Successful bladder management for a pure urogenital sinus anomaly.

We report on a case of a pure urogenital sinus anomaly presented with bladder distention. A seven-day-old girl with an abdominal distension was referred to the Division of Urology, Hakodate Central Hospital, Hakodate, Japan. A common urogenital sinus without abnormalities in the labium, clitoris or anus was found. A computed tomography (CT) scan documented a distended bladder without hydrometrocolpos or hydroureteronephrosis. Cystography performed at 44 days revealed a large flaccid bladder without ureteral reflux. Urinary management by an indwelling urethral catheter was maintained until 3 months, when an endoscopic examination was performed and a stenotic urethral-type urogenital sinus with low confluence was diagnosed. Parents successfully instituted clean intermittent catheterization as a temporary urinary management, and postvoid residual urine gradually decreased. At 2 years of age, flap vaginoplasty was performed. In an urodynamic study performed postoperatively, the detrusor pressure during voiding was 40-50 cmH(2)O. The patient maintained spontaneous voiding without consequences. Appropriate urinary care is essential to prevent urological complications in cases with a pure urogenital sinus anomaly.