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Feenstra HMA, van Dijk EHC, Cheung CMG, Ohno-Matsui K, Lai TYY, Koizumi H, Larsen M, Querques G, Downes SM, Yzer S, Breazzano MP, Subhi Y, Tadayoni R, Priglinger SG, Pauleikhoff LJB, Lange CAK, Loewenstein A, Diederen RMH, Schlingemann RO, Hoyng CB, Chhablani JK, Holz FG, Sivaprasad S, Lotery AJ, Yannuzzi LA, Freund KB, Boon CJF. Central serous chorioretinopathy: An evidence-based treatment guideline. Prog Retin Eye Res 2024; 101:101236. [PMID: 38301969 DOI: 10.1016/j.preteyeres.2024.101236] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/12/2023] [Revised: 12/30/2023] [Accepted: 01/02/2024] [Indexed: 02/03/2024]
Abstract
Central serous chorioretinopathy (CSC) is a relatively common disease that causes vision loss due to macular subretinal fluid leakage and it is often associated with reduced vision-related quality of life. In CSC, the leakage of subretinal fluid through defects in the retinal pigment epithelial layer's outer blood-retina barrier appears to occur secondary to choroidal abnormalities and dysfunction. The treatment of CSC is currently the subject of controversy, although recent data obtained from several large randomized controlled trials provide a wealth of new information that can be used to establish a treatment algorithm. Here, we provide a comprehensive overview of our current understanding regarding the pathogenesis of CSC, current therapeutic strategies, and an evidence-based treatment guideline for CSC. In acute CSC, treatment can often be deferred for up to 3-4 months after diagnosis; however, early treatment with either half-dose or half-fluence photodynamic therapy (PDT) with the photosensitive dye verteporfin may be beneficial in selected cases. In chronic CSC, half-dose or half-fluence PDT, which targets the abnormal choroid, should be considered the preferred treatment. If PDT is unavailable, chronic CSC with focal, non-central leakage on angiography may be treated using conventional laser photocoagulation. CSC with concurrent macular neovascularization should be treated with half-dose/half-fluence PDT and/or intravitreal injections of an anti-vascular endothelial growth factor compound. Given the current shortage of verteporfin and the paucity of evidence supporting the efficacy of other treatment options, future studies-ideally, well-designed randomized controlled trials-are needed in order to evaluate new treatment options for CSC.
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Affiliation(s)
- Helena M A Feenstra
- Department of Ophthalmology, Leiden University Medical Center, Leiden, the Netherlands
| | - Elon H C van Dijk
- Department of Ophthalmology, Leiden University Medical Center, Leiden, the Netherlands
| | - Chui Ming Gemmy Cheung
- Singapore Eye Research Institution, Singapore National Eye Centre, Singapore; Duke-NUS Medical School, National University of Singapore, Singapore
| | - Kyoko Ohno-Matsui
- Department of Ophthalmology and Visual Science, Tokyo Medical and Dental University, Tokyo, Japan
| | - Timothy Y Y Lai
- Department of Ophthalmology & Visual Sciences, The Chinese University of Hong Kong, Hong Kong Eye Hospital, Kowloon, Hong Kong
| | - Hideki Koizumi
- Department of Ophthalmology, Graduate School of Medicine, University of the Ryukyus, Nishihara, Japan
| | - Michael Larsen
- Department of Ophthalmology, Rigshospitalet, Glostrup, Denmark; Faculty of Health Sciences, University of Copenhagen, Copenhagen, Denmark
| | - Giuseppe Querques
- Department of Ophthalmology, University Vita-Salute, IRCCS San Raffaele Scientific Institute, Milan, Italy
| | - Susan M Downes
- Oxford Eye Hospital, John Radcliffe Hospital, Oxford University Hospitals NHS Foundation Trust, Oxford, UK; Nuffield Laboratory of Ophthalmology, Nuffield Department of Clinical Neuroscience, University of Oxford, Oxford, UK
| | - Suzanne Yzer
- Department of Ophthalmology, Radboud University Medical Center, Nijmegen, the Netherlands
| | - Mark P Breazzano
- Retina-Vitreous Surgeons of Central New York, Liverpool, NY, USA; Department of Ophthalmology & Visual Sciences, State University of New York Upstate Medical University, Syracuse, NY, USA
| | - Yousif Subhi
- Department of Ophthalmology, Rigshospitalet, Copenhagen, Denmark; Department of Clinical Research, University of Southern Denmark, Odense, Denmark
| | - Ramin Tadayoni
- Ophthalmology Department, AP-HP, Hôpital Lariboisière, Université de Paris, Paris, France
| | - Siegfried G Priglinger
