目的 探讨Sturge-Weber综合征的病理变化和CT、MRI表现特征,评价其诊断价值.方法 回顾分析15例Sturge-Weber综合征的临床及影像学资料.所有病例均行常规螺旋CT扫描,其中2例有面部三叉神经分布区皮肤血管瘤的患儿,CT扫描未见异常,而进行MRI增强检查.结果 15例CT图像中,13例平扫显示患侧皮层钙化,其中轻度钙化4例,中度钙化8例,重度钙化1例;2例无钙化.2例MRI增强图像中,显示皮层下脑回状强化及软脑膜血管瘤1例;显示侧脑室脉络从增大,室管膜下静脉扩张及深静脉血管团1例.所有患者均有不同程度脑萎缩,其中6例受累脑灰白质不清;侧室脉络从增大4例;颅盖板障增厚6例.结论 脑叶皮层下"脑回样"钙化是Sturge-Weber综合征的特征性表现,CT和MRI是诊断该病的主要检查手段,MRI增强扫描确诊早期Sturge-Weber 综合征有一定价值.%Objective To discuss the pathologic change of sturge-weber syndrome and the manifestation features of CT and MRI to estimate its diagnostic value. Methods Retrospecting and analyzing the clinical and imaging materials of 15 Sturge-Weber syndrome. All patients underwent conventional spiral CT scans of which 2 patients with facial vascular nerves angioma in the trigeminal nerve distribution to find no abnormality so we used MRI enhancing check.Results Among 15 CT images, 13plain scans showed cortical calcification in affected side of which there were 4 with light calcification, 8 with medium calcification, 1 with heavy calcification and 2 with no calcification. In 2 MRI enhancing images,one showed the subcortical gyriform enhancement and the pia mater haemangioma and the other one showed choroids plexus enlargement of lateral venicles, dilated subependymal veins and deep vein vasvular. And all patients had cerebral atrophy in variouds degrees of which there were 6 troubled by unclear encephalon ray matter, 4 with choroids plexus enlargement of lateral ventricles and 6 with crania diploetic prominence.Conclusion The lobe subcortical calcification of"gyriform" is the characteristic manifestation of SturgeWeber syndrome and CT and MRI are the main checking means and it is valuable to use MRI enhancing scans to make a definite diagnosis of early Sturge-Weber syndrome.
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