Multifocal choroiditis

MULTIFOCAL CHOROIDITIS-NON INFECTIVE UVEITIS

1. MULTIFOCAL CHOROIDITIS DR SHRUTI LADDHA

2. MC-Female Young to middle age small randomly distributed chorioretinal scars Responds to corticosteroids Recurence common CNV develops in 1/3 rd of cases Lesions tends to leave scars

3. CLINICAL FEATURES Photopsia and scotoma Photopsia is present also when there is no clinical evidence of reactivation of the disease May be bilateral –asymmetrical or may be unilateral Anterior granulomatous or sometimes non granulomatous inflammation FUNDUS- atrophic yellow-white foci with pigment spots that sometimes can become adjacent to each other and form a ribbon of pearls. posterior pole or the periphery or both. In the active phases of disease new lesions are not always visible and can be very discreet on FA, whereas ICGA is the most sensitive method to detect new lesions

4. Active stage -Lesions appear much less well defined with blurred borders Multiple yellow foci typically seen in MFC (healed stage)

5. ICGA Scars- hypofluroscent Active –new hypofluroscent patches, sometimes peripapillary hypofluroscence -enlargement of blind spot-regress if therapy started early L eft picture shows quiet stage. Middle picture shows reactivation of MFC 8 months later with a parafoveal CNV. Far right image shows healed stage of choriocapillaritis and cicatricial membrane (arrows) 6 months later after systemic corticosteroid therapy

6. FFA Scars –late staining or masking effect due to pigmentation Active- silent on FFA or late hyperfluroscence If CNV present-classical signs of leakage Left image shows late hyperfluorescence indicating both CNV (parafoveal) and reactivation of MFC. Image on the right taken 6 months after introduction of systemic corticosteroid therapy shows complete healing with only scars (late hyperfluorescence ) with no staining and no CNV activity

7. Early ICGA and FA frames, showing extended Hypofluorescent area on ICGA while FA is practically normal showing only a parafoveal hyperfluorescent spot due to CNV. Intermediate phase of ICGA and FA (about 10 minutes) with more extended and well visible zones of hypofluorescent choriocapillaris nonperfusion on ICGA And parafoveal hyperfluorescent rim due to CNV, while FA shows leakage and staining towards the fovea Very late time frames showing even more clearly ICGA hypofluorescent choriocapillaris nonperfusion (left) together with hyperfluorescent rim due to CNV, while FA shows foveal hyperfluorescentce and strong retinal or subretinal staining coming from the inner retina in response to outer retina ischaemia produced by choriocapillaris nonperfusion

8. OCT All the 3 areas of foci show profound re-modelling of outer retina with a bump (nodular lesion at and above the level of RPE) for all 3 foci. The overlying inner retinal is also disorganised and there is pooling of fluid under the retinal around the 3 foci

9. FAF Increased autofluorescence in those areas that have silent (meaning without FA signs), ICGA hypofluorescent lesions Hypoautofluorescence in the cicatricial areas. After corticosteroid therapy hyperautofluorescence disappears in parallel with resolution of ICGA hypofluorescence . The areas showing hyperautofluorescence go beyond the ICGA hypofluorescent areas indicating that dysfunction of cells and inflammatory involvement go even beyond the areas detected by ICGA .

10. Bright hyperautofluorescence is present all over the fundus (left image) that decreases sustantially after corticosteroid treatment (right)

11. VISUAL FIELD Small scotomas corresponding to chorioretinal scars Active phase-scotomas are larger and correspond to choriocapillaris non perfusion shown on ICGA. Visual field recovery is well correlated with the regression of ICGA hypofluorescent areas.

12. ERG MERG- diffusely depressed MERG recovered partially only in 78% of patients. This might be the explanation why patients continue to be symptomatic and have photopsia despite apparent quiescence of the disease

13. TREATMENT Oral steroid Sub tenons corticosteroid-CME CNVM – generally regress in response to steroid ,if not then additional anti-VEGF

14. PUNCTATE INNER CHOROID S ubset of multifocal choroiditis. Similar characteristics except smaller lesion. More common in young, myopic females. CNVM T/t – steroids ,PST

15. SUBRETINAL FIBROSIS Some patient may show to subretinal fibrosis Suggest subclinical MFC It might also reflect the propensity of the disease to develop subretinal neovascularization that might result in spontaneous fibrosis in some cases.

