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PHACE Syndrome:  The Association of Posterior Fossa Brain Malformations, Hemangiomas, Arterial Anomalies, Coarctation of the Aorta and Cardiac Defects, and Eye Abnormalities

Ilona J. Frieden, MD; Vail Reese, MD; Debra Cohen, MD
Arch Dermatol. 1996;132(3):307-311. doi:10.1001/archderm.1996.03890270083012.
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Background:  Large facial hemangiomas can have associated central nervous system malformations, particularly the Dandy-Walker posterior fossa malformations. Abnormal arteries, especially those of the central nervous system, coarctation of the aorta, cardiac defects, and unusual ophthalmologic abnormalities can also occur.

Observations:  We describe two patients with large facial hemangiomas, congenital cataracts, and structural arterial abnormalities, particularly of the central nervous system vasculature. One of these infants also had a Dandy-Walker malformation detected on prenatal ultrasound at 12 weeks' gestation, suggesting that this syndrome had its origin during the first trimester of pregnancy. This infant also had a lingual thyroid and developed symptomatic hypothyroidism, possibly induced by interferon alfa therapy of her hemangioma. These cases are discussed, along with 41 previously reported cases with similar findings.

Conclusions:  Large facial hemangiomas may have a distinctive group of associated arterial, central nervous system, and ophthalmologic anomalies. We propose the acronym PHACE syndrome to emphasize the characteristic findings of this neurocutaneous syndrome: posterior fossa malformations, hemangiomas, arterial anomalies, coarctation of the aorta and cardiac defects, and eye abnormalities.(Arch Dermatol. 1996;132:307-311)

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