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

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.

• Authors: Jialong Jiang, Xuefeng Wang, Jicheng Hu, Sheng Wang
• Journal: Paediatric anaesthesia (Paediatr Anaesth) Vol. 32 Issue 9 Pg. 1070-1072 (09 2022) ISSN: 1460-9592 [Electronic] France
• PMID: 35762567 (Publication Type: Case Reports)
• Copyright: © 2022 The Authors. Pediatric Anesthesia published by John Wiley & Sons Ltd.

Chemical References

• Anesthetics

Topics

• Anesthetics
• Cardiac Surgical Procedures
• Channelopathies
• Child
• Hereditary Sensory and Autonomic Neuropathies (complications, surgery)
• Humans
• Hypohidrosis (complications)
• Pain
• Pain Insensitivity, Congenital