- Department of Ophthalmology, Hospital of the Ludwig-Maximilians-University, Munich, Germany
| | - Laurenz J B Pauleikhoff
- Department of Ophthalmology, Amsterdam University Medical Centers, University of Amsterdam, Amsterdam, the Netherlands; Eye Center, Medical Center - University of Freiburg, Faculty of Medicine, University of Freiburg, Germany
| | - Clemens A K Lange
- Department of Ophthalmology, St. Franziskus Hospital, Muenster, Germany
| | - Anat Loewenstein
- Division of Ophthalmology, Tel Aviv Medical Center, Tel Aviv University, Tel Aviv, Israel
| | - Roselie M H Diederen
- Department of Ophthalmology, Amsterdam University Medical Centers, University of Amsterdam, Amsterdam, the Netherlands
| | - Reinier O Schlingemann
- Department of Ophthalmology, Amsterdam University Medical Centers, University of Amsterdam, Amsterdam, the Netherlands; Ocular Angiogenesis Group, Amsterdam University Medical Centers, Location AMC, University of Amsterdam, Amsterdam, the Netherlands; Department of Ophthalmology, University of Lausanne, Jules-Gonin Eye Hospital, Fondation Asile des Aveugles, Lausanne, Switzerland
| | - Carel B Hoyng
- Department of Ophthalmology, Radboud University Medical Center, Nijmegen, the Netherlands
| | - Jay K Chhablani
- Department of Ophthalmology, University of Pittsburgh School of Medicine, Pittsburgh, PA, USA
| | - Frank G Holz
- Department of Ophthalmology, University of Bonn, Bonn, Germany
| | - Sobha Sivaprasad
- NIHR Biomedical Research Centre, Moorfields Eye Hospital NHS Foundation Trust, London, UK
| | - Andrew J Lotery
- Clinical and Experimental Sciences, Faculty of Medicine, University of Southampton, Southampton, UK
| | - Lawrence A Yannuzzi
- Vitreous Retina Macula Consultants of New York, New York, NY, USA; LuEsther T. Mertz Retinal Research Center, Manhattan Eye, Ear, and Throat Hospital, New York, NY, USA; Department of Ophthalmology, New York University Grossman School of Medicine, New York, USA; Department of Ophthalmology, Manhattan Eye, Ear and Throat Hospital, New York, NY, USA
| | - K Bailey Freund
- Vitreous Retina Macula Consultants of New York, New York, NY, USA; Department of Ophthalmology, New York University School of Medicine, New York, NY, USA
| | - Camiel J F Boon
- Department of Ophthalmology, Leiden University Medical Center, Leiden, the Netherlands; Department of Ophthalmology, Amsterdam University Medical Centers, University of Amsterdam, Amsterdam, the Netherlands.
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Hilely A, Au A, Lee WK, Fogel Levin M, Zur D, Romero-Morales V, Santina A, Lee JS, Loewenstein A, Sarraf D. Pachyvitelliform maculopathy: an optical coherence tomography analysis of a novel entity. Br J Ophthalmol 2024; 108:753-759. [PMID: 37451830 DOI: 10.1136/bjo-2022-322553] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/08/2022] [Accepted: 06/14/2023] [Indexed: 07/18/2023]
Abstract
PURPOSE To describe the optical coherence tomography features of pachyvitelliform maculopathy (PVM), an acquired vitelliform lesion (AVL) associated with pachychoroid disease. METHODS This study was a retrospective, multicentre, observational analysis.Medical records and multimodal imaging were reviewed in all patients with pachychoroid disease and AVL. Visual acuity, central choroidal thickness (CCT), AVL dimensions, total choroidal area, luminal choroidal area, stromal choroidal area and choroidal vascular index were measured in all eyes with PVM and compared with normal age-matched control eyes. RESULTS Mean age of the PVM group (17 eyes of 17 patients) was 71.41 years. Average follow-up was 33.15 months. Baseline VA was 20/40 in the PVM group and declined to 20/100 (p=0.006). AVLs were all detected overlying pachyvessels with optical coherence tomography and were all hyperautofluorescent with fundus autofluorescent imaging. Mean CCT in the PVM group was significantly greater (352.35 µm) than the CCT in the control group (226.88 µm, p<0.001). Retinal pigment epithelium (RPE) disruption was present in 64.71% of eyes with PVM at baseline and 41.18% developed macular atrophy at the end of follow-up. CONCLUSIONS PVM, defined by the presence of AVL associated with pachychoroid features, is a distinct novel entity of the pachychoroid disease spectrum. This study suggests a possible pathogenesis of RPE dysfunction secondary to a thick choroid, leading to accumulation of undigested photoreceptor outer segments and AVL. Clinicians should be aware of this common cause of vitelliform lesions and the poor visual prognosis due to the high risk of atrophy development.
