The Cleft Collective: protocol for a longitudinal prospective cohort study.

INTRODUCTION: Cleft lip and/or palate (CL/P) affects 1 in 700 live births globally. Children born with CL/P and their families face various challenges throughout the child's development. Extant research is often limited by small numbers and single-centre data. The Cleft Collective, a national cohort study in the UK, aims to build a resource, available to collaborators across the globe, to understand causes, best treatments and long-term outcomes for those born with CL/P, ultimately seeking to enhance their quality of life through improved understanding and care. METHODS AND ANALYSIS: A longitudinal prospective cohort study of children born with CL/P and their families. Recruitment occurs across the UK and started in November 2013. Recruitment will continue until September 2027 with an estimated final sample of 4822 children born with CL/P (1157 cleft lip including/excluding the alveolus; 2112 cleft palate only; 1042 unilateral cleft lip and palate and 511 bilateral cleft lip and palate). Biological samples are collected from all recruited members of the family. Parental and child questionnaires are collected at key time points throughout the child's development. Surgical data are collected at the time of surgical repair of the child's cleft. Consent is obtained to link to external data sources. Nested substudies can be hosted within the cohort. Regular engagement with participants takes place through birthday cards for the children, social media posts and newsletters. Patient and Public Involvement is conducted through the Cleft Lip And Palate Association and Cleft Collective Patient Consultation Group who provide insightful and essential guidance to the Cleft Collective throughout planning and conducting research. ETHICS AND DISSEMINATION: The Cleft Collective was ethically approved by the National Research Ethics Service committee South West-Central Bristol (REC13/SW/0064). Parental informed consent is required for participation. Findings from the Cleft Collective are disseminated through peer-reviewed publications, conference presentations, newsletters and social media.

Outcome measures for children with speech sound disorder: an umbrella review.

OBJECTIVE: Speech sound disorder (SSD) describes a 'persistent difficulty with speech sound production that interferes with speech intelligibility or prevents verbal communication'. There is a need to establish which care pathways are most effective and efficient for children with SSD. Comparison of care pathways requires clearly defined, evidence-based, interventions and agreement on how to measure the outcomes. At present, no definitive list of assessments, interventions or outcomes exists. The objective of this umbrella review paper is to provide a rigorous and detailed list of assessments, interventions and outcomes which target SSD in children. DESIGN: In December 2022, a systematic search of Ovid Medline, OVID Embase, CINAHL, PsycInfo and Cochrane and a number of grey literature platforms were undertaken. 18 reviews were included, and subsequently 415 primary research articles were assessed for data related to assessments, interventions or outcomes. The AMSTAR (Assessing the Methodological Quality of Systematic Reviews) framework was used to assess the quality of the retained reviews. SETTING: Reviews were retained which took place in any setting. PARTICIPANTS: The population is children of any age with a diagnosis of SSD of unknown origin. PRIMARY AND SECONDARY OUTCOME MEASURES: Reviews reporting outcomes, assessment and interventions for children with SSD. RESULTS: Extraction and analysis identified 37 assessments, 46 interventions and 30 outcome measures used in research reporting of SSD. Not all of the listed outcomes were linked to specific outcome measurement tools, but these were measurable through the use of one or more of the assessments extracted from the retained reviews. CONCLUSIONS: The findings of this review will be used to develop a Core Outcome Set for children with SSD. The findings are part of a rigorous process essential for advancing healthcare research and practice in the specific area of speech and language therapy for children with SSD. PROSPERO REGISTRATION NUMBER: CRD42022316284.

Association of Perioperative Antibiotics with the Prevention of Postoperative Fistula after Cleft Palate Repair.

