PREDNISOLONE EYE DROPS AS A POTENTIAL TREATMENT IN NONNEOVASCULAR PACHYCHOROID-RELATED DISEASES.

PURPOSE: To investigate the functional and structural outcomes after treatment with prednisolone eye drops in the following pachychoroid-related diseases: chronic central serous chorioretinopathy, pachychoroid pigment epitheliopathy, and peripapillary pachychoroid syndrome. METHODS: In this retrospective study, 54 eyes of 48 patients with pachychoroid-related disease were treated with prednisolone acetate 1% eye drops 3 times a day. Change in macular volume and retinal central subfield thickness on optical coherence tomography was measured. In addition, the foveal or complete resolution of fluid and the change in visual acuity were studied. RESULTS: The follow-up visit was at a mean of 41.2 ± 14.5 days. In the 44 eyes with chronic central serous chorioretinopathy, a significant reduction in retinal central subfield thickness ( P < 0.001) and macular volume ( P < 0.001) was observed. Foveal intra- or subretinal fluid resolved completely in 22% of the eyes. In the 8 peripapillary pachychoroid syndrome eyes, a reduction in the nasal retinal thickness was observed ( P = 0.025). One of the 2 pachychoroid pigment epitheliopathy eyes showed structural improvement. No significant change in visual acuity was observed in any of the pachychoroid spectrum diseases. CONCLUSION: In patients with chronic central serous chorioretinopathy, peripapillary pachychoroid syndrome, and pachychoroid pigment epitheliopathy, anatomical improvement was observed after therapy with prednisolone eye drops. Visual acuity did not change significantly.

Rare genetic variation in VE-PTP is associated with central serous chorioretinopathy, venous dysfunction and glaucoma.

Central serous chorioretinopathy (CSC) is a fluid maculopathy whose etiology is not well understood. Abnormal choroidal veins in CSC patients have been shown to have similarities with varicose veins. To identify potential mechanisms, we analyzed genotype data from 1,477 CSC patients and 455,449 controls in FinnGen. We identified an association for a low-frequency (AF=0.5%) missense variant (rs113791087) in the gene encoding vascular endothelial protein tyrosine phosphatase (VE-PTP) (OR=2.85, P=4.5×10-9). This was confirmed in a meta-analysis of 2,452 CSC patients and 865,767 controls from 4 studies (OR=3.06, P=7.4×10-15). Rs113791087 was associated with a 56% higher prevalence of retinal abnormalities (35.3% vs 22.6%, P=8.0×10-4) in 708 UK Biobank participants and, surprisingly, with varicose veins (OR=1.31, P=2.3×10-11) and glaucoma (OR=0.82, P=6.9×10-9). Predicted loss-of-function variants in VEPTP, though rare in number, were associated with CSC in All of Us (OR=17.10, P=0.018). These findings highlight the significance of VE-PTP in diverse ocular and systemic vascular diseases.

Perspectives and Update on the Global Shortage of Verteporfin (Visudyne®).

An ongoing global shortage of verteporfin (Visudyne®) limits the treatment possibilities for several chorioretinal diseases, including central serous chorioretinopathy, choroidal hemangioma, and polypoidal choroidal vasculopathy. Verteporfin is required to perform photodynamic therapy in these ocular diseases. Therefore, the current situation has a substantial impact on eye care worldwide. The worldwide supply of verteporfin appears to be manufactured by a single factory, which is situated in the United States. The distribution of verteporfin is done by different companies for different regions of the world. Official communication on the shortage by the responsible companies has been scarce and over the past years several promises with regards to resolution of the shortage have not been fulfilled. The delivery of new batches of verteporfin is at irregular intervals, unpredictable, and may not be fairly balanced between different regions or countries in the world. To ensure a fair distribution of available verteporfin within a country, several measures can be taken. In the Netherlands, a national committee, consisting of ophthalmologists, is in place to arrange this. On the European level, the European Union and European Medicine Agency have plans to monitor medicine shortages more closely and to intervene if necessary. With a more intensified monitoring and regulation of medicine supplies, future impending shortages may be prevented. Remarkably, the amount of medicine shortages is increasing, having a significant and sometimes irreversible impact on patient care. Thus, efforts should be undertaken to minimize the consequences and, whenever possible, to prevent future medicine shortages.

