Genetics of Klippel-Trenaunay-Weber Syndrome Follow-up

Updated: Sep 17, 2018
  • Author: Ravi Sunderkrishnan, MD; Chief Editor: Maria Descartes, MD  more...
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Follow-up

Complications

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  • For complications of Klippel-Trenaunay-Weber syndrome, see Clinical.

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Prognosis

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  • Recognition of the spectrum of severity is important, both in venous disease and in limb hypertrophy.

    • Jacob et al found that the rate of progression of limb length discrepancy was neither uniform nor predictable. [20] In Jacob et al's series, only 11% of patients had hypertrophy severe enough to warrant epiphysiodesis.

    • Amputation of an extremity is rarely necessary.

    • Patients with Klippel-Trenaunay syndrome (KTS) face a lifetime of potential problems, including cellulitis, lymph seepage, gangrene, skin breakdown, thrombophlebitis, and internal and superficial hemorrhage.

  • Parkes Weber syndrome presents more severe problems, with hypertrophy, lymphedema, and cardiac hypertrophy. In one study by Robertson, 1 of 28 patients died from cardiac failure, and 5 of 28 required amputation of an extremity. [8]

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Patient Education

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