SCREENING PRACTICES AND LATE DIAGNOSIS OF HYDROXYCHLOROQUINE RETINOPATHY IN ASIAN PATIENTS.

PURPOSE: To investigate the associations between screening practices and late diagnosis in Asian patients with hydroxychloroquine retinopathy. METHODS: In total, 92 Korean patients with hydroxychloroquine retinopathy were included and separated into late diagnosis and earlier diagnosis groups according to the retinopathy stage at the time of diagnosis. Details of screening practices regarding timing and modalities for baseline and annual monitoring examinations were compared between the two groups. Adherence to the current American Academy of Ophthalmology guidelines was compared between the two groups. RESULTS: Timing of baseline and initial monitoring examinations was appropriate as per the Academy of Ophthalmology guidelines in only 5.3% of patients with late diagnosis. There were significant differences in the proportions of patients receiving initial monitoring at 5 years of use and those receiving annual monitoring between the late and earlier diagnosis groups ( P = 0.003 and <0.001, respectively). The duration from the start date of hydroxychloroquine therapy to the first monitoring examination was significantly prolonged in the late diagnosis group ( P < 0.001). Multivariate logistic regression revealed significant association of the time duration with the first monitoring examination ( P = 0.042) and age ( P = 0.028) with late diagnosis. CONCLUSION: Results of this study suggest that poor adherence to the Academy of Ophthalmology guideline, particularly delayed initial monitoring, may be associated with late diagnosis of hydroxychloroquine retinopathy.

Use of OCT Retinal Thickness Deviation Map for Hydroxychloroquine Retinopathy Screening.

PURPOSE: To investigate the use of a retinal thickness deviation map generated from swept-source (SS) OCT images for hydroxychloroquine retinopathy screening. DESIGN: Retrospective cohort study. PARTICIPANTS: This study included 1192 Korean patients with a history of hydroxychloroquine treatment: 881 patients (1723 eyes) in the discovery set and 311 patients (591 eyes) in the validation set. Patients were screened for retinal toxicity using SS OCT, fundus autofluorescence, and standard automated perimetry. METHODS: According to the 2016 American Academy of Ophthalmology guidelines, hydroxychloroquine retinopathy was diagnosed by the presence of abnormalities on ≥1 objective structural tests alongside corresponding visual field defects. The 12 × 9-mm2 macular volume SS OCT scan was performed, and the retinal thickness deviation map was generated automatically using the built-in software. On this map, yellow (retinal thickness, <5% of the normative level) or red (<1% of the normative level) pixels were defined as abnormal. Abnormal findings were evaluated, and diagnostic criteria were developed based on the discovery set data; criteria were validated using the validation set data. MAIN OUTCOME MEASURES: The rate and patterns of abnormalities on the retinal thickness deviation map and sensitivity and specificity of the diagnostic criteria. RESULTS: The retinal thickness deviation map showed the following abnormal patterns in eyes with hydroxychloroquine retinopathy: pericentral (36.0%) or parafoveal (6.1%) ring, mixed-ring (34.2%), central island (13.2%), and whole macular thinning (10.5%). The criterion of ≥5 contiguous red pixels showing 1 of the 5 characteristic patterns in both eyes yielded the greatest diagnostic performance (sensitivity and specificity of 98.2% and 89.1% and of 100% and 87.5% in the discovery and validation set data, respectively). Moreover, the area of abnormal pixels on the map was correlated significantly with the mean deviation (P < 0.001) and pattern standard deviation (P < 0.001) on the Humphrey 30-2 test in eyes with hydroxychloroquine retinopathy. CONCLUSIONS: The retinal thickness deviation map may facilitate the objective evaluation of hydroxychloroquine retinopathy because it does not require subjective, morphologic evaluation of the outer retinal layers. The map has the potential to enhance hydroxychloroquine retinopathy screening when used in conjunction with conventional screening methods.

Long-Term Progression of Pericentral Hydroxychloroquine Retinopathy.

