Klippel-Trenaunay Syndrome

Klippel-Trenaunay syndrome (KTS) is a rare congenital (present at birth) disorder. It changes the way your soft tissues, bones and blood vessels develop and causes a red “port-wine stain” birthmark on one limb. Many people with KTS also have abnormalities in their lymphatic system. This system helps maintain fluid balance in your body.

Although Klippel-Trenaunay syndrome doesn’t have a cure, many treatments can help you manage symptoms. Early diagnosis and treatment (often soon after birth) can reduce the risk of health complications related to KTS.

Some providers use the abbreviation CLVM to refer to Klippel-Trenaunay syndrome. CLVM refers to the changes in your:

Capillaries (C), the small blood vessels connecting your veins and arteries
Lymphatic system (L), the part of your immune system that transports a body fluid called lymph
Veins (V), the blood vessels that carry blood to your heart

The M is for malformation, meaning a part of your body that didn’t develop in a typical way.

KTS gets its name from the two French doctors who discovered it, Maurice Klippel and Paul Trenaunay. They identified the syndrome in 1900. Experts estimate that KTS affects around 1 in 100,000 people around the world. Anyone can get it; race or sex doesn’t matter.

Symptoms

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Complications

Blood clots are a dangerous complication of atrial fibrillation (AFib). Blood clots can lead to stroke.

The risk of stroke from AFib increases as you grow older. Other health conditions also may increase the risk of a stroke due to AFib. These conditions include:

High blood pressure.
Diabetes.
Heart failure.
Some types of heart valve disease.

Blood thinners are commonly prescribed to prevent blood clots and strokes in people with atrial fibrillation.

Prevention

Healthy lifestyle choices can reduce the risk of heart disease and may prevent atrial fibrillation (AFib). Here are some basic heart-healthy tips:

Control high blood pressure, high cholesterol and diabetes.
Don't smoke or use tobacco.
Eat a diet that's low in salt and saturated fat.
Exercise at least 30 minutes a day on most days of the week unless your health care team says not to.
Get good sleep. Adults should aim for 7 to 9 hours daily.
Maintain a healthy weight.
Reduce and manage stress.

By Mayo Clinic Staff

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Diagnosis & treatment

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