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Aim: To describe the major congenital anomalies present in children with postneonatally acquired cerebral palsy (CP), and to compare clinical outcomes and cause of postneonatally acquired CP between children with and without anomalies., Method: Data were linked between total population CP and congenital anomaly registers in five European and three Australian regions for children born 1991 to 2009 (n=468 children with postneonatally acquired CP; 255 males, 213 females). Data were pooled and children classified into mutually exclusive categories based on type of congenital anomaly. The proportion of children with congenital anomalies was calculated. Clinical outcomes and cause of postneonatally acquired CP were compared between children with and without anomalies., Results: Major congenital anomalies were reported in 25.6% (95% confidence interval [CI] 21.7-29.9) of children with postneonatally acquired CP. Cardiac anomalies, often severe, were common and present in 14.5% of children with postneonatally acquired CP. Clinical outcomes were not more severe in children with congenital anomalies than those without anomalies. Cause of postneonatally acquired CP differed with the presence of congenital anomalies, with cerebrovascular accidents predominating in the anomaly group. Congenital anomalies were likely associated with cause of postneonatally acquired CP in 77% of children with anomalies., Interpretation: In this large, international study of children with postneonatally acquired CP, congenital anomalies (particularly cardiac anomalies) were common. Future research should determine specific causal pathways to postneonatally acquired CP that include congenital anomalies to identify opportunities for prevention., What This Paper Adds: One-quarter of children with postneonatally acquired cerebral palsy (CP) have a major congenital anomaly. Cardiac anomalies, often severe, are the most common anomalies. Causes of postneonatally acquired CP differ between children with and without congenital anomalies., (© 2021 Mac Keith Press.)

Aim: To describe the frequency and types of major congenital anomalies present in children with pre- or perinatally acquired cerebral palsy (CP), and compare clinical outcomes for children with and without anomalies., Method: This multi-centre total population collaborative study between Surveillance of Cerebral Palsy in Europe, Australian Cerebral Palsy Register, and European Surveillance of Congenital Anomalies (EUROCAT) involved six European and three Australian regions. Data were linked between each region's CP and congenital anomaly register for children born between 1991 and 2009, and then pooled. Children were classified into mutually exclusive categories based on type of anomaly. Proportions of children with congenital anomalies were calculated, and clinical outcomes compared between children with and without anomalies., Results: Of 8201 children with CP, 22.8% (95% confidence interval [CI] 21.9, 23.8) had a major congenital anomaly. Isolated cerebral anomalies were most common (45.2%), with a further 8.6% having both cerebral and non-cerebral anomalies. Cardiac anomalies only were described in 10.5% of children and anomalies associated with syndromes were also reported: genetic (8.0%), chromosomal (5.7%), and teratogenic (3.0%). Clinical outcomes were more severe for children with CP and congenital anomalies, particularly cerebral anomalies., Interpretation: This large, international study reports major congenital anomalies in nearly one-quarter of children with pre- or perinatally acquired CP. Future research must focus on aetiological pathways to CP that include specific patterns of congenital anomalies., What This Paper Adds: Congenital anomalies were reported in 23% of children with pre- or perinatally acquired cerebral palsy. A higher proportion of children born at or near term had anomalies. The most common type of anomalies were isolated cerebral anomalies. Clinical outcomes were more severe for children with congenital anomalies (particularly cerebral)., (© 2020 Mac Keith Press.)

