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Background Neurofibromatosis, Von Hippel Lindau disease, and tuberous sclerosis complex are classified under the term phakomatoses. They are characterized by ocular vascular abnormalities such as vascular tortuosity, corkscrew retinal vessel configuration, moyamoya-like aspect, microaneurysms, hemangioblastomas, and focal sheathing of retinal arteries, possibly due to abnormal formation, migration, and differentiation of neural crest cells. These alterations can be the first sign or the hallmark of disease and can be related to vasoproliferative tumors. Purpose Novel imaging technologies in ophthalmology, such as near-infrared reflectances and spectral domain optical coherence tomography, have improved our knowledge in the diagnosis of these pathologies. Previously undetected macular vascular alterations have been reported in phakomatoses using optical coherence tomography angiography. This review will summarize the ophthalmic vascular abnormalities and novel imaging methods in the phakomatoses. Conclusion Active research is being led into the ophthalmic management of these conditions and their complications, and owing to elevated vascular endothelial growth factor production from hemangioblastoma, hamartoma, and retinal vascular proliferative tumors, increasing interest in this line of therapy has been conducted although research is still ongoing in this area.

Purpose To describe the presence of subretinal lipid globules (SLG), analyze the multimodal imaging features inherent in their optical properties, and provide a means to distinguish them from other retinal structures and clinical signs. Design Retrospective cohort study. Methods The clinical data and multimodal imaging features of 39 patients (49 eyes) showing SLG were evaluated. Patients underwent color fundus photography, near-infrared reflectance (NIR), spectral-domain (SD) and swept-source (SS) optical coherence tomography (OCT) and OCT angiography. In vitro phantom models were used to model OCT optical properties of water, mineral oil, and intralipid droplets and to investigate the optical mechanisms producing hypertransmission tails beneath SLG. Results The SLG were not visible in color fundus photographs or in NIR images. With both SD- and SS-OCT B-scans, SLG appeared as 31-157 μm, round, hyporeflective structures demonstrating a characteristic hypertransmission tail previously described with lipid globules found in the choroid and in neovascular membranes. Similarly, with en face OCT, SLG appeared as small, round, hyporeflective structures. SLG were encountered most often in eyes with neovascular age-related macular degeneration (AMD) that had type 1 macular neovascularization (MNV) (91.1%). Of those eyes, 93.3% were receiving intravitreal antivascular endothelial growth factor (VEGF) therapy (median of 15 injections) with a mean follow-up of 52.6 months. The number of prior injections positively correlated with the number of SLG. The detection of MNV preceded the presence of SLG in 66.7% of cases. En face OCT showed that, in many eyes (49%), SLG appeared in clusters of >10. In 38.8% of eyes, SLG were found overlying type 1 MNV, and in 44.9% of eyes, often those with more numerous SLG, the SLG were located near the lesion border. In 2 eyes with AMD followed for nonexudative type 1 MNV, SLG were detected prior to the detection of other imaging signs of exudation. SLG were observed in several other exudative macular diseases. Phantom models demonstrated that the hypertransmission tail beneath SLG is related to a lensing effect produced by these hyporeflective spherical structures. Conclusions SLG are a newly recognized OCT feature frequently seen in eyes receiving intravitreal anti-VEGF therapy for type 1 MNV due to AMD. OCT B-scans show SLG as small, round, hyporeflective structures with a characteristic hypertransmission tail. This OCT signature is influenced by the OCT focal plane, and it relates to reduced signal attenuation through oil and a lensing effect created by a higher refractive index compared to surrounding tissue.

PURPOSE To use swept-source optical coherence tomography and swept-source optical coherence tomography angiography to investigate potential relationships between choroidal vascular hyperpermeability (CVH) seen with indocyanine green angiography (ICGA), choriocapillaris flow density, and choroidal thickness in eyes with pachychoroid pigment epitheliopathy. METHODS Patients with pachychoroid pigment epitheliopathy were prospectively imaged with 12-mm × 12-mm swept-source optical coherence tomography, 12-mm × 12-mm swept-source optical coherence tomography angiographyA, and ICGA. Binarized choriocapillaris OCTA images were superimposed with ICGA images in which CVH area had been isolated. Choriocapillaris flow density within or outside the quadrants of CVH was calculated and the ratio of these two values was determined. The presence of CVH and choroidal thickness was evaluated at 9 locations within a central 3-mm × 3-mm area to explore the relationship between these 2 factors. RESULTS Ten eyes from 10 patients were enrolled in the present study. Choriocapillaris flow density within quadrants of CVH area was significantly lower compared with quadrants without CVH (P < 0.001). The mean choriocapillaris flow density ratio was 0.86 ± 0.10 (range: 0.65-0.99). From among the 90 locations in 10 study eyes, 48 were within areas of CVH. Choroidal thickness was greater in quadrants of CVH compared with areas without CVH (P < 0.001, 455 ± 122 µm vs. 297 ± 93 µm). CONCLUSION Reduced choriocapillaris flow density, increased choroidal thickness, and CVH appear to co-localize in eyes with pachychoroid pigment epitheliopathy.

Glaucoma is a group of optic neuropathies characterized by a progressive degeneration of retina ganglion cells (RGCs) and their axons that precedes functional changes detected on the visual field. The macular ganglion cell complex (GCC), available in commercial Fourier-domain optical coherence tomography, allows the quantification of the innermost retinal layers that are potentially involved in the glaucomatous damage, including the retinal nerve fiber (RNFL), ganglion cell and inner plexiform layers. The average GCC thickness and its related parameters represent a reliable biomarker in detecting preperimetric glaucomatous damage. The most accurate GCC parameters are represented by average and inferior GCC thicknesses, and they can be associated with progressive visual field loss. Although the diagnostic accuracy increases with more severe glaucomatous damage and higher signal strength values, it is not affected by increasing axial length, resulting in a more accurate discrimination of glaucomatous damage in myopic eyes with respect to the traditional RNFL thickness. The analysis of the structure-function relationship revealed a good agreement between the loss in retinal sensitivity and GCC thickness. The use of a 10-2° visual field grid, adjusted for the anatomical RGCs displacement, describes more accurately the relationship between RGCs thickness and visual field sensitivity loss.

