Intentional retinal injury with handheld lasers is an underrecognized form of self-harm.

Self-inflicted damage to the retina using handheld lasers is a growing and underrecognized form of self-harm. Here was share retinal images from two patients with histories of major depressive disorder and self-harm behaviors that ultimately resulted in legal blindness. Mental health providers should be aware of this clinical entity as they are in the best position to screen for laser pointer access in at risk individuals and prevent permanent vision loss.

Risk Factors and Disease Course for Blood-Brain Barrier Disruption-Associated Maculopathy.

Importance: Blood-brain barrier disruption (BBBD) is a systemic therapy for malignant central nervous system (CNS) tumors that has been linked to poorly understood pigmentary maculopathy. Objectives: To examine the rate of and risk factors for the development of BBBD-associated maculopathy and to assess whether there can be visually significant progression after completion of systemic therapy. Design, Setting, and Participants: In this retrospective case series, data from February 1, 2006, through December 31, 2019, were collected from patients treated with osmotic BBBD at a single tertiary referral center who had subsequent ophthalmic evaluation. Exposures: Treatment with BBBD therapy for any malignant CNS tumor. Main Outcomes and Measures: Rate and potential risk factors for developing BBBD-associated maculopathy and changes in visual acuity and retinal imaging characteristics after completion of BBBD therapy. Results: Of 283 patients treated with BBBD and chemotherapy for a CNS malignant neoplasm, 68 (mean [SD] age, 46.0 [17.9] years; 25 [38.5%] female) had an ophthalmic examination after starting systemic therapy. After excluding 3 patients because of bilateral media opacities, pigmentary maculopathy was present in 32 of 65 patients (49.2%) treated with BBBD. The number of BBBD treatment sessions, but not age, CNS malignant cancer type, or systemic chemotherapy agent, was associated with maculopathy development (odds ratio, 1.30; 95% CI, 1.12-1.50; P = .001). After completion of BBBD therapy, progressive enlargement of geographic atrophy occurred in 5 eyes of 3 patients, and choroidal neovascularization developed in 1 eye. Conclusions and Relevance: In this case series, an association was found between BBBD-related maculopathy and the number of BBBD treatment sessions, suggesting a dose-dependent effect. In some cases, maculopathy progression, including enlargement of geographic atrophy, occurred years after completion of systemic therapy. These findings may have important implications for patient education and ophthalmic monitoring.

Filtering-Associated Endophthalmitis after Trabeculectomy or Tube-Shunt Implant.

PURPOSE: To determine the rate, clinical features, and outcomes of filtering-associated endophthalmitis in eyes that underwent trabeculectomy or tube-shunt implantation. DESIGN: Retrospective database study with selective chart review. PARTICIPANTS: Eyes that underwent incisional glaucoma surgery at our institution between January 1, 2012, and December 31, 2019. METHODS: An electronic medical record database was used to identify all eyes that underwent trabeculectomy or tube-shunt implantation during the study period. Date of surgery, date of last ophthalmology clinic visit, and filtering-associated endophthalmitis diagnoses were obtained and used to perform a Kaplan-Meier analysis. The log-rank test was used to compare rates of filtering-associated endophthalmitis after trabeculectomy and tube-shunt implantation. Microbiology, management, and clinical outcomes data from patients with filtering-associated endophthalmitis were also collected and analyzed. MAIN OUTCOME MEASURES: Cumulative risk of filtering-associated endophthalmitis as determined by Kaplan-Meier analysis. Visual acuity improvement to within 2 lines of baseline at 3 months of follow-up and globe salvage at last available follow-up in eyes with filtering-associated endophthalmitis. RESULTS: Kaplan-Meier analysis of 1582 eyes that underwent incisional glaucoma surgery yielded a 5-year cumulative incidence for filtering-associated endophthalmitis of 1.32%. No statistically significant differences were found between rate of endophthalmitis after trabeculectomy or tube-shunt implantation (P = 0.761, log-rank test). Seven of 16 cases (43.8%) of filtering-associated endophthalmitis showed positive culture results from either a vitreous sample or explanted tube shunt. Recovery of vision to within 2 lines of pre-endophthalmitis baseline was achieved in 53% of patients at 3 months of follow-up. Rate of globe salvage was 100% at last available follow-up. CONCLUSIONS: Risk of filtering-associated endophthalmitis is persistent and relatively constant for at least 5 years after incisional glaucoma surgery. The overall prognosis of filtering-associated endophthalmitis remains poor; however, good visual and anatomic outcomes can be achieved in some patients with prompt intervention.

