Visual Function Classification System for children with cerebral palsy: development and validation.

AIM: To develop and validate the Visual Function Classification System (VFCS), which was created to classify how children with cerebral palsy (CP) use visual abilities in daily life. METHOD: The process of development and validation of the VFCS involved four phases: (1) drafting of the five levels from the analysis of literature and clinical experience; (2) validation of constructs and revision of the levels for concept meaningfulness, using nominal group process; (3) refinement by international Delphi survey; and(4) assessment of interrater reliability among professionals and with caregivers, and of test-retest reliability. RESULTS: Five nominal groups involved 29 participants; 65 people completed the first round and 51 the second round of the Delphi survey. Construct validity was demonstrated within an expert group and external validation through several stakeholders, with the involvement of patients and families to ensure meaningfulness of the concept. Discussions continued until consensus was reached about the construct and content of the five levels. Participants in the reliability study included 29 professionals, 39 parents, and a total sample of 160 children with CP (mean age [SD] 6y 6mo [3y 4mo]; median 5y 7mo, range 1-19y). Absolute interrater agreement among professionals was 86% (weighted κ=0.88; 95% confidence interval [CI] 0.83-0.93). Test-retest reliability was high (weighted κ=0.97; 95% CI 0.95-0.99). Parent-professional interrater reliability on 39 children was moderate (weighted κ=0.51; 95% CI 0.39-0.63). INTERPRETATION: The VFCS has been appropriately constructed and provides a reliable system to classify visual abilities of children with CP both in clinical and in research settings. WHAT THIS PAPER ADDS: The Visual Function Classification System is a valid and reliable system. It classifies visual abilities of children with cerebral palsy in clinical and research settings. At a clinical level, it can be used to harmonize communication among professionals and identify patients' strengths and weaknesses. In research settings, it can be used to stratify patients, define natural history evolution, and interpret intervention studies.

SISTEMA DE CLASIFICACIÓN DE LA FUNCIÓN VISUAL PARA NIÑOS CON PARÁLISIS CEREBRAL: DESARROLLO Y VALIDACIÓN: OBJETIVO: Desarrollar y validar el Sistema de Clasificación de la Función Visual (VFCS, siglas en inglés), que fue creado para clasificar cómo los niños con parálisis cerebral (PC) usan las habilidades visuales en la vida diaria. MÉTODO: El proceso de desarrollo y validación del VFCS involucró cuatro fases: (1) elaboración de los cinco niveles a partir del análisis de la literatura y la experiencia clínica; (2) la validación de constructos y la revisión de los niveles para el significado de los conceptos, utilizando un proceso de grupo nominal; (3) refinamiento por encuesta internacional de Delphi; (4) evaluación de la confiabilidad entre evaluadores entre profesionales y con los cuidadores, y de confiabilidad de prueba y reevaluación RESULTADOS: Cinco grupos nominales incluyeron 29 participantes; 65 personas completaron la primera ronda y 51 la segunda ronda de la encuesta de Delphi. La validez de constructo se demostró dentro de un grupo de expertos y una validación externa a través de varias partes interesadas, con la participación de los pacientes y las familias para garantizar el significado del concepto. Las discusiones continuaron hasta que se llegó a un consenso sobre el constructo y el contenido de los cinco niveles. Los participantes en el estudio de confiabilidad incluyeron 29 profesionales, 39 padres y una muestra total de 160 niños con PC (edad media [DS] 6 años 6 meses [3 años 4 meses]; mediana 5 años 7 meses, rango 1-19 años). El acuerdo de evaluador absoluto entre profesionales fue del 86% (κ ponderada = 0,88; intervalo de confianza del 95% [IC] 0,83-0,93). La fiabilidad de Test-Retest fue alta (κ ponderada = 0,97; IC del 95%: 0,95 a 0,99). La confiabilidad entre los padres y profesionales entre 39 niños fue moderada (ponderada κ = 0,51; IC del 95%: 0,39 a 0,63). INTERPRETACIÓN: El VFCS se ha construido de manera adecuada y proporciona un sistema confiable para clasificar las habilidades visuales de los niños con PC, tanto en el ámbito clínico como en el de investigación.

