Clinical and Multimodal Imaging of Acute Outer Retinopathy: Expanding the Spectrum of Acute Annular Outer Retinopathy
Issued Date
2025-01-01
Resource Type
eISSN
24686530
Scopus ID
2-s2.0-105009897214
Pubmed ID
40436146
Journal Title
Ophthalmology Retina
Rights Holder(s)
SCOPUS
Bibliographic Citation
Ophthalmology Retina (2025)
Suggested Citation
Ramtohul P., Cicinelli M.V., Chen F.K., Oh D.J., Freilich B.D., Singer M.A., Hartley M.J., Biswas J., Boulanger E., Bae K., Lim H.Y., Sujirakul T., Gascon P., Blinder K.J., Fardeau C., Pockar S., Androudi S., Nakashizuka H., Kitagawa Y., Shinojima A., Miserocchi E., Freund K.B. Clinical and Multimodal Imaging of Acute Outer Retinopathy: Expanding the Spectrum of Acute Annular Outer Retinopathy. Ophthalmology Retina (2025). doi:10.1016/j.oret.2025.05.027 Retrieved from: https://repository.li.mahidol.ac.th/handle/20.500.14594/111217
Title
Clinical and Multimodal Imaging of Acute Outer Retinopathy: Expanding the Spectrum of Acute Annular Outer Retinopathy
Author's Affiliation
University of Melbourne
The University of Western Australia
Aix Marseille Université
NYU Grossman School of Medicine
Hôpital Universitaire Pitié Salpêtrière
The University of Texas Health Science Center at San Antonio
Seoul National University College of Medicine
San Raffaele Scientific Institute
Keio University School of Medicine
University of Thessaly
Università Vita-Salute San Raffaele
Ramathibodi Hospital
Fondation Adolphe de Rothschild
Sankara Nethralaya
Manchester Royal Eye Hospital
Vitreous Retina Macula Consultants of New York
Nihon University Hospital
Sunderland Eye Infirmary
The Retina Institute
Clinique Juge
Centre Monticelli Paradis
Retina Center of New Jersey
The University of Western Australia
Aix Marseille Université
NYU Grossman School of Medicine
Hôpital Universitaire Pitié Salpêtrière
The University of Texas Health Science Center at San Antonio
Seoul National University College of Medicine
San Raffaele Scientific Institute
Keio University School of Medicine
University of Thessaly
Università Vita-Salute San Raffaele
Ramathibodi Hospital
Fondation Adolphe de Rothschild
Sankara Nethralaya
Manchester Royal Eye Hospital
Vitreous Retina Macula Consultants of New York
Nihon University Hospital
Sunderland Eye Infirmary
The Retina Institute
Clinique Juge
Centre Monticelli Paradis
Retina Center of New Jersey
Corresponding Author(s)
Other Contributor(s)
Abstract
Purpose: 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. Design: Retrospective, observational, longitudinal, multicenter case series. Participants: 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: Specific multimodal imaging signatures of AOR were identified, including findings from ophthalmoscopy, fundus autofluorescence (FAF), fluorescein angiography, indocyanine green angiography (ICGA), and 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-grayish outer retinal lesions corresponding to hyperautofluorescent changes on FAF and the angular sign of Henle fiber layer hyperreflectivity (ASHH) on OCT. Fundus autofluorescence 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%). Fluorescein angiography and ICGA findings were mostly unremarkable. Lesion progression primarily occurred within the initial weeks after 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: Acute outer retinopathy 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 AOR. Financial Disclosure(s): Proprietary or commercial disclosure may be found in the Footnotes and Disclosures at the end of this article.