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- •Foreword I
- •Foreword II
- •Preface
- •Acknowledgments
- •Contents
- •1: History of the Treatment of Anorectal Malformations
- •1.1 Introduction
- •1.2 The Early Times
- •References
- •2: Basic Anatomy and Physiology of Bowel Control
- •2.1 Internal Sphincter
- •2.2 General Anatomic Principles in Anorectal Malformations
- •2.3 Nerves
- •2.4 Blood Supply
- •2.5 Basic Physiology Principles of Bowel Control
- •References
- •3: Prenatal Diagnosis
- •3.1 Male Fetuses
- •3.1.1 Abnormal Sacrum (Fig. 3.3)
- •3.1.2 Tethered Cord
- •3.1.3 Absent Kidney (Fig. 3.5)
- •3.1.4 Vertebral Anomalies
- •3.1.5 Hydronephrosis (Fig. 3.6)
- •3.2 Female Fetuses
- •3.2.2 Pelvic Cystic Mass
- •3.2.3 Cloacal Exstrophy
- •References
- •4: Neonatal Management
- •4.1 Introduction
- •4.2 Most Common Scenario
- •4.4 Physical Examination
- •4.4.1 Male Patients
- •4.5 Female Babies
- •4.6 Neonatal Management
- •4.7 Cloacal Exstrophy
- •References
- •5: Colostomy
- •5.1 Introduction
- •5.2 Stoma Locations
- •5.3 Ileostomies
- •5.4 To Divert or Not to Divert, That Is the Question
- •5.5 Recommended Types of Colostomies
- •5.5.1 Newborn Babies with Anorectal Malformations
- •5.6 Left Transverse Colostomy
- •5.7 Cecostomies
- •5.8 Creation of a Colostomy
- •5.8.1 Surgical Technique
- •5.9 Colostomy in Cases of Cloaca with Hydrocolpos
- •5.10 Other Types of Colostomies
- •5.11 Colostomy Care
- •5.12 Colostomy Closure
- •5.13 Surgical Technique
- •5.14 Errors and Complications in Colostomies
- •5.16 Prolapse
- •5.17 Surgical Treatment for Prolapse
- •5.18 Malposition of the Stomas
- •References
- •6: Imaging
- •6.1 Introduction
- •6.2 Prenatal Diagnosis
- •6.3 Neonatal Imaging
- •6.4.1 Anatomic Facts and Timing
- •6.5 The Old Invertogram
- •6.6 High-Pressure Distal Colostogram
- •6.7 Technique
- •6.8 Most Common Errors
- •6.9 Not Showing the Coccyx and the Sacrum During the Fluoroscopy Studies
- •6.11 Distal Colostogram in Cloacas
- •6.12 Monitoring Constipation
- •6.13 Radiology During the Bowel Management Program
- •6.14 Monitoring the Urinary Tract
- •References
- •7: Bowel Preparation in Pediatric Colorectal Surgery
- •7.1 Major Procedures
- •7.2 Primary Procedures for the Treatment of Anorectal Malformation During the Newborn Period
- •7.3 Primary Pull-Through in Newborn Patients with Hirschsprung’s Disease
- •7.4 Patients with Hirschsprung’s Disease with Enterocolitis After the Neonatal Period
- •7.5 Patients with Hirschsprung’s Disease Beyond the Neonatal Period, Without Enterocolitis
- •7.6 Colostomy Closures
- •References
- •8: Recto-perineal Fistula
- •8.2 Associated Defects
- •8.3 Diagnosis
- •8.3.1 Female Patients
- •8.3.2 Male Patients
- •8.4 Management
- •8.5 Dilatations
- •8.6 Cutback Operation
- •8.7 Minimal Posterior Sagittal Anoplasty
- •8.7.1 Male Patients
- •8.7.2 Surgical Technique
- •8.7.3 Female Patients
- •8.8 Postoperative Care
- •References
- •9: Rectourethral Bulbar Fistula
- •Introduction
- •Associated Defects
- •Posterior Sagittal Anorectoplasty
- •Surgical Technique
- •Functional Results
- •References
- •10: Rectourethral Prostatic Fistula
- •10.1 Introduction
- •10.2 Associated Defects
