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Vascular complications of Ehlers-Danlos syndrome: CT findings
Linda C Chu1, Pamela T Johnson, Harry C Dietz
1The Russell H. Morgan Department of Radiology and Radiological Science, Johns Hopkins Hospital, 601 N Caroline St, JHOC 3254, Baltimore, MD 21287, USA.
Insights
Vascular Ehlers-Danlos syndrome patients often experience early vascular issues. CT scans reveal aneurysms and dissections, prompting a shift towards earlier surgical intervention in some cases.
Area of Science:
- Vascular Medicine
- Radiology
- Genetics
Background:
- Vascular Ehlers-Danlos syndrome (vEDS) is a rare genetic connective tissue disorder.
- Patients with vEDS are at high risk for life-threatening vascular complications, including arterial dissections and aneurysms.
- Early diagnosis and management are crucial for improving patient outcomes.
Purpose of the Study:
- To review computed tomography (CT) imaging findings in patients with vascular Ehlers-Danlos syndrome.
- To discuss current management strategies for vascular abnormalities in vEDS.
- To highlight the evolving approach to surgical intervention.
Main Methods:
- Review of relevant literature on vEDS and vascular pathology.
- Analysis of characteristic CT findings in vEDS patients.
- Discussion of contemporary treatment guidelines and case examples.
Main Results:
- Vascular complications in vEDS commonly affect multiple arterial segments.
- Aneurysms and arterial dissections are the most frequent imaging findings on CT.
- Imaging plays a critical role in identifying disease extent and guiding management.
Conclusions:
- Vascular Ehlers-Danlos syndrome presents with widespread vascular involvement, primarily aneurysms and dissections.
- The management paradigm is shifting from conservative care to earlier surgical consideration in select vEDS patients.
- Multidisciplinary care and advanced imaging are essential for optimal vEDS management.
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.
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