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Objective - To present a case of Herlyn-Werner-Wanderlich syndrome with ectopic uropoetic remnants, diagnosed only after extensive diagnostic workup, and to stress the importance of an active search in selected cases. Case report - We present a case of an 11-year-old female who was diagnosed with Herlyn-Werner-Wunderlich syndrome and soon after resection of the hemivaginal septum began to complain of urinary incontinence. She underwent extensive diagnostic workup, including voiding cystourethrography and surgical exploration, which excluded a vesico-vaginal fistula. Urodynamic studies for urinary incontinence were normal. Correct diagnosis was achieved with magnetic resonance urography, which revealed an ectopic ureter with insertion into the previously obstructed hemivagina. The ipsilateral ectopic dysplastic kidney was not recognised on several abdominal ultrasound or magnetic resonance urography examinations, but was eventually confirmed with Technetium-99m dimercaptosuccinic acid renal scintigraphy, followed by single-photon emission computed tomography. Laparoscopic surgical removal of the poorly functioning ectopic remnant of the left kidney was performed. Conclusion - The close relationship between female genital and uropoetic anomalies should lead us to examine the urogenital system when a genital anomaly is identified and vice-versa. The increased awareness and availability of modern diagnostic techniques, which efficiently disclose these lesions, should prompt the correct diagnosis of these complex anomalies.

Urinary incontinence following vaginal septum resection in Herlyn-Werner-Wunderlich syndrome – A case report