Central serous chorioretinopathy: An evidence-based treatment guideline.

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.

Staphyloma-induced Serous Maculopathy: Natural Course and Treatment Effects.

PURPOSE: To study the natural course of staphyloma-induced serous maculopathy (SISM) and the effects of treatments. DESIGN: Retrospective case series. PARTICIPANTS: This retrospective analysis included 26 eyes of 20 patients with SISM and at least 12 months of follow-up. METHODS: Medical records were reviewed for patient demographics, such as age, sex, spherical equivalent, best-corrected visual acuity (BCVA), type of staphyloma, and imaging characteristics. Spectralis OCT B-scans were evaluated for the presence and height of the serous retinal detachment (SRD) at each follow-up visit. An SRD episode was defined as a period with SRD in 1 patient. MAIN OUTCOME MEASURES: Changes in SRD height and BCVA. RESULTS: Twenty-six eyes of 20 patients (70% female) were included. The mean age was 54 ± 11 years, and the mean spherical equivalent was -4.8 ± 3.3 diopters at baseline. The staphyloma was located inferior in 12 eyes (46%), inferonasal in 7 eyes (27%), and nasal in 7 eyes (27%). The mean follow-up duration was 73 ± 34 months. During follow-up, the SRD height fluctuated in all eyes, with a mean change of 125 ± 56 µm. The SRD disappeared completely during follow-up in 13 eyes (50%) and then reappeared in 7 eyes (35%). Resolution occurred spontaneous in 8 eyes (31%). The median time of an SRD episode was 25 (interquartile range 14-57) months. Treatment was performed in 20 eyes (77%) and led to resolution of SRD in 3 of the 15 photodynamic therapy treatments (21%), 2 of 5 (40%) anti-VEGF series, and 2 of 4 eyes (50%) treated with topical prednisolone. Best-corrected visual acuity at the final visit (0.42 ± 0.25) was not significantly different from BCVA at baseline (0.34 ± 0.27 logarithm of the minimum angle of resolution, P = 0.07), nor was BCVA change significantly different between treated eyes (n = 19) and nontreated eyes (n = 7, P = 0.3). CONCLUSION: Serous retinal detachment in patients with SISM fluctuated over time and resolved without treatment in 31% of the eyes. Because treatment does not change the course of BCVA, a wait-and-see policy is advocated in these patients on the exclusion of treatable causes of SRD. FINANCIAL DISCLOSURE(S): Proprietary or commercial disclosure may be found in the Footnotes and Disclosures at the end of this article.

Incidence and Risk Factors of Second Eye Involvement in Myopic Macular Neovascularization.

PURPOSE: To report the cumulative incidence and risk factors of second eye involvement after diagnosis of myopic macular neovascularization (MNV) in the first eye. DESIGN: Retrospective analysis of longitudinal data from a tertiary hospital in the Netherlands. PARTICIPANTS: Patients with high myopia (spherical equivalent [SE] ≤ - 6 diopters [D]), of European ethnicity, who were diagnosed with active MNV lesion in 1 eye between 2005 and 2018. Fellow eyes were free of MNV or macular atrophy at baseline, and data were collected on the SE, axial length, and presence of diffuse or patchy chorioretinal atrophy and lacquer cracks. METHODS: Incidence rate and 2-, 5-, and 10-year cumulative incidences were calculated; hazard ratios (HRs) of second eye involvement were analyzed for potential risk factors using Cox proportional hazard models. MAIN OUTCOMES MEASURES: Incidence of second eye involvement after onset of myopic MNV in the first eye. RESULTS: We included 88 patients over a period of 13 years with a mean age of 58 ± 15 years, mean axial length of 30 ± 1.7 mm and SE -14 ± 4 D at baseline. Twenty-four fellow eyes (27%) developed a myopic MNV during follow-up. This resulted in an incidence rate of 4.6 (95% confidence interval [CI], 2.9-6.7) per 100 person-years and a cumulative incidence of 8%, 21%, and 38% at 2, 5, and 10 years, respectively. Mean time until MNV development in the fellow eye was 48 ± 37 months. Patients aged < 40 years at the initial presentation had a 3.8 times higher risk of bilateral myopic MNV (HR, 3.8; 95% CI, 1.65-8.69; P = 0.002). The presence of lacquer cracks in the second eye seemed to increase risk, but this did not reach statistical significance (HR, 2.25; 95% CI, 0.94-5.39; P = 0.07). CONCLUSIONS: Our study of high myopes of European descent shows very similar incidence rates for second eye myopic MNV compared with Asian studies. Our findings substantiate the importance for clinicians to monitor closely and create awareness, especially in younger patients. FINANCIAL DISCLOSURE(S): The authors have no proprietary or commercial interest in any materials discussed in this article.

