An incipient late-onset retinal degeneration with a C1QTNF5 mutation: a case report with an 11-year follow-up.

PURPOSE: The purpose of this study was to describe and diagnose the difficulty in a long-term follow-up (eleven years) patient with a very early presentation of late-onset retinal degeneration (L-ORD) and the significance of electrophysiological examinations and follow-up in assessing undiagnosed inherited retinal diseases. METHODS: This is an observational case report of a 56-year-old woman, with scattered multiple yellow-white retinal dots firstly diagnosed as fundus albipunctatus. Ten years after presentation, a deterioration in rod and cone responses in ff-ERG was detected, which allowed us to discard the first diagnostic hypothesis and proceed with a genetic testing. RESULTS: Ten years after presentation, she presented a clear progression of the abnormal photoreceptor response with a cone and rod involvement in ff-ERG, which was not compatible with the previous suspicion of fundus albipunctatus. Six months later, genetic testing results together with the typical progression of atrophic patchy lesions in multimodal imaging allowed a certain diagnosis of L-ORD, caused by an already reported pathogenic variant in the C1QTNF5 gene (c.563C > T; p. Pro188 Leu). CONCLUSIONS: We demonstrate the importance of the ff-ERG examination and the follow-up (or ERG and imaging repetition) in the differential diagnosis of an incipient L-ORD, which can be easily misdiagnosed in the early stages, before the appearance of the characteristic chorioretinal atrophy seen with the progression of this rare disease.

NONEXUDATIVE INTRARETINAL FLUID IN INTERMEDIATE AGE-RELATED MACULAR DEGENERATION.

BACKGROUND: To describe the occurrence of nonexudative intraretinal fluid (IRF) in intermediate age-related macular degeneration. METHODS: A retrospective study was designed to include consecutive cases with intermediate age-related macular degeneration associated with IRF. A multimodal imaging approach was used to confirm diagnosis of IRF in intermediate age-related macular degeneration. Multimodal imaging included color fundus photograph, fundus autofluorescence, fluorescein angiography, indocyanine green angiography, optical coherence tomography, and optical coherence tomography angiography. RESULTS: Ten eyes of 10 patients (2 male and 8 female patients, ages 68-80 years) showing IRF in intermediate age-related macular degeneration were included in the study. The mean best-corrected visual acuity was 20/40 Snellen equivalent. Multimodal imaging including fluorescein angiography/indocyanine green angiography and optical coherence tomography demonstrated the absence of macular neovascularization in all cases; optical coherence tomography-angiography did not detect any abnormal flow signal associated with IRF. Seven of 10 patients developed IRF in correspondence of pigment epithelium detachment. Three of 10 patients presented IRF in correspondence of an area of nascent geographic atrophy. CONCLUSION: Nonexudative intraretinal fluid in intermediate age-related macular degeneration is a novel, distinctive feature that is characterized by the presence of IRF with no evidence of macular neovascular lesions. The authors described different phenotypes of IRF in intermediate age-related macular degeneration. The definite diagnosis of this condition requires further studies with thorough application of multimodal imaging.

In vivo cone-photoreceptor density comparison between eyes with subretinal drusenoid deposits and healthy eyes using high magnification imaging.

PURPOSE: To compare photoreceptor density automated quantification in eyes with subretinal drusenoid deposits (SDD) and healthy controls using Heidelberg Spectralis High Magnification Module (HMM) imaging. METHODS: Twelve eyes of 6 patients with intermediate AMD, presenting with SDD were included, as well as twelve eyes of healthy controls. Individual dot SDD within the central 30° retina were examined with infrared confocal laser ophthalmoscopy, HMM, and spectral-domain optical coherence tomography (SD-OCT). Photoreceptor density analysis was performed on the best-quality image using the ImageJ Foci Picker plugin, after the removal of SDD from the HMM image. Correlations were made between the HMM quantified photoreceptor density, SD-OCT characteristics, stage, and number of SDD. RESULTS: Mean age was 75.17 ± 2.51 years in the SDD group (3 males, 3 females) versus 73.17 ± 3.15 years in the healthy control group (p = 0.2). Defects in the overlying ellipsoid zone were present on SD-OCT in 8/12 (66.66%) eyes. The mean ± standard deviation foci detected (i.e., cone photoreceptors) was 7123.75 ± 3683.32 foci/mm2 in the SDD group versus 13,253 ± 3331.00 foci/mm2 in the healthy control group (p = 0.0003). The number of SDD was associated with a reduction in foci density, p = 0.0055, r = - 0.7622. CONCLUSION: The decreased cone density in eyes with SDD may correlate with a decrease in retinal function in intermediate AMD eyes independent of neovascular complications or outer retinal pigment epithelial atrophy.

