Cognitive, academic, and behavioral functioning in school-aged children born with esophageal atresia.

PURPOSE: To characterize cognitive, academic, and behavioral functioning in children who underwent neonatal surgical repair of esophageal atresia (OA) and compare outcomes according to clinical characteristics (presence of additional congenital anomalies, longer hospitalization, and prematurity). METHODS: Intellectual, language, attention, and executive functioning were assessed in 71 5-year-olds and 72 8-year-olds born with OA. At 8 years, memory and academic skills were also assessed. Parents rated children's executive functioning and behavior via questionnaires. Outcomes were compared to normative data and within subgroups of the sample. RESULTS: Intellectual functioning varied depending on the assessment tool, with some evidence of lower than expected intellectual development in children with OA. At 5 years, children with OA showed age-appropriate language and self-regulation, but reduced verbal attention. At 8 years, the OA group had lower than expected sustained attention, divided attention, and mathematics but typical memory and literacy. Parents consistently reported increased working memory difficulties. Other executive functioning and behavioral symptoms were transiently observed. Findings did not consistently differ according to clinical characteristics. CONCLUSIONS: Children with OA may be at risk of transient and persisting cognitive difficulties, particularly in attention and working memory. Difficulties were not strongly associated with additional congenital anomalies, longer hospitalization, or prematurity. LEVEL OF EVIDENCE: Level IV.

Neonatal neuroimaging after repair of congenital diaphragmatic hernia and long-term neurodevelopmental outcome.

Objective: Previous outcome reports of congenital diaphragmatic hernia (CDH) have described neuroimaging anomalies and neurodevelopmental impairment. However, the link between imaging and outcome has not been described. We aimed to determine whether routine postoperative neonatal neuroimaging in infants with CDH detects later neurodevelopmental impairment. Methods: In a prospective cohort study within a clinical service in The Royal Children's Hospital Newborn Intensive Care. Cerebral ultrasound was performed in 81 children and MRI in 57 children who subsequently underwent neurodevelopmental follow-up after surgery for CDH. MRI scans were analyzed using a scoring system designed to identify injury, maturation and volume loss. Neurodevelopmental assessment occurred at 2 years (48) and neurocognitive assessment at 5 years (26) and/or 8 years (27). Brain imaging scores corrected for gestational age at scan time were correlated with outcome measures, adjusting for known clinical confounders. Results: Clinically significant findings were identified on MRI of 16 (28%) infants. Mean scores were in the normal range for all domains assessed at each age. Language impairment was seen in 23% at 2 years and verbal intellectual impairment in 25% at 8 years. Mean cognitive scores were lower in 2-year-old children with white matter injury on MRI (p=0.03). Mean motor scores were lower in 2-year-old children with brain immaturity (p=0.01). Associations between MRI and 5-year and 8-year assessments were no longer significant when adjusting for known clinical confounders. Conclusions: Neuroimaging abnormalities were associated with worse neurodevelopment at 2 years, but not with later neurocognitive outcomes, after accounting for clinical risk factors.

Cognition and behaviour in children with congenital abdominal wall defects.

AIM: To characterise neurodevelopment at age two years and cognition and behaviour at age five years in children born with abdominal wall defects (gastroschisis or exomphalos). STUDY DESIGN: Participants were treated as neonates for gastroschisis or exomphalos and invited for routine clinical follow-up at ages two and five years. Thirty-nine two year-olds and 20 five year-olds with gastroschisis and 20 two year-olds and 10 five year-olds with exomphalos returned for age-appropriate assessments of development (two years) and intellectual functioning (IQ), executive function, and behavioural problems. Results were compared with normative data from the tests and published data from local term-born children. RESULTS: For both gastroschisis and exomphalos two year-olds, neurodevelopment was in line with the test normative data, but below the level of local normative data for all domains (effect sizes from -0.4 to -1.4 standard deviations). At five years, children with gastroschisis performed similarly to the normative mean for IQ but had high rates of various executive functioning problems on parent report (18-41% compared with 7% expected from norms). There was also a tendency for increased frequency of internalising problems (33% compared with normative expectation of 16%). Five year-olds with exomphalos also performed similarly to the normative mean for IQ and had low rates of executive and behavioural problems. CONCLUSIONS: Survivors of gastroschisis and exomphalos may be at risk of poor neurodevelopment in toddlerhood, depending on the reference group, and children with gastroschisis may be particularly at risk for executive functioning difficulties despite an IQ within normal limits.