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Affiliation(s)
- Assaf Hilely
- Division of Ophthalmology, Tel Aviv Sourasky Medical Center affiliated to Sackler Faculty of Medicine Tel Aviv University, Tel Aviv, Israel
| | - Adrian Au
- Retinal Disorders and Ophthalmic Genetics Division, Stein Eye Institute David Geffen School of Medicine at UCLA, Los Angeles, California, USA
| | - Won Ki Lee
- Department of Ophthalmology, Nune Eye Hospital, Seoul, Republic of Korea
| | - Miri Fogel Levin
- The Goldschleger Eye Insitute, The Chaim Sheba Medical Center, Tel Hashomer, Israel
| | - Dinah Zur
- Division of Ophthalmology, Tel Aviv Sourasky Medical Center affiliated to Sackler Faculty of Medicine Tel Aviv University, Tel Aviv, Israel
| | - Veronica Romero-Morales
- Retinal Disorders and Ophthalmic Genetics Division, Stein Eye Institute David Geffen School of Medicine at UCLA, Los Angeles, California, USA
| | - Ahmad Santina
- Retinal Disorders and Ophthalmic Genetics Division, Stein Eye Institute David Geffen School of Medicine at UCLA, Los Angeles, California, USA
| | - Jong Suk Lee
- Department of Ophthalmology, Nune Eye Hospital, Seoul, Republic of Korea
| | - Anat Loewenstein
- Division of Ophthalmology, Tel Aviv Sourasky Medical Center affiliated to Sackler Faculty of Medicine Tel Aviv University, Tel Aviv, Israel
| | - David Sarraf
- Retinal Disorders and Ophthalmic Genetics Division, Stein Eye Institute David Geffen School of Medicine at UCLA, Los Angeles, California, USA
- Greater Los Angeles VA Healthcare Center, Los Angeles, California, USA
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Сentral serous chorioretinopathy and vitelliform dystrophies occurring in adults: predictors of differential diagnosis. OPHTHALMOLOGY JOURNAL 2021. [DOI: 10.17816/ov64310] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/08/2022] Open
Abstract
AIM: To study predictors in order to optimize the differential diagnosis of persistent central serous chorioretinopathy (CSCR) and different forms of vitelliform dystrophies occurring in adults.
MATERIALS AND METHODS: Ninety eyes of 61 patients with long-term serous retinal detachments were recruited in study. All patients underwent ophthalmologic examination including family history, best corrected visual acuity, biomicroscopy, and multimodal imaging including fundus photo, SD-OCT, OCT-A, BAF, FA, ICGA. After the studies, the patients were divided into two groups: with vitelliform dystrophies 30 eyes of 30 patients and with CSCR 31 eyes of 31 patients. Diagnostic predictors found in both groups were scrutinized, mathematical models were obtained, and their recognition quality was assessed by the area under ROC curve. The criteria for all types of research were studied and the predictive value was assessed with the use of ROC analysis.
RESULTS: The most informative non-invasive predictors for the diagnosis of vitelliform dystrophies occurring in adults are the following: a positive family history of the disease, brightness and gradient of hyperautofluorescence (hyperAF), typical hyperAF in the form of a crescent and beads, the presence of massive subretinal deposits and deposits in the form of stalactites. The most informative non-invasive predictors for the diagnosis of persistent CSCR are the following: additional hypoAF or hyperAF points or areas outside the main focus, hyperreflective dots in the neurosensory retina and an increase in choroidal thickness, irregular pigment epithelial detachments, presence of CNV. The highest predictive value for both groups was determined for BAF studies.
CONCLUSIONS: The results obtained make it possible to optimize the differential diagnosis of persistent CSCR and different forms of vitelliform dystrophies occurring in adults.
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Dinc UA, Tatlipinar S, Yenerel M, Görgün E, Ciftci F. Fundus autofluorescence in acute and chronic central serous chorioretinopathy. Clin Exp Optom 2021; 94:452-7. [DOI: 10.1111/j.1444-0938.2011.00598.x] [Citation(s) in RCA: 34] [Impact Index Per Article: 8.5] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/29/2022] Open
Affiliation(s)
- Umut Asli Dinc
- Yeditepe University Hospital, Eye Center, Ophthalmology Department, Istanbul, Turkey, E‐mail:
| | - Sinan Tatlipinar
- Yeditepe University Hospital, Eye Center, Ophthalmology Department, Istanbul, Turkey, E‐mail:
| | - Melda Yenerel
- Yeditepe University Hospital, Eye Center, Ophthalmology Department, Istanbul, Turkey, E‐mail:
| | - Ebru Görgün
- Yeditepe University Hospital, Eye Center, Ophthalmology Department, Istanbul, Turkey, E‐mail:
| | - Ferda Ciftci
- Yeditepe University Hospital, Eye Center, Ophthalmology Department, Istanbul, Turkey, E‐mail:
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van Dijk EHC, Boon CJF. Serous business: Delineating the broad spectrum of diseases with subretinal fluid in the macula. Prog Retin Eye Res 2021; 84:100955. [PMID: 33716160 DOI: 10.1016/j.preteyeres.2021.100955] [Citation(s) in RCA: 42] [Impact Index Per Article: 10.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/30/2020] [Revised: 02/14/2021] [Accepted: 02/19/2021] [Indexed: 02/08/2023]
Abstract
A wide range of ocular diseases can present with serous subretinal fluid in the macula and therefore clinically mimic central serous chorioretinopathy (CSC). In this manuscript, we categorise the diseases and conditions that are part of the differential diagnosis into 12 main pathogenic subgroups: neovascular diseases, vitelliform lesions, inflammatory diseases, ocular tumours, haematological malignancies, paraneoplastic syndromes, genetic diseases, ocular developmental anomalies, medication-related conditions and toxicity-related diseases, rhegmatogenous retinal detachment and tractional retinal detachment, retinal vascular diseases, and miscellaneous diseases. In addition, we describe 2 new clinical pictures associated with macular subretinal fluid accumulation, namely serous maculopathy with absence of retinal pigment epithelium (SMARPE) and serous maculopathy due to aspecific choroidopathy (SMACH). Differentiating between these various diseases and CSC can be challenging, and obtaining the correct diagnosis can have immediate therapeutic and prognostic consequences. Here, we describe the key differential diagnostic features of each disease within this clinical spectrum, including representative case examples. Moreover, we discuss the pathogenesis of each disease in order to facilitate the differentiation from typical CSC.
