Dysplasia of the main veins (DMV) is known by the names of the authors who described this pathology as Klippel Trenone syndrome. The clinical picture of Klippel Trenone syndrome (CTS) in the classical version is characterized by a triad of symptoms: vascular spots, varicose atypical veins, hypertrophy of soft tissues and bones with an increase in the volume and length of the affected limb. The incidence of this defect from all vascular malformations is 49.6%. To diagnose a malformation, ultrasound is used Dopplerography, MRI and MSCT with contrast, ascending venography. Vasocontrast methods reveal various options for surgical anatomy of the veins of the affected limbs. To assess the anatomy of deep veins, we used upward contrasting, since we consider this species to be more accurate and low-cost. The study is based on the results of examination and treatment of 200 patients. Long-term results were analyzed in 108 patients with congenital malformations of the vessels of the lower extremities aged 1 year to 18 years, who were in the angiomicrosurgical department of the Pediatric Medical University from 2005 to 2015. Patients were divided into 2 groups: the embryonic type 70 people (of which 42 boys and 28 girls), and the fetal type 38 people (16 of them boys, 22 girls). In this paper, we analyze the results of ascending phlebography of the extremities, which is considered the gold standard for examining vein malformations. Contrast phlebography in 108 patients with dysplasia of the main veins, hypo- and dysplasia of segments of the deep and superficial veins were detected 63%; atypical location of superficial or deep veins with the presence of congenital pathways of the collateral outflow 30%, aplasia of the veins 7%. Conclusions: phlebography data accurately determine the surgical anatomy of blood vessels in patients with dysplasia of the main veins. The data obtained during the ascending phlebography, in almost 100% of cases coincide with intraoperative data thus allowing the development of optimal tactics of operations in the veins, and also scrutinize the pathology of musculoskeletal system.
Anatomic and surgical features of lower extremities blood vessels in case of major veins dysplasia in children with various type and severity of the disease according to data of contrast flebography
