Oliveira Carlos Rogério Degrandi, Paris Valter César, Pereira Renato Augusto, Lara Felipe Souza Thyrso de
CET, Santa Casa, Santos.
Rev Bras Anestesiol. 2009 Sep-Oct;59(5):602-9. doi: 10.1016/s0034-7094(09)70085-6.
Congenital insensitivity to pain and Anhidrosis (CIPA) or hereditary sensory and autonomic neuropathy type IV (HSAN IV) is a rare autosomal recessive neuropathy of the group of hereditary sensory and autonomic neuropathies (HSAN) characterized by insensitivity to pain, anhidrosis, and mental retardation. Since it is a rare condition, reports on the anesthetic conduct in patients with CIPA are not easily found in the literature. The objective of this report was to present the anesthetic conduct in a patient with CIPA undergoing left ankle arthrodesis with placement of an implant, and to discuss the characteristics of this disorder that concern anesthesiologists the most.
A female patient with a history of CIPA was admitted for left ankle arthrodesis due to Charcot arthropathy. In the operating room, the patient was monitored with an electrocardiograph, bispectral index, 95% SEF, non-invasive blood pressure, and peripheral hemoglobin saturation; she was pre-medicated with midazolam and underwent intravenous anesthesia with propofol and cisatracurium. The administration of analgesics was not necessary. After tracheal intubation, monitoring of end-expiratory pressure of carbon dioxide and esophageal temperature were added. The patient did not develop postoperative complications. She was discharged from the hospital on the second postoperative day.
Although there is insensitivity to pain, some patients present tactile hyperesthesia that can cause unpleasant feelings during surgical manipulation. Despite reports in the literature of patients undergoing neuroaxis blocks, and even procedures without anesthesia, intravenous anesthesia, which provided adequate conditions for the anesthetic-surgical procedure was used in this case.
先天性无痛觉伴无汗症（CIPA）或遗传性感觉和自主神经病变IV型（HSAN IV）是遗传性感觉和自主神经病变（HSAN）组中的一种罕见常染色体隐性神经病变，其特征为无痛觉、无汗症和智力迟钝。由于该病症罕见，关于CIPA患者麻醉处理的报道在文献中不易找到。本报告的目的是介绍一名接受左踝关节融合术并植入植入物的CIPA患者的麻醉处理情况，并讨论该病症中最让麻醉医生关注的特征。
一名有CIPA病史的女性患者因夏科氏关节病入院接受左踝关节融合术。在手术室中，对患者进行心电图、脑电双频指数、95%频谱边缘频率、无创血压和外周血红蛋白饱和度监测；患者术前使用咪达唑仑进行预处理，然后接受丙泊酚和顺式阿曲库铵静脉麻醉。无需使用镇痛药。气管插管后，增加了二氧化碳呼气末压力和食管温度监测。患者未发生术后并发症。术后第二天出院。
尽管患者无痛觉，但一些患者存在触觉过敏，这可能在手术操作过程中引起不适。尽管文献中有关于患者接受神经轴阻滞甚至无麻醉手术的报道，但本病例使用了静脉麻醉，为麻醉手术过程提供了充分条件。
