Comparison of subretinal aflibercept vs ranibizumab vs bevacizumab in the context of PPV, pneumatic displacement with subretinal air and subretinal tPA in naïve submacular haemorrhage secondary to nAMD. "The Submarine Study".

OBJECTIVE: To compare efficacy and safety profile of subretinal aflibercept, ranibizumab, and bevacizumab in the context of pars plana vitrectomy, pneumatic displacement with subretinal air and subretinal tPA for subretinal macular haemorrhage (SMH) due to naïve neovascular age-related macular degeneration (nAMD). DESIGN: Retrospective interventional cohort study. PARTICIPANTS: 123 eyes of 123 patients treated with subretinal aflibercept (n = 41, 33%), ranibizumab (n = 41,33%), and bevacizumab (n = 41, 33%). METHODS: Review of electronic medical records for best corrected visual acuity (BCVA), central subfoveal thickness (CST), and intraocular pressure (IOP) at baseline and 24 months after treatment. MAIN OUTCOME MEASURES: BCVA, CST, and number of intravitreal anti VEGF over 24 months. RESULTS: Mean age of patients was 80.5 ± 5.5 years, 43.9% were female. Mean time from symptom onset until surgery was 1.1 days (range 0-3 days). In all cases, the SMH did not reach the arcades. CST at baseline was 627 ± 140 µ, 739 ± 54 µ, and 793 ± 93 µ (p = 0.0001) for aflibercept, ranibizumab, or bevacizumab, respectively. Baseline BCVA (logMAR) was 0.65 ± 0.13, 0.69 ± 0.96, and 0.74 ± 0.81 (p = 0.0041) for aflibercept, ranibizumab, and bevacizumab, respectively. All three groups showed statistically significant improvement in BCVA and CST (for all groups: p < 0.001). There was no statistically significant difference at the final BCVA (p = 0.789). The mean number of anti VEGF given during follow-up period was 5.2 ± 0.81, 4.4 ± 0.63, and 5.5 ± 0.95 (p = 0.0001) for aflibercept, ranibizumab, and bevacizumab, respectively. CONCLUSION: This study shows that aflibercept, ranibizumab, and bevacizumab in a subretinal manner in the context of PPV, pneumatic displacement with subretinal air and subretinal tPA for subretinal macular haemorrhage secondary to naïve nAMD work with the same efficacy and safety profile.

POLYPOIDAL LESIONS ASSOCIATED WITH CHOROIDAL NEVI.

PURPOSE: To investigate eyes with polypoidal lesions associated with choroidal nevi, their multimodal imaging characteristics, and long clinical follow-up. METHODS: Multicenter, retrospective case series study of patients with polypoidal lesions overlying choroidal nevi. Demographic and clinical information were recorded. Multimodal imaging including color fundus photography, optical coherence tomography, optical coherence tomography angiography, fundus fluorescein angiography, indocyanine angiography, and A- and B-scan ultrasonography were analyzed for nevus and polypoidal lesion characteristics. RESULTS: Fourteen eyes (14 patients; mean age: 70.3 ± 6.7 years) with polypoidal lesions overlying choroidal nevi were included. The mean follow-up duration was 50.0 ± 27.9 months (range 12-108). All nevi were pigmented on color fundus photography, flat on ultrasonography with a mean basal diameter of 3.8 ± 0.4 mm. In all but one eye, optical coherence tomography showed a shallow irregular pigment epithelium detachment overlying the nevus. A total of 11/14 eyes (78.6%) had exudative activity, 9 eyes received intravitreal anti-vascular endothelial growth factor injections, and one eye required intravitreal anti-vascular endothelial growth factor combined with photodynamic therapy. Mean visual acuity was 20/32 at baseline and 20/50 at final visit. CONCLUSION: We present the largest known cohort of eyes with polypoidal lesions associated with choroidal nevi with up to 9 years follow-up. The exudative degree of the polypoidal lesion in this condition is variable and treatment decisions should be taken on an individual basis. We hypothesize that choroidal ischemia because of altered choroidal vasculature rather than Haller layer hyperpermeability plays a role in the formation of polypoidal lesions overlying nevi.

