(81) Management of OHVIRA Syndrome Diagnosed in Pregnancy

Background: The syndrome of obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) is typically diagnosed in adolescence after menarche. Patients present with dysmenorrhea and abdominal pain in the setting of a renal anomaly, and imaging demonstrates uterine didelphys with an obstructed hemivagina. Rarely, the diagnosis may be delayed until adulthood. In this case, we present a patient with OHVIRA diagnosed and surgically managed during pregnancy.

Case: This 34-year-old patient presented in her G1 pregnancy for routine prenatal care. On nuchal translucency ultrasound, the patient was found to have uterine didelphys with the pregnancy in the right uterine horn and a fluid collection measuring 3.7x2.5x1.9 cm in the left upper vagina. On examination, only one cervix was visualized along with a mass to the left of the cervix; there was no obvious microperforation. An MRI was obtained, consistent with previous findings and further revealed left renal agenesis. The patient was counseled on the new diagnosis of OHVIRA in pregnancy and recommended to undergo vaginoplasty for septum excision to reduce the risk of pyocolpos. She underwent an uncomplicated vaginoplasty at 19 weeks and 2 days with collaboration of pediatric gynecology and maternal fetal medicine. She was diagnosed with fetal growth restriction at 34 weeks (EFW 4%, AC 6%) and had antenatal surveillance with twice weekly NST and weekly UA dopplers. Follow up growth ultrasound demonstrated EFW 1% and AC < 1% and thus she underwent scheduled Cesarean section at 38 weeks and 1 day for breech presentation; birth weight was 2429g.

Comments: This case highlights a unique presentation of a rare congenital malformation of Mullerian and Wolffian ducts. Given the patient’s initial diagnosis during pregnancy, there were special considerations to the surgical technique and timing. Limited reports of OHVIRA during pregnancy highlight the risks associated with vaginal septum rupture and infection if untreated (Zivkovic,2014); thus, we discussed options for vaginoplasty during the pregnancy versus postpartum with the patient, who opted for intervention in the second trimester. The patient’s pregnancy was also complicated by two known risks in pregnancies with Mullerian anomalies; fetal malpresentation and growth restriction. OHVIRA is typically diagnosed in adolescence, though some delays in diagnosis are common in patients with microperforations (Wozniakowska,2013) or patients treated for dysmenorrhea without imaging (Jha,2020). This case highlights an unusual presentation of initial OHVIRA diagnosis during routine ultrasound in pregnancy, vaginoplasty in the second trimester, and delivery of healthy term infant.