PMC3046995_01
Fundus appearance of the right A) and left B) eyes. The bone spicule deposits are clustered just anterior to the posterior pole. The cystoid macular edema is unremarkable in this photograph of both eyes. The white line indicates the optical coherence tomography scan line in Figure 4.
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Typical fundus changes of the bone spicule intraretinal pigmentation in the mid-periphery and attenuated retinal vessels (Figure 1) and almost extinguished electroretinogram (ERG) recordings of all responses by portable ERG (LE-3000 ; Tomey Corporation, Nagoya, Japan), including single flash, cone, rod, and flicker (Figure 2), strongly supported the previous diagnosis of RP.
The electroretinogram (ERG) of the current case compared with that of a normal eye. The patient has no ERG responses, including flash, cone, rod, and 30-Hz flicker.
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Typical fundus changes of the bone spicule intraretinal pigmentation in the mid-periphery and attenuated retinal vessels (Figure 1) and almost extinguished electroretinogram (ERG) recordings of all responses by portable ERG (LE-3000 ; Tomey Corporation, Nagoya, Japan), including single flash, cone, rod, and flicker (Figure 2), strongly supported the previous diagnosis of RP.
Goldmann kinetic perimetry indicates the marked constriction of the V-4 isopter and the inferior peripheral remaining visual field.
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Goldmann kinetic perimetry showed extensive bilateral visual field loss (Figure 3).
Spectral domain optical coherence tomography images of the right A) and left B) eyes. In the right eye, cystoid macular edema (CME) at the fovea and posterior vitreoschisis (arrow) in the nasal quadrant is seen without a posterior vitreous detachment (PVD). In the left eye, minimal CME at the fovea with a focal PVD (arrowhead) and posterior vitreoschisis (arrow) in the nasal quadrant are seen.
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Seven years after the first visit, SD-OCT (Cirrus  HD OCT; Carl Zeiss Meditec, Dublin, CA) (Figure 4) showed CME and no evidence of PVD but posterior vitreoschisis in the nasal quadrant bilaterally. In the right eye (Figure 4A), CME was evident, and no vitreofoveal separation was observed. However, in the left eye (Figure 4B), minimal changes were seen in the CME associated with a focal shallow PVD over the fovea.