Pediatric Ophthalmology

Catherine Qui-Macaraig, MD
10 warning signs of treatable eye disease in the newborn
Needs early definitive treatment
•1. Leukocoria
•2. Drooping Eyelid
•3. Enlarged Cornea of one or both eyes
10 warning signs of treatable eye disease in the newborn and infant.

Serious but infrequent

•Cataract - 1 in 250,000
•Glaucoma - 1 in 10,000
•Retinoblastoma - 1 in 20,000

10 warning signs of treatable eye disease in the newborn and infant.

•Should be evaluated soon

•#4 Tearing, Discharge, Redness
•#5 Strabismus
•#6 Nystagmus
•#7 Prematurity - Low Birth Weight - requires monitoring of the retina for development
10 warning signs of treatable eye disease in the newborn and infant
Should be evaluated at next routine visit
•#8 Abnormal Head Posture
•#9 Pupil Defect
•#10 Anisocoria

The Bruckner Test

•View two eyes simultaneously through a direct ophthalmoscope
•Infant is upright and in a darkened room
•Detects medial opacities, refractive errors, strabismus, large posterior fundus lesions

1. White Pupil (Leukocoria)

Congenital Cataract
•1) Diagnosis must be early to avoid
- amblyopia
- nystagmus if bilateral
•2) Surgical treatment should be early
•3) Requires special surgical technique - different from adults.
•4) Optical rehabilitation is difficult with contact lenses or glasses.

•5) Any delay in treatment or lapse in optical or amblyopia treatment results in amblyopia.
•6) Even in the best of circumstances vision after congenital cataract treatment is never normal.

Congenital Cataracts
Bilateral Retinoblastoma
2. Drooping Lid

Lump or swelling of lids

•- this can be a sign of cellulitis, tumor, hemangioma, etc.
- amblyopia – from pupillary occlusion

Capillary Hemangioma
Marcus Gunn Jaw-Winking Phenomenon

Drooping of the lid (or lids)

•chin-up head back posture which retards motor development.
3 Enlarged Cornea of One or Both Eyes
Congenital glaucoma
•with tearing and photophobia.
• requires early and specialized surgical treatment including goniotomy, trabeculotomy, and
filtering
Congenital Glaucoma
•EARLY: - Epiphora
- Increase IOP
- Glaucomatous optic disc cupping.
Photophobia, loss of corneal luster

LATE:
•- corneal diameter greater than 10.5 mm
•- increased anterior chamber depth
•- corneal opacity

Congenital Glaucoma
•7 year old with microcephaly
•Cong. GL OU
•Goniotomy at 12 days
•Repeat surgery 2 weeks later on OS-
•Trabeculectomy-trabeculotomy

4 Excessive Tearing or Discharge

This can be a sign of
•glaucoma
•infection
•light sensitivity – from corneal problems, uveitis, etc.

all of which require attention.

4 Excessive Tearing or Discharge

•The most common cause is a blocked tear drainage system.
•treated initially with gentle pressure over the tear sac and antibiotic drops.
Symptoms
•Manifest by age 1 month
•Epiphora
•Sticky mucoid or mucopurulent discharge
•Bilateral involvement is common

NLD Obstruction
•gentle probing done before age 1 year.
•Persistent tearing usually requires probing with general anesthesia often with placement of a silicone
tube

5 Crossed Eyes

Eyes that deviate constantly should be evaluated immediately!

Ocular Alignment in Neonates
•Orthotropia 22.7 %
•Exotropia 61.1
•Intermittent Exotropia 13.0
•Esotropia 0.2
•Intermittent Esotropia 0.5
•ET to XT 2.5

•Majority are orthotropic by 6 months of age

Strabismus Evaluation
Cardinal Gazes
Cardinal Gazes
Hirschberg Test
Krimsky
Cover Test
•Cover-uncover Test – shows a manifest deviation e.g. esotropia

•Alternate Cover Test – shows latent deviations too e.g. exophoria

•Orthophoria- no shift on cover testing

Cover Testing
esotropia
Exotropia
Epicanthus
•Vertical folds of skin over the medial canthi
Pseudostrabismus
Pseudostrabismus
Who has crossed eyes?

