Cardiff Acuity Cards:

for infants until 1 yr

Selected Topics in Pediatric ´ Visual acuity testing in children depend on : ´ fixation and following behavior starts to develop at
Ophthalmology and • Age of the child about the age of 6 weeks and fully established at the
age of 3 months.
• Examiner skills
Approach to Blind Infant • Preference /Resources
´ We depend on the estimation of visual acuity and
symmetry between the two eyes . between the two eyes .
Raed Shatnawi, MD, FRCS

Topics to be covered 0-4 weeks Kays pictures (matching): 1-3 yrs

´ Visual examination in different age groups
´ Presentation of children with subnormal vision
´ Diseases causing blindness with nystagmus
´ Diseases causing blindness without nystagmus
´ Amblyopia
´ Strabismus
´ Newborn to 1 month → blink to bright light.
´ 6 weeks to 3 months → fix & follow.
´ 1y to 3y → Cardiff acuity card (CAC).
´ 3 – 5 yrs → Sheridan-Gardiner.
´ > 5 yrs → Snellen optotypes
2) response to hand motion threat
3)gross objection and crying to contralateral occlusion of
the eye indicating poor vision , that means if you
occluded the good eye the baby starts crying or tries to
move your hand because the unoccluded eye has a
poor vision.

Introduction 0-4 weeks Sheridan-Gardiner Test

3-5 yrs
´ Children are born with very poor visual acuity but they 1) blinking to bright light (BTL) ´ After the age of 5 we use
are not blind , they can see! ´ bilateral blinking right/left when exposed to light (sun for Snellen optotypes.
´ Visual acuity is usually severely reduced about 6/300 example)
because the fovea is covered by a thick layer of ´ observed visual behavior
ganglion cells , the eye is still immature.
´ It's an estimation of the visual acuity plus comparison of
´ With age, peripheral migration of the ganglion cells both eyes
leaves the fovea thin naked structure with sharp
excellent vision around the fifth year of age. ´ Up till the first month of life we depend on observed
visual behavior.
´ So if a 3 year old child had a vision of 6/9 ,it is considered
normal for his age.
Children who present with blind
behavior
´ Normal newborns blink when exposed to sunlight , smile
when seeing their mother’s face or follow the mother
while moving
´ All or some of these normal visual behaviors are lost
´ If Hx & Ex indicates poor visual behavior (Blindness) ,
we classify this into two broad categories:
1) blind behavior with Nystagmus.
2) blind behavior without Nystagmus.
Blind infant with Nystagmus:
´ Characteristic of the disease causing it:
1) Bilateral
2) Significant
3) Anterior visual pathway problem (from the cornea to
the chiasm)
4) Appeared & not corrected during the first 3 months of
life.
* First 3 months of life = critical period of visual
development.
Nystagmus Congenital Cataract
´ Corneal opacity. ´ Vitreous hemorrhage
´ Corneal dystrophy. ´ Foveal hypoplasia
´ Nystagmus appears only after the first 3 months of life. ► It is an involuntary , oscillatory eye movement. ´ Corneal cloudiness. ´ Albinism ´ Bilateral congenital cataract causes nystagmus
´ The appearance of nystagmus indicates severity and ► Two types may appear : (severe irreversible blindness if not treated within the first
´ Congenital glaucoma. ´ Retinal detachment
irreversibility of the vision loss. 1) Jerk nystagmus : a slow drift of the eyes in one direction 3 months)
followed by a rapid recovery movement in the other ´ Congenital cataract. ´ Optic nerve hypoplasia
´ Unilateral cataract does not cause nystagmus. Also it
direction. ´ Aniridia ´ Optic nerve coloboma causes blind eye but not blind infant.
2) pendular nystagmus : slow eye movement that is equal ´ Optic atrophy
in velocity.
► As for the direction of movement it might be horizontal ,
vertical , or multiplanar.
Blindness and nystagmus Nystagmus Congenital glaucoma
´ Two types: ´ In congenital nystagmus the patients feel that the ´ Triad of symptoms for congenital glaucoma :
A) Diseases affecting the anatomical integrity of the eye outside world is stable while in acquired nystagmus the photophobia – hazy cornea- hyperlacrimation.
(Abnormal eye examination). world is unstable (shaking)-oscillopsia. ´ Has bad prognosis
B) Diseases affecting the function of the eye (Normal eye
´ Medical treatment for glaucoma has no role in children
examination. But the child is blind).
except in preparing the child for surgery to decrease
´ So, blind infant + Nystagmus + Normal eye exam è must IOP or further reduction of IOP after surgery
receive electrophysiological testing
Electrophysiological Tests in infants Congenital Glaucoma Causes of congenital cataract
1) ERG ´ Differential diagnosis of congenital glaucoma: ´ Roughly one third is hereditary with autosomal dominant
Electroretinogram: measures the function of the
Diseases affecting the anatomical 1.Foreign bodies being the most common
photoreceptors (cones & rods: each photoreceptor cell
has its specific wave).
integrity of the eye: 2-Congenital nasolacrimal duct obstruction ´ One third is associated with a syndrome or metabolic
disease or TORCH.
2) VEP
´ One third is unknown (idiopathic).
Visual Evoked Potential: measures the function of the ´ Treatment :
optic nerve. ´ Infants with suspected cataract or a family history of
Always surgical ( goniotomy, trabeculotomy ). congenital cataracts should be assessed by an
´ These two investigations are done to prove & reach the
ophthalmologist shortly after birth, as soon as possible.
specific diagnosis of the vision loss.

