Abstract

Retinitis pigmentosa (RP), a degenerative disorder presenting with a wide variety of ocular and systemic manifestations, is a condition first reported in 1857. Over more than a century of research, strong evidence has emerged of zonular weakness in RP, as indicated by pre-operative and intra-operative phacodonesis, as well delayed post-operative intraocular lens displacement. The occurrence of bilateral subluxation of lens in a patient with RP has seldom been reported.
We report a late presentation of bilateral inferior subluxation of lens, with visible elongated superior zonular fibres, associated with the classic triad of RP. The patient had no systemic abnormalities suggestive of a syndromic presentation. This appears to be the second case of bilateral concurrent spontaneous ectopia lentis associated with retinitis pigmentosa, with no familial inheritance or syndromic association. It serves as a wake-up call for urgent need of further research in understanding the etiology of RP.