Aim: To determine the proportion of children with cerebral palsy (CP) who had major congenital anomalies, describe the types of disorders, and report on the children's functional outcomes., Method: Data were extracted from the Bangladesh Cerebral Palsy Register (BCPR). Descriptive analyses were conducted on children with CP and major congenital anomalies. Odds ratios (ORs) with 95% confidence intervals (CIs) were calculated to measure the association between major congenital anomalies, clinical severity, and presence of comorbidities., Results: Between January 2015 and December 2016, 726 children with CP were newly registered with the BCPR (277 females, 449 males; mean age [SD] at registration 90mo [54mo], 4mo-18y). Seventy-eight children (11%) had a major congenital anomaly. Neurological (86%) and musculoskeletal congenital anomalies (10%) were the most common. Microcephaly was the most common congenital anomaly (83%). The odds of severe functional motor limitations (OR=2.4, 95% CI=1.9-2.9), epilepsy (OR=1.6, 95% CI=1.1-2.1), visual impairment (OR=2.6, 95% CI=2.0-3.2), presence of strabismus (OR=3.9, 95% CI=3.8-4.4), hearing (OR=1.2, 95% CI=0.6-1.9), speech (OR=5.4, 95% CI=4.6-6.2), and intellectual impairments (OR=2.3, 95% CI=1.8-2.8) were higher in children with congenital anomalies compared to children without., Interpretation: The proportion of children with major congenital anomalies in the BCPR (11%) was lower than that identified in higher-income countries. This may be because of differences in how congenital anomalies are diagnosed as well as the impact of survival bias. In Bangladesh, children with CP and major congenital anomalies are more likely to have severe functional motor limitations and associated comorbidities., What This Paper Adds: Eleven per cent of children with cerebral palsy (CP) in Bangladesh had major congenital anomalies. Neurological and musculoskeletal congenital anomalies were the most common. Severe functional motor limitations and associated comorbidities were more common in children presenting with CP and major congenital anomalies., (© 2019 Mac Keith Press.)

Aim: To determine survival of infants with major congenital anomalies (CA) and assess the effect of co-existing anomalies and gestational age., Methods: All liveborn infants with major CA born in New South Wales (NSW), Australia, 2004-2009 were identified from the NSW Register of Congenital Conditions. Deaths were identified via record linkage to death registrations and five-year survival was estimated using Kaplan-Meier methods., Results: There were 8521 liveborn infants with CA of whom 617 (7.2%) died within the first five years of life. Half of deaths occurred in the first week of life. The overall five-year survival rate was 92.8% (95%CI: 92.2-93.3) and 83.2% (95%CI: 79.0-87.4) for syndromes, 83.4% (95%CI: 80.9-85.9) for multiple, 85.1% (95%CI: 82.6-87.5) for chromosomal, 95.3% (95%CI: 94.8-95.8) for isolated and 96.2% (95%CI: 94.3-98.1) for non-Q chapter anomalies. Five-year survival for chromosomal, syndromes and sub-groups was higher for isolated compared with multiple anomalies ranging from 77.5% to 98.9% and 68.6% to 89.5%, respectively. Survival was lower for preterm (79.4%; 95%CI: 77.5-81.4) than for term infants (95.8%; 95%CI: 95.3-96.3)., Conclusion: Nine in ten infants with major CA survive up to five years, although there is variability in survival across CA groups. Survival of infants with major congenital anomalies has improved in recent years., (©2019 Foundation Acta Paediatrica. Published by John Wiley & Sons Ltd.)

Congenital anomalies are a strong risk factor for cerebral palsy, particularly for children born at term. This systematic review aimed to address gaps in our understanding of the association between congenital anomalies and cerebral palsy. Eight population-based studies (n = 10 081) were identified. Congenital anomalies were reported in 12% to 32% of children with pre/perinatal brain injury and 20% of children with postneonatal brain injury. Variation between studies included study cohort inclusion criteria and the definitions and classification of included anomalies. The most common cerebral anomalies were microcephaly and hydrocephaly, whereas circulatory system anomalies were the most common noncerebral anomalies. The proportion of congenital anomalies was higher in children born at term than preterm. Synthesizing the highest quality data published, this review identified that congenital anomalies are common in cerebral palsy. New collaborative research, addressing sources of variation, is vital to identify pathways to cerebral palsy that include specific congenital anomalies, and explore opportunities for prevention.

