Herlyn-Werner-Wunderlich syndrome - Common presentation of an unusual variation: A Case Report

Herlyn-Werner-Wunderlich syndrome also known as uterus didelphys with obstructing hemivaginal septum and ipsilateral renal agenesis is a rare syndrome with only a few hundred reported cases described since 1922. Uterus Didelphys is a type of lateral fusion disorder of mullerian ducts. Mullerian duct anomalies have an incidence of 2–3%, while obstructing hemivaginal septum and ipsilateral renal agenesis constitutes 0.16–10% of these mullerian duct anomalies. A female case referred to our radiology department for evaluation of lower abdominal pain revealed Uterus Didelphys with Obstructing Hemivaginal Septum and Ipsilateral Renal Agenesis on imaging. Understanding the imaging findings, in conjunction with the clinical presentation, is critical for early diagnosis in attempting to prevent complications such as endometriosis or adhesions from chronic infections with subsequent infertility. Management depends on the presence or absence of an obstruction. Surgical removal of the vaginal septum is the preferred approach for relieving an obstruction.