Ann Thorac Surg 2009;88:e11-e13. doi:10.1016/j.athoracsur.2009.05.024
© 2009 The Society of Thoracic Surgeons
Case Reports
A Large Pericardial Cyst Complicated by a Pericarditis in a Young Man With a Mediastinal Mass
Alexandra McMillan, MDa,
Carolina A. Souza, MD, PhDb,
John P. Veinot, MDc,
Michel Turek, MDa,
Paul Hendry, MDd,
Gonzalo G. Alvarez, MD, MPHa,*
a Department of Medicine, The Ottawa Health Research Institute, University of Ottawa at The Ottawa Hospital, Ottawa, Ontario, Canada
b Department of Radiology, The Ottawa Health Research Institute, University of Ottawa at The Ottawa Hospital, Ottawa, Ontario, Canada
c Department of Clinical Pathology, The Ottawa Health Research Institute, University of Ottawa at The Ottawa Hospital, Ottawa, Ontario, Canada
d Department of Cardiac Surgery, The Ottawa Health Research Institute, University of Ottawa at The Ottawa Hospital, Ottawa, Ontario, Canada
Accepted for publication May 4, 2009.
* Address correspondence to Dr Alvarez, 501 Smyth Rd, Ottawa, Ontario, K1H 8L6, Canada (Email: galvarez{at}ohri.ca).
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Abstract
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This is a case report of a 30-year-old man who presented with acute right-sided pleuritic chest pain. A chest roentgenogram performed in the emergency room shows a large, well-circumscribed transparent lesion obscuring the right heart border. Further imaging revealed a large pericardial cyst. The patient was taken to surgery as a result of clinical deterioration, and the knowledge that large pericardial cysts, although usually benign, can cause serious complications, including death. Pathology revealed an inflamed pericardial cyst with fibrinous pericarditis.
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Introduction
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Pericardial cysts occur in approximately 1 in 100, 000 persons and are the most common primary tumor of the pericardium [1]. Accounting for 7% of all mediastinal tumors, most pericardial cysts are found in the right costophrenic angle, approximately 25% are found in the left costophrenic angle, and the remaining 8% are found in the posterior and anterior superior mediastinum [2]. The differential of a cyst in the mediastinum includes pericardial, bronchogenic, enteric, and thymic cysts. After the initial chest roentgenogram of our patient, the diagnosis was believed to be most consistent with a pericardial cyst due to his young age and anterior mediastinum location; however, lymphoma, thymoma, mesenchymal, or extragonadal germ cell tumors were also considered.
A 30-year-old man presented to the emergency room with acute right-sided, pleuritic chest pain that awoke him from sleep. The pain was associated with significant orthopnea and symptoms of presyncope when standing. On examination, his vitals were stable with no pulsus paradox appreciated. He was noted to be in mild respiratory distress, unable to lie down at less than a 45° angle, limited by his increased work of breathing and worsening chest pain. A cardiovascular examination did not reveal a pericardial rub and was otherwise unremarkable. Pertinent investigations included a creatine kinase of 46 U/L and a troponin of 0.18 µg/L. His electrocardiogram was unremarkable. A posteroanterior chest roentgenogram at the time of presentation showed a well-circumscribed, transparent lesion in the right cardiophrenic angle, obscuring the right heart border.
During his admission, the patient had significant respiratory distress develop, and he could not lie supine. Repeat imaging revealed interval enlargement of the pericardial cyst and pericardial effusion, bilateral pleural effusions, and pulmonary edema. A repeat transthoracic echocardiogram showed no signs of tamponade. A contrast-enhanced computed tomographic scan of the chest performed the next day after admission confirms the presence of a well-circumscribed lesion in the right paracardiac area, adjacent to the right atrium. Measurement of the computed tomographic scan attenuation value demonstrated fluid attenuation (15 Hounsfield Unit), indicating a cystic lesion. Small bilateral pleural effusions were noted, and there was a trace of a pericardial effusion. Two days later, a contrast-enhanced computed tomographic scan (Fig 1) was performed that showed an interval increase in the bilateral pleural effusions and pericardial effusion (arrows). The right paracardiac lesion (asterisk) was slightly enlarged with no changes in the computed tomographic attenuation value. A transthoracic echocardiogram confirmed an inhomogeneous mass (posterolateral to the right atrium) and a small pericardial effusion with no obvious communication between the cyst and pericardium.
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Fig 1. Contrast-enhanced computed tomographic scan of the chest with mediastinal window settings demonstrates a slightly enlarged well circumscribed water attenuation lesion (asterisk) adjacent to the right atrium (ra). Small bilateral pleural effusions are noted (thin arrows) and there is a trace of pericardial effusion (thick arrow).
