Neonatal encephalopathy in New Zealand: Demographics and clinical outcome.

AIM: To establish the incidence of moderate to severe neonatal encephalopathy (NE) in term infants from New Zealand and to document demographic characteristics and neonatal outcomes. METHODS: Cases were reported monthly via the New Zealand Paediatric Surveillance Unit (NZPSU). Data were collected from paediatricians for neonatal items and lead maternity carers for pregnancy and birth details. Term neonatal deaths in the Perinatal and Maternal Mortality Review Committee dataset that were because of hypoxia and/or neonatal deaths from hypoxic ischaemic encephalopathy were added to the cases identified via the NZPSU, if they had not previously been ascertained. RESULTS: For the period January 2010 to December 2012, there were 227 cases, equivalent to a rate of 1.30/1000 term births (95% CI 1.14-1.48). Rates of NE were high in babies of Pacific and Indian mothers but only reached statistical significance for the comparison between Pacific and NZ European. There was also a significant increase in NE rates with increasing deprivation. Resuscitation at birth was initiated for 209 (92.1%) infants with NE. Mechanical ventilation was required, following neonatal unit admission, in 171 (75.3%) infants. Anticonvulsants were used in 157 (69.2%) infants with phenobarbitone (65.6%), phenytoin (14.5%) and benzodiazapines (21.1%), the most common. Cooling was induced in 168 infants (74%) with 145 (86.3%) reported as commenced within a 6-h window. CONCLUSIONS: The rate of NE in New Zealand is consistent with reported international rates. Establishing antecedent factors for NE is an important part of improving care, which may inform strategic efforts to decrease rates of NE.

13q33.2 deletion: a rare cause of ambiguous genitalia in a male newborn with growth restriction.

UNLABELLED: 13q deletion is a rare cause of ambiguous genitalia in the male newborn, and can be associated with mental retardation of varying degree, retinoblastoma, and malformations of the brain, eye, genitourinary and gastrointestinal tract, depending on the level of the deletion. We present a male neonate with ambiguous genitalia and IUGR with a 13q33.2 deletion, and a paternal balanced translocation. Microarray analysis found the genes involved to be on chromosome 13 in the region 102989254bp-109214509bp. This deletion encompasses the EFNB2 gene, which has been implicated in genital malformations in 13q deletion cases. CONCLUSIONS: We find a link between haploinsufficiency of the EFNB2 gene and the presence of ambiguous genitalia and hypospadia in patients with a 13q.33 deletion. This work emphasizes the importance of early diagnosis of this condition due to the link with mental retardation and the need for follow up and management.

Neonatal intensive care utilization and neonatal outcome of infants born to women aged 40 years and over in New Zealand.

BACKGROUND: Increased maternal age is associated with pregnancy complications and there are few data available on neonatal outcome and utilization of neonatal resources. Our first aim was to use national New Zealand data to determine if the outcomes following admission to NICU are different for infants born to women aged 40 years and over, compared with those born to women under 40 years of age. The second aim was to document trends in the requirement of neonatal intensive care in infants born to women aged 40 years and older. METHOD: Eligible infants were identified from registration with the Australian and New Zealand Neonatal Network for 1995-2004 inclusive. The relationship between maternal age and neonatal outcome was tested using univariate and multivariate analysis, and trends in the number of infants in maternal age groups below 35 years, 35-39 years and over 40 years were determined. RESULTS: On multivariate analysis using logistic regression, maternal age over 40 years was not found to be associated with a significant increase in the odds ratio for the composite poor outcome. However, over the 10-year period, there was an increase in the number of admissions and the percentage of admissions of infants born to women over 40 years of age. CONCLUSION: Although the number of infants admitted for neonatal care following birth to women over 40 years of age has increased, these infants do not appear to have an increased risk of severe abnormal outcome.

Chest radiographs after removal of chest drains in neonates: clinical benefit or common practice?

BACKGROUND: Chest drain insertion is a common procedure in neonatal care. Routine radiography after removal of chest drains increases radiation exposure, handling and cost, but there are few data proving clinical benefit. OBJECTIVES: To review current practice and determine the yield of routinely obtained chest radiographs (CXR). METHODS: A retrospective chart review of all infants undergoing removal of chest tubes in a single tertiary neonatal unit in New Zealand between January 1998 and July 2004 was performed. RESULTS: In total, 119 infants were identified, from the database, to have a chest drainage performed. In 19 cases, the procedure was needle aspiration or the drain was removed outside of our unit, hence these were excluded. The remaining 100 patients with 110 episodes of chest drain removal after 174 chest tube insertions were analysed. In asymptomatic infants, routine radiography showed some reaccumulation of air in nine of 35 cases of pneumothorax or of fluid in two of the five cases of pleural effusion, but chest tube reinsertion was not required. In the 12 clinically symptomatic infants, chest tubes were reinserted in five cases (four reaccumulations of pneumothorax and one pleural effusion), and one infant had symptomatic right upper lobe collapse. In the remaining infants, there were no abnormalities on CXR accounting for deterioration. CONCLUSIONS: Given the low yield for routine radiography after chest drain removal, we suggest that close observation is likely to detect clinically relevant recurrence of pneumothorax.

