Herlyn-Werner-Wunderlich (HWW) Syndrome in an Adolescent

Herlyn-Werner-Wunderlich (HWW) syndrome, also known as obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome, is a rare Müllerian duct anomaly characterized by the triad of uterus didelphys, obstructed hemivagina, and ipsilateral renal agenesis. Early diagnosis is essential to prevent complications and preserve reproductive potential. We report the case of a 14-year-old female patient with significant dysmenorrhea and menstrual irregularities since menarche. The initial pelvic ultrasound was unremarkable. Nine months later, she presented repeatedly to the emergency department with left flank pain and vomiting. Further evaluation revealed the absence of the left kidney, uterus didelphys with an obstructed left hemi-uterus, hematocolpos, hematosalpinx, left ovarian endometrioma, and left renal agenesis on magnetic resonance imaging (MRI). Surgical management included laparoscopic hemihysterectomy of the left uterus and ipsilateral salpingectomy. Postoperatively, the patient was started on continuous progestogen-only therapy, with marked improvement in symptoms and quality of life. HWWsyndrome typically manifests soon after menarche with cyclic pelvic pain, dysmenorrhea refractory to treatment, or pelvic mass. MRI is the gold standard for diagnosis, allowing precise anatomical characterization and guiding surgical planning. Without a functional or communicating obstructed hemicavity, hemihysterectomy with salpingectomy is an effective approach, preventing recurrence and reducing the risk of complications such as infection and endometriosis. This case highlights the importance of maintaining a high index of suspicion for HWW syndrome in adolescents with refractory dysmenorrhea and associated renal anomalies. Early diagnosis and individualized surgical management can relieve symptoms, improve quality of life, and preserve reproductive potential.