A Case Series of “Solitary Idiopathic Choroiditis” and Proposal of a Nomenclature Change to “Idiopathic Scleroma”

Abstract

<b><i>Objective:</i></b> To define the characteristics of solitary idiopathic choroiditis (SIC) in a consecutive series of patients and propose a nomenclature change to idiopathic scleroma. <b><i>Materials and Methods:</i></b> Electronic patient records were retrospectively interrogated to identify all patients diagnosed with SIC between 2002 and 2019 in a tertiary referral ophthalmic hospital in the United Kingdom. <b><i>Results:</i></b> Thirty-four eyes of 34 patients were found to have SIC. The mean age at diagnosis was 48 years (range 24–78) and 23 patients (68%) were female. All lesions were located posterior to the equator, most frequently in the inferotemporal quadrant (13 eyes, 38%). The lesions had a mean largest basal diameter of 1.2 ± 0.4 disc diameters (range 0.5–2) and their distance to the optic disc had a mean of 1.2 ± 0.9 disc diameters (range 0–3.3). All lesions were intrascleral on enhanced depth imaging optical coherence tomography, demonstrating a hypo-reflective zone within the sclera, with an underlying hyper-reflective zone in some cases. No lesion enlarged or developed features consistent with active inflammation after a median follow-up time of 0.9 years (range 0–16.8). <b><i>Discussion/Conclusion:</i></b> Optical coherence tomography shows SIC to be an intrascleral lesion. Furthermore, we found no evidence of any inflammatory component. A nomenclature change to idiopathic scleroma is appropriate to prevent unnecessary investigation.

Diagnostic imaging of retinal astrocytomas: A case-series

“…The demographic of patients diagnosed with idiopathic scleroma is similar to that of RAs. A study of 34 scleroma patients reported all were asymptomatic and the mean age at presentation was 48 years old [ 12 ]. These lesions however are distinctly different on examination and imaging because they are scleral in origin.…”

“…Idiopathic scleromas can also be distinguished from RA by their location which is deep to the retina. Although, similar to RAs, they too have been described as yellow-white or orange lesions on fundoscopy with well-defined borders in 71% of cases [ 12 ]. Surface calcifications is not a typical feature of these choroidal or scleral lesions.…”

“…These appear as bright areas of hyperfluorescence which correlate to lipofuscin seen on fundoscopy; lipofuscin is not a described feature of RA [ 8 , 10 ]. Yellow-white idiopathic scleromas can usually be identified as hyper-reflective, poorly defined lesions on FAF but the orange lesions are more difficult to identify [ 12 ].…”

Pediatric Mass Lesions of the Fundus

“…Bu nedenle, yazarlar tarafından "idiyopatik skleroma" teriminin lezyon için daha uygun olacağı öne sürülmüştür. [4] Yazarlar EDİ mod OCT ile lezyonların sklerada olduğu ve inflamasyonun eşlik etmediği doğrulandıktan sonra sistemik inflamatuar nedenlerin araştırılmasına veya anti-inflamatuar tedaviye gerek olmadığını belirtmişlerdir. [4] Malign lezyonlardan farklı olarak kendi kedini sınırlayan lezyonlardır.…”

“…[4] Yazarlar EDİ mod OCT ile lezyonların sklerada olduğu ve inflamasyonun eşlik etmediği doğrulandıktan sonra sistemik inflamatuar nedenlerin araştırılmasına veya anti-inflamatuar tedaviye gerek olmadığını belirtmişlerdir. [4] Malign lezyonlardan farklı olarak kendi kedini sınırlayan lezyonlardır. Fakat inflamatuar ataklarla lezyon alevlense de genellikle stabilize olur.…”