Obstructed Hemivagina with Ipsilateral Renal Anomaly (OHVIRA) Syndrome- A Rare Congenital Anomaly

Objective: We present a rare case of Obstructed Hemivagina and Ipsilateral Renal Anomaly (OHVIRA) Syndrome with a single septate uterus. This syndrome is shrouded in controversies concerning its nomenclature and development. We attempt to highlight the different viewpoints surrounding this unusual condition, which can be quite distressing for the affected young girls and confusing the treating doctors. Design: Case report. Materials and Methods: Case details and review of literature. Results: A 14 year old girl was presented with recent onset severe dysmenorrhea and abdominal mass of twenty week sized uterus. She had already received treatment from different physicians and gynecologists, for her ailment, in her hometown. Imaging studies in our hospital revealed that this patient had absent right kidney, massive hematometrocolpos on right side with normal endometrial echo seen on the left side. She was diagnosed to have OHVIRA syndrome. She underwent septostomy of the vaginal septum, drainage of hematometrocolpos and hysterolaproscopy that showed single septate uterus with a broad fundus. Conclusion: OHVIRA is a rare syndrome of mullerian&wolffianduct abnormality. A simple excision of the vaginal septum can relieve the patient of her symptoms. Given the rarity of the syndrome it is frequently misdiagnosed or diagnosed late. Delayed diagnosis leads to endometriosis endangering her already compromised fertility potential. Hence greater awareness and early diagnosis and timely surgery can prevent future complications.