Causes of visual impairment in children

What not to miss in paediatric
eye care
Louise Allen MD FRCOphth
Consultant Paediatric Ophthalmologist
Cambridge University Hospitals NHS Trust
Childhood SVI in UK
6/36
• Prevalence:
15/10,000 in kids <16
years in UK
• Most cases are
diagnosed in 1st year
of life
• Based on visual
acuity / visual fields
6/60
Causes of VI in UK
27% AVOIDABLE / TREATABLE
• Cataract (5%)
• Glaucoma (3%)
• Uveitis (2%)
• Refractive error (2%)
• Raised ICP (4%)
• ROP (3%)
• Non-accidental injury (3%)
• Retinoblastoma (1%)
• Diabetes (1%)
73% UNAVOIDABLE
• Cerebral visual impairment
(50%)
• Retinal dystrophies (10%)
• Optic nerve anomalies (5%)
6-8 week check
The 6-8 week eye check
Test
• Visual responses to your
face and large toy
• Steady fixation
• Eye movements
• Corneal clarity and size
• Red reflex
To exclude
• Problems with visual
development
• Nystagmus
• Microphthalmia, congenital
glaucoma
• Cataract, media opacity,
retinoblastoma
Questions
Ask
• Does the baby look at
mum’s face when feeding?
• Does the baby ever seem
visually alert?
• Do the eyes wobble?
• Does the baby light gaze
• Birth history
• Is there any FH /
consanguinity
Cause
• If not the vision is poor
• May suggest resolving
delayed visual maturation
• Nystagmus indicates neuroor ocular pathology
• Suggests ocular pathology
• ? Hypoxia / prematurity
• May suggest inherited
ocular pathology
Simple exam tips – red reflex
If the baby is restless, let them feed while you
examine them:
Use a bright ophthalmoscope (can set on +3)
Dim the room lights
Look for:
Shadows on the red reflex (partial cataracts)
Absent reflex (severe cataracts or media opacity)
White reflex
Assess behavioural visual function
• Does the baby have eye contact with mother?
• Does the baby fixate on your face?
• Will the baby fixate and follow a large, bright
toy?
• Is there an obvious squint?
• Is there nystagmus?
Assess behavioural visual function
• Does the baby have eye contact with mother?
• Does the baby fixate on your face?
• Will the baby fixate and follow a large, bright
toy?
• Is there an obvious squint?
• Is there nystagmus?
Look at the face
• Cranio-Facial anomalies
• Dacryocystoceles
• Naevus Flammeus
Look at the eyelids -torch
• Are the eyelids normal ?
• Are there any other risk
factors risks for eye
disease?
Look at the eye structure-torch
• Are the eyes a normal
size?
• Is the pupil round?
• Clarity of corneal light
reflection
Look at the pupils - torch
• Is the pupil round and
central?
Examine the red reflex
Which is the odd one out?
a)
c)
b)
d)
Answer-d)
• A)
– Nuclear cataract
(commonest type with
Down syndrome)
• B)
– Anterior polar cataract
• C)
– Lamellar type cataract
(often familial)
• D)
– White reflex due to
retinal coloboma
– NB vertically oval pupil
Poor vision at 6-8 week check?
• Urgent referral – fax local eye unit and chase
– Babies with poor red reflexes ( very tight window
of opportunity)
– All babies with nystagmus
• Review in primary care:
– Babies with normal exam, good RR, no FH, normal
development: likely to be delayed visual
maturation, review at 12 weeks of age, if no
improvement refer to clinic for investigation
Outcomes from the 6-8 screen:
• Abnormal eye examination
• Urgent referral to paed ophth
• Concerns about vision (but no
nystagmus), + Risk factors but normal
examination
– Routine referral to paed ophth
• No risk factors, normal examination
– Pre-school screening
• Unsatisfactory examination: review 1-2
weeks
Problems with screening
• 35% congenital cataracts are identified at
newborn check
• Additional 12% at the 6-8 week check
• Newborn screening misses 50% of congenital
cataracts
• Diagnosis is delayed until poor visual
development is identified, 33% >1 year of age
EXAMINATION TECHNIQUES IN KIDS
Examination in infants
Test
Why
• Fix and follow face and • Checking vision is OK
toy with each eye
• Look at eye movements • ? Full range, may need to
while doing this
hold head
• Is there nystagmus?
• Suggests poor vision /
ocular pathology
• Use ophthalmoscope to • Asymmetry suggests squint
assess light reflex
symmetry
• Examine red reflex
• Ensure no media opacity
Testing vision in children
• Snellen with matching sheet
• Helpful technology
– Isight app
– Kays pictures app
Doctor –my child has:
•
•
•
•
•
Sticky, wet eyes
Sore red eyes
Funny pupils on flash photography
“Lazy eye”
Lump on his / her eyelid
LACRIMAL PROBLEMS
Questions
ASK
• When did the discharge
start?
CAUSE
• Purulent discharge with
conjunctivitis =
ophthalmia neonatorum
• Do the eyes become red • CNLDO does not usually
cause red eyes
when sticky?
• Suggests CNLDO
• Are the eyes watering
too?
• Is the child sensitive to
• Consider corneal / uveal
light or in pain?
pathology
Examining the lacrimal system
Look at the tear film, is it
elevated?
Is the eye crusty with
discharge?
Is the eye normal – white,
good RR?
Is the child photophobic?