Widefield oct-angiography-based classification of sickle cell retinopathy.

PURPOSE: To assess the capillary non-perfusion in different concentric sectors on widefield optical coherence tomography angiography (WF-OCTA) and to correlate the ratio of non-perfusion (RNP) to the severity of sickle cell retinopathy (SCR). METHODS: This retrospective, cross-sectional study included eyes of patients with various sickle cell disease (SCD) genotypes having undergone WF-OCTA and ultra-widefield color fundus photography (UWF-CFP). Eyes were grouped as no SCR, non-proliferative SCR or proliferative SCR. RNP was assessed on WF-OCTA montage in different field-of-view (FOV) sectors centered on the fovea: 0-10-degrees circle excluding the foveal avascular zone, the 10-30-degrees circle excluding the optic nerve, the 30-60-degrees circle, and the full 60-degrees circle. RESULTS: Forty-two eyes of twenty-eight patients were included. Within each SCR group, mean RNP of the FOV 30-60 sector was higher than all other sectors (p < 0.05). Mean RNP of all sectors were significatively different between no SCR group and proliferative SCR group (p < 0.05). To distinguish no SCR versus non-proliferative SCR FOV 30-60 had a good sensitivity and specificity of 41.67% and 93.33%, respectively (cutoff RNP > 22.72%, AUC = 0.75, 95% CI 0.56-0.94, p = 0.028). To differentiate non-proliferative versus proliferative SCR, FOV 0-10 had good sensitivity and specificity of 33.33% and 91.67%, respectively (cutoff RNP > 18.09, AUC = 0.73, 95% CI 0.53 to 0.93, p = 0.041). To discern no SCR versus proliferative SCR, all sectors had optimal sensitivity and specificity (p < 0.05). CONCLUSION: WF OCTA-based RNP provides non-invasive diagnostic information regarding the presence and severity of SCR, and correlates with disease stage in certain FOV sectors.

VITRECTOMY RESULTS FOR RHEGMATOGENOUS RETINAL DETACHMENT WITH CONCOMITANT MACULAR HOLE IN NONMYOPIC PATIENTS.

PURPOSE: To report on results of pars plana vitrectomy with ILM peeling in patients with rhegmatogenous retinal detachment (RRD) and concomitant macular hole (MH) and to assess for preoperative associated conditions related to this type of RRD. METHODS: Patients undergoing surgical repair for RRD between 2014 and 2021 were reviewed, and subjects with concomitant, non-causal, macular hole were identified. We studied post-operative macular status, retinal reattachment rate and visual acuity. RESULTS: Over 532 eyes operated on for RRD, 11 (2.06%) had a concurrent non-causal macular hole. Preoperative PVR B or superior was recorded in 86 eyes (16.6%) of the entire cohort and in 6 eyes (54.54 %) with RRD and concomitant MH (p=0.00001). Severe hypotony with choroidal detachment was present in 15 eyes (2.81%) of the entire cohort and in 3 eyes (27.27%) with RRD and concomitant MH (p=<0.00001). CONCLUSIONS: RRD with concomitant MH is an infrequent association. Retinal reattachment and anatomical hole closure can be achieved in most of cases but despite this fact, functional recovery is usually not good. Preoperative PVR is a more frequent finding in this group of patients, as well as severe hypotony with choroidal detachment.

CHOROIDAL VASCULARITY INDEX IN HYDROXYCHLOROQUINE TOXIC RETINOPATHY: A Quantitative Comparative Analysis Using Enhanced Depth Imaging In Spectral Domain Optical Coherence Tomography.

