Radiological Category Thoracic Cardiovascular Thoracic Chest Principal Modality

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Radiological Category: Thoracic Cardiovascular; Thoracic Chest Principal Modality (1): CT Principal Modality (2): Fluoroscopy/

Radiological Category: Thoracic Cardiovascular; Thoracic Chest Principal Modality (1): CT Principal Modality (2): Fluoroscopy/ General Radiology Case Report # 777 Submitted by: Bilal Anwer, M. D. Faculty reviewer: Emma Ferguson, M. D. , The University of Texas Medical School at Houston Date accepted: 15 March, 2011

Case History 52 year old male present to the emergency room with chest pain

Case History 52 year old male present to the emergency room with chest pain and dysphagia. PMHx: Hypertension, Coronary Artery Disease, Umbilical Hernia PSHx: Two Vessel CABG in 2000 Soc. Hx: 60 pack years smoking, daily alcohol use. FHx: Noncontributory Physical Exam: Tachycardia, normal S 1/S 2, normotensive; clear lungs; afebrile; abdomen soft and non-tender

Radiological Presentation: CXR

Radiological Presentation: CXR

Radiological Presentation: CT CHEST

Radiological Presentation: CT CHEST

Radiological Presentation: CT CHEST

Radiological Presentation: CT CHEST

Radiological Presentation: CT CHEST

Radiological Presentation: CT CHEST

Radiological Presentation: CT CHEST

Radiological Presentation: CT CHEST

Radiological Presentation: CT CHEST

Radiological Presentation: CT CHEST

Case History The patient was admitted for further work up. The following day, he

Case History The patient was admitted for further work up. The following day, he had a sudden episode of hematemesis, hypoxia, and hypotension. Do the prior images provide a clue as to the etiology of the current presentation? The patient was taken to Interventional Radiology for emergent angiography.

Radiological Presentation: Aortogram

Radiological Presentation: Aortogram

Radiological Presentation: Aortogram

Radiological Presentation: Aortogram

Radiological Presentation: Aortogram

Radiological Presentation: Aortogram

Radiological Presentation: Aortogram

Radiological Presentation: Aortogram

Test Your Diagnosis Which one of the following is your choice for the appropriate

Test Your Diagnosis Which one of the following is your choice for the appropriate diagnosis? After your selection, go to next page. • Esophageal abscess with prominent ductus diverticulum • Traumatic Esophageal Injury with Aortic Pseudoaneurysm • Esophageal Carcinoma with Penetrating Aortic Ulcer

Radiological Presentation: Aortogram

Radiological Presentation: Aortogram

Radiological Presentation: Aortogram

Radiological Presentation: Aortogram

Radiological Presentation: Aortogram

Radiological Presentation: Aortogram

Radiological Presentation: Aortogram

Radiological Presentation: Aortogram

Radiological Presentation: Aortogram

Radiological Presentation: Aortogram

Radiological Presentation: Aortogram

Radiological Presentation: Aortogram

Radiological Presentation: Aortogram

Radiological Presentation: Aortogram

Radiological Presentation: Aortogram

Radiological Presentation: Aortogram

Radiological Presentation: Aortogram

Radiological Presentation: Aortogram

Radiological Presentation: Aortogram

Radiological Presentation: Aortogram

Radiological Presentation: Aortogram

Radiological Presentation: Aortogram

Test Your Diagnosis Which one of the following is your choice for the appropriate

Test Your Diagnosis Which one of the following is your choice for the appropriate diagnosis? After your selection, go to next page. Based on the Aortogram, what else would you add to the list of differentials below? • Esophageal abscess with prominent ductus diverticulum • Traumatic esophageal Injury with aortic pseudoaneurysm • Esophageal carcinoma with penetrating aortic ulcer

Radiological Presentation There is a lytic scapula lesion (left). Dual Energy subtraction image (right)

Radiological Presentation There is a lytic scapula lesion (left). Dual Energy subtraction image (right) better demonstrates numerous bilateral pulmonary nodules. The left paratracheal stripe is abnormally thickened with a small left pleural cap vs. thickening Aortic wall outpouching with circumferential esophageal wall thickening, and severe luminal stenosis. Note the loss of fat plane between the esophagus and aorta.

Radiological Presentation A repeat DSA run at an oblique angle and using the power

Radiological Presentation A repeat DSA run at an oblique angle and using the power injector uncovered active extravasation from the descending aorta into an anteromedial tubular structure. Initial AP DSA Aortogram images with hand injection provide a more accurate depiction of the descending aortic wall outpouching size and significance. Note the length of aortic wall compromise on the DSA image sequence, which points to aortic wall compromise beyond a focal defect.

