Female counterpart of the Zinner syndrome in men: decoding the ‘OHVIRA’ syndrome

The obstructed hemivagina and ipsilateral renal agenesis (OHVIRA) syndrome, a rare Mullerian duct anomaly, is a triad of uterine anomalies with ipsilateral renal agenesis and obstructed hemivagina. The aetiopathogenesis of this developmental anomaly is debatable, with several theories being postulated to explain its occurrence. We report two cases of this rare syndrome which were detected on imaging done for unrelated reasons. Case 1 is a 24-year-old woman who presented with primary infertility. After an incidental detection of the syndrome and ruling out a male factor, she has been planned for excision of the vaginal septum. Case 2 is a 47-year-old woman with OHVIRA detected during the evaluation of urolithiasis and is asymptomatic for the syndrome. The OHVIRA syndrome can be asymptomatic or may present as haematocolpos, pelvic pain and infertility which requires surgical intervention, after which successful pregnancies have been reported.