Publications by authors named "Robert Halliday"

24 Publications

  • Page 1 of 1

Trajectories of post-surgical pain in infants admitted to neonatal intensive care.

Eur J Pain 2020 10 20;24(9):1822-1830. Epub 2020 Jul 20.

Department of Health Professions, Faculty of Medicine, Human and Health Sciences, Macquarie University, Sydney, NSW, Australia.

Background: The aim of this study was (a) to statistically identify distinct trajectories of pain following surgery in infants less than 6 months of age, and (b) to compare these trajectories to descriptions of chronic pain in infants in the neonatal intensive care unit (NICU).

Methods: This was a retrospective medical record review of infants admitted to a NICU between 2008 and 2018 following surgery. All infants who underwent one major procedure to the abdomen or thorax and returned to the NICU following surgery were included. Pain was assessed regularly using a validated Pain Assessment Tool. Group-based trajectory analysis was used to determine the trajectory of recovery from pain following surgery.

Results: A total of 726 infants were included in the study. A two-group trajectory model, defined as typical and atypical pain trajectories, was selected. The typical group (n = 467) consisted of infants who had significantly fewer days (1.5 ± 2.3 vs 5.3 ± 5.5, p < .001) and recorded instances of pain (2.0 ± 3.4 vs 9.7 ± 10.5, p < .001) compared to infants in the atypical group (n = 259). The incidence of iatrogenic neonatal abstinence syndrome was greater in the atypical than the typical group (11% vs 5%, p = .001).

Conclusions: This study has revealed two distinct pain trajectories in infants after surgery. While recovery from pain occurs within days in the typical group, the atypical group demonstrates pain for a significantly longer period, often weeks and months following surgery. This latter group adheres closely to current descriptions of chronic pain in infants.
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http://dx.doi.org/10.1002/ejp.1629DOI Listing
October 2020

Abdominal compartment syndrome monitoring in neonates with an acute abdomen - A pilot, retrospective, observational study.

J Pediatr Surg 2020 Jul 6;55(7):1296-1301. Epub 2019 Nov 6.

The Department of Surgery, The Children's Hospital Westmead, Sydney, Australia; Faculty of Medicine and Health, University of Sydney, Sydney, Australia.

Background: Abdominal compartment syndrome (ACS) is a pathological increase of the intra-abdominal pressure (IAP) with dysfunction of one or more organs. There is lack of clarity in neonates regarding what intravesical pressure (IVP) value, a surrogate marker for IAP, indicates the need for intervention for ACS.

Methods: The medical records at a Children's Hospital NICU were reviewed to identify all neonates that had IVP/s monitored over a 10-year period (2008-2017). Demographic parameters, IVPs, and important clinical outcomes were obtained. Associations between IVP monitoring and clinical outcomes were explored.

Results: Forty-six neonates had IVP monitoring, with 4 (8%) being diagnosed with ACS requiring further operative intervention. There was no significant correlation between IVP and need for surgery. There was a significant positive correlation between the maximum IVP and the need for total parenteral nutrition (rs = 0.350, p = 0.017), ventilator support (rs = 0.321, p = 0.034) and length of stay (rs = 0.362, p = 0.016) and between a diagnosis of ACS and neonatal mortality (rs = 0.299, p = 0.044).

Conclusions: IVP monitoring and raised IVP did not correlate with the need for surgical intervention. Raised IVP was associated with neonatal morbidity and maybe neonatal mortality. A large, prospective, observational study is required to evaluate the role of IVP monitoring in ACS and its associated outcomes.

Level Of Evidence: III.
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http://dx.doi.org/10.1016/j.jpedsurg.2019.08.003DOI Listing
July 2020

Developmental outcome at 3 years of age of infants following surgery for infantile hypertrophic pyloric stenosis.

Pediatr Surg Int 2019 Mar 7;35(3):357-363. Epub 2018 Nov 7.

Sydney Medical School, The University of Sydney, Sydney, Australia.

Purpose: The study compared neurodevelopmental outcome at 3 years of age of infants with infantile hypertrophic pyloric stenosis (IHPS) who underwent pyloromyotomy with healthy control infants in New South Wales, Australia.

Methods: Infants with IHPS as well as controls were recruited between August 2006 and July 2008. Developmental assessments were performed using the Bayley scales of infant and toddler development (version III) (BSITD-III) at 1 and 3 years of age.

