Velopharyngeal Gap Size During Sustained Vowel Production Correlates With Perceptual Ratings of Hypernasality in Connected Speech.

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 Mage 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.

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.

Do Palatoplasty Procedures Resolve Hypernasality as Effectively as Pharyngoplasty Procedures in Patients with 22q11.2 Deletion Syndrome?

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.

Growth Patterns Between Ages 0 and 36 Months Among US Children With Orofacial Cleft: A Retrospective Cohort Study.

BACKGROUND: Little is known about how young children with orofacial cleft grow over time. OBJECTIVE: To characterize longitudinal growth patterns from ages 0 to 36 months in US children with an orofacial cleft. DESIGN: A retrospective cohort study. PARTICIPANTS/SETTING: Children with cleft lip, cleft lip and palate, or cleft palate who were younger than age 36 months at a hospital encounter between 2010 and 2019 (N = 1334) were included. The setting was a US tertiary care children's hospital with a cleft center that serves a 5-state region. MAIN OUTCOME MEASURE: Weight-for-age z scores (WAZ) and length-for-age z scores (LAZ). STATISTICAL ANALYSES PERFORMED: Longitudinal growth patterns were characterized using generalized linear mixed models to estimate mean WAZ and LAZ from age 0 to 36 months. RESULTS: Growth in infants with cleft slowed dramatically during the first 3 to 4 months of life, rebounded with catch-up growth until age 12 months for cleft lip and cleft palate and until age 36 months for cleft lip and palate. When comparing populations, children with any type of cleft demonstrated subpar growth compared with World Health Organization standards. Growth deficits were more common in those with cleft lip and palate and cleft palate compared with those with cleft lip. The intraclass coefficient showed that most of the variability in the WAZ (65%) was between individuals, whereas 35% was within an individual. The intraclass coefficient for LAZ showed that most of the variability in the LAZ (74%) was between individuals, whereas 26% was within an individual. The proportion of variance attributable to cleft type and/or comorbidities accounted for <5% of the variance for WAZ and LAZ. WAZ and LAZ were lower in children with comorbidities than those without comorbidities with cleft and World Health Organization standards. CONCLUSIONS: Infants with cleft lip and palate, cleft palate, and a cleft with comorbidities have higher rates of poor growth than peers with cleft lip and a cleft with no comorbidities, respectively.

Magnetic Resonance Imaging of the Velopharynx: Clinical Findings in Patients with Velopharyngeal Insufficiency.

BACKGROUND: Magnetic resonance imaging (MRI) is the only imaging modality capable of directly visualizing the levator veli palatini (LVP) muscles: the primary muscles responsible for velopharyngeal closure during speech. MRI has been used to describe normal anatomy and physiology of the velopharynx in research studies, but there is limited experience with use of MRI in the clinical evaluation of patients with velopharyngeal insufficiency (VPI). METHODS: MRI was used to evaluate the velopharyngeal mechanism in patients presenting for VPI management. The MRI followed a fully awake, nonsedated protocol with phonation sequences. Quantitative and qualitative measures of the velopharynx were obtained and compared with age- and sex-matched individuals with normal speech resonance. RESULTS: MRI was completed successfully in 113 of 118 patients (96%). Compared with controls, patients with VPI after cleft palate repair had a shorter velum (P < 0.001), higher incidence of LVP discontinuity (P < 0.001), and shorter effective velar length (P < 0.001). Among patients with persistent VPI after pharyngeal flap placement, findings included a pharyngeal flap base located inferior to the palatal plane [11 of 15 (73%)], shorter velum (P < 0.001), and higher incidence of LVP discontinuity (P = 0.014). Patients presenting with noncleft VPI had a shorter (P = 0.004) and thinner velum (P < 0.001) and higher incidence of LVP discontinuity (P = 0.014). CONCLUSIONS: MRI provides direct evidence of LVP muscle anomalies and quantitative evaluation of both velar length and velopharyngeal gap. This information is unavailable with traditional VPI imaging tools, suggesting that MRI may be a useful tool for selecting surgical procedures to address patient-specific anatomic differences.