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Affiliation(s)
- Elon H C van Dijk
- Department of Ophthalmology, Leiden University Medical Center, Albinusdreef 2, 2333 ZA, Leiden, the Netherlands
| | - Camiel J F Boon
- Department of Ophthalmology, Leiden University Medical Center, Albinusdreef 2, 2333 ZA, Leiden, the Netherlands; Department of Ophthalmology, Amsterdam University Medical Centers, University of Amsterdam, Meibergdreef 9, 1105 AZ, Amsterdam, the Netherlands.
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Schmitz-Valckenberg S, Pfau M, Fleckenstein M, Staurenghi G, Sparrow JR, Bindewald-Wittich A, Spaide RF, Wolf S, Sadda SR, Holz FG. Fundus autofluorescence imaging. Prog Retin Eye Res 2021; 81:100893. [PMID: 32758681 DOI: 10.1016/j.preteyeres.2020.100893] [Citation(s) in RCA: 72] [Impact Index Per Article: 18.0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/30/2020] [Revised: 07/20/2020] [Accepted: 07/25/2020] [Indexed: 12/20/2022]
Abstract
Fundus autofluorescence (FAF) imaging is an in vivo imaging method that allows for topographic mapping of naturally or pathologically occurring intrinsic fluorophores of the ocular fundus. The dominant sources are fluorophores accumulating as lipofuscin in lysosomal storage bodies in postmitotic retinal pigment epithelium cells as well as other fluorophores that may occur with disease in the outer retina and subretinal space. Photopigments of the photoreceptor outer segments as well as macular pigment and melanin at the fovea and parafovea may act as filters of the excitation light. FAF imaging has been shown to be useful with regard to understanding of pathophysiological mechanisms, diagnostics, phenotype-genotype correlation, identification of prognostic markers for disease progression, and novel outcome parameters to assess efficacy of interventional strategies in chorio-retinal diseases. More recently, the spectrum of FAF imaging has been expanded with increasing use of green in addition to blue FAF, introduction of spectrally-resolved FAF, near-infrared FAF, quantitative FAF imaging and fluorescence life time imaging (FLIO). This article gives an overview of basic principles, FAF findings in various retinal diseases and an update on recent developments.
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Affiliation(s)
- Steffen Schmitz-Valckenberg
- Department of Ophthalmology, University of Bonn, Bonn, Germany; John A. Moran Eye Center, University of Utah, Salt Lake City, USA
| | - Maximilian Pfau
- Department of Ophthalmology, University of Bonn, Bonn, Germany; Department of Biomedical Data Science, Stanford University, USA
| | | | - Giovanni Staurenghi
- Department of Biomedical and Clinical Science "Luigi Sacco", Luigi Sacco Hospital University of Milan, Italy
| | - Janet R Sparrow
- Departments of Ophthalmology and Pathology and Cell Biology, Columbia University Medical Center, New York, NY, USA
| | - Almut Bindewald-Wittich
- Department of Ophthalmology, University of Bonn, Bonn, Germany; Augenheilkunde Heidenheim MVZ, Heidenheim, Germany
| | - Richard F Spaide
- Vitreous Retina Macula Consultants of New York, New York, NY, USA
| | - Sebastian Wolf
- Department of Ophthalmology, Inselspital, Bern University Hospital, University of Bern, Bern, Switzerland
| | - Srinivas R Sadda
- Doheny Eye Institute, David Geffen School of Medicine, University of California, Los Angeles, USA
| | - Frank G Holz
- Department of Ophthalmology, University of Bonn, Bonn, Germany.