Results: Of the 43 infants originally assessed at 1 year, 39 returned for assessment at 3 years (90%). The majority were term infants (77%). Assessments were also performed on 156 control infants. Infants with IHPS scored significantly lower on four of the five Bayley subsets (cognitive, receptive and expressive language and fine motor) compared to control infants. Analysis of co-variance showed statistically significant results in favour of the control group for these four subsets.

Conclusion: Compared with the outcomes at 1 year, infants with IHPS at 3 years of age continue to score below controls in four of the BSITD-III subscales. This suggests they should have developmental follow-up with targeted clinical intervention. There is a need for further studies into functional impact and longer term outcomes.
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http://dx.doi.org/10.1007/s00383-018-4408-0DOI Listing
March 2019

Newborn self-inflating manual resuscitators: precision robotic testing of safety and reliability.

Arch Dis Child Fetal Neonatal Ed 2019 Jul 18;104(4):F403-F408. Epub 2018 Oct 18.

Neonatal Intensive Care Unit, Westmead Hospital, Westmead, New South Wales, Australia.

Aim: A controlled bench test was undertaken to determine the performance variability among a range of neonatal self-inflating bags (SIB) compliant with current International Standards Organisation (ISO).

Introduction: Use of SIB to provide positive pressure ventilation during newborn resuscitation is a common emergency procedure. The United Nations programmes advocate increasing availability of SIB in low-income and middle-income nations and recommend devices compliant with ISO. No systematic study has evaluated variance in different models of neonatal SIB.

Methods: 20 models of SIB were incrementally compressed by an automated robotic device simulating the geometry and force of a human hand across a range of precise distances in a newborn lung model. Significance was calculated using analysis of variance repeated measures to determine the relationship between distance of SIB compression and delivered ventilation. A pass/fail was derived from a composite score comprising: minimum tidal volume; coefficient of variation (across all compression distances); peak pressures generated and functional compression distance.

Results: Ten out of the 20 models of SIB failed our testing methodology. Two models could not provide safe minimum tidal volumes (2.5-5 mL); six models exceeded safety inflation pressure limit >45 cm HO, representing 6% of their inflations; five models had excessive coefficient of variation (>30% averaged across compression distances) and three models did not deliver inflation volumes >2.5 mL until approximately 50% of maximum bag compression distance was reached. The study also found significant intrabatch variability and forward leakage.

Conclusion: Compliance of SIBs with ISO standards may not guarantee acceptable or safe performance to resuscitate newborn infants.
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http://dx.doi.org/10.1136/archdischild-2018-315391DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6764255PMC
July 2019

Effect of Delayed Cord Clamping on Cerebral Oxygenation in Very Preterm Infants.

Neonatology 2019 10;115(1):13-20. Epub 2018 Sep 10.

University of Sydney, Camperdown, New South Wales, Australia.

Background And Objective: The mechanism of reported benefits of delayed cord clamping (DCC) are unclear. We aimed to determine whether DCC compared to immediate cord clamping (ICC) in very preterm infants improves cerebral oxygenation in the first 24 h.

Study Design: This is a prospective study of a subset of infants at < 30 weeks of gestation who were randomised to DCC (≥60 s) or ICC (< 10 s) and required an indwelling arterial catheter. Regional cerebral oxygenation (rScO2), blood pressure, PI, and peripheral saturation were measured and cerebral fractional tissue oxygen extraction (cFTOE) calculated for the following 3 time intervals: 3-6, 6-12, and 12-28 h of age. Functional ultrasound measures including superior vena cava flow, right ventricular output, ductus arteriosus size and shunt and anterior cerebral artery resistive index were determined.

Results: The mean (±SD) gestation and birth weight of the 51 study infants were 27 ± 1 weeks and 1,046 ± 241 g respectively. Twenty infants received DCC and 31 received ICC. Baseline demographics were similar between the 2 groups. Comparing DCC and ICC infants, there was no difference in rScO2 or cFTOE at any time point. Three out of 20 infants did not receive DCC due to clinical concerns. A sensitivity analysis revealed that cord clamping ≥30 s was significantly associated with increased rScO2 and decreased cFTOE at all 3 time points after adjusting for gestation.

Conclusion: Although DCC was not associated with changes in cerebral oxygenation overall, sensitivity analysis suggested a possible effect of an increased rScO2 and a decreased cFTOE with ≥30 s of DCC.
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http://dx.doi.org/10.1159/000492712DOI Listing
December 2019

Contemporary incidence of stroke (focal infarct and/or haemorrhage) determined by neuroimaging and neurodevelopmental disability at 12 months of age in neonates undergoing cardiac surgery utilizing cardiopulmonary bypass.

