The theoretical and practical problems posed by aneurysms of the ascending aorta with mucoid infiltration of the Media and the aortic leaflets are discussed with reference to two suggestive cases. These histological changes are not specific for Marfan's syndrome, the relation with which is very inconstant. Incomplete replacement of the affected tissues risks a recurrence which is especially serious when the sinus of Valsalva is involved. Therefore, total replacement of the ascending aorta with aortic valve replacement and reimplantation of the coronary arteries is generally indicated. Histology of the excised tissues should be performed routinely with specific stains for mucopolysaccharides. Close follow-up of operated patients with this condition is recommended, especially those with only partial replacements.

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