背景:亚历山大病是一种罕见的疾病,进行性脑白质营养不良,由于临床表现包括发育迟缓,患者容易在全身麻醉下出现并发症,癫痫发作,吞咽困难,呕吐,和睡眠呼吸暂停。然而,对麻醉结果的研究是有限的。
目的:我们的目的是描述患者特征,麻醉技术,以及在四级监护儿童医院接受磁共振成像和/或腰椎穿刺的亚历山大病患者的麻醉相关并发症。
方法:我们对入选前瞻性观察性研究的亚历山大病患者的麻醉结果进行了回顾性分析。纳入的患者诊断为亚历山大病,并在我们机构接受了磁共振成像和/或腰椎穿刺。我们排除了用于其他程序或外部机构的麻醉剂。收集的数据包括患者特征,麻醉技术,药物,以及麻醉下和随后24小时的并发症。我们酌情进行了描述性统计。
结果:40例接受64例手术的患者符合纳入标准。56例(87.5%)需要全身麻醉或监测麻醉护理(MAC),8例(12.5%)不需要。全身麻醉/MAC组往往比非麻醉患者年轻(中位年龄6岁[IQR3.8;9]vs.14.5年[IQR12.8;17.5])。在这两组中,吞咽困难(78.6%vs.87.5%,分别),癫痫发作(62.5%vs.25%),和反复呕吐(17.9%vs.25%)经常报告术前症状。吸入诱导是常见的(N=48;85.7%),两个(3.6%)进行了快速序列诱导。严重的并发症很少见,没有误吸或癫痫发作。8例(14.3%)发生麻黄碱治疗低血压。每位患者(1.8%)出现术后出现躁动或呕吐。53例(94.6%)是门诊手术。没有住院患者需要提高护理敏锐度。
结论:在这项单中心研究中,亚历山大病患者在接受全身麻醉/MAC时没有出现频繁或不可逆的并发症.共病症状在麻醉后没有增加。一些患者可能不需要麻醉来完成短期手术。
BACKGROUND: Alexander disease is a rare, progressive leukodystrophy, which predisposes patients to complications under general anesthesia due to clinical manifestations including developmental delay, seizures, dysphagia, vomiting, and sleep apnea. However, study of anesthetic outcomes is limited.
OBJECTIVE: Our aim was to describe patient characteristics, anesthetic techniques, and anesthesia-related complications for Alexander disease patients undergoing magnetic resonance imaging and/or lumbar puncture at a quaternary-care children\'s hospital.
METHODS: We performed a retrospective review of anesthetic outcomes in patients with Alexander disease enrolled in a prospective observational study. Included patients had diagnosed Alexander disease and underwent magnetic resonance imaging and/or lumbar puncture at our institution. We excluded anesthetics for other procedures or at outside institutions. Collected data included patient characteristics, anesthetic techniques, medications, and complications under anesthesia and in the subsequent 24 h. We performed descriptive statistics as appropriate.
RESULTS: Forty patients undergoing 64 procedures met inclusion criteria. Fifty-six procedures (87.5%) required general anesthesia or monitored anesthesia care (MAC) and eight (12.5%) did not. The general anesthesia/MAC group tended to be younger than nonanesthetized patients (median age 6 years [IQR 3.8; 9] vs. 14.5 years [IQR 12.8; 17.5]). In both groups, dysphagia (78.6% vs. 87.5%, respectively), seizures (62.5% vs. 25%), and recurrent vomiting (17.9% vs. 25%) were frequently reported preprocedure symptoms. Inhalational induction was common (N = 48; 85.7%), and two (3.6%) underwent rapid sequence induction. Serious complications were rare, with no aspiration or seizures. Hypotension resolving with ephedrine occurred in eight cases (14.3%). One patient each (1.8%) experienced postprocedure emergence agitation or vomiting. Fifty-three (94.6%) were ambulatory procedures. No inpatients required escalation in acuity of care.
CONCLUSIONS: In this single-center study, patients with Alexander disease did not experience frequent or irreversible complications while undergoing general anesthesia/MAC. Co-morbid symptoms were not increased postanesthesia. Some patients may not require anesthesia to complete short procedures.