Shaggy aorta syndrome: recurrent mesenteric embolus.

Clinical Description

An 85-year-old man, with left internal carotid artery occlusion and history of right occipital lobe infarct, on peritoneal dialysis for 6 years underwent emergent laparotomy for peritonitis after his dialysis catheter had started draining brown-colored, murky fluid suggestive of enteric contents. He was found to have multiple ischemic small bowel segments. The superior mesenteric artery, however, had a strong pulse. Necrotic bowel segments were resected, left in discontinuity, and a second look was planned. Work-up did not reveal a cardiac source for the emboli. Computed tomography revealed diffusely calcified aorta and the appearance of an ulcerated ‘shaggy aorta’ (Fig. 1). Systemic anticoagulation was initiated. The patient had a pronged hospitalization requiring multiple reoperations, developed multisystem organ failure and ventilator dependent respiratory failure. The last operation was aborted when ischemia of both small and large bowel was found. A family discussion was held, and the patient was provided palliative care and he soon expired (post-operative day 50 from his index operation).

Figure 1

Panels a and b depict sagittal views of the thoracic and abdominal aorta respectively with corresponding axial views on CT demonstrating the ulcerated atheromatous plaques (white arrows). Panel c demonstrates three-dimensional reconstruction of the thoracic aorta.

Discussion

Many patients with diffusely atherosclerotic aorta may not experience any distal embolic episodes. ‘Shaggy Aorta syndrome’ is a condition where the aorta is diffusely atherosclerotic with ulcerated plaques and presents with a clinical syndrome of multiple visceral arterial embolic episodes [1]. Imaging studies of both thoracic and abdominal aorta should be obtained not only to make the diagnosis but also to define the extent of the disease, the segments spared and hence plan management. Treatment options range from anticoagulation/antiplatelet therapy with or without prosthetic replacement of the entire diseased segment to extra-anatomic bypasses. Recently, endovascular stent graft exclusion of the disease segments has also been reported [2].