Survival and neurodevelopmental outcomes in extremely preterm infants 22-24 weeks of gestation born in Western Australia.

AIM: The management of births at borderline viability continues to present dilemmas for health professionals and parents. The aim of the study was to review local outcomes of infants born between 22 and 24 weeks of gestation between 2004 and 2010 in Western Australia (WA) to aid perinatal counselling. METHODS: Survival data for the study were sourced retrospectively from the Neonatal Clinical Care Unit and Department of Health records of births in WA. Neurodevelopmental follow-up outcomes were assessed using the most recent standardised assessment (Griffiths, Bayley-III and Wechsler Preschool and Primary Scale of Intelligence, 3rd Ed) and medical examination of infants/children 12 months to 8 years from follow-up clinic appointments. RESULTS: At these gestations, 159 survivors represented 72% of neonatal intensive care unit admissions, 53% of WA live births and 26% of WA live and still births; 5% of live births survived at 22 weeks, 46% at 23 weeks and 77% at 24 weeks. Of the 14 outborn/retrieved infants, 4 survived (29%). At a median age of 59 months, disabilities were severe in 13% of children (22-23w = 19%; 24w = 11%). The median test quotient was 90. Moderate and severe cognitive disability was found in 16%, cerebral palsy was found in 7% (n = 11), and 55% of children were free from impairment as defined in this study. CONCLUSION: At these gestations, survival figures varied markedly with the chosen population denominator. Regional data are essential for valid population comparison. While many developmental difficulties occurred in these children, 78% were free from moderate or severe impairment at ages 3-5 years.

The relationship between sleep problems and working memory in children born very preterm.

In two studies, the relationship between sleep and working memory performance was investigated in children born very preterm (i.e., gestation less than 32 weeks) and the possible mechanisms underlying this relationship. In Study 1, parent-reported measures of snoring, night-time sleep quality, and daytime sleepiness were collected on 89 children born very preterm aged 6 to 7 years. The children completed a verbal working memory task, as well as measures of processing speed and verbal storage capacity. Night-time sleep quality was found to be associated with verbal working memory performance over and above the variance associated with individual differences in processing speed and storage capacity, suggesting that poor sleep may have an impact on the executive component of working memory. Snoring and daytime sleepiness were not found to be associated with working memory performance. Study 2 introduced a direct measure of executive functioning and examined whether sleep problems would differentially impact the executive functioning of children born very preterm relative to children born to term. Parent-reported sleep problems were collected on 43 children born very preterm and 48 children born to term (aged 6 to 9 years). Problematic sleep was found to adversely impact executive functioning in the very preterm group, while no effect of sleep was found in the control group. These findings implicate executive dysfunction as a possible mechanism by which problematic sleep adversely impacts upon cognition in children born very preterm, and suggest that sleep problems can increase the cognitive vulnerability already experienced by many of these children.

Servo controlled versus manual cooling methods in neonates with hypoxic ischemic encephalopathy.

BACKGROUND: Therapeutic hypothermia is known to improve outcomes in neonates with hypoxic ischemic encephalopathy (HIE). There are no studies that have compared servo controlled cooling (SCC) versus manually controlled cooling (MCC) methods in HIE. AIM: To compare the outcomes of SCC versus MCC in neonates with HIE. METHODS AND OUTCOME MEASURES: Between Jan 2008 and May 2011, MCC with cool-gel packs was used to achieve rectal temperatures of 33.5 to 34.5°C in our units. Subsequently, we changed to SCC to achieve rectal temperatures of 33 to 34°C. 105 neonates received SCC whereas 95 received MCC. Retrospective study with multivariate analysis was conducted comparing thermoregulation (primary outcome) and neurodevelopmental outcomes of SCC versus MCC. RESULTS: In the SCC group, 72.3% had stage 2 or 3 HIE versus 77.9% in the MCC. The remaining had stage 1, but attending neonatologists decided to provide cooling. Prescribed rectal temperatures were achieved in 80.5% (5768/7168) instances in SCC versus 72.9% (4449/6108) in MCC (p<0.0001). There were no significant differences in the incidence of 'death or moderate-severe disability' [SCC 26/85 vs MCC 26/87, adjusted odds ratio: 1.29, 95% CI: 0.48, 3.42; p=0.614]. The results were similar after excluding stage 1 HIE [SCC 25/66 vs MCC 26/69, adjusted odds ratio: 1.01, 95% CI: 0.50, 2.02; p=0.981]. CONCLUSIONS: SCC resulted in better thermoregulation in neonates undergoing therapeutic hypothermia. There were no significant differences in neurodevelopmental outcomes, but the study was underpowered to answer this. Randomized trials are needed to fine-tune various aspects of TH in neonates with HIE.

