Repeatability of swept-source optical coherence tomography angiography automated macular vessel density.

INTRODUCTION: Optical Coherence Tomography Angiography Ratio Analysis (OCTARA) is capable of visualizing inner and outer retinal vascular plexuses, choriocapillaris, and larger choroidal vasculature in vivo without contrast injection. The aim of this study was to assess the intrasession repeatability of automated vessel density measurements using Triton Swept-Source Optical Coherence Tomography Angiography (OCTA) innovative algorithm OCTARA in retinal and choroidal vasculature. METHODS: To study population between 65-90 years old with no eye diseases. For each subject measurements were performed four times. The intraclass correlation coefficient and the coefficient of variation were calculated to analyze repeatability of the OCTARA automatically generated vessel density measurements. RESULTS: A total of 35 eyes were included in the study. The intraclass correlation coefficient of the global vessel density in the superficial capillary plexus and the deep capillary plexus were 0.963 and 0.975, respectively, and the coefficient of variation were 5.4% and 4.4%, respectively. The intraclass correlation coefficient of the rest of the global measurements was indicative of good reliability with the exception of the deep choroid layer with an intraclass correlation coefficient of 0.6 indicative of moderate reliability. CONCLUSIONS: Our results proved excellent repeatability of automated vessel density measurements in the superficial and deep capillary plexus layers in our cohort using a OCTARA algorithm indicating that it may be a reliable diagnostic tool. It also showed good reliability in choriocapillary and mid choroid layer. These findings may be of value in assessing the significance of differences in capillary density measurements over time and across different settings.

New insights in the pathogenic mechanism of multiple sclerosis: Is Epstein-Barr virus associated with optic nerve involvement?

INTRODUCTION: There are no reports in the literature studying the possible relationship between Epstein-Barr virus (EBV) and optic nerve involvement in multiple sclerosis (MS). The aim of our study was to analyze the association between EBV antibodies titres and optical coherence tomography (OCT) and OCT angiography (OCTA) quantitative parameters. METHODS: We conducted a retrospective study. The study included 98 eyes of 49 patients with MS. Years of MS duration, relapse count, history of optic neuritis (ON), and immunoglobulin (Ig) G antibodies to the EBV viral capsid antigen (VCA) were recorded from each patient. Also, OCT analysis (including retinal nerve fibre layer (RNFL) thickness and ganglion cell-inner plexiform layer (GCIPL) thickness) and OCTA analysis (including perfusion density (PD) and flux index (FI) of the radial peripapillary capillary plexus) were performed in each participant. RESULTS: No significant associations were observed between anti-EBV antibody levels and OCT or OCTA parameters (p > 0,05). Correlation analysis between OCT and OCTA measurements showed a significant positive correlation between RNFL thickness and GCIPL thickness with peripapillary PD and FI (p < 0,035). Subgroup analysis revealed a significant diminution of RNFL thickness, GCIPL thickness and peripapillary PD and FI (p < 0,05) in the ON group. CONCLUSION: We were unable to demonstrate a significant association between anti-EBV VCA IgG antibody titres and OCT or OCTA parameters. Nonetheless, further longitudinal studies are needed to explore the possible association of EBV with optic nerve involvement in MS.

Optical coherence tomography angiography analysis in patients with intracranial hypertension.

INTRODUCTION: Evidence on peripapillary microvasculature in intracranial hypertension (IH) after the regression of papilledema is still scarce. The aim of this preliminary study was to determine the association between structural changes in the optic nerve and the retina and peripapillary microvasculature in patients with IIH. METHODS: We conducted a retrospective study. The study included 39 eyes of 21 patients with IIH. Treatment for IIH and history of obesity were registered from each patient. Moreover, OCT analysis including retinal nerve fiber layer (RNFL) thickness and ganglion cell-inner plexiform layer (GCIPL) thickness, and OCTA analysis including perfusion density (PD) and flux index (FI) of the radial peripapillary capillary plexus were performed. RESULTS: Correlation analysis revealed a high correlation between GCIPL thickness and peripapillary PD and FI (p < 0,05, r > 0,7), whereas the degree of correlation between RNFL thickness and peripapillary microvascular parameters was low (p < 0,05, r < 0,7). Patients with regressed papilledema had significantly lower GCIPL thickness and peripapillary PD than control subjects (p < 0,05). CONCLUSION: Peripapillary microvascular measurements are highly correlated with GCIPL thickness in patients with IIH. Moreover, GCIPL thickness and peripapillary PD are significantly inferior in patients with regressed papilledema compared to control group. Thus, we suggested that peripapillary microvascular parameters may be an early indicator of optic nerve atrophy in patients with IIH.

