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 until Leaute-Labreze 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 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 on 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 illnesses.
Keywords: Airways hemangioma, beard hemangioma, beta blockers, PHACES syndrome, propranolol, subglottic hemangioma.
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