Novel retinopathy identified in pediatric retinal vasculitis

Abstract

Purpose : A unique form of retinopathy was identified during the management of pediatric patients with retinal vasculitis and described in the index case series.

Methods : Pediatric patients diagnosed with uveitis were evaluated between January 2020 and December 2020 at a tertiary uveitis clinic. Wide angle fundus photo (WAFP), fundus autofluorescence (FAF), and optical coherence tomography (OCT) images were analyzed to identify potential retinopathy. Retinal vasculitis was detected by fluorescein angiography (FA). Images from both initial and follow-up visits were registered using image registration software. Subjects whose image quality was not sufficient for analysis were excluded. Boundaries of the lesions pertaining to the retinopathy were manually identified and annotated on WAFP using imaging tool. A custom MATLAB algorithm was utilized to assess the progression of the lesion.

Results : 54 pediatric uveitis patients were identified; 5 were excluded due to unavailability of WAFP and/or FA. Among the 49 patients, 26 were diagnosed with retinal vasculitis and were treated.
The retinopathy was detected in 20 patients (30 eyes) during the course of treatment. Mean age of the 20 subjects was 12.8±3.36 years; 40% were female. Retinal vasculitis was observed in 8 patients with panuveitis, 8 patients with chronic anterior uveitis, 3 patients with posterior uveitis, and 1 patient with intermediate uveitis.
On WAFP, the lesions appeared as dark areas that were diffuse, mid-peripheral or peripheral (Image 1A). All subjects (30 eyes) showed hypoautofluorescence on FAF at the same locations as the WAFP (Image 1B). 8 patients (14 eyes) who had OCT images of the retinopathy area demonstrated ellipsoid zone (EZ) disruption (Image 1C).
Progression of the retinopathy over time was analyzed in 10 eyes; all eyes showed improvement of vasculitis with therapy. Mean area of retinopathy decreased from 670.82 mm² (baseline) to 479.92 mm² (last follow-up), which was not statistically significant (p=0.16).

Conclusions : Pediatric retinal vasculitis may be associated with a unique retinopathy pattern. Improvement in the retinopathy may not necessarily correlate to disease activity. The retinopathy might serve as a marker of previous/ongoing vasculitic events.

This is a 2021 ARVO Annual Meeting abstract.

 

Image 1: Retinopathy localized to the mid-peripheral area in the left eye is shown on WAFP (blue dashes) (A) and FAF (white arrows) (B). Ellipsoid zone (EZ) disruption is shown on OCT (C).