Posterior Uveitis approxs about 10-30% of all uveitis cases. It presents in varied clinical entities. The pt should be throughly examined and investigated. Hope this ppt will serve some help in doing so..!
Inflammation of retina or choroid posterior to vitreous
SUN working group classification of uveitis and the primary site of
Primary site of
Pars planitis, posterior
Focal, multifocal, diffuse
vitreous and retina or
Global variation and pattern changes in epidemiology of uveitis
SR Rathinam, P Namperumalsamy
Aravind Eye Hospital and PG Institute of Ophthalmology, 1, Anna Nagar, Madurai - 625 020, India
In paediatric uveitis, anterior uveitis accounts for 30-40%,
posterior uveitis 40-50%, intermediate uveitis 10-20% and
Cunningham ET Jr. Uveitis in Children. Ocular Immunology and
Pattern of uveitis in a referral uveitis clinic in India
Debashis Das, Jyotirmay Biswas, Sudha K Ganesh
A standard clinical protocol was followed for each case. Of the 465 new uveitic cases,
anterior uveitis was most commonly encountered (170 cases, 36.5%), followed by
posterior uveitis (132 cases, 28.4%), intermediate uveitis (92 cases, 19.8%) and
panuveitis (71 cases, 15.3%).
FINDING THE TRUTH..!
Critical questions for diagnosis:
1. Is it posterior uveitis only or is it part of a panuveitis?
2. Is it choroiditis, retinitis, or retinochoroiditis?
3. Is there associated involvement of the optic nerve head and/
or the retinal vessels?
4. Does the clinical feature fit into any known infective or noninfective entity?
5. Is there associated anterior segment inflammation, vitritis,
6. Is it associated with other systemic features?
7. Is it recurrent? If so, how has it responded to previous
8. Is it associated with an immunocompromised state?
9. Is it a masquerade syndrome?
Diagnostic criteria- malar rash
Choroidopathy: Fundus examination may reveal
multiple serous retinal detachments, retinal pigment
epithelium detachments and a central serous
Retinopathy: It typically occurs bilaterally but may be
unilateral or asymmetric. The most common findings
include cotton-wool spots and hemorrhages. Less
common findings include hard exudates, retinal edema,
visual acuity loss or metamorphopsia, and vascular
Ant uveitis- U/L or B/L
Specific tests- Anti nuclear Ab Assay
Reduced corneal sensations
Sectoral atrophy of iris
Peripheral retinal periarteritis
ARN- retinal necrosis in periphery which rapidly
PORN- rapidly progressive visual loss
Full thickness necrosis and early macular
PCR based assay.
Acute retinal necrosis
(ARN): classical presentation
of herpetic viruses.
Triad of moderate to severe
vitritis, arteritis, and
periphlebitis and confluent
peripheral retinal necrosis is
diagnostic of ARN, which can
present as a panuveitis.
Progressive outer retinal necrosis
(PORN): Necrotizing retinitis
Confluent areas of outer retinal
whitening with minimal vitritis
involving the posterior pole and
sparing of retinal vessels at the early
stage, the typical “cracked mud
appearance”is virtually diagnostic of
PORN is frequently bilateral,
occurring exclusively in
immunocompromised state, such as in
patients with HIV infection, and is
associated with rapid development of
rhegmatogenous retinal detachment
or optic atrophy.
Ant seg- sarcoid granulomas, iris nodules
Post seg- vitritis, periphlebitis, snow balls
Diagnosis- x-ray chest
Ocular sarcoidosis (A) Large iris
nodules; (B) nodular involvement of the
trabecular meshwork; (C) snowballs
Periphlebitis in sarcoidosis. (A) Periphlebitis
with involvement of the optic nerve head; (B)
occlusive periphlebitis and disc oedema; (C)
Choroidal and retinal involvement in sarcoidosis. (A) Small peripheral
choroidal granulomas; (B) confluent choroidal infiltrates; (C) multifocal
choroiditis; (D) multiple small retinal granulomas
Toxoplasmosis in the immunocompromised host
The classic clinical triad of retinochoroiditis,
cerebral calcifications, and convulsions defines
Hydrocephalus and right
anophthalmos; (B) axial
CT shows cerebral
Ocular Toxoplasmosis is the most common cause of
Posterior uveitis, the lesions develop in deep retina,
few to no vitreous cells may be present.
