CLINICAL CHARACTERISTICS AND MULTIMODAL IMAGING FINDINGS IN UNILATERAL FROSTED BRANCH ANGIITIS: A CASE REPORT.

PURPOSE: Our aim is to report a comprehensive multimodal imaging case of unilateral frosted branch angiitis in a 40-years-old Caucasian female . METHODS: Case report involving clinical examination, ultra- wide field fundus photograph, ultra-wide field fluorescein angiography (UWFA), optical coherence tomography (OCT) and optical coherence tomography angiography (OCTA). RESULTS: A 40 years old patients presented with unilateral acute vision loss. On fundus examination, extensive retinal veins sheathing, macular edema and vascular congestion were observed while UWFA revealed an hyperfluorescent "hot" optic disc and blood retinal barrier disruption. OCTA displayed foveal avascular zone (FAZ) enlargement and excluded papillary neovascularization. Extensive laboratory work-up for infectious, autoimmune and inflammatory disorders were negative, thus, a diagnose of acute idiopathic unilateral frosted branch angiitis was made. Intravitreal injection of dexamethasone implant was administered with a good clinical response. CONCLUSIONS: Multimodal imaging is crucial to correctly diagnose and treat FBA. Up to our knowledge, the use of OCTA as a complementary tool to the diagnostic process in FBA has been described in literature just once as a photo essay of cytomegalovirus-related FBA and it might be of great value for better characterizing clinical features of this disorder and for following disease activity in a non-invasive fashion.

Multimodal imaging in Schubert-Bornschein congenital stationary night blindness.

BACKGROUND: Schubert-Bornschein (SB) is the most common type of people with congenital stationary night blindness (CSNB). The aim of the study is to describe the optical coherence tomography (OCT) and optical coherence tomography angiography (OCTA) findings in patients with SB CSNB. METHODS: Prospective, observational case series including three patients with genetically confirmed CSNB along with matched controls, who underwent complete ophthalmic examination and multimodal imaging. RESULTS: On SD-OCT, a significant focal outer plexiform layer (OPL) thickening and a corresponding focal outer nuclear layer (ONL) thinning were identified in the macular area (p < 0.001). OCTA analysis overall showed decreased density of macular deep capillary plexus (mDCP) and macular choriocapillaris (mCC) (p = 0.008 and p = 0.033, respectively). DCP vessel density in the area corresponding to OPL thickening was significantly increased compared to the remaining retina (p < 0.001). CONCLUSION: SB CSNB is characterized by retinal vascular impairment, as detected on OCTA.

IMMUNOGLOBULIN G4-RELATED OPHTHALMIC DISEASE MIMICKING INTRAOCULAR LYMPHOMA: A CASE REPORT.

PURPOSE: To describe a case of immunoglobulin G4-related choroiditis mimicking intraocular lymphoma. METHODS: The patient underwent a complete ophthalmological evaluation including multimodal imaging, with structural optical coherence tomography, fluorescein angiography, indocyanine green angiography, ultra-widefield color, and autofluorescent fundus photographies to assess the ocular involvement. RESULTS: Patient's best-corrected visual acuity was of 20/25 in the right eye and 20/20 in the left eye. Fundus appearance showed abnormal yellowish choroidal lesions and moderate vitritis in both eyes. Fluorescein angiography was within normal limits, whereas indocyanine green angiography showed areas of choroiditis in both eyes, and structural optical coherence tomography scans disclosed retinal small roundish lesions in the corresponding regions. Laboratory examinations and lymph node biopsy led to the final diagnosis of immunoglobulin G4-related disease. CONCLUSION: We describe a case of immunoglobulin G4-related choroiditis mimicking intraocular lymphoma. The proper use of multimodal imaging associated with laboratory investigations was helpful to reach the correct diagnosis.

Bilateral acute retinal necrosis during treatment with alemtuzumab for multiple sclerosis.

