Uveitis is less common in children than
In adults, accounts for fewer than 10% of
reported cases of uveitis, but its diagnosis
and management can be particularly
challenging. Young children are often
asymptomatic either because of inability
to express complaints or because of the
truly asymptomatic nature of their
disease. Even in advanced cases, parents
may not be aware of severe visual
impairment until the development of
externally visible changes such as band
keratopathy, strabismus, or leukocoria.
When diagnosing uveitis in children, as
with adults, it is important first to localize
the disease. When it is limited to the
anterior segment, the two most common
causes are juvenile idiopathic arthritis
and chronic idiopathic anterior uveitis. If
the disease affects the posterior
segment, infectious causes such as
TORCH (toxoplasmosis, rubella,
cytomegalovirus, herpes) and
toxocariasis must be considered.
In panuveitis, conditions such as sarcoidosis,
syphilis, and masquerade syndromes like
leukemia may be involved.
patients diagnosed with juvenile idiopathic
arthritis, sarcoidosis, and other conditions
must be routinely evaluated for ocular
Connection between specialists .
There is no universally accepted
technique for classifying uveitis. Many
authors choose to classify uveitis based on
causative factor. Those texts generally
group uveitis into inflammatory or infective
categories. Classification can also be based
on time course of the disease, with acute,
subacute, and chronic types as the major
Many ophthalmic texts also classify
uveitis in terms of white cell types using
granulomatous and nongranulomatous
as the major subclassificatin.
The pediatrician find it easier to classify
uveitis based on the anatomical
structures involved, uveitis will be
classified into four categories: anterior
uveitis, intermediate uveitis, posterior
uveitis, and masquerade syndromes
JIA-associated Anterior Uveitis
JIA is the most common systemic association
of pediatric uveitis. It is defined as arthritis of
at least 6 weeks’ duration without any other
identifiable cause in children younger than 16
years of age. The International League of
Associations for Rheumatology (ILAR) has
classified JIA into seven subtype
Patients with systemic-onset JIA have extraocular
manifestations Uveitis is extremely rare in this
the oligoarticular subtype is diagnosed when fewer
than 5 joints are involved during the first 6 months
of the disease. Chronic anterior uveitis is most
commonly associated with oligoarticular JIA.
Polyarticular JIA is diagnosed when 5 or more joints
are affected during the first 6 months of the
disease. Polyarticular JIA may be rheumatoid
factor (RF) positive or negative. Uveitis is rare in
the RF-positive group, however approximately
10% of patients with RF-negative polyarticular JIA
Psoriatic arthritis is a less common
subtype of JIA which may be
associated with chronic anterior
uveitis in 10–20% of cases. Patients
with arthritis who do not fulfill any of
these categories are classified as the
“other” JIA subgroup.
Risk factors for ocular involvement in
patients with JIA include female sex,
oligoarticular arthritis, young age at onset
of arthritis, antinuclear antibody (ANA)
seropositivity and RF seronegativity
The first line of treatment is topical
corticosteroids, and mydriatic,Topical
corticosteroids are used frequently during
exacerbations and tapered as the
inflammation subsides. If topical steroids are
insufficient to control the inflammation, depot
steroid injections or even systemic steroids
are used. In cases of uveitis where significant
doses of steroids are needed to control the
inflammation, and they cannot be easily
tapered, immunosuppressive agents are often
used as an adjunct therapy.
band keratopathy develop in More than 50% of patients
These are usually not visually significant unless they
Cataracts can develop in up to one-third of patients
recently a diagnosis of JIA has been
acontraindication to lens implantation however,
some ophthalmologists are attempting lens
implantation with newer (less inflammatory)
Glaucoma occurs in about 20% of these patients
and does not respond well to medical therapy
Early detection and treatment of JIA
associated uveitis are of most
importance to preserve vision in
This category of disease accounts for about
15% of pediatric anterior uveitis cases.
juvenile ankylosing spondylitis,
juvenile Reiter’s syndrome,
juvenile psoriatic arthritis,
Boys are more commonly affected,
Usually >8 years of age,
These patients often present with acute recurrent
uveitis that is bilateral and asymmetric.
A large majority of these patients are positive HLA-
B27 ,most are RF negative.
Some juvenile ankylosing spondylitis patients can
present with a severe acute anterior uveitis and
Treatment and prognosis
similar to JIA including topical
corticosteroids and mydriatic agents.
The acute recurring bouts of inflammation
tend to taper off as these children age.
The first episode of uveitis is often the most
severe, and if treated early, the visual
prognosis is very good.
There are two distinct subgroups of
sarcoidosis that present with ocular
Childhood sarcoidosis is seen in children
< 5 years old, is more common in girls, and
is characterized by arthritis with a skin rash.
