X-Linked Retinoschisis

Macula photographs and red free images (right - top, left - bottom)

Fundus photos show a radial spoke-wheel pattern centered on the fovea which is characteristic of X-linked retinoschisis.

Fundus photograph and red-free (right eye)

Fundus photograph and red-free (left eye)

Spectralis OCT volume and line scans (Right eye)

OCT reveals retinoschisis of both the inner and outer nuclear layers involving the entire posterior pole in each eye, most marked in the macular area. The retinoschisis cavities have coalesced within the foveal area in each eye to form a cyst-like cavity. There is mild disturbance of ellipsoid zone at the fovea.

Spectralis OCT volume and line scans (left eye)

OCT reveals retinoschisis of both the inner and outer nuclear layers involving the entire posterior pole in each eye, most marked in the macular area. The retinoschisis cavities have coalesced within the foveal area in each eye to form a cyst-like cavity. There is mild disturbance of ellipsoid zone at the fovea.

Fundus photographs (right and left eye)

Optomap widefield images (right and left eye)

Widefield imaging shows retinoschisis in the inferior periphery of the right eye.

Optomap image of the Inferior fundus (right eye)

A magnified image of the inferior retina shows the retinoschisis extending out to the far periphery. There are areas of chorioretinal atrophy, abnormal vitreoretinal attachments, and dendriform vessels.

Fundus autofluorescence images (right and left eye)

FAF imaging shows a hyper-fluorescent ring around each macula

Spectralis OCT macular line scans (right and left eye)

Both maculae show a notable loss of the outer nuclear layer, external limiting membrane, and ellipsoid zone. More pronounced hyporeflective schitic cavities were noted in the left than the right.

Spectralis OCT line scans through an area of inferior retinoschisis

OCT shows abnormal vitreoretinal attachments and traction in the area of schisis inferiorly in the right eye.