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Vitreoretinal lymphoma (VRL) is a rare B-cell intraocular neoplasia characterized by poor long-term prognosis and lack of effective therapies. It mainly involves the vitreous humor, the retina, and the retinal pigment epithelium (RPE), although anterior segment involvement can occur. VRL is classified as a lymphoma of immune privileged sites, along with testis lymphoma and primary central nervous system lymphoma (PCNSL). VRL and PCNSL are strictly connected indeed: 80% of VRL develop PCNSL, while 20% of patients with PCNSL present VRL during natural history of lymphoma. Due to the lack of worldwide consensus about diagnosis, therapy, and follow-up timing, VRL represents one of the most challenging ocular affections.VRL commonly masquerades as a posterior uveitis, and misdiagnosis often occurs because of partial response to topical steroids. Gold standard for diagnosis is cytological analysis of vitreous humor. However, this technique lacks sensitivity and supplemental molecular analyses can improve the diagnostic process. Multimodal imaging allows ophthalmologists to empower their clinical suspicion and a comprehensive examination can highlight typical features of VRL and justify further invasive procedures.There is no consensus about VRL therapy, and none of the therapeutical scheme has demonstrated to prevent cerebral involvement and improve patient's overall survival. Intravitreal injections of chemotherapeutics drugs, ocular radiation therapy and systemic chemotherapy can be considered in the treatment of VRL. Once cerebral involvement occurs, systemic chemotherapy must be included in the treatment as a life-saving therapy. Further multicentric studies are required to find out the best treatment of patients with VRL.

BackgroundIntermediate and posterior manifestations of vitreoretinal lymphoma (VRL) are well characterised. However, there is limited information on anterior segment involvement in VRL. This study aimed to describe the anterior manifestations of VRL, and their association with molecular testing.MethodsRetrospective analysis of patients with biopsy-proven VRL. Study variables included anterior segment manifestations, findings from slit-lamp photos and in vivo confocal microscopy (IVCM) when available. MYD88 L265P mutation and cytology in the aqueous humour, retinal and systemic findings were also analysed.ResultsThe analysis included 108 eyes of 55 VRL patients. Anterior segment involvement was present in at least one visit in 55 eyes (51%) of 33 patients (60%); it included keratic precipitates (dendritiform with branching and irregular margins in 33 eyes, dust-like in 16 eyes and large granulomatous in 12 eyes), cells in the anterior chamber (51 eyes) and posterior synechiae (2 eyes). IVCM was available for 41 eyes and showed different morphologies of keratic precipitates, including floral, spikes and mulberry patterns (66%, 56% and 20%, respectively). MYD88 L265P mutation in the aqueous humour was detected in 10/21 (48%) eyes with no anterior segment involvement and 24/37 (65%) eyes with anterior segment involvement.ConclusionsAnterior segment manifestations are often present in VRL and include dendritiform and dust-like keratic precipitates. IVCM in VRL can identify different patterns associated with keratic precipitates. MYD88 L265P mutation in the aqueous humour of VRL patients can also be found in eyes without significant anterior segment involvement.

Byline: Aniruddha. Agarwal, Suryaprakash. Sharma, Aman. Kumar, Alessandro. Marchese, Shobha. Sehgal, Manpreet. Singh, Nalini. Gupta, Aman. Sharma, Amod. Gupta, Anita. Agarwal, Vishali. Gupta A 36-year-old male suffered a traumatic open [...]

Byline: Alessandro. Marchese, Francesco. Bandello, Giuseppe. Querques A 90-year-old lady with advanced age-related macular degeneration showed on structural optical coherence tomography (OCT) of the right eye a subretinal aneurysmal lesion [...]

Byline: Parthopratim. Dutta Majumder, Alessandro. Marchese, Francesco. Pichi, Itika. Garg, Aniruddha. Agarwal Autoimmune retinopathy (AIR) refers to a group of rare autoimmune retinal degenerative diseases presumably caused by cross-reactivity of [...]

Supplemental Digital Content is Available in the Text.Patients with myopic macular neovascularization do not maintain visual gains, mainly because of recurrences. Elderly patients and those with large neovascular lesions proximal to the fovea have an increased risk of recurrences.

