Abstract
BackgroundHerlyn-Werner-Wunderlich syndrome is a rare congenital anomaly characterized by uterus didelphys, obstructed hemivagina, and ipsilateral renal agenesis. The most common presentation is abdominal pain, dysmenorrhea, and abdominal mass secondary to hematocolpos. We present the first case report on Herlyn-Werner-Wunderlich syndrome from Bangladesh.Case presentationA 15-year-old Asian girl presented with lower abdominal pain of 3 months’ duration. She had had menarche 3 months earlier and had a regular menstrual cycle with cyclical abdominal pain. Abdominal examination found a tender mass on the right iliac fossa. Further evaluation with ultrasound revealed distended endometrial cavity filled with complex fluid and nonvisualization of the right kidney. Pelvic magnetic resonance imaging showed absent right kidney and two separate endometrial stripes surrounded by endometrium and a muscular layer. The right endometrial cavity and cervix were distended with blood. This magnetic resonance imaging finding is consistent with Herlyn-Werner-Wunderlich syndrome with uterine didelphyis, right-sided hematometra resulting from obstructed hemivagina, and ipsilateral agenesis of the right kidney. The vaginal septum was resected for vaginoplasty. She was discharged 5 days after surgery and came for follow-up after 7 days. Vaginal examination revealed a healthy wound with no adhesion of the vaginal wall. She also informed us that she had started regular menstruation without any pain 30 days after the operation.ConclusionAn unusual presentation of regular menstruation and nonspecific abdominal pain delays the diagnosis, which can lead to complications such as endometriosis and infertility. Awareness is required; otherwise, misdiagnosis clearly can occur.
Highlights
Herlyn-Werner-Wunderlich syndrome is a rare congenital anomaly characterized by uterus didelphys, obstructed hemivagina, and ipsilateral renal agenesis
We describe a case of a young girl with the triad of uterus didelphys, obstructed hemivagina, and right renal agenesis who was diagnosed by pelvic ultrasound, with confirmation by magnetic resonance imaging (MRI), and successfully managed by transvaginal resection of the vaginal septum
We present a case of HWW syndrome in a 15-year-old girl who presented with a regular menstrual cycle and cyclical abdominal pain since her menarche
Summary
Herlyn-Werner-Wunderlich syndrome is a rare congenital anomaly characterized by uterus didelphys, obstructed hemivagina, and ipsilateral renal agenesis. The right endometrial cavity and cervix were distended with blood This magnetic resonance imaging finding is consistent with Herlyn-Werner-Wunderlich syndrome with uterine didelphyis, right-sided hematometra resulting from obstructed hemivagina, and ipsilateral agenesis of the right kidney. We describe a case of a young girl with the triad of uterus didelphys, obstructed hemivagina, and right renal agenesis who was diagnosed by pelvic ultrasound, with confirmation by magnetic resonance imaging (MRI), and successfully managed by transvaginal resection of the vaginal septum. Case presentation A 15-year-old Asian girl presented to the emergency department of Cumilla Medical College and Hospital, Bangladesh, with increasing pain in the right lower abdomen of 3 months’ duration. Colicky pain in the right lower abdomen with the onset of
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