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Signs and Treatments for Klippel-Trenaunay Syndrome

Signs and Treatments for Klippel-Trenaunay Syndrome

Klippel-Trenaunay syndrome is a rare disorder, affecting only one newborn per 20,000-40,000 births. It causes abnormalities in the vascular system with no known cure. The primary goal of treatment is symptom relief and preventive care.

Board-certified physician Dr. Eric Mai at STL Vein & Cosmetics develops personalized long-term treatment plans for patients with Klippel-Trenaunay syndrome to alleviate or reduce symptoms. Treatment plans also focus on preventing potential future complications of the disorder. Read on to learn more about Klippel-Trenaunay syndrome, symptoms, and treatment.

What is Klippel-Trenaunay syndrome? 

Klippel-Trenaunay syndrome (KTS) is a rare congenital (from birth) disorder affecting your blood vessels (veins and capillaries), lymphatic system, bones, and soft tissues (skin and muscles) of a limb. The cause of KTS is unknown.

KTS affects all ethnicities and genders equally. While the disorder is present at birth, the first and only sign is often a red-purple birthmark called a port-wine stain. KTS may be difficult to diagnose until limb hypertrophy or varicose veins are noticeable.

Signs of Klippel-Trenaunay syndrome

KTS impacts the development of bones, blood vessels, and soft tissues like your skin and muscles. The disorder is characterized by three distinct features: a port-wine stain birthmark, hypertrophy (abnormal overgrowth) of underlying bones and soft tissues, and vein malformations. 

Port-wine stain

A port-wine stain is a congenital birthmark caused by a vascular malformation resulting from abnormal blood vessel growth. Depending on where a port-wine stain is located, it can be completely harmless or cause complications later in life. Sometimes it’s also a sign of a more serious underlying condition (such as KTS) that needs treatment.

These birthmarks are a collection of blood vessels that grow close to the skin, giving the appearance of a dark mass. A port-wine stain is often very subtle in childhood, appearing as a vague discoloration, but the birthmark darkens with age, often turning deep red or purple, like the port wine it’s named after. 

Lymphatic irregularities

Your lymphatic system is made up of lymph nodes and vessels that protect you from viruses and bacteria, playing an essential role in your immune system and the healing process. It fights off diseases and infections in your body. For those with KTS, the lymphatic system experiences overgrowth and dysfunction, causing leakage and swelling in soft tissue.


Hypertrophy is an overgrowth of bones and soft tissue. A person without KTS has bones that eventually stop growing. Those with KTS have bones and soft tissue that never stop growing. Limb enlargement can range from minimal to severe. Excessive growth in length is typical with KTS, but some patients experience the opposite, having a limb shorter than normal. 

Venous disease

Your valves pump blood back up to your heart. Vein disease occurs when these valves malfunction. Patients with KTS have valves that eventually break down, causing varicose veins and sometimes deeper malformed veins that become more pronounced with age.

Treatment for Klippel-Trenaunay syndrome

Because KTS impacts different body parts, symptoms vary, and every patient experiences unique challenges. Early diagnosis of Klippel-Trenaunay syndrome is vital in creating a long-term treatment plan.

The diagnosis of KTS is based on physical signs and symptoms. Computed axial tomography (CAT) scans and magnetic resonance imaging (MRI) tests might be necessary to determine the extent of the disorder and how we can most effectively help you manage it.

The National Organization of Rare Disorders (NORD) recommends laser therapy or sclerotherapy depending on the symptom.

Schedule a consultation with Dr. Mai at STL Vein & Cosmetics for expert diagnosis and care. Call the office or request an appointment online today.

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