Hereditary retinal degeneration in the Rhode Island Red chicken. I. Histology and ERG.

Hereditary blindness in Rhode Island Red chickens was analyzed at various post-hatching stages by light microscopy and electrophysiological recordings. At the time of hatching the retina of affected chicks appeared morphologically normal and identical to that of control, non-affected chicks. Whereas the electroretinographic (ERG) response to light stimulus in normal chicks was near the adult level at the time of hatching, no ERG either under light- or dark-adapted conditions was measurable in affected chicks at any stage examined. Photoreceptor cells of affected animals were seen to undergo degenerative changes after about one week post-hatching. Decrease in number of outer segments, spaces between inner segments and large spaces in the outer nuclear layer were apparent by Day 10. By Day 21, most of the photoreceptor inner segments appeared swollen, and the decrease in number of outer segments and photoreceptor nuclei was noteworthy. By the end of the second month no outer segments were seen and the majority of identifiable inner segments were from cones, a larger proportion than normally present being double cones. By six months, very few photoreceptor inner segments and nuclei remained; most inner segments were deformed and diminutive but usually contained a clearstaining oil droplet characteristic of the principal member of the double cone. In all stages after one week of age, pycnotic nuclei and thinning of inner retinal layers accompanied photoreceptor degeneration. In all specimens examined, degeneration of retinal cells was more pronounced in the superior central retina than in the periphery. Pathological changes were frequently also noted in the pigment epithelium overlying degenerating retina. Because the chick retina is well developed at birth, contains a fovea and a significant cone population and because cones (particularly one specific type) survive rods, we believe that this congenitally-blind chicken may be a useful model for studies on human hereditary retinal degenerations.