Background: There is debate amongst surgeons regarding the use of antibiotics to prevent fistulae after palatoplasty. Prescribing should be evidence based, as antibiotic stewardship is integral to reducing antibiotic resistance. Our aim was to determine whether differing perioperative regimens affect the prevalence of postoperative fistulae. Methods: The sample comprised participants from the Cleft Collective who had undergone palatoplasty. Participants were recruited across all 16 UK cleft centers between 2013 and 2021. The exposure was perioperative antibiotic regimen prescribed at the time of palatoplasty. The primary outcome was the presence of palatal fistula. Results: Fistula data were available for 167 participants when exploring antibiotic regimen and for 159 when exploring antibiotic agent. There was no evidence to suggest a difference in fistula rate between those receiving antibiotics on induction only versus as an inpatient or up to 7 days postoperatively (χ2 = 4.57; P = 0.10). There was no evidence to suggest a difference in fistula rate between those who received co-amoxiclav and those who had an alternative antibiotic (χ2 = 0.16; P = 0.69). Postoperative fistulae increased with the extent of the cleft (χ2 = 20.39; P < 0.001). When adjusting for cleft type, no evidence of an association between antibiotic regimen and fistulae was found (inpatient antibiotics: OR 1.36; 95% confidence interval, 0.53-3.51; antibiotics up to 7 days postoperatively: OR 0.68; 95% confidence interval, 0.26-1.80). Conclusions: The choice of antibiotic and dosing regimen does not influence the formation of postoperative fistulae. These results should be supported by interventional trials.

Should left- and right-sided unilateral cleft lip and palate patients be grouped together when reporting the outcomes?

OBJECTIVES: Investigate associations between cleft laterality in patients with non-syndromic unilateral cleft lip and palate (UCLP) and oral-health, dental-arch, speech, audiological, psychological and nasolabial-aesthetic outcomes. METHODS: Secondary data analysis of the outcomes of 5-year-old children with non-syndromic complete UCLP identified from three studies: Cleft Collective (n = 155), Cleft Care UK (CCUK) (n = 266) and Clinical Standards Advisory Group (CSAG) study (n = 238). Outcome measures included occlusal assessment using the 5-year-old's index score, speech intelligibility rating using the CAPS-A Audit tool, audiological assessment using pure tone audiometry, nasolabial aesthetic assessment using the Asher-McDade tool, oral-health assessment using decayed, missing, filled teeth scores and parent-reported outcomes. Logistic regression with adjustment for age, sex and index-of-multiple-deprivation scores were performed. RESULTS: No differences were found in patient-reported outcomes between the left and right clefts in the Cleft Collective study. From the CCUK study, right clefts had poorer speech (n = 236; 95% CI 1.09, 3.42; and P = .03) and hearing outcomes (n = 211; 95% CI 1.03, 3.43; P = .04). In the CSAG study, patients with left clefts were more likely to be teased (n = 213; 95% CI 0.26, 0.85; and P = .01). CONCLUSION: Weak associations between cleft laterality, speech, hearing and psychological outcomes were found, however the findings were inconsistent across the studies. This study contributes to evidence of associations between laterality and outcomes in children born with UCLP.

Prevalence and Factors Associated with Behavioral Problems in 5-Year-Old Children Born with Cleft Lip and/or Palate from the Cleft Collective.

OBJECTIVES: To determine the UK prevalence of behavioral problems in 5-year-old children born with isolated or syndromic cleft lip and/or palate (CL/P) compared to the general population and identify potentially associated factors. DESIGN: Observational study using questionnaire data from the Cleft Collective 5-Year-Old Cohort study and three general population samples. MAIN OUTCOME MEASURE: The Strengths and Difficulties Questionnaire (SDQ). PARTICIPANTS: Mothers of children (age: 4.9-6.8 years) born with CL/P (n = 325). UK general population cohorts for SDQ scores were: Millennium Cohort Study (MCS) (n = 12 511), Office of National Statistics (ONS) normative school-age SDQ data (n = 5855), and Avon Longitudinal Study of Parents and Children (ALSPAC) (n = 9386). RESULTS: By maternal report, 14.2% of children born with CL/P were above clinical cut-off for behavioral problems, which was more likely than in general population samples: 7.5% of MCS (OR = 2.05 [1.49-2.82], P < 0.001), 9.8% of ONS (OR = 1.52 [1.10-2.09], P = 0.008), and 6.6% of ALSPAC (OR = 2.34 [1.70-3.24], P < 0.001). Children in the Cleft Collective had higher odds for hyperactivity, emotional and peer problems, and less prosocial behaviors. Maternal stress, lower maternal health-related quality of life and family functioning, receiving government income support, and maternal smoking showed evidence of association (OR range: 4.41-10.13) with behavioral problems, along with maternal relationship status, younger age, and lower education (OR range: 2.34-3.73). CONCLUSIONS: Findings suggest elevated levels of behavioral problems in children born with CL/P compared to the general population with several associated maternal factors similar to the general population.

