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BACKGROUND: Obtaining peripheral intravenous catheter (PIVC) access in children with severe neurological impairment (SNI) is often challenging and commonly associated with complications, including dislodgement, phlebitis and extravasation. In severe cases, extravasation injury may lead to tissue necrosis, ulceration and long-term morbidity. The aim of this study was to determine the relative incidence of PIVC complications secondary to lower limb cannulation, compared to upper limb, in children with SNI. METHODS: A single centre, retrospective, observational review was conducted. Patients with SNI, admitted at a tertiary paediatric centre over 6 months between July and December 2022, were included. RESULTS: One-hundred fifty-five PIVC procedures were conducted in 110 children over the study period. Complications were more common in lower limb PIVCs (12/16, 75%) compared to upper limb (58/139, 42%), p = 0.01. CONCLUSION: Upper limb cannulation is preferred in children with SNI.
Subject(s)
Catheterization, Peripheral , Child , Humans , Retrospective Studies , Catheterization, Peripheral/adverse effects , Catheterization, Peripheral/methods , Upper Extremity , Hospitalization , IncidenceABSTRACT
INTRODUCTION: The most common cause of morbidity and mortality in children with severe cerebral palsy (CP) is respiratory disease. BREATHE-CP (Better REspiratory and Airway Treatment and HEalth in Cerebral Palsy) is a multidisciplinary research team who have conducted research on the risk factors associated with CP respiratory disease, a systematic review on management and a Delphi study on the development of a consensus for the prevention and management of respiratory disease in CP. These strategies have not been investigated; therefore, it is not known if implementation is feasible, if they improve patient outcomes or if they are acceptable for families. METHODS AND ANALYSIS: Mixed-method feasibility pilot randomised controlled trial with economic analysis. Twenty children with CP aged 0-12 years who are at risk of respiratory disease will be followed up for 1 year. All children will receive baseline assessments for comparison. The control group will receive usual care from their treating teams. The intervention group will receive comprehensive assessments from physiotherapy, speech pathology and respiratory medicine. An individualised investigation and treatment plan will then be made. Participants in both groups will complete fortnightly patient-reported outcome surveys to assess symptoms and health service use. Analysis will include assessments of acceptability through qualitative interviews, implementation by ability to recruit, randomise and retain, practicality including costs of intervention and hospitalisation, and explore efficacy through quality-of-life surveys and decreased health service use for respiratory-related symptoms. ETHICS AND DISSEMINATION: Ethics and governance approvals have been obtained through Child and Adolescent Health Service Human Research Ethics Committee. At completion, this study will lead to the design of the definitive protocol to test intervention efficacy that maximises recruitment, retention and adherence to interventions. TRIAL REGISTRATION NUMBER: Australian New Zealand Clinical Trials Registry (ACTRN12620000114943).
Subject(s)
Cerebral Palsy , Feasibility Studies , Humans , Cerebral Palsy/therapy , Pilot Projects , Child, Preschool , Child , Infant , Randomized Controlled Trials as Topic , Hospitalization , Male , Female , Infant, Newborn , Respiratory Tract Diseases/therapy , AustraliaABSTRACT
PURPOSE: To study seizure manifestations and outcomes in children with cortical versus white matter injury, differences potentially explaining variability of epilepsy in children with cerebral palsy. METHODS: In this population-based retrospective cohort study, MRIs of children with cerebral palsy due to ischemia or haemorrhage were classified according to presence or absence of cortical injury. MRI findings were then correlated with history of neonatal seizures, seizures during childhood, epilepsy syndromes, and seizure outcomes. RESULTS: Of 256 children studied, neonatal seizures occurred in 57 and seizures during childhood occurred in 93. Children with neonatal seizures were more likely to develop seizures during childhood, mostly those with cortical injury. Cortical injury was more strongly associated with (1) developing seizures during childhood, (2) more severe epilepsy syndromes (infantile spasms syndrome, focal epilepsy, Lennox-Gastaut syndrome), and (3) less likelihood of reaching > 2 years without seizures at last follow-up, compared to children without cortical injury. Children without cortical injury, mainly those with white matter injury, were less likely to develop neonatal seizures and seizures during childhood, and when they did, epilepsy syndromes were more commonly febrile seizures and self-limited focal epilepsies of childhood, with most achieving > 2 years without seizures at last follow-up. The presence of cortical injury also influenced seizure occurrence, severity, and outcome within the different predominant injury patterns of the MRI Classification System in cerebral palsy, most notably white matter injury. CONCLUSIONS: Epileptogenesis is understood with cortical injury but not well with white matter injury, the latter potentially related to altered postnatal white matter development or myelination leading to apoptosis, abnormal synaptogenesis or altered thalamic connectivity of cortical neurons. These findings, and the potential mechanisms discussed, likely explain the variability of epilepsy in children with cerebral palsy and epilepsy following early-life brain injury in general.
