Congenital indolent anhidrosis is an autosomal recessive disorder categorized as a hereditary sensory and autonomic neuropathy (HSAN), the symptoms of which include a loss of thermal and pain perception throughout the body, sweating disorders, decreased autonomic function, and mental retardation. We report a patient requiring intensive dental treatment under general anesthesia. The patient was a 20-year-old male (height, 163.8 cm ; weight, 41.7 kg ; BMI, 15.5). General anesthesia was required for the treatment of multiple dental caries and wisdom teeth removal because of coexisting mental retardation. Anesthesia was induced with remifentanil (0.2 μg/kg/min), propofol (4 μg/ml, estimated target concentration), and rocuronium (30 mg). The remifentanil and propofol doses were titrated to within 0.05-0.2 μg/kg/min and 2.0-2.5 μg/ml, respectively, so as to maintain a BIS value of 35-45. The intraoperative body temperature was maintained at 36.6°C-36.9°C using a warming device. The dental treatment required 3 hours to complete, and the patient recovered smoothly after the cessation of the anesthesia. This case required a relatively low dose of remifentanil, as shown by the BIS-guided titration. Previous reports have described the use of minimum doses of narcotics and sedatives during general anesthesia for patients with congenital painless anhidrosis. To avoid awareness and profound circulatory suppression, BIS monitoring was considered to be useful for titrating the anesthesia. In conclusion, our experience performing general anesthesia in a patient with congenital anhidrosis and pain insensitivity suggested that BIS-guided titration of the anesthesia dose was useful.
