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      Bilateral foveal retinoschisis accompanying unilateral peripheral retinoschisis

      case-report

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          Abstract

          X-linked juvenile retinoschisis is a rare hereditary retinal disease characterized by a tangential splitting of the neurosensory retina which may cause early-onset visual impairment. Existence of the retinal neurosensory layer splitting on cross-sectional images of optical coherance tomography (OCT) and the absence of leakage on fluorescein angiography (FA) help confirming the diagnosis. Such diagnostic tests are also helpful in determining the management of the disease. However, most of the retinoschisis cavities remain stable and rarely extend to the posterior pole, many authors suggest laser prophylaxis to avoid the potential risk of retinal detachment due to holes in the outer retinal layer. Herein, we report a case with bilateral foveal retinoschisis accompanying unilateral peripheral retinoschisis who was evaluated with detailed ophthalmologic examination. Visual acuity, fundoscopy, OCT, and FA remained stable in the second year of follow-up after prophylactic argon laser treatment.

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          Most cited references11

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          X linked retinoschisis.

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            X-linked retinoschisis: an update.

            X-linked retinoschisis is the leading cause of macular degeneration in males and leads to splitting within the inner retinal layers leading to visual deterioration. Many missense and protein truncating mutations have now been identified in the causative retinoschisis gene (RS1) which encodes a 224 amino acid secretory retinal protein, retinoschisin. Retinoschisin octamerisation is implicated in cell-cell interactions and cell adhesion perhaps by interacting with beta2 laminin. Mutations cause loss of retinoschisin function by one of the three mechanisms: by interfering with protein secretion, by preventing its octamerisation or by reducing function in the secreted octamerised protein. The development of retinoschisis mouse models have provided a model system that closely resembles the human disease. Recent reports of RS1 gene transfer to these models and the sustained restoration of some retinal function and morphology suggest gene replacement may be a possible future therapy for patients.
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              Foveomacular schisis in juvenile X-linked retinoschisis: an optical coherence tomography study.

              To explore the structural features of juvenile X-linked retinoschisis using spectral-domain optical coherence tomography (OCT). Retrospective, observational cross-sectional study. Eighteen male patients (34 eyes) who were diagnosed with juvenile X-linked retinoschisis at the Eye & ENT Hospital of Fudan University over an 18-month period were included. Their OCT images, which were obtained using spectral-domain OCT (Cirrus HD-OCT; Carl Zeiss Meditec), were analyzed. The anatomic location of the schisis cavity in juvenile X-linked retinoschisis was characterized by direct inspection of OCT images. On OCT, the schisis cavity was visible at the fovea in all 34 eyes, and it was associated with increased retinal thickness. Schisis was present at the retinal nerve fiber layer in 4 eyes, at the inner nuclear layer in 29 eyes, and at the outer nuclear layer/outer plexiform layer in 22 eyes. In most cases, widespread foveomacular schisis was detected using OCT; however, in 9 eyes (6 patients), the schisis was confined to the fovea. Schisis of the inner nuclear layer and outer nuclear layer/outer plexiform layer almost always involved the foveal center, but retinal nerve fiber layer schisis was seen only in the parafoveal area. Despite conventional wisdom, in patients with X-linked retinoschisis, the schisis cavity can occur in a number of different layers of the neurosensory retina (retinal nerve fiber layer, inner nuclear layer, and outer nuclear layer/outer plexiform layer). In addition, different forms of schisis may affect different locations in the macula (foveal vs parafoveal), and, in most eyes, the schisis involves the entire foveomacular region. Copyright 2010 Elsevier Inc. All rights reserved.
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                Author and article information

                Journal
                Indian J Ophthalmol
                Indian J Ophthalmol
                IJO
                Indian Journal of Ophthalmology
                Medknow Publications & Media Pvt Ltd (India )
                0301-4738
                1998-3689
                April 2014
                : 62
                : 4
                : 513-515
                Affiliations
                [1]Department of Ophthalmology, Dokuz Eylul University School of Medicine, Izmir, Turkey
                [1 ]Dr. Behçet Uz Children's Hospital, Izmir, Turkey
                Author notes
                Correspondence to: Prof. Nilufer Kocak, Department of Ophthalmology, Dokuz Eylul University School of Medicine, Inciralti, Balçova 35340, Izmir, Turkey. E-mail: nkocak@ 123456yahoo.com
                Article
                IJO-62-513
                10.4103/0301-4738.100005
                4064240
                23571248
                5aa416c4-0a69-4178-b2a4-a83adec7932c
                Copyright: © Indian Journal of Ophthalmology

                This is an open-access article distributed under the terms of the Creative Commons Attribution-Noncommercial-Share Alike 3.0 Unported, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

                History
                : 06 April 2011
                : 06 December 2011
                Categories
                Brief Communications

                Ophthalmology & Optometry
                fluorescein angiography,optical coherance tomography,prophylactic argon laser treatment,retinoschisis

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