Prevalence of ocular complications of sickle cell disease in children seen at a tertiary health facility in Southern Ghana

Abstract Objectives To determine the spectrum of ocular complications of sickle cell disease (SCD) and age of onset of proliferative sickle cell retinopathy (PSR) in children attending the pediatric clinic at a tertiary hospital in southern Ghana. Methods A cross-sectional study of all children with SCD in steady state, ages 2 to 16 years. Demographic and clinical data were recorded using predesigned forms. The ocular manifestations of SCD were classified as either proliferative or non-proliferative based on the presence or absence of neovascularization, respectively. Results Included in this study were 294 children, (53.4% males), mean age 9.2 ± 3.7 years and majority had HbSS-199 (67.9%) and HbSC-74 (25.3%) genotypes. Comma-shaped conjunctiva vessels were the commonest anterior segment manifestation occurring in 85% of children. Non-proliferative posterior segment signs included: venous tortuosity 69 (23.5%), salmon-patch hemorrhages 4 (1.4%), iridescent spots 38 (12.9%), and black sunburst 24 (8.2%). Prevalence of PSR of any stage among children with HbSS was 2.5% (5/199) and 8.1% (6/74) among those with HbSC (P = .0366). The earliest age PSR was detected in this cohort was 8 years and for PSR stage 3, 13 years. No child was blind from eye complications of SCD or any other cause. Conclusion Comma-shaped conjunctiva vessels were the commonest anterior segment manifestation of SCD, and retinal venous tortuosity was the most common posterior segment manifestation. Proliferative retinopathy was more common in HbSC genotype.