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Aim: To describe the distribution of neuroimaging patterns in a term/late preterm population‐based cohort with cerebral palsy (CP), ascertain associations between neuroimaging patterns and neonatal well‐being, estimate the proportion with antenatal or perinatal timing of neuropathology, and apply this information to the understanding of common mechanisms of brain injury and causal pathways. Method: The cohort for this observational study comprised 1348 persons born between 1999 and 2017 in Victoria, Australia. Using algorithms designed for the study, neonatal well‐being and timing of brain injury were tabulated for the whole cohort and across neuroimaging patterns and birth epochs. Results: Clinical and demographic profiles, neonatal well‐being, and timing of brain injury differed across neuroimaging patterns. An estimated 57% of the cohort had a complicated neonatal period. Timing of brain injury was antenatal in 57% and perinatal in 41%. A decrease in the relative proportions of perinatal timing of brain injury was observed over a period when the rates of CP in live births at term decreased. Interpretation: This study begins to bridge the knowledge gap about causation in CP, moving towards better description of the main mechanisms of brain injury and their contribution within CP cohorts, and facilitating the ability to monitor changes over time and the success of preventive measures. What this paper adds: In a population‐based, term/late preterm cohort with cerebral palsy, 57% had a complicated neonatal period.In the same cohort, 57% had presumed antenatal timing of brain injury.The relative proportion with perinatal injury decreased over time. What this paper adds: In a population‐based, term/late preterm cohort with cerebral palsy, 57% had a complicated neonatal period.In the same cohort, 57% had presumed antenatal timing of brain injury.The relative proportion with perinatal injury decreased over time. This original article is commented on by O'Shea on pages 829–830 of this issue. [ABSTRACT FROM AUTHOR]

Background: Fetoscopic laser coagulation of placental anastomoses reverses the pathological process in twin-to-twin transfusion syndrome, thereby increasing survival, but there are a paucity of studies addressing long-term neurodevelopmental outcome of survivors. This study aimed to ascertain the presence of neurodevelopmental disabilities in child survivors of monochorionic pregnancies managed by placental laser photocoagulation in the Australian state of Victoria. Methods: All pregnancies undergoing placental laser photocoagulation with the Victorian Fetal Therapy Service between 2006–2017 were included. Information on each surviving child, including demographics, perinatal course, and developmental progress was collected from parents, and consent was sought to complete the Child Behaviour Checklist. Interviewers evaluated whether this information was consistent with a diagnosis of any of 14 neurodevelopmental conditions. A three-tiered outcome measure was allocated for each child: (1) unimpaired or developmentally normal, (2) mild or moderate neurological impairment, or (3) severe neurological impairment. Clinical predictors for adverse outcome were identified. Results: Of 116 pregnancies (113 twin, 3 triplet), 96 (83%) resulted in 1 + surviving fetuses. 57/113 (50%) twin pregnancies resulted in 2 survivors, 36 (32%) in 1 survivor, and 20 (18%) in no survivors. Of the 235 fetuses, 154 (65.5%) survived to follow-up. Survival increased from 59% in 2006–2008 to 73% in 2015–2017. 90/154 (58%) survivors were followed up at a mean age of 7.5 [SD 3.0] years. Based on parental interview and Child Behaviour Checklist data, 28/90 (31%) participants were assessed as having neurodevelopmental impairment, 27 of mild-moderate severity and 1 severe. Speech/language disorders, attention deficit (hyperactivity) disorders, and fine motor impairment were most common. Neonatal length of stay conferred the highest risk of impairment. Conclusion: Substantial variation exists between fetal therapy services in the type and length of neonatal follow-up following fetoscopic laser coagulation, contributing to a lack of data on long-term outcomes. The findings from this study support increasingly urgent calls to undertake systematic and sustained follow-up of fetoscopic laser coagulation survivors until school age. Information from this study may assist parents in their decision-making when offered fetal surgery. Importantly, it highlights a group for targeted surveillance and early intervention. [ABSTRACT FROM AUTHOR]

