What is Klippel Trenaunay Syndrome? - Part 2

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What is KT?

Klippel-Trenaunay Syndrome is a rare, congenital, vascular disorder affecting one or more limbs. Klippel-Trenaunay Syndrome was first noted in a 1900 publication of Archives Generales de Medecine. In the article, Du Naevis Variquex, Osteo-Hypertrophique, French physicians Klippel and Trenaunay described a clinical syndrome with three major symptoms (Gloviczki, 1982). These main symptoms are often referred to as the "triad." The triad consists of hemangiomas, bone and soft tissue hypertrophy, and vein varicosities. Hemangiomas are often apparent at birth, or by two weeks of age (Samuel, 1995). The hemangioma, or nevus, is usually confined to a part of the limb. In other cases, the entire limb is affected by the hemangioma (Samuel, 1995). Capillary hemangiomas are the most common type and are called port wine stains due to the red and purple color (Letts, 1977). Bone and soft tissue hypertrophy is a result of increased growth around an organ. In many cases, limb length is affected and t he length of the limb is different than the normal limb. The soft tissue hypertrophy is symmetrical around the affected extremity. In most cases, the girth of the limb is larger, although atrophy is common in some patients. Varicose veins are often v ery noticeable in Klippel-Trenaunay Syndrome patients. Varicose veins result from damaged or defective valves in a vein. A vein becomes damaged when the smooth muscle in the wall of veins weakens and the valves cannot support the weight of blood. T he pressure on the valve causes it to collapse and it no longer functions properly. Klippel-Trenaunay Syndrome patients are affected by other symptoms as well (See Table 1). These symptoms are variably expressed and may not have identical effects on other patients. Each case of Klippel-Trenaunay Syndrome is unique. In 1907, Parkes and Weber described a disorder with the same symptoms involved in Klippel-Trenaunay Syndrome with the addition of arteriovenous fistula. This derivative of Klippel-Trenaunay Syndrome was called Klippel-Trenaunay-Weber Syndrome.


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