Ocular manifestations of sickle cell disease

Sickle cell disease (SCD) is the most common genetic disease worldwide. The increase in life expectancy of SCD patients in recent years has led to the emergence of more complications of the disease, e.g. ocular, which in the past were uncommon. This review describes current knowledge of the ocular manifestations of patients with SCD. SCD can affect virtually every vascular bed in the eye and can cause blindness in the advanced stages. The most significant ocular changes are those which occur in the fundus, which can be grouped into proliferative sickle retinopathy, and non-proliferative retinal changes based on the presence of vascular proliferation. This distinction is important because the formation of new vessels is the single most important precursor of potentially blinding complications. Although various systemic complications of SCD are known to be more common in patients with the Hb SS genotype, visual impairment secondary to proliferative sickle retinopathy is more common in patients with the Hb SC genotype. There is also an increase with age in the incidence and prevalence rates of all ocular complications of SCD. It is therefore recommended that all patients with SCD undergo periodic ophthalmological screening from the age of 10 years.