- •10.3 Surgical Repair
- •References
- •11: Recto-bladder Neck Fistula
- •11.2 Associated Defects
- •11.2.1 Sacral Defects
- •11.2.2 Spinal-Associated Defects
- •11.2.3 Urologic-Associated Defects
- •11.2.5 Neurosurgical-Associated Defects
- •11.2.6 Cardiovascular-Associated Defects
- •11.2.7 Other Associated Defects
- •11.3 Diagnosis
- •11.4 Treatment
- •11.4.1 Colostomy
- •11.4.2 Main Repair
- •11.4.3 Laparotomy
- •11.4.4 Laparoscopy
- •11.5 Special Problems
- •11.6 Functional Results
- •11.6.1 Fecal Control
- •11.6.2 Urinary Control
- •References
- •12: Imperforate Anus Without Fistula in Males and Females
- •12.1 Introduction
- •12.2 Anatomic Characteristics
- •12.3 Main Repair
- •12.4 Function and Results
- •References
- •13: Minimally Invasive Approach to Anorectal Malformations
- •13.1 Introduction
- •13.2 Males
- •13.3 Females
- •References
- •14: Rectal Atresia
- •14.1 Treatment
- •14.2 Surgical Repair
- •References
- •15: Rectovestibular Fistula
- •15.2 Associated Defects
- •15.2.1 Sacral
- •15.2.2 Spinal
- •15.2.3 Urologic
- •15.2.4 Gynecologic
- •15.2.5 Gastrointestinal
- •15.2.6 Tethered Cord
- •15.2.7 Cardiovascular
- •15.3 Diagnosis
- •15.4 Treatment
- •15.4.1 Colostomy or No Colostomy
- •15.5 Main Repair (Animation 15.1)
- •15.6 Complications
- •15.7 Functional Results
- •15.9 Surgical Technique
- •References
- •16: Cloaca, Posterior Cloaca and Absent Penis Spectrum
- •16.1 Cloaca
- •16.1.1.1 Associated Defects
- •16.1.1.2 Goals of Treatment
- •16.1.1.3 Neonatal Management
- •16.1.1.4 Main Repair
- •Cloacas with a Common Channel of Less Than 1 cm
- •Cloacas with a 1–3 cm Common Channel
- •Cloacas with a 3- to 5-cm Common Channel (Animation 16.3)
- •Carving of the Pubic Cartilage Maneuver
- •Separations of Vagina(s) from the Urinary Tract (Animation 16.3)
- •Vaginal Switch
- •Vaginal Replacement
- •Vaginal Replacement with Rectum
- •Vaginal Replacement with Colon
- •Vaginal Replacement with Small Bowel
- •Cloacas with Extremely Long Common Channels
- •16.1.1.5 Postoperative Care
- •16.1.2 Urologic Concerns
- •16.1.3 Gynecologic Concerns
- •16.1.4 Reoperations
- •16.1.4.1 Persistent Urogenital Sinus
- •16.1.4.3 Acquired Urethral Atresia or Stricture
- •16.1.4.4 Sequelae from Catastrophic Complications
- •16.1.5 Transpubic Approach
- •16.2 Posterior Cloaca and Absent Penis Spectrum
- •16.2.1 Surgical Repair
- •References
- •17: Cloacal Exstrophy and Covered Cloacal Exstrophy
- •17.1 Neonatal Approach
- •17.2 Pull-Through or “Permanent Stoma”
- •17.3 Covered Cloacal Exstrophy
- •References
- •18: General Principles for the Postoperative Management of Patients with Anorectal Malformations
- •18.1 General Care
- •18.2 Local Care
- •18.3 Anal Dilatations
- •18.4 Avoiding Constipation
- •18.5 Toilet Training
- •19: Postoperative Evaluation
- •References
- •20: Bowel Management for the Treatment of Fecal Incontinence
- •20.1 Introduction
- •20.2 Goals of the Bowel Management Program
- •20.3 Evaluation of the Patient for Bowel Management
- •20.5 Laxative Trial
- •20.6 About Our Program
- •20.7 Content of the Enema
- •20.8 Rationale to Change the Type of Enema
- •20.9 Bowel Management for the Treatment of Severe Diaper Rash