6 Nystagmus
Dancing Eyes
-usually indicates subnormal vision
-also often present in cerebral visual impairment

7 Head Tilt, Turn,Chin up or Chin down

•Usu. starting at the time of sitting up or walking
Head tilting can be a sign of
•strabismus
•disease of the central nervous system
•an abnormality of the neck muscles
Head tilt regardless of cause should be evaluated by an ophthalmologist.
8 Defect or Missing Part of the Pupil
iris coloboma may be accompanied by retinal colobomata
9 Inequality of the Pupil Size
•Persistent inequality of the pupil size can be a sign of serious disease or it may be an innocent finding.
•If persistent, inequality of pupil size should be evaluated by an ophthalmologist.
10 Prematurity
AAP/ AAO Screening Recommendation
•a birth weight less than or equal to 1500 g
•GA less than or equal to 28 weeks.
also

ROP in Infants w/ BW >1250g

•ROP in 18% of 1118 preemies from 1250g to 1600g
•Stage 1 disease 11%
•Stage 2 disease 3%
•Stage 3 disease 3%
•Stage 4 disease <1%

•Gestational ages up to 32 weeks

Recommendation
•Screen 32 weeks and below
•Or < 1500g

Visual Acuity in Children

•Visual acuity assessment
•Conditions which predispose to amblyopia
Visual Acuity Testing
Testing Methods:
•Infants
•Pre-verbal children
•Verbal Children
Visual Acuity Assessment in Infants
Visual Acuity Assessment in Infants: Importance
•permits early, efficient detection of preventable or treatable causes of visual loss
•to monitor response to therapy
•provides clues to development in other neurologic systems
Normal Visual Development
•OKN- birth
•Ocular alignment stabilized- 1 month
•Fixation- 2 months
•Response to visual threat- 2-5 months
•Smooth pursuit- 6-8 weeks

Normal Visual Development (cont’d)

•Accommodation- 4 months
•Stereopsis- 3-7 months
•Foveal maturation- 4 months
•Contrast sensitivity- 7 months
•Optic nerve myelination- 7 months to 2 years

Age-related Visual Acuity Estimates by Test Method

Birth to 2 months
•Acuity at 20/400 to 20/60

Methods:
•Observation
response to light- blinks, flinches
fixes and follows objects
•OKN Drum
Clinical Assessment in the Nursery
•neonates fix and follow gross fixation targets
•human face is most attractive
•absence of fixation may mean lack of attention
•babies follow a full arc of 180 degrees only at 4 months
2 months to 6 months
•Acuity 20/50 +

Methods:
•Observation:
blink to threat
reach for objects
•OKN Drum

The Bruckner Test

•View two eyes simultaneously through a direct ophthalmoscope
•Infant is upright and in a darkened room
•Detects medial opacities, refractive errors, strabismus, large posterior fundus lesions

Clinical Assessment of Visual Function in Infants

•Lid Popping Responses
•Vestibulo-ocular Reflex
•OKN Drum
•Fixation Preference
•Preferential Looking Techniques

Lid Responses
•Suddenly reduce room light
•Upper lids should retract to expose 1 to 2 mm of sclera
•In newborn to 6 month old infants
•Absent in significant retinal or optic nerve abnormalities
•May be present in milder forms of visual loss

OKN Drum
•An involuntary pursuit response
•CF at 3 to 5 feet
•Pursuit and saccadic systems can be investigated
•Requires the child’s attention
•More for evaluation of movement disorders

Vestibulo-ocular Reflex (VOR)

•Spin child to induce a vestibular nystagmus
•Nystagmus should dampen in 30 to 60 seconds due to fixation
•Continued beating implies low vision
Vestibulo-ocular responses
Fixation Preference
•Most commonly used
•Known as the ‘csm’ method
•With a manifest strabismus, cover fixing eye for deviating eye to take up fixation
•With straight eyes, induce a tropia
Fixation Method: CSM
•Central: 20/20 to 20/200
•Steady: 20/200 or better