Neurodevelopmental outcomes of neonates undergoing surgery under general anesthesia for malrotation of intestines.

BACKGROUND: It is difficult to differentiate between the potential adverse effects of general anesthesia (GA) on the developing brain and the role of associated co-morbidities and syndromes that can adversely affect neurodevelopmental outcomes in neonates undergoing GA. Neonates with malrotation of the intestines without volvulus usually do not have co-morbidities or syndromes. In addition, majority of them recover very well after surgery and are discharged home within a few days. Neonates with malrotation are a clean cohort of babies to study the role of a single episode of GA on the developing brain. AIMS: The study aimed to evaluate the neurodevelopmental outcomes of neonates undergoing GA for malrotation surgery. STUDY DESIGN: Retrospective review of neonates born at gestational age of ≥32weeks undergoing laparotomy for malrotation. OUTCOME MEASURES: Neurodevelopment in the study cohort at the age of one year. RESULTS: 33 eligible infants were identified from the departmental database. All 33 survived and were assessed using the Griffiths Mental Development Scales (GMDS) at one year. Mean general quotient (GQ) of the study population was 98 (SD 7.33) which was similar to the population norms (100.2, SD 12.8); p value 0.10. None of the infants developed cerebral palsy, tone abnormality, sensorineural deafness or blindness. There was no significant difference in the centiles at birth versus one year for weight and length (p values 0.454 and 0.178 respectively). Reassuringly, the head circumference centiles at one year showed a trend towards higher values (p value: 0.0735). CONCLUSION: One year developmental outcomes of neonates undergoing surgery under GA for malrotation were similar to population norms.

Impact of hypoglycaemia on neurodevelopmental outcomes in hypoxic ischaemic encephalopathy: a retrospective cohort study.

BACKGROUND: Low blood glucose levels (BGLs) in infants are known to adversely affect neurodevelopmental outcomes. However, this risk is not well explored in infants with hypoxic ischaemic encephalopathy (HIE) that receive therapeutic hypothermia (TH). Additionally, little information is available on the optimal BGLs to target in infants with HIE. AIM: To explore the association between hypoglycaemia and neurodevelopmental outcomes at different BGL thresholds (2.6 and 3.0 mmol/L) in neonates with HIE treated with TH. METHODS: Retrospective cohort study. Clinical information and 2-year neurodevelopmental data using Bayley Scales of Infant Development, third edition (BSID-III) and disabilities were recorded for infants born in Western Australia with HIE and treated with TH between February 2008 and February 2012. Multivariable logistic regression models explored the association between hypoglycaemia and neurodevelopmental outcomes. RESULTS: 122 infants underwent a total of 1616 BGL estimations before and during 72 hours of TH. Hypoglycaemia (BGL<2.6 mmol/L) occurred in 38/122 (31%) infants and 11/122 (9%) had recurrent hypoglycaemia (three or more episodes). Infants with recurrent hypoglycaemia (<2.6 mmol/L) had significantly lower mean BSID-III cognitive, language and socioemotional subscale scores. On multivariable analysis, recurrent hypoglycaemia (<2.6 mmol/L) was associated with increased odds of death or disability (adjusted OR 8.15; 95% CI 1.31 to 50.58; p=0.024). Recurrent hypoglycaemia (<3.0 mmol/L) during the first 12 hours of life was also associated with severe disability among survivors (adjusted OR 11.13; 95% CI 2.06 to 59.89; p=0.005). CONCLUSIONS: Early recurrent hypoglycaemia was associated with increased risk of death or severe disability in neonates undergoing TH for HIE. Prospective studies are needed to identify the ideal target BGL in this population.

