Patient and surgical factors that affect the development of velopharyngeal insufficiency.

Velopharyngeal insufficiency (VPI) is a complication that occurs following cleft palate (CP) repair, and the patient/surgical factors that contribute to the development of VPI have not been completely described. The objectives of this study were to identify patient and surgical factors that may increase the risk of development of VPI and to identify whether there are any factors that predispose patients to the development of VPI that requires surgery versus VPI that resolves with non-operative management. Data was prospectively collected for all non-syndromic patients with a CP undergoing primary CP repair by a single surgeon between 2002 and 2018. Patient factors were recorded from patient charts, and anatomic measurements were recorded by the primary surgeon at the time of palate repair. The rates of VPI requiring surgery were compared between (1) patients with cleft lip/palate versus isolated CP, (2) patient gender, (3) unilateral or bilateral cleft, (4) complete or incomplete cleft, (5) the type of primary palatoplasty performed, and (6) the presence of palatal fistula. Data were analyzed retrospectively using a combination of Chi-square tests, t-tests, and one-way ANOVA. Overall, 229 patients were identified in this study, and the total rate of VPI was identified as 30.1%. The risk of VPI development was associated with bilateral cleft lip/palate (p = 0.01), increased pre-operative cleft width (p = 0.03), and von Langenbeck palatoplasty (p<0.01). No factors were found to be associated with operative versus non-operative VPI. Moving forward, at-risk patients should be identified early and monitored closely for the development of VPI.

Surgical interventions in velopharyngeal dysfunction: comparative perceptual speech and nasometric outcomes for three techniques.

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.

Characteristics of velopharyngeal dysfunction in 22q11.2 deletion syndrome: a retrospective case-control study.

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.

Velopharyngeal dysfunction from intranasal substance abuse: Case series and review of literature.

OBJECTIVE: Intranasal substance abuse with cocaine or opioids can result in complications involving the midline nasal and oral structures. When the defect involves the velopharyngeal musculature, this leads to velopharyngeal dysfunction (VPD). This article aims to illustrate this clinical entity through a series of four patients and a review of the literature. METHODS: A series of four cases of VPD due to intranasal narcotic use and their management are discussed. A comprehensive search in PubMed was conducted for cases of VPD associated with intranasal drug use in the English-language literature. RESULTS: Four female patients presented with symptoms of VPD, including worsening nasal regurgitation, poor speech intelligibility, and hypernasal speech. One patient presented with nasopharyngeal stenosis. All patients admitted to intranasal cocaine, methamphetamine, and/or prescription narcotics use prior to the onset of symptoms. The diagnosis was confirmed with abnormal velopharyngeal musculature seen on nasopharyngoscopy. Both conservative and surgical treatment options were proposed. A literature review identified nine cases of VPD. Erosion of the velum was seen in all cases. Reported treatments included obturator prosthetic, local flap, and free flap. Ancillary investigations were not consistently pursued to rule out other etiologies to VPD. CONCLUSION: Intranasal illicit drug use can result in destructive changes leading to VPD. This is the largest case series to date of this difficult clinical problem. Management principles including options for conservative and surgical interventions are summarized. LEVEL OF EVIDENCE: 4 Laryngoscope, 128:2721-2725, 2018.

Neurofibromatosis and velopharyngeal insufficiency: is there an association?

OBJECTIVE: Velopharyngeal insufficiency (VPI) is an often underrecognized disorder of palatal and nasopharyngeal closure that leads to the production of hypernasal speech. However, the potential clinical association between VPI and neurofibromatosis type 1 (NF1) remains undefined in the literature. The purpose of this study sought to identify and describe the potential clinical association of VPI in NF1 patients. DESIGN: A combined retrospective and prospective study. SETTING: Tertiary referral centre. METHODS: The NF1 database from 1998 to 2007 from the Medical Genetics Unit of our institution was used for this project. All NF1 patients seen during this period were sent a letter soliciting their participation in the study, which was designed to screen for the presence of VPI. MAIN OUTCOME MEASURES: Perceptual testing was undertaken using the American Cleft Palate-Craniofacial Association (ACPA) clinical database form and acoustic measurement of nasal flow, including standard nasometry and nasalance scores. A comprehensive chart review was also performed. RESULTS: One hundred forty-nine NF1 patients were identified from the database; 18 patients responded to our request for participation, with 3 additional participants recruited from recent clinical visits. Eleven of these 21 patients exhibited VPI based on perceptual evaluation and nasometry screening. CONCLUSIONS: This preliminary study attempted to identify a potential association between NF1 and VPI. Although this sample of NF1 patients was small, the finding of 11 of 21 patients being positively identified with some degree of VPI is of clinical interest, and further research is warranted.