Interact Cardiovasc Thorac Surg 2018 04;26(4):644-650

Heart Centre for Children, The Children's Hospital at Westmead, Sydney, Australia.

Objectives: When evaluated prospectively, acute brain injury is reported in up to 75% of neonates undergoing cardiopulmonary bypass (CPB), predominantly white matter injury rather than stroke. This study investigates the incidence of stroke (focal infarct and/or haemorrhage) detected by neuroimaging in contemporary clinical practice, whereby magnetic resonance imaging/computed tomography routinely occurs in response to clinical events, comparing those undergoing the Norwood procedure with those undergoing other neonatal procedures involving CPB, and defines the relationship between stroke and neurodevelopmental disability (NDD) at 12 months of age.

Methods: One hundred and twenty neonates underwent CPB between July 2011 and December 2014: 25 Norwood procedures and 95 non-Norwood procedures. Data were retrospectively collected including clinical data and 12-month neurodevelopmental assessment using the Bayley Scales of Infant and Toddler Development, Third Edition (BSID-III).

Results: Stroke was detected in 12% of neonates in current clinical practice: 24% of the Norwood group vs 8% of the non-Norwood group (P = 0.03). Significant predictors of stroke in the univariate analysis included the Norwood procedure, lowest operative temperature and use of extracorporeal membrane oxygenation (P < 0.05). The lowest operative temperature and use of extracorporeal membrane oxygenation remained significant in the multivariate analysis (P < 0.05). Fifty-seven percent were assessed using the BSID-III assessment, and 68% demonstrated NDD in at least 1 subscale. In neonates who suffered stroke, the incidence of NDD was significantly greater in 4/5 subscales compared with those with no injury (P < 0.05). The Norwood group had a significantly greater incidence of NDD in 2/5 subscales when compared with the non-Norwood group (P < 0.05).

Conclusions: Stroke, established by neuroimaging in contemporary clinical practice, was detected in 12% of neonates having CPB, and those undergoing the Norwood procedure have a 3-fold risk of injury. Stroke was associated with NDD at 12 months of age.
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http://dx.doi.org/10.1093/icvts/ivx375DOI Listing
April 2018

Developmental outcomes at three years of age of infants with esophageal atresia.

J Pediatr Surg 2016 Feb 5;51(2):249-51. Epub 2015 Nov 5.

Sydney Medical School, The University of Sydney, Australia; Douglas Cohen Department of Paediatric Surgery, The Children's Hospital at Westmead, Sydney, Australia. Electronic address:

Background/aims: Previous work assessing early developmental outcomes of infants at one year of age following surgery for esophageal atresia (OA) and/or tracheoesophageal fistula (TOF) found a significantly lower score in expressive language compared with control infants. The purpose of this study was to determine whether these differences were still evident at three years of age.

Methods: Between August 2006 and July 2008, infants who required surgical correction of OA/TOF were prospectively enrolled as part of a large study which examined developmental outcomes following surgery. Patients were reassessed using the Bayley Scales of Infant and Toddler Development Version III. Cognition, expressive and receptive language, fine motor and gross motor skills scores were compared with both the standardized norms and also with contemporaneous control infants.

Results: 24 of the 31 infants (77%) assessed at one year were reassessed at three years of age. There was no significant difference between the Bayley scores of the infants with OA/TOF and the standardized norms of the tests in four of the subsets: cognition, expressive language, fine and gross motor skills. Infants with OA scored higher than normative values for the receptive language subscale (P=0.001). When compared with the controls, there were no significant differences in any of the subscales.

Conclusion: Compared with the outcomes at one year, infants with OA/TOF have improved and were scoring within the normal range for the assessment at three years. This may in part be because of the early intervention services which many of these children were provided.
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http://dx.doi.org/10.1016/j.jpedsurg.2015.10.067DOI Listing
February 2016

Developmental outcomes at 3 years of age following major non-cardiac and cardiac surgery in term infants: A population-based study.

J Paediatr Child Health 2015 Dec 17;51(12):1221-5. Epub 2015 Jun 17.

Grace Centre for Newborn Care, The Children's Hospital at Westmead, Sydney, New South Wales, Australia.

Objective: The objective of this study was to determine whether there remain developmental differences between term infants at 3 years of age following major non-cardiac surgery (NCS) and cardiac surgery (CS) compared with healthy control infants in New South Wales (NSW), Australia.

