Abstract

JIMENEZ CASTILLO, MI; MOLEON RODRIGUEZ, FJ  and  MARTIN MASOT, R. Possible PHACE(S) syndrome. Rev Pediatr Aten Primaria [online]. 2017, vol.19, n.73, pp.51-56. ISSN 1139-7632.

Infantile hemangiomas are a frequent reason for consultation in Pediatrics. When extensive cutaneous hemangiomas are observed and specially if they affect the face, we we should suspect a neurocutaneous disorder called PHACE(S) syndrome. Diagnosis must be based on clinical criteria in order to evaluate and treat possible complications that sometimes can be lethal. A proper management of the patients requires a multidisciplinary intervention, with the purpose of reducing stress and preventing the ocurrence of complications of the hemangioma and the disorders associated to the syndrome.Beta-blockers are employed as first-line treatment of cutaneous hemangiomas, but in these patients a prior screening of possible cerebrovascular and cardiac malformations is needed. Endocrine alterations are rare, so a high diagnostic suspicion is required to avoid going unnoticed.

Keywords : Skin abnormalities; Stroke; Cerebral arterial diseases; Cerebral infarction; Dandy-Walker; Hemangioma.

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