Kim Seong Hyop, Baek Seung Woo, Kwon Won Kyoung, Kim Duk Kyung, Yoon Tae Gyoon, Lim Jeong Ae, Woo Nam Sik, Kim Tae Yop
Department of Anesthesiology and Pain Medicine, Konkuk University School of Medicine, Seoul, Korea.
Korean J Anesthesiol. 2009 Sep;57(3):371-375. doi: 10.4097/kjae.2009.57.3.371.
Loeys-Dietz Syndrome (LDS) is a recently described autosomal dominant aortic aneurysm syndrome with widespread systemic involvement. It is characterized by the triad of 1) arterial tortuosity and aneurysms, 2) hypertelorism, and 3) bifid uvula or cleft palate. A 12-year-old boy with LDS was scheduled to undergo correction of aortic valve regurgitation due to aortic annuloectasia. We report our clinical experiences of a case of LDS patient with brief review of related literatures and relevant anesthetic problems.
洛伊斯-迪茨综合征（LDS）是一种最近被描述的常染色体显性遗传的主动脉瘤综合征，伴有广泛的全身受累。其特征为三联征：1）动脉迂曲和动脉瘤；2）眼距过宽；3）悬雍垂裂或腭裂。一名患有LDS的12岁男孩因主动脉瓣环扩张计划接受主动脉瓣反流矫正术。我们报告了一例LDS患者的临床经验，并简要回顾了相关文献及相关麻醉问题。
