Abstract
Purpose :
Sickle cell retinopathy (SCR) has been reported to occur in 10% of children using standard screening techniques. We performed a prospective, observational clinical study to determine if novel retinal imaging devices, including ultra-widefield fluorescein angiography (UWFA), spectral-domain optical coherence tomography (SD-OCT), and optical coherence tomography angiography (OCT-A), would detect a higher frequency of disease in children with sickle cell disease (SCD).
Methods :
A consecutive series of children with SCD of any phenotype, ages 10-19 years, and age-equivalent controls are described. Examinations including acuity, standard slit-lamp biomicroscopy, UWFA, SD-OCT and OCT-A were performed. 2 children were not able/refused to undergo UWFA testing and two UWFA tests (4 eyes) were considered inadequate. Only one control to date has undergone UWFA testing.
Results :
Sixteen children with SCD (mean age 14.9 years) and 3 controls (mean age 16.3 years) were evaluated. No patients had visual complaints at time of examination. While 22/32 SCD eyes (68.8%) had retinopathy on biomicroscopy, UWFA was more sensitive: all 24/24 SCD eyes (100%) had peripheral arterial occlusion (Goldberg I), and 21/24 eyes (87.5%) had peripheral arteriovenous anastomoses (Goldberg II) that were undetectable by biomicroscopy. No patients had neovascular seafans (Goldberg III), vitreous hemorrhage (Goldberg IV), or retinal detachment (Goldberg V). SD-OCT showed thinning of the temporal macula in 6/32 eyes (18.8%), and OCT-A of the same 6/32 eyes (18.8%) revealed flow voids in both the superficial and deep retinal capillary plexus in the area of thinned retina. Both the SD-OCT and OCT-A abnormalities were undetectable with biomicroscopy. Control patients demonstrated no abnormalities by biomicroscopy, SD-OCT, OCT-A, or UWFA.
Conclusions :
All 16 children with SCD in this case series demonstrated evidence of SCR using the combination of biomicroscopy, UWFA, SD-OCT, and OCT-A imaging. Findings from these sensitive imaging modalities suggest that pediatric SCR is more prevalent than previously suspected. Larger cross-sectional and prospective analyses are needed to confirm these findings and identify their significance for detecting pathology. If confirmed, these approaches may enhance early screening for patients at risk of the vision-threatening consequences of SCR.
This is an abstract that was submitted for the 2017 ARVO Annual Meeting, held in Baltimore, MD, May 7-11, 2017.