Professional Documents
Culture Documents
syndromes
Introduction
• Associated with a viral prodrome but well defined etiology is lacking for
these conditions.
Treatment:
• Many white dot syndrome are benign and self limited.
• A few white dot syndrome are persistent and progressive with significant visual
consequences if untreated like MCP, Serpiginous choroiditis and birdshot
choroidopathy
• Mainstay of treatment includes local or systemic corticosteroids
DIFFERENTIAL DIAGNOSIS
• SYPHILIS • SARCOIDOSIS
• PRIMARY OCULAR HISTOPLASMOSIS • VKH SYNDROME
SYNDROME • SYMPATHETIC OPHTHALMIA
• TUBERCULOSIS • INTRAOCULAR LYMPHOMA
• DIFFUSE UNILATERAL SUBACUTE NEURO
RETINITIS.
Bird shot Choroidoretinopathy
• It is a bilateral and chronic process affecting choroid.
• Females are affected more then men within age group 40s-60s.
• HLA-A29 mutation is strongly associated with bird shot retinopathy.(seen in 95%
of patient)
• Symptoms - blurred vision, floaters, photopsia, defective colour vision and severe
nyctalopia despite normal visual acuity may also be a presenting symptom.
• Fundus –Multiple ill defined small, cream-colored, choroidal patches less than
one disc diameter in size, scattered around the optic disc and radiate to the
equator in a “shotgun” pattern involving posterior pole and midperiphery retina.
• The lesions radiate outward from the disc but spare the macula.
• ICGA- the birdshot lesions appear hypofluorescent during the intermediate phase
of angiography and appear to be bordered by medium-to-large vessels.
• OCT - shows significant thinning of the retina and choroid in the peripheral
locations. Macular edema is confirmed with OCT.
• ERG- normal, but with time show rod and cones abnormalities.
Rx-
• Anti- VEGF’s therapy for choroidal neovascularization and CME patients can be
given.
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 with delayed development in fellow
eye.
• 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 )
• Signs- anterior uveitis in 50% cases, vitritis, multifocal choroiditis.
Rx-
• Topical or systemic steroids. In chronic cases immunosuppressive agents can
be considered.
• Choroidal Neovascularisation can be managed with anti-VEGF therapy
with or without the use of corticosteroids.
Late staining on FFA
Punctate inner
choroidopathy
• It is an idiopathic inflammatory disease of the choroid.
• Young myopic females are affected with bilateral involvement.
• Association with Epstein-Barr virus and with HLA-DR2 positivity has been
reported.
• Symptoms – blurring of vision, floaters, photopsia or paracentral scotomas.
• Signs- anterior uveitis or vitritis is mild or may be absent.
• Cells and flare in the anterior chamber or vitreous cavity are typically absent.
The lack of vitreous inflammation is a hallmark of PIC.
• Fundus- several small yellow-white macular spots with fuzzy borders at the
level of the inner choroid and retina which may evolve into sharply demarcated
atrophic scars with little pigmentation.
• Serous retinal detachment may occur overlying an active lesion.
Rx-
• Systemic Corticosteroids are used in the active stages. CNV can be managed
with anti-VEGF injections in combination with oral steriods.
(C) FA arterial phase (D) FA late phase of eye
Sub-Retinal Fibrosis and Uveitis
Syndrome
• It is a rare form of panuveitis of unknown etiology affecting otherwise
healthy myopic women between the ages of 14 and 34 years.
• Autoimmune etiology has been implicated with the demonstration of local
antibodies and B-lymphocytes.
• 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.
• Fundus- multiple small (100-200 micron), ill-defined, grey white spots sparing the
fovea (perifoveal) at the level of the RPE or deep retina. These spots fade away
and are frequently missed but leave behind a characteristics orange granular
macular pigmentary change which is pathognomic and a dulled reflex.
• Rarely CNV may develop and may be the presenting sign.
• FFA- it may reveal disc capillary leakage and late punctate staining,
characteristically in a wreath like pattern.
• ICGA- multiple hypofluorescent spots that are more numerous than those
seen clinically or in FA.
• Visual field defects are variable and range from generalized depression,
paracentral or temporal scotoma to enlargement of the blind spot.
( D/D- Retrobulbar optic neuritis)
• Acute lesions heal over a period of 2-6 weeks with RPE pigmentary alterations &
resultant chorioretinal atrophy.
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
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 but highly assymetrical, chronic, and progressive
inflammatory condition.
• Its etiology is unknown.
• Associated with HLA- B7 and retinal s-antigen.
• The disease is usually recurrent over years with relatively poor prognosis.
• Symptoms – patient present with painless, unilateral, paracentral scotomata
and decreased vision.
• Signs – mild vitritis.
• 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
• Late complications include retinal vein occlusion, macular hole, subretinal fibrosis
and CNV usually occurring at the border of an old scar.
• 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