Pathogenesis of Central Serous ChorioRetinopathy and the Link between Choroidal Hyperpermeability and RPE Pump Reversal.

PURPOSE: To describe 6 cases of acute central serous chorioretinopathy (CSCR) and the response to laser treatment, focusing on the underlying pathogenic mechanism. METHODS: Multimodal imaging from 6 eyes of 6 patients with acute and recurrent CSCR were reviewed including fundus autofluorescence (FAF), fluorescein angiography (FA), indocyanine green angiography (ICGA) and optical coherence tomography (OCT) at baseline and after laser therapy. RESULTS: In 3 out of the 6 cases with acute CSCR, the hyporeflective lucency sign was identified with cross sectional and en face OCT and co-localized with an intense active inkblot retinal pigment epithelium (RPE) leak on FA. The development of this sign was suggestive of active leakage into the subretinal space displacing overlying subretinal hyperreflective material (SHRM) and suggestive of a reversal of RPE pump function. All six cases were treated with focal laser to directly target the intense leakage points with remarkable resolution of the fluid due to destruction of the RPE cells mediating reversal of pump function. CONCLUSIONS: Unlike chronic CSCR in which degenerative changes of the RPE lead to oozing of fluid into the subretinal space, in acute forms of CSCR including bullous CSCR, there are focal leaks of the RPE that actively drive fluid into the subretinal space suggestive of RPE pump reversal. We propose that pachychoroid disease causes increased hydrostatic pressure and increased resistance to the RPE pump thereby triggering a reversal in pump function. Understanding this concept can have therapeutic implications.

Autoimmune retinopathy associated with systemic autoimmune disease: a case series.

OBJECTIVE: To describe various presentations of autoimmune retinopathy (AIR) associated with systemic autoimmune diseases. DESIGN: Case series. PATIENTS AND METHODS: Four patients with systemic autoimmune disorders and AIR are described in this report. The clinical and multimodal imaging characteristics, systemic work-up, genetic testing results, management, and course of disease are detailed. RESULTS: The multimodal retinal features of 4 cases of AIR including the findings of fundus autofluorescence, optical coherence tomography, and electrophysiology necessary to document progressive photoreceptor loss are described. Each case of AIR was associated with a complicated autoimmune disorder. Case 1 was associated with chronic inflammatory demyelinating polyneuropathy and showed marked improvement with systemic steroid and intravenous immunoglobulin therapy. Case 2 was associated with rheumatoid arthritis, and the AIR condition progressed despite systemic immune therapy. Case 3 was associated with Lambert-Eaton myasthenic syndrome, and AIR developed 6 years later and stabilized with systemic immune therapy. Case 4 was associated with necrobiotic xanthogranuloma followed by AIR and was managed by systemic immune therapy. CONCLUSIONS: AIR in association with these systemic conditions is rarely reported. Our cases highlight the gaps in our current understanding of the definition, systemic associations, pathogenesis, and management of AIR and the importance of multimodal imaging and a multidisciplinary approach in managing patients with suspected AIR.

Visual outcomes and complications of combined versus sequential pars plana vitrectomy and phacoemulsification for epiretinal membrane.

BACKGROUND: Symptomatic epiretinal membrane (ERM) often requires surgical intervention via pars plana vitrectomy (PPV), for which cataract development is a common complication. There is insufficient data on the visual outcomes and complications of combined phacovitrectomy (COMB) in comparison to sequential PPV with deferred cataract surgery (SEQ) for ERM. METHODS: A retrospective dataset analysis of 8 National Health Service ophthalmology departments. The main outcome measures were postoperative visual acuity (VA), operative complications, postoperative cystoid macular oedema (CMO) and recurrent ERM. RESULTS: We included 898 and 299 eyes in the COMB and SEQ groups, respectively. Both procedures resulted in significantly better VA across all follow-up intervals (24 weeks). The proportion of eyes with Snellen VA of at least 20/40 at 12-24 weeks was comparable in both groups (47.8% [COMB] vs. 54.7% [SEQ], p = 0.4456). More eyes in the COMB group experienced posterior capsular rupture (2.9% vs. 0%, p = 0.0009) and iatrogenic retinal trauma (2.4% vs. 0%, p = 0.0023). However, regression analysis revealed that combined surgery did not predict either complication. There were no significant differences in the rates of CMO (6.5% [COMB] vs. 9% [SEQ], p = 0.1522) and recurrent ERM (2.1% [COMB] vs. 3.3% [SEQ], p = 0.2758) between both groups. CONCLUSION: Both combined and sequential procedures are comparably effective and safe means for managing eyes with ERM.

