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.).

Scandcleft Project Trial 3: Comparison of Speech Outcomes in Relation to Sequence in 2-Stage Palatal Repair Procedures in 5-Year-Olds With Unilateral Cleft Lip and Palate.

OBJECTIVE: To compare speech outcome following different sequencing of hard and soft palate closure between arms and centers within trial 3 and compare results to peers without cleft palate. DESIGN: A prospective randomized clinical trial. SETTING: Two Norwegian and 2 British centers. PARTICIPANTS: One hundred thirty-six 5-year-olds with unilateral cleft lip and palate were randomized to either lip and soft palate closure at 3 to 4 months and hard palate closure at 12 months (arm A) or lip and hard palate closure at 3 to 4 months and soft palate closure at 12 months (arm D). MAIN OUTCOME MEASURES: A composite measure of velopharyngeal competence (VPC), overall assessment of VPC from connected speech (VPC-Rate). Percentage of consonants correct (PCC), active cleft speech characteristics (CSCs), subdivided by oral retracted and nonoral errors, and developmental speech characteristics (DSCs). RESULTS: Across the trial, 47% had VPC, with no statistically significant difference between arms within or across centers. Thirty-eight percent achieved a PCC score of >90%, with no difference between arms or centers. In one center, significantly more children in arm A produced ≥3 active CSCs (P < .05). Across centers, there was a statistically significant difference in active CSCs (arm D), oral retracted CSCs (arm D), and DSCs (arms A and D). CONCLUSIONS: Less than half of the 5-year-olds achieved VPC and around one-third achieved age-appropriate PCC scores. Cleft speech characteristics were more common in arm A, but outcomes varied within and across centers. Thus, outcome of the same surgical method can vary substantially across centers.

Associations Between Hypernasality, Intelligibility, and Language and Reading Skills in 10-Year-Old Children With a Palatal Cleft.

BACKGROUND: This study investigated the associations between hypernasality and intelligibility, and language and reading skills in 10-year old children with a cleft palate ± lip. DESIGN: Cross-sectional data collected during routine assessments of speech and language in a centralized treatment setting. PARTICIPANTS: Children aged 10, born with cleft palate ± lip from 4 birth cohorts (N = 123). OUTCOME MEASURES: Hypernasality and intelligibility: Swedish Articulation and Nasality Test-N; language: Language 6-16 (Sentence recall, Serial recall, Vocabulary); reading: word chain test and reading comprehension test. RESULTS: A total of 71.3% of the children had no occurrence of hypernasality and 82.8% had intelligibility scores within the normal range. For all children with hypernasality and intelligibility within the normal range, reading and language scores were also within normal ranges. Children with presence of hypernasality had significantly lower language skills, with mean scores within the lower normal range. Children with reduced intelligibility had lower scores on reading comprehension. CONCLUSIONS: The findings highlight a possible association between hypernasality and language skills, and intelligibility and reading skills. Cleft teams should consider routine assessments of language and reading skills in children with speech impairment, in order to identify potential needs for intervention as early as possible.

Adults with 22q11.2 deletion syndrome have a different velopharyngeal anatomy with predisposition to velopharyngeal insufficiency.

