Animal studies suggest that the retinal dysfunction in diabetic subjects that precedes overt clinical vasculopathy may be due to a retinal dopamine deficit. We analyzed levels of dopamine (DA) and its primary metabolite, 3,4-dihydroxyphenylacetic acid (DOPAC), in the vitreous of diabetic and non-diabetic human subjects. Adult patients undergoing pars plana vitrectomy for non-hemorrhagic indications were prospectively recruited from the Emory Eye Center in Atlanta, GA. Vitreous samples were analyzed using high performance liquid chromatography (HPLC) to measure levels of DOPAC and DA in the vitreous specimens. Vitreous samples from 9 diabetic patients and 20 from non-diabetic patients were analyzed. No eyes had apparent diabetic retinopathy. Mean normalized DA concentration in vitreous of diabetic subjects was 0.76 ± 0.12 pg/μL vs. 0.73 ± 0.08 pg/μL in non-diabetic vitreous (p = 0.849). DOPAC concentration was 8.84 ± 0.74 pg/μL in vitreous of diabetic subjects vs. 9.22 ± 0.56 pg/μL in vitreous of non-diabetic subjects (p = 0.691). No difference was observed in the concentrations of DA and DOPAC in the vitreous of people without diabetes compared to those with diabetes without retinopathy.

Purpose: This study examines the impact of corneal surface lubricants used during pars plana vitrectomy on corneal edema. Methods: This prospective, observational, clinical study occurred at an academic institution. Participants were individuals aged 18 years and older who had already consented to undergo pars plana vitrectomy, without pre-existing corneal pathology. A corneal lubricant was chosen by the surgeon. Corneal thickness was measured preoperatively and postoperatively using pachymetry and anterior segment optical coherence tomography (AS-OCT). Main outcome measure was change in corneal thickness as measured by pachymetry. Results: Forty-one patients completed the study protocol. The 23 subjects in the SHCS group had a significantly smaller increase in corneal thickness as measured by pachymetry compared with the 18 subjects in the HPMC group (29.9 mm vs. 58.1 mm, P value 0.02). When measured by anterior segment optical coherence tomography, the SHCS group had a smaller increase in corneal thickness compared with the HPMC group (0.04 mm vs. 0.06 mm, P value 0.09) but did not reach significance. Conclusion: SHCS is associated with reduced postoperative increase in corneal pachymetry as compared to HPMC.

Background/Aims A series at a single clinical centre recently demonstrated an association between the interstitial cystitis drug pentosan polysulfate sodium (PPS) and a vision-threatening pigmentary maculopathy. The aim of this study was to determine if an association exists between PPS use and macular disease in a large national cohort. Methods A retrospective, matched cohort study using data from a large US medical claims database from 2002 to 2016 was performed. A total of 3012 and 1604 PPS users were compared with 15 060 and 8017 matched controls at 5 and 7 years, respectively. The primary outcome measures included (1) any new diagnosis of a hereditary or secondary pigmentary maculopathy (atypical maculopathy outcome), and (2) any new diagnosis of dry age-related macular degeneration (AMD) or drusen in addition to the aforementioned diagnoses (atypical maculopathy+AMD outcome). Results At the 5-year and 7-year follow-up, 9 (0.3%) and 10 (0.6%) PPS patients progressed to the atypical maculopathy outcome compared with 32 (0.2%) and 25 (0.3%) control patients, respectively. 103 (3.4%) and 87 (5.4%) PPS patients developed the atypical maculopathy+AMD outcome compared with 440 (2.9%) and 328 (4.1%) control patients at 5 and 7 years, respectively. At 5 years, multivariate analysis showed no significant association (p>0.13). At 7 years, PPS users had significantly increased odds of having the atypical maculopathy+AMD outcome (OR=1.41, 95% CI 1.09 to 1.83, p=0.009). Conclusions PPS exposure was associated with a new diagnosis of macular disease at the 7-year follow-up in a large national cohort.

