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

Abstract

The obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome is a rare congenital anomaly of the Mullerian (paramesonephric) and Wolffian (mesonephric) ducts. It was initially reported in 1922 by Purslow and also referred to as the HerlynWerner-Wunderlich syndrome. It typically occurs concurrently with uterine didelphys and ipsilateral renal agenesis (1-5). Ipsilateral renal agenesis can be accompanied with other urologic anomalies simultaneously. A blind ectopic ureter combined with ipsilateral agenesis and Mullerian duct anomalies is extremely rare (6, 7). To our knowledge, a blind ectopic ureter associated with OHVIRA syndrome has not been reported in English literature. The diagnosis of ureteral anomalies can be challenging due to their rare incidence and cystic features that can be mistaken for an adnexal lesion in female patients. Regarding this matter, MRI is considerably helpful not only for diagnosing Mullerian duct anomalies, but also for detecting and characterizing associated non-uterine anomalies (1-5). In this case report, we present an unusual case of a 13-year-old girl with OHVIRA syndrome and a blind ectopic megaureter, which was This is an Open Access article distributed under the terms of the Creative Commons Attribution Non-Commercial License (http://creativecommons.org/licenses/ by-nc/3.0/) which permits unrestricted non-commercial use, distribution, and reproduction in any medium, provided the original work is properly cited. Received: July 19, 2015 Revised: August 19, 2015 Accepted: August 27, 2015