PURPOSE: To assess the clinical significance of suspended scattering particles in motion (SSPiM) and different cystic phenotypes in diabetic macular oedema (DME) treated with dexamethasone implant (DEX-i). METHODS: A retrospective review of type 2 diabetic patients with DME treated with a DEX-i was conducted. Swept-source optical coherence tomography angiography (OCTA, PLEX Elite 9000) with a 3-mm volume cube was performed. Regions of interest were delineated with Fiji software (version 2.1.0/1.53.c) in the superficial vascular complex (SVC) and deep capillary plexus (DCP) at baseline, 2- and 4-months after DEX-i. SSPiM was defined as regions of variable reflectivity with a decorrelation signal. Without a detectable decorrelation signal, its counterpart was addressed as ‘corpuscular,’ while hyporeflective cysts were optical empty without hyperreflective material enclosed. RESULTS: After treatment, the hyporeflective component demonstrated substantial reabsorption in the SVC (–95.4% at 2- and –84.4% at 4-months, p < 0.01 both) and DVC (–84.4%, 2-months), with a less critical decrease of the corpuscular component in the SVC (2-months: –41.9%, p = 0.001 and 4 months: –1.8%, p = 0.73), and not significant in the DVC. SSPiM did not significantly change in the SVC and DVC neither at 2- and 4-months (p > 0.05, all). CONCLUSIONS: After a single DEX-i, the clearance of different cystic phenotypes proceeds with resorption of hyporeflective, followed by corpuscular components. SSPiM demonstrated minimal response, indicating a severe BRB breakdown that may require repeated treatment to reach a satisfactory anatomical response.

Near-infrared reflectance (NIR) is a noninvasive, contactless, and rapid in vivo imaging technique for visualizing subretinal alterations in the photoreceptor layer, retinal pigment epithelium, and choroid. The present report describes the application of this imaging method in retinal and choroidal pathologies affecting young patients where scarce cooperation, poor fixation, and intense glare sensation can result in a challenging clinical examination. A literature search of the MEDLINE database was performed using the terms “near-infrared reflectance” and “spectral-domain optical coherence tomography.” Articles were selected if they described the diagnostic use of NIR in children or young adults. Of 700 publications, 42 manuscripts published between 2005 and 2020 were inherent to children or young adults and were considered in this narrative literature review. The first disease category is the phakomatoses where NIR is essential in visualizing choroidal alterations recognized as cardinal biomarkers in neurofibromatosis type 1, microvascular retinal alterations, and retinal astrocytic hamartomas. Another diagnostic application is the accurate visualization of crystals of various nature, including the glistening crystals that characterize Bietti crystalline dystrophy. Acute macular neuropathy and paracentral acute middle maculopathy represent a further disease category with young adulthood onset where NIR is not only diagnostic but also essential to monitor disease progression. A further interesting clinical application is to facilitate the detection of laser-induced maculopathy where funduscopic examination can be normal or subnormal. In conclusion, NIR imaging has a noninterchangeable role in diagnosing certain retinal diseases, especially in children and young adults where there is scarce collaboration and a lack of evident clinical findings. Moreover, this technique can reveal unique retinal and choroidal biomarkers highly specific to rare conditions.

PURPOSE To describe clinical features including multimodal imaging in a rare case of presumed embolic cilioretinal artery occlusion with long-term follow-up examination. METHODS A 61-year-old man presented after noticing an acute-onset central scotoma in the left eye during coronary arteriography. Fundus color photography, red-free imaging, near-infrared reflectance, spectral domain optical coherence tomography (OCT), fluorescein angiography, and swept-source OCT angiography were performed. RESULTS Color and red-free fundus imaging both showed retinal whitening in the territory corresponding to a cilioretinal artery in the left eye. Consistent with an ischemic event, the near-infrared reflectance showed a hyporeflective area in the same location. Spectral domain OCT showed hyperreflectivity and generalized thickening of the inner nuclear layer, ganglion cell layer, and retinal nerve fiber layer. Seven years later, color, red-free, and near-infrared reflectance imaging appeared normal, but the spectral domain OCT showed thinning of all retinal layers in the affected area. Furthermore, swept-source OCT angiography demonstrated markedly reduced capillary flow signal in the superficial and deep capillary plexuses, corresponding to a persistent scotoma described by the patient. CONCLUSION This case report illustrates hallmark features of a rare presumed embolic cilioretinal artery occlusion and its long-term manifestations with thorough historical and multimodal imaging data. When standard fundus imaging with color, red-free, and near-infrared reflectance appears normal years after a retinal vascular insult, OCT and OCT angiography can corroborate a patient's persistent visual deficit and provide clarity to the diagnosis.

Serena Fragiotta,1 Pedro Fernández-Avellaneda,2 Mark P Breazzano,3 Gianluca Scuderi1 1NESMOS Department, Ophthalmology Unit, St. Andrea Hospital, University of Rome “La Sapienza”, Rome, Italy; 2Department of Ophthalmology, Basurto University Hospital, Bilbao, Spain; 3Wilmer Eye Institute, Johns Hopkins Hospital, Johns Hopkins University, Baltimore, MD, UACorrespondence: Serena FragiottaNESMOS Department, Ophthalmology Unit, St. Andrea Hospital, University of Rome “La Sapienza”, Via di Grottarossa 1035-1039, Rome, 00189, ItalyTel +39 3293276433Fax +39 0633776628Email s.fragiotta@hotmail.itAbstract: Cuticular drusen are part of the spectrum of age-related macular degeneration (AMD) with particular clinical and multimodal imaging characteristics. This drusen subpopulation shares several high-risk single nucleotide polymorphisms with AMD. Despite this feature, they can manifest at a relatively young age, presenting with a female preponderance. Multimodal imaging is essential for characterizing such lesions, using a combination of color fundus photographs, optical coherence tomography (OCT), fluorescein angiography (FA), and fundus autofluorescence (FAF). The classic starry-sky pattern visible on FA and the typical central hypoautofluorescent lesion with hyperautofluorescent rim on FAF is considered the result of a central retinal pigment epithelium (RPE) erosion from these triangular elevations of the RPE-basal lamina. This finding may also be responsible for the typical choroidal hypertransmission appreciated through OCT. The clinical course of cuticular drusen may be relatively benign at early stages, with small drusen presenting at a young age. However, the presence of clinical phenotypes characterized by diffuse involvement and/or accompanying large drusen in patients older than 60 years may confer a significant risk for either macular neovascularization or geographic atrophy.Keywords: cuticular drusen, spectral-domain optical coherence tomography, fundus autofluorescence, fluorescein angiography, multimodal imaging

