1. Article
Pediatric Cerebral Visual Impairment
Notes from the American Conference on Pediatric Cerebral Visual Impairment
Dominick M. Maino, OD, MEd, FAAO, FCOVD-A
Professor of Pediatrics/Binocular Vision, Illinois College of Optometry
Keywords: blindness, children’s hospital, of Occupational Therapy, Creighton University,
cortical visual impairment, Pediatric cerebral visual Omaha, NE and Dominick M. Maino, OD, MEd,
impairment FAAO, FCOVD-A; Professor of Pediatrics/Binocular
Vision Illinois Eye Institute/Illinois College of
The Children’s Hospital and Medical Center of Optometry, Chicago, Il. The program was developed
Omaha, NE recently sponsored a symposium with and moderated by neuro-ophthalmologist, Richard
the purpose being to bring together professionals H. Legge, M.D.; Adjunct Assistant Professor,
from several fields of study. These professional were Department of Ophthalmology, University of
to share information, learn from each other, discuss Nebraska Medical Center, Omaha, NE. The audience
controversial topics, and develop a document suitable included optometrists, ophthalmologists, other MDs,
for publication detailing principles that we could all occupational therapists, physical therapists, speech
agree upon. A second document may also be developed and language therapists and a large number of teachers
that discusses the controversies in this area and the of the visually impaired.
foundations for these controversies. These documents
or transactions will serve as reference guides to all Conference Topics
involved professionals, with derivative publications 1. Defining Pediatric Cerebral Visual Impairment
for the lay public to follow. The definition of brain related visual impairment
The professional team invited to keynote this had been and even today is often confusing,
conference included: Mark Borchert, M.D.; Associate misunderstood and imprecise. It is now, however,
Professor of Clinical Ophthalmology and Neurolog, frequently referred to as Pediatric Cerebral Visual
University of Southern California, The Vision Center, Impairment (PCVI). Initially Pediatric Cerebral
Children’s Hospital Los Angele, Los Angeles, CA; Visual Impairment had also been referred to as
Christine Roman Lantzy, Ph.D.; Director, Pediatric Pediatric Cortical Visual Impairment and mistaken
View Program, The Western Pennsylvania Hospital. for Delayed Visual Development.1
CVI Consultant, The American Printing House for Commentary in the Journal of Visual Impairment
the Blind Educational Consultant, Pittsburgh, PA; and Blindness2 noted that in North America the phrase
Jacy VerMaas-Lee, M.A., OTR/L; Assistant Professor Cortical Visual Impairment was frequently used while
elsewhere Cerebral Visual Impairment was considered
Correspondence regarding this article should be emailed to dmaino@ico.edu the preferred terminology.
or sent to Dominick M. Maino, OD, MEd, Illinois College of Optometry,
3241 S. Michigan Ave., Chicago, IL 60616. All statements are the
The story of the development of the concepts of
author’s personal opinion and may not reflect the opinions of the College visual impairment due to brain injury begins in the
of Optometrists in Vision Development, Optometry & Vision Development 19th century. Later during World War I, wounded
or any institution or organization to which the author may be affiliated. veterans with brain injury displayed an ability to
Permission to use reprints of this article must be obtained from the editor.
Copyright 2012 College of Optometrists in Vision Development. OVD is
perceive motion in the “blind, non-seeing” visual
indexed in the Directory of Open Access Journals. Online access is available field. This ability to sense motion, lights, and colors
at http://www.covd.org. even though the individual has brain injury induced
Maino D. Pediatric Cerebral Visual Impairment. Optom Vis Dev
blindness may be conscious or subconscious. This
2012:43(3):115-120 is also referred to as statokinetic dissociation or the
Riddoch phenomenon when discussing adults.3 The
Volume 43/Number 3/2012 115

2. ability to sense such motion was called blindsight4 learning abnormalities. Improvement in the infant’s
which also appeared to include the ability to ‘sense’ vision takes longer and the end point visual acuity is
objects in one’s way so that these could be avoided typically not of the same quality as in DVM I. Many in
when walking into a room or down a hall way. this category have intellectual disability, seizures, and
Prior to the 1980’s adults with bilateral insult to other developmental issues. In DVM III, the children
the occipital cortex were referred to as having cortical frequently have congenital nystagmus and albinism.
