IDIOPATHIC PENETRATION OF CILIA INTO THE POSTERIOR SEGMENT PRESENTING AS SECTORAL SCLERITIS WITH PROGRESSIVE INTRAOCULAR INFLAMMATION.

PURPOSE: To report two cases of idiopathic intraocular cilia presenting as sectoral scleritis with progressive intraocular inflammation. METHODS: Both patients were treated with intravitreal antibiotics and underwent pars plana vitrectomy where the cilia were removed and identified on histopathology. RESULTS: One patient developed a retinal detachment while being treated for presumed endophthalmitis. The intraocular cilium was discovered during pars plana vitrectomy. In the second case, the cilium was detected on dilated fundus exam and was believed to be the cause of the patient's scleritis and vitritis. Therapeutic vitrectomy was performed. In both cases, the cilia were positively identified on histopathology. CONCLUSION: Idiopathic intraocular penetration of cilia should be considered in the differential diagnosis of sectoral scleritis with progressive intraocular inflammation.

Refractive Error and Retinopathy Outcomes in Type 1 Diabetes: The Diabetes Control and Complications Trial/Epidemiology of Diabetes Interventions and Complications Study.

PURPOSE: To determine the relationship between refractive error and diabetic retinopathy (DR). DESIGN: Clinical trial. PARTICIPANTS: Type I diabetes individuals with serial refractive error and DR stage measurements over 30 years in the Diabetes Control and Complications Trial (DCCT) and Epidemiology of Diabetes Interventions and Complications (EDIC) follow-up study. METHODS: Stage of DR was measured every 6 months from standard fundus photographs, and refractive error was measured annually during the 6.5 years of DCCT; then, both were staggered every fourth year during EDIC with the full cohort measured at EDIC years 4 and 10. Outcomes of DR were 2- or 3-step progression, presence of proliferative DR (PDR), clinically significant macular edema (CSME), diabetic macular edema (DME), or ocular surgery. Myopia, emmetropia, and hyperopia were defined as a spherical equivalent of ≤-0.5, >-0.5 and <0.5, and ≥0.5, respectively. MAIN OUTCOME MEASURES: For each outcome separately, Cox proportional hazard (PH) models assessed the association between the refractive error status and the subsequent risk of that outcome, both without and with adjustment for potential risk factors. RESULTS: Hyperopia was associated with a higher risk of 2-step progression (hazard ratio [HR], 1.29; 95% confidence interval [CI], 1.05-1.59), 3-step progression (HR, 1.35; 95% CI, 1.05-1.73), and PDR (HR, 1.40; 95% CI, 1.02-1.92) compared with emmetropia in unadjusted models. These associations remained significant after adjustment for DCCT treatment group, cohort, age, sex, smoking, duration of diabetes, systolic and diastolic blood pressures, pulse, low-density lipoprotein, high-density lipoprotein, triglycerides, albumin excretion rate, and DCCT/EDIC mean updated hemoglobin A1c (HbA1c) (2-step progression: HR, 1.28; 95% CI, 1.03-1.58; 3-step progression: HR, 1.30; 95% CI, 1.00-1.68; PDR: HR, 1.38; 95% CI, 1.00-1.90). Myopia was not associated with any of the 5 DR outcomes in the unadjusted models and only marginally associated with 2-step progression (HR, 1.11; 95% CI, 1.00-1.24) in the adjusted models. CONCLUSIONS: Myopia is not associated with DR progression risk. Hyperopia is an independent risk factor for 2-step and 3-step DR progression and PDR.

Quantitative assessment of choriocapillaris flow deficits in diabetic retinopathy: A swept-source optical coherence tomography angiography study.

