Choroiditis

Choroiditis

1. Dr. Samarth Mishra CHOROIDITIS

2. INTRODUCTION Inflammation of choroid; associated with the highest risk of severe vision loss. (Standardization of Uveitis Nomenclature (SUN) Working Group) Always Involving retina, Retinal vessels, optic nerve head.

3. CLASSIFICATION ANATOMICAL – Choroiditis Chorioretinitis Retinochoroiditis Neuro -uveitis AETIOLOGICAL – infective/non-infective

4. INFECTIOUS 1. Parasitic – Toxoplasmosis – Toxocariasis – Onchocerciasis – Cysticercosis 2. Bacterial – - tuberculosis - syphilis 3 . Viral – Herpes viruses • ARN • CMV retinitis Epstein-Barr virus – Rubella – Rubeola (measles) – West Nile virus

5. 3. Fungal – Candidiasis – Aspergillosis – Cryptococcosis – Coccidioidomycosis

6. NON-INFECTIOUS CAUSE Multifocal Choroiditis and Panuveitis Punctate Inner Choroidopathy Subretinal Fibrosis and Uveitis Serpiginous choroidopathy Acute retinal pigment epitheliitis Birdshot choroidopathy 􀁺 Retinal Vasculitis – Behcets – SLE – Wegeners granulomatosis – PAN – Eales disease – Frosted-branch angiitis

7. SYMPTOMS Floaters Impaired central vision ( pain or painless ) Pain, redness & photophobia if associated with ant. Segment involvement Metamorphopsia , micro/ macropsia Perception of black spot

8. SIGNS – Inflammatory cells & vitritis Exudates, Edema & infiltrations in retina / choroid Sheathing of vessels Other signs – Disc edema Retinal haemorrhages Spill-over uveitis Complicated cataract Glaucoma RD Choroid neovascularisation

9. CHOROIDITIS Focal / multifocal /diffuse/central/ juxtapapillary Granulomatous or non-granulomatous/ exudative choroiditis Ophthalmoscopic picture – 1 . Active lesion – early stage - yellowish area with hazy edges & ill defined margin due to infiltration & exudation , lie deeper to retinal vessels Late stage – bruch’s membrane destroyed – infiltration of leukocytes to retina & vitreous ↓ organisation of exudation due to fibroblastic activity of stroma ↓ Firm fusion of retina & choroid due to destruction of normal structure by fibrous tissue

10. Old choroiditis lesion – White c olour lesion due to fibrous tissue deposition, thinning & atrophy – white reflex from sclera Surrounded by black zone of pigment from RPE RETINITIS - Focal /multifocal / geographic /diffuse Active lesions – whitis retinal opacities with indistinct boarder due to surronding edema Later on boarder become well defined VASCULITIS – Periphlebitis > periarteritis Active vasculitis – yellowish/grey-white, patchy perivascular sheathing, with haemorrhage

11. TOXOPLASMOSIS Most common cause of choroiditis in immuno competent patient. Intraocular infection is often accompanied by CNS involvement in immuno compromised patient. Caused by toxoplasma gondii . Infest >10% of adults in northern temperate countries & > 50% of adults in mediterranean & tropical countries .

12. Three forms of the parasite: - tachyzoite ( trophozoite ) – invassive form responsible for acute infection, - bradyzoite ( tissue cyst) – latent or recurent infection - sporozoite ( oocyst ). MODE OF INFECTION Ingestion of undercooked meat containing bradyzoites . Ingestion of oocyst from contaminated hand, food or water. Transplacental – 40% of fetus is affected if mother is infected during pregnancy.

13. PATHOGENESIS Clinically, the infestation starts as a focal area of retinitis, with an overlying vitritis . Atypical, severe toxoplasmic retinochoroiditis in the elderly can mimic ARN. zonal granuloma with intense central necrosis surrounded by successive layers of mononuclear cells - ↑ ed Plasma cells at periphery → secrete antibodies → destruction of the free parasites in the extracellular space → cyst formation by parasites Proliferation of the RPE cells, and healing of the lesion is associated with scar formation

14. HISTOLOGY Found in three forms: free, pseudocysts , or in true cysts. a. Rarely, the protozoa may be found in a free form in the neural retina. b. Multiplies in the confines of the cell membrane → a group of protozoa surrounded by the retinal cell membrane → pseudocyst C. If environment becomes inhospitable, an Intracellular protozoan ( trophozoite ) may transform into bradyzoite , surround itself with a self-made membrane, multiply, and then form a true cyst.

