Ectopic ureter with complete ureteric duplication in the female child

Twenty-two female children with complete ureteric duplication and ectopic ureter (two bilateral) were seen in a 15-year period. The most common clinical presentation was dribbling urinary incontinence with "normal" micturition. Other presentations included urinary infection and vaginal discharge. Four cases were diagnosed after antenatal recognition of ureterohydronephrosis. The clinical diagnosis was supported by various radiological investigations but ultrasonography (US) proved to be particularly reliable in diagnosing ectopic ureter. The most common sites of opening of the ectopic ureter were the urethral margin or the urethrovaginal septum, although in seven cases the site was not identified. Twenty-one kidneys were managed by upper pole heminephrectomy and three by ureteropyelostomy, removing as much of the ectopic ureter as possible via the renal approach. The distal ectopic ureter was removed via a separate suprapubic incision at the initial operation in four cases, and in two cases, delayed excision of the distal ectopic ureter was necessary. All surgical specimens were examined histologically and only two heminephrectomy specimens showed features of renal dysplasia. Dribbling urinary incontinence was cured in all cases, although in one patient the entire kidney was lost after heminephrectomy. Ectopic ureter should be suspected in girls with dribbling urinary incontinence. The diagnosis is best supported by US together with conventional radiology. The majority of cases can be managed by heminephrectomy, but when adequate function is demonstrated in the upper pole, ureteropyelostomy is recommended.