Published online March 1, 2006
PEDIATRICS Vol. 117 No. 3 March 2006, pp. 959-964 (doi:10.1542/peds.2005-1683)

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EXPERIENCE AND REASON

Early Stroke and Cerebral Vasculopathy in Children With Facial Hemangiomas and PHACE Association

Beth A. Drolet, MD^a, Magdalene Dohil, MD^b, Meredith R. Golomb, MD^c, Robert Wells, MD^a, Luann Murowski, DO^a, Joan Tamburro, DO^d, Jack Sty, MD^a and Sheila Fallon Friedlander, MD^b

^a Departments of Pediatrics and Dermatology, Medical College of Wisconsin, and Department of Radiology, Children’s Hospital of Wisconsin, Milwaukee, Wisconsin
^b Department of Pediatrics and Dermatology, University of California, San Diego California
^c Department of Neurology, Division of Pediatric Neurology
^d Department of Dermatology, Indiana University School of Medicine, Indianapolis, Indiana

PHACE association is a rare neurocutaneous syndrome that may include posterior fossa malformations, hemangiomas, arterial anomalies, coarctation of the aorta and cardiac defects, eye abnormalities, and sternal defects. The arterial abnormalities may be developmental or acquired and usually involve the cervical and cerebral vasculature. We believe that infants with PHACE association are at increased risk of arterial ischemic stroke and describe 5 infants with facial hemangiomas and structural and acquired arterial anomalies, all of whom suffered an arterial ischemic stroke during infancy.

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Key Words: hemangioma • stroke • PHACE • vasculopathy

Abbreviations: AIS, arterial ischemic stroke • MRA, magnetic resonance angiography

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Accepted Jul 27, 2005.

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