Quantitative Assessment of Retinal and Choroidal Microvasculature in Asymptomatic Patients with Carotid Artery Stenosis.

SIGNIFICANCE: Carotid disease contributes to 15 to 20% of all ischemic strokes, one of the leading causes of permanent disabilities and mortality globally. With its growing prevalence and the inflicted disability rates, screening for anomalies that precede the onset of its serious complications is of crucial global significance. PURPOSE: This study aimed to assess the relationship between retinal and choroidal perfusion changes with the degree of stenosis using quantitative swept-source optical coherence tomography angiography in patients with internal carotid artery stenosis. METHODS: A retrospective cohort study was conducted in 72 eyes with carotid stenosis. According to the degree of stenosis, the participants were divided into a healthy group (group 1: 34 eyes), a mild-moderate stenosis group (group 2: 22 eyes), and a severe stenosis group (group 3: 16 eyes). Swept-source optical coherence tomography angiography was performed to scan macular fovea. Capillary density values in the different retinal and choroidal layers were the major measurements for our study. RESULTS: Mean vessel density in the midchoroid layer was significantly higher in groups 2 and 3 compared with group 1. Deep choroid disclosed significantly superior vascular density values in group 3 compared with groups 2 and 1. Superficial and deep capillary plexus showed decreased vascular density values when comparing group 3 with groups 1 and 2, although they were not significant. CONCLUSIONS: Our report provides the first evidence that choroidal microvascular changes were correlated with severity of carotid artery stenosis. Optical coherence tomography angiography can sensitively detect subtle, early changes in the ocular blood in carotid disease representing a useful, noninvasive, and objective approach to the retinal microvasculature.

Two-Year Results of a Treat and Extend Regimen with Aflibercept in Caucasian Patients with Pachychoroid Neovasculopathy.

INTRODUCTION: There are few reports evaluating the treatment of pachychoroid neovasculopathy (PNV) in white patients. The purpose of this study is to analyze the results of a treat and extend regimen with aflibercept in white patients with PNV after 2 years of follow-up. METHODS: We performed a retrospective study in 31 eyes of 26 patients with PNV treated with a treat an extend regimen of intravitreal aflibercept. The mean age was 63,84 ± 7.92 years. There were 9 males (35%) and 17 females (65%). Best-corrected visual acuity (BCVA), central macular thickness (CMT), choroidal subfoveal thickness (CST), choroidal thickness (CT) under type 1 choroidal neovascularization (CNV), pigment epithelium detachment (PED) height, and presence of subretinal fluid (SRF), intraretinal fluid (IRF) and wet macula, were evaluated at baseline and after 3, 6, 12, and 24 months. RESULTS: BCVA remained stable during the follow-up (p 0.161). A significant diminution of CMT was found (p 0.001). Conversely, PED height diminution was not significative (p 0.260). CST and CT under type 1 CNV improved significantly during the follow-up (p 0.005 and 0.009, respectively). Also, wet macula improved after 24 months (p < .001). The average number of intravitreal injections was 12.34 ± 6.01. CONCLUSION: Treat and extend regimen with intravitreal aflibercept in white patients with PNV may be effective for improving CMT, CST, CT under type 1 CNV and wet macula, and to stabilize vision, with a personalized regimen of intravitreal injections.

Twelve-year follow up of a case of autosomal recessive bestrophinopathy with transient resolution of retinal edema in one eye.