Vitrectomized vs non-vitrectomized eyes in DEX implant treatment for DMO-Is there any difference? the VITDEX study.

OBJECTIVE: We aimed to compare visual and anatomical outcome in vitrectomized and non-vitrectomized eyes treated with dexamethasone (DEX) implant due to diabetic macular oedema (DMO). DESIGN: Multicenter, retrospective, interventional study. PARTICIPANTS: 236 eyes from 234 patients with DMO with or without previous vitrectomy performed with follow-up of 12 months. METHODS: Records were reviewed for cases of DMO treated with DEX implant in vitrectomized and not vitrectomized eyes. Best corrected visual acuity (BCVA), central subfoveal thickness (CST), and intraocular pressure (IOP) were recorded at baseline and 12 months after treatment with DEX implants. Correlations between vitreous status and visual and anatomical outcome, as well as safety profile were analysed. MAIN OUTCOME MEASURES: BCVA and CST over follow-up period. SECONDARY OUTCOMES: cataract rate formation, intraocular pressure increase, number of implants needed. RESULTS: The non-vitrectomized group included 130 eyes (55.1%), the vitrectomized group included 106 eyes (44.9%). The groups were well balanced for age and gender (p = 0.540, and p = 0.053, respectively). Both groups showed statistically significant improvement in BCVA and CST (for all groups: p < 0.001). There was no significant difference between the groups in terms of change in vision (p = 0.89) and anatomy (p = 0.65). The mean number of DEX implants given during follow-up was 3.5 in both groups, and there was no significant difference between the groups (p = 0.81). CONCLUSION: We demonstrated similar anatomical and functional efficacy of DEX implant in non-vitrectomized and vitrectomized eyes. Its efficacy was not influenced by full vitrectomy for diabetic retinopathy complications. Safety profile was well balanced between groups.

Risk factors and clinical significance of prechoroidal cleft in eyes with neovascular age-related macular degeneration in Caucasian patients.

OBJECTIVE: The objective of the study was to investigate the prevalence, risk factors and clinical significance of prechoroidal cleft (PC) among neovascular age-related macular degeneration (nAMD) patients in a Caucasian population. DESIGN: A retrospective observational cohort study. METHODS: A total of 140 patients with naive nAMD were treated with anti-vascular endothelial growth factor (VEGF) injections and a follow-up of ≥24 months. Optical coherence tomography (OCT) scans were graded for the presence of PC, central subfield foveal thickness (CSFT), maximal retinal thickness (MRT), pigment epithelial detachment (PED), presence of intraretinal fluid (IRF), subretinal fluid (SRF) and subretinal hyper-reflective material (SHRM) at baseline, 3, 6, 12 and 24 months. Best corrected visual acuity (BCVA) and anti-VEGF treatments were recorded. RESULTS: Out of 140, 21 eyes (15%) developed PC. BCVA improved significantly from 0.68 ± 0.56 to 0.62 ± 0.59 logMAR after 24 months (p = 0.008). The change in BCVA was not related to the presence of cleft (p = 0.208). Multivariate analysis confirmed that higher baseline CSFT (p = 0.011, OR = 1.004, 95%, CI 1.001-1.007) and the presence of multi-layered PED (p < 0.001, OR = 21.153, 95%, CI 5.591-80.026) were both predictive for development of PC. Eyes with PC received more injections than eyes without PC. CONCLUSION: Prechoroidal cleft was found in 15% of Caucasian nAMD patients treated with anti-VEGF injections and was related to greater retinal and PED height, as well as presence of multi-layered PED. Eyes with PC required more anti-VEGF injections. The presence of PC correlates with disease activity, and intensive anti-VEGF suppression can preserve vision.

Naïve subretinal haemorrhage due to neovascular age-related macular degeneration. pneumatic displacement, subretinal air, and tissue plasminogen activator: subretinal vs intravitreal aflibercept-the native study.

