Timing of Primary Surgery for Cleft Palate.

BACKGROUND: Among infants with isolated cleft palate, whether primary surgery at 6 months of age is more beneficial than surgery at 12 months of age with respect to speech outcomes, hearing outcomes, dentofacial development, and safety is unknown. METHODS: We randomly assigned infants with nonsyndromic isolated cleft palate, in a 1:1 ratio, to undergo standardized primary surgery at 6 months of age (6-month group) or at 12 months of age (12-month group) for closure of the cleft. Standardized assessments of quality-checked video and audio recordings at 1, 3, and 5 years of age were performed independently by speech and language therapists who were unaware of the trial-group assignments. The primary outcome was velopharyngeal insufficiency at 5 years of age, defined as a velopharyngeal composite summary score of at least 4 (scores range from 0 to 6, with higher scores indicating greater severity). Secondary outcomes included speech development, postoperative complications, hearing sensitivity, dentofacial development, and growth. RESULTS: We randomly assigned 558 infants at 23 centers across Europe and South America to undergo surgery at 6 months of age (281 infants) or at 12 months of age (277 infants). Speech recordings from 235 infants (83.6%) in the 6-month group and 226 (81.6%) in the 12-month group were analyzable. Insufficient velopharyngeal function at 5 years of age was observed in 21 of 235 infants (8.9%) in the 6-month group as compared with 34 of 226 (15.0%) in the 12-month group (risk ratio, 0.59; 95% confidence interval, 0.36 to 0.99; P = 0.04). Postoperative complications were infrequent and similar in the 6-month and 12-month groups. Four serious adverse events were reported (three in the 6-month group and one in the 12-month group) and had resolved at follow-up. CONCLUSIONS: Medically fit infants who underwent primary surgery for isolated cleft palate in adequately resourced settings at 6 months of age were less likely to have velopharyngeal insufficiency at the age of 5 years than those who had surgery at 12 months of age. (Funded by the National Institute of Dental and Craniofacial Research; TOPS ClinicalTrials.gov number, NCT00993551.).

Inter-centre comparison of data on surgery and speech outcomes at 5 years of age based on the Swedish quality registry for patients born with cleft palate with or without cleft lip.

BACKGROUND: The objective of the Swedish cleft lip and palate registry (CLP registry) is to promote quality control, research and improvement of treatment, by the comparison of long-term results. The aim was to compare data from the CLP registry among the six treatment centres, regarding data on surgery and speech outcomes at 5 years of age. METHODS: The participants were 430 children born in Sweden from 2009 to 2014, with cleft palate with or without cleft lip and without known syndromes and/or additional malformations. The number of primary and secondary palatal surgeries up to 5 years of age, timing of the last primary palatal surgery, percentage consonants correct, percentage non-oral speech errors and perceived velopharyngeal competence at 5 years were assessed. Multivariable binary logistic regression adjusted for sex and cleft type was used to compare results between the six centres. RESULTS: At one centre (centre 4), the palate was closed in one to three stages, and at the remaining centres in one or two stages. At centre 4, more children underwent a higher number of palatal surgeries, and the last primary palatal surgery was performed at a higher age. Children in centre 4 were also less likely to achieve ≥86% correct consonants (OR = 0.169, P = < 0.001), have no non-oral speech errors (OR = 0.347, P = < 0.001), or have competent or marginally incompetent velopharyngeal competence (OR = 0.244, P = < 0.001), compared to the average results of the other centres. No clear association between patient volume and speech outcome was observed. CONCLUSIONS: The results indicated the risk of a negative speech result if the last primary palatal surgery was performed after 25 months of age. Whether the cleft in the palate was closed in one or two stages did not affect speech outcome. The Swedish CLP registry can be used for open comparisons of treatment results to provide the basis for improvements of treatment methods. If deviating negative results are seen consistently at one centre, this information should be acted upon by further investigation and analysis, making changes to the treatment protocol as needed.

Speech in a consecutive series of children born with cleft lip and palate with and without syndromes and/or additional malformations.

