1. M
Evaluation of non seeing
infant
BY
DR DIWA LAMICHHANE
BIRATNAGAR EYE HOSPITAL, BIRATNAGAR 19 – 05 - 2013

2. Outline
 Epidemiology
 Visual development in infant
 Causes of non seeing infant
 Evaluation of non seeing infant

3. The prevalence of blindness increases from less than 0.01 per
100 in preschool- age children to 8.58 per 100 persons over the
age of 60 .
Bulletin of the World Health Organization, 63 (2): 375-386 (1985)

4.  Nystagmus is absent in cortical blindness and is not found often in
association with unilateral visual defects.

5. Visual development
 Complex maturation process
 Structural changes occur both in the eye & CNS
 Normal vision develops as a result of both genetic coding &
experience in a normal visual environment
Assessment
 Fixation target
 Blink reflex to bright light 30weeks after birth
 Pupillary light reflex 29- 31 wks of gestation

6. Visual development
6 weeks
 Make and maintain eye contact
 React with facial expression
2-3mths
 Interested in bright object
Untill 4mths
 Disconjugate eye movements
Skew deviation, sunsetting present as a transient deviation in new born

7. Visual development
Sign of actual poor vision development
 Wandering eye movement
 Lack of response to familiar faces & objects
 Nystagmus
 Staring at bright lights
 Forceful rubbing of the eyes( oculodigital reflex )

8. Causes
The causes of SVI and blindness may be:
 Prenatal
 Perinatal
 Postnatal

9. Causes..
 Congenital anomalies
 Anophthalmos, microphthalmos, coloboma, congenital cataract,
infantile glaucoma, and neuro-ophthalmic lesions
 Acquired during the perinatal period
 Ophthalmia neonatorum, ROP, & cortical visual impairment.

10. Most common causes
 Anterior segment anomalies
 Glaucoma
 Cataract
 Optic nerve hypoplasia
 Optic atrophy
 Leber congenital amaurosis
 Achromatopsia
 Congenital infection syndrome
/ TORCH
 Cortical visual impairment
 Delay in visual maturation
 ROP
 X-linked retinoschisis
 Congenital motor nystagmus
 Albinism
 Coloboma

11. Evaluation of normal infant

12. General history of the infant
Parents or caretakers are asked routinely
 Young child’s visual behavior with family members and at playtime
 Whether the child responds to a silent smile
 Enjoys silent mobiles
 Follows objects around the environment.
Pertinent observations include strabismus, nystagmus, persistent
staring, and inattention to objects.

13. Family history of ocular disease
 Such as blindness
 Poor vision
 Need for thick glasses
 Difficulty ambulating in dim
 Illumination
 Photophobia
 Color vision deficiencies
 ‘Lazy eye’ or amblyopia
 Strabismus
 Nystagmus
 Leukocoria
 History of eye surgery.

14. Family history of systemic disorders
 That may be associated with ocular abnormalities
 Connective tissue
 Cardiovascular defects associated with
 Marfan’s syndrome
Midfacial hypoplasia
 Arthropathy associated with stickler’s syndrome
 Dental and umbilical abnormalities in rieger’s syndrome
 Urinary tract abnormalities in lowe’s syndrome
 Neurologic and skin abnormalities in the phakomatoses

15. Family history of systemic disorders
 Unusual physical traits, developmental delay, mental retardation, &
early death
 Denial, illegitimacy, incest, paternal substitutions, & natural variability
in expression of inherited disorders make the process even more
difficult.
 A standard pedigree diagram of nuclear family

16.  Male – X – linked disorder
 If sibling has similar condition not present in previous generations –
AR disease

17. Prenatal and perinatal history
 About the pregnancy, delivery, birth weight, gestational age, neonatal
health.
 Maternal Illness, Infection, Radiation, Trauma
 Teratogenic potential of medications as ethyl alcohol, particularly in
infants or children with dysmorphic features.
 The relatively free passage of substances from mother’s
circulation into breast milk provides infant with yet another
route of ingestion.
 The importance of prematurity & its relationship to retinopathy of
prematurity is well recognized.

18.  Observing various components of the examination while taking
history.
 Child’s alertness, interaction with parents, head position, fixation &
following ability, steadiness of gaze, gross alignment of eyes can
usually be observed .
 Attention or crying it is sometimes best to interrupt history & begin
more entertaining aspects of examination.
 Additional historical information can be obtained either during
examination or after examination steps are completed.
History..

19. General examination
 Child’s general physical appearance, alertness, overall size, weight,
body structure & interaction with adults accompanying child.
 Systemic disorders may be associated with ocular abnormalities, one
should undress the child & examine various areas such as head,
neck, integument, thorax, abdomen, genitalia, skeletal structure
using inspection, palpation, & auscultation.
 The child’s head size & shape eg. microcephaly & macrocephaly
 Unusual skull contours, such as plagiocephaly is related to certain
types of strabismus.

