Diagnosis and Therapeutic Intervention of Vision Function and Functional Vision Anomalies in Pediatric Cortical Visual Impairment

Diagnosis and Therapeutic
Intervention of Vision Function and
Functional Vision Anomalies in PCV

Dominick M. Maino, OD, MEd, FAAO, FCOVD-A
Professor of Pediatrics/Binocular Vision
Illinois Eye Institute/Illinois College of Optometry
Lyons Family Eye Care
Chicago, Il
dmaino@ico.edu
ICO.edu
LyonsFamilyEyeCare.com
MainosMemos.com

Vision Function and Functional Vision Anomalies in PCV
The American Conference on Pediatric Cortical Visual Impairment
brings together professionals in optometry, ophthalmology,
occupational therapy and visual educational psychology to increase
the understanding of the definition, diagnosis and management of
cortical vision loss in children.
(Dr Dominick Maino, PCVI Society Founding Board member, Dr. Joseph Maino, Dr. Kerri Pillen)

1. History and Definition of pediatric cortical visual
impairment (PCVI).
2. Describe the diagnostic criteria utilized in optometry.
3. Discuss the treatment techniques.

Pediatric Cerebral Visual Impairment
 History of CVI
 Brain injury 19th century
with Phineas P. Gage

 World War I, wounded
veterans with brain injury
 Displayed perceived motion
in the “blind, non-seeing”
visual field.
 Ability to sense motion,
lights, and colors
 Conscious or subconscious.

 Statokinetic dissociation (in children)
 greater reduction in sensitivity to stationary visual stimuli relative to
similar targets in motion
 Riddoch phenomenon (adults)
 Ability to sense movement even though blind
 “See” moving objects…but not stationary ones
 Blindsight
 Ability to ‘sense’ objects in the way

 Movement in the peripheral visual field may elicit a
smile in the blind child with quadraplegia and profound
intellectual disability.
 Children who are fed with a spoon may intermittently
open their mouths to receive food when the spoon is
moved in an arc from the peripheral visual fields, but
not when it approaches the mouth from straight
ahead.

 For those children who understand language stating what
is being seen as the child reacts to it may enhance both
visual and language development.
 Such children may rock to and fro. Whether this generates
an image is difficult to know.
 Rarely, children with cerebral blindness who are mobile
move slowly around obstacles. This phenomenon has been
called travel vision (Blindsight).
 Alesterlund L, Maino D. That the blind may see: A review: Blindsight and its implications for
optometrists. J Optom Vis Dev 1999;30(2):86-93

 1980’s adults with bilateral occipital cortex insult
(cortical blindness)
 Term applied to children.
 Cortical visual impairment used in the 1980’s
onward
 Definition of CVI includes injury lateral
geniculate nucleus/visual cortex

 Reduced visual acuity identifying feature.
Many children damage to white matter
surrounding the ventricals (perventricular
leukomalacia PVL)
 Cerebral Visual Impairment now used
(especially in Europe)

Cerebral vs Cortical Visual Impairment
 Cerebral visual impairment: inclusive term
 Reduced visual acuity
 Oculomotor anomalies
 Visual field loss
 Vision information processing problems
 Cognitive Visual Dysfunction (CVD)
 Used to identify visual perceptual anomalies
 Used to identify vision information processing
problems

 Classification of CVI
 Ocular visual impairment: Refractive state. Optics,
Eye health
 Cerebral visual impairment: Neuro-pathway
problems, cortical problems, oculomotor
dysfunction, vision information processing (dorsal
and ventral streaming processing mechanisms)

The ventral stream (also known as the "what
pathway") travels to the temporal lobe and is
involved with object identification. The dorsal stream
(or, "where pathway") terminates in the parietal lobe
and process spatial locations.

