An Optical Coherence Tomography and Fundus Autofluorescence Imaging Study of Peripapillary Acute Zonal Occult Outer Retinopathy

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Abstract

Purpose

To evaluate peripapillary acute zonal occult outer retinopathy in a 67-year-old man.

Methods

Images were obtained using fundus photography, fluorescein angiography (FA) and indocyanine green angiography (ICGA), fundus autofluorescence (FAF) imaging, and optical coherence tomography (OCT). Visual field testing and multifocal electroretinogram (mfERG) were also performed to evaluate retinal function.

Results

Fundus photographs showed subtle pigmentary changes in the peripapillary region, while FAF imaging showed clearly defined hypofluorescent areas in the peripapillary region. Intense hyperfluorescent lesions were also seen underneath hypofluorescent areas. A transmission defect with a granular hyperfluorescence was visible on FA, and ICGA showed hypofluorescence within the lesion. The outer border of the peripapillary zone appeared to block the underlying choroidal fluorescence. The photoreceptor inner segment/outer segment line was absent on OCT images from this area in both eyes, and hyperreflective punctate drusen-like materials were present at the outer border of the peripapillary zone. Visual field testing by Goldmann perimetry showed blind-spot enlargement, and mfERG showed corresponding amplitude reductions.

Conclusions

We speculate that the intense hyperautofluorescent material at the outer border of the peripapillary zone might be accumulated drusenoid lipofuscin.

Related collections

Most cited references 10

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Photoreceptor outer segment abnormalities as a cause of blind spot enlargement in acute zonal occult outer retinopathy-complex diseases.

Richard Spaide, Hideki Koizumi, K. Bailey Freund (2008)

To investigate the correlation between visual field (VF) defects in diseases of the acute zonal occult outer retinopathy (AZOOR)-complex and their spectral-domain optical coherence tomographic (OCT) findings. Observational case series. Patients with AZOOR, multiple evanescent white dot syndrome (MEWDS), and multifocal choroiditis and panuveitis (MCP) examined in a private practice retinal referral center had threshold VF testing and spectral-domain OCT examination performed using a device capable of obtaining a block of 128 B-scans in a 6 x 6-mm region centered on the optic nerve and macula. The areas of defects in the boundary between the inner segments (IS) and the outer segments (OS) of the photoreceptors, termed the IS/OS boundary, were compared with the VF defects measured. There were 18 evaluable eyes among one patient with MEWDS, two with AZOOR, and seven with MCP. In the 14 eyes with blind spot enlargement [corrected] corresponding IS/OS boundary defects were found in the [corrected] peripapillary region, while no IS/OS boundary defects were found in the four [corrected] eyes without blind spot enlargement. IS/OS boundary defects were seen over chorioretinal scars and areas of neovascularization and no widespread defects were seen [corrected] elsewhere in the fundus. The IS/OS boundary defects showed improvement, as did the blind spot enlargement, spontaneously in the patient with MEWDS and after treatment with immunosuppression in the patients with AZOOR. The spectral-domain OCT finding of IS/OS boundary defects, implicating photoreceptor OS perturbation, appears to explain the blind spot enlargement in patients with AZOOR-complex diseases. These defects are not necessarily permanent.

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Acute zonal occult outer retinopathy.

Dinelli M Monson, Justine Smith (2015)

Acute zonal occult outer retinopathy (AZOOR) is a rare unilateral or bilateral disease of unknown etiology characterized by focal degeneration of photoreceptors. A total of 131 cases of AZOOR (205 eyes), including the variant known as acute annular outer retinopathy, have been reported in the English language literature. In this group of predominantly white individuals, average age at presentation was 36.7 years, and the male:female ratio was 1:3.2. The majority of patients complained of the acute onset of a scotoma, which was associated with photopsia. Visual acuity was 20/40 or better in 74% of tested eyes, and fundus examination was unremarkable in 76% of eyes. Blind spot enlargement, with or without other field defects, was observed in 75% of the visual fields examined, and electroretinographic abnormalities were recorded in 99% of patients tested. Typically patients retained good visual acuity, although retinal pigment epithelial disturbances commonly developed over time. It was unusual for visual field loss to continue beyond six months. Various treatments have been attempted in patients with AZOOR--including systemic corticosteroids, other systemic immunosuppressive agents, and different antimicrobials--but none have been proven effective. Copyright © 2011 Elsevier Inc. All rights reserved.

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Acute zonal occult outer retinopathy: a long-term follow-up study1 1InternetAdvance publication at ajo.com June 26, 2002.

J.Donald Gass, Anita Agarwal, Ingrid Scott (2002)

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Author and article information

Journal

Journal ID (nlm-ta): Case Rep Ophthalmol

Journal ID (iso-abbrev): Case Rep Ophthalmol

Journal ID (publisher-id): COP

Title: Case Reports in Ophthalmology

Publisher: S. Karger AG (Allschwilerstrasse 10, P.O. Box · Postfach · Case postale, CH–4009, Basel, Switzerland · Schweiz · Suisse, Phone: +41 61 306 11 11, Fax: +41 61 306 12 34, karger@karger.ch )

ISSN (Electronic): 1663-2699

Publication date Collection: Jan-Apr 2013

Publication date (Electronic): 26 January 2013

Publication date PMC-release: 26 January 2013

Volume: 4

Issue: 1

Pages: 11-16

Affiliations

Department of Ophthalmology, Jichi Medical University, Tochigi, Japan

Author notes

*Shinji Makino, 3311-1 Yakushiji, Shimotsuke, Tochigi 329-0498 (Japan), E-Mail makichan@ 123456jichi.ac.jp

Article

Publisher ID: cop-0004-0011

DOI: 10.1159/000346731

PMC ID: 3573782

PubMed ID: 23467270

SO-VID: e12d680c-c609-4f9a-b9b3-390e11eb23ea

License:

This is an Open Access article distributed under the terms of the Creative Commons Attribution-Noncommercial-No-Derivative-Works License ( http://creativecommons.org/licenses/by-nc-nd/3.0/). Users may download, print and share this work on the Internet for noncommercial purposes only, provided the original work is properly cited, and a link to the original work on http://www.karger.com and the terms of this license are included in any shared versions.

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Figures: 2, References: 10, Pages: 6

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