Non-Mydriatic Ultra-Widefield Fundus Photography in a Hematology Clinic Shows Utility for Screening of Sickle Cell Retinopathy.

Abstract Purpose To determine the feasibility and accuracy of non-mydriatic ultra-widefield (UWF) fundus photographs taken in a hematology clinic setting for screening of sickle cell retinopathy (SCR) Design Prospective cohort study Methods Setting: Single-site study at the Johns Hopkins Sickle Cell Center for Adults and the Wilmer Eye Institute Study Population: Ninety eyes of 46 consecutive adult participants with sickle cell disease (SCD) Observation Procedure: Bilateral non-mydriatic fundus photos taken by clinic personnel during the participants’ routine hematology appointment were graded by two masked retina specialists at the Wilmer Eye Institute for the presence of non-proliferative SCR (NPSR) and proliferative sickle retinopathy (PSR). A third retina specialist adjudicated in cases of grader disagreement. All participants underwent the standard dilated fundus exam (DFE) within two years of acquisition of UWF photos. Main Outcome Measure: Sensitivity and specificity of non-mydriatic UWF images for the detection of NPSR and PSR Results PSR was noted in 19 of 90 eyes that underwent DFE and 9 of 67 gradable UWF images. Inter-rater agreement between the two graders was moderate with κ = 0.65 (0.43-0.87) for PSR. For gradable UWF photos, the sensitivity and specificity of detecting SCR using the non-mydriatic photos compared to the DFE were 85.2% and 62.5% for NPSR, respectively, and 69.2% and 100% for detection of PSR, respectively. One participant required ophthalmic therapy in both eyes for active sea-fan neovascularization. Conclusions UWF imaging shows utility in screening for SCR and may help identify patients with PSR who require a DFE and who may benefit from treatment.