Purpose: To evaluate the effect of IOP lowering on the capillary density of optic nerve head and retinal nerve fiber layer in patients with primary open angle glaucoma. Methods: Twenty eyes of 14 glaucomatous patients and 15 eyes of nine normal patients were enrolled. The most appropriate hypotonic treatment was applied to every patient. A HD Angio Disc 4.5 scan (Avanti-AngioVue) was performed at baseline and after a month in the glaucomatous eyes. The following parameters were analyzed: Radial Papillary Capillaries (RPC) density, inside disc, peripapillary, superior-hemi, inferior-hemi, quadrants, and peripapillary, hemi-superior, hemi-inferior, and quadrants RNFL thickness. Optic nerve head analysis was also evaluated. In addition, the RPC density and the RNFL were assessed in the eight sectors provided by the software. Results: The RPC density did not significantly change after IOP reduction ( p > 0.05). The inferior-temporal ( p = 0.005) and inferior-nasal sectors ( p Conclusion: The glaucomatous eyes presented rarefaction of the radial papillary capillaries density in the inferior sectors, but no significant changes in the density after IOP-lowering medications.

PURPOSE To investigate the association between choroidal caverns, choroidal vascular hyperpermeability (CVH), and pachyvessels in eyes with pachychoroid disease. METHODS This was a retrospective review of swept-source optical coherence tomography and indocyanine green angiography imaging performed on eyes with pachychoroid disease. RESULTS Imaging from 21 eyes with pachychoroid disease entities (8 eyes with pachychoroid pigment epitheliopathy, 11 eyes with central serous chorioretinopathy, and 3 eyes with pachychoroid neovasculopathy) from 11 patients (mean 49.5 years, male/female: 10/1, all white) was available for review. In all study eyes, pachyvessels traversed the areas of CVH visible in mid- and late-phase indocyanine green angiography. A total of 504 choroidal caverns were identified in 11 study eyes (52%). Of the 504 choroidal caverns, 445 (88%) were seen within the areas of CVH compared with 59 (12%), which were detected outside the areas of CVH (P < 0.001). Eyes with multiple caverns had an increased choroidal thickness when compared with eyes with ≤1 cavern (P < 0.001). CONCLUSION Choroidal caverns, found primarily in the areas of indocyanine green angiography CVH traversed by pachyvessels, were detected in 52% of eyes with pachychoroid disease. The presence of choroidal caverns in these cases may indicate a loss of normal choroidal architecture associated with dilated Haller layer veins and increased choroidal thickness.

PURPOSE To investigate the risk factors predictive for the development of neovascular age-related macular degeneration (NVAMD) by means of spectral-domain optical coherence tomography. METHODS Retrospective study of 73 eyes graded Stage 2 and Stage 3 according to the AMD International Grading System with minimum follow-up of 24 months. Drusenoid pigment epithelial detachment, hyperreflective foci, external limiting membrane, inner ellipsoid band, and retinal pigment epithelium integrity were analyzed at baseline and last follow-up. Binary logistic regression model analyzed significant predictors of neovascular conversion. RESULTS The discontinuity of external limiting membrane, inner ellipsoid band, and retinal pigment epithelium bands were significantly more prevalent in the NVAMD group at baseline and last follow-up (P < 0.001). Hyperreflective foci represented the single most important predictor of neovascular conversion (Exp [B], 15.15; P = 0.005) as confirmed by Kaplan-Meier curve (P = 0.002). Drusenoid pigment epithelial detachment width was significantly greater in NVAMD group than control subjects at baseline and last follow-up (P < 0.001), and its delta value also resulted a significant neovascular predictor (Exp [B], 0.99; P = 0.04). CONCLUSION Hyperreflective foci significantly increase the risk of NVAMD progression. The delta width of drusenoid pigment epithelial detachment also predicts disease progression, integrating the stratification of NVAMD progression risk.

Acute exudative polymorphous vitelliform maculopathy (AEPVM) is a rare bilateral maculopathy characterized by chronic and long-term course. We report a case of AEPVM with an unusual presentation and management in a middle-aged man. He presented with clinical features of bilateral AEPVM accompanied by multiple intraretinal cysts, with a sudden increase of intraretinal fluid and visual function deterioration over a span of few days. Therefore, we administered empirically an intravenous bolus injection of methylprednisolone. One week after, there was a full recovery of visual acuity and cystic intraretinal spaces completely disappeared.

This pilot study aims to investigate choroidal vascular status in eyes with adult-onset foveomacular vitelliform dystrophy (AOFVD), early age-related macular degeneration (AMD), and age-matched controls. In this retrospective study, choroidal thickness (CT) was measured manually using spectral domain optical coherence tomography images of the fovea, and 500 and 1500 µm from the nasal and temporal regions in the fovea. The horizontal B-scan was imported into Fiji software. Choroidal vascularity index (CVI) and luminal and stromal areas were calculated. A total of 36 eyes from 36 patients, including 18 eyes with AOFVD and 18 eyes with CD, and 16 eyes of healthy subjects were included. CVI was significantly different among subgroups (ANOVA, p = 0.004). Eyes with AOFVD presented a higher CVI (+0.03 ± 0.01, p = 0.001) than eyes with CD and controls (p = 0.03). No differences in CVI were detected between controls and eyes with CD (p = 0.25). AOFVD eyes accounted for the greatest luminal area, particularly significant in comparison with healthy controls (+0.27 ± 0.11, p = 0.02). AOFVD eyes present a greater CVI than eyes with CD and controls. The major choroidal involvement is on the luminal component, further corroborating a possible role of the choroidal vasculature in the pathological manifestations of AOFVD disease.

