INCIDENCE AND TYPES OF CATARACTS IN PATIE NTS WITH RETINITIS-PIGMENTOSA

Purpose: To study the incidence and types of cataract in retinitis pig mentosa (RP) and their variations among different forms of RP. Patient s and methods: This analysis was based on data from 473 patients with RP (autosomal dominant, n=87; autosomal recessive, n=79; x chromosomal recessive, n=23; simplex RP, n=215; Usher's syndrome n=80; M. Refsum and others, n=9) that were retrieved from the literature and patient c harts in our clinic. Results: Posterior subcapsular cataract(PSC) deve loped with the following frequencies for the different genetic types o f RP: autosomal dominant, 45.3%; autosomal recessive, 44.0%; x chromos omal recessive, 40.7%; simplex RP,46.1%; Usher's syndrome, 52.9%. PSC was the only type of lens opacity in patients with Usher's syndrome an d autosomal recessive RP. PSC development correlated with early onset of RP symptoms. Nuclear cataracts showed a statistically significant h igher frequency in patients with simplex RP (14.8%) than in other gene tic types (0-5.9%) (P < 0.01). In addition, nuclear cataracts develope d in simplex RP at a significantly later age (69.6+/-12.4 years) than PSC (44.4+/-12.3 years) (P < 0.001). Patients with cataracts showed si gnificantly worse visual fields than patients with clear lenses (P=0.0 0067). Conclusions: The typical RP cataract(PSC) was found in similar frequencies among all genetic types of RP. PSC was the only type of le ns opacity in patients with Usher's syndrome and autosomal recessive R P. Nuclear cataracts developed on average 20 years later than PSC and had their highest incidence in patients with simplex RP. Patients with cataracts showed significantly worse visual field results, indicating a more pronounced retinal pathology.