Division of Pediatric Anesthesia and Pain Medicine, Arkansas Children's Hospital/University of Arkansas for Medical Sciences, Little Rock, AR 72202, USA.
J Neurosurg Anesthesiol. 2011 Apr;23(2):100-9. doi: 10.1097/ANA.0b013e3181f84fac.
Moyamoya disease is a rare chronic cerebrovascular disease seen both in children and adults. It has a progressive course, but may have a variable clinical presentation. The disease causes ischemic stroke, intracranial hemorrhage, headache, seizures, and transient ischemia attack in children and in adults. Although the pathogenesis of the disease remains unknown, research suggests a genetic predisposition. There are also undefined systemic processes involved in this vasculopathy. Better noninvasive diagnostic techniques for diagnosis of the Moyamoya disease have been developed, but medical treatment can still be challenging. However, various surgical revascularization procedures have shown to provide symptomatic benefit in a majority of these patients. In addition, the anesthetic management of these patients has evolved over the years with an increased understanding of the disease. These have specifically resulted from the identification of risk factors for perioperative complications and outcomes related to the use of anesthetic agents. Finally, research in the last 3 decades has led to the recognition of the importance of pain control, the increased use of regional anesthesia, and better monitoring techniques in providing high quality and safe patient care to patients with Moyamoya disease. This article will provide a comprehensive review of the disease and its anesthetic management.
烟雾病是一种罕见的慢性脑血管疾病，可发生于儿童和成人。它具有进行性病程，但临床表现可能多种多样。该疾病可导致儿童和成人发生缺血性卒、颅内出血、头痛、癫痫发作和短暂性脑缺血发作。尽管该疾病的发病机制尚不清楚，但研究表明存在遗传易感性。该血管病变还涉及未明的系统性过程。目前已经开发出用于烟雾病诊断的更好的非侵入性诊断技术，但药物治疗仍然具有挑战性。然而，大多数此类患者中，各种血管重建手术已显示出可提供症状改善。此外，随着对该疾病认识的提高，这些患者的麻醉管理也在多年来不断发展。具体而言，这是由于确定了与麻醉药物使用相关的围手术期并发症和结局的危险因素。最后，过去 30 年来的研究认识到控制疼痛、增加使用区域麻醉和更好的监测技术在为烟雾病患者提供高质量和安全的患者护理方面的重要性。本文将全面回顾该疾病及其麻醉管理。