Evaluation of Sleep Quality and Fatigue in Patients with Usher Syndrome Type 2a.

Purpose: To study the prevalence, level, and nature of sleep problems and fatigue experienced by Usher syndrome type 2a (USH2a) patients. Design: Cross-sectional study. Participants: Fifty-six genetically confirmed Dutch patients with syndromic USH2a and 120 healthy controls. Methods: Sleep quality, prevalence, and type of sleep disorders, chronotype, fatigue, and daytime sleepiness were assessed using 5 questionnaires: (1) Pittsburgh Sleep Quality Index, (2) Holland Sleep Disorders Questionnaire, (3) Morningness-Eveningness Questionnaire, (4) Checklist Individual Strength, and (5) Epworth Sleepiness Scale. For a subset of patients, recent data on visual function were used to study the potential correlation between the outcomes of the questionnaires and disease progression. Main Outcome Measures: Results of all questionnaires were compared between USH2a and control cohorts, and the scores of the patients were compared with disease progression defined by age, visual field size, and visual acuity. Results: Compared with the control population, patients with USH2a experienced a poorer quality of sleep, a higher incidence of sleep disorders, and higher levels of fatigue and daytime sleepiness. Intriguingly, the sleep disturbances and high levels of fatigue were not correlated with the level of visual impairment. These results are in accordance with the patients' experiences that their sleep problems already existed before the onset of vision loss. Conclusions: This study demonstrates a high prevalence of fatigue and poor sleep quality experienced by patients with USH2a. Recognition of sleep problems as a comorbidity of Usher syndrome would be a first step toward improved patient care. The absence of a relationship between the level of visual impairment and the severity of reported sleep problems is suggestive of an extraretinal origin of the sleep disturbances. Financial Disclosures: Proprietary or commercial disclosure may be found after the references.

POTENTIAL TREATMENT FOR PERIPAPILLARY PACHYCHOROID SYNDROME.

PURPOSE: The purpose of this study was to describe the effect of topical prednisolone on intraretinal fluid in patients with peripapillary pachychoroid syndrome. METHODS: We selected 11 consecutive patients (17 eyes) with a diagnosis of peripapillary pachychoroid syndrome, who were treated with topical prednisolone (Pred Forte [PF] 10 mg/mL) three times daily for 4 weeks. We tapered off PF among patients who demonstrated a reduction of intraretinal fluid. RESULTS: Of the included 17 eyes, the average follow-up before PF treatment ranged from 6 to 192 months, during which patients experienced no apparent reduction of intraretinal fluid. The baseline mean best-corrected VA (BCVA) was 0.6 (20/33) Snellen. The median subfoveal and peripapillary choroidal thickness were 430 µm and 202 µm, respectively. All patients showed an initial reduction of intraretinal fluid after 4 weeks of topical prednisolone. Six patients (35%) experienced a prolonged reduction of intraretinal fluid when the dosage was reduced to once daily. On tapering off PF, four eyes (24%) experienced a recurrence of intraretinal fluid. Four eyes (24%) experienced elevated intraocular pressure above 26 mmHg. In two eyes, PF was discontinued, on which intraretinal fluid reappeared. The BCVA seemed to be improved in 9 eyes (53%) and remained equal in 4 eyes (24%). CONCLUSION: In this case series of patients with peripapillary pachychoroid syndrome, we observed a reduction of peripapillary intraretinal fluid after treatment with topical prednisolone for 4 weeks in all 17 eyes. The disappearance of intraretinal fluid seemed to concede with a slight improvement in BCVA for some cases. Thus, topical prednisolone may prove to be a viable treatment option in peripapillary pachychoroid syndrome.

