Improvement of central visual function following steroid pulse therapy in acute zonal occult outer retinopathy

Abstract

The purpose of this study was to report a patient with acute zonal occult outer retinopathy (AZOOR) unilaterally, who received steroid pulse therapy. A 42-year-old woman presented with photopsias and severe vision loss in her left eye. Visual acuity was 0.04, and Humphrey visual field testing showed overall depression with a mean deviation (MD) value of -25.78 dB in the left eye. Fundus and angiographic examinations found no specific abnormal findings, leading to a diagnosis of AZOOR. Optical coherence tomography showed attenuation of the photoreceptor inner segment/outer segment junction (IS/OS) line. Focal macular electroretinography (fmERG) demonstrated that there were non-detectable responses at 5°, 10° and 15° (in diameter). Following steroid pulse therapy, her visual acuity was 1.0, her MD value improved to -16.08 dB, and there were both partial recovery of the IS/OS line and apparent improvements of fmERG responses (at 10° and 15°). The present findings suggest that steroid pulse therapy might potentially be an effective treatment in some AZOOR patients.

Changes in Optical Coherence Tomography Findings in Acute Zonal Occult Outer Retinopathy

“…Our OCT findings were similar to those previously reported for other AZOOR patients [3, 4, 5, 6, 7, 8, 9, 10, 11, 12, 13, 14, 15]. It has been reported that the natural course of AZOOR is varied [1, 2].…”

“…There have been several detailed reports about AZOOR patients who showed morphological and functional improvements in their visual function [4, 8, 9, 10, 11, 12, 13, 14, 15]. Spaide et al [4] reported restoration of the IS/OS line in the improved areas of the visual field, but electrophysiological alterations of these patients were not demonstrated in the report.…”

“…To our knowledge, there are few reports in the literature describing the abnormalities of the COST line in AZOOR [8]. However, there are several detailed reports about AZOOR patients who showed subjective and objective improvements in visual function [4, 8, 9, 10, 11, 12, 13, 14, 15]. Here, we report an AZOOR patient with partial restoration of the IS/OS line.…”

“…Initially, minimal or no fundus changes occur, but enlarged blind spots, abnormalities on electroretinograms (ERGs) and permanent visual field loss often occur in this slowly progressing form of retinal pigment epithelium degeneration [1, 2]. Optical coherence tomography (OCT) images from eyes with AZOOR show a loss or irregularity of the photoreceptor inner segment/outer segment (IS/OS) line in areas corresponding to reduced multifocal ERG (mfERG) responses and visual field defects [3, 4, 5, 6, 7, 8, 9, 10, 11, 12, 13, 14, 15]. Recently, the cone outer segment tip (COST) line has been recognized as a thin, highly reflective line located between the IS/OS junction and the retinal pigment epithelium [16].…”

Paraneoplastic and non-paraneoplastic retinopathy and optic neuropathy: Evaluation and management

“…Some experts feel that AZOOR is a unique entity that may be more precisely defined by multimodal imaging: 1) autofluorescence, demonstrating characteristic zonal degeneration of the outer retina and RPE, typically beginning around the nerve and progressing to the periphery, and 2) high-definition spectral domain OCT, showing zonal or annular loss of the photoreceptor layer and IS/OS complex. 81,143,184,199,263,267 5.2.…”