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Obstructed hemivagina and ipsilateral renal agenesis (OHVIRA) syndrome is a rare congenital defect of the M?llerian ducts characterized by a triad of uterus didelphys, unilateral obstructed hemivagina, and ipsilateral renal agenesis. It was previously known as Herlyn?Werner?Wunderlich syndrome. OHVIRA is typically diagnosed after menarche and is often associated with recurrent severe dysmenorrhea and vague lower abdominal pain, although the presentation can be variable. Due to the normal onset of menarche from the non-obstructed side, diagnosis of OHVIRA can easily be missed, which can lead to complications such as infection, pelvic inflammatory disease, and endometriosis. Renal, uterine, and vaginal pathologies can be diagnosed with the use of ultrasonography and magnetic resonance imaging (MRI). Here in, we present a case of OHVIRA syndrome in a 16-year old girl who presented with lower abdominal pain. In cases in which the diagnosis is not delayed, treatment consists of vaginal septostomy. Delayed diagnosis may lead to pelvic infections in patients with complications and subsequent need for hemi-hysterectomy. This case underscores the importance of maintaining a high index of suspicion for OHVIRA syndrome in adolescents with non-specific abdominal or pelvic symptoms.