Abstract

Ophthalmological examinations were performed on 59 of the 74 (80%) children with homozygous sickle cell (SS) disease and on 37 of the 54 (69%) children with sickle cell-haemoglobin C (SC) disease, aged 5-7.5 years, within the cohort study of sickle cell disease. Arteriolar sheathing was the commonest retinal vessel abnormality, occurring in 30/59 (51%) SS children and in 11/37 (30%) SC children. Peripheral arteriolar closure was observed in 14 (24%) SS children and in 6 (16%) SC children. Arteriovenous anastomoses were seen in 3 children, but proliferative retinopathy was not identified. Capillary changes often occurred in patients without confluent closure, suggesting that complex remodelling of the capillary bed may precede retinal non-perfusion. Discrete retinal patches similar to schisis cavities resulting intraretinal haemorrhages were found in 22 (37%) SS children and in 9 (24%) SC children, but haemorrhages were observed in only 2 patients (1 SS, 1 SC). Vitreous opacities were common and were generally associated with retinal vessel disease. Retinal changes were consistently more common in children with SS disease, though the differences failed to reach statistical significance. The prevalence of peripheral vascular closure and retinal patches showed a significant upward trend with age. These observations contrast with the greater prevalence of proliferative retinopathy characterising SC disease in adults.