Cleft Defects Research Articles

Histologic Evaluation of the Effects of Folinic Acid Chitosan Hydrogel and Botulinum Toxin A on Wound Repair of Cleft Lip Surgery in Rats.

The aim of the present study was to compare the effects of folinic acid chitosan hydrogel and botulinum toxin A on the wound repair of cleft lip surgery in rat animal models. Cleft lip defects were simulated by triangular incisions in the upper lip of 40 Wistar rats. Then, the rats were randomly assigned to four groups: control (CTRL), chitosan hydrogel (CHIT), and folinic acid chitosan hydrogel (FOLCHIT), in which the wounds were covered by a gauze pad soaked in normal saline, chitosan hydrogel, and folinic acid chitosan hydrogel, respectively for 5 min immediately after closure; and botulinum toxin A (BOT) with the injection of 3 units of botulinum toxin A in the wound region. Fibroblast proliferation, collagen deposition, inflammatory cell infiltration, neovascularization, and epithelial proliferation and each parameter were rated on days 14 and 28. Statistical analysis was performed by Kolmogorov-Smirnov test, Shapiro-Wilk test, Kruskal-Wallis, and post-hoc tests (α = 0.05). The mean score for fibroblast proliferation was significantly higher in the FOLCHIT group compared with the BOT group at days 14 and 28 (p < 0.001, p = 0.012, respectively). At day 28, collagen deposition was significantly higher in the FOLCHIT group compared with the BOT group (p = 0.012). No significant difference was observed between the inflammatory infiltration of the study groups at the two time points (p = 0.096 and p = 1.000, respectively). At day 14, vascular proliferation of group FOLCHIT was significantly higher than groups CTRL and CHIT (p = 0.001 and p = 0.006, respectively). The epithelial proliferation in the FOLCHIT group was significantly higher than groups CHIT and CTRL at day 14 (p = 0.006 and p = 0.001, respectively) and day 28 (p = 0.012). In simulated lip cleft defects, topical application of folinic acid induces faster initial regeneration by higher inflammation and cellular proliferation, at the expense of a higher tendency for scar formation by slightly higher fibroblast proliferation and collagen deposition. While injection of botulinum toxin A provides less fibroblast proliferation and collagen deposition, and thus lower potential for scar formation compared with the folinic acid group. Therefore, in wounds of the esthetic zone, such as cleft lip defects, the application of botulinum toxin A shows promising results.

Osteoconductive Silk Fibroin Binders for Bone Repair in Alveolar Cleft Palate: Fabrication, Structure, Properties, and In Vitro Testing.

Osteoconductive silk fibroin (SF) binders were fabricated for the bone repair of an alveolar cleft defect. Binders were prefigureared by mixing different ratios of a mixture of random coils and SF aggregation with SF fibrils: 100:0 (SFB100), 75:25 (SFB75), 50:50 (SFB50), 25:75 (SFB25), and 0:100 (SFB0). The gelation, molecular organization, structures, topography, and morphology of the binders were characterized and observed. Their physical, mechanical, and biological properties were tested. The SF binders showed gelation via self-assembly of SF aggregation and fibrillation. SFB75, SFB50, and SFB25 had molecular formation via the amide groups and showed more structural stability than SFB100. The morphology of SFB0 demonstrated the largest pore size. SFB0 showed a lowest hydrophilicity. SFB100 showed the highest SF release. SFB25 had the highest maximum load. SFB50 exhibited the lowest elongation at break. Binders with SF fibrils showed more cell viability and higher cell proliferation, ALP activity, calcium deposition, and protein synthesis than without SF fibrils. Finally, the results were deduced: SFB25 demonstrated suitable performance that is promising for the bone repair of an alveolar cleft defect.

