Kapoor Ravish, Mann David G, Mossad Emad B
From the *University of Texas MD Anderson Cancer Center, Houston, Texas; and †Texas Children's Hospital, Houston, Texas.
A A Case Rep. 2017 Sep 15;9(6):182-185. doi: 10.1213/XAA.0000000000000561.
Loeys-Dietz syndrome (LDS) is a rare connective tissue disorder predisposing to aortic and arterial aneurysms. Presentations are classified into subtypes based on gene mutations. Pregnancy in patients with LDS is considered very high risk due to the potential for aortic dissection and uterine rupture. We report successful management of an elective cesarean delivery in a 16-year-old patient with LDS type IV using epidural anesthesia. Perioperative considerations and multidisciplinary management specific to taking care of parturients with LDS are discussed.
洛伊茨-迪茨综合征（LDS）是一种罕见的结缔组织疾病，易引发主动脉和动脉动脉瘤。根据基因突变将临床表现分为不同亚型。由于存在主动脉夹层和子宫破裂的风险，LDS患者怀孕被认为风险极高。我们报告了一例采用硬膜外麻醉成功为一名16岁IV型LDS患者实施择期剖宫产的病例。并讨论了围手术期的注意事项以及照顾LDS产妇的多学科管理方法。
