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伴有凸轮轴畸形-关节病-髋内翻-多发性关节炎-心肌病综合征的青少年严重髋关节病和发育不良的全髋关节置换术。

Total hip arthroplasty in adolescents with severe hip arthropathy and dysplasia associated with camptodactyly-arthropathy-coxa vara-pericarditis syndrome.

机构信息

Department of Orthopaedic Surgery, University of Michigan, Ann Arbor, MI, USA.

出版信息

J Arthroplasty. 2012 Sep;27(8):1581.e5-8. doi: 10.1016/j.arth.2012.01.007. Epub 2012 Mar 3.

Abstract

Camptodactyly-arthropathy-coxa vara-pericarditis (CACP) syndrome is a rare disorder. Patients with this syndrome experience early symptomatic arthropathy of the hips. We report a case of adolescent siblings with bilateral arthropathy associated with CACP syndrome in which total hip arthroplasty was performed as treatment of severe associated disability. Postoperative Harris Hip Scores for patient 1 were 86 for the right at 18 months and 96 for the left at 12 months. Postoperative Harris Hip Score at 6 months for patient 2 was 53; however, he had good range of motion and lacked deformity. Based on our limited experience and the limited available clinical data, we feel that total hip arthroplasty is a reasonable treatment option for adolescents with debilitating hip arthropathy associated with CACP syndrome.

摘要

凸轮轴-关节病-股骨颈干角发育不良-心包炎(CACP)综合征是一种罕见的疾病。患有这种综合征的患者会出现早期髋关节症状性关节炎。我们报告了一对青少年兄妹的病例,他们患有双侧关节炎,伴发 CACP 综合征,为此进行了全髋关节置换术以治疗严重的相关残疾。患者 1 右侧术后 18 个月的 Harris 髋关节评分为 86,左侧术后 12 个月的 Harris 髋关节评分为 96。患者 2 术后 6 个月的 Harris 髋关节评分为 53,但他的活动范围良好,且无畸形。根据我们有限的经验和有限的临床数据,我们认为对于患有与 CACP 综合征相关的致残性髋关节关节炎的青少年,全髋关节置换术是一种合理的治疗选择。

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