The First Affiliated Hospital of USTC, Division of Life Sciences and Medicine, University of Science and Technology of China, Hefei, Anhui, 230036, China.
Paediatr Anaesth. 2022 Sep;32(9):1070-1072. doi: 10.1111/pan.14515. Epub 2022 Jul 7.
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.
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。