chorioretinopathies of unknown etiology (whitedots)

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

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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

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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