Bilateral Single Ectopic Ureters: A Challenging Entity to Achieve Continence and Preserve Renal Function

Abstract

Purpose Bilateral single ectopic ureters (BSEU) are associated to cervical dysplasia, hypoplastic bladder, urinary incontinence, and sometimes renal failure. The therapeutical challenge is to achieve continence with spontaneous voiding and preservation of renal function. The ideal management would be a conservative ureteral reimplantation and urethrocervicoplasty (UCP) to allow bladder growth, without requirement for enterocystoplasty and CIC. Our aim was to report our results in a cohort of 11 patients with BSEU. Material and Methods Retrospective study including prospective follow-up of patients from 1988 to 2009 in two tertiary centres. Modes of presentation, associated anomalies, surgeries, and their results on continence and renal function were reviewed. Results All 11 patients were females with a mean age of 11 years (range 2-21). Modes of presentation were prenatal diagnosis in 4, incontinence and/or UTI in 5, and an abnormal perineum at birth in 2. All had associated anomalies: either urinary (5 VUR, 6 ureteral bifidity, 10 hypoplastic bladder) and/or extra-urinary (5 caudal, 4 mullerian, 4 vertebral, 3 cardiac, 3 ophthalmic). Four had a degree of renal insufficiency, and 7 asymmetrical nuclear renal function. All had a median of 4 surgeries (range 1-6). Mean follow-up was 4.5 years (range 0.5-15). Only 3 had a reconstruction (i.e. ureteral reimplantation and UCP and/or cervical injections of bulking agents) without cystoplasty: 2 were continent, 1 was still too young. Seven needed reconstruction and enterocystoplasty with a catheterizable channel (mitrofanoff or monti): 5/7 were continent on CIC. One is awaiting reconstruction. One had renal transplantation. Conclusions Conservative management of bilateral single ectopic ureters is challenging but very seldom sufficient to achieve continence without a bladder augmentation.