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Chronic Aortic Dissection as a Cause of Fever of Unknown Origin
from Southern Medical Journal Posted 01/15/2003.
Luis Gorospe, MD,
África Sendino, MD, Ph.D,
Raquel Pacheco, MD,
Ana Alonso, MD,
Francisco J. Barbado, MD, Ph.D,
Juan J. Vázquez, MD, Ph.D
Abstract and Introduction
Abstract
Chronic aortic dissection presenting as a prolonged febrile syndrome is an uncommon condition. We believe that only 22 cases have been previously reported. We present a case of a patient with an aortic dissection whose diagnosis was delayed because persistent fever, malaise, and night sweats dominated his clinical picture. These complaints may be accompanied by an increased erythrocyte sedimentation rate, leukocytosis, thrombocytosis, and anemia of chronic disease. Knowledge of this atypical presentation, a high degree of suspicion, and investigation using an adequate imaging method will help to avoid missing this potentially lethal entity.
Introduction
The presentation of aortic dissection may be more subtle than the classic textbook description of an acutely ill patient with excruciating chest pain of abrupt onset. In rare cases, chronic aortic dissection can have atypical prominent features, such as persistent fever, night sweats, malaise, and elevated acute phase response laboratory parameters. This misleading clinical picture may cause the underlying diagnosis to be delayed and may prompt an extensive but unfruitful search for a systemic illness. We describe one such case in which the patient had prolonged high fever associated with chronic dissection of the thoracic aorta that was not detected when he was first admitted to hospital. We also review reported cases of patients with chronic aortic dissection and prolonged febrile syndrome.
Case Report
A 61-year-old man was admitted to our hospital for evaluation of self-limited, moderately intense upper abdominal pain radiating to the back, of sudden onset, associated with a 2-week history of fever and profuse night sweats. Seventeen days before admission, the patient was seen in the emergency department after an abrupt episode of dull retrosternal and upper abdominal pain that radiated to the left arm and was accompanied by diaphoresis. At that time, he was found to have blood pressure of 210/120 mm Hg and a tortuous aorta shown by a chest x-ray film, but normal findings on abdominal ultrasonography, unremarkable serial electrocardiograms, and normal cardiac enzyme values. Once the blood pressure was controlled, he was sent home. However, 2 days after this episode, night sweats and fever developed with daily temperature spikes up to 38.8°C (101.8°F). The patient's medical history was significant only for hypertension of 6 years' duration, treated with captopril and atenolol.
Physical examination on admission was unremarkable, except for a temperature of 39°C, an enlarged right axillary lymph node, and blood pressure of 160/100 mm Hg. Serial cardiac enzyme determinations and electrocardiographic findings were normal. A chest x-ray film disclosed only a tortuous aorta. Urinalysis, abdominal x-ray films, and ultrasonography showed no abnormalities. Initial laboratory data revealed a white blood cell count of 13,700/mm3, a platelet count of 426,000/mm3, and an elevated erythrocyte sedimentation rate (ESR) (84 mm/hr), with normal hemoglobin and biochemical test values. Serum albumin and globulin levels were normal, as were results of protein electrophoresis. Additional studies included several negative blood and urine cultures, repeated negative serologic tests for various infectious agents (syphilis, typhoid fever, brucella, rickettsiae, toxoplasma, hepatitis B and C viruses, Epstein-Barr virus, and cytomegalovirus) and autoantibodies, and normal C3 and C4 levels. Although a
purified protein derivative skin test was positive, an extensive search for acid-fast bacilli (including mycobacterial cultures of samples from blood, urine, sputum, and right axillary lymph node) was negative.
Gallium and technetium scans, upper gastrointestinal series, abdominal computed tomography (CT), and ocular funduscopy were all unremarkable. Transthoracic echocardiography showed mild left ventricular hypertrophy, but no pericardial effusion or vegetations. However, thoracic CT revealed widening of the descending aorta and an intramural thrombus within a false lumen in the left anterolateral wall of the descending thoracic aorta, which extended from the level of the origin of the left subclavian artery to just above the diaphragm (Fig 1). This finding was confirmed by transesophageal echocardiography (Fig 2) and thoracic magnetic resonance imaging (MRI) (Fig 3).
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Figure 1. (click image to zoom) Axial contrast-enhanced computed tomography of chest shows intramural thrombus of descending aorta.
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Figure 2. (click image to zoom) Transesophageal echocardiogram shows hematoma within false lumen of aortic dissection.
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Figure 3. (click image to zoom) Axial T1-weighted magnetic resonance imaging of mediastinum and great vessels shows intramural thrombus in left anterolateral wall of descending aorta, producing medial displacement of intima.
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Chronic aortic dissection type III was diagnosed, and conservative medical management was chosen after surgical consultations. Until the eighth hospital day, the patient had daily spiking temperatures to 39°C. During this time, he had only one brief episode of mild, dull, retrosternal pain, but electrocardiogram and cardiac enzyme levels remained normal. Over the subsequent days, fever spontaneously remitted, and he was discharged on the 23rd hospital day, receiving a regimen of hypotensive medications (captopril and atenolol at higher doses), which controlled his blood pressure at approximately 120/80 mm Hg. The patient has remained afebrile, and the laboratory parameters of acute inflammatory response have returned to normal levels.
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