To identify the risk factors associated with myopic macular neovascularization (mMNV)–related complications in patients treated with intravitreal anti-VEGF agents.

Supplemental Digital Content is Available in the Text.A punctate inner choroidopathy–like reaction may occur in patients with apparently unrelated chorioretinal diseases. Our study expands the spectrum of reactive, inflammatory lesions presumably promoted by loss of the outer retinal immune privilege in pre-existent chorioretinal disorders.

Supplemental Digital Content is Available in the Text.In this longitudinal study, patients with acute leukemia in the active phase had reduced vessel density, increased capillary diameter, and lower fractal dimension on optical coherence tomography angiography than healthy eyes, which correlated with bone marrow function failure. The retinal perfusion improved with disease remission.

Supplemental Digital Content is Available in the Text.Patients with posterior uveitis underwent concurrent MultiColor confocal scanning laser ophthalmoscope imaging and conventional color fundus photography. The ability of MultiColor imaging in detecting vitreoretinal surface abnormalities, retinal fluid, retinal hemorrhages, disease activity, and depth/location of lesions was studied compared with color fundus photography.

This study revealed the frequency, risk factors, and functional prognosis of chorioretinal atrophy associated with vitreoretinal lymphoma.

Supplemental Digital Content is Available in the Text. Retinal pigment epithelium tears secondary to macular neovascular membrane may enlarge over time. Enlarging retinal pigment epithelium dehiscence is characterized by sharply demarcated borders and absence of perilesional hyperautofluorescence. The presence of retinal pigment epithelium tear–associated repair proliferation is associated with slower rates of retinal pigment epithelium dehiscence expansion.

Supplemental Digital Content is Available in the Text.Cataract extraction during vitrectomy and the presence of outer retinal abnormalities at baseline are associated with higher odds of external limiting membrane/ellipsoid zone defects after vitrectomy. The rate of functional improvement varies as a function of the baseline visual acuity and severity of retinal layers' distortion before surgery.

Purpose:The aim of this study was to report the presentation, treatment, and outcomes of vitreoretinal lymphoma (VRL) associated with hemorrhagic mass-like lesions (HMLs) in the retina. Methods:This study was a retrospective analysis of patients with HMLs associated with VRL seen at a single tertiary referral center. For each patient, the clinical charts, the fundus imaging, and the treatment outcomes were reviewed. Results:Three eyes of 2 patients had VRL with HMLs. In all study eyes, HMLs were preceded by an area of retinitis-like retinal infiltration and evolved into elevated hemorrhagic masses. Two eyes had multiple relapses with HMLs. All HMLs regressed with treatment and were replaced by extensive chorioretinal atrophy. Conclusion:VRL can present with HMLs. HMLs seem to correspond to massive intraretinal infiltration by VRL, mimicking a solid mass. Despite response to therapy, HMLs are associated with poor anatomical and functional outcomes.

Placoid lesions of the retina may be secondary to a wide spectrum of acquired inflammatory conditions that have been reported as single entities with different presentation and clinical course. These conditions include acute posterior multifocal placoid pigment epitheliopathy, persistent placoid maculopathy, serpiginous choroiditis, serpiginous-like choroiditis, relentless placoid chorioretinitis and acute syphilitic posterior placoid chorioretinitis. In this article, we will group these conditions under the name of ‘placoids’. The recognition of the specific condition may be challenging in clinical practice, often resulting in diagnostic and therapeutic delay. Given the complex nature of placoids and their similarities, a systematic approach including differentiating between infectious and non-infectious aetiologies increases the chance of reaching the correct diagnosis. Detailed history and comprehensive clinical examination are the first steps to formulate a diagnostic hypothesis that should be corroborated by multimodal imaging and appropriate investigations. The advent of multimodal imaging has made it possible to extensively study placoids and revealed a constellation of specific findings that may help clinicians in the diagnostic process. The treatment of the conditions other than syphilis is complex and sometimes challenging. Our article is aimed at giving an overview of the individual entities associated with placoids and discussing the differential diagnosis. A practical and systematic approach is then proposed.

