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Jonathan Li, Lloyd Bender, Gui-Shuang Ying, Gil Binenbaum; Prevalence and Onset of Pediatric Sickle Cell Retinopathy. Invest. Ophthalmol. Vis. Sci. 2018;59(9):4270.
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© ARVO (1962-2015); The Authors (2016-present)
Children with sickle cell disease (SCD) can develop proliferative retinopathy with vision loss, but lack of consensus exists regarding screening regimens. We sought to determine the prevalence, age at onset, and risk factors associated with sickle cell retinopathy (SCR) to inform development of screening guidelines for asymptomatic children.
Retrospective cohort study of children with SCD over a 4 year period was performed. Outcomes were prevalence, age at onset, and type of SCR, based upon examination by an ophthalmologist. Markers of SCD severity (#ER or hospital admissions for crises, # blood transfusions, hydroyxurea therapy and dose, transcranial-Doppler-confirmed cerebral vasculopathy), genotype, gender, and race were evaluated as SCR risk factors.
Of 393 children (mean age 9.6±4.6 years, range 0-18), 208 (52%) had SS, 113 (28%) had SC, and 77 (19%) had sickle cell trait. 53 (13.4%) children had SCR; 44/398 (11.1%, 95% CI 8.3%-14.5%) had non-proliferative-retinopathy (NP-SCR), 9/398 (2.3%, 1.2%-4.2%) had proliferative-retinopathy (P-SCR). Prevalence was higher for SC than SS for NP-SCR (21% vs. 9%) and P-SCR (5% vs. 1%); onset for SC was earlier than SS for NP-SCR (4.8 vs. 6.1 years) and P-SCR (12.2 vs. 15.4 years). No other risk factors were significantly associated with SCR. Clinical markers of SCD severity were not associated with SCR.
Based upon our study and literature review, while screening could begin at age 5 years for NP-SCR, screening of children without ophthalmologic symptoms to identify treatment-requiring P-SCR could begin later, at age 9 years for SC and age 13 years for SS. Clinical markers of SCD severity were not associated with SCR and are not necessary for screening guidelines.
This is an abstract that was submitted for the 2018 ARVO Annual Meeting, held in Honolulu, Hawaii, April 29 - May 3, 2018.
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