Overlap of Genetic Loci for Central Serous Chorioretinopathy With Age-Related Macular Degeneration.

Importance: Central serous chorioretinopathy (CSC) is a serous maculopathy of unknown etiology. Two of 3 previously reported CSC genetic risk loci are also associated with AMD. Improved understanding of CSC genetics may broaden our understanding of this genetic overlap and unveil mechanisms in both diseases. Objective: To identify novel genetic risk factors for CSC and compare genetic risk factors for CSC and AMD. Design, Setting, and Participants: Using International Classification of Diseases, Ninth (ICD-9) and Tenth (ICD-10) Revision code-based inclusion and exclusion criteria, patients with CSC and controls were identified in both the FinnGen study and the Estonian Biobank (EstBB). Also included in a meta-analysis were previously reported patients with chronic CSC and controls. Data were analyzed from March 1 to September 31, 2022. Main Outcomes and Measures: Genome-wide association studies (GWASs) were performed in the biobank-based cohorts followed by a meta-analysis of all cohorts. The expression of genes prioritized by the polygenic priority score and nearest-gene methods were assessed in cultured choroidal endothelial cells and public ocular single-cell RNA sequencing data sets. The predictive utility of polygenic scores (PGSs) for CSC and AMD were evaluated in the FinnGen study. Results: A total of 1176 patients with CSC and 526 787 controls (312 162 female [59.3%]) were included in this analysis: 552 patients with CSC and 343 461 controls were identified in the FinnGen study, 103 patients with CSC and 178 573 controls were identified in the EstBB, and 521 patients with chronic CSC and 3577 controls were included in a meta-analysis. Two previously reported CSC risk loci were replicated (near CFH and GATA5) and 3 novel loci were identified (near CD34/46, NOTCH4, and PREX1). The CFH and NOTCH4 loci were associated with AMD but in the opposite direction. Prioritized genes showed increased expression in cultured choroidal endothelial cells compared with other genes in the loci (median [IQR] of log 2 [counts per million], 7.3 [0.6] vs 4.7 [3.7]; P = .004) and were differentially expressed in choroidal vascular endothelial cells in single-cell RNA sequencing data (mean [SD] fold change, 2.05 [0.38] compared with other cell types; P < 7.1 × 10-20). A PGS for AMD was predictive of reduced CSC risk (odds ratio, 0.76; 95% CI, 0.70-0.83 per +1 SD in AMD-PGS; P = 7.4 × 10-10). This association may have been mediated by loci containing complement genes. Conclusions and Relevance: In this 3-cohort genetic association study, 5 genetic risk loci for CSC were identified, highlighting a likely role for genes involved in choroidal vascular function and complement regulation. Results suggest that polygenic AMD risk was associated with reduced risk of CSC and that this genetic overlap was largely due to loci containing complement genes.

High Levels of C-Reactive Protein with Low Levels of Pentraxin 3 as Biomarkers for Central Serous Chorioretinopathy.

