In cases of aortic aneurysm involving degeneration of the media layer, which syndrome is commonly recognized?

The syndrome commonly recognized in cases of aortic aneurysm involving degeneration of the media layer is Marfan's syndrome. This genetic disorder is caused by mutations in the FBN1 gene, which encodes fibrillin-1, an important protein for connective tissue integrity. Individuals with Marfan’s syndrome have a tendency to develop cardiovascular abnormalities, particularly affecting the aorta, leading to dilation, dissection, or aneurysm due to the defective elastic fibers in the media layer of the aortic wall.

The degeneration of the media layer, characterized by cystic medial necrosis, results in weakened structural support for the aorta, making it prone to aneurysms. In patients with Marfan's syndrome, the aortic root is particularly affected, which can lead to significant cardiovascular complications.

While Ehlers-Danlos syndrome can also be associated with vascular problems and may lead to aneurysms, the specific pattern of aortic involvement and the mechanism involving the media layer degeneration is most pronounced in Marfan's syndrome.