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VITRITIS IN INDIAN POPULATION

Dr.Vishali Gupta , Dr.Reema Bansal , Dr.Amod Gupta

INTRODUCTION They are typically located inferiorly, but may also be


found superiorly or divided into multiple foci.
Vitritis, or more accurately known as vitreous humor
inflammation, is an important clinical entity. It is the Chronicity produces gliosis and collagen deposition
most consistent sign of intermediate uveitis (cyclitis) similar to that seen in preretinal membranes12,13.
and can also occur in association with certain non-
DIFFERENTIAL DIAGNOSIS OF VITRITIS
uveitis entities including intraocular lymphoma, trauma,
etc. The inflammation could be induced by an infectious Vitritis could be due to infectious or non-infectious
agent or trauma, but in majority the underlying etiologies. Certain non –uveitis conditions might
mechanisms appear to be autoimmune in nature. The present as vitritis and mimick chronic uveitis.These
term ‘Intermediate uveitis’ encompasses the are termed as ‘masquerade syndromes’.
inflammation of pars plana that could be of various
Infectious causes
etiologies including sarcoidosis, tuberculosis, etc. The
term ‘pars planitis’ is used when the intermediate 1. Tuberculosis
uveitis is idiopathic in nature.
2. Syphilis
CLINICAL PRESENTATION
3. Toxoplasmosis
Patients complain of floaters or blurry vision. Pain,
4. Toxocariasis
photophobia and redness are unusual. Children may
be more prone to develop anterior segment 5. Lyme disease
inflammation including posterior synechiae 1, 2 and band
6. Endogenous endophthalmitis
keratopathy 3, 4 Vitritis is the most consistent sign of
intermediate uveitis.The vitreous humor cellular 7. Parasitic infestations e.g.,cysticercosis
reaction has the appearance of “dust” at the slit lamp
Non-infectious causes
biomicroscope5.Vitritis may become so dense so as to
obscure the retina entirely6,7and cause profound loss 1. Sarcoidosis
of vision. These white cells are present in both the
anterior and posterior vitreous (diffuse clouding). 2. Behcet’s disease
Examination of posterior vitreous humor is necessary 3. Multiple sclerosis
to evaluate fully the degree of vitritis, as it is usually
most prominent posteriorly.Characteristic mobile, 4. Inflammatory bowel disease
round, white, focal vitreous humor opacities, dubbed 5. Fuch’s heterochromic uveitis
“oeufs de formi” (ant’s eggs) in French8, can be seen
in the inferior peripheral vitreous. They lie close to 6. Collagen vascular disease
the retina but are not in contact with it. They are not 7. Eales’ disease
specific for parsplanitis and may occur with any kind
of inflammation of the peripheral fundus or with an Masquerade syndromes
extensive and diffuse uveitis9.Later,the vitreous shows 1. CNS lymphoma
degenerative changes with fibre-like cylinderical
condensations of coarse vitreous strands10,11. Posterior 2. Other tumors e.g., Retinoblastoma, malignant
vitreous detachment is common. melanoma, medullloepithelioma

The hallmark of pars planitis are the white or yellowish- 3. Metastasis from breast, lung or renal carcinomas
white exudates (posterior hypopyon) and collagen into the eye
bands (snow banks) over the pars plana, with 4. Paraneoplastic syndromes
extension into the ora serrata and peripheral retina.
Following are some of the common entities:
44 Journal of the Bombay Ophthalmologists’ Association Vol. 13 No. 2