Can MRI Replace Nasopharyngoscopy in the Evaluation of Velopharyngeal Insufficiency?

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.

Factors Associated with Delayed Palatoplasty Before, During, and After the COVID-19 Pandemic.

BACKGROUND: Cleft palatoplasty is typically performed around 10 to 12 months of age in the US, and delays can negatively affect speech development. Early during COVID-19, elective surgeries were canceled. The aims of this study were to (1) identify overall risk factors for greater age at palatoplasty and (2) analyze delays in palatoplasty during COVID-19. METHODS: This study was part of a larger prospective, multicenter comparative study of speech outcomes in palatoplasty. Participants underwent palatoplasty between March 2019 and September 2022 at 18 pediatric hospitals in the United States. Ages were corrected for prematurity. Dates of palatoplasty were divided into 4 periods corresponding to different phases of the pandemic. Factors analyzed included region, language, adoption status, sex, ethnicity, race, rurality, health insurance type, and cleft type. Analyses were performed using ANOVA, Student's test, and multivariable linear regression, with a P value of ≤0.05 being significant. RESULTS: Nine hundred twenty-eight participants were included. Average corrected age at palatoplasty was 374 days. In univariable analysis, palatoplasty was performed later in children who were Hispanic (P=0.003), of a race other than White, Black, or Asian (P<0.001), and without private insurance (P<0.001). On multivariable regression, predictors of delayed palatoplasty were Hispanic ethnicity (P=0.015), from other race (P<0.001), and without private insurance (P<0.001). During COVID-19, disproportionate delays occurred in patients who were female, of other races, from nonrural areas, and on Medicaid. CONCLUSIONS: Palatoplasty was performed later in vulnerable populations. Some of these populations were also disproportionately affected by COVID-19 delays. Providers should be aware of these differences as they pertain to equitable access to craniofacial care. LEVEL OF EVIDENCE: III.

Comparative Effectiveness of Secondary Furlow and Buccal Myomucosal Flap Lengthening to Treat Velopharyngeal Insufficiency.

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.

Standardization of Videorecorded Speech Samples for Children with Cleft Palate: Methods to Facilitate High-Quality Speech Outcomes Data Collection.

BACKGROUND: Collection of high-quality videorecorded speech samples is essential for speech outcomes research. SOLUTION: Cleft palate team SLPs were trained to collect standard videorecorded speech samples in the clinic setting across 20 sites in North America. Standard training and equipment was provided. WHAT WE DID THAT IS NEW: Quality management procedures were developed and utilized to verify video quality and protocol adherence. Over 97% of speech samples collected by trained SLPs met defined quality standards.

3D Printing and Surgical Simulation for Management of Large and Giant Congenital Melanocytic Nevi.

Tissue expansion with subsequent adjacent tissue transfer is often the preferred and sometimes the only option for reconstruction of large and giant congenital melanocytic nevi. Successful reconstruction with maximal efficiency and optimal aesthetic outcome requires careful planning of the tissue transfer, which itself requires careful selection of the tissue expander size and positioning. Unfortunately, there is little opportunity to gain experience in these skills due to the rarity of this condition. In situations where there is a rare condition that requires a complex technical procedure with much interoperative decision-making, surgical experience can be supplemented with the use of surgical simulation. In this article, we report on the use of three-dimensional patient imaging, three-dimensional printing, and surgical simulation for planning the reconstruction of large and giant congenital melanocytic nevi. We describe how this technology allows us to simulate multiple different approaches to expander placement and adjacent tissue transfer. We also describe how these simulations can be used to create cutting guides to guide final incision design and reduce intraoperative decision-making. Finally, we discuss how these models can be used to educate patients and families about the process and outcomes of nevus excision and reconstruction.