blindness. At this time, this term was also applied to Their vision starts to improve later than infants with
children. Cortical visual impairment was used in the DVM type I and can improve to low-normal levels.
late 1980’s onward with the definition of CVI being When Delayed Visual Maturation is associated with
injury between the lateral geniculate nucleus and the retinal, optic nerve and macular anomalies, it is
visual cortex with reduced visual acuity being the referred to as being Type IV.8
identifying feature. When it was noted that many
children had damage to the white matter surrounding Defining PCVI. Variability with defining various
the ventricals of the brain (periventricular leukomalacia disorders is not all that uncommon. For instance
PVL), the term cerebral visual impairment was coined Autism used to be a relatively rare anomaly. Once this
and was used to describe the condition (especially in definition was altered to reflect a spectrum of individuals
Europe). with behaviors that have autistic like characteristics, the
Cerebral visual impairment is a more inclusive number of those on the Spectrum is now considered
term that allows for not only significantly reduced (by some) to have reached almost epidemic status.9,10,11
visual acuity but also the frequently associated Interestingly the neurological/brain changes associated
oculomotor anomalies, visual field loss, and vision with this disorder can even mimic many of the behaviors
information processing problems seen in children.3 seen in those with PCVI as well.12
Some researchers suggest that the phrase cognitive Should we be concerned about how PVCI is
visual dysfunction (CVD) be used to identify the defined? Absolutely. There are instances where not
many visual perceptual anomalies associated with this only do the numbers of individuals with the diagnosis
condition.5 increase exponentially (like that which occurred
for Autism), but can also decrease significantly.
Colanbrander classified the various areas associated When the American Association on Intellectual and
with CVI, these included: Developmental Disabilities changed the definition
1.
Ocular visual impairment: Anomalies of of mental retardation by decreasing the IQ cut
refractive state and optics and eye health. off point from 80 to 70 and by adding adaptive
2.
Cerebral visual impairment: Abnormalities behavior qualifications they instantly cured hundreds
associated with pathway problems, cortical of thousands of those with mental retardation
problems, and oculomotor dysfunction as well overnight.­ The AAIDD has not only changed the
13
as vision information processing anomalies definition of mental retardation, but also the words
(dorsal and ventral streaming processing used to describe the condition. Many years ago the
mechanisms).6 classifications used such derogatory terms as idiot and
moron; then mental retardation and now, the preferred
Delayed Visual Maturation. (DVM) describes terminology in this area is either developmental
infants who appear to be visually impaired, but usually disability or intellectual disability.
demonstrate improved visual abilities by the age of 6 What we call a thing is very important for to
months, often without treatment. At this point the name it is to have power over it.
children frequently then go on to mirror more normal Post conference commentary: After the meeting
infant visual development.7 Even though infants with the group of presenters met to review all that was
DVM were first described in the 1920s, there is little discussed the previous day. It was decided that the
consensus as to the etiology of this disorder. There term “pediatric cortical visual impairment” was the
are several types of DVM with type I being described preferred terminology to use since this is very specific
earlier in this paragraph. DVM type II is characterized about the group of individuals being discussed (those
by problems with attention and fixation but is also with cortical visual impairment only and no other
usually associated with other neurological and/or developmental, cognitive or developmental issues). I
116 Optometry Vision Development

3. noted that most practitioners, therapists, and teachers Directionality, visual motor integration, non-motor
see children with additional issues such as motor perceptual skills, and auditory perceptual/processing
challenges, vision information dysfunction, and skills all have a role to play in child development.
