Perry, Jamie L.

Purpose: The purpose of this study was to investigate the relationship between perceptual ratings of hypernasality made during connected speech and velopharyngeal (VP) gap size measured in millimeters in the sagittal plane during sustained vowel production using magnetic resonance imaging (MRI). Method: A retrospective cross-sectional analysis was completed. A subgroup of 110 participants from another study with an M age of 10.1 years presenting for management of VP insufficiency was included. Perceptual ratings of hypernasality during connected speech and measurement of gap size during sustained /i/ production on MRI were performed by raters blinded to the participants' medical and surgical history. Results: There was a moderate-to-strong, positive correlation ( r = .61; p < .001) between hypernasality ratings and VP gap size measured on MRI using sustained /i/. The odds of a higher hypernasality rating increased as the gap size increased (odds ratio = 1.34; 95% CI [1.20, 1.49]; p < .001). The predicted probability for hypernasality ratings of none/minimal/mild steadily decreased as the gap size increased indicating that lower ratings of hypernasality were associated with smaller gap sizes. For the rating of “moderate” hypernasality, the predicted probability of the rating steadily increased up to 8 mm and then decreased as the gap size continued to increase. The predicted probability for a hypernasality rating of “severe” consistently increased as the gap size increased. Conclusions: Hypernasality ratings made at the connected speech level were significantly associated with VP gap size as measured during sustained vowel production. These findings suggest sustained vowel production elicited on MRI may adequately characterize VP gap size in the evaluation of VP insufficiency.

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.

Objective Compare the effectiveness of palatoplasty and pharyngoplasty procedures at resolving hypernasality in patients with 22q11.2 deletion syndrome (22q). Design Retrospective cohort study. Setting Metropolitan children's hospital. Patients Fourteen patients with 22q presenting for management of velopharyngeal insufficiency. Interventions Palatoplasty or pharyngoplasty procedure. Main Outcome Measure Resolution of hypernasality 12 months postoperatively. Results Both procedure groups had a mean preoperative velopharyngeal gap of 6.2 mm during phonation. No patient who underwent palatoplasty achieved resolution of hypernasality; 1/7 patients had worse hypernasality, 4/7 had no change, and 2/7 had improved hypernasality. In contrast, hypernasality was resolved in 6/7 patients in the pharyngoplasty group, which was significantly ( P = .03) higher than the palatoplasty group. Conclusions In patients with 22q, palatoplasty procedures may be less effective than pharyngoplasty procedures at resolving hypernasality. This may be due to underlying anatomic or physiologic differences, such as increased pharyngeal depth and hypodynamic muscles.

Objective To investigate whether flexible nasopharyngoscopy, when performed in addition to magnetic resonance imaging (MRI), influences the type of surgery selected or success of surgery in patients with velopharyngeal insufficiency (VPI). Design Cohort study. Setting A metropolitan children's hospital. Patients Patients with non-syndromic, repaired cleft palate presenting for management of VPI. Interventions MRI and nasopharyngoscopy or MRI alone for preoperative imaging of the velopharyngeal mechanism. Main Outcome Measures (1) Surgical selection and (2) resolution of hypernasality. All speech, MRI, and nasopharyngoscopy measurements were performed by raters blinded to patients’ medical and surgical history. Results Of the 25 patients referred for nasopharyngoscopy, 76% completed the exam. Of the 41 patients referred for MRI, the scan was successfully completed by 98% of patients. Completion of nasopharyngoscopy was significantly (p=0.01) lower than MRI. Surgical selection did not significantly differ (p=0.73) between the group receiving MRI and nasopharyngoscopy and the group receiving MRI alone, nor was there a significant difference between these groups in the proportion of patients achieving resolution of hypernasality postoperatively (p=0.63). Percent total velopharyngeal closure assessments on nasopharyngoscopy and MRI were strongly correlated (r=0.73). Conclusions In patients receiving MRI as part of their preoperative VPI evaluation, the addition of nasopharyngoscopy did not result in a difference in surgical selection or resolution of hypernasality. Routine inclusion of nasopharyngoscopy may not be necessary for the evaluation of velopharyngeal anatomy when MRI is available.

Background: Secondary Furlow (Furlow) and buccal myomucosal flaps (BMMF) treat velopharyngeal insufficiency by lengthening the palate and retropositioning the levator veli palatini muscles. The criteria for choosing one operation over the other remain unclear. Methods: A single-center retrospective cohort study was conducted. Thirty-two patients with nonsyndromic, repaired cleft palate were included. All patients underwent a Furlow or BMMF. Outcome measures included (1) resolution of hypernasality 12 months postoperatively, (2) degree of improvement of hypernasality severity; and (3) change in velar length, as measured on magnetic resonance imaging scans obtained preoperatively and 12 months postoperatively. All measures were performed by raters blinded to participants’ medical and surgical history. Results: Hypernasality was corrected to normal in 80% of the Furlow group and in 56% of the BMMF group. Patients receiving BMMF had more severe hypernasality during preoperative speech evaluation. Both groups had a median decrease of two scalar rating points for severity of hypernasality (P = 0.58). On postoperative magnetic resonance imaging, patients who underwent Furlow had a median increased velar length of 6.9 mm. Patients who received BMMF had a median increased velar length of 7.5 mm. There was no statistically significant difference between groups regarding increase in velar length (P = 0.95). Conclusions: Furlow and BMMF procedures increase velar length with favorable speech outcomes. The same degree of improvement for hypernasality was observed across groups, likely explained by the similar increase in velar length achieved. Anatomic changes in palate length and levator veli palatini retropositioning persist 1 year after surgery.

