Obstructed Hemivagina and Ipsilateral Renal Agenesis (OHVIRA) Syndrome Associated with Bicornuate Uterus with Recurrent Hematopyo-metra with Reactive Thrombocytosis, A Case Report

The development of the female urinary and genital system is deeply linked at the embryological level. About 10% of infants are born with abnormality of the genitourinary system, and anomalies in one system are often mirrored by anomalies in another system.¹ The OHVIRA syndrome is a rare set of anomalies affecting the female urogenital tract. The syndrome consists of obstructed hemivagina with ipsilateral renal agenesis with uterine anomalies. The true incidence of the OHVIRA syndrome is not precisely known, but according to the available literature it is estimated between 0.1-3.5 percent of all mullerian anomalies. It is mostly reviewed in western literature under pediatric surgery, as the median age of presentation is 12.5 years (birth-25yrs), but in our setup it usually falls into the gynecologists purview as the age of presentation is in early childbearing age. A 22 year old married patient was admitted in our hospital as a referred case of abdominopelvic mass with dysmenorrhea. The patient after complete evaluation was shown to have a uterus bicollis unicornis, with obstructed hemivagina absent right kidney with unilateral right hydrosalpinx and reactive thrombocytosis. Here, we present the diagnostic modalities that helped us reach the diagnosis of this rare syndrome, its associated features and surgical management of an adult patient in a low cost setting.