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.

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.

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.

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.

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.

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.

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 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.

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.

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.

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.

Identifying Psychosocial Risk Factors Among Families of Children With Craniofacial Conditions: Validation of the Psychosocial Assessment Tool-Craniofacial Version.

OBJECTIVE: To evaluate the psychometric properties of the Psychosocial Assessment Tool-Craniofacial Version (PAT-CV), a screening instrument for psychosocial risk in families of children with craniofacial conditions, and to examine risk classification of patients in a craniofacial population. DESIGN: Prospective, cross-sectional, single-center study. SETTING: Interdisciplinary cleft lip and palate/craniofacial center at a US children's hospital. PARTICIPANTS: Parents/caregivers (n = 242) of 217 children ages 1 month to 17 years being treated for a congenital syndromic or nonsyndromic craniofacial condition completed the PAT-CV and validating measures from July 2015 to July 2016. The PAT-CV was completed by 121 caregivers a second time to assess test-retest reliability. MAIN OUTCOME MEASURES: PAT-CV, Child Behavior Checklist, Adult Self-Report, Pediatric Quality of Life Inventory, Craniofacial Experiences Questionnaire, and Family Environment Scale. RESULTS: Construct validity of the PAT-CV was supported by significant correlations ( P < .001) between PAT-CV total and subscale scores and the validated measures. PAT-CV total scores categorized 59.9% of families within the universal risk group, 32.3% within the targeted risk group, and 7.8% within the clinical range. Good criterion validity was indicated by significantly higher scores ( P < .0001) obtained on the validated measures for those in the targeted and clinical risk groups. Internal consistency (Cronbach's α = 0.86) and test-retest reliability for the PAT-CV total score ( r = 0.77, P < .0001) were acceptable. CONCLUSIONS: The PAT-CV appears to be a reliable and valid screening instrument for psychosocial risk. Accurate identification of risk and implementation of appropriate interventions may contribute to improvements in medical and psychosocial outcomes.

A Quality Improvement Initiative to Improve Feeding and Growth of Infants With Cleft Lip and/or Palate.

OBJECTIVE: The purpose of this quality improvement initiative was to improve feeding and growth outcomes in infants with cleft lip and/or palate (CL/P). DESIGN: Institute for Healthcare Improvement quality improvement model. SETTING: Large pediatric academic medical center in the Midwestern United States. PARTICIPANTS: One hundred forty-five infants with nonsyndromic CL/P ages 0 to 12 months. INTERVENTIONS: Key drivers included (1) caregiver education and resources, (2) care coordination and flow, and (3) provider education and training. Interventions were designed around these themes and included targeting improved team communication, increased social work consultations, patient tracking, staff education, improved access to feeding equipment, and the launch of a new cleft palate feeding team. MAIN OUTCOME MEASURE(S): The primary outcome measure was the percentage of new patients with CL/P who met criteria for failure to thrive (FTT) per month. The secondary outcome measure was the frequency of hospitalization for infants with CL/P with a primary reason for admission of feeding difficulties or FTT. RESULTS: The institutional FTT rate for infants with CL/P decreased from 17% to 7% ( P < .003). The frequency of hospitalization for FTT improved from once every 30 days to once every 118 days. CONCLUSIONS: Targeted interventions aimed at improving feeding efficiency and effectiveness, as well as changes in care delivery models, can reliably promote improvements in feeding and growth outcomes for infants with CL/P, even with psychosocial risk factors present.

Simonart's Band: Its Effect on Cleft Classification and Recommendations for Standardized Nomenclature.

OBJECTIVE: Accurate classification of cleft lip plays an important role in communication, treatment planning, and comparison of outcomes across centers. Although there is reasonable consensus in defining cleft types, the presence of Simonart's band can make classification challenging. Our objective was to survey cleft care providers to determine what all consider to be Simonart's band, how its presence effects cleft lip classification, and to provide recommendations for standardized nomenclature. DESIGN: A multiple-choice survey was e-mailed to 1815 members of the American Cleft Palate-Craniofacial Association, assessing each respondent's definition of Simonart's band and its effect on cleft classification. Cleft classification was drawn from the ICD system diagnosis billing codes. Descriptive analysis was performed. RESULTS: Three hundred seventy-three providers completed the survey (20.5% response), the majority of whom were surgeons (61.5%); 87.1% agreed with the definition that a Simonart's band is "any soft tissue bridge located at the base of the nostril or more internally, between the segmented ridges." However, only 41.8% felt that the presence of a Simonart's band rendered a cleft lip incomplete; 54.4% felt that an alveolar cleft was the defining difference between a complete and an incomplete cleft lip. When asked to define the child with a cleft involving the upper lip that extends into the naris but interrupted by a soft tissue bridge located only at the base of the nostril or more internally, without a cleft of the alveolar ridge and palate, 61.4% classified this as an incomplete cleft lip, 32.7% as a complete cleft lip, and 5.9% as an unspecified cleft lip. CONCLUSIONS: Responses revealed wide discrepancy in the classification of cleft phenotypes and in the interpretation of the significance of anatomical components in the classification of a cleft lip. We discuss the difficulty in aligning classification based on unclear definition of terms and variable anatomic parameters. We highlight this issue in the face of a need for comparability in clinical evidence-based practices. To ensure precision and uniformity in cleft classification, we recommend that use of the term "Simonart's band" be abandoned while incorporating a notation of the integrity of the nasal sill into the LAHSHAL system. We propose a uniform definition of incomplete versus complete cleft lip, wherein a cleft lip will be classified as complete in the presence or absence of narrow bands of tissue present at the base of the nasal sill or more internally.

Intra-amniotic transient transduction of the periderm with a viral vector encoding TGFß3 prevents cleft palate in Tgfß3(-/-) mouse embryos.

Cleft palate is a developmental defect resulting from the failure of embryonic palatal shelves to fuse with each other at a critical time. Immediately before and during palatal fusion (E13-E15 in mice), transforming growth factor ß3 (TGFß3) is expressed in the palatal shelf medial edge epithelium (MEE) and plays a pivotal role in palatal fusion. Using Tgfß3(-/-) mice, which display complete penetrance of the cleft palate phenotype, we tested the hypothesis that intra-amniotic gene transfer could be used to prevent cleft palate formation by restoring palatal midline epithelial function. An adenoviral vector encoding Tgfß3 was microinjected into the amniotic sacs of mouse embryos at successive developmental stages. Transduced Tgfß3(-/-) fetuses showed efficient recovery of palatal fusion with mesenchymal confluence following injection at E12.5 (100%), E13.5 (100%), E14.5 (82%), and E15.5 (75%). Viral vectors injected into the amniotic sac transduced the most superficial and transient peridermal cell layer but not underlying basal epithelial cells. TGFß3 transduction of the peridermdal cell layer was sufficient to induce adhesion, fusion, and disappearance of the palatal shelf MEE in a cell nonautonomous manner. We propose that intra-amniotic gene transfer approaches have therapeutic potential to prevent cleft palate in utero, especially those resulting from palatal midline epithelial dysfunction.