Purpose To characterize the topographic relationships among vitreous structures, including the premacular bursa, prevascular vitreous fissures, cisterns, and lacunae, in healthy participants using en face and cross-sectional swept-source (SS) OCT. Design Prospective, comparative study. Participants Sixty eyes of 60 healthy participants (age range, 4–35 years). Eyes of individuals younger than 20 years (n = 29) were compared with eyes of individuals 20 years of age or older (n = 31). Methods From each study eye, 12 × 12-mm SS OCT volume scans comprising 1024 × 1024 A-scans centered at the fovea were acquired. Main Outcome Measures En face and cross-sectional data were analyzed to characterize topographic relationships between hyperreflective spaces anterior to the vitreoretinal interface. Results Prevascular vitreous fissures are an almost universal feature of human eyes. Cisterns became more prevalent over the course of the first 20 years (r = 0.49; P = 0.002). In 97% of eyes in individuals older than 20 years, en face and cross-sectional SS OCT showed the premacular bursa and prepapillary gap merge at a distance superior to the optic nerve and then follow a superonasal course anteriorly. However, only 69% of individuals younger than 20 years demonstrated such a connection (P = 0.01). A close topographic relationship of vitreous fissures and cisterns to the underlying vasculature of the posterior pole was visible on en face projections. En face imaging readily distinguished these spaces. Degenerative, eyewall-parallel fissure planes and their course were described for the first time in a 3-dimensional manner. The fissure planes were rare in younger eyes (12%) and significantly more common in older eyes (42%; P Conclusions En face SS OCT demonstrated that (1) premacular bursa and Cloquet’s canal are not connected in younger patients, but are connected in older patients; (2) prevascular vitreous fissures overly the retinal vessels; and (3) cisterns are continuous with prevascular fissures.

To investigate the prevalence and progression of vitreo-macular interface disorders (VMID) phenotypes and their natural history in retinitis pigmentosa (RP). A total of 257 eyes of 145 RP patients with VMID were retrospectively evaluated. Patients were divided according to the VMID subtypes into epiretinal membranes (ERMs), vitreo-macular traction (VMT) group, and macular hole (MH). Serial eye-tracked spectral-domain optical coherence tomography (SD-OCT) and best-corrected visual acuity (BCVA) changes were analyzed for a mean follow-up of 36.95 months. The status of posterior vitreous cortex was also considered. A control group of 65 eyes belonging to 65 RP patients with no macular changes was also recruited. VMID and control groups had the same baseline BCVA (0.50 vs 0.44 LogMAR) and did not differ in terms of phakic status. Different VMID groups had similar BCVA at baseline (p = 0.98). ERM represented the most prevalent disorder (207/257 eyes, 80.5%), followed by 35/257 (13.6%) VMT, and 15/257 Lamellar MH (LMH) eyes (5.8%). There were no cases of full thickness MH. Throughout the 36.9 months of follow-up, BCVA decreased an average 0.09 LogMAR from 0.31 to 0.4 in VMID patients and 0.01 in controls. VMID subgroup analysis showed a significant BCVA decrease in ERM patients (− 20.29%, p

Purpose To report new multimodal imaging features that enhance our understanding of the inflammatory and ischemic nature of acute idiopathic maculopathy (AIM) and to correlate structural and functional changes due to a reversible initial choroidal ischemia over a clinical course of 3.5 months. Methods A 31-year-old man presented with acute central vision loss in his right eye due to coxsackievirus-associated AIM. Serial multimodal retinal imaging including confocal true color fundus photography, blue-light fundus autofluorescence (BAF), near-infrared reflectance (NIR), spectral domain optical coherence tomography and swept-source optical coherence tomography (SD-OCT + SS-OCT), and en face SS-OCT angiography (SS-OCTA) were performed over a 3.5-month follow-up. Eidon true color confocal scanner camera (Centervue, Padova, Italy) was used for color and BAF imaging. Near-infrared reflectance and SD-OCT images were obtained with the Heidelberg Spectralis OCT (HRA2 + OCT; Heidelberg Engineering, Heidelberg, Germany). For SS-OCT and en face SS-OCT and SS-OCTA images, the PLEX Elite 9,000 (Carl Zeiss Meditec, Inc, Dublin, CA) was used. Central alterations in choriocapillaris flow were analyzed with SS-OCTA using the University of Washington choriocapillaris (CC) flow deficit quantification algorithm available through the ARI Network. Flow deficit area and density values were analyzed and compared between the first and last examinations. Corresponding en face OCT imaging was used to distinguish true flow defects from artifacts secondary to shadowing. Results In the acute stage of AIM, a bacillary layer detachment appearing as a yellow-grayish foveal elevation surrounded by a hypopigmented parafoveal ring was evident in a Bull's eye configuration, corresponding to a hyperreflective ring upon NIR and a hyperautofluorescent ring with BAF. SD + SS-OCT showed mostly intraretinal fluid consistent with a bacillary layer detachment in conjunction with a thickened inner choroid. At presentation, SS-OCTA demonstrated a marked reduction of choriocapillaris flow signal. At 1 week, early resolution of retinal fluid was followed by restoration of the ellipsoid zone at 5 weeks, while restoration of the interdigitation zone and reduction in retinal pigment epithelium/Bruch membrane complex thickening occurred more slowly. Swept-source OCT angiography showed a gradual, but incomplete, recovery of inner choroidal flow signal at 3.5-month follow-up. Conclusion Acutely, AIM may present with a photoreceptor splitting foveal bacillary layer detachment associated with a marked reduction in inner choroidal flow signal on SS-OCTA. Thereafter, restoration of the outer retinal layers and gradual normalization of choroidal flow signal appear to support the often-benign nature of the disease.

