Longitudinal Study of Peripapillary Thinning in Sickle Cell Hemoglobinopathies.

PURPOSE: To determine the rate of retinal nerve fiber layer (RNFL) thinning in patients with sickle cell hemoglobinopathies. Design; Prospective cohort study METHODS: Sixty-seven patients averaging 35.8±11.5 years old at enrollment with electrophoretically-confirmed sickle cell hemoglobinopathies followed by the University of Illinois at Chicago retina clinic for ≥1 year were included. Exclusion criteria included a history of diabetes, uncontrolled hypertension, glaucoma, ocular opacities, other retinopathies, and previous retinal procedures. The optic nerve head retinal nerve fiber layer thicknesses were measured with Spectral-domain optical coherence tomography (Heidelberg Spectralis, Heidelberg Engineering, Inc, Carlsbad, CA, USA) at enrollment and subsequent follow-ups. Linear mixed models were used to estimate rates of thinning.

RESULTS: A total of 122 eyes were followed for 3.8±2.0 years (range 1 to 8 years). Mean global peripapillary RNFL thickness was 100.9±13.0 μm at baseline. Global peripapillary RNFL thickness decreased at a rate of 0.98 (95% confidence intervals (CI) 0.77 to 1.19) μm/year. A history of stroke was associated with a faster rate of global RNFL thinning (1.72±0.20 vs. 0.79±0.12 μm/year, P <0.001), whereas a history of hypertension was associated with a slower rate of thinning (0.33±0.27 vs. 1.14±0.12 μm/year, P = 0.002).

CONCLUSIONS: Peripapillary RNFL thinning in patients with sickle cell hemoglobinopathies occurred faster in patients with a history of stroke and slower in those with controlled hypertension. Future studies will compare these rates to those of healthy age- and race-matched individuals.