Cephalic Fibromuscular Dysplasia in 32 Patients: Clinical Findings and Radiologic Features

The clinical and angiographic features of 32 patients with cephalic fibromuscular dysplasia (FMD) are reported. All of our patients were women, 78% of whom were between 41 and 70 years of age. At examination, 18 (56%) patients had the sudden onset of focal ischemic neurologic deficits. Seven (22%) patients had intracranial berry aneurysms, which ruptured in five patients but were asymptomatic in two. The most common angiographic pattern was the “string of beads” deformity, which involved both extracranial internal carotid arteries at or distal to the third cervical vertebral level. The vertebral artery was affected in six cases, while three cases had intracranial involvement. Less common angiographic findings in this series consisted of segmental fusiform dilation of the artery, and lesions in the form of a septum that extended across the lumen. Twelve patients were followed up for an average of four years. Progression of FMD lesions was shown in two of the six patients who had repeated angiograms. The literature contains reports of only nine cases of cephalic FMD with repeated angiograms, three of which demonstrated progression. Our experience suggests a beneficial role for surgery in patients with specific symptoms in the distribution of the affected vessel, when coexistent disease is negligible or absent.