Anesthetic management during adenotonsillectomy for twins with congenital insensitivity to pain with anhidrosis: two case reports

Abstract

BackgroundCongenital insensitivity to pain with anhidrosis is a rare autosomal recessive disorder characterized by hyperpyrexia, anhidrosis, pain insensitivity, self-inflicted injuries, and intellectual disability. The anesthetic management of these patients is challenging owing to the high risk of perioperative complications resulting from their autonomic dysfunction, such as hyperthermia, hypotension, and bradycardia, which result from autonomic nervous system dysfunction.Case presentationTwo 3-year-old Han Chinese identical male twins (weighing 13.5 kg and measuring 93 cm tall) were previously diagnosed as having congenital insensitivity to pain with anhidrosis based on clinical features and genetic screening. According to the presence of loud snoring and heavy breathing during sleep and neck radiograph findings, they were diagnosed as having tonsil and adenoid hypertrophy and needed adenotonsillectomy. Because of innate analgesia, some reports suggested that patients with congenital insensitivity to pain with anhidrosis do not require perioperative pain control. Accordingly, our patients did not receive opiates. We describe the general anesthetic management of these patients using sevoflurane and propofol, but without opiates, for adenotonsillectomy. Remarkable tachycardia and hypertension occurred during airway manipulation and when the surgical stimuli increased, and their temperatures increased from 36 °C and 36.8 °C to 37.8 °C and 38.5 °C, respectively. Patients with congenital insensitivity to pain with anhidrosis lack pain sensation, but they may have tactile hyperesthesia. Surgical noxious stimuli may therefore produce a stress response and unpleasant sensations, leading to hemodynamic fluctuation and temperature increase.ConclusionsOn the basis of these findings, we suggest that careful intraoperative opiate titration may be justified to blunt the surgical stress response and promote hemodynamic and temperature stability in similar patients; we also recommend the preparation of warming and cooling devices and continuous temperature monitoring in these patients. Since anesthetic management of these patients is not simple, careful attention is required.

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

“…Some studies found that the Bispectral index (BIS) and Narcotrend are useful tools for monitoring the depth of anesthesia in such patients. 15,20 Despite the necessity to warm our patient to prevent hypothermia, the temperature must be closely monitored to detect and manage hyperthermia. There is no evidence of a correlation between CIPA patients and malignant hyperthermia.…”

“…Therefore, better perioperative body temperature management is necessary. Some studies found that the Bispectral index (BIS) and Narcotrend are useful tools for monitoring the depth of anesthesia in such patients 15,20 . Despite the necessity to warm our patient to prevent hypothermia, the temperature must be closely monitored to detect and manage hyperthermia.…”

“…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 15 . Although epinephrine, norepinephrine, and cortisol levels were not significantly elevated throughout the procedure, 16 it was not ruled out that the patient's stress levels were raised.…”

Hemodynamic Response to Massive Bleeding in a Patient with Congenital Insensitivity to Pain with Anhidrosis

“…Intraoperative bradycardia was also observed in 2.8% of CIPA cases [ 13 ]. In pediatric patients, cardiovascular complications were reported to be common [ 3 ] and to sometimes induce hemodynamic change by an unpleasant sensation [ 3 , 14 ]; therefore, attention should be paid to hemodynamics in patients with CIPA, even if a compensatory response is present.…”

Role of Genetics in Anesthesiology

“…So that in some cases, patients with this condition do not require opiates during surgery because they already have congenital analgesia. 23…”