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White dot syndromes
- 1. White dot syndromes Dr.Nikhil. R.P
- 2. • Introduction • Birdshot retinopathy(BCR) • Multifocal choroiditis and panuveitis(MCP) • Punctate inner choroidopathy (PIC) • Subretinal fibrosis and uveitis syndrome(SFU) • Multiple evanesecent white dot syndrome (MEWDS) • Acute posterior multifocal placoid pigment epitheliopahty(APMPPE) • Serpiginous choroidopathy
- 3. Introduction • The whitespot syndromes (WSS) are a group of diseases characterized by inflammation and dysfunction of the outer retina, retinal pigment epithelium, choroid, or a combination of these. • The WSS each have distinct features, but do share some characteristics. Blurred vision, photopsias, visual field changes, floaters and changes in contrast sensitivity can occur • Unilateral or bilateral . Etiology of WDS is unknown. Both Infectious cause & autoimmune etiology have been hypothesized.
- 4. • WDS occursin families with inherited immune dysregulation that predisposes to autoimmunity triggered by some exogenous agent • The age of onset is generally greater than 50, but can range from the second to the sixth decade of life.
- 5. Bird shot retinopathy •The term birdshot retinochoroidopathy was first used in 1980 by Ryan and Maumenee • It is a bilateral, chronic process with vitritis, retinal vasculitis, and cystoid macular edema • Female prepondance with mean age 53 yrs • HLA-A29 is strongly associated with BCR • Inflammation appears to be a primary feature
- 6. • Symptoms -blurred vision, floaters, photopsias, Severe nyctalopia despite normal visual acuity may also be a presenting symptom • Fundus - These birdshot lesions can be oval or round in shape, typically about one-half or one-quarter disc diameter in size. Multiple, small, cream-colored, choroidal patches scattered around the optic disc and radiate to the equator in a “shotgun” pattern • They tend to cluster near the optic nerve and most commonly nasal and inferior to the disc • They may appear to follow choroidal blood vessels peripherally.
- 7. • Other findings- retinal vasculitis, optic disc edema, CME (in about 50% of patients) and epiretinal membrane (ERM) formation • Diffuse narrowing of the retinal arterioles, perivascular nerve fibre layer haemorrhages, and tortuosity of retinal vessels. Choroidal neovascularization (CNV) can occur • Inactive lesions consist of well delineated atrophic spots • Histopathology- These suggest that the spots may be related to accumulation of lymphocytes in the choroid at multiple levels, occasionally associated with hemorrhage. • Some of the foci were adjacent to the choroidal vessels
- 8. • FFA -The lesions can hypofluorescence in the early phase, and there can be diffuse hyperfluorescence in the late phases • ICGA- the birdshot lesions appear hypofluorescent during the intermediate phase of angiography and appear to be bordered by medium-to-large vessels. • OCT - They found that there is significant thinning of the retina and choroid in the peripheral locations. Macular edema is confirmed • Differential diagnosis – TB, sarcoidosis, presumed ocular histoplasmosis(POHS), toxoplasmosis, syphilis
- 9. • Tuberculosis- focalelevated dome shaped choroidal granulomas • Sarcoidosis- multiple small, pale yellow punched out choroidal lesions • Presumed ocular histoplasmosis- multiple white atrophic chorioretinal histo spots(200microns) • Syphilis – multifocal chorioretinitis, large pale yellow subretinal lesions ( acute syphilitic posterior placoid choroidopathy) • Toxoplasmosis – clusters of grey- white lesions(25-75microns), satellite lesions near old scar
- 11. Rx- Corticosteroids have beenthe mainstay of treatment. Oral, sub-Tenon, intraocular, and most recently sustained release fluocinolone acetonide have been used • Immunosuppressives like – cyclosporine, azathioprine, methotrexate have been used as monotherapy or as an adjunctive • Anti- VEGF’s therapy for choroidal neovascularization and CME patients are given
