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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

458
26 Posterior Sagittal Approach for the Treatment of Other Conditions
Table 26.1 History of the posterior approach
Verneuil [ 3 ] 1874 Coccygectomy
Kocher [
Cripps [
Allingham [
Kraske [
Bevan, Arthur [
Table 26.2 Transsphincteric posterior approach to the
rectum
Cripps [ 5 , 6 ] 1876 England
Bevan [
David [
Larkin [
Kilpatrick [
Beneventi [
Oh [
Mason [
Dahl [
Henderson [
Criado [
Westbrook [
Madsen [
Jorge [
4 ] 1875 Supported the idea
1876 Division sphincters (36
5 ,
6 ]
7 ] 1879 Same plus colostomy
1 ,
2 ]
8 ,
9 ]
8 , 9 ] 1917 United States
10 ] 1943 United States
11 ] 1959 United States
12 ] 1969 Urol. England
13 ] 1971 Urol. New York
14 ] 1972 New York
15 ] 1972 England
16 ] 1974 Urol., Salt Lake
17 ] 1981 Urol., Salt Lake
9 ] 1981 Baltimore
18 ] 1982 Arkansas
19 ] 1987 Denmark
20 ] 1987 Pennsylvania
cases, CA)
1885 Same plus partial
removal of sacrum
1917 United States, complete
division of sphincters
transsphincteric approach for the treatment of
rectal tumors. We could not fi nd a publication
prior to ours, in 1982, indicating that this
approach has been used for children and/or for
the treatment of anorectal malformations.
Apparently the posterior transsphincteric
approach for the treatment of tumors is no longer
popular, most likely due to the fact that those
patients suffered from a signifi cant morbidity,
and in addition, modern techniques allowed to
treat those conditions in a less invasive manner
and with less morbidity.
Because of our experience with the posterior
sagittal approach, some colleagues referred to
us patients suffering from different problems,
unrelated to anorectal malformations. These
included urogenital sinus with and without
adrenal hyperplasia, vaginal or urethral fi stula,
and different kinds of sequelae from trauma.
What all those conditions had in common was
the fact that the location of the defect or tumor
was diffi cult to access through the abdomen or
through the perineum. We thought that a possible alternative could be a posterior sagittal,
transsphincteric, trans-anorectal approach that
will give us access to the urogenital tract. Prior
to trying this approach in human beings, we
performed an experimental study to be sure that
this surgical approach will not affect the bowel
control of the patients, since most of them had
normal anorectum and normal bowel control
[ 21 ]. The study in animals demonstrated that
the trans-anorectal approach did not affect
bowel control.
26.2 Urogenital Sinus with Normal Rectum
This particular malformation occurs more often
associated to adrenal hyperplasia. Our experience
has been mainly in cases without adrenal hyperplasia. This is a rather infrequent condition.
Figure 26.1 shows a diagram of the sagittal view
of a patient suffering from this malformation.
The rectum is normal, is well located, and has a
normal sphincter mechanism. The vagina is connected to the urethra, creating a urogenital sinus
of different lengths. The malformation occurs in
the form of a spectrum. In other words, we have
seen patients with a very short common channel
(low implantation of the vagina) (Fig.
well as cases with a higher junction of the vagina
and urethra (Fig. 26.1b ), including connections
directly into the bladder, or even more complex,
creating a single vesicovaginal chamber.
Figure 26.2a shows a radiologic study consisting
of the injection of contrast material into the bladder and into the vagina. Figure 26.2b, c shows
and MRI study of a urogenital sinus. The length
of the common channel, as mentioned when cloacas are discussed, can only be measured accurately endoscopically (cystoscopy).
26.1a ) as

26.2 Urogenital Sinus with Normal Rectum
459
There are many publications describing different ways to repair urogenital sinus. Most
publications are related to cases of adrenal
hyperplasia. Signifi cant contributions were
ab
made by very distinguished surgeons [ 22 – 30 ].
Those techniques are still useful, mainly to
repair urogenital sinus with a short common
channel (less than 3 cm).
Fig. 26.1 Diagram shows a urogenital sinus with normal rectum. ( a ) Short common channel. ( b ) Long common
channel
ab
Fig. 26.2 Cystovaginogram of a patient with a urogenital
sinus. ( a ) Short common channel. ( b ) MRI study of a uro-
genital sinus with long common channel. ( c ) MRI study of
a urogenital sinus with hydrocolpos, h hydrocolpos, v
vesicostomy, cc common channel

