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Syndrome, Klippel-Trenaunay-Weber (KTW)

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Definition

A congenital malformationSyndrome, Klippel-Trenaunay-Weber (KTW): A congenital malformationsyndrome characterized by the triad of asymmetric limb hypertrophy, hemangiomata, andnevi. "Asymmetric limb hypertrophy" is enlargement of one limb and not thecorresponding limb on the other side, the enlarged limb being 3 times more likely to be aleg than an arm in KTW; and the limb enlargement is of bone as well as soft tissue. Thehemangiomas, abnormal nests of blood vessels that proliferate inappropriately andexcessively, cover a remarkable range from small innocuous capillary hemangiomas("strawberry marks") to huge cavernous hemangiomas. The nevi are pigmented moles on the skin; in KTW there are often also dark linear streaks on the skin, streaks due totoo much pigment. There can be other abnormalities but the triad is the consistentclinical centerpiece of the disease. Most persons with KTW have an enlarged leg and dorelatively well without treatment or, for example, with only compression from an elasticstocking. Skin ulcers and other skin problems can occur over the swollen leg. Usually, thetreatment is conservative. Surgery is almost never needed. The only possible exceptionsare the very rare situations in which the leg reaches gigantic proportions or secondaryclotting difficulties arise (due to trapping and destruction of blood platelets in a hugehemangioma). Then, amputation may become necessary. The cause of KTW syndrome is unknown.


 

 

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