20. Ocular examinations
 Position and size of the orbits
 Position of the globes
which can usually be compared by looking down from over the child’s
forehead.
 Eye lids
Palpebral fissures compared with respect to contour, size, location
position & movement of the upper & lower eyelids, presence of
epicanthal folds.
 Intercanthal & interpupillary distances is measured & compared with
standard nomograms such as those listed in
Smith’s recognizable patterns of human malformation

21.  Lacrimal system
Size of tear meniscus, presence of epiphora, patency of lacrimal
puncta, appearance of areas overlying lacrimal drainage system
& lacrimal gland.
 Orbital structures
Should be palpated & auscultated, with emphasis on areas of
special concern.
Ocular examinations

22. Visual acquity
 Infants & children up to the age of 2.5 to 3 years are generally
unable to accomplish recognition visual acuity tests.
 It is evaluated by using objective techniques, such testing in clinical
office setting is performed by observing eye movements that are
produced in response to visual stimulation.
 The ability to ‘fixate and follow’ a target is the principal clinical acuity
test used to assess central visual function in infants & young
children.
Ocular examinations

23.  Normal motor responses to visual stimulation in child consist of fast,
voluntary, refixation saccades & slow, smooth pursuit movements
that occur as eyes follow a moving target.
 Human face is generally strongest visual stimulus for young infant,
even newborn infants intermittently attempt to fixate on a human face
placed in proximity.
 The innate visual interest in the human face may well be related to
infant-mother bonding. Visually induced motor response may be
enhanced by holding the infant in a comfortable but upright position
and placing the examiner’s face close to the infant while examiner
slowly moves from side to side.
If there is no reaction, test should be repeated with mother
holding child using her own face as test
Ocular examinations

24.  Young infants are not capable of producing normal slow, smooth
pursuit movements seen in older children and adults. Instead, young
infants display jerky, hypometric saccades in the same direction as
target moves.
The examiner must also be careful not to rotate the infant during
acuity testing, because rotation induces vestibuloocular
reflex (VOR), which is a powerful non visual stimulus for eye
movement.
 The status of visual motor system must be appropriately evaluated
before concluding that the lack of eye movement
Ocular examinations

25.  However, in neonates, tonic phase predominates, whereas fast
component may be observed intermittently. By 4 weeks of age,
both slow & fast phases of vestibular nystagmus are easily elicited in
almost all normal infants.
 The young infant who appears to be blind but in whom a VOR fast
phase also fails to develop may simply be unable to generate normal
hypometric, saccadic following movements in response to visual
stimulation. This may occur in children with CNS damage, such as
cerebral palsy, & congenital oculomotor apraxia.
 Truly blind child also fails to visually suppress VOR in usual 3–5 s after
cessation of Rotation.Instead, blind children may have nystagmus that
persists for 15–30.

26.  By 2 or 3 months of age, most normal infants quite consistently fixate
on & follow a nearby human face, as well as small toys such as
finger puppets.
 Motor responses to visual stimulation should be evaluated at both
near and distance
 By 6 months of age, most infants fixate on moving toys or cartoons at
the end of a 20-ft examining room.
 In the office setting, pediatric ophthalmologists commonly use a
vertical array of two or three electrically operated mechanical toys
located at the end of the examining room.
 the left as the left eye,

27.  Each toy is individually illuminated & controlled by a foot pedal. The
normal child fixates on toy that is illuminated & moving, & small
vertical refixation saccades are easily observed as the child’s
attention is directed from one toy to another when the foot pedal is
pressed. The noise generated by the toys is not sufficiently
directional to elicit refixation saccades, because the toys are spaced
about 1 ft apart.

28. Assessment visual acuity using fixation responses is generally
recorded using the CSM method.
C – central fixation, implying that fixation is foveal.
S – steady fixation, the eye being tested is steadily fixating on a
stationary or slowly moving target.
Wandering eye movements or nystagmus indicates that fixation is not
steady.
M - eye maintains fixation after cover is removed from opposite eye.
Determining whether

29.  The CSM method may not adequately describe the reduced acuity in
a child with a severe visual disability. In this situation, it may be
appropriate to employ a narrative description of the level of visual
responses, such as
Visual acuity both eyes = No ‘fixation’ or ‘following’ efforts
with human face, toys, or hand light at 1 ft distance.
Mild photoaversion to bright halogen indirect ophthalmoscope light.

