Thinned levator veli palatini muscle in velocardiofacial syndrome patients are related to widened velopharyngeal gap and production of hypernasal speech, and can give negative impact on postoperative surgical outcome of pharyngeal flap surgery.
Background:The pharyngeal flap is one of the most common secondary surgeries for the correction of velopharyngeal insufficiency (VPI) for patients with cleft palate and/or cleft lip and palate. This study aimed to demonstrate the effectiveness of the precision pharyngeal flap surgery performed by the senior author. Materials and Methods: Children with VPI, who underwent precision pharyngeal flap, were retrospectively examined. All surgical procedures were performed by the senior author. The flap size was individually configured based on the patients' preoperative nasopharyngoscopic analysis and speech function evaluation. Pre-and post-operative velopharyngeal functions were assessed using perceptual speech evaluation and nasometric analysis; factors affecting surgical outcomes were determined. Results: Of 138 patients, 112 (women: 53, men: 59) were included in analyses, according to the inclusion and exclusion criteria. The median follow-up period was 21 months (range: 9-120). Postoperative perceptual speech evaluation revealed improved velopharyngeal function in 108 (96.4%). There were no reports of postoperative hyponasality (preoperative, 1.8% versus postoperative, 0%; P ¼ 0.053). Other parameters of perceptual speech evaluation (hypernasality, nasal emission, articulation error, and intelligibility) showed significant improvement postoperatively (P < 0.01). Postoperative nasalance scores revealed sufficient postoperative resonance rating in 96% of patients. No patients experienced postoperative complications (bleeding, airway obstruction, and surgical wound dehiscence).Conclusions: Individually configured pharyngeal flaps designed based on preoperative nasopharyngoscopic examination coupled with precise surgical techniques led to the high surgery success rate for VPI treatment.
Objective
A significant number of patients with KS have cleft palate (CP) or submucous cleft palate (SMCP) and show delayed speech development. However, few reports have discussed the characteristics of CP in KS and the outcomes of postoperative speech development. The purpose of this study was to investigate the characteristics and surgical outcomes of CP in patients with KS, and to discuss the importance of the diagnosis of CP or SMCP.
Methods
We conducted a retrospective study on patients with clinically diagnosed KS who underwent palatoplasty. Clinical and surgical data were collected from patients’ medical records, and velopharyngeal function was evaluated using nasopharyngoscopy and speech analysis.
Results
In 11 cases, 5 patients had CP (45.5%) and 6 had SMCP (54.5%). Four patients who were genetically tested had a pathogenic variant of KMT2D. Seven of nine patients (77.8%) who underwent conventional palatoplasty showed velopharyngeal insufficiency and hypernasality. All patients who underwent pharyngeal flap surgery achieved velopharyngeal competency. Statistical analysis revealed a statistically significant difference in postoperative results between non-syndromic and KS patients.
Conclusion
Patients with SMCP may be more common than previously reported. The results showed that it is difficult to produce optimal results with conventional palatoplasty; therefore, pharyngeal flap surgery should be considered as a treatment to obtain favorable results. Pharyngeal flap surgery in patients with KS should be carefully designed based on speech evaluation and nasopharyngoscopic findings.
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