- •20.10 Bowel Management Through a Stoma
- •References
- •21: Operations for the Administration of Antegrade Enemas
- •21.1 Introduction
- •21.2 Our Preferred Technique
- •21.4 Continent Neo-appendicostomy
- •References
- •22: Reoperations
- •22.1 Introduction
- •22.4.1 Recurrent Fistula (17 Cases)
- •22.4.2 Persistent Rectourethral Fistula (24 Cases)
- •22.4.3 Acquired Fistula (9 Cases)
- •22.5 Posterior Urethral Diverticulum (32 Cases)
- •22.6 Acquired Rectal Atresia or Stenosis (83 Cases)
- •22.7 Presacral Masses
- •22.9 Prolapse
- •References
- •23: Urologic Problems in Anorectal Malformations
- •23.1 Introduction
- •23.2 Neonatal Approach
- •23.4 Most Common Urologic Abnormalities in Male Patients with Anorectal Malformations
- •23.4.1 Absent Kidney
- •23.4.2 Urethral Problems
- •23.6 Hypospadias
- •23.7 Ectopic Ureters in Males
- •23.8 Ectopic Ureters in Females
- •23.9 Ectopic Vas Deferens
- •23.10 Ectopic Verumontanum
- •23.11 Megalourethra
- •23.13 Neurogenic Bladder
- •23.14 Postoperative Problems
- •23.16 Sexual Problems
- •23.17 Tethered Cord
- •23.18 The Ultimate Concern, Kidney Function
- •References
- •24: Hirschsprung’s Disease
- •24.1 Introduction
- •24.2 Historical Review
- •24.3 Incidence, Inheritance, and Associated Anomalies
- •24.4 Pathogenesis
- •24.5 Genetics
- •24.6 Clinical Manifestations and Differential Diagnosis
- •24.7 Histologic Diagnosis
- •24.8 Differential Diagnosis
- •24.9 Early Management
- •24.10 Surgical Treatment
- •24.10.1 The Authors’ Approach
- •24.11 Total Colonic Aganglionosis
- •24.13 Problems, Complication, and Sequela Secondary to Operations for Hirschsprung’s Disease
- •24.13.1.1 Fecal Incontinence
- •24.13.2 Non-preventable Complications
- •24.13.3 Partially Preventable Complications
- •References
- •25: Idiopathic Constipation and Other Motility Disorders
- •25.2 Incidence, Social Impact, and Relevance
- •25.3 Etiology
- •25.3.2 Rectal Manometry
- •25.3.5 Botulinum Toxin Injection
- •25.4 Pathogenesis
- •25.5 Natural History and Clinical Manifestations
- •25.6 Diagnosis
- •25.6.1 Colonic Transit Time
- •25.6.2 The Evaluation of Severity: Search for Objective “Instruments”
- •25.7 Management
- •25.7.3 Electric Stimulation
- •25.8 Surgical Treatment
- •25.8.2 Colonic Resection
- •References
- •26: Posterior Sagittal Approach for the Treatment of Other Conditions
- •26.1 The Kraske Operation
- •26.2 Urogenital Sinus with Normal Rectum
- •26.3 Urogenital Sinus with Normal Rectum and Adrenal Hyperplasia
- •26.4 Acquired Urethral Atresia
- •26.5 Acquired Rectourethral Fistula
- •26.6 Giant Seminal Vesicle
- •26.7 Urethral Tumors
- •26.8 Acquired Rectovaginal Fistula
- •26.9 Rectal Tumors
- •26.10 Presacral Masses
- •26.11 Surgical Technique
- •26.12 Posterior Sagittal Approach, Its Application in Cases with Hirschsprung’s Disease
- •26.13 Vaginal Atresia with Normal Rectum
- •References
- •27: Miscellaneous Conditions
- •27.1 Part I: Perianal Abscess and Fistula

6
1 History of the Treatment of Anorectal Malformations
These ideas, like in many other historic events,
illustrate how naive we tend to be. Therefore, we
like to say that every time we try to cheat on
Mother Nature, she teaches us a lesson.
