19/10/2022
Obstructed hemivagina, ipsilateral renal agenesis (OHVIRA) syndrome is one such complex congenital developmental anomaly of the genitourinary tract characterized by didelphic uterus, obstructed hemivagina, and ipsilateral renal agenesis. Incidence of Mullerian duct anomalies ranges from 0.8% to 4% and the incidence of the OHVIRA syndrome is estimated to be between 0.1-3.5% of all Mullerian anomalies. The classical presentation is of a young girl presenting with severe dysmenorrhea. Pelvic pain is the most common presenting symptom (90%) followed by an abdominal mass (40%) and pressure symptoms. Patients can also present at a later age with foul-smelling vaginal discharge due to pyocolpos. Because of regular menstruation in these patients (at menarche), diagnosis is often delayed. Patients can present with complications like endometriosis, hydrosalpinx, secondary infection and pelvic adhesions causing chronic pelvic pain. Thus if not diagnosed and treated in time, it can lead to long-term problems like infertility.