Salivary gland developmental mechanics.

The salivary gland undergoes branching morphogenesis to elaborate into a tree-like structure with numerous saliva-secreting acinar units, all joined by a hierarchical ductal system. The expansive epithelial surface generated by branching morphogenesis serves as the structural basis for the efficient production and delivery of saliva. Here, we elucidate the process of salivary gland morphogenesis, emphasizing the role of mechanics. Structurally, the developing salivary gland is characterized by a stratified epithelium tightly encased by the basement membrane, which is in turn surrounded by a mesenchyme consisting of a dense network of interstitial matrix and mesenchymal cells. Diverse cell types and extracellular matrices bestow this developing organ with organized, yet spatially varied mechanical properties. For instance, the surface epithelial sheet of the bud is highly fluidic due to its high cell motility and weak cell-cell adhesion, rendering it highly pliable. In contrast, the inner core of the bud is more rigid, characterized by reduced cell motility and strong cell-cell adhesion, which likely provide structural support for the tissue. The interactions between the surface epithelial sheet and the inner core give rise to budding morphogenesis. Furthermore, the basement membrane and the mesenchyme offer mechanical constraints that could play a pivotal role in determining the higher-order architecture of a fully mature salivary gland.

Treatment Outcomes of Lip Taping in Patients with Non-syndromic Cleft Lip and/or Palate: A Systematic Review and Meta-analysis.

OBJECTIVE: To conduct a systematic review of the data in peer-reviewed medical literature and evaluate the effectiveness of lip taping as a pre-surgical naso-alveolar molding (NAM) technique in infants with cleft lip and/or palate. DESIGN: An electronic search of various databases for relevant studies, regardless of date, from inception to June 2023 was carried out and evaluated. After completing the electronic search and applying our inclusion/exclusion criteria, 6 studies-2 randomized control trials, 2 non-randomized studies, and 2 case series-were included. Data extraction of relevant articles was done independently by 2 authors. Quality assessment was done using the JBI prevalence critical appraisal tool and certainty of evidence was carried out by GRADE approach. MAIN OUTCOME MEASURES: Nasolabial Aesthetics, Dentoalveolar Relationship. RESULTS: A total of six studies were included in the current review. Meta-analysis was carried out, and forest plots were obtained for a single mean from the lip-taping group. 3 studies had a low risk of bias, while 3 studies displayed a serious risk of bias. Significant improvement in various outcome measures was noted with lip taping when compared with the control group although the certainty of evidence was very low. CONCLUSION: When compared to no therapy, lip taping appears to ameliorate dentoalveolar measurements and nasolabial aesthetics. To increase our knowledge of lip taping, more research will be needed in the future, as there are not many studies to prove lip taping is better than other treatment approaches.

A Longitudinal Investigation of Nasolabial Changes With and Without Revision Surgery in Patients with Non-Syndromic Unilateral Cleft Lip and Palate.

OBJECTIVE: To determine a baseline of anticipated change in nasolabial appearance following primary repair of unilateral cleft lip/palate and evaluate the degree to which revision surgery improves nasolabial appearance. DESIGN: Retrospective chart review. SETTING: Patients treated at the Lancaster Cleft Palate Clinic interdisciplinary clinic. PATIENTS: Twenty-three patients with complete unilateral cleft lip and palate who underwent primary surgical repair and 19 additional patients who underwent subsequent revision surgery were included. INTERVENTIONS: Patients in the non-revision group underwent a Tennison-Randall triangular flap lip repair at 3mo. Patients in the revision group underwent a modification of the Nakajima straight-line repair after primary Tennison-Randall triangular flap lip repair at an average age of 141mo. MAIN OUTCOME MEASURES: A modification of the Asher-McDade Aesthetic Index was utilized to evaluate Nasolabial Frontal (NLF), Nasolabial Profile (NLP), Vermillion Border (VB), and total change in appearance. Scores for patients in the revision group were evaluated before and after revision while appearance for patients without revision was evaluated at 3 distinct ages. Scores were averaged across time-points and inter-rater reliability was assessed. RESULTS: Nasolabial appearance in the non-revision sample did not change significantly over time, except for nasal profile. Scores improved after revision surgery - NLP: 3.48 to 2.97, (p = 0.001); NLF: 3.50 to 2.95 (p = 0.001); and Total Nasolabial Score: 3.29 to 3.01 (p = 0.004), with no significant change in VB. CONCLUSION: Decisions regarding need for nasolabial revision surgery may be made as early as 5yo with successful outcomes following secondary surgery improving appearance except for vermillion border appearance.

