Congenital Arterial and Arteriovenous Dysplasia

Abstract

Angiodysplasias are a combination of several different congenital malformations. They are polydysplasias with arterial, venous, capillary, and lymphatic components. Pathologic changes involve not only the vascular systems, but all other tissues as well: skin, connective tissues, bones, cartilage, and nerves. The classification of the different clinical pictures is based mainly on the vascular system predominant in each type of angiodysplasia [10,11,13,16,19]. The treatment of angiodysplastic anomalies is sometimes palliative, symptomatic, or cosmetic (reduction of the difference in length between the limbs, surgery of varices, extirpation of hemangiomas, lymphangiomas, and other vascular and connective tissue proliferations localized on the body’s surface). Most therapeutic measures are focused on the arterial components of the anomaly, on arterial hypervascularization, vascular proliferations, and especially on pathologic arteriovenous shunts. The aim of treatment is not only to eliminate active arteriovenous fistulas and vascular proliferations by resecting and inducing obliteration, but also to minimize hypervascularization. These therapeutic measures are not suited to angiodysplastic changes that are limited to the venous system.