Genotype Phenotype Correlation and Variability in Microcephaly Associated With Chorioretinopathy or Familial Exudative Vitreoretinopathy.

Purpose: The purpose of this study was to analyze the natural history and phenotypic overlap of patients with microcephaly and a chorioretinopathy or familial exudative vitreoretinopathy (FEVR) ocular phenotype caused by mutations in KIF11, TUBGCP4, or TUBGCP6. Methods: Patients diagnosed with congenital microcephaly and chorioretinopathy or FEVR were included. Molecular investigations consisted of targeted genetic sequencing. Data from medical records, ophthalmologic examination and imaging, electroretinography, and visual fields were analyzed for systemic and ophthalmic features and evidence of posterior segment disease progression. Results: Twelve patients from 9 families were included and had a median of 8 years of follow-up. Nine patients had KIF11 variants, two had heterozygous TUBGCP6 variants, and one had heterozygous variants in TUBGCP4. All patients had reduced visual function and multiple individuals and families showed features of both chorioretinopathy and FEVR. Progression of posterior segment disease was highly variable, with some degree of increased atrophy of the macula or peripheral retina or increased vitreoretinal traction observed in 9 of 12 patients. Conclusions: Microcephaly due to mutations in KIF11, TUBGCP4, or TUBGCP6 can be associated with retinal disease on a spectrum from chorioretinal atrophy to FEVR-like posterior segment changes. Visually significant disease progression can occur and patients should be monitored closely by a team experienced in ophthalmic genetics.

Disease Course in Patients With Pentosan Polysulfate Sodium-Associated Maculopathy After Drug Cessation.

Importance: Recent studies have linked a vision-threatening maculopathy with long-term use of pentosan polysulfate sodium (PPS). Objective: To evaluate the disease course in PPS-associated maculopathy after drug cessation. Design, Setting, and Participants: In this retrospective case series, patients diagnosed with PPS-associated maculopathy with at least 6 months of follow-up after drug cessation who were treated at the Emory Eye Center, Atlanta, Georgia, or the Casey Eye Institute, Portland, Oregon, were included. Data were collected from April 2014 through November 2019. Main Outcomes and Measures: Change in visual acuity and retinal imaging characteristics over time. Results: Of the 11 included patients, all were female, and the median (interquartile range [IQR]) age was 53 (44-63) years. Participants had a baseline visit at a median (IQR) of 2 (0-4) months after drug cessation and were subsequently observed for a median (IQR) of 12 (8-26) months. The median (IQR) cumulative PPS exposure was 1.97 (1.55-2.18) kg. No eyes exhibited a demonstrable improvement in disease after discontinuing PPS. A total of 9 of 11 patients (82%) reported worsening visual symptoms at the final visit. The mean (SD) logMAR visual acuity was 0.14 (0.23) and 0.14 (0.34) at the baseline and final visit, respectively. Visual acuity improved by 2 or more Snellen lines in 1 eye (5%) and declined by 2 or more Snellen lines in 2 eyes of 1 patient (9%). There was evolution in the pattern of fundus autofluorescence changes and/or optical coherence tomography findings in all eyes. A total of 17 eyes (77%) exhibited expansion of the area of involved tissue. A total of 7 eyes (32%) had macular retinal pigment epithelium atrophy at the baseline visit, and atrophy enlarged after discontinuation of PPS in all 7 eyes, with a median (IQR) growth rate of 0.32 (0.13-0.38) mm per year. Conclusions and Relevance: These retrospective data among 11 patients suggest PPS-associated maculopathy continues to evolve after drug cessation for at least 10 years. In some cases, progressive retinal pigment epithelium atrophy encroaches on the foveal center and thus may pose a long-term threat to central vision.

