Uterus didelphys with unilateral imperforate hemivagina and ipsilateral renal agenesis

Eight patients with a double uterus, unilateral vaginal obstruction, and ipsilateral renal agenesis are described. The most common clinical presentation was that of the onset of pelvic pain and dysmenorrhea, in association with the finding of a pelvic mass. In seven patients, a window was created between two vaginae by transvaginal route. In one patient, the blind vagina with hematocolpos and attending uterus were extirpated by an abdominal approach. The postoperative courses were uncomplicated in all patients. A greater awareness of this syndrome should lead to accurate diagnosis. Excision of the obstructing vaginal septum offers a complete relief of symptoms while preserving reproductive capacity.