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OBJECTIVE: To characterize the patterns of somatic catch-up growth from infancy to adolescence in patients with cleft palate (CP). STUDY DESIGN: We assessed 474 nonsyndromic patients with isolated cleft palate (n = 69) and unilateral and bilateral cleft lip and palate (n = 271; n = 134) who underwent palatoplasty between 1988 and 2017 and had longitudinal physical growth data at birth (T0), cheiloplasty (T1), palatoplasty (T2), childhood (T3), and adolescence (T4). The z scores of weight (ZWT), height (ZHT), and body mass index (ZBMI) were compared among the CP types (isolated cleft palate, unilateral cleft lip and palate, and bilateral cleft lip and palate) and time points (T1, T2, T3, and T4). Subgroup analyses were performed to investigate the growth of patients with malnourishment (z score < -1) at T1 or T2. A generalized linear model was used to investigate the effects of gestational age and cardiac anomalies on the longitudinal changes in ZHT and ZBMI. RESULTS: Regardless of the time point, the overall ZHT, ZWT, and ZBMI approximated 0 in all CP types, indicating few differences from the mean values of noncleft children. Significant catch-up growth occurred in ZHT and ZWT from T1 to T4 for all CP types (all P < .05). Despite the recovery of ZHT and ZBMI in most patients with malnourishment, these values remain relatively low until adolescence. Patients who were born at preterm stage or had surgically repaired cardiac anomalies grew well. CONCLUSIONS: Even in infants with CP and malnutrition, preterm birth, or cardiac anomalies, rapid catch-up growth can occur prior to palatoplasty with the help of comprehensive cleft care.
Assuntos
Fenda Labial , Fissura Palatina , Desnutrição , Nascimento Prematuro , Criança , Feminino , Lactente , Humanos , Recém-Nascido , Adolescente , Fissura Palatina/complicações , Fissura Palatina/cirurgia , Fenda Labial/cirurgia , Estudos Longitudinais , Maxila , CefalometriaRESUMO
The purpose of this study was to investigate the distribution and phenotype of Goldenhar syndrome (GS) and its association with other anomalies. The samples consisted of 18 GS patients (6 males and 12 females; mean age at investigation, 7.4 ± 4.8 y) who were treated or followed up at the Department of Orthodontics, Seoul National University Dental Hospital between 1999 and 2021. The prevalence of side involvement and degree of mandibular deformity (MD), midface anomalies, and association with other anomalies were evaluated using statistical analysis. The prevalence of unilateral and bilateral MD did not differ (55.6% versus 44.4%). In unilateral MD cases, there was a tendency for higher prevalence of more severe Pruzansky-Kaban types than mild ones (type I, 10%; type IIa, 10%; type IIb, 50%; type III, 30%). Despite hypoplasia of the condyle/ramus complex, compensatory mandibular body growth occurred in 33.3% of GS patients (more severe side in bilateral MD cases, 37.5%, and ipsilateral side in unilateral MD cases, 30%). Class II molar relation was more prevalent than class I and class III molar relations (72.2% versus 11.1% versus 16.7%, P <0.01). Al total of 38.9% of patients had congenitally missing tooth. #7 facial cleft was found in 44.4% of patients. In midface anomalies, ear problem was the most common anomaly, followed by hypoplasia/absence of zygomatic arch and eye problem (88.9% versus 64.3% versus 61.1%, P <0.01). Association with the midface, spine, cardiovascular, and limb anomalies did not differ between unilateral and bilateral MD cases. These results might provide a basic guideline for diagnosis and treatment planning for GS patients.
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The purpose of this study was to classify the skeletal phenotypes of adult patients with skeletal class III (C-III) malocclusion and unilateral or bilateral cleft lip and palate using principal component analysis and cluster analysis. The samples consisted of 81 adult C-III patients with cleft lip and palate (CLP) who underwent orthognathic surgery (OGS) or distraction osteogenesis (59 males and 22 females; 50 unilateral cleft lip and palate and 31 bilateral cleft lip and palate; mean age when lateral cephalograms were taken, 22.2±4.6 y). Thirteen angular and one ratio cephalometric variables were measured. Using 4 representative variables obtained from principal component analysis (SNA, SNB, Gonial angle, and Bjork sum), K-means cluster analysis was performed to classify the phenotypes. Then, statistical analysis was conducted to characterize the differences in the variables among the clusters. Five clusters were obtained from 3 groups: severely retrusive maxilla and moderately retrusive mandible group: cluster-1 (23.5%, severely hyperdivergent pattern), cluster-4 (27.2%, moderately hyperdivergent pattern), and cluster-5 (11.1%, normodivergent pattern); moderately retrusive maxilla and normal mandible group: cluster-2 (30.9%, normodivergent pattern); normal maxilla and moderately protrusive mandible group: cluster-3 (7.4%, normodivergent pattern). Although skeletal phenotypes were diverse, distribution of sex and cleft type did not differ among 5 clusters ( P >0.05). Sixty-two percent of cleft patients showed a severely retrusive maxilla and moderately retrusive mandible (cluster-1, cluster-4, and cluster-5), which indicated that these are the main cause of skeletal C-III malocclusion in CLP patients who were treated with OGS. Therefore, it is necessary to consider presurgical orthodontic treatment and surgical planning based on the skeletal phenotypes of CLP patients.
