Eur J Cardiothorac Surg 2002;21:564-565
© 2002 Elsevier Science NL
Extensive mediastinal lipomatosis in a patient with severe aortic valve stenosis
Dirk F. Peeka*,
Robin H. Heijmena,
Sjef M. Ernstb,
Marc A. Schepensa
a Department of Cardiothoracic Surgery, St. Antonius Hospital, P.O. Box 2500, 3430 EM Nieuwegein, the Netherlands
b Department of Cardiology, St. Antonius Hospital, Nieuwegein, The Netherlands
Received 6 June 2001;
received in revised form 3 December 2001;
accepted 18 December 2001.
* Corresponding author. Tel.: +31-30-609-2047; fax: +31-30-609-2120
e-mail: drpeek{at}hotmail.com
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Abstract
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Mediastinal lipomatosis is a rare benign condition characterized by a large amount of mature adipose tissue in the mediastinum. We present the case of an 86-year-old male who was admitted to the hospital for analysis of his progressive dyspnea. After careful examination, the patient was diagnosed with severe aortic valve stenosis and extensive mediastinal lipomatosis. This rare coincidence of aortic valve disease and mediastinal lipoma was treated by aortic valve replacement and an extensive debulking procedure.
Key Words: Mediastinum • Lipomatosis • Aortic valve stenosis • Cardiomegaly
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1. Introduction
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Mediastinal lipomatosis is a rare benign condition characterized by a large amount of mature adipose tissue within the anterior mediastinum due to hyperplasia of lipocytes [1–3]. In the majority of cases this massive fat accumulation is asymptomatic. However, its mass effect may cause dyspnea due to compression of the lung. In this report, we describe a patient presenting with progressive dyspnea and a systolic murmur because of aortic valve stenosis, in whom a plain chest X-ray showed bilateral widening of the mediastinal silhouette due to extensive mediastinal lipomatosis.
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2. Case report
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An 86-year-old man was admitted to the hospital for analysis of progressive dyspnea of several years’ duration and a chest X-ray showing an enlarged mediastinum. His medical history revealed hemoptosis, recurrent periods of pulmonary emboli and myocardial infarction. On physical examination we observed an aged man with gynecomasty on his left chest and a systolic murmur over the aortic valve. The chest X-ray showed bilateral mediastinal widening mimicking enormous cardiomegaly (Fig. 1)
. The mediastinal silhouette was not of an homogeneous density. Scrutinizing the chest X-ray revealed that the contour line of the cardiac silhouette was faintly visible, indicating that the global enlargement was due to an extracardiac cause. There was also an elevated right hemidiaphragm suggesting dysfunction of the right phrenic nerve. Computed tomographic (CT)-scanning of the chest (Fig. 2) revealed a large intrathoracic mass, extending to both pleural cavities compressing the lungs, which was located in the anterior mediastinum. The inhomogeneous aspect suggested the presence of a lipoma. Echocardiography showed severe aortic stenosis (mean pressure gradient 104 mmHg, estimated aortic valve area 0.6 cm2). Laboratory analysis revealed no abnormalities. The patient was scheduled for elective aortic valve replacement and debulking of the mediastinal lipomatosis.
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Fig. 1. Chest X-ray showing bilateral enlargement of the mediastinal silhouette mimicking enormous cardiomegaly. The cardiac silhouette, however, is still faintly visible, suggesting a mediastinal mass.
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Fig. 2. Computed tomographic scan demonstrating a symmetrical accumulation of lobulated adipose tissue within the mediastinum.
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Intraoperatively, we found a large lipomatous mass completely covering the pericardium and extending towards the left and right parasternal region. The tumor was adherent to the right jugular vein and anonymous vein. The dysfunctional right phrenic nerve could not be dissected free from the lipoma, and hence was transected en bloc. Following the debulking of the mediastinal mass of about 1.5 kg, the aortic valve replacement was performed uneventfully. Histologic analysis showed lobules of mature fat with some atypia confirming our preoperative diagnosis of mediastinal lipoma. There was no evidence of malignancy.
The postoperative course was complicated by transient atrial fibrillation and a right pleural effusion requiring drainage. The chest X-ray showed a reduced mediastinal mass on both sides. One week postoperatively the patient was discharged to his referring physician in good clinical condition for further rehabilitation.
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3. Discussion
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In this case report we present a rare case of extensive mediastinal lipomatosis combined with severe aortic valve stenosis. There was no history of thoracic trauma, obesitas [4], Cushing's syndrome [5], or chronic steroid intake [6], which are medical conditions known to be associated with mediastinal lipoma.
Because of progressive dyspnea due to this combined etiology, the patient was planned for elective aortic valve replacement and debulking of the mediastinal mass. As the lipoma, consisting of nonencapsulated mature adipose tissue, progressed throughout the entire mediastinum, its infiltrative behavior, although benign, prevented complete removal. The elevated right hemidiaphragm due to paralysis of the phrenic nerve may be explained by compression of the lipoma.
The initial plain chest X-ray may be misinterpreted as enormous cardiomegaly. Close evaluation, however, clearly illustrated the fainted contour of the heart's silhouette. For differential diagnosis between cardiac and extracardiac causes of mediastinal enlargement, CT-scanning is obligatory [7].
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Abstract
1. Introduction
