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AIM: To assess the efficacy and safety of a heavy silicone oil (Densiron 68) in the management of inferior retinal detachment recurrence. • METHODS: A retrospective non-comparative consecutive case series study. Forty-nine cases of complex inferior retinal detachment were treated using Densiron 68 heavy silicone oil (HSO) as the endotamponade. Our main purpose was anatomic reattachment following Densiron 68 removal. Functional outcomes, rate of recurrences, the presence of inflammatory complications and intraocular pressure alterations were evaluated. • RESULTS: Forty-nine patients affected by complex retinal re-detachment were recruited. The mean follow-up was 7.6 (±1.5) mo. The mean best corrected visual acuity after Densiron 68 removal was 0.95 logMAR, standard error (SE: 0.068). Retinal reattachment was 61.2% after first surgery and 81.6% after second surgery. Nineteen cases (38.8%) had recurrences when intraocular heavy silicon oil was in situ, 26.3% (5 cases) of which involved the inferior retina. • CONCLUSION: Densiron 68 efficiently fills the inferior retinal periphery and might lower the risk of inferior proliferative vitreoretinopathy development, in particular after a standard silicon oil tamponade that reduces the proliferative process in the upper quadrants of the retina.

Purpose: To investigate the prevalence and features of cystoid spaces (CS) in patients with confirmed genetic diagnosis of choroideremia (CHM) using swept source optical coherence tomography (OCT). Methods: We retrospectively reviewed CHM patients examined at the Regional Reference Center for Hereditary Retinal Degenerations at the Eye Clinic in Florence. We took into consideration genetically confirmed CHM patients with ophthalmological and swept source optical coherence tomography (OCT) examinations. The presence/absence and location of cystoid spaces in the retina of each eye were reported. Results: A total of 42 eyes of 21 CHM patients were included in our series. The average age of the patients was 36.5 ± 20.1 (range, 13–73 years). The average best-corrected visual acuity (BCVA) for all patients was 0.63 ± 1.00 logMar (range, 0–2,80). CS were present in 15 eyes of eight patients (8/21, 38%). In all cases, CS were located in inner nuclear layer (INL); in five eyes of three patients, CS were detected also in ganglion cell layer (GCL). CS appeared as microcistoyd abnormalities and were detected in retinal areas characterized by retinal pigment epithelium (RPE) and outer retinal layers atrophy at the transition zone. Conclusions: Cystoid spaces in choroideremia showed peculiar features; they are clusters of small-size extrafoveal degenerative cysts mainly located in inner nuclear layer at the transition zone where outer retinal layers and RPE are severely damaged.

Fabry Disease (FD) is a rare X-linked lysosomal storage disorder characterized by systemic and ocular involvement. It has been described an increasing in retinal and conjunctival vessel tortuosity and this feature represents an important marker for the disease. Currently, there is not an objective method to measure and quantify this parameter. We tested a new semi-automatic software measuring retinal and conjunctival vessel tortuosity from eye fundus and conjunctival digital images in a group of FD patients. We performed an observational case-control study evaluating three mathematical parameters describing tortuosity (sum of angle metric [SOAM], product of angle distance [PAD], triangular index [I2e]) obtained from fundus and conjunctival pictures of 11 FD patients and 11 age and sex-matched controls. Both eyes were considered. Mann-Whitney test was used to compare the FD group versus the control group and, within the FD group, male versus female patients. Linear regression analysis was performed to evaluate the possible association of retinal and conjunctival vessels tortuosity parameters with age and with specific markers of systemic disease's progression. The tortuosity parameters (SOAM, PAD and I2e) were significantly higher in retinal vessels and in conjunctival nasal vessels in FD patients in comparison with the controls (p=0.003, p=0.002, p=0.001 respectively for retina) (p=0.023, p=0.014, p=0.001 respectively for nasal conjunctiva). No significant association was found between retinal and conjunctival tortuosity parameters and increasing age or systemic involvement markers. Vessel tortuosity represents an important clinical manifestation in FD. A computer-assisted analysis of retinal and conjunctival vasculature demonstrated an increased vessels tortuosity in patients affected by Fabry disease. This non-invasive technique might be useful to help the diagnosis in early stages, to establish disease severity and monitor its progression.

