Klippel-Trenaunay Syndrome

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What is Klippel-Trenaunay Syndrome?

Klippel-Trenaunay syndrome is a rare congenital (present at birth) vascular anomaly that results in your child having a large number of abnormal blood vessels. Klippel-Trenaunay syndrome is a complicated condition, and it affects each child in different ways. KTS often has three characteristic signs:

— Capillary malformation covering one or more limbs (arm or leg)

— Hypertrophy ( excessive growth of a limb) OR atrophy( withering or smaller limb)

— Abnormal blood vessels, including veins, capillaries and lymphatic vessels (vessels that drain fluid that leaks from arteries and veins and return that fluid to the bloodstream)


KTS


What causes Klippel-Trenaunay and who gets it?

No one knows the precise cause of KTS. Some doctors think it’s caused by a mutation that occurs before birth (but not one that’s inherited) in the cells that form the lymphatic system, veins and other tissues. No known food, medication or activity during pregnancy can cause Klippel-Trenaunay.

Is there a treatment for KTS?

Because there is no cure for KTS and it’s a progressive condition it is believed that treating your child’s Klippel-Trenaunay syndrome symptoms is the most effective way to manage the disease.

The first step is to have your child evaluated by members of an experienced interdisciplinary vascular anomalies team. This team may include: Dermatology, Hematology, Orthopedics, Interventional Radiology and setting up a supportive care plan.


kts


Resources:

Sturge-Weber.org
Vascular Birthmarks Foundation
RareDiseases.org

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