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RETINAL Cases & Brief Reports:
doi: 10.1097/ICB.0000000000000026
Case Report

IDIOPATHIC MULTIFOCAL CHOROIDITIS WITH PERIPAPILLARY ZONAL INFLAMMATION: A MULTIMODAL IMAGING ANALYSIS

Jung, Jesse J. MD*,†,‡,§; Mrejen, Sarah MD†,‡; Freund, K. Bailey MD*,†,‡,§; Yannuzzi, Lawrence A. MD*,†,‡,§

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Author Information

*Department of Ophthalmology, New York University School of Medicine, New York, New York;

Vitreous Retina Macula Consultants of New York, New York, New York;

LuEsther T. Mertz Retinal Research Center, Manhattan Eye, Ear, and Throat Hospital, New York, New York; and

§Department of Ophthalmology, Edward S. Harkness Eye Institute, Columbia University College of Physicians and Surgeons, New York, New York.

Reprint requests: Lawrence A. Yannuzzi, MD, Vitreous Retina Macula Consultants of New York, Fifth Floor, 460 Park Avenue, New York, NY 10022; e-mail: layannuzzi@gmail.com

Supported by the LuEsther T. Mertz Retinal Research Center, Manhattan Eye, Ear, and Throat Institute, and The Macula Foundation, Inc.

None of the authors have any conflicting interests to disclose.

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Abstract

Purpose:

To report a case of idiopathic multifocal choroiditis with transient peripapillary zonal inflammation that was followed with multimodal imaging and explain the mechanism by which chorioretinal atrophy may occur.

Methods:

Observational case report. Review of the clinical examination, ocular imaging, and progression of idiopathic multifocal choroiditis.

Results:

A 30-year-old white myopic man presented with complaints of worsening vision and a loss of visual field for 1 month in his left eye. Using multimodal imaging, including wide-field imaging with fluorescein angiography, fundus autofluorescence, and high-definition spectral-domain optical coherence tomography, he was found to have a macula involving peripapillary zonal inflammation consistent with acute idiopathic multifocal choroiditis involving the outer photoreceptor layer, ellipsoid layer, retinal pigment epithelium, and choroid. This area of inflammation was monitored with multimodal imaging over 7 months and slowly improved along with the patient's vision. Imaging allowed us to view the development of chorioretinal scars from several, but not all, acute inflammatory white spots.

Conclusion:

Multifocal choroiditis is an inflammatory disorder affecting the outer photoreceptors, ellipsoid layer, retinal pigment epithelium, and choroid; and areas of acute inflammation may improve over time but can also leave permanent chorioretinal atrophy including focal lesions or peripapillary zonal atrophy.

Idiopathic multifocal choroiditis (MFC) is an inflammatory white dot disorder associated with punched-out chorioretinal lesions and/or curvilinear chorioretinal streaks,1,2 vitritis, occasionally secondary choroidal neovascularization,3 and enlarged blind spots.4,5 Over time, eyes with idiopathic MFC may rarely develop zonal, multizonal, or diffuse chorioretinal atrophy.6 In this report, we present a case of idiopathic MFC that developed transient peripapillary inflammation and enlargement of the blind spot. Using multimodal imaging, we demonstrate the mechanism by which acute inflammatory changes within the outer photoreceptor layer, ellipsoid layer, retinal pigment epithelium (RPE), and choroid may lead to chorioretinal atrophic lesions and zonal atrophy.

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Case Report

A healthy 30-year-old highly myopic white man presented with complaints of worsening vision and loss of a zone of visual field for 1 month in his left eye. Visual acuity was 20/20 in the right eye and 20/400 in the left eye. On presentation, anterior segment examination was unremarkable. Dilated fundus examination in the right eye was unremarkable except for minimal myopic peripapillary atrophy. Dilated fundus examination in the left eye, color photographs (Figure 1A), and fundus autofluorescence imaging (Figure 1B) demonstrated inflammatory white spots, punched-out chorioretinal atrophic spots, and a zonal area of peripapillary acute inflammation that co-localized precisely with an area of outer retinal disruption involving both the ellipsoid and digitation zones seen with spectral-domain optical coherence tomography (Figure 1C). Indocyanine green revealed multiple hypofluorescent spots correlating to the atrophic chorioretinal lesions in the early frames. The later indocyanine green frames demonstrated active choroiditis with granular changes within the superior macula and hypofluorescent spots correlating with the small hypopigmented white spots seen on color imaging. Fluorescein angiography on the wide-field imaging system accentuated the area of acute inflammation within the peripapillary area extending into the superior macula with multiple hyperfluorescent spots. The focal atrophic chorioretinal lesions also had a ring of surrounding hyperfluorescence and a central hypofluorescent scar. He was diagnosed with idiopathic MFC in the left eye and started on a brief course of systemic corticosteroids and acyclovir.

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On follow-up 7 months later, his visual symptoms had improved, and best-corrected visual acuity was 20/20 in both eyes. The beginning of his recovering coincided with the 1-month course of systemic corticosteroids. Dilated fundus examination in the left eye revealed scattered chorioretinal atrophic lesions with resolution of the peripapillary zone of inflammation and white spots, although there were a few new chorioretinal pigmentary scars. The resolution of this zone of inflammation (Figure 1, E and F) coincided with the restoration of the ellipsoid and digitation zones (Figure 1G). Choroidal thickness in this area decreased from 213 μm to 140 μm, consistent with a transient choroiditis.7,8 One atrophic chorioretinal spot developed within this area, producing a defect of the outer retinal layers and RPE band on spectral-domain optical coherence tomography (Figure 1, D and H).