BACKGROUND: When evaluating speech in children with cleft palate with or without cleft lip (CP/L), children with known syndromes and/or additional malformations (CP/L+) are usually excluded. The aim of this study was to present speech outcome of a consecutive series of 5-year-olds born with CP/L, and to compare speech results of children with CP/L + and children with CP/L without known syndromes and/or additional malformations (CP/L-). METHODS: One hundred 5-year-olds (20 with CP/L+; 80 with CP/L-) participated. All children were treated with primary palatal surgery in one stage with the same procedure for muscle reconstruction. Three independent judges performed phonetic transcriptions and rated perceived velopharyngeal competence from audio recordings. Based on phonetic transcriptions, percent consonants correct (PCC) and percent non-oral errors were investigated. Group comparisons were performed. RESULTS: In the total group, mean PCC was 88.2 and mean percent non-oral errors 1.5. The group with bilateral cleft lip and palate (BCLP) had poorer results on both measures compared to groups with other cleft types. The average results of PCC and percent non-oral errors in the CP/L + group indicated somewhat poorer speech, but no significant differences were observed. In the CP/L + group, 25 % were judged as having incompetent velopharyngeal competence, compared to 15 % in the CP/L- group. CONCLUSIONS: The results indicated relatively good speech compared to speech of children with CP/L in previous studies. Speech was poorer in many children with more extensive clefts. No significant differences in speech outcomes were observed between CP/L + and CP/L- groups.

Internationally adopted children with and without a cleft lip and palate showed no differences in language ability at school-age.

AIM: The aim of this study was to investigate language ability in internationally adopted children aged 7-8 years with and without a unilateral cleft lip and palate. METHODS: We compared 27 internationally adopted children with a unilateral cleft lip and palate, adopted from China, with a group of 29 children without a cleft lip and palate, adopted from different countries. Participants were recruited from two cleft lip and palate teams in Sweden and through adoption organisations. Assessments were performed using standardised tests of speech and of receptive and expressive language ability. In addition, a parental questionnaire in which speech, language and communication aspects were rated was used. RESULTS: There were no significant differences in language ability between the groups. The only difference was related to speech ability, where the internationally adopted children with unilateral cleft lip and palate scored significantly lower. However, a high proportion of children in both groups scored low on measures of expressive language compared with test norms. CONCLUSION: The results suggested that having a cleft lip and palate did not increase the risk of language difficulties. Instead, being internationally adopted may be associated with a risk of delayed language development lasting for several years post-adoption.

Scores of the Cleft Hearing, Appearance and Speech Questionnaire (CHASQ) in Swedish Participants With Cleft lip and/or Cleft Palate and a Control Population.

OBJECTIVE: The primary aim of this study was to investigate whether there was any difference in scores of the Cleft Hearing, Appearance and Speech Questionnaire (CHASQ) between patients with cleft lip and/or cleft palate (CL/P) and a control population. The second aim was to compare CL/P and control population scores in this study with a British norm CL/P population. DESIGN: Single-site, cross-sectional study with an age-matched control population. SETTING: Participants were recruited from a hospital, a school, and a sports club. They answered the CHASQ in the hospital or at home. PARTICIPANTS: Sixty-four participants with CL/P (7-19 years of age) and a control population of 56 participants without CL/P (9-20 years of age). MAIN OUTCOME MEASURE: CHASQ. RESULTS: There was no statistically significant difference in satisfaction with cleft-related features between the CL/P and the control population. Participants with CL/P were significantly more satisfied with non-cleft-related features than the control population. Cleft Hearing, Appearance and Speech Questionnaire scores were also similar to earlier established British normative data of a CL/P population. CONCLUSION: The results indicated that children and young people with CL/P were as satisfied with their appearance, hearing, and speech as children and young people without CL/P. Swedish CHASQ scores were also similar to British scores.

Comparison of Corresponding Scores From the Cleft Hearing Appearance and Speech Questionnaire (CHASQ) and CLEFT-Q in Swedish Patients With Cleft Lip and/or Palate.