Lacrimal Drainage Anomalies in Goldenhar, Rubinstein-Taybi, and Ectodermal-Ectrodactyly-Clefting Syndromes.

OBJECTIVE: To describe in detail the lacrimal drainage system anomalies and review of literature in patients with Goldenhar syndrome, Rubinstein-Taybi syndrome (RTS), and Ectodermal-Ectrodactyly-Clefting syndrome (EECS), their management and outcomes. METHODS: A retrospective chart review from January 2011-June 2023 of all cases presenting to the Dacryology clinic with Goldenhar syndrome, RTS, and EECS was obtained. Data collected included demographics, laterality, clinical presentations, proximal and distal lacrimal drainage anomalies, associated systemic features, management, and outcomes. RESULTS: Eight children with Goldenhar syndrome (n = 13), three with RTS (n = 5) and three with EECS (n = 5) presented with lacrimal drainage system involvement. Cases with Goldenhar syndrome showed male predominance (5/8), and the mean age at presentation was 14.75 months. Four cases had simple CNLDO, seven cases with complex CNLDO (4 - buried probe and 3 - atonic sacs) and a single neonate presented with bilateral dacryocele. Patients with RTS presented with mean age of 36.33 months with male predominance. Probing under endoscopic guidance explored the anatomy thoroughly and those with altered nasal anatomy increased the probability of complex CNLDO. Those with EECS (n = 5) presented with a greater involvement of proximal lacrimal drainage system compared with Goldenhar syndrome and RTS, including anomalies like punctal agenesis, incomplete punctal canalization (IPC), ectopic puncta, canalicular stenosis, and complex CNLDO. CONCLUSIONS: A step-wise approach to assessing the proximal and lacrimal drainage system in those affected with craniofacial malformations and addressing them can result in satisfactory outcomes for the majority of patients.

Contemporary Prevalence of Oral Clefts in the US: Geographic and Socioeconomic Considerations.

Background: Socio-economic status, living environments, and race have been implicated in the development of different congenital abnormalities. As orofacial clefting is the most common anomaly affecting the face, an understanding of its prevalence in the United States and its relationship with different determinants of health is paramount. Therefore, the purpose of this study is to determine the modern prevalence of oral-facial clefting in the United States and its association with different social determinants of health. Methods: Utilizing Epic Cosmos, data from approximately 180 US institutions were queried. Patients born between November 2012 and November 2022 were included. Eight orofacial clefting (OC) cohorts were identified. The Social Vulnerability Index (SVI) was used to assess social determinants of health. Results: Of the 15,697,366 patients identified, 31,216 were diagnosed with OC, resulting in a prevalence of 19.9 (95% CI: 19.7-20.1) per 10,000 live births. OC prevalence was highest among Asian (27.5 CI: 26.2-28.8) and Native American (32.8 CI: 30.4-35.2) patients and lowest among Black patients (12.96 CI: 12.5-13.4). Male and Hispanic patients exhibited higher OC prevalence than female and non-Hispanic patients. No significant differences were found among metropolitan (20.23/10,000), micropolitan (20.18/10,000), and rural populations (20.02/10,000). SVI data demonstrated that OC prevalence was positively associated with the percentage of the population below the poverty line and negatively associated with the proportion of minority language speakers. Conclusions: This study examined the largest US cohort of OC patients to date to define contemporary US prevalence, reporting a marginally higher rate than previous estimates. Multiple social determinants of health were found to be associated with OC prevalence, underscoring the importance of holistic prenatal care. These data may inform clinicians about screening and counseling of expectant families based on socio-economic factors and direct future research as it identifies potential risk factors and provides prevalence data, both of which are useful in addressing common questions related to screening and counseling.

