Rehabilitation of Combined Hard and Soft Palate Defects with a Soft Relining Prosthesis: A Clinical Report.

It remains a significant challenge in prosthetic rehabilitation for combined hard and soft palate defects on account of two primary reasons. At first, conventional impressions can hardly get an accurate analogue and usually bring about a terrible experience for the patients. Secondly, conventional hard denture base resins used in obturator prostheses exhibit limitations in marginal sealing, undercut retention, and elastic buffering when in contact with the soft palate. This article presents a case where combined hard and soft palate defects were successfully and rapidly reconstructed by using digital intraoral impression technology and denture soft reline material.

The Source of Nasal Rustle (Nasal Turbulence): An Overview of Current Evidence.

OBJECTIVE: Nasal rustle (also called nasal turbulence) refers to a loud distracting sound that sometimes occurs with audible nasal emission (ANE) during the production of pressure-sensitive consonants in patients with velopharyngeal insufficiency (VPI). This article examines evidence for two hypotheses of causality: vibration of the soft palate (velar flutter) and periodic motion of mucus above the velopharyngeal port (turbulent mucus). CONCLUSION: A review of the relevant literature shows inconclusive evidence to support velar flutter as a cause of nasal rustle. In contrast, clinical observations and research involving high-speed nasopharyngoscopy suggest that nasal rustle is the result of turbulent mucus above a small velopharyngeal opening. Therefore, it is our contention that a plausible explanation for nasal rustle is one of turbulent mucus and not velar flutter.

The Levator Veli Palatini: Are all Segments Created Equal?

INTRODUCTION: The levator veli palatini (LVP) muscle has two segments with distinct roles in velopharyngeal function. Previous research suggests longer extravelar segments with shorter intravelar segments may lead to a more advantageous mechanism for velopharyngeal closure. The purpose of this study was to examine whether the distribution of the LVP intravelar and extravelar segments differs between children with cleft palate with and without VPI and controls. METHODS: The study included 97 children: 37 with cleft palate +/- lip with VPI, 37 controls, and 19 with cleft palate with normal resonance. Measures included mean LVP length, mean extravelar LVP length, and intravelar LVP length. RESULTS: Overall mean LVP length was similar (P = .267) between controls and children with cleft palate (with and without VPI). However, there was a significant difference (P < .001) between group for both intravelar and extravelar LVP lengths: the intravelar segment was significantly longer in those with VPI compared to controls and children with cleft palate and normal resonance; and the extravelar segment was significantly shorter in those with VPI compared to controls and children with cleft palate and normal resonance. CONCLUSIONS: Results from this study demonstrate a significant difference between the distribution of the functional segments of the LVP among children with VPI, with a more disadvantageous distribution of the muscle segments among those with VPI.

Multidisciplinary Velopharyngeal Dysfunction Evaluation Helps Detect Non-classic Cases of 22q11.2 Deletion.

OBJECTIVE: To explore the role of multidisciplinary velopharyngeal dysfunction (VPD) assessment in diagnosing 22q11.2 deletion syndrome (22q) in children. DESIGN: Retrospective cohort study. SETTING: Multidisciplinary VPD clinic at a tertiary pediatric hospital. PATIENTS, PARTICIPANTS: Seventy-five children with genetically confirmed 22q evaluated at the VPD clinic between February 2007 and February 2023, including both previously diagnosed patients and those newly diagnosed as a result of VPD evaluation. INTERVENTIONS: Comprehensive review of medical records, utilizing ICD-10 codes and an institutional tool for keyword searches, to identify patients and collect data on clinical variables and outcomes. MAIN OUTCOME MEASURES: Characteristics of children with 22q, pathways to diagnosis, and clinical presentations that led to genetic testing for 22q. RESULTS: Of the 75 children, 9 were newly diagnosed with 22q following VPD evaluation. Non-cleft VPI was a significant indicator for 22q in children not previously diagnosed, occurring in 100% of newly diagnosed cases compared to 52% of cases with existing 22q diagnosis (P = .008). Additional clinical findings leading to diagnosis included congenital heart disease, craniofacial abnormalities, and developmental delays. CONCLUSIONS: VPD evaluations, particularly the presence of non-cleft VPI, play a crucial role in identifying undiagnosed cases of 22q. This underscores the need for clinicians, including plastic surgeons, otolaryngologists, and speech-language pathologists, to maintain a high degree of suspicion for 22q in children presenting with VPI without a clear etiology. Multidisciplinary approaches are essential for early diagnosis and management of this complex condition.