Assuntos
Fenda Labial , Fissura Palatina , Má Oclusão Classe III de Angle , Masculino , Feminino , Humanos , Adulto , Fenda Labial/cirurgia , Fenda Labial/complicações , Fissura Palatina/cirurgia , Fissura Palatina/complicações , Análise de Componente Principal , Má Oclusão Classe III de Angle/cirurgia , Má Oclusão Classe III de Angle/etiologia , Mandíbula/cirurgia , Maxila/cirurgia , CefalometriaRESUMO
The purpose of this study was to classify and characterize facial asymmetry (FA) phenotypes in adult patients with unilateral cleft lip and palate (UCLP) and skeletal class III malocclusion. The samples comprised 52 adult UCLP patients (36 men and 16 women; mean age, 22.43 y) who had undergone orthognathic surgery for correction of class III malocclusion. After measurement of 22 cephalometric parameters in posteroanterior cephalograms taken 1 month before orthognathic surgery, principal component analysis was performed to obtain 5 representative parameters [deviation (mm) of ANS (ANS-dev), maxillary central incisor contact point (Mx1-dev), and menton (Me-dev); cant (degree) of the maxillary anterior occlusal plane (MxAntOP-cant) and mandibular border (MnBorder-cant)]. K-means cluster analysis was conducted using these representative parameters. The differences in cephalometric parameters among the clusters were statistically analyzed. The FA phenotypes were classified into 4 types: No-cant-and-No-deviation type (cluster-4, n=16, 30.8%); MxMn-cant-MxMn-dev to the cleft-side type (cluster-3, n=4, 7.7%); Mx-cant-Mn-shift to the cleft-side type (cluster-2, n=15, 28.8%); and Mn-cant-Mn-dev to the noncleft-side type (cluster-1, n=17, 32.7%). Asymmetry in the maxilla and/or mandible were observed in 70% of patients. One third of patients (cluster-2 and cluster-3; sum, 36.5%) exhibited significant cant of MxAntOP induced by cleft and cant or shift of the mandible to the cleft side. Another one third of patients (cluster-1, 32.7%) demonstrated significant deviation and cant of the mandible to the noncleft-side despite cleft in the maxilla. This FA phenotype classification might be a basic guideline for diagnosis and treatment planning for UCLP patients.
Assuntos
Fenda Labial , Fissura Palatina , Má Oclusão Classe III de Angle , Feminino , Humanos , Fenda Labial/cirurgia , Assimetria Facial/cirurgia , Fissura Palatina/cirurgia , Análise de Componente Principal , Estudos Retrospectivos , Má Oclusão Classe III de Angle/diagnóstico por imagem , Má Oclusão Classe III de Angle/cirurgia , Maxila/cirurgia , CefalometriaRESUMO
ABSTRACT: The purpose of this study was to investigate the type and frequency of use of treatment modalities (Tx-Mods) in patients with syndromic craniosynostosis (SC) using longitudinal follow-up data. A total of 28 patients with SC (24 Crouzon, 2 Apert, and 2 Antley-Bixler syndromes), who were treated at the Department of Orthodontics, Seoul National University Dental Hospital, Seoul, South Korea between 1998 and 2020, was included. According to the degree of midface hypoplasia (MH) at the initial visit (T1), the patients were divided into the mild-MH (78°≤SNA < 80°, n = 8), moderate-MH (76≤SNA < 78°, n = 7), and severe-MH (SNA < 76°, n = 13) groups. T1-age and Tx-Mods, including cal-varial surgery (CALS), orthopedic treatment (OPT), fixed orthodontic treatment, and midface advancement surgery in childhood (MAS-child) and adulthood (MAS-adult), were investigated. Complexity of MAS-adult was graded as follows: 0, no surgery; 1, orthognathic surgery; 2, distraction osteogenesis (DOG); 3, combination of distraction osteogenesis and orthognathic surgery. Then, statistical analysis was performed. Percentage distribution of Tx-Mods was 71.4% in CALS, 21.4% in MAS-child, 42.9% in OPT, 100% in fixed orthodontic treatment, and 89.3% in MAS-adult. 92.9% of patients underwent MAS more than once. The number of MAS increased according to the severity of MH ( P < 0.05). The complexity of MAS-adult increased as T1-age and severity of MH increased (all P < 0.05); whereas it decreased when CALS and OPT were performed (all P < 0.05). However, MAS in childhood did not guarantee the avoidance of additional MAS in adulthood ( P > 0.05). These findings may be used as basic guidelines for successful treatment planning and prognosis prediction in patients with SC.
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Disostose Craniofacial , Craniossinostