Asian Cardiovasc Thorac Ann 2000;8:268-270
© 2000 Asia Publishing EXchange Pte Ltd
Cerebral Circulation Via Right Vertebral Artery in Takayasu's Arteritis
Mehmet 
ah Topcuo
lu, MD,
Hafize Yaliniz, MD,
Acar Tokcan, MD,
Hacer Bozdemir, MD,1,
Yakup Sarica, MD,1
Department of Cardiovascular Surgery
1
Department of Neurology
Çukurova University School of Medicine
Adana, Turkey
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For reprint information contact: Mehmet ah Topcuolu, MD Tel: 90 322 338 6627 Fax: 90 322 338 6656 email: sahtopcu{at}yahoo.com Department of Cardiovascular Surgery, Çukurova University School of Medicine, Balcali, Adana 01330, Turkey.
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Abstract
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A 42-year-old woman had left fronto-orbital aching and amaurosis for 6 months. Fluorescein angiography of the left eye showed vasculitis. Aortography revealed total occlusion of both subclavian arteries, both carotid arteries, and the left vertebral artery, with serious narrowing of the abdominal aorta. The right vertebral artery was spared. Blood flow in the middle and anterior cerebral arteries was normal in spin-echo and phase-contrast magnetic resonance studies. Immuno-histochemical findings indicated Takayasu's arteritis.
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Introduction
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Takayasu's arteritis mainly involves the territory of the large aortopulmonary arteries. The epidemiology of this disease is not well known except for a very strong female predominance (male to female ratio is 1:7) and it occurs mostly in women under 40 years of age.
1
,
2
The disease is frequent in Japan, India, and other parts of Asia, as well as South America; it is less common in Europe and North America.
2
The early preocclusive phase of Takayasu's arteritis is difficult to diagnose because of nonspecific symptoms of systemic inflammation; in one-third of cases, the history does not provide sufficient information for diagnosis.
1
Therefore, Takayasu's arteritis can be diag-nosed mostly in the late phase that is characterized by various occlusive vascular syndromes.
1
,
3
Severe cerebral ischemia, renal and pulmonary hypertension, pseudocoarc-tation syndrome, mesenteric, coronary, and peripheral ischemia may occur in various combinations, depending on the distribution of the disease.
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Case Report
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A 42-year-old woman suffering from left fronto-orbital pain and amaurosis for 6 months was treated with antimigraine agents. Because of gradual loss of vision and transient weakness of the left arm, she was admitted to the department of neurology. She denied any previous medical problems. On physical examination, the left arm was pulseless. The right arm pulse rate was 84 beats•min
–1
and blood pressure was 100/55 mm Hg. Lower limb pulses and arterial pressure were normal and there was no peripheral edema. Neurological findings were normal. Neurophthalmological examination showed that the right eye was completely normal but angiomatoses enlarge-ments were seen in the left papillar and peripapillary regions. The left pupil was fixed and dilated with vision reduced to finger counting at one meter. Reduced blood flow in the central retinal and posterior ciliary arteries was demonstrated by Doppler studies. Fundus fluorescein angiography of the left eye revealed leakage due to collateral vasculitis, peripheral microaneurysm, and retinal ischemia, while only small ischemic foci were seen on the right side.
Selective angiography of the aortic arch showed total occlusion of the left subclavian artery and bilateral common carotid arteries at the beginning of the trunk. The brachycephalic truncus and right subclavian artery were visible at the trunk, but the right subclavian artery was occluded after branching to the vertebral artery. Several collaterals emerging from the neck and shoulder region supplied the brachial artery (
Figure
1
). Abdominal angiography indicated serious narrowing of the abdominal aorta at the infrarenal level with a double renal artery on the right side (
Figure
2
). Spin-echo and phase-contrast magnetic resonance angiography revealed normal blood flow in the middle and anterior cerebral arteries (
Figures
3
and
4
). Computed tomography scans of the head, pulmonary perfusion scintigraphy, and routine hemogram and erythrocyte sedimentation rate were normal. Immuno-histochemical studies of the skin demonstrated immune complex accumulation on the vessel walls. These clinical and laboratory findings led to a diagnosis of Takayasu's arteritis. Prednisolone treatment (gradually tapered) was applied and surgical therapy was suggested, which the patient declined. She was discharged and failed to return for follow-up.