Sidedness in Unilateral Orofacial Clefts: A Systematic Scoping Review.

OBJECTIVE: An overview of the literature relating to the sidedness of unilateral cleft lip with or without cleft palate to map current knowledge on the cause and impact of directional asymmetry. DESIGN: Scoping review with a systematic search of Medline and Embase from inception to May 2023. PATIENTS, PARTICIPANTS: Humans born with a left or right unilateral cleft lip with or without a cleft palate. MAIN OUTCOME MEASURES: Cleft sidedness as a co-occurrence, an outcome or an exposure. RESULTS: Forty studies were eligible for inclusion and confirmed the predilection for the occurrence of left sided cleft lips; 12 studies reported cleft sidedness co-occurring with another phenotype, 11 studies report sidedness as an outcome and 17 studies as an exposure. Phenotypes which were reported to co-occur with either left or right sided clefts included congenital dental anomalies, handedness and additional congenital anomalies. Variables investigated as a potential cause of left or right sided clefts as an outcome included chromosomal anomalies, genetic variants and environmental factors. Outcomes investigated in relation to cleft sidedness as an exposure included facial anatomical features, facial growth, educational attainment, functional and psychological characteristics. More studies showed worse outcomes in right sided clefts versus left sided clefts than vice versa, although studies were inconsistent, and a quality assessment was not performed. CONCLUSIONS: The field of cleft sidedness research is expanding and there are promising early findings to differentiate cause and outcome by sidedness of the cleft.

Speech sound disorder or DLD (phonology)? Towards a consensus agreement on terminology.

BACKGROUND: The publication of phase 2 of the CATALISE project in 2017 clarified terminology for children with developmental language disorder (DLD) or delay but unintentionally muddied the water for children with unintelligible speech. A diagnostic label of DLD (phonology) indicates poor prognosis and phonological disorder that persists into middle childhood. However, in contrast to other diagnostic labels that fall under the overarching term of speech sound disorder (SSD), DLD (phonology) does not elucidate the characteristics of the child's speech nor does it point us in the direction of appropriate intervention. AIMS: The aim of this paper is to discuss terminology in SSD leading to an evidence-based model which builds on the model of DLD developed in CATALISE, supports descriptive diagnosis and signposts intervention. METHODS: Following a focused review of literature proposing or describing terminology for SSD, an expert group of researchers in developmental SSD proposed a revised model of existing terminology. Groups of UK speech and language therapists (SLTs) who provide services for children with SSD were asked to comment on its acceptability and feasibility. DISCUSSION: A three-level terminology model was developed. This comprised an overarching Level 1 term; Level 2 terms that differentiated SSD of unknown origin from SSD with associated or underlying conditions; and specific diagnostic terms at Level 3 to support further assessment and intervention decisions. Consulted SLTs generally expressed agreement with the proposed terminology and a willingness to adopt it in practice. CONCLUSIONS: Existing terminology for childhood SSD provides a good basis for clinical decision-making. A modified version of Dodd's (2005) terminology was found to be acceptable to UK SLTs. There is an evident overlap of SSD with CATALISE terminology. However more detailed and specialist terminology than 'DLD (phonology)' is required to support clinical decision-making. It is proposed that endorsement by the UK Royal College of Speech and Language Therapists would obviate the need for a Delphi process. WHAT THIS PAPER ADDS: What is already known on this subject Over nearly a hundred years, as our knowledge and understanding of speech sound disorder (SSD) has increased, so has the terminology that is used to describe those disorders. Current terminology not only describes subtypes of SSD but can also signpost us to effective interventions. With the publication, in 2017, of phase 2 of CATALISE a new term of 'developmental language disorder (DLD) (phonology)' was introduced with the unintentional consequence of challenging more specific descriptive terms for SSD. What this paper adds In the context of CATALISE and DLD (phonology), the history and nature of SSD terminology are reappraised. Building on the model of DLD developed in CATALISE, a tiered model that supports descriptive diagnosis and signposts intervention is proposed for discussion. Clinical implications of this study The proposed model of terminology for SSD provides descriptive and detailed labels that will support accuracy in differential diagnosis of developmental SSD by speech and language therapists. Furthermore, a decision-making tree for SSD demonstrates the pathway from diagnostic use of the terminology to the selection of evidence-based, effective interventions.