OBJECTIVE: The primary aim of this study was to compare corresponding scores between 2 existing cleft-specific patient-reported outcome measures (PROMs)-Cleft Hearing Appearance and Speech Questionnaire (CHASQ) and CLEFT-Q. The second aim of the study was to investigate patient opinion on the 2 PROMs. DESIGN: Cross-sectional questionnaire study. SETTING: Participants were recruited from a University Hospital. They answered CHASQ and CLEFT-Q either in the hospital or at home. PARTICIPANTS: Thirty-three participants with cleft lip and/or palate, aged 10 to 19 years. MAIN OUTCOME MEASURE: CHASQ and CLEFT-Q. RESULTS: The CHASQ scores and the corresponding CLEFT-Q scores on appearance correlated significantly. Corresponding scores regarding speech did not correlate significantly. A majority, 15 (58%) participants, answered that they liked CLEFT-Q more than CHASQ, 18 participants (69%) thought CHASQ was easier to complete, and 19 (76%) thought CLEFT-Q would better inform health care professionals. CONCLUSION: Both instruments showed strengths and limitations. Clinicians will have to consider each instrument's respective qualities when choosing to implement either PROM.

Coverage, reporting degree and design of the Swedish quality registry for patients born with cleft lip and/or palate.

BACKGROUND: The objective of the Swedish cleft lip and palate (CLP) registry is to promote quality control, research and improvement of treatment, by comparison of the long-term results of surgery, orthodontics and speech from all six Swedish CLP centres. The purpose of the study was to investigate the coverage and reporting degree of the Swedish CLP registry, and to describe the design of the registry and discuss questions of reliability and validity of the data included. METHODS: All six Swedish CLP centres participate in the registry. All children in Sweden with cleft lip and/or cleft palate, born from 2009 onwards, are included in the registry. Baseline data such as cleft type (ICD-10 diagnosis), heredity, birth weight and additional deformities and/or syndromes, as well as pre-surgical treatment, are recorded at first visit. Data on surgical treatment are recorded continuously. Treatment outcome regarding dentofacial development and speech are recorded at follow-ups at 5, 10, 16 and 19 years of age. Data on dentofacial development are also recorded 1 year after orthognathic surgery. In addition, data on babbling and speech are recorded at 18 months of age. Coverage degree and reporting degree of surgery was assessed by comparison with registrations in the Swedish Central patient registry. Reporting degree of orthodontic and speech registrations at 5 years of age was assessed by comparison with registrations at baseline. RESULTS: The average coverage degree for children born 2009 to 2018 was 95.1%. For cleft-related surgeries, the average reporting degree was 92.4%. Average reporting degree of orthodontic registrations and speech registrations at age 5 years was 92 and 97.5% respectively. CONCLUSION: In order to achieve valid and reliable data in a healthcare quality registry, the degree of coverage and reporting needs to be high, the variables included should be limited and checked for reliability, and the professionals must calibrate themselves regularly. The Swedish CLP registry fulfils these requirements.

Reliability of Speech Variables and Speech-Related Quality Indicators in the Swedish Cleft Lip and Palate Registry.

OBJECTIVE: To assess the reliability of speech variables and speech-related quality indicators in the Swedish quality registry for cleft lip and palate (CLP). DESIGN: Retrospective study. SETTING: Primary care university hospitals. PARTICIPANTS: Fifty-two 5-year-old children with unilateral CLP and 41 with bilateral CLP. MAIN OUTCOME MEASURES: Registry data for "percent nonoral errors" and "perceived velopharyngeal competence" (VPC) were compared to reassessments by 4 independent judges based on audio recordings. Interjudge agreement for "percent consonants correct" (PCC) and the reliability of 3 quality indicators were also assessed. Agreement was calculated with single measures intraclass correlation coefficient (ICC) for articulation outcomes, quadratic weighted κ and ICC for VPC, and percentage agreement and κ for quality indicators. RESULTS: When the agreement between registry data and the judges' reassessments was assessed, the ICC was 0.79 for percent nonoral errors. For VPC, the κ coefficient was 0.66 to 0.75 and the ICC was 0.73. Interjudge agreement for PCC calculated with ICC was 0.85. For the quality indicator "proportion of children with ≥86% correct consonants," all 4 judges were in agreement for 72% of the cases. For "proportion of children without nonoral speech errors" and "proportion of children with competent or marginally incompetent velopharyngeal function," the agreement between registry data and the 4 judges was 89% and 85%, respectively. CONCLUSIONS: The results indicate that registry data on PCC, percent nonoral errors, VPC, and the quality indicators "proportion of children without nonoral speech errors" and "proportion of children with competent or marginally incompetent velopharyngeal function" are reliable.

Validation of reported dentoalveolar relationships in the Swedish Quality Registry for Cleft Lip and Palate.

