Term Pregnancy in Herlyn-Werner-Wunderlich Syndrome: Successful Management of Congenital Obstructed Hemivagina and Ipsilateral Renal Agenesis (OHVIRA).

BACKGROUND Obstructed hemivagina and ipsilateral renal agenesis (OHVIRA) syndrome, also known as Herlyn-Werner-Wunderlich syndrome, is a rare congenital anomaly characterized by a didelphic uterus, an obstructed hemivagina, and ipsilateral renal agenesis. This report describes a case of successful term pregnancy in a 20-year-old woman with OHVIRA syndrome. CASE REPORT A 20-year-old Saudi woman presented with primary infertility after 3 years of unsuccessful conception attempts, including failed assisted reproductive techniques. Her medical history was unremarkable, and menstrual cycles were regular but variable in flow. Pelvic ultrasound revealed a didelphic uterus and a fluid collection in the vaginal canal measuring 36.0×17.3 mm. MRI confirmed a didelphic uterus with an obstructed right hemivagina and hematocolpos. A renal ultrasound revealed right-sided renal agenesis, consistent with OHVIRA syndrome. Surgical management involved drainage of hematocolpos and excision of the obstructing vaginal septum. The patient recovered without complications. Eleven months later, she presented with missed menstruation and a positive ß-hCG level of 23 000 mIU/L. Ultrasound confirmed an intrauterine pregnancy in the right uterus. The pregnancy was uneventful, and she delivered a healthy male infant weighing 2.4 kg via elective cesarean section at 38 weeks of gestation. CONCLUSIONS Early recognition and accurate diagnosis of OHVIRA syndrome using appropriate imaging are essential for timely surgical intervention, which can significantly improve reproductive outcomes. This case highlights that successful full-term pregnancy is achievable following proper management.