What is the definition of Vascular Ehlers-Danlos Syndrome?

Vascular Ehlers-Danlos syndrome is a more severe form of Ehlers-Danlos syndrome, which is a genetic disorder that affects the connective tissues, such as the skin, blood vessel walls, and joints. Vascular Ehlers-Danlos syndrome can cause the walls of blood vessels, the intestines, or the uterus to rupture.

What are the symptoms for Vascular Ehlers-Danlos Syndrome?

Symptoms of vascular Ehlers-Danlos syndrome include a thin nose, thin upper lip, small earlobes, and prominent eyes, with translucent skin that bruises easily, and fragile blood vessels. Other symptoms of vascular Ehlers-Danlos syndrome may include short stature, premature aging of skin, receding gums, glaucoma, hypermobility of small joints, high blood pressure, collapsed lung, joint dislocations, undescended testicles, uterine prolapse or rupture, and cognitive impairment.

What are the current treatments for Vascular Ehlers-Danlos Syndrome?

There is no cure for vascular Ehlers-Danlos syndrome. Treatment for vascular Ehlers-Danlos syndrome is mostly focused on alleviating symptoms and preventing complications, such as joint dislocations. Treatment for vascular Ehlers-Danlos syndrome includes pain relievers, such as acetaminophen, ibuprofen, and naproxen sodium; medications to lower blood pressure; physical therapy; braces; and surgery to repair joints or ruptured blood vessels and organs. Patients with Ehlers-Danlos syndrome should be regularly screened for aneurysms, which are ballooning of the arteries with possible rupture. Heavy lifting and contact sports should be avoided. Avoid chewing gum, hard foods, and ice. Wear supportive shoes, and sleep with body pillows on a dense foam mattress to provide joint support. Women with vascular Ehlers-Danlos syndrome who are considering pregnancy should meet with a genetic counsellor.
Clinical Trial
  • Status: Recruiting
  • Participants: 3000
  • Start Date: December 2015
Pathogenetic Basis of Aortopathy and Aortic Valve Disease
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  • Intervention Type: Procedure
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  • Start Date: April 1, 2017
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