OBJECTIVES: The present study validated data that had been reported to the Swedish Quality Registry for Cleft Lip and Palate (CLP) under new requirements from 2016, when use of the 5-year-old (5YO) and the Modified Huddart and Bodenham (MHB) indices for rating occlusion in children born with unilateral CLP (UCLP) was introduced. MATERIALS AND METHODS: The sample included blinded study casts (n = 97) and photos (n = 4) of 5-year-old children who had been born with UCLP in 2009-2011 and were enrolled at one of six cleft centres in Sweden. Fourteen orthodontists from the centres assessed the patients (n = 101) using the 5YO and the MHB indices. Median 5YO and MHB scores of the 14 assessments were compared with original registry data (n = 61). Each centre devised code keys to protect the identities of their patients in the registry. RESULTS: Interrater agreement among the 14 orthodontists was good for the 5YO index (quadratic-weighted kappa: 0.72-0.92) and the MHB index (intraclass correlation coefficient: 0.991-0.994). Comparisons of median 5YOs for each identifiable child with their registry data (n = 61) found total agreement for 70.5 per cent. Comparisons between median MHBs and registry data showed very good or good agreement in 93.4 per cent of the cases. LIMITATIONS: Two teams lost their code keys, which reduced the sample to 61 patients. CONCLUSIONS: The dentoalveolar outcome data in the CLP registry was trustworthy. There was good agreement among the Swedish cleft teams assessing the 5YO and MHB indices in children born with UCLP at age 5 years.

Problems With Reliability of Speech Variables for Use in Quality Registries for Cleft Lip and Palate-Experiences From the Swedish Cleft Lip and Palate Registry.

OBJECTIVE: To test the reliability of the speech data in the Swedish quality registry for cleft lip and palate. DESIGN: Retrospective study. SETTING: Primary care university hospital. PARTICIPANTS: Ninety-four children born with cleft palate with or without cleft lip between 2005 and 2009 who had been assessed and registered in the quality registry at the age of 5 years. MAIN OUTCOME MEASURES: Data in the registry on percent oral consonants correct, percent oral errors, percent nonoral errors, perceived velopharyngeal function (PVPF), and intelligibility were compared with results based on reassessments by 3 independent raters from audio recordings. Agreement was calculated by the intraclass correlation coefficient (ICC), quadratic weighted kappa, and percentage agreement. RESULTS: Absolute agreement calculated by average measures ICC for percent oral consonants correct, percent oral errors, and percent nonoral errors was above >0.90. Single measures ICC for percent oral consonants correct was 0.82, for percent oral errors 0.69, and for percent nonoral errors 0.83. The kappa coefficient for PVPF was 0.5 to 0.59 and for intelligibility 0.65 to 0.77. Exact percentage agreement for PVPF was 33% and for intelligibility 47.8%. CONCLUSIONS: The data on oral consonants correct and nonoral errors in the quality registry seem to be reliable. The data on oral errors, PVPF, and intelligibility should be interpreted with caution. If differences among treatment centers are detected, one should go back and examine the collected raw data before drawing any definitive conclusions about treatment outcome.

Speech in 5-Year-Olds With Cleft Palate With or Without Cleft Lip Treated With Primary Palatal Surgery With Muscle Reconstruction According to Sommerlad.

OBJECTIVE: To evaluate speech in 5-year-olds with cleft palate with or without cleft lip (CP±L) treated with primary palatal surgery in 1 stage with muscle reconstruction according to Sommerlad at about 12 months of age. DESIGN: Retrospective study. SETTING: Primary care university hospital. PARTICIPANTS: Eight 5-year-olds with cleft soft palate (SP), 22 with cleft soft/hard palate (SHP), 33 with unilateral cleft lip and palate, and 17 with bilateral CLP (BCLP). MAIN OUTCOME MEASURES: Percent oral consonants correct (POCC), percent consonants correct adjusted for age (PCC-A), percent oral errors, percent nonoral errors, and variables related to velopharyngeal function were analyzed from assessments of audio recordings by 3 independent speech-language pathologists. RESULTS: The median POCC was 75.4% (range: 22.7%-98.9%), median PCC-A 96.9% (range: 36.9%-100%), median percent oral errors 3.4% (range: 0%-40.7%), and median percent nonoral errors 0% (range: 0%-20%), with significantly poorer results in children with more extensive clefts. The SP group had significantly less occurrence of audible nasal air leakage than the SHP and the BCLP groups. Before age 5 years, 1.3% of the children underwent fistula surgery and 6.3% secondary speech improving surgery. At age 5 years, 15% of the total group was perceived as having incompetent velopharyngeal function. CONCLUSIONS: Speech was poorer in many children with more extensive clefts. Children with CP±L had poorer speech compared to normative data of peers without CP±L, but the results indicated relatively good speech compared to speech of children with CP±L in previous studies.