PURPOSE: To investigate patient-reported visual function among individuals taking pentosan polysulfate (PPS) for interstitial cystitis. METHODS: A 27-item online survey was distributed to an international mailing list of individuals with interstitial cystitis in November 2018. Demographic characteristics, PPS exposure history, subjective visual function, and previous macular diagnoses were queried. The impact of PPS use, grouped by tertile of cumulative exposure, on visual function and macular diagnoses was assessed with multivariate logistic regression. RESULTS: The survey was completed by 912 respondents. Eight hundred and sixty-one (96.4%) were women, and the median age was 55 [interquartile range (IQR), 45-64 years]. Among PPS users, the median exposure was 547.5 g (IQR, 219-1,314 g). Respondents in the highest PPS exposure tertile were more likely to report difficulty with reading small print [adjusted odds ratio 2.29, 95% confidence interval (CI) 1.15-4.57] and to have a diagnosis of macular degeneration and/or pigmentary maculopathy (adjusted odds ratio 2.41, 95% CI 1.44-4.03) than unexposed respondents. CONCLUSION: In this large sample of individuals with interstitial cystitis, those in the highest PPS exposure category were more likely to have difficulties reading small print and to report a previous diagnosis of macular disease. Further study of objective measures of visual function in PPS users is warranted.

PURPOSE. Individuals with pentosan polysulfate sodium (PPS) maculopathy commonly report symptoms of prolonged dark adaptation and difficulty reading. We hypothesize that PPS maculopathy causes degradation of visual function not fully captured with visual acuity testing. METHODS. Subjects with PPS maculopathy underwent multimodal evaluation of retinal structure and function. Structural changes were graded as moderate or advanced. Patient-reported visual function was assessed with the National Eye Institute Visual Function Questionnaire 39 (NEI-VFQ-39) and Low Luminance Questionnaire (LLQ). Objective functional evaluations included Early Treatment of Diabetic Retinopathy Study (ETDRS) best-corrected visual acuity (BCVA), Pelli-Robson contrast sensitivity, mesopic microperimetry, and dark adaptometry. Functional testing results were correlated with structural disease category. RESULTS. Thirteen patients (26 eyes), median age 62 years (range, 37-76), completed the study. Median ETDRS letter score was 82 (Snellen equivalent 20/25). Median NEI-VFQ-39 and LLQ composite scores were 65 (range, 33-88) and 41 (range, 20-92), respectively. Median contrast sensitivity was 1.65 (range, 0.15-1.95), and median mesopic microperimetry average thresholds and percent reduced thresholds were 26 decibels (range, 0.4-28.6) and 21.6% (range, 0-100%), respectively. Median rod intercept time was 14.1 minutes (range, 4.4-20.0). Eyes with advanced disease based on retinal structure had significantly worse retinal function for several testing modalities. CONCLUSIONS. PPS maculopathy causes considerable visual function degradation that is not fully captured with BCVA testing. There was good correlation between other measures of visual function and disease severity. These findings deepen our concern regarding this patient safety issue.

To report a rare presentation of the pericentral pattern of hydroxychloroquine (HCQ) retinal toxicity in a Caucasian female. Observations: The patient presented with 20 years of exposure to HCQ, at a daily dose of 5.2mg/kg of actual body weight, and manifested a pericentral-only phenotype of HCQ toxicity, as demonstrated with detailed structural and functional testing. Conclusions and importance: Although rare, the pericentral pattern of HCQ toxicity may occur in Caucasian patients in the absence of paracentral changes.