Prevalence and Distribution of Macular Fluid with Central Retinal Artery Occlusion and Anterior Ischemic Optic Neuropathy.

Purpose: To examine the prevalence and distribution of fluid within a cohort of eyes with acute central retinal artery occlusion (CRAO) and non-arteritic anterior ischemic optic neuropathy (AION) using optical coherence tomography (OCT). Methods: A retrospective analysis of patient records and OCT imaging. Patients presenting with acute CRAO or AION who had available macular OCT imaging and no co-morbidities known to cause macular fluid were included in the analysis. Baseline characteristics, visual acuity (VA), and fluid presence and distribution among the retinal layers were recorded. Results: In the 16 eyes with acute CRAO, fluid was noted in 5 eyes (31%), which was mainly subretinal (3 eyes) or intraretinal located within the outer retinal layers (3 eyes). Only one eye had inner retinal cysts. Of the 11 eyes with acute AION, fluid was present in 8 eyes (73%). Subretinal fluid was noted in 4 eyes and extended to the foveal area in 3 of them, and outer retinal versus inner retinal cysts were noted in 6 versus 3 eyes, respectively. None of the eyes showed hard exudate deposition. In the small subset of eyes with CRAO and macular fluid that were followed-up, VA improved, while in eyes with AION, VA remained stable. Conclusion: Macular fluid on OCT is not an uncommon feature of acute CRAO and AION and is mainly distributed within the outer retinal layers or subretinal space. Fluid is an understudied feature of retinal and optic nerve head infarction and may have a role in predicting neuronal damage extent and visual outcome.

Retinal Ischemic Perivascular Lesions Are Associated With Myocardial Infarction in Patients With Coronary Artery Disease.

PURPOSE: To evaluate the association of retinal ischemic perivascular lesions (RIPLs) with myocardial infarction (MI) among patients diagnosed with coronary artery diseases (CAD). DESIGN: Retrospective cross-sectional study. METHODS: Consecutive patients (317 patients) with CAD who underwent macular spectral domain optical coherence tomography (SD-OCT) were captured. Patients with CAD who developed MI were compared to those without MI. SD-OCT were reviewed by 2 independent and masked graders for the presence of RIPLs. Medical records were reviewed. Multivariate logistic regression analysis was used to evaluate the relationship between RIPLs and MI including the following covariates age, gender, smoking status, hypertension, diabetes, dyslipidemia and body mass index. RESULTS: Of 317 patients with CAD for whom OCT scans were available to study, there were 54 (17%) with a history of MI. A higher prevalence of RIPLs was observed in the MI group compared to the non-MI group (59.3% vs 35.7%; P < .001). The presence of RIPLs was significantly associated with MI with an odds ratio of 3 (1.91-4.74; P < .001), after adjusting for age, gender, smoking status, hypertension, diabetes, dyslipidemia, and body mass index. CONCLUSIONS: The presence of RIPLs, detected with SD-OCT, is significantly associated with MI in patients with CAD. These findings underscore the potential clinical utility of incorporating RIPL evaluation in the medical management of CAD.

Cystoid macular edema as a complication of central retinal artery occlusion.

Purpose: To describe the development of cystoid macular edema (CME) as a complication of central retinal artery occlusion (CRAO) in 2 cases. Observations: The first patient was a 51-year-old female who presented with acute loss of vision in the left eye. Multimodal retinal imaging revealed a CRAO with a perfused cilioretinal artery. CME acutely developed one week after presentation. Cystoid spaces predominantly involved the outer nuclear layer (ONL) on optical coherence tomography (OCT) and completely resolved in two weeks. The second case was a 50-year-old man who presented with acute vision loss in the right eye for 3 weeks. Multimodal retinal imaging illustrated an acute CRAO of the right eye. Four weeks later, visual acuity spontaneously improved to 20/20 and was maintained at 20/20 for more than 2 years. After 28 months, the patient returned with a recurrent drop of vision in the right eye. Cross sectional and en face OCT revealed CME in the right eye without leakage on FA. Cystoid spaces predominantly involved the inner nuclear layer (INL) and resolved with intravitreal anti-VEGF injection combined with carbonic anhydrase inhibitor (CAI) and steroid topical drop therapy. Conclusions and Importance: CME can rarely complicate both the acute and chronic phase of CRAO. In the acute phase, cystoid spaces were transient and confined to the ONL on OCT. While in the chronic phase, cystoid spaces were confined to the INL on OCT and angiographically silent on FA. Further studies are needed to identify the incidence, underlying pathophysiology and visual prognosis of CME in cases of CRAO.

