Wunderlich--Herlyn--Werner syndrome

Abstract

A 25 year old married and nulliparous woman with regular menstrual cycles presented with dyspareunia, infertility, increasing dysmenorrhea and recent onset vulval swelling in the gynaec outpatient department at Bhaskar medical college & Bhaskar General Hospital, Moinabad, Telangana. Gynaecological examination, ultrasound, MRI & laparoscopy helped in the diagnosis of right hematometrocolpos with noncommunicating right hemivagina,uterus didelphys with absent right kidney and right ureter commonly named as Wunderlich--Herlyn--Werner syndrome or Obstructed Hemivagina with Ipsilateral Renal Agenesis(OHVIRA) syndrome. Left half of uterus, cervix , vagina along with left kidney and ureter were normal. Vaginal septal resection was done draining out the hematometrocolpos and patient’s symptoms were relieved. It also improved the chances for fertility. Wunderlich -Herlyn--Werner syndrome or Obstructed Hemivagina and ipsilateral renal agenesis (OHVIRA syndrome) is a rare mullerian anomaly.