INTERMEDIATE UVEITIS (PARS PLANITIS)

It denotes inflammation of pars plana part of ciliary
body and most peripheral part of the retina.
Etiology. It is an idiopathic disease usually affecting
both eyes (80 percent) of children and young adults.
Pars planitis is a rather common entity, constituting 8
percent of uveitis patients.
Clinical features. Symptoms. Most of the patients
present with history of floaters. Some patients may
come with defective vision due to associated cystoid
macular oedema.
Signs. The eye is usually quiet. Slit-lamp examination
may show: mild aqueous flare, and fine KPs at the back
of cornea. Anterior vitreous may show cells. Fundus
examination with indirect ophthalmoscope reveals the
whitish exudates present near the ora serrata in the
inferior quadrant. These typical exudates are referred
as snow ball opacities. These may coalesce to form a
grey white plaque called snow banking.
Complications of long-standing pars planitis include:
cystoid macular oedema, complicated cataract and
tractional retinal detachment.
Treatment
1. Corticosteroids administered systemically and as
repeated periocular injections may be effective in
some cases.
2. Immunosuppressive drugs may be helpful in
steroid resistant cases.
3. Peripheral cryotherapy is also reported to be
effective.

TOXOCARIASIS

It is an infestation caused by an intestinal round worm
of dogs (Toxocara canis) and cats (Toxocara catis).
The young children who play with dogs and cats or
eat dirt are infested by ova of these worms. These
ova develop into larva in the human gut, and then
produce the condition visceral larva migrans (VLM).
Ocular toxocariasis. It is ocular infestation by these
larva and is almost always unilateral. Clinically it can
present as follows:
1. Toxocara chronic endophthalmitis. It usually
presents with leucocoria due to marked vitreous
clouding. The condition is seen in children
between the age of 2-10 years and mimics
retinoblastoma.
2. Posterior pole granuloma. It presents as a
yellow-white, round, solitary, raised nodule, about
1-2 disc diameter in size, located either at the
macula or in the centrocaecal area. The condition
is usually seen in children between 5 and 15
years of age, presenting with unilateral loss of
vision.
3. Peripheral granuloma. It is situated anterior to
the equator and may be associated with vitreous
band formation. It may present from 6 to 40 years
of age.
Diagnosis is made on the basis of clinical picture and
ELISA blood test.
Treatment. It consists of periocular (posterior sub-
Tenon) injection of steroid and systemic steroids. Pars
plana vitrectomy may be required in unresponsive
patients with endophthalmitis and in patients with
vitreous band formation.

VOGT-KOYANAGI-HARADA (VKH) SYNDROME

It is an idiopathic multisystem disorder which includes
cutaneous, neurological and ocular lesions. The
disease is comparatively more common in Japanese
who are usually positive for HLA-DR4 and DW15.
Clinical features
1. Cutaneous lesions include: alopecia, poliosis and
vitiligo.
2. Neurological lesions are in the form of meningism,
encephalopathy, tinnitus, vertigo and deafness.
3. Ocular features are bilateral chronic granulomatous
anterior uveitis, posterior uveitis and
exudative retinal detachment.
Treatment. It comprises steroids administered
topically, periocularly and systemically.

PANOPHTHALMITIS

It is an intense purulent inflammation of the whole
eyeball including the Tenon’s capsule. The disease
usually begins either as purulent anterior or purulent
posterior uveitis; and soon a full-fledged picture of
panophthalmitis develops, following through a very
short stage of endophthalmitis.
Etiology
Panophthalmitis is an acute bacterial infection.
Mode of infection and causative organisms are
same as described for infective bacterial
endophthalmitis (page 150, 151).
Clinical picture
Symptoms. These include:
Severe ocular pain and headache,
Complete loss of vision,
Profuse watering,
Purulent discharge,
Marked redness and swelling of the eyes, and
Associated constitutional symptoms are malaise
and fever.
Signs are as follows (Fig.7.20):
1. Lids show a marked oedema and hyperaemia.
2. Eyeball is slightly proptosed, ocular movements
are limited and painful.

3. Conjunctiva shows marked chemosis and ciliary
as well as conjunctival congestion.
4. Cornea is cloudy and oedematous.
5. Anterior chamber is full of pus.
6. Vision is completely lost and perception of light
is absent.
7. Intraocular pressure is markedly raised.
8. Globe perforation may occur at limbus, pus comes
out and intraocular pressure falls.
Complications include:
Orbital cellulitis
Cavernous sinus thrombosis
Meningitis or encephalitis
Treatment
There is little hope of saving such an eye and the
pain and toxaemia lend an urgency to its removal.
1. Anti-inflammatory and analgesics should be
started immediately to relieve pain.
2. Broad spectrum antibiotics should be
administered to prevent further spread of infection
in the surrounding structures.

3. Evisceration operation should be performed to
avoid the risk of intracranial dissemination of
infection.

Endophthalmitis

Endophthalmitis is defined as an inflammation of the
inner structures of the eyeball i.e., uveal tissue and
retina associated with pouring of exudates in the
vitreous cavity, anterior chamber and posterior
chamber.
Etiology
Etiologically endophthalmitis may be infectious or
non-infectious (sterile).
A. Infective endophthalmitis
Modes of Infection
1. Exogenous infections. Purulent inflammations are
generally caused by exogenous infections
following perforating injuries, perforation of
infected corneal ulcers or as postoperative
infections following intraocular operations.

