title: [Angiodysplasia and the skeletal system].
abstract: 1. Congenital angiodysplasias of the extremities are often associated with skeletal disorders, i.e., overgrowth (local giantism, hyperplasia) or reduced growth (hypoplasia). From a clinical point of view three types of combined lesions may be differentiated: (a) a secondary local giantism induced by hemodynamically active congenital a.v. fistulas, usually of intra- and extraosseous location (Weber-type); - (b) local giantism in combination with venous and/or lymphatic angiodysplasias; in this group skeletal overgrowth seems not to be caused by the vascular anomalies but represents more probably a coordinated "inborn error" of tissue composition and distribution (Klippel-Trenaunay-type); - (c) hemangiomatosis usually of venous (cavernous) type, affecting soft tissues as well as bones in association with skeletal hypoplasia. There is a retardation of bone growth by substitution and destruction of the epiphysial cartilages by the intraosseous hemangioma (Servelle-Martorelltype). In most cases (a-c) the use of special angiological investigations allows a clear diagnosis particularly concerning the pathogenesis of skeletal disorders. 2. In looking for an unequivocal terminology the clinical classification of these anomalies should be based on the leading clinical symptome, i.e., the giantism or the angiodysplasia. In this respect the vascular findings take on a predominant position. Paying regard to historical aspects the classification may be completed by the additional term "Klippel-Trenaunay" type, "Weber" type, "Servelle-Martorell" type. 3. An exact angiologic diagnosis gives further informations about prognosis and choice of treatment (group I: Early operative elimination or reduction of the a-v shuntvolume; group II and III: "wait and see!"; usually conservative treatment with compression bandages).
mesh_terms: D000798:Angiomatosis / Q000150:complications*; D001165:Arteriovenous Malformations / Q000601:surgery; D001859:Bone Neoplasms / Q000150:complications*; D002648:Child; D005260:Female; D006130:Growth Disorders / Q000150:complications*; D006391:Hemangioma / Q000601:surgery; D006801:Humans; D007715:Klippel-Trenaunay-Weber Syndrome / Q000601:surgery; D008297:Male; D008875:Middle Aged; D011696:Purpura, Thrombocytopenic / Q000601:surgery; D012878:Skin Neoplasms / Q000601:surgery; D013341:Sturge-Weber Syndrome / Q000601:surgery; D013577:Syndrome; D013997:Time Factors
vernacular_title: Angiodysplasie und Skeletsystem