16. PRESUMED OCULAR HISTOPLASMOSIS SYNDROME (POHS) Histoplasma capsulatum-fungus Missipi , Ohio, Italy, Central America, Turkey, Israel and Australia. Pulmonary inhalation In case of a positive histoplasma skin test and the presence of multifocal choroiditis the diagnosis of POHS has to be considered In non-endemic areas this terminology should not be applied and the diagnosis of POHS should be questioned unless there is a positive skin test

17. The criteria that differentiate POHS from multifocal choroiditis are the absence of anterior chamber reaction, punched-out multifocal lesions also in the periphery ( histospots ) peripapillary scaring asymptomatic course unless neovascular membranes develop. ICGA-does not show hypofluorescent areas seen in MFC in case of active disease but shows pinpoint hyperfluorescent ICGA spots not detected by funduscopy or fluorescein angiography.

18. Serpiginous Choroiditis Bilateral, chronic, progressive, recurrent inflammation of the choriocapillaris, choroid and retinal pigment epithelium, of unknown aetiology. Disease was called geographic choroidopathy and placoid chorioretinitis Rare cause of posterior uveitis (usually< 5%) Affect mostly healthy young to middle-aged adults M>F

19. CLINICAL FEATURES Unilateral Decrease in central vision, Metamorphopsia, scotoma. No inflammation is usually seen in the anterior segment or anterior vitreous. Peripapillary serpentine lesions in the fundus are characteristic Classic peripapillary case of serpiginous choroiditis with focal choriocapillaris and RPE atrophy, RPE clumping at the border of the lesions and fibrosis

20. PERIPAPILLARY GEOGRAPHIC SC 80% begins with patches of grayish or creamy yellow sub-retinal infiltrates originating in the peripapillary area and progressing usually toward the periphery like a serpentine. The overlying retina is usually edematous with rare cases of serous retinal detachments. Resolves in 6-8 weeks l eaving focal choriocapillaris and RPE atrophy. Recurrences usually occur at the edges of previous atrophic scars Late stage- chorioretinal atrophy, subretinal fibrosis, and extensive RPE pigment clumping.

21. MACULAR SC Atypical Begins at macula Worst prognosis –due to early macular involvement and high risk of CNVM

22. AMPIGINOUS CHOROIDITIS Also described as “relentless placoid chorioretinitis” Evolution of acute posterior multifocal placoid pigment epitheliopathy (APMPPE) can mimic the clinical course of SC. New small, isolated round white plaque-like lesions similar to those seen in APMPPE. These isolated lesions coalesced leaving the typical serpiginous atrophic lesions

23. FFA The active lesions, at the borders of the old atrophic lesions, block fluorescein early and show diffuse late staining and leakage of dye. In the atrophic areas, there is early hypofluorescence secondary to atrophy of choriocapillaris and progressive hyperfluorescence at the margins of the lesions phlebitis Active inferior lesions are dark at early phases of fluorescein and become hyperfluorescent at late phase of fluorescein angiogram. It is associated with a perivascular inflammatory leakage of the infero -temporal vein. A diffuse late staining of the underlying sclera can also be observed in the superior inactive lesions

24. ICGA Best to evaluate extend and activity of disease and its follow-up H ypofluorescent areas beginning from the early to the late phase which supposed to correspond to choriocapillaris non-perfusion or delayed filling and corresponding to a primary inflammatory choriocapillaropathy . Undetected lesions with the fluorescein angiography can be apparent on ICGA.

25. OCT CME Retinal spot atrophy-diffuse thinning of retina Loss of photoreceptors and RPE

26. VISUAL FIELDS Scotoma corresponding to geographic lesions

27. PATHOGENESIS Inflammation HLA B7 or C3 decreased blood level May be infectious-TB,HZV V asculopathy either primary or secondary to systemic disease and characteristic angiographic findings of choriocapillaris non-perfusion,

28. COMPLICATIONS CNVM Branch retinal vein occlusion, Periphlebitis Pigment epithelium detachment Serous retinal detachment Cystoid macular oedema Optic disk neovascularisation, Serous detachments Subretinal fibrosis.

29. TREATMENT Fundus photo and angiography may be necessary to decide the treatment Steroids and immunosuppressive therapy