Recurrent Aortic Aneurysms In Behçet Disease

Case Report

A 22-year-old white male with a 10-year history of gastrointestinal complaints, primarily of diarrhea and lactose and gluten intolerance, presented with a new onset of severe lower back pain. He had undergone previous endoscopic and radiologic investigations with no evidence of inflammatory bowel disease, celiac disease, or other gastrointestinal and infectious pathology. His family history was negative for any form of connective tissue disease, autoimmune disorders, or vasculitis.

Abdominal computed tomography (CT) revealed a 1.5 cm aneurysm at the celiac artery origin. Initially viewed as an incidental finding, a repeat CT scan 2 weeks later demonstrated significant expansion of this celiac trunk aneurysm to a maximal diameter of 3.5 cm (Figure 1). The patient was subsequently referred to our vascular surgery service.

OPEN IN VIEWER

Figure 1.

Celiac artery trunk aneurysm with a maximal anteroposterior diameter of 3.5 cm. The aneurysm is abutting the proximal portion of the superior mesenteric artery. The aorta proximal and distal to this area appears to be normal.

The etiology of this aneurysm was initially unknown. However, its rapid growth and the patient's recent symptoms of upper respiratory tract infection raised the clinical suspicion of a mycotic etiology. Laboratory investigations revealed a mild leukocytosis (11.9 × 10⁹ units/L). Multiple blood, urine, sputum, stool, and intestinal biopsy cultures showed no evidence of bacterial growth. The patient denied any history of intravenous drug use.

Given the risk for rupture and presumed infectious etiology, it was recommended that the patient undergo open thoracoabdominal repair of the aneurysm. Intraoperatively, the area of the aneurysm was identified and there were numerous inflammatory lymph nodes intimately adherent to the aneurysm, which were left in place. As described previously, ¹² a temporary shunt was constructed with a 10 mm Dacron graft sutured proximally end to side from the thoracic aorta proximal to the aneurysm and distally to the left common iliac artery to provide renal and distal extremity perfusion during cross-clamping of the supraceliac and suprarenal aorta. On opening of the aneurysm, a necrotic-appearing pseudoaneurysm with a blowout of the anterior wall of the aorta with a 3 cm defect involving the origin of the celiac artery was seen. The distal celiac artery itself was no longer identifiable. The aorta was resected distally to the level of the origin of the superior mesenteric artery. A 14 mm interposition Dacron graft was used to reconstruct the aorta. The 10 mm Dacron shunt was removed, and a cuff of graft in the chest was oversewn and left in place. The remainder of the operation was uncomplicated.

Postoperative convalescence was uncomplicated, and the patient was discharged home on postoperative day 7. Pathologic analysis of the aortic tissue from the operating room showed no gross and/or microscopic evidence of infection, vasculitis, or lymphoma. Gram stains and cultures were all negative. Nevertheless, the patient continued on intravenous antibiotics for 6 weeks.

Four-week follow-up with a CT scan revealed a new aortic pseudoaneurysm in the lower descending thoracic aorta. The pseudoaneurysm measured 2.8 cm in diameter and was associated with a hematoma. The inferior margin of the aortic blowout was approximately 3 cm above the previous proximal aortic graft anastomosis near the site of the proximal shunt graft cuff (Figure 2). This new pseudoaneurysm was at significant risk of rupture. Given the recent open procedure, an endovascular repair was planned.

OPEN IN VIEWER

Figure 2.

A new aortic pseudoaneurysm in the lower descending thoracic aorta. The pseudoaneurysm measured 2.8 cm in diameter and was associated with a hematoma that measured 4.3 cm. The inferior margin of the aortic blowout was approximately 3 cm above the previous proximal aortic graft anastomosis.

Prior to the planned endovascular procedure, the patient revealed clinical criteria suggestive of BD, according to the International Study Group of Behcet's Disease. ⁷ He had a long history of recurrent aphthous mouth ulcers, sporadic undiagnosed genital ulcers, and lower limb bilateral erythema nodusa. A skin pathergen test was negative. Ophthalmologic evaluation did not reveal obvious eye lesions. Laboratory investigations demonstrated a significantly elevated erythrocyte sedimentation rate, 78 mm/h (normal 0–10 mm/h), and C-reactive protein level, 56.6 mg/L (normal < 5 mg/L). Immunologic studies also revealed mildly elevated IgG, IgA, C3, and C4 levels. The initial celiac trunk aneurysm and new thoracic pseudoaneurysm showed radiologic characteristics that were similar to aneurysms reported in patients with known BD. ⁷

The patient then underwent urgent endovascular repair of the new-onset thoracic blowout. The procedure was carried out under general anesthesia via a femoral approach. A single 28 mm Valiant thoracic endograft (Medtronic, Santa Rosa, CA) successfully sealed the pseudoaneurysm. On a completion angiogram, there was adequate filling of the superior mesenteric and renal arteries. The aneurysm was completely excluded, with no obvious endoleak.