AIM: To find out if subjects with 22q11.2 deletion syndrome (DS) have a different velopharyngeal anatomy which could cause velopharyngeal insufficiency (VPI). METHODS: A prospective study of 16 subjects >16 years of age with 22q11.2 DS, without overt cleft palate and without previous VPI surgery, and 48 healthy controls >18 years of age were included in the study. Speech was recorded and scored blindly by two independent senior speech therapists. All 64 individuals had MRI scans, which were analyzed blindly by a consultant radiologist. RESULTS: Subjects with 22q11.2 DS had a mild degree of weak pressure consonants (mean score); borderline to mild degree of hypernasality and audible nasal emission (mean score). All controls had normal speech. When comparing subjects (22q11.2 DS) to controls, we found the subjects to have the following: A shorter distance between left and right points of origin of the levator veli palatini muscle (LVP) (p < 0.0001); a more obtuse angle of origin of the LVP (bilaterally) (p < 0.009); a thinner LVP bilaterally and in the midline (p < 0.0001); a shorter LVP bilaterally (p < 0.0001); a shorter velum (p = 0.007); a larger osseous pharyngeal depth:velar length ratio (p = 0.01); a more obtuse anterior cranial base angle (nasion to sella to basion) (p < 0.0001) and posterior cranial base angle (sella to basion to foramen magnum) (p < 0.0001); a wider velopharyngeal width (p = 0.002) and a larger pharyngeal airway volume (p = 0.0007). CONCLUSION: Compared with healthy controls, adults with 22q11.2 DS showed a different velopharyngeal anatomy, which will make these individuals more prone to VPI.

Validity of auditory perceptual assessment of velopharyngeal function and dysfunction - the VPC-Sum and the VPC-Rate.

Overall weighted or composite variables for perceptual auditory estimation of velopharyngeal closure or competence have been used in several studies for evaluation of velopharyngeal function during speech. The aim of the present study was to investigate the validity of a composite score (VPC-Sum) and of auditory perceptual ratings of velopharyngeal competence (VPC-Rate). Available VPC-Sum scores and judgments of associated variables (hypernasality, audible nasal air leakage, weak pressure consonants, and non-oral articulation) from 391 5-year olds with repaired cleft palate (the Scandcleft project) were used to investigate content validity, and 339 of these were compared with an overall judgment of velopharyngeal competence (VPC-Rate) on the same patients by the same listeners. Significant positive correlations were found between the VPC-Sum and each of the associated variables (Cronbachs alpha 0.55-0.87, P < 0.001), and a moderately significant positive correlation between VPC-Sum and VPC-Rate (Rho 0.698, P < 0.01). The latter classified cases well when VPC-Sum was dichotomized with 67% predicted velopharyngeal competence and 90% velopharyngeal incompetence. The validity of the VPC-Sum was good and the VPC-Rate a good predictor, suggesting possible use of both measures depending on the objective.

Speech, language, and reading skills in 10-year-old children with palatal clefts: The impact of additional conditions.

BACKGROUND: This study examined speech (hypernasality and intelligibility), language, and reading skills in children with a cleft palate, specifically investigating additional conditions to the cleft, in order to differentiate challenges related to a cleft only, and challenges associated with an additional condition. DESIGN: Cross-sectional data collected during routine assessments of speech and language in a centralised treatment setting. PARTICIPANTS: Children born with cleft with palatal involvement from four birth cohorts (n=184), aged 10. OUTCOME MEASURES: Speech: SVANTE-N; Language: Language 6-16; Reading: Word Chain Test and Reading Comprehension Test. RESULTS: Descriptive analyses revealed that 123 of the children had a cleft only (66.8%), while 61 children (33.2%) had a cleft that was associated with an additional condition (syndrome, developmental difficulty, attentional difficulties). Due to close associations with the outcome variables, children with specific language impairments and dyslexia were excluded from the sample (n=14). In the total cleft sample, 33.1% had mild to severe hypernasality, and 27.9% had mild to severe intelligibility deviances. Most children with intelligibility and hypernasality scores within the normal range had a cleft without any other condition. A high number of children with developmental difficulties (63.2%) or AD/HD (45.5%) had problems with intelligibility. Hypernasality scores were also associated with developmental difficulties (58.8%), whereas most children with AD/HD had normal hypernasality scores (83.3%). As could be expected, results demonstrated that children with a cleft and an additional condition had language and reading scores below average. Children with a cleft only had language and reading scores within the normal range. Among the children with scores below average, 33.3-44.7% had no other conditions explaining difficulties with language and reading. CONCLUSIONS: The findings highlight the need for routine assessments of language and reading skills, in addition to assessments of speech, in children with a cleft, in order to identify potential problems as early as possible. Study designs need to take additional difficulties into account, so that potential problems with language and reading are not ascribed the cleft diagnosis, and can be followed by appropriate treatment and interventions.

