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Sturge-Weber Syndrome

definition

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.

synonyms

Sturge-Weber Syndrome, Angiomatosis Encephalotrigeminalis

UMLS

Angio ocul-orbit-thalam-enceph, Angiomatosis oculo - orbital - thalamo - encephalic syndrome, Angiomatosis, encephalocutaneous, ANGIOMATOSIS, ENCEPHALOFACIAL, Angiomatosis, encephalotrigeminal, DIMITRI DISEASE, Dimitri-Sturge-Weber disease, Disease, Dimitri-Sturge-Weber, Disease, Sturge, Disease, Sturge-Dimitri, Disease, Sturge-Weber, Encephalocutan. angiomatosis, Encephalocutaneous angiomatosis, Encephalofacial haemangiomat, Encephalofacial haemangiomatosis, Encephalofacial hemangiomatosis, Encephalotrigeminal angiomatosis, Neuroretinoangiomatosis, PHAKOMATOSIS, STURGE-WEBER, Sturge - Weber syndrome, Sturge disease or syndrome, Sturge Kalischer Weber Syndrome, Sturge Syndrome, STURGE WEBER DIMITRI SYNDROME, Sturge Weber Syndrome, Sturge-Dimitri disease or syndrome, Sturge-Kalischer-Weber Syndrome, Sturges Syndrome, Sturge's Syndrome, Sturge-Weber - Dimitri syndrome, STURGE-WEBER DISEASE, Sturge-Weber disease or syndrome, Sturge-Weber sequence, Sturge-Weber Syndrome, Sturge-Weber-Dimitri syndr, Sturge-Weber-Dimitri Syndrome, Syndrome, Sturge, Syndrome, Sturge-Kalischer-Weber, Syndrome, Sturge's, Syndrome, Sturge-Weber, Syndrome, Sturge-Weber-Dimitri

images

1 images found for this diagnose diagnosis: Port-Wine Stain, Sturge-Weber Syndrome