Labib Smael, Adnane Berdai Mohamed, Abourazzak Sanae, Hida Mustapha, Harandou Mustapha
Department of anesthesia and intensive care, University Hospital Hassan II, Fez, Morocco.
Pan Afr Med J. 2011;9:33. doi: 10.4314/pamj.v9i1.71209. Epub 2011 Jul 25.
Congenital Insensitivity to pain with anhydrosis (CIPA) is a rare inherited disease. It is classified as hereditary sensory and autonomic neuropathy type IV. Pain insensitivity and autonomic deficits are present, but touch and pressure sensitivity are unimpaired. Mental retardation is usually present. We report a family case of a 5 years old girl and 2 years old boy with congenital insensitivity to pain, while discussing the clinical features and the anesthetic strategy of such patients. Patients with Congenital Insensitivity to Pain with anhydrosis may undergo surgery because of susceptibility to trauma due to absence of pain. The clinical features may intrinsically possess anesthetic challenges.
先天性无痛觉伴无汗症（CIPA）是一种罕见的遗传性疾病。它被归类为遗传性感觉和自主神经病变IV型。存在痛觉缺失和自主神经功能缺陷，但触觉和压力觉不受影响。通常伴有智力发育迟缓。我们报告了一例5岁女孩和2岁男孩患有先天性无痛觉的家族病例，同时讨论此类患者的临床特征和麻醉策略。先天性无痛觉伴无汗症患者可能因无痛觉导致易受创伤而需要接受手术。其临床特征可能本身就带来麻醉挑战。
