LEUKOCORIA

DEFINITION
Leukocoria
A white pupillary reflex (leukos:
white, kore: pupil), pink-white, or
yellow-white pupillary reflection.
Normal reflex red reflex

The red reflex refers to the reddish-orange

reflection of light from the eye's retina that is
observed when using an ophthalmoscope or
retinoscope from approximately 30 cm / 1 foot.
This examination is usually performed in a dimly
lit or dark room.

Normal red reflex

Leukocoria bilateral

Leukocoria unilateral

Diferential diagnosis of leukocoria:

Cataract
Retinoblastoma
Toxocariasis
Coats disease
Retinopathy of prematurity
(ROP)
Persistent hyperplastic primary
vitreous (PHPV)
Retinal detachment
Norries disease

CATARACT
Cataract any opacity in the lens
Factors : aging, trauma, toxins,
systemic
disease
(such
as
diabetes), smoking, and heredity.
Childhood congenital cataract
sign :
leukocoria
absent red pupillary reflex
abnormal eye movements
(nystagmus)
Strabismus

RETINOBLASTOMA
Retinoblastoma
is
the
most
common intraocular malignant
neoplasm of childhood affecting
1:20,000 to 1:34,000 children.
The
tumor
occurs
most
frequently within the first 2
years of life and may even be
found at birth.
Signs: leukocoria, strabismus,
poor
vision,
spontaneous
hyphema, or a red, painful eye.

RETINOBLASTOMA
A

A. Leukocoria in the left

eye
B. This surgically
excised eye is almost
filled by a creamcolored intraocular
retinoblastom

RETINOBLASTOMA

Typical
appearance
of
intraretinal
retinoblastoma.
Opaque,
yellow-white
macular tumor fed and drained by dilated,
tortuous retinal blood vessels.

RETINOBLASTOMA
A

A. B-scan ultrasonography of retinoblastoma. Solid,

posterior
intraocular
mass
contains
strong
particulate reflections attributable to intralesional
calcification.
B. Computed tomography of bilateral intraocular

RETINOBLASTOMA
Treatment option:
Intravenous chemotherapy
Enucleation
Radiation therapy
Lasertherapy
Cryotherapy

RETINOBLASTOMA - Chemotherapy

PRETREATMENT

Same lesion after

two cycles of
chemotherapy

TOXOCARIASIS
Toxocariasis

a
nematode
infection (Toxocara canis)
Retinal damage is the result of the
host's
inflammatory
response
(severe eosinophilic reaction) that
becomes granulomatous
The patient may have a history of
contact with puppies or eating
dirt.

TOXOCARIASIS
Present :
Occular symptoms : redness,
photophobia & pain.
Leukocoria, strabismus, decreased
vision.
Ophthalmic physical examination :
A
large
peripheral
(anterior)
retinal or choroid inflammatory
mass,
associated
with
a
vitreoretinal reaction secondary
retinal detachment.

TOXOCARIASIS

A white intravitreous mass of a

toxocaral eosinophilic granuloma

COATS DISEASE
Congenital
retinal
telangiectasis
(Coats' disease) is an idiopathic
retinal vascular disorder that usually
affects
young
male
patients
unilaterally in their first or second
decade of life. No tumor mass.
Retinal telangiectasia.
Retinal capillary nonperfusion.
Dilated intercapillary spaces.
Lipid exudate.
Subretinal fluid
Retinal detachment

COATS DISEASE
A

A. Coats disease in children

B. vascular
abnormalities
with
aneurysmal
dilatation,
telangiectasia, exudation, and
severe lipid deposition in the

COATS DISEASE - Treatment

Photocoagulation Obliterate
Cryotherapy
telangiectasia
Enucleated
severe

later stage

RETINOPATHY
OF PREMATURITY (ROP)
Vasoproliferative

retinopathy affecting
premature infants exposed to high
oxygen
INCIDENCE
Prematurity (<32/40 weeks)
Birth weight (30% < 1000gm affected)
Exposed to high oxygen
90% ROP regresses spontaneously, 5%
blindness

RETINOPATHY
OF PREMATURITY (ROP)
early stages of ROP
a band of glomeruloid capillaries
proliferates at the junction between
the peripheral nonperfused and the
posterior perfused retina
break through the internal limiting
membrane and invade the vitreous,
inciting fibrosis and contraction
the retina is folded on itself by the
organized vitreous, forming a
fibroneural mass that drags the
macula and optic disc temporally
The end stage total retinal

SCREENING

Indications
Almost all infants with ROP have a gestational age of
31 weeks or less (regardless of birth weight) or a birth
weight of 1250 g or less; these indications are generally
used to decide whether a baby should be screened for
ROP,
Any premature baby with severe illness in perinatal
period (Respiratory distress syndrome, sepsis, blood
transfusion, Intra ventricular haemorrhage, apnoeic
episodes, etc.) may also be offered ROP screening.

Timing
Retinal examination with scleral depression is
generally recommended for patients born before
3032 weeksgestation, or 46 weeks of life,
whichever is later. It is then repeated every 13
weeks until vascularization is complete (or until
disease progression mandates treatment).

RETINOPATHY
OF PREMATURITY (ROP)
LOCATION
zone 1 centred on
disc, 2x disc
to fovea
distance
zone 2 - outer
limit equator
temporally,
ora nasally
zone 3 temporal
peripheral
crescent

Staging (ROP) : 1
Stage 1:
The flat,
white border
between
avascular
and vascular
retina seen
superiorly is
called a
demarcation
line.

Staging (ROP) : 2
Stage 2:
The elevated
mesenchym
al ridge has
height.
Highly
arborized
blood
vessels from
the
vascularized
retina dive
into the

Staging (ROP) : 3
Stage 3:
Vessels on
top of the
ridge project
into the
vitreous
cavity. This
extraretinal
proliferation
carries with
it a
fibrovascula
r membrane

RETINOPATHY
OF PREMATURITY (ROP)
Treatment :
Laser photocoagulation
Cryotherapy
Vitrectomy surgery

STENT HYPERPLASTIC PRIMARY

OUS (PHPV)
Persistent

hyperplastic primary
vitreous (PHPV) is a congenital
anomaly in which the primary
vitreous fails to regress in utero.
Highly vascular mesenchymal tissue
nurtures the developing lens during
intrauterine life. In PHPV, the
mesenchymal tissue (fibrovascular)
forms a mass behind the lens.
A gray-yellow retrolental membrane
may produce leukocoria, with the
subsequent suspicion of

PERSISTENT HYPERPLASTIC
PRIMARY
VITREOUS
(PHPV)
Sign :
Leukocoria
Small eye (microphthalmia)
Episodes of intraocular
(intravitreous) hemorrhage.
Retinal detachment
traction from fibrovascular
mass.

NORRIES DISEASE
Norrie disease, or the progressive
oculoacousticocerebral degeneration
of Norrie, is a rare, X-linked recessive
heritable disorder characterized by
bilateral leukocoria caused by retinal
detachment. Affected boys classically
have a triad of blindness, deafness,
and mental retardation. Apparent at
birth or in early infancy, the ocular
findings usually progress to phthisis
bulbi.

RETINAL DETACHMENT
A
retinal
detachment
(RD)
describes the separation of the
neurosensory retina (NSR) from
the retinal pigment epithelium
(RPE) caused by a breakdown of
the forces that attach the NSR to
the RPE.
Toxocariasis,
PHPV,
Coats disease

ROP,

RETINAL DETACHMENT

Treatment:
Cryotherapy
laser photocoagulation using
a slit-lamp delivery system
and
laser using the indirect
ophthalmoscopic delivery

CONCLUSION

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