It frequently presents as a focal necrotizing retinitis,
adjacent to a larger, atrophic chorioretinal scar,often
located in macula in congenital cases.
The hallmark of the disease is a
which may be primary or
In primary ocular
toxoplasmosis, a unilateral
focus of necrotizing retinitis is
present at the posterior pole in
more than 50% of cases
The area of necrosis usually
involves the inner layers of the
retina and is described as a
whitish fluffy lesion surrounded
by retinal edema
Active toxoplasma retinitis. (A) Typical
‘satellite’ lesion adjacent to an old scar; (B)
two small foci; (C) severe vitreous haze and
‘headlight in the fog’ appearance
Progression of toxoplasma retinitis. (A) Mild
fluffy haze adjacent to an old scar at
presentation; (B) after 2 weeks the area of
retinitis is larger and denser; (C) after 7
weeks the retinitis has nearly resolved.
The retina is the primary site for the multiplying
parasites, while the choroid and the sclera may
be the sites of contiguous inflammation
When the optic nerve becomes involved by
toxoplasmosis, the typical manifestation is optic
neuritis or papillitis associated with edema, often
called Jensen disease.
The sheath of the optic nerve may serve as a
conduit for the direct spread of Toxoplasma
organisms into the optic nerve from an adjacent
cerebral infection. This also results in optic
neuritis or papillitis
Posterior vitreous detachment is commonly seen,
and patients may develop precipitates of
inflammatory cells on the posterior vitreous face,
referred to as vitreous precipitates
The Critical sign include
white yellow retinal
lesion with hazy Vitreous
as a result of presence of
Vitreous cells (Headlight
Old Choroiretinal scar
can be seen often
adjacent to new white
yellow lesion but it is not
Toxoplasma antigens are responsible for a
hypersensitivity reaction that may result in
retinal vasculitis and granulomatous or
nongranulomatous anterior uveitis.
Posterior synechiae may complicate the course of
anterior uveitis, and keratic precipitates (KP)
may be seen
KP may appear in the classic Arlt distribution in
milder nongranulomatous configurations and
Some patients present with the stellate KP
pattern, characterized by a diffuse homogeneous
distribution pattern and a stellate fibrillar KP
As the lesion heals, it appears as a punched-out
scar, revealing white underlying sclera. This
results from extensive retinal and choroidal
necrosis surrounded by variable pigment
With reactivation of live tissue cysts located at
the border of the scars (recurrent ocular
toxoplasmosis), the areas of newly active
necrotizing retinitis are usually adjacent to old
scars (so-called satellite lesions)
In some patients, multiple grayish white dots at
the level of the retinal pigment epithelium (RPE)
appear. No associated vitreous reaction occurs
with this manifestation
As in other inflammatory conditions, macular
edema may be seen
Rarely, ocular inflammation without the
necrotizing retinochoroiditis can occur in patients
with acquired toxoplasmosis.
These patients present with retinal vasculitis,
vitreitis, and anterior uveitis. Later, they may
develop retinochoroidal scars that suggest that
the inflammatory reaction was secondary to T
Rarely, retinal and optic nerve neovascularization
The neovascularization usually regresses with
resolution of the inflammation
The exact etiology of neovascularization of the optic
nerve and the retina is not well understood.
Retinal ischemia associated with severe retinal
vasculitis may predispose to neovascularization of
The areas of retinitis are the result of tissue cysts
bursting and releasing bradyzoites that
transform into tachyzoites, which in turn invade
These destructive lesions are usually larger than
1 disc diameter and appear as soft, white, fluffy
infiltrates surrounded by retinal edema with
the tachyzoites come under increasing
attack by the host’s immune response, they
gradually transform back into bradyzoites.
cells will be found in the vitreous
overlying the active lesion.
inflammatory exudates are
frequently present around retinal vessels
peripheral to an area of active inflammation.
patients presents initially
with severe unilateral papillitis, macular
hard exudates distributed in a star
fashion, and vitreal inflammation.