Treatment of Congenital Cataract
´ Lensectomy + posterior capsulotomy + anterior
vitrectomy.
´ IOL is contraindicated before the age of 2 yrs due to
small size of infants eyes compared to the IOL.
´ However, there is increasing reports of successful IOL
implantation before the age of 2 yrs
´ Prognosis is guarded depending on the age of onset,
timing of surgery and postoperative optical and visual
rehabilitation.
Aniridia
´ Absence of iris tissue
´ Associated with foveal hypoplasia (causes blindness and
nystagmus)
´ AD and sporadic
´ Sporadic: caused by deletion of Ch. 11p which may be
part of WAGR association.
´ Wilms tumor (nephroblastoma), Aniridia, Genitourinary
defects, Retardation: requires regular renal U/S until the
pt is 16 yrs old to detect Wilms tumor.
Vitreous hemorrhage: from shaken
baby syndrome (child abuse) and
other trauma
´ shaking baby syndrome (risk factors) :-
1)first child in the family
2)single parent
3)daycare
4)Child with chronic Medical illnesses or syndromes.
´ Mechanism: acceleration-deceleration movement
causes shearing forces on the retinal delicate blood
vessels which rupture and bleed in the retina and
vitreous
Foveal Hypoplasia
´ Causes: Aniridia and Albinism
´ Two types of Albinism :
A) Oculocutaneous : autosomal recessive, with fair hair ,
skin , and eyes. And is due to tyrosinase enzyme
deficiency.
B) Ocular : involving only the eye ,X linked recessive.
´ Associated foveal hypoplasia results in significant
reduction in visual acuity and nystagmus.
´ Most patients are also photophobic because of the
missing filter function of the pigmented layer of the iris
Pathophysiology of ROP Treatment of ROP
´ In preterm infants, retinal blood vessels are immature. ´ In early cases: argon laser to ischemic retina.
They don’t reach the retinal periphery. ´ In complicated cases (retinal detachment): complex
´ The peripheral relatively ischemic retina will produce vitreoretinal surgeries
vascular endothelial growth factor (VEGF) which will ´ Prognosis for stage 4 and 5 is poor leaving the baby
stimulate new blood vessel formation.
blind.
´ In 90% of cases, this process is successful. In 10% of cases,
this will develop neovascularizations which lack
important structures in their wall and so they leak, bleed
and rupture easily causing the formation of fibrovascular
membranes and traction on the retina and eventually
retinal detachment.
Retinopathy of Prematurity) ROP :
Stages of ROP: Optic Nerve diseases
Five stages
´ Risk Factors :-
´ Stage 1: Demarcation line ´ Optic nerve coloboma: incomplete growth of the optic
A) Gestational Age <32 weeks (if more the risk of ROP is nerve. If bilateral and severe will result in blindness and
ZERO !) ´ Stage 2: Ridge
nystagmus
´ Stage 3: Extraretinal neovascularization
B) Birth weight <1.5 kg ´ Optic Nerve Hypoplasia (ONH): if bilateral and severe
´ Stage 4: Partial retinal detachment
´ Babies with the above 2 risk factors are included in the will result in blindness and nystagmus.
screening program ´ Stage 5: Total retinal detachment
´ De Morsier Syndrome (septo-optic dysplasia): Bilateral
´ ROP occurs in 10% of all babies at risk. ´ Treatment is indicated in stages 3, 4, and 5. ONH with atrophy of the anterior pituitary gland and
absent corpus callosum and septum pellucidum.
´ 10% of all ROP babies will progress to a stage requiring
treatment. 90% of ROP will regress spontaneously.
Screening for ROP:
´ Other risk factors of ROP : ´ Done by experienced pediatric ophthalmologist for all
1. Hyper-oxygenation at the NICU babies GA< 32 weeks. Disease affecting the function of
2. Intraventricular hemorrhage ´ First screening exam is done 4 weeks after birth or at GA the eye (Retinal photoreceptors)
of 32 weeks whichever LATER.
3. Neonatal sepsis
´ When to stop screening:
4. Necrotizing enterocolitis
1. Full maturation of the retinal vasculature: complete
peripheral retinal vascularization
2. Baby reached corrected GA 44 weeks

