Angio-osteohypertrophy

Definition

Klippel-Trenaunay syndrome is a group of findings consisting of:

• Multiple port wine stains or other blood vessel malformations (dark spots of skin).
• Excessive growth of bones and soft tissue in the area of increased blood vessels (this may also occur in areas without increased vascularity). This occurs most commonly in the lower limbs, but may affect any limb, the face and head, or internal organs.
• Varicose veins.

Most cases of Klippel-Trenaunay Syndrome occur for no apparent reason, although a few cases are thought to be inherited disorders (possibly passed as an autosomal dominant trait with low penetrance)

Most individuals with Klippel-Trenaunay Syndrome do well despite their cosmetic appearance, although there can be associated psychological problems.

Alternative Names

Klippel-Trenaunay-Weber Syndrome; KTS; Angio-osteohypertrophy; Nevus varicosus osteohypertrophicus syndrome; Hemangiectasia hypertrophicans; Nevus verucosus hypertrophicans