PURPOSE: To quantitatively assess choriocapillaris (CC) flow deficits in eyes with diabetic retinopathy (DR) using swept-source optical coherence tomography angiography (SS-OCTA). METHODS: Diabetic subjects with different stages of DR and age-matched healthy subjects were recruited and imaged with SS-OCTA. The en face CC blood flow images were generated using previously published and validated algorithms. The percentage of CC flow deficits (FD%) and the mean CC flow deficit size were calculated in a 5-mm-diameter circle centered on the fovea from the 6×6-mm scans. RESULTS: Forty-five diabetic subjects and 27 control subjects were included in the study. The CC FD% in diabetic eyes was on average 1.4-fold greater than in control eyes (12.34±4.14% vs 8.82±2.61%, P < 0.001). The mean CC FD size in diabetic eyes was on average 1.4-fold larger than in control eyes (2151.3± 650.8µm2 vs 1574.4±255.0 µm2, P < 0.001). No significant difference in CC FD% or mean CC FD size was observed between eyes with nonproliferative DR and eyes with proliferative DR (P = 1.000 and P = 1.000, respectively). CONCLUSIONS: CC perfusion in DR can be objectively and quantitatively assessed with FD% and FD size. In the macular region, both CC FD% and CC FD size are increased in eyes with DR. SS-OCTA provides new insights for the investigations of CC perfusion status in diabetes in vivo.

Optical coherence tomography findings in Cohen syndrome.

Cohen syndrome is a rare disease that causes myopia and retinal degeneration in the setting of developmental delay and characteristic craniofacial features. We report optical coherence tomography (OCT) abnormalities in 4 patients with Cohen syndrome, 2 of whom have longitudinal follow-up. All subjects had schisis-like changes, with cystoid spaces in the inner retina as well as diffuse outer retinal atrophy sparing the subfoveal region. Ophthalmologic findings in 1 patient led to the work-up that resulted in a diagnosis of Cohen syndrome, suggesting that characteristic retinal abnormalities visualized by fundus examination and OCT may represent distinguishing features of this syndrome.

New developments in angiography for the diagnosis and management of diabetic retinopathy.

The most common microvascular complication of diabetes is diabetic retinopathy, the leading cause of blindness in adults of working age. Our understanding of the vascular changes in diabetic retinopathy was enhanced by the demonstration of fluorescein angiography (FA) in the human retina for the first time in 1961. It was subsequently integrated with digital fundoscopic imaging to become an invaluable technique in evaluation of the retinal vasculature. The recent development of OCT-angiography (OCT-A) has revolutionized the clinician's ability to examine the retinal vasculature without the need for injection of a contrast dye. By coupling OCT, which can provide noninvasive cross-sectional imaging of the central retina, with angiography in OCT-A, one can reveal retinal perfusion by allowing visualization of the depth-resolved retinal capillary plexus. OCT-A has allowed for more precise delineation of changes in the retinal microvasculature, specifically the alterations of retinal vasculature and loss of capillary perfusion from chronic microvascular occlusion in diabetic retinopathy.

Prognostic Utility of Whole-Genome Sequencing and Polymerase Chain Reaction Tests of Ocular Fluids in Postprocedural Endophthalmitis.

PURPOSE: To associate detection of potential pathogen DNA in endophthalmitis with clinical outcomes. DESIGN: Prospective cohort study. METHODS: Patients in whom endophthalmitis was diagnosed following an intraocular procedure were recruited. Clinical outcome data from baseline, week-1, month-1, and month-3 visits were collected. Intraocular biopsy samples were cultured by standard methods. Quantitative polymerase chain reaction (qPCR) was performed for specific pathogens and whole-genome sequencing (WGS). RESULTS: A total of 50 patients (mean age 72 years old; 52% male) were enrolled. Twenty-four cases were culture-positive and 26 were culture-negative. WGS identified the cultured organism in 76% of culture-positive cases and identified potential pathogens in 33% of culture-negative cases. Month-1 and -3 visual acuities did not vary by pathogen-positive versus pathogen-negative cases as detected by either culture or WGS. Visual outcomes of Staphylococcus epidermidis endophthalmitis were no different than those of pathogen-negative cases, whereas the patients infected with other pathogens showed worse outcome. Higher baseline bacterial DNA loads of bacteria other than those of S epidermidis detected by WGS were associated with worse month-1 and -3 visual acuity, whereas the S epidermidis loads did not appear to influence outcomes. Torque teno virus (TTV) and Merkel cell polyomavirus (MCV) were detected by qPCR in 49% and 19% of cases, respectively. Presence of TTV at presentation was associated with a higher rate of secondary pars plana vitrectomy (P = .009) and retinal detachment (P = .022). CONCLUSIONS: The presence and higher load of bacteria other than S epidermidis detected by WGS or DNA from TTV by qPCR in ocular fluids is associated with worse outcomes in post-procedure endophthalmitis.