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Yata N, Yasukawa T, Kawamura M, Hirano Y, Ogura Y. Macular hole and serous pigment epithelial detachment in bilateral acquired vitelliform lesions. Am J Ophthalmol Case Rep 2020; 18:100628. [PMID: 32140615 PMCID: PMC7049591 DOI: 10.1016/j.ajoc.2020.100628] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/12/2019] [Revised: 11/16/2019] [Accepted: 02/21/2020] [Indexed: 11/30/2022] Open
Abstract
Purpose Acquired vitelliform lesions (AVLs) are associated with age-related macular degeneration and other variable macular disorders. AVLs often lead to outer retinal atrophy, sometimes accompanying a macular hole and choroidal neovascularization. The purpose of this study was to report a rare case with bilateral AVLs, in which one eye had accompanied a macular hole and the second eye a serous pigment epithelial detachment (sPED). Observations A 66-year-old woman complained of bilateral metamorphopsia. AVLs were observed in the right eye and a flat sPED in the left eye. The best-corrected visual acuity (BCVA) was 20/17 in both eyes. Fluorescein angiography revealed local leakage in the right eye and pattern dystrophy-like hypofluorescence in both eyes. The sPED progressed with AVLs in the left eye and was treated with a combination therapy of intravitreal aflibercept, a sub-Tenon's injection of triamcinolone acetonide, and photodynamic therapy (IVA/STTA/PDT), which successfully flattened the sPED and sustained good vision for 4 years. The right eye was treated with intravitreal ranibizumab and tissue plasminogen activator, which enhanced absorption of the vitelliform material. However, 14 months later, a macular hole with typical metamorphopsia formed above a subretinal fibrotic scar at the vitelliruptive stage. Although pars plana vitrectomy closed the macular hole, enlargement of the outer retinal atrophy worsened the BCVA to 20/100. Conclusions and importance We successfully treated one eye with a sPED with AVLs using the combination therapy of IVA/STTA/PDT, while the second eye with a macular hole secondary to AVLs ultimately developed outer retinal atrophy with visual loss.
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Affiliation(s)
- Nana Yata
- Department of Ophthalmology and Visual Science, Nagoya City University Graduate School of Medical Sciences, Nagoya, Japan
| | - Tsutomu Yasukawa
- Department of Ophthalmology and Visual Science, Nagoya City University Graduate School of Medical Sciences, Nagoya, Japan
| | - Mihoko Kawamura
- Department of Ophthalmology and Visual Science, Nagoya City University Graduate School of Medical Sciences, Nagoya, Japan
| | - Yoshio Hirano
- Department of Ophthalmology and Visual Science, Nagoya City University Graduate School of Medical Sciences, Nagoya, Japan
| | - Yuichiro Ogura
- Department of Ophthalmology and Visual Science, Nagoya City University Graduate School of Medical Sciences, Nagoya, Japan
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Wang D, Au A, Gunnemann F, Hilely A, Scharf J, Tran K, Sun M, Kim JH, Sarraf D. Pentosan-associated maculopathy: prevalence, screening guidelines, and spectrum of findings based on prospective multimodal analysis. Can J Ophthalmol 2020; 55:116-125. [PMID: 31973791 DOI: 10.1016/j.jcjo.2019.12.001] [Citation(s) in RCA: 35] [Impact Index Per Article: 7.0] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/25/2019] [Revised: 12/10/2019] [Accepted: 12/11/2019] [Indexed: 01/31/2023]
Abstract
OBJECTIVE To describe the prevalence and spectrum of multimodal imaging findings of pentosan polysulfate sodium (PPS)-associated maculopathy and to recommend dosage-related screening guidelines. DESIGN Cross-sectional study. METHODS Patients previously or currently treated with PPS at University of California, Los Angeles, were randomly ascertained and prospectively screened for PPS-associated maculopathy with multimodal retinal imaging. Daily and cumulative dosages of PPS exposure were calculated for each patient. Images were studied to identify the characteristic findings of toxicity. The prevalence of PPS-associated maculopathy and screening guidelines were determined. RESULTS The prevalence of PPS-associated maculopathy in this cohort was 20% (10/50 patients). Both average duration of PPS therapy and average cumulative dosage were significantly lower in the unaffected (6.3 ± 6.6 years, 691.7 ± 706.6 g) versus the affected groups (20.3 ± 6.6 years, 3375.4 ± 1650.0 g, p < 0.001). Near-infrared reflectance (NIR) illustrated characteristic punctate retinal pigment epithelium (RPE) macular lesions early. Fundus autofluorescence (FAF) showed speckled autofluorescence in the posterior pole with peripapillary extension. Co-localization with optical coherence tomography (OCT) displayed focal RPE thickening and, in more severe cases, RPE atrophy in the macula and even the periphery. CONCLUSIONS A prevalence of 20% in this study cohort suggests a significant risk of macular toxicity for PPS-treated patients. Characteristic alterations are best detected with FAF and NIR. More significant PPS exposure was associated with more severe atrophy. We recommend an initial baseline eye examination to include OCT and, most importantly, NIR and FAF with annual retinal imaging thereafter especially with cumulative dosages approaching 500 g. Patients exposed to greater than 1500 g of PPS are at significant risk of retinal toxicity.
