Retinitis pigmentosa: clinical observations and correlations.

Abstract

This thesis presents the results of a study of 384 eyes of 192 patients with a mean age of 39.1 years who presented with typical retinitis pigmentosa. The major findings are outlined below, together with suggested hypotheses: Cataract was found in 46.4% of the eyes. Among these, 93.6% showed posterior subcapsular opacification. The incidence of cataract increased with age. The vitreous degeneration that is characteristic of the RP syndrome and begins in childhood was described as showing dust-like, particulate matter throughout the gel; posterior vitreous separation; formation of a posterior matrix of coarse, white, interconnected strands and opacities; and final collapse of the residual gel. Ultrastructural studies of vitreous material from eight eyes revealed that the particles were isolated pigment granules and the coarse strands were composed of condensed collagen fibers. Notwithstanding the vitreous degeneration and prevalence of myopia in RP, neurosensory retinal breaks and/or rhegmatogenous detachment were found in only 7 (1.8%) of the 384 eyes studied. Premature separation of the vitreous from the retina, absence of lattice retinal degeneration, and perhaps a stronger than normal RPE-neurosensory retinal bond are thought to be possible protective factors. Rather than searching for a "toxin," elaborated by diseased retina, that causes vitreous degeneration and cataract formation, it is suggested that the ocular media be studied for an absence of moieties that are normally produced by healthy retina for vitreous and lens maintenance. The classic criteria for diagnosis of RP were met by 96.3% of eyes that showed retinal vascular attenuation and by 52.0% that showed pallor of the optic disc. Less frequent manifestations included solitary retinal hemorrhage, peripheral microaneurysms, telangiectasia, and fluorescein leakage at the macula and disc. Seven additional cases with a Coats'-like retinal detachment were added to the 14 already presented in the literature. Two of the seven had autosomal dominant RP, the first such cases reported. The vascular malformations and detachments were most often inferior. Unlike typical Coats' syndrome, the condition was usually bilateral, showed no sex preference, and appeared to affect older individuals.(ABSTRACT TRUNCATED AT 400 WORDS)

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