Active toxoplasmosis simultaneously
involving both the retina and the optic
nerve is unusual.
CME or CNVM is seen on rare
occasion or as late sequel.
diagnosis is usually based on the clinical
appearance of the fundus lesion. Serologic evidence
of exposure to Toxoplasma organisms serves as
Serum antitoxoplasma antibody titers can be
determined by several techniques, to include the
Enzyme-linked immunosorbent assay (ELISA)
Indirect fluorescent antibody test
Indirect hemagglutination test
Sabin-Feldman dye test
Serologic findings are important in determining whether
acute or chronic systemic infection is present.
Acute systemic toxoplasmosis has traditionally been
diagnosed by seroconversion.
Anti-Toxoplasma immunoglobulin G (IgG) titers
present a 4-fold increase that peak 6-8 weeks following
infection, then decline over the next 2 years, but remain
detectable for life
Anti-Toxoplasma IgM appears in the first week of the
infection and then declines in the next few months.
The presence of anti-Toxoplasma immunoglobulin A
(IgA) has also been shown to be detectable in acute
infection; however, since the titers can last for more
than 1 year, its value in helping to diagnose an acute
phase is limited.
Value of Serological Tests for Toxoplasmosis
* No previous infection: < 1:16
* Prevalent in general population: 1:16-1:256
* Suggests recent infection: > 1:256
* Suggests active infection: > 1:1024
In children: any titer is significant
In adults: > 1:64 indicates active infection
techniques are usually reserved for cases
such as immunocompromised patients.
Ocular fluids can demonstrate the presence of
intraocular antibody production.
Polymerase chain reaction can detect the
A fluorescent treponemal antibody absorption (FTAABS) test should be obtained to rule out syphilis.
Fluorescein angiography (FA) of active lesions shows
hypofluorescence during the early phase of the study,
followed by progressive hyperfluorescence secondary to
Indocyanine green (ICG) of active lesions are mostly
hypofluorescent. ICG has imaged hypofluorescent
satellite lesions that are not imaged by FA and are not
seen during clinical examination.
The etiology of such hypofluorescent lesions is unknown
but suspected of being a noninfectious, perilesional
It is indicated in the presence of ocular media
opacities, especially vitreous opacities. The most
common findings include intravitreal punctiform
echoes, thickening of the posterior hyaloid,
partial or total posterior vitreous detachment,
and focal retinochoroidal thickening.
INDICATIONS FOR TREATMENT
Lesions within the vascular arcade,
Lesions near the optic disc (Jensen papillitis),
Lesions in the papillomacular bundle
Large lesions irrespective of location are treated.
Patients with severe debilitating vitritis are also
In the case of ocular toxoplasmosis, several
therapeutic regimens have been recommended.
Triple drug therapy refers to pyrimethamine,
sulfadiazine, and prednisone.
Quadruple therapy refers to pyrimethamine,
sulfadiazine, clindamycin, and prednisone.
(Pyrimethamine should be combined with folinic
acid to avoid hematological complications.)
The duration of treatment varies depending on
the patient's response but usually lasts for 4-6
Exerts bacteriostatic action through competitive antagonism with
para-aminobenzoic acid (PABA).
Double strength (DS) tab - 800 mg sulfamethoxazole & 160 mg
Regular strength tab- 400 mg sulfamethoxazole & 80 mg
1 DS tab bid for first 2 wk; then, 1 regular tab bid for next 3-4 wk
<2 months: Not recommended
>2 months: 8 mg/kg/d of trimethoprim plus 40 mg/kg/d of
sulfamethoxazole divided bid
Safety for use during pregnancy has not been established.
