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Korean J Anesthesiol. 2009 Sep;57(3):371-375. Korean. Case Report.
Kim SH , Baek SW , Kwon WK , Kim DK , Yoon TG , Lim JA , Woo NS , Kim TY .
Department of Anesthesiology and Pain Medicine, Konkuk University School of Medicine, Seoul, Korea. pondkim@unitel.co.kr
Abstract

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.

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