OBJECTIVE: We aimed to compare visual and anatomical outcome in subretinal aflibercept vs. intravitreal aflibercept in the context of Pars Plana Vitrectomy (PPV), pneumatic displacement with subretinal air and subretinal tPA in patients with naïve submacular haemorrhage (SMH) secondary to neovascular age-related macular degeneration (nAMD). DESIGN: Retrospective interventional cohort study. PARTICIPANTS: 80 patients treated with subretinal aflibercept vs. intravitreal aflibercept in the context of PPV, subretinal air and subretinal tPA in patients with SMH secondary to naïve nAMD. METHODS: Records were reviewed. Best corrected visual acuity (BCVA), central subfoveal thickness (CST), and intraocular pressure (IOP) were recorded at baseline and 24 months after treatment. MAIN OUTCOME MEASURES: BCVA, CST, and number of anti VEGF treatment over follow-up period. RESULTS: The average duration from onset of symptoms to surgery was 1.26 days (range 0-3 days). Based on review of OCT images, SMH was subretinal in all 80 patients (100%), and sub-RPE in 29 patients (36.3%). Forty-one patients (51.25%) were treated with subretinal aflibercept ("subretinal group"), and 39 patients (48.75%) were treated with intravitreal aflibercept injections ("intravitreal group"). The groups were well balanced for age and gender p = 0.6588, and p = 0.263, respectively). Both groups showed statistically significant improvement in BCVA and CST (for all groups: p < 0.001). The mean number of anti VEGF given during follow-up period was statistically significantly lower in the "subretinal group" (p < 0.0001). CONCLUSION: This study shows better management of the CNV, with a statistically significant lower need for anti-VEGF injections when treated with subretinal aflibercept compared to intravitreal application.

Half-Dose Photodynamic Therapy as a Novel Treatment Protocol for Circumscribed Choroidal Hemangioma.

We present a case series of four patients with circumscribed choroidal hemangioma (CCH) treated with half-dose PDT, proposing this as a novel treatment protocol. Four patients with CCH were included, and then evaluated using multimodal imaging, including optical coherence tomography (OCT), fluorescein angiography, indocyanine green angiography, fundus autofluorescence, and ultrasound following treatment with half-dose and full-fluence PDT. Following half-dose PDT, all patients showed significant shrinkage of the hemangioma, functional improvement, and decrease of intra- and sub-retinal fluid. All patients remained stable after a single PDT treatment, with a follow-up of up to 60 months. No side effects were shown. This is the first report showing long term efficacy of half-dose PDT treatment in cases with CCH. The outcomes from this pilot study are comparable with results using full dose PDT protocols and it can be considered as a viable treatment option for CCH during the ongoing global verteporfin shortage.

Polypoidal Choroidal Vasculopathy Based on Non-ICGA Criteria in White Patients With Neovascular Age-Related Macular Degeneration.

PURPOSE: To determine prevalence of probable polypoidal choroidal vasculopathy (PCV) among White patients with neovascular age-related macular degeneration (nAMD) using non-indocyanine green angiography (ICGA) criteria DESIGN: Multicenter, multinational, retrospective, cross-sectional study. METHODS: A total of 208 treatment-naive eyes from Hispanic and non-Hispanic White individuals diagnosed with nAMD were included. All underwent color fundus photography (CFP), optical coherence tomography (OCT), and fluorescein angiography (FFA). De-identified images of study eyes were sent to 2 groups of graders. Group 1 reviewed CFP, OCT, and FFA to confirm nAMD diagnosis. Group 2 reviewed CFP and OCT to determine highly suggestive features for PCV. Probable PCV diagnosis defined as the presence of ≥2 of 4 highly suggestive features for PCV: notched or fibrovascular pigment epithelial detachment (PED) on CFP, sharply-peaked PED, notched PED, and hyperreflective ring on OCT. RESULTS: Eleven eyes were excluded because of poor image quality (6) or non-nAMD diagnosis (5). Of 197 eligible eyes (197 patients), the mean age (SD) was 78.8 years (8.9), 44.2% were men, 26.4% were Hispanic, and 73.6% were non-Hispanic White individuals; 41.1%, 23.4%, 9.1%, and 2.5% had ≥1, ≥2, ≥3, and 4 highly suggestive features. Results showed that 23.4% (95% CI, 17.6%-29.9%) had probable PCV diagnosis. Predominantly occult CNV was more frequently found in probable PCV than nAMD subgroup (84.8% vs 64.9%, P = .01). Hispanic White individuals had a lower prevalence of probable PCV than non-Hispanic White individuals (9.6% vs 28.2%, P = .006) CONCLUSIONS: These findings suggest that probable PCV occurs between 17.6% and 29.9% in White individuals with nAMD, and more commonly in non-Hispanic than in Hispanic White individuals.