- 12. Multifocal choroiditis andpanuveitis • Multifocal choroiditis and panuveitis (MCP) is an idiopathic inflammatory disorder of unknown etiology affecting the choroid & retina. • It is most often seen in myopic women between second and sixth decade of life presenting with photopsia, decreased vision, floaters, photophobia • Usually bilateral but may be asymmetric • The etiology is not known but may be due to sensitization of antigens within photoreceptors & retinal pigment epithelial cells by an exogenous pathogen ( Epstain barr virus or HSV )
- 13. • Signs- anterioruveitis, vitritis, multifocal choroiditis. The choroiditis lesions are 50-200 microm in diameter, yellow-white at the level of RPE or inner choroid and have punched out appearance with pigmentation of the edges • Seen throughout the fundus , mainly posterior to the equator. It is associated with CME, deep choroidal neovascular membrane, ERM . subretinal fibrosis can develop • Inactive lesions – sharply defined margins and pigmented borders • Optic disc edema and enlargement of blind spot may be present • Differential diagnosis- TB, sarcoidosis, syphilis, APMPPE, BCR, POHS, VKH
- 14. • FFA -FA in the acute stage show early hypofluorescence & late staining on Fluorescein angiography • ICGA shows hypofluorescent lesions suggestive of active choroiditis commonly clustered around disc far more numerous than those seen on flurorescein angiography or clinical exam. • HVF- may show large defects not corresponding to with examination findings Rx- • topical, systemic steroids. In chronic cases immunosuppressive agents can be considered • CNV can be managed with anti-VEGF therapy with or without the use of corticosteroids.
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- 17. Punctate inner choroidopathy •Young myopic females are affected. • bilateral involvement • It has similarities wit MCP but involvement is predominantly macular • Symptoms – blurring of vision, floaters, photopsia • Signs- anterior uveitis or vitritis is mild , lesions are bilateral, multiple, small, welldefined, yellow-white, usually 100-200 microns diameter and are limited to the posterior pole
- 18. • Serous retinaldetachment may occur overlying an active lesion • These lesions advance forming punched out atrophic scars leaving depigmented halo. Choroidal neovascular membranes occur in between 40 to 75% of patients from healed scars. Recurrences are common • FA – hyperfluorescence and late staining of lesions • ICG shows numerous hypofluorescent spots in the middle and late phases • Differential diagnosis – Sarcoidosis, TB, syphilis, APMPPE, BCR, POHS Rx- • Systemic Corticosteroids are used in the active stages. CNV can be managed with anti-VEGF injections in combination with oral steriods.
- 19. (C) FA arterialphase (D) FA late phase of eye
- 20. Sub-Retinal Fibrosis andUveitis Syndrome • is a rare form of panuveitis of unknown etiology affecting otherwise healthy myopic women between the ages of 14 and 34 years • Symptoms – blurring of vision in one eye the later both eyes, • Signs- mild to moderate vitritis, with whiteyellow lesions (50-500 μm) located in the posterior pole to midperiphery at the level of the RPE. • these lesions are accompanied by the appearance of turbid subretinal exudation & this differentiates SFU from others like MFC & PIC
- 21. • Over thenext several months to years, the subretinal fibrin and turbid exudates coalesce into large, white, stellate zones of subretinal fibrosis to involve most of the retina and choroid. • Serous neurosensory retinal detachment, CME, and CNV may also be observed • On FFA, the acute lesions show early hyperfluorescence followed by late leakage. • The disease course is marked by chronic recurrent inflammation and the visual prognosis is guarded • Treatment is directed towards early diagnosis and aggressive management to prevent fibrosis setting in the other eye. • Once severe subretinal fibrosis develops, treatment has little benefit
- 23. Multiple evanescent whitedot syndrome • It is an uncommon idiopathic disease. Etiology may be infective or inflammatory • Young adult females , 25-50% among them having a preceding viral like illness • Symptoms- Unilateral blurring of vision, photopsia, floaters, dyschromatopsia • Signs- vitritis (mild) with disc edema may be present , multiple small, discrete, perifoveal white to orange spots (100-200 microm) at the level of the RPE or deep retina.
