To describe the clinical course and provide initial imaging perspectives in acute annular outer retinopathy (AAOR) by reviewing multimodal imaging (MMI) findings. Observational case series study. Nine patients (12 eyes) with AAOR. Review clinical charts and MMI findings. Clinical medical records and multimodal imaging features via fundus photographs, optical coherence tomography (OCT), fundus autofluorescence (AF) and OCT angiography (OCTA), as applicable. Twelve eyes of nine patients (18-39 years) were identified. The clinical course was subdivided acutely progressive stage (APS), stationary stage (SS) and atrophic stage (AS) (1-3 weeks, 3 weeks to 3 months and >3 months, respectively). During APS, advancement of peripapillary annular band was noted in all eyes, accompanied by absent ellipsoid zone, increased choroidal thickness (ChT) and thinned outer nuclear layer (ONL) within the affected area. During SS, annular band disappeared on fundus photograph with improved ONL with improved ONL. During AS, affected area became depigmented with scattered pigmentation, exhibiting hyper-AF or mixed AF. Other manifestations included retinal atrophy and decreased ChT. OCTA revealed an enlarged foveal avascular zone in each stage, especially in AS. Seven eyes of 5 patients showed no macular involvement with best corrected visual acuity (BCVA) of 20/20, while 5 eyes of 5 patients showed macular involvement with poorer BCVA of HM-20/400 at the last visit. AAOR mostly occurs in young individuals with unilateral involvement, especially in those with mild to moderate myopia. The observed variations in the clinical course of AAOR, documented through MMI, aid in the prompt recognition and understanding of this rare entity.

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. Retrospective, observational, longitudinal, multicenter case series. 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). Clinical characteristics, multimodal imaging findings, laboratory evaluations, genetic testing, natural history, therapeutic management, and outcomes were reviewed and analyzed. 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. 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. 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. Proprietary or commercial disclosure may be found in the Footnotes and Disclosures at the end of this article.

This case report discusses a diagnosis of acute annular outer retinopathy in a 29-year-old female patient who presented with recent onset of visual field defect and photopsia.

Acute Zonal Occult Outer Retinopathy (AZOOR) is characterized by sudden visual impairment, often presenting with scotoma and photopsia in young to middle-aged adults, showing a female predominance. This condition, distinct from genetic disorders like retinitis pigmentosa, lacks a hereditary basis and exhibits unique fundus changes and imaging features indicative of outer retinal dysfunction. Recent advancements have broadened our understanding, identifying variants like Acute Annular Outer Retinopathy (AAOR) and Multizonal outer retinopathy and retinal pigment epitheliopathy (MORR), each with specific clinical presentations and imaging characteristics. The diagnosis of AZOOR and its variants primarily relies on excluding other conditions through comprehensive evaluation, including imaging and serological testing. Treatment approaches, including the use of corticosteroids and immunosuppressives, remain debated, with some evidence suggesting benefits in the early stages. The prognosis of AZOOR varies, with most patients experiencing stabilization, although complications like choroidal neovascularization may occur, requiring targeted therapy. This manuscript elucidates the complexity of AZOOR, emphasizing the necessity of high clinical suspicion and the role of advanced imaging in diagnosis and management.