Pyrimethamine (Daraprim) is administered as a loading dose
of 50 mg followed by 25–50 mg daily for 4 weeks in
combination with oral folinic acid 5 mg (mixed with orange
juice) three times a week to prevent thrombocytopenia,
leucopenia and folate deficiency. Weekly blood counts should
be performed. In AIDS pyrimethamine is avoided because of
possible pre-existing bone marrow suppression and the
antagonistic effect of zidovudine when the drugs are
Sulfadiazine 1 g q.i.d. for 3–4 weeks is usually given in
combination with pyrimethamine. Side-effects of
sulphonamides include renal stones, allergic reactions and
Other systemic options include clindamycin, spiramycin,
tetracyclines, atovaquone, azithromycin and clarithromycin.
Topical steroids may be given for anterior uveitis but
periocular depot injections are contraindicated as they may
lead to uncontrolled progression.
must be exercised if photocoagulation or
cryotherapy is being considered in the treatment of
Intraretinal hemorrhages, vitreous hemorrhage, and
retinal detachment have been reported as complications
of such treatment. Tissue cysts can exist in a normalappearing retina.
plana vitrectomy may be indicated in cases of
retinal detachment secondary to vitreous traction
or in cases where vitreous opacities persist.
The principal mode of transmission worldwide is
through the ingestion of oocysts shed into the
environment in the feces of cats.
These organisms remain viable in the soil for at
least a year and become a source of infection for
mice, rats, birds, and other prey.
In one series, 40% of patients had a final visual
acuity of 20/100 or worse, and 16% of patients
had a visual acuity between 20/40 and 20/80.
Toxoplasma retinitis frequently reactivates, and
recurrence rates approach 50% at 3 years
Patients with recurrent disease are more likely
to have a permanent visual disability.
Choroidal neovascular membrane
Branch retinal vein occlusion
Branch retinal artery occlusion
Tractional retinal detachment
Secondary glaucoma may occur with anterior uveitis that is
secondary to the obstruction of the outflow channels by the
Destruction of the trabecula by chronic inflammation and
anterior synechiae may also create a chronic
pharmacologically nonresponsive glaucoma.
Cystoid macular edema
4 – 8 yr old
H/o contact with dogs/ geophagia
Post pole / peripheral granuloma
RD, cyclitic membrane
ELISA, USG, CT scan
Chronic toxocara endophthalmitis may cause a
cyclitic membrane and a white pupil.
Toxocara granuloma. (A)
Juxtapapillary granuloma: (B)
posterior pole granuloma
associated with a localized
tractional retinal detachment;
(C) peripheral granuloma with
a vitreous band extending to
Marked vitreous inflammation, yellow grey
strands extending into vitreous from
CT- no calcification
Positive serological test is supportive but not
An idiopathic inflammatory venous occlusion that
primarily affects the peripheral retina of adults.
Henry Eales, a British ophthalmologist 
It is a diagnosis of exclusion, as many other retinal
disorders can mimic Eales disease
Retinal changes include perivascular phlebitis,
peripheral non perfusion, and neovascularization.
Visual loss is characteristically caused by bilateral
recurrent vitreous haemorrhage and its sequelae.
Rare in developed countries ,more commonly
reported from the Indian subcontinent.
Healthy young adults
20 to 30 years
Associated with TB
Symptoms- floaters or decrease of visual acuity.
70 to 80% of patients develop bilateral
Charmis in 1965 classified Eales' disease into
Stage I : Mild periphlebitis of small peripheral
Stage II : Perivasculitis of the venous capillary
system is widespread.
Stage III: New vessel formation with vitreous
Stage IV : Retinitis proliferans and tractional
Eales disease. (A) Peripheral
vascular sheathing and occlusion
in the superotemporal quadrant;
haemorrhage from new vessels
In the early stages of the disease - venous
dilatation in the periphery with tortuosity and
discontinuity of veins.
Vascular sheathing ranges from thin white lines
limiting the blood column on both sides to heavy
Superficial flame-shaped haemorrhages are
seen in the areas of sheathed vessels
Later the arteries also attenuate in the
The involved vessels become obliterated and an
avascular area develops in the periphery better
visualized by FFA
Intraretinal haemorrhages , increase in vascular
tortuosity , collateral formation around occluded
Junction - sharply demarcated.
