Self-Reported Functional Vision in USH2A-Associated Retinal Degeneration as Measured by the Michigan Retinal Degeneration Questionnaire.

Purpose: The purpose of this study was to evaluate self-reported functional vision (FV) and the impact of vision loss in patients with USH2A-associated retinal degeneration using a patient-reported outcome (PRO) measure, the Michigan Retinal Degeneration Questionnaire (MRDQ), to correlate MRDQ scores with well-established visual function measurements. Design: An observational cross-sectional study (n = 93) of participants who had Usher Syndrome Type 2 (USH2, n = 55) or autosomal recessive non-syndromic retinitis pigmentosa (ARRP; n = 38) associated with biallelic variants in the USH2A gene. Methods: The study protocol was approved by all ethics boards and informed consent was obtained from each participant. Participants completed the MRDQ at the 48-month study follow-up visit. Disease duration was self-reported by participants. One-way ANOVA was used to compare subgroups (clinical diagnosis, age, disease duration, and full-field stimulus threshold [FST] Blue-Red mediation) on mean scores per domain. Spearman correlation coefficients were used to assess associations between MRDQ domains and visual/retinal function assessments. Results: Of the study sample, 58% were female participants and the median disease duration was 13 years. MRDQ domains were sensitive to differences between subgroups of clinical diagnosis, age, disease duration, and FST Blue-Red mediation. MRDQ domains correlated with static perimetry, microperimetry, full-field stimulus testing, and best-corrected visual acuity (BCVA). Conclusions: Self-reported FV measured by the MRDQ, when applied to USH2 and ARRP participants, had good distributional characteristics and correlated well with visual function tests. MRDQ adds a new dimension of understanding on vision-related functioning and establishes this PRO tool as an informative measure in evaluating USH2A outcomes.

Recalcitrant Fusarium Fungal Endophthalmitis: Novel Surgical Technique Combining Vitrectomy with Intralesional Injection of Voriconazole and Povidone Iodine.

PURPOSE: To show the feasibility while comparing functional and anatomical outcomes of vitrectomy combined with intralesional injection of voriconazole and povidone iodine for recalcitrant fusarium fungal endophthalmitis. METHODS: In a noncomparative interventional case, vitrectomy combined with intralesional injection of voriconazole and povidone iodine was performed in a single patient. RESULTS: Following vitrectomy and intralesional injection, our patient's fungal endophthalmitis stabilized and vision improved to 20/25. He did not require further intravitreal injections or vitrectomy. CONCLUSIONS: Vitrectomy and intralesional injection for the treatment of refractory filamentary fungal endophthalmitis proved to be safe and effective for this case.

A Case of Retinal Atherosclerotic Oxalosis due to Hyperoxaluria in a Patient with Short Gut Syndrome.

PURPOSE: To describe a severe case of crystalline retinopathy secondary to hyperoxaluria from short gut syndrome. METHODS: Case report. RESULTS: A 62-year-old Caucasian female with short gut syndrome and end-stage renal disease from renal oxalosis presented with chronic bilateral vision loss. She had previously been treated for presumed occlusive vasculitis. Visual acuity on initial exam was 20/400 OD and 20/100 OS with an afferent pupillary defect of the right eye.Exam revealed attenuated retinal vasculature and diffuse crystalline infiltration of retinal arterial lumens and throughout the retinas bilaterally. Optical coherence tomography revealed inner retinal atrophy with crystalline deposition in the inner retinal layers. Fluorescein angiography demonstrated delayed vascular filling and dropout consistent with severe ischemic vasculopathy. It was concluded that the short-gut syndrome led to over-absorption of oxalate with subsequent hyperoxaluria leading to retinal atherosclerotic oxalosis. CONCLUSION: Retinal calcium oxalate deposits due to hyperoxaluria have been previously noted; however, this degree of severe retinal vascular infiltration has not been described. Our patient was receiving hemodialysis, which is associated with high rebound increases in systemic oxalate concentrations. It is important to keep hyperoxaluria in mind as a potential cause of retinopathy in patients with end-stage renal disease presenting with vision loss.

Accuracy of intravitreal injection volume for aflibercept pre-filled syringe and BD Luer-Lok one-milliliter syringe.

