Pediatric Ophthalmology
Julie M. Lange, MD
Assistant Professor
Division of Pediatric Ophthalmology
Julie.Lange@osumc.edu
Primary Learning Objective
 Recognize important preventable and treatable causes
of blindness or vision loss in infants and children
Secondary Learning Objectives
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define strabismus
define amblyopia
identify causes of amblyopia
define esotropia and exotropia
define suppression
understand the basis for treatment of amblyopia,
strabismus and refractive error in childhood
Secondary Learning Objectives
(continued)
 interpret tests of ocular alignment including corneal light
reflex and cover-uncover testing
 recognize pseudostrabismus
 interpret abnormalities of the red reflex
 describe retinopathy of prematurity
 recognize ophthalmologic features of child abuse
 list common causes of leukocoria
 identify abnormal ocular motility disturbances presenting
in childhood
Red Reflex testing
 This is a basic test easily done by any practitioner.
 A direct ophthalmoscope is used to assess the red reflex
in each eye. The examiner is looking for any opacities,
irregularities or asymmetries in the red reflex. ANY
abnormality needs to be referred to an ophthalmologist.
Examples of abnormal red reflexes
Retinoblastoma
Cataract
Congenital polar
cataract
dislocated lens
Anisometropia: unequal refractions
Vision Screening Terminology
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“Blinks to Light”
“Fix and Follow”
Children’s Figures
HOTV chart
Snellen chart
Vision Screening Terminology
Blinks to Light
 This term is used mostly to describe vision in newborn
eye exams.
 An infant is expected to blink to light at birth, but does
not usually show consistent fixation behavior until 2-3
months of age.
Fix and Follow
• By 4 months of age, most infants will fixate on an object
and be able to follow it.
• When checking fixation, have the patient fixate on an
object with 1 eye at a time. It is best to use an object,
rather than a light, to test vision because even a person
with low vision can sometimes see lights well.
Vision Screening Terminology
Children’s Figures
 A chart consisting of pictures such as a hand and a car
which are familiar to most children.
 Because the objects have very different shapes and are
easily differentiated, this chart is not ideal.
HOTV Chart
 A chart consisting of only 4 letters (H, O, T, V). It is often
used in conjunction with a hand-held chart which the
child can point to in order to indicate the letter on the wall
chart. This is useful in preverbal children and in those
who do not yet know the alphabet.
Vision Screening Terminology
Snellen Chart
 Snellen refers to the traditional eye chart consisting of a
variety of letters which is read aloud. This is the most
accurate chart to use, but its use is obviously limited by
the age and literacy of the child.
Refractive Error
 Every child has a refractive error. If the refractive error is
high, glasses are warranted.
 Children have the ability to accommodate much more
than adults, therefore small amounts of symmetric
hyperopia are not corrected.
 Also, small amounts of myopia are not usually corrected
due to the fact that the majority of a child’s daily activities
are near tasks.
 Glasses can be given as early a 6 months old and can
prevent and/or treat amblyopia.
 Amblyopia= less than normal
best-corrected vision in 1 or both eyes.
Causes include:
 Large refractive error in both eyes
 Difference in refractive error between the 2 eyes is called
anisometropia. The more blurred eye will actually lose vision
because the child starts to prefer the better seeing eye and
only continues to develop vision in that eye.
 Strabismus or misalignment of the eyes. The child will prefer
1 eye while the more frequently deviated eye will lose vision.
This is done to avoid diplopia which is inevitably the result if
the eyes are misaligned.
 Structural abnormality of the eye can prevent a clear image
and therefore proper visual development (cataract, corneal
opacity, vitreous opacity, etc.)
Amblyopia Treatments
 The first step is to put the child in the proper glasses if a
significant refractive error exists
 Patching of the better seeing eye several hours per day
in order to force the use of the weaker eye
 Cycloplegic eye drops can be used to blur the better
seeing eye temporarily
 In cases of a structural abnormality, it is sometimes
possible to address the problem directly (cataract
surgery, corneal transplant, etc.) These patients often
need patching after their surgery as well.
Amblyopia Treatments
 Amblyopia treatments work best when implemented
early. Around the age of 9 years old, a child’s bestcorrected vision is cemented.
