Intracranial hemorrhage in children with sickle cell disease.

We treated two children with sickle cell disease and intracranial hemorrhage. The incidence of intracranial hemorrhage is increased in sickle cell disease, although not as markedly as that of cerebral infarction. Intracranial hemorrhage has a higher mortality, a lower rate of permanent neurologic impairment, and occurs more often in older patients than does cerebral infarction. Intracranial hemorrhage in adults is likely to be an intracerebral hemorrhage or a subarachnoid hemorrhage secondary to an aneurysm. Children, however, are more likely to have subarachnoid hemorrhage without an identifiable aneurysm. We hypothesize that both hemorrhages and infarcts are due to large-vessel cerebral vasculopathy secondary to the abnormal rheologic features of sickled cells.