Multimodal Imaging of Dark Without Pressure Lesions in Pediatric Retinal Vasculitis

Abstract Purpose: A unique form of retinopathy-plausibly dark without pressure (DWP) was identified during the management of pediatric patients with retinal vasculitis (RV) and described in the index case series. Methods:Forty-three consecutive pediatric uveitis patients presented to tertiary care-unit were evaluated. We assessed DWP-like lesions in pediatric patients with RV. Wide angle fundus photographs (WAFP), fundus autofluorescence (FAF), and optical coherence tomography (OCT) images were analyzed to identify potential retinopathy. Incidence of DWP in pediatric RV patients was also analyzed. WAFP were evaluated using image-J software to measure retinopathy area (RA). Results:Twenty-six of 43 patients were diagnosed with RV and were treated. Retinopathy was detected in 20 patients (30 eyes/76.9%) during treatment. Mean age was 12.8±3.36 years; 40% were female. On WAFP, the lesions appeared as dark areas that were diffuse, mid-peripheral or peripheral. All 20 patients (30 eyes) showed hypo-autofluorescence on FAF at the same locations as the WAFP. Twelve patients (17 eyes) who had OCT images of the RA demonstrated ellipsoid zone disruption. Progression of the retinopathy overtime was analyzed in 28 eyes; all eyes showed improvement of RV with therapy. Mean RA significantly decreased from 394.67 mm2 (time of retinopathy detection) to 365.88 mm2 (last follow-up) (p=0.03). Conclusions:Pediatric RV may be associated with a retinopathy pattern described as DWP previously. Improvement in the RA may be related to improvement of disease activity, but further studies are needed to understand the underlying pathophysiology. Such findings might serve as a marker of previous/ongoing RV in pediatric patients.