Faculty Bibliography

PURPOSE/OBJECTIVE:To describe the multimodal imaging findings of the angular sign of Henle fiber layer (HFL) hyperreflectivity (ASHH) at baseline and follow-up in patients with contusion maculopathy. METHODS:Eleven eyes of ten patients were captured with multimodal imaging after non-penetrating ocular blunt trauma from a soccer ball, fist, or airsoft pellet. Baseline clinical and imaging characteristics and follow-up outcomes are presented. RESULTS:Hyper-reflective lesions extending along the HFL from the ellipsoid zone (EZ) to the outer plexiform layer consistent with ASHH were identified with optical coherence tomography (OCT). Mean presenting visual acuity (VA) was logMAR 0.59 ± 0.64 (Snellen VA 20/77, range 20/25 to counting fingers) and follow-up visual acuity was logMAR 0.43 ± 0.35 (Snellen VA 20/53, range 20/20 to 20/200). Additional OCT findings included external limiting membrane attenuation and retinal pigment epithelium (RPE) disruption. On follow-up, resolution of ASHH was accompanied by outer nuclear layer thinning with varying degrees of EZ attenuation and RPE loss. A macular hole was detected in one patient on follow-up. CONCLUSION/CONCLUSIONS:ASHH is a distinctive acute OCT feature of contusion maculopathy secondary to blunt injury, causing disruption of the photoreceptors and presumably anterograde alterations in the HFL. Associated RPE alterations may ensue, either acutely or delayed, and are a biomarker of persistent structural abnormalities and variable visual outcomes.

PURPOSE/OBJECTIVE:To explore the impact of home optical coherence tomography (OCT)-guided treatment personalization for type 3 macular neovascularization (MNV) in age-related macular degeneration (AMD). METHODS:A prospective home OCT trial of a patient with a one-month history of type 3 MNV under anti-angiogenic treatment (two "loading" injections). During the 23.4-week study period, the patient's treatment regimen was managed through regular self-imaging, enabling continuous monitoring of retinal fluid dynamics. RESULTS:Over the observation period, the patient performed 143 home OCT measurements (mean 6.1 per week, standard deviation 1), which prompted three in-office visits where three anti-angiogenic injections were delivered at the discretion of the investigator. The mean individualized treatment intervals established through home OCT were 8 weeks, with the patient maintaining stable visual acuity at 20/20 Snellen. Complete resolution of intraretinal fluid was noted between injection. Notably, home OCT revealed unexpected fluctuations in retinal fluid measured as hyporeflective spaces in the absence of treatment. Two out of three injections were administered after the retinal fluid volume had decreased by more than half within two, and eight days following an initial transient spike. CONCLUSION/CONCLUSIONS:The neovascular subtype as defined by the Consensus Nomenclature for Reporting Neovascular AMD Data (CONAN) may serve as a useful management framework when approaching home OCT guided treatment decisions. In type 3 disease, home OCT-guided management demonstrates significant potential for optimizing treatment. Further research is warranted to elucidate the dynamics of retinal fluid variations in different MNV subtypes.

OBJECTIVE:To develop a consensus nomenclature for Optical Coherence Tomography Angiography (OCTA) findings in retinal vascular diseases (RVD). DESIGN/METHODS:Expert consensus using standardized online surveys with modified Likert scale. PARTICIPANTS/METHODS:RVD imaging experts, OCT biomedical engineers and the members of the International Retinal Imaging Society (IntRIS) METHODS: A PubMed literature review identified quantitative and qualitative terms forming the basis for a consensus-building process using a modified Delphi method. Agreement levels were categorized as "Accepted" (median ≥ 6), "Considerable Consensus" (median 6-7, IQR ≤ 3), "Strong Consensus" (median ≥ 8, IQR ≤ 2), and "Refined Strong Consensus" (median ≥ 8, IQR ≤ 2, with ≥ 70% responses in the 8-10 range). A multidisciplinary expert panel refined the terminology through three survey rounds, leading to a final survey conducted by IntRIS members. MAIN OUTCOME MEASURES/METHODS:Consensus on OCTA nomenclature in RVD RESULTS: The literature review identified 58 relevant papers, yielding 51 quantitative and 108 qualitative terms. A series of three surveys was used to refine the nomenclature framework for describing OCTA findings. The selected framework includes a generic term ("OCTA signal"), adjective terms ("presence/absence", "decreased/increased", "normal/abnormal"), and descriptive/etiologic terms ("of unknown cause", "due to blockage", "due to non-perfusion"). In the final survey among 44 IntRIS members, the framework achieved strong consensus for overall acceptance (median: 8.0, IQR: 7.0-9.0). The term "OCTA signal" met refined strong consensus criteria (median: 8.0, IQR: 8.0-9.0, with ≥ 70% of responses in the 8-10 range). Adjective terms, including "absence/presence" and "increased/decreased," were also rated with strong consensus (median: 8.0, IQR: 7.0-9.0). Similarly, descriptive/etiologic terms achieved strong consensus (median: 8.0, IQR: 7.0-9.0). Adoption of the framework for clinical practice and scientific reporting was rated with strong consensus (clinical: median 8.0, IQR: 7.0-9.0; scientific: median 9.0, IQR: 8.5-10.0). CONCLUSIONS:This study establishes a strong consensus framework for reporting OCTA findings in RVD for clinical and scientific contexts.

