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H. F. Fine, E. Kim, R. F. Spaide, L. A. Yannuzzi; Characterization of Type 3 Acute Zonal Occult Outer Retinopathy. Invest. Ophthalmol. Vis. Sci. 2008;49(13):971.
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Recently, Yannuzzi and colleagues described a revised classification system for acute zonal occult outer retinopathy (AZOOR). Type III AZOOR in this classification scheme includes those cases which initially present as a white spot syndrome. The purpose of this study is to characterize the clinical, angiographic, electrophysiologic, and autofluorescence findings of patients with Type III AZOOR.
A multi-center, retrospective review was performed to gather available documentation on cases of purported AZOOR. Cases were reviewed by a single examiner to determine if they met the clinical and imaging criteria for AZOOR.
Of all reviewed cases, 161 met the diagnostic criteria of AZOOR. Three cases had evidence of a preceding white spot syndrome and were classified as Type III. The age range was 29 - 51 years. These cases all resembled the multifocal evanescent white dot syndrome (MEWDS) on presentation, with small white migratory spots about the posterior pole that resolved leaving residual foveal granularity. In one patient, the pattern of hyperautofluorescence on presentation coincided with the subsequent area of involvement of AZOOR. In another, the syndrome followed placement of a purified protein derivative (PPD) skin test. All cases of Type III AZOOR developed zones of photoreceptor and retinal pigment epithelial atrophy, progressive visual field loss, and electroretinographic abnormalities.
Antecedent white spot syndromes were rare (1.9%) in a series of 161 patients diagnosed with AZOOR. Autofluorescence findings in MEWDS can foreshadow the development of AZOOR.
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