Herlyn-Werner-Wunderlich syndrome: An unusual presentation with pyocolpos

Herlyn-Werner-Wunderlich syndrome is a rare congenital anomaly of the urogenital tract, which is characterized by the triad of uterus didelphys, obstructed hemivagina, and ipsilateral renal agenesis. It usually presents at puberty with pelvic pain, dysmenorrhea, and a vaginal or pelvic mass. Although rare, it may present with purulent vaginal discharge due to secondary infection of the obstructed hemivagina, making diagnosis difficult. A careful pelvic examination to identify the cervix and vagina is the key to the diagnosis of Müllerian duct anomalies and magnetic resonance imaging can provide additional useful information. The optimal treatment is full excision and marsupialization of the obstructing vaginal septum so that both uteri can drain through the patent vagina. The authors report a case of a 22-year-old female with an unusual presentation of Herlyn-Werner-Wunderlich syndrome complicated by pyocolpos, which was successfully managed by vaginal septum resection and drainage of pus.

Multiple Developmental Urogenital Anomalies: A Therapeutic Challenge.

Case report: In cases of atretic lower vagina, drainage of hematocolpos per se is inadequate as recurrent hematocolpos from re-stenosis is common. Surgical reconstruction in these cases should be directed to relieve obstruction and ensure continued vaginal patency. A 14-year-old girl reported with primary amenorrhea and recurrence of cyclical lower abdominal pain due to re-stenosis following a primary intervention for hematocolpos. Evaluation identified an atretic lower vagina and multiple associated urinary tract anomalies like unascended right kidney, malrotated left duplex collection system, ureteric diverticula and bladder diverticulum with left ureter opening into it. Though associated Grade IV vesicoureteral reflux (VUR) posed a management dilemma, drainage of hematocolpos and restoration of vaginal continuity by pull through of the proximal vagina and approximating its edges to fourchette relieved the patient of pain, restored menstruation, resolved the VUR and obviated the need for extensive urinary reconstructive procedures. Conclusion: In case of atretic lower vagina, drainage of hematocolpos per se is inadequate as recurrent hematocolpos from re-stenosis is common and surgical reconstruction should be directed to relieve obstruction and ensure continued vaginal patency. Coexisting developmental urinary tract anomalies may not require immediate surgical intervention.