Growth and risk of adverse neuro-developmental outcome in newborns with congenital heart disease: A single-centre retrospective study.

BACKGROUND: Mortality in infants with CHD is decreasing and focus has shifted to improving long-term outcomes. Growth and neurodevelopmental outcomes are important long-term endpoints for both clinicians and parents. OBJECTIVE: To evaluate growth and determine the impact of growth on neurodevelopmental outcomes at one year of age in infants who had an operative or therapeutic catheter procedure for CHD in neonatal period. METHODS: This was a single-centre retrospective cohort study of infants born at term gestation with CHD. Demographic details, measurements of growth, and scores of Bayley Scales of Infant and Toddler Assessment (third edition), were collected. Enrolled study participants were categorised in subgroups based on the procedures required prior to one-year assessment. Regression analysis was performed to explore predictive ability of anthropometric measurements on mean scores of developmental assessment. RESULTS: A total of 184 infants were included in the study. Mean z-scores for weight and head circumference at birth were age-appropriate. Mean scores for various developmental domains ranged from borderline to normal except for infants with single ventricular physiology who had gross motor delay concomitant with growth failure. In this group, z-score of weight at one-year-assessment predicted mean score of cognition (p-value 0.02), fine motor (p-value 0.03) and gross motor nearly (p-value 0.06). CONCLUSIONS: Infants born at term gestation, with CHD, without genetic diagnosis had normal fetal growth. Postnatal growth restriction and developmental delay was most marked in infants with single ventricle physiology, suggesting careful nutritional and developmental monitoring.

Bullous eruptions in transient abnormal myelopoiesis with normal phenotype.

Cutaneous lesions are common manifestation of congenital leukaemia especially myeloid type with incidence of 25%-50% in reported cases. It is relatively rare in transient abnormal myelopoiesis (TAM) seen in trisomy 21 (~10%). The rashes seen in leukaemia and TAM are different. We report a case with a rare presentation of confluent bullous eruption in a phenotypically normal neonate with trisomy 21 restricted to haematopoietic blast cells. This rash resolved rapidly after low-dose cytarabine therapy with normalisation of total white cell counts. The risk of Down syndrome-associated myeloid leukaemia in such cases is still high (19%-23%) in first 5 years and rare thereafter.

Growth and risk of adverse neuro-developmental outcome in infants with congenital heart disease: A systematic review.

AIM: Congenital heart disease (CHD) is one of the most common birth defects affecting around 1:100 infants. In this systematic review, we aimed to determine impact of growth on neurodevelopmental outcomes of infants with CHD. METHODS: Studies that reported association of growth with developmental outcomes in infants with CHD who had surgery, were included. The search strategy was prospectively registered. Relevant studies were identified by electronic searches. The Cochrane Central Register of Controlled Trials, MEDLINE and EMBASE were searched from their earliest date to February 2022. RESULTS: Twenty studies met inclusion criteria. Choice of growth measures, developmental assessment tools and timing of assessment varied widely precluding conduct of a meta-analysis. Seventeen studies reported on infants who had cardio-pulmonary bypass. Birth weight was reported in thirteen studies and was associated with adverse outcome in nine. Head circumference at birth and later predicted developmental outcomes in five. Impaired postnatal growth was associated with adverse developmental outcome in seven studies. CONCLUSION: Growth in infants with congenital heart disease, specifically single ventricle physiology can predict adverse neurodevelopmental outcome. Included studies showed significant clinical heterogeneity. Uniformity should be agreed by various data registries with routine prospective collection of growth and developmental data.

Growth and risk of adverse neurodevelopmental outcome in infants with congenital surgical anomalies: a systematic review.

