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Klippel-Trénaunay-Weber Syndrome or KTS is a congenital medical condition in which blood vessels and/or lymph vessels fail to form properly.
There exists some controversy over the terminology.
Although the cause and processes surrounding Klippel-Trenaunay Syndrome (KTS) are poorly understood, the birth defect is diagnosed by the presence of a combination of these symptoms (often on approximately ¼th of the body, though some cases may present more of less affected tissue):
Note: In some cases, patients may present without port-wine stains. Such cases are very rare and may be classified as atypical Klippel-Trenaunay Syndrome.
Note that KTS can either affect blood vessels, lymph vessels, or both. The condition most commonly presents with a mixture of the two. Those with veinous involvements are subject to an overall harder lifestyle due to the increased pain and complications.
The birth defect affects men and women equally, and is not limited to any racial group. It not certain if it is genetic in nature, although testing is ongoing.[7] There is some evidence that it may be associated with a translocation at t(8;14)(q22.3;q13).[8] Some researchers have suggested VG5Q has an association.[9]
KTS is a complex syndrome, and no single treatment is applicable for everyone. Treatment is decided on a case-by-case basis with the individual’s doctors.
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