Kamio Masaki, Nagata Chikako, Sameshima Hirotsugu, Togami Shinichi, Kobayashi Hiroaki
Department of Obstetrics and Gynecology, Faculty of Medicine, Kagoshima University, Kagoshima, Japan.
J Obstet Gynaecol Res. 2018 Jul;44(7):1326-1329. doi: 10.1111/jog.13656.
Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) syndrome is a rare complex of structural abnormalities of the female urogenital tract. A nulliparous 37-year-old woman was referred to our department for an acute abdomen. Multiple uterine myomas and painful right vaginal bulge were found on gynecological examination. Transvaginal ultrasonography revealed a cystic mass and a right-side cervix that was hidden behind the longitudinal vaginal septum. For these findings, pyocolpos arising from OHVIRA syndrome was suspected. Her general condition gradually worsened within an hour, and she developed septic shock and was hospitalized in the intensive care unit (ICU). Laboratory data revealed disseminated intravascular coagulation (DIC) and multiple organ failure. Her general condition gradually improved as a result of intensive care and transvaginal puncture drainage of the abscess. Hysterectomy, performed 2 months after the acute septic episode, confirmed the diagnosis of septate uterus with obstructed hemivagina. To date, there is no documented case of OHVIRA syndrome with an eventual septic shock.
梗阻性半阴道并同侧肾异常（OHVIRA）综合征是一种罕见的女性泌尿生殖道结构异常的复合体。一名37岁未生育的女性因急腹症转诊至我科。妇科检查发现多个子宫肌瘤和右侧阴道疼痛性肿物。经阴道超声检查发现一个囊性肿物以及隐藏在纵向阴道隔后方的右侧宫颈。基于这些发现，怀疑为OHVIRA综合征所致的积脓阴道。她的一般状况在一小时内逐渐恶化，发展为感染性休克并入住重症监护病房（ICU）。实验室检查结果显示弥散性血管内凝血（DIC）和多器官功能衰竭。经过重症监护和经阴道穿刺引流脓肿后，她的一般状况逐渐改善。急性感染发作2个月后进行的子宫切除术确诊为伴有梗阻性半阴道的纵隔子宫。迄今为止，尚无OHVIRA综合征最终发展为感染性休克的文献报道病例。