Aim: Males typically outnumber females in cerebral palsy (CP) cohorts. To better understand this 'male disadvantage' and provide insight into causal pathways to CP, this study used 1983 to 2009 Australian CP and population birth cohorts to identify associations and trends with respect to biological sex and CP.Method: Within birth gestation groups, sex ratios were calculated to evaluate any male excess in the CP cohort compared with livebirths, neonatal deaths, neonatal mortality and survival rates, neonatal survivors, and CP rates in survivors. Sex- and gestation-specific trends in neonatal mortality, CP rates, and CP sex ratios were assessed by plotting their annual frequencies and fitting quadratic curves.Results: Over-representation of males in preterm live births partly explained the male excess in the CP cohort after preterm birth, especially at 28 to 31 weeks. Higher CP rates in male neonatal survivors also contributed to the male excess in CP, particularly at <28 and 37+ weeks. Higher neonatal mortality rates in males at all gestations had little impact on the CP sex ratio. There was no clearly discernible change over time in the CP sex ratio.Interpretation: Gestation-specific associations between sex and CP provide insight into causal pathways to CP and suggest sex-specific differences in response to neuroprotective strategies. [ABSTRACT FROM AUTHOR]

Aim To establish a prevalence estimate for drooling and explore factors associated with drooling in a population sample of children with cerebral palsy (CP) aged 7 to 14 years living in Victoria, Australia. Method A self-report questionnaire was used to collect data on drooling from parents of children born between 1996 and 2001, and registered with the Victorian Cerebral Palsy Register. Results A total of 385 children (231 males, 154 females; mean age 10y 9mo [SD 1y 7mo], range 8-14y) were studied. The clinical type and distribution of CP were spastic (341), ataxic (16), dyskinetic (17), hypotonic (10), and unknown (1). Distribution in Gross Motor Function Classification System (GMFCS) levels was I (103), II (98), III (52), IV (63), V (61), and unknown (8). After adjustment for topographical pattern of motor impairment and GMFCS level, 40% were reported to have experienced drooling between 4 years of age and the time of completing the questionnaire. A significantly higher prevalence of drooling was found in children with poor gross motor function and in those with more severe presentations of CP, including poor head control, difficulty with eating, and inability to sustain lip closure ( p<0.001 for each). Drooling was shown to be significantly associated with both intellectual disability and epilepsy in this group of children ( p<0.001 for both). Interpretation With a prevalence of 40%, drooling is an important comorbidity in CP. It was considered severe in 15% of children. Poor oromotor function was associated with drooling and could be the target of interventions for this under-researched problem. [ABSTRACT FROM AUTHOR]

Aim: The aim of this study was to describe the rates, predictors and causes of mortality in a population sample of individuals with Prader-Willi syndrome (PWS). Methods: One hundred sixty-three individuals with PWS (90 males and 73 females, ages: 3 weeks to 60 years) were identified from the Victorian PWS Register. Information on demographics, age at diagnosis, genetic mechanism, age at which obesity developed and last known body mass index measurement were extracted. Notification and causes of death were obtained through linkage with Australian national and state of Victoria death indexes. Survival analysis was used to estimate the probability of survival and the effect of obesity on survival. Mortality rate ratios were calculated to investigate the effect of the factors listed above on mortality. Results: Fifteen deaths were recorded (nine males and six females), corresponding to an 87% probability of survival to 35 years. The probability of survival was significantly lower for individuals with known obesity ( P= 0.03), but there was no strong evidence for an effect on survival for the other factors studied. Cardiac or respiratory conditions were common causes of death after the age of 15 years. Conclusions: The effect of known obesity on the probability of survival and the causes of death reported in this and other studies suggest an important association between obesity and early death in adults with PWS. This finding highlights the critical nature of preventative and intervention strategies aimed at minimising the effects of hyperphagia in individuals with PWS. [ABSTRACT FROM AUTHOR]

