VISUAL IMPAIRMENT IN RETINITIS PIGMENTOSA.

Area of Science:

• Ophthalmology
• Genetics
• Retinal Dystrophies

Background:

• Retinitis pigmentosa (RP) is a group of inherited retinal diseases causing progressive vision loss.
• Patients with RP often experience declining peripheral, night, and central vision.
• Previous literature suggested a high risk of total blindness in RP patients.

Purpose of the Study:

• To investigate the progression of visual acuity loss in a large cohort of retinitis pigmentosa (RP) patients.
• To evaluate the prevalence of severe vision loss, including no light perception, in RP.
• To examine the accuracy of existing literature statements regarding blindness in RP.

Main Methods:

• Retrospective cross-sectional analysis of 1,095 RP patients' medical records.
• Exclusion of patients with sector RP or delimited forms of the disease.
• Focus on patients with 20/200 or worse vision in the better-seeing eye (n=215).

Main Results:

• Only 0.46% of the total RP cohort progressed to no light perception in either eye.
• 92% of all examined RP patients maintained the ability to read a visual acuity chart.
• 6.8% of patients had severely impaired vision, perceiving only hand motion, count fingers, or light perception.

Conclusions:

• The risk of progressing to total blindness (no light perception) is very low in retinitis pigmentosa.
• The majority of RP patients in this cohort retained some level of functional vision.
• Findings challenge previous assertions about the inevitability of total blindness in RP.