THE TARGET SIGN: A Near Infrared Feature and Multimodal Imaging in a Pluri-Ethnic Cohort with RDH5-Related Fundus Albipunctatus.

PURPOSE: Retinol dehydrogenase 5 (RDH5)-related fundus albipunctatus can present with phenotypic variability. Our purpose was to investigate new clinical characteristics and multimodal imaging findings in patients from different ethnic origins, carrying different mutations. METHODS: Multicenter international retrospective case series of 18 patients with genetically confirmed RDH5-related fundus albipunctatus. Patients' files were reviewed for fundus images, visual acuity, macular optical coherence tomography scans, near-infrared images, fundus autofluorescence, electroretinogram, and genetic mutations. Imaging and electroretinogram findings. RESULTS: All eyes (n = 36, 100%) showed small circular findings seen on near-infrared images, termed as the "target sign," correlating to the yellowish dots seen clinically and to the distinct hyperreflective linear lesions on optical coherence tomography at the level between external limiting membrane and retinal pigment epithelium. Perifoveal atrophy with foveal sparing was seen in 4 eyes of 2 patients (both RDH5-c.160C>T, p.R54X mutation). Fundus autofluorescence revealed small hyperautofluorescent dots (n = 16, 44.4%). Scotopic electroretinograms were significantly reduced in all cases with an electronegative pattern, 66.7% displayed cone dysfunction. CONCLUSION: Our results show distinct imaging findings present in all patients with fundus albipunctatus independent of ethnicity or genetic mutation. Our results can facilitate the current algorithm to diagnose RDH5-related fundus albipunctatus and allow for targeted genetic testing.

ACQUIRED VITELLIFORM LESIONS: A Novel Finding in Eyes with Peripapillary Pachychoroid Syndrome.

PURPOSE: To describe a novel finding of acquired vitelliform lesions in eyes with peripapillary pachychoroid syndrome. METHODS: A retrospective, multicenter, observational case series including seven patients with peripapillary pachychoroid syndrome and concomitant acquired vitelliform lesions. Medical records and multimodal imaging findings were comprehensively reviewed. RESULTS: Six of seven patients were men, with a mean age of 72.6 years. Mean baseline best-corrected visual acuity was 0.11 ± 0.07 (Snellen equivalent 20/25), and all patients presented with baseline best-corrected visual acuity of 20/30 or better. Mean follow-up time was 24.4 months (range 1-54 months). At last follow-up, best-corrected visual acuity was stable in 13/14 eyes, and deteriorated in one eye because of subfoveal atrophy.Fundus examination revealed peripapillary retinal pigment epithelium alterations and pigment migration in all eyes. Fundus autofluorescence showed mottled hyperautofluorescence and hypoautofluorescence in the peripapillary region, and hypoautofluorescent lesions corresponding to the pigmentary changes. Optical coherence tomography showed nasal choroidal thickening and pachyvessels with overlying retinal pigment epithelium irregularity. All eyes presented with peripapillary intraretinal fluid. Subretinal fluid was present in five eyes of three patients. Acquired vitelliform lesions were present in 11 eyes. CONCLUSION: The novel association between peripapillary pachychoroid syndrome and acquired vitelliform lesions is characterized by the classic findings of both entities, and visual prognosis is accordingly good.

Next-generation anti-VEGF agents for diabetic macular oedema.

The treatment and outcomes of diabetic macular oedema (DMO) have improved with the introduction of intravitreal injections. However, real-world data reveal that the burden of DMO treatment causes large gaps in outcomes between randomized clinical trials and daily clinical practice. Long-lasting intravitreal drugs and devices for DMO might reduce this disparity by achieving optimal treatment due to more feasible injection regimens. In this manuscript, we cover pharmacodynamics, preliminary results from clinical trials, and safety behavior about brolucizumab, faricimab, conbercept, KSI-301, and port-delivery system WR42221. These treatments might present the first step to control the global epidemic of diabetic eye disease in real life.

