Herlyn Werner Wunderlich Syndrome with Vagino-umbillical Fistula: A Most Unique Presentation

Abstract

The Herlyn–Werner–Wunderlich (HWW) syndrome is an uncommon Mullerian duct anomaly composed of a triad of obstructed hemivagina, renal agenesis, uterine didelphys with a prevalence of [Formula: see text] females. Aside from its usual presentation of cyclic hypogastric pain and abdominal mass, there has been no other reported case in literature of the syndrome presenting with a vagino-umbilical fistula. Presented here is a unique case of a 35-year-old woman presenting with purulent, foul-smelling discharge extruding from a fistula on her umbilicus and her vagina associated with a tender hypogastric mass. Three-dimensional transvaginal ultrasonography aided the diagnosis of HWW syndrome with a concomitant vagino-umbilical fistula. Excision of the vaginal septum together with antibiotic coverage resolved her symptoms. The syndrome’s embryopathogenesis, classification, diagnostics, definitive surgical management and other issues during follow up are discussed.