these fistulas
was created, behind the urethra (in males). A were “rectovaginal.” This is something that we
Penrose drain was then passed through the tun- know was most likely a misnomer or lack of
nel. The rectum was then to be pulled down accurate examination of the genitalia because, as
through that tunnel. He also adopted the principle we mentioned, congenital rectovaginal fistulas in
of a transabdominal endorectal dissection in our experience are almost nonexistent; 81 % of
order to try to avoid damage to the innervation of the patients with a “high malformation” received
the pelvic organs. This is the same principle that a colostomy, and 62 % of those colostomies were
Soave and Boley proposed for the treatment of done in the transverse colon. The mortality
Hirschsprung’s disease. Kiesewetter’s operation reported was 19 %.
was called a sacro-abdominoperineal endorectal During the years of 1969–1970, the senior
pull-through. author had the privilege of meeting Dr. Justin
In 1967, Dr. Rehbein [44] also proposed an Kelly. Dr. Kelly, a fully trained pediatric surgeon
abdominal sacroperineal procedure. He presented from Melbourne, Australia, had been greatly
70 cases, 55 males and 15 females. He empha- influenced by Dr. Douglas Stephens. Dr. Kelly
sized the importance of preserving the “puborec- went to Boston Children’s Hospital as a clinical
talis muscle.” fellow for two extra years. There, he lectured and
We want to express our recognition to many communicated the ideas of Dr. Stephens to all
prominent surgeons from all over the world who members of the surgical staff. Dr. Peña (senior
demonstrated special interest and dedication to author) was at Boston Children’s Hospital as a
the field of anorectal malformations. We are research fellow in 1969 and as a resident in 1970
unable to mention all of them; therefore, we and 1971. During that time, he learned from Dr.
selected the most prominent ones including: Kelly’s, Dr. Stephen’s, and Dr. Smith’s ideas
• Santull [45], Hanley [46], Lynn [47], Partridge about the management of anorectal malforma-
[48], Trusler [49], Cozzi [50], Soave [51], tions. Upon his return to Mexico City, Dr. Peña
Louw [52], Knutrud [53], Nixon and Puri became the Chief of Surgery at the New National
[54], Smith [55], Holschneider [56], Varma Institute of Pediatrics.
[57], Chatterjee [58], Rintala [59], Endo [60], From 1972 to 1980, the senior author of this
Scharli [61], Ito [62], Brayton [63], Aluwihare book operated, in Mexico City, on 56 cases of
[64], and Banu [65] the so-called, at that time, “high” imperforate
In 1970, in Melbourne, an international com- anus. He followed the principles proposed by
mittee was created to design an “international Stephens and learned from Dr. Kelly (sacral
classification of anorectal malformations.” That approach) and laparotomy when necessary as
“international classification” was adopted by all proposed by Kiesewetter, and Rehbein (sacro-
of the pediatric surgeons in the world, but abdominoperineal pull-through). During those
because of its complexity, it was not used in the 8 years, he became aware of the fact that, in the
everyday practice by most pediatric surgeons earlier cases, he was opening the abdomen very
(Fig. 1.2) [66]. often (80 % of the time) to repair these malfor-
In 1971 [67], the American Academy of mations, but after 8 years of experience, he was
Pediatrics proposed a joint effort between differ- opening the abdomen only 20 % of the time. He
ent institutions to learn about the different thera- attributed this to the fact that the sacral incision
peutic modalities as well as the results of the that Stephens originally proposed (very small)
treatment of anorectal malformations and sent 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, urinary tract in male patients and to avoid the
he decided to use an electrical stimulator with the need of a laparotomy. He also proposed the use of
specific purpose to identify the “puborectalis an electrical stimulator to identify the sphincter
sling.” Eventually, he felt the need to divide part mechanism and also to divide in the midline the
of the sphincter mechanism that was visible dur- muscle that he found, in order to facilitate, again,
ing this operation. He thought that this muscle the dissection of the fistula and proposed to resu-
mechanism must have been the “puborectalis ture the muscle behind the rectum at the end of
sling.” He specifically proposed to divide part of the procedure. This was basically a step prior to
what he thought was the “puborectalis muscle” to the full posterior sagittal anorectoplasty proposed
have better exposure and to facilitate the separa- later. The paper provoked a significant discus-
tion of the rectum from the urinary tract. He sion, particularly between the Australian mem-
decided to present that experience of 56 cases at bers of the audience. They specifically mentioned
the annual meeting of the Pacific Association of that the muscle that was shown in the movie in
Pediatric Surgeons in March 1980 in Colorado that presentation was not the “puborectalis sling”
Springs, Colorado, United States. Basically, in because that structure was only seen in Australia,
this presentation, he proposed to make a longer by Dr. Stephens, in autopsies. In other words, for
midsagittal incision to have better exposure to years, the world’s pediatric surgical community
facilitate the separation of the rectum from the had been talking about how to preserve the
10 1 History of the Treatment of Anorectal Malformations

“puborectalis sling” structure during an opera- near Wisconsin. He invited pediatric surgeons
tion. Yet, nobody has seen such structure, except well known for their interest and experience in
for Dr. Stephens in his autopsy specimens!! the surgical treatment of anorectal malformations
After that meeting, Dr. Peña went back to from all over the world. These surgeons included
Mexico and decided to use a much longer, poste- Dr. Peter deVries, Dr. Subir Chatterjee (India),
rior, midsagittal incision, running from the mid- Dr. Durham Smith (Australia), Dr. Nicolas
dle portion of the sacrum to the base of the Martin del Campo (Mexico City), Dr. Alberto
scrotum, trying to stay exactly in the midline and Peña, Dr. Stephen Dolgin, Dr. Sid Cywes (South
using an electrical stimulator. The purpose of that Africa), Dr. Morihiro Saeki (Japan), Dr. Jotaro
incision was to clarify the controversy about the Yokorama (Japan), Dr. Donnellan (Chicago), Dr.
characteristics and location of the “puborectalis Dale Johnson (Salt Lake City), and Dr. Alex
sling.” During the meeting at Colorado Springs, Holschneider from Germany (Fig. 1.3). As a
he had conversations with different members of result of that meeting, a new classification was
the Pacific Association of Pediatric Surgeons, created, called the “Wingspread classification.”
including Dr. Peter deVries. Most surgeons were Fortunately, that new classification was more
very negative about the idea of “cutting the simplified and therefore more useful and yet,
sphincters.” Dr. deVries, on the other hand, was from our point of view, was still very deficient
enthusiastic and showed a supportive attitude (Fig. 1.4 Wingspread classification) [70].
about the idea. On August 10, 1980, in Mexico In May of 2005, Dr. Holschneider promoted
City at the National Institute of Pediatrics, Dr. another meeting in Krickenbeck, Germany
Peña and Dr. Miguel Vargas performed the first (Fig. 1.5), to rediscuss the subject and a new clas-
posterior sagittal anorectoplasty in a female sification, known as Krickenbeck classification,
patient. They were surprised by the fact that they was created (Fig. 1.6). This Krickenbeck classifi-
were unable to identify anything that looked like cation we feel is much better than the two previ-
a “puborectalis sling.” Dr. Pieter deVries visited ous ones [71].
Dr. Peña and joined him to explore four more From August 10, 1980, until the day of sending
patients in September of 1980. The results of this manuscript to be printed, the authors have
these initial operations were presented at a round- operated on over 2,032 cases, documenting their
table at the World Symposium of Pediatric findings and recording them into a database.
Surgery in Acapulco in September of 1980. Dr. Efforts have been made to try to follow all these
Stephens was part of that roundtable. cases on a long-term basis. The senior author of
Subsequently, Dr. deVries invited Dr. Peña to go this book traveled to many countries, invited by
to Sacramento, California, to operate on four generous pediatric surgeons who helped contrib-
more patients. The experience was presented at ute to accumulate the largest series of cases of this
the Pacific Association of Pediatric Surgeons in condition, operated mostly by a single person, fol-
Hawaii together by Dr. Peña and Dr. deVries. lowing as consistently as possible similar princi-
Subsequently, Dr. Peña and Dr. deVries pre- ples and techniques. Here is a list of the surgeons
sented their experience at the meeting of the with whom the authors are deeply in debt for their
American Academy of Pediatrics in the fall of generosity in inviting the senior author and shar-
1981 in New Orleans. After that, unexpectedly, ing their patients to be operated upon.
Dr. Pieter deVries published the first paper on What started as a controversy related to the
posterior sagittal anorectoplasty [68]. Following anatomy of the sphincter mechanism in patients
that, Dr. Peña submitted another paper 2 months with anorectal malformations represented the main
later on the same subject [69]. catalyst and motivation to study, learn, and collect
In 1984, Dr. Stephens promoted a meeting to an enormous amount of information related with
discuss the possibility of creating a new, more anorectal malformations and associated defects.
practical classification and to rediscuss the whole In 1980, we were not aware of the fact that we
subject of anorectal malformation in Wingspread, were “opening a Pandora’s box.” The controversy
1.2 The Early Times 11

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

over the anatomy of the sphincter mechanism, older patients who had come to our clinic who
seen in retrospect, appears today of little signifi- were born with an anorectal malformation and
cance, compared to the fascinating observations were subjected to blind or semi-blind operations
that emerged from the surgical exploration, eval- that resulted in a urethral stricture and/or acquired
uation, and long-term follow-up of so many urethral atresia, neurogenic bladder, impotence,
patients. retrograde ejaculation, painful ejaculation, and
The road has been long, exciting, wonderful, many other problems.
and illuminating. We have been learning many Now we know that the separation of the rec-
important lessons, and now we are certain that tum from the urogenital tract under direct vision
the more we learn about the subject, the more is a technically demanding maneuver; it is there-
intriguing questions we must answer. The dictum fore easy to understand how much damage we
that “It’s not the unanswered questions, but rather could provoke doing the operation blindly, and
the unquestioned answers” crystallizes well the sadly that is what we were doing prior to 1980.
evaluation of this problem in surgery. This has Being directly exposed to the intrinsic anat-
been a lifetime, very enjoyable, fascinating, hum- omy of these malformations only made more
bling, and extraordinary experience. obvious our naivety when trying to create
Opening the pelvis posterior-sagittally oversimplified classifications of a spectrum of
allowed us, for the first time, to be directly defects, without really knowing the true anatomy.
exposed to the peculiar, complex, intrinsic anat- The most conspicuous lesson learned through all
omy of these defects. Much beyond the anatomic these years is that we have been dealing with a
characteristics of the sphincter mechanism, we spectrum of defects. The more cases we operate
learned about the detailed anatomy of the junc- on, the wider the spectrum becomes.
tion between the rectum and the urogenital tract. Classifications that divide a spectrum of defects,
With that knowledge came the awareness of the in categories such as “high” and “low” or even
potential damage that we could provoke while “high,” “intermediate,” and “low,” represent
trying to separate on these structures blindly. oversimplifications that misguide rather than
Suddenly, we had an explanation for the many help. Now we know that in surgery, it is not
12 1 History of the Treatment of Anorectal Malformations

Fig. 1.4 Wingspread


classification of anorectal
malformation

advisable to use radical terms such as “always” In 1982, Dr. Maricela Zarate, an ex-resident of
or “never.” Being exposed directly to the anatomy Dr. Peña in Monterrey, N.L., Mexico, had a
of these malformations also gave us a unique patient with a cloaca. The available literature at
opportunity to correlate the anatomic findings that time related to the surgical treatment of clo-
with the final functional results and the potential aca was very scant (see Chap. 16).
devastating functional sequelae. In 1982 in Monterrey, Mexico, Dr. Peña, Dr.
Confronted with an anatomy never described Maricela Zarate, and Dr. Marshall Schwartz (vis-
before, we were obligated, by common sense, to iting from the United States) operated via poste-
describe technical maneuvers never described rior sagittal on a girl with a cloaca (Fig. 1.7). That
before. Many remarkable experiences were wait- girl is now an adult, has bowel and urinary con-
ing ahead of us. 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 1 History of the Treatment of Anorectal Malformations

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- patients must be treated in specialized centers
genital mobilization to facilitate the repair of where they will be treated by a multidisciplinary
cloacas [79]. team. With that in mind, we presented the idea to
• Describe the precise anatomy of a malforma- some leaders of prominent children’s hospitals
tion called posterior cloaca [80] in the United States. Dr. Richard Azizkhan had
• Create and implement a bowel management the vision and courage to adopt the plan, and the
program aimed to keep clean patients suffer- Colorectal Center for Children was created at the
ing from fecal incontinence [81–84]. Cincinnati Children’s Hospital Medical Center
It was there, in Long Island, New York, where in July 2005.
it became clear to us that children suffering from
anorectal malformation required and deserved
better care. Yes, we were very proud because we
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65. Banu T, Hannan MJ, Aziz MA, Hoque M, Laila K 84. Bischoff A, Levitt MA, Peña A (2009) Bowel man-
(2006) Rectovestibular fistula with vaginal malforma- agement for the treatment of pediatric fecal inconti-
tions. Pediatr Surg Int 22(3):263–266 nence. Pediatr Surg Int 25(12):1027–1042
66. Santulli TV, Kiesewetter WB, Bill AH Jr (1970)
Anorectal anomalies: a suggested international clas-
sification. J Pediatr Surg 5(3):281–287
Basic Anatomy and Physiology
of Bowel Control 2

The reader may be surprised for not finding in In addition, through the years we found that
this chapter many of the traditional terms histori- what we observed as part of the sphincter mecha-
cally used to refer to the different portions of the nism in one patient was never the same as the one
sphincter mechanism of the human being. We use that we saw in another one. After a large experi-
a different terminology that we believe is realis- ence with the surgical treatment of anorectal mal-
tic, useful, and with important practical and tech- formations, we are now certain to believe that we
nical implications for the practicing surgeon. are dealing with a spectrum of anatomic
This is a result of our observations of the differ- variations.
ent anatomic variants, found in more than 2,032 We are aware of the fact that human beings
surgical explorations of the pelvis and the ano- (and surgeons are not exceptions) prefer to deal
rectum of patients suffering from anorectal mal- with artificial, man-made, classifications to refer
formations, as well as many others operated to and to discuss biological phenomenon. Yet, we
resect tumors and to repair pelvic organs (ure- like to say that Mother Nature does not like our
thra, vagina, and rectum). classifications and continues producing humans,
In the early times, when we performed the first animals, and biological specimens following a
posterior sagittal approaches to repair anorectal spectrum type of pattern. We recognize that it is
malformations, influenced by the traditional con- not easy from the clinical point of view to talk
cepts expressed in the available textbooks on the about spectrums, but we could not ignore reality.
subject [1, 2], we were looking for the “puborec- Figure 2.1 shows what we think is the best
talis sling,” the “pubococcygeal muscle,” the photograph ever taken of the pelvic anatomy of a
“pubourethralis muscle,” the “iliococcygeal mus- male cadaver. The authors managed to show a
cle,” the “superficial portion of the external perfect sagittal section. We feel admiration and
sphincter,” the “deep portion of the external respect for such achievement. This magnificent
sphincter,” and the “internal sphincter,” and we photograph was reproduced with permission
were rather frustrated for not seeing what was from the excellent “Colon Atlas of Human
described in the textbooks. Or at least, what we Anatomy” by R.M.H. McMinn Emeritus
were seeing was very different to what was Professor of Anatomy, Royal College of
described. 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
Electronic supplementary material Supplementary
material is available in the online version of this chapter at Book Medical Publishers, Inc., Chicago 1977,
10.1007/978-3-319-14989-9_2. page 248.

A. Peña, A. Bischoff, Surgical Treatment of Colorectal Problems in Children, 17


DOI 10.1007/978-3-319-14989-9_2, © Springer International Publishing Switzerland 2015
18 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
Fig. 2.1 Photograph of a sagittal section of a human from the upper portion (levator) that is made out
cadaver. 1 Rectus abdominis; 2 Extraperitoneal fat; of horizontal fibers that compress the rectum
3 Sigmoid colon; 4 Promontory of sacrum; 5 Rectum; from behind. However, these different portions
6 Coccyx; 7 Anococcygeal body; 8 External anal sphinc- of the sphincter mechanism do not contract sep-
ter; 9 Anal canal with anal columns of mucous membrane;
10 Perineal body; 11 Ductus deferens; 12 Epididymis; arately; in real life, they contract in a massive
13 Testicle; 14 Spongy part of urethra and corpus unified way (Animations 2.1, 2.2, 2.3, 2.4, and
spongiosum; 15 Corpus cavernosum; 16 Bulbospongiosus; 2.5). The muscle fibers running longitudinally
17 Perineal membrane; 18 Sphincter urethrae; (parallel to the rectum) elevate the anus when
19 Membranous part of urethra; 20 Pubic symphysis;
21 Prostate gland; 22 Prostatic part of urethra; 23 Seminal they contract, whereas those fibers that surround
colliculus; 24 Bristle in ejaculatory duct; 25 Internal ure- the rectum are the ones that produce the com-
thral orifice; 26 Bladder; 27 Bristle passing up into right pression of the rectum posteriorly. Perhaps the
ureteral orifice; 28 Rectovesical pouch point of maximal contraction of this funnel-like
muscle structure is what has been considered
Figure 2.2 shows an MR image of the sagittal the puborectalis sling, but we must emphasize
section of the pelvis of a normal child. It is really that there is no real anatomic separation of these
remarkable what modern imaging technology has structures. Near the skin, the funnel-like muscle
achieved. We invite the readers to look in a very joins other types of muscle that run superficial
detailed way these two pictures (Figs. 2.1 and under the skin and divide into two portions, one
2.2). The same muscle structures shown in the on each side of the anus, and that is why we call
cadaver are present in the MRI picture. A non- those “parasagittal fibers.” The contraction of
biased, objective view of these two figures does these muscles may produce the impression of
not allow us to identify separately the puborecta- closing the anus in a circular fashion; yet, actu-
lis, ischiococcygeal, pubourethralis, and iliococ- ally these are parasagittal fibers that join one
cygeal muscles and deep external sphincter, side with the other, posterior and anterior to the
superficial external sphincter, and internal anal opening. The parasagittal fibers run actu-
sphincter. ally perpendicular to the muscle complex. We
Rather, one can see a muscle structure that arbitrarily use the term “levator” to refer to the
comes from the anterior aspect of the lowest upper portion of the funnel-like muscle mecha-
2 Basic Anatomy and Physiology of Bowel Control 19

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 wise unnecessary diagrams showing their
lower portion. The point where the muscle com- preconceptions and biases [6–13].
plex and parasagittal fibers cross represents the It is even more impressive to see how rather
limits of the sphincter. bizarre anatomic concepts, like the idea of a “tri-
When the anatomy of the anorectal sphincters ple loop” without any evidence to support its
is presented in this rather simplistic way, it is existence, are accepted, published, and repeated
easy to teach and to learn. between surgeons [14, 15].
Traditional anatomy concepts have been In a medical community where everybody
repeated from one generation to another, full of seem to be able to see clearly structures such as
details or concepts without clinical relevance. the puborectalis muscle and all the other portions
The medical students are frequently obligated to of the sphincter mechanism, very few dare to
learn some of those concepts. express skepticism and disagree [16, 17]. During
We have been impressed by publications our literature review, we found an excellent hon-
related with the anatomy of the sphincters; the est paper written by Dr. Arthur F. Dalley II, PhD,
authors frequently show real photographs of the [17]. After a very thoughtful discussion, he con-
anatomy, yet they over-impose arrows to show cludes saying: “I recommend that the three-part
inexistent, imaginary structures [2–5]. In other external anal sphincter be removed from gross
words, photographs show the real anatomy, anatomy texts, dissectors and atlases and be rel-
whereas the diagrams or the arrows show what egated to the junkyard of anatomic trivia where it
the authors wanted to see. may languish for the sake of the historical anato-
More recently, advances in the technology of mist or the rare individual who spends time carv-
imaging show images (Fig. 2.2) of the real anat- ing out the most meticulous of dissections.” This
omy; however, again the authors fabricate other- 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 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
Fig. 2.5 Diagram showing the most common anatomic smaller, the sphincter mechanism weaker, and
sphincter pattern seen in patients with prostatic fistula
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
Fig. 2.6 Diagram showing the most common anatomic that the patient most likely will have fecal incon-
sphincter pattern seen in patients with bladder neck tinence. In fact, as we will discuss in our results,
fistula patients with recto-perineal fistulas have a 100 %
chance of bowel control provided they have a
vis of a normal human being. Figure 2.3 shows good operation. Patients with bulbar fistula have
the most benign of all defects which is the peri- 85 % chances, prostatic fistula 60 % chances, and
neal fistula. The sphincter mechanism in these bladder neck fistula only 20 % chances. Similarly,
types of cases is almost normal. The rectum, on characteristically, the sacrum usually is more and
the other hand, deviates in its lowest portion to more primitive, as we go into higher and higher
22 2 Basic Anatomy and Physiology of Bowel Control

locations of the rectum, as well as the zero possibility of having bowel and urinary con-
characteristics of the sphincter and the space trol. Patients with anorectal malformations are
available between the sacrum and pubis. The represented by a spectrum in terms of sacrum
chance of suffering from tethered cord also deficiency that goes from patients with normal
increases in higher malformations. The exception sacrum to patients with completely absent
is represented by some patients with perineal fis- sacrum.
tulas. This particular group of patients has more We know that under normal circumstances,
tendencies to suffer from presacral masses and the nerves that innervate the bladder neck urinary
tethered cord. The anatomic differences shown in tract and corpora come from the orifices of the
Figs. 2.3, 2.4, 2.5, and 2.6 and Animations 2.2, sacrum and run lateral to the rectum in order to
2.3, 2.4, 2.5, and 2.6 are seen in real life. However, reach the corpora, the bladder neck, and the rec-
as it is well known by all surgeons, in medicine tum itself. Therefore, from the early times, Dr.
and surgery, there are no “nevers” and there are Douglas Stephens [25] recommended (and is still
no “always.” In other words, it is possible to see, valid) to try to remain exactly in the midline as
although very unusual, a perineal fistula with much as possible during our surgical explora-
very poor sphincter mechanism and also to see a tions. Once the surgeon reaches the rectum, all
recto-bladder neck fistula with a rather good- the dissection of the rectum must be performed,
looking sphincter mechanism, but those are staying as close as possible to the rectal wall to
exceptions. Most of the time, as the rectum is avoid the damage of nerves that supposedly run
located higher connecting to the urogenital tract, lateral to the rectum. In other words, the further
the sacrum tends to be shorter, the distance away from the rectum, the more chances to injure
between the sacrum and pubis decreases, and the nerves.
characteristics of the sphincter become more and Concerning the anatomy of the pelvic auto-
more rudimentary and weak. 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
2.3 Nerves is essential to remain in the midline while
approaching the pelvis and to dissect the rectum
We surgeons frequently refer to the “lack of remaining as close as possible to the bowel wall,
nerves” when we deal with patients with anorec- in order to minimize the possibilities to injure
tal malformations with bad functional prognoses. autonomic nerves and avoid neurogenic bladder
Yet, we actually never see the nerves when oper- and impotence.
ating on these patients. There are no precise sci- We have evidence from patients that had pre-
entific studies that give evidence of the presence vious failed attempted repairs and that were born
or absence, as well as characteristics and precise with a “good” malformation and yet they suf-
location, of the pelvic nerves in cases of anorectal fered from fecal and sometimes urinary inconti-
malformations. We work, always assuming, that nence. Reading the operative reports of those
the higher the location of the rectum and its con- patients, one finds that the surgeon actually got
nection to the urogenital tract, as well as the more lost and certainly went out of the midline, which
deficient sacrum is, the more deficient the nerves may explain the nerve damage.
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 2.4 Blood Supply
also assume that in a patient with absent sacrum,
all the nerves that normally come out of the seg- Unfortunately, there are no scientific detailed
ments of the sacrum and innervate the pelvic anatomic studies of the blood supply of the pelvic
organs are absent, or deficient, and that may organs of patients with anorectal malformations.
explain why patients with absent sacrum have Again, what we have learned from our surgical
2.5 Basic Physiology Principles of Bowel Control 23

explorations is that it seems like the rectum has C. Rectosigmoid motility and reservoir function
an excellent intramural blood supply, as evi- A. Sensation – this is the first indispensable ele-
denced by the fact that in every case of anorectal ment for bowel control. The anal canal is per-
malformation that we operate on, we separate the haps the most sensitive part of the human
rectum from the urogenital tract, and then we body. There, we are capable of discriminating
have to mobilize the rectum enough to reach the gas, from liquid and from solid fecal matter
perineum. In order to do that, we performed a cir- [27]. The anal canal remains collapsed by the
cumferential dissection, dividing all the extrinsic effect of the muscle tone of the sphincter
vessels and bands that hold the rectum up in the mechanism that surrounds it. When the fecal
pelvis. In other words, we are basically devascu- matter (liquid, solid, or gas) reaches the anal
larizing the rectum. We sacrifice its entire extrin- canal due to active rectal peristalsis, we per-
sic blood supply. Yet, provided the wall of the ceive it and, depending on the surrounding
rectum remains intact and the inferior mesenteric circumstances, decide to use our voluntary
vessels are preserved, the intramural blood sup- sphincter mechanism, to occlude the lumen
ply of the proximal part provides enough to of the anal canal and avoid a bowel move-
maintain alive the distal part of the rectum. This ment, until the circumstances are appropriate
finding should not be extrapolated to other parts to have a bowel movement.
of the colon. The blood supply of the colon in Above the anal canal, in the rectum, we do
patients with anorectal malformations is basi- not have the exquisite sensation described for
cally the same as in normal individuals, except in the anal canal. However, a distention of the
patients with cloacal exstrophies and patients rectum with a balloon causes a vague sensa-
with a malformation called “rectal pouch” in tion of fullness that is known as propriocep-
which the entire colon is represented by a single tion [28].
saccular piece of colon with a very abnormal The implications of these facts for us sur-
bizarre type of blood supply (picture). geons are obvious. We must try to preserve
intact the anal canal during our operations in
patients with normal anal canal, such as
2.5 Basic Physiology Principles patients suffering from Hirschsprung’s dis-
of Bowel Control ease, severe constipation, inflammatory
bowel disease, and familial polyposis.
We take care of many babies born with severe The overwhelming majority of patients
anatomic deficiencies; we can certainly repair with anorectal malformations are born with-
their anatomy, but we cannot restore their normal out an anal canal, except for a rare malforma-
function; however, we try to help them to have a tion called rectal atresia. Patients with
normal social life, with the implementation of perineal fistula have a rather primitive anal
our “bowel management program.” Unfortunately, canal. This means that patients with anorectal
there is another large group of patients who suffer malformations, under the best circumstances,
from fecal incontinence as a consequence of a do not have “perfect” bowel control. Many
technically deficient operation. That is obviously patients born with a malformation with good
something highly regrettable, but most important functional prognosis behave like normal chil-
is the fact that it is preventable. For that, it is dren, yet when they have a severe episode of
imperative for the surgeon to know a few basic diarrhea, it becomes evident that they are less
but extremely important anatomic and physio- than perfect in terms of bowel control.
logic principles. B. Sphincter – the voluntary sphincter mecha-
In order to have bowel control, it is necessary nism maintains a certain tone constantly.
to have three very important elements: However, there is a common misconception
A. Sensation consisting on believing that a sudden relax-
B. Sphincter ation of the sphincter will produce escape of
24 2 Basic Anatomy and Physiology of Bowel Control

feces. That is simply not true. In fact, a human does not have episodes of diarrhea or multiple,
being can only have a bowel movement when irregular, unpredictable bowel movements.
the rectosigmoid has a peristaltic wave that Using our imagination, we conceive the pos-
pushes the stool out. Human beings actually sibility of manipulating the rectosigmoid motil-
use the voluntary sphincter occasionally, to ity, using pharmacologic agents, in order to
prevent the passing of gas or fecal matter in paralyze the rectosigmoid when required and to
inadequate circumstances. provoke a peristaltic wave to empty the rectosig-
C. Rectosigmoid motility and reservoir func- moid when the surrounding circumstances are
tion – after many years of working with appropriate. In fact, we consider that kind of
patients suffering from bowel control prob- treatment more likely to be successful rather than
lems, we came to realize that rectosigmoid trying to reconstruct the sphincters or use artifi-
motility and its reservoir function are the cial sphincters, without taking into consideration
most important element for bowel control. the two more important elements that are sensa-
The rectosigmoid remains relaxed most of the tion and motility.
time, acting as a reservoir of fecal matter.
That is an extremely important function,
since it allows human beings to have a social References
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16(6):482–489 28. Turell R, Krakauer JS, De Maynard AL (1953)
19. Penninckx F, Lestar B, Kerremans R (1992) The inter- Colonic and anorectal function and disease. Surg
nal anal sphincter: mechanisms of control and its role Gynecol Obstet 96(4):313–339; contd
Prenatal Diagnosis
3

Significant advances had been achieved in the hours or days of life are crucial for the future of
field of prenatal diagnosis in general, as a conse- the baby. Some mistakes that occur in the early
quence of amazing developments in the imaging management of these babies may have impor-
technology. The use of ultrasound to visualize the tant repercussions for the future quality of life
fetus in utero already meant a great step in the of the baby. That is the reason why in cases of
diagnosis of gross malformations in utero [1–8]. complex defects that affect the colorectal area
Subsequently, the MRI amplified the possibilities as well as the urogenital tract, we must advise to
of making much more accurate diagnosis of mul- deliver the baby in a specialized center, where a
tiple congenital defects [9–11]. multidisciplinary team of experts, with the nec-
At the present time, we cannot claim that we essary experience in that field, takes care of
can make accurate prenatal diagnoses of anorec- him/her.
tal malformations. More specifically, we cannot
determine the precise type of defect that the fetus
has. Yet, with the current technology, we can 3.1 Male Fetuses
make gross diagnoses that allow us to make
important decisions and formulate meaningful A frequent in utero finding in babies with anorec-
recommendations [12–19]. tal malformations is the presence of a dilated
The benefits of the prenatal diagnosis in ano- bowel (Fig. 3.1). This is a nonspecific finding that
rectal malformations, as well as in all defects, can be present in other conditions such as
include the possibility of giving the parents a Hirschsprung’s disease.
fairly accurate idea of the functional prognosis The presence of intraluminal calcifications
of the future baby, which will influence his/her makes the dilated bowel sign more significant,
quality of life. This, beyond ethical and moral since we know that the mixing of urine with
issues, will help the parents to make important meconium frequently produces calcifications
decisions related to the possibility of terminat- (Fig. 3.2). We must keep in mind that over 80 %
ing the pregnancy. In addition, depending on the of the patients with anorectal malformations have
specific type of defect, as well as its complexity, a rectal urinary fistula, and meconium may go
we can advise the parents as to the best possible into the urinary tract as well as urine into the
place where the baby should be delivered, in rectum.
order to receive optimal, comprehensive, and There are several signs that can be clearly seen
high-quality care. In some of the most serious by ultrasound and particularly with an MRI study
anorectal and urogenital malformations, the that will make the diagnosis of anorectal malfor-
therapeutic decisions taken during the first few mation more likely. These signs include:

A. Peña, A. Bischoff, Surgical Treatment of Colorectal Problems in Children, 27


DOI 10.1007/978-3-319-14989-9_3, © Springer International Publishing Switzerland 2015
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 29

Fig. 3.4 Tethered cord diagnosed in utero. Arrow shows


the low lying location of the conus

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
Fig. 3.5 Absent kidney in a fetus with anorectal require special care to protect those kidneys
malformation 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 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

Fig. 3.8 Omphalocele, in utero diagnosis. O omphalo- 1. Baronciani D, Scaglia C, Corchia C, Torcetta F,
cele, M meningocele 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-
Fig. 3.9 “Elephant trunk” (intussuscepted prolapsed ings and differential diagnosis. Obstet Gynecol 72(3
ileum) emerging in between two hemibladders. Arrow Pt 2):464–469
shows uterine wall 6. Lande IM, Hamilton EF (1986) The antenatal sono-
graphic visualization of cloacal dysgenesis. J
Ultrasound Med 5(5):275–278
bladder which is described or diagnosed prena- 7. Odibo AO, Turner GW, Borgida AF, Rodis JF,
tally as “absent bladder.” Campbell WA (1997) Late prenatal ultrasound fea-
The omphalocele (Fig. 3.8) can be easily seen tures of hydrometrocolpos secondary to cloacal
anomaly: case reports and review of the literature.
in utero. The elephant trunk can also be seen Ultrasound Obstet Gynecol 9(6):419–421.
(Fig. 3.9), as well as the separated pubic bones doi:10.1046/j.1469-0705.1997.09060419.x
and the meningocele (Fig. 3.8). 8. Ohno Y, Koyama N, Tsuda M, Arii Y (2000) Antenatal
The prenatal diagnosis is particularly impor- ultrasonographic appearance of a cloacal anomaly.
Obstet Gynecol 95(6 Pt 2):1013–1015
tant in this extremely serious malformation con- 9. Hung YH, Tsai CC, Ou CY, Cheng BH, Yu PC, Hsu
sidering the very poor quality of life that these TY (2008) Late prenatal diagnosis of hydrometrocol-
patients will have during their future life, particu- pos secondary to a cloacal anomaly by abdominal
larly when they have a severe spinal deficiency ultrasonography with complementary magnetic reso-
nance imaging. Taiwan J Obstet Gynecol 47(1):79–
that may translate into incapacity to walk, for 83. doi:10.1016/s1028-4559(08)60059-5
life. We believe that it is extremely important for 10. Hayashi S, Sago H, Kashima K, Kitano Y, Kuroda T,
pediatric surgeons, pediatric urologists, as well Honna T, Natori M (2005) Prenatal diagnosis of fetal
32 3 Prenatal Diagnosis

hydrometrocolpos secondary to a cloacal anomaly by persistent cloaca. Congenit Anom (Kyoto) 49(3):116–
magnetic resonance imaging. Ultrasound Obstet 117. doi:10.1111/j.1741-4520.2009.00236.x
Gynecol 26(5):577–579. doi:10.1002/uog.2584 15. Warne S, Chitty LS, Wilcox DT (2002) Prenatal diag-
11. Picone O, Laperelle J, Sonigo P, Levaillant JM, nosis of cloacal anomalies. BJU Int 89(1):78–81
Frydman R, Senat MV (2007) Fetal magnetic reso- 16. Cilento BG Jr, Benacerraf BR, Mandell J (1994)
nance imaging in the antenatal diagnosis and man- Prenatal diagnosis of cloacal malformation. Urology
agement of hydrocolpos. Ultrasound Obstet Gynecol 43(3):386–388
30(1):105–109 17. Cacciaguerra S, Lo Presti L, Di Leo L, Grasso S,
12. Mori M, Matsubara K, Abe E, Matsubara Y, Gangarossa S, Di Benedetto V, Di Benedetto A (1998)
Katayama T, Fujioka T, Ito M (2007) Prenatal diag- Prenatal diagnosis of cloacal anomaly. Scand J Urol
nosis of persistent cloaca associated with VATER Nephrol 32(1):77–80
(vertebral defects, anal atresia, tracheo-esophageal 18. Morikawa M, Yamada T, Cho K, Yamada H,
fistula, and renal dysplasia). Tohoku J Exp Med Minakami H (2006) Prenatal diagnosis and therapy of
213(4):291–295 persistent cloaca: a case report. Fetal Diagn Ther
13. Baier SR, Tank ES, Watson PT (2001) Persistent 21(4):343–347. doi:10.1159/000092463
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colpos. J Diagn Med Sonog 17(4):220–224. MA, Lim FY, Hall J, Pena A (2012) Prenatal counsel-
doi:10.1177/87564790122250471 ing for cloaca and cloacal exstrophy-challenges faced
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Y, Sugiura-Ogasawara M (2009) Prenatal diagnosis of 788. doi:10.1007/s00383-012-3133-3
Neonatal Management
4