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.

Correlation between Cleft Width and Adjacent Teeth Inclination in Patients with Unilateral Cleft Lip and Palate Using CBCT: A Retrospective Study.

OBJECTIVES: To evaluate buccolingual and mesiodistal inclination of cleft-adjacent maxillary permanent central incisors (U1) and canines (U3) in patients with unilateral cleft lip and palate (UCLP) in the late mixed dentition and to investigate the correlation between the alveolar cleft width and cleft-adjacent teeth inclination using cone beam computed tomography (CBCT). DESIGN: Observational cross-sectional retrospective study. SETTING: Department of Orthodontics, Hama University Dental School. PATIENTS: 32 patients with UCLP (22 boys, 10 girls; mean age = 10.53 ± 1.51 years). MAIN OUTCOME MEASURE: Buccolingual and mesiodistal inclination of maxillary central incisors and canines were measured on both sides. Then, the cleft and non-cleft sides were compared using the Wilcoxon signed-rank test. Pearson's correlation was used to explore the association between the alveolar cleft width and cleft-adjacent U1 and U3 buccolingual and mesiodistal inclination. RESULTS: The cleft-adjacent central incisors were significantly inclined lingually and distally compared with their non-cleft antimeres (P = .003, P < .001, respectively). The cleft-adjacent canines were significantly inclined buccally and mesially compared with their non-cleft antimeres (P < .001, for both). A positive correlation was found between the buccolingual inclination of cleft-adjacent U1 and the alveolar cleft width (r = 0.49, P = .004). CONCLUSIONS: Patients with UCLP demonstrated a significant lingual and distal inclination of cleft-adjacent U1 and a significant buccal and mesial inclination of cleft-adjacent U3. The buccolingual inclination of cleft-adjacent U1 tends to increase with increasing the alveolar cleft width; however, the correlation was weak.

Three-dimensional Analysis of Maxillary Morphology in Infants with Unilateral Cleft Lip and Palate.

OBJECTIVE: To three-dimensionally (3D) analyze the maxillary morphology of infants with unilateral cleft lip and palate (UCLP) and preliminarily classify the alveolar arch to assist in personalization of sequence therapy. DESIGN: Retrospective study. SETTING: Patients with UCLP referred to outpatients' clinic. PARTICIPANTS: 84 nonsyndromic infants with complete UCLP were recruited (58 boys, 26 girls, mean age 29.48 days). MAIN OUTCOME MEASURE: Morphometric analysis was conducted on 3D maxillary models. Principal component analysis (PCA) and cluster analysis were combined to classify maxillary phenotypes preliminarily. The Wilcoxon Signed Rank test and the Kruskal-Wallis test were used to compare differences between variables. A P value less than .05 was considered statistically significant. RESULTS: The maxilla was divided into three types: narrow, homogenous and broad, accounting for 9.52%, 23.81% and 66.67% respectively. The alveolar cleft site (median value) was located in 61% of the total length of the alveolar arch. In the comparison of anterior and total alveolar lengths, the non-cleft side had longer alveolar bone than the affected side, a difference of approximately 2 mm. Pairwise comparisons of variables describing alveolar symmetry revealed significant differences in all subjects; whereas type C had poorer arch symmetry than types A and B, mainly in terms of anterior and overall symmetry. CONCLUSIONS: In infants with UCLP, the maxillary alveolar arch was inherently asymmetrical with partially bone missing (about 2 mm). Significant differences in alveolar bone morphology and symmetry exist between different types of infants, with individuals with broad clefts (type C, the largest proportion) having the worst maxillary development.

Genetic Associations of Oral Clefts in Arabs.

