Herlyn-Werner-Wunderlich syndrome in a multiparous female

Herlyn-Werner-Wunderlich syndrome is a rare complex congenital anomaly of the urogenital tract involving Mullerian ducts and mesonephric ducts. It is also called OHVIRA syndrome (Obstructed Hemivagina and Ipsilateral Renal agenesis). It is characterized by a triad of uterus didelphys, obstructed hemivagina and ipsilateral renal agenesis. Patients usually present after menarche with pelvic pain, dysmenorrhea, mass, and rarely with primary infertility in later years. We report a case of a multiparous female who presented to the hospital with pain in lower abdomen for the past 2 months and acute retention of urine for 1 day. This is an atypical presentation in a multiparous female that has been described in a single case report so far.¹ Intravenous pyelogram and MRI of the patient revealed uterine didelphys, obstructed right hemivagina causing hematohemicolpos and right renal agenesis. Thorough knowledge of imaging features can enable a radiologist to make a correct diagnosis even in an atypical presentation.