Non-arteritic anterior ischemic optic neuropathy with Cilioretinal artery occlusion: a case report
To describe a peculiar case of concurrent non-arteritic anterior ischemic optic neuropathy (NAION) and cilioretinal arteries occlusion (CLRAO) without other causative agents which responded well to intravenous and intravitreal injection of corticosteroids.
A 41-year-old woman presented with painless vision loss in the right eye for 1 week. Fundus examinations showed marked disc swelling, flame-shaped hemorrhage over the superior nerve fiber area, and well-demarcated retinal ischemia superior to the fovea in the right eye. Under the impression of NAION with branch retinal artery occlusion, the patient was treated with intravenous and intravitreal injection of corticosteroids. Two months later, as the disc swelling and retinal ischemia resolved, we found that the occluded artery was the cilioretinal artery and not the ordinary branch retinal artery.
CLRAO may be concomitant with the setting of NAION, the physicians should be aware that CLRAO may be misinterpreted as BRAO owing to profound disc edema during the early stages of the disease.
KeywordsNon-arteritic anterior ischemic optic neuropathy Cilioretinal artery occlusion Branch retinal artery occlusion
Anterior ischemic optic neuropathy
Branch retinal artery occlusion
Cilioretinal arteries occlusion
Non-arteritic anterior ischemic optic neuropathy
Optical Coherence Tomography
Optic nerve head
Posterior ciliary arteries
Non-arteritic anterior ischemic optic neuropathy (NAION) is due to acute ischemia of the optic nerve head (ONH), whose main supply of blood is from the circulation of the posterior ciliary arteries (PCA). The vast majority of NAION cases result from transient non-perfusion or hypoperfusion of ONH circulation . Cilioretinal arteries also arise from short PCA. Thus, if retinal vascular occlusion occurs, the presence of a cilioretinal artery can significantly influence visual morbidity. It is interesting that although both the optic nerve head and cilioretinal arteries are supplied by PCA, concomitant anterior ischemic optic neuropathy (AION) and cilioretinal arteries occlusion (CLRAO) are uncommon in clinical practice. If it does occur, it is almost always arteritic and always pathognomonic for giant cell arteritis; other causes have been reported, including overdose of Viagra® . Here we report on a peculiar case involving concurrent NAION and CLRAO without other causative agents.
Discussion and conclusion
The cilioretinal artery is present in 20% of the population, and the PCA supplies the optic nerve head as well as the cilioretinal artery. When CLRAO is combined with AION, it is nearly pathognomonic for giant cell arteritis associated arteritic-AION . Our patient was relatively young, had normal ESR and did not have temporal tenderness or headache, which made giant cell arteritis unlikely. Furthermore, unlike small peripapillary hemorrhaging in most NAION cases, profound hemorrhaging along superior fiber area revealed poorer local circulation compared with others. In our case, marked optic disc swelling inherent to NAION may have resulted in local compartmental effects around the disc, which may have further compromised the circulation of the cilioretinal artery. This then led to concomitant CLRAO. Furthermore, the physicians should be aware that CLRAO may be misinterpreted as BRAO owing to profound disc edema during the early stages of the disease.
Little is known regarding the optimal management of NAION. The main debate in recent decades is whether or not to use steroids . Although there is no consensus, steroids are thought to decrease optic disc edema and improve circulation in the optic nerve head . The visual loss in NAION was investigated to be due not just to ischemia but also to associated inflammation, and this has shown in both animals with experimental NAION and in the few cases of acute NAION in humans that have been studied . Some case series also showed treatment efficacy in intravitreal injection of triamcinolone for high intraocular steroid concentration without systemic adverse effects . Our case showed that CLRAO may be concomitant with the setting of NAION. High dose intravenous corticosteroids and intravitreal injection of triamcinolone may decrease optic disc edema and improve circulation in both the optic nerve head and cilioretinal artery with favorable results.
In conclusion, CLRAO may be concomitant with the setting of NAION, the physicians should be aware that CLRAO may be misinterpreted as BRAO owing to profound disc edema during the early stages of the disease.
We thank the patient for providing permission to share her information.
YYY and MSH had full access to all of the data in the study and take responsibility for the integrity of the data and the accuracy of the data analysis. Concept and design: MSH. Acquisition, analysis, or interpretation of data: YYY and MSH. Both authors read and approved the final manuscript.
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Consent for publication
Written informed consent was obtained from the patient for publication of this case report and any accompanying images.
The authors declare that they have no competing interests.
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