Large Vessel Vasculopathy in Children with Sickle Cell Disease: an MRI study of Infarct Topography and Focal Atrophy

Publication date: Available online 7 December 2016
Source:Pediatric Neurology
Author(s): Kristin P. Guilliams, Melanie E. Fields, Dustin K. Ragan, Yasheng Chen, Cihat Eldeniz, Monica L. Hulbert, Michael M. Binkley, James N. Rhodes, Joshua S. Shimony, Robert C. McKinstry, Katie Vo, Hongyu An, Jin-Moo Lee, Andria L. Ford
BackgroundLarge-vessel vasculopathy (LVV) increases stroke risk in pediatric sickle cell disease (SCD) beyond the baseline elevated stroke risk in this vulnerable population. The mechanisms underlying this added risk and its unique impact on the developing brain are not established.MethodsWe analyzed magnetic resonance imaging (MRI) and angiography (MRA) scans of 66 children with SCD and infarcts by infarct density heatmaps and Jacobian determinants, a metric utilized to delineate focal volume change, to investigate if infarct location, volume, frequency, and cerebral atrophy differed among hemispheres with and without LVV.ResultsInfarct density heatmaps demonstrated infarct "hot spots" within the deep white matter internal borderzone region in both LVV and non-LVV hemispheres, but with greater infarct density and larger infarct volumes in LVV hemispheres (2.2 mL vs 0.25 mL, p <0.001). Additional scattered cortical infarcts in the internal carotid artery territory occurred in LVV hemispheres, but were rare in non-LVV hemispheres. Jacobian determinants revealed greater atrophy in gray and white matter of the parietal lobes of LVV compared to non-LVV hemispheres.ConclusionLVV in SCD appears to increase ischemic vulnerability in the borderzone region, as demonstrated by the increased frequency and extent of infarction within deep white matter, and increase risk of focal atrophy. Scattered infarctions across the LVV-affected hemispheres suggest additional stroke etiologies of vasculopathy (i.e. thrombo-embolism) in addition to chronic hypoxia-ischemia.



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