Differences in velopharyngeal pressures during speech sound production in patients with unilateral cleft lip and palate (UCLP) and healthy individuals.

Background: During articulation the velopharynx needs to be opened and closed rapidly and a tight closure is needed. Based on the hypothesis that patients with cleft lip and palate (CLP) produce lower pressures in the velopharynx than healthy individuals, this study compared pressure profiles of the velopharyngeal closure during articulation of different sounds between healthy participants and patients with surgically closed unilateral CLP (UCLP) using high resolution manometry (HRM). Materials and methods: Ten healthy adult volunteers (group 1: 20-25.5 years) and ten patients with a non-syndromic surgically reconstructed UCLP (group 2: 19.1-26.9 years) were included in this study. Pressure profiles during the articulation of four sounds (/i:/, /s/, /ʃ/ and /n/) were measured by HRM. Maximum, minimum and average pressures, time intervals as well as detection of a previously described 3-phase-model were compared. Results: Both groups presented with similar pressure curves for each phoneme with regards to the phases described and pressure peaks, but differed in total pressures. An exception was noted for the sound /i:/, where a 3-phase-model could not be seen for most patients with UCLP. Differences in velopharynx pressures of 50% and more were found between the two groups. Maximum and average pressures in the production of the alveolar fricative reached statistical significance. Conclusions: It can be concluded that velopharyngeal pressures of patients with UCLP are not sufficient to eliminate nasal resonance or turbulence during articulation, especially for more complex sounds. These results support a general understanding of hypernasality during speech implying a (relative) velopharyngeal insufficiency.

A comparison of maxillofacial growth in Chinese children with isolated cleft palate treated with two different palatoplasty techniques without relaxing incisions: a preliminary study.

OBJECTIVE: To assess the maxillofacial growth of patients with isolated cleft palate following the Sommerlad-Furlow modified technique and compare it with the effect of the Sommerlad technique. STUDY DESIGN: A Retrospective Cohort Study. METHODS: A total of 90 participants, 60 patients with non-syndromic isolated soft and hard cleft palate (ISHCP) underwent primary palatoplasty without relaxing incision (30 patients received the Sommerlad-Furlow modified (S-F) technique and 30 received Sommerlad (S) technique). While the other 30 were healthy noncleft participants with skeletal class I pattern (C group). All participants had lateral cephalometric radiographs at least 5 years old age. All the study variables were measured by using stable landmarks, including 11 linear and 9 angular variants. RESULTS: The means age at collection of cephalograms were 6.03 ± 0.80 (5-7 yrs) in the S group, 5.96 ± 0.76 (5-7 yrs) in the S-F group, and 5.91 ± 0.87 (5-7 yrs) in the C group. Regarding cranial base, the results showed that there were no statistically significant differences between the three groups in S-N and S-N-Ba. The S group had a significantly shortest S-Ba than the S-F & C groups (P = 0.01), but there was no statistically significant difference between S-F and C groups (P = 0.80). Regarding skeletal maxillary growth, the S group had significantly shorter Co-A, S- PM and significantly less SNA angle than the C group (P = < 0.01). While there was no significant difference between S-F & C groups (P = 0.42). The S group had significantly more MP-SN inclination than the C group (P = < 0.01). Regarding skeletal mandibular growth, there were no statistically significant differences in all linear and angular mandibular measurements between the three groups, except Co-Gn of the S group had a significantly shorter length than the C group (P = 0.05). Regarding intermaxillary relation, the S-F group had no significant differences in Co-Gn-Co-A and ANB as compared with the C group. The S group had significantly less ANB angle than S-F & C groups (P = 0.01 & P = < 0.01). In addition, there were no significant differences in all angular occlusal measurements between the three groups. CONCLUSION: As a preliminary report, Sommerlad-Furlow modified technique showed that maxillary positioning in the face tended to be better, and the intermaxillary relationship was more satisfactory than that in Sommerlad technique when compared them in healthy noncleft participants.

Middle-ear effusion in children with cleft palate: congenital or acquired?