- 24. • Recovery occursover weeks, often leaving residual signs (granular macular pigmentary change which is pathognomonic) • Differential diagnosis – BCR, APMPPE, sarcoidosis • FFA- early hyperfluorescene of the dots with late staining • ICGA- hypofluorescent spots that are often more than FFA • Visual field defects are variable and range from generalized depression, paracentral or temporal scotoma to enlargement of the blind spot • ERG- decreased a-wave amplitude
- 25. • The prognosisis excellent with visual recovery in 2-10 weeks without treatment. However, residual symptoms including photopsias and enlargement of the blind spot may persist for months. • Recurrences are uncommon (10-15 % of patients) and have a similarly good prognosis. • Treatment is not needed in view of the favourable natural course.
- 28. Acute posterior multiocalplacoid pigment epitheliopathy(APMPPE) • It is an uncommon idiopathic inflammatory disease • Young middle aged adults of both genders • An antecedent viral prodrome occurs, and it is speculated to occur because of cell mediated immunity to viral antigen.(mumps, adenovirus, coxsackivirus) . • Associated with erythema nodosum, Wegener granulomatosis, polyarteritis nodosa, cerebral vasculitis, scleritis and episcleritis, sarcoidosis and ulcerative colitis (auto immune) • HLA B7 and HLA- DR2 are associated
- 29. • Symptoms –bilateral diminision of vision (subacute), photopsia, fellow eye is affected within few days or weeks, headache and other neurological symptoms are common • Signs – mild to moderate vitritis, multiple, large, flat, yellow creamy to placoid lesions at RPE level. They are 1-2 disc diameters in size and are located throughout the posterior pole. • Acute lesions heal over a period of 2-6 weeks with RPE pigmentary alterations & resultant chorioretinal atrophy. • Atypical findings include papillitis, retinal vasculitis, retinal vascular occlusive disease, retinal neovascularization, and exudative retinal detachment. • Differential diagnosis – serpiginous choroidopathy, TB, sarcoidosis
- 30. • FFA- ofactive lesions – hypofluorescence and late staining • ICGA – demonstrates non perfusion of choriocapillaries • Lumbar puncture to be done in patients with neurological symptoms Rx- • APMPPE is a self limiting condition which needs no treatment. • Recurrences are less • There are no convincing data to suggest that treatment with systemic corticosteroids is beneficial in altering the visual outcome. • It may be used in patients with extensive macular involvement, CNS, vasculitis & other associated autoimmune conditions
- 32. Serpiginous choroidopathy • Itis also known as geographical helicoid peripapillary choroidopathy (GHPC). • It affects healthy patients from the second to seventh decades of life. Men and women are affected equally. • It is usually bilateral, chronic, and progressive inflammatory condition. • Its etiology is unknown. • Associated with HLA- B7 • The disease is usually recurrent over years with relatively poor prognosis • Symptoms – Unilateral , blurring of central vision,
- 33. • Signs –mild vitritis, active lesions - Fundus shows asymmetric bilateral disease with characteristic gray white lesions at the level of the RPE with a pseudopodial or geographic extension from the peripapillary area into the posterior fundus • The disease starts around optic disc and extends gradually. Around 5% cases have the disease starting at central macula • The healed inactive chorioretinal lesions appear as well-demarcated geographic atrophic areas with or without pigment epithelial hyperplasia. • Recurrent attacks are typical with a progressive centrifugal extension • Late complications include retinal vein occlusion, macular hole, subretinal fibrosis and CNV usually occurring at the border of an old scar.
- 34. • FFA- showsearly hypofluorescence and then late staining of the active edge of the lesion • ICGA- hypofluorescence throughout all phases of the study for both acute and old lesions • Other investigations - investigated for TB, syphilis, sarcoidosis, Rx- systemic steroids- prednisolone60-80mg/dl, • A case report described using an intravitreous fluocinolone acetonide implant that resulted in ongoing control of the disease for 14 months postoperative follow-up. This delivery route avoids the side-effects of systemic corticosteroids. Cataract and glaucoma- adverse effects • immunosuppressive (cyclosporine, azathioprine,cyclophosphamide) may be effective alone or in combination,
- 37. References • Ryan’s retinaedition 6 – section 4 chapter 79 • White dot syndromes - Delhi J Ophthalmol 2015; 25 (4): 223-232