Risk of aspiration during anesthesia in patients with congenital insensitivity to pain with anhidrosis: Case reports and review of the literature

Congenital insensitivity to pain with anhidrosis (CIPA) is a rare autosomal recessive disease, characterized by episodes of unexplained fever, anhidrosis, pain insensitivity despite intact tactile perception, self-mutilating behavior, mental retardation, and autonomic nervous system (ANS) abnormalities. We present a case series of three patients with CIPA who underwent semielective orthopedic surgery under general anesthesia complicated by intraoperative regurgitation, and subsequent aspiration in two of the three cases. All three patients were nil per os (NPO) for at least 8 h prior to surgery. Two patients had their airways maintained with a laryngeal mask airway (LMA), and one patient had an endotracheal tube (ETT). The patients with an LMA suffered aspiration of gastric contents and subsequently developed hypoxic cardiac arrest. Although the patient with an ETT in situ regurgitated intraoperatively, the presence of the ETT prevented aspiration and any further potential complications. We review the perioperative complications typically observed in patients with CIPA and discuss the risks of using an LMA in these patients. We recommend that patients with CIPA always should be considered as having a "full stomach", regardless of the duration of their NPO status, due to their coexisting ANS abnormalities. Therefore, rapid-sequence induction with an ETT should be utilized for the anesthetic management in every patient with CIPA.