There is a striking predominance of
children affected by this early-onset
Is also seen in children, most commonly
between the ages of 8 and 15 years, and is
three times more common in African–
American children than in Caucasians. This
form is seen with equal frequency in boys
and girls. These children have pulmonary
findings, and may have
anterior uveitis is the most common
presentation, granulomatous in nature
sarcoidosis can present with intermediate,
Angiotensin-converting enzyme (ACE)
levels, ACE levels must be age-matched
because children have higher ACE levels
Serum lysozyme levels,
Chest X-ray, and gallium
Scanning of the lungs and lacrimal glands.
Definitive diagnosis is made by biopsy of a
nodule showing non caseating granulomas
TREATMENT AND PROGNOSIS
Treatment of the anterior uveitis consists of
topical corticosteroids and mydriatic
agents. Often systemic corticosteroids that
are used to treat pulmonary complications
can control the ocular disease. While
dramatic improvement is seen clinically
with systemic and topical steroids, the
chronic nature of the uveitis associated
with sarcoidosis often leads to the
development of both cataracts and
glaucoma. Both of these complications can
be treated surgically with good results
CLINICAL PRESENTATION AND DIAGNOSIS
Both (HSV) and (HZV) can cause anterior uveitis.
The inflammation is generally unilateral, can be
either acute or chronic, and is often associated
with an increase in IOP. Herpetic uveitis typically
occurs in conjunction with significant corneal
involvement. Children typically present with
photophobia and decreased vision. HSV and
HZV uveitis can also present with concomitant
Sector iris atrophy as a result of HZV iritis
Gonioscopic photograph of a layered
hyphema in a patient with HZV iritis
TREATMENT AND PROGNOSIS
Diagnosis is usually confirmed by corneal culture
topical or systemic antiviral agents, corticosteroids,
Corticosteroids should be administered later in the
course of the disease as they can prevent corneal
epithelial wound healing.
Prognosis is good, with the good visual outcome
depending on the level of corneal scarring.
Glaucoma can occur with active inflammation and
is usually well treated with medical management.
In cases of HZV uveitis, sector iris atrophy can be
Idiopathic Intermediate Uveitis (Pars
According to the anatomic classification
of uveitis by the Standardization of Uveitis
Nomenclature (SUN) Working Group, the
term “intermediate uveitis” defines a
subset of uveitis where the vitreous is the
primary site of inflammation.
Pars planitis is a diagnostic term that defines
a subset of idiopathic intermediate uveitis
where there is snowbank or snowball
formation. This disease typically affects
children and adolescents.
the association of intermediate uveitis with a
systemic disease is very rare in children.
Associations between idiopathic intermediate
uveitis and HLA-DR2 and HLA-DR15 have
been reported suggesting an immunogenetic
Typical clinical findings include mild to
moderate anterior segment inflammation,
diffuse vitreous cells and haze, and
snowballs(accumulations of white blood cells in
the anterior vitreous ) and snowbanks
(exudates over the pars Plana) located inferiorly
Band keratopathy and posterior synechiae may
be seen in childhood pars planitis but are very
rare in adults.
Optic disc edema and cystoid macular edema are
the most frequent complications.
snow ball opacities and vitreous haze in
a child with pars planitis
The peak age of onset for Behçet disease is in
the third or fourth decade of life. Although
the onset of recurrent oral ulcers in
childhood is not uncommon, patients
typically fulfill the diagnostic criteria after the
age of 16 years. There are no internationally
accepted diagnostic criteria for childhood-
onset Behçet disease.
In a recent international registry of patients
suspected of pediatric Behçet disease, the
presenting symptom was isolated recurrent oral
ulcers in 83%, and the diagnosis was confirmed
by an expert committee in 62% of registered
From an ophthalmological point of view,
pediatric Behçet uveitis is defined as onset of
uveitis at 16 years of age or younger
irrespective of the age for fulfilling the diagnostic
Mean age at onset is in late childhood (10–15
a male predominance in the pediatric age
group, similar to adult-onset Behçet uveitis.
A positive family history has been reported in
20–47% of pediatric cases from endemic areas,
implying the role of genetic factor.
bilateral involvement and recurrent panuveitis
with retinal vasculitis (occlusive in nature ,
both arteries and veins are affected
Don’t forget shifting hypopyon
1-painful oral aphthus ulcer recurrent at least
+ at least one of the following
-recurrent genital ulcers
-skin lesion(erythema nodosum or
-+ve pethargy test
Complications and prognosis
Cataract, macular edema or maculopathy,
and optic atrophy are the most common
While visual prognosis has been reported to
be better than adults in some series.
posterior segment complications of Behçet's disease. branch retinal vein
occlusion caused by periphlebitis; retinal infiltrates; massive retinal exudation;
atrophy and vascular sheathing: posterior segment manifestation
The most common cause of pediatric posterior
at least 50% of cases.