Cleft Summit 2022: The Impact of a Unified Voice.

OBJECTIVE: The inaugural Cleft Summit aimed to unite experts and foster interdisciplinary collaboration, seeking a collective understanding of velopharyngeal insufficiency (VPI) management. DESIGN: An interactive debate and conversation between a multidisciplinary cleft care team on VPI management. SETTING: A two-hour discussion within a four-day comprehensive cleft care workshop (CCCW). PARTICIPANTS: Thirty-two global leaders from various cleft disciplines. INTERVENTIONS: Cleft Summit that allows for meaningful interdisciplinary collaboration and knowledge exchange. MAIN OUTCOME MEASURES: Ability to reach consensus on a unified statement for VPI management. RESULTS: Participants agreed that a patient with significant VPI and a dynamic velum should first receive a surgery that lengthens the velum to optimize patient outcome. A global, multicenter prospective study should be done to test this hypothesis. CONCLUSION: The 1st Cleft Summit successfully distilled global expertise into actionable best-practice guidelines through iterative discussions, fostering interdisciplinary collaboration and paving the way for a transformative multi-center prospective study on VPI care.

A 10-Year Nationwide Analysis of Risk Factors of Readmission and the Implications of Same-Day Discharge Following Velopharyngeal Insufficiency Correcting Surgery.

OBJECTIVE: To investigate risk factors for readmission and the implications of same-day discharge for surgical management of velopharyngeal insufficiency (VPI). DESIGN: Retrospective cohort. SETTING: Multi-institutional/national. PATIENTS AND PARTICIPANTS: Patients who underwent VPI-correcting surgery (n = 4479) were identified in the National Surgical Quality Improvement Program Pediatric database from 2012-2021. MAIN OUTCOMES MEASURE(S): 30-day unplanned readmission. RESULTS: A total of 3878 (86.6%) patients were admitted inpatient following surgical intervention, while 601 (13.4%) were discharged on the same day. Thirty-day readmission rate was 1.7% across all patients. Based on multivariate logistic regression, patient factors identified as significant predictors of 30-day readmission included ASA class 4 (OR 11.22 [95% CI 1.01-124.91]; p = 0.049), steroid use (OR 7.30 [95% CI 2.22-23.97]; p = 0.001), and gastrointestinal disease (OR 2.48 [95% CI 1.22-5.00]; p = 0.012). Upon interaction analysis, patients with cardiac or neuromuscular disease who were discharged on the same day of surgery were associated with a higher readmission rate than those admitted to the hospital (cardiac disease RR 6.72 [95% CI 1.41-32.06]; p = 0.017) and (neuromuscular disease RR 12.39 [95% CI 1.64-93.59]; p = 0.015). CONCLUSIONS: Approximately 90% of VPI-correcting procedures are completed inpatient nationwide. Cardiac and/or neuromuscular disease significantly increased the patients' readmission risk when discharged on the same day of surgery. The inpatient setting should remain the best practice as adequate resources are available to mitigate life-threatening complications.

A Single Institution 19 Year Comparison of Furlow and Straight Line Palatoplasty Techniques in Bilateral Cleft Lip and Palate.

OBJECTIVE: This study aims to compare patients' speech correcting surgery and fistula rates between the Furlow and Straight Line (SLR) palatoplasty techniques when combined with greater palatine flaps for complete bilateral cleft lip and palate (BCLP) repair. DESIGN: This was a single-center IRB approved retrospective cohort study. SETTING: This study took place at an urban tertiary academic center. PATIENTS, PARTICIPANTS: All patients with BCLP anomalies that underwent repair between January 2003 and August 2022 were included. Patients with index operations at an outside institution or incomplete medical charting were excluded. INTERVENTIONS: A total of 1552 patients underwent palatoplasty during the study period. Of these, 192 (12.4%) met inclusion criteria with a diagnosis of BCLP. MAIN OUTCOME MEASURES: Primary outcomes of this study included rate of fistula and incidence of speech correcting surgery. Secondary outcomes included rate of surgical fistula repair. RESULTS: One hundred patients underwent SLR (52.1%) and 92 Furlow repair (47.9%). There was no significant difference in fistula rates between the SLR and Furlow repair cohorts (20.7% vs. 15.0%; p = 0.403). However, SLR was associated with lower rates of speech correcting surgery when compared to the Furlow repair (12.5% vs. 29.6%; p = 0.011). CONCLUSIONS: This study compares the effect of Furlow and SLR on speech outcomes and fistula rates in patients with BCLP. Our findings suggest that SLR resulted in an almost three times lower rate of velopharyngeal dysfunction requiring surgical intervention in patients with BCLP, while fistula rates remained similar.

