Fibrous dysplasia

被引:0
作者
Waltermann, A. [1 ]
Westhoff, B. [1 ]
机构
[1] Univ Klinikum Dusseldorf, Dusseldorf, Germany
来源
ORTHOPADIE | 2024年 / 53卷 / 10期
关键词
McCune-Albright syndrome; Pathological fracture; Bone deformities; Benign bone tumor; Skeletal disease; BONE; DEFORMITY; LESIONS;
D O I
10.1007/s00132-024-04548-w
中图分类号
R826.8 [整形外科学]; R782.2 [口腔颌面部整形外科学]; R726.2 [小儿整形外科学]; R62 [整形外科学(修复外科学)];
学科分类号
摘要
Fibrous dysplasia is a sporadically occurring benign skeletal disease characterized by the replacement of normal bone tissue with excessively proliferating cellular fibrous tissue. It can occur in a monostotic or polyostotic form. Depending on the location, number and size of the lesions, the clinical picture can vary from an asymptomatic disease to a severe disability. Typical problems are bone pain, bone deformities and pathological fractures. In combination with endocrinopathies and/or skin manifestations (caf & eacute; au lait spots), it is referred to as the McCune-Albright syndrome. The diagnosis is mainly carried out radiologically and the bony lesions are characterized by a cloudy, frosted glass-like aspect. Causal treatment is not possible. Orthopedic treatment includes pain relief, bone stabilization, deformity correction and, if necessary, lesion cleansing as well as the prevention of progression by means of antiresorptive medication. Pathological fractures are preferably stabilized with intramedullary osteosynthesis procedures.
引用
收藏
页码:805 / 816
页数:12
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