(10) Abortion Considerations for Patients with OHVIRA Syndrome

Background: Obstructed hemivagina with ipsilateral renal agenesis (OHVIRA) is a rare congenital Müllerian anomaly presenting in female adolescents with progressive pelvic pain, dysmenorrhea, and/or vaginal bulge due to the development of hematocolpos in the obstructed hemivagina. This case describes a unique presentation of OHVIRA in a pregnant adolescent with spontaneous vaginal septum perforation. It emphasizes important considerations for pregnancy and abortion care in those with underlying Müllerian anomalies.

Case: A 17-year-old G1P0010 healthy female presented to the pediatric and adolescent gynecology clinic for prolonged vaginal bleeding. Two months prior, she had a missed abortion at 13 weeks’ gestation and presented to an outside hospital where she was noted to have retained products of conception protruding from a lateral defect in the vaginal wall. This was an unexpected finding. She underwent a suction dilation and curettage from site of the spontaneous vaginal septum perforation. She was referred to pediatric and adolescent gynecology due to concerns for atypical anatomy. She was unable to tolerate an in-office pelvic exam. Pelvic ultrasound noted fundal indentation with the septum extending to the cervix. Pelvic MRI noted an absent left kidney, two separate uterine horns diverging at the level of the fundus with two cervices consistent with OHVIRA. No hematocolpos was noted. Renal ultrasound confirmed solitary right kidney.

She underwent vaginoscopy under anesthesia which demonstrated an obstructed left vaginal septum with a 1.2 cm perforation. Excision of vaginal septum was performed. Following resection of the septum, 2 cervices was noted consistent with a didelphys uterus. The patient made an uncomplicated recovery.

Comments: Patients with reproductive tract anomalies who undergo procedural termination of pregnancy are at increased risk of complications, including uterine perforation, adhesion formation, and repeat attempts at termination. There are few reports on procedural abortions in patients with uterine anomalies and even fewer for patients with OHVIRA. In pregnant individuals with OHVIRA, a missed abortion can present similarly, and microperforation of the obstructed vaginal septum may result in retained products of conception, necessitating further intervention. There may be utility to consider medical means first to induce abortion in patients with suspected Müllerian anomalies. If surgical evacuation is considered, a good understanding of possible Mullerian anomalies and ultrasound guidance may increase success of the procedure and decrease incidence of retained products of conception and uterine perforation in those with a uterine anomaly.