A superficial femoral artery aneurysm in a patient with Marfan's syndrome

Abstract

A 69-year-old man presented with intermittent claudication of his right leg at a distance of 200 yards and recent onset of rest pain in his right foot sufficient to wake him at night, with discolouration of his big toe. He had also noticed a pulsatile lump in his right thigh which had gradually increased in size over the preceding year. He was a non-smoker and there had been no history of injury to the leg. On examination he had a Marfanoid appearance. There was a pulsatile mass on the medial lower aspect of his right thigh with a gangrenous right big toe. All pulses were present except the dorsalis pedis on the right. An echocardiogram demonstrated mild dilatation of the aortic root with thickening of the aortic valve. There was no aortic regurgitation or mitral valve prolapse. Ultrasound examination of the mass on his leg showed an eccentric saccular aneurysm 5 cm across wi th colour flow seen within it on Doppler. An arteriogram was performed via a right femoral artery approach. This demonstrated a large superficial femoral artery (SFA) aneurysm on the right (Fig. 1) with two vessel run-off to the foot, predominant ly from the posterior tibial artery. In addition there was a 4 cm aneurysm of the right common iliac artery seen on the arteriogram and confirmed on contrast enhanced CT of the pelvis (Fig. 2). At operation he was found to have a large distal aneurysm of the superficial femoral artery, lined with thrombus. This was resected and replaced with a reversed long saphenous vein graft. Review of the fragments from the artery by two consultant histopathologists showed severe atheroma with focal mucoid degeneration and elastic fragmentation of the media consistent wi th a diagnosis of Marfan's syndrome. Postoperatively the foot remained well perfused and there were no further embolic episodes, indicating the vein graft had removed the source of embolic showers.