Central serous chorioretinopathy imaging biomarkers.

PURPOSE: To identify the factors predicting the visual and anatomical outcomes in eyes with central serous chorioretinopathy (CSCR) through 12 months. METHODS: Patients with diagnosis of CSCR, either acute or chronic, were included in this multicentric, retrospective study. Demographic factors; systemic risk factors; central macular thickness (CMT), subfoveal choroidal thickness (SFCT), linear extent of ellipsoid zone (EZ) and interdigitation zone damage on optical coherence tomography; details of leak on fluorescein angiography and indocyanine green angiography were included as predictors of anatomical and visual outcomes. Regression analysis was performed to correlate the changes in best corrected visual acuity (BCVA) and resolution of disease activity. RESULTS: A total of 231 eyes of 201 patients with a mean age (49.7±11.8 years) were analysed. A total of 97 and 134 eyes were classified as acute and chronic CSCR. BCVA (0.35±0.31 to 0.24±0.34; p<0.001), baseline optical coherence tomography (OCT) parameters including CMT (p<0.001), subretinal fluid (SRF) height (p<0.001) and SFCT (p=0.05) showed a significant change through 12 months. Multivariate regression analysis showed change in CMT (p≤0.01) and SRF height at baseline (p=0.05) as factors predictive of good visual outcome. Logistic regression analysis revealed changes in both CMT (p=0.009) and SFCT (p=0.01) through 12 months to correlate with the resolution of disease. CONCLUSION: OCT parameters such as changes in both CMT and SFCT along with subfoveal EZ damage can be predictive of disease resolution whereas changes in CMT and baseline SRF height correlate well with changes in BCVA through 12 months.

Photodynamic therapy as a treatment option for peripapillary pachychoroid syndrome: a pilot study.

BACKGROUND: To investigate the anatomical and functional results in eyes with peripapillary pachychoroid syndrome (PPS) undergoing photodynamic therapy (PDT). METHODS: A total of 25 eyes from 23 patients with PPS treated with PDT were retrospectively evaluated in this multicentric study. Main outcome measure was the proportion of eyes that achieved treatment success, defined as a decrease in both subretinal fluid (SRF) height and central subfield thickness (CST), at 3 months after PDT compared to baseline. Secondary outcomes were the change in CST, SRF, and best-corrected visual acuity (BCVA) 3 months after treatment and predictive factors for treatment success. When available, data between 3 and 12 months were also reviewed. RESULTS: Treatment success was achieved in 16 eyes (64%). In the total cohort, CST decreased significantly from 356 ± 118 µm at baseline to 282 ± 90 µm and 270 ± 91 µm at 1 and 3 months, respectively (p < 0.001). Maximal SRF height decreased significantly from 102 ± 83 µm at baseline to 38 ± 46 µm and 32 ± 42 µm at 1 and 3 months, respectively (p < 0.001), and remained stable at month 6 (29 ± 44 µm) and month 12 (23 ± 35 µm). BCVA improved significantly from baseline to month 3 (p = 0.021). CONCLUSIONS: PDT can be considered an efficacious treatment option in patients with PPS. Prospective data with longer follow-up in a bigger cohort are needed in order to determine the optimal treatment algorithm in this relatively novel disease.

Detection of Diabetic Retinopathy from Ultra-Widefield Scanning Laser Ophthalmoscope Images: A Multicenter Deep Learning Analysis.

