ArticlePediatric Cerebral Visual ImpairmentNotes from the American Conference on Pediatric Cerebral Visual ImpairmentDomin...
ability to sense such motion was called blindsight4         learning abnormalities. Improvement in the infant’swhich also ...
noted that most practitioners, therapists, and teachers    Directionality, visual motor integration, non-motorsee children...
(Thinking Outside the Box,36 Maino’s Memos,37                   Table 1: Medication Side Effects adapted from RJPinterest3...
however, that the American Conference on Pediatric                                 16.	 Woodhouse JM. Abnormalities of ref...
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  1. 1. ArticlePediatric Cerebral Visual ImpairmentNotes from the American Conference on Pediatric Cerebral Visual ImpairmentDominick M. Maino, OD, MEd, FAAO, FCOVD-AProfessor 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 developedOmaha, NE recently sponsored a symposium with and moderated by neuro-ophthalmologist, Richardthe 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 ofto share information, learn from each other, discuss Nebraska Medical Center, Omaha, NE. The audiencecontroversial topics, and develop a document suitable included optometrists, ophthalmologists, other MDs,for publication detailing principles that we could all occupational therapists, physical therapists, speechagree upon. A second document may also be developed and language therapists and a large number of teachersthat discusses the controversies in this area and the of the visually impaired.foundations for these controversies. These documentsor transactions will serve as reference guides to all Conference Topicsinvolved professionals, with derivative publications 1. Defining Pediatric Cerebral Visual Impairmentfor 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 VisualUniversity of Southern California, The Vision Center, Impairment (PCVI). Initially Pediatric CerebralChildren’s Hospital Los Angele, Los Angeles, CA; Visual Impairment had also been referred to asChristine Roman Lantzy, Ph.D.; Director, Pediatric Pediatric Cortical Visual Impairment and mistakenView Program, The Western Pennsylvania Hospital. for Delayed Visual Development.1CVI Consultant, The American Printing House for Commentary in the Journal of Visual Impairmentthe Blind Educational Consultant, Pittsburgh, PA; and Blindness2 noted that in North America the phraseJacy VerMaas-Lee, M.A., OTR/L; Assistant Professor Cortical Visual Impairment was frequently used while elsewhere Cerebral Visual Impairment was consideredCorrespondence 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 ofauthor’s personal opinion and may not reflect the opinions of the College visual impairment due to brain injury begins in theof Optometrists in Vision Development, Optometry & Vision Development 19th century. Later during World War I, woundedor any institution or organization to which the author may be affiliated. veterans with brain injury displayed an ability toPermission 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” visualindexed in the Directory of Open Access Journals. Online access is available field. This ability to sense motion, lights, and colorsat http://www.covd.org. even though the individual has brain injury inducedMaino D. Pediatric Cerebral Visual Impairment. Optom Vis Dev blindness may be conscious or subconscious. This2012:43(3):115-120 is also referred to as statokinetic dissociation or the Riddoch phenomenon when discussing adults.3 TheVolume 43/Number 3/2012 115
  2. 2. ability to sense such motion was called blindsight4 learning abnormalities. Improvement in the infant’swhich also appeared to include the ability to ‘sense’ vision takes longer and the end point visual acuity isobjects in one’s way so that these could be avoided typically not of the same quality as in DVM I. Many inwhen 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 childrenthe 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 withchildren. 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 withinjury between the lateral geniculate nucleus and the retinal, optic nerve and macular anomalies, it isvisual cortex with reduced visual acuity being the referred to as being Type IV.8identifying feature. When it was noted that manychildren had damage to the white matter surrounding Defining PCVI. Variability with defining variousthe ventricals of the brain (periventricular leukomalacia disorders is not all that uncommon. For instancePVL), the term cerebral visual impairment was coined Autism used to be a relatively rare anomaly. Once thisand was used to describe the condition (especially in definition was altered to reflect a spectrum of individualsEurope). with behaviors that have autistic like characteristics, the Cerebral visual impairment is a more inclusive number of those on the Spectrum is now consideredterm that allows for not only significantly reduced (by some) to have reached almost epidemic status.9,10,11visual acuity but also the frequently associated Interestingly the neurological/brain changes associatedoculomotor anomalies, visual field loss, and vision with this disorder can even mimic many of the behaviorsinformation processing problems seen in children.3 seen in those with PCVI as well.12Some researchers suggest that the phrase cognitive Should we be concerned about how PVCI isvisual dysfunction (CVD) be used to identify the defined? Absolutely. There are instances where notmany visual perceptual anomalies associated with this only do the numbers of individuals with the diagnosiscondition.