A Rare Case of OHVIRA Syndrome with Urethral Stenosis

A bstrAct Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) is a rare anomaly of the urogenital system. The characteristic triad of this syndrome, which was initially reported in 1950, is didelphys uterus, obstructed hemivagina, and ipsilateral renal agenesis (Embrey 1 ). The prevalence of congenital Müllerian duct anomalies is reported to be 1%. 2 A 24-year-old girl got admission to MGH on 15.3.2019 with lower abdominal pain and difficulty in the passing of urine. Her menarche was established 6 months back with a regular cycle and associated with dysmenorrhea. Magnetic resonance imaging (MRI) established the diagnosis of OHVIRA. She underwent surgery for drainage of the hematocolpos and excision of the vaginal septum and urethral dilatation with cystoscopy followed by an uncomplicated recovery and the patient had normal menstrual cycles after surgery.