Genetics of Klippel-Trenaunay-Weber Syndrome Treatment & Management

  • Author: Bruce Buehler, MD; Chief Editor: Bruce Buehler, MD   more...
 
Updated: Apr 16, 2012
 

Medical Care

  • Most patients with Klippel-Trenaunay syndrome (KTS) can be conservatively treated with compression stockings or pneumatic pumps. Compression stockings decrease edema, act as a barrier for minor trauma, and reduce venous insufficiency.
  • In most series, patients with thrombophlebitis have been acutely treated without long-term prophylactic anticoagulants. However, aspirin is probably indicated in all patients.
  • Monitor cardiac status in all patients with arteriovenous malformations (AVMs).
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Surgical Care

  • Servelle reported successful surgical intervention (resection or ligation of abnormal blood vessels) in more than 700 patients with Klippel-Trenaunay syndrome.[6] Most medical centers have tried to avoid surgical intervention. Surgical treatment can be complicated by infection, lymph seepage, and skin breakdown. In a series by the Mayo Clinic, surgical ligation and stripping of varicose veins produced improvement in only 40% of patients.[7] Venous varicosities recur after surgery in 90% of patients. Intravenous sclerotherapy has been proposed as an alternative to surgical intervention in Klippel-Trenaunay syndrome and to embolization in Parkes Weber syndrome. Reports have detailed the use of a sclerosant in microfoam.
  • Clinicians at all centers agree that a leg length discrepancy of more than 2 cm warrants epiphysiodesis. Hypertrophied digits with severe deformity and infection may require amputation.
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Consultations

  • Psychologist: Psychological support is important because of the cosmetic effects of Klippel-Trenaunay syndrome and Parkes Weber syndrome. A lay support group, the Klippel-Trenaunay Syndrome Support Group, is available.
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Activity

  • Patient activities are as tolerated.
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Contributor Information and Disclosures
Author

Bruce Buehler, MD  Professor, Department of Pediatrics and Genetics, Director RSA, University of Nebraska Medical Center

Bruce Buehler, MD is a member of the following medical societies: American Academy for Cerebral Palsy and Developmental Medicine, American Academy of Pediatrics, American Association on Mental Retardation, American College of Medical Genetics, American College of Physician Executives, American Medical Association, and Nebraska Medical Association

Disclosure: Nothing to disclose.

Specialty Editor Board

Michael Fasullo, PhD  Senior Scientist, Ordway Research Institute; Associate Professor, State University of New York at Albany; Adjunct Associate Professor, Center for Immunology and Microbial Disease, Albany Medical College

Michael Fasullo, PhD is a member of the following medical societies: American Society for Biochemistry and Molecular Biology, Environmental Mutagen Society, Genetics Society of America, and Radiation Research Society

Disclosure: Nothing to disclose.

Mary L Windle, PharmD  Adjunct Associate Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Nothing to disclose.

Margaret M McGovern, MD, PhD  Professor and Chair of Pediatrics, Stony Brook University, New York

Margaret M McGovern, MD, PhD is a member of the following medical societies: American Academy of Pediatrics and American Society of Human Genetics

Disclosure: Genzyme Grant/research funds PI

Paul D Petry, DO, FACOP, FAAP  Consulting Staff, Freeman Pediatric Care, Freeman Health System

Paul D Petry, DO, FACOP, FAAP is a member of the following medical societies: American Academy of Osteopathy, American Academy of Pediatrics, American College of Osteopathic Pediatricians, and American Osteopathic Association

Disclosure: Nothing to disclose.

Chief Editor

Bruce Buehler, MD  Professor, Department of Pediatrics and Genetics, Director RSA, University of Nebraska Medical Center

Bruce Buehler, MD is a member of the following medical societies: American Academy for Cerebral Palsy and Developmental Medicine, American Academy of Pediatrics, American Association on Mental Retardation, American College of Medical Genetics, American College of Physician Executives, American Medical Association, and Nebraska Medical Association

Disclosure: Nothing to disclose.

Additional Contributors

The authors and editors of eMedicine gratefully acknowledge the contributions of previous author James H Tonsgard, MD, to the original writing and development of this article.

References

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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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