PURPOSE: To investigate choroidal involvement in eyes of patients treated with hydroxychloroquine (HCQ), by quantifying the choroidal vascularity index (CVI) and other choroidal biomarkers. METHODS: Vertical enhanced depth imaging spectral domain optical coherence tomography (SD-OCT) scans were performed in eyes with either advanced-stage or mild HCQ toxic retinopathy, as well as in healthy age-matched and sex-matched controls. Based on SD-OCT scans, the subfoveal and mean choroidal thickness (ChT) was measured. The CVI, total choroidal area (TCA), luminal choroidal area (LCA), and stromal choroidal area (SCA) were calculated based on a binarization image process. These variables were computed and compared between the three groups (i.e., advanced stage, mild toxicity, and healthy controls). RESULTS: Forty-eight eyes of 47 patients under HCQ (26 eyes presented with advanced stage HCQ toxicity and 22 eyes with mild toxicity) and 34 eyes of 31 healthy controls were included. Both CVI and ChT were significantly different between the three groups ( P < 0.001, P < 0.001). When comparing the advanced stage toxicity group to healthy controls, both the subfoveal and the mean ChT were diminished ( P < 0.001). The CVI, TCA, LCA, and SCA were significantly lower in the advanced stage of toxicity group when compared with controls ( P < 0.001, <0.00001, <0.0001, and P = 0.0094, respectively). CONCLUSION: Our study suggests that eyes with HCQ toxic retinopathy, especially at advanced stages, present with choroidal impairment, giving further pathophysiological insights into the unfolding of this retinal toxicity.

PHOTOBIOMODULATION THERAPY FOR LARGE SOFT DRUSEN AND DRUSENOID PIGMENT EPITHELIAL DETACHMENT IN AGE-RELATED MACULAR DEGENERATION: A Single-Center Prospective Pilot Study.

PURPOSE: To evaluate visual acuity and morphologic changes after photobiomodulation (PBM) for patients affected with large soft drusen and/or drusenoid pigment epithelial detachment associated with dry age-related macular degeneration. METHOD: Twenty eyes with large soft drusen and/or drusenoid pigment epithelial detachment age-related macular degeneration were included and treated using the LumiThera Valeda Light Delivery System. All patients underwent two treatments per week for 5 weeks. Outcome measures included best-corrected visual acuity, microperimetry-scotopic testing, drusen volume, central drusen thickness, and quality of life score at baseline and month 6 (M6) follow-up. Data of best-corrected visual acuity, drusen volume, and central drusen thickness were also recorded at week 5 (W5). RESULTS: Best-corrected visual acuity significantly improved at M6 with a mean score gain of 5.5 letters ( P = 0.007). Retinal sensitivity decreased by 0.1 dB ( P = 0.17). The mean fixation stability increased by 0.45% ( P = 0.72). Drusen volume decreased by 0.11 mm 3 ( P = 0.03). Central drusen thickness was reduced by a mean of 17.05 µ m ( P = 0.01). Geographic atrophy area increased by 0.06 mm 2 ( P = 0.01) over a 6-month follow-up, and quality of life score increased by 3,07 points on average ( P = 0.05). One patient presented a drusenoid pigment epithelial detachment rupture at M6 after PBM treatment. CONCLUSION: The visual and anatomical improvements in our patients support previous reports on PBM. PBM may provide a valid therapeutic option for large soft drusen and drusenoid pigment epithelial detachment age-related macular degeneration and may potentially slow the natural course of the disease.

NEW ARTIFICIAL INTELLIGENCE ANALYSIS FOR PREDICTION OF LONG-TERM VISUAL IMPROVEMENT AFTER EPIRETINAL MEMBRANE SURGERY.

