An unusual presentation of uterus didelphys with obstructed hemivagina with ipsilateral renal agenesis

Objective: To report a rare mullerian anomaly of uterus didelphys with obstructed hemivagina and renal agenesis, and its unusual presentation that mimicked an ischiorectal swelling. Design: Case report. Setting: Private clinic and surgical center. Patient(s): A 17-year-old woman referred for evaluation of a suspected mullerian anomaly. Intervention(s): Clinical, hysteroscopic, and laparoscopic evaluation of the anomaly, with treatment and follow-up assessments. Main Outcome Measure(s): Assessment of the anomaly according to the standard mullerian classification system with a subsequent literature search. Result(s): Successful drainage of hematocolpos and hematometra, and subsequent normal menstruation. Conclusion(s): This rare mullerian anomaly, also known as Herlyn-Werner-Wunderlich syndrome (HWWS), presented as a huge paravaginal mass. Good long-term outcome occurred after vaginal septectomy. This diagnosis should be suspected in women who have a pelvic mass and ipsilateral renal agenesis. (Fertil Steril (R) 2008;90:849. e9-e10. (C) 2008 by American Society for Reproductive Medicine.)