Department of Anesthesiology and Perioperative Medicine, Oregon Health & Science University, 3181 SW Sam Jackson Park Road, Mail Code UHN-2, Portland, OR, 97239, USA.
Division of Maternal Fetal Medicine, Department of Obstetrics and Gynecology, Cedars-Sinai Medical Center, Los Angeles, CA, USA.
J Anesth. 2021 Apr;35(2):288-302. doi: 10.1007/s00540-021-02911-1. Epub 2021 Mar 8.
Female carriers are more common than males with hemophilia and unrecognized factor VIII or IX deficiency is associated with intrauterine growth retardation, epidural hematomas, blood transfusion, and peripartum hemorrhage. A review was conducted to assess the evidence for professional society recommendations for > 50% factor levels during labor. Two searches of Pubmed, CINAHL, Cochrane, and Google Scholar were completed in October 2019. The first for case reports and series described neuraxial techniques in patients with hemophilia-regardless of sex, age, or pregnant status. The second for case reports and series described bleeding outcomes of parturients with hemophilia. Primary outcomes were diagnosis of neuraxial hematoma (first search) and postpartum bleeding complications (second search). Thirteen articles (n = 134) described neuraxial techniques in patients with hemophilia. Neuraxial hematoma with paraplegia occurred in 3/134 patients-all had a factor level of 1%. Nineteen articles (2712 deliveries in 2657 women) described bleeding outcomes. Postpartum hemorrhage occurred in 7.1% (193/2712) of deliveries, of which 60% necessitated blood transfusion. Postpartum bleeding complications were twice as likely (51.0% [25/49] vs. 25.6% [52/203], P < 0.001) with factor activity < 50%. Therefore, factor levels should be assessed and increased above 50% prior to neuraxial technique and delivery.Trial registration: PROSPERO 2018 CRD42018110215.
女性携带者比男性血友病携带者更为常见，未被识别的因子 VIII 或 IX 缺乏与宫内生长迟缓、硬膜外血肿、输血和围产期出血有关。进行了一项综述，以评估专业学会建议在分娩期间因子水平>50%的证据。2019 年 10 月，在 Pubmed、CINAHL、Cochrane 和 Google Scholar 中完成了两次搜索。第一次搜索是针对描述血友病患者（无论性别、年龄或妊娠状态如何）接受神经轴技术的病例报告和系列研究。第二次搜索是针对描述血友病产妇出血结局的病例报告和系列研究。主要结局是诊断神经轴血肿（第一次搜索）和产后出血并发症（第二次搜索）。13 篇文章（n=134）描述了血友病患者的神经轴技术。在 134 例患者中，有 3 例发生神经轴血肿伴截瘫，所有患者的因子水平均为 1%。19 篇文章（2657 名妇女的 2712 次分娩）描述了出血结局。产后出血发生在 2712 次分娩中的 7.1%（193/2712），其中 60%需要输血。产后出血并发症的发生率是因子活性<50%的两倍（51.0%[25/49]比 25.6%[52/203]，P<0.001）。因此，应在进行神经轴技术和分娩前评估并增加因子水平至>50%。试验注册：PROSPERO 2018 CRD42018110215。