other non-cortical anomalies. For these individuals Unfortunately those with disability tend to have both
I suggested that the term, pediatric “cerebral visual functional vision and vision function anomalies that
impairment” is much more appropriate. interfere with the development of appropriate vision
and auditory information processing ability.28
2. etermining Visual Function in Children with
D Post conference commentary: Some members of
Pediatric Cortical Visual Impairment the group thought that the use of electro-diagnostics
There are numerous areas that require a significant (VEP, ERG, EOG) were not needed and only
number of assessment procedures to ascertain the confirmed what you already knew. These tests added
level of ability of those with pediatric cerebral/cortical to the costs involved in managing patients and used
visual impairment. We need to assess vision function valuable resources unwisely. I noted that the use of
as well as functional vision. VEPs could establish a valuable objective baseline
An assessment of vision function can include of vision function that could be utilized to confirm
determination of the clarity of vision (visual acuity, improvement once therapy was begun and could help
contrast sensitivity, refractive error), oculomotor guide the therapist in what approaches worked best.
ability (pursuits and saccades; convergence and
divergence), accommodation (focusing), depth 3. herapeutic Strategies For the Treatment of
T
percep­tion (3D vision) and eye health.14,15,16 It is Pediatric Cerebral Visual Impairment
also often appropriate to use special diagnostic All treatment should begin by paying attention
tools such as the EOG (electrooculogram), ERG to the basics. These basics include the various areas of
(electroretinogram) and the VER (visually evoked vision function and eye health discussed above. Any
response; VEP, visual evoked potential) to determine problems that need to be addressed to insure the best
the level of ability present. Those with a wide range of possible eye health should be instituted. If uncorrected
disability tend to show many anomalies in the various refractive error is present, it should be diagnosed and
areas of vision function noted above.17,18,19,20 a prescription for glasses given to the child. It has
An assessment of functional vision should then been noted that even correcting a relatively small
be conducted as well. Those with disability tend to amount of refractive error for those with traumatic
have functionally induced disability that often overlays brain injury can improve these individuals’ quality
pathologically induced disability, so that the end result of life.29 Remember that spectacles can not only be
is often greater than one might expect from either corrective/compensative in nature but they can also
anomaly individually. For instance a large amount be therapeutic as well.
of uncorrected refractive error (hyperopia, myopia, Children with high amounts of hyperopia and
astigmatism) could cause amblyopia (a functional those with accommodative dysfunction (including
anomaly) that magnifies any vision loss due to individuals with Down Syndrome, Cerebral Palsy
cerebral/cortical impairment. The amblyopia also and brain injury) often benefit from a multi-focal
induces numerous vision information processing prescription where an added “+” power is given either in
anomalies that impede a child’s daily living skills a multifocal prescription (bifocal) or as a secondary pair
development and his or her ability to navigate the of spectacles to use for specific tasks. Individuals with
world about them. Children with Down Syndrome significantly decreased vision at near can also benefit
for example have very poor accommodative abilities from high “+” adds and the magnification that results.
that can interfere with all near-point activities from Once the refractive prescription is determined and
using a computer to reading a book.21,22,23 Those with corrected, and any therapeutic applications addressed
Cerebral Palsy will display oculomotor, visual motor appropriately for use with a spectacle prescription
integration and accommodative problems along with (bifocals, prism, sector occlusion, etc), then it is time
high refractive errors as well.24,25,26 to determine other therapeutic interventions required
Another area of concern is that of vision for any additional vision function anomalies present. It
information processing (VIP) and the development was suggested that Facebook can be a unique resource
of appropriate visual perceptual skills.27 Laterality/ for therapeutic ideas as well as other internet resources
Volume 43/Number 3/2012 117

4. (Thinking Outside the Box,36 Maino’s Memos,37 Table 1: Medication Side Effects adapted from RJ
Pinterest38). Therapeutic pro­ ures to improve
ced­ Donati RJ, Maino DM, Bartell H, Kieffer M. Polypharmacy and
the Lack of Oculo-Visual Complaints from those with Mental
eye movement and hand-eye, accommodation, Illness and Dual Diagnosis.Optometry 2009;80:249-254.