Aim The aim of this study was to assess overall and gestational age-specific trends in the rate of cerebral palsy (CP) in Victoria, Australia, and to compare these findings with other population data. Method Individuals born in Victoria from 1970 to 2004 with non-postneonatally acquired CP were identified from a population register; 3491 were included in the study (1963 males, 1528 females). After a literature review, comparison data were extracted from publications using previously devised inclusion criteria. Rates were calculated per 1000 live births for all CP and by gestational age group: these were tabulated and plotted by year of birth. Results Data from nine registries, including the Victorian register, showed an increase in the rates of CP over the 1970s and 1980s, consistently seen in extremely preterm (<28wks) survivors but also in those born at term (≥37wks). Since the early 1990s, CP rates either stabilized or decreased, particularly for children born extremely preterm. Interpretation Increases in the rates of CP during the 1970s and 1980s are in part because of the increasing survival of extremely preterm infants that occurred without a concomitant improvement in neurological outcomes. Evidence from population samples now suggests that this trend has been reversed since the mid- to late 1990s. [ABSTRACT FROM AUTHOR]

The aim of this study was to describe the distribution of types of motor disorder (motor type) in a population-based registry of children with cerebral palsy (CP), to examine any trends in motor type distribution over time, and to make comparisons with other populations. Individuals born in Victoria, Australia, between 1970 and 2003 with congenital CP were identified from the Victorian Cerebral Palsy Register, 3297 of whom were included in the study (1840 males, 1457 females). Those who had a hypotonic motor type, those whose brain abnormality was believed to have developed after 28 days of life, and those with missing data were excluded from the study. The proportions of each motor type were calculated and plotted by year of birth. Logistic regression analyses were used to assess the mean change in odds per year of birth. A systematic review of the international literature was undertaken and comparison data were extracted based on previously devised criteria. The degree of heterogeneity was assessed and comparisons were made according to geographical region and reporting period. In Victoria, the predominant motor types for individuals with CP born between 1970 and 2003 were spasticity (91%), ataxia (5%), and dyskinesia (4%). There was no clear trend for any motor type over time. The mean proportions from two other large data sets were similar but there was considerable heterogeneity between 29 individual CP registries, mainly because of differences in the classification of mixed motor types. A more objective method for determining the predominant motor type is required to make the classification of motor type in CP more consistent and reliable. [ABSTRACT FROM AUTHOR]

Aim: To use the information from a population-based cerebral palsy register to describe post-neonatally acquired cerebral palsy, to determine trends over time and relate any aetiological trends to community preventative measures. Methods: Data on cases of post-neonatally acquired cerebral palsy, between the birth years 1970 and 1999, were generated from the Victorian Cerebral Palsy Register. Distributions, rates and trends over time were calculated for the entire cohort and for subgroups according to gender, age at acquisition, aetiology, gestation at delivery, birthweight, maternal age at delivery and parity. Results: 339 cases were found with post-neonatally acquired cerebral palsy, accounting for 10.7% of all cerebral palsy and giving an overall rate of 1.98/10 000 live births. There was a statistically significant fall in the overall rate of post-neonatally acquired cerebral palsy ( P = 0.001) over the study period. Significant falls were seen in post-neonatal cerebral palsy due to infection, traumatic head injury and hypoxia and other acute encephalopathies, but not cerebrovascular accidents. Conclusion: A large proportion of post-neonatally acquired cerebral palsy is preventable. While the reported decline in cases in Victoria is encouraging, it will be important to monitor these trends over time, and continue public health measures to further reduce preventable causes. [ABSTRACT FROM AUTHOR]

Aim: To provide an updated description of the rates, trends, and predictors of mortality of individuals with cerebral palsy (CP), born in the Australian state of Victoria between 1970 and 2012. Method: Data were extracted for 4807 individuals (2091 females; 2716 males). The probability of survival to 30th June 2017 was calculated using the Kaplan–Meier method. Mortality rates were calculated per 1000 person‐years using age strata and compared with population mortality rates to produce mortality ratios. Cox proportional hazards regression was used to calculate hazard ratios for selected demographic and clinical characteristics and to estimate the effect of birth epoch on 15‐year survival. Results: There were 666 recorded deaths. Compared to the general population, mortality was higher for all persons with CP and highest for children aged 1 to 15 years (45–62 times). We observed 35% improvement in the probability of survival to 15 years for births in the 2000s relative to the 1970s (hazard ratio 0.65, 95% confidence interval [CI] 0.49, 0.86), but only 4% improvement for the subgroup with complex CP (hazard ratio 0.96, 95% CI 0.69, 1.33). Interpretation: The observed improvements in survival for those born in the 2000s is likely related predominantly to a proportional reduction in complex CP within the cohort. What this paper adds: Length of survival improved for Australians with cerebral palsy (CP) born this millennium.Improved survival was mainly because of a proportional reduction in complex CP.A small improvement in length of survival was seen for children with complex CP. [ABSTRACT FROM AUTHOR]

