Table 3.

Presentation, management, and complications of brachiocephalic arteries pathology in patients with vEDS.

	Arterial Pathology	N	Mutation	Age range	Presentation	Management	Complications/Comments
Carotid Artery	Dissection	5	MIN (4), HI (1)	26–51	Surveillance (2), Symptomatic (1), Trauma (2)	Non-operative	None
	Stenosis	2	MIN (1), HI (1)	36–41	Surveillance	Non-operative	FMD appearance (HI)
	Ectasia	3	MIN (2), HI (1)	36–63	Surveillance	Non-operative	None
	Aneurysm	7	MIN	22–56	Surveillance (6), Symptomatic (1)	Non-operative (6), Coiling & stenting (1)	TIA post coiling (case in Figure 5)
	CCF	5	MIN	25–46	Symptomatic	Endovascular	One required repeat embolization 2 years later
Vertebral Artery	Dissection	6	MIN (5), HI (1)	22–52	Surveillance (3), Symptomatic (3)	Non-operative	PCA infarct (age 52), Stenosis on F/U (n=2)
	Ectasia	1	MIN	36	Surveillance	Non-operative	None
	Aneurysm	1	MIN	29	Surveillance	Non-operative	0.5 cm at V2
Subclavian Artery	Dissection	2	MIN	41	Surveillance	Non-operative	Bilateral in same case
	Ectasia	1	MIN	52	Surveillance	Non-operative	None
	Aneurysm	3	MIN	26–34	Surveillance	Non-operative (3),	None
	Aneurysm	1	MIN	26	Symptomatic	R SCA ligation, L axillary to R SCA bypass	Bypass Thrombosis, reintervention, alive at age 27
	Aneurysm	1	MIN	18–32	Rupture (R SCA)	Endovascular stenting R CCA/ R SCA	No complications (case in Figure 7)
	Aneurysm	1	MIN	32	Rupture (L SCA)	Carotid Subclavian bypass	Post op infarcts, prolonged vent dependence, tracheostomy

CCF: Carotid-Cavernous fistula, CCA: Common Carotid Artery. SCA: Subclavian artery, Op: Operative, F/U: Follow-up, R: right, L: Left, MIN: mutations that lead to minimal normal type III collagen production. HI: mutations that lead to 50% normal type III collagen production.