Multimodal Retinal Imaging in Spinocerebellar Ataxia Type 1 Maculopathy

Objectives: The objective of the study was to investigate and report the multimodal ocular imaging findings associated with spinocerebellar ataxia type 1 (SCA 1) associated maculopathy. Methods: A full ophthalmologic assessment was completed in a 70-year-old male with confirmed SCA1 and noted progressive bilateral vision loss. Investigations included dilated fundus examination, full-field electroretinography, and swept-source optical coherence tomography (OCT). Results: On neurologic and ophthalmologic examination, he was found to have hypermetric saccades, horizontal nystagmus, and reduced color vision bilaterally. His best-corrected visual acuity was confirmed to be 20/80 OD and 20/100 OS at the time of consultation. Initial fundus photography was most notable for bilateral hypopigmentation of the fovea. Corresponding OCT imaging demonstrated an attenuation of the ellipsoid zone, in keeping with photoreceptor loss. Conclusion: The ocular imaging results suggest that the vision loss in the presented case occurred in the context of pigmentary macular dystrophy secondary to photoreceptor dysfunction and retinal pigment epithelial degeneration. This association offers an explanation with respect to the progressive vision loss, but further analyses would be required to determine the temporal correlation of clinical symptoms with imaging abnormalities. These findings suggest that SCA1 be considered as a potential cause for vision impairment, with possible benefits of visual assessment at the time of diagnosis.

Remote Imaging Opportunities, Innovations, and Considerations for Teleophthalmology

Imaging has emerged as a key tool for ophthalmologists to quickly and accurately diagnose and help manage ophthalmic conditions. The importance of useful teleimaging technology will increase as remote appointments and surveillance becomes more widely utilized in ophthalmology. This review article describes the current state of remote imaging including the results of many “store and forward” studies. It also summarizes potential emerging teleimaging modalities such as home optical coherence tomography, remote slit lamps, and smartphone imaging. While published studies highlight many possible utilities for teleimaging, further clinical validation and technology improvement need to occur before teleimaging can become more ubiquitous.

Safety and efficacy of ALG-1007 topical ophthalmic solution – A synthetic peptide that regulates inflammation, in patients with dry eye disease: An exploratory Phase I, open-label, single-center clinical study

Objectives: The objective of the study was to evaluate the safety and efficacy of ALG-1007 topical ophthalmic solution in patients with dry eye disease (DED). Materials and Methods: This Phase I, prospective, open-label, 12-week study enrolled subjects ≥18 years old with symptoms of DED for at least 6 months and at least one of the following: Total ocular staining score ≥2 or tear film breakup time (TBUT) ≤7 s. Subjects were randomized to four treatment arms: 0.125%, 0.25%, 0.4%, and 0.6% ALG-1007. Subjects received the test drug, 1 drop twice daily, and were followed at multiple time points for 12 weeks. SICCA total ocular staining score, corneal and conjunctival staining score, TBUT, and subject-reported symptoms using the visual analog scale (VAS) symptom index were assessed at baseline and at every visit. The primary safety outcome was percentage and severity of adverse events (AEs). Results: Forty eyes (21 patients) were assigned randomly to four treatment groups (n = 10 per group). Improvement in TBUT, SICCA, and VAS was seen in all groups. The highest dose tested (0.6%) was compared to the lowest dose tested (0.125%) based on change from baseline for all assessments using analysis of variance. Improvement was significantly greater in 0.6% treatment group in terms of TBUT, conjunctiva staining, SICCA, burning, discomfort, photophobia, and the composite symptom score. No serious AEs were reported after 12 weeks of treatment. Conclusion: Outcome measures improved in all the treatment groups. At the highest dose, ALG-1007 demonstrated statistically significant improvement compared to the lowest dose in 7 out of 12 assessments, indicating a dose response. This suggests that the active pharmaceutical ingredient in ALG-1007 is effective in improving signs and symptoms of DED. ALG-1007 was well-tolerated with minimal instillation discomfort and no reported serious AEs.

Preferential Hyperacuity Home Testing for Monitoring Intermediate Age-related Macular Degeneration in Routine Clinical Practice

Objectives: A home monitoring device (ForeseeHome; Notal Vision, Tel Aviv, Israel) detected choroidal neovascularization in age-related macular degeneration (AMD) earlier than home and office monitoring. This study describes device usage in routine clinical practice. Materials and Methods: An Institutional Review Board-approved retrospective chart review of intermediate AMD patients who used the device at a single tertiary care center. Primary outcomes were duration and frequency of usage and outcomes of alerts generated. Results: Sixty-two patients (106 eyes) completed monitoring 4.2 ± 2.1 times per week (avg ± std dev) from February 2015 to February 2019. Forty-five eyes discontinued monitoring after an average of 65 days (range 13–237 days), due to poor quality tests (62%), and false-positive alerts (16%). Eyes that discontinued device monitoring were older and had poorer visual acuity than eyes that continued monitoring (P < 0.01). Fourteen alerts were generated, of which one represented conversion to exudative AMD. Two additional converted eyes were identified by routine office visit and Amsler grid monitoring. Conclusions: Home monitoring has the potential to improve the detection of exudative AMD. Identifying barriers to device utilization are necessary in the AMD population.

