[Klippel-Trenaunay syndrome: clinical and surgical aspects]

The Klippel-Trenaunay syndrome is a rare syndrome of uncertain etiology. The characteristic elements are flat angiomatosis, hypertrophy of soft tissue and bone tissue and alterations of the venous system, with the exclusion of hemodynamically significant arteriovenous fistulae. The authors report a clinical case and review the international literature. Treatment is conservative in the majority of cases; surgery is reserved for patients with disabling morphological and functional alterations.