PURPOSE OF REVIEW:Management of diabetic tractional retinal detachments (TRDs) remains technically challenging. The purpose of this review is to provide an updated overview of current surgical techniques and important considerations when treating this condition. RECENT FINDINGS:Recent advances in imaging have provided critical insights into hyaloid status and tractional forces in diabetic TRDs, greatly aiding surgical planning. Updates in vitrectomy instruments have also greatly improved anatomic success rates. However, a comprehensive preoperative evaluation and plan remains key in optimizing visual outcomes for this difficult condition. SUMMARY:The surgical management of diabetic TRDs requires several key elements for successful outcomes. Timely intervention and presurgical planning are crucial, particularly in rapidly progressing cases. The use of advanced imaging and instruments enhances visualization and precision during surgery. Finally, a collaborative multispecialty approach with clear communication with patients about their prognosis is fundamental to effective care. These strategies collectively improve patient outcomes and promote visual recovery in those affected by diabetic TRDs.

Pentosan polysulfate sodium (PPS) maculopathy is a recently described condition of macular toxicity. We report the prevalence (15%), the clinical characteristics, and the multimodal imaging (MMI) features of PPS maculopathy based on a final prospective analysis of a cohort of 154 subjects and highlight the association with inflammatory bowel disease (IBD). In addition, we summarize the most recent and relevant literature on PPS maculopathy.This study and other recent analyses provide solid evidence of the toxic effects of PPS on the retina and choroid and refute the concept that the maculopathy is an association of the underlying disease, interstitial cystitis. Moreover, this study and others raise a major concern that PPS may also be toxic to the bowel. Vigilant surveillance of patients receiving this potentially harmful drug is critical.We review the recommended screening guidelines, the pathophysiology and MMI spectrum of disease, the differential diagnosis including MMI clues to an accurate diagnosis, and the progression of retinopathy that can take place despite discontinuation of the drug, emphasizing the critical importance of early detection of toxicity.

PurposeTo evaluate the association of retinal ischemic perivascular lesions (RIPLs) with myocardial infarction (MI) among patients diagnosed with coronary artery diseases (CAD).DesignRetrospective cross-sectional study.MethodsConsecutive 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 two 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.ResultsOf 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<0.001). Presence of RIPLs was significantly associated with MI with an odds ratio of 3 (1.91-4.74; p<0.001), after adjusting for age, gender, smoking status, hypertension, diabetes, dyslipidemia and body mass index.ConclusionThe 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.

PURPOSE:To describe the optical coherence tomography features of pachyvitelliform maculopathy (PVM), an acquired vitelliform lesion (AVL) associated with pachychoroid disease. METHODS:This study was a retrospective, multicentre, observational analysis.Medical records and multimodal imaging were reviewed in all patients with pachychoroid disease and AVL. Visual acuity, central choroidal thickness (CCT), AVL dimensions, total choroidal area, luminal choroidal area, stromal choroidal area and choroidal vascular index were measured in all eyes with PVM and compared with normal age-matched control eyes. RESULTS:Mean age of the PVM group (17 eyes of 17 patients) was 71.41 years. Average follow-up was 33.15 months. Baseline VA was 20/40 in the PVM group and declined to 20/100 (p=0.006). AVLs were all detected overlying pachyvessels with optical coherence tomography and were all hyperautofluorescent with fundus autofluorescent imaging. Mean CCT in the PVM group was significantly greater (352.35 µm) than the CCT in the control group (226.88 µm, p<0.001). Retinal pigment epithelium (RPE) disruption was present in 64.71% of eyes with PVM at baseline and 41.18% developed macular atrophy at the end of follow-up. CONCLUSIONS:PVM, defined by the presence of AVL associated with pachychoroid features, is a distinct novel entity of the pachychoroid disease spectrum. This study suggests a possible pathogenesis of RPE dysfunction secondary to a thick choroid, leading to accumulation of undigested photoreceptor outer segments and AVL. Clinicians should be aware of this common cause of vitelliform lesions and the poor visual prognosis due to the high risk of atrophy development.

