Karen Barrows, PT, DPT, MHS, PCS Working with Children with Cortical Visual Impairment From a PT/OT Perspective Karen Barrows,  PT, DPT, MHS, PCS

Objectives Name the ten characteristics associated with CVI Understand the three phases of CVI progression Learn treatment techniques and therapy modifications to support and facilitate the vision of a person with CVI Generate treatment modifications and techniques to support persons with visual loss from cortical visual impairments (CVI) Understand the visual differences in those with CVI

CORTICAL VISUAL IMPAIRMENT…A WHOLE NEW PARADIGM “Visual impairment due to damage or disorder of the visual pathways and visual centers in the brain, including the pathways servicing visual perception, cognition, and visual guidance of movement.” Can coexist with ocular motor disorders and can be the result of perinatal brain dysfunction or trauma. CVI affects nearly 2/1000 live births and accounts for 20-25% of visually impaired children in developed countries. (Dutton, Lueck 2015) A child with CVI meets the following criteria: 1. an eye exam that can’t fully explain the child’s use of vision 2. history or presence of neurological issues 3. presence of behavior or visual responses collectively associated with CVI

Two main streams of neural visual information: Dorsal: visual pathway between the occipital and posterior parietal lobes, the “where” and “how” pathway. It is used to localize and visually search, switch attention, and visual guidance of movement, mostly subconsciously. Ventral: visual path between occipital and temporal lobes. Determines “what is it” to understand what we see, conscious vision. The right temporal lobe provides the main library of all the faces we recognize, the left lobe is the main library of shapes and objects

Characteristics associated with CVI: (Roman-Lantzy 2018) Color Movement Latency Fields Complexity of object Complexity of array Complexity-sensory Complexity of faces Light Distance Reflexes Novelty Absence of visually guided reach

Three phases associated with the development of CVI, based on Dr Three phases associated with the development of CVI, based on Dr. Roman Lantzy CVI range testing Phase I: score of 0-3 on the range and refers to the least visually aware level. The focus of this phase is in building visual behavior throughout the child’s day, emphasizes environmental modifications to allow the child to use his vision more consistently in preferred environment and with preferred objects. Phase II: score of 3-7 on the range and refers to integrating function and vision. The focus of this phase is to build functional use of vision Phase III: score of 7+, the focus is on more challenging visual skills such as negotiating the environment, reading, and use of vision throughout the day.

Basic Modifications for all phases: have a plain background available/reduce clutter have the ability to reduce the light intensity of a room and cover strong light sources eliminate artificial aromas reduce/eliminate unnecessary sounds wear plain clothing or cover busy patterns when working face to face have CVI appropriate toys/objects/technology available

Basic Modifications for all phases: have options for positioning the child and the objects being seen have child wear corrective lenses if available be mindful of the need for rest breaks work as a team to provide visual reinforcement during a child’s day obtain visually specific information such as acuity, visual fields, and CVI range when expecting physically demanding skill, expect visual needs to be simplified

“3 Ds” of intervention program Development: provide stimulation and activities at the child’s current developmental levels and increase complexity over time Diversity of visual stimulation: initiate with regard of preferred objects, advance to generalized objects then novel Duration: increase the length of time of visual use over the day

Phase I (Roman-Lantzy 2018) building visual behavior throughout the child’s day, emphasizes environmental modifications to allow the child to use his vision more consistently in preferred environment and with preferred objects.

COLOR: (Roman-Lantzy 2018) Determine if there is a preferred color, highly saturated primary colors often best Keep objects limited to one color Have objects stand out against a neutral background

Movement: (Roman-Lantzy 2018) Gently move object (gentle shake) within child’s visual field Use objects that have reflective properties (perceived as movement) Limit distraction of unintended outside movement i.e.. people walking close by, other objects moving

Video: Josian with red balloon video

Latency (Roman-Lantzy 2018) Determine the length of time an object needs to be presented before a response is given. Keep in mind that this can change throughout the day due to fatigue, interest, well being Note the length of time required to respond to a variety of objects and activities to determine those most preferred Use preferred objects/activities to increase visual use and slowly add more novel objects to determine progression and challenge the child’s visual use (discuss with TVI how to progress to novel) Video: Leonie with yellow light video