PURPOSE: To report 12-year follow-up of a patient with ARB. CASE REPORT: A 25-year-old man presented with blurred vision in his both eyes (OU). Best-corrected visual acuity (BCVA) was 20/63 Snellen equivalent in the right eye (OD) and 20/32 Snellen equivalent in the left eye. The intraocular pressures and anterior segment examination were unrevealing in OU. Posterior segment examination revealed multiple yellowish flecks and dots in the posterior pole in OU. Optical coherence tomography (OCT) showed subretinal fluid (SRF), intraretinal hyporeflective spaces, elongated and shaggy photoreceptors and outer retinal defects. Fundus autofluorescence demonstrated mottling hyperautofluorescence and hypoautofluorescence in the posterior pole in OU. Fluorescein angiography illustrated hyperfluorescence in the posterior pole and surrounding the arcades in OU. Multifocal electroretinography objectified mild to markedly abnormal responses in all ring areas in OU. Molecular genetic testing confirmed two heterozygous sequence variations in the BEST1 gene. At 4 years of follow-up, OCT revealed a complete resolution of SRF and a partial resolution of intraretinal hyporeflective spaces in the OD with corresponding improvement in the BCVA to 20/23 Snellen equivalent in the OD, even though outer retinal defects persisted. Our patient denied recent changes in his alimentary habits and medical history at that time. Posteriorly, SRF and intraretinal hyporeflective spaces reappeared in the OD. CONCLUSION: To the best of our knowledge, this is the first case report of ARB with a transient resolution of retinal edema in one eye without medical treatment and dietary therapy.

Optical coherence tomography angiography quantitative analysis of retinal and choroidal microvasculature in patients with dyslipidemia.

INTRODUCTION: The purpose of this investigation was to report swept source-optical coherence tomography angiography (SS-OCTA) quantitative information of retinal and choroidal microvascularization in patients with dyslipidemia (DL). METHODS: We performed a retrospective study. The study enrolled 37 eyes of 20 patients with DL and 40 eyes of 23 healthy subjects. OCTA quantitative parameters (vessel density (VD) and foveal avascular zone (FAZ) area of superficial capillary plexus (SCP), middle capillary plexus (MCP), deep capillary plexus (DCP) and choriocapillaris (CC)) in 6 mm × 6 mm and 4,5 mm × 4,5 mm cubes were recorded. RESULTS: No differences in VD in SCP, MCP and DCP were demonstrated between DL group and control group (p > 0,05). Conversely, VD in the central region at CC was diminished in patients with DL in both cubes (p < 0,05). Moreover, total VD in CC was decreased in the DL group in 6 mm × 6 mm cube (p < 0,05). Regarding FAZ area, we demonstrated and enlargement of FAZ in each retinal capillary plexus, but it did not reach statistical significance (p > 0,05). CONCLUSION: We objectified a diminution of VD in the CC, suggesting that DL mainly affects the choroidal microvasculature. Nonetheless, further studies with a larger population are needed.

Swept-source optical coherence tomography angiography automatic analysis of microvascular changes secondary to systemic hypertension.

INTRODUCTION: The aim of our study is to report swept-source optical coherence tomography angiography (SS-OCTA) quantitative parameters of retinal and choroidal microvasculature in patients with systemic hypertension (HTN) using a built-in software of SS-OCTA. METHODS: We performed a retrospective study. This study enrolled 93 eyes of 51 subjects with HTN and 71 eyes of 38 healthy subjects. OCTA quantitative parameters (vessel density (VD) and foveal avascular zone (FAZ) area of superficial capillary plexus (SCP), middle capillary plexus (MCP), deep capillary plexus (DCP), total capillary plexus (TCP) and choriocapillaris (CC)) of the OCTA cube of 4,5 mm × 4,5 mm were recorded. RESULTS: A decrease of parafoveal VD in CC, DCP and TCP were demonstrated between HTN group and control group (p < 0,05). Conversely, no differences were demonstrated in parafoveal VD of SCP and MCP (p > 0,05). Subgroup analysis revealed a diminution of central VD at SCP, DCP and TCP in patients taking one antihypertensive drug compared to patients treated with two medications (p < 0,05). Correlation analysis showed a significant, albeit weak, negative correlation between HTN duration, and parafoveal VD in the SCP and FAZ area at SCP, DCP and TCP (p < 0,05 and r < 0,300). CONCLUSION: When normative data are available, OCTA might be used as a potential tool in the prevention and follow-up of end-organ damage secondary to HTN. Nonetheless, further studies are needed to confirm this hypothesis.

Optical coherence tomography biomarkers in patients with macular edema secondary to retinal vein occlusion treated with dexamethasone implant.

