Klippel-Trènaunay-Weber Syndrome
(KTW)

Medical Author: David Perlstein, MD FAAP
Medical Editor: William C. Shiel, Jr., MD, FACP, FACR

• What is Klippel-Trènaunay-Weber (KTW) syndrome?
• What is a port-wine stain?
• What is asymmetrical limb hypertrophy?
• Are there other abnormalities in Klippel-Trènaunay-Weber syndrome?
• How is intelligence affected?
• What is the basic defect in Klippel-Trènaunay-Weber syndrome?
• What causes Klippel-Trènaunay-Weber syndrome?
• What is the prognosis (outlook) with Klippel-Trènaunay-Weber syndrome?
• Why is it called Klippel-Trènaunay-Weber syndrome?
• Alternative names for Klippel-Trènaunay-Weber (KTW) syndrome

What is Klippel-Trènaunay-Weber (KTW) syndrome?

Klippel-Trènaunay-Weber (KTW) syndrome is a condition characterized by a triad of findings:

• Port-wine stain or "birthmark" (capillary malformations in the skin)
  
  
• Soft tissue and bony hypertrophy (excessive growth of the soft tissue and/or bones) most often involving a single limb.
  
  
• Vascular anomalies such as varicose veins.

What is a port-wine stain?

A port-wine stain is a certain type of hemangioma. This capillary hemangioma has a recognizable appearance. It is usually a deep violet ("port-wine") colored lesion with fairly linear borders. These are most often found on the affected hypertrophied limb. They are generally flat or only slightly raised compared to the surrounding unaffected skin surface. The actual depth of the lesion is unpredictable and less commonly may even affect muscle and bone.

In addition to the port-wine stain, individuals with Klippel-Trènaunay-Weber also develop varicose veins. These often can be seen at birth as a large superficial vein extending from the lower leg all the way up to the buttocks. This vein has been referred to as the "Klippel-Trènaunay" vein and may not be obvious until later in childhood.

Occasionally affected individuals develop an arteriovenous fistula (abnormal connection between an artery and a vein). This is known as the Parkes-Weber variant of KTW. These patients usually have numerous fistulae that can result in heart failure if untreated.

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