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WHITE DOT SYNDROMES
Leo Francis Pacquing
Resident-in-Training Year3
DOH EYE CENTER
Mon, APRIL 27, 2015
WHITE DOT SYNDROME DIFFERENTIAL
• Syphilis
• Diffuse Unilateral Subacute
Neuroretinitis
• Ocular Histoplasmosis
Syndrome
•...
WHITE DOT SYNDROMES
• Birdshot
• APMPPE
• Serpiginous Choroiditis
• MCP
• PIC
• SFU
• MEWDS
• ARPE
• AZOOR
WHITE DOT SYNDROMES
in general…
• Bilateral Involvement although asymmetrical
(except MEWDS) is a RULE
• Majority of patie...
WHITE DOT SYNDROMES
etiology
• Unknown
– Infectious
– Autoimmune/ Inflammatory
• May be inherited Immune dysregulation tha...
WHITE DOT SYNDROMES
Acute Zonal Occult Outer Retinopathy (AZOOR)
Multifocal Choroiditis and Panuveitis (MCP)
Multiple Evan...
Lymphoma-- PIOL
RetinoVitreal Lymphoma;
BIRDSHOT RetinoChoroidopathy
HLA-A29 is 96% Sensitive
Multifocal, Hypopigmented Ovoid
Cream lesions (50-1500 um)
Symptoms?...
Vitritis Sequelae?
FA Findings Doesn't’t typically highlight spots
ICGA Findings Shows spots- More numerous on exam
ERG
AF Findings
Delayed 3...
WHITE DOT SYNDROME?
SYPHILIS
•SUDDEN
Onset of Bilateral,
Asymmetric Visual
loss associated
with scotoma
Acute Posterior Multifocal Placoid Pigment Epitheliopathy (APMPPE)
50% have prodromal illness
Symptoms: BOV, Scotomata, Ph...
A P M P P E Associations
Non-Infetious
• Erythema Nodosum
• Wegener’s Granulomatosis
• Polyarthritis Nodosa
• Cerebral Vas...
APMPPE
FA Findings Blocks early, Stains Late
Blocks early
ICGA Findings Hypofluorescent Spots= No. in FA
AF Findings HypoA...
APMPPE PROGNOSIS
RISK FACTORS FOR VISION LOSS
FOVEAL Involvement
Older Age
Unilateral Disease
Longer interval of the initi...
Serpiginous Choroiditis
Asymmetric Gray White lesions at the level of the RPE in a
Pseudopodial /Geographic manner from th...
Serpiginous Choroiditis
FA Findings: Blocks early, Stains Late (*plus the CNV findings if Present)
ICG Findings: Hypofluor...
FUNDOSCOPY FAF
Serpiginous Choroiditis SOURCE: AAO 2013-2015
Serpiginous Choroiditis
FUNDOSCOPY FAF
SOURCE: AAO 2013-2015
WHITE DOT SYNDROMES
Acute Zonal Occult Outer Retinopathy (AZOOR)
Multifocal Choroiditis and Panuveitis (MCP)
Multiple Evan...
• Features of Both APMPPE and Serpiginous
• lesions are similar both clinically and angiographically, but the
clinical cou...
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 (Monot...
Tb-SLC
Ocular Histoplasmosis Syndrome
Multiple white atrophic chorioretinal scar
(“spots”)
Peripapillary Pigment changes
CNV Macu...
Young Myopic Female 1/3
Symptoms:
Vitritis:
CNV:
Photopsia, enlarged blind spot, BV
Yes
Yes, 28% at presentation
Multifoca...
FA findings : Blocks Early, stains Late for active lesions
ICGA findings: Hypofluorescent Spots are more numerous than FA
...
1999
2002
PHOTO FAF
RESOLVING VKH
Punctate Inner Choroidopathy (PIC)
Young Myopic Female 2/3
Symptoms: Photopsia, Metamophopsia, BV
Vitritis
CNV YES!; 79% A...
FA Findings: Early Hyperfluorescence, late staining
ICGA Findings: Hypofluorescent spont = FA
FAF Meh
IMT: NO
TREATMENT
St...