convergence/divergence, and other aspects of both Systemic Oculovisual
vision function and functional vision, as well as visual Side Effects Side Effects
Antipsychotics Bone marrow Blurred vision
stimulation activities were presented as well. depression
Although children with cortical/cerebral visual Muscle spasms/ Light sensitivity
impairment have significant neurological impedi­ twitches
Breast enlargement Visual Disturbances
ments, the principles of neuroplasticity can be applied (M F)
to the various therapeutic approaches utilized for this High body Mydriasis
temperature
population.30 In most instances the therapy is not Antidepressants Abdominal pain/ Blurred vision
rehabilitative in nature, but rather habilitative. This constipation
difference is important to remember when caring for Abnormal dreams/ Increased risk of
thinking Glaucoma
those with PCVI. Abnormal Visual Disturbances
Post conference commentary: The large number of ejaculation/orgasm
teachers in the audience often directed the discussion Anxiety Photophobia
towards diagnosing and treating the educational Anticonvulsants Memory problems/ Blurred vision
amnesia
concerns present for these children. Christine Roman Sedation Dimming of vision
Lantzy, Ph.D, frequently referred to her text, Cortical Insomnia Diplopia
Visual Impairment: An Approach to Assessment and Bronchitis Involuntary eye
Intervention, as the best way to determine various movements
Fluid retention Dry eye
levels of function and to treat PCVI.31 Anti-Parkinson’s Abnormal dreams/ Vision abnormalities
insomnia
4. ow Do Environmental Factors, Medications
H Increased muscle Blurred vision
tone/weakness
and Non-Visual Handicaps Affect the Involuntary Mydriasis
Evaluation and Treatment of Pediatric movements
Hallucinations Decreased
Cerebral Visual Impairment? accommodation
Individuals with a handicap tend to be prescribed Tranquilizers Breast development Risk of narrow angle
many more medications than those not demonstrating in men GLC
Breathing problems Cycloplegia/Mydriasis
a disability. They also often have a slightly higher affinity
for the development of adverse effects due to various Insomnia Decreased vision
environmental factors. A paper in Optometry discussed Tardive dyskinesia Capsular cataract
adults with not only a developmental disability but also Antianxiety Anemia Decreased
accommodation
a psychiatric illness that noted many of these individuals Seizures Nystagmus
were taking 10 or more, high powered neurotropic Blood disorders Diplopia
and systemic medications. Interestingly, seldom did Unusual excitement Mydriasis
any of these individuals complain of symptoms related
to their disability, systemic anomalies, or medication mental illnesses in children. Pediatric depression is also
side effects.32 Certainly those who are significantly being diagnosed often, let alone all the medications
younger than the population described above may also currently being used for behavioral issues such as
find it difficult to communicate their needs, wants and attention deficit hyperactivity disorder.34
symptoms as well. One of the major environmental hazards those
Various medications, alternative and comple­ with disability encounter are people. Many do not
mentary medical therapies33 and even more traditional know how to respond to an individual with a disability.
allopathic approaches to health care can result in They may make assumptions that are false and then
adverse, unintended events. (See Table 1). Although act on those assumptions. This is true not only for
you may think that a child is too young for many lay individuals but also for teachers and health care
of these major drugs, you should realize that various professionals.35
psychiatric anomalies such as pediatric bipolar Post conference commentary: Little was discussed
disorder is now one of the most frequently diagnosed on this topic after the meeting. It was decided,
118 Optometry Vision Development

5. however, that the American Conference on Pediatric 16. Woodhouse JM. Abnormalities of refraction and accommodation and their
management. In Dutton GN, Bax M (Eds) Visual Impairment in Children
Cortical Visual Impairment would be held on a due to Damage to the Brain. Clinics in Developmental Medicine. Mac
Kieth Press 2010: 98-105.