Maculopathy Associated With Osmotic Blood- Brain Barrier Disruption and Chemotherapy in Patients With Primary CNS Lymphoma.

BACKGROUND AND OBJECTIVE: To describe the incidence, characteristics, and risk factors of a pigmentary maculopathy in patients with primary central nervous system (CNS) lymphoma treated with blood-brain barrier disruption (BBBD) therapy. PATIENTS AND METHODS: This retrospective chart review included patients with biopsy-proven primary CNS lymphoma treated with or without BBBD therapy who underwent an ophthalmic examination after starting systemic treatment. Clinical data and all available retinal imaging were analyzed. RESULTS: Fifty-eight patients met inclusion criteria. Twenty-one of 36 patients treated with BBBD therapy had a bilateral pigmentary maculopathy. None of the 22 patients treated with conventional chemotherapy had similar changes. Additional findings in patients treated with BBBD included geographic retinal pigment epithelium atrophy, subretinal fluid, and in one case, choroidal neovascularization. Some cases of maculopathy resulted in reduced visual acuity. The presence of a pigmentary maculopathy was associated with a higher number of BBBD treatment sessions (20.1 vs 13.3, P = .016), but not vitreoretinal lymphoma involvement or intravitreal methotrexate injections. CONCLUSION: In this cohort, 58.3% of patients with primary CNS lymphoma treated with BBBD and chemotherapy were found to have a bilateral pigmentary maculopathy. This maculopathy can result in reduced visual acuity and is associated with the number of BBBD treatment sessions. [Ophthalmic Surg Lasers Imaging Retina. 2020;51:S5-S12.].

Culture-Proven Endophthalmitis After Intravitreal Injection: A 10-Year Analysis.

BACKGROUND AND OBJECTIVE: To report on the microbiology, management, and visual outcomes of intravitreal injection (IVI)-associated, culture-proven endophthalmitis. PATIENTS AND METHODS: All patients seen at a tertiary referral center with culture-proven endophthalmitis associated with an IVI between June 2007 and July 2017 were included in this retrospective analysis. RESULTS: Thirty-five patients with culture-positive endophthalmitis following IVI were identified. All gram-positive organisms (34 of 35) were susceptible to vancomycin. Cases due to pathogens associated with oral or respiratory flora were common (31.4%, n = 11), presented earlier (2.0 days vs. 4.6 days, P < .001), were more likely to undergo pars plana vitrectomy (81.8% vs. 25.0%, P = .002) and had worse visual acuity outcomes. CONCLUSION: IVI-associated endophthalmitis pathogens and anti-microbial susceptibilities in the Pacific Northwest are similar to those reported from other geographic locations. Bacteria associated with the oral and respiratory flora are common isolates that result in a more aggressive course and worse visual outcomes. [Ophthalmic Surg Lasers Imaging Retina. 2019;50:33-38.].

Evaluation of Automatically Quantified Foveal Avascular Zone Metrics for Diagnosis of Diabetic Retinopathy Using Optical Coherence Tomography Angiography.