OBJECTIVE: This study aims to investigate genetic association between Non-syndromic Cleft lip with or without palate (NCLP) and 14 specific Single Nucleotide Polymorphism (SNPs) reported to be associated with NCLP from previous Genome Wide Association Studies (GWAS). DESIGN: A prospective case-control study. SETTING: Ministry of Health (MOH) Cleft and Craniofacial Clinic and Kuwait University. PATIENTS/PARTICIPANTS: One hundred sixty-four NCLP patients were recruited from MOH Cleft and Craniofacial clinic, and 491 controls from the Kuwait DNA bank established at Kuwait University. INTERVENTIONS: Total gDNA was extracted from whole blood withdrawn from patients and genotyped by real time PCR. Hardy-Weinberg Equilibrium was tested, and the set p value for significance (p < 0.05) was adjusted using the Benjamini - Hoochberg procedure to achieve 5% false discovery rate. MAIN OUTCOME MEASURES: Logistic regression multivariate analysis was used to test statistically significant differences between cases and controls. Genotyping both groups for the variants was determined through the allele discrimination software program. RESULTS: There was statistically significant difference between cases and controls in relation to two SNPs; LOC102724968 (rs13041247) (MAF cases/control = C (0.28/0.39) OR Homozygous = 1.30; 95% CI = 1.09-1.56, p = 0.0041) and PVT1 (rs987525) (MAF cases/control = A (0.41/0.27) OR heterozygous = 1.48; 95% CI =1.12-1.95, p = 0.0073), increasing the susceptibility to NCLP. CONCLUSIONS: Genetic variations are associated with the occurrence of oral clefts. Customized Next Generation Sequencing (NGS) panel to the Arab ethnicity is encouraged. In Addition, national preconception genetic carrier screening tests should expand to include common craniofacial anomalies.

Evaluation of Anxiety in Turkish Parents of Newborns with Cleft Palate with or Without Cleft Lip.

OBJECTIVE: (1) To compare anxiety between parents of newborns with cleft lip and palate (CLP), isolated cleft palate (CP), and healthy newborns and (2) to evaluate anxiety between parental dyads within these groups. DESIGN: A cross-sectional study. SETTING: University Hospital. PARTICIPANTS: Surveys were completed by 20 mothers and 20 fathers of newborns with CLP, 21 mothers and 21 fathers of newborns with CP, and 23 mothers and 23 fathers of healthy newborns (controls). MAIN OUTCOME MEASURE: The State-Trait Anxiety Inventory (STAI) assessed parental anxiety. Mothers of newborns with a cleft reported on concerns regarding cleft-related issues and facial appearance. RESULTS: State and trait anxiety were generally in the moderate range for parents of newborns with a cleft, while control parents had low state anxiety and moderate trait anxiety. Mothers of newborns with CP and CLP had significantly higher state and trait anxiety levels than control mothers (p < .05). Fathers of newborns with CLP had a higher state anxiety level than control fathers. When maternal and paternal anxiety was compared within the groups, only trait anxiety scores were significantly higher in mothers of newborns with CLP than that of fathers (p < .05). More than half of mothers of newborns with a cleft were concerned about their newborn's feeding, speech, and palate. CONCLUSIONS: Parents of children with a cleft may need psychological support in the early postnatal period. It is important for neonatal cleft team providers to help reduce parental anxiety and educate families about cleft care, with a focus on feeding.

Predictors of Adverse Outcomes Following Cleft Palate Repair: An Analysis of Over 2500 Patients Using International Smile Train Data.

OBJECTIVE: The objective of this study was to use data from Smile Train's global partner hospital network to identify patient characteristics that increase odds of fistula and postoperative speech outcomes. DESIGN: Multi-institution, retrospective review of Smile Train Express database. SETTING: 1110 Smile Train partner hospitals. PATIENTS/PARTICIPANTS: 2560 patients. INTERVENTIONS: N/A. MAIN OUTCOME MEASURE(S): Fistula occurrence, nasal emission, audible nasal emission with amplification (through a straw or tube) only, nasal rustle/turbulence, consistent nasal emission, consistent nasal emission due to velopharyngeal dysfunction, rating of resonance, rating of intelligibility, recommendation for further velopharyngeal dysfunction assessment, and follow-up velopharyngeal dysfunction surgery. RESULTS: The patients were 46.6% female and 27.5% underweight by WHO standards. Average age at palatoplasty was 24.7 ± 0.5 months and at speech assessment was 6.8 ± 0.1 years. Underweight patients had higher incidence of hypernasality and decreased speech intelligibility. Palatoplasty when under 6 months or over 18 months of age had higher rates of affected nasality, intelligibility, and fistula formation. The same findings were seen in Central/South American and African patients, in addition to increased velopharyngeal dysfunction and fistula surgery compared to Asian patients. Palatoplasty technique primarily involved one-stage midline repair. CONCLUSIONS: Age and nutrition status were significant predictors of speech outcomes and fistula occurrence following palatoplasty. Outcomes were also significantly impacted by location, demonstrating the need to cultivate longitudinal initiatives to reduce regional disparities. These results underscore the importance of Smile Train's continual expansion of accessible surgical intervention, nutritional support, and speech-language care.

