Ohvira syndrome with rare presentations – A case report

Abstract

Herlyn Werner Wunderlich syndrome is a very rare congenital anomaly of the urogenital tract involving Mullerian ducts and mesonephric ducts. It is synonymous with OHIVRA syndrome which is Obstructed Hemivagina with Ipsilateral Renal Agenesis syndrome. Very few case reports have been reported regarding the syndrome. Herein, we report a case of 20 year old unmarried female with unusual presentation of OHVIRA syndrome with complaints of spotting per vagina and with vaginal cyst on examination. Diagnosis was confirmed by MRI which showed features of OHVIRA with pyometra caused by the obstructed hemivagina. She was managed surgically by diagnostic laparoscopy and per vaginal resection of the vaginal septum and drainage of around 50cc of pyometra followed by visualisation of two separate cervical canals post procedure. As a rare entity, a high degree of clinical suspicion is necessary to diagnose and intervene at the right time to ensure optimum results and prevent complications and preserve fertility.