The media have called it "a degenerative leg ailment" and "a debilitating leg condition." Both "Klippel-Trenaunay syndrome" and "Klippel-Trenaunay-Weber syndrome" are used interchangeably by broadcasters and writers. The proper name is Klippel-Trenaunay-Weber syndrome (KTW), now called Parkes-Weber syndrome. Although debilitating and painful, many people who have it do well with little treatment. Surgery is only needed in severe cases.
How it starts People are born with KTW. Researchers aren't sure why it happens, because it doesn't seem to be passed down genetically. Usually the first sign noticed is a large port wine stain. This is a problem with the tiny blood vessels (capillaries) under the skin, which causes the skin to turn dark red or even purple, be raised up, and there may be bumps on it that bleed easily. Most often the port wine stain is large and since KTW usually affects a leg (three times more likely than other parts), the birthmark covers, for example, from buttock and hip to toe on the affected side of the body.
How it progresses KTW causes other blood vessel problems in the leg it affects. The blood vessels grow excessively and form tangled bunches (hemangiomas), which can range from small and not very noticeable to huge masses. There can be varicose veins and twisted vessels. Sometimes hemangiomas may grow inside the body, in the internal organs.
Another problem KTW causes is the overgrowth of the soft tissues and bones in the leg (hypertrophy). Sometimes this happens in the leg without the blood vessel problems. It is this overgrowth that makes it hard to use and move the leg. The toes may also be affected.
The result The blood vessel problems and the overgrowth lead to bleeding, pain, skin infections (cellulitis), difficulty walking, and blood clots (which may cut off blood circulation in the leg, or travel to other parts of the body and cause damage). Each person with KTW is affected in his or her own way, and the problems experienced may be only mildly uncomfortable to severely disabling.
Treatment There is no cure for KTW. Many people with KTW are helped by simple treatments such as elastic compression stockings, which reduce pain and swelling. Laser therapy can reduce or eliminate the port wine stain. Sometimes surgery is needed to remove a large hemangioma or to remove excess tissue from a leg that is badly overgrown. In rare instances, the affected leg may need to be amputated because of blood clots or severe overgrowth.
Information provided on AnswerPond.com is not intended to substitute for informed expert medical, psychiatric, psychological, tax, legal, computer, investment, accounting, or other professional advice. For questions that require professional advice, please consult a qualified provider who is licensed in your state or country.
