Department of Neuroanesthesia, National Institute of Mental Health and Neurosciences, Bangalore, 560 029, India.
J Anesth. 2010 Aug;24(4):594-7. doi: 10.1007/s00540-010-0963-9. Epub 2010 Jun 3.
Morquio syndrome is a hereditary mucopolysaccharide disorder presenting with an abnormality of the craniocervical junction from childhood. We describe an adult patient who presented with Morquio syndrome who had subglottic narrowing of the airway, restrictive pulmonary disease, and mild mitral regurgitation and trivial aortic regurgitation. The anesthetic management of this patient for atlantoaxial stabilization is presented.
黏多糖贮积症Ⅳ型是一种遗传性黏多糖代谢障碍疾病,从儿童期开始就表现出头颈交界区的异常。我们描述了一位成年黏多糖贮积症Ⅳ型患者,其存在声门下气道狭窄、限制性肺疾病以及轻度二尖瓣反流和轻微主动脉瓣反流。本文介绍了该患者行寰枢椎稳定术的麻醉管理。