Introduction: Tuberous sclerosis complex (TSC) is a rare hereditary phakomatosis. The clinical features can include benign growths in the central nervous system, tumors of various visceral organs, and retinal or optic disc astrocytic hamartomas in the nerve fiber layer. Here we present the case of a child with known TSC developing Coats-like manifestations.Case description: A 22-month-old girl with known TSC and retinal hamartoma followed since birth presented for the development of exotropia and leukocoria in the left eye. Fundus examination of the left eye showed blurred optic disc, macular star, and yellow retinal exudation in the temporal area. In addition, the left eye showed marked retinal vascular tortuosity and telangiectasias. The patient underwent brain and orbit magnetic resonance imaging, revealing heterotopic gray matter nodulations along ependyma of both lateral ventricles, with partial calcification, and a posterior flattening of the left eye.Conclusion: This report shows a rare case of Coats-like disease in a child with tuberous sclerosis. In case of presence of Coats’ manifestations associated with atypical retinal or systemic findings, genetic diseases should be considered in the differential diagnosis.

We report a case of ocular drug toxicity consistent with bilateral Vogt-Koyanagi-Harada (VKH) like disease in a patient with cutaneous melanoma treated with Dabrafenib/Trametinib therapy. A 53-year-old man with a history of metastatic cutaneous melanoma, treated with Dabrafenib/Trametinib, developed a severe acute panuveitis with granulomatous anterior uveitis and multiple serous retinal detachments. The ocular inflammatory reaction was classified as a bilateral Vogt-Koyanagi-Harada disease. Intraocular inflammation resolved after discontinuation of chemotherapeutic agents and aggressive topical and systemic corticosteroid therapy. The present case outlines the importance of recognizing VKH-like syndrome as a possible consequence of therapy with dabrafenib and trametinib.

This article describes choroidal localizations in immunoglobulin G4–related disease. Both systemic and fundus characteristics suggested the suspect of systemic and intraocular lymphoma. Multimodal imaging was an important tool to make the right diagnosis showing areas of bilateral choroiditis at indocyanine green angiography and roundish lesions at structural optical coherence tomography scans.

Choroidal neovascularization is an extremely rare complication of syphilitic chorioretinitis. Herein, we illustrate the importance of multimodal imaging, particularly optical coherence tomography angiography, to detect the presence of choroidal neovascularization associated with syphilitic chorioretinitis. We also illustrate the clinical course after treatment.

Objectives: To investigate the risk of developing central nervous system (CNS) lymphoma in patients with vitreoretinal lymphoma (VRL) presenting with unilateral versus (vs.) bilateral ocular involvement. Methods: Retrospective, multicentre cohort study from January 1, 1984 to December 31, 2020. Results: There were 218 eyes of 127 patients with isolated VRL of the confirmed or presumed diffuse large B-cell subtype in the absence of known CNS or systemic lymphoma. Overall, mean patient age at presentation was 67 years (median 68, range 22–93 years), with 52 (40%) male, and 118 (90%) Caucasian. By univariate Cox regression analysis, two factors were predictive of decreased risk for development of CNS lymphoma, including initial presentation with unilateral VRL (versus bilateral VRL) (HR 0.5 [0.2–0.9], p= 0.02) and use of systemic chemotherapy for initial treatment of isolated ocular disease (HR 0.2 [0.1–0.6], p= 0.002). Both factors remained significant on multivariate and competing risk analyses. Progression from unilateral to bilateral VRL, patient age at presentation, and ocular structures involved (vitreous, subretinal space, subretinal pigment epithelial space) were not significantly associated with CNS lymphoma risk. Conclusion: Initial presentation with unilateral VRL and treatment of isolated VRL with systemic chemotherapy were associated with lower risk of developing CNS lymphoma. Further study is required to determine whether select patients with isolated VRL might benefit from systemic chemotherapy in the prevention of CNS lymphoma.

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.

