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.

An Assessment of Adverse Events in Patients with 22q11.2 Deletion Syndrome Undergoing Palatoplasty: An Analysis of the NSQIP Pediatric Database.

BACKGROUND: The National Surgical Quality Improvement Program (NSQIP) Pediatric database has been used to identify factors related to adverse surgical outcomes in pediatric and craniofacial surgical procedures. Focusing on a historically "higher-risk" population, our aim was to assess the impact of demographics, comorbidities, and 22q11.2 deletion syndrome (22QDS) diagnosis on 30-day postoperative complications in patients undergoing primary palatoplasty. METHODS: We used the 2012-2020 NSQIP Pediatric database to identify patients ≤3 years with and without 22q11.2 deletion syndrome who underwent primary palatoplasty. Demographics, comorbidities, and perioperative characteristics were compared between those with and without 22QDS. Logistic regression was used to determine if children with 22QDS were more likely to experience a 30-day postoperative complication or readmission. RESULTS: There were 10,745 patients ≤3 years old who underwent primary palatoplasty; 83 (0.8%) of whom had 22QDS and 10,662 (99.8%) did not. Children with 22QDS were older when they underwent primary palatoplasty and more likely to have comorbidities. A total of 513 patients (4.8%) experienced a postoperative complication within 30 days and 255 were readmitted (2.4%). Of the 513, 8 (9.6%) had a 22QDS diagnosis and 505 (4.7%) did not. A diagnosis of 22QDS was not a significant independent risk factor for a complication (adjusted odds ratio (aOR) = 1.13; 95% confidence interval (CI): 0.50-2.54) or readmission (aOR = 1.74; 95% CI: 0.74-4.13) within 30 days. CONCLUSION: This study found that the diagnosis of 22QDS was not an independent predictor of post-palatoplasty complication risk, and in fact 30-day complications are rare for those patients undergoing cleft palate repair, even among those patients with 22QDS.

Feeding Management and Palate Repair Timing in Infants with Cleft Palate with and without Pierre Robin Sequence: A Multisite Study.

OBJECTIVES: Compare the feeding management practices in infants with cleft palate with and without Pierre Robin sequence (PRS) and determine if specific feeding difficulties or interventions predict delayed palate repair. DESIGN: Retrospective cross-sectional study. SETTING: Seventeen cleft palate teams contributed data. PATIENTS: 414 infants were included in this study: 268 infants with cleft palate only and 146 infants with cleft palate and PRS. PROCEDURES: Data were collected via parent interview and electronic health records. MAIN OUTCOME MEASURES: Outcomes for the primary objective included categorical data for: history of poor growth, feeding therapy, milk fortification, use of enteral feeding, and feeding difficulties. The outcome for the secondary objective was age in months at primary palate repair. RESULTS: Infants with PRS had a significantly higher prevalence of feeding difficulties (81% versus 61%) and poor growth (29% versus 15%) compared to infants with cleft palate only. Infants with PRS received all feeding interventions-including feeding therapy, milk fortification, and enteral feeding-at a significantly higher frequency. Infants with PRS underwent primary palate repair at a mean age of 13.55 months (SD = 3.29) which was significantly (P < .00001) later than infants with cleft palate only who underwent palate repair at a mean age of 12.05 months (SD = 2.36). Predictors of delayed palate repair included diagnosis of PRS as well as Hispanic ethnicity and a history of poor growth. CONCLUSIONS: These findings can be used to establish clinical directives focused on providing early, multimodal feeding interventions to promote optimal growth and timely palate repair for infants with PRS.

A Comparative Effectiveness Study of Speech and Surgical Outcomes: Study Overview.

