Klippel-Trenaunay Syndrome (KTS)

Overview

Klippel-Trenaunay syndrome (KTS) is a rare congenital vascular disorder in which a limb may be affected by port wine stains (red-purple birthmarks involving blood vessels), varicose veins, and/or too much bone and soft tissue growth. The limb may be larger, longer, and/or warmer than normal. The cause is unknown.

When should I seek treatment for KTS?

Symptoms and severity of KTS vary for each patient. Symptoms can include bleeding from the affected limb, a skin infection, blood in the urine, or rectal/vaginal bleeding. You could experience pain in the limb, blood clots, anemia and seizures.

Seek treatment to alleviate pain and symptoms as necessary.

What are the treatment options for KTS?

The goals of treatment are to reduce symptoms and reduce the risk of complications. Your health care provider will recommend the treatment option that is right for you.

At Cleveland Clinic, treatment for KTS involves multidisciplinary expertise. We work with each individual patient to identify his/her specific needs and tailor therapy as appropriate. Examples of treatment options are detailed below. Please speak to your healthcare provider about the specifics of your case.

Medications and iron supplements may be prescribed, as well as compression stockings to alleviate pain and swelling.

Surgery and other minimally invasive therapies are sometime necessary to treat KTS and its complications.

When is surgery or minimally invasive therapies necessary for KTS?

Surgery may be necessary for KTS to reduce the threat of serious complications. Surgical treatments are performed in the hospital or outpatient setting by a vascular surgeon.

Ligation and Stripping

Ligation means the surgical tying of veins through a small incision in the skin to prevent pooling of blood. Ligation may be used in conjunction with vein stripping, or removal of the vein. In many instances, the vein is removed using a minimally invasive surgical procedure called venous ablation.

Sclerotherapy

Sclerotherapy may be recommended. Sclerotherapy involves the injection of a solution directly into the varicose veins that cause them to collapse and disappear. Several sclerotherapy treatments usually are required to achieve the desired results. Foam sclerotherapy is a variation of the procedure performed under ultrasound guidance that involves the injection of a foaming agent mixed with a sclerosing agent. The foaming agent moves blood out of the vein so the sclerosing agent will have better contact with the vein wall. Sclerotherapy can eliminate the pain and discomfort of varicose veins and helps prevent complications such as venous hemorrhage and ulceration.

Laser Therapy

Laser therapy is available to lighten or remove the port wine stain. Laser and pulse-light therapy are also used to heat the blood vessel to shrink it. Laser therapy is most effective for the treatment of small varicose veins. Laser therapy may be used as an additional treatment after sclerotherapy, endovenous procedures, or surgery of larger veins. Lasers are also being used for endovenous ablation of larger varicose veins. Laser treatments may require several sessions, spaced at six-week intervals, to properly treat the vein.

Endovenous Thermal Ablation

Endovenous thermal ablation is a newer version of ligation and stripping . During this procedure, a laser or high-frequency radio waves are used to create intense local heat in the varicose vein. The technology is different with each type of energy source, but both forms of local heat close up the targeted vessel. This treatment closes off the problem veins but leaves them in place so there is minimal bleeding and bruising. Compared with ligation and stripping, endovenous thermal ablation results in less pain and a faster return to normal activities, with similar cosmetic results.

What outcomes can I expect from treatment?

At Cleveland Clinic, we work with each patient to treat the specific symptoms of KTS. Outcomes are generally very good; your healthcare provider will be able to provide more detail.