Abstract: Klippel-Trenaunay syndrome (KTS) is a rare congenital vascular disorder with three clinical features: port-wine stain, abnormal overgrowth of soft tissues and bones, and venous/lymphatic malformations. The etiology and pathogenesis remain unknown. Symptoms and severity of KTS vary greatly among each individual. The goals of treatment are to alleviate symptoms and minimize the risk of complications. This article reviewed the clinical presentation and diagnostic studies, and provided management guidelines to help care for patients with this syndrome.

Key words: Klippel-Trenaunay syndrome, Overgrowth of limb, Clinical practice guidelines