British Journal of Radiology (2004) 77, 615-619
© 2004 British Institute of Radiology
doi: 10.1259/bjr/45993201
Endovascular management of multiple arterial aneurysms in Behcet's disease
Y Hama, MD
T Kaji, MD
Y Iwasaki, MD
T Kawauchi, MD
M Yamamoto, MD
and
S Kusano, MD
Department of Radiology, National Defense Medical College, 3-2 Namiki, Tokorozawa, Saitama, 359-0042, Japan
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Abstract
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We report a case of Behcet's disease complicated by four arterial aneurysms successfully treated by coil embolisation and stent placement. Percutaneous endovascular repair offers a safe alternative to surgical management of this serious condition.
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Introduction
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Behcet's disease is an inflammatory disorder of unknown cause, characterized by recurrent oral aphthous ulcers, genital ulcers, and uveitis [1, 2]. However, Behcet's disease is now recognized as a systemic disorder with mucocutaneous, ophthalmical, neurological, cardiovascular, pulmonary, gastrointestinal, urogenital and musculoskeletal involvement [1–4]. Cardiovascular involvement appears in only 2–28% of patients, however, it is the most common cause of mortality in patients with Behcet's disease [3–6]. Therapy of arterial lesions remains controversial. Surgical repair of arterial lesions including stenosis and/or occlusions and aneurysms is often unsuccessful because of aneurysm recurrence or arterial thrombosis at the site of surgical repair or elsewhere [7–10]. In some cases, aneurysms are multiple and surgical repair is of limited value [5, 11, 12]. Recently, endovascular management has been attempted for treatment of vascular involvement in Behcet's disease [4, 13–15]. However, when aneurysms are multiple, endovascular treatment is still difficult and challenging. To our knowledge, there is no report on a patient with four or more arterial aneurysms treated solely by endovascular management. We describe a case of four arterial aneurysms in Behcet's disease successfully treated by coil embolisation and stent placement.
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Case report
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A 42-year-old woman with a history of Behcet's disease was admitted because of low back pain. Behcet's disease was identified following a history of polyarthralgias, recurrent oral aphthous ulcers, and genital ulceration. No history of recent or remote trauma, drug abuse, or infective endocarditis was elicited. For the last year, the patient had been successfully treated with methylprednisolone (8 mg day–1) and colchicine (1 mg day–1). At the time of admission, there was no clinical or biological sign of evolution of the Behcet's disease.
Contrast-enhanced CT demonstrated a right internal iliac artery aneurysm (Figure 1a
). Angiography revealed a large saccular aneurysm originating from the right internal iliac artery (Figure 1b). However, multiple aneurysms of both subclavian arteries and of the infrarenal abdominal aorta and occlusion of the right renal artery were also present. After discussion with our vascular surgery team, surgical intervention was rejected due to the multifocality of aneurysms and the potential risk of recurrence. After informed consent was obtained, endovascular placement of coils and metallic stents was attempted. Each lesion was treated on a separate day.
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Figure 1. (a) Contrast enhanced CT image demonstrated an aneurysm of the right internal iliac artery. Uterine leiomyomas were also seen. (b) An angiogram revealed a large saccular aneurysm arising from the proximal right internal iliac artery. (c) Post-embolisation angiography confirmed occlusion of the aneurysm.
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With a 4 Fr shepherd's hook catheter in the right internal iliac artery, a microcatheter (Nadeshiko, JMS, Tokyo, Japan) was advanced coaxially into the neck of the aneurysm over a 0.016-inch hydrophilic guidewire (GT wire; Terumo, Tokyo, Japan). Five fibred platinum coils (Vortx; Boston Scientific, Cedex, France) and 3 interlocking detachable coils (IDCs) (Boston Scientific; Natick, MA, USA) were deposited in the aneurysm and its neck. Post-embolisation angiography confirmed occlusion of the aneurysm (Figure 1c
).
6 weeks later, the aneurysm of the abdominal aorta was treated by transcatheter embolisation using metallic coils. Abdominal aortography showed a large saccular aneurysm arising from the abdominal aorta and occlusion of the right renal artery (Figure 2a). With a 4 Fr shepherd's hook catheter in the neck of the aneurysm, a microcatheter (Nadeshiko; JMS, Tokyo, Japan) was advanced coaxially into the aneurysm over a 0.016-inch hydrophilic guidewire (GT wire; Terumo, Tokyo, Japan). Six fibred platinum coils (Vortx; Boston Scientific, Cedex, France) and 2 IDCs were deposited in the aneurysm. Post-embolisation angiography confirmed occlusion of the aneurysm (Figure 2b).
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Figure 2. (a) Frontal abdominal aortogram revealed a large saccular aneurysm at the origin of the right renal artery. Fusiform dilatation of the left renal artery and occlusion of the right renal artery were also noted. (b) Post-embolisation aortogram confirmed occlusion of the aneurysm.