Aim: To describe post‐neonatally acquired (PNN) cerebral palsy (CP) in terms of temporal trends in prevalence, clinical and sociodemographic profiles, known causes and associations between causes, and sociodemographic variables. Method: Numerator data, a count of children with PNN‐CP confirmed at 5 years of age (n = 523), was drawn from two Australian state CP registers (birth years 1973–2012). Poisson regression was used to investigate temporal trends in the prevalence of PNN‐CP by 5‐year intervals, calculated per 10 000 live births. Using data from all state and territory Australian CP registers (n = 469), distributions of clinical characteristics, PNN‐CP causes, and sociodemographic factors were tabulated (birth years 1995–2012). χ2 and logistic regression analyses were used to assess associations between sociodemographic profile, Australian reference data, and known causes. Results: A significant temporal decline in PNN‐CP in Victoria (p = 0.047) and Western Australia (p = 0.033) was observed. The most common proximal causes of PNN‐CP were cerebrovascular accidents (34%, n = 158), infection (25%, n = 117), and non‐accidental injuries (12%, n = 58). Children born to teenage mothers, Aboriginal and/or Torres Strait Islander mothers, or children born in remote areas were over‐represented in this cohort compared with reference data (all p ≤ 0.001). Infectious causes were strongly associated with teenage motherhood (odds ratio 3.0 [95% confidence interval 1.1–8.2], p = 0.028) and remote living (odds ratio 4.5 [95% confidence interval 2.0–10.2], p < 0.001). Interpretation: Although prevalence of PNN‐CP has declined, the over‐representation of priority populations, and the relative severity of a condition that is largely preventable, suggest the need for more specific primary preventive measures and support. What this paper adds: Prevalence of post‐neonatally acquired (PNN) cerebral palsy (CP) in Australia significantly declined between 1973 and 2012.Cerebrovascular accidents are the most common proximal cause of PNN‐CP.Children born in remote areas are at greater risk of PNN‐CP. [ABSTRACT FROM AUTHOR]

Aim: The aim of this study was to describe the patterns of therapy service use for a sample of children and adolescents with cerebral palsy over a 1 year period and to identify factors associated with frequency of therapy and parental satisfaction with therapy frequency.Methods: Parents of 83 children completed a survey on their child's use of occupational therapy, physiotherapy and speech and language pathology services over the previous year. Participants were randomly selected from a sample stratified by age and Gross Motor Function Classification System (GMFCS) level.Results: During the year prior to survey completion, 83% of children had received occupational therapy, 88% had received physiotherapy and 60% had received speech and language pathology services. Frequency of therapy was higher for younger children (P < 0.01), those classified at GMFCS levels IV-V (P < 0.05) and those attending schools specifically for children with disabilities.Conclusions: Current structures for therapy service delivery for children with cerebral palsy are systems-based, and age-based funding systems and the organisation of services around the education system are preventing the delivery of needs-based therapy. Paediatricians that care for children and young people with cerebral palsy need to pay particular attention to those that may miss out on therapy due to age or school type, and support these families in accessing appropriate therapy. [ABSTRACT FROM AUTHOR]