Does the recording medium influence phonetic transcription of cleft palate speech?

BACKGROUND: In recent years, analyses of cleft palate speech based on phonetic transcriptions have become common. However, the results vary considerably among different studies. It cannot be excluded that differences in assessment methodology, including the recording medium, influence the results. AIMS: To compare phonetic transcriptions from audio and audio/video recordings of cleft palate speech by means of outcomes of per cent correct consonants (PCC) and differences in consonant transcriptions. METHODS & PROCEDURES: Thirty-two 3-year-olds born with cleft palate were audio and audio/video recorded while performing a single-word test by picture naming. The recordings were transcribed according to the International Phonetic Alphabet. The transcriptions from the audio and the audio/video recordings were analysed with regard to PCC, differences in the use of phonetic consonant symbols and the use of diacritics. OUTCOMES & RESULTS: PCC was higher when the calculation was based on transcriptions from audio recordings than when based on audio/video recordings. No such differences were seen when age-appropriate consonant processes were scored as correct. Consonants were not excluded as often in the transcriptions from audio/video recordings as they were in the transcriptions from audio recordings, and more target consonants were transcribed as dental/alveolar in the transcriptions from audio/video recordings and palatal/velar/uvular in the transcriptions from audio recordings than vice versa. Further, interdental articulation, linguolabial articulation and audible nasal air leakage were more common in the transcriptions from audio/video recordings than in those from audio recordings. All these differences were statistically significant. CONCLUSIONS & IMPLICATIONS: Phonetic transcription is influenced to some extent by visual cues. However, as long as age-appropriate articulatory and phonological simplification processes are scored as correct when evaluating consonant production in the speech of young children born with cleft palate using phonetic transcription, the recording medium does not seem to affect the results.

Verbal competence in narrative retelling in 5-year-olds with unilateral cleft lip and palate.

BACKGROUND: Research regarding expressive language performance in children born with cleft palate is sparse. The relationship between articulation/phonology and expressive language skills also needs to be further explored. AIMS: To investigate verbal competence in narrative retelling in 5-year-old children born with unilateral cleft lip and palate (UCLP) and its possible relationship with articulation/phonology at 3 and 5 years of age. METHODS & PROCEDURES: A total of 49 children, 29 with UCLP treated according to three different procedures for primary palatal surgery and a comparison group of 20 children (COMP), were included. Longitudinally recorded audio files were used for analysis. At ages 3 and 5, the children were presented with a single-word test of word naming and at age 5 also the Bus Story Test (BST). The BST was assessed according to a test manual. The single-word test was phonetically transcribed and the percentage of consonants correct adjusted for age (PCC-A) was calculated. Differences regarding the BST results within the UCLP group were analysed. The results were compared with the results of the COMP group, and also with norm values. In addition, the relationship between the results of the BST and the PCC-A scores at ages 3 and 5 years was analysed. OUTCOMES & RESULTS: No significant group differences or correlations were found. However, 65.5% of the children in the UCLP group had an information score below 1 standard deviation from the norm value compared with 30% in the COMP group. CONCLUSIONS: A larger proportion of children in the UCLP group than in the COMP group displayed problems with retelling but the differences between the two groups were not significant. There was no association between the BST results in the children with UCLP and previous or present articulatory/phonological competence. Since group size was small in both groups, the findings need to be verified in a larger study.

Phonology in Swedish-speaking 3-year-olds born with cleft lip and palate and the relationship with consonant production at 18 months.

