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- •Foreword
- •Foreword
- •Preface
- •Contents
- •Contributors
- •Introduction
- •Epidemiology
- •Etymology
- •Bladder Exstrophy Pathophysiology
- •Conclusion
- •References
- •Normal Development
- •Introduction
- •Prenatal Imaging
- •Prenatal Counseling
- •Epispadias
- •Classic Bladder Exstrophy
- •Cloacal Exstrophy
- •BEEC Variants
- •Prenatal Management
- •Genetic Counseling
- •Conclusion
- •References
- •3: Bladder Exstrophy Genetics: Our Current Understanding
- •Bladder Exstrophy Genetics
- •Copy Number Variant (CNV) Studies
- •Gene Expression Studies
- •Genome-wide Association Study (GWAS)
- •Future Directions
- •References
- •4: Prenatal and Postnatal Imaging of the Bladder Epispadias-Exstrophy Complex
- •Introduction
- •Prenatal Imaging Findings
- •Bladder Exstrophy
- •Cloacal Exstrophy
- •Isolated Epispadias
- •Exstrophy Variants
- •Postnatal Imaging Findings
- •Urinary System
- •Musculoskeletal System
- •Spine
- •Conclusions
- •References
- •Introduction
- •Bladder Growth
- •Urinary Continence
- •Conclusions
- •References
- •6: Complete Primary Repair of Bladder Exstrophy and Epispadias
- •Bladder Neck Reconstruction, Bladder/Urethral Closure
- •Pubic Bone Closure
- •Umbilicoplasty
- •Immobilization
- •Urethral Plate Dissection
- •“Grady Monsplasty”
- •Complications
- •Conclusion
- •References
- •Introduction
- •Prenatal Diagnosis
- •Anatomic Anomalies
- •Immediate vs Delayed Closure
- •Surgical Reconstruction
- •Immobilization Techniques
- •Epispadias Repair
- •Achieving Urinary Continence
- •Proposed Follow-Up
- •Future Directions
- •Conclusion
- •References
- •8: The Kelly Procedure
- •Introduction
- •Tension-Free Bladder Neck Construction
- •Postoperative Management
- •References
- •Introduction
- •Anesthesia
- •Incision
- •Bladder Plate Mobilization
- •Radical Corporal Detachment
- •Osteotomy
- •Ischiopubic Osteotomy
- •Transverse Innominate Osteotomy
- •Corporal-Urethral Separation
- •Reconstruction
- •Postoperative Management
- •Follow-Up
- •Results
- •Conclusion
- •References
- •Introduction
- •Surgical Procedures
- •References
- •Bilateral Ureteral Advancement Reimplantation
- •Pelvic Osteotomy
- •Preoperative Testosterone Administration
- •Epispadias Repair
- •Penile Skin Reconstruction
- •Continence Enhancement
- •Conclusion
- •Introduction
- •Background
- •Modified Perineal Approach Surgical Technique
- •Discussion
- •References
- •Introduction
- •Posterior Iliac Osteotomies
- •Anterior/Double Iliac Osteotomies [3, 14]
- •Anterior Oblique Iliac Osteotomies [5, 11]
- •Anterior Bilateral Superior Pubic Rami Osteotomies [4]
- •Postoperative Immobilization
- •Complications/Long-Term Outcomes
- •References
- •Ureteral Reimplantation
- •Inguinal Hernia
- •Monsplasty
- •Umbilicoplasty
- •References
- •Introduction
- •Ureterosigmoidostomy
- •The Sigma-Rectum Pouch (Mainz Pouch II)
- •The Cologne Pouch
- •Conclusion
- •References
- •15: Cloacal Exstrophy
- •Introduction
- •Epidemiology
- •Embryologic Etiology
- •Prenatal Findings
- •Urinary
- •Gastrointestinal
- •Neurologic
- •Musculoskeletal
- •Genital
- •Management
- •Neonatal
- •Surgical Reconstruction
- •Secondary Procedures
- •Outcomes
- •Urinary Continence
- •Renal
- •Fecal Continence
- •Gender Rearing
- •Nutrition
- •Mobility
- •Psychosocial Outcomes
- •Conclusion
- •References
- •16: Male Epispadias
- •Embryology
- •Anatomic Features
- •Epispadias Repair
- •Pelvic Osteotomy
- •Modified Cantwell-Ransley Repair
- •Urethral Reconstruction
- •Bladder Neck Reconstruction
- •The Mitchell Repair
- •Initial Dissection
- •Penile Disassembly
- •Proximal Dissection
- •Bladder Neck Reconstruction
- •Primary Closure
- •Skin Closure
- •Outcomes
- •Fistula Formation
- •Urethral Stricture
- •Residual Curvature
- •Urinary Continence
- •Sexual Function
- •Renal Function
- •Female Epispadias
- •Outcomes
- •Conclusion
- •References
- •Introduction
- •Pre-operative Factors
- •Technical Aspects
- •Management
- •Failed Genital Reconstruction
- •Ureterosigmoidostomy
- •Augmentation Cystoplasty
- •References
- •Background
- •Preoperative
- •Monitoring
- •Intraoperative Management
- •Postoperative Management
- •Conclusion
- •References
- •Mental Health Concerns
- •Local Priority
- •Resources
- •Clinical Care
- •Capacity Building
- •Research
- •General Principles
- •References
- •Introduction
- •Defining Continence
- •Continence versus Dryness
- •Dry Interval: How Long Is Long Enough?
