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DOI: 10.1055/a-2730-1186
Acute Zonal Occult Outer Retinopathy after Cataract Surgery
Akute zonale okkulte äußere Retinopathie nach KataraktoperationAuthors
Background
Acute zonal occult outer retinopathy (AZOOR) is a rare retinal disorder first described by Gass in 1992 [1]. It presents with sudden scotomas and photopsia, predominantly affecting young myopic women and often progressing from unilateral to bilateral involvement. Diagnosis relies on multimodal imaging and functional testing, with characteristic findings on optical coherence tomography (OCT), fundus autofluorescence (FAF), and electroretinography (ERG). ERG typically shows impaired photoreceptor function, and retinal pigment epithelium atrophy develops over time. While the pathogenesis remains unclear, autoimmune and viral triggers have been suggested, supported by associations with autoimmune diseases and antiretinal antibodies [2], [3].
Publication History
Received: 24 August 2025
Accepted: 20 October 2025
Article published online:
02 December 2025
© 2025. Thieme. All rights reserved.
Georg Thieme Verlag KG
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References
- 1 Gass JD. Acute zonal occult outer retinopathy. Donders lecture – The Netherlands Ophthalmological Society, Maastricht, Holland. J Clin Neurol Ophthalmol 1993; 13: 79-97
- 2 Forooghian F. Prevalence of Antiretinal Antibodies in Acute Zonal Occult Outer Retinopathy: A Comprehensive Review of 25 Cases. Am J Ophthalmol 2017; 179: 210-211
- 3 Gass JD. Are acute zonal occult outer retinopathy and the white spot syndromes (AZOOR complex) specific autoimmune diseases?. Am J Ophthalmol 2003; 135: 380-381
- 4 Guijarro A, Munoz N, Alejandre N. et al. Long term follow-up and effect of immunosuppression in acute zonal occult outer retinopathy. Eur J Ophthalmol 2020;
- 5 Chen SN, Yang CH, Yang CM. Systemic corticosteroids therapy in the management of acute zonal occult outer retinopathy. J Ophthalmol 2015; 2015: 793026
- 6 Kitakawa T, Hayashi T, Takashina H. et al. Improvement of central visual function following steroid pulse therapy in acute zonal occult outer retinopathy. Doc Ophthalmol 2012; 124: 249-254
- 7 Lin BR, Russell JF, Al-Khersan H. et al. A systematic review of acute zonal occult outer retinopathy with a focus on attempted treatment modalities. Curr Ophthalmol Rep 2022; 10: 168-178
- 8 Neri P, Ricci F, Giovannini A. et al. Successful treatment of an overlapping choriocapillaritis between multifocal choroiditis and acute zonal occult outer retinopathy (AZOOR) with adalimumab (Humira). Int Ophthalmol 2014; 34: 359-364
- 9 Fujiwara T, Imamura Y, Giovinazzo VJ. et al. Fundus autofluorescence and optical coherence tomographic findings in acute zonal occult outer retinopathy. Retina 2010; 30: 1206-1216
- 10 Mrejen S, Khan S, Gallego-Pinazo R. et al. Acute zonal occult outer retinopathy: a classification based on multimodal imaging. JAMA Ophthalmol 2014; 132: 1089-1098
- 11 Trese MGJ, Cohen SR, Besirli CG. Recovery of outer retina in acute idiopathic blind spot enlargement (AIBSE). American Journal of Ophthalmology Case Reports 2016; 1: 13-15
- 12 Horton JC, Parker AB, Botelho JV. et al. Spontaneous Regeneration of Human Photoreceptor Outer Segments. Sci Rep 2015; 5: 12364
- 13 Matsui Y, Matsubara H, Ueno S. et al. Changes in outer retinal microstructures during six month period in eyes with acute zonal occult outer retinopathy-complex. PLoS One 2014; 9: e110592
- 14 Gass JD, Agarwal A, Scott IU. Acute zonal occult outer retinopathy: a long-term follow-up study. Am J Ophthalmol 2002; 134: 329-339
