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Klippel-Trenaunay-Weber Syndrome Workup

  • Author: Camila K Janniger, MD; Chief Editor: Dirk M Elston, MD  more...
Updated: Jun 22, 2016

Imaging Studies

In many instances, a thorough history and physical examination are all that is required to diagnose Klippel-Trenaunay-Weber syndrome (KTWS). However, when complications are present, imaging studies can be useful. Color Doppler sonography is an accurate, reliable, and noninvasive way to evaluate patients with possible KTWS.[25]

Multidetector row computed tomography arteriography may be of value in the preoperative assessment of patients with KTWS.[26]

Evaluation of the deep venous system can be completed with duplex scanning contrast venography, ultrasonography, contrast venography and arteriography, and nuclear MRI studies. Arteriography is especially helpful in the diagnosis of an arteriovenous fistula.

MRI is also helpful in imaging the soft tissue hypertrophy. In addition, magnetic resonance angiography can be very helpful in identifying and defining vascular malformations.

In the case of major limb length discrepancies, serial radiographic studies, including but not limited to scanograms, orthoroentgenograms, and CT scans, for measurement of limb length are necessary. Clinical measurements can only guarantee measurement within 0.5-1.0 cm, whereas radiographic examinations can be used to determine the exact differences to within 0.1 cm. These studies help to determine how fast a limb is growing and may help in determining proper timing for limb length equalization procedures.

Prenatal diagnosis by ultrasonography has been reported.

In lesions extending onto the perineum or abdomen, performing imaging studies can be helpful to rule out internal involvement. Vascular malformations have been reported throughout the gastrointestinal tract. Although this typically does not cause symptoms, gastrointestinal bleeding has been reported. Similarly, vascular malformations have also been reported within the genitourinary tract.

Contributor Information and Disclosures

Camila K Janniger, MD Clinical Professor of Dermatology, Clinical Associate Professor of Pediatrics, Chief of Pediatric Dermatology, Rutgers New Jersey Medical School

Camila K Janniger, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

Specialty Editor Board

Michael J Wells, MD, FAAD Associate Professor, Department of Dermatology, Texas Tech University Health Sciences Center, Paul L Foster School of Medicine

Michael J Wells, MD, FAAD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, Texas Medical Association

Disclosure: Nothing to disclose.

Lester F Libow, MD Dermatopathologist, South Texas Dermatopathology Laboratory

Lester F Libow, MD is a member of the following medical societies: American Academy of Dermatology, American Society of Dermatopathology, Texas Medical Association

Disclosure: Nothing to disclose.

Chief Editor

Dirk M Elston, MD Professor and Chairman, Department of Dermatology and Dermatologic Surgery, Medical University of South Carolina College of Medicine

Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

Additional Contributors

Jean Paul Ortonne, MD Chair, Department of Dermatology, Professor, Hospital L'Archet, Nice University, France

Jean Paul Ortonne, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.


The authors and editors of Medscape Reference gratefully acknowledge the contributions of previous author, Jane H. Lisko, MD, and Frederick Fish, MD, to the development and writing of this article.

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Klippel-Trenaunay syndrome in a young person. Note the port-wine stain extending to the buttocks. These lesions can be associated with venous malformations involving the rectum and bladder.
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