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Aim: To develop a data‐driven subgrouping method to identify and profile subtypes of early‐onset childhood cerebral visual impairment (CVI). / Method: Sixty‐three children with suspected or diagnosed congenital CVI were recruited (28 males, 35 females, median age=8y, range=5–16y). Cognitive, basic, and higher‐order vision functions were assessed and quality of life, functional vision questionnaire, neurodevelopmental, and ophthalmological data were collected. Cluster analysis and other statistical analyses were undertaken to determine and validate the subgrouping. / Results: Forty‐three participants completing the full test battery were included in cluster analysis, revealing two subgroups. Group A1 (n=15) showed selective visual perception and visuomotor deficits. Group A2 (n=28) showed more severe and broader visual perception and visuomotor deficits, and variable visual acuity. A third, lower‐functioning group, Group B (n=20), was differentiated and showed significant visual acuity reduction compared with Group A (p

BACKGROUND This study investigated mother-infant interactions, including maternal maintaining of infant attentional focus and sensitivity, with infants with congenital severe and profound visual impairment (VI) and the association with developmental trajectories from one to three years. METHOD Fifty-five infants and mothers were video-recorded playing together with a standard set of toys at Time 1 (T1) mean age 12.95 months (8.13-17.05 months). Maintain was categorized as the mother following and maintaining the child's focus, and Sensitivity, the mother's responsiveness and contingency to infant behaviour. Vision level was measured using the Near Detection Scale. Cognition and language were measured at T1, 12 months later (T2) and 24 months later (T3) using the Reynell-Zinkin Scales. RESULTS Cross-sectional analyses showed that mothers of infants with severe VI (basic form vision) produced higher rates of Maintain compared to those with children with profound VI (light perception at best). Linear mixed-effects models examining developmental progression from T1 to T3 (controlling for vision level) showed an average increase of 5 DQ points (CI 95%: 1.03-9.08) in verbal comprehension for higher Sensitivity. No significant findings were found for Maintain. CONCLUSIONS The findings suggest that mother-infant interactions (maternal Maintain) are associated with level of vision at infancy, but only maternal Sensitivity has a long-term positive association with advances in verbal comprehension from infancy to about three years. They highlight the need for incorporating strategies related to parent-infant interactions, including increased sensitivity, into early intervention for children with visual impairment.

AIM This study examined cross-sectional and longitudinal patterns of parenting stress, adult anxiety, and depression in mothers of children with profound or severe visual impairment (PVI or SVI) at 1 year and 2 years of age. METHOD Mothers of a national longitudinal cohort (OPTIMUM Project) of infants with congenital disorders of the peripheral visual system and PVI (light perception at best) or SVI (basic 'form' vision of non-light reflecting objects) participated. Infant age at baseline (T1 ) was 8 to 16 months. Mothers completed the Parenting Stress Index - Short Form and the Hospital Anxiety and Depression Scale at T1 (n=79) and at follow-up 12 months later (T2 ) (n=73). RESULTS Mothers of the total group had higher parenting stress levels (34.6% in clinical range) than community normative data at T1 (p=0.017). Mothers of infants in the PVI subgroup had elevated stress at T1 (p=0.014) and T2 (p=0.009). The PVI subgroup was also elevated in the Difficult Child subscale at T2 (p=0.001). Within-sample differences in parenting stress between the visual impairment subgroups were found at T2 only: the PVI subgroup scored higher than the SVI subgroup (p=0.029). Adult anxiety and depression in the total group were not elevated compared with community normative data at T1 and T2 ; however, higher parenting stress was related to raised adult anxiety and depression levels at T1 and T2 (p=0.001). Regression analysis found parenting stress and lower child vision level (T1 ) predicted parenting stress (T2 ) (p=0.001; 42% variance). INTERPRETATION Mothers of 1-year-old infants with visual impairment showed raised risk for parenting stress, which continued to be elevated for children with PVI and those perceived as 'difficult' at 2 years. This was also a psychological risk, with greater adult anxiety and depression in those mothers with raised parenting stress. The clinical significance is that identification of parenting stress and targeted parenting, and behavioural support of the child in the first years of life is highly indicated. WHAT THIS PAPER ADDS Mothers of infants with visual impairment are at increased risk of parenting stress. Parenting stress was higher in mothers of children with profound visual impairment than those with severe visual impairment. High levels of parenting stress and lower infant vision at 1 year of age predicted higher parenting stress at 2 years of age.

