Double uterus-hemivagina-renal agenesis syndrome

Double uterus-hemivagina-renal agenesis syndrome, also known as Herlyn-Werner-Wunderlich (HWW) syndrome or obstructed hemivagina and ipsilateral renal anomaly (OHVIRA), is a rare congenital malformation involving the Müllerian (paramesonephric) and Wolffian (mesonephric) duct systems. It is characterized by a triad of findings: uterus didelphys (double uterus), obstructed hemivagina (typically caused by a longitudinal vaginal septum), and ipsilateral renal agenesis (absence of one kidney on the same side as the vaginal obstruction). These three features arise from a developmental defect during embryogenesis affecting the coordinated development of the reproductive and urinary tracts. The condition predominantly affects females and is often diagnosed around the time of puberty (menarche), when menstrual blood accumulates behind the obstructed hemivagina, forming a hematocolpos. Patients typically present with progressive pelvic pain, a pelvic mass, dysmenorrhea, or abnormal vaginal discharge that may become foul-smelling if secondary infection occurs. Some patients may also develop hematometra (blood accumulation in the uterus) or endometriosis due to retrograde menstruation. Renal agenesis is usually asymptomatic and may be discovered incidentally during imaging for the gynecological symptoms. Diagnosis is established through imaging modalities such as pelvic ultrasound, MRI, and sometimes hysteroscopy or laparoscopy. Treatment is primarily surgical and involves excision or marsupialization of the obstructing vaginal septum to allow drainage of the obstructed hemivagina. This procedure is generally curative for the obstructive symptoms and has excellent outcomes. Following appropriate surgical management, most patients retain normal menstrual function and have favorable reproductive potential, although they may face a higher risk of obstetric complications related to the uterine anomaly. Long-term follow-up is recommended to monitor for potential complications including endometriosis and to provide appropriate obstetric counseling.

Common questions about Double uterus-hemivagina-renal agenesis syndrome