PURPOSE: To investigate the long-term progression of pericentral hydroxychloroquine retinopathy. DESIGN: Multicenter, retrospective cohort study. PARTICIPANTS: Eighty eyes (60 with pericentral pattern) of 41 Korean patients with hydroxychloroquine retinopathy followed up for 2 years or more after drug cessation. METHODS: Patients were screened for hydroxychloroquine retinopathy using spectral-domain or swept-source OCT, fundus autofluorescence (FAF), and Humphrey visual field (VF) tests. Follow-up was divided into short-term (≤2 years) and subsequent periods, and progression was evaluated in each period and severity group. Retinopathy progression on OCT was defined as increased length of the ellipsoid zone defect, decreased distance from the fovea to the photoreceptor defects, or newly developed or enlarged retinal pigment epithelium defects. On FAF, progression was defined as an increase in the area of hyperautofluorescence or hypoautofluorescence. Functional progression was defined as a regression coefficient of less than 0 dB/year for mean deviation and more than 0 dB/year for pattern standard deviation, based on linear regression analysis of 3 or more VF tests. Structural and functional progression rates were calculated using the slopes of retinal thicknesses on the Early Treatment Diabetic Retinopathy Study grid and perimetric parameters over time, respectively. MAIN OUTCOME MEASURES: Structural and functional progression of retinopathy. RESULTS: Approximately one third of eyes with early pericentral retinopathy showed limited progression during the short-term period after drug cessation, but they subsequently showed stable or improved photoreceptors. Most eyes with moderate pericentral retinopathy showed continuous progression, particularly when converted to the severe stage. Severe eyes showed progressive damage throughout the follow-up period. In all severity groups, the rates of retinal thinning decreased over time. In eyes with pericentral retinopathy showing progression, circumferential enlargement of retinal damage was prominent in earlier stages, whereas centripetal enlargement of the ring-shaped lesion was noted in advanced stages. Functional progression, noted in 58.7% of the pericentral eyes, corresponded with structural progression. CONCLUSIONS: Pericentral hydroxychloroquine retinopathy showed severity-dependent progression. Moderate pericentral retinopathy usually progressed, but centripetal progression threatening the fovea was remarkable mostly in severe retinopathy. Our results suggest that early detection of retinopathy may minimize the risk of progression to foveal involvement in pericentral retinopathy.

RECURRENCE OF IDIOPATHIC EPIRETINAL MEMBRANE AND ITS PREDISPOSING FACTORS: An Optical Coherence Tomography Study.

PURPOSE: To evaluate the incidence and clinical course of recurrent epiretinal membrane (ERM) after ERM surgery and to identify predisposing optical coherence tomography (OCT) findings for the recurrence. METHODS: Postoperative ERM recurrence, defined as reappearance of the membrane after its removal or regrowth of the remnant membrane, was investigated in 301 eyes with idiopathic ERM followed up for more than 6 months after macular surgery by fundus photographs and spectral-domain OCT. The incidences of recurrent ERM and its associated clinical characteristics were assessed. Preceding OCT findings in the area subsequently showing recurrent ERM were evaluated at early postoperative periods. RESULTS: Among the 301 eyes that underwent ERM peeling, 119 (39.5%) and 86 (28.6%) showed ERM recurrence on OCT images and biomicroscopic examination/fundus photographs, respectively, during the follow-up period (average: 18.1 months). Neighboring remnant membrane and hyperreflective dots on the retinal surface at 1 week after the surgery and postoperative inner retinal wrinkling persisting for ≥1 month were predisposing OCT findings for ERM recurrence, with an odds ratio of 6.48 (95% confidence interval, 3.51-12.0), 3.48 (95% confidence interval, 1.81-6.70), and 6.11 (95% confidence interval, 3.30-11.3), respectively (all P < 0.001). CONCLUSION: Incidence of ERM recurrence varies depending on the definition used for the recurrence. Optical coherence tomography examination may be useful for the prediction and sensitive detection of recurrent ERM.

TOXIC EFFECTS OF HYDROXYCHLOROQUINE ON THE CHOROID: Evidence From Multimodal Imaging.

