Klippel-Trénaunay Syndrome

What is Klippel-Trénaunay syndrome? What does it look like?

Klippel-Trénaunay Syndrome

Klippel-Trénaunay Syndrome in the lower extremity.  

Klippel-Trenaunay syndrome (kli-PEL TRAY-now-NAY SIN-drohm) (KTS) is a rare disorder that affects blood vessels and surrounding tissues. It is present at birth (congenital) and usually affects the legs. It often has 4 characteristic signs:

  • Port wine stain covering one or both arms and/or legs
  • Vascular anomalies, usually swollen or malformed veins
  • An arm or leg is larger or smaller than the other. These changes in size can also affect the arms, face, head or internal organs.
  • In some children, the bone in one part of a limb, such as a toe or finger, may grow unusually large and thick.

What causes KTS?

KTS is a rare disorder that is present at birth (congenital). We do not know what causes it. It is the most common condition that includes more than one vascular malformation, such as like capillary, venous, lymphatic or arteriovenous malformations.

Will KTS change?

Children with KTS usually do not have limbs or bones that are bigger than usual when they are born. They may begin to get bigger within the first few months or years of life, or may become visible during puberty.

Many children with KTS have a port wine stain. Sometime between puberty and age 30, the stain can change and get small, blood-filled, bubble-like lesions that can bleed.

How is KTS related to Parkes-Weber syndrome?

Parkes-Weber syndrome (PWS) is different from KTS. In PWS, children have abnormal channels (arteriovenous fistula, or fistulas) that connect arteries and veins. This can lead to heart failure if it isn’t treated. This does not happen in KTS.

PWS often causes arms and legs to get bigger than does KTS.

KTS does not include arteriovenous malformations (AV malformations). An angiogram will be normal in KTS and abnormal in PWS.

In PWS, the arm or leg is usually pinker and warmer because the fistula s carry extra blood to that area.

PWS is rarer than KTS and is more likely to affect an arm, although it can also affect the legs.

Symptom PWS KTS
Abnormal channels that connect arteries and veins (fistulas) Yes No
Arms and legs get bigger Yes No
Arteriovenous malformations Yes No
Angiogram results Abnormal Abnormal

How will Seattle Children’s diagnose KTS?

The doctor will examine your child and ask you about their medical history. The doctor may also use the following imaging tests:

  • Doppler ultrasound and MRI (magnetic resonance imaging) to see if there are problems with veins deep in your child’s body
  • MRI to evaluate your child’s soft tissue and any tissue enlargement (hypertrophy)
  • An angiogram to diagnose an abnormal connection between an artery and a vein (arteriovenous fistula) in PWS

These imaging tests also help our doctors watch the arm or leg for changes. We also measure it to see if it is longer than the other arm or leg. This helps doctors treat arms or legs that grow too large.

How will Seattle Children’s treat KTS?

There are many different treatment options for patients with KTS depending on your child’s symptoms:

  • Compression garments, like tight-fitting, elastic sleeves, supply blood flow back to the heart (chronic vein insufficiency). They prevent lymphedema and recurrent bleeding from capillary or venous malformations of the limb. They also protect the limb from trauma. Learn more about compression garments (PDF).
  • Pain medicine, antibiotics and elevating the limb to manage swelling.
  • Medicines to prevent blood clotting (anticoagulant therapy).
  • Heel inserts or surgery may be considered when one leg is longer than another.
  • Your child may be referred to an orthopedic specialist to help develop a treatment plan.
  • Laser therapy to lighten the color of the port wine stain, treat pain or help ulcers or open sores heal.
  • Sclerotherapy (SCLAIR-oh-THAIR-uh-pee) to block unneeded veins.

Some patients with KTS need surgery. This includes:

  • Vein ligation: Clamping off a section of veins to prevent blood flow through a damaged section. It promotes blood flow through veins that are not damaged.
  • Vein stripping: Using a metal wire to remove swelling inside a damaged vein.
  • Vein resection: Removing a section of veins from the body.

Why choose Seattle Children’s Vascular Anomalies Program?

Seattle Children’s Vascular Anomalies Program is nationally recognized. It is 1 of only 2 vascular anomalies programs on the West Coast. Doctors from across the United States send children here for treatment because of our specialized expertise. We see more children with vascular anomalies than any other hospital in the Pacific Northwest.

Our Vascular Anomalies team brings together experts, including otolaryngologists, dermatologists, plastic surgeons, ophthalmologists, geneticists and interventional radiologists. These doctors work together to develop a treatment plan that fits your child’s needs. We schedule your visit so your child sees all the specialists they need on the same day in the same location.

We follow your child throughout their life. Our Vascular Anomalies team is available to help you and answer questions.

We participate in many clinical trials and studies of new treatments that might be more effective than standard therapy. This means some of our patients get the newest treatments before they are widely available. Learn more about our research.

Contact Us 

If you have questions about a consultation or second opinion, email us or call 206-987-4606. If you would like an appointment, ask your child’s primary care provider to refer you.

Providers, see how to refer a patient