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Fovea-threatening and fovea-involving peripheral Coats disease: effects of posture and intervention

BACKGROUND: We believe that our experience with patients presenting with Coats disease and macular sparing should be shared with our colleagues. We would like to show the effect of posture and prompt intervention in cases with fovea-threatening and/or fovea-involving peripheral Coats disease (FTPCD)...

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Autores principales: de Souza, Eduardo Cunha, Rosa, Evandro, de Oliveira Dias, João Rafael, Malerbi, Fernando Korn, Leal, Bruno Campelo, Junior, Helio Paulo Primiano
Formato: Online Artículo Texto
Lenguaje:English
Publicado: BioMed Central 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9205031/
https://www.ncbi.nlm.nih.gov/pubmed/35715849
http://dx.doi.org/10.1186/s40942-022-00382-4
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author de Souza, Eduardo Cunha
Rosa, Evandro
de Oliveira Dias, João Rafael
Malerbi, Fernando Korn
Leal, Bruno Campelo
Junior, Helio Paulo Primiano
author_facet de Souza, Eduardo Cunha
Rosa, Evandro
de Oliveira Dias, João Rafael
Malerbi, Fernando Korn
Leal, Bruno Campelo
Junior, Helio Paulo Primiano
author_sort de Souza, Eduardo Cunha
collection PubMed
description BACKGROUND: We believe that our experience with patients presenting with Coats disease and macular sparing should be shared with our colleagues. We would like to show the effect of posture and prompt intervention in cases with fovea-threatening and/or fovea-involving peripheral Coats disease (FTPCD). This association has been poorly debated in our specialty and literature. We call the attention for the unexpexted scenario of observing the lost of the fovea during some types of traditional and prompt interventional treatments of these cases with previous 20/20 vision (something that we have been studying and observing for many years). In order to publish our best representative cases, we have chosen 8 Brazilian patients (age range, 7–62 years; 5 male) with FTPCD. All patients underwent multimodal imaging and different treatments (observation, sleep-posture repositioning, laser, intraocular steroids, and/or anti-vascular endothelial growth factor therapy). All patients, initially, informed to adopt a sleeping lateral-down position, favoring exudation shifting to the fovea pre-treatment. Most promptly-treated patients in this way (n = 4), developed subretinal fluid and exudates in the macula and some had irreversible central visual loss (n = 3). Patients with recent fovea-involving exudation who changed postural sleep position (to protect the foveal area) before and during treatment fared better, with some preserved central vision and an intact fovea (n = 5). The fundus status was correlated with the gravitational effects of posture before and after treatment. Despite prepared as an observational/interventional study, with a small number of cases, the most difficult part is documenting the sleep position of these patients and its influence in the outcomes as there is not good way to prove how well or poorly the positioning occurred in our cases. Finally, we also intended to call the attention to the fact that Coats disease must be studied in all its clinical stage variants and not only seen as a potential blinding and incurable ocular disease. CASE PRESENTATION: This study is a retrospective and/or interventional analysis of eight cases with a less severe clinical variant of classic Coats disease that we refer to as fovea-threatening and fovea-involving peripheral Coats disease (FTPCD). All cases were unilateral with no systemic disease or family history of Coats disease. The bilateral anterior segment and intraocular pressure were normal in all patients. The characteristics of all patients are shown in the Table. CONCLUSION: The funduscopic features of FTPCD are fundamental to disease understanding and optimal management. Habitual posturing may affect the fundus morphologic features of retinal exudation as observed in all current patients with exudative peripheral Coats disease. When sleep habitual posture is not observed in patients with FTPCD, the effects of prompt invasive treatments can cause rapid visual loss because of foveal subretinal pooling of exudates post-treatment. Initial vigilant adjusting of the habitual sleep posture for several patients with FTPCD, before the indication of traditional invasive treatments (laser and/or pharmacologic medications) can result in improved vision and fundoscopic morphologic features. SUPPLEMENTARY INFORMATION: The online version contains supplementary material available at 10.1186/s40942-022-00382-4.