ADVERSE EVENTS OF THE ARGUS II RETINAL PROSTHESIS: Incidence, Causes, and Best Practices for Managing and Preventing Conjunctival Erosion.

PURPOSE: To analyze and provide an overview of the incidence, management, and prevention of conjunctival erosion in Argus II clinical trial subjects and postapproval patients. METHODS: This retrospective analysis followed the results of 274 patients treated with the Argus II Retinal Prosthesis System between June 2007 and November 2017, including 30 subjects from the US and European clinical trials, and 244 patients in the postapproval phase. Results were gathered for incidence of a serious adverse event, incidence of conjunctival erosion, occurrence sites, rates of erosion, and erosion timing. RESULTS: Overall, 60% of subjects in the clinical trial subjects versus 83% of patients in the postapproval phase did not experience device- or surgery-related serious adverse events. In the postapproval phase, conjunctival erosion had an incidence rate of 6.2% over 5 years and 11 months. In 55% of conjunctival erosion cases, erosion occurred in the inferotemporal quadrant, 25% in the superotemporal quadrant, and 20% in both. Sixty percent of the erosion events occurred in the first 15 months after implantation, and 85% within the first 2.5 years. CONCLUSION: Reducing occurrence of conjunctival erosion in patients with the Argus II Retinal Prosthesis requires identification and minimization of risk factors before and during implantation. Implementing inverted sutures at the implant tabs, use of graft material at these locations as well as Mersilene rather than nylon sutures, and accurate Tenon's and conjunctiva closure are recommended for consideration in all patients.

Effect of prior glaucoma surgery on intraocular pressure immediately after anti-vascular endothelial growth factor injection.

BACKGROUND: To characterize how prior incisional glaucoma surgery affects the intraocular pressure (IOP) elevation immediately following intravitreal anti-VEGF injections (IVI). METHODS: Single institution, experimental study. Patients with a history of incisional glaucoma surgery who were receiving anti-VEGF injections were recruited as well as control eyes. Pre- and post-injection IOP measurements were compared as well as time to recovery to within 5 and 10 mmHg of baseline IOP. RESULTS: Ten eyes with a history of glaucoma surgery and 29 control eyes receiving anti-VEGF injections were included. The most common indication for intravitreal anti-VEGF injection was proliferative diabetic retinopathy in both surgical and control eyes (50% vs 45%, p = 1.00). Post-injection IOP was significantly decreased compared to baseline IOP after anti-VEGF injection in surgical versus control eyes (26.5 ± 8.9 mmHg vs 44.2 ± 8.5 mmHg, respectively, p < 0.001). The mean change in IOP following intravitreal anti-VEGF injection was lower in surgical eyes (10.7 ± 6.6 mmHg vs 28.6 ± 8.3 mmHg, p < 0.001). The mean time for the IOP to return to within 10 mmHg of pre-injection IOP was less in surgical eyes (5.2 ± 4.1 min vs 13.3 ± 7.6 min, p = 0.002). CONCLUSIONS: Eyes with prior incisional glaucoma surgery demonstrated a significantly lower post-injection IOP elevation and a faster recovery to within 10 mmHg of their pre-injection IOP. Incisional glaucoma surgery may be considered for patients where the attenuation of post-injection IOP elevation is needed and other less invasive measures have failed.

CAPTCHA as a Visual Performance Metric in Active Macular Disease.