Patient Factors Influencing Speech Outcomes in Velopharyngeal Function Following Initial Cleft Palate Repair: A Systematic Review and Meta-Analysis.

OBJECTIVE: Identification of patient factors influencing velopharyngeal function for speech following initial cleft palate repair. DESIGN: A literature search of relevant databases from inception until 2018 was performed using medical subject headings and keywords related to cleft palate, palatoplasty and speech assessment. Following three stage screening data extraction was performed. SETTING: Systematic review and meta-analysis of relevant literature. PATIENTS/PARTICIPANTS: Three hundred and eighty-three studies met the inclusion criteria, comprising data on 47 658 participants. INTERVENTIONS: Individuals undergoing initial palatoplasty. MAIN OUTCOME MEASURES: Studies including participants undergoing initial cleft palate repair where the frequency of secondary speech surgery and/or velopharyngeal function for speech was recorded. RESULTS: Patient factors reported included cleft phenotype (95% studies), biological sex (64%), syndrome diagnosis (44%), hearing loss (28%), developmental delay (16%), Robin Sequence (16%) and 22q11.2 microdeletion syndrome (11%). Meta-analysis provided strong evidence that rates of secondary surgery and velopharyngeal dysfunction varied according to cleft phenotype (Veau I best outcomes, Veau IV worst outcomes), Robin Sequence and syndrome diagnosis. There was no evidence that biological sex was associated with worse outcomes. Many studies were poor quality with minimal follow-up. CONCLUSIONS: Meta-analysis demonstrated the association of certain patient factors with speech outcome, however the quality of the evidence was low. Uniform, prospective, multi-centre documentation of preoperative characteristics and speech outcomes is required to characterise risk factors for post-palatoplasty velopharyngeal insufficiency for speech. SYSTEMATIC REVIEW REGISTRATION: Registered with PROSPERO CRD42017051624.

Social, emotional and behavioural difficulties associated with persistent speech disorder in children: A prospective population study.

Purpose: Social, emotional and behavioural difficulties (SEBD) in childhood are associated with negative consequences across the life course. Children with developmental language disorder have been identified as being at risk of developing SEBD but it is unclear whether a similar risk exists for children with speech sound disorder, a condition which impacts on children's ability to make themselves understood and has been shown to be associated with poor educational outcomes. Methods: Participants were children who attended the 8-year-old clinic in the Avon Longitudinal Study of Parents and Children (N = 7390). Children with speech sound disorder that had persisted beyond the period of typical speech acquisition (persistent speech disorder [PSD]) at age 8 were identified from recordings and transcriptions of speech samples (N = 263). Parent-, teacher- and child-reported questionnaires and interviews including the Strengths and Difficulties Questionnaire, Short Moods and Feelings Questionnaire and measures for antisocial and risk-taking behaviour were used to provide outcome scores for SEBD at 10-14 years in a series of regression analyses. Results: Following adjustment for biological sex, socio-economic status and Intelligence Quotient, children with PSD at age 8 were more likely to show peer problems at age 10-11 years compared with their peers, as reported by teachers and parents. Teachers were more likely to report problems with emotionality. Children with PSD were no more likely to report symptoms of depression than their peers. No associations were observed between PSD, risk of antisocial behaviour, trying alcohol at age 10 or smoking cigarettes at age 14. Conclusions: Children with PSD may be at risk in terms of their peer relationships. This could impact on their wellbeing and, while not observed at this age, may lead to depressive symptoms in older childhood and adolescence. There is also the potential that these symptoms may impact on educational outcomes.

Understanding the prevalence and manifestation of anxiety and other socio-emotional and behavioural difficulties in children with Developmental Language Disorder.