Subject(s)
Brain Injuries , Cerebral Palsy , Epilepsies, Partial , Epilepsy , Seizures, Febrile , Spasms, Infantile , White Matter , Child , Infant, Newborn , Humans , Retrospective Studies , Cerebral Palsy/complications , Cerebral Palsy/diagnostic imaging , White Matter/diagnostic imaging , Epilepsy/complications , Spasms, Infantile/complications , Brain Injuries/complications , Brain Injuries/diagnostic imaging , ElectroencephalographyABSTRACT
BACKGROUND: Intractable feeding intolerance in children with severe neurological impairment (SNI) is poorly defined and understood. OBJECTIVES: (1) To describe 9 children with SNI, where intractable feeding intolerance was thought to be a contributor to their deterioration or death. (2) To consider terminology to describe the severe end of the spectrum of feeding difficulties in children with SNI. RESULTS: Mean age at death was 10.3 years (range: 5 - 15.6), and median time from palliative care referral to death was 3.1 months. Location of death was home (n = 3), hospice (n = 1), and hospital (n = 5) with 1 death in intensive care. Gastrointestinal "failure" or "dysfunction" were documented for 7 children, (median time between documentation and death was 3.9 months (range: .1 to 13.1)). All children were fed via a gastrostomy tube during their life (median age of insertion 2.5 years (range: 1.2 to 6.8 years)), and 7 via the jejunal route (median age of insertion 9.2 years (range 2.4 to 14.7 years)). Children lived a median of 9 percent of their lives after jejunal tube feeding was commenced. No child had home-based parenteral nutrition. Multiple symptom management medications were required. CONCLUSION: 'Intractable feeding intolerance' describes a clinical crossroads in a child's life where there is an opportunity to consider the appropriateness of further interventions. Further work should explore predictors of intractable feeding intolerance and the delicate balance between cause or contributor to death. The importance of clinician-family prognostic conversations and goal-concordant care both during life and in the terminal phase is highlighted.
Subject(s)
Hospice and Palliative Care Nursing , Hospices , Child , Humans , Infant, Newborn , Infant , Child, Preschool , Adolescent , Palliative Care , Retrospective Studies , Enteral NutritionABSTRACT
Cerebral palsy is a life-long condition and the most common cause of physical disability in childhood [...].
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Objective: To determine the frequency of paroxysmal nonepileptic events in children with cerebral palsy due to brain injury who have epilepsy and to describe the factors associated with paroxysmal nonepileptic events. Methods: Retrospective, population-based study of children from the Victorian CP Register born 1999-2006. Neuroimaging, medical records, electroencephalograms (EEG), and EEG requests were analyzed. Results: Of the included 256 children, 87 had epilepsy. EEGs (with video correlation) were available for 82 of 87. Eighteen (18/82, 22%) had epileptic events captured on EEG. Twenty-one (21/82, 26%) had paroxysmal nonepileptic events captured on EEG. The majority (13/18, 77%) of children with epileptic events also had paroxysmal nonepileptic events captured. Ten parents and carers continued to report events as epileptic despite there being no ictal EEG correlate for specific events on multiple EEGs. There were no clear associations to identify which children would have ongoing paroxysmal nonepileptic events reported. Conclusions: Paroxysmal nonepileptic events were captured on EEG in one-fourth of children from this cerebral palsy cohort with epilepsy and available EEG. Half the parents and carers reported previously identified paroxysmal nonepileptic events as epileptic on subsequent EEGs, highlighting the need for clearer counseling so that parents better understand seizure semiology in children with EEG-proven paroxysmal nonepileptic events.
