骨纤维异常增殖症的临床分型及其影像学诊断

目的提高对骨纤维异常增殖症的临床分型认识以及影像学诊断水平。方法结合文献，对经病理证实的27例骨纤维异常增殖症的临床、x线平片、CT和MR资料进行观察分析。根据病变的范围和临床表现进行分型，探讨其各自影像学表现。结果27例骨纤维异常增殖症中，单骨局灶型病变4例，均发生于股骨上段，表现为边界清楚、形态规则且伴有硬化边的囊状病变，病灶内密度高于肌肉，可见斑片状、条絮状钙质样高密度影；单骨广泛型和多骨广泛型病变分别为6例和14例，均表现为囊状膨胀性骨质破坏，毛玻璃样密度，累及范围广泛，边界不清，无硬化边，在MRI上T1WI以等信号为主，脂肪抑制T2WI以稍高信号为主，增强扫描后不均匀较明显强化；多骨广泛型病变合并皮肤色素沉着、性早熟等临床表现即Albright综合症3例。结论不同类型的骨纤维异常增殖症，其临床和影像学表现有所不同，但均以病灶内出现条絮状骨化呈毛玻璃样密度为影像学诊断的共同特征。

Analysis on clinical typing and imaging diagnosis of fibrous dysplasia of bone

Objective To improve the recognization of clinical classification and the level of imaging diagnosis in fibrous dysplasia of bone. Methods Combinating of literature review, 27 cases of pathologically confirmed fibrous dysplasia of bone were analyzed about clinical and radiographic aspects. According to the scope of disease and clinical findings, the clinical classification and their respective imaging were investigated. Results Of 27 cases of fibrous dysplasia of bone, the single focal lesions of bone were seen in 4 cases which occurred in the proxi- mal femur, showing a clear boundary of sclerosis, regular shape and density of lesions higher than muscle. Patchy or floceus high-density ossification can be seen. The extensive lesions in single bone and multiple bone were found in 6 cases and 14 cases respectively, which were shown cystic-like expansible bone destruction, groundglass density and wide range without sclerotic border. MR signal was mainly isointensity on T1WI and hypefintensity signal on T2W1 with unevenly marked enhancement after injection of contrast medium. More extensive lesions of bone with skin pigmentation, precocious puberty and other clinical manifestations, that is, Albfight syndrome were seen in 3 cases. Conclusion The clinical and imaging findings may be different in the different types of fibrous dysplasia of bone, but ossification within lesion and ground -glass density image are their common features of diagnosis.