Purpose To explore patterns of disease progression in nonneovascular age-related macular degeneration (AMD) associated with hyperreflective crystalline deposits (HCDs) in the sub-retinal pigment epithelium-basal laminar space. Methods Retrospective review of medical records, multimodal imaging, and longitudinal eye-tracked near-infrared reflectance (NIR) and optical coherence tomography (OCT) spanning ≥2 years. NIR/OCT images were analyzed with ImageJ software to identify HCD morphology and location. Associated macular complications were reviewed from the time of HCD detection to the most recent follow-up, using NIR/OCT. Results Thirty-three eyes with HCDs from 33 patients (mean age: 72 ± 7.5 years) had 46.7 months (95% confidence limits: 33.7, 59.6) of serial eye-tracked NIR/OCT follow-up. Baseline best-corrected visual acuity (BCVA) was 0.44 logMAR (Snellen equivalent 20/55). At a mean of 11.3 months (3.1, 19.6) after HCD detection, 31/33 (93.9%) eyes had developed macular complications including de novo areas of complete retinal pigment epithelium and outer retinal atrophy (cRORA) in 21/33 (64%) eyes, enlargement of preexisting cRORA in 4/33 (12%) eyes, and incident macular neovascularization in 3/33 (9%) eyes. Movement and clearance of HCDs in 9/33 (27%) eyes was associated with enlargement of preexisting cRORA (r = 0.44, P = 0.02). BCVA at the last follow-up visit had decreased to 0.72 logMAR (20/105). Conclusions Eyes with nonneovascular AMD demonstrating HCDs are at risk for vision loss due to macular complications, particularly when movement and clearance of these structures appear on multimodal imaging. HCD reflectivity and dynamism may be amenable to automated recognition and analysis to assess cellular activity related to drusen end-stages.

Background Aneurysmal type 1 neovascularization (AT1) is a term recently introduced to better describe the aneurysmal dilatation that may arise from neovascular lesions, more commonly known as polypoidal choroidal vasculopathy. The proposed term, AT1, includes an expanded clinical spectrum of aneurysmal (polypoidal) lesions observed in both different ethnicities and associated with varied clinical phenotypes. Case presentation A 61-year-old woman of European descent was referred for a new, asymptomatic retinal hemorrhage found on routine examination. Ophthalmoscopy revealed cuticular drusen in both eyes best appreciated on fundus autofluorescence, and a hemorrhagic retinal pigment epithelium detachment above the superior arcade in the right eye. In the fellow eye, a reddish appearing pigment epithelial detachment was noted nasal to the optic nerve. Indocyanine green angiography showed findings of AT1 in both eyes. Optical coherence tomography angiography showed intrinsic flow signal within the aneurysmal lesions. Conclusions Eyes with cuticular drusen may develop AT1 which, to our knowledge, has not been described. This is an important observation because the documented coexistence of AT1 in the setting of a variant of age-related macular degeneration lends supports to this new understanding of AT1 as a growth pattern of neovascular tissue proliferating between the RPE and Bruch membrane, rather than as a distinct disease entity.

Objective To assess the role of CD3+ CD20+ CD4- CD8- double-negative (DN) or CD3+CD20+ CD4/CD8+ T cells and the related pro-inflammatory cytokines in the humor aqueous, in mediating retinal microvascular changes in patients with chronic plaque-type moderate to severe psoriasis. Design. A total of 76 patients (57.6 ± 11.7 years) with chronic plaque-type psoriasis were initially evaluated. Nineteen patients (19 eyes) and 19 healthy volunteers (19 eyes) were subjected to dermatological evaluation with Psoriasis Area Severity Index (PASI) and the Dermatology life quality index (DLQI). Retinal images were processed using an automatized software. On the same day, a venous sample was collected and analyzed using multiparametric flow cytometry. Three out of 6 patients who presented cataract, consented to perform surgery with humor aqueous collection. The samples were analyzed using a Multi-Analyte ELISA kit for the simultaneous quantification of IL1α, IL1β, IL2, IL4, IL6, IL8, IL10, IL12, IL17A, IFNγ, TNF-α, GMCSF. Results The CD3+CD4+/CD8+CD20+CD56- T cells expression was greater in the psoriatic patients (+73.9%, P Conclusion Our findings demonstrated that CD20+ T cell subpopulation is highly represented in psoriasis regardless of the use of immunomodulatory therapies, and the diffuse microvascular alterations suggested possible endothelial damage as mainstream for the genesis of psoriatic-mediated complications as further supported by the comparable concentrations of cytokines, at least as humor aqueous content, with respect to healthy eyes.

Background/aimsTo study the multimodal imaging findings of a large series of eyes with cilioretinal artery obstruction (CILRAO) and describe the systemic associations.MethodsMulticentre, retrospective chart review from 12 different retina clinics worldwide of eyes with CILRAO, defined as acute retinal whitening in the distribution of the cilioretinal artery, were identified. The clinical, systemic information and multimodal retinal imaging findings were collected and analysed.ResultsA total of 53 eyes of 53 patients with CILRAO were included in the study. In 100% of eyes, fundus photography illustrated deep retinal whitening corresponding to the course of the cilioretinal artery. Twenty-eight patients (52.8%) presented with isolated CILRAO (baseline best-corrected visual acuity (BCVA) 20/50, final BCVA 20/25) associated with nocturnal hypotension, 23 patients (43.4%) with CILRAO secondary to central retinal vein occlusion (CRVO) (baseline BCVA 20/40, final BCVA 20/20) and two patients with CILRAO due to biopsy-proven giant cell arteritis (GCA) (baseline BCVA 20/175, final BCVA 20/75). With spectral domain optical coherence tomography (SD-OCT), a hyper-reflective band involving the inner nuclear layer (ie, paracentral acute middle maculopathy or PAMM) was noted in 51 eyes (28/28 eyes with isolated CILRAO and 23/23 eyes with CILRAO+CRVO) corresponding to the retinal whitening. In the two eyes with CILRAO+GCA, SD-OCT illustrated hyper-reflective ischaemia of both the middle and inner retina.ConclusionsIsolated CILRAO and CILRAO secondary to CRVO are the result of hypoperfusion or insufficiency, rather than occlusion, of the cilioretinal artery and are associated with PAMM or selective infarction of the the inner nuclear layer. With GCA, there is complete occlusion of the cilioretinal artery producing ischaemia involving both the middle and inner retina associated with worse visual outcomes.

