Desflurane used in a patient with congenital insensitivity to pain with anhidrosis during septic shock.

Congenital insensitivity to pain with anhidrosis (CIPA) is a rare autonomic recessive disorder characterized by congenital analgesia, absence of sweating and mental retardation. Because of these abnormalities, the anesthetic managements in patients with CIPA deserve special attention. Here we report a 22-year-old man with CIPA receiving left above-knee amputation due to severe lower extremity infection. General anesthesia was maintained with desflurane, an intervention that has never been reported, and the whole course of operation was uneventful. This is also the first reported case of CIPA in Taiwan.