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12 Pediatric Sickle Cell Disease and Stroke

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Key Points

Implementation of the Stroke Prevention Trial in Sickle Cell Anemia (STOP) primary prevention strategy that uses transcranial Doppler (TCD) screening resulted in lower rates in stroke admissions in California (limited evidence). Presence of silent infarcts on magnetic resonance (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 TCD examination (strong evidence). Management of children with SCD and acute stroke requires immediate non-contrast computed tomography (CT) to exclude intracranial hemorrhage (moderate–strong evidence). Children with symptoms of stroke and negative CT for hemorrhage require urgent MRI/diffusion-weighted imaging (DWI)/ MR angiography (MRA) to assess the degree and extent of brain structural abnormalities and positron emission tomography (PET)/single photon emission CT (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. (2011). 12 Pediatric Sickle Cell Disease and Stroke. In: Medina, L., Blackmore, C., Applegate, K. (eds) Evidence-Based Imaging. Springer, New York, NY. https://doi.org/10.1007/978-1-4419-7777-9_12

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