粘多糖病Ⅰ型的X线、CT诊断

Diagnosis of X-ray、CT with mucopolysaccharidosis types

目的分析粘多糖病Ⅰ型的X线、CT表现,提高对本病的认识。方法回顾分析粘多糖病Ⅰ型的X线表现特点和CT检查及图像后处理表现。结果粘多糖病Ⅰ型具有较典型的骨骼X线改变,(1)头大,蝶鞍呈乙型;(2)掌指骨粗短;(3)上、下肢骨中央膨胀两端变细;(4)脊椎后凸成角,后凸椎体前下缘成鸟嘴状突出,上下椎体呈卵圆形;(5)肋骨增宽呈船桨;(6)髋臼变浅,髋臼角增大;(7)股骨颈增长,髋内翻或外翻。结论粘多糖病Ⅰ型的影像表现有一定的特异性,X线照片、CT检查及图像后处理技术有利于提高粘多糖病Ⅰ型的诊断水平。

Objective To analyze the X-ray and CT manifestations of mucopolysaccharidosis type I ,thus to improve the knowledge about it. Methods Respectively analyzed the X-ray, CT and image post-processing manifestations of mucopolysaccharidosis type I . Results Mucopolysaccharidosis type I had characteristic X-ray changes of bones： ① Eniarged skull and hookshape Sella Turcica, ② Short and thick phalanges and metacarpal Bones, ③Dilated central part and thin ends of upper and lower limb bones, ④Kyphosis deformity of spine, beak-shape stick out of anteroinferior margin of Kyphosis deformity vertebra, ⑤Broadening ribs as paddle shape, ⑥Shallow acetabulum and enlarged Acetabular Angle,⑦Prolonged femoral neck, Coxa Vara or coxa valga. Conclusion Mucopolysaccharidosis type I has characteristic imaging manifestations. X-ray, CT examination and image post-processing can improve the diagnostic level of Mucopolysaccharidosis type I .