SISTEMA DE CLASSIFICAÇÃO DA FUNÇÃO VISUAL PARA CRIANÇAS COM PARALISIA CEREBRAL: DESENVOLVIMENTO E VALIDAÇÃO: OBJETIVO: Desenvolver e validar o Sistema de Classificação da Função Visual (SCFV), que foi criado para classificar como crianças com paralisia cerebral (PC) usam capacidades visuais na vida diária. MÉTODO: O processo de desenvolvimento e validação do SCFV envolve quatro fases: (1) rascunho dos cinco níveis a partir da análise da literatura e experiência clínica; (2) validação de construtos e revisão dos níveis de significância dos conceitos, usando processo nominal de grupos; (3) refinamento por meio de levantamento Delphi internacional; (4) avaliação da confiabilidade inter-examinadores entre profissionais e cuidadores, e confiabilidade teste-reteste. RESULTADOS: Cinco grupos nominais envolveram 29 participantes; 65 pessoas completaram a primeira rodada e 51 a segunda rodada do levantamento Delphi. A validade de constructo foi demonstrada em um grupo de especialistas, e a validade externa por meio de vários interessados, com envolvimento de pacientes e famílias para assegurar a significância do conceito. As discussões continuaram até que fosse atingido consenso sobre o constructo e o conteúdo dos cinco níveis. Os participantes no estudo de confiabilidade incluíram 29 profissionais, 39 pais e uma amostra total de 160 crianças com PC (média de idade [DP] 6a 6m [3a 4m]; mediana 5a 7m, variação 1-19a). A confiabilidade inter-examinadores absoluta entre profissionais foi 86% (κ ponderado=0,88; intervalo de confiança [IC] a 95% 0,83-0,93). A confiabilidade teste-reteste foi alta (κ ponderado =0,97; IC 95% 0,95-0,99). A confiabilidade inter-examinadores pais-profissionais em 39 crianças foi moderada (κ ponderado =0,51; IC 95% 0,39-0,63). INTERPRETAÇÃO: O SCFV foi elaborado apropriadamente e é um sistema confiável para classificar as capacidades visuais de crianças com PC em ambientes clínicos e acadêmicos.

Neurological assessment in infants discharged from a neonatal intensive care unit.

BACKGROUND: Longitudinal motor assessment in infants at different neurodevelopmental risk has not been previously evaluated using structured assessments. AIM: To verify if the Hammersmith Infant Neurological Examination (HINE) is a good tool to predict the neuromotor outcome in infants discharged from a level II-III Neonatal Intensive Care Unit (NICU) METHODS: In this cohort analysis, 1541 infants discharged from our NICU between January of 2002 and the April 2006 were enrolled and assessed using the HINE at 3, 6, 9, 12 months. At two years, these infants were further assessed, and grouped into infants with normal outcome (1150), with mild disability (321) and with cerebral palsy (70), RESULTS: Correlation analysis of Spearman showed a significant (p < 0.0001) and moderate (r(2) = -0.55 to -0.73) negative correlation between HINE scores (3, 6, 9, 12 months) and neurological outcome at two years. Cut-off scores for each assessment' age were provided as predictive value for cerebral palsy. DISCUSSION: This study mainly showed that HINE, as soon as the first months of life, helps in the process of prediction of neurological outcome at two years of age in a heterogeneous population of infants discharged from an NICU.

Spectrum of gross motor and cognitive functions in children with cerebral palsy: gender differences.

BACKGROUND: Multiple differences between males and females are reported both in physiological and pathophysiological conditions. AIMS: To test the hypothesis that gender could influence the motor and cognitive development in children with cerebral palsy (CP). METHODS: Prospective, cross-sectional. One hundred seventy one children with CP (98 males and 73 females) were evaluated for motor (Gross Motor Function Measure, Gross Motor Function Classification System) and cognitive (Bayley II, Wechsler Scales) functions. Eighty-four of them were assessed before and other eighty-seven children after 4 years of age. RESULTS: No gender-related differences were observed in children with diplegia or quadriplegia, both for motor and cognitive functions. On the contrary, females with hemiplegia scored significantly better (P < 0.01) in cognitive functions and in the dimension D (standing) of the Gross Motor Function Measure, under the age of 4 years. These differences were not observed after this age. CONCLUSIONS: In this study we point out that gender might influence differently the psycho-motor development of children with hemiplegia and of those with a more severe clinical involvement as diplegia and quadriplegia.

