Asian Cardiovasc Thorac Ann 2007;15:e16-e19
© 2007 Asia Publishing EXchange Ltd
Treatment for Intercostal Arterial Aneurysm in Neurofibromatosis Type 1
Isao Matsumoto, MD,
Yasuhiko Ohta, MD,
Yoshio Tsunezuka, MD,
Masaya Tamura, MD,
Makoto Oda, MD,
Go Watanabe, MD
Department of General and Cardiothoracic Surgery, Kanazawa University, Kanazawa, Japan
For reprint information contact: Isao Matsumoto, MD, Tel: 81 76 265 2355, Fax: 81 76 222 6833, Email: mat{at}p2223.nsk.ne.jp, Department of General and Cardiothoracic Surgery, Kanazawa University, Takara-machi 13-1, Kanazawa 920-8641, Japan
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ABSTRACT
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We report a valuable case in which a spontaneous intercostal arterial aneurysmal rupture associated with neurofibromatosis type 1, and a re-rupture after embolization of the aneurysm were successfully treated by endovascular embolization without surgery. Our case calls for attention as it indicates that recurrent aneurysmal rupture can occur even after an embolization or surgery. It is important to carefully observe the course of the patient by follow-up computed tomography (CT) or angiography in such cases.
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INTRODUCTION
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Arterial vasculopathy in von Recklinghausen’s disease (neurofibromatosis type 1, NF-1) is a somewhat rare condition (incidence, 3.6% of patients).1 However, it has been suggested that all patients with neurofibromatosis have some degree of vasculopathy, because the lesions can remain clinically undetected. There have been several recent reports describing acute intrathoracic hemorrhage, which is frequently fatal in patients with neurofibromatosis.1–4 Most cases of hemothorax are caused by spontaneous arterial aneurysmal rupture. Although most such cases are treated surgically with emergency thoracotomy, a non-operative approach with endovascular embolization has been used to treat hemodynamically stable patients. However, although the aneurysm was successfully embolized, the patients underwent surgical intervention to remove hematoma clots and decortications.2–4 Some patients died as a result of fatal sudden bleeding, which had been initially controlled.1 Here, we report a case in which spontaneous intercostal arterial aneurysmal rupture associated with NF-1, and a re-rupture after embolization of the aneurysm were successfully treated by endovascular embolization without surgery.
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CASE REPORT
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A 49-year-old man presenting with left back pain visited the nearest hospital. The patient had a clinical history of NF-1. The left back pain and dyspnea suddenly deteriorated. A chest X-Ray revealed left massive pleural effusion (Figure 1A
). As hemothorax due to dissecting aortic aneurysm was suspected, the patient was referred to our hospital. On arrival at our hospital, the patient had an arterial pressure of 90/40 mm Hg, a heart rate of 120 beats·min–1, and a hemoglobin level of 11.5 g·dL–1. A chest CT scan revealed left massive pleural effusion with high-density areas, suggesting hemothorax. The CT findings did not indicate a dissecting aneurysm of the aorta. The CT failed to show a source of bleeding.
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Figure 1. (A) Chest X-Ray revealed left massive pleural effusion; (B) Chest CT scan revealed a pool of contrast medium in the 11th intercostal space.
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The patient was administered intravenous infusion of lactate Ringer solution, and a left chest tube was inserted. Approximately 1000 mL of blood was removed. After transfer to the intensive care unit, a non-operative approach was chosen because of the patient’s stable hemodynamic condition. However, bleeding from the chest tube persisted at a rate of 30 mL·hr–1. In order to decide whether to perform surgery, chest CT was performed 6 hours after arrival. This chest CT revealed a pool of contrast medium 3 cm in diameter in the 11th intercostal space (Figure 1B
). Therefore, intercostal angiography of the thoracic aorta was performed, and revealed a false aneurysm in the left 11th intercostal artery (Figure 2A). The 11th intercostal artery was contrasted selectively. The proximal extremitas of the aneurysm was embolized, using Tornado Embolization Microcoils (Cook Incorporated, Minnesota, MN, USA) and a Trufill Pushable Coil (Johnson & Johnson Cordis endovascular system, Miami, FL, USA). After embolization, the aneurysm was not detected (Figure 2B).
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Figure 2. (A)Intercostal angiography revealed a false aneurysm; (B) After embolization, the aneurysm was not detected.
