Radio-diagnosis of Herlyn Werner Wunderlich Syndrome a rare case of urogenital abnormality

Herlyn-Werner-Wunderlich syndrome (HWWS) is a very rare urogenital abnormality, which is characterized by Mullerian duct anomaly with associated mesonephric duct anomaly. This syndrome is also known as OHVIRA (Obstructed hemivagina with Ipsilateral Renal Anomaly). The incidence of Mullerian Duct Anomaly is 2-3%, and incidence of OHVIRA is 0.16 to 10% of that. The three important components of Herlyn-Werner-Wunderlich (HWW) syndrome are didelphys uterus, obstructed hemivagina and renal agenesis on the same side. Typically presents with pelvic pain in young females after menarche. Presentation in the reproductive age group is unusual. The most common renal anomaly associated with this condition is renal agenesis. Other renal anomalies include renal dysplasia, duplication of kidneys and ureters, crossed fused ectopia and ectopic ureter. Ultrasound and Magnetic resonance imaging are considered as the vital modalities for detection. MRI is the gold standard modality for assessing the anatomical characteristics of the uterus, cervix, and vagina. Early diagnosis helps to prevent complications such as endometriosis, pyocolpos, adhesions, infertility. The unique feature of our case is a presentation in the reproductive age group. Only very few case reports of OHVIRA with a presentation in the reproductive age group are available in the literature. Whenever there is a renal anomaly or renal agenesis, screening of pelvic organs is important in a female child for early detection of Mullerian duct anomalies and prevention of further complications.