In this case series of 10 eyes, the authors describe and analyze the clinical and multimodal imaging features of central bouquet hemorrhage. Purpose:To describe the clinical characteristics, multimodal imaging features, and anatomic basis of a distinctive pattern of deep retinal hemorrhages located in the central fovea, a presentation referred to as "central bouquet hemorrhage."Methods:Retrospective, observational, multicenter case series of eyes with central bouquet hemorrhage. Multimodal imaging features were reviewed and analyzed.Results:Ten eyes from 10 patients (4 women and 6 men), with a mean age of 55.6 +/- 21.7 years (range 25-84 years) were included. Underlying etiologies were neovascular age-related macular degeneration (40%), lacquer cracks in pathological myopia (30%), macular telangiectasia Type 2 (10%), proliferative diabetic retinopathy (10%), and ocular trauma associated with angioid streaks (10%). On ophthalmoscopy, all eyes with central bouquet hemorrhage displayed a deep retinal hemorrhage with round margins in the central fovea and associated with petaloid hemorrhages radiating in the surrounding Henle fiber layer. Cross-sectional optical coherence tomography showed a well-delineated round hyperreflective lesion involving the central foveal Henle fiber layer/outer nuclear layer in all cases. Accompanying hyperreflective hemorrhages tracking along the obliquely oriented Henle fiber layer were present in all eyes. Resolution occurred in all patients, either spontaneously (30%) or after treatment with intravitreal anti-vascular endothelial growth factor injections (70%), and was associated with partial visual acuity improvement (from 20/113 to 20/36).Conclusion:"Central bouquet hemorrhage" is a novel descriptive term describing a characteristic round pattern of intraretinal blood in the fovea associated with Henle fiber layer hemorrhage and encountered in a spectrum of macular disease.

Purpose:Distinguishing an iris melanoma from an iris nevus can be challenging as few clinical features other than documented growth are helpful in making the diagnosis. In this study, we compared the presenting intraocular pressure (IOP) and cup-to-disc ratio (CDR) between affected and unaffected eyes in patients with iris melanoma and iris nevus. Patients and methods:This was a single-institution retrospective case series of patients treated for iris melanoma and iris nevus from January 2013 to October 2022. Thirty-nine subjects with iris melanoma and forty age-matched patients with iris nevus were included. We analyzed the difference in IOP, CDR, and diagnosis of glaucoma between affected and unaffected eyes in patients with iris melanoma and control iris nevus cohort. Results:The average IOP for eyes with iris melanoma and iris nevus was 18.8±6.1 mmHg and 14.6±3.5 mmHg (P<0.001), respectively. The average CDR was 0.36±0.27 and 0.24±0.14 (P=0.02), respectively. The average IOP of the contralateral unaffected eye in iris melanoma patients was 16.3±3.5 mmHg, significantly less than the affected eye (P=0.03). The average CDR of the contralateral unaffected eye in iris melanoma was 0.25±0.15, which was trending towards being less than the affected eye (P=0.05). There was no difference in the average IOP (P=0.89) or average CDR (P=0.49) between the affected and unaffected eye in patients with iris nevus. Conclusion:We demonstrate that patients with iris melanoma are more likely to have greater IOP and CDR in the affected eye compared to the unaffected eye, and a diagnosis of unilateral glaucoma than eyes with iris nevus. Patients with iris melanoma had greater IOP asymmetry between the affected and unaffected eye. Therefore, IOP and CDR asymmetry may suggest a diagnosis of iris melanoma.

PURPOSE:To describe and study hyporeflective subretinal pigment epithelium (RPE) spaces in large drusen and drusenoid pigment epithelial detachment before collapse. METHOD:Retrospective longitudinal study enrolled patients with large and very large drusen caused by intermediate age-related macular degeneration. The following optical coherence tomography parameters were assessed: drusen size (maximum width and height), optical coherence tomography biomarkers of RPE atrophy, presence of intraretinal and subretinal fluid, acquired vitelliform lesion, and sub-RPE regions of hyporeflectivity within the pigment epithelial detachment compartment. RESULTS:Of the 50 eyes from 41 patients (mean age 77.1 ± 9 years, 78% women) with large and very large drusen, 16 eyes progressed to collapse. Eyes with sub-RPE hyporeflective spaces (n = 8 eyes, 50%) were associated with greater drusen width and height than those without sub-RPE hyporeflective spaces. At the collapse visit, eyes with sub-RPE hyporeflective spaces displayed poorer visual acuity and greater incomplete RPE and outer retinal atrophy and complete RPE and outer retinal atrophy length than eyes without sub-RPE hyporeflective spaces ( P = 0.004 and P = 0.04, respectively). CONCLUSION:Sub-RPE hyporeflective spaces are a novel optical coherence tomography finding of large and very large drusen that collapse to atrophy. Progressive RPE dysfunction and failure may lead to reduced drusenoid material formation and progressive degenerative hydration of the large drusen before collapse, but this awaits confirmation with histopathological analysis.

