11例先天性无痛无汗症的骨关节病影像学分析

Imaging Features of Osteoarthrosis in Congenital Insensitivity to Pain with Anhidrosis(11 Cases Report)

目的探讨先天性无痛无汗症(CIPA)并发骨关节病的影像学表现。方法回顾性分析11例经临床实验室检查及影像学证实的CIPA患者的影像资料,11例均行X线检查,其中5例行CT检查,3例行MRI检查。结果 11例共有30处病变,其中18处主要位于骨干或干骺部,好发于双足跖骨,以骨质增生硬化为主要表现,位于关节的12处病变表现为骨端增生或骨质吸收、碎裂,关节脱位。结论 CIPA累及的关节病变有一定的影像特征,结合临床可作出较正确的诊断。

Objective To investigate the imaging findings of osteoarthrosis in congenital insensitivity to pain with an- hidrosis （CIPA）. Methods The imaging data of 11 patients with CIPA were retrospectively analyzed. All the patients un- derwent X-ray examination, 5 patients underwent CT and 3 patients underwent MRI examination. Results There were 30 lesions found in ll patients, Among them, 18 lesions involved diaphysis or metaphysis, mostly in metatarsal bones with bone sclerosis. The other 12 lesions involved joints, with features of bone hyperplasia, absorption, fragmentation or joint dis- location. Conclusion Osteoarthrosis in patients with CIPA had certain image characteristics. Combined with clinical symptoms and imaging features could make the correct diagnosis.