Endovascular repair of a ruptured thoracic saccular aneurysm in a patient with MAGIC Syndrome: Case report and review of the literature

Purpose: To present the succesfull endovascular repair of impending rupture of thoracic saccular aneurysm in patient with MAGIC syndrome. Case: A 51-year-old man with a history of MAGIC syndrome was transferred to our vascular unit with a 3 days history of thoracic pain and hemoptysis. Contrast enhanced computed tomography depicted a contained rupture of a saccular aneurysm in the distal part of descending thoracic aorta. The patient was successfully managed with thoracic endovascular aortic repair (TEVAR) and Anti-TNFa factor. His immediate postoperative course was uneventful. Follow up at 2 months depicted thrombosed saccular aneurysm with excellent patency and absence of migration and endoleak. Conclusion: Mid-term outcome of endovascular repair in patients with MAGIC syndrome seems to be safe. Due to the nature of the disease and its rarity, more studies are necessary to establish its efficacy. *Correspondence to: Tigkiropoulos K, Vascular Unit,1st Department of Surgery, Aristotle University Thessaloniki, Papageorgiou General Hospital, Thessaloniki, Greece, Tel: 00306944173723; E-mail: kostastig@yahoo.com Received: July 23, 2019; Accepted: July 30, 2019; Published: August 02, 2019 Introduction MAGIC (Mouth And Genital ulcers with Inflamed Cartilage) syndrome is a very rare entity, which includes clinical manifestations of Behçet’s disease (BD) and Relapsing Polychondritis (RP) [1]. Aneurysms are considered a common complication in patients with MAGIC syndrome (21.1%), even under immunosuppression therapy, requiring emergency surgery due to its clinical presentation. Vascular involvement occurs in 14.7%-27.7% of Behcet’s Disease patients [2-4]. Aortic aneurysm is well described pathology even in this subpopulation, and its optimal treatment with immunosuppressant therapy and surgical treatment, remains to be defined. In Relapsing Polychondritis patients, aneurysms or aortitis develop in 5-10% of the patients and are multiple in 50% of them, usually involving the ascending aorta [2]. They are characterized by their presence even during remission of the disease and under lifelong immunosuppression therapy [3]. Aortic involvement is generally asymptomatic, most patients present with sudden rupture of the arterial wall with devastating and poor outcome. These aneurysms typically have a faster growing rate and rupture more easily, due to the inflammatory nature of the underlying disease. The recommended treatment for aneurysmatic disease in patients with Behçet’s Disease and Relapsing Polychondritis is based on series of isolated cases reports with small number of patients, including lifelong immunosuppressant therapy and surgical intervention, either open or endovascular. We report the successful endovascular repair of a ruptured saccular aneurysm in descending thoracic aorta followed by Anti-TNFa therapy in a patient with MAGIC syndrome.