Diagnosis of Klippel-Trenaunay syndrome begins with a physical exam. Referral to a vascular malformations specialist is helpful for evaluation and treatment recommendations. During the evaluation your doctor:

  • Asks questions about your family and medical history
  • Does an exam to look for swelling, varicose veins and port-wine stains
  • Visually evaluates growth of bones and soft tissues

Several diagnostic tests can help your doctor evaluate and identify the type and severity of the condition and help determine treatment. Some tests include:

  • Duplex scanning. This test uses high-frequency sound waves to create detailed images of blood vessels.
  • Scanogram. Also called scanner photography, this X-ray technique helps to see images of bones and measure their lengths.
  • MRI and magnetic resonance angiography. These procedures help differentiate between bone, fat, muscle and blood vessels.
  • CT scan. A CT scan creates 3-D images of the body that helps look for blood clots in veins.
  • Contrast venography. This procedure involves injecting a dye into veins and taking X-rays that can reveal abnormal veins, blockages or blood clots.


Although there's no cure for Klippel-Trenaunay syndrome, your doctor can help you manage symptoms and prevent complications.

Because KTS may affect many systems in the body, your health care team may include specialists in vascular medicine and surgery, skin diseases (dermatology), interventional radiology, orthopedic surgery, physical therapy and rehabilitation, and other areas as needed.

You and your doctor can work together to determine which of the following treatments are most appropriate for you. Treatments may include:

  • Compression therapy. Bandages or elastic garments are wrapped around affected limbs to help prevent swelling, problems with varicose veins and skin ulcers. These bandages or elastic garments often need to be custom-fit. Intermittent pneumatic compression devices — leg or arm sleeves that automatically inflate and deflate at set intervals — may be used.
  • Physical therapy. Massage, compression and limb movement as appropriate may help relieve lymphedema in arms or legs and swelling of the blood vessels.
  • Orthopedic devices. These may include orthopedic shoes or shoe inserts to compensate for differences in leg length.
  • Epiphysiodesis (ep-ih-fiz-e-OD-uh-sis). This is an orthopedic surgical procedure that effectively can stop length overgrowth of the lower limb.
  • Embolization. This procedure, performed through small catheters placed into the veins or arteries, blocks blood flow to certain blood vessels.
  • Laser therapy. This procedure may be used to lighten port-wine stains and to treat early blebs on the skin.
  • Laser or radiofrequency ablation of veins. This minimally invasive procedure is used to close off abnormal veins.
  • Sclerotherapy. A solution is injected into a vein, which creates scar tissue that helps close the vein.
  • Surgery. In some cases, surgical removal or reconstruction of affected veins, removal of excess tissue, and correction of the overgrowth of bone may be beneficial.
  • Medication. Early research indicates that a drug called sirolimus (Rapamune) may help to treat symptomatic complex vascular malformations, but it may have significant side effects and more studies are needed.

In addition, treatment may be needed for complications such as bleeding, pain, infection, blood clots or skin ulcers.

Lifestyle and home remedies

Strategies to help manage Klippel-Trenaunay syndrome symptoms include:

  • Keep appointments. Regularly scheduled appointments can help your doctor identify and address problems early. Ask your doctor about a schedule of appointments appropriate for you.
  • Use orthopedic shoes, if recommended. Orthopedic shoes or shoe inserts may improve your physical function.
  • Follow your doctor's recommendations on physical activity. Encouraging use of affected limbs as appropriate may help relieve lymphedema and swelling of the blood vessels.
  • Elevate affected limbs. When possible, raising your leg or other affected limb can help reduce lymphedema.
  • Notify your doctor of changes. Work with your doctor to manage your symptoms and prevent complications. Contact your doctor if you have symptoms of blood clots or an infection or if you experience increased pain or swelling.

Coping and support

Living with Klippel-Trenaunay syndrome can be a challenge. Support and advocacy groups can provide a connection with other people who have KTS. The K-T Support Group and other organizations provide educational materials, resources and information about support groups. Ask your doctor if there is a local support group in your area.

Talking with a psychologist, psychiatrist or other mental health professional may also be helpful to many people with KTS.

Preparing for your appointment

Most cases of Klippel-Trenaunay syndrome are found at birth. So if the doctor suspects that your child has this syndrome, diagnostic tests and treatment will likely begin before your child leaves the hospital.

Your child's doctor will look for developmental problems at regular checkups and give you the opportunity to discuss any concerns. It's important to take your child to all regularly scheduled well-baby visits and annual appointments.

Here's some information to help you prepare for the appointment.

What you can do

If your doctor believes your child shows signs of KTS, basic question to ask include:

  • What diagnostic tests will be needed?
  • When will I get the results of the tests?
  • What specialists might be needed?
  • What medical conditions related to this syndrome need to be addressed right now?
  • How will you help me monitor my child's health and development?
  • Can you suggest educational materials and local support services regarding this syndrome?

What to expect from your doctor

Your child's doctor will ask you a number of questions. Be ready to answer them to reserve time to go over points you want to focus on. The doctor may ask:

  • What symptoms does your child have that concern you?
  • Has your child had any procedures or treatments for these symptoms?
  • Has your child had any previous difficulty with infections or blood clots?
  • Did you have any problems with your pregnancy or your child's birth?
  • Can you tell me about your family's medical history?
  • Is your child having any problems dealing with family, social activities or school?

Klippel-Trenaunay syndrome care at Mayo Clinic

Dec. 19, 2017
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