460
26 Posterior Sagittal Approach for the Treatment of Other Conditions
c
Fig. 26.2 (continued)
Fig. 26.3 Diagram showing the incision used for the
trans-anorectal approach of a urogenital sinus
Fig. 26.4 External appearance of the perineum of a
patient with a urogenital sinus
The trans-anorectal approach is an excellent
way to expose areas of the pelvis that are otherwise diffi cult to approach abdominally or
perineally [ 31 – 33 ]. Figure 26.3 shows the inci-
sion that we used for the trans-anorectal
approach of a urogenital sinus. In the fi gure,
the patient is placed in the prone position, and
the incision runs from the middle portion of the
sacrum, passing through the anus itself, and
continues all the way down to the single urogenital orifi ce. Figure
26.4 shows the external
appearance of the perineum of a patient with a
urogenital sinus in the prone position. A midsagittal incision is performed using a needle-tip
cautery, changing from cutting to coagulation,
to provide meticulous hemostasis. The incision
continues through the skin, subcutaneous tissue, parasagittal fi bers, muscle complex, and
levator mechanism until we identify the posterior rectal wall. The posterior rectal wall is
divided, placing silk temporary sutures, one in

26.2 Urogenital Sinus with Normal Rectum
461
Fig. 26.5 Diagram showing the trans-anorectal incision
(the arrows show the anterior and posterior limits of the
sphincter)
front of the other, in corresponding locations
of the rectum edges in order to identify them
during the reconstruction of the rectum and the
sphincter mechanism. The incision continues
dividing the anterior rectal wall and the sphincter mechanism, anterior to the anal opening,
and runs all the way to the single orifi ce in the
perineum until we identify the posterior wall of
the urogenital sinus. Figure
26.5 shows a dia-
gram of the incision, and the arrows show the
posterior and anterior limits of the anal sphincter. Figure 26.6 shows an intraoperative view
of the same stage of the operation, and again,
the arrows show the anterior and posterior lim-
Fig. 26.6 Intraoperative appearance of the trans-
anorectal approach (the arrows show the anterior and pos-
terior limits of the sphincter), the arrows show the limits
of the sphincter
its of the sphincter. The posterior wall of the
urogenital sinus is divided exactly in the midline to expose the common channel, the urethral orifi ce, and the vaginal orifi ce. A Foley
catheter is introduced into the bladder.
Figure
26.7 shows a diagram of the anatomic
fi ndings. Figure 26.8 shows a photograph of
the intraoperative fi ndings after the urogenital
sinus has been opened.
Prior to the use of the maneuver called “total
urogenital mobilization,” the repair of this
defect consisted in separating the vagina from

462
26 Posterior Sagittal Approach for the Treatment of Other Conditions
Fig. 26.7 Diagram showing the anatomy of the urogenital
sinus, after the posterior wall of the sinus has been divided,
a Foley catheter is introduced through the urethra. Arrows
show the limits of the sphincter
Fig. 26.9 Diagram showing the separation of the vagina
from the urinary tract
Fig. 26.8 Intraoperative view of the anatomy. Arrows
show the limits of the sphincter
the urinary tract (Figs. 26.9 and 26.10 ). Once the
vagina was separated and mobilized enough to
reach the labia with no tension, the urethra was
reconstructed, using what used to be the common
channel (Fig.
26.11 ). The vagina was pulled down
behind the urethra and sutured to the neolabia.
The anorectum and anterior and posterior sphincter mechanism are reconstructed (Fig. 26.12 ).
In 1997, we described a maneuver called
“total urogenital mobilization” [
34 ], in an attempt
to simplify the repair of cloacas. This maneuver
turned out to be very useful to repair cloacas with
common channels shorter than 3 cm. As a natural
consequence of this, we decided to repair urogenital sinuses with a normal rectum, using a
transanal approach combined with the total urogenital mobilization (see Chap. 16 , Sect. 16.1.1.4
on cloacas).
The total urogenital mobilization was well
received by many surgeons [ 35 – 39 ]. However, as

