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

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          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.

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

          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.

            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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              Fundus autofluorescence and optical coherence tomographic findings in acute zonal occult outer retinopathy.

              The purpose of this study was to investigate the fundus autofluorescence and optical coherence tomography findings in eyes with acute zonal occult outer retinopathy (AZOOR). A retrospective observational case series of the fundus autofluorescence and spectral domain optical coherence tomography in a series of patients with AZOOR. There were 19 eyes of 11 patients (10 women), who had a mean age of 49.1 +/- 13.9 years. Fundus autofluorescence abnormalities were seen in 17 of the 19 eyes, were more common in the peripapillary area, and were smaller in extent than the optical coherence tomography abnormalities. Nine eyes showed progression of hypoautofluorescence area during the mean follow-up of 69.7 months. The mean thickness of the photoreceptor layer at fovea was 177 microm in eyes with AZOOR, which was significantly thinner than controls (193 microm, P = 0.049). Abnormal retinal laminations were found in 12 eyes and were located over areas of loss of the photoreceptors. The subfoveal choroidal thickness was 243 microm, which is normal. Fundus autofluorescence abnormalities in AZOOR showed distinct patterns of retinal pigment epithelial involvement, which may be progressive. Thinning of photoreceptor cell layer with loss of the outer segments and abnormal inner retinal lamination in the context of a normal choroid are commonly found in AZOOR.

                Author and article information

                Case Reports in Ophthalmology
                S. Karger AG
                January – April 2013
                26 January 2013
                : 4
                : 1
                : 11-16
                Department of Ophthalmology, Jichi Medical University, Tochigi, Japan
                Author notes
                *Shinji Makino, 3311-1 Yakushiji, Shimotsuke, Tochigi 329-0498 (Japan), E-Mail makichan@jichi.ac.jp
                346731 PMC3573782 Case Rep Ophthalmol 2013;4:11–16
                © 2013 S. Karger AG, Basel

                Open Access License: This is an Open Access article licensed under the terms of the Creative Commons Attribution-NonCommercial 3.0 Unported license (CC BY-NC) ( http://www.karger.com/OA-license), applicable to the online version of the article only. Distribution permitted for non-commercial purposes only. Drug Dosage: The authors and the publisher have exerted every effort to ensure that drug selection and dosage set forth in this text are in accord with current recommendations and practice at the time of publication. However, in view of ongoing research, changes in government regulations, and the constant flow of information relating to drug therapy and drug reactions, the reader is urged to check the package insert for each drug for any changes in indications and dosage and for added warnings and precautions. This is particularly important when the recommended agent is a new and/or infrequently employed drug. Disclaimer: The statements, opinions and data contained in this publication are solely those of the individual authors and contributors and not of the publishers and the editor(s). The appearance of advertisements or/and product references in the publication is not a warranty, endorsement, or approval of the products or services advertised or of their effectiveness, quality or safety. The publisher and the editor(s) disclaim responsibility for any injury to persons or property resulting from any ideas, methods, instructions or products referred to in the content or advertisements.

                Page count
                Figures: 2, Pages: 6
                Published: January 2013


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