In 1930, Owen Wangensteen and Carl Rice
published a paper describing a method of radiologically determining the height of the blind rectal
end to select the best surgical approach for patients
with anorectal malformations [ 33 ]. The technique
that they described is well known as an “invertogram.” It consisted of putting the newborn baby
upside down for several minutes and taking an
x-ray fi lm of the pelvis to determine the location of
the blind end of the rectum, as well as the distance
from the blind end of the rectum to the anal skin.
The blind end of the rectum can be seen because it
is full of gas. That method still has some value.
However, we use a variation of it in less than 5 %
of all cases, in those in whom there is no clinical
evidence of the location of the distal rectum. Yet,
we have learned through the years that the same
image that Wangensteen and Rice were able to
obtain with the invertogram can be achieved by
placing the patient in prone position with the pelvis elevated and taking a cross- table, lateral fi lm.
We have learned many lessons from the external examination of the perineum of the babies, as
well as other more sophisticated imaging
methodology.
In 1934, William Ladd and Robert E. Gross
[ 34 ] published a very comprehensive series of
cases. Their publication also included good
embryologic description. They also included a
detailed table of associated malformations. This
is extremely important since, as the reader will be
able to see in this textbook, the frequency of the
associated defects in cases of anorectal malformations is very signifi cant and those associated
defects have a vital role in the prognosis of these
patients. Ladd and Gross’s publication is a beautiful one; it has very elegant drawings done personally by Dr. Robert Gross, illustrating the
development of female malformations. The mortality in their series was 26 %.
In 1936, Stone [ 35 ] published a paper entitled
“Imperforate Anus with a Rectovaginal Cloaca.”
In 1938, J. K. Berman [ 36 ] published a paper
on 23 cases of anorectal malformations with
47 % mortality. He opposed the use of colostomies in his patients because of its high mortality
and proposed an incision running from the perineal body to the coccyx in newborns, with local
anesthesia. He used 0 size chromic catgut. He
described only the pull-through of the bowel,
leaving the fi stula to the urinary tract untouched
until the patient was older!!
In 1948, Rhoads et al. [ 37 ] (Fig. 1.1 ) pub-
lished their experience with the fi rst survivor of a
primary abdominoperineal pull-through, without
a colostomy. After that publication, many surgeons tried to perform that kind of operation,
sometimes with success, but many other times
with serious catastrophic results, and therefore,
years after that, this approach was reconsidered.
Fig. 1.1 Photograph –
Dr. Peña with Dr. Jonathan
Rhoad

1.2 The Early Times
7
Lately, many others have been trying to approach
newborn babies primarily without a colostomy.
As will be seen in this textbook, that approach is
sometimes justifi ed, but not always.
In 1953, Douglas Stephens published his fi rst
landmark paper on the subject, in Australia [ 38 ].
Dr. Stephens has the unique distinction of being
the fi rst person who studied the anatomy of the
pelvis in patients who died from an anorectal malformation. From his studies, he concluded that the
key part of the sphincter mechanism to achieve
bowel control in these cases was the “puborectalis
sling.” It took time for his concept to be learned
and accepted by the world community of pediatric
surgeons, but within a few years, most pediatric
surgeons recognized that was something to be
considered seriously, and therefore, the “era of the
puborectalis” began. From that time, most surgeons tried to design operations aimed to preserving the “puborectalis sling,” which was considered
key for bowel control. Unfortunately, it is not easy
to obtain cadavers of children born with anorectal
malformations because most children with anorectal malformations survive, and therefore the
number of specimens studied by Dr. Stephens was
very limited. In retrospect, we believe that his
conclusions are not valid because his studies were
performed in a limited number of the most severe
cases, not representative of what we call the spectrum of anorectal malformations. The cases
(cadavers) that he studied we think are not representative of the most common types of malformations that we see. Yet, one of his recommendations
is still valid: he recommended pulling the bowel
down, as close as possible to the urethra. In addition, Dr. Douglas Stephens published a book [
2 ]
that represents the document with the largest
amount of information related to the subject of
anorectal malformations at that time.
In 1955, Sir Denis Browne, a prominent surgeon from Great Ormond Street Hospital in
London, proposed that patients with rectovaginal
fi stulas had a normal sphincter located at the
vagina site [ 39 ]. We now know that that never
happens. He also suggested that female patients
with vaginal, as well as males with rectoprostatic,
fi stulas have no sphincter mechanism, which, as
we know now, is mostly inaccurate.
