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.

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.

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: Optic Nerve Atrophy Secondary to Sjögren Syndrome.

SIGNIFICANCE: Optic neuropathy associated with Sjögren syndrome is rare and usually has an acute onset. PURPOSE: This study aimed to report a case of asymmetric optic nerve atrophy attributed to Sjögren syndrome. CASE REPORT: A 37-year-old woman was referred to neuro-ophthalmology service because of right optic nerve atrophy of unknown etiology. The patient was asymptomatic. Best-corrected visual acuity was 20/200 Snellen equivalent in the right eye and 20/20 Snellen equivalent in the left eye. The right eye had a relative afferent pupillary defect. Visual field demonstrated dense temporal loss, superior arcuate involvement, and an inferior paracentral defect in the right eye. Slit-lamp examination showed mild fluorescein staining of the cornea, moderate lissamine green staining of the conjunctiva, and abnormal tear breakup time in both eyes. Fundus examination revealed diffuse pallor of the right optic disc and a normal left optic disc. Optical coherence tomography showed inferior and superior retinal nerve fiber layer atrophy in the right eye and inferior retinal nerve fiber layer atrophy in the left eye. A diagnosis of right optic nerve atrophy was made. Immunologic studies were significant for positive anti-Ro and anti-La antibodies. MRI of the brain and orbit ruled out any intracranial or white-matter pathology. A diagnosis of optic nerve atrophy secondary to Sjögren syndrome was suspected, so corticosteroid treatment was started. CONCLUSIONS: Optic nerve atrophy may be the initial manifestation of Sjögren syndrome. Therefore, optic neuropathy associated with Sjögren syndrome remains a diagnostic challenge. In these cases, specific antibodies such as anti-Ro and anti-La facilitate early diagnosis and can prevent vision-threatening complications.

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.

Effect of Dexamethasone Intravitreal Implant on Visual Acuity and Foveal Photoreceptor Integrity in Macular Edema Secondary to Retinal Vascular Disease.

PURPOSE: The aim of this study was to evaluate the effect of the dexamethasone intravitreal (DEX) implant on the external limiting membrane (ELM) and ellipsoid zone (EZ) integrity in treatment-naïve patients with macular edema (ME) secondary to retinal vascular disease (RVD). METHODS: This is a retrospective study conducted on patients with ME secondary to RVD, who underwent a DEX implant. RESULTS: One-hundred eyes were included. Mean age was 70.3 ± 11.1 years. Mean ELM integrity significantly improved from 1,575.9 ± 285.9 µm at baseline to 1,711.7 ± 244.0 µm at month 3 (p < 0.0001). Similarly, there was a significant improvement in EZ integrity from baseline to month 3 (1,531.5 ± 317.1 vs. 1,694.3 ± 252.8 µm, respectively, p < 0.0001). At month 3, mean visual acuity (VA) gain was 9.9 ± 14.1 letters (p < 0.0001). Mean central retinal thickness (CRT) significantly decreased by -193.2 ± 185.7 µm from baseline to month 3 (p < 0.0001). Mean changes in VA and CRT were significantly correlated with baseline ELM integrity (p = 0.0065 and p = 0.0046, respectively) and EZ integrity (p = 0.0300 and p = 0.0035, respectively). At month 3, the proportion of eyes which had an intact ELM (mean difference 16.0%, 95% CI 5.4-26.4%, p = 0.0033) and EZ (mean difference 12.0%, 95% CI 1.8-22.1%, p = 0.0210) was significantly higher than at baseline. CONCLUSIONS: DEX implant was able to significantly improve ELM and EZ integrity in naïve patients with ME.

Case Report: Tapioca Iris Melanoma: An Uncommon Presentation of an Uncommon Tumor.

