Klippel-Trenauney Syndrome  (KTS)

What is Klippel-Trenaunay syndrome?

Klippel-Trenaunay syndrome (KTS) is a rare condition in which a child has many abnormal blood vessels. It is a congenital condition, meaning a baby is born with it.

KTS can affect veins, capillaries and lymphatic vessels. Children with KTS often have capillary malformations (port-wine stains), abnormal veins and lymphatic vessels. They may also have one limb that is larger than the other.

What are the symptoms of Klippel-Trenaunay syndrome?

The symptoms of KTS can vary from one child to another. Some common symptoms in children include:

• A port-wine stain, a birthmark caused by a capillary malformation on the skin
• Abnormal blood vessels or lymphatic vessels
• Overgrowth of a limb (hypertrophy), most often a leg
• Skin infections (cellulitis)
• Pain in the overgrown limb
• Bleeding from abnormal capillaries near the surface of the skin
• Blood clots

What causes Klippel-Trenaunay syndrome?

Experts do not know the exact cause of KTS. It is thought that KTS could be the result of a random genetic mutation that affects the cells of the lymphatic system, veins and other tissues.

How is Klippel-Trenaunay syndrome treated?

There is no cure for KTS, so treatment is based on caring for symptoms. Your care team at Arkansas Children's is experienced in treating KTS and will work with you to create the best treatment plan for your child. Treatment options may include:

• Medicines to help control pain or fight infections
• Blood thinning medication to prevent blood clots
• Compression garments, which are tight-fitting pieces of clothing that can help reduce swelling and pain in the affected limb
• Debulking surgery to remove abnormal blood vessels and excess tissue