´ Blind infant with nystagmus and normal eye examination
→ do electrophysiological investigations: ERG & VEP
Leber’s congenital amaurosis (LCA)
´ (LCA) is an AR inherited retinal degenerative disease
characterized by very poor vision at birth.
´ Total damage to rods and cones.
´ ERG: Extinguished ERG. Flat line (no waves for rods or
cones).
´ ERG pathognomonic.
Other retinal diseases
´ Con’s dystrophy.
´ Congenital stationary Night blindness (CSNB).
´ Retinitis pigmentosa.
´ Achromatopsia
´ ERG in the above conditions is characteristic.
Blind infant without Nystagmus
´ The problem is posterior to the chiasm (in the brain).
´ Role of the pediatrician and pediatric neurologist in
establishing the diagnosis.
´ History of:
convulsion disorder, brain hypoxia, birth asphyxia, cerebral
palsy and or developmental delay.
v If no nystagmus is present, the diagnosis falls into 2
categories based on whether CNS abnormalities are
present or not.
1. CVI (Central visual impairment)
2. DVM (Delayed Visual Maturation)
v Very high refractive errors (myopia or hyperopia) may
lead to behavior that mimics blindness.
´ In both groups of poor visual behavior always exclude
high refractive errors by:
´ always do dilated fundus Ex..
´ always do cycloplegic refraction.
CVI:
´ History of : birth asphyxia, obstructed labor, seizures
´ Helpful signs include
ü Preference of bright colored objects,
ü Staring at bright light, and
ü Turning of the head whenever they attempt to look to
an object of interest.
´ CT scan and MRI as well as pediatric neurologist are
necessary for proper diagnosis.
DVM
´A diagnosis of exclusion and retrospection.
´This refers to: Visually impaired infants with normal
ocular examinations and normal neuroimaging. Normal
ERG. No nystagmus.
´ The exact etiology is unknown
´ The visual prognosis is excellent but this retrospective
diagnosis should be made only when visual function
indeed improves to normal or near-normal levels by the
end of the first year of life.
WHO definitions of vision impairments Amblyopia: Treatment
´ Blindness: Visual acuity worse than 3/60 in the best eye ´ Patching the good eye: usually 2-6 hours according to
with best glasses correction (counting fingers at 3 m) or severity.
visual field loss less than 10º. ´ Atropine eye drops in the good eye: this will cause
´ Low vision: Visual acuity better than 3/60 but worse than temporary loss of accommodation and transient blurring
6/12 in the better eye with best correction. of vision in the good eye.
´ Amblyopia: Difference in best corrected visual acuity of ´ When to stop treatment of amblyopia:
2 Snellen lines or more between the two eyes in the 1. Equal vision in both eyes
absence of organic lesion.
2. No improvement after 6 months of treatment: persistent
Amblyopia: Classification
´ Sensory deprivation amblyopia :
- A disease causes entry of light and pictures into the
eye in the early vision development. STRABISMUS
- examples are congenital cataracts or corneal opacity
- can be treated until the age of 5 years.
Amblyopia: Classification
´ Strabismus amblyopia
- the brain suppresses the deviated eye which with time
becomes amblyopic.
- Adults don’t suppress è diplopia.
- can be treated until age of 8 years.
´ Refractive errors(anisometropic) amblyopia:
- different refractive errors between the 2 eyes and
suppression of the worse image and eye.
- can be treated until age of 11 years.
 Pseudostrabismus Orthotropia Alternate Cover Test