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Affiliation(s)
- Derrick Wang
- Retinal Disorders and Ophthalmic Genetics, Stein Eye Institute, University of California, Los Angeles, Los Angeles, CA
| | - Adrian Au
- Retinal Disorders and Ophthalmic Genetics, Stein Eye Institute, University of California, Los Angeles, Los Angeles, CA
| | - Frederic Gunnemann
- Retinal Disorders and Ophthalmic Genetics, Stein Eye Institute, University of California, Los Angeles, Los Angeles, CA
| | - Assaf Hilely
- Retinal Disorders and Ophthalmic Genetics, Stein Eye Institute, University of California, Los Angeles, Los Angeles, CA; Division of Ophthalmology, Tel Aviv Medical Center, Sackler Faculty of Medicine, Tel Aviv University, Tel Aviv, Israel
| | - Jackson Scharf
- Retinal Disorders and Ophthalmic Genetics, Stein Eye Institute, University of California, Los Angeles, Los Angeles, CA
| | - Khoi Tran
- Retinal Disorders and Ophthalmic Genetics, Stein Eye Institute, University of California, Los Angeles, Los Angeles, CA
| | - Michel Sun
- Retinal Disorders and Ophthalmic Genetics, Stein Eye Institute, University of California, Los Angeles, Los Angeles, CA
| | - Ja-Hong Kim
- Department of Urology, UCLA Medical Center, David Geffen School of Medicine at UCLA, Los Angeles, CA
| | - David Sarraf
- Retinal Disorders and Ophthalmic Genetics, Stein Eye Institute, University of California, Los Angeles, Los Angeles, CA; Greater Los Angeles VA Healthcare Center, Los Angeles, CA.
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9
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Spaide RF, Ooto S, Curcio CA. Subretinal drusenoid deposits AKA pseudodrusen. Surv Ophthalmol 2018; 63:782-815. [PMID: 29859199 DOI: 10.1016/j.survophthal.2018.05.005] [Citation(s) in RCA: 183] [Impact Index Per Article: 26.1] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 09/27/2017] [Revised: 05/17/2018] [Accepted: 05/21/2018] [Indexed: 01/30/2023]
Abstract
A distinction between conventional drusen and pseudodrusen was first made in 1990, and more recently knowledge of pseudodrusen, more accurately called subretinal drusenoid deposits (SDDs), has expanded. Pseudodrusen have a bluish-white appearance by biomicroscopy and color fundus photography. Using optical coherence tomography, pseudodrusen were found to be accumulations of material internal to the retinal pigment epithelium that could extend internally through the ellipsoid zone. These deposits are more commonly seen in older eyes with thinner choroids. Histologic evaluation of these deposits revealed aggregations of material in the subretinal space between photoreceptors and retinal pigment epithelium. SDDs contain some proteins in common with soft drusen but differ in lipid composition. Many studies reported that SDDs are strong independent risk factors for late age-related macular degeneration. Geographic atrophy and type 3 neovascularization are particularly associated with SDD. Unlike conventional drusen, eyes with SDD show slow dark adaptation and poor contrast sensitivity. Outer retinal atrophy develops in eyes with regression of SDD, a newly recognized form of late age-related macular degeneration. Advances in imaging technology have enabled many insights into this condition, including associated photoreceptor, retinal pigment epithelium, and underlying choroidal changes.
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Affiliation(s)
- Richard F Spaide
- Vitreous Retina Macula Consultants of New York and LuEsther T. Mertz Retinal Research Center, Manhattan Eye, Ear and Throat Hospital, New York, New York, USA.
| | - Sotaro Ooto
- Vitreous Retina Macula Consultants of New York and LuEsther T. Mertz Retinal Research Center, Manhattan Eye, Ear and Throat Hospital, New York, New York, USA; Department of Ophthalmology, Kyoto University Graduate School of Medicine, Kyoto, Japan
| | - Christine A Curcio
- Department of Ophthalmology, School of Medicine, University of Alabama at Birmingham, Birmingham, Alabamas, USA
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Abstract
PURPOSE Central serous chorioretinopathy (CSC) is a condition characterized by serous detachment of the neurosensory retina at the level of the retinal pigmented epithelium (RPE) as a result of leakage from the choriocapillaris. The pathophysiology of CSC is not completely understood rendering treatment and management decisions more complex. When an observational approach and topical medical therapy are unsuccessful, a surgical intervention may be necessary. Here, we examine the role of modified photodynamic therapy (PDT) in recurrent CSC with multiple points of RPE leakage. CASE REPORT A 37-year-old male patient presented a case of recurrent CSC in the left eye. After an initial presentation of a large area of submacular fluid, near complete resolution was seen in 8 weeks using a topical NSAID protocol and close monitoring. Two weeks later, a second serous neurosensory detachment larger than the first occurred. The recurrent CSC was successfully treated with focal grid laser photocoagulation and modified PDT resulting in complete resolution and return of visual function 4 months after the initial presentation. CONCLUSIONS CSC is a multifactorial condition with the potential to severely impair daily visual function. Spectral domain optical coherence tomography retinal imaging has provided a remarkable tool in the diagnosis and monitoring of CSC. The addition of FA and ICG can create a highly precise picture of the subretinal fluid. Although most patients will spontaneously resolve, a subset of patients may benefit from medical therapy including topical nepafenac 0.1%. A smaller subset will require surgical intervention. Modified PDT with ICG-guided laser photocoagulation has a growing body of evidence as an effective treatment for recurrent CSC. Modified PDT may be the best course of action for chronic, non-resolving RPE leakage for both paramacular and foveal leaks leading to minimization of adverse visual effects.
