Absence Of Retinal Pigment Epithelium Research Articles

Effects of Repeated Photic Exposures on the Monkey Macula

The posterior pole of the eyes of rhesus monkeys was exposed to the light of an indirect ophthalmoscope. The tissue changes caused by repeated (three to five) photic injuries were compared with those produced by a single photic insult. The ophthalmoscopic and angiographic examinations showed a progressive involvement of the foveal area after multiple exposures to light. Histopathologic study showed that after one exposure the retinal pigment epithelium appeared focally atrophic. Plaques of proliferated retinal pigment epithelium could also be seen, and on the anterior surface of these plaques a layer of relatively cuboidal hypopigmented retinal pigment epithelial cells was present. With multiple exposures, focal areas of total absence of retinal pigment epithelium were seen in these eyes. The eyes that received only one exposure showed partially regenerated photoreceptor outer segments. After repeated photic exposures, the photoreceptor outer segments failed to regenerate even 6 to 8 months after the last exposure. In one eye exposed five times for 60 minutes, areas of total absence of photoreceptor cells could be seen, and the inner nuclear layer was directly apposed to Bruch's membrane. These experimental exposures to the light of an indirect ophthalmoscope were 60 to 120 minutes long and were not comparable to clinical exposures of the ophthalmoscope in patient examination. Our observation highlighted the cumulative effects of repeated light injury to the retinal pigment epithelial and photoreceptor cells and also showed the similarity between this animal model of photic maculopathy and the changes present in the eyes of human patients with atrophic agerlated macular degeneration.

Choroideremia: A Clinical, Electron Microscopic, and Biochemical Report

An asymptomatic 19-year-old male with choroideremia had diffuse loss of retinal pigment epithelium (RPE) and choroid except for the periphery and macula. Fluorescein angiography of the arteriovenous phase showed absence of retinal pigment epithelium and exaggerated visualization of choroidal vessels in involved areas. The mother was a typical carrier with pigment stippling of the midperipheral retina. Histopathologic examination of affected areas of one eye showed marked degeneration of the outer and midretina with loss of retinal pigment epithelium and Bruch's membrane, absence of choriocapillaris, chorioretinal adhesions and gliosis. Atrophy of inner and mid-choroid was also observed. Pigmented macrophage-like cells had migrated into the outer and midretinal layers. Electron microscopy disclosed macrophage-like cells with trilaminar structures and photoreceptor phagosomes in the RPE and outer retina. Remnants of photoreceptor outer segments were adherent to the plasma membranes of the macrophage-like cells. Biochemical analysis of retinal tissue samples for interphotoreceptor retinoid-binding protein (IRBP) showed marked reduction in the 146K bands in the equator and posterior pole in the patient compared to controls. Cyclic nucleotide content was altered in the retinal equator. Cyclic AMP was several-fold higher in the RPE-choroid complex of the affected eye than in the control