Stroke without Cerebral Vasculopathy in Sickle Cell Disease Children: Which Causes? Which Treatment?

Sickle cell disease (SCD) is a severe hemoglobinopathy due to abnormal hemoglobin S (HbS). One of the most serious complications of SCD is cerebral vasculopathy (CV) leading to ischemic stroke in 8% of homozygous SS children without prevention strategy. Typical CV in SCD is a stenosis of one or more intracranial or cervical arteries, including internal carotid arteries, anterior cerebral arteries and middle cerebral arteries. However, several studies have reported the occurrence of stroke in SCD children in the absence of typical cerebral vasculopathy.