OHVIRA syndrome, is a rare in-utero developmental defect affecting the female urogenital system, classically
described as obstructed hemivagina and ipsilateral renal anomaly. Majority of the patients have clinical presentations of
abdominal or pelvic pain, usually shortly post-menarche. Early detection and prompt treatment relieve symptoms and
prevent complications. Similar presentations may be due to other entity of Mullerian abnormalities associated with single
renal agenesis. Three cases illustrating the clinical presentations, the dilemma in achieving diagnosis and the treatment
advocated for the patients. Case 1 is a classic OHVIRA syndrome while the other 2 cases are variants of the classic
syndrome; the second case showed a contralateral renal agenesis and the third case had variants of Mullerian anomaly.
High index of suspicion can help in the diagnosis of this rare OHVIRA syndrome, and there are possibilities of
discovering new rare syndromes yet to be named.
Keywords: Mullerian duct anomaly, obstructed hemivagina, OHVIRA, uterine didelphy, vaginoplasty.
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