SARCOIDOSIS
Characteristic Ocular Findings
Granulomatous Anterior Uveitis
• Vitreous Inflammation (Diffuse
Inflammation
Sn...
MULTIPLE EVANESCENT WHITE DOT SYNDROME (MEWDS)
Young Myopic Female 3/3
Symptoms: ACUTE UNILATERAL
Photopsias, Enlarged bli...
FA FINDINGS :
Punctate HYPER fluorescent
spots that surrounds the fovea
in a wreath-like configuration
ICGA FINDINGS:
Show...
Shows spots that are more
numerous than FA/Examination
ICGA FINDINGS:
MULTIPLE EVANESCENT WHITE DOT SYNDROME
MEWDS
SYMPATHETIC OPHTHALMIA
1st one to raise his/hand
3 clues
CLUE1: NOT a white dot Syndrome
CLUE 2 Diffuse non Necrotizing
Gr...
ARN
SLE
WHITE-YELLOW LESIONS (50-500 flm)
located in the posterior pole to midperiphery
at the level of the RPE
Significant anteri...
Difficult DX.. CALLED OCCULT FOR A REASON
Acute zonal occult outer retinopathy
(AZOOR)
Symptoms:
Unilateral Photopsias, Va...
WHITE DOT SYNDROMES
Acute Zonal Occult Outer Retinopathy (AZOOR)
Multifocal Choroiditis and Panuveitis (MCP)
Multiple Evan...
MCP PIC
OLDER AGE?
SMALLER SPOTS
VITRITIS
CNV AT PRESENTATION 28% 79%
WORSE VA prognosis
APMME SERPIGINOUS
VIRAL PRODROME
PAPILLITIS
VITRITIS
CNV AT PRESENTATION NO 25%
WORSE VA prognosis
50% NO
POSSIBLE NO
Mild...
BIRDSHOT APMPPE SERPIGINOUS MCP PIC MEWDS
BLOCKS
EARLY
STAINS
LATE
yes yes yes yes no no
ICGA
SPOTS
more
Numerous
on FA
Ye...
GOOD MORNING
Chorioretinopathies of Unknown Etiology (WhiteDots)
Chorioretinopathies of Unknown Etiology (WhiteDots)
Chorioretinopathies of Unknown Etiology (WhiteDots)
Chorioretinopathies of Unknown Etiology (WhiteDots)
Chorioretinopathies of Unknown Etiology (WhiteDots)
Chorioretinopathies of Unknown Etiology (WhiteDots)
Chorioretinopathies of Unknown Etiology (WhiteDots)
Chorioretinopathies of Unknown Etiology (WhiteDots)
Chorioretinopathies of Unknown Etiology (WhiteDots)
Chorioretinopathies of Unknown Etiology (WhiteDots)
Chorioretinopathies of Unknown Etiology (WhiteDots)
Chorioretinopathies of Unknown Etiology (WhiteDots)
Chorioretinopathies of Unknown Etiology (WhiteDots)
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Chorioretinopathies of Unknown Etiology (WhiteDots)

Reported 2015, my last report in UVEA; based on AAO--With the help of Dr, Daniel Baskin and Dr, Pia Galvante

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Chorioretinopathies of Unknown Etiology (WhiteDots)

  1. 1. WHITE DOT SYNDROMES Leo Francis Pacquing Resident-in-Training Year3 DOH EYE CENTER Mon, APRIL 27, 2015
  2. 2. WHITE DOT SYNDROME DIFFERENTIAL • Syphilis • Diffuse Unilateral Subacute Neuroretinitis • Ocular Histoplasmosis Syndrome • Tuberculosis • Sarcoidosis • Sympathetic Ophthalmia • VKH • Intraocular Lymphoma • Pneumocystis Choroidopathy • Candidiasis
  3. 3. WHITE DOT SYNDROMES • Birdshot • APMPPE • Serpiginous Choroiditis • MCP • PIC • SFU • MEWDS • ARPE • AZOOR
  4. 4. 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
  5. 5. WHITE DOT SYNDROMES etiology • Unknown – Infectious – Autoimmune/ Inflammatory • May be inherited Immune dysregulation that predisposes to Autoimmunity
  6. 6. WHITE DOT SYNDROMES Acute Zonal Occult Outer Retinopathy (AZOOR) Multifocal Choroiditis and Panuveitis (MCP) Multiple Evanescent White Dot Syndrome (MEWDS) Acute Annular Outer Retinopathy (AAOR) Punctate Inner Choroidopathy( PIC) Acute Idiopathic Blind Spot Enlargement Syndrome (AIBES) AZOOR-Complex Diseases Acute Posterior Multifocal Placoid Pigment Epitheliopathy (APMPPE) Serpigenous Choroidopathy Birdshot Retinochoidopathy *duh” doesn’t have white dots on them * * *
  7. 7. Lymphoma-- PIOL RetinoVitreal Lymphoma;
  8. 8. BIRDSHOT RetinoChoroidopathy HLA-A29 is 96% Sensitive Multifocal, Hypopigmented Ovoid Cream lesions (50-1500 um) Symptoms? BOV, floaters, Nyctalopia Confirmatory and not Diagnostic Vitritis? Common but variable severity CNV? Rare Vitiliginous ChorioRetinitis Fundoscopy Choroid and RPE
  9. 9. Vitritis Sequelae?
