5 March 2018 What are they Debatable White or grey or pale or yellowish lesions in the deep retina Acute onset mostly in in young healthy people Between them features can overlap some think they are different expressions of the same entity ID: 917658
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Slide1
White Dot Syndromes
Jeffrey Whitehead, M.D.
5 March 2018
Slide2What are they?
Debatable
White or grey or pale or yellowish lesions in the deep retina
Acute onset, mostly in in young healthy people
Between them features can overlap- some think they are different expressions of the same entity
Often “run their course” and are ultimately benign
Slide3What are they?
Significant female preponderance in most
Probably under-diagnosed (OK, certainly)
A posterior uveitis, sometimes with vitreous cell and optic nerve involvement (transient edema to atrophy)
Not always pretty- stroke and death are associated rarely with one
Inflammatory- frequently associated with an
antecedant
viral illness
Slide4What’s confusing
Other than everything:
Often not white, often no dots
“Syndrome” connotes a permanent, often genetically or environmentally determined, set of signs- not true for these
Many infectious etiologies have white dots
Why isn’t ocular
histoplasmosis
syndrome included?
Slide5What’s more confusing
Some have no clinically identifiable
fundoscopic
changes- no white and no dot
Some seem like they want to act benign and turn out to be… tuberculosis…metastatic cancer…primary intraocular lymphoma…syphilis…leukemia…fungal
endophthalmitis
(all have white dots)
Sometimes the
fundoscopic
findings have disappeared by the time the patient arrives
Slide6But hey have cool acronyms
MEWDS
AZOOR
APMPPE
PIC
MFC with
panuveitis
SFUS
Slide7Workup & History
With a careful workup and history, the technician working with me will have given me the diagnosis:
“She’s 26, sees flashing lights in the right eye for two days, whether eye is open or closed, vision is blurry in that eye, I measured 20/80 and the left eye is 20/15, says the eyes always saw the same, doesn’t wear glasses or contacts, on confrontational fields she says she can’t see my finger nasally. Had a bad cold a month ago.”
I’m thinking MEWDS and warning myself not to make assumptions without a careful exam
Slide8Nothing is simple except when it is
As a clinician, I am always on guard against seeing what I expect- the brain is powerful in a not-always-appropriate way.
In this case, I surely did not expect to see white puffy lesions below the retina, on the retinal surface, and in the vitreous. White dots maybe, but no white dot syndrome.
Not MEWDS, which was what I was expecting, but
candidal
endophthalmitis
. Further questioning revealed she had undergone bariatric surgery and had been in the hospital for 3 months due to complications.
Slide9Imaging & Other Tests
Good old-fashioned
colour
photographs
OCT: focused on areas dictated by the exam
Visual field testing
Fluorescein angiography
Sometimes
electroretinography
(ERG, EOG,
mfERG
)
Chest X-Ray
Labwork
Slide10Just to be clear
White dots often don’t mean a white-dot syndrome
White dot syndromes sometimes don’t have white dots
They usually, not always, run a benign course
Even so, lingering effects, however minor, may last years
History often unlocks the diagnostic door
Slide11Case 1
Female, 37, healthy
1 week history of stationary black spots in left eye
Headaches, but not necessarily more than usual
20/20 OU
Normal exam, anterior segment and nerve
Trace vitreous cells
Slide12Retina & RPE
OD: deep white fuzzy lesions near fovea, another superior to nerve
OS: jigsaw
plaqueoid
macular and posterior pole lesions, white and grey some with defined edges, others fuzzy
NB:
Fuzzy implies active lesions, sharp-edged inactive
Retinal vessels were normal with signs of leakage or inflammation
Slide13Slide14Slide15Slide16Slide173 days later
Complaints of “spottier vision”
20/20 OU
Headaches no worse, still typical for her
Slide18Day 14
Feels vision is maybe a little better, black spots gray now
20/20 OU
Exam: pigmentation in OS lesions
Slide19Slide20Slide21Day 33
Feels vision is worse- “like looking in a funhouse mirror”
20/20 as always for us
Fundus
autofluorescence
: sharp-edged white lesions
Exam: progressive pigmentation OS and continued healing
Slide22Slide23Slide24Month 4
Less distortion, no headaches, vision close to normal
20/20 (what’s new?)