15. CLINICAL MANIFESTATION OF TOXOPLASMOSIS The most frequent form of infection with T. gondii is subclinical, and is discovered by serologic testing for antibodies to Toxoplasma organisms. clinical entities of toxoplasmosis: - congenital toxoplasmosis acquired systemic toxoplasmosis toxoplasmosis in the immunocompromised host acquired or reactivation of a latent infection

16. CONGENITAL TOXOPLASMOSIS Results from transplacental transmission of T. gondii . Incidence of congenital infection varies with the trimester during which the mother becomes infected. lowest incidence occurs in the first trimester (15% to 20%), and highest incidence in the third trimester (59%). If infection occurs in – 1 st trimester → spontaneous abortion - 3 rd trimester → subclinical infection

17. COURSE OF DISEASE:- Healing of the retinitis is associated with a decrease in retinal edema and flattening of the lesion with evidence of scar formation surrounded by variable amounts of pigment . lesion may appear as a punched out scar with underlying sclera resulting from extensive retinal and choroidal necrosis surrounded by pigment proliferation , it may become a conglomerate or proliferated retinal pigment cells, or it may be small and appear as a pigment clump in the retina.

18. OCULAR MANIFESTATION Ocular findings include involvement of the retina, choroid, retinal vessels, macula, optic nerve, vitreous, and anterior uvea. TYPICAL – -Focus of retinitis surrounded by fuzzy retinal edema -Pigmented atrophic retinochoroiditic scar -Vitreous cells and exudates -Focal retinal vasculitis - Hyperemia of optic nerve head -Cells and flare in the anterior chamber

19. Atypical Manifestations:- Juxtapapillar retinitis Retrobulbar neuritis Rhegmatogenous RD Pars planitis Punctate outer retinitis Serous macular detachment BRAO/BRVO Retinal or subretinal neovascularization Choroidoretinal vascular anastomosis Panuveitis

20. Toxoplasmic retinitis – focus of necrotising retinitis surrounded by retinal edema, retinal vasculitis . - Solitary inflammatory focus near an old pigmented scar. ( satelite lesion ) - Severe vitritis impairing visualisation of fundus → HEADLIGHT IN FOG appearance. -In immunocompromised – multifocal retinal lesion often B/L, less vitritis - simulate ARN . There may be sec. nongranulomatous inflammation of choroid & sclera. When choroid is involved called as retinochoroiditis .

21. Occurs in 3 morphological variants :- In most severe disease – lesion of > 1 DD, dense & elevated lesion, & are largely destructive. - assosiated with severe vitritis & ant. Chamber reaction. Prompt therapy is needed regardless of site of lesion. 2. 2 nd variant :- punctate lesion of inner retina, mild inflammation, no therapy needed unless the lesion is close to macula. 3. 3 rd variant :- punctate lesion in outer retina with mild vitritis , spontaneous resolution

22. OPTIC NERVE optic neuritis or papillitis associated with edema , Later Juxtapapillary Retinochoroiditis and Macular Star develop. VITREOUS -Posterior vitreous detachment common -precipitates of inflammatory cells on the posterior vitreous face ANTERIOR UVEA - Anterior uveitis (granulomatous or nongranulomatous ) may be associated with Toxoplasma retinochoroiditis

23. COMPLICATIONS Posterior synaechiae Macular edema Dragging of macula RD Choroidal neovascularisation BRAO/BRVO Optic atrophy Cataract Glaucoma u/l pigmentary retinopathy

24. DIAGNOSIS Classic fundus finding Serological testing – Sabin-Feldman dye test, ELISA, IFA test , IHA test, agglutination test . PCR in vitreous sample Isolation of organism from aquous , vitreous CNS imaging Fluorescein angiographic - presence a dark hypofluorescent center of the lesion surrounded by an area of hyperfluorescence . ICG OCT, USG