Study Design: Between 2006 and 2008, term infants who required NCS or CS within the first ninety days of life were enrolled in a prospective population-based study. Their developmental outcome was then compared with a cohort of healthy term infants. Infants initially assessed at 1 year of age were then re-assessed at 3 years of age using the Bayley scales of infant and toddler development (version-

Iii) Results: Of the 539 term infants assessed at 1 year of age, 417 returned for the 3-year assessment, with 378 complete assessments. The mean scores for the infants who underwent CS (P < 0.001) were significantly lower in all subscales of the assessment compared with the controls, while the mean scores for the infants who underwent NCS were significantly lower in three of the subscales (P < 0.05). The infants who underwent CS scored significantly lower in four of the subscales (P < 0.05), compared with the infants who underwent NCS.

Conclusion: The second phase of this unique population-based study provides further data on the outcomes of infants who underwent major NCS and CS. Major surgery in infants continues to be associated with developmental delay at 3 years of age compared with control infants; however the majority of the delay is mild. The risk remains higher in CS group with the pattern and severity of delay similar to that observed in the first study.
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http://dx.doi.org/10.1111/jpc.12943DOI Listing
December 2015

The impact of surgery on the developmental status of late preterm infants - a cohort study.

J Neonatal Surg 2015 Jan-Mar;4(1). Epub 2015 Jan 10.

Grace Centre for Newborn Care, The Children's Hospital at Westmead, Sydney, NSW, Australia ; Sydney Medical School, The University of Sydney, Australia.

Aims: Despite increasing evidence in the literature regarding the impact of late prematurity on subsequent developmental impairment, the developmental outcome of late preterm infants who undergo major surgery remains unclear. The aim of this study therefore was to determine the developmental outcome for a cohort of late preterm surgical population.

Methods: Late preterm infants with a gestational age from 34-36 weeks inclusive who were enrolled in the state-wide prospective Development After Infant Surgery (DAISy) study and who had undergone non-cardiac major surgery within the first ninety days of life were eligible for inclusion. Infants were assessed at one and three years of ages.

Results: Forty-six infants were enrolled in the study, of which 38 infants had a complete developmental assessment at one year of age. Of these infants, late preterm infants scored significantly lower than the standardized norms of the assessment on the expressive language and gross motor subscales. At three years of age 26 infants were reassessed: late preterm infants who underwent major surgery only scored significantly lower than the standardized norms on the cognitive subscale (p less than 0.001).

Conclusions: These data provide the evidence that late preterm infants who undergo major non-cardiac surgery are at risk of developmental impairment and consideration should be given to enrolling this cohort in multi-disciplinary developmental follow-up clinics.
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http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4420401PMC
May 2015

Growth in children with congenital diaphragmatic hernia during the first year of life.

J Pediatr Surg 2014 Sep 26;49(9):1363-6. Epub 2014 Feb 26.

Department of Respiratory Medicine, The Children's Hospital at Westmead, Sydney, Australia; Discipline of Paediatrics and Child Health, Sydney Medical School, University of Sydney, Sydney, Australia.

Purpose: Infants with congenital diaphragmatic hernia (CDH) have high rates of mortality and long-term morbidity, including poor growth and failure to thrive. The aim of this study was to describe growth patterns during the first year of life in infants with congenital diaphragmatic hernia in a non-ECMO cohort.

Methods: Medical records of infants with CDH admitted to our center between January 2005 and December 2011 were reviewed. Infants with anthropometric measurements at 3, 6 and 12months were included. Anthropometric measurements were obtained for the first year of life. Logistic regression analyses were performed to find predictive associations with failure to thrive (FTT).

Result: Of the 45 survivors, 38 were seen twice (84%) and 24 (53%) were seen on three occasions to age 12months. Poor growth was observed with weight being most affected. FTT was present in 63% during the first six months of life. Days of mechanical ventilation were the only predictor of FTT. Besides poor weight gain, height and head circumference were also reduced. However, catch-up growth occurred during the second half of infancy and at age 12months failure to thrive had reduced by two thirds to 21%.

Conclusions: Poor growth is a common early finding in CDH patients, which improves during infancy. This emphasizes the importance of close follow-up and aggressive nutritional management in CDH patients.
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http://dx.doi.org/10.1016/j.jpedsurg.2014.02.081DOI Listing
September 2014

Neurodevelopmental outcome in Congenital Diaphragmatic Hernia survivors during the first three years.

