Sturge-Weber综合征在小于1岁婴儿的颅脑MRI表现

目的：回顾性分析Sturge-weber综合征在小于1岁婴儿中的颅脑MRI表现及其临床诊断价值。方法：回顾性分析7例小于1岁的Sturge—Weber综合征患儿的MRI图像，7例行MRI平扫，6例行MRI增强扫描。结果：本组患儿均有面部三叉神经分布区的血管瘤。5例病变累及单侧大脑半球，均可见病变区脑白质于T2wI上信号明显减低，伴有局部脑萎缩改变，其中4例可见患侧脉络丛扩大；2例累及双侧大脑半球脑叶。6例增强后均可见脑回表面软脑膜强化，其中5例可见异常强化的软脑膜血管瘤，1例同时见幕下结构受累。结论：对临床怀疑为Sturge—Weber综合征的婴儿，MRI增强扫描可以帮助明确诊断及确定病变范围，为临床评估患儿的预后提供有效的参考信息。

Cerebral MRI features of Sturge-Weber syndrome in infants less than ly of age

Objective：Retrospective study of cerebral MR imaging features of Sturge-Weber syndrome （SWS） in in- fant less than ly of age was performed,and their clinical Significance was evaluated. Methods：The MRI features of 7 infants less than l y age with SWS were retrospectively analyzed. All had plain MRI,6 patients had enhanced MRI. Results： All pa- tients had characteristic features of angioma involving facial-trigerminal nerves distributed area. Unilateral cerebral sphere was involved in 5 patients, the signal intensities of involved white matters on T2 WI were significantly reduced,accompanied with local brain atrophy and and choroid plexus enlargement （n= 4）;2 patients had bilateral cerebral spheres involved. In the6 infants with contrast-enhanced MRI, letomeningeal enhancement could be revealed, 5 patients had marked enhanced leptomeningeal angioma, 1 patient had infratentorial structures involvement simultaneously. Conclusion： For infants with clinically suspected SWS,contrast enhanced MRI is helpful in the diagnosis and demonstration of involved regions, provides significant reference for the prognosis.