4.1 Introduction particular malformation only occurs in 1 % of all


patients born with anorectal malformations [10].
In dealing with most congenital anomalies, errors
that occur during the first few hours or days of
life may have serious consequences and sequelae. 4.2 Most Common Scenario
Early accurate diagnoses, as well as adequate
therapeutic decisions, require a high index of sus- As pediatric surgeons, we are called to see a baby
picion from pediatricians, neonatologists, pediat- that is just born and has no anus. There are two
ric surgeons, pediatric urologists, nurses, and very important questions to be answered.
other physicians and surgeons who take care of 1. Does the baby have a serious associated defect
neonatal babies with congenital anomalies. that may kill him/her within the next few
Our eyes only see what our mind suspects. hours or days of life?
2. Does the baby need some sort of primary anal
It is hard to believe that in this twenty-first cen- repair or a colostomy?
tury in developed countries, we still hear of These questions should be answered in the
patients born with imperforate anus that were sent same order that they are presented here. In other
home as “normal babies.” Subsequently, it was the words, the surgeon must refrain from jumping
mother who made the diagnosis or the babies suf- into trying to find the answer of the second ques-
fer from bowel perforation and some of them die tion but rather concentrate during the first 24 h of
[1–9]. We cannot overemphasize the importance life in trying to answer the first question.
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 4.3 Answering the Two Most
pediatric surgery; it is not enough by looking at the Important Questions
external appearance of the anus, but it is rather
necessary to introduce a little catheter or thermom- At this point, the neonatologist and the surgeon
eter to be sure that the anus is patent. There is one should remember that about 30 % of all babies with
specific type of defect in which the babies have a anorectal malformation have some sort of cardio-
normal-looking anus externally, a normal anal vascular condition [11]. However, only one third of
canal, approximately 1–2 cm deep, and then an that 30 % have serious hemodynamic repercus-
atresia. That type of defect is the one that can only sions that mandate to implement some sort of med-
be diagnosed by trying to pass a thermometer, ical or surgical treatment [11]. The most common
catheter, or an instrument through the anus. That cardiac congenital anomalies seen in these cases

A. Peña, A. Bischoff, Surgical Treatment of Colorectal Problems in Children, 33


DOI 10.1007/978-3-319-14989-9_4, © Springer International Publishing Switzerland 2015
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 35

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 hemisacrum, which means that the patient has a
healthy. presacral mass; this finding has important thera-
Taking a baby to the operating room to repair peutic and prognostic implications.
an anorectal malformation or to open a colostomy An ultrasound of the lower sacrolumbar spine
only to find that the baby gets very sick during is extremely useful and must be done during the
the operation because he has a serious cardiac, first hours of life to determine whether or not the
esophageal, or kidney problem is an undesirable baby has tethered cord (Fig. 4.7). This is also
and preventable experience. important to determine the functional prognosis
All babies with anorectal malformations must for bowel and urinary control.
have an abdominal x-ray film that shows the degree All these studies can be done during the first
of bowel dilatation, the characteristics of the spine, 24 h of life. Babies with anorectal malformations
and the characteristics of the sacrum. The presence are usually not born with a distended abdomen. It
of intraluminal calcifications in the rectosigmoid takes a few hours for the abdomen to start becom-
most likely is due to the passing of urine to the ing distended. It is after 24 h of life that the
bowel. Urine mixed with meconium may produce abdominal distention becomes critical, and a
calcifications [14, 15]. The sacral films must management decision must be taken.
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, 4.4 Physical Examination
and sexual function. Traditionally, we evaluated
the sacrum by counting the number of vertebrae. 4.4.1 Male Patients
We found this to be a rather limited nonuseful way,
and therefore, we created what we call the sacral During our first contact with the baby with ano-
ratio (Figs. 4.5 and 4.6) (see Chap. 6). rectal malformation, we should dedicate a special
Taking x-ray films of the sacrum is mandatory time for a meticulous detailed examination of
also because we must rule out the presence of a the baby’s perineum. Babies with anorectal
36 4 Neonatal Management

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- between the tip of the coccyx and the anal dim-
ances of their perineum, and they have very ple, the poorer the prognosis, either because the
important clinical significance. anal dimple is located too anteriorly or because
The presence of a flat bottom, meaning the sacrum is very short or both. We call it a
absence of the normal midline groove that all “good-looking perineum” when the baby has a
human beings have between both buttocks, is well-formed midline groove and a well-located
usually associated with malformations with bad anal dimple (Fig. 4.9a). Even by touching that
prognosis and very highly located rectum area, one can see the contraction of the sphincter
(Fig. 4.8). In addition, most patients with anorec- of the anal dimple.
tal malformations have an anal dimple that repre- The most “benign” of all anorectal malforma-
sents the point in the perineum where the patients tions is called “perineal fistula.” The rectum
have the largest concentration of sphincter fibers opens into the perineum, anterior to the sphincter
(Fig. 4.9). The more prominent the anal dimple, in a rather narrow orifice. This malformation is
the better the quality of the sphincter and there- also known as a “low defect.”
fore the prognosis. The absence of an anal dimple A perineal fistula in a male patient may have
is a very bad sign, usually present in poor prog- different external manifestations. A common one
nosis type of defects. The location of the anal is the presence of a malformation called “bucket
dimple varies from patient to patient. The closer handle” (Fig. 4.11) which is a prominent skin
the anal dimple to the scrotum, the worse the band under which we can pass a mosquito clamp.
prognosis (Fig. 4.10). The longer the distance Another external manifestation of a perineal
4.4 Physical Examination 37

a Anterior-Posterior Lateral
A

A B

C
B

BC
= 0.77
AB
C

Normal Ratio: BC =.74


AB
b Anterior-Posterior Lateral

Fig. 4.5 Normal sacral ratio. (a) Diagram. (b) Radiograph

fistula can be a subepithelial fistula (Fig. 4.12). The exception would be the group of patients
The fistula tract may be full of meconium, giving with perineal fistulas associated to an abnormal
the appearance of a “black ribbon.” Other times, sacrum and a presacral mass. Ironically, the pres-
it may be full of white mucous material ence of a presacral mass and hemisacrum seems
(Fig. 4.13). The black or white subepithelial tract to be more frequently associated to perineal fistu-
may extend toward the scrotum in the midline or las than to other anorectal defects.
even to the base of the penis. Occasionally, one may see a patient with a
A perineal fistula can be repaired with an ano- subepithelial fistula or a “bucket handle” malfor-
plasty during the neonatal period without a colos- mation; we try to repair the defect only to find
tomy and has an excellent functional prognosis. 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


neck fistula
Fig. 4.7 Spinal ultrasound of a newborn baby. (a)
Normal. (b) Tethered cord
dure and open a colostomy. That would depend
on the degree of experience of the operator.
fistula and the rectal pouch is located high in the The presence of bifid scrotum (Fig. 4.15) is
pelvis. That is a real exceptional situation usually associated to a rather complex defect;
(Fig. 4.14). Under those circumstances, the sur- most likely the rectum is located high in the
geon has to decide to continue the operation to pelvis, connecting to the urinary tract very high
mobilize the rectum down or to abort the proce- (bladder neck or prostatic fistula), although there
4.5 Female Babies 39

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 adequately as an anus. One can identify the anal
that we usually repair at the same time that we dimple very clearly located posterior to the peri-
repair the anorectal malformation (see Chap. 23, neal or the vestibular fistula.
Sect. 23.5). 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
4.5 Female Babies the genitalia (Fig. 4.18).
When the female baby has no anus and the
In female patients, the surgeon must be particu- external genitalia look rather small (Fig. 4.19),
larly careful in the examination of the baby’s the surgeon should suspect that the baby has a
perineum and genitalia. One should put the baby cloaca. A cloaca is defined as a malformation in
in a convenient lithotomy position with some- which the baby is born with a single perineal ori-
body holding the baby’s legs. We should have fice. Early diagnosis by inspection of this defect
good illumination and magnifying glasses if nec- is extremely important. The surgeon must
essary and take the time to clean the genitalia and remember that about 90 % of the patients with
to separate the labia to see if there is meconium cloacas may have serious urologic problems. The
and precisely where it comes from. Some fistulas surgeon must take the time and be meticulous
are very narrow, and it takes several hours, some- enough to separate the little labia of those small-
times up to 24 h, for the babies to pass meco- looking female genitalia and will be able to see a
nium. The most common defect seen in babies is single perineal orifice, and by doing that, he/she
a malformation called vestibular fistula. As we already made a diagnosis of a cloaca. Some
separate the labia, we can see the urethral open- babies are born with genitalia that induce the
ing, the hymen, and the vaginal orifice, and doctor to make a diagnosis of intersex or a disor-
immediately behind that, but still within the der of sexual development. That is because the
introitus of the baby, one can see another little patients have a structure that looks like a phallus
orifice that we call vestibular fistula (Fig. 4.16). (Fig. 4.20). They have a single perineal orifice
The second most common defect that we see and no testicles, and therefore, the doctors are
is what we call perineal fistula; the orifice is incapable of saying whether the baby is male or
located somewhere between the normal location female. In fact, about 60 of our patients with clo-
of the anus (anal dimple) and the vestibule of the aca born in other institutions have been sent to us
genitalia (Fig. 4.17). Frequently, that orifice, ves- with a label or misdiagnosis of “intersex.” These
tibular or perineal, is too narrow to function families were told that the baby had an
40 4 Neonatal Management

Fig. 4.11 “Buckle handle” malformation in a baby with


a perineal fistula

Fig. 4.10 Location of the anal dimple. (a) Normal dis-


tance between the scrotum and the anal dimple (malfor- Fig. 4.12 “Black ribbon” appearance of a subepithelial
mation with good functional prognosis). (b) Anal dimple fistula, external manifestation of a perineal fistula
next to the scrotum (malformation with less than optimal
functional prognosis). Arrow shows the anal dimple
malformation. In fact, we have never seen a baby
with a cloaca with sexual developmental disor-
undetermined gender and therefore needs a full der. The key for the diagnosis in this case is to
evaluation by a geneticist, endocrinologist, and palpate that prominent structure that looks like a
urologist. A series of tests were run, only to find phallus. In a real phallic hypertrophy like in cases
out that the baby actually is XX and is otherwise of adrenal hyperplasia, one can palpate the cor-
a normal female, except for the cloaca pora inside that structure, whereas in babies with
4.6 Neonatal Management 41

Fig. 4.13 “White ribbon” appearance of a subepithelial


fistula, external manifestation of a perineal fistula

cloacas, the palpation reveals that there is only Fig. 4.14 Intraoperative aspect of a long narrow perineal
folded prominent skin with no corpora and that fistula communicating with a very high rectum
makes the diagnosis of a cloaca with no need to
rule out an intersex. aspiration. A urinalysis is ordered particularly in
A baby with Down syndrome and absent anal male babies, looking for the presence of meco-
orifice has over 90 % chances to have an imperfo- nium in the urine. We also prescribe intravenous
rate anus with no fistula (see Chap. 12). 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-
4.6 Neonatal Management nium to determine whether or not the fistula is
competent to decompress the abdomen and avoid
When we see the baby for the first time, we must abdominal distention. We must explain to the
make a series of management suggestions to our neonatologist that during the following 20–24 h,
colleagues, neonatologists, or pediatricians. the baby should have the diagnostic studies that
These include to start the administration of intra- we already mentioned, including a chest film, an
venous fluids, to maintain the baby with nothing abdominal film, an echocardiogram, an ultra-
by mouth, and to introduce a nasogastric tube to sound of the lumbosacral spine, an ultrasound of
avoid vomiting. The nasogastric tube does not the kidneys, and an ultrasound of the pelvis. In
interfere with the development of abdominal dis- babies with cloacas, we emphasize the need to do
tention that the baby will have in the following an ultrasound of the kidneys and also an ultra-
24 h, but will avoid the risk of vomiting and 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 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,
Fig. 4.20 “Phallus-like” prominent skin in a patient with attend school, and avoid psychological sequelae.
a cloaca. Frequently confused with “intersex” On the other hand, if the baby was born with a
good prognosis type of defect, we have the plea-
Many parents of patients born with anorectal sure to tell the parents the good news.
malformations describe their unfortunate experi- Once we have all of the information that we
ence of having a newborn baby with an anorectal already described and we are certain that the
malformation. They mention that they were a baby does not have a serious associated malfor-
young happy couple with great expectations and mation that requires urgent care, we are ready to
hopes about having a baby. After the delivery of answer the second question, related to the possi-
the baby, a doctor appeared in the mother’s room bility of opening a colostomy or doing some sort
and said that the baby had “no anus.” Most par- of primary operation to create an anal opening.
ents never heard of this malformation because it Most of the time, with the meticulous examina-
is not the type of malformation that people like to tion of the perineum, the result of the urinalysis,
talk about. As a consequence, most parents never or the obvious presence of meconium in the
heard of the existence of this malformation. Then urine, as well as the results of the ultrasound and
the doctors tell the family that the baby is going the radiology studies, with a good index of suspi-
to have an operation, and subsequently, they cion, we have enough information to make a
come back to say that the operation was “suc- decision. Occasionally, after 24 h, in spite of all
cessful.” The parents feel happy and take the the studies and examinations, we still do not have
baby home, but most of the time nobody dis- a clear idea of what we are going to do (primary
cusses the future of the baby. At that time, the repair or colostomy). In the old times, it was cus-
baby is either on diapers or has a colostomy. tomary to take an upside-down film [16] in lateral
Eventually, the baby undergoes the main repair of position and with a marker in the anal dimple,
the malformation followed by a colostomy clo- with the specific purpose of measuring the dis-
sure. Since the baby is usually still wearing tance between the skin of the perineum and the
4.6 Neonatal Management 45

blind end of the rectum full of gas. At some point, the rectosigmoid to become distended. In addi-
it became obvious that the same image obtained tion, the rectum is surrounded by a striated
with the upside-down film could be obtained with funnel-like sphincter mechanism (see Chap. 2)
the baby in a prone position, with the pelvis with a significant tone. The muscle tone keeps the
elevated [17] (Fig. 4.21). In addition, it was also most distal part of the rectum collapsed, until the
risky to put the baby upside down, for the risk of intraluminal pressure is high enough to overcome
vomiting and aspiration. the muscle tone. This occurs usually after 24 h;
The cross-table lateral film renders a reliable therefore, all imaging diagnostic tests aimed to
image when it is taken 24 h post birth. When the detect the location of the blind rectum are inac-
rectal bubble is located well below the coccyx, curate when performed before 24 h of life. This
the surgeon knows where to expect to find the important fact is rarely mentioned. This is the
rectum (Fig. 4.21). reason why we emphasize to spend the first 24 h
Babies with a flat bottom, poor sacrum, and of the baby’s life in trying to rule out serious
tethered cord need a colostomy most of the time. associated conditions and try to determine the
Also, in babies that are passing meconium with location of the rectum to decide the surgical
the urine, we suggest to open a colostomy. approach after a 24 h period.
Patients with “bucket handle” malformations, In order to determine the position of the rectal
subepithelial fistula, or an obvious perineal fis- pouch, different authors suggest using a perineal
tula opening can be repaired primarily during the ultrasound [18–21]. Others are enthusiastic about
newborn period. the perineal injection of contrast material [22–
There is one particular physiologic event 25]. More advance and sophisticated imaging
worth discussing, because of its diagnostic and technology has been used, including CT scan
therapeutic implications. At birth, most babies [26] and MRI [27–29].
with anorectal malformations do not have a dis- None of the publications related with the opti-
tended bowel and abdomen. It takes 18/24 h for mal imaging diagnostic studies to determine the

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
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
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Holland AJ (2010) Delayed diagnosis of ano-
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11. Stoll C, Alembik Y, Dott B, Roth MP (2007)
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forate anus. Pediatr Surg Int 3:110–113
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later (sometimes more), the patient is usually intraluminal meconium in newborn males with imper-
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(1992) Intraabdominal calcifications in the newborn:
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31(361):47–49 Mitra SK, Pathak IC (1983) Prone cross-table lateral
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(2001) Colorectal perforations in neonates with ano- “low” imperforate anus. J Pediatr Surg 14(6):798–800
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4. Maletha M, Khan TR, Gupta A, Kureel SN (2009) Shkolnik A (1989) Ultrasound of the distal pouch in infants
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Colostomy
5

5.1 Introduction separate both stomas enough, as to allow the


proximal (functional) stoma to be covered by a
Colostomy is a procedure designed to divert the stoma bag without including the distal stoma
fecal stream from the normal passage to the rec- (Fig. 5.1). Another way to achieve a totally divert-
tum, creating an opening between the colon and ing procedure is by closing the distal end, a
the abdominal wall. This procedure was created maneuver that is known as a “Hartmann pouch.”
to relieve the obstruction of the colon produced The closure of the distal stoma leaves the patients
by acquired or congenital conditions. Another with a completely closed blind loop distal bowel;
indication is to avoid the passing of stool through this, in the absence of a fistula, creates a mucocele
an operated area, trying to prevent complications, (accumulation of mucus) which will represent a
such as infection and/or dehiscence [1–6]. serious problem for the patient, as most muco-
Historically, it is considered that the first celes eventually become infected. Mucoceles may
colostomy in pediatrics was performed in 1783 also occur in cases in which the fistula that con-
by Antoine Dubois in a 3-day-old infant with an nects the colon with the urogenital tract is very
imperforate anus [7]. narrow and does not allow the passing of mucus.
A colostomy can be permanent, when it is In addition, patients with a Hartmann pouch can-
considered that there is no way to reconstruct the not have contrast studies done through the distal
colon distal to the stoma or there is no way to stoma to evaluate their anatomy prior to recon-
establish bowel control, and it is considered that struction because there is no access to it. This is a
the quality of life is better with a stoma as com- serious deficiency, because we firmly believe the
pared without stoma. most valuable diagnostic test in patients with ano-
Temporary colostomies are those created for a rectal malformation consists in the injection of
period of time until the anatomic or functional contrast material through the distal stoma (high-
circumstances of the patients allow the reestab- pressure distal colostogram) (see Chap. 6).
lishment of the colonic transit. Colostomies can A partially diverting stoma, by definition,
be divided into two groups: totally diverting and allows most of the stool to come out of the body,
partially diverting. but some of the stool still may go into the distal
Totally diverting colostomies are those that colon. This happens when both stomas are
divert the entire fecal stream and do not allow the together and covered by a single stoma bag, and
spillage or passing of stool into the bowel distal to the surgeons open a colostomy known as “loop
the stoma. In order to achieve total diversion of colostomy” (Fig. 5.2).
the stool, it is necessary to separate the proximal The indication to divert the fecal stream totally
and distal bowel after the colon is divided and to or partially depends on the specific problem of

A. Peña, A. Bischoff, Surgical Treatment of Colorectal Problems in Children, 49


DOI 10.1007/978-3-319-14989-9_5, © Springer International Publishing Switzerland 2015
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, diverting colostomy (loop or both stomas located
the surgeon should keep in mind that over 85 % too close one to the other) exposes the patient to
of all the patients have a connection between the the passing of stool into the urinary tract and/or
distal colon and the urogenital tract. In addition, to a distal impaction of stool that cannot pass
5 % of the total group of anorectal malformations through a narrow fistula. Loop colostomies are
has a completely blind distal bowel. This is very very appealing for most surgeons. We think the
important to remember, because a partially 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 5 Colostomy

Fig. 5.5 Diagram of a left transverse 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


Fig. 5.6 Diagram showing a descending colostomy with with colorectal problems. These are indicated
separated stoma (preferred by the authors) when, for some specific reason, we cannot use the
colon to perform the diversion. In other words, the
stoma(s) is opened in the left upper quadrant entire colon does not function, such as in cases of
of the abdomen (Fig. 5.5). total colonic aganglionosis or when the patient was
• Descending colostomy: The bowel is divided born with no colon or has lost the entire colon. An
immediately distal to the descending colon, in ileostomy is sometimes used when a patient had a
the first mobile portion of the sigmoid, and the pull-through of the ascending colon, and therefore
stoma is usually opened in the left lower quad- the patient needs a diversion proximal to this.
5.4 To Divert or Not to Divert, That Is the Question 53

5.4 To Divert or Not to Divert, mation may need a colostomy, and yet another
That Is the Question patient with exactly the same type of defect, but
under different surrounding circumstances, may
In general, pediatric surgeons and general sur- not require a colostomy. This includes how sick
geons are looking for safe ways to perform the patient is, how severe are their associated
colorectal procedures without a protective colos- defects, how advanced is the technology avail-
tomy. By doing this, the patients are prevented able for the patient, how much experience the
from having two extra serious operations with surgeons have in the performance of primary pro-
significant morbidity (colostomy opening and cedures done without a colostomy, and how
colostomy closure). That is the reason why to sophisticated is the infrastructure that surrounds
perform colorectal surgery without a colostomy the patient, including laboratory, intensive care,
is always an attractive idea (see Chap. 4). Taking surgical technology, availability of central venous
advantage of modern surgical technology, effi- access, hyperalimentation, and a clean
cient methods to clean the colon, the possibility environment.
of keeping the patient with nothing by mouth for A colostomy definitely still has a recognized
a period of time receiving parenteral nutrition, protective value in the postoperative course of
the use of sophisticated surgical techniques, and most colorectal and anorectal operations. In other
the availability of powerful antibiotics, allow, words, not opening a protective colostomy has a
nowadays, to perform successful operations on definite increased risk for the patient. Admittedly,
the colorectal tract without a protective colos- many colorectal procedures can be done success-
tomy. Yet, catastrophic complications still hap- fully without a protective colostomy, but cannot
pen [8]. It is true, the incidence of these be done without the acceptance of a certain
complications is much less than in the past, but degree of risk.
unfortunately they still occur. That is why the Finally, we believe that when a surgeon is con-
question whether “to divert or not to divert” is fronted with the difficult decision of whether to
still unanswered and remains a matter of open or not to open a colostomy in a specific
controversy. patient, he or she should always try to imagine
In general, pediatric surgeons keep moving in what he would do if he was dealing with his own
the direction of doing more and more primary son or daughter.
procedures without a protective colostomy. We Most of the patients, who come to us after the
believe that it is good to move in that direction, to neonatal period, already have a colostomy, and
save our patients from the potential morbidity therefore we do not have to deal with this
associated with stomas that are still high [3, dilemma.
9–30]. In a full-term, newborn baby without severe
It is also very important to remember that in associated defects, we do not open a colostomy if
dealing with the treatment of anorectal malfor- the baby has one of the following malformations:
mations, a postoperative wound infection has perineal fistula, vestibular fistula, imperforate
consequences much more serious than in cases of anus with no fistula, and rectourethral bulbar fis-
other surgical conditions. A wound infection due tula. In the case of the last two malformations
to the repair of an anorectal malformation means (imperforate anus without fistula and rectoure-
not only that the patient will suffer the inconve- thral bulbar fistula), we expect to see the distal
niences and risks related with the infection itself, end of the rectum full of gas, located below the
but, in addition, the final functional prognosis coccyx in a cross-table lateral film. Based on our
(bowel and urinary control) may be jeopardized. experience, we can confidently operate primarily
We are against universal indications for a pro- on these types of cases without a colostomy with
cedure. In other words, we believe that a colos- good results. All other patients with anorectal
tomy is indicated under certain specific malformations, at our institution, receive a colos-
circumstances; a patient with a specific malfor- tomy, not only to protect the patient from the
54 5 Colostomy

operation to repair the malformation, but also for (d) It virtually eliminates the chances of hyper-
other reasons, including the fact that we need a chloremic acidosis from resorption of urine
stoma in order to do a high-pressure distal colos- [31, 32].
togram which we consider the most valuable (e) It does not interfere with the pull-through.
diagnostic test in patients with anorectal malfor- (f) It will not prolapse when done properly.
mations. Other sophisticated, state-of-the-art Transverse colostomies are not recommended
imaging modalities still cannot compete with the in anorectal malformations for several reasons:
accuracy of the anatomic information that we (a) It is impossible to irrigate the distal colon
obtain with a high-pressure distal colostogram. that remains full of meconium for the weeks
Most of the serious catastrophes we have seen or months after the colostomy is created.
occurred in cases that were surgically explored at (b) It has a tendency to provoke a severe megar-
other institutions without a high-pressure distal ectosigmoid, as a consequence of the pres-
colostogram [8]. In addition, higher anorectal ence of meconium that was never removed,
malformations have a higher incidence of serious plus the accumulation of mucus produced by
associated defects, mainly urologic, cardiac, and the entire defunctionalized colon and des-
gastrointestinal, which means higher-risk patients. quamation of mucosa cells.
In fact, the longer the period of time
between the colostomy opening and the final
5.5 Recommended Types repair, the greater the megarectosigmoid
of Colostomies (Fig. 5.8). This will translate eventually into
severe constipation, difficult to manage, after
5.5.1 Newborn Babies the repair of the malformation.
with Anorectal Malformations (c) Patients with recto-urinary fistulas not only
have a tendency to pass meconium from the
In newborn babies with anorectal malformations, colon into the urinary tract, but also they tend
in whom we consider that a colostomy is indi- to pass urine into the colon, which is
cated, we prefer to open a descending colostomy, absorbed, producing metabolic hyperchlore-
with widely separated stomas, located in the left mic acidosis [33, 34]. The long defunctional-
lower quadrant of the abdomen (Fig. 5.6) [1, 30]. ized segment allows this to occur.
In our series of 2,032 patients, only 75 of them (d) The incidence of urinary tract infection is
had a colostomy done at our institution. Over 200 higher than in cases with descending
cases underwent a primary repair without a colos- colostomies.
tomy; most of those suffered from perineal or ves- (e) The high-pressure distal colostogram (the
tibular fistula. All of the others came to our most valuable diagnostic study in anorectal
institution with a colostomy already open. As the malformations) is difficult to do, may not be
reader can imagine, that means that we have seen accurate, and is risky. It is not accurate,
almost all kinds of colostomies and have learned the because it is very difficult to exert enough
advantages and disadvantages of each type. That hydrostatic pressure, when the injection of
gave us an illuminating experience related to colos- contrast material is done from the transverse
tomies [30]. Based on that experience, we con- colon to fill up and to demonstrate the fistula
cluded that a descending colostomy with separated site, located all the way down to the rectum.
stomas is the best one, for the following reasons: In an attempt to demonstrate the location of
(a) It effectively diverts the entire fecal stream. the fistula, the colon may perforate. We have
(b) It significantly decreases the chances of uri- had two cases with such a complication. This
nary tract infection. incident has never happened in patients with
(c) It avoids the formation of megarectosigmoid descending colostomies.
because it allows the irrigation and cleaning of The opening of a loop colostomy in the trans-
the distal bowel and avoids distal fecal spillage. verse colon is perhaps the worst type of colostomy
5.8 Creation of a Colostomy 55

Fig. 5.9 Prolapsed transverse colostomy

5.7 Cecostomies

Opening of colostomies in the cecum, in general,


Fig. 5.8 Colostogram in a patient with transverse colos- has no indication in anorectal malformations. All
tomy, showing the characteristic narrow (non-used) distal of the problems mentioned when discussing right
colon, with a megarectosigmoid
transverse colostomies are more serious in cases
of cecostomies.
that we have seen, because in addition to all of the In the type of descending colostomy that we
problems that we have seen with transverse colos- recommend, the proximal stoma will not pro-
tomies, the patients pass stool into the distal bowel lapse due to the fact that it belongs to the fixed
which increases the chance of urinary tract infec- portion of the descending colon. On the other
tion and frequently produces fecal impaction in hand, the distal stoma belongs to the mobile
the distal stoma. Figure 5.8 shows the characteris- portion of the sigmoid, and therefore it has a
tic situation of a patient that had a bad loop trans- higher risk of prolapse. To avoid that, we specifi-
verse colostomy with fecal impaction in the distal cally recommend creating a very small (about
bowel. Those fecal impactions cannot be relieved 3 mm diameter) distal stoma (mucous fistula)
by washing the colon. It is sometimes necessary to (Fig. 5.6).
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 5.8 Creation of a Colostomy
tendency to prolapse (Fig. 5.9).
5.8.1 Surgical Technique

5.6 Left Transverse Colostomy In a newborn baby with anorectal malformation,


we try to perform a colostomy not before 24 h
The complications that we mentioned about right after the baby is born. In the chapter dedicated to
transverse colostomies are similar in a left trans- the management of a newborn with an anorectal
verse colostomy. malformation, we explain in detail the reasons
56 5 Colostomy

Fig. 5.10 Diagram showing an oblique preferred inci-


sion for a neonatal colostomy
Fig. 5.11 Trans-operatory picture, a dilated sigmoid
colon, full of meconium, is seen
why we want to wait 24 h. However, we do not
want to wait much more than that time, because The lower and medial end of the incision rep-
perforations of the bowel have been reported to resents the location of the mucous fistula site,
occur in patients after 24 h of life. It is true that which we create intentionally very small
occasionally, patients can live many days passing (Fig. 5.10) to avoid prolapse. Both stomas must
meconium through the recto-urinary fistula with- be separated enough so as to be able to use a
out perforation, but that is an exception. There stoma bag only on the proximal stoma, away
are reports of catastrophes that occurred in from the mucous fistula.
patients who suffered colon perforation and died The abdomen is opened, and it becomes very
because a colostomy was not done on time (see obvious that there is a big, dark loop of bowel
Chap. 4). The patient is taken to the operating (Fig. 5.11) that represents a very dilated sigmoid
room, and under general anesthesia, the abdomi- full of meconium. One should not try to manipu-
nal wall is washed, prepped, and draped in the late this very tense, dilated sigmoid loop of
usual manner. The incision that we recommend is bowel, because this may result in injuries to the
an oblique one, running from the left flank down seromuscular layer. Also, one should not try to
to the left lower quadrant of the abdomen create the stoma in the most dilated part of this
(Fig. 5.10). The upper part of the incision is colon, because that would make a huge stoma
located at the same site where we expect to create difficult to manage and more prone to suffer pro-
the proximal stoma. This point is located at equal lapse. We rather should look for the less-dilated
distance between the umbilicus, the ribs, and the descending colon, normally fixed to the left retro-
anterior superior iliac crest. We intentionally peritoneum. The descending colon detaches from
want the proximal, functional stoma to be sur- the left parieto-colic space and becomes mobile
rounded by normal skin and to be located as far (Fig. 5.12). At that particular point, we select a
away as possible from a prominence, irregularity, portion of the bowel, long and mobile enough, to
or structure that may interfere with the placement comfortably reach the anterior abdominal wall.
of a stoma bag. These structures are the umbili- Before we divide the bowel at the selected loca-
cus, the rib cage, and the iliac bone. When the tion, we specifically suggest putting a purse-
stomas are open too close to one of these struc- string suture with a 5-0 suture. In the center of the
tures, the stoma therapist, the nurses, and the purse-string suture, we make an opening in the
mothers struggle trying to place a stoma bag that bowel wall and introduce a 12 Foley catheter into
lays flat, avoiding leakage of stool and resultant the lumen of the very dilated colon with meco-
skin irritation. nium. The purse-string suture is tied to avoid
5.8 Creation of a Colostomy 57

Fig. 5.14 A catheter is introduced through the center of


the purse string to irrigate and remove all the meconium.
Fig. 5.12 The proximal stoma must be created using the The bowel collapses and is well perfused
first mobile portion of the descending colon

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
Fig. 5.13 A purse-string suture is placed on the anterior
wall of the selected loop of the colon, where the stoma
lower and medial end of the incision. The last 2
will be located 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
leakage of meconium in the operative field is necessary to do irrigations of the distal bowel if
(Fig. 5.13). The dilated colon is then irrigated indicated and also to allow access to the distal
with warm saline solution for a period of about stoma to perform a high-pressure distal colosto-
10–15 min until the entire sigmoid is completely gram. For this, we do not need a large stoma that
decompressed and free of meconium. This may bleed and interfere with the quality of life of
maneuver is extremely valuable for several rea- the patient. The tiny lumen also helps to avoid
sons. First, because it will allow the surgeon to prolapse.
manipulate a collapsed, well-perfused bowel The proximal stoma must be meticulously
(Fig. 5.14) and to perform a neat operation pre- constructed. The bowel is sutured to the fascia
serving the bowel integrity. In addition, the and peritoneum, being sure not to produce a stric-
patient will have a clean, collapsed colon for the ture and/or ischemia and being sure that it is per-
rest of the weeks or months before the pull- fectly open and patent. There are no concerns
through or main repair is done. We have evidence about prolapse because this stoma is placed at the
to believe that this helps to avoid the formation of 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 59

