Paediatric Ophthalmology
Glen Gole
Dept of Ophthalmology,
Royal Children’s Hospital, Brisbane
Dept of Paediatrics and Child Health,
University of Qld
[email protected]
VISUAL ACUITY
Defn: The resolving power of the eye
SHERIDAN GARDINER TEST
Visual Development
• Requires clear images and aligned eyes
during early childhood
• The two eyes compete with one another from
birth to make connections on the cortex
• Vision at birth 6/480, 6/36 at 6 mos, 6/9 at
age 3
• Fusion and stereopsis develop at about 4
mos of age (coincident with foveal
maturation)
• Can occur only up to age 8 years
Amblyopia
• Amblyopia: Poor vision due to abnormal
visual experience in early life
• Prevalence is about 3%
• Prevalence is decreased when screening and
early intervention are instituted (supported by
RCTs)
• Prevalence is higher in medically
underserved populations
ANISOMETROPIA
• A difference in the refractive
error of the two eyes
• Can be easily detected
using the Bruckner reflex
STRABISMUS (SQUINT)
SQUINT
DEFN:
Misalignment of the Eyes
TERMINOLOGY:
Eso
Convergent
Exo
Divergent
-Phoria
A Latent Squint
(held in place by fusion)
-Tropia
A Constant Squint
AMBLYOPIA
REFRACTIVE
ERRORS
SQUINT TYPES
•
Esotropia: A constant convergent squint (crossed eyes)
•
•
•
Exotropia: A constant divergent squint
•
•
•
a) Infantile Esotropia
b) Acquired Esotropia
a) Intermittent
b) Constant
Others
•
•
•
a) IV N palsy
b) III N palsy
c) Vertical squints
Infantile Esotropia
•
•
•
•
•
Not truly congenital
Onset usually between four and six months of age
Typically have moderate to large angle of squint
Treatment is surgical, anytime after six months
Early surgery=better chance for binocularity
DETECTION OF SQUINT
•
•
•
•
•
External Appearance
Asymmetry of Corneal Light Reflexes
(Hirschberg Test)
Cover Test
Bruckner Test
Amblyopia
Corneal light reflections
(reflexes)
BRÜCKNER REFLEX
•
Comparison of fundus red reflexes when viewed simultaneously at
arms length through direct ophthalmoscope
•
Good for detecting small angle squints and amblyopia
•
Sensitivity 86% and specificity 68% for detecting amblyopia risk factors
•
In children with amblyopia, 95% sensitive
MANAGEMENT OF SQUINT
•
•
•
•
•
Correct Diagnosis of Type
Exclude of Treat Systemic or Ocular
Disease
Correct Refractive Errors
Treat Amblyopia
Surgery
Strabismus Management
•
•
Necessity for follow up throughout
childhood
If glasses don’t straighten eyes,
surgery is necessary in order to
maximise binocular potential
Aims of Strabismus
Surgery:
To allow binocularity to develop
To restore binocularity
To improve appearance
Measuring the misalignment
Ophthalmia Neonatorum
Any severe conjunctivitis in the
newborn period
Aetiology
•
•
•
•
Bacterial - N Gonorrhoeae
- Ps aeruginosa
- Staph aureus
- Str pneumoniae
- H influenzae
Chlamydia
Viral-HSV type II
Chemical
Investigation
•
•
•
Conjunctival swabs (culture/Gram stain
Conjunctival scrapings (Chlamydia PCR)
Immunofluorescent studies (HSV/Chlamydia)
Blockage of the
Nasolacrimal Duct
CHILDHOOD GLAUCOMA
Infantile Glaucoma
• Affects 1/8-10,000 children
(M East 1/2,500)
• Primary congenital
glaucoma usu sporadic
80%>recessive, rarely
dominant
• 60% present by 6/12,
80% by 1
• 2/3 male, 2/3 bilateral
• GLC3 gene-2p21, 1p36
End Stage Infantile Glaucoma
LEUKOCORIA
LEUKOCORIA
(WHITE PUPIL)
•
•
Commonest cause - Congenital cataract
Must always exclude Retinoblastoma
CONGENITAL CATARACTS
•
•
•
•
Hereditary
Metabolic
Infectious
Systemic Disease
Early Surgery
• Early surgery definitely gives
better results
• Operate around 6-8 weeks
• Allows time for optical
correction before amblyopia
develops (12 weeks)
• But surgery before 4 weeks
increases glaucoma risk
Optical Correction
•
•
•
•
•
•
Glasses-poor results
Contact lenses- currently best option
70-80% of bilateral aphakes-6/18 or better
50% on monocular aphakes-6/18 or better
Not for “dull”, diabolical, distant or dirty”
Allow changes in lens power
•
IOL’s-not for infants
Every Newborn Child
should have the Fundus
(Red) Reflex examined
before discharge from the
nursery.
