Late presentation, MR imaging features and surgical treatment of Herlyn-Werner-Wunderlich syndrome (classification 2.2); a case report

BACKGROUND: Herlyn-Werner-Wunderlich syndrome is a very rare congenital genitourinary anomaly characterized by uterus didelphys, blind hemivagina and ipsilateral renal agenesis. CASE PRESENTATION: Authors present a case of Herlyn-Werner-Wunderlich syndrome in a 19-year-old unmarried woman who presented with pelvic pain and pelvic mass. MR imaging revealed the typical features of didelphys uterus, obstructed right hemivagina and ipsilateral renal agenesis. The patient subsequently underwent surgery. CONCLUSIONS: Herlyn-Werner-Wunderlich syndrome would be suspected in patients with unilateral absent kidney and pelvic mass. Ultrasonography and MR imaging can well depict the disease entity and surgery is the treatment of choice for obstructed hemivagina.