Risk factors associated with cleft lip and palate birth defects in Jordan: A retrospective case-controlled study

Risk factors associated with cleft lip and palate birth defects in Jordan: A retrospective case-controlled study

ПРИМЕНЕНИЕ ДИОДНОГО ЛАЗЕРА, УЛЬТРАФОНОФОРЕЗА И РАЗОБЩАЮЩЕЙ НЕБНОЙ ПЛАСТИНКИ В ПРЕДОПЕРАЦИОННОЙ ПОДГОТОВКЕ К РЕОПЕРАЦИИ ДЕФЕКТА НЕБА У ДЕТЕЙ ПОСЛЕ УРАНОПЛАСТИКИ

Objectives. Analysis of oral fluid in children with congenital cleft palate and palate defect after uranoplasty.&#x0D; Purpose. To analyze oral fluid in children with congenital cleft palate and palate defect after uranoplasty to determine the levels of pro-inflammatory (IL-1β, IL-6, TNF-α) and anti-inflammatory (IL-4, IL-10) cytokines and secretory immunoglobulin sIgA and evaluate physico-chemical (salivation rate, kinematic viscosity, pH) and biochemical parameters (calcium, magnesium, phosphorus, protein) of mixed saliva.&#x0D; Methodology. The article presents the results of studying the parameters of the oral fluid of 109 children aged 6–12 years with congenital cleft palate and palate defect after uranoplasty and 50 practically healthy children of the same age.&#x0D; Results. Hyperstimulation of pro-inflammatory (IL-1β, IL-6, TNF-α) and anti-inflammatory (IL-4, IL-10) cytokines in the oral fluid and a decrease in the average level of secretory immunoglobulin sIgA. In children with congenital cleft palate and defects remaining after uranoplasty, there was a deviation in the physicochemical and biochemical parameters of the oral fluid, which are manifested by a decrease in the rate of salivation, an increase in its viscosity and a shift in pH to the acid side. In contrast to the group of children without dental pathology, children with cleft palate and defects after uranoplasty showed a decrease in the content of total protein, phosphorus and calcium in the mixed saliva and an increase in the content of magnesium.&#x0D; Conclusions. The obtained laboratory data indicate the presence of a local inflammatory process in the oral cavity and a decrease in local immunological anti-inflammatory factors. This should be taken into account for the development of methods for preoperative sanitation of the oral mucosa in children with congenital cleft palate and a palate defect after uranoplasty at the stage of preparation for reoperation.

ИММУНОЛОГИЧЕСКИЕ, ФИЗИКО-ХИМИЧЕСКИЕ И БИОХИМИЧЕСКИЕ ПОКАЗАТЕЛИ РОТОВОЙ ЖИДКОСТИ У ДЕТЕЙ С ВРОЖДЕННОЙ РАСЩЕЛИНОЙ НЕБА И ПОСЛЕОПЕРАЦИОННЫМ ДЕФЕКТОМ НЕБА

Objectives. Analysis of oral fluid in children with congenital cleft palate and palate defect after uranoplasty.&#x0D; Purpose. To analyze oral fluid in children with congenital cleft palate and palate defect after uranoplasty to determine the levels of pro-inflammatory (IL-1β, IL-6, TNF-α) and anti-inflammatory (IL-4, IL-10) cytokines and secretory immunoglobulin sIgA and evaluate physico-chemical (salivation rate, kinematic viscosity, pH) and biochemical parameters (calcium, magnesium, phosphorus, protein) of mixed saliva.&#x0D; Methodology. The article presents the results of studying the parameters of the oral fluid of 109 children aged 6–12 years with congenital cleft palate and palate defect after uranoplasty and 50 practically healthy children of the same age.&#x0D; Results. Hyperstimulation of pro-inflammatory (IL-1β, IL-6, TNF-α) and anti-inflammatory (IL-4, IL-10) cytokines in the oral fluid and a decrease in the average level of secretory immunoglobulin sIgA. In children with congenital cleft palate and defects remaining after uranoplasty, there was a deviation in the physicochemical and biochemical parameters of the oral fluid, which are manifested by a decrease in the rate of salivation, an increase in its viscosity and a shift in pH to the acid side. In contrast to the group of children without dental pathology, children with cleft palate and defects after uranoplasty showed a decrease in the content of total protein, phosphorus and calcium in the mixed saliva and an increase in the content of magnesium.&#x0D; Conclusions. The obtained laboratory data indicate the presence of a local inflammatory process in the oral cavity and a decrease in local immunological anti-inflammatory factors. This should be taken into account for the development of methods for preoperative sanitation of the oral mucosa in children with congenital cleft palate and a palate defect after uranoplasty at the stage of preparation for reoperation.

