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NAION or not NAION? A literature review of pathogenesis and differential diagnosis of anterior ischaemic optic neuropathies

Eye volume 38, pages 418–425 (2024)Cite this article

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A Correction to this article was published on 13 December 2023

This article has been updated

Abstract

Purpose

To offer a comprehensive review of the available data regarding non-arteritic anterior ischaemic optic neuropathy and its phenocopies, focusing on the current evidence to support the different existing aetiopathogenic hypotheses for the development of these conditions.

Conclusions and importance

Due to the limited array of responses of the neural tissue and other retinal structures, different aetiopathogenic mechanisms may result in a similar clinical picture. Moreover, when the insult occurs within a confined space, such as the optic nerve or the optic nerve head, in which different tissues (neural, glial, vascular) are highly interconnected and packed together, determining the primary noxa can be challenging and may lead to misdiagnosis. Anterior ischaemic optic neuropathy is a condition most clinicians will face during their everyday work, and it is important to correctly differentiate among resembling pathologies affecting the optic nerve to avoid unnecessary diagnostic procedures. Combining a good clinical history and multimodal imaging can assist diagnosis in most cases. The key remains to combine demographic data (e.g. age), with ophthalmic data (e.g. refractive error), systemic data (e.g. comorbidities and medication), imaging data (e.g. retinal OCT) with topographic signs (e.g. focal neurology).

Methodology

Papers relevant for this work were obtained from the MEDLINE and Embase databases by using the PubMed search engine. One author (MPMG) performed the search and selected only publications with relevant information about the aetiology, pathogenic mechanisms, risk factors as well as clinical characteristics of phenocopies (such as vitreopapillary traction, intrapapillary haemorrhage with adjacent peripapillary subretinal haemorrhage or diabetic papillopathy) of non-arteritic anterior ischaemic optic neuropathy (NAION). The terms “non-arteritic ischaemic optic neuropathy/NAION”, “vitreopapillary traction”, “vitreopapillary traction AND non-arteritic ischaemic optic neuropathy/NAION”, “posterior vitreous detachment AND non-arteritic ischaemic optic neuropathy/NAION”, “central retinal vein occlusion AND non-arteritic ischaemic optic neuropathy/NAION”, “disc oedema/disc oedema”, “diabetes mellitus AND non-arteritic ischaemic optic neuropathy/NAION” and “diabetic papillopathy” were searched on PubMed. From each of these searches, publications were selected based on their title, obtaining a total of 115 papers. All papers not written in English were then excluded, and those whose abstracts were not deemed relevant for our review, according to the aforementioned criteria. Subsequent scrutiny of the main text of the remaining publications led us (MPMG, AP, ZS) to include references which had not been selected during our first search, as their titles did not contain the previously mentioned MeSH terms, due to their significantly relevant contents for our work. A total of 62 publications were finally consulted for our review. The literature review was last updated on 24-Aug-2022.

摘要

目的: 全面回顾非动脉炎性前缺血性视神经病变及其的可用数据, 重点关注目前证据, 以支持目前这些疾病进展的不同致病假说。结论和重要性: 由于神经组织和其他视网膜结构的反应有限, 不同的致病机制可能导致相似的临床表现。此外, 当病变发生在一个有限的空间内时, 如视神经或视神经头部, 其中不同的组织(神经、神经胶质、血管)高度相互关联并聚集在一起, 确定原发性病变具有挑战性, 并可能导致误诊。前缺血性视神经病变是大多数临床医生在日常工作中都会面临的情况, 正确区分视神经的相似病变对于避免不必要的诊断程序非常重要。在大多数情况下, 结合良好的临床病史和多模式成像可以帮助诊断。关键仍然是将人口统计数据(例如年龄)与眼科数据(例如眼屈光不正)、系统数据(例如合并症和药物)、图像数据(例如视网膜OCT)与地形图标记(例如局灶性神经病学)结合起来。

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Fig. 1: This diagram depicts the proposed site of occlusion of the short posterior ciliary arteries (SPCAs) as the most widely accepted cause of ischaemia in non-arteritic anterior optic neuropathy (NAION).
Fig. 2: Flowchart depicting the venous aetiology for ischemia in NAION as proposed by Levin et al.
Fig. 3: OCT Spectralis scans through the right optic disc of an 86-year-old female.
Fig. 4: Multimodal imaging of a 22-year-old female, who was diagnosed with intrapapillary haemorrhage with adjacent peripapillary subretinal haemorrhage (IHAPSH) in her right eye.
Fig. 5: Fundus pseudocolour image of the right eye of an 86-year-old female diagnosed with non-arteritic anterior optic neuropathy (NAION).
Fig. 6: Flowchart exemplifying management of patients diagnosed with anterior vasculopathic optic neuropathy in our clinic.

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Data availability

The authors confirm that the data supporting the findings of this study are available within the article.

Change history

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Authors and Affiliations

  1. Moorfields Eye Hospital, London, UK

    M. Pilar Martin-Gutierrez, Axel Petzold & Zubin Saihan

  2. UCL Institute of Ophthalmology, London, UK

    Axel Petzold

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All authors attest that they meet the current ICMJE criteria for Authorship. MPMG was responsible for conducting the literature search, extracting and analysing data, updating reference lists and writing the report. AP and ZS analysed data and interpreted results, as well as revised and contributed to writing the report.

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Correspondence to M. Pilar Martin-Gutierrez.

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The original online version of this article was revised. In the subsection ‘Vasculopathic factors’, the following sentences have been corrected: “However, they differ in subsequent structural damage as evidenced by OCT changes, as both may show thinning of the retinal nerve fibre layer and macular ganglion cell layer, but thinning of the macular inner nuclear layer, typically present in NAION, is not observed in diabetic papillopathy (31). Also, although the exact aetiopathogenic mechanism of a diabetic papillopathy has not been fully elucidated, it seems to be different to the acute onset of ischemia in NAION.” The updated version reads as follows: “NAION and diabetic papillopathy may present with similar clinical features in the initial presentation, as well as subsequent structural damage as evidenced by OCT changes, as both may show thinning of the retinal nerve fibre layer and macular ganglion cell layer, with preservation of the macular inner nuclear layer (31). However, although the exact aetiopathogenic mechanism of a diabetic papillopathy has not been fully elucidated, it seems to be different to the acute onset of ischemia in NAION.”

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Martin-Gutierrez, M.P., Petzold, A. & Saihan, Z. NAION or not NAION? A literature review of pathogenesis and differential diagnosis of anterior ischaemic optic neuropathies. Eye 38, 418–425 (2024). https://doi.org/10.1038/s41433-023-02716-4

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