A comparative study of the aortic wall in patients with Marfan's syndrome and Erdheim's disease.

The materiel of intraoperative biopsies (378) and autopsies was used to study the morphology of the aortic wall in patients with Marfan's syndrome (62) and Erdheim's disease (133). Histological, histochemical and electron microscopy research methods were employed with an assay of the results obtained. It has been demonstrated that in connective tissue dysplasia in the aorta, the histological structure of the intima undergoes substantial changes alongside the pathology of the tunica media. It is marked by the formation of a focal and diffuse thickening with the predominance of the interstitial substance, rich in glycosaminoglycans, and of a small amount of fibrous structures. The morphological signs of the impairment of endothelial permeability (vacuolisation of cytoplasm, thinning of its peripheral portions and basal membrane, expansion of intercellular fissures) are identifiabe, which leads to the disorder of aortic wall metabolism and early development of atherosclerosis. The changes in the intima together with the pathology of the tunica media ere predisposing factors of aneurysm formation, dissection and rupture of the aorta.