Classification of events contributing to postneonatal cerebral palsy: Development, reliability, and recommendations for use.

AIM: This paper introduces the Surveillance of Cerebral Palsy in Europe (SCPE) classification of events contributing to postneonatally acquired cerebral palsy, presents its interrater reliability, and describes the cases identified in the SCPE database. METHOD: The development of the classification, based on literature review and expert discussions, resulted in six main categories and 19 subcategories. The first chronological event designated as the primary event was mainly reported. Interrater reliability was assessed through online exercise providing 24 clinical vignettes representing single/complex pathways. Percent agreement and Gwet's AC1 index of reliability were estimated. Primary events were described using data of 221 children born between 2008 and 2012. RESULTS: Thirty-nine professionals (21 registries) participated in the reliability exercise. Substantial overall agreement was reached (0.75), with some contrast between complex (0.48, moderate agreement) and single events involved (0.89, almost perfect). The distribution of primary events showed that 32.1% were infections (category A), 23.1% head injuries (B), 15.4% related to surgery or medical interventions (C), 13.1% cerebrovascular accidents (D), 9.1% hypoxic brain damaging events of other origins (E), and 7.2% miscellaneous (F). INTERPRETATION: This classification allows all the events involved to be recorded while consistently reporting the primary event, and may be used in different settings. WHAT THIS PAPER ADDS: A standardized classification enables the description of the events contributing to postneonatal cerebral palsy (CP). The first chronological event in complex pathway leading to CP is coded. Category choice and coding of the primary event identify preventable situations. The detailed 2-level classification is easy to use in various settings. Substantial overall interrater reliability shows that main categories can be consistently differentiated.

Hospital admissions of school-age children with an intellectual disability: A population-based survey.

AIM: To describe the profiles of hospital admissions of school-age children identified with a learning disability (ICD-11 intellectual developmental disorder) and/or safeguarding needs compared to children without learning disability, in a population where proactive identification of learning disabilities in children is embedded in practice. METHOD: Data were collected about the reasons for and duration of hospital admissions of school-age children living in the study catchment area between April 2017 and March 2019; the presence (or absence) of learning disability and/or safeguarding flags in the medical record was also noted. The impact of the presence of flags on the outcomes was explored using negative binomial regression modelling. RESULTS: Of 46 295 children in the local population, 1171 (2.53%) had a learning disability flag. The admissions of 4057 children were analysed (1956 females; age range 5-16 years, mean 10 years 6 months, SD 3 years 8 months). Of these, 221 out of 4057 (5.5%) had a learning disability, 443 out of 4057 (10.9%) had safeguarding flags, 43 out of 4057 (1.1%) had both, and 3436 out of 4057 (84.7%) had neither. There was a significantly increased incidence of hospital admissions and length of stay in children with either or both flags, compared to children with neither. INTERPRETATION: Children with learning disabilities and/or safeguarding needs have higher rates of hospital admissions than children without. Robust identification of learning disabilities in childhood is required to make the needs of this group visible in routinely collected data as the first step towards needs being appropriately addressed. WHAT THIS PAPER ADDS: Children with learning disabilities must be consistently identified in populations so that their needs are made visible. Information about these needs must be collected from educational, health, and social care sources and scrutinized systematically. Children with learning disabilities and safeguarding needs have an increased incidence of hospital admissions and length of stay.

'YourTube' the role of different diets in gastrostomy-fed children: Baseline findings from a prospective cohort study.

AIM: To assess the risks, benefits, and resource implications of home-blended food for children with gastrostomy tubes compared with a formula diet. METHOD: This prospective cohort study of children (aged 0-18 years) collected baseline data on gastrointestinal symptoms, nutritional intake, anthropometric outcomes, parent and child quality of life, and resource use. A propensity score-weighted generalized linear mixed model was used to compare children receiving a home-blended versus formula diet. RESULTS: Baseline data were obtained for 180 children (2019-2021; 107 males, 73 females; mean age 9 years 7 months [SD 4 years 5 months]). Children receiving a home-blended diet (n = 104) had similar diagnoses and age but more lived in areas of lower deprivation and parental education was higher compared to the parents of children receiving a formula diet (n = 76). Children receiving home-blended diets had significantly better gastrointestinal scores than those receiving formula diets (B = 13.8, p < 0.001). The number of gut infections and tube blockages were similar between the two groups but with fewer stoma site infections in the group receiving home-blended food. Children receiving a home-blended diet had more fibre in their diet compared to children receiving a formula diet. INTERPRETATION: Home-blended diets should be seen as a safe option for children who are gastrostomy-fed unless clinically contraindicated. Equality of access to home-blended diets for children with gastrostomy should be assessed by local clinical teams.