Rheumatology suggested starting immediate prednisone (60 mg twice a day) and cyclophosphamide treatment (1g IV q 4 weeks). Hematology recommended full-dose dalteparin sodium (Fragmin) anticoagulation because of the patient's previous central line venous thrombosis, and the patient was on colchicine (2.4 mg/d) for back pain, which was believed to occur from sacroileitis. Ophthamologic examination detected no obvious eye pathology. A follow-up CT scan 4 weeks following endovascular repair showed no recurrent evidence of aneurysmal disease. Colonoscopy, ultrasound endoscopy of the pancreas, and upper endoscopy showed no signs of pathology or features of BD.

Discussion

This is a case of a celiac artery trunk aneurysm presenting as the initial radiologic sign of BD. Although celiac artery aneurysms in BD have been reported previously, ^7,8,13 they are a very rare pathologic presentation. BD is a chronic autoimmune disorder characterized by recurrent oral ulcers and any of several systemic manifestations, including genital ulcers, ocular disease, skin lesions, neurologic disease, and vascular disease involving both venous and arterial blood vessels of all sizes. It is most common along the ancient Silk Road, with prevalence as high as 80 to 370 cases per 100,000 in Turkey. ¹⁴ It tends to affect young adults and is often misdiagnosed because of the lack of a specific diagnostic marker. BD in the North American population is exceedingly rare, 0.2 to 6 per 100,000, and is often not initially considered in the differential diagnosis of younger patients presenting with new arterial lesions.

However, only a few diseases involve both the arterial and venous systems, such as systemic lupus erythematous, Buerger's disease, antiphospholipid syndrome, and cocaine use. Previous longitudinal cohort studies have suggested that the 20-year mortality for the disease is around 9.8%, with the mortality and morbidity of the disease highest among young males and usually confined to the early years of its course, with evidence of “burning out” as the disease progresses. The exception seems to be vascular involvement, which can appear 5 to 10 years after the original diagnosis. ¹⁵ With the loss of arterial wall integrity, BD predisposes to false aneurysms and ruptures, making early diagnosis and treatment paramount. ¹⁶ However, some authors have recommended, whenever possible, against operating during the active phase of the disease, when inflammatory markers are elevated. Instead, they recommend delaying surgery until remission through immunosuppressive treatments is achieved, defined as the normalization of inflammatory markers. ¹⁶ In addition to other connective tissue disorders, such as Marfans and Ehlers-Danlos syndrome, that are associated with aneurysmal disease in younger patients, we suggest that BD should be considered in such situations.

Recurrent anastomotic pseudoaneurysms have been reported to occur in 30 to 50% of open repairs of aneurysms in patients with BD. ⁷ Some reports suggest that recurrence may be precipitated by cessation of immunotherapy drugs. ¹⁷ In our case, the site for the recurrent aortic blowout following the initial open repair did not occur at the proximal anastomosis of the interposition graft but rather at the site of the thoracic aorta to left iliac artery shunt. This shunt was used to aid in perfusion of the kidneys during the cross-clamping; however, we would caution against such techniques given the added risk of pseudoaneurysm formation in the BD population.

Placement of endovascular stent grafts for repair of thoracic pseudoaneurysms in BD has been previously reported and represents a potentially beneficial surgical approach for the treatment of arterial lesions of BD. ^11,18 The benefits of an endovascular approach are not only the minimally invasive benefits but also the lack of a need to cross-clamp the aorta, the minimal amount of aortic manipulation, and the avoidance of creating suture lines. These factors may lower the risk of recurrent anastomotic aneurysms and result in a significant improvement in patient mortality. Although short-term success has been described, the longer-term durability of this type of repair with BD remains unclear.

In this case, the delay in the diagnosis of BD was complicated by the patient's chronic gastrointestinal history and possible symptoms of constitutional infection, which suggested a mycotic disease process. Early diagnosis may have important implications in the surgical management for aneurysmal disease in BD as advancements in endovascular techniques represent a promising approach.