SIGNIFICANCE: Iris tumors are rare conditions, and there is a relative paucity of recent published data on its broad clinical spectrum. Tapioca iris melanoma is a rarer yet devastating form with wide and challenging differential diagnoses because of its amelanotic nodular appearance. PURPOSE: This study aimed to report the challenging presentation of an uncommon iris melanoma, describing the clinical and histological findings and comparing them with the existing published data. CASE REPORT: An uncommon clinicopathological report on the tumor unusual localization, patient age, absence of elevated IOP and heterochromia, and negative S-100 stain that caused diagnostic uncertainty is presented. The patient remains free of metastatic disease 7 years after a complete tumor full-thickness excision. CONCLUSIONS: Tapioca iris melanomas are uncommon tumors with a presentation/surgical management that differs from other malignant tumors. Ophthalmologists should consider it among the vast differential diagnoses when observing amelanotic lesions, even without the hallmark signs being evident.

Case Report: Nutritional and Toxic Optic Neuropathy: A Diagnostic Dilemma.

SIGNIFICANCE: Nutritional and toxic optic neuropathies are rare disorders characterized by visual impairment due to optic nerve damage by a toxin, usually with coexisting nutritional deficiencies. Its pathophysiology is still unclear, and multiple mechanisms implicated act synergistically to bring about this condition. The decline in its incidence and its confusing clinical appearance make diagnosing nutritional and toxic optic neuropathies challenging. PURPOSE: This is an observational clinical case report of an atypical clinical case of a nutritional and toxic optic neuropathy with a subacute presentation and papilledema at the time of diagnosis. The patient provided written informed consent for medical information and images to be published. CASE REPORT: A 47-year-old man presented with progressive, painless bilateral decrease in central vision over 15 days. The patient had a long-standing history of alcohol abuse and was a heavy smoker. The examination revealed dyschromatopsia, 20/400 visual acuity on both eyes, and no relative afferent pupillary defect. Funduscopy revealed bilateral papilledema. A visual field test showed generalized depression with centrocecal involvement in the left eye. Laboratory studies evidenced decreased vitamin B12/B1 and red blood cell folate levels, increased acute phase reactants, hypertransaminasemia, and macrocytic anemia. Serologies and methanol in urine were negative. After the discontinuation of tobacco use and alcohol accompanied by vitamin supplementation, our patient's visual field, visual acuity, and papilledema improved remarkably. After 5 months, visual acuity and funduscopy were normal. CONCLUSIONS: Although some hallmark signs were visible in this case, its subacute presentation and the presence of papilledema at diagnosis caused some diagnostic uncertainty. Nutritional and toxic optic neuropathy is a rare and challenging diagnosis because of a lack of biomarkers. Eye care clinicians should consider nutritional and toxic optic neuropathies to prevent severe and irreversible visual damage resulting from underdiagnosis and mismanagement.

Intravitreal dexamethasone implant Ozurdex® in naïve and refractory patients with different subtypes of diabetic macular edema.

BACKGROUND: Diabetic macular edema (DME) can be treated with different alternatives, among them Dexamethasone intravitreal implant 0.7 mg (DEX 0.7) has demonstrated that may improve both central macular thickness (CMT) and best corrected visual acuity (BCVA). This study aimed to evaluate the effect of the intravitreal dexamethasone implant Ozurdex® in patients with different subtypes of diabetic macular edema over a 6-month follow-up period. METHODS: Eighty-four (29 naïve and 55 previously treated) eyes were included in this retrospective study. For each patient, the BCVA [Early Treatment Diabetic Retinopathy Study (ETDRS) charts] and macular thickness on optical coherence tomography (OCT) at baseline visit and within the 2nd, 4th, and 6th months of follow-up were obtained. The main outcomes measurements were the mean change in BCVA and in CMT with respect to the baseline value. The percentage of patients gaining ≥5 letters and ≥ 10 letters in BCVA was also analyzed. RESULTS: A total of 84 eyes, 29 (34.5%) naïve and 55 (65.5%) non-naïve, from 69 patients were included in the study. BCVA at baseline was 58.8 (16.4) and 61.8 (11.6) in naïve and refractory patients, respectively, p = 0.4513. At every visit, BCVA significantly improved from baseline in naïve and non-naïve eyes (p < 0.0001 and p = 0.0003, respectively; Friedman rank sum test). At baseline, the mean CMT was 466.2 (189.7) µm and 448.1 (110.7) µm in the naïve and non-naïve patients, respectively (p = 0.5830); and decreased to 339.3 (92.5) µm and 357.5 (79.1) µm, respectively (p = 0.0004 and p < 0.0001, respectively, Wilcoxon signed-rank test). The proportion of patients gaining ≥10 letters was significantly greater in the naïve group, p = 0.0199. CONCLUSION: The intravitreal dexamethasone implant (Ozurdex) is effective for the treatment of diabetic macular edema, even in refractory cases that have failed to respond to previous therapies.