INTRODUCTION: Alemtuzumab is a humanized monoclonal antibody used as a treatment of multiple sclerosis (MS) and chronic lymphocytic leukemia. It decreases T cell count leading to significant immunosuppression, with increased risk of systemic and ocular infections. Herein, we report a unique case of bilateral acute retinal necrosis (ARN) caused by varicella-zoster virus (VZV) in a patient affected by MS under treatment with alemtuzumab. CASE DESCRIPTION: A 36-year-old man with a relapsing-remitting MS under treatment with alemtuzumab developed bilateral visual loss. Anterior segment examination displayed granulomatous keratic precipitates and 3+ cells in the anterior chamber, while fundoscopy showed bilateral 1+ vitritis and peripheral retinal necrosis, complicated by retinal detachment in the left eye. The high viral load for VZV in aqueous humor samples had a univocal interpretation for viral reactivation. In addition to systemic therapy with acyclovir, the patient was treated with bilateral intravitreal injections of foscarnet and underwent pars-plana vitrectomy and silicone oil tamponade for retinal detachment in the left eye. CONCLUSION: This report shows a unique case of bilateral ARN caused by VZV associated with alemtuzumab. Any visual loss in MS patients under biologic therapy should not be underestimated, performing an accurate differential diagnosis with optic neuritis.

The importance of clinical presentation on long-term outcomes of external dacryocystorhinostomies: Our experience on 245 cases.

PURPOSE: External dacryocystorhinostomy (EXT-DCR) is a surgical approach commonly used to treat post-canalicular acquired lacrimal obstruction whose success rate has been described, equally with endoscopic DCR, to be superior to other available treatments. METHODS: At San Raffaele Hospital, Milan (Italy), from January 2008 to December 2020, 245 EXT-DCRs were performed. All patients underwent routine pre-operative work-up including Jones tests and probing and irrigation of the lacrimal pathway; when necessary, a multidisciplinary approach with nasal endoscopy was performed. The surgical approach was followed by positioning of a bicanalicular stent which was left in place for 6 months. Success was defined as resolution of clinical signs/symptoms and free lacrimal flow on functional test. RESULTS: Of the cases enrolled, 26.9% were treated for recurrent epiphora (group 1), and 73.1% for epiphora associated with chronic dacryocystitis (group 2). After a median follow-up of 71 months, group 1 had success at T0 (1 month) and T1 (long-term) of 81.8% and 60.6%, respectively, compared to 93.8% and 77.7% in group 2. Statistical analysis showed a significant association with surgery both at T0 (p = 0.018) and T1 (p = 0.012) with group 2 showing better outcomes. Additionally, the cosmetic outcome of the external scar was defined as invisible in 91.8% of cases and slightly visible in 8.2%. CONCLUSIONS: External dacryocystorhinostomy provides long-term reliable results particularly in case of chronic dacryocystitis. Moreover, the optimal esthetic outcome of the external scar should be no longer considered the only guiding principle of treatment modality, particularly in the elderly.

Morphological and Functional Relationship Between OCTA and FA/ICGA Quantitative Features in AMD-Related Macular Neovascularization.

Background: The aim was to study the relationship between quantitative information provided by optical coherence tomography (OCT) angiography (OCTA) and conventional angiography in macular neovascularization (MNV) secondary to age-related macular degeneration (AMD). Methods: The research was designed as an interventional, prospective study. We included 66 eyes (66 patients) affected by naïve MNV. Multimodal imaging included structural OCT, OCTA, fluorescein angiography (FA), and indocyanine green angiography (ICGA). The follow-up lasted 1 year. Patients were treated by PRN anti-VEGF injections. Based on FA/ICGA examinations, we divided the patients into two categories: low vessel tortuosity (VT) (<8.40) and high VT (>8.40), correlating VT with the MNV area, leakage area, speckled fluorescence (SF) quadrants and MNV area/leakage area ratio. Results: Mean baseline BCVA was 0.50 ± 0.61 LogMAR, improved to 0.31 ± 0.29 LogMAR after 1 year (p < 0.01), with a mean number of 7 ± 2 anti-VEGF injections. The patients revealed type-1 MNV in 36 eyes (55%), mixed type 1 and 2 MNV in 18 eyes (27%), and type-2 MNV in 12 eyes (18%). MNV eyes in high-VT MNV featured poorer BCVA, CMT, and OCTA parameters, higher SF quadrants, and less exudation, compared with low-VT MNV (p < 0.01). Moreover, 30% of high-VT MNV eyes developed outer retinal atrophy. Conclusions: Low VT MNV turned out to be more exudative at the baseline but less damaging to the outer retinal structures, whereas high VT MNV proved to be less exudative but more prone to lead to atrophic changes and visual function deterioration. VT may be usefully applied to artificial intelligence-based models designed to characterize MNV secondary to AMD.