OBJECTIVE: Cleft palates are one of the most common congenital malformations. Because of the loss of Eustachian tube function, middle-ear ventilation is reduced. The aim of this study was to determine if middle-ear effusions were present at birth or at the three-month audiological evaluation. METHOD: A total of 53 children with a cleft palate were included. Data review included the results of newborn hearing screening, microscopic findings, a tympanometry, a free field audiometry and intra-operative findings. RESULTS: A total of 58.4 per cent of patients had a median, 26.4 per cent had a bilateral, 11.3 per cent had a unilateral and 3.8 per cent had a limited soft palate cleft. Newborn hearing screening showed a pass in 83.1 per cent of newborns bilaterally. The first ear microscopy showed a bilateral middle-ear effusion in 90.6 per cent of cases. During cleft surgery, bilateral paracentesis was performed in all cases, and in 90.6 per cent middle-ear effusion was sucked out. CONCLUSION: The majority of children with a cleft palate do not present with middle-ear effusion at birth. It develops within several days or weeks of life.

Speech Development in Cleft Palate with and without Robin Sequence.

BACKGROUND: Robin sequence is defined as the triad of micrognathia, glossoptosis, and upper airway obstruction. In up to 85 percent, it is associated with cleft palate. Many studies have reported worse speech development in Robin sequence children after cleft palate repair. The authors investigated speech development in isolated Robin sequence with cleft palate versus children with cleft palate only at the age of 5 to 6 years. METHODS: All Robin sequence children were treated with the Tübingen palatal plate after birth. Data were collected using the German version of the Great Ormond Street Speech Assessment. Audio and video recordings were reviewed and analyzed separately by two blinded senior phoniatricians based on the German version of the Universal Reporting Parameters for Cleft Palate Speech, and scored to enable comparability of speech outcomes. RESULTS: Forty-four children (Robin sequence, n = 22; cleft palate only, n = 22) were included. Robin sequence children were significantly older at surgery (11.8 months versus 7.1 months; p < 0.001) but younger at study (70.5 months versus 75.2 months; p = 0.035). They also had more severe cleft of the palate (p = 0.006). All children studied showed good to very good speech development without serious impairment. None of the reported parameters on the German version of the Universal Reporting Parameters for Cleft Palate Speech showed significant group differences; the median total score in the Robin sequence group was 23 (interquartile range, 16.5 to 27.5) versus 19 (interquartile range, 17 to 23) in the cleft palate-only group. Statistical analysis revealed no significant effect of group (Z = -1.47; p = 0.14). CONCLUSIONS: No group differences in speech development were found at age 5 to 6 years. Isolated Robin sequence does not necessarily represent a risk for impaired speech development. CLINICAL QUESTION/LEVEL OF EVIDENCE: Risk, II.

Functional aspects of the Eustachian tube by means of 3D-modeling.

The extent of dysfunction of the Eustachian tube (ET) is relevant in understanding the pathogenesis of secondary otological diseases such as acute or chronic otitis media. The underlying mechanism of ET dysfunction remains poorly understood except for an apparent genesis such as a nasopharyngeal tumor or cleft palate. To better describe the ET, its functional anatomy, and the biomechanical valve mechanism and subsequent development of diagnostic and interventional tools, a three-dimensional model based on thin-layer histology was created from an ET in this study. Blackface sheep was chosen as a donor. The 3-D model was generated by the coherent alignment of the sections. It was then compared with the cone-beam computed tomography dataset of the complete embedded specimen taken before slicing. The model shows the topographic relation of the individual components, such as the bone and cartilage, the muscles and connective tissue, as well as the lining epithelium with the lumen. It indicates a limited spiraling rotation of the cartilaginous tube over its length and relevant positional relationships of the tensor and levator veli palatine muscles.

Three-Dimensional Anatomy of the Palatopharyngeus and Its Relation to the Levator Veli Palatini Based on Micro-Computed Tomography.