yearly basis over the next two years with support
17. Woodhouse M, Maino D. Down Syndrome. In Taub M, Bartuccio M,
from the Children’s Hospital and Medical Center Maino DM (Eds). Visual Diagnosis and Care of the Patient with Special
of Omaha, NE. A website, Brainblind.org (http:// Needs. Lippincott, Williams Wilkins. Available May 2012 from
http://goo.gl/zB7Dl.
www.brainblind.org/) is being developed and should
18. Berry-Kravis E, Maino D. Fragile X Syndrome. Lippincott, Williams
be online sometime during the summer of 2012 for Wilkins. Available May 2012 from http://goo.gl/zB7Dl.
additional information. 19. Maino D. Overview of special populations. In Scheiman M, Rouse M. (eds)
Optometric management of learning related vision problems. St. Louis,
For those in attendance, the first ever American MO: Mosby Inc. 2006;85-106.
Conference on Pediatric Cerebral/Cortical Visual 20. Maino D. The young child with developmental disabilities: An introduction
Impairment began a process to determine the to mental retardation and genetic syndromes. In Moore BD (ed) Eye
care for infants and young children. Butterworth--Heinemann, Newton,
optimum way to diagnose and treat those with PCVI. MA.1997:285-300.
Many left with answers. More left with questions. The 21. Wesson M, Maino D. Oculo-visual findings in Down syndrome, cerebral
planning process continues for the next conference. palsy, and mental retardation with non-specific etiology. In Maino D (ed).
Diagnosis and Management of Special Populations. Mosby-Yearbook, Inc.
What would you, as developmental optometrists, St. Louis, MO. 1995:17-54.
want out of such a conference? Contact Dr. Dominick 22. Cregg M, Woodhouse JM, Pakeman VH, et al. Accommodation and
refractive error in children with Down syndrome: cross sectional and
Maino for additional information (dmaino@ico.edu). longitudinal studies. Invest Ophthal Vis Sci. 2001;42:55-63.
23. John FM, Bromham NR, Woodhouse JM, Candy TR. Spatial vision deficits
References in infants and children with Down syndrome. Invest Ophthal Vis Sci.
2004;45:1566-1572.
Note: URLs are functional hyperlinks to internet addresses. 24. Taub M, Reddell AS. Cerebral Palsy. In Taub M, Bartuccio M, Maino
1. Kran BS. Vision Impairment and Brain Damage. In Visual Diagnosis and D. (Eds) Visual Diagnosis and Care of the Patient with Special Needs;
Care of the Patient with Special Needs. Taub M, Bartuccio M, Maino D, Lippincott Williams Wilkins. New York, NY;2012:31-40.
Eds. Lippincott Williams Wilkins, NY, NT 2012;135-145. 25. Wesson M, Maino D. Oculo-visual findings in Down syndrome, cerebral
2. Colenbrander A. What’s in a name? Appropriate terminology for CVI. J palsy, and mental retardation with non-specific etiology. In Maino D (ed).
Vis Impair Blindness 2010;583-585. Available from http://goo.gl/xl4sb last Diagnosis and Management of Special Populations. Mosby-Yearbook, Inc.
accessed 3/12 St. Louis, MO. 1995:17-54.
3. Lueck AH. Cortical or Cerebral Visual Impairment in children: A Brief 26. Hyvarinen L. Classification of visual functioning and disability in
Over View. J Vis Impair Blindness 2010;585-592. children with visual processing disorders. In Dutton GN, Bax M (Eds)
Visual Impairment in Children due to Damage to the Brain. Clinics in
4. Alesterlund L, Maino D. That the blind may see: A review: Blindsight and Developmental Medicine 2010. Mac Kieth Press: 265-281.
its implications for optometrists. J Optom Vis Dev 1999;30(2):86-93
27. Scheiman M, Rouse M. (Eds) Optometric management of learning related
5. Good WV, Jan JE, Burden SK, Skoczenski A, Candy R. Recent advances in vision problems. St. Louis, MO: Mosby Inc. 2006.
cortical visual impairment. Dev Med Child Neurol 2001;43:56-60.