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 andwith 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 developmentalinfants 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 tomonths, 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 meetinginfant visual development.7 Even though infants with the group of presenters met to review all that wasDVM were first described in the 1920s, there is little discussed the previous day. It was decided that theconsensus as to the etiology of this disorder. There term “pediatric cortical visual impairment” was theare several types of DVM with type I being described preferred terminology to use since this is very specificearlier in this paragraph. DVM type II is characterized about the group of individuals being discussed (thoseby problems with attention and fixation but is also with cortical visual impairment only and no otherusually associated with other neurological and/or developmental, cognitive or developmental issues). I116 Optometry Vision Development
  3. 3. noted that most practitioners, therapists, and teachers Directionality, visual motor integration, non-motorsee children with additional issues such as motor perceptual skills, and auditory perceptual/processingchallenges, 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 bothI suggested that the term, pediatric “cerebral visual functional vision and vision function anomalies thatimpairment” is much more appropriate. interfere with the development of appropriate vision and auditory information processing ability.282. 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 onlynumber of assessment procedures to ascertain the confirmed what you already knew. These tests addedlevel of ability of those with pediatric cerebral/cortical to the costs involved in managing patients and usedvisual impairment. We need to assess vision function valuable resources unwisely. I noted that the use ofas 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 confirmdetermination of the clarity of vision (visual acuity, improvement once therapy was begun and could helpcontrast sensitivity, refractive error), oculomotor guide the therapist in what approaches worked best.ability (pursuits and saccades; convergence anddivergence), accommodation (focusing), depth 3. herapeutic Strategies For the Treatment of Tpercep­tion (3D vision) and eye health.14,15,16 It is Pediatric Cerebral Visual Impairmentalso often appropriate to use special diagnostic All treatment should begin by paying attentiontools 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. Anyresponse; VEP, visual evoked potential) to determine problems that need to be addressed to insure the bestthe level of ability present. Those with a wide range of possible eye health should be instituted. If uncorrecteddisability tend to show many anomalies in the various refractive error is present, it should be diagnosed andareas 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 smallbe conducted as well. Those with disability tend to amount of refractive error for those with traumatichave functionally induced disability that often overlays brain injury can improve these individuals’ qualitypathologically induced disability, so that the end result of life.29 Remember that spectacles can not only beis often greater than one might expect from either corrective/compensative in nature but they can alsoanomaly individually. For instance a large amount be therapeutic as well.of uncorrected refractive error (hyperopia, myopia, Children with high amounts of hyperopia andastigmatism) could cause amblyopia (a functional those with accommodative dysfunction (includinganomaly) that magnifies any vision loss due to individuals with Down Syndrome, Cerebral Palsycerebral/cortical impairment. The amblyopia also and brain injury) often benefit from a multi-focalinduces numerous vision information processing prescription where an added “+” power is given either inanomalies that impede a child’s daily living skills a multifocal prescription (bifocal) or as a secondary pairdevelopment and his or her ability to navigate the of spectacles to use for specific tasks. Individuals withworld about them. Children with Down Syndrome significantly decreased vision at near can also benefitfor 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 andusing a computer to reading a book.21,22,23 Those with corrected, and any therapeutic applications addressedCerebral Palsy will display oculomotor, visual motor appropriately for use with a spectacle prescriptionintegration and accommodative problems along with (bifocals, prism, sector occlusion, etc), then it is timehigh 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. Itinformation processing (VIP) and the development was suggested that Facebook can be a unique resourceof appropriate visual perceptual skills.27 Laterality/ for therapeutic ideas as well as other internet resourcesVolume 43/Number 3/2012 117
