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Table 2 Differential diagnosis of posterior scleritis

From: Choroidal involvement in non-infectious posterior scleritis

 

Posterior scleritis

Central serous chorioretinopathy (CSC) [15, 40]

Vogt-Koyanagi-Harada disease (VKH) [15, 40, 41]

Choroidal melanomaa [13, 40]

Typical clinical characteristics

 Laterality

Mostly unilateral

Unilateral or bilateral

Bilateral (second eye involvement within 2 weeks)

Unilateral

 Presenting symptoms

Acute painful vision loss (typically worse at night or on eye movements)

Painless vision loss

Blurred vision, photophobia, ocular pain. Associated systemic symptoms depending on disease stage.

Painless visual changes (rarely painful when necrotic). Can be asymptomatic.

 Associated clinical signs

Anterior scleritis, anterior uveitis, vitreous inflammation, choroidal folds, optic disc edema.

Round/oval serous retinal detachment with or without detachment of retinal pigment epithelium (RPE), RPE changes (focal or multifocal).

No associated inflammatory signs.

Granulomatous anterior uveitis, vitritis, diffuse choroiditis, Dalen-Fuchs nodules, sunset glow fundus, optic disc edema.

Frequent associated skin changes, CNS findings (incl. Cerebrospinal fluid pleocytosis)

Pigmented or amelanotic elevated choroidal mass, lipofuscin often present. Serous RD and/or sentinel vessel possible. Associated inflammation rare.

 Response to steroids

Improvement of pain and imaging findings

Can worsen presenting signs and symptoms

Improvement of pain and imaging findings

None

Multimodal imaging findings

 B-scan ultrasound

T-sign, increased thickness of the posterior coats (> 2.0 mm), nodular subtype possible (sessile or dome shaped lesion with high internal reflectivity)

Serous RD possible

Serous RD possible

Dome-shaped or mushroom-shaped choroidal lesion with typically low to medium internal reflectivity. Choroidal excavation and serous RD possible.

 OCT

Choroidal folds, serous retinal detachment, macular oedema

Subretinal fluid (SRF) (can be multifocal), RPE-detachment(s), intraretinal fluid (IRF) possible, atrophic RPE-changes possible

Serous retinal detachment with typical fibrinous septa

Dome-shaped solid choroidal mass, accompanying SRF possible

 EDI-OCT

Localized choroidal thickening in affected eye in acute stages, choroidal thinning after treatment or in advanced stages

Diffuse choroidal thickening, dilated large vessels in Haller’s layer with thinning of overlying smaller vessels in Sattler’s layer and choriocapillaris

Choroidal thickening in acute stages, choroidal thinning after treatment or in advanced stages

Dome-shaped solid choroidal mass with smooth surface

 FA

Early pinpoint leaks with late pooling in cases with subretinal fluid

Focal leak in acute phase (typically described as ‘ink blot’ or ‘smokestack’ leakage pattern). Multifocal leakage (‘hot spots’) and pooling under detached RPE possible. Disc leak absent.

Focal areas of delayed choroidal perfusion, multiple pinpoint regions of leakage at the RPE-level, disc hyperfluorescence

Multiple areas of pinpoint leakage

 ICGA

Choroidal vasculitis, zonal choroidal hyperfluorescence (± pinpoint leakage), choroidal perfusion delay, enlargement of draining choroidal veins, and hypofluorescent dark dots

Early phase: large, dilated, densely packed choroidal vessels. Mid-to late-phase: choroidal vascular hyperpermeability (focal or multifocal hyperfluorescent staining with indistinct borders).

Early phase: hyperfluorescence. Early-to-mid-phase: hypofluorescence.

Mixed pattern of fluorescence, blockage of fluorescence in pigmented lesions

  1. aChoroidal melanoma serves as an example of choroidal malignancies in this table. Other tumors such as metastatic deposits or choroidal lymphoma have different clinical and imaging characteristic and should also be borne in mind