Identifying phenotypic expansions for congenital diaphragmatic hernia plus (CDH+) using DECIPHER data.

Congenital diaphragmatic hernia (CDH) can occur in isolation or in conjunction with other birth defects (CDH+). A molecular etiology can only be identified in a subset of CDH cases. This is due, in part, to an incomplete understanding of the genes that contribute to diaphragm development. Here, we used clinical and molecular data from 36 individuals with CDH+ who are cataloged in the DECIPHER database to identify genes that may play a role in diaphragm development and to discover new phenotypic expansions. Among this group, we identified individuals who carried putatively deleterious sequence or copy number variants affecting CREBBP, SMARCA4, UBA2, and USP9X. The role of these genes in diaphragm development was supported by their expression in the developing mouse diaphragm, their similarity to known CDH genes using data from a previously published and validated machine learning algorithm, and/or the presence of CDH in other individuals with their associated genetic disorders. Our results demonstrate how data from DECIPHER, and other public databases, can be used to identify new phenotypic expansions and suggest that CREBBP, SMARCA4, UBA2, and USP9X play a role in diaphragm development.

Outcomes for gastrostomy-fed children and their parents: qualitative findings from the 'Your Tube' study.

AIM: To identify child and parent outcomes relevant to having a gastrostomy, and to specify outcomes believed to be particularly salient to type of diet (formula vs blended food). METHOD: Twenty parents, two children (both 12y), and 41 professionals (dietitians [n=10]; nurses [n=12]; paediatricians [n=12]; speech and language therapists [n=7)]) were recruited. Parents and children were interviewed; professionals participated in focus groups. Children (2-18y) represented included those on formula (n=11), blended-food (n=7), and mixed (n=2) diets. All had been tube-fed for at least 6 months. Neurological, genetic, and metabolic conditions were represented. RESULTS: Participants identified a range of children's outcomes relevant to a gastrostomy, including physical health, gastrointestinal symptoms, sleep, and time spent feeding. The children described experiences of exclusion caused by being tube-fed. Time, sleep, and emotional health were regarded as most salient to understanding parents' gastrostomy outcomes. Participants believed type of diet would most likely effect gastrointestinal symptoms, time spent feeding, sleep, and physical health. INTERPRETATION: Findings indicate a number of refinements to, and allow further specification of, the current 'initial' core outcome set for tube-fed children. Findings also have implications for choice of outcomes measures. Further qualitative research with children and young people is needed. What this paper adds Sleep is a key outcome for children and parents. Gastrointestinal symptoms and physical health were regarded as outcomes most likely to be affected by type of diet. Well-being and participation were identified as key distal outcomes. Gastrostomies are complex interventions. Further specification of the core outcome set is possible.

Something hazy drifting in the air .

-A 7-year-old boy with a background of autism presented to the paediatric emergency department with his left arm 'feeling strange' then became difficult to rouse. On examination, he was found to have left arm weakness and a left-sided facial droop without forehead muscle involvement. Three hours later, his symptoms had completely resolved and he was suspected to have had a first seizure. He was admitted for observation and an electroencephalography which showed slower rhythms in the right posterior quadrant, which was reported as within normal appearances for his age. He was discharged home the following day. Three days later he became lethargic and vomited. His parents reported dysphasia with use of single-word phrases only, he also appeared confused. He re-presented to the paediatric emergency department, where he was found to have increased tone in the left arm, ankle clonus and an upgoing plantar reflex on the left hand side.

Access to Intrathecal Baclofen Treatment for Children with Cerebral Palsy in European Countries: An SCPE Survey Reveals Important Differences.

AIM: The aim is to study access to intrathecal baclofen (ITB) for children with cerebral palsy (CP) in Europe, as an indicator of access to advanced care. METHODS: Surveys were sent to CP registers, clinical networks, and pump manufacturers. Enquiries were made about ITB treatment in children born in 1990 to 2005 by sex, CP type, level of gross motor function classification system (GMFCS) and age at the start of treatment. Access to ITB was related to the country's gross domestic product (GDP) and % GDP spent on health. RESULTS: In 2011 population-based data from Sweden, Norway, England, Portugal, Slovenia, and Denmark showed that 114 (3.4%) of 3,398 children with CP were treated with ITB, varying from 0.4 to 4.7% between centers. The majority of the children were at GMFCS levels IV-V and had bilateral spastic CP. In Sweden, dyskinetic CP was the most commonly treated subtype. Boys were more often treated with ITB than girls (p = 0.014). ITB was reported to be available for children with CP in 25 of 43 countries. Access to ITB was associated with a higher GDP and %GDP spent on health (p < 0.01). Updated information from 2019 showed remaining differences between countries in ITB treatment and sex difference in treated children was maintained. CONCLUSION: There is a significant difference in access to ITB for children with CP across Europe. More boys than girls are treated. Access to ITB for children with CP is associated with GDP and percent of GDP spent on health in the country.

