Inflammatory Chorioretinopathies of Unknown

Etiology

white dot syndromes

• a group of idiopathic multifocal inflammatory conditions involving the retina and the choroid

• acute posterior multifocal placoid pigment epitheliopathy (APMPPE)

• birdshot chorioretinopathy• multiple evanescent white dot

syndrome (MEWDS)• multifocal choroiditis with panuveitis

(MFC)• serpiginous choroiditis

• Discrete, multiple, well-circumscribed yellow- white lesions at the level of the retina, outer retina, RPE, choriocapillaris, and choroid

• The etiology of the white dot syndromes is unknown

• Bilateral involvement ( MEWDS)• younger than 50 years of age(birdshot retinochoroidopathy and serpiginous)

Common presenting symptoms:

• Photopsias• Blurred vision• Nyctalopia• Floaters• Visual field loss (blind spot enlargement )• Mild vitritis ( usually)

differential diagnosis :

• Syphilis• Diffuse unilateral subacute

neuroretinitis (DUSN)• Tuberculosis• Toxoplasmosis• Pneumocystis choroidopathy• Candidiasis• Acute retinal necrosis (ARN)

• Ocular histoplasmosis syndrome (OHS)• Sarcoidosis• Sympathetic ophthalmia• VKH syndrome• Intraocular lymphoma

• Morphology • Evolution• Distinct natural histories• Angiographic behavior

• a prodromal viral syndrome can be identified

Acute posterior multifocal placoid pigment epitheliopathy

(APMPPE)

• Healthy young adults• Typically surrounding an influenza-like

illness (50%)• Men and women being affected equally• Usually nonrecutrent disease

• A sudden onset of bilateral• Asymmetric visual loss associated

with central and paracentral scotoma

• Minimal anterior segment inflammation • Mild to moderate vitritis

Funduscopic findings:• multiple, large, flat, yellow-white

placoid lesions at the level of the RPE, varying in size from 1 to 2 disc areas, located throughout the posterior pole to the equator

• CME is uncommon

• The lesions resolve over a period of 2 to 6 weeks

• leaving a permanent geographic-shaped alteration in the RPE

• The diagnosis of APMPPE is based on the characteristic clinical presentation and characteristic FA findings during the acute phase of the disease

fluorescein angiography:• Early hypofluorescenc• Staining in the late phase

Serpiginous choroidopathy

• Uncommon• Chronic, progressive inflammatory • Adult men and women equally • Second to sixth decades of age life• Minimal vitreous involvement• A quiet anterior chamber

• Gray-white lesions at the level of the RPE projecting in a pseudopodial or geographic manner from the optic nerve in the posterior fundus

• Acute lesions are commonly located adjacent to atrophic scars

• The disease course is marked by progressive centrifugal extension, with marked asymmetry between the 2 eyes

Fluorescein angiography :• Early hypofluorescence of the active lesions• Staining of the active edge of the lesion

in the later stage

• Systemic immunomodulation has been suggested as first-line therapy because

corticosteroids alone are ineffective

Multiple evanescent white dot syndrome

(MEWDS)

• Unilateral (80%) • Central or peripheral scotoma• Healthy young (10-47 years)• Moderately myopic females (90%)• Frequently surrounding a flulike

prodrome

• multiple, discrete white orangish spots(100-200 μm) at the level of the RPE or deep retina, typically in a perifoveal location

• These spots are transitory and are frequently missed; they leave instead a granular macular pigmentary change

• Few associated vitreous cells• Mild blurring of the optic disc

• Punctate hyperfluorescent lesions in a wreathlike configuration surrounding the fovea that stain late

• The prognosis is excellent, and vision is completely recovered in 2-10 weeks without treatment

Birdshot retinochoroidopathy

• Females (common)• The fourth decade of life• HLA-A29 (80%-98%)

• Anterior segment inflammation may be minimal or lacking

• Varying degrees of vitritis ( commonly)

• Multifocal,hypopigmented, ovoid, cream-colored lesions (50-1500 μm) at the level of the choroid and RPE in the postequatorial fundus

• These lesions radiate from the optic nerve and follow the larger choroidal vessels

• Retinal vasculitis• CME• Optic nerve head inflammation

Fluorescein angiography :• Mild hyperfluorescence and staining in

the late phase

• Identifying active retinal vasculitis, CME, and optic nerve head leakage

• The course is generally marked by multiple exacerbarions and remissions

Treatment:

• Systemic corticosteroids• Corticosteroid-spadng immunomodulatory

agents• Periocular corticosteroid injections

Conclusion:

• Because of the significant overlap among them, the various white dot syndromes may just represent a spectrum of the same disease