2. Endogenous or metastatic endophthalmitis. It
may occur rarely through blood stream from some
infected focus in the body such as caries teeth,
generalised septicaemia and puerperal sepsis.
3. Secondary infections from surrounding
structures. It is very rare. However, cases of
purulent intraocular inflammation have been
reported following extension of infection from
orbital cellulitis, thrombophlebitis and infected
corneal ulcers.
Causative organisms
1. Bacterial endophthalmitis. The most frequent
pathogens causing acute bacterial endophthalmitis
are gram positive cocci i.e., staphylococcus
epidermidis and staphylococcus aureus. Other
causative bacteria include streptococci, pseudomonas,
pneumococci and corynebacterium.
Propionio bacterium acnes and actinomyces are
gram-positive organisms capable of producing
slow grade endophthalmitis.
2. Fungal endophthalmitis is comparatively rare. It
is caused by aspergillus, fusarium, candida etc.
B. Non-infective (sterile) endophthalmitis
Sterile endophthalmitis refers to inflammation of inner
structures of eyeball caused by certain toxins/toxic
substances. It occurs in following situations.
1. Postoperative sterile endophthalmitis may occur
as toxic reaction to:
Chemicals adherent to intraocular lens (IOL) or
Chemicals adherent to instruments.
2. Post-traumatic sterile endophthalmitis may occur
as toxic reaction to retained intraocular foreign
body, e.g., pure copper.
3. Intraocular tumour necrosis may present as
sterile endophthalmitis (masquerade syndrome).
4. Phacoanaphylactic endophthalmitis may be
induced by lens proteins in patients with
Morgagnian cataract.
Note: Since postoperative acute bacterial
endophthalmitis is most important, so clinical features
and treatment described below pertain to this
condition.
Clinical picture of acute bacterial endophthalmitis
Acute postoperative endophthalmitis is a
catastrophic complication of intraocular surgery with
an incidence of about 0.1%. Source of infection in
most of the cases is thought to be patient’s own
periocular bacterial flora of the eyelids, conjunctiva,
and lacrimal sac. Other potential sources of infection
include contaminated solutions and instruments, and
environmental flora including that of surgeon and
operating room personnel.
Symptoms. Acute bacterial endophthalmitis usually
occurs within 7 days of operation and is characterized
by severe ocular pain, redness, lacrimation,
photophobia and marked loss of vision.
Signs are as follows (Fig. 7.18):
1. Lids become red and swollen.
2. Conjunctiva shows chemosis and marked
circumcorneal congestion.
Note: Conjunctival congestion, corneal oedema,
hypopyon and yellowish white exudates in the
vitreous seen in the pupillary area behind the IOL.
3. Cornea is oedematous, cloudy and ring infiltration
may be formed.
4. Edges of wound become yellow and necrotic and
wound may gape (Fig. 7.19) in exogenous form.
5. Anterior chamber shows hypopyon; soon it
becomes full of pus.
6. Iris, when visible, is oedematous and muddy.
7. Pupil shows yellow reflex due to purulent
exudation in vitreous. When anterior chamber
becomes full of pus, iris and pupil details are not
seen.
8. Vitreous exudation. In metastatic forms and in
cases with deep infections, vitreous cavity is
filled with exudation and pus. Soon a yellowish
white mass is seen through fixed dilated pupil.
This sign is called amaurotic cat’s-eye reflex.
9. Intraocular pressure is raised in early stages, but
in severe cases, the ciliary processes are
destroyed, and a fall in intraocular pressure may
ultimately result in shrinkage of the globe.

