Sickle Cell Retinopathy

The disease

Sickle cell retinopathy is an autosomal recessive genetic disorder resulting from a mutation of the gene coding for hemoglobin.
This disease can affect different organs including the eyes, which is affected at the retinal level giving the sickle cell retinopathy.

The need for screening

The early stages of sickle cell retinopathy do not cause symptoms, this is the reason why it is essential to regularly screen patients with sickle cell disease.

Diagnosis and follow-up

The diagnosis of sickle cell retinopathy is mainly ophthalmoscopic. Further investigations may be necessary to evaluate the severity of the retinopathy, such as OCT (optical coherence tomography), fluorescein angiography or even OCT angiography.

Sickle cell retinopathy: large field retinography showing the presence of two neovessels, in the temporal and in the nasal periphery (blue arrows).

Fluorescein angiography of the same patient showing the two neovessels (blue arrows).

The formation of pathological retinal vessels is the main complication.

It is caused by chronic retinal ischemia. Once neovessels are present they can lead to a retinal detachment or a vitreous hemorrage.

Screening for sickle cell patients is therefore essential: it enables early detection, prevention of the possible complications and early treatment. This follow-up is carried out in the ophthalmology department of the intercommunal hospital of Créteil, in collaboration with the pediatric department.