The white dot

syndromes
Group of inflammatory chorio-retinopathies of
unknown etiology which have in common a unique and
characteristic appearance of multiple yellow-white
lesions affecting multiple layers of the retina, retinal
pigment epithelium (RPE), choriocapillaris, and the
choroid.
 D.D
 Multiple evanescent white dot syndrome(MEWDS)
 Acute posterior multifocal placoid pigment
epitheliopathy(APMPPE)
 Punctate inner Choroidopathy(PIC)
 Birdshot chorioretinopathy
 Multifocal choroiditis and panuveitis
 Serpiginous Choroidopathy
 Multiple evanescent white dot
syndrome(MEWDS)

 Young adult (F>M)

 Unilateral
 Small, subtle, deep grey white or yellow white dots deep in retina
 Orange macular granularity
 Mild vitritis
 Aqueous cells rarely present
 generally characterized by a good visual prognosis with no need
for treatment.
 Color fundus photographs

Color fundus photographs of the left eye showing a

hyperemic, swollen optic disc and multiple white dots
of the posterior pole.
 Fundus auto fluorescence (FAF)

reveals
 typical pattern of multiple hyper auto-fluorescent
roundish dots predominantly located at the posterior
pole and around the disk.
 The spots can be separated from each other or grouped
in a coalescent pattern
 Sparing fovea
 Spectral domain optical coherence tomography
(SD-OCT)

 Reveal moderately reflective focal lesions

within outer photoreceptor layer
 the lesions show a disruption of the IS/OS
junction.
 The RPE appears preserved in most of the
cases
 Fluorescein angiography (FA)

findings include
 generic signs of inflammation such as hot disk
 more characteristic features represented by:
multiple hyper-/hypo-fluorescent dots corresponding to the
retinal lesions in the early phases of the angiogram (punctate
lesions)that appear on closer examination as wreath like
pattern in early fluorescein that become hyper-fluorescent
and more visible in the late phases of the exam
 Indocyanine green angiography (ICGA)

 Multiple hypo-fluorescent spots in posterior pole & hypo-

fluorescence around ONH in pt. with enlarged blind spots
 OCT imaging of the optic disc

OCT imaging of the optic disc

confirmed optic disc swelling
which was reflected by the
enlarged blind spot (EBS) seen
on the Humphrey Visual Field
(HVF)
Follow up
 Acute posterior multifocal placoid pigment
epitheliopathy(APMPPE)

 a rare inflammatory disease, classified as part of the spectrum of  Young adult (F=M)
the white dot syndromes  Associated with HLA-B7 & DR2
  the most likely primary inflammatory involvement of the  Bilateral
choriocapillaris with secondary photoreceptor disruption  Symmetrical
 generally a self-limited condition
 requires no treatment
 has a good prognosis.
 Color fundus photo

 Multiple creamy yellow-white lesions throughout the

macula.
 There is mild vitritis on exam
 mild disc edema.
 Spectral domain optical coherence
tomography (SD-OCT)

 Bilateral disruption of the outer retinal

and ellipsoid zone, with hyper-
reflectivity of outer retinal layers and
retinal pigment epithelium (RPE)
 bilateral increase in choroidal
thickness.
four distinct OCT phases of APMPPE:
 beginning with a dome-shaped elevation with disruption of the
photoreceptor junction, that flattens soon after.
 Two weeks later, the second stage is characterized by the detection
of a distinct separation between the photoreceptor junction and the
RPE.
 The third stage, occurring six weeks after disease onset, is
characterized by increased RPE hyper-reflectivity and union of the
RPE and photoreceptor junction
 followed by the resolution phase, starting at three months post
disease onset, with the reformation of two distinct visible layers of
photoreceptors and RPE. However, focal areas of
photoreceptors/RPE atrophy can develop.
 Fundus auto fluorescence (FAF)

Bilateral initial hypoautofluorescence

corresponding to the hyper-reflectance of the
outer retinal layers on optical coherence
tomography (OCT)
 Fluorescein angiography (FA)

Laminar phase of fluorescein angiogram

Late phase of fluorescein angiogram demonstrating late
demonstrating early hypo-fluorescence of the
staining of lesions
lesions
Punctate inner Choroidopathy(PIC)

Young myopic females

Eventually bilateral but asymmetrical
All same age
No vitritis
 Color fundus photographs

 small (100-200 µm) yellow-white chorio-retinal

lesions in the posterior pole
 rarely extending to the midperiphery.
 Fluorescein angiography (FA)

