Dr. Mariya Nazish Memon
MBBS,FCPS,Fellow Pead Ophth & strabismus(ASEH)
Senior Registrar , Head of Unit
Pediatric Ophthalmology and Strabismus
Liaquat university Eye hospital, Hyderabad
OBJECTIVES
 Enlist common causes of white pupil in children
 Identify the child with serious visual and life
threatening problem
 Understand the immediate need of referral to
Ophthalmologist
Leucokoria
 White pupillary reflex
 “amaurotic cat’s eye”
 Greek word
“leucos” (white) and
“korê” (pupil)
Causes of White Pupil in children
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Cataract
Retinoblastoma
Retinopathy of prematurity
Persistent fetal vasculature
Coats disease
Toxocariasis
Coloboma (fissure or cleft) of choroid or optic disc
Retinal dysplasias
Uveitis
Vitreous hemorrhage
Importance
Infancy and early
childhood is an
important time for
visual development.
 The eyes grow and
emmetropise
 Vision improves
 Stereopsis matures
 Accommodation
develops
Congenital cataract
Opacification of the
crystalline lens present
at the time of birth or
develop after birth
during maturity period
of the lens
Important facts
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33% - idiopathic - may be unilateral or bilateral
33% - inherited - usually bilateral
33% - associated with systemic disease - usually bilateral
Other ocular anomalies present in 50%
Classification of congenital cataract
Anterior polar
Lamellar
Posterior polar
Central pulverulent
Coronary
Sutural
Cortical spoke-like
Focal dots
Causes of cataract in healthy neonate
Hereditary
(usually dominant)
Idiopathic
With ocular anomalies
. PHPV
• Aniridia
• Coloboma
• Microphthalmos
• Buphthalmos
Iatrogenic pediatric cataract
•Laser photoablation for ROP or tumor
• External beam radiation
• steroid therapy
• Damage to posterior capsule due to
posterior vitrectomy
Causes of cataract in unwell neonate
Intrauterine infections
• Rubella
• Toxoplasmosis
• Cytomegalovirus
•Herpes simplex
• Varicella
Metabolic disorders
• Galactosaemia
• Hypoglycaemia
• Hypocalcaemia
• Lowe syndrome
Chromosomal abnormalities
• Down syndrome (trisomy 21)
• Patau syndrome (trisomy 13)
• Edward syndrome (trisomy 18)
Management
OCULAR EXAMINATION
 Visual behavior
 Density of cataract
 Morphology
 Associated ocular pathology
 Pupillary reflex
 Ocular Ultrasound(B Scan)
Systemic investigations
Serology:
TORCHS titre and VDRL
Urine analysis:
for amino acids(lowe syndrom) and reducing
substance after drinking milk(galactosaemia)
Blood test:
Fasting blood sugar,serum calcium and phosphours,
red-cell GPUT and galactokinase level
Indications for Surgery
Bilateral dense Cataracts
B. Unilateral dense Cataracts
C. Partial unilateral /bilateral cataract
A.
Management
 Surgery:
o Lens matter aspiration,
posterior capsulotomy,
anterior vitrectomy +/_
IOL implantation
 Visual rehabilitation:
o Spectacles
o Contact lenses
o IOL implantation
o Ambyopia therapy
Retinoblastoma
 Most common intraocular tumour of childhood
 May be heritable(40%) or non-heritable(60%)
 Located chromosome- 13q14
 malignant transformation of primitive retinal cells
before final differentiation.
 As these cells disappear in the first few years of life, the
tumour is seldom seen after 3 years of age
Presentations of Retinoblastoma
• Leukocoria - 60% • Strabismus - 20%• Secondary glaucoma
• Anterior segment invasion
• Orbital inflammation• Orbital invasion
Signs/Growth pattern
Endophytic
Exophytic
Investigations
Ultrasound
C T Scan
Investigations
MRI
Poor Prognostic Factors
Optic nerve involvement
Choroidal invasion
Large tumour
Anterior location
Poor cellular differentiation
Older children
MANAGEMENT
Depends on size, location and staging of tumour
Treatment of small (3 mm diameter) tumours
 Photocoagulation
 Cryotherapy
 Chemotherapy
Medium sized (upto 12 mm) tumours
 Chemotherapy
 External beam radiation
Large tumours
 Chemotherapy
 Enucleation
Treatment
Extraocular extension
Chemotherapy
Radiotherapy
Metastatic Disease
High dose chemotherapy
Intra-thecal chemotherapy
Total body radiotherapy
Follow-up
 Heritable Retinoblastoma patients can develop
recurrences and need to be followed up regularly
 Examine the patients every 6-8wks till 3yrs,every 6
months till the age of 5 yrs and then annually till the
age of 10 years.
