Phace Syndrome in Children: Two Case Reports

PHACE(S) syndrome combines: posterior fossa brain malformations, face hemangioma, arterial cerebrovascular abnormalities, cardiovascular abnormalities, eye abnormalities, and ventral developmental defects (Sternal defects or supra-umbilical rope). The diagnosis is based on the association of an infant hemangioma exceeding 5cm in size on the face, neck, scalp with 1 major criteria or 2 minor criteria. Imaging, especially Gadolinium MRI and MRA of the brain, neck, and aortic arch, transthoracic echocardiography, or even cardiac MRI play a key role in the detection of associated neurological and cardiovascular abnormalities. We report 2 cases of PHACE syndrome revealed by 2 different clinical presentations.