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      Pathomechanisms in central serous chorioretinopathy: A recent update

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          Abstract

          Background

          Central serous chorioretinopathy (CSCR) is a potentially blinding choroidal disease. Despite decades of research, the pathological mechanisms of CSCR are still poorly understood. In recent years, there has been a strong emphasis on choroidal dysfunction as a primary cause of CSCR.

          Main Body

          The concept of the pachychoroid disease spectrum and pachychoroid-driven processes are central to current theories regarding the pathophysiological underpinnings of CSCR. Choroidal hyperpermeability and subsequent leakage of fluid seen in CSCR may be due to several causes. Among them are venous congestion, inflammation, mineralocorticoid receptor activation, systemic factors including hemodynamic changes, obstructive sleep apnea, phosphodiesterase inhibitor use, pregnancy, and genetic predispositions. Congestion of vortex veins that drain blood from the choroid may contribute to the dilation of Haller vessels and cause fluid leakage. Vortex veins exit the eye through the sclera; thus, increased scleral thickness has been proposed to be a factor in venous congestion. Asymmetric vortex vein drainage may similarly result in congestion of the local venous system. Vortex vein anastomoses may overload the venous system and form secondary to venous congestion. Recent studies suggest inflammation and mineralocorticoid activation may factor into the development of CSCR, though more research in these areas is called for. Systemic conditions and genetics may predispose individuals to develop CSCR.

          Conclusions

          By striving to understand the molecular and physiological mechanisms of this disease, we can better diagnose and treat CSCR to improve outcomes for patients.

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          Most cited references55

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          Central serous chorioretinopathy: Recent findings and new physiopathology hypothesis.

          Central serous chorioretinopathy (CSCR) is a major cause of vision threat among middle-aged male individuals. Multimodal imaging led to the description of a wide range of CSCR manifestations, and highlighted the contribution of the choroid and pigment epithelium in CSCR pathogenesis. However, the exact molecular mechanisms of CSCR have remained uncertain. The aim of this review is to recapitulate the clinical understanding of CSCR, with an emphasis on the most recent findings on epidemiology, risk factors, clinical and imaging diagnosis, and treatments options. It also gives an overview of the novel mineralocorticoid pathway hypothesis, from animal data to clinical evidences of the biological efficacy of oral mineralocorticoid antagonists in acute and chronic CSCR patients. In rodents, activation of the mineralocorticoid pathway in ocular cells either by intravitreous injection of its specific ligand, aldosterone, or by over-expression of the receptor specifically in the vascular endothelium, induced ocular phenotypes carrying many features of acute CSCR. Molecular mechanisms include expression of the calcium-dependent potassium channel (KCa2.3) in the endothelium of choroidal vessels, inducing subsequent vasodilation. Inappropriate or over-activation of the mineralocorticoid receptor in ocular cells and other tissues (such as brain, vessels) could link CSCR with the known co-morbidities observed in CSCR patients, including hypertension, coronary disease and psychological stress.
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            Pachychoroid disease

            Pachychoroid is a relatively novel concept describing a phenotype characterized by attenuation of the choriocapillaris overlying dilated choroidal veins, and associated with progressive retinal pigment epithelium dysfunction and neovascularization. The emphasis in defining pachychoroid-related disorders has shifted away from simply an abnormally thick choroid (pachychoroid) toward a detailed morphological definition of a pathologic state (pachychoroid disease) with functional implications, which will be discussed in this review. Several clinical manifestations have been described to reside within the pachychoroid disease spectrum, including central serous chorioretinopathy, pachychoroid pigment epitheliopathy, pachychoroid neovasculopathy, polypoidal choroidal vasculopathy/aneurysmal type 1 neovascularization, focal choroidal excavation, peripapillary pachychoroid syndrome. These conditions all exhibit the characteristic choroidal alterations and are believed to represent different manifestations of a common pathogenic process. This review is based on both the current literature and the clinical experience of our individual authors, with an emphasis on the clinical and imaging features, management considerations, as well as current understanding of pathogenesis of these disorders within the context of the recent findings related to pachychoroid disease.
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              EN FACE IMAGING OF PACHYCHOROID SPECTRUM DISORDERS WITH SWEPT-SOURCE OPTICAL COHERENCE TOMOGRAPHY.

              To correlate clinical manifestations with choroidal morphology in pachychoroid disorders, including central serous chorioretinopathy, pachychoroid pigment epitheliopathy, pachychoroid neovasculopathy, and polypoidal choroidal vasculopathy, using en face swept-source optical coherence tomography (OCT).
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                Author and article information

                Contributors
                jay.chhablani@gmail.com
                Journal
                Int J Retina Vitreous
                Int J Retina Vitreous
                International Journal of Retina and Vitreous
                BioMed Central (London )
                2056-9920
                20 January 2023
                20 January 2023
                2023
                : 9
                : 3
                Affiliations
                [1 ]GRID grid.21925.3d, ISNI 0000 0004 1936 9000, Department of Ophthalmology, , University of Pittsburgh School of Medicine, ; Pittsburgh, PA USA
                [2 ]GRID grid.460812.9, Sarojini Devi Eye Hospital, ; Hyderabad, India
                [3 ]Bahamas Vision Centre and Princess Margaret Hospital, Nassau, NP Bahamas
                Author information
                http://orcid.org/0000-0003-1772-3558
                Article
                443
                10.1186/s40942-023-00443-2
                9854068
                36670451
                9103778c-6137-4372-8c5c-a84ba22e5477
                © The Author(s) 2023

                Open AccessThis article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article's Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article's Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/. The Creative Commons Public Domain Dedication waiver ( http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated in a credit line to the data.

                History
                : 22 December 2022
                : 10 January 2023
                Categories
                Review
                Custom metadata
                © The Author(s) 2023

                choroid,central serous chorioretinopathy,pachychoroid disease,retina

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