CAUSES OF PAN UVEITIS AND MANAGEMENT

OF SYMPATHETIC OPHTHALMITIS

Photo Credit: Kanski, 7th Edition

Photo Credit: Kanski, 7th Edition

Dr. Chima Emmanuel Edoga

Photo Credit: Kanski, 7th Edition

Junior Resident, Department of Ophthalmology, ESUT-TH, Parklane, Enugu State, Nigeria

May 12th, 2016

CONTENTS

Total Slides = 20

Introduction
Causes of Pan-Uveitis

Management of Sympathetic Ophthalmitis

INTRODUCTION
On the basis of anatomy, the SUN (Standardisation of Uveitis Nomenclature)

working group classified Uveitis into the following:

1. Anterior uveitis
2. Intermediate uveitis
3. Posterior uveitis
4. Pan-uveitis
Pan-uveitis is a subset of uveitis where there is inflammation of all segments
of the uveal tissue without a predominant site of inflammation
The primary sites of inflammation in pan-uveitis are the anterior chamber,
vitreous, and retina or choroid
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INTRODUCTION

SUN (Standardization of
Uveitis Nomenclature)
Classification of Uveitis

CAUSES OF PAN-UVEITIS
1. Infectious endophthalmitis
2. Sympathetic Ophthalmitis
3. Tuberculosis
4. Syphilis
5. Toxoplasmosis
6. Toxocariasis
7. Cysticercosis
8. Sarcoidosis
9. VogtKoyanagiHarada Syndrome (VKH Syndrome)
10. Behets disease
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MANAGEMENT OF SYMPATHETIC OPHTHALMITIS

Sympathetic ophthalmitis is a bilateral granulomatous pan-uveitis occurring after
penetrating ocular trauma, often associated with uveal prolapse, or, less frequently,
following intraocular surgery, usually multiple vitreo-retinal procedures or laser ciliary
ablation procedures, particularly direct contact lasers
The traumatized eye is referred to as the exciting eye and the fellow eye, which also
develops uveitis, is the sympathizing eye
It is mostly a presumptive diagnosis since histological proof is frequently lacking
Management will involve
1. History taking
2. Examination
3. Investigations
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4. Treatment (+ Differential Diagnosis and Prognosis)

MANAGEMENT OF SYMPATHETIC OPHTHALMITIS

HISTORY
Age: Can occur at any age
Sex: Males are more commonly affected as they are more likely to suffer from penetrating eye
injury. Following surgery, males and females are equally affected
Incidence: About 0.20.5% after penetrating eye injury and 0.01% following intraocular
surgery
Presentation: between 2 weeks and 3 months after initial injury in 65% of cases is and
within the first year in 90% of all cases. Can occur anytime between 1week and 66 years

MANAGEMENT OF SYMPATHETIC OPHTHALMITIS

SYMPTOMS: Symptoms produced by uveitis depend on
Which part of the uveal tract is inflamed
The rapidity of onset (sudden or insidious)
The duration of the disease (short or prolonged)
The course of the disease (acute, chronic, or recurrent)
History of causative trauma
The exciting eye is frequently red, irritable
The sympathizing eye
Irritation
blurred vision (due to cells and fibrin in the AC, Lens opacification [cataract], vitreous haze and
macular edema)
Photophobia
compromised near vision (due to loss of accommodation)
Floaters and scotoma

MANAGEMENT OF SYMPATHETIC OPHTHALMITIS

HISTORY Contd..
Pain: May be due to iritis leading to release of mediators of pain, Ciliary spasm, which
radiates over a large area supplied by the trigeminal nerve or 2o glaucoma
Other symptoms resulting from complications of SO such as Cataract, glaucoma,
choroidal neovascularization (CNV), sub-retinal fibrosis and atrophy of the optic nerve,
retina or choroid
SIGNS
VA: mild to severely diminished (worse in the exciting eye, if Px still seeing with it)
Globe: Exciting eye may be Pthisical or reduced size; Sympathazing eye is be intact
Eye Brow: Vertiligo (also in VKH syndrome)
Lids: Poliosis (also in VKH syndrome); nodules
IOP: May be normal or elevated (if associated with Glaucoma)
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Movt: May be normal or reduced