Purpose To investigate the effect and the safety of intravitreal aflibercept in patients affected by choroidal neovascularization secondary to angioid streaks with a long-term follow-up.Methods Multicentre, open-label, phase IIb study (EYLEA-STRIE, EudraCT Number 2014-000986-30) involving four Italian centres (IRCCS Ospedale San Raffaele (Milano), Fondazione G.B. Bietti (Roma), Policlinico (Milano), Ospedale Luigi Sacco (Milano)). Patients with active choroidal neovascularization secondary to angioid streaks with foveal involvement were prospectively enrolled and followed for 18 months. All the patients received intravitreal 2 mg/0.05 mL aflibercept at the time of enrolment, followed by a pro-re-nataregimen for 48 weeks. Best-corrected visual acuity and central macular thickness were measured monthly. Adverse events were monitored at each visit.Results Twenty-three eyes of 20 patients were analysed. Mean number of injections per patient was 4.30 ± 1.2. At week 48, the best-corrected visual acuity was 0.42 ± 0.40 LogMAR (p = 0.6 from baseline) and 18 eyes (81.8%) featured stability within 15 letters. The central macular thickness significantly reduced (p = 0.03). Eleven ocular non-serious adverse events and two serious adverse events were observed (one case of endophthalmitis and one case of acute gastritis were reported).Conclusion Intravitreal aflibercept represents a valid option for the management of choroidal neovascularization complicating angioid streaks. Further studies with longer follow-up and different therapeutic regimens are warranted to ascertain the best control of the disease.

Introduction: To investigate choroidal thickness changes related to the clinical activity of inflammatory choroidal neovascularization in punctate inner choroidopathy/multifocal choroiditis as compared to myopic choroidal neovascularization.Materials and methods: Consecutive inflammatory choroidal neovascularization secondary to punctate inner choroidopathy/multifocal choroiditis, and myopic choroidal neovascularization were retrospectively reviewed. By means of enhanced-depth imaging optical coherence tomography, choroidal thickness was assessed at the same location before choroidal neovascularization development, at choroidal neovascularization onset (baseline), and after treatment.Results: Eleven eyes with inflammatory choroidal neovascularization and 11 eyes with myopic choroidal neovascularization were analyzed. Choroidal thickness beneath inflammatory choroidal neovascularization significantly increased at baseline and decreased after therapy (“Sponge sign”), reaching preclinical values. In particular, mean choroidal thickness under inflammatory choroidal neovascularization was 145 ± 85 µm at the preclinical stage, increased to 210 ± 103 µm at baseline (p = 0.006), and decreased to 136 ± 87 µm after treatment (p = 0.017). Conversely, no significant choroidal thickness changes were disclosed in myopic choroidal neovascularization eyes, under any location.Conclusion: Optical coherence tomography–based choroidal thickness evaluation may represent an additional useful tool to monitor inflammatory choroidal neovascularization activity. Moreover, choroidal thickness under choroidal neovascularizations could be used to discriminate the origin of choroidal neovascular membrane, either inflammatory or myopic, in doubtful cases and guide the therapeutic management.

Objectives: To inspect the inter-reader agreement of different diagnostic modalities in identifying choroidal neovascularization (CNV) activity secondary to angioid streaks (AS) and to analyze the prevalence of subretinal hyper-reflective material (SHRM) in active CNV. Methods: Retrospective study of patients with AS with active CNV; optical coherence tomography (OCT), OCT angiography (OCTA), fundus fluorescein angiography (FFA), and indocyanine green angiography (ICGA) from each patient were collected. Agreement between two readers using different diagnostic modalities is presented as free-marginal kappa (k) and 95% confidence interval (CI). Results: This study included 19 eyes of 12 patients with active CNV (5 naive and 14 previously treated). Agreement among readers on CNV activity was excellent for OCT (k=0.88; 95% CI 0.71–1.00), good for FFA (k= 0.70; 95% CI 0.46–0.94) and ICGA (k= 0.58; 95% CI 0.31–0.84), and poor using OCTA (k= 0.39; 95% CI 0.11–0.68). SHRM was the most common OCT finding associated with active CNV (100%); fuzzy borders were present in 53% of SHRM cases at baseline. Conclusions: Identification of CNV activity in AS is challenging; OCT was the best modality to inspect active CNV. The identification of SHRM contributed to recognizing active CNV. Further studies are needed to assess the role of SHRM in anticipating prognosis and guiding treatment of CNV secondary to AS.

