Kao Sheng-Chin, Ting Chien-Kun, Cheng Kai-Wen, Lin Su-Man, Tsou Mei-Yung, Chan Kwok-Hon, Tsai Shen-Kou
Department of Anesthesiology, Taipei Veterans General Hospital, Taipei, Taiwan, ROC.
J Chin Med Assoc. 2004 Jun;67(6):305-7.
Congenital insensitivity to pain with anhidrosis (CIPA) is a rare autonomic recessive disorder characterized by congenital analgesia, absence of sweating and mental retardation. Because of these abnormalities, the anesthetic managements in patients with CIPA deserve special attention. Here we report a 22-year-old man with CIPA receiving left above-knee amputation due to severe lower extremity infection. General anesthesia was maintained with desflurane, an intervention that has never been reported, and the whole course of operation was uneventful. This is also the first reported case of CIPA in Taiwan.
先天性无痛觉伴无汗症（CIPA）是一种罕见的常染色体隐性遗传病，其特征为先天性痛觉缺失、无汗及智力发育迟缓。由于这些异常情况，CIPA患者的麻醉管理值得特别关注。在此，我们报告一名22岁患有CIPA的男性患者，因严重下肢感染接受了左大腿截肢手术。术中使用地氟醚维持全身麻醉，这一干预措施此前未见报道，且手术全程顺利。这也是台湾地区首例报道的CIPA病例。