Slide25Slide26Slide27APMPPE
Acute posterior multifocal
placoid
pigment
epitheliopathy
Probably a manifestation of inflammation or infection at level of RPE &/or
choriocapillaris
No sex predilection, usually under 30
Frequently a viral
prodrome
reported
1-4% may have CNS disease:
vasculitis
with
vaso
-occlusive
sequelae
Slide28APMPPE
Sudden onset of
photopsia
, blurred vision or
scotomata
Most cases bilateral; one eye can precede other
May have inflammatory cells in AC &/or vitreous, or not
Papillitis
can be seen
Multiple deep cream-
coloured
spots deep to retina, usually in macula or posterior pole
Vision 20/20 to 20/40
Slide29Case 2
Female, 23, high
myope
(-10ish) seen previously for a basic retinal exam 20/25 & 20/20 on that visit, lacquer cracks and punctate small white lesions within arcades OU
2 months later presented with decreased vision in her right eye: 20/70 & 20/20 over a week or two
Punctate lesions unchanged, no cells or other inflammatory signs, spot of blood in macula with thickened retina
OCT revealed
hyperfluorescent
ingrowth from choroid into retina:
choroidal
neovascular
membrane
Slide30Slide31Slide324 months later
Patient reports decreased vision and distortion OD
20/25 and 20/20
Exam reveals no blood, no visible thickening, and subtle
opacification
of the fovea
This instance exemplifies the extraordinary subtlety of the OCT image
Slide33Treatment
Treated with anti-VEGF therapy
4 weeks later vision “ is world
s
better” 20/30 & 20/20
Chose observation
4 weeks later vision is 20/25 & 20/20
Slide34Slide35PIC
Punctate Inner
Choroidopathy
Young, female, myopic
No inflammation
May be a subset of another entity to follow- multifocal
choroiditis
&
panuveitis
. As with most of the white dot syndromes, the borders between them can blur
Lesion can look like
histo
spots (but no
peripapillary
atrophy)
Slide36Case 3
Male, 23
Woke up with a blurred haze in left eye
20/20 20/150
No previous illness in last few months
Some flickers of light- eyes open or shut, day or night
Slide37Exam
Normal anterior segment, clear lens (23!)
Sparse vitreous cell
Scattered grey lesions mostly outside the arcades, small and indistinct. Easy to miss.
They seemed in the outer retina, had blurred edges
Nothing else in the retina seemed amiss
Slide38Slide39Slide40Slide41Slide42Course
Day 6: 20/20 20/100
Grey lesions disappearing
Day 36: 20/20 20/30
Grey lesions gone
Macula now granular with tiny white dots (actual white dots!) like hard
drusen
Slide43MEWDS
Multiple evanescent white dot syndrome
Patients mostly young females
ERG changes reverse after episode (RPE changes)
FA shows early non or faint fluorescence with late staining
May be part of AIBSES syndrome (acute
idiiopathic
blind spot enlargement syndrome)
Slide44Scary Symptoms
Female, 28
10 days ago, nightly headaches started, became intractable 6 days ago
Left eye vision went bad- ED gave her Reglan & Benadryl
Vision on presentation 20/15 20/200 (used to be equal)
OD:
placoid
lesions temporal to macula
OS:
placoid
lesions in macula with
subretinal
fluid
Slide45Emergency!
