马方综合征(M arfan’s syndrom e),又叫细长指,蜘蛛指等,是一种遗传性泛发性原发性结缔组织异常,为常染色体显性遗传[1]。本病主要侵犯骨骼,眼及心血管系统。近年来笔者注意收集2家医院共8例马方综合征的病例,均有典型临床表现和影像改变。8例中有3例手术证实,有1例尸检证实,? Marfan syndrome (Mfan’s syndrom e), also known as slender fingers, spider fingers, etc., is a hereditary generalized primary connective tissue abnormalities, autosomal dominant inheritance [1]. The disease mainly affects the bones, eyes and cardiovascular system. In recent years, I pay attention to the collection of two hospitals in a total of 8 cases of Marfan syndrome, both typical clinical manifestations and imaging changes. In 8 cases, 3 cases were confirmed by operation and 1 case was confirmed by autopsy.