Background: Congenital insensitivity to pain and anhidrosis (CIPA) is a rare autosomal disease characterized by episodes of unexplained fever, systemic analgesia, anhidrosis, and mental distress. Only a few reports of the anesthetic management of patients with CIPA have been published.1
Case Report: A 37-year-old man (153 cm, 69 kg) with CIPA underwent posterior spinal fusion for thoracic spondylotic myelopathy. Anesthetic induction involved intravenous propofol (3 mcg/ml), fentanyl (100 mcg), and rocuronium (70 mg), and anesthetic maintenance was achieved by propofol at a bispectral index of 40 to 60. After intubation and at the time of skin incision, the patient’s blood pressure and heart rate increased. We administered 50 mcg of fentanyl and maintained anesthesia with propofol (2.0–2.5 mcg/ml) and remifentanil (0.02 mcg/kg/min). The blood pressure and heart rate then remained stable during surgery. We also continuously monitored the rectal temperature and maintained it at 36°C to 37°C using an air conditioner and warming device. After extubation, the patient reported no sore throat, wound pain, or shivering, and his postoperative course was uneventful.
Discussion: The most important points of management of patients with CIPA are selection of the most appropriate analgesic drug and maintenance of body temperature. We used fentanyl and remifentanil as the analgesic drugs in the present case. To our knowledge, no reports have described the anesthetic management of patients with CIPA using remifentanil. Because of their insensitivity to pain, anesthesia can be maintained without analgesia in patients with CIPA. However, despite the lack of pain sensation, patients with CIPA still have pressure sensation, and some require fentanyl for airway manipulation.2 In our patient, the blood pressure and heart rate increased at the time of skin incision, and fentanyl was therefore administered. We were otherwise able to maintain anesthesia with a low dose of remifentanil to prevent reaction to surgical stimuli without hyperalgesia3 or shivering.
References:
1. Pediatric international 57: 30-36, 2015.
2. Anesth Analg 121: 1316-1320, 2015.
3. Anaesthesia 71: 1347-62
Learning points: We herein report the safe anesthetic management of a patient with congenital insensitivity to pain and anhidrosis (CIPA) using remifentanil without hyperalgesia or shivering. Remifentanil, a short-acting opioid, might be useful for the management of patients with CIPA.

04 / Anesthetic management of a patient with congenital insensitivity to pain and anhidrosis using remifentanil