Chapter 32: Congenital Vascular Malformations

INTRODUCTION

Vascular malformations, angiodysplasias represent a group of diseases, which is characterized by congenital vascular defects encountered in an enormous complexity and variety. In earlier times, these diseases could only be described^1,2,3 (Figure 32-1) and pathomorphology could not be clarified.

DIAGNOSTICS

Since then various noninvasive and invasive diagnostic tools are available. It is mandatory that at first special diagnostic procedures have to be performed so that the different forms and types can be associated. Only after this identification a tactic of treatment can be worked out.

If we follow these principles, we will be able to reach the main basic diagnostic goals, i.e.,

1. definition of the predominantly involved vascular system,

2. fixation of the extent of the malformation and the involvement of adjacent structures, and

3. definition of the disturbed hemodynamics: local, regional, or systemic.

CLASSIFICATION

The essential finding of the last decades was the clear differentiation of vascular anomalies in vascular tumors and vascular malformations. As there existed a discontent among the international specialists dealing with vascular malformations about the classification, in 1988 during the "7th International Workshop on Vascular Malformations" in Hamburg by a consensus conference, a classification was worked out concerning the species and anatomopathological form of the defects^4,4A,5 (see Table 32-1). These have two essential morphologic forms: truncular and exctratruncular.

THERAPEUTIC TACTICS

A vascular surgical treatment of congenital vascular defects can be successful only if a strict indication for surgery is performed and if the basic principles of the therapeutic strategy are followed,^4,6,7,9 i.e.,

1. start in early childhood (at 3–7 years of age);

2. influence the pathophysiologic processes and abolish the hemodynamic disfunction;

3. adopt a harmonized individual therapy;

4. perform surgery radically without loss of function;

5. perform a stepwise surgical treatment;

6. perform a multidisciplinary therapy.

Within these therapeutic strategies, surgical ...