- •Dry Intervals: What Is Meaningful
- •Diversion Versus Continence
- •Timing
- •Challenging Dogma
- •References
- •Introduction
- •Preoperative Counseling
- •Bladder Neck Bulking Agent Injection
- •Artificial Urinary Sphincter
- •Bladder Neck Reconstruction
- •Bladder Neck Closure
- •Continent Catheterizable Channel: Mitrofanoff Principle
- •Augmentation Cystoplasty
- •Continent Urinary Diversion
- •References
- •22: Urinary Reconstruction for Bladder Exstrophy in the Developing World: Special Consideration and Technique
- •Introduction
- •Operative Technique
- •The Final Reconstruction
- •Young-Dees-Leadbetter Bladder Neck Plasty
- •Bladder Neck Closure
- •Operative details
- •Discussion
- •Conclusion
- •References
- •Introduction
- •Nephrology Evaluation
- •Measuring Kidney Function
- •Evaluating Blood Pressure
- •Imaging Studies
- •Transplant
- •References
- •Introduction
- •Post-operative Nursing Care
- •Pain Control
- •Immobilization
- •Orthopedic Care
- •Parental Teaching
- •Conclusion
- •Bibliography
- •Introduction
- •Pelvic Floor Musculature
- •Physical Therapy Evaluation
- •Participation
- •Activity
- •Impairment
- •Physical Therapy Intervention
- •Pre-toilet Training
- •Toilet Training
- •Post-toilet Training
- •Day Versus Night
- •Constipation
- •References
- •Pediatric Psychology
- •Infancy
- •Childhood
- •Adolescence
- •Adulthood
- •Future Directions
- •References
- •Females
- •Males
- •Erectile Function
- •Ejaculatory Function
- •Recommendations
- •Literature
- •Gynecologic Anatomy
- •Puberty
- •Pelvic Organ Prolapse
- •Fertility
- •Obstetric Considerations
- •Conclusions
- •References
- •Introduction
- •Patient Advocacy
- •Peer Support
- •Local Support Groups
- •Medical Advisory Council
- •Annual Conferences
- •Global Health Inequities
- •Global Health Initiatives
- •Advocacy Considerations
- •Patient-Directed Research
- •Patient Advisory Councils
- •Conclusion
- •References
- •Index

15 Cloacal Exstrophy
high-pressure bladder dynamics, pyelonephritis, vesicoureteral reux, and stone
formation from chronic urinary stasis. A single-institutional study reported a quarter of cloacal exstrophy patients having at least stage 1 chronic kidney disease [3].
251
Fecal Continence
A recent study reported nearly 80% of patients with intestinal diversion. Twenty
percent of remaining patients underwent pull-through, half with a concomitant
Malone antegrade colonic enema. However, there is signicant institutional variability in rates of intestinal diversion [60]. Another single-institutional study with
long-term follow-up reported more than a 90% rate of intestinal diversion, with 8%
of patients undergoing pull-through [3]. There is wide variability of reported rates
of fecal continence despite pull-through (30–85%) [58, 60].
Sexual Function andFertility
Sexual function in individuals affected by cloacal exstrophy depends on the extent
of genital and Müllerian anomalies, which can vary signicantly in severity.
Testicular histology, architecture of rete testis, epididymis, and vas deferens are
typically preserved in genetic males, although natural paternity has not been
reported [40, 61]. A recent systematic review of four studies reported that 18% of
females were sexually active [61]. Successful pregnancy to term is reported,
although uterine prolapse, preterm birth, loss of pregnancy, and cesarean births are
also reported [3, 43, 62–65].
Gender Rearing
Historically, genetically male patients with diminutive phallus not suitable for
reconstruction often underwent early orchiectomy and were raised as females.
However, cloacal exstrophy males typically have normal functioning testicles, thus,
normal androgen imprinting in utero. Many genetic males initially assigned female
gender have reconverted to male gender roles in adolescence and adulthood [33].
Currently, most pediatric urologists favor gender assignment according to genetic
karyotype [66].
Nutrition
There is a high prevalence of malnutrition and short bowel syndrome due to congenital malformation of the gastrointestinal tract. Cloacal exstrophy patients have lower
median height-for-age and weight-for-age z-scores compared to the healthy controls.
