Muraoka Ayako, Tsuda Hiroyuki, Kotani Tomomi, Kikkawa Fumitaka
J Reprod Med. 2016 May-Jun;61(5-6):290-4.
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.
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.
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.
据报道，苗勒管异常在2%至3%的女性中出现。双子宫、阴道半段梗阻并同侧肾缺如（OHVIRA）综合征是一种罕见病症，仅有少数该综合征患者在孕期发病的病例被报道。
一名35岁、未孕的女性，孕18周时因宫颈机能不全前来就诊。她的首发症状为生殖器出血。超声检查和磁共振成像确诊为OHVIRA综合征，且患侧妊娠得到确认，羊膜囊自宫颈突出至阴道腔。她随后住院，并接受了宫缩抑制剂以治疗频繁的子宫收缩。孕30周时，她突发剧烈腹痛。因此，我们进行了急诊剖宫产，当时发现子宫表面异常静脉丛破裂导致严重腹腔积血。
合并苗勒管异常的妊娠较为罕见，孕期严重腹腔积血对母亲和胎儿均可能危及生命。因此，临床医生在处理合并OHVIRA综合征的妊娠时应考虑到急性腹腔积血的诊断。