PURPOSE: To investigate the choroidal changes that occur in hydroxychloroquine (HCQ) retinopathy using multimodal imaging including fluorescein angiography, indocyanine green angiography, and optical coherence tomography (OCT) angiography and to correlate these changes with retinal findings obtained using OCT and fundus autofluorescence. METHODS: In 20 patients (n = 40 eyes) with systemic lupus erythematosus or rheumatoid arthritis diagnosed to have HCQ retinopathy, imaging modalities including swept-source OCT, fundus autofluorescence, fluorescein angiography, indocyanine green angiography, and OCT angiography were used to evaluate retinal and choroidal changes associated with retinopathy. The assessments included specific findings such as presence of hyperfluorescent or hypofluorescent lesions on angiography and signal void zones on OCT angiography, their frequencies, and the correlations among the retinal and choroidal findings. These findings were also correlated with the severity of retinopathy. Retinopathy progression was defined using fundus autofluorescence and OCT and correlated with the retinal/choroidal findings. RESULTS: Fluorescein angiography demonstrated a hyperfluorescent area, which reflects a defective retinal pigment epithelium, with multiple tiny dark spots within the area. Indocyanine green angiography showed a hypofluorescent area with dark spots, which was matched to the hypoautofluorescent area on fundus autofluorescence. Although there were no specific morphologic abnormalities in the choroid layers using en face choroidal imaging, OCT angiography demonstrated signal void areas on the choriocapillaris in the areas of the retinal pigment epithelium defect. Even after cessation of HCQ, there was progression of retinopathy in eyes with choroidal involvement, particularly on the area of choroidal findings. CONCLUSION: Multimodal imaging demonstrates choriocapillaris degeneration in eyes with HCQ retinopathy, particularly those with severe retinopathy. The choroidal change was associated with outer retinal toxicity of HCQ.

VISUALIZATION OF THE MACULA IN GAS-FILLED EYES: Spectral Domain Optical Coherence Tomography Versus Swept-Source Optical Coherence Tomography.

PURPOSE: To compare the visualization of the macula between spectral domain optical coherence tomography (SD-OCT) and swept-source OCT (SS-OCT) in gas-filled eyes. METHODS: Sixty-one patients with common indications of gas tamponade, including 27 with macular holes and 34 with rhegmatogenous retinal detachment, who were treated with vitrectomy and gas tamponade were imaged using both SD-OCT (3D-OCT 2000, Topcon, Tokyo, Japan) and SS-OCT (DRI-OCT, Topcon) at 1, 3, and 7 days after the surgery. Good visualization of the macular area was defined as 1) visible foveal contour and discriminable status of hole (open or closed) for macular hole and 2) visible macular contour and distinguishable status for the presence of subretinal fluid for rhegmatogenous retinal detachment. The frequencies of good visualization were compared between SD-OCT and SS-OCT in each case. RESULTS: Among 27 eyes with macular hole, good visualization at Day 1 was observed in 16 (59.3%) with SD-OCT and 24 (88.9%) with SS-OCT using a line scan protocol. For the cases with rhegmatogenous retinal detachment, good visualization at the day was noted in 12 (35.3%) and 25 (73.5%) eyes with SD-OCT and SS-OCT, respectively. For each scan protocol, the differences in good visualization of the macula between SD-OCT and SS-OCT were statistically significant for macular hole and rhegmatogenous retinal detachment (all P < 0.05). There were no significant differences between line and volume scan protocols, although good visualization was more frequently noted using the line scan protocol. CONCLUSION: In gas-filled eyes, SS-OCT performed significantly better than SD-OCT to visualize the macula. Using SS-OCT may lead to better decisions on further treatment during the early postoperative period, especially about postoperative positioning.

Intravitreal dexamethasone implant therapy for the treatment of cystoid macular Oedema due to hydroxychloroquine retinopathy: a case report and literature review.

BACKGROUND: Cystoid macular oedema (CMO) is an uncommon complication associated with hydroxychloroquine (HCQ) retinopathy threatening central vision. We report a patient with HCQ retinopathy and CMO, for which an intravitreal dexamethasone implant was used, which led to complete resolution of oedema. CASE PRESENTATION: A 57-year-old woman with systemic lupus erythematosus (SLE) complaining of blurred vision in both eyes was diagnosed with bilateral HCQ retinopathy and CMO based on characteristic photoreceptor defects and cystoid spaces on optical coherence tomography, hypo-autofluorescence on fundus autofluorescence, and corresponding visual field defects. After treatment with systemic acetazolamide and topical dorzolamide, CMO showed partial resolution in the right eye. Owing to worsening renal function, an intravitreal dexamethasone implant was placed in the right eye, which resulted in resolution of CMO and visual improvement from 20/50 to 20/30. CONCLUSION: Intravitreal dexamethasone implant may be effective for the treatment of CMO in HCQ retinopathy, particularly for the cases refractory to systemic or topical carbonic anhydrase inhibitors.

