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Pref ace
It is a great pleasure for us to present to the consideration of the pediatric
surgical community this book on the surgical treatment of colorectal problems in children.
From the time of the fi rst description of the posterior sagittal anorectoplasty for the treatment of anorectal malformations in 1982 until the publication of this book, we were able to accumulate a very large series of cases of
anorectal malformations, with no similar precedent. The experience gained
has been invaluable. We wanted to share our experience with all pediatric
surgeons, particularly the young generation. We hope that they will fi nd 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
benefi t many children all over the world.
From the beginning of our experience, we realized that we were confronting a very unusual kind of diffi culty, and that is the fact that we were working
in an anatomical area that was not well known by most pediatric surgeons.
Prior to the posterior sagittal approach, the patients were surgically explored
either through the abdomen or through the perineum with preconceived ideas
about the anatomical area and without a direct exposure to the intrinsic anatomy of these defects. After 1982, the descriptions of our surgical technique
were not like descriptions of any other surgical technique, in which the surgeons already know the anatomy, for instance, the mediastinum or the intraabdominal organs. In dealing with anorectal malformations, we were seeing
for the fi rst time, directly, the anatomy of these defects, and soon, we learned
that we were dealing with a spectrum of malformations and that each specifi c
type of defect would require a different surgical maneuver to be repaired.
In 1982, we presented our “new approach” (posterior sagittal) to one of the
master pediatric surgeons, Dr. Hardy Hendren. He encouraged us to continue
using the approach but emphasized the importance of presenting our technique with better, rather impeccable, audiovisual material in order to be successful in introducing this approach and to gain the acceptance of the pediatric
surgical community.
As a consequence, we have been making a great effort to document our
presentations with high-quality photographic material including videos and
animations. The reader, therefore, will fi nd that this book is a very graphic
one. We believe that it is extremely important to document with good illustrations and photographs all that we have learned. We want this to be essentially
xi
xii
a practical book, a reliable guide for all pediatric surgeons and pediatric
urologists.
A very important motivation for us to write this book is the fact that we
receive many patients who underwent technically defi cient operations in
other parts of the world and suffered serious complications. Therefore, this
book puts particular emphasis on the surgical technique and attention to
details as well as the importance of observing meticulous delicate operations.
Since anorectal malformations represent a spectrum, it is diffi cult for a general pediatric surgeon to be exposed to all the different anatomical variants of
these defects. There is not a single technique to repair all anorectal malformations; each type of defect represents a different anatomical variant, and the
surgeon must be prepared to deal with it. We also wanted to put emphasis on
clarity and simplicity.
All cases discussed in this book were operated by the senior author and by
the junior author during the last 5 years.
In order to make all the concepts more understandable and simple, dealing
with an anatomical area that is not well known by most surgeons, we introduced another modality of teaching, represented by 27 animations, that we
hope will simplify the understanding of the surgical techniques and concepts
expressed in this book.
We are very proud to present an entire book with illustrations made by a
single person. All illustrations in this book were made by Ms. Lois Barnes
under the personal guidance of the senior author. Ms. Barnes is an excellent
medical illustrator and old friend with whom we have been working for
30 years. It took many years to establish a unique form of communication
between the medical illustrator and us. Due to the large number of illustrations that she performed under our guidance, it is a great pleasure nowadays
to have a common language between a surgeon and an artist. We speak the
same language. Therefore, the illustrations made in the last few years represented much less effort from both surgeons and the illustrator.
The animations required, again, many hours of working together with
ingenious, intelligent experts in computer animations and establishing a communication between a surgical mind and an engineer, computer expert type of
mind. We believe that in the future, teaching surgery will be highly simplifi ed
with this kind of audiovisual material.
The reader will fi nd that even though the book is related to the surgical
treatment of colorectal problems in children, we did not include infl ammatory bowel disease. The reason for that is simple: we do not have enough
experience as to be able to say something new and (or) different from what is
already written in the enormous literature on the subject.
The reader will also be surprised to fi nd that even though this book was
fi nished in November 2014, it is not considered an “evidence-based surgical
book.” It is rather a book based on personal observations made through a
large experience in the management of these problems. We understand very
well that the future in surgery will be related very much to the concept of
“evidence-based” procedures. However, in dealing with anorectal malformations, some surgeons have tried to compare the results of the posterior sagittal
anorectoplasty with other techniques; they found a serious limitation: the
Preface
Preface
xiii
series are not nearly comparable. The number of cases that we accumulated
over the last 30 years has no precedent. If one tries to compare this technique
with others, we fi nd the problem that the numbers are 10 or 100 times greater
in our series. In addition, most of the publications on anorectal malformations, even in the year 2014, unfortunately are still presented following the
old nomenclature discussing “high,” “intermediate,” and “low” malformations. This makes it impossible to compare the results. Through the entire
book, we emphasize the importance of recognizing the existence of different,
specifi c types of defects, each one requiring different surgical maneuvers and
each one with a different functional result. In addition, we keep emphasizing
the importance of describing the characteristics of the sacrum and the anomalies of the spinal cord if we want to discuss results. Trying to compare our
results with old techniques is an impossible task. We are more concerned
with trying to be sure that all new generations of pediatric surgeons learn the
real, true, intrinsic anatomy of anorectal malformations and learn to repair
these malformations in an optimal way.