PURPOSE: CAPTCHA (completely automated public turing test to tell computers and humans apart) was designed as a spam prevention test. In patients with visual impairment, completion of this task has been assumed to be difficult; but to date, no study has proven this to be true. As visual function is not well measured by Snellen visual acuity (VA) alone, we theorized that CAPTCHA performance may provide additional information on macular disease-related visual dysfunction. METHODS: This was designed as a pilot study. Active disease was defined as the presence of either intraretinal fluid (IRF) or subretinal fluid (SRF) on spectral-domain optical coherence tomography. CAPTCHA performance was tested using 10 prompts. In addition, near and distance VA, contrast sensitivity, and reading speed were measured. Visual acuity matched pseudophakic patients were used as controls. Primary outcome measures were average edit distance and percent of correct responses. RESULTS: 70 patients were recruited: 33 with active macular disease and 37 control subjects. Contrast sensitivity was found to be significantly different in both the IRF (p < 0.01) and SRF groups (p < 0.01). No significant difference was found comparing the odds ratio of average edit distance of active disease (IRF, SRF) vs. control (OR 1.09 (0.62, 1.90), 1.10 (0.58, 2.05), p=0.77, 0.77) or percent correct responses of active disease vs. control (OR 0.98 (0.96, 1.01), 1.09 (0.58, 2.05), p=0.22, 0.51) in CAPTCHA testing. The goodness of fit using logistic regression analysis for the dependent variables of either IRF or SRF did not improve accounting for average edit distance (p=0.49, p=0.27) or percent correct (p=0.89, p=0.61). CONCLUSIONS: Distance VA and contrast sensitivity are positively correlated with the presence of IRF and SRF in active macular disease. CAPTCHA performance did not appear to be a significant predictor of either IRF or SRF in our pilot study.

Retinal Anatomy and Electrode Array Position in Retinitis Pigmentosa Patients After Argus II Implantation: An International Study.

PURPOSE: To assess the retinal anatomy and array position in Argus II retinal prosthesis recipients. DESIGN: Prospective, noncomparative cohort study. METHODS: Setting: International multicenter study. PATIENTS: Argus II recipients enrolled in the Post-Market Surveillance Studies. PROCEDURES: Spectral-domain optical coherence tomography images collected for the Surveillance Studies (NCT01860092 and NCT01490827) were reviewed. Baseline and postoperative macular thickness, electrode-retina distance (gap), optic disc-array overlap, and preretinal membrane presence were recorded at 1, 3, 6, and 12 months. MAIN OUTCOME MEASURES: Axial retinal thickness and axial gap along the array's long axis (a line between the tack and handle); maximal retinal thickness and maximal gap along a B-scan near the tack, midline, and handle. RESULTS: Thirty-three patients from 16 surgical sites in the United States and Germany were included. Mean axial retinal thickness increased from month 1 through month 12 at each location, but reached statistical significance only at the array midline (P = .007). The rate of maximal thickness increase was highest near the array midline (slope = 6.02, P = .004), compared to the tack (slope = 3.60, P < .001) or the handle (slope = 1.93, P = .368). The mean axial and maximal gaps decreased over the study period, and the mean maximal gap size decrease was significant at midline (P = .032). Optic disc-array overlap was seen in the minority of patients. Preretinal membranes were common before and after implantation. CONCLUSIONS: Progressive macular thickening under the array was common and corresponded to decreased electrode-retina gap over time. By month 12, the array was completely apposed to the macula in approximately half of the eyes.

Decreased severity of age-related macular degeneration in amblyopic eyes.

AIM: To evaluate whether people with age-related macular degeneration (AMD) and a history of amblyopia have equal severity of AMD in both eyes. METHODS: Billing records were used to locate all people with a history of amblyopia and AMD evaluated between 1 January 2003 and 1 June 2015 at a single ophthalmology institute. Two ophthalmic graders blinded to amblyopia status determined the severity of AMD in each eye using fundus photos and a validated grading scale. RESULTS: A total of 14 people were found to have AMD and a documented history of amblyopia. Average patient age was 77.0 years and average best corrected visual acuity was 20/160 in eyes with a history of amblyopia and 20/40 in fellow eyes without amblyopia. Eyes with a history of amblyopia were found to have a lower AMD severity score (mean lower score: -1.38; paired t-test P=0.019). Of the 11 people with asymmetric disease severity, 10 individuals had worse AMD in the non-amblyopic eye while one person had worse AMD in the amblyopic eye (P=0.0067). CONCLUSIONS: Our pilot study suggests that eyes with a history of amblyopia may manifest decreased severity of AMD compared with non-ambylopic eyes in the same patient. Further research is warranted to investigate this clinical observation.

