Faculty Bibliography

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 describe the clinical and multimodal imaging features, and long-term outcomes, of acquired vitelliform lesions (AVLs) in angioid streaks (AS). METHODS:Retrospective case series including 14 patients (23 eyes) with AS-related AVLs. Clinical data, color fundus photography, fundus autofluorescence, spectral-domain optical coherence tomography (OCT), en face OCT, and OCT angiography were evaluated at baseline and final visits. Snellen visual acuity (VA), lesion dimensions, subfoveal choroidal thickness (SFCT), and outer retinal integrity were recorded. RESULTS:AS were secondary to pseudoxanthoma elasticum in 64.3% and idiopathic in 35.7%. Baseline VA was 0.18 ± 0.17 LogMAR (20/30) and declined to 0.43 ± 0.33 LogMAR (20/50) over a mean follow-up of 77 months (p<0.001). AVLs were often foveal (78.3%), multifocal (82.6%), and peripapillary (73.9%), with OCT detecting subretinal hyperreflective material in all eyes. Both lesion width and SFCT decreased over time. Complete retinal pigment epithelium (RPE) and outer retinal atrophy increased from 17.4% to 69.6%, and exudative choroidal neovascularization developed in 26.1%. CONCLUSION/CONCLUSIONS:AS-related AVLs represent a rare phenotype reflecting multifactorial pathogenesis involving Bruch's membrane alterations and RPE dysfunction. Over prolonged follow-up, lesion size decreased, yet progressive retinal atrophy led to significant vision loss. Further research is warranted to clarify disease progression and optimize treatment approaches.

En face optical coherence tomography (OCT) is a practical and informative imaging modality to noninvasively visualize distinct retinal and choroidal layers by providing coronal images using boundary-specific segmentation. Ongoing research with this method is generating breakthroughs in the illustration of new perspectives of retinal disease. The clinical value of en face OCT as an advanced retinal imaging tool is growing steadily and it has unveiled many new insights into the pathoanatomy of retinal disorders. Moreover, this modality can capture various en face OCT biomarkers that correspond to different cell or tissue subtypes, which were previously only identified through histological or electron microscopy methods, underscoring the significance of this technique in providing valuable pathoanatomical information. In this comprehensive review, we will systematically summarize the en face OCT findings across a broad spectrum of retinal diseases, including disorders of the vitreoretinal interface and retinal vascular system (e.g. paracentral acute middle maculopathy or PAMM and diabetic retinopathy), in addition to the en face OCT features of other conditions such as age-related macular degeneration, pachychoroid disease spectrum, myopic degeneration, uveitis and inflammatory disorders, inherited retinal dystrophies, and drug toxicity. We will discuss and highlight the unique clinical and pathoanatomical findings uncovered with en face OCT of each these diseases mentioned above.

PURPOSE/OBJECTIVE:To report the clinical and multimodal imaging (MMI) findings and long-term follow-up of stellate nonhereditary idiopathic foveomacular retinoschisis (SNIFR) contiguous with midperipheral retinoschisis (MPRS) and to describe a severe SNIFR variant termed CARPET (Central Anomalous Retinoschisis with mid-PEripheral Traction). DESIGN/METHODS:Retrospective case series. SUBJECTS/METHODS:Eleven patients (15 eyes) with SNIFR contiguous with MPRS in at least one eye at baseline or final follow-up. METHODS:MMI features, including cross-sectional and en face macular and peripheral spectral-domain optical coherence tomography (OCT) and OCT angiography, were reviewed in all cases at baseline and at the final follow-up visit. MAIN OUTCOME MEASURES/METHODS:Various courses (including progression, regression, or stability) of MPRS or SNIFR over time were evaluated. RESULTS:MPRS exhibited centripetal progression to SNIFR in 5 eyes of 3 patients with follow up of 67, 60, and 27 months, respectively, with maintenance of excellent visual acuity (range: 20/25-20/20) in 4 of these 5 eyes. In 2 eyes of 2 patients (including 1 eye with initial centripetal progression of MPRS to SNIFR), MPRS contiguous with SNIFR spontaneously resolved with long-term follow-up (77 and 48 months, respectively). SNIFR contiguous with MPRS partially regressed after 48 months in one patient, and was stable after 54 months in another. A distinctive midperipheral microvasculopathy, associated with MPRS that was contiguous with SNIFR, was identified in 7 eyes of 4 patients. Finally, 3 eyes of 3 patients exhibited additional unique features, including central neurosensory detachment and outer lamellar macular hole, which were associated with significant midperipheral traction, representing a severe variant subtype of SNIFR that we refer to as CARPET. Two of these 3 eyes progressed with short-term follow-up of 6 and 2 months, respectively, while the schisis resolved and vision improved after pars plana vitrectomy in the third case. CONCLUSIONS:MPRS can progress to SNIFR over multiple years of follow-up. SNIFR with MPRS can also spontaneously resolve or remain stable. MPRS can additionally be complicated by a midperipheral inner retinal microvasculopathy. Finally, CARPET may represent a unique and severe variant form of SNIFR driven by midperipheral vitreoretinal traction and associated with significant vision loss.

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/UNASSIGNED:The purpose of this study was to develop ground-truth histology about contributors to variable fundus autofluorescence (FAF) signal and thus inform patient selection for treating geographic atrophy (GA) in age-related macular degeneration (AMD). METHODS/UNASSIGNED:One woman with bilateral multifocal GA, foveal sparing, and thick choroids underwent 535 to 580 nm excitation FAF in 6 clinic visits (11 to 6 years before death). The left eye was preserved 5 hours after death. Eye-tracked ex vivo imaging aligned sub-micrometer epoxy resin sections (n = 140, 60 µm apart) with clinic data. Light microscopic morphology corresponding to FAF features assessed included drusen-driven atrophy, persistent hyperautofluorescence (hyperFAF) islands and peninsulas within atrophy, and hyperFAF and hypoautofluorescence (hypoFAF) inner junctional zone (IJZ) and outer junctional zone (OJZ) relative to descent of external limiting membrane (ELM). Atrophy growth rate was calculated. RESULTS/UNASSIGNED:HypoFAF atrophic spots appeared in association with drusen, and then expanded and coalesced. Over drusen (n = 45, all calcified), RPE was continuous and thin, photoreceptors were short or absent, and initially intact ELM descended where RPE was absent. In persistent hyperFAF within atrophy and in the OJZ, the RPE was continuous and dysmorphic, photoreceptors were present and short, and BLamD was thick. In the IJZ, mottled FAF corresponded to dissociated RPE atop persistent BLamD. Overall linear growth rate (0.198 mm/ year) typified multifocal GA. CONCLUSIONS/UNASSIGNED:FAF in GA is locally multifactorial, with photoreceptor shortening potentially promoting hyperFAF by increasing incoming excitation light available to RPE fluorophores. RPE dysmorphia may lead to either longer or shorter pathlength for excitation light. At both atrophy initiation and expansion Müller glia are major participants.

We report clinical and imaging features of Valsalva-induced choroidal hemorrhage (VICH) in high myopia, highlighting choroidal venous congestion, and hyperpermeability in dominant vortex vein systems, and luminal compression at the crest of deep myopic staphylomas.

PURPOSE/OBJECTIVE:This study aimed to delineate the characteristics, prevalence, and outcomes of neovascularization (NV), particularly aneurysmal type 1 NV, in patients with angioid streaks (AS) secondary to pseudoxanthoma elasticum (PXE), and to introduce a clinical classification based on multimodal imaging. DESIGN/METHODS:Retrospective longitudinal cohort study. PARTICIPANTS/METHODS:Eighty-five patients (168 eyes) with AS secondary to PXE at 2 tertiary referral centers. METHODS:Data collection included demographic, medical, and ocular histories. Diagnostic methods comprised fundus photography, autofluorescence, indocyanine green angiography, OCT, and OCT angiography. MAIN OUTCOME MEASURES/METHODS:Prevalence of type 1 NV, visual acuity (VA), risk of exudation. RESULTS:Type 1 NV was identified in 127 eyes (76%), with 85 of these (67%) showing exclusively type 1 NV. These lesions often originated around the disc, at sites of Bruch membrane dehiscences, and followed the path of AS, extending to the macula in 101 eyes (80%). Despite 65% of type 1 NV remaining nonexudative, 35% evolved into exudative over 5 years, and 11 eyes experienced midperipheral subretinal hemorrhages. Aneurysmal dilations, observed in 57% of eyes, substantially increased exudation risk (hazard ratio = 3.86, P = 0.02). Despite treatment, VA significantly deteriorated in exudative type 1 NV (P = 0.02). Type 2 NV, detected in 42 eyes (33%), often coexisted with type 1 NV and was associated with poorer visual outcomes and higher rates of macular atrophy. A classification of AS was developed, ranging from empty AS (stage 0, no NV) to advanced NV (stage 3, both type 1 and type 2 NV). CONCLUSIONS:Type 1 NV predominates in AS. Although predominantly nonexudative, its progression correlates with substantial visual impairment, similar to the deficits observed with type 2 NV. Aneurysmal type 1 NV poses a significant exudation risk, underscoring the need for vigilant monitoring. FINANCIAL DISCLOSURE(S)/BACKGROUND:Proprietary or commercial disclosure may be found in the Footnotes and Disclosures at the end of this article.