Abstract
Klippel-Trenaunay syndrome is a congenital vascular anomaly consisting of the clinical triad of port-wine nevus (cutaneous capillary hemangloma), with varicose veins on the lateral aspect and osseous and soft tissue hypertrophy of the affected extremity. Klippel-Trenaunay-Weber syndrome or Parkes-Weber syndrome refers to the same triad plus arteriovenous fistulas/ectasia in the affected extremity. Involvement is most commonly unilateral (85%) and in a lower extremity (95%). Associated findings include hemanglomas of the liver, spleen, colon, bladder, and spine, as well as lymphanglomas of limbs and trunk.
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