Severe Hemoperitoneum During Pregnancy with Obstructed Hemivagina and Ipsilateral Renal Anomaly Syndrome: A Case Report.

BACKGROUND: Müllerian tract anomalies have been reported in 2-3% of females. Uterus didelphys, obstructed hemivagina, and ipsilateral renal agenesis (OHVIRA) syndrome is a rare condition, with only a few cases of the syndrome occurring during pregnancy having been reported.

CASE: A 35-year-old, nulli-gravid woman at 18 weeks of gestation was referred due to cervical incompetence. Her first symptom was genital bleeding. Ultrasonography and MRI led to the diagnosis of OHVIRA syndrome, and pregnancy was confirmed on the affected side with the amniotic sac found to be protruding from the cervix into the vaginal cavity. She was subsequently hospitalized and received a tocolytic agent to treat frequent uterine contractions. At 30 weeks of gestation she experienced abrupt and acute abdominal pain. We therefore performed emergent cesarean section, at which time severe hemoperitoneum due to the rupture of an anomalous venous plexus on the surface of the uterus was noted.

CONCLUSION: Pregnancies with Müllerian tract anomalies are rare, and severe hemoperitoneum during pregnancy can be life-threatening for both the mother and fetus. Therefore, clinicians should keep a diagnosis of acute hemoperitoneum in mind in the management of pregnancies complicated by OHVIRA syndrome.