PURPOSE: To predict improvement of best-corrected visual acuity (BCVA) 1 year after pars plana vitrectomy for epiretinal membrane (ERM) using artificial intelligence methods on optical coherence tomography B-scan images. METHODS: Four hundred and eleven (411) patients with Stage II ERM were divided in a group improvement (IM) (≥15 ETDRS letters of VA recovery) and a group no improvement (N-IM) (<15 letters) according to 1-year VA improvement after 25-G pars plana vitrectomy with internal limiting membrane peeling. Primary outcome was the creation of a deep learning classifier (DLC) based on optical coherence tomography B-scan images for prediction. Secondary outcome was assessment of the influence of various clinical and imaging predictors on BCVA improvement. Inception-ResNet-V2 was trained using standard augmentation techniques. Testing was performed on an external data set. For secondary outcome, B-scan acquisitions were analyzed by graders both before and after fibrillary change processing enhancement. RESULTS: The overall performance of the DLC showed a sensitivity of 87.3% and a specificity of 86.2%. Regression analysis showed a difference in preoperative images prevalence of ectopic inner foveal layer, foveal detachment, ellipsoid zone interruption, cotton wool sign, unprocessed fibrillary changes (odds ratio = 2.75 [confidence interval: 2.49-2.96]), and processed fibrillary changes (odds ratio = 5.42 [confidence interval: 4.81-6.08]), whereas preoperative BCVA and central macular thickness did not differ between groups. CONCLUSION: The DLC showed high performances in predicting 1-year visual outcome in ERM surgery patients. Fibrillary changes should also be considered as relevant predictors.

Optical coherence tomography angiography analysis of changes in the retina and the choroid after hemodialysis for end stage kidney disease.

PURPOSE: To study effects of hemodialysis (HD) on retinal and choroidal vasculature in patients with end-stage kidney disease (ESKD) using optical coherence tomography angiography (OCTA). METHODS: In this prospective study, we investigated eyes of patients undergoing hemodialysis for ESKD from Mondor University Hospital. Only one eye/patient was considered. Subfoveal choroidal thickness (SCT) was measured on enhanced-depth imaging optical coherence tomography (EDI-OCT) before and after the hemodialysis session. OCTA was used to extract retinal vascular density (superficial and deep capillary plexus, SCP, DCP) and choriocapillaris non-perfusion. Clinical, demographic and biological parameters (Blood B-Nitric Peptid rate prior to HD session) were reviewed. RESULTS: Twenty patients (mean age 53.2 ± 13.6 years, 10 males and 10 females) were included in this prospective study. SCT significantly decreased after the HD session (234.3 ± 56.14 µm before HD to 211.9 ± 60.79 µm after hemodialysis (Wilcoxon signed-rank test, p = 0.003)). Non-perfusion in the choriocapillaris significantly increased after HD (41.65 ± 3.58 before HD, 42.95 ± 3.19 after HD, p = 0.036) while no significant modification of the vascular density was observed in the retinal vasculature (SCP, DCP) around the macular zone or the optic nerve. An increased plasma B-Nitric Peptide (BNP) level prior to the onset of the HD session was significantly correlated with the decrease of the SCT (r = 0.45, p = 0.043). CONCLUSION: Hemodialysis in patients with ESKD is associated with a significant decrease in SCT and an increase in non-perfusion in the choriocapillaris on OCTA. A High BNP level prior to the onset of the hemodialysis appears to be correlated with the decrease in SCT.

Neovascular age-related macular degeneration: advancement in retinal imaging builds a bridge between histopathology and clinical findings.

PURPOSE: To provide a review of the salient histological and imaging features in neovascular age-related macular degeneration (AMD) that will be integrated in order to have a better comprehension of the pathogenesis and clinical aspects of this disease. METHODS: A literature review of histology and imaging features in neovascular AMD was conducted. RESULTS: Histology has granted a detailed characterization of neovascular AMD ex vivo. In details, histological features in these eyes have offered important insights into the pathogenesis of neovascular AMD. In addition, histology donated a detailed characterization of the different types of macular neovascularization (MNV) that may complicate AMD. The introduction of optical coherence tomography angiography (OCTA) has enormously amplified our knowledge of neovascular AMD through in vivo assessment of the anatomical and pathological characteristics of this disease. New insights elucidating the morphological features of the choriocapillaris confirmed that this vascular structure plays a crucial role in the pathogenesis of neovascular AMD. OCTA also offered a detailed visualization of MNV complicating neovascular AMD. CONCLUSIONS: New imaging technologies offer a remarkable chance to build a bridge between histology and clinical findings in neovascular AMD.