BACKGROUND: It is well-documented that children with Developmental Language Disorder (DLD) have a higher likelihood of experiencing anxiety, as well as other socio-emotional and behavioural (SEB) difficulties. Despite this, there is little consensus as to how these difficulties manifest. This study aims to understand the prevalence of broader SEB difficulties and anxiety, informing intervention development by understanding the relationships between them. METHODS: A mixed-methods, case-control study was conducted. First, an online survey was completed by 107 parents of either children with DLD ("DLD sample"; n = 57) or typically developing children ("typical sample"; n = 50), aged 6-12 years old. Binary SEB statements informed by previous qualitative work (e.g. "my child requires routine/sameness"; "my child has frequent tantrums") provided an insight into the prevalence of SEB difficulties in both DLD and typical samples. Validated measures of anxiety, emotion regulation, intolerance of uncertainty, insistence on sameness, family stress and coping mechanisms were also collected. Correlation and mediation analyses were run using these validated measures to understand the manifestation of anxiety in children with DLD in more detail. Qualitative interviews were then carried out with a select panel of survey respondents (n = 4). RESULTS: The DLD sample scored significantly higher on all binary SEB statements than the typical sample: experiencing anxiety (80.7%, p < .05), requiring routine and sameness (75.4%, p < .001) and emotional dysregulation (75.4%; p < .001) were the most common difficulties reported for children with DLD. Using the validated scales, family stress and coping mechanisms were found to only correlate with the manifestation of anxiety in the typical group, not the DLD group. "Intolerance of uncertainty" and "insistence on sameness" were found to fully mediate the relationship between DLD diagnosis and symptoms of anxiety. Parent's interviews provided contextual support for the analysis, as well as highlighting sensory sensitivities as a focus for future research. CONCLUSIONS: Parents of children with DLD appear to cope well with their children's complex SEB needs. Intervention focussing on intolerance of uncertainty may help the management of difficulties with anxiety. Behaviours such as insistence on sameness should be investigated further, as potential indicators for anxiety amongst children with DLD.

Exploring the Psychosocial Experiences of Individuals with Developmental Language Disorder During Childhood: A Qualitative Investigation.

Children with Developmental Language Disorder (DLD) often experience co-occurring psychosocial difficulties, the developmental trajectories of which are still not fully understood. This study sought to explore the manifestation of such difficulties during childhood, through first-hand accounts of those with DLD and their close relatives. Individual semi-structured interviews were conducted with 11 mothers of children with DLD (aged 6-12 years old) and were analysed alongside the secondary data from interviews of five adults with DLD. Interviews were conducted online; all participants resided in Europe and were fluent in spoken and written English. A process of interpretive phenomenological analysis resulted in the development of five overall themes: experiencing anxiety, social frustrations, maintaining factors, childhood strengths and the parenting experience. Cognitive appraisals appeared particularly important during childhood in both escalating and maintaining anxiety, low self-esteem, emotion dysregulation and social frustrations. High levels of isolation and stress were experienced by all mothers. The findings suggest parents in the United Kingdom and Ireland require more support and guidance at the point of diagnosis than is currently provided. Emphasis was given to the link between children's experience of anxiety and social behaviours, such as withdrawal, as well as their intolerance of uncertainty. Internalising symptoms were a prioritisation for intervention during childhood by both parents and adults with DLD.

The development of a digital story-retell elicitation and analysis tool through citizen science data collection, software development and machine learning.