Pediatric Cortical Visual Impairment
Reduced visual acuity due to a
“brain problem”

 Delayed Visual Maturation (DVM)
 DVM type I Visually impaired infants: improved visual
abilities by the age of 6 months, often without
treatment.
 DVM type II: attention problems, associated with
neurological/learning abnormalities. Improvement
takes longer
 DVM III: children have nystagmus, albinism. Vision
improves later, can improve to low-normal levels.
 DVM IV: associated with retinal,
optic nerve, macular anomalies

 Defining Other Disorders and PCVI
 Variability with defining disorders not uncommon
 Autism rare anomaly
 Definition altered so that the number of those on
the Spectrum is now considered epidemic
 Legal, legislative, health care, insurance issues

 Should we be concerned about how PVCI is defined?
Absolutely!
 American Association on Intellectual and
Developmental Disabilities changed definition of
mental retardation
 Decreasing IQ cut off point from to 80 to 70
 Added adaptive behavior qualifications
 Result: instantly cured hundreds of thousands of those
with mental retardation/intellectual disability
overnight

What we call a thing is very
important
To name it is to have power
over it

Determining Vision Function and
Functional Vision in Children with

Diagnostic Approaches & Strategies
1.Case History
2.Visual Acuity
3.Refractive Error
4.Vision Function Assessment
5.Ocular Health
6.Special Tools

Vision Function
Clarity of vision (visual acuity, contrast
sensitivity, refractive error)
Oculomotor ability (pursuits and
saccades; convergence and divergence)
Accommodation (focusing)
Depth perception (3D vision)

Vision Function
Eye health
Biomicroscopy
Tonometry
Dilated Fundus Evaluation
Special diagnostic tools
EOG (electrooculogram)
ERG (electroretinogram)
VER/VEP (visually evoked response
visual evoked potential)

Functional Vision
Functionally induced disability that overlays
pathologically induced disability
Uncorrected refractive error : Amblyopia
Constant Strabismus: Amblyopia
Oculomotor dysfunction, Binocular vision
dysfunction, Accommodative dysfunction:
Attention

Functional vision
Vision information processing (VIP)/
Visual perceptual skills
laterality/directionality
visual motor integration
non-motor perceptual skills
auditory perceptual/processing

Vision Function
Clarity of vision
What is visual acuity?
What is contrast sensitivity?
What is refractive error?

Vision Function
Clarity of vision
What is visual acuity?
The ability to see a certain size
object at a certain distance.

Vision Function
Clarity of vision
What is contrast sensitivity?

Contrast sensitivity measures the ability to
see details at low contrast levels. Visual
information at low contrast levels is
particularly important:
1. in communication, since the faint
shadows on our faces carry the visual
information related to facial
expressions.

2. in orientation and mobility, where
we need to see such critical low-contrast
forms as the curb, faint shadows, and
stairs when walking down. In traffic, the
demanding situations are at low
contrast levels, for example, seeing in
dusk, rain, fog, snow fall, and at night.

3. in every day tasks, where there
are numerous visual tasks at low
contrast, like cutting an onion on a
light colored surface, pouring coffee
into a dark mug, checking the
quality of ironing, etc.

4. in near vision tasks like reading
and writing, if the information is at
low contrast as in poor quality copies
or in a fancy, barely readable
invitation, etc.
from http://www.lea-test.
fi/en/vistests/pediatric/cstests/cstests.html

Tests of
Visual
Acuity and
Contrast
Used with
permission

Pursuits,
Saccades,
Convergence

Retinoscopy
Book (Getman)
1. At the Free and Easy reading level, the reflex varied from neutral to with motion and
was bright, had sharp edges and had a pinkish color.
2. At the instructional reading level (which was defined as maintaining the reading task
with comprehension in spite of being stressed) the reflex was a varying fast against motion
while the color was bright, sharp, and very pink.
3. At the frustration reading level (which was defined as reading with minimal
comprehension) the reflex showed a slow against motion with a dull brick red color.
Bell (Apell)
Dynamic (#5 & #6 OEP)
MEM (Haynes)

Retinoscopy
Bell (Apell)
Target(Wolf Wand) directly in front of the retinoscope
Patient fixates the target
Move the ball toward the patient slowly and smoothly
The distance of the target from the patient is recorded for a change in motion or
other changes of interest
Expect to see a change from “with” to “against” on the way in at 35 - 42
cm. (14 - 17inches) and a change from “against” to with at 37.5 - 45 cm. (15 -18
inches).
MEM (Haynes)

Retinoscopy
Distance retinoscopy (#4) in place
Patient behind the phoropter
Examiner’s retinoscope at a 20 (twenty) inch distance from the patient.
Patient fixates small letters or a picture
Patient looks, reads, names, and interact visually with the target
Plus spheres added until against motion is seen
Then plus spheres are reduced until the first neutral (no motion) response
Lens in the phoropter is recorded as the “gross” finding.
The #5 retinoscopy, like the other “21 point findings”, had no specific meaning by
itself until it was compared to other findings as part of the total analytical
examination. The #5, like the 14B gross, will approximate the most plus lens
acceptable for near.

