English

Abstract

INTRODUCTION: Congenital anomalies of the mullerian duct system can result in various urogenital anomalies and, Herlyn Werner Wunderlich syndrome (HWW syndrome) is one such rare anomaly. This syndrome is characterized by uterus didelphys with blind hemi vagina and ipsilateral renal agenesis.1 it is also known as OHVIRA (Obstructed Hemi vagina with Ipsilateral Renal Anomaly) syndrome.2 Mullerian anomalies are frequently associated with renal anomalies, the incidence ranges from 0.2 to 7.4%.3 Diagnosis of this condition is rarely made in prepubertal girls, and rather it is diagnosed after menarche. Common clinical presentation is pelvic pain shortly after menarche with an associated vaginal or pelvic mass.4 this triad of obstructed hemi vagina, uterus didelphys and ipsilateral renal agenesis was first reported in 1950.5 The embryonic origin of HWW syndrome is not known, but early and accurate diagnosis of this condition is needed to enable treatment at an early stage. Surgery can provide relief from pelvic pain and preserve fertility by preventing endometriosis. Abdominal ultrasound and MRI help in the diagnosis. However, MRI has been considered as the imaging modality of choice.