Klippel-Trenaunay syndrome (KTS) is a complex congenital disorder that historically has been defined as the triad of capillary malformation, venous malformation, and limb overgrowth.
The characteristics of this syndrome are a mixed venous-lymphatic malformation usually involving the extremities. There is usually a port wine like stain on the affected limb and there is usually a difference in size between the affected and nonaffected limb, the affected one being larger. The growth tends to be slowly progressive and can be painful with cramping.
There is currently no cure for KTS, and treatment is centered around preventing progression and controlling symptoms. Compression garments are important as well as a regimen of physical therapy, with swimming a good activity to exercise the limb and improve circulation while minimizing swelling. Laser treatments can be done to treat vesicles that sometime form on the skin and surgery is not curative but can be recommended in some cases.
At Arkansas Children’s, a comprehensive, multi-disciplinary Vascular Anomalies Center diagnoses and treats children and adults with congenital vascular anomalies.