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      Changes in Optical Coherence Tomography Findings in Acute Zonal Occult Outer Retinopathy

      case-report

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          Abstract

          Purpose: To report a case of acute zonal occult outer retinopathy (AZOOR) in a 39-year-old woman. Methods: Images were obtained with fundus photography and optical coherence tomography (OCT). A multifocal electroretinogram (mfERG) was also obtained to evaluate retinal function. Results: The patient's right visual acuity was 0.8. Fundus photographs showed no specific abnormal findings. OCT showed attenuation of the photoreceptor inner segment/outer segment (IS/OS) line and the cone outer segment tip (COST) line in the right eye. The mfERG showed corresponding amplitude reductions. One month after the initial visit, her right visual acuity improved to 1.2. There was partial recovery of the IS/OS line; however, the COST line was still absent, and there was no apparent improvement in the mfERG responses. Conclusions: Our findings showed a discrepancy between the microstructural findings and visual function during recovery of AZOOR.

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          Most cited references28

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          Characterization of outer retinal morphology with high-speed, ultrahigh-resolution optical coherence tomography.

          To visualize, quantitatively assess, and interpret outer retinal morphology by using high-speed, ultrahigh-resolution (UHR) OCT. Retinal imaging was performed in the ophthalmic clinic in a cross-section of 43 normal subjects with a 3.5-microm, axial-resolution, high-speed, UHR OCT prototype instrument, using a radial scan pattern (24 images, 1500 axial scans). Outer retinal layers were automatically segmented and measured. High-definition imaging was performed with a 2.8-microm axial-resolution, high-speed, UHR OCT research prototype instrument, to visualize the finer features in the outer retina. Quantitative maps of outer retinal layers showed clear differences between the cone-dominated fovea and the rod-dominated parafovea and perifovea, indicating that photoreceptor morphology can explain the appearance of the outer retina in high-speed, UHR OCT images. Finer, scattering bands were visualized in the outer retina using high-definition imaging and were interpreted by comparison to known anatomy. High-speed UHR OCT enables quantification of scattering layers in the outer retina. An interpretation of these features is presented and supported by quantitative measurements in normal subjects and comparison with known anatomy. The thick scattering region of the outer retina previously attributed to the retinal pigment epithelium (RPE) is shown to consist of distinct scattering bands corresponding to the photoreceptor outer segment tips, RPE, and Bruch's membrane. These results may advance understanding of the outer retinal appearance in OCT images. The normative measurements may also aid in future investigations of outer retinal changes in age-related macular degeneration and other diseases.
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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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                Author and article information

                Journal
                COP
                COP
                10.1159/issn.1663-2699
                Case Reports in Ophthalmology
                S. Karger AG
                1663-2699
                2013
                September – December 2013
                04 September 2013
                : 4
                : 3
                : 99-104
                Affiliations
                Department of Ophthalmology, Jichi Medical University, Shimotsuke, Japan
                Author notes
                *Shinji Makino, Department of Ophthalmology, Jichi Medical University, 3311-1 Yakushiji, Shimotsuke, Tochigi 329-0498 (Japan), E-Mail makichan@jichi.ac.jp
                Article
                355108 PMC3806683 Case Rep Ophthalmol 2013;4:99-104
                10.1159/000355108
                PMC3806683
                24163675
                4968d2b1-5e3c-4b98-981b-c32afd95be69
                © 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.

                History
                Page count
                Figures: 2, Pages: 6
                Categories
                Published: September 2013

                Vision sciences,Ophthalmology & Optometry,Pathology
                Cone outer segment tip,Photoreceptor inner segment/outer segment junction,Multifocal electroretinogram,Acute zonal occult outer retinopathy,Optical coherence tomography

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