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一名雷特综合征患儿在无阿片类药物平衡麻醉下进行牙科手术,该患儿每次尝试喂食时都会抽搐:病例报告。

Dental procedure under opioid-free balanced anaesthesia in a child with Rett syndrome who convulsed on every attempt to feed: Case report.

作者信息

Ponde Vrushali, Desai Ankit, Ekambaram Karthic, Thakur Selabh

机构信息

Children's Anesthesia Services and Department of Anesthesiology, Surya Children Hospital, Mumbai, Maharashtra, India.

Department of Pediatric and Preventive Dentistry, Surya Children Hospital, Mumbai, Maharashtra, India.

出版信息

Indian J Anaesth. 2020 Apr;64(4):325-327. doi: 10.4103/ija.IJA_812_19. Epub 2020 Mar 28.

Abstract

Rett syndrome is a genetic neurodevelopmental disorder which occurs in females and includes autism, spasticity, hypotonia, microcephaly, scoliosis, stereotyping, abnormal respiratory control and seizures. They are at an increased predisposition for QT interval prolongation and cardiac arrhythmias. An 8-year-old severely intellectually impaired girl with Rett syndrome was referred to us for anaesthesia for multiple dental abscess drainage and rehabilitation. Her frequency of convulsions had increased and she convulsed every time an attempt was made to feed her. The pain of chewing exacerbated the convulsions. The cornerstone of our management was to provide adequate pain relief, anaesthesia without muscle relaxant and opioids, and judicious use of local anaesthetics. We discuss the anaesthetic management and its advantages and limitations in this case report.

摘要

雷特综合征是一种发生于女性的遗传性神经发育障碍,其症状包括自闭症、痉挛、肌张力减退、小头畸形、脊柱侧弯、刻板动作、呼吸控制异常和癫痫发作。她们发生QT间期延长和心律失常的易感性增加。一名患有雷特综合征的8岁重度智力障碍女孩因多处牙脓肿引流及康复治疗被转诊至我院接受麻醉。她的抽搐频率增加,每次试图喂她时都会抽搐。咀嚼疼痛加剧了抽搐。我们治疗的关键是提供充分的疼痛缓解、不使用肌肉松弛剂和阿片类药物的麻醉以及谨慎使用局部麻醉剂。我们在本病例报告中讨论麻醉管理及其优缺点。

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本文引用的文献

1
Medical Issues in Adults with Rett Syndrome - A National Survey.雷特综合征成年患者的医学问题——一项全国性调查
Dev Neurorehabil. 2020 Feb;23(2):106-112. doi: 10.1080/17518423.2019.1646341. Epub 2019 Jul 25.
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Rett Syndrome: A Genetic Update and Clinical Review Focusing on Comorbidities.雷特综合征:遗传更新与临床综述,关注共病。
ACS Chem Neurosci. 2018 Feb 21;9(2):167-176. doi: 10.1021/acschemneuro.7b00346. Epub 2017 Dec 15.
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9
Spinal cord monitoring for scoliosis surgery in Rett syndrome: can these patients be accurately monitored?
J Pediatr Orthop. 2008 Apr-May;28(3):342-6. doi: 10.1097/BPO.0b013e318168d194.

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