Orofacial Deformities in 3 Related Rhesus Monkeys (Macaca mulatta) Resembling Human Binder's Syndrome.

Binder's syndrome is a rare congenital deformity characterized by midface hypoplasia, particularly around the nasomaxillary area. Genetic etiology or developmental failure caused by prenatal exposure to teratological agents has been considered. In this article, we present 3 related rhesus monkeys born with orofacial deformities similar to those found in infants with the Binder phenotype. For the first time, a primate biomodel for this condition is presented. The clinical description and association with management and environmental factors are discussed. These findings reinforce the knowledge about the relationship between possible vitamin K metabolism interference and Binder's syndrome.

Defining the Treatment Gap in Nasoalveolar Molding: Factors Affecting the Utilization of NAM in an Urban Cleft Center.

BACKGROUND: Many cleft centers incorporate NasoAlveolar Molding (NAM) into their presurgical treatment protocols. However, there are limited data on eligible patients who do not receive or complete NAM. This study characterizes the demographics associated with non-utilization or completion of NAM. METHODS: A single-institution retrospective review was performed of all patients with cleft lip and alveolus undergoing primary unilateral and bilateral cleft lip repair from 2012-2020. Patients were grouped based on utilization or non-utilization of NAM. Demographic and treatment data were collected, including documented reasons for not pursuing or completing NAM. RESULTS: Of 230 eligible patients, 61 patients (27%) did not undergo or complete NAM (no-NAM). In this group, 37 (60.7%) received no presurgical intervention, 12 (19.7%) received presurgical nostril retainers, 3 (4.9%) received lip taping, 1 (1.6%) received a combination of taping/nostril retainers, and 8 (13.1%) discontinued NAM. The most common reasons for not receiving NAM were sufficiently aligned cleft alveolus (21.3%), medical complexity (16.4%), late presentation (16.4%), and alveolar notching (18%). Compared to the NAM group, the no-NAM group had significantly lower rates of prenatal cleft diagnosis/consult, and significantly higher proportion of non-married and non-English speaking caregivers. Multivariable analysis controlling for insurance type, primary language, prenatal consult, marital status, and age at first appointment found that age at first appointment is the only statistically significant predictor of NAM utilization (P < .001). CONCLUSIONS: Common reasons for non-utilization of NAM include well-aligned cleft alveolus, medical complexity, and late presentation. Early presentation is an important modifiable factor affecting rates of NAM utilization.

Sagittal Growth Restriction of the Midface Following Isolated Cleft Lip Repair: A Systematic Review and Meta-Analysis.