Purpose: To describe an automated algorithm to quantify the foveal avascular zone (FAZ), using optical coherence tomography angiography (OCTA), and to compare its performance for diagnosis of diabetic retinopathy (DR) and association with best-corrected visual acuity (BCVA) to that of extrafoveal avascular area (EAA). Methods: We obtained 3 × 3-mm macular OCTA scans in diabetic patients with various levels of DR and healthy controls. An algorithm based on a generalized gradient vector flow (GGVF) snake model detected the FAZ, and metrics assessing FAZ size and irregularity were calculated. We compared the automated FAZ segmentation to manual delineation and tested the within-visit repeatability of FAZ metrics. The correlations of two conventional FAZ metrics, two novel FAZ metrics, and EAA with DR severity and BCVA, as determined by Early Treatment Diabetic Retinopathy Study (ETDRS) charts, were assessed. Results: Sixty-six eyes from 66 diabetic patients and 19 control eyes from 19 healthy participants were included. The agreement between manual and automated FAZ delineation had a Jaccard index > 0.82, and the repeatability of automated FAZ detection was excellent in eyes at all levels of DR severity. FAZ metrics that incorporated both FAZ size and shape irregularity had the strongest correlation with clinical DR grade and BCVA. Of all the tested OCTA metrics, EAA had the greatest sensitivity in differentiating diabetic eyes without clinical evidence of retinopathy, mild to moderate nonproliferative DR (NPDR), and severe NPDR to proliferative DR from healthy controls. Conclusions: The GGVF snake algorithm tested in this study can accurately and reliably detect the FAZ, using OCTA data at all DR severity grades, and may be used to obtain clinically useful information from OCTA data regarding macular ischemia in patients with diabetes. While FAZ metrics can provide clinically useful information regarding macular ischemia, and possibly visual acuity potential, EAA measurements may be a better biomarker for DR.

Wide-Field OCT Angiography Investigation of the Relationship Between Radial Peripapillary Capillary Plexus Density and Nerve Fiber Layer Thickness.

Purpose: To characterize the density and distribution of the radial peripapillary capillary plexus (RPCP) and its relationship with retinal nerve fiber layer (NFL) thickness in healthy subjects. Methods: Using spectral-domain optical coherence tomography (OCT), split-spectrum amplitude decorrelation angiography algorithm and automated montaging, wide-field OCT angiography (OCTA) was used to measure the RPCP capillary density (CD) and NFL thickness. Polar sector-average CD and thickness maps were also created on each eye. Results: Wide-field OCTA (8 × 8 mm) in 10 healthy eyes from 10 subjects demonstrated the distribution of the RPCP throughout the posterior pole. RPCP-CD decreases with distance from the disc, but along the arcuate nerve fiber bundles relatively dense (> half maximum density) RPCP extends more than 5 mm from the disc and includes regions superior to and inferior to the macula. The RPCP-CD and NFL thickness are highly correlated (R2 = 0.85, P < 0.001) and fit well with a nonlinear stacked-layer model. The model fit suggests that the RPCP is present when the NFL is thicker than 17.9 µm and reaches a ceiling area density of 84% and that the RPCP has an apparent volume density of 19% at the current instrument transverse resolution. This indicates that capillary overlap can be expected to occur when NFL thickness reaches 40 µm. Conclusions: The wide distribution of dense overlapping RPCP suggests that wider (up to 8 mm vertical and 7 mm horizontal) OCTA scans may be better investigate capillary loss in the early stages of glaucoma or other optic neuropathies.

Quantitative Analysis of En Face Spectral-Domain Optical Coherence Tomography Imaging in Polypoidal Choroidal Vasculopathy.

BACKGROUND AND OBJECTIVE: Spectral-domain optical coherence tomography (SD-OCT) imaging can be used to visualize polypoidal choroidal vasculopathy (PCV) lesions in the en face plane. Here, the authors describe a novel lesion quantification technique and compare PCV lesion area measurements and morphology before and after anti-vascular endothelial growth factor (VEGF) treatment. PATIENTS AND METHODS: Volumetric SD-OCT scans in eyes with PCV before and after induction anti-VEGF therapy were retrospectively analyzed. En face SD-OCT images were generated and a pixel intensity thresholding process was used to quantify total lesion area. RESULTS: Thirteen eyes with PCV were analyzed. En face SD-OCT PCV lesion area quantification showed good intergrader reliability (intraclass correlation coefficient = 0.944). Total PCV lesion area was significantly reduced after anti-VEGF therapy (2.22 mm2 vs. 2.73 mm2; P = .02). The overall geographic pattern of the branching vascular network was typically preserved. CONCLUSION: PCV lesion area analysis using en face SD-OCT is a reproducible tool that can quantify treatment related changes. [Ophthalmic Surg Lasers Imaging Retina. 2017;48:126-133.].

