Klippel-Trenone syndrome. Etiology, pathogenesis, diagnosis and treatment

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Dysplasia of the main veins (DMV) is known by the names of authors had described this pathology as the Klippel-Trenone syndrome (KTS). Many authors consider the cause of the syndrome to be the impact of various teratogenic factors. These include drugs, infectious agents, radiation exposure, domestic and occupational hazards. Teratogenic factors can damage embryo vessels, causing local stasis and hemorrhages, which can be cause for the perverse formation of veins and surrounding tissues. The clinical picture of severe and extremely severe degrees embryonic type DMV is quite simple. It include asymmetric hypertrophy of the extremities, “disfiguring” in patients with extremely severe degree, extensive cyanotic vascular spots, often accompanied by papillomatous nevus of the skin. The spots are located on the anterolateral surface of the thigh and lower leg. Embryonic veins, which can be found under the spots - a characteristic pathognomonic sign of the KTS. Disturbances in the shape of the limb and external signs of angiodysplasia (vascular spots, atypical veins) in children with mild to moderate severity degree are less pronounced and can be inconstant. Examination and treatment of children with DMV, depending on the severity of the lesion, it is rational to start from the time of detection to 6 years. Phlebography reveal various variants of violation of the surgical anatomy of the veins of the affected limbs. Medium and light forms of dysplasia of the main veins should be differentiated with similar forms of fetal type, congenital Parkes Weber syndrome and acquired iliofemoral thrombosis (atypical veins above the bosom). Sometimes there are combinations of dysplasia of the main, deep, intermuscular and superficial veins. The following operations, according to the indications, are performed: phlebectomy and perforant veins ligation; embryonic veins removal and main outflow correction; musculoskeletal system surgery, abdominal and retroperitoneal surgery, as well as amputation of affected limb. Treatment including surgical and non-surgical methods should be comprehensive and should be performed in highly specialized, multidisciplinary hospitals. (For citation: Azarov MV, Kupatadze DD, Nabokov VV. Klippel-Trenone syndrome. Etiology, pathogenesis, diagnosis and treatment. Pediatrician (St. Petersburg). 2018;9(2):78-86. doi: 10.17816/PED9278-86).