Fig. 5.19 Creation of a window in the vaginal septum of


a case with bilateral hydrocolpos
Fig. 5.17 Both stomas are meticulously “matured.” The
operation is finished
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
Fig. 5.18 Colostomy aspect weeks after operation 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
meticulous technique, delicate care of the tissues, vagina does not necessarily drain the other one.
and irrigation of every layer of the wound closure. Therefore, the recommendation in cases of two
The final result is cosmetically adequate, and the hemivaginas is to be ready to open one of the
stomas are easy to manage by the mothers. Our dilated vaginas and create a window in the vagi-
incidence of prolapse in this kind of colostomy is nal septum to be sure that both large hemivaginas
zero (Fig. 5.18). (hydrocolpos) are drained through a simple stoma
When we open a technically correct type of or with a single catheter (Fig. 5.19).
colostomy and place a stoma bag, usually there is When the hydrocolpos is not large enough to
no need to change the bag for the following 3 reach the anterior abdominal wall, then it must be
days. The first removal of the bag must be done drained with a catheter. We like to use a Pezzer or a
very gently. When it is difficult to apply a bag, or pigtail catheter that is exteriorized through the
there are frequent episodes of leakage of stool, abdominal wall (Fig. 5.20). The pigtail is helpful
this probably means that the colostomy was not because over the next several weeks, all the swell-
done correctly. The mucous fistula must be pro- ing of the vagina decreases, the vagina then tends to
tected from contact with the diaper with a little move away from the abdominal wall, and the cath-
piece of Vaseline gauze. eters frequently come out. The “pigtail” catheter
60 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


Fig. 5.20 Catheter drainage of a newborn with Sometimes the surgeons feel that the patient does
hydrocolpos
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,
Fig. 5.21 Subumbilical midline incision used in cases of 3-mm-diameter stoma, mucous fistula attached
large bilateral hydrocolpos. The stomas are separated, and next to the proximal stoma (Fig. 5.22). This is a
there is no incision in between them kind of a loop colostomy but with a reduced size
mucous fistula lumen, which reduces the chances
will remain in place. In order to drain the hydrocol- of stool spillage into the distal part.
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 5.11 Colostomy Care
and drainage of a hydrocolpos). In these cases, we
recommend a midline subumbilical incision The postoperative care of the colostomy is easy
(Fig. 5.21). This incision provides an excellent when the colostomy was made technically cor-
exposure to the pelvic anatomy and allows the sur- rect. Colostomies done in a technically incorrect
geon to perform the diversion of the hydrocolpos. manner represent a challenge and a nightmare for
5.12 Colostomy Closure 61

a b

Fig. 5.22 Stoma with a very small mucous fistula, placed together. (a) Diagram. (b) Photograph

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
Fig. 5.23 Defective colostomy. Unable to adapt a stoma normal size, after the colostomy is closed.
bag In cases of extremely severe, grotesque size dis-
crepancy between both ends (proximal and distal)
of the colostomy, we recommend a couple of good
stoma therapists, nurses, and mothers. It is almost technical maneuvers that proved to be very useful:
impossible to adapt a stoma bag in a case of A. End-to-side anastomosis
stoma that has irregularities in the surrounding B. Lateral window diversion
skin (Fig. 5.23). When the stoma is surrounded (a) End-to-side anastomosis
by normal skin, we usually use benzoin to cover In general, we recognize and recom-
the skin that will be in contact with the appliance. mend an end-to-end anastomosis as an
The orifice in the stoma bag is tailored according ideal way to close a colostomy. However,
to the size of the patient’s stoma. All this is done in cases of severe size discrepancy, an
by us in the operating room. We believe the sur- end-to-side anastomosis has demon-
geon should pay a lot of attention to the feedback strated to be equally useful and safer
provided by mothers, nurses, and stoma thera- (Fig. 5.24).
pists, concerning the quality of stomas. It is our (b) Lateral window diversion
impression that surgeons do not pay enough In cases of extreme size discrepancy
attention to these details. (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 Fig. 5.25 Colostomy closure. Packing of the proximal
window can also be used as a communi- stoma
cation to inject contrast material and
evaluate the function of the anastomosis
and growth of the distal bowel. 5.13 Surgical Technique
The preoperative preparation for colostomy
closure includes only irrigation of the proximal The patient is taken to the operating room. Under
stoma with saline solution. The distal stoma does general anesthesia, the abdominal wall is
not have to be irrigated because that has been washed, prepped, and draped in the usual fash-
clean from the time of the main procedure. In ion. A packing gauze impregnated with Betadine
cases of loop stomas, both ends need to be irri- is placed in the proximal stoma (Fig. 5.25).
gated. Prior to the definitive colorectal reconstruc- Multiple 5-0 silk sutures are placed at the muco-
tion, only the distal stoma needs to be irrigated. cutaneous junction, including both stomas
The patients are admitted to the hospital the (Fig. 5.26). These multiple silk sutures are used
day before surgery and receive a normal break- to apply uniform traction to both stomas to facil-
fast followed by clear fluids until midnight. The itate the dissection. Usually, when the stomas are
proximal stoma is irrigated as many times as nec- not too distant from one another, we use a wedge
essary until the nurses believe that the fluid that resection of both stomas with a piece of skin in
comes back is clear. We do not give GoLYTELY®1 between (Fig. 5.27). A needle-tip cautery is used
to these patients because we do not need or expect to perform this elliptical incision. The incision is
them to have a completely clean colon. done while applying uniform traction to both
stomas, and it goes through the skin, subcutane-
1
ous tissue, aponeurosis muscle, and peritoneum,
GoLYTELY® PEG 3350 236 g, sodium sulfate 22.74 g,
staying in our dissection, as close as possible to
sodium bicarbonate 6.74 g, sodium chloride 5.86 g, and
potassium chloride 2.97 g (4,000 mL) [regular and pine- the bowel wall, but without touching the bowel
apple flavor]. wall itself (Fig. 5.28).
5.13 Surgical Technique 63

Once both stomas have been completely sepa- Allen clamps to resect the part of the bowel that
rated from the abdominal wall, the packing gauze used to be attached to the abdominal wall
is removed from the proximal one. We use Baby (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.
Fig. 5.26 Multiple silk sutures are placed at the mucocu- The wound is finally covered with flexible collo-
taneous junction of both stomas. Traction is applied dion (Fig. 5.31).

a b

Fig. 5.27 Wedge incision. (a) Diagram. (b, c) Intraoperative pictures


64 5 Colostomy

At the beginning of the operation, the patient Occasionally, the patient has abdominal disten-
receives intravenous metronidazole and a broad- tion or vomits after the surgery; under those cir-
spectrum antibiotic. These medications will be cumstances, we may insert a nasogastric tube and
administered for 48 h postoperatively. At the end keep the patient fasting until the ileus resolves.
of the operation, we do not insert a nasogastric Colostomy closures must be done using a deli-
tube in the majority of our patients, but we keep cate and meticulous technique. This is an opera-
them fasting. The following day after surgery, if tion with serious potential complications [31, 32,
the patient had no nausea or vomiting and the 33–37].
abdomen is not distended, we start oral feedings. We are very proud of our results in colostomy
The patient usually stays in the hospital 3–4 days. 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.
Fig. 5.28 Both stomas are meticulously dissected and We put special emphasis in a meticulous hemosta-
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
5.14 Errors and Complications in Colostomies 65

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 (Fig. 5.32) leaving a very short piece of bowel
wall, leaving no dead spaces and irrigating each between the distal stoma (mucous fistula) and the
plane. We never had a case of a wound infection, end of the rectum (blind end or fistula site). This
despite all wounds being closed primarily. 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
5.14 Errors and Complications distal colostogram is so important. The first piece
in Colostomies of information that the surgeon must obtain from
this study is related to the length of bowel avail-
Over 1,500 patients came to us with a colostomy able for pull-through, distal to the mucous fistula
created at another institution. As can be imagined, (Fig. 5.33). A colostomy located too distal must
we have seen literally all kinds of colostomies. be ruled out before embarking in a misadventur-
From that experience, we learned about the poten- ous, failed attempted repair. The surgical alterna-
tial advantages and disadvantages, as well as tives when confronted with that problem are:
complications of each type [30]. The most com- A. Colostomy revision
mon error seen by us in patients with anorectal Closing the colostomy and reopening a
malformations, who underwent a colostomy more proximal one, exteriorized through the
opening at another institution, consists in having same abdominal orifice, and doing the main
the stoma created too distal into the sigmoid colon 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 site bowel, leaving the patient without a
bowel (mucous fistula) from the abdominal colostomy or opening a new one more
wall, to allow its mobilization and leaving proximal.
the distal bowel closed as a Hartmann pouch Alternative A: Colostomy revision is probably
(Fig. 5.35). the safest one, although it represents an extra
C. Close the colostomy and perform the pull- operation for the patient.
through at the same time. Alternative B: Repair the malformation, leaving
• Leaving the patient without a protective the patient with a Hartmann pouch (Fig. 5.35),
colostomy or may represent a future technical challenge,
• Opening a new more proximal stoma depending on how low the pouch is located. It
D. Resect the short piece of bowel located is a technically demanding operation to close
between the mucous fistula and blind end of a colostomy performing a bowel anastomosis
the fistula, pulling through the colostomy behind the bladder. In fact, if the upper end of
5.14 Errors and Complications in Colostomies 67

Fig. 5.32 Colostomy created too distal

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. 5.33 Distal colostogram showing a very short piece


of bowel distal to the stoma

the Hartmann pouch is located behind the pos- Fig. 5.35 Diagram showing a pull-through of a short dis-
tal colon. The mucous fistula had to be separated from the
terior urethra, the operation may be almost
abdominal wall. The distal stoma is closed (Hartmann
impossible to perform, not to mention the risk pouch). Alternatively, sometimes the distal stoma can be
involved. created in a lower part of the abdomen
68 5 Colostomy

Alternative C: Closing the colostomy and doing In patients with typical Hirschsprung’s dis-
the pull-through, leaving the patient without a ease, we resect the aganglionic rectosigmoid and
colostomy, is feasible but involves a certain anastomose the descending normoganglionic
degree of risk. We advise in such a case to colon to the anal canal, and patients have bowel
leave the patient fasting for 10 days, receiving control, provided the anal canal is preserved
parenteral nutrition. In addition, to do this intact. Patients with anorectal malformations are
kind of operation requires a total bowel born without an anal canal, and their sphincter
preoperative preparation. mechanism is represented by a spectrum that
Opening a new, more proximal colostomy includes cases with almost normal sphincter (in
is of course safer but requires one more major one extreme of the spectrum) to patients with
operation (colostomy closure). absent sphincter (in the other extreme of the
Alternative D: (Resect the little, distal piece of spectrum). Many patients operated from an ano-
bowel) We consider this alternative formally rectal malformation behave as if they were fecally
contraindicated. The most distal piece of continent; yet, they cannot tolerate sudden
bowel represents the future rectum for the changes in the consistency of the stool or sudden
patient. We have learned that the preservation peristaltic waves. For this reason, we insist that it
of this part of the intestine is extremely impor- is extremely important to try to preserve to the
tant. Our observations in multiple patients best of our capacity as much bowel as possible.
lead us to believe that the colon’s motility is We know that the rectosigmoid in patients
slower in its most distal part. In fact, the nor- with anorectal malformations suffers from hypo-
mal rectosigmoid acts mainly as a reservoir of motility, which is reflected in a marked tendency
stool, except at the time of defecation, when to constipation. Resection of the rectum may
the rectosigmoid has a very active, massive decrease the severity of the constipation problem,
peristaltic wave that allows the emptying of its but may also provoke tendency to diarrhea,
entire contents that usually represents the which, as we mentioned, will turn into inconti-
stool formed over a period of 12–48 h. In nent a patient with borderline bowel control.
between episodes of defecation, the rectum We have a large experience with patients that
remains virtually paralyzed (acting as a reser- have come to our clinic to receive bowel manage-
voir), receiving and storing stool. This is an ment for the treatment of fecal incontinence. In
extremely important function that allows us, some of them, the surgeons found it easier to sim-
human beings, to function socially, without ply remove the distal short rectum and pull the
using the toilet constantly. The observation of colostomy down. Those patients always become
the way the different types of colostomies incontinent even in cases born with a good func-
pass stool represents a clear demonstration of tional prognosis type of defect.
this. The more distal the colostomy, the longer In addition, as mentioned in the chapter of
the periods without passing stool. bowel management, that group of patients
Elimination of the rectum from the fecal (hypermotility, tendency to diarrhea) is much
stream results in an almost constant passing of more difficult to manage.
stool. This may be managed relatively well by an
otherwise normal individual in whom the anal
canal and sphincter mechanism are intact. Yet, in 5.15 The Case of Upper
patients with anorectal malformations, this is not Sigmoidostomy
tolerated at all and may well represent the differ-
ence between bowel control and fecal inconti- An interesting error occurs when the surgeon
nence. In other words, it is necessary to have an tries to open a transverse colostomy (either right
intact anal canal (sensation and sphincter mecha- or left sided) and actually creates what we have
nism) in order to maintain bowel control with an called an “upper sigmoidostomy” (Fig. 5.36).
absent rectosigmoid. 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- quadrant stoma does not necessarily mean that
sion and grabs the first visible segment of the the portion of the colon employed is the right
colon, erroneously assuming that it is either a transverse. Only with a distal colostogram one
right or left transverse colon. One must always can objectively determine the characteristics of
keep in mind that in cases of anorectal malforma- the colostomy.
tions, the sigmoid colon is very dilated and Complications in colostomies are divided into
redundant, reaching the upper abdomen. The sur- immediate and late. Immediate complications
geon must take the time to observe carefully the include dehiscence of the stoma, retraction, and
characteristics of the piece of colon that he infection. These three complications usually
selected, to be sure that that is the correct portion occur together. This represents a catastrophe
of the colon. usually related to a poor technique, a colostomy
The negative implications and inconveniences opened in a very sick patient, or both. A tense
of this type of colostomy (upper sigmoidostomy) anastomosis between the bowel and the
are obvious. The attached (tethered) sigmoid to abdominal wall, plus a devascularization of the
the abdominal wall will interfere with the bowel, may explain the retraction and dehis-
pull-through. cence. A poor surgical technique with severe
Again, we cannot overemphasize the impor- contamination may explain the infection [30].
tance of the distal colostogram in the planning of
the main repair of an anorectal malformation. Late complications include
The location of the stoma in the abdominal wall Parastomal hernia: This is also a technical prob-
does not necessarily correspond to the portion of lem that is avoidable by using a meticulous surgi-
the colon employed. In other words, a right upper 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 5 Colostomy

Fig. 5.43 A “sausage-


like” mass is easily
palpable, which represents
the reduced prolapse

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
Fig. 5.44 Incision made on the area of the palpable skin, and that is why we also emphasize the use
“sausage-like” mass and away from the stoma. The abdo-
men is entered and the prolapsed bowel is easily identi-
of midline incisions in patients with potential
fied. While closing the peritoneum and fascia, the stitches colorectal problems.
take the bowel wall 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
see the dilated colon that used to be prolapsed only the proximal stoma. They have to include
and now is full of the packing gauze (sausage) both stomas into the same bag, which means
(Fig. 5.44). We start by closing the peritoneum potential passage of stool into the distal bowel that
and posterior fascia, including in our stitches a may provoke urinary tract infections and/or fecal
bite of the colonic wall (“sausage”) that used to impaction distally. Stomas that are located too far
be prolapsed. We finish by closing that incision one from the other (Fig. 5.45) represent a problem
and removing the packing gauze. The bowel will because at the time of colostomy closure, the
not prolapse again. We have seen one case of patient will need a very long incision in order to
recurrence, but we have done at least 25 of these 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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Imaging
6

6.1 Introduction 6.3 Neonatal Imaging

Advances in imaging technology have been The first 24 h of life, before making the decision
extremely beneficial for the management of to open a colostomy or to perform a primary
patients with anorectal malformations. Every repair, represents a window of opportunity to
day, we learn about technologic innovations that diagnose potential associated defects. The chest
frequently surpass our imagination. Concurrently film taken during the first 24 h of life also allows
with these, the surgeons have to speculate less us to see the integrity of the thoracic vertebra and
and less, like in the past [1], and have the privi- ribs. In addition, it helps us in the diagnosis of
lege of making very precise anatomic diagnosis esophageal atresia and potential cardiac malfor-
that allows the planning of a complex reconstruc- mations (Fig. 6.1).
tion in a very accurate manner. The abdominal x-ray film allows us to see
In dealing with the spectrum of anorectal mal- and rule out the possibility of hemivertebra
formations, we depend very much on the images (Fig. 6.2). The early detection of these types of
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.

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 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
Fig. 6.2 Abdominal film showing a hemivertebrae suffer from different degrees and types of sacral
abnormalities. The sacral abnormalities present
like a spectrum with ratios similar to normal chil-
malformations is relevant to establish the func- dren, in what we call the good side of the spec-
tional prognosis in these babies. The abdomi- trum. However, in the “bad” extreme of the
nal film must include an AP view of the sacrum spectrum, we see patients with a sacral ratio of 0.
(Fig. 6.3). It is also important to take a lateral We found that it is extremely unusual for a
abdominal film that allows a more accurate mea- patient with anorectal malformation and with a
surement of the sacral ratio (Figs. 6.3 and 6.4). ratio lower than 0.4 to have bowel control. To have
Traditionally, the number of sacral vertebrae is a normal sacral ratio is a good prognostic sign, but
counted to evaluate the quality of the sacrum. Most it does not mean that the patient will necessary
pediatric surgeons agree that when a patient has have bowel control, since there are other factors
less than three sacral vertebrae, the prognosis for that influence the final functional results.
bowel and urinary control is not good. Many others One of the relatively common associated
while discussing the subject of the sacrum use defects in children with anorectal malformations
rather nondescriptive terms such as “dysplastic” or are defects of the radial bone (Fig. 6.5).
“hypoplastic.” There is no question that the pres- Sometimes, the defect in the forearms and the
ence of the sacrum as well as its integrity is crucial hands is very obvious (Fig. 6.5a). Other times,
to determine the functional prognosis of the patient. one can see only a slight radial deviation of the
We found the terms “dysplastic” or “hypo- hand that would make us suspect this defect.
plastic” very inaccurate. In addition, we found An ultrasound study is also part of the group of
that there are cases with five sacral vertebrae, yet imaging studies that must be done during the first
very abnormal ones, which results in an extremely 24 h of life before the baby suffers from abdomi-
short sacrum associated to fecal and sometimes nal distention. The kidney ultrasound is perhaps
urinary incontinence. We thought that it was nec- the most important part of the evaluation of this
essary to create a more objective way to evaluate 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 6 Imaging

some sort of urologic-associated condition. We defects is absent or multicystic kidney (Fig. 6.7).
specifically look for hydronephrosis (Fig. 6.6). The ultrasound must include the rest of the abdo-
One of the most common urologic anatomic 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
Fig. 6.4 Lateral film of sacrum tethered cord can only be done reliably with an

a b

Fig. 6.5 Absent radial bone – a frequently associated defect. (a) External appearance. (b) Radiologic appearance
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- department might have negative consequences;
eral anesthesia in babies (Fig. 6.9c). sometimes they fall into urinary retention as a con-
Some surgeons routinely perform a voiding cys- sequence of injuries provoked by a failed attempt
tourethrogram in male babies with anorectal mal- to pass a catheter. When the baby has hydronephro-
formations. We do not believe this routine is sis and megaureter, that is when we consider an
necessary. When the baby has normal kidneys by indication for a voiding cystourethrogram. Other
ultrasound, no evidence of megaureters, and is surgeons believe that the voiding cystourethrogram
passing urine normally, we do not see the relevance would allow them to determine the size and loca-
of the voiding cystourethrogram. Over 80 % of the tion of the rectourethral fistula. We considered a
male patients with anorectal malformations have a voiding cystourethrogram a non-reliable study for
connection between the rectum and the urinary the diagnosis of the fistula location. Most of the
tract (fistula), and at the location of the fistula, time that study does not show the fistula.
sometimes there is a kink of the urethra that inter- Occasionally, one can see a kink of the urethra that
feres with the passing of a catheter. Rough manipu- “suggests” where the fistula is located but certainly
lations of this baby’s urethra in the radiology 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 wide spectrum that goes from almost normal
Location Prior striated sphincter mechanism to almost absent
to the Colostomy sphincters.
The sphincter mechanism in normal individu-
6.4.1 Anatomic Facts and Timing als is represented by a funnel-like voluntary mus-
cle structure, the upper limits of that funnel being
To understand the rationality of the imaging during the pubococcygeal line (Fig. 6.11). That funnel-
the neonatal period to determine the location of the like muscle mechanism is a continuum of a stri-
rectum and the fistula, it is extremely important for ated muscle that runs all the way down to the skin
the clinician and the radiologist to understand the of the perineum. The upper part of the funnel-like
anatomy of the pelvis of babies with anorectal mal- mechanism inserts in the pubic bone and sur-
formations, this is illustrated in Animation 6.1. rounds the rectum. The contraction of those fibers
The sphincter mechanism in babies with compresses the rectum from behind. During sur-
anorectal malformations is represented by a 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


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
Fig. 6.10 VCUG showing a kink in the urethra suggest- (prostatic fistula) or into the lowest portion of the
ing the fistula location posterior urethra (rectourethral bulbar fistula)
84 6 Imaging

a b

Fig. 6.11 Funnel-like normal sphincter mechanism. (a) Relaxed. (b) Contracted

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
Fig. 6.12 Diagram of a perineal fistula. Most of the rec-
abdominal x-ray film during the first few hours
tum is surrounded by the funnel-like sphincter of life, we will never find the gas of the rectum
mechanism located below the pubococcygeal line and cer-
tainly that does not mean that the baby has a
“very high malformation” since most likely
(Fig. 6.13). In cases of recto-bladder neck fistula (90 % chance), the rectum is located below the
which represents the highest of all defects in pubococcygeal line but is compressed by the
male patients, the rectum opens in the bladder sphincter mechanism. Interestingly, in our lit-
neck and is not surrounded by this sphincter erature review, we only found one author [4]
6.4 Determination of the Fistula Location Prior to the Colostomy 85

a b

Fig. 6.13 Diagram showing a rectourethral fistula. (a) Prostatic. (b) Bulbar

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
Fig. 6.14 Diagram showing recto-bladder neck fistula. distention represents, as expected, an increase of
The bowel is not surrounded by sphincter muscle the intraluminal pressure of the bowel, and at
some point, that pressure overcomes the muscle
tone of the funnel mechanism that surrounds the
who suggested that the contraction of the rectum, and then one can see the real location of
“puborectalis muscle” must be taken into con- the gas inside the rectum (Fig. 6.15).
sideration to interpret radiologic studies in the We have been exposed to patients that are
newborn. referred to us after failed attempted repairs. Some
86 6 Imaging

without the necessary pressure to overcome the


a
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
b
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
Fig. 6.15 Cross-table lateral film in a newborn baby when the study is performed too early in life, it is
with imperforate anus. (a) Six hours old. (b) Twenty-four
hours old not reliable.
Later on, we learned about the “inherent errors
and disadvantages of the invertogram” [6, 7]. In
of those babies were subjected to diagnostic imag- addition, we have learned that one can obtain
ing studies during the first few hours of life that exactly the same image of the invertogram by
led the surgeons to erroneously conclude that the placing the patient in prone position with the pel-
baby had a “high imperforate anus.” As a conse- vis elevated (Fig. 6.15) [8].
quence, the surgeons made one of two decisions, We were able to compare the two images
either to open a colostomy (that was not indicated) obtained with the invertogram and with this
or even worse, to perform an abdominoperineal cross-table lateral film and found that it is exactly
procedure in a baby that had, for instance, an the same.
unnoticed perineal fistula. Some of those patients The cross-table lateral film has the great
that had a non-indicated colostomy subsequently advantage of avoiding the positioning of the baby
have received a distal colostogram without enough upside-down with the risk of vomiting and
hydrostatic pressure, which induced the surgeons aspiration.
“to confirm” the diagnosis of “high imperforate Furthermore, we have learned that with a good
anus.” Again, the distal colostogram was done 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


Fig. 6.17 Distal colostogram showing a very short piece
have a colostomy. The baby is taken to the radiol- of bowel distal to the colostomy pull-through is not
ogy suite and a no. 8 Foley catheter is introduced feasible
through the mucous fistula of the colostomy.
Many babies, unfortunately, are subjected to a
loop type of colostomy, and this makes it diffi- has available for the pull-through, from the colos-
cult, sometimes for the radiologist, to find in tomy site to the most distal end or fistula site.
which direction the catheter should be introduced This is extremely important, since the most com-
to be sure that the contrast material is injected in mon error that we have seen in the location of the
the distal bowel. The mother usually knows colostomies is a too-distal colostomy, meaning
which one is the orifice that produces stool and that the surgeon left a really short piece of bowel
the one that is just a mucous fistula. The catheter beyond the colostomy site, which will certainly
is inserted about 5 cm, and the balloon is inflated interfere with the pull-through. Trying to repair
with 2 mL of air or water. Traction is applied on an anorectal malformation without this crucial
the catheter to be sure that the balloon impacts on information frequently ends in a disaster.
the stoma serving as a plug that will prevent the Figure 6.17 shows a very short piece of bowel
contrast from leaking out, even after we apply distal to a colostomy. Figure 6.18 shows a colos-
hydrostatic pressure during the injection. We tomy done in the descending colon leaving a loop
only use water-soluble contrast material. The of the sigmoid colon long enough to perform a
injection of the contrast is done using a 60 mL comfortable pull-through. Once we obtained that
syringe with a catheter tip connected to the Foley information, the patient is then turned into metic-
catheter and is done by hand. Under fluoroscopic ulous lateral position. A lead marker is placed in
control, with the baby in supine position, the the anal dimple. During the fluoroscopic study in
injection starts and continues until the contrast lateral position, the images must include the
seems to reach the most distal part of the bowel. sacrum, the coccyx, the lead marker of the anal
At this point, we obtain very valuable informa- dimple, and the entire lower pelvis. The radiolo-
tion related with the length of bowel that the baby gist must know that we are planning on a potential
6.7 Technique 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
Fig. 6.18 Distal colostogram showing a redundant piece the contrast goes rather toward the bladder. The
of bowel distal to the colostomy pull-through is feasible 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
posterior sagittal approach, and therefore, we become then evident. The injection continues
want to know the position of the rectum and fis- until the baby is forced to void. During the void-
tula as related to the coccyx and the perineal skin ing, we have the best possible images of the anat-
(anal dimple). A common error from a radiologist omy of the rectum and the urinary tract
is to show only the fistula location without the (Fig. 6.20). Babies with suspected urinary tract
other points already mentioned that would orient problems, such as hydronephrosis and evidence
the surgeon in terms of the location of the fistula. of reflux, must receive prophylactic antibiotics
The injection continues, applying hydrostatic prior to this study because we must keep in mind
pressure on the syringe and pulling on the Foley that we are pushing the contrast passing through
catheter to avoid leakage of the contrast a contaminated distal rectum.
(Animation 6.2). The contrast material runs The high-pressure distal colostogram, as most
through the distal bowel and stops in a horizontal studies, has some potential risks. In over 1,000
line that corresponds to the pubococcygeal line male patients we have operated, we have evi-
(Fig. 6.19). The fact that this line is horizontal dence of two perforations of the bowel that
and the fact that it is located in the pubococcygeal occurred during the injection of contrast material.
level indicate that it is not the end of the rectum. One of them had a transverse colostomy. We do
That image is given by the contraction of the not advocate the use of transverse colostomies in
“funnel-like” muscle mechanism. At that point, anorectal malformations, and one of the reasons
90 6 Imaging

a b

Fig. 6.19 Distal colostogram showing (a) the contrast “high malformation.” (b) Same study after applying more
material ending in a horizontal line at the level of the hydrostatic pressure
pubococcygeal line, giving the wrong impression of a

is because it is very difficult to generate enough very abnormal type of bowel with very abnormal
hydrostatic pressure through a transverse colos- blood supply, and we believe that could be a pre-
tomy to be able to achieve our goals in trying to disposing factor to explain the perforation,
determine the anatomy of the recto-urinary fis- although we do not have evidence of that.
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- 6.8 Most Common Errors
trast material. Since we were dealing with a
defunctionalized portion of the bowel, we thought Some patients come to us with a distal colosto-
that what all the baby would require would be gram that has been done in another institution.
intravenous fluids, antibiotics, and observation. We have seen many errors including passing the
However, to our surprise and alarm, that baby Foley catheter too far (Fig. 6.21). This may give
went into severe hypovolemic shock that required the false impression that the patient has a very
immediate resuscitation followed by a laparot- short piece of bowel distal to the colostomy. That
omy to clean the peritoneal cavity and close the is why we emphasized that the catheter should be
perforation of the colon. We attribute this severe introduced only 5 cm and then pulled back to
reaction to the fact that the contrast is very hyper- ensure that the balloon is impacted against the
osmolar. The second case was a patient that had a 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 93

Fig. 6.23 Distal colostogram in a patient that previously


had an attempted failed repair Fig. 6.24 Voiding cystogram showing a recurrent recto-
urethral fistula

the degree of constipation that the patient will


suffer later in life. distal rectum because the contrast material
Some patients come to us after a failed escapes through the fistula site and one cannot
attempted repair. They suffered from catastro- generate enough pressure to show the real size of
phes and misadventures during an attempted the rectum (Fig. 6.25). These may give a false
operation. They still have the colostomy, and we impression of a long narrow fistula when actually
had to do a distal colostogram without knowing the rectum is distended all the way down to reach
what we are going to find. We may find that the a point a few millimeters from the skin. A real
rectum is completely atretic (Fig. 6.23). We may long narrow fistula is an extremely unusual
find also that the patient has also persistence or condition.
recurrent rectourethral fistula (Fig. 6.24).