The authors report a pseudo-vitelliform subretinal lesion associated with multiple retinal pigment epithelium apertures in vitreoretinal lymphoma; the case might represent a sign of macular infiltration, a paraneoplastic maculopathy, or coexistence of both. Retinal pigment epithelium apertures are a novel finding in vitreoretinal lymphoma.

Purpose: To evaluate the efficacy and the safety of curcumin-phosphatidylcholine complex in children affected by juvenile idiopathic arthritis–associated uveitis as an adjunctive treatment to chronic systemic immunosuppressive therapy.Methods: In this retrospective, longitudinal study, we treated patients affected by juvenile idiopathic arthritis–associated uveitis with residual low-grade inflammatory activity in the anterior chamber with one tablet of curcumin-phosphatidylcholine complex per day, over a year. Low-grade inflammatory activity was characterized by flare 1+ at slit-lamp examination and 10–50 photon counts per ms) at the FC500 laser flare meter. Inactivity of uveitis was defined as complete disappearance of flare at the slit-lamp examination and values <10 ph/ms at laser flare meter. Conversely, recurrence of the uveitis was defined as a one-step increase from baseline in anterior chamber cells levels or laser flare meter measurements >50 ph/ms.Results: A total of 22 out of 27 patients (81%) achieved inactivity at the end of the study. Five patients (19%) did not show a significant reduction in anterior chamber flare, remaining stable throughout the follow-up. Only three episodes of flare-ups in three different patients were recorded. Overall, the treatment was well tolerated by all patients and no ocular discomfort, ocular side effects, or allergic reactions were registered.Conclusion: Adjunctive therapy with curcumin in patients affected by juvenile idiopathic arthritis–associated uveitis improves mild chronic anterior chamber flare and presents a good safety profile. Despite being mild, anterior chamber inflammation should be minimized to avoid the development of sight-threatening complications in these patients.

The aim is to present the changes in ultra-widefield and widefield multimodal imaging, including optical coherence tomography angiography of a 33-year-old woman diagnosed with Susac syndrome, over 1 year of follow-up. Fundus examination and multimodal imaging revealed bilateral arterial occlusion of multiple vascular branches with retinal ischemia. Over 1 year follow-up, best-corrected visual acuity improved while retinal ischemia gradually resolved. Widefield optical coherence tomography angiography showed reperfusion of macular large vessels, but not of the small capillaries. Despite anatomical improvement, functional defects of the visual field persisted. In conclusion, widefield and ultra-widefield imaging provided high-resolution details of the central and peripheral damages in Susac syndrome.

The primary aim of this study was to summarize and illustrate the main structural cross-sectional optical coherence tomography findings encountered after vitreoretinal surgery for rhegmatogenous retinal detachment. This was a non-systematic review of literature on structural cross-sectional optical coherence tomography findings after vitreoretinal surgery for rhegmatogenous retinal detachment. Adequate illustrations of the main findings described were found after a retrospective analysis of imaging and charts of patients operated at the department where this study was performed. The main structural cross-sectional optical coherence tomography findings after vitreoretinal surgery for rhegmatogenous retinal detachment included persistent subretinal fluid, subretinal blebs, retinal folds, subretinal perfluorocarbon liquids, macular alterations related to silicone oil, epiretinal membranes, proliferative vitreoretinopathy, cystoid macular edema, macular holes, and recurrent retinal detachment. In conclusion, optical coherence tomography was a useful tool after vitreoretinal surgery for rhegmatogenous retinal detachment. Some optical coherence tomography findings may not be evident on fundus examination, and optical coherence tomography can reveal essential details for the clinical management and the visual prognosis. Other findings, despite being visible on funduscopic examination, may be better assessed with the aid of optical coherence tomography. All these elements contribute to support the importance of tomographic assessment in the follow-up of eyes treated for vitreoretinal conditions.

This study was aimed at reporting a set of complications associated with retinal pigment epithelium (RPE) humps in high myopia. Data included three eyes from three different patients. Complications observed over RPE humps were the development of choroidal neovascularization, active inflammatory lesions of multifocal choroiditis, and simple bleeding. Regular follow-up with appropriate examinations can help to recognize these events and offer the most adequate treatment in a timely manner.[[Ophthalmic Surg Lasers Imaging Retina. 2020;51:119–123.]