PURPOSE/OBJECTIVE:To develop imaging and consensus-based guidelines on the application of multimodal imaging in multiple evanescent white dot syndrome (MEWDS). DESIGN/METHODS:Consensus agreement guided by literature, and an expert committee using a nominal group technique (NGT). METHODS:The expert committee employed a structured NGT with multiple rounds of discussion, conflict resolution, and anonymous voting to: (1) establish imaging criteria for diagnosing and monitoring MEWDS using color fundus photography (CFP), optical coherence tomography (OCT), fundus autofluorescence (FAF), fundus fluorescein angiography (FFA), indocyanine green angiography (ICGA), and OCT angiography (OCTA); and (2) develop consensus-based recommendations for assessing specific characteristics in patients with MEWDS. These formal recommendations were derived from a structured NGT using illustrative cases of MEWDS and were further voted upon by the entire task force. RESULTS:The diagnosis of acute MEWDS is supported by distinct multimodal features on CFP, multi-focal disruption of the ellipsoid/interdigitation zone with overlying outer retinal hyper-reflectivity with OCT, and hyper-autofluorescent spots with FAF (short-wave blue/green). In complex cases, wreath-like lesions on FFA and the absence of early hypofluorescence on ICGA help differentiate MEWDS from other chorioretinopathies. The lack of specific choroidal changes on OCT and preserved signal on OCTA on retinal and inner choroidal slabs also aid in diagnosis. CONCLUSIONS:Multimodal imaging is essential for diagnosing MEWDS and differentiating it from other non-infectious uveitis types, extending the Standardization of Uveitis Nomenclature (SUN) classification. These imaging criteria enable detailed assessment of disease activity and offer valuable insights into MEWDS pathogenesis.

PURPOSE/OBJECTIVE:To characterize the clinical and multimodal imaging features of central bouquet hemorrhage (CBH) with Henle fiber layer (HFL) involvement in highly myopic eyes, and to investigate the relationships between hemorrhage characteristics, reabsorption time, and visual outcomes. METHODS:Multicenter, retrospective analysis of highly myopic eyes with CBH involving the HFL, confirmed by optical coherence tomography (OCT). RESULTS:Eighteen eyes from 18 subjects were included for analysis. The mean age of the cohort was 39 ± 13.7 years (range: 17-69) and 61% of subjects were female. Mean refractive error was -14.8 ± 3.14 diopters (range: -9 D to -22 D). All eyes demonstrated a combined CBH with HFL component, while a subretinal component was present in 83.3% of cases. Myopic choroidal neovascularization (CNV) was excluded in all eyes using optical coherence tomography angiography (OCTA) or dye-based angiography (fluorescein or indocyanine green). No correlation was observed between hemorrhage size and visual outcomes or reabsorption time. Hemorrhage cleared after a mean of 2.63 months, and the radial HFL hemorrhage component resolved first. All eyes showed improvement in visual acuity from baseline. Persistent OCT alternations after resolution of hemorrhage included ellipsoid zone disruption (88.9%) and hyperreflective changes in HFL (77.8%). Anti-VEGF injections were administered to 6 eyes (33.3%) and did not correlate with a significant visual or anatomical benefit. CONCLUSION/CONCLUSIONS:CBH with HFL involvement in high myopia was associated with significantly improved visual outcomes from baseline but structural alterations can persist after clinical resolution. The size of the hemorrhage did not correlate with resorption time, and anti-VEGF treatment did not affect outcome. These findings provide new insights into the natural history and management of nonneovascular CBH in highly myopic eyes.

