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Purpose: The vitelliform stage is the typical phenotypic manifestation of Best vitelliform macular dystrophy (BVMD). As yet, no study has focused specifically on the clinical changes occurring in the vitelliform stage over the follow-up., Methods: The survey takes the form of a prospective observational study with a 5-year follow-up. Twenty-one eyes of 11 patients in the vitelliform stage were examined annually. The primary outcome was the identification of the changes in the vitelliform lesion over a 5-year follow-up. Secondary outcomes included changes in structural optical coherence tomography (OCT) parameters and the correlation with the BCVA variation over the follow-up., Results: Spectral domain OCT at baseline showed one subform characterized by solid vitelliform deposition, in 81% of eyes, and another subform characterized by a combination of solid deposition and subretinal fluid, in 19% of eyes. Overall, 62% of eyes showed an increase in the area of vitelliform deposition. Once the maximal area was reached, a progressive flattening of the vitelliform deposition took place, with subsequent flattening of the vitelliform lesion and formation of subretinal fluid. Hyperreflective foci (HF) increased in number as long as the vitelliform area continued to expand, with no variation in HF when the vitelliform lesion flattened or the subretinal fluid formed., Conclusions: The vitelliform stage reveals more subforms with clinical variations over the follow-up. Our data suggest that the substage before the flattening of the lesion, thus before the so-called subretinal fluid accumulates and when the visual acuity is still high, might offer the best opportunity for an optimal therapeutic approach.

Purpose: To evaluate the efficacy of intravitreal anti-VEGF injections in choroidal neovascularization (CNV) related to pattern dystrophy-like deposit in pseudoxanthoma elasticum (PXE)., Methods: One-year prospective, interventional study. Nine eyes were recruited in the ophthalmology departments of San Raffaele University and University of Barcelona. Each patient underwent best corrected visual acuity (BCVA) measurement on ETDRS chart, slit-lamp biomicroscopy, fluorescein angiography (FA), indocyanine green angiography (ICGA), and optical coherence tomography (OCT). The protocol included a first anti-VEGF injection, followed by monthly evaluations with re-treatments based on new funduscopic hemorrhages, fluid on OCT or leakage on FA and/or ICGA. Primary outcome measures were the mean BCVA changes. Secondary outcomes included central macular thickness (CMT) variations and the number of injections needed., Results: At month 12, mean BCVA significantly improved from 20/45 to 20/35 Snellen equivalent, with 3 eyes gaining at least 3 ETDRS lines. Mean CMT decreased from 297 ± 22 to 262 ± 13 μm, after 5.5 ± 4.0 injections. No leakage was observed at the end of follow-up., Conclusions: Intravitreal anti-VEGF injections represent an effective treatment for CNV related to pattern dystrophy-like deposit in PXE, with an improvement of BCVA and CMT. Mean injection number is in line with other studies performed in CNV secondary to angioid streaks.

Purpose: To describe the hyperreflective foci (HF) characteristics in eyes affected by Stargardt disease (STGD), correlating HF with the atrophy progression at 1-year follow-up., Methods: Prospective observational case series with 1-year follow-up. Twenty-eight eyes (14 patients) affected by STGD and 28 eyes (14 age- and sex-matched healthy patients) used as control group were recruited. All patients underwent a complete ophthalmologic examination including fundus autofluorescence and spectral-domain optical coherence tomography. The primary outcome was the identification of HF specific location in STGD and their modification over a 1-year follow-up. Secondary outcome included the correlation between the number and the location of HF and atrophic changes., Results: HF turned out to be more frequent in STGD patients compared with healthy controls (p < 0.001). In particular, mean number of HF in the pathological edge was significantly higher than in the healthy edge of the atrophy (p < 0.001) and in the foveal area (p < 0.001). A negative correlation was found between the total HF number in the pathological edge and the atrophic area at baseline. HF number in the outer retina of the pathological edge significantly decreased between the baseline and the final follow-up examination (p = 0.011). The enlargement of the atrophic area in eyes with more than five outer retinal HF in the pathological edge at baseline was significantly less than that found in the eyes with fewer than five HF (p = 0.010)., Conclusions: HF are most common at the pathological margin of the central atrophy, with outer retina foci being more frequently found in smaller atrophic lesions.

Purpose: To describe the morphological macular changes detected by spectral domain optical coherence tomography (SD-OCT) in eyes with retinitis pigmentosa (RP) and to analyze their correlation with the visual function., Methods: Twenty-two patients (44 eyes) patients affected by RP were recruited. The following structures were evaluated on SD-OCT: outer plexiform layer (OPL), outer nuclear layer (ONL), external limiting membrane (ELM), photoreceptor inner/outer segment (IS/OS) junction, photoreceptor outer segment/retinal pigmented epithelium (OS/RPE) junction, inner limiting membrane thickening (ILMT), ganglion cell complex (GCC), and cystoid macular edema (CME). The relation between each SD-OCT finding and BCVA was evaluated at uni- and multivariate analysis., Results: Mean age and mean best-corrected visual acuity (BCVA) were 51 ± 17.5 years and 0.4 ± 0.5 LogMAR, respectively. Univariate linear regression model revealed a correlation between BCVA and the absence of ELM, IS/OS, ONL, and OS/RPE layers (R (2) values were, respectively, 0.51, 0.57, 0.48, and 0.68, with p values all <0.0001). At multivariate regression analysis, the absence of OS/RPE and ELM layers remained the only variables independently associated with a decrease of BCVA (R (2) = 0.85, t = 3.49, p = 0.0014)., Conclusions: Data show that in patients afflicted with RP, ELM and OS/RPE layers are independently associated with BCVA on multivariate regression analysis. These results highlight the key-role of external retinal layers in determining the visual function impairment attributable to RP.

