Foreign-born 5-year-old children with cleft palate had poorer speech outcomes than their native-born peers.

AIM: Speech difficulties are common in children with cleft palate, but research on foreign-born children is limited. This study aimed to compare speech outcomes, surgery and speech intervention in 5-year-old foreign-born and Swedish-born children with cleft palate with or without cleft lip. METHODS: This retrospective study analysed data from the Swedish cleft lip and palate registry for children born between 2009 and 2016 using Pearson's Chi-squared test and binary logistic regression. RESULTS: Among 160 foreign-born (106 boys, 54 girls) and 847 Swedish-born (479 boys, 368 girls) 5-year-olds, foreign-born children had significantly lower rates of sufficient velopharyngeal competence (77% vs. 86%), age-appropriate consonant production (28% vs. 60%), and speech without non-oral speech errors (70% vs. 86%). Differences remained after adjustment for cleft type, gender and additional diagnosed conditions. After further adjustments for age at completed primary palatal surgery, differences in age-appropriate consonant production and speech without non-oral speech errors remained significant. Foreign-born children underwent completed primary palatal surgery at older ages and received more secondary palatal surgery and speech intervention than Swedish-born peers. CONCLUSION: Foreign-born children showed poorer speech outcomes than Swedish-born peers, despite more secondary palatal surgery and speech intervention. Age at completed primary palatal surgery could partly explain these differences.

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.

A systematic review of differences in outcome between one and two stage palate repair in cleft lip and palate.

The aim of this systematic review was to determine whether one-stage palatoplasty for children born with cleft lip and palate shows overall advantages in outcome compared with two-stage palatoplasty. The included studies were controlled studies of syndromic and non-syndromic children born with unilateral cleft lip and palate, bilateral cleft lip and palate, or isolated cleft palate. The interventions studied were one-stage palatoplasty and two-stage palatoplasty starting with the soft palate. The outcomes were facial growth, speech, hearing, presence of fistulae, other complications related to surgery, health-related quality of life, and health economics. In total, 14 original studies were included. Results were dichotomized into showing advantage for one- or two-stage palatoplasty for the respective outcome and compared with the results from six included systematic reviews. No overall advantage for either surgical strategy was found for any of the outcome measures. The certainty of evidence was highest for the presence of fistulae, followed by facial growth and speech. For several outcomes, the quality of the existing evidence was too low to allow for any conclusions to be drawn. Neither one- nor two-stage palatoplasty showed significant advantages in clinical outcomes compared with the other. Other aspects such as ethics, economics, or surgeon's preference might hence be of more importance. Homogenous choices of outcome measures and defined minimal clinically important differences would facilitate further research.

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.

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.

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.).

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.

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.

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.

Speech in 5-year-olds born with unilateral cleft lip and palate: a Prospective Swedish Intercenter Study.

Studies on the impact of cleft palate surgery on speech with stringent methodology are called for, since we still do not know the best timing or the best method for surgery. The purpose was to report on speech outcome for all Swedish-speaking 5-year-olds born with a non-syndromic unilateral cleft lip and palate (UCLP), in 2008-2010, treated at Sweden's six cleft palate centres, and to compare speech outcomes between centres. Speech was assessed in 57 children with percent consonants correct adjusted for age (PCC-A), based on phonetic transcriptions from audio recordings by five independent judges. Also, hypernasality and perceived velopharyngeal function were assessed. The median PCC-A for all children was 93.9, and medians in the different groups varied from 89.9 to 96.8. In the total group, 9 children (16%) had more than mild hypernasality. Twenty-two children (38.5%) were perceived as having competent/sufficient velopharyngeal function, 25 (44%) as having marginally incompetent/insufficient velopharyngeal function, and 10 children (17.5%) as having incompetent/insufficient velopharyngeal function. Ten children were treated with secondary speech improving surgery and/or fistula surgery. No significant differences among the six groups, with eight to ten children in each group, were found. The results were similar to those in other studies on speech of children with UCLP, but poorer than results in normative data of Swedish-speaking 5-year-olds without UCLP. Indications of differences in frequency of surgical treatment and speech treatment between centres were observed.

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.

Phonology in Swedish-speaking 3-year-olds born with unilateral cleft lip and palate treated with palatal closure in one or two stages.

BACKGROUND: Phonological disorders are common in 3-year-olds born with cleft palate compared to non-cleft peers. However, published results have been based on small samples. The purpose was to expand the knowledge on phonology of Swedish-speaking 3-year-olds with unilateral cleft lip and palate (UCLP), treated with primary palatal closure in one or two stages. METHODS: The phonology of 26 children with UCLP was assessed with percentage consonants correct adjusted for age (PCC-A) and number of consistent phonological simplification processes (NCP) at age 3. Fifteen of the children were treated with minimal incision technique in one stage (OS) at 13 months and 11 with a two-stage closure (TS) with soft palate repair at 4 months and hard palate repair at 12 months. Their results were compared and then merged with previously obtained data from 10 children treated with OS and nine children treated with TS. Finally, the merged results were compared with those of 20 peers without UCLP. RESULTS: No significant differences between the first two groups were found. In the merged results, NCP in the OS group was significantly lower than in the TS group. The UCLP group displayed significantly poorer results on PCC-A and NCP than peers without UCLP. CONCLUSION: Surgical procedure did not have a clear impact on phonology at age 3 years. Since children with UCLP are at risk of having impaired phonology at age 3, the results confirm the necessity of having both a phonological and articulatory approach when assessing and treating children born with cleft palate.

Subjective outcomes after treatment for velopharyngeal dysfunction.

BACKGROUND: Velopharyngeal dysfunction (VPD) can have various causes and may be a significant disability for the affected patient. Treatment options include surgery and speech therapy, but the success rates are often inconsistent. METHODS: In this study, self-assessment questionnaires were sent out to 222 Swedish patients with VPD. The questionnaire included questions about satisfaction with speech, perceived speech quality, perceived improvement from VPD-surgery, and/or speech therapy. Out of 117 (52.7%) respondents, 114 (51.4%) patients were included in the study. The participants were 7-71 years of age (median = 14 years), diagnosed with cleft palate, neurological/developmental delay, congenital hypernasality, or acquired VPD. All patients had previously undergone videofluoroscopy, and 61.4% had undergone VPD-surgery. RESULTS: Seventy-one per cent of the patients perceived their speech to be normal or slightly deviant, but only 55% were satisfied with their speech. Sixty per cent of the operated on patients felt that the treatment had improved their speech much or very much, 10% thought that they had moderate improvement, and 30% stated that they had no or little improvement. Out of the patients that had received speech therapy, 41% felt that the treatment had improved their speech much or very much, 21% thought that they had moderate improvement, and 33% stated that they had no or little improvement. CONCLUSION: In conclusion, most patients with VPD in this study who underwent evaluation and treatment felt that surgery and speech therapy had improved their speech, but only about half of them were in the end satisfied with the quality of their speech.

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.

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.