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

• Main Authors: Urvashi Chhikara, Latika Sahu, Asmita Rathore
• Format: Article
• Language: English
• Published: Wolters Kluwer Medknow Publications 2017-01-01
• Series: MAMC Journal of Medical Sciences
• Subjects: Mullerian; obstructed hemivagina; OHVIRA; renal agenesis; uterine didelphys
• Online Access: http://www.mamcjms.in/article.asp?issn=2394-7438;year=2017;volume=3;issue=1;spage=37;epage=39;aulast=Chhikara

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.