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

Abstract

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.

Vaginoscopic resection of hemivagina, in a 20‐year‐old virgin female with prior misdiagnosis of OHVIRA syndrome as a bicornuate uterus: A case report

“…Therefore, as shown in a Video S1, using a rigid resectoscope is helpful. 5,11 In conclusion, OHVIRA syndrome can be misdiagnosed due to its rarity, resulting in the need for more invasive interventions than vaginoscopy. Unnecessary and invasive interventions can lead to uterine damage and affect patient's fertility and pregnancy outcomes.…”

“…On the other hand, in some populations, cultural issues lead to patient's and their parents' preferences for virginity preservation. Therefore, as shown in a Video S1, using a rigid resectoscope is helpful 5,11 …”