The effect of oral acetazolamide on cystoid macular edema in hydroxychloroquine retinopathy: a case report.

BACKGROUND: Hydroxychloroquine (HCQ) retinopathy can accompany other retinal complications such as cystoid macular edema (CME), which leads to central visual loss. We report a case of CME with HCQ retinopathy that improved with the use of oral acetazolamide, and discussed the possible mechanisms of CME in HCQ retinopathy using multimodal imaging modalities. CASE PRESENTATION: A 62-year-old patient with systemic lupus erythematosus (SLE) and HCQ retinopathy developed bilateral CME with visual decline. Fluorescein angiography (FA) showed fluorescein leakage in the macular and midperipheral area. After treatment with oral acetazolamide (250 mg/day) for one month, CME was completely resolved, best corrected visual acuity (BCVA) improved from 20/50 to 20/25, and FA examination showed decreased dye leakage in the macular and midperipheral areas. CONCLUSIONS: In cases of vision loss in HCQ retinopathy, it is important to consider not only progression of maculopathy, but also development of CME, which can be effectively treated with oral acetazolamide.

Choroidal thickness change following vitrectomy in idiopathic epiretinal membrane and macular hole.

PURPOSE: To investigate changes in choroidal thickness following vitrectomy in eyes with epiretinal membrane (ERM) and macular hole (MH) METHODS: Choroidal thicknesses at the fovea and at 1 and 3 mm superior, inferior, temporal, and nasal to the fovea were measured using enhanced-depth imaging optical coherence tomography (EDI-OCT) before and 1 week, 1, 3, 6, and 12 months after vitrectomy. In 95 eyes with ERM and 56 with MH, the thicknesses were compared between baseline and each postoperative visit. The postoperative thickness changes from baseline were compared between the ERM and MH groups. Clinical factors associated with choroidal thickness change were also evaluated. RESULTS: All choroidal thicknesses were significantly increased 1 week after vitrectomy compared to baseline in the ERM group (i.e. mean subfoveal choroidal thickness increased from 218 to 233 µm, P < 0.001). In the MH group, the increase was statistically significant only for inferior choroidal thicknesses. Such significant increase in choroidal thicknesses was not observed from 1 month postoperative in both groups. Postoperative SFCT change was significantly associated with combined cataract extraction (P = 0.026) and surgical indication (P = 0.010) at 1 week postoperative and with baseline SFCT at all postoperative visits (all P < 0.05). CONCLUSIONS: Choroidal thickness may temporarily increase following vitrectomy at the early postoperative period and subsequently decrease to the baseline value. Compared to the ERM group, the MH group showed location-dependent choroidal thickness change at early postoperative period, which might be explained by position-dependent tamponading effect of gas.

SUBRETINAL FIBROSIS AFTER ANTIVASCULAR ENDOTHELIAL GROWTH FACTOR THERAPY IN EYES WITH MYOPIC CHOROIDAL NEOVASCULARIZATION.

PURPOSE: To assess the incidence and risk factors of subretinal fibrosis and their impact on visual outcome in eyes with myopic CNV. METHODS: Medical records of 72 eyes treated with antivascular endothelial growth factor (anti-VEGF) therapy in a pro re nata regimen for myopic CNV that followed up for more than 1 year were retrospectively reviewed. The presence of subretinal fibrosis after anti-VEGF therapy was determined using both fundus photographs and optical coherence tomography. The incidence and risk factors of subretinal fibrosis were evaluated, and best-corrected visual acuity was compared between the eyes with and without subretinal fibrosis. RESULTS: The incidences of subretinal fibrosis during the 1-year and whole follow-up period were 31.9% and 36.1%, respectively. Occurrence of subretinal fibrosis was associated with frequent CNV recurrence (P = 0.005) and poor baseline best-corrected visual acuity (P = 0.044) in a Cox proportional hazard model. Anatomically, the eyes with subretinal fibrosis showed more frequent progression of chorioretinal atrophy (95.7% vs. 71.4%, P = 0.027) and less frequent photoreceptor recovery (17.4% vs. 65.3%, P < 0.001) after anti-VEGF therapy. Occurrence of subretinal fibrosis was associated with poor vision at baseline (P = 0.011) and the final visit (P = 0.008) when compared with the findings in the nonoccurrence group. CONCLUSION: Development of subretinal fibrosis after anti-VEGF therapy was common in eyes with myopic CNV and was associated with CNV recurrence. Myopic eyes with subretinal fibrosis show progressive chorioretinal atrophy and poor visual outcome.