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spelling pubmed-92050312022-06-18 Fovea-threatening and fovea-involving peripheral Coats disease: effects of posture and intervention de Souza, Eduardo Cunha Rosa, Evandro de Oliveira Dias, João Rafael Malerbi, Fernando Korn Leal, Bruno Campelo Junior, Helio Paulo Primiano Int J Retina Vitreous Case Report BACKGROUND: We believe that our experience with patients presenting with Coats disease and macular sparing should be shared with our colleagues. We would like to show the effect of posture and prompt intervention in cases with fovea-threatening and/or fovea-involving peripheral Coats disease (FTPCD). This association has been poorly debated in our specialty and literature. We call the attention for the unexpexted scenario of observing the lost of the fovea during some types of traditional and prompt interventional treatments of these cases with previous 20/20 vision (something that we have been studying and observing for many years). In order to publish our best representative cases, we have chosen 8 Brazilian patients (age range, 7–62 years; 5 male) with FTPCD. All patients underwent multimodal imaging and different treatments (observation, sleep-posture repositioning, laser, intraocular steroids, and/or anti-vascular endothelial growth factor therapy). All patients, initially, informed to adopt a sleeping lateral-down position, favoring exudation shifting to the fovea pre-treatment. Most promptly-treated patients in this way (n = 4), developed subretinal fluid and exudates in the macula and some had irreversible central visual loss (n = 3). Patients with recent fovea-involving exudation who changed postural sleep position (to protect the foveal area) before and during treatment fared better, with some preserved central vision and an intact fovea (n = 5). The fundus status was correlated with the gravitational effects of posture before and after treatment. Despite prepared as an observational/interventional study, with a small number of cases, the most difficult part is documenting the sleep position of these patients and its influence in the outcomes as there is not good way to prove how well or poorly the positioning occurred in our cases. Finally, we also intended to call the attention to the fact that Coats disease must be studied in all its clinical stage variants and not only seen as a potential blinding and incurable ocular disease. CASE PRESENTATION: This study is a retrospective and/or interventional analysis of eight cases with a less severe clinical variant of classic Coats disease that we refer to as fovea-threatening and fovea-involving peripheral Coats disease (FTPCD). All cases were unilateral with no systemic disease or family history of Coats disease. The bilateral anterior segment and intraocular pressure were normal in all patients. The characteristics of all patients are shown in the Table. CONCLUSION: The funduscopic features of FTPCD are fundamental to disease understanding and optimal management. Habitual posturing may affect the fundus morphologic features of retinal exudation as observed in all current patients with exudative peripheral Coats disease. When sleep habitual posture is not observed in patients with FTPCD, the effects of prompt invasive treatments can cause rapid visual loss because of foveal subretinal pooling of exudates post-treatment. Initial vigilant adjusting of the habitual sleep posture for several patients with FTPCD, before the indication of traditional invasive treatments (laser and/or pharmacologic medications) can result in improved vision and fundoscopic morphologic features. SUPPLEMENTARY INFORMATION: The online version contains supplementary material available at 10.1186/s40942-022-00382-4. BioMed Central 2022-06-17 /pmc/articles/PMC9205031/ /pubmed/35715849 http://dx.doi.org/10.1186/s40942-022-00382-4 Text en © The Author(s) 2022 https://creativecommons.org/licenses/by/4.0/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/ (https://creativecommons.org/licenses/by/4.0/) . The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/ (https://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.
spellingShingle Case Report
de Souza, Eduardo Cunha
Rosa, Evandro
de Oliveira Dias, João Rafael
Malerbi, Fernando Korn
Leal, Bruno Campelo
Junior, Helio Paulo Primiano
Fovea-threatening and fovea-involving peripheral Coats disease: effects of posture and intervention
title Fovea-threatening and fovea-involving peripheral Coats disease: effects of posture and intervention
title_full Fovea-threatening and fovea-involving peripheral Coats disease: effects of posture and intervention
title_fullStr Fovea-threatening and fovea-involving peripheral Coats disease: effects of posture and intervention
title_full_unstemmed Fovea-threatening and fovea-involving peripheral Coats disease: effects of posture and intervention
title_short Fovea-threatening and fovea-involving peripheral Coats disease: effects of posture and intervention
title_sort fovea-threatening and fovea-involving peripheral coats disease: effects of posture and intervention
topic Case Report
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9205031/
https://www.ncbi.nlm.nih.gov/pubmed/35715849
http://dx.doi.org/10.1186/s40942-022-00382-4
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