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15 Cloacal Exstrophy
high-pressure bladder dynamics, pyelonephritis, vesicoureteral reux, 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 signicant 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 andFertility
Sexual function in individuals affected by cloacal exstrophy depends on the extent of genital and Müllerian anomalies, which can vary signicantly 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, 6265].

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 rened 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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15 Cloacal Exstrophy
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34. Judy BF, Materi J, Lee RP, Tracz JA, Patel J, Weber-Levine C, Crigger C, Mistry P, Gearhart JP, Jackson EM.Spinal Dysraphism in Exstrophy: A single-center study of a 39-year prospective database. J Neurosurg Pediatr. 2023;32:98–105. https://doi.org/10.3171/2023.3.PEDS22447.
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41. Benz K, Maruf M, Hatheway C, Kasprenski M, Jayman J, Docimo S, Schneck F, Gearhart J.The Intravesical phallus in patients with cloacal Exstrophy: an embryologic conundrum. J Pediatr Urol. 2018;14(428):e1–428.e5. https://doi.org/10.1016/j.jpurol.2018.05.001.
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org/10.1053/j.sempedsurg.2010.12.007.
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46. Tirrell TF, Demehri FR, Henry OS, Cullen L, Lillehei CW, Warf BC, Gates RL, Borer JG, Dickie BH.Safety of delayed surgical repair of Omphalocele-Exstrophy-imperforate anus­spinal defects (OEIS) complex in infants with signicant comorbidities. Pediatr Surg Int. 2021;37:93–9.
47. Jayman J, Tourchi A, Feng Z, Trock BJ, Maruf M, Benz K, Kasprenski M, Baumgartner T, Friedlander D, Sponseller P, etal. Predictors of a successful primary bladder closure in cloacal Exstrophy: A multivariable analysis. J Pediatr Surg. 2019;54:491–4. https://doi.org/10.1016/j.
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T. Lee and J. Borer

Male Epispadias

16
PaulA.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.
Classication 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 reux. 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 Table16.1, Cendron etal. 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 etal. series showing that an abnormal bladder neck can be found also in
Abnormal pubic diastasis VUR
n
Abnormal bladder neck
Continence procedures Continent
Age>5years 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 difcult. 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 deciency 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 conguration with excess irregular glans epithelium adjacent to the urethral plate.
The ureterovesical junction may be affected in complete epispadias, and the inci­dence of reux 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 signicant.
16 Male Epispadias
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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 conguration. 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 ofSurgical Repair ofEpispadias [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 modication 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 etal. reported a modied 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 modied Cantwell procedure which had 3 components: (1) corporal rotation dorsally with cavernocavernostomy, (2) urethral mobilization, and (3) improved glanuloplasty and dorsal skin cover­age [12].
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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
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Current Approaches toMale Epispadias Repair
From the various techniques described in the previous section, two reconstructive procedures are currently mostly utilized: the modied Cantwell-Ransley repair and the Mitchell repair [13, 14].
Repair of isolated epispadias is performed at around 6–9months of age.
Parenteral injection of testosterone preoperatively with two injections (5weeks prior and 2weeks prior) of 2mg/kg of testosterone enanthate has been shown to increase penile length by a mean of 2.7cm and increase penile circumference by a