Herlyn-Werner-Wunderlich syndrome: Report of a delayed diagnosed case with video presentation of the operative technique of vaginal septum resection

Abstract

Herlyn-Werner-Wunderlich (HWW) syndrome is a rare congenital anomaly of Mullerian duct development characterized by uterus didelphys with blind hemivagina and ipsilateral renal agenesis. We present a case of a 29-year-old nulliparous woman, who was referred to our hospital complaining of chronic pelvic pain, dyspareunia, and a palpable mass in her vagina. At the age of 12, she underwent surgery because of a didelphys uterus diagnosis. Subsequently, she was operated on twice for endometriomas. At our institution, clinical and imaging findings revealed an obstructed hemivagina setting the diagnosis of HWW syndrome. Some of the various syndrome types may go unnoticed for months or even years after the onset of menstruation. Early diagnosis, followed by proper surgical treatment, is the key to avoid potentially severe complications.

Keywords: Herlyn-Werner-Wunderlich syndrome; didelphys uterus; obstructed hemivagina; vaginal septum resection.