In 1954, Dr. William Potts [
40 ] published a
paper related to the treatment of 22 “rectovaginal
fi stulas,” 8 rectourethral fi stulas, 9 recto-perineal
fi stulas, and 12 rectovesical fi stulas. The anoplasty that he proposed for the most common
type of malformation seen in females that we
now know by the name of rectovestibular fi stula
is still known as the “Potts’ anoplasty” and consists of dissecting the rectum from the vestibule
and passing it behind a bridge of skin, to be
placed within the limits of the sphincter. In retrospect, now we believe that what he described as
“rectovaginal fi stulas” were actually cases of rectovestibular fi stulas, since now we recognize that
real rectovaginal fi stulas are extremely unusual
malformations. Unless we postulate the theory
that the type of pathology changes through the
years, it is diffi cult to believe that surgeons had
many cases of rectovaginal fi stulas, which we
fi nd now to be a malformation that is almost
nonexistent.
In 1960, Scott, Swenson, and Fisher published
one of the fi rst papers on long-term follow-up
results. Their patients suffered from a mortality
of 12.7 %; 4.8 % was operative deaths. In their
study, they reported 68 % incontinence in patients
with the so-called “high” malformations and
89 % good results in what were described as
“low” malformations [ 41 ].
In 1963, Kiesewetter et al. [ 42 ] reported
their experience with 146 patients followed
over a period of 16 years. These surgeons had a
demonstrated special interest in the management of anorectal malformations. Their mortality was 19.2 %; 86 cases were followed on a
long-term basis. The authors divided their
series into “high malformations,” where the
rectum was located two centimeters above the
anal skin, and “low malformations,” for those
where the rectum was closer to the skin. They
obtained 72 % “good results” in “low malformations” and 45 % “bad results” in “high malformations.” They mention that it was important
to preserve, as much as possible, the distal part
of the bowel, which is a concept that we support
at the present time. They reported 24.4 % of the
cases having vestibular fi stulas, which is similar to what we report.

8
1 History of the Treatment of Anorectal Malformations
In 1966, Dr. Kiesewetter [ 43 ] supported the
idea proposed by Stephens that the puborectalis
muscle was the only available muscle useful to
achieve bowel control. He adopted Stephens’
idea of a sacral incision to preserve the puborectalis sling. Through that incision, a blind tunnel
was created, behind the urethra (in males). A
Penrose drain was then passed through the tunnel. The rectum was then to be pulled down
through that tunnel. He also adopted the principle
of a transabdominal endorectal dissection in
order to try to avoid damage to the innervation of
the pelvic organs. This is the same principle that
Soave and Boley proposed for the treatment of
Hirschsprung’s disease. Kiesewetter’s operation
was called a sacro-abdominoperineal endorectal
pull-through.
In 1967, Dr. Rehbein [ 44 ] also proposed an
abdominal sacroperineal procedure. He presented
70 cases, 55 males and 15 females. He emphasized the importance of preserving the “puborectalis muscle.”
We want to express our recognition to many
prominent surgeons from all over the world who
demonstrated special interest and dedication to
the fi eld of anorectal malformations. We are
unable to mention all of them; therefore, we
selected the most prominent ones including:
• Santull [ 45 ], Hanley [ 46 ], Lynn [ 47 ], Partridge
[ 48 ], Trusler [ 49 ], Cozzi [ 50 ], Soave [ 51 ],
Louw [ 52 ], Knutrud [ 53 ], Nixon and Puri
[ 54 ], Smith [ 55 ], Holschneider [ 56 ], Varma
[
57 ], Chatterjee [ 58 ], Rintala [ 59 ], Endo [ 60 ],
Scharli [ 61 ], Ito [ 62 ], Brayton [ 63 ], Aluwihare
[ 64 ], and Banu [ 65 ]
In 1970, in Melbourne, an international com-
mittee was created to design an “international
classifi cation of anorectal malformations.” That
“international classifi cation” was adopted by all
of the pediatric surgeons in the world, but
because of its complexity, it was not used in the
everyday practice by most pediatric surgeons
(Fig. 1.2 ) [ 66 ].
