Detection and Management of Cerebral Vasculopathy

Abstract

Cerebral vasculopathy in children with sickle cell anemia is responsible for strokes and silent cerebral infarcts and is the most debilitating complication providing motor sequelae and cognitive deficiency. However, the most important advance in pediatric management is the detection of children at a risk of stroke using transcranial Doppler with chronic transfusion applied in children detected at risk, which reduces the stroke risk from 11% to less than 2%. In this chapter, we will describe the place of Doppler, magnetic resonance imaging (MRI), and magnetic resonance angiography (MRA) with neck assessment and the place of different treatments, i.e., chronic transfusion, hydroxyurea, new drugs, and stem cell transplantation.