Nystagmus
•Wandering – poor prognosis
•Pendular – often secondary to central visual loss
•Jerk – more commonly of motor origin; with unexpectedly good vision in a compensatory head position

Preferential Looking Techniques

•Forced choice PLT assume the child will prefer to look at a patterned object
•Teller Acuity Cards-for clinical use

Visual Acuity
In Pre-verbal Children
Visual Acuity Assessment in Preverbal Children
•Sheridan-Gardner Test
•HOTV
•‘E’ Game
•Landolt C
•Lea Symbols
HOTV
•matching each test letter to one of the four letters H, O, T, and V printed on a card that can be
held in the child's hands

Illiterate E

•indicate with fingers the direction of the legs of a letter E that is rotated to point up, down, left or right

Snellen Equivalents
Lea Symbols
Lea Symbols
•more similar in configuration to Snellen letters than the Allen pictures
•carefully calibrated and assessed for reliability
Testing Procedure
•Position the child 20 feet from the chart
•Cover one eye
•Prompt her to read one line at a time
•Read until majority of responses on a line are erroneuous
•Acuity is recorded as the line above the last
Points to Consider
•Normal preschoolers often test no better than 20/30 or 20/40
•May do better on 2nd eye tested because of practice with 1st eye
•May do worse on 2nd eye tested because of waning interest or fatigue

Factors which affect acuity testing

•Short attention span
•Kids like to peek and memorize
•Lack of familiarity with the pictured objects

What to do
Short attention span
•Have several brief sessions beginning with alternate eyes
•Test only a few sample letters per line
•Record observations relating to the child's general behavior during testing as well as the numeric result
What to do
Kids like to peek and memorize
•Be constantly alert to peeking and guessing or memorization
•Hold the occluder yourself
•Test eye in question first

Lack of familiarity with the pictured objects

•Testing should begin with a review of the cards up close

Verbal Children:
Visual Acuity Testing

Snellen Acuity

•Numerator- testing distance in feet or meters

•Denominator- distance from which a normal eye can read the line

Basic Ophthalmologic Exam:

Visual Acuity Testing
Snellen Chart

•Number
- distance in feet or m from which a normal eye can read all the letters
Basic Ophthalmologic Exam:
Visual Acuity Testing
•Example:

20 testing distance
60 distance at which N eye can see same line
Subjective Acuity Assessment
•Motoric responses required
•Fixation preference persists in non-amblyopic patients
•Bilateral loss may be missed
•Requires highly skilled personnel
Objective Tests
•Motor response not required
•Less dependence on examiner
Visual Evoked Potentials

•Flash-presence of LP
-albinism testing
•Pattern Reversal VEP

• Sweep VECP

Flash VECP

Pattern Reversal VEP

•Supra-granular layers in striate cortex
•Highly reliable
•prolonged in MS, optic neuritis
•Macular disorders
•Affected in amblyopia
•Limitations: non-specific; time-consuming

Swept Spatial Frequency VECP

•VA estimate based on Cortical Electrical
Response
•VEP amplitude measured to reversing gratings
•Bar width changes from wide to thin
•32 bar widths tested in 10 second period (sweep)

Sweep (cont’d)

Pediatric applications:
•Acuity measured newborn through senescence
•Amblyopia
•Organic visual loss
•Motility restrictions precluding behavioral measures
Sweep (cont’d)
Limitations

•Fixate for ten seconds

•Electrode placements could be non-optimal
•Optimal temporal rate changes with age
Binocular VECPs
•Gross stereopsis develops by 3-5 months
•Correlates well with binocular fusion
•Documents effects of early surgery in infantile esotropia

Amblyopia
Definition
•Amblyopia is an acquired defect in monocular vision caused by abnormal visual experience early in life.
Amblyopia

•Decreased visual acuity

•Abnormal visual development
•Unilateral or bilateral
•Normal eye exam
Amblyopia
•The most common cause of visual impairment in children
•Also, the leading cause of monocular visual loss in the 20 to 60+ age group, surpassing GL, diabetic
retinopathy, macular degeneration and cataract

