Funduscopic examination and SD-OCT in detecting sickle cell retinopathy among pediatric patients.

PURPOSE: To compare the results of fundus examination and spectral domain optic coherence tomography (SD-OCT) in detecting retinal changes in pediatric patients with sickle cell disease at a single center.

METHODS: In this prospective study, conducted over a period of 19 months, consecutive African American patients with sickle cell disease underwent complete ophthalmologic examination, and SD-OCT images of the maculas of both eyes were obtained.

RESULTS: A total of 69 (37 males) patients aged 5-20 years (mean 12.89 ± 4.09; range, 2-20) with sickle cell disease (SC, 26; SS, 36; Sβ+, 5; Sβ0 thalassemia, 2) were examined. Patients' visual acuity range was 20/20 to 20/40. On funduscopic examination, 11 of 69 showed signs of retinopathy, whereas 47 of 68 showed inner retina thinning in the watershed zone temporal to the fovea on SD-OCT. On average, SD-OCT diagnosed disease 1.78 years earlier than fundus examination. Of patients <10 years of age, 1 was diagnosed with retinopathy by funduscopy, whereas retinal changes were evident on SD-OCT in 12 of 22. Fundus examination showed no significant difference in retinal findings between SS/Sβ0 and SC genotypes. On SD-OCT, SS/Sβ0 showed worse disease process than SC in frequency of diagnosis (82% vs 56%), bilateral involvement (87% vs 43%), and foveal involvement (18% vs 0).

CONCLUSIONS: The peripheral retina could be visualized on fundus examination but not easily imaged on SD-OCT, which, however, had a higher detection rate and offered earlier diagnosis. In our patient cohort SD-OCT showed that the severity of retinal change was associated with more severe sickle cell disease genotypes (SS and Sβ0).