Klippel-Trenaunay-Weber Syndrome (cont.)
How is intelligence affected?
Intelligence is usually not impaired in Klippel-Trènaunay-Weber. However, when there are
hemangiomas on the face (facial hemangiomatosis), there may also be hemangiomas
in the brain which can cause
seizures (convulsions) or
mental retardation.
Fortunately, such problems are rare in Klippel-Trènaunay-Weber.
What is the basic defect in Klippel-Trènaunay-Weber
syndrome?
It is not completely known, but some researches believe part of the defect is
related to the control of angiogenic cells (precursors of blood vessels). Others
believe it is caused by some injury to the developing embryo within the womb,
resulting in venous compression and resultant abnormal venous pressures, causing
varicosities and limb enlargement. In other words, we don't currently know the
precise events that lead to the development of Klippel-Trènaunay-Weber.
What causes Klippel-Trènaunay-Weber
syndrome?
Most cases of Klippel-Trènaunay-Weber are sporadic. They occur without warning, with no prior
case in the family; however recently there have been some cases that run in
families.
What is the prognosis (outlook) with Klippel-Trènaunay-Weber
syndrome?
Most persons with Klippel-Trènaunay-Weber have an enlarged leg and they do relatively well
without any significant treatment. It can be helpful to wear compression
stockings to prevent venous pooling in the affected extremity (varicose vein
management). However, some Klippel-Trènaunay-Weber patients do have considerable pain. Skin ulcers,
infections and other skin problems can occur, but usually the treatment is
conservative. Surgery is almost never needed.
Next: Why is it called Klippel-Trènaunay-Weber syndrome? »
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