Stroke in sickle cell disease

Sickle cell disease (SCD) is the second commonest cause of stroke in the pediatric population. Cerebrovascular complications in SCD may involve large or small vessels and present as large infarctions, intracranial or subarachnoid hemorrhage, moyamoya disease or silent infarcts. Predictors of stroke in SCD include high blood flow velocity on transcranial Doppler, low hemoglobin value, absence of alpha-thalassemia, high white cell count, hypertension, silent brain infarction, and a history of chest crisis. Regular blood transfusions are used for primary and secondary prevention of stroke in SCD. Other management strategies include stem cell transplantation, and surgery for moyamoya disease.