Terminology and classification of congenital vascular malformations. 2007

B B Lee, and J Laredo, and T S Lee, and S Huh, and R Neville
Department of Surgery, Georgetown University, Washington DC, USA. bblee38@comcast.net

Venous malformation (VM) is a congenital vascular malformation (CVM) that develops along the venous system through the various stages of embryogenesis. Older terminology and classification were often misleading and confusing. A newer classification system has emerged that is based on advances in the study of these conditions and is useful in contemporary management. The Hamburg classification was introduced after reappraisal of older terminology and has become the standard system for contemporary classification, which is based on anatomical, pathological and embryological criteria.

UI MeSH Term Description Entries
D007715 Klippel-Trenaunay-Weber Syndrome A congenital disorder that is characterized by a triad of capillary malformations (HEMANGIOMA), venous malformations (ARTERIOVENOUS FISTULA), and soft tissue or bony hypertrophy of the limb. This syndrome is caused by mutations in the VG5Q gene which encodes a strong angiogenesis stimulator. Klippel-Trenaunay Disease,Angio-Osteohypertrophy Syndrome,Angioosteohypertrophy Syndrome,Congenital Dysplastic Angiopathy,KTW Syndrome,Klippel Trenaunay Syndrome,Klippel-Trenaunay Syndrome,Klippel-Trénaunay-Weber Syndrome,Angio Osteohypertrophy Syndrome,Angio-Osteohypertrophy Syndromes,Angioosteohypertrophy Syndromes,Angiopathies, Congenital Dysplastic,Angiopathy, Congenital Dysplastic,Congenital Dysplastic Angiopathies,Disease, Klippel-Trenaunay,Dysplastic Angiopathies, Congenital,Dysplastic Angiopathy, Congenital,KTW Syndromes,Klippel Trenaunay Disease,Klippel Trenaunay Weber Syndrome,Klippel Trénaunay Weber Syndrome,Klippel-Trenaunay Syndromes,Syndrome, Angio-Osteohypertrophy,Syndrome, Angioosteohypertrophy,Syndrome, KTW,Syndrome, Klippel Trenaunay,Syndrome, Klippel-Trenaunay,Syndrome, Klippel-Trenaunay-Weber,Syndrome, Klippel-Trénaunay-Weber,Syndromes, Angio-Osteohypertrophy,Syndromes, Angioosteohypertrophy,Syndromes, KTW,Syndromes, Klippel-Trenaunay
D009626 Terminology as Topic Works about the terms, expressions, designations, or symbols used in a particular science, discipline, or specialized subject area. Etymology,Nomenclature as Topic,Etymologies
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D054079 Vascular Malformations A spectrum of congenital, inherited, or acquired abnormalities in BLOOD VESSELS that can adversely affect the normal blood flow in ARTERIES or VEINS. Most are congenital defects such as abnormal communications between blood vessels (fistula), shunting of arterial blood directly into veins bypassing the CAPILLARIES (arteriovenous malformations), formation of large dilated blood blood-filled vessels (cavernous angioma), and swollen capillaries (capillary telangiectases). In rare cases, vascular malformations can result from trauma or diseases. Malformation, Vascular,Malformations, Vascular,Vascular Malformation

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