Sickle cell anemia: intracranial stenosis and silent cerebral infarcts in children with low risk of stroke

Adv Med Sci. 2014 Mar;59(1):108-13. doi: 10.1016/j.advms.2013.09.001. Epub 2014 Mar 27.


Purpose: Children with sickle cell anemia (SCA), who have mean blood flow velocities <170 cm/s in the terminal internal carotid (tICA) or middle cerebral (MCA) arteries on transcranial Doppler ultrasonography (TCD), are considered to be at low risk of stroke. The prevalence of intracranial stenosis, which raises the risk of stroke, is not known in these children. Here, we estimated the prevalence of stenosis and explored its association with silent cerebral infarcts determined based on Magnetic Resonance (MR) scans.

Patients/methods: We studied prospectively a cohort of 67 children with SCA without prior clinically overt stroke or TIA (median age 8.8 years; range limits 2.3-13.1 years; 33 females) and with TCD mean velocity <170 cm/s. They underwent MR imaging of the brain and MR angiography of intracranial arteries.

Results: In 7 children (10.5%, 95% CI: 4.9-20.3%) we found 10 stenoses, including 4 with isolated left tICA stenosis and 3 with multiple stenoses. We found silent infarcts in 26 children (37.7%, 95% CI: 27.2-49.5%). The median number of infarcts in an affected child was 2 (range limits: 1-9), median volume of infarcts was 171 mm(3) (range limits: 7-1060 mm(3)), and median infarct volume in relation to total brain volume was 0.020% (range limits: 0.001-0.101%). The number and volume of infarcts were significantly higher in children with arterial stenosis (both p=0.023).

Conclusions: The prevalence of intracranial arterial stenosis in children with SCA classified as at low risk of stroke by TCD mean velocity <170 cm/s is high. Children with stenosis are at higher risk of brain parenchymal injury as they have more silent cerebral infarcts.

Keywords: Children; Intracranial stenosis; Sickle cell disease; Silent cerebral infarct; Stroke risk; Transcranial Doppler.

Publication types

  • Research Support, N.I.H., Extramural

MeSH terms

  • Adolescent
  • Anemia, Sickle Cell / diagnostic imaging
  • Anemia, Sickle Cell / physiopathology*
  • Cerebral Infarction / complications*
  • Cerebral Infarction / diagnostic imaging
  • Cerebral Infarction / epidemiology
  • Child
  • Child, Preschool
  • Constriction, Pathologic / complications*
  • Constriction, Pathologic / diagnostic imaging
  • Constriction, Pathologic / epidemiology
  • Female
  • Follow-Up Studies
  • Humans
  • Intracranial Arteriosclerosis / complications*
  • Intracranial Arteriosclerosis / diagnostic imaging
  • Intracranial Arteriosclerosis / epidemiology
  • Male
  • Prevalence
  • Prognosis
  • Prospective Studies
  • Stroke / diagnostic imaging
  • Stroke / etiology*
  • Ultrasonography, Doppler, Transcranial