The Origin of the Cerebral Palsies: Contribution of Population-Based Neuroimaging Data.

BACKGROUND: Surveillance of cerebral palsy in Europe (SCPE) presents the first population-based results on neuroimaging findings in children with cerebral palsy (CP) using a magnetic resonance imaging classification system (MRICS). METHOD: MRIs of children with CP born between 1999 and 2009 from 18 European countries were analyzed. MRICS identifies patterns of brain pathology according to timing during brain development which was analyzed with respect to CP subtypes and gestational age. RESULTS: MRIs or written reports from 3,818 children were available. The main clinical characteristics were similar to the 5,415 without such data. Most frequent was predominant white matter injury (49%), followed by predominant gray matter injury (21%). Maldevelopments were found in 11% of cases. Miscellaneous findings were present in 8.5% and normal findings in 10.6%. MRI patterns of children with unilateral spastic, bilateral spastic, and dyskinetic CP were mainly lesional (77, 71, and 59%, respectively), whereas children with ataxic CP had more maldevelopments, miscellaneous, and normal findings (25, 21, and 32%, respectively). In children born preterm, predominant white matter injury was most prevalent (80% in children born <32 weeks of gestation). CONCLUSION: Analysis of MRI in the European CP database identified CP as a mainly lesional condition on a large population basis, maldevelopments were relatively uncommon. An exception was ataxic CP. Children born preterm mostly presented with a lesion typical for their gestational age (GA) at birth. The decreasing prevalence of CP in this group suggests that progress in perinatal and neonatal medicine may lead to a reduction of these lesions.

Austerity and families with disabled children: a European survey.

AIM: To describe the impact austerity measures have had on families with disabled children across Europe and on professionals providing services for them. METHOD: Cross-sectional surveys were disseminated via professional and family networks in 32 European countries for 3 months from December 2016. RESULTS: Families (n=731), of whom 45% met UNICEF criteria for severe poverty, and professionals (n=959) responded from 23 and 32 countries respectively. Respondents were grouped into those from countries with and without austerity. The direct and indirect impact of austerity cuts and worse working conditions were reported more often by professionals from countries with austerity, compared to those without. Most families reported services to be worse in quality than 3 years ago. Families with completely dependent disabled children said the needs of their disabled children are significantly less well met now, compared to 10 years ago. INTERPRETATION: A decline in quality of services for disabled children was reported by most family and many professional respondents across Europe, regardless of austerity. Where implemented, austerity measures were reported to have impacted significantly on families with disabled children. What this paper adds Stigma about disability remains a challenge in many countries across Europe. Most families and many health care professionals reported worsening quality of services than 3 years ago, regardless of austerity. Austerity cuts are reported to have impacted especially negatively on families with dependent disabled children.

The Health, Functioning and Wellbeing Summary Traffic Light Communication Tool: a survey of families' views.

AIM: To ascertain families' views about the utility of the Health, Functioning and Wellbeing Summary (HFWS) in positively supporting communication in clinics. METHOD: The HFWS was co-developed with families and members of the multidisciplinary team. A sequential convenience sample of 60 families who attended clinics in Sunderland, UK in 2015 agreed to participate and answered eight questions about their views on the tool's usefulness. Data were recorded and analysed in Microsoft Excel. RESULTS: All families agreed that they had been able to discuss everything they had wanted to discuss with the doctor. All of the serious concerns that they had recorded on the HFWS had been addressed in the consultation. Most (58 out of 60; 97%) reported that the tool was easy to understand and complete, and 57 out of 60 (95%) reported that it helped them to gather their thoughts about what they wanted to discuss. Consultations that used the tool were thought to better address the needs of 49 out of 60 families (87.1%), than those in which it was not used. INTERPRETATION: The HFWS was acceptable to most families surveyed, improved communication from their perspective, and ensured that the issues that mattered most to them were addressed in medical consultations. The tool has been translated into a number of other languages. Its acceptability and utility in other settings requires further study.

Prevalence and characteristics of autism spectrum disorders in children with cerebral palsy.