Retinoscopy
MEM Retinoscopy (Monocular Estimation Method)
Patient reads grade appropriate material usually affixed to the retinoscope
Working distance is the patient’s Harmon distance
Quickly “dip” lenses in front of the reading material
A +.25 to +.75 lag of accommodation is considered normal
A high lag would be +1.00 or greater
Accommodative excess would be any AM noted
Also note variability of reflex, color and other variability
Stress Point Retinoscopy
Kraskin & Harmon
For more info see: http://www.oepf.org/VTAids/Retinoscopy.pdf

Color vision, copy forms,
Matching, Visual Fields

Therapeutic Strategies for the
Treatment of
Pediatric Cerebral Visual
Impairment

 Treatment begins with the basics.
 Vision function
 Refractive correction
 Spectacles therapeutic
 Eye health

 Treatment with spectacle/lenses
 multi-focal prescription/bifocal
 prism
 occlusion

 Treatment with spectacle/lenses/computer devices
 task specific glasses
 high “+” adds (magnification)
 Telescopes
 Microscopes

 Vision Therapy
 Oculomotor/hand-eye/
accommodation
& fusion
 Biocular
 Binocular
 Integration

 Vision Therapy
 Integration/Stabilization
 Visual stimulation
 Vision information
processing
 Vestibular/Vision
Apps 4 Vision Development
http://www.sovoto.com/group/apps4VisionDevelopment

Perceptual Learning vs Vision Therapy

Thinking Outside the LightBox

Think Outcomes!

What follows is supplemental information
to the presentation, resources and
references that are not necessarily a part of
this presentation, but which I thought you
would like to have for your own
information.

How Do Environmental Factors,
Medications and Non-Visual Handicaps
Affect the Evaluation and Treatment of
Pediatric Cerebral Visual Impairment?

For individuals with disability…
 Medications: Prescribed many more medications
 Higher affinity for adverse effects due to
environmental/systemic factors
 Seldom complain of symptoms related to their disability,
systemic anomalies, or medication side effects

 Alternative and complementary
medical therapies
Maino D. Evidence based medicine and CAM: a review. Optom Vis Dev 2012;43(1):13-17
Lemer P. Complementary and Alternative Approaches. In Taub M, Bartuccio M, Maino D.
Visual Diagnosis and Care of Patients with Special Needs. Lippincott, Williams, Wilkins.
2012
 Traditional allopathic approaches

 Mental illnesses in children
 Pediatric Bipolar disorder
 Pediatric depression

 Major environmental hazard: People
 do not know how to respond
 make assumptions
 true for lay individuals, teacher, health care
professionals

Medication Side Effects
Antidepressants
Abdominal pain/constipation Blurred vision
Abnormal dreams/thinking Increased risk of
Disturbances
Anxiety Photophobia

Anticonvulsants
Memory problems/amnesia Blurred vision
Sedation Dimming of vision
Insomnia Diplopia
Bronchitis Involuntary eye movements
Fluid retention Dry eye

Anti-Parkisons
Abnormal dreams/insomnia Vision abnormalities
Increased muscle tone/weakness Blurred vision
Involuntary movements Mydriasis
Hallucinations Decreased
accommodation

Tranquilizers
Breast development in men Risk of narrow angle GLC
Breathing problems Cycloplegia/Mydriasis
Insomnia Decreased vision
Tardive dyskinesia Capsular cataract

Anti-anxiety
Anemia Decreased accommodation
Seizures Nystagmus
Blood disorders Diplopia
Unusual excitement Mydriasis