 If not treated, amblyopia can cause blindness.
 If significant strabismus exists once equal vision is
obtained, eye muscle surgery is warranted.
Patching is
the most
common
treatment
for
amblyopia
Checking eye movements
 Oculomotor exam
-Fixation with each eye individually
-Range of movement of each eye
-Steady? Look for nystagmus
-Straight? Look for strabismus
-Don’t forget to look at the pupils and eyelids
Ocular motility disorders that may
present in childhood
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Nystagmus
Cranial Nerve 6 palsy
Convergence Insufficiency
Strabismus
Esotropia
Exotropia
Hyper/Hypotropia
Nystagmus
 Nystagmus is a term to describe fast, uncontrollable
movements of the eyes.
 1. Infantile: present at or soon after birth, can be
associated with eye disease. Visual acuity in these
patients is often nor correctable to 20/20.
 2. Acquired:
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Secondary to medication
Head injury
Inner ear disorder
Stroke
B12 deficiency
Multiple sclerosis
Brain tumors
Cranial Nerve 6 Palsy
 CN 6 controls the lateral rectus extraocular muscle
 In a CN6 palsy, there will be limitation to abduction in 1
or both eyes and a misalignment of the eyes in certain
gazes
 This can be congenital or acquired
 It can be secondary to increased intracranial pressure or
recent head trauma
 This warrants urgent referral to an ophthalmologist
and/or ER
Look for abnormal head positions
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This can indicate
A null point for nystagmus
An avoidance of diplopia in CN 6 palsy
Eye preference in amblyopia
Hypertropia in head tilts
Strabismus
 Orthophoric: describes eyes that are aligned.
 Strabismus is a misalignment of the eyes. It can be
constant or intermittent. Strabismus is not normal and
should be taken seriously.
Strabismus
Esotropia
(ET)
eye drifts
inward
Exotropia
(XT)
eye drifts
outward
Corneal light reflex testing
 Assess alignment of eyes by having patient look at a
penlight. The light reflex should fall within the pupil
bilaterally.
 If the light reflex is asymmetric in 1 of the eyes, a
misalignment may be present.
The light reflex on the child’s left eye shows
the eye is turned inward (esotropia)
 Orthophoric: eyes aligned
 Esotropia: L eye turned in
 Exotropia: L eye turned out
 Hypertropia: L eye drifted
upward
Alternate Cover Testing:
3 Steps to Detect Eye Deviation
 Step 1: The “Cover Test"
 Cover one eye while the patient fixates on an object (ie,
toy, parent) During and after placement of the cover,
the uncovered eye is observed for any movements
that indicate the need to refocus.
Alternate Cover Testing:
3 Steps to Detect Eye Deviation
 Step 2: The “Uncover Test"
 Cover one eye, but focus the examination on the
covered eye once the cover is removed. If the justuncovered eye moves to regain focus, a strabismus is
present.
Alternate Cover Testing:
3 Steps to Detect Eye Deviation
 Step 3: The “Alternate Cover Test"
 Occlude each eye in an alternating manner. If the
affected eye moves in an attempt to regain fixation, a
strabismus is present.
Cover Testing
 http://www.richmondeye.com/eyemotil.asp
 This is an interactive webpage where the user can
cover and uncover the eyes on the screen to see
fixation/refixation movements indicating strabismus.
Pseudostrabismus
A false appearance of strabismus. It is most
commonly seen in infants and toddlers who
have a wide nasal bridge or epicanthal folds.
These facial features can partially cover the
medial part of each eye giving the appearance
that the eyes are crossed inward when actually
upon testing, the eyes are orthophoric.
Epicanthal folds causing pseudostrabismus
Pseudoesotropia
look at the
light reflexes
Pseudoesotropia
 When testing a child’s fixation. One should always be
looking for eye preference. Children should be able to
use either eye and not have a strong preference. If a
preference is noted; this is a sign of amblyopia.
 When strabismus is present, preference is easier to
assess. The examiner should make certain the child
can fixate and maintain fixation with either eye.
 When strabismus is not present, the examiner must
occlude each eye separately and test the fixation and
ability to maintain fixation. Children who prefer one eye
will strongly object to cover of that eye.