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CHOROIDAL THICKENING IN PATIENTS WITH CUTICULAR DRUSEN COMBINED WITH VITELLIFORM MACULAR DETACHMENT. Retina 2015; 36:1111-8. [PMID: 26536099 DOI: 10.1097/iae.0000000000000831] [Citation(s) in RCA: 6] [Impact Index Per Article: 0.6] [Reference Citation Analysis] [Abstract] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Indexed: 11/26/2022]
Abstract
PURPOSE To analyze the subfoveal choroidal thickness (SFCT) in patients with cuticular drusen. METHODS Retrospective, monocentric, study of consecutive patients examined with enhanced depth imaging spectral domain optical coherence tomography (EDI SD-OCT, Cirrus, Zeiss) between 2009 and 2014 in a tertiary care center. Measurements of the height of the subfoveal vitelliform detachment and SFCT were manually performed. RESULTS Thirteen patients, 3 men and 10 women, aged from 35 to 73 (mean: 53.6 years) were selected. For the 24 eyes without macular atrophy at first visit, SFCT ranged from 195 to 559 µm (mean ± SD = 317.5 ± 93). The SFCT was significantly thicker in 12 eyes with vitelliform macular detachment at presentation (369 ± 96, median = 368.5) than in 12 eyes without (266 ± 58, median = 257.5) (P = 0.007), whereas the 2 groups did not differ in age (P = 0.35) or refractive error (P = 0.56). No correlation was observed between SFCT and the height of the foveal detachment. For 10 eyes followed up longer than 24 months (mean: 38.9 months), the SFCT significantly decreased over time, from 375 ± 96 (median = 368.5) to 303 ± 138 (median = 319) µm (P = 0.022). CONCLUSION Eyes with cuticular drusen combined with vitelliform macular detachment present with choroidal thickening, suggesting that the choroidal vasculature may play a role in the occurrence of macular detachments in patients with cuticular drusen. The life cycle of these vitelliform lesions evolves from translucent subretinal fluid to the accumulation of yellowish material eventually resolving and leading to atrophy with marked and rapid thinning of the choroid.
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Adult-onset foveomacular vitelliform dystrophy: A fresh perspective. Prog Retin Eye Res 2015; 47:64-85. [DOI: 10.1016/j.preteyeres.2015.02.001] [Citation(s) in RCA: 68] [Impact Index Per Article: 6.8] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/08/2014] [Revised: 02/01/2015] [Accepted: 02/04/2015] [Indexed: 01/06/2023]
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Mateo-Montoya A, Mauget-Faÿse M. Helicobacter pylori as a risk factor for central serous chorioretinopathy: Literature review. World J Gastrointest Pathophysiol 2014; 5:355-358. [PMID: 25133035 PMCID: PMC4133532 DOI: 10.4291/wjgp.v5.i3.355] [Citation(s) in RCA: 9] [Impact Index Per Article: 0.8] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 01/26/2014] [Revised: 05/04/2014] [Accepted: 05/29/2014] [Indexed: 02/06/2023] Open
Abstract
Helicobacter pylori (H. pylori), a Gram-negative bacterium, is one of the most frequent causes of gastrointestinal infections worldwide. It has been associated as a pathogen for the human body with many systemic diseases, including different eye diseases. We will focus on a specific eye disease called idiopathic central serous chorioretinopathy (ICSCR). This disease is characterized by a serous detachment of the neurosensory retina in the macular region, which affects the vision to different degrees. Currently, the pathophysiology of ICSCR is not clear and there is no effective treatment. However, several potential risk factors have been elucidated. One of the factors that has more frequently been associated with ICSCR is stress. As H. pylori was identified as a possible etiological factor for occlusive arterial diseases in young people who were particularly stressed, it was thought that H. pylori might also be present in ICSCR. Therefore, some physicians started to test its presence in patents with ICSCR. If H. pylori happened to be associated with ICSCR, the treatment of gastrointestinal infection could also improve visual symptoms and help to remediate this eye disease. Although H. pylori is highly prevalent in the general population, a true correlation seems to exist. We present a review on the relationship between ICSCR and H. pylori.