Purpose: To present 2 cases of acute total loss of vision after scleral buckle surgery for rhegmatogenous retinal detachment. Methods: A retrospective chart review of 2 cases and an analysis of the literature were performed. Results: An 18-year-old woman and a 67-year-old woman suffered complete loss of vision in their operative eye after primary scleral buckle surgery with encircling bands. Conclusions: Profound ocular ischemia resulting in total acute vision loss is a rare and devastating outcome of primary scleral buckle procedures and may be caused by strangulation of the eye with an encircling band. Attention paid to the key tenets of this often successful and useful surgical technique may lower the risk for this complication.

PURPOSE:To analyze the systemic and ocular outcomes in patients with young-onset type 2 diabetes (YO-DM2) based on grade of presenting diabetic retinopathy (DR). METHODS:Retrospective cohort study analysis of empaneled patients with type 2 diabetes <40 years old with retinopathy screening within the Los Angeles Department of Health Services between 01/01/2017-07/01/2021 were included. Patients were stratified based on presenting severity of DR determined on fundus photographs or clinical examination. Patient's systemic co-morbidities and ocular outcomes were then compared across each group. Procedural (e.g. intravitreal injections) and surgical interventions (e.g. pars plana vitrectomy) were documented as performed by the treating physician. RESULTS:2795 patients were screened from 12,456 patients diagnosed with diabetes younger than age 40 (22.4 %). Of these, 1496 patients were diagnosed with type 2 DM. 1084 (72.4 %) of patients presented without DR, 307 (20.5 %) presented with non-proliferative diabetic retinopathy (NPDR), and 105 (7.0 %) of patients presented with proliferative diabetic retinopathy (PDR). Increasing presenting diabetic retinopathy severity was associated with longer duration of diabetes, greater systemic comorbidities (e.g. diabetic foot disease, neuropathy, chronic kidney or end stage renal disease), worse baseline and final visual acuity, and required more procedural and surgical interventions. CONCLUSIONS:Worse presenting DR severity in patients young-onset type 2 diabetes was associated with greater comorbid systemic and ocular disease with worse visual acuity outcomes. <1 % of patients without diabetic retinopathy or with mild NPDR were likely to progress to PDR. Diabetic kidney disease was an independent risk factor for developing neovascular glaucoma and retinal detachments. Prompt evaluation and intervention in patients with YO-DM2 may help reduce the associated systemic and ocular morbidity.

PURPOSE:To determine whether macular infarction measured as hyper-reflectivity of the middle and inner retinal layers predicts long-term visual acuity outcomes in participants with central retinal vein occlusion (CRVO) or hemi-retinal vein occlusion (HRVO). DESIGN:Clinical cohort study using post hoc secondary analysis of phase 3 clinical trial data. METHODS:This post hoc secondary analysis of the phase 3 Study of COmparative Treatments for REtinal Vein Occlusions 2 (SCORE2) clinical trial included 310 of the 362 participants with macular edema secondary to CRVO/HRVO who were randomized to injections of aflibercept or bevacizumab. Month 01 (M01) optical coherence tomography (OCT) images were analyzed using the following grading scheme: no infarction (grade 0), only middle retinal infarction (grade 1), diffuse middle and patchy inner retinal infarction (grade 2), and diffuse middle and inner retinal infarction (grade 3). Visual acuity letter score (VALS), central subfield thickness (CST), and number of anti-vascular endothelial growth factor (anti-VEGF) injections were correlated with the infarction severity grade at month 01. RESULTS:More severe macular infarction, with both middle and inner retinal layer hyper-reflectivity (ie, grades 2 and 3), was associated with worse M00 VALS and was predictive of VALS at M01 to M60 (P < .001). More severe infarction was associated with greater CST at presentation; however, after the first anti-VEGF injection, CST decreased and was similar across all grades at all time points (P > .05) with similar number of injections. CONCLUSIONS:Participants with more severe macular infarction at M01, as graded with OCT, exhibited worse visual outcomes despite significantly improved macular edema from month 6 to 5 years. This suggests that macular infarction may drive visual acuity after retinal fluid is treated with anti-VEGF.

