Ruptured Suprarenal Abdominal Aortic Pseudoaneurysm with Superior Mesenteric and Celiac Arteries Occlusion, Revealing Behçet's Disease: A Case Report

Abstract

Behçet’s disease (BD) is a multisystemic, chronic autoimmune inflammatory vasculitic disease with an unknown etiology. Although the literature reports that vascular involvement occurs in 7% to 38% of all BD cases, the arteries are rarely involved; however, arterial involvement is usually associated with significant mortality and morbidity. We report the case of a young female patient who presented to the emergency department with severe abdominal pain and a history of weight loss. The patient was evaluated using computed tomography angiography, which revealed a ruptured suprarenal aortic pseudoaneurysm with occlusion of both the superior mesenteric and celiac arteries. Urgent surgery was performed with aortic repair with an interposition graft and superior mesenteric artery embolectomy. The patient’s clinical history and radiological imaging findings were strongly suggestive of the diagnosis of BD with vascular involvement.

An Abdominal Aortic Pseudoaneurysm Revealing Behçet’s Disease

“…Venous involvement is predominant and often happens during the first five years after the onset of BD. Venous, arterial, and cardiac impairments of BD often coexist [ 8 , 9 ].…”

“…The inflammation process is responsible for abnormal localized dilatation of the vessel wall. The rupture of both intima and media layers causes leaking of the blood which becomes bounded by the outer vessel layer (adventitia) or perivascular tissue, resulting in the formation of a pseudoaneurysm, whereas in the true aneurysm, the media layer is weakened and the vessel is dilated within a soft spot in the vessel wall resulting in the formation of a true aneurysm, frequently saccular, where the blood is contained within all three layers of the vessel wall [ 9 , 13 ].…”

“…Endoscopy exposes the patient to the risk of aneurysm at the puncture site [ 15 , 18 ] whereas open surgical repair is complex due to the extended healing duration and the difficulty to suture the friable vessel wall. Thus, surgeons prefer interventional endoscopy, although there is not enough evidence to state that it provides better prognosis for BD patients with pseudoaneurysm [ 9 ].…”

Case report and analysis: Behçet’s disease with lower extremity vein thrombosis and pseudoaneurysm

“…Diagnosis can be based on the International Study Group (ISG) for BD ( 41 ). Non-invasive examinations such as color Doppler ultrasonography, computed tomography angiography, and magnetic resonance angiography are usually preferred for diagnosis of BD vascular lesions, because vascular invasive operations in BD patients may induce thrombi or pseudoaneurysms ( 42 ). This raises the following question, regarding our case: is the occurrence of a pseudoaneurysm of the right CIA directly related to thrombolysis surgery of the right lower limb?…”