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M. Mkrtchyan, S. M. Sundquist, A. Solovyev, B. J. Lujan, Y. Zhang, C. E. Thirkill, J. L. Duncan, A. Roorda; Retinal Heterogeneity of Patients with AZOOR. Invest. Ophthalmol. Vis. Sci. 2009;50(13):348.
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© ARVO (1962-2015); The Authors (2016-present)
To correlate visual function with high-resolution structural imaging in patients with Acute Zonal Occult Outer Retinopathy (AZOOR).
Best-corrected visual acuity (VA), Goldmann kinetic and automated perimetry and fundus-guided microperimetry, full-field and multifocal electroretinography (ffERG and mfERG), spectral domain optical coherence tomography (SDOCT) and Adaptive Optics Scanning Laser Ophthalmoscopy (AOSLO) imaging were used to correlate retinal structure and function in 4 patients with AZOOR.
4 female patients, aged 18-51, presented with acute focal loss of visual field or visual acuity, photopsia, minimal funduscopic changes, and electroretinographic abnormalities. Scotomata and photopsias were initially progressive, then persistent, and were unilateral in 2 and bilateral in 2 patients. 1 of the 4 had a history of autoimmune hepatitis and thyroiditis; there was no history of autoimmune disease in the other 3 patients. In 2 patients macular disease expression was symmetric, while in 2 interocular asymmetry was observed. MfERG response densities were decreased with delayed timing in the affected areas and ffERG amplitudes showed interocular asymmetry in 3 of the 4 patients. Structural properties of the cones were imaged in all subjects using AOSLO and compared with unaffected regions in the same eye, the unaffected eye and 20 normal eyes. All patients had retinal function loss that correlated with structural changes. 3 patients had structural changes in the optical components of the photoreceptors, where discrete regions of increased cone spacing with irregular cone packing and abnormal retinal morphology on SDOCT corresponded to visual field defects and mfERG abnormalities. In the fourth patient, who had severe loss of VA, a dense central scotoma and reduced mfERG responses, the main difference was relative thinning of the outer nuclear layer in the affected eye. The laminar appearance of the optical components of the photoreceptors (ELM-RPE), cone spacing and cone packing arrangement were normal despite loss of visual function.
AOSLO imaging demonstrated anatomical heterogeneity among patients with the clinical diagnosis of AZOOR. In all patients abnormalities in retinal structure corresponded to reduced visual function, but in 1 patient the structural changes were not observed in the optical component of the photoreceptors.
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