Intermediate Uveitis Cuffing of retinal vessels with inflammatory cells is a


common feature and there is occasionally obliterative
Intermediate uveitis is a common type of uveitis in
vasculitis. These changes are most common in the
children and young adults. It is one of the four major
terminal branches of retinal veins, and arterioles are
categories of uveitis in the classification scheme
rarely involved30. Neovascularisation of the disc (NVD),
proposed by the International Uveitis Study Group14.
of the retinal periphery (NVE), or of the iris (NVI) can
This classification scheme subdivides uveitis into
occur in most severe disease. NVD or NVE may lead
anterior, intermediate, posterior and panuveitis based
to vitreous hemorrhage and NVI to neovascular
on the principal anatomic site of inflammation. The
glaucoma.
diagnosis of intermediate uveitis is made when
intraocular inflammation primarily involves the Papillitis and optic nerve edema are uncommon.
vitreous, peripheral retina and pars plana ciliaris. Cystoid macular edema (CME) is common in pars
planitis, being the most common cause of decreased
The term intermediate uveitis has been designated
visual acuity31-34. Vitreous traction may be involved in
by the International Uveitis Study Group for the entities
the generation of CME. Cataract is common in
that have been previously called cyclitis, peripheral
protracted pars planitis. Retinal traction, retinal
uveitis, chronic cyclitis, vitritis and pars planitis 15-21.
detachment, subretinal exudation with serous retinal
Epidemiology detachment are rare.

In a large series from uveitis referral practices, Aetiology


intermediate uveitis represented 4-25% of
As in any case of uveitis, it is important to rule out
patients 22, 23-26. Pars planitis occurs in patients between
following causes:
the ages of 5-40 years and has a bimodal distribution
with a young group in the age range 5-15 years and Tuberculosis
an older group in the age range 20-40 years. There
Tuberculosis may affect any ocular
has been no striking sex predilection or familiar
structure.Tuberculous uveitis may appear as a chronic
tendency.
granulomatous iridocyclitis, peripheral uveitis,
In our population from North India, it constituted 198 disseminated choroiditis or panuveitis. The associated
of 1233(16.06%) patients of uveitis seen between iridocyclitis is usually granulomatous, characterized
January 1996 and June 2001.Out of these,181(91.4%) by mutton-fat KPs and extensive posterior
were idiopathic in nature, tuberculosis was the etiology synechiae.The presence of choroidal tubercles
in 8(4%) cases, sarcoidosis in 8(4%) and classically suggests hematogenous dissemination.
toxoplasmosis in 1(0.5%) case 27. Retinal periphlebitis in TB is usually a result of
extension from choroidal disease. An immune
Pathogenesis
mechanism has been proposed in the pathogenesis
The pathogenesis of intermediate uveitis largely is of retinal periphlebitis in patients with tuberculin
unknown. Autoimmune reactions against vitreous, hypersensitivity. The diagnosis is based on clinical
peripheral retina and ciliary body have been proposed. features with a positive tuberculin skin test, pulmonary
Several studies have looked for human leukocyte radiographic evidence of TB, PCR positivity from
antigen (HLA) associations in Intermediate Uveitis. intraocular fluids and a response to empiric anti-
Davis et al found associations with HLA – DR 2 and tuberculous therapy.
HLA – DQI28.
Toxoplasma Retinochoroiditis
Clinical features
Active toxoplasma lesions are round or oval, yellow-
Patients complain of floaters or blurred vision. white, adjacent to a pigmented old scar or satellite
lesions and are usually in the posterior retina.
Vitritis is the most consistent sign of intermediate
Peripheral lesions have been described, including a
uveitis. Presence of the characteristic pars plana
wide ring-like lesion near the extreme periphery,
exudates (snow balls) and snowbanking facilitates
resembling the snowbanking seen in pars planitis 35,36
diagnosis. Eyes with exudates have been considered
.Active toxoplasmosis may cause such a heavy vitreal
to be more severely involved29.
June 2004 45

reaction that the retinal lesion itself cannot be directly Vitritis as the primary manifestation of ocular syphilis
visualized (“headlight in the fog”).There may be in patients with HIV infection:
“spillover” anterior segment inflammation with small
HIV positive patients with syphilis may present with
to medium-sized ,round white or large, mutton-fat
atypically dense vitritis. In these patients, vitritis may
KPs in the cornea.It is usually acute in onset in contrast
be the first manifestation of syphilis. The regimen for
to the insidious onset in intermediate uveitis. Diffuse
neurosyphilis provides effective therapy. Moreover, in
or segmental vasculitis may be seen.
some patients, syphilitic vitritis may be the initial
Toxocariasis manifestation of HIV disease.