'Suspension Palatoplasty' - A method of surgical correction of VPI post Cleft Palate repair.

OBJECTIVE: To design the technique of 'Suspension Palatoplasty' for Velopharyngeal Insufficiency (VPI) post Cleft Palate (CP) based on optimal spatial positioning of palate at the time of VPI correction, by using a non-obstructive, high, midline pharyngeal flap for predictable velopharyngeal closure and normal speech. To evaluate the results of CP patients with VPI operated using the technique of 'Suspension palatoplasty'. DESIGN: An ambi-spective longitudinal clinical study. SETTING: Comprehensive cleft care clinic in a private trust hospital. PATIENTS, PARTICIPANTS: Patients operated using the 'Suspension Palatoplasty' technique for VPI post CP repair between 2014 and 2018 with a minimum follow-up period of 5 years. INTERVENTIONS: 'Suspension Palatoplasty' - Double Opposing Z (DOZ) plasty with palatal myoplasty is used to revise soft palate and a narrow superiorly based pharyngeal flap is used to suspend it for a dynamic velopharyngeal closure. MAIN OUTCOME MEASURE: Speech outcome and surgical complications. RESULTS: 70 out of 119 studied were found to have normal speech (59%), and another 25 patients (21%) had acceptable speech. Thus 95 out of 119 patients (80%) had normal or near-normal speech and did not require any further speech therapy or surgeries. 12 patients had snoring without difficulty in breathing. One patient had symptoms suggestive of obstructive sleep apnea. Younger patients had a higher percentage of normal speech outcomes. Many of our adult patients also attained normal speech. CONCLUSION: 'Suspension Palatoplasty' aims to achieve normal speech with little effort. It has minimal side effects. The author has performed 403 cases to date.

Surgical treatment of velopharyngeal dysfunction: Incidence and associated factors in the Swedish cleft palate population.

INTRODUCTION: Speech in children with cleft palate can be affected by velopharyngeal dysfunction, which persists after primary palate repair. The incidence of surgery to correct velopharyngeal dysfunction in this patient group has previously been reported as 2.6-37%. We aimed to investigate the incidence of velopharyngeal dysfunction surgery in Swedish children with cleft palate and to examine potential associations of independent variables with this incidence. METHODS: In this cohort study, we analysed data from the Swedish cleft lip and palate quality registry for 1093 children with cleft palate with or without cleft lip. Kaplan-Meier analysis was used to estimate the risk of having velopharyngeal dysfunction surgery. Multivariable Cox proportional hazards models were used to estimate the associated effect of cleft subtype, additional diagnoses, gender, and age at and number of stages for primary palate repair on the primary outcome. RESULTS: The risk of having velopharyngeal dysfunction surgery was 25.6%. Complete primary palate repair after the age of 18 months or in more than one stage was associated with a higher risk, but it could not be determined which of these was the more significant factor. Cleft soft palate was associated with a significantly lower risk than other cleft subtypes. CONCLUSIONS: Primary palate repair at a higher age or in more than one stage may increase the risk of having velopharyngeal dysfunction surgery. Further analysis of potential unknown confounding factors and the association between the incidence of velopharyngeal dysfunction and surgery to correct this condition is needed.

Management of Velopharyngeal Dysfunction (VPD) Following Cleft Palate Repair: A Comprehensive Decision-Making Process Based on Severity and Structural Deficiencies.

