Multimodality Imaging of Hemangiectasia Hypertrophicans (Klippel-Trenaunay-Parkes-Weber Syndrome)
A 64-year-old man with known congenital arteriovenous malformations of the left leg was referred to the department of angiology because of aggravated leg pain and non-healing skin ulceration over the medial ankle. He had suffered recurrent skin ulcerations since the age of 10. Inspection showed hyperpigmentation of the skin of the calf, extensive varicose veins of the left leg, a length difference of 3 cm in the legs, and a difference of the femoral circumference of 7 cm. The ankle-brachial index was normal on the right leg but was decreased to 0.6 on the left. Color-coded duplex sonography showed convoluted arteriovenous shunts in the femoral and popliteal region with a volume of up to 2.500 mL/min. To evaluate potential therapeutic strategies, we performed contrast-enhanced 3-dimensional magnetic resonance angiography (Figure 1A), multislice computed tomography angiography (Figure 1B), and selective intra-arterial digital subtraction angiography (Figure 1C). An additional x-ray of the left calf showed diffuse osteolysis of the tibia.