Short bowel syndrome and enterocystoplasty are associated with lower z-scores [67].

252
T. Lee and J. Borer
Mobility
Many patients with cloacal exstrophy are able to ambulate. Among 63 patients, 37%
were ambulated without aids, 13% ambulated with abnormal gait, and 14% ambulated with leg braces or a walker adjunct. Thirty-ve percent were wheelchair
dependent, and 2% were bedbound [3].
Psychosocial Outcomes
More attention and care should be given to psychosocial outcomes among patients
with cloacal exstrophy. A single-institutional study reported anxiety and/or depression in two-thirds of their patients and chronic pain in more than one-fourth of
patients. In the same study, 80% of adults attended college and were employed [3].
Conclusion
Cloacal exstrophy, also known as OEIS, involves an anterior abdominal wall defect
and a spectrum of urinary, gastrointestinal, skeletal, spinal, and genital anomalies.
The initial goal of genitourinary reconstruction is to bring the two hemibladder
plates together by excising the omphalocele and separating the cecal plate from the
bladder halves. The cecal plate is tubularized and placed in continuity with the
blind-ending hindgut, which is exteriorized and matured as an end colostomy. The
second surgical step is similar to reconstruction of classic bladder exstrophy, with
steps including bladder closure, reconstruction of external genitalia, and approximation of pubic rami with the aid of osteotomies. Given the high prevalence of
spinal defects in the cloacal exstrophy population, volitional voiding is typically not
an option. When patients and parents/caregivers are socially ready and properly
counseled, bladder neck closure/reconstruction and continent catheterizable channels may be created with or without enterocystoplasty in order to achieve urinary
continence. There is high prevalence of Müllerian abnormalities, which are typically able to be properly assessed and reconstructed around the time of adolescence.
Modern medical management and rened surgical techniques have enabled longer
life expectancies for patients with cloacal exstrophy, with reduced rates of malnutrition, sepsis, and renal failure. As patients are surviving childhood and living to
adulthood, continual efforts must be made to improve long-term outcomes and
quality of life.
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T. Lee and J. Borer

Male Epispadias
16
PaulA.Merguerian
Embryology
The embryological theories of epispadias have been explained in a previous chapter.
It is believed that abnormalities of the cloacal membrane may be responsible for its
occurrence. A large cloacal membrane prevents the normal migration of mesenchymal tissue but is prone to rupture leading to the spectrum of anomalies. In epispadias, the rupture causes nonunion of the distal portion of the urinary tract.
Anatomic Features
The defect involves an absence of the dorsal aspect of the urethra and overlying skin.
Classication of the defect based on the location of the urethral meatus alone is
simplistic. It does not take into account other anatomic and functional features such
as appearance of the bladder neck, presence or absence of pubic diastasis, and presence of vesicoureteral reux. The classic assumption is that patients with glandular
and penile epispadias are continent in most cases. However, the BN may be abnormal even in distal cases and may affect their ability to achieve continence [3]. In
Table16.1, Cendron etal. have shown that patients with penile epispadias may have
an abnormal bladder neck and continence is achieved only in 63% of these patients.
The most common form of this anomaly is penopubic epispadias with the defect
affecting the bladder neck and the entire urethra. These patients are incontinent of
urine and will require a bladder neck reconstruction. The more distal the urethral
defect, the less likely that the bladder neck is involved and the continence rate is higher.
P. A. Merguerian (*)
Seattle Children’s Hospital, University of Washington, Seattle, WA, USA
e-mail: paul.merguerian@seattlechildrens.org
© The Author(s), under exclusive license to Springer Nature
Switzerland AG 2025
A. R. Shukla, R. S. Joshi (eds.), Bladder Exstrophy and Epispadias,
https://doi.org/10.1007/978-3-031-91238-2_16
257

258
P. A. Merguerian
Table 16.1
penile epispadias and continence is achieved in only 63% of patients with penile epispadias
Epispadias
type
Glandular 4 1(25%) 2(50%) 0 0 3(75%) 3(75%)
Penile 8 3(38%) 1(38%) 3(38%) 0 5(63%) 5(63%)
Penopubic 14 12(86%) 5(29%) 7(50%) 7(50%) 9(64%) 11(79%)
Total 26 16(62%) 8(31%) 10(38%) 7(27%) 17(65%) 19(73%)
Cendron etal. series showing that an abnormal bladder neck can be found also in
Abnormal
pubic
diastasis VUR
n
Abnormal
bladder
neck
Continence
procedures Continent
Age>5years
at follow-up
All patients with epispadias have a variable degree of dorsal curvature. In its
most severe presentation, the penis is directed against the abdominal wall making
sexual function difcult. The dorsal curvature is due to an intrinsic deformity of the
corporal bodies and not due to tethering of the corpora by the urethral plate [4].
