Abstract

Introduction - Behcets disease is a multisystem disorder which can present with various vascular manifestations in 12% of the patients(1). Being classified under variable vessel vasculitis, it may involve both the arteries and the veins of all sizes(2). The spectrum of presentation includes venous thrombosis, systemic or pulmonary arterial anuerysms, stenosis or occlusions. The most common vascular presentation is venous thrombosis(3). The incidence of arterial manifestations in Behcets disease is comparably less(4). The aorta is the mostly commonly involved artery reported in literature followed by the femoral artery and the pulmonary artery. Aneurysm is the most common presentation in the aorta and rupture of a large artery aneurysm is the most common cause of death(1). The pathogenesis involved is the inflammation of the vasa vasorum leading to medial destruction and fibrosis(5). Management options include open surgical repair or endovascular approach, both done under a cover of immunosuppressive regimen. In the present era, open vascular repair can be avoided as it involves a high risk of perioperative complications in view of the inflammatory pathology leading to fragile vessel walls and also due the high risk of post operative psuedoaneurysm formation(6). Endovascular treatment options for an aortic aneurysm include stent grafts or coil embolisation, with the former being the most commonly reported modality in literature(7) . Coil embolisation has been used for both saccular aneurysms of the aorta and intracranial vessels(8). Methods - We report a case of saccular aneurysm in the celiac region of the abdominal aorta of size 2.8cm anteroposterior X 2.2cm transverse X 2.1cm superioinferior, occluding the celiac trunk in an 18 year old young male with Behcets disease. On initial evaluation his systemic inflammatory markers (ESR and CRP) were raised and the SMA origin was partially occluded by acute thrombus. In view of the same he was started on steroids, immunosuppressive medications and anticoagulation. His symptoms improved well on conservative management. On one month follow up the saccular aneurysm showed significant increase in size of 3.7cm anteroposterior X 2.9cm transverse X 3.6cm superioinferior necessitating urgent intervention to prevent life threatening complications like rupture. He underwent supra-SMA aortic stenting with bare metal stent and coil embolisation of the aneurysm sac. Post operatively he was discharged on steroids and immunosupression. Results - Immediate post operative and 1st month follow up duplex showed no flow in the aneurysm sac. Conclusion - This case report exemplifies that an endovascular approach can be used to treat a saccular aneurysm of the aorta with bare metal stent and coils without the risk of distal embolisation of coils. References1)Park JH, Han MC, Bettmann MA. Arterial manifestations of Behcet’s disease. AJR Am J Roentgenol 1984; 143:821–825.2)Lie.JT, Vascular involvement in Bechets disease: arterial and venous and vessels of all sizes, J Rheumatol 1992; 19:402-10 .3)Koc Y, Gullu I, Akpek G, et al. Vascular involvement in Behcet’s disease. J Rheumatol 1992; 19:402–410.4)N. Y. Jung, S. K. Kim, E. C. Chung, H. Park, and Y. K. Cho, Endovascular treatment for rupture of intrahepatic artery aneurysm in a patient with Behcet’s syndrome,” American Journal of Roentgenology, vol. 188, no. 5, pp.W400–W402, 2007.5)A. Freyrie, O. Paragona, G. Cenacchi, G. Pasquinelli, G. Guiducci, and G. L. Faggioli, True and false aneurysms in Behcet’s disease: case report with ultrastructural observations, Journal of Vascular Surgery, vol. 17, no. 4, pp. 762–767, 1993.6)Sasaki S, Yasuda K, Takigami K, Shiiya N, Matsui Y, Sakuma M. Surgical experiences with peripheral arterial aneurysms due to vasculo-Behcet’s disease. J Cardiovasc Surg (Torino) 1998; 39:147–150.7)J. H. Park, J. W. Chung, J. H. Joh, et al., Aortic and arterial aneurysms in Behcet disease: management with stent-grafts- Initial experience, Radiology, vol. 220, no. 3, pp. 745–750, 2001.8)W. H. Kim, D. Choi, J.-S. Kim, Y.-G. Ko, Y. Jang, and W. H. Shim, “Effectiveness and safety of endovascular aneurysm treatment in patients with vasculo-Behc¸et disease,” Journal of EndovascularTherapy, vol. 16, no. 5, pp. 631–636, 2009.

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