OBJECTIVES: To review our experience with female children who have unilateral renal agenesis or renal dysplasia associated with cystic lesions in the reproductive system. METHODS: Between 1991 and 2007, we treated 26 patients with unilateral renal agenesis or renal dysplasia associated with pelvic, retrovesical or interlabial cystic lesions. In 16 patients, an abnormality either of the kidney or a cystic lesion was detected during the perinatal period. Another 10 patients presented clinical symptoms, including urinary incontinence in five, urinary tract infection in one, and vaginal discharge in four. RESULTS: Based on clinical features and imaging findings, the patients were divided into four groups: those with Gartner's duct cyst (Group 1, n = 9); those with uterovaginal duplication with obstructed hemivagina (Herlyn-Werner-Wunderlich syndrome [HWW]; Group 2, n = 3); those with both Gartner's duct cyst and HWW syndrome (Group 3, n = 3); and those without definitive diagnosis (Group 4, n = 11). In Group 1, leakage completely stopped after nephrectomy in three patients, whereas six patients continued to be incontinent despite the removal of dysgenetic kidneys. In Group 2, after the excision of a vaginal septum, no patient presented urinary incontinence. In patients in Group 3, both the mesonephric remnant and mullerian structures were confirmed on imaging or through endoscopy. CONCLUSIONS: Because of the high coincidence of genital and renal anomalies, it is recommended that genital systems in female patients with renal anomalies associated with cystic lesions behind the lower urinary tract be investigated. The best imaging modality to use remains under scrutiny. It is importantt to follow the patients until the age of puberty. The importance of a long-term follow-up in these patients needs to be emphasized.
OBJECTIVES: To review our experience with female children who have unilateral renal agenesis or renal dysplasia associated with cystic lesions in the reproductive system. METHODS: Between 1991 and 2007, we treated 26 patients with unilateral renal agenesis or renal dysplasia associated with pelvic, retrovesical or interlabial cystic lesions. In 16 patients, an abnormality either of the kidney or a cystic lesion was detected during the perinatal period. Another 10 patients presented clinical symptoms, including urinary incontinence in five, urinary tract infection in one, and vaginal discharge in four. RESULTS: Based on clinical features and imaging findings, the patients were divided into four groups: those with Gartner's duct cyst (Group 1, n = 9); those with uterovaginal duplication with obstructed hemivagina (Herlyn-Werner-Wunderlich syndrome [HWW]; Group 2, n = 3); those with both Gartner's duct cyst and HWW syndrome (Group 3, n = 3); and those without definitive diagnosis (Group 4, n = 11). In Group 1, leakage completely stopped after nephrectomy in three patients, whereas six patients continued to be incontinent despite the removal of dysgenetic kidneys. In Group 2, after the excision of a vaginal septum, no patient presented urinary incontinence. In patients in Group 3, both the mesonephric remnant and mullerian structures were confirmed on imaging or through endoscopy. CONCLUSIONS: Because of the high coincidence of genital and renal anomalies, it is recommended that genital systems in female patients with renal anomalies associated with cystic lesions behind the lower urinary tract be investigated. The best imaging modality to use remains under scrutiny. It is importantt to follow the patients until the age of puberty. The importance of a long-term follow-up in these patients needs to be emphasized.