25. TREATMENT Pyrimethamine : Loading dose: 100 mg (1st day), followed by 25 mg once daily + Sulfadiazine: 4 Gr daily divided in every 6 hours For 4 to 6 weeks . Oral corticosteroids must be initiated at least 24 hours after starting anti parasitic drugs. Folinic acid also given Other drugs used in various combinations include: Clindamycin, Trimethoprim + Sulphamethoxazol ( Co- Trimoxazole ), Spiramycin , Azithromycin Therapy regimens used during Pregnancy: Spiramycin - 2 gr/day in two divided doses Standard regimen for newborns- Pyrimethamine + Sulfadiazine + Folinic acid

26. SURGICAL T/t – - Pars Plana Vitrectomy : to remove Vitreous Opacities, or to relieve the persistent Vitreo -Retinal traction . Scleral Buckling: in cases complicated with Retinal Detachment. PHOTOCOAGULATION AND CRYOTHERAPY - Both photocoagulation and cryotherapy cause destruction of the Toxoplasma cyst and the tachyzoites in the retina

27. TOXOCARIASIS Toxocara canis : nematode Dog primary host Children who have pica, close contact with Puppies. Unilateral, Male > female, Children , young adults DDx of leukocoria (r/o RB) 􀁺 Ova ingested – Visceral larva migrans – Larvae encyst in tissues – Never mature in humans – no ova in stool

28. CLINICAL FEATURES Granuloma in the Peripheral Retina and Vitreous. Posterior Pole Granuloma . Chronic Endophthalmitis .

29. DIAGNOSIS Anti- Toxocara antibodies – Any serum titer significant – Higher titer in aqueous Eosinophils in aqueous/vitreous TREATMENT Medical treatment is directed toward the inflammatory response that produces Structural Damage and decreased vision - with Topical or Systemic Steroids. Antihelminthic therapy for Ocular Toxocariasis do not alter natural course of the disease

30. Cysticercosis - South/Central America, Africa , Asia Larva of Taenia solium Violent uveitis as endophthalmitis Treatment : vitrectomy for subretinal cysticercus Onchocerciasis River blindness” - blackflies Microfilariae in cornea, aqueous; skin nodules Attenuated vessels, perivascular sheathing, RPE atrophy; optic atrophy late Ivermectin 150 mic /kg q year x 10 years

31. TUBERCULOUS UVEITIS The most common presentation is disseminated choroiditis . Deep in the choroid, appear yellow, white, or gray , and are fairly well circumscribed. Vast majority of cases, the lesions present in the posterior pole. single tubercle, focal choroiditis,which can occur at the posterior pole, typically elevated and may be accompanied by an overlying serous retinal detachment Subretinal abscess is formed progressively from a choroidal tubercle, which can be single or multiple. Periphlebitis often b/l

32. SYPHILITIC UVEITIS present as chorioretinitis , neuroretinitis , salt-pepper retinopathy, optic neuritis, papilloedema , and optic perineuritis . Syphilis can present with placoid choroidal lesions. Unusual manifestation of syphilis is acute necrotizing retinopathy, which mimics ARN. In HIV-positive patients, ocular syphilis is more closely associated with neurological abnormalities

33. Rubella Congenital – Ocular involvement increased if contracted during 1st trimester – Cataract - retention of cell nuclei in embryonic nucleus – Chronic nongranulomatous iritis ; iris atrophy - “ Salt-and-pepper” fundus – vision, ERG unaffected – Choroidal neovascularization rare complication Acquired – German measles – Conjunctivitis, keratitis, iritis , bilateral retinitis with exudative detachment

34. Measles ( Rubeola ) 􀁺 Congenital – “Salt-and-pepper” fundus 􀁺 Acquired – Macular edema , neuroretinitis , attenuated vessels 􀁺 Subacute sclerosing panencephalitis (SSPE) – Slow virus - mutation of measles virus – Encephalitis with progressive deficits – Disc edema , optic atrophy, macular - infiltrate/ hemorrhage /gliosis – Supportive treatment; poor prognosis

