Childhood arterial ischemic stroke (AIS) is a rare, but serious, medical condition affecting children (age range, 29 days to 18 years), which is associated with high morbidity. The overall annual incidence is estimated at 1.2 to 8 per 100,000 children, however, it is likely that this is an understimate, as the index of suspicion for AIS is typically low in the pediatric population and data contributing to incidence estimates rely mainly on retrospective studies.1–3
Although risk factors for AIS in the adult population have been well described, there is still a limited understanding of all the risk factors in the pediatric population, and a large minority of cases is designated as idiopathic.
The acute and long-term outcomes related to childhood AIS are concerning, including mortality, recurrent events, and neurologic sequelae, which have been previously reviewed in detail.4,5
In the International Pediatric Stroke Study (IPSS), a multicenter, international observational study evaluating risk factors, treatment options, and outcomes, 22 of 612 children (3%) died before hospital discharge.6
The mortality rate, however, appears to increase with a recurrent event and has been reported in a prospective cohort study to be as high as 15% among patients with recurrent stroke.7
In general, the risk of recurrence is ~7 to 20% within 5 years of the initial event; however, the risk increases substantially for children with moyamoya or other arteriopathies.7–10
In a retrospective cohort, Fullerton and colleagues reported a 5-year cumulative recurrence rate of 66% in children with identified vascular abnormalities.10
Similarly, Sträter et al reported an increased risk of recurrent AIS in children with vascular abnormalities.7
A considerable number of children with AIS will also suffer from neurologic impairments. In the largest published cohort study, Goldenberg and colleagues noted that 74% of children had a neurologic deficit at the time of hospital discharge.6
This is similar to the observed rate of neurologic deficits in several studies with follow-up beyond 1 year.11–14
Childhood stroke is also associated with an increased risk of seizures, behavioral disorders, and cognitive impairments.13–15
While recent publications in the field of childhood AIS have begun to investigate the utility of prognostic indicators and the safety of therapeutic regimens, there remains an insufficient understanding of optimal therapeutic strategies to improve outcomes. In this narrative review, we discuss the current understanding of etiologies, consensus-based treatment recommendations, recent treatment data, and prognostic factors in childhood AIS. We also identify priorities for future research.