BACKGROUND: Age-related macular degeneration (AMD) shares many similarities with cardiovascular disease (CVD) pathophysiology. We sought to determine the relationship of AMD to the progression of coronary artery calcium (CAC) using data from the Multi-Ethnic Study of Atherosclerosis (MESA). METHODS: Our cohort consisted of 5803 adults aged 45 to 84 years free of known cardiovascular disease (CVD). Retinal photographs were taken during visit 2 (Aug 2002-Jan 2004). CAC was measured with computed tomography at visit 1 (July 2000-Aug 2002) and visit 5 (April 2010-Dec 2011) and changes between visits were determined. RESULTS: Participants were categorized as with (n = 244) and without AMD (n = 5559) at visit 2. At visit 5, 92 participants with and 2684 without AMD had CAC scores. Among those with detectable CAC at baseline (>0 at visit 1), CAC progression was greater in persons with compared to those without AMD after multivariable adjustment (530 ± 537 vs. 339 ± 426 Agatston units, P<0.01). CONCLUSIONS: The presence of AMD in a diverse population without known clinical CVD independently predicted higher 10-year CAC progression in participants with baseline CAC >0. The retinal exam might be a useful tool for pre-clinical assessment and prevention of CVD events.

Purpose: To study peripapillary comet lesions (PCL) in Italian patients affected with pseudoxanthoma elasticum (PXE). Methods: Retrospective review of fundoscopic and swept-source (SS) optical coherence tomography (OCT) images of patients with PXE examined at the Regional Reference Center for Hereditary Retinal Degenerations at the Careggi Teaching Hospital of Florence from 2012 to 2017. Results: From 148 eyes of 74 patients affected with PXE, we identified 24 eyes of 14 patients (11 were female) with a mean age of 39 years (range, 20–58 years) characterized by peripapillary comet lesions. Of these 24 eyes, 15 eyes (of 10 patients) were characterized by comet rain. The smallest comet lesion at the OCT examination appeared as a focal roundish hyper-reflective alteration at the level of the outer retinal segments and RPE-Bruch’s membrane complex; the larger lesions appeared as circular and ovoid structures with hyper-reflective borders in the outer nuclear layer. Conclusion: The comet lesion formation process involves the outer layers of the retina and RPE/Bruch’s membrane complex. It consists of a degenerative/rearrangement process of the photoreceptors which occurs in an area of focal altered RPE/Bruch’s membrane resembling the outer retinal tubulation.

Purpose: To evaluate choroidal thickness (CT) in retinitis pigmentosa (RP) using enhanced depth imaging (EDI) optical coherence tomography (OCT). Methods: A retrospective analysis of a group of patients with RP who underwent EDI-OCT was performed. Choroidal thickness measurements were compared with those of age- and sex-matched healthy subjects. In the RP group, the possible association between subfoveal CT and some clinical parameters (visual acuity, age, age at disease onset, duration of the disease, macular thickness, visual field loss, electroretinography [ERG]) was evaluated. Results: The study recruited 39 patients with RP with an average age of 43.3 ± 11.3 years while the control group consisted of 73 healthy subjects with an average age of 42.9 ± 12.10 years. On average, CT was significantly thinner in the RP group compared to the controls (p<0.0001). In the RP group, we could not find any significant association between CT and the considered clinical parameters even if there was a trend for decreasing CT with increasing age (r = −0.23, p = 0.096). In the control group, subfoveal CT showed a slightly significant correlation with age (r = −0.21, p = 0.04) but not with macular thickness and visual acuity. Conclusions: In our series, CT was significantly lower in the RP group in comparison with the controls, as measured by EDI-OCT, but did not correlate with age, age at onset, duration of the disease, macular thickness, visual acuity, visual field loss, or ERG responses. Although the clinical implications of choroidal changes in RP have not yet been clearly determined, the evaluation of choroidal features may provide information that could be useful to clarify the pathophysiology of the disease.

