Endometriosis in association with Herlyn-Werner-Wunderlich syndrome.

OBJECTIVE: To describe the clinical characteristics of patients with Herlyn-Werner-Wunderlich syndrome (HWWS) and explore the relationship between an obstructive genital abnormality and pelvic endometriosis.

DESIGN: Case series.

SETTING: University hospital.

PATIENT(S): A total of 94 women with Herlyn-Werner-Wunderlich syndrome (HWWS).

INTERVENTION(S): Retrospectively reviewed charts of patients diagnosed with HWWS and follow-up contact.

MAIN OUTCOME MEASURE(S): Data for each patient regarding demographics, presenting symptoms, concomitant complications, and anatomic variations were obtained from operation notes and clinical records.

RESULT(S): The occurrence of pelvic endometriosis was 19.15% (18/94) in patients with HWWS. All ovarian endometrial cysts (100%) occurred ipsilateral to the vaginal septum. Pelvic endometriosis was significantly more frequent in patients with complete hemivaginal obstructions (10/27, 37.0%) than those with incomplete obstructions (8/67, 11.9%).

CONCLUSION(S): One-fifth of patients with HWWS were susceptible to pelvic endometriosis, and all of the ovarian endometriotic cysts were ipsilateral to the vaginal septum. Pelvic endometriosis in adolescents appeared to be related to the obstructed genital abnormality. Vaginal septum resection should be the first step in treatment and surgery has an important role in the treatment of endometriosis and pelvic adhesion.