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Discussion

Idiopathic MFC is believed to be an autoimmune-mediated disease occurring in genetically susceptible individuals.9 Multimodal imaging helps define the inflammatory foci that show structural damage at the level of the outer retina over areas of transient choroidal thickening.7,8 In our case, we observed a zone of peripapillary loss of transparency (Figure 1A) that was believed to be due to the acute accumulation of presumed inflammatory cell infiltrate within the subretinal space (Figure 1, C and D).7,8 On fundus autofluorescence imaging, the area was hyperautofluorescent relative to the background autofluorescence (Figure 1B) because of the disruption of the outer photoreceptor and ellipsoid layer (Figure 1, C and D). The increase in autofluorescence may be related to an outer retinal disruption with photopigment loss at the level of the outer segments of the photoreceptors and subsequent unmasking of the underlying autofluroescence emitted by the RPE10,11 and/or increased fluorophores within the RPE.12 After treatment and resolution, there was restoration of the outer retinal architecture and peripapillary zone of inflammation, but a more prominent area of subretinal inflammation developed into an atrophic scar. We hypothesize that recurrent episodes of inflammation could continue to produce additional atrophic changes around the nerve.6

Peripapillary involvement occurs in other related idiopathic inflammatory entities such as multiple evanescent white dot syndrome and acute zonal occult outer retinopathy, but varies regarding the degree of inflammation and reversibility of the structural changes.5 With multiple evanescent white dot syndrome–associated blind spot enlargement, the corresponding outer retinal changes observed with optical coherence tomography usually resolve completely.3–5 In MFC, the inflammation often leaves a legacy of chorioretinitic scars and peripapillary atrophy,4,5 but interestingly as in our case, the area of inflammation may disappear without any lasting damage. In acute zonal occult outer retinopathy, the inflammatory process primarily targets the outer retina in a zonal topography demarcated in some cases by a pathognomonic orange to gray line on ophthalmoscopic examination that appears as a coarse granular region of mixed hyperautofluorescent and hypoautofluorescent on fundus autofluorescence imaging.10 These zonal areas never resolve and usually progress as visualized on multimodal imaging.13

This case of idiopathic MFC demonstrates the mechanism by which inflammation may lead to chorioretinal atrophic lesions or, in more severe cases not represented by this report, zonal atrophy.6 Areas of acute inflammation involving the photoreceptors, RPE, and inner choroid may resolve completely or lead to permanent chorioretinitic scarring. Although much of the underlying pathophysiology of idiopathic MFC still remains a mystery, it seems clear that the range of clinical findings relates in part to the degree and duration of inflammation and the resilience of the retina and RPE to inflammatory damage.

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References

1. Dreyer RF, Gass JDM. Multifocal choroiditis and panuveitis. A syndrome that mimics ocular histoplasmosis. Arch Ophthalmol. 1984; 102:1776–1784.

2. Borodoker N, Cunningham ET Jr, Yannuzzi LA, et al. Peripheral curvilinear pigmentary streak in multifocal choroiditis. Arch Ophthalmol. 2002; 120:520–521.

3. Callanan D, Gass JDM. Multifocal choroiditis and choroidal neovascularization associated with the multiple evanescent white dot and acute idiopathic blind spot enlargement syndrome. Ophthalmology. 1992; 99:1678–1685.

4. Reddy CV, Brown J Jr, Folk JC, et al. Enlarged blind spots in chorioretinal inflammatory disorders. Ophthalmology. 1996; 103:606–617.

5. Spaide RF, Koizumi H, Freund KB. Photoreceptor outer segment abnormalities as a cause of blind spot enlargement in acute zonal occult outer retinopathy-complex diseases. Am J Ophthalmol. 2008; 146:111–120.

6. Jung JJ, Khan S, Mrejen S, et al. Idiopathic multifocal choroiditis with outer retinal or chorioretinal atrophy. Retina. 2013;

In press


7. Spaide RF, Goldberg N, Freund KB. Redefining multifocal choroiditis and panuveitis and punctate inner choroidopathy through multimodal imaging. Retina. 2013; 33:1315–1324.

8. Vance SK, Khan S, Klancnik JM, Freund KB. Characteristic spectral-domain optical coherence tomography findings of multifocal choroiditis. Retina. 2011; 31:717–723.

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10. Burns SA, Elsner AE, Lobes LA Jr. Foveal cone photopigment bleaching in central serous retinopathy. Appl Opt. 1988; 27:1045–1049.

11. Freund KB, Mrejen S, Jung JJ, et al. Increased fundus autofluorescence related to outer retinal disruption. JAMA Ophthalmol.

In press


12. Schmitz-Valckenberg S, Holz FG, Bird AC, Spaide RF. Fundus autofluorescence imaging review and perspectives. Retina. 2008; 28:385–409.

13. Mrejen S, Khan S, Gallego-Pinazzo R, Jampol LM, Yannuzzi LA, et al. Schepens lecture: AZOOR. JAMA Ophthalmol. 2013;

In press


Keywords:

chorioretinitic scarring; idiopathic multifocal choroiditis; multimodal imaging; peripapillary zonal inflammation; white dot syndromes

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