AIM: To evaluate the prevalence of co-occurring autism spectrum disorders (ASDs) among children with cerebral palsy (CP), and to describe their characteristics. METHOD: The data of 1225 CP cases from four population-based registers (Iceland, Sweden, and two in France) and one population-based surveillance programme (North East England, UK) participating in the Surveillance of Cerebral Palsy in Europe Network (SCPE) were analysed. The ASD diagnoses were systematically recorded using category F84 of the International Classification of Diseases, 10th Revision. The registers provided data on children born between 1995 and 2006, while the cross-sectional survey in the UK concerned children aged 0 to 19 years, registered in 2010. RESULTS: Among the children with CP, 107 had an associated diagnosis of ASD - i.e., 8.7% of the study population (95% confidence interval 7.2-10.5). This proportion varied across centres from 4.0% to 16.7% but was independent of CP prevalence. Male sex, co-occurring epilepsy, intellectual disability, and better walking ability were associated with the coexistence of ASD. INTERPRETATION: Our findings support the need for a multidisciplinary approach to management of children with CP to adequately identify and address all facets of presentation, including ASD.

MRI classification system (MRICS) for children with cerebral palsy: development, reliability, and recommendations.

AIM: To develop and evaluate a classification system for magnetic resonance imaging (MRI) findings of children with cerebral palsy (CP) that can be used in CP registers. METHOD: The classification system was based on pathogenic patterns occurring in different periods of brain development. The MRI classification system (MRICS) consists of five main groups: maldevelopments, predominant white matter injury, predominant grey matter injury, miscellaneous, and normal findings. A detailed manual for the descriptions of these patterns was developed, including test cases (www.scpenetwork.eu/en/my-scpe/rtm/neuroimaging/cp-neuroimaging/). A literature review was performed and MRICS was compared with other classification systems. An exercise was carried out to check applicability and interrater reliability. Professionals working with children with CP or in CP registers were invited to participate in the exercise and chose to classify either 18 MRIs or MRI reports of children with CP. RESULTS: Classification systems in the literature were compatible with MRICS and harmonization possible. Interrater reliability was found to be good overall (k=0.69; 0.54-0.82) among the 41 participants and very good (k=0.81; 0.74-0.92) using the classification based on imaging reports. INTERPRETATION: Surveillance of Cerebral Palsy in Europe (SCPE) proposes the MRICS as a reliable tool. Together with its manual it is simple to apply for CP registers.

Prospective pilots of routine data capture by paediatricians in clinics and validation of the Disabilities Complexity Scale.

AIMS: To pilot prospective data collection by paediatricians at the point of care across England using a defined terminology set; demonstrate feasibility of data collection and utility of data outputs; and confirm that counting the number of needs per child is valid for quantifying complexity. METHOD: Paediatricians in 16 hospital and community settings collected and anonymized data. Participants completed a survey regarding the process. Data were analysed using R version 3.1.2. RESULTS: Overall, 8117 needs captured from 1224 consultations were recorded. Sixteen clinicians responded positively about the process and utility of data collection. The sum of needs varied significantly (p<0.01) by level of gross motor function ascertained using the Gross Motor Function Classification System for children with cerebral palsy; epilepsy severity as defined by level of expertise required to manage it; and by severity of intellectual disability. INTERPRETATION: Prospective data collection at the point of clinical care proved possible without disrupting clinics, even for those with the most complex needs, and took the least time when done electronically. Counting the number of needs was easy to do, and quantified complexity in a way that informed clinical care for individuals and related directly to validated scales of functioning. Data outputs could inform more appropriate design and commissioning of quality services.

Quantifying multifaceted needs captured at the point of care. Development of a Disabilities Terminology Set and Disabilities Complexity Scale.

AIMS: To develop a Disabilities Terminology Set and quantify the multifaceted needs of disabled children and their families in a district disability clinic population. METHOD: Data from structured electronic clinic letters of children attending paediatric disability clinics from June 2007 to May 2012 in Sunderland, north-east England collected at the point of clinical care were analysed to determine appropriate terms for consistent recording of each need and issue. Terms were collated to count the number of needs per child. RESULTS: A Systemized Nomenclature of Medicine - Clinical Terms subset of 296 terms was identified and published, and 8392 consultations for 1999 children were reviewed. The required number of clinic appointments correlated strongly with the number of needs identified. Children with intellectual disabilities in addition to cerebral palsy and epilepsy had more than double the number of conditions, technology dependencies, and family-reported issues than those without. Disabled children who subsequently died had the highest burden of needs (p=0.007). INTERPRETATION: Detailed data about needs generated outputs useful for local care pathway development and service planning. Sufficient evidence was provided for successful business cases leading to the appointment of additional paediatric disability consultants. Counting numbers of needs and issues quantifies complexity in a straightforward way. This could underpin needs-based commissioning of services.

