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WHITE DOT SYNDROMES
Dr.Leo Francis Pacquing
WHITE DOT SYNDROMES
 Birdshot
 APMPPE
 Serpiginous Choroiditis
 MCP
 PIC
 SFU
 MEWDS
 AZOOR
WHITE DOT SYNDROME DDX
 Syphilis
 Diffuse Unilateral Subacute
Neuroretinitis
 Ocular Histoplasmosis
Syndrome
 Tuberculosis
 Sarcoidosis
 Sympathetic Ophthalmia
 VKH
 Intraocular Lymphoma
 Pneumocystis Choroidopathy
 Candidiasis
 ARN
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
WHITE DOT SYNDROMES
etiology
• Unknown
– Infectious
– Autoimmune/ Inflammatory
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
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:
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
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
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
APMPPE PROGNOSIS
FOVEAL Involvement
Older Age
Unilateral Disease
Longer interval of the initial and fellow eye
involvement
Recurrence
RISK FACTORS FOR VISION LOSS
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
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
Serpiginous Choroiditis
FUNDOSCOPY FAF
SOURCE: AAO 2013-2015
New lesions and recurrent attacks
are typical, with up to 38% of patients
Reaching final VA of 20/200 &CF
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
APMME SERPIGINOUS
VIRAL PRODROME
PAPILLITIS
VITRITIS
CNV AT PRESENTATION NO 25%
WORSE VA prognosis
50% NO
POSSIBLE NO
Mild to Mod Minimal
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
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
hyper reflectivity of the choroid
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
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
MCP PIC
OLDER AGE?
SMALLER SPOTS
VITRITIS
CNV AT PRESENTATION 28% 79%
WORSE VA prognosis
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
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
Shows spots that are more
numerous than FA/Examination
ICGA FINDINGS:
MULTIPLE EVANESCENT WHITE DOT SYNDROME
MEWDS
abnormal photoreceptor inner outer segment
junction reflectivity
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
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
Young myopic female
28 % Hx of autoimmune disease
Acute zonal occult outer retinopathy
(AZOOR)
Symptoms:
Unilateral Photopsias, Variable Scotomata
VITRITIS? Mild
CNV? RARE
Funduscopy : MINIMAL funduscopic changes
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
White dot syndrome
White dot syndrome
White dot syndrome

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White dot syndrome

  • 1. WHITE DOT SYNDROMES Dr.Leo Francis Pacquing
  • 2.
  • 3. WHITE DOT SYNDROMES  Birdshot  APMPPE  Serpiginous Choroiditis  MCP  PIC  SFU  MEWDS  AZOOR
  • 4. WHITE DOT SYNDROME DDX  Syphilis  Diffuse Unilateral Subacute Neuroretinitis  Ocular Histoplasmosis Syndrome  Tuberculosis  Sarcoidosis  Sympathetic Ophthalmia  VKH  Intraocular Lymphoma  Pneumocystis Choroidopathy  Candidiasis  ARN
  • 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
  • 6. WHITE DOT SYNDROMES etiology • Unknown – Infectious – Autoimmune/ Inflammatory
  • 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
  • 12. APMPPE PROGNOSIS FOVEAL Involvement Older Age Unilateral Disease Longer interval of the initial and fellow eye involvement Recurrence RISK FACTORS FOR VISION LOSS
  • 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
  • 16.
  • 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
  • 19. APMME SERPIGINOUS VIRAL PRODROME PAPILLITIS VITRITIS CNV AT PRESENTATION NO 25% WORSE VA prognosis 50% NO POSSIBLE NO Mild to Mod Minimal
  • 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
  • 22.
  • 23.
  • 24.
  • 25. hyper reflectivity of the choroid
  • 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
  • 28. MCP PIC OLDER AGE? SMALLER SPOTS VITRITIS CNV AT PRESENTATION 28% 79% WORSE VA prognosis
  • 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
  • 33. MEWDS
  • 34. abnormal photoreceptor inner outer segment junction reflectivity
  • 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
  • 37. Young myopic female 28 % Hx of autoimmune disease Acute zonal occult outer retinopathy (AZOOR) Symptoms: Unilateral Photopsias, Variable Scotomata VITRITIS? Mild CNV? RARE Funduscopy : MINIMAL funduscopic changes
  • 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