A Phaces Syndrome Unmasked by Propranolol Interruption in a Tetralogy of Fallot Patient: Case Report and Extensive Review on New Indications of Beta Blockers

ISSN: 1875-533X (Online)
ISSN: 0929-8673 (Print)


Volume 21, 38 Issues, 2014


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Current Medicinal Chemistry

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A Phaces Syndrome Unmasked by Propranolol Interruption in a Tetralogy of Fallot Patient: Case Report and Extensive Review on New Indications of Beta Blockers

Author(s): Gabriele Bronzetti, Annalisa Patrizi, Federica Giacomini, Francesco Savoia, Beatrice Raone, Maurizio Brighenti, Marco Bonvicini, Iria Neri and Gaetano D. Gargiulo

Affiliation: Clinica Dermatologica, via Massarenti 1, 40138, Bologna, Italy

Abstract

Infantile hemangiomas (IHs) are the most common benign tumors of infancy and usually they don't require specific therapy. In 10-20% of cases IHs are able to generate complication and medical/surgical intervention is needed. For many decades standard treatment consisted in oral or intralesional corticosteroids untill Lèautë-Labrëze and colleagues published the first report on the efficacy of propranolol for cutaneous infantile hemangiomas in 2008. IHs can be sometimes part of complex syndrome. Here we report the case of a patient with tetralogy of Fallot operated at 5 month of age who stopped propranolol treatment for hypoxic spells and unusually developed facial and subglottic IHs configuring the diagnosis of PHACES syndrome (posterior fossa brain malformations, hemangioma, arterial anomalies, cardiac defects and/or aortic coarctation, ocular anomalies and sternal defects). To our knowledge this is the first first report in the international literature of a delayed appearance of an infantile hemangioma involving the skin and the airways (PHACES syndrome). The pathophysiological explanation relies in the mechanism of action of propranolol which seems to act initially with vasoconstriction, down-regulating proangiogenetic factors and inducing endothelial cell apoptosis. Many decades since their introduction β-blockers are useful in a growing group of diseases. The pleiotropic effect of β-adrenoceptors antagonists is not yet deeply understood, residing in neurohormonal regulation systems and angiogenesis and proving to be an effective treatment from cardiovascular to oncological illness

Keywords: Airways hemangioma, beard hemangioma, beta blockers, PHACES syndrome, propranolol, subglottic hemangioma

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Article Details

Volume: 21
First Page: 1
Last Page: 16
Page Count: 16
DOI: 10.2174/0929867321666140304094345
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