Clinical and Imaging Characteristics of Posterior Scleritis in Children.

Abstract

Background: Posterior scleritis (PS) is a rare phenotype of scleritis. Comprehensive epidemiological studies on PS in children are limited. We aimed to report on its clinical and imaging features in one of the largest pediatric series to date.

Methods: Retrospective review of medical files.

Results: Included were 12 children (7 girls, mean±SD age at diagnosis 13.75 ± 3.3 years,18 eyes, mean follow-up ±SD of 46.7 ± 45.9 months). All patients presented with eye pain followed by headache. Papillitis was the most common presenting sign (16 eyes, 88.9%). Seven patients (58%) showed laboratory markers of systemic inflammation. One patient had periodic fever, aphthous stomatitis, pharyngitis, adenitis (PFAPA). In 2 other patients, Behҫet disease and inflammatory bowel disease were diagnosed subsequently. Initial echography showed increased posterior scleral thickness (mean±SD 2.9 ± 0.68 mm) with a significant reduction four ± 2 months later (mean±SD 1.85 ± 0.62 mm). Retinal nerve fiber layer (RNFL) was thickened in all examined eyes (mean±SD 136.2 ± 28.4 µm) at presentation and it significantly decreased with treatment (109.8 ± 8.2 µm two months later). In 5 patients (42%), diagnosing PS was prompted by Brain imaging. PS was part of orbital inflammatory syndrome in 4 patients (33.3%). All patients were treated with systemic steroids and 91% required steroid-sparing agent. Adalimumab was added in 2 patients. A chronic course was observed in 63.6%. Remission was achieved in 4 patients. Mean±SD presenting LogMAR VA was 0.23 ± 0.3 with marked improvement to 0.04 ± 0.07 one month later.

Conclusion: Searching for an associated autoinflammatory/autoimmune disease is an important step in patients' triaging. B-scan ultrasound remains the gold standard test. OCT proved to be of practical importance in delineating the magnitude of RNFL thickness and could serve as a potential imaging biomarker of disease activity.