纤维发育不良致病理性骨折1例

B. Supriyadi
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引用次数: 0

摘要

纤维性发育不良(FD)被描述为一种生长障碍,其特征是正常骨元素逐渐被纤维性骨组织取代。FD影响的骨骼表现为骨骼弱化,易发生病理性骨折。我们报告了一例8岁男孩的FD,他在跌倒后出现右上大腿疼痛,头部和足部出现肿块而来到医院。x线片显示畸形为膈肌扇形,右股骨上段近三分之一处、大转子和小转子处有透光性病变,远端畸形为完全横向不连续性。在其他骨骼中没有发现类似的病变。畸形区的骨结构和骨小梁减少,皮质变薄。MRI示骨尺寸增大,T1WI呈低信号,T2WI呈不均匀高信号。纤维发育不良伴病理性骨折是一种罕见的病例。x线片表现为透膜扇形,透光率为磨玻璃,表现为谢泼德氏弯曲畸形。该病变伴有远端病变的病理性骨折(脆性骨折)。以骨发育异常为特征的纤维发育不良在T1W1上呈不均匀的低信号,在T2WI上呈高信号。
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Pathological fracture in fibrous dysplasia: a case report
Fibrous dysplasia (FD) is described as a growth disorder characterized by the progressive replacement of normal bone elements by fibrous-osseus tissue. Bones affected by FD is presented with bone weakening and prone to pathological fracture. We reported a case of FD in an 8-years-old boy, who came to the hospital with pain in the upper right thigh after falling with bumps in his head and foot. An X-ray revealed a deformity as a diasteal scalloping with radiolucency lesions on one-third proximal of the right upper femur, greater trochanter, and lesser trochanter, with complete transverse discontinuity in the distal deformity. No similar lesion was found in the other bones. Bone structure and trabeculation in the deformity area has decreased and the cortex was thinning. On magnetic resonance imaging (MRI), bone size was enlarged, hypointense on T1WI and inhomogeneous hyperintense on T2WI. Fibrous dysplasia with a pathological fracture is a rare case. The appearance on the X-ray was diasteal scalloping with a ground-glass radiolucency represented with Shepherd's crook deformity. This lesion was accompanied by a pathological fracture (fragility fracture) on distal lesion. Fibrous dysplasia which characterized by bone developmental anomaly gave an inhomogeneous hypointense on T1W1 and hyperintense on T2WI.
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