Fibromuscular dysplasia

Case contributed by Charles Nhan

Presentation

Presented to the emergency department with left shoulder pain and a one-month history of crescendo angina. Troponin negative and stress test unremarkable.

Patient Data

Age: 70 years
Gender: Male

Oblique reconstruction of a coronary CT, demonstrating narrowing of the proximal right coronary artery. Axial and additional reconstructions demonstrating the loop-like appearance of the right coronary artery proximally.

CT angiogram of the neck, chest, abdomen, and pelvis

Reconstructed oblique/coronal CT angiogram centered at the level of the neck, demonstrating beaded appearance of both internal carotid arteries.

CT angiogram of the upper abdomen and oblique sagittal reconstruction demonstrating multifocal narrowing and dilatation of the celiac trunk.

Oblique reconstructions of the CT angiogram centered in the upper abdomen demonstrates subtle beaded appearance of both renal arteries.

Case Discussion

Fibromuscular dysplasia is an idiopathic vascular disease with fibromuscular thickening of the arterial wall. The intima, media, or adventitia can be involved, with medial involvement by far the most common. By location, the most common areas involved are the renal and cervical (carotid or vertebral) arteries.

The classic appearance is the "string of beads" where there are alternating concentric stenosis and dilatations as seen in involvement of the media.

Other, rarer manifestations are also possible including vascular loops, aneurysms, or fusiform ectasia. 

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