Ann Thorac Surg 2002;73:958-960
© 2002 The Society of Thoracic Surgeons
Case report
Intramural hematoma of the thoracic aorta in a woman with neurofibromatosis
Omar A. Ali, MD*a,
Nsidinanya Okike, MDa,
Robert G. Hogan, MDa,
Richard C. Becker, MDa
a University of Massachusetts-Memorial Medical Center, Worcester, Massachusetts, USA
Accepted for publication June 1, 2001.
* Address reprint requests to Dr Ali, University of Massachusetts-Memorial Medical Center, 55 Lake Ave N, Worcester, MA 01655, USA
e-mail: oali23{at}aol.com
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Abstract
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The natural history and preferred approach to patients with intramural hematoma of the aorta remains controversial. We describe herein the management of the case of a 45-year-old woman with acute onset of chest pain, intramural hematoma of the arch and proximal descending aorta, and left pleural effusion. In this particular case, a nonsurgical approach led to complete recovery of the patient and documented resolution of the hematoma.
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Introduction
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Intramural hematoma is a localized hemorrhage within the wall of the aorta in the absence of a defined intimal flap. Although the mechanism and natural history of this clinical entity are poorly defined, rupture of the vaso vasorum is believed to be the inciting event. A patient with neurofibromatosis presented with intramural hematoma. Her clinical presentation appeared to be the result of her underlying disease.
A 45-year-old woman who had been diagnosed with neurofibromatosis (von Recklinghausen’s disease) 5 years earlier presented to an outlying hospital following the sudden onset of severe left-sided subscapular pain.
The pain was described as sharp in character and as radiating to the anterior chest. Initial blood pressure was 85 mm Hg systolic (in both arms) with a heart rate of 76 beats per minute. Breath sounds were decreased over the left lung. Examination of the heart and abdomen was unremarkable. The neurologic examination was normal, and the peripheral pulses were symmetric. The physical examination was otherwise notable for multiple neurofibromas on the face and upper extremities. A plain chest radiograph revealed a left pleural effusion. The mediastinal diameter was within normal limits. Computed tomography of the chest with intravenous contrast (Fig 1)
demonstrated a large mediastinal hematoma with a contiguous left pleural effusion. There was no evidence of aortic dissection. The initial hemoglobin level was 9.8 g/dL. Following fluid resuscitation, the patient was transferred to our facility for further evaluation.
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Fig 1. Computed tomographic scan demonstrates a mediastinal hematoma (a) and contiguous left pleural effusion (b).
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Upon arrival, the patient underwent transesophageal echocardiography (Fig 2),
which showed intramural hematoma without evidence of an intimal flap. Contrast aortography was performed, demonstrating a lack of run-off through a number of intercostal vessels without evidence of intimal tear. A diagnosis was made of intramural hematoma involving the distal aortic arch and the descending aorta with subsequent bleeding into the mediastinum and pleural cavity. A chest tube was placed, and 800 mL of blood was drained.
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Fig 2. Transesophageal echocardiography demonstrates intramural hematoma (arrow) without evidence of intimal flap.
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A decision was made to treat the patient conservatively. She was closely monitored in the intensive care unit, and ß-blocker therapy was initiated for heart rate and blood pressure control. The patient’s hematocrit responded to initial blood transfusion, and there was no evidence of recurrent bleeding. A computed tomographic scan obtained 1 month after hospital discharge (Fig 3) showed complete resolution of the mediastinal hematoma and pleural effusion without evidence of aortic dissection or underlying tumor. A subsequent magnetic resonance imaging scan at 3 months demonstrated no underlying pathology.
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Fig 3. Follow-up computed tomographic scan at 1 month demonstrates complete resolution of the mediastinal hematoma and pleural effusion.
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Comment
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Neurofibromatosis is an autosomal disorder of both mesoderm and ectoderm dysplasia commonly characterized by neurofibromas and café-au-lait spots [1]. Though primarily a disease involving neurocutaneous tissues, vascular lesions have been described, with an incidence of 3.6% by one series [2]. Cases detailing neurofibromatous involvement of the adventitia of blood vessels have also been published [3]. Because neurofibromatosis is a disorder characterized by tumor formation in tissues of neural crest origin and because cells contributing to vascular smooth muscle proliferation in the aorta and its branches are derived from neural crest, it has been suggested that blood vessels may express the NF1 mutation [4]. If this is indeed the case, abnormal proliferation of neural crest cells could account for blood vessel degeneration [1] and subsequent hemorrhage.
In addition to being at risk for vessel wall abnormalities, patients with neurofibromatosis can experience hemorrhage within the tumors themselves. Numerous cases of hemorrhage into neurofibromas have been reported, several of these with fatal outcome [1]. Fuyano and colleagues [5] described the case of a 17-year-old boy with neurofibromatosis and spontaneous hemothorax. Following drainage of 1,700 mL of blood, thoracotomy was performed, revealing an intrathoracic hypervascular tumor that had ruptured. Rupture of the subclavian artery, intercostal artery, and intercostal vein have all been described in patients with neurofibromatosis [5]. In one case, an autopsy confirmed that the adventitia and media of the artery in the region of the rupture were invaded by neurofibroma [1]. Our case is unusual in reporting a patient with neurofibromatosis and an intramural hematoma of the aorta.
Intramural hematoma is characterized by an aortic wall hematoma developing in the absence of a demonstrable intimal flap or penetrating ulceration [6]. In our patient, contrast aortography demonstrated an absence of blood flow emerging from a number of intercostal vessels, secondary to blood collection within the aortic wall. There was no intimal flap evident on transesophageal echocardiography, computed tomographic scan, or magnetic resonance imaging. The source of hemorrhage was likely related to an underlying tumor or abnormal smooth muscle proliferation stemming from the patient’s underlying disease. It is believed that intramural hematomas of the aorta represent spontaneous hemorrhage of the aortic vasovasorum [6]. In this case, tumor involvement may have caused rupture of the vasovasorum and subsequent intramural hemorrhage. The patient remained stable during her hospital course, without evidence of recurrent bleeding.
In summary, neurofibromatosis is associated with vascular abnormalities that can either directly (smooth muscle proliferation) or indirectly (tumor in vessel) result in hemorrhagic events. Intramural hematoma of the aorta should be considered among such patients in whom the presenting signs and symptoms elicit an index of suspicion. The natural history of aortic intramural hematoma has not been fully elucidated. Though it is a pathologically distinct entity, there exists some clinical overlap with aortic dissection and penetrating ulcer. Currently, the management of aortic intramural hematoma is indistinct from that of aortic dissection. Involvement of the descending aorta requires blood pressure control and monitoring. A nonsurgical approach is warranted unless complications develop. Patients with hematoma involving the ascending aorta (type A) have a higher morbidity and mortality rate than those with hematoma involving the descending aorta (type B), and they should be treated surgically [6, 7]. Several series have demonstrated a substantial reduction in mortality in those patients with hematoma of the ascending aorta whose cases were managed surgically [7]. In our patient, a decision was made not to proceed with surgical intervention, given the unclear site of initial hemorrhage and lack of involvement of the proximal aorta. The patient has remained active and free of events now 24 months from her initial hospitalization.
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References
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Abstract
Introduction