Background: In order to leverage the potential benefits of technology to speech and language therapy language assessment processes, large samples of naturalistic language data must be collected and analysed. These samples enable the development and testing of novel software applications with data relevant to their intended clinical application. However, the collection and analysis of such data can be costly and time-consuming. This paper describes the development of a novel application designed to elicit and analyse young children's story retell narratives to provide metrics regarding the child's use of grammatical structures (micro-structure) and story grammar (macro-structure elements). Key aspects for development were (1) methods to collect story retells, ensure accurate transcription and segmentation of utterances; (2) testing the reliability of the application to analyse micro-structure elements in children's story retells and (3) development of an algorithm to analyse narrative macro-structure elements. Methods: A co-design process was used to design an app which would be used to gather story retell samples from children using mobile technology. A citizen science approach using mainstream marketing via online channels, the media and billboard ads was used to encourage participation from children across the United Kingdom. A stratified sampling framework was used to ensure a representative sample was obtained across age, gender and five bands of socio-economic disadvantage using partial postcodes and the relevant indices of deprivation. Trained Research Associates (RA) completed transcription and micro and macro-structure analysis of the language samples. Methods to improve transcriptions produced by automated speech recognition were developed to enable reliable analysis. RA micro-structure analyses were compared to those generated by the digital application to test its reliability using intra-class correlation (ICC). RA macro-structure analyses were used to train an algorithm to produce macro-structure metrics. Finally, results from the macro-structure algorithm were compared against a subset of RA macro-structure analyses not used in training to test its reliability using ICC. Results: A total of 4,517 profiles were made in the app used in data collection and from these participants a final set of 599 were drawn which fulfilled the stratified sampling criteria. The story retells ranged from 35.66 s to 251.4 s in length and had word counts ranging from 37 to 496, with a mean of 148.29 words. ICC between the RA and application micro-structure analyses ranged from 0.213 to 1.0 with 41 out of a total of 44 comparisons reaching 'good' (0.70-0.90) or 'excellent' (>0.90) levels of reliability. ICC between the RA and application macro-structure features were completed for 85 samples not used in training the algorithm. ICC ranged from 0.5577 to 0.939 with 5 out of 7 metrics being 'good' or better. Conclusion: Work to date has demonstrated the potential of semi-automated transcription and linguistic analyses to provide reliable, detailed and informative narrative language analysis for young children and for the use of citizen science based approaches using mobile technologies to collect representative and informative research data. Clinical evaluation of this new app is ongoing, so we do not yet have data documenting its developmental or clinical sensitivity and specificity.

The Association Between Atypical Speech Development and Adolescent Self-Harm.

BACKGROUND: Adolescent self-harm is a major public health issue internationally. Various factors associated with adolescent self-harm have been identified, including being bullied and experiencing mental health problems. Stuttering and speech sound disorder are associated with both of these factors. It was hypothesized that both stuttering and speech sound disorder would be associated with self-harm. This is the first study to explore the relationship between communication disorders and adolescent self-harm. METHOD: Secondary analysis of a large, longitudinal, prospective, community sample, the Avon Longitudinal Study of Parents and Children, was carried out. Clinicians identified children who stuttered or exhibited speech sound disorder at the age of 8 years. When the cohort members were 16 years old, they were asked to complete a questionnaire about self-harm. Multinomial logistic regression was used to examine the associations between stuttering and speech sound disorder and the self-harm outcomes, adjusting for other relevant factors. RESULTS: Of 3,824 participants with data for both speech status and self-harm, 94 (2.5%; 95% confidence interval [CI; 2.0, 3.0]) stuttered at 8 years of age and 127 (3.3%; 95% CI [2.8, 3.9]) displayed speech sound disorder. Speech sound disorder at the age of 8 years was associated with self-harm with suicidal intent in both unadjusted and adjusted models. Differences between the adjusted and unadjusted models were small, suggesting that speech sound disorder is largely an independent risk factor for self-harm with suicidal intent. Stuttering at the age of 8 years was not associated with adolescent self-harm, and there was no association between speech sound disorder and self-harm without suicidal intent. CONCLUSION: Compared with individuals without speech sound disorder, adolescents with speech sound disorder at the age of 8 years have twice the risk of reporting self-harm with suicidal intent, even when other important predictors are taken into account. SUPPLEMENTAL MATERIAL: https://doi.org/10.23641/asha.22573030.

Outcome measures for children with speech sound disorder: an umbrella review protocol.

INTRODUCTION: Speech sound disorder (SSD) describes a 'persistent difficulty with speech sound production that interferes with speech intelligibility or prevents verbal communication'. There is a need to establish which care pathways are most effective and efficient for children with SSD. Comparison of care pathways requires clearly defined, evidence-based interventions and agreement on how to measure the outcomes. At present, no list of assessments, interventions or outcomes exists.The objective of this paper is to provide a rigorous and detailed protocol for an umbrella review of assessments, interventions and outcomes that target SSD in children. The protocol details the development of a search strategy and trial of an extraction tool. METHODS AND ANALYSES: The umbrella review has been registered with PROSPERO (CRD42022316284). Papers included can use a review methodology of any sort but must include children of any age, with an SSD of unknown origin. In accordance with the Joanna Briggs Institute scoping review methods guidelines, an initial search of the Ovid Emcare and Ovid Medline databases was conducted. Following this, a final search strategy for these databases were produced. A draft extraction form was developed. ETHICS AND DISSEMINATION: Ethical approval is not needed for an umbrella review protocol. Following the systematic development of an initial search strategy and extraction form, an umbrella review of this topic can take place. Dissemination of findings will be through peer-reviewed publications, social media, and patient and public engagement.