AIMS: To provide an overview of the Cleft Outcomes Research NETwork (CORNET) and the CORNET Speech and Surgery study. The study is (1) comparing speech outcomes and fistula rate between two common palate repair techniques, straight-line closure with intra-velar veloplasty (IVVP) and Furlow Double-Opposing Z-palatoplasty (Furlow Z-plasty); (2) summarizing practice variation in the utilization of early intervention speech-language (EI-SL) services; and (3) exploring the association between EI-SL services and speech outcomes. DESIGN: Prospective, longitudinal, observational, comparative effectiveness, multi-center. SITES: Twenty sites across the United States. PARTICIPANTS: One thousand two hundred forty-seven children with cleft palate with or without cleft lip (CP ± L). Children with submucous cleft palate or bilateral sensorineural severe to profound hearing loss were excluded from participation. INTERVENTIONS: Straight-line closure with IVVP or Furlow Z-plasty based on each surgeon's standard clinical protocol. MAIN OUTCOME MEASURE(S): The primary study outcome is perceptual ratings of hypernasality judged from speech samples collected at 3 years of age. Secondary outcomes are fistula rate, measures of speech production, and quality of life. The statistical analyses will include generalized estimating equations with propensity score weighting to address potential confounders. CURRENT PROGRESS: Recruitment was completed in February 2023; 80% of children have been retained to date. Five hundred sixty two children have completed their final 3-year speech assessment. Final study activities will end in early 2025. CONCLUSIONS: This study addresses long-standing questions related to the effectiveness of the two most common palatoplasty approaches and describes CORNET which provides an infrastructure that will streamline future studies in all areas of cleft care.

The Impact of Race and Ethnicity on Surgical Risk and Outcomes Following Palatoplasty: An Analysis of the NSQIP Pediatric Database.

OBJECTIVE: To determine how race and ethnicity affect palatoplasty 30-day outcomes. DESIGN: Retrospective review. PATIENTS/SETTING: The 2012 to 2019 National Surgical Quality Improvement Program (NSQIP) Pediatric database was used to identify patients ≤ 2 years who underwent primary palatoplasty. We compared demographics, comorbidities, and 30-day outcomes among different racial and ethnic groups. Logistic regression was used to determine independent risk factors for adverse events. MAIN OUTCOME MEASURES: Increased risk for adverse events and postoperative surgical outcomes, including complications, readmission, and prolonged length of stay. RESULTS: A total of 8537 patients were identified in the database. African-American patients had the highest proportion of premature infants and infants with a BMI < 15% at the time of repair. Asian patients underwent palatoplasty at a later age compared to other races (12.7 months vs 11.7-12.1 months). Postoperatively, the odds of a complication were significantly higher in Asian patients (aOR = 1.73, 95% CI: 1.17-2.57) and other/unknown patients (aOR = 1.40, 95% CI: 1.05-1.86), but not among African American (aOR = 1.02, 95% CI: 0.70-1.47) or Hispanic (aOR = 0.93, 95% CI: 0.69-1.26) patients. Other/unknown patients were more likely to require postoperative ventilation (aOR = 2.34, 95% CI: 1.38-3.95). The odds of readmission were highest in Asian and other/unknown patients. African American, Hispanic, and other/unknown patients were more likely than Caucasian patients to be hospitalized > 2 days postoperatively. CONCLUSION: This study highlights ethnic differences in presentation and 30-day outcomes following palatoplasty. Further evaluation of disparities in cleft care should be performed to improve healthcare access and surgical outcomes.

Buccal Fat Pad in Primary and Secondary Cleft Palate Repair: A Systematic Review of the Literature.

OBJECTIVE: To review the evidence supporting the use of buccal fat pad (BFP) in primary and secondary cleft palate repair and its short- and long- term clinical outcomes. DESIGN: Systematic review conducted by 2 independent reviewers following PRISMA guidelines. SETTING: NONE PARTICIPANTS: Articles were identified from three databases (Pubmed/Medline, Embase and Web of Science). Search terms included "cleft palate", "palatoplasty", "palate repair", "buccal fat pad". INTERVENTIONS: Use of BFP in primary and secondary cleft palatoplasty. MAIN OUTCOME MEASURES: Primary outcomes were immediate postoperative complications, postoperative fistula, and maxillary growth. Secondary outcomes were palatal length, speech, and donor site morbidity. RESULTS: Ninety-one reports were retrieved after excluding duplicates. Twenty-three studies were included (13 case series and 10 comparative studies). Overall level of evidence was low. Randomized and non-randomized studies had a high risk of bias. In primary palatoplasty, BFP was more frequently used filling lateral relaxing incisions(57.4%), or in the hard-soft palate junction and covering mucosal defects(30.1%). In these patients, post operative fistula incidence was 2.8%. Two studies found wider transverse maxillary dimensions after BFP use. No higher incidence of bleeding, infection, dehiscence, or flap necrosis was reported. In secondary palatoplasty, no recurrent fistulas were reported for patients undergoing BFP for fistula repair. CONCLUSIONS: BFP appears to be associated with a favorable impact in fistula prevention and management, as well as in transverse maxillary growth. However, there is a high heterogeneity among studies, high risk of bias and overall low quality of evidence. More high-quality research with long-term follow-up is warranted.

