Ophthalmologic Examination Integrated With The Functional Aspects of CVI Sharon S Lehman, MD FAAP Chief Ophthalmology Nemours Children’s Clinic Clinical Professor of Ophthalmology and Pediatrics Robison D Harley MD Endowed Chair of Pediatric Ophthalmology

12 month ex 23-week premature infant with tracheostomy, h/o retinopathy of prematurity who has been recently diagnosed with cerebral palsy. Child’s mother is concerned that child looks past her, stares at lights and looks away when reaching for an object.

OPHTHALMOLOGIST REPORT: IMPRESSION: ROP RESOLVED; NORMAL EYE EXAM RECOMMENDATIONS: F/U 1 YEAR

What my exam should include… Obtain history focused on child’s visual behavior/characteristics Validate parent’s observations Learn patient’s expectations for their child Perform complete ophthalmologic examination Provide medical diagnosis and medical necessity Refer for services for visually impaired Make specific recommendations tailored to individual patient Provide information to patient’s team Provide educational materials/support group information Provide opportunity for parent’s to ask questions

Classic Ophthalmologic Exam – Exam #1 Focus: History and Structure History with CVI modified questionnaire Physical-Classic Ophthalmologic Exam Visual acuity Pupillary reaction External Anterior and posterior segment Alignment and motility Cycloplegic refraction

CVI Ophthalmologic Exam #2 Focus: Function Classic Ophthalmologic Exam #1 History with CVI modified questionnaire Structural Physical exam + Physical Accommodative amplitude/dynamic retinoscopy Convergence amplitude/NPC Visual attention/fixation/visually guided behavior Latency Saccades/pursuits Directionality of line Contrast Sensitivity

CVI Ophthalmologic Exam #3 Focus: Higher Level Function Classic Ophthalmologic Exam #1 + CVI Ophthalmologic Exam #2 Physical Executive functions Figure ground skills Comparison of size and shape Visual center

(History and Structure) CVI Type 1 Exam (History and Structure) CVI Type 3 Exam (Higher Level Function) CVI Type 2 Exam (Function) Low function High function Phase 1 Phase 2 Phase 3

What my exam is not… It is NOT a substitute for evaluation by Teacher of Visually Impaired It is NOT a substitute for evaluation by Occupational Therapist It is NOT a substitute for evaluation by Physical Therapist It is NOT a substitute for evaluation by Learning/Reading Specialist

“Please tell me about your child’s vision”. HISTORY “Please tell me about your child’s vision”. There is no better expert than a child’s parent. They will often tell you most of you need to know.

CVI Modified Questionnaire Preference for specific colors:  Improved visual response with movement: Latency (delay in response to visual stimuli Abnormal field of vision (eg: hemianopsia or island of visual response) Difficulty with visual complexity: Preference for looking at lights and non purposeful gaze Difficulty with distance viewing Atypical visual behavior (eg: frequently looking up or away while tracking, eccentric viewing) Difficulty with visual novelty Absent or clumsy visually guided motor responses Adapted from: Roman-Lantzy, Christine; Cortical Visual Impairment: an approach to assessment and intervention; American Foundation for the Blind; New York, New York; 2007 M

Multidisciplinary team approach Parents/family Primary care physician Occupational therapist Pediatric ophthalmologist Pediatric neurologist Physical therapist Speech therapist Educational specialist Feeding specialist Teacher of visually impaired Teacher of the hearing impaired

EXAM #1 History with CVI modified questionnaire and basic ophthalmologic exam r/o structural eye abnormalities/confounding factors Determine any necessary optical correction Determine any necessary medical or surgical ophthalmologic treatment Child with limited visual response who is learning to use vision

EXAM #2 History and Modified CVI Questionnaire and Exam #1 Additional aspects of exam Accommodative amplitude/dynamic retinoscopy Convergence amplitude/NPC Visual attention/fixation/visually guided behavior Latency Saccades/pursuits Directionality of line Contrast Sensitivity Child with higher visual function learning to use vision more effectively along with other functions of daily life

EXAM #3 History and Modified CVI Questionnaire and Exam #1 + Exam #2 Physical Executive functions Figure ground skills Comparison of size and shape Visual center CVI characteristics improved or resolving with higher level visual function deficits (Cerebral?)