Current Surgical Practice for Children Born with a Cleft lip and/or Palate in the United Kingdom.

OBJECTIVE: This study describes primary surgical reconstructions performed for children born with a cleft lip and/or palate (CL ± P) in the United Kingdom (UK). DESIGN: Data forms completed at the time of surgery included details on timing, technique, and adjuncts used during the operative period. Demographic data on participants were validated via parental questionnaires. SETTING: Data were obtained from the Cleft Collective, a national longitudinal cohort study. PATIENTS: Between 2015 and 2021, 1782 Cleft Collective surgical forms were included, relating to the primary reconstructions of 1514 individual children. RESULTS: The median age at primary cheiloplasty was 4.3 months. Unilateral cleft lips (UCL) were reconstructed with an anatomical subunit approximation technique in 53%, whereas bilateral cleft lips (BCL) were reconstructed with a broader range of eponymous techniques. Clefts of the soft palate were reconstructed at a median age of 10.3 months with an intravelar veloplasty in 94% cases. Clefts of the hard palate were reconstructed with a vomer flap in 84% cases in a bimodal age distribution, relating to reconstruction carried out simultaneously with either lip or soft palate reconstruction. Antibiotics were used in 96% of cases, with an at-induction-only regimen used more commonly for cheiloplasties (P < .001) and a 5 to 7-day postoperative regime used more commonly for soft palatoplasties (P < .001). Perioperative steroids were used more commonly in palatoplasties than cheiloplasties (P < .001) but tranexamic acid use was equivalent (P = .73). CONCLUSIONS: This study contributes to our understanding of current cleft surgical pathways in the UK and will provide a baseline for analysis of the effectiveness of utilized protocols.

Syndromes associated with Robin sequence: a national prospective cohort study.

OBJECTIVES: To determine the prevalence of syndromic Robin sequence (RS) in the UK and if this group of patients had an increased need for airway and feeding management compared with a non-syndromic RS cohort. DESIGN: A prospective national multicentre study of cases submitted to the Cleft Collective cohort studies. SETTING: Specialist cleft services in the UK. PATIENTS: 259 participants who fulfilled the diagnosis of RS. This group was compared with 548 participants with cleft palate only (CPO). MAIN OUTCOME MEASURES: The primary outcome measure was the presence of a syndrome in patients with RS and CPO. Secondary outcome measures included the use of airway and feeding adjuncts. RESULTS: An associated syndrome was seen in 28% of patients with RS and 14% of patients with CPO. The most common syndrome for the RS group was Stickler syndrome (27%). Syndromic status was significantly higher among patients with RS compared with those with CPO (OR 2.36, 95% CI 1.65 to 3.39; p<0.001). Patients with syndromic RS have an increased reliance on airway adjuncts compared with the patients without syndromic RS (OR 2.02, 95% CI 1.13 to 3.64; p=0.018). There was no evidence of a difference in the use of feeding adjuncts between syndromic and non-syndromic RS groups (OR 2.43, 95% CI 0.78 to 7.58; p=0.126). CONCLUSION: The presence of a syndrome has implications for management of patients with RS. Early identification of a syndrome may help prevent the consequences of a missed syndromic diagnosis. Routine ophthalmological and genetic screening for Stickler syndrome should be mandatory for all patients with RS.

The Fluency Trust Residential Course for young people who stutter: A pragmatic feasibility study.