26.2 Urogenital Sinus with Normal Rectum
ab
463
Fig. 26.10 Vagina completely separated from the urinary tract and mobilized enough to reach the perineum.
( a ) Diagram. ( b ) Operative
expected, some surgeons, mainly urologists,
voiced their concerns about the trans-anorectal
approach. Consequently, some of them decided
to do the total urogenital mobilization, but not
perform the full trans-anorectal approach, but
rather to divide only the anterior portion of the
sphincter and anorectum [ 40 , 41 ] or perform the
mobilization without touching the anus [ 42 , 43 ].
Also, a limited urogenital mobilization was
described [ 44 ]. We agree with the basic idea of
being as less invasive as possible, but we also
believe that we should not hesitate to divide the
rectum and anus, in order to achieve our goal of
performing an adequate anatomic repair.
Figure 26.13 shows the operative fi eld of a
urogenital sinus. We started that particular case
with an incision that included the sphincter
mechanism located anterior to the anus. We tried
to mobilize the urogenital sinus, as much as possible without opening the rectum (Fig. 26.13a ).
We then found that exposure was not good enough
to achieve a satisfactory mobilization, and therefore we elected to divide the entire anorectum in
the midline. Figure 26.13b shows the improved
exposure achieved with the full trans- anorectal
approach. The total urogenital mobilization is
performed with a magnifi cent exposure. The suspensory ligaments of the urethra and vagina are
divided. By doing that, we gained enough length
to bring the urethra and vagina down (Fig. 26.13c,
d ). What used to be the common channel is
divided in the midline, creating two fl aps that will

464
26 Posterior Sagittal Approach for the Treatment of Other Conditions
Fig. 26.11 Diagram showing the urethra reconstructed
using the original common channel
be sutured to the neolabia. The urethral meatus is
sutured a few millimeters behind the clitoris
(Fig. 26.13e ). Figure 26.14 shows the fi nal exter-
nal appearance.
We must always keep in mind that we are dealing with a spectrum of defects, and therefore, the
surgeon must be prepared to implement different
surgical maneuvers, adequate for each one of the
anatomic variants of the malformation that we
deal with. For very low vaginal-urethral confl uence, the surgeon can use any technique, because
the repair is relatively easy. On the other hand, for
cases with extremely high implantations of the
vagina, such as vaginovesical communication, the
transabdominal approach represents the ideal way
to do it. In the middle of those extremes are malformations shown in these diagrams with common channels that vary from 2 to 4 cm.
The size of the vagina is also another important
factor. Some patients with this type of urogenital
sinus also have hydrocolpos, like in the patients
with cloacas. The presence of hydrocolpos, of
course, must alert the surgeons about the possibility
of megaureters and hydronephrosis (see chapters on
cloacas). However, the presence of hydrocolpos
also means that the surgeon has more vaginal tissue
Fig. 26.12 Diagram
showing the reconstructed
vagina, perineal body,
sphincter mechanism
anterior to the rectum, and
anterior rectal wall. The
posterior rectal wall was
closed using two layers of
interrupted long-term
absorbable sutures. The
sphincter mechanism
posterior to the anus is
meticulously
re-approximated

26.2 Urogenital Sinus with Normal Rectum
ab
465
cd
Fig. 26.13 Intraoperative view of the repair of a urogeni-
tal sinus. ( a ) An attempt is made to mobilize the urogeni-
tal sinus, with a limited incision, trying not to open the
rectum. The exposure is limited. ( b ) Improved exposure
after extending the incision through the anus and rectum.
R divided rectum, u urogenital sinus. ( c ) Operative picture
of a case with a short common channel. It was possible to
mobilize the urogenital sinus without dividing the rectum.
( d ) Operative picture showing a total urogenital mobiliza-
tion. The urethra and vagina have been fully mobilized.
( e ) Operative picture. The urethral meatus is sutured a few
millimeters behind the clitoris