BACKGROUND: Although multiple studies have been reported on the palatopharyngeus and levator veli palatini, their subtle anatomy and functions remain unclear. The authors elucidated the relationship between these muscles and their functional implications based on three-dimensional digital techniques. METHODS: Cadaveric specimens were stained with iodine-potassium iodide and scanned using micro-computed tomography. The muscle fibers were drawn on the exported Imaging and Communications in Medicine images to reconstruct a three-dimensional model and further simplified. RESULTS: In the soft palate, the palatopharyngeus was divided into three bundles. The largest inferior head was found to attach to the palatine aponeurosis, soft palate, and the hard palate on the oral side, which occupied approximately the anterior 28.4 to 36.2 percent of the soft palate in the midline. The superior head was thin and attached to the palatine aponeurosis and the surrounding mucosa on the nasal side. The posterior head was located posterior to the levator veli palatini with fibers attaching to the levator veli palatini and the median portion of the uvula. The levator veli palatini was clasped by the three heads of the palatopharyngeus. The fasciculi of the palatopharyngeus converged into a bundle of muscles at the pharynx and inserted into the lateral and posterior pharyngeal wall. CONCLUSIONS: The palatopharyngeus is the largest muscle that connects the soft palate and pharyngeal wall; it closely coordinates with the levator veli palatini to control levator veli palatini overlifting, narrow the velopharyngeal port with the help of the superior constrictor, and elevate the pharynx. The palatopharyngeus and levator veli palatini help each other in velopharyngeal closure through coordination from other muscles.

Agenesia e fatores associados em pacientes portadores de fissura labiopalatina: uma revisão de literatura / Agenesis and associated factors in patients with cleft lip and palate: a review of the literature

Objetivo: verificar as principais agenesias dentárias e os fatores associados em portadores de fissura labiopalatina por meio de uma revisão de literatura. Métodos: foi realizada uma revisão de literatura utilizando as bases de dados PubMed, SciELO e Google Acadêmico. Revisão de literatura: foram incluídos 34 artigos, sendo 2 casos clínicos, 15 estudos retrospectivos, 10 revisões de literatura, 4 estudos de caso-controle e 5 estudos transversais; além de 3 referências escolhidas através de outras buscas para complementar a revisão. A partir dos estudos incluídos no trabalho, foi possível verificar que a maior prevalência ocorre no sexo masculino. E a fissura que mais acomete a população é a transforame incisivo. Pôde-se analisar também que não há causa exata, porém, existem fatores predisponentes, como os ambientais e os hereditários. Considerações finais: esta revisão de literatura poderá auxiliar os profissionais de saúde para a realização de diagnóstico e planejamento precoces, a fim de encontrar o melhor e mais eficiente tratamento para cada caso. O tratamento precoce, por uma equipe multidisciplinar, certamente garantirá uma melhor qualidade de vida aos pacientes.(AU)

Objective: to verify the main dental agenesis and associated factors in cleft lip and palate through a literature review. Methods: a literature review was carried out using PubMed, SciELO and Google Scholar databases. Literature review: 34 studies were included, 2 clinical studies, 1 retrospective study, 10 literature reviews, 4 case-control studies and 5 cross-sectional studies. In addition to 3 references chosen through other searches complementary to the review. From the included studies, it was verified a higher frequency of cleft lift and palate for the male sex, and a higher frequency of more severe clefts, such as the incisive transformation, in comparison to the labiopalatine. It can also be analyzed that there is no exact cause; however, there are environmental and hereditary predisposing factors. Final considerations: this literature review may help health professionals to carry out early diagnosis and treatment planning, in order to find the best and most efficient treatment for each case. Early treatment by a multidisciplinary team will certainly guarantee a better quality of life for patients.(AU)

Expanding the clinical phenotype of RASopathies in 38 Turkish patients, including the rare LZTR1, RAF1, RIT1 variants, and large deletion in NF1.

