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

OBJECTIVE

To report a rare müllerian 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 müllerian 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 müllerian classification system with a subsequent literature search.

RESULT(S): Successful drainage of hematocolpos and hematometra, and subsequent normal menstruation.

CONCLUSION(S): This rare müllerian 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.