MRI Findings of Obstructed Hemivagina and Ipsilateral Renal Agenesis (OHVIRA syndrome) with a Blind Megaureter: Case Report

Abstract

Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome is anuncommon congenital abnormality of the female urogenital tract characterized bythe triad of uterine didelphys, obstructed hemivagina, and ipsilateral renal agenesis. A 13-year-old female presented with acute lower abdominal pain. Magneticresonance imaging (MRI) revealed uterine didelphys, hematometrocolpos, obstructedhemivagina, and right ipsilateral agenesis, consistent with OHVIRA syndrome. Also, awell-defined mass with fluid signal intensity, mimicking adnexal neoplasm was seenin the right lower pelvic cavity adjacent to the posterior wall of the bladder. Vaginalseptotomy and drainage of hematometrocolpos were done initially, but unilateralhysterectomy was later performed to relieve the patient’s symptoms. The cystic massin the right lower pelvic cavity was also excised and confirmed as a blind megaureter.