chorioretinopathies of unknown etiology (whitedots)
TRANSCRIPT
WHITE DOT SYNDROMES
Leo Francis Pacquing
Resident-in-Training Year3DOH EYE CENTER
Mon, APRIL 27, 2015
WHITE DOT SYNDROME DIFFERENTIAL
• Syphilis
• Diffuse Unilateral Subacute Neuroretinitis
• Ocular Histoplasmosis Syndrome
• Tuberculosis
• Sarcoidosis
• Sympathetic Ophthalmia
• VKH
• Intraocular Lymphoma
• Pneumocystis Choroidopathy
• Candidiasis
WHITE DOT SYNDROMES
• Birdshot
• APMPPE
• Serpiginous Choroiditis
• MCP
• PIC
• SFU
• MEWDS
• ARPE
• AZOOR
WHITE DOT SYNDROMESin 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 SYNDROMESetiology
• Unknown– Infectious– Autoimmune/ Inflammatory
• May be inherited Immune dysregulation that predisposes to Autoimmunity
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-ComplexDiseases
Acute Posterior Multifocal Placoid Pigment Epitheliopathy (APMPPE) Serpigenous Choroidopathy
Birdshot Retinochoidopathy *duh” doesn’t have white dots on them
**
*
Lymphoma-- PIOL
RetinoVitreal Lymphoma;
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
Vitritis Sequelae?
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:
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, Photopsias
Vitritis: Mild to moderate in 50%
CNV: Rare
APMPPE
Fundoscopy: Multiple large flat,yellow-white placoid lesions at the level of RPE
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 FAAF Findings HypoAF lesions
IMT? NO
APMPPE PROGNOSIS
RISK FACTORS FOR VISION LOSS
FOVEAL Involvement
Older Age
Unilateral Disease
Longer interval of the initial and fellow eye involvement
Recurrence
Serpiginous Choroiditis
Asymmetric Gray White lesions at the level of the RPE in aPseudopodial /Geographic manner from the Optic Nerve
Symptoms:
Vitritis?
CNV?
BOV, Scotomas ,
Minimal
25%
Helicoid Choroidopathy
Fundoscopy
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”
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 Evanescent White Dot Syndrome (MEWDS)
Acute Annular Outer Retinopathy (AAOR)
Punctate Inner Choroidopathy( PIC)
Acute Idiopathic Blind Spot Enlargement Syndrome (AIBES)
AZOOR-ComplexDiseases
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)
• 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
New lesions and recurrent attacks are typical, with up to 38% of patientsReaching 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
Tb-SLC
Ocular Histoplasmosis Syndrome
Multiple white atrophic chorioretinal scar (“spots”)
Peripapillary Pigment changes
CNV Maculopathy
NO VITRITIS (vit cells)
WHITE DOT?
Young Myopic Female 1/3
Symptoms:
Vitritis:
CNV:
Photopsia, enlarged blind spot, BV
Yes
Yes, 28% at presentation
Multifocal Choroiditis and Panuveitis
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
1999
2002
PHOTO FAF
RESOLVING VKH
Punctate Inner Choroidopathy (PIC)
Young Myopic Female 2/3
Symptoms: Photopsia, Metamophopsia, BV
Vitritis
CNV YES!; 79% At Presentation
FA Findings: Early Hyperfluorescence, late staining
ICGA Findings: Hypofluorescent spont = FA
FAF Meh
IMT: NO
TREATMENT
Steroids (P) (S)
Anti VEGF
Laser Photocoag
PDT
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
MULTIPLE EVANESCENT WHITE DOT SYNDROME (MEWDS)
Young Myopic Female 3/3
Symptoms: ACUTE UNILATERALPhotopsias, Enlarged blind spot, BV
AIBES
VITRITIS: Variable
CNV Rarely
FUNDOSCOPY: ACUTE PHASEMultiple discrete white to orange Spots (100-200um) at the level of the RPE or Deep retina typicallyIn a PERIFOVEAL location
“EVANESCENT”Because those spots are TRANSITORY and frequently MISSED
GRANULAR PIGMETARY CHANGE
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
…
Shows spots that are more numerous than FA/Examination
ICGA FINDINGS:
MULTIPLE EVANESCENT WHITE DOT SYNDROME
MEWDS
SYMPATHETIC OPHTHALMIA
1st one to raise his/hand3 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
ARN
SLE
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
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
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-ComplexDiseases
Acute Posterior Multifocal Placoid Pigment Epitheliopathy (APMPPE)
Serpigenous Choroidopathy
Birdshot Retinochoidopathy
Ampiginous Choroiditis (Aka, Relentless Placoid Chorioretinitis)
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 to Mod Minimal
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
GOOD MORNING