Treatment
An early diagnosis and vigorous therapy is the
hallmark of the treatment of endophthalmitis.
Following therapeutic regime is recommended for
suspected bacterial endophthalmitis.
A. Antibiotic therapy
1. Intravitreal antibiotics and diagnostic tap should
be made as early as possible. It is performed
transconjunctivally under topical anaesthesia from
the area of pars plana (4-5 mm from the limbus). The
vitreous tap is made using 23-gauge needle followed
by the intravitreal injection using a disposable
tuberculin syringe and 30-gauge needle.
The main stay of treatment of acute bacterial
endophthalmitis is intravitreal injection of antibiotics
at the earliest possible. Usually a combination of two
antibiotics – one effective against gram positive
coagulase negative staphylococci and the other
against gram-negative bacilli is used as below :
First choice: Vancomycin 1 mg in 0.1 ml plus
ceftazidime 2.25 mg in 0.1 ml.
Second choice: Vancomycin 1 mg in 0.1 ml plus
Amikacin 0.4 mg in 0.1 ml.
Third choice: Vancomycin 1 mg in 0.1 ml plus
gentamycin 0.2 mg in 0.1 ml.
Note:
Some surgeons prefer to add dexamethasone 0.4
mg in 0.1 ml to limit post-inflammatory consequences.
Gentamycin is 4 times more retinotoxic (causes
macular infarction) than amikacin. Preferably the
aminoglycosides should be avoided.
The aspirated fluid sample should be used for
bacterial culture and smear examination. If vitreous
aspirate is collected in an emergency when
immediate facilities for culture are not available, it
should be stored promptly in refrigerator at 4°C.
If there is no improvement, a repeat intravitreal
injection should be given after 48 hours taking
into consideration the reports of bacteriological
examination.
2. Subconjunctival injections of antibiotics should
be given daily for 5-7 days to maintain therapeutic
intraocular concentration :
First choice : Vancomycin 25 mg in 0.5 ml plus.
Ceftazidime 100 mg in 0.5 ml
Second choice : Vancomycin 25 mg in 0.5 ml plus
Cefuroxime 125 mg in 0.5 ml
3. Topical concentrated antibiotics should be
started immediately and used frequently (every 30
minute to 1 hourly). To begin with a combination of
two drugs should be preferred, one having a
predominant effect on the gram-positive organisms
and the other against gram-negative organisms as
below:
Vancomycin (50 mg/ml) or cefazoline (50mg/ml)
plus.
Amikacin (20 mg/ml) or tobramycin (15 mg%).
4. Systemic antibiotics have limited role in the
management of endophthalmitis, but most of the
surgeons do use them.
Ciprofloxacin intravenous infusion 200 mg BD
for 3-4 days followed by orally 500 mg BD for
6-7 days, or
Vancomycin 1 gm IV BD and ceftazidime 2 g IV
8 hourly, or
Cefazoline 1.5 gm IV 6 hourly and amikacin 1 gm
IV three times a day.
B. Steroid therapy
Steroids limit the tissue damage caused by
inflammatory process. Most surgeons recommend
their use after 24 to 48 hours of control of infection
by intensive antibiotic therapy. However, some
surgeons recommend their immediate use

(controversial). Routes of administration and doses
are:
Intravitreal injection of dexamethasone 0.4 mg in
0.1ml.
Subconjunctival injection of dexamethasone 4
mg (1ml) OD for 5-7 days.
Topical dexamethasone (0.1%) or predacetate
(1%) used frequently.
Systemic steroids. Oral corticosteroids should
preferably be started after 24 hours of intensive
antibiotic therapy. A daily therapy regime with 60
mg prednisolone to be followed by 50, 40, 30, 20
and 10 mg for 2 days each may be adopted.
C. Supportive therapy
1. Cycloplegics. Preferably 1% atropine or
alternatively 2% homatropine eyedrops should
be instilled TDS or QID.
2. Antiglaucoma drugs.In patients with raised
intraocular pressure drugs such a oral
acetazolamide (250 mg TDS) and timolol (0.5%
BD) may be prescribed.
D. Vitrectomy operation should be performed if the
patient does not improve with the above intensive
therapy for 48 to 72 hours or when the patient presents
with severe infection with visual acuity reduced to
light perception. Vitrectomy helps in removal of
infecting organisms, toxins and enzymes present in
the infected vitreous mass.

ANTERIOR UVEITIS (IRIDOCYCLITIS)

CLINICAL FEATURES
Though anterior uveitis, almost always presents as a
combined inflammation of iris and ciliary body
(iridocyclitis), the reaction may be more marked in iris
(iritis) or ciliary body (cyclitis). Clinically it may
present as acute or chronic anterior uveitis. Main
symptoms of acute anterior uveitis are pain,
photophobia, redness, lacrimation and decreased
vision. In chronic uveitis, however the eye may be
white with minimal symptoms even in the presence of
signs of severe inflammation.
Symptoms
1. Pain. It is dominating symptom of acute anterior
uveitis. Patients usually complain of a dull aching
throbbing sensation which is typically worse at night.
The ocular pain is usually referred along the
distribution of branches of fifth nerve, especially
towards forehead and scalp.
2. Redness. It is due to circumcorneal congestion,
which occurs as a result of active hyperaemia of
anterior ciliary vessels due to the effect of toxins,
histamine and histamine-like substances and axon
reflex.
3. Photophobia and blepharospasm observed in
patients with acute anterior uveitis are due to a reflex
between sensory fibres of fifth nerve (which are
irritated) and motor fibres of the seventh nerve,
supplying the orbicularis oculi muscle.
4. Lacrimation occurs as a result of lacrimatory reflex
mediated by fifth nerve (afferent) and secretomotor
fibres of the seventh nerve (efferent).
5. Defective vision in a patient with iridocyclitis may
vary from a slight blur in early phase to marked
deterioration in late phase. Factors responsible for
visual disturbance include induced myopia due to
ciliary spasm, corneal haze (due to oedema and KPs),
aqueous turbidity, pupillary block due to exudates,
complicated cataract, vitreous haze, cyclitic
membrane, associated macular oedema, papillitis or
secondary glaucoma. One or more factors may
contribute in different cases depending upon the
severity and duration of the disease.
Signs
Slit lamp biomicroscopic examination is essential to
elicit most of the signs of uveitis (Fig. 7.8).
I. Lid oedema usually mild, may accompany a severe
attack of acute anterior uveitis.
II. Circumcorneal congestion is marked in acute
iridocyclitis and minimal in chronic iridocyclitis. It
must be differentiated from superficial congestion
occurring in acute conjunctivitis.
III. Corneal signs include; corneal oedema, KPs
and posterior corneal opacities.

