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Improved Turnaround Times | Median time to first decision: 12 days

Research ArticlePediatric Neuroimaging

The Bone Does Not Predict the Brain in Sturge-Weber Syndrome

R.R. Warne, O.M. Carney, G. Wang, D. Bhattacharya, W.K. Chong, S.E. Aylett and K. Mankad
American Journal of Neuroradiology July 2018, DOI: https://doi.org/10.3174/ajnr.A5722
R.R. Warne
aFrom the Department of Paediatric Neuroradiology (R.R.W., O.M.C., D.B., W.K.C., K.M.), Great Ormond Street Hospital for Children, NHS Foundation Trust, London, UK
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O.M. Carney
aFrom the Department of Paediatric Neuroradiology (R.R.W., O.M.C., D.B., W.K.C., K.M.), Great Ormond Street Hospital for Children, NHS Foundation Trust, London, UK
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G. Wang
cDepartment of Biostatistics (G.W.), University of Sydney School of Public Health, Sydney, New South Wales, Australia 2006.
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D. Bhattacharya
aFrom the Department of Paediatric Neuroradiology (R.R.W., O.M.C., D.B., W.K.C., K.M.), Great Ormond Street Hospital for Children, NHS Foundation Trust, London, UK
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W.K. Chong
aFrom the Department of Paediatric Neuroradiology (R.R.W., O.M.C., D.B., W.K.C., K.M.), Great Ormond Street Hospital for Children, NHS Foundation Trust, London, UK
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S.E. Aylett
bNeurosciences (S.E.A.), Great Ormond Street Hospital for Children and Developmental Neurosciences University College London, NHS Foundation Trust, London UK
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K. Mankad
aFrom the Department of Paediatric Neuroradiology (R.R.W., O.M.C., D.B., W.K.C., K.M.), Great Ormond Street Hospital for Children, NHS Foundation Trust, London, UK
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  • Fig 1.
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    Fig 1.

    Associated findings of SWS. A 4-month-old boy with SWS. A, Postcontrast T1WI of the orbits shows a left ocular choroidal hemangioma (clinically confirmed). B, Coronal T2WI of the brain reveals volume loss of the left hemisphere with associated accelerated myelination (arrow). C, Postcontrast axial T1WI of the brain shows an enlarged and enhancing left glomus angioma (arrow) and prominent transmedullary veins (circle).

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    Fig 2.

    Calvarial bone marrow abnormality. A 6-month-old boy with right PWS only. A, Axial T2-weighted STIR shows high-signal abnormality in the right calvaria (arrows). B, Graphic of a child with right-sided PWS. Reproduced with permission from Scio21/Bigstock.com.

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    Fig 3.

    Bilateral PWS with bilateral calvarial marrow abnormality. An 8-month-old boy with SWS. Axial T2-weighted-STIR (A) and axial T1-weighted postcontrast (B) images reveal a bilateral marrow T2 high-signal calvarial abnormality and enhancement. C, Axial T1WI postcontrast shows a left-sided temporo-occipital pial angioma. Coronal T1-weighted precontrast (D) and postcontrast (E) imaging show bilateral marrow and dural enhancement.

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    Fig 4.

    SWS with absent PWS. An 8-month-old girl with SWS. A, Axial T2-weighted STIR shows left temporo-occipital calvarial thinning with no bone marrow abnormality. B, Axial postcontrast T1WI shows a corresponding thick enhancing left hemispheric pial angioma.

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    Fig 5.

    The interpeduncular cistern sign. Upper panel, a 9-month-old boy with SWS. A and B, Postcontrast axial T1WI shows a left-sided unilateral pial angioma and a zoomed-in view of the interpeduncular cistern confirming the unilaterality (arrow). Lower panel, a 10-month-old girl with SWS. C and D, Bilateral pial angiomas with a zoomed-in view confirming the bilaterality (arrows), positive for the “warning sign of Warne-Mankad.”

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    Fig 6.

    “Warning sign of Warne-Mankad.” Bilateral interpeduncular cistern enhancement. A 10-month-old girl with SWS and bilateral PWS. A, Axial T2-weighted STIR shows bilateral hemispheric volume loss. B, Axial postcontrast T1WI shows a right-frontal and left-hemispheric pial angioma with the warning sign confirming the bilateral interpeduncular cistern enhancement (C). Coronal T2-weighted STIR (D) shows bilateral calvarial high signal and marrow enhancement (E). Coronal postcontrast FLAIR image (F) shows bilateral marrow enhancement and left-sided dural thickening.

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    Fig 7.

    Schematic illustration of the major calvarial diploic venous channel routes over the skull. Reproduced with permission from Springer Nature (Tsutsumi et al16). MMV indicates middle meningeal vein; OC, occipitocervical route; OFO, orbital part of the fronto-orbital route; OP, occipitoparietal route; PFO, pterional part of the fronto-orbital route; PFP, pteriofrontparietal route; PP, pterygoid plexus; SS, sigmoid sinus; SSS, superior sagittal sinus; TS, transverse sinus.

Tables

  • Figures
  • Intraosseous signal abnormality versus same-sided PWS

    PWSIntraosseous Signal Abnormality
    PresentAbsent
    Present10910
    Absent312
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The Bone Does Not Predict the Brain in Sturge-Weber Syndrome
R.R. Warne, O.M. Carney, G. Wang, D. Bhattacharya, W.K. Chong, S.E. Aylett, K. Mankad
American Journal of Neuroradiology Jul 2018, DOI: 10.3174/ajnr.A5722
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Cite this article
R.R. Warne, O.M. Carney, G. Wang, D. Bhattacharya, W.K. Chong, S.E. Aylett, K. Mankad
The Bone Does Not Predict the Brain in Sturge-Weber Syndrome
American Journal of Neuroradiology Jul 2018, DOI: 10.3174/ajnr.A5722

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