Objective: To describe presenting clinical features and surgical techniques that are associated with successful surgical repair of pediatric rhegmatogenous retinal detachment (RRD). Design: Retrospective interventional case series. Subjects: 212 eyes of 191 patients, aged 0–18 years, undergoing surgical repair for RRD between 2001 and 2015 with a minimum follow up of 3 months. Methods: Patients were divided into three age groups (0–6 years, 7–12 years, 13–18 years) and comparisons were made using bivariate and multivariable generalized estimating equation models. A mixed means model was used to examine visual acuity in each age group over time. Main Outcome Measures: Complete reattachment of the retina at final follow up. Results: Of a total of 212 eyes, 166 (78%) achieved total reattachment at final follow up. Mean follow up was 36.3 months. RRD associated with Stickler syndrome was more likely to occur in the younger cohorts (odds ratio [OR] 0.45, 95 % confidence interval [CI] 0.22 – 0.91), while RRD associated with blunt trauma was more likely to occur in the oldest cohort (OR 2.3, 95% CI 1.2–4.4). Subtotal RRD was more likely to be successfully repaired than total RRD (OR 3.6, 95% CI 1.5 – 8.4, p = 0.0100), and eyes with previous vitreoretinal surgery were less likely to have successful repair (OR 0.30, 95% CI 0.12 – 0.78, p = 0.0258). There was no significant difference between age groups in the rate of surgical success (p = 0.55). There was a significantly higher success rate with primary scleral buckle (SB) (63%, OR 2.2, 95% CI 1.1–4.5) and combined scleral buckle/vitrectomy (SB/PPV) (68%, OR 2.3 95% CI 1.1–5.1) compared to vitrectomy (PPV) alone (51%). Conclusions: Most pediatric patients with RRD achieved complete reattachment with surgery. Success was more common in patients with a subtotal RRD at presentation. Previous vitreoretinal surgery was a risk factor for failure. Younger patients were more likely to present with RRD involving the macula but there was no difference between age groups in successful reattachment at final follow up. Primary PPV had a lower rate of success than SB or combined SB/PPV.

Autosomal dominant neovascular inflammatory vitreoretinopathy (ADNIV) is a rare autoimmune condition that typically presents as progressive uveitis and vitreoretinal degeneration between the second and third decades of life. Though traditionally attributed to inherited mutations of the CAPN5 gene, few reports of de novo variants exist. This report of vision and hearing loss in a 3 year-old girl describes the youngest documented case of ADNIV due to a de novo pathogenic c.865C>T (p.Arg289Trp) CAPN5 variant, illustrating the early stages of this enigmatic disease process.

Objective: To assess long-term effects of genotype on chorioretinopathy severity in subjects with mitochondrial trifunctional protein (MTP) disorders. Design: Retrospective case series. Participants: Consecutive patients with MTP disorders evaluated at a single center from 1994 to 2015, including 18 subjects with long-chain 3-hydroxyacyl-CoA dehydrogenase deficiency (LCHADD) and 3 subjects with trifunctional protein deficiency (TFPD). Methods: Local records from all visits were reviewed. Every subject underwent complete ophthalmic examination and was evaluated by a metabolic physician and dietitian. Nine subjects underwent ancillary fundoscopic imaging including optical coherence tomography (OCT) and OCT angiography. Main Outcome Measures: The primary outcome measure was best-corrected visual acuity (logMAR) at the final visit. Secondary outcome measures included spherical equivalent refraction, electroretinogram (ERG) b-wave amplitudes, and qualitative imaging findings. Results: Subjects were followed for a median of 5.6 years (range 0.3–20.2). The median age of LCHADD subjects at initial and final visits was 2.3 and 11.9 years, while the median age for TFPD subjects at initial and final visits was 4.7 and 15.5 years. Four long-term survivors over the age of 16 years were included (three subjects with LCHADD and one subject with TFPD). LCHADD subjects demonstrated a steady decline in visual acuity from an average logMAR of 0.23 (Snellen equivalent 20/34) at baseline to 0.42 (Snellen equivalent 20/53) at the final visit, whereas TFPD patients maintained excellent acuity throughout follow up. Subjects with LCHADD, but not TFPD, showed an increasing myopia with a mean decrease in spherical equivalent refraction of 0.24 diopters per year. Multimodal imaging demonstrated progressive atrophy of the outer retina in LCHADD, often preceded by the formation of outer retinal tubulations and choriocapillaris dropout. ERG findings support the more severe clinical profile of LCHADD subjects compared with TFPD; the function of both rods and cones are diffusely attenuated in LCHADD but within normal limits for TFPD subjects. Conclusions: Despite improved survival with early diagnosis, medical management, and dietary treatment, subjects with the LCHADD subtype of MTP disorder continue to develop visually disabling chorioretinopathy. Multimodal imaging is most consistent with choriocapillaris loss exceeding photoreceptor loss.