Subject(s)
Brain Injuries , Cerebral Palsy , Epilepsy , Child , Humans , Cerebral Palsy/complications , Retrospective Studies , Epilepsy/complications , Seizures/etiology , Electroencephalography/methodsABSTRACT
Seizures occur in approximately one-third of children with cerebral palsy. This study aimed to determine epilepsy syndromes in children with seizures and cerebral palsy due to vascular injury, anticipating that this would inform treatment and prognosis. We studied a population-based cohort of children with cerebral palsy due to prenatal or perinatal vascular injuries, born 1999-2006. Each child's MRI was reviewed to characterize patterns of grey and white matter injury. Children with syndromic or likely genetic causes of cerebral palsy were excluded, given their inherent association with epilepsy and our aim to study a homogeneous cohort of classical cerebral palsy. Chart review, parent interview and EEGs were used to determine epilepsy syndromes and seizure outcomes. Of 256 children, 93 (36%) had one or more febrile or afebrile seizures beyond the neonatal period and 87 (34%) had epilepsy. Children with seizures were more likely to have had neonatal seizures, have spastic quadriplegic cerebral palsy and function within Gross Motor Function Classification System level IV or V. Fifty-six (60%) children with seizures had electroclinical features of a self-limited focal epilepsy of childhood; we diagnosed these children with a self-limited focal epilepsy-variant given the current International League Against Epilepsy classification precludes a diagnosis of self-limited focal epilepsy in children with a brain lesion. Other epilepsy syndromes were focal epilepsy-not otherwise specified in 28, infantile spasms syndrome in 11, Lennox-Gastaut syndrome in three, genetic generalized epilepsies in two and febrile seizures in nine. No epilepsy syndrome could be assigned in seven children with no EEG. Twenty-one changed syndrome classification during childhood. Self-limited focal epilepsy-variant usually manifested with a mix of autonomic and brachio-facial motor features, and occipital and/or centro-temporal spikes on EEG. Of those with self-limited focal epilepsy-variant, 42/56 (75%) had not had a seizure for >2 years. Favourable seizure outcomes were also seen in some children with infantile spasms syndrome and focal epilepsy-not otherwise specified. Of the 93 children with seizures, at last follow-up (mean age 15 years), 61/91 (67%) had not had a seizure in >2 years. Children with cerebral palsy and seizures can be assigned specific epilepsy syndrome diagnoses typically reserved for normally developing children, those syndromes commonly being age-dependent and self-limited. Compared to typically developing children with epilepsy, self-limited focal epilepsy-variant occurs much more commonly in children with cerebral palsy and epilepsy. These findings have important implications for treatment and prognosis of epilepsy in cerebral palsy, and research into pathogenesis of self-limited focal epilepsy.
Subject(s)
Cerebral Palsy , Epilepsies, Partial , Epilepsy , Spasms, Infantile , Child , Infant, Newborn , Humans , Adolescent , Spasms, Infantile/complications , Cerebral Palsy/complications , Electroencephalography , Syndrome , SeizuresABSTRACT
Neuromuscular scoliosis is a common feature in children with severe neurological impairment (SNI), including those with severe cerebral palsy. Surgical correction of scoliosis is the mainstay of treatment. This group of patients also have associated medical complexity. The complication rates post-surgery are high, although, for many, they are worth the risk. There are currently no published practice guidelines or care pathways for children with SNI who are undergoing scoliosis corrective surgery. In response to the high uptake of this surgery, coupled with the expected complication rates, our hospital established a perioperative clinic. The purpose of this paper is to describe our perioperative approach. This clinic has developed into a service beyond perioperative care and, with the collaborative meeting, enables shared decision-making to identify the right candidate for surgery. The process involves surgical expertise, understanding the family and child at the centre, and optimisation of medical care pre- and post-surgery. In this paper, we describe the process in a step-by-step manner. We provide clinical vignettes, as well as the proformas that we use, and we highlight the benefits of the team-based process.
ABSTRACT
Cerebral palsy (CP) is a broad diagnosis unbound by aetiology and is based on a clinical examination demonstrating abnormalities of movement or posture. CP represents a static neurological condition, provided that neurodegenerative conditions, leukoencephalopathies and neuromuscular disorders are excluded. In paediatrics, the genetic conditions associated with CP are rapidly increasing, with primary and overlapping neurodevelopmental conditions perhaps better categorised by the predominant clinical feature such as CP, intellectual disability, autism spectrum disorder or epilepsy. Progress in molecular genetics may challenge what constitutes CP, but a genetic diagnosis does not negate the CP diagnosis. As clinicians working in the field, we discuss the changing tide of CP. Neuroimaging provides essential information through pattern recognition and demonstration of static brain changes. We present examples of children where a layered clinical diagnosis or dual aetiologies are appropriate. We also present examples of children with genetic causes of CP to highlight the challenges and limitations of neuroimaging to provide an aetiological diagnosis. In consultation with a geneticist, access to genomic testing (exome or genome sequencing) is now available in Australia under Medicare billing for children under the age of 10 with dysmorphic features, one or more major structural organ anomalies, (an evolving) intellectual disability or global developmental delay. We encourage the uptake of genomic testing in CP, because it can be difficult to tell whether a child has an environmental or genetic cause for CP. A specific genetic diagnosis may change patient management, reduce guilt and enable more distinctive research in the future to assist with understanding disease mechanisms.
