Large-Vessel Vasculopathy in Children With Sickle Cell Disease: A Magnetic Resonance Imaging Study of Infarct Topography and Focal Atrophy. Pediatr Neurol 2017 Apr;69:49-57
Date
02/06/2017Pubmed ID
28159432Pubmed Central ID
PMC5365370DOI
10.1016/j.pediatrneurol.2016.11.005Scopus ID
2-s2.0-85011074013 (requires institutional sign-in at Scopus site) 37 CitationsAbstract
BACKGROUND: Large-vessel vasculopathy (LVV) increases stroke risk in pediatric sickle cell disease 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.
METHODS: We analyzed magnetic resonance imaging and angiography scans of 66 children with sickle cell disease 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.
RESULTS: Infarct density heatmaps demonstrated infarct "hot spots" within the deep white matter internal border zone region in both LVV and non-LVV hemispheres, but with greater infarct density and larger infarct volumes in LVV hemispheres (2.2 mL versus 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 with non-LVV hemispheres.
CONCLUSION: Large-vessel vasculopathy in sickle cell disease appears to increase ischemic vulnerability in the borderzone region, as demonstrated by the increased frequency and extent of infarction within deep white matter, and increased risk of focal atrophy. Scattered infarctions across the LVV-affected hemispheres suggest additional stroke etiologies of vasculopathy (i.e., thromboembolism) in addition to chronic hypoxia-ischemia.
Author List
Guilliams KP, Fields ME, Ragan DK, Chen Y, Eldeniz C, Hulbert ML, Binkley MM, Rhodes JN, Shimony JS, McKinstry RC, Vo KD, An H, Lee JM, Ford ALAuthor
Dustin K. Ragan PhD Assistant Professor in the Radiology department at Medical College of WisconsinMESH terms used to index this publication - Major topics in bold
AdolescentAnemia, Sickle Cell
Atrophy
Brain
Cerebral Angiography
Cerebral Infarction
Child
Child, Preschool
Cross-Sectional Studies
Female
Humans
Magnetic Resonance Imaging
Male
Retrospective Studies
Young Adult
