The association of obstructed hemivagina with ipisilateral renal anomaly (OHVIRA) is a well-described entity. While there has been an increased familiarity with this disorder, the exact incidence of OHVIRA is unknown. Our aim was to review the available literature on this topic, look at common presentations, and uncommon presentations. This condition is a well-described entity but requires careful evaluation, because unique presentations do occur. Diagnostic challenges include time of presentation and symptoms associated with presentation. Surgical challenges include microperforation of the hemivaginal septum, pelvic inflammatory disease, thick septum, or high septum. Each of these must be managed carefully. Although a solitary kidney is frequently thought to be associated with OHVIRA, dysplastic kidneys, pelvic kidneys, or ectopic ureters can occur. Prompt and accurate diagnosis is essential for relief of symptoms and prevention of complications. To minimize problems associated with delayed diagnosis, magnetic resonance imaging evaluation is recommended along with referral to a center with expertise in these conditions.
Keywords: Dysplastic kidneys; Ectopic ureters; Obstructed hemivagina with ipsilateral renal anomaly; Pelvic kidneys; Solitary kidney.
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