OHVIRA syndrome: a rare mullerian anomaly with diagnostic and management challenges: a case report and review of literature

Abstract

Herlyn–Werner–Wunderlich syndrome, also known as OHVIRA syndrome is a rare complex congenital developmental anomaly characterized by the triad of uterine didelphys, obstructed hemivagina, causing hematometrocolpos and ipsilateral renal anomaly. A 19-year-old nulligravida presented with foul-smelling vaginal discharge for 11 months and cyclical lower abdominal pain for 2 months. She had regular menstrual cycles since menarche. Examination revealed a single cervix with an anterolateral vaginal bulge draining mucopurulent discharge. Imaging with ultrasonography and MRI confirmed uterine didelphys, hematocolpos, right renal agenesis and hemivaginal septum. The patient had undergone prior aspiration elsewhere. Diagnostic hysterolaparoscopy confirmed the findings. Excision of the vaginal septum revealed the obstructed cervix and a vaginal mould was placed postoperatively. The patient remains asymptomatic on follow-up and has conceived. High index of suspicion during evaluation of obstructive reproductive tract anomalies, early diagnosis through imaging modalities, multidisciplinary approach, timely surgical management and follow up yield good reproductive outcomes and quality of life.