6.11 Distal Colostogram


6.10 Distal Colostogram in Cloacas
in Female Patients
In patients born with cloacas, the distal colosto-
In the past, we used distal colostogram also in gram is still a very valuable study, but this is true
female patients with vestibular fistulas and some- particularly if it is combined with a three-
times with perineal fistulas. We do not recom- dimensional rotational scan.
mend doing that kind of study because we Depending on the specific anatomy of the clo-
consider that it is not useful for the diagnosis and aca, the distal colostogram may show only the
treatment of the patient. The injection of contrast rectum followed by the common channel
material through the distal stoma in that kind of (Fig. 6.26) or may show also the one vagina or
patient would show an image similar to a long two vaginas (Fig. 6.27). It is extremely unusual to
narrow fistula, and even if we apply more hydro- be able to fill up the urethra and bladder through
static pressure, we never are able to distend the a distal colostogram. The study in cloacas can be
94 6 Imaging

Fig. 6.25 Distal colostogram in a patient with rectoves-


tibular fistula, giving a false impression of a long narrow Fig. 6.27 Distal colostogram in a patient with a cloaca
fistula 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,
Fig. 6.26 Distal colostogram in a patient with a cloaca and colostomies. We insert catheters in every
showing only the rectum single orifice that we can see in the pelvis, and
6.12 Monitoring Constipation 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
Fig. 6.28 Distal colostogram combined with injection from
not the number of bowel movements but the
below showing the rectum, urethra, bladder, and vagina
capacity to empty completely the rectosigmoid
during the bowel movement. In fact, some of
then the contrast material is injected sequentially the babies that have multiple bowel movements
through each one of those catheters, and the scan in a day are the most severely constipated, and
is done rotationally which gives extraordinary eventually those tiny bowel movements become
useful images (see videos 6.1, 6.2 and 6.3 show- episodes of soiling. After years of speculating
ing the rotational studies). This imaging repre- and guessing as to whether or not the baby was
sents the ultimate technology. Cloacas can be constipated, we learned that the only objective
repaired without the help of this study, but cer- way to know if the baby is emptying the colon
tainly this type of study allows us to be more pre- is radiologically.
cise in our preoperative diagnosis and to plan our After the colostomy is closed, we recom-
procedures in a more accurate way. 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-
6.12 Monitoring Constipation pects the child is constipated, we suggest taking
an abdominal x-ray film. Every patient needs a
The most common sequelae in terms of bowel different amount of laxative, and to determine
function in patients with anorectal malformation whether or not we are using the right amount,
is constipation. Constipation produces dilatation we used abdominal x-ray films. In other words,
of the rectosigmoid and sometimes the entire by trial and error, we prescribe a specific amount
colon. When constipation is not treated properly, of laxative and then, after a few days, we take an
it produces fecal impaction, and that interferes abdominal film to be sure that the amount of
with bowel control in those patients that are born laxative that we are using is the correct amount.
with potential for fecal continence. Those patients Many patients come to us after having an ano-
may behave like the child with severe idiopathic rectal malformation repair either by us or by
96 6 Imaging

a b

Fig. 6.29 Contrast enema done to evaluate colonic motility. (a) Hypomotility. (b) Hypermotility

others at other institutions, and they were not completely clean in the underwear for 24 h. To do
subjected to any kind of monitoring through this, we take an abdominal x-ray film every day
years. We suspect clinically that the patients are over a period of 1 week. In addition, also daily
constipated, and to evaluate the degree of con- we hear the clinical information from the parents
stipation that they suffer from, we order a and adjust the type of enema accordingly.
contrast enema with water-soluble material and
without colon preparation. The contrast material
must fill up the entire colon, and we request to 6.14 Monitoring the Urinary Tract
show us post-evacuation films which give us an
idea of the degree of hypomotility that the As previously mentioned, 50 % of the patients
patient suffers from (Fig. 6.30). 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-
6.13 Radiology During the Bowel lem is vesicoureteral reflux. Many times the
Management Program babies are born with significant kidney damage
that occurred in utero. All patients require a close
In Chap. 20, the reader may find a description of monitoring of the urinary tract, since we have
our bowel management program, created to help seen many patients who have a tendency to dete-
patients who suffer from fecal incontinence. riorate with time. Even under normal circum-
Basically, over a period of 1 week, we determine stances in babies with good anorectal
the enema that is capable of emptying at least all malformations, we always like to follow them
of the left side of the colon, to keep the patient through life. In a patient that has normal kidneys
6.14 Monitoring the Urinary Tract 97

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

cystograms, and urodynamic studies, as well as a terminalis is by an MRI study that is the most
frequent nephrologic monitoring. Patients with accurate way to determine whether or not the
neurologic deficits including abnormal sacrum, patient has tethered cord (Fig. 6.9c).
tethered cord, or complex cloacas are, by defini- Imaging plays a very important role in the
tion, urologic patients that require very close diagnosis, follow-up, and management of patients
follow-up monitoring of the urinary function. already operated (see Chap. 19).
Many of the complex cloacas eventually will
require urinary reconstructions including bladder
augmentations and Mitrofanoff. References
For patients that have been operated on at other
institutions and come to us, for instance, for bowel 1. Peña A (1987) Anatomical considerations relevant to
management due to fecal and/or urinary inconti- fecal continence. Semin Surg Oncol 3(3):141–145
nence, we evaluate the entire colorectal anatomy, 2. Peña A (1996) Anorectal malformations. Semin
Pediatr Surg 4(1):35–47
pelvic anatomy, and urinary tract. We specifically
3. Niedzielski J, Midel A (1998) Sacroiliac ratio in children:
order a magnetic resonance imaging (MRI) study natural evolution and clinical implications. Surg Childh
with a specific technique that includes the placing Int 6:78–80. doi:10.1016/S0022-3468(99)90600-0
of a big Foley catheter in the rectum (Fig. 6.30a 4. Berdon WE, Baker DH, Santulli TV, Amoury R
(1968) The radiologic evaluation of imperforate anus.
and b). This will allow us to see the location of the
An approach correlated with current surgical con-
rectum as related to the sphincter mechanism. In cepts. Radiology 90(3):466–471
addition, it is an excellent study to make a diagno- 5. Wangensteen OH, Rice CO (1930) Imperforate anus:
sis of a posterior urethral diverticulum which is a a method of determining the surgical approach. Ann
Surg 92(1):77–81
complication that we have seen in the past in
6. Berdon WE, Baker DH (1967) The inherent errors in
patients that were operated abdominoperineally, measurements of inverted films in patients with
having a rectourethral bulbar fistula; the surgeons imperforate anus. Ann Radiol (Paris) 10(3):235–240
found it difficult to reach the lower end of the 7. Narasimharao KL, Nair PM, Mitra SK, Pathak IC
(1984) Hypoxia during invertography. Indian Pediatr
bowel, amputated the bowel, and left a piece of
21(12):971–973
rectum attached to the urethra (Figs. 6.30c and d). 8. Narasimharao KL, Prasad GR, Katariya S, Yadav K,
That piece of rectum becomes a diverticulum that Mitra SK, Pathak IC (1983) Prone cross-table lateral
produces mucus, forms stones, and produces view: an alternative to the invertogram in imperforate
anus. AJR Am J Roentgenol 140(2):227–229
pseudourinary incontinence, and in one specific
9. Willital GH (1971) Advances in the diagnosis of anal
case, the patient developed an adenocarcinoma. and rectal atresia by ultrasonic-echo examination.
With the advent of laparoscopic procedures, we J Pediatr Surg 6(4):454–457
are seeing again this problem of posterior urethral 10. Schuster SR, Teele RL (1979) An analysis of ultra-
sound scanning as a guide in determination of “high” or
diverticulum, when the surgeons try to approach
“low” imperforate anus. J Pediatr Surg 14(6):798–800
laparoscopically a rectourethral bulbar fistula, 11. Oppenheimer DA, Carroll BA, Shochat SJ (1982)
finding difficult to reach the lower end of the rec- Sonography of imperforate anus. Radiology 148(1):
tum Animation 5 (Posterior urethral Diverticulum 127–128
12. Baunin C, Blancher A (1986) Radiologic examination
in a patient with a recto-urethral bulbar fistula
of anorectal malformations. Chir Pediatr 27(5):
approached laparoscopically). The voiding cysto- 239–245
urethrogram may or may not show the posterior 13. Donaldson JS, Black CT, Reynolds M, Sherman JO,
urethral diverticulum and therefore is not the ideal Shkolnik A (1989) Ultrasound of the distal pouch in
infants with imperforate anus. J Pediatr Surg 24(5):
method of diagnosis. The diagnosis of this entity
465–468
is confirmed cystoscopically, and the repair is 14. Tashev P, Chatalbashev N, Kazakov K (1991)
described in Chap. 22. Application of ultrasonography in the evaluation of
When the babies come to us for the first time imperforate anus. Folia Med (Plovdiv) 33(3):36–40
15. Wagner ML, Harberg FJ, Kumar AP, Singleton EB
with anorectal malformations and did not have
(1973) The evaluation of imperforate anus utilizing
spinal ultrasound during the first 3 months of life, percutaneous injection of water-soluble iodide con-
the only way to know the location of the conus trast material. Pediatr Radiol 1(1):34–40
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16. Motovic A, Kovalivker M, Man B, Krausz L (1979) 24. Cremin BJ, Cywes S, Louw JH (1972) A rational
The value of transperineal injection for the diagnosis radiological approach to the surgical correction of
of imperforate anus. Ann Surg 190(5):668–670 anorectal anomalies. Surgery 71(6):801–806
17. Kurlander GJ (1967) Roentgenology of imperforate 25. Lernau OZ, Jancu J, Nissan S (1978) Demonstration
anus. Am J Roentgenol Radium Ther Nucl Med 100(1): of rectourinary fistulas by pressure gastrografin
190–201 enema. J Pediatr Surg 13(6):497–498
18. Kohda E, Fujioka M, Ikawa H, Yokoyama J (1985) 26. Gross GW, Wolfson PJ, Pena A (1991) Augmented-
Congenital anorectal anomaly: CT evaluation. pressure colostogram in imperforate anus with fistula.
Radiology 157(2):349–352 Pediatr Radiol 21(8):560–562
19. Ikawa H, Yokoyama J, Sanbonmatsu T, Hagane K, 27. Wang C, Lin J, Lim K (1997) The use of augmented-
Endo M, Katsumata K, Kohda E (1985) The use of pressure colostography in imperforate anus. Pediatr
computerized tomography to evaluate anorectal Surg Int 12(5–6):383–385
anomalies. J Pediatr Surg 20(6):640–644 28. Niedzielski JK, Midel A (1998) Is augmented-pressure
20. Krasna H, Nosher JL, Amorosa J, Rosenfeld D (1988) distal colostography useful in the diagnostics of ano-
Localization of the blind rectal pouch in imperforate rectal malformations? Surg Childh Int VI(1):28–31
anus with the CT scanner. Pediatr Surg Int 3: 29. Soccorso G, Thyagarajan MS, Murthi GV, Sprigg A
114–119 (2008) Micturating cystography and “double urethral
21. Martuciello G, Taccone A, Fondelli P, Moran Penco catheter technique” to define the anatomy of anorectal
JM, Dodero P (1990) Tomografía Axial malformations. Pediatr Surg Int 24(2):241–243
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¿Una indicación pre y postoperatoria? Cir Pediatr Prochazka A (2013) The diagnostic value of
4(3):173–178 MRI fistulogram and MRI distal colostogram
22. Taccone A, Martucciello G, Dodero P, Delliacqua A, in patients with anorectal malformations. J
Marzoli A, Salomone G, Jasonni V (1992) New con- Pediatr Surg 48(8):1806–1809. doi:10.1016/j.
cepts in preoperative imaging of anorectal malforma- jpedsurg.2013.06.006
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Bowel Preparation in Pediatric
Colorectal Surgery 7

Colorectal operations are considered “contami- tion, following preoperative management, we


nated procedures” and represent, by definition, find no convincing reason to change. We under-
cases with a higher risk of infection. Therefore, stand that perhaps, we could have the same good
patients must be subjected to some forms of bowel results without bowel preparation; however, it is
preparation prior to the operation. In addition, the not easy to change a routine that resulted in zero
use of prophylactic antibiotics must be considered. infections. We also suspect that a meticulous sur-
Through the last 30 years, we have been fol- gical technique is the key to avoid complications,
lowing the evolution of the concepts related with rather than the bowel preparation or the antibiot-
the different modalities of bowel preparation, as ics. Interestingly, during the last 30 years, we
well as the use of different antibiotics by mouth, have changed the type of antibiotics used pre-
intravenously, and pre- and postoperatively. and postoperatively according to the recommen-
Most of the literature is related with adults. dations of the epidemiology departments of the
There are an enormous number of publications institutions in which we worked, yet the results
related to adult conditions. We selected only a were the same: no infections.
few papers representative of the tendency to According to Breckler et al. [6], the over-
avoid mechanical bowel preparation [1–4] and whelming majority of pediatric surgeons in the
antibiotics. Some surgeons found no evidence to United States still use mechanical bowel prepara-
support the use of mechanical bowel prepara- tion and preoperative intravenous antibiotics.
tions. Even more, some publications present data Leys et al. [7] found slightly higher number of
indicating that the bowel preparations seem to anastomotic leaks and infections in pediatric
result in a higher incidence of infections and patients who underwent a mechanical bowel prep-
dehiscence! The incidence of wound infections aration. Breckler et al. reported 14 % of wound
in the adult literature varies from 4 to 19 % with infections in colostomy closures and no differ-
minor variations in favor of those patients who ences between those who received antibiotics and
did not receive preoperative colonic preparation. those patients who did not [8]. Serrurier et al.,
The question for us, pediatric surgeons, is if also in pediatric cases [9], found significantly
we should change our routines, based on the higher number of wound infections (14 %) in a
information obtained from adult data. Our per- series of 272 colostomy closures, of patients who
sonal answer is no. The incidence of wound received mechanical bowel preparation, whereas
infections reported in adult patients with and only 5 % of those without preparation suffered
without bowel preparations is still much higher from this complication. Similar results were
than in our cases [5]. If we closed 649 consecu- reported by others [10, 11]. Pennington et al. [12]
tive colostomies without a single case of infec- in a retrospective analysis of the series of 42

A. Peña, A. Bischoff, Surgical Treatment of Colorectal Problems in Children, 101


DOI 10.1007/978-3-319-14989-9_7, © Springer International Publishing Switzerland 2015
102 7 Bowel Preparation in Pediatric Colorectal Surgery

American Children’s Hospitals, (5,473 patients) col electrolyte solution) to clean the entire gastro-
not surprising, found that only 22.9 % of all intestinal tract in pediatric patients [13–18].
patients received an “evidence-based prepara-
tion.” However, the “evidence-based guidelines”
that they allude to are related with adult patients. 7.1 Major Procedures
The question comes again: Should we change our
routines based on the adult literature? The ques- Major procedures include primary or secondary
tion is even more difficult to answer when our pull-throughs for anorectal malformations, for
results are excellent. In summary, we are not con- Hirschsprung’s disease, or for idiopathic consti-
vinced that following adult “evidence-based” rou- pation, performed without a protective colostomy.
tines will benefit our patients. Anoplasties and resection of rectal prolapse are
We are very proud of our excellent record of also included in this category. These patients must
zero wound infections in colostomy closures. We be admitted, most of the time, 24 h before the
close all wounds primarily and leave no drains. operation. Severely constipated or fecally
The patients received irrigations of the proximal impacted patients, sometimes, are admitted 2 days
stoma, with saline solution and intravenous anti- before surgery to be subjected to our protocol of
biotics during anesthesia and 48 h postoperatively. disimpaction before the cleaning of the gastroin-
On the other hand, we had cases of infections or testinal tract (See Chap. 25, Sect. 25.7.1).
minor dehiscent anoplasties in cases subjected to The morning of admission the patient is only
primary pull-throughs without a colostomy. Those allowed to take clear fluids by mouth. Usually
patients received GoLYTELY® (total bowel prep- around noon time, the administration of
aration). The dehiscences that we have seen GoLYTELY®2 is started, at the rate of 25 mL/
occurred usually between the 5 and 8 days post- kg/h until clear. Most of the time, this is adminis-
operatively while the patients are still fasting. We tered through a nasogastric tube. Occasionally,
take those patients to the operating room, resu- some adolescents volunteer to drink GoLYTELY®
tured the dehiscent areas, and prolong the period at the right speed, in order to try to avoid a naso-
of fasting for 2–3 more days. A complete dehis- gastric tube. Many of them try, yet, usually the
cence will receive a colostomy, which is a very attempt is unsuccessful because they do not drink
unusual event. A question to be answered is if a fast enough or they have nausea, and therefore,
dehiscence, occurring in a case with bowel prepa- they need the nasogastric tube. It usually takes
ration, has less serious consequences than in cases about 4 h for the colon to be completely cleaned.
without bowel preparation. We believe it does. Some patients suffer from nausea and vomiting
The type of colonic preparation that we recom- during the administration of GoLYTELY®. When
mend varies, depending on the type of surgical that happens, the patient benefits from the admin-
procedure that the patient is going to receive, as istration of an antiemetic medication, but we con-
well as the specific circumstances of the patient. tinue the administration of the GoLYTELY®, at a
Thus, the bowel preparation required for a slightly slower rate, because we have seen that
newborn baby that is going to be subjected to a most of the GoLYTELY® still goes through the
primary, major, colorectal procedure without a pylorus and through the bowel. During the
colostomy is different than the one required for administration of GoLYTELY®, the patient usu-
an older patient. ally suffers from a certain degree of abdominal
There is plenty of evidence related with the distention and cramps. Soon enough, they start
safety of using GoLYTELY®1 (polyethylene gly- passing stool through the rectum. Later on, this
becomes liquid stool that becomes more and
1
more clear, until it is free of stool. Patients with
GoLYTELY® PEG 3350 236 g, sodium sulfate 22.74 g,
Hirschsprung’s disease frequently require rectal
sodium bicarbonate 6.74 g, sodium chloride 5.86 g, and
potassium chloride 2.97 g (4,000 mL) [regular and pine-
2
apple flavor]. See footnote 1.
7.2 Primary Procedures for the Treatment of Anorectal Malformation During the Newborn Period 103

irrigation to help relieve this distention during the paction consists of the administration of three
bowel prep. After 4 h of the administration of enemas per day and daily radiologic monitoring,
GoLYTELY®, the nurse or the resident must see to be sure that the colon is free of fecal impaction
what is coming out through the rectum of the (see chapter on Idiopathic constipation). When
patient. A yellow or greenish color represents this is achieved, the patient can be admitted to
bile that is excreted in the bowel and is consid- follow the protocol of administration of
ered acceptable. On the other hand, the presence GoLYTELY®.
of particles of fecal matter is considered unac- Once the patient is in the operating room,
ceptable, and therefore, the administration of prior to the operation, we routinely pass a large
GoLYTELY® should continue. Subsequently, the Foley catheter through the rectum, to evacuate
resident or the nurse should check what is com- the remaining bowel prep fluid that is in the
ing out of the rectum every hour as the prep con- colon.
tinues to run, until the goal of a clean colon is Otherwise, this fluid becomes an inconvenience
achieved. At that point, the nasogastric tube can during the surgical procedure. Also, we pass that
be pulled out, and the patient is allowed to drink tube, in order to do a last irrigation before we start
clear fluids. (Clear fluids by mouth are not the operation. Surprisingly, sometimes, even when
allowed during the administration of the it was reported that the patient was clean, we find
GoLYTELY® because we were told by the com- stool in the colon. Occasionally, we have to cancel
pany that produces GoLYTELY® that the glucose a case because we were not successful in cleaning
contents allow for inflow of fluid into the colon the colon even in the operating room. We are very
lumen, leading to dehydration.) proud of our postoperative results.
Most patients pass clear liquid through the
rectum after 4 h, but others require a longer
period of time. Occasionally, we see patients that 7.2 Primary Procedures
continue having GoLYTELY® through the night, for the Treatment
and then come to the operating room, only for us of Anorectal Malformation
to find out that they are still not clean. During the Newborn Period
It has been our experience that many patients
who received GoLYTELY® the day before an There are some malformations that we repair pri-
operation come to the operating room suffering marily without a colostomy during the newborn
from some degree of dehydration and a mild period. It has been our experience that these
degree of metabolic acidosis. This seems to be patients behave better from the point of view of
more significant in babies. Therefore, it is our the possibility of infection, dehiscence, and
routine to administer intravenous fluids during retraction, as compared to older patients. We
the entire process to patients younger than 2 years speculate that, perhaps, due to the fact that the
of age. In patients older than 2 years of age, intra- meconium has not been colonized during the first
venous fluids are desirable, but not vital. few days of life, the chances of a wound infection
In patients with a significant degree of mega- may be less.
colon and chronic fecal impaction, it is very Full-term, stable newborns, who are other-
important to go through the process of fecal wise healthy, without major associated defects,
disimpaction prior to the administration of born at our institution or nearby and having what
GoLYTELY®. The administration of we call a “benign anorectal malformation,”
GoLYTELY® through a nasogastric tube in receive a primary procedure during the newborn
patients with fecal impaction and severe megar- period without a full bowel preparation. These
ectosigmoid sometimes makes them feel very cases include rectovestibular fistulas, recto-peri-
uncomfortable. Their abdomen becomes very neal fistulas in males and females, anorectal mal-
distended, they complain of severe cramps, and formation without fistula in both male or female
they feel very sick. The protocol of fecal disim- patients, or rectourethral bulbar fistulas. In the
104 7 Bowel Preparation in Pediatric Colorectal Surgery

last two types of malformations, we expect to are performed without a protective colostomy;
see the distal rectal end, full of gas, located yet, we request consent for a colostomy in case it
below the coccyx, on the cross table lateral film is necessary.
(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- 7.4 Patients with Hirschsprung’s
ing unnecessary risks of injuring the urinary Disease with Enterocolitis
tract. These patients are operated on at our insti- After the Neonatal Period
tution usually within the first 48 h of life without
bowel preparation. A baby with enterocolitis is an extremely delicate,
sick patient. Therefore, he or she must be managed
in an expeditious way. The management includes
7.3 Primary Pull-Through recto-colonic irrigations, adequate aggressive
in Newborn Patients hydration, and administration of metronidazole
with Hirschsprung’s Disease (intravenously at the beginning, eventually by
mouth and later through rectal irrigations).
When a baby is born at our institution, or is Occasionally we add broad-spectrum antibiotics.
brought during the first days of life, with the The irrigations are performed as often as nec-
diagnosis of Hirschsprung’s disease, we first con- essary to decompress the colon and alleviate the
firm the diagnosis, and then, we introduce a naso- abdominal distention. It is important to keep in
gastric tube for gastric decompression and start a mind what is happening in the colon. Stasis
program of rectal irrigations, performed as often occurs, leading to bacterial overgrowth, secretory
as necessary to decompress the colon. It is very diarrhea, and dehydration. It is this cycle that
important for doctors and nurses to understand must be broken by the irrigations. During the
the difference between a rectal irrigation and an acute stages of enterocolitis, the irrigations will
enema. A patient with Hirschsprung’s disease has produce a characteristic type of stool that is par-
a congenital, intrinsic incapacity to empty the ticularly fetid and frequently with abnormal bac-
colon due to a severe motility disorder. Therefore, teria, such as Clostridium difficile. We keep these
in general, enemas are to be avoided, as they may patients fasting, receiving parenteral nutrition
simply be retained in the colon and can worsen and with the irrigations per rectum until they
the distention. Irrigation, on the other hand, con- become asymptomatic. Fairly soon, the patient
sists of passing a large-lumen tube, through the has a flat abdomen, no vomiting, and no diarrhea.
rectum and irrigating with small amounts In addition, the fetid stools disappear and are
(10 mL) of saline solution at a time. The saline replaced by clear fluid with bile. At this point, we
solution is injected with a catheter-tip syringe consider that the patient is free of enterocolitis
through the tube just to clear its lumen, to allow and therefore ready to be operated on. If we feed
decompression of the colonic contents. these patients, they may develop enterocolitis
Characteristically, the patient passes gas and liq- again. Therefore, we prefer to do the operation at
uid stool in an explosive manner through the this point. These patients require longer periods
lumen of the tube. A dramatic clinical improve- of rectal irrigations and fasting than neonates
ment can be observed in these babies after every before they are ready for an operation. In fact,
irrigation. Within a couple of days, the abdomen some of these patients receive a diverting colos-
is usually flat, and what we obtain with the irriga- tomy when we feel that the patient is not improv-
tions is only bile. At that point, and provided the ing with the medical treatment described.
diagnosis has been histologically confirmed, the Sometimes it takes 1 or 2 weeks for the patient to
patient is ready to undergo a primary pull- be ready for the operation to be sure that there is
through. We do not administer GoLYTELY® in no evidence of enterocolitis. We do not adminis-
these kinds of patients. Most of these operations ter GoLYTELY® in these kinds of patients.
References 105

7.5 Patients with Hirschsprung’s We have done over 700 colostomy closures, and
Disease Beyond we are very proud of the fact that none of our
the Neonatal Period, patients suffered from a wound infection [5]. None
Without Enterocolitis of the patients had any kind of Penrose drainage
left in the wound or in the abdomen, and all wounds
Occasionally, we see patients at school age, or were closed primarily including a subcuticular
even adolescence, who come to us suffering stitch. Only two patients had postoperative compli-
from Hirschsprung’s disease. They usually have cations: one of the patients had an anastomosis
a very severe megacolon and yet no history of dehiscence, and the other patient had a colonic per-
episodes of enterocolitis. We believe that this foration proximal to the anastomosis. We do not
type of patient suffers from a “benign type” of have an explanation for that complication.
Hirschsprung’s disease. For reasons that we do
not know, these patients never had an episode
of enterocolitis and behave in a more benign 7.7 Patients with a Colostomy
way than the rest of the patients with Who Will Have a Repair
Hirschsprung’s disease. The colon preparation of an Anorectal
in these patients is the same as the one described Malformation
for major pull-throughs. In other words, if the
patient is fecally impacted, we first disimpact When the colostomy is totally diverting (com-
them, and then we bring them to the hospital to pletely separated stomas, with the bag covering
administer GoLYTELY®, 1 or 2 days before the only the proximal stoma), and the surgeon is sure
operation. 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-
7.6 Colostomy Closures essary to irrigate the distal one.
Sometimes, even when the rectum is consid-
The preparation of the bowel in these patients is ered reachable from below, the length of the
much more simple. The patient has a normal colon available between the distal stoma and the
breakfast the day prior to surgery and comes to most distal end of the rectum is so short that it is
the hospital thereafter. The rest of the day, the fair to assume that this distance will interfere
patient is maintained on clear fluids by mouth with the pull-through, making it necessary to
until 6 h before surgery. During that day, the mobilize the proximal stoma. In such cases, it is
nurses irrigate the proximal stoma with saline necessary to clean the entire gastrointestinal tract
solution. There is no need to irrigate the distal with GoLYTELY® as previously described. In
stoma, which is, by definition, clean and free of addition, in certain cases that may require a vagi-
stool. The patient receives irrigations through a nal replacement, a clean colon allows the surgeon
no. 10 Foley catheter with injections of 10–20 mL to consider the bowel from the proximal stoma as
of saline solution, allowing the saline to come an option for the neovagina.
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 References
GoLYTELY® for this preparation.
When the patient has two stomas that are 1. Stellato TA, Danziger LH, Gordon N, Hau T, Hull
located too close one to the other, and both sto- CC, Zollinger RM Jr, Shuck JM (1990) Antibiotics in
mas are included under the same stoma bag, it is elective colon surgery. A randomized trial of oral, sys-
temic, and oral/systemic antibiotics for prophylaxis.
our routine to irrigate both stomas the day before Am Surg 56(4):251–254
surgery. The same routine is applied for patients 2. van Geldere D, Fa-Si-Oen P, Noach LA, Rietra PJ,
who have a loop colostomy. Peterse JL, Boom RP (2002) Complications after
106 7 Bowel Preparation in Pediatric Colorectal Surgery

colorectal surgery without mechanical bowel prepara- erative mechanical bowel preparation. J Pediatr Urol
tion. J Am Coll Surg 194(1):40–47 8(2):201–204. doi:10.1016/j.jpurol.2011.01.015
3. Wille-Jørgensen P, Guenaga KF, Matos D, Castro AA 11. Leal AJ, Tannuri AC, Tannuri U (2013) Mechanical
(2005) Pre-operative mechanical bowel cleansing or bowel preparation for esophagocoloplasty in children:
not? An updated meta-analysis. Colorectal Dis is it really necessary? Dis Esophagus 26(5):475–478.
7(4):304–310 doi:10.1111/j.1442-2050.2012.01378.x
4. Dahabreh IJ, Steele DW, Shah N, Trikalinos TA 12. Pennington EC, Feng C, St Peter SD, Islam S, Goldin
(2014) Oral mechanical bowel preparation for AB, Abdullah F, Rangel SJ (2014) Use of mechanical
colorectal surgery [Internet], Report No. bowel preparation and oral antibiotics for elective
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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 8 Recto-perineal Fistula

a b

Fig. 8.2 Perineal fistula with a significant anterior mislocation of the anal opening. (a) Picture. (b) Diagram

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
Fig. 8.3 “Fourchette” type of fistula
for bowel control. In fact, in our experience,
100 % of the patients operated by us have bowel
extremely rare. In addition, we believe that control provided the sacrum is normal. The
in the event of seeing a case suffering from majority of patients have a good sacrum, but
such condition, we would be very hesitant to there are exceptions, as we will be discussing
operate. below.
8.2 Associated Defects 109

The chances for these patients to have bowel we document a case of perineal fistula with a
control are 100 % provided they receive a good presacral mass, we have to screen the other
operation. Ironically, the incidence of constipa- members of the family for the same defect. This
tion in these patients is the highest of the entire is extremely important because we have seen
spectrum of anorectal malformations!! In fact, patients that were diagnosed as having a peri-
we observed that the higher the malformation, neal fistula received an operation focused only
the poorer the prognosis for bowel control, but in the anal defect, and the patients were left with
the better the prognosis for constipation, or the a presacral mass and sometimes with very nega-
other way around, the lower and more benign the tive consequences. In addition, having diag-
malformation, the highest chances of bowel con- nosed a sacral defect with a presacral mass
trol, but also the highest possibilities of suffering changes completely the functional prognosis for
severe constipation. The reason for this is these patients. This, we believe, it is extremely
unknown. Interestingly, higher malformations important to diagnose, to discuss with the par-
require much more perirectal dissection in order ents, and to adjust their expectations in terms of
to bring the rectum down to the perineum. This future functional prognosis. In addition, as will
dissection means that we divide the vessels and be described later, this complex triad requires a
the nerves that surround the rectum in order to specific therapeutic strategy.
mobilize it down. In other words, higher rectums The association of anorectal malformation,
require more denervation, yet they have less con- sacral defect, and presacral mass is well known
stipation!! Patients with perineal fistulas require [32–61] and is frequently called Currarino
a minimal degree of mobilization and therefore triad [38].
denervation, and yet they have the worst degree Unfortunately, there is a group of patients who
of constipation. were born with perineal fistula, as previously men-
Another important characteristic of this par- tioned, a defect with an excellent functional prog-
ticular defect is that there is a group of patients nosis, and yet, they end up suffering from fecal
with perineal fistulas that run in families. It is incontinence after several therapeutic misadven-
well documented in the literature that about 1 % tures and catastrophic events. Those are totally
of all patients with anorectal malformations preventable problems consecutive to the lack of
have a sibling with an anorectal malformation knowledge of the management of these patients.
[14]. In other words, traditionally, when the par-
ents ask about the risk of having a second child
with an anorectal malformation, the standard 8.2 Associated Defects
answer from geneticists, pediatricians, and us is
1 %. Yet, a deeper and more thoughtful analysis Following the same pattern already observed in the
of the question reveals that, actually, we have entire spectrum of anorectal malformations,
never seen a family with two cloacas. In con- perineal fistulas have the lowest incidence of
trast, we have several families that have two or urologic-associated defects or functional disorders.
more siblings with perineal fistula frequently According to our database, 18 % of female patients
associated to a presacral mass and sacral defect. and 27 % male patients with perineal fistulas suffer
The familiar characteristic of this condition has from urologic problems. Absent kidney occurs in
been published many times [15–31]. Some other 2–4 % of the cases, vesicoureteral reflux in 3–5 %,
members of the family may have the sacral hypospadias in 1 %, ectopic ureters in less than
defect and the presacral mass and not the peri- 1 %, vaginal septum in 2 %, hyposadias in 1 %,
neal fistula. For this reason, in a patient with undescended testicle in 2 %, bifid scrotum in 8 %,
perineal fistula, it is mandatory to document the and hydronephrosis in 6 %.
integrity of the sacrum with an AP x-ray film of Vertebral anomalies also are less common in
the sacrum to rule out a sacral defect because of these defects as compared to the others.
the high incidence of sacral defects and presa- Hemivertebra occurs in less than 2–4 % of the
cral masses associated to perineal fistulas. Once cases, and sacral defects are very unusual. The
110 8 Recto-perineal Fistula

sacra in these patients are usually normal except untouched, most of the times, that strip of mucosa
in those patients who are born with presacral suffers from metaplasia and changes into some-
mass and hemisacrum. thing that looks very much like skin. Yet, we have
Patent ductus arteriosus occurs in less than seen patients of 5–7 years old in which that
3 %, atrial septal defect in 2 %, ventricular septal mucosa continued producing some degree of
defect in 1 %, tetralogy of Fallot in 1 %, and wetness, which prompted us to operate. The
esophageal atresia in 1 %. operation of the perineal groove requires only
Tethered cord is present in 29 % of male removal of a very thin layer of mucosa and sutur-
patients and 13 % of female patients. This rela- ing together in the midline the skin of the peri-
tively high frequency of tethered cord is explained neal body. This operation is usually done in
by the fact that patients with perineal fistula have conjunction with the mobilization of the anal
a high frequency of association with sacral opening back to the center of the sphincter.
defects and presacral masses. Sometimes, the anus looks like it is surrounded
by a normal sphincter mechanism. Yet, with an
electrical stimulator, under anesthesia, it is
8.3 Diagnosis extremely easy to demonstrate that it is just an
optical illusion. The bulk of the sphincter mecha-
8.3.1 Female Patients nism is located behind the anal opening and
extends like a horseshoe on both sides of the anus,
The diagnosis of a perineal fistula in a female but there is no sphincter whatsoever between the
patient is a straightforward one. It is enough to female genitalia and the anal opening.
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). 8.3.2 Male Patients
As previously mentioned, the orifice may be
located very close to the female genitalia or very The diagnosis in the male patient may be a little
close to the center of the sphincter, and so the bit trickier. The baby may have a very obvious
degree of mislocation varies from patient to orifice in the perineum through which one can
patient. There is also a specific defect called by see meconium coming out. In such case, the diag-
Stephens [62] “perineal groove” which is a strip nosis is easy (Fig. 8.5). The fistula is always
of mucosa that runs between the female genitalia located anterior to the center of the sphincter. We
and the anus. This defect is frequently associated have never seen a case in which the anal opening
to perineal fistulas (Fig. 8.4). When this is left 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
Fig. 8.4 Picture of a perineal groove the perineum. One should not hesitate to use
8.3 Diagnosis 111

Fig. 8.5 Perineal fistula in a male. The anal orifice is


very obvious

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
Fig. 8.6 Very small perineal fistula
both circumstances the surgeon “confirmed” his
erroneous belief that he was dealing with a case
magnifying glasses if necessary to look carefully of “high” anorectal malformation. Because of
for an orifice that sometimes is inconspicuous. that, he went ahead with an abdominoperineal
Figures 8.7, 8.8, 8.9, and 8.10 show different operation designed for the treatment of more
appearances of the same malformation (perineal complex malformations, an operation that is not
fistula). indicated in these cases, and that leaves the
We intentionally eliminated confusing terms patients fecally incontinent. This is something
used in the past to refer to this defect. These terms tragic that should never occur if one follows spe-
include: “anal membrane,” “membranous steno- cific steps in the diagnosis of these defects.
sis,” “covered anus,” “anocutaneous fistula,” and The perineum of these patients may have dif-
“translevator defect” [63–68]. ferent external appearances. Sometimes they
112 8 Recto-perineal Fistula

Fig. 8.8 The discoloration of the skin, posterior to the


fistula opening represents the location of the sphincter
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
Fig. 8.9 Arrow shows fistula opening a little out of the believes that it runs at a subcuticular level until it
midline reaches the rectum, and therefore, it can be treated
8.3 Diagnosis 113

a b

Fig. 8.11 Bucket handle malformations. Three different variants (a–c)

like a regular perineal fistula. Yet, surprisingly, from the urethra. Fortunately, in most of the cases,
one may find that the fistula runs rather parallel to one can say that if one is able to see a perineal
the urethra, deeper and deeper in to the perineum, orifice passing meconium, it means that we are
and through the corpora (Fig. 8.15). As a conse- authorized to operate without a colostomy in the
quence, trying to follow that structure, the sur- way that it was described.
geon may provoke significant bleeding. Actually, A sacral defect seen in an anterior/posterior
these specific variants are exceptions to the rule. view of an x-ray film (Fig. 8.16) is always associ-
These malformations are considered rather com- ated to a presacral mass [38], and the defect can
plex because they require more mobilization of be very small (Fig. 8.17) or giant (Fig. 8.18). It
the rectum as well as to be separated completely can be located laterally, giving the impression of a
114 8 Recto-perineal Fistula

Fig. 8.12 Perineal fistula located at the base of the penis 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
Fig. 8.13 “Black ribbon” malformation. Variant of a mass. An MRI study will provide more accurate
perineal fistula (subepithelial with meconium) information about the characteristics of the mass,
8.3 Diagnosis 115