Choroidal vascularity index in eyes with central macular atrophy secondary to age-related macular degeneration and Stargardt disease.

PURPOSE: To compare macular atrophy (MA) secondary to age-related macular degeneration (AMD) and Stargardt disease (STGD) using the choroidal vascularity index (CVI). METHODS: In this multicentric retrospective study, two distinct cohorts were collected: patients with MA secondary to AMD and MA secondary to STGD. All patients were investigated using a multimodal imaging approach, including CVI in the subfoveal 1000 µm area. Of note, the CVI is not influenced by aging, which allows comparisons between different cohorts. RESULTS: Seventy eyes were included: 35 eyes of 35 patients (mean age 78 ± 7 years) in the AMD group and 35 eyes of 35 patients (mean age 41 ± 16 years, p < 0.001) in the STGD group. Choroidal thickness was significantly lower in the AMD group in comparison to the STGD group (151 ± 80 µm vs 353 ± 105 µm, p < 0.001). The total choroidal area (TCA) was significantly greater in the STGD group in comparison to the AMD group (1.734 ± 0.958 mm2 vs 0.538 ± 0.391 mm2, respectively, p < 0.001). Interestingly, the CVI was significantly lower in AMD patients in comparison to STGD patients (27.322 ± 15.320% vs 49.880 ± 7.217%, respectively, p < 0.001), and this difference was confirmed in the subgroup of patients over 50 years old. CONCLUSION: Our results corroborate the hypothesis that large choroidal vessels were impaired to a greater extent in AMD than in STGD. CVI may help in differentiating AMD from STGD in the presence of MA, better understanding of the pathogenesis, and monitoring of therapeutic response.

SUBRETINAL TRANSIENT HYPOREFLECTIVITY IN AGE-RELATED MACULAR DEGENERATION: A Spectral Domain Optical Coherence Tomography Study.

PURPOSE: To describe and assess the prognostic significance of subretinal transient hyporeflectivity (STHR) on a novel spectral domain optical coherence tomography (SD-OCT) in age-related macular degeneration. METHODS: Consecutive patients with age-related macular degeneration (AMD) presenting STHR, defined as a small, well-defined, round subretinal, hyporeflective lesion, on SD-OCT and without exudative signs were included. Clinical examination and SD-OCT (SPECTRALIS, Heidelberg Engineering, Heidelberg, Germany) were analyzed at inclusion, 1 month before inclusion, and until the onset of exudative signs during the 12-month follow-up. RESULTS: Thirty-five STHR in 21 eyes of 20 patients were included. Among the 21 eyes, 2 eyes had early AMD, 1 eye had nonexudative asymptomatic macular neovascularization, and 18 eyes presented late AMD: 17 eyes neovascular AMD and 1 eye geographic atrophy. During the 2-month follow-up, 97.1% (34/35) of STHR disappeared. During the 12-month follow-up, 57.1% of eyes (12/21) developed exudative signs on 1 eye with early AMD and 11 eyes with neovascular AMD. CONCLUSION: Subretinal transient hyporeflectivity is a novel SD-OCT sign in patients with AMD. The eyes with isolated STHR should be closely monitored on a monthly basis to detect further exudation.

A COMMON FINDING IN FOVEAL-SPARING EXTENSIVE MACULAR ATROPHY WITH PSEUDODRUSEN IMPLICATES BASAL LAMINAR DEPOSITS.

