A 10-year follow-up of asymptomatic Charcot hip joints caused by CIPA syndrome (congenital insensitivity to pain with anhidrosis) with failure of any surgical reconstructive treatment

对由CIPA综合征(先天性痛觉缺失伴无汗症)引起的无症状夏科氏髋关节进行10年随访,这些患者均未接受任何手术重建治疗。

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Abstract

Congenital insensitivity to pain with anhidrosis (CIPA) is a rare genetic disorder. Patients with CIPA lack among other things the protective sensation of pain. Orthopaedic manifestations are fractures, infections and hip joint dislocation. We report a 10-year follow-up of late developing hip dysplasia with CIPA and the result of several reconstructive surgical procedures. The final outcome was asymptomatic Charcot hip joints with superior migration and dislocation of both hips without pain. In conformance with the literature standard operative techniques in these rare cases usually seem to fail. A multidisciplinary conservative approach with close observation and activity modification is the proposed treatment of choice.

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