Table 2.

Presentation and management of arterial pathology in patients with vascular Ehlers-Danlos syndrome by artery involved. The % given is the percentage of all arterial pathology

Arteries N (%)	% Male	Median Age (IQR)	Known vEDS diagnosis	Missense mutation	Dissection	Aneurysm, Pseudoaneur ysm	Rupture	Medical Management	Endovascular Embolization	Endovascular Stenting	Open Repair
Carotid N=17 (12.2)	5 (41.7)	27 (24–36)	4 (33.3)	8 (66.7)	6 (50)	5 (41.7), 1 (8.3)	0	100%	0	0	0
Vertebral N=6 (4.3)	2 (33.3)	27.5 (22–32.5)	3 (50)	5 (83.3)	4 (66.6)	1 (16.7)	0	100%	0	0	0
Celiac N=14 (10.5)	6 (40)	44 (33–50)	12 (80)	11 (73.3)	7 (46.7)	7 (46.7), 2 (13.3)	2 (13.3)	13 (86.7)	1 (6.7)*	0	1 (6.7)
Gastric N=3 (2.2)	0	37 (36–37)	2 (66.7)	2 (66.7)	1 (33.3)	0	0	3 (100)	0	0	0
Phrenic N=1 (0.7)	0	58	1 (100)	1 (100)	0	1 (100)	0	1 (100)	0	0	0
Splenic N=11 (7.9)	3 (27.3)	44 (31–51)	6 (54.5)	6 (54.5)	2 (18.2)	8 (72.7)	4 (36.4)	4 (36.4)	6 (54.5)	0	1 (9.4)
Hepatic N=8 (5.8)	5 (62.5)	47 (38.5–50.5)	5 (62.5)	4 (50)	0	7 (87.5), 1 (12.5)	1 12.5)	5 (62.5)	2 (25)	1 (12.5) (Figure 3)	0
Superior mesenteric N=6 (4.3)	1 (16.7)	45.5 (39–54.4)	5 (83.3)	3 (50)	2 (33.3)	2 (33.3)	1 (16.7)	5 (83.3)	1 (16.7)*	0	0
Renal N=17 (12.2)	12 (70.6)	37 (30.5–41)	9 (52.9)	13 (76.5)	7 (41.2)	8 (47.1), 1 (5.9)	0	14 (82.4)	1 (5.9)	0	2 (11.8)
Iliac N=23 (16.5)	18 (78.3)	41 (30–48)	12 (52.2)	19 (82.6)	7 (30.4)	16 (69.6)	1 (4.3)	17 (73.9)	1 (4.2)	2 (8.7)	3 (13)
Femoral N=2 (1.4)	1 (50)	27,33	0	2 (100)	1	1	0	1	0	0	1
Popliteal N=1 (0.7)	0	26	0	1 (100)	0	1	0	1 (100)	0	0	0
Posterior Tibial N=3 (2.2)**	2 (100)	19,32	1 (50)	1 (50)	0	3	0	2	0	0	1

^*Same individual, death due to multiple mesenteric arterial ruptures

^**One individual had bilateral posterior tibial arteries aneurysms