Survival of infants with congenital anomalies requiring surgical correction has increased dramatically, shifting the focus of clinical care and research toward optimising growth and neurodevelopment.To determine the impact of growth on neurodevelopmental outcomes of infants with congenital surgical anomalies. Studies that reported association of growth with developmental outcomes in infants with congenital anomalies who had surgery, were eligible for inclusion. The search strategy was prospectively drafted, and relevant studies were identified by electronic searches. The Cochrane Central Register of Controlled Trials (CENTRAL, The Cochrane Library 2022, issue 1), MEDLINE and EMBASE from their earliest date to February 2022 were searched. Seven studies met the inclusion criteria. Variability in selection of growth measures, developmental assessment tools and assessment endpoints for neurodevelopment, precluded a meta-analysis. Four studies reported the association between growth and neurodevelopmental outcomes in infants with gastroschisis with two noting adverse outcomes in infants who were small for gestational age. Birthweight, reported in four studies, was not associated with adverse developmental outcomes. Postnatal growth, reported in three studies, was associated with adverse outcome. The data linking growth restriction in infants with congenital surgical anomalies and neurodevelopmental outcome is limited. There is limited published research examining the longitudinal effects of intra- and extra-uterine growth parameters on neurodevelopmental outcomes.

Outcomes for infants with congenital diaphragmatic hernia: A single campus review with low extracorporeal membrane oxygenation utilisation.

AIM: To report the outcome for infants with congenital diaphragmatic hernia (CDH) and identify clinical factors affecting outcome from a tertiary perinatal surgical campus where extracorporeal membrane oxygenation (ECMO) is available however rarely utilised. METHODS: A retrospective cohort study of infants with CDH born in a co-located perinatal neonatal intensive care unit (NICU) or retrieved into and managed at a surgical NICU: 2003-2018. RESULTS: One hundred and fifty-nine infants with CDH were identified. One hundred and twenty were born in the co-located hospital and 39 retrieved from outlying hospitals. Survival of all patients with CDH was 74.8%; The survival for all isolated left CDH was 89% and the survival of post-surgery was 93%. Two patients went onto ECMO and both died. Associated major congenital anomalies were seen in 13.2%. Low birthweight (< 2500 g), 5-min Apgar <5, antenatal diagnosis, right-sided diaphragmatic hernia, herniation of the liver, associated major congenital anomalies, high oxygenation index (>25) on day 1, inotrope use, inhaled nitric oxide and need for high-frequency ventilation were associated with increased mortality on univariate analysis. Neurodevelopmental follow-up was commenced in 2013. Forty-three infants were discharged and developmental data were available for 36 at 4 months (83%) and 32 at 1 year (73%). Outcomes revealed normal motor scores and expressive language with mild delay in the receptive language at 1 year. CONCLUSION: Despite the low use of ECMO, our centre's results reveal excellent survival and neurodevelopmental outcomes consistent with or better than international data.

Management and referral of acute laryngotracheitis - Croup: The New South Wales Neonatal and Paediatric Emergency Transport Service experience.

AIM: To evaluate the experience of NETS (The Newborn and paediatric Emergency Transport Service) New South Wales (NSW) with children referred with a provisional diagnosis of croup. To examine the adherence of referral hospitals to NSW Health policy guidelines and to establish criteria for the need to transfer and the level of escort required for a child with croup to a higher level of care. METHODS: A retrospective audit of all children with a provisional diagnosis of croup referred to NETS over the epoch: 2005-2015. The NETS database was searched for keywords: croup and laryngotracheitis. Demographics, retrieval characteristics and management strategies were collected. RESULTS: A total of 432 patients were identified. The number of children triaged to a medically led NETS team for transfer decreased (P < 0.001) whilst those transferred into more local regional or metropolitan hospitals (P < 0.001) increased. The number of adrenaline doses was not associated with intensive care admissions or the need for invasive ventilation. CONCLUSION: Medically led retrieval teams are not necessary for safe transfer of children with a provisional diagnosis of croup unless the airway is secured with an endotracheal tube. Transfer to more local hospitals for paediatric care should be considered and time allowed for medications to take effect prior to movement.

Improving Intubation Success in Pediatric and Neonatal Transport Using Simulation.

