烟雾病的血管壁解剖病理——从基础到影像

Anatomical pathology of arterial wall in moyamoya disease:from basic research to imaging

解剖病理是理解疾病发病机制的重要基础。烟雾病主干血管病变段的血管壁解剖病理表现为内膜层增厚,增厚成分为层状排列的纤维和细胞,其中有大量平滑肌细胞;内弹力层呈波浪状迂曲,常为双层;中膜平滑肌层变薄。虽然内膜层是增厚的,但高分辨MRI表明大脑中动脉的外径是缩小的。而烟雾血管大部分呈扩张性改变,血管壁变薄,扩张严重者可出现内弹力层变薄、断裂,甚至形成微动脉瘤;少部分烟雾血管,尤其是靠近病变段的穿支动脉,可发生与主干血管类似的狭窄性改变。未来烟雾病血管壁病理学研究要想获得突破性进展,依赖活体标本的获取或动物模型的建立。

Anatomical pathology consists an important basis for the understanding of pathogenesis.Anatomicopathological changes of stenosed arterial wall of moyamoya disease(MMD)include eccentric fibrocellular thickening of intima with abundant smooth muscle cells inside,waving torturosity in the internal elastic lamina which was often duplicated,and thinning of the medial smooth muscle layer.Though the intima was thickened,high-resolution magnetic resonance imaging showed that the outer diameter of middle cerebral artery was diminished.Most of the moyamoya vessels presented dilated changes,including luminal dilation and mural thinning,while severely dilated ones presented thinning or dissecting of internal elastic laminas,even microaneurysms.Some moyamoya vessels,such as perforating arteries close to diseased trunks,presented stenotic changes similar to those of stenosed main trunks.Future breakthrough in pathological studies of MMD arterial depends on the access to arterial wall samples from patients or development of animal models.