The infective agent is the intracellular protozoan
Cats are the definitive host, with human infection a
result of ingestion of the encysted organism in
undercooked meats. but ingestion of encysted
protozoans in immuno competent individuals usually
Congenital systemic toxoplasmosis
Trancplacental when a pregnant woman contracts the
acute form. If the mother is infected before the
pregnancy, the fetus will be unaffected.
infection during early pregnancy may result in
infection during late pregnancy may cause
generalized convulsions, paralysis, fever and visceral
involvement. Intracranial calcification may be seen on
plain skull radiographs
intracranial calcification in congenital toxoplasma
Macular scar in congenital
Unilateral ,sudden onset visual loss and photophobia
Spillover anterior uveitis (granulomatous )
Solitary inflammatory focus near old pigmented
Multiple foci un common
Sever vitritis obscuring the fundus picture ,however
the active focus still visible (headlight in the frog
Active retinochoroiditis in
Ocular toxocariasis is caused by the canine roundworm
Infection commonly occurs after ingestion of soil
contaminated by the roundworm eggs.
Ocular toxocariasis is more common in boys and
usually there is a history of geophagia.
Ocular involvement is usually unilateral and presents
either as endophthalmitis or as a granuloma
(peripheral or central).
posterior pole granuloma;
optic nerve head granuloma;
severe fibrosis and tractional retinal detachmen
Retinal traction to the optic nerve head and
macular scarring in toxocariasis.
Anterior traction on the optic nerve head from
toxocariasis peripheral granuloma
CLINICAL PRESENTATION AND DIAGNOSIS
more in darker pigmented individuals,
three stages. The first stage is commonly mistaken for a
viral infection, with flu-like symptoms, headache, and
tinnitus or hearing loss.
Stage 2 is the ophthalmic stage where patients develop
bilateral panuveitis, hyperemia of the optic disc, and
serous retinal detachments.
This stage is often when the patient presents
with pain, photophobia, and decreased vision.
Stage 3, the convalescent stage
dermatologic manifestations appear. These
include poliosis (whitening/graying of a
patch of hair), vitiligo and alopecia in Stage
3 ophthalmic disease is characterized by
retinal depigmentation, proliferation of
retinal pigment epithelium, which can
cause a puckering of the macula and
development of peripheral yellow/white
deposits under the retina (Dalen–Fuchs
Multiple serous retinal detachments in VKH.
Diagnosis is made based on clinical findings
since the exact cause of VKH is unknown.
Systemic autoimmune response to retinal,
uveal, and cutaneous melanocytes.
Laboratory studies are usually not helpful in
making the diagnosis, but a lumbar puncture
with pleocytosis is supportive. Ophthalmic
ultrasound during active inflammation
shows findings consistent with panuvei tis
bilateral panuveitis seen after penetrating
ocular traum the injured eye (exciting eye)
often develop inflammation first, and the
uninjured eye (sympathizing eye)
followingweeks to months later .
It develops within 3 months (70%) of the
original injury; however, it may develop in as
short as 5 days and as long as 42 years.
Sympathetic ophthalmia, can be prevented
with early removal of the injured eye if
there is no visual potential. Because of the
rarity of sympathetic ophthalmia, if there is
any potential vision, removal is not always
High-dose systemic steroids, along with
topical and periocular injections, are
Cyclosporine can help in unremitting cases
Once inflammation has started ,some
studies recommend removal of the exiting
eye. This is controversial, and other studies
have shown no benefit of removal.
Visual prognosis is good with up to 75% of
patients retaining good vision (>20/50).
Many of those patients require long-term
steroids to retain that vision.
The most common pediatric malignant ocular tumor
Retinoblastoma presents most commonly as either
leukocoria , strabismus, or with signs of ocular
The inflammation from retinoblastoma often causes a
red, painful eye that is photophobic. A hypopyon may
be as well as a ‘pseudohypopyon’, which actually
represents a layering of the tumor cells in the anterior
The most common ocular presentation of systemic
leukemia is retinal hemorrhages on fundus
Leukemic infiltrates within the uvea may lead to
pseudoanterior iritis, which can layer out in the
anterior chamber forming a hypopyon .
Choroidal involvement generally presents as serous
retinal detachments and optic nerve involvement
presents as papilledema.
Definitive diagnosis is made with bone marrow
biopsy and smear. Aqueous tap maybe be
performed for cytology
Pediatric uveitis is a complex, chronic, and
challenging condition for the patient,
parent, and physician. Close monitoring,
cooperation with other specialists,
aggressive treatment, and consideration of
amblyopia are all key to prevent
complications from this disease.