BACKGROUND: Pediatric and neonatal first-pass intubation rates are higher in adult trained retrieval services than in neonatal or pediatric trained services. Some authors have attributed this to more frequent opportunities to practice the skill in the adult population. OBJECTIVE: The aim of this study was to increase the first-pass intubation rate without adverse events by introducing daily intubation simulation at our mixed neonatal and pediatric retrieval service. METHODS: This prospective cohort study performed from July to December 2018 in our mixed neonatal and pediatric retrieval service involved 16 medical staff performing simulated intubation at commencement of their retrieval shift with a retrieval nurse. Checklists for neonatal and pediatric intubation were introduced to the retrieval service for the intervention cohort. Participants were asked to complete questionnaires about intubation performed on retrieval to gather data not routinely collected by the service. RESULTS: Seven hundred and sixty-eight patients were retrieved by the service and 70 patients required intubation by the retrieval team during the intervention period. First-pass intubation rates were higher during the intervention period compared with a historical cohort, despite less intubations being performed overall. First-pass intubation rates improved from 59% to 78% in neonatal patients (P = 0.032), 58% to 65% in pediatric patients (P = 0.68) and from 58% to 74% overall (P = 0.043). There were no severe adverse events detected during the intervention period. Minor adverse events were associated with multiple attempts at intubation (P < 0.001). Overall compliance with simulation protocol was 43.5%, and on average, each doctor completed simulation once per month. CONCLUSIONS: Simulation is a useful adjunct to support neonatal and pediatric intubation training in the current environment of reducing intubation frequency.

Umbilical venous catheterisation: emergency central venous access which saves lives in coarctation of the aorta.

We describe a 9-day-old baby with coarctation of the aorta who required urgent resuscitation including intubation and cardiac compressions. Despite the commencement of prostaglandin E1 (PGE1) to reopen the ductus arteriosus via the intraosseous route, postductal saturations remained unrecordable for a further 45 min. Within 3 min of administration of PGE1 via an umbilical venous catheter (UVC), saturations were recordable at 92%. UVC access was the sentinel intervention that irrevocably altered the clinical prognosis. This baby boy has survived with excellent neurodevelopmental outcome. Clinicians are less familiar with UVCs outside of the newborn period. Our data demonstrate successful placement in neonates up to 28 days of age. We hope this case encourages clinicians to consider the UVC as first-line central venous access in collapsed neonates. In cases of suspected left heart obstruction, we argue that UVCs are the optimal route.

Retrieval of neonatal and paediatric patients on extracorporeal membrane oxygenation support in New South Wales, Australia.

New South Wales has recently added the capability of extracorporeal membrane oxygenation to the neonatal and paediatric retrieval process and this paper describes the early experiences and protocol development for the first eight cases transported.

Improving diagnostic accuracy in neonates with left heart obstruction in a transport setting.

AIM: Differentiating left heart obstruction (LHO) from other severe illness in the neonatal period is challenging, and important for guiding clinical management. The aim of this study was to identify factors distinguishing LHO from non-LHO in neonates. METHODS: A retrospective, cohort study of neonates referred to the Newborn and Paediatric Emergency Transport Service, New South Wales, with suspected LHO during the epoch 1996-2016. RESULTS: A total of 273 neonates were included; 240 with confirmed LHO. Administration of prostaglandin E1 to infants with a structurally normal heart was not associated with impaired acid-base or oxygenation status. Pre-transport diagnostic accuracy of LHO was 74.4%; sensitivity 84.5%, positive predictive value 86.0%. On multivariable logistic regression, hepatomegaly (odds ratio 2.54; 95% confidence interval 1.05-6.16) was associated with confirmed LHO. CONCLUSIONS: A low threshold for prostaglandin E1 infusion should be maintained in infants with suspected LHO. Hepatomegaly is associated with a diagnosis of LHO and may be more useful than other parameters in predicting the condition.

Neurocritical care of high-risk infants during inter-hospital transport.

The centralisation of neonatal intensive care in recent years has improved mortality, particularly of extremely preterm infants, but similar improvements in morbidity, such as neurodevelopmental impairment, have not been seen. Integral to the success of centralisation are specialised neonatal transport teams who provide intensive care prior to and during retrieval of high-risk neonates when in-utero transfer has not been possible. Neonatal retrieval aims to stabilise the clinical condition and then transfer the neonate during a high-risk period for patient. Transport introduces the hazards of noise and vibration; acceleration and deceleration forces; additional handling and temperature fluctuations. The transport team must stabilise the infant fully prior to transport as when on the move they are limited by space and movement to effectively attend to clinical deterioration. Inborn infants have better neurodevelopmental outcome compared with the outborn and aetiology of this seems to be multifactorial with the impact of transport itself during critical illness, remaining unclear. To improve the neurological outcomes for transported infants, it seems imperative to integrate the advancing intensive care neuromonitoring tools into the transport milieu. This review examines current inter-hospital transport neuromonitoring and how new modalities might be applied to the neurocritical care delivered by specialist transport teams.

