Ajayan Neeraja, Hrishi Ajay Prasad, Kumar Ashutosh, Sethuraman Manikandan
From the Department of Neurocritical Care, Cambridge University Hospitals, Cambridge, United Kingdom.
Division of Neuroanesthesia, Department of Anaesthesiology, Sree Chitra Tirunal Institute for Medical Sciences, Thiruvananthapuram, India.
A A Pract. 2022 Jun 7;16(6):e01593. doi: 10.1213/XAA.0000000000001593. eCollection 2022 Jun 1.
Arterial tortuosity syndrome is a rare hereditary autosomal recessive connective tissue disorder characterized by elongation and tortuosity of the large- and medium-sized arteries. We present the case of a 13-year old child with arterial tortuosity syndrome who underwent occipital encephaloduroarteriomyosynangiosis for posterior circulation insufficiency. The constellation of clinical features in our patient portended significant anesthetic concerns, including difficult airway due to craniofacial abnormalities, risk of stroke, and myocardial infarction due to intracerebral and coronary arterial tortuosity and stenosis. The pertinent anesthetic implications are summarized, and we describe the anesthetic technique and use of multimodal neuromonitoring relevant for the case.
动脉迂曲综合征是一种罕见的常染色体隐性遗传性结缔组织疾病,其特征为大中型动脉伸长和迂曲。我们报告一例13岁动脉迂曲综合征患儿,因后循环供血不足接受枕部脑硬脑膜动脉肌层血管吻合术。我们患者的一系列临床特征预示着重大的麻醉问题,包括因颅面异常导致的气道困难、因脑内和冠状动脉迂曲及狭窄而引发中风和心肌梗死的风险。总结了相关的麻醉影响,并描述了针对该病例的麻醉技术和多模式神经监测的应用。