Changes in the cornea related to sickle cell disease: a pilot investigation

Purpose: To investigate corneal structural changes (central corneal thickness, endothelial cell count, and cellular morphology) in patients with sickle cell disease (SCD). Methods: This prospective study included 56 patients with SCD and 50 age- and sex-matched healthy subjects without any eye disease aside from refractive errors. Endothelial cell density (ECD), percentage of hexagonality, and the coefficient of variation in cell size (CV) were measured using noncontact specular microscopy, and central corneal thickness (CCT) was measured by pachymetry. Results: The mean CCT value was 509.6 +/- 20.7 mu m in the study group and 520.8 +/- 23.6 mu m in the control group. The mean ECD, CV, and percentage of hexagonality values in the study group were 2712 +/- 335 cells/mm(2), 34.5 +/- 5.3%, and 57.2 +/- 6.6%, respectively, and 3030 +/- 247 cells/mm(2), 31.6 +/- 5.0%, and 60.4 +/- 6.9% in the control group, respectively. Endothelial cell density (p = 0.001), CCT (p = 0.011), CV (p = 0.005), and percentage of hexagonality values (p = 0.018) were significantly different between the study and control groups. Conclusions: The results of the current study indicate that patients with SCD had considerable morphologic changes in the structure of the cornea when compared to healthy subjects.