30. Evaluation of non seeing infants

31. Evaluation..
1. History..
 Perinatal problems including prematurity, IUGR, Fetal distress,
bradycardia, meconium staning, & oxygen deprivation.
 Systemic abnormalities
 Delayed mild stones

32. Evaluation..
2. Examination
 Fixation target
 OKN
 VEP
 Preferential looking
 Blink reflex to bright light several days after birth
 Pupillary light reflex 29- 31 wks of gestation

33. Evaluation..
FIXATION TARGETS
 Such as a human face
 Stripes, dots, or checkerboards are preferred
 Infants younger than 3months of age follow by means of hypometric
saccades when the target is small
 Term infants may generate smooth pursuit movements to a large
target such as an opticokinetic drum.
 Because saccadic palsies are common in young children who have
central nervous system damage, spinning an upright child
demonstrates the presence of saccades as the rapid recovery phase
of the spin-induced nystagmus

34. Estimated visual acuity at different ages
from Dabson and Teller and Hoyat et al
Age OKN Preferential
looking
VEP
1 M 6/120 6/120 6/120
2M 6/60 6/60 6/60
6M 6/30 6/30 6/6-6/12
Age at which6/6
is achieved
20-30M 24-36M 6-12M

35. Evaluation..
OPTICOKINETIC NYSTAGMUS
 Visual angle subtended by the smallest
strip width that elicits an eye movement.
 Measures acuity by means of a motor
response technique (eye movement ).
 Subcortical areas of the occipital cortex
may generate opticokinetic responses.
 Binocular acuity 20/400(6/120) at birth and
reach 20/20(6/6) by 26-30 months.

36. Evaluation..
VISUALLY EVOKED RESPONSE
Used to evaluate acuity in
 Aphakic
 Amblyopic
 Strabismic
 large refractive errors
 Although the test directly evaluates vision by means of a sensory
process, a waveform of normal appearance has been recorded in the
occasional decorticate infant who later behaves as if blind, which
implies a subcortical contribution

37. Evaluation
Forced choice preferential looking
 Based on observation that infants prefer to view a pattern stimulus
rather than a homogeneous field.
 Using flat, calibrated, square-wave gratings
 Observed by a trained individual.
 Term newborn differentially responds to 20/400 (6/120) gratings; the
response to 20/20 (6/6) gratings occurs at 18–24months

38. Evaluation
 Infant exposed to pair of stimuli consisting
of field of black and white stripes and
indentically sized gray field of equal
luminance.
 Location of stripes shifted randomly from
right to left
 Fineness of stripes reduced till infant can
no longer differentiate stripe and
background.

39. VA in infant..
Forced choice prefential looking device Teller acuity cards

40. Evaluation..
2. Examination
 Visual fixation
 Crispness and equality of pupillary light responses
 Ocular alignment & motility
 Nystagmus or roving eye movements
 Detailed fundus examination
 Infant with normal but immature visual system may be unresponsive
to even very bright light that is indistinguishable from blindness
 Fixation & follow response can be elicited with moving red light
horizontally /vertically in front of infant in otherwise unresponsive
baby

41. Examination with hand held slit lamp

42. Evaluation..
2. Examination..
Pupillary response
 Sluggish in ant visual pathway disease such as ON hypoplasia or
atrophy, ON coloboma, morning glory disc anomaly
 Paradoxical pupillary phenomenon ( constriction to darkness) –
diiffuse retinal disease eg ON hypoplasia, cone dystrophy
Pupillary responses are normal in infant with cortical visual impairment

43. Evaluation..
2. Examination..
Nystagmus
 Begins at 2-3 mths, not at birth
 Nystagmus implies presence of at least some visual function.
Roving eye movements
 Total or near total blindness
Congenital motor nystagmus
 No organic eye abnormality, mild to moderate reduction in visual
acuity.

44. Evaluation..
2. Examination..
Abnormal binocular alignment
 < 1 yr - Exotropia
 > 1 yr - Esotropia

45. Evaluation..
3. Investigation
Electrophysiological tests
 ERG
Poor vision with normal appearing ocular structure:
Leber congenital amaurosis
Achromatopsia
Blue-cone monochromatism
X-linked or AR CSNB
 VEP

46. Evaluation..
3. Investigation..
Imagings
 CT
 MRI
 Specialized laboratory studies based on consultation with:
 Paediatric neurologist
 Endocrinologist
 Neurosurgeon
 Geneticist

48. Take home message..
 Childhood blindness has an adverse effect on growth, development,
social, and economic opportunities.
 Severe visual impairment (SVI) and blindness in infants must be
detected as early as possible to initiate immediate treatment to
prevent deep amblyopia.

49. References…
 AAO Section 6 Paediatric Ophthalmoloy and strabismus.
 Yanoff and Duker Ophthalmology 3rd edition.
 Jakobiec Principles and practice of ophthalmology vol 4, 3rd edition.

50. THANK YOU

51.  FIXATION TARGETS
Targets: mother’s face, flashlight
 CSM method
 Vertical prism or induced tropia test

52. • Catford drum test
• child is made to observe an oscillating drum with black dots of
varying sizes
• Smallest dot that evokes pendular eye movements denotes
visual acuity

53. Indirect assessment of VA
I. Blink response
II. Menace reflex
III. Test based on fixation reflex
 Fixation behaviour test
 Binocular fixation pattern
 Central, steady and maintained (CSM)