Finally, we would like very much to be able to transmit to the young generation of pediatric surgeons our passion for delicate, meticulous, fi ne surgical technique, which is the essence of our specialty. There is no other medical
or surgical specialty as curative as pediatric surgery. We become pediatric
surgeons because we like the idea that we could repair with our hands a serious congenital malformation and change the quality of life of a baby. It is
extremely important for us to recognize that a mistake in the management of
these patients will leave sequelae for life.
This book also puts a special emphasis on the detailed repair of those malformations that are considered to have a good functional prognosis. One thing
that we cannot afford is to take care of a child with a malformation with a
good functional prognosis, perform a technically defi cient surgical procedure, 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 prominent daily participation in our task. We want to express our gratitude to hundreds of surgeons, not mentioned here, from all over the world who trusted us
to operate on their little patients. They made it possible for us to accumulate
the experience that we want to share with the new generation. We are sure
that we have missed many important names of individuals that contributed to
fi nish 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 Calcifi cations . . . . . 30
3.2.2 Pelvic Cystic Mass . . . . . . . . . . . . . . . . . . . . . . . . . . . 30
3.2.3 Cloacal Exstrophy . . . . . . . . . . . . . . . . . . . . . . . . . . . 30
References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 31
4 Neonatal Management . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 33
4.1 Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 33
4.2 Most Common Scenario . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 33
4.3 Answering the Two Most Important Questions . . . . . . . . . . . 33
4.4 Physical Examination . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 35
4.4.1 Male Patients . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 35
4.5 Female Babies . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 39
4.6 Neonatal Management . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 41
4.7 Cloacal Exstrophy . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 46
References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 47
xvii
xviii
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
Contents
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 Defi nition, Frequency, and Prognosis . . . . . . . . . . . . . . . . . 107
8.2 Associated Defects . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 109
8.3 Diagnosis . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 110
8.3.1 Female Patients . . . . . . . . . . . . . . . . . . . . . . . . . . . . 110
8.3.2 Male Patients . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 110
8.4 Management . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 117
8.5 Dilatations . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 117
8.6 Cutback Operation . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 117
8.7 Minimal Posterior Sagittal Anoplasty . . . . . . . . . . . . . . . . . 118
8.7.1 Male Patients . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 118
8.7.2 Surgical Technique . . . . . . . . . . . . . . . . . . . . . . . . . 119
8.7.3 Female Patients . . . . . . . . . . . . . . . . . . . . . . . . . . . 122
8.8 Postoperative Care . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 123
References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 125
9 Rectourethral Bulbar Fistula . . . . . . . . . . . . . . . . . . . . . . . . . . . . 129
9.1 Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 129
9.2 Associated Defects . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 129
9.3 Posterior Sagittal Anorectoplasty . . . . . . . . . . . . . . . . . . . . . 132
9.4 Surgical Technique . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 132
9.5 Functional Results . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 148
References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 148
10 Rectourethral Prostatic Fistula . . . . . . . . . . . . . . . . . . . . . . . . . . 151
10.1 Introduction . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 151
10.2 Associated Defects . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 151
10.3 Surgical Repair . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 153
10.4 Posterior Sagittal Anorectoplasty . . . . . . . . . . . . . . . . . . . . . 153
10.5 Postoperative Care and Functional Results . . . . . . . . . . . . . 161
References . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 161
11 Recto-bladder Neck Fistula . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 163
11.1 Defi nition and Frequency . . . . . . . . . . . . . . . . . . . . . . . . . . . 163
11.2 Associated Defects . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . 164
11.2.1 Sacral Defects. . . . . . . . . . . . . . . . . . . . . . . . . . . . . 164
11.2.2 Spinal-Associated Defects . . . . . . . . . . . . . . . . . . . 164
11.2.3 Urologic-Associated Defects . . . . . . . . . . . . . . . . . 164
11.2.4 Gastrointestinal-Associated Defects . . . . . . . . . . . 165
11.2.5 Neurosurgical-Associated Defects . . . . . . . . . . . . . 165
11.2.6 Cardiovascular-Associated Defects . . . . . . . . . . . . 165
11.2.7 Other Associated Defects . . . . . . . . . . . . . . . . . . . . 165