The vascular abnormalities at the junction
include microaneurysms, veno-venous shunts,
venous beading and occasionally hard exudates
and cotton-wool spots.
NVE, is more common than NVD and is usually
located at the junction between the perfused and
Bleeding from neovascularization is common, usually
recurrent, and is one of the major causes of visual loss
Signs of fibrous organization, retinitis proliferans or
even tractional retinal detachment.
Some patients may develop uveitis, complicated
cataract, rubeosis iridis, and secondary neovascular
glaucoma in the late stage of the disease
Active vasculitis is characterized by staining of
the vessel wall or even frank extravasation
Retinal oedema is characterized by increased
permeability and diffuse extravasation of the
The new vessels become distinctly outlined
during the AV phase .
These soon begin to leak the dye.
Monitoring the regression and disappearance of
new vessels during treatment and follow-up
The diagnostic clinical picture of Eales' disease is the
evidence of peripheral venous inflammation, past or
Inflammation of the vein causes variable degrees of
Retinal ischaemia stimulates neovascular growth from
the surrounding normal vasculature
Occasionally with time, there is a tendency for the new
vessels to regress. Get replaced by glial tissue.
The glial tissues on contraction cause several retinal
The macula may get distorted or detached.
Rhegmatogenous retinal detachment.
Type III (immune complex mediated) and/or type
IV delayed hypersensitivity reaction to an
infectious agent probably leads to retinal changes
in Eales' disease.
Both antiretinal autoimmunity and circulating
immune complexes may act as
Antiendothelial cell antibodies
Predominant involvement of CD4 positiveT-cells
Indicate that a cell-mediated immune mechanism
Raised alpha-globulins and reduced albumin
levels in the serum
Raised serum alpha-1 acid glycoprotein (an
important immunoregulatory protein formed by
lymphocytes and epithelial cells)
PDGF, IGF-I, epidermal growth factor (EGF),
TGF-a and TGF-(3), play a key role in the process
Epiretinal membrane (ERM) in Eales' disease,
composed of several neovascular channels with
glial cells, macrophages , fibrocytes, retinal
pigment epithelial cells ,collagen material and
Significant lymphocytic infiltration.
Predominance of T-cells in the ERM and SRM
indicate a cell-mediated immune reaction
involved in the formation of these membranes.
Corticosteroids, photocoagulation with or without
anterior retinal cryoablation (ARC), and
High doses of oral steroids, e.g. prednisolone 2
mg/kg body wt, gradually tapering off
Periocular depot steroids (hydrocortisone or depomedrol) given as deep posterior sub-Tenon
injection in active retinal vasculitis.
The ATT regimen usually includes two drugs
(rifampicin 450 mg; isoniazid 300 mg once daily)
for nine months.
Usually reserved for cases with acute phlebitis
with massive infilteration, nodule formation, and
complete obliteration of segments of the vein.
Xenon arc photocoagulation in the area posterior
to the vortex vein and cryotherapy anterior to
the vortex vein.
Cryotherapy consisted of 4 to 5
ophthalmoscopically controlled transconjunctival
applications in each quadrant involved , not more
than two quadrants were treated in one
Laser photocoagulation- currently, the most
accepted modality of treatment in Eales' retinopathy
Beneficial in stage II and III of Eales' disease.
Focal treatment of flat retinal new vessels, sectoral
scatter photocoagulation of areas of capillary
dropouts and direct treatment of neovascular frond
into the vitreous have been found beneficial in
proliferative Eales' retinopathy
Unresolving vitreous haemorrhage
Tractional retinal detachment involving the
Multiple vitreous membranes with or without
tractional retinal detachment
Combined tractional and rhegmatogenous retinal
Age- 25- 35 yrs
Diagnostic criteria Major- Recurrent oral aphthae
Pathergy, HLA B51, ESR, CRP
NG ant uveitis of explosive nature
Obliterative , necrotizing vasculitis
Both arteries and veins in post pole
Ocular Behçet syndrome. (A) Hypopyon in a white eye; (B) retinal
infiltrates; (C) occlusive vasculitis; (D) end-stage disease
B/L gran panuveitis
After surgery / penetrating trauma
Dalen Fuchs nodules – multiple, periphery
FFA- multiple hyper spots at RPE level
Nodules may show early blocked fluorescence
B scan – choroidal thickening
Vogt–Koyanagi–Harada (VKH) syndrome is an
idiopathic multisystem autoimmune disease featuring
inflammation of melanocyte-containing tissues such as
the uvea, ear and meninges.