An evaluation of communication apprehension in adolescents with velopharyngeal inadequacy.

OBJECTIVE: This investigation evaluated the construct of communication apprehension (CA) in adolescents with velopharyngeal inadequacy (VPI). DESIGN: Prospective comparative study. SETTINGS: The investigation was conducted in a hospital-based clinic, voice laboratory, and the homes of participants. PARTICIPANTS: Two groups of children participated in this study. The first group was composed of 14 children between the ages of 8 and 14 years who attended the velopharyngeal inadequacy (VPI) clinic at our institution, and the second group was composed of 14 age-matched children from the community with no history of speech or voice disorders. INTERVENTIONS: The study involved completion of the Measure of Elementary Communication Apprehension (MECA) by all 28 participants (14 experimental and 14 control subjects). Reliability of the subjects' responses to the MECA was also evaluated. OUTCOME MEASURES: Communication apprehension scores obtained from the MECA were the main focus of the study. RESULTS: Children with VPI experienced significantly higher levels of CA than did those in the control group. Moderate-to-good reliability of the MECA was found. CONCLUSIONS: 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.

Superiorly based pharyngeal flap for velopharyngeal insufficiency: intermediate and longer-term perceptual speech and nasometric outcomes.

OBJECTIVE: To evaluate the intermediate and longer-term perceptual and objective speech outcomes in velopharyngeal insufficiency (VPI) patients treated with a superiorly based pharyngeal flap. DESIGN: Retrospective review. SETTING: Tertiary care centre, London, Ontario. METHODS: Forty consecutive VPI patients (mean age 14.5 years) from 2004 to 2008 who had a first time superiorly based pharyngeal flap were included. The modified Hogan flap and lateral port control technique was used with a 70° endoscope to provide intraoperative coaxial and magnified lateral port visualization. MAIN OUTCOME MEASURES: The American Cleft Palate-Craniofacial Association (ACPA) perceptual speech assessment and nasalance measured via nasometry using the MacKay-Kummer Simplified Nasometric Assessment Procedures Revised (SNAP-R). A comparison of mean outcomes from the pretreatment versus an intermediate (ACPA 4.3 months; SNAP-R 4.0 months) and longer-term (ACPA 19.5 months) posttreatment time point was performed, and the distribution of successful outcomes was assessed. RESULTS: Mean perceptual scores improved significantly early after surgery (p < .0001; n  =  33) and in the longer term (p < .01; n  =  21), with high success rates for hypernasality (87.9% and 80.9%; p < .0001), audible nasal emission (90.9% and 90.5%; p < .0001), overall intelligibility (75.7% and 81.0%; p < .01), and compensatory articulation (81.8% and 85.7%; p < .01). Likewise, significant improvements were observed in posttreatment nasal (n  =  38; p < .01) and mean oral SNAP-R scores (n  =  39; p < .001), which demonstrated success rates of 100% (p < .0001) and 73% (p < .0001), respectively. Overcorrection was not observed in perceptual and nasometric testing. CONCLUSION: The modified Hogan flap and lateral port control technique with the use of a 70° endoscope is highly successful in treating VPI. These results indicate that speech outcome improvement occurs early after surgery and is maintained over time.

Velopharyngeal insufficiency clinic: the first 18 months.

OBJECTIVES: To profile the presentation and management of patients seen at a velopharyngeal insufficiency (VPI) clinic and to explore the role of commonly used investigations in the workup of VPI. DESIGN: Retrospective review. SETTING: Tertiary care VPI clinic. METHODS: A computerized database created at the time of patient assessment was accessed to review 75 patients seen over an 18-month span. MAIN OUTCOME MEASURES: Descriptive analysis of patient demographics, presenting complaints, pathophysiology, and treatment. The incidence of chromosome 22q microdeletion in patients presenting to such a clinic was also determined. RESULTS: The most common etiology was persistent VPI following prior cleft palate surgery. Eleven percent of presenting patients were identified with 22q microdeletion by fluorescent in situ hybridization testing. CONCLUSIONS: VPI is the result of a wide number of etiologies, with a high incidence of 22q microdeletion identified. Routine genetic testing in VPI clinics is advocated.