Congenital Esotropia
Congenital Esotropia
 Arises before 6 months of age
Typical characteristics include:
 Large and constant
 alternating/cross fixation
 not significantly hyperopic, usually normal refractive error
 full abduction
 possible nystagmus, often the latent type
Cross-fixation in
congenital ET
Esotropia
 Esotropia arising after 6 months of age is usually
classified as accomodative or non accomodative.
 Accomodative: patient has a high hyperopic
prescription and the eyes are aligned in glasses
 Non-Accomodative: patient has low refractive error and
glasses do not significantly change the alignment.
Esotropia
 Esotropia is often associated with amblyopia and needs
be treated right away.
ESOTROPIA
Esotropia much improved in glasses.
Therefore, this is Accomodative
Esotropia.
Bifocals are sometimes used to add more plus
lenses to the bottom of the existing prescription to
help with more esotropia at near.
Exotropia: temporal deviation of the eyes
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XT
Less risk of amblyopia
Often begins as intermittent with some control
Can progress and become more constant
EXOTROPIA
Exotropia
 Exotropia is sometimes helped my myopic (nearsighted)
glasses, but large deviations usually require strabismus
surgery to correct.
Suppression
 If strabismus arises in a child with binocular vision, the
child experiences diplopia. Therefore, the child will
usually suppress one eye as an adaptation to avoid
diplopia. This often leads to Amblyopia.
Strabismus surgery
 Basic concept is to weaken or strengthen an eye
muscle
Methods to weaken:
 recess (move the insertion of a muscle posteriorly)
 insert a spacer into the muscle therefore effectively
making the muscle longer
 Botox
 disinsertion
Methods to strengthen:
 resect, tuck, advance
Methods to weaken muscle:
 Recess: move the insertion of a muscle posteriorly
 insert a spacer into the muscle therefore effectively
making the muscle longer
 botox
 disinsertion
Methods to strengthen:
 resect: excise part of the muscle, thereby making it
shorter
 Tuck: essentially folding a segment of muscle on itself
and securing it in place, thereby tightening it
 Advance: moving the insertion anteriorly
A recession (weakening) of the
medial rectus is the surgery most
often performed for the correction of
non-refractive esotropia.
It is done by disinserting the muscle
from the globe and then reattaching
it to the globe posterior to its original
insertion.
1. An incision is made through
conjunctiva and tenons fascia. The
desired eye muscle is then located with
a muscle hook.
2. Here the medial rectus muscle is isolated with a
muscle hook and then cleared of connective tissue.
3. The muscle is then secured with a double armed suture just
anterior
to its insertion onto the globe.
4. The muscle is then excised from the globe with the sutures
preventing loss of the muscle.
5. Calipers are then used to measure and mark a predetermined
distance from the original insertion of the muscle.
The muscle is then reattached to the globe using the double-armed
suture already in place to make partial-thickness scleral passes at the
marks made by the calipers.
Suture are then tied and cut. The conjunctiva is re-approximated and
sometimes sutured.
The same procedure is often performed on the other eye.
Strabismus Surgery Video
http://webeye.ophth.uiowa.edu/eyeforum/video/PediatricOp
hth-Strabismus-vids/medial-rectus-recession.htm
Retinopathy of Prematurity
 Retinopathy of prematurity (ROP) is a potentially blinding
eye disorder that primarily affects premature infants
weighing 1500 grams or less or with a gestation of less
than 30 weeks.
ROP
• Retinal blood vessels begin to develop approximately 3
months after conception and usually complete their
development before the birth of a full term infant.
• If an infant is born very prematurely, eye development is
often not complete and the growth of the retinal vessels
may be disrupted. The vessels may stop growing or grow
abnormally from the retina into vitreous. These abnormal
vessels are very fragile and bleed easily.
Retinopathy of Prematurity
ROP
 These babies are at high risk of retinal detachment.
ROP can cause blindness. ROP exams are done on all
babies who fall within the risk guidelines. Because there
are no external signs of this diagnosis, parents need to
be reminded of the importance of these exams.
ROP
 Progression of ROP can lead to a retinal
detachment. The scar tissue associated with the
fibrovascular ridge contracts, pulling the retina
away from the wall of the eye. Eyes with retinal
detachments due to ROP are very difficult to
operate on and these babies often have poor visual
outcomes.
