Tele-Ophthalmology Screening for Sickle Cell Retinopathy Using Ultra-Widefield Fundus Photography

Abstract

Purpose : This is a proof-of-concept study to determine the accuracy of non-mydriatic ultra-widefield (UWF) fundus photography taken by clinic personnel in screening for sickle cell retinopathy (SCR) in a non-ophthalmology clinic setting.

Methods : This prospective study compared SCR findings on UWF fundus photography to the gold standard dilated fundus exam (DFE). Adults 18 years or older with sickle cell disease and a DFE within two years of acquisition of fundus photos were included. An UWF fundus camera was used to obtain bilateral non-mydriatic fundus photos by clinic personnel during the participants’ routine hematology appointment. These photos were graded by two separate masked retina specialists to assess image quality, visualization of peripheral retina, presence of non-proliferative SCR, and presence of proliferative sickle retinopathy (PSR). A third retina specialist reviewer assessed photos in instances of disagreement.

Results : Forty-five participants (88 eyes) met inclusion criteria. The average age was 37.2 ± 10.8 years. Hemoglobin SS disease was the most common genotype (n = 30), followed by hemoglobin SC (n = 9) and sickle cell beta thalassemia (n = 6). Non-proliferative SCR and PSR were documented in 31.8% and 21.6% of eyes, respectively, on DFE. When comparing UWF grading to DFE for the presence of non-proliferative SCR, 54.4% of eyes were graded accurately and 20.5% were graded inaccurately (p = 0.014). For the 18 eyes graded inaccurately by UWF imaging, most were false positives (n = 16). UWF image quality was insufficient for grading in 18 eyes. No consensus was reached for 4 eyes. When assessing for PSR, 65.9% of eyes were graded accurately and 4.5% were graded inaccurately by UWF imaging, all as false negatives. UWF image quality was insufficient for grading for 23 eyes and there was no consensus for 3 eyes. When UWF image quality was sufficient, UWF grading was accurate for non-proliferative SCR and PSR in 72.7% and 93.5% of eyes, respectively (p < 0.001 for both). Of note, one participant had active sea-fan neovascularization detected on UWF imaging and DFE, and was treated with laser photocoagulation.

Conclusions : UWF imaging provides a useful screening modality for SCR, particularly for proliferative PSR. Tele-ophthalmology-based SCR screening in a hematology clinic may improve adherence to SCR screening recommendations and identify patients with PSR who may require treatment.

This is a 2021 ARVO Annual Meeting abstract.