Klippel-Trenaunay Syndrome

Klippel Trenaunay syndrome is a rare and complex combined vascular malformation that is present at birth. By definition, it includes evidence of malformations involving every vascular component except arteries. In addition, overgrowth of the involved extremity is common.

Localized or diffuse capillary malformations (port-wine stains) can overly areas of venous and lymphatic malformations. Overgrowth of the limb will include all tissue types including muscle, bone, joints, and nerves. Internal organs may also be affected.

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Child with Klippel-Trenaunay syndrome of the chest and arm.
Child with Klippel-Trenaunay
syndrome of the chest and arm.

As with other types of vascular malformation, treatment is typically directed at symptoms that are present:

  • Frequent pain
  • Disfigurement
  • Infection
  • Ulceration
  • Limitation of movement or decreased function

Treatment can include:

  • Compression garments
  • Aspirin
  • Sclerotherapy
  • Surgery

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