PURPOSE: To evaluate the impact of optical coherence tomography (OCT) biomarkers on intravitreal dexamethasone (DEX) implant clinical outcomes in patients with macular edema secondary to retinal vein occlusion (RVO-ME). METHODS: Retrospective study conducted on a cohort of patients with RVO-ME, either naïve or previously treated, who underwent treatment with DEX implant and had a follow-up of 6 months. Anatomic success was defined as a central retinal thickness (CRT) < 250 µm or a relative reduction of CRT ≥10% from baseline. The primary endpoint was the mean change in CRT from baseline to month-6. Secondary end-points included changes in BCVA, the impact of baseline OCT biomarkers on functional and anatomic outcomes; and the impact of treatment on the different OCT biomarkers. OCT biomarkers associated with functional and anatomic outcomes were estimated using a logistic regression model. RESULTS: Fifty-seven eyes were included in the study. Baseline CRT was significantly decreased from 567.6 ± 226.2 µm to 326.9 ± 141.0 µm at month-6 (p < 0.0001). Baseline BCVA was significantly lower in the eyes with disrupted external limiting membrane (ELM) (mean 40.3 ± 21.3 letters) than in those with non-disrupted (mean 68.6 ± 10.7 letters) or partially-disrupted ELM (mean 59.6 ± 13.2 letters), p = 0.0001 and p = 0.0011, respectively. Baseline BCVA was significantly lower in eyes with > 20 hyperreflective foci (HRF) than in those with < 10 HRF (p = 0.0388). The eyes with disorganization of the retinal inner layers (DRIL) had lower baseline BCVA than those without DRIL (Hodges-Lehmann median difference: - 12.0 letters, 95% CI: - 25.0 to - 5.0 letters, p = 0.0042). At month-6, 26 (45.6%); 24 (42.1%), and 20 (35.1%) eyes achieved a BCVA improvement ≥5, ≥10, and ≥ 15 letters respectively. Forty (70.2%) eyes were classified as anatomic success at month-6. Logistic regression analysis found none factor significantly associated with success in the multivariate analysis. CONCLUSIONS: The results of this study suggested a positive impact of DEX on CRT and BCVA in eyes with RVO-ME. No OCT-biomarkers were identified as predictors of clinical-outcomes. Additionally, presence of DRIL, presence of HRF (> 20), or disrupted ELM were significantly associated with worse baseline BCVA.

New insights in pathogenic mechanism of hydroxychloroquine retinal toxicity through optical coherence tomography angiography analysis.

INTRODUCTION: There is only a unique report with a small sample size studying hydroxychloroquine (HCQ) retinal toxicity with swept-source (SS) optical coherence tomography angiography (OCTA). The aim of this study was to quantify OCTA quantitative parameters in patients who underwent HCQ therapy. METHODS: We conducted a retrospective study. The study included 43 eyes of 22 patients taking HCQ for more than 5 years (high-risk group), 57 eyes of 29 patients taking HCQ for 5 years or less (low-risk group) and 25 eyes of 50 age-matched healthy controls. OCTA quantitative parameters (vessel density (VD) and foveal avascular zone (FAZ) area in superficial capillary plexus (SCP), middle capillary plexus (MCP), deep capillary plexus (DCP), total capillary plexus (TCP), and choriocapillaris (CC)) were recorded. RESULTS: In the low-risk group, VD in the SCP and MCP was increased compared to control group (p value <.05). In the high-risk group, VD in the SCP, MCP and TCP was increased (p value <.05). The subgroup analysis revealed an increased VD at SCP in systemic lupus erythematosus (SLE) high-risk patients, an increased VD at TCP and CC in rheumatoid arthritis (RA) high-risk subjects, and a decreased VD at CC level in the high-risk group patients with Sjögren's syndrome (SS) and connective tissue disease (CTD) (p value <.05). Furthermore, we demonstrated a significant enlargement of FAZ area at MCP level in the high-risk group patients with SS and CTD (p value <.05). CONCLUSION: We demonstrated an increase of VD in patients who underwent HCQ treatment, so we suggest that HCQ retinal toxicity is not vascular mediated.

Peripapillary pachychoroid neovasculopathy: A novel entity.