Subject(s)
Autism Spectrum Disorder , Cerebral Palsy , Epilepsy , Intellectual Disability , Aged , Child , Humans , Cerebral Palsy/etiology , Cerebral Palsy/complications , Intellectual Disability/etiology , Intellectual Disability/complications , Autism Spectrum Disorder/complications , National Health ProgramsABSTRACT
BACKGROUND: While classified as a rare condition, a congenital disorder of the corpus callosum (DCC) is one of the most commonly identified brain anomalies in newborns, occurring in 1:4000 live births. Advances in imaging techniques have improved early diagnosis for children, yet adults with a DCC-who may present with extreme heterogeneity in cause and impact-often experience challenges in receiving a definitive diagnosis and accessing appropriate services and supports. To date, the dearth of evidence documenting the lived experiences of adults with DCC has made it difficult to determine adequate policy and service responses. This exploratory research aims to address this gap by presenting the first qualitative examination of the experiences and impact of complete or partial agenesis of the corpus callosum among adults. RESULTS: Eight face-to-face interviews were conducted with Australian adults, aged 23-72 years, to explore their lived experience. Data was collected in four Australian states from June to August 2017. Thematic and interpretive analyses were employed to analyse data. Three emergent themes described difficulties related to: (1) reactions to the diagnosis; (2) access to supports and key life domains, and (3) identifying as an adult. Interview analysis described lived experiences typically outlining a lifetime of exclusion and misunderstanding from family, educators and disability and health support services. CONCLUSIONS: This paper contributes to filling the knowledge gap around a rare congenital brain disorder affecting the lives of adults. Findings confirm a considerable lack of information and support for adults living with corpus callosum disorders. Greater professional and societal understanding is needed to improve access to the key life domains of education, employment and social inclusion for adults with a DCC. To instigate truly effective change, social research must tackle the issues of applicability and impact to alter the dominance of uninformed practices, hindered by prevailing myths. This research paves the way for further phenomenological studies in which participant narrative is vital. Further research will elicit stronger policy and service responses for all current and emerging adults with a DCC.
Subject(s)
Disabled Persons , Nervous System Malformations , Adult , Aged , Agenesis of Corpus Callosum , Australia , Child , Corpus Callosum , Humans , Infant, Newborn , Middle Aged , Young AdultSubject(s)
Disabled Persons , Sialorrhea , Child , Humans , Salivary Ducts , Sialorrhea/etiology , Sialorrhea/surgery , Submandibular GlandABSTRACT
Respiratory illness is the leading cause of mortality in children with cerebral palsy (CP). Although risk factors for developing chronic respiratory illness have been identified, comprehensive clinical care recommendations for the prevention and management of respiratory illness do not currently exist. We invited over 200 clinicians and researchers from multiple disciplines with expertise in the management of respiratory illness in children with CP to develop care recommendations using a modified Delphi method on the basis of the RAND Corporation-University of California Los Angeles Appropriateness Method. These recommendations are intended for use by the wide range of practitioners who care for individuals living with CP. They provide a framework for recognizing multifactorial primary and secondary potentially modifiable risk factors and for providing coordinated multidisciplinary care. We describe the methods used to generate the consensus recommendations, and the overall perspective on assessment, prevention, and treatment of respiratory illness in children with CP. WHAT THIS PAPER ADDS: The first consensus statement for preventing and managing respiratory disease in cerebral palsy (CP). Risk factors for respiratory disease in CP should be identified early. Individuals with CP at risk of respiratory disease require regular assessment of risk factors. Effective partnerships between multidisciplinary teams, individuals with CP, and families are essential. Treatment of respiratory disease in individuals with CP must be proactive.