Intravitreal dexamethasone implants for the treatment of refractory scleritis combined with uveitis in adult-onset Still's disease: a case report.

BACKGROUND: Adult-onset Still's disease is a systemic inflammatory disease which presents with uveitis and scleritis in the eye. Intravitreal dexamethasone implants are used for the treatment of refractory uveitis. CASE PRESENTATION: A 19-year-old woman diagnosed to have adult-onset Still's disease for fevers, joint pain, and a salmon-colored bumpy rash presented with scleritis and uveitis in the left eye. Topical and systemic steroids with oral methotrexate failed to control the inflammation. We performed intravitreal injections of dexamethasone implants for side effects of steroid and refractory ocular inflammation. The therapy resulted in improvements in the patient's uveitis with reductions in scleral vessel engorgement and redness. There was no recurrence of uveitis or scleritis during 4 months following treatment. CONCLUSIONS: Intravitreal injections of dexamethasone implants may result in clinical improvements of refractory scleritis combined with uveitis.

Neonatal systemic inflammation in rats alters retinal vessel development and simulates pathologic features of retinopathy of prematurity.

BACKGROUND: Alteration of retinal angiogenesis during development leads to retinopathy of prematurity (ROP) in preterm infants, which is a leading cause of visual impairment in children. A number of clinical studies have reported higher rates of ROP in infants who had perinatal infections or inflammation, suggesting that exposure of the developing retina to inflammation may disturb retinal vessel development. Thus, we investigated the effects of systemic inflammation on retinal vessel development and retinal inflammation in neonatal rats. METHODS: To induce systemic inflammation, we intraperitoneally injected 100 µl lipopolysaccharide (LPS, 0.25 mg/ml) or the same volume of normal saline in rat pups on postnatal days 1, 3, and 5. The retinas were extracted on postnatal days 7 and 14, and subjected to assays for retinal vessels, inflammatory cells and molecules, and apoptosis. RESULTS: We found that intraperitoneal injection of LPS impaired retinal vessel development by decreasing vessel extension, reducing capillary density, and inducing localized overgrowth of abnormal retinal vessels and dilated peripheral vascular ridge, all of which are characteristic findings of ROP. Also, a large number of CD11c+ inflammatory cells and astrocytes were localized in the lesion of abnormal vessels. Further analysis revealed that the number of major histocompatibility complex (MHC) class IIloCD68loCD11bloCD11chi cells in the retina was higher in LPS-treated rats compared to controls. Similarly, the levels of TNF-α, IL-1ß, and IL-12a were increased in LPS-treated retina. Also, apoptosis was increased in the inner retinal layer where retinal vessels are located. CONCLUSIONS: Our data demonstrate that systemic LPS-induced inflammation elicits retinal inflammation and impairs retinal angiogenesis in neonatal rats, implicating perinatal inflammation in the pathogenesis of ROP.

Photoreceptor change and visual outcome after idiopathic epiretinal membrane removal with or without additional internal limiting membrane peeling.

PURPOSE: To compare the postoperative photoreceptor status and visual outcome after epiretinal membrane removal with or without additional internal limiting membrane (ILM) peeling. METHODS: Medical records of 40 eyes from 37 patients undergoing epiretinal membrane removal with residual ILM peeling (additional ILM peeling group) and 69 eyes from 65 patients undergoing epiretinal membrane removal without additional ILM peeling (no additional peeling group) were reviewed. The length of defects in cone outer segment tips, inner segment/outer segment junction, and external limiting membrane line were measured using spectral domain optical coherence tomography images of the fovea before and at 1, 3, 6, and 12 months after the surgery. RESULTS: Cone outer segment tips and inner segment/outer segment junction line defects were most severe at postoperative 1 month and gradually restored at 12 months postoperatively. The cone outer segment tips line defect in the additional ILM peeling group was significantly greater than that in the no additional peeling group at postoperative 1 month (P = 0.006), and best-corrected visual acuity was significantly worse in the former group at the same month (P = 0.001). There was no significant difference in the defect size and best-corrected visual acuity at subsequent visits and recurrence rates between the two groups. CONCLUSION: Patients who received epiretinal membrane surgery without additional ILM peeling showed better visual and anatomical outcome than those with additional ILM peeling at postoperative 1 month. However, surgical outcomes were comparable between the two groups, thereafter. In terms of visual outcome and photoreceptor integrity, additional ILM peeling may not be an essential procedure.

