Behcet's Disease Presenting as Bilateral Common Femoral Artery Pseudo-aneurysms
Article Outline
- Abstract
- Case report
- Discussion
- Conclusion
- Funding
- Conflict of Interest
- Ethical Approval
- References
- Copyright
Abstract
We present a 25-year-old Chinese male patient who was diagnosed with Behcet's disease after suffering bilateral femoral artery pseudo-aneurysms. This was treated surgically by excising the pseudo-aneurysm and achieving vascular continuity by harvesting the great saphenous vein as a conduit. Arterial pseudo-aneurysms are rare vascular manifestations in Behcet's disease and management with vein is advisable as patients are frequently on long-term immunosuppression which puts them at risk of infections with prosthetic grafts.
Keywords: Pseudo-aneurysm, Behcet's disease, Vasculitis
Behcet's disease is a rare chronic, relapsing, inflammatory disease, with arterial pseudo-aneurysms being one of its many clinical manifestations.
Case report
A 25-year-old Chinese male patient presented with a 2-week-old spontaneous right groin pain and swelling. He denied any trauma or intravenous drug use. He had no claudication, lower limb swelling, fever or groin discharge. However, he had the alpha-thalaessemia trait.
Examination revealed a tender, 3-cm, expansile, pulsating mass in the right groin, with audible bruit on auscultation. The rest of the vascular system was normal.
A computed tomography (CT) aortogram showed two saccular non-leaking pseudo-aneurysms of the right common femoral artery (CFA) (Fig. 1).
Figure 1
Two lobulated, saccular pseudo-aneurysms of the right common femoral artery, medially and laterally, measuring 3.1
×2.5cm and 1.0×0.6cm, respectively.
Rutherford Morrison incision was used to achieve extra-peritoneal proximal control of the right external iliac artery (EIA). The CFA was exposed with a vertical groin incision and distal control secured. The pseudo-aneurysm was excised and the great saphenous vein was harvested as a conduit for end-to-end anastomosis between the EIA and bifurcation of the CFA.
Histology showed an aneurysm wall with fibrosis and inflammation. Culture grew no bacteria or fungi. He developed right ileo-femoral deep vein thrombosis after surgery. Protein C, protein S, anti-thrombin III, anti-cardiolipin antibody and antinuclear antibody tests were negative. Lupus anticoagulant was weakly present. He had warfarin anticoagulation for 1 year.
Two years later, he developed a similar episode of spontaneous left groin swelling. A CT aortogram confirmed a non-leaking, fusiform pseudo-aneurysm of the left CFA (Fig. 2). Repair of the left CFA pseudo-aneurysm was performed in a similar fashion. Histology of the vessel wall showed chronic arteritis and arteriosclerosis.
Figure 2
Pseudo-aneurysm of left common femoral artery arising anteriorly, measuring 3.2
×2.6cm. No recurrence of pseudo-aneurysm was noted in the right groin.
Further examination revealed recurrent aphthous mouth ulcers, penile ulcers, pathergy over intravenous cannulation sites and lower limb erythematous nodules, with biopsy consistent for erythema nodosum. A diagnosis of Behcet's disease was made. Azathioprine and prednisolone were commenced to control disease progression, and warfarin to prevent further thromboses. Further imaging (CT brain and echocardiogram) and ophthalmology assessment were normal. All immunological markers were repeated and found to be similar to the earlier results.
He has been on a follow-up regimen since 23 months and has suffered no further arterial pseudo-aneurysms or flare up of Behcet's disease or recurrent pseudo-aneurysms from the surgery site. He has not developed any post-thrombotic complications.
Discussion
Behcet's disease is a chronic, relapsing, inflammatory disease characterised by recurrent oral aphthous ulcers and numerous potential systemic manifestations, including genital ulcers, ocular disease, skin lesions, neurological disease, vasculitis and arthritis.1
It affects mainly young adults aged between 20 and 40 years of Middle Eastern and Far Eastern ancestry.1 The aetiology and pathogenesis are unknown and likely represent aberrant immune activity triggered by exposure to an agent in patients with a genetic predisposition.
