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

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

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