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 described1,2,3 (Figure 32-1) and pathomorphology could not be clarified.
Gigantism in combination with a vascular malformation of a right leg.
Reproduced, with permission, from Friedberg H. Riesenwuchs des rechten Beines. Virchows Arch. 1867;40:353.
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.,
definition of the predominantly involved vascular system,
fixation of the extent of the malformation and the involvement of adjacent structures, and
definition of the disturbed hemodynamics: local, regional, or systemic.
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 defects4,4A,5 (see Table 32-1). These have two essential morphologic forms: truncular and exctratruncular.
TABLE 32-1.Classification of Congenital Vascular Defects According to Their Species and Anatomic Form ("Hamburg Classification 1988") |Favorite Table|Download (.pdf) TABLE 32-1. Classification of Congenital Vascular Defects According to Their Species and Anatomic Form ("Hamburg Classification 1988")
| ||Anatomical Forms |
|Species ||Truncular ||Extratruncular |
|Predominantly arterial defects ||Aplasia or obstruction dilatation ||Infiltrating limited |
|Predominantly venous defects ||Aplasia or obstruction dilatation ||Infiltrating limited |
|Predominantly lymphatic defects ||Aplasia or obstruction dilatation ||Infiltrating limited |
|Predominantly AV shunting defects ||Deep AV fistulae superficial AV fistulae ||Infiltrating limited |
|Combined vascular defects ||Arterial and venous, (without AV-shunt) Hemolymphatic (with or without AV-shunt) ||Infiltrating hemolymphatic Limited hemolymphatic |
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.,
start in early childhood (at 3–7 years of age);
influence the pathophysiologic processes and abolish the hemodynamic disfunction;
adopt a harmonized individual therapy;
perform surgery radically without loss of function;
perform a stepwise surgical treatment;
perform a multidisciplinary therapy.