Variation in health care for children and young people with cerebral palsies: a retrospective multicentre audit study.

AIM: To explore the provision and variations in care for children and young people with cerebral palsies (CP) registered with the population-based North of England Collaborative Cerebral Palsy Survey (NECCPS). METHOD: This is a retrospective multicentre record audit of 389 children with CP (220 males, 148 females, 21 no data; median age at time of audit 12y 3mo), born between 1995 and 2002. Data were collected on cranial magnetic resonance imaging (MRI), hip and spine surveillance and management, and pain presence and management. Variations over time and between the districts in the north of England (Northumberland, North and West Cumbria, North and South Tyneside, Newcastle-upon-Tyne, Gateshead, Sunderland, Durham, Darlington, Bishop Auckland, Hartlepool, Stockton-on-Tees, Middlesbrough, Redcar, and Cleveland), and by socio-economic status (SES) (estimated from the Index of Multiple Deprivation [IMD] 2004) were estimated by generalized estimating equations. RESULTS: There was significant variation between districts in access to MRI (p<0.001), orthopaedic surgeons (p=0.005), recording state of spine (p<0.001), and discussions about pain (p<0.001). Fifty-seven per cent (95% CI 52-62) had evidence of a reported MRI brain scan, the proportion of which increased over time (p<0.001). Sixty-seven per cent (95% CI 62-71) had a discussion about pain recorded. Of those in pain, 87% (95% CI 80-93) had a pain management plan. The proportion with documented discussion about pain increased with increasing SES (p=0.04). INTERPRETATION: The provision of care for children with CP in the north of England varies between districts. Internationally agreed, evidence-based standards are urgently needed to ensure more equitable health care and improved outcomes for all.

Trends in hospital admissions during transition from paediatric to adult services for young people with learning disabilities or autism: Population-based cohort study.

Background: Transition from paediatric to adult health care may disrupt continuity of care, and result in unmet health needs. We describe changes in planned and unplanned hospital admission rates before, during and after transition for young people with learning disability (LD), or autism spectrum disorders (ASD) indicated in hospital records, who are likely to have more complex health needs. Methods: We developed two mutually exclusive cohorts of young people with LD, and with ASD without LD, born between 1990 and 2001 in England using national hospital admission data. We determined the annual rate of change in planned and unplanned hospital admission rates before (age 10-15 years), during (16-18 years) and after (19-24 years) transition to adult care using multilevel negative binomial regression models, accounting for area-level deprivation, sex, birth year and presence of comorbidities. Findings: The cohorts included 51,291 young people with LD, and 46,270 autistic young people. Admission rates at ages 10-24 years old were higher for young people with LD (54 planned and 25 unplanned admissions per 100 person-years) than for autistic young people (17/100 and 16/100, respectively). For young people with LD, planned admission rates were highest and constant before transition (rate ratio [RR]: 0.99, 95% confidence interval [CI] 0.98-0.99), declined by 14% per year of age during (RR: 0.86, 95% CI: 0.85-0.88), and remained constant after transition (RR: 0.99, 95% CI: 0.99-1.00), mainly due to fewer admissions for non-surgical care, including respite care. Unplanned admission rates increased by 3% per year of age before (RR: 1.03, 95% CI: 1.02-1.03), remained constant during (RR: 1.01, 95% CI: 1.00-1.03) and increased by 3% per year after transition (RR: 1.03, 95% CI: 1.02-1.04). For autistic young people, planned admission rates increased before (RR: 1.06, 95% CI: 1.05-1.06), decreased during (RR: 0.95, 95% CI: 0.93-0.97), and increased after transition (RR: 1.05, 95%: 1.04-1.07). Unplanned admission rates increased most rapidly before (RR: 1.16, 95% CI: 1.15-1.17), remained constant during (RR: 1.01, 95% CI: 0.99-1.03), and increased moderately after transition (RR: 1.03, 95% CI: 1.02-1.04). Interpretation: Decreases in planned admission rates during transition were paralleled by small but consistent increases in unplanned admission rates with age for young people with LD and autistic young people. Decreases in non-surgical planned care during transition could reflect disruptions to continuity of planned/respite care or a shift towards provision of healthcare in primary care and community settings and non-hospital arrangements for respite care. Funding: National Institute for Health Research Policy Research Programme.