1. Corneal oedema is due to toxic endothelitis and
raised intraocular pressure when present.
2. Keratic precipitates (KPs) are proteinaceouscellular
deposits occurring at the back of cornea.
Mostly, these are arranged in a triangular fashion
occupying the centre and inferior part of cornea due
to convection currents in the aqueous humour (Fig.
7.9). The composition and morphology of KPs varies
with the severity, duration and type of uveitis.
Following types of KPs may be seen:
i. Mutton fat KPs. These typically occur in
granulomatous iridocyclitis and are composed of
epithelioid cells and macrophages. They are large,
thick, fluffy, lardaceous KPs, having a greasy or
waxy appearance. Mutton fat KPs are usually a
few (10 to 15) in number (Fig. 7.9B).
ii. Small and medium KPs (granular KPs). These are
pathognomic of non-granulomatous uveitis and
are composed of lymphocytes. These small,
discrete, dirty white KPs are arranged irregularly
at the back of cornea. Small KPs may be hundreds
in number and form the so called endothelial
dusting.

iii. Red KPs. These are formed when in addition to
inflammatory cells, RBCs also take part in
composition. They may be seen in haemorrhagic
uveitis.
iv. Old KPs. These are sign of healed uveitis. Either
of the above described KPs with healing process
shrink, fade, become pigmented and irregular in
shape (crenated margins). Old mutton fat KPs
usually have a ground glass appearance due to
hyalinization.
3. Posterior corneal opacity may be formed in longstanding
cases of iridocyclitis.
IV. Anterior chamber signs
1. Aqueous cells. It is an early feature of iridocyclitis.
The cells should be counted in an oblique slit-lamp
beam, 3-mm long and 1-mm wide, with maximal light
intensity and magnification, and graded as :
– = 0 cells,
± = 1–5 cells,
+1 = 6–10 cells,
+2 = 11-20 cells,
+3 = 21–50 cells, and
+4 = over 50 cells
2. Aqueous flare. It is due to leakage of protein
particles into the aqueous humour from damaged
blood vessels. It is demonstrated on the slit lamp
examination by a point beam of light passed obliquely
to the plane of iris (Fig. 7.10). In the beam of light,
protein particles are seen as suspended and moving
dust particles. This is based on the ‘Brownian
movements’ or ‘Tyndal phenomenon’. Aqueous flare
is usually marked in nongranulomatous and minimal
in granulomatous uveitis. The flare is graded from ‘0’
to +4. Grade :
0 = no aqueous flare,
+1 = just detectable;
+2 = moderate flare with clear iris details;
+3 = marked flare (iris details not clear);
+4 = intense flare (fixed coagulated aqueous
with considerable fibrin).
3. Hypopyon. When exudates are heavy and thick,
they settle down in lower part of the anterior chamber
as hypopyon (sterile pus in the anterior chamber)
(Fig. 7.11).
4. Hyphaema (blood in the anterior chamber): It may
be seen in haemorrhagic type of uveitis.

5. Changes in depth and shape of anterior chamber
may occur due to synechiae formation.
6. Changes in the angle of anterior chamber are
observed with gonioscopic examination. In active
stage, cellular deposits and in chronic stage peripheral
anterior synechiae may be seen.
V. Iris signs
1. Loss of normal pattern. It occurs due to oedema
and waterlogging of iris in active phase and due to
atrophic changes in chronic phase. Iris atrophy is
typically observed in Fuchs’ heterochromic
iridocyclitis.
2. Changes in iris colour. Iris usually becomes muddy
in colour during active phase and may show
hyperpigmented and depigmented areas in healed
stage.
3. Iris nodules (Fig. 7.12). These occur typically in
granulomatous uveitis.

Koeppe’s nodules are situated at the pupillary
border and may initiate posterior synechia.
Busacca’s nodules situated near the collarette
are large but less common than the Koeppe’s
nodules.
4. Posterior synechiae. These are adhesions between
the posterior surface of iris and anterior capsule of
crystalline lens (or any other structure which may be
artificial lens, after cataract, posterior capsule (left
after extracapsular cataract extraction) or anterior
hyaloid face. These are formed due to organisation
of the fibrin-rich exudates. Morphologically, posterior
synechiae may be segmental, annular or total.
i. Segmental posterior synechiae refers to adhesions
of iris to the lens at some points (Fig. 7.8).
ii. Annular posterior synechiae (ring synechiae are
360o adhesions of pupillary margin to anterior
capsule of lens. These prevent the circulation of
aqueous humour from posterior chamber to
anterior chamber (seclusio pupillae). Thus, the
aqueous collects behind the iris and pushes it
anteriorly (leading to ‘iris-bombe’ formation) (Fig.
7.13). This is usually followed by a rise in
intraocular pressure.
iii. Total posterior synechiae due to plastering of
total posterior surface of iris with the anterior
capsule of lens are rarely formed in acute plastic
type of uveitis. These result in deepening of
anterior chamber (Fig. 7.14).
5. Neovascularsation of iris (rubeosis iridis)
develops in some eyes with chronic iridocyclitis.