Findings and Differentials Findings: Careful inspection of the initial CXR reveals multiple pulmonary nodules

Findings and Differentials Findings: Careful inspection of the initial CXR reveals multiple pulmonary nodules and a destructive scapula lesion with suggestion of left paratracheal stripe widening and left apical pleural abnormality. The lateral view did not demonstrate obvious tracheoesophageal stripe thickening or a posterior mediastinal mass. The CT shows circumferential esophageal wall thickening with severe luminal stenosis and a prominent right paratracheal lymph node. However, it is the subtle small focal aortic wall outpouching with loss of fat plane between the esophagus and aorta that proved to be a more significant finding with catastrophic consequences. Active extravasation from the aorta into an adjacent tubular structure is demonstrated on the aortogram. Differentials: • Disseminated infection with esophageal abscess and mycotic aortic aneurysm rupture with subsequent aortoesophageal fistula. • Post-traumatic esophageal Injury with aortic pseudoaneurysm rupture and subsequent aortoesophageal fistula. Incidental pulmonary nodules and scapula destructive lesion. • Metastatic esophageal carcinoma with aortic wall invasion and adjacent penetrating aortic ulcer resulting in aortoesophageal fistula

Discussion Infectious Etiology: Disseminated atypical infections in immunocompromised patients can present in advanced stages.

Discussion Infectious Etiology: Disseminated atypical infections in immunocompromised patients can present in advanced stages. Infective aortitis can present as a mycotic pseudoaneurysms that are typically saccular and contain eccentric thrombus. They have a propensity for the ascending aorta due to its close proximity with endocarditis. Any cause of intimal injury can result in seeding of pathogens. It is not uncommon to see pulmonary nodules from opportunistic infections and osteomyelitis in immunocompromised patients. Candida and CMV are the most common culprits behind esophagitis in AIDS patient. Figure on the left shows Candida esophagitis with giant plaques. Esophageal abscess and potential perforation are typically caused by chronic foreign body impaction. Complications can lead to an aortoesophageal fistula, which is a rare but catastrophic event. Levine M et al. Agarwal P P et al. Retroesophageal mediastinal abscess and a mycotic pseudoaneurysm of the descending thoracic aorta (arrow).

Discussion Traumatic Etiology: Extraluminal esophageal injury due to trauma is rare and typically involves

Discussion Traumatic Etiology: Extraluminal esophageal injury due to trauma is rare and typically involves the cervical or thoracic esophagus. It has a high association with vascular, tracheal, and spinal cord injury. Intraluminal injury may be from instrumentation, foreign body impaction, or barotrauma. Signs include leakage of oral contrast material, pneumomediastinum, pleural effusion, irregularity of the esophageal mucosa. Traumatic pseudoaneurysm formation in blunt trauma patients that reach the hospital alive involves the aortic isthmus in 90% of the cases. These can calcify, enlarge and rupture years after the initial trauma. Figure on the left shows posttraumatic saccular pseudoaneurysm at the aortic isthmus. Agarwal P P et al.

Discussion There have been a few cases of esophageal carcinoma invading the aorta resulting

Discussion There have been a few cases of esophageal carcinoma invading the aorta resulting in aortoesophageal fistula. Hollander et al. reported a review based on 500 reported cases of aortoesophageal fistulas in 1991. Of those, 17% were secondary to advanced esophageal carcinoma. Yoshifumi et al. describe a case of advanced esophageal carcinoma successfully treated with endoluminal stent graft similar to this case. This case shows a loss of fat plane between the esophagus and aorta suggesting aortic wall invasion. Infact, the tumor progressed in a similar fashion along approximately 10 cm of the esophagus. Additionally, the patient had significant atherosclerotic calcific plaques involving the descending aorta with a few penetrating ulcers. Aortic wall tumor invasion in combination with a penetrating aortic ulcer proved to be a near fatal combination, as demonstrated by CT and aortogram images.

Discussion Penetrating aortic ulcer is an ulcerating atherosclerotic lesion that penetrates the elastic lamina

Discussion Penetrating aortic ulcer is an ulcerating atherosclerotic lesion that penetrates the elastic lamina and results in hematoma formation within the media of the aortic wall. Figure on the left demonstrates the progression of a penetrating atherosclerotic ulcer. (a) demonstrates an ulcer penetrating through the intima into the media (b) This can lead to aortic dissection (c, d), aortic aneurysm (e), or rupture (f). Arrows indicate possible routes of progression. Hayashi H et al.

Discussion Spontaneous rupture of penetrating ulcers is rare without aneurysmal dilatation. Most cases involve

Discussion Spontaneous rupture of penetrating ulcers is rare without aneurysmal dilatation. Most cases involve predisposing factors such as hypertension and atherosclerosis. a b c Hayashi H et al. Figures above shows a penetrating ulcer in the descending thoracic aorta with surrounding intramural hematoma (a). Autopsy photo depicts ulceration (arrow) and intramural hematoma extending to the esophageal wall (b). Low-power photomicrograph shows atheromatous ulcer penetrating the media and intramural hematoma extending under the thickened intima (c).