Aim: While there is evidence of parental needs in the neonatal intensive care unit (NICU), parents of newborns admitted for general surgery are an under-researched population. This study aimed to identify needs in parents of newborns admitted to the NICU for general surgery and whether health-care professionals meet these needs., Methods: This was a prospective cohort study of 111 parents (57% mothers) of newborns admitted to a surgical NICU for general surgery in Australia from January 2014 to September 2015. Parents completed the Neonatal Family Needs Inventory (NFNI), comprising 56 items in five subscales (Support, Comfort, Information, Proximity, Assurance) at admission and discharge, as well as the Social Desirability Scale (SDS). Data were analysed using parametric and non-parametric techniques., Results: At both admission and discharge, parents rated Assurance (M = 3.8, standard deviation (SD) = 0.24) needs as the most important, followed by Proximity (M = 3.6, SD = 0.32) and Information (M = 3.5, SD = 0.38). Mothers rated Assurance significantly more important than fathers (P < 0.02). Overall, parents' most important needs were having questions answered honestly (M = 3.96, SD = 0.19), seeing their infant frequently and knowing about the medical treatment (both M = 3.95, SD = 0.23). The 10 most important needs were met for more than 96% of parents, with no evidence of response bias., Conclusions: Reassurance is a priority need for parents in the surgical NICU. Mothers' and fathers' needs may be best met by practices based on family-centred, individualised care principles., (© 2018 Paediatrics and Child Health Division (The Royal Australasian College of Physicians).)

Introduction: Cerebral palsy (CP), an umbrella term for non-progressive conditions of cerebral origin resulting in motor impairments, is collectively the most common cause of physical disability in childhood. Cerebral and/or non-cerebral congenital anomalies are present in 15%-40% of children with CP. In order to identify effective prevention strategies for this substantial proportion of CP, a comprehensive understanding of the epidemiology of these congenital anomalies is required. International collaboration is needed, as previous attempts have fallen short due to a lack of power, since the anomalies are individually rare and CP comprises many clinical descriptions. The aim of this study is to generate new knowledge about the aetiologies of CP through a focused investigation into the role of congenital anomalies., Methods and Analysis: This collaborative, population-based data linkage study includes nine geographic regions (six in Europe, three in Australia) served by both congenital anomaly and CP registers. Register data for children with CP (both with and without congenital anomalies) and children with specific congenital anomalies (without CP) born between 1991 and 2009 will be linked and de-identified within each region. The resulting linked data sets will be quality assured, recoded, harmonised and then pooled into one data set. Analysis of the combined data set will include: frequencies/proportions of congenital anomalies and outcomes (type of CP, severity, impairments); descriptive analyses comparing timing of congenital anomaly development and brain injury/abnormality responsible for CP; ORs to calculate the odds of CP following a specific congenital anomaly; and identification of anomalies on causal pathways to CP., Ethics and Dissemination: Ethics approval for this collaborative study, The Comprehensive CA-CP Study, has been obtained from the Cerebral Palsy Alliance Human Research Ethics Committee (EC00402). Study findings will be disseminated at conferences and published in peer-reviewed journals, and recommendations will be made regarding the collection and classification of congenital anomaly data by CP registers., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2018. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ.)

Proportions of cases of cerebral palsy (CP) with congenital anomalies recorded in Australian CP registers range from 15% to 40%. The anomalies seen in CP are extremely variable. We have identified that CP registers often do not have quality data on congenital anomalies, necessitating linkage with congenital anomaly registers. However, a lack of unified processes and definitions in congenital anomaly registers and data collections means that linkages are complex, need to be carefully planned, and limitations acknowledged. Historically in CP research, congenital anomalies have been classified by International Classification of Disease codes, then combined into brain and other major and minor anomalies. Systems have been developed to classify congenital anomalies into aetiologically related groups, but such a classification has yet to be trialled in CP. It is anticipated that primary prevention of a small proportion of cases of CP is possible through the primary prevention of congenital anomalies, especially those due to teratogens. Owing to the anticipated low prevalence of each subgroup, global collaboration will be required to further these lines of enquiry., (© 2016 The Authors. Developmental Medicine & Child Neurology © 2016 Mac Keith Press.)