Early fluorescein angiography Late fluorescein angiography

early hyper-fluorescence and late

staining of the lesions
 Indocyanine green angiography (ICGA)

shows multiple mid-phase hypo-fluorescent lesions in

the peripapillary posterior pole
 The lesions progress to deep, punched-out chorio-retinal scars, often with surrounding
hyperpigmentation
 The disease course is self-limited and there is generally a good visual prognosis, though vision can
be limited in the presence of choroidal neovascularization.
Birdshot chorioretinopathy

 Middle aged adult (F>M)

 Associated with HLA-A29
 Bilateral
 typically affects peripheral vision more than central vision, except when
macular complications develop
 posterior uveitis 
 a gradual decline in visual acuity over time due to cystoid macular
edema and retinal atrophy
 The mainstay treatment of BSCR is steroid-sparing immunomodulatory
therapy (IMT)
 Fundus photograph

Essential criteria for the diagnosis of BSCR

(1) bilateral disease
(2) three or more characteristic birdshot lesions defined as
 creamy-colored
 irregular or elongated, choroidal lesions
 with indistinct borders inferior and nasal to the disk
(3) low-grade anterior chamber inflammation (less than 1+ anterior
chamber reaction)
(4) low-grade vitreous inflammation (less than 2+ vitreous cells or
haze)
 Fundus autofluorescence

 Fundus auto-fluorescence did not show abnormality in the

periphery.
 However, hypo- and hyper-autofluorescent patches are seen
in the macula
 Fluorescein angiography

 Fluorescein angiography demonstrated

 few hypo-fluorescent foci in early (a) and late (b)
phases indicating choroidal inflammation.
 However, there were no vascular and optic nerve
leakages.
 There were also no evidences to suggest the presence of
choroidal neovascularization
 Spectral-domain optical coherence tomography
(OCT)

(SD-OCT) shows choroidal thinning and RPE

changes in addition to intraretinal cystic spaces
in the macula
 Multifocal choroiditis and panuveitis

 a rare disease
 It mainly affects otherwise healthy young adults although
children and elderly individuals are also affected
 F>M
 Bilateral asymmetrical involvement
 Moderate myopia is frequently associated with MCP.
 Severity and prognosis are very variable.
 Fundus photograph

 Characteristic findings include punched-out white-yellow

dots (50-200 µm) throughout the fundus and vitritis
 The lesions eventually turn into atrophic scars with variable
amounts of hyperpigmentation.
 Peripheral chorio-retinal streaks can sometimes be visualized
 Fluorescein angiography

(B) early venous phase fluorescein angiogram showing

variable hypo- and hyper-fluorescence of the lesions and lacy hyper-
fluorescence at the fovea indicating choroidal neovascularization
(C) late phase
showing hyper-fluorescence at the fovea due to leakage from
choroidal neovascularization
Serpiginous Choroidopathy

Middle age adult (F=M)

Eventually bilateral but asymmetrical
Associated with  HLA-B7
Recurrent
Mild vitritis
Poor prognosis & macular scarring
 Fundus photograph

 It is characterized by atrophic scars with

 Active
 yellow-gray borders
 often extending in a serpent-like pattern from the optic disc.
 Initially peripapillary & at posterior pole then outward
spread
 Fundus auto-fluorescence

There is a hyper-auto-fluorescent border at the

temporal border of the lesion indicating active
inflammation.
 Fluorescein angiography

the helicoid pattern of chorio-retinal scarring extending fromFluorescein Angiogram:

the optic nerve. FAF shows hypoautofluorescence of the lesion  There is blockage of fluorescence in the early frames in the
borders area of the active disease(central hypo + hyper rim)
 In the late frames (labeled numbers 3 and 4), the margins of
this area hyper-fluoresce(hyper leaks into lesion spotty
staining)
During a period of quiescence,
color fundus photography demonstrates :
 a helicoid pattern of chorio-retinal atrophy emanating from
the optic nerve (Baseline Color)
fundus auto-fluorescence shows:
 corresponding areas of hypo-auto-fluorescence (Baseline
FAF).
5 months later, despite immunosuppressive therapy, disease
activity recur with a new area of retinal whitening on color
fundus photography (5 months Color, arrow).
 The newly active lesion appeared mostly hyper-auto-
fluorescent on FAF.
 OCT demonstrated outer retinal loss and RPE disruption in
the areas of lesions.
To summarize….
Thank u