Stroke is a rare cause of childhood morbidity and mortality. Vascular emergencies result from disruption of the arterial supply or venous return leading to energy failure and cell death. Acute development of a focal neurologic deficit is the hallmark of clinical presentation, although age and comorbid clinical findings may obscure the diagnosis. Children have higher rates of acute seizures and headaches complicating stroke presentation. The risk factors promoting ischemia and hemorrhage vary in the fetus, neonate, child, and adolescent, making the diagnostic workup challenging and broad in these diseases. Evidence supporting hyperacute treatment of vascular emergencies in children remains incomplete, with uncertainties about the safety and implementation of strategies used in adults with similar pathology. Throughout this disease, the affected child requires expertise from child neurologists familiar with stroke management and the resulting complications of developmental delay, cerebral palsy, epilepsy, cognitive difficulties, and neurobehavioral symptoms.
While the overall rate of stroke in neonates and children is low, high-risk intervals and populations are well defined. Among infants born at term, the risk of ischemic stroke is estimated at 1 in 3500 live births, with an overall stroke rate, including hemorrhage, of 1 in 2700 live births.1 Throughout life, the 2 highest single-week risks for stroke are at the time of delivery—for both the mother and child.2 Older infants and children have a lower incidence of stroke, with estimates ranging from 1.3 to 5.4 per 100,000 children.3 A population-based study indicates the rate of incident stroke has been stable over the past 2 decades.3
Unlike adults, in whom stroke is strongly skewed toward ischemic disease (~85%), pediatric cerebrovascular presentation is equally ischemic and hemorrhagic, the latter associated with congenital vascular malformations that are symptomatic in childhood.4 Stroke is more common in males and is found in higher rates in Black and Asian children in the United States.4 The highest-risk group is children with sickle cell disease—disproportionately affecting Black families—with a rate of 761 cases of stroke per 100,000 persons.5 Fortunately, case rates for sickle cell disease–related stroke are falling in developed countries that have widespread screening and prevention strategies in place.6 A similar finding has thus far not been observed in sub-Saharan Africa, where multiple studies have reported the problems of screening and treatment allocation.7,8
ETIOLOGIES OF CHILDHOOD STROKE
The causes of ischemic and hemorrhagic stroke are many. A comprehensive review of risk must consider the age, genetic predeterminants, exposures, comorbid diagnoses, and medical condition at the onset of stroke (Table 32–1).9,10 Note significant overlap between risk factors for arterial, venous, and hemorrhagic stroke subtypes, suggesting a child may be at risk for multiple events. Arterial ischemic stroke is most highly associated with arteriopathy, which is also a predictor of stroke recurrence, and cardiac disease.9,...