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Katherine A Joltikov, Jennifer I Lim; Reduced Macular Microvasculature in Adults over 40 years old with Sickle Cell Disease. Invest. Ophthalmol. Vis. Sci. 2020;61(7):1322.
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© ARVO (1962-2015); The Authors (2016-present)
The prevalence of proliferative sickle retinopathy (PSR) increases with age. The purpose of this study is to compare macular microvasculature in older adults with sickle cell disease with healthy controls.
Adults 40 years and older with HbSS, HbSC, HbS thalassemia, and healthy controls were selected from a database of sickle cell patients enrolled in a longitudinal study at the University of Illinois at Chicago. All subjects underwent a comprehensive ophthalmic exam with optical coherence tomography angiography (OCT-A). The superficial capillary plexus (SCP) and deep capillary plexus (DCP) vessel density (VD), and the foveal avascular zone (FAZ) area size were measured for all subjects. Statistical analysis was performed using ANOVA, Tukey- Kramer HSD, and Pearson’s Correlation.
Twenty-three HbSS (50.2 ± 5.6 years, range 41 – 59 years), 19 HbSC (48.3 ± 7.6 years, range 40 – 68 years), 4 HbS Thal (54.8 ± 6.8 years, range 46 – 61 years), and 11 healthy control eyes (55.0 ± 9.6 years, range 42 – 68 years) were included. In the HbSS group, there was 1 eye with stage 3 PSR, and 22 eyes with stage 2 PSR; in the HbSC group there were 6 eyes with stage 3 PSR, 15 eyes with stage 2 PSR, and 1 eye without retinopathy; and in the HbS Thal group there were 3 eyes with stage 2 PSR and 1 eye without retinopathy. Gender and age were not significantly different for Goldberg stage or Hb subtype. FAZ area was increased in HbSC eyes compared to controls (p=0.03). The VD in the SCP and DCP was lower in HbSC eyes compared with HbSS eyes (p=0.04). Age was correlated with both increased FAZ area (p=0.02) and decreased VD in the control group (p=0.004). There was no correlation between age and FAZ or VD in the sickle cell eyes.
Sickle cell patients over 40 have increased FAZ area and decreased VD, which are worst for HbSC. FAZ and VD are relatively unchanged with increasing age after 40 years, compared with controls. These findings provide insight into the pathophysiology of sickle cell retinopathy in older adults.
This is a 2020 ARVO Annual Meeting abstract.
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