  4. 4. (Thinking Outside the Box,36 Maino’s Memos,37 Table 1: Medication Side Effects adapted from RJPinterest38). 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 Mentaleye movement and hand-eye, accommodation, Illness and Dual Diagnosis.Optometry 2009;80:249-254.convergence/divergence, and other aspects of both Systemic Oculovisualvision function and functional vision, as well as visual Side Effects Side Effects Antipsychotics Bone marrow Blurred visionstimulation activities were presented as well. depression Although children with cortical/cerebral visual Muscle spasms/ Light sensitivityimpairment have significant neurological impedi­ twitches Breast enlargement Visual Disturbancesments, the principles of neuroplasticity can be applied (M F)to the various therapeutic approaches utilized for this High body Mydriasis temperaturepopulation.30 In most instances the therapy is not Antidepressants Abdominal pain/ Blurred visionrehabilitative in nature, but rather habilitative. This constipationdifference is important to remember when caring for Abnormal dreams/ Increased risk of thinking Glaucomathose with PCVI. Abnormal Visual Disturbances Post conference commentary: The large number of ejaculation/orgasmteachers in the audience often directed the discussion Anxiety Photophobiatowards diagnosing and treating the educational Anticonvulsants Memory problems/ Blurred vision amnesiaconcerns present for these children. Christine Roman Sedation Dimming of visionLantzy, Ph.D, frequently referred to her text, Cortical Insomnia DiplopiaVisual Impairment: An Approach to Assessment and Bronchitis Involuntary eyeIntervention, as the best way to determine various movements Fluid retention Dry eyelevels of function and to treat PCVI.31 Anti-Parkinson’s Abnormal dreams/ Vision abnormalities insomnia4. 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 anglemany more medications than those not demonstrating in men GLC Breathing problems Cycloplegia/Mydriasisa disability. They also often have a slightly higher affinityfor the development of adverse effects due to various Insomnia Decreased visionenvironmental factors. A paper in Optometry discussed Tardive dyskinesia Capsular cataractadults with not only a developmental disability but also Antianxiety Anemia Decreased accommodationa psychiatric illness that noted many of these individuals Seizures Nystagmuswere taking 10 or more, high powered neurotropic Blood disorders Diplopiaand systemic medications. Interestingly, seldom did Unusual excitement Mydriasisany of these individuals complain of symptoms relatedto their disability, systemic anomalies, or medication mental illnesses in children. Pediatric depression is alsoside effects.32 Certainly those who are significantly being diagnosed often, let alone all the medicationsyounger than the population described above may also currently being used for behavioral issues such asfind it difficult to communicate their needs, wants and attention deficit hyperactivity disorder.34symptoms as well. One of the major environmental hazards those Various medications, alternative and comple­ with disability encounter are people. Many do notmentary 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 thenadverse, unintended events. (See Table 1). Although act on those assumptions. This is true not only foryou may think that a child is too young for many lay individuals but also for teachers and health careof these major drugs, you should realize that various professionals.35psychiatric anomalies such as pediatric bipolar Post conference commentary: Little was discusseddisorder is now one of the most frequently diagnosed on this topic after the meeting. It was decided,118 Optometry Vision Development
  5. 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 ChildrenCortical 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 Specialof 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 introductionImpairment 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, cerebralplanning 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 andMaino 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 deficitsReferences 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, Maino1. 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, cerebral2. 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 in4. 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 related5. 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 in7. 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 Dual11. 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 Dev12. 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 Williams13. 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 Disabilities14. 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 5/1215. 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/DRBgzVolume 43/Number 3/2012 119

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