BACKGROUND: To evaluate the accuracy of intravitreal injection volume of the pre-filled syringe (PFS) in which aflibercept is packaged compared to the BD Luer-Lok 1-mL syringe. METHODS: Ophthalmologists injected their typical intravitreal volume for aflibercept using either the PFS or BD Luer-Lok 1-mL syringe for 5 times each. The injected fluid was weighed using a micro-scale and converted to volume. The volume of fluid injected was also evaluated when the 0.05 mL line on the PFS was lined up to the tip or base of the dome-shaped plunger. RESULTS: Injection volume was measured for 12 physicians. The average injected fluid volume was 74.22 ± 15.87 µL for PFS and 53.42 ± 4.61 µL for the BD Luer-Lok 1-mL syringe (p < 0.0001). The average deviation in volume injected for the PFS was higher compared to the BD Luer-Lok 1-mL syringe (11.36 µL vs. 3.35 µL, p < 0.0001). When the PFS was lined up with the tip of the dome-shaped plunger at the 0.05-mL line, the average injected volume was 71.03% higher. CONCLUSIONS: The intravitreal injection volume and variability using the new PFS were significantly higher than the volume injected using the BD Luer-Lok 1-mL syringe previously used, potentially leading to higher rates of visually significant elevation of intraocular pressures.

Intraocular Povidone Iodine During Pars Plana Vitrectomy for Severe and Atypical Endophthalmitis.

BACKGROUND AND OBJECTIVE: To describe the experience and clinical outcomes of povidone iodine (PI) infusion in the setting of pars plana vitrectomy for the treatment of endophthalmitis. MATERIALS AND METHODS: This was a retrospective case series of 12 patients with clinical and/or culture evidence of endophthalmitis requiring pars plana vitrectomy with 0.025% PI used in vitreous irrigation solution during vitrectomy. The primary endpoint was clinical resolution of the infection. Secondary endpoints included visual recovery, need for repeat surgery, and ocular toxicity RESULTS: There were 11 eyes that showed clinical or culture evidence of resolution of infection postoperatively (91.7%); 10 eyes had improvement in vision postoperatively (83.3%). Overall uncorrected visual acuity improved from 20/5321 (2.43 ± 0.58 logMAR) to 20/375 (1.27 ± 1.05 logMAR) (P = .0003). No clinical evidence of ocular toxicity or unexplained vision loss due to PI was observed. CONCLUSIONS: PI infusion during pars plana vitrectomy for endophthalmitis appears safe and led to excellent post-surgical results in a traditionally high-risk cohort. [Ophthalmic Surg Lasers Imaging Retina. 2021;52:485-490.].

Quantifying Areas of Vascular Leakage in Sickle Cell Retinopathy Using Standard and Widefield Fluorescein Angiography.

BACKGROUND AND OBJECTIVE: To evaluate neovascular surface area change in sickle cell retinopathy following scatter photocoagulation treatment in wide- and standard-field fluorescein angiography (FA) scans using ImageJ software. PATIENTS AND METHODS: Images of 11 patients with wide- or standard-field FA scans pre- and post-treatment for sickle cell retinopathy were evaluated retrospectively by two graders using ImageJ. Graders traced lesions in the late arteriovenous phase and calculated the lesion area and intensity relative to the optic disc. Changes in area and intensity pre- to post-treatment were assessed using dependent t-tests. RESULTS: Pre- to post-treatment, lesion area decreased by 3.34 ± 2.43 to 3.66 ± 3.72 disc areas (P < .001), whereas intensity decreased by 11.36 ± 25.87 to 22.97 ± 69.25 units (P = .104). Neovascular area declined status post-aphotocoagulation by 3.83 ± 3.65 disc areas (P = .003) for widefield images and by 2.81 ± 3.55 discs areas (P = .034) for standard fluorescein images. No statistical difference in area reduction was appreciated between imaging modalities (P = .652) CONCLUSION: Neovascular area decreased significantly following scatter photocoagulation in both standard-field and widefield FA scans without an appreciable difference between imaging modalities. [Ophthalmic Surg Lasers Imaging Retina. 2020;51:153-158.].

Intravenous Prostaglandin E1 Infusion for Acute Central Retinal Artery Occlusion.

BACKGROUND AND OBJECTIVE: To evaluate the efficacy of systemic prostaglandin E1 (PGE1) infusion within the first 24 hours of acute central retinal artery occlusion (CRAO). PATIENTS AND METHODS: Best corrected visual acuity (BCVA) was analyzed in a case series of six eyes from six patients (mean age: 69.33 years) with acute CRAO who were treated with twice-daily intravenous infusion of 40 µg PGE1. Therapy continued until the patient no longer experienced visual acuity improvements for 24 hours. RESULTS: Average time to presentation was 8.33 hours (range: 2 to 12 hours). The logMAR BCVA at presentation was 2.73. BCVA at the final visit 1 month after initial presentation was 1.48 (P = .025). All patients experienced vision improvement. No systemic adverse events were experienced. CONCLUSION: Intravenous PGE1 infusion resulted in significant visual improvement in patients presenting with acute CRAO and is well tolerated with few adverse effects. [Ophthalmic Surg Lasers Imaging Retina. 2019;50:S5-S8.].