British Journal of Radiology (2007) 80, e81-e84
© 2007 British Institute of Radiology
doi: 10.1259/bjr/47379807
Endovascular therapy for massive haemothorax caused by ruptured extracranial vertebral artery aneurysm with neurofibromatosis Type 1
M Hieda, MD
1
N Toyota, MD
1
H Kakizawa, MD
1
N Hirai, MD
1
T Tachikake, MD
1
Y Yahiro, MD
1
Y Iwasaki, MD
2
J Horiguchi, MD
1 and
K Ito, MD
1
1 Department of Radiology, 2 Department of Emergency and Critical Care Medicine, Division of Medical Intelligence and Informatics, Programs for Applied Biomedicine, Graduate School of Biomedical Science, Hiroshima University, Hiroshima, Japan
Correspondence: M Hieda, 1-2-3, Kasumi-cho, Minami-ku, Hiroshima, 734-8551, Japan. E-mail: hieda{at}hiroshima-u.ac.jp
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Abstract
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Extracranial vertebral artery aneurysm is uncommon, and the common cause is penetrating trauma. Rupture of extracranial vertebral artery aneurysm into the thoracic cavity is extremely rare and fatal due to haemorrhagic shock by massive haemothorax. We report an intrathoracic rupture of the extracranial vertebral artery aneurysm with neurofibromatosis Type 1, successfully treated by coil and liquid embolisation.
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Introduction
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Extracranial vertebral artery aneurysm is rare and a common cause is penetrating trauma [1]. Other causal factors are arteriosclerosis, syphilis, rheumatoid arthritis, vertebral fracture and inherited disorders [2, 3]. Rupture of the aneurysm into the thoracic cavity is extremely rare [4] and is fatal due to haemorrhagic shock through intrathoracic massive haemorrhage. To our knowledge, there is only one reported case of vertebral artery aneurysm rupturing into the thoracic cavity with neurofibromatosis Type 1 (NF Type 1) [5]. Herein we present the first case of a spontaneous rupture of an extracranial vertebral artery aneurysm with NF Type 1 into the thoracic cavity successfully treated by endovascular therapy.
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Case report
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A 36-year-old woman was admitted to our emergency room presenting with haemoptysis, back pain, chest pain and dyspnoea. There was nothing particular about the past and family history. She became comatose instantly and her systolic blood pressure was less than 90 mmHg and haemoglobin level was 7.6 g dl–1. In spite of rapid administration of vasopressors and blood transfusion, the systolic pressure was around 40–50 mmHg and she was intubated.
CT showed left intrathoracic massive haemorrhage and a 4.9 cmx3.7 cm aneurysm with mural thrombus in the left thoracic outlet (Figure 1
). Rupture of the left extracranial vertebral artery aneurysm was suspected. After placement of a thoracic drainage tube, she underwent endovascular therapy. Informed consent was obtained from her family.
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Figure 1. (a) Axial CT image shows an aneurysm in the left thoracic outlet (arrow) and extravasation (arrowhead). (b) Reformatted coronal CT image reveals a 4.9 cmx3.7 cm aneurysm with mural thrombus (arrow) and massive intrathoracic haemorrhage (arrowhead).
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An angiogram revealed that an aneurysm located close to the ostium of the left vertebral artery had ruptured into the left thoracic cavity (Figure 2). Although a balloon occlusion test was impossible, the right vertebral artery was present within normal size (Figure 2a). A microcatheter was advanced into the distal side of the left vertebral artery aneurysm to isolate the aneurysm by coil embolisation. This was followed by administration of a total amount of 2500 IU heparin (Figure 2c), and the distal side of the aneurysm was occluded with interlocking detachable coils (IDC; Boston Scientific/Target Therapeutics, Fremont, CA) and microcoils (Tornado Embolization Coil; Cook, Bloomington, IN) (Figure 3a). Although embolisation of the proximal side of the artery was attempted next, the ostium of the aneurysm was so close to the left subclavian artery that coil embolisation was impossible due to risk of coil migration into the main subclavian artery (Figure 3a). As the aneurysmal neck was also wide, coils migrated into the huge aneurysmal sac. The aneurysmal sac was therefore packed with coils as densely as possible (Figure 3b). In total, we placed seven microcoils (one 7 mm/3 mm, three 6 mm/2 mm, three 10 mm/5 mm in size) and nine interlocking detachable coils (one 6 mm/20 cm, one 7 mm/20 cm, three 12 mm/20 cm, three 10 mm/20 cm, one 5 mm/12 cm in size). However, as extravasation was still present, injection of liquid embolic agent [n-butyl cyanoacrylate (NBCA) B. Braun, Tuttlingen, Germany] was attempted. NBCA was mixed with iodized oil (Lipiodol; Andre Guerbet, Aulnaysous-Bois, France) at an equal ratio of NBCA to Lipiodol. After injection of a total amount of 0.8 ml mixture, an arteriogram demonstrated complete embolisation of the aneurysm, disappearance of extravasation and preservation of the left subclavian artery (Figure 3c). The patient's blood pressure increased after embolisation. CT showed retention of the NBCA/Lipiodol mixture in the aneurysm and in the proximal side of the left vertebral artery. Haemoptysis due to perforation of the aneurysm into the left lung stopped.
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Figure 2. (a) Aortogram shows an aneurysm (arrow) in the left thoracic outlet and the right vertebral artery is present within normal size (arrowhead). (b) Left subclavian arteriogram reveals that the aneurysm (arrow) is located close to the ostium of the left vertebral artery. (c) The microcatheter is inside the aneurysm of the left vertebral artery.
