ANDERS ZIENNeurovisual disorders in children
CVI
Patricia Delbeke18-02-2019
WHAT ?
• CVI = Cerebral Visual Impairment
• Disorder in the visual system without structural ocular abnormalities
• Brain problem: disturbance of higher order visual functioning
WHERE IS WALLY ?
WHERE IS THE CHOCO ?
Occipital lobes
• Break up information
• ~ Post Office :• Movement
• Colour
• Contrast
• Vision Acuity
• Visual Field
Visual Fields
• Right Visual Field => Left Visual Cortex
• Left => Right
LR
Cystic Encephalomalacia F-T-P Left Right hemianopsia
2 functional streams
• Ventral stream• Temporal lobes
• Dorsal stream• Posterior parietal lobes
• Motor cortex
• Frontal lobes
• Conscious vision
Ventral stream “What” area
• Temporal lobes • Right Face recognition / Route finding
• Left Object & Words recognition
• “Visual library”
Dorsal stream “Where” area
• Posterior parietal lobes:• to be aware of the whole visual scene & to select out
• Motor cortex• visually-guided movements
• to take a book / to lift the feet
• Frontal lobes :• work strategy : to make choices
• eye movements
ACTION
PLANNING
Interconnections - Bidirectional
Cerebral Visual Impairment = CVI
• Visual disturbance on account of • Oculomotor incoordination and
• Visual perceptual impairment
Pathology of the visual cortices and their interconnecting pathways
• Heterogeneity of expression• Complexity of visual processing
• Children (≠ adults)• Early-onset damage brain is followed by brain growth and development
• Diffuse damage
• Brain plasticity : Training => alters visual outcome
• CVI can improve over time
CVI : causes
• Hypoxic-ischemic encephalopathy 65%
• CNS malformations 12%
• Metabolic and neurodegenerative D 10%
• Hydrocephalus 3%
• CNS infections 3%
• Epilepsy 2%
• Neoplasia 2%
• Traumata 3%
Journal of Child Neurology March 2007
Ischemic damage : premature infant
• ↓ Perfusion of the brain
• Focal necrosis
• Periventricular white matter = watershed vascular supply
• Periventricular leucomalacia
• CP and CVI
PVL and Papil atrophy
• Retrograde transsynaptic degeneration• Axonal loss
Papil : Pallor and cupping
Damage ventral stream (Temporal Left)
• Visual agnosia : • Shape and objects
• Letters : reading disorder
• ~ Right hemianopia
Damage ventral stream (Temporal Right)
• Face recognition (Prosopagnosia)
• Facial expression
• Orientation (Topographic agnosia)
• ~ Left hemianopia
Damage dorsal stream
• Simultaneous perception• To much information
• “Crowding”
• Depth-interpretation
• Visual guidance of movement
• Strabismus - Nystagmus
• ~ lower VF defect
Diagnose CVI in children < 3 yrs old
• ‘Observation’
• Variable, poor, short response to visual stimuli
• Preference for looking at light
• Difficulties to look and handle
• Color preference
• Better visual performance with movement
CVI bilan in children > 3-4 yrs old
• Multidisciplinary• Ophthalmologist
• Anamnesis – Questionnaire / complete ocular examination
• Orthoptist / Psychologist• Orthoptic evaluation / Visual-perceptual tests / IQ test
• Neuropediatrician• Developmental examination
• Neuroimaging
• Co-working with COS – Revalidation Centra – CPRC – Home Guidance
Case 1 Elle 5 yrs old
• > COS : suspicious of CVI• Difficulty selecting a toy in a box DS - too complex visual scene
• Tripping over obstacles DS - impaired visually guided movement
• Getting lost when furniture is moved VS - impaired orientation
• 3th nursery class - GON type 4
• Atcd : • Premature GA 28 Wks
• Perinatal cerebral hemorrhage Left with hydrocephalus
• CP right-sided
1/ MRI brain
• Loss of volume of white matter periventricular
R L R L
Axial Coronal
2/ Ophthalmological examinationOphthalmologist & Orthoptist
• Microstrabismus - No stereo
• Suboptimal vision : 0,6 BE with +3 D
• No VF defects
• Fundus :
3/ IQ testing Psychologist
• WPPSI-III NL T 104 V 108 P 95
• Block design• Spatial visualization
• Motor skill
4/ Visual-Perceptual examinationOrthoptist - Psychologist
• In-Zicht : screeningstool to observe the visual skills
Fag foto KS fotopicnic foto
“busy” background Visual spatial perception Viewing strategy
example result
• L94 Visual perceptual battery• Object Recognition test
• Visual information is presented in a complex manner
<- Overlapping line drawings
<- Unconventional object views
Score < Pc 3
Conclusion
• Visual-perceptual problems
• Dammage visual pathways and associated visual areas => CVI
• Tips and Tricks• Continuation of GOn • Training viewing strategy• Visual comfort
• in front of the class, use finger-ruler, structured working sheets• Home Guidance: advice family - learn to cycle
As ophthalmologist : always exclude ocular pathology
• Refraction errors ? • Myopie – Hypermetropia – Astigmatism
• Accomodation problems for reading ? • Readingglasses
• Cataract ? Retinal disorders ?