Acute Central Serous Chorioretinopathy Subtypes as Assessed by Multimodal Imaging.

Purpose: To differentiate acute central serous chorioretinopathy (CSC) subtypes by multimodal imaging. Methods: The research was designed as a prospective, interventional study. Naive patients with acute CSC were followed for 24 months. Overall, 96 CSC patients (96 eyes) and 210 controls (210 eyes) were included. Multimodal imaging allowed the study to classify CSC into retinal pigment epithelium-related CSC (RPE-CSC) and choroidal-related CSC (choroidal-CSC) subtypes. The RPE-CSC type was characterized by normal choroidal thickness (CT) in association with disseminated RPE alterations. The choroidal-CSC type was distinguished by identifying a pachychoroid. All the patients underwent eplerenone or verteporfin photodynamic therapy (PDT). Patients developing macular neovascularization (MNV) underwent anti-VEGF injections. Quantitative measurements included central macular thickness (CMT), choroidal thickness (CT), Sattler layer thickness (SLT) and Haller layer thickness (HLT). Results: Considering the CSC patients as a whole, baseline BCVA was 0.18 ± 0.25 LogMAR, increasing to 0.13 ± 0.21 LogMAR after 24 months (P < 0.01), whereas baseline CMT improved from 337 ± 126 µm to 244 ± 84 µm after 24 months (P < 0.01). We found the following subdivision of CSC eyes: RPE-CSC type (45%) and choroidal-CSC type (55%). Overall, MNV were detected in 18 eyes (19%), 13 eyes (72%) in the RPE-CSC subgroup and five eyes (28%) in the choroidal-CSC subgroup. Forty eyes responded to eplerenone (57% of RPE-CSC and 47% of choroidal-CSC), whereas 38 eyes required PDT (43% of RPE-CSC and 53% of choroidal-CSC). Conclusions: Acute CSC includes two main clinical manifestations, displaying differing features concerning retinal and choroidal involvement. Translational Relevance: This study identified two clinically different acute CSC subtypes on the basis of quantitative pachychoroid cutoff values.

Structural OCT Parameters Associated with Treatment Response and Macular Neovascularization Onset in Central Serous Chorioretinopathy.

INTRODUCTION: This study aimed to assess quantitative factors associated with treatment response and macular neovascularization (MNV) onset in central serous chorioretinopathy (CSC) through an artificial intelligence-based approach. METHODS: The study was designed as an interventional, prospective case series with a planned follow-up of 36 months. We included only eyes demonstrating the first episode of CSC. All the patients underwent eplerenone or photodynamic therapy (PDT) treatment. Eyes developing MNV underwent anti-VEGF injections. We developed an artificial intelligence-based model to assess predictive quantitative structural optical coherence tomography (OCT) factors related to treatment response and onset of MNV. Main outcome measures were best-correct visual acuity (BCVA), central macular thickness (CMT), retinal thickness (RT), retinal pigment epithelium (RPE) thickness, choroidal thickness, Sattler's layer thickness (SLT), Haller's layer thickness, retinal and choroidal hyperreflective foci (HF), and MNV. RESULTS: We included 96 naïve CSC eyes (96 patients). Baseline BCVA was 0.18 ± 0.25 logMAR, which increased to 0.16 ± 0.27 logMAR after 3 years (p > 0.05). Baseline CMT was 337 ± 126 µm, which improved to 229 ± 40 µm after 3 years (p < 0.01). We observed good response to eplerenone in 40/78 (51%) eyes, whereas 38/78 (49%) eyes underwent PDT. The artificial intelligence model showed choroidal HF and age as determining factors of good response to eplerenone or PDT. RPE thickness < 36 µm, RT < 300 µm, and SLT < 50 µm increased probability of 50% of having MNV. CONCLUSIONS: CSC response to eplerenone or PDT is influenced by choroidal HF and patient age. RPE and SLT represent relevant factors for onset of MNV.

Macular neovascularization in AMD, CSC and best vitelliform macular dystrophy: quantitative OCTA detects distinct clinical entities.