Prognostic value of a scorable neurological examination from 3 to 12 months post-term age in very preterm infants: a longitudinal study.

UNLABELLED: AIMS AND STUDY DESIGN: The Hammersmith Infant Neurological Examination proved effective in predicting locomotor function in very preterm infants after 9 months of age. We performed the examination in a cohort of 103 very preterm infants (gestational age below 32 weeks) as early as 3 months' post-term age, and longitudinally at 6, 9 and 12 months. Our aim was to establish the frequency distribution of the optimality scores at each age period, to explore the predictive value of the examination from 3 months onwards as to developmental outcome and locomotor function at 2 years, and to explore its longitudinal consistency. RESULTS: The results showed that this standardized neurological examination can be performed in preterm infants as early as 3 months' post-term age to predict motor outcome at 2 years, and that its high predictive value is consistent across the first year of life due to an effective combination of different items for each age period. CONCLUSIONS: We confirm the high predictive value of this neurological examination in very preterm infants after 9 months and extend it to the assessments performed as early as 3 months post-term. This is of great relevance as in very preterm infants early prediction of motor function is essential for a prompt planning of therapeutic interventions.

Development of the forward parachute reaction and the age of walking in near term infants: a longitudinal observational study.

BACKGROUND: Near term infants are a main part of preterms. They are at higher risk for mortality and morbidity than term infants and could show a quite different development of tone and reflexes from them. The aim of the present study was to describe longitudinally, in a large sample of healthy near term infants, the development of the forward parachute reaction (FPR) and its correlation with the age of acquisition of independent walking. METHODS: The assessment of FPR (as absent, incomplete or complete) was performed at 3, 6, 9, 12 months of corrected age in 484 infants, with a gestational age between 35.0 and 36.9 weeks. The age of acquisition of independent walking was monitored until its appearance. A correlation analysis was done between the age of walking and the acquisition of a complete or incomplete FPR, using the Spearman Rank correlation. The Mann-Withney U test was used to identify significant gestational age differences for the age of FPR appearance. RESULTS: Most of infants had a two-step development pattern. In fact, they showed at first an incomplete and then a complete FPR, which was observed more frequently at 9 months. An incomplete FPR only, without a successive maturation to a complete FPR, was present in the 21% of the whole sample. Infants with a complete FPR walked at a median age of 13 months, whereas those with an incomplete FPR only walked at a median age of 14 months. CONCLUSION: We identified two groups within our sample of near term infants. The first group showed a progressive maturation of FPR, whereas the second one was characterised by the inability to get a complete pattern, within the one year observation's period. Furthermore, we observed a trend toward a delayed acquisition of independent walking in the latter group of infants.

Visual function at 35 and 40 weeks' postmenstrual age in low-risk preterm infants.

OBJECTIVES: The objectives of this study were to (1) assess visual function in low-risk preterm infants at 35 and 40 weeks' postmenstrual age, (2) compare preterm visual abilities at term-equivalent age with term-born infants, and (3) evaluate effects of preterm extrauterine life on early visual function. METHODS: Visual function was assessed by using a validated test battery at 35 and 40 weeks' postmenstrual age in 109 low-risk preterm infants who were born at <31 weeks' gestation. The preterm findings were compared with data from term-born infants collected by using the same test protocol. RESULTS: All preterm infants completed both assessments. The 35-week responses were generally less mature than those at 40 weeks. Preterm infants at both ages were significantly more mature than term-born infants for ocular movements and vertical and arc tracking and at 40 weeks for stripe discrimination. In contrast, tracking a colored stimulus, attention at distance, and stripe discrimination were more mature at term age (in both term-born and preterm infants) than at 35 weeks. CONCLUSIONS: Our findings provide data for visual function at 35 and 40 weeks' postmenstrual age in low-risk preterm infants. The results suggest that early extrauterine experience may accelerate the maturation of aspects of visual function related to ocular stability and tracking but does not seem to affect other aspects that may be more cortically mediated.

Neurological examination of preterm infants at term equivalent age.