La enfermedad respiratoria es la principal causa de mortalidad en niños con parálisis cerebral (PC). Aunque se han identificado los factores de riesgo para desarrollar enfermedades respiratorias crónicas, actualmente no existen recomendaciones completas de atención clínica para la prevención y el tratamiento de las enfermedades respiratorias. Invitamos a más de 200 médicos e investigadores de múltiples disciplinas con experiencia en el manejo de enfermedades respiratorias en niños con PC para desarrollar recomendaciones de atención utilizando un método Delphi modificado sobre la base del Método de adecuación RAND Corporation - Universidad de California en Los Ángeles. Estas recomendaciones están destinadas a ser utilizadas por la amplia gama de profesionales que atienden a personas que viven con PC. Proporcionan un marco para reconocer factores de riesgo multifactoriales primarios y secundarios potencialmente modificables y para proporcionar atención coordinada multidisciplinaria. Describimos los métodos utilizados para generar las recomendaciones de consenso, y la perspectiva general sobre la evaluación, prevención y tratamiento de enfermedades respiratorias de niños con PC.
Doença respiratória é a principal causa de mortalidade em crianças com paralisia cerebral (PC). Embora fatores de risco para desenvolver doença respiratória crônica tenham sido identificados, recomendações abrangentes de cuidado clínico e gerenciamento de doença respiratória não existem atualmente. Convidamos cerca de 200 clínicos e pesquisadores de múltiplas disciplinas com experiência no manejo de doença respiratória em crianças com PC para desenvolver recomendações de cuidado usando um método Delphi com base no método de Apropriação da Corporação RAND - Universidade da Califórnia. Estas recomendações são para uso de profissionais que atendem indivíduos com PC. Elas oferecem uma estrutura para reconhecer fatores de risco multifatoriais potencialmente modificáveis e prover cuidado multidisciplinar. Descrevemos métodos usados para gerar as recomendações do consenso, e a perspectiva geral de avaliação, prevenção e tratamento de doença respiratória em crianças com PC.
Subject(s)
Cerebral Palsy/complications , Cerebral Palsy/therapy , Consensus , Practice Guidelines as Topic/standards , Respiration Disorders/etiology , Respiration Disorders/therapy , Adolescent , Adult , Cerebral Palsy/diagnosis , Delphi Technique , Humans , Respiration Disorders/diagnosis , Respiration Disorders/prevention & control , Young AdultABSTRACT
Cerebral palsy (CP) is the most common cause of disability in childhood. Respiratory illness is the most common cause of mortality, morbidity, and poor quality of life in the most severely affected children. Respiratory illness is caused by multiple and combined factors. This review describes these factors and discusses assessments and treatments. Oropharyngeal dysphagia causes pulmonary aspiration of food, drink, and saliva. Speech pathology assessments evaluate safety and adequacy of nutritional intake. Management is holistic and may include dental care, and interventions to improve nutritional intake, and ease, and efficiency of feeding. Behavioral, medical, and surgical approaches to drooling aim to reduce salivary aspiration. Gastrointestinal dysfunction, leading to aspiration from reflux, should be assessed objectively, and may be managed by lifestyle changes, medications, or surgical interventions. The motor disorder that defines cerebral palsy may impair fitness, breathing mechanics, effective coughing, and cause scoliosis in individuals with severe impairments; therefore, interventions should maximize physical, musculoskeletal functions. Airway clearance techniques help to clear secretions. Upper airway obstruction may be treated with medications and/or surgery. Malnutrition leads to poor general health and susceptibility to infection, and improved nutritional intake may improve not only respiratory health but also constipation, gastroesophageal reflux, and participation in activities. There is some evidence that children with CP carry pathogenic bacteria. Prophylactic antibiotics may be considered for children with recurrent exacerbations. Uncontrolled seizures place children with CP at risk of respiratory illness by increasing their risk of salivary aspiration; therefore optimal control of epilepsy may reduce respiratory illness. Respiratory illnesses in children with CP are sometimes diagnosed as asthma; a short trial of asthma medications may be considered, but should be discontinued if ineffective. Overall, management of respiratory illness in children with CP is complex and needs well-coordinated multidisciplinary teams who communicate clearly with families. Regular immunizations, including annual influenza vaccination, should be encouraged, as well as good oral hygiene. Treatments should aim to improve quality of life for children and families and reduce burden of care for carers.
