Lewis J R Pryce, Gibson P H
Department of Orthopaedic Surgery, Nelson R. Mandela School of Medicine, University of Kwa-Zulu Natal, South Africa.
J Bone Joint Surg Br. 2010 Feb;92(2):289-92. doi: 10.1302/0301-620X.92B2.23104.
The management of joint replacement in lysosomal storage diseases has not been well reported. We present three patients with progressive degenerative changes of the hips who required bilateral total hip replacement in early childhood. The stature of the patients make it essential to have access to appropriately scaled prostheses. Consideration has to be given to associated disorders of the skeleton which must be carefully screened to ensure safety in providing appropriate anaesthesia as well as ensuring that there is no cardiac abnormality. In one patient, a periprosthetic fracture was sustained in one hip in the early post-operative course requiring internal fixation. The patient made a full recovery and all six hips were clinically and radiologically satisfactory at mid-term review.
溶酶体贮积病中关节置换的管理尚未得到充分报道。我们介绍了三名患有髋关节进行性退行性改变的患者，他们在幼儿期就需要进行双侧全髋关节置换。患者的身材使得必须使用尺寸合适的假体。必须考虑相关的骨骼疾病，必须仔细筛查这些疾病，以确保在提供适当麻醉时的安全性，并确保没有心脏异常。在一名患者中，术后早期一侧髋关节发生假体周围骨折，需要内固定。患者完全康复，中期复查时所有六个髋关节在临床和放射学上均令人满意。
