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.
Abstract

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