The intravesical phallus in patients with cloacal exstrophy: An embryologic conundrum

Karl Benz, Mahir Maruf, Clark Hatheway, Matthew Kasprenski, John Jayman, Steven Docimo, Francis Schneck, John Phillip Gearhart

Research output: Contribution to journalArticle

Abstract

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 presented]

Original languageEnglish (US)
JournalJournal of Pediatric Urology
DOIs
StateAccepted/In press - Jan 1 2018

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Urinary Bladder
Biopsy
Newborn Infant
Bladder Exstrophy
Nervous System Malformations
Karyotype
Embryonic Development
Blood Vessels
Anatomy
Pathology
Kidney

Keywords

  • Cloacal exstrophy
  • Intravesical phallus
  • Variant

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Urology

Cite this

The intravesical phallus in patients with cloacal exstrophy : An embryologic conundrum. / Benz, Karl; Maruf, Mahir; Hatheway, Clark; Kasprenski, Matthew; Jayman, John; Docimo, Steven; Schneck, Francis; Gearhart, John Phillip.

In: Journal of Pediatric Urology, 01.01.2018.

Research output: Contribution to journalArticle

Benz, Karl ; Maruf, Mahir ; Hatheway, Clark ; Kasprenski, Matthew ; Jayman, John ; Docimo, Steven ; Schneck, Francis ; Gearhart, John Phillip. / The intravesical phallus in patients with cloacal exstrophy : An embryologic conundrum. In: Journal of Pediatric Urology. 2018.
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