Neuroimaging in assessment of risk of stroke in children with sickle cell disease

Purpose: Stroke and subclinical "silent infarcts" are major causes of morbidity in children with Sickle Cell Disease (SCD). Ischemic strokes are more common in younger children while hemorrhagic strokes are more frequent in adults. The goal of neuroimaging in acute stroke is to document whether the stroke is ischemic or hemorrhagic, to assess the extent of parenchymal abnormalities and to determine the presence of other cerebrovascular lesions. Computed Tomography (CT) is the primary modality for the assessment of acute stroke patients because of its 24/7 availability and ability to exclude hemorrhagic causes. Magnetic resonance imaging (MRI) and MR angiography (MRA) are recommended to determine precisely extent of infarction and detect cerebrovascular abnormalities. The goal of neuroimaging in patients with hemorrhagic stroke is to identify an arteriovenous malformation or aneurysm(s) amenable to surgery or catheter intervention. The risk of first stroke is very high in asymptomatic children with intracranial arterial mean velocities over 200 cm/s on transcranial Doppler (TCD) examination. The risk can be substantially reduced if chronic blood transfusions are timely implemented. Large cerebral vessel disease detected by TCD can be confirmed or excluded by MRI/MRA. Those with evidence of parenchymal and/or cerebrovascular lesions should be followed by preventive therapy. In patients with neurologic symptoms and negative MRI/MRA findings Positron Emission Tomography or single photon emission CT is recommended. There are no specific neuroimaging findings that suggest that blood transfusions can be safely halted in children with SCD.