Herlyn-Werner-Wunderlich Syndrome: A Rare Triad of Müllerian and Renal Anomaly
Herlyn-Werner-Wunderlich Syndrome: A Rare Triad of Müllerian and Renal Anomaly Herlyn-Werner-Wunderlich (HWW) syndrome, also referred to as Obstructed Hemivagina and Ipsilateral Renal Anomaly (OHVIRA), is a rare congenital disorder characterized by a unique triad: uterine didelphys (complete duplication of the uterus and cervix), obstructed hemivagina (often presenting as hematocolpos), and ipsilateral renal agenesis. Though rare, timely diagnosis is crucial to prevent long-term complications like endometriosis, infertility, and infection. 1. Etiology and Pathogenesis Herlyn-Werner-Wunderlich syndrome results from abnormal development and fusion of the Müllerian (paramesonephric) ducts during embryogenesis. Normally, these ducts fuse in the midline to form the uterus, cervix, and upper two-thirds of the vagina, while the Wolffian (mesonephric) ducts contribute to kidney and ureteral development. In HWW syndrome: One Müllerian duct fails to fuse or canalize properly. A longitudinal...