Background: Vitreoretinal adhesions play a key role in the vector forces exerted on the vitreoretinal interface, leading to tractional retina deformation and macular hole formation. The aim of this study was to identify the presence of vitreopapillary and vitreofoveal adhesions in idiopathic epiretinal membranes (ERMs) with spectral-domain optical coherence tomography (SD-OCT) and to evaluate their influence on the vitreoretinal interface., Methods: Sixty-five eyes (65 patients) with idiopathic ERM and 64 healthy eyes (64 patients) underwent SD-OCT analysis. We studied vitreopapillary and vitreofoveal adhesion prevalence in eyes with idiopathic ERM using different SD-OCT patterns ("adherent" or "tractional"). We analyzed their influence on central foveal thickness (CFT), on retinal nerve fiber layer (RNFL) thickness, and on morphological modifications (foveal depression profile and inner/outer photoreceptor junction)., Results: Vitreopapillary adhesion was present in 51.6 % of normal eyes and in 24.6 % of eyes with idiopathic ERM, while vitreofoveal adhesion was found in 14.1 % of normal eyes and in 15.4 % of eyes with ERM. Vitreopapillary adhesion prevalence was significantly higher in the tractional ERM subgroup (p = 0.01), than in the adherent ERM subgroup. Both adhesions had no influence on CFT, RNFL thickness, or inner segment/outer segment junction status., Conclusions: Our study suggests that vitreoretinal adhesions may influence the pathogenesis and course of idiopathic ERM. The absence of vitreopapillary adhesion in the adherent type, and its presence in the tractional type, seems to play a key role in ERM characterization.

Background: Photodynamic therapy with verteporfin (Visudyne; Novartis, Bülach, Switzerland) has been proposed for the treatment of subfoveal choroidal neovascularization secondary to pathologic myopia. We retrospectively evaluated the effects of verteporfin therapy of subfoveal recurrences of extrafoveal myopic CNV previously treated with thermal laser photocoagulation., Methods: Twelve eyes, previously treated with thermal laser photocoagulation for extrafoveal choroidal neovascularization, received photodynamic therapy with verteporfin for subfoveal recurrence of choroidal new vessels. Thirteen eyes that did not receive photodynamic therapy served as control group. Vision and fluorescein angiography outcomes were analyzed on all study visits (every 3 months) through month 12. Visual acuity was measured in Snellen lines., Results: On average, at the month 12 examination the verteporfin-treated group had gained 2 lines and the untreated group had lost 1 line of vision. Eleven eyes of the verteporfin-treated group compared with nine eyes of the untreated group lost fewer than 3 lines of vision, including four eyes versus none improving at least 1 line of vision., Conclusion: Photodynamic therapy with verteporfin might increase the chance of stabilizing or improving vision in patients with subfoveal recurrences of extrafoveal myopic CNV previously treated with thermal laser photocoagulation. A prospective, randomized study on larger series of patients is mandatory.

Purpose: To describe angiographic features detectable on fluorescein angiography (FA) and indocyanine green angiography (ICGA) early after laser photocoagulation of choroidal neovascularisation (CNV) in age-related macular degeneration (AMD)., Methods: Thirty-five eyes of patients with AMD and juxtafoveal or extrafoveal CNV referred to the angiographic centre of the Eye Clinic of Trieste were considered. Ophthalmological assessment included FA and ICGA performed 2 days before and 30 min after laser treatment, and then 1, 2, 7, 14, 21 and 28 days after photocoagulation. Further clinical angiographic examinations were carried out 2, 3, 4 and 6 months after treatment. Photocoagulation was performed for classic CNV on FA and occult CNV on FA, appearing as well-defined focal spot on ICGA., Results: Our results show that interpretation of early post-treatment angiographic examinations may be awkward because diffuse leakage on FA and hot spots on ICGA are normally detectable soon after laser treatment and thereafter during the first 2 weeks. Later, at the 3-week control, leakage on FA and hot spots on ICGA are visible in 62.8% and in 37% of cases respectively; they disappear completely by the 4-week control., Conclusion: Difficulty in analysing FA and ICGA in the early post-photocoagulation period underlines the importance of the decision regarding when to perform the first reliable post-laser control and how to improve its interpretation. We suggest that the first angiographic control be performed 3 weeks after treatment, strictly monitoring those eyes showing leakage or marginal hot spots over the following weeks. Overlapping the post-laser hypofluorescent area on the pre-laser lesion can ensure the complete coverage of CNV, and analysis of the retinal and choroidal vascular pattern inside and near the photocoagulated area during the different angiographic phases, albeit difficult, is essential for the interpretation of the angiographic lesions.

Background: Macular branch retinal vein occlusion (MBRVO) is a subgroup of branch retinal vein occlusion in which the occlusion is limited to a small venous vessel draining a sector of the macular region. The present study aimed to evaluate prospectively whether grid laser treatment is useful in improving the visual acuity of eyes affected by MBRVO with significant macular edema., Methods: Ninety-nine patients affected by MBRVO of recent onset were prospectively studied during a 24-month period; eyes were randomly assigned to the control group, the early grid laser treatment group or the delayed grid laser treatment group. Clinical and angiographic features were recorded during the whole follow-up; parameters such as visual acuity and macular edema were carefully evaluated, the latter even by means of stereophotography., Results: With respect to the baseline mean visual acuity values, a statistically significant improvement was noted at the 3-month follow-up and at the 1-year follow-up. No additional improvement was noted at the 2-year follow-up. No statistically significant difference was found between the treated groups and the control group., Conclusions: We hypothesize that the abrupt ischemic damage subsequent to MBRVO is the main factor causing the central function impairment. Grid laser treatment is not able to reduce the macular edema more than the natural evolution, and does not improve visual acuity.