Scandcleft randomised trials of primary surgery for unilateral cleft lip and palate: 4. Speech outcomes in 5-year-olds - velopharyngeal competency and hypernasality.

BACKGROUND AND AIM: Adequate velopharyngeal function and speech are main goals in the treatment of cleft palate. The objective was to investigate if there were differences in velopharyngeal competency (VPC) and hypernasality at age 5 years in children with unilateral cleft lip and palate (UCLP) operated on with different surgical methods for primary palatal repair. A secondary aim was to estimate burden of care in terms of received additional secondary surgeries and speech therapy. DESIGN: Three parallel group, randomised clinical trials were undertaken as an international multicentre study by 10 cleft teams in five countries: Denmark, Finland, Sweden, Norway, and the UK. METHODS: Three different surgical protocols for primary palatal repair were tested against a common procedure in the total cohort of 448 children born with a non-syndromic UCLP. Speech audio and video recordings of 391 children (136 girls, 255 boys) were available and perceptually analysed. The main outcome measures were VPC and hypernasality from blinded assessments. RESULTS: There were no statistically significant differences between the prevalences in the arms in any of the trials. VPC: Trial 1, A: 58%, B: 61%; Trial 2, A: 57%, C: 54%; Trial 3, A: 35%, D: 51%. No hypernasality: Trial 1, A: 54%, B: 44%; Trial 2, A: 47%, C: 51%; Trial 3, A: 34%, D: 49%. CONCLUSIONS: No differences were found regarding VPC and hypernasality at age 5 years after different methods for primary palatal repair. The burden of care in terms of secondary pharyngeal surgeries, number of fistulae, and speech therapy visits differed. TRIAL REGISTRATION: ISRCTN29932826.

Scandcleft randomised trials of primary surgery for unilateral cleft lip and palate: 5. Speech outcomes in 5-year-olds - consonant proficiency and errors.

BACKGROUND AND AIM: Normal articulation before school start is a main objective in cleft palate treatment. The aim was to investigate if differences exist in consonant proficiency at age 5 years between children with unilateral cleft lip and palate (UCLP) randomised to different surgical protocols for primary palatal repair. A secondary aim was to estimate burden of care in terms of received additional secondary surgeries and speech therapy. DESIGN: Three parallel group, randomised clinical trials were undertaken as an international multicentre study by 10 cleft teams in five countries: Denmark, Finland, Norway, Sweden, and the UK. METHODS: Three different surgical protocols for primary palatal repair were tested against a common procedure in the total cohort of 448 children born with non-syndromic UCLP. Speech audio- and video-recordings of 391 children (136 girls and 255 boys) were available and transcribed phonetically. The main outcome measure was Percent Consonants Correct (PCC) from blinded assessments. RESULTS: In Trial 1, arm A showed statistically significant higher PCC scores (82%) than arm B (78%) (p = .045). No significant differences were found between prevalences in Trial 2, A: 79%, C: 82%; or Trial 3, A: 80%, D: 85%. Across all trials, girls achieved better PCC scores, excluding s-errors, than boys (91.0% and 87.5%, respectively) (p = .01). CONCLUSIONS: PCC scores were higher in arm A than B in Trial 1, whereas no differences were found between arms in Trials 2 or 3. The burden of care in terms of secondary pharyngeal surgeries, number of fistulae, and speech therapy visits differed. TRIAL REGISTRATION: ISRCTN29932826.

A Scandcleft randomised trials of primary surgery for unilateral cleft lip and palate: 1. Planning and management.

