Carotid Artery Fibromuscular Dysplasia

Abstract

This chapter provides a comprehensive review of carotid artery fibromuscular dysplasia (FMD), an idiopathic, segmental, non-inflammatory, non-atherosclerotic vasculopathy characteristically seen in the mid- or distal segments of the internal carotid artery and often diagnosed with non-invasive vascular imaging. Although the etiological risk factors associated with carotid FMD remain largely unclear, recent genomics studies confirm shared associations with cervical artery dissection, migraine, and an inverse association with atherosclerotic disease. Despite a higher risk for carotid artery dissection and intracranial aneurysms, the overall natural history of carotid FMD is generally favorable and supports reassurance for patients in whom it is discovered incidentally. Management of migraine and pulsatile tinnitus can improve quality of life for symptomatic patients. Ongoing collaborative research and involvement in patient registries is needed to better understand the pathophysiology and optimal prevention and management of its cerebrovascular manifestations.