Vascular Ehlers-Danlos Syndrome (VEDS)

Our approach to Vascular Ehlers-Danlos syndrome (VEDS)

Children with vascular Ehlers-Danlos syndrome often struggle with heart problems. At Stanford Children’s Health, we collaborate closely with our renowned heart specialists. Our advanced heart surgeons are sometimes the only ones in the world who can perform a complex heart surgery, even when you have been told it is not possible elsewhere.

About Vascular Ehlers-Danlos syndrome

As with other connective tissue disorders, Ehlers-Danlos syndrome involves a mutated gene that affects proteins that make up connective tissue. With vascular Ehlers-Danlos syndrome, this protein is collagen III, and the specific gene is COL3A1. When collagen III is abnormal in the body, skin, joints, blood vessels, and organs such as the heart and lungs are affected.

Children with vascular Ehlers-Danlos syndrome can have mild or severe signs and may have characteristic facial features such as a small chin, thin nose and lips, and deep-set, large eyes. Skin can appear translucent with veins easily visible.

Signs of vascular Ehlers-Danlos syndrome

  • Heart and artery problems: As with other syndromes, tissues that make up the walls of arteries and the heart are weak, leaving arteries prone to tearing or stretching. When not treated, complications can become serious, including an enlarged aorta, aneurysms, lung collapse, early onset varicose veins, and more.
  • Organ problems: Rupture of hollow organs like the stomach, intestines, and bladder can occur.
  • Skin conditions: Thin skin, premature aging of the hands and feet, fragile gums, and easy or spontaneous bruising.
  • Bone and joint issues: Flexible joints, including double joints and excess movement in the joints. Tendons and muscles that can tear, hip dislocation, clubfoot.
  • Eye concerns: Thinning cornea or carotid-cavernous sinus fistula (sudden eye redness).

Learn more by visiting the Ehlers-Danlos Society or the VEDS Movement >