PURPOSE: To develop a deep learning (DL) system that can detect referable diabetic retinopathy (RDR) and vision-threatening diabetic retinopathy (VTDR) from images obtained on ultra-widefield scanning laser ophthalmoscope (UWF-SLO). DESIGN: Observational, cross-sectional study. PARTICIPANTS: A total of 9392 UWF-SLO images of 1903 eyes from 1022 subjects with diabetes from Hong Kong, the United Kingdom, India, and Argentina. METHODS: All images were labeled according to the presence or absence of RDR and the presence or absence of VTDR. Labeling was performed by retina specialists from fundus examination, according to the International Clinical Diabetic Retinopathy Disease Severity Scale. Three convolutional neural networks (ResNet50) were trained with a transfer-learning procedure for assessing gradability and identifying VTDR and RDR. External validation was performed on 4 datasets spanning different geographical regions. MAIN OUTCOME MEASURES: Area under the receiver operating characteristic curve (AUROC); area under the precision-recall curve (AUPRC); sensitivity, specificity, and accuracy of the DL system in gradability assessment; and detection of RDR and VTDR. RESULTS: For gradability assessment, the system achieved an AUROC of 0.923 (95% confidence interval [CI], 0.892-0.947), sensitivity of 86.5% (95% CI, 77.6-92.8), and specificity of 82.1% (95% CI, 77.3-86.2) for the primary validation dataset, and >0.82 AUROCs, >79.6% sensitivity, and >70.4% specificity for the geographical external validation datasets. For detecting RDR and VTDR, the AUROCs were 0.981 (95% CI, 0.977-0.984) and 0.966 (95% CI, 0.961-0.971), with sensitivities of 94.9% (95% CI, 92.3-97.9) and 87.2% (95% CI, 81.5-91.6), specificities of 95.1% (95% CI, 90.6-97.9) and 95.8% (95% CI, 93.3-97.6), and positive predictive values (PPVs) of 98.0% (95% CI, 96.1-99.0) and 91.1% (95% CI, 86.3-94.3) for the primary validation dataset, respectively. The AUROCs and accuracies for detecting both RDR and VTDR were >0.9% and >80%, respectively, for the geographical external validation datasets. The AUPRCs were >0.9, and sensitivities, specificities, and PPVs were >80% for the geographical external validation datasets for RDR and VTDR detection. CONCLUSIONS: The excellent performance achieved with this DL system for image quality assessment and detection of RDR and VTDR in UWF-SLO images highlights its potential as an efficient and effective diabetic retinopathy screening tool.

GRAding of functional and anatomical response to DExamethasone implant in patients with Diabetic Macular Edema: GRADE-DME Study.

To analyze functional and anatomical response patterns to dexamethasone (DEX) implant in diabetic macular edema (DME), to describe proportion of responders and non-responders, and to propose a new DME grading system. Retrospective, multicenter, observational cohort study. Naïve and non-naïve DME patients were treated with DEX, with visual acuity (VA) ≥ 0.2 logMAR and central subfield thickness (CST) of ≥ 300 µm. Functional and anatomical responses were graded after 2 and 4 months, and categorized as early and stable improvement, early and progressive improvement, pendular response, delayed improvement, and persistent non-response. 417 eyes were included (175 treatment naïve eyes). Compared to non-naïve eyes, naïve eyes showed a very good functional response (VA gain ≥ 10 letters) more frequently after 2 and 4 months (56% and 57% [naïve] vs. 33% and 28% [non-naïve], p < 0.001). A VA gain < 5 letters (non-response) after 2 and 4 months was seen in 18% and 16% of naïve eyes, and in 49% and 53% of non-naïve eyes (p < 0.001). A lack of anatomical response was rare in both groups, but more frequently in non-naïve eyes (12% vs. 4%, p = 0.003). Functionally and anatomically, naïve eyes showed most frequently an early and stable improvement (functionally: 77/175 44%; anatomically: 123/175 eyes, 70%). Most non-naïve eyes experienced no significant improvement functionally (97/242 eyes, 40%), despite a mostly early and stable improvement anatomical response pattern (102/242 eyes, 42%). Functional but not anatomical response patterns were influenced by baseline VA. Naïve and non-naïve eyes show different functional and anatomical response patterns to DEX implant. Functional non-responders are rare in naïve eyes, whereas anatomical non-response is unusual in both groups.

Longer-acting treatments for neovascular age-related macular degeneration-present and future.

The treatment of neovascular AMD (nAMD) has been revolutionized by the introduction of anti-vascular endothelial growth factor (VEGF) agents. Though, there is a tremendous gap between the outcomes in randomized clinical trials and real-world settings, where long-term outcomes are not as good as expected. This is due to undertreatment, i.e., fewer injection and low monitoring frequency. Treatment burden due to frequent injections remains a major limitation. Long-lasting treatments provide promising solutions for this unmet need by achieving better results with less mandatory injections. This review aims to cover the current state in this field and also discuss the mechanism of action, data from pivotal trials, and safety profile of long-acting treatments in present and future, going into details about the following agents: Brolucizumab, Faricimab, Abipicar, and Conbercept.