OBJECTIVE: To describe a comprehensive decision-making process for surgical correction of structural Velopharyngeal Dysfunction (VPD) following cleft palate repair and evaluate its efficacy. DESIGN: Retrospective study. SETTING: Tertiary care hospital. PATIENTS: 300 consecutive patients with unilateral or bilateral cleft lip and palate (CLP) or isolated cleft palate (CP) diagnosed with clinical VPD following cleft palate repair between 2009 and 2014. Of these 206 patients had structural VPD and underwent surgical correction. INTERVENTIONS: Surgical corrections were carried out according to the comprehensive two stage decision making process developed by the investigators. Step 1 of decision-making involved visualisation of the VP sphincter function by nasoendoscopy. This was followed by step 2 which involved per-operative identification of scarring, tissue loss, hypoplasia and other structural deficiencies in the soft palate and septal mucoperiosteum. The choice of operation was then made from a repertoire of interrelated and escalating surgical procedures consisting of palate revision and pharyngoplasties ranging from most anatomical to the least. MAIN OUTCOME MEASURES: Evidence of postoperative restoration of VP function on nasoendoscopy, evaluation of speech for hypernasality, understandability, acceptability and symptoms of obstructive sleep apnea. RESULTS: Complete VP closure was demonstrated in 94% of patients treated using this algorithm. There was significant improvement in all speech parameters (p < 0.00001). CONCLUSION: Our comprehensive decision-making process is designed to effectively correct structural VPD according to the severity of structural and functional deficiencies in the soft palate and avoid over treatment.

Preoperative Imaging in Patients with 22q11 Deletion Syndrome Undergoing Velopharyngeal Surgery.

OBJECTIVE: To evaluate the utility of preoperative imaging before velopharyngeal dysfunction (VPD) surgery in children with 22q11 Deletion Syndrome (22qDS) in evaluating internal carotid artery (ICA) medialization. DATA SOURCES: PubMed, Scopus, and CINAHL. REVIEW METHODS: Following PRISMA guidelines, a systematic review was performed. Studies of children with 22qDS who underwent preoperative imaging (MRA or CTA) to identify ICA anomalies were included. High-risk medialized ICAs were defined as either submucosal, retropharyngeal, Pfeiffer Grade III-IV, or <3 mm from the pharyngeal mucosa. Meta-analyses of proportions were performed. RESULTS: Eleven studies met inclusion criteria, comprising 398 patients with 22qDS (weighted mean age 7.6 years). In 372 patients with imaging, the rate of ICA medialization on imaging was 47.1% (95%CI 29.2-65.5), of which 46.3% (95%CI 27.4-65.8) were determined high risk. Operative plans were modified in 19.4% (95%CI 5.7-38.8) of 254 surgeries due to medialized ICA. In studies attempting to use nasopharyngoscopy pulsations to identify medialization for 214 patients, the true-positive rate was 53.9% (95%CI 27.5-79.2) and the false-positive rate was 16.2% (95%CI 7.9-26.8). Nine of eleven studies (81.8%) recommended universal preoperative imaging of the ICAs in children with 22qDS undergoing VPD surgery. No cases of perioperative bleeding secondary to ICA injury were identified. CONCLUSION: Although most studies endorse routine preoperative imaging to assess for ICA medialization in children with 22qDS undergoing VPD surgery, only a minority of these cases led to surgical modification. Additional studies are needed to compare outcomes in children with and without preoperative imaging given the low rates of ICA injury in the literature. LEVEL OF EVIDENCE: N/A Laryngoscope, 2023.

Speech Outcomes of Buccal Myomucosal Flap Palatal Lengthening for Treatment of Velopharyngeal Insufficiency: Systematic Literature Review and Meta-Analysis.

