IDIOPATHIC CHORIORETINOPATHIES. PRESENTATION

OPHTHALMOLOGY

1. IDIOPATHIC CHORIORETINOPATHIES Dr . SANA TAKREEM KHAN

2. WHITE DOT SYNDROME They are group of posterior uveitis disorder of unknown cause and share a characteristic appearance of multiple whitish- yellow inflammatory lesions located at the level of outer retina,retinal pigment epithelium and choroid. The WDS are inflammatory in mechanism and predominantly non-infectious. Some patients often describe a viral prodrome,but the WDS have also been linked to variety of other inciting factors including-bacterial infections, vaccinations,and certain haplotypes .

3. Patients typically present with blurred vision, scotomas , photopsias and floaters. Incidence- 0.45 in 100,000 per year

4. WHITE DOT SYNDROME Multiple evanescent white dot syndrome Acute posterior multifocal placoid pigment epitheliopathy Birdshot chorioretinopathy Serpiginous choroidopathy Punctate inner choroidopathy Multifocal choroiditis and panuveitis Subretinal fibrosis and uveitis

5. MULTIPLE EVANESCENT WHITE DOT SYNDROME Clinical Presentation Young to middle-aged female Acute, painless, U/L LOV Patient may notice photopsias and scotomas , particularly in temporal visual field.

6. Pathophysiology of MEWDS Hypoperfusion of the choriocapillaries leading to ischemic damage to overlying tissue. Immune damage of RPE causing photoreceptor decline. Immune attack targeting photoreceptors themselves

7. Fundus in MEWDS Fundus reveals flat,multifocal,grey -white lesions that appear to extend as deep as RPE layer. Lesions are found outside the macula in the posterior pole (100-300 micrometer in dia ) Charecteristic finding- Foveal granularity.

8. FA

9. ICGA OF MEWDS Multiple hypofluorescent spots in the posterior pole and hypofluorescence around the optic nerve head, particularly in patients with enlarged blind spots.

10. OCT IN MEWDS Changes in outer retina. Loss of retinal photoreceptors inner and outer segment which can return to a more normal appearance after the acute episode. Disruption of ellipsoid zone hyperreflective material in RPE, ellipsoid zone and outer nuclear layer

11. Differential diagnosis APMPPE Birdshot retinochoroidopathy Acute retinal pigment epithelitis Diffuse unilateral subacute neuroretinitis

12. Management of MEWDS Self limited disease with almost all patients regaining good visual acuity within 3-9 weeks. The lesion disappear without scarring, and photopsias and scotoma gradually resolve. Occasionally patients may have persistent blind spot enlargement. Rare Complication: Choroidal neovascularization (require treatment with laser photocoagulation or intravitreal bevacizumab )

13. Acute Posterior Multifocal Placoid Pigment Epitheliopathy Clinical Presentation Young adults B/L, acute, painless LOV One third of patients- symptoms of fever, myalgia,headache,and malaise are noted prior to the onset of ocular symptoms.

14. APMPPE Pathogenesis Infectious primer: viruses, TB, vaccination Linked to HLA-DR2, HLA-B7, suggesting genetic predisposition to disease. Immune condition linked to APMPPE include psoriasis, sarcoidosis , erythema nodosum , granulomatosis with polyangitis , polyarteritis nodosa and diabetes. APMPPE with concomitant cerebral vasculitis has also been described. The most recent literature identifies the choriocapillaries as the primary site of immune attack, which results in destruction of the overlying photoreceptors.

15. Fundus Fundus reveals vitreous cells and flat yellow-white placoid lesions in the posterior pole, ranging in size from 0.5 to several disc diameters.

16. FA Early hypofluorescence and late hyperfluorescence of active lesions

17. Differential Diagnosis Sarcoidosis MEWDS Birdshot retinochoroidopathy

18. MANAGEMENT The disease is self-limited with spontaneous recovery of vision in most cases. In rare cases, recurrences may occur within 6 months. In cases of foveal involvement, corticosteroids maybe considered. Corticosteroids may be beneficial in cases of associated cerebral vasculitis .

19. Serpiginous Choroiditis Clinical Presentation Also known as geographic choroidopathy . Affects middle-aged males. Patients presents with U\L or B\L visual loss when macula is involved, and they also notice photopsias and scotomata .

20. Fundus Geographic yellow to yellow-grey choroiditis that extends from optic nerve. Active lesions usually are found at the border of inactive lesions and appear in an interlocking polygonal pattern that spreads out towards the periphery from the optic nerve. Macular involvement is common

21. FA Hypofluorescence is present in the center of the lesion, and hyperfluorescence at rim of the lesions in early phase. Active lesions are hyperfluorescent in late phase. Inactive lesions are hypofluorescent in early phase and staining of sclera is visible in late phase.

22. Differential Diagnosis APMPPE MCP ARMD Sarcoidosis

23. Management Within a few weeks, active lesion convert into inactive lesion with eventual retinal pigment epithelial atrophy. If foveal involvement is absent, visual prognosis is good and no treatment is necessary. If fovea is involved, treatment with anti- inflmmatory medication is recommended. A study involving long-term treatment with Prednisolone,Cyclosporine and Azathioprine demonstrated a possible benefit. Recurrence are common.

24. Birdshot Retinochoroidopathy

25. Birdshot Retinochoroidopathy Also known as vitiliginous choroiditis . Clinical Presentation Typically affects females in fourth to fifth decade of life. It is chronic, B/L, manifest with severe retinitis and stromal choroiditis . Painless, insidious impairment of central vision, floaters, loss of colour vision. >90% of patients are HLA-A29 positive

26. Fundus Multiple depigmented yellow-white patches scattered throughout the fundus ,similar to shotgun scatter of a birdshot.

27. FA FA- extensive vascular leakage, with disc and vessel staining and can show diffuse macular oedema . Fovea is often spared.

28. ICGA ICGA- lesions are more numerous. They are hypoflourescent during early and intermediate phases and isofluorescent later.

29. OCT OCT- will confirm macular edema, photoreceptor and ellipsoid zone disruption and choroidal thinning.

30. Management Sub- Tenon triamcinolone acetonide (40mg), in patients with monocular disease. Upto 90% patients need systemic treatment.Referral to uveitis specialist is considered. Systemic steroids are used initially. But immunomodulatory treatment is often needed. ( mycophenolate followed by infliximab ).

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