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      Hemoglobin S/O Arab: Retinal Manifestations of a Rare Hemoglobinopathy

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          Hemoglobin S/O<sub>Arab</sub> (Hgb S/O<sub>Arab</sub>) disease is a rare hemoglobinopathy which presents similarly to sickle cell retinopathy, with only three prior reports that describe associated retinal findings. In this report, we present ophthalmic examination findings in 2 patients with Hgb S/O<sub>Arab</sub>. One patient exhibited peripheral ischemia and sunburst lesions without neovascular disease, and the other patient developed proliferative retinopathy of both eyes and multiple posterior-pole branch retinal artery occlusions in one eye. To our knowledge, this is the first case of retinal arterial occlusive disease in Hgb S/O<sub>Arab</sub>, and the first report of fundus autofluorescence and OCT angiography in Hgb/O<sub>Arab</sub> retinopathy.

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          Most cited references 13

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          Natural history of untreated proliferative sickle retinopathy.

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            The ocular manifestations of sickle-cell disease: a prevalence and natural history study.

            Prophylactic photocoagulation may have a role in the treatment of proliferative sickle retinopathy in selected patients with SC disease, but none of the studies reported to date have established that such treatment of these eyes improves the long-term visual outcome as compared with the natural history as documented in this study. The similar visual outcomes in the eyes reviewed in this natural history study as compared with those that have been treated with photocoagulation should not be unexpected, because there is greater predilection for spontaneous involution or autoinfarction of the neovascular tissue in SC disease as opposed to the neovascularization that develops in other retinal vascular disease. A controlled, multicenter clinical trial designed to study those eyes at greatest risk should be considered. Such a trial should specify patient age (15 to 30 years), hemoglobin type (SC disease), and a minimum threshold of active proliferative disease (60 degrees or greater). Such criteria have been suggested by others. By comparing the outcome of eligible eyes randomly assigned to treatment or observation and followed for an extended period, it will be possible to learn whether laser photocoagulation offers a better prognosis than the natural history of proliferative sickle retinopathy. There is no question that in some eyes with proliferative sickle retinopathy, nonclearing vitreous hemorrhage and/or retinal detachment will develop. Clear definition of the risk factors leading to these advanced stages, however, is lacking, and the value of treatment is uncertain.
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              Sudden-onset blindness in sickle cell disease due to retinal artery occlusion.

              Central retinal artery occlusion (CRAO) is a rare and potentially devastating cause of acute blindness in sickle cell disease (SCD) that is unique compared to classic sickle retinopathy. Few details related to this complication in SCD are known, including its risk factors, pathogenesis, presentation, treatment and outcomes. We present three patients with SCD and retinal artery occlusion. The overall variability in clinical presentation, treatment and prognosis reported in the literature underscores the need for a greater understanding of these factors as they relate to this complication in SCD. (c) 2007 Wiley-Liss, Inc.

                Author and article information

                Case Reports in Ophthalmology
                S. Karger AG
                May – August 2020
                27 May 2020
                : 11
                : 2
                : 189-195
                aHarvey and Bernice Jones Eye Institute, University of Arkansas for Medical Sciences, Little Rock, Arkansas, USA
                bCollege of Medicine, University of Arkansas for Medical Sciences, Little Rock, Arkansas, USA
                cSouthwest Eye Care, Albuquerque, New Mexico, USA
                Author notes
                *Sami Uwaydat, Harvey and Bernice Jones Eye Institute, University of Arkansas for Medical Sciences, 4301 W Markham St., Slot 523, Little Rock, AR 72205 (USA), SHUwaydat@uams.edu
                507879 PMC7315179 Case Rep Ophthalmol 2020;11:189–195
                © 2020 The Author(s). Published by S. Karger AG, Basel

                This article is licensed under the Creative Commons Attribution-NonCommercial 4.0 International License (CC BY-NC). Usage and distribution for commercial purposes requires written permission. 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.

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                Figures: 4, Pages: 7
                Case Report


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