Department of Anesthesiology and Pain Medicine, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea.
Korean J Anesthesiol. 2012 Aug;63(2):169-72. doi: 10.4097/kjae.2012.63.2.169. Epub 2012 Aug 14.
Myotonic dystrophy is a rare genetic disorder characterized by muscle atrophy and weakness. Surgical treatment of this condition poses various problems for the anesthesiologist. We describe the anesthetic management of a 10-month-old infant with congenital myotonic dystrophy, who was scheduled for endoscopic third ventriculostomy under general anesthesia. Anesthesia was induced with thiopental sodium, fentanyl, and vecuronium, and thereafter maintained via continuous infusion of propofol and remifentanil. The train-of-four ratio was monitored throughout the operation, and muscle relaxation was reversed with pyridostigmine and glycopyrrolate at the end of the procedure. We show that total intravenous anesthesia using propofol and remifentanil is a satisfactory anesthetic technique in very young patients with congenital myotonic dystrophy.
肌强直性营养不良是一种罕见的遗传性疾病，其特征为肌肉萎缩和无力。这种疾病的外科治疗给麻醉师带来了各种问题。我们描述了一例 10 个月大的先天性肌强直性营养不良婴儿在全身麻醉下接受内镜第三脑室造瘘术的麻醉管理。麻醉诱导使用硫喷妥钠、芬太尼和维库溴铵，然后通过持续输注丙泊酚和瑞芬太尼维持麻醉。在整个手术过程中监测四成肌松监测，在手术结束时使用吡啶斯的明和格隆溴铵逆转肌肉松弛。我们表明，在先天性肌强直性营养不良的非常年幼的患者中，使用丙泊酚和瑞芬太尼的全静脉麻醉是一种令人满意的麻醉技术。