PURPOSE/OBJECTIVE:To develop consensus nomenclature amongst international retinal specialists for the distinctive optical coherence tomography (OCT) finding of a lesion originating from the retinal capillary bed, measuring ≥100 µm in size, and characterized by a hyperreflective wall with a hyporeflective lumen. METHODS:A comprehensive literature search was performed from inception to January 2024 on three databases to elicit publications reporting on relevant vascular abnormalities and corresponding nomenclature. A panel of retinal specialists with expertise in this topic reviewed the list of candidate terms and proposed other names for the lesion of interest. A refined list was then incorporated into a Delphi survey, which was distributed to the general membership of the International Retinal Imaging Society (IntRIS). Consensus was defined as at least 70% agreement amongst participants. RESULTS:An expert panel (n=11) reviewed candidate names for the lesion, with poor agreement noted amongst panel members regarding the relevant nomenclature. In the first Delphi survey, (n=70 IntRIS members), the need for a unified nomenclature was highlighted and two leading candidate names were established: large retinal capillary aneurysm (LRCA, n=38, 54.3%) and retinal capillary macroaneurysm (n=14, 20.0%). A second follow-up survey (n=54 IntRIS members) established LRCA (n=44, 81.5%) as the consensus term to identify the OCT vascular abnormality. CONCLUSION/CONCLUSIONS:This Delphi project reached consensus on a unifying term, large retinal capillary aneurysm, for a specific and signature OCT lesion. Identification of this characteristic OCT finding and adoption of this term may facilitate diagnosis, guide therapeutic decisions, and improve clinical and scientific communication.

PURPOSE/OBJECTIVE:To describe the clinical features, multimodal imaging findings, natural history, and treatment outcomes of acute outer retinopathy (AOR), which represents an expanded spectrum of acute annular outer retinopathy (AAOR). DESIGN/METHODS:Retrospective, observational, longitudinal, multicenter case series. PARTICIPANTS/METHODS:Twenty-three patients (15 female, 8 male) with a mean age of 41.8 ± 18.6 years (range: 14-86 years) and a mean follow-up duration of 3.7 ± 1.5 years (range: 1-12 years). METHODS:Clinical characteristics, multimodal imaging findings, laboratory evaluations, genetic testing, natural history, therapeutic management, and outcomes were reviewed and analyzed. MAIN OUTCOMES MEASURES/METHODS:Specific multimodal imaging signatures of AOR were identified, including findings from ophthalmoscopy, fundus autofluorescence (FAF), fluorescein angiography (FA), indocyanine green angiography (ICGA), and optical coherence tomography (OCT). Humphrey visual field testing, full-field electroretinography (ERG), and multifocal ERG were analyzed. Baseline features and the natural course of the disease were delineated. RESULTS:Thirty-eight eyes from 23 patients were analyzed. Presenting symptoms included photopsia (87%), blurred vision (57%), and scotoma (57%). On ophthalmoscopy, AOR was acutely characterized by yellow-greyish outer retinal lesions corresponding to hyperautofluorescent changes on FAF and the angular sign of Henle fiber layer hyperreflectivity (ASHH) on OCT. FAF imaging revealed ring-like hyperautofluorescent lesions surrounding the optic disc in 18% of eyes. Additional lesion patterns on FAF included perivenular (53%), sectoral (16%), and spot-like distributions (13%). FA and ICGA findings were mostly unremarkable. Lesion progression primarily occurred within the initial weeks following presentation and stabilized in size beyond this period in the majority of eyes. Over time, affected areas progressed to outer retinal atrophy with pigmentary changes. Foveal sparing was observed in 68% of the eyes. None of the therapeutic interventions appeared effective in halting the progression to complete outer retinal atrophy or preventing lesion enlargement. CONCLUSIONS:AOR is characterized by early photoreceptor disruption, evidenced by ASHH on OCT, leading to rapid outer retinal atrophy and subsequent degeneration of the retinal pigment epithelium within the damaged zones. Although distinct patterns of lesion distribution were observed, their consistent features on multimodal imaging support their inclusion within a unified disease spectrum termed acute outer retinopathy.