VISUAL AND ANATOMICAL OUTCOMES AFTER DIABETIC TRACTION AND TRACTION-RHEGMATOGENOUS RETINAL DETACHMENT REPAIR.

PURPOSE: To evaluate visual and anatomical outcomes of diabetic tractional retinal detachment repaired with pars plana vitrectomy. METHODS: Operative records were used to retrospectively identify all patients with tractional retinal detachments secondary to proliferative diabetic retinopathy surgically repaired with pars plana vitrectomy between November 1, 2009, and January 1, 2015 at the LAC + USC (Los Angeles County + University of Southern California) Medical Center. RESULTS: A total of 403 eyes with diabetic tractional retinal detachment in 359 patients were included. Successful reattachment of the retina was achieved in 87.6% of eyes after one surgery and 92.6% of eyes at the final follow-up. Best-corrected visual acuity at the final follow-up improved two or more lines in 56.3% of eyes, was stable in 23.8% of eyes, and decreased two or more lines in 19.9% of eyes. Eyes repaired with 23-gauge and 25-gauge vitrectomy systems had similar success rates as eyes treated with 20-gauge instrumentation (P = 0.73). Eyes receiving silicone oil tamponade had lower single-surgery reattachment rates (77.6% vs. 87.6%; P = 0.013), lower reattachment rates at the final follow-up (85.7% vs. 92.6%; P = 0.048), and higher rates of vision loss (34.7% vs. 19.9%; P < 0.0001) but were more likely to have concurrent rhegmatogenous detachment (47.0% vs. 21.3%; P < 0.0001) and macula involving detachment (74.5% vs. 60.0%; P < 0.0001). CONCLUSION: In this large, single-center retrospective study of patients with advanced diabetic tractional retinal detachment, vitrectomy achieved excellent anatomical outcome and improved or stabilized vision in 80.1% of eyes. Smaller gauge vitrectomy systems were found to have similar outcomes to 20-gauge instrumentation.

Atypical chronic central serous chorioretinopathy with cystoid macular edema: Therapeutic response to medical and laser therapy.

PURPOSE: To describe an atypical case of chronic central serous chorioretinopathy (CSCR). METHODS: A 58-year-old man with longstanding, bilateral visual impairment was self-referred for a second opinion. RESULTS: Findings by direct ophthalmoscopy, optical coherence tomography, fluorescein angiography, and fundus autofluorescence (FAF) were suggestive of atypical, chronic CSCR. Treatment with oral anti-mineralocorticoids resulted in moderate improvement, and photodynamic therapy (PDT) had minimal effect. CONCLUSION: Chronic CSCR may lack cardinal features of CSCR. Once retinal degenerative changes ensue, current treatments may not be effective in improving anatomical and visual outcomes in patients with chronic CSCR.

Quantitative microvascular analysis of retinal venous occlusions by spectral domain optical coherence tomography angiography.