Assessment of the Velopharyngeal Mechanism at Rest and During Speech in Children With 22q11.2DS: A Cross-Sectional Study.

OBJECTIVE: Velopharyngeal dysfunction (VPD) associated with 22q11.2 deletion syndrome (22q11.2DS) has a complex etiology. This study had 3 aims: (1) assess differences in velopharyngeal and levator muscle configuration during rest versus sustained speech production (2) compare differences in velopharyngeal changes between children with and without 22q11.2DS (3) examine the relationship between adenoid thickness, pharyngeal depth, and velopharyngeal changes. DESIGN: Cross-sectional. METHODS: A total of 22 participants, 11 with 22q11.2DS and 11 controls with normal speech and velopharyngeal anatomy (ages 4-12 years), underwent nonsedated MRI at rest and during sustained /i/. Differences in velar and levator muscle contraction across the 2 different conditions were analyzed, using matched paired t-tests. Mean differences across participant groups were examined. Correlation analyses were also conducted. RESULTS: When comparing differences between rest and sustained phoneme production (aim 1), significant (P < .05) differences were noted for all velar and levator muscle variables. For differences in velopharyngeal changes between children with and without 22q11.2DS (aim 2), VP ratio and effective VP ratio were noted to be significantly different. Pharyngeal depth and adenoid thickness were correlated with velar and levator muscle change measures and ratios (aim 3). CONCLUSION: Results from this study provide quantitative in vivo measurements of the contracted levator muscle and velum in young children with 22q11.2DS. Results demonstrated that VP ratio and EVP ratio are significantly different between children with and without 22q11.2DS and that pharyngeal depth is a strong clinical determinant of VPD in children with 22q11.2DS.

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.

Airway and Feeding Outcomes in Pierre Robin Sequence: A Comparison of Three Management Strategies.

BACKGROUND: Controversy remains regarding optimal management of Pierre Robin sequence (PRS). The goal of this study was to compare airway and feeding outcomes in infants with PRS who underwent surgical intervention, specifically mandibular distraction osteogenesis (MDO) or tongue-lip adhesion (TLA), or who had conservative management (CM) without surgery. METHODS: All consecutive patients treated for PRS at a pediatric academic medical center, with at least one year follow-up, were included. Patients who underwent tracheostomy as an index procedure were excluded. Patients were divided into those who underwent MDO, TLA or CM. Feeding status and data from initial and follow-up polysomnograms were collected. Comparisons between groups were made using the Kruskal-Wallis test, followed by Mann-Whitney pairwise comparison with a Bonferroni correction, when appropriate. RESULTS: 67 neonates were included. 19 underwent TLA, 29 underwent MDO and 19 underwent CM. The proportions of syndromic patients were similar between groups. Patients undergoing CM had the lowest baseline AHI (9.1), but there were no significant differences between TLA (20.1) and MDO (25.4). At follow-up, the three groups had similar mean AHI (MDO 1.3, TLA 4.2, CM 4.5). A similar proportion of patients achieved AHI 5 or less (TLA 89.5%, MDO 96.6%, CM 84.2%). At one year, there were no significant differences in weight percentiles or in risk of failure-to-thrive between groups. One patient from the TLA group required a tracheostomy. CONCLUSION: The three treatment modalities achieved high airway and feeding success rates. All three modalities should have a place in the armamentarium of the craniofacial surgeon.

Cleft Palate Repair Postoperative Management: Current Practices in the United States.

