Is cystic medial necrosis associated with ehlers-danlos syndrome (eds)?

Yes. Eds is a disease of the connective tissue with several sub-types. The connective tissue layer of the arteries can be affected and this can lead to cystic medial necrosis. This is a degenerative disease of the media of the aorta and other arteries with loss of elastic tissue and muscle cells. It is a pathological substrate for aortic dissection. Not all people affected get cmn.

Related Questions

What is ehlers-danlos syndrome (eds)?

"Ehlers–Danlos" Ehlers–danlos syndrome (also known as "cutis hyperelastica") is an inherited tissue disorder caused by a defect in the body's synthesis of collagen. Collagen in connective tissue helps tissues to resist deformation. In the skin, muscles, ligaments, blood vessels and organs, collagen plays a very significant role and with increased elasticity or "stretchiness" they may not work right. Read more...
"Ehlers–Danlos" Ehlers–danlos syndrome (also known as "cutis hyperelastica") is an inherited tissue disorder caused by a defect in the body's synthesis of collagen. Collagen in connective tissue helps tissues to resist deformation. In the skin, muscles, ligaments, blood vessels and organs, collagen plays a very significant role and with increased elasticity or "stretchiness" they may not work right. Read more...
E-D Syndrome. Ehlers-danlos syndrome is a group of inherited disorders. The characteristics include extremely loose joints and hyperelastic skin. This skin is very prone to brusing. People with the syndrome also are prone to damaged blood vessels and can actually have dissections (tears) of their aorta inner wall. Read more...

Could I have type 3 (hypermobility) ehlers danlos syndrome or hypermobility syndrome? My parents don't have eds.

Unlikely. Ehlers danlos is an inherited condition, so if it doesn't run in your family, it's very unlikely that you have it. If your joints are unusually flexible you may just be more limber than normal. But if you are worried about it, see your doctor and find out for sure. Read more...
See details. The difference is a matter of degree. Eds is most often a recessively inherited disorder so parents usually do not have the disease. Read more...

Does aortic root dilation associated with Ehlers-Danlos syndrome usually develop in childhood? Or can it first show up in adulthood?

Unlikely. Although there are many different subtypes of Ehlers-Danlos syndrome, the overall incidence of aortic rd is less common than in Marfan's where it is thought to be about 80%. The attached paper looked at the hypermotile subtype of EDS and found onset usually before age 14 and this seemed to stabilize in adulthood. Read more...