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The Authors describe a case of Klippel-Trénaunay Weber Syndrome in a 12 year old girl admitted in the Department of cardio vascular surgery of the "Nuovo Ospedale San Giovanni di Dio"; various flat angiomas were present in her lower limbs from birth, which increased in size with body growth; marble skin, teleangectasica edema were also present. Her lower left limb was asymmetric, no angiomas were present in the internal organs. It is the first case in her family, no minor signs of the syndrome were present in her parents.
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