Executive function in adolescents born <1000 g or <28 weeks: a prospective cohort study.

BACKGROUND AND OBJECTIVES: Extremely preterm (EP; <28 weeks) birth and extremely low birth weight (ELBW; <1000 g) are risk factors for poor cognitive outcomes, including in executive function (EF; higher-order cognitive skills necessary for goal-directed, adaptive functioning and important for academic and behavioral-emotional outcomes). We aimed to (1) extend the limited data on EF in EP/ELBW survivors in adolescence compared with normal birth weight controls, and (2) determine changes in EF between ages 8 and 17 years in both groups. METHODS: Two hundred twenty-eight EP/ELBW and 166 control adolescents (mean age, 17 years) from a prospective geographical cohort were assessed with multiple EF tasks, and parent- and self-ratings of behavioral EF. The Rey Complex Figure and Behavior Rating Inventory of Executive Function parent report were also administered at age 8 years, enabling examination of change in scores between childhood and adolescence. RESULTS: EP/ELBW adolescents performed more poorly than controls in verbal processing speed, attentional control, cognitive flexibility, and goal-setting (effect sizes, -0.7 to -0.2 SD), but not psychomotor reaction time. Group differences were of similar magnitude across tasks. From childhood to late adolescence, EP/ELBW children improved their accuracy of the Rey Complex Figure copy more than controls. According to parents, executive behaviors were largely stable over time in both groups. CONCLUSIONS: Adolescents born EP/ELBW have poorer EF skills across multiple domains than controls. From childhood to late adolescence, different aspects of EF improved, but others did not, underscoring the need for multidomain, longitudinal assessments in this high-risk population.

Visuomotor function in children treated for acute lymphoblastic leukaemia with chemotherapy only.

This study aimed to evaluate visuomotor function in children treated for acute lymphoblastic leukaemia (ALL). The performance of 64 children, 1-7 years post-chemotherapy for ALL, was compared to that of their healthy peers (n = 56) on visuomotor integration (VMI) and motor coordination (MC) tasks. Children posttreatment for ALL displayed significantly reduced VMI, but not MC, performances as compared to controls. Children treated on chemotherapy-only ALL regimes are at heightened risk for visuomotor integration deficits. Monitoring of visuomotor skills and implementation of appropriate interventions targeting higher level visuomotor integration skills should form an important component of any ALL long-term effects program.

School-age outcomes of extremely preterm or extremely low birth weight children.

OBJECTIVE: Research is required to monitor changes in the nature of neurobehavioral deficits in extremely preterm (EP) or extremely low birth weight (ELBW) survivors. This study examines cognitive, academic, and behavioral outcomes at age 8 years for a regional cohort of EP/ELBW children born in 1997. METHODS: The EP/ELBW cohort comprised all live births with a gestational age <28 weeks (EP) or birth weight <1000 g (ELBW) born in the state of Victoria, Australia, in 1997. Of 317 live births, 201 (63.4%) survived to 2 years of age.A term/normal birth weight (T/NBW) cohort was recruited comprising 199 infants with birthweights ≥2500 g or gestational age ≥37 weeks [corrected]. Measures of intellectual ability, educational achievement, and behavior were administered at age 8. RESULTS: Retention was 94% for the EP/ELBW group and 87% for the T/NBW group. The EP/ELBW group performed poorer than the T/NBW group on measures of IQ, educational achievement, and certain behavioral domains, even after adjustment for sociodemographic factors and exclusion of children with neurosensory impairment. The rate of any neurobehavioral impairment was elevated in the EP/ELBW group (71% vs 42%), and one-half of subjects had multiple impairments. The outcomes for those with <750 g birth weight or <26 weeks' gestational age were similar to those with a birth weight of 750 to 999 g or a gestational age of 26 to 27 weeks, respectively. CONCLUSIONS: Despite ongoing improvements in the management of EP/ELBW infants, the rate of neurobehavioral impairment at school-age remains too high relative to controls.