& BULL; PURPOSE: To describe the progression of pentosan polysulfate sodium (PPS) maculopathy after drug discontinuation qualitatively and quantitatively using multimodal imaging assessmen.& BULL; DESIGN: Prospective case series.& BULL; METHODS: Patients with PPS maculopathy were evaluated after discontinuation of PPS. Near-infrared reflectance (NIR), fundus autofluorescence (FAF), and optical coherence tomography (OCT) were evaluated in all patients at baseline and at the final follow-up visit at least 12 months later. A qualitative and quantitative analysis of the retinal imaging findings was performed. Patterns of disease progression were evaluated. Area of disease involvement on FAF, retinal pigment epithelium (RPE) atrophy on FAF and NIR, and retinal layer thicknesses on OCT were measured at baseline and at the follow-up visit.& BULL; RESULTS: A total of 26 eyes were included, with a follow-up period ranging from 13 to 30 months. The dis-eased area measured on FAF showed significant expansion in all eyes from baseline to follow-up despite drug cessation ( P = .03) with a median linearized rate of change of 0.42 mm/y. There was significant reduction in the central macular thickness ( P = .04), inner nuclear layer thickness ( P = .003), outer nuclear layer thickness ( P = .02), and subfoveal choroidal thickness ( P = .003) at follow-up vs baseline. New areas of RPE atrophy on FAF in the macula developed in 4 eyes while preexisting atrophic lesions increased in size in 5 eyes.& BULL; CONCLUSION: Eyes with baseline PPS maculopathy all exhibited remarkable progression with qualitative and quantitative multimodal imaging analysis despite drug discontinuation. Disease progression may be attributed to underlying inner choroidal ischemia or RPE impairment. (Am J Ophthalmol 2023;255: 57-67. Published by Elsevier Inc.)

To analyze the systemic and ocular outcomes in patients with young onset type 2 diabetes (YO-DM2) based on grade of presenting diabetic retinopathy (DR). Retrospective cohort study analysis of empaneled patients with type 2 diabetes <40 years old with retinopathy screening within the Los Angeles Department of Health Services between 01/01/2017–07/01/2021 were included. Patients were stratified based on presenting severity of DR determined on fundus photographs or clinical examination. Patient's systemic co-morbidities and ocular outcomes were then compared across each group. Procedural (e.g. intravitreal injections) and surgical interventions (e.g. pars plana vitrectomy) were documented as performed by the treating physician. 2795 patients were screened from 12,456 patients diagnosed with diabetes younger than age 40 (22.4 %). Of these, 1496 patients were diagnosed with type 2 DM. 1084 (72.4 %) of patients presented without DR, 307 (20.5 %) presented with non-proliferative diabetic retinopathy (NPDR), and 105 (7.0 %) of patients presented with proliferative diabetic retinopathy (PDR). Increasing presenting diabetic retinopathy severity was associated with longer duration of diabetes, greater systemic comorbidities (e.g. diabetic foot disease, neuropathy, chronic kidney or end stage renal disease), worse baseline and final visual acuity, and required more procedural and surgical interventions. Worse presenting DR severity in patients young-onset type 2 diabetes was associated with greater comorbid systemic and ocular disease with worse visual acuity outcomes. <1 % of patients without diabetic retinopathy or with mild NPDR were likely to progress to PDR. Diabetic kidney disease was an independent risk factor for developing neovascular glaucoma and retinal detachments. Prompt evaluation of YO-DM2 may help reduce the systemic and ocular morbidity of diabetes.