In 1971 [ 67 ], the American Academy of
Pediatrics proposed a joint effort between different institutions to learn about the different therapeutic modalities as well as the results of the
treatment of anorectal malformations and sent
questionnaires to the members of the surgical
section of the academy. From the answers, they
were able to put together 1,116 patients from 51
institutions, 58 % were males and 42 % females.
The incidence of fi stula in males was 72 % and in
females was 19 %, and 28.7 % of these fi stulas
were “rectovaginal.” This is something that we
know was most likely a misnomer or lack of
accurate examination of the genitalia because, as
we mentioned, congenital rectovaginal fi stulas in
our experience are almost nonexistent; 81 % of
the patients with a “high malformation” received
a colostomy, and 62 % of those colostomies were
done in the transverse colon. The mortality
reported was 19 %.
During the years of 1969–1970, the senior
author had the privilege of meeting Dr. Justin
Kelly. Dr. Kelly, a fully trained pediatric surgeon
from Melbourne, Australia, had been greatly
infl uenced by Dr. Douglas Stephens. Dr. Kelly
went to Boston Children’s Hospital as a clinical
fellow for two extra years. There, he lectured and
communicated the ideas of Dr. Stephens to all
members of the surgical staff. Dr. Peña (senior
author) was at Boston Children’s Hospital as a
research fellow in 1969 and as a resident in 1970
and 1971. During that time, he learned from Dr.
Kelly’s, Dr. Stephen’s, and Dr. Smith’s ideas
about the management of anorectal malformations. Upon his return to Mexico City, Dr. Peña
became the Chief of Surgery at the New National
Institute of Pediatrics.
From 1972 to 1980, the senior author of this
book operated, in Mexico City, on 56 cases of
the so-called, at that time, “high” imperforate
anus. He followed the principles proposed by
Stephens and learned from Dr. Kelly (sacral
approach) and laparotomy when necessary as
proposed by Kiesewetter, and Rehbein (sacroabdominoperineal pull-through). During those
8 years, he became aware of the fact that, in the
earlier cases, he was opening the abdomen very
often (80 % of the time) to repair these malformations, but after 8 years of experience, he was
opening the abdomen only 20 % of the time. He
attributed this to the fact that the sacral incision
that Stephens originally proposed (very small)
was being gradually enlarged throughout those

1.2 The Early Times
9
High deformities
1. Anorectal agenesis
a. Without fistula
Ano-rectal agenesis (No fistula)
b. With fistula
i. Recto-vesical fistula
ii. Recto-urethral fistula
2. Rectal atresia
Intermediate deformities
1. Anal agenesis
a. Without fistula
Anal Agenesis (no fistula)
b. With fistula
Recto-bulbar fistula
2. Ano-rectal stenosis
Low deformities (translevator)
1. At normal anal site
i. Covered anus - complete
ii. Anal stenosis
Covered anal stenosis
2. At perineal site
i. Anterior Perineal Anus
ii. Ano-cutaneous fistula
(Covered anus – incomplete)
Miscellaneous deformities
i. Imperforate anal membrane
ii. Anal membrane stenosis
iii. Vesico-Intestinal Fissure
iv. Duplications of the anus, rectum and genitourinary tracts
v. Combination of deformities
Melbourne classification, 1970
Males
Melbourne classification, 1970
High deformities
1. Anorectal agenesis
a. Without fistula
b. With fistula
i. Recto-vesical fistula
ii. Recto-cloacal fistula
iii. Recto-vaginal fistula
2. Rectal atresia
Intermediate deformities
1. Anal agenesis
a. Without fistula
Anal Agenesis (no fistula)
b. With fistula
i. Recto-Vaginal fistula
ii. Recto-vestibular fistula
2. Ano-rectal stenosis
Low deformities (translevator)
1. At normal anal site
i. Covered anus - complete
ii. Anal stenosis
Covered anal stenosis
2. At perineal site
i. Anterior Perineal Anus
ii. Ano-cutaneous fistula
(Covered anus – incomplete)
3. At vulvar site
i. Vulvar anus
ii. Ano-vulvar fistula
iii. Ano-vestibular fistula
Miscellaneous deformities
i. Imperforate anal membrane
ii. Anal membrane stenosis
iii. Vesico-Intestinal Fissure
iii. Perineal groove
iv. Perineal canal
v. Vesico-intestinal fissure
vi. Duplications of the anus, rectum and genitourinary tracts
vii. Combination of deformities
Females
Fig. 1.2 International classifi cation of anorectal malformation. Melbourne, Australia 1971
8 years, becoming longer and longer. In addition,
he decided to use an electrical stimulator with the
specifi c purpose to identify the “puborectalis
sling.” Eventually, he felt the need to divide part