RASopathies are a group of disorders caused by pathogenic variants in the genes encoding Ras/mitogen-activated protein kinase pathway and share overlapping clinical and molecular features. This study is aimed to describe the clinical and molecular features of 38 patients with RASopathies. Sanger or targeted next-generation sequencing of related genes and multiplex ligation-dependent-probe amplification analysis for NF1 were performed. The pathogenic variant detection rate was 94.4%. While PTPN11 was responsible for 50% of 18 patients with Noonan syndrome (NS), SOS1, LZTR1, RIT1, and RAF1 were responsible for the remaining 27.8%, 11.1%, 5.5%, and 5.5%, respectively. Three variants in LZTR1 were novel, of which two were identified in the compound heterozygous state in a patient with intellectual disability and hypertrophic cardiomyopathy, whereas the third variant was found in the heterozygous state in a patient with pulmonary stenosis and normal intelligence. We described pyloric stenosis, knee dislocation, and cleft palate in patients with SOS1, RIT1, and RAF1 variants, respectively, that was not previously reported. We detected a PTPN11 variant in three patients from same family with NS with multiple lentigines. BRAF and MAP2K2 variants were found in eight patients with Cardiofaciocutaneous syndrome. Two variants in HRAS were detected in two Costello syndrome patients, one with a mild and the other with a severe phenotype. While large NF1 deletions were identified in four Neurofibromatosis-NS patients with intellectual disability, intelligence was normal in one patient with missense variant. In conclusion, this study provided three novel variants in LZTR1 and expanded the clinical phenotype of rare RASopathies.

Long-term growth patterns in children born with cleft lip and/or palate. A systematic review.

INTRODUCTION: Introduction: in children with cleft lip and/or palate nutritional status and growth may be impaired due to early life feeding difficulties. Objective: to review the existing literature on the nutritional prognosis during childhood of patients undergoing surgery for cleft lip and/or palate (CLP), their body composition and growth patterns from 2 to 10 years of age, and the possible effects of their early nutritional status on the long-term onset of overweight. Methods: a systematic search of growth and body composition parameters in 2-10 year-old CLP children, including cross-sectional and longitudinal studies, and using the Pubmed and Scopus databases. From the 2,983 retrieved articles, 6 were finally included. Results: two studies out of 6 were longitudinal and the other 4 were cross-sectional, including very heterogeneous samples. Weight and height were used as growth parameters in 2 studies; 2 studies used body mass index (BMI); and the remaining 2 used indexes of nutritional status derived from anthropometric measures. The studies showed discrepancies among results: 3 of them found growth differences between children with CLP and their counterparts, whereas the other 3 did not. The two longitudinal studies did not show any significant differences between the mean BMI z-scores or growth curves of cleft patients and their counterparts. When differences existed, the most affected group was that under 5 years, syndromic children, and adopted children with CL/P. Conclusions: the literature is scarce comparing growth patterns between children with CLP and controls, and results cannot confirm that children with CLP aged 2-10 years, excluding those with syndromes or belonging to vulnerable populations, have different growth patterns or a worse nutritional status than their counterparts.

INTRODUCCIÓN: Introducción: en niños con fisura labial y/o palatina, el estado nutricional y el crecimiento pueden verse afectados debido a dificultades en la alimentación. Objetivos: revisar el pronóstico nutricional de pacientes sometidos a cirugía de fisura labiopalatina (FLP), su composición corporal y sus patrones de crecimiento de los 2 a los 10 años, así como los posibles efectos del estado nutricional durante la primera infancia sobre la aparición posterior de sobrepeso. Métodos: búsqueda sistemática de parámetros de crecimiento y composición corporal en niños con CLP de 2 a 10 años, incluyendo estudios transversales y longitudinales en las bases de datos Pubmed y Scopus. De los 2983 artículos potencialmente relevantes, 6 fueron finalmente incluidos. Resultados: dos estudios de 6 fueron longitudinales y los otros 4, transversales, con muestras muy heterogéneas. El peso y la altura se utilizaron como parámetros de crecimiento en 2 estudios; 2 estudios utilizaron el índice de masa corporal (IMC), y los otros 2, índices del estado nutricional a partir de medidas antropométricas. Los estudios mostraron discrepancias entre los resultados: 3 de ellos encontraron diferencias de crecimiento entre los niños con CLP y sus coetáneos, mientras que los otros 3, no las encontraron. Los dos estudios longitudinales no mostraron diferencias significativas entre el IMC (z-score) ni entre las curvas de crecimiento de los pacientes con FLP y sus coetáneos. Cuando existían diferencias, el grupo más afectado fue el de los menores de 5 años, niños sindrómicos y niños adoptados con CL/P. Conclusiones: la literatura sobre los patrones de crecimiento de los niños con CLP es escasa y los resultados no pueden confirmar que los niños con CLP de 2 a 10 años, excluyendo aquellos con síndromes o pertenecientes a poblaciones vulnerables, tengan patrones de crecimiento diferentes o un peor estado nutricional que sus coetáneos.

