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

Research ArticlePediatric NeuroimagingF

Cervical and Intracranial Arterial Anomalies in 70 Patients with PHACE Syndrome

C.P. Hess, H.J. Fullerton, D.W. Metry, B.A. Drolet, D.H. Siegel, K.I. Auguste, N. Gupta, A.N. Haggstrom, C.F. Dowd, I.J. Frieden and A.J. Barkovich
American Journal of Neuroradiology November 2010, 31 (10) 1980-1986; DOI: https://doi.org/10.3174/ajnr.A2206
C.P. Hess
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H.J. Fullerton
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D.W. Metry
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B.A. Drolet
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D.H. Siegel
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K.I. Auguste
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N. Gupta
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A.N. Haggstrom
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C.F. Dowd
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I.J. Frieden
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A.J. Barkovich
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  • Fig 1.
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    Fig 1.

    Representative arterial dysgenesis images are shown as 3D volume renderings from MRA source data, generated by using OsiriX (http://www.osirix-viewer.com/Downloads.html).32 A, Enlargement of the right ICA, MCA, and ACA (white asterisks). There is also an accessory left MCA (red arrow), and the left PCA is duplicated with an anomalous branching pattern (red asterisk). B, Proximal irregularity of a markedly enlarged left ICA (red arrows) and tortuosity of the right PCA (white asterisk). The origin of the right MCA (white arrow) is also anomalous, arising from the cavernous ICA. C, Looping course of the left M1 segment (white arrow) and enlarged left A1/A2 junction (red arrow). D, Marked dysgenesis of the cavernous and supraclinoid right ICA (red arrow) and contralateral dysgenesis of the cavernous and supraclinoid left ICA (white arrows). Note also that the right ICA is not visualized proximal to the cavernous sinus, the right PcomA supplies the right MCA, and the right MCA is markedly narrowed (white asterisks).

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    Fig 2.

    Anomalous course and/or origin. A, 3D rendering from MRA shows elongation of the right PcomA, an anomalous connection between the right P1 segment and the right MCA (red arrow). The right ICA and left PCA are also diffusely narrow (white arrow), and the right A1 segment is hypoplastic (white asterisk). B, 3D rendering from CTA shows the tortuous looped course of the supraclinoid right ICA (white arrow). Note also the presence of 2 ACAs, a separate artery of the corpus callosum, and aneurysms at the right carotid terminus and distal right A1 segment (white asterisks). Volume renderings were generated by using OsiriX.32

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    Fig 3.

    Arterial narrowing and nonvisualization. All images are shown as maximum-intensity-projection reconstructions from MRA source data. A, Submentovertex projection showing nonvisualization of the left ICA. B, AP projection shows long-segment narrowing of the entire visualized course of the right ICA (red arrow). C, AP projection shows nonvisualization of the left ICA from the distal cervical to the cavernous segments. The intradural left VA is also absent. D, AP projection shows absence of the entire left ICA and long-segment tapered narrowing and luminal irregularity of the distal cervical right ICA (red arrow).

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    Fig 4.

    Examples of segmental and pleurisegmental ICA dysgenesis. Lesions of the ICA, especially dysgenesis, narrowing, and nonvisualization, often involve the entire length of the artery (red arrows, A) or relatively long portions of the artery corresponding to developmental segments (red arrow, B). The right proximal MCA shows diminished flow-related enhancement as well (red asterisk, A). There is segmental dysgenesis of the petrous left ICA with irregular enlargement of the artery (B). Note also a markedly enlarged left ECA, which supplies a large hemangioma, and an absent right A1 segment.

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    Table 1:

    Location of arterial abnormalities

    LocationNo. (%)
    ICA53/70 (76%)
        Cervical35
        Petrous33
        Cavernous27
        Supraclinoid32
    MCA13/70 (20%)
    ACA11/70 (16%)
    PCA10/70 (14%)
    BA or VA5/70 (7%)
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    Table 2:

    Structural brain abnormalities

    LocationNo.
    Posterior fossa
        Cerebellar hypoplasia18
        Atrophy of cerebral peduncle2
    Malformations of cortical development
        Callosal dysgenesis3
        Hemispheric hypoplasia2
        Subependymal heterotopia2
        Polymicrogyria2
    Extra-axial lesions
        Intracranial hemangioma3
        Lipoma2a
    Other
        Thin corpus callosum2
        Ocular abnormality2b
        Hippocampal malrotation2
        Cranial nerve dysplasia (7/8 complex)1
        Pituitary ectopia1
    • a One interhemispheric lipoma was seen in association with callosal dysgenesis; 1 infracollicular lipoma was seen in isolation.

    • b One case each of coloboma and microphthalmia.

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American Journal of Neuroradiology: 31 (10)
American Journal of Neuroradiology
Vol. 31, Issue 10
1 Nov 2010
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Cite this article
C.P. Hess, H.J. Fullerton, D.W. Metry, B.A. Drolet, D.H. Siegel, K.I. Auguste, N. Gupta, A.N. Haggstrom, C.F. Dowd, I.J. Frieden, A.J. Barkovich
Cervical and Intracranial Arterial Anomalies in 70 Patients with PHACE Syndrome
American Journal of Neuroradiology Nov 2010, 31 (10) 1980-1986; DOI: 10.3174/ajnr.A2206

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Cervical and Intracranial Arterial Anomalies in 70 Patients with PHACE Syndrome
C.P. Hess, H.J. Fullerton, D.W. Metry, B.A. Drolet, D.H. Siegel, K.I. Auguste, N. Gupta, A.N. Haggstrom, C.F. Dowd, I.J. Frieden, A.J. Barkovich
American Journal of Neuroradiology Nov 2010, 31 (10) 1980-1986; DOI: 10.3174/ajnr.A2206
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