Herlyn–Werner–Wunderlich syndrome

Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA), also known as Herlyn–Werner–Wunderlich syndrome, is a rare syndrome constituting 0.16–3% of all the mullerian duct anomalies. Patients are symptomatic shortly after menarche when hematocolpos develop during menstruation, resulting in dysmenorrhea and a pelvic mass. Ultrasonography and magnetic resonance imaging (MRI) are the usual investigations for the diagnosis. Vaginoplasty with septum resection is the treatment of choice to prevent complications such as endometriosis or adhesions from chronic infections with subsequent infertility. Here, we present a case of 13-year-old girl with similar presentation during her second menses and who was symptomatically relieved by vaginal septostomy with marsupialisation. Key words: Didelphic uterus; Herlyn–Werner–Wunderlich syndrome; mullerian duct anomalies; Obstructed hemivagina and ipsilateral renal anomaly