Takayasu's arteritis (TA) is an inflammatory and obliterative disease of large and medium-sized arteries, with a predilection for the aorta and its major branches.1 Characteristic ophthalmic features may include hypotensive or hypertensive retinopathy.23 This study describes a patient with retinal arterial occlusion, who was subsequently found to have an aortic arch syndrome with clinical and radiological features compatible with TA.
A forty-year-old woman presented with left temporal headache and foggy vision in the left eye for ten days. There was no history of any other systemic illness, or easy fatigability. Ocular evaluation revealed visual acuity of light perception in the left eye, and 6/6 in the right. An examination of the left eye showed a normal anterior segment with a briskly reacting pupil. Ophthalmoscopy revealed a sheathed nasal branch retinal artery and absence of tertiary branches of the lower temporal arteriole, with wedge-shaped choroidal scars in the nasal mid-periphery (Figure 1), suggestive of Elschnig's wedge infarcts. There was no arterio-venous anastomosis. Fundus Fluorescein angiography (FFA) revealed initial dye transit of more than a minute, and showed non-filling of tertiary branches of the central retinal artery in the posterior pole, with an area of transmission defect nasal to the disc (Figure 2). The right eye was normal.
The patient was clinically diagnosed to have ocular ischaemia and subjected to a detailed systemic evaluation. Examination revealed absent pulses in the left upper limb. The blood pressure was unrecordable in the left upper limb, 76/60 in the right upper limb and 130/80 in the lower limbs. Aortography showed totally blocked ostia of the left subclavian and left common carotid arteries on the arch aortogram (Figure 3). The right subclavian was blocked in the third part, while the brachial artery was reformed through multiple collaterals. She was diagnosed to have an aortic arch syndrome, as is commonly seen in Takayasu aorto-arteritis.
The patient was unsuccessfully treated with oral Prednisolone 40 mg o.d. over three months, the retinal arterioles progressively attenuated and the optic disc turned pale (Figure 4).
Takayasu's disease is an uncommon inflammatory arteritis predominantly affecting the aorta, its branches and the pulmonary arteries. The ocular features in TA result from the ocular hypoperfusion secondary to obliteration of these arteries. Classical features of Takayasu retinopathy were described by Uyama and Asayama in 1976,4 and they include dilatation of small vessels, capillary microaneurysm formation, arterio-venous anastomosis and further ocular complications. The variability of ischaemic changes in TA probably depends upon the parts of the carotid arteries which are occluded, and the duration and rate of ocular vascular insufficiency, in addition to the development of collateral blood supply. Additional ophthalmological features may include hypertensive retinopathy secondary to renal artery stenosis.
The histopathological changes in TA are predominantly found in the outer part of the media and adventitia of the arteries; regions which are nourished by the vasa vasorum.5 This suggests that the primary involvement of small vessels also contributes to the development of the features of TA. It is now known that TA may affect small vessels as well, though the involvement of distal branch arteries is very uncommon. Although systemic small vessel involvement in TA567 has been described, retinal arterial involvement has not been reported.
In the present case, the involvement of distal branches of a retinal arteriole in the posterior pole was noted clinically, and confirmed on FFA. The clinical features of pulseless disease, along with radiological features of the left subclavian and common carotid artery stenosis were suggestive of TA. There was no evidence of retinal arteriolar embolus, and there was a progressive narrowing of the involved retinal arteriole. Based on these findings, we conclude that it was apparently a secondary arteriolar occlusion due to proximal involvement of the major vessels that caused the symptoms and signs in this patient.
A literature review using MEDLINE failed to reveal any mention of retinal arteriolar occlusion as part of TA. This case illustrates how a branch retinal arteriolar occlusion along with other features of ischaemic retinopathy such as a delayed arm-retina circulation time and choroidal infarcts, led to the ultimate diagnosis of this condition.
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