Ocular findings in children with sickle cell haemoglobin C disease in Jamaica

Abstract

The ophthalmological findings in 54 Jamaican children with sickle cell hemoglobin C disease are reported. Evidence of peripheral retinal vessel disease was present in 94% and retinitis proliferans in 11%. Retinitis proliferans was noted as early as 7 yr of age and was more common in patients with high hemoglobin levels. There was an unequivocal progression in severity of retinopathy in 8 out of 11 children examined 2 yr previously. The pathological processes leading to sickle cell proliferative retinopathy are well established in childhood and attempts at prophylactic therapy should be instituted at an early age.