Tassinari Enrico, Boriani Luca, Traina Francesco, Dallari Dante, Toni Aldo, Giunti Armando
1st Division, Istituto Ortopedico Rizzoli, Via Pupilli 1, 40136, Bologna, Italy.
Chir Organi Mov. 2008 Sep;92(2):123-6. doi: 10.1007/s12306-008-0046-3. Epub 2008 May 16.
We report two cases of bilateral cementless total hip arthroplasty in two young women affected by Morquio-Brailsford syndrome. Morquio-Brailsford disease belongs to the mucopolysaccharidoses; it shows growth retardation with disproportional dwarfism. Usually patients are affected by a severe joint degeneration from their 2nd or 3rd decade. Young age, severe dysplasia, and joint size are the main technical problems for a total hip replacement. Accurate radiographic and CT planning allows the use of standard prostheses instead of custom-made ones.
我们报告了两例患有莫尔基奥-布雷斯福德综合征的年轻女性接受双侧非骨水泥型全髋关节置换术的病例。莫尔基奥-布雷斯福德病属于黏多糖贮积症;表现为生长发育迟缓伴不成比例的侏儒症。通常患者在二三十岁时就会出现严重的关节退变。年轻、严重发育不良和关节尺寸是全髋关节置换术的主要技术难题。精确的影像学和CT规划使得可以使用标准假体而非定制假体。