Syndrome characterized by a naevus flammeus in the distribution of the first branch of the trigeminal nerve associated with a vascular malformation of the ipsilateral meninges and cerebral cortex. Ocular involvement including hemianopia and glaucoma may also occur, but it is not essential for this diagnosis. The usual cutaneous finding is a port wine stain involving much of one side of the face and extending to the forehead and upper eyelid. Port wine stains may be present on limbs or trunk in addition to the face. Associated angiomatosis of various inner organs has been recorded in a small number of patients.
Sturge-Weber Syndrome, Angiomatosis Encephalotrigeminalis
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