P1.053: One Kidney, Two Problems: An Underrecognized and Curable Cause of Chronic Pelvic Pain in Adolescent Females

Introduction: Obstructed Hemivagina and Ipsilateral Renal Anomaly (OHVIRA) syndrome is an underrecognized congenital malformation of the female urogenital tract. The classic anatomy is characterized by uterine didelphys with a longitudinal vaginal septum obstructing the excretion of menstrual blood from one side and ipsilateral renal agenesis. These patients require operative intervention for symptomatic uterine obstruction and are prone to delays in diagnosis.

Case Description: We present twelve patients with OHVIRA variants who were treated at our institution between 2018-2024. Patients were diagnosed between ages eleven and twenty-four (mean age 14.3, SD 3.3), usually one to three years after menarche (mean age 11.8, SD 1.6), except in the case of the twenty-four-year-old who had menarche sixteen years prior. Six patients had a known solitary kidney at the time of diagnosis; but had not been evaluated for a Mullerian anomaly until they developed abdominal pain and progressive dysmenorrhea. Those without a known solitary kidney similarly presented with chronic cyclic pelvic pain, and a renal anomaly was identified incidentally on imaging for the pain. Each patient was evaluated with a pelvic ultrasound, pelvic MRI, and/or CT, identifying hematocolpos and Mullerian anomalies. Individual patient characteristics are outlined in Table 1. All patients underwent surgical intervention beginning with a vaginal exam under anesthesia (EUA) followed by an excision of the vaginal septum with efflux of old menses (n=7), a septostomy with an interval procedure for completion excision (n=2), or laparoscopic excision of obstructed non-communicating rudimentary uterine horns (n=2). Those who had a septostomy initially had significant hematocolpos, and therefore difficult visualization of anatomy. Subsequent procedures were planned so as not to damage the cervices. In one case, the septum was not visualized at the time of EUA. Since her presenting symptoms improved, she is followed annually with imaging to ensure no reaccumulating of blood and continued resolution of pelvic pain. At initial follow-up, all patients had improvement of pelvic symptoms. Long term follow-up was heterogeneous and only one patient is followed regularly by an adult gynecologist for management of endometriosis.

Discussion: Despite the association of renal agenesis with an obstructed reproductive tract, patients with OHVIRA may have a delay in diagnosis due to seemingly normal menstruation from the non-obstructed uterine horn. For patients with renal agenesis, anticipatory guidance and prescreening after thelarche should be considered to avoid misdiagnosis and chronic pain. The case of our twenty-four-year-old patient underscores this importance.

Conclusion: Pediatric providers should maintain a high suspicion for OHVIRA as operative intervention is necessary and curative of symptoms. Management is multidisciplinary and should involve pediatric surgery, urology, and gynecology. In our experience, surgical intervention improves quality of life and may allow anticipation of possible reproductive challenges.