Diagnosis, Mechanisms, and Differentiation of Inflammatory Diseases of the Outer Retina: Photoreceptoritis versus Choriocapillaritis; A Multimodal Imaging Perspective
Background and aim: Inflammatory diseases that affect the outer retina do so by different mechanisms. Some of them result from the direct, primary involvement of the outer retina (primary photoreceptoritis) such as acute zonal outer occult retinopathy (AZOOR). Others affect the photoreceptors second...
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| Format: | Article |
| Language: | English |
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MDPI AG
2022-09-01
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| Series: | Diagnostics |
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| Online Access: | https://www.mdpi.com/2075-4418/12/9/2179 |
| _version_ | 1797489420001083392 |
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| author | Ioannis Papasavvas Alessandro Mantovani Carl P. Herbort |
| author_facet | Ioannis Papasavvas Alessandro Mantovani Carl P. Herbort |
| author_sort | Ioannis Papasavvas |
| collection | DOAJ |
| description | Background and aim: Inflammatory diseases that affect the outer retina do so by different mechanisms. Some of them result from the direct, primary involvement of the outer retina (primary photoreceptoritis) such as acute zonal outer occult retinopathy (AZOOR). Others affect the photoreceptors secondarily due to the inflammatory involvement of the choriocapillaris. This results in choriocapillaris non-perfusion that damages the photoreceptors due to the ensuing ischaemia, a mechanism characterising primary inflammatory choriocapillaropathies (PICCPs) such as multiple evanescent white dot syndrome (MEWDS), idiopathic multifocal choroiditis (MFC), and others. Thanks to multimodal imaging (MMI), it is now possible to differentiate between these two mechanisms of outer retinal damage. The aim of this study is to determine the MMI characteristics that allow us to differentiate primary photoreceptoritis, including AZOOR, from PICCPs such as MEWDS and MFC. Methods: A series of eight PICCPs cases (five typical MEWDS and three typical active MFC cases) and four typical primary photoreceptoritis/AZOOR cases (five eyes) that had undergone complete MMI investigation, including fundus photography (FP), blue light fundus autofluorescence (BL-FAF), spectral domain optical coherence tomography (SD-OCT), OCT angiography (OCT-A, when available), fluorescein angiography (FA), and indocyanine green angiography (ICGA) were analysed, pointing out the differences that allow us to distinguish primary photoreceptoritis from PICCPs. Results: All primary photoreceptoritis/AZOOR cases showed (1) faint fundus pallor around the fovea, (2) BL-FAF hyperautofluorescence, (3) loss of photoreceptor outer segments (PROS) on SD-OCT, (4) absence of choriocapillary drop-out on OCT-A, (5) normal FA or faint FA hyperfluorescence, and (6) conserved ICGA fluorescence/no hypofluorescent areas; (1), (2), (3), and (5) indicated loss of photoreceptor outer segments, and (4) and (6) indicated conserved choriocapillaris circulation. For PICCPs, (a) fundus showed discreet white dots or none (in MEWDS) and punched-out scars in MFC, (b) BL-FAF hyperautofluorescence, (c) loss of PROS on SD-OCT, (d) FA faint hyperfluorescence in MEWDS, also minimal in active MFC lesions (e) in all cases ICGA hypofluorescent areas; (b) and (c) indicating loss of PROS, and (e) indicating choriocapillaris non-perfusion in all cases. The OCT-A did not show consistent findings with faint or no capillary drop-out in MEWDS and MFC. Conclusions: MMI combining the SD-OCT and BL-FAF clearly showed loss of PROS in both groups, while the ICGA determined whether this was due to choriocapillaris non-perfusion in PICCPs or whether the choriocapillaris was intact in case of primary photoreceptoritis. The FA and OCT-A were found to be less useful and/or less sensitive for the appraisal of both these entities. |
| first_indexed | 2024-03-10T00:17:16Z |
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| id | doaj.art-c084ff82a54b4f38ab531235e34ca483 |
| institution | Directory Open Access Journal |
| issn | 2075-4418 |
| language | English |
| last_indexed | 2024-03-10T00:17:16Z |
| publishDate | 2022-09-01 |
| publisher | MDPI AG |
| record_format | Article |
| series | Diagnostics |
