Ann Thorac Surg 2006;81:1877-1879
© 2006 The Society of Thoracic Surgeons
Case report
Cartilaginous and Osseous Metaplasia With Bone Marrow Formation in the Aortic Valve of a Patient With Giant Cell Aortitis
Nicholas A. Charokopos, MD, PhD
a
,
*
,
Polichronis Antonitsis, MD
a
,
Efthymia Rouska, MD
a
,
Ioannis Kostopoulos, MD, PhD
b
,
Adnan Charaf, MD
a
,
Panagiotis K. Spanos, MD, PhD
a
a Department of Thoracic and Cardiovascular Surgery, AHEPA University Hospital, Aristotelian University of Thessaloniki, Thessaloniki, Greece
b Department of Pathology Medical School, Aristotelian University of Thessaloniki, Thessaloniki, Greece
Accepted for publication July 13, 2005.
* Address correspondence to Dr Charokopos, 22 Grigoriou E' St, Panorama, Thessaloniki, 55 236 Greece (Email: charokoposnick{at}hotmail.com).
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Abstract
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We describe the case of a 40-year-old man who presented with an ascending aortic aneurysm and aortic valve regurgitation. The patient underwent a Bentall surgical procedure. Pathologic findings were consistent with giant cell aortitis with synchronous cartilaginous and osseous metaplasia with bone marrow formation in the degenerated aortic valve. The coexistence of these findings has not been previously reported in the English language literature.
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Introduction
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Giant cell arteritis is a granulomatous inflammation of large-sized and medium-sized arteries of unknown cause. It most commonly affects temporal arteries. Extracranial manifestations of the disease involve the ascending aorta and aortic arch in about 39% of cases [1]. Cartilaginous and osseous metaplasia with synchronous presence of functioning marrow is a rare pathologic variant of calcific aortic disease.
This is a case report of an ascending aortic aneurysm formation due to giant cell aortitis with concurrent cartilaginous and bone metaplasia in a calcific aortic valve.
A 40-year-old man was referred to our department for evaluation of a widened mediastinum found on a routine chest roentgenogram. The patient underwent a series of diagnostic investigations, including a transthoracic echocardiogram that showed marked dilatation of the ascending aorta with grade III aortic regurgitation. A computerized tomographic scan revealed the presence of an ascending aortic aneurysm with a diameter of 7.1 cm.
The patient had a clear past medical history. A modified Bentall procedure was performed with a composite valve conduit. The gross external appearance of the aorta was normal. The patient had an uneventful recovery and was discharged on postoperative day 9.
Pathologic examination (hematoxylin-eosin sections) revealed extensive degenerative lesions of the aortic valve with cartilaginous and osseous metaplasia together with bone marrow formation (Fig 1).
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Fig 1. Intense degenerative lesions of the aortic valve with osseous metaplasia (arrow) and bone marrow formation (arrowhead). (Hematoxylin and eosin; x20.)
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Multiple cross-sections of the aortic wall showed nodular formation with central eosinophilic necrosis surrounded by histiocytic elements, chronic inflammatory infiltrates, and multinucleated giant cells consistent with giant cell aortitis (Fig 2).
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Fig 2. Nodular lesion of the aortic wall with central eosinophilic necrotic material and peripheral inflammatory infiltrate, histiocytes, and multinuclear giant cells. (Hematoxylin and eosin; x100.)
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Four weeks later the patient was referred to the rheumatology unit for steroid or immunosuppressive treatment.
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Comment
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Giant cell arteritis is a systemic vasculitis that most commonly affects temporal arteries of the head and neck in elderly individuals. It presents with signs and symptoms of an arterial occlusive disease due to the inflammatory process. Headache, jaw claudication, vision loss, fever, and polymyalgia rheumatica are typical clinical features. Involvement of the aorta and its major branches is termed giant cell aortitis and comprises 15% of cases of giant cell arteritis [2]. In this setting, ascending aortic aneurysm may occur with the risk of serious complications such as aneurysm rupture, aortic regurgitation, dissection, stroke, and myocardial infarction [3–5]. In most series giant cell aortitis is usually asymptomatic in presentation. Evidence of a past history of temporal arteritis or polymyalgia rheumatica is found in the minority of this group of patients [6].
Cartilaginous metaplasia in a calcific aortic valve is an uncommon pathologic finding. A few cases have been reported in the English literature. Seemayer and colleagues [7] described hyaline cartilage in the aortic valve of a 22-year-old man with endocarditis. Groom and Starke [8] reported severe calcific aortic sclerosis with foci of hyaline cartilage in the aortic valve of a 49-year-old man.
Calcific aortic stenosis leads to massive calcium deposition in the valve leaflets that morphologically resemble bone formation. However, osseous metaplasia in an aortic valve with synchronous presence of functioning marrow is rarely encountered. Arumugam and colleagues [9] and Fernandez Gonzalez and colleagues [10] have described osseous metaplasia with bone marrow formation in the aortic valve of a 43-year-old woman and a 69-year-old man, respectively. Both patients presented with aortic stenosis. Recent studies have demonstrated the expression of various bone-associated proteins in stenotic valves, such as bone sialoprotein and bone morphogenetic protein-2, which indicate that valvular calcification may be an actively regulated process [11].
This is the first reported case with concurrent existence of giant cell aortitis with ascending aortic aneurysm formation and subsequent aortic regurgitation with cartilaginous and osseous metaplasia with bone marrow formation in a degenerated aortic valve. This unique combination of pathologic findings can present with minimal or no symptoms. Surgical correction as indicated is mandatory to avoid potential lethal complications.
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References
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- Lie JT. Aortic and extracranial large vessel giant cell arteritisa review of 72 cases with histopathologic documentation. Semin Arthritis Rheum 1995;24:422-431.[Medline]
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- Soorae AS, McKeown F, Cleland J. Aortic valve replacement for severe aortic regurgitation caused by idiopathic giant cell aortitis Thorax 1980;35:60-63.[Abstract/Free Full Text]
- Kerr LD, Chang Y, Spiera H, Fallon JT. Occult active giant cell aortitis necessitating surgical repair J Thorac Cardiovasc Surg 2000;120:813-815.[Free Full Text]
- Vered Z, Pras M, Horowitz A, Rath S, Neufeld HN. Severe aortic regurgitationa rare presentation of giant cell arteritis. Clin Cardiol 1986;9:509-511.[Medline]
- Zehr KJ, Mathur A, Orzulak TA, Mullany CJ, Schaff HV. Surgical treatment of ascending aortic aneurysms in patients with giant cell aortitis Ann Thor Surg 2005;79:1512-1517.[Abstract/Free Full Text]
- Seemayer TA, Thelmo WL, Morin J. Cartilaginous transformation of the aortic valve Am J Clin Pathol 1973;60:616-620.[Medline]
- Groom DA, Starke WR. Cartilaginous metaplasia in calcific aortic valve disease Am J Clin Pathol 1990;93:809-812.[Medline]
- Arumugam SB, Sankar NM, Cherian KM. Osseus metaplasia with functioning marrow in a calcified aortic valve J Card Surg 1995;10:610-611.[Medline]
- Fernandez Gonzalez AL, Montero JA, Martinez Monzonis A, Gil O, Alemany P. Osseous metaplasia and hematopoietic bone marrow in a calcified aortic valve Tex Heart Inst J 1997;24:232.[Medline]
- Kaden JJ, Bickelhaupt S, Grobholz R, et al. Expression of bone sialoprotein and bone morphogenetic protein-2 in calcific aortic stenosis J Heart Valve Dis 2004;13:560-566.[Medline]
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Abstract
Introduction