Sequential Dexamethasone and Aflibercept Treatment in Patients with Diabetic Macular Edema: Structural and Functional Outcomes at 52 Weeks.

OBJECTIVE: The aim of this paper is to compare intravitreous aflibercept versus dexamethasone implant followed by aflibercept (sequential treatment group) in patients with diabetic macular edema (DME). METHODS: We conducted an observational retrospective study in naïve DME patients, 15 treated only with aflibercept (a monthly injection for the first 5 consecutive doses, followed by an injection every 2 months), and 15 treated with a single dexamethasone implant followed by bimonthly aflibercept. Best-corrected visual acuity (BCVA), central macular thickness (CMT), and qualitative features as well as adverse events were assessed at baseline and at 2, 6, and 12 months. RESULTS: BCVA increased from 70.8 ± 4.1 to 83.5 ± 2.7 letters with aflibercept and from 75.6 ± 2.7 to 86.5 ± 2.5 with sequential treatment (p = 0.551). CMT decreased from 411 ± 26.1 to 288.1 ± 10.5 with aflibercept and from 411.4 ± 24.3 to 260.8 ± 17.9 in the sequential treatment group. The differences between the 2 groups, in terms of visual gain and decreased MT, were not statistically significant (p > 0.05). Nine and 6 injections and 9 and 7 monitoring visits were performed. CONCLUSION: Sequential treatment in DME, starting with dexamethasone and followed by aflibercept, is a promising alternative that can reduce the treatment burden in the first year without statistically significant differences in terms of visual gain and decreased MT compared to aflibercept only.

One-Year Outcomes of the Treat-and-Extend Approach with Aflibercept in Age-Related Macular Degeneration: Effects on Typical Choroidal Neovascularization and Retinal Angiomatous Proliferation.

OBJECTIVE: To investigate functional/morphological outcomes of the treat-and-extend regimen (TER) with aflibercept in typical choroidal neovascularization (CNV) and retinal angiomatous proliferation (RAP) secondary to exudative age-related macular degeneration (AMD). METHODS: This was a retrospective study of 37 eyes treated with 2 mg aflibercept according to a TER protocol. Examinations included best corrected visual acuity (BCVA), numbers of injections, and visits needed. Additionally, quantitative/qualitative analyses with fluorescein angiography and spectral- domain optical coherence tomography were conducted at baseline as well as at 3, 6, and 12 months. RESULTS: BCVA significantly improved from 0.6 ± 0.27 to 0.4 ± 0.34 logMAR. The final mean numbers of injections were 8.03 ± 1.27 and 7.28 ± 0.75 and the numbers of visits 6.5 ± 1.09 and 7.14 ± 1.57 in typical CNV and in RAP or atypical CNV, respectively, and they did not differ between the different subtypes of CNV (p > 0.05). CONCLUSIONS: Aflibercept in TER is effective for all exudative AMD subtypes. The patient's visual gain, the mean number of injections, and the number of visits needed did not depend on the subtype of CNV.

Swept-source optical coherence tomography early findings in patients with carotid artery disease.