´ Strabismus is a malalignment of the eyes.
´ Two types of strabismus:
1. Comitant strabismus : where equal angle of deviation in
different positions of gaze .
´ The term applies to a false appearance of squint in the ´ It measures the total angle of deviation including phoria
absence of any deviation and it may occur under and tropia.
different condition ´ Combined with prisms to exactly measure the angle of
´ Visual axes are well aligned toward fixation object deviation.

2. Incomitant strabismus :where unequal angle of deviation ´ Any abnormality of the lids, canthii (epicanthal folds),
in different positions of gaze ,and it is two types restrictive wide nasal bridge or orbit may lead to pseudostrabismus
Cover – Uncover test
(e.g.: thyroid ophthalmopathy and myasthenia gravis) and Esophoria, abnormal, common
paralytic (e.g: 6th and 3rd nerve palsy).
Only seen when eye is covered
Often asymptomatic, no
complaints

G.Vicente,MD

Terminology 71

Ortho: straight eyes ´Pseudostrabismus

Eso = inward deviation
Exo= outward deviation
Hyper= upward deviation
Hypo=downward deviation
Cover – Uncover test
Phoria = hidden latent tendency for deviation when one
Exophoria, abnormal, common
eye is occluded
Only seen when eye is covered
Tropia = manifest obvious deviation
Often asymptomatic, no
Fakhruddin Aliasger 7/10/2013
complaints.

G.Vicente,MD

Hirschberg light Test

´ Used to test tropia but not phoria.

´ It is performed by shining a light in the person's eyes and
observing the light reflection from the corneas.
´ Nasal corneal light reflex: exotropia Alternate Cover test
Exotropia, intermittent
´ Temporal corneal light reflex: esotropia
May be visible with or without
´ Inferior corneal light reflex: hypertropia Cover – Uncover test alternate cover
Orthophoria, normal
´ Superior corneal light reflex: hypotropia May have intermittent
No complaints, diplopia, especially when
asymptomatic tired or sick
Mom sees misalignment
every now and then.
G.Vicente,MD G.Vicente,MD
G.Vicente,MD
 Guidelines to management of
strabismus

´ Treat the underlying disease

´ Correct refractive errors if any
´ Treat amblyopia if possible
´ Surgically treat the residual angle of strabismus:
resection or recession to strengthen or weaken the
Alternate Cover test muscle, respectively.
Exotropia, Constant
May be visible with or without
alternate cover
May or may not have
constant diplopia
G.Vicente,MD

How much to operate…

20

Alternate Cover test with

Prism
Exotropia, Constant
Use prism to quantitate the
deviation.
Change prism power until
movement is neutralized.
Use this number to plan
G.Vicente,MD
surgery

Approach to a patient with

strabismus : Thank you . . .
Dr. Raed A Shatnawi, MD, FRCS (Glasg)
´ History Assistant Professor of Ophthalmology
´ Previous surgeries, glasses or patching Hashemite University College of Medicine

´ Clinical examination Senior Consultant of Pediatric Ophthalmology

Fellow of the Royal College of Physicians & Surgeon of Glasgow (UK)
1)Visual acuity
Member of the American Association of Pediatric Ophthalmology & Strabismus
2)Hirschberg light test
Fellow of the International Council of Ophthalmology
3)Alternate cover test Children’s Memorial Hospital / Northwestern University (Chicago, IL, USA)
4)Cycloplegic refraction test Nationwide Children’s Hospital, Ohio State University, Columbus, OH, USA
5)Dilated fundus examination Great Ormond Street Hospital for Sick Children, University College of London,
London, UK.