Clinic, diagnosis and treatment of children with dysplasia of the main veins

Dysplasia of the main veins is one of the little-studied problems of childhood, which often leads to the erroneous diagnosis and the wrong treatment tactics of this defect. In the angiomyrosurgical department of the Federal State Sanitary and Epidemiological Service, there were 270 children with dysplasia of the main veins in the SPbGPMU of the Russian Ministry of Health. The appearance of children with dysplasia of the main veins, in the "classical" version, is very characteristic. The extremity is disproportionately increased in volume and elongated. Smooth or bulging vascular spots are observed on the skin (often outside) as a geographical map, cyanotic (purple, "port wine") color. The pathognomonic sign is the presence of an embryonic vein, the trunk of which is of different length and caliber (from 3 to 25 mm) passes along the outer or anterior-external surface of the limb. Sometimes there is gigantism of feet, macrodactyly, syndactyly. However, the above classic clinical picture, which allows confidently diagnose dysplasia of the main veins, is not always revealed. In this regard, we examined the general, characteristic for the majority of patients factors, and those that are most important in determining the severity of the lesion in the preoperative period are identified. We propose a scheme of rational approach to the examination and treatment of sick children (270 patients), which should help optimize the treatment of patients with this malformation. It is established that the volume and type of surgical interventions depend on the type of defect and severity of the lesion.