Abstract
Purpose :
With emerging therapies underway for Inherited Retinal Diseases (IRDs), better documentation and qualitative assessment of peripheral retinal findings in these unique eyes may facilitate more accurate disease assessment and prove useful in potential therapeutic interventions. Here we performed a retrospective, observational study to better understand the utility and limitations of ultra-widefield imaging(UWFI) in assessing retinal findings in patients with IRDs.
Methods :
Charts of 229 consecutive patients with clinically confirmed IRDs seen over a 14-month period at the IRD Clinics at University of Wisconsin were reviewed for presence of UWFI and clinical and genetic diagnosis. Those with UWFI were included. A standard ETDRS 7-field template was placed on ultra-widefield fundus photos and those images with peripheral pathology extending beyond the template were identified and correlated to genetic and clinical diagnosis. Ultra-widefield fundus autofluorescence (FAF) images were also reviewed for peripheral findings. A comparison of color fundus photos and FAF were independently graded by two reviewers for ability to better delineate retinal pathology. Imaging artifacts were noted and classified.
Results :
UWFI by Optomap (Optos PCL, Scotland, UK) was completed in 153/229 patients for a total of 306 eyes. Retinal pathology outside the standard ETDRS 7-field on fundus color photos was present in 55% (168/306 eyes). The most common diagnoses were Retinitis Pigmentosa (27%), Stargardt disease (8%), and Cone-Rod Dystrophy (6%). Ultra-widefield FAF showed peripheral findings in 80% of eyes (244/306). Of those with pathology beyond the ETDRS 7-field by color photos, ultra-widefield FAF better highlighted the extent of retinal degenerations in 47.6% (80/168 eyes). Imaging artifacts were identified in 50% of eyes and imaging acquisition artifacts were the most common (66%).
Conclusions :
Ultra-widefield color fundus photography documented disease features extending beyond the standard ETDRS 7-field in over 50% of eyes, highlighting its utility in identifying pathology that may have been previously unrecognized. Ultra-widefield FAF in particular may better elucidate subtle changes in the retinal periphery in this unique population where degeneration at the level of the retina pigment epithelium can commonly occur. Imaging artifacts are common and should be recognized, as these can influence image interpretation and utility.
This abstract was presented at the 2019 ARVO Annual Meeting, held in Vancouver, Canada, April 28 - May 2, 2019.