OBJECTIVE: To describe current postoperative management practices following cleft palate repair. DESIGN: A survey was administered to cleft surgeons to collect information on their demographic characteristics, surgical training, surgical practice, and postoperative management preferences. SETTING: Eighteen tertiary referral hospitals across the United States.Participants: Surgeons (n = 67) performing primary cleft palate repair. RESULTS: Postoperative diet restrictions were imposed by 92% of surgeons; pureed foods were allowed at one week after surgery by 90% of surgeons; a regular diet was allowed at one month by 80% of surgeons. Elbow immobilizers and/or mittens were used by 85% of surgeons, for a median duration of two weeks. There was significant disagreement about postoperative use of bottles (61% allow), sippy cups (68% allow), pacifiers (29% allow), and antibiotics (45% prescribe). Surgeon specialty was not associated with any aspect of postoperative management (p > 0.05 for all comparisons). Surgeon years in practice, a measure of surgeon experience, was associated only with sippy cup use (p < 0.01). The hospital at which the surgeon practiced was associated with diet restrictions (p < 0.01), bottle use (p < 0.01), and use of elbow immobilizers or mittens (p < 0.01); however, many hospitals still had disagreement among their surgeons. CONCLUSIONS: Surgeons broadly agree on diet restrictions and the use of elbow immobilizers or mittens following palate repair. Almost all other aspects of postoperative management, including the type and duration of diet restriction as well as the duration of immobilizer use, are highly individualized.

Improving Psychosocial Risk Assessment and Service Provision for Craniofacial Team Patients: A Quality Improvement Project.

To improve psychosocial risk assessment and service provision for children with craniofacial conditions presenting for annual interdisciplinary team visits.Institute for Healthcare quality improvement model.U.S. pediatric academic medical center.Caregivers of children ages 0-17 years with craniofacial conditions presenting for 1692 team visits between August 2017 and July 2019.Key drivers included: (1) standardizing pre-visit triage processes; (2) administering the Psychosocial Assessment Tool-Craniofacial Version (PAT-CV); (3) utilizing PAT-CV scores in real time to add patients to psychosocial provider schedules; and (4) family education. Interventions included improving patient screening, increasing PAT-CV completion rate, altering clinic flow, providing patient and parent education about psychosocial services, and altering team member roles to fully integrate PAT-CV administration and scoring in the clinic.The primary outcome was the percentage of patients identified for psychosocial consultations via nurse triage, PAT-CV score, family or provider request who completed consultations. The secondary outcome was the percentage of patients completing needed psychosocial consultations based on elevated PAT-CV scores.Use of the PAT-CV resulted in an increase in the percentage of patients with elevated psychosocial risk who received a psychosocial consultation from 86.7% to 93.4%. The percentage of children receiving psychosocial consultation at their annual team visit due to elevated PAT-CV scores increased from 72% to 90%.Integrating a validated psychosocial risk screening instrument can improve risk identification and psychosocial consultation completion. A combination of risk screening approaches may be indicated to identify patients in need of psychosocial services.

Core Outcome Set for Reporting Outcomes of Interventions for Velopharyngeal Dysfunction: Final Results of the COS-VPD Initiative.

OBJECTIVE: To date, the recording of outcomes of interventions for velopharyngeal dysfunction (VPD) has not been standardized. This makes a comparison of results between studies challenging. The aim of this study was to develop a core outcome set (COS) for reporting outcomes in studies examining the management of VPD. DESIGN: A two-round Delphi consensus process was used to develop the COS. PATIENTS, PARTICIPANTS: The expert Delphi panel comprised patients and caregivers of patients with VPD, surgeons and speech and language therapists specializing in cleft palate, and researchers with expertise in VPD. INTERVENTIONS: A long list of outcomes was derived from the published literature. In each round of a Delphi survey, participants were asked to score outcomes using the Grading of Recommendations, Assessment, Development, and Evaluations scale of 1 to 9, with 1 to 3 labeled "not important," 4 to 6 labeled "important but not critical," and 7 to 9 labeled "critical." MAIN OUTCOME MEASURE: Consensus criteria were specified a priori. Outcomes with a rating of 75% or more of the panel rating 7 to 9 and 25% or fewer rating 1 to 3 were included in the COS. RESULTS: A total of 31 core outcomes were identified from the Delphi process. This list was condensed to combine topic areas to produce a final COS of 10 outcomes, including both processes of care and patient-reported outcomes that should be considered for reporting in future studies of VPD. CONCLUSIONS: Implementation of the COS-VPD will facilitate consistency of outcomes data collection and comparison of results across studies.

