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Intracranial Infantile Hemangiomas Associated With PHACE Syndrome

C.D. Judda, P.R. Chapmana, B. Kochb and C.J. Sheac

a Inland Imaging, Spokane, Wash
b Children’s Hospital Medical Center, Cincinnati, Ohio
c Northwest Pediatric Ophthalmology, Spokane, Wash


Figure 1
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  		Fig 1. Six-week-old girl with right periorbital and IAC/CPA hemangiomas.

A, Axial T1 postcontrast image with fat suppression at presentation showing enhancing right orbital and IAC/CPA masses, relative hypoplasia of the right internal carotid artery (arrowhead), and subtle right cerebellar hypoplasia.

B, Coronal T1 postcontrast image with fat suppression at the time of presentation demonstrating the enhancing right CPA mass with a small number of flow voids.

C, D, Axial and coronal T1 postcontrast, fat-suppressed images obtained 2 months following steroid therapy with significant involution of the right IAC/CPA mass (arrowhead, D).


Figure 2
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  		Fig 2. Eight-week-old girl with large left periorbital hemangioma extending into left cavernous sinus, Meckel cave, and the left IAC.

A, B, Axial T1 postcontrast, fat-suppressed images at presentation showing a left orbital mass and fullness of the left cavernous sinus (A), as well as a cone-shaped enhancing mass in the left IAC (B). Also evident are left cerebellar hypoplasia and relative left internal carotid hypoplasia (arrowhead, A).

C, Coronal T2 image at presentation with isointense soft-tissue mass enlarging Meckel cave on the left.

D, Axial T1 postcontrast image at 1 year follow-up showing nearly complete involution of the left IAC mass.

E, Coronal T1 postcontrast image in the same plane as panel C at 1-year follow-up showing an enlarged Meckel cave and resolution of the soft tissue mass.


Figure 3
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  		Fig 3. Three-week-old girl with large right periorbital and IAC/CPA hemangiomas.

A, Axial postcontrast CT at presentation showing right orbital enhancing mass with large, isoattenuated enhancing mass in the ipsilateral IAC/CPA.

B, Axial T1 postcontrast image with fat suppression at 3-month follow-up showing moderate involution of the right CPA/IAC enhancing mass. Note marked involution of right periorbital tissue.


Figure 4
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  		Fig 4. Three-week-old girl with multiple superficial hemangiomas involving the right scalp, orbit, and pinna, as well as a large right IAC/CPA hemangioma and small fourth ventricle hemangioma.

A, Axial T1 postcontrast image with fat suppression at presentation showing right IAC/CPA enhancing mass. Note ipsilateral internal carotid hypoplasia and linear enhancement in fourth ventricle.

B, Axial T1 postcontrast image with fat suppression 3 months following steroid therapy showing significant involution of the IAC/CPA mass.





Copyright © 2008 by the American Society of Neuroradiology. Print ISSN: 0195-6108 Online ISSN: 1936-959X