Purpose: Pars plana vitrectomy has been reported to increase the risk of ocular hypertension and open-angle glaucoma. The authors conducted a systematic review of randomized and nonrandomized studies to compare the incidence of open-angle glaucoma and ocular hypertension in vitrectomized versus nonvitrectomized eyes. Methods: A literature search was performed using MEDLINE and EMBASE until August 2016. Data on ocular hypertension and open-angle glaucoma incidence and mean intraocular pressure after at least 1 year were pooled using random-effects metaanalysis models. Because only nonrandomized studies were retrieved, ROBINS-I tool was used to assess risk of bias in the review. Results: Seven included studies had a paired design to compare the outcomes of vitrectomized versus fellow eyes, with mean follow-up of least 12 months. Four studies (851 patients) provided data on open-angle glaucoma: incidence in vitrectomized versus nonvitrectomized eyes was 7.8% and 4.8%, respectively, yielding a metaanalytic odds ratio of 1.67 (95% CI: 1.08-2.57). Six studies (1,060 patients) reported on the occurrence of ocular hypertension, which was 5.8% in vitrectomized eyes versus 3.1% in fellow eyes (odds ratio: 2.03, 95% CI: 0.97-4.22), without significant differences in the mean postoperative intraocular pressure (mean difference 0.31 mmHg, 95% CI: 20.26 to 0.89). Conclusion: Although the review found increased risk of open-angle glaucoma with pars plana vitrectomy, the studies were heterogenous or inconsistent regarding ocular hypertension and intraocular pressure increase. Larger studies should be conducted in homogenous cohorts of patients undergoing macular surgery, excluding complex conditions such as retinal detachment or diabetic retinopathy.

Objective To evaluate the success of an intravitreal injection of ocriplasmin to release symptomatic vitreomacular traction (VMT) and close a full-thickness macular hole. Methods and analysis An observational retrospective multicentre study conducted in Italy. Patients with symptomatic distortion and loss of vision secondary to VMT were included in the study. The patients received a single injection of ocriplasmin and were followed up for 1, 3 and 6 months. Best-corrected visual acuity (BCVA) and spectral domani OCT (SD-OCT) were performed for patient assessment, and adverse events were recorded and analysed. Results 74 patients (74 eyes) were included in the study. 44 of 74 eyes (59.5%) experienced complete release of the VMT. Macular hole closure was obtained in eight eyes (40%). BCVA improved about three lines after 3 months of follow-up in the patients with VMT resolution in comparison with the patients who did not have VMT resolution (p<0.0001). In 55/74 eyes of 55 patients (74.3%), no adverse events were reported, and most of them were transitory (17/19; 89.5%). The mean time to resolve VMT was 27.4±21.9 days. No cases of retinal tear, retinal detachment or lens destabilisation were observed. Conclusion Ocriplasmin is a potential alternative treatment for patients with symptomatic VMT and has a good safety profile. A more careful selection of patients, in clinical practice, may increase the success rate.

Purpose: To compare the prevailing patterns of Haller vessel arrangements at the posterior pole between healthy eyes and those with central serous chorioretinopathy (CSC) using en face optical coherence tomography. Methods: Eyes of normal subjects and patients with acute or chronic CSC underwent optical coherence tomography imaging (RTVue 100; Optovue Inc, Fremont, CA). En face sections at the level of the Haller layer were classified by two masked graders into five mutually exclusive morphologic categories (temporal herringbone, branched from below, laterally diagonal, double arcuate, and reticular). The relative prevalence of each Haller vessel arrangement pattern was determined for each phenotype. Results: Numbers of eyes examined were as follows: 154 eyes of 77 normal subjects; 41 eyes of 31 patients with acute CSC; and 39 eyes of 33 patients with chronic CSC. The mean age of participants was 44.4 ± 14.6 years for healthy subjects (M:F = 37:40), 48.5 ± 8.2 years (M:F = 24:7) for acute CSC, and 65.3 ± 13.1 years (M:F = 28:5) for chronic CSC. The relative prevalence of each Haller vessel arrangement pattern differed by phenotype. The temporal herringbone pattern was most prevalent in healthy eyes (49.2%), whereas a reticular pattern was most prevalent in eyes with acute and/or chronic CSC (combined, 48.8%). Conclusion: A significant difference was observed in the prevalence of respective Haller vessel arrangement patterns between eyes of normal subjects and those of patients with either acute or chronic CSC. Although further study is needed to determine the mechanistic factors underlying these differences, and the hemodynamic implications, our data suggest that en face optical coherence tomography may find a formal role in choroidal disease classification.

