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NINDS Klippel-Trenaunay Syndrome (KTS) Information Page


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What is Klippel-Trenaunay Syndrome (KTS)?

Klippel-Trenaunay syndrome (KTS) is a rare congenital malformation involving blood and lymph vessels and abnormal growth of soft and bone tissue.  Typical symptoms include hemangiomas (abnormal benign growths on the skin consisting of masses of blood vessels) and varicose veins.  Fused toes or fingers, or extra toes or fingers, may be present.  In some cases, internal bleeding may occur as a result of blood vessel malformations involving organs such as the stomach, rectum, vagina, liver, spleen, bladder, kidneys, lungs, or heart.  Individuals are also at risk for blood clots. The cause of the disorder is unknown. A similar port-wine stain disorder in which individuals have vascular anomalies on the face as well as in the brain is Sturge-Weber syndrome. These individuals may experience seizures and mental deficiency.  In some cases, features of the Klippel-Trenaunay syndrome and Sturge-Weber syndrome coincide.  Another overlapping condition is the Parkes-Weber syndrome, which is characterized by abnormal connectivity between the arterial and venous system (arteriovenous fistulas).

Is there any treatment?

There is no cure for KTS. Treatment is symptomatic. Laser surgery can diminish or erase some skin lesions. Surgery may correct discrepancies in limb size, but orthopedic devices may be more appropriate.

What is the prognosis?

KTS is often a progressive disorder, and complications may be life-threatening.  However, many individuals can live well while managing their symptoms.

What research is being done?

The NINDS supports research on congenital disorders such as KTS with the goal of finding new means to treat and prevent them.

NIH Patient Recruitment for Klippel-Trenaunay Syndrome (KTS) Clinical Trials

Organizations

Column1 Column2
Vascular Birthmarks Foundation
P.O. Box 106
Latham, NY   12110
hvbf@aol.com
http://birthmark.org
Tel: 877-VBF-4646 (823-4646)

Sturge-Weber Foundation
P.O. Box 418
Mt. Freedom, NJ   07970
SWF@sturge-weber.org
http://www.sturge-weber.org
Tel: 973-895-4445 800-627-5482
Fax: 973-895-4846

National Organization for Rare Disorders (NORD)
55 Kenosia Avenue
Danbury, CT   06810
orphan@rarediseases.org
http://www.rarediseases.org
Tel: 203-744-0100 Voice Mail 800-999-NORD (6673)
Fax: 203-798-2291

 


Prepared by:
Office of Communications and Public Liaison
National Institute of Neurological Disorders and Stroke
National Institutes of Health
Bethesda, MD 20892



NINDS health-related material is provided for information purposes only and does not necessarily represent endorsement by or an official position of the National Institute of Neurological Disorders and Stroke or any other Federal agency. Advice on the treatment or care of an individual patient should be obtained through consultation with a physician who has examined that patient or is familiar with that patient's medical history.

All NINDS-prepared information is in the public domain and may be freely copied. Credit to the NINDS or the NIH is appreciated.

Last updated September 30, 2011