Acquired pseudoduplication of the optic disc in pathologic myopia.

PURPOSE: To elucidate the mechanism of acquired pseudoduplication of the optic disc and its association with pathologic myopia. METHODS: The prevalence of pseudoduplication of the optic disc was estimated by reviewing 128 consecutive patients diagnosed as having pathologic myopia between January 2010 and December 2012. The pseudodisc was investigated at the scleral level using enhanced depth imaging spectral-domain optical coherence tomography to elucidate pathologic changes. Fluorescein angiography or indocyanine green angiography was performed to identify the vessel origin. RESULTS: Among 128 patients with pathologic myopia, 3 patients (2.3%) showed pseudoduplication of the optic disc. Enhanced depth imaging optical coherence tomography showed chorioretinal atrophy and focal scleral excavation in the area showing the pseudodisc. The round scleral excavation gave the underlying visible peribulbar tissue a pinkish appearance, which could be mistaken as the optic disc. Fluorescein angiography or indocyanine green angiography showed that the vessel within the pseudodisc was the short posterior ciliary artery. CONCLUSIONS: Although not a common presentation, scleral excavation associated with pathologic myopia accompanied by a ciliary artery penetrating the excavation's center could make a lesion mimicking pseudoduplication of the optic disc.

Risk of Post-COVID-19 Uveitis and Risk Modification by Vaccination: A Nationwide Retrospective Cohort Study.

This study aimed to evaluate the risk of uveitis, one of the most common ocular manifestations of COVID-19, in individuals with a history of uveitis and COVID-19 infection while discriminating the effects of COVID-19 infection and vaccinations. We analyzed nationwide data from 235,228 individuals with a history of uveitis prior to COVID-19 infection and evaluated incidences and hazard ratios (HRs) of post-COVID-19 uveitis for different post-infection periods, including early- (within 30 days) and delayed-onset ones. The cumulative incidences of post-infection uveitis at 3, 6, and 12 months were calculated as 8.5%, 11.8%, and 14.0%, respectively. The HR of post-COVID-19 uveitis was 1.21 (95% confidence interval [CI]: 1.07-1.37) and was particularly higher in the early-onset period (1.42, 95% CI: 1.24-1.61). Vaccinated individuals showed a modestly elevated risk of uveitis relative to pre-infection, while unvaccinated ones exhibited substantially higher risks in the early-onset period: the HR of post-infection uveitis before vaccination was 3.61 (95% CI: 1.35-9.66), whereas after vaccination, it was 1.21 (95% CI: 1.05-1.39). COVID-19 infection was associated with a higher risk of uveitis, which was mitigated by vaccination. Vigilance in the monitoring of uveitis is warranted for recently COVID-19-infected individuals with a history of uveitis, particularly unvaccinated individuals.

Clinical Applications and Future Directions of Smartphone Fundus Imaging.

The advent of smartphone fundus imaging technology has marked a significant evolution in the field of ophthalmology, offering a novel approach to the diagnosis and management of retinopathy. This review provides an overview of smartphone fundus imaging, including clinical applications, advantages, limitations, clinical applications, and future directions. The traditional fundus imaging techniques are limited by their cost, portability, and accessibility, particularly in resource-limited settings. Smartphone fundus imaging emerges as a cost-effective, portable, and accessible alternative. This technology facilitates the early detection and monitoring of various retinal pathologies, including diabetic retinopathy, age-related macular degeneration, and retinal vascular disorders, thereby democratizing access to essential diagnostic services. Despite its advantages, smartphone fundus imaging faces challenges in image quality, standardization, regulatory considerations, and medicolegal issues. By addressing these limitations, this review highlights the areas for future research and development to fully harness the potential of smartphone fundus imaging in enhancing patient care and visual outcomes. The integration of this technology into telemedicine is also discussed, underscoring its role in facilitating remote patient care and collaborative care among physicians. Through this review, we aim to contribute to the understanding and advancement of smartphone fundus imaging as a valuable tool in ophthalmic practice, paving the way for its broader adoption and integration into medical diagnostics.