BACKGROUND: To perform a quantitative optical coherence tomography (OCT) angiography (OCTA) analysis of macular neovascularization (MNV) secondary to age-related macular degeneration (AMD), central serous chorioretinopathy (CSC) and best vitelliform macular dystrophy (BVMD), with the aim of highlighting quantitative features indicating different clinical entities. METHODS: Study design: prospective, interventional. We recruited patients affected by AMD, CSC or BVMD, complicated by naive MNV. All patients underwent complete ophthalmologic examination and multimodal imaging. They were treated with anti-VEGF injections, following a pro-re-nata regimen. The ensuing follow-up lasted 1 year. Quantitative dye-based angiography, OCT, and OCTA parameters were analysed to obtain cutoff values able to distinguish two clinically different patient subgroups for each retinal disease. The main outcome measures were best-corrected visual acuity (BCVA), central macular thickness, vessel density of superficial, deep and choriocapillaris plexa, vessel tortuosity (VT) of MNV, vessel dispersion of MNV, number of injections, MNV/leakage ratio, MNV size, speckled fluorescence, and outer retinal atrophy. RESULTS: Ninety-eight eyes affected by MNV (98 patients) were analysed. These included 66 eyes affected by AMD, 18 displaying CSC, and 14 eyes with BVMD. BCVA was alike in the three groups, both at baseline and after 1 year (p > 0.05). An MNV VT cutoff of 8.40 at baseline detected two patient subgroups differing significantly in terms of morpho-functional features, found both at baseline and at the end of the follow-up. CONCLUSIONS: Quantitative OCTA suggested that the MNV's VT might be able to provide a better characterization of two different morpho-functional manifestations in AMD, CSC and BVMD.

QUANTITATIVE OPTICAL COHERENCE TOMOGRAPHY ANGIOGRAPHY PARAMETER VARIATIONS AFTER TREATMENT OF MACULAR NEOVASCULARIZATION SECONDARY TO AGE-RELATED MACULAR DEGENERATION.

PURPOSE: Macular neovascularization (MNV) secondary to age-related macular degeneration can be characterized by quantitative optical coherence tomography angiography. The aim of the study was to assess the evolution of quantitative optical coherence tomography angiography parameters after 1 year of antivascular endothelial growth factor injections. METHODS: Naive age-related macular degeneration-related MNV eyes were prospectively recruited to analyze optical coherence tomography and optical coherence tomography angiography parameters, including MNV vessel tortuosity (VT) and reflectivity, at baseline and at the end of the follow-up. Macular neovascularization eyes were categorized by a MNV VT cutoff, and quantitative parameter variations were documented after 1 year of treatment. We divided MNV eyes into Group 1 (MNV VT < 8.40) and Group 2 (MNV VT > 8.40). RESULTS: Thrity naive age-related macular degeneration-related MNV eyes (30 patients) were included. Our cohort included 18 Type 1 MNV and 12 Type 2 MNV lesions. Baseline central macular thickness (411 ± 85 µm) improved to 323 ± 54 µm at 1 year (P < 0.01). Only Group 1 MNV displayed significant visual improvement. Macular neovascularization VT values remained stable over the follow-up in both subgroups. Group 2 MNV eyes showed increased MNV reflectivity and increased MNV area at the end of the follow-up. Quantitative retinal capillary plexa parameters were found to be worse in Group 2 MNV. Outer retinal atrophy occurred in 2 of the 18 eyes in MNV Group 1 (11%) and in 6 of the 12 eyes in MNV Group 2 (50%) after 1 year. Vessel density proved to be always worse in Group 2 than in Group 1. CONCLUSION: Macular neovascularization VT provides information on the blood flow and identifies two subgroups with different final anatomical and visual outcomes, regardless of the treatment effect.

Combination of Mustardè cheek advancement flap and paramedian forehead flap as a reconstructive option in orbital exenteration.

PURPOSE: To describe a combinatory technique made of Mustardè cheek advancement flap and paramedian forehead flap as a reconstructive option after orbital exenteration. METHODS: We retrospectively reviewed all patients who underwent orbital exenteration and subsequent reconstruction with this technique at our Head and Neck Department, Divisions of Ophthalmology and Otolaryngology, at San Raffaele Hospital, Milan, Italy. RESULTS: Three patients were treated with the aforementioned technique, following orbital exenteration due to malignancies. All of them were affected by recurrent diseases arising from the ocular components or periorbital structures: one basal cell carcinoma and two squamous cell carcinomas. Excellent result was achieved considering skin texture and colour match, aesthetic results and intra and post-operative complications (only minor, surgically controlled haemorrhage and minimal dehiscence subsequent to defective healing occurred). CONCLUSION: The proposed technique can be considered by the head and neck reconstructive surgeon as a good option in extended orbital exenteration.