Developmental outcomes in cerebrospinal fluid proven enteroviral meningitis in neonates > 32 weeks of gestation.

AIM: The aim of this study is to assess the short-term and long-term (1 year) outcomes of cerebrospinal fluid (CSF) confirmed enteroviral meningitis in neonates > 32 weeks of gestation. METHODS: A retrospective audit of neonates admitted between 1 July 2002 to 30 June 2012. RESULTS: Thirty-three neonates were diagnosed with enteroviral meningitis based on a positive CSF enteroviral PCR. Physical growth and neurodevelopmental outcomes at 1 year corrected for prematurity were available for 24 infants. All infants were alive at 1 year. The median weight, length and head circumference at 1 year were in the 72nd, 62nd and 78th centile and were comparable with the birth parameters. The mean general quotient (GQ) was 98.5 (SD 7.1) and was not significantly different from the population mean of 100.2 (P = 0.27). None of the infants had a GQ > 2SD below the population mean. Neurological recovery was complete in the 24 neonates assessed except one, who developed cerebral palsy, epilepsy and progressive hydrocephalus requiring ventriculoperitoneal shunt at 1 year. CONCLUSION: Neonatal enteroviral meningitis was associated with optimal growth and neurodevelopment in the majority of the infants at 1 year corrected for prematurity. Longer term studies are needed to better define developmental outcomes.

How do you think she feels? Vulnerability in empathy and the role of attention in school-aged children born extremely preterm.

The aim of this study was to examine empathic competence in children born extremely preterm (EP, <28 weeks) given vulnerabilities in social relationships. Empathy in typically developing children is mediated by executive functions. Executive functioning is also impaired in preterm children. Of particular interest in this study are the attentional components of executive functioning as mediators of empathic development. Thirty-two 7-year-old EP children and 40 age-matched term children participated in the Project K.I.D.S program and completed the Kids Empathy Development Scale (KEDS), Wechsler Intelligence Scale for Children (WISC-IV), and Test of Everyday Attention for Children (TEA-Ch). Children born extremely preterm exhibited poorer performance on all measures. The mediating role of attention in empathy competence was not supported by mediation modelling when FSIQ was controlled. As predicted, the EP group showed weaker empathic development relative to typically developing children. They also showed poorer attentional abilities. However, the effect of preterm birth on empathy was not mediated by executive-level attention. The cognitive mechanisms underpinning poor empathy competence in EP children remain unclear. Future research needs to examine the role of inhibition, social-emotional recognition, and regulation.

Outcomes of infants with Apgar score of zero at 10†min: the West Australian experience.

BACKGROUND: Infants who have an Apgar score of zero at 10 min of age are known to have poor long-term prognosis. Expert committee guidelines suggest that it is reasonable to cease resuscitation efforts if the asphyxiated infant does not demonstrate a heart beat by 10 min of life. These guidelines are based on data from the era when therapeutic hypothermia was not the standard of care for hypoxic ischaemic encephalopathy (HIE). Hence, we aimed to review our unit data from the era of therapeutic hypothermia to evaluate the outcomes of infants who had an Apgar score of zero at 10 min and had survived to reach the neonatal intensive care unit. METHODS: Retrospective chart review. STUDY PERIOD: 2007-2013. RESULTS: 13 infants (gestational age ≥35 weeks) with Apgar scores of zero at 10 min were admitted to the neonatal intensive care unit. All were born outside the tertiary perinatal centre. Of them, eight died before discharge. The type and duration of follow-up varied. Of the five survivors, three had normal cognitive scores (100, 100 and 110) on Bayley III assessment at 2 years of age and one had normal Griffiths score (general quotient (GQ) 103) at 1 year. Only one infant developed severe spastic quadriplegia. CONCLUSIONS: 4 out of 13 (30.7%) infants with 10 min Apgar scores of zero who survived to reach the neonatal intensive care unit had normal scores on formal developmental assessments. Information from large databases (preferably population based) is necessary to review recommendations regarding stopping delivery room resuscitation in term infants.

Neurodevelopmental outcomes at 7†years' corrected age in preterm infants who were fed high-dose docosahexaenoic acid to term equivalent: a follow-up of a randomised controlled trial.

