Abstract
Purpose: :
To determine visual acuity loss in patients with autosomal recessive retinitis pigmentosa as it relates to the presence of macular lesions.
Design: :
Retrospective chart reviews.
Methods: :
One hundred and forty five patients with retinitis pigmentosa who either had a positive family history of parental consanguinity or an affected sister as a criterion for recessive inheritance were included in the study. Best corrected visual acuities at the most recent visit and macular status of the better eye were used for data analysis. The macular status was classified as either having no lesion, a bull’s eye-appearing atrophic lesion, or a geographic atrophic lesion. Those with no lesions included patients with a blunted foveal reflex, an epiretinal membrane or mild non-specific pigment mottling. A bull’s eye lesion was defined as a reddish-brown lesion in the fovea accompanied by surrounding retinal pigment epithelial (RPE) hypopigmentation. A geographic atrophic lesion was described as a homogenous hypopigmented degenerative change in the macula that included the fovea.
Results: :
Regardless of age, 68 (47%) patients had visual acuity of 20/40 or better, 95 (66%) 20/70 or better, and 109 (75%) had better than 20/200 in at least one eye. . In our population of 145 patients, 36 (25%) were considered legally blind based on the visual acuity criterion of 20/200 or worse in the better eye, and 15 of these patients had either hand motion, count fingers or light perception vision. None of the patients in our cohort were found to have no light perception in both eyes. An evaluation of the macular status (n = 132 eyes) demonstrated that 55 (41.6%) patients had no macular lesion, 26 (19.7%) had a bull’s eye-appearing lesion, and 51 (38.6%) had a geographic atrophic lesion. All patients with no macular lesion had a visual acuity better than 20/200, while 41 (53%) patients who had an atrophic lesion (either bull’s eye or geographic) had an acuity better than 20/200.
Conclusions: :
These data can be used to counsel patients on the ranges of potential visual acuity impairment likely to be observed in different age groups.
Keywords: retinal degenerations: hereditary