Abstract
BACKGROUND: Vascular Ehlers-Danlos syndrome (vEDS) is a rare disorder with poor prognosis, owing to associated vascular complications. However, the most prevalent arterial problems in patients with vEDS are not well known. METHODS: We retrospectively examined 20 consecutive patients diagnosed with vEDS and examined their clinical events, image findings, and therapies. RESULTS: The age at first complication requiring admission was 29 ± 13 years. The observational period was 67 ± 30 months. Of the 20 patients, 17 took celiprolol at final assessment. At the final follow-up, the total number of complications relating to lesions and requiring admission was 16 for pulmonary lesions (8 patients), 16 for bowel lesions (8 patients), 5 for tendon/ligament lesions (2 patients), 18 for the branch arteries of the abdominal aorta (10 patients), 2 for the aorta (2 patients), and 7 for other arteries (6 patients). Of 54 arterial involvements (aneurysms, dissections, and ruptures), both with and without symptoms, 43 (80%) were in branches of the abdominal aorta (celiac artery and branches, 8; superior mesenteric artery, 4; renal arteries, 3; iliac arteries and branches, 28), 2 (4%) were in the aorta, and 9 were in other arteries. The diameter of the sinus of Valsalva was 29 ± 5 mm, within the normal range. During follow-up, 3 patients died due to suspected ruptures in a branch of the celiac artery, the superior mesenteric artery, and the aorta. CONCLUSION: Our findings indicate that lesions involving the branch arteries of the abdominal aorta, rather than aorta, were the most prevalent lesion type in patients with vEDS.