Inflammatory Chorioretinopathies of Unknown Etiology
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Transcript of Inflammatory Chorioretinopathies of Unknown Etiology
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