Leukocoria

Presented By
Sayed Hamzah
I11108081

Definition
(Leukos: white, Kore: pupil)
Leukokoria is an abnormal pupillary light
reflection that usually results from an
intraocular abnormality and is seen most often
in children.

Shields JA, Shields CL, Parsons HM. Retina 1991;11:232-43

Congenital Cataract
Opacity of the lens at birth, either hereditary or
acquired throughout the placenta
Signs :
Leukocoria
Nystagmus
Strabismus
Blunted red reflex

etiology

Idiopathic (most common).

Familial
Galactosemia
PHPV
Rubella
Lowe syndrome (oculocerebrorenal syndrome
Others

Type of Cataract

Coronary cataract : Single or

multiple finger- or bowling pinshaped opacities that ring the
peripheral cortex

Cerulean cataract : Small,

bluish punctate opacities of the
peripheral cortex (anterior,
posterior or both)

Treatment
Referral to a pediatrician to treat any underlying
disorder.
Treat associated ocular diseases.
Cataract extraction,
Treat amblyopia in children younger than 9 to 11 years
A dilating agent (e.g., phenylephrine 2.5%, t.i.d.,
homatropine 2% t.i.d., or scopolamine 0.25% q.d.) may
be used as a temporizing measure, allowing peripheral
light rays to pass around the lens opacity and reach the
retina. This rarely is successful long-term.

Retinoblastoma
Retinoblastoma is a tumor that originates from
immature retinoblasts in the neural retina; it is
the most frequent intraocular tumor in children.
The incidence ranges from 1/14,000 to 1/20,000
live births, depending on the country
Over 90% of cases are diagnosed before age 5
years

Clinical presentations

leukocoria (from 60 to 80% of cases)

Strabismus
red eyes
Excessive tearing
enlarged ocular globe due to increased intraocular
pressure
loss of vision
Less common forms are : aseptic orbit cellulitis due to
tumor necrosis, altered color of the iris (heterochromia),
intraocular bleeding and pseudouveitis.

Diagnosis
Family history
Ophthalmoscopy
A grayish white, vascularized retinal tumor
CT scan detecting calcium within the tumor
Ultrasoundthe size of the lesions and may find
calcification within the tumor
MRI extraocular involvement and invasion of
the optic nerve

Treatment

Intravenou chemotherapy
Enucleation
Radiation therapy
External beam radiation therapy
Plaque radiotherapy
Laser therapy
Photocoagulation
Transpupillary thermotherapy (TTT)
Cryotherapy
Observation (for spontaneously arrested retinoblastoma,
retinoma)

RETINOPATHY OF PREMATURITY
(ROP)
Vasoproliferative retinopathy affecting premature infants
exposed to high oxygen
ROP affects immature blood vessels of the retina. It
occurs weeks after birth
INCIDENCE
47% in infants with birth weights between 1000 and
1251g , 81.6% for infants weighing less than 1000g
(2lb3oz) at birth.
80% of infants born at less than 28 weeks gestational
age, 60% of infants born at 28-31weeks developed ROP
Oxygen duration

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

Coats Disease
A localized, congenital, retinal vascular disorder
consisting of abnormal telangiectatic segments
of blood vessels that result in leakage

Characteristic

Retinal telangiectasia
Retinal capillary nonperfusion
Dilated intercapillary spaces.
Lipid exudate
Leukokoria
Subretinal fluid
Usually unilateral
Male predominance
Fibrovascular macular scars

Diagnose

Clinical manifestation :
poor vision
Strabismus
Leukokoria
Examination :
ophthalmoscopically vascular abnormalities are
seen in association with lipid deposition and
subretinal exudate
Ancillary testingFluorescein angiography is a
useful tool for delineating the nature and extent of
the vascular abnormalities present in this disease.

Classification
Shield et al :
Stage 1 : retinal
telangiectasia only
Stage 2 : telangiectasia
& exudation
A : extrafoveal exudation
B : foveal exudation

Stage 3 : exudative retinal detachment

A : Subtotal retinal detachment
B : Total retinal detachment

Stage 4 : total retinal detachment & glaucoma

Stage 5 : advanced end-stage disease

Treatment
The main goal : to obliterate the telangiectasiae to
facilitate the reabsorption of exudates and maintain
as much visual acuity as possible
Laser photocoagulation or cryotherapy to destroy
anomalous vasculature.
intravitreal proliferation and traction detachment
vitreous surgery may improve the clinical course

PHPV (PRIMER HIPERPLASTIK PERSISTEN

VITREUS)
developmental malformation of the eye where the
embryological primary vitreous and hyaloid vasculature
fail to regress.
Persistent hyperplastic primary vitreous (PHPV) is a
rare,non-hereditary,
It presents unilaterally in 90% of cases and can be
associated with microphthalmia and cataract formation.
It is usually not associated with other congenital defects.
In the majority of cases the fibrovascular membrane, fed
by the persistent hyaloid artery, adheres to the posterior
capsule of the crystalline lens.

