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?
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.
Next: What is asymmetrical limb hypertrophy? »
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