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IMAGE Image Thoracic Aortic Aneurysm in the Presence of Mediastinal and Retroperitoneal Fibrosis Pankaj Saxena, MCh, DNB a,∗ , Vanaja Sivapathasingam, MBBS b , Mauro Vicaretti, FRACS, PhD c and Robert J. Costa, FRACS b a University of Western Australia, Department of Cardiothoracic Surgery, Sir Charles Gairdner Hospital, Nedlands, WA 6009, Australia b Department of Cardiothoracic Surgery, Westmead Hospital, Wentworthville, NSW 2145, Australia c Department of Vascular Surgery, Westmead Hospital, Wentworthville, NSW 2145, Australia Inflammatory aneurysms of thoracic aorta are rare. Of all the aneurysms, only 5–10% are inflammatory in nature and are almost exclusively confined to the infrarenal segment of the aorta. We hereby present a patient with a large inflammatory aneurysm involving the thoracic aorta in association with mediastinal fibrosis and idiopathic retroperitoneal fibrosis. (Heart, Lung and Circulation 2009;18:294–295) © 2008 Published by Elsevier Inc on behalf of Australasian Society of Cardiac and Thoracic Surgeons and the Cardiac Society of Australia and New Zealand. Keywords. Aortic aneurysm; Mediastinal fibrosis; Retroperitoneal fibrosis A 61-year-old woman presented with acute renal fail- ure and sepsis. She had history of hypertension and hypercholesterolaemia. Chest X-ray revealed significant mediastinal widening (Fig. 1A). A computed tomographic (CT) scan of chest and abdomen revealed the presence of a large thoracoabdominal aneurysm (ascending aorta 7 cm in diameter, arch and descending thoracic aorta 5.5–6 cm in maximal diameter) with bilateral hydronephrosis (Fig. 2). The abdominal aorta in the infrarenal segment was normal in diameter. There was evidence of generalised fibro- sis involving the retroperitoneal area with distortion of the ureters. There was marked thickening of the thoracic aorta. There was no evidence of aortic dissection. Cys- tourethrogram and retrograde pyelography revealed the presence of multiple strictures along the length of ureters bilaterally (Fig. 1B). The ureteric obstruction was relieved by bilateral ureteric stenting. The patient was managed with antibiotics to treat urosepsis related to vancomycin- resistant enterococci and acute renal failure was managed with dialysis. Cystoscopy revealed urothelial carcinoma in situ, which was treated with immunotherapy. Cystoscopy revealed a clearance of tumor 4 months later. Echocardiography revealed normal left ventricular systolic function with moderate aortic regurgitation. Coro- nary angiogram did not demonstrate any obstructive disease in coronary arteries. Received 13 December 2007; received in revised form 25 February 2008; accepted 10 March 2008; available online 29 May 2008 ∗ Corresponding author at: Department of Cardiothoracic Surgery, Sir Charles Gairdner Hospital, University of Western Australia, Nedlands, WA 6009, Australia. Tel.: +61 8 9346 3333; fax: +61 8 9346 2344. E-mail address: [email protected] (P. Saxena). Operative repair was undertaken. Median sternotomy was performed. A large 10 cm × 10 cm aneurysm of ascending aorta and arch was found. The wall of aorta was 1 cm in thickness (Fig. 3A). There was evidence of dense thickening of periaortic tissues. The patient underwent aortic valve replacement with 21mm Perimount Magna valve (Edward Lifesciences, Irvine, CA), replacement of ascending aorta and aortic arch with a 24 mm dacron graft and elephant trunk procedure using deep hypothermic circulatory arrest. The initial post-operative course was uncomplicated and the patient was extubated on post- operative day 1. She was discharged home on day 7. The patient was doing well on follow up at 4 weeks. Semi- elective repair of her descending thoracic aortic aneurysm is planned. Histopathology was suggestive of mediastinal fibrosis with multiple areas of infiltration by lymphocytes and plasma cells with fragmentation and degeneration of elas- tic fibres in the wall of aorta (Fig. 3). Periaortic tissue was densely sclerosed with similar lymphoplasmacytic infiltrate. There was no evidence of presence of syphilis, granulomatous disorders, fungal infection or malignancy. Discussion The presence of thoracic aortic aneurysm in a patient with fibro-inflammatory disorders of mediastinum and retroperitoneal area is an extremely rare condition. 1 The presence of retroperitoneal and mediastinal fibro- sis along with inflammation of aorta is suggestive of a common aetiological factor. Secondary factors such as malignancy, autoimmune disorders, granulomatous dis- eases, exposure to radiotherapy and use of drugs such as methysergide have been implicated in previously pub- lished literature. 2–3 © 2008 Published by Elsevier Inc on behalf of Australasian Society of Cardiac and Thoracic Surgeons and the Cardiac Society of Australia and New Zealand. 1443-9506/04/$36.00 doi:10.1016/j.hlc.2008.03.081