In chidren, toxocariasis is a major diagnostic Lyme Disease


consideration when dealing with unilateral intermediate
It may present with unilateral or bilateral uveitis which
uveitis 37.Patients with ocular toxocariasis are usually
usually resolves rapidly on treatment with antibiotics.
free of systemic findings. The characteristic unilateral
Rarely, it can cause intermediate uveitis. Serology for
chronic endophthalmitis with peripheral granuloma and
Borrelia burgdorferi should be performed if suspicion
tractional bands extending from the disc to the
is high based on exposure or review of systems which
posterior pole is distinct from intermediate uveitis.
include erythema migrans like skin rash, cardiac
History of infected puppies or pica and serological
conduction defects, and cranial nerve palsies.
testing (ELISA) for toxocara antigen are extremely
helpful. Endogenous endophthalmitis

Sarcoidosis Endogenuos infections, bacterial or fungal, reach the


eye via the bloodstream, usually as part of a
Sarcoidosis should be suspected in all patients with
disseminated infection. The infective element initiates
intermediate uveitis. Unlike pars planitis, sacoid uveitis
a focal choroiditis, retinitis or cyclitis. When this initial
is slightly more common in females, with an older
focus of infection extends into the vitreous, it produces
age of presentation 38 .Chest radiograph, serum ACE
inflammation and involves all structures of the eye,
levels, serum lysozyme and gallium scans support the
producing endogenous endophthalmitis. Vitreous
diagnosis, but only tissue biopsy (transbronchial lung
inflammation may be so severe that it obscures view
biopsy) confirms it.
of the fundus and makes clinical diagnoses difficult
Behcet’s Disease .Sudden onset and a compatible history are helpful in
making a correct diagnosis 45.
Characterized mainly by its retinal vascular
involvement, Behcet’s disease has usually bilateral Cysticercosis
ocular involvement with recurrent episodes of uveitis
Although any structure within or around the eye can
39
.An anterior uveitis is frequent with an associated
be involved in ocular cysticercosis, the vitreous and
hypopyon. An isolated vitritis is not characteristic but
retina are most likely affected46.The parasite may be
is significant .HLA typing(HLA-B 51),a compatible
present within the vitreous or in the subretinal space
history and systemic evaluation are helpful. 47
.Vitritis is usually severe in ocular cysticercosis.
Syphilis
Multiple Sclerosis
Anterior uveitis is the most common finding of
Patients with multiple sclerosis may develop ocular
secondary syphilis 40.The pathogenesis of uveitis
disease resembling other forms of intermediate uveitis.
appears to be related to both infectious and
Retinal periphlebitis and vitritis have been reported to
immunogenic components. Posterior uveitis can occur
occur in 5-20% of patients of multiple sclerosis 48
as diffuse or localized choroiditis or chorioretinitis 41
.Breger suggested an autoimmune disorder directed
,most often affecting the posterior pole 42 and
against an unknown antigen, common to both eye and
juxtapapillary area 43 .Progressive posterior uveitis can
myelin nerve sheath 49.Multiple sclerosis does not have
lead to disc edema, vitritis, retinal vasculitis, uveal
to be active for intermediate uveitis to be present.
effusion and exudative retinal detachment 44 .Late
Intermediate uveitis may precede multiple sclerosis
syphilis may present with anterior or posterior uveitis.
by more than 7 years 50 .A subset of intermediate
46 Journal of the Bombay Ophthalmologists’ Association Vol. 13 No. 2

uveitis patients may be at a risk for the subsequent detect the primary cancer. Vitreous biopsy may reveal
development of multiple sclerosis 51. the presence of malignant cells.

