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.).

Clinical application of usage-based phonology: Treatment of cleft palate speech using usage-based electropalotography.

PURPOSE: To investigate whether a novel electropalatography (EPG) therapy, underpinned by usage-based phonology theory, can improve the accuracy of target speech sounds for school-aged children and adults with persistent speech sound disorder (SSD) secondary to cleft palate +/- lip. METHOD: Six consecutively treated participants (7-27 years) with long-standing speech disorders associated with cleft palate enrolled in a multiple baseline (ABA) within-participant case series. The usage-based EPG therapy technique involved high-volume production of words. Speech was assessed on three baselines prior to therapy, during weekly therapy, at completion of therapy, and 3 months post-therapy. Percent correct of target phonemes in untreated words and continuously connected speech were assessed through acoustic phonetic transcription. Intra- and inter-transcriber agreement was determined. RESULT: Large to medium treatment effect sizes were shown for all participants following therapy (15-33 sessions). Percentage of targets correct for untreated words improved from near 0% pre-therapy, to near 100% for most target sounds post-therapy. Generalisation of target sounds to spontaneous connected speech occurred for all participants and ranged from 78.95-100% (M = 90.66; SD = 10.14) 3 months post-therapy. CONCLUSION: Clinically significant speech change occurred for all participants following therapy. Response to the novel therapeutic technique is encouraging and further research is indicated.

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.

Improved management of placenta accreta spectrum disorders: experience from a single institution.

OBJECTIVES: To assess the applicability of a standardized multidisciplinary protocol for managing placenta accreta spectrum (PAS) disorders and its impact on the outcomes. METHODS: We compared patients with PAS manage by a standardized multidisciplinary protocol (T2) to historic controls managed on a case-by-case basis by individual physicians between (T1). The primary outcome is composite maternal morbidity. Secondary outcomes were the rates of surgical complications, estimated blood loss, number of blood products transfused, intensive care unit admissions, ventilator use, and birth weight. Multivariate logistic analysis was used to identify independent predictors of composite maternal morbidity. RESULTS: During T1 and T2, we managed 39 and 36 patients with confirmed PAS, respectively. During T2, the protocol could be implemented in 21 cases (58%). Compared to T1, patients managed during T2 had 70% less composite maternal morbidity (95% CI: 0.11-0.82) and lower blood loss (median, 2,000 vs. 1,100 mL, p=0.008). Also, they were 68% less likely to require transfusion of blood products (95% CI: 0.12-0.81; p=0.01), including fewer units of packed red blood cells (median, 2 vs. 0, p=0.02). Management following the protocol was the only independent factor associated with lower composite maternal morbidity (OR: 0.22; 95% CI: 0.05-0.95; p=0.04). Selected maternal and neonatal outcomes were not different among 12 and 15 patients with suspected but unconfirmed PAS disorders managed during T1 and T2, respectively. CONCLUSIONS: Most patients can be managed under a standardized multidisciplinary protocol for PAS disorders, leading to improved outcomes.

Assessment of prelinguistic vocalizations in real time: a comparison with phonetic transcription and assessment of inter-coder-reliability.

This study investigated reliability of naturalistic listening in real time (NLRT) compared to phonetic transcription. Speech pathology students with brief training in NLRT assessed prelinguistic syllable inventory size and specific syllable types in typically developing infants. A second study also examined inter-coder reliability for canonical babbling, canonical babbling ratio and presence of oral stops in syllable inventory of infants with cleft palate, by means of NLRT. In study 1, ten students independently assessed prelinguistic samples of five 12-month-old typically developing infants using NLRT and phonetic transcription. Coders assessed syllable inventory size as more than twice as large using phonetic transcription as NLRT. Results showed a strong correlation between NLRT and phonetic transcription (syllables with more than five occurrences) for syllable inventory size (r = .60; p < .001). The methods showed similar results for inter-coder reliability of specific syllable types. In study 2, three other students assessed prelinguistic samples of twenty-eight 12-month-old infants with cleft palate by means of NLRT. Results revealed perfect inter-coder agreement for presence/absence of canonical babbling, strong correlations between the three coders' assessment of syllable inventory size (average r = .83; p < .001), but more inter-coder variability for agreement of specific syllable types. In conclusion, NLRT is a reliable method for assessing prelinguistic measures in infants with and without cleft palate with inter-coder agreement levels comparable to phonetic transcription for specific syllable types.

Velopharyngeal insufficiency: high detection rate of genetic abnormalities if associated with additional features.

OBJECTIVE: To review the clinical and molecular-genetic characteristics of 34 children who were referred to the clinical genetics department with a presenting diagnosis of definite or suspected velopharyngeal insufficiency (VPI, defined as the inability to close off the nasal from the oral cavity during speech) or hyponasal/hypernasal speech. All the patients referred also had additional anomalies and did not therefore comprise the whole VPI population. METHODS: Patients were clinically investigated by a clinical geneticist. Fluorescent in situ hybridisation for chromosome 22q11 deletion and/or array comparative genomic hybridisation (array CGH) analysis was performed in all cases. A literature review was performed using the Pubmed online database. RESULTS: Microdeletions or microduplications were identified in half of the patients. Six patients (∼18% of total) carried a chromosome 22q11 microdeletion, one patient had a chromosome 22q11 microduplication, and four patients had microdeletions in other chromosomes that were considered likely to be associated with the phenotype. One patient had KBG syndrome. Thus, an underlying genetic abnormality was found in approximately one-third (35%) of our patients. An additional seven patients harboured copy number variations that were considered benign or of unknown significance. CONCLUSIONS: We present an overview of patients with VPI or hyponasal/hypernasal speech with additional anomalies and their clinical and genetic findings. In one-third of these patients, an underlying genetic abnormality was identified. This has important implications for family counselling and medical follow-up. Furthermore, we recommend array CGH testing in all patients with VPI and associated anomalies because of the high percentage of copy number variants identified in these patients.