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congenital dysplastic angiomatosis


Definition: autosomal dominant angiomatosis in which there is dysplasia of the underlying tissues, sometimes with overgrowth of bone (Klippel-Trenaunay-Weber syndrome), or encephalotrigeminal angiomatosis (Sturge-Weber syndrome) in which there is an angioma in the distribution of one or more branches of the trigeminal nerve, with vascular anomalies and calcification of the cerebral cortex.

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