PURPOSE: Peripapillary pachychoroid syndrome is a new variant of pachychoroid disease recently described. It is important to establish the features and pathogenic mechanisms of this novel entity and its possible association with type 1 neovascularization. The aim of this study is to report a case of peripapillary pachychoroid neovasculopathy, a novel entity of pachychoroid spectrum disease. CASE REPORT: A 51-year-old woman was referred to our macula service due to macular pigmentary changes in her right eye. On examination, best corrected visual acuity was 20/20 Snellen equivalent in both eyes. Funduscopy revealed pigmentary changes in the papillonasal bundle in her right eye with mottled autofluorescence in the same area where pigmentary changes. Spectral-domain optical coherence tomography illustrated a shallow irregular pigment epithelium detachment (PED) which appears as "double layer sign" overlying pachyvessels, with hyper-flow signal beneath it and a large network of neovascularization revealed by cross-sectional optical coherence tomography angiography. Indocyanine green angiography confirmed the pachyvessels and choroidal hyper-permeability in the peripapillary region. A diagnosis of peripapillary pachychoroid neovasculopathy was made and good visual and anatomical outcomes were achieved with a treat-and-extend regimen with intravitreal aflibercept. CONCLUSION: We report a novel entity within the pachychoroid spectrum disease, the peripapillary pachychoroid neovasculopathy, which could improve our understanding of the pathogenic mechanism of choroidal neovascularization in pachychoroid disorders.

Optical coherence tomography angiography analysis of aneurismal type 1 neovascularization in a cohort of white patients.

INTRODUCTION: To evaluate the detection rate of aneurysmal type 1 neovascularization (AT1) in Caucasian patients using optical coherence tomography angiography (OCTA) and to describe OCTA characteristics of AT1 in a cohort of white patients. METHODS: We conducted an observational retrospective study in 44 eyes of 43 patients with AT1. All patients underwent a complete ophthalmologic examination, including fundus imaging, indocyanine green angiography, optical coherence tomography, and OCTA. Branching vascular network (BVN) and polyp detection rates by OCTA were evaluated. Furthermore, we described BVN and polyp morphologies on en face OCTA and flow of polyps on B-scan OCTA. RESULTS: En face OCTA revealed BVN in 84.09% of cases and polypoidal lesions in 86.36% of cases. B-scan OCTA showed BVN and polyps in 95.45% and 93.18% of the patients, respectively. "Trunk" BVNs (51.35%) and "ring" polyps (47.37%) were the most frequent morphologies observed in our cohort, and "patchy hyperflow" (80.49%) signal was the most common visualized in our patients. Regarding OCT parameters, CT under polyps was higher in patients with positive detection of polyps on B-scan OCTA. CONCLUSION: OCTA is a possible diagnosis tool in Caucasian patients with AT1. To the best of our knowledge, this is the largest study performed in Caucasian patients regarding OCTA diagnostic abilities and features.

Multimodal imaging of two cases of retinal vein occlusion secondary to Waldenstrom macroglobulinemia.

PURPOSE: To report multimodal imaging characteristics of two cases of retinal vein occlusion (RVO) secondary to Waldenstrom macroglobulinemia (WM). CASE REPORT: Case 1: An 82-year-old woman presented with vision loss. She had a history of WM. Best-corrected visual acuity (BCVA) was 20/100 Snellen equivalent in the right eye (OD) and 20/63 in the left eye (OS). Fundoscopy showed bilateral hemorrhages in posterior pole and along superotemporal arcade. Fluorescein angiography illustrated no macular leakage. Optical coherence tomography (OCT) revealed macular edema (ME). Optical coherence tomography angiography (OCTA) demonstrated abnormalities in choriocapillaris. A diagnosis of bilateral branch RVO was made and ME was treated with intravitreal dexamethasone, achieving stability. Case 2: A 65-year-old man presented with venous dilation, tortuosity, and intraretinal hemorrhages. BCVA was 20/20 in both eyes (OU). OCT showed ME and hyperreflective dots in choroid. A diagnosis of bilateral central RVO was made. Laboratory evaluation and bone narrow biopsy confirmed a diagnosis of WM. After that, our patient consulted because of vision loss. BCVA was 20/400 in OU. Fundoscopy and OCT demonstrated a worsening of the intraretinal hemorrhages and the ME. OCTA showed damage of choriocapillaris. Thus, intravitreal dexamethasone and plasmapheresis was advised. Two months after, BCVA was 20/40 in the OD and 20/32 in the OS. Also, fundoscopy and OCT improved. CONCLUSION: Early treatment of WM is truly important, with the aim of achieving a decrease in IgM levels in order to avoid toxic effect over the RPE that results in refractory ME.

