Stroke is one of the major complications in children with
sickle cell disease (SCD).
Ischemic stroke is associated with small asymptomatic subcortical
infarcts to large territorial lesions causing major disability.
Intracranial hemorrhages may be caused by
aneurysm rupture or by leakage from moyamoya vessels or venous sources. There have been no
acute stroke treatment studies in SCD, but hydration and exchange transfusion are often recommended. However, there is an evidence base for primary and to some extent secondary
stroke prevention. Primary prevention of
stroke was demonstrated in the
Stroke Prevention Trial in
Sickle Cell Anemia (STOP), in which children with transcranial Doppler (TCD) mean blood flow velocities of 200 cm/second (previously shown to indicate high
stroke risk) or higher were randomized to either regular
blood transfusions or no transfusion. The study showed a very significant 90% reduction in first
stroke with transfusion. In STOP2, discontinuing transfusions after 30 months or more (even with normal TCD) resulted in a high rate of reversion to abnormal TCD values and
stroke. TCD screening of all children with SCD, and initiation and maintenance of chronic transfusion to maintain
hemoglobin S below 30% in the high-risk group, is the only proven prevention strategy for
stroke in SCD.
Hydroxyurea is being studied as secondary
stroke prevention at this time. No recommendation specific to SCD regarding the use of
antiplatelet agents or
anticoagulants in
ischemic stroke can be made.
Bone marrow transplantation can be curative for SCD, and limited data support its use to prevent
stroke in SCD.