Abstract

Three premature infants with no family history of eye disease showed bilateral cystoid maculopathy on post-mortem examination. These macular lesions are indistinguishable macroscopically from the macular lesions of sex-linked juvenile retinoschisis. Cystoid change was observed at various vertical retinal levels. The oldest child had a schisis cavity within the nerve fibre layer. All cases had reduced numbers of retinal ganglion cells and central nervous system (CNS) abnormalities. The authors believe these are the first reported cases of a cystoid macular lesion present at birth and the first description of the pathological features of a cystoid macular lesion in infants.