Midface hypoplasia (MFH) is a long-term sequela of cleft lip and palate repair, and is poorly understood. No study has examined the aggregate data on sagittal growth restriction of the midface following repair of the lip, but not palate, in these patients.A systematic review of 3780 articles was performed. Twenty-four studies met inclusion criteria and 11 reported cephalometric measurements amenable to meta-analysis. Patients with Veau class I-III palatal clefts were included so long as they had undergone only lip repair. Groups were compared against both noncleft and unrepaired controls.Cephalometrics were reported for 326 patients (31.3% female). Noncleft controls had an average SNA angle of 81.25° ± 3.12°. The only patients demonstrating hypoplastic SNA angles were those with unilateral CLP with isolated lip repair (77.4° ± 4.22°). Patients with repaired CL had SNA angles similar to noncleft controls (81.4° ± 4.02°). Patients with unrepaired CLP and CL tended toward more protruding maxillae, with SNA angles of 83.3° ± 4.04° and 87.9° ± 3.11°, respectively. Notably, when comparing SNA angles between groups, patients with CLP with isolated lip repair had significantly more hypoplastic angles compared to those with repaired CL (P < .0001). Patients with CLP with isolated lip repair were also more hypoplastic than noncleft controls (P < .0001). In contrast, there was no significant difference between the SNA of patients with repaired CL and controls (P = .648).We found that cleft lip repair only appeared to contribute to MFH in the setting of concurrent cleft palate pathology, suggesting that scarring from lip repair itself is unlikely to be the predominant driver of MFH development. However, studies generally suffered from inadequate reporting of timing, technique, follow-up time, and cleft severity.

Screening for Academic Risk Among Students With Cleft Lip and/or Palate: Patterns of Risk and Qualities of Effective Tools.

This study evaluated the effectiveness of academic screening measures in relation to parent-reported diagnoses.Multicenter, retrospective cohort study including structured interviews, questionnaires, and chart reviews.Six North American cleft centers.Children (n = 391) with cleft lip and/or palate, ages 8 to 10 years of age (192 male) and their guardians were recruited during regular clinic visits.Parent and child ratings on the Pediatric Quality of Life Inventory (PedsQL) School Scale, child report on CleftQ School Scale, parent report on the Adaptive Behavior Assessment System-Third Edition Functional Academics (ABAS-FA) Scale and Child Behavior Checklist (CBCL) School Competency Scale, parent interview, and medical chart review.Risk for concerns ranged from 12% to 41%, with higher risk reflected on the CBCL-SC compared to other measures. Males with cleft palate were consistently at the highest risk. Only 9% of the sample had a parent-reported diagnosis of a learning or language disability. Ratings from the ABAS-FA and CBCL-SC had the highest utility in identifying those with language and/or learning concerns.As cleft teams work to develop standardized batteries for screening and monitoring of patients, it is important to evaluate the effectiveness of measures in identifying those at highest risk. When screening for language and learning disorders, questions related to potential academic struggles, such as increased school effort or increased school distress, are most useful. Referrals for follow-up evaluation are recommended for those identified at high risk.

Ocular Manifestations in Patients Affected by p63-Associated Disorders: Ectrodactyly-Ectodermal Dysplasia-Clefting (EEC) and Ankyloblepharon-Ectodermal Defects-Cleft Lip Palate (AEC) Syndromes.

BACKGROUND/AIMS: The Ectrodactyly-Ectodermal dysplasia-Clefting (EEC) and Ankyloblepharon-ectodermal defect-cleft lip/palate (AEC) syndromes are rare autosomal dominant diseases caused by heterozygous mutations in the p63 gene. Patients are characterized by abnormalities of the skin, teeth, and hair and have limb defects, orofacial clefting and ectodermal dysplasia. In addition, they often show ocular surface alterations, leading to progressive corneal clouding and eventually blindness. Here, we present 8 cases describing patients affected by EEC (n = 6, with 5 sporadic and 1 familial cases) and AEC (n = 2, both sporadic cases) syndromes. We attempt to provide a description of the ocular disease progression over the years. METHODS: Clinical examinations and monitoring of ocular parameters for the assessment of limbal stem cell deficiency were constantly performed on patients between 2009 and 2023. Quantitative data and comparison with existing cases described in the literature are reported. RESULTS: The therapies supplied to patients were essential for the management of the symptoms, but unfortunately did not halt the progression of the pathology. CONCLUSIONS: A constant monitoring of the patients would help avoid the sudden worsening of symptoms. If the progression of the disease slows down, it would allow for the development of newer therapeutic strategies aimed at correcting the genetic defect.

Dynamic Molding of Cleft Nasal Cartilage in Unilateral Complete Cleft Lip and Palate Using Simple Segmented NAM.

