Anesthetic management of patients with congenital insensitivity to pain with anhidrosis: a retrospective analysis of 358 procedures performed under general anesthe... Academic Article uri icon


  • BACKGROUND Congenital insensitivity to pain with anhidrosis (CIPA) is a rare autosomal recessive disorder characterized by recurrent episodic fevers, anhidrosis, absent reaction to noxious stimuli, self-mutilating behavior, and mental retardation. The anesthetic management of patients with CIPA is challenging. Autonomic nervous system abnormalities are common, and patients are at increased risk for perioperative complications. METHODS In this study, we describe our experience with 35 patients with CIPA who underwent 358 procedures requiring general anesthesia between 1990 and 2013. RESULTS During surgery, 3 patients developed hyperthermia intraoperatively (> 37.5 C) without prior fever. There were no cases of intraoperative hyperpyrexia (> 40 C). Aspiration was suspected in 2 patients, and in another patient aspiration was prevented by the use of endotracheal tube …

publication date

  • January 1, 2015