Acute urinary retention due to didelphys uterus associated with an obstructed hemivagina in a 5-month-old infant.

A double uterus associated with an obstructed hemivagina is a rare malformation syndrome that has been described in about one hundred cases in the world literature. The malformation is diagnosed after menarche because of symptoms caused by hematocolpos or hematometra, and it is occasionally associated with ipsilateral renal agenesis. In the case described here, the malformation was symptomatic when the patient was 5 months old, and was associated with ipsilateral renal hypoplasia. The clinical and etiologic implications are discussed.