儿童戈谢病累及股骨和膝关节的MRI表现

目的观察戈谢病儿童股骨和膝关节的MRI表现。方法 39例戈谢病患儿接受3.0T MR检查,包括冠状位T1W、T2W、STIR序列,检查部位为双侧股骨包括膝关节,分析和描述其MRI特点。结果 38例发现骨髓浸润,其中32例表现为稍长T1稍短T2信号;28例T1WI、T2WI骨髓腔信号不均匀减低,4例信号弥漫均匀减低;6例伴骨梗死。16例骨干、干骺端及骨骺同时受累;18例骨干及干骺端受累;单纯累及骨干或干骺端者各2例。结论儿童戈谢病骨髓浸润MRI表现具有一定特征性,综合不同序列MR图像的信号特点,可评估骨髓浸润范围、判断是否伴发合并症,为临床治疗提供信息。

MRI findings of femur and knee joint involvement of Gaucher disease in children

Objective To observe MRI characteristics of femur and knee joint involvement in children with Gaucher disease. Methods Follow-up data of 39 children with Gaucher disease who underwent 3.0T MR scan were analyzed. A standard MRI protocol was performed in all patients using T1-, T2-weighted and STIR-sequence of the bilateral femurs and knee joints. The manifestations of MRI were analyzed and described. Results Bone involvements were found in 38 patients. Mild hyposignal on T1WI and T2WI were revealed in 32 patients, including hertergenous signal in 28 and homogeous in 4 patients. Osteonecrosis was observed in 6 patients. Bone marrow infiltration involvement of femoral diaphysis, epithysis and metaphysis were found in 16 patients, both femoral diaphysis and metaphysis in 18 patients, only diaphysis or epithysis/metaphysis each in 2 patients. Conclusion MRI manifestations of bone marrow involvement of Gaucher disease in children have characteristics. Comprehensive analysis of different sequences MR imaging is valuable for diagnosis and assessing bone infiltration and complications, and is helpful for clinical treatment.