Two-year results of a treat and extend regimen with aflibercept in Caucasian patients with aneurysmal type 1 neovascularization.

INTRODUCTION: There are a few reports investigating the treatment of aneurysmal type 1 neovascularization (AT1) in Caucasian patients. The aim of this study is to evaluate the 2-year results of a treat and extend regimen with aflibercept in Caucasian patients with AT1. METHODS: We conducted an observational retrospective study in 28 eyes of 26 patients with naïve AT1 treated with a treat an extend regimen of intravitreal aflibercept. Best corrected visual acuity (BCVA), central macular thickness (CMT), pigment epithelium detachment (PED) height, presence of dry macula, and regression rate of polypoidal lesions were assessed at baseline and at 12 and 24 months. RESULTS: BCVA was significantly increased by 9.03 ± 16 letters (p < 0.01) and 9.2 ± 16.87 letters (p < 0.01) after the 12 and 24 months follow-up. A significant decrease of CMT was found at 12 and 24 months (p < 0.01). Nevertheless, significant changes in PED height were not observed (0.1 < p > 0.05). At 12 and 24 months of follow-up, dry macula was achieved in a total of 10 eyes (35.71%) and 15 eyes (53.57%). The regression rate of polypoidal lesions was 25% (7 eyes) and 35.71% (10 eyes) after 12 and 24 months. The mean number of intravitreal injections was 7.81 ± 3.20 the first year and 6.11 ± 3.49 the second year. CONCLUSION: To the best of our knowledge, treat and extend regimen with intravitreal aflibercept in Caucasian patients may be effective for improving BCVA, CMT, wet macula, and regression rate of polypoidal lesions.

Quantitative analysis of retinal and choroidal vasculature in patients with chorioretinal folds secondary to hyperopia.

INTRODUCTION: There is a lack of information on quantitative parameters of optical coherence tomography angiography (OCTA) in cases of chorioretinal folds (CRF). The aim of this study was to compare OCTA retinal and choriocapillary vessel density (VD) between normal subjects and patients with CRF. METHODS: We conducted an observational retrospective study. A total of 16 eyes of eight patients were recruited (eight eyes with CRF and eight control eyes). Data on best-corrected visual acuity (BCVA), refractive error, central macular thickness (CMT), central subfoveal thickness (CST), and OCTA findings (VD and foveal avascular zone (FAZ) area in superficial capillary plexus (SCP), middle capillary plexus (MCP), and deep capillary plexus (DCP), and VD in choriocapillaris (CC)) were recorded in each eye. RESULTS: Compared with control group, CRF group showed decreased VD in the foveal region of SCP and MCP (p value 0.003 and 0.001), and increased VD in nasal region of SCP and MCP (p value 0.02 and 0.001), and in parafoveal area of MCP (p value 0.005). No differences were found in DCP and CC layers. Furthermore, we observed an enlargement of FAZ in CRF group at SCP and MCP slabs (p value <0.001 and 0.015). Respect to optical coherence tomography parameters, we demonstrated a thicker choroid in the CRF group (p value 0.002). CONCLUSION: To the best of our knowledge, this is the first study reported in the literature quantifying VD of retinal capillary plexus and CC in a group of patients with a diagnosis of CRF secondary to hyperopia.

Vitamin A retinopathy secondary to Billroth II anastomosis triggered after the beginning of dialysis.

PURPOSE: To report a case of vitamin A retinopathy secondary to Billroth II anastomosis triggered after the beginning of dialysis in a patient with a chronic renal failure. CASE REPORT: A 73-year-old male complained of nyctalopia that had started 9 months ago, coinciding with the beginning of dialysis. His medical history is remarkable for hepatic cirrhosis and Billroth II anastomosis 20 years ago. Best-corrected visual acuity (BCVA) was 60 letters in both eyes. Dilated fundus examination showed faint white-yellowish dots. Optical coherence tomography (OCT) illustrated hyperreflective dots and small hyporreflective cavities between the retinal pigment epithelium (RPE) and the ellipsoid zone (EZ). En face OCT showed multiple hyperreflective dots that coincide with white-yellowish dots of the fundus, and multiple hyporreflective defects which correspond to hyporreflective cavities seen in the OCT. Visual field examination showed concentric narrowing of the visual field. A diagnosis of vitamin A deficiency was confirmed and oral vitamin A supplementation was initiated. One month after treatment, the patient reported a subjective improvement of nyctalopia, and BCVA ameliorated up to 80 and 85 letters. Fundus examination, OCT, and en face OCT showed a diminution of the observed lesions. Moreover, visual field improved. CONCLUSION: Early diagnosis of vitamin A deficiency can prevent irreversible visual sequelae. This highlights the crucial role of ophthalmologists in the prompt detection of this condition. A lifelong monitoring should be needed in patients undergoing biliopancreatic diversion surgery. Furthermore, OCT and en face OCT becomes a main tool in the diagnosis and monitor response to treatment.

