Arteriopathy influences pediatric ischemic stroke presentation, but sickle cell disease influences stroke management

Purpose: Sickle cell disease (SCD) and arteriopathy are pediatric stroke risk factors that are not mutually exclusive. The relative contribution of SCD and arteriopathy to stroke risk is unknown, resulting in unclear guidance for management when both risk factors are present. We hypothesized that despite similarities in presentation and arteriopathy, stroke management differs in children with SCD.Methods: We compared presentation and management of children with SCD enrolled in the International Pediatric Stroke Study to non-SCD, non-cardiac children, according to SCD and arteriopathy (A) status. Regression modeling determined relative contribution of SCD and arteriopathy in variables with significant differences in frequency.Results: Among 930 childhood arterial ischemic strokes, there were 98 children with SCD, 67 of whom had arteriopathy, and 466 without SCD, 392 of whom had arteriopathy. Arteriopathy, regardless of SCD status, increased likelihood of hemiparesis (OR 1.94 95% confidence intervals [CI] 1.46, 2.56) and speech abnormalities (OR 1.67; CI 1.29, 2.19). Arteriopathy also increased likelihood of headache, but only among those without SCD (OR 1.89 CI 1.40, 2.55). Echocardiograms were less frequently obtained in children with SCD (OR 0.58; CI 0.37, 0.93), but the frequency of abnormalities among those with echocardiography results were similar in both groups (p=0.57). Children with SCD were less likely to receive antithrombotic therapy, even in the presence of arteriopathy (OR 0.14; CI 0.08, 0.22). Arteriopathy was associated with a significantly higher likelihood of antithrombotic therapy in children without SCD (OR 5.36; CI 3.55, 8.09).Conclusion: Arteriopathy, and not SCD status, was most influential of stroke presentation. However, SCD status influenced stroke management, as children with SCD were less likely to have echocardiograms or receive antithrombotic therapy. Further work is needed to determine whether management differences are warranted.