INTRODUCTION: A feasibility study of The Fluency Trust Residential Course (FTRC) for adolescents who stutter was conducted. The study aimed to measure key areas of a feasibility trial, for example, recruitment and retention, outcome measure completion, outcome measure reliability, and acceptability of the intervention to inform future research into the FTRC. METHODS: Quantitative and qualitative methods were used. Participants were 23 adolescents (12-17 years), 23 parents and 2 Speech-Language Pathologists (SLPs) from the FTRC. Data collection included: outcome measure collection via a pre-test post-test quasi-experimental design (including two baseline measures), intervention fidelity checklists, semi-structured interviews with adolescents to explore acceptability of the intervention and semi-structured interviews with SLPs to explore their experiences of research participation and views on a future trial. RESULTS: Recruitment, retention and outcome measure completion levels were all 100%. Intervention fidelity was 95% and there were no adverse events. Outcome measures showed good test- re-test reliability: Progress Questionnaire Child Intraclass Correlation Coefficient (ICC) = 0.87 (95% CI = 0.69-0.94 sig< 0.001) and Progress Questionnaire Parent ICC = 0.88 (95% CI = 0.70-0.95 sig< 0.001). Descriptive statistics showed that group medians and means of all outcome measures shifted in a positive direction between pre and post-tests (9 weeks follow-up). Twenty-five percent of young people showed changes on the Progress Questionnaire Child that were above the minimal important difference. Seventy-five percent of parents showed changes on the Progress Questionnaire Parent that were above the minimal important difference. Acceptability of the intervention by adolescents was high. SLPs reported participation was manageable and they were pleased to be part of the research. CONCLUSION: Quantitative and qualitative data suggest that a future definitive trial of the FTRC is indicated after additional development work and feasibility testing. Recommendations for further research are included.

Maternal Stressful Life Events During the Periconceptional Period and Orofacial Clefts: A Systematic Review and Meta-Analysis.

OBJECTIVE: To assess whether women who experience stressful life events during the periconceptional period are at higher risk of giving birth to a baby with an orofacial cleft (OFC). DESIGN: Systematic review and meta-analysis of studies reporting the proportion of babies born with OFC to mothers exposed and unexposed to population-level or personal-level stressful life events during the periconceptional period. Six electronic databases were searched from inception to August 2020. Risk of bias was assessed using the Newcastle-Ottawa scale. Odds ratios (ORs) for the odds of OFC in babies of exposed mothers relative to unexposed controls were extracted and/or calculated. Random effects meta-analysis was undertaken, stratified by cleft subtype. RESULTS: Of 12 eligible studies, 8 examined experience of personal events and 4 examined population-level events. Studies demonstrated low-moderate risk of bias and there was indication of publication bias. There was some evidence that personal stressful life events were associated with greater odds of cleft lip and/or palate (six studies, OR 1.63, 95% confidence interval (CI) 1.16, 2.30, P = 0.001) and cleft palate only (six studies, OR 1.45, 95% CI 1.02, 2.06, P = 0.04). Population-level events were associated with higher odds of OFC in studies that did not specify subtype (three studies, OR 1.64, 95% CI 1.19, 2.25, P = 0.002), but subtype stratified analyses were underpowered. Heterogeneity was high. CONCLUSIONS: Limited evidence indicated a weak positive association between maternal stressful life events during the periconceptional period and risk of OFC in the offspring, but further studies with greater consistency in research design are needed.

Parent/Caregiver Views of the Effectiveness of Speech-Language Pathology for Children Born With Cleft Palate Delivered via Telemedicine During COVID-19.

PURPOSE: The purpose of this study was to describe and examine parent views of speech-language pathology (SLP) for children born with cleft palate delivered via telemedicine during the COVID-19 pandemic in the United Kingdom (UK). METHOD: Parents were asked whether they found this method of delivery "very effective," "somewhat effective," or "not at all effective." Free text was then invited. There were 212 responses. Ordinal chi-square, Kruskal-Wallis, or Fisher's exact tests examined associations between parent views of effectiveness and biological variables and socioeconomic status. Free text responses were analyzed using qualitative content analysis. RESULTS: One hundred and forty (66.0%) respondents reported that SLP delivered via telemedicine was "somewhat effective," 56 (26.4%) "very effective," and 16 (7.6%) "not at all effective." There was no evidence of an association between parent reported effectiveness and any of the explanatory variables. Parent-reported challenges impacting on effectiveness included technology issues and keeping their children engaged with sessions. Importantly, telemedicine was viewed as "better than nothing." CONCLUSIONS: Most parents reported that they felt SLP delivered via telemedicine during the first few months of the COVID-19 pandemic in the UK was at least "somewhat effective." It is important to interpret this in the context of there being no other method of service delivery during this time and that this study only represents families who were able to access SLP delivered via telemedicine. Further work is needed to identify which children with cleft palate might benefit from SLP delivered via telemedicine to inform postpandemic service provision.