VKH can be subdivided into Vogt–Koyanagi disease,
characterized mainly by skin changes and anterior
uveitis, and Harada disease, in which neurological
features and exudative retinal detachments
Possible trigger factors include cutaneous injury or a
viral infection which may lead to sensitization of
1. Prodromal phase: lasting a few days is characterized by
neurological and auditory manifestations. Meningitis causing
headache and neck stiffness.
Encephalopathy is less frequent and may manifest with
convulsions, paresis and cranial nerve palsies.Auditory
features include tinnitus, vertigo and deafness.
2. Acute uveitic phase: follows soon thereafter and is
characterized by bilateral granulomatous anterior or
multifocal posterior uveitis and exudative retinal
3. Convalescent phase: follows several weeks later and is
characterized by: Localized alopecia, poliosis and vitiligo.
Focal depigmented fundus lesions (sunset glow fundus) and
depigmented limbal lesions (Sugiura sign).
4. Chronic-recurrent phase: is characterized by smouldering
anterior uveitis with exacerbations
CRITERIA FOR VKH
1. Absence of a history of penetrating ocular trauma
2. Absence of other ocular disease entities
3. Bilateral uveitis
4. Neurological and auditory manifestations
5. Integumentary findings, not preceding onset of central
nervous system or ocular disease, such as alopecia, poliosis
complete VKH, criteria 1–5 must be present.
incomplete VKH, criteria 1–3 and either 4 or 5 must be
probable VKH (isolated ocular disease), criteria 1–3 must
Posterior uveitis occurs in patients with Harada disease and is frequently
bilateral. In chronological order the findings are as follows:
Diffuse choroidal infiltration and papillitis.
Multifocal detachments of the sensory retina and disc oedema.
The chronic phase shows diffuse RPE atrophy (sunset glow fundus) which
may be associated with small peripheral atrophic spots .
Complications include CNV and subretinal fibrosis.
FA of the acute phase shows multifocal hyperfluorescent dots at the level
of the RPE and then accumulation of dye in the subretinal space. The
chronic phase shows areas of hyperfluorescence due to RPE window
Treatment involves high-dose oral prednisolone (60–100 mg/day) that may
be augmented with 3-day intravenous pulse therapy with
methylprednisolone (500–1000 mg/day). Steroid-resistant patients may
Sunset glow fundus
Active Harada disease. (A) Multifocal
serous retinal detachments; (B) FA
venous shows multiple hyperfluorescent
spots; (C) late phase shows extensive
areas of hyperfluorescence due to pooling
of dye under the serous detachments
Ant uveitis- granulomatous, KPs
h/o fever, sweats, weight loss, headache, localised back
ache, abd pain.
Choroiditis is caused by direct infection.
Unilateral focal or (less frequently) multifocal.
Extensive diffuse choroiditis may occur in patients
with AIDS .
Large solitary choroidal granulomas are uncommon.
Choroiditis may occasionally resemble serpiginous
Periphlebitis is often bilateral and represents a
manifestation of hypersensitivity to the bacillus. It
may be mild and innocuous or occlusive resulting in
severe retinal ischaemia and secondary retinal
In TB granuloma, OCT reveals an area of localized
adhesion between the choriocapillaris–RPE layer and
overlying the neurosensory retina (“contact” sign),
possibly due to inß ammatory adhesions overlying the
granuloma that cause the neurosensory retina to stick
to the RPE at that point.
Treatment is initially with at least three drugs
(isoniazid, rifampicin and pyrazinamide) and then
with isoniazid and rifampicin.