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PURPOSE: TNR, encoding Tenascin-R, is an extracellular matrix glycoprotein involved in neurite outgrowth and neural cell adhesion, proliferation and migration, axonal guidance, myelination, and synaptic plasticity. Tenascin-R is exclusively expressed in the central nervous system with highest expression after birth. The protein is crucial in the formation of perineuronal nets that ensheath interneurons. However, the role of Tenascin-R in human pathology is largely unknown. We aimed to establish TNR as a human disease gene and unravel the associated clinical spectrum. METHODS: Exome sequencing and an online matchmaking tool were used to identify patients with biallelic variants in TNR. RESULTS: We identified 13 individuals from 8 unrelated families with biallelic variants in TNR sharing a phenotype consisting of spastic para- or tetraparesis, axial muscular hypotonia, developmental delay, and transient opisthotonus. Four homozygous loss-of-function and four different missense variants were identified. CONCLUSION: We establish TNR as a disease gene for an autosomal recessive nonprogressive neurodevelopmental disorder with spasticity and transient opisthotonus and highlight the role of central nervous system extracellular matrix proteins in the pathogenicity of spastic disorders.
Subject(s)
Muscle Spasticity , Neurodevelopmental Disorders , Central Nervous System , Extracellular Matrix , Homozygote , Humans , Muscle Spasticity/genetics , Neurodevelopmental Disorders/geneticsABSTRACT
PURPOSE: To delineate the genotype-phenotype correlation in individuals with likely pathogenic variants in the CLTC gene. METHODS: We describe 13 individuals with de novo CLTC variants. Causality of variants was determined by using the tolerance landscape of CLTC and computer-assisted molecular modeling where applicable. Phenotypic abnormalities observed in the individuals identified with missense and in-frame variants were compared with those with nonsense or frameshift variants in CLTC. RESULTS: All de novo variants were judged to be causal. Combining our data with that of 14 previously reported affected individuals (n = 27), all had intellectual disability (ID), ranging from mild to moderate/severe, with or without additional neurologic, behavioral, craniofacial, ophthalmologic, and gastrointestinal features. Microcephaly, hypoplasia of the corpus callosum, and epilepsy were more frequently observed in individuals with missense and in-frame variants than in those with nonsense and frameshift variants. However, this difference was not significant. CONCLUSIONS: The wide phenotypic variability associated with likely pathogenic CLTC variants seems to be associated with allelic heterogeneity. The detailed clinical characterization of a larger cohort of individuals with pathogenic CLTC variants is warranted to support the hypothesis that missense and in-frame variants exert a dominant-negative effect, whereas the nonsense and frameshift variants would result in haploinsufficiency.
Subject(s)
Epilepsy , Intellectual Disability , Microcephaly , Biological Variation, Population , Corpus Callosum , Epilepsy/genetics , Humans , Intellectual Disability/genetics , Microcephaly/genetics , PhenotypeABSTRACT
BACKGROUND: Respiratory disease is a leading cause of hospitalizations and deaths in young people with cerebral palsy (CP). It is insidious and multifactorial. Clinical management can be complex. This systematic review describes effects of interventions for the prevention and management of respiratory disease in young people with CP. METHODS: Nine databases (Cochrane, CINAHL, Embase, EMCare, MEDLINE, PEDro, OpenGrey, ScienceDirect, and SpeechBITE) were searched. Eligibility criteria were as follows: the population included at least 50% individuals with CP aged under 26 years, the intervention was for chronic respiratory illness, the outcomes were any measurable indicators of respiratory health or morbidity; the study design could be any original study reporting effects of an intervention on measurable outcomes, and the study was published January 1998 or later. The American Academy for Cerebral Palsy and Developmental Medicine methodology guided study appraisal and synthesis. The review was registered with PROSPERO (reference number CRD42018086314). RESULTS: The search yielded 3,347 papers; 37 papers (reporting 34 studies) of these met the eligibility criteria. They included 582 participants with CP (ranging from 1 to 77 across studies) with ages from 5 months to 25 years. Interventions were diverse and included: airway clearance techniques, exercise, positioning, mealtime management, salivary management, upper airway interventions, antibiotics, gastro-intestinal interventions, and spinal surgery. There were no interventions aimed at prevention of respiratory disease in this population. Research designs were classified as Levels 2 (n = 3), 3 (n = 2), 4 (n = 25), and 5 (n = 4). CONCLUSIONS: Evidence for most respiratory interventions for young people with CP is absent or weak. No controlled trials demonstrated significant effects on respiratory morbidity, owing to their scarcity, weak designs, and inadequate power. There is an urgent need for well-designed prospective controlled studies investigating prevention and management of respiratory disease in young people with CP.