Ophthalmic Shingles with Simultaneous Acute Retinal Necrosis in the Opposite Eye.

Purpose: To report a case of acute retinal necrosis (ARN) occurring in the opposite eye to the ophthalmic shingles eruption.Design: Case report.Methods: Clinical examination and multimodal imaging review, including ultra-widefield photography and fluorescein angiography (FA).Results: A 49-years-old man attended the Uveitis Service for concurrent acute visual loss in the right eye and ophthalmic shingles skin eruption of the left ophthalmic trigeminal branch. Fundus examination of the right eye revealed multiple yellowish patchy areas of retinitis in the peripheral retina consistent with ARN. Multimodal imaging and laboratory tests confirmed the diagnosis.Discussion: Herpes Zoster Ophtalmicus (HZO) represents the 10-20% of Herpes Zoster (HZ) cases and ARN is a rare but sight-threatening complication due to the viral widespread along the retina. By definition, the reactivation of Varicella Zoster virus (VZV) has a unilateral clinical expression, due to the centrifugal neural pathway followed by the virus from the neural ganglia, and HZO, as well, results located on the same side of the skin affection. The case presented contradicted this postulate.Conclusion: prompt diagnosis, through fundus examination and FA, and proper antiviral therapy were the mainstays to counteract the posterior uveitis. Nowadays, vaccination is a safe weapon to efficaciously reduce the ZVZ incidence among general population.

Photoreceptor alteration in intermediate age-related macular degeneration.

The aim of this study was to analyze photoreceptor alterations occurring in eyes with intermediate age-related macular degeneration (AMD) and to investigate their associations with choriocapillaris (CC) flow. In this retrospective case-control study, we collected data from 35 eyes with intermediate AMD from 35 patients who had swept source optical coherence tomography structural and angiography imaging obtained. A control group of 35 eyes from 35 healthy subjects was included for comparison. Our main outcome measure for comparison between groups was the normalized reflectivity of en face image segmented at the ellipsoid zone (EZ) level, which was calculated to quantify the photoreceptor damage. OCTA metrics to quantify CC flow signal were also computed. These metrics were measured in a circle centered on the fovea and with a diameter of 5 mm. In intermediate AMD eyes, the macular area occupied by drusen was identified. Therefore, the EZ reflectivity and CC flow signal were separately measured in regions without drusen ("drusen-free" region). Measurements were generated using previously published algorithms. Mean ± SD age was 74.1 ± 6.8 years in the intermediate AMD group and 72.1 ± 6.0 years in the control group (p = 0.206). The normalized EZ reflectivity was 0.76 ± 0.10 in the intermediate AMD group and 0.85 ± 0.08 in the control group (p < .0001). In the "drusen-free" region, the normalized EZ reflectivity was 0.77 ± 0.10 (p < .0001  vs. healthy controls) and was positively correlated with the CC flow signal density (ρ = - 0.340 and p = 0.020). In conclusion, eyes with intermediate AMD exhibit a diffuse reduced EZ normalized reflectivity, and this reduction is correlated with CC flow signal in the regions without drusen. This study supports the concept of the damage of the unit comprised of photoreceptor, CC, and intervening tissues as an early event in AMD.

Vitreomacular traction quantitative cutoffs for the assessment of resolution after ocriplasmin intravitreal treatment.