Early Hum Dev 2014 Aug 3;90(8):413-5. Epub 2014 Jun 3.

Department of Respiratory Medicine, The Children's Hospital at Westmead, Sydney, Australia; Discipline of Paediatrics and Child Health, Sydney Medical School, University of Sydney, Sydney, Australia.

Objective: To determine neurodevelopmental outcome in Congenital Diaphragmatic Hernia (CDH) survivors during the first three years of life.

Methods: Admitted CDH patients were assessed at the age of 12 (n=18) and 36 months (n=15) using the Bayley Scales of Infant and Toddler Development Third Edition. Neurodevelopmental results of CDH patients were compared with published norms and with a healthy matched control group.

Results: At 12 months, receptive language was mildly delayed in 6% of patients and 6% of patients demonstrated mildly delay in expressive language and gross motor skills. Eighteen percent of CDH patients had severely delayed scores for gross motor skills. At 36 months, expressive language scores were mildly delayed in 21% of patients. When compared to the control group, CDH patients had similar neurodevelopmental scores at 12 and 36 months of age.

Conclusion: CDH is not necessarily associated with impaired neurodevelopmental outcomes during the first three years of life.

Summary: The aim of this study was to describe neurodevelopmental outcomes in Congenital Diaphragmatic Hernia (CDH) survivors. At 12 months, three (18%) patients had severely delayed scores for gross motor skills. At 36 months, expressive language scores were mildly delayed in three (21%) patients. Neurodevelopmental outcomes of CDH patients were no different to healthy matched controls at 12 and 36 months.
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http://dx.doi.org/10.1016/j.earlhumdev.2014.04.016DOI Listing
August 2014

A comparison of the performance of healthy Australian 3-year-olds with the standardised norms of the Bayley Scales of Infant and Toddler Development (version-III).

Arch Dis Child 2014 Jul 6;99(7):621-4. Epub 2014 Feb 6.

Background: Standardised developmental tests are now widely used in neurodevelopmental assessments of infants and children. In 2006, the revised and updated version of the Bayley Scales of Infant and Toddler Development (version III) replaced the previous version and is now widely used in neonatal developmental follow-up clinics. Several papers from Australia have highlighted underestimation of developmental impairment up to age 2 using this revised version. We aimed to ascertain how a cohort of healthy 3-year-old children performed compared to the standardised norms of the Bayley Scales of Infant and Toddler Development (version-III).

Method: Term healthy newborn control infants from the prospective Development after Infant Surgery (DAISy) study were included. At 3 years of age, the mean scores on each of the five subscales for 156 children were compared with the standardised norms.

Results: At 3 years of age, the mean scores were higher than the standardised norms on four of the subscales, cognition (<0.05), receptive and expressive language and fine motor (p<0.001). There was no significant difference in the gross motor scale (p=0.435).

Conclusions: Healthy term Australian children have a statistically significantly higher mean score on the Bayley Scales of Infant and Toddler Development (version-III) compared with the standardised means in four of the subtests, with the greatest difference in receptive language. This has implications for the assessment of children as the test may miss those with a minor delay and not reflect the severity of delay of infants that it does identify. We recommend that consideration ought to be given to re-standardising this assessment on Australian children.
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http://dx.doi.org/10.1136/archdischild-2013-304834DOI Listing
July 2014

Early developmental outcome following surgery for oesophageal atresia.

J Paediatr Child Health 2013 Jun 19;49(6):467-70. Epub 2013 Apr 19.

Grace Centre for Newborn Care, The Children's Hospital at Westmead, Westmead, New South Wales, Australia.

Aim: To compare the developmental outcome of infants with oesophageal atresia with or without trachea-oesophageal fistula (OA/TOF) who underwent surgery in early infancy with healthy control infants in New South Wales, Australia.

Methods: Infants diagnosed with OA/TOF requiring surgical intervention were enrolled prospectively between 1 August 2006 and the 31 December 2008. Healthy control infants were enrolled in the same time period. The children underwent a developmental assessment at 1 year of age (corrected) using the Bayley Scales of Infant and Toddler Development (Version III).

Results: Of 34 infants with OA/TOF that were enrolled, 31 had developmental assessments. The majority (75%) were term infants (≥37 weeks gestation) with a mean birth weight of 2717 g. Fourteen infants (44%) had an associated birth defect and one infant with multiple associated anomalies subsequently died. Developmental assessments were also performed on 62 control infants matched for gestational age. Infants with OA/TOF had a mean score significantly lower on the expressive language subscale (P < 0.05) compared with the control infants.