28. Dutton GN, Macdonald E, Drummond SR, Saidkasimova S, Mitchell K.
6. Colenbrander A. The functional classification of brain damage related vision Clinical features of perceptual and cognitive visual impairment in children
loss. J Vis Impair Blind 2009;103:118-123. with brain damage of early onset. 2010 In Dutton GN, Bax M (Eds)
Visual Impairment in Children due to Damage to the Brain. Clinics in
7. Cool SJ. Normal and adnormal visual development. In Maino D (ed).
Developmental Medicine. Mac Kieth Press 2010:106-116.
Diagnosis and Management of Special Populations. Mosby-Yearbook Inc St.
Louis, MO. 1995. Reprinted Optometric Education Program Foundation, 29. Leslie S. Myopia and accommodative insufficiency associated with moderate
Santa Anna, CA. 2001:1-15. head trauma. Optom Vis Dev 2009;40(1):25-31.
8. Delayed Visual Maturation: A Visual-Inattention Problem . Available from 30. Maino D, Donati, R, Pang, Viola S, Barry S. Neuroplasticity. In Taub M,
http://goo.gl/jLn29 last access 3/12 Bartuccio M, Maino D. (Eds) Visual Diagnosis and Care of the Patient with
Special Needs; Lippincott Williams Wilkins. New York, NY;2012:275-288.
9. Autism: The Hidden Epidemic? Available from http://goo.gl/t5z0J last
accessed 3/12 31. Lantzy CR. Cortical Visual Impairment: An Approach to Assessment and
Intervention. Available from American Foundation for the Blind at http://
10. Maino DM. Viola, SG, Donati R. The Etiology of Autism. Opt Vis Dev
goo.gl/53MKS
2009:(40)3:150-156. Available from http://www.covd.org/Portals/0/
Article_Etiology%20of%20Autism.pdf last accessed 3/12 32. RJ Donati RJ, Maino DM, Bartell H, Kieffer M. Polypharmacy and the
Lack of Oculo-Visual Complaints from those with Mental Illness and Dual
11. Maino D. Partly cloudy with a chance of meatballs. Optom Vis Dev
Diagnosis.Optometry 2009;80:249-254.
2009;40(3):134-135 available from http://goo.gl/kuMPb last accessed 3/12
33. Maino D. Evidence based medicine and CAM: a review. Optom Vis Dev
12. Viola SG, Maino DM. Brain anatomy, electrophysiology and visual
2012;43(1):13-17
function/perception in children within the autism spectrum disorder. Opt
Vis Dev 2009;40(3):157-163. Vailable from http://goo.gl/oqQ26 last 34. Schnell PH, Maino D, Jespersen R. Psychiatric Illness and Associated
accessed 3/12 Oculo-visual Anomalies. In Taub M, Bartuccio M, Maino D. (Eds) Visual
Diagnosis and Care of the Patient with Special Needs; Lippincott Williams
13. FAQ on Mental Disability. American Association on Intellectual and
Wilkins. New York, NY;2012:111-124.
Developmental Disability. Available from http://goo.gl/nvcFA
35. The Ten Commandments of Communicating with People With Disabilities
14. Schlange D, Maino D. Clinical behavioral objectives: assessment techniques
- DVD Resource Guide. available from http://goo.gl/8tmQc last accessed
for special populations. In Maino D (ed) Diagnosis and Management of
3/12
Special Populations Mosby-Yearbook, Inc., St. Louis, MO. 1995:151-88.
36. Thinking Outside the Light Box http://goo.gl/6O1EM, accessed 5/12
15. Taub M. Examination Procedures. In Taub M, Bartuccio M, Maino
DM (Eds). Visual Diagnosis and Care of the Patient with Special Needs. 37. http://www.MainosMemos.blogspot.com
Available from http://goo.gl/zB7Dl.
38. Pinterest http://goo.gl/DRBgz
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