Anterior Segment-Optical Coherence Tomography features in Blau syndrome

Concilio, Marina; Cennamo, Gilda; Giordano, M; Fossataro, Federica; D’Andrea, Luca; Ciampa, Nicola; Naddei, Roberta; Orlando, Francesca; Tranfa, Fausto; Alessio, Maria

doi:10.1016/j.pdpdt.2021.102278

Cited by 3 publications

(5 citation statements)

References 6 publications

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“…As demonstrated in a recent case report of two siblings, an 8-year-old female and a 5-year-old male, with Blau syndrome, diagnosed with genetic testing, with one having uveitis and the other asymptomatic, AS-OCT allowed for non-invasive imaging of both patients. 95 Findings in the 8-year-old patient included hyperreflective dots in the aqueous humor and hyperreflective dots on the posterior corneal surface, thought to correspond to anterior chamber cells and keratic precipitates, respectively, as visualized on biomicroscopy; these anterior-segment findings, as well as any others, were absent in the 5-year-old male. 95 …”

Section: Imaging In Pediatric Uveitismentioning

confidence: 82%

“… 95 Findings in the 8-year-old patient included hyperreflective dots in the aqueous humor and hyperreflective dots on the posterior corneal surface, thought to correspond to anterior chamber cells and keratic precipitates, respectively, as visualized on biomicroscopy; these anterior-segment findings, as well as any others, were absent in the 5-year-old male. 95 …”

Section: Imaging In Pediatric Uveitismentioning

confidence: 82%

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Multimodal imaging in pediatric uveitis

et al. 2021

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Pediatric uveitis accounts for up to 10% of all uveitis cases, so special attention must be paid to ensure early diagnosis as well as treatment and follow-up of these young patients in order to decrease the risk of possible ocular complications and consequently vision loss. Multimodal imaging has been an effective and important adjunct in the diagnoses and management of uveitis, especially in children. Reviewed here are the currently utilized modalities, advances, as well as their applications in juvenile idiopathic arthritis–associated uveitis, pars planitis, retinal vasculitis, tubulointerstitial nephritis and uveitis syndrome, Behçet disease, Blau syndrome, and Vogt–Koyanagi–Harada syndrome.

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Section: Imaging In Pediatric Uveitismentioning

confidence: 82%

Section: Imaging In Pediatric Uveitismentioning

confidence: 82%

Multimodal imaging in pediatric uveitis

et al. 2021

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“…Anterior segment OCT is a method that allows for the quantitative evaluation of cells in the anterior chamber, which requires refinement of standardization for routine use [ 45 ]. Its use in children has been described, for example, in Blau syndrome as a test to improve the diagnosis of retinal and corneal lesions in children with this condition [ 46 , 47 ].…”

Section: Resultsmentioning

confidence: 99%

“…It is a useful tool for evaluating hypotony of the uveal membrane of the eye, and allows differentiation between hypotony against a background of mechanical damage to structures and that caused by chronic inflammation [ 59 ]. The use of UBM requires patient cooperation, which is a significant limitation among pediatric patients [ 47 ].…”

Section: Resultsmentioning

confidence: 99%

Diagnosis and Treatment of Uveitis in Children: A Summary of the Latest Data from a 5-Year Literature Review (2018–2023)

Modrzejewska,

Zdanowska

2024

JCM

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Pediatric uveitis has a low incidence. It is very diverse in its presentation and is often the first sign of a severe systemic disease. The pediatric population poses a special therapeutic and diagnostic challenge due to the potentially adverse effects of therapeutic agents on the young body and difficult cooperation with the patient during the examination, as well as the increased risk of complications that can lead to severe disability. The most commonly diagnosed type of uveitis is non-infectious, with first-line therapy consisting of systemic corticosteroids followed by disease-modifying drugs (methotrexate (MTX), mycophenolate mofetil (MMF), and cyclosporin A (CsA)). In severe, refractory cases, biologic therapy is used. The authors reviewed the current literature on the etiology, diagnostic tools, and treatment of uveitis in the pediatric population covering the years 2018–2023, presenting current methods of modern diagnosis and treatment. The reason for writing this article was the need to update the knowledge on uveitis, driven by the increasing prevalence of autoimmune uveitis in the pediatric population. This trend presents significant challenges in diagnosing and treating the disease, as well as managing its complications. Correctly identifying the pathogenetic factor of uveitis can facilitate the diagnosis of the systemic disease underlying the ocular infection and enable the timely implementation of systemic treatment. Furthermore, the emergence of new diagnostic methods necessitates a revision and update of ophthalmic knowledge, essential for both ophthalmologists and other specialists involved in the treatment of uveitis.

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“…[ 28 29 30 ] Concilio et al . [ 31 ] have recently reported the relevance of anterior segment OCT (AS-OCT) in an 8-year-old girl with bilateral anterior granulomatous uveitis with underlying BS. AS-OCT in this patient revealed high-intensity reflective layers in the anterior cornea along with hyperreflective dots on the posterior corneal surface indicative of anterior chamber inflammation and deposition of inflammatory cells on the posterior corneal surface.…”

Section: Ophthalmic Manifestations Of Bsmentioning

confidence: 99%