VI. Pupillary signs
1. Narrow pupil. It occurs in acute attack of
iridocyclitis (Fig. 7.8B) due to irritation of sphincter
pupillae by toxins. Iris oedema and engorged radial
vessels of iris also contribute in making the pupil
narrow.
2. Irregular pupil shape. It results from segmental
posterior synechiae formation. Dilatation of pupil with
atropine at this stage results in festooned pupil
(Fig. 7.8A and Fig. 7.15).
3. Ectropion pupillae (evertion of pupillary margin).
It may develop due to contraction of fibrinous exudate
on the anterior surface of the iris.

4. Pupillary reaction becomes sluggish or may even
be absent due to oedema and hyperaemia of iris which
hamper its movements.
5. Occlusio pupillae results when the pupil is
completely occluded due to organisation of the
exudates across the entire pupillary area.
VII. Changes in the lens
1. Pigment dispersal on the anterior capsule of lens
is almost of universal occurrence in a case of anterior
uveitis.
2. Exudates may be deposited on the lens in cases
with acute plastic iridocyclitis.
3. Complicated cataract may develop as a
complication of persistent iridocyclitis. Typical
features of a complicated cataract in early stage are
‘polychromatic luster’ and ‘bread-crumb’ appearance
of the early posterior subcapsular opacities. In the
presence of posterior synechiae, the complicated
cataract progresses rapidly to maturity (Fig. 7.15).
VIII. Change in the vitreous
Anterior vitreous may show exudates and
inflammatory cells after an attack of acute iridocyclitis.
COMPLICATIONS AND SEQUELAE
1. Complicated cataract. It is a common
complication of iridocyclitis as described above.
2. Secondary glaucoma. It may occur as an early or
late complication of iridocyclitis.
i. Early glaucoma. In active phase of the disease,
presence of exudates and inflammatory cells in
the anterior chamber may cause clogging of
trabecular meshwork resulting in the decreased
aqueous drainage and thus a rise in intraocular
pressure (hypertensive uveitis).
ii. Late glaucoma in iridocyclitis (postinflammatory
glaucoma) is the result of pupil
block (seclusio pupillae due to ring synechiae
formation, or occlusio pupillae due to organised
exudates) not allowing the aqueous to flow
from posterior to anterior chamber. There may
or may not be associated peripheral anterior
synechiae formation.
3. Cyclitic membrane. It results due to fibrosis of
exudates present behind the lens. It is a late
complication of acute plastic type of iridocyclitis.
4. Choroiditis. It may develop in prolonged cases
of iridocyclitis owing to their continuity.

5. Retinal complications. These include cystoid
macular oedema, macular degeneration, exudative
retinal detachment and secondary periphlebitis
retinae.
6. Papillitis (inflammation of the optic disc). It may
be associated in severe cases of iridocyclitis.
7. Band-shaped keratopathy. It occurs as a
complication of long-standing chronic uveitis (Fig.
5.17), especially in children having Still’s disease.
8. Phthisis bulbi. It is the final stage end result of
any form of chronic uveitis. In this condition,
ciliary body is disorganised and so aqueous
production is hampered. As a result of it the eye
becomes soft, shrinks and eventually becomes a
small atrophic globe (phthisis bulbi).
DIFFERENTIAL DIAGNOSIS
1. Acute red eye. Acute iridocyclitis must be
differentiated from other causes of acute red eye,
especially acute congestive glaucoma and acute
conjunctivitis. The differentiating features are
summarised in Table 7.1.
2. Granulomatous versus non-granulomatous
uveitis. Once diagnosis of iridocyclitis is established,
an attempt should be made to know whether the
condition is of granulomatous or non-granulomatous
type. The main clinical differences between the two
are summarised in Table 7.2.
3. Etiological differential diagnosis. Efforts should
also be made to distinguish between the different
etiological varieties of iridocyclitis. This may be
possible in some cases after thorough investigations
and with a knowledge of special features of different
clinical entities, which are described under the subject
of ‘special types of iridocyclitis’ (see page 154).
INVESTIGATIONS
These include a battery of tests because of its varied
etiology. However, an experienced ophthalmologist
soon learns to order a few investigations of
considerable value, which will differ in individual case
depending upon the information gained from
thorough clinical work up. A few common
investigations required are listed here:
1. Haematological investigations
TLC and DLC to have a general information
about inflammatory response of body.
ESR to ascertain existence of any chronic
inflammatory condition in the body.
Blood sugar levels to rule out diabetes mellitus.
Blood uric acid in patients suspected of having
gout.
Serological tests for syphilis, toxoplasmosis,
and histoplasmosis.
Tests for antinuclear antibodies, Rh factor,
LE cells, C-reactive proteins and antistreptolysin-
0.
2. Urine examination for WBCs, pus cells, RBC
and culture to rule out urinary tract infections.
3. Stool examination for cyst and ova to rule out
parasitic infestations.
4. Radiological investigations include X-rays of
chest, paranasal sinuses, sacroiliac joints and
lumbar spine.
5. Skin tests. These include tuberculin test, Kveim’s
test and toxoplasmin test.
TREATMENT OF IRIDOCYCLITIS
I. Non-specific treatment
(a) Local therapy
1. Mydriatic-cycloplegic drugs. These are very
useful and most effective during acute phase of
iridocyclitis. Commonly used drug is 1 percent
atropine sulfate eye ointment or drops instilled 2-3
times a day. In case of atropine allergy, other
cycloplegics like 2 percent homatropine or 1 percent
cyclopentolate eyedrops may be instilled 3-4 times a
day. Alternatively for more powerful cycloplegic effect
a subconjunctival injection of 0.25 ml mydricain (a
mixture of atropine, adrenaline and procaine) should
be given. The cycloplegics should be continued for
at least 2-3 weeks after the eye becomes quiet,
otherwise relapse may occur.
Mode of action. In iridocyclitis, atropine (i) gives
comfort and rest to the eye by relieving spasm of iris
sphincter and ciliary muscle, (ii) prevents the
formation of synechiae and may break the already
formed synechiae, (iii) reduces exudation by
decreasing hyperaemia and vascular permeability and
(iv) increases the blood supply to anterior uvea by
relieving pressure on the anterior ciliary arteries. As
a result more antibodies reach the target tissues and
more toxins are absorbed.
2. Corticosteroids, administered locally, are very
effective in cases of iridocyclitis. They reduce
inflammation by their anti-inflammatory effect; being