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Figure 1.
Selective angiography of the aortic arch showing cerebral arterial circulation to be totally dependent on the right vertebral artery.
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Figure 2.
Angiography of the abdominal aorta showing serious narrowing at the infrarenal level with a double renal artery on the right side.
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Figure 4.
Phase-contrast magnetic resonance imaging showing normal blood flow in the middle and anterior cerebral arteries.
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Discussion
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Because Takayasu's arteritis involves the aortic arch and its branches in almost 75% of cases, vertigo, syncope, transient ischemic attacks, stroke, blurred vision, diplopia, amaurosis fugax or persistent amaurosis are the most common presenting features. The most consistent finding for a diagnosis of Takayasu's arteritis is angiographically determined bilateral occlusion of the subclavian and common carotid arteries.
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,
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Occlusion of large arteries supplying the cephalic structures, without neurological symptoms, is a characteristic of this disease. However, it is very rare to find normal cerebral blood supply provided by the right vertebral artery only, as seen in this patient. Pokrovsky
1
noted one case of bilateral occlusion of the carotid arteries with vertebral artery occlusion on one side among 300 patients with Takayasu's arteritis, and recently, Elewaut and colleagues
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described a similar case. Normal cerebral blood flow and computed tomog-raphy scans, and a lack of neurological findings in spite of a history of amaurosis fugax and transient ischemic attacks in these patients, are compatible with our obser-vations.
The development of neurological changes due to infarction in the territory of the cerebral vessels depends on inter and intraindividual factors such as collateral circulation between intra and extracranial arteries. The circle of Willis is one of the most important bridges between left and right hemispheric circulation, as well as between the carotid and basilar circulation. During acute obstruction of any artery supplying the circle of Willis, hemodynamic compensation cannot contend, whereas chronic occlusive diseases such as Takayasu's arteritis, may allow develop-ment of collateral blood supply and postpone clinical catastrophe. Thus, occlusion of the internal carotid artery may be compensated by external carotid artery collaterals via the ophthalmic artery during the silent period (early phase) of the disease. Occlusion of the proximal vertebral artery may promote the opening of a number of anas-tomotic channels such as those between the occipital artery from the external carotid and muscular branches of the vertebral artery. Both ascending cervical arteries as well as the opposite vertebral artery are also important sources of blood supply to the brain. All such collaterals were well established in our patient, as seen angiogra-phically. Hence, normal blood flow in the intracranial vessels was indicated by Doppler studies, although only one vertebral artery supplied the cerebral circulation.
In Takayasu's disease, also known as nonspecific aorto-arteritis, classification is based on anatomic localization of lesions; type III infers involvement of both the abdominal aorta and the arcus. Medical therapy during the occlusive stage of this disease is controversial. It was reported by Hall and colleagues
6
that in pulseless patients receiving several months of steroid therapy, the chance of the pulse returning is 50% and that angiographic findings show improvements, although the general consensus is not in agreement. The effects of cytostatic and anticoagulant agents are also controversial. Surgical interventions such as bypass may have disappointing results and carry a mortality rate of 25% to 30%.
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In the occlusive stage of Takayasu's arteritis, bypass between the ascending aorta and the carotid or innominate artery is the selected method for palliation of cerebral ischemic events and slowing of progression.
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This patient declined surgery but her findings are reported because of their extreme rarity.
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References
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Pokrovsky AV. Nonspecific aortoarteritis. In: Rutherford RB, editor. Vascular surgery. 3rd ed. Philadelphia: Saunders, 1989:217–37.
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Elewaut DS, Duprez DA, De Buyzere ML, Kunnen MF, Clement DL. Total dependence of the cerebral circulation on the right vertebral artery in Takayasu's disease—a case report. Int J Angiology
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Abstract
Introduction