This study aimed to assess optical coherence tomography (OCT) parameters associated with vitreomacular traction (VMT) resolution after ocriplasmin intravitreal injection and also associated with the development of vitreomacular complications. Study designed was a retrospective case series. Structural OCT images were acquired at baseline and over the follow-up after treatment. We developed a mathematical model to provide quantitative parameters associated with VMT resolution. Moreover, we adopted the same model to assess the quantitative parameters associated with development of further vitreomacular complications or with the worsening of the coexisting condition. Main outcome measures were BCVA, central macular thickness (CMT), VMT reflectivity, VMT size, VMT resolution, epiretinal membrane (ERM), macular holes. 73 eyes of 73 VMT patients (mean age 73 ± 9 years) were recruited. The mean follow-up duration was 2.6 ± 1.1 years. Mean baseline BCVA was 0.38 ± 0.18 LogMAR, improving to 0.26 ± 0.20 at the end of the follow-up (p < 0.01). Baseline CMT was 431 ± 118 µm, improving to 393 ± 122 µm at the end of the follow-up (p < 0.01). 38/73 eyes (52%) showed only VMT, whereas 35/73 eyes (48%) also showed coexisting alterations at baseline. VMT resolved in 40/73 eyes (55% of cases). Our model disclosed VMT reflectivity as the most involved parameter in VMT resolution. VMT size showed less influence on the success of ocriplasmin treatment. ERM was negatively associated with VMT resolution. Moreover, VMT reflectivity values and ERM represented the most important parameters for the onset of vitreomacular complications.

Choroidal Patterns in Retinitis Pigmentosa: Correlation with Visual Acuity and Disease Progression.

Purpose: The main aim was to identify different choroidal patterns in retinitis pigmentosa (RP) and to assess their clinical and anatomical meanings after 1 year of follow-up. Methods: Forty-five patients with RP (29 men; mean age 44.5 ± 11.7 years) and 45 healthy controls (29 men; mean age 44.2 ± 9.8 years) were recruited. Optical coherence tomography (OCT) and OCT angiography (OCTA) images were obtained. By means of structural OCT, the following three choroidal patterns were identified: normal-appearing choroid (pattern 1), reduced Haller and Sattler layers (pattern 2), and pattern 2 + choroidal caverns (pattern 3). Main outcome measures were best-corrected visual acuity (BCVA), central macular thickness (CMT), choroidal thickness (CT), vessel density, vessel tortuosity, vessel dispersion, vessel rarefaction, and choroidal stromal index (CSI). Results: Mean BCVA was 0.27 ± 0.30 LogMAR for patients with RP and 0.0 ± 0.0 LogMAR for controls (P < 0.01). CMT, CT, CSI, and OCTA parameters were statistically different between patients with RP and controls (P < 0.01). Choroidal patterns 1, 2, and 3 were identified in 20 (44%), 15 (33%), and 10 (23%) patients with RP, respectively. Several statistically significant correlations were also found. Interestingly, after 1 year of follow-up, only the pattern 3 subgroup showed significant worsening of BCVA, CMT, and OCTA parameters (P < 0.01). Conclusions: Choroidal patterns were associated with different RP clinical forms as well as with different progression after 1 year. Translational Relevance: Choroidal patterns evaluation may provide useful clinical information for patients with RP.

Optical Coherence Tomography Angiography in Extensive Macular Atrophy with Pseudodrusen-Like Appearance.

Purpose: Analyses of quantitative features of optical coherence tomography angiography (OCTA) in patients affected by extensive macular atrophy with pseudodrusen-like appearance (EMAP). Methods: In a prospective case-control study, patients and age- and gender-matched healthy controls underwent complete ophthalmologic examination, including best corrected visual acuity (BCVA) measurement, biomicroscopy, fundus autofluorescence and spectral-domain optical coherence tomography (Spectralis HRA; Heidelberg Engineering GmbH, Heidelberg, Germany), and OCTA scans (DRI OCT Triton; Topcon Corporation, Tokyo, Japan). Vessel density in the superficial capillary plexus and deep capillary plexus (DCP) in the retina and choriocapillaris (CC) in the macula and optic disc were measured. The one-way analysis of variance test with Bonferroni correction was used for statistical assessments. Results: Seven patients (14 eyes) and 10 controls were included in the study. The mean follow-up period was 3 ± 0.8 years. The mean BCVA of patients at baseline was 0.81 ± 0.43 (logarithm of the minimum angle of resolution [LogMAR]) and 1.05 ± 0.38 (LogMAR) at the final follow-up visit (P = 0.006). Quantitative analyses of retinal vessels revealed significant alterations, especially in the DCP and CC, in both atrophic and junctional zones in retina of EMAP patients compared with preserved zones and controls. Conclusions: OCTA analysis characterized three different retinal regions in EMAP disease, corresponding to progressively deeper perfusion defects. Further investigations are warranted to explore the correlation between DCP changes and the extension of atrophy. Translational Relevance: By expanding our pilot study, we may better define EMAP on the basis of vascular changes and eventually recognize earlier the direction of enlargement of atrophy by means of OCTA analyses.

