Large-Vessel Vasculopathy in Children With Sickle Cell Disease: A Magnetic Resonance Imaging Study of Infarct Topography and Focal Atrophy.
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.



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.



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.



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.
Department of Neurology, Washington University School of Medicine, Saint Louis, Missouri; Department of Pediatrics, Washington University School of Medicine, Saint Louis, Missouri.
Kristin P
KP
Guilliams
Department of Pediatrics, Washington University School of Medicine, Saint Louis, Missouri.
Melanie E
ME
Fields
Department of Neurology, Washington University School of Medicine, Saint Louis, Missouri.
Dustin K
DK
Ragan
Department of Neurology, Washington University School of Medicine, Saint Louis, Missouri.
Yasheng
Y
Chen
Department of Radiology, Washington University School of Medicine, Saint Louis, Missouri.
Cihat
C
Eldeniz
Department of Pediatrics, Washington University School of Medicine, Saint Louis, Missouri.
Monica L
ML
Hulbert
Department of Neurology, Washington University School of Medicine, Saint Louis, Missouri.
Michael M
MM
Binkley
Department of Neurology, Washington University School of Medicine, Saint Louis, Missouri.
James N
JN
Rhodes
Department of Pediatrics, Washington University School of Medicine, Saint Louis, Missouri; Department of Radiology, Washington University School of Medicine, Saint Louis, Missouri.
Joshua S
JS
Shimony
Department of Pediatrics, Washington University School of Medicine, Saint Louis, Missouri; Department of Radiology, Washington University School of Medicine, Saint Louis, Missouri.
Robert C
RC
McKinstry
Department of Radiology, Washington University School of Medicine, Saint Louis, Missouri.
Katie D
KD
Vo
Department of Radiology, Washington University School of Medicine, Saint Louis, Missouri.
Hongyu
H
An
Department of Neurology, Washington University School of Medicine, Saint Louis, Missouri; Department of Radiology, Washington University School of Medicine, Saint Louis, Missouri. Electronic address: leejm@wustl.edu.
Jin-Moo
JM
Lee
Department of Neurology, Washington University School of Medicine, Saint Louis, Missouri. Electronic address: forda@wustl.edu.
Andria L
AL
Ford
D000293:Adolescent; D000755:Anemia, Sickle Cell / Q000150:complications* / Q000000981:diagnostic imaging*; D001284:Atrophy / Q000150:complications / Q000000981:diagnostic imaging; D001921:Brain / Q000000981:diagnostic imaging; D002533:Cerebral Angiography; D002544:Cerebral Infarction / Q000150:complications* / Q000000981:diagnostic imaging*; D002648:Child; D002675:Child, Preschool; D003430:Cross-Sectional Studies; D005260:Female; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D012189:Retrospective Studies; D055815:Young Adult
MRI; atrophy; heatmap; infarction; sickle cell; stroke; vasculopathy