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Dolz-Marco R, Gallego-Pinazo R, Díaz-Llopis M. Natural course of adult-onset foveomacular vitelliform dystrophy: a spectral-domain optical coherence tomography analysis. Am J Ophthalmol 2012; 153:389; author reply 389-90. [PMID: 22264960 DOI: 10.1016/j.ajo.2011.11.027] [Citation(s) in RCA: 1] [Impact Index Per Article: 0.1] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/31/2011] [Revised: 11/04/2011] [Accepted: 11/16/2011] [Indexed: 10/14/2022]
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Kandula S, Zweifel S, Freund KB. Adult-Onset Vitelliform Detachment Unresponsive to Monthly Intravitreal Ranibizumab. Ophthalmic Surg Lasers Imaging Retina 2010; 41 Suppl:S81-4. [PMID: 21117608 DOI: 10.3928/15428877-20101031-09] [Citation(s) in RCA: 10] [Impact Index Per Article: 0.7] [Reference Citation Analysis] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 10/01/2009] [Accepted: 03/30/2010] [Indexed: 11/20/2022]
Affiliation(s)
- Sushma Kandula
- LuEsther T. Mertz Retinal Research Center, Manhattan Eye, Ear, and Throat Hospital, New York, NY, USA
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Subfoveal Deposits Secondary to Idiopathic Epiretinal Membranes. Ophthalmology 2009; 116:1794-8. [DOI: 10.1016/j.ophtha.2009.06.005] [Citation(s) in RCA: 20] [Impact Index Per Article: 1.3] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/01/2008] [Revised: 05/29/2009] [Accepted: 06/03/2009] [Indexed: 10/20/2022] Open
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Abstract
PURPOSE To review the pathophysiologic principles underlying increased autofluorescence from the outer retina and subretinal space using selected diseases as examples. METHODS The ocular imaging information and histopathologic features, when known, were integrated for diseases causing increased autofluorescence from the outer retina and subretinal space. Inferences were taken from this information and used to create a classification scheme. RESULTS These diseases are principally those that cause separation of the outer retina from the retinal pigment epithelium, thereby preventing proper phagocytosis of photoreceptor outer segments. The separation can arise from increased exudation into the subretinal space or inadequate removal of fluid from the subretinal space. Lack of normal outer segment processing initially leads to increased accumulation of outer segments on the outer retina and subretinal space. Over time, this material is visible as an increasingly thick coating on the outer retina, is yellow, and is autofluorescent. Over time, atrophy develops with thinning of the deposited material and decreasing autofluorescence. The accumulated material is ultimately capable of inducing damage to the retinal pigment epithelium. Diseases causing accumulation of the material include central serous chorioretinopathy, vitelliform macular dystrophy, acute exudative polymorphous vitelliform maculopathy, choroidal tumors, and vitreomacular traction syndrome. CONCLUSION The physical separation of the retinal outer segments from the retinal pigment epithelium hinders proper phagocytosis of the outer segments. Accumulation of the shed but not phagocytized outer segments plays a role in disease manifestations for a number of macular diseases.
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Spaide RF, Noble K, Morgan A, Freund KB. Vitelliform Macular Dystrophy. Ophthalmology 2006; 113:1392-400. [PMID: 16877078 DOI: 10.1016/j.ophtha.2006.03.023] [Citation(s) in RCA: 109] [Impact Index Per Article: 5.7] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/25/2005] [Revised: 03/06/2006] [Accepted: 03/06/2006] [Indexed: 11/28/2022] Open
Abstract
PURPOSE To investigate and integrate the photographic, angiographic, and tomographic findings from a group of patients with various stages of vitelliform macular dystrophy type 2 (VMD2; also known as Best's disease) and use this information to propose mechanisms of disease pathogenesis. DESIGN Retrospective observational case series. PARTICIPANTS Nine consecutive patients seen in a private practice referral setting by the authors. METHODS Patients with VMD2 were imaged with conventional fundus and autofluorescence photography, fluorescein angiography, fundus photography, and optical coherence tomography (OCT). MAIN OUTCOME MEASURES The integrated ocular imaging findings. RESULTS Early stage lesions were smaller and had accumulation of yellowish material in the central macula. This material was highly autofluorescent and appeared to be located on the outer retinal surface by OCT. Later stages were characterized by larger lesions with central clearing of the yellowish material and deposition of autofluorescent subretinal material at the outer borders of the lesion. Both early and late lesions had a subretinal fluid component with no reflectivity as detected by OCT. Fluorescein angiography showed transmission defects with a suggestion of late leakage, much like that seen in chronic central serous chorioretinopathy (CSC). CONCLUSIONS Similar to that seen in CSC, patients with VMD2 have an accumulation of material on the outer retina, which may represent shed photoreceptor outer segments in association with subretinal fluid.
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Affiliation(s)
- Richard F Spaide
- Vitreous, Retina, Macula Consultants of New York and the LuEsther T. Mertz Retina Research Laboratory, Manhattan Eye, Ear, and Throat Hospital, New York, New York 10022, USA.
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