Pathophysiology of amblyopia
•No retinal changes - ERG OK
•Lateral geniculate layers subserving amblyopic eyes atrophic
•Cortical ocular dominance columns representing amblyopic eye less responsive to stimulus and show
changes microscopically
Clinical Behavior in Amblyopia

•Vision reduced - less than 20/40, or 2 lines difference

•Relatively better vision in reduced illumination (scotopic sight) compared to normal
•Slow reading

Vision reduced by crowding

Predisposing Factors
•Poor clarity – media opacities
•Poor focus - refractive errors
•Poor aim - strabismus
Deprivation Amblyopia
Unilateral Congenital Cataract
•More severe amblyopia

Bilateral Congenital Cataract

Strabismic Amblyopia
•Constant use of left eye causes suppression of right eye amblyopia

•Alternation with alternate suppression avoids amblyopia.

Anisometropic Amblyopia
•difficult to detect because there are no obvious physical signs
•usually only found at vision testing

Ametropic Amblyopia

Uncorrected high hyperopia is an example of this bilateral amblyopia.

Importance of Early Detection
•Vision develops early
•Treatment depends on plasticity of the visual system
Sensitivity to development and treatment
Visual maturity after this age, amblyopia is not likely to occur and in the usual case is not successfully
treated.
Amblyopia: Screening
•Amblyopia is preventable
•Early detection is key to effective treatment
•Screening is the responsibility of the pediatrician or primary care physician
Amblyopia
Catherine Qui-Macaraig, MD
Associate Professor
UST Faculty of Medicine and Surgery
Definition
•Amblyopia is an acquired defect in monocular vision caused by abnormal visual experience early in life
Amblyopia

•Decreased visual acuity

•Abnormal visual development
•Unilateral or bilateral
•Normal eye exam
Amblyopia
•The most common cause of visual impairment in children
•Also, the leading cause of monocular visual loss in the 20 to 60+ age group, surpassing GL, diabetic reti
nopathy, macular degeneration and cataract

Development of the Nervous System: Forces acting on epithelia

Driving forces:
Birth of cells distant from where they function
Migration
Death of excess cells

Modulating forces:
Cell specialization-migrates to where they function
Connectivity- synapses
Synaptogenesis
•The major activity going on in the brain at birth
•Brings the brain from infant to adult form
•The mechanism by which experience of the outer world molds and modifies the brain during the first few
years of life
Visual Development
•Retinal ganglion cells ---LGN---layer 4C of occipital cortex

•Visual experience produces a modular organization of columns in the cortex for orientation, binocular
disparity, motion direction, color, etc.
Visual Development
•Receptive field- a neural unit which transduces light energy into nerve energy and sends it to the cortex
–to be decoded by other receptive fields into the neural analog of the world as our eyes and brain see it
•-brain starts off as being relatively insensitive but becomes sharper like pixels

Visual Deprivation
•Hubel and Wiesel sewed one eyelid of a kitten shut
•atrophic LGN: cells of layers 2, 3, 5 of ipsilateral n. and cells of layers 1, 4, 6 of contralateral n.
•Layer 4C of cortex: shrinkage of input from deprived eye and expansion of opposite columns
•Change in LGN is attributed to loss of axons and terminals to support the cell bodies
Critical Period of Visual Development
•A period of rapid visual development
•When the visual system is sensitive to abnormal input caused by stimulus deprivation, strabismus, or
significant refractive errors
•Mixed data: 1st 2 months
3 – 4 yrs. depending on input manipulation

Amblyopia defined
•“lazy eye”
•A loss of visual acuity without an identified organic cause
•Abnormal visual development 2ndary to abnormal visual stimulation
•Practically, at least 2 snellen lines poorer than sound eye
Functional vs. Organic Amblyopia
•Organic
- caused by structural abnormalities of the eye or brain, independent of sensory input
Characteristics of Amblyopia
•Crowding phenomenon
•Neutral density filter effect
•Decreased contrast sensitivity
•Eccentric fixation
•Slow reading

