Objective
To identify and describe the dynamic features of velopharyngeal dysfunction (VPD) in patients with 22q11.2 deletion syndrome relative to patients with non-syndromic cleft palates.
Study design
Retrospective case-control study.
Setting
Pediatric tertiary care center.
Subjects and methods
A total of 30 children (aged 9–16 years) with VPD were included in this study. Fifteen children with a definitive diagnosis of 22q11.2 deletion syndrome requiring surgical VPD repair were included in the 22q11.2 deletion syndrome group. Fifteen age- and sex-matched children with non-syndromic cleft palate requiring surgical VPD repair were included in the non-syndromic cleft palate group for comparison. Velar displacement, lateral pharyngeal wall displacement, and lateral pharyngeal wall motion pattern data were extracted from preoperative Multiview Videofluoroscopy imaging studies of all children and compared across groups.
Results
Lateral pharyngeal wall displacement was found to be reduced in the 22q11.2 deletion syndrome group (U = 29.50,
p
= .001, r = .63). However, measures of velar displacement were not observed to differ between groups. Similarly, lateral pharyngeal wall motion pattern distributions were not found to differ across these two groups.
Conclusions
Velopharyngeal dysfunction in patients with 22q11.2 deletion syndrome showed differences in dynamic velopharyngeal function when compared to non-syndromic cleft palate patients. The current findings provide initial insights into the unique aspects of velopharyngeal dysfunction for patients with 22q11.2 deletion syndrome. These findings may guide further efforts directed toward understanding the dynamic velopharyngeal characteristics of this population and potentially optimize surgical management and functional outcomes.
Based on these data, children with VPI may be experiencing increased levels of CA. Consequently, the experience of high CA has the potential to affect the communication performance and social functioning of children with VPI.
Background
The aim of this study was to evaluate speech outcomes following surgical intervention for velopharyngeal dysfunction (VPD). Perceptual speech outcome data were subsequently analyzed in conjunction with patient factors such as congenital abnormalities, presence of cleft lip and/or palate, and age of repair. We hope to aid in the eventual creation of treatment algorithms for VPD, allowing practitioners to tailor surgical technique selection to patient factors.
Methods
A retrospective analysis was performed for all patients who underwent surgical correction of VPD at London Health Sciences Centre between the years 2005 and 2018. Two hundred and two consecutive VPD patients (median age 10.6 years) were followed for an average of 20.2 months after having undergone a superiorly based pharyngeal flap (121), Furlow palatoplasty (72), or sphincteroplasty (9). Speech outcomes were measured via the American Cleft Palate-Craniofacial Association (ACPA) perceptual speech assessment, and MacKay-Kummer Simplified Nasometric Assessment Procedures Revised (SNAP-R) was used to measure nasalence. Comparisons of mean preoperative and postoperative outcomes were made, as well as analyses regarding surgical procedure, syndrome, cleft status, and age.
Results
Mean perceptual scores improved significantly postoperatively (p < .0001), and successful perceptual resonance was identified in 86.1% patients (n = 174). Postoperative perceptual speech scores for three ACPA domains were superior with pharyngeal flap compared to both Furlow palatoplasty and sphincteroplasty ([hypernasality: p < .001, p < .02], [audible nasal emissions: p < .002, p < .05], [velopharyngeal function: p < .001, p < .05]). Success rate was higher in pharyngeal flap (94.2%) than in Furlow palatoplasty (75.0%, p < .001) or sphincter pharyngoplasty (66.7%, p < .001). No significant difference was identified in success rate based on syndrome or cleft status.
Conclusion
Operative management of VPD is highly effective in improving perceptual speech outcomes. Given proper patient selection, all three procedures are viable treatment options for VPD. For those patients identified as appropriate to undergo a pharyngeal flap, robust improvements in speech outcomes were observed.
Graphical abstract
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