PURPOSE: To characterize structural and clinical alterations preceding the diffuse macular atrophy in extensive macular atrophy with pseudodrusen (EMAP) and their evolution toward atrophic changes. METHODS: A retrospective chart review was performed of patients with early-onset reticular pseudodrusen (i.e., pre-EMAP) younger than 55 years and EMAP with foveal sparing. Patients were included if they had complete medical records and multimodal imaging. RESULTS: A total of 12 patients were reviewed, of whom 4 of 12 patients (7 eyes) presented a pre-EMAP stage, characterized by the presence of pseudodrusen-like deposits without atrophic changes, while the remaining 8 of 12 patients (10 eyes) exhibited EMAP with foveal sparing (60.1 ± 6.4 years). Subretinal deposits of various stages tended to fade, leaving subretinal pigment epithelium accumulation of hyperreflective material with a physical separation between the retinal pigment epithelium-basal lamina and the Bruch membrane, along with the persistence of hyperreflective material after retinal pigment epithelium loss. These findings preceded atrophy development in a pre-EMAP stage and the EMAP stage with foveal sparing. CONCLUSION: Our findings presented distinct multimodal imaging features in eyes with reticular pseudodrusen depicting a peculiar phenotype of rapidly progressing atrophy in midlife. The disease spectrum may include other forms of geographic atrophy allied by thickened basal laminar deposits.

EFFICACY AND SAFETY OF AFLIBERCEPT FOR THE TREATMENT OF IDIOPATHIC CHOROIDAL NEOVASCULARIZATION IN YOUNG PATIENTS: The INTUITION Study.

PURPOSE: To evaluate the mean change in visual acuity at 52 weeks in patients with idiopathic choroidal neovascularization treated with aflibercept. METHODS: We conducted a prospective noncomparative open-label Phase-II trial. The dosage regimen evaluated in this study was structured into two periods: (1) from inclusion to 20 weeks: a treat-and-extend period composed of three mandatory intravitreal injections, and complementary intravitreal injections performed if needed; (2) from 21 weeks to 52 weeks: a pro re nata period composed of intravitreal injections performed only if needed. RESULTS: A total of 19 patients were included, and 16 completed the 52-week study. At baseline, the mean best corrected visual acuity was 66.56 (±20.72) letters (≈20/50 Snellen equivalent), and the mean central retinal thickness was 376.74 µm (±93.77). At 52 weeks, the mean change in the best-corrected visual acuity was +19.50 (±19.36) letters [95% confidence interval = +9.18 to +29.82]. None of the patients included lost ≥15 letters at 24 weeks or 52 weeks. The mean change in central retinal thickness was -96.78 µm (±104.29) at 24 weeks and -86.22 µm (±112.27) at 52 weeks. The mean number of intravitreal injections was 5.4 (±3.0) at 52-weeks. No ocular serious adverse events related to the treatment were reported. CONCLUSION: The present analysis shows clinically significant functional and anatomical treatment effect of aflibercept in case of idiopathic choroidal neovascularization. The treat-and-extend regimen proposed after the first injection seems adequate to treat most neovessels.

FOCAL CHOROIDAL THICKNESS HEMODYNAMICS AS a SIGN OF MACULAR NEOVASCULARIZATION ACTIVITY IN PATHOLOGIC MYOPIA.

PURPOSE: To analyze the relationship between a focal increase of choroidal thickness (ChT) and exudative activity of macular neovascularization (MNV) secondary to pathologic myopia. METHODS: Retrospective analysis including eyes with pathologic myopia presenting with a focally increased ChT underneath active MNV. All patients included were treated, and ChT was measured before and after each intravitreal injection by two experienced ophthalmologists. RESULTS: Fifty-two eyes of 52 patients with myopic MNV (19 men and 33 women) were included in this analysis. ChT at T-1 averaged 51.09 ± 33.56 µ m, whereas at the time of MNV activation (T0), ChT was significantly thicker: 85.11 ± 43.99 µ m ( P < 0.001). After a single intravitreal injection, the ChT significantly decreased to 53.23 ± 34.15 µ m ( P < 0.001). CONCLUSION: This study showed that focal ChT variations may be considered an interesting corollary sign of MNV in high myopic patients, indicating the activity of myopic neovascularization.

EFFICACY OF HALF-DOSE PHOTODYNAMIC THERAPY VERSUS HIGH-DENSITY SUBTHRESHOLD MICROPULSE LASER FOR TREATING PIGMENT EPITHELIAL DETACHMENTS IN CHRONIC CENTRAL SEROUS CHORIORETINOPATHY.