Exposure to repeat doses of antenatal glucocorticoids is associated with altered cardiovascular status after birth.

OBJECTIVE: To determine if exposure to more than one course of antenatal glucocorticoids is associated with changes in infant blood pressure and myocardial wall thickness in the first month after birth. DESIGN: Prospective cohort study. SETTING: Tertiary neonatal intensive care unit. PARTICIPANTS: Mothers who were eligible for but declined to enter a randomised trial of repeated doses of antenatal glucocorticoids (ACTORDS)-that is, who had a singleton, twin, or triplet pregnancy at <32 weeks gestation, had received an initial course of glucocorticoids seven or more days previously, and were considered to be at continued risk of preterm birth. MAIN OUTCOME MEASURES: Blood pressure daily for the first week then weekly until 4 weeks of age. End diastolic interventricular septal and left ventricular posterior wall (EDIVS and EDLVPW) thickness at 48-72 hours after birth. RESULTS: Thirty seven women were enrolled and delivered 50 infants. Thirty mothers (39 infants) were exposed to one course of glucocorticoids, and seven mothers (11 infants) to more than one course. Blood pressures were higher in the first week after birth in infants exposed to multiple courses of glucocorticoids, and in infants with a latency between last exposure and delivery of less than seven days. Systolic blood pressure on day 1 was >2SD above published normal ranges in 67% of babies exposed to multiple courses and 24% of babies exposed to a single course of glucocorticoids (p = 0.04). There was no difference between groups in thickness of the EDIVS or EDLVPW. However, 44/50 (88%) babies had EDIVS and 49/50 (98%) babies had EDLVPW thickness >2 SD above the expected mean for birth weight and gestation. EDIVS but not EDLVPW thickness increased with increasing latency (mean 0.02 mm/day, p = 0.03). CONCLUSION: Future randomised trials should assess the long term effects of exposure to antenatal glucocorticoids, particularly multiple courses, on the cardiovascular status of the infant.

Variation in identifying neonatal percutaneous central venous line position.

OBJECTIVE: The study objective was to obtain data on interpretation, including intra and interobserver variation and action taken for a given line tip location, for a series of radiographs demonstrating neonatal long lines. METHODS: Nineteen radiographs taken to identify line tip position were digitized and published on an internet site. One film was included twice in order to assess intraobserver variation giving a total of 20 images. Fourteen used radio-opaque contrast and five no contrast. Australian and New Zealand Neonatal Network members and National Women's Hospital NICU staff were invited to participate in the study. For each radiograph, participants were asked to identify if long line tip could be identified, the likely anatomical position and desired action. Interobserver agreement was assessed by the maximum proportion of agreement per radiograph and by the number of different options selected. Intraobserver agreement was assessed by comparing the two reports from the duplicate radiograph. RESULTS: Twenty-seven responses were received. Overall, 50% of the reports stated that the long line tips could be identified. The most commonly reported position was in the right atrium (31%) and most commonly reported action was to pull the line back (53%). The median agreement of whether the line was seen was 68%, agreement on position 62% and agreement on action 86%. On analysis of intraobserver variability, from the identical radiographs, 27% of respondents differed on whether the line tip could be visualized. CONCLUSION: Interobserver and intraobserver reliability was poor when using radiographs to assess long line tips. The major determinant of line repositioning was the perceived location.

Does radio-opaque contrast improve radiographic localisation of percutaneous central venous lines?

BACKGROUND: Percutaneous central venous lines (long lines) are commonly used in neonatal practice. The position of these lines is important, because incorrect placement may be associated with complications. AIMS: To determine whether the addition of radio-opaque contrast material improves the localisation of long line tips over plain radiography. METHODS: Radiographs taken to identify long line position were identified in two periods; 106 radiographs without contrast taken between October 1999 and August 2000, and 96 radiographs with contrast between September 2001 and July 2002. Two observers independently reviewed each radiograph to identify the position of the line tip. The formal radiology report was recorded as a third observer. RESULTS: The use of contrast increased the proportion of radiographs in which all observers reported they could see the long line tip (53 (55%) v 41 (39%)). It also increased the proportion where they agreed on anatomical position (57 (59%) v 39 (37%)) and there was a higher kappa coefficient for agreement (0.56 v 0.33). CONCLUSIONS: The use of contrast while taking radiographs for the localisation of long line position improves the likelihood that an observer can see a long line tip and reduces inter-observer variability. Even using contrast, precise localisation of a long line tip can be difficult.

Primary pulmonary hypoplasia: a case report and review of the literature.

A case of primary pulmonary hypoplasia in a term female neonate presenting with severe respiratory distress at birth is reported. Respiratory failure persisted and she died at 12 days of age. Primary pulmonary hypoplasia is a rare condition not associated with other maternal or fetal disorders.

Staphylococcus aureus endocarditis in preterm neonates.