BILATERAL CHOROIDAL METASTASIS WITH CONCURRENT UNILATERAL RHEGMATOGENOUS RETINAL DETACHMENT IN A PATIENT WITH STAGE IV BREAST CANCER.

PURPOSE: To describe an unusual case of bilateral choroidal metastasis with simultaneous rhegmatogenous retinal detachment in the right eye. METHODS: Case Report. PATIENT: A 65-year-old woman with Stage IV breast cancer and gradual deterioration of vision in the right eye. RESULTS: Dilated fundus examination of the right eye revealed a large solitary choroidal mass with exudative retinal detachment and with an incidental discovery of a lower peripheral break with subretinal fluid, and the left eye revealed multiple choroidal masses. The diagnosis of bilateral CM was confirmed using optical coherence tomography. Pars plana vitrectomy with silicone oil injection was performed for the right eye followed by external beam radiotherapy to both choroids, which resulted in anatomical and visual success for 6 months. CONCLUSION: Prompt ophthalmologic evaluation is a must in patients with advanced breast or lung malignancy with recent blurring of vision. External beam radiotherapy is a safe, available, relatively cheap, and effective means for managing CM. Choroidal metastasis with a concurrent vision-threatening pathology requiring intraocular surgery represents a dilemma, and the risks and benefits should be weighed carefully with proper counseling of the patient.

Bacillary layer detachment with malignant choroidal tumors: a case series.

PURPOSE: To study the incidence and characteristics of bacillary layer detachment (BALAD) occurring with the two most common choroidal malignancies, choroidal metastasis and choroidal melanoma. METHODS: A retrospective multicentric record analysis. Eyes with a diagnosis of choroidal melanoma or choroidal metastasis that had good-quality fundus photography and spectral domain optical coherence tomography (OCT) scans of the macular and tumor regions allowing for delineation of the retinal layers were included for analysis. Qualitative image evaluation was done by two independent graders for the presence, location, and OCT features of BALAD, as well as any associated intraretinal or subretinal fluid. Demographic and clinical data were also retrieved. RESULTS: Of the 11 eyes with choroidal metastasis and 7 eyes with choroidal melanoma that were included in the final analysis, 6 (54.5%) and 1 (14.3%) had BALAD, respectively. The BALAD co-localized with the subretinal fluid in all cases and with the intraretinal fluid in 1/3 cases (33.3%), was foveal in location in 3 eyes (42.9%), was overlying the tumor in 6 eyes (85.7%), and varied in number and size. Reflectivity within the BALAD was consistently higher than the vitreous and adjacent subretinal fluid, and discernable suspended hyperreflective particles were noted in 5 eyes (71.4%). CONCLUSION: BALAD is relatively common with choroidal metastasis. The OCT features described supplement our recognition of this new entity.

Clinical and multimodal imaging characteristics of eyes with Vogt-Koyanagi-Harada disease: An Egyptian experience.

BACKGROUND: Vogt-Koyanagi-Harada (VKH) disease is a vision-threatening inflammatory disorder that is challenging in diagnosis and management. METHODS: Retrospective, record-based analysis of 54 eyes belonging to 27 adult patients that fulfilled the revised diagnostic criteria for VKH between January 2018 and January 2021. Demographic, clinical, and imaging data on presentation and during follow-up visits were collected for each patient. Available imaging studies included B-scan ultrasonography (B-scan US), spectral domain optical coherence tomography (OCT), fundus fluorescein angiography (FFA), and OCT angiography (OCT-A). RESULTS: The female-to-male ratio was 2.38:1. Nineteen patients (70.37%) presented during an initial attack, while eight patients (29.63%) presented during recurrence. The most commonly presenting sign in the posterior segment was exudative retinal detachment (44 eyes, 81.48%). B-scan US was utilized in 4 eyes (7.41%), OCT was utilized in 48 eyes (88.89%) with the most common finding being subretinal fluid (43 eyes, 89.58%), FFA was performed in 39 eyes (72.22%) with the most common finding being punctate hyperfluorescence and late dye pooling (33 eyes, 84.62%), and OCT-A was performed in 30 eyes (55.56%), in which choriocapillaris flow deficit that correlated with disease activity was detectable in 25 eyes (83.33%). Improved visual acuity was noted in 85% of the eyes that were followed up. CONCLUSION: Early diagnosis and treatment of VKH result in favorable visual outcome. Multimodal imaging, with the recent addition of OCT-A, provides complementary data that could serve in diagnosis and monitoring.

Visual outcomes and postoperative complications of eyes with dropped lens fragments during cataract surgery: multicenter database study.