of the sphincter mechanism that was visible during this operation. He thought that this muscle
mechanism must have been the “puborectalis
sling.” He specifi cally proposed to divide part of
what he thought was the “puborectalis muscle” to
have better exposure and to facilitate the separation of the rectum from the urinary tract. He
decided to present that experience of 56 cases at
the annual meeting of the Pacifi c Association of
Pediatric Surgeons in March 1980 in Colorado
Springs, Colorado, United States. Basically, in
this presentation, he proposed to make a longer
midsagittal incision to have better exposure to
facilitate the separation of the rectum from the
urinary tract in male patients and to avoid the
need of a laparotomy. He also proposed the use of
an electrical stimulator to identify the sphincter
mechanism and also to divide in the midline the
muscle that he found, in order to facilitate, again,
the dissection of the fi stula and proposed to resuture the muscle behind the rectum at the end of
the procedure. This was basically a step prior to
the full posterior sagittal anorectoplasty proposed
later. The paper provoked a signifi cant discussion, particularly between the Australian members of the audience. They specifi cally mentioned
that the muscle that was shown in the movie in
that presentation was not the “puborectalis sling”
because that structure was only seen in Australia,
by Dr. Stephens, in autopsies. In other words, for
years, the world’s pediatric surgical community
had been talking about how to preserve the

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

1.2 The Early Times
Fig. 1.3 Picture of the group of pediatric surgeons who met in Wingspread
11
over the anatomy of the sphincter mechanism,
seen in retrospect, appears today of little signifi cance, compared to the fascinating observations
that emerged from the surgical exploration, evaluation, and long-term follow-up of so many
patients.
The road has been long, exciting, wonderful,
and illuminating. We have been learning many
important lessons, and now we are certain that
the more we learn about the subject, the more
intriguing questions we must answer. The dictum
that “It’s not the unanswered questions, but rather
the unquestioned answers” crystallizes well the
evaluation of this problem in surgery. This has
been a lifetime, very enjoyable, fascinating, humbling, and extraordinary experience.
Opening the pelvis posterior-sagittally
allowed us, for the fi rst time, to be directly
exposed to the peculiar, complex, intrinsic anatomy of these defects. Much beyond the anatomic
characteristics of the sphincter mechanism, we
learned about the detailed anatomy of the junction between the rectum and the urogenital tract.
With that knowledge came the awareness of the
potential damage that we could provoke while
trying to separate on these structures blindly.
Suddenly, we had an explanation for the many
older patients who had come to our clinic who
were born with an anorectal malformation and
were subjected to blind or semi-blind operations
that resulted in a urethral stricture and/or acquired
urethral atresia, neurogenic bladder, impotence,
retrograde ejaculation, painful ejaculation, and
many other problems.
Now we know that the separation of the rectum from the urogenital tract under direct vision
is a technically demanding maneuver; it is therefore easy to understand how much damage we
could provoke doing the operation blindly, and
sadly that is what we were doing prior to 1980.
Being directly exposed to the intrinsic anatomy of these malformations only made more
obvious our naivety when trying to create
oversimplifi ed classifi cations of a spectrum of
defects, without really knowing the true anatomy.
The most conspicuous lesson learned through all
these years is that we have been dealing with a
spectrum of defects. The more cases we operate
on, the wider the spectrum becomes.
Classifi cations that divide a spectrum of defects,
in categories such as “high” and “low” or even
“high,” “intermediate,” and “low,” represent
oversimplifi cations that misguide rather than
help. Now we know that in surgery, it is not

12
Fig. 1.4 Wingspread
classifi cation of anorectal
malformation
1 History of the Treatment of Anorectal Malformations
advisable to use radical terms such as “always”
or “never.” Being exposed directly to the anatomy
of these malformations also gave us a unique
opportunity to correlate the anatomic fi ndings
with the fi nal functional results and the potential
devastating functional sequelae.