Purpose: To investigate the association between the 2 acute phase proteins, C-reactive protein (CRP) and pentraxin 3 (PTX3) with central serous chorioretinopathy (CSCR), as PTX3 is a glucocorticoid-induced protein. Design: Cross-sectional multicenter study. Participants: Patients with CSCR compared with age- and sex-matched healthy participants. Methods: Patients with CSCR from 3 centers in Europe were included in the study. The clinical form of CSCR was recorded. Blood samples from patients with CSCR and healthy participants were sampled, and high-sensitivity CRP and PTX3 levels were measured in the serum. Main Outcome Measures: C-reactive protein and PTX3 serum level comparison between patients with CSCR with age- and sex-matched healthy participants. Results: Although CRP levels were higher in patients with CSCR (n = 216) than in age- and sex-matched controls (n = 130) (2.2 ± 3.2 mg/l vs. 1.5 mg/l ± 1.4, respectively, P = 0.037), PTX3 levels were lower in patients with CSCR (10.5 ± 19.9 pg/ml vs. 87.4 ± 73.2 pg/ml, respectively, P < 0.001). There was no significant difference in CRP or PTX3 levels between patients with acute/recurrent and chronic CSCR. Conclusions: In patients with CSCR, high CRP and low PTX3 levels suggest a form of low-grade systemic inflammation together with a lack of glucocorticoid pathway activation, raising new hypotheses on the pathophysiology of CSCR. Financial Disclosures: The author(s) have no proprietary or commercial interest in any materials discussed in this article.

INCREASING EVIDENCE FOR THE SAFETY OF FOVEA-INVOLVING HALF-DOSE PHOTODYNAMIC THERAPY FOR CHRONIC CENTRAL SEROUS CHORIORETINOPATHY.

PURPOSE: A retrospective study was performed with data from the prospective randomized controlled trials, PLACE and SPECTRA, assessing the risk of foveal atrophy and the likelihood of structural and functional improvement on optical coherence tomography, after foveal half-dose photodynamic therapy in chronic central serous chorioretinopathy. METHODS: A total of 57 chronic central serous chorioretinopathy patients received a single half-dose photodynamic therapy with a treatment spot that included the fovea. Optical coherence tomography scans and fundus autofluorescence images were analyzed for structural improvement and possible atrophy development, at baseline and at several visits after treatment. Main outcome measures were integrity of the external limiting membrane and ellipsoid zone on optical coherence tomography and hypoautofluorescence on fundus autofluorescence. RESULTS: The subfoveal external limiting membrane was graded as continuous in 21 of 57 of patients (36.8%) at baseline, and the subfoveal ellipsoid zone was graded as continuous in 5 of 57 patients (8.8%) at first visit, which improved to 50 of 51 (98.0%) and 32 out of 51 (62.7%) at the final visit at 2 years, respectively (both P < 0.001). Hypoautofluorescent changes on fundus autofluorescence were present in 25 of 55 patients (45.5%) at baseline and in 23 of 51 patients (45.1%) at the final visit ( P = 0.480). CONCLUSION: In patients with chronic central serous chorioretinopathy who received a single, foveal, half-dose photodynamic therapy, a significant improvement in structure and function was seen at the final follow-up. None of the patients developed foveal atrophy.

A view from the clinic - Perspectives from Dutch patients and professionals on high myopia care.

PURPOSE: To understand and compare perspectives of patients and professionals on current ophthalmologic care for high myopia, and to identify challenges and future opportunities. METHODS: Self-reported data were collected through two online questionnaires. Patient perspective was obtained from highly myopic members of a patient organisation based in the Netherlands using a 17-item questionnaire consisting of open and multiple-choice questions regarding personal experience with myopia care. The ophthalmologist perspective was obtained from practising Dutch ophthalmologists with a 12-item questionnaire of multiple-choice questions on work-related demographics, myopia care in daily practice and need for improvement. The response rate for patients was 27% (n = 136/500) and for ophthalmologists, 24% (n = 169/716). RESULTS: Patients were highly concerned about personal progressive loss of vision (69%) and feared their psychological well-being (82%) in case this would happen. The quality of performance of care provided by ophthalmologists was rated as excellent or satisfactory by 64% of the patients. These ratings for multidisciplinary care and insurance reimbursement were as low as 28% and 18% respectively. The mean concern among ophthalmologists about the rise in high myopia was 6.9 (SEM 0.1) on a 10-point scale. Sixty-nine per cent of the ophthalmologists reported that asymptomatic myopic patients should not be examined regularly at outpatient clinics. Ophthalmologists urged the development of clinical guidelines (74%), but did report (95%) that they informed patients about risk factors and complications. This contrasted with the view of patients, of whom 42% were discontent with information provided by ophthalmologists. CONCLUSIONS: These questionnaires demonstrated that the current clinical care delivered to highly myopic patients is in need of improvement. The expected higher demand for myopia care in the near future requires preferred practice patterns, professionals specifically trained to manage myopic pathology, accurate and comprehensive information exchange and collaboration of in- and out-of-hospital professionals across the full eye care chain.

