Klippel-Trenaunay syndrome

• Alternative Names

  Klippel-Trenaunay-Weber syndrome; KTS; Angio-osteohypertrophy; Nevus varicosus osteohypertrophicus syndrome; Hemangiectasia hypertrophicans; Nevus verucosus hypertrophicans

• Causes, incidence, and risk factors

  Most cases of Klippel-Trenaunay syndrome occur for no apparent reason. However, a few cases are thought to be passed down through families (inherited), possibly as an autosomal dominant trait.

• Symptoms
  • Many port wine stains or other blood vessel problems, including dark spots on the skin.
  • Varicose veins (may be seen in early infancy, but are more likely to be seen later in childhood or adolescence)

  Other possible symptoms:

  • Bleeding from the rectum
  • Blood in the urine
• Signs and tests

  Persons with this condition may have excessive growth of bones and soft tissue. This occurs most commonly in the legs, but it also may affect the arms, face, head, or internal organs.

• Support Groups

  It may be helpful to join a support group in which members share common problems and concerns.

  The following organizations provide further information on Klippel-Trenaunay syndrome:

  • The Klippel-Trenaunay Syndrome Support Group --www.k-t.org
  • Vascular Birthmarks Foundation -- www.birthmark.org
• Expectations (prognosis)

  Most individuals with Klippel-Trenaunay syndrome do well, despite their cosmetic appearance. However, there can be related psychological problems.

• References

  Garzon M, Huang J, Enjolras O, Frieden I. Vascular malformations. Part II: associated syndromes. J Am Acad Derm. April 2007; 56(4): 541-64.

Review Date: 10/14/2009

Reviewed By: Luc Jasmin, MD, PhD, Departments of Anatomy and Neurological Surgery, University of California, San Francisco, CA. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.