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Vol. 19 - Num. 73

Clinical Reviews

Possible PHACE(S) syndrome

M.ª Inés Jiménez Castilloa, Francisco Javier Moleón Rodríguezb, Rafael Martín Masotc

aPediatra. CS Almanjayar. Granada. España.
bMIR-MFyC. CS Cartuja. Granada. España .
cMIR-Pediatría. Hospital Materno Infantil Virgen de las Nieves. Granada. España.

Correspondence: MI Jiménez. E-mail: inesjc2005@hotmail.com

Reference of this article: Jiménez Castillo MI, Moleón Rodríguez FJ, Martín Masot R. Possible PHACE(S) syndrome. Rev Pediatr Aten Primaria. 2017;19:51-6.

Published in Internet: 10-01-2017 - Visits: 7396

Abstract

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

Cerebral arterial diseases Cerebral infarction Dandy-Walker Hemangioma Skin abnormalities Stroke

 

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