Investigation of Unilateral and Asymmetric Pigmentary Retinopathy

Abstract

Purpose:: To present a retrospective evaluation of patients seen with the diagnosis of unilateral RP (pigmentary retinopathy) or asymmetic RP, to evaluate etiologies, and to examine the phenotype of cases seen over a 22-year period.

Methods:: From a database of 3000 RP patients, patients were identified who had unilateral or asymmetric pigmentary retinopathy. Clinical data was reviewedincluding standardardized electroretinography, Goldmann visual fields, photography, and clinical examinations. Cases were categorized into three groups: (1) unilateral pigmentary retinopathy, in which one eye had a normal visual field and the other eye typical RP; group (2) with asymmetric pigmentary retinopathy, visual fields are asymmetric, with the lesser eye measuring no more than 50% of the greater; group (3) patients initially with unilateral disease who progressed to asymmetric disease.

Results:: Thirty-eight cases were identified with unilateral (17 case) or asymmetric pigmentary retinopathy (21 cases), 29 females and 9 males (average 38.4 and 51.1 years respectively). In four cases of unilateral RP, the good eye later developed a pigmentary retinopathy such that the cases could be re-classified as asymmetric pigmentary retinopathy. Most cases were idiopathic, but five cases had documented head trauma, 3 cases various forms of chorioretinitis, 2 cases of neonatal issues, and one X-linked RP carrier with asymmetric findings. The mean visual acuity in the worse affected eye was 20/30 (range 20/20 to 20/400) while the better eyes mean was 20/20. Other than the X-linked carrier, no hereditary forms of unilateral or asymmetric RP were found.

Conclusions:: Presenting cases of unilateral or asymmetric pigmentary retinopathy are generally female in their 30 and 40’s, while males are older and tended to have worse central vision. Most cases were idiopathic, and head trauma could be documented in a number of cases. No positive family histories were found in any of these patients except one X-linked carrier.

Keywords: retinal degenerations: hereditary