PCVI: References
 Dutton GN, Bax M. (eds). Visual impairment in children due to damage to
the brain. Clinics in Developmental Medicine. no 186. MacKieth Press.
London;2010.
 Strategies for dealing with visual problems due to cerebral visual
impairment: Gillian McDaid, Debbie Cockburn, Gordon N Dutton available
from http://www.ssc.education.ed.ac.uk/courses/vi&multi/vjan08i.html
 Alesterlund L, Maino D. That the blind may see: A review: Blindsight and
its implications for optometrists. J Optom Vis Dev 1999;30(2):86-93
 Kran B. Mayer L. Vision impairment and brain damage. In Taub M,
Bartuccio M, Maino D. (Eds) Visual Diagnosis and Care of the Patient with
Special Needs. Lippincott Williams & Wilkins , NY, New York; 2012:135-
146.

PCVI: References
 Colenbrander A. What’s in a name? Appropriate terminology
for CVI. J Vis Impair Blind. 2010:583-585
 Roman Lantzy CA, Lantzy A. Outcomes and opportunities: A
study of children with cortical visual impairment. J Vis Impair
Blind. 2010:649-653.
 http://www.aph.org/cvi/define.html
 Cerebral Visual Impairment in Periventricular Leukomalacia:
MR Correlation: Available from
http://www.ajnr.org/content/17/5/979.full.pdf

References
 Luek AH. Cortical or cerebral visual impairment in children: A brief
overview. J Vis Impair Blind. 2010:585-592.
 Woodhouse JM, Maino DM. Down syndrome: In Taub M, Bartuccio
M, Maino D. (Eds) Visual Diagnosis and Care of the Patient with
Special Needs. Lippincott Williams & Wilkins , NY, New York; 2012:31-
40.
 Wesson M, Maino D. Oculo-visual findings in Down syndrome,
cerebral palsy, and mental retardation with non-specific etiology. In
Maino D (ed). Diagnosis and Management of Special Populations.
Mosby-Yearbook, Inc. St. Louis, MO. 1995:17-54.
 Taub M, Reddell A. Cerebral Palsy. In Taub M, Bartuccio M, Maino D.
(Eds) Visual Diagnosis and Care of the Patient with Special Needs.
Lippincott Williams & Wilkins , NY, New York; 2012:21-30.

References
 Ciuffreda K, Kapoor N. Acquired brain injury. In
Taub M, Bartuccio M, Maino D. (Eds) Visual
Diagnosis and Care of the Patient with Special
Needs. Lippincott Williams & Wilkins , NY, New
York; 2012:95-100.
 Roman-Lantzy, C. Cortical visual impairment: An
approach to assessment and intervention. AFB
Press, NY, New York; 2007.
 http://www.MainosMemos.com

 Resources
 Facebook (http://www.facebook.com/Thinkingoutsidethelightbox)
 Pinterest http://pinterest.com/achampine0302/cortical-visual-impairment-
cvi-goodies/
 Blogs http://www.MainosMemos.blogspot.com
 Apps http://www.sovoto.com/group/apps4VisionDevelopment
 Infant Visual Stimulations https://itunes.apple.com/us/app/infant-visual-
stimulation/id427443223
 Infant Visual Stimulation Video
https://www.youtube.com/watch?v=Eyj5PqwUn0w
 COVD http://www.COVD.org
 OEPF http://www.OEPF.org
 Vision Help blog http://visionhelp.wordpress.com/
 MainosMemos http://www.MainosMemos.blogspot.com

Dominick M. Maino, OD, MEd, FAAO, FCOVD-A
Professor of Pediatrics/Binocular Vision Illinois Eye Institute
Illinois College of Optometry
3241 S. Michigan Ave. Chicago, Il 60616
Lyons Family Eye Care
3250 N. Lincoln Ave. Chicago, Il 60657
dmaino@ico.edu
ICO.edu
LyonsFamilyEyeCare.com
MainosMemos.com

Diagnosis and Therapeutic Intervention of Vision Function and Functional Vision Anomalies in Pediatric Cortical Visual Impairment

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Diagnosis and Therapeutic Intervention of Vision Function and Functional Vision Anomalies in Pediatric Cortical Visual Impairment