Retinoblastoma
• Malignant eye cancer
• occurs 1/18,000 live
births
• Often results in loss of
eye if tumour growth
can not be controlled
• many tumours now
treatable with laser
RETINOBLASTOMA
PRESENTATION
RETINOBLASTOMA
• Leukocoria
• Strabismus
• Others
• Recessive Gene
• ‘Two Hit’ Hypothesis
• Dominant Inheritance
RETINOBLASTOMA
• Multiple Tumors in One Eye
• Bilateral Involvement
• One Tumor in One Eye
Germinal
Mutation
Somatic
Mutation
Non-Accidental Injury
(NAI)
NAI
•
•
Inconsistent history
History inadequate to explain degree of
injury
Childhood Blindness
•
•
•
•
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Childhood Blindness
Third World
•
1.5 million blind
•
75% of causes preventable or curable
Half million new cases per year
•
60-80% die within two years of blindness •
•
•
Developing World-mostly preventable
measles, xerophthalmia
Developed World
Cerebral vision impairment
Retinopathy of prematurity
optic nerve hypoplasia
PAEDIATRIC LOW VISION CLINIC-QUEENSLAND
List of Major Causes of Low Vision (%)
1974-81
(N=468)
1982-86
(N=283)
1986-89
(N=295)
1990-92
(N=221)
1993-94
(N=125)
1996-99
(N=242)
2.4
9.9
14.2
24.9
21.5
31
4.1
5.6
4.8
4
21.5
7.4
Albinism
7.8
7.8
7.8
7.6
10.8
7.8
Optic Atrophies
11.3
11.3
11.9
5.3
9
9.1
Retinopathy of Prematurity
Maculopathies
(incl Stargardt’s Disease)
2.6
2.8
5.8
5.8
9
4.9
3.9
2.1
5.4
4.9
4.5
2.1
Retinitis Pigmentosa
2.6
0.7
1.4
4
4.5
4.9
Coloboma
0.9
1.8
2.4
3.1
3.6
2.5
Optic Nerve Hypoplasia
1.9
3.1
5.1
3.1
3.6
2.4
Congenital Cataracts
15.8
10.2
11.9
3.1
2.7
1.2
Myopia
1.7
3.1
3.6
6.2
2.7
4.1
Nystagmus
Retinopathies
(incl. Lebers, rod monochromatism
16.5
17
8.1
13.8
1.8
8
1.9
2.8
3.4
1.8
1.8
8.6
Ectopia Lentis
4.5
3.2
3.4
1.3
Rubella
Others
8.4
13
2.2
16.3
1
10.5
0.4
10.5
Condition
Cortical Vision Impairment
(incl. Hemianopia)
Aniridia/structural defects
(incl. aphakia)
1.2
12.5
3.3
Poor Vision in Childhood
Apparently Blind Child
Presentation:
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•
•
•
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Failure of visual development
Family history
Nystagmus
Strabismus (squint)
White pupil
•
•
•
•
•
Anterior Visual Pathway Disease
(Nystagmus)
Posterior Visual Pathway Disease (CVI)
Delayed Visual Maturation
Generalised Developmental Delay
Autism
Cortical Vision Impairment
• Poor vision due to damage or non
development of the occipital cortex
• Now commonest cause of impaired
vision in childhood (30% in Qld)
• Largest subgroup is premature infants
• However, cortex is only rarely damaged
in isolation
Neural Development
Cerebral Vision Impairment-a better
term
• Poor vision due to damage or non development of
the visual areas of the brain (40% of brain has some
visual function)
• More accurately reflects the often widespread nature
of injury to the visual system, not just cortex
• Dutton GN, Jakobsen LK
Cerebral visual impairment in children
Semin Neonatol 2001Dec;6(6);477-485
Child with Poor Vision
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Acute intervention (PLVC)
Mobility
Low Vision Aids
Counselling for Parents
Medicolegal issues
Whole of life cost $1m
Visual Problems in Premature Infants
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Cerebral Vision Impairment
Retinopathy of Prematurity (ROP)
Amblyopia
Refractive Errors
Strabismus
Glaucoma (acute and late after ROP)
Visual Problems in Premature Infants
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•
•
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Cerebral Vision Impairment
Retinopathy of Prematurity (ROP)
Amblyopia
Refractive Errors
Strabismus
Glaucoma (acute and late after ROP)
Retinopathy of Prematurity (ROP)
A retinal vascular disorder
characterized by abnormal
vasoproliferation in the retina of
premature infants.
