Disease [References] Age

Sex

Laterality

Symptoms Vision loss, Scotoma Viral Prodroma Meningeal symptoms

Retinal findings Yellow- white creamy placoid lesions that may be confluent at level of RPE and choriocapillaris Multiple 10-100 white Lesions at level of outer retina, RPE and choroid, disc edema Peripapillary atrophic scars with leading edge of retinal whitening, edema, and fluid. Lesions involve the RPE, retina, choriocapillaris and choroid Numerous creamy white/yellow lesions at the level of outer retina and choroid radiating from disc most prominent nasally

Location

A/C cells

Vitreous cells

RSFA/ICG Early hypofluorescence and late hyperfluorescence of active lesions Punctate staining of RPE Wreath sign, Disc leakage. ICG- Multiple hypocyanescent spots in posterior pole

Visual Fields

EOG ERG

Prognosis

Etiology

APMPPE

2nd -3rd Decades

M=F

Bilateral

Posterior pole

none

Minimal

Central scotoma

N/A Reduced awave amplitude in acute phases which resolves

Excellent

Unknown

Treatment None except steroids for cerebral vasculitis

Course

Differential Diagnosis Metastases Viral retinitis Tosplasma RC Pneumocystis C APMPPE AMN MCP Birdshot DUSN PIOL Sarcoidosis Tuberculous Choroiditis APMPPE RPC MCP OHS Pars Planitis Papilledema PIOL VKH MEWDS OHS SO Sarcoidosis \OHS (novitritis), VKH PIC SFU SO Birdshot Serpiginous Myopic Degeneration, Sarcoidosis Sarcoidosis, OHS Toxoplasmosis MCP Birdshot

Self limited 2- 12 weeks Self-limited 4-12 week; 10-15% of cases may recur Progressive serpentine lesion progression into macula 33%-CNV 20%<20/400

MEWDS

2nd - 4th decades

F>>M

Unilateral

Photopsias Scotoma

Posterior Pole

none

Minimal to mild

Enlarged Blind Spot

Excellent

Unknown

None

Serpiginous Choroiditis

2nd-7th Decades

M=F

Starts Unilateral becomes Bilateral

Scotoma Vision Loss Metamorphopsia

Peripapillary serpentine progression into macula Radiating away from disc involving nearly entire retina

none

Minimal

Early Hypofluorescence and late hyperfluorescence of active lesions

Scotomata

N/A Reduction of B-wave, prolonged implicit times Late Extinguished

Guarded/Poor

Unknown Tuberculous choroiditis looks same

Steroids IMA

Birdshot Retinochoroidopathy

3rd-6th Decades

F>M

Bilateral

Floaters Blurred Vision Nyctalopia

Minimal

Moderate to Severe

Disc leakage, CME, perivascular staining Early Hypofluorescent and late hyperfluorescent lactive lesions, CNVM, ICGHypocyanescent spots around optic disc Same as MCP but Fibrotic scars hyperfluorescent and stain in late stages of disease Acute Early Hyperfluorescence and late staining and leakage with pooling in subretinal space, CNVM No leakage, Hyperfluorescence in a bull's eye pattern

Constriction of peripheral field

Guarded

Unknown Autoimmune reaction to Retinal S-Ag

Steroids IMA

Gradually Progressive even with treatment but can be slowed

Multifocal Choroiditis and Panuveitis Syndrome (MCP) Progressive Subretinal Fibrosis and Uveitis Syndrome (SFU)

2nd-6th Decades

F>>M

Bilateral

Floaters Photopsias Blurred Vision Enlarged blind spot

2nd-3rd Decades

F>>M

Bilateral

Floaters, Vision loss

Disc hyperemia Multiple (100s)50-350 yellow-white creamy to punched out lesions at level of choroid White subretinal fibrotic lesions progressively coalesce involve most of retina and choroid; lesions involve the choroid and RPE Small yellow-white lesions at level of inner choroid with serous retinal detachment, older atrophic scars, CNV Cluster of 2-4 browngray spots at level of RPE around fovea become more black and eventually fade

Peripapillary Posterior Pole Initially Posterior pole Then Entire retina and choroid

Mimimal

Moderate to severe

Enlarged blind spot,/ scotoma

N/A

GuardedPoor if CNV involving fovea

Unknown

Steroids, Rarely IMA

Waxing and waning and progressive, 25-33% may develop CNV

Steroids Enlarged Blind spot, Scotoma N/A Poor Unknown IMA (required) Steroids for Serous RD involving fovea, Treat CNV Relentlessly Progressive

Severe

Severe

Punctate Inner Choroidopathy (PIC) [19] Acute Retinal Pigment Epitheliitis [22]

2 -4 decades

nd

th

F>>M

Bilateral

Myopia Scotoma Metamorphopsia Photopsia

Peripapillary Posterior pole

None

Minimal - Mild

Scotoma

N/A EOGSubnormal; ERGnormal

Guarded Poor if Subfoveal CNVM

Unknown

Waxing Waning With Recurrences

Same as MCP Rubella retinitis AMN PIC CSR

2nd -4th Decades

M=F

Unilateral

Minimal to severe visual loss

Macula

none

none

Normal

Good

?viral

None

Spontaneous improvement in 8-12 weeks

Acute Macular NeuroRetinopathy (AMN) [23]