Case 2 Amin 7 yrs old
• Part of a twin
• Neurosensorial hearing loss - Cochlear Implant
• ASS
• Difficulties to cath a ball
• Needs light to see better
• Suspicious of visual-perceptual problems : CVI ?
Fundus
Concentric VF defect Nightblindness
Usher Syndrome – No CVI
Case 3 Stan 8 yrs old
• > Independent care and counseling centre : suspicious of CVI
• 2nd primary school – GOn type 4
• DCD with dysgraphia
• T IQ 98 “discrepancy” V IQ 108 P IQ 88
1/ MRI Brain
• Prominent enlargement of “perivascular” Virchow Robin spaces• Developmental brain disorder
BumpingFluctuations in visionCatching a ballReading the clock – timeline…
2/ Questionnaire
=> Negative score
3/ Ophthalmological examinationOphthalmologist & Orthoptist
• Nl Vision : 1,0 BE • BUT :
Incapable to switch • matching-paper <> optotypes
• desk <> blackboard
• Ocular motility• No dissociation between head and eyes• Synkinesias
• Mouth movements • Frowning
3/ Visual-Perceptual examinationOrthoptist - Psychologist
• DEM test (Developmental Eye Movement) In-Zicht
horizontal lines < Pc 1vertical lines Pc 90
• DTVP-2 (Developmental Test of Visual Perception)
visual perception : normal visual-motor integration : low
Conclusion
• Discrepancy IQ – Abnormal MRI – features of CVI• No CVI
• DCD with visual-motor problems
• Tips and tricks • Training gaze movements far<>close
• Training viewing strategy
• Structured work sheets
• Typ 10
Case 4 Quinten 12 yrs old
• Autism
• 2 strokes – interval 10 days – cause ?
• Occlusion both aa cerebri post
Ischemia occipital lobesR > L
Ischemia R cerebellum
Cortical blindnessVA < 0,05No colour perception
• Center for Visual Revalidation• Digital working – dactylo
• Software Supernova : magnifier and screen reader
• Bednet
• Visual perceptual problems ?
1/ Ophthalmological examinationOphthalmologist & Orthoptist
• Vision : 1,0 BE
• Nl colour
• Goldmann VF : Le Hemianopia
• Torticollis headturn to the Left
2/ IQ testing Psychologist
• WISC-V-NL• TIQ: 89, below average
• Verbal Comprehension: Q 116, above average
• Processing Speed: Q 45, very weak
3/ Visual-Perceptual examinationOrthoptist - Psychologist
Motivated boy +++
• DEM-test < Pc1 : severe problems with scanning
• DTVP-test < PC1
• In-Zicht : crowding – complex designs – time consuming
Conclusion
• CVI - hemianopia
• Tips and trics• Type 9 with CVI approach – Home Guidance
• Preferred seating in the classroom, left
• He needs• Extra time to look at material
• Less visual complexity (classroom, working sheets)
• Vision breaks
Thank you