OBJECTIVE: To determine if improvements in cognitive outcome detected at 18 months' corrected age (CA) in infants born <33 weeks' gestation receiving a high-docosahexaenoic acid (DHA) compared with standard-DHA diet were sustained in early childhood. DESIGN: Follow-up of a multicentre randomised controlled trial. Randomisation was stratified for sex, birth weight (<1250 vs ≥1250 g) and hospital. SETTING: Five Australian tertiary hospitals from 2008 to 2013. PARTICIPANTS: 626 of the 657 participants randomised between 2001 and 2005 were eligible to participate. INTERVENTIONS: High-DHA (≈1% total fatty acids) enteral feeds compared with standard-DHA (≈0.3% total fatty acids) from age 2-4 days until term CA. PRIMARY OUTCOME: Full Scale IQ of the Wechsler Abbreviated Scale of Intelligence (WASI) at 7 years CA. Prespecified subgroup analyses based on the randomisation strata (sex, birth weight) were conducted. RESULTS: 604 (92% of the 657 originally randomised) consented to participate (291 high-DHA, 313 standard-DHA). To address missing data in the 604 consenting participants (22 for primary outcome), multiple imputation was performed. The Full Scale IQ was not significantly different between groups (high-DHA 98.3, SD 14.0, standard-DHA 98.5, SD 14.9; mean difference adjusted for sex, birthweight strata and hospital -0.3, 95% CI -2.9 to 2.2; p=0.79). There were no significant differences in any secondary outcomes. In prespecified subgroup analyses, there was a significant sex by treatment interaction on measures of parent-reported executive function and behaviour. Scores were within the normal range but girls receiving the high-DHA diet scored significantly higher (poorer outcome) compared with girls receiving the standard-DHA diet. CONCLUSIONS: Supplementing the diets of preterm infants with a DHA dose of approximately 1% total fatty acids from days 2-4 until term CA showed no evidence of benefit at 7 years' CA. TRIAL REGISTRATION NUMBER: Australian New Zealand Clinical Trials Registry: ACTRN12606000327583.

Growth and developmental outcomes of infants with hirschsprung disease presenting in the neonatal period: a retrospective study.

OBJECTIVES: To describe the presentation and progress over the first year of life of neonates with Hirschsprung disease, to describe their physical and developmental outcomes at 12 months of age, and to compare the outcomes of infants with short- vs long-segment Hirschsprung disease. STUDY DESIGN: A retrospective study of neonates born with Hirschsprung disease in Western Australia between January 1, 2001, and December 31, 2010, to review their presentation, progress, growth, and development at 12 months of age. RESULTS: Fifty-four infants were identified (40 with short and 11 with long segment and 3 with total colonic aganglionosis); 9 infants had a recognized syndrome and 1 infant died, unrelated to Hirschsprung disease. A primary pull-through procedure was performed in 97% and 21% of neonates with short- and non-short-segment Hirschsprung disease, respectively; 17 (31%) infants developed anal stenosis requiring dilatations. Enterocolitis occurred in 14 (26%) infants. Griffiths Mental Development Scale scores (1 year) were available in 31 of 45 nonsyndromic survivors: mean general quotient (94.2, SD 8.89) was significantly less than the population mean (P = .007), but the number of infants with developmental delay was within the expected range. Physical growth, except length, appeared adequate in nonsyndromic infants. There were no significant differences in the outcomes of infants with short- vs non-short-segment Hirschsprung disease. CONCLUSIONS: At 1 year of age, many infants with Hirschsprung disease have ongoing gastrointestinal problems. Their overall growth appears satisfactory, and most infants are developing normally; however, their mean general quotient appears shifted to the left. Longer-term studies will better define developmental outcomes.

Outcomes of hypoxic ischaemic encephalopathy treated with therapeutic hypothermia using cool gel packs - experience from Western Australia.

