Objective: Patients with vascular Ehlers-Danlos syndrome are predisposed to premature vascular complications. The objective of this article is to review the CT findings and current thoughts about the management of vascular pathologic abnormalities and diseases in patients with Ehlers-Danlos syndrome.
Conclusion: The manifestation of vascular Ehlers-Danlos syndrome frequently involves multiple vascular segments, with aneurysms and dissections being the most common imaging findings. The traditional practice of avoiding surgery until complications develop is being modified in select cases.