Confronted with an anatomy never described
before, we were obligated, by common sense, to
describe technical maneuvers never described
before. Many remarkable experiences were waiting ahead of us.
In 1982, Dr. Maricela Zarate, an ex-resident of
Dr. Peña in Monterrey, N.L., Mexico, had a
patient with a cloaca. The available literature at
that time related to the surgical treatment of cloaca was very scant (see Chap. 16 ).
In 1982 in Monterrey, Mexico, Dr. Peña, Dr.
Maricela Zarate, and Dr. Marshall Schwartz (visiting from the United States) operated via posterior sagittal on a girl with a cloaca (Fig. 1.7 ). That
girl is now an adult, has bowel and urinary control, 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)
Major clinical groups
Perineal (cutaneous) fistula
Rectourethral fistula
Prostatic
Bulbar
Rectovesical fistula
Vestibular fistula
Cloaca
No fistula
Anal stenosis
Fig. 1.6 Krickenbeck classifi cation of anorectal
malformation
Rare/regional variants
Pouch colon
Rectal atresia/stenosis
Rectovaginal fistula
H fistula
Others
technology. Willital [
repair an anorectal malformation using this therapeutic modality. In the year 2000, Dr. Keith
Georgeson published his experience with seven
cases, in a detailed description of the operation
[ 73 ]. Following Georgeson, many other surgeons
have been using minimally invasive techniques to
repair anorectal malformations (see Chap.
In July 1985, the senior author moved to Long
Island, New York, United States, and became
Chief of Pediatric Surgery at Schneider Children’s
Hospital until June 30, 2005. During those
protects the innocent,” since that case from
Monterrey, seen in retrospect, after having operated on over 531 patients with a cloaca, we now
belongs to the “good side of the spectrum” of cloacas. Later on, we would be confronted with
much more challenging cases. To repair those
complex cases would require a great deal of
creativity, imagination, and dedication.
A very important positive development occurred
in the fi eld of colorectal problems of children and
that is the introduction of the minimally invasive
20 years, we were able to accumulate a very large
experience and moved forward in our attempt to
benefi t more children. We were able to:
• Find further applications for the posterior sagittal approach [ 74 , 75 ]
• Describe the transanorectal approach for the
treatment of urogenital sinus with normal rectum and other conditions [ 76 , 77 ].
• Create a protocol of medical and surgical
management of patients with idiopathic constipation [ 78 ].
72 ] published his attempt to
13 ).

14
1 History of the Treatment of Anorectal Malformations
Fig. 1.7 Picture taken during the fi rst posterior sagittal approach to repair a cloaca in Monterrey, Mexico
• Describe the maneuver known as total urogenital mobilization to facilitate the repair of
cloacas [ 79 ].
• Describe the precise anatomy of a malformation called posterior cloaca [ 80 ]
• Create and implement a bowel management
program aimed to keep clean patients suffering from fecal incontinence [ 81 – 84 ].
It was there, in Long Island, New York, where
it became clear to us that children suffering from
anorectal malformation required and deserved
better care. Yes, we were very proud because we
were able to repair the anatomy of the malformation, but we learned that at least 25 % of all our
cases suffered from fecal incontinence because
they were born with severe anatomic defi ciencies. We also learned that many of the 75 %
group of patients had a “borderline” bowel control and require supervision and help for life. We
understood that 25 % of our patients required
neurosurgical services, 30 % of them had orthopedic problems, most of them need the help of a
gastroenterologist, at least 50 % of them need a
pediatric urologist, and most girls will benefi t
from the advice of a pediatric gynecologist.
There was an obvious conclusion that these
patients must be treated in specialized centers
where they will be treated by a multidisciplinary
team. With that in mind, we presented the idea to
some leaders of prominent children’s hospitals
in the United States. Dr. Richard Azizkhan had
the vision and courage to adopt the plan, and the
Colorectal Center for Children was created at the
Cincinnati Children’s Hospital Medical Center
in July 2005.
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