Fields (Roman-Lantzy 2018) Determine preferred fields of visual use, remember that lower visual fields are often impaired therefore present objects in a higher field with the use of slant boards or other supportive surface Most often a child prefers a peripheral field for visual regard, one is often preferred over another Present an object/activity in the preferred field and over time work toward accessing the other lateral field, superior, midline, and inferior

Complexity of object (Roman-Lantzy 2018) Keep object to one color without designs to break the outline of the object

Complexity of Array (Roman-Lantzy 2018) Keep background as neutral as possible by reducing light in room and using a neutral screen/wall (black, white, cream etc.) Assess the environment and cover visually complex areas that the child may be working in, such as sheets to cover a bookcase, use of an invisiboard to block a busy view, or use a purposefully blank wall as a backdrop for focused activities

Complexity-Sensory (Roman-Lantzy 2018) Assess the sensory levels of the treatment area Keep sound, intense light sources that are distracting, smell, and temperature at reduced/modulated levels or reduce distractions if possible

Complexity-Faces (Roman-Lantzy 2018) Do not assume that the child should look at your face. At this phase the face is too complex to process. Looking away and listening to your voice will likely be your best response (face video) Josian face regard

Light (Roman-Lantzy 2018) Reduce the ambient lighting in the room to reduce distraction of the environment and reduce overall complexity Provide direct light sources on the object to gain increased visual attention, trial different color lights such as red and yellow Utilize objects that are lit from within or behind. A light box is a good tool or an ipad with a very simple application i.e.. Fluidity, Electra Video: Savannah activating CVI friendly toy video

Distance (Roman-Lantzy 2018) Determine the distance that the child uses his vision best. Start by holding a simple object within a foot of the child and slowly progress outward. Assess the child’s ability to stay focused on the object. As each session occurs, you may find that the distance of best visual use varies due to fatigue, illness, and complexity of object. Generally the closer the object, the easier to use vision. Describe CK with ipad beyond 2 feet

Video: Cyrus walking with ipad video

Novelty (Roman-Lantzy 2018) What are the child’s favorite/preferred toys toy may appear to be too visually complex, but the child may be focusing on the sound and its familiarity Slowly increase the novelty of object by presenting new objects with visual similarities Present familiar and slightly novel objects within a session as tolerated Novel objects presented repeatedly become familiar and preferred to

Phase II (Roman-Lantzy 2018) integrating function and vision. The focus of this phase is to build functional use of vision

Color Child can now attend to more colors although highly saturated are still preferred Color highlighting of an object’s salient, 3-D or 2-D features is needed

Video: Abigail standing with ipad video

Video: Emmett pouring video

Movement Movement may still be needed to elicit attention but not always to sustain attention Still easily distracted with movement in close environment (approximately 8 feet)

Latency Latency is steadily reducing especially with familiar objects and when well rested and feeling well Latency can increase when the child has tired of the object/activity or is fatigued or ill

Fields The child is able to localize objects in an additional peripheral field although may still prefer one Continue to present objects in other fields to determine the child’s ability to localize and fixate, don’t always work in the preferred field, but encourage using another

Complexity of object The child can localize or fixate on objects that have two or more colored surfaces Two dimensional objects may be introduced on backlit surfaces, i.e.. ipad

Complexity of array General lighting of room may increase Backgrounds may need to stay neutral but may start to become patterned with two colors Simple two-dimensional objects may be detected especially familiar Backlighting or focused light may not be required though may improve focus on novel objects

Complexity-sensory Now able to visually localize and fixate even with low intensity, familiar sounds and when other single sensory inputs are present As the child progresses, more complex sensory backgrounds may be tolerated

Complexity-faces Brief fixation on familiar faces may be possible, especially parents Brief eye contact with image in mirror may be possible