Rapid diagnosis of APMPPE with likely CNS involvement
High dose oral steroids started right away
Counseled patient about potential seriousness of disease
Informed her the eye will almost certainly heal, but the effects of a stroke can be permanent
Critical for close communication and follow-up
Slide46Slide47Slide48Slide49Progress
4 days later: 20/15 20/100
ph
20/40
Headaches gone, feels physically better, steroid taper
14 days later: 20/15 20/60 but vision seems worse, headache back but milder. Prednisone 5 mg /day begun
Another 14 days later: resolving macular lesions, no headache, vision 20/20 20/25
6 months: headache-free, stable vision, 20/15 20/20
Slide50APMPPE
This is a case where it was critical to err on the side of caution- the new onset, severe, abnormal headaches suggested CNS disease. This is associated with the potential for
vasculitis
with stroke and death possible.
The down side of steroids are nothing compared to this.
The next patient is why.
Slide51APMPPE Gone Bad
This patient is now in his early 50s, in for a routine exam
20 years previously he developed what was almost certainly APMPPE, given his residual RPE changes and history of sudden blurry vision, dark spots, and bad headaches in his early 30s
Let’s just say he was not treated appropriately
He suffered 2 CVAs and was left with left homonymous
hemianopsia
, and, remarkably, 20/15 vision OU
Slide52Slide53Slide54Case 5
Female, 36
1 week history of cloudy vision in left eye, floaters and a black line. Saw outside ophthalmologist who put her on
durezol
1 drop q 2 hours OS, and referred her to me
VA: 20/20 20/80
Dense vitreous cell OS, mild OD
Scattered
histo
-like lesions in OD periphery, dense larger lesions peripheral and macular OS
Slide55Differential
Sarcoidosis
, tuberculosis, syphilis, Lyme disease, viral infection,
Bartonella
…
Lab tests, chest
xray
, PPD, all negative
Heavy cell and left eye macular lesions prompted me to put her on high dose oral prednisone
Presumptive diagnosis of multifocal
choroiditis
with
panuveitis
Slide56One day later
20/70 OS, no perceived improvement
Nerve now mildly edematous
Vitreous cell clumping
On 80 mg
predisone
daily, plus
durezol
OU
qid
Slide57Day 4
20/20 20/60, vision per patient just as cloudy
Disc edema improving
Lesions less fuzzy
Pressure good at 14 mg HG OU
Slide58Day 9, 14, 21
Feels OS vision improving
Stopped oral prednisone
Intermitant
photopsias
, 20/20 & 20/60
Sharp-edged macular lesions
Day 14: 20/20 and 20/40
Day 21 20/20 and 20/60, return with 1 drop daily in 2 weeks
Slide59Slide60Slide61Slide62Slide63Slide64Day 35
Worsening vision OS, 20/20 and 20/80
1+ cell in left AC, none in OD
Some active cells in vitreous OS, with old clumped cells
Consult with UW colleague, who recommended a longer course of prednisone- back on 60 mg for 6 weeks
Day 65: 20/20 and 20/50, quieter exam
Still going…
Slide65MFC with panuveitis
Multifocal
choroiditis
with
panuveitis
May well be a different manifestation of PIC and SRFS
Most common in females in their 30s (6-69)
Bilateral, though many second eyes asymptomatic
Syptoms
include decreased central vision,
paracentral
scotomata
, floaters, and
phtophobia
VA at presentation ranges from 20/20 to LP
Slide66Further signs
Several dozen to hundred yellow-grey-white in the mainly peripheral RPE/choroid, round or oval
Some active lesions have
subretinal
fluid
Lesions as they atrophy resemble OHS lesions
May have transient optic disc edema, and CME
Late complications include CNVM in 25-30%
Slide67Diseases similar clinically to MFC
Viral: CMV, HSV, HZV, EBV,
cocksakie
virus
Bacterial:
endophthalmitis
, syphilis, TB, septic
choroiditis
Fungal:
Histo
, candida,
coccidiomycosis
,
cryptococcus
Protozoal
: toxoplasmosis,
Pneumomocystis
carinii
Helminthic: diffuse
uniateral
subacute
neuroretinitis
Insect:
ophthalmia
nodosa
,
ophthalmomyiasis