LONG-TERM TREATMENT OUTCOMES AFTER BEVACIZUMAB THERAPY FOR MACULAR NEOVASCULARIZATION IN WHITE PATIENTS WITH HIGH MYOPIA.

PURPOSE: To report long-term treatment outcomes of intravitreal bevacizumab in myopic macular neovascularization (MNV). METHODS: Retrospective analysis of longitudinal, clinical data of patients with high myopic MNV treated with intravitreal bevacizumab. One-hundred and seventeen eyes of 106 patients were followed from first injection up to 12 years. Outcome measures were best-corrected visual acuity change during follow-up and myopic MNV recurrence. RESULTS: Mean (±SD) baseline best-corrected visual acuity (0.56 ± 0.46 logMAR, 20/80) significantly improved after first treatment (0.33 ± 0.33, 20/50, P < 0.001). At 4 years (n = 86), best-corrected visual acuity was no longer significantly better than at baseline (0.55 ± 0.57, P = 0.30) and continued to deteriorate to 0.84 ± 0.76 (20/125) at 10 years (n = 27). Of the 27 eyes (23%) who reached 10 years of follow-up, 53% developed MNV-related chorioretinal atrophy. The cumulative incidence of recurrent myopic MNV was 34% at 2 years and 59% at 5 years. Best-corrected visual acuity decrease in eyes with or without recurrent MNV was similar ( P = 0.58). Patchy chorioretinal atrophy (hazard ratio 3.0, P = 0.02) and subfoveal MNVs (hazard ratio 2.5, P = 0.048) were significantly associated with recurrent MNV. CONCLUSION: This retrospective myopic MNV study revealed that visual improvement after intravitreal bevacizumab injections was not maintained over time. Macular neovascularization recurrences occurred frequently but did not alter the already poor visual prognosis.

Usher syndrome type IV: clinically and molecularly confirmed by novel ARSG variants.

Usher syndrome (USH) is an autosomal recessively inherited disease characterized by sensorineural hearing loss (SNHL) and retinitis pigmentosa (RP) with or without vestibular dysfunction. It is highly heterogeneous both clinically and genetically. Recently, variants in the arylsulfatase G (ARSG) gene have been reported to underlie USH type IV. This distinct type of USH is characterized by late-onset RP with predominantly pericentral and macular changes, and late onset SNHL without vestibular dysfunction. In this study, we describe the USH type IV phenotype in three unrelated subjects. We identified three novel pathogenic variants, two novel likely pathogenic variants, and one previously described pathogenic variant in ARSG. Functional experiments indicated a loss of sulfatase activity of the mutant proteins. Our findings confirm that ARSG variants cause the newly defined USH type IV and support the proposed extension of the phenotypic USH classification.

Long-term visual and anatomic outcomes of patients with peripapillary pachychoroid syndrome.

BACKGROUND/AIMS: To analyse the long-term anatomic and visual outcomes of patients with peripapillary pachychoroid syndrome (PPS), a recently described entity in the pachychoroid disease spectrum. METHODS: This study retrospectively included patients from several retina centres worldwide. Visual acuity (VA), retinal thickness and choroidal thickness at baseline, 6 months and final follow-up were assessed. Temporal trends in VA and anatomic characteristics were evaluated. Visual and anatomic outcomes in eyes that were observed versus those that were treated were analysed. RESULTS: Fifty-six eyes of 35 patients were included with mean follow-up of 27±17 months. Median VA was 20/36 at baseline and remained stable through follow-up (p=0.77). Retinal thickness significantly decreased subfoveally (p=0.012), 1.5 mm nasal to the fovea (p=0.002) and 3.0 mm nasal to the fovea (p=0.0035) corresponding to areas of increased thickening at baseline. Choroidal thickness significantly decreased subfoveally (p=0.0030) and 1.5 mm nasal to the fovea (p=0.0030). Forty-three eyes were treated with modalities including antivascular endothelial growth factor injection, photodynamic therapy, and others. VA remained stable in treated eyes over follow-up (p=0.67). An isolated peripapillary fluid pocket in the outer nuclear layer was characteristic of PPS. CONCLUSION: Patients with PPS experienced decreased retinal oedema and decreased choroidal thickening throughout the course of disease. While some patients experienced visual decline, the overall visual outcome was relatively favourable and independent of trends in retinal or choroidal thickening.