PURPOSE: Comparing the effect of half-dose photodynamic therapy and high-density subthreshold micropulse laser treatment on retinal pigment epithelial detachments (PEDs) in chronic central serous chorioretinopathy. METHODS: This study included data from the PLACE trial, a prospective randomized controlled trial comparing half-dose photodynamic therapy and high-density subthreshold micropulse laser treatment in chronic central serous chorioretinopathy. Main outcome measurements were changes in both the foveal PED and the highest PED within the macula at baseline compared with first and final evaluation visit. RESULTS: At baseline, a macular PED was detected in 76.9% of patients (123/160), and a PED within 1,500 µm from the foveal center in 37.5% of patients (60/160). In the half-dose photodynamic therapy arm (61 patients), there was a significantly larger decrease in the highest macular PED compared with the high-density subthreshold micropulse laser treatment arm (62 patients) at both first and final evaluation visits (P < 0.001 and P = 0.012, respectively). The decrease of highest foveal PED was significant at first visit (P = 0.025). CONCLUSION: Half-dose photodynamic therapy is superior to high-density subthreshold micropulse laser treatment with regard to a statistically significant reduction in the height of macular PEDs in active chronic central serous chorioretinopathy. These findings may also have implications for other diseases within the pachychoroid disease spectrum that can present with PEDs.

Prognostic factors of idiopathic epiretinal membrane surgery and evolution of alterations of the central cone bouquet.

PURPOSE: To describe the structural changes observed postoperatively in epiretinal membranes (ERM), in particular the alterations in the central cone bouquet (CB), and to identify prognostic factors that might predict postoperative outcome. METHODS: We included 125 eyes of 117 patients who underwent idiopathic ERM removal with at least a 6-month follow-up. For each patient, spectral-domain optical coherence tomography (SD-OCT) was performed and best-corrected visual acuity (BCVA) was measured, before and after surgery. RESULTS: Before surgery, 44 eyes (35.2%) presented CB alterations: 65.9% a cotton ball sign, 15.9% a foveolar detachment and 18.2% a pseudovitelliform lesion. Median BCVA increased from 20/63 to 20/32 post-operatively (p = .001) with a mean follow-up of 17 months. The disappearance of CB alterations after surgery was observed in 97.7% of eyes. In stage 3 and 4 ERM, ectopic inner foveal layers persisted in 76.7% of eyes after surgery. Postoperative BCVA was correlated with change in central macular thickness and initial BCVA and was not correlated with the presence of preoperative CB alteration, the initial stage of ERM, the presence of postoperative dissociated optical nerve fiber layer, and the disappearance of ectopic inner fiber layers. The combination of cataract surgery and capsulotomy did not seem to change visual outcome and seemed to accelerate visual recovery. Incidentally, general anesthesia was correlated with final BCVA. CONCLUSION: ERM surgery allowed a significant gain in BCVA and the disappearance of CB alterations in the great majority of cases. CB alteration did not show to be associated with poor visual prognosis.

QUANTITATIVE OPTICAL COHERENCE TOMOGRAPHY ANGIOGRAPHY FEATURES OF INACTIVE MACULAR NEOVASCULARIZATION IN AGE-RELATED MACULAR DEGENERATION.

PURPOSE: To compare quantitative optical coherence tomography angiography parameters between treatment-naïve quiescent macular neovascularizations (MNVs) and previously treated nonexudative Type 1 MNVs, in patients with age-related macular degeneration. METHODS: The eyes included in the study were analyzed by fluorescein angiography, indocyanine green angiography, spectral-domain optical coherence tomography, and optical coherence tomography angiography. According to their medical history and multimodal imaging evaluation, Type 1 MNVs were divided into 2 groups: 1) treatment-naïve quiescent MNVs; 2) previously treated nonexudative Type 1 MNVs. Quantitative optical coherence tomography angiography parameters, including perfusion density (PD), fractal dimension (FD), and lacunarity (LAC) were calculated. Receiver operating characteristic curves, showing the ability of PD, FD, and LAC to discriminate between the two MNV groups, were built. RESULTS: Twenty-two eyes with treatment-naïve quiescent MNVs and 20 eyes with MNVs previously treated nonexudative Type 1 MNVs were analyzed. Mean FD and LAC were statistically different between the two study groups (P < 0.05). Lacunarity showed the best discrimination ability, followed by FD and PD (area under curve = 0.83, 0.78, 0.62, respectively). CONCLUSION: Results suggest that FD and LAC may be useful optical coherence tomography angiography biomarkers to objectively discriminate inactive MNVs with different prognosis, such as treatment-naïve quiescent MNVs and previously treated nonexudative Type 1 MNVs, in age-related macular degeneration patients.

