Anesthesia for patients with congenital insensitivity to pain and anhidrosis: a questionnaire study in Japan.

Abstract

UNLABELLED:

We investigated the anesthetic management of patients with congenital insensitivity to pain and anhidrosis (CIPA) in Japan. CIPA is a rare inherited disease characterized by a lack of pain sensation and thermoregulation. Although lacking pain sensation, some patients do have tactile hyperesthesia. Thus, anesthetics are a necessity during operations. We also determined that because patients with CIPA have problems with thermoregulation, temperature management is a concern during the perioperative period and sufficient sedation is necessary to avoid accidental fractures. Additionally, it was found that the use of muscle relaxants does not present a problem, malignant hyperthermia is not associated with CIPA, and that the possibility of abnormalities in the autonomic nervous system must be taken into consideration. Therefore, patients with CIPA can be safely managed with anesthesia.

IMPLICATIONS:

We investigated the anesthetic management of patients with congenital insensitivity to pain and anhidrosis. We clarified the following three important points: anesthesia is necessary, temperature management must be maintained, and there must be sufficient perioperative sedation in the anesthetic management of patients with congenital insensitivity to pain and anhidrosis.

• Authors: Toshiya Tomioka, Yutaka Awaya, Kenji Nihei, Hiroshi Sekiyama, Shigehito Sawamura, Kazuo Hanaoka
• Journal: Anesthesia and analgesia (Anesth Analg) Vol. 94 Issue 2 Pg. 271-4, table of contents (Feb 2002) ISSN: 0003-2999 [Print] United States
• PMID: 11812683 (Publication Type: Journal Article)

Chemical References

• Anesthetics

Topics

• Adolescent
• Adult
• Anesthesia, General (methods)
• Anesthetics
• Body Temperature Regulation
• Child
• Hereditary Sensory and Autonomic Neuropathies (physiopathology)
• Humans
• Intubation, Intratracheal
• Preanesthetic Medication
• Sensory Thresholds
• Surveys and Questionnaires