BACKGROUND AND AIMS: Longstanding uncertainty surrounds the selection of surgical protocols for the closure of unilateral cleft lip and palate, and randomised trials have only rarely been performed. This paper is an introduction to three randomised trials of primary surgery for children born with complete unilateral cleft lip and palate (UCLP). It presents the protocol developed for the trials in CONSORT format, and describes the management structure that was developed to achieve the long-term engagement and commitment required to complete the project. METHOD: Ten established national or regional cleft centres participated. Lip and soft palate closure at 3-4 months, and hard palate closure at 12 months served as a common method in each trial. Trial 1 compared this with hard palate closure at 36 months. Trial 2 compared it with lip closure at 3-4 months and hard and soft palate closure at 12 months. Trial 3 compared it with lip and hard palate closure at 3-4 months and soft palate closure at 12 months. The primary outcomes were speech and dentofacial development, with a series of perioperative and longer-term secondary outcomes. RESULTS: Recruitment of 448 infants took place over a 9-year period, with 99.8% subsequent retention at 5 years. CONCLUSION: The series of reports that follow this introductory paper include comparisons at age 5 of surgical outcomes, speech outcomes, measures of dentofacial development and appearance, and parental satisfaction. The outcomes recorded and the numbers analysed for each outcome and time point are described in the series. TRIAL REGISTRATION: ISRCTN29932826.

Speech, Language, and Reading in 10-Year-Olds With Cleft: Associations With Teasing, Satisfaction With Speech, and Psychological Adjustment.

BACKGROUND: Despite the use of multidisciplinary services, little research has addressed issues involved in the care of those with cleft lip and/or palate across disciplines. The aim was to investigate associations between speech, language, reading, and reports of teasing, subjective satisfaction with speech, and psychological adjustment. DESIGN: Cross-sectional data collected during routine, multidisciplinary assessments in a centralized treatment setting, including speech and language therapists and clinical psychologists. PARTICIPANTS: Children with cleft with palatal involvement aged 10 years from three birth cohorts (N = 170) and their parents. OUTCOME MEASURES: Speech: SVANTE-N. Language: Language 6-16 (sentence recall, serial recall, vocabulary, and phonological awareness). Reading: Word Chain Test and Reading Comprehension Test. Psychological measures: Strengths and Difficulties Questionnaire and extracts from the Satisfaction With Appearance Scale and Child Experience Questionnaire. RESULTS: Reading skills were associated with self- and parent-reported psychological adjustment in the child. Subjective satisfaction with speech was associated with psychological adjustment, while not being consistently associated with speech therapists' assessments. Parent-reported teasing was found to be associated with lower levels of reading skills. Having a medical and/or psychological condition in addition to the cleft was found to affect speech, language, and reading significantly. CONCLUSIONS: Cleft teams need to be aware of speech, language, and/or reading problems as potential indicators of psychological risk in children with cleft. This study highlights the importance of multiple reports (self, parent, and specialist) and a multidisciplinary approach to cleft care and research.

Superiorly based pharyngeal flap for treatment of velopharyngeal insufficiency in patients with 22q11.2 deletion syndrome.

BACKGROUND: There are no previous blinded studies for comparison of preoperative versus postoperative perceptual speech assessments when using a pharyngeal flap for treating velopharyngeal insufficiency (VPI) in patients diagnosed with 22q11.2 deletion syndrome. The aim of the study was to evaluate the effect of superiorly based pharyngeal flap surgery on speech in these patients using blinded judgments of experienced speech therapists. METHODS: A retrospective study of 12 consecutive patients who had undergone pharyngeal flap surgery for treatment of VPI between 2002 and 2009 was conducted. Seven girls and 5 boys between 4 and 15 (median, 6) years old at the time of surgery were included in the study. Six patients were born with a submucous cleft palate (including 2 occult), and 1 patient, with an overt cleft palate. The remaining 5 patients had no signs of a palatal pathology. All palatal clefts had been repaired before pharyngeal flap surgery except in 2 patients with occult submucous cleft palate. Preoperative and postoperative audio recordings were blinded for scoring independently by 3 senior speech therapists. RESULTS: There was a significant improvement in hypernasality (P = 0.002), audible nasal emission (P = 0.033), weak pressure consonants (P = 0.008), and speech intelligibility (P = 0.021) after pharyngeal flap surgery. Hyponasality did not develop significantly with surgery. One patient was diagnosed with obstructive sleep apnea. CONCLUSIONS: Superiorly based pharyngeal flap resulted in a significant speech improvement in 12 consecutive patients with 22q11.2 deletion syndrome having VPI.