"Mine did not breastfeed", mothers' experiences in breastfeeding children aged 0 to 24 months with oral clefts in Uganda.

BACKGROUND: Appropriate breastfeeding is vital for infant and young child nutrition. Annually, oral clefts affect 0.73 per 1000 children in Uganda. Despite this low incidence, children with a cleft face breastfeeding difficulty which affect their nutrition status. In addition, knowledge on maternal experiences with breastfeeding and support is limited. We explored maternal perceptions, experiences with breastfeeding and support received for their children 0 to 24 months with a cleft attending Comprehensive Rehabilitative Services of Uganda (CoRSU) Hospital. METHODS: This cross-sectional study combined quantitative and qualitative methods. We consecutively recruited 32 mothers of children with a cleft aged 0 to 24 months attending CoRSU hospital between April and May 2018. A structured questionnaire collected data on breastfeeding practices and device use (n = 32). To gain a broad understanding of mothers' perceptions and experiences with breastfeeding and support received, we conducted two Focus Group Discussions (in each, n = 5), and 15 In Depth Interviews. Descriptive statistics were analyzed using SPSS software. Qualitative data were analyzed thematically. RESULTS: Of the 32 children with a cleft, 23(72%) had ever breastfed, 14(44%) were currently breastfeeding, and among those under 6 months, 7(35%) exclusively breastfed. Of 25 mothers interviewed in IDIs and FGDs, 17(68%; IDIs = 8/15, FGD1 = 5/5 and FGD2 = 4/5) reported the child's failure to latch and suckle as barriers to breastfeeding. All ten mothers who used the soft squeezable bottle reported improved feeding. Nineteen (76%) mothers experienced anxiety and 14(56%), social stigma. Family members, communities and hospitals supported mothers with feeding guidance, money, child's feeds and psycho-social counselling. Appropriate feeding and psycho-social support were only available at a specialized hospital which delayed access. CONCLUSIONS: Breastfeeding practices were sub-optimal. Mothers experienced breastfeeding difficulties, anxiety and social stigma. Although delayed, feeding, social and psycho-social support helped mothers cope. Routine health care for mothers and their children with a cleft should include timely support.

Genetic variants in S-adenosyl-methionine synthesis pathway and nonsyndromic cleft lip with or without cleft palate in Chile.

BACKGROUND: The S-adenosyl-methionine (SAM) availability is crucial for DNA methylation, an epigenetic mechanism involved in nonsyndromic cleft lip with or without cleft palate (NSCL/P) expression. The aim of this study was to assess the association between single-nucleotide polymorphisms (SNPs) of genes involved in SAM synthesis and NSCL/P in a Chilean population. METHODS: In 234 cases and 309 controls, 18 SNPs in AHCY, MTR, MTRR, and MAT2A were genotyped, and the association between them and the phenotype was evaluated based on additive (allele), dominant, recessive and haplotype models, by odds ratio (OR) computing. RESULTS: Three deep intronic SNPs of MTR showed a protective effect on NSCL/P expression: rs10925239 (OR 0.68; p = 0.0032; q = 0.0192), rs10925254 (OR 0.66; p = 0.0018; q = 0.0162), and rs3768142 (OR 0.66; p = 0.0015; q = 0.0162). Annotations in expression database demonstrate that the protective allele of the three SNPs is associated with a reduction of MTR expression summed to the prediction by bioinformatic tools of its potentiality to modify splicing sites. CONCLUSIONS: The protective effect against NSCL/P of these intronic MTR SNPs seems to be related to a decrease in MTR enzyme expression, modulating the SAM availability for proper substrate methylation. However, functional analyses are necessary to confirm our findings. IMPACT: SAM synthesis pathway genetic variants are factors associated to NSCL/P. This article adds new evidence for folate related genes in NSCL/P in Chile. Its impact is to contribute with potential new markers for genetic counseling.

Surgical management in a severe OSA patient diagnosed with Stickler syndrome.