  10. 10. FA Findings Doesn't’t typically highlight spots ICGA Findings Shows spots- More numerous on exam ERG AF Findings Delayed 30 Hz Flicker Implicit Time Diminished scotopic B wave amplitudes Hypoautofluorescence (meh) Treatment: Initially-Systemic Cortecosteroids YES!!IMT:
  11. 11. WHITE DOT SYNDROME? SYPHILIS
  12. 12. •SUDDEN Onset of Bilateral, Asymmetric Visual loss associated with scotoma
  13. 13. Acute Posterior Multifocal Placoid Pigment Epitheliopathy (APMPPE) 50% have prodromal illness Symptoms: 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
  14. 14. 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
  15. 15. APMPPE FA Findings Blocks early, Stains Late Blocks early ICGA Findings Hypofluorescent Spots= No. in FA AF Findings HypoAF lesions IMT? NO
  16. 16. APMPPE PROGNOSIS RISK FACTORS FOR VISION LOSS FOVEAL Involvement Older Age Unilateral Disease Longer interval of the initial and fellow eye involvement Recurrence
  17. 17. 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
  18. 18. Serpiginous Choroiditis FA Findings: Blocks early, Stains Late (*plus the CNV findings if Present) ICG Findings: Hypofluorescent lesions throughout FAF : Active lesions are HyperAF; inactive Lesions are HypoAF IMT? YES!!! AAO: FAF is "Exquisitely Sensitive”
  19. 19. FUNDOSCOPY FAF Serpiginous Choroiditis SOURCE: AAO 2013-2015
  20. 20. Serpiginous Choroiditis FUNDOSCOPY FAF SOURCE: AAO 2013-2015
  21. 21. WHITE DOT SYNDROMES Acute Zonal Occult Outer Retinopathy (AZOOR) Multifocal Choroiditis and Panuveitis (MCP) Multiple Evanescent White Dot Syndrome (MEWDS) Acute Annular Outer Retinopathy (AAOR) Punctate Inner Choroidopathy( PIC) Acute Idiopathic Blind Spot Enlargement Syndrome (AIBES) AZOOR-Complex Diseases Acute Posterior Multifocal Placoid Pigment Epitheliopathy (APMPPE) Serpigenous Choroidopathy Birdshot Retinochoidopathy *duh” doesn’t have white dots on them * * * Ampiginous Choroiditis (Aka, Relentless Placoid Chorioretinitis)
  22. 22. • Features of Both APMPPE and Serpiginous • lesions are similar both clinically and angiographically, but the clinical course is atypical for both entities. • Posterior and peripheral lesions simultaneously with macular involvement. Relentless Placoid Chorioretinopathy
  23. 23. New lesions and recurrent attacks are typical, with up to 38% of patients Reaching final VA of 20/200 &CF
  24. 24. 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
  25. 25. Tb-SLC
  26. 26. Ocular Histoplasmosis Syndrome Multiple white atrophic chorioretinal scar (“spots”) Peripapillary Pigment changes CNV Maculopathy NO VITRITIS (vit cells) WHITE DOT?
