AJDRAJNR - American Journal of Neuroradiology

Published ahead of print on January 25, 2008
doi: 10.3174/ajnr.A0937
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PHACES Association: A Neuroradiologic Review of 17 Patients

V.S. Ozaa, E. Wangb, A. Berensteine,f, M. Wanere,f, D. Leftone,f, J. Wellsd and F. Bleia,c

a Department of Pediatrics, New York University School of Medicine, New York, NY
b Department of Radiology, New York University School of Medicine, New York, NY
c Department of Surgery (Plastic), New York University School of Medicine, New York, NY
d Department of Neurology, New York University School of Medicine, New York, NY
e Vascular and Birthmarks Institute of New York, Roosevelt Hospital, New York, NY
f Albert Einstein School of Medicine, Yeshiva University, New York, NY


Figure 1
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  		Fig 1. A, Eight initial embryonic developmental patterns are identified. B, Final segmental patterns are extracted from image analysis: S1 (frontotemporal), S2 (maxillary), S3 (mandibular), S4 (frontonasal). Reproduced with permission from J Pediatr 139:117-23, copyright 2001 by the AAP.


Figure 2
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  		Fig 2. A, Color photograph of patient 10, illustrating a hemangioma in an S3 distribution. Note additional thoracic and supraumbilical raphe. B, Color photograph of patient 1 with S1-S4 cutaneous distribution.


Figure 3
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  		Fig 3. A, Axial T1-weighted image of the brain demonstrates decreased size of the left hemisphere and abnormally thickened left frontoparietal cortex (arrowheads). Also demonstrated is an abnormal pattern of sulcation with thickened gyri. B, Coronal T2-weighted image of the brain demonstrates prominent thickening of the left temporoparietal cortex with poor gray-white discrimination. Well-defined gray-white differentiation on the right (arrows) is marked for normal comparison.


Figure 4
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  		Fig 4. A, Coronal maximum-intensity-projection reconstruction from intracranial time-of-flight MRA demonstrates an ectatic and tortuous appearance of the left precavernous and cavernous ICA (arrow). B, 3D MRA rendering from NOVA with flow measurement through the cervical left ICA. The left ICA at the skull base is markedly irregular and tortuous.


Figure 5
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  		Fig 5. Postcontrast coronal T1-weighted image of the brain again demonstrates the abnormally enhancing mass (arrow) just below right-sided portions of the hypothalamus. Vague surface enhancement along the right temporal lobe is noted (arrowheads).





Copyright © 2009 by the American Society of Neuroradiology. Print ISSN: 0195-6108 Online ISSN: 1936-959X