OHVIRA with a Wrist: Obstructed Hemivagina Ipsilateral Renal Anomaly with Urogenital Sinus in 2 Patients

Background: OHVIRA syndrome is a rare diagnosis involving the triad of obstructed hemivagina, uterine anomaly, and ipsilateral renal anomaly. OHVIRA syndrome can be associated with other anomalies due to abnormal embryologic development of the urogenital system. Cases: A 14-year-old female with known left renal agenesis, long-standing urinary incontinence, and history of recurrent urinary tract infections presented with abdominal distention. A 4-year-old female with known right renal agenesis and urinary incontinence was found to have a single common channel at the introitus that communicated with the bladder and a hemivagina on the left. Summary and Conclusion: It is important to identify the presence of a urogenital sinus in the OHVIRA setting, as surgical management for these patients may be affected. In both cases, the urogenital sinus was preserved as the vaginal opening.