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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

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Inflammatory Chorioretinopathies of Unknown Etiology

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  1. Inflammatory Chorioretinopathies of Unknown Etiology white dot syndromes

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

  3. acute posterior multifocal placoid pigment epitheliopathy (APMPPE) • birdshot chorioretinopathy • multiple evanescent white dot syndrome (MEWDS) • multifocal choroiditis with panuveitis (MFC) • serpiginous choroiditis

  4. Discrete, multiple, well-circumscribed yellow- white lesions at the level ofthe retina, outer retina, RPE, choriocapillaris, andchoroid

  5. The etiology of the white dot syndromes is unknown

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

  7. Common presenting symptoms: • Photopsias • Blurredvision • Nyctalopia • Floaters • Visual field loss (blind spotenlargement) • Mild vitritis ( usually)

  8. differential diagnosis : • Syphilis • Diffuse unilateral subacuteneuroretinitis (DUSN) • Tuberculosis • Toxoplasmosis • Pneumocystischoroidopathy • Candidiasis • Acute retinal necrosis (ARN)

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

  10. Morphology • Evolution • Distinct natural histories • Angiographic behavior

  11. a prodromal viral syndrome can be identified

  12. Acute posterior multifocal placoid pigment epitheliopathy(APMPPE)

  13. Healthy young adults • Typically surrounding an influenza-like illness (50%) • Men and women being affected equally • Usually nonrecutrent disease

  14. A sudden onset of bilateral • Asymmetric visual loss associated with central and paracentral scotoma

  15. Minimal anterior segment inflammation • Mild to moderate vitritis

  16. 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

  17. CME is uncommon

  18. The lesions resolve over a period of 2 to 6 weeks • leaving a permanent geographic-shaped alteration in the RPE

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

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

  21. Serpiginous choroidopathy

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

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

  24. Acute lesions are commonly located adjacent to atrophic scars

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

  26. Fluorescein angiography : • Early hypofluorescence of the active lesions • Staining of the active edge of the lesion in the later stage

  27. Systemic immunomodulation has been suggested as first-line therapy because corticosteroids alone are ineffective

  28. Multiple evanescent white dot syndrome (MEWDS)

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

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

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

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

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

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

  35. Birdshot retinochoroidopathy

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

  37. Anterior segment inflammation may be minimal or lacking • Varying degrees of vitritis ( commonly)

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

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

  40. Retinal vasculitis • CME • Optic nerve head inflammation

  41. Fluorescein angiography : • Mild hyperfluorescence and staining in the late phase • Identifying active retinal vasculitis, CME, and optic nerve head leakage

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