Case #25 – Retinitis Pigmentosa

Retinitis Pigmentosa – Page 19 of 49

Case 1: Patient vs. Normal Control Flash Electroretinogram (ERG) of Left Eye The flash electroretinogram (ERG) is the single best test of overall retina function. Retinitis pigmentosa patients have a reduced rod and cone response, which is sometimes unmeasurable 1 ERG results often show retinal function is diminished several years before night blindness, visual

Retinitis Pigmentosa – Page 19 of 492021-11-30T22:46:55+00:00

Retinitis Pigmentosa – Page 18 of 49

Case 1: Patient vs. Normal Control Topcon 3D OCT Radial Scan Image of Left Eye The presence of the PIL under the fovea predicts the patient's normal visual acuity. The absence of the PIL outside the macula predicts the visual field loss.

Retinitis Pigmentosa – Page 18 of 492021-11-30T22:46:47+00:00

Retinitis Pigmentosa – Page 17 of 49

Case 1: Topcon 3D OCT Horizontal Scan Images of Left Eye Similar findings were noted in the left eye. Only a small segment Of the PIL was present under the fovea and the RPE appeared attenuated.

Retinitis Pigmentosa – Page 17 of 492021-11-30T22:46:41+00:00

Retinitis Pigmentosa – Page 15 of 49

Case 1: Topcon 3D OCT Horizontal Scan Images of Right Eye The horizontal OCT scans superior (top image) and inferior (bottom image) to the macula reveal an absent PIL. The only region where the PIL was present was present was a small segment under the fovea (middle image).

Retinitis Pigmentosa – Page 15 of 492021-11-30T22:46:27+00:00

Retinitis Pigmentosa – Page 14 of 49

The Osterberg Graph Only 5% of photoreceptors in the retina are cones, and these are concentrated at the fovea. As reported by Osterberg in 1935, the density of cones drops off rapidly (but not abruptly) just several degrees from the fovea, and rods begin to predominate outside of the central 5 degrees or so.

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Retinitis Pigmentosa – Page 13 of 49

Histological Section as Compared to the OCT Image  Although the photoreceptor integrity line, or the PIL (defined as the Junction between the inner and outer segments) is barely visible in most histological sections, it is highly prominent in normal SD OCTs. The PIL, as shown above, should be continuous throughout the entire scan

Retinitis Pigmentosa – Page 13 of 492021-11-30T22:46:14+00:00