Anesthetic Management of Patients with Congenital Insensitivity to Pain with Anhidrosis

Abstract

BACKGROUND Congenital insensitivity to pain with anhidrosis (CIPA) is a rare autosomal recessive disorder characterized by recurrent episodic fevers, anhidrosis, absent reaction to noxious stimuli, self-mutilating behavior, and mental retardation. The anesthetic management of patients with CIPA is challenging. Autonomic nervous system abnormalities are common, and patients are at increased risk for perioperative complications. METHODS In this study, we describe our experience with 35 patients with CIPA who underwent 358 procedures requiring general anesthesia between 1990 and 2013. RESULTS During surgery, 3 patients developed hyperthermia intraoperatively (>37.5°C) without prior fever. There were no cases of intraoperative hyperpyrexia (>40°C). Aspiration was suspected in 2 patients, and in another patient aspiration was prevented by the use of endotracheal tube, early detection of regurgitation, and aggressive suctioning. One patient had cardiac arrest requiring cardiopulmonary resuscitation. Intraoperative bradycardia was observed in 10 cases, and postoperative bradycardia was observed in 11 cases. CONCLUSIONS Regurgitation, hyperthermia, and aspiration were uncommon, but the incidence of bradycardia was higher than has been reported in previous studies. CIPA remains a challenge for anesthesiologists. Because of the rare nature of this disorder, the risk of various complications is difficult to predict.

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

“…Zlotnik et al [14] examined 358 operations performed under general anesthesia in 35 CIPA patients. The ratio of the anesthetic agents used in these operations was; thiopental 4%, ketamine 27%, propofol 71%, opioid 8%, muscle relaxants 27%.…”

“…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.…”

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

“…These physiological responses occur because these cranial nerves are intact in patients with CIPA. A previous study showed that tachycardia within 10 minutes after induction occurred in 31% of CIPA patients and that the patients could respond to airway manipulation [ 13 ]. Intraoperative bradycardia was also observed in 2.8% of CIPA cases [ 13 ].…”

“…A previous study showed that tachycardia within 10 minutes after induction occurred in 31% of CIPA patients and that the patients could respond to airway manipulation [ 13 ]. 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.…”

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

“…Consequently, patients with CIPA are susceptible to numerous anesthetic complications due to autonomic abnormalities, including hemodynamic instability, particularly hypotension, bradycardia, and hyperthermia [ 4 ]. Due to the rarity of CIPA, anesthesia management experience is limited, with previous reports describing cases of orthopedic, maxillofacial, or debridement surgery associated with disease-related trauma [ 5 ]. Here, we describe the anesthetic management of young twins with CIPA who underwent airway surgery (adenotonsillectomy) unrelated to the conditions underlying CIPA, which is normally associated with a stress response.…”