SIGNIFICANCE The authors analyze factors influencing bivariate contour ellipse area (BCEA) in healthy and pathologic eyes and how such factors may affect isolated (static BCEA) or microperimetric fixation (dynamic BCEA). They conclude that aging increases both dynamic BCEA and examination time, whereas static BCEA offers less variance and the maximum distinction with pathologic eyes. PURPOSE The aim of this study was to assess factors influencing BCEA and the recording method that offer less variability and thus maximum distinction between healthy and pathologic eyes. METHODS A total of 136 eyes were retrospectively reviewed, 85 eyes without ophthalmic disorders (logMAR acuity ≦0.0) and 51 eyes with late age-related macular degeneration (AMD) were enrolled. All patients underwent two consecutive examinations, a 30-second isolated fixation (static BCEA) and a microperimetric test with continuous fixation recording (dynamic BCEA). All the examinations were carried out using MP1 microperimeter (NAVIS software version 1.7.6; Nidek Technologies, Padova, Italy). RESULTS Dynamic BCEA was significantly correlated with age (r = 0.38, P < .001), total (r = 0.32, P = .03), and tracking time (r = 0.33, P = .02) in controls, whereas no significant relationships were found in the AMD group. The greatest difference between static and dynamic BCEA was observed in 70- to 79-year decade in healthy subjects (P < .01). Logistic regression analysis showed that late AMD status was significantly predicted by ±2 SD and ±3 SD static BCEA (both P = .03). CONCLUSIONS Dynamic BCEA is influenced by a certain degree of variability in advanced-age healthy subjects. In such cases, the use of ±2 SD and ±3 SD static BCEAs seems to offer a more accurate detection of fixation stability changes in the AMD group with respect to normal subjects.

Microperimetric biofeedback training (MBFT) is a visual rehabilitative strategy based on fixation stability improvement reinforcing or creating a new preferential fixation locus. The rationale consists in reeducating visual system to a new visual condition, promoting retina-brain transmission, and thus cortical plasticity. The use of MBFT found is major application in visual diseases involving central vision, but later it revealed promising functional outcomes even in myopia, inherited retinal degenerations and nystagmus. However, the use of microperimetric biofeedback is still limited due to poor knowledge of the procedure and inconsistent standards of practice, and thus an incipient skepticism on its efficacy. This review provides an overview of the rationale, current implications, procedures and future perspectives of microperimetric biofeedback training.

Purpose To assess foveal microstructural changes influencing retinal sensitivity (RS) and fixation stability using microperimeter MP-1 in intermediate age-related macular degeneration (AMD). Methods In this cross-sectional study, 22 eyes of 22 patients (mean age: 75 ± 9.02 years) with intermediate AMD were enrolled. Retinal sensitivity and bivariate contour ellipse area (BCEA) were obtained by microperimetry MP-1 (Humphrey 10-2 68-loci grid) under mesopic conditions. Drusen type, drusenoid pigment epithelial detachment, hyperreflective foci (HF), integrity of external limiting membrane (ELM), inner ellipsoid zone (ISel), RPE/Bruch’s membrane complex (RPE/B) and subfoveal choroidal thickness were analyzed in the foveal region and compared with RS and BCEA. Spearman’s rank correlation coefficient was used to evaluate the relationship between variables. Logistic regression analysis was also used to assess morphological predictor influencing RS or BCEA. Results RS was strongly and inversely related with the presence of HF (r = −0.66, P = 0.001), integrity of ELM (r = −0.70, P

To evaluate visual acuity and contrast sensitivity after bilateral implantation of AcrySof® IQ ReSTOR® +2.50 D or +3.00 D.A total of 56 eyes with cataract had bilateral implantation of a multifocal IOL. Inclusion criteria were corneal astigmatism less than 1.0 diopters, axial length between 22.0 and 24.0 mm, and no associated ocular disease. Outcome measures recorded 12 months postoperatively were binocular uncorrected and corrected distance, intermediate and near visual acuities, and contrast sensitivity. Postoperative quality of life and patient satisfaction were assessed with the VF-14 Questionnaire.Twenty months postoperatively, the mean uncorrected and best-corrected distance visual acuities, UCIVA at 60 cm, and contrast sensitivity were similar in all two groups. UCNVA were better in the +3.00 IOL group than in the +2.50 D group. Patients reported good performance for visual tasks.Apodized multifocal IOLs seem a good compromise for non-spectacles life in elderly patients; our study indicates that this solution has a good long-term stability on patients' visual function.

Purpose To analyze the evolution of type 1 neovascularization associated with vascularized serous pigment epithelial detachment (vsPED) using three-dimensional, volumetric, en face optical coherence tomography angiography (OCTA). Methods This was a retrospective case series from four tertiary medical centers. OCTA images were analyzed at baseline and at the 3-, 6-, 12-, 18-, and 24-month follow-up visit when available. Visual acuity, number of injections, PED maximal height and PED area and volume, and choroidal neovascularization (CNV) flow area and progression were determined at each visit. Qualitative and quantitative analysis of CNV progression (including CNV/PED flow area) and final PED morphology was performed to determine anatomic outcomes. Results Twenty-four eyes in 22 patients were studied. Median follow-up was 20 months. Across all eyes, maximum PED height decreased from 395.5 to 369.5 μm while CNV/PED flow ratio increased from 27.3% to 40.2%. Median visual acuity was unchanged at 20/40. Final PED outcomes included filled fibrovascular versus persistent vsPED. Filled vsPEDs decreased in PED height and volume and displayed a multilayered morphology in contrast to persistent vsPEDs. Fibrovascular PEDs received on average seven less injections as compared to persistent vsPEDs. Conclusions Three-dimensional, volumetric, en face OCTA analysis of vsPED progression illustrated two anatomic outcomes: filled, typically multilayered fibrovascular PED versus persistent vsPED. The filled multilayered PED displayed a reduction in PED height and volume, greater CNV/PED flow ratio, and fewer anti-VEGF injections versus the persistent vsPED and may represent a more stable anatomic outcome while the persistent vsPED may indicate a more unstable morphology.

