Vaginoscopic Incision of Vaginal Septum With Preservation of the Hymen in a Child With Obstructed Hemi-Vagina Ipsilateral Renal Agenesis (OHVIRA) Syndrome

Obstructed hemi-vagina ipsilateral renal agenesis (OHVIRA) syndrome, also known as Herlyn-Werner-Wunderlich syndrome (HWWS) is a rare variant of Mullerian duct anomalies that usually presents after menarche. Although there is increasing awareness about OHVIRA syndrome, high suspicion is needed for the diagnosis. Awareness of the syndrome is crucial for the management and to prevent serious complications. Surgical techniques and age at the surgery are still debatable but, minimally invasive vaginoscopic resection of the vaginal septum should be considered when feasible as it not only allows division of septum with preservation of hymen, but it provides excellent visualization, is less traumatic, and has promising postoperative outcomes. Hymenal integrity is of great concern in specific populations with cultural values. In this report, we present the case of a 24-month-old girl diagnosed with OHVIRA syndrome during a routine follow-up for renal agenesis and was managed with vaginoscopic incision of the vaginal septum using a pediatric cystoscope while maintaining the hymenal integrity.