Anesthesia Procedure for Congenital Insensitivity to Pain in a Child with Anhidrosis Syndrome: A Rare Case

Abstract

Congenital insensitivity to pain with anhidrosis (CIPA) syndrome is a neuropathy characterized by insensitivity to pain, impaired thermoregulation, anhidrosis, and mental retardation. A 9-year old boy with CIPA syndrome, underwent 2 operations for a calcaneal ulcer. During the first operation standard monitorization was performed. In the second operation, Bispectral Index (BIS) monitoring was added and temperature was monitored with an esophageal probe. In the first operation, in which anesthesia induction was applied with ketamine and midazolam, extremity movements with surgical stimuli were seen. Despite pain insensitivity, as extremity movements were seen with surgical stimuli, propofol was administered in the second operation. Throughout the operation, the BIS values varied from 19 58 and body temperature was measured as 36.1°C 36.9°C. In conclusion, despite the absence of pain sensitivity in CIPA syndrome cases, there is an absolute need for the administration of anesthesia in surgical procedures because of tactile hyperesthesia. (J Nippon Med Sch 2017; 84: 237 240)

A case report: Anesthetic management for open‐heart surgery in a child with congenital insensitivity to pain with anhidrosis

“…According to current literature, some patients can be operated on while sedated 12 ; however, it is believed that while these patients are insensitive to pain, paradoxically, they may have hyperalgesia. Some CIPA cases have reported perioperative somatotropic reactions caused by increasing surgical stimulation and postoperative pain complaints 13,14 . Therefore, it has been proposed that opioid analgesics should be used in surgical treatment for this patient population.…”

“…Some CIPA cases have reported perioperative somatotropic reactions caused by increasing surgical stimulation and postoperative pain complaints. 13,14 Therefore, it has been proposed that opioid analgesics should be used in surgical treatment for this patient population. In addition, it has been found that heart rate, blood pressure, and body temperature all rise significantly when patients are intubated without opioid analgesics.…”

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

“…The experience of anesthesia management is also limited due to the rare occurrence of the syndrome. 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.…”

Two Case Reports of Hereditary Sensory Autonomic Neuropathy Type IV

“…This gene is responsible for nerve growth factor (NGF) effects, and it helps in the development of the nociceptive, sensory and sympathetic neurons during the embryological period. 2 Symptoms of this disease begins in infancy. Common clinical presentations include anhidrosis, insensitivity to noxious stimuli leading to self-mutilating behavior, and intellectual disability.…”