What is the Klippel-Weber-Trenone Syndrome

What is the Klippel-Weber-Trenone Syndrome?

As is known, congenital pathologies are transmitted togenetic level from parents or arise from a violation of embryogenesis. Anomalies can be formed from any tissues or organs. Most often they are detected immediately after the birth of the child, but sometimes manifest much later. An example of a congenital anomaly is the Klippel-Weber-Trenone syndrome. This disease refers to the pathologies of the vascular system, it affects primarily the veins of the lower extremities.

Unlike many other congenital anomalies, the Klippel-Weber-Trenone syndrome can have a favorable prognosis for life. It is achieved with the help of timely surgical treatment of the disease.

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Pathology: Klippel-Trenone-Weber Syndrome - what is it?

This disease is known since the beginning of the 20th century,when it was first described by two scientists - Trenone and Klippel. Later, Dr. Weber supplemented already available information about this pathology. It is known that the ailment has another name - osteohypertrophic venous nevus.

The Klippel-Weber-Trenone syndrome occurs inmainly among the male population. At this time, information about the main cause of the development of the disease and its epidemiology is not enough. The main symptoms of pathology are extensive "birthmarks" (nevuses) on the legs, various diameters of the lower extremities, varicose veins. Despite the early development of clinical signs and the severity of the lesion, in some cases, the syndrome is treatable. This advantage is achieved due to the emergence of new technologies in the field of vascular surgery.

Syndrome of the reason

Causes of the problem

Since the disease was described comparativelyrecently (about 100 years ago), at the moment there is no reliable information about its etiology. Nevertheless, there are several theories according to which the Klippel-Trenone-Weber syndrome develops. The causes of pathology are associated with hereditary burden. It is understood that the main etiological factor is the mutation of genes. According to another theory, the cause of the disease lies in the violation of embryogenesis in the early stages of pregnancy. Under the influence of unfavorable environmental factors (chemical poisoning, psychoemotional loads, irradiation with radiation) occurs aplasia of the venous system. Her underdevelopment is also associated with infectious agents. According to this theory, the cause of the pathology is bacteria or viruses that got into the mother's body during pregnancy.

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Clinical picture of the disease

The first manifestations of the syndromeKlippel-Trenone-Weber are already visible during the period of newborn. In rare cases, they occur later - in childhood. The classic symptoms of the disease are the following symptoms:

  1. Angiomas are vascular spots. Most often they are on one leg and cover a large area. Color angiomas can be different: from light to dark purple. Under the influence of physical factors (friction, impact), the skin in the area of ​​"birthmarks" is easily damaged, bleeding appears.
  2. Varicose veins of the superficial veins. Clinical manifestations of this trait are the tortuosity, thickening, enlargement and soreness of the vessels of the lower limb.
  3. Hypertrophy of the affected leg. Because of the defeat of deep veins and their proliferation, the limb increases in diameter. In some cases, the bone system is included in the process. Then the affected leg can be longer than the healthy limb.

Sometimes one of the signs of the disease is absentor does not appear externally (for example, varicose veins). In some cases, the pathology progresses rapidly, and the lesion passes from the extremities to the trunk and upper humeral girdle.

Diagnostics

To suspect the Klippel-Weber-Trenone syndrome is possibleon extensive angiomas. This symptom appears first. Later, varicose disease and hypertrophy of the extremity join. A characteristic sign of this pathology is one-sided defeat. If a disease is suspected, laboratory and instrumental diagnostics are performed. First and foremost, ultrasound and dopplerography of the lower extremities are performed. A feature of this syndrome is the lack of correlation between venous pressure and cardiac activity. This sign is reflected by a straight line in the conduct of venography.

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Clippel-Trenone-Weber Syndrome: treatment of pathology

Elimination of the disease depends on the degreeDeep vein lesions. With significant narrowing, shunting is performed. If the main vein is affected in a large area, then the vessel is transplanted with artificial material. As an additional treatment, sclerotherapy (preparations "Trombovar", "Fibro-Wayne"), wearing compression linen is applied. Also, a constant reception of anticoagulants ("Warfarin" medication) is necessary. In some cases, the operation on the vessels can be performed with a laser.



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