466
26 Posterior Sagittal Approach for the Treatment of Other Conditions
e
Fig. 26.13 (continued)
Fig. 26.14 Final external appearance
to work with and more chances to bring the vagina
down. Patients with very small vaginas and long
common channels represent a challenge and may
require a partial vaginal replacement.
The fi rst trans-anorectal operations performed
by us included a protective colostomy.
Subsequently, as we gained confi dence in these
kinds of techniques, we performed the same operations without a colostomy. We followed the same
strict rules of bowel preparation (see chapter on
bowel preparation). The patients remained
7–10 days with nothing by mouth, receiving parenteral nutrition; our patients did not have infections,
dehiscence, or other kinds of complications.
Our current way to approach these patients is
to determine the length of the common channel
endoscopically. For those that are extremely long
common channels (vaginovesical fi stula), we
prepare the patient to be approached through the
perineum and also through the abdomen. In addition some of those patients may require a partial
vaginal replacement. If we believe that the patient
can be treated trans-anorectally without a laparotomy (common channel between 2 and 4 cm),
we prepared the bowel as described and take the
patient to the operating room. We start by making
an incision running from the anus to the urogenital sinus, trying not to open the rectum and see if
it is possible to do the total urogenital mobilization without dividing the anterior rectal wall or
the posterior rectal wall (Fig.
26.13a ). Sometimes
this is feasible, but if we feel uncomfortable
about the exposure, we do not hesitate to divide
fi rst the sphincter mechanism located anterior to
the anus or even the posterior rectal wall and the
posterior sphincter mechanism (Fig. 26.13b ).
The trans-anorectal approach consists in
dividing the anus and the rectum exactly in the
midline. By doing that, we obtain a magnifi cent
exposure to the urogenital tract. However, the
procedure requires a meticulous bowel preparation or a colostomy, followed by a delicate, scrupulous technique with special attention to details.
Our experience with the repair of a urogenital
sinus with normal rectum includes 16 cases. We
are extremely happy, because bowel control has
been normal in all of them. The patients also
showed evidence of urinary control. None of our
patients reached the age of sexual function, and
therefore, we cannot comment on that. However,
we expect them to have an examination under
anesthesia at the age when they expect to become
sexually active. We will not be surprised to fi nd
that they may need an external introitoplasty like
we do in patients with cloaca, because they have
a ringlike fi brous band that may interfere with

26.4 Acquired Urethral Atresia
467
satisfactory sexual activity and may require a
small operation to take care of that.
26.3 Urogenital Sinus with Normal Rectum and Adrenal Hyperplasia
We have used the same approach in patients with
adrenal hyperplasia. However, we must admit that
these groups of patients (with virilization) are
more diffi cult to repair. First of all, they require a
clitoral recession. In spite of doing that procedure,
one deals with the presence of hypertrophic corpora that makes the operation technically more
diffi cult. In addition, the vagina in those types of
patients is usually much smaller, both in length
and diameter. All of that makes the operation
technically more demanding. Our experience with
this group of patients is limited to three cases, and
therefore, we cannot claim that this is the ideal
way to repair this malformation.
26.4 Acquired Urethral Atresia
Male patients, who suffer from severe pelvic
trauma, frequently have severe urethral injuries
that result in an acquired atresia of the posterior
urethra. These represent a surgical challenge, not
only to be able to reconnect both ends of the posterior urethra, but also to try to obtain urinary
control after the repair.
Traditional approaches to this problem include
perineal operations [ 45 – 49 ] or transpubic
approach [ 50 – 56 ]. We have a modest but signifi -
cant experience with the trans-anorectal approach
that has also been used by others [ 57 – 60 ].
Six patients have been referred to us suffering
from this type of problem. These patients underwent several previous attempts to repair, using a
perineal approach, and all of them were unsuccessful. Figure 26.15a shows a cystogram and
urethrogram of one of these patients. The gap
between the blind ends measures over 3 cm. This
study was done injecting contrast material
through a suprapubic tube and simultaneously
through the urethral meatus. This was an adult
patient who suffered a very severe pelvic trauma
that destroyed the posterior urethra. The patient
was ejaculating through the suprapubic tube and
had three previous failed attempts to repair the
problem. Figure 26.15b shows a diagram trying
to show the anatomy of the same patient. In addition to the disruption of the posterior urethra, the
patient also had a mild stenosis of the penile urethra. The subset of the fi gure shows the transanorectal incision used to approach this urethra.
a b
Fig. 26.15 Disrupted posterior urethra, a mild penile urethral stricture and the trans-anorectal incision. ( a ) Diagram.
( b ) Contrast study – injection of contrast done through a suprapubic tube and through the penis
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