Klippel-Trenaunay syndrome
Alternative names:
Klippel-Trenaunay-Weber Syndrome, angio-osteohypertrophy;
Nevus Varicousus Osteohypertrophicus Syndrome; hemangiectasia
hypertrophicans; nevus verucosus hypertrophicans
Definition:
A group of findings consisting of:
- Multiple port wine stainsor other vascular nevi
- Hypertrophy (excessive growth) of bones and soft tissue
in the area of increased vascularity,(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.
- Minor or additional findings may include other abnormalities
such as webbing of the fingers (syndactyly)
and extra digits (polydactyly).
Most cases of Klippel-Trenaunay Syndrome are sporadic. A
few cases are thought to be heritable disorders possibly passed
as autosomal dominant trait with low penetrance. Despite cosmetic
appearance most individuals with Klippel-Trenaunay Syndrome
do well.
Updated Date: 02/09/00
Updated By:J. Gordon Lambert, MD, Associate Medical Director,
Utah Health Informatics and adam.com
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