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病例报告:一例先天性无痛无汗症患儿行心脏直视手术的麻醉管理。

A case report: Anesthetic management for open-heart surgery in a child with congenital insensitivity to pain with anhidrosis.

机构信息

The First Affiliated Hospital of USTC, Division of Life Sciences and Medicine, University of Science and Technology of China, Hefei, Anhui, 230036, China.

出版信息

Paediatr Anaesth. 2022 Sep;32(9):1070-1072. doi: 10.1111/pan.14515. Epub 2022 Jul 7.

Abstract

Congenital insensitivity to pain with anhidrosis (CIPA) is a rare disease also known as hereditary sensory and autonomic neuropathy. CIPA is characterized by a lack of pain sensitivity and impaired development of sweat glands. Surgery is required for patients with self-mutilation and skeletal developmental disorders. Due to the disease's rarity and intricacy, anesthesia poses its challenges. Although there have been a few cases of CIPA patients receiving surgery and anesthesia, the number is very limited. Here, we report a case of a child with CIPA who underwent open-heart surgery and discuss the anesthetic considerations. We conclude that patients with CIPA undergoing open-heart surgery require some opioids, that muscle relaxants and volatile anesthetics should be used with extreme caution, and that airway management and temperature control require special attention.

摘要

先天性无痛无汗症(CIPA)是一种罕见的疾病,也称为遗传性感觉和自主神经病。CIPA 的特征是缺乏疼痛敏感性和汗腺发育不良。对于有自残和骨骼发育障碍的患者,需要进行手术。由于疾病的罕见性和复杂性,麻醉存在挑战。尽管有少数 CIPA 患者接受手术和麻醉的案例,但数量非常有限。在这里,我们报告了一例 CIPA 患儿接受心脏直视手术的病例,并讨论了麻醉注意事项。我们的结论是,接受心脏直视手术的 CIPA 患者需要使用一些阿片类药物,肌松药和挥发性麻醉剂应谨慎使用,气道管理和体温控制需要特别注意。

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Spinal anesthesia in a patient with congenital insensitivity to pain with anhidrosis.先天性无痛觉伴无汗症患者的脊髓麻醉。
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