Congenital insensitivity to pain: How should anesthesia be managed?

Abstract

Congenital insensitivity to pain syndrome is a rare, sensorial and autonomic neuropathy characterized by unexplained fever, insensitivity to pain and anhidrosis. Patients may require anesthesia even for minor surgical procedures due to mental retardation and trauma arising from self-mutilating behavior.

A child diagnosed with congenital insensitivity to pain syndrome was scheduled for gastric endoscopy under sedation due to suspected ingestion of a household cleaning disinfectant. Deep sedation was achieved, and spontaneous respiration was maintained. We did not encounter any complications.

There is limited data regarding the safe anesthesia management in these patients because of the rarity of the disease. Therefore, we think that more clinical experience, case reports and studies are needed to establish the appropriate anesthesia management.