Biomechanical consequences of alveolar cleft defect

Cleft palate is a common birth defect, requiring interventions that must be timed according to maxillary growth and patients often require multiple procedures to repair an alveolar cleft defect. Mechanical variables, such as pre‐surgical defect shape and size, have been suggested to affect the success of surgical repair, but the biomechanical consequences of these variables are not well understood. We hypothesize that mechanical performance of bony craniofacial structures will vary with cleft morphology.3D surface models of alveolar cleft defects were created from cone‐bean (CB) CT scans of adolescent and young adult patients (n=36; IRB: 201601711). Surface semilandmarks were used to conduct a cluster analysis, identifying common axes of variation which broadly differentiated between defects that are mediolaterally wider from those that are superoinferiorly taller.The centroid cleft defect shape from Cluster I was selected for preliminary biomechanical analysis. To isolate the mechanical effects of a defect of this shape/size, we used a previously published human cranial finite element model (FEM) as a baseline for model construction and comparison of performance. Patient CBCT was scaled and registered to the FEM and used to reconstruct the defect in the maxilla of the baseline model. Identical boundary conditions (material properties, muscle forces and joint and bite constraints) were applied to the cleft and non‐cleft FE models, to simulate biting on different teeth.Our results reveal notable and unexpected differences in deformation and strain regimes of the cleft and non‐cleft models. Although both models show characteristic regions of elevated strain on the biting‐side, the cleft‐model experiences a marked reduction (50‐90%) in strain on the balancing (non‐biting) side, due to the cleft disrupting the transmission of internal forces across the maxilla and palate from the biting to the balancing side. Importantly, regions that experience diffuse low‐level shear strains in our baseline model drop to nearly undetectable levels in the cleft model.We conclude that it is possible that the reduction of strain below this critical threshold could impede osteogenic mechanical signaling. This has important clinical implications for alveolar cleft defect repair and the development of novel scaffold‐based methods that both survive in vivo and restore functional orofacial load paths.

Outcomes of primary repair of sternal cleft defects: Providing a "bony cover".

Sternal clefts are rare congenital anterior chest wall defects created by a lack of midline thoracic fusion. Various surgical repairs have been proposed to provide protection to underlying viscera in these defects. This study aims to perform primary sternal cleft repair using techniques, leading to the provision of a complete bony cover and to assess their outcomes on follow-ups. During 2009-2020, seven patients were referred to our unit with sternal defects. Out of them, four infants with sternal clefts underwent primary repair using bilateral perichondrial flap creation of the sternal bars and sliding costal chondrotomy at our institute. In one of them with a wider defect, bilateral "intraperiosteal" sliding clavicular osteotomy was additionally performed to achieve tension-free closure. Satisfactory surgical outcomes were achieved with an uneventful postoperative period. On follow-up, all four patients are thriving well and have a stable anterior chest wall. Those with follow-ups longer than 5 years showed evidence of bone formation. Bony cover to the heart can be provided in all varieties of sternal cleft defects using primary surgical repair early in infancy. The delay in surgical correction increases the complexity of the procedure and may require the use of prosthetic material which has its own disadvantages.

Specialized Pro-Resolving Mediators Reduce Scarring After Cleft Lip Repair.

ObjectiveResidual scarring after cleft lip repair surgery remains a challenge for both surgeons and patients and novel therapeutics are critically needed. The objective of this preclinical experimental study was to evaluate the impact of the methyl-ester of pro-resolving lipid mediator lipoxin A4 (LXA4-ME) on scarring in a novel rabbit model of cleft lip repair.MethodsA defect of the lip was surgically created and repaired in eight six-week old New Zealand white rabbits to simulate human cleft lip scars. Rabbits were randomly assigned to topical application of PBS (control) or 1 ug of LXA4-ME (treatment). 42 days post surgery all animals were euthanized. Photographs of the cleft lip area defect and histologic specimens were evaluated. Multiple scar assessment scales were used to compare scarring.ResultsAnimals treated with LXA4-ME exhibited lower Visual Scar Assessment scores compared to animals treated with PBS. Treatment with LXA4-ME resulted in a significant reduction of inflammatory cell infiltrate and density of collagen fibers. Control animals showed reduced 2D directional variance (orientation) of collagen fibers compared to animals treated with LXA4-ME demonstrating thicker and more parallel collagen fibers, consistent with scar tissue.ConclusionsThese data suggest that LXA4-ME limits scarring after cleft lip repair and improves wound healing outcomes in rabbits favoring the resolution of inflammation. Further studies are needed to explore the mechanisms that underlie the positive therapeutic impact of LXA4-ME on scarring to set the stage for future human clinical trials of LXA4-ME for scar prevention or treatment after cleft lip repair.

