Multimodality imaging approach in a patient with Klippel-Trenaunay syndrome

Klippel-Trenaunay syndrome (KTS) is a rare congenital disorder presenting with asymmetric limb hypertrophy, cutaneous capillary malformations and lower extremity varicosities. We discuss a 27-year-old man born with varicosities on both lower extremities, which progressively enlarged. Physical examination showed a grossly enlarged right hand. There were multiple compressible varicosities, diffuse port-wine stains on the right leg and limb-length discrepancy on the left leg. CT angiogram and Doppler ultrasound revealed several venous varicosities. Ectatic veins in the right leg converge into the lateral marginal vein of Servelle, an embryonic vein, typically seen in KTS patients. KTS is diagnosed clinically and imaging plays a role in differentiating this from other disease entities that present similarly. Doppler ultrasound is the initial imaging of choice to characterise varicosities and to identify thrombosis and reflux. Plain radiographs confirm limb hypertrophy. MRI and CT angiograms are useful to evaluate vascular anomalies and its accompanying soft tissue changes.