PURPOSE: To analyze the visual outcomes and postoperative complications of phacoemulsification cataract surgery in eyes with posterior capsule rupture (PCR) and dropped lens fragments (DLFs) in comparison with a reference group with uneventful surgery. SETTING: 8 UK National Health Service departments. DESIGN: Retrospective comparative nonrandomized study. METHODS: Demographic, medical history, and ocular examination data were automatically extracted from the electronic records. The main outcome variables were postoperative visual acuity (VA), and the development of postoperative cystoid macular edema (CME) as well as rhegmatogenous retinal detachment (RRD) and epiretinal membrane (ERM) requiring surgery. RESULTS: The analysis included 175 589 eyes in the reference group, 2751 eyes in the PCR group, and 519 eyes in the DLF group. During all postoperative intervals, the mean VA in the DLF and PCR groups was significantly worse than the reference group ( P < .001). On multivariate analysis, the odds of having a VA ≤0.3 logMAR at 4 to 12 weeks postoperatively among eyes with DLF and PCR were 88% and 73% lower than the reference group ( P < .001). More eyes developed CME in the DLF and PCR groups ( P < .001). The odds of requiring RRD and ERM surgery were 3.6 and 2.1 times higher in the DLF group, and 1.8 and 1.3 times higher in the PCR group, respectively, as compared with the reference group. CONCLUSIONS: Eyes undergoing phacoemulsification complicated by PCR, and more so with DLF, have worse visual outcomes and higher chances of CME, ERM, and RRD when compared with uneventful surgery.

Bilateral macular retinitis in patients with presumed rift valley fever from Sudan: A case series.

BACKGROUND: Epidemic retinitis occurs seasonally following febrile infections in endemic regions. Rift valley fever (RVF) is endemic to Sudan, with a recent outbreak starting in 2019. METHODS: Retrospective case series of 3 patients travelling from Sudan with post-febrile retinitis, and with a history and clinical picture suggestive of RVF retinitis. RESULTS: The three patients were adult males with underlying medical conditions and underwent fundus fluorescein angiography that confirmed bilateral retinitis and occlusive vasculitis involving the posterior pole. Optical coherence tomography showed distortion of the macular layers. Case 2 presented 1 month following febrile illness and had retinal thinning, with optical coherence tomography angiography showing marked reduction in vessel density. CONCLUSION: We present multimodal imaging data of three cases with presumed RVF retinitis from a recent outbreak in Sudan. The unavailability of standardized methods of testing for RVF, as is the case for most epidemic retinitis-causing pathogens, makes the diagnosis challenging.

Optical coherence tomography and electrophysiological analysis of proptotic eyes due to thyroid-associated ophthalmopathy.

PURPOSE: To study the degree of morphological and functional changes in thyroid-associated ophthalmology (TAO) patients using optical coherence tomography and electrophysiological studies and investigate their clinical correlation. METHODS: A cross-sectional study including 40 patients with TAO and a control group with age- and sex-matched healthy subjects. All subjects underwent a complete ophthalmological examination, proptosis evaluation, spectral domain optical coherence tomography, and electrophysiological tests (pattern and multifocal electroretinograms, and visual evoked potentials). RESULTS: On multiple regression analysis, the degree of proptosis and P50 amplitude were the most important predictors of central foveal thickness (CFT) (ß = - 0.56 and 0.39 and p = 0.03 and 0.01, respectively); while duration of the disease, and clinical activity score (CAS) were the most important predictors of average RNFL thickness (ß = 0.67 and - 0.81 and p = 0.004 and < 0.001, respectively). CONCLUSION: In the absence of fundus changes, macular thinning along with functional alterations noticed by pattern and multifocal electroretinogram could indicate presence of subclinical retinopathy in TAO cases.

Visual outcomes and complications of combined vs sequential cataract surgery and pars plana vitrectomy: multicenter database study.