Objective: Nasal morphology remodeling by nasoalveolar molding (NAM) before primary lip repair gives better post-surgery outcomes. However, traditional NAM has high technical sensitivity, a steep learning curve, frequent need for modifications, and interrupts lifting forces on the nasal cartilage during oral movements, thereby reducing overall efficacy of device. To eliminate interaction and interdependency of the nasal bulb with alveolar molding plate, we propose segmenting NAM (SNAM) into nasolabial complex molding (NCM) and alveolar complex molding (ACM). NCM addresses ala-septum, lip-premaxilla via lambda-nasal hook and lip taping. ACM addresses palate-alveolus via Yen-modified feeding plate. When the hook is suspended by elastic traction, nasal complex undergoes a natural rotation, where cleft-side ala lifts and septum medializes. Lip taping and feeding plate augment positive molding of cleft segments and maintain transverse relation. The current study outlines SNAM treatment strategy and assesses the effect on nasal deformity. Design and setting: Retrospective observational cohort study set in a comprehensive cleft care centre. Patients: Twenty patients with unilateral complete cleft lip and palate who underwent SNAM were selected. Main outcome measures: Retrospectively, standardized basilar views, pre-, and post-SNAM therapy were evaluated for nostril height, and nostril width, as a ratio of cleft to non-cleft side along with columellar deviation angle. Results: SNAM resulted in significantly improved nostril symmetry with no complications observed. Nostril height ratio increased from 0.35 ± 0.10 to 0.78 ± 0.17. Nostril width ratio decreased from 3.14 ± 0.66 to 2.18 ± 0.42. Columellar deviation angle increased from 26.5 ± 6.30 to 58.5 ± 9.88 degrees. Conclusions: Dynamic correction of the nasal deformity in UCLP is possible through SNAM therapy.

Median mandibular cleft in SAMS syndrome - A rare case report.

Rationale: SAMS syndrome is a rare genetic disorder characterized by midline facial clefting, skeletal anomalies, and other defects. Salient Features: Among the craniofacial manifestations of SAMS syndrome is the presence of a median mandibular cleft (MMC). MMC is a rare occurrence and in this syndrome, it poses a complex challenge for both functional and aesthetic reasons. Patient. Findings: This rare case report describes the successful correction of an MMC in an 18-month-old child diagnosed with SAMS syndrome. Treatment: This report describes the presentation, diagnosis and treatment. The surgical intervention involved a meticulous, single stage, osseous reconstruction. The mechanism of MMC in SAMS syndrome is discussed. Outcomes: Early intervention for MMC in SAMS syndrome patients can offer promising outcomes.

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

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

Knowledge assessment on cleft lip and palate among recently graduated dentists: a cross-sectional study.

BACKGROUND: The complex presentation, associated co-morbidities and multi-disciplinary requirements dictate the requirement for in-depth knowledge in order to effectively manage patients with cleft lip and palate (CLP). We aimed to develop a validated questionnaire for cleft lip and palate knowledge assessment and to evaluate the knowledge of cleft lip and palate among a group of recently-graduated dentists. MATERIALS AND METHODS: A multiple-site, cross-sectional questionnaire-based study was conducted. The study population included recently graduated dentists involved in a dental internship program. A bespoke questionnaire was developed and validated, with internal consistency assessed using Cronbach's alpha and factor analysis performed. A 47-item prototype was distilled into a 15-item questionnaire. This was distributed to the participants with a response rate of 67% obtained. RESULTS: The overall proportion of correct responses among dental interns was moderate (73%). The best results were found in relation to CLP treatment including the effect of unfavorable surgical outcomes on speech (89.5%) and the impact of CLP on the occlusion (87.6%). The lowest rate of correct responses (26.7%) was identified in relation to the association between CLP and smoking. CONCLUSION: A validated CLP questionnaire was developed, permitting evaluation of the knowledge of cleft lip and palate and its management among recently graduated dentists. There is limited appreciation among dental interns of the risk factors for CLP as well as post-surgical complications. Given that general dentists are often the gatekeepers for the management of patients with cleft lip and palate, it is important that the findings of this survey are used to inform the curriculum and teaching of cleft lip and palate.