Bleeding Severity and Phenotype in 22q11.2 Deletion Syndrome-A Cross-Sectional Investigation.

OBJECTIVES: To prospectively quantify bleeding severity and elaborate hemorrhagic symptoms in children with 22q11.2 deletion syndrome (22q11DS) using 2 validated bleeding assessment tools (BATs), namely the Pediatric Bleeding Questionnaire and the International Society on Thrombosis and Hemostasis BAT (ISTH-BAT). We also sought to compare subjects' bleeding scores to unaffected first-degree family members. STUDY DESIGN: Children with 22q11DS and unaffected first-degree family members were recruited for the study. Two validated BATs were administered by a pediatric hematologist. Additional clinical and laboratory data were abstracted from patient medical records. Standard descriptive and nonparametric statistical methods were used. RESULTS: In total, 29 eligible subjects and controls were assessed. Median age (range) of subjects and controls was 8 (5-17) years and 38 (9-56) years, respectively. In total, 17 of 29 subjects had a positive bleeding score on ISTH-BAT compared with 1 of 29 control patients (P < .0001). Median ISTH-BAT score in subjects was 3 (0-12), compared with 2 (0-6) in control patients (P = .022). Median Pediatric Bleeding Questionnaire score in subjects was 2 (-1 to 12). The most frequent bleeding symptoms reported in subjects with 22q11DS were epistaxis (69%) and bruising (52%). Eighteen subjects had been surgically challenged, and 6 were noted to have increased perioperative hemorrhage. CONCLUSIONS: Children with 22q11DS have increased bleeding scores compared with their first-degree unaffected relatives. The majority of the bleeding symptoms described were mucocutaneous.

Orofacial Manifestations of Stickler Syndrome: An Analysis of Speech Outcome and Facial Growth After Cleft Palate Repair.

PURPOSE: The purpose of this study was to characterize airway problems, speech outcomes, and facial growth in patients with Stickler syndrome undergoing cleft palate repair. METHODS: A retrospective, longitudinal study was performed at the Children's Hospital of Philadelphia on 25 patients with Stickler syndrome and 53 nonsyndromic patients with clefts of the secondary palate repaired between 1977 and 2000. Airway problems were characterized by the incidence of Pierre Robin Sequence (PRS) and the necessity for surgical airway management. Speech was analyzed using the Pittsburgh weighted values for speech symptoms associated with velopharyngeal incompetence (VPI). Longitudinal anthropometric measurements represented up to 12 years of longitudinal cephalofacial growth. RESULTS: Seventy-two percent of patients with Stickler syndrome were diagnosed with PRS, 55.6% of whom required surgical airway management. Conversely, 20.8% of nonsyndromic patients were diagnosed with PRS (P < 0.0001), 18% of whom required surgical intervention (P < 0.05). Speech outcomes were poorer in patients with Stickler syndrome with 40% demonstrating borderline VPI and 13.3% demonstrating VPI, compared with 21.8% and 9.1%, respectively, in the nonsyndromic group. Both groups exhibited significantly shallower upper and mid facial depths and wider upper facial breadths when compared with normal standards of facial growth. Although there was a tendency toward decreased facial depths in patients with Stickler syndrome relative to nonsyndromic patients, the differences were nonsignificant. CONCLUSIONS: Patients with Stickler syndrome show significant potential for early airway compromise and a poorer prognosis for speech outcome after cleft palate repair. Their cephalofacial growth does not differ significantly from that of nonsyndromic cleft palate patients.

Impact of Cranial Base Abnormalities on Cerebellar Volume and the Velopharynx in 22q11.2 Deletion Syndrome.