Quadruple therapy with the addition of ethambutol is
necessary in pulmonary TB and in resistant cases.
Concomitant systemic steroid therapy is also
frequently necessary. The steroid dose needs to be
adjusted when given with rifampicin.
Tuberculous choroiditis (A) Diffuse
involvement in a patient with AIDS;
(B) choroidal granuloma
(A) Tuberculous choroiditis resembling
serpiginous choroidopathy; (B) FA shows
corresponding areas of hyper- and
Cytomegalovirus retinitis. (A)
Indolent retinitis; (B)
fulminating disease; (C)
advanced disease involving the
optic nerve head; (D) large
posterior retinal tear with
shallow localized detachment
PRESUMED OCULAR HISTOPLASMOSIS
Histoplasmosis is caused by Histoplasma capsulatum
acquired by inhalation of infective mycelia fragments
and/or spores with dust particles.
The organisms then pass via the bloodstream to the
spleen, liver and, on occasion, the choroid, setting up
multiple foci of granulomatous inflammation.
Absence of intraocular inflammation.
The acute stage may manifest with localized swelling
of the choroid which may also lead to changes in the
Atrophic ‘histo’ spots consist of roundish, slightly
irregular, yellowish-white lesions about 200 µm in
diameter, often associated with pigment clumps within
or at the margins of the scars. The lesions are
scattered in the mid-retinal periphery and posterior
Peripapillary atrophy may be diffuse or focal, or a
combination of both.
Linear streaks in the midperiphery are found in 5% of
histoplasmosis syndrome; (A)
Peripheral ‘histo’ spots; (B)
atrophy and histo spots; (C)
NHL of the eye and CNS ( Intraocular CNS lymphoma)
50 to 60 yrs
Diagnosis- CNS evaluation
Multifocal choroiditis with panuveitis
Adults (20-50) ( F > M )
Bilateral but asymmetrical
Deep,discrete, grey-yellow spots
Mixed fresh and old
Mid-periphery and fewer at posterior pole
Moderate to severe panuveitis
Middle age (F = M)
Eventually bilateral but asymmetrical
Deep, grey-white lesions with hazy
Initially peripapillary and at
posterior pole then outward spread
Many substances fluorescence spontaneously (emit
light of a particular wavelength) when illuminated by
light of a different wavelength.
This fluorescence emission, arising from endogenous
fluorophores, is an intrinsic property of cells and is called
FAF : excitation 488nm,emission >500nm
Utilizes fluorescent properties of lipofuscin to study the
health and viability of the retinal pigment
Studying Lipofuscin through FAF
Lipofuscin (LF) is a mixture of autofluorescent
pigments that accumulate in post-mitotic cells
In the RPE, LF granules accumulate in the lysosomal
compartment mainly as a by-product of constant
phagocytosis of shed PR outer segments.
Accumulation of lipofuscin and its major flurophore
A2E [formed out of residues of all-trans retinal ] can
be visualized with FAF.
A2E can disable the function of RPE cell
Increase or decrease of FAF intensity is associated
with different disease stages.
AUTOFLUORESCENCE IN POSTERIOR
In the acute phases of APMPPE,
AF imaging shows more lesions as
compared to FFA or
This indicates that RPE damage
occurs secondary to choroidal
changes. During the follow up,
many lesions developed increased
pigmentation centrally, with a
depigmented halo clinically.
The central portions of the lesions
appeared to have increased
pigment and showed hyperautofluorescence.
The depigmented halo appeared to
show decreased, almost absent AF,
suggesting atrophy or absence of
functional RPE cells.
Acute phase of MEWDS, AF
photography shows less numerous,
but increased AF areas,
corresponding to the focal
hypocyanescent spots seen on
indocyanine green angiography
(ICG), probably due to the
excitation of the photoreceptorretinal pigment epithelium
Following resolution of the
lesions, AF and ICG return to a
Multifocal choroiditis and
In MCP, AF shows numerous hypo
autofluorescent spots corresponding
to the clinically visible chorioretinal
scars, but more in number.
This indicates more extensive RPE
damage compared to the clinically