Klippel-Trenaunay Syndrome

Klippel-Trenaunay syndrome (KTS) is a disorder that is when you are born (congenital). It is a circulatory disorder characterized by:

  • Port-wine-stains. This is also called capillary malformation. It is the result of abnormal development of small blood vessels. It appears as flat, pink to red patches on the skin.

  • An overgrowth that grows out of proportion to the rest of the body. Usually only one limb is affected.

  • Abnormal development of vessels that clear lymph fluid in the body (lymphatic malformations).

  • Abnormal development of blood vessels that return blood back to the heart (venous malformations).

  • Arteries and veins may be connected in the wrong way (arteriovenous fistula).

  • Blood clots may form in the involved veins (venous thromboembolism). This may be life threatening. The cause of the disorder is unknown. It is not hereditary.

  • KTS affects mostly the legs, varicose veins maybe present.

TREATMENT

There is no cure for KTS. Treatment is symptomatic. Laser therapy can be used to diminish or decrease the appearance of the pink or red discoloration on the skin (capillary malformation). Surgery may correct difference in limb size, but orthopedic devices may be needed. Therefore close follow up with orthopedic surgery is advised.

PROGNOSIS

KTS is not life-threatening but impacts the quality of life. Limb enlargement progresses very rapidly until it suddenly stops. The risk of infection is often higher in individuals with vascular abnormalities.