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  • Title: [PHACES syndrome].
    Author: Morcillo Azcárate J, Bernabeu-Wittel J, Fernández-Pineda I, Conejo-Mir MD, Tuduri Limousin I, Aspiazu Salinas DA, de Agustín Asensio JC.
    Journal: Cir Pediatr; 2010 Apr; 23(2):92-4. PubMed ID: 21298917.
    Abstract:
    INTRODUCTION: PHACES syndrome associates a segmental facial hemangioma with cerebral malformations, aortic branches/cranial arteries anomalies, cardiac defects, eye anomalies or ventral wall defects. The aim of this study is to analyze our experience with this syndrome. MATERIAL AND METHODS: Retrospective study of the cases seen at our unit in the last year. RESULTS: We treat 4 cases; 3 girls and 1 child. Besides the segmental hemangioma they presented: 3 vascular cerebral malformations; 2 structural cardiopathies; 2 cerebral malformations, 1 microftalmia. We did not find ventral wall defects. A case received treatment with two cycles of metilprednisolone i.v. and oral prednisone, with favourable course; two cases received initial treatment with oral prednisone continued of oral propanolol in rising pattern up to 2 mg/kg/day, Obtaining both the detention of the tumour growth and regression of the lesion, with very good tolerance. A 7-year-old patient has been treated with colouring pulse laser for her residual lesions. CONCLUSIONS: When we see a segmental facial hemangioma we must perform a wide diagnostic study in order to discard a PHACES syndrome. Multidisciplinar approach to the patient by a wide expert's group gets an earlier diagnose and improves the outcome. Propranolol is a promising therapeutic alternative.
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