Secondary Alveolar Bone Grafting in Patients with Cleft Lip and Palate: A Step-by-Step Video Series.

Secondary alveolar bone grafting is one of the key surgical procedures performed to restore dental arch continuity and facilitate tooth eruption in patients with cleft lip and palate. Harvest of cancellous bone graft from the iliac crest has become the gold standard; however. there is no consensus regarding the ideal technique. An optimal bone harvest technique must be aimed at producing minimal donor-site morbidity and patient discomfort. The success of the bone grafting procedure depends largely on the surgical technique. To restore cleft alveolar defects, one must be able to conceptualize the different underlying aspects of the problem to perform an effective surgical repair. It is important for surgeons-in-service to adopt newer techniques that significantly enhance the overall outcome. This article describes two techniques of iliac bone harvest and the principles involved in execution of the alveolar bone grafting procedure. The accompanying four-part video series depicts the bone harvest, flap design, dissection of the recipient cleft alveolus, and bone grafting in a step-by-step manner.

High prevalence of deleterious mutations in concomitant nonsyndromic cleft and outflow tract heart defects

Our previous work demonstrating enrichment of outflow tract (OFT) congenital heart disease (CHD) in children with cleft lip and/or palate (CL/P) suggests derangements in common underlying developmental pathways. The current pilot study examines the underlying genetics of concomitant nonsyndromic CL/P and OFT CHD phenotype. Of 575 patients who underwent CL/P surgery at Children's Hospital Los Angeles, seven with OFT CHD, negative chromosomal microarray analysis, and no recognizable syndromic association were recruited with their parents (as available). Whole genome sequencing of blood samples paired with whole‐blood‐based RNA sequencing for probands was performed. A pathogenic or potentially pathogenic variant was identified in 6/7 (85.7%) probands. A total of seven candidate genes were mutated (CHD7, SMARCA4, MED12, APOB, RNF213, SETX, and JAG1). Gene ontology analysis of variants predicted involvement in binding (100%), regulation of transcription (42.9%), and helicase activity (42.9%). Four patients (57.1%) expressed gene variants (CHD7, SMARCA4, MED12, and RNF213) previously involved in the Wnt signaling pathway. Our pilot analysis of a small cohort of patients with combined CL/P and OFT CHD phenotype suggests a potentially significant prevalence of deleterious mutations. In our cohort, an overrepresentation of mutations in molecules associated with Wnt‐signaling was found. These variants may represent an expanded phenotypic heterogeneity within known monogenic disease genes or provide novel evidence of shared developmental pathways. The mechanistic implications of these mutations and subsequent developmental derangements resulting in the CL/P and OFT CHD phenotype require further analysis in a larger cohort of patients.

A Novel and Practical Protocol for Three-Dimensional Assessment of Alveolar Cleft Grafting Procedures.

To evaluate the reproducibility and accuracy of a new, easy-to-use volumetric assessment of the alveolar cleft. Twelve cone-beam computed tomography (CBCT) datasets of patients with a unilateral cleft lip, alveolus, and palate were evaluated by two investigators. Residual alveolar cleft calcified volume one year after surgery was analyzed by using standardized landmarks to determine the borders of the cleft defect and semi-automatically segment the alveolar cleft defect. The Dice-coefficient between observers for the segmented preoperative alveolar cleft defect was 0.81. Average percentage of residual alveolar cleft calcified material was 66.7% one year postoperatively. This study demonstrates a reliable and practical semi-automatic three-dimensional volumetric assessment method for unilateral clefts using CBCT.