MANAGEMENT OF SYMPATHETIC OPHTHALMITIS

Cornea: KPs (Keratic Precipitates)
Most commonly reported corneal finding in uveitis
They are small aggregates of inflammatory cells that accumulate on the endothelial surface of
the cornea
Distribution
Generally, due to the convection currents in the AC, the cells are seen in the lower part of
the endothelium (von Arlts triangle with apex pointing up and base pointing down)
They are very rarely seen dispersed throughout the endothelium (except in Fuchs
heterochromic iridocyclitis)
Appearance
Early on in the disease they are fine
With time, they appear crenated, glassy or pigmented
Granulomatous inflammation is associated with large yellow Kps [mutton fat KPs]
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KPs vary in size from flecks the size of cornea guttata to 1 mm in diameter

MANAGEMENT OF SYMPATHETIC OPHTHALMITIS

SIGNS
AC: SUN grading of AC cells and flare;
Performed by estimating the number of cells in a 1 mm by 1 mm slit beam field, employing
adequate light intensity and magnification and must be performed before pupillary dilatation,
which can lead to shedding of pigment cells into the aqueous
Grade

No. of Cells in AC

Grade

Description of AC Flare

<1

None

0.5+

1-5

1+

6-15

1+

Faint

2+

16-25

2+

Moderate (iris and lens details clear

3+

26-50

3+

Marked (Iris and lens details hazy)

4+

>50

4+

Intense (Fibrin or plastic aqueous)

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MANAGEMENT OF SYMPATHETIC OPHTHALMITIS

IRIS:
Nodules
Koeppe: These are situated at the pupillary border (Fig a)
Busacca: These are situated on the iris stroma away from the papillary border (Fig b)
Berlin: At the angles

Anterior and posterior synechiae, Heterochromia, Stromal atrophy, Rubeosis iridis

Fig a

Fig b

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MANAGEMENT OF SYMPATHETIC OPHTHALMITIS

PUPIL
Miosis (due to pupillary sphincter spasm and predisposes to the formation of posterior synechiae)
or Mydiasis
LENS
Cataract
VITREOUS:
Haze: SUN Working Group Grading of Vitreous haze below
Snowballs: whitish focal collections of inflammatory cells and exudate, usually most numerous
Grade
Vitreous Haze Severity
in the inferior vitreous
0

Good view of Nerve fibre layer (NFL)

+1

Clear disc and vessels but hazy NFL

+2

Disc and vessels hazy

+3

Only disc visible

+3

Disc not visible

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MANAGEMENT OF SYMPATHETIC OPHTHALMITIS

FUNDUS:
Multifocal choroidal infiltrates in the mid-periphery
Sub-RPE infiltrates (corresponding to DalenFuchs nodules seen on histology)
Exudative retinal detachment may occur in severe cases
Residual chorio-retinal scarring may cause visual loss when involving the macula. It may confer
a sunset glow appearance, see fig a (also seen in VKH)

Fig a

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MANAGEMENT OF SYMPATHETIC OPHTHALMITIS

DIFFERENTIAL DIAGNOSIS:
1. Vogt-Koyanagi-Haradas syndrome (VKH syndrome):
Also similar to SO with bilateral uveitis but commoner in patients of Japanese descent
However, it often has neurological (meningitis and rarely encephalopathy with cranial nerve paresis
and other focal lesions) and auditory manifestations (tinnitus, vertigo and deafness)
There is absence of a history of penetrating ocular trauma or intraocular surgery
They have serous retinal detachment and optic nerve involvement more frequently than do patients
with sympathetic ophthalmia

