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

J Stroke Cerebrovasc Dis. 2017 Aug;26(8):1678-1682. doi: 10.1016/j.jstrokecerebrovasdis.2017.03.025. Epub 2017 Jun 21.

Abstract

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.

Keywords: Ehlers–Danlos syndrome; carotid aneurysm; carotid dissection; cerebral aneurysm; cerebrovascular malformation; vertebral dissection.

MeSH terms

  • Adolescent
  • Adult
  • Case-Control Studies
  • Cerebrovascular Disorders / diagnosis
  • Cerebrovascular Disorders / epidemiology*
  • Chi-Square Distribution
  • Comorbidity
  • Databases, Factual
  • Ehlers-Danlos Syndrome / diagnosis
  • Ehlers-Danlos Syndrome / epidemiology*
  • Female
  • Hospitalization*
  • Humans
  • Inpatients
  • Logistic Models
  • Male
  • Middle Aged
  • Multivariate Analysis
  • Odds Ratio
  • Prevalence
  • Risk Assessment
  • Risk Factors
  • Time Factors
  • United States / epidemiology
  • Young Adult