[Klippel-Trenaunay syndrome].
Klippel-Trenaunay syndrome (KTS) is a rare complex vascular congenital malformation. The characteristic triad is an association of a cutaneous capillary angioma of a limb, venous malformations, and hypertrophy of soft tissue and/or bone. Diagnosis is essentially clinical. Work-up of the lesion may involve noninvasive imaging: Doppler ultrasound, standard radiography, or magnetic resonance imaging (MRI). The presence of arteriovenous malformations is sought by clinical examination or ultrasound: they rule out a diagnosis of KTS. Management is multidisciplinary and involves especially venous control and orthopedic management of unequal limb lengths.
Samimi
Mahtab
M
Université François-Rabelais de Tours, BP 3223, 37032 Tours cedex 1, France. samimi.mahtab@yahoo.fr
Lorette
Gérard
G
D001165:Arteriovenous Malformations / Q000175:diagnosis; D003937:Diagnosis, Differential; D006391:Hemangioma / Q000503:physiopathology; D006801:Humans; D006984:Hypertrophy; D007715:Klippel-Trenaunay-Weber Syndrome / Q000175:diagnosis* / Q000503:physiopathology / Q000628:therapy; D008279:Magnetic Resonance Imaging; D018608:Ultrasonography, Doppler; D054079:Vascular Malformations / Q000503:physiopathology