Case Study
Emphysematous aortitis leading to esophagopleural fistula
Asian Cardiovascular & Thoracic Annals 0(0) 1–4 ß The Author(s) 2016 Reprints and permissions: sagepub.co.uk/journalsPermissions.nav DOI: 10.1177/0218492316653309 aan.sagepub.com
Furkan Ufuk1, Duygu Herek2 and Nevzat Karabulut2
Abstract Emphysematous aortitis is a form of septic aortitis. It is a rare disease with a high mortality rate, necessitating prompt diagnosis and aggressive treatment. We present the computed tomography features of emphysematous aortitis leading to esophageal rupture and esophageal-pleural fistula in a 76-year-old female with rheumatoid arthritis.
Keywords Aneurysm, false, Aneurysm, infected, Aortitis, Arthritis, rheumatoid, Esophageal perforation, Fistula
Introduction Aortitis is a rare clinical entity involving inflammation and thickening of the aortic wall due to infectious or noninfectious conditions. Noninfectious aortitis can be observed in Behc¸et disease, polyarteritis nodosa, sarcoidosis, Takayasu’s disease, and temporal arteritis. It can also be seen in connective tissue diseases such as systemic lupus erythematosus and rheumatoid arthritis. Infectious aortitis may be due to tuberculosis, syphilis, Streptococcus, Neisseria, or Salmonella infection, and rarely, secondary to viral or fungal pathogens such as varicella-zoster virus, herpes virus, or aspergillosis. Aortitis is often overlooked during the initial work-up in patients with nonspecific symptoms and systemic diseases. Magnetic resonance imaging (MRI) and computed tomography (CT)-angiography play critical roles in evaluation of aortitis.1 Air collection within the aortic wall (emphysematous aortitis) is evidence of infection.2 Radiologists should be familiar with the imaging findings in aortitis, and should bear it in mind in the differential diagnosis of aortic wall enhancement or thickening.
inflammatory polyarthralgia and polyarthritis, and had been taking oral prednisolone 16–32 mg day 1 for 6 years following a diagnosis of rheumatoid arthritis. Family history was not significant. She had poor oral hygiene. Physical examination revealed a soft abdomen and epigastric tenderness without guarding. Laboratory findings included raised white blood cell count (16.4 103/mL), low hemoglobin (8.9 g dL 1), elevated lactate dehydrogenase (428 U L 1), high erythrocyte sedimentation rate (49 mm h 1), and increased C-reactive protein (9.2 U mL 1). Rheumatoid factor and anticyclic citrullinated peptide antibody were positive. Abdominal ultrasonography was unremarkable. Oral and intravenous contrast-enhanced CT of the abdomen showed thickening and enhancement of the segmental thoracoabdominal aortic wall with periaortic fat stranding, suggestive of aortitis (Figure 1). The aortic diameter was 29 mm. Broad-spectrum antibiotic therapy (meropenem and teicoplanin) was initiated, however, the fever and elevated C-reactive protein did not resolve. A blood culture was negative. On day 3 of antibiotic treatment, the patient’s general condition deteriorated and she developed abdominal pain, nausea, and vomiting. Thoracoabdominal CT showed an aortic
Case report A 74-year-old woman presented with abdominal pain, weakness, nausea, and vomiting for 12 h. She had normal blood pressure (115/80 mm Hg), a pulse rate of 79 beats min 1, oxygen saturation 98% in room air, and a temperature of 38.3 C. She had a history of
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Department of Radiology, Sandikli State Hospital, Afyonkarahisar, Turkey Department of Radiology, University of Pamukkale, Turkey
Corresponding author: Furkan Ufuk, Sandikli State Hospital, 03500 Afyonkarahisar, Turkey. Email:
[email protected]
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Figure 1. Axial oral and intravenous contrast-enhanced computed tomography showing thickening and enhancement of the segmental thoracoabdominal aortic wall (white arrow), periaortic fat stranding, and the esophagus (white dashed arrow).
