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IntroductionTreatment of the anterior segment problems in cystinosis is challenging as oral cysteamine is ineffective in the treatment of corneal problems because of its avascular structure. Although cysteamine eye drops have been formulated to counter this issue, the stability of cysteamine in these off-licensed formulations and treatment compliance are major problems. The aim of this retrospective study was to determine the efficacy of a compounded preparation of aqueous 0.5% cysteamine eye drops in the management of corneal complications of cystinosis.MethodsData of patients attending the multidisciplinary cystinosis clinic at the University Hospitals Leuven, Belgium between January 2015 and December 2018 were analyzed. All cystinosis patients were treated with the compounded preparation of aqueous 0.5% cysteamine eye drops and oral cysteamine.ResultsA total of 12 patients were treated with the compounded preparation of aqueous 0.5% cysteamine eye drops, of whom 75% were aged > 18 years (n = 9). The mean instillation frequency of the cysteamine eye drops was 3.3 drops/eye per day, and the mean number of hospital visits was two per year. All patients showed photophobia, > 30% corneal infiltration, blepharospasm, eye pain and conjunctival hyperemia during the study period. None of these symptoms improved with treatment with aqueous compounded 0.5% cysteamine eye drops. The corneal cystine crystal score was ≥ 2 in all patients at the last  visit.ConclusionTreatment with the compounded preparation of aqueous 0.5% cysteamine eye drops, combined with oral cysteamine, was not effective in reducing corneal cystine crystal deposition and other ocular symptoms in these patients with cystinosis.FundingRecordati Rare Diseases.

Purpose This retrospective observational study reports early results on a cohort of neovascular age-related macular degeneration (nAMD) patients switched to brolucizumab, a recently approved anti-vascular endothelial growth factor (anti-VEGF). Patients and Methods We evaluated best-corrected visual acuity (BCVA), treatment interval, central subfield retinal thickness (CST) and the presence of intra-retinal (IRF), subretinal (SRF) and/or sub-retinal pigment epithelium (sub-RPE) fluid on optical coherence tomography (OCT). Concurrently, patients were carefully examined for signs of intra-ocular inflammation (IOI) and other adverse events. Results Seventeen patients (19 eyes) were included. The difference in BCVA at baseline compared to the last examination following brolucizumab injection was not statistically significant (Wilcoxon signed-rank test, p=0.247). Mean CST decrease was −5.16 ±48.28 µm (p=0.647). A morphological improvement in IRF was observed in four eyes, with a complete resolution in 50% (n=2) and a decrease in 50% (n=2). Regarding SRF (total n=15), resolution was seen in 46.67% (n=7), decrease in 26.67% (n=4) and stabilization in 13.33% (n=2). Increase in SRF was observed in 13.33% (n=2). Of 14 eyes with sub-RPE fluid, 7.14% (n=1) demonstrated a resolution, 42.86% (n=6) a decrease, 50% (n=7) a stabilization and none an increase in fluid. Mean treatment interval was increased by 4.08 ±1.40 weeks (p<0.001). Treatment was discontinued in seven eyes (41.18%), including four cases due to IOI. In all four cases, inflammation was mild and resolved under corticosteroid treatment. No cases of vasculitis were observed. Conclusion This study provides additional data suggesting that brolucizumab is a beneficial alternative for patients refractory to other anti-VEGF therapies. It can provide a morphological reduction in fluid and prolong the treatment interval, while maintaining a stable BCVA and CST. However, as a higher occurrence of IOI is probable, patients should be informed, selected and monitored carefully. Signs of inflammation should be detected early and treated promptly.

Aim: To evaluate the MONA.health artificial intelligence screening software for detecting referable diabetic retinopathy (DR) and diabetic macular edema (DME), including subgroup analysis. Methods: The algorithm’s threshold value was fixed at the 90% sensitivity operating point on the receiver operating curve to perform the disease classification. Diagnostic performance was appraised on a private test set and publicly available datasets. Stratification analysis was executed on the private test set considering age, ethnicity, sex, insulin dependency, year of examination, camera type, image quality, and dilatation status. Results: The software displayed an area under the curve (AUC) of 97.28% for DR and 98.08% for DME on the private test set. The specificity and sensitivity for combined DR and DME predictions were 94.24 and 90.91%, respectively. The AUC ranged from 96.91 to 97.99% on the publicly available datasets for DR. AUC values were above 95% in all subgroups, with lower predictive values found for individuals above the age of 65 (82.51% sensitivity) and Caucasians (84.03% sensitivity). Conclusion: We report good overall performance of the MONA.health screening software for DR and DME. The software performance remains stable with no significant deterioration of the deep learning models in any studied strata.