Fig. 8.19 Bifid sacrum, the mass is located in the


Fig. 8.16 Hemisacrum
midline

Fig. 8.17 Small sacral defect

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
Fig. 8.18 Giant sacral defect. The size of the presacral associated to perineal fistulas or to “rectal steno-
mass corresponds to the size of the sacral defect sis” represent an excellent example of the impor-
tance of having a good index of suspicion. The
as well as the possible communication with the presence of the mass may dramatically change the
dural space (anterior meningocele) (Fig. 8.20). functional prognosis for bowel and urinary func-
The most common types of presacral masses are, tion, particularly if the sacral defect is large.
116 8 Recto-perineal Fistula

In other words, a perineal fistula with normal rectal stenosis (Fig. 8.21). The perineal appear-
sacrum has an excellent functional prognosis, yet ance of these patients deserves a special com-
the presence of a mass is a rather devastating find- ment. This type of perineum is known as “funnel
ing. The surgical strategy also changes, since it anus” (Fig. 8.22). It certainly looks like a funnel.
must include the resection of the mass at the same The pectinate line is not visible outside because it
time of the repair of the anorectal malformation. seems to be located higher, by the pushing effect
This resection can be a rather easy procedure or produced by the mass. This external appearance
can also be a formidable operation that requires must prompt us to order an AP x-ray film of the
the participation of a neurosurgeon. sacrum and an MRI of the pelvis, in order to con-
Some patients are born with a normally firm the diagnosis of the presacral mass and rec-
located anus and a presacral mass producing a 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 117

8.4 Management the wrong type of operation or technically incor-


rect procedure that ended up in a series of compli-
Since these defects represent the simplest of all cations that will be described later. Therefore, we
anorectal malformations, the operation designed can say that it is preferable for the patient to receive
to repair these defects is also a limited, rather anal dilatations rather than a poor operation. The
simple, yet meticulous operation. Operations to fact is that these patients will have bowel control
repair these defects are performed without a pro- with and without an operation. We explain that, by
tective colostomy. The exceptions to this rule the fact that the mislocation of the anal orifice is
include cases of misdiagnosis. More specifically, only present in the lowest part of the rectum
some male patients are born with no anal open- (Fig. 8.2), most of the rectum, however, is well
ing; they rather have a tiny perineal orifice that located traversing within the funnel-like muscle
goes unrecognized, and the surgeon erroneously structure and is only deviated in the lowest part. It
believed that the patient had a “high” malforma- is this type of case that exemplifies the fact that a
tion and opens a non-indicated colostomy. perfect location of the anus, within the sphincter
When the patients are born full term at our mechanism, is not a precondition to have bowel
institution, with no serious associated defects, we control. Bowel control then seems to depend on
operate on them without bowel preparation within many other factors besides anal location.
the first 72 h of life. Unfortunately, however, Anal dilatations, from our point of view, are then
patients frequently come to us weeks or months indicated under specific circumstances. One of
after they were born, and in that type of case, we those could be a patient that has multiple serious
feel it is safer to prepare the gastrointestinal tract associated defects and abdominal distention.
with GoLYTELY as described in the chapter Premature babies with severe cardiac conditions
related to colonic preparation. In the case of a that are not in good condition to be taken to the
newborn operated in the first hours of life, we feed operating room are also good candidates to receive
the patient usually 3–5 days after the operation. anal dilatations. The protocol of dilatation should be
We believe that those babies operated early, with- as the one described here (Chap. 18). Another rea-
out bowel preparation, have a better postoperative son to do dilatations is the case in which the surgeon
course and less chance of infection, perhaps due has no experience with performing a formal poste-
to the fact that the bacterial proliferation in the rior sagittal anoplasty for these patients. In that case,
bowel is nil or very limited. However, in older the chances of hurting the patient are much less with
patients, we are stricter in the protocol of manage- anal dilatations than with an operation. The opera-
ment. We use GoLYTELY for total bowel prepa- tion also could be done later. Anal dilatations in
ration (see Chap. 7), insert a central line, and keep cases of perineal fistula are sometimes rather diffi-
the patients 10 days with nothing by mouth. cult and painful. The reason is that one has to dilate
During the newborn period, there are several a congenital stricture type of anomaly. This is not
therapeutic options for these patients. The first the same as in cases of postoperative dilatations
one is simple dilatation of the perineal orifice. after a technically correct operation; in those cases
usually the dilatations are uncomfortable, but not
painful. Very painful dilatation usually means a
8.5 Dilatations congenital stricture or ischemia that occurred during
the performance of an anoplasty.
Dilatations usually help the patient to eliminate the
stool and avoid abdominal distention. This treat-
ment modality is preferred by many doctors all 8.6 Cutback Operation
over the world, based on the fact that these patients
will have bowel control with and without an opera- The cutback operation is another therapeutic
tion. In fact, misadventure and catastrophic events alternative for these patients. The indications are
in these patients occur when somebody performed similar to the ones of the anal dilatation. In fact,
118 8 Recto-perineal Fistula

it is a procedure that can be done with local handling of tissues, and avoiding excessive burn-
anesthesia. Again, we do not think that it should ing. There is a significant difference in the surgical
be the final ideal operation for these patients, but technique for perineal fistulas in male and female
it is certainly an alternative for patients who are patients.
extremely sick or in situations in which the sur- Obviously, when dealing with patients with
geon does not have the necessary training to perineal fistulas associated to a presacral mass, it
perform a formal posterior sagittal anoplasty. The is necessary to use a full posterior sagittal inci-
cutback procedure consists of making an incision sion [47, 69–71].
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 8.7.1 Male Patients
cosmetic appearance after the cutback is less than
optimal, yet this operation does not really hurt A few authors referred to this malformation
the sphincter mechanism or the innervation of the using the terminology that we used, calling this
bowel, and therefore it is considered a good con- defect “recto-perineal fistula” [72–75]. The real
temporizing preliminary operation, designed to challenge in male patients with perineal fistula is
facilitate the passing of stool and alleviate the the separation of the rectum from the urethra
abdominal distention. (Fig. 8.24). In fact, the most common and feared
intraoperative accident of this procedure is the
urethral injury. This happens when the surgeon
8.7 Minimal Posterior Sagittal underestimates the complexity of this operation
Anoplasty and takes the baby to the operating room without
a Foley catheter in the bladder. Many surgeons
We believe that the ideal procedure to repair a peri- operate on these babies in the lithotomy posi-
neal fistula is what we call “minimal posterior sag- tion; we do it in prone position. One must keep
ittal anoplasty.” Our experience with this operation in mind that the urethra of an infant or a newborn
includes 174 cases with excellent results from the male baby is an extremely delicate thin structure
functional and cosmetic point of view (Fig. 8.23). and can be divided inadvertently. That is why we
We recognize, however, that even when it is a cannot overemphasize the need to use a Foley
small procedure, it is technically demanding. This catheter in all of these cases. In addition, during
is the kind of operation that requires experience, the repair, one must keep thinking very specifi-
meticulous technique, familiarity with the delicate 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
Fig. 8.23 External appearance of an excellent operative patient to anal dilatations and/or cutback type of
result procedure.
8.7 Minimal Posterior Sagittal Anoplasty 119

Fig. 8.24 Diagram of a perineal fistula in a male patient, pre- and postoperative

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,
Fig. 8.25 Suprapubic cystogram and anterior urethro- the anterior posterior diameter of the sphincter
gram in a patient who suffered from an accidental com-
mechanism is approximately 2–3 cm. The incision
plete division of the urethra, during the repair of a perineal
fistula. (a) Blind end of the distal urethra. (b) Distal ure- is continued deeper through the entire sphincter
thra draining next to the anus mechanism. The surgeon must keep in mind the
120 8 Recto-perineal Fistula

Fig. 8.26 Surgical repair of a perineal fistula in a male


patient – multiple silk stitches on the fistula site

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
Fig. 8.27 Posterior sagittal incision, dividing the sphinc-
while applying traction to the rectum, we are also
ter mechanism 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
fact that near the fistula site (most distal rectum), important sign that indicates that the surgeon is
the rectal wall is almost in intimate contact with the dangerously getting close to the urethra is to find
skin; as we become more proximal in the bowel, the kind of bleeding that is seen when working in
the distance between the skin and the bowel is spongiosum/cavernosum type of tissue of the
greater. The entire sphincter mechanism is divided penis. This indicates that we are dissecting very
until we are able to identify the characteristic white 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


a Foley catheter (Fig. 8.32).
The operation in female patients is usually sim- We have been impressed by the fact that one
pler than in the male patients because there is no of the anorectal malformations for which the
risk of injuring the urethra. The equivalent to the parents have more problems in making a deci-
urethra would be the vagina, yet it is extremely sion about to operate or not to operate is pre-
unusual to see cases of vaginal injuries during the cisely a perineal fistula in female patients. We
repair of a perineal fistula. This is due to the fact feel morally obligated to tell the parents that
that the vagina and rectum are usually signifi- babies with perineal fistulas will have bowel
cantly separated although one should not under- control with and without an operation. Another
estimate the possibility of injuring the vagina. very important fact in these cases is that the
The technique is the same as described for male patients will suffer from severe constipation
patients except for the fact that there is no possi- 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 123

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 Fig. 8.33 Contrast study showing a dilated rectum and a
narrow lower portion compressed by a presacral mass
why we believe that these female patients will
benefit from this operation, and that is the fact
that leaving the patient with an anteriorly operating room accompanied by a neurosurgeon
located anal orifice means that they have a very because it is a rather unpleasant surprise to find
short perineal body. We have seen teenage that the dura has been open and the patient runs the
patients that when they discover the character- risk of suffering meningitis and (or) cerebrospinal
istics of their perineal anatomy, they express fluid fistula. After the mass has been resected, the
their dissatisfaction, and they demand a repair anterior meningocele is resected and the dura is
later in life, which is a more uncomfortable closed with nonabsorbable sutures. Sometimes it
experience than when it is done in the newborn is necessary to develop muscle and cartilage
period. Also, a vaginal delivery of a baby may patches to be sure that the dura space is well
have higher chances of a rectal injury in a sealed. The mass must always be resected because
mother with a short perineum. Because of this, we have seen patients later in life that suffer from
we believe that it is indicated to do the opera- an infection of the mass, and also there is a certain
tion in female patients provided the surgeon is degree of risk of malignancy [41].
familiarized with the surgical technique and is Once the mass is resected, we can mobilize the
delicate enough to do it. In male cases, we can- rectum down. We should not try to dilate the long
not argue that the operation will be good for narrow fistula that is the result of the compression
those kinds of reasons, and therefore we simply of the presacral mass. Usually that portion of rec-
tell the parents that the advantage of the opera- tum is non-dilatable and therefore must be resected.
tion is a better cosmetic effect as compared Attempts to dilate this kind of rectum have failed in
with cutback or simple dilatations. We respect several cases and were referred to us after many
the parents’ decision. attempts of doing that. One must go and find the
Cases with perineal fistulas associated to a pre- upper rectum and pull it down to the perineum.
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 8.8 Postoperative Care
one because it requires resection of the mass. Most
presacral masses do not have an anterior meningo- As we previously described, these babies, when
cele component. However, many have and there- operated in the newborn period with meconium
fore if the pediatric surgeon has no experience in in the bowel, can be fed after 3–5 days, and they
dealing with anterior meningoceles must go to the do not need parenteral nutrition. When the babies
124 8 Recto-perineal Fistula

are operated weeks or months later with real stool fecal impaction. We went ahead with our protocol
in their colons, we keep them 10 days with noth- of disimpaction (see Chap. 25, Sect. 25.7.1).
ing by mouth, receiving parenteral nutrition. Following that, we determined, by trial and error
We must anticipate a high degree of constipa- and radiologic monitoring, the laxative requirement
tion in these patients. We cannot overemphasize of the patient. We usually found that it was a very
the importance of assuming that the patient is large amount of laxative, what the patient required
going to be severely constipated and assuming to allow the emptying of the rectosigmoid as radio-
that he will need an amount of laxatives much logically demonstrated. Once we reached that
larger than what other patients need. The magni- amount of laxative, we found that the patient was
tude of constipation is worse when the defect is actually fecally continent and all the patient
associated with a presacral mass. required from the beginning was the administra-
Constipation must be treated aggressively to tion of the right amount of laxatives. Those are
prevent severe consequences. We must keep in very rewarding experiences in dealing with that
mind that these babies are born with an abnormal kind of patients.
rectum that does not have the normal peristalsis, Unfortunately, we also have been exposed to
and therefore these patients need help to empty about 35 patients that had the same symptomatol-
their rectum. We must also remember that every ogy just described, but unfortunately a surgeon sus-
patient has a different laxative requirement that is pected that the patient could have Hirschsprung’s
not what it says in traditional books for the man- disease. We strongly believe that Hirschsprung’s
agement of constipation. These patients need 2, 3, disease is not more common in patients with ano-
4, 5, and 10 times larger dosages of laxatives than rectal malformations than in the general popula-
other patients need. Constipation means incapacity tion. Yet, the incidence of constipation in patients
to empty the rectum which results in accumulation with anorectal malformation is extremely high, and
of stool that leads to formation of a fecal impaction most of us pediatric surgeons were trained to sus-
that produces megacolon. All pediatric surgeons pect Hirschsprung’s disease whenever we deal with
are familiar with the fact that hollow viscus sub- a child with constipation. Consequently, those
jected to an abnormal dilatation loses its peristaltic patients received a rectal biopsy, and occasionally,
efficiency. This phenomenon has been observed in those rectal biopsies show no ganglion cells which,
cases of colonic, duodenal, and small bowel from our point of view, do not necessarily make the
obstruction, as well as in megaureters. A dilated diagnosis of Hirschsprung’s.
bowel loses it peristalsis, and therefore, constipa- In a patient with Hirschsprung’s disease, we
tion produces retention, retention produces dilata- expect to see not only absent ganglion cells, but we
tion, dilatation produces lack of peristalsis, lack of also request an accurate description of the site
peristalsis produces more constipation, creating a where the biopsy was taken from, and also we
vicious cycle that ends up with patients that behave expect from the pathology department to tell us
like being incontinent when actually they suffer whether or not there was an increase in the activity
from overflow pseudoincontinence (see Chap. 18). of acetylcholinesterase as well as the presence of
This is highly inconvenient because we must keep hypertrophic nerves. If we do not find that kind of
in mind that we are dealing with patients that have abnormalities and in the absence of symptoms of
an excellent prognosis for bowel control. We have enterocolitis, we simply do not believe that these
seen 25 patients that came to our clinic suffering patients have Hirschsprung’s. Some of these
from “fecal incontinence” and severe constipation. patients, as we said, had a biopsy done at other
When evaluated for the purpose of providing institutions that showed unfortunately absent gan-
bowel management, we found that they were born glion cells as a single finding with a dilated rectum,
with one of these malformations, they received a which the surgeons considered enough evidence
technically correct operation, and yet they suffered for the diagnosis of Hirschsprung’s and went ahead
from “fecal incontinence.” A contrast enema with an abdominoperineal resection. An abdomi-
revealed that they had a huge rectosigmoid with noperineal resection in a constipated patient with
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Rectourethral Bulbar Fistula
9

9.1 Introduction urethra) (Fig. 9.3) [1, 2, 6]. Some authors use the
term “ano-penile-urethral fistula” [6].
We define this malformation as a defect in a male Until the moment of writing this manuscript,
in which the rectum is abnormally communicat- we have had experience with the treatment of 231
ing with the lowest portion of the posterior ure- cases of rectourethral bulbar fistula. Two hundred
thra (Fig. 9.1). Figure 9.2 shows the characteristic and nine of them were primary operations and 22
image of a distal colostogram performed in a were reoperations.
patient with this malformation. As we have mentioned several times, every
This is the most common anorectal malforma- case represents an anatomic variant. Therefore,
tion defect in males in our series. This, of course, one can expect to see cases of rectourethral bulbar
should not be interpreted as an accurate reflection fistula in which the rectum opens into the urethra a
of the incidence of this defect in the general pop- little higher than the bulbar area. In other words,
ulation, since ours is a referral center, which the rectum connects with the urethra anywhere
means that in general, we tend to receive more between the prostatic urethra (middle portion of
complex defects and less of the benign and lower the posterior urethra) and the bulbar portion of the
type of malformations. urethra (the lowest part of the posterior urethra).
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 9.2 Associated Defects
common defect. The reason for this, we believe,
is that most authors unfortunately are still using A significant number of our patients with recto-
the terminology of “high,” “intermediate,” and urethral bulbar fistula have some sort of associ-
“low”. Some authors consider this an “intermedi- ated defect, mainly urologic. Hypospadias occur
ate” malformation [1, 5]. in 10 % of the cases, vesicoureteral reflux in
Consistent with the concept that dictates that about 13 % of the cases, and absent kidney in
these malformations occur in a spectrum fashion, 10 % of the cases.
it is relatively common to see cases in which the The sacrum, in this type of malformation, is
fistula is connected even more anteriorly (penile 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
Electronic supplementary material Supplementary
material is available in the online version of this chapter at evaluated for the presence of the tethered cord;
10.1007/978-3-319-14989-9_9. seven of them had that defect (15 %).

A. Peña, A. Bischoff, Surgical Treatment of Colorectal Problems in Children, 129


DOI 10.1007/978-3-319-14989-9_9, © Springer International Publishing Switzerland 2015
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


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 %.
Fig. 9.2 Distal colostogram showing a rectourethral Patent ductus arteriosus occurs in 8 % of the
bulbar fistula 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 9 Rectourethral Bulbar Fistula

pressure in order to force the meconium through 9.3 Posterior Sagittal


the fistula into the urethra. Therefore, one should Anorectoplasty
not expect meconium in the urine in the first 20 h
of life (see Chap. 4 of this book). We perform this operation as early in life as pos-
If the baby is born in an institution where the sible, since we try to avoid unpleasant memories
surgeons have a demonstrated interest and expe- related to the operations and rectal dilations in
rience in the surgical management of neonatal the patient. When the patients are born at our
patients, it is conceivable that these patients could institution, they receive a colostomy at birth,
be treated posterior sagittally during the newborn and 2–4 weeks after that operation, assuming
period without a colostomy. The fundamental that the baby has recovered well, is growing,
requirement to perform that kind of treatment is and developing normally, the baby is ready to
that the surgeon could see the image of the rec- undergo this operation. Many times, the patients
tum located well below the coccyx as delineated are referred to us later in life, and that is why not
by the presence of gas. By seeing that image in a all of our patients have been operated early
cross-table lateral film, one can be sure that by in life.
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 9.4 Surgical Technique
our institution with a colostomy already opened
at another hospital. Under good general anesthesia and endotracheal
If the surgeon has no experience in the man- intubation, the patient is placed first in lithotomy
agement of neonates with anorectal malforma- position. We use a baby cystoscope routinely in
tions, it is much safer to open a colostomy all of our cases to confirm the diagnosis of recto-
(Chap. 5). urethral bulbar fistula. We take advantage of this
A laparoscopic approach is justified and conve- procedure to learn more about the anatomy of the
nient in cases of anorectal malformations that posterior urethra. We have been learning impor-
would require a laparotomy; yet, it has been done tant anatomic facts from the cystoscopies in male
in these kinds of cases [8–20]. We are definitely patients with anorectal malformations, including
opposed to that approach in this particular malfor- abnormalities in the verumontanum, abnormal
mation. A posterior sagittal approach done in these locations of the prostatic utricle, and ectopic ure-
patients is an operation that takes approximately teral openings in the bladder neck and posterior
2 h. The rectum is minimally dissected, just enough urethra. We also look at the bladder neck as well
to reach the perineum, which is very close. The as the ureteral orifices and the bladder in general.
abdomen is not entered; the results are very good. At the end of this procedure, we pass a Foley
The patient has mild pain, can eat the same day of catheter into the bladder. Sometimes the recto-
the operation, and can be discharged the following urethral bulbar fistula is a large one, and due to its
day. We do not understand the rationale of attempt- location, the catheter tends to go into the rectum.
ing the repair of these defects through the abdo- This happens in approximately 20 % of the cases.
men. A consequence of laparoscopic attempts to If this happens, we can try to introduce a special
repair bulbar fistulas is the posterior urethral diver- catheter with a curved tip, called “coude cathe-
ticula (pieces of rectum left attached to the urethra), ter.” We must try to direct the curve of the cathe-
due to the fact that the surgeon was unable to reach ter anteriorly and cephalad in order to avoid the
the end of the bowel (which resides well below the catheter going into the rectum. This may or may
peritoneal reflection) through the abdomen, laparo- not work. If it does not work, then we can take a
scopically [21] (Animation 9.1). Also, a much regular Foley catheter and introduce a lacrimal
more extensive circumferential dissection is done probe in its tip (Fig. 9.6) (photograph). By doing
laparoscopically which is unnecessary. that, we can direct the tip of the catheter conve-
9.4 Surgical Technique 133

niently into the posterior urethra. If this maneu- to avoid any potential injuries. The chest should
ver does not work, we can pass a wire through a be slightly elevated from the table, with rubber
cystoscope, followed by the catheter. Most of the foam (Fig. 9.7) to be sure that the shoulders fall
time, we are successful in placing the catheter in forward to avoid stretching of the brachial
the bladder using one of the two described plexus. The tips of the toes should not rest on the
maneuvers. Occasionally, it is impossible to do table. The penis should be free from the rubber
this and we have to leave the catheter in the rec- foam that is placed in the groin to elevate the
tum. During the operation, when we open the rec- pelvis.
tum, we have the opportunity to redirect the The perineum and perineal area are washed,
catheter toward the bladder under direct vision. prepped, and draped in the usual manner. The
With the Foley catheter in place, the patient is sterile towels are sutured to the skin to avoid their
placed in prone position with the pelvis elevated. displacement during the operation (Fig. 9.8).
The positioning of the patient is the responsibil- Because of the position of the patient, the towels
ity of the surgeon. One must be absolutely sure that surround the operative field tend to fall away,
that all of the pressure areas are well cushioned 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
Fig. 9.6 Lacrimal probe introduced in the tip of a Foley
fine needle-tip cautery is used to avoid excessive
catheter in order to direct it into the bladder and avoid tissue burning. A very sharp needle allows the
getting through the fistula into the rectum surgeon to decrease the intensity of the electric

Fig. 9.7 Prone position


with adequate cushioning
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 9 Rectourethral Bulbar Fistula

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 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

between these two silk stitches. As we identify Most of the catastrophic events that have
the lumen of the rectum, we place 5-0 silk sutures, occurred in cases that were referred to us from
taking the edges of the rectum in each side of the other institutions occurred at this particular stage:
incision (Fig. 9.18). trying to separate the rectum from the urinary
The incision then is extended distally, yet tract without following the basic principles. The
remaining exactly in the midline. As we open first principle is to have a distal colostogram good
with the needle-tip cautery gradually, we keep enough to know that what we are dealing with is
placing 5-0 silk stitches, taking the edges of the indeed the rectum, and we are not blindly explor-
rectal wall. The use of retractors placed on the ing the pelvis of a baby. The most common
rectal mucosa must be avoided, because it trau- source of mistakes in these cases is the lack of a
matizes the tissues, producing edema and bleed- distal colostogram. The surgeons entered looking
ing that may interfere with the visualization of for a rectum that was not there; they actually did
the fistula. The fistula will become evident just by not know the location of the rectum and suffered
continuing the incision in the midline. A last 5-0 terrible misadventures in looking for it. In the
silk stitch is placed, taking the most distal part of process of searching for the rectum, they rather
our incision, including the edge of the fistula site found the urethra, prostate, seminal vesicles, vas
(Figs. 9.19 and 9.20). At this point, we are just deferens, and nerves that must be preserved to
about to start the most delicate part of the avoid neurogenic bladder and impotence. Trying
operation, which is the separation of the rectum to pass a big right-angle clamp around the rectum
from the urinary tract. is a dreadful old, traditional maneuver that must
9.4 Surgical Technique 137

Fig. 9.16 Distal colostogram showing a rectourethral


bulbar fistula

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-
Fig. 9.17 Two silk stitches are placed, taking the poste- ments is a risky business that frequently produces
rior rectal wall, one on each side of the midline serious injuries (Animation 9.1).
138 9 Rectourethral Bulbar Fistula

The next step is to separate the rectum from 6-0 silk stitches taking the rectal mucosa about 2
the urinary tract, being sure that we do not injure or 3 mm above the fistula site, forming a hemi-
any of the important elements of the urinary tract circumference cephalad to the fistula orifice. The
in that area. In order to do that, we place multiple 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
Fig. 9.19 Operative view of the open rectum are not supposed to see the vas deferens or the

a b c

Fig. 9.20 The fistula is found always in the midline at ing the lower edge of the fistula, lacrimal probe introduced
the most distal part of the rectal opening. (a) Arrow show- in the fistula. (c) Diagram
ing the fistula. (b) Last stitch is placed in the midline, tak-
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- feel that we have gained enough length of rectum
tion, until the rectum is completely separated to bring it down to the perineum (Fig. 9.27). It is
from the urinary tract (Fig. 9.25). At that point, rather unusual to have to open the peritoneum
we estimate how much length is necessary in when dealing with rectourethral bulbar fistulas.
order to bring the rectum down to suture it to the Opening the peritoneum is frequently necessary
skin with no tension. Rectourethral bulbar fistula in cases of rectoprostatic fistulas but only rarely
cases require a minimal dissection on the rectum in this defect.
to reach the skin, due to the fact that the rectum is Once we gain enough rectal length, we then
located rather low in the pelvis. This dissection is evaluate whether or not the patient needs tapering
performed in a circumferential manner. of the rectum. This is a maneuver that we have
Uniform traction is applied on all the multi- used very often in the past and we are using less
ple silk stitches, including the 5-0 silk stitches and less now. We believe that this is a manifesta-
that we placed originally when we opened the tion of the fact that now patients are receiving
posterior rectal wall and the multiple 6-0 silk better colostomies (descending colon). An ade-
stitches. We like to say that “traction creates quate colostomy decreases the frequency of
a plane,” and by doing that, we can identify megarectum that we frequently saw in patients
bands that represent the vessels and nerves that that had transverse colostomies and were left
hold the rectum up in the pelvis (Animation many months without a repair. Those patients
9.2). These tension bands are identified as developed a megarectum that later translated into
grooves. The bands must be separated from the severe constipation. We believe that patients are
rectal wall, burned, and divided. These vessels now operated on earlier in life, and they have bet-
must be burned carefully because they have a ter colostomies (not transverse). Irrigating and
tendency to bleed when they retract into the cleaning the distal bowel during the opening of
pelvis (Fig. 9.26). the colostomy result in a collapsed rectum (no
9.4 Surgical Technique 141

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 The fistula is closed with three or four inter-
to have to do tapering of these rectums. It is rupted stitches of 6-0 Vicryl sutures. Those
almost impossible to irrigate and clean a distal stitches take the white fascia that used to cover
rectosigmoid through a transverse colostomy. the rectum and urethra. No special effort is made
When a tapering is necessary, we should taper to suture mucosa to mucosa at the fistula orifice
the rectum to the size of the available space, (Fig. 9.30). In over thousand cases of male
within the limits of the sphincter mechanism. The patients with anorectal malformations operated
posterior aspect of the rectum is resected accord- by us, we have never had a recurrence of a recto-
ingly (Fig. 9.28). One should not taper the ante- urethral fistula.
rior rectal wall as this would leave a suture line The rectum is then placed within the limits of
located against the urethral fistula closure suture the sphincter. The levator muscle is sutured
line, which is a predisposing factor for a fistula together behind the rectum in the midline with
formation. The tapering may include 30–60 % of interrupted 5-0 Vicryl sutures. This can be done
the rectal wall. The posterior rectal wall is with the rectum located up and away from its
repaired with two layers of interrupted 5-0 Vicryl future location to facilitate the visualization of
sutures (Fig. 9.29). the levator muscle (Fig. 9.31) or can be done with
142 9 Rectourethral Bulbar Fistula

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 very difficult to identify these sphincter struc-
rectum is not very bulky. One can see the edge of tures. Fortunately, in cases of rectourethral bulbar
the levator muscle, or one can use the electrical fistula, this sphincter mechanism is easy to iden-
stimulator to be able to see it better. tify. The posterior edges of the muscle complex
We identify the junction between the levator from each side are sutured together in the midline
and the muscle complex at the place where they with interrupted 5-0 Vicryl sutures (Fig. 9.33).
create a 90° angle (Fig. 9.32). Actually, both These stitches take a bite of the posterior rectal
structures (levator and muscle complex) are part wall in order to anchor the rectum in a good posi-
of a continuum of muscle. This angle is less tion, and we think that that helps to prevent pro-
noticeable in cases with a poor sphincter lapse. The limits of the sphincters are electrically
mechanism. determined and marked with temporary 5-0 silk
Some patients with very poor sphincter mech- stitches (Fig. 9.34). The perineal body is recon-
anism (mainly rectoprostatic or recto-bladder structed, bringing together the anterior limits of
neck fistula) simply have no available space the muscle complex and the anterior limits of the
between the levator and the urethra. There is no sphincter in those cases in which our posterior
way to reconstruct the levator behind the rectum. sagittal incision was extended beyond the ante-
Also, in patients with a very flat bottom, and rior limits of the sphincter (Fig. 9.35). The ischio-
therefore very poor sphincter mechanisms, it is 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 9 Rectourethral Bulbar Fistula

a b

Fig. 9.29 The posterior rectal wall is repaired with two layers of interrupted sutures. (a) Before suturing. (b) After
suturing

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
Fig. 9.30 Fine absorbable sutures are used to close the the use of a bulky Foley bag or “urinometer.” In
urethral side of the fistula. Arrow shows the fistula site addition, the skin of the patient’s perineum is bet-
ter protected. We have never seen a case of an
take full-thickness bowel, since sutures placed “ascending urinary tract infection” attributable to
taking only the mucosa do not hold well to the this technique.
skin. The sutures should be tied but not to the We try to leave the catheter in place for one
point of cutting the tissue with excessive force. 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 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
Fig. 9.32 The limits between the levator muscle and the
muscle complex are arbitrarily determined at the place
intraoperative nerve injury. This is prevented by
where both structures form an angle. a angle at the junc- following the basic principles of the operation,
tion of levator muscle complex, L levator, M muscle mainly staying exactly in the midline during the
complex dissection of the rectum and being careful in the

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
9.4 Surgical Technique 147

a b

Fig. 9.34 The limits of the sphincter at the skin level (a) Electrical stimulation to determine the limits. (b) The
have been electrically determined and marked with limits of the sphincter marked with temporary silk stitches
temporary silk stitches, prior to the levator reconstruction.