Atypical Presentations of Hydroxychloroquine Retinopathy: A Case Series Study.

Background/Objective: Hydroxychloroquine retinopathy, traditionally characterized by parafoveal or pericentral outer retinal damage, is explored for atypical presentations in Asian patients. This challenges conventional beliefs regarding onset, retinopathy pattern, and associated visual field defects. Methods: Ninety-five patients diagnosed with hydroxychloroquine retinopathy at Hanyang University Hospital underwent screening from January 2010 to December 2023. Swept-source optical coherence tomography (SS-OCT), ultra-widefield fundus autofluorescence (UWF-FAF), and automated visual fields (VF) were employed for detailed structural and functional evaluations. Multifocal electroretinography was performed in selected cases requiring additional objective evidence of retinal toxicity. Results: Among 95 patients, 14 (14.7%) exhibited atypical presentations, including very early onset (n = 1), (far) peripheral-dominant damages (n = 4), perivascular involvement (n = 1), bitemporal hemianopsia due to nasal extensive lesions (n = 1), unilateral involvement (n = 2), and asymmetric involvement in retinopathy pattern or severity between the eyes (n = 7). These findings underscore the importance of utilizing expanded imaging techniques, such as ultra-widefield FAF imaging, to identify atypical presentations of retinal involvement. Conclusions: Screening physicians should consider these atypical presentations to ensure timely diagnosis and appropriate management in patients undergoing hydroxychloroquine treatment.

COVID-19 Vaccine-Associated Uveitis in Patients With a History of Uveitis.

Importance: Understanding the potential risk of uveitis recurrence after COVID-19 vaccination in individuals with a history of uveitis is crucial for vaccination strategies and clinical monitoring. Objective: To investigate the risk of uveitis recurrence after COVID-19 vaccination in a cohort of individuals with a history of uveitis. Design, Setting, and Participants: This retrospective population-based cohort study included individuals diagnosed with uveitis between January 1, 2015, and February 25, 2021, in South Korea. After excluding individuals without COVID-19 vaccination or with SARS-CoV-2 infection, individuals with a history of uveitis who had received at least 1 dose of a messenger RNA (BNT162b2 [Pfizer-BioNTech] or mRNA-1273 [Moderna]) or adenovirus vector-based (ChAdOx1 [AstraZeneca] or Ad26.COV2.S [Janssen]) COVID-19 vaccine were included. Data were analyzed from February 26, 2021, to December 31, 2022. Exposure: Demographic and clinical data, along with vaccination details, were retrieved from the Korean National Health Insurance Service and Korea Disease Control and Prevention Agency databases. Main Outcomes and Measures: Outcomes of interest were incidence and risk of postvaccination uveitis in association with different COVID-19 vaccines and periods before and after COVID-19 vaccination. Uveitis was categorized by onset (early, within 30 days, or delayed) and type (anterior or nonanterior). Hazard ratios (HRs) with 95% CIs were calculated to evaluate the risk of uveitis following COVID-19 vaccination, stratified according to vaccine type and vaccination period. Results: Of 543 737 individuals with history of uveitis, 473 934 individuals (mean [SD] age, 58.9 [17.4] years; 243 127 [51.3] female) had documented COVID-19 vaccination and were included in analysis. The cumulative incidence of postvaccination uveitis was 8.6% at 3 months, 12.5% at 6 months, and 16.8% at 1 year, predominantly of the anterior type. Variations in the risk of postvaccination uveitis were observed across different vaccines and intervaccination periods. The risk of early postvaccination uveitis was increased for individuals receiving the BNT162b2 (HR, 1.68; 95% CI, 1.52-1.86), mRNA-1273 (HR, 1.51; 95% CI, 1.21-1.89), ChAdOx1 (HR, 1.60; 95% CI, 1.43-1.79), and Ad26.COV2.S (HR, 2.07; 95% CI, 1.40-3.07) vaccines. The risk of uveitis was higher particularly between the first and second vaccination doses (HR, 1.64; 95% CI, 1.55-1.73). Conclusions and Relevance: These findings suggest that there was an elevated risk of uveitis following COVID-19 vaccination, with the vaccine type and period mediating this risk. For individuals with a history of uveitis, clinicians should consider the potential risk of uveitis recurrence in vaccination strategies and clinical monitoring.

