Gynatresia in Herlyn-Werner-Wunderlich Syndrome: A Case Report

Background: Female genital malformations are a consequence of abnormalities in the differentiation of the Müllerian ducts at different phases during embryogenesis, resulting in a variety of clinical outcomes. HerlynWerner-Wunderlich (HWW) syndrome is a rare congenital anomaly of the female urogenital tract involving the paramesonephric ducts (Müllerian ducts). The syndrome is characterized by the triad of uterus didelphys, obstructed hemivagina and ipsilateral renal agenesis. Case: This paper describes the case of a 14-year-old girl presenting with pelvic pain, abdominal swelling and the triad that is characteristic of HWW syndrome. Diagnosis was based on the patient’s clinical history and imaging findings. The patient underwent surgery involving complete drainage of a hematocolpos retained in the obstructed vaginal cavity and resection of the vaginal septum. The procedure was successful in providing symptomatic relief. Twelve months following surgery, the patient’s menstrual cycles were regular, with no dysmenorrhea. Discussion: Greater understanding of this syndrome is crucial in ensuring that diagnosis is reached at an early stage, thus preventing complications such as retrograde menstruation, endometriosis, infections, adhesions, infertility, and acute or chronic pelvic pain.