Purpose: The purpose of our study was to investigate morpho-functional features of the preferred retinal location (PRL) and the transition zone (TZ) in a series of patients with recessive Stargardt disease (STGD1). Methods: Fifty-two STGD1 patients with at least one ABCA4 mutation, atrophy of the central macula (MA) and an eccentric PRL were recruited for the study. Microperimetry, fundus autofluorescence (FAF), spectral-domain optical coherence tomography (SD-OCT) were performed. The location and stability of the PRL along with the associated FAF pattern and visual sensitivities were determined and compared to the underlying retinal structure. Results: The mean visual sensitivity of the PRLs for the 52 eyes was 10.76 +/- 3.70 dB. For the majority of eyes, PRLs were associated with intact ellipsoid zone (EZ) bands and qualitatively normal FAF patterns. In 17 eyes (32.7%) the eccentric PRL was located at the edge of the MA. In 35 eyes (67.3%) it was located at varying distances from the border of the MA with a TZ between the PRL and the MA. The TZ was associated with decreased sensitivity values (5.92 +/- 4.69 dB) compared to PRLs (p<0.05), with absence/disruption of the EZ band and abnormal FAF patterns (hyper or hypo-autofluorescence). Conclusions: In STGD1 eccentric PRLs are located away from the border of MA and associated with intact EZ bands and normal FAF. The TZ is characterized by structural and functional abnormalities. The results of multimodal imaging of the PRL and TZ suggest a possible sequence of retinal and functional changes with disease progression that may help in the planning of future therapies; RPE dysfunction appears to be the primary event leading to photoreceptor degeneration and then to RPE loss.

Background: "Dragged- fovea diplopia syndrome" is a type of central binocular diplopia that is secondary to a foveal displacement, caused by epiretinal membranes (ERMs) or other macular diseases. Its management is difficult, because prisms are not effective. Case Reports: Two cases of dragged-fovea diplopia syndrome were presented. Both patients were affected with a unilateral epiretinal membrane. Therefore, the pathophysiology underlying their diplopia was the conflict between central and peripheral fusion mechanisms. Conclusions: Diplopia caused by ERM "shift" deserves a complex management. We suggest to be careful about subjective symptoms and to optimize the residual visual function to customize the orthoptic management. A strict cooperation between ophthalmologists and orthoptists could lead to a successful outcome.

Purpose. To compare the vitrectomy time, clinical outcomes, and complications between 27-gauge (27-G) and 25-gauge (25-G) vitrectomy in patients with primary rhegmatogenous retinal detachment (PRRD). Methods. Prospective, nonrandomized, comparative, interventional study. Forty consecutive patients with PRRD were recruited. Twenty patients underwent the 27-gauge procedure and twenty patients had the 25-gauge procedure. The main outcome measure of the study was the actual vitrectomy time. Results. The mean duration of vitreous removal was 23.2 min (SD 6.5) with 27-G vitrectomy and 19.6 min (SD 7.3) with 25-G vitrectomy, resulting in a difference of 3.6 min (95% confidence interval (95%CI): -8.0 to 0.8 mins, p=0.11). Mean logMAR visual acuity improved from 1.70 ± 1.18 preoperatively to 0.12 ± 0.14 at final postoperative visit (p<0.001) in the 27-G group and from 1.52 ± 1.15 preoperatively to 0.22 ± 0.30 at final postoperative visit (p<0.001) in the 25-G group. The anatomical success rate after a single operation was 90.0% and 85.0% in the 27-G and in the 25-G groups (p=0.63), respectively. Intraoperative iatrogenic retinal breaks (IRBs) occurred in 2 eyes in the 27-G group and 1 eye in the 25-G group. Conclusions. Twenty-seven-gauge vitrectomy may be a safe and effective surgery for the treatment of PRRD.

Purpose: To describe clinical and molecular characteristics in a group of Italian female choroideremia (CHM) carriers and report fundus patterns. Methods: We retrospectively studied 11 female carriers belonging to six CHM families examined at the Regional Reference Center for Hereditary Retinal Degenerations at the Eye Clinic in Florence. We took into consideration patients with a comprehensive ophthalmological examination, fundus photography, optical coherence tomography (OCT), full field electro-retinography (ERG), and visual field (VF). All patients were screened for mutations of the CHM gene. Results: Fundus examination revealed retinal abnormalities in all female carriers (11/11) in the study; in particular four fundus patterns were identified: pattern A (RPE dystrophy involving only the peripheral retina), pattern B (RPE dystrophy involving the peripheral retina and the posterior pole with small hypo-pigmented RPE areas), pattern C (RPE dystrophy involving the peripheral retina and the posterior pole with small yellowish well-defined dots), and pattern D (RPE dystrophy involving the peripheral retina and the posterior pole with large hypo-pigmented RPE areas and well-defined yellowish dots). Pattern D was characterized by widespread macular subretinal drusenoid deposits (SDD). Half of the observed mutations were novel mutations. A genotype-phenotype correlation was not identified. Conclusions: Retinal dystrophy and SDD were detected in our female CHM carriers, and fundus patterns have been described in this study. The recognition of specific fundoscopic patterns may permit a correct diagnosis, an appropriate molecular investigation and genetic counseling.