2nd-4th decades

F>>M

Unilateral or bilateral

Visual loss and paracentral scotomata, Viral Prodrome in some patients

Several red-brown small circular to oval petaloid lesions at level of outer retina and RPE around fovea

Macula

none

none

Normal

Paracentral scotomas that correlate to shape of foveal petaloid lesions

Normal

Good

?viral

None

Vision improves but scotomas fade slowly over months. Retinal lesions do not fade. Recurrences possible

Acute Retinal pigment epitheliitis, MEWDS, APMPPE, CSR, Optic neuritis, Old Inner retinal infarcts

Acute Zonal Occult Outer Retinopathy (AZOOR) [25]

2nd-4th decades

F>>M

Unilateral or bilateral

Photopsias; Visual field loss

Initially- normal, then narrowed retinal vessels. Then Depigmented RPE zones correlating to visual field loss; rare acute lesions look progressive line or area of inflammation at level of outer retina and RPE

Perivascular in posterior pole and mid periphery

None

Rare cells

Early- Normal. Later in courseWindow defects in areas of depigmented RPE

Large superotemporal and occasional central zones of field loss that increase in size over a few days or weeks and then stabilize. Permanent.

ERGSeverely reduced awave amplitudes

Fair

Unknown

None

Vision remains 20/40 or better but severe visual field loss can occur.

Cancer associated retinopathy, Retinal vasculitis, Syphilitic Chorioretinitis, DUSN, RP

Relentless Placoid ChorioRetinitis (RPC) ( ampiginous serpiginous) [21]

2nd-6th Decades

M=F

Bilateral more common

Vision loss, pericentral scotomas, photopsias, floaters, and rarely, pain

Placoid white-yelllow lesions similar to APMPPE or serpiginous one dis diameter or less in size. Lesions are at level of RPE, choriocapillaris, and choroid, and outer retina. Heal over weeks with chorioretinal atrophy. But usually the lesions increase in size and new lesions develop in all patients.

Mid- and Farperipheral lesions prior to macular involvement

Variable

Variable

Early hyperfluorescence and late hyperfluorescene of active lesions

Paracentral and peripheral scotomas

N/A

Fair to Good

Unknown

Steroids, Other IMA

Growth of subacute lesions and new lesions develop 5-24 months after diagnosis. Relapses common. 50 to 100s of lesions develop in most patients in periphery and posterior pole. Central vision remains 20/40 or better even with macular involvement.

APMPPE PPM Viral retinitis, Choroidal Vasculitis, Serpiginous, MCP, Birdshot, VKH, Choroidal Mets, Syphilitic CR, Sarcoidosis, Tuberculous choroiditis

Unifocal Helioid Choroiditis [27,28]

1st -3rd decades

M=F

Unilateral

Vision loss and/or metamorphopsia

One disc-diameter, yellow-white, thick (up to 1mm) choroidal lesion with overlying subretinal fluid and, sometimes, hemorrhages

Posterior pole macula

Usually none , rarely severe

Usually none, rarely severe

Hyperfluorecent choroidal lesions that leak. Choroidal neovascularization may be seen.

N/A

N/A

Variable depending on the proximity of lesion to macula

Unknown

No effective treatment found Oral Steroid for acute disease but does not prevent CNV. CNV treated with laser, PDT, or antiVEGF agents

Growth of lesion with increasing subretinal fluis for 1-18 months with gradual reduction of retinal thickness and fluid

Histoplasma choroiditis Toxoplasma RC Sarcoidosis Fungal choroiditis

Persistent Placoid Maculopathy (PPM) [29]

6th-7th Decades

M>>F

Bilateral Symmetric

Decreased Vision Photopsias

Well-delineated Jigsaw patterned whitish plaque-like lesion at level of RPE and Choriocapillaris involving fovea and not contiguous with the optic disc

Posterior pole and macula

None

None

Acute macular lesions hypofluorescent early and partially fill but do not leak in late phases unless CNV ICG- Persistent hypofluorescence of macular lesions

N/A

N/A

Poor: due to subfoveal CNV

Unknown

Initially good vision maintained for years but nearly all eventually develop CNV

Macular Serpiginous, APMPPE, RPC, Syphilitic Placoid chorioretinitis, Choroidal vasculitis

Unilateral Acute Idiopathic Maculopathy [24]

2nd-4th Decades

M=F

Unilateral

Flu-like Prodrome preceding sudden, vision loss

Wedge shaped exudative detachment of macula with irregular borders White-grey thickening of RPE

Macula

None

Mild vitreous cells overlying macular lesion

Early phases show alternating areas of hyperand hypofluorescence beneath the serous retinal detachment Late phases show staining of RPE and pooing of fluorescein

Central scotoma

N/A

Good (>=20/25)

Association with Coxsackie virus and hand, ,foot, and mouth disease

None

Neurosensory retinal detachment resolves spontaneously with RPE atrophy in central maculagiving Bulls Eye appearance

Idiopathic CNV CSCR VKH syndrome Serpiginous Posterior scleritis APMPPE Placoid syphilitic CR RPE detachment

Legend: RC- Retinochoroiditis C- Choroiditis CSR- Central serous retinochoroidopathy VKH- Vogt-Koyanagi-Harada Syndrome SO Sympathetic Ophthalmia RPE Retinal pigment epithelium