35. West Nile Virus Creamy target like chorioretinal lesions , 300 – 1000 μ m, scattered diffusely Hypofluorescent centrally and hyperfluorescent edges Multifocal chorioretinitis – Vitritis , iridocyclitis – Occlusive retinal vasculitis - multiple branch artery occlusions – Optic nerve: optic neuritis, mild disc edema 􀁺 Congenital - chorioretinal scarring

36. FUNGAL UVEITIS

37. Ocular Histoplasmosis / presumed occular histoplasmosis syndrome Histoplasma capsulatum 20 – 50 yr , HLA-B7 – associated with macular lesions Punched-out CR scars (“ histo spots”) Peripapillary atrophy, Macular scar, No vitreous cells FA - – Active choroiditis : early hypofluorescence with late staining – CNV: early hyperfluorescence with late leakage

38. Treatment - – Extrafoveal CNV: photocoagulation - Juxtafoveal CNV: photocoagulation – Risk of CNV in fellow eye with histo spot in macula : 25% @ 3 years SF CNV- Photodynamic therapy / Steroids/Anti-VEGF/ Combination Subfoveal surgery- benefit for POHS if < 20/100

39. Candidiasis Immunosuppressed patients , indwelling catheters, hyperalimentation Incidence of endophthalmitis in candidemia – No antifungal treatment – 10 – 37% – On antifungal treatment – 3 % Choroidal infection with secondary retinal, vitreous involvement – fluffy yellow lesions Treatment – IV, periocular , intravitreal antifungals (amphotericin B, ketoconazole) – Consider vitrectomy if significant vitritis

40. NON INFECTIOUS CAUSE MULTIFOCAL CHOROIDITIS & PANUVEITIS ( MCP ) 􀁺 Female > male 􀁺 “Pseudo-POHS” – histo spots 􀁺 Vitritis , +/- anterior uveitis 􀁺 Macular CNV in 1/3 􀁺 FA: Early hyperfluorescence with late staining 􀁺 Diagnosis of exclusion- TB, syphilis , sarcoidosis , 􀁺 Treatment – Steroids – Local/ Systemic – Other immunosuppressives

41. Punctate Inner Choroidopathy (PIC ) Subgroup of MCP Young, female, myopic No inflammation Multiple small white spots with fuzzy boarder at Inner choroid & retina - 40 % dev. CNV Subretinal fibrosis and uveitis - Young , female, African-American, bilateral - Chronic vitritis , CME - Gliotic yellow-white subretinal lesions, gradually coalesce Poor prognosis Corticosteroids (esp for CME) – other immunosuppressives

42. Serpiginous Choroidopathy (geographic, helicoid) Adults ; usually bilateral, Yellow- gray lesions Start from optic nerve, progress centrifugally Active lesions adjacent to scarred inactive area Mild vitritis , NVD, CNV FA – Early in disease – like AMPPE – Later in disease – window defects Steroids , other immunosuppressives Poor prognosis

43. Birdshot Retinochoroidopathy ( BSRC) Vitiliginous choroiditis Females > males, 30 – 70 years old Symptoms : Painless Visual Loss Floaters , Photophobia, Nyctalopia , and Disturbances in Color Vision . Scattered round or oval cream-colored spots, May become Confluent resulting in larger Geographic areas of Hypopigmentation. CME Vitritis Disc edema Arteriolar narrowing

44. FA – Retinal lesions often silent – CME, periphlebitis Diagnostic – HLA-A29 - 50-80 % birdshot ERG may be reduced Treatment – Corticosteroids – may help in ~ 50% – immunosuppressives - Mycophenolate , Cyclosporine • Quiescent 2 yrs then slow taper

45. CONCLUSION Choroiditis entities have very characteristic clinical features and diagnosis is mainly clinical. Essential to differentiate infective and non-infective conditions as their management is diametrically opposite. Empirical use of systemic steroids or immunosuppressives in all cases of Choroiditis should be absolutely avoided. Follow-up all patients with Choroiditis even after the resolution of lesions for complications related to the disease .

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