OBJECTIVE: Buccal myomucosal flaps (BMF) anatomically lengthen the palate in the treatment of velopharyngeal insufficiency (VPI). We systematically reviewed the existing literature on speech outcome of BMF palatal lengthening. DESIGN: Three databases were used to identify studies of interest published in English. Studies that did not use standardized speech assessments were excluded. PRISMA checklist was followed, and the risk of bias in the included studies was assessed. SETTING: Long-term follow up of patients. PATIENTS: With history of cleft palate presenting with VPI. INTERVENTION: BMF palatal lengthening. MAIN OUTCOME MEASURE: Random-effects model meta-analyses were performed for hypernasality, intelligibility, and nasal air emission score improvements, which were derived from reported preoperative and postoperative scores, and controlled for variability of scales and timing of postoperative assessment. RESULTS: From the initial 7115 articles, 13 were included in this review. Two of these had a significant patient overlap and a study with a smaller patient population was excluded. All 12 included articles met the National Institutes of Health Quality Assessment Tool criteria. Six retrospective studies evaluated 230 patients and six prospective studies evaluated 181 patients. The most common indications for BMF were large size of the velopharyngeal gap and prior surgery for VPI. Meta-analyses demonstrated effect sizes below zero, confirming the improvement of standardized assessment scores in patients with VPI after BMF palatal lengthening. Egger regressions revealed low risk of publication bias. CONCLUSIONS: BMF palatal lengthening provides adequate treatment for VPI in patients with large velopharyngeal gap size and a history of prior unsuccessful surgery.

Using "Real-World Data" to Study Cleft Lip/Palate Care: An Exploration of Speech Outcomes from a Multi-Center US Learning Health Network.

OBJECTIVE: To assess the ability of a cleft-specific multi-site learning health network registry to describe variations in cleft outcomes by cleft phenotypes, ages, and treatment centers. Observed variations were assessed for coherence with prior study findings. DESIGN: Cross-sectional analysis of prospectively collected data from 2019-2022. SETTING: Six cleft treatment centers collected data systematically during routine clinic appointments according to a standardized protocol. PARTICIPANTS: 714 English-speaking children and adolescents with non-syndromic cleft lip/palate. INTERVENTION: Routine multidisciplinary care and systematic outcomes measurement by cleft teams. OUTCOME MEASURES: Speech outcomes included articulatory accuracy measured by Percent Consonants Correct (PCC), velopharyngeal function measured by Velopharyngeal Competence (VPC) Rating Scale (VPC-R), intelligibility measured by caregiver-reported Intelligibility in Context Scale (ICS), and two CLEFT-Q™ surveys, in which patients rate their own speech function and level of speech distress. RESULTS: 12year-olds exhibited high median PCC scores (91-100%), high frequency of velopharyngeal competency (62.50-100%), and high median Speech Function (80-91) relative to younger peers parsed by phenotype. Patients with bilateral cleft lip, alveolus, and palate reported low PCC scores (51-91%) relative to peers at some ages and low frequency of velopharyngeal competency (26.67%) at 5 years. ICS scores ranged from 3.93-5.0 for all ages and phenotypes. Speech Function and Speech Distress were similar across phenotypes. CONCLUSIONS: This exploration of speech outcomes demonstrates the current ability of the cleft-specific registry to support cleft research efforts as a source of "real-world" data. Further work is focused on developing robust methodology for hypothesis-driven research and causal inference.

'Suspension Palatoplasty' - A new Method of Primary Palate Repair for Speaking Un-Repaired Clefts.

OBJECTIVE: To design a technique of palate repair with predictable Velo Pharyngeal (VP) closure and normal speech - 'Suspension Palatoplasty' in speaking unrepaired Cleft Palate (CP) patients. To evaluate the results of patients with CP operated using the technique of 'Suspension palatoplasty' from 2014 -2018 in terms of surgical complications and speech outcomes. DESIGN: An ambi-spective longitudinal clinical study. SETTING: Comprehensive cleft care clinic in a private trust hospital. PATIENTS/ PARTICIPANTS: Patients above 6 years of age with isolated CP, cleft lip and palate (CLP), operated using 'Suspension Palatoplasty' technique between 2014 and 2018 were included with minimum follow up period of 5 years. Subjective speech evaluation was done by two speech therapists and analysed. INTERVENTION: Author designed the technique of 'Suspension Palatoplasty' which aims to suspend the soft palate repaired by 'Hybrid Palatoplasty' technique, close to the nasopharynx using narrow pharyngeal flap with the pedicle based superiorly at adenoids, for an acceptable VP closure. MAIN OUTCOME MEASURES: Speech outcome and surgical complications were assessed. RESULTS: Out of 94 cases operated with age ranging from 6 to 45 years, 59 (62.8%) had normal speech, 12 (12.8%) had articulation errors but with no Velo-Pharyngeal Insufficiency (VPI) and 23 patients (24.4%) had hypernasality/ nasal emission (Suspected VPI). None of them had difficulty in breathing but one patient had snoring. CONCLUSION: 'Suspension Palatoplasty' is a simple primary operation with excellent speech outcomes with no obstructive sequelae in patients with speaking unrepaired cleft palate.

