MAY 12, 2022
The ability of a standard dilated fundus examination (DFE) to detect sickle cell retinopathy was compared to the sensitivity and specificity of ultra-wide fundus photographs, which could be taken during regular hematology appointments.
This single-site prospective cohort study enrolled patients 18 and older with sickle cell disease (SCD). All participants underwent a DFE within 2 years of acquisition of ultra-wide fundus (UWF) photographs using the Optos UWF camera without pupillary dilation. Current screening guidelines recommend yearly DFEs for patients with SCD, but missed appointments are not uncommon. The purpose of this study was therefore to assess the feasibility and accuracy of nonmydriatic UWF photographs taken in a hematology clinic for screening of sickle cell retinopathy (SCR).
Proliferative sickle retinopathy (PSR) was detected in 19 of 90 eyes that underwent DFE and 9 of 67 gradable UWF images. For gradable images, the sensitivity and specificity of detecting SCR with the UWF photos as compared to DFE were 85.2% and 62.5% for nonproliferative sickle retinopathy (NPSR), respectively, and 69.2% and 100% for detection of PSR. Interrater analysis of the UWF photos showed a large variability between the 2 graders.
This study had several limitations. First, the number of participants is small. Second, 8 patients had to be excluded from the study because of nonadherence with the DFE. Third, the requirement was that the image be obtained within 2 years of the DFE. The disease could have progressed within this 2-year window and thus affect the concordance between the exam and the UWF image. Finally, there was a large percentage of ungradable images due to poor quality and artifacts.
There is a high rate of non-adherence to yearly DFEs in patients with SCD. This study demonstrates that adding UWF imaging technology to the hematology office may improve access to ophthalmic care and identify patients with SCR who might have progressed to vision loss before diagnosis. The high rate of ungradable images shows that further training of the technician obtaining images would be necessary to improve image quality.