Klippel-Trenaunay syndrome with review of literature

Klippel-Trenaunay syndrome (KTS) is a rare disorder with an incidence of 3-5/1,00,000. It is characterized by the triad of vascular malformation (capillary hemangioma or port wine stain), venous varicosity and soft tissue and/ or bony hypertrophy. The vascular malformation is usually limited to a single extremity, though multiple extremities can be involved. Alternative names given for Klippel Trenaunay Syndrome are Klippel-Trenaunay-Weber syndrome; Angio-osteohypertrophy; Nevus varicosus osteohypertrophicus syndrome; Hemangiectasia hypertrophicans and Nevus verucosus hypertrophicans.