Optometric Care of Children with Developmental Disabilities Kia B. Eldred, OD, FAAO Diplomate in Low Vision

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1 Optometric Care of Children with Developmental Disabilities Kia B. Eldred, OD, FAAO Diplomate in Low Vision 1. Demographics Who is the Developmentally Delayed child? The child who is significantly behind in development so that information gathered during the examination must be done so with measures that would not be age appropriate. Congenital malformations occur in 3% of births 2. Causes of Developmental Delay a. Cerebral Palsy 2 to 2.5 cases per 1000 births b. Autism 2.6 cases per 1,000 births, second child 9% c. Down Syndrome 1 in 800 births d. Fetal Alcohol Syndrome.3 to 2.2 cases per 1,000 births 3. Need for Examination Increased population who are served in the educational systems as more children survive from improved medical care at birth. Teachers need to know the best methods to educate the children. Quality of life and the child s long term abilities are determined by what they are learning on a daily basis in school and in preschool. 4. NOVA clinic Our current clinic at the University of Houston College of Optometry (Non- Invasive Objective Visual Assessment Clinic) Started in 1993 with myself and Dr. Ruth Manny as a team. Visited several other clinics in US UAB, UC Berkeley Primary objective is to assess multiply-impaired children CASE Patient History BR 8 year old female Chief Visual Complaint School feels BR may need glasses History Present Illness Sees better up close (school report) Mother feels BR sees well Goals Quantify vision, improve function

2 Past VA measures: FF and CSM Birth History Uncomplicated pregnancy 26 to 30 weeks twin gestation (twin B) Birth weight 3 lbs 2.5 oz (1432 grams) APGAR 6 (1 min) 8 (5 min) Hospitalized 2 months 5 days after birth Required O 2 for 39 days Medical History Periventricular leukomalacia (PVL) Cerebral Palsy Patent Ductus Ateriosis requiring 36 days Tympanostomy tubes (prior to 17 months) Allergies None Family Medical History Non-contributory Ocular History (review of past records) Retinopathy of Prematurity 7 wks: Grade I Nasal & Temporal OU Clear demarcation line with tortuosity Normal posterior pole ROP regressed over the next 16 months (examined at 2, 3, 7, & 18 months) 18 mo: normal fundus Medications Carbamazepine (Tegretol) - used in treating generalized tonic-clonic and mixed pattern seizures Ocular Side effects Diplopia Blurred vision Mydriasis Decreased accommodation Heavy feeling to the eyes Neurological reactions Downbeat nystagmus Drowsiness Ataxia Warnings Monitor blood counts Caution in patients with Cardiac problems Liver dysfunction, Renal problems

3 More current medications Keppra, Trileptal, Lamictal, Topamax, Zonegran, Felbatol, Gabitril, Neurotin Educational History Mainstreamed in 1st grade Northside Elementary Angleton ISD Services from Region IV Occupational therapy Physical therapy Speech therapy History Ocular History (From records) 7 mo: Aligned 17 mo: 35 AET 20 mo: 60 AET bilateral MR recession 6 mm 23 mo: 10 AXT 25 mo: 15 AXT, 10 RHT, OS preferred begin occlusion 2hrs OS mo: LXT, 30 RHT Occlusion 4 hrs OS Patching discontinued: date uncertain Second strabismus surgery in future A. Measurement of Visual Acuity Visual Acuity is difficult to assess Difficulty with Fix and Follow Color may be helpful Preferential viewing most practical method- reliable method and validated as a measure The tests we typically use in NOVA clinic include Teller Acuity and Cardiff Cards. Measurement of Visual Acuity Level of acuity may vary greatly in this population If the cause is Cortical Visual Impairment it may vary from normal to very visually impaired. Visual crowding may be a problem when comparing grating to optotype acuity testing. (ie children tested with grating acuity in infancy could show reduced optotype acuity in childhood). Visual Acuity Lea Symbols - without correction OD 4/12.5 (20/62.5) OS 4/12.5 (20/62.5)