Retinopathy of Prematurity
Proliferative retinopathy
affects low birth weight
premature infant.
RISK FACTOR
Major Risk Factor:
 Prematurity < 32 weeks gestation (< 30 weeks)
 Low birth weight < 1500 gm (<1250 gm)
 Supplemental Oxygen.
Minor Risk Factor:
 Maternal: Complications of pregnancy, use of beta
blockers.
 Fetal: Hypercarbia, Sepsis, Vitamin E deficiency,
Intraventicular haemorrhage, Recurrent apnea, RDS,
Indomethacin treatment for PDA.
RETINAL VASCULARIZATION
STAGING
STAGING
Stage:5.
Funnel shaped Total
retinal detachment
SYMPTOMS
Symptoms of severe ROP include:
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Nystagmus (Abnormal eye movements)
Amblyopia (Lazy eye)
Strabismus (Crossed eyes)
Myopia (Severe near sightedness)
Leucocoria (White-looking pupils )
Glaucoma
Cataract
Retinal detachment
Screening for ROP
 All pre mature born at or before 32 weeks of gestation
 All premature with birth weight of 1500 gms or less
 Screening should start 4 weeks after birth
Management
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In 80% of infant ROP will regress spontaneously
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Treatment is indicated in stage 3 disease
 Argon laser in the periphery
 Cryotherapy (trans-scleral)
 Anti VEGF intravitreal injection
 RD surgery for stage IV and V
Persistent fetal
vasculature(PFV/PHPV)
 Unilateral
 Failure of regression of primary vitreous/hyaloid
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system
Typically present with leukocoria,squint or Nystagmus
Persistent anterior fetal vasculature
Persistent posterior fetal vasculature
Visual prognosis depends on amount of
microphthalmia and involvement of posterior pole
Persistent anterior fetal vasculature
 Retrolental mass with
elongated ciliary
processes
 Advanced cases ass:with
Cataract formation
Persistent posterior fetal vasculature
 Confined to posterior
segment
 Dense white membrane
or prominent retinal fold
extends from optic disc
to ora serrata,ass:with
retinal detachment.
COATS DISEASE
 Idiopathic retinal vascular
talengiectasia with
intraretinal and sub retinal
exudation and retinal
detachment
 Unilateral
 Seventy-five percent are
male
 Presents in 1st
decade(avg:5yrs) with
unilateral visual loss,
strabismus and leucokoria
Ocular Toxocariasis
 infestation of dog with Toxocara canis
 Human infestation:accidental ingestion of soil or food
contaminated with ova shed in dog faeces
 Very young children who eat dirt or are in close contact
with puppies are at risk
 In human intestine ,ova develop into larva ,penetrate
intestinal wall and travel to various
organs.liver,lungs,skin,brain and eyes.
 Larva die,disintegrate and cause an inflamatory
reaction and granuloma formation.
Ocular Toxocariasis
 Presents as strabismus,
leukocoria or unilateral
visual loss
 Ch:Endophthalmitis:
(2-9yrs)mey cause cyclitic
membrane and white
pupil.
Posterior pole granuloma
in an otherwise quiet
eye.(6-14yrs) may
resemble endophytic Rb.
Coloboma of Choroid/Optic disc
 Incomplete closure of the
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embryonic fissure
Unilateral /bilateral
Sharply circumscribed,
white area devoid of blood
vessels in the inferior
fundus
Large Coloboma may
involve the disc and give
rise to leucokoria
Complication: Retinal
detachment
Retinal Dysplasia
 Faulty differentiation of retina and vitreous
 Isolated or ass:with systemic conditions such as Norrie
disease and incontinentia pigmenti
 Presents with congenital blindness with roving eye
movement
 Pink or white retrolental masses resulting in
leucokoria
 microphthalmos,shallow anterior chamber and
elongated ciliary processes
Retinal Dysplasia
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Norrie disease:
XL reccessive
Males are blind at birth or infancy
Sys:cochlear deafness,mental
retardation
Incontinentia pigmenti
XL Dominent
Affecting girls and lethal in utero for
boys
One third children develop retinal
detachment in 1st yr of life
Vesiculobullous rash on trunk and
extremities
Malformation of
teeth,hair,nails,bones and CNS
Conclusion
 Family physician play crucial role in the management
of eye problem in children
 Vision screening even with limited equipments can
identify most important causes of visual loss
THANK YOU