Ultrastructural and histochemical studies of vascular lesions in Marfan's syndrome, with report of 4 autopsy cases.

Abstract

Histochemical observation was conducted on arterial lesion in 4 cases of Marfan's syndrome and electron microscopic observation was also carried out on the aorta and gastroepiploic artery in 2 of the 4 cases. Acid mucopolysaccharide (AMPS) noted In cystic medionecrosis demonstrated meshwork of spheres and threads, and showed the same structure as the ground substances widely distributed in the mesenchymal connective tissues including normal arteries and as AMPS in cystic medionecrosis‐like lesions such as arteriosclerosis, mesoaortitis syphilitica, and aortitis syndrome (Takayasu's disease). In histochemical observation, there was found no distinct qualitative difference between AMPS in Marfan's syndrome and that in controls. The overaccumulation of AMPS is not a direct cause of vascular lesions in Marfan's syndrome.The pattern of degeneration of elastic fibers was peculiar to Marfan's syndrome but it also resembled senile change. Degeneration of elastic fibers was accompanied by regeneration in the same area. There was observed no electron microscopic anomaly in collagen fibers nor was found anomaly of fibrlllogenesis in media smooth muscle cells. From the point of view of cell pathology, attribution of the cause of vascular lesion merely to some enzyme deficiency results in much contradiction.