Successful management of Herlyn–Werner–Wunderlich syndrome in a 17-year-old virgin girl in the challenging socio-cultural-logistic setting of Syria: a case report

Abstract

Introduction and Importance: Herlyn–Werner–Wunderlich syndrome (HWWS) is a rare developmental anomaly of the female reproductive system caused by a failure of fusion during Mullerian duct development. The triad of uterus didelphys, obstructed hemivagina, and ipsilateral renal agenesis characterizes HWWS. The most common presenting symptoms are dysmenorrhoea, pelvic pain, primary infertility in later years, and an abdominal mass due to hematometrocolpos. Case Presentation: A 17-year-old girl presented to the authors’ department with recurrent low back pain, which was neither responsive to analgesics nor associated with urinary complaints, vomiting, or fever. Imaging techniques confirmed she had the triad of uterus didelphys, obstructed hemivagina, and right renal agenesis. Clinical Discussion: The genital system is the same for males and females before 6 weeks of pregnancy. HWWS is a rare congenital disorder as a result of the failure of fusion during Mullerian duct development. It consists of a didelphic uterus, hemivaginal septum, and unilateral renal agenesis. Conclusion: Shame and social stigma associated with virginity continue to endanger the lives of many girls in Syria. To complicate matters further, the low resources in Syria produced by war pose a difficult challenge in managing many gynecological conditions, including HWWS, like this case, in which endoscopic technologies were not available, necessitating open surgery while keeping in mind preserving hymen intactness. So, the authors indicate that preserving virginity could be conducted even though the approach is open surgery by very careful intervention and experienced surgeons.