The Multidisciplinary Guidelines for Diagnosis and Referral in Cerebral Visual Impairment.

Introduction: Cerebral visual impairment (CVI) is an important cause of visual impairment in western countries. Perinatal hypoxic-ischemic damage is the most frequent cause of CVI but CVI can also be the result of a genetic disorder. The majority of children with CVI have cerebral palsy and/or developmental delay. Early diagnosis is crucial; however, there is a need for consensus on evidence based diagnostic tools and referral criteria. The aim of this study is to develop guidelines for diagnosis and referral in CVI according to the grade method. Patients and Methods: We developed the guidelines according to the GRADE method 5 searches on CVI (children, developmental age ≤ 18 years) were performed in the databases Medline, Embase, and Psychinfo, each with a distinct topic. Results: Based on evidence articles were selected on five topics: 1. Medical history and CVI-questionnaires 23 (out of 1,007). 2. Ophthalmological and orthoptic assessment 37 (out of 816). 3. Neuropsychological assessment 5 (out of 716). 4. Neuroradiological evaluation and magnetic resonance imaging (MRI) 9 (out of 723). 5. Genetic assessment 5 (out of 458). Conclusion: In medical history taking, prematurity low birth weight and APGAR (Appearance, Pulse, Grimace, Activity, Respiration) Scores (<5) are important. Different questionnaires are advised for children under the age of 3 years, older children and for specific risk groups (extremely preterm). In ophthalmological examination, eye movements, specially saccades, accommodation, crowding, contrast sensitivity and visual fields should be evaluated. OCT can show objective signs of trans-synaptic degeneration and abnormalities in fixation and saccades can be measured with eye tracking. Screening of visual perceptive functioning is recommended and can be directive for further assessment. MRI findings in CVI in Cerebral Palsy can be structured in five groups: Brain maldevelopment, white and gray matter lesions, postnatal lesions and a normal MRI. In children with CVI and periventricular leukomalacia, brain lesion severity correlates with visual function impairment. A differentiation can be made between cortical and subcortical damage and related visual function impairment. Additional assessments (neurological or genetic) can be necessary to complete the diagnosis of CVI and/or to reveal the etiology.

Deficits in vision and visual attention associated with motor performance of very preterm/very low birth weight children.

AIM: To extend understanding of impaired motor functioning of very preterm (VP)/very low birth weight (VLBW) children by investigating its relationship with visual attention, visual and visual-motor functioning. METHODS: Motor functioning (Movement Assessment Battery for Children, MABC-2; Manual Dexterity, Aiming & Catching, and Balance component), as well as visual attention (attention network and visual search tests), vision (oculomotor, visual sensory and perceptive functioning), visual-motor integration (Beery Visual Motor Integration), and neurological status (Touwen examination) were comprehensively assessed in a sample of 106 5.5-year-old VP/VLBW children. Stepwise linear regression analyses were conducted to investigate multivariate associations between deficits in visual attention, oculomotor, visual sensory, perceptive and visual-motor integration functioning, abnormal neurological status, neonatal risk factors, and MABC-2 scores. RESULTS: Abnormal MABC-2 Total or component scores occurred in 23-36% of VP/VLBW children. Visual and visual-motor functioning accounted for 9-11% of variance in MABC-2 Total, Manual Dexterity and Balance scores. Visual perceptive deficits only were associated with Aiming & Catching. Abnormal neurological status accounted for an additional 19-30% of variance in MABC-2 Total, Manual Dexterity and Balance scores, and 5% of variance in Aiming & Catching, and neonatal risk factors for 3-6% of variance in MABC-2 Total, Manual Dexterity and Balance scores. CONCLUSION: Motor functioning is weakly associated with visual and visual-motor integration deficits and moderately associated with abnormal neurological status, indicating that motor performance reflects long term vulnerability following very preterm birth, and that visual deficits are of minor importance in understanding motor functioning of VP/VLBW children.

A functional approach to cerebral visual impairments in very preterm/very-low-birth-weight children.

BACKGROUND: Cerebral visual impairment (CVI) is a major cause of visual impairment, with very preterm birth/very low birth weight (VP/VLBW) being a major risk factor. There is no generally accepted definition of CVI. This study aims to investigate the usefulness of an empirically-based functional definition of CVI. METHODS: One-hundred-five VP/VLBW children and 67 controls participated. CVI was defined after comprehensive oculomotor, visual sensory and perceptive assessment, and validated against vision problems in daily life and in terms of intellectual, behavioral, emotional and social functioning, as well as use of therapeutic services. RESULTS: Twenty-four per cent of the VP/VLBW children met criteria for CVI, compared to 7% of controls (P = 0.006, OR: 3.86, 95% CI: 1.40-10.70). VP/VLBW children with CVI had lower performance IQ, but not verbal IQ, than those without CVI. Visual problems in daily life were confirmed in VP/VLBW children classified with CVI. Additionally, difficulties in behavioral and social functioning were most prominent among VP/VLBW children with CVI. CONCLUSION: In VP/VLBW children, CVI defined in terms of visual function deficits is accompanied by intellectual, behavioral, and social impairments, validating our operational definition of CVI. CVI might act as a marker for developmental problems in VP/VLBW children.