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At surgery, there was some tension of the pericardium, but this was not sufficient enough to cause noncompliance of the pericardial sac to result in the clinical manifestations of cardiac tamponade. A median sternotomy was performed, with the pericardium stripped to reveal diffuse fibrinous pericarditis, and a posterior loculated fluid collection was drained, which improved both hemodynamic and respiratory function. The large cystic mass was removed from the right pleural space, and the pathology report revealed an inflamed pericardial cyst with fibrinous pericarditis. No perioperative complications arose. The patient was discharged home to follow-up.
The excised oblong cyst was 11.0 cm x 9.0 cm x 7.0 cm. Externally, the cyst was severely congested with a small piece of attached parietal pericardium that was separate from the main cyst. Attached fat was noted, and the fat separated the cyst and the piece of pericardium. No attached thymic tissue was seen. The attached pericardium had a shaggy, ragged red appearance, grossly consistent with organizing fibrinous pericarditis.
A cut section of the cyst showed a unilocular cyst filled with clear fluid, with mural fibrin and yellowish strands of tissue within. No necrosis or calcification was noted (Fig 2). To the left of the photograph, a small attached piece of inflamed pericardium can be noted (arrow). The cyst wall was generally thin with no bone or cartilage. No communication between the cyst and the pericardium was noted. By microscopy, the cyst lining had foci of organizing fibrin similar to the attached parietal pericardium on the external surface (Fig 3). The wall was free of epithelium and no cartilage was seen. Gram stain and culture for bacteria, acid fast bacillus stain, and Grocott silver stain for fungi, bacteria, and cytology were all negative.
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Comment
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Our hypothesis is that the patient had pericarditis develop that subsequently caused inflammation of the pericardial cyst. Supporting this hypothesis is both the pathologic report, which revealed a fibrinous inflammatory process identical in appearance and age occurring both within the cyst and in the pericardium, and the fact that the cyst did not contain clear echocardiographic free fluid, but hemorrhagic fluid instead. The patient's right-sided chest pain was likely the result of pericarditis with associated pleuritis, as the inflamed cyst abutted the parietal surface of the pleura. As the inflammatory process ensued, the pericardial effusion increased, and the inflamed cyst began to enlarge within the right hemithorax. His dyspnea and associated orthopnea are symptoms characteristic of pericarditis and pleuritis, which may have been exacerbated by the significant compression of atelectasis and the enlarging pleural effusion noted on the imaging. The absence of a pericardial rub was attributed to the serous fluid that separated the two layers of the pericardium. The elevated troponin was attributed to his pericarditis. The cause of the fibrinous pericarditis remains idiopathic, as our extensive workup did not reveal any evidence of infection, malignancy, uremia, connective tissue disease, or trauma.
Most asymptomatic, pericardial cysts are often discovered incidentally on chest roentgenograms [3]. Rarely, life-threatening complications as a result of a pericardial cyst have been reported, which includes heart failure [2], tamponade, erosion of cardiac wall and adjacent structures [4], and spontaneous rupture [5]. Review of the literature revealed no similar cases to the one presented here. Although generally believed that asymptomatic pericardial cysts require only close follow-up [6], indications for resection include a large size, unclear diagnosis, or a symptomatic patient [2]. Percutaneous aspiration is an attractive alternative to surgical resection with one study showing no evidence of recurrence of the cyst at a 3-year follow-up [3]. In fact, recent literature would suggest that percutaneous aspiration be the first line of treatment [7]. Alternatively, videoscopic thoracoscopic resection provides another less invasive option to minimize perioperative morbidity [8].
In conclusion, our case highlights an atypical presentation of pericarditis, which was complicated by an inflamed pericardial cyst. The adjacent fibrinous pericarditis may have irritated the shared wall, resulting in inflammation of the cyst. Not all large pericardial cysts are clinically benign, and they may result in serious sequelae including death. Although no communication was noted between the cyst and the pericardium on imaging before the surgery, due to the clinical deterioration of the patient and the knowledge that communication was possible, surgery was undertaken. The patient had an uneventful postoperative stay, and an echocardiogram 3 months later was normal, with no evidence of reoccurrence.
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Acknowledgments
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The authors would like to thank Drs Sundaresan, Belanger, and Astrope for their contributions in managing the case.
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References
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- Feigin DS, Fenoglio JJ, McAllister HA, et al. Pericardial cysts: a radiologic-pathologic correlation and review Diagn Radiol 1977;125:15-20.
- Chopra PS, Duke DJ, Pellet JR, et al. Pericardial cyst with partial erosion of the right ventricular wall Ann Thorac Surg 1991;51:840-841.[Abstract/Free Full Text]
- Patel J, Park C, Michaels J, Rosen S, Kort S. Pericardial cyst: case reports and a literature review Echocardiography 2004;21:269-272.[Medline]
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- Szinicz GM, Taxer F, Riedlinger J, et al. Thoracoscopic resection of a pericardial cyts Thorac Cardiovasc Surg 1992;40:190-191.[Medline]
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Abstract
Introduction
