What is Klippel Trenaunay?

Varied in its presentation but is classically described as an enlarged extremity on one side of the body with an overlying port-wine stain. Classically, there is an underlying vascular tumor comprised of veins or lymphatic vessels. Excessive bone growth in the involved limbs may occur as well.

What causes Klippel Trenaunay?

There is no clear evidence at this time of this syndrome being an inheritable disorder.

What is the "life cycle" of a Klippel Trenaunay?

This bony enlargement usually is not present at birth, but may appear within the first few months or years of life. It may become a problem particularly during puberty. The abnormality may involve single or multiple extremities as well as the face or trunk. There may be periods of rapid enlargement and then cessation of growth. Occasionally, there may be abdominal and pelvic involvement.

Symptoms vary with the severity of the dominant vascular component:

Soft tissue swelling and enlargement can be seen if lymphatic anomalies are dominant.

Intermittent episodes of painful thrombosis (clotting) can be expected if venous malformations are dominant.

Possible infections causing redness, and irritation. These must be treated immediately with an aggressive course of antibiotics.

Rectal and urinary tract bleeding may be seen in rare instances if gastrointestinal lesions are involved.

How is a Klippel Trenaunay diagnosed?

MRI and CT Scans may outline the extent of involvement but surgical debulking is beneficial only in rare cases. Indications for radiologic studies of organs and bones are best decided by clinical evaluation.

How is a Klippel Trenaunay treated?

There is no total cure for Klippel-Trenaunay Syndrome.

Sequential pneumatic tourniquet & compression garments: may be used to help with symptomatic control of swelling.

Surgery:
Sclerotherapy: Recently, advances have been made with radiologic injection of an irritating solution into abnormal veins with subsequent reduction in the size of the vessels. This may be repeated and although not a permanent solution, it remains in many instances the best treatment. This may be used in conjunction prior to surgery.

Is there any support for the children or families?

Children's Medical Center of Dallas offers various areas of support to children and their families. A social worker is available if families have questions regarding the family's ability to optimally meet the child's special health care needs. Also there is spiritual support available to patients and families on a 24-hour basis through Pastoral Care. If you have any questions, please contact our office at (214) 456-8888.

Be very careful when utilizing the Internet to gain insight into your child's condition. The Internet, while being very informative, can be equally uninformative. The Internet can provide you with information on your child's condition, but it does not address the needs of your particular child. Each child is unique and no two children follow the same course. The Internet will typically show you the most spectacular (and usually worst) cases for "shock value". Individual evaluation by a specialist is critical.

The Fogelson Plastic Surgery &
Craniofacial Center for Children
Children's Medical Center of Dallas
6300 Harry Hines Boulevard
Suite 600
Dallas, Texas 75235