The proximal defects are also associated with diastasis of the symphysis pubis.
It was previously thought that the individual corpora cavernosa were of normal caliber but appeared shorter because of the wide separation of the crural attachments,
the prominent dorsal chordee, and the shortened urethral groove. However, magnetic resonance imaging (MRI) in adult men with bladder exstrophy compared to
age- and race-matched controls showed that the anterior corporeal length of male
patients with bladder exstrophy was almost 50% shorter than that of normal controls. The posterior length of the corporeal body was the same as in age-matched
controls. The diameter of the anterior corporeal segment was 30% greater than normal. Therefore, the penis appears short not only because of the diastasis of the pubic
symphysis but also because of marked congenital deciency of anterior corporeal
tissue [5].
The verumontanum is exposed on the urethral plate. The vas deferens and ejaculatory ducts are not affected. The prostate is also incompletely formed in proximal
epispadias [6].
In the epispadias phallus, the neurovascular bundle’s course is altered. Rather
than being dorsal and close to the midline, the neurovascular bundle lies ventrolateral which is consistent with a medial-to-lateral rotational deformity [7]. At the
level of the symphysis, the neurovascular bundles are slightly lateral to the midline
at the 11 and 1 o’clock positions. As the nerves course along the phallus distally,
they become more ventral and at the distal end are at the 8 and 4 o’clock positions.
The glans has a attened conguration with excess irregular glans epithelium
adjacent to the urethral plate.
The ureterovesical junction may be affected in complete epispadias, and the incidence of reux has been reported to be between 30% and 40% [8, 9] and in some
series up to 80% [10]. In male patients, this has not been shown to be clinically
signicant.

16 Male Epispadias
259
Epispadias Repair
Male epispadias is one of the most challenging problems that pediatric urologists
encounter. The ideal outcome is to render the genitourinary anatomy to its normal
location and conguration. The goals of repair include the following:
1. Correction of dorsal curvature
2. Creation of a straight urethra
3. Adequate skin coverage
4. Satisfactory cosmetic outcome
5. Maintenance of erectile function
6. Urinary continence
History ofSurgical Repair ofEpispadias [11]
In 1845, Dieffenbach freshened the lateral tissue edges and placed approximating
sutures, and in 1869, Thiersch rotated local skin aps to cover the urethral defect. It
was not until 1895 that Cantwell performed the rst true urethroplasty for complete
epispadias. Cantwell completely mobilized the dorsal urethral plate from the corpora cavernosa and transplanted the tubularized urethra below the corpora cavernosa which were then rotated dorsally and approximated in the midline (Fig.16.1).
In 1918, Young in an attempt to prevent devascularization injury to the urethral
plate described a modication of the Cantwell approach. The urethra was detached
from the right corpus cavernosum, but it was left attached to the left corpus cavernosum and simply rotated ventrally beneath the corpora.
In 1984, Lepor etal. reported a modied Young urethroplasty in which the urethra remained dorsal to the corpora and was tubularized in continuity in a ThierschDuplay fashion from the prostate to the glans penis. Also in 1984 Thmalla and in
1987 Monfort applied the Duckett transverse island ap to male epispadias repair.
Koff and Eakins recognized that rotation of the corporal bodies could correct
dorsal curvature. Their techniques involved dissection of the corpora from the urethra and inferior pubic ramus and medial-to-lateral rotation of the corporal bodies in
opposite directions with ventral xation. The urethra remained dorsally located.
To correct the intrinsic nature of the dorsal curvature, Woodhouse and Kellett
preferred a fascial release of the tunica albuginea dorsally. This extensive dissection
required full mobilization of the urethral plate and preserving the neurovascular bundle.
The combination of the Woodhouse and Kellett concept with the Cantwell repair
resulted in the modern-age repair of epispadias as the modied Cantwell procedure
which had 3 components: (1) corporal rotation dorsally with cavernocavernostomy,
(2) urethral mobilization, and (3) improved glanuloplasty and dorsal skin coverage [12].

260
B
A
AA
P. A. Merguerian
B
A
A
B
C
BB
A
A
AA
Fig. 16.1 Cantwell epispadias repair. Dorsal urethral plate (b) mobilized from corpora, tubular-
ized and positioned ventral to the corpora. Corpora cavernosa rotated dorsal to urethra and
approximated
A
A
Current Approaches toMale Epispadias Repair
From the various techniques described in the previous section, two reconstructive
procedures are currently mostly utilized: the modied Cantwell-Ransley repair and
the Mitchell repair [13, 14].
Repair of isolated epispadias is performed at around 6–9months of age.
Parenteral injection of testosterone preoperatively with two injections (5weeks
prior and 2weeks prior) of 2mg/kg of testosterone enanthate has been shown to
increase penile length by a mean of 2.7cm and increase penile circumference by a
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