There are no specific pathognomonic laboratory tests or histological findings. The diagnosis is based on clinical criteria (Table 1).2
Table 1. Criteria for diagnosis of Behcet's disease (International Study Group for Behcet's Disease).2
| Criterion | Required features |
|---|---|
| Recurrent oral ulceration | Aphthous ulceration with at least three episodes in any 12-month period |
| Plus any two of the following: | |
| Recurrent genital ulceration | Aphthous ulceration or scarring |
| Eye lesions | Anterior or posterior uveitis, cells in vitreous on slit-lamp examination or retinal vasculitis |
| Skin lesions | Erythema nodosum-like lesions; papulopustular skin lesions or pseudofolliculitis with characteristic acneiform nodules |
| Pathergy test | Papule 2mm or more in size, developing 24–48h after pricking the skin with a 20–25-gauge needle |
Vascular involvement occurs between 7.7% and 60%, develops within 10 years of initial diagnosis and affects males more frequently. Vascular histological features include perivascular lymphocytic and plasma cell infiltration, endothelial cell proliferation and swelling, disruption of elastic lamina, degeneration of the tunica media and vasculitis of the small vessels in vasa vasorum. These lead to stenoses, aneurysm formation and thromboses in arteries and veins. Behcet's vasculopathy is divided into venous occlusion, arterial occlusion and aneurysm formation.1, 3, 4
Venous involvement is most common, with 50% of the patients with a history of venous thrombosis. Thrombosis can affect not only the superficial and deep veins of lower and upper extremity but may also involve the central veins. The increased thrombogenicity has been attributed to pro-thrombotic states (Factor V Leiden mutation and anticardiolipin antibodies), endothelial injury and defective fibrinolysis.1, 3
Occlusive arterial disease can involve any site in the upper and lower extremities, but are more common in the distal limb run-off. Bypass surgery can be therapeutic, but with increased risk of graft failure due to pro-thrombotic tendency. Patency can be enhanced by anticoagulation.3
Arterial pseudo-aneurysms occur more frequently than do occlusive arteriopathy. The aorta is most often affected, followed by pulmonary, femoral, subclavian, popliteal and carotid arteries. Open surgery is the mainstay of treatment; however, it is complicated by recurrent pseudo-aneurysms in the operated artery or in arteries adjacent to arterial bypasses. Endovascular stenting is promising but has potential complications of pseudo-aneurysm formation at the puncture site and thrombosis induced by the vascular catheter.4
Conclusion
This report highlights a rare presentation of Behcet's disease in a Chinese male with recurrent pseudo-aneurysms of the femoral arteries. Surgical repair of the pseudo-aneurysm with native vein conduits is recommended as these patients have pro-thrombotic tendency and require long-term immunosuppression and may fare poorly with prosthetic grafts. Immunosuppressants help prevent further vasculopathy. Early diagnosis and treatment is the key to the management and prevention of future complications. Autoimmune conditions should be considered in patients presenting with spontaneous non-traumatic recurrent pseudo-aneurysms.
Funding
None declared.
Conflict of Interest
None declared.
Ethical Approval
None declared.
References
- . Behcet syndrome. Curr Opin Rheumatol. 2004;16:38
- . Criteria for diagnosis of Behcet's disease. Lancet. 1990;335:1078–1080
- . Vascular involvement in Behcet's disease. J Rheumatol. 1992;19:402–410
- . The Vascular manifestations of Behcet's disease: angiographic and CT findings. Br J Radiol. 2000;73:1270–1274
PII: S1533-3167(09)00005-3
doi:10.1016/j.ejvsextra.2009.02.001
© 2009 European Society for Vascular Surgery. Published by Elsevier Inc. All rights reserved.
Refers to article:
- Behcet's Disease presenting as Bilateral Common Femoral Artery Pseudo-aneurysms , 01 April 2009