Objective Young children with congenital visual impairment (VI) are at increased risk of behavioral vulnerabilities. Studies on ‘at risk’ populations suggest that frontal EEG asymmetry may be associated with behavioral risk. We investigated frontal asymmetry at 1 year (Time 1), behavior at 2 years (Time 2) and their longitudinal associations within a sample of infants with VI. Frontal asymmetry in the VI sample at 1 year was also compared cross-sectionally to an age-matched typically sighted (TS) group. Methods At Time 1, 22 infants with VI and 10 TS infants underwent 128-channel EEG recording. Frontal asymmetry ratios were calculated from power spectral density values in the alpha frequency band. At Time 2, Achenbach Child Behavior Checklist data was obtained for the VI sample. Results 63.6% of the VI sample and 50% of the TS sample showed left frontal asymmetry; no significant difference in frontal asymmetry was found between the two groups. 22.7% of the VI sample had subclinical to clinical range ‘internalizing’ behavior difficulties. Greater left frontal asymmetry at one year was significantly associated with greater emotionally reactive scores at two years within the VI sample ( r = 0.50, p = 0.02). Conclusions Left frontal asymmetry correlates with later behavior risk within this vulnerable population. Significance These findings make an important first contribution regarding the utility of frontal EEG asymmetry as a method to investigate risk in infants with VI.