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
Fig. 9.35 The perineal body was reconstructed, bringing
together the anterior limits of the sphincter. This was done that they cannot see erections after the operation
prior to the pull-through of the rectum can be interpreted in the same way.
148 9 Rectourethral Bulbar Fistula

References
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of congenital rectobulbar fistula. Aust N Z J Surg
40(3):248–252
2. Gupta AK, Bhargava S, Rohtagi M (1986) Anal
agenesis with recto-bulbar fistula. Pediatr Radiol 16:
222–224
3. Currarino G (1994) Imperforate anus associated with a recto-
bulbar-cutaneous fistula. J Pediatr Surg 29(1):102–105
4. Currarino G (1996) The various types of anorectal fistula
in male imperforate anus. Pediatr Radiol 26:512–522
5. Glasier CM, Seibert JJ, Golladay ES (1987)
Intermediate imperforate anus: clinical and radio-
graphic implications. J Pediatr Surg 22(4):351–352
6. Ohno K, Nakamurs T, Azuma T, Yoshida T, Yamada
H, Hayashi H, Masahata K (2008) Anopenile urethral
fistula. Pediatr Surg Int 24:487–489. doi:10.1007/
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closed 7. Shumyle A, Taiwo AL, Peña A, Sheldon C, Levitt MA
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8. Kubota A, Kawahara H, Okuyama H, Oue T, Tazuke
tion and absence of erections) are temporary and Y, Tanaka N, Okada A (2005) Laparoscopically
patients recover, but it is still a very worrisome assisted anorectoplasty using perineal ultrasono-
sign. In patients with very abnormal sacra or graphic guide a preliminary report. J Pediatr Surg
myelomeningocele, one can expect those kinds 40:1535–1538. doi:10.1016/jpedsurg.2005.06.008
9. Lima M, Antonellini C, Ruggeri G, Libri M, Gargano
of problems, but in patients with a normal sacrum, T, Mondardini MC (2006) Laparoscopic surgical
they are considered iatrogenic and therefore treatment of anorectal malformations. Pediatr Med
unacceptable. Chir 28(4–6):79–82
Two weeks after surgery, the baby comes to the 10. Ichijo C, Kaneyama K, Hayashi Y, Koga H, Okazaki
T, Lane GJ, Kurosaki Y, Yamataka A (2008) Midterm
clinic, and we show the parents how to perform postoperative clinicoradiologic analysis of surgery for
anal dilatations. We give them a copy of our pro- high/intermediate-type imperforate anus: prospective
tocol (See Chap. 5) that they are supposed to fol- comparative study between laparoscopy-assisted and
low religiously. Dilatation should not be painful; posterior sagittal anorectoplasty. J Pediatr Surg 43(1):
158–162. doi:10.1016/j.jpedsurg.2007.09.037
they are uncomfortable but not painful if the oper- 11. Rollins MD, Downey EC, Meyers RL, Scaife ER
ation was done correctly. Usually, about 2 months (2009) Division of the fistula in laparoscopic-assisted
after these operations, the colostomy is closed. repair of anorectal malformations-are clips or ties nec-
essary? J Pediatr Surg 44(1):298–301. doi:10.1016/j.
jpedsurg.2008.10.032
12. Podevin G, Petit T, Mure PY, Gelas T, Demarche M,
9.5 Functional Results Allal H, Becmeur F, Varlet F, Philippe P, Weil D,
Heloury Y (2009) Minimally invasive surgery for
Ninety-seven patients were older than 3 years anorectal malformation in boys: a multicenter study.
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voluntary bowel movements. Half of them occa- 13. Yamataka A, Kato Y, Lee KD, Lane G, Kusafuka J,
sionally had marks (soiling) in the underwear. Okazaki T (2009) Endoscopy-assisted laparoscopic
Ninety-two percent of patients are urinary conti- excision of rectourethral fistula in a male with imper-
forate anus. J Laparoendosc Adv Surg Tech 19(Suppl
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problem with constipation that has not been 14. Koga H, Kato Y, Shimotakahara A, Miyano G, Lane
treated well. The use of laxatives frequently takes GJ, Okazaki T, Yamataka A (2010) Intraoperative
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45(2):397–400. doi:10.1016/j.jpedsurg.2009.10.085 and functional results. J Pediatr Surg 48(3):591–596.
15. López PJ, Guelfand M, Angel L, Paulos A, Cadena Y, Escala doi:10.1016/j.jpedsurg.2012.08.001
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for anorectal malformation: is resection of the diverticulum external sphincter for rectourethral fistula: an easier
always necessary? Arch Esp Urol 63(4):297–301 and more physiologic approach. J Pediatr Surg
16. De Vos C, Arnold M, Sidler D, Moore SW (2011) A 48(6):1450–1453. doi:10.1016/j.jpedsurg.2013.03.007
comparison of laparoscopic-assisted (LAARP) and 20. Yamataka A, Yoshida R, Kobayashi H, Lane GJ,
posterior sagittal (PSARP) anorectoplasty in the out- Kurosaki Y, Segawa O, Kameoka S, Miyano T (2002)
come of intermediate and high anorectal malforma- Intraoperative endosonography enhances laparoscopy-
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17. England RJ, Warren SL, Bezuidenhout L, Numanoglu J Pediatr Surg 37(12):1657–1660
A, Millar AJ (2012) Laparoscopic repair of anorectal 21. Koga H, Okazaki T, Yamataka A, Kobayashi H, Yanai
malformations at the Red Cross War Memorial T, Lane GJ, Miyano T (2005) Posterior urethral diver-
Children’s Hospital: taking stock. J Pediatr Surg ticulum after laparoscopic-assisted repair of high-type
47(3):565–570. doi:10.1016/j.jpedsurg.2011.08.006 anorectal malformation in a male patient: surgical
18. Jung SM, Lee SK, Seo JM (2013) Experience with treatment and prevention. Pediatr Surg Int 21(1):
laparoscopic-assisted anorectal pull-through in 25 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 10 Rectourethral Prostatic Fistula

teral reflux in 26 % of cases, and hypospadias in that a surgeon that has experience with the pos-
7.3 % of cases. Undescended testicles were pres- terior sagittal approach can easily and safely
ent in 8 % of these cases. Ten percent of the find the rectum posterior sagittally, separate it
patients had a bifid scrotum. As can be seen, the from the urinary tract, and mobilize the rectum
high frequency of associated urologic defects down safely, provided the colostomy is well
puts this malformation into a category of serious located (not too distal). We also believe that a
defect. The average AP sacral ratio for a patient well-trained laparoscopic surgeon can relatively
with rectoprostatic fistula is 0.55 for patients with easily separate the rectum from the urinary tract
voluntary bowel movements and 0.47 for those via laparoscopy. In other words, we believe that
who are fecally incontinent. The lateral average to decide how to approach these patients is
ratio is 0.64 for continent patients and 0.58 for something that should be done based on the spe-
incontinent patients, which is significantly lower cific circumstances of the surgeon and the
than the sacral ratio of patients with rectourethral patient. Some of the serious catastrophes and
bulbar fistula and significantly higher than the failed attempted repairs that we have seen hap-
ratio for cases with for recto-bladder neck fistu- pening at other institutions precisely occurred in
las. Twenty-two percent of rectoprostatic fistula these types of high prostatic fistulas. In retro-
patients suffer from tethered cord which, again, is spect, the surgeon either did not have a good
a higher incidence than in cases of rectourethral distal colostogram (Animation 10.1) or simply
bulbar fistula. Hemivertebrae occur in 8 % of operated on the patient without a distal colosto-
cases, and they occur mainly in the lumbar spine. gram, entered posterior sagittally looking for a
Esophageal atresia occurs in 14 % of cases and rectum that was located much higher than what
duodenal atresia in 2 % of cases. Patent ductus he thought, could not find the rectum, but rather
arteriosus occurs in 7 % of cases, but only one found structures that he was not looking for,
fourth of them require some sort of intervention such as seminal vesicles, vas deferens, urethra,
due to hemodynamic problems. Atrial septum or during the search, damaged important nerves
defects occur in 8 % of cases but did not require of the urogenital tract, resulting in neurogenic
any type of treatment. Ventricular septum defects bladder, a complication considered totally pre-
occur in 6 % of cases, but only one third of them ventable [4].
required therapeutic intervention. Tetralogy of The perineum of patients with prostatic fistula
Fallot occurred in 2 % of cases. may show signs of what we call bad prognosis.
As previously suggested, the common wall The midline groove may not be so prominent,
located between the rectum and the urethra above and the anal dimple may not be represented by a
the fistula site is shorter in cases of rectoprostatic real fossette but rather by a group of fibers in the
fistula when compared to those of rectourethral midline raphe (Fig. 10.2).
bulbar fistula (Fig. 10.1). This fact makes the We believe that all patients born with recto-
separation of the rectum from the urethra techni- prostatic fistula benefit from a diverting colos-
cally easier. Yet, once the rectum has been sepa- tomy at birth and the malformation must be
rated from the urinary tract, the mobilization repaired in a second operation. One of the main
required in order to pull the rectum down is a arguments in favor of this approach is the fact
more complex and technically demanding that the colostomy, in addition to decompressing
maneuver. the gastrointestinal tract and saving the baby’s
Some of the rectoprostatic fistulas are located life, allows us to perform a good high-pressure
close the bladder neck but not quite into the distal colostogram, which is the only and best
bladder and therefore can be approached by way to provide information about the precise
both a posterior sagittal incision and laparo- location of the rectum and the fistula (Fig. 10.3).
scopically through the abdomen. This particular It is this study that allows us to follow a specific
type of defect represents a matter of controversy strategy during the repair of this malformation
in terms of which approach is better. We believe and to avoid catastrophic complications.
10.4 Posterior Sagittal Anorectoplasty 153

a b

Fig. 10.2 Photograph showing the perineum of two patients with prostatic fistula. (a) prone position. (b) supine position

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
Fig. 10.3 Colostogram (BU and PR) comparing the images
approach is ideal to repair abnormalities of the
of a bulbar fistula with a prostatic. P = Prostatic, B = Bulbar
posterior urethra at the same time than the repair of
the anorectal malformation.
10.3 Surgical Repair The higher the location of the fistula, the more
frequently we may see ectopic ureters. When
Two to four weeks after the colostomy has been these abnormal ureters are ectopically connected
opened, provided the patient is growing and devel- to the posterior urethra, they must be dealt with,
oping normally, the main repair can be performed. during the posterior sagittal approach.
It is not an urgent procedure; if the surgeon is not The patient is positioned prone as previously
familiarized with the anatomy of little babies, he described for the posterior sagittal approach. The
can wait until the baby is bigger or reaches the size posterior sagittal incision runs from the middle
that the surgeon is accustomed to operate on. portion of the sacrum to the anal dimple. We put
154 10 Rectourethral Prostatic Fistula

special emphasis in making the incision exactly fistula with a high rectum. The rectum in patients
midline using the electrical stimulator to try to with rectoprostatic fistula is found to be much
leave equal amounts of muscle in both sides of smaller than in cases with bulbar fistula.
midline. We go through the skin, subcutaneous Unfortunately, the appearance of the white fascia
tissue, parasagittal fibers, ischiorectal fossa, and after we have divided the levator muscle does not
levator mechanism (see Chap. 9, Sect. 9.4). Once allow us to determine or predict where the rectum
we divide the levator mechanism, we have to is going to be found. The distal colostogram is the
keep in mind the image of the distal colostogram main guideline that we should follow. Two silk
to determine where to exactly look for the rec- stitches are placed as high as possible on the pos-
tum. Figure 10.4 shows a diagram of rectobulbar terior aspect of what we think is the rectum in
and rectoprostatic fistula. In general, in patients front of the coccyx or above the coccyx assuming
with rectoprostatic fistula, the surgeon must look that we are holding on the rectum (Fig. 10.5).
for the rectum immediately below the coccyx. In Using traction on these silk sutures, we can divide
those particular cases where the rectum seems to the white fascia that covers the rectum, as well as
be located a little higher, close to the bladder the perirectal fat, bands, and vessels located
neck, we have to look for the rectum above the deeper than the white fascia. By doing this, we
coccyx. This is extremely important because the notice that the rectal wall gives up, and we can
possibility of producing extra damage to the uro- mobilize it lower (Fig. 10.6). We continue the
genital tract increases in cases of rectoprostatic dissection, staying as close as possible to the

Fig. 10.4 Diagrams showing a bulbar and a prostatic fistula


10.4 Posterior Sagittal Anorectoplasty 155

Fig. 10.5 Finding the rectum immediately under the Fig. 10.7 The rectum is open and the fistula identified
coccyx

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
Fig. 10.6 Pulling the rectum down see the white fascia and the extrinsic blood sup-
156 10 Rectourethral Prostatic Fistula

Fig. 10.8 Further rectal mobilization to see the lumen Fig. 10.10 The rectum is already separated from the
and the fistula better. Arrow showing the fistula 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-
Fig. 10.9 Sutures placed in the upper circumference of tum up in the pelvis. The dissection is performed
the fistula in a systematic circumferential manner dividing
10.4 Posterior Sagittal Anorectoplasty 157

Fig. 10.11 The rectum fully mobilized Fig. 10.12 Electrical stimulation to determine the limits
of the sphincter. Stimulator touching the posterior limit of
the sphincter in one side
those bands. Dividing bands and vessels allows
gaining length, which allows us to see new bands
and vessels previously unrecognized. Sometimes, to gain length, and yet the rectum survives.
all what we can see is a groove, which represents Sometimes we see some duskiness of the rectum,
a tense band. We grab those vessels, separate but provided the bowel wall was not damaged,
them from the bowel wall, and burn them, putting we know that we did not interfere with the intra-
special emphasis on not burning the bowel wall. mural blood supply, and therefore the rectal
Very soon, the peritoneal reflection is identified blood supply will be good, and the rectum will
and opened, which allows mobilizing the rectum survive.
even more. The dissection continues until the rec- The limits of the sphincter are determined
tum is mobilized enough to be anastomosed to with an electrical stimulator and are temporarily
the anal dimple with no tension (Fig. 10.11). marked with 5-0 silk stitches (Figs. 10.12, 10.13,
Another very important piece of information 10.14, and 10.15). The perineal body, in those
that we obtain from the distal colostogram is the cases in which it was opened, is reconstructed,
amount of bowel available between the distal bringing together the anterior limits of the
stoma and the end of the bowel. In other words, sphincter (Fig. 10.16). The rectum is placed in
we want to be sure that we are not dealing with a front of the levator and within the limits of the
patient who has a defective colostomy with a muscle complex. Figure 10.17 shows a fully
very short piece of rectum attached to the abdom- reconstructed perineal body.
inal wall that interferes with the pull-through. In this type of malformation, we began to see
We have been happily surprised in dealing a significant degree of what we call “caudal
with patients with rectoprostatic fistulas; we dis- regression.” We see, for instance, that the levator
sected and divided all the extramural blood sup- muscle is located much deeper in our incisions
ply of a very impressive length of bowel in order than in cases of rectourethral bulbar fistula. This
158 10 Rectourethral Prostatic Fistula

Fig. 10.15 The rectum will be located between the four


sutures

Fig. 10.13 Stimulator touching the anterior limit of the


opposite side

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
Fig. 10.14 Limits of the sphincter were determined and suture the levator muscle behind the rectum
marked with temporary stitches 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 10 Rectourethral Prostatic Fistula

Fig. 10.20 Beginning the anoplasty

Fig. 10.22 Anoplasty finished. Holding sutures in place.


Open anus

Fig. 10.21 Trimmed off one side of the extra rectum

absorbable sutures (Fig. 9.29, Chap. 9). As we Fig. 10.23 Anoplasty finished. No holding sutures. Anus
closed
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 the distal rectum and dilatation of it. In addi-
that was a consequence of two facts: first, a tion, when a proximal (ascending or transverse)
colostomy that was opened too proximal, it colostomy is created, the surgeon cannot
tends to produce a megarectum. We believe that remove the meconium accumulated in the distal
is consecutive to the desquamation of mucosal colon. The other fact that contributes to the for-
cells as well as mucus production and persistent mation of a megarectum is the long period of
peristalsis; all this produces an accumulation in time elapsed between the opening of the colos-
References 161

tomy and the main repair. Nowadays, fortu- Long-term follow-up of these patients, in our
nately surgeons are opening better colostomies hands, indicates that roughly 66 % of the patients
(descending, with separate stomas) that allow after 3 years of age have voluntary bowel move-
them to clean the distal bowel from day 1, and ments provided they had a good sacrum. However,
they are also performing earlier and earlier 80 % of them suffer from occasional soiling of
repairs that avoid the great number of cases of the underwear. This means that we are dealing
megarectum that we have seen in the past. with a much more serious condition as compared
The operations to repair rectoprostatic fistulas with bulbar fistula. Ninety-two percent of these
take more time than rectourethral bulbar fistula patients had urinary control.
cases because of the mobilization of the rectum. The sacral ratio seems to have a definite influ-
On the other hand, we must keep in mind that in ence on the functional prognosis for bowel con-
cases of rectourethral bulbar fistula, the common trol, since 65 % of the patients with a ratio higher
wall between both structures (rectal and urethral) than 0.7 had voluntary bowel movements,
is longer, and therefore it requires more time to whereas only 35 % of them had voluntary bowel
separate the rectum from the urethra. movements when their sacral ratio was lower
than 0.4. All patients with sacral ratio higher
than 0.7 were urinary continent, whereas only
10.5 Postoperative Care and 35 % of them were continent when their ratio
Functional Results was lower than 0.4.

These patients usually eat the same day of sur-


gery even when we open the peritoneum. Again, References
we have been impressed by the fact that these
operations are not particularly painful and the 1. Peña A (1988) Posterior sagittal anorectoplasty:
patients do not need morphine postoperatively. results in the management of 332 cases of anorectal,
malformations. Pediatr Surg Int 3:94–104
We like to administer intravenous antibiotics for
2. Endo M, Hayashi A, Ishihara M, Maie M, Nagasaki
48 h and keep the Foley catheter for one entire A, Nishi T, Saeki M (1999) Analysis of 1,992 patients
week. The patient remains in the hospital 48 h with anorectal malformations over the past two
after the operation. One week after surgery, the decades in Japan. Steering Committee of Japanese
Study Group of Anorectal Anomalies. J Pediatr Surg
patient comes to our clinic in the early morning
34(3):435–441
to have the Foley catheter removed and then 3. Rintala RJ (1996) Anorectal malformations—man-
remains in the clinic a couple of hours so that we agement and outcome. Semin Neonatol 1(3):219–230.
can be sure the patient is voiding with no diffi- doi:10.1016/S1084-2756(96)80040-6
4. Hong AR, Acuña MF, Peña A, Chaves L, Rodriguez
culty before going home. Two weeks after sur-
G (2002) Urologic injuries associated with repair of
gery we start the process of anal dilatations anorectal malformations in male patients. J Pediatr
following our specific protocol (see Chap. 18). 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 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
Fig. 11.3 Cystoscopic aspect of a verumontanum located arteriosus, 5 % with tetralogy of Fallot, 2 % with
at the trigone tricuspid atresia, and 2 % with pentalogy of
Fallot.

A serious, not previously known anomaly


found in 45 % (15 cases) of those patients in
whom we performed a cystoscopy was an ecto- 11.2.7 Other Associated Defects
pic verumontanum. Six of them were located
in the trigone (Fig. 11.3), five in the bladder Five percent of these patients suffer from hand
neck, and four immediately below the bladder abnormalities and 5 % from lower extremity
neck. Based on this experience, we now con- abnormalities (equinovarus).
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 11.3 Diagnosis
and orgasms but they ejaculate into the blad-
der. Theoretically for these patients to have We must suspect the presence of these very
children, it will require special maneuvers to complex malformations when we see a new-
retrieve the sperm from the urine followed by born baby with imperforate anus and flat
artificial insemination. bottom. The midline groove in between the
When the clinician makes the diagnosis of the buttocks that we see in normal children is not
recto-bladder neck fistula type of malformation, present. In addition, we frequently see in these
he/she should be aware of the fact that he/she is patients a sphincter mechanism located right at
dealing with a patient with a potential serious the base of the scrotum (Fig. 11.4). The
urologic condition. presence of a bifid scrotum (Fig. 11.5) also
suggests that the malformation that the baby
has is rather complex, most likely very high
11.2.4 Gastrointestinal-Associated (recto-bladder neck fistula). The diagnosis is
Defects confirmed with a distal colostogram and
subsequently by cystoscopy that is performed
The incidence of esophageal atresia is 15 %, at the time of the main repair. The distal colos-
Meckel’s diverticulum 2 %, and abdominal wall togram can be performed after the colostomy is
defects 4 %. 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 167

associated defects that interfered with his growth baby is born. However, as we previously men-
and development, then the patient can be oper- tioned, many of these patients come to our insti-
ated, at our institution, within a month after the 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
Fig. 11.7 Distal colostogram showing a good length of repair.
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
168 11 Recto-bladder Neck Fistula

The fact that the patient has a recto-bladder open in the bladder neck and provoke either vesi-
neck fistula means that we have to go into the coureteral reflux, ureterovesical obstruction, or
abdomen either by laparotomy, laparoscopy, or urinary incontinence. When the ureter is ectopic
both, in addition to the posterior sagittal approach. and located into the posterior urethra, usually it is
We start the operation by putting the patient in associated with a severe stricture, megaureter,
the lithotomy position and performing a cystos- and severe renal damage (Fig. 11.2). It is com-
copy. The cystoscopy is extremely valuable. We mon for these patients to end up with a nephrec-
have been learning a great deal about the anat- tomy. The location of the fistula is frequently
omy of the male urethra, bladder neck, and tri- visualized at the bladder neck with the cysto-
gone in these patients. As previously mentioned, scope. A Foley catheter is placed in the bladder.
it is not uncommon to find that these patients
have no verumontanum located in the posterior
urethra. Rather than that, we find the verumonta- 11.4.3 Laparotomy
num located in the trigone. In retrospect, now we
have an explanation for the adult patients that A total body preparation is performed on these
were born with these kinds of defects and have no patients (Fig. 11.9). This means to wash, prep,
ejaculation. Further studies demonstrate that they and drape both lower extremities, the perineum,
actually ejaculate in the bladder. This must be buttocks, perianal area, lower abdomen, and lum-
differentiated from the concept of retrograde bar portion; in other words, the entire lower body
ejaculation. We are referring to a patient that has is included in the sterile field. The cautery plate is
the verumontanum located in the trigone and placed in the back of the patient and is protected
ejaculates directly into the bladder. This is dem- with a plastic drape. The arms of the patient are
onstrated later in life, by finding sperm in the placed in the upward position because they belong
urine after an ejaculation, as well as the cystos- to the nonsterile part of the field. Both legs of the
copy (Fig. 11.3). It is not unusual in this type of patient are covered with stockinettes or with an
patients to find, also, ectopic ureters. We have elastic bandage to avoid loss of temperature. The
learned that the higher the malformation, the proximal stoma of the colostomy is packed with
more chances of the patient to have ectopic ure- packing gauze impregnated with an antiseptic
ters. The ectopia in this type of defect usually solution to avoid contamination. The skin of the
means that the ureters are located closer to the abdominal wall is covered with a plastic drape.
bladder neck or even below the bladder neck into We can start the operation either from below
the posterior urethra. Sometimes, the ureters or through the abdomen. We more often now

a b

Fig. 11.9 Total body preparation – diagram. (a) Supine. prep the entire body below the chest. (e) Wash and prep
(b) Legs up. (c) Sequence of photographs of bowel prepa- the back. (f–h) Sterile sheets on table. (i, j) Covering sto-
ration. (a) Holding legs up. (b) Cautery plate up in the mas (k) Foley catheter inserted
back. (c) Packing gauze in proximal stoma. (d) Wash and
11.4 Treatment 169

c
ca cb

cd

cc

cf

ce

ch

cg

Fig. 11.9 (continued)


170 11 Recto-bladder Neck Fistula

ci cj

ck

Fig. 11.9 (continued)

approach the abdomen first which can be done by Both vas deferens seen behind the bladder run
laparotomy or laparoscopy. We consider this par- distally toward the bladder neck; the ureters are
ticular defect to be a good indication for a laparo- seen retroperitoneally, and they also seem to be
scopic approach. More information related with running toward the bladder neck. Our specific
the indications for laparoscopy in anorectal mal- recommendation is to place a 4-0 silk stitch on
formations can be found in Chap. 13. The abdo- the anterior wall of the sigmoid to apply traction.
men is entered through a midline incision running About 1 or 2 cm from the peritoneal floor, the
from the umbilicus down to the pubis. A needle- serosa of the anterior wall of the sigmoid is
tip cautery is used changing from cutting to coag- divided in order to create a plane of dissection as
ulation to provide meticulous hemostasis. The close as possible to the bowel wall, but without
peritoneal cavity is entered. The urachal remnant damaging it. This plane of dissection is followed
and obliterated umbilical arteries are identified all around the bowel, separating the mesenteric
and divided. A clamp is placed on the urachal fat from the sigmoid. Once we have created a
remnant of the bladder to apply caudal traction. plane all around the bowel, a Silastic vessel loop
The lateral avascular attachments of the bladder is passed around the rectum in order to have a
to the abdominal wall are divided with cautery to more effective handle for traction (Fig. 11.11a).
have easy access to the lower pelvis. By pulling Applying traction on the vessel loop, it is very
on the bladder out of the abdomen and toward the easy to continue a circumferential dissection of
pubis, caudally, we can see the posterior wall of the bowel distally. Very soon, within a centimeter
the bladder as well as the peritoneal floor, sig- or two from our initial dissection, one can appre-
moid, both vas deferens, and ureters (Fig. 11.10). 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 173

c
a

Fig. 11.14 Diagram showing the blood supply of a rec-


tosigmoid in a case with a colostomy and ligated vascular
colonic arcade
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 when they were located very high (Figs. 11.15
systems
and 11.16).
When we separate the bowel from the bladder
supply, which allows sacrificing all of its extrin- neck, as previously mentioned, we divide the
sic vessels without compromising its vascularity avascular attachments of the bowel in order to
provided the bowel wall is maintained intact and identify the mesenteric vessels. It is very easy to
the inferior mesenteric vessels are not ligated. In pull on the bowel and identify exactly what is
other words, we can ligate several peripheral limiting the pull-through. At the beginning, one
branches of the inferior mesenteric vessels, can see that what is limiting us are the vessels,
being sure to preserve at least one or two proxi- and we can selectively ligate the peripheral
mal branches (Fig. 11.15 and Animation 11.3). branches of the mesenteric vessels as close as
All this, provided we maintain intact the integ- possible to the bowel and see how we gain more
rity of the rectal wall. Damaging the rectal wall and more length until we have the necessary
interferes with the intramural blood supply, and length for the pull-through. However, occasion-
the distal blood supply suffers. Every time we ally in the process, we find that we are no longer
ligate one of the peripheral branches of the infe- limited by the vessels but rather limited by the
rior mesenteric vessels, we do it very close to the colostomy itself. Under those circumstances, we
rectal wall, and that allows us to gain length. We must take the colostomy down. To do that, mul-
must be sure to visualize that at least one good tiple 5-0 silk stitches are placed at the mucocuta-
branch from the inferior mesenteric vessels neous junction of the mucous fistula. If the patient
remains intact, reaching the bowel wall and that has a loop colostomy (which we consider
the bowel wall remains intact; by doing that, the contraindicated in anorectal malformations),
bowel blood supply is going to be good. then, unfortunately, we had to take down the
Following those recommendations, we have entire colostomy, which makes the procedure
been able to pull down all of these rectums, even 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 opened yet, we can guess whether or not there is
the mucous fistula. By doing that, we may have enough length by pulling the bowel outside of the
enough length of bowel to reach the perineum. abdomen caudally toward the genitalia. We have
We can go ahead with the pull-through and decide learned that we have enough distal bowel to reach
whether to leave the upper part of the pulled- the perineum if the distal end of the bowel reaches
through bowel closed, as what is called about 4 cm below the lower edge of the pubic
“Hartmann pouch,” or to close the colostomy and bone (Fig. 11.16). If we do not have this kind of
do the pull-through, leaving the patient without a length, that means that we have to work more on
protective colostomy and a colonic anastomosis the blood supply or to take down the colostomy
in the pelvis (Fig. 11.19). If one decides to leave in order for the bowel to reach.
it as a “Hartmann pouch,” we want to be sure that The perineal approach can be done in two
the length of the distal bowel is enough for the ways. One is simply lifting the legs up, putting a
blind upper end of the bowel to be found above bulky roll below the pelvis of the patient. By
the peritoneal reflection at the time of the colos- doing that, the perineum of the patient is well
tomy closure; otherwise, it may become a techni- exposed, horizontally, and we can work comfort-
cally demanding type of procedure. ably (Fig. 11.17c). The incision that we make in
In order for us to learn whether or not there is these patients does not have to be a full-length
enough length of colon for the pull-through, we posterior sagittal one. An incision that runs from
can open from below and see exactly if we have the base of the scrotum and about 5 or 6 cm
enough length passing the rectum behind the pos- posteriorly usually provides plenty of exposure
terior urethra. If the perineum has not been to create a safe abdominal perineal path. Our
11.4 Treatment 175

a b

Fig. 11.16 How to gain length in a case of a very high Blood supply of the rectum is provided by intramural ves-
rectum. (a) Diagram – divide peripheral branches of the sels. (b, c) Intraoperative picture of the same maneuver.
inferior mesenteric vessels. Maintain intact rectal wall. (d) Bowel reaches the perineum

incision goes through the skin, parasagittal making a very difficult task to accommodate a
fibers, muscle complex, and levator mechanism. rectum through it. The entire procedure is done
However, these patients often have very poor with a Foley catheter in place. In the process of
sphincter mechanism, and sometimes it is very opening the perineum, we frequently stop to pal-
difficult to identify each one of the components pate the catheter in the urethra located in the
of the sphincter mechanism. In addition, we find deepest portion of the “V” formed by the pubic
different degrees of “caudal regression.” This bones. As we progress deeper through the poste-
means that the pelvis may be extremely narrow, 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 177

d e

Fig. 11.17 (continued)

entire striated sphincter mechanism, we find a within the limits of the sphincter as previously
whitish fascia which represents the entrance to demonstrated in the other chapters. Sometimes,
the abdominal cavity. A safety path is obtained in these operations, we find a minimal amount of
remaining as much as possible in the midline. a sphincter mechanism, and therefore, the loca-
Once we enter into the abdomen, we pass a tion of the anus is determined in a rather arbi-
clamp to grasp the distal end of the bowel when trary way. Many of these patients will have a
dealing with a laparoscopic approach or to grasp poor prognosis anyway, due to the lack of a
the sutures holding the distal rectum when the sphincter, poor sacrum, tethered cord, and other
abdomen was opened (Fig. 11.17). Looking spinal abnormalities.
from the abdominal side, we must remain away Prior to the abdominal closure, we must close
from the ureters and the vas deferens. This space the defect created between the mesentery of the
has to be wide enough to avoid compression of pulled bowel and the posterior abdominal wall
the rectum. The rectum then is pulled under (Fig. 11.18). We had experience with two cases in
direct vision, and the anoplasty is performed whom that space was left open and the patients
178 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
Fig. 11.18 Closing the mesenteric defect pain produced by the incision in the abdomen.
Because of this, the laparoscopic approach is
indicated to treat this particular malformation.
suffered from intestinal obstruction within the The exposure and view of the peritoneal floor
first 5 days postoperatively. They required an obtained laparoscopically is excellent (see Chap.
emergency laparotomy to reduce multiple loops 13). The dissection of the distal rectum is easily
of small bowel which were trapped in that defect. done, as previously described, until the rectum
Also, before we pull the colon through, we becomes narrow. At that point, unfortunately, the
evaluate the degree of dilatation of the rectum; if division of the fistula cannot be done as accu-
it is considered to be too bulky, it must be tapered rately as when it is done with a laparotomy. Yet,
resecting a portion of the posterior wall of the we have not seen complications from the laparo-
rectum and closing with two layers of interrupted scopic ligation of the fistula. It is important to
sutures. keep in mind that in this particular type of ano-
The posterior wall of the rectum must be rectal malformation (recto-bladder neck fistula),
anchored to the neighbor tissue with 5-0 long- the rectum reaches the bladder neck in a “T”
term absorbable sutures. If the patient gets a good fashion. In other words, there is no common wall
muscle complex, the rectum is anchored to the between the rectum and the urinary tract located
muscle complex as demonstrated in other malfor- above the location of the fistula, like it happens in
mations. The anoplasty is performed as previ- the cases of prostatic fistula and even more in
ously described for other malformations. cases of rectal urethrobulbar fistula. Our observa-
Another way to do the posterior sagittal por- tions in 1,113 surgical repairs of anorectal mal-
tion of the operation consists in packing the formations in male patients allowed us to learn
abdominal wound, covering it with a plastic that the lower the malformation, the longer the
drape, turning the patient into prone position, and common wall between the rectum and the urinary
opening posterior sagittally as previously tract. Therefore, in the highest of all defects
described. However, more and more, we do not (recto-bladder neck fistula), the dissection of the
11.6 Functional Results 179

rectum is easier, and it does not include the risk bowel and take the proximal stoma down as a pull-
of injuring the urinary tract. In lower malforma- through. In general, we do not like to do this
tions such as rectoprostatic and particularly in because that means the patient will lose its natural
bulbar fistula, the common wall between the rec- bowel reservoir which will give him a tendency to
tum and urinary tract is much longer, and there- have diarrhea, making the bowel management to
fore, it is not that easy simply to ligate the fistula keep him artificially clean more difficult. Another
like in the laparoscopic approach in cases of possibility would be to take the proximal stoma,
recto-bladder neck fistula. That is one of the rea- separate it from the abdominal wall, close the
sons why we consider the laparoscopic approach colostomy, and pull together down to the perineum,
formally contraindicated in patients with recto- the distal bowel with what used to be the proximal
urethral bulbar fistulas. stoma attached and anastomosed. If we do some-
The separation of the rectum from the bladder thing like that, we have to make a decision to (a)
neck and the ligation of the fistula are easy maneu- keep the patient postoperatively without a colos-
vers. The mobilization of the rectum and the cau- tomy, with parenteral nutrition, and nothing by
terizing of the vessels to allow the rectum to reach mouth for 10 days or (b) open a more proximal
the perineum without undue tension, on the other colostomy (Fig. 11.20). The decision is a clinical
hand, may not be so easy. The burning of selected one and will depend on how secure the surgeon
mesenteric vessels may not be as accurate as when feels about the blood supply of the distal rectum
it is done with an open abdomen; accidental burn- and the surgical technique observed in general. We
ing of important neighbor vessels may occur. In have only removed one rectum in cases like this,
addition, it is not uncommon to find that the rec- because the patient had only a 4 cm portion of
tum is too bulky to be placed within the limits of bowel, and if we anastomosed it to the proximal
the sphincter. A tapering of the rectum is required stoma, the anastomosis would be located too low.
in such cases. It is at this point when sometimes We felt that it was an unnecessary risk to do that
we have decided not to continue the laparoscopic and preferred to pull down the colostomy itself.
approach and go into to a formal laparotomy. In When we did that, we had to keep the patient
some cases, we have concluded the entire proce- 10 days with nothing by mouth or to open a more
dure laparoscopically successfully. proximal colostomy. We almost never open a
proximal colostomy. We have, rather, kept the
patient with nothing by mouth for 10 days.
11.5 Special Problems Another reason to open the abdomen even in
cases of prostatic or bulbar fistulas is when the
11.5.1 Dealing with Inadequate colostomy interferes with the pull-through of the
Colostomies (Too Distal) rectum because this has been created too distally.
We have seen this happening very often.
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 11.6 Functional Results
blood supply. We have learned how to do it, but
sometimes the upper part of the rectum has to be Our experience includes 110 patients. The func-
detached from the abdominal wall in order to be tional evaluation is only done in patients older than
pulled down. Once the pull-through is completed, 3 years of age and that have been in touch with us.
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 11.6.1 Fecal Control
impossible task to close the colostomy (Fig. 11.19).
At that point, we have to make a decision. One Forty-seven patients were evaluated after the age
possibility would be to resect the distal piece of of 3, and we found that 12 of them (25 %) had
180 11 Recto-bladder Neck Fistula

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 0.4–0.69, and 18 of them (81.8 %) had urinary
these patients soiled occasionally in the underwear. control. When the sacral ratio was less than 0.4,
Only 10 % were totally continent. All patients that three out of 13 patients (23 %) had urinary con-
received bowel management were kept totally trol. The fact that a significant number of patients
clean in the underwear. Among 21 patients with have urinary control does not mean that the uri-
sacral ratio of 0.7 and up, 7 (33.3 %) had voluntary nary tract is working properly. Some patients
bowel movements. Patients with sacral ratio of have urinary control but cannot empty the blad-
0.41–0.69, (20 %) had voluntary bowel move- der well. In addition, a significant number of
ments. None of the patients with a sacral ratio cases with this malformation suffer from vesico-
lower than 0.4 had voluntary bowel movements. ureteral reflux. That explains why a significant
number of patients 17/54 (31 %) are treated with
clean intermittent catheterization.
11.6.2 Urinary Control We consider it extremely important to alert
and to warn the parents about the future of these
Forty-nine patients were available to evaluate uri- babies as soon as we make the diagnosis of recto-
nary control. Thirty-nine of them (78 %) had uri- bladder neck fistula. This is extremely important
nary control. Among 19 patients with sacral ratio in order to adjust the expectations of the parents
higher than 0.7, 13 (68.4 %) had urinary control. concerning the future of the baby and to avoid
Twenty-two patients had a sacral ratio of further frustration.
References 181