To determine the sensitivity and specificity of velar notching seen on nasopharyngoscopy for levator veli palatini (LVP) muscle discontinuity and anterior positioning. Nasopharyngoscopy and MRI of the velopharynx were performed on patients with VPI as part of their routine clinical care. Two speech-language pathologists independently evaluated nasopharyngoscopy studies for the presence or absence of velar notching. MRI was used to evaluate LVP muscle cohesiveness and position relative to the posterior hard palate. To determine the accuracy of velar notching for detecting LVP muscle discontinuity, sensitivity, specificity, and positive predictive value (PPV) were calculated. A craniofacial clinic at a large metropolitan hospital. Participants Thirty-seven patients who presented with hypernasality and/or audible nasal emission on speech evaluation and completed nasopharyngoscopy and velopharyngeal MRI study as part of their preoperative clinical evaluation. Among patients with partial or total LVP dehiscence on MRI, presence of a notch accurately identified discontinuity in the LVP 43% (95% CI 22-66%) of the time. In contrast, the absence of a notch accurately indicated LVP continuity 81% (95% CI 54-96%) of the time. The PPV for the presence of notching to identify a discontinuous LVP was 78% (95% CI 49-91%). The distance from the posterior edge of the hard palate to the LVP, known as effective velar length, was similar in patients with and without notching (median 9.8 mm vs 10.5 mm, P = 1.00). The observation of a velar notch on nasopharyngoscopy is not an accurate predictor of LVP muscle dehiscence or anterior positioning.

Objective To present the design and methodology for an actively enrolling comparative effectiveness study of revision palatoplasty versus pharyngoplasty for the treatment of velopharyngeal insufficiency (VPI) Design Prospective observational multicenter study Setting Twelve hospitals across the United States and Canada Participants Individuals who are 3–23 years of age with a history of repaired cleft palate and a diagnosis of VPI, with a total enrollment target of 528 participants Interventions Revision palatoplasty and pharyngoplasty (either pharyngeal flap or sphincter pharyngoplasty), as selected for each participant by their treatment team Main Outcome Measure(s) The primary outcome is resolution of hypernasality, defined as the absence of consistent hypernasality as determined by blinded perceptual assessment of a standard speech sample recorded twelve months after surgery. The secondary outcome is incidence of new onset obstructive sleep apnea. Statistical analyses will use propensity score matching to control for demographics, medical history, preoperative severity of hypernasality, and preoperative imaging findings. Results Study recruitment began February 2021. As of September 2022, 148 participants are enrolled, and 78 have undergone VPI surgery. Enrollment is projected to continue into 2025. Collection of postoperative evaluations should be completed by the end of 2026, with dissemination of results soon thereafter. Conclusions Patients with VPI following cleft palate repair are being actively enrolled at sites across the US and Canada into a prospective observational study evaluating surgical outcomes. This study will be the largest and most comprehensive study of VPI surgery outcomes to date.

This clinical case study describes the velopharyngeal anatomy and physiology in a patient who presented with SATB2-associated syndrome (SAS) and velopharyngeal insufficiency (VPI) in the absence of an overt cleft palate. The clinical presentation, treatment, outcome, and the contribution of anatomical findings from MRI to surgical treatment planning for this rare genetic disorder, SAS, are described. This case study contributes to our current understanding of the anatomy and physiology of the velopharyngeal mechanism in an individual born with SAS and non-cleft VPI. It also details the changes following bilateral buccal myomucosal flaps in this patient.

Background Non-sedated MRI is gaining traction in clinical settings for visualization of the velopharynx in children with velopharyngeal insufficiency. However, the behavioral adaptation and training aspects that are essential for successful pediatric MRI have received limited attention. Solution We outline a program of behavioral modifications combined with patient education and provider training that has led to high success rates for non-sedated velopharyngeal MRI in children.

Purpose: To investigate the musculus uvulae morphology in vivo in adults with normal velopharyngeal anatomy and to examine sex and race effects on the muscle morphology. We also sought to provide a preliminary comparison of musculus uvulae morphology in adults with normal velopharyngeal anatomy to adults with repaired cleft palate. Methods: Three-dimensional magnetic resonance imaging data and Amira 5.5 Visualization Modeling software were used to evaluate the musculus uvulae in 70 participants without cleft palate and 6 participants with cleft palate. Muscle length, thickness, width, and volume were compared among participant groups. Results: Analysis of covariance analysis did not yield statistically significant differences in musculus uvulae length, thickness, width, or volume by race or sex among participants without cleft palate when the effect of body size was accounted for. Two-sample t test revealed that the musculus uvulae in participants with repaired cleft palate is significantly shorter ( P = .008, 13.65 mm vs 16.07 mm) and has less volume ( P = .002, 51.08 mm 3 vs 97.62 mm 3 ) than participants without cleft palate. Conclusion: In adults with normal velopharyngeal anatomy, the musculus uvulae is a cylindrical oblong-shaped muscle lying on the nasal surface of the soft palate, with its greatest bulk located just nasal to the levator veli palatini muscle sling. In participants with repaired cleft palate, the musculus uvulae is substantially reduced in volume. This diminished muscle bulk located just at the point where the palate contacts the posterior pharyngeal wall may contribute to velopharyngeal insufficiency in children with repaired cleft palate.