Risk for PHACE Syndrome in Infants With Large Facial Hemangiomas
Por:
Haggstrom, AN, Garzon, MC, Baselga, E, Chamlin, SL, Frieden, IJ, Holland, K, Maguiness, S, Mancini, AJ, McCuaig, C, Metry, DW, Morel, K, Powell, J, Perkins, SM, Siegel, D, Drolet, BA
Publicada:
1 ago 2010
Resumen:
OBJECTIVES: This study was conducted to determine the prevalence of posterior fossae of the brain, arterial anomalies, cardiac anomalies, and eye anomalies (PHACE) in infants with large facial hemangiomas. The extracutaneous manifestations of PHACE may be associated with significant morbidity, and the prevalence of PHACE in patients with facial hemangiomas has not previously been reported.
METHODS: A multicenter prospective study was conducted with 108 infants who had large facial hemangiomas and were systematically evaluated for manifestations of PHACE. The prevalence of PHACE and its extracutaneous manifestations in this cohort was calculated. The relationship between hemangioma distribution and the manifestations of PHACE was analyzed.
RESULTS: Thirty-three (31%) of 108 had PHACE. Thirty of the 33 patients with PHACE had >1 extracutaneous finding. The risk for PHACE syndrome was higher in infants with larger hemangiomas and in those with hemangiomas that encompassed >1 facial segment. The most common extracutaneous anomalies observed in infants with PHACE were of the arteries of the cerebrovasculature (91%) and cardiac anomalies (67%). Upper face (frontotemporal and frontonasal) hemangiomas were commonly observed in infants with PHACE; isolated maxillary hemangiomas were rarely associated with PHACE.
CONCLUSIONS: In infants with large facial hemangiomas, one-third have extracutaneous manifestations consistent with the diagnosis of PHACE syndrome, most commonly cerebrovascular and cardiovascular anomalies. The high prevalence of arterial anomalies in this cohort has implications for clinical management and future research regarding the pathophysiology of PHACE. Pediatrics 2010; 126: e418-e426
Filiaciones:
Haggstrom, AN:
Indiana Univ, Dept Dermatol, Indianapolis, IN 46204 USA
Indiana Univ, Dept Pediat, Indianapolis, IN 46204 USA
Garzon, MC:
Columbia Univ, Dept Dermatol, New York, NY 10027 USA
Columbia Univ, Dept Pediat, New York, NY 10027 USA
Baselga, E:
Hosp Santa Creu I Sant Pau, Dept Dermatol, Barcelona, Spain
Chamlin, SL:
Northwestern Univ, Feinberg Sch Med, Dept Pediat, Evanston, IL USA
Northwestern Univ, Feinberg Sch Med, Dept Dermatol, Evanston, IL USA
Frieden, IJ:
Univ Calif San Francisco, Dept Dermatol, San Francisco, CA 94143 USA
Holland, K:
Med Coll Wisconsin, Dept Dermatol, Milwaukee, WI 53226 USA
Maguiness, S:
Univ Calif San Francisco, Dept Dermatol, San Francisco, CA 94143 USA
Kaiser Permanente, San Francisco, CA USA
Mancini, AJ:
Northwestern Univ, Feinberg Sch Med, Dept Pediat, Evanston, IL USA
Northwestern Univ, Feinberg Sch Med, Dept Dermatol, Evanston, IL USA
McCuaig, C:
Univ Montreal, Dept Pediat, Div Dermatol, CHU St Justine, Montreal, PQ H3C 3J7, Canada
Metry, DW:
Baylor Coll Med, Dept Dermatol, Houston, TX 77030 USA
Morel, K:
Columbia Univ, Dept Dermatol, New York, NY 10027 USA
Columbia Univ, Dept Pediat, New York, NY 10027 USA
Powell, J:
Univ Montreal, Dept Pediat, Div Dermatol, CHU St Justine, Montreal, PQ H3C 3J7, Canada
Perkins, SM:
Indiana Univ, Dept Med, Div Biostat, Indianapolis, IN 46204 USA
Siegel, D:
Oregon Hlth & Sci Univ, Dept Dermatol, Portland, OR 97201 USA
Drolet, BA:
Med Coll Wisconsin, Dept Dermatol, Milwaukee, WI 53226 USA
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