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

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.