Vitelliform lesions (VLs) are associated with a wide array of macular disorders but are the result of one common pathway: retinal pigment epithelium (RPE) impairment and phagocytic dysfunction. VLs are defined by the accumulation of yellowish subretinal material. In the era of multimodal advanced retinal imaging, VLs can be further characterized by subretinal hyperreflectivity with optical coherence tomography and hyperautofluorescence with fundus autofluorescence. VLs can be the result of genetic or acquired retinal diseases. In younger patients, VLs usually occur in the setting of Best disease. Additional genetic causes of VL include pattern dystrophy or adult-onset vitelliform macular dystrophy. In older patients, acquired VLs can be associated with a broad spectrum of etiologies, including tractional, paraneoplastic, toxic, and degenerative disorders. The main cause of visual morbidity in eyes with VLs is the onset of macular atrophy and macular neovascularization. Histopathological studies have provided new insights into the location, nature, and lifecycle of the vitelliform material comprised of melanosomes, lipofuscin, melanolipofuscin, and outer segment debris located between the RPE and photoreceptor layer. Impaired phagocytosis by the RPE cells is the unifying pathway leading to VL development. We discuss and summarize the nature, pathogenesis, multimodal imaging characteristics, etiologies, and natural course of vitelliform maculopathies.

A recent wave of pharmacologic and technologic innovations has revolutionized our management of retinal diseases. Many of these advancements have demonstrated efficacy and can increase the quality of life while potentially reducing complications and decreasing the burden of care for patients. Some advances, such as longer-acting anti-vascular endothelial growth factor agents, port delivery systems, gene therapy, and retinal prosthetics have been approved by the US Food and Drug Administration, and are available for clinical use. Countless other therapeutics are in various stages of development, promising a bright future for further improvements in the management of the retinal disease. Herein, we have highlighted several important novel therapies and therapeutic approaches and examine the opportunities and limitations offered by these innovations at the new frontier. KEY MESSAGESNumerous pharmacologic and technologic advancements have been emerging, providing a higher treatment efficacy while decreasing the burden and associated side effects.Anti-vascular endothelial growth factor (anti-VEGF) and its longer-acting agents have dramatically improved visual outcomes and have become a mainstay treatment in various retinal diseases.Gene therapy and retinal prosthesis implantation in the treatment of congenital retinal dystrophy can accomplish the partial restoration of vision and improved daily function in patients with blindness, an unprecedented success in the field of retina.

PURPOSE: To analyze imaging characteristics and the clinical course of patients demonstrating coincident lesions of paracentral acute middle maculopathy (PAMM) and acute macular neuroretinopathy (AMN) in the same eye. DESIGN: Retrospective, observational case series. METHODS: Lesions from patients presenting with coincident PAMM and AMN in the same eye were evaluated with multimodal imaging including optical coherence tomography (OCT). The association with ocular and systemic findings was also investigated. RESULTS: Fifteen subjects (17 eyes) were included in the study. The mean age was 44.4 +/- 15.3 years and the follow-up period ranged from 1 to 32 weeks (mean, 11.9 +/- 11.4 weeks). The mean visual acuity was 0.8 +/- 0.6 logarithm of minimal angle of resolution (Snellen equivalent 20/126) at baseline and 0.3 +/- 0.4 logarithm of minimal angle of resolution (Snellen equivalent 20/40) at the last follow-up. PAMM and AMN lesions occurred in the setting of Purtscher's retinopathy (4 eyes, 3 patients), retinal vein occlusion (7 eyes, 7 patients), central retinal artery occlusion (1 eye, 1 patient), and idiopathic retinal vasculitis (1 eye, 1 patient). In 4 eyes (3 patients), an association with other ocular disorders was not identified as evaluated with multimodal imaging. Of the total cohort, 11 eyes (64.7%) showed extension of the AMN hyperreflective bands in Henle's fiber layer with a Z-shaped morphology on OCT B-scan. CONCLUSIONS: The presence of coincident PAMM and AMN suggests a common pathophysiologic etiology. This may be the result of retinal vein impairment and hypoperfusion at the level of the deep retinal capillary plexus possibly leading to injury to the Muller glia or photoreceptors in Henle's fiber layer. (C) 2021 Elsevier Inc. All rights reserved.