Orthotopic continent urinary diversion after radical cystectomy in pediatric patients with genitourinary rhabdomyosarcoma.

摘要

PURPOSE: We present our experience with orthotopic continent urinary diversion following radical cystectomy due to genitourinary rhabdomyosarcoma in children not responding to radiotherapy or chemotherapy. MATERIALS AND METHODS: Four children with persistent genitourinary rhabdomyosarcoma underwent radical cystectomy with reconstruction of an orthotopic ileal neobladder. RESULTS: Average followup was 50.7 months. One patient died at 12 months postoperatively due to pulmonary recurrence. All patients were able to void completely at regular intervals. Renal function was normal in all patients. Average reservoir capacity was 250 ml. Daytime and nighttime urinary continence was achieved in all patients. CONCLUSIONS: The orthotopic continent urinary diversion is technically feasible even in small children. It represents a safe long-term option and offers good quality of life after disfiguring surgery. In fact, patients are able to void spontaneously through the urethra.