BACKGROUND: Approximately 50% of children born with cleft palate present speech difficulties around 3 years of age, and several studies report on persisting phonological problems after palatal closure. However, studies on early phonology related to cleft palate are few and have so far mainly been carried out on English-speaking children. Studies on phonology related to cleft palate in languages other than English are also warranted. AIMS: To assess phonology in Swedish-speaking children born with and without unilateral cleft lip and palate (UCLP) at 3 years of age, and to identify variables at 18 months that are associated with restricted phonology at age 3 years. METHODS & PROCEDURES: Eighteen consecutive children born with UCLP and 20 children without cleft lip and palate were included. Transcriptions of audio recordings at 18 months and 3 years were used. Per cent correct consonants adjusted for age (PCC-A), the number of established phonemes, and phonological simplification processes at 3 years were assessed and compared with different aspects of consonant inventory at 18 months. OUTCOMES & RESULTS: PCC-A, the number of established phonemes, and the total number of phonological processes differed significantly at 3 years between the two groups. Total number of oral consonants, oral stops, dental/alveolar oral stops and number of different oral stops at 18 months correlated significantly with PCC-A at 3 years in the UCLP group. CONCLUSIONS & IMPLICATIONS: As a group, children born with UCLP displayed deviant phonology at 3 years compared with peers without cleft lip and palate. Measures of oral consonant and stop production at 18 months might be possible predictors for phonology at 3 years in children born with cleft palate.

Speech and phonology in Swedish-speaking 3-year-olds with unilateral complete cleft lip and palate following different methods for primary palatal surgery.

Objective : To describe and compare speech and phonology at age 3 years in children born with unilateral complete cleft lip and palate treated with three different methods for primary palatal surgery. Design : Prospective study. Setting : Primary care university hospitals. Participants : Twenty-eight Swedish-speaking children born with nonsyndromic unilateral complete cleft lip and palate. Interventions : Three methods for primary palatal surgery: two-stage closure with soft palate closure between 3.4 and 6.4 months and hard palate closure at mean age 12.3 months (n = 9) or 36.2 months (n = 9) or one-stage closure at mean age 13.6 months (n = 10). Main Outcome Measures : Based on independent judgments performed by two speech-language pathologists from standardized video recordings: percent correct consonants adjusted for age, percent active cleft speech characteristics, total number of phonological processes, number of different phonological processes, hypernasality, and audible nasal air leakage. The hard palate was unrepaired in nine of the children treated with two-stage closure. Results : The group treated with one-stage closure showed significantly better results than the group with an unoperated hard palate regarding percent active cleft speech characteristics and total number of phonological processes. Conclusions : Early primary palatal surgery in one or two stages did not result in any significant differences in speech production at age 3 years. However, children with an unoperated hard palate had significantly poorer speech and phonology than peers who had been treated with one-stage palatal closure at about 13 months of age.

Comment on Ombashi, van der Goes, Versnel, Khonsari, van der Molen: guidance to develop a multidisciplinary, international, pediatric registry: a systematic review, Orphanet Journal of Rare diseases, 2023.

Recently, Ombashi et al. published a systematic review aiming to identify the pitfalls in the development and implementation as well as factors influencing long-term success of a multidisciplinary, international registry for cleft care on a global scale. The purpose of this letter to the editor is to highlight that the review failed to include the Swedish quality registry for patients born with cleft lip and palate, which fulfils the inclusion criteria. The Swedish cleft lip and palate registry is multidisciplinary, has a high coverage and reporting degree, and most outcome measures have been checked for reliability and validity. It is regularly used for open comparisons between treatment centers. Several research studies have been published based on the Swedish cleft lip and palate registry, and more are ongoing. The information we provide about the Swedish cleft lip and palate registry complements and expands the information of the results reported by Ombashi et al. in their research.

Surgical treatment of velopharyngeal dysfunction: Incidence and associated factors in the Swedish cleft palate population.

INTRODUCTION: Speech in children with cleft palate can be affected by velopharyngeal dysfunction, which persists after primary palate repair. The incidence of surgery to correct velopharyngeal dysfunction in this patient group has previously been reported as 2.6-37%. We aimed to investigate the incidence of velopharyngeal dysfunction surgery in Swedish children with cleft palate and to examine potential associations of independent variables with this incidence. METHODS: In this cohort study, we analysed data from the Swedish cleft lip and palate quality registry for 1093 children with cleft palate with or without cleft lip. Kaplan-Meier analysis was used to estimate the risk of having velopharyngeal dysfunction surgery. Multivariable Cox proportional hazards models were used to estimate the associated effect of cleft subtype, additional diagnoses, gender, and age at and number of stages for primary palate repair on the primary outcome. RESULTS: The risk of having velopharyngeal dysfunction surgery was 25.6%. Complete primary palate repair after the age of 18 months or in more than one stage was associated with a higher risk, but it could not be determined which of these was the more significant factor. Cleft soft palate was associated with a significantly lower risk than other cleft subtypes. CONCLUSIONS: Primary palate repair at a higher age or in more than one stage may increase the risk of having velopharyngeal dysfunction surgery. Further analysis of potential unknown confounding factors and the association between the incidence of velopharyngeal dysfunction and surgery to correct this condition is needed.