anti-allergic, are of special use in allergic type of
uveitis; and due to their antifibrotic activity, they
reduce fibrosis and thus prevent disorganisation and
destruction of the tissues. Commonly used steroidal
preparations contain dexamethasone, betamethasone,
hydrocortisone or prednisolone (see page 428).
Route of administration: Locally, steroids are used
as (i) eye drops 4-6 times a day, (ii) eye ointment at
bed time, and (iii) Anterior sub-Tenon injection is
given in severe cases.
3. Broad spectrum antibiotic drops, though of no use
in iridocyclitis, are usually prescribed with topical
steroid preparations to provide an umbrella cover for
them.
(b) Systemic therapy
1. Corticosteroids. When administered systemically
they have a definite role in non-granulomatous
iridocyclitis, where inflammation, most of the times,
is due to antigen antibody reaction. Even in other
types of uveitis, the systemic steroids are helpful due
to their potent non-specific anti-inflammatory and
antifibrotic effects. Systemic corticosteroids are
usually indicated in intractable anterior uveitis
resistant to topical therapy.
Dosage schedules. A wide variety of steroids are
available.Usually, treatment is started with high doses
of prednisolone (60-100 mg) or equivalent quantities

of other steroids (dexamethasone or betamethasone).
Daily therapy regime is preferred for marked
inflammatory activity for at least 2 weeks. In the
absence of acute disease, alternate day therapy
regime should be chosen. The dose of steroids is
decreased by a week’s interval and tapered completely
in about 6-8 weeks in both the regimes.
Note: Steroids (both topical and systemic) may cause
many ocular (e.g., steroid-induced glaucoma and
cataract) and systemic side-effects. Hence, an eagle’s
eye watchfulness is required for it.
2. Non-steroidal anti-inflammatory drugs (NSAIDS)
such as aspirin can be used where steroids are
contraindicated. Phenylbutazone and oxyphenbutazone
are potent anti-inflammatory drugs of particular
value in uveitis associated with rheumatoid disease.
3. Immunosuppressive drugs. These should be used
only in desperate and extremely serious cases of
uveitis, in which vigorous use of steroids have failed
to resolve the inflammation and there is an imminent
danger of blindness. These drugs are dangerous and
should be used with great caution in the supervision
of a haematologist and an oncologist. These drugs
are specially useful in severe cases of Behcet’s
syndrome, sympathetic ophthalmia, pars planitis and
VKH syndrome. A few available cytotoxic
immunosuppressive drugs include cyclophosphamide,
chlorambucil, azathioprine and
methotrexate. Cyclosporin is a powerful anti-T-cell
immunosuppressive drug which is effective in cases
resistant to cytotoxic immunosuppressive agents, but
it is a highly renal toxic drug.
(c) Physical measures
1. Hot fomentation. It is very soothing, diminishes
pain and increases circulation, and thus reduces the
venous stasis. As a result more antibodies are brought
and toxins are drained. Hot fomentation can be done
by dry heat or wet heat.
2. Dark goggles. These give a feeling of comfort,
especially when used in sunlight, by reducing
photophobia, lacrimation and blepharospasm.
II. Specific treatment of the cuase
The non-specific treatment described above is very
effective and usually eats away the uveal
inflammation, in most of the cases, but it does not
cure the disease, resulting in relapses. Therefore, all
possible efforts should be made to find out and treat
the underlying cause. Unfortunately, in spite of the
advanced diagnostic tests, still it is not possible to
ascertain the cause in a large number of cases.
So, a full course of antitubercular drugs for
underlying Koch’s disease, adequate treatment for
syphilis, toxoplasmosis etc., when detected should
be carried out. When no cause is ascertained, a full
course of broad spectrum antibiotics may be helpful
by eradicating some masked focus of infection in
patients with non-granulomatous uveitis.
III. Treatment of complications
1. Inflammatory glaucoma (hypertensive uveitis).
In such cases, drugs to lower intraocular pressure
such as 0.5 percent timolol maleate eyedrops twice a
day and tablet acetazolamide (250 mg thrice a day)
should be added, over and above the usual treatment
of iridocyclitis. Pilocarpine and latanoprost eye drops
are contraindicated in inflammatory glaucoma.
2. Post-inflammatory glaucoma due to ring
synechiae is treated by laser iridotomy. Surgical
iridectomy may be done when laser is not available.
However, surgery should be performed in a quiet eye
under high doses of corticosteroids.
3. Complicated cataract requires lens extraction with
guarded prognosis in spite of all precautions. The
presence of fresh KPs is considered a contraindication
for intraocular surgery.
4. Retinal detachment of exudative type usually
settles itself if uveitis is treated aggressively. A
tractional detachment requires vitrectomy and
management of complicated retinal detachment, with
poor visual prognosis.
5. Phthisis bulbi especially when painful, requires
removal by enucleation operation.

