Intracranial and Extracranial Vascular Stenosis as Risk Factors for Stroke in Sickle Cell Disease

Pediatr Neurol. 2021 Jan:114:29-34. doi: 10.1016/j.pediatrneurol.2020.10.006. Epub 2020 Oct 22.


Background: Prevalence and contribution of intracranial and extracranial arterial stenosis to stroke risk were assessed prospectively in children and young adults with sickle cell disease.

Methods: In this cross-sectional study, children and young adults (mean = 19.4 years) with sickle cell disease underwent neurological examination, brain MRI, and magnetic resonance angiography of the head and neck. Two neuroradiologists independently recorded infarcts and arterial stenosis. Clinical features and stroke outcomes were compared between participants with and without stenosis and between children and young adults. Logistic regression analysis assessed the association of variables of interest with overt stroke and silent cerebral infarct.

Results: Of 167 participants (79 children and 88 young adults), 20 (12.0%) had intracranial stenosis, all in the anterior circulation, and nine had concurrent extracranial stenosis. No participants had isolated extracranial stenosis. Participants with intracranial stenosis were more likely than those without stenosis to have an overt stroke (70% vs 5%, P < 0.001) or silent cerebral infarct (95% vs 35%, P < 0.001). Logistic regression analysis indicated that intracranial stenosis was strongly associated with overt stroke when compared with participants with silent cerebral infarct alone and strongly associated with silent cerebral infarct when compared with participants with normal brain MRI; male sex and age were also significant predictors of silent cerebral infarct.

Conclusions: Intracranial stenosis was strongly associated with both overt stroke and silent cerebral infarct; prevalence of intracranial stenosis was similar to prior estimates in sickle cell disease. Extracranial stenosis without concurrent intracranial stenosis did not occur and thus could not be evaluated as an independent risk factor for stroke.

Keywords: Child; Sickle cell disease; Silent cerebral infarction; Stenosis; Stroke.

Publication types

  • Research Support, N.I.H., Extramural
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adolescent
  • Adult
  • Anemia, Sickle Cell / complications*
  • Anemia, Sickle Cell / epidemiology
  • Arterial Occlusive Diseases / complications*
  • Arterial Occlusive Diseases / diagnostic imaging
  • Arterial Occlusive Diseases / epidemiology
  • Cerebral Infarction / diagnostic imaging
  • Cerebral Infarction / epidemiology
  • Cerebral Infarction / etiology
  • Child
  • Constriction, Pathologic / complications
  • Constriction, Pathologic / diagnostic imaging
  • Constriction, Pathologic / epidemiology
  • Cross-Sectional Studies
  • Female
  • Humans
  • Intracranial Arterial Diseases / complications*
  • Intracranial Arterial Diseases / diagnostic imaging
  • Intracranial Arterial Diseases / epidemiology
  • Magnetic Resonance Angiography
  • Magnetic Resonance Imaging
  • Male
  • Neck / blood supply
  • Neck / diagnostic imaging
  • Prevalence
  • Risk Factors
  • Stroke / diagnostic imaging
  • Stroke / epidemiology
  • Stroke / etiology*
  • Young Adult