Occlusive retinal vasculopathy with macular branch retinal artery occlusion as a leading sign of atypical hemolytic uremic syndrome - a case report.

BACKGROUND: Hemolytic Uremic Syndrome (HUS) is a rare disorder characterized by the triad of microangiopathic hemolytic anemia, thrombocytopenia, and acute renal failure, considered within the group of thrombocytic microangiopathies. Ocular complications in HUS are very rare. Here, we report an adult patient who suffered from acute onset of paracentral scotoma, caused by branch retinal artery occlusion (BRAO), as a leading symptom of atypical HUS. CASE PRESENTATION: A 39-year-old healthy male was lately diagnosed with essential hypertension and mild renal impairment. He complained about acute onset of central scotoma in his left eye. Fundus examination revealed marked narrowing of retinal vessels, cotton wool spots and few retinal hemorrhages in both eyes. The patient was diagnosed with bilateral ischemic retinal vasculopathy and acute macular BRAO in his left eye. Workup revealed thrombocytopenia, worsening renal failure. Renal biopsy showed signs of chronic thrombotic microangiopathy. The patient was diagnosed with atypical HUS (aHUS) and started on plasmapheresis, together with eculizumab. As his condition continued to worsen, he was put on renal replacement therapy. Due to a persistent monoclone of IgG1, the patient underwent bone marrow biopsy which revealed Monoclonal Gammopathy of renal significance, triggering a HUS and treatment was initiated accordingly. Two months after initial presentation, the patient developed neovascularization of the optic disc (NVD) in his left eye, and was treated with 3 monthly intravitreal bevacizumab injections with complete regression of the NVD. The patient suffered from myocardial infarction in the later course and was lost for follow-up. He returned 11 months after the last bevacizumab injection because of sudden loss of vision in his left eye caused by a dense vitreous hemorrhage. Biomicroscopy revealed a new NVD in his right eye. The patient underwent panretinal photocoagulation in both eyes with regression of neovascularization. Vision improved and remained 20/20 in both eyes. CONCLUSION: We present a case report showing retinal ischemia can be linked with aHUS. As clinal diagnosis might be challenging, physicians should be aware of the rare ocular manifestations of this devastating multi-organ disease. In case of retinal ischemia, panretinal photocoagulation should be initiated soon to avoid blinding complications.

Subclinical subretinal fluid detectable only by optical coherence tomography in choroidal naevi-the SON study.

BACKGROUND: Subretinal fluid is a risk factor for growth and malignant transformation of choroidal naevi, however it is unclear if this applies to subclinical fluid that is only detectable by optical coherence tomography (OCT). The objective of this study was to determine the prevalence and associations of subclinical but OCT-detectable subretinal fluid over choroidal naevi. METHODS: Cross-sectional study of 309 consecutive cases of choroidal naevi imaged by OCT between July 2017 to January 2019. Multicentre international study involving ten retinal specialist centres. All patients presenting to retinal specialists had routine clinical examination and OCT imaging. The prevalence of subclinical OCT-detectable subretinal fluid over choroidal naevi and its associations with other features known to predict growth and malignant transformation were noted and analysed. RESULTS: Of 309 identified consecutive cases, the mean patient age was 65 years, 89.3% of patients were Caucasian and 3.9% were Asian. The prevalence of subclinical but OCT-detectable subretinal fluid associated with choroidal naevi was 11.7% (36/309). Naevi with fluid were associated with larger basal diameters, greater thickness, presence of a halo, orange pigmentation, hyperautofluorescence, and hypodensity on B-scan ultrasonography. CONCLUSION AND RELEVANCE: Of choroidal naevi where subretinal fluid is not visible on clinical examination, 11.7% demonstrate subretinal fluid on OCT scans. These naevi more commonly exhibit features known to be associated with growth and transformation to melanoma. The presence of subclinical OCT-detectable fluid over choroidal naevi may assist in their risk stratification.