2. Intraocular lymphoma
Presents with vitreal cells and choroidal abnormality
If lymphoma is suspected, careful systemic workup, including neurological evaluation, should be
performed
If necessary, a vitreous sample must be obtained for diagnostic purpose and IL-10/IL-6 ratio should
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be found

MANAGEMENT OF SYMPATHETIC OPHTHALMITIS

INVESTIGATIONS
Elaborate lab investigations are usually avoided since it may not be reasonable to test for all causes
Lab Investigations
1. Haematological: FBC
2. Serology: HIV
3. Chemical Pathology: FBS, Fasting lipid profile, Liver and Renal Function Tests
4. Histopathology

There is granulomatous inflammation throughout the uveal tissue, except for the choriocapillaris
The inflammatory cells involved are primarily lymphocytes, epithelioid cells and multinucleated
giant cells
Dalen-Fuchs nodules, which are clumps of lymphocytes and epithelioid cells are found in
approximately one-third of enucleated eyes just behind the retinal pigment epithelium
The retinal pigment epithelium generally remains normal.
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MANAGEMENT OF SYMPATHETIC OPHTHALMITIS

INVESTIGATIONS
Imaging
1. OCT is useful for quantifying and monitoring change
2. B-scan ultrasonography may demonstrate choroidal thickening and exudative
retinal detachment
3. FA shows multiple foci of leakage at the level of the RPE, with sub-retinal
pooling in the presence of exudative retinal detachment. If, Dalen-Fuchs nodules
or vasculitis is present, lesions will fluoresce. Optic disc staining might be present in late
stages even in the absence of clinical papillitis
4. ICGA shows hypo-fluorescent spots in active disease, which resolve with
treatment
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MANAGEMENT OF SYMPATHETIC OPHTHALMITIS

TREATMENT
A. Enucleation (of a severely injured exciting eye)
Prompt enucleation of the injured eye is known to be preventative
In general, the time frame required for this approach is believed to be within 2 weeks from the
penetrating injury
The problem with this approach is that with current advanced surgical techniques, many eyes
once considered nonviable may now have a fair prognosis
Furthermore, the incidence of sympathetic ophthalmitis from penetrating injury is decreasing
The benefit of enucleation of the inciting eye, once the disease process is apparent in the
sympathizing eye, is more controversial
Evaluation of the literature revealed reports of varying success rates
The decision to enucleate a traumatized eye or an inciting eye should be made very cautiously
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Not infrequently, the inciting eye may ultimately become the eye with the better vision

MANAGEMENT OF SYMPATHETIC OPHTHALMITIS

B. Steroids are the basis of treatment
High dose oral prednisolone (12 mg/kg/day) is given for several months, and gradually tapered
according to response over 3 6 months
This may be preceded by intravenous methylprednisolone pulse therapy (5001000 mg/day)
Supplementary topical steroids (and cycloplegics) may be given to target anterior uveitis
Peri- and intraocular steroids, including slow-release intra-vitreal implants, may facilitate reduced
systemic treatment
C. Immuno-suppressives (in conjunction with steroids, for resistant cases or as steroid-sparing agents)
Azathioprine: 1 mg/kg/d orally initially; not to exceed 2.54 mg/kg/day
Ciclosporin (Cyclosporine): 2.55 mg/kg/day orally initially; not to exceed 10 mg/kg/day
Methotrexate: 7.512.5 mg/wk orally initially; not to exceed 25 mg/wk; folate (1 mg/d) is given
concurrently to minimize nausea
D. Biological blockers may be considered
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Infliximab, Adalimumab

MANAGEMENT OF SYMPATHETIC OPHTHALMITIS

PROGNOSIS
Prognosis depends on the severity and location of disease and the response to treatment
With aggressive therapy, 75% of sympathizing eyes retain a visual acuity of better than 6/60
Long-term follow-up is mandatory because relapses occur in 50% of cases, and may be delayed
for several years

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