Conclusions: This study found a lower than expected developmental score for infants following surgery for OA/TOF in the expressive language subscale compared with the healthy control infants. These findings support concerns over the potential impact of OA/TOF and its effects on development. Further studies, including continuing developmental review to determine whether these differences persist and their functional importance, should be performed.
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http://dx.doi.org/10.1111/jpc.12206DOI Listing
June 2013

Which high-risk infants should we follow-up and how should we do it?

J Paediatr Child Health 2012 Sep;48(9):789-93

Grace Centre for Newborn Care, The Children's Hospital at Westmead, Sydney Medical School, The University of Sydney, Sydney, New South Wales, Australia.

Early detection of neurodevelopmental delay and appropriate intervention has been associated with improved academic and social outcomes. Identifying those who are at high risk and might benefit is not straightforward. Approximately 2% of infants are admitted to a neonatal intensive care unit after birth and these babies are known to be at high risk of developmental impairment. While it is well recognised that the extreme preterm infant is at high risk of developmental impairment, there is increasing evidence of a risk in late preterm infants as well as those undergoing major cardiac and non-cardiac surgery. Not all infants are enrolled in multidisciplinary follow-up clinics with easy access to early intervention. These clinics are expensive to run with both limited and conflicting data on their long-term value. This review will concentrate on identifying which infants are at risk, reviewing the aetiology of the risk factors and the efficacy of follow-up clinics.
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http://dx.doi.org/10.1111/j.1440-1754.2012.02540.xDOI Listing
September 2012

Early developmental outcomes following major noncardiac and cardiac surgery in term infants: a population-based study.

J Pediatr 2012 Oct 10;161(4):748-752.e1. Epub 2012 May 10.

Grace Centre for Newborn Care, The Children's Hospital at Westmead, Sydney, New South Wales, Australia.

Objective: To ascertain developmental differences between term infants after major noncardiac surgery and cardiac surgery compared with healthy control infants in New South Wales, Australia.

Study Design: This prospective population-based cohort study enrolled infants between August 1, 2006, and December 31, 2008, who required major noncardiac surgery within the first 90 days of life. Developmental outcomes were compared in these children, cohorts of term infants requiring cardiac surgery, and healthy controls. Infants were assessed at 1 year of age using the Bayley Scales of Infant and Toddler Development, Third Edition (BSID-III).

Results: Of the 784 infants enrolled, 688 (90.2%) of infants alive at 1 year were assessed. Of these, 539 infants were term and were included in the present analysis. Compared with controls, the infants who underwent cardiac surgery had significantly lower (P < .001) mean scores in all 5 BSID-III subscales, and the infants who underwent noncardiac surgery had significantly lower (P < .05) mean scores in 4 of the 5 BSID-III subscales. The greatest difference was in the incidence of gross motor delay in both the cardiac surgery group (OR, 0.25; 95% CI, 0.16-0.41) and the noncardiac surgery group (OR, 0.41; 95% CI, 0.26-0.63).

Conclusion: This unique population-based prospective study compared the developmental outcomes of infants who underwent major noncardiac surgery and cardiac surgery. Major surgery in infants was found to be significantly associated with developmental delay at 1 year of age compared with control infants. These data have important implications for interventions and clinical review in the first year of life.
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http://dx.doi.org/10.1016/j.jpeds.2012.03.044DOI Listing
October 2012

Recombinant parathyroid hormone therapy for severe neonatal hypoparathyroidism.

J Pediatr 2012 Feb 1;160(2):345-8. Epub 2011 Nov 1.

Children's Hospital at Westmead, Sydney, Australia.

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http://dx.doi.org/10.1016/j.jpeds.2011.09.022DOI Listing
February 2012

Early developmental outcome of infants with infantile hypertrophic pyloric stenosis.

J Pediatr Surg 2010 Dec;45(12):2369-72

Grace Centre for Newborn Care, The Children's Hospital at Westmead, Discipline of Paediatrics and Child Health, Sydney Medical School, The University of Sydney, New South Wales 2145, Australia.

Purpose: The study aimed to compare the developmental outcome of infants with infantile hypertrophic pyloric stenosis (IHPS) who underwent pyloromyotomy with healthy control infants in New South Wales (NSW), Australia.