 A gray-yellow retrolental membrane may

produce leukocoria, with the subsequent
suspicion of retinoblastoma.

PHPV
Diagnostic Consideration
Based on the characteristic clinical picture
(symptoms and findings) and additional
ultrasound studies (when the posterior
segment is obscured by lens opacities)
Treatment
Treatment options include cataract surgery and
removal of the fibrovascular membrane.

Ocular Toxocariasis
Ocular toxocariasis is a unilateral disorder that
presents as strabismus, leukocoria or decreased
vision.
Can be associated with systemic oxocariasis

INCIDENCE
Toxocariasis is the most common nematode
infection affecting the eye in the United States;
however, the exact incidence of ocular
toxocariasis in unknown.
ETIOLOGY
Most cases of human toxocariasis are caused by
infection with the dog intestinal roundworm
Toxocara canis or, rarely, the cat Toxocara
catis.Ocular toxocariasis results from invasion of
the eye by the second- or third-stage larva of the
nematode.

Clinical Features
80% less than 16 years.
Another common manifestation is chronic
endophthalmitis.
often present anterior uveitis, hypopyn,
posterior synechiae, cyclitic membrane, vitritis,
and retinal detachment.
Leukocoria because of the severe
inammatory reaction
decreased visual Acuity
amblyopia,
strabismus as a result of damage to the macula.

Systemic manifestation

known as visceral larval migrans (VLM).

occurs most commonly in children less than 6 years
eosinophilia,
fever
hepatosplenomegaly
If ocular disease and VLM are both present, ulike symptoms
Less common manifestations pulmonary
symptoms, splenomegaly, and seizures.

 The most common presentation is a unilateral granuloma of the posterior

pole or peripheral retina typically round, elevated, andup to two disc
diameters in size
Vitritis
Peripheral granuloma hazy white elevated lesions located in the
peripheral retina.
Peripheral granuloma located in the inferior retina may resemble pars
planitis in some children
Vitreous membranes are often visible radiating from the peripheral lesion
and may form radial retinal folds extending to the optic nerve head.

Toxocara canis- elevated granuloma

Toxocara canis retinitis.

Diagnosis
Diagnosis is based upon clinical features
observed in a young patient and should be
confirmed at least by the presence of specific IgG
in the serum (ELISA test, 90% specificity and
91% sensibility).

Treatment
based on : severity of inammation, macular
involvement, and the visual potential of the eye.
periocular or systemic corticosteroids active
vitritis
topical corticosteroids and cycloplegic agents
anterior uveitis, Thiabendazole at 50mg/kg/day for 7
days may be considered in children who fail to
respond to systemic corticosteroid therapy
antihelminthic therapy potential toxicity
Surgical procedures refractory vitritis, vitreous
membranes, epiretinal membrane, and traction or
rhegmatogenous retinal detachment.

Retinal detachment
Retinal detachment is a disorder of the eye in
which the retina peels away from its underlying
layer of support tissue
incidence : 1:10 000
Variety of ocular and systemic disorders are
associated with pathological vitreous
liquefaction, premature vitreous detachment,
and extensive sites of vitreoretinal adhesion

Pathophysiology
extensive liquefaction within the vitreous cavity
reduction in both the shock-absorbing capabilities
and the stability of the gel associated by aging
Accelerated vitreous liquefaction :
significant myopia
post cataract surgery (40%)
severe ocular trauma (10-15 %)
intraocular inflammation
a variety of other congenital, inherited, or acquired
ocular disorders.

Pathology

Classical pathogenesis of
rhegmatogenous retinal
detachment. The detached vitreous
gel has caused a retinal tear by
exerting traction upon the retina at
the site of a vitreoretinal adhesion.
Liquid in the vitreous cavity passes
through the break into the
subretinal space.

 Rhegmatous retinal
detachment
Traction retinal
detachment

Exudative retinal
detachment

Combined retinal
detachment

Treatment
scleral buckling techniques and the creation of a
chorioretinal adhesion around each break to
eliminate and counteract vitreoretinal traction
Vitrectomy techniques
Pneumatic retinopexy

Optic Disk Coloboma

An optic disk coloboma is the result of incomplete
closure of the embryonic optic cup.
The optic disk is enlarged with a funnel-shaped
depression with whitish tissue and a peripapillary
pigment ring.
The retinal vessels extend outward across the
margin of the disk in a radial pattern without a
central trunk vessel.
Patients with optic disk coloboma often have
decreased visual acuity and visual field defects.

 retinochoroidal colobomas that has received

particular attention CHARGE syndrome
(coloboma, heart disease, atresia choanae,
retarded growth, genital hypoplasia, ear
anomalies, with or without deafness

Norrie 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.

END
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