先天性无痛觉伴无汗症患儿的麻醉管理
Anesthetic management of children with congenital insensitivity to pain with anhidrosis.
作者信息
Qiu Yongsheng, Zhao Liyuan, Yao Dongdong, Jia Yingping
机构信息
Department of Anesthesiology Children's Hospital Affiliated to Zhengzhou University Henan Children's Hospital Zhengzhou Children's Hospital Zhengzhou Henan China.
Department of Anesthesiology Brigham and Women's Hospital Harvard Medical School Boston USA.
出版信息
Pediatr Investig. 2019 Oct 28;4(4):296-298. doi: 10.1002/ped4.12152. eCollection 2020 Dec.
INTRODUCTION
Congenital analgesia is a rare autosomal recessive hereditary disease. The primary damage of congenital analgesia is central structure damage of comprehensive pain perception.
CASE PRESENTATION
A 1-year-old Han Chinese boy was admitted to hospital because of a tongue bite. He had no response to noxious stimulation of the body surface and was diagnosed with congenital analgesia. A small dose of remifentanil was intravenously injected during anesthetic induction to reduce the stress response caused by endotracheal intubation. A certain depth of anesthesia should be guaranteed during anesthetic induction and surgery to alleviate the stress response induced by endotracheal intubation and the operation.
CONCLUSION
Opioid analgesics are not required for general anesthesia in patient with congenital insensitivity to pain. With a heat dissipation barrier in patients with congenital insensitivity to pain with anhidrosis, body temperature, end-tidal carbon dioxide and bispectral index should be monitored.
引言
先天性无痛觉是一种罕见的常染色体隐性遗传病。先天性无痛觉的主要损害是全面疼痛感知的中枢结构损伤。
病例介绍
一名1岁汉族男孩因咬伤舌头入院。他对体表的有害刺激无反应，被诊断为先天性无痛觉。麻醉诱导期间静脉注射小剂量瑞芬太尼以减轻气管插管引起的应激反应。麻醉诱导和手术期间应保证一定的麻醉深度，以减轻气管插管和手术引起的应激反应。
结论
对疼痛不敏感的患者全身麻醉不需要使用阿片类镇痛药。对于伴有无汗的疼痛不敏感患者，应监测体温、呼气末二氧化碳和脑电双频指数。
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