OHVIRA syndrome: A rare Mullerian abnormality
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Uterine didelphys, an obstructed hemivagina and ipsilateral renal agenesis form the constellation of OHVIRA syndrome . The incidence ranges 0.5-5%. An embryological arrest in fusion of mesonephric and paramesonephric ducts in 8th week of gestation is responsible for this entity.

Here presents a case of a 17 year old girl, presented with severe dysmenorrhea since menarche, attained at the age of 14. She had previously received medical management for the same, however her symptoms had worsened over the last 6 months. On examination her vital parameters were normal. The Systematic examination was unremarkable. Local genital examination revealed a normal vaginal orifice. A possibility of Mullerian anomaly was suspected. An ultrasonography revealed an absent right kidney and suspicion of hematometra. An MRI was advised for detailed evaluation, revealing two uterine cavities, one filled with hypoechoic collection suggestive of hematometra and a huge hematocolpos on the same side.

In summary the clinical presentation is perplexing. MRI is highly sensitive, especially useful in these cases. The mainstay of treatment is to relieve the obstruction by resection of the vaginal septum. The advent in hysteroscopy and vaginoscopy with the safety of bipolar energy and saline media makes this feasible . Future fertility rates are good, with 80% conception rate.

Source : https://www.jmig.org/article/S1553-4650(20)30118-7/fulltext?rss=yes
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