Figure 2. (a) Axial and (b) coronal reformatted oral and intravenous contrast-enhanced computed tomography showing aortic pseudoaneurysm and air bubbles (*) in the aortic wall and periaortic regions.
pseudoaneurysm at the level of the gastroesophageal junction, which had increased rapidly to 43 mm in diameter. Moreover, air bubbles and fat stranding were observed in paraesophageal and periaortic regions (Figure 2). These findings suggested infected mycotic aneurysm and emphysematous aortitis; blood culture was positive for Streptococcus oralis. The patient underwent thoracic endovascular aortic repair (TEVAR) with continuing antibiotic therapy. On day 2 post-TEVAR, she developed shortness of breath and acute thoracoabdominal pain. Chest radiography showed right pneumothorax, and CT revealed leakage of oral contrast
material from the esophageal lumen at the level of the aortic pseudoaneurysm and a right esophagopleural fistula (Figure 3). The patient continued to deteriorate and died 2 h after the last CT scan.
Discussion Aortitis is a life-threatening condition that must be diagnosed and treated early and aggressively. However, the clinical manifestations may be nonspecific, as in our case, and include fever, weakness, nausea, vomiting, and back pain. It should be suspected in
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Figure 3. Axial oral contrast-enhanced computed tomography showing a right esophageal-pleural fistula (white arrow) and right pleural contrast material (*).
elderly or immunocompromised patients who develop bacteremia with prolonged fever and pain.3 Blood cultures should be obtained from any patient with aortic wall enhancement associated with fever, leukocytosis, and increased C-reactive protein levels.1 All of these manifestations were present in our patient. Bacterial colonization of the aortic wall may occur by direct extension, traumatic inoculation, or hematogenous spread. Hematogenous seeding is the most widely accepted etiology. Abscesses, venous thrombosis, and aortoenteric fistula may occur due to direct bacterial extension into adjacent soft tissues.4 We speculate that esophageal rupture in our patient was due to direct bacterial extension from the aorta, leading to esophageal wall erosion. Risk factors for mycotic pseudoaneurysm development include atherosclerosis, infective endocarditis, septicemia, arterial injury, intravenous drug use, and immunosuppression.3 Our patient had 2 risk factors: septicemia due to poor oral hygiene, and immunosuppression due to steroid use. Imaging plays a pivotal role in the diagnosis of aortitis. Irregular rim enhancement in the aortic wall is seen on CT or MRI in the early stages. Over time, the aortic wall may become thicker and more irregular with mural thrombi and slight periaortic fat stranding. In later stages, mycotic aneurysm, aortic wall emphysema, and more apparent fat stranding may be seen. A mycotic aneurysm is characteristically seen as a noncalcified asymmetric saccular aneurysm, often in an uncommon location, as in our patient. Rapid aneurysmal expansion suggests mycotic aneurysm.3 Initially, aortic wall thickening and enhancement was the only
finding in our case. We observed a pseudoaneurysm and air bubbles on CT 3 days later, and esophageal rupture 6 days later. This case emphasizes the importance of the initial CT findings, specifically, thickening and enhancement of the aortic wall, which may be subtle and overlooked. It is important to record any abnormal enhancement and recommend close follow-up. Broad-spectrum antibiotics and surgical repair are the mainstays of treatment for mycotic aortitis. Incomplete resection of the infected vessel or inadequate antibiotic therapy is fatal. Following large initial doses of antibiotics and prolonged therapy, in-situ graft placement may be successful.5 However, in our case, TEVAR did not prevent complications. If open surgery had been performed, the esophageal situation would have become clearer and could have been treated. We think that oral nutrition should be stopped when infectious aortitis is diagnosed. Esophageal perforation with esophagopleural fistula is rare and life-threatening. It is usually iatrogenic or secondary to malignancy or surgery.6 Esophagopleural fistula caused by aortitis has not been reported previously but we should consider this unusual yet serious condition in the setting of aortitis. We speculate that periaortic inflammation extended to the esophagus and mediastinal pleura before TEVAR, and created the fistula. Declaration of conflicting interests The author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.
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Funding The author(s) received no financial support for the research, authorship, and/or publication of this article.
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3. Restrepo CS, Ocazionez D, Suri R and Vargas D. Aortitis: imaging spectrum of the infectious and inflammatory conditions of the aorta. Radiographics 2011; 31: 435–451. 4. Skourtis G, Papacharalambous G, Makris S, et al. Primary aortoenteric fistula due to septic aortitis. Ann Vasc Surg 2010; 24: 825.e7–11. 5. Katabathina VS and Restrepo CS. Infectious and noninfectious aortitis: cross-sectional imaging findings. Semin Ultrasound CT MR 2012; 33: 207–221. 6. Wei L, Wang F and Chen S. A late diagnosed case of spontaneous esophageal perforation in an elderly patient. Int J Clin Exp Med 2015; 8: 11594–11597.
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