To investigate the effects of home-based early intervention in children with severe visual impairment (SVI) using the Developmental Journal for babies and young children with visual impairment (DJVI).A longitudinal observational study was undertaken with a national cohort (OPTIMUM) of infants with congenital disorders of the peripheral visual system (CDPVS) and profound-SVI; and followed up after 12 months and 24 months. Intervention was categorized according to the practitioner diary records of their usual practice over 12 months from baseline comparing those receiving the DJVI and those receiving 'Other Support'. Outcome measures of cognition and language, behaviour difficulties, parenting stress, and satisfaction with parent-practitioner partnership were collected.In the 54 children (26 males, 28 females, baseline mean age 13.5mo, SD 2.3mo, range 8-17mo) with 'total' CDPVS (including 16 'complex' and 38 'simple' with or without known brain disorder respectively), linear mixed effects pointed towards acceleration in sensorimotor understanding and expressive language especially in the 'simple' subsample (11.72 developmental quotient, 95% confidence interval -1.17 to 24.61, p0.05) in those receiving the DJVI. Vision level also predicted outcomes (p0.05). The DJVI group showed improvements in behavioural withdrawal (ηInfants and young children with visual impairment receiving home-based early intervention using the DJVI with a structured developmental approach had better outcomes than those receiving 'other' home-based early interventions. Moderate to large effect improvements were found in child cognition and language, behaviour and parenting stress and the perceived practitioner-parent relationship, although cognition did not reach 5% significance level.Early intervention using the Developmental Journal for babies and young children with visual impairment was associated with enhanced developmental outcomes compared to other approaches. Improvements were also found in child behaviour, parenting stress, and perceived parent practitioner outcomes. Type and complexity of visual impairment also influenced outcomes.INTERVENCIÓN TEMPRANA DOMICILIARIA EN LACTANTES Y NIÑOS PEQUEÑOS CON DISCAPACIDAD VISUAL USANDO EL DIARIO DE DESARROLLO: ESTUDIO LONGITUDINAL DE COHORTE: OBJETIVO: Investigar los efectos de la intervención temprana en el hogar en niños con discapacidad visual grave utilizando el Diario de Desarrollo para bebés y niños pequeños con discapacidad visual (DJVI). MÉTODO: Se realizó un estudio observacional longitudinal con una cohorte nacional (OPTIMUM) de bebés con trastornos congénitos del sistema visual periférico (CDPVS) y discapacidad visual profunda-severa; y seguimiento después de 12 meses y 24 meses. La intervención se categorizó de acuerdo con los registros del diario de desarrollo del médico en su práctica habitual más de 12 meses desde el inicio, comparando los que recibieron el DJVI y los que recibieron “otro apoyo”. Se recopilaron las medidas de resultado de la cognición y el lenguaje, las dificultades de comportamiento, el estrés de los padres y la satisfacción con la asociación entre padres y profesionales. RESULTADOS: En los 54 niños (26 varones, 28 mujeres, edad media de referencia 13,5 meses, DS 2,3 meses, rango 8-17 meses) con CDPVS ‘total’ (incluidos 16 ‘complejo’ y 38 ‘simple’ con o sin trastorno cerebral conocido respectivamente), los efectos mixtos lineales apuntan hacia la aceleración en la comprensión sensoriomotora y el lenguaje expresivo, especialmente en la submuestra “simple” (cociente de desarrollo 11,72, intervalo de confianza del 95% -1,17 a 24,61, p0,05) en los que recibieron el DJVI. El nivel de visión también predijo resultados (p0,05). El grupo DJVI mostró mejoras en la abstinencia conductual (ηINTERVENÇÃO PRECOCE DOMICILIAR EM LACTENTES E CRIANÇAS JOVENS COM DEFICIÊNCIA VISUAL USANDO O DEVELOPMENTAL JOURNAL: ESTUDO DE COORTE LONGITUDINAL: OBJETIVO: Investigar os efeitos da intervenção precoce domiciliar em crianças com deficiência visual severa usando o Developmental Journal para lactentes e crianças jovens com deficiência visual (DJDV). METODO: Um estudo observacional longitudinal foi realizado com uma coorte nacional (OPTIMUM) de crianças com distúrbios congênitos do sistema visual periférico (DCSVP) e deficiência visual grave-profunda, estes foram acompanhados após 12 meses e 24 meses. A intervenção foi categorizada de acordo com os registros diários do profissional de sua prática habitual ao longo de 12 meses, a partir de uma linha de base, comparando aqueles que receberam a DJDV e os que receberam “outro suporte”. Resultados dos testes de cognição e linguagem, dificuldades de comportamento, estresse parental e satisfação com a parceria entre pais e profissionais, foram coletados. RESULTADOS: Nas 54 crianças (26 do sexo masculino e 28 do feminino, média de idade na linha de base de 13,5 meses; DP 2,3 meses; variação de 8 a 17 meses) com DCSVP total (incluindo 16 ‘complexos’ e 38 ‘simples’ com ou sem distúrbio cerebral conhecido, respectivamente), efeitos mistos lineares apontaram para um avanço na compreensão sensório-motora e de linguagem expressiva, especialmente, no subgrupo ‘simples’ (11,72 quociente de desenvolvimento, IC 95% -1,17 a 24,61; p0,05) naqueles que receberam o DJDV. Nível visual também foi preditivo dos desfechos (p0,05). O grupo DJDV apresentou melhora no comportamento de retraimento social (η

The childhood condition of visual difficulties caused by brain damage, commonly termed cortical or cerebral visual impairment (CVI), is well established but has no internationally accepted definition. Clarification of its core features is required to advance research and clinical practice. This systematic review aimed to identify the definitions of childhood CVI in the original scientific literature to describe and critically appraise a consensual definition of the condition. MEDLINE, EMBASE, PsychINFO, CINAHL and AMED databases were searched in January 2017. Studies were included if they (1) were published original research, (2) contained a childhood CVI sample, (3) contained a definition of CVI and (4) described their CVI identification/diagnostic method. Thematic analysis identified concepts within definitions and narrative synthesis was conducted. Of 1150 articles, 51 met inclusion criteria. Definitions were subdivided according to detail (descriptive definition, description not reaching definition status and diagnostic/operationalising criteria). Three themes concerning visual deficits, eye health and brain integrity were identified (each containing subthemes) and analysed individually across definitions. The most common themes were ‘visual impairment’ (n=20), ‘retrochiasmatic pathway damage’(n=13) and ‘normal/near normal eye health’ (n=15). The most consensual definition identified here may not be the best quality for advancing our understanding of CVI. We argue for the alternative definition: CVI is a verifiable visual dysfunction which cannot be attributed to disorders of the anterior visual pathways or any potentially co-occurring ocular impairment. We propose reporting guidelines to permit comparison across studies and increase the evidence base for more reliable clinical assessment and diagnosis.