Pearls and pitfalls in contemporary management of marginal velopharyngeal inadequacy among children with cleft palate.

Marginal velopharyngeal inadequacy (MVPI) is a particular status of velopharyngeal closure after cleft palate repair. The physiological and phonological characteristics of patients with MVPI are significantly different from those with typical velopharyngeal insufficiency. The pathological mechanisms and diagnostic criteria of MVPI are still controversial, and there is limited evidence to guide the selection of surgical and non- surgical management options and a lack of recognized standards for treatment protocols. Based on a systematic study of the relevant literatures, this review identifies specific problems that are currently under-recognized in the diagnosis and treatment of MVPI and provides guidelines for further exploration of standardized and reasonable intervention protocols for MVPI.

Assessing the Agreement of Hypernasality and Audible Nasal Emission Ratings Between Audio-Recordings and a Clinic Setting.

Assess agreement of hypernasality and audible nasal emission (ANE) ratings between audio-recordings and a clinic setting.Cross-sectional study using retrospective clinical recordings.Audio-recording ratings by two trained speech language pathologists.Percent agreement and intra- and inter-rater reliability of perceptual ratings.Intra-rater reliability (AC2) of 167 audio-recorded speech samples for the primary and secondary raters, respectively, was 0.82 and 0.79 for hypernasality; for ANE, it was 0.57 and 0.75. Inter-rater reliability was 0.77 for hypernasality and 0.63 for ANE. When comparing ratings made from audio-recording versus the original clinical ratings, intra-rater reliability was 0.85 and 0.61 (primary and secondary rater, respectively) for hypernasality and 0.21 and 0.34 for ANE.Ratings for hypernasality made from audio recordings were consistent with clinical evaluation, while ratings of ANE were not. ANE ratings made from audio recordings may not be a valid measure of velopharyngeal insufficiency speech characteristics.

Preliminary Development of an MRI Atlas for Application to Cleft Care: Findings and Future Recommendations.

OBJECTIVE: To introduce a highly innovative imaging method to study the complex velopharyngeal (VP) system and introduce the potential future clinical applications of a VP atlas in cleft care. DESIGN: Four healthy adults participated in a 20-min dynamic magnetic resonance imaging scan that included a high-resolution T2-weighted turbo-spin-echo 3D structural scan and five custom dynamic speech imaging scans. Subjects repeated a variety of phrases when in the scanner as real-time audio was captured. SETTING: Multisite institution and clinical setting. PARTICIPANTS: Four adult subjects with normal anatomy were recruited for this study. MAIN OUTCOME: Establishment of 4-D atlas constructed from dynamic VP MRI data. RESULTS: Three-dimensional dynamic magnetic resonance imaging was successfully used to obtain high quality dynamic speech scans in an adult population. Scans were able to be re-sliced in various imaging planes. Subject-specific MR data were then reconstructed and time-aligned to create a velopharyngeal atlas representing the averaged physiological movements across the four subjects. CONCLUSIONS: The current preliminary study examined the feasibility of developing a VP atlas for potential clinical applications in cleft care. Our results indicate excellent potential for the development and use of a VP atlas for assessing VP physiology during speech.

Incorporating Velopharyngeal MRI into the Clinical Decision-Making Process for a Patient Presenting with Velopharyngeal Dysfunction Following a Failed Palatoplasty.

This clinical report describes the implementation of magnetic resonance imaging (MRI) to evaluate a patient with long-standing velopharyngeal dysfunction. She was referred to the craniofacial clinic at age 10 with no prior surgical history and subsequently completed a Furlow palatoplasty due to a suspected submucous cleft palate. However, results were unfavorable with minimal improvement in speech or resonance. The clinical presentation, treatment, outcomes, and contributions from MRI for secondary surgical planning are described. Addition of MRI into the clinical workflow provided insights into the anatomy and physiology of the velopharyngeal mechanism that were unable to be obtained from nasendoscopy and speech evaluation alone.