Crowding Phenomenon
•Better VA with single optotypes than multiple optotypes
•Contour interaction
•One to two snellen lines better with isolated letters
•Clinically shows possibility of improvement in visual acuity with adequate therapy
•Neurons have large receptive fields; inc. spatial summation and lateral inhibition
Neutral density filter effect
•Reduces overall luminance
•In amblyopia, VA same as or better than sound eye
•In organic disease, VA worse with profound decrease
•More consistent in strabismic than anisometropic amblyopes
Decreased Contrast Sensitivity
•Central visual acuity – the smallest high contrast sensitivity which can be detected
•There is loss of high spatial frequencies which increases with the severity of amblyopia
•It represents a neural loss of foveal function
Eccentric Fixation
•Develops in patients with dense amblyopia
•A general area of viewing, not a single point
•Present in monocular and binocular conditions

Classification
•Strabismic
•Anisometropic
•Due to high refractive errors
•Deprivation
Strabismic Amblyopia
•Caused by constant unilateral suppression of cortical activity related to the deviated eye
•Usually in constant tropias
•Most common form
Anisometropic Amblyopia
•Usually identified through vision screening at school age
•Hyperopic and astigmatic – only 1 – 2 d can produce it
•Myopic >3d; -6d often results in severe visual loss
Amblyopia Due to High Refractive Errors
•Hyperopia > 5d
•Myopia > 10d
•Astigmatism > 2d
Deprivation Amblyopia
•Amblyopia ex anopsia
•Medial opacities-congenital cataract; Peter’s anomaly
•Congenital ptosis
•Occlusion amblyopia
•Unilateral worse than bilateral

Amblyopia Treatment
•Establish a clear retinal image
•Correct ocular dominance
Establishing a Clear Retinal Image
•Cataract removal
•Ptosis surgery
•Corneal transplant
•Full hyperopic correction for high hyperopes
•Full astigmatic correction for meridional amblyopes
Correct Ocular Dominance
•Patching
•Penalization
Patching
•Cover the sound eye to stimulate neurodevelopment of amblyopic eye

•Full-time Occlusion
-most effective
 -for all but one waking hour
Patching
•Part-time – not as effective
- when not tolerated; not practical
- for maintenance occlusion
Age Limits for Occlusion

•8 years? 9 years? …none.

•Try full-time occlusion for three months max.

•Progress is slower in the older patient.
Patching: Technique
•Adhesive orthoptic patches- opticlude
-wet well before removing
•Eye pad with black cloth and micropore tape
•Black cloth sleeve over glasses
Occlusion Amblyopia
•Reversal amblyopia

•Prevention - see the patient in one week per year of life of patching, max. of 4 weeks
•Be very careful in patching infants
< 6 months: < or = 50% of waking hours
6 – 12 months: 80% of waking hours
Penalization
•Blurring the sound eye to force fixation to the amblyopic eye

•Optical- overplussing lens such that sound eye is used for near

Atropine Penalization
•If emmetropic, only blurs at near
•If hyperopic, blurs distance and near

•Not recommended for dense amblyopia unless highly hyperopic

The Ophthalmologist treats amblyopia but the primary care physician detects amblyopia.
Vision Screening
Purpose: to identify children with one or more of the following conditions:

•Decreased vision in one or both eyes

•Eye conditions which could lead to decreased vision
•Strabismus (crossed or wall eye)
•Other eye related conditions such as: ptosis, nystagmus, head tilt, etc.

The best age for vision screening:

As near as possible to the 3rd birthday

Recognition Acuity at 3 years
Observe for:

•eye crossing
•"wall" eye
•nystagmus
•droopy lid
•abnormal head positions

Recognition Acuity at 3 years

•Thank you!

STRABISMUS

Angle Kappa
The angle bet visual and central pupillary line

A nasally centered pupillary light reflex gives the appearance of exotropia and is physiologic.