Purpose. To evaluate possible speeding up recovery time after retinal detachment (RD) surgery using biofeedback strategy.Methods. A total of 52 eyes were selected. After surgery, patients were divided into two groups: group A, including patients submitted to biofeedback with MP-1 strategy; group B, patients who received common care strategy. Biofeedback strategy was started 15 days after the suspension of cycloplegic eye drops in buckling procedure or after silicone oil removal in the vitrectomized eyes. Controls were scheduled at baseline and 6, 12, and 18 weeks.Results. At baseline, there was no significant difference in BCVA between groups (P=0.4230). At the end of biofeedback treatment (WK 6) BCVA of group A was significantly better (P<0.001) than group B and BCVA was still better in group A than group B at WK 12 (P=0.028) and at WK 18 (P=0.041).Conclusions. Visual recovery after RD surgery is still unclear, and it does not depend on entity of the RD. Our data demonstrate that in biofeedback group there was a significant recovery in visual performances that still remains evident after 3 months from the baseline.

Purpose To compare mean choroidal thickness in patients with adult-onset foveomacular vitelliform dystrophy (AOFVD) and healthy subjects, to analyze patients with AOFVD in order to evaluate choroidal thickness disease-related changes, also in relation to the different stages of AOFVD disease and to the retinal pigment epithelium (RPE) features, and to correlate mean choroidal thickness with age in both groups. Methods In this prospective observational cross-sectional study, a total of 63 eyes of 51 consecutive subjects were examined, consisting of a control group (n = 28 eyes) and the AOFVD group (n = 35 eyes). A complete ophthalmologic examination, fundus autofluorescence, and spectral-domain optical coherence tomography were performed in all patients. Results Mean subfoveal choroidal thickness was 214.78 ± 62.35 µm in healthy subjects and 222.31 ± 73.29 µm in the AOFVD group (p = 0.33). In the vitelliruptive group, the mean choroidal thickness was significantly thicker than in the control group at each choroidal location. Mean choroidal thickness was significantly increased in the pseudohypopyon stage when compared to the vitelliform one (+66.34 µm, p = 0.02). Eyes with subretinal fluid (SRF) showed significantly thicker choroid when compared with those without SRF. No significant correlations were found between age and choroidal thickness in the study group. Conclusions The study of the choroid in patients with AOFVD suggested a possible role in the pathologic changes during the different stages of disease, and could help us to evaluate progression of the disease. Greater choroidal thickness associated with SRF and RPE bumps are signs of RPE alterations and could be related to evolution of the AOFVD lesion to a different stage.

Ebola virus disease (EVD—formerly known as Ebola hemorrhagic fever) is a severe hemorrhagic fever caused by lipid-enveloped, nonsegmented, negative-stranded RNA viruses belonging to the genusEbolavirus. Case fatality rates may reach up to 76% of infected individuals, making this infection a deadly health problem in the sub-Saharan population. At the moment, there are still no indications on ophthalmological clinical signs and security suggestions for healthcare professionals (doctors and nurses or cooperative persons). This paper provides a short but complete guide to reduce infection risks.

Background: To evaluate retinal function and fixation stability in major depressive disorder (MDD) and the influence of antidepressant therapy, using the MP-1 microperimeter. Methods: 25 patients with MDD (57 ± 13.73 years) and 25 healthy subjects (HS) (56.41 ± 15.73 years) were enrolled. According to the Diagnostic and Statistical Manual of Mental Disorders 4th edition (DSM-IV), single (296.2) or recurrent episode (296.3) of MDD was included. Retinal sensitivity, fixation stability, and bivariate contour ellipse area (BCEA) were obtained using MP-1. Then patients were divided according to antidepressant therapy: SSRI/SNRI associated with atypical antipsychotic (group A) and SSRI/SNRI monotherapy (group B) and pharmacological washout (group C). Differences between groups were estimated using the Kruskal-Wallis test with post hoc Mann-Whitney analysis. Spearman’s rank correlation was used for comparisons. Results: Mean retinal sensitivity was significantly reduced in the MDD group than HS (P= 0.02). Six (24%) eyes had a relatively unstable fixation, and mean BCEA was larger than control group (P =0.04). The Kruskal-Wallis analysis of variance showed significant differences in log BCEA between groups (P=0.01), but no differences in mean retinal sensitivity between groups (P=0.09). Conclusion: MDD patients showed impairment in retinal sensitivity and fixation stability respect to the HS. Despite these findings, the impairment of fixation stability seems to be related to antidepressant therapy.

Introduction: To compare mean best-corrected visual acuity (BCVA), retinal sensitivity (RS), and bivariate contour ellipse area (BCEA) in patients with adult-onset foveomacular vitelliform dystrophy (AOFVD) and healthy subjects (HSs), reporting also functional disease-related changes in the different stages of the AOFVD disease. Materials and Methods: In this observational cross-sectional study, a total of 19 patients (30 eyes; 12 female and 7 male) with AOFVD were enrolled, and 30 patients (30 eyes; 16 female and 14 male) were recruited as age-matched control group (74.36 ± 9.17 years vs. 71.83 ± 6.99 years respectively, P = 0.11). All patients underwent a complete ophthalmologic examination, fundus autofluorescence and fluorescein angiography, spectral-domain optical coherence tomography and microperimetry (MP)-1 analysis. The data collection included mean BCVA, mean RS measured by means of MP-1, BCEA, and central retinal thickness. Results: All the functional parameters (BCVA, RS, and BCEA) were significantly worse in AOFVD group than HS. Subgroup analysis showed that the most significant functional changes, quantified by mean BCVA, RS, and BCEA, were in the atrophic stage (P = 0.03, P = 0.01, and P = 0.001, respectively). All the functional parameters were well correlated in the different stages. Conclusions: This study further confirms the good visual prognosis in the AOFVD eyes. Fixation stability measurement using BCEA demonstrates good evaluation of visual performance integrating traditional functional parameters. It may also serve for further rehabilitative purposes in atrophic eyes.

