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Research ArticlePediatric Neuroimaging

Predicting Ischemic Risk Using Blood Oxygen Level–Dependent MRI in Children with Moyamoya

N. Dlamini, M. Slim, F. Kirkham, M. Shroff, P. Dirks, M. Moharir, D. MacGregor, A. Robertson, G. deVeber and W. Logan
American Journal of Neuroradiology December 2019, DOI: https://doi.org/10.3174/ajnr.A6324
N. Dlamini
aFrom the Division of Neurology (N.D., M. Slim, M.M., D.M., A.R., G.d.V., W.L.)
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  • ORCID record for N. Dlamini
M. Slim
aFrom the Division of Neurology (N.D., M. Slim, M.M., D.M., A.R., G.d.V., W.L.)
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F. Kirkham
dDevelopmental Neurosciences Unit and Biomedical Research Centre (F.K.), University College London Great Ormond Street Institute of Child Health, London, UK.
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M. Shroff
bDepartment of Pediatrics, and Departments of Diagnostic Imaging (M. Shroff)
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P. Dirks
cSurgery (P.D.), The Hospital for Sick Children, Toronto, Ontario, Canada
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M. Moharir
aFrom the Division of Neurology (N.D., M. Slim, M.M., D.M., A.R., G.d.V., W.L.)
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D. MacGregor
aFrom the Division of Neurology (N.D., M. Slim, M.M., D.M., A.R., G.d.V., W.L.)
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A. Robertson
aFrom the Division of Neurology (N.D., M. Slim, M.M., D.M., A.R., G.d.V., W.L.)
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G. deVeber
aFrom the Division of Neurology (N.D., M. Slim, M.M., D.M., A.R., G.d.V., W.L.)
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W. Logan
aFrom the Division of Neurology (N.D., M. Slim, M.M., D.M., A.R., G.d.V., W.L.)
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    Fig 1.

    Representative BOLD-CVR parametric maps demonstrating normal (positive) (A) reactivity and abnormal (negative) reactivity (B, arrow).

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    Fig 2.

    Direct adjusted survival curves for ischemic-free survival as a function of the moyamoya comorbidities (A) (HR for idiopathic moyamoya: 3.71; 95% CI, 1.1–12.8; P = .03), sex (B) (HR for males: 0.65; 95% CI, 0.19–2.23; P = .49), and age group (C) (HR for older than 8 years: 0.62; 95% CI, 0.18–2.1; P = .44). Age as a continuous variable was not predictive of ischemic events. Age is represented in years.

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    Table 1:

    Baseline clinical and demographic characteristics

    Total Sample (n = 37)
    Demographic characteristics
    Female (No.) (%)21 (56.8)
    Age at moyamoya diagnosis (median) (IQR, 25–75) (yr)10 (6.3–11.8)
    Age at initial CVR (median) (IQR, 25–75) (yr)10.7 (7.5–14.7)
    Time to follow-up (median) (IQR, 25–75) (mo)20.8 (13.7–84.1)
    Moyamoya classification
        Idiopathic14 (37.8)
        Syndromic23 (62.2)
         NF19 (24.3)
         Trisomy 21/other chromosomal disorders7 (18.9)
         Sickle cell disease5 (13.5)
         Postradiation vasculopathy2 (5.4)
    Clinical presentation
        Stroke (No.) (%)12 (32.4)
         Bilateral2 (5.4)
         Right6 (16.2)
         Left4 (10.8)
        TIA (No.) (%)8 (21.6)
        Seizure (No.) (%)3 (8)
        Headaches (No.) (%)8 (21.6)
        Asymptomatic (No.) (%)6 (16.2)
        Other (No.) (%)2 (5.4)
    Radiographic findings (No.) (%)
        Parenchymal
         Not ischemic8 (21.6)
         Watershed12 (32.4)
          Deep white matter1 (2.7)
          Cortical12 (32.4)
        Cortical3 (8.1)
        Cortical ischemic and watershed14 (37.8)
        Vascular
         Moyamoya laterality (No.) (%)
          Left8 (21.6)
          Right6 (16.2)
          Bilateral23 (62.2)
        Grade of stenosis (No.) (%)
        50%–74% Occlusion5 (13.5)
        ≥75% Occlusion32 (86.5)
    • Note:—NF1 indicates neurofibromatosis type 1.

    • View popup
    Table 2:

    Comparison of clinical and demographic characteristics based on steal statusa and ischemic events

    Steal StatusaP ValueIschemic EventsP Value
    No Steal (n = 12)Steal (n = 25)No (n = 26)Yes (n = 11)
    Age at baseline CVR (median) (IQR, 25–75) (yr)9.3 (5.6–14.5)10.8 (8.3–15).2810.9 (8.3–15)10.3 (7.2–12.5).44
    Female (No.) (%)5 (41.7)16 (64).2914 (53.9)7 (63.6).72
    Moyamoya classification (No.) (%).69.06
        Idiopathic4 (33.3)10 (40)7 (26.9)7 (63.6)
        Syndromicb8 (66.7)15 (60)19 (73.1)4 (36.4)
         NF12 (16.7)7 (28)7 (26.9)2 (18.2)
         Trisomy 21/other chromosomal disorders3 (25)4 (16)6 (23.1)1 (9.1)
         Sickle cell disease1 (8.3)4 (16)4 (15.4)1 (9.1)
         Postradiation vasculopathy2 (16.7)02 (7.7)0
    Stroke (No.) (%)4 (33.3)8 (32)111 (42.3)1 (9.1).06
    TIA (No.) (%)2 (16.7)6 (24)15 (19.2)3 (27.3).67
    Seizure (No.) (%)2 (16.7)1 (4).242 (7.7)1 (9.1)1
    Headaches (No.) (%)3 (25)5 (20)16 (23.1)2 (18.2)1
    Asymptomatic (No.) (%)2 (16.7)4 (16)13 (11.5)3 (27.3).33
    Others (No.) (%)02 (8.3).5402 (18.2).08
    Moyamoya laterality (No.) (%).77.78
        Left3 (25)5 (20)6 (23.1)2 (18.2)
        Right1 (8.3)5 (20)5 (19.2)1 (9.1)
        Bilateral8 (66.7)15 (60)15 (57.7)8 (72.7)
    • Note:—NF1 indicates neurofibromatosis type 1.

    • ↵a Steal status before ischemic event occurrence or the end of follow-up.

    • ↵b Comorbidities falling under syndromic moyamoya are shown for informative purposes only and were not included in the inferential analysis.

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Predicting Ischemic Risk Using Blood Oxygen Level–Dependent MRI in Children with Moyamoya
N. Dlamini, M. Slim, F. Kirkham, M. Shroff, P. Dirks, M. Moharir, D. MacGregor, A. Robertson, G. deVeber, W. Logan
American Journal of Neuroradiology Dec 2019, DOI: 10.3174/ajnr.A6324
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N. Dlamini, M. Slim, F. Kirkham, M. Shroff, P. Dirks, M. Moharir, D. MacGregor, A. Robertson, G. deVeber, W. Logan
Predicting Ischemic Risk Using Blood Oxygen Level–Dependent MRI in Children with Moyamoya
American Journal of Neuroradiology Dec 2019, DOI: 10.3174/ajnr.A6324

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