Sail sign in neonatal pneumomediastinum: a case report.

BACKGROUND: Pneumomediastinum is an uncommon cause of neonatal respiratory distress. Clinical history and examination of the neonate may be uninformative in determining the aetiology of the respiratory distress. Chest x-ray can be diagnostic of pneumomediastinum however is often difficult to interpret. CASE PRESENTATION: A 36 week gestation newborn was delivered by emergency caesarean section, intubated and given intermittent positive pressure ventilation via Neopuff™ for apnoea before being extubated to CPAP. Chest radiography initially showed bilateral upper zone opacities, presenting a diagnostic dilemma; however subsequent films demonstrated a pneumomediastinum which was managed conservatively and resolved. CONCLUSIONS: The "sail sign" describes an uncommon radiological appearance of a pneumomediastinum in neonates and infants. With careful conservative management, a spontaneous resolution without longterm sequelae can be expected.

Feasibility and utility of portable ultrasound during retrieval of sick preterm infants.

AIM: Document the incidence of haemodynamic pathology in critically ill preterm newborns requiring transport. METHOD: A transport neonatologist performed cardiac and cerebral ultrasound before and after transportation of infants born ≤30 weeks gestation. RESULTS: Forty-four newborns were studied in 2008-2015; of them, 21 were transported by road, 19, by helicopter and four, by fixed wing: median birthweight, 1130 g (680-1960 g) and median gestation, 27 weeks (23-30); 30 of 44 were male babies. Antenatal steroid course was complete in two babies. Ultrasound in the referring hospital was at a mean of two hours: 47 minutes (00:15-7:00) of age. Low systemic blood flow was common: 50% had right ventricular output <150mL/kg/min and 23%, a superior vena cava flow <50mL/kg/min. at stabilisation. Cranial US: 10 Grade I IVH, 2 Grade II IVH, 1 Grade IV IVH and 32 normal scans pretransport. After transport, three further Grade I IVH were reported. Mortality was higher in the babies with low systemic blood flow: 4 of 12 (33%) died vs 1 of 31 (6%) in the normal flow group (OR = 7.2, 95% CI: 1.1 to 47, p = 0.022). CONCLUSION: Point-of-care ultrasound during the retrieval of preterm infants confirms a high incidence of haemodynamic pathology. The use of ultrasound during transport may provide an opportunity for earlier targeted circulatory support.

Feasibility and utility of portable ultrasound during retrieval of sick term and late preterm infants.

AIM: To determine the role of clinician performed ultrasound (CPU) during the retrieval and transport of critically ill term and near term newborns. METHODS: A neonatologist with portable ultrasound accompanied a sample of newborn retrievals to perform cardiac and cerebral ultrasound before and after transportation. RESULTS: A total of fifty-five babies were studied. Median birthweight: 3350 g (2220-5030 g). CPU led to a change in the planned receiving hospital in ten babies. Eleven babies were suspected congenital heart disease (CHD) prior to retrieval: eight confirmed CHD by CPU and three normal structure. One transported to a children's hospital for cardiology review was confirmed as having normal structure; one to a perinatal hospital where normal structure was confirmed and one baby died at the referring hospital and postmortem confirmed normal structure. In five babies with clinical pulmonary hypertension, CPU revealed unsuspected CHD. The destination was changed to a paediatric cardiology centre, avoiding a second retrieval. Eleven babies had evidence of haemodynamic compromise allowing targeting of inotropes. CONCLUSION: This is the first study of CPU during retrieval of high-risk infants. Ultrasound in retrieval is feasible, allows accurate triage of babies to cardiac centres and may allow more accurate targeting of fluid and inotrope support.

Outcomes of high-risk obstetric transfers in New South Wales and the Australian Capital Territory: The High-Risk Obstetric Transfer Study.

