The intravesical phallus in patients with cloacal exstrophy: An embryologic conundrum. Issue 5 (October 2018)

• Record Type: Journal Article
• Authors: Benz, Karl; Maruf, Mahir; Hatheway, Clark; Kasprenski, Matthew; Jayman, John; Docimo, Steven; Schneck, Francis; Gearhart, John
• Is Part Of: Journal of pediatric urology. Volume 14:Issue 5(2018)
• Page Start: 428.e1
• Page End: 428.e5
• Publication Date: 2018-10
• Subjects: Cloacal exstrophy -- Variant -- Intravesical phallus; Pediatric urology -- Periodicals; Urologic Diseases -- Periodicals; Urogenital Diseases -- Periodicals; Urologic Surgical Procedures -- Periodicals; Child; Infant; Urologie pédiatrique -- Périodiques; Appareil urinaire -- Maladies -- Périodiques; Pédiatrie; Urologie; Pediatric urology; Périodique électronique (Descripteur de forme); Ressource Internet (Descripteur de forme); Electronic journals; Periodicals; Electronic journals; 618.926
• DOI: 10.1016/j.jpurol.2018.05.001
• Languages: English
• ISSNs: 1477-5131

Abstract

Summary: Background: Phalluses present inside the extrophied bladder of cloacal exstrophy (CE) newborns have been sporadically reported in the literature; this clinical entity has largely unknown origins and may represent an extremely rare anomaly of CE. Objective: Along with nearly doubling the number of reported intravesical phalluses in the literature, this study aims to outline the common anatomic features and discuss the implications for theories of CE embryogenesis. Study design: The authors retrospectively identified patients with CE and a potential intravesical phallus between 1997 and 2017 at two high-volume centers. Information was obtained about karyotype, age at closure, neurologic and renal anomalies, diastasis, phallus anatomy, and phallus biopsy pathology. Results: Six genotypic males met the inclusion criteria. Five of six had a diastasis greater than 6 cm. Four of six had paired corporal bodies in the intravesical phallus, one had a single corporal body, and one had a corporal-like structure. Five of six patients had a phallus located midline in the caudal aspect of the bladder; one was located midline in the bladder dome. Phallic biopsies were obtained in three of six patients. Two showed glanular and corporal tissue while the other showed vascular proliferation morphologically similar to that of erectile tissue. Discussion: Previous reports suggested that a superior vesicle fissure configuration, fusion of the corporal bodies, and fused bladder plates were common findings with an intravesical phallus. With the addition of new cases, the only consistent variable between patients is a phallus located anywhere along the bladder plate that can comprise a corporal-like structure, a single corporal body, or fused corporal bodies. These findings have implications for several embryologic theories. Although this is a retrospective review with a limited number of patients, the condition is exceedingly infrequent making it only observable retrospectively over decades at high volume centers. Conclusions: The study outlined common anatomic features of the intravesical phallus in cloacal exstrophy and discussed the subsequent embryologic implications. In cloacal exstrophy newborns with presumed aphallia, meticulous inspection of the bladder plate and biopsy of any potential phallic structures can prevent resection of phallic tissue. Figure The phallus-like structure (green arrow) located midline at the level of the trigone in a 14-month-old genetic XY male with cloacal exstrophy who had undergone a tethered cord repair, an omphalocele repair, and a colostomy fecal diversion. (For interpretation of the references to color/colour in this figure legend, the reader is referred to the Web version of this article.)