Aetiology Of Uveitis

Despite a great deal of experimental research and
many sophisticated methods of investigations,
etiology and immunology of the uveitis is still largely
not understood. Even today, the cause of many
clinical conditions is disputed (remains presumptive)
and in many others etiology is unknown. The
etiological concepts of uveitis as proposed by Duke
Elder, in general, are discussed here.
1. Infective uveitis. In this, inflammation of the uveal
tissue is induced by invasion of the organisms. Uveal
infections may be exogenous, secondary or
endogenous.
i Exogenous infection wherein the infecting
organisms directly gain entrance into the eye
from outside. It can occur following penetrating
injuries, perforation of corneal ulcer and postoperatively
(after intraocular operations). Such
infections usually result in an acute iridocyclitis
of suppurative (purulent) nature, which soon
turns into endophthalmitis or even
panophthalmitis.
ii Secondary infection of the uvea occurs by spread
of infection from neighbouring structures, e.g.,
acute purulent conjunctivitis. (pneumo-coccal and
gonococcal), keratitis, scleritis, retinitis, orbital
cellulitis and orbital thrombophlebitis.
iii Endogenous infections are caused by the entrance
of organisms from some source situated elsewhere
in the body, by way of the bloodstream.
Endogenous infections play important role in the
inflammations of uvea.
Types of infectious uveitis. Depending upon the
causative organisms, the infectious uveitis may be
classified as follows:
i. Bacterial infections. These may be granulomatous
e.g., tubercular, leprotic, syphilitic,

brucellosis or pyogenic such as streptococci,
staphylococci, pneumococci and gonococcus.
ii. Viral infections associated with uveitis are herpes
simplex, herpes zoster and cytomegalo inclusion
virus (CMV).
iii. Fungal uveitis is rare and may accompany
systemic aspergillosis, candidiasis and
blastomycosis. It also includes presumed ocular
histoplasmosis syndrome.
iv. Parasitic uveitis is known in toxoplasmosis,
toxocariasis, onchocerciasis and amoebiasis.
v. Rickettsial uveitis may occur in scrub typhus
and epidemic typhus.
2. Allergic (hypersensitivity linked) uveitis. Allergic
uveitis is of the commonest occurrence in clinical
practice. The complex subject of hypersensitivity
linked inflammation of uveal tissue is still not clearly
understood. It may be caused by the following ways:
i. Microbial allergy. In this, primary source of
infection is somewhere else in the body and the
escape of the organisms or their products into the
bloodstream causes sensitisation of the uveal tissue
with formation of antibodies. At a later date a
renewal of infection in the original focus may again
cause dissemination of the organisms or their
products (antigens); which on meeting the sensitised
uveal tissue excite an allergic inflammatory response.
Primary focus of infection can be a minute
tubercular lesion in the lymph nodes or lungs. Once
it used to be the most common cause of uveitis
worldwide, but now it is rare. However, in developing
countries like India tubercular infections still play an
important role. Other sources of primary focus are
streptococcal and other infections in the teeth,
paranasal sinuses, tonsils, prostate, genitals and
urinary tract.
ii. Anaphylactic uveitis. It is said to accompany
the systemic anaphylactic reactions like serum
sickness and angioneurotic oedema.
iii. Atopic uveitis. It occurs due to airborne
allergens and inhalants, e.g., seasonal iritis due to
pollens. A similar reaction to such materials as
danders of cats, chicken feather, house dust, egg
albumin and beef proteins has also been noted.
iv. Autoimmune uveitis. It is found in association
with autoimmune disorders such as Still’s disease,
rheumatoid arthritis, Wegener’s granulomatosis,
systemic lupus erythematosus, Reiter’s disease and
so on.
In phacoanaphytic endophthalmitis, lens proteins
play role of autoantigens. Similarly, sympathetic
ophthalmitis has been attributed to be an
autoimmune reaction to uveal pigments, by some
workers.
v. HLA-associated uveitis: Human leucocytic
antigens (HLA) is the old name for the
histocompatibility antigens. There are about 70 such
antigens in human beings, on the basis of which an
individual can be assigned to different HLA
phenotypes. Recently, lot of stress is being laid on
the role of HLA in uveitis, since a number of
diseases associated with uveitis occur much more
frequently in persons with certain specific HLAphenotype.
A few examples of HLA-associated
diseases with uveitis are as follows:
HLA-B27. Acute anterior uveitis associated with
ankylosing spondylitis and also in Reiter’s
syndrome.
HLA-B5: Uveitis in Behcet’s disease.
HLA-DR4 and DW15: Vogt Koyanagi Harada’s
disease.
3. Toxic uveitis. Toxins responsible for uveitis
can be endotoxins, endocular toxins or exogenous
toxins.
i. Endotoxins, produced inside the body play a
major role. These may be autotoxins or microbial
toxins (produced by organisms involving the body
tissues). Toxic uveitis seen in patients with acute
pneumococcal or gonococcal conjunctivitis and in
patients with fungal corneal ulcer is thought to be
due to microbial toxins.
ii. Endocular toxins are produced from the ocular
tissues. Uveitis seen in patients with blind eyes,
long-standing retinal detachment and intraocular
haemorrhages is said to be due to endocular toxins.
Other examples are uveitis associated with intraocular
tumours and phacotoxic uveitis.
iii. Exogenous toxins causing uveitis are irritant
chemical substances of inorganic, animal or
vegetative origin. Certain drugs producing uveitis
(such as miotics and cytotoxic drugs) are other
examples of exogenous toxins.
4. Traumatic uveitis. It is often seen in accidental or
operative injuries to the uveal tissue. Different
mechanisms which may produce uveitis following
trauma include:

