Management of vascular complications of Behcet's Disease in the endovascular era

Background: Behcet's Disease is an autoimmune disease whose vascular manifestations include both deep venous thrombosis and arterial aneurysm formation. Issues regarding diagnosis, timing of intervention, and technical aspects of surgery will be discussed.
Methods: This is a case report and review of the literature.
Results: A 58-year-old woman presented to an outside hospital with the acute onset of left leg edema. Duplex ultrasound confirmed the diagnosis of deep vein thrombosis of the left femoral vein and an IVC filter was placed. She was discharged on warfarin and experienced gradual resolution of the left leg swelling. Two months later, she developed obstruction of the inferior vena cava and right ilio-femoral venous thrombosis. Computerized tomography demonstrated compression of the inferior vena cava by a 5cm saccular aneurysm involving the infrarenal aorta (Figure 1). In addition, a 4 cm. aneurysm was identified in the left internal iliac artery.
Based on her history of typical cutaneous lesions, the diagnosis of Behcet's Disease was made. Due to the known risk of pseudoaneurysm formation following conventional surgical repair, endovascular treatment was elected (1). The right common femoral artery was exposed through a conventional incision. Other arterial punctures including an arterial line were avoided. The left hypogastric artery aneurysm was embolized using a combination of 6 though 10 mm hemostatic coils.Using intravascular ultrasound and fluoroscopic guidance, an 18 mm x 85 mm AneuRx limb extension (Medtronic Santa Rosa, CA) was used to exclude the infrarenal aortic pseudoaneurysm.
She was discharged the following day without complication. At 12 weeks follow-up, she has no buttock claudication and resolving right lower extremity edema. A follow-up CTA demonstrated complete exclusion of the aortic aneurysm and thrombosis of the left internal iliac aneurysm (Figure 2).
Conclusion: Behcet's Disease is an autoimmune vasculitis characterized by the triad of recurrent oral aphthous ulcers, genital lesions, and uveitis. Vascular manifestations of the disease are rare; however they are the major determinants of morbidity and mortality. Vascular surgeons should be aware of the concomitant occurrence of venous thromboembolic events with arterial aneurysm formation in this patient population. If open surgical repair is planned, it is best to avoid surgical intervention during the active stage of the disease. Endovascular treatment for peripheral aneurysms has been described in the past with encouraging results (2). Clearly, close follow-up and regular surveillance will be required to detect any new or recurrent arterial problems in this patient population.
References
1.
Hosaka A. Miyata T. Shigematsu H. Shigematsu K. Okamoto H. Ishii S. Miyahara T. Yamamoto K. Akagi D. Nagayoshi M. Nagawa H. Long-term outcome after surgical treatment of arterial lesions in Behcet disease. Journal of Vascular Surgery. 42(1):116-21, 2005 Jul.2.
Nitecki SS, Ofer A, Karram T, Schwartz H, Engel A, Hoffman A. Abdominal aortic aneurysm in Behcet's disease: new treatment options for an old and challenging problem. IMAJ 2004;6:152-5.