MYOPIC PRESENTATION OF CENTRAL SEROUS CHORIORETINOPATHY.

PURPOSE: To increase insight into the myopic presentation of central serous chorioretinopathy (CSC) by comparing a large group of myopic patients with CSC with reference groups with only one of the diagnoses. METHODS: Myopic patients with CSC (spherical equivalent ≤-3D, n = 46), emmetropic patients with CSC (spherical equivalent -0.5 to 0.5 D, n = 83), and myopic, non-CSC patients (n = 50) were included in this multicenter cross-sectional study. Disease characteristics and imaging parameters, such as subfoveal choroidal thickness and indocyanine green angiography patterns, were compared between cases and reference groups. RESULTS: In myopic patients with CSC, median subfoveal choroidal thickness (286 µm [IQR 226-372 µm]) was significantly thicker than subfoveal choroidal thickness in myopic, non-CSC patients (200 µm [IQR 152-228 µm], P < 0.001) but thinner than emmetropic patients with CSC (452 µm [IQR 342-538 µm], P < 0.001). They also had pachyvessels in 70% of the eyes comparable with emmetropic CSC (76%, P = 0.70). Choroidal hyperpermeability was frequently present on indocyanine green angiography in both myopic and emmetropic CSC eyes. Need for treatment, treatment success, and recurrence rate were not significantly different between CSC groups. CONCLUSION: Myopic CSC presents with similar imaging and clinical characteristics as emmetropic CSC, apart from their thinner choroids. Keeping in mind the structural changes of myopia, other imaging characteristics could aid the diagnostic process.

Central Serous Chorioretinopathy - an Overview. / Chorioretinopathia centralis serosa ­ ein Überblick.

Central serous chorioretinopathy (CSCR) is characterised by retinal serous detachment usually localised in the macular region. CSCR predominantly affects men between 30 and 50 years of age. Traditional classification differentiates between acute (duration shorter than 4 to 6 months) and chronic disease (duration longer than 4 to 6 months). The pathogenesis is multifactorial and current thinking assumes the presence of localised choroidal hyperpermeability with subsequent secondary changes in the retinal pigment epithelium (RPE). The symptoms of acute CSCR include central blurred vision, often with deterioration in visual acuity. Optical coherence tomography (OCT) reveals subretinal fluid (SRF) and/or single retinal pigment epithelial detachments. Fluorescein angiography (FA) usually shows a leaking point with absent or only minor RPE changes in the acute phase and indocyanine green angiography (ICG) highlights circumscribed areas of thickened and hyperpermeable choroid. Acute cases may show spontaneous resolution of SRF, but may also recur and/or become chronic. After the initial diagnosis, spontaneous remission is seen in about 70 to 80% of cases, with a recurrence rate of about 50%. Due to the favourable spontaneous course, it is recommended to wait for 4 to 6 months after the first symptoms manifest. Steroid therapy is considered as a major risk factor. Chronic cases are characterised by slow deterioration in visual acuity with reduced contrast and colour perception. There are extensive RPE changes, with secondary degenerative changes of the photoreceptors. The disease can by complicated by choroidal neovascularisation (CNV), especially in elderly patients. The literature lists a number of treatments: The leakage point (visible in the FA) can be treated by focal laser therapy, either micropulse laser or, if sufficiently distant from the fovea, by argon laser coagulation. Randomised trials in chronic CSCR demonstrated good outcomes with photodynamic therapy. With observation periods ranging from 3 to 6 months, several case series reports found improvement after systemic administration of mineralocorticoid receptor antagonists, carbonic anhydrase inhibitors or non-steroidal anti-inflammatory drugs. In the presence of secondary CNV, anti-VEGF treatment should be initiated. It is unclear whether the combination with PDT might be useful.