Stickler syndrome is a genetic disorder of connective tissue. One of the major symptoms associated with this disorder is an oro-facial malformation, which may cause a submucous cleft or a complete cleft of the hard palate. A 32-year-old man diagnosed with Stickler syndrome and a submucosal cleft palate (SMCP) visited our hospital with a chief complaint of excessive daytime sleepiness. The patient was diagnosed with severe obstructive sleep apnea (OSA), and administration of a polysomnography test revealed an apnea-hypopnea index (AHI) of 30.9 events/hour (h). Auto-titrating continuous positive airway pressure was initiated to control the OSA symptoms and subsequently the patient showed some improvement. However, due to continuous velopharyngeal insufficiency symptoms, intravelar veloplasty was performed. Three months after surgery, the AHI had decreased to 12.4 events/h. Recent studies have described a greater risk for OSA in individuals with cleft palate, than in the general population. The present case demonstrates surgical success in a patient with OSA and SMCP, suggesting that palatal surgery may be considered an optional surgical treatment for OSA patients with SMCP.

Preliminary evaluation of pre-speech and neurodevelopmental measures in 7-11-week-old infants with isolated oral clefts.

BACKGROUND: The purpose of this research study was to evaluate the earliest markers of vocal functioning and neurological development in infants with isolated oral cleft of the lip and/or palate (iCL/P). METHODS: Participants were recruited through advertisements and clinic visits at a local mid-western university. A total of eight participants (four unaffected and four with iCL/P), ranging in age from 7.29 to 11.57 weeks, were enrolled and completed demographic and pre-speech measures. A subset of six males (four unaffected and two with iCL/P) successfully completed a structural magnetic resonance imaging scan. RESULTS: Patterns of disrupted vocal control and reduced myelinated white matter were found in participants with iCL/P. CONCLUSIONS: The findings of this study provide a foundation from which to build further research on the neuronal development of infants with oral clefts: the need to evaluate measures of cortical development, inclusion of information on anesthesia exposure and airway obstruction, and suggestions for avoiding identified pitfalls/blocks to obtaining data are discussed. IMPACT: Research in children with isolated oral clefts has demonstrated higher rates of learning disorders connected to subtle differences in brain structure. There is no work evaluating the potential impact of exposure to anesthesia on development. This is the first known attempt to evaluate brain structure and function in infants with isolated oral clefts before exposure to anesthesia. Potential trends of early vocal issues and structural brain differences (less myelinated white matter) were identified in infants with isolated oral clefts compared to unaffected controls. Differences in brain structure and function in infants with isolated oral clefts may be present before surgery.

Ischial tuberosity: new donor site for bone grafts in animal cleft research.

In the context of cleft repair in animal research in rat models, different areas can be used for bone grafting. The aim of the present study was to present the tuberosity of the ischium as a new donor site and to evaluate its quality in relation to an artificial alveolar cleft. Four weeks after creating experimental alveolar clefts in seven Wistar rats, the repair was performed in the now twelve-week-old male animals using bone blocks grafted from the ischial tuberosity. Two days before surgery and two as well as twenty-eight days after surgery, microCT scans were performed, and the grafted bone blocks were analyzed regarding height, width, thickness, and volume. Additionally, bone mineral density (BMD) and bone volume fraction (BV/TV) were measured in the repaired cleft. The mean bone volume of the graft was about 19.77 ± 7.77mm3. Immediately after jaw reconstruction the BMD and BV/TV were about 0.54 ± 0.05 g/cm3 and 54.9 ± 5.07% for the transplant and about 1.13 ± 0.08 g/cm3 and 94.5 ± 3.70%, respectively, for the surrounding bone. Four weeks later the BMD and BV/TV were about 0.57 ± 0.13 g/cm3 and 56.60 ± 13.70% for the transplant and about 11.17 ± 0.07 g/cm3 and 97.50 ± 2.15%, respectively, for the surrounding bone. A hip fracture was found in four of the animals after surgery. The ischial tuberosity offers large bone blocks, which are sufficient for cleft repair in the rat model. However, the bone quality regarding BMD and BV/TV is less compared with the surrounding bone of the alveolar cleft, even after a period of 4 weeks, despite recognizable renovation processes.

Three-dimensional finite element analysis of initial displacement and stress on the craniofacial structures of unilateral cleft lip and palate model during protraction therapy with variable forces and directions.