СОСТОЯНИЕ ТКАНЕЙ ПАРОДОНТА У ДЕТЕЙ С ВРОЖДЕННОЙ РАСЩЕЛИНОЙ НЕБА И ДЕФЕКТОМ ПОСЛЕ УРАНОПЛАСТИКИ

The article is devoted to the study of the state of periodontal tissues in children with congenital cleft palate and defects remaining after uranoplasty.&#x0D; Objectives. To determine the characteristics of the state of periodontal tissues in children with defects remaining after uranoplasty in congenital cleft palate and to compare the obtained clinical data with a group of apparently healthy children.&#x0D; Methodology. The article presents the results of a clinical dental examination of 109 children aged 6-12 years with congenital cleft palate after uranoplasty and residual defects. In the examined children, the hygienic state of the oral cavity was assessed, the severity of gingivitis and the condition of the periodontal tissues were determined. The hygienic state of the oral cavity was assessed by the Fedorov-Volodkina index (1964), the Green-Vermillion IGR-U (C. Green, JR Vermillion, OHI-S), the periodontal condition using the complex periodontal index KPI (Leus P.A., 1988) and papillary-marginal-alveolar index (PMA, C. Parma, 1960).&#x0D; Results. In children with remaining postoperative defects with severe congenital cleft palate, the level of hygiene is more often characterized as "poor" and "unsatisfactory" than in healthy children; the highest incidence of periodontal diseases in childhood was revealed. In most cases, children with postoperative palate defects were found to have mild and moderate severity of gingivitis according to the PMA index.&#x0D; Conclusions. The results of the study made it possible to obtain clinical and dental data on periodontal diseases in children with congenital cleft palate and defects remaining after uranoplasty. To prepare a patient for a reconstructive plastic surgery to eliminate the remaining palate defect, it is necessary to introduce a set of measures to treat periodontal diseases and improve the health of the oral cavity into the rehabilitation algorithm for children with congenital cleft palate.

ОЦЕНКА МИКРОБИОЛОГИЧЕСКОГО ПРОФИЛЯ В ОБЛАСТИ ОСТАТОЧНОГО ДЕФЕКТА НЕБА ПОСЛЕ УРАНОПЛАСТИКИ У ДЕТЕЙ С ВРОЖДЕННОЙ РАСЩЕЛИНОЙ НЕБА

The article is devoted to the study of the microbiological profile of the mouth and nose in children with congenital cleft palate and remaining defects after uranoplasty.&#x0D; Objectives. To determine the microbial profile of the mouth and nose in children with a cleft palate and defects after uranoplasty and conditionally healthy children in the control group.&#x0D; Methodology. The microflora of the oral cavity and nose of 109 children aged 6-12 years with a severe form of congenital cleft palate (combined cleft of the upper lip, alveolar ridge and palate, as well as a wide isolated cleft of the palate) after uranoplasty and residual palate defects in the dispensary was studied observation of the maxillofacial surgeon of the Republican Children's Clinical Hospital of the Republic of Bashkortostan and 50 apparently healthy children of the same age group.&#x0D; In the examined children, microbiological studies of biological materials were carried out using standard methods in the conditions of the bacteriological laboratory of the Republican Children's Clinical Hospital, Ufa. The material was taken with a sterile cotton swab from the mucous membrane in the area of the palate defect and from the side of the nasal cavity and sown on various nutrient media. The presence of pathogenic and opportunistic microorganisms was determined.&#x0D; Results. All 109 examined children with remaining postoperative defects with congenital cleft palate revealed atypical microflora in the oral cavity and in the nasal cavity, mostly represented by pathogenic streptococci and staphylococci, yeast-like fungi.&#x0D; Conclusions. The data obtained as a result of the study on the presence of pathogenic microflora from the oral cavity and nose in the area of the residual palate defect after uranoplasty in children with congenital cleft palate is the basis for the need to develop therapeutic and prophylactic measures aimed at improving the microbiocenosis of adjacent cavities at the stage of preparing the patient for reconstructive - plastic surgery to eliminate a defect in the palate.

Preliminary Study in Relationship Between Cleft Lip and Palatal Bony Defect in Submucous Cleft Palate.

Submucous cleft palate, presenting as varying degrees of palatal bony defect, can be difficult to detect in its early stage. The connection between submucous cleft palate and cleft lip has been noticed by clinicians but are rarely reported in literature.Aims:To investigate the correlation between the degree of deformity of palatal bony defect and that of cleft lip.Patients and Methods:Thirty-four patients with unilateral (n = 23) or bilateral (n = 11) cleft lip presenting with submucous cleft palate were included. Patients were divided into 3 groups according to the degree of malformation of cleft lip (microform, incomplete, and complete). The length and width of palatal bony defect was then measured from the palatal computer tomography.Results:In patients with unilateral cleft lip, the proportions of microform cleft lip, incomplete cleft lip, and complete cleft lip were 17.4%, 60.9%, and 21.7%, respectively. In patients with bilateral cleft lip, there were 3 cases with microform and 1 case with incomplete cleft lip on both sides. No correlation was found between the length or relative width of palatal bony defect with the side (P length = 1.000; P relative width = 0.262) or the severity (P length = 0.605; P relative width = 0.254) of cleft lip.Conclusions:The form of cleft lip presenting with submucous cleft palate varies, and there was no correlation with the length or relative width of palatal bony defect. Advanced imaging techniques for children with cleft lip may assist the early diagnosis of submucous cleft palate.