This article describes three extremely low birth weight infants with Staphylococcus aureus septicemia associated with insertion of a percutaneous central venous catheter who later developed endocarditis. Echocardiography demonstrated large vegetations although only one infant had a murmur. Following a 6-week course of intravenous flucloxacillin and netilmicin, the endocarditis completely resolved and further intervention was unnecessary, although one baby died later as a result of volvulus and chronic lung disease. Echocardiography should be performed to exclude invasive infection in infants with S. aureus septicemia even when there is no murmur or other evidence of endocarditis. If endocarditis is identified, a good outcome is possible with appropriate aggressive antibiotic therapy.

Intrauterine T-cell activation and increased proinflammatory cytokine concentrations in preterm infants with cerebral lesions.

Brain injury is common in very preterm infants, and intrauterine infection is a frequent antecedent of preterm birth. We examined the relation of cerebral damage to intrauterine antigen exposure and inflammation in 50 infants who were born at 23-29 weeks' gestation. Higher concentrations of cytokines (tumour necrosis factor alpha [TNF-alpha], and interleukins [IL], 1beta, 6, and 10) and CD45RO(+) T lymphocytes in umbilical blood predicted cerebral lesions detected by magnetic resonance imaging very soon after delivery. Our results suggest that infants who mount an immune response in utero are at higher risk of cerebral lesions.

Outcome of infants receiving in-utero transfusions for haemolytic disease.

AIMS: To review the outcome of infants who received in-utero transfusion (IUT) at National Women's Hospital (NWH). Specifically to determine postnatal exchange and top-up transfusion requirements, to examine perinatal factors useful for predicting this and review neurodevelopmental status of recipients. METHODS: A review was undertaken of infants delivered at NWH between 1991 and 2000, who had received one or more IUT for haemolytc disease. The mean gestational age at birth was 34 weeks and birth weight 2298 g. The median number of IUT was four (range 1-9). The mean gestational age at the first IUT was 25 weeks and at last IUT was 31 weeks. RESULTS: All 37 infants survived to discharge. Postnatal exchange transfusion was required in ten and top up transfusion in 27 infants. Late transfusion (>3 weeks) was required in 21 infants. Follow-up information on 36 infants revealed one born prematurely following death of a co-twin had cerebral palsy and developmental delay. No other infant had audiology or neurodevelopmental abnormality. CONCLUSIONS: This review helps to confirm the good outcome following IUT although some infants continue to require transfusions for up to four months after birth.

Neurodevelopmental outcome of infants treated with head cooling and mild hypothermia after perinatal asphyxia.

OBJECTIVES: To determine the neurodevelopmental outcome of infants treated with head cooling with systemic hypothermia after hypoxic-ischemic encephalopathy. STUDY DESIGN: Infants >/=37 weeks' gestation, who had an umbilical artery pH

Chest physiotherapy and porencephalic brain lesions in very preterm infants.

OBJECTIVE: National Women's Hospital is one of two hospitals to report a destructive brain lesion, namely encephaloclastic porencephaly (ECPE), in extremely preterm infants. It has been associated with non-cephalic presentation, early hypotension and the number of chest physiotherapy treatments in the first month. The aim of the present study was to determine the temporal relationship between ECPE and chest physiotherapy use in very low-birth weight (VLBW) infants in our unit. METHODOLOGY: Cerebral ultrasound scan reports, post-mortem reports, clinical and physiotherapy records and, if indicated, original ultrasound films were reviewed for all VLBW babies admitted between 1985 and 1998. RESULTS: Over the 14 year period in question, 2219 babies with a birth weight < or = 1500 g were admitted. Encephaloclastic porencephaly was found in only the 13 previously reported babies born between 1992 and 1994. Encephaloclastic porencephaly was excluded in 1564 (70%) babies. In 621 (28%) babies who did not have late ultrasound scans, ECPE was thought to be unlikely either because the babies never had any chest physiotherapy (n=479) or because they had chest physiotherapy but were known to be neurodevelopmentally normal on follow up (n=142). Data were incomplete for 21 babies (0.9%). The number of chest physiotherapy treatments per baby decreased from a median of 95 prior to 1989 to 38 and the age of starting treatment increased from 5 to 8 days after 1990. The use of chest physiotherapy ceased in 1995. CONCLUSIONS: Encephaloclastic porencephaly emerged as a problem at a time when the use of chest physiotherapy had decreased. The cluster of cases seen between 1992 and 1994, although associated with the number of chest physiotherapy treatments given, began to appear because of some other factor.

Pervasive seizures caused by hypoxic-ischemic encephalopathy: treatment with intravenous paraldehyde.

Seizures are commonly associated with hypoxic-ischemic encephalopathy. Although the majority of cases are controlled with first- or second-line therapy, others develop pervasive seizures, requiring multiple anticonvulsants. To provide data on the incidence of seizures and response to anticonvulsant therapies, a cohort of 90 term infants with hypoxic-ischemic encephalopathy treated at our institution between January 1, 1995, and July 1, 1999, was reviewed. Of the 60 infants who developed seizures, 59 received phenobarbital initially; in 29 cases, the seizures resolved. The remaining 30 infants received phenytoin as a second-line anticonvulsant, and seizures stopped in 10 cases. The 20 infants with ongoing pervasive seizures were treated with intravenous paraldehyde.