Direct mechanical effects of trauma.
Irritative effects of blood products after
intraocular haemorrhage (haemophthalmitis).
Microbial invasion.
Chemical effects of retained intraocular foreign
bodies; and
Sympathetic ophthalmia in the other eye.
5. Uveitis associated with non-infective
systemic diseases. Certain systemic diseases
frequently complicated by uveitis include:
sarocoidosis, collagen related diseases (polyarteritis
nodosa (PAN), disseminated lupus erythematosus
(DLE), rheumatic and rheumatoid arthritis), metabolic
diseases (diabetes mellitus and gout), disease of the
central nervous system (e.g., disseminated sclerosis)
and diseases of skin (psoriasis, lichen planus,
erythema nodosum, pemphigus and so on).
6. Idiopathic uveitis. It may be specific or nonspecific.
i. Idiopathic specific uveitis entities include the
conditions which have certain special characteristics
of their own e.g., pars planitis, sympathetic
ophthalmitis and Fuchs’ hetero-chromic iridocyclitis.
ii. Nonspecific idiopathic uveitis entities include
the condition which do not belong to any of the
known etiological groups. About more than 25
percent cases of uveitis fall in this group.

Classification Of Uveitis

I. ANATOMICAL CLASSIFICATION
1. Anterior uveitis. It is inflammation of the uveal
tissue from iris up to pars plicata of ciliary body.
It may be subdivided into :
Iritis, in which inflammation predominantly
affects the iris.
Iridocyctitis in which iris and pars plicata part
of ciliary body are equally involved, and
Cyclitis, in which pars plicata part of ciliary
body is predominantly affected.
2. Intermediate uveitis. It includes inflammation of
the pars plana and peripheral part of the retina
and underlying ‘choroid’. It is also called ‘pars
planitis'.
3. Posterior uveitis. It refers to inflammation of the
choroid (choroiditis). Always there is associated
inflammation of retina and hence the term
‘chorioretinitis’ is used.
4. Panuveitis. It is inflammation of the whole uvea.
II. CLINICAL CLASSIFICATION
1. Acute uveitis. It has got a sudden symptomatic
onset and the disease lasts for about six weeks
to 3 months.
2. Chronic uveitis. It frequently has an insiduous
and asymptomatic onset. It persists longer than
3 months to even years and is usually diagnosed
when it causes defective vision.
III. PATHOLOGICAL CLASSIFICATION
1. Suppurative or purulent uveitis.
2. Non-suppurative uveitis. It has been further
subdivided in two groups (Wood’s classification).
(i) Non-granulomatous uveitis, and
(ii) Granulomatous uveitis

IV. ETIOLOGICAL (DUKE ELDER'S)
CLASSIFICATION
1. Infective uveitis
2. Allergic uveitis
3. Toxic uveitis
4. Traumatic uveitis
5. Uveitis associated with non-infective systemic
diseases
6. Idiopathic uveitis

CONGENITAL COLOBOMA OF THE UVEAL TRACT

Congenital coloboma (absence of tissue) of iris (Fig.
7.6), ciliary body and choroid (Fig. 7.7) may be seen
in association or independently. Coloboma may be
typical or atypical.

Typical coloboma is seen in the inferonasal
quadrant and occurs due to defective closure of
the embryonic fissure.
Atypical coloboma is occasionally found in other
positions.
Complete coloboma extends from pupil to the optic
nerve, with a sector-shaped gap occupying about
one-eighth of the circumference of the retina, choroid,
ciliary body, iris, and causing a corresponding
indentation of the lens where the zonular fibres are
missing.