Reviewing the Role of Ultra-Widefield Imaging in Inherited Retinal Dystrophies.

Inherited retinal dystrophies (IRD) are a heterogeneous group of rare chronic disorders caused by genetically determined degeneration of photoreceptors and retinal pigment epithelium cells. Ultra-widefield (UWF) imaging is a useful diagnostic tool for evaluating retinal integrity in IRD, including Stargardt disease, retinitis pigmentosa, cone dystrophies, and Best vitelliform dystrophy. Color or pseudocolor and fundus autofluorescence images obtained with UWF provide previously unavailable information on the retinal periphery, which correlates well with visual field measurement or electroretinogram. Despite unavoidable artifacts of the UWF device, the feasibility of investigations in infants and in patients with poor fixation makes UWF imaging a precious resource in the diagnostic armamentarium for IRD.

Multimodal evaluation of central and peripheral alterations in Stargardt disease: a pilot study.

BACKGROUND: The clinical phenotype of Stargardt disease (STGD) can be extremely heterogeneous, with variable macular and peripheral retinal involvement. The study aim was to correlate peripheral ultrawide field (UWF) involvement with macular alterations, as assessed by structural optical coherence tomography (OCT) and OCT angiography (OCTA), in order to identify potentially different phenotypes. METHODS: The study involved patients with STGD and healthy controls. We performed a complete ophthalmologic assessment and multimodal imaging, including OCT, OCTA, fundus autofluorescence and UWF imaging. Patients with STGD were subdivided according to the peripheral involvement. OCT and OCTA quantitative parameters were analysed. The main outcome of the study was the classification of UWF subtypes and the correlation between UWF subtypes and macular involvement. RESULTS: Seventy STGD eyes (19 male; mean age 41.3±13.2 years) and 70 healthy eyes (35 male; 50%; mean age 41.2±9.8 years) were included in the analyses. Mean best-corrected visual acuity was 0.60±0.45 LogMAR for the STGD group and 0.0±0.0 LogMAR for controls (p<0.01). All clinical and imaging findings proved to be statistically worse in patients with STGD than in the control subjects (p<0.01). UWF types were distributed as follows: type I (49%), type II (34%), type III (17%). Type III patients proved to be significantly worse in terms of visual function and OCT and OCTA imaging parameters. CONCLUSIONS: The UWF autofluorescence performed in the present study suggests that there exist three different STGD phenotypes. Each phenotype is associated with variable OCT and OCTA impairment. Further studies providing a better assessment of the peripheral retinal involvement in STGD are warranted.

Choroidal Patterns in Stargardt Disease: Correlations with Visual Acuity and Disease Progression.

BACKGROUND: To identify different choroidal patterns in Stargardt disease (STGD) and to assess their clinical correlates. METHODS: 100 STGD eyes (29 males; mean age 42.6 ± 16.5 years) and 100 control eyes (29 males; mean age 43.2 ± 8.5 years) were included. Optical coherence tomography (OCT) and OCT angiography (OCTA) images were obtained. Four different choroidal patterns, quantitative OCT and OCTA parameters were assessed and statistically analyzed. The main outcome was the correlation between each choroidal pattern and anatomical and functional retinal status. Furthermore, we assessed structural and best corrected visual acuity (BCVA) changes of each STGD subgroup after one-year. RESULTS: Mean BCVA was 0.63 ± 0.44 LogMAR for STGD patients and 0.0 ± 0.0 LogMAR for controls (p < 0.01). All quantitative parameters appeared deteriorated in STGD compared to controls (p < 0.01). Choroidal patterns were distributed as follows: Pattern 1 (normal appearing choroid) (15%), Pattern 2 (reduced Sattler or Haller layer) (29%), Pattern 3 (reduced Sattler and Haller layers) (26%), Pattern 4 (Pattern 3 + choroidal caverns) (30%). More advanced patterns significantly correlated with a more severe loss of retinal structural integrity. Furthermore, only Pattern 3 and Pattern 4 showed remarkable signs of progression after one year. CONCLUSIONS: Choroidal patterns were related with retinal structural status and BCVA loss, and with different disease progression.