Study examines sickle cell disease and risk of large vessel retinal vascular occlusion

(Image credit: AdobeStock/Sebastian Kaulitzki)

A team of researchers set out to examine the potential for differences in the risk of developing large vessel retinal vascular occlusions in patients with sickle cell states.

In a retrospective cohort study, published in Ophthalmology Retina, patients with sickle cell disease or trait evaluated by an ophthalmologist were compared to matched controls without sickle cell disease or trait also evaluated by an ophthalmologist.¹

According to the study, the researchers used deidentified data from a national database from 2006 to 2024. They used International Classification of Diseases 10 codes to select for retinal vascular occlusions. Propensity score matching was performed with respect to age, sex, race, ethnicity, smoking, hypertension, diabetes, dyslipidemias, and obesity, resulting in HbSS, HbSC, and sickle cell trait (SCT) cohorts and matched control cohorts.

The study featured main outcome measures, including risk ratios and 95% confidence intervals (CI) of retinal vascular occlusion diagnosis, including central retinal artery occlusion (CRAO), branch retinal artery occlusion (BRAO), central retinal venous occlusion (CRVO), branch retinal venous occlusion (BRVO), and corneal dystrophy as a negative control, given sickle cell disease or trait.

The researchers found that after propensity score matching, HbSS (n = 10,802, mean ± standard deviation age of 38.6 ± 20.6 years), HbSC (n = 4,296, 34.3 ± 17.8 years), and SCT (n=15,249, 39.8 ± 23.7 years) cohorts were compared to control cohorts (n=10,802, 38.7 ± 20.7 years; n=4,296, 34.6 ± 18.0 years; n=15,249, 39.9 ± 23.8 years, respectively).¹

Researchers further noted that patients with sickle cell disease (HbSS) had higher risk of developing any retinal vascular occlusion (RR 2.33; 95% CI 1.82-3.00), CRAO (RR 2.71; 95% CI 1.65-4.47) and BRAO (RR 4.90; 95% CI 2.48-9.67) than matched controls.

Moreover, the study found that patients diagnosed with HbSC disease had an increased risk (RR 3.14; 95% CI 1.95-5.06) of developing any retinal vascular occlusion than matched controls without sickle cell disease. Patients with sickle cell trait did not have higher risk of developing retinal vascular occlusions (RR 1.01; 95% CI 0.81-1.26) than matched controls.¹

“In a retrospective cohort study, patients with HbSS sickle cell disease have an increased risk of developing retinal vascular occlusions, and more specifically CRAO and BRAO compared to patients without sickle cell disease,” the researchers concluded in the study.

Reference:

1. Gabriel T. Kaufmann, Matthew Russell, Priya Shukla, Rishi P. Singh, Katherine E. Talcott,Retrospective Cohort Study of Sickle Cell Disease and Large Vessel Retinal Vascular Occlusion Risk in a National US Database, Ophthalmology Retina. Published July 20, 2024. Accessed August 8, 2024.