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Klippel-Trenaunay Syndrome

Klippel-Trenaunay Syndrome

Klippel-Trenaunay syndrome is a congenital malformation of the venous system, characterized by varicose veins in the lower limbs, increase in volume (hypertrophy) of the affected areas and the appearance of skin angioma (tumor on the skin surface).

Untreated, the condition can have major complications, from massive bleeding to thrombophlebitis and pulmonary thrombembolism.

In 90% of cases, Klippel-Trenaunay's disease is one-sided and most commonly occurs in the leg area, but one of the upper limbs may also be affected.

Causes

Klippel-Trenaunay syndrome is caused by a congenital anomaly in the venous system. Normally, the fetus develops in the intrauterine environment a ventral vascular system (primitive femoral artery and vein) and a dorsal and sciatic system.


Starting with the second month of pregnancy, the femoral system continues to develop, while the sciatic is atrophied. If the sciatic system persists, the lateral venous anomalies responsible for Klippel-Trenaunay syndrome arise.
Abnormalities are present in the lower limbs or more rarely (1/6) in the upper limbs, and less often the syndrome affects the lower limbs bilaterally or the upper and lower limbs on the same side.

Types of Klippel-Trenaunay syndrome

Klippel-Trenaunay syndrome can be of three types, depending on the symptomatology.

Type I

Complete anomaly, with lateral and posterior skin tumor. At the level of the skin, diffuse lesions appear that can extend into the area of ​​the muscles and even the bones. Hemorrhoidal vessels are affected, resulting in the presence of hemorrhoids with recurrent rectal bleeding. Increasing the length and diameter of the limbs is also possible.

Type II

Venous anomaly, without marked increase in the volume of the limbs. Patients experience weight loss in the extremities and progression to chronic venous insufficiency and pulmonary embolism.

Type III

The minor form of the disease, diffuse varicose veins, spots and other less invasive lesions appear.

Treatment

Initially, the treatment is a conservative one: elastic sock for compression, lower limb lift, trauma avoidance and orthopedic prosthesis of the limb hypertrophy. Depending on the stage of the disease, there are surgical procedures specific to the complications. Rarely, the unfavorable evolution of the disease involves the amputation of the limb.
Patients with this syndrome have a good evolution, which allows them a normal social activity under the appropriate treatment conditions.