eMedicine Specialties > Dermatology > Diseases of the Vessels

Klippel-Trenaunay-Weber Syndrome: Treatment & Medication

Author: Camila K Janniger, MD, Clinical Professor of Dermatology, Clinical Associate Professor of Pediatrics, Chief of Pediatric Dermatology, New Jersey Medical School
Contributor Information and Disclosures

Updated: Jun 2, 2009

Treatment

Medical Care

Management of Klippel-Trenaunay-Weber syndrome (KTWS) can be divided into medical and surgical interventions.

  • Treatment is conservative and symptomatic. Compression garments are indicated for chronic venous insufficiency, lymphedema, recurrent cellulitis, and recurrent bleeding from capillary or venous malformations of the extremity. The compression garment may also protect the limb from trauma. Intermittent pneumatic compression pumps may also provide benefit. However, in some patients with absent or hypoplastic deep venous systems, elastic compression may increase venous stasis and cause discomfort. Pain management can be a very important aspect of caring for patients with KTWS. Referral to a pain clinic and/or a multidisciplinary team including a pain management specialist is recommended.13
  • Cellulitis and thrombophlebitis can be managed with analgesics, elevation, antibiotics, and corticosteroids. In patients with a history of recurrent cellulitis, intermittent or prophylactic antibiotics may be considered. Anticoagulant therapy is indicated in acute thrombosis and prophylactically prior to surgical procedures. Given the risk of thrombotic events, women with KTWS should avoid using oral contraceptive pills.
  • Regarding limb hypertrophy, heel inserts are generally sufficient for limb discrepancies of 1.5 cm or less. For greater discrepancies, orthopedic surgery may be considered. Possible orthopedic procedures include osteotomy, epiphysiodesis, or epiphyseal stapling. Rarely, amputation is required due to recurrent infections, nonhealing ulcers, or recurrent bleeding.
  • Women with KTWS have been reported to have normal pregnancies. These patients should be monitored carefully with serial ultrasounds because previously asymptomatic arteriovenous malformations within the uterine wall may become pronounced with the additional blood flow to the uterus during pregnancy.14,15

Surgical Care

  • Laser treatment of the hemangioma can be effective in lightening the color of the port-wine stain. Currently, the flashlamp-pumped pulsed dye laser is the treatment of choice in vascular lesions. Laser treatment is also indicated in the case of ulceration. Ulceration of hemangiomas can be painful and can impair functional abilities. When treated with laser, ulcers often heal more quickly. Laser treatment is most effective when performed early, as it can improve the long-term appearance of the port-wine stain and thereby also improve function. Typically, many treatments are required to achieve the desired effect. Laser treatment only helps with the superficial component of the hemangioma.16
  • Surgical intervention in the treatment of varicosities and venous malformations is controversial. One might consider surgery for either significant cosmetic deformity or the symptoms of pain, heaviness of the leg, bleeding, or infectious complications. Venous stripping, ligation, excision, or sclerotherapy are contraindicated unless the surgery involves the superficial system and the underlying deep system is normal or demonstrates only mild-to-moderate reflux.
  • Inadequate evaluation prior to excision increases surgical complications. Symptomatic superficial varicosities can be removed without harm and with benefit to the patient when an adequate preoperative examination is performed. Although Baskerville et al demonstrated that some 90% of treated varicosities redevelop, treatment can provide lasting improvement for years.3 Successful treatment of incompetent valves in the femoral vein of the affected limb with contralateral saphenous vein transplant has been reported.
  • Debulking procedures have limited use and may damage venous and lymphatic structures, leading to increased edema in the affected limb. The potential risks and benefits must be carefully weighed before attempting surgical intervention.
  • Radiotherapy has been reported to be of help in some cases of KTWS. The radiation may help to induce regression of hemangiomas; however, the results can be slow to develop.17
  • Endovenous laser therapy of the greater saphenous vein is gaining support for the management of varicosities in the general public and in patients with KTWS.18  This therapy has been used alone and in combination with other surgical interventions. It is a novel and minimally invasive approach for the management of some varicosities.

More on Klippel-Trenaunay-Weber Syndrome

Overview: Klippel-Trenaunay-Weber Syndrome
Differential Diagnoses & Workup: Klippel-Trenaunay-Weber Syndrome
Treatment & Medication: Klippel-Trenaunay-Weber Syndrome
Follow-up: Klippel-Trenaunay-Weber Syndrome
References
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References

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Further Reading

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Keywords

Klippel-Trenaunay-Weber syndrome, KTWS, Parkes Weber syndrome, Klippel-Trenaunay syndrome, KTS, port-wine stain, varicose veins, bony and soft tissue hypertrophy, arteriovenous malformation

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Contributor Information and Disclosures

Author

Camila K Janniger, MD, Clinical Professor of Dermatology, Clinical Associate Professor of Pediatrics, Chief of Pediatric Dermatology, New Jersey Medical School
Camila K Janniger, MD is a member of the following medical societies: American Academy of Dermatology
Disclosure: Nothing to disclose.

Medical Editor

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 and American Dermatological Association
Disclosure: Nothing to disclose.

Pharmacy Editor

Michael J Wells, MD, Associate Professor, Department of Dermatology, Texas Tech University Health Sciences Center
Michael J Wells, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, and Texas Medical Association
Disclosure: Nothing to disclose.

Managing Editor

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, and Texas Medical Association
Disclosure: Nothing to disclose.

CME Editor

Joel M Gelfand, MD, MSCE, Medical Director, Clinical Studies Unit, Assistant Professor, Department of Dermatology, Associate Scholar, Center for Clinical Epidemiology and Biostatistics, University of Pennsylvania
Joel M Gelfand, MD, MSCE is a member of the following medical societies: Society for Investigative Dermatology
Disclosure: AMGEN Consulting fee Consulting; AMGEN Grant/research funds None; Genentech Consulting fee Consulting; Centocor Consulting fee Consulting; Centocor Grant/research funds None; Covance Consulting fee Consulting; Shire  Consulting

Chief Editor

Dirk M Elston, MD, Director, Department of Dermatology, Geisinger Medical Center
Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology
Disclosure: Nothing to disclose.

 
 
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