Uterus Didelphys with an Obstructed Unilateral Vagina by a Transverse Vaginal Septum Associated with Ipsilateral Renal Agenesis: OHVIRA Syndrome − An Unusual Case Report and Review of Literature

Abstract

The triad of uterine didelphys, obstructed hemivagina, and unilateral renal agenesis, called as OHVIRA syndrome is the least common of all Mullerian malformations. We report a case of a 16-year-old girl who presented with lower abdominal pain and amenorrhea. Radiological investigations led us to the diagnosis of OHVIRA syndrome. She was hospitalized and operated upon. OHVIRA syndrome, though a rare Mullerian anomaly, should be kept as a differential diagnosis in a young girl presenting with cyclical pain and amenorrhea with renal agenesis so that surgical management can be performed before long-term complications set in.

Keywords

• Mullerian
• obstructed hemivagina
• OHVIRA
• renal agenesis
• uterine didelphys