A complex urologic problem demonstrates how far pediatric urology has progressed.

Abstract

OBJECTIVE: We present the experience at Connecticut Children's Medical Center (CCMC) with single-system bilateral ectopic ureters (SSBUE), a rare clinical entity which continues to challenge the pediatric urologist. Innovative diagnostic and reconstructive techniques are utilized in an effort to preserve renal function and develop urinary continence in a select group of patients. METHODS: A review of the English language literature was performed to identify all female patients with SSBUE and evaluate their treatment and outcomes. In addition, we present our experience with three cases of SSBUE at CCMC and describe the recent advances in the management of such patients. RESULTS: From 1937 to 1999, 25 articles described 54 female patients with SSBUE. Patients were treated with a variety of reconstructive procedures that resulted in a low overall continence rate of 20% (eight of 39 reported outcomes). We evaluated three patients with SSBUE at CCMC. Our first patient had undergone three previous operations prior to her successful procedure at CCMC utilizing a new technique to achieve urinary continence in which a portion of the anterior bladder wall is used for bladder neck reconstruction. Our last patient was identified antenatally by the maternal-fetal obstetrician, evaluated postnatally by the pediatric urologist, and underwent formal urodynamics and proximal urinary diversion for an obstructed upper collecting system. No previous reports focus on antenatal identification, accurate postnatal diagnosis, urodynamic assessment and early intervention including upper urinary tract diversion, and staged Pippi Salle bladder neck reconstruction. CONCLUSION: The CCMC experience with SSBUE demonstrates the recent achievements in complex urinary tract reconstruction that have taken place in pediatric urology. We describe diagnostic studies such as formal urodynamics and the growing trend to identify urologic problems antenatally. A team approach involving the obstetrician, pediatric urologist, neonatologist, and the pediatrician has been beneficial. As pediatric urology moves into the new millennium there are prospects for even more innovative diagnostic and therapeutic options for children with urologic problems.