Massage the lacrimal sac –
any discharge?
Is the area overlying the
sac inflamed?
Examination findings
Feature
Cause
• White eyes with high
tear film
• Discharge with pressure
on the lacrimal sac
• Cornea clear and
normal size
• Is there a lump /
erythema at the site of
the lacrimal sac
• CNLDO
• CNLDO
• Try to exclude glaucoma
• Consider
dacryocystocele/
dacryocystitis
Management of CNLDO
•
•
•
•
•
•
Re-assure - >90% improve spontaneously
Don’t swab!
Show parent how to do lacrimal sac massage
Vaseline on lower lid
Only use topical antibiotics if the eye is red
Refer at one year of age if no better
SORE / PAINFUL RED EYE
History
Ask
• History of trauma
• History of previous episodes
• Duration
• Is the vision affected /
photophobia
• Is there discharge – what
type
• Are the symptoms getting
worse
• Medical history
Why
• ? FB or sub-tarsal FB
• ? Anterior uveitis
• ?More serious pathology
• Epiphora suggests abrasion
or FB, muco-purulent
suggests conjunctivitis
• ? Any link with uveitis
If blepharospasm prevents
examination instil:
Examination
Test
• VA assessment
• Is the eye white or red
• Is the discharge watery or
muco-purulent
• Instil fluorescein and
examine anterior segment
• Is injection circumcorneal or
forniceal?
• Examine red reflex
Why
• To exclude serious
pathology
• Red eye suggests FB,
conjunctivitis, uveitis
• Expect FB / abrasion with
watery discharge
• Look for abrasions, corneal
FB
• Will help to exclude serious
pathology,
Injection
Circum-corneal injection
• Corneal pathology
• Uveitis
Forniceal injection
• Conjunctivitis
Simple tips for removing FBs
Instil proxymetacaine prior to VA assessment
Set the ophthalmoscope on +20D to examine the
cornea
If you see a corneal FB, use a cotton bud soaked in
proxymetacaine to remove it
If you see linear abrasions on the superior cornea,
there is probably a sub-tarsal FB
Pre-septal cellulitis
– Normal motility
– No proptosis
If obvious infected bite/ cyst / pox /HZV, systemically
well. Rx with augmentin +/-aciclovir and keep a close
watch.
If unable to examine eye – refer urgently
If associated with URTI, high risk of progressing to
orbital cellulitis, Rx with augmentin, usually need
referral
Orbital cellulitis
• Orbital cellulitis
– Proptosis and
abnormal motility
– Secondary to URTI
/sinusitis
– Refer urgently
– Iv cephalosporin
(+metronidazole if
>8yo)
– Older children may
need abscess dainage
FUNNY PUPIL ON FLASH PHOTO
True leukocoria
• Causes
– cataract
– retinoblastoma
– retinal coloboma
• Refer urgently
Pseudo vs true-leukocoria
• Pseudo-leukocoria
– Flash reflects off ON in
one eye if looking 15
degrees of axis
– Anisometropia
– Squint – reflex looks
brighter in the
deviating eye
“LAZY EYE”:?PTOSIS ? SQUINT
Ptosis
Hx / Exam
• Congenital / acquired
• Compare pupil sizes
• Examine eye movements
• Examine corneal light and
red reflex
Why
• Congenital ptosis may be
neurological but more likely
to be due to levator muscle
dystrophy
• Neurological causes cause
anisocoria and / or
ophthalmoplegia
• Visually significant ptosis
will obscure light reflex
Ptosis
Possible squints in infants
Principle: Good vision in each eye is needed to
keep the eyes aligned!
• Neonates commonly have a variable angle
intermittent misalignment until the vision
develops
• Don’t worry too much at 6 week check unless
the squint is constant
• By 12 weeks the vision should be good and
eyes should be aligned – then worry!
Bruckner and Hirschberg test
• Are the corneal light
reflections
symmetrical?
• Compare the red
reflex
• Check eye
movements (esp full
abduction)
Krimsky test
Tips for childhood squints
• Usually concomitant, intermittent onset
• Causes:
– Due to brain injury (onset in infancy)
– Accommodative squints (onset 18mo-3 yrs)
– Divergent squints (onset usually over 3yrs)
– Sensory squint due to poor vision
Be wary of sudden onset or incomitant
squints with diplopia
Important points in the history
Ask
• When was it first noticed,
has it changed / become
more frequent since?
• How often is it noticed?
• Is it always seen in one eye
or does it alternate?
• Are there concerns
regarding vision?
• Birth and developmental
Hx?
• FH of squint / refractive
error?
Why
• Best outcome when squints
are still intermittent
• Establishes ability to control
• If alternating, less likely to
be amblyopic
• Poor vision causes squints
• ? Brain injury
• At higher risk of squint
Beware!!
• Acute onset squints
– Can result from intra-cranial pathology
– Check abduction, raised ICP causes bilateral VIs
– Check optic disc appearance
• Constant squints in young children
– Can result from poor uniocular vision secondary to
intra-ocular pathology eg unilateral cataract
• Always note if red reflexes are normal and
whether the squint is constant or incomitant
when referring
YOU CAN
• Prevent avoidable visual impairment in
children by
– Identifying cataract / glaucoma at baby check
– Recognising uveitis
– Identifying squint / amblyopia
• Prevent serious harm by
– Identification of raised ICP
– Orbital cellulitis