Genetics of Klippel-Trenaunay-Weber Syndrome Treatment & Management

Updated: Aug 29, 2016
  • Author: Ravi Sunderkrishnan, MD; Chief Editor: Maria Descartes, MD  more...
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Treatment

Medical Care

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  • 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

There are subtle differences between the surgical interventions for Klippel-Trenaunay syndrome and Parkes Weber syndrome. 

Servelle reported successful surgical intervention (resection or ligation of abnormal blood vessels) in more than 700 patients with Klippel-Trenaunay syndrome. [15] 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. [16] Venous varicosities recur after surgery in 90% of patients. Intravenous sclerotherapy has been proposed as an alternative to surgical intervention.

Clinicians at all centers agree that a leg-length discrepancy of more than 2 cm warrants epiphysiodesis in Klippel-Trenaunay syndrome. However, it is not preferred in Parkes Weber syndrome, since it can exacerbate the fast-flow lesions, especially if near the knee. [14]

Debulking surgeries to remove excess girth, while possible in Klippel-Trenaunay syndrome, are best avoided in Parkes Weber syndrome due to the arteriovenous malformations.

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Consultations

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  • 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

 

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  • Patient activities are as tolerated.
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