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SYMPATHETIC

OPHTHALMIA
Dr.Rajesh Babu B

MS, FMRF, MSc (CEH) DLSHTM, UK


Consultant
Uveitis & Ocular Immunology
Ocular Epidemiology & Community Eye Health
Narayana Nethralaya , Bangalore

Definition
Epidemiology
Theories of pathogenesis
Clinical manifestations
Symptoms
Examination findings
Complications
Pathology
Differential diagnosis
Fluorescein angiography findings
Treatment
Prognosis

SYMPATHETIC OPHTHALMIA

Sympathetic

ophthalmia is defined as a
bilateral granulomatous panuveitis that
occurs after the uvea of one eye is
subjected to a penetrating injury due to
either accidental trauma or surgery.
The term sympathetic ophthalmia was
coined by Mackenzie in the first half of the
19th century.

Definition

Incidence:
Prior to 1950 ~ 2% (16% during Civil War)
Retrospective studies: 0.2 - 0.5% after penetrating
trauma and 0.01% after intraocular surgery.
Prospective study: 0.03/100,000

Inheritance: No proven role. Postulated


correlation of HLA DRB1, DQA1 reported in
Japanese and UK series
Gender: M = F postsurgical; M > F traumatic
No racial predisposition
All ages, possibly increasing in the elderly

Epidemiology

Albert D, Diaz-Rohena R. A historical review of sympathetic ophthalmia and its epidemiology.Survey Ophthalmology1989; 34:
114.
Kilmartin D, Dick A, Forrester J. Prospective surveillance of sympathetic ophthalmia in the UK and Republic of Ireland .Br J

Difficult

to measure because it has always


been a relatively rare disease; as a
result of improvements in modern surgical
and medical treatment, it has become
even more uncommon.

Prevalence

Changing Trends in Sympathetic


Ophthalmia
TREND

HISTORICAL

CURRENT

Cause

Post trauma

Post surgery (esp. vitreoretinal)

Patients

Males and children (reflecting trauma


peaks)

No gender preference (reflects positive


impact of injury prevention programs) and
increasingly elderly patients (reflects
impact of ocular surgery)

Incidence

Considered disappearing 30 years ago

Probably increasing (underdiagnosed?)

Onset

For 65%, within 2-8 weeks; for 90% <


1yr

Many delayed presentations as well

Presentation

Granulomatous panuveitis

Any clinical uveitis

Inciting Eye

Enucleation within 2 weeks of trauma for


prevention of SO

Enucleation solely for the prevention of SO


is questionable

Visual Prognosis

Poor

Reasonable due to modern


immunosuppression

It has long been thought that uveal pigment is


somehow released by trauma and incites an
inflammatory reaction.
Neurogenic (extension from one eye to the other
through the optic nerves or via the ciliary nerves)
Infectious theory: tuberculosis, Actinomyces,
Rickettsia, virus
Combined ciliary nerve and bacterial theories
Allergic or Anaphylactic theory: Autoimmunity
against uveal melanin, uveal melanocytes, retinal
pigment epithelium, or retinal antigens (S & IRBP)
Role of Ocularof
Immune
Privilege
Theories
pathogenesis

Not

all cases of ocular injury progress to SO


Ocular immune privelege has a role to play
Antigen released by the trauma induces
ACAID
An Immunosupressive Microenvironment
Evolutionary protective mechanism
Thwarts any process that endangers vision

Role of Ocular Immune Privilege

Streilein JW (ed):Ocular Immune Privilege-Protection that preserves sight. Karger Gazzete. The eye
in focus. No.64 http://www.karger.ch/gazette/64/streilein/index.htm accessed 1 Feb 2010
Streilein JW (ed): Immune Response and the Eye. Chem Immunol. Basel, Karger, 1999, vol 73.

The

interval between ocular injury and the


onset of sympathetic ophthalmia has been
reported to be as short as 5 days or as
long as 66 years.
In general, 65% of sympathetic
ophthalmia cases occur 2 weeks to 2
months after injury
90% occur before 1 year.

Clinical manifestations

The

traumatized eye in SO, either from


an accidental penetrating injury or
following intraocular surgery,
characteristically exhibits a
persistent granulomatous
inflammatory reaction.

Clinical manifestations

SYMPTOMS
THE EARLIEST SYMPTOM MAY BE DECREASED
ACCOMMODATION AND THE EARLIEST SIGN,
RETROLENTICULAR FLARE AND CELLS IN THE FELLOW
EYE.
Irritable red eye with or without decreased vision.
Other symptoms include photophobia & transient
hyperopia.
Cutaneous and neurologic changes (alopecia, poliosis,
vitiligo, dysacousia, tinnitus, vertigo, and cells in the
cerebrospinal fluid), which are classically associated with
Vogt-Koyanagi-Harada syndrome, may rarely accompany
SO.

Bilateral granulomatous panuveitis:


Mutton fat keratic precipitates,
Cells and flare in the anterior chamber,
vitreous cells,
Isolated or confluent patches of yellowwhite choroidal infiltrates.
If undetected it eventually progresses to
panuveitis with exudative retinal
detachment.

On examination

Although not
pathognomonic, are
quite suggestive of SO
and may indicate a more
severe stage of SO.
Dalen-Fuchs nodules
are small, discrete,
yellowish infiltrates
at the level of the RPE
that are most often seen
and are largest in the
retinal-choroidal
periphery.

Dalen Fuch Nodules

Extensive anterior and posterior


synechiae, pupillary membrane
formation.
Rubeosis, glaucoma, cataract
Papillitis, optic atrophy
Exudative retinal detachment,
Chorioretinal scarring,
Choroidal neovascularization,
Phthisis.