Maxillary protraction and expansion is recommended to treat midfacial deficiency in patients with cleft lip and palate (CLP), where amount and direction of forces can change displacement and stress. This study assessed the initial displacement and stresses using Facemask and Maxgym forces with and without RME at +20∘, 0∘, and -20∘ angulation using a finite element (FE) model of unilateral cleft lip and palate (UCCLP). The Initial displacement and stress were more for protraction with expansion as compared to only protraction. Asymmetric displacement was observed with more on cleft than on noncleft side and more on dental than skeletal structures. Palatal plane rotated less upward, increased arch width and decreased arch length was observed with protraction with expansion.

Assessment of Masticatory Muscle Function in Patients with Bilateral Complete Cleft Lip and Palate and Posterior Crossbite by means of Electromyography.

Aim: The aim of this study was to evaluate the electrical activity of the masticatory muscles in children with a bilateral complete cleft lip and palate (BCCLP) and posterior crossbite as well as in noncleft subjects with no malocclusion. Another purpose of the study was to examine the possible factors associated with this muscle activity. Methods: The study included 52 children with mixed dentition and Class I occlusions (20 patients with nonsyndromic BCCLP and 32 subjects with no clefts). All the cleft patients had posterior crossbite. The surface electromyography (sEMG) was used to identify the electrical potentials of the temporalis and masseter muscles. The electromyographical (EMG) recordings were taken with a DAB-Bluetooth Instrument (zebris Medical GmbH, Germany) at rest and during maximum voluntary clenching (MVC). The relationships between muscle EMG activity and independent variables were identified through multivariate logistic regression analysis. Results: The EMG activity of the temporalis muscles at rest was significantly higher in BCCLP patients with malocclusion in comparison with the noncleft subjects with normal occlusion. During MVC, significantly lower electrical potentials of the temporalis and masseter muscles were observed in cleft patients compared to the noncleft group. The presence of BCCLP, unilateral posterior crossbites, increased vertical overlap, and increased overjet are factors strongly associated with higher temporalis muscle EMG activity at rest. Conclusion: The use of surface electromyography in imaging muscle function showed that children with BCCLP and posterior crossbite exhibited altered masticatory muscle potentials at rest and during clenching. The presence of unilateral posterior crossbites, increased vertical overlap, and increased overjet had a significant impact on temporalis muscle activity in cleft patients. This knowledge is important in the aspect of early and proper diagnosis and orthodontic treatment of malocclusions, thereby achieving correct occlusion and improvement in muscle function.

Characteristics of velopharyngeal dysfunction in 22q11.2 deletion syndrome: a retrospective case-control study.

OBJECTIVE: To identify and describe the dynamic features of velopharyngeal dysfunction (VPD) in patients with 22q11.2 deletion syndrome relative to patients with non-syndromic cleft palates. STUDY DESIGN: Retrospective case-control study. SETTING: Pediatric tertiary care center. SUBJECTS AND METHODS: A total of 30 children (aged 9-16 years) with VPD were included in this study. Fifteen children with a definitive diagnosis of 22q11.2 deletion syndrome requiring surgical VPD repair were included in the 22q11.2 deletion syndrome group. Fifteen age- and sex-matched children with non-syndromic cleft palate requiring surgical VPD repair were included in the non-syndromic cleft palate group for comparison. Velar displacement, lateral pharyngeal wall displacement, and lateral pharyngeal wall motion pattern data were extracted from preoperative Multiview Videofluoroscopy imaging studies of all children and compared across groups. RESULTS: Lateral pharyngeal wall displacement was found to be reduced in the 22q11.2 deletion syndrome group (U = 29.50, p = .001, r = .63). However, measures of velar displacement were not observed to differ between groups. Similarly, lateral pharyngeal wall motion pattern distributions were not found to differ across these two groups. CONCLUSIONS: Velopharyngeal dysfunction in patients with 22q11.2 deletion syndrome showed differences in dynamic velopharyngeal function when compared to non-syndromic cleft palate patients. The current findings provide initial insights into the unique aspects of velopharyngeal dysfunction for patients with 22q11.2 deletion syndrome. These findings may guide further efforts directed toward understanding the dynamic velopharyngeal characteristics of this population and potentially optimize surgical management and functional outcomes.

