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Klippel-Trenaunay syndrome

Definition

Klippel-Trenaunay syndrome is a rare condition that is typically present at birth. The syndrome usually involves port wine stains, excess growth of bones and soft tissue, and varicose veins.

Alternative Names

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

Causes

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

Exams 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:

Outlook (Prognosis)

Most people with Klippel-Trenaunay syndrome do well, although the condition may affect their appearance. Some people have psychological problems from the condition.

There can sometimes be abnormal blood vessels in the abdomen, which may need to be evaluated.

References

James WD, Berger TG, Elston DM. Andrews' Diseases of the Skin: Clinical Dermatology. 11th ed. Philadelphia: Pa; 2011:chap 28.

Morelli JG. Vascular disorders. In: Kliegman RM, Stanton BF, St. Geme III JW, Schor NF, Behrman RE, eds. Nelson Textbook of Pediatrics. 19th ed. Philadelphia, Pa: Elsevier Saunders; 2011:chap 642.


Review Date: 10/29/2013
Reviewed By: Chad Haldeman-Englert, MD, FACMG, Wake Forest School of Medicine, Department of Pediatrics, Section on Medical Genetics, Winston-Salem, NC. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Bethanne Black, and the A.D.A.M. Editorial team.
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