References
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of anorectal malformations. Am J Surg 180:370–376
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reconstruction of high anorectal anomalies. Pediatr
Endosurg Innov Tech 2:5–11
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high imperforate anus—a new technique. J Pediatr
Surg 35:927–931
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laparoscopy-assisted anorectal pull-through for high
imperforate anus. J Pediatr Surg 36:1659–1661
5. Borzi PA, Mackay AJ, Lander MM (2001)
Laparoscopic assisted abdominoperineal pullthrough
for anorectal agenesis (LAAP)—early experience.
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6. Takehara H, Tashiro S, Ishibashi H (2001)
Laparoscopic surgery for congenital colorectal dis-
ease in children. Pediatr Endosurg Innov Tech 2001:5
7. Behamou EM (2001) Anorectal malformation: treat-
ment by laparoscopy. Pediatr Endosurg Innov Tech
5:209–213
8. Yamataka A, Yoshida R, Kobayashi H (2002)
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9. Sydorak RM, Albanese CT (2002) Laparoscopic
repair of high imperforate anus. Semin Pediatr Surg
11:217–225
Fig. 11.20 Diagram showing a pulled-through short 10. Iwanaka T, Arai M, Kawashima H (2003) Findings of
piece of rectum with a colostomy closure and opening of pelvic musculature and efficacy of laparoscopic mus-
a more proximal colostomy cle stimulator in laparoscopy-assisted anorectal pull-
through for high imperforate anus. Surg Endosc
17:278–281
Once we make the diagnosis of this malfor- 11. Raghupathy RK, Moorthy PK, Rajamni G (2003)
mation, we tell the parents what we know about Laparoscopically assisted anorectoplasty for high
the future bowel function of the baby, and at the ARM. J Indian Assoc Pediatr Surg 17:278–281
12. Kudou S, Iwanaka T, Kawashima H (2005) Midterm
same time, we tell them that we will always be
follow-up study of high-type imperforate anus after
there to help them. We offer them our bowel laparoscopically assisted anorectoplasty. J Pediatr
movement program to be started when the Surg 40:1923–1926
patient is 3 years old in order for the patient to 13. Kubota A, Kawahara H, Okuyama H (2005)
Laparoscopically assisted anorectoplasty using peri-
go to school like a normal child with normal
neal ultrasonographic guide. A preliminary report. J
underwear and to be adapted and accepted into Pediatr Surg 40:1535–1538
the society. 14. Lima M, Tursini S, Ruggeri G (2006) Laparoscopically
When the patient has vesicoureteral reflux, assisted anorectal pull-through for high imperforate
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we leave a suprapubic cystostomy tube at the
Surg Tech 16:63–66
time of the main repair. Prior to the colostomy 15. Hakguder G, Ates O, Caglar M (2006) A unique
closure, we perform a suprapubic cystogram to opportunity for the operative treatment of high ano-
determine the presence and magnitude of rectal malformations: laparoscopy. Eur J Pediatr Surg
16:449–455
the reflux. Also, a urodynamic evaluation will
16. Lima M, Antonellini C, Ruggeri G (2006)
help to determine the best urologic future Laparoscopic surgical treatment of anorectal malfor-
management. mations. Pediatr Med Chir 28:79–82
182 11 Recto-bladder Neck Fistula

17. Vick LR, Gosche JR, Boulanger SC (2007) Primary 25. El-Debeiky MS, Safan HA, Shafei IA (2009) Long-term
laparoscopic repair of high imperforate anus in neona- functional evaluation of fecal continence after laparo-
tal males. J Pediatr Surg 42:1877–1881 scopic-assisted pull-through for high anorectal malfor-
18. Georgeson K (2007) Laparoscopic-assisted anorectal mations. J Laparoendosc Adv Surg Tech 19:S51–S54
pull-through. Semin Pediatr Surg 16:266–269 26. Hay SA (2009) Transperineal rectovesical fistula
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laparoscopic approach. Eur J Pediatr Surg. doi:10.105 tal malformations—are clips or ties necessary?
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postoperative clinicoradiologic analysis of surgery for assisted laparoscopic excision of rectourethral fistula
high/intermediate-type imperforate anus: prospective in a male with imperforate anus. J Laparoendosc Adv
comparative study between laparoscopy-assisted and Surg Tech 19:S241–S243
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43:158–163 challenges of the laparoscopic approach for patients
23. Grapin-Dagorno C, Fayad F (2008) Surgical treat- with anorectal malformation and rectobladderneck
ment of high-type imperforate anus: role of laparos- fistula. J Laparoendosc Adv Surg Tech 19:264–291
copy. Bull Acad Natl Med 192:913–918 31. Al-Hozaim O, Al-Maary J, AlQahtani A (2010)
24. Ramanujam TM, Yik YI, Shitasanan N (2008) Laparoscopic-assisted anorectal pull-through for ano-
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Presented at the 15th Annual Meeting of the J Pediatr Surg 45:1500–1504
International Pediatric Colorectal Club. Salamanca,
Spain, June 2008
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 Imperforate Anus Without Fistula in Males and Females

a b

Fig. 12.2 Distal colostogram done in a patient without a (b) Increased hydrostatic pressure allows us to see the real
fistula. (a) The lack of hydrostatic pressure gives the false location of the blind end of the bowel
impression that the rectum ends at the pubococcygeal line.

that those series included many misdiagnosed forate anus with no fistula, dividing the cases into
cases, in whom a fistula was not demonstrated “high” and “low” location of the rectum [6].
due to a lack of adequate imaging technology. In our series of 83 cases, 76 of them had the
Even at present time, we see cases that come to rectum located at the level of the bulbar urethra.
our center with a diagnosis of anorectal malfor- Five cases had the blind rectum located at the
mation “without fistula,” based on a distal colos- level of the prostatic urethra, and we have only
togram done with an inadequate technique (lack seen two blind rectums, floating into the
of hydrostatic pressure). When we repeat the dis- peritoneum [7]. That is an extremely unusual
tal colostogram, applying enough hydrostatic defect, and we believe it belongs to another cate-
pressure, a fistula is demonstrated (Fig. 12.2). gory. This is a debatable issue [8].
Some authors suggest that the absence of fis-
tula may contribute for these patients to suffer
12.2 Anatomic Characteristics from colon perforation earlier than those with fis-
tula [9]. The diagram in Fig. 12.1 shows that the
Figure 12.1 shows a diagram of an imperforate rectum and the posterior urethra share a long
anus with no fistula. Interestingly, the blind end common wall. This means that the repair of this
of the rectum is located at the same level of the malformation is not necessarily easier than the
bulbar urethra in the overwhelming majority of one of a rectourethral fistula since the surgeon
cases. In the past, the literature referred to imper- has to work in that common wall and separate the
12.3 Main Repair 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
Fig. 12.3 The white fascia. Deeper to the levator mecha-
continue opening the rectum distally to reach
nism, a white fascia is seen which covers the posterior
rectal wall. Arrow shows the white fascia its blind end, to confirm that there is no fistula.
As we open the posterior rectal wall, 5-0 silk
rectum from the urinary tract preserving intact stitches are placed taking the edges of the rec-
the last one. 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.
12.3 Main Repair The surgeon must remember at this point that the
anterior rectal wall is intimately attached to the
The posterior sagittal approach represents the posterior urethra and will require a meticulous
ideal way to repair this malformation [1, 7, 10]. dissection in order to separate the rectum with-
A Foley catheter is inserted in the bladder. The out injuring the urinary tract. For that, multiple
patient is turned into the prone position, with the 5-0 silk stitches are placed taking the edge of the
pelvis elevated. The skin of the perineum and rectum in its lowest portion, and the dissection is
both buttocks are washed, prepped, and draped done using uniform traction. Applying uniform
in the usual manner. A midline incision is done traction with multiple silk stitches, a circumfer-
with the needle-tip cautery running from the ential dissection is performed dividing bands
middle portion of the sacrum down to the base of and vessels that held the rectum up in the pel-
the scrotum. The incision divides the skin, sub- vis (Fig. 12.5). Since the rectum is located sig-
cutaneous tissue, parasagittal fibers, and muscle nificantly low, once it is completely separated,
complex. After the parasagittal fibers have been usually it requires a rather minor dissection to
divided, we go through the ischiorectal fossa to gain enough length to achieve a low-tension ano-
find the levator mechanism. When the levator plasty. In this particular malformation, it is not
muscle is divided, a white fascia appears very uncommon to find a very dilated rectum since the
clearly (Fig. 12.3). This white fascia must be patient had no fistula. Therefore, the chances for
divided and separated from the real bowel wall. 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- posterior rectal wall to anchor the rectum in a
tions. This is particularly true in those patients good position. The anoplasty is performed in the
who have spent a long time, from the time of center of the sphincter with 16 circumferential
colostomy until the time of the main repair. If stitches of 6-0 Vicryl sutures. The ischiorectal
the rectum is too bulky, it must be tapered, and fossa is obliterated with the same suture mate-
for that, we resect the posterior aspect of the rial as well as the subcutaneous tissue, and the
rectal wall and closed the rectum with two lay- skin is closed with a subcuticular monofilament
ers of interrupted long-term absorbable sutures absorbable suture. These patients require mini-
(Fig. 12.6). The rectum is then placed in front mal medication for pain. They can eat and drink
of the levator mechanism, which is closed with the same day as surgery. The Foley catheter can
5-0 interrupted long-term absorbable sutures. be removed the next day. Dilatations are started
The posterior edges of the muscle complex on 2 weeks after surgery according to our protocol.
each side of the midline are reapproximated in Montalvo et al. designed an ingenious tech-
the midline and sutured with interrupted 5-0 nique called “posterior flap anorectoplasty”
long-term absorbable sutures, taking a bite of the [11–13] to repair anorectal malformations
References 187

through a posterior sagittal approach, but without without fistula coming to our center with a colos-
separating the rectum from the urinary tract. The tomy that is meant to remain on permanent basis.
bulging posterior wall of the rectum is used to Their doctors advised the parents to keep the
create a flap that is tubularized and switched colostomy for life, because the patients with
down to create the anus. We have no experience Down syndrome are all incontinent. Our experi-
with the procedure; however, we believe that it ence, as presented here, is quite different.
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 References
used in patients with fistula, due to the risk of a
1. Torres R, Levitt MA, Tovilla JM, Rodriguez G, Peña
recurrent fistula. 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
12.4 Function and Results 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,
In terms of bowel control, the results are, as Cohen T (1989) Anorectal malformations and Down
expected, different in patients with Down syn- syndrome. Am J Med Genet 34(3):330–331
drome and patients without Down syndrome. In 4. Heinen F, Bailez M, Solana J (1994) Imperforate anus
and Down syndrome. [El ano imperforado en el
our series, patients without Down syndrome have Sindrome de Down]. Rev Cir Infant 4(2):72–76
an 85 % chance of having voluntary bowel move- 5. de Buys Roessingh AS, Mueller C, Wiesenauer C,
ments by the age of 3. This means that this mal- Bensoussan AL, Beaunoyer M (2009) Anorectal mal-
formation has an excellent functional prognosis. formation and Down’s syndrome in monozygotic
twins. J Pediatr Surg 44(2):e13–e16. doi:10.1016/j.
The incidence of constipation, however, seems to jpedsurg.2008.10.116
be higher than in other types of defects, since we 6. Stephens FD, Smith ED (1971) Chapter 7: Incidence,
know now that a megarectum relates directly frequency of types etiology. In: Ano-rectal malforma-
with the chances of constipation. Surprisingly, tions in children. Year Book Medical Publishers, Inc,
Chicago, pp 160–171
60 % of the patients with Down syndrome have 7. Bischoff A, Frischer J, Dickie BH, Peña A (2014)
voluntary bowel movements by the age of three. Anorectal malformation without fistula: a defect with
Of course, we must remember that Down syn- unique characteristics. Pediatr Surg Int 30(8):763–
drome babies have different degrees of develop- 766. doi:10.1007/s00383-014-3527-5
8. Black CT, Sherman JO (1989) The association of low
mental delay, and therefore, we very much imperforate anus and Down’s syndrome. J Pediatr
depend on that, to try to predict the chances of Surg 24(1):92–94
bowel control. 9. Chan KW, Lee KH, Tsui SY, Wong YS, Pang KY,
Patients with Down syndrome have a well- Mou JW, Tam YH (2014) Bowel perforation in new-
born with anorectal malformation and no fistula at
known tendency to be constipated. Patients with presentation. J Pediatr Surg 49(3):390–394. doi:00
anorectal malformations also have a tendency to 10. Fanjul M, Molina E, Cerdá J, Parente A, Laín A,
be constipated, provided their original rectum is Cañizo A, Carrera N (2009) Characteristics of the
not resected and patients with anorectal malfor- anorectal atresia without fistula. Based on 12 cases.
Cir Pediatr 22(1):45–48
mation with no fistula have a higher incidence of 11. Montalvo-Marín A, Victoria-Morales G (1993)
constipation. Therefore, we must expect these Anorectoplasty with a distal tubular flap via a poste-
patients to suffer from severe constipation. After rior sagittal approach. Bol Med Hosp Infant Mex
the colostomy closure, the surgeon should be 50(7):499–502. Spanish
12. Zea-Salazar LE, Cabrera-Johnson M (1994)
very aggressive and proactive in giving the right Anorrectolpastia Sagital posterior y colgajo rectal
amount of laxatives to avoid constipation. When [Posterior sagittal anorectoplasty and rectal flap]. Rev
not treated adequately, this problem may produce Cir Infant 3:112–114
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RM, Diaz-Lira MA (1998) Continence in patients
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with Down syndrome and anorectal malformation
Minimally Invasive Approach
to Anorectal Malformations 13

13.1 Introduction repaired either way laparoscopically or posterior


sagittally, and others in cases in whom we believe
The minimally invasive approach to the repair of that laparoscopy is formally contraindicated.
congenital malformation is here to stay. All of us, Our basic contention and belief is that laparos-
pediatric surgeons, are concerned about inflicting copy is primarily indicated to replace a laparot-
pain and/or being too invasive to our patients. omy. In other words, a classic indication is a
Every effort aimed to reduce the suffering of our procedure that is usually done opening the abdo-
patients, as well as the length of stay in the hospi- men and now can be done equally well, through
tal and subsequently the cost of the operations, is the small orifices of the ports necessary for mini-
welcomed. We embrace ideas that have, as the mally invasive operation. In male patients with
end result, a less traumatic operation without anorectal malformations, we have to open the
compromising the standard of care or provoking abdomen 10 % of the times, specifically in those
more sequelae in our patients. That explains why cases that have a recto-bladder neck fistula. We
the minimally invasive approach to repair malfor- consider that group a good potential indication
mations had so much impact in pediatric surgery for laparoscopy. We use the word “potential”
and the surgical field in general. because even in those particular types of defects,
Since the introduction of this new technology, laparoscopy has certain limitations, as will be
there are now operations in which the laparo- shown later.
scopic or thoracoscopic approach represents the Approximately 30 % of patients with cloacas
gold standard. There are others in which these require a laparotomy. Yet, all of them require a
new approaches have not been applied, and there posterior approach in order to repair the urogeni-
is another group in which the approach is contro- tal component of the malformation. Conceivably,
versial. Anorectal malformations have not been a laparoscopic approach could be useful to sepa-
an exception, and many pediatric surgeons have rate the rectum from the bladder neck in those
used and continue trying to use more and more unusual cases that have the rectum connected to
the laparoscopic approach to the repair of anorec- the bladder neck. Yet, those patients require
tal malformations, many of them with the correct rather sophisticated and technically demanding
indication, others in malformations that can be 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-
Electronic supplementary material Supplementary
material is available in the online version of this chapter at tions. We found 52 papers published between 1998
10.1007/978-3-319-14989-9_13. and 2014. Forty-one of them [1–41] presented

A. Peña, A. Bischoff, Surgical Treatment of Colorectal Problems in Children, 189


DOI 10.1007/978-3-319-14989-9_13, © Springer International Publishing Switzerland 2015
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 13 Minimally Invasive Approach to Anorectal Malformations

Table 13.2 Summarized results of a literature review on minimally invasive approach to anorectal malformation
185 prostatic fistula

357 93 Bladder neck


males 63 Bulbar

16 “Urethral”

29 “vaginal”
446 45
8 Vestibular
cases females
8 cloaca

17 without fistula
44
21 not specified
gender not
2 perineal
specified
2 rectal atresia
2 “High”

series of cases (Table 13.1). Ten papers [42–52] • Rectourethral bulbar fistula
were rather informative, written by experts but • Anorectal malformation with no fistula
without discussing specific cases. • Perineal fistula
The total number of cases reported in the lit- We believe that mainly those patients with
erature according to our review is 446. These recto-bladder neck fistulas may benefit from the
included 357 males, 45 females, and 44 in whom laparoscopic approach. In our own experience,
the gender was not specified (Table 13.2). the laparoscopic approach provides an excellent
The group of male cases included 185 recto- exposure of the peritoneal floor and the distal part
prostatic fistulas, 93 bladder neck, 63 bulbar, and of the bowel as it approached the bladder neck
16 labeled as “urethral” without specifying the (Animation 13.1). The dissection of the rectum,
location of the fistulas. as well as the ligation of the fistula, is an easy
The female group included 29 “vaginal fis- maneuver. The main reason for this is that in ano-
tula,” 8 vestibular, and 8 cloacas. The group of rectal malformations, the higher the malforma-
patients without gender specification included 21 tion, the shorter the common wall existing
cases, 17 without a fistula, 2 recto-perineal, 2 between the rectum and the urinary tract, and
“rectal atresia,” and 2 cases labeled simply as therefore the rectum ends in the bladder neck in a
“high” (Table 13.2). T fashion, which makes the ligation of the fistula
an easy task, without any risk of damaging neigh-
boring structures. To repair this malformation,
13.2 Males the next step after the ligation of the fistula is the
mobilization of the rectum. We found that some-
Anorectal malformations in males are divided times this is feasible, and the entire procedure
into the following categories: can be completed after making a small incision in
• Recto-bladder neck fistula the perineum. Yet, sometimes the mobilization of
• Rectourethral prostatic fistula the rectum (as explained in Chap. 11) requires a
13.2 Males 193

delicate and selective ligation of mesenteric ves- that patients with anorectal malformation without
sels, preserving the necessary arcades in order to fistula should not be approached laparoscopi-
preserve the blood supply of the distal rectum. cally. The rectum is located very close to the
Laparoscopically, this maneuver is done using perineum, and therefore it requires a minimal dis-
cautery, which is a less than optimal way to section to be mobilized. A laparoscopic approach
divide vessels, because the extension of the burn implies in these cases a full dissection of the rec-
is not controlled accurately and may damage col- tum, which is actually unnecessary. In our series,
lateral vessels necessary to preserve the blood the operation takes 2 h, the patients have minimal
supply of the distal rectum. In addition, some of pain, the blood loss is negligible, and the patient
these patients have a very dilated rectum that can be discharged 24 or 48 h after the operation.
requires tapering. We found difficult to perform Rectourethral bulbar fistula is fortunately
the tapering of the bowel laparoscopically, and the most common defect that we see in male
therefore we have to open the abdomen. It is patients. The rectum is located approximately
important to remember that unfortunately, these 2 cm above the skin and connects to the bulbar
groups of patients (recto-bladder neck fistula) urethra at the same level of those cases of imper-
have a very poor functional prognosis. Only forate anus with no fistula. Eighty-five percent
20–25 % of our patients in this category have vol- of our patients with normal sacrum have bowel
untary bowel movements, and about half of those control, and 94 % of them have urinary control.
suffer from soiling. These patients are born with The operation takes approximately 2½ h, and the
serious congenital deficiencies in their sphinc- patients eat the same day, have minimal pain, and
teric mechanism and frequently are associated to can be discharged the next day. We find it very
very poor sacra. difficult to have a less invasive procedure than
Ninety-three cases of our literature review the one that we perform. Again, trying to repair
belong to this category of recto-bladder neck fis- these malformations laparoscopically represents
tula. We believe that all pediatric surgeons agree a maximally invasive approach since the entire
that this malformation represents an excellent rectum has to be dissected from above. When
indication for a laparoscopic approach. these malformations are repaired posterior sagit-
Perineal fistulas are malformations that can tally, we only dissect the distal part of the rectum
be repaired with an operation that takes less as much as necessary to bring the rectum down;
than 45 min with 100 % chance of bowel con- we do not have to go all the way to the perito-
trol. Therefore, we do not see the reason to neum. As a consequence of attempting the repair
attempt a less invasive procedure than the one of this malformation laparoscopically, a signifi-
that we do at present time. Yet, we found two cant number of patients suffered from recurrent
publications that included perineal fistula rectourethral fistula or a large persistent posterior
repaired laparoscopically [2, 12]. urethral diverticulum which represents a piece
Imperforate anus with no fistula is a malfor- of rectum left attached to the urethra [53, 54]
mation in which the rectum is blind; it has no (Animation 13.2) (Fig. 13.1). We believe that
communication with the urinary tract. Half of laparoscopically the surgeons found it impos-
these patients have Down syndrome. Our experi- sible to reach the bulbar fistula site and ampu-
ence shows that this is a benign condition if we tated the rectum leaving a piece of rectum that
consider the good functional prognosis. Ninety later gave significant symptoms. We believe that
percent of our patients without Down syndrome the laparoscopic approach of this malformation
have bowel control, and 70 % of our Down is contraindicated. Unfortunately, many surgeons
syndrome patients also have voluntary bowel keep trying [5, 6, 11–13, 17, 24–26, 30, 34, 35,
movements. 37–39].
We found that 17 patients (from our literature Rectourethral prostatic fistula is the second
review) with this defect underwent a laparoscopic most common malformation that we have seen in
repair [2, 7, 10, 17, 22, 23, 25, 34, 37]. We believe 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 “rectourethral fistula” [7, 10, 13, 22]. Operating a
best way to approach it. The posterior sagittal male baby with an anorectal malformation
approach in these malformations renders a 66 % without knowing the detailed anatomy represents
chance of bowel control by the age of three. a potential misadventure with serious conse-
Ninety-two percent of the patients have urinary quences for the patient.
control. In most of these patients, the posterior
sagittal dissection of the rectum requires to open
the peritoneum. In other words, the dissection is 13.3 Females
a complete one, due to the fact that the rectum is
located high in the patient’s pelvis. If a surgeon is Anorectal malformations in females are divided
particularly well trained laparoscopically and into the following categories:
prefer to approach this type of malformation lap- • Perineal fistula
aroscopically, rather than posterior sagittally, we • Vestibular fistula
do not see a real difference between going one • Cloacas
way or the other. In addition, some of the recto- • Complex malformations
prostatic fistulas are located a little higher than Perineal fistulas in females are repaired with a
others closer to the bladder neck; in those cases, minimal posterior sagittal operation that takes
we have seen complications when inexperienced less than an hour; the patients have minimal pain,
surgeons try to repair this malformation via pos- 100 % of them have bowel control, and therefore
terior sagittal. Perhaps, if they have good training we do not see a justification to do a maximally
in laparoscopic techniques, it will be better for invasive operation dissecting the entire rectum
them to approach those malformations that way. through the abdomen to repair these malforma-
We were happy to find in our literature review tions. Yet, as previously mentioned, some cases
that 185 out of 357 male cases operated laparo- are occasionally done laparoscopically.
scopically had this kind of defect. Vestibular fistulas are by far the most common
Unfortunately, we found in our literature defects in female patients with anorectal malfor-
review that many surgeons still use the old termi- mations. In our series, 95 % of these patients with
nology that we consider inadequate and mislead- a normal sacrum have bowel control. The opera-
ing. They still refer to “high, intermediate, and tion to repair this defect takes us approximately
low” malformations. Sixteen of the published 2½ h. The patients can eat the same day when
cases received the nonspecific diagnosis of operated with a colostomy and can be discharged
13.3 Females 195

the next day after surgery. The most important We are obviously biased against the laparo-
anatomic feature in these patients is the common scopic approach of cases with rectourethral bul-
wall existing between the rectum and the vagina bar fistula because of the cases that we see at our
(see Chap. 15). The real challenge in the repair of center, suffering from a complication consecutive
these malformations is to make two walls out of to the laparoscopic approach, including urethral
one, preserving intact the posterior vaginal wall injuries and posterior urethral diverticula
and the anterior rectal wall. It is difficult for us to [53, 54].
imagine why somebody wants to dissect the We are aware of the fact that these complica-
entire rectum through the abdomen, in order to tions are not necessarily a consequence of the
repair these malformations and still claim that it laparoscopy, since we have seen similar compli-
is less invasive [9, 17, 36, 37]. In some of those cations occurring during the repair of anorectal
patients in whom the surgeons attempted a lapa- malformations with other techniques. However,
roscopic repair, actually, they left a piece of rec- these complications occurred mainly in patients
tum attached to the vestibule and mobilized more suffering from rectourethral bulbar fistulas.
colon from above, which is against the basic prin- In our literature review, we found that 29
ciples of the repair of anorectal malformations. patients were operated laparoscopically with the
Cloacal malformations are the second most diagnosis of rectovaginal fistula [2, 3, 6–10, 17,
common anorectal malformations seen by us in 23, 28, 29, 34–36].
females. The repair of a cloaca requires separa- We consider that real vaginal fistula is an
tion of the rectum from the urinary tract followed almost nonexistent malformation. In our series
by the mobilization and repair of the urethra and of over 1,000 female patients, we have seen only
vagina. For that, most of the time we use a maneu- seven real rectovaginal fistula cases (see Chaps.
ver called total urogenital mobilization. There are 15 and 16). Most of the cases that came to us
reports of laparoscopic attempted repairs of clo- with the diagnosis of “rectovaginal fistula,” in
acas [7, 9, 10, 17, 23, 29]. The authors actually retrospect, were misdiagnosed cloacas or rec-
only repaired the rectal component of the malfor- tovestibular fistula. If these authors found 29
mation, and then they performed a posterior sagit- vaginal fistulas out of 45 female cases, we have
tal approach from below in order to repair the reason to suspect that they were not really vagi-
urogenital component of the malformation. In nal fistulas.
other cases the surgeons repaired the rectal com- Some authors [10, 17, 35] try to evaluate the
ponent and left the urogenital tract intact! In other clinical results using an old score that includes
words, we cannot say that the laparoscopic “number of bowel movements” as indicative of
approach has been used to repair cloacas, but, results; “many bowel movements” were consid-
rather, we should say that it has been used to ered as bad and “few bowel movements” were
repair the rectal component of cloacal malforma- considered “good”!! The number of bowel move-
tions. Yet, in our experience, in over 500 cloacas, ments relates to many factors (diet, length of the
the rectum can be reached posterior sagittally in colon, inflammatory factors, degree of dilatation
the majority of cases; therefore, we do not see a of the rectosigmoid); none of them related to the
reason to try to go through the peritoneal cavity to type of repair (laparoscopic or posterior
repair a malformation that can be repaired from sagittal).
below. Again, in theory, laparoscopy could be We were negatively impressed by the fact that
helpful to mobilize a rectum that can only be only eight of the papers that we reviewed men-
mobilized through the abdomen. Yet, the patient tioned the characteristics of the sacrum of the
still will require a posterior sagittal approach any- patients [5, 13, 14, 17, 21, 28, 30]. We think that
way to repair the urogenital sinus. it is impossible to discuss functional results in the
Laparoscopy can also be useful instead of a lapa- treatment of anorectal malformations without
rotomy for the examination of the intra-abdominal specifying the characteristics of the sacrum of the
anatomy in complex anorectal malformations. patients. The presence or absence of tethered
196 13 Minimally Invasive Approach to Anorectal Malformations

cords was only mentioned by five authors [2, 4, laparotomy. There is no current evidence of bet-
17, 28, 40]. The presence of this anomaly is rec- ter clinical results. There are a significant number
ognized as a negative prognostic factor, mainly of cases in which laparoscopy has been used with
for the urinary function. no real justification.
One of the rationales to justify the laparo- We are happy to see that many authors recog-
scopic approach in patients with anorectal mal- nize the risk of urethral damage and leaving a
formations is the short length of stay. We were posterior urethral diverticulum and are showing a
very surprised to find that the length of stay was high degree of creativity, using sophisticated
only mentioned in three papers [22, 35, 41], and technology to avoid these complications [12, 24,
the authors reported 5–11 days’ range of length 30]. Others are already limiting the use of lapa-
of stay. In our series, 90 % of our male cases did roscopy to cases with bladder neck or prostatic
not require a laparotomy, and they remained in fistula [37].
the hospital 48 h postoperatively. In females, We strongly suggest to all surgeons interested
with the exception of complex cloacas that in performing laparoscopic repair of anorectal
required a laparotomy, the length of stay was malformations to follow the following
similar to the males. guidelines:
Ten publications [6, 10, 17, 22, 32–35, 37, 41] • Use an adequate terminology; try to be accu-
compared the functional results obtained with rate in the type of malformation treated.
laparoscopy and the traditional PSARP (posterior • Refer to the characteristics of the sacrum as
sagittal anorectoplasty). Most of the authors con- well as the presence or absence of tethered
cluded that the differences found were not sig- cord, in order to discuss clinical results.
nificant. Others suggested that the results • Avoid confusing terms such as “high,” “inter-
obtained with the use of laparoscopy are better mediate,” and “low.”
[17, 41]. • When discussing cloacal approach laparo-
Four of the papers [6, 10, 22, 35] evaluated scopically, be specific as to “repair of a clo-
their results using rectal manometry, which we aca” or repair of the “rectal component of the
consider a non-reliable method (see Chap. 25). cloaca.” Be specific in saying exactly what
Some authors mentioned the “potential nega- was done with the urogenital component,
tive effect of the scarring produced by the poste- which represents the real technical challenge.
rior sagittal approach.” That is a reasonable We firmly believe that minimally invasive
theoretical concern; however, we strongly sug- techniques will play an increasing positive role in
gest for those authors and the readers to read our the management of many congenital anomalies
publication [55] describing our experimental including of course anorectal malformations.
experience (in dogs), comparing different types However, we must be cautious to avoid negative
of rectal operations and their effect on bowel con- results as a consequence of excessive enthusiasm
trol. The posterior sagittal incision did not affect or the desire of notoriety.
bowel control. In addition, from the clinical point
of view [56], we have operated multiple cases
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posterior sagittal (PSARP) anorectoplasty in the out- copy. Bull Acad Natl Med 192(5):913–918; discussion
come of intermediate and high anorectal malforma- 918–919
tions. S Afr J Surg 49(1):39–43 48. Al-Hozaim O, Al-Maary J, AlQahtani A, Zamakhshary M
35. Tong QS, Tang ST, Pu JR, Mao YZ, Wang Y, Li SW, (2010) Laparoscopic-assisted anorectal pull-through for
Cao QQ, Ruan QL (2011) Laparoscopically assisted anorectal malformations: a systematic review and the need
anorectal pull-through for high imperforate anus in for standardization of outcome reporting. J Pediatr Surg
infants: intermediate results. J Pediatr Surg 46(8):1578– 45(7):1500–1504. doi:10.1016/j.jpedsurg.2009.12.001
1586. doi:10.1016/j.jpedsurg.2011.04.059 49. Yamataka A (2011) Correspondence reply to letter to the
36. Miglani RK, Murthy D, Bhat RS, Ashok KK (2012) editor by Hamrick et al. J Pediatr Surg 46:1018–1019
Anorectal anomalies in adults-laparoscopic manage- 50. Japanese Multicenter Study Group on Male High
ment and review of literature. Indian J Surg 74(4):301– Imperforate Anus (2013) Multicenter retrospective
304. doi:10.1007/s12262-011-0394-3 comparative study of laparoscopically assisted and
37. England RJ, Warren SL, Bezuidenhout L, Numanoglu conventional anorectoplasty for male infants with rec-
A, Millar AJ (2012) Laparoscopic repair of anorectal toprostatic urethral fistula. J Pediatr Surg 48(12):2383–
malformations at the Red Cross War Memorial 2388. doi:10.1016/j.jpedsurg.2013.08.010
Children’s Hospital: taking stock. J Pediatr Surg 51. Bischoff A, Levitt MA, Peña A (2011) Laparoscopy and its
47(3):565–570. doi:10.1016/j.jpedsurg.2011.08.006 use in the repair of anorectal malformations. J Pediatr Surg
38. Jung SM, Lee SK, Seo JM (2013) Experience with 46(8):1609–1617. doi:10.1016/j.jpedsurg.2011.03.068
laparoscopic-assisted anorectal pull-through in 25 52. Shawyer AC, Livingston MH, Cook DJ, Braga LH
males with anorectal malformation and rectourethral (2015) Laparoscopic versus open repair of recto-
or rectovesical fistulae: postoperative complications bladderneck and recto-prostatic anorectal malforma-
and functional results. J Pediatr Surg 48(3):591–596. tions: a systematic review and meta-analysis. Pediatr
doi:10.1016/j.jpedsurg.2012.08.001 Surg Int 31(1):17–30
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53. Peña A, Hong AR, Midulla P, Levitt M (2003) 55. Peña A, Amroch D, Baeza C, Csury L, Rodriguez G
Reoperative surgery for anorectal anomalies. Semin (1993) The effects of the posterior sagittal approach
Pediatr Surg 12(2):118–123 on rectal function (experimental study). J Pediatr Surg
54. Alam S, Lawal TA, Peña A, Sheldon C, Levitt MA 28(6):773–778
(2011) Acquired posterior urethral diverticulum 56. Peña A, Filmer B, Bonilla E, Mendez M, Stolar C
following surgery for anorectal malformations. (1992) Transanorectal approach for the treatment of
J Pediatr Surg 46(6):1231–1235. doi:10.1016/j. urogenital sinus: preliminary report. J Pediatr Surg
jpedsurg.2011.03.061 27(6):681–685
Rectal Atresia
14

Rectal atresia is a very unique malformation that Interestingly, the sphincter mechanism is excel-
deserves a special description. It happens in our lent in most cases. There is one particular malfor-
experience, in about 1 % of all cases of anorec- mation similar to this one that is represented by a
tal malformations. In this defect, the anus seems stricture or by atresia of the rectum, associated to a
to be completely normal, including the quality of presacral mass and a sacral defect (see Chap. 8,
the sphincter and the location of the anal orifice. Sect. 8.2), which is a completely different type of
However, deep inside the anus, just at the junction of defect. The only thing they have in common is the
the anal canal with the rectum, there is an atresia or fact that the rectum is narrow or atretic.
narrowing (stenosis) (Fig. 14.1). Occasionally, we We believe that rectal atresia with normal
see atresias or stenosis located at a different level. sacrum and no presacral mass is unique, because
The space that separates the dilated blind rectum, the sphincter mechanism is normal and also
from the anal canal, is represented by a septum that because these patients do not have the typical
sometimes is extremely thin and can be perforated, association with all the defects that we see in
and other times it is very thick. In some unusual other anorectal malformations. As a conse-
cases, there is a significant separation between the quence, the prognosis for these patients is excel-
blind upper rectum and the lower anal canal. 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 14 Rectal Atresia

significant tendency to suffer from severe consti- cal modification maneuver [5] (Fig. 14.4). Most
pation because they are born with a blind, very of the patients that we operated on came to us
dilated rectum. These malformations have been already with a colostomy in place. Since the
previously described in the literature [1–5]. patient has a colostomy, one can perform a distal
Rectal atresia has been traditionally described colostogram and simultaneously introduce a
in the old textbooks. The baby is born with a metallic dilator in the anal canal to have a lateral
normal-looking anus, and the nurse or the pedia- image of the atresia and estimate the distance
trician tries to pass a thermometer through the between the upper pouch and the anal canal. If
anus and finds an obstruction. In fact, part of a we could make the diagnosis early in an other-
routine examination of every “normal” newborn wise healthy newborn baby, we would recom-
is to check the patency of the anus, unless the mend to do the operation without a colostomy.
baby is already passing meconium.

