Aneurysms of the Ascending Aorta Due to Medial Degeneration Associated with Arachnodactyly (Marfan's Disease)

Two cases are described of women showing aneurysm of the ascending aorta due to medial degeneration.In both there was evidence of Mar- fan's (1896) " dolichostenomelie " (arachno- dactyly), a condition which in its fully developed form includes, as well as long, thin fingers and limbs, such changes as joint hyperextensibility, dolichocephaly, and dislocation of the optic lens (Rados, 1942).Severe aortic medial degeneration has been reported in some 25 cases of this disease (Marvel and Genovese, 1951), causing in some dissecting aneurysms and in others true aneurysms of the aortic arch.Because non-syphilitic aneurysms of the latter kind are rare, it is tempting to regard them all as manifetations of either latent or overt Marfan's disease (#4ywlin, 1952).This we hold, at least in older people, to be a mistaken view, since in old age there is commonly a non-specific medial degeneration which can give rise to aneurysm of the aortic arch (Turnbull, 1915;Rottino, 1939).Such cases, however, lack the additional cardiac abnormalities frequently encountered in Marfan's disease, and it is the combination of cardiac and aortic lesions which we regard as diagnostic of the latter, even in its incomplete form.Case Reports Case 1.-A woman, aged 24 years, had been seen 12 years earlier (1938) by Dr. William Evans, when a systolic murmur was heard at the left sternal edge.There was no aortic incompetence, but cardioscopy showed prominence of the ascending aorta and the aortic knuckle.