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Research ArticleExtracranial Vascular

Cervicofacial Venous Malformations Are Associated with Intracranial Developmental Venous Anomalies and Dural Venous Sinus Abnormalities

W. Brinjikji, I.T. Mark, V.M. Silvera and J.B. Guerin
American Journal of Neuroradiology July 2020, 41 (7) 1209-1214; DOI: https://doi.org/10.3174/ajnr.A6617
W. Brinjikji
aFrom the Departments of Radiology (W.B., I.T.M., V.M.S., J.B.G.)
bNeurosurgery (W.B.), Mayo Clinic, Rochester, Minnesota.
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I.T. Mark
aFrom the Departments of Radiology (W.B., I.T.M., V.M.S., J.B.G.)
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V.M. Silvera
aFrom the Departments of Radiology (W.B., I.T.M., V.M.S., J.B.G.)
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J.B. Guerin
aFrom the Departments of Radiology (W.B., I.T.M., V.M.S., J.B.G.)
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    FIG 1.

    Demonstration of the metameric distribution of somatic mutations affecting venous endothelial progenitor cells that could presumably affect the cerebral venous system if they occur before cell migration. Reprinted with permission of the Mayo Foundation for Medical Education and Research. All rights reserved.

  • FIG 2.
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    FIG 2.

    A 38-year-old man with a history of large trans-spatial cervicofacial venous malformation causing periodic airway obstruction. A, MRV shows a large and ectatic torcula and medial right transverse sinus. B, Postcontrast T1 MR imaging of the face shows an extensive venous malformation predominantly involving the right face, tongue, and hypopharynx. C, Postcontrast MR imaging shows a DVA in the right cerebellum.

  • FIG 3.
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    FIG 3.

    A 5-year-old child with history of right orbital venous malformation. A, T2 with fat saturation MR imaging shows a large venous malformation involving the right orbit and face. B, MRV shows a markedly ectatic torcula, which includes the entirety of the straight sinus. C–E, T1 postcontrast MRIs show multiple developmental venous anomalies, including the bilateral cerebellar hemispheres, brain stem, right temporal lobe, and right frontal lobe.

  • FIG 4.
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    FIG 4.

    A 38-year-old man with extensive airway malformation. A, T2 FS MR imaging shows an extensive venous malformation involving the airway and tongue base. B, Axial postcontrast MR imaging shows extensive DVAs of the right frontal lobe, basal ganglia, and temporal lobe. C, 7T SWI shows the extensive DVAs and multiple cavernomas in the radicles of the DVAs.

  • FIG 5.
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    FIG 5.

    A 45-year-old man with developmental delay and bilateral facial VMs (not shown). A, Sagittal T1 MR imaging shows an ectatic torcula, which is also confirmed on axial T1 postcontrast MR imaging (B). C and D, Axial postcontrast MR imaging shows multiple extensive DVAs involving the left cerebral hemisphere. The patient also had polymicrogyria of the right hemisphere.

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American Journal of Neuroradiology: 41 (7)
American Journal of Neuroradiology
Vol. 41, Issue 7
1 Jul 2020
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Cite this article
W. Brinjikji, I.T. Mark, V.M. Silvera, J.B. Guerin
Cervicofacial Venous Malformations Are Associated with Intracranial Developmental Venous Anomalies and Dural Venous Sinus Abnormalities
American Journal of Neuroradiology Jul 2020, 41 (7) 1209-1214; DOI: 10.3174/ajnr.A6617

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Cervicofacial Venous Malformations Are Associated with Intracranial Developmental Venous Anomalies and Dural Venous Sinus Abnormalities
W. Brinjikji, I.T. Mark, V.M. Silvera, J.B. Guerin
American Journal of Neuroradiology Jul 2020, 41 (7) 1209-1214; DOI: 10.3174/ajnr.A6617
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