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Figure 3. (a) Left vertebral arteriogram during coil embolisation shows that coils migrated into the huge aneurysmal sac (arrow). The distal portion of the left vertebral artery was occluded with microcoils (arrowhead). (b) Left subclavian arteriogram, after packing the aneurysm with coils, shows incomplete embolisation and extravasation (arrow). (c). Left subclavian arteriogram, post-liquid embolisation, shows that there was no aneurysm and no extravasation into the left thoracic cavity.
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MRI after embolisation revealed spotty cerebellar infarcts caused by microthrombi probably created by the vertebral artery embolisation. A few days later, brain oedema caused by post-resuscitative encephalopathy occurred. Although brain oedema improved 1 week later, decorticate rigidity appeared possibly due to hypoxic encephalopathy. It took about 3 h from the start of symptoms to intervention. However, if the aneurysm had not been embolised, uncontrollable bleeding into the thoracic cavity would have caused death. At a later date, it was discovered that the patient had NF Type 1 due to the evidence of café au lait spots and neurofibromas on the skin.
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Discussion
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Extracranial vertebral artery aneurysm is extremely rare. It is caused chiefly by penetrating wounds to the neck, most commonly gunshot or stab wounds [1]. In addition, non-penetrating diseases and conditions such as arteriosclerosis, syphilis, rheumatoid arthritis and vertebral fracture have been reported as causal factors [2]. Although there was no such causal factor in our case, it was discovered after therapy that the patient had NF Type 1 that is rarely associated with extracranial vertebral artery aneurysm. NF type 1 is complicated by abnormality of the blood vessels [6]. Vascular changes such as aneurysm are due to the fragility of the vascular wall, secondary to NF Type 1 because of proliferation of neurofibromatosis and dysplastic smooth muscle [7]. Although eight cases of vertebral artery aneurysm with NF Type 1 have previously been reported in the literature, rupture of an aneurysm into the thoracic cavity is reported in only one case that unfortunately ended fatally despite vigorous efforts to save the patient [5]. There have also been other cases of ruptured aneurysms elsewhere in the arterial tree. Our case is the first reported case to date saved by endovascular therapy.
Treatment options for vertebral artery aneurysms include resection of the aneurysm with ligation, direct repair of the vertebral artery and endovascular therapy [1]. Although ligation of the vertebral artery in the presence of a normal contralateral vertebral artery has been well tolerated, neurological complications after ligation occur in as many as 8% of cases [4]. The risk of neurological deficits is higher in patients who lack blood flow in the contralateral vertebral artery. Another treatment option would have been to coil embolise the vertebral artery distal to the aneurysm and then isolate the aneurysm by deployment of a covered stent across its origin. But it was difficult to apply this method in such a life-threatening situation due to non-availability of the appropriate stent-graft in our hospital. We first chose metallic coils as the standard embolic material to control massive extravasation. However, coil embolisation resulted in incomplete embolisation because the ostium of the aneurysm was so close to the left subclavian artery that it risked occluding the main trunk of the left subclavian artery due to coil migration. Therefore, a mixture of NBCA and Lipiodol was chosen as an alternative embolic material. Though the existence of a normal contralateral vertebral artery was confirmed, spotty cerebellar infarcts occurred after embolisation. We believed that microthrombi in the distal occluded vessels had scattered to the cerebellum. However, in our case, NBCA played a critical role in occluding the aneurysm successfully and preserving the left subclavian artery.
In conclusion, intrathoracic haemorrhage, although rare, should be considered as one of the major complications of vertebral artery aneurysm rupture with NF Type 1. Prompt diagnosis and suitable therapy are paramount in order to save the patient without neurological deficits or hypoxic encephalopathy. In our case, we determined that the aneurysm had been present for a long time as CT revealed mural thrombus in the aneurysm. Earlier diagnosis and preventive therapy of rupture are important.
Received for publication January 9, 2006.
Revision received March 22, 2006.
Accepted for publication April 6, 2006.
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References
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- Anand VK, Raila FA, McAuley JR, Reed JM. Large pseudoaneurysm of the extracranial vertebral artery. Otolaryngol Head Neck Surg 1993. 109:1057–60.
- Sumimura J, Nakao K, Miyata M, Kamiike W, Yokota H, Kawashima Y. Vertebral aneurysm of the neck. J Cardiovasc Surg (Torino) 1988. 29:63–5.
- Reid JD, Weigelt FA. Forty-three cases of vertebral artery trauma. J Trauma 1988. 28:1007–12.
- Blickenstaff KL, Weaver FA, Yellin AE, Stain SC, Finck E. Trends in the management of traumatic vertebral artery injuries. Am J Surg 1989. 70:489–91.
- Miyazaki T, Ohta F, Daisu M, Hoshii Y. Extracranial vertebral artery aneurysm ruptured into the thoracic cavity with neurofibromatosis type 1: case report. Neurosurgery 2004. 54:1517–21.
- Greene JF, Jr, Fitzwater JE, Burgess J. Arterial lesion associated with neurofibromatosis. Am J Clin Pathol 1974. 62:481–7.
- Deans WR, Bloch S, Leibrock L, Berman BM, Skultety FM. Arteriovenous fistula in patients with neurofibromatosis. Radiology 1982. 144:103–7.
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Abstract
Introduction
