Interrater reliability of photographic assessment of thyroid eye disease using the VISA classification.

PURPOSE: To determine the interrater reliability (IRR) of thyroid eye disease (TED) photographic assessment using the VISA classification. To assess whether a VISA grading atlas improves ophthalmology trainees' performance in photographic assessment of TED. METHODS: A prospective, partially randomized, international study conducted from September 2021 to May 2022. Online study invitation was emailed to a volunteer sample group of 68 ophthalmology college accredited consultants and trainees, and 6 were excluded from the study. Participants were asked to score 10 patient photographs of TED using only the inflammation and motility restriction components of the VISA classification. IRR was compared between groups of practitioners by their level of experience. A clinical activity grading atlas was randomized to 50% of the ophthalmology trainees. RESULTS: Overall rater ICC was 0.96 for inflammation and 0.99 for motility restriction. No statistically significant difference in IRR between rater groups was identified. Trainees with a grading atlas had the highest IRR for inflammation (ICC = 0.95). Each subcomponent of the inflammation and motility restriction components of VISA classification had an ICC considered good to excellent. The mean overall rater score was 4.6/9 for inflammation and 3.5/12 for motility restriction. For motility restriction there was a reduced mean score variance among all raters when scoring photographs with more severe motility restriction. CONCLUSION: IRR using the inflammation and motility restriction components of the VISA classification was excellent. A VISA grading atlas improved trainee performance in grading inflammation.

Paracentral acute middle maculopathy associated with eosinophilic granulomatosis with polyangiitis.

PURPOSE: To present a case of paracentral acute middle maculopathy (PAMM) associated with transient loss of vision from eosinophilic granulomatosis with polyangiitis (EGPA)-associated ischaemic vasculitis. METHODS: Case report and review of literature. RESULTS: A 52 year-old man presented with recurrent episodes of amaurosis of the right eye. A diagnosis of EGPA was established with the combination of late-onset asthma, peripheral eosinophilia, patchy parenchymal consolidation and diffuse pansinusitis. Fundus examination displayed cotton wool spots in the right eye. Optical coherence tomography (OCT) of the macula showed features of paracentral focal inner nuclear layer/outer plexiform layer hyperreflectivity consistent with PAMM. He was treated with corticosteroids and cyclophosphamide. At 2-months following treatment his EGPA was in remission, the patient reported no recurrence of visual symptoms. Dilated fundoscopy revealed resolution of the cotton wool spots. A repeat OCT macula showed resolution of PAMM. CONCLUSION: Our case supports sublethal hypoxia of the middle retinal layers as the underlying mechanism for PAMM. Ophthalmologists should be cognizant to look for PAMM on OCT in patients presenting with transient visual loss and be aware of EGPA-associated ischaemic vasculitis as a rare cause of amaurosis.