Background: Children undergoing open heart surgery are at risk of neurological injury. A cohort of 35 patients, who had undergone cardiac surgery during infancy, had a significant reduction in Bayley Scale of Infant Development scores at a 12-month assessment. This cohort has now reached an appropriate age to reassess developmental progress. Methods: Detailed psychometric testing was conducted on 20 children from the original cohort using the Weschler Preschool and Primary Scale of Intelligence, the Wide Range Assessment of Memory and Learning, and the Wechsler Individual Achievement Test. Parents completed the Connor’s Rating Scale, the Behaviour Rating Scale of Executive Functioning, and the Child Behaviour Checklist. Results: The mean age of the cohort at assessment was 6.6 (standard deviation 0.4) years. Mean scores on all tests of intelligence, memory, academic achievement, and executive function fell within the average range. Of the children, 20–35% were found to have significant difficulties across these areas. Mean scores in the areas of social, emotional, behavioural, and psychological functioning also fell within the average range. Of the children studied, 35% had clinically significant problems in these areas. There was only a weak association between the 12-month scores and the Full-Scale Intelligence Quotient at 6 years. Conclusion: Detailed psychometric testing of these children suggests that they generally function in the average range; however, a significant proportion falls below age expectations in all the areas assessed. This highlights the importance of long-term follow-up with routine developmental screening to allow identification of a subgroup that may benefit from early educational and behavioural intervention.

Core social interaction behaviors were examined in young children 0–36 months of age who were hospitalized for accidental (n = 61) or inflicted (n = 64) traumatic brain injury (TBI) in comparison to typically developing children (n = 60). Responding to and initiating gaze and joint attention (JA) were evaluated during a semi-structured sequence of social interactions between the child and an examiner at 2 and 12 months after injury. The accidental TBI group established gaze less often and had an initial deficit initiating JA that resolved by the follow-up. Contrary to expectation, children with inflicted TBI did not have lower rates of social engagement than other groups. Responding to JA was more strongly related than initiating JA to measures of injury severity and to later cognitive and social outcomes. Compared to complicated-mild/moderate TBI, severe TBI in young children was associated with less responsiveness in social interactions and less favorable caregiver ratings of communication and social behavior. JA response, family resources, and group interacted to predict outcomes. Children with inflicted TBI who were less socially responsive and had lower levels of family resources had the least favorable outcomes. Low social responsiveness after TBI may be an early marker for later cognitive and adaptive behavior difficulties. (JINS, 2013, 19, 1–11)

We have determined the influence of the severity of retinopathy of prematurity (ROP) on development at 3 years of age in infants <29 weeks gestation from a population-based cohort. Primary analysis of surviving infants born <29 weeks gestational age (GA) from 1998 to 2001 in New South Wales and the Australian Capital Territory were grouped according to stage of ROP. Infants with periventricular leukomalacia, Grade III or IV intraventricular haemorrhage, hydrocephalus, major congenital abnormalities, Stage 4 or 5 ROP, cerebral palsy or a severe hearing impairment were excluded. Infants with Stage 3 ROP were matched for GA, birthweight and gender to those with no ROP, Stage 1 and Stage 2 ROP. The four groups were then compared for their 3-year-old developmental outcome, using the Griffiths Mental Development Scale. Development was also compared for those infants with Stage 3 ROP who were either treated or not treated with laser therapy. A secondary multivariate regression analysis on developmental outcome was performed with all infants included in the analysis. In neurologically comparable groups and in the multivariate analysis, there was no association between ROP and developmental outcome. There was also no difference in the Griffiths assessment at 3 years between those who were or were not treated for severe ROP. Neither severity of ROP nor treatment for severe ROP were related to developmental outcome at 3 years of age in a large population-based cohort of infants born <29 weeks gestation.

Cognitive, functional, educational achievement and behavioural measures were employed to assess neurobehavioral status in 57 of 60 participants who were initially enrolled in the Baltimore–Washington Infant Study, and who survived surgical correction of complete transposition (concordant atrioventricular and discordant ventriculo-arterial connections). Charts were reviewed to investigate the relationship between birth variables, surgical strategy and developmental outcomes. Higher preoperative weight was associated with better outcomes on the Stanford–Biner Short-term Memory subtest, while lower preoperative oxygen tension was associated with better outcomes on the Abstract/Visual Reasoning subtest and a test of Visual–Motor Integration. Longer total bypass time was associated with poor outcomes on the Short-term Memory subtests. Higher average flow rates during cooling and rewarming were associated with higher scores in the test of short term memory but poorer outcomes on a test for visual motor integration. Longer cooling times were associated with higher scores on the test for Visual–Motor Integration. Patients suffering seizures scored lower on the Stanford–Biner Composite, as well as in their tests of achievement. The data indicate that non-verbal. skills may be particularly sensitive to variations in surgical strategies employed to correct complete transposition. Overt neurological events, such as seizures, were related to global deficits in intellectual functioning. Prospective studies evaluating systemic variations in surgical procedures and attempts to prevent and manage perioperative neurological events are important for further investigation of neurodevelopmental outcomes in children surviving surgical correction.

At 1 year we assessed the neurodevelopmental outcomes in infants undergoing cardiac surgery, seeking to explore the predictive value of perioperative markers of cerebral injury. We prospectively enroled 47 neurodevelopmentally normal infants prior to planned cardiac surgery. Postoperative monitoring consisted of 10-channel video synchronised, continuous electroencephalography from 6 to 30 h, Doppler assessment of cerebral blood flow in the anterior cerebral artery at 1, 2, 3 and 5 h, and measurement of serum S-100B at 0 and 24 h. Neurodevelopmental assessments were performed using the second edition of the Bayley Scale of Infant Development. Follow-up at 1 year was available on 35 infants. The mean age of these patients at surgery had been 57 ± 15 days. We observed clinical seizures in 1 patient, with 3 other patients having electroencephalographic abnormalities. At follow-up of 1 year, neurodevelopmental scores were lower than preoperative scores, with mean mental scores changing from 103 ± 5 to 94 ± 13 (p = 0.001), and mean motor scores changing from 99 ± 8 to 89 ± 20 (p = 0.004). No association was found between electroencephalographic abnormalities, reduced cerebral blood flow, or elevation of serum S-100B levels and impaired neurodevelopmental outcome at 1 year. Infants with electroencephalographic abnormalities had elevation of the levels of S-100B in the serum (p = 0.02). At 1 year of follow-up, infants undergoing cardiac surgery demonstrated a reduction in the scores achieved using the second edition of the Bayley Scale of Infant Development. They require ongoing assessment of their progress. Electroencephalographic abnormalities, cerebral blood flow, or levels of S-100B in the serum were not useful perioperative markers for predicting a poor neurodevelopmental outcome in the clinical setting.