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 51-1) and pathomorphology could not be clarified.

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.

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 51-1). These have two essential morphologic forms: truncular and exctratruncular.

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 and nonsurgical treatment can be achieved.¹⁰ Nonsurgical methods have been proved true when surgery is not possible to perform or, when an operation is not yet possible to be performed.

The indication for a sole treatment by compression bandages as a result of a therapeutic frustration, as recommended by Baskerville et al,¹¹ cannot be accepted in the up-to-date knowledge and experiences.

The onset of surgery has a special importance in children with a vascular bone syndrome and limb length discrepancy.^{12,13,14,15,16,17,18} Here the optimal occasion for surgery is the age between 3 and 7 years, because during this period a full compensation or partial compensation of the length discrepancy of the limbs can be expected.^{19,20,21,21A,21B,21C,22} The consequence is that in the first place in such cases vascular surgery has to be considered to gain a reduction or even a removal of a length discrepancy. If later an additional treatment to correct the length discrepancy has to be performed, the following indications should be followed strictly:

1. 
   

   Surgery only has to be performed in the affected limb,

   
   
2. 
   

   when there is no indication for vascular surgery,

   
   
3. 
   

   when in a severe discrepancy after vascular surgery the correction of the length is not sufficient,

   
   
4. 
   

   after the end of growth (the temporary epiphysiodesis is excluded).

Recommendations for orthopedic surgery in order to gain a correction of the length discrepancy of the legs, as recommended by Servelle^23,24 or Ilizarow,²⁵ on the not affected leg have to be declined strictly.

Vascular surgical treatment is indicated for the five main types of peripheral vascular malformations which cause vascular insufficiency, cardiac overload and limb length discrepancy, disfiguration, and disfunction. The bases of this treatment are six different therapeutic strategies, which are linked to special indications and special techniques of surgery^{7,13,14,15,21,21A,21B,21C,26,27,28,29,30}:

1. 
   

   Reconstructive vascular surgery

   
   
2. 
   

   Operations to remove the vascular defect

   
   
3. 
   

   Operations to reduce the hemodynamic activity of the vascular defects

   
   
4. 
   

   Combined treatment

   
   
5. 
   

   Unconventional surgical methods

   
   
6. 
   

   Multidisciplinary treatment

Reconstructive Vascular Surgery

Reconstructive Vascular Surgery has to be performed following the rules of vascular surgery. These are quite rare cases in vascular malformations. Figures 51-2,51-3,51-4 demonstrate a typical clinical example.

Operations to Remove the Vascular Defect

In the venous circulation the marginal vein represents a main indication for the tactic of treatment, which is the operation to remove the vascular defect. The marginal vein is a dysplastic vein, which is localized at the lateral side of the thigh and shank region and it is formed already during the sixth week of the embryo. For the basics of treatment it is mandatory to know whether the principal veins are normal or hypoplastic.

Concerning the classification of the marginal veins, the types of the different segments as well as the types of draining veins were worked out by Weber³¹ (Figure 51-5). This classification is useful and important for the tactics of any treatment of such lesions.