Autologous fat transplantation to the velopharynx for treating persistent velopharyngeal insufficiency of mild degree secondary to overt or submucous cleft palate.

BACKGROUND: Autologous fat transplantation to the velopharynx has been described in a few smaller studies including heterogeneous groups of patients for the treatment of velopharyngeal insufficiency (VPI). The aim of this study was to evaluate speech and to measure velopharyngeal closure with magnetic resonance imaging (MRI) in patients who underwent autologous fat transplantation for the treatment of persistent VPI of mild degree secondary to overt or submucous cleft palate. METHODS: A prospective study of 16 patients with persistent VPI of mild degree secondary to overt or submucous cleft palate who underwent autologous fat transplantation to the velopharynx. The patients were injected with a median of 5.6 (3.8-7.6) ml autologous fat to the velopharynx. Pre- and 1-year postoperative audio recordings were blinded for scoring independently by three senior speech therapists. Hypernasality, hyponasality, nasal turbulence and audible nasal emission were scored on a five-point scale. Pre- and 1-year postoperative MRIs were obtained during vocal rest and during phonation in 12 patients. Data measured were the velopharyngeal distance in the sagittal plane and the velopharyngeal gap area in the axial plane. RESULTS: Hypernasality improved significantly (p=0.030), but not audible nasal emission (p=0.072) or nasal turbulence (p=0.12). The velopharyngeal distance during phonation decreased significantly (p=0.013), but not the velopharyngeal gap area (p=0.16). There was no significant correlation between speech and MRI results. CONCLUSION: Autologous fat transplantation to the velopharynx improved hypernasality significantly, but not audible nasal emission or nasal turbulence in patients with persistent VPI of mild degree secondary to overt or submucous cleft palate. Given the low number of patients and the lack of a control group, the value of fat transplantation for the treatment of mild VPI is not proven for sure.

Speech and magnetic resonance imaging results following autologous fat transplantation to the velopharynx in patients with velopharyngeal insufficiency.

OBJECTIVE: To measure velopharyngeal closure with magnetic resonance imaging (MRI) and to evaluate speech when treating velopharyngeal insufficiency (VPI) with autologous fat transplantation to the velopharynx. PATIENTS: Nine patients were recruited. Six patients had undergone cleft palate repair and subsequently developed VPI. Three were noncleft patients of which one had developed VPI after nasopharyngeal cancer treatment; another patient had developed VPI after combined adenotonsillectomy, and a third patient had VPI of unknown etiology. MAIN OUTCOME MEASURE: Preoperative and 1-year postoperative MRIs were obtained during vocal rest and during phonation. Data measured were the velopharyngeal distance in the sagittal plane and the velopharyngeal gap area in the axial plane. Preoperative and 1-year postoperative audio recordings were blinded for scoring independently by three senior speech therapists. RESULTS: When comparing preoperative and 1-year postoperative MRI during phonation we found a significant reduction of the median velopharyngeal distance from 4 to 0 mm (p = .011), and a significant reduction of the median velopharyngeal gap area from 42 to 34 mm(2) (p = .038). Nasal turbulence improved significantly (p = .011). Hypernasality/hyponasality and audible nasal emission did not change significantly. CONCLUSIONS: Autologous fat transplantation to the velopharynx resulted in a significant reduction of the velopharyngeal distance and the velopharyngeal gap area during phonation, as measured by MRI. This was in accordance with a significant improvement in nasal turbulence. However, hypernasality and audible nasal emission did not change significantly and could not be correlated to the MRI findings.