Table 2.

Diagnostic Criteria of Vascular Ehlers-Danlos Syndrome (3).

Inheritance
	Autosomal dominant
Major criteria
	Family history of vascular Ehlers-Danlos Syndrome with documented causative variant in COL3A1
	Arterial rupture at a young age
	Spontaneous sigmoid colon perforation in the absence of known diverticular disease or other bowel pathology
	Uterine rupture during the third trimester in the absence of previous C-section and/or severe peripartum perineum tears
	Carotid-cavernous sinus fistula formation in the absence of trauma
Minor criteria
	Bruising unrelated to identified trauma and/or in unusual sites such as cheeks and back
	Thin, translucent skin with increased venous visibility
	Characteristic facial appearance
	Spontaneous pneumothorax
	Acrogeria
	Talipes equinovarus
	Congenital hip dislocation
	Hypermobility of small joints
	Tendon and muscle rupture
	Keratoconus
	Gingival recession and gingival fragility
	Early onset varicose veins (under age 30 and nulliparous if female)