5. WHITE DOT SYNDROMES
in general…
• Bilateral Involvement although asymmetrical (except
MEWDS) is a RULE
• Majority of patients are younger than 50 y/0 (except
Birdshot Retinochoroidopathy & Serpiginous Choroiditis
• Female Preponderance in Birdshot choroidopathy, PIC,
MCP, AZOOR &MEWDS
7. BIRDSHOT RetinoChoroidopathy
HLA-A29 is 96% Sensitive
Multifocal, Hypopigmented Ovoid Cream lesions (50-1500 um),
nasal or radial distribution follow the underlying choroidal vessels
No pigmentation over the time
Symptoms? BOV, floaters, Nyctalopia
Confirmatory and not Diagnostic
Vitritis? Common but variable severity
CNV? Rare
Vitiliginous ChorioRetinitis
Fundoscopy
prognosis Up to 88% of patients with aggressive therapy maintain their vision
8. FA Findings
Doesn't’t typically highlight spots
No transmission defect
Quenching phenomenon
ICGA Findings Shows spots- More numerous on exam
ERG
AF Findings
Delayed 30 Hz Flicker Implicit Time Diminished scotopic B wave amplitudes
Both cone and rod abnormal response
Hypoautofluorescence
more numerous and not uniformly correspondent with the birdshot lesions,
suggesting that the choroid and RPE may be affected independently.
Treatment: Initially-Systemic Cortecosteroids
YES!!IMT:
9. Acute Posterior Multifocal Placoid Pigment Epitheliopathy (APMPPE)
Self limited disease
50% have prodromal illness
Symptoms: SUDDEN Onset of Bilateral, Asymmetric Visual loss
BOV, Scotomata, Photopsias
Vitritis: Mild to moderate in 50%
CNV: Rare
APMPPE
Fundoscopy: Multiple large flat,yellow-white placoid lesions at the level of RPE 1 to 2 disc areas
minimal AC inflammation
10. A P M P P E Associations
Non-Infetious
• Erythema Nodosum
• Wegener’s Granulomatosis
• Polyarthritis Nodosa
• Cerebral Vasculitis
• Scleritis & Episcleritis
• Ulcerative Colitis
Infectious
• Group A Streptococcus
• Adenovirus Type 5
• TB
• Lyme Disease
• Mumps
• Hepatitis B vaccination
11. APMPPE
FA Findings Blocks early, Stains Late
Blocks early
ICGA Findings Hypofluorescent Spots= No. in FA
AF Findings HypoAF lesions
treatment Systemic steroids
(Unclear effect)
IMT? NO
13. Serpiginous Choroiditis
Asymmetric Gray White lesions at the level of the RPE in a
Pseudopodial /Geographic manner from the Optic Nerve
Symptoms:
Vitritis?
CNV?
BOV, Scotomas ,
Minimal
25%
Helicoid Choroidopathy
Fundoscopy
chronic, progressive disease
More common in PPD + pateints
quiet anterior chamber
14. Serpiginous Choroiditis
FA Findings:
Blocks early, Stains Late
early hyper or late leakage = CNV
ICG Findings: Hypofluorescent lesions
FAF : Active lesions are HyperAF; inactive Lesions are HypoAF
treatment Systemic steroids + IMT
17. New lesions and recurrent attacks
are typical, with up to 38% of patients
Reaching final VA of 20/200 &CF
18. The addition of SYSTEMIC IMT at the outset
has been suggested as CORTICOSTEROIDS
ALONE ARE INEFFECTIVE
Cyclosporine (Monotherapy)
Prednisone, cyclosporine, Azathioprine (Triple
Therapy) –RAPID REMMISION OF ACUTE DSE
PROLONGED THERAPY—SINCE RECURRENCE is
Frequently Observed.
Anti-VEGF and FOCAL laser photocoagulation
for CNV
20. Young Myopic Female 1/3
Symptoms:
Vitritis:
CNV:
Photopsia, enlarged blind spot, BV
Yes
R/O PIC and OHS
Yes, 28% at presentation
Multifocal Choroiditis and Panuveitis
funduscopy: PUNCH OUT YELLOW WHITE DOTS
prognosis Chronic and recurrent
Up to 75% permanent vision loss
21. FA findings : Blocks Early, stains Late for active lesions
Atrophic lesion : window defect
ICGA findings: Hypofluorescent Spots are more numerous than FA
FAF: Active lesions are HyperAF; Inactive lesions HypoAF
Treatment Systemic steroid + IMT
26. Punctate Inner Choroidopathy (PIC)
Young Myopic Female 2/3
Symptoms: Photopsia, Metamophopsia, BV
Vitritis
CNV YES!; 79% At Presentation
NEVER
CME Rare
prognosis Self limited
Good in absence of CNV
27. FA Findings: Early hyper, late staining
ICGA Findings: Hypofluorescent spont = FA
FAF Active lesions are HyperAF; Inactive lesions HypoAF
TREATMENT
Steroids
Anti VEGF
Laser Photocoag
PDT
IMT: NO
29. MULTIPLE EVANESCENT WHITE DOT SYNDROME (MEWDS)
Young Myopic Female 3/3
Symptoms: ACUTE UNILATERAL
Photopsias, Enlarged blind spot, BV
VITRITIS: Variable
CNV Rare
FUNDOSCOPY: ACUTE PHASE
Multiple discrete white to orange
Spots (100-200um) at the level
of the RPE or Deep retina typically
In a PERIFOVEAL location
“EVANESCENT”
Because those spots are
TRANSITORY and frequently
MISSED
GRANULAR PIGMETARY CHANGE
prognosis Excellent
w/o treatment recovery achievd in 2-10 weeks
30. FA FINDINGS :
Punctate HYPER fluorescent
spots that surrounds the fovea
in a wreath-like configuration
ICGA FINDINGS:
Shows spots that are more
numerous than FA/Examination
ERG FINDINGS:
Diminished A-Wave &
Early Receptor Potential amplitudes
Treatment observation
31.
32. Shows spots that are more
numerous than FA/Examination
ICGA FINDINGS:
MULTIPLE EVANESCENT WHITE DOT SYNDROME
35. WHITE-YELLOW LESIONS (50-500 flm)
located in the posterior pole to midperiphery
at the level of the RPE
Significant anterior segment inflammation
and mild to moderate VITRITIS are
typically present BILATERALLY
36. Subretinal fibrosis and uveitis
• Female > 95% (healthy myopic women between the
ages of 14 and 34 years)
• FA: early block (hypo)and hyper; late staining w/o leak
• Complication: neurosensory RD , CME , CNVM
• Tx: cortiocosteroid , IMT
38. FAF:
HYPO-AF for the atrophied
choriocapillary and HYPER-AF at the
BORDER of the expanding lesion
OCT: Loss of ellipsoid zone & ONL
FA:
early stage of dis:NL
HYPER, HYPO and window defects
corresponding to zones of RPE
derangements
cortiocosteroid , IMT , Antivirals
Unclear effect of treatment
on vision and disease
course
TX
EOG:
Delayed 30 Hz FlickerERG:
reduction in light rise
