Unusual clinical presentations, pathogenesis and radiological review of Herlin-Werner-Wunderlich syndrome: A case report and literature review

Abstract

Herlyn-Werner-Wunderlich (HWW) syndrome is a rare anomaly of the female urogenital tract characterized by the combination of uterine didelphys, obstructed hemivagina, and ipsilateral renal anomalies. The exact incidence of the syndrome remains unknown, but it has been reported to be 1 per 2000 to 1 per 28,000 women. It is believed that the triad is a mesonephric duct-induced paramesonephric duct anomaly. In majority of the cases with complete hemivaginal obstruction, the pathology is diagnosed after menarche due to retained menstrual flow. While the common clinical presentations are dysmenorrhea, pelvic pain, intermenstrual bleeding, and pelvic mass, it can also manifest itself with unusual gastrointestinal and urinary tract symptoms. We present a case of HWW syndrome with gastrointestinal symptoms like worsening constipation and abdominal fullness.The unusual clinical presentation of this syndrome makes diagnosis more challenging. To solve such medical puzzles and prevent complications, detailed history-taking and radiological guidance are critical.

Keywords

• Mullerian duct Anomaly
• Wollfian duct
• Hemivagina
• Genitourinary system
• Constipation