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.