14.2 Surgical Repair


14.1 Treatment
The patient is placed in the prone position and we
If one could think in an ideal indication for a pos- approach the malformation posterior sagittally.
terior sagittal approach, this would be the malfor- We go through the skin, subcutaneous tissue,
mation which seems to be more indicated. The parasagittal fibers, ischiorectal fossa, and the
defect is easily repaired through a posterior sagit- entire sphincter mechanism to expose and open
tal incision. In our initial cases, we simply remove completely the anal canal and the upper blind rec-
the septum that separates the upper rectum from tum. One can see in most cases the pectinate line,
the anal canal and created an end-to-end anasto- at the same location as the atresia (Fig. 14.2a).
mosis (Figs. 14.2 and 14.3). Subsequently, we Unfortunately, we still see some of these
found some cases in which the size discrepancy patients, previously operated in whom the sur-
between the upper blind rectum and the small geon considered that the little anal canal was use-
anal canal was very severe, and in order to expand less and therefore decided to resect it and pulled
the size of the anal canal, we introduced a techni- down the dilated piece of rectum. That is rather

a b

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
14.2 Surgical Repair 203

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, and the anal canal is going to be permanently col-
represents the area of sensation that will provide lapsed by the effect of the sphincter mechanism
bowel control to these patients. It is, therefore, that keeps the anal canal closed all the time,
very important to preserve that little anal canal. except during defecation; therefore, these babies
Sometimes the size of the anal canal is too small. must be subjected to the same protocol of anal
For that, we introduced a technical modification dilatations that we already described.
[5], consisting in mobilizing the posterior rectal Some surgeons [4] went as far as to perform a
wall, down to the skin of the anus (Fig. 14.4), “laparoscopic transanal approach” to repair this
enlarging the circumference of the anus. We real- malformation. To demonstrate that something
ize that by doing that, the posterior aspect of the can be done does not mean that it must be done.
anus will no longer be a real anal canal, but rather We cannot justify to change a limited, painless,
a rectal wall. However we manage to preserve bloodless, quick, minimally invasive, non-
most of the circumference of the original anal laparoscopic procedure for a laparoscopic inva-
canal, which will provide enough sensation to sive operation that includes an unnecessary total
have bowel control. We must keep in mind that rectal dissection.
after we finish this procedure, the anastomosis Our experience includes 11 cases and has
that we created between the upper dilated rectum 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 1754.2005.00763.x
4. Nguyen TL, Pham DH (2007) Laparoscopic and
1. Dias RG, Santiago Ade P, Ferreira MC (1982) Rectal transanal approach for rectal atresia: a novel alterna-
atresia: treatment through a single sacral approach. tive. J Pediatr Surg 42(11):E25–E27. doi:10.1016/j.
J Pediatr Surg 17(4):424–425 jpedsurg.2007.08.049
2. Upadhyaya P (1990) Rectal atresia: transanal, end-to-end, 5. Hamrick M, Eradi B, Bischoff A, Louden E, Pena A,
rectorectal anastomosis: a simplified, rational approach to Levitt MA (2012) Rectal atresia and stenosis: unique
management. J Pediatr Surg 25(5):535–537 anorectal malformations. J Pediatr Surg 47(6):1280–
3. Kisra M, Alkadi H, Zerhoni H, Ettayebi F, 1284. doi:10.1016/j.jpedsurg.2012.03.036
Benhammou M (2005) Rectal atresia. J Paediatr
Rectovestibular Fistula
15

15.1 Definition/Frequency previous failed attempted repair. The difference


in terms of bowel control is significantly differ-
Rectovestibular fistula is the most important ent. Therefore, we can repeat what other pediat-
anorectal malformation in females. This is due ric surgeons through history have said, and that
to the fact that, by far, it is the most common is that “these patients have a single opportunity
defect seen in females. Two hundred and ninety to have a good repair.”
of our 1,123 female patients were born with this In the old literature [1] one can find that this
malformation. Two hundred and seventeen were malformation received different names, includ-
operated primarily by us, and 73 were reopera- ing “anovestibular fistula.” The authors believed
tions due to a previous failed attempted repair. that this was a more benign variant of defect and
Interestingly, our series include 531 patients that these patients had a very short fistula and a
with a cloaca. However, we are convinced that very low-lying rectum. They also believed that
this high number of cloacas in our series is malformation owed to be distinguished from a
because ours is a referral center. We believe that “rectovestibular fistula,” which has a long, nar-
vestibular fistula is much more common in the row fistula and a rectum located higher in the pel-
general population. Most of the vestibular fis- vis, and, therefore, they believed that the
tula cases are operated at the place where the prognosis was not as good as the one observed in
babies are born, whereas many cloacas are cases of “anovestibular fistula” [1, 2]. We also
referred to us due to the complexity of the repair. found the term “vestibular anus”; the authors
When this malformation is repaired with a believed that some patients were born with an
meticulous surgical technique, the recovery of otherwise normal anus located in the vestibule of
the patients is excellent, and the functional the female genitalia [2]. We have never seen this
results are also very good. Unfortunately, type of defect. We do not use those three terms
another characteristic of this malformation is mentioned here, because we found that all our
the fact that it is frequently mismanaged. We patients with an anal opening located in the vesti-
compared the results obtained in a group of bule can be repaired with the same surgical pro-
patients that were repaired primarily by us with cedure and have the same functional prognosis;
those of the group that had a secondary proce- therefore, we consider the old terminology
dure due to the fact that the patient underwent a impractical and misleading. It is true that some
patients have a longer fistula than others; those
cases may require more dissection to bring the
Electronic supplementary material Supplementary
material is available in the online version of this chapter at rectum down. However, our results are uniformly
10.1007/978-3-319-14989-9_15. good, regardless the type of fistula.

A. Peña, A. Bischoff, Surgical Treatment of Colorectal Problems in Children, 205


DOI 10.1007/978-3-319-14989-9_15, © Springer International Publishing Switzerland 2015
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 15 Rectovestibular Fistula

in general, the frequency of association of all the Figure 15.6a shows the perineum of one of
defects is rather low. Yet, it is significant enough these patients, and there is no vaginal opening.
to be searched for. 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
15.2.1 Sacral function as a neovagina and pulling the upper
rectum down to the perineum.
We were able to measure the sacral ratio in 113 Eighty percent of the patients with vestibular fis-
of our cases and found that the average AP ratio tula and absent vagina are born with agenesis of the
was 0.57 and lateral was 0.7. Six percent of these internal genitalia (uterus and fallopian tubes). In
cases had a ratio lower than 0.4. This is consis- such cases the vagina is replaced with a piece of
tent with the fact that we consider this malforma- colon; this is done only for the patient to have sexual
tion a “benign” one, with good functional function. Twenty percent of the patients have a
prognosis. Fourteen cases had a hemisacrum and uterus and a blind ending of vagina, usually located
a presacral mass, and as previously mentioned, very high in the pelvis (Fig. 15.6c). In that type of
presacral masses occur more frequently in lower case, the lower vagina is replaced with a piece of
defects. colon with dual purpose (sexual and reproductive).
Some cases of vestibular fistula with absent
vagina can be repaired without vaginal replacement,
15.2.2 Spinal but rather pulling down their native vagina. That can
only be done in cases with a large blind vagina.
Approximately, 9 % of our patients had some Five percent suffered from some sort of septa-
form of spinal defect, mainly hemivertebra. tion disorder of the Müllerian structures. These
included a vaginal septum, always associated
with the presence of two hemicervices and two
15.2.3 Urologic hemiuteri (Fig. 15.7). Three patients had a unilat-
eral streak ovary; the rest had two normal ovaries.
Ten percent of vestibular fistula cases had a sin- Two patients had a perineal lipoma, and one
gle kidney, which as we know is the most com- patient had a labial hemangioma.
mon anatomic abnormality associated to all We were able to see patients born with a ves-
anorectal malformations, and 13 % of patients tibular fistula that came to us as adolescents; they
had vesicoureteral reflux, which is consistent had a repair in the past, but the surgeons missed
with the fact that this disorder is the most com- the diagnosis of a vaginal septum. These vaginal
mon functional urologic abnormality seen in ano- septa can only be detected when the surgeon sus-
rectal malformation cases. Hydronephrosis was pects their existence. Based on these findings, it
present in 6 % of the cases. is our routine and our recommendation to perform
a vaginoscopy with a pediatric cystoscope in all
patients with vestibular fistula. The presence of a
15.2.4 Gynecologic vaginal septum may, in some cases, interfere with
tampon placement and sexual intercourse when
There are not many reports in the literature, the patient grows up. But more important than
related to this very important assoc [3, 4]. A ret- that is the fact that the presence of a vaginal sep-
rospective review of our patients with vestibular tum means, by definition, that the patient has two
fistula showed that 17 % of them had associated hemiuteri, representing a partial or total septation
genital anomalies [5]. Eight percent had absent disorder. Hemiuteri have important gynecologic
vaginas or vaginal atresia. Figure 15.6 shows the and obstetric implications. We know that patients
different types of absent vaginas or vaginal atre- with hemiuteri may have a higher degree of infer-
sias encountered. 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 Fifty-seven patients were evaluated with an
to provide these patients with special gyneco- ultrasound (first 3 months of life) or with an
logic and obstetric care later in life. MRI, looking for spinal cord anomalies; twenty
It is our routine to do a vaginoscopy in every of them had tethered cord (35 %). These figures
case of vestibular fistula. We perform that study are higher than the average of all anorectal mal-
with a baby cystoscope, during the same anesthe- formations, and we believe that this is explained
sia given for the repair. Figure 15.8 shows the by the fact that the incidence of presacral masses
aspect of a normal infant cervix. is also high in this malformation. Tethered cord
is very common in cases with presacral mass.

15.2.5 Gastrointestinal
15.2.7 Cardiovascular
Six percent of our patients with vestibular fistula
had an associated esophageal atresia, one patient Twenty-seven patients (9 %) had an atrial septal
without a fistula, and all the others with a tra- defect. Twenty-two (8 %) had a ventricular sep-
cheoesophageal fistula; 1 % had a form of duode- tum defect. Fourteen (5 %) had a patent ductus
nal obstruction (atresia or stenosis). 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 211

Fig. 15.9 Aspect of a normal cervix in a baby with a ves-


tibular fistula

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 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
Fig. 15.8 Vestibular fistula with a vaginal septum. Arrow and the surgeons only repaired the rectal compo-
shows the fistula site
nent of the malformation, because they were
thinking that the patient only had a rectovaginal
of Fallot. Most of these defects (approx. 80 %) fistula (see Chap. 16, Sect. 16.1.4).
did not require treatment, since the patients were Prior to 1980, the literature [2, 6–13] reported
hemodynamically stable. an elevated number of cases of “rectovaginal fis-
212 15 Rectovestibular Fistula

tula” in female patients. In contrast, those authors colostomy is different for every surgeon and his/
reported very few vestibular fistula cases and her different surrounding circumstances. It very
very rare cloaca cases. A few publications after much depends on the experience of the surgeon,
1980 persist reporting “rectovaginal fistula the clinical condition of the patient, and the infra-
cases.” Interestingly, looking at the diagrams of structure of the hospital where the patient is
most of those publications, they actually show treated.
vestibular fistulas, although they call them “vagi- In general, at our institution, if a baby is born
nal fistula.” The term “vestibular fistula” has been with a vestibular fistula, we operate on her within
used correctly by some authors with large experi- the first 5 days of life without a colostomy, pro-
ence in the management of these defects vided the baby is in good clinical condition, is
[14–21]. full term, and does not have severe associated
We believe that this is not a simple semantic defects.
problem, but rather has important clinical Consider the case of a premature baby with a
implications [22]. We have seen patients born cardiac condition and vestibular fistula. Under
with vestibular fistula that were previously those circumstances, dilatations of the fistula
misdiagnosed as “vaginal fistula” and under- may prove to be useful for the patient to be able
went a type of repair designed to repair “high” to pass stool, eat, and grow. That would allow the
malformations, namely, a contraindicated surgeon to postpone the decision of colostomy or
abdominoperineal (open or laparoscopic) pro- primary repair. On the other hand, a full-term
cedure that resulted in fecal incontinence. We baby in good clinical condition without associ-
also have seen that at least 30 patients born ated defects in an institution with a good infra-
with a cloaca received the wrong diagnosis of structure and a pediatric surgeon with experience
“rectovaginal fistula” and underwent a repair in the management of this defect, the patient can
only of the rectal component of the be operated before starting her feedings, at a time
malformation, leaving the patient with a uro- when the patient is still passing meconium,
genital sinus [22]. because when it is done in that way, the patient
actually does not need any kind of bowel
preparation.
15.4 Treatment Most of our patients come to us after the new-
born period and with a colostomy already opened
15.4.1 Colostomy or No Colostomy at another institution, sometimes in another coun-
try. Many other patients come to us after several
This is a frequently debated subject. Many sur- months of passing stool with difficulty through
geons claim that they routinely repair vestibular the non-operated vestibule, with severe constipa-
fistulas without a colostomy and they have “good tion and megacolon. Those patients are also
results” [12, 23–27]. Many others prefer to open treated without a colostomy at our institution, but
a colostomy in all cases with a vestibular fistula. our routine includes the admission of the patient
In the meantime, we see many patients that 1 or 2 days prior to the main operation, insertion
underwent a repair of a vestibular fistula without of a nasogastric feeding tube, and administration
a colostomy and suffered from serious complica- of GoLYTELY1 at a rate of 25 mL/kg/h until the
tions, including dehiscence and retraction of the colon is completely clean. The patient receives a
rectum as well as reopening of the fistula. PICC line and parenteral nutrition for a period of
However, this recurrent fistula is frequently an 7–10 days postoperatively.
acquired rectovaginal fistula, due to the fact that When the patient has a colostomy, the opera-
during the attempted repair, the posterior wall of tion can be done without following this routine,
the vagina was damaged.
As discussed in the Chap. 5, we believe that 1
GoLYTELY… (Polyethylene glycol/electrolytes.) Braintree
the answer for this question of colostomy or no Laboratories, Braintree, MA, USA
15.5 Main Repair 213

but rather irrigating only the distal stoma of the scope, because sometimes one can see a vaginal
colostomy the day before surgery. In that case, septum that is only present in the lower part of
the baby can eat the same day of the operation; the vagina, and the upper part has a single cervix
she will stay for 48 h in the hospital receiving (Fig. 15.8). Most of the times, however, the sep-
intravenous antibiotics. Our experience is that the tum is complete, and one can see two cervices at
pain that these patients experience postopera- the end of the vagina.
tively is rather minimal. We have operated on pri- The patient is then turned into the prone posi-
marily without a colostomy in approximately tion with the pelvis elevated, and the perineum,
50 % of our cases. as well as the genitalia and perianal area, is
We use a posterior sagittal approach to repair washed, prepped, and draped in the usual man-
these malformations. Other approaches do exist, ner. Most of the times, one can see the anal ori-
and the most traditional and popular was fice in the vestibule, and in such case multiple
described by Dr. Potts and is called a fistula trans- 5-0 silk stitches are placed at the mucocutane-
plant [28–32]. Some surgeons describe an opera- ous junction of the anal opening (Fig. 15.10).
tion called “anterior sagittal approach” [33–37]. These stitches serve the purpose of applying
We found that the word “anterior” in those publi- uniform traction to facilitate the separation of
cations was actually not referring to the incision, the rectum from the vagina. Occasionally, the
but rather to the position of the patient; in other fistula is located so deep that it is impossible to
words, the patient is positioned in lithotomy posi- do this; in such case, we first make the incision
tion, rather than prone, but the incision is always and go deep enough to be able to see the edges
posterior to the fistula, because there is no way to of the fistula and apply multiple 5-0 silk stitches
make an incision anterior to the fistula site. In (Fig. 15.11). We use the electrical stimulation to
other words, the so-called “anterior sagittal determine the limits of the sphincter and to
approach” is actually a posterior sagittal approach guide ourselves to try to stay as much as possi-
performed in lithotomy position. ble exactly in the midline, dividing the entire
Interestingly, Professor Francesco Rizzoli sphincter mechanism leaving equal portions of
from Bologna, Italy, published in 1869 [38] the the sphincter in both sides of the midline. For
technique now referred as “anterior sagittal this, we use a needle-tip cautery, changing from
approach”; his publication includes magnificent cutting to coagulation. The size of the incision
illustrations. usually is shorter than the regular posterior sag-
The essential components of the posterior sag- ittal anorectoplasty. The incision usually runs
ittal approach described below avoid the flaws from the lowest part of the sacrum and coccyx
observed in those other techniques, and the most down to the fistula orifice, passing through the
common problems seen in our reoperations were sphincter mechanism. We divide the entire
retraction of the anoplasty and an inadequate sphincter, including the parasagittal fibers, the
perineal body (anteriorly located anal orifice). muscle complex, and the levator mechanism.
Deeper to the levator mechanism, one can iden-
tify the characteristic white fascia that covers
15.5 Main Repair (Animation 15.1) the posterior wall of the rectum (Fig. 15.12).
Figure 15.13 shows the aspect of the rectum
The patient is brought to the operating room, and with traction sutures. Traction creates the plane.
we start the procedure with the patient in the The white fascia is removed from the posterior
lithotomy position in order to perform a rectal wall, including the extrinsic blood supply of
vaginoscopy using a baby cystoscope. We do this the rectum. We do this to identify the real rectal
with the specific purpose to rule out vaginal wall completely clean. The dissection is then
malformations. Although a vaginal septum can extended to the lateral walls of the rectum
be simply seen by separating the labia without (Fig. 15.13). The next step consists of extending
the use of a cystoscope, we prefer to use a cysto- 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 215

Fig. 15.13 Diagram showing the dissection of rectum

length from 1 to 3 cm and that there is no real


Fig. 15.12 “White fascia” after dividing the entire plane of dissection between both structures. In
sphincter mechanism, the rectum is identified covered by
the white fascia 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-
in this description, meaning to identify the poste- tomic feature of this malformation. Surgeons
rior rectal wall; continue the dissection to the lat- must keep in mind that the main challenge in the
eral walls of the rectum and then from there, repair of these defects is the separation of the rec-
applying uniform traction on the multiple 5-0 silk tum from the vagina and should take it as a per-
sutures; and continue the dissection from the lat- sonal challenge. The separation of the rectum
eral walls of the rectum down to the skin. One from the vagina requires a very meticulous, deli-
must expect to find important vessels that provide cate surgical technique. It cannot be done by
the blood supply of the lower rectum while blunt dissection. We like to perform this separa-
dissecting the lateral walls of the rectum. tion using the needle-tip cautery while applying
A Weitlaner retractor is used to achieve adequate, traction on the rectal wall and checking with a
optimal exposure. At this point, we are ready to lacrimal probe the thickness of the anterior rectal
initiate the most important part of the operation, wall and the posterior vaginal wall very fre-
which is the separation of the rectum from the quently, to be sure that we are not getting too
vagina. close to one or the other (Fig. 15.14). As we
One must keep in mind that the rectum and progress in this meticulous dissection, the wall of
vagina share a common wall with a variable the rectum, as well as the wall of the vagina,
216 15 Rectovestibular Fistula

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 until we can leave a normal rectal wall in front of
getting close to the point where both are expected the vaginal orifice or suture.
to be completely separated and have a full thick- We must always remember that in dealing with
ness. At this time, the surgeon should not be anorectal malformations, the real challenge in the
overconfident, because in that point he could surgical repair is represented by the separation of
injure either the rectum or the vagina (Fig. 15.14). the structures, namely, the rectum from vagina,
The dissection must continue until the rectum has the rectum from urethra, and the vagina from ure-
been completely separated from the vagina thra, because all those structures share a common
(Fig. 15.14). wall without a plane of dissection. Most of the
It is extremely common for surgeons to ask complications that we have seen in patients who
what happens and what to do in the event of acci- underwent failed attempted repairs of anorectal
dentally opening either the vagina or the rectum. malformations occur during the separation of
Our routine answer is as follows: if it happens that these structures.
we opened the vaginal wall, but maintained intact Sometimes when the rectum has been fully
the rectal wall, one can actually leave the vaginal separated from the vagina, we find that we have
orifice of the injury open, provided the rectum is enough rectal length to do an anoplasty without
intact, and the anoplasty is not under tension, and tension and with good blood supply. However,
the patient is going to do alright. Something simi- many other times, the rectum needs further mobi-
lar can be said when the orifice is created in the lization. To do this, one must continue applying
rectum, but the vaginal wall is intact. What is con- uniform traction on the multiple silk stitches. By
sidered nonacceptable is to have an injury of the doing this, it becomes evident that there are some
rectal wall in front of an injury to the vaginal wall, bands and vessels holding the rectum up in the
leaving sutures in front of sutures, since that is pelvis. These must be separated from the rectum,
considered an obvious predisposing factor for the independently burned and divided in a circumfer-
formation of a rectovaginal fistula. Under such ential manner, continuing until we have enough
circumstances (vaginal injury and a rectal injury), rectal length to create an anastomosis without
one must continue the dissection of the rectum tension.
15.5 Main Repair 217

a b

Fig. 15.15 Perineal body reconstructed. (a) Diagram. (b) Intraoperative diagram

The incision required to repair rectovestibu- stitches must relieve most of the tension of the
lar fistulas includes the opening of the muscle perineal body to be sure that the skin edges in the
complex and part of the levator mechanism. perineal body come together with no tension. We
Sometimes, it is not necessary to open com- close the skin of the perineal body with 6-0 Vicryl
pletely the levator mechanism, and therefore we sutures, only to be sure that the edges of the skin
call this a limited posterior sagittal anorecto- have come together, but those sutures hold no ten-
plasty. However, we are convinced that the size sion. Figure 15.15 shows the repaired perineal
of incision does not affect, in any way, the future body. The rectum then is located within the limits
functional prognosis, provided all of the other of the sphincter immediately behind the perineal
important surgical steps are done correctly. body. The posterior edges of the muscle complex
Once the rectum has been separated from the and levator are sutured together in the midline
vagina and mobilized, in preparation for the using 5-0 long-term absorbable sutures, including
reconstruction, the limits of the sphincter are elec- a bite to the posterior rectal wall to anchor it in
trically determined and marked with temporary normal location (Fig. 15.16). These stitches are
silk stitches. The goal at this stage is to bring aimed to avoid retraction and prolapse. The
together the anterior limits of the sphincter and by ischiorectal fossa, as well as the subcutaneous tis-
doing that to reconstruct the perineal body of the sue, is obliterated using 5-0 long-term absorbable
patient (Fig. 15.15). This is the space that sepa- sutures, and the skin is closed either with subcu-
rates the vagina from the rectum. It is extremely ticular 5-0 monofilament, absorbable, or inter-
important to use strong sutures (5-0 or 4-0 long- rupted 6-0 long-term absorbable sutures.
term absorbable sutures depending on the patient’s The anoplasty is done as previously described,
age) to approximate both sides of the perineal using two layers of interrupted 5-0 or 6-0 long-
body. There, we usually find a fibrous tissue that term absorbable sutures (Fig. 15.17). We try to
surrounded the original vestibular fistula. We use trim off as little as possible rectal tissue, but we
this tissue to anchor our stitches. These deep do not hesitate to remove all of the tissue that is
218 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
Fig. 15.16 Diagram showing sutures taking the posterior vagina, as well as the perineum in these patients,
edges of the muscle complex, including a bite to the pos-
terior rectal wall to anchor it
is remarkably normal looking (Fig. 15.19).

a b

Fig. 15.17 Anoplasty and wound closed. (a) Diagram. (b) Intraoperative picture
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


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
Fig. 15.18 Photograph of a finished operation provoke chronic fecal impaction and overflow

Fig. 15.19 External


appearance 2–3 months
postoperatively
220 15 Rectovestibular Fistula

pseudoincontinence. We usually have a long con- another institution. In fact, from our total series
versation with the parents and explain in detail the of 290 patients with vestibular fistula, 73 of them
importance of taking care of the constipation. We are reoperations. We believe that this is a reflec-
emphasize the fact that the constipation that these tion of the fact that surgeons in general probably
patients suffer from is much more severe than the underestimate the complexity of the repair of this
common idiopathic constipation of the general defect. As previously mentioned, vestibular fis-
pediatric population. The amount of laxatives that tula is by far the most common anorectal malfor-
these patients need sometimes is two, three, four, mation seen in females. The functional prognosis
or five times higher than in other types of patients. in girls when they are born with a good sacrum,
We try to make the parents paranoid against the have no tethered cord, and receive a good opera-
problem of constipation. We also emphasize the tion is excellent. Unfortunately, patients who
fact that the amount of laxatives that these patients underwent a failed attempted repair followed by
require to empty the colon every day cannot be a reoperation do not have the same good func-
predicted. We determine the amount of laxatives tional prognosis. Eighty percent of them have
by trial and error over a period of several days, voluntary bowel movements as compared to
taking abdominal x-ray films to be sure that the 90 % for those operated primarily.
patient empties the colon. If the patients are Probably, the surgeons find it relatively easy to
receiving breast feedings at the time of our opera- imagine that the orifice of the rectum located in
tion, most likely they will not need laxatives until the vestibule could easily be moved back to the
they start decreasing the amount of breast milk normal location of the anus. In reality, the repair
and receiving another type of formula. of this malformation is a delicate and technically
Sexual life in these patients is normal, and as demanding procedure.
we have seen, many of our patients are becoming The most common scenario in dealing with
adults and are getting married. They also can reoperations for vestibular fistulas is a patient
deliver babies vaginally, since we did not actually that was operated without a protective colostomy
injure the vagina which preserves a normal elas- and soon after suffered from dehiscence and
ticity in most of its circumference, since we only retraction of the rectum, followed by opening of
dissected the posterior vaginal wall. the rectum into the posterior vaginal wall. In
In the past, some surgeons [2] claimed that other words, the original malformation was a ves-
these patients could have a normal life without an tibular fistula, but the patient comes with a real
operation or simply doing a “cutback” type of acquired rectovaginal fistula secondary to a poor
procedure to enlarge the anal opening. We have initial operation. During the re-exploration, our
seen that this is not true. First of all, the bowel most common finding in this specific type of
control under those circumstances is rather poor. problem has been an intact common wall between
In addition, when these patients grow up, they the rectum and vagina. In other words, the sur-
feel very unhappy about the fact that they have geons try to repair the malformation but failed to
the anus located immediately behind the vagina separate the rectum completely from the vaginal
with no perineal body. This gives them insecurity wall. They still tried to pull the rectum down
and psychological problems, and in addition, a which was left, we think, under tension, because
vaginal delivery is contraindicated, because it it was still attached to the vaginal wall. As a con-
will produce severe rectal damage. 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
15.8 Reoperations in Patients injured, and therefore when the rectum retracted,
with Vestibular Fistula it reopened into the posterior vaginal wall creat-
ing an acquired vaginal fistula.
From all anorectal defects treated by us, it is the Another common scenario in reoperations for
vestibular fistula type of case that most frequently vestibular fistula is a group of patients that
came to us after a failed attempted repair at 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 patients with vestibular fistula. There was an old
consisted in making a posterior slit in the poste- belief that went from generation to generation
rior edge of the anal opening in the vestibule that by leaving the rectum attached to the vagina,
and suturing it horizontally like a Heineke- as time went by, the perineal body would grow,
Mikulicz type of procedure. That procedure which is definitely not true.
only enlarges the anal opening and leaves the Another finding that is interesting to mention
rectum attached to the vaginal wall with no peri- is the fact that in some of these patients, we found
neal body (Fig. 15.20). We believe that, perhaps, that they had two hemivaginas, and such malfor-
in cases of perineal fistula, the cutback proce- mation was never mentioned in the operative
dure could be considered an acceptable thera- reports of the previous surgeons (Fig. 15.21).
peutic alternative, but we strongly believe that Again, we like to say that “our eyes see only what
this type of operation is contraindicated in our mind suspects.”
222 15 Rectovestibular Fistula

Fig. 15.22 External appearance of the perineum of different patients referred to us, after failed attempted repairs

15.9 Surgical Technique the fact that most of these patients have an intact
common wall between the rectum and vagina,
Reoperations for recurrent or dehiscent, which reflects the fact that the surgeons did an
retracted vestibular fistulas are currently done by incomplete mobilization of the rectum. We go
us without a protective colostomy. Figure 15.22 ahead and make two walls out of one. In other
shows examples of cases that came to us after words, we separate the rectum from the vagina
a failed attempted repair of their malformation. as previously described in the primary proce-
However, we follow the precautions already dure. We must suture the defect of the posterior
mentioned in the chapter related to bowel prepa- vaginal wall. Once the rectum has been com-
ration. We take the baby to the operating room pletely separated, we then mobilize the rectum
with the bowel completely clean. As part of enough to guarantee that an intact anterior rectal
our routine, we perform vaginoscopy and cys- wall is left in front of the vaginal sutures. We
toscopy to rule out the presence of associated are convinced that the vaginal defect can even
defects (mainly vaginal septum). We place the be left unsutured, and it will heal normally pro-
patient in prone position with the pelvis elevated vided the rectal wall left behind is intact. We
and make a posterior sagittal incision following dissect the rectum enough to guarantee that the
the specifications already described. Multiple rectal wall in front of the vagina is completely
5-0 silk stitches are placed at the mucocutane- normal and also to be sure that the anastomo-
ous junction of the rectovaginal fistula or the sis between the rectum and the skin of the anal
rectal opening in order to apply uniform trac- dimple is performed without tension. Before we
tion. Through the posterior sagittal incision, all do the anoplasty, we repair the posterior vaginal
structures are divided in the midline until the wall with long-term absorbable sutures, deter-
posterior rectal wall is identified and then the mine the limits of the sphincter, and continue
dissection of the rectum proceeds, first on the the operation as described for primary cases,
lateral walls and eventually in the common wall reconstructing the perineal body and doing the
between the rectum and the vagina. As previ- anoplasty. The patients remain 10 days fasting
ously mentioned, we have been impressed by and receiving parenteral nutrition.
References 223

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Cloaca, Posterior Cloaca
and Absent Penis Spectrum 16

16.1 Cloaca (a) The first is the possibility of compressing the


trigone of the bladder, producing an extrinsic
16.1.1 Definition and Management ureterovesical obstruction, megaureter, and
hydronephrosis.
A cloaca is a malformation that affects the rec- (b) The second possibility is that the hydrocol-
tum and urogenital tract in females. These girls pos, left undrained, may become infected;
are born with a single perineal orifice. The vagina, creating a pyocolpos that eventually may
urethra, and rectum are fused together inside the perforate, which is a catastrophic event with
pelvis, creating a single common channel that risk of death. In addition, the resulting
opens into a single orifice in the location where inflammation may scar the vagina and impact
the urethra normally opens (Fig. 16.1). The the future reconstruction.
length of the common channel varies from case Approximately 60 % of the patients with clo-
to case, from 1 to about 10 cm with an average of acas also have a double Müllerian system con-
approximately 3 cm. sisting of the presence of two hemiuteri and two
Thirty percent of these patients suffer in addi- hemivaginas [1]. This septation disorder may be
tion from a very dilated vagina full of fluid and/or partial or total. In addition, it can be symmetric or
mucus, called hydrocolpos [1] (Fig. 16.2). The asymmetric. In the asymmetric types, the double
reason why these very dilated vaginas retain fluid Müllerian system phenomenon is frequently
remains a mystery, since they are never really associated with a unilateral atresia of the
atretic. We speculate that there must be some sort Müllerian structure. When this goes unrecog-
of valve mechanism that interferes with the emp- nized, it may produce an accumulation of men-
tying of the fluid. Most of the patients with strual blood at the age of puberty, as well as
hydrocolpos, in addition, have duplicate retrograde menstruation into the peritoneal cavity
Müllerian systems (Fig. 16.3). (Fig. 16.4) which produces rather dramatic signs
The hydrocolpos may produce two important of an acute abdomen and requires an emergency
complications: 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-
Electronic supplementary material Supplementary
material is available in the online version of this chapter at ence of a single perineal orifice. On the very bad
10.1007/978-3-319-14989-9_16. side of the spectrum, one may find patients with a

A. Peña, A. Bischoff, Surgical Treatment of Colorectal Problems in Children, 225


DOI 10.1007/978-3-319-14989-9_16, © Springer International Publishing Switzerland 2015
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, new. We were able to detect and read old publica-