PURPOSE: To compare the visual outcomes and rates of intraoperative complications in eyes that underwent combined cataract extraction (CE) and pars plana vitrectomy (combined group) with those that underwent sequential surgery (sequential group). SETTING: Multicenter study. DESIGN: Retrospective chart review. METHODS: CE data set pooled from 8 UK sites between 2000 and 2015. The main outcome measures were the mean postoperative visual acuity (VA) and the rate of intraoperative complications in both groups. RESULTS: 2236 eyes in the combined group and 2270 eyes in the sequential group were included in this study. Mean preoperative VA was 1.0 logMAR in both groups. The mean logMAR postoperative VA was worse in the combined group than in the sequential group ( P < .0001) at all timepoints, however, the differences in visual improvement between both groups decreased with longer follow-up time: 1.0 ± 0.7 vs 0.6 ± 0.6, 0.7 ± 0.6 vs 0.4 ± 0.5, and 0.7 ± 0.6 vs 0.5 ± 0.5 at 0 to 4 weeks, 4 to 12 weeks, and 12 to 24 weeks, respectively. Proportions of eyes that gained >3 logMAR units were 49% in the combined group and 66.2% in the sequential group ( P < .0001). Logistic regression analysis showed that sequential surgery (odds ratio, 2.1) was a predictor for reaching 20/40 vision by 6 months. In the combined group, there was a statistically significantly higher rate of posterior capsular rupture. CONCLUSIONS: Postoperative visual gain was less in the combined group with a higher rate of posterior capsular rupture as compared with sequential phacovitrectomy. However, small differences in visual improvements between both groups by 6 months were observed.

Perfluorocarbon liquid-assisted vitreo-dissection in eyes with firmly adherent posterior hyaloid.

BACKGROUND: Induction of posterior vitreous detachment (PVD) is a critical step during pars plana vitrectomy. Multiple techniques and utilities have been proposed for assistance with this step with no consensus on the safest and most effective means, especially in eyes with firmly adherent posterior hyaloid. Viscodissection or the utilization of perfluorocarbon liquid (PFCL) can be used to dissect the posterior hyaloid and widely adherent epiretinal membranes. METHODS: A technique of PFCL dissection of the posterior hyaloid in eyes with abnormal adhesion of the posterior hyaloid. After core vitrectomy, breaking into the posterior hyaloid face is made via active aspiration and cutting or a sharp dissection. This is followed by active and slow injection of PFCL into the potential space between the posterior cortical vitreous and the neurosensory retina. A wave of PFCL propagates anteriorly causing "vitreo-dissection" of the peripheral cortical vitreous. RESULTS: The technique was effective and safe in 8 successive cases, 4 cases with vitreoretinal traction syndrome and 4 with diabetic tractional membranes. CONCLUSION: The technique can be considered in cases with abnormal firmly adherent posterior hyaloid when induction of PVD proves difficult.

Tailoring Screening Guidelines for Retinopathy of Prematurity in Egypt: An Exploratory Multicentric Study.

Background: Retinopathy of prematurity (ROP) is increasing in incidence in developing nations, including Egypt. Secondary prevention requires timely detection through the development of regional screening guidelines, which should be preceded by large-scale studies to characterize the population at risk. Methods: A prospective, multicentric exploratory study that included five large tertiary institutions in an urban Egyptian setting. All infants born with gestational age (GA) < 37 weeks and/or birth weight (BW) ≤ 2000 grams were screened. More mature and heavier infants with unstable clinical course were also included. The primary outcome measure was the rate of ROP and high-risk disease occurrence in relation to underlying risk factors. Results: Of the 768 eyes (384 screened infants), 347 eyes (45.2%) had stage 1 or higher disease, and 43 eyes (5.6%) had high-risk disease. Eyes with stage 1 or higher ROP and treatment-requiring ROP had a mean (± SD) GA of 33.4 (± 2.6) weeks and 32.8 (± 3.2) weeks, and BW of 1842.3 (± 570.1) grams and 1747.6 ± (676.2) grams, respectively. Treatment-requiring eyes belonged to infants that had significantly lower GA and significantly higher prevalence of co-morbidities than non-treatment-requiring eyes. Conclusion: The incidence of ROP and high-risk disease in an urban Egyptian setting are similar to those in comparable settings elsewhere and locally. This exploratory study supports tailoring local screening criteria for ROP, and may aid the future development of national guidelines.

Persistent Subretinal Fluid Following Successful Rhegmatogenous Retinal Detachment Surgery.

PURPOSE: To provide an updated, critical summary of the literature on the topic of persistent subretinal fluid (PSF) following successful rhegmatogenous retinal detachment surgery. METHODS: Narrative literature review. RESULTS: PSF remains an insufficiently studied topic. Incidence rates vary significantly between reports, but pars plana vitrectomy seems associated with lower rates than buckle surgery. Multiple etiologies and risk factors have been proposed, none being conclusive. PSF gradually resolves in most cases which may be a lengthy process, often with no effect on potential final visual acuity. There is concern that some cases with PSF may sustain photoreceptor damage, retinal displacement, or retinal fold formation. There is no current evidence to support any treatment modality over observation in uncomplicated cases of PSF. CONCLUSION: Future large, well-controlled, prospective trials could help elucidate incidence rate, etiological factors, and sequelae of PSF, as well as the value of different interventions in its prevention and management.