Chloroquine and Hydroxychloroquine Toxicity

Chloroquine and hydroxychloroquine have been used for decades as antimalarials and also as immunomodulating therapies for rheumatic diseases such as systemic lupus erythematosus and rheumatoid arthritis. They also have antiviral properties and are currently used empirically for the treatment of coronavirus disease 2019 (COVID-19). Retinal toxicity is a potential complication of these medications. Current ophthalmic screening and dosing recommendations aim to decrease the risk of developing retinopathy or prevent its progression. Baseline fundus examination is not currently recommended before initiating chloroquine or hydroxychloroquine for COVID-19 due to presumed very low risk of retinal toxicity. However, doses of the drugs used for the treatment of COVID-19 exceed the recommended doses, and patients often have additional risk factors. Research in the future is warranted to confirm the risk and incidence of toxicity with this novel use.

Trends in neovascular glaucoma management: Practice patterns of glaucoma and retina specialists in the United States

Purpose: To characterize practice patterns between and within retina and glaucoma subspecialists treating neo- vascular glaucoma (NVG). Methods: Preliminary survey study consisting of 8-questions developed based on actual clinical cases presenting to our institution between June 2014 and June 2016. The survey was administered in the form of clinical vignettes and circulated electronically using Survey Monkey ©. to members of the American Glaucoma Society (AGS) and American Society of Retina Specialists (ASRS). Results: 109 glaucoma and 130 retina specialists initiated the survey. For initial management of patients with good visual acuity (VA) and NVG, retina specialists preferred anti-VEGF therapy (P < 0.01) while glaucoma specialists preferred combined anti-VEGF and tube shunt surgery (P < 0.01). Following initial therapy in patients with good VA, glaucoma specialists preferred surgery (P < 0.01) and retina specialists preferred PRP (P < 0.01). In patients with poor VA, PRP and anti-VEGF were equally favored, with a preference for surgery by glaucoma specialists compared to retina specialists (P < 0.01). Conclusion: NVG is a complex problem within the field of ophthalmology that often requires management by both retina and glaucoma specialists. Agreement between both subspecialties for the use of anti-VEGF agents as first line treatment, either alone or in concert with other therapies was noted. Multiple differences exist in next step in therapy and long-term management. Future consensus panels may mitigate discrepancies in NVG management and work toward development of care paths for more efficient care delivery.

Changes in Macular and Peripheral Perfusion Following Anti-vascular Endothelial Growth Factor Treatment for Patients with Diabetic Retinopathy

Diabetic retinopathy (DR) is a common complication of diabetes mellitus. Imaging techniques such as the gold standard of fluorescein angiography (FA) and the recent advent of optical coherence tomography angiography (OCTA) have allowed for detailed visualization of the microvasculature to assess and quantify macular perfusion. The development of ultra-widefield FA (UWFA) has allowed clinicians to examine ischemia within the periphery. As our imaging and diagnostic methods continue to be refined, it is important to understand how to best utilize these technologies to characterize disease severity and determine how it can guide treatment and prognosis. With the increasing use of anti-vascular endothelial growth factor (VEGF) agents for the treatment of DR, we can characterize the changes in perfusion on the microvascular level. The focus of this review is to summarize and identify the changes in both macular and peripheral perfusion with anti-VEGF treatment for patients with DR utilizing FA, UWFA, and OCTA. There remains a need for more prospective, long-term clinical trials with controls to better understand the characterization of the retinal microvasculature using these imaging techniques. In clinical practice, physicians likely will need to utilize a multimodal imaging approach to appropriately characterize the degree and progression of ischemia in DR.

Current Understanding of the Pathophysiology of Disorganization of the Retinal Inner Layers and Relationship to Visual Acuity

In clinical practice, visual acuity (VA) outcomes after treatment may be associated with multiple optical coherence tomography (OCT) variables including central subfield thickness (CST), ellipsoid zone (EZ) disruption , epiretinal membrane, vitreoretinal interface abnormalities, and disorganization of retinal inner layers (DRIL). Since CST has shown only a modest correlation with VA, additional OCT-based prognostic factors can be useful. DRIL is an OCT derived variable that may be a prognostic factor. Ischemia and inflammation may explain the pathogenesis of DRIL but are not well understood. Visual transmission pathways may be interrupted and affect VA. This review highlights the various studies on DRIL and VA, specifically within diabetic macular edema (DME), diabetic retinopathy, retinal vein occlusion, and Uvetic macular edema (ME). Based on the current literature review, DRIL’s prognostic value in predicting VA is not well elucidated but shows potential to be utilized in clinical practice.

Multimodal Imaging of Adult-onset Coats’ Disease

The majority of Coats’ disease cases are diagnosed in the first decade of life, though cases with symptom onset and first diagnosis in adulthood are well documented. While the cases diagnosed in childhood and adulthood share many of the characteristic features that define Coats’ disease, adult cases have been shown to present with less severe disease manifestations and an increased incidence of macroaneurysms with perianeurysmal hemorrhage. We present two cases of Coats’ disease diagnosed in adulthood that demonstrate prominent arteriolar macroaneurysms with perianeurysmal hemorrhages and contribute to an understanding of the adult Coats’ phenotype.