Late-onset anastomotic pseudoaneurysm development 21-years after surgical treatment of arterial lesions in a patient with Behçet’s disease

Vascular manifestations may dominate the clinical presentation in some Behçet’s disease (BD) patients. Pseudoaneurysms are a major contributory factor to the overall mortality in BD patients; however, data on the long-term outcomes after vascular reconstructive surgery in BD are relatively scarce. We herein report the case of a patient with BD who developed an anastomotic pseudoaneurysm 21 years after surgical treatment of multiple arterial lesions, including an abdominal aortic aneurysm (AAA). A 32-year-old man initially presented with a left inguinal lump. He was found to have a saccular AAA, a symptomatic left common femoral artery aneurysm, and left superficial femoral artery occlusion, and underwent surgery for these arterial lesions. A bifurcated graft replacement of the infrarenal aorta and a left leg graft of the bifurcated graft to the distal superficial femoral artery bypass with a polytetrafluoroethylene graft were performed. The diagnosis of BD was also made at the time of the operation based on genital ulcers, erythema nodosum, and a history of recurrent oral ulcers. Immunosuppressive therapy was initiated after the operation, and his signs and symptoms of BD disappeared within 1 month after the operation. Immunosuppressive therapy was tapered and adjusted to the minimum dose needed to maintain remission. He had been treated with colchicine 1.5 mg/day for 15 years before the present admission. Postoperatively, he underwent regular imaging follow-up by duplex ultrasound or computed tomography (CT) every 6 months. Four years earlier (17 years after the operation), he had been diagnosed with diffuse dilatation of the left popliteal artery with a diameter of 20 mm; the dilatation did not show any remarkable change in size thereafter. After 21 years of regular follow-up, duplex ultrasound demonstrated saccular dilatation of the right common iliac artery that had not been present on the previous study performed 6 months earlier. Enhanced CT confirmed an anastomotic pseudoaneurysm of the right common iliac artery (Figure 1A, B). On admission, he had no symptoms or signs of BD. His erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) level were normal. Endovascular repair of the anastomotic pseudoaneurysm was planned. Under general anaesthesia, right femoral access was obtained by surgical cut-down. After embolization of the right internal iliac artery, two Viabahn stent grafts (11 mm diameter × 5 cm length and 13 mm diameter × 5 cm length; WL Gore & Associates, Flagstaff, AZ, USA) were positioned and deployed. A completion angiogram demonstrated successful exclusion of the aneurysm (Figure 2A, B). The postoperative course was uneventful, and the patient was discharged on postoperative day 5. At 15 months’ follow-up, he was in good condition without any symptoms or signs of BD or recurrence of the aneurysm. Vascular involvement is reported to occur in 7–38% of BD patients (1). While venous involvement is more common, aneurysm formation and its fatal rupture is one of the main causes of excess mortality associated with BD. The results of surgical treatment of arterial lesions in BD patients have not been satisfactory owing to frequent postoperative complications, such as graft occlusion and anastomotic pseudoaneurysms. Previous studies have indicated that a lack of postoperative immunosuppressive therapy (2) and elevated inflammatorymarkers (3) are associated with an increased risk of recurrent pseudoaneurysms. In a study by Hosaka et al, anastomotic pseudoaneurysm was reported as a relatively early complication, occurring within the first 18 months postoperatively (4). However, some authors have reported the unpredictable recurrence of pseudoaneurysms in patients with normal ESR and CRP levels under immunosuppressive therapy (5). Patients who developed pseudoaneurysm 5 years or more after their operation have also been reported (6). In the present case, dilatation of the left popliteal artery developed 17 years postoperatively, and an anastomotic pseudoaneurysm that required additional intervention developed 21 years postoperatively during clinical remission of BD. Although the exact cause of the arterial changes in the present case, which occurred with a longer delay than previously reported cases, is unclear, weakness of Scand J Rheumatol 2019;48:415–417 415