Klippel-Trenaunay Syndrome

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  • Consists of
    • skin port-wine stain (usually on a limb),
    • limb overgrowth,
    • venous abnormalities in the overgrown limb
      • most notably varicose veins.
  • Mainly a venous anomalies
  • AVMs and fistulas are not part of this disorder, for which no genetic cause is known (in contrast with Parkes-Weber and CM-AVM Syndrome).
  • It is probably incorrect to consider KT in the differential diagnosis of spine or brain arteriovenous shunts.
  • However, some believe that Sturge-Weber Syndrome and KT Syndrome are expressions of the same disease involving either the brain or the extremities.