Methods: Infants diagnosed with IHPS requiring surgical intervention were enrolled prospectively between August 1, 2006, and July 31, 2008. Healthy control infants were enrolled in the same period. The children underwent a developmental assessment at 1 year of age (corrected) using the Bayley Scales of Infant and Toddler Development (Version III).

Results: Of 52 infants with IHPS who were enrolled, 43 had developmental assessments. Most (90.6%) were term infants (>36 weeks gestation) with a median birth weight of 3237.8 g. Two infants (8%) had an associated birth defect, and survival was 100%. Developmental assessments were also performed on 211 control infants. Infants with IHPS scored significantly lower on the cognitive, receptive language, fine motor, and gross motor subscales compared to the control infants.

Conclusions: This unique study found lower than expected developmental scores for infants after surgery for IHPS than for healthy control infants. These findings raise concerns over the potential impact of IHPS and its surgical treatment. Further studies, including continuing developmental review to determine whether these differences persist and their functional importance, should be performed.
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http://dx.doi.org/10.1016/j.jpedsurg.2010.08.035DOI Listing
December 2010

Developmental outcomes following major surgery: what does the literature say?

J Paediatr Child Health 2011 Nov 6;47(11):766-70. Epub 2010 Oct 6.

Grace Centre for Newborn Care, The Children's Hospital at Westmead, Australia.

Relative to the wealth of information in the medical literature regarding developmental outcome for infants who have had cardiac surgery available, few studies specifically detail how those who have undergone major surgery grow and develop. The few published studies tend to be disease specific, making their results difficult to translate to a more general setting. As mortality for most infants who require surgery in infancy continues to decrease, the focus for researchers and clinicians should be on how these children will grow and develop. As parents realise that their infant will survive, this becomes their next major concern. The most common conditions requiring early major surgery have been reviewed in relation to data on infant developmental outcomes.
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http://dx.doi.org/10.1111/j.1440-1754.2010.01867.xDOI Listing
November 2011

A population-based study of the outcome after small bowel atresia/stenosis in New South Wales and the Australian Capital Territory, Australia, 1992-2003.

J Pediatr Surg 2008 Mar;43(3):484-8

Department of Paediatrics and Child Health, Grace Centre for Newborn Care, The Children's Hospital at Westmead, The University of Sydney, Westmead, NSW 2145, Australia.

Purpose: The purpose of the study was to describe the incidence, epidemiology, and survival of infants with small bowel atresia/stenosis in New South Wales (NSW) and the Australian Capital Territory (ACT), Australia.

Methods: A population-based cohort study was conducted of infants diagnosed with small bowel atresia/stenosis in NSW and the ACT from 1992 to 2003. Data were obtained from the prospectively collated NSW and ACT Neonatal Intensive Care Units' data collection. Individual risk factors for mortality were assessed using the chi(2) test.

Results: The incidence of small bowel atresia/stenosis in NSW and the ACT was 2.9 per 10,000 births. Of 299 infants identified with small bowel atresia, 13 were stillborn. Of the 286 live born infants, most (52%) were delivered preterm (<37 weeks' gestation) with an 87% survival, whereas 48% were term with a 98% survival. More than half the infants (54%) had an associated birth defect. The overall mortality was 8%. Prematurity and low birth weight were identified as independent risk factors for mortality (P < .001).

Conclusions: This study of small bowel atresia/stenosis provides population-based outcomes for clinicians and families. It is important to investigate infants with small bowel atresia for associated birth defects. Although the mortality rate has decreased over the last 50 years, it remains substantial at 8% and is higher in premature and low birth weight infants.
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http://dx.doi.org/10.1016/j.jpedsurg.2007.10.028DOI Listing
March 2008

Outcomes following surgery for congenital heart disease in low-birthweight infants.

J Paediatr Child Health 2007 May;43(5):370-5

The Children's Hospital at Westmead, New South Wales, Australia.

Aim: To describe cardiac surgery, survival and outcomes for low-birthweight (< or = 2500 g) infants undergoing surgery for congenital heart disease.

Methods: Using data from a prospectively collected population-based database of admissions to neonatal intensive care units in New South Wales and the Australian Capital Territory, we identified all low-birthweight infants undergoing cardiac surgery between 1992 and 2001. Infants with only a persistent ductus arteriosus were excluded. Two-year cardiac and neurodevelopmental outcome data were sought from hospital medical records.

