在PHACE综合征中神经成像发现的长期演变的多中心研究
Elizabeth George1, Mitchell Braun2, Rachel Vassar3
1Department of Radiology and Biomedical Imaging, University of California San Francisco, San Francisco, CA, USA.
概括
近三分之一的患有PHACE综合征的儿童患有渐进性脑血管动脉病变. 长期成像显示这种进展,尽管中风在PHACE患者中并不常见.
科学领域:
- 神经皮肤疾病 神经皮肤疾病
- 儿科神经学 儿科神经学
- 血管异常 血管异常
背景情况:
- PHACE综合征 (后腔异常,血管瘤,动脉异常,心脏异常,眼睛异常) 是一种复杂的神经皮肤疾病.
- 椎动脉异常是需要长期监测的关键特征.
研究的目的:
- 为了评估PHACE综合征的长期神经影像演变.
- 专注于动脉病变和内血管瘤的进展.
主要方法:
- 从16个地点对83名PHACE患者的神经成像进行了回顾性审查.
- 首次扫描 (大约1岁) 与最新可用的扫描 (平均年龄14岁) 的比较.
- 评估结构异常,心脏病发作,血管异常和渐进性动脉病变.
主要成果:
- 在29.4%的患者中观察到渐进性动脉病变,包括7%的莫亚莫亚.
- 最初的血管风险评分没有预测动脉病变的进展.
- 内血管瘤与梅克尔洞穴的扩大有关,这表明了进化变化.
结论:
- 随着时间的推移,相当一部分PHACE患者会发展成渐进性脑血管动脉病变.
- 脑卒中很少发生,但动脉病变进展的风险需要进一步调查,以找到最佳的监测策略.
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