[Klippel-Trenaunay syndrome]

Abstract

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.