A 1 month old presents with a 2 week history of a red slightly raised plaque on the left forehead, periorbital area and malar cheek with a rim of vasoconstriction around it. The lesion has been thickening each day per mm. What syndrome is likely?
PHACES syndrome (posterior fossa malformations, haemangioma, aerial anomalies, cardiac anomalies and coarctation of the aoa, eye abnormalities, sternal cleft and/or supraumbilical raphe) An association between posterior fossa brain abnormalities and large facial infantile haemangiomas is now well recognized, and there is evidence that the association may occur more commonly in female infants. The facial haemangiomas most characteristically take the form of extensive plaques, that appear to occupy one or more facial dermatomes, and they may initially be mistaken for po-wine stains. They may be either unilateral or bilateral. Ulceration is common. The commonest posterior fossa abnormality is the Dandy-Walker syndrome, but other abnormalities have included arachnoid cyst, cerebellar atrophy with enlarged cisterna magna and fouh ventricle, cerebellar atrophy with vermis agenesis, and agenesis of the corpus callosum. These posterior fossa abnormalities may be reflected clinically by macrocephaly, enlarging head circumference, hemiparesis, developmental delay, or may be found on imaging in the absence of symptoms.Ipsilateral intracranial haemangiomas may also occur; they are generally asymptomatic and appear to regress spontaneously in parallel with associated extracranial lesions. These patients may also have cardiac anomalies, paicularly coarctation of the aoa, pharyngeal or laryngeal haemangioma, a wide variety of eye abnormalities, including glaucoma, and ventral developmental defects, most characteristically sternal clefting or a supraumbilical raphe.
Cutis Marmorata Telangiectatica Congenita
Kasabach Merritt Phenomenon
LUMBAR syndrome
PHACE syndrome
Naevi and vessel related disorders