The biomechanical evaluation of magnetic forces to drive osteogenesis in newborn's with cleft lip and palate.

This study examined the potential for dental magnets to act as a driving force for osteogenesis in the palate of newborns with a unilateral cleft lip and palate. In the first part of the study dental magnets were arranged in a set up mimicking a distraction device and the curves of the magnetic attraction force versus gap distance curves generated, with and without the presence of palatal rugae tissue in between both sides of the distraction device. The attraction forces ranged from 1 to 12 N depending on the gap distance and the presence of soft tissue in the gap. In the second part of the study these forces were used as input for a 3D finite element model of the palate of a newborn affected by unilateral cleft lip and palate. In the analysis of load transfer, it was found that the strains generated by a magnetically induced distraction exceed 1,500 µstrain suggesting that bone locally is submitted to mild overload leading to bone apposition.

The Impact of Middle Eastern Crisis on Cleft Care: Evaluation of Demographic and Perioperative Data in Syrian Refugees With Cleft Lip and Palate.

The cleft lip and palate (CL/P) define a heterogeneous group of congenital deformities, which are morphologically highly diverse, with a complex and multifactorial etiology. Affected children may experience social problems due to negative effects on speech, hearing, facial appearance, as well as negative psychological effects on the parents. In 2011, after the civil war began in Syria, a great wave of immigration began to Turkey and other neighboring countries. Refugees may not be able to receive optimal health care because of cultural differences, socioeconomic status, language problems, and psychosocial problems. To increase awareness about this issue, the authors investigated the demographic, perioperative, and post-operative data of Syrian refugee patients with CL/P who were admitted to our cleft center between January 2016 and May 2019. Sixty-eight refugees with CL/P were detected as the result of the screening. Unlike the protocol the authors follow in our center, cleft lip repair was performed at an average of 7.6 months and cleft palate repair was performed at an average of 28.7 months of age. The rate of fistula was found 26.2%.The civil war in Syria has caused the repair of the patients with cleft lip and palate at a later age, hampered the follow-up and treatment, and caused more complications. Considering the demographic, social, economic and cultural characteristics of the patients, it was demonstrated that the necessary health precautions and infrastructure should be provided on the pillar of plastic surgery.

Evaluation of nasal airflow resistance of operated unilateral cleft lip and palate surgery patients compared with skeletal class III individuals.

OBJECTIVE: The purpose of this study was to measure the nasal airflow resistance of operated unilateral cleft lip and palate (UCLP) patients using the anterior rhinomanometry method compared with non-cleft skeletal class III individuals with isolated maxillary hypoplasia serving as the control group. MATERIAL&METHODS: Sixteen UCLP patients who have undergone lip and palate reconstructions and alveolar cleft grafting but not advanced surgeries as the study group and 16 non-cleft skeletal class III participants as the control group (corresponded for age and sex to the study group) were included in this prospective study. All of the patients had class III occlusion due to isolated maxillary hypoplasia. Nasal airflow was measured with anterior rhinomanometry (in ml/sec) for each nostril separately. For evaluation of nasal side subgroups of the UCLP group for nasal resistance, the cleft sides and non-cleft sides of individuals were examined in two separate groups. The nasal side resistance of the control group was evaluated as the average of both nasal side resistances. The nasal resistances for nasal sides and total nasal resistance were measured in Pa/L/sec. The observed data were analyzed using Kolmogorov-Smirnov test used for normality, and the Student t-test and Mann Whitney U test used to determine the differences between the groups. P values of <0.05 were considered statistically significant. RESULTS: As the primary outcome of this study, it was found that both the cleft sides and non-cleft sides of the UCLP individuals had statistically significantly higher nasal resistance than the healthy skeletal class III control group (p < 0.05). It was revealed that the cleft side had more nasal resistance than the non-cleft side for both inspiration and expiration, but the results were not statistically significant (p = 0.41/p = 0.28). CONCLUSION: The results showed that operated UCLP patients have nasal resistance and breathing problems, even when lip, palate, and alveolar cleft operations have been performed.