Anesthetic management of a child with congenital insensitivity to pain with anhidrosis: A case report

Abstract

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Congenital insensitivity to pain with anhidrosis (CIPA) is a rare, autosomal recessive disease classified as hereditary sensory and autonomic neuropathy type VI. Patients with CIPA are characterized by insensitivity to pain, episodes of unexplained fever, anhidrosis, self-mutilating behavior, intellectual disability, and autonomic nervous system abnormalities. The clinical features may intrinsically pose anesthetic challenges. We present a case of a patient with CIPA who underwent tumor biopsy under general anesthesia using a Supreme laryngeal mask airway without any complications. The anesthetic management of this condition is discussed.

Keywords

• congenital insensitivity to pain with anhidrosis
• autonomic neuropathy type VI
• anesthesia
• pediatrics
• laryngeal mask airway