Herlyn-Werner-Wunderlich syndrome - an uncommon cause of abdominal pain

Abstract

Introduction: Herlyn–Werner–Wunderlich (HWW) syndrome is an uncommon congenital anomaly of the Müllerian ducts characterized by the coexistence of uterine didelphys, an obstructed hemivagina, and ipsilateral renal agenesis. Symptoms typically appear after menarche, most often as progressively worsening dysmenorrhea, or pelvic pain. Early recognition of this condition is essential to prevent long-term reproductive and renal complications. Case Presentation: A 12-year-old adolescent with a known history of right renal agenesis presented to the pediatric emergency department with progressively worsening dysmenorrhea over six months following menarche. Physical examination revealed a palpable mass in the right iliac fossa. Imaging studies confirmed right renal agenesis and identified hematometra, hematocolpos, uterine didelphys, and an obstructive longitudinal vaginal septum on the right side. A diagnosis of HWW syndrome was established. The patient underwent elective vaginal septoplasty with drainage of the hematocolpos and hematometra, performed without complications. At six-month follow-up, she remained asymptomatic and exhibited regular menstrual cycles. Discussion: HWW syndrome is an infrequent yet important cause of secondary dysmenorrhea and pelvic pain in adolescents. Because one hemivagina remains patent, menstrual blood can drain normally on that side, while the obstruction of the opposite hemivagina leads to progressive accumulation of blood and formation of a hematocolpos. If the condition is not identified promptly, patients may develop complications, including infections, endometriosis, or impaired fertility. Conclusions: Clinicians should consider HWW syndrome in adolescents with renal anomalies who present with dysmenorrhea or pelvic pain. Early multidisciplinary evaluation supports timely treatment and protects future reproductive health.