The white dot syndromes are a group of diseases characterized by inflammation of the outer retina, retinal pigment epithelium, and choroid. They include birdshot retinochoroidopathy, multifocal choroiditis and panuveitis, punctate inner choroidopathy, subretinal fibrosis and uveitis syndrome, multiple evanescent white dot syndrome, acute posterior multifocal placoid pigment epitheliopathy, and serpiginous choroidopathy. The etiology is unknown but may be autoimmune or infectious. Patients typically present with blurred vision, photopsias, and floaters. Examinations reveal multiple cream-colored lesions throughout the fundus. Treatment involves corticosteroids and immunosuppress

1. White dot syndromes
Dr. Nikhil. R.P

2. • Introduction
• Bird shot 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 white spot 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 occurs in 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- focal elevated 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 been the 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 and panuveitis
• 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- anterior uveitis, 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.

15. Late staining on FFA

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 retinal detachment 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 arterial phase (D) FA late phase of eye

20. Sub-Retinal Fibrosis and Uveitis 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 the next 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 white dot 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 occurs over 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 prognosis is 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 multiocal placoid 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- of active 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
• It is 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- shows early 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 retina edition 6 – section 4 chapter 79
• White dot syndromes - Delhi J Ophthalmol 2015; 25 (4): 223-232