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 openheart 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.

A Systematic Review of Congenital Insensitivity to Pain, a Rare Disease

“…Cautionary words have been issued by Takeuchi et al against the use of remifentanil due to the attendant risk of complications [ 15 ]. Similarly, ketamine emerges as contraindicated, as demonstrated by Jiang et al, who have observed an augmented likelihood of aspiration and regurgitation with its application [ 14 ]. Jiang et al and Qiu et al universally endorse intraoperative precautions, including temperature monitoring and fasting [ 14 , 17 ].…”