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.

Anaesthetic Management of Two Siblings with Congenital Pain Insensitivity Syndrome with Anhydrosis

“…Özmete and colleagues administered propofol for depth sedation after premedication with midazolam, atropine, and ketamine for endoscopic intervention in CIPA syndrome patient. [13] Only midazolam administration was enough in our cases.…”

“…Although general anesthesia was preferred in most of the studies, [12] there were case presentations which only sedation was performed. [13] Even though general anesthesia provides surgical comfort for surgeon, it increases the susceptibility to anesthesia complications due to hemodynamic instability and hyperthermia because of autonomic dysfunction. Therefore, we decided to performed sedation first.…”

“…In some studies, bispectral index (BIS) monitoring was used to evaluate the depth of anesthesia. [13][14] We could not monitor the BIS because the conditions in our clinic were not appropriate.…”

Genetic pain loss disorders

“…During induction, there should be a focus on minimising the risk of regurgitation and aspiration, as gastrointestinal dysautonomia, if present, can lead to delayed gastric emptying, and rapid sequence induction has been proposed as the safest induction method 265 . Intraoperative autonomic instability, including hyperpyrexia (life-threatening rise in the body temperature), hypotension and bradycardia (a resting heart rate slower than 60 bpm), is a rare but life-threatening complication in some patients; close temperature monitoring should be used with active cooling or heating if required, and patients prehydrated 266,267 . However, in those with familial dysautonomia, peri-operative complications are common and careful planning and monitoring are required 268 .…”