UNLABELLED: Therapeutic hypothermia is the standard clinical practice for neonates with moderate to severe hypoxic ischaemic encephalopathy (HIE). AIM: To describe the two year neurodevelopmental outcomes of neonates who were routinely cooled using cool gel packs for HIE in Western Australia. METHODS: Retrospective study. Cases were identified from the neonatal databases. Information was collected from chart review. RESULTS: 65 infants received therapeutic hypothermia, of which 13 had mild, 35 moderate and 17 had severe HIE. There were no serious adverse effects attributable to cooling. All 13 infants with mild HIE survived, of whom developmental outcomes were available on nine; none had severe disability. Among 52 infants with moderate to severe HIE, there were nine deaths (17%) and developmental outcomes were available on 39; the incidence of severe disability was 23%. The risk of death or severe disability was 40% in infants with moderate to severe HIE. Physical growth was adequate at two years of age. CONCLUSIONS: Neonates undergoing therapeutic hypothermia with cool gel packs had both good survival rates and long term neurodevelopmental outcomes and met international benchmarks.

Growth and developmental outcomes of infants with gastroschisis at one year of age: a retrospective study.

BACKGROUND: The aim of the study was to describe the physical growth and developmental outcomes of babies born with gastroschisis. METHODS: We retrospectively reviewed all cases of gastroschisis in Western Australia born between 1997 and 2010. RESULTS: In the 128 pregnancies with fetal gastroschisis, 117 babies were live born. 112 (95.7%) survived to one year. 19% had z scores of<-1.28 for weight at birth (<10th centiles) compared with 30% at one year. Neurodevelopmental data were available in 88/112 (79%) of survivors (Griffiths scores in 67; reports of ages and stages questionnaire (ASQ) in 21). The mean GQ at 12 months was 99 (SD 9.8). Suboptimal neurodevelopmental outcomes were noted in eight. Complex gastroschisis (present at birth) and acquired gut related complications were associated with adverse long term outcomes. The incidence of acquired gut complications was least (5%) in those who underwent silo reduction as the primary management. However, on univariate and multivariate analysis, the type of primary reduction did not significantly influence the outcome. CONCLUSIONS: A large proportion of infants with gastroschisis exhibit suboptimal weight gain during the first year. The incidence of adverse developmental outcomes appears to be low.

Poractant alfa versus beractant for respiratory distress syndrome in preterm infants: a retrospective cohort study.

AIM: Poractant alfa and beractant are the commonly used animal derived surfactants in preterm infants with respiratory distress syndrome. Between 2005 and 2007, poractant alfa and beractant were alternated every month in our neonatal intensive care unit for 27 months. The aim of this study was to compare the outcomes of preterm infants who received poractant alfa versus beractant. METHOD: Single-centre, retrospective cohort study of inborn preterm infants <32 weeks gestation (23-31(+6) ). RESULTS: Six hundred sixty-four preterm infants (<32 weeks) were born during the study period, of which 415 received surfactant (poractant alfa: 214; beractant: 201). Infants in the poractant alfa group were 2.8 days younger than beractant (27.0 ± 2.3 vs. 27.4 ± 2.3 weeks; P = 0.03). All other baseline characters including Clinical Risk Index for Babies II scores were similar for both groups. No significant differences were found for the following outcomes: death or chronic lung disease (78/212 vs. 59/200; P = 0.28); death (24/214 vs. 15/201, P = 0.24); moderate to severe chronic lung disease (63/212 vs. 46/200; P = 0.45) and moderate to severe disability (20/163 vs. 19/151, P = 0.98) between poractant alfa and beractant, respectively. CONCLUSIONS: The results of our study do not support the need for preferential use of poractant alfa or beractant.

Outcomes of preterm neonates with frontal horn cysts: a retrospective study.

Isolated paraventricular frontal horn cysts are sometimes encountered on cranial ultrasound examinations of preterm neonates. The etiology and clinical significance of these lesions are unclear. The authors aimed to identify antenatal/intrapartum risk factors associated with the occurrence of these cysts and to assess developmental outcomes of preterm neonates with isolated frontal horn cysts. A retrospective cohort study with matched control design was used. A total of 28 cases were matched for gestation with 56 controls. No antenatal/intrapartum factors were associated with these cysts. At corrected age of 1 year, there was no difference in the mean general quotient between cases and controls (97.75 ± 17.28 vs 94.94 ± 9.86; P = .410). In all, 1 case and no controls had a diagnosis of cerebral palsy and 1 case and 3 controls had general quotients less than 80. The authors conclude that isolated paraventricular frontal horn cysts are benign, with no effect on neurodevelopment.