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Photo Essay |

Outer Retinal Corrugations in X-Linked Juvenile Retinoschisis

Anita Agarwal, MD; Usha Sathish Rao, BS
Arch Ophthalmol. 2007;125(2):278-279. doi:10.1001/archopht.125.2.278.
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A 19-year-old man (sibling A) had a decrease in central vision for a few weeks in December 2004. His best-corrected visual acuity was 20/80 OD and 20/60 OS. Fundus examination showed bilateral foveal schisis and gray-white folds in the outer retina temporal to the fovea in the right eye only (Figure 1A and C). A fluorescein angiogram showed parallel hyperfluorescent lines corresponding to the folds (Figure 1B). In April 2006, the folds in the right eye had changed in location and orientation (Figure 2A).The left fundus showed outer retinal folds that were not present previously (Figure 3A). An optical coherence tomography scan through the folds corroborated their location in the outer retina (photoreceptors and outer plexiform layers) (Figure 2B).An optical section through the fovea confirmed the schisis cavity (Figure 3B).

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Figure 1.

A, Right fundus photograph depicting macular schisis and temporal gray-white “flying seagull”–like corrugations in sibling A in December 2004 (arrow). B, Fluorescein angiogram showing hyperfluorescent lines corresponding to the corrugations (arrow).C, Fundus photograph of the left eye with “bicycle spoke”–like appearance of the foveal schisis and absence of corrugations.

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Figure 2.

A, Change in the distribution and orientation of the folds in sibling A in April 2006 (arrows) (compare with Figure 1A). B, Optical coherence tomography through the corrugations confirming their location in the outer retina (photoreceptor and outer plexiform layers; arrows). C, Location of the retinal corrugations corresponding to the optical coherence tomography scan (arrows).

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Figure 3.

A, Appearance of new-onset corrugations in the left eye in sibling A in April 2006 (compare with Figure 1C). B, Optical coherence tomography through the foveal schisis cavity showing loculations at multiple levels in the left eye in sibling A.

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Figure 5.

Disappearance of the corrugations in sibling B in April 2006.

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Figure 4.

Left fundus photograph with macular schisis and gray-white corrugations temporal to the schisis cavity in sibling B in 2000.

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