Aim: To describe the characteristics of emergency department (ED) presentations due to complications from gastrostomy or gastrojejunal feeding tubes among children with cerebral palsy (CP), the complexity of complications and the management approaches taken.Methods: The Victorian CP Register was linked to the ED databases of Victoria's two tertiary paediatric hospitals, and data on presentations due to feeding tube complications were identified based on discharge diagnosis codes. Additional data on presentations were extracted from medical records.Results: Over 5 years, there were 234 ED presentations due to feeding tube-related complaints among a CP cohort (n = 2183). ED notes were located for 183 of the 234 presentations. The majority of presentations (90%) involved children with severe gross motor impairment. A total of 46% of presentations (n = 84) was triaged as lower urgency, and 68% (n = 124) took place between 08:00 am and 06:00 pm. The most common presenting complaint was tube dislodgement (n = 105; 70%). No investigations were recorded in the majority of cases, and in almost 90% of cases, the feeding tube was successfully replaced in the ED, usually by an ED physician (n = 74) and less frequently by a surgeon (n = 9), gastroenterologist (n = 2) or nurse (n = 8); 9% (n = 17) resulted in a hospital admission.Conclusions: Most ED presentations due to feeding tube complaints in children with CP are in children with severe gross motor impairment but are able to be managed in the ED. As such, it is likely that care givers and other health professionals could manage some of the complications experienced in primary health-care settings closer to home. [ABSTRACT FROM AUTHOR]

OBJECTIVE: The goal of this study was to describe the prevalence, syndromes, and evolution of seizure disorders in children with cerebral palsy (CP) due to white matter injury (WMI). METHODS: For this population-based cohort study, brain MRI scans and medical records were reviewed in children in the Victorian Cerebral Palsy Register born between 1999 and 2006 recorded as having WMI. Children were excluded if they had features of an undiagnosed syndrome, associated cortical malformation or injury, or no medical contact in the preceding year. Included were 166 children with CP and isolated WMI due to presumed vascular insufficiency or hemorrhage; 87 were born preterm. Seizure and CP details were obtained from medical records and interviews, and EEG recordings were reviewed. RESULTS: Forty-one children (25%) had seizures beyond the neonatal period. Four children had West syndrome, which resolved with treatment. Thirteen children had febrile seizures that they outgrew. Thirty children had focal epilepsy with seizure manifestations and EEG discharges typical of early-onset childhood occipital epilepsy or childhood epilepsy with centrotemporal spikes; 23 have outgrown these seizures. Two children had idiopathic generalized epilepsy; it was ongoing in 1 child. Fourteen children had evolution from 1 epileptic syndrome to another. At last follow-up (median age, 12.7 years; minimum age, 9.7 years), 80% had not had a seizure for >2 years. CONCLUSIONS: The electroclinical features of seizure disorders associated with CP and WMI are those of the age-limited, epileptic syndromes of childhood, with favorable outcome in the majority. The findings have important implications for counseling and drug treatment. [ABSTRACT FROM AUTHOR]

The purpose of this study was to investigate the frequency and spectrum of magnetic resonance imaging (MRI) abnormalities in a population of children with cerebral palsy (CP) who were born in the years 2000 and 2001 in Victoria, Australia. In 2000 and 2001, 221 children (126 males, 95 females; mean age 6y [SD 7mo], range 5–7y) with CP, excluding those with CP due to postneonatal causes (6% of all cases), were identified through the Victorian Cerebral Palsy Register. All medical records were systematically reviewed and all available brain imaging was comprehensively evaluated by a single senior MRI radiologist. MRI was available for 154 (70%) individuals and abnormalities were identified in 129 (84%). The study group comprised 88% with a spastic motor type CP; the distribution was hemiplegia in 33.5%, diplegia in 28.5%, and quadriplegia in 37.6% of children. Overall, pathological findings were most likely to be identified in children with spastic hemiplegia (92%) and spastic quadriplegia (84%). Abnormalities were less likely to be identified in non-spastic motor types (72%) and spastic diplegia (52%). The most common abnormalities identified on MRI were periventricular white matter injury (31%), focal ischaemic/haemorrhagic lesions (16%), diffuse encephalopathy (14%), and brain malformations (12%). Dual findings were seen in 3% of patients. This is the first study to document comprehensively the neuroimaging findings of all children identified with CP born over a consecutive 24-month period in a large geographical area. [ABSTRACT FROM AUTHOR]