Overview
Klippel-Trenaunay syndrome (KTS) is a rare congenital (present at birth) disorder. It changes the way your soft tissues, bones and blood vessels develop and causes a red “port-wine stain” birthmark on one limb. Many people with KTS also have abnormalities in their lymphatic system. This system helps maintain fluid balance in your body.
Although Klippel-Trenaunay syndrome doesn’t have a cure, many treatments can help you manage symptoms. Early diagnosis and treatment (often soon after birth) can reduce the risk of health complications related to KTS.
Some providers use the abbreviation CLVM to refer to Klippel-Trenaunay syndrome. CLVM refers to the changes in your:
- Capillaries (C), the small blood vessels connecting your veins and arteries
- Lymphatic system (L), the part of your immune system that transports a body fluid called lymph
- Veins (V), the blood vessels that carry blood to your heart
The M is for malformation, meaning a part of your body that didn’t develop in a typical way.
KTS gets its name from the two French doctors who discovered it, Maurice Klippel and Paul Trenaunay. They identified the syndrome in 1900. Experts estimate that KTS affects around 1 in 100,000 people around the world. Anyone can get it; race or sex doesn’t matter.
Advertisement