(History and Structure) CVI Type 1 Exam (History and Structure) CVI Type 3 Exam (Higher Level Function) CVI Type 2 Exam (Function) Low function High function Phase 1 Phase 2 Phase 3

What my exam should include… Obtain history focused on child’s visual behavior/characteristics Validate parent’s observations Learn patient’s expectations for their child Perform complete ophthalmologic examination Provide medical diagnosis and medical necessity Refer for services for visually impaired Make specific recommendations tailored to individual patient Provide information to patient’s team Provide educational materials/support group information Provide opportunity for parent’s to ask questions

12 month ex 23-week premature infant with tracheostomy, h/o retinopathy of prematurity who has been recently diagnosed with cerebral palsy. Child’s mother is concerned that child looks past her, stares at lights and looks away when reaching for an object.

OPHTHALMOLOGIST REPORT: IMPRESSION: ROP RESOLVED; NORMAL EYE EXAM RECOMMENDATIONS: F/U 1 YEAR

Ophthalmologist’s Report Impression/Diagnosis Cortical visual impairment Severe visual impairment both eyes Retinopathy of prematurity Stage 2 Zone II both eyes – resolved Cerebral palsy Premature infant Hypermetropia both eyes not requiring correction

Ophthalmologist’s Report Recommendations Treat underlying medical conditions. No glasses are necessary Recommendations: Use high contrast and brightly colored visual materials. Use additional cues (lights, sound, vibration and movement) to attract visual attention. Avoid overstimulation. Provide support for child’s body when performing visual tasks. Avoid verbal distractions when child is performing visual tasks. Perform visual tasks in non-distracting environment. Refer for evaluation for services for vision.

Ophthalmologist’s Report Recommendations Copy of diagnosis, medical necessity and recommendations sent to patient’s team Educational material and support group information given to family Family’s questions answered, they expressed understanding and agree with plan Include contact information so other members of child’s team can share information.

PEDIATRIC OPHTHALMOLOGISTS: Would you like to learn more about CVI PEDIATRIC OPHTHALMOLOGISTS: Would you like to learn more about CVI? GOOD NEWS! Reference: Lehman SS, Attitudes Concerning Cortical Visual Impairment Among Pediatric Ophthalmologists and Teachers of the Visually Impaired¿ presented at 2016 American Conference on Pediatric Cortical Visual Impairment, Omaha, Nebraska, July 8-9, 2016

Modalities to Assist Pediatric Ophthalmologists with CVI Providing education and templates for: History Physical examination Providing diagnosis Referral for services Making recommendations/interventions

TEMPLATES FOR RECOMMENDATIONS/INTERVENTIONS Should not be cookie cutter. Need to be specific to each child’s circumstances Need to be incorporated into child’s activities of daily living Need to include environmental modifications Need to include developmentally appropriate recreation (play) for older children

TEMPLATES FOR CVI INTERVENTIONS/RECOMMENDATIONS FOR PEDIATRIC OPHTHALMOLOGISTS Characteristic Interventions for child with limited function (lower phase) Interventions for child with better function (higher phase) Preference for specific color Exploit color preference in choice of visual objects of regard. Use objects of preferred color for ADL. Have teacher, instructor, or therapist wear preferred color in order to maintain interest and localization for following (e.g.: learning to drive motorized chair).

TEMPLATES FOR CVI INTERVENTIONS/RECOMMENDATIONS FOR PEDIATRIC OPHTHALMOLOGISTS Characteristic Interventions for child with limited function (lower phase) Interventions for child with better function (higher phase) Latency Provide extra time for localization and following of visual stimuli. Gradually work toward decreasing time needed for localization and following. Provide extra time for completion for educational tasks.

Please email me at: slehman@nemours.org I need your feedback about improving the pediatric ophthalmologist’s care of children with CVI Please email me at: slehman@nemours.org