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4 months after the procedure, the aneurysm of the right subclavian artery (Figure 3a) was treated by endovascular placement of a metallic stent. After intravenous injection of isosorbide dinitrate (2.5 mg/5 ml) and heparin (3000 U) to avoid vascular spasm and thromboembolism, a 9 Fr vascular sheath (Super Sheath; Medikit, Tokyo, Japan) was advanced into the right brachial artery. A 0.035-inch hydrophilic guidewire (Radifocus; Terumo, Tokyo, Japan) was inserted retrograde into the lumen of the descending aorta. A 7 mm x 40 mm covered stent (Passager; Boston Scientific, Natick, MA, USA) was deployed to completely exclude the aneurysm. Immediately after the procedure, angiography demonstrated complete occlusion of the aneurysm and good patency of the stent-graft (Figure 3b). Endoleakage was not identified. Oral anticoagulation therapy (warfarin 2 mg day–1) was started on the day of stent-graft placement.
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Figure 3. (a) An angiogram revealed a saccular aneurysm of the right subclavian artery. (b) After stent-graft placement, an angiogram showed complete occlusion of the aneurysm.
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2 weeks after the procedure, the aneurysm of the left subclavian artery (Figure 4a) was treated in the same way. However, due to difficulty in evaluating the size and shape of the aneurysm, three-dimensional CT imaging of the left subclavian artery was obtained (Figure 4b). After intravenous injection of isosorbide dinitrate (2 mg/4 ml) and heparin (1000 U), an 8 Fr vascular sheath (Super Sheath; Medikit, Tokyo, Japan) was advanced into the left subclavian artery. A 0.035-inch hydrophilic guidewire (Radifocus; Terumo, Japan) was inserted retrogradly into the lumen of the descending aorta. A 6 mm x 40 mm covered stent (Passager; Boston Scientific, Natick, MA, USA) was deployed to completely exclude the aneurysm. Further balloon expansion was needed due to the endoleakage after stent-graft treatment. Immediately after the procedure, angiography demonstrated complete occlusion of the aneurysm with no endoleakage (Figure 4c).
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Figure 4. (a) An angiogram revealed a saccular aneurysm of the left subclavian artery. The configuration of the aneurysm was not clearly demonstrated. (b) CT angiography with three-dimensional volume rendering reconstructions clearly demonstrated the configuration of the aneurysm. The aneurysm was arising from the posterior aspect of the left subclavian artery. (c) After stent-graft placement and remodelling with a 10 mm angiography balloon, an angiogram showed complete occlusion of the aneurysm with no endoleakage.
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The patient had an uneventful post-treatment course and follow-up CT scans 22 months after the initial treatment confirmed persistent occlusion of the aneurysms and that stent-graft patency was maintained.
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Discussion
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Arterial involvement in Behcet's disease can be either occlusive or aneurysmal degeneration. However, the leading cause of death in patients with Behcet's disease is a rupture of a large arterial or aortic aneurysm [4, 5]. The pathogenesis of the aneurysmal degeneration is thought to be vasculitis resulting in obliterative endarteritis of the vasa vasorum supplying the medium-sized and large-sized vessels [16]. Thus, arterial aneurysms can occur throughout the body either synchronously or metachronously [1, 5, 10–12].
Surgery is available for resecting arterial aneurysms and replacing grafts. Many attempts have been made to manage these serious lesions by surgical treatment. However, there are several reports concerning recurrence after surgical management in about half of cases [5, 11, 12, 17]. To prevent complications or recurrences after surgical repair, endovascular insertion of a stent-graft and/or transcatheter arterial embolisation is a reasonable alternative. Recently, these endovascular treatments have been reported and considered to be safe and effective for aortic and arterial aneurysms in Behcet's disease [4, 13, 18]. The technical success rate of stent-graft insertion is higher than 90%; successful insertion results in the exclusion of the aneurysm and complete thrombosis [4]. Due to the high success rates of endovascular treatments, minimal invasiveness, and the possibility of systemic recurrences of aneurysms, endovascular treatment seems to be reasonable. However, as far as we know, there is no report on a patient with multiple arterial (more than two lesions) and aortic aneurysms treated solely by endovascular managements in Behcet's disease. We treated the four aneurysms on 4 different days. Since in Behcet's disease the formation of an aneurysm occasionally occurs at the puncture site, it is suggested that angiography be postponed until the inflammatory reaction subsides [19]. To avoid the treatment-related complications and reduce the patient's physical and mental burden, we performed endovascular treatment one-by-one on 4 different days after evaluating the inflammatory reactions.
The potential limitation of this treatment is the short duration of follow-up: each arterial lesion is stable 22 months after the initial treatment in this case. However, on a literature review of the arterial aneurysms treated with bypass grafts in patients with Behcet's disease, the mean time to aneurysm formation at the anastomotic sites was 3.3 months, with the longest primary patency noted at 6 months [20]. The time to graft occlusion was an average of 8.3 months with the longest reported patency being 16 months [20]. In our case, an elective endovascular treatment may be responsible for the favourable outcome due to the reasons as mentioned above.
In conclusion, a single case report cannot prompt a treatment recommendation even in a rare disease. However, endovascular treatments are an attractive alternative for the management of multiple arterial and aortic aneurysms in patients with Behcet's disease.
Received for publication June 5, 2003.
Revision received August 11, 2003.
Accepted for publication November 11, 2003.
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Abstract
Introduction