Aim: To describe neurodevelopmental outcomes of neonates following cardiac or non-cardiac surgery for major birth defects., Methods: From 1 June 2002 to 31 July 2004, infants born ≥ 33 weeks gestation who underwent major birth defect surgery were enrolled prospectively. Infants were assessed at a mean corrected age of 24 months (standard deviation (SD) = 8 months, range 18-36 months) using the Bayley Scales of Infant Development: Second Edition., Results: Of the 118 study infants, 79 (66%) were male, the mean gestation was 38.5 weeks (SD 1.9 weeks) and mean birthweight was 3194 g (SD 653 g). Forty-five infants (47%) had undergone general surgery for non-cardiac defects. The majority of infants (73%) performed below average in cognitive and language skills. Mental delay was found in 41% of infants; 16% were significantly delayed. Fine and gross motor skills were below average in 60% of infants. Twenty-six percent of infants had motor delay; 9% were significantly delayed. Both the mean Mental Development Index (M = 88, SD = 19.8) and mean Psychomotor Development Index (M = 93, SD = 19.3) were significantly below the normative mean (d = 0.8, P < 0.001 and d = 0.5, P < 0.001, respectively). One in five children had global developmental delay. There was no significant difference in outcome between the cardiac and general surgery groups., Conclusions: The majority of infants performed below average on a standardised test of infant development. Our results show that infants requiring newborn surgery for major birth defects are at high risk of adverse neurodevelopmental outcomes. We recommend that follow-up programmes include systematic multidisciplinary developmental monitoring and early intervention., (© 2010 The Authors. Journal of Paediatrics and Child Health © 2010 Paediatrics and Child Health Division (Royal Australasian College of Physicians).)

Background: Evidence exists of the important role of the mother-child relationship in child development, yet with the exception of prematurity, little is known of the impact of biologic risk on this relationship., Aims: We investigated the quality of the mother-child interaction in association with early development in toddlers who had newborn surgery for major birth defects., Methods: Ninety-three toddlers (Mean age=24.7 months, SD=4.6) requiring newborn surgery for major birth defects, underwent developmental testing and participated in a videotaped free play interaction with their mothers. Interactions were rated using the National Institute of Child Health and Human Development schema. Interaction ratings were compared with normative data and within group differences were also examined in relation to child developmental status., Results: Overall mean interaction ratings were suboptimal and significantly different from normative data; with the exception of child negative mood. Forty-one children (44%) had developmental delay. Mother and child interactive behaviour ratings were consolidated into three factors (Maternal Responsivity, Maternal Intrusiveness, and Child Positive Interactivity) for multivariate analyses. Compared to children developing normally, those with developmental delay showed significantly less positive interactivity and had mothers who were less responsive and more intrusive. Child behaviour with the tester was related to child behaviour with the mother., Conclusion: Quality of mother-child interaction is nonoptimal in children with major birth defects requiring newborn surgery. Mothers of infants with developmental delay are most at risk of interaction difficulties and may benefit from early identification and timely intervention., (Copyright © 2010 Elsevier Ltd. All rights reserved.)

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.

The objective of the study was to identify the origin (s) of the association between cerebral palsy (CP) and birth defects in the absence of cerebral birth defects. Data from the 1980 to 1994 Western Australian birth cohorts (355 659 neonatal survivors) were linked to the Cerebral Palsy Register (941 links) and the Birth Defects Registry (17070 links). Associations between CP (congenital or acquired) and birth defects (cerebral or exclusively non-cerebral) were estimated. The origin of the association between non-cerebral defects and acquired CP was investigated with an observational study, and the origin of the association between non-cerebral defects and congenital CP was investigated with a blinded case-control study of births with non-cerebral defects with or without CP. With non-cerebral defects, the odds ratio for CP was 4.8 (95% CI 3.1-7.4) if acquired and 4.7 (3.9-5.7) if congenital. For acquired CP, the association arose primarily as a result of cardiac defects. For congenital CP, the association arose partly from ascertainment bias and partly from defects known to be associated with cerebral defects (but not identified in these data). However, a significant portion remained unexplained. The presence of non-cerebral defects should heighten clinical alertness to the possibility of CP and of cerebral birth defects.