 Depending upon the stage and zone of the ROP,
treatment may be indicated in an attempt to prevent
vision loss. The gold standard treatment is laser. It is
used to photocoagulate the avascular retina to eliminate
the hypoxia which drives the abnormal vessel growth.
Laser spots are applied confluently to the avascular
retina between the ridge and the ora serrata.
 Over the last 5-6 years, physicians have begun injecting
anti-VEGF molecules into the eyes of babies with severe
ROP. Their retinal vascular abnormalities often
significantly improve after the injection. This is an offlabel use of intravitreal bevacizumab. This drug is most
commonly used for exudative macular degeneration.
 However, there are still many questions surrounding
bevacizumab It has many potential side-effects and our
understanding of the short- and long-term effects of
VEGF inhibition in newborns is extremely limited.
 The implications of VEGF blockade in an infant in whom
physiologic VEGF is required for normal organogenesis
are not known.
Leukocoria
 Leukocoria, literally "white pupil”, occurs when the pupil
is pale yellow or white rather than the usual black
Common Causes of Leukocoria
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cataract
retinal detachment
retinopathy of prematurity
retinal malformation
intraocular infection (endophthalmitis)
retinal vascular abnormality
intraocular tumor (retinoblastoma).
Retinoblastoma
 Most common primary intraocular malignancy of
childhood
 1/15,000 live births
 3% of all childhood cancers
RB
 Retinoblastoma is a rare type of eye cancer that arises
from the retina and develops in early childhood.
 In most children with retinoblastoma, the disease affects
only one eye. However, 1 out of 3 children with
retinoblastoma develops cancer in both eyes.
 The most common first sign is leukocoria. This white
reflex is often noted in photographs. Other signs of
retinoblastoma include strabismus, eye pain, and eye
inflammation
 If diagnosed early, retinoblastoma is often curable.
However, if not treated promptly, it can spread beyond
the eye to other parts of the body and be life-threatening
RB
 When retinoblastoma is associated with a gene mutation
that occurs in all of the body's cells, it is known as
germinal retinoblastoma. People with this form of
retinoblastoma have an increased risk of developing
other life-threatening cancers such as pinealoma,
osteosarcoma, and melanoma.
Endophytic RB
grows from retina into vitreous
causing vitreous seeds
Exophytic RB
grows outward from retina
resulting in a mass beneath
the retina
Presentation of Retinoblastoma
 60% leukocoria
 20% strabismus
 Chronic uveitis
Child Abuse
 Suspicion should be aroused when apparently traumatic
physical exam findings do not match with the history
given by the caretaker (like multiple fractures on the face
and head after a single short fall). Also, if the history
does not match the developmental age of the child (like
a 1 month old rolling off a bed or a 8 month old climbing
out of a crib or high chair).
 Any physician who suspects abuse may have occurred
is required by law in every U.S. state to report it to the
proper authorities.
Ocular Signs of Child Abuse
hyphema
Characteristic petal-shaped
cataract from trauma
Subconjunctival
hemorrhage
Shaken Baby Syndrome
(Abusive Head Trauma)
• Shaken baby syndrome (SBS) is a triad of
medical symptoms:
1. subdural hematoma
2. retinal hemorrhage
3. brain swelling
•
•
These findings lead doctors to infer child
abuse caused by intentional shaking.
In a majority of cases there is no visible sign
of external trauma.
Shaken Baby Syndrome
(Abusive Head Trauma)
• SBS is often fatal and can cause severe brain
damage, resulting in lifelong disability.
• Estimated death rates (mortality) among infants
with SBS range from 15% to 38%. Up to half of
deaths related to child abuse are reportedly due to
shaken baby syndrome.
• Nonfatal consequences of SBS include varying
degrees of visual impairment (including blindness),
motor impairment (e.g. cerebral palsy) and
cognitive impairments.
Extensive retinal hemorrhages found in multiple layers of the retina and in all 4
quadrants. A large schisis cavity (hemorrhagic cyst) is seen overlying the
• A child with abnormal eye findings should
have a dilated eye examination.
• It is safe to dilate a baby’s eyes at any age.
Thank you for completing this module
Questions? Contact me at:
Julie.Lange@osumc.edu
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