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

Abstract

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.

Postoperative Adverse Outcomes in Intellectually Disabled Surgical Patients: A Nationwide Population-Based Study

“…Moreover, perioperative antibiotic coverage and aggressive pulmonary hygiene are also crucial to prevent postoperative pulmonary complications in ID patients with high susceptibility, including congenital central alveolar hypoventilation syndrome (Ondine's curse) [38], Langer-Giedion syndrome [39], mucopolysaccharide storage disorders [40] and cri du chat syndrome [41]. ID patients with congenital insensitivity to pain with anhidrosis should be managed as “full stomach” patients to prevent perioperative regurgitation and aspiration, because their gastric emptying is delayed due to autonomic nervous system dysfunction [42].…”

Anesthetic Management of Patients with Congenital Insensitivity to Pain with Anhidrosis

“…13 In this study, we observed only 2 cases of regurgitation with aspiration and 1 case of regurgitation without aspiration, representing about 0.8% of the cases. It has previously been suggested that all CIPA patients should be considered having a “full stomach,” regardless of their nil per os status, because of their risk of aspiration.…”

Anesthetic Management of Congenital Insensitivity to Pain with Anhidrosis in a Child: A Case Report

“…The autonomic nervous system abnormalities are common in patients with CIPA, leading to disturbances in gastric myoelectric activity and therefore gastric emptying delay and dyspeptic symptoms, predisposing CIPA patients to an increased risk of regurgitation and aspiration when they are anesthetized [8,9]. In our case the throat reflex was maintained during TIVA with spontaneous respiration via facemask, avoiding aspiration if the patient regurgitated.…”

“…In our case the throat reflex was maintained during TIVA with spontaneous respiration via facemask, avoiding aspiration if the patient regurgitated. Previous cases report demonstrated that laryngeal mask airway (LMA) is not appropriate for the airway management of CIPA patients, leading regurgitation, aspiration and even hypoxic cardiac arrest [9]. For those patients, surgical procedures can be done with superficial anesthesia but the gag reflex from stimulation of the pharynx with LMA may not be inhibited.…”