Visual search and attention in five-year-old very preterm/very low birth weight children.

INTRODUCTION: This study aimed to establish visual search performance and attention functioning in very preterm/very low birth weight (VP/VLBW) children using novel and well established measures, and to study their contribution to intellectual functioning. METHODS: Visual search and attention network efficiency were assessed in 108 VP/VLBW children and 72 age matched term controls at 5.5 years corrected age. Visual search performance was investigated with a newly developed paradigm manipulating stimulus density and stimulus organization. Attention functioning was studied using the Attention Network Test (ANT). Intellectual functioning was measured by a short form of the Wechsler Preschool and Primary Scale of Intelligence. Data were analyzed using ANOVAs and multiple regression analyses. RESULTS: Visual search was less efficient in VP/VLBW children as compared to term controls, as indicated by increased search time (0.31 SD, p = .04) and increased error rate (0.36 SD, p = .02). In addition, VP/VLBW children demonstrated poorer executive attention as indicated by lower accuracy for the executive attention measure of the ANT (0.61 SD, p < .001). No differences were found for the alerting (0.06 SD, p = .68) and orienting attention measures (0.13 SD, p = .42). Visual search time and error rate, and executive attention, collectively, accounted for 14% explained variance in full scale IQ (R(2) = .14, p < .001). DISCUSSION: VP/VLBW children were characterized by less efficient visual search ability and reduced executive attention. Visual attention dysfunctions contributed to intelligence, suggesting the opportunity to improve intellectual functioning by using interventions programs that may enhance attention capacities.

The crucial role of the predictability of motor response in visuomotor deficits in very preterm children at school age.

AIM: We aimed to clarify the underpinnings of widespread visuomotor deficits in very preterm children. METHOD: Fifty-eight very preterm children (26 males, 32 females; mean [SD] age 7 y 6 mo [5 mo], gestational age 29.2 wks [1.6]; birthweight 1237 g [336]), recruited from a tertiary level neonatal intensive care unit, and 64 age-matched, comparison children born at term (28 males, 36 females; mean age [SD] 7 y 8 mo [7 mo]) participated. IQ was measured using a short form of the Wechsler Intelligence Scale for Children (3rd edition). A research diagnosis of developmental coordination disorder (DCD) was defined as a score below the 15th centile on the Movement Assessment Battery for Children. Visuomotor performance was assessed using a computerized task, in which children followed a predictable (structured condition) or an unpredictable (non-structured condition) trail on a touch screen using their index finger. RESULTS: Forty-six per cent of the very preterm children had a research diagnosis of DCD, compared with 16% of children born at term (p<0.001, odds ratio 4.69 [95% CI 2.01-10.99]). No group difference in visuomotor performance was present for the structured condition. In the non-structured condition, children born very preterm with and without a research diagnosis of DCD had poorer visuomotor performance than those born at term. INTERPRETATION: The predictability of the required motor response plays a crucial role in visuomotor deficits in very preterm children, regardless of DCD status.

Sustained developmental effects of the infant behavioral assessment and intervention program in very low birth weight infants at 5.5 years corrected age.

OBJECTIVE: To evaluate the effect of the Infant Behavioral Assessment and Intervention Program (IBAIP) in very low birth weight (VLBW) infants on cognitive, neuromotor, and behavioral development at 5.5 years corrected age (CA). STUDY DESIGN: In a randomized controlled trial, 86 VLBW infants received post discharge IBAIP intervention until 6 months CA, and 90 VLBW infants received standard care. At 5.5 years CA, cognitive and motor development, and visual-motor integration were assessed with the Wechsler Preschool and Primary Scale of Intelligence, third Dutch version, the Movement Assessment Battery for Children, second edition, and the Developmental Test of Visual Motor Integration. Neurologic conditions were assessed with the neurologic examination according to Touwen, and behavior with the Strengths and Difficulties Questionnaire. RESULTS: At 5.5 years CA, 69 children in the intervention and 67 children in the control group participated (response rate 77.3%). Verbal and performance IQ-scores<85 occurred significantly less often in the intervention than in the control group (17.9% vs 33.3%, P=.041, and 7.5% vs 21.2%, P=.023, respectively). However, after adjustment for differences, only the OR for performance IQ was significant: 0.24, 95% CI: 0.06-0.95. Adjusted mean scores on Wechsler Preschool and Primary Scale of Intelligence, third version subtasks block design and vocabulary, the Movement Assessment Battery for Children, second edition component aiming and catching, and the Developmental Test of Visual Motor Integration were significantly better in the intervention group. No intervention effect was found on the Strengths and Difficulties Questionnaire. CONCLUSION: The IBAIP leads, 5 years after the early neurobehavioral intervention, to improvements on performance IQ, ball skills, and visual-motor integration at 5.5 years CA.