Results: A total of 121 low-birthweight infants underwent cardiac surgery, of whom 34% had a congenital syndrome or non-cardiac birth defect. Most (81%) underwent a palliative surgical procedure in the neonatal period. There were 19 early (15.7%) and 19 late deaths giving a 2-year mortality of 31%. Factors associated with mortality included birthweight below 1500 g (P = 0.006), low weight at surgery (P = 0.028) and Apgar score at 1 min (P = 0.019). No single factor predicted 30-day mortality. By 2 years of age, 27 (33% of survivors) were known to have neurodevelopmental delay. Although 22 children are known to be developing normally, the neurodevelopmental status of 34 children was not known.

Conclusions: These surgical data were comparable to previous single-institution studies. This group had a high risk of disability due to prematurity, low birthweight and associated conditions. There is a need to prospectively assess and manage neurodevelopmental outcomes in this group.
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http://dx.doi.org/10.1111/j.1440-1754.2007.01082.xDOI Listing
May 2007

A population-based study of congenital diaphragmatic hernia outcome in New South Wales and the Australian Capital Territory, Australia, 1992-2001.

J Pediatr Surg 2006 Jun;41(6):1049-53

Department of Neonatology, The Children's Hospital at Westmead, Sydney, NSW 2145, Australia.

Purpose: The aim of the study was to describe the incidence and survival of infants born with congenital diaphragmatic hernia (CDH) in the state of New South Wales (NSW) and the Australian Capital Territory (ACT), Australia.

Methods: A population-based cohort study of all infants inclusive up to 1 month of age diagnosed with CDH in NSW and the ACT between 1992 and 2001 was conducted. Data sources were the NSW and ACT Neonatal Intensive Care Data Collection (Neonatal Intensive Care Units Study), NSW Birth Defects Register, Population Health Research Centre of ACT Health, and NSW Midwives Data Collection. Individual risk factors for mortality were assessed using the chi(2) test with P < .05 being considered statistically significant. Multivariate analysis was performed using logistic regression to adjust for potential confounding variables.

Results: From the databases used, the incidence of CDH in NSW and the ACT was 1 per 3800 births. Of 242 infants identified with CDH, 8% underwent termination of pregnancy, 10% were stillborn after 20 weeks' gestation, and 82% were liveborn. Most liveborn infants (70%) were delivered at term with a 64% survival, whereas 30% were preterm with a 35% survival. For liveborn infants, the overall preoperative mortality was 35% with 56% surviving to discharge. Logistic regression identified a low 5-minute Apgar score, prematurity, and air leak as independent risk factors for mortality.

Conclusions: This population-based study of CDH provides us with baseline data for our states. Mortality is high in preterm infants and in the preoperative period. Avoiding preterm delivery and improving preoperative stabilization are the measures most likely to improve survival.
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http://dx.doi.org/10.1016/j.jpedsurg.2006.01.073DOI Listing
June 2006

Severe transient hyperinsulinaemic hypoglycaemia: two neonates without predisposing factors and a review of the literature.

Eur J Pediatr 2004 Jan 29;163(1):38-41. Epub 2003 Oct 29.

Institute of Endocrinology and Diabetes, The Children's Hospital at Westmead, Corner Hawkesbury Road and Hainsworth Street, Locked Bag 4001, NSW 2145, Westmead, Sydney, Australia.

Unlabelled: We report on transient hyperinsulinism (HI), presenting as severe congenital HI, in two neonates born without intrauterine growth restriction, maternal diabetes, perinatal asphyxia or Rhesus/platelet isoimmunisation. The neonates developed early (<6 h of life), symptomatic, non-ketotic hypoglycaemia (0-0.66 mmol/l), associated with elevated insulin levels (40-200 mU/l), and required high glucose infusion rates (22-24 mg/kg per min) to maintain normoglycaemia. However, both babies were diazoxide-sensitive and did not require glucose infusions beyond 2 weeks of life. Neither neonate had elevated serum ammonia levels or evidence of a metabolic disorder.

Conclusion: Transient hyperinsulinism can occur in newborns delivered uneventfully without significant perinatal complications. The unusual sensitivity to medical treatment in these cases of neonatal-onset hyperinsulinaemic hypoglycaemia underscores the importance of careful medical management of severe congenital hyperinsulinism. Careful consideration of the indication and if necessary, timing and extent of pancreatectomy is required, while maintaining euglycaemia to protect the developing brain.
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http://dx.doi.org/10.1007/s00431-003-1328-2DOI Listing
January 2004