| spelling | doaj.art-c084ff82a54b4f38ab531235e34ca4832023-11-23T15:49:59ZengMDPI AGDiagnostics2075-44182022-09-01129217910.3390/diagnostics12092179Diagnosis, Mechanisms, and Differentiation of Inflammatory Diseases of the Outer Retina: Photoreceptoritis versus Choriocapillaritis; A Multimodal Imaging PerspectiveIoannis Papasavvas0Alessandro Mantovani1Carl P. Herbort2Retinal and Inflammatory Eye Diseases, Centre for Ophthalmic Specialized Care (COS), Rue Charles-Monnard 6, 1003 Lausanne, SwitzerlandDepartment of Ophthalmology, Valduce Hospital, 22100 Como, ItalyRetinal and Inflammatory Eye Diseases, Centre for Ophthalmic Specialized Care (COS), Rue Charles-Monnard 6, 1003 Lausanne, SwitzerlandBackground and aim: Inflammatory diseases that affect the outer retina do so by different mechanisms. Some of them result from the direct, primary involvement of the outer retina (primary photoreceptoritis) such as acute zonal outer occult retinopathy (AZOOR). Others affect the photoreceptors secondarily due to the inflammatory involvement of the choriocapillaris. This results in choriocapillaris non-perfusion that damages the photoreceptors due to the ensuing ischaemia, a mechanism characterising primary inflammatory choriocapillaropathies (PICCPs) such as multiple evanescent white dot syndrome (MEWDS), idiopathic multifocal choroiditis (MFC), and others. Thanks to multimodal imaging (MMI), it is now possible to differentiate between these two mechanisms of outer retinal damage. The aim of this study is to determine the MMI characteristics that allow us to differentiate primary photoreceptoritis, including AZOOR, from PICCPs such as MEWDS and MFC. Methods: A series of eight PICCPs cases (five typical MEWDS and three typical active MFC cases) and four typical primary photoreceptoritis/AZOOR cases (five eyes) that had undergone complete MMI investigation, including fundus photography (FP), blue light fundus autofluorescence (BL-FAF), spectral domain optical coherence tomography (SD-OCT), OCT angiography (OCT-A, when available), fluorescein angiography (FA), and indocyanine green angiography (ICGA) were analysed, pointing out the differences that allow us to distinguish primary photoreceptoritis from PICCPs. Results: All primary photoreceptoritis/AZOOR cases showed (1) faint fundus pallor around the fovea, (2) BL-FAF hyperautofluorescence, (3) loss of photoreceptor outer segments (PROS) on SD-OCT, (4) absence of choriocapillary drop-out on OCT-A, (5) normal FA or faint FA hyperfluorescence, and (6) conserved ICGA fluorescence/no hypofluorescent areas; (1), (2), (3), and (5) indicated loss of photoreceptor outer segments, and (4) and (6) indicated conserved choriocapillaris circulation. For PICCPs, (a) fundus showed discreet white dots or none (in MEWDS) and punched-out scars in MFC, (b) BL-FAF hyperautofluorescence, (c) loss of PROS on SD-OCT, (d) FA faint hyperfluorescence in MEWDS, also minimal in active MFC lesions (e) in all cases ICGA hypofluorescent areas; (b) and (c) indicating loss of PROS, and (e) indicating choriocapillaris non-perfusion in all cases. The OCT-A did not show consistent findings with faint or no capillary drop-out in MEWDS and MFC. Conclusions: MMI combining the SD-OCT and BL-FAF clearly showed loss of PROS in both groups, while the ICGA determined whether this was due to choriocapillaris non-perfusion in PICCPs or whether the choriocapillaris was intact in case of primary photoreceptoritis. The FA and OCT-A were found to be less useful and/or less sensitive for the appraisal of both these entities.https://www.mdpi.com/2075-4418/12/9/2179primary photoreceptoritisprimary inflammatory choriocapillaropathies (PICCPs)multiple evanescent white dot syndrome (MEWDS)idiopathic multifocal choroiditis (MFC)indocyanine green angiography (ICGA) |
| spellingShingle | Ioannis Papasavvas Alessandro Mantovani Carl P. Herbort Diagnosis, Mechanisms, and Differentiation of Inflammatory Diseases of the Outer Retina: Photoreceptoritis versus Choriocapillaritis; A Multimodal Imaging Perspective Diagnostics primary photoreceptoritis primary inflammatory choriocapillaropathies (PICCPs) multiple evanescent white dot syndrome (MEWDS) idiopathic multifocal choroiditis (MFC) indocyanine green angiography (ICGA) |
| title | Diagnosis, Mechanisms, and Differentiation of Inflammatory Diseases of the Outer Retina: Photoreceptoritis versus Choriocapillaritis; A Multimodal Imaging Perspective |
| title_full | Diagnosis, Mechanisms, and Differentiation of Inflammatory Diseases of the Outer Retina: Photoreceptoritis versus Choriocapillaritis; A Multimodal Imaging Perspective |
| title_fullStr | Diagnosis, Mechanisms, and Differentiation of Inflammatory Diseases of the Outer Retina: Photoreceptoritis versus Choriocapillaritis; A Multimodal Imaging Perspective |
| title_full_unstemmed | Diagnosis, Mechanisms, and Differentiation of Inflammatory Diseases of the Outer Retina: Photoreceptoritis versus Choriocapillaritis; A Multimodal Imaging Perspective |
| title_short | Diagnosis, Mechanisms, and Differentiation of Inflammatory Diseases of the Outer Retina: Photoreceptoritis versus Choriocapillaritis; A Multimodal Imaging Perspective |
| title_sort | diagnosis mechanisms and differentiation of inflammatory diseases of the outer retina photoreceptoritis versus choriocapillaritis a multimodal imaging perspective |
| topic | primary photoreceptoritis primary inflammatory choriocapillaropathies (PICCPs) multiple evanescent white dot syndrome (MEWDS) idiopathic multifocal choroiditis (MFC) indocyanine green angiography (ICGA) |
| url | https://www.mdpi.com/2075-4418/12/9/2179 |
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