Retinal Vascularization
• Begins at 16 wks
gestation
• Usually complete by
36-38 wks gestation
• Proceeds from disc as
wave of mesenchymal
cells which then lays
down the primitive
capillary network
Pathogenesis of ROP
L. MacKeene - Toronto
41
L. MacKeene - Toronto
42
Retinopathy of
Prematurity
• Laser cures over
90% of affected
infants
• Diode laser can be
used in nursery
• Blindness now <1%
of premature infants
Diode Laser treatment of Zone 1 ROP
Retinopathy of Prematurity
• The only children now developing severe
ROP are extreme premature infants (eg 17
weeks premature) who are likely to have
multiple handicaps
• We now understand many of the underlying
molecular mechanisms
• Treatments to slow blood vessel growth by
non surgical means will be developed within
the next few years
Gene Therapy in Ophthalmology
• 87 eye diseases caused by a specific genetic
defect- 20 genes have been identified
• Many of these disorders affect children eg
retinal dystrophies such as Lebers amaurosis
• Inject good gene into the eye eg on a virus
carrier
• Transplant into eye genetically modified cells
• Animal and human trials are underway
• Retinitis pigmentosa may become a treatable
disease within a decade
WHEN TO REFER TO AN
OPHTHALMOLOGIST
• any sight threatening condition
• If unsure about diagnosis
• if condition is not responding as
it should
• when encountering an
uncommon condition for the first
time
• any child with a white pupil
Your professional tasks
• Have a successful career and keep your
important relationships together-spouse,
children, friends.
• Medicine is a long, demanding career, pace
yourself so you have something left for
yourself at the end.
• Look after your colleagues and yourselfdrugs, alcoholism, depression and suicide
are professional hazards. None of us is
bulletproof-smell the roses when you can.
Retinopathy of Prematurity (ROP)
A retinal vascular disorder
characterized by abnormal
vasoproliferation in the retina of
premature infants.
Retinal Vascularization
• Begins at 16 wks
gestation
• Usually complete by
36-38 wks gestation
• Proceeds from disc as
wave of mesenchymal
cells which then lays
down the primitive
capillary network
Pathogenesis of ROP
L. MacKeene - Toronto
41
L. MacKeene - Toronto
42
Retinopathy of
Prematurity
• Laser cures over
90% of affected
infants
• Diode laser can be
used in nursery
• Blindness now <1%
of premature infants
Diode Laser treatment of Zone 1 ROP
Retinopathy of Prematurity
• The only children now developing severe
ROP are extreme premature infants (eg 17
weeks premature) who are likely to have
multiple handicaps
• We now understand many of the underlying
molecular mechanisms
• Treatments to slow blood vessel growth by
non surgical means will be developed within
the next few years
VISUAL FUNCTIONING IN CVI
• acuity fluctuates
• notices moving objects more
than static objects
• sees better in familiar
environments
• lacks visual curiosity
• tires easily during visual activities
RECOVERY FROM CVI
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•
•
•
•
Light perception
Colour vision
Movement
Form perception
Acuity
Retcam Screening
Very useful for telemedicine
Gene Therapy in Ophthalmology
• 87 eye diseases caused by a specific genetic
defect- 20 genes have been identified
• Many of these disorders affect children eg
retinal dystrophies such as Lebers amaurosis
• Inject good gene into the eye eg on a virus
carrier
• Transplant into eye genetically modified cells
• Animal trials are underway
• Retinitis pigmentosa may become a treatable
disease within a decade