Acute zonal occult outer retinopathy: towards a set of diagnostic criteria

• Published in: British journal of ophthalmology Vol. 89; no. 1; pp. 70 - 73
• Main Authors: Francis, P J, Marinescu, A, Fitzke, F W, Bird, A C, Holder, G E
• Format: Journal Article
• Language: English
• Published: BMA House, Tavistock Square, London, WC1H 9JR BMJ Publishing Group Ltd 01.01.2005; BMJ; BMJ Publishing Group LTD; Copyright 2005 British Journal of Ophthalmology
• Subjects: Acute Disease; acute idiopathic blind spot enlargement syndrome; acute macular neuroretinitis; acute zonal occult outer retinopathy; Adult; Aged; AIBSE; AMN; AZOOR; Biological and medical sciences; Clinical Science - Extended Reports; electro-oculography; Electrooculography - methods; electrophysiology; electroretinogram; Electroretinography - methods; EOG; ERG; Female; Fundus Oculi; Humans; Light; Male; MCP; Medical sciences; MEWDS; MIC; Middle Aged; multifocal choroiditis with panuveitis; multifocal inner choroiditis; multiple evanescent white dots syndrome; Ophthalmology; P-POHS; Patients; pattern ERG; PERG; PIC; pseudo-presumed ocular histoplasmosis syndrome; punctate inner choroiditis; Retina; Retinal Diseases - diagnosis; Retinal Diseases - pathology; Retinal Diseases - physiopathology; Retinopathies; Retrospective Studies; Scotoma - physiopathology; Syndrome; Vision Disorders - physiopathology; Eye disease; Retinopathy; Diagnosis; Outer; Ophthalmology; Acute
• ISSN: 0007-1161; 1468-2079
• DOI: 10.1136/bjo.2004.042416

Background: Individuals with acute zonal occult outer retinopathy (AZOOR) present with initially progressive scotomata and photopsia. Characteristically, the extent of the visual field defect is unexplained by fundal examination, but there is marked retinal dysfunction evident electrophysiologically. It is the authors’ experience that a group of patients exhibit characteristic clinical and electrophysiological abnormalities, which serve as criteria for a working diagnosis. Methods: A retrospective observational case series of 28 patients were identified with the clinical diagnosis of AZOOR who shared similar abnormal electrophysiology. Details of the history and ophthalmic findings were obtained from the case notes. Results: Electrophysiology demonstrated a consistent pattern of dysfunction both at the photoreceptor/retinal pigment epithelial complex but also at inner retinal levels, essentially comprising a delayed 30 Hz flicker ERG and a reduction in the EOG light rise. Conclusion: This study determines diagnostic criteria applicable to a group of patients with AZOOR, typically those with classic symptomatology. Electrophysiological testing can help avoid lengthy, costly, and potentially invasive investigations, and the unnecessary use of immunosuppressive therapy.