Prevalence and Severity of Diabetic Retinopathy in Patients with Macular Telangiectasia Type 2.

PURPOSE: To study the prevalence and severity of diabetic retinopathy (DR) in patients with macular telangiectasia type 2 (MacTel 2). DESIGN: Retrospective case series. PARTICIPANTS: Patients with a diagnosis of MacTel 2 treated at the Rotterdam Eye Hospital or Erasmus Medical Center between 2014 and 2018 were included. METHODS: The following information was retrieved from patient files: demographics, history of diabetes mellitus and hypertension, presence of DR, and severity of DR, that is, mild, moderate, severe, or proliferative. Presence of diabetic macular edema (DME) was assessed using OCT. MAIN OUTCOME MEASURES: Presence and severity of DR. RESULTS: Two hundred six eyes of 103 patients were included. At the onset of MacTel 2, the mean age was 61 years (standard deviation [SD], 9.8 years) and 64 (62%) were women. Mean follow-up was 71 months (SD, 60 months). Diabetes mellitus type 2 was present in 50 patients (49%) and hypertension was present in 47 patients (46%). Mild DR was present in 22 eyes (11%), of which 14 eyes (7%) showed signs at baseline and 8 eyes (4%) showed signs at a later time during follow-up. Ten eyes (5%) demonstrated remission of mild DR during follow-up. Both eyes (1%) in 1 patient progressed to moderate DR. Severe DR, proliferative DR, and DME did not occur. CONCLUSIONS: Although diabetes mellitus was highly prevalent among MacTel 2 patients, no patients showed severe or proliferative DR or DME. These findings suggest that MacTel 2 could have a protective effect on the progression of DR. We hypothesize that our results may be explained by the role of Müller cells in the development of MacTel 2 and DR, and therefore a link between both diseases warrants additional studies.

ANATOMICAL CHANGES ON SEQUENTIAL MULTIMODAL IMAGING IN PERIFOVEAL EXUDATIVE VASCULAR ANOMALOUS COMPLEX.

PURPOSE: To report a series of 21 patients with perifoveal exudative vascular anomalous complex (PEVAC) and to investigate the anatomical changes over time. METHODS: We conducted a retrospective study. Clinical data of consecutive patients, presenting at the Rotterdam Eye Hospital between 2014 and 2019, were analyzed. The data collected included best-corrected visual acuity, fundus photography, optical coherence tomography (OCT), OCT-angiography, fluorescence angiography, and indocyanine green angiography. RESULTS: We included 21 patients with a PEVAC lesion with a mean follow-up of 24.3 ± 13.8 months (range, 9-46 months). Patients with PEVAC were on average 75.3 ± 11.1 years (range, 53-90 years). The large perifoveal vascular aneurysmal abnormality was associated with small retinal hemorrhages in six patients and hard exudates in three patients. The PEVAC lesion was associated with intraretinal cystic spaces on OCT in 15 patients. Twelve of 21 patients showed no changes in cystic spaces on OCT during follow-up: 9 patients had stable cystic spaces and 3 patients had no cystic spaces. In contrast, in 9 of 21 patients, we observed changes in cystic spaces on OCT during follow-up. In two patients, cystic spaces appeared during follow-up, and in seven patients, there was a spontaneous resolution of cystic spaces. In three of these seven patients, the PEVAC lesion completely disappeared. Two patients, with stable intraretinal cystic spaces on OCT, were treated with intravitreal injections of anti-vascular endothelial growth factor without improvement. CONCLUSION: Perifoveal exudative vascular anomalous complex is an idiopathic perifoveal retinal vascular abnormality that is associated with intraretinal cystic spaces. These intraretinal cystic spaces associated with a PEVAC lesion, and even the PEVAC lesion itself, can have a spontaneous resolution over time.