Didelphic uterus, double vagina, unilateral vaginal obstruction with hematocolpos, and ipsilateral renal agenesis: The coexistence of endometriosis

Background: The infrequent syndrome of didelphic uterus, double vagina, unilateral vaginal obstruction with hematocolpos, and ipsilateral renal agenesis can be associated with pelvic and abdominal endometriosis presumably through the mechanism of menstrual blood reflux. The use of various techniques to diagnose the syndrome and the observation of the natural history of associated endometriosis in a case of severe disease are reported. Cases: Three cases of this disorder are reported. The patients were adolescent girls with recent onset of symptoms of different intensities. Individual diagnostic imaging techniques were not equally helpful in all three patients. The presence of endometriosis ranged from no clinically identifiable to Stage IV disease. A simple surgical technique was used to relieve the obstruction and simultaneous laparoscopy facilitated the procedure.Conclusion: Didelphic uterus, double vagina, unilateral vaginal obstruction with hematocolpos, and ipsilateral renal agenesis may have somewhat diverse manifestations. Although single diagnostic techniques may be misleading, a combination of diagnostic methods often proves efficacious. Surgery is generally easy and devoid of complications. Endometriosis, when present, may not need specific treatment. The long-term outcome is excellent.