4 Issues High Function Forced choice, matching, verbal Forced choice pairings Circle/Apple Square/House Circle/House B. Visual Fields Visual Fields vary central scotomata, constricted fields, peripheral field defects, hemianopic defects or Swiss cheese fields. Patients with CVI see moving objects better than static objects. Bright object brought in from the peripheral fields and observing the patient for a turn of the head or eyes. The larger and brighter the stimulus the less accurate the field, but sometimes we have no choice to attract their attention!! Restrictions are probably related to interruption of the axons in the optic radiation. May also have variability when measuring because of problems with simultaneous attention. This may explain problems in crowded situations and may affect reading. Concerns Repeatability Stimulus control Contributions Text placement Mobility Adaptations C. Binocular Vision Constant >60 A XT OS Fixation preference Issues Visual fields Surgical management Strabismus measurement If possible cover test is performed, but often not possible due to poor fixation or nystagmus Hirschberg with Krimsky are helpful for the squirming child. Helpful to keep in mind the functional implications of strabismus i.e. does it assist them to have a more functional field if they have some type of field loss? Strabismus therapy Amblyopia therapy can assist the CVI child with monocularly decreased acuity from strabismus. It must be kept in mind that the child could lose out on valuable developmental learning time if the worse eye has very poor vision.

5 Surgery is controversial, especially with PVL, as esotropia has been seen to spontaneously convert to exotropia in these children. Although some studies indicate acceptable cosmetic results from surgery. D. Eye Movements Defective smooth pursuits and inability to perform visually guided saccadic movements have been documented. Typically patients with CP have more difficulty because of head instability. B.R. had fairly smooth eye movements E. Accommodation MEM retinoscopy is used to measure and variable amounts of accommodation are found. Down Syndrome patients have been found to have high lags of accommodation secondary to inadequate accommodative/convergence systems B.R. findings: Dynamic Retinoscopy Large Lag (>1.50D) Variable accommodation F. Color Vision B.R. Pease-Allen Test 3 mo progress) Passed Blue/Yellow Fail Red/Green Issues Saturation Future Design G. Contrast Sensitivity Testing Not able to perform with every patient (fatigue) Used to make recommendations and for mobility problems. Test used in NOVA clinic: Cambridge Plates H. Refractive Error Wet Retinoscopy (1% cyclopentolate) OD x 090 OS x 100 G. Pupillary Reflex Pupillary light reflexes intact with CVI and most of the other syndromes mentioned Pupillary fibers leave the optic tract before the Lateral Geniculate Body. CVI by definition is due to lesions posterior to the LGN. Poor reflexes if coexistent disorder of the anterior visual pathway etc. I. Ocular Health External evaluation

6 Pupils: 3mm, D 1+, C 1+, (-)APD Anterior Segment: Unremarkable J. Intraocular Pressure (Tonopen) OD 16mm, OS 20mm Diation Tonometer option K. Fundus evaluation Unremarkable Regressed ROP by review of past records Optic Disk Characteristics with PVL It can be difficult to view the disk Early PVL, before 28 weeks may be associated with small disks Normal sized disks with large cupping and a reduced neuro-retinal rim may be due to later lesions - 28 weeks and later gestation. Spectrum of optic disk appearances L. Treatment Rx for full-time wear (Monovision) OD x 090; OS DS Polycarbonate Continue with services Issues Bifocal Posture and motor control Cognitive Function Difficulty testing visually and motorically impaired children This population typically performs poorly in tasks requiring spatial and visuoperceptual abilities. Difficulties in processing multiple targets and problems making saccades to peripheral locations are the main factors for the decreased perceptual impairment. Usually these children score much higher on verbal tests, even those with mild visual problems. Recommendation for Function The primary goal of most exams is to obtain more information for education and function. Very little information on this population. Key recommendations are to reduce visual clutter, determine appropriate near point size materials, use sound or color if possible. Orientation can be a problem, may need a cane. May need to use books on tape, computers with speech, CCTV Recommendations for Function Adjust lighting for optimum performance

7 Give child time to respond Use the same name for objects consistently Use consistent visual cues i.e. always use a red bowl at home and at school for meals Touch may be needed to be used with visual cues Recommendations for Function Moving objects may assist the child s attention The child may use peripheral rather than central vision The child may reach away after looking at an object M. Significant Areas of Assessment to Optimize Visual Function Visual Acuity Accommodation Visual Fields Color Vision N. Outcome Wears Rx full-time, likes spectacles More attentive at near Distance VA Lea symbols, cc (3 months later) OD 10/20; OS 10/50 Near VA Lea symbols dominoes, cc (13 months later) OD.30/2M; OS.30/1.25M Accommodation OD Large lag OS Accurate from 80cm to 15.2cm O. Ups and Downs in NOVA clinic Drawbacks Intense Inability to assist Rewards Expanding knowledge base Strength of caregivers Improve patient s quality of life Special rapport