Complications of vaginal atresia in association with a duplicated mullerian duct

A mullerian duct duplication with a vaginal atresia can present as a painful abdominal mass in a female caused by a hematometrocolpos and a hemosalpinx. This set of signs and symptoms, if caused by an associated unilateral vaginal atresia, may be found in a female with normal menstrual periods, and (1) can be misdiagnosed with often tragic consequences caused by unnecessary salpingectomy, or even hysterectomy; (2) can be successfully treated by creating an adequate channel for drainage through the area of vaginal atresia; and (3) has a high association with unilateral renal agenesis. Because of this last point, recognition of either a mullerian duct duplication or unilateral renal agenesis should prompt an investigation for the other abnormality.