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PEDIATRIC ANESTHESIA

Anesthesia for Patients with Congenital Insensitivity to Pain and Anhidrosis: A Questionnaire Study in Japan

Toshiya Tomioka, MD, PhD^*, Yutaka Awaya, MD, PhD, Kenji Nihei, MD, PhD, Hiroshi Sekiyama, MD^*, Shigehito Sawamura, MD, PhD^*, and Kazuo Hanaoka, MD, PhD^*

*Department of Anesthesiology, Faculty of Medicine, The University of Tokyo; Department of Pediatrics, Seibo International Catholic Hospital; and Department of Pediatric Neurology, National Pediatric Hospital, Tokyo, Japan

Address correspondence and reprint requests to Toshiya Tomioka, Department of Anesthesiology, Faculty of Medicine, The University of Tokyo, 3-1 Hongo 7-chome, Bunkyo-ku, Tokyo, 113-8655, Japan. Address e-mail to tomiokat-ane{at}h.u-tokyo.ac.jp

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.

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