INTRARETINAL HYPERREFLECTIVE LINES.

PURPOSE: To report intraretinal hyperreflective lines related to various macular conditions. METHODS: All cases were imaged with color photographs, autofluorescence images, and spectral-domain optical coherence tomography, some with fluorescein and/or indocyanine green angiography. Demographic data, imaging, course and outcome were retrospectively analyzed. RESULTS: Forty-nine eyes of 43 patients (16 men and 27 women) were included. Hyperreflective vertical lines (38 eyes) or curvilinear lines along the Henle fiber layer (11 eyes) were present in association with various macular conditions: adult vitelliform dystrophy or pattern dystrophy (24 eyes) frequently associated with an epiretinal membrane (six eyes) and/or thick choroid (nine eyes), age-related maculopathy or macular degeneration (nine eyes), partial resorption of subretinal or intraretinal hemorrhages (five eyes), idiopathic macular microhole (two eyes), vitreomacular traction (three eyes), multiple evanescent white dot syndrome (three eyes), fundus flavimaculatus (two eyes), and pachychoroid pigment epitheliopathy (one eye). The lines fully vanished in cases of hemorrhages, multiple evanescent white dot syndrome or resolution of vitreomacular traction, but usually persisted with gradual thinning in the other conditions. CONCLUSION: The present series showed that intraretinal hyperreflective lines could occur in various inflammatory, degenerative, or tractional conditions. They could reflect a previously unrecognized reaction to various photoreceptor, Müller cell, and/or retinal pigment epithelium damage.

CHORIOCAPILLARIS FLOW IMPAIRMENT IN TYPE 3 MACULAR NEOVASCULARIZATION: A Quantitative Analysis Using Swept-Source Optical Coherence Tomography Angiography.

PURPOSE: To quantitatively analyze choriocapillaris alterations using swept-source optical coherence tomography angiography in eyes presenting with Type 3 macular neovascularization (MNV) and to compare these alterations with eyes presenting with intermediate AMD (iAMD). METHODS: Macular 3 × 3-mm swept-source optical coherence tomography angiography scans were retrospectively analyzed in eyes with Type 3 MNV and in eyes with iAMD. The choriocapillaris en face slabs were extracted from the swept-source optical coherence tomography angiography device after manual segmentation. En face choriocapillaris flow images were compensated with en face choriocapillaris structure images, followed by the Phansalkar local thresholding method using a window radius of 4 and 8 pixels. The percentage of flow deficits (FD%), the number, size, and total area of FDs were computed for comparison. A secondary analysis was performed in the four corners of the image to include equidistant regions in all eyes. RESULTS: Twenty-six Type 3 MNV eyes of 21 patients and 26 iAMD eyes of 17 patients were included. Compared with iAMD eyes, eyes with Type 3 MNV displayed a higher FD% (41.37% ± 14.74 vs. 19.80% ± 9.63 using radius 4 pixels [P < 0.001]; 45.24% ± 11.9 vs. 26.63% ± 8.96 using radius 8 pixels [P < 0.001]). The average size of FDs was significantly larger in Type 3 MNV eyes compared with iAMD eyes (P < 0.001), whereas the number of FDs was significantly lower in Type 3 MNV compared with iAMD eyes (P < 0.001). CONCLUSION: Type 3 MNV eyes present with increased choriocapillaris flow impairment compared with iAMD eyes. Reduced choriocapillaris perfusion may contribute to Type 3 MNV development and pathogenesis.