Analysis and Reporting of Randomized Trials in Cleft Palate Surgery: Learning from the Timing of Primary Surgery (TOPS) Trial.

The Timing of Primary Surgery (TOPS) trial was published August 2023 in the New England Journal of Medicine and is a milestone achievement for a study focused on cleft palate. Due to the complexity of outcome reporting in cleft and the rarity of such comparative trials, TOPS presents a useful opportunity to critically review the design, analysis and reporting strategies utilised. This perspective article focused on the inclusion of participants, the choice of the primary outcome measure and the analysis of ordinal data within the trial. Considerations for future comparative studies in cleft care are discussed.

The relationship between auditory behaviours and speech and language development in children with cleft lip and palate.

PURPOSE OF REVIEW: Children born with a cleft lip and palate (CLP) are at risk of speech problems. There is a high prevalence of conductive hearing loss due to otitis media with effusion and recent research has highlighted broader difficulties with language and educational attainment. There has been increased interest in the impact of auditory processing on a child's development, but little study into links between auditory skills and speech and language development. RECENT FINDINGS: Children with CLP consistently struggle with auditory tasks. Difficulties processing words in a noisy background, auditory attention and temporal processing are frequently reported. A recent study in the United Kingdom investigated the auditory behaviours of 95 children aged 5-8 years with nonsyndromic CLP and compared these with speech and language profiles. Auditory difficulties correlated with speech development and most auditory behaviours correlated with language outcomes. There was also a correlation of language with speech outcomes, suggesting a more complex and interdependent relationship. SUMMARY: Children with CLP present with high levels of difficulties with auditory skills. The potential impact of this on broader development needs further research, but clinicians should ensure that auditory skills beyond the hearing test are monitored, and advice given to parents to minimize the impact.

Inter-rater reliability in classification of canonical babbling status based on canonical babbling ratio in infants with isolated cleft palate randomised to Timing of Primary Surgery for Cleft Palate (TOPS).

Canonical babbling (CB) is commonly defined as present when at least 15% of all syllables produced are canonical, in other words a canonical babbling ratio (CBR) ≥0.15. However, there is limited knowledge about inter-rater reliability in classification of CB status based on CBR and inter-rater differences in assessment of CBR. We investigated inter-rater reliability of experienced Speech Language Therapists (SLTs) on: classification of CB status based on CBR ≥ 0.15, CBRs and the total number of syllables per infant used to calculate CBR.Each infant (n = 484) was video-recorded at a clinical site in play interaction with their parent as part of the randomised controlled trial Timing of Primary Surgery for Cleft Palate. Each recording was subsequently assessed by three independent SLTs, from a pool of 29 SLTs. They assessed the recordings in real time.The three assessing SLTs agreed in classification of CB status in 423 (87.4%) infants, with higher complete agreement for canonical (91%; 326/358) than non-canonical (77%; 97/126). The average difference in CBR and total number of syllables identified between the SLT assessments of each infant was 0.12 and 95, respectively.This study provided new evidence that one trained SLT can reliably classify CB status (CBR ≥ 0.15) in real time when there is clear distinction between the observed CBR and the boundary (0.15); however, when the observed CBR approaches the boundary multiple SLT assessments are beneficial. Thus, we recommend to include assessment of inter-rater reliability, if the purpose is to compare CBR and total syllable count across infants or studies.Trial registration number here: www.clinicaltrials.gov, identifier NCT00993551.

Investigating the Impact of Patient-Related Factors on Speech Outcomes at 5 Years of Age in Children With a Cleft Palate.

To investigate the relationship between patient-related factors (sex, cleft type, cleft extent, and Robin Sequence [RS]) and speech outcome at 5 years of age for children born with a cleft palate ± lip (CP ± L).3157 Children (1426 female:1731 male) with a nonsyndromic CP ± L, born between 2006 and 2014 in England, Wales, and Northern Ireland.Perceptual speech analysis utilized the Cleft Audit Protocol for Speech-Augmented (CAPS-A) rating and UK National Speech Outcome Standards: Speech Standard 1 (SS1)-speech within the normal range, SS2a-no structurally related speech difficulties or history of speech surgery, and SS3-speech without significant cleft-related articulation difficulties.Odds of achieving SS1 were lower among boys (aOR 0.771 [CI 0.660-0.901]), those with clefts involving the lip and palate (vs palate only) (UCLP-aOR 0.719 [CI 0.591-0.875]; BCLP-aOR 0.360 [CI 0.279-0.463]), and clefts involving the hard palate (incomplete-aOR 0.701 [CI 0.540-0.909]; complete-aOR 0.393 [CI 0.308-0.501]). Similar relationships with these patient factors were observed for SS3. SS2 was affected by the extent of hard palate involvement (complete; aOR 0.449 [CI 0.348-0.580]). Although those with CP and RS were less likely to meet all 3 standards than those without RS, odds ratios were not significant when adjusting for sex and cleft extent.Sex, cleft type, and extent of hard palate involvement have a significant impact on speech outcome at 5 years of age. Incorporating these factors into risk-adjustment models for service-level outcome reporting is recommended.

