Klippel-Trenaunay-Weber Syndrome

• What is Klippel-Trenaunay-Weber (KTW) syndrome?
• What is asymmetrical limb hypertrophy?
• What are hemangiomas?
• What are nevi?
• Are there ever other abnormalities in KTW?
• How is intelligence affected?
• How is KTW syndrome classed in the annals of medicine?
• What is the basic defect in KTW?
• What causes KTW?
• What is the prognosis (outlook) with KTW?
• Why is it called Klippel-Trenaunay-Weber syndrome?
• Alternative names for Klippel-Trenaunay-Weber (KTW) syndrome

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

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)
• Venous malformations & lymphatic abnormalities.

There is usually asymmetrical limb hypertrophy.

What is asymetrical limb hypertrophy?

By "asymmetric limb hypertrophy" is meant enlargement of one limb and not the corresponding limb on the other side. The enlarged limb is three times more likely to be a leg and not an arm in KTW. However, KTW is a somewhat erratic condition, one that is loath to follow hard and fast rules so that, for example, upon occasion there is enlargement of two limbs or a buttock or part of the trunk of the body. The enlargement is always not just of soft tissue but of bone, too.

Next: What are hemangiomas? »

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