遗传性出血性毛细血管扩张症产妇剖宫产的麻醉考量：一例病例报告
Anesthetic considerations for cesarean delivery in a parturient with hereditary hemorrhagic telangiectasia: a case report.
作者信息
Kamimura Yuji, Nakanishi Toshiyuki, Boku Sato Aiji, Kako Eisuke, Tanaka Motoshi, Sobue Kazuya
机构信息
Department of Anesthesiology and Intensive Care Medicine, Nagoya City University Graduate School of Medical Sciences, 1 Kawasumi, Mizuho-cho, Mizuho-ku, Nagoya, 467-8601, Japan.
Department of Anesthesiology, Aichi Gakuin University School of Dentistry, 2-11 Suemori-dori, Chikusa-ku, Nagoya, 464-8651, Japan.
出版信息
JA Clin Rep. 2021 Mar 1;7(1):19. doi: 10.1186/s40981-021-00420-4.
BACKGROUND
Hereditary hemorrhagic telangiectasia (HHT), also known as Rendu-Osler-Weber syndrome, is a rare autosomal dominant disorder characterized by mucocutaneous telangiectasia and arteriovenous malformations (AVMs). There are some anesthetic considerations for cesarean delivery in a parturient with HHT.
CASE PRESENTATION
A 27-year-old parturient with pulmonary hemorrhage was admitted to our tertiary perinatal center. She was first diagnosed with HHT and a cesarean delivery using spinal anesthesia at 37 weeks of gestation was initially planned. However, magnetic resonance imaging (MRI) at 32 weeks of gestation revealed spinal AVM ranging from the thoracic to the lumbar levels. Thus, elective cesarean delivery under general anesthesia was planned. The parturient had an uneventful perioperative course.
CONCLUSIONS
HHT should be considered as a differential diagnosis when parturients develop pulmonary hemorrhage. In a cesarean delivery of parturients with HHT, spinal MRI during pregnancy can help in deciding the anesthetic procedure to be used.
背景
遗传性出血性毛细血管扩张症（HHT），也称为伦杜-奥斯勒-韦伯综合征，是一种罕见的常染色体显性疾病，其特征为黏膜皮肤毛细血管扩张和动静脉畸形（AVM）。对于患有HHT的产妇，剖宫产分娩存在一些麻醉方面的考虑因素。
病例介绍
一名27岁的患有肺出血的产妇被收治到我们的三级围产期中心。她最初被诊断为HHT，最初计划在妊娠37周时采用脊髓麻醉进行剖宫产。然而，妊娠32周时的磁共振成像（MRI）显示脊髓AVM范围从胸段到腰段。因此，计划在全身麻醉下进行择期剖宫产。该产妇围手术期过程顺利。
结论
当产妇出现肺出血时，应将HHT视为鉴别诊断之一。在患有HHT的产妇进行剖宫产时，孕期的脊髓MRI有助于确定所使用的麻醉方法。
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