PURPOSE: Ocular ischemic syndrome can be the first and only hint of life-threatening carotid artery disease. The early recognition of carotid stenosis-related retinal signs, as well as the comprehension of the pathophysiology behind retinal changes could become relevant for physicians to predict the risk of stroke. The aim of this study is to assess the carotid artery disease-induced early structural retinochoroidal changes by means of swept-source optical coherence tomography (SS-OCT). METHODS: A prospective observational study was conducted in 72 eyes with carotid stenosis. According to the degree of stenosis, the participants were divided into a normal group (34 eyes), a mild-moderate stenosis group (22 eyes), a severe stenosis group (16 eyes). SS-OCT and OCTA were performed to scan macular fovea. Central macular thickness (CMT), subfoveal choroidal thickness (SCT) and foveal avascular zona (FAZ) area were the major measurements for our study. RESULTS: CMT was significantly thicker in group 3 when compared to group 2 and 1. SCT was significantly thinner in group 3 vs group 1, being thicker in group 2 when compared to group 1. No significant differences were obtained when comparing FAZ in the superficial and middle capillary plexus although it was significant when comparing the FAZ in the deep capillary plexus between group 1 and 3. CONCLUSION: internal carotid artery stenosis greater than 70% leads to a significant increase in CMT and a decrease in SCT prior the development of clinical findings of ocular ischemia syndrome.

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.

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.

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.

The contribution of indocyanine green angiography in a case of unilateral retinal pigment epithelium dysgenesis.

PURPOSE: To further enhance understanding of the expanded clinical spectrum of unilateral retinal pigment epithelium dysgenesis (URPED) via numerous imaging modalities including novel markers of highly detailed indocyanine green angiography (ICGA) features. METHODS: Retrospective, observational, case report. RESULTS: URPED in this patient is expressed as a solitary, flat and pigmented lesion in the posterior pole with RPE hyperplasia and atrophic changes. An epiretinal membrane (ERM) causing fine, tortuous retinal vessels and retinal folds was observed. Green and blue excitation light fundus autofluorescence showed a biphasic appearance with hypoautofluorescent rounded lesions and a reticular configuration of normal RPE. Fundus fluorescein angiography revealed diffuse hypofluorescence and hyperfluorescent wisps of leakage in late-phases. Early-phase of ICGA evidenced diffuse hypocianescence and a delineated hypercianescent scalloped margin appeared in the late-phase, together with focal hypocianescent spots. SD-OCT demonstrated irregularity of the RPE with fibrosis and hyperplastic changes combined with atrophic areas. Flat RPE detachments intermingled with healthy-appearing RPE were also observed together with thinning of the outer retina. ERM with thickening and disorganization involving the whole retina was present. Optical coherence tomography angiography (14 × 14 mm) revealed an oval shape foveal avascular zone and vascular anomalies such as tortuosity and looping. CONCLUSION: URPED is an extremely rare clinical entity with only a few cases reported. In this case the almost pathognomonic differential features of URPED were best appreciated with ICGA imaging. To our knowledge, this is the first reported case of URPED with these abnormal findings on ICGA meaning it could be part of the spectrum of the disease.

Streptococcus vestibularis atypical infectious keratits after intracorneal ring segment implantation.

PURPOSE: To report the first case of an atypical infectious keratitis caused by Streptococcus vestibularis after ICRS implantation. CASE REPORT: A 65-year-old man underwent intracorneal ring segments (ICRS) implantation for the treatment of a pellucid marginal corneal degeneration (PMCD) in his both eyes (OU). Three weeks after the surgery of the right eye (OD), the patient presented with pain, redness, and vision loss in his OD. BCVA was 20/100 Snellen equivalent in the OD. Slit-lamp examination revealed punctate whitish infiltrates along the ring segment with associated corneal stromal edema in the OD. Corneal scrapings were obtained for gram satins and bacterial and fungal cultures, and topical treatment with hourly ciprofloxacin was started. Two days later, corneal stromal edema worsened with involvement of the ring channel. Thus, the ring segment was removed and sent for cultures, and the ring channel was irrigated with vancomycin. Culture from ring segment came back positive for Streptococcus vestibularis, so topical targeted antiobiotherapy with vancomycin and erythromycin was applied. At 3 weeks of follow-up, BCVA was of 20/40 Snellen equivalent in the OD and a subtle corneal leucoma in the OD was observed in the slit-lamp examination. CONCLUSION: Although infectious keratitis is a rare and serious disorder associated with ICRS implantation, its early recognition and management is essential in order to avoid sight-threatening complications. Thus, a prompt obtainment of corneal curettage samples and removal of ring segment becomes decisive to initiate a targeted antiobiotherapy.

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.

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.