  27. 27. Young Myopic Female 1/3 Symptoms: Vitritis: CNV: Photopsia, enlarged blind spot, BV Yes Yes, 28% at presentation Multifocal Choroiditis and Panuveitis
  28. 28. FA findings : Blocks Early, stains Late for active lesions ICGA findings: Hypofluorescent Spots are more numerous than FA FAF: Active lesions are HyperAF; Inactive lesions HypoAF IMT helpful? Yes
  29. 29. 1999 2002 PHOTO FAF
  30. 30. RESOLVING VKH
  31. 31. Punctate Inner Choroidopathy (PIC) Young Myopic Female 2/3 Symptoms: Photopsia, Metamophopsia, BV Vitritis CNV YES!; 79% At Presentation
  32. 32. FA Findings: Early Hyperfluorescence, late staining ICGA Findings: Hypofluorescent spont = FA FAF Meh IMT: NO TREATMENT Steroids (P) (S) Anti VEGF Laser Photocoag PDT
  33. 33. SARCOIDOSIS Characteristic Ocular Findings Granulomatous Anterior Uveitis • Vitreous Inflammation (Diffuse Inflammation Snowballs or string of pearls • Periphlebitis • Candlewax drippings (nodular granulomas along venules) • Yellow White Choroidal exudates • CME • Occlusive Retinal Vasculature • ON edema • Retinal, Choroidal, Optic Nerve Granuloma
  34. 34. MULTIPLE EVANESCENT WHITE DOT SYNDROME (MEWDS) Young Myopic Female 3/3 Symptoms: ACUTE UNILATERAL Photopsias, Enlarged blind spot, BV AIBES VITRITIS: Variable CNV Rarely 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
  35. 35. 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 AF IMT Helpful: No …
  36. 36. Shows spots that are more numerous than FA/Examination ICGA FINDINGS: MULTIPLE EVANESCENT WHITE DOT SYNDROME
  37. 37. MEWDS
  38. 38. SYMPATHETIC OPHTHALMIA 1st one to raise his/hand 3 clues CLUE1: NOT a white dot Syndrome CLUE 2 Diffuse non Necrotizing Granulomatous PANUVEITIS CLUE 3 PHOTOPHOBIA 67% Decrease in accomodation (13%) DALEN FUCHS SPOTS
  39. 39. ARN
  40. 40. SLE
  41. 41. 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
  42. 42. Difficult DX.. CALLED OCCULT FOR A REASON Acute zonal occult outer retinopathy (AZOOR) Symptoms: Unilateral Photopsias, Variable Scotomata VITRITIS? Mild CNV? RARELY ERG: Delayed 30 Hz Flicker CR-ATROPHY Lipofufucin laden cells
  43. 43. WHITE DOT SYNDROMES Acute Zonal Occult Outer Retinopathy (AZOOR) Multifocal Choroiditis and Panuveitis (MCP) Multiple Evanescent White Dot Syndrome (MEWDS) Acute Annular Outer Retinopathy (AAOR) Punctate Inner Choroidopathy( PIC) Acute Idiopathic Blind Spot Enlargement Syndrome (AIBES) AZOOR-Complex Diseases Acute Posterior Multifocal Placoid Pigment Epitheliopathy (APMPPE) Serpigenous Choroidopathy Birdshot Retinochoidopathy Ampiginous Choroiditis (Aka, Relentless Placoid Chorioretinitis)
  44. 44. MCP PIC OLDER AGE? SMALLER SPOTS VITRITIS CNV AT PRESENTATION 28% 79% WORSE VA prognosis
  45. 45. APMME SERPIGINOUS VIRAL PRODROME PAPILLITIS VITRITIS CNV AT PRESENTATION NO 25% WORSE VA prognosis 50% NO POSSIBLE NO Mild to Mod Minimal
  46. 46. BIRDSHOT APMPPE SERPIGINOUS MCP PIC MEWDS BLOCKS EARLY STAINS LATE yes yes yes yes no no ICGA SPOTS more Numerous on FA Yes No Yes Yes NO YES IMT yes no yes yes no no
  47. 47. GOOD MORNING

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