Additional diagnoses in children with cleft lip and palate up to five years of age.

Cleft lip and palate (CL/P) is the most common congenital craniofacial malformation and is often associated with additional diagnoses. The purpose of this study was to explore the cumulative five-year incidence of additional diagnoses for patients with cleft lip and palate. Further aims were, type of cleft and type of additional diagnose and to validate CLP registry data on additional diagnoses. Data from the CLP registry regarding children with CL/P in the Southern Health Care Region were retrieved and based on the registry, participants were selected. A review of medical records of participants born 2006-2016 was performed and data regarding participant characteristics and additional diagnoses were collected. Of the 250 participants included in the review of medical records, 90 participants (36%) had an additional diagnosis. Of the total number of identified additional diagnoses (n = 137), cardiovascular system (20.4%) and extremities and skeletal system (17.5%) were the most prevalent categories. The comparison between medical records and the CLP registry of all children showed a 14.4 percentage points higher incidence of additional diagnoses in the medical records. Roughly every third child received an additional diagnosis and diagnoses related to the cardiovascular system were the most frequent. This study also shows that additional diagnoses were under-reported in the CLP registry. Future research is necessary to strengthen associations of additional diagnoses to CL/P.

Long-term speech outcome after anterior distraction osteogenesis of the maxilla in patients with cleft lip and palate.

Advancement of the maxilla may increase the distance between the soft palate and the posterior pharyngeal wall in patients with cleft lip and palate, implying a risk of velopharyngeal dysfunction. The aim was to evaluate long-term speech outcome in a consecutive series of patients treated with distraction osteogenesis (DO). Fourteen out of the 16 patients agreed to participate. A long-term speech follow-up was performed 1.5 to 13.5 years after DO. For two participants, audio recordings before DO were missing, and for another one, it was incomplete. The percentage of consonants correct (PCC) based on phonetic transcription and perceived velopharyngeal competence rated on a three-point scale were assessed before and after DO by three independent judges, based on audio recordings of reading of standardised sentences. Also, the participants were asked how they perceived their speech after DO. Changes in PCC were insignificant. Four participants perceived deteriorated speech related to DO. In two cases, the subjective deterioration did not correlate to results from perceptual assessment. In two others, the subjective deterioration correlated with the perceptual assessment, and the velopharyngeal function was judged as being incompetent after DO. After secondary velopharyngeal surgery, velopharyngeal function improved to competent in one case and marginally incompetent in the other. The results need to be interpreted with caution due to methodological limitations but indicate that some patients develop deteriorated velopharyngeal function after DO. The impact on articulation needs to be further explored. It is important that patients are informed before treatment of the risk of velopharyngeal dysfunction after DO.

Speech in 7- and 10-year-olds born with a unilateral cleft lip and palate: a continued prospective Swedish intercentre study.

The aim of this study was to report longitudinal speech results in consecutively selected children from each of the six cleft centres in Sweden and to compare the results between centres. The children were born with a non-syndromic unilateral cleft lip and palate, and results from the same cohort at 5 years of age have previously been reported. Background data on medical care in terms of surgery, speech therapy, and hearing between 5 and 10 years of age were collected. Speech recordings of 56 children at 7 years and 54 at 10 years of age were blindly and independently assessed by four speech-language pathologists experienced in cleft palate speech. This resulted in measures of percent consonant correct (PCC) and perceived velopharyngeal competence rated on a three-tier scale. No statistically significant differences were found between centres. PCC scores at 7 years of age ranged from 44-100% (median 97.5) and at 10 years of age from 86-100% (median 100). Competent or marginally incompetent velopharyngeal function was found in 95% of the 7-year-olds and 98% of the 10-year-olds. Speech results were slightly better than previous reports of speech in children born with a unilateral cleft lip and palate.