Bladder Agenesis Associated with Crossed Fused Renal Ectopia and Vertebral Anomalies: A Rare Entity.

A 9-month-old girl presented with dribbling urine and no normal voiding since birth. There was no history of urinary tract infection. On examination, the child appeared healthy. An abdominal examination was normal. On separation of the labia, a single opening with leaking urine was seen; no separate urethral opening was identified (Figure 1). Abdominal ultrasonography revealed a crossed left renal ectopic kidney. Computed tomography also showed a normally excreting crossed fused ectopic kidney/lump kidney (Figure 2A), ureters draining into a urogenital sinus, and butterfly and block lumbar vertebrae (Figure 2B). The serum creatinine level was normal (0.3 mg/dL). Endoscopy using a pediatric cystoscope inserted through the genital opening revealed a urogenital sinus in which both ureteric openings were located (Figure 3); both ureters refluxed when the sinus was filled with contrast (Figure 4). We plan to divert the ureters to a cutaneous stoma. Agenesis of the urinary bladder is extremely rare; only 23 living cases have been reported. Agenesis is attributable to injury to the urogenital sinus at weeks 5~7 of embryogenesis. The condition is associated with renal and skeletal anomalies. Young age is not a contra-indication for continent urinary diversion using a self-catheterizable pouch.