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Klippel-Trenaunay Syndrome

Conditions

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Klippel-Trenaunay Syndrome (KTS) is a very rare condition in which blood vessels do not form properly. It can affect the capillary, venous, and lymphatic vessels.

Signs and Symptoms

KTS is usually present at birth (congenital) and can often involve the legs.

Characteristics may include:

  • A flat, pink to reddish area on skin (known as a port wine stain)
  • Malformed or swollen blood vessels
  • One side of the body or limb is larger than the other (known as hypertrophy)

Common problems may include:

  • Pain of the affected area or limb
  • Bleeding
  • Blood clots – due to the slow movement of blood in the abnormal vessels
  • Skin infections – due to abnormal blood flow to the area

Diagnosis and Tests

Diagnosis and tests may consist of:

  • Physical exam
  • Blood work
  • Imaging
  • Doppler ultrasound to see if there are problems with the blood vessels
  • Magnetic Resonance Imaging (MRI) to see if there are problems with blood vessels and to look for tissue or bone overgrowth (hypertrophy)

Treatment

Management of KTS involves a multidisciplinary team. Treatment goals include reducing the symptoms and risks associated with KTS.

Care may include:

  • Compression therapy – to reduce the pain and swelling, and allow for improved movement of affected area
  • Anticoagulation – medication to help prevent clots from forming and to help reduce pain
  • Sclerotherapy – a solution is injected into the abnormal vessels to help make them shrink
  • Surgical intervention