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Published on: November 5, 2019
Ocular manifestations of sickle cell disease
A O Fadugbagbe1, R Q Gurgel, C Q Mendonça
1Liverpool School of Tropical Medicine, UK.
Insights
Sickle cell disease (SCD) can cause serious eye problems, including blindness. Regular eye exams are crucial for early detection and management of these vision-threatening complications in SCD patients.
Area of Science:
- Ophthalmology
- Hematology
- Genetics
Background:
- Sickle cell disease (SCD) is a prevalent global genetic disorder.
- Increased life expectancy in SCD patients has revealed previously uncommon complications, particularly ocular issues.
- SCD affects ocular vasculature, potentially leading to vision loss in advanced stages.
Purpose of the Study:
- To review current knowledge on ocular manifestations in sickle cell disease patients.
- To highlight the significance of proliferative sickle retinopathy as a precursor to vision-threatening complications.
- To differentiate ocular complication prevalence based on sickle cell genotypes (Hb SS vs. Hb SC).
Main Methods:
- Literature review of current knowledge on SCD ocular manifestations.
- Categorization of retinal changes into proliferative and non-proliferative based on vascular proliferation.
- Analysis of genotype-specific prevalence of visual impairment.
Main Results:
- Sickle cell disease affects nearly all ocular vascular beds, with potential for blindness.
- Proliferative sickle retinopathy, characterized by new vessel formation, is a key indicator of severe complications.
- Visual impairment from proliferative sickle retinopathy is more common in Hb SC genotype patients than Hb SS.
- Ocular complication incidence and prevalence increase with age.
Conclusions:
- All sickle cell disease patients are at risk for sight-threatening ocular complications.
- Early and regular ophthalmological screening from age 10 is recommended for all SCD patients.
- Distinguishing between proliferative and non-proliferative changes is critical for managing blinding risks.
Abstract:
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.
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