Table 4.

Clinical criteria for vascular Ehlers-Danlos syndrome (vEDS) according to the revised 2017 EDS nosology.

Major Criteria	Minor Criteria
1. Family history with documented COL3A1 variant 2. Arterial rupture at a young age 3. Spontaneous sigmoid colon perforation 4. Uterine rupture during the third trimester in the absence of previous C-section and/or severe peripartum perineum tears 5. Carotid-cavernous sinus fistula formation in the absence of trauma	1. Bruising unrelated to identified trauma 2. Thin, translucent skin with increased venous visibility 3. Characteristic facial appearance 4. Spontaneous pneumothorax 5. Acrogeria 6. Talipes equinovarus 7. Congenital hip dislocation 8. Hypermobility of small joints 9. Tendon and muscle rupture 10. Keratoconus 11. Gingival recession/fragility 12. Early-onset varicose veins
Minimal criteria suggestive for vEDS: Family history of the disorder, arterial rupture or dissection in individuals less than 40 years of age, unexplained sigmoid colon rupture, or spontaneous pneumothorax in the presence of other features consistent with vEDS.