Anesthetic management of a patient with congenital insensitivity to pain with anhidrosis by coadministration of remifentanil

BACKGROUND: Congenital insensitivity to pain with anhidrosis (CIPA) is a rare autosomal recessive disease characterized by unexplained fever, systemic insensitivity to pain, anhidrosis, and mental distress. Anesthetic management is challenging because autonomic dysfunction can induce perioperative complications. Only a few reports of anesthetic management of CIPA patients have been published. We herein present a case of successful management of the same patient on two occasions using small doses of fentanyl and remifentanil. CASE PRESENTATION: A 37-year-old man with CIPA underwent two orthopedic operations. We were able to balance the dose of remifentanil to avoid the extremes of hyperalgesia when the dose is too low and shivering when the dose is too high. CONCLUSION: To our knowledge, no reports have described the anesthetic management of CIPA patients with remifentanil. We consider anesthetic management with coadministration of remifentanil to be potentially useful for such patients.