Lauwers M H, Van Lersberghe C, Camu F
Department of Anaesthesia, Flemish Free University of Brussels Medical Centre, Belgium.
Anaesthesia. 1994 Oct;49(10):876-8. doi: 10.1111/j.1365-2044.1994.tb04263.x.
Kearns-Sayre syndrome is an extremely rare mitochondrial myopathy, characterised by retinitis pigmentosa associated with progressive external ophthalmoplegia. Cardiac conduction abnormalities are common and range from bundle branch block to third degree atrioventricular block. Generalised degeneration of the central nervous system has also been reported. We describe the anaesthetic management of a child afflicted by this syndrome. The major anaesthetic complication in this disease is sudden third degree atrioventricular block which may lead to death in the absence of an artificial cardiac pacemaker.
卡恩斯-塞尔综合征是一种极其罕见的线粒体肌病，其特征为色素性视网膜炎伴进行性眼外肌麻痹。心脏传导异常很常见，范围从束支传导阻滞到三度房室传导阻滞。也有中枢神经系统广泛变性的报道。我们描述了一名患该综合征儿童的麻醉处理。该病主要的麻醉并发症是突然发生的三度房室传导阻滞，若无人工心脏起搏器，可能导致死亡。