BACKGROUND: In New South Wales and the Australian Capital Territory, in utero transfers to manage maternal or neonatal risks are highly challenging owing to geography and centralisation of tertiary perinatal care. AIMS: The study aims to document the outcomes of high-risk obstetric transfers. MATERIALS AND METHODS: A prospective observational study was conducted from 2010 to 2011 documenting urgent requests for obstetric transfers to tertiary centres across NSW/ACT for pregnancies 20 weeks' gestation or greater. Outcomes of transfers were allocated apriori to 'delivered at the receiving hospital', 'failed/delayed transfer' or 'discharged/transferred undelivered'. Our hypothesis is that each outcome has a specific group of associated clinical factors. RESULTS: Of the 249 transfer requests included in the study, 40% delivered at the receiving hospital, 7% were failed/delayed transfers, and 45% were discharged/transferred undelivered. Cases delivering at the receiving hospital were significantly associated with older mothers, twin pregnancies, pregnancy induced hypertension (PIH) or premature rupture of membranes (PROM) with/without threatened preterm labour (TPL) as the indications for transfer and having three indications for transfer. Cases that were discharged/transferred undelivered were significantly associated with singleton pregnancies, TPL and/or antepartum haemorrhage (APH) as the indication for transfer and having one indication for transfer. There were no significantly associated factors for failed/delayed transfers. CONCLUSIONS: The study confirms the hypothesis that particular transfer outcomes are associated with different factors. The findings also show that less than half of urgent obstetric transfers result in delivery at the receiving hospital, suggesting that there exists significant opportunities for further research into predicting preterm delivery, thereby improving the care of women with high-risk pregnancies.

Potentially avoidable neonatal retrievals in New South Wales: a retrospective analysis.

OBJECTIVE: To identify areas for improvement in outcomes in retrieved newborns by reviewing newborn retrieval activity and evaluating potentially avoidable retrievals from each referring hospital stratified by the level of service delivery over the study period. DESIGN: A retrospective analysis of newborn retrievals from 1 January 2006 to 31 December 2009. SETTING: Newborn and Paediatric Emergency Transport Service (NETS). PARTICIPANTS: Newborns less than 72 hours old retrieved by NETS in the study period. Each retrieval was classified as potentially avoidable, unavoidable or unclassified, based on predefined criteria. MAIN OUTCOME MEASURES: Newborn retrieval rates (per 10,000 live births) and potentially avoidable retrievals for each referring hospital level and overall. RESULTS: There were 2494 newborn retrievals over the study period, with an annual mean of 623 total and 30 potentially avoidable retrievals. There was a reduction in the potentially avoidable retrieval rate (per 10,000 live births) over the study period (from 3.9 in 2006 and 4.2 in 2007 to 2.2 in 2008 and 2.3 in 2009) despite an increase in the total retrieval rate over the same time. Discretionary caesarean, defined as elective (pre-labour) caesarean section without documented fetal or maternal indications before 39 completed weeks of gestation, accounted for two-thirds of the potentially avoidable retrievals. CONCLUSIONS: Potentially avoidable retrievals were a small but significant proportion and are becoming less frequent. Discretionary caesarean is the most common cause of potentially avoidable retrieval. Strict implementation of the elective caesarean section policy directive has the potential to reduce morbidity and the costs related to retrieval.

Transporting newborns with transposition of the great arteries.

AIMS: The aim of the study was to examine the prevalence and management of outborn babies with a post-natally confirmed diagnosis of transposition of the great arteries (TGA) requiring transport by the Newborn and Paediatric Emergency Transport Service (NETS), New South Wales during the epoch 1991-2010. METHOD: A retrospective audit of NETS database and case notes. The physiological status, interventions and any complications encountered from the point of referral to NETS (pre-transport), stabilisation (transport) and subsequent admission to the receiving hospital (post-transport) were evaluated. RESULTS: One hundred fifty-seven infants with TGA were transported, with an average of eight per year (1:11,598 births). Seven (4%) had an antenatal diagnosis, and 72 (46%) had a post-natal diagnosis prior to referral. Physiological and clinical parameters demonstrated overall clinical stability; however, 47% of the babies had a PaO(2) <30 mmHg, and approximately one-fifth had oxygen saturations <70%. Rates of mechanical ventilation and prostaglandin E(1) administration were approximately 50%. A quarter of transported babies encountered a transport-related event, including one death and two babies for which a decision was reached to forego life-sustaining treatment at the referring hospital. CONCLUSIONS: Most newborns with TGA remain stable or improve during transport. There is a rate of adverse events; however, this reinforces the need to facilitate delivery where there is ready access to interventional paediatric cardiology services.

