Urfalioglu Aykut, Arslan Mahmut, Duman Yakup, Gisi Gökce, Oksuz Gözen, Yildiz Hüseyin, Oksuz Hafize, Balaban Ayşe
Department of Anesthesia and Reanimation, Kahramanmaras Sutcu Imam University, School of Medicine.
Department of Plastic and Reconstructive Surgery, Kahramanmaras Sutcu Imam University, School of Medicine.
J Nippon Med Sch. 2017;84(5):237-240. doi: 10.1272/jnms.84.237.
Congenital insensitivity to pain with anhidrosis (CIPA) syndrome is a neuropathy characterized by insensitivity to pain, impaired thermoregulation, anhidrosis, and mental retardation. A 9-year old boy with CIPA syndrome, underwent 2 operations for a calcaneal ulcer. During the first operation standard monitorization was performed. In the second operation, Bispectral Index (BIS) monitoring was added and temperature was monitored with an esophageal probe. In the first operation, in which anesthesia induction was applied with ketamine and midazolam, extremity movements with surgical stimuli were seen. Despite pain insensitivity, as extremity movements were seen with surgical stimuli, propofol was administered in the second operation. Throughout the operation, the BIS values varied from 19-58 and body temperature was measured as 36.1°C-36.9°C. In conclusion, despite the absence of pain sensitivity in CIPA syndrome cases, there is an absolute need for the administration of anesthesia in surgical procedures because of tactile hyperesthesia.
先天性无痛觉伴无汗症（CIPA）综合征是一种神经病变，其特征为对疼痛不敏感、体温调节受损、无汗以及智力发育迟缓。一名患有CIPA综合征的9岁男孩因跟骨溃疡接受了2次手术。第一次手术时进行了标准监测。第二次手术时增加了脑电双频指数（BIS）监测，并使用食管探头监测体温。在第一次手术中，使用氯胺酮和咪达唑仑进行麻醉诱导，术中可见手术刺激引起的肢体运动。尽管对疼痛不敏感，但由于手术刺激时可见肢体运动，因此在第二次手术中给予了丙泊酚。整个手术过程中，BIS值在19 - 58之间变化，体温测量值为36.1°C - 36.9°C。总之，尽管CIPA综合征患者对疼痛不敏感，但由于存在触觉过敏，在外科手术中绝对需要进行麻醉。
