为患有弗里德赖希共济失调的儿科患者矫正脊柱侧弯的麻醉。

Anaesthesia for correction of scoliosis in pediatric patient with Friedreich's ataxia.

作者信息

Agámez Medina G L, Pantin E J, Lorthé J, Therrien P J

机构信息

Servicio de Anestesia y Reanimación, Hospital Universitario Fundación Alcorcón, Alcorcón, Madrid, España.

Department of Anesthesiology, Robert Wood Johnson Medical School, Rutgers University, New Jersey, USA.

出版信息

Rev Esp Anestesiol Reanim. 2015 Jan;62(1):42-5. doi: 10.1016/j.redar.2014.03.001. Epub 2014 Apr 26.

DOI:10.1016/j.redar.2014.03.001

PMID:24775406

Abstract

Friedreich ataxia (FA) is an inherited autosomal recessive disease characterized by a neurological degenerative process of the cerebellum, spinal cord, and peripheral nerves. FA is associated with ataxia, dysarthria, motor and sensory impairment, scoliosis, cardiomyopathy, and diabetes. There is a significant risk of perioperative major complications during the anesthetic management of these patients. We present the case of a fourteen-year-old patient with FA, who had a posterior spinal fusion and instrumentation underwent to total intravenous anesthesia.

摘要

弗里德赖希共济失调（FA）是一种常染色体隐性遗传病，其特征为小脑、脊髓和周围神经出现神经退行性病变。FA与共济失调、构音障碍、运动和感觉障碍、脊柱侧弯、心肌病及糖尿病相关。在对这些患者进行麻醉管理期间，围手术期发生重大并发症的风险很高。我们报告一例14岁FA患者的病例，该患者接受了后路脊柱融合内固定手术，并采用了全静脉麻醉。