Early microvascular retinal changes in optical coherence tomography angiography in patients with type 1 diabetes mellitus.

PURPOSE: Diabetic retinopathy (DR) can lead to significant vision loss and blindness and has a particularly high prevalence in patients with type 1 diabetes (DM1). In this study, we investigate quantitative differences in optical coherence tomography angiography (OCTA) data between DM1 patients with no or mild signs of retinopathy and non-diabetic subjects. METHODS: Optical coherence tomography angiography (OCTA) imaging was performed on DM1 patients with no or mild nonproliferative diabetic retinopathy and healthy, age-matched controls. Parafoveal vessel density and foveal avascular zone (FAZ) area in the deep capillary plexus (DCP) and superficial capillary plexus (SCP) were calculated with automated quantification software and compared between patient cohorts. RESULTS: A significant decrease in parafoveal vessel density was seen in the DCP of DM1 patients compared to non-diabetic controls (57.0 ± 3.3% versus 60.7 ± 2.4%, p < 0.001). There was no significant difference in SCP parafoveal vessel density, DCP FAZ area, or SCP FAZ area between cohorts. CONCLUSION: M1 patients with no or mild signs of retinopathy have reduced parafoveal vessel density in the DCP on OCTA when compared to non-diabetic controls. These OCTA findings suggest that parafoveal capillary nonperfusion is an early process in DM1-related retinal changes and occurs initially at the level of the DCP. Further investigation is needed to understand the prognostic role of these vascular changes.

LONGITUDINAL MICROPERIMETRY EVALUATION AFTER INTRAVITREAL OCRIPLASMIN INJECTION FOR VITREOMACULAR TRACTION.

PURPOSE: To determine whether improvements in microperimetry testing are associated with anatomic resolution after ocriplasmin treatment in patients with symptomatic vitreomacular adhesion (VMA)/vitreomacular traction and relatively preserved baseline best-corrected visual acuity (BCVA). METHODS: Patients with vitreomacular traction received a single 125-µg intravitreal ocriplasmin injection and were followed longitudinally for 6 months with optical coherence tomography, BCVA testing, and microperimetry. Visual function changes were compared between eyes with and without VMA resolution on optical coherence tomography. RESULTS: Eleven of 16 eyes (68.8%) achieved VMA resolution after treatment. Mean baseline BCVA was relatively good (79 ± 3 Early Treatment Diabetic Retinopathy Study letters; 20/52); no patients had a ≥2-line improvement in BCVA over the 6-month follow-up period. In the group with VMA resolution, mean retinal sensitivity significantly increased in the central 4° (15.2 ± 1.9 dB vs. 18.9 ± 0.7 dB, P < 0.001) when comparing baseline and final follow-up microperimetry testing. No change in mean retinal sensitivity was found in the group without VMA resolution. CONCLUSION: Microperimetry demonstrates a significant gain in retinal sensitivity, particularly in the central 4° area, in eyes with anatomic resolution after treatment of vitreomacular traction with intravitreal ocriplasmin injection, even when no significant gain in BCVA is seen.

LONGITUDINAL QUANTITATIVE EVALUATION OF OUTER RETINAL LESIONS IN ACUTE POSTERIOR MULTIFOCAL PLACOID PIGMENT EPITHELIOPATHY USING OPTICAL COHERENCE TOMOGRAPHY.