Screening practices and risk assessment for maculopathy in pentosan polysulfate users across different exposure levels.

In this population-based cohort study, we investigated screening practices for maculopathy and incidences of specific macular/retinal conditions in pentosan polysulfate (PPS) users and assessed the relationship between these outcomes and drug exposure levels. Using a health claims database that covers approximately 50 million Koreans, we identified 138,593 individuals who were prescribed PPS between 2010 and 2021. For the 133,762 PPS users who initiated therapy between 2012 and 2021, the cumulative PPS dose for each participant was evaluated, and based on their cumulative PPS dose, patients were categorized into the high-risk (≥ 500 g), low-risk (50-500 g), and minimal exposure (< 50 g) groups. We analyzed the performance and methods of these examination methods used between 2018 and 2021 and compared them among cumulative dose groups to determine whether high-risk users underwent maculopathy screening more frequently or appropriately. We assessed the cumulative incidence of overall macular degeneration and maculopathy excluding common macular diseases following PPS therapy initiation. Most PPS users (99.7%) received a cumulative PPS dose < 500 g and the high- and low-risk groups comprised 445 (0.3%) and 22,185 (16.6%) patients, respectively. During the study period, monitoring examinations were conducted in 52.6% and 49.4% of high- and low-risk patients, respectively, revealing no significant difference between the two groups (P = 0.156). No significant differences were observed in the annual percentages of patients receiving ophthalmic examinations between the high- and low-risk groups (all P > 0.05). The cumulative incidences of overall macular degeneration and maculopathy excluding common macular diseases in high-risk users were 19.3% and 9.0%, respectively, which were significantly different from those of low-risk users (both P < 0.001). Multivariate Cox regression analysis revealed significantly higher risks of maculopathy excluding common macular diseases in the low- (Hazard ratio [HR] of 1.55 [95% CI 1.13-2.12]) and high-risk groups (HR of 1.66 [95% CI 1.22-2.27]) compared to the minimal exposure group. Our findings suggest a need for increased emphasis on PPS maculopathy screening in high-risk patients, highlighting raising awareness regarding exposure-dependent risks and the establishment of screening guidelines.

Drug exposure and risk factors of maculopathy in tamoxifen users.

Tamoxifen, a pivotal therapy for hormone receptor-positive breast cancer, is known for its efficacy in reducing breast cancer recurrence and mortality. However, concerns about potential ocular complications, particularly maculopathy, have emerged. This study aims to investigate the risk and associated factors of diverse macular conditions in tamoxifen users, considering drug exposure, demographics, and systemic diseases. A nationwide cohort of tamoxifen users, comprised of 14,267 tamoxifen users, was analyzed using the health insurance review and assessment database in South Korea. Demographic and clinical characteristics were examined, and the cumulative incidence of macular diseases was stratified by age and cumulative tamoxifen dosage. We conducted logistic regression analysis to identify potential risk factors among clinical variables such as age, sex, indications for tamoxifen use, and systemic diseases associated with various macular conditions. Additionally, Cox proportional hazard models were used to determine the baseline clinical characteristics predictive of these macular conditions, with subsequent calculation of hazard ratios. Cumulative incidences of overall macular diseases, other maculopathy excluding common macular diseases, and macular edema were 26.4, 11.4, and 6.5%, respectively. The incidence of various macular conditions increased with age and the cumulative tamoxifen dose. Age, cumulative dose group, and liver diseases demonstrated significant associations with overall macular diseases and maculopathy excluding common macular diseases in multivariate logistic regression analyses (all P < 0.05). Furthermore, age emerged as significant predictive factors of maculopathy in Cox proportional hazard models. Tamoxifen-induced maculopathy poses a concern for prescribing physicians and ophthalmologists, and this study provides valuable insights into its risk and risk factors. This study may contribute to evidence-based guidelines for tamoxifen maculopathy screening, emphasizing the importance of considering age, cumulative dose, and liver diseases for recommendation on screening timing and frequency.