Light Changing of room lighting usually not required Child may be distracted by direct light sources (windows) but can be more easily re-directed to task Visual fixation may occur with 2 dimensional materials on a lighted surface

Distance Ability to locate and fixate on a target that is farther away, 6-10 feet, possible or more, familiar objects are often located more easily

Novelty Able to fixate on objects that are similar to familiar objects or on novel objects after several exposures. Able to view 2 and 3 dimensional material although may not be able to interpret what the object is. Some visual curiosity seen

Visually Guided Reach Often in this phase a child will quickly glance at the object, look away and then reach Video: Savannah reaching for switch

Phase III (Roman-Lantzy 2018) focus on more challenging visual skills such as negotiating the environment, reading, and use of vision throughout the day

Color Color highlighting of materials or environment may be needed Video: Striped ball rolling

Movement May be occasionally needed to get attention but not to sustain May be distracted with objects farther away, 8+ feet Movement will continue to alert to object

Latency Reduced periods of latency and reduced length of latency noted Occurs primarily when tired, ill, hungry, postictal

Fields Able to visually fixate in three fields though may still prefer one or two Lower visual field may still be atypical (often likely)

Complexity of Object Objects can be fixated upon and recognized with 3+colors or patterns on surface Two dimensional images may not require backlighting Use salient features to identify objects/images

Complexity of Array Able to visually fixate on objects against more complex backgrounds 2 dimensional images detected against a background with 3+ additional elements (more complex pictures)

Complexity - Sensory Able to visually fixate with increasing levels of competing sensory information Novel environments may require a reduction in sensory complexity

Complexity- Faces Eye contact with familiar and unfamiliar people increases May be less attentive to a new or unfamiliar person More typical response to mirror image

Light Light gazing no longer an issue May attend to strong light sources when tired, ill, or stressed Backlighting continues to assist in regard and identification of 2 dimensional objects

Distance Able to locate and fixate on targets farther away, as great at 10+ feet Able to see and attend to objects in familiar and novel settings May attend to large moving objects at great distances, 15+ feet

Novelty Utilizes salient features to recognize and identify new objects or images Visual curiosity in new environments is seen

Visually guided reach Visually guided reach and grasp is seen at all times

Reminders A child’s ability to use vision under any characteristic can fluctuate greatly due to internal and external issues such as illness, fatigue, novelty and complexity of environment Do not assume an expected level of visual use at the start of each activity, assess the child’s function at the time and modify as needed If the child is not appropriately supported or is purposefully being challenged to hold positions with physical effort, expect visual skills to be further challenged Do these characteristics resemble behaviors seen in ASD? Even if person is in Phase III, some characteristics such as complexity and fields may never fully resolve

Questions/Discussion

References Fulton, A. (2017, April). Ophthalmology and Cortical Visual Impairment in Third Annual CVI Symposium. Perkins School for the Blind, Watertown, MA. Lueck, AH, Dutton, G. N. (Eds.). (2015). Vision and the brain, understanding cerebral visual impairment in children. NY: AFB Press. Holbrook, C., Koenig, A. J. (Eds.). (2000). Foundations of education, 2nd edition: Vol. 1. NY: AFB Press. Roman-Lantzy, C. (2018). Cortical visual impairment, an approach to assessment and intervention, 2nd edition: NY: AFB Press. Presenter, Mazel, E. (2016, July). Cortical visual impairment. presented at Perkins School for the Blind, Watertown, MA. Presenter, Roman-Lantzy, C. (2016). Perkins CVI training initiative. Presented at Perkins School for the Blind, Watertown, MA

References Third annual CVI symposium: best practices and current research, Dr. Anne Fulton, 4/7/2017 Foundations of education seconds edition Vol 1. M. Cay Holbrook, Alan J. Koenig Eds, AFB Press NY 2000 Vision and the Brain, understanding cerebral visual impairment in children, Amanda Hall Lueck, Gordon N. Dutton Eds, AFB Press 2015 NY Cortical Visual Impairment-an approach to assessment and intervention, 2nd ed, Christine Roman-Lantzy AFB Press NY 2018