OBJECTIVE: The purpose of this study was to analyze the relationship between cranial base, cerebellar, craniofacial, and velopharyngeal (VP) variables in individuals with 22q11.2 deletion syndrome (22q11DS). METHODS: Thirteen typically developing healthy children and 13 age- and sex-matched individuals with 22q11DS completed a magnetic resonance imaging scan, which was used to examine craniofacial and VP variables. RESULTS: A statistically significant difference was noted in cerebellum volumes, F1,24 = 7.947, P = .010, posterior nasal spine to posterior pharyngeal wall (PNS-PPW), F1,24 = 4.878, P = .037, nasion-sella-basion (NSB) cranial base angles, F1,24 = 7.253, P = .013, and sella-basion-opisthion (SBO) cranial base angles, F1,24 = 9.134, P = .006, between children with 22q11DS and controls. The cerebellum volume was significantly reduced and cranial base angles were significantly more obtuse in individuals with 22q11DS. In the 22q11DS group, cerebellum volume was significantly correlated with sella-basion (SB) length, osseous pharyngeal depth, the PNS-PPW length, and velar length (P < .05). The PNS-PPW length was correlated with SB length, basion-opisthion length, NSB angle, SBO angle, and the VP ratio (P < .05). CONCLUSION: This study supports previous findings on anatomical differences among individuals with 22q11DS and has expanded our current understanding of the potential relationship between craniofacial and VP variables in at least a subset of children with 22q11DS. Results provide preliminary insights into the potential relationship between a decrease in cerebellar volume, obtuse cranial base angles, and unfavorable VP dimensions.

Impact of Interdisciplinary Team Care for Children With 22q11.2 Deletion Syndrome.

OBJECTIVE: To evaluate disease-specific guideline adherence among children with 22q11.2 deletion syndrome receiving multidisciplinary team care through a 22q specialty clinic compared to children not receiving team care. DESIGN: Retrospective chart review; quality improvement project. SETTING: Tertiary care pediatric hospital. PATIENTS: One hundred eighty-nine patients with 22q11.2 deletion syndrome were categorized into those receiving team care and those not receiving team care. Guideline adherence was compared between the 2 groups. MAIN OUTCOME MEASURE(S): Percent adherence across 8 disease-specific guidelines. RESULTS: A Welch t test revealed mean adherence among patients receiving team care was significantly higher (83% vs 42%, P < .001) compared those not receiving team care. Among team patients with a single 22q Center visit, a paired samples t test showed that mean adherence increased from 63% before the clinic encounter to 86% six months after the encounter (P < .001). Some guidelines were more likely to be associated with provider nonadherence, whereas others were more likely to be associated with patient nonadherence. CONCLUSIONS: Multidisciplinary team care is associated with significantly higher guideline adherence in children with 22q11DS. Additional research is needed to investigate the effect of team care on long-term health outcomes in children with 22q11DS.

Differences in the Tensor Veli Palatini Muscle and Hearing Status in Children With and Without 22q11.2 Deletion Syndrome.

PURPOSE: To investigate the dimensions of the tensor veli palatini (TVP) muscle using high image resolution 3-dimensional magnetic resonance imaging (MRI) of the soft palate among children with normal velopharyngeal and craniofacial anatomy and to compare values to individuals with a diagnosis of 22q11.2 deletion syndrome (22q11DS). We also sought to determine whether there is a relationship between hypoplasia of the TVP and severity of middle ear dysfunction and hearing loss. METHODS: Three-dimensional MRI were used to collect and analyze data obtained across 53 children between 4 and 12 years of age, including 40 children with normal velopharyngeal and craniofacial anatomy and 13 children with a diagnosis of 22q11.2 DS. Tensor veli palatini muscle length, thickness, and volume as well as bihamular distance were compared among participant groups. RESULTS: A Welch's t-test revealed that the TVP in participants with 22q11DS is significantly shorter (P = .005, 17.3 vs 19.0 mm), thinner (P < .001, 1.1 vs 1.8 mm), and less voluminous (P < .001, 457.5 vs 667.3 mm3) than participants without 22q11DS. Participants with 22q11DS also had a greater (P = .006, 27.7 vs 24.7 mm) bihamular distance than participants without 22q11DS. There was an inverse relationship between TVP abnormalities noted above and the severity of audiologic and otologic histories. CONCLUSION: The TVP muscle is substantially reduced in volume, length, and thickness in children with 22q11DS. These findings serve as preliminary support for the association of patient hearing and otologic severity and TVP dysmorphology.

Velopharyngeal Structural and Muscle Variations in Children With 22q11.2 Deletion Syndrome: An Unsedated MRI Study.

