Purpose: To assess whether and to what extent macro- and/or microstructural modifications are present in the brain of patients with selective central visual loss due to a juvenile macular degeneration, Stargardt's disease (STGD), taking advantage of the complementary information provided by voxel-based morphometry (VBM) and diffusion tensor imaging (DTI). Methods: Eighteen patients with clinical and molecular diagnosis of STGD related to ABCA4 mutations and 23 normally sighted volunteers of comparable age and sex were enrolled. Structural T1-weighted (T1w) volumes, for brain tissue volume assessment by segmentation, and DTI, for the investigation of diffusivity parameters via a tract-based spatial statistics (TBSS) procedure, were acquired at 3 Tesla in all subjects. All patients underwent a complete ophthalmologic examination, including best-corrected visual acuity (BCVA), biomicroscopy, ophthalmoscopy, electroretinography (ERG), microperimetry, and optical coherence tomography (OCT). Correlations between imaging data and clinical measures were tested. Results: Stargardt's disease patients showed a significant gray matter (GM) loss bilaterally in the occipital cortices, extending into the right precuneus, and in the fronto-orbital cortices. At TBSS, significant reductions in fractional anisotropy were detected throughout large regions in the supratentorial white matter (WM), more pronounced in the posterior areas. Gray matter volume correlated directly with mean visual sensitivity in the right middle frontal and left calcarine gyri, and inversely with retinal thickness in the left supramarginal gyrus. Conclusions: In STGD, widespread microstructural WM alterations are present, suggestive of minor fiber loss coupled with GM loss, also in cortical regions not traditionally linked to visual pathways, at least partly related to the retinal damage. Gray and white matter integrity in Stargardt's disease was assessed by postprocessing of magnetic resonance imaging data. Widespread microstructural WM alterations were detected, only partly related to the retinal damage, coupled with cortical atrophy in regions not directly linked to visual pathways.

Purpose: to evaluate the effect of biofeedback (BF) rehabilitation on the visual function and on the activity of primary visual cortex (PVC) in patients with Stargardt's disease owing to mutations in the ABCA4 gene (STGD1). Methods: This was a single-center, controlled, randomized study. Twenty-four patients with STGD1 were randomized into two groups: a treatment group (TG) undergoing BF rehabilitation and a control group (CG). Treatment with BF consisted of a 10-minute session per eye performed weekly for 12 weeks. The subjects underwent a baseline and 3-month follow-up visits, including best-corrected visual acuity (BCVA), reading test, microperimetry, and functional magnetic resonance imaging (fMRI). The fMRI studies were acquired sequentially using a passive viewing condition and an active reading task. The primary outcomes were the change in the fMRI activation of primary visual cortex and the change in reading ability. Results: After treatment, the patients in the TG were able to read smaller characters (P = 0.002) with a greater reading speed (P = 0.014) compared with patients in the CG. The fMRI studies showed a significant effect (P < 0.001) of BF on primary visual cortex activation in the TG compared with the CG. Finally, we observed significant (P < 0.05) improvements of best-corrected visual acuity, macular sensitivity, and fixation stability parameters in the TG compared with the CG. Conclusions: Our study showed that visual rehabilitation using BF improved the usage of residual visual function in patients with STGD1. Translational Relevance: Our findings show that the BF treatment compared with no treatment at all resulted in benefits. The specificity of the treatment could be examined to determine whether BF can be included in clinical practice.

Background: Corneal collagen cross-linking (CXL) is considered an effective procedure for slowing down or eliminating the progression of keratoconus. New techniques, in combination with CXL, have been proposed to stop the evolution of keratoconus and improve the visual function. Objective: To evaluate the effectiveness of combined photorefractive keratectomy (PRK) with mitomycin-C (MMC) application and CXL in the management of grade 1–2 keratoconus over a 2-year follow-up. Methods: Fifteen eyes underwent topography-guided PRK with 0.02% MMC application immediately followed by standard CXL. Results: Best corrected visual acuity improved from 0.15 ± 0.11 logMAR to 0.08 ± 0.09 logMAR at 24 months ( p < 0.0001) in treated eyes. Mean steepest meridian keratometry reduced from 48.79 ± 3.22 D at baseline to 46.16 ± 3.11 D at 24 months ( p < 0.0001). Mean flattest meridian keratometry reduced from 45.18 ± 2.17 D preoperatively to 44.35 ± 2.19 D at 24 months ( p < 0.0001). Conclusion: Simultaneous topography-guided PRK with MMC 0.02% application and standard CXL is a safe, promising and effective procedure in the treatment of mild and moderate keratoconus.

Purpose: retinal vein occlusion (RVO) is classified as either branch (BRVO) or central (CRVO) RVO. The gold standard treatment for macular oedema (MO) secondary to RVO is intravitreal anti-vascular endothelial growth factor (anti-VEGF) injections. Our study aimed to compare ranibizumab and aflibercept outcomes over a maximum follow-up of five years. Methods: this retrospective study assessed treatment-naïve RVO patients. Active disease was treated with three loading injections followed by a pro-re-nata (PRN) regimen of an anti-VEGF agent. Visual outcomes and injection frequency were analyzed, with patients stratified according to RVO subtype, anti-VEGF agent used, baseline vision, and age. Results: 316 CRVO-affected eyes and 467 BRVO-affected eyes were analysed. Visual benefits between different treatments did not significantly differ, except in year 1 in ranibizumab-treated BRVO eyes. However, aflibercept-treated CRVO and BRVO eyes required significantly fewer injections during the follow up period. Furthermore, our results confirm that younger patients achieve better visual outcomes with fewer intravitreal injections. Overall, half of our patients did not require further injections after 1 year from diagnosis. Conclusion: the results demonstrate that anti-VEGF treatment of RVO benefits vision for up to 5 years. Our findings are the first to suggest that compared to ranibizumab, fewer aflibercept injections may be required over five years follow up. Prospective randomised trials are needed to confirm this, alongside further attention to OCT scan features and the effect of patient demographics on treatment outcomes.