PURPOSE: To quantify the external limiting membrane (ELM) disruption and photoreceptor volume changes in eyes with acute posterior multifocal placoid pigment epitheliopathy (APMPPE) using Spectral Domain Optical Coherence Tomography (SD-OCT) at the acute and resolution phases. METHODS: Retrospective study of 10 eyes of 5 patients with APMPPE. Intact ELM and the Bruch's membrane were manually traced using ImageJ software and their lengths from each scan of the Spectral Domain Optical Coherence Tomography macular volume were summed. The ratio of intact ELM length/Bruch's membrane length was calculated. Also, two-dimensional areas of specific regions of interest were demarcated between the intact ELM and Bruch's Membrane in every cross-sectional B-scan. Total volume of photoreceptors was calculated by multiplying the total area by the distance between B-scans. RESULTS: There was a statistically significant increase in ELM/Bruch's membrane ratio (P value = 0.022), mean photoreceptors volume (P value = 0.028), and a significant linear positive correlation between change of intact ELM/Bruch's membrane ratio and percent change of photoreceptor volume (r = 0.927, P value = 0.001) when comparing baseline and final follow-up visit, independent of total follow-up length. CONCLUSION: Using Spectral Domain-Optical Coherence Tomography, we showed that quantitative evaluation of ELM disruption and the volume of photoreceptor recovery can help us to follow the clinical course of APMPPE.

Fundus Autofluorescence Patterns of Submacular Fluid Resolution Following Repair of Macula-Involving Rhegmatogenous Retinal Detachments.

BACKGROUND AND OBJECTIVE: Submacular fluid (SMF) can persist for months to years following rhegmatogenous retinal detachment (RD) repair. The authors' objective was to describe fundus autofluorescence (FAF) and optical coherence tomography (OCT) findings associated with the absorption of persistent submacular fluid (SMF) following RD repair. PATIENTS AND METHODS: Retrospective review of clinical data and FAF and OCT imaging from sequential postoperative visits in a cohort of patients with persistent SMF following RD repair. RESULTS: In 11 of 13 eyes with persistent SMF, patches of decreased FAF signal corresponded to SMF on OCT. In eight eyes, there was a hypo- to hyperautofluorescence transition at the time of SMF resolution. These areas of increased FAF signal correlated with inner segment/outer segment (IS/OS) junction loss on OCT. CONCLUSION: FAF imaging can be informative when following SMF after RD repair; a hypo- to hyper-FAF signal transition correlates with SMF resolution and photoreceptor loss. [Ophthalmic Surg Lasers Imaging Retina. 2016;47:1020-1029.].

Macular sub-layer thinning and association with pulmonary function tests in Amyotrophic Lateral Sclerosis.

Amyotrophic Lateral Sclerosis (ALS) is a complex neurodegenerative disorder that may have anterior visual pathway involvement. In this study, we compare the macular structure of patients with ALS to healthy controls, and examine correlations between macular sub-layer thickness measurements and pulmonary function tests and disease duration. ALS patients underwent optical coherence tomography (OCT) imaging to obtain macular cube scans of the right eye. Macular cube OCT data from age-matched healthy subjects were provided by the OCT reading center. Semi-automated retinal segmentation software was used to quantify macular sub-layers. Pulmonary function tests and time since symptom onset were collected retrospectively from the electronic medical records of ALS patients. Macular retinal nerve fiber layer was significantly thinner in ALS patients compared to healthy controls (P < 0.05). Total macular and other sub-layer thicknesses were not reduced in the ALS cohort. Macular retinal nerve fiber layer thickness positively correlated with forced vital capacity % predicted and forced expiratory volume in 1 second % predicted (P < 0.05). In conclusion, analysis of OCT measurements supports the involvement of the anterior visual pathway in ALS. Subtle structural thinning in the macular retinal nerve fiber layer correlates with pulmonary function tests.

A case of recurrent, self-inflicted handheld laser retinopathy.

A 17-year-old young man presented with vision loss and discrete, bilateral foveal lesions. The patient returned 6 weeks later with worsening vision, prominent bilateral retinal lesions, and a full-thickness macular hole in the right eye consistent with recurrent self-inflicted handheld laser retinopathy. After instructing the family to remove the patient's access to laser pointers, follow-up examination revealed spontaneous closure of the macular hole but minimal vision improvement. Recurrent ocular exposure to handheld lasers can masquerade as an organic process in patients who withhold pertinent history. Clinicians should rely on the distinguishing features seen on examination and multimodal imaging to make the diagnosis of handheld laser retinopathy.