BACKGROUND: We previously reported the neurological findings of the Dubowitz neonatal examination in a cohort of 157 low-risk preterms born between 25 and 33 weeks gestational age (GA) and examined at term equivalent age (TEA). Median and range of scores were wider than those found in term-born infants and preterms showed a different neurological behaviour in specific items. However, the cohort number was too small to draw any definitive conclusion about the distribution of findings. AIMS: We provide normative data from a low-risk cohort of 380 preterm infants; we also assess the findings and their relationship to motor outcome in preterms with major cranial ultrasound (US) abnormality. STUDY DESIGN: We assessed, at TEA, 380 low-risk preterms born <35 weeks gestation (range 25-34.9, median 29) with normal 2 year motor outcome and 85 preterm infants with major US abnormality. RESULTS: At TEA low-risk preterms had less flexor limb tone, poorer head control but better visual following than term-born infants. For 28/34 of the neurological items the range and median scores were similar across gestational ages. In infants with major US lesions the range and median scores differed from low-risk preterms in 20/34 items; 40% of infants developing a diplegia and 80% developing a tetraplegia had >7 items outside the 90th centile; all infants with >12 items outside the 90th centile developed a tetraplegia. CONCLUSIONS: We provide reference values for the neurological examination of low-risk preterms at TEA. In infants with major US abnormality the number of items outside the 90th centile was an indicator of outcome severity.

Neuromotor development in infants with cerebral palsy investigated by the Hammersmith Infant Neurological Examination during the first year of age.

BACKGROUND: The Hammersmith Infant Neurological Examination (HINE) is a simple and scorable method for assessing infants between 2 and 24 months of age. AIMS: The purpose of this retrospective study was firstly, to evaluate the neuromotor development of infants with cerebral palsy (CP) by the HINE, during the first year of age; secondly, to correlate the scoring of this neurological tool with levels of the Gross Motor Function Classification System (GMFCS). METHODS: A cohort of 70 infants with a diagnosis of CP at 2 years of age was evaluated by the HINE at 3, 6, 9 and 12 months of corrected age and by GMFCS at 2 years of age. RESULTS: The main results indicate that at 3-6 months, infants with quadriplegia (IV and V levels of GMFCS) and those with severe diplegia (III level) scored below 40, whereas those with mild or moderate diplegia (I-II level) and hemiplegia (I-II level) mainly scored between 40-60. Interestingly, the 26% of infants with hemiplegia scored > or =67 at 12 months. We observed a strong (r=-0.82) and significant (p<0.0001) negative correlation between the scores of the neurological examination and the levels of GMFCS. CONCLUSIONS: Our results point out that the HINE can give additional information about neuromotor development of infants with CP from 3-6 months of age, strictly related to the gross motor functional abilities at 2 years of age.

Application of a neonatal assessment of visual function in a population of low risk full-term newborn.

BACKGROUND: We have previously developed and described a battery of 9 items suitable for assessing different clinical aspects of visual function in newborn infants. AIM OF THE STUDY: Application of the test battery to a cohort of low risk term-born infants at 48 and 72 h after birth 1) to define the normative distribution of results for each item and 2) to document any effect of postnatal age. STUDY DESIGN AND SUBJECTS: 124 term-born low risk infants were assessed at 48 h; fifty of them were re-assessed 24 h later at 72 h. RESULTS: The visual test battery was successfully completed in 110 of the 124 infants assessed at 48 h and in all the 50 infants assessed at 72 h after birth. For 3 of the 9 items (fixation on a black/white target of concentric circles, on a coloured (red/yellow) face and horizontal tracking), the findings were very similar at both ages. For the remaining 6 items the range of findings was wider. There was a statistical difference in the responses obtained at 48 and 72 h for vertical and arc tracking (p<0.05) and the ability to discriminate stripes and attention at distance (p<0.001). CONCLUSION: Our results provide information on the visual abilities in a low risk population of term-born infants and the distribution of frequency of their visual responses to our battery of visual tests. These findings may be used as reference data when using our visual test battery in both clinical and research settings.

The development of vision.

Disorders of visual function are common findings in children with neonatal brain lesions of antenatal and perinatal onset. In the last few years the development of age appropriate batteries for assessing visual function in the first years and the combined use of neuroimaging and neurophysiological techniques have allowed to achieve better understanding of the mechanisms underlying development of vision in low risk infants and in those with brain lesions. We will review the main models of visual development and the tests available to assess visual function in infancy, focusing on the recently described battery of tests for assessing early visual abilities in preterm and full term infants.