A comparison of working memory profiles in school-aged children with Specific Language Impairment, Attention Deficit/Hyperactivity Disorder, Comorbid SLI and ADHD and their typically developing peers.

The association between specific language impairment (SLI), attention deficit/hyperactivity disorder (ADHD), and working memory (WM) was examined. WM has been implicated in language acquisition and models of ADHD; however, evidence for WM deficits in SLI and ADHD has been inconsistent. The components of Baddeley's WM model were investigated in 18 children with SLI, 16 children with ADHD, 11 children with comorbid SLI+ADHD, and 24 typically developing (TD) children. The presence of SLI resulted in deficits in more components of WM than the presence of ADHD indicating that children with SLI are more vulnerable to WM deficits than those with ADHD.

Attention problems in a representative sample of extremely preterm/extremely low birth weight children.

The aim of this study was to examine attention in a large, representative, contemporary cohort of children born extremely preterm (EP) and/or extremely low birth weight (ELBW). Participants included 189 of 201 surviving children born EP (<28 weeks' gestation) or ELBW (<1,000 g) in 1997 in the state of Victoria, Australia. A comparison group of 173 of 199 children born full term and normal birth weight (FT/NBW) were randomly selected matching for birth hospital, expected due date, gender, mother's country of birth, and health insurance status. Participants were assessed at 8 years of age on subtests from the Test of Everyday Attention for Children (TEA-Ch) and the Wechsler Intelligence Scale for Children-4th Edition (WISC-IV). Measures of selective attention, sustained attention, attention encoding, and executive attention (inhibition, shifting attention, and divided attention) were administered. To assess behavioral elements of inattention, the primary caregiver completed the Behavior Rating Inventory of Executive Function (BRIEF) and the Conners' ADHD/DSM-IV Scale (CADS-P). The EP/ELBW group performed more poorly across all cognitive and behavioral measures than the FT/NBW group, with the exception of inhibition. The EP/ELBW group also had significantly elevated rates of impairment in selective, sustained, shifting and divided attention, as well as attention deficit hyperactivity disorder (ADHD) symptoms. No significant gender or gradient effects (e.g., <26 weeks' gestation vs. ≥ 26 weeks' gestation) were identified. Neonatal medical factors were not strong predictors of attention, although necrotizing enterocolitis (NEC) and cystic periventricular leukomalacia (PVL) were independent predictors of selective attention. In conclusion, our comprehensive assessment of attention provides strong evidence that children born EP/ELBW are at increased risk for attentional impairments, and as such, this population should be monitored closely during early and middle childhood with a focus on attention functioning.

Underestimation of developmental delay by the new Bayley-III Scale.

OBJECTIVE: To assess the ability of the third edition of the Bayley Scales of Infant and Toddler Development (Bayley-III) to detect developmental delay in 2-year-old children who were extremely preterm and those carried to term. DESIGN: Prospective cohort study. SETTING: The state of Victoria, Australia. PARTICIPANTS: Subjects were consecutive surviving children who were born either at less than 28 weeks' gestational age (extremely preterm) or with less than 1000 g birth weight (extremely low-birth-weight; n = 221) in the state of Victoria, Australia, in 2005 and randomly selected controls who were both carried to term and of normal birth weight (n = 220). MAIN OUTCOME MEASURE: Children were assessed by psychologists blinded to knowledge of group at 2 years of age, corrected for prematurity with the new Bayley-III scale. RESULTS: Follow-up rates of both cohorts were high (>92%). Mean values for all composite and subtest scores for the extremely preterm/extremely low-birth-weight group were significantly below those of the control group (P < .001), with the magnitude of all group differences being in excess of two-thirds SD. Mean values for the extremely preterm/extremely low-birth-weight group approached the normative mean, but in contrast, the mean values for the control group were higher than expected, with composite scores being between 0.55 and 1.23 SD above the normative mean. Proportions of children with developmental delay were grossly underestimated using the reference values, but were within the expected range when computed relative to the mean (standard deviation) for the controls. CONCLUSION: The Bayley-III scale seriously underestimates developmental delay in 2-year-old Australian children.