Does the enteral feeding advancement affect short-term outcomes in very low birth weight infants?

BACKGROUND AND OBJECTIVES: Controversy exists regarding the optimal enteral feeding regimen of very low birth weight infants (VLBW). Rapid advancement of enteral feeding has been associated with an increased rate of necrotizing enterocolitis. In contrast, delaying enteral feeding may have unfavorable effects on nutrition, growth, and neurodevelopment. The aim is to compare the short-term outcomes of VLBW infants in tertiary care centers according to their enteral feeding advancement. PATIENTS AND METHODS: We prospectively studied the influence of center-specific enteral feeding advancement in 1430 VLBW infants recruited from 13 tertiary neonatal intensive care units in Germany on short-term outcome parameters. The centers were post hoc stratified to "rapid advancement to full enteral feeds" (median duration of advancement to full enteral feeds < or =12.5 days; 6 centers), that is, rapid advancement (RA), or "slow advancement to full enteral feeds" (median duration of advancement to full enteral feeds >12.5 days; 7 centers), that is, slow advancement (SA). RESULTS: VLBW infants born in centers with SA (n = 713) had a significantly higher rate of sepsis compared with VLBW infants born in centers with RA (n = 717), which was particularly evident for late-onset sepsis (14.0% vs 20.4%; P = 0.002). Furthermore, more central venous lines (48.6% vs 31.1%, P < 0.001) and antibiotics (92.4% vs 77.7%, P < 0.001) were used in centers with SA. CONCLUSIONS: Center differences in enteral feeding advancement occur and may have a significant impact on short-term outcomes such as nosocomial sepsis. Large, multicenter, prospective trials are required to further elucidate the optimal feeding strategy for VLBW infants.

Stabilising the newborn for transfer - basic principles.

BACKGROUND: Rural general practitioners involved in obstetric service delivery may have occasion to support the sick or premature newborn requiring transfer. This should be achievable for short periods of time in most rural hospitals. OBJECTIVE: This article discusses the planning priorities, and the equipment and skills required for care of the sick or premature newborn. DISCUSSION: With careful planning, attention to detail, and maintenance of clinical skills and equipment, newborn intensive care can be provided in most hospitals for brief periods while awaiting the arrival of the retrieval team.

A quality audit of the service delivered by the NSW Neonatal and Paediatric Transport Service.

AIM: To discover areas of NSW Neonatal and Paediatric Transport Service's (NETS) work with which the parents, referring and receiving doctors are dissatisfied and respond to them. METHODS: An anonymous survey of referring doctors, parents of patients transported by NETS and receiving hospital doctors between July and December 2005. RESULTS: Referring doctors: Fifty-seven per cent of the 288 (30% response rate) doctors who responded were paediatricians and 43% worked in rural settings. Over 90% responded positively about communication with the NETS team at referral and retrieval. Useful feedback included the need to be more time efficient in phone communication and during stabilisation of the child and to improve feedback about management and patient outcomes. Parents: Forty-seven per cent of 152 responses (15% response rate) came from rural families. The majority (>98%) of parents felt that the NETS team were helpful and supportive of them. Parents reported being able to travel with their child 60% of the time and of those who could not, 95% could explain why. Receiving doctors: Ninety-three per cent of 218 responses (42% response rate) thought that the referral was appropriate, that the NETS teams carried out their advice correctly (98%) and that the child's needs were reported accurately by the team (90%). In a minority of retrievals important concerns were raised about ventilation, sedation, patient assessment and management. CONCLUSION: Most retrievals happen in a way that referring consultants, parents and receiving consultants find appropriate. Important suggestions for improvement in service delivery and some areas of risk to patient safety have been identified. Processes for overcoming these situations are being developed and implemented.