Abstract
Purpose::
To estimate the overall prevalence, prevalence by genetic type, and effect on visual acuity of macular cysts in patients with retinitis pigmentosa.
Methods::
We measured visual acuities with a projected Snellen chart and with Early Treatment Diabetic Retinopathy Study (ETDRS) charts and searched for macular cysts with optical coherence tomography in 316 patients with typical forms of retinitis pigmentosa.
Results::
We documented macular cysts in 28% of the patients, 40% of whom had cysts in only one eye. Macular cysts were found most often in patients with dominant disease and least often in patients with X-linked disease (p = 0.006), a variation that could not be explained by age differences. In eyes with macular cysts, both Snellen and ETDRS acuities were inversely related to central foveal thickness (p = 0.002 and p = 0.03, respectively). On average, visual acuity decreased by about 2 letters for each 100 µm increase in retinal thickness.
Conclusions::
Macular cysts are a common occurrence in retinitis pigmentosa, especially in patients with dominantly-inherited disease. On average, cysts cause a mild impairment in visual acuity that increases with increasing retinal thickness.
Keywords: retinal degenerations: hereditary • macula/fovea • imaging/image analysis: clinical