PURPOSE: To quantitatively evaluate the retinal microvasculature in human subjects with retinal venous occlusions (RVO) using optical coherence tomography angiography (OCTA). DESIGN: Retrospective, cross-sectional, observational case series. PARTICIPANTS: Sixty subjects (84 eyes) were included (20 BRVO, 14 CRVO, 24 unaffected fellow eyes, and 26 controls). METHODS: OCTA was performed on a prototype, spectral domain-OCTA system in the 3x3mm central macular region. Custom software was used to quantify morphology and density of retinal capillaries using four quantitative parameters. The vasculature of the segmented retinal layers and nonsegmented whole retina were analyzed. MAIN OUTCOME MEASURES: Fractal dimension (FD), vessel density (VD), skeletal density (SD), and vessel diameter index (VDI) within the segmented retinal layers and nonsegmented whole retina vasculature. RESULTS: Nonsegmented analysis of RVO eyes demonstrated significantly lower FD (1.64±0.01 vs 1.715±0.002; p<0.001), VD (0.32±0.01 vs 0.432±0.002; p<0.001), and SD (0.073±0.004 vs 0.099±0.001; p<0.001) compared to controls. Compared to the fellow eye, FD, VD and SD were lower (p<0.001), and VDI was higher (p<0.001). FD, VD, and SD progressively decreased as the extent (or type) of RVO increased (control vs BRVO vs CRVO; p<0.001). In the unaffected fellow eye FD, VD and SD showed significant differences when compared to control eyes or affected RVO eyes (p<0.001). CONCLUSIONS: Quantitative OCTA of the central 3x3mm macular region demonstrates significant differences in capillary density and morphology among subjects with BRVO and CRVO compared to controls or unaffected fellow eyes in all vascular layers. The unaffected fellow eyes also demonstrate significant differences when compared to controls. OCTA allows for noninvasive, layer-specific, quantitative evaluation of RVO-associated microvascular changes.

Sustained Resolution of Macular Retinoschisis After Trabeculectomy in a Patient With Progressive Glaucoma.

PURPOSE: To evaluate the clinical characteristics of a patient with primary open-angle glaucoma in whom macular retinoschisis resolved completely after trabeculectomy consistently lowered intraocular pressure (IOP). METHODS: A single case report. RESULTS: We report a case of retinoschisis involving the macula in a patient with primary open-angle glaucoma in the absence of myopic maculopathy, optic nerve anomaly, or x-linked retinoschisis. The patient's glaucoma was associated with progressive visual field loss in the setting of IOP fluctuations related to posture. A trabeculectomy reduced IOP and posture-related IOP fluctuations with subsequent resolution of macular retinoschisis. In the 1-year postoperative period following trabeculectomy, the patient has remained without retinoschisis and visual fields have been stable. CONCLUSIONS: Improved IOP control resulting in resolution of retinoschisis may distinguish retinoschisis associated with glaucoma from other forms of retinoschisis.

Improvements in vision-related quality of life in blind patients implanted with the Argus II Epiretinal Prosthesis.

BACKGROUND: The purpose of this analysis is to report the change in quality of life (QoL) after treatment with the Argus II Epiretinal Prosthesis in patients with end-stage retinitis pigmentosa. METHODS: The Vision and Quality of Life Index (VisQoL) was used to assess changes in QoL dimensions and overall utility score in a prospective 30-patient single-arm clinical study. VisQoL is a multi-attribute instrument consisting of six dimensions (injury, life, roles, assistance, activity and friendship) that may be affected by visual impairment. Within each dimension, patients were divided into two groups based on how much their QoL was affected by their blindness at baseline (moderate/severe or minimal). Outcomes were compared within each dimension sub-group between baseline and the combined follow-up periods using the Friedman test. In addition, data from the six dimensions were combined into a single utility score, with baseline data compared to the combined follow-up periods. RESULTS: Overall, 80 per cent of the patients reported difficulty in one or more dimensions pre-implant. Composite VisQoL utility scores at follow-up showed no statistically significant change from baseline; however, in three of the six VisQoL dimensions (injury, life and roles), patients with baseline deficits showed significant and lasting improvement after implantation with Argus II. In two of the three remaining dimensions (assistance and activity), data trended toward an improvement. In the final VisQoL dimension (friendship), none of the patients reported baseline deficits, suggesting that patients had largely adjusted to this attribute. CONCLUSION: Patients whose vision negatively affected them with respect to three VisQoL dimensions (that is, getting injured, coping with the demands of their life and fulfilling their life roles) reported significant improvement in QoL after implantation of the Argus II retinal prosthesis. Furthermore, the benefit did not deteriorate at any point during the 36-month follow-up, suggesting a long-term, durable improvement.