[Congenital angiodysplasia of the limbs; diagnosis and therapy]

Abstract

A complete check-up of vascular morphology and haemodynamics in patients resulted in a differentiation of three clinical entities: (I) type F. P. Weber: multiple active arteriovenous fistulas inducing overgrowth of the skeleton, (2) type Klippel-Trenaunay: venous angiodysplasia with the triad localized gigantism, varicose veins, naevus flammeus, (3) type Servelle-Martorell: systemic haemangiomatosis including the bone and soft tissues resulting in hypoplasia of the skeleton. Early diagnosis and differentiation of these different clinical types are needed for the assessment of the spontaneous course of the disease and choice of an optimal therapeutical approach. In the presence of active arteriovenous fistula (type F. P. Weber) the therapeutic principle should be focused on the normalisation of the shunt volume by surgery (skeletonisation) or catheter embolisation. In patients with venous angiodysplasias (type Klippel-Trenaunay or Servelle-Martorell) the therapy is aimed at prevention or reduction of deep venous insufficiency and is basically conservative by external compression bandages.