Publication Only: Sickle cell disease
Sickle cell disease results in localized ischemia and sometimes infarction of local tissues in the area of vascular sickling of erythrocytes.This process can result in a variety of ocular abnormalities; all structures can be affected, ranging from orbital tissues to the retina. Macular thinning has been described in patients with sickle cell disease.
The aim of this study was to investigate the effect of asymptomatic childhood sickle cell anemia on choroidal, macular and retinal nerve fiber layer thickness (RNFL) by spectral-domain optical coherence tomography(SD-OCT).
Twenty sickle cell patients (40 eyes) without ocular diseases and 30 healthy control patients (60 eyes) underwent SD-OCT. We used SD-OCT to take all measurements of ocular thickness, as well as measured intraocular pressure, axial length, and central corneal thickness. We later analyzed correlations of hemoglobin S levels and number of crises with ocular parameters.
A statistically significant difference emerged between patients with sickle cell anemia and the healthy controls in terms of mean values of subfoveal and temporal choroidal thickness (p = 0.004 and p = 0.042, respectively). There was no statistically significant difference between the two groups in central and paracentral macular thickness. In addition, there was no statistically significant difference between the two groups in the thickness of the retinal nerve fiber layer.There was no statistically significant difference correlation analyses between the hemoglobin S level and the study parameters. Correlation analysis of the number of crisis and working parameters revealed a negative correlation with subfoveal choroidal thickness and superonasal quadrant RNFL thickness (p = 0.036 and p = 0.002, respectively)
Manual segmentation of SD-OCT images revealed quantitative and qualitative changes in asymptomatic sickle cell patients.Further studies are required in a larger number of patients to detect a possible association between the retinal thinning seen on SD-OCT images and peripheral retina ischemia.