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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.