Psychosocial Maternal Perception of the Outcome of Pre Surgical Infant Orthopedics in Infants Born with Cleft Lip and Palate.

The setback of having a child with cleft lip and palate deeply affects the parents psychology. The Pre Surgical Infant Orthopaedics(PSIO) is a promising procedure for improvement in facial morphology prior to primary lip repair. The current study examines the perception of effects of PSIO procedures on the facial appearance of newborns with cleft lip/palate by their mothers and finds its correlation if any, with the change in psychosocial status of the mothers. The mothers of 50 infants(0-6 months) born with cleft lip/palate rated the nasal morphology, extent of cleft defect and overall facial morphology on a Likert's scale before and after PSIO procedure. This was correlated with changes in mother's depression, anxiety and stress levels by using the validated Hindi-version of Depression Anxiety and Stress scale(DASS-42) index before and after PSIO. The mothers reported a significant improvement in all the morphological parameters for their infants with PSIO which correlated well with significant reduction in the DASS scores from 22.54(severe) to 7.10(normal) for depression, 20.64(extremely severe) to 6.46(normal) for anxiety and 24.7(severe) to 8.4(normal) for stress. The changes in facial morphology by PSIO procedures are well perceived by mothers and significantly improves their depression, anxiety and stress levels.

Rehabilitation using dental implants following closure of large alveolar cleft defect by HYDIS-TB appliance

Restoration of missing oral tissues in patients with cleft lip and palate is the biggest challenge in their rehabilitation. In this report, we described the postdistraction rehabilitation and 1-year follow-up of the regenerated bone by implant-supported prosthesis and orthodontic tooth movement. A novel distraction appliance regenerated natural bone which is compatible for both implant placement and orthodontic tooth movement, thereby restoring adequate function and esthetics.

Active nasoalveolar molding using 3-directional expansion screw for arch alignment in bilateral cleft lip and palate

• A 3-D expansion screw can retract protrusive premaxilla and expand cleft arches simultaneously. • This facilitates quick premaxillary distalization in newborn with bilateral cleft lip and palate. • A separate nasal stent can thus be given early corresponding to period of increased estrogen levels. The alignment of cleft segments is the primary step in presurgical orthopedics and is more critical in the bilateral clefts, in which the arch is segmented into 3 units. The projecting premaxilla poses a great challenge to the alignment of the segmented arch because of its unrestrained forward movement. The lateral segments are often collapsed with anterior narrowing. This results in great difficulty in correctly positioning the premaxillary segment during primary cheiloplasty as there is inadequate space available to align the anterior segment between the collapsed lateral segments. The current report suggests active nasoalveolar molding using a 3-directional expansion screw for simultaneous retraction of the anterior segment and the expansion of the lateral cleft segment to provide adequate space between them to receive the retracted segment. The nasal stent can be given as a separate entity very early in the treatment as the obscuring premaxilla is quickly retracted to allow for nasal stent attachment to the alae. Thus, nasal correction can be initiated early and independently from oral corrections. A 3-dimensional model superimposition revealed retraction of 2.8 mm of the premaxilla and 3.8 mm expansion during a treatment period of 3 months. The cleft defect was reduced by 4.8 mm on the right side and 10.2 mm on the left side, with all 3 cleft segments in good alignment. Good columellar elongation was achieved with satisfactory nasal contour and symmetry.