Fuch’s Heterochromic cyclitis Paraneoplastic Syndromes:

It can produce a unilateral, chronic, low grade anterior Cancer-associated retinopathy (CAR) may manifest
segment inflammation with spillover of the cells into as uveitis. Histologically, there is destruction of
the vitreous cavity. There is no snowbank. Multiple photoreceptors 54 which is thought to be immune-
stellate keratic precipitates and heterochromia iridis mediated. Visual loss may be explained by optic disc
constitute this diagnosis. Posterior synechiae preclude pallor, vascular sheathing and RPE disturbances.
the diagnosis. Vitritis may be significant to cause CME.
Bilateral Diffuse Uveal Melanocytic Proliferation
Transient Vitreous Inflammatory Reactions associated (BDUMP) is another paraneoplastic disorder simulating
with combination antiretroviral therapy in patients with as uveitis, found in association with a systemic
AIDS and Cytomegalovirus Retinitis malignant process. Multiple, round, raised, subretinal
red patches with or without exudative retinal
Patients with AIDS and CMV Retinitis may develop
detachment and vitreous cells can be seen.
transient intraocular inflammation associated with
combination antiretroviral therapy.This inflammation LABORATORY INVESTIGATIONS
reflects an improved immune response against
The diagnosis of intermediate uveitis is clinical. Routine
cytomegalovirus.
tests should include a complete blood count to look
Masquerade Syndromes for WBC abnormalities (as in a malignant masquerade
syndrome), erythrocyte sedimentation rate, a
These comprise a group of disorders that occur with
tuberculin skin test, chest radiograph to screen for
intraocular inflammation and are often misdiagnosed
tuberculosis and sarcoidosis, and TPHA for syphilis.
as chronic idiopathic uveitis. Apart from certain non-
Fluorecein angiography is useful for retinal or
malignant conditions masquerading as uveitis, many
choroidal pathologies, subtle CME and capillary non-
of the masquerade syndromes are malignant
perfusion.
processes.
Serological tests (ELISA) may be indicated for
Intraocular Lymphoma
toxoplasmosis, toxocariasis, HIV and Lyme disease.
Vitritis without fundus lesions has been reported with Aqueous tap and Vitreous biopsy may be required for
intraocular lymphoma 52,53 .Elderly patients presenting cytological or microbiological evaluation and
with vitreous cells may be indicative of intraocular Polymerase chain reaction. In suspected cases of
lymphoma. Diagnostic vitrectomy, cytological endogenous endophthalmitis, blood culture and urine
evaluation of CSF and neuroimaging are necessary culture may prove contributory. MRI may be required
for establishing diagnosis. to look for multiple sclerosis. Cranial imaging with
lumbar puncture are indicated to rule out large cell
Other malignant conditions
lymphoma.
Tumors such as retinoblastoma, malignant melanoma
TREATMENT
or medulloepithelioma can disseminate into the
vitreous and simulate intermediate uveitis. Atypical The goal of the treatment is to ameliorate vision
vitreous cells and a mass, detectable by fundoscopy threatening complications secondary to intermediate
or ultrasonography constitute these diagnosis .In uveitis like CME, cataract, glaucoma and exudative
children. apart from retinoblastoma and retinal detachment.
medulloepithelioma, acute mylogenous leukemia and
If a specific cause of intermediate uveitis is diagnosed
juvenile xanthogranuloma can masquerade as uveitis.
(such as tuberculosis, sarcoidosis, toxoplasmosis,
Breast, lung and renal carcinomas can metastasize to syphilis),then the treatment is directed against the
the choroid and produce signs and symptoms of uveitis. particular disorder and a relevant medical consultation
Such patients need a complete medical evaluation to is sought for systemic involvement.
June 2004 47

