Fibromuscular dysplasia of the internal carotid artery: long-term surgical results

Fifty-eight patients underwent 72 operations for symptomatic fibromuscular dysplasia (FMD) between 1970 and 1986. There were 35 females and 23 males aged between 36 and 76 years (average 56). Among the 72 operated on lesions (11 bilateral) FMD stenotic lesions (string of beads, tubular, focal) were isolated (32) or associated with elongation (tortuosity, coiling, kink) in 24 cases, FDM aneurysms (7), and dissecting pseudoaneurysms (9). The surgical techniques included graduated or balloon intra-luminal dilatation either isolated (29) or associated with resection-anastomosis (35), saphenous graft (4) and reconstructive aneurysmorrhaphy (4). Bifurcation endarterectomy was combined in 14 patients. There was no hospital death. There were 2 neurologic deficits after operation (1 transient) and 12 transient nervous disorders due to dissection near the base of the skull required in one third of the cases. The follow-up period extends from 6 to 22 years. Five patients were lost to follow-up. Out of the 53 remaining patients, 44 (83%) are living and asymptomatic. Nine patients died: 4 from myocardial infarction, 3 from cancer, 2 from neurologic disease. Three late successful reoperations were observed: 1 aneurysm formation following graduated dilatation, 2 anastomotic stenosis. Surgical intraluminal dilatation either isolated or combined with reconstructive techniques is a safe and durable operation relieving symptoms. The benefits of repair are long lasting and should be offered to patients with symptomatic FMD carotid lesions. The medical management of asymptomatic cases allows to study the natural history of the disease whose causes are not so far fully known.