A Closer Look at Delayed Primary Cleft Surgery and Unrepaired Cleft Lip and/or Palate in 5 UK Cleft Centers.

BACKGROUND: There may be many reasons for delays to primary cleft surgery. Our aim was to investigate the age of children undergoing primary cleft lip or primary cleft palate repair in 5 cleft centers within the United Kingdom. Identify the reasons for delayed primary cleft lip repair (beyond 6 months) and delayed primary palate repair (beyond 13 months). Identify children who had a cleft lip and/or palate (CL±P) that was intentionally unrepaired and the reasons for this. METHODS: A retrospective, multicenter review of patients born with a CL±P between December 1, 2012, and December 31, 2016. Three regional cleft centers, comprising of 5 cleft administrative units in the United Kingdom participated. RESULTS: In all, 1826 patients with CL±P were identified. Of them, 120 patients had delayed lip repair, outside the expected standard of 183 days. And, 178 patients in total had delayed palate repair, outside the expected standard of 396 days. Twenty (1%) patients had an unrepaired cleft palate. CONCLUSIONS: This large retrospective review highlights variations between centers regarding the timing of lip and palate surgery and details the reasons stated for delayed primary surgery. A small number of patients with an unrepaired cleft palate were identified. All had complex medical problems or comorbidities listed as a reason for the decision not to operate and 50% had a syndromic diagnosis. The number of patients receiving delayed surgery due to comorbidities, being underweight or prematurity, highlights the importance of the cleft specialist nurse and pediatrician within the cleft multidisciplinary team.

Early Communication Behaviors in Infants With Cleft Palate With and Without Robin Sequence: A Preliminary Study.

OBJECTIVE: To investigate the early communication behaviors in infants with nonsyndromic isolated cleft palate (iCP) and Robin sequence (RS). DESIGN: Group comparison using parent report. PARTICIPANTS: There were 106 participants included in this study. Two groups were selected from the UK Cleft Collective resource. Parents had completed the Language ENvironment Analysis Developmental Snapshot questionnaire when their child turned 13 months. There were 78 participants in the iCP group and 28 in the RS group. MAIN OUTCOME MEASURE(S): Total number of communication behaviors reported on the questionnaire. Subdomains for expressive and receptive language and social communication behaviors were also analyzed. RESULTS: There were no statistically significant group differences. Parents of infants with RS reported fewer later communication behaviors compared to the iCP group. Infants in both groups had fewer communication behaviors compared to the normative sample. Across the whole sample, post hoc analysis revealed a significant correlation between severity of the cleft and social communication behaviors and expressive but not receptive language. Infants with a cleft of the hard and soft palate were more likely to be in the RS group (odds ratio: 7.04 [95% CI: 1.55-32.04]; P = .01). CONCLUSIONS: Both groups reported similar levels of early communication. Some divergence of more complex language skills was seen, although there were no significant group differences. A relationship with the diagnosis of a cleft of the hard or soft palate with expressive language behaviors was found. Further study into the impact of cleft severity on early speech development and the relationship with later language skills is needed along with longitudinal follow-up of this population.

Linguistic and auditory processing skills in non-syndromic children with cleft palate: A scoping review.

BACKGROUND: Cleft lip and/or palate (CL/P) is the most common congenital craniofacial condition. Children born with CL/P are at increased risk of persistent speech difficulties related to velopharyngeal incompetence (VPI) and compensatory articulation problems. It has also been reported that they achieve poorer results academically than their peers. There is a further body of evidence to suggest delayed language skills. These potentially related outcomes are often reported separately. AIM: To review published and unpublished research into the nature of difficulties related to spoken and written language across all non-syndromic cleft diagnoses. To review any evidence of associations between comorbidities. METHOD: A scoping review was carried out in October 2016 and updated in June 2019 following published methodology (Arksey & O'Malley, 2005; Levac et al. 2010). RESULTS: A search of the literature over the two time points found 38 papers in total. Three main themes were found: oral language skills, reading and auditory processing difficulties. CONCLUSIONS: There is evidence of early language delay in children born with CL/P. Evidence of persistent oral language problems is less conclusive. Many studies have reported scores within the average range for language, auditory processing and reading but poorer outcomes when compared to non-cleft control groups. However, studies have used a range of outcome measures, making comparisons difficult. Moreover there is no clear evidence how these difficulties might relate to speech outcomes or educational achievement and no comparison to other populations with speech, language and communication needs (SLCN).