Purpose: To evaluate the efficacy and safety of needle revision and examine factors predictive of failure.Methods: In total, 157 eyes of 131 patients that underwent needle revision augmented with either 5-fluorouracil or betamethasone for trabeculectomy failure were included in this retrospective study. Complete failure was defined as additional glaucoma surgery, ciliodestructive procedures, loss of light perception, sight-threatening complications, hypotony maculopathy, and surgical bleb revision. Success was defined as intraocular pressure ⩽ 18 (criterion A), ⩽15 (criterion B), and ⩽12 mmHg (criterion C) reached with (qualified) or without (complete) medications, and absence of any criteria of complete failure.Results: The median (interquartile range) follow-up was 25.0 (41.0) months. Complete failure rates were 19%, 26%, and 31% at 1, 2, and 3 years, respectively. For criterion A, qualified and complete success rates were, respectively, 77% and 69% at 1 year, 66% and 51% at 2 years, and 60% and 47% at 3 years. For criterion B, qualified and complete success rates were, respectively, 67% and 61% at 1 year, 48% and 42% at 2 years, and 44% and 39% at 3 years. For criterion C, qualified and complete success rates were, respectively, 43% and 41% at 1 year, 27% and 25% at 2 years, and 24% and 23% at 3 years. High baseline intraocular pressure and primary surgery were associated with higher and lower risks of complete failure, respectively.Conclusion: Needle revision is an effective and safe procedure to rescue failing trabeculectomy postponing or avoiding further glaucoma surgery. Eyes with low target intraocular pressure may have poor long-term outcomes.

Choroidal melanoma (CM) is the most commonly diagnosed primary intraocular malignancy in adults. Gamma knife radiosurgery (GKR) has demonstrated reliable results in the treatment of CM, but it is limited by the risk of radiation-induced ocular complications. To optimize the dose of radiation focused on the tumor, and limit side effects, the eye should be properly immobilized before treatment. A recently released ultra-widefield imaging instrument (Optomap California; Optos, Dunfermline, Scotland, UK) allows for an interactive three-dimensional (3D) virtual reconstruction of the globe, called 3D WrapTM. The authors share their experience with this rapid, reliable, and relatively easy support in planning globe positioning before GKR treatment.

BackgroundThe 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.MethodsThe 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.ResultsSeventy 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.ConclusionsThe 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.

Aims: To assess hyperreflective foci (HF) number in angioid streaks (AS) by means of spectral-domain optical coherence tomography (SD-OCT). Methods: Observational and cross-sectional study. Sixty-two eyes with AS and 62 controls underwent best-corrected visual acuity (BCVA), fundoscopy, and SD-OCT. HF were assessed on the horizontal scan of a six-line radial OCT, in the fovea (1500-μm diameter) and parafovea (500 μm external to the fovea), and sub-classified as retinal or choroidal, small or large. Eyes were distributed in one of four groups, as carrying foveal AS without choroidal neovascularization (CNV) (14 eyes), extra-foveal AS without CNV (14), active CNV (20), and inactive CNV (14). Primary outcome was HF assessment in AS. Secondary outcomes included their correlations with BCVA. Results: AS-affected eyes had higher HF numbers. Our sub-analysis revealed that patients with active CNV had a larger number of retinal and choroidal HF than all the other groups, whereas retinal and choroidal foci were significantly increased in inactive CNV only with respect to controls. Interestingly, patients with foveal AS showed HF number increase in the choroid and fovea. BCVA deterioration positively correlated with the total HF number found in the fovea and the choroid. Conclusion: HF are significantly increased in patients with AS. Despite being especially evident in active CNV, the increasing number in eyes without CNV might suggest new pathogenetic aspects of the disease.