Simultaneous Furlow Palatoplasty and Tonsillectomy for the Treatment of Velopharyngeal Insufficiency and Tonsillar Hypertrophy.

OBJECTIVE: To determine whether performing tonsillectomy at the time of Furlow palatoplasty for the treatment of cleft palate related velopharyngeal insufficiency (VPI) incurs increased surgical complications or compromises speech outcomes. DESIGN: A retrospective review of patients who had Furlow palatoplasty and the outcomes of surgery in the treatment of cleft palate related VPI. SETTING: A single academic center between January 2015 and January 2022. PARTICIPANTS: Patients with submucous cleft (SMC) palate or patients with prior straight line primary palatoplasty presenting with VPI. INTERVENTIONS: Simultaneous conversion Furlow palatoplasty and tonsillectomy. MAIN OUTCOME MEASURE(S): Primary outcome measures include preoperative and postoperative Modified Pittsburgh Weighted Speech Scale (mPWSS), and postoperative surgical complications. RESULTS: Eight patients (25%) underwent Furlow palatoplasty and concomitant tonsillectomy, while 24 patients (75%) underwent Furlow palatoplasty alone. A significantly lower median postoperative mPWSS score, corresponding to better velopharyngeal function, was reported for patients in the Furlow-tonsillectomy group (0, IQR 0-0) compared to the Furlow only group (1, IQR 0-9, p = 0.046). No surgical complications were encountered in either group. Five patients (20.8%) in the Furlow only group required subsequent surgery for persistent VPI. No patients in the Furlow-tonsillectomy group required additional surgical treatment for VPI (0%, p = 0.16). CONCLUSIONS: Tonsillectomy at time of Furlow palatoplasty is utilized in patients with both VPI and baseline tonsillar hypertrophy to lessen the risk of postoperative obstructive breathing. Tonsillectomy performed concurrently with Furlow palatoplasty is safe, without increased risk of surgical complications, and does not compromise post-Furlow palatoplasty speech outcomes.

Sphincter Pharyngoplasty for Velopharyngeal Dysfunction: Impact of 22q11.2 Deletion Syndrome.

OBJECTIVE: Patients with 22q11.2 deletion syndrome (22q11DelS) often present with velopharyngeal dysfunction (VPD). VPD in patients with 22q11DelS is multifactorial beyond velopharyngeal insufficiency (VPI) alone, and differences in surgical outcomes are poorly understood. Our objective was to determine whether patients with 22q11DelS have an increased risk for persistent VPI after sphincter pharyngoplasty compared to patients without 22q11DelS. METHODS: We completed a retrospective cohort study of patients with 22q11DelS undergoing sphincter pharyngoplasty between 1995 and 2019 using a VPD clinic database. Patients with 22q11DelS were compared to a cohort of 2:1 frequency-matched (age, degree of velopharyngeal closure) patients without 22q11DelS. Variables included patient characteristics, surgical history, perceptual speech evaluation, and degree of closure on nasopharyngoscopic evaluations. Primary outcomes included postoperative VPI severity and hypernasality. Speech and nasopharyngoscopic characteristics were compared using Fisher's exact test. Postoperative VPI severity and hypernasality were compared between groups via relative risks (RR) from mixed effects Poisson regression models, with random effects of age and velopharyngeal closure. RESULTS: 134 patients (51 22q11DelS, 83 matched) were included, with mean age of 7.3 years (standard deviation 3.0) and 50% male. Cohorts had similar preoperative speech characteristics and nasopharyngoscopic findings. Patients with 22q11DelS had similar postoperative VP function as patients without 22q11DelS (RR 0.85, CI 0.46-1.57 for VPI severity, RR 0.83, CI 0.45-1.53 for hypernasality). Even after adjusting by preoperative variables, no differences were seen between both groups. CONCLUSION: Matched for age and pre-operative velopharyngeal closure, patients with and without 22q11DelS and VPI had similar benefits after sphincter pharyngoplasty. LEVEL OF EVIDENCE: Non-randomized controlled cohort study, 3 Laryngoscope, 133:2813-2820, 2023.