Klippel-Trenaunay and Parkes-Weber Syndrome

Also known as: Klippel-Trenaunay-Weber Syndrome

What is Klippel-Trenaunay and Parkes-Weber Syndrome?

This is a rare congenital medical condition of abnormal blood and lymph vessel formation, cutaneous capillary malformation, and skeletal or soft tissue hypertrophy.

Diagnosis is made clinically based on one or more distinct symptoms. These associated symptoms include the following:

  • port-wine stains with sharp borders
  • varicose veins
  • hypertrophy of bone and soft tissues with local limb enlargement or shrinking
  • an improperly developed lymph system that may lead to local limb swelling
Pain and difficulty walking may occur and, in more severe cases, the limb hypertrophy may be so severe as to prompt amputation. Though cases vary, symptoms affect approximately ¼ of the body.

Treatment for Klippel-Trenaunay and Parkes-Weber Syndrome

Treatment for this condition is complex and should be approached on an individual, case-by-case manner. Treatment is usually focused on increasing quality of life and decreasing physical deformities. Surgical debulking may be indicated for severe cases. Non-surgical options are also available and include sclerotherapy to potentially close large vascular malformations and compression garments to alleviate pain and inflammation.

This page was last updated on: December 18, 2020 05:00 PM

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