Changing Management of Focal Cerebral Arteriopathy of Childhood From 2010 to 2022

BACKGROUND: The most common cause of arterial ischemic stroke in healthy children, focal cerebral arteriopathy (FCA), can progress rapidly over days with worsening brain injury. A 2017 retrospective Swiss study of corticosteroid treatment for FCA changed practice. To assess its impact, we compared the FCA cohorts from the 2 VIPS (Vascular Effects of Infection in Pediatric Stroke) prospective cohort studies. METHODS: The VIPS II study prospectively enrolled 205 children (29 days to 18 years) with arterial ischemic stroke at 22 centers, December 2016 to January 2022. The local team measured 12-month outcomes using the pediatric stroke outcome measure. A neuroradiologist and pediatric vascular neurologist independently reviewed all clinically obtained imaging and clinical data to classify the cause of arterial ischemic stroke. The neuroradiologist measured the FCA Severity Score on vascular imaging performed at any time poststroke. We compared the VIPS II FCA cohort to the previously published FCA cohort from VIPS I (2010-2014; 37 centers). RESULTS: Of 75 children with definite arteriopathy enrolled in VIPS II, 32 (43%) had FCA, compared with 41 of 127 (32%) of definite arteriopathy cases in VIPS I. The median age was 11.3 years (56% male) in VIPS I and 11.4 years (55%) in VIPS II. Treatment with intravenous corticosteroids increased from 2 of 41 (5%) of FCA patients in VIPS I to 18 of 32 (56%) in VIPS II. The VIPS II FCA cases were more severe at baseline (median FCA Severity Score 6 versus 4; P=0.006). There were no significant differences in either the change in FCA Severity Score (baseline to maximum) or the 12-month neurological outcomes. CONCLUSIONS: Treatment of FCA with corticosteroids increased dramatically between the VIPS I and VIPS II studies. VIPS II cases were more severe at baseline, but we observed no significant difference in disease progression or neurological outcomes. Given the low level of evidence supporting corticosteroid therapy, pediatric stroke centers should enroll FCA patients into ongoing FCA corticosteroid treatment trials.