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.