The role of surgeon's learning on the outcomes of alveolar bone graft for cleft repair

Teaching cleft repair in the operating room may not sufficiently prepare craniofacial residents to perform the procedure self-reliantly. This study aimed to assess the influence of surgeons' experience level on the operative time and graft survival ratio after alveolar bone grafts for cleft repair. This was a retrospective review of 100 consecutive alveolar bone graft patients treated for unilateral alveolar cleft by one craniofacial surgeon between 2012 and 2019. An alveolar bone graft from the iliac bone was performed using the lateral sliding flap. Sex, age, cleft width, cleft defect volume, and operative time were recorded, and the 1-year postoperative graft survival ratio was calculated. Multiple regression analysis was performed to identify factors that affect the operative time and survival ratio. Factors affecting the operative time were the experience level of the surgeon and the presence of a cleft palate. Factors affecting the graft survival ratio were the cleft defect volume and experience level of the surgeon. In cleft lip and alveolus, the survival ratio was constant, and the operative time stabilised after approximately 20 cases. In cleft lip and palate, more experience was required to master the technique, and the operative time and survival ratio were stabilised only after approximately 40 cases. Surgeons' experience level influenced the outcomes of alveolar bone grafting. Although surgical training is currently undergoing a paradigm shift from on-the-job training to simulation-based training, clinical experience is critical to be self-sufficient.

Downregulation of WNT8A, LRP5, LRP6 and FGF8 in Malay Non-Syndromic Cleft Lip and/or Palate Patients

Non-syndromic cleft lip and/or palate (NSCL/P) is a one of the most common birth defects occurs as a result of multi-factorial determinants such as genetic and environmental factors. Genetic factor has been studying widely across different population in identifying genes causing cleft defects. This study aims to validate the role of fibroblast growth factors (FGFs) and signalling molecules Wingless-type (Wnt) to the occurrence of cleft lip and/or palate among Malay population. Tissue skin was obtained from consented NSCL/P patients who underwent the cleft lip repair operation at the upper lip skin area and non NSCL/P individual was obtained from patient having traumatic injury at the lip area as a control. Expression pattern of FGF8, FGF10, Wnt8a, and LRP5/6 were tested and validated using Western Blot (WB) and quantitative Reverse Transcriptase-PCR (qRT-PCR). The fold change difference of FGF8 (0.614 ± 0.1012-fold), FGF10 (0.7188 ± 0.1017-fold) and Wnt8a protein (0.9051 ± 0.0142-fold) was downregulated by 1-fold in cleft lip (CL) tissues compared to the normal meanwhile LRP5/6 protein (1.2201 ± 0.1404-fold) was found upregulated by 1-fold. Validation testing confirmed that expression of FGF8 (p=0.014), Wnt8a (0.0762 ± 0.0227), LRP5 (0.3577 ± 0.1362) and LRP6 (0.3093 ± 0.2541) were significantly reduced in CL tissues compared to normal. This is the first study identified defective regulation of Wnt8a, LRP5, LRP6 and FGF8 in NSCL/P among Malays. These novel findings clearly explained the important role of growth factors FGF and Wnt signalling pathway in lip and palate formation during craniofacial development.

Congenital heart disease in a cohort of Sudanese patients with cleft defects

Background and objectives:Cleft defects are among the most visible congenital defects worldwide and congenital heart disease (CHD)being one of the most common associated anomalies. This study aimed to assess the prevalence of CHDamong a cohort of Sudanese patients with cleft lip and/or palate.Patients and Methods:All patients who presented to Soba University Hospital with cleft lip and/or palate from March 2009 toMarch 2015 were included.Results:Out of 381 patients included; 168 patients (44%) had cleft lip and palate (CLP), 156 (41%) had cleft lip (CL)and isolated cleft palate (CP) was found in 57 patients (15%). Facial dysmorphic features were identifiedin 57 (15%) and cardiac defects in 42 (11%) patients. Ventricular septal defect (VSD) was diagnosed in 16patients (38%), and ASD in 12 (30.9%). Other macroscopic anomalies were identified in 57 patients (15%)and were associated with CHD (P&lt;0.001). Significant association was found between the type of cleft andCHD (P&lt;0.002), as cardiac defects were maximally observed among CP cases (21%) followed by CLPcases (13%) then CL cases (5.12%). Significant association was also found between facial dysmorphicoccurrence and CHD (P &lt; 0.001).Conclusion:CHD is a common anomaly in cleft population. The pattern of CHD is consistent with the literature withVSD being the most frequent. The cardiac defects are most prevalent in CP group. Echocardiography isjustified for screening of CHD due to the relatively higher incidence of CHD among clefts patients than ingeneral population