PMC2724032_01
Case 1 Findings in a 23-year-old woman with AZOOR at onset (kindly provided by Professor Hiroyuki Iijima of Yamanashi University Hospital). A) Fundus photograph of posterior pole of the right eye. Fundus appears normal. B) Visual field defect characterized by enlargement of blind spot and deep scotomas in the inferior and nasal visual field of her right eye.
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OD and 1.5 OS, and the anterior segment was normal in both eyes. Ophthalmoscopy of her right eye showed a nearly normal fundus except for a white oval lesion of about one disc diameter near the temporal vascular arcade (Figure 1A). Perimetry detected an enlargement of the blind spot and deep scotomas in the inferior and nasal visual field of her right eye (Figure 1B).
Case 1 Findings one year later. A) Nearly normal panoramic fundus photograph of the right eye. B) Persistent visual field defect characterized by enlargement of blind spot and scattered scotomas. C) Single-flash full-field electroretinograms (ERGs) were within the normal limits in both eyes. D) Flicker (30-Hz) ERG from both eyes showing a reduction in the amplitude and significant phase delay in the amplitude in the right eye.
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The fundi were normal except for a small pigmented lesion in the right eye (Figure 2A). However, the scotoma that was connected to the blind spot and the nasal scotomas were still present (Figure 2B). The 30-Hz flicker ERGs, but not the full field ERGs were slightly reduced in the right eye (Figures 2C and 2D).
Case 1 Findings one year later. A) Optical coherence tomographic image of a 6-mm horizontal scan showing the loss of middle line and slightly decreased IS/OS line (arrow). B) Retinal volume map of the right and left eyes. The retina in right eye is thinner than that of the fellow eye by volume scan analysis. Values underlined = reduced by <10% and by dotted lines by <3%.. Abbreviations: S, superior; T, temporal; I, inferior; N, nasal.
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Examination by spectral domain optical coherence tomography (SD-OCT; Spectralis OCT; Heidelberg Engineering, Heidelberg, Germany) showed that the IS/OS line was not distinct with an absence of the middle line between the IS/OS and retinal pigment epithelium (RPE) in the temporal area of the macula but not in the fovea of the right eye (Figure 3A). The results of this volume scan clearly showed a decrease in the mean retinal thickness in the superior and temporal areas of the nine areas corresponding to the Early Treatment Diabetic Retinopathy Study (ETDRS) areas (Figure 3B).
PMC2724032_02
Case 2 Findings in a 29-year-old woman with AZOOr at onset. A) Nearly normal fundus photograph of posterior pole of the left eye. B) Dense superior visual field defects. C) Topographic map of multifocal ERGs showing markedly reduced responses in the macular area. D) Time-domain optical coherence tomography image showing a reduction of the outer nuclear layer.. Abbreviations: S, superior; I, inferior.
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The anterior segment and ophthalmoscopic findings appeared normal (Figure 4A) except for an old focal retinal detachment without macular involvement in the inferotemporal area of the left eye. A dense visual field defect was detected in the superior field (Figure 4B). The multifocal ERGs recorded from the macular area of the left eye were reduced (Figure 4C). Time-domain OCT (OCT3000; Zeiss, Oberkochen, Germany) showed an irregularity or loss of the IS/OS line in the fovea and a reduction in the thickness of the outer nuclear layer (ONL) (Figure 4D).
Case 2 Panoramic fundus photograph of left eye four years after onset. The localized retinal detachment (*) is well-demarcated by pigmentation (arrow).
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The localized retinal detachment was not altered (Figure 5).
Case 2 Findings four years after onset. A) Spectral domain optical coherence tomography image of a 6-mm vertical scan showing the attenuated IS/OS line (arrow) and severely decreased outer nuclear layer (*), outer plexiform layer and inner nuclear layer. B) Decreased retinal thickness in left eye compared with fellow eye by volume scan analysis. Underlines; decreased less than 10% or red line; less than 40%.. Abbreviations: S, superior; T, temporal; I, inferior; N, nasal.
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SD-OCT showed a greater loss of the IS/OS line, and an alteration of the outer nuclear layers especially in the inferior macular area (Figure 6A). The retinal thickness was markedly reduced with a decrease of both the outer and inner nuclear layers (Figure 6B).