Complications

The inflammatory changes in the exciting and


sympathizing eyes are the same, except for
features of trauma in the exciting eye.
Granulomatous Uveitis
The minimal and classic changes for the
histopathologic diagnosis of sympathetic
ophthalmia are diffuse lymphocytic
infiltration of the uveal tract with epithelioid
cell nests, pigment phagocytosis by the
epithelioid cells, absence of necrosis, and
sparing of the retina and choriocapillaris by
the granulomatous process.

Pathology

CASELLA, Antnio Marcelo Barbante et al. Sympathetic ophthalmia - histopathological


correlation with fluorescein and indocyanine green angiography: case report.Arq. Bras.
Oftalmol.[online]. 2008, vol.71, n.6 [cited 2010-01-31], pp. 886-889
http://www.scielo.br/img/revistas/abo/v71n6/a25fig02.jpg

The

uveal tract is usually diffusely


thickened (massively in some) by an
infiltration of lymphocytes in which various
numbers of nests of epithelioid cells
displaying pigment phagocytosis are
present.

Small, deep-yellow white


lesions called Dalen-Fuch's
nodules in the choroid. These
nodules occur mostly in the
periphery and consist of
epithelioid cells located just
internal to Bruch's membrane.
Histopathologically they are
nodular aggregations of
lymphocytes and epitheloid cells
with proliferation of retinal
pigment epithelium.
Dalen-Fuchs nodules are
composed of a mixture of
histiocytes, depigmented retinal
pigment epithelial cells with
lipofuscin and desmosomes, and
small numbers of T lymphocytes
of the suppressor-cytotoxic
subset
THE CHORIOCAPILLARIS IS
TYPICALLY SPARED.
Eosinophils are a feature of
sympathetic uveitis, especially in
early cases.

Dalen-Fuch's nodules

Focal nongranulomatous to diffuse


nonnecrotizing granulomatous infiltrate
Focal choriocapillary involvement
Chorioretinal adhesions
Retinal detachment
Optic atrophy
Retinal perivasculitis
Mild inflammatory involvement of the meninges
Preferential anterior segment inflammation

ATYPICAL PATHOLOGIC FEATURES

Multiple areas of
Early
Hyperfluorescence
and leakage at the
level of the RPE
(Dalen-Fuchs
nodules) and the
choroid (choroidal
granuloma) in most
cases, very similar to
those seen in
Harada's disease.
These sites (window
defects) correspond to
the Dalen-Fuchs
nodules observed
Presumably
the hyperfluorescent or hypofluorescent nature in the
clinically.

early phase is determined by whether the Dalen-Fuchs nodules have


an intact or a disrupted overlying RPE.

FLUORESCEIN ANGIOGRAPHY

If there is a serous retinal


detachment, pooling of dye in
the late frames of the
angiogram can be observed.
The FFA of the eye with
inactive S.O will
characteristically have
scattered multiple window
defects.

CASELLA, Antnio Marcelo Barbante et al. Sympathetic ophthalmia histopathological correlation with fluorescein and indocyanine green
angiography: case report.Arq. Bras. Oftalmol.[online]. 2008, vol.71, n.6
[cited 2010-01-31], pp. 886-889

Hyperemic disc with blurred


disc margins and tortuous
dilated vessels

Disc hyperfluorescence
with blurring of
margins in late phase

(C and D) FFA showing multiple areas (encircled) of


pinhead-sized leaks (arrow)
Sampangi R, Venkatesh P, Mandal S, Garg SP. Recurrent neovascularization of the disc in
sympathetic ophthalmia. Indian J Ophthalmol 2008;56:237-9

Vogt-Koyanagi-Harada

syndrome
Phacoanaphylactic uveitis
Sarcoidosis
Chronic idiopathic uveitis
Other granulomatous uveitis induced by
mycobacteria or fungi

DIFFERENTIAL DIAGNOSIS

Sympathetic
Ophthalmia

Vogt-Koyanagi-Harada
Syndrome

Age

All ages

20-50 years of age

Racial predisposition

None

Asian and Black

Penetrating trauma

Almost always present

Absent

Skin changes

Uncommon or unrelated

Common (60-90%)

CNS findings

Uncommon

Common (85%)

Hearing dysfunction

Uncommon

Common (75%)

Retinal serous
detachment

Uncommon

Frequently seen

Choriocapillaris
involvement

Usually absent

Frequently seen

CSF findings

Usually normal

Pleocytosis (84%)

Differences between SO & VKH

Role of Enucleation of the Inciting Eye


Conventionally said to prevent SO if within 2 weeks of
inciting event
Controversial whether enucleation is of benefit once SO
develops

Pharmacological Therapy
Current:
Corticosteroids - oral, periocular, intraocular, topical
Steroid-sparing agents - Imuran, Cellcept, Cyclosporine A
Prophylactic steroids do not prevent sympathetic
uveitis but can improve the visual outcome and can alter
the histopathologic features.

Treatment

The relapsing nature of SO and the potential toxicity of


treatment modalities warrant a careful long-term follow up
of patients with this disease.
Spontaneous improvement rarely occurs and if left
untreated, SO leads to loss of vision and phthisis bulbi.
The use of corticosteroid and other
immunosuppressive agents together with the
advancements in microsurgical techniques for wound repair
have improved the prognosis of SO,
50% of patients achieving a final visual acuity of
20/40 or better in at least 1 eye.
Cataract, secondary glaucoma, and chronic
maculopathy are the major causes of visual loss.

Prognosis

Thank You
drrajeshbabu@yahoo.com