OBJECTIVE: The 22q11.2 deletion syndrome (22q11.2DS) is the most common genetic cause of velopharyngeal dysfunction; however, limited information exists regarding variations in velopharyngeal anatomy in this clinically challenging population. The purpose of this study was to examine velopharyngeal characteristics among young children with 22q11.2DS in comparison to a normative cohort using an innovative, nonsedated magnetic resonance imaging (MRI) scanning protocol. METHODS: Fifteen children with 22q11.2DS and 15 age- and gender-matched controls with normal velopharyngeal anatomy (ages 4-12) successfully completed the MRI protocol. Eighteen velopharyngeal and 2 related craniofacial measures were examined. Analysis of covariance was used to compare differences between the experimental and the control groups. RESULTS: The 22q11.2DS group demonstrated a significantly thinner velum (P < .0005) and a larger pharyngeal depth (P = .007) compared to the matched control group. Findings in the current study also demonstrated that the levator veli palatini muscle is significantly shorter (P = .037) and thinner (P = .025) in the 22q11.2DS cohort, with a significantly shorter origin-to-origin distance (P < .0005) and a greater angle of origin (P = .001) compared to healthy peers. CONCLUSION: Children with 22q11.2DS demonstrated multiple variations that may contribute to velopharyngeal dysfunction by altering the anatomic characteristics of the velopharyngeal port, the levator muscle, and associated structures. This investigation represents the first and largest attempt to characterize velopharyngeal anatomy in children with 22q11.2DS using a nonsedated MRI protocol.

Assessment of Psychosocial Risk in Families of Children With Craniofacial Conditions Using the Psychosocial Assessment Tool-Craniofacial Version.

OBJECTIVES: Using the Psychosocial Assessment Tool-Craniofacial Version (PAT-CV), this study measured variation in psychosocial risk in families of children with craniofacial conditions by demographic and clinical characteristics, frequency of condition-related problems, concordance between caregivers' report of risk, and association between risk level and psychosocial service utilization. DESIGN: Caregivers (n = 242) of 217 children with craniofacial conditions completed the PAT-CV, a psychosocial risk screener. Medical records were also abstracted. RESULTS: The PAT-CV scores varied significantly by insurance type and syndromic versus nonsyndromic diagnosis type. Language problems were most commonly reported on the Craniofacial Problems subscale, though 13% to 56% of parents endorsed specific problems. Mothers and fathers reported similar risk levels. Families with past social work or behavioral health consults had higher PAT-CV scores than those without consultations. CONCLUSION: The PAT-CV efficiently screens for psychosocial risk and craniofacial-specific problems. This tool may help clinicians identify families in need of intervention.

Surgical Care for School-Aged Youth With Cleft: Results From a Multicenter, Prospective Observational Study.

OBJECTIVE: To explore factors related to completion of surgery recommendations among children with cleft lip and palate (CLP) or cleft palate only (CPO). DESIGN: Multicenter prospective longitudinal cohort study (2009-2015). SETTING: Six cleft centers in the United States. PATIENTS/PARTICIPANTS: A diverse sample of 1186 youth aged 7.5 to 18.5 years with CLP or CPO and a caregiver. RESULTS: Data were collected from child-caregiver dyads at baseline and up to 3 follow-up visits. Of the 765 surgeries recommended during the study period, 83 were postponed and 597 were completed; this represents a completion rate of 78%. There were multiple reasons identified by patients for why the remaining 85 recommended surgeries were not completed. Children with nonprivate insurance were more likely to postpone recommended surgeries. Hispanic/Latino and Asian children had fewer surgeries compared to whites/Caucasians. Greater severity in rated speech differences was associated with more surgeries. Among participants who had completed all recommended surgeries, providers rated over 86% as having normal or mildly different facial appearance and 83% as having a normal or mild speech intelligibility rating. Similarly, caregivers rated 80% of facial appearance and 78% of speech positively. Approximately 25% of children had an additional surgical recommendation at the end of the study. CONCLUSIONS: The majority of surgical recommendations were completed with positive outcomes in facial appearance and speech intelligibility by provider and caregiver ratings. Several factors contributed to surgical recommendations not being completed and the results underscore the importance of examining socioeconomic and demographic disparities in surgical care.