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[Morquio-Brailsford综合征的人工关节置换术]

[Endoprosthetic joint replacement in Morquio-Brailsford syndrome].

作者信息

Heisel J, Hesselschwerdt H J

机构信息

Orthopädische Universitätsklinik und Poliklinik Homburg/Saar.

出版信息

Z Orthop Ihre Grenzgeb. 1996 Mar-Apr;134(2):189-94. doi: 10.1055/s-2008-1039794.

Abstract

Morquio-Brailsford's disease belongs to the mucopolysaccharidoses, it shows growth-retarding with dysproportional dwarfism. Due to bone deformations and joint dysplasias very early severe arthritic changes arise with serious functional disabilities, especially in the loaded lower extremities; this may lead to early alloplastic joint replacement surgery. By means of two patients with respective bilateral THR and singular total knee replacement intraoperative complications and postoperative follow up (up to 12 years) are represented casuistically. Unfavourable anatomic properties (dysplasia, size of joints) were the main technical surgery problem; the use of individually manufactured implants might be recommended in some of these cases. The achieved subjective and objective functional results of our cases were-compared to the preoperative situation-altogether quite satisfactory.

摘要

莫尔基奥-布雷斯福德病属于黏多糖贮积症,表现为生长迟缓伴不成比例的侏儒症。由于骨骼畸形和关节发育异常,很早就会出现严重的关节炎变化,并导致严重的功能障碍,尤其是在负重的下肢;这可能导致早期进行人工关节置换手术。本文通过两名分别接受双侧全髋关节置换术(THR)和单例全膝关节置换术的患者,以个案的形式介绍了术中并发症及术后随访情况(长达12年)。不利的解剖学特征(发育异常、关节大小)是主要的手术技术问题;在某些情况下,可能建议使用定制的植入物。与术前情况相比,我们病例所取得的主观和客观功能结果总体上相当令人满意。

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