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Table 2 Clinical and multimodal imaging findings seen in the white dot syndromes and their masqueraders

From: Multi-modal imaging and anatomic classification of the white dot syndromes

  Sympathetic ophthalmia VKH Sarcoidosis OH PIC MCP MEWDS APMPPE
Anterior exam findings Possible AC cell and mutton-fat keratic precipitates; thickened iris AC cell and flare AC cell with mutton-fat keratic precipitates; Iris, conunctival and/or scleral nodules No inflammation Typically absent inflammation Periodic AC cell and flare No inflammation No AC inflammation
Posterior examination findings Vitritis; depigmentation of the choroid; Dalen-Fuchs nodules Choroidal depigmentation; Possible chorioretinal atrophy “String of pearls” or “snowball” vitreous opacities; peripheral periphlebitis; choroidal granulomas; optic nerve head granulomas “Punched-out” chorioretinal lesions in the mid-periphery and posterior pole (“histo spots”); chorioretinal peripapillary atrophy (PPA); CNV Small (100–300 μm), well-delineated, yellow-white lesions in posterior pole at the RPE, inner choroid or choriocapillaris; Possible yellow-white chorioretinal scars; CNV Greyish-yellow, jogsaw-puzzle-shaped lesions at levels of the RPE and choriocapillaris emanate from optic nerve; lesions evolve into punched-out scars with pigmented borders; CNV Ill-defined round, yellowish–white lesions in perimacular area and occasionally peripheral to the arcades; granular fovea Vitritis; numerous, yellow, creamy colored placoid lesions are seen in posterior pole in various stages of evolution
OCT Diffuse choroidal thickening, subretinal fluid, and irregular IS/OS junction and ELM Thickened choroid; possible sereous retinal detachment Hyporeflective thickening of the choroid Loss of intrinsic reflectance Focal elevation of RPE with underlying hyporeflective space and focal atrophy of the outer retina and RPE; focal hyperreflective dots in inner choroid; focal thinning of choroid adjacent to lesions Drusen-like sub-RPE material; choroidal hyperreflectivity below lesions, and overlying vitreous cells Disruption of ellipsoid zone; accumulation of hyperreflective material that rests on RPE and extends anteriorly Hyperreflectivity of outer retinal layers in early stages; disruption of IS/OS junction and outer retina; RPE atrophy
Fluorescein autofluorescence   Peripheral FAF abnormalities   Lesions correspond to round hypoautofluorescence Hypoautofluorescent spots with hyperautofluorescent margin Hypoautofluorescent lesions in pole and periphery Areas of hyperautofluorescence in acute phase; possible pinpoint hypoautofluorescence corresponding to foveal granularity Hypoautofluorescent lesions that appear later and less numerous than APMPPE lesions seen clinically
Fluorescein angiography Disk leakage; numerous progressively hyperfluorescent dots at level of the RPE; possible early focal blockage of background choroidal fluorescence Hypofluorescent pinpoint dots in early phase followed by multiple focal areas of leakage and subretinal dye accumulation at late phase Hypofluorescence, isofluorescence, early blocking with late staining, and hyperfluorescence Early window defect pattern of hyperfluorescence with late progressive staining of mid-peripheral atrophic spots and atrophic macular scars Early hyperfluorescence, late staining (more than seen on exam); window defects of atrophic lesions Early hypofluoresence with late hyperfluorescent staining Early hyperfluorescent lesions in wreathlike configuration in mid-retina Early hypofluorescence that subsequently hyperfluorescence in late venous phase
ICGA Numerous hypocyanescent patches in intermediate phase that progress to isocyanescent in late phase Early choroidal stromal vessel hypercyanescence and vascular leakage; hypocyanescent dark dots at level of choroid in late phase; possible disc hyperfluorescence Hypofluorescent granulomas Early increased hypercyanescence from CNV Hypofluorsecent spots (same number as seen on FA) Hypocyanescent spots within choroid (quantities greater than lesions seen on exam) Hypocyanescent dots in early to mid-phases More numerous hypocyanescent lesions than those seen on ophthalmoscopy
ERG/EOG Normal Normal Normal Normal Normal Diffuse loss of function ERG: reduced a-wave; ± abnormal EOG; both typically normalize following resolution ERG: moderate reduction of a- and b-wave amplitudes in acute phase; EOG: abnormal in acute phase but improves with disease resolution
  SPC RPC BCR Neoplastic-PVRL Choroidal lymphoma Syphilis Tuberculosis
Anterior exam findings Mild AC inflammation AC inflammation Typically absent AC inflammation Rare anterior cell AC inflammation is less common AC inflammation common AC inflammation
Posterior examination findings Vitritis; grayish or creamy yellow sub-retinal infiltrates in peripapillary region or macula that progress in irregular serpentine or helicoid fashion centrifugally Vitirits; numerous creamy white lesions initially in peripherally then involvement of posterior pole or macula; bilateral Vitirits; multiple cream or yellowish-white oval lesions varying in size from 1/4 to 1 disk diameter; longer diameter radiating from optic nerve to the periphery Clumped vitreous cells and multiple irregular yellowish white sub-RPE; punched-out lesions leading to a disciform-like scar; retinal vasculitis Vitreous cell; multiple yellow subretinal infiltrates; creamy thickening of choroid diffusely and RPE clumping Yellow, placoid, chorioretinal lesions in posterior pole or within macula Small flat, yellow-white lesions with indistinct borders in the choroid
OCT Hyper-reflectivity and thickening of outer retina; increased reflectance of choroid; disruption of IS/OS junction Pigment epithelial detachment with hyperreflectivity of inner and outer retinal layers CME; focal or generalized disruption of IS/OS junction; possible thinning or absence of Sattler layer; possible appearance of generalized atrophy of the choroid “Placid, rippled, or stormy (seasick)” appearance possible pigment epithelial detachments and exudates above the RPE Tumor cells in sub-RPE Loss of IS/OS junction and ELM “Contact sign”—localized adhesion between RPE-choriocapillaris layer and overlying neurosensory retina
Fluorescein autofluorescence Active lesions are hyperautofluorescent; inactive lesions are hypoautofluorescent Widespread hypoautofluorescence involving the posterior pole and mid-peripheral retina Hypoautofluorescent areas corresponding to areas of chorioretinal atrophy Hyperautofluorescence of RPE over lymphoma deposits; hypoautofluorescent retinal deposits overlying the RPE    
Fluorescein angiography Early hypofluorescence and late hyperfluorescence of the border; Window defects of old lesions Early hypofluorescence and late staining Optic disc hyperfluorescence; vascular leakage; late CME; prolonged arteriovenous transit time (“quenching”) Staining of subretinal deposits; RPE window defects; diffuse RPE granularity RPE granularity; blockage by RPE pigment clumps or disrupted RPE; late staining Hypofluorescent lesions; hypo- and hyperfluorescence in faded part of the lesions, followed by progressive hyperfluorescence Active tubercles—hypofluorescent lesions during dye transit then hyperfluorescent in late frames; tuberculomas—early hyperfluorescence; serpiginous-like choroiditis—hypofluorescence of active edge with late hyperfluorescence of advancing edge
ICGA Hypocyanescent lesions during active phase; hypercyanescence in healing phase; hypocyanescent lesions with clearly defined margins in inactive phase Hypocyanescence lesions that perist into late phase Areas of blockage in early to midphase   Hypocyanescent lesions   
ERG/EOG Normal Normal ERG: delayed 30-Hz implicit time and diminished scotopic b-wave amplitudes; normal EOG Normal   ERG: possibly markedly reduced. EOG: normal