XXXX ( @entity3932 , Klippel-Trenaunay-Weber Syndrome): A Review.

@entity1 @entity154 @entity410 @entity1032 @entity455 @entity227 @entity2695 @entity3932 @entity726 @entity693 @entity600 @entity1939 @entity4848 @entity5995 @entity4904 @entity3809 @entity1211 @entity1462
@entity3932 ( @entity3932 ) ( @entity4904 ) is a @entity4848 associated with @entity3809 of the face, @entity410 , @entity1032 , and usually ipsilateral @entity693 . The classic form affects leptomeninges, eyes, and face. Although the precise etiology and pathogenesis are unclear, the @entity154 is primary @entity1211 with failure of the primordial embryonic venous plexus to regress. A spontaneous somatic mutation in fibroblast fibronectin gene expression in the @entity1462 may occur during embryonic development. Ocular involvement is characterized by conjunctival, episcleral, @entity5995 , and @entity455 . The @entity455 have been inconsistently described as @entity2695 , @entity2695 , and @entity1462 . Based on the endothelial cellular activity, they can be @entity1462 (or @entity3809 ), which never regress spontaneously. Congenital, developmental, and adult-onset @entity726 are often seen when the @entity600 involve the distribution of the first branch of the trigeminal nerve.Both mechanical and vascular causes have been proposed to account for the development of @entity726 . The mechanical theory is based on obstruction of aqueous outflow secondary to developmental anterior chamber angle abnormalities, and the vascular theory is based primarily on elevated episcleral venous pressure. Management of @entity726 in @entity1 with @entity3932 is often challenging and is aimed at controlling intraocular pressure and preventing progressive @entity227 and @entity1939 . It also carries an increased risk for surgical complications. This review summarizes the literature regarding the genetics, clinical features, and management of ocular complications of @entity3932 with special focus on @entity726 .