Case Report: Multimodal Imaging Features of an ABCA4 Cone Dystrophy.

SIGNIFICANCE: Cone dystrophies and cone-rod dystrophies are a group of rare inherited pathologies characterized by degeneration of cone photoreceptors and subsequent rod involvement. The identification of causative genes is essential for diagnosis, and advanced imaging is acquiring great value in the characterization of the different phenotypic expressions. PURPOSE: We describe genotype-phenotype associations of an autosomal recessive ABCA4-associated cone dystrophy using multimodal imaging. CASE REPORT: A 34-year-old woman presented with progressive visual acuity decay. Visual acuity was 20/32 for her right eye and 20/25 for her left eye. A central scotoma was detected on a 10-2 Humphrey visual field in both eyes. Funduscopy revealed perifoveal retinal pigment epithelial changes, and fundus autofluorescence using blue excitation light showed decreased autofluorescence in the central fovea of both eyes with surrounding annular ring of increased autofluorescence in the perifoveal zone; green excitation light fundus autofluorescence was more accurate in the characterization of the size, perimeter, and circularity of central hypofluorescent lesions. Optical coherence tomography revealed an incomplete focal cavitation in both foveas, and optical coherence tomography angiography images showed a reduction in the superficial and deep capillary plexus density, an increased foveal avascular area, and subtle voids in choriocapillaris blood flow. Electroretinography was consistent with cone dystrophy, and molecular testing revealed the alteration of the ABCA4 gene. CONCLUSIONS: The identification of an incomplete focal cavitation could alert the clinician to consider early ABCA4 central cone dystrophy. The patient in this case also exhibited reduced vessel density in the foveal area. Both of these characteristics could be important features related to the underlying genetic mutation.

Case Report: Ipilimumab-induced Multisystem Autoimmune-like Toxicities Suggestive of Vogt-Koyanagi-Harada-like Syndrome.

SIGNIFICANCE: The emergence of new cancer therapies has dramatically improved outcomes in metastatic melanoma. Immune checkpoint inhibitors have been the most effective treatment. Although, as a direct consequence of the immune dysregulation induced by them, adverse effects termed immune-related adverse events are observed in more than 60% of the patients. PURPOSE: We describe the clinical presentation of Vogt-Koyanagi-Harada-like syndrome in a patient with concomitant systemic melanoma treatment with ipilimumab, a cytotoxic T lymphocyte-associated antigen 4 blocker. METHODS: This study aimed to report a case of ipilimumab-induced vitritis, papillitis, and skin and auditory signs suggestive of Vogt-Koyanagi-Harada-like syndrome. CASE REPORT: A 64-year-old woman with metastatic melanoma presented with bilateral blurred vision and hearing loss upon completion of three cycles of treatment with ipilimumab. Ophthalmologic examination revealed a bilateral granulomatous uveitis with intense vitritis and papillitis. The result of optical coherence tomography was normal, and fluorescein angiography confirmed the bilateral papillary edema. Ipilimumab was withdrawn, and treatment with oral and systemic steroids led to a rapid improvement in the ophthalmologic and auditory manifestations. Three months after initial presentation, the patient developed vitiligo and poliosis. CONCLUSIONS: Vogt-Koyanagi-Harada-like syndrome can develop in the process of immunological deregulation by ipilimumab in the treatment of metastatic melanoma and can correlate temporally with the efficacy of the drug in tumor regression. These observations may help elucidate the underlying mechanism of Vogt-Koyanagi-Harada syndrome as well as the relation between tumor-associated tolerance and autoimmunity.