Morphological findings during retinal development and maturation in hereditary rod-cone degeneration in Abyssinian...

The sequence of structural changes involved in postnatal photoreceptor differentiation, maturation and early degeneration was studied in young Abyssinian cats and kittens with hereditary rod-cone degeneration and compared to maturation in normal controls. In affected cats the earliest change seen was disorientation of outer segment discs in the majority of the rods, while other rods appeared to develop and mature normally. Such disorientation of discs (at oblique angles or parallel to the longitudinal axis of the outer segment, or whorls of discs) is considered as 'immaturity', since controls also showed a substantial number of disoriented rod outer segment discs at this young age. At postnatal day 35 the difference between affected animals and controls was marked with a high frequency of immature appearing rod outer segment discs in affected animals, while all rod outer segment discs were adult-like and arranged in an orderly manner in controls. Cones seemed unaffected at this age. More severe changes in affected rod outer segments in the form of disintegration of discs (vacuolization and clumping of disc material, or formation of debris), which we consider to represent degeneration, were first observed at the time when retinal maturation normally occurs in the cat, i.e. 150 days postnatally. Subsequently a drop-out of rods was seen, primarily of rods with disoriented and disintegrated outer segment discs, followed by a slow, progressive degeneration of rods that had developed and matured normally. Cones appeared normal during the time of retinal development and maturation and it was not until the age of 2-3 years (Narfström and Nilsson, 1986, Incest. Ophthalmol, Vis. Sci. 27, 1569-76) that degenerative changes were seen also in cones.