Klippel-Trenaunay Syndrome (KTS)

What is Klippel-Trenaunay Syndrome (KTS)?

Klippel-Trenaunay syndrome (KTS) is a congenital circulatory disorder characterised by hemiangiomas (abnormal benign growths on the skin consisting of masses of blood vessels), arteriovenous abscesses, and varicose veins, usually on the limbs. The affected limbs may be enlarged and warmer than normal. Fused toes or fingers, or extra toes or fingers, may be present. Bleeding may occur, often as a result of a rectal or vaginal tumour. The cause of the disorder is unknown. A similar port-wine stain disorder in which individuals have vascular anomalies and limb enlargement is Sturge-Weber syndrome. These patients may experience seizures and mental deficiency.

Is there any treatment?

There is no cure for KTS. Treatment is symptomatic. Laser surgery can diminish or erase some skin lesions. Surgery may correct discrepancies in limb size, but orthopaedic devices may be more appropriate.

What is the prognosis?

KTS is a progressive disorder, and complications may be life-threatening.