Completely Separated Scrotum and Vesicointestinal Fistula Without Exstrophy as a Novel Manifestation of Aphallia: A Case Report

• DOI: 10.1016/j.urology.2009.04.055
• Publication Date: 2009-07-09T05:25:00Z
• Authors: Yuya Nakano; Madoka Aizawa; Sumie Honma; Yuichi Osa
• Keywords: Omphalocele; Cloacal exstrophy; Agenesis; Renal agenesis

A male infant born at 32 weeks of gestation and weighing 1.44 kg had penile agenesis, bifid scrotums, an anorectal anomaly, and suspected omphalocele. An operation revealed complete urethral agenesis, duplicated and blind bowels, a vesicointestinal fistula without exstrophy, and a urachal fistula; however, no omphaloceles were detected. To our knowledge, this is the first report on aphallia accompanied with completely separated scrotum and a vesicointestinal fistula without exstrophy; this condition is associated with urorectal septum malformation sequence (URSMS) and covered cloacal exstrophy. The possible embryogenesis is discussed in this study.