Drei-Quadranten-Syndrom bei Klippel-Trenaunay-Syndrom und primären Lymphödemen beider Beine

A 34-year-old woman with extensive Klippel-Trenaunay syndrome is presented. Clinical and radiological tests demonstrated that three quadrants are affected with haemangiomas, varicose veins and bone abnormalities. In addition, our patient suffers from congenital lymphoedema of both legs. Associated findings such as intestinal haemangiomata, vascular hamartomas, lipomas or arteriovenous abnormalities of the spinal cord were not apparent. The treatment is only symptomatic with compression bandages and an orthopaedic appliance for compensation of the different leg length.