Purpose: Retinal detachment is a frequent complication after removal of silicone oil (ROSO). A retrospective study was conducted to determine whether staining-assisted removal of silicone oil (st-ROSO) allowed better identification and removal of proliferative vitreoretinopathy (PVR) processes compared with a conventional removal of silicone oil technique. Methods: All individuals underwent pars plana vitrectomy (PPV) and silicone oil fill-in for complicated retinal detachments. In conventional removal of silicone oil (Group 1), no staining was used. In staining-assisted removal of silicone oil (Group 2), a mixture of trypan blue and brilliant blue G dyes was used to identify proliferative vitreoretinopathy and subclinical epiretinal membrane. Results: After the first 3-month follow-up, 15.9% of patients (N = 608) developed a retinal detachment. Retinal detachment occurred in 22.8% of patients in Group 1 (n = 284) and 9.8% of patients in Group 2 (n = 324; P < 0.001). In Group 2, proliferative vitreoretinopathy removal was performed in 153 eyes (47.2%). Conclusion: The incidence of retinal redetachment was significantly lower after staining-assisted removal of silicone oil compared with a conventional technique. Staining-assisted removal of silicone oil allowed better identification and removal of proliferative vitreoretinopathy processes.

Purpose: To study the reading performance of patients with Stargardt disease (STGD) and the relationship between clinical vision measurements and vision-related quality of life (VRQOL). Methods: We studied both eyes of 16 patients with STGD. Each patient was examined for best-corrected visual acuity (Early Treatment Diabetic Retinopathy Study [ETDRS]), reading ability (MNREAD and REX charts), contrast sensitivity (Pelli-Robson charts), fixation study (MP1 microperimeter), and VRQOL (25-item National Eye Institute Visual Function Questionnaire [NEI VFQ-25]). The correlation pattern among these variables was examined and an exploratory factor analysis was used to investigate dimensionality of both visual function and VRQOL. Results: Mean ETDRS visual acuity was about 20/160 (0.9 logMAR). All studied psychophysical measures were highly or moderately correlated with MNREAD reading speed (p<0.05 level). A similar correlation was found between psychophysical measures and VRQOL, which was higher for MNREAD measures of acuity (r = -0.75) and speed (r = 0.74). Accordingly, exploratory factor analysis suggested that a single latent dimension explained most of the variance of vision psychophysical measures as well as of VRQOL. Conclusions: We propose that reading ability should be assessed in patients with STGD, since we found that both MNREAD reading speed and visual acuity are strong determinants of quality of life. The observed relation between reading ability and VRQOL in STGD suggests that in these patients appropriate low vision rehabilitation can improve both reading performance and consequently VRQOL. Finally, our data support the use of reading speed and visual acuity as important outcome measures for monitoring STGD progression.

Purpose: To evaluate the efficacy of an intravitreal ocriplasmin injection using anterior chamber paracentesis to release symptomatic vitreomacular traction (VMT).Methods: Five patients with symptomatic VMT were enrolled.All patients underwent a complete ophthalmologic examination including acquisition of spectral-domain optical coherence tomography.Before intravitreal injection of ocriplasmin, anterior chamber paracentesis was performed in the study eye to induce mild ocular hypotonia.Control visits were performed the day after the injection, at 1 week, and after 1, 2, and 3 months.Results: In 4 patients, we had complete release of VMT and visual improvement after the intravitreal ocriplasmin injection preceded by anterior chamber paracentesis.No adverse events were observed.Conclusions: In our small case series, anterior chamber paracentesis performed before intravitreal ocriplasmin seemed to increase the efficacy of the drug in the resolution of symptomatic VMT.Our success estimate is imprecise due to small sample size (95% confidence interval 0.28 to 0.99) and no definitive conclusion can be reached.Further research is worth being conducted to assess the potential usefulness of paracentesis before ocriplasmin injection to increase vitreoretinal traction release rate.