Background: To evaluate visual recovery after rehabilitation with microperimetric biofeedback in patients submitted to surgery for retinal detachment. Methods: We have randomly divided 44 eyes of 44 patients after surgery for retinal detachment into two group: group A, 23 eyes, submitted to biofeedback training with microperimetry MP-1, 10 training sessions, once a week, ten minutes for each eye; group B (control group), 21 eyes, treated with common care strategy. We have compared best correct visual acuity (BCVA) of two groups at baseline, 6, 12 and 18 weeks with Student’s t test. Results: At baseline the mean BCVA was 0.6 ± 0.43 logMAR in group A and 0.66 ± 0.67 logMAR in group B (p=0.74). At 6 weeks after training the mean BCVA of group A was 0.27 ± 0.29 logMAR significantly better (p=0.02) than group B (0.67 ± 0.67 logMAR). At 12 weeks the mean BCVA was 0.18 ± 0.25 logMAR in group A better than the control group in which the mean BCVA was 0.60 ± 0.66 logMAR (p=0.01). At 18 weeks visual performances were still better in biofeedback group than in group B (p=0.01) in which the mean BCVA was 0.58 ± 0.68 logMAR. Conclusions: Microperimetric biofeedback allowed a better and faster visual recovery after surgery for retinal detachment than normal condition.

Cystoid macular edema (CME) is a condition that involves the macula, causing painless vision loss. In this paper, we report a case of niacin-induced bilateral cystoid macular edema (CME) in a middle-age woman taking low dose of niacin (18 mg of nicotinic acid). Optical coherence tomography (OCT) showed retinal thickening and cystoid spaces in both eyes, whereas fluorescein angiography (FA; HRA 2, Heidelberg Engineering) revealed the absence of fluorescein leakage also in later phases. Four weeks after discontinuation of therapy there were a complete disappearance of macular edema at funduscopic examination and an improvement of visual acuity in both eyes. Furthermore OCT showed a normal retinal profile in both eyes. In our opinion considering the wide availability of niacin, medical monitoring and periodical examination should be considered during niacin administration. To our knowledge, this is the first report in the literature that described the very low-dose niacin-induced bilateral niacin maculopathy.

Objective To evaluate which of 2 measuring units (bivariate contour ellipse area [BCEA] vs Fujii) yields more accurate measurements of fixation stability, obtained using the MP-1 device, in patients with neovascular age-related macular degeneration (nAMD) treated with intravitreal injections of ranibizumab, during a 12-month follow-up period. Design Small retrospective, noncomparative, interventional case series. Participants A total of 25 eyes in 25 patients (13 males, 12 females; mean age 71.72 ± 7.98 years). Methods All participants were older than 50 years, diagnosed with active subfoveal choroidal neovascularization, had best corrected visual acuity (BCVA) values above 20/100, and all lesion types were included. All patients underwent a loading phase with 3 consecutive intravitreal injections of 0.05 mg ranibizumab at monthly intervals. Patients were retreated after the third injection if they exhibited a 100-μm increase in macular thickness or evidence of intraretinal and/or subretinal fluid and new subretinal hemorrhage, observed with spectral-domain optical coherence tomography and fluorescein angiography. The data collected included BCVA and mean macular sensitivities, BCEA, and fixation patterns, performed at baseline and at months 4 and 12, using the MP-1 device. Results The mean total injection number was 5.92 ± 1.18 (minimum 3, maximum 8). Mean BCVA at baseline was 0.55 ± 0.28 logMAR and increased significantly to 0.50 ± 0.33 logMAR. Mean macular sensitivity at baseline was 7.06 ± 4.59 dB and increased significantly to 8.40 ± 4.82. Mean BCEA was 2.19 ± 1.38 deg 2 and decreased significantly to 1.68 ± 1.43 deg 2 . Fixation stability patterns, according to the protocol set out by Fujii, did not change significantly during follow-up. Conclusions Compared with Fujii fixation stability patterns, BCEA correlated better with variations in macular sensitivity and BCVA. BCEA can be added to the traditional parameters used to evaluate the efficacy of intravitreal injections in patients with nAMD.

Toxocara canisis a nematode parasite, commonly found in dogs. This roundworm parasite can invade the eye, causing visual impairment.Toxocarashould be considered as a possible causative agent of posterior and diffuse uveitis, and it could be considered in the differential diagnosis of retinoblastoma. Ocular manifestations vary from severe endophthalmitis to silent incidental findings on a routine examination. We report a case of ocular toxocariasis in a 24-year-old Asiatic female that presented to us complaining of visual impairment. Fundoscopic examination revealed a posterior pole granuloma and exudative retinal detachment along with exudates. Presentation, clinical findings, morphological changes, and treatment are discussed. The enzyme-linked immunosorbent assay serology forToxocara caniswas performed, demonstrating the positivity for IgG and IgE. Treatment with the antihelminthic albendazole was initiated. Fluorescein angiography (FA; HRA 2, Heidelberg engineering) and optical coherence tomography (OCT; Spectralis, Heidelberg tomography) were performed, and results have been reported.

Aims: The aim was to evaluate circulating levels of reactive oxygen species (ROS) and changes in central macular thickness (CMT) in patients with nonproliferative diabetic retinopathy (NPDR) after antioxidant supplementation. Materials and Methods: A total of 68 patients (68 eyes) with NPDR were enrolled. Patients were randomly divided into two groups: Treated with antioxidant supplement (Group A) and untreated control group (Group B). Each tablet, for oral administration, containing pycnogenol 50 mg, Vitamin E 30 mg and coenzyme Q10 20 mg. CMT and free oxygen radical test (FORT) were analyzed at baseline (T0), 3 (T1) and 6 (T2) months in both groups. Results: In Group A, FORT levels and CMT were significantly reduced over time (P < 0.001 for both). In Group B, FORT levels were increased (P < 0.001) and CMT did not vary significantly (P = 0.81) over 3 time points. Conclusions: This is the first study showing the reduction of ROS levels in patients with NPDR thanks to antioxidant therapy. Moreover, our findings have suggested also an influence on retinal thickness.