White dot syndromes

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A range of inflammatory diseases of the retina, RPE and choroid
There are three well defined/distinct clinical syndromes:
- AMPEE
- Birdshot
- Serpiginous chorioretinopathy
The remaining white dot syndromes exist on a spectrum
All tend to affect young women (typically myopes) except Serpiginous Choroidopathy and Birdshot which affect the middle-aged.

Acute posterior multifocal placoid pigment epitheliopathy (AMPPE)

Young adults
Usually bilateral
Flu-like prodrome
Possibly related to patchy choroidal ischaemia/vasculitis

Clinical features

Reduced vision sequentially
Post-equatorial placoid lesions of the RPE: creamy white then fading
Later pigmentary changes
Mild vitritis

Tests

FFA: early hypofluorescence and late hyperfluorescence (‘block early, stain late’)
ICG: hypofluorescence of placoid lesions
OCT: dome elevation of the placoid lesions at the outer retina
OCTA: islands of altered flow and non-perfusion
AF: hypofluorescence in acute phase

Natural history

Self-limiting within 2-3 months
- Rarely progresses to a serpiginous choroiditis requiring steroids.
Associated with CNS vasculitis: any neurological symptoms (such as headache require prompt neurology evaluation.

Birdshot chorioretinopathy aka vitiliginous chorioretinitis

Bilateral
Unknown aetiology
Middle-aged Caucasians
F>M slightly
HLA-A29 association

Ocular features

Reduced vision
Reduced colour vision
Floaters
Night blindness
Oval cream-coloured lesions radiating from the optic disc to the equator associated with choroidal vessels
Moderate vitritis
Vasculitis
CMO
Lesions become atrophic with time
CNV
Optic atrophy

Tests

HLA testing to identify HLA-A29 (>95% patients): if negative consider sarcoidosis which can appear similar
OCT: CMO and outer retinal changes
OCTA: disruption to the RPE with reducing underlying choroidal blood flow
FFA: early hypofluorescence of lesions with late hyperfluorescence. Hyperfluorescence of the disc, retinal vessel leakage, CMO
- May show ‘quenching’: quick fading of the dye from retinal circulation
ICG: hypofluorescent spots showing inactive lesions (more lesions seen on ICG than FFA)
VF
ERG: reduced b-wave amplitude and latency
EOG: reduced Arden index

Hot Topic

Electrodiagnostics can play a useful role in diagnosis of Birdshot

Management

Corticosteroids and long-term immunosuppression with MMF

Serpiginous choroidopathy

Rare
Bilateral
Middle aged
Resembles AMPEE but has much worse prognosis
Associated with TB and syphilis (or these cause a similar picture)

Clinical Features

Reduced vision
Asymptomatic until macular involvement
Serpiginous, placoid or geographic lesions at the RPE/inner choroid level: greyish-yellow, spread centrifugally from the disc
- Become atrophic over months with irregular depigmentation/pigmentation
Mild vitritis
CNV: rare
Subretinal scarring

Tests

OCT: hyperreflective outer retina and RPE, choroidal hyperreflectivity (‘reverse shadowing’)
AF: demarcates lesions with hyperfluorescent active edge and hypofluorescent inactive lesions due to loss of RPE
FFA: early hypofluorescence and late staining (and early hyperfluorescence at the edge of lesions)
- ICG: hypofluorescence

Management

Corticosteroids/immunosuppression in the acute phase eg mycophenolate, cyclosporine, azathioprine, prednisolone
IVT anti-VEGF

Multiple evanescent white dot syndrome (MEWDS)

Rare
Typically affects young myopic women
Unilateral
Flu-like prodrome

Ocular Features

Reduced vision
Scotomata
Photopsia
Transient RAPD
White dots at the level of the outer retina/RPE
Orange-white dots at the fovea (‘foveal granularity’)
Mild vitritis

Tests

OCT: attenuation of the IS-OS junction (ellipsoid zone) resolves within weeks/months. In the right clinical context, disruption of the normal IS-OS and RPE layers is effectively diagnostic of MEWDS
FFA: wreath-like punctate hyperfluorescence corresponding to white dot areas with late staining, disc leakage
ICG: hypofluorescent dots around optic disc
ERG: reduced a-wave

Natural history

Self-limiting within 2-3 months

Acute retinal pigment epitheliitis (ARPE, Krill disease)

Rare
Unilateral
Young adults
Self-limiting within 1-3 months
Slightly reduced vision
Metamorphopsia
Foveal pigment spots with yellow-white halo
FFA: hyperfluorescent spots
OCT: hyperreflective accumulations within the RPE

Acute zonal occult outer retinopathy (AZOOR)

Rare
May be bilateral (60% eventually involve second eye) especially in males
Affects young, myopic females (like MEWDS)
Flu-like prodrome

Ocular Features

Acute scotomata (zonal ** field loss**) worse in bright conditions
Photopsia
Fundal lesions: on OCT/AF these have a demarcating line at the level of the outer retina in a trizonal pattern of sequential involvement: outer retina, RPE and choroid
Mild vitritis
Retinal/choroidal atrophy and RP-like pigmentation

Tests

ERG: variably abnormal in a patchy distribution
EOG: loss of light rise
FFA: may be normal initially

Management

Best treatment is unclear
Tends to stabilize by 6 months but causes visual field defects corresponding to areas of RPE atrophy

Multifocal choroiditis with panuveitis (MCP) and punctate inner choroidopathy (PIC)

Inflammatory conditions of the choroid and retina
Bilateral
Grey or yellow-white lesions
MCP: visible inflammation (especially vitritis)
PIC: no visible inflammation. Tends to affect younger patients
F>M
There may be a viral aetiology

Clinical Features

Reduced vision
Scotomata
Photopsia
MCP: choroidal lesions, vitritis, anterior uveitis, CMO, subretinal fibrosis, CNV (CNV is the most common visually significant complication of MCP)
PIC: quiet eye, inner choroidal/retinal lesions, yellow-white with fuzzy borders then become atrophic and pigmented scars similar to POHS, serous RD, CNV (visual prognosis is very good unless CNV develop)

Tests

OCT
AF: reveals active lesions at an early stage with hyperfluorescent spots with hypofluorescent centre
OCTA
FFA: early punctate hypofluorescence and late hyperfluorescence of lesions, may reveal CNV
ICG: hypofluorescent lesions

Management

IVT anti-VEGF for CNV
Intravitreal corticosteroids
Immunosuppression may have a role
Rescue corticosteroids may be used in CMO