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The patient’s general condition improved, and his back pain disappeared. Five days after embolization, severe back pain suddenly recurred. Chest CT revealed a new pool of contrast medium (diameter, 1.2 cm) at the aorta side of the embolic part of the 11th intercostal artery. Recurrence of intercostal aneurysmal rupture was diagnosed. Accordingly, angiography was performed again, revealing a new false aneurysm at the aorta side of the embolic part of the 11th intercostal artery (Figure 3A). Both the distal and proximal extremitas of the aneurysm were successfully embolized using Tornado Embolization Microcoils. Angiography confirmed the exclusion of the lesion and the absence of other potential bleeding foci (Figure 3B). The patient’s back pain disappeared, and his general condition improved again. After blood drainage ceased and the hematoma of the left chest cavity decreased, the chest tube was extubated. The patient was discharged from hospital 15 days later. At the last follow-up (12 months after discharge), the patient was free of symptoms, and a chest X-Ray did not show hematoma of the left chest cavity.
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Figure 3. (A) Angiography was performed again, revealing a new false aneurysm at the aorta side of the embolic section; (B) Angiography confirmed the exclusion of the lesion.
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DISCUSSION
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Spontaneous hemothorax is a life threatening complication of NF-1. The cause of hemothorax has been assumed to be arterial vasculopathy associated with neurofibromatosis, and especially with arterial aneurysmal rupture. Previous reports1–4 indicate that the most common sources of arterial bleeding among thoracic vessels are the intercostal and subclavian arteries. Other affected arteries that have been reported include the internal thoracic artery, the innominate artery, the thyrocervical trunk, the phrenic artery, and the vertebral artery. Bleeding appears to occur more frequently on the left side.1,4
There are 2 hypotheses proposing the cause of this arterial fragility.5 One hypothesis is that neurofibromatous invasion of the media reduces the strength of the vessel wall. The second hypothesis is that tissue compresses the vasa vasorum of the large artery, resulting in a weakened segment of the artery secondary to ischemia. Greene et al6 described 2 categories of vasculopathy: 1) a condition involving larger vessels such as the aorta and the carotid and proximal renal arteries, which are surrounded by neurofibromatous or ganglioneuromatous tissue in which intimal proliferation, thinning of the media, and fragmentation of elastic tissue leading to stenosis or aneurysm formation can occur; 2) a condition not related to neural malformation, but rather reflecting dysplasia of small vessels.
Pezzetta et al7 reviewed and discussed the prognosis of 20 patients with spontaneous hemothorax associated with NF-1. Fourteen of the patients in their review underwent thoracotomy, and only 9 of these patients survived. Overall mortality in their series was 45%. This high mortality rate is due to difficulty in identifying the bleeding source and its acute clinical course with hemodynamic compromise. There is a case report in which the patient’s condition was improved by chest drainage alone.7 However, most such patients eventually die due to uncontrollable bleeding. If the bleeding source is not identified, surgical intervention may be required at any time. If the bleeding source is identified, angiography and embolization should be considered. Additional aortic stent-grafting can be useful to control bleeding from an intercostal aneurysm.8
In the present case, we successfully controlled bleeding from an intercostal aneurysm using only embolization. We were able to effectively remove the intrathoracic hematoma by chest drainage, and thus successfully treated the patient without surgery. However, after the first embolization, the intercostal aneurysm recurred at the proximal side of the previously embolized section. This indicates arterial fragility particular to NF-1. It is well known that bleeding often occurs repeatedly in NF-1, and an uncontrollable bleeding will prove to be fatal.1 Although we were able to successfully treat the second rupture, our case calls for attention since it indicates that recurrent aneurysmal rupture can occur even after an embolization or after surgery. It is important to carefully observe the course of the patient, even if the patient’s condition appears to be stable. This case convinced us that follow-up enhanced CT scan or angiography must be performed periodically in such cases of an association with NF-1.
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REFERENCES
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- Dominguez J, Sancho C, Escalante E, Morera JR, Moya JA, Bernat R. Percutaneous treatment of a ruptured intercostal aneurysm presenting as massive hemothorax in a patient with type I neurofibromatosis. J Thorac Cardiovasc Surg 2002;124:1230–2.[Free Full Text]
- Leier CV, DeWan CJ, Anatasia LF. Fatal hemorrhage as a complication of neurofibromatosis. Vasc Surg 1972;6:98–101.[Medline]
- Greene JF Jr, Fitzwater JE, Burgess J. Arterial lesions associated with neurofibromatosis. Am J Clin Pathol 1974;62:481–7.[Medline]
- Pezzetta E, Paroz A, Ris HB, Martinet O. Spontaneous hemothorax associated with von Recklinghausen’s disease. Eur J Cardiothorac Surg 2003;23:1062–4.[Abstract/Free Full Text]
- Topel I, Steinbauer M, Paetzel C, Kasprzak PM. Endovascular therapy of a ruptured intercostal artery aneurysm. J Endovasc Ther 2004;11:219–21.[Medline]
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ABSTRACT
INTRODUCTION
