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Sickle Cell Disease and Stroke

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Evidence-Based Imaging in Pediatrics

Key Points

■ Implementation of the Stroke Prevention Trial in Sickle Cell Anemia (STOP) primary prevention strategy that uses transcranial Doppler screening resulted in lower rates in stroke admissions in California (limited evidence).

■ Presence of silent infarcts on MR scans in asymptomatic children with SCD is associated with higher risk for future stroke (limited evidence).

■ The risk of first stroke can be substantially reduced by chronic transfusions in asymptomatic children with SCD and hemoglobin (Hb) SS, in whom intracranial arterial mean velocities are over 200 cm/s on transcranial Doppler examination (strong evidence).

■ Management of children with SCD and acute stroke requires immediate non-contrast CT to exclude intracranial hemorrhage (moderate–strong evidence).

■ Children with symptoms of stroke and negative CT for hemorrhage require urgent MRI/DWI/MRA to assess the degree and extent of brain structural abnormalities and PET/SPECT or MRS to determine the degree of ischemia (moderate evidence).

■ Presence of intracranial arterial stenosis and new lesions on MR imaging in patients with stroke history is associated with high risk for recurrent stroke (limited evidence).

■ There are no specific neuroimaging findings which can suggest that blood transfusions be safely halted in children with SCD (strong evidence).

■ No data were found that evaluate the cost-effectiveness of the different neuroimaging modalities in the evaluation of symptomatic and asymptomatic patients with SCD and suspected stroke (limited evidence).

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Correspondence to Jaroslaw Krejza .

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Krejza, J., Swiat, M., Tomaszewski, M., Melhem, E.R. (2010). Sickle Cell Disease and Stroke. In: Medina, L., Applegate, K., Blackmore, C. (eds) Evidence-Based Imaging in Pediatrics. Springer, New York, NY. https://doi.org/10.1007/978-1-4419-0922-0_5

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