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Klippel-Trenaunay syndrome

Definition:

Klippel-Trenaunay syndrome is a rare condition that is 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, 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.



Treatment:



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:



Expectations (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 need to be evaluated.



Complications:



Calling your health care provider:



Prevention:



References:

Garzon M, Huang J, Enjolras O, Frieden I. Vascular malformations. Part II: associated syndromes. J Am Acad Derm. 2007;56(4):541-564.




Review Date: 11/14/2011
Reviewed By: Chad Haldeman-Englert, MD, 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, Medical Director, A.D.A.M., Inc.

The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition. A licensed medical professional should be consulted for diagnosis and treatment of any and all medical conditions. Call 911 for all medical emergencies. Links to other sites are provided for information only -- they do not constitute endorsements of those other sites. © 1997- A.D.A.M., Inc. Any duplication or distribution of the information contained herein is strictly prohibited.
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