Sickle Cell Retinopathy - Retina Club : Retina Club

A and B. Color retinography (Clarus 500, Carl Zeiss Meditec ASG, Jena, Germany) of the right and left eyes of a patient with sickle cell retinopathy, showing fans of fibrous neovascular tangles in the temporal periphery of both eyes after treatment with photocoagulation and anti-VEGF.

C and D. Macular OCT (Cirrus 5000, Carl Zeiss Meditec ASG, Jena, Germany) of the right and left eyes, without pathological alterations.

Description

Sickle cell retinopathy can be present in patients with sickle cell disease, an autosomal recessive hemoglobinopathy caused by a mutation in the gene that encodes the beta chain of hemoglobin.

It progresses with an initial non-proliferative phase in which areas of peripheral ischemia and hemorrhages with a characteristic salmon-colored appearance develop. Visual loss may occur due to macular ischemia.

This chronic ischemia can lead to the formation of new blood vessels in the proliferative phase.

Comments

Sickle cell trait (heterozygosity) and sickle cell disease (homozygosity) are much more common in populations of African descent, as this trait confers some protection against malaria. This is why our level of suspicion for this pathology should be higher in patients with this characteristic.

Indication

A 33-year-old Colombian man with known sickle cell anemia and asymptomatic at the ocular level is referred from hematology to rule out ophthalmological involvement. With visual acuity of 20/20 in both eyes, anterior segment without alterations and IOP within range, he presented, however, fan-shaped neovascularization with salmon-colored hemorrhages in the temporal periphery of both eyes. The macular OCT, normal in the right eye, revealed a detachment of the pigment epithelium superior to the fovea in the asymptomatic left eye. Fluorescein angiography revealed large areas of ischemia in the temporal periphery of both eyes, with the formation of extensive neovascular tangles over them. Argon laser photocoagulation of the ischemic areas was performed in both eyes, and an intravitreal anti-VEGF injection was scheduled with a 1-week interval. After 2 months, complete fibrosis of the neovascular tangles was evident in both eyes without the need for further interventions to date.