The mainstay of the treatment of intermediate uveitis total of 18 months. Oral steroids are tapered over a
is periocular corticosteroid injections or oral steroids. period of 6-8 weeks. ATT, in our experience, reduces
Posterior sub-tenon Triamcinolone Acetonide injection the recurrence rate.
may be given every 6-8 weeks until resolution of CME
The “classic” antimicrobial therapy for active ocular
or return of 20/20 visual acuity. Giving the injection
toxoplasmosis consists of a combination of
superotemporally in the sub-Tenon space as far
pyrimethamine, sulphadiazine and corticosteroids with
posteriorly and close to the globe as possible, results
Folic acid supplementation. Initiation of oral
in a deposit of the drug closer to the macula. Posterior
corticosteroid therapy is delayed for 24-48 hours after
injection also reduces the risk of intraocular pressure
starting antimicrobial agents to allow adequate blood
rise.
levels of antimicrobials 55.The other approach is to
When a series of periocular injections have failed, use Clindamycin in a dose of 300 mg QID as the sole
oral Prednisolone in the dose of 1.0 mg/kg daily are antimicrobial agent in combination with corticosteroids
given and tapered over 6-12 weeks, depending upon 56
,started a day before oral corticosteroids and
the response .All possible complications of continued for a few days after tapering off steroids
corticosteroids should be looked for on follow-ups. for a total period of 6 weeks. Clindamycin appears to
Topical betamethasone and cycloplegics are given for be concentrated in ocular tissue and can penetrate
associated anterior segment inflammation. tissue cyst walls.

Severe, bilateral, uncontrolled cases of vitritis and Penicillin remains the treatment of choice for syphilis
patients who are intolerant to corticosteroids may 57
.Patients with acive syphilitic uveitis are treated as
require the use of immunosuppressive agents. for neurosyphilis-Penicilllin G Sodium 2-4 million units
Cyclophosphamides, Methotrexate, Azathioprine, every 4 hours intravenous for 2 weeks after a negative
Chlorambucil and Cyclosporin A have proven to be skin test.
effective. But these agents have serious systemic side
Core Vitrectomy with intravitreal antibiotics
effects which should be monitored by an experienced
(antibacterial or anti fungal) along with administration
physician. Very severe cases may need Combination
of intravenous antibiotics is recommended for
therapy (Prednisolone plus immunosuppressives, or
endogenous endophthalmitis.
two or three different immunosuppressives).
Other specific indications for vitrectomy include
Surgical management
intravitreal or subretinal cysticercosis, ocular
Pars plana vitrectomy may be indicated for diagnostic toxocariasis for granuloma excision and tractional
as well as therapeutic purpose to remove vitreous retinal detachment.
antigens and inflammatory cells and mediators, playing
PROGNOSIS
an important role in CME.
The visual prognosis depends upon status of the macula
Other indications of vitrectomy, although rare, include
and vitreous opacification in the early course of the
vitreous opacification, cataract, tractional retinal
disease. Cataract, secondary glaucoma and tractional
detachment, epiretinal membranes and vitreous
retinal detachment affect the visual outcome in later
hemorrhage.
stages.
Specific causes
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Contact Details
Department of Ophthalmology
Post graduate Institute of
Medical Education and Research
Chandigarh.
June 2004 51

Fig.1. A 26-years old male with a “headlight in the fog” Fig.2. An 18-years old male with endogenous endophthalmitis
appearance OD.ELISA was positive for IgM in the vitreous OS.Pars plana vitrectomy was done and intravitreal
and serum.Treated with Clindamycin and oral antibiotics given.Vision improved from 6/36 to 6/6.
corticosteroids.vision improved from 6/60 to 6/12.

Fig.3 . A 50-years old male with severe vitritis OD.Vision was 4/60.Routine investigations were normal .
Vitreous biopsy revealed B- cell Lymphoma.MRI ruled out CNS lymphoma.Pars plana vitrectomy
was done and intravitreal methotrexate given.Vision improved to 6/12.

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