A temporally displaced pupillary light reflex gives the appearance of esotropia and is uncommon,
occurring in some cases of very high myopia.
 Extraocular Muscles

Spiral of Tillaux
•The line of insertion where the rectus muscles insert in sclera gradually farther from the limbus beginning
with the medial rectus at 5.5mm (range 3.0 to 6.0mm), inferior rectus 6.5mm, lateral rectus 6.9mm and
superior rectus 7.7mm.

•It is also the line of insertion of posterior Tenon's capsule which then proceeds to the limbus as the
episclera fused with the underlying sclera.

Recti
The rectus muscles are each 40mm long.
They receive innervation on the global surface at the junction of the middle and posterior 1/3 of the
muscle.
The pulleys are located on the orbital surface at the junction of the middle and posterior 1/3 of the globe.
The pulleys are fibromuscular structures that act as functional origins of recti

Coronal view - normal location of pulleys

Inferior Oblique
Superior Oblique
The S.O. has its functional origin in the trochlea. This unique structure allows an 8mm increase in the
trochlear insertion distance in upgaze and an 8mm decrease in distance between the S.O. insertion
and trochlea in downgaze.
Action
Action
Blood Supply
A long posterior ciliary artery travels from the back of the eye in sclera beneath the horizontal recti.
The oblique muscles do not contribute to the blood supply of the anterior segment of the eye.

lateral rectus
•innervated by the sixth cranial nerve.
•The nucleus is located in the brain stem and the nerve is uncrossed.
superior oblique
•innervated by the fourth cranial nerve which crosses to reach the muscle.
•The nerve passes through the "stiff" tentorium making it susceptible to the shearing force produced by
brain oscillation in closed head trauma.
third cranial nerve
Innervates
•the medial rectus
•superior rectus
•inferior rectus
•inferior oblique
•levator palpebri.

•The sympathetic nerve supply travels along the innervation to the inferior oblique.
•The parasympathetic supply travels in the nasociliary nerve.

Principles and definitions

•Primary position of gaze- looking straight ahead
•Field of action- direction where muscle exerts greatest force
•Agonist- muscle that pulls eye into direction of gaze
•Antagonist- opposing muscle which relaxes

•Synergistic muscles – muscles that have same field of action

ex. RSR and LIO are synergists for upgaze

Sherrington’s law
•Reciprocal innervation of antagonistic muscles
•- synergists are stimulated and antagonists inhibited
•Ex. Right gaze- RLR and LMR stimulated
• RMR and LLR inhibited
Hering’s Law
•Equal innervation given to yoke muscles
•Yoke- a pair of agonists with same primary action
•Ex . RLR and LMR are the yoke pair for right gaze
•Importance: secondary deviation in paretic muscles

•Ductions-monocular rotations
•Versions- binocular rotations

•Primary deviation- normal eye fixing

•Secondary deviation – paretic eye fixing

•Prism diopter-unit of angular measurement

• 1 degree of arc = 1 pd -
Evaluation
Cardinal Gazes

Hirschberg Test
Krimsky
Phoria vs Tropia
•Heterotropia- manifest strabismus
- present under binocular viewing conditions

•Heterophoria- latent strabismus

- only after binocular vision is interrupted
Cover Test
•Cover-uncover Test – shows a manifest deviation e.g. esotropia

•Alternate Cover Test – shows latent deviations too e.g. exophoria

•Orthophoria- no shift on cover testing

Cover Testing
Pseudo-strabismus
esotropia

Accomodative Esotropia

Bifocals for High AC/A Accomodative ET

Exotropia
Hypertropia
Incomitant Strabismus
Deviation varies in different fields of gaze
•Duane’s
•Brown’s
•III N Palsy
•VI N Palsy
•IV N Palsy
Duane’s Type 1
The Etiology of Duane Syndrome
•IIIrd N regenerates to lateral r. causing co-contraction of LR on attempted adduction
Brown’s Syndrome
IVth Nerve Palsy
VIth Nerve Palsy

Non-surgical: Fresnel Prisms

Strabismus surgery
Adjustable sutures