Anesthesia Management of a Patient With Familial Cold Autoinflammatory Syndrome: A Case Report

Cryopyrin-associated periodic syndrome (CAPS) is a rare autosomal dominant inherited disorder comprised of 3 phenotypes and characterized by repetitive systemic inflammation. Familial cold autoinflammatory syndrome (FCAS) is 1 phenotype of CAPS with symptoms that include fever, fatigue, rashes, headaches, and arthralgia.¹ FCAS/CAPS symptoms are triggered by cold stimulation and continue for a few hours or several days. In the case presented below, we prewarmed the patient before anesthesia induction and monitored her core and peripheral temperatures at 3 different locations to avoid perioperative redistribution hypothermia and triggering of a FCAS/CAPS episode.

CASE REPORT

A 13-year-old female patient (height 162 cm; weight 42 kg; body mass index 16 kg/m²) with FCAS/CAPS was scheduled to undergo the extraction of an impacted tooth (mandibular right second premolar) under general anesthesia. She had been treated with canakinumab (interleukin [IL]-1 inhibitor) every 2 months during the winter, and her symptoms were well controlled with no other systemic disease or comorbidities. Because a reduction in body temperature was a concern, we planned to closely monitor the patient's temperature intraoperatively via the axilla, forehead, and rectum.

The patient was prewarmed using a forced-air warming system for 10 minutes prior to induction of general anesthesia, and the warming system was used continuously until the end of surgery. Rapid intravenous induction was accomplished using a continuous infusion of remifentanil 0.5 μg/kg/min and boluses of propofol 80 mg and rocuronium bromide 30 mg. Nasal intubation with a 6.0-cuffed endotracheal tube through the left naris was performed using a McGrath MAC (Medtronic) #3 video laryngoscope. General anesthesia was maintained with sevoflurane 1.5%, oxygen, and air, along with a continuous infusion of remifentanil 0.2–0.3 μg/kg/min. A total of 3.6 mL of 2% lidocaine with 1:100,000 epinephrine was administered for local anesthesia.

The patient's rectal temperature remained stable. Her forehead temperature did not decrease, but rather a gradual increase was noted after anesthesia induction. Her axillary temperature decreased by about 0.8° C during anesthesia induction, quickly recovering 10 minutes prior to the start of surgery (Figure). The time under anesthesia was 1 hour 17 minutes, and removal of the tooth took 37 minutes. The patient was extubated without any complications following successful completion of the surgical procedure and discharged from the hospital on the following day without any symptoms of FCAS/CAPS.

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DISCUSSION

CAPS is a very rare genetic disorder characterized by episodes of repetitive systemic inflammation that are triggered by cold and can persist for several hours or days. Cryopyrin associates with caspase-1 and an adaptor protein called apoptosis-associated speck-like protein containing a caspase recruitment domain and forms a large protein complex called the inflammasome. The inflammasome converts biologically inactive prointerleukin (proIL)-1β to active IL-1β, a potent proinflammatory cytokine, thereby causing an inflammatory response.²

The prewarming of the surgical table, the linen covers, and the patient is useful for preventing perioperative hypothermia primarily attributed to heat redistribution following general anesthesia induction.³ Redistribution hypothermia may disappear during prolonged surgeries of more than 1 to 3 hours as heat loss from the patient's core plateaus after 3 to 5 hours.⁴ Lee et al⁵ showed that prewarming using a forced-air warming system for 10 minutes before induction significantly decreased intraoperative hypothermia. In cases with a relatively short surgical time less than 1 hour, such as the present case, sufficient prewarming of the patient before anesthesia induction should be considered.

We planned to measure the patient's temperature with 3 sites to monitor for the onset of FCAS/CAPS, which can be induced by increasing differences between core and peripheral temperatures. Forehead temperature generally correlates well the temperature of the distal esophagus.⁶ In the present case, neither the forehead temperature nor the rectal temperature, which reflects the patient's central or core temperature, decreased during anesthesia. The axillary temperature, which primarily reflects the patient's peripheral temperature, temporarily decreased during induction. However, it increased within 30 minutes and was consistent with the other 2 temperature trends. Therefore, prewarming prior to induction substantially prevents the decrease in core temperature caused by redistribution (heat loss from central to periphery) and may also lessen intraoperative heat loss by increasing peripheral tissue temperature to resemble core temperature,⁷ helping to prevent redistribution hypothermia during short operations.

This report was originally published in the Journal of the Japanese Dental Society of Anesthesiology. 2021;49:111–113.