Ohvira syndrome – a case report

Back ground: OHVIRA syndrome is a very rare congenital Uro-genital anomaly associated with both the Mullerian (paramesonephric) and Wolffian (mesonephric) systems. It is characterized by a triad, OHV – Obstructed Hemi vagina, IRA—Ipsilateral Renal Agenesis with associated uterus didelphs. Mullerian Duct anomalies 0.8-4% of the women who came for evaluation of infertility. OHVIRA syndrome-0.1-3.5% of the Mullerian Duct Anamoly. Aims: To discuss and analyse the radiological and embryological co-relation in OHVIRA syndrome and study the genesis of both the urinary and genital systems. Materials and Methods: A 17 year old girl, nulligravida, presented to Kamineni Institute of Medical Sciences, Narketpally, with progressive development of cyclic lower abdominal discomfort and a large abdomino- pelvic mass. She was thoroughly investigated including the 3 imaging modalities like USG, CECT and MRI which was in favour of OHVIRA syndrome. Observation: On examination revealed a large, tense, protuberant mass extending slightly beyond her umbilicus, corresponding to 20weeks of gestation. Conclusions: An early correct diagnosis is the goal to relieve the symptom and prevent complications caused by retrograde menstruation which may lead to endometriosis and subsequently to conception abilities; a two step vagino-plasty can be performed to correct the anomaly.