Novel retinopathy in pediatric retinal vasculitis: long term follow up
Cigdem Yasar, MD
Cigdem Yasar1, Hashem Ghoraba1, Jonathan Regenold 1, Chris Or1, Muhammad Sohail Halim1, Gunay Uludag1, Irmak Karaca1, Azadeh Mobasserian1, Amir Akhavanrezayat1, Jaclyn Hwang1, Mimbay Yasar2, Hassan Khojasteh1, Yasir J. Sepah1, Diana Do1, Quan Dong Nguyen1
1.Byers Eye Institute at Stanford University, Ophthalmology Department,Palo Alto/CA/USA
2.Bingöl Devlet Hastanesi,Ophthalmology Department, Bingöl/TURKEY
In this case series, we aim to report long term follow up data of novel retinopathy associated with pediatric retinal vasculitis.
Pediatric patients diagnosed with retinal vasculitis at a tertiary uveitis clinic were reviewed between January 2020 and December 2021. Eyes with novel retinopathy were included if follow-up period was more than 12 months. The retinopathy was characterized by three findings: dark retinal areas on Optos wide angle fundus photography (WAFP) with corresponding hypoautofluorescence and ellipsoid zone disruption on optical coherence tomography (Figure).
Retinal vasculitis was graded using fluorescein angiography (FA) per the Angiography Scoring for Uveitis Working Group score. Lesion area was calculated using Image-J software. Full field electroretinogram (ffERG) was also performed. Demographic and clinical data including lesion area and FA severity score were collected at 3 time points: baseline, within 2 months from ffERG and at last follow-up visits.
17 pediatric patients (27 eyes) with retinal vasculitis and novel retinopathy were included. Mean age was 12.7±3.4 years and 35 % were female. Mean follow up duration was 29.4±12.1 months. FA scores decreased from 8±5.04 at baseline to 2.1 ± 2.1 at the last follow up visit (P<0.001). 30% of eyes did not show any disease activity at the last follow-up visit.
The retinopathy persisted in all eyes at the last follow-up visit. However, mean lesion area significantly decreased from 407.6±239.5 mm2 at baseline to 378.4±222.6 mm2 at last follow-up visit (P=0.04). FA scores at baseline were significantly correlated with lesion areas at both baseline (P=0.01) and last follow-up visit (P=0.01). However, FA scores at the last follow-up visit did not correlate with the lesion areas (P=0.9).
ffERG was performed in 11 patients. lesion areas, at the time of ffERG, were negatively correlated with amplitudes of scotopic rod b wave (P=0.03), mixed a wave (P=0.05), mixed b wave (P=0.001), cone b wave (P=0.05), and 32 Hz flicker (P=0.006). Delayed 32 Hz flicker timing was also correlated with lesion areas (P=0.009).
Conclusion: Pediatric retinal vasculitis is associated with a novel retinopathy whose area is correlated with decreased retinal function and higher initial disease activity. The retinopathy can persist even after resolution of inflammation.