Arterial complications of vascular Ehlers-Danlos syndrome

J Vasc Surg. 2016 Dec;64(6):1869-1880. doi: 10.1016/j.jvs.2016.06.120. Epub 2016 Sep 26.


Background: Vascular Ehlers-Danlos syndrome (EDS) is a relatively rare genetic syndrome that occurs owing to disorders in the metabolism of fibrillary collagen. These defects affect the soft connective tissues resulting in abnormalities in the skin, joints, hollow organs, and blood vessels. Patients with these defects frequently present at a young age with spontaneous arterial complications involving the medium-sized arteries. Complications involving the hollow organs, such as spontaneous colonic perforation, are observed as well. Given the fragility of the soft tissue, open and endovascular intervention on patients with vascular EDS is fraught with high complication rates.

Methods: A PubMed search was performed to identify manuscripts published related to vascular EDS. This search included more than 747 articles. These findings were cross-referenced using key terms, including endovascular, embolization, surgery, genetics, pathophysiology, connective tissue disorders, vascular complications, systematic review, type III collagen, and COL3A1.

Results: The references in key articles and review articles were evaluated for additional resources not identified in the PubMed search. Care must be taken to balance the risk of intervention vs the risk of continued observation. Life-threatening hemorrhage, however, mandates intervention.

Conclusions: With careful, altered approaches to tissue handling, endovascular approaches may provide a safer option for managing the arterial complications observed in patients with vascular EDS. Additional hope may also be found in the use of pharmacologic agents that reduce the incidence and severity of the arterial complications.

Publication types

  • Review
  • Systematic Review

MeSH terms

  • Aneurysm / diagnostic imaging
  • Aneurysm / genetics
  • Aneurysm / mortality
  • Aneurysm / therapy*
  • Collagen Type III / genetics
  • Computed Tomography Angiography
  • DNA Mutational Analysis
  • Ehlers-Danlos Syndrome / complications*
  • Ehlers-Danlos Syndrome / diagnosis
  • Ehlers-Danlos Syndrome / genetics
  • Ehlers-Danlos Syndrome / mortality
  • Endovascular Procedures* / adverse effects
  • Endovascular Procedures* / mortality
  • Genetic Predisposition to Disease
  • Humans
  • Mutation
  • Phenotype
  • Predictive Value of Tests
  • Risk Factors
  • Treatment Outcome


  • COL3A1 protein, human
  • Collagen Type III