Increased Prevalence of Cerebrovascular Disease in Hospitalized Patients with Ehlers–Danlos Syndrome

Sarasa T. Kim, Harry Cloft, Kelly Flemming, David F Kallmes, Giuseppe Lanzino, Waleed Brinjikji

Research output: Contribution to journalArticlepeer-review

4 Scopus citations


Background and Purpose Small studies have suggested that Ehlers–Danlos syndrome (EDS) is associated with a number of cerebrovascular complications. We sought to determine whether a clinical diagnosis of EDS is associated with a higher prevalence of cerebrovascular diseases than the general population by performing a case-control study of hospitalized patients in the Nationwide Inpatient Sample (NIS). Methods Using the 2000-2012 NIS, we performed a case-control study matching cases of EDS to controls without such a diagnosis. The prevalence of various cerebrovascular diseases between the 2 groups was compared, and multivariate logistic regression was used to adjust for suspected comorbidities. Results Between 2000 and 2012, there were a total of 9067 discharges carrying a diagnosis of EDS. On univariate analysis, patients with EDS were more likely to be hospitalized for carotid dissection (.2% versus.01%, odds ratio [OR] = 18.0, confidence interval [CI] = 2.41-135.12, P <.0001), vertebral dissection (.1% versus 0%, P =.008), cervical artery aneurysm (.1% versus.01%, OR = 9.01, CI = 1.14-71.11, P <.0001), cerebral aneurysm (.4% versus.09%, OR = 4.89, CI = 2.28-10.47, P <.0001), and cerebrovascular malformation (.1% versus.02%, OR = 5, CI = 1.10-22.85, P =.021), compared to the controls. On multivariate analysis adjusted for age, race, and comorbidities, EDS patients had significantly higher odds of carotid dissection (OR = 15.02, CI = 3.08-270.87, P <.0001), vertebral dissection (OR = 2406539.5, P =.0037), cervical artery aneurysm (OR = 11.75, CI = 2.11-220.71, P =.0026), cerebral aneurysm (OR = 5.59, CI = 2.69-13.18, P <.0001), and cerebrovascular malformation (OR = 4.67, CI = 1.20-30.87, P =.0243). Conclusions Carotid and vertebral dissections, cervical and cerebral aneurysms, as well as other cerebrovascular malformations are more common in hospitalized patients with EDS compared to controls.

Original languageEnglish (US)
Pages (from-to)1678-1682
Number of pages5
JournalJournal of Stroke and Cerebrovascular Diseases
Issue number8
StatePublished - Aug 1 2017


  • carotid aneurysm
  • carotid dissection
  • cerebral aneurysm
  • cerebrovascular malformation
  • Ehlers–Danlos syndrome
  • vertebral dissection

ASJC Scopus subject areas

  • Surgery
  • Rehabilitation
  • Clinical Neurology
  • Cardiology and Cardiovascular Medicine


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