Discussion Plain films are a main stay for initial workup and can provide important

Discussion Plain films are a main stay for initial workup and can provide important clues. The left paratracheal stripe (left image) is formed by contact between the left upper lobe and the mediastinal fat adjacent to the left tracheal wall. Abnormal widening may be caused by a large left-sided pleural effusion, Gibbs J M et al. lymphadenopathy, neoplasm , Gibbs J M et al. Tracheoesophageal (TE) stripe is or mediastinal hematoma. formed by air within the trachea and right lung outlining the posterior tracheal wall and A potential mimic of an intervening soft tissues. Vascular, aortic ulcer includes a esophageal lesions, lymphatic prominent ductus malformations, mediastinitis, and diverticulum. hematomas may cause abnormal It is a convex focal bulge its thickening. along the anterior undersurface of the isthmic region of the aortic arch as shown by the images to the right. Agarwal P P et al.

Discussion Aortoenteric fistula is a life threatening condition and may be primary or secondary.

Discussion Aortoenteric fistula is a life threatening condition and may be primary or secondary. Primary aortoenteric fitulas are rare (0. 007 per million) and almost always associated with pre-existing aortic aneurysms. Secondary aortoenteric fistulas are far more common and may be a consequence of aortic reconstructive surgery or stent placement with subsequent infection. Vu Q D M et al. Agarwal P P et al. CT images by Vu show a penetrating aortic ulcer adjacent to mediastinal soft tissue mass, which proved to be an aortoesophageal fistula upon surgery. Agarwal CT image shows an aortoesophageal fistula with mediastinal hematoma.

Discussion The patient in this case had a primary aortoesophageal fistula due to esophageal

Discussion The patient in this case had a primary aortoesophageal fistula due to esophageal pathology causing aortic wall compromise in combination with a penetrating ulcer. The subtle findings on the initial CXR in combination with CT images and history point to an esophageal malignancy with metastatic disease. Though on initial workup, the primary clinical concern and course of treatment focused on metastatic esophageal carcinoma, the tumor invasion of the aortic wall in combination with penetrating aortic ulcer proved to be potentially life threatening. Most authors suggest surgical treatment with grafting as the treatment of choice for penetrating aortic ulcers particularly in cases with persistent or recurrent pain, hemodynamic instability, and a rapidly expanding aortic diameter. But in patients who are high risk surgical intervention, endovascular stent repair is the treatment of choice. Given the current patient’s advanced metastatic disease and history of coronary artery bypass, a decision was made to employ stent graft repair with a TAG Gore endograft as shown on the left.

Diagnosis Metastatic esophageal carcinoma with localized invasion and adjacent penetrating aortic ulcer resulting in

Diagnosis Metastatic esophageal carcinoma with localized invasion and adjacent penetrating aortic ulcer resulting in aortoesophageal fistula

References Marc S. Levine MD, Rona Woldenberg MD, Hans Herlinge MD, Igor Laufer MD.

References Marc S. Levine MD, Rona Woldenberg MD, Hans Herlinge MD, Igor Laufer MD. Opportunistic Esophagitis In AIDS: Radiographic Diagnosis. December 1987 Radiology, 165, 815 -820. Catherine A. Young, MD, JD, Christine O. Menias, MD, Sanjeev Bhalla, MD and Srinivasa R. Prasad, MD. CT Features of Esophageal Emergencies. October 2008 Radio. Graphics, 28, 1541 -1553. Prachi P. Agarwal, MD, Aamer Chughtai, MD, Frederick R. K. Matzinger, MD and Ella A. Kazerooni, MD, MS. Multidetector CT of Thoracic Aortic Aneurysms. March 2009 Radio. Graphics, 29, 537 -552 Hideyuki Hayashi, MD, Yohjiro Matsuoka, MD, Ichiro Sakamoto, MD, Eijun Sueyoshi, MD, Tomoaki Okimoto, MD, Kuniaki Hayashi, MD and Naofumi Matsunaga, MD. Penetrating Atherosclerotic Ulcer of the Aorta: Imaging Features and Disease Concept. July 2000 Radio. Graphics, 20, 995 -1005. Quan D. M. Vu, MD, Christine O. Menias, MD, Sanjeev Bhalla, MD, Christine Peterson, MD, Lisa Lihaun Wang, MD and Dennis M. Balfe, MD. Aortoenteric Fistulas: CT Features and Potential Mimics. January 2009 Radio. Graphics, 29, 197 -209.

References Jerry M. Gibbs, MD, Chitra A. Chandrasekhar, MBBS, Emma C. Ferguson, MD and

References Jerry M. Gibbs, MD, Chitra A. Chandrasekhar, MBBS, Emma C. Ferguson, MD and Sandra A. A. Oldham, MD. Lines and Stripes: Where Did They Go? —From Conventional Radiography to CT. January 2007 Radio. Graphics, 27, 33 -48. Yoshifumi Ikeda, MD * , Naomi Morita, MD, Hideko Kurihara, MD, Masanori Niimi, MD, Kota Okinaga, MD. A primary aortoesophageal fistula due to esophageal carcinoma successfully treated with endoluminal aortic stent grafting. J Thorac Cardiovasc Surg 2006; 131: 486 -487 Hollander JE, Quick G. Aortoesophageal fistula. a comprehensive review of the literature. Am J Med 1991; 91: 279 -287 Written permission to reprint all referenced images was obtained from the Radiological Society of North America and respective authors.