AimsTo investigate the clinical spectrum of choroidal neovascularisation (CNV) in patients with dome-shaped macula with the aid of multimodal imaging.MethodsIn this cross-sectional, retrospective, observational study, we reviewed charts and imaging studies of 144 eyes of 79 consecutive patients with dome-shaped macula evaluated at a tertiary referral centre, including optical coherence tomography angiography (OCTA), fluorescein angiography (FA) and indocyanine green angiography (ICGA). Three masked graders evaluated the prevalence and type of CNV. Primary outcome measures were the diagnostic agreement between OCTA and FA±ICGA, the multimodal imaging characteristics and prevalence of CNV associated with dome-shaped macula.ResultsWe identified 30 eyes with dome-shaped macula complicated by CNV with an estimated prevalence of 20.8% (95% CI 14.2 to 27.4). Twenty-two eyes (73%) showed a type 2 CNV while eight eyes (27%) showed a type 1 CNV. Serous macular detachment was present in 39 eyes (27%) and harboured a CNV in five cases (13%). The overall diagnostic agreement between OCTA and FA±ICGA was excellent (k=0.894, p<0.001), but only the use of both techniques allowed the identification of the whole pool of CNVs.ConclusionEyes with dome-shaped macula may either develop typical myopic CNV (ie, type 2 CNV) or pachychoroid-associated CNV (ie, type 1 CNV), similar to those observed in central serous chorioretinopathy. Accordingly, in case of serous macular detachment complicating dome-shaped macula, the presence of an associated CNV should be considered. When suspecting a CNV associated with dome-shaped macula, the application of both OCTA and FA±ICGA is superior to the use of one technique alone.

AimsPeculiar retinal signs of vitreoretinal lymphoma (VRL) have been identified. However, limited information on the vitreous features of VRL is available. This study aims to characterise the vitreous involvement in VRL with the help of multimodal imaging.MethodsIn this retrospective, observational study, we reviewed charts and imaging of all patients with biopsy-proven VRL seen from January 2016 to April 2018 at a single referral centre. These included ultrawide-field imaging, ophthalmic ultrasonography and slit-lamp photography. The main outcome measures were patterns of vitreous haze of VRL, as observed by combining clinical and multimodal imaging information.ResultsTwenty-six eyes of 13 patients were included. At presentation, vitreous haze was present in 24 eyes (92%) and was the only sign of VRL in 4 eyes (15%). Three patterns of vitreous haze were identified in VRL. An aurora borealis pattern was present in 12 eyes and showed linear opacities with a myriad of cells aligned along the vitreous fibrils. A string of pearls pattern was present in two eyes at baseline and developed in other four eyes after vitrectomy, showing fine fibrils connecting bunches of inflammatory material. A non-specific pattern was observed in 10 eyes. Ophthalmic ultrasound showed corpuscular material correlating with the grading of vitreous haze.ConclusionVRL shows different patterns of vitreous haze. Multimodal imaging, including ultrawide-field imaging and slit-lamp photography, helps in recognising these patterns, raising suspicion for VRL.

The authors report a case of unilateral curvilinear streaks occurring in a patient with pseudoxanthoma elasticum. These represent a previously unreported finding in this disease, as usually found in inflammatory or infective conditions.[[Ophthalmic Surg Lasers Imaging Retina. 2018;49:e292–e295.]

The aim of this study was to analyze retrospectively optical coherence tomography angiography features of eyes affected by angioid streaks with and without choroidal neovascularization and to propose a new classification criterion.

To describe the evolution of treatment-naïve quiescent choroidal neovascularizations (CNVs) in age-related macular degeneration (AMD) by means of OCT angiography (OCTA).

This study aimed to assess the prevalence and clinical manifestations of ocular graft-versus-host disease (GVHD) after allogeneic hematopoietic stem cell transplantation (allo-HSCT).

Evaluation of foveal avascular zone measurement by means of optical coherence tomography angiography revealed high reproducibility and reliability both under basal condition and in different experimental settings after vasoactive stimuli. Automated measurements for nonflow areas provided significantly larger diameters than manual.

To estimate the prevalence of treatment-naïve quiescent choroidal neovascularization (CNV) among eyes with pachychoroid neovasculopathy and to investigate the OCT angiography (OCT-A) features.

Purpose To report a case of sudden decrease in visual acuity possibly due to a cardiogenic embolism in a patient who underwent cardiac resynchronization therapy (CRT) device implantation.Methods A 62-year-old man with severe left ventricular systolic dysfunction and a left bundle branch block was referred to our department because of a sudden decrease in visual acuity. Nine days earlier, he had undergone cardiac transapical implantation of a CRT device, which was followed, 2 days later, by an inflammatory reaction. The patient underwent several general and ophthalmologic examinations, including multimodal imaging.Results At presentation, right eye (RE) best-corrected visual acuity (BCVA) was counting fingers and RE pupil was hyporeactive. Fundus examination revealed white-centered hemorrhagic dots suggestive of Roth spots. Fluorescein angiography showed delay in vascular perfusion during early stage, late hyperfluorescence of the macula and optic disk, and peripheral perivascular leakage. The first visual field test showed complete loss of vision RE and a normal left eye. Due to suspected giant cell arteritis, temporal artery biopsy was performed. Thirty minutes after the procedure, an ischemic stroke with right hemisyndrome and aphasia occurred. The RE BCVA worsened to hands motion. Four months later, RE BCVA did not improve, despite improvement in fluorescein angiography inflammatory sign.Conclusions We report a possible cardiogenic embolism secondary to undiagnosed infective endocarditis causing monocular visual loss after CRT device implantation. It remains unclear how the embolus caused severe functional damage without altering the retinal anatomical structure.

To investigate the application of noninvasive ultra-widefield (UWF) imaging in patients with angioid streaks secondary to pseudoxanthoma elasticum (PXE) and to compare detected findings with those obtainable with 7 standard 30° fields (7SF) imaging.

AimsTo describe the vascular changes in patients affected by non-neovascular age-related macular degeneration (AMD), featuring reticular pseudodrusen (RPD), drusen, or both RPD and drusen by means of optical coherence tomography angiography (OCT-A).MethodsCross-sectional observational case series. Patients with non-neovascular AMD presenting at the Medical Retina Service of the Department of Ophthalmology, University Vita-Salute San Raffaele in Milan were recruited. Patients underwent best-corrected visual acuity, biomicroscopy, infrared reflectance, short-wavelength fundus autofluorescence and OCT-A (AngioPlex, CIRRUS HD-OCT 5000, Carl Zeiss Meditech, Dublin, USA). Main outcome was quantification of vessel density, stromal tissue, and vascular/stromal (V/S) ratio at the choriocapillaris (CC), the Sattler and Haller's and the whole choroid layers among different groups of patients with non-neovascular AMD by means of binarised OCT-A scans.Results45 eyes of 34 patients were enrolled (15 eyes of 11 patients with RPD, group 1; 15 eyes of 11 patients with drusen, group 2; 15 eyes of 12 patients with mixed phenotype, group 3). The CC, the Sattler and Haller's and the whole choroid vessel density were reduced in all groups of patients (p=0.023, p=0.007 and p=0.011 in group 1, group 2 and group 3 for the CC; p=0.021, p=0.037 and p=0.043 in group 1, group 2 and group 3 for the Sattler and Haller's density; p=0.016, p=0.002 and p<0.001 in group 1, group 2 and group 3 for the choroidal density), with significantly lower V/S ratios compared with healthy controls.ConclusionsPatients with non-neovascular AMD show significant choroidal vascular depletion and fibrotic replacement, suggesting a possible role in the pathogenesis and progression of the disease.

Purpose To report a case of juxtapapillary polypoidal choroidal neovascularization (PCNV) associated with choroidal nevus investigated by means of optical coherence tomography angiography (OCT-A).Methods Case report.Results A 72-year-old woman presented with visual loss and metamorphopsia in her left eye for 5 days secondary to PCNV that developed on the border of a juxtapapillary choroidal nevus. Fluorescein angiography, indocyanine green angiography, and spectral-domain OCT confirmed the diagnosis. En face OCT-A disclosed a large tangled hyperreflective PCNV spreading from the optic disc at different levels of the choriocapillaris; the polyp lumina appeared hyporeflective. The patient was treated with 3 intravitreal injections of anti-vascular endothelial growth factor with partial functional recovery.Conclusions Our case showed the application of OCT-A in the diagnosis of a case of active PCNV complicating a benign intraocular tumor.