{"doi":"10.1016/j.xops.2025.100823","title":"Multimodal Imaging and Dark-Adapted Chromatic Perimetry in BEST1 Vitelliform Macular Dystrophy: Identification of Outcome Measurements","abstract":"Objective: To characterize longitudinal multimodal imaging characteristics of Best vitelliform macular dystrophy (BVMD) and describe novel outcome measurements for future clinical trials. Design: A single-center retrospective cohort study. Subjects: A total of 36 eyes of 18 patients with genetically and clinically confirmed diagnoses of BVMD were evaluated. Methods: Patients underwent complete ophthalmic examination and multimodal imaging including spectral-domain OCT and short-wavelength autofluorescence. Dark-adapted chromatic (DAC) perimetry was obtained in 8 of 18 patients. Main Outcome Measures: Longitudinal changes in lesion width, area, and measures of retinal thickness, including outer nuclear layer (ONL) thickness, were evaluated as primary outcome measures. The changes were measured at every visit to the ophthalmology clinic, with a difference between the first and most recent individual's visit of 76.56 ± 33.36 months (range 10-123 months), on average. Scotopic sensitivity as measured by DAC perimetry was compared within and outside the lesion. Results: < 0.05). Conclusions: Features of multimodal imaging, including measurements of ONL thickness along the temporal and nasal borders of the lesion, as well as scotopic sensitivity as measured by DAC perimetry, may serve as valuable outcome measurements for treatment trials for BVMD. Financial Disclosures: Proprietary or commercial disclosure may be found in the Footnotes and Disclosures at the end of this article.","journal":"Ophthalmology Science","year":2025,"id":552656,"datarank":0.10397207708399181,"base_score":0.6931471805599453,"endowment":0.6931471805599453,"self_citation_contribution":0.10397207708399181,"citation_network_contribution":0.0,"self_endowment_contribution":0.10397207708399181,"citer_contribution":0.0,"corpus_percentile":21.9,"corpus_rank":9873,"citation_count":1,"citer_count":0,"citers_with_citation_signal":0,"citers_with_endowment":0,"datacite_reuse_total":0,"is_dataset":true,"is_dataset_confidence":0.7114,"is_data_producer":false,"deposit_databanks":null,"is_oa":true,"file_count":0,"downloads":0,"has_version_chain":false,"published_date":"2025-01-01","fair_score":null,"fair_percentile":null,"algorithm_id":"datarank_citation_only_1hop_v6","ranking_scope":"data_only","authors":[{"id":380267,"name":"Jin Kyun Oh","orcid":"0000-0002-5350-9412","position":1,"is_corresponding":false},{"id":300381,"name":"Sara D. Ragi","orcid":null,"position":2,"is_corresponding":false},{"id":380266,"name":"Joonpyo Kim","orcid":"0000-0003-0789-7685","position":3,"is_corresponding":false},{"id":1449450,"name":"Remy S Manzi","orcid":null,"position":4,"is_corresponding":false},{"id":965915,"name":"Emily Sun","orcid":"0000-0002-9488-3750","position":5,"is_corresponding":false},{"id":380264,"name":"Thiago Cabral","orcid":"0000-0002-1201-8772","position":6,"is_corresponding":false},{"id":380271,"name":"Rubens Belfort","orcid":"0000-0002-8422-3898","position":7,"is_corresponding":false},{"id":386533,"name":"Vivienne C. Greenstein","orcid":null,"position":8,"is_corresponding":false},{"id":263802,"name":"Janet R. Sparrow","orcid":"0000-0001-7448-5950","position":9,"is_corresponding":false},{"id":299097,"name":"Stephen H. Tsang","orcid":"0000-0001-9082-2427","position":10,"is_corresponding":false},{"id":380265,"name":"José Ronaldo Lima de Carvalho","orcid":"0000-0002-7024-1702","position":0,"is_corresponding":true}],"reference_count":31,"raw_metadata":{"citation_network_status":"fetched"},"created_at":"2026-07-19T02:54:37.527189Z","pmid":"40688488","pmcid":"PMC12270738","fwci":null,"citation_percentile":null,"influential_citations":0,"oa_status":null,"license":null,"views":0,"total_file_size_bytes":0,"version_count":0,"fair_f":null,"fair_a":null,"fair_i":null,"fair_r":null,"fair_zscore":null,"fair_rationale":null,"fair_model":null,"fair_agent_version":null,"fair_fulltext_source":null,"fair_has_llm":null,"fair_computed_at":null,"clinical_trials":[],"software_tools":[],"db_accessions":[],"linked_datasets":[],"topics":[]}