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Sturge-Weber syndrome

Pronunciation: sturj va'ber

Definition: in its complete form, a triad of unilateral occurrence of congenital capillary malformation (flame nevus) in the distribution of the trigeminal nerve; ipsilateral leptomeningeal vascular malformations with intracranial calcification and neurologic signs; and vascular malformation of the choroid plexus, often with secondary glaucoma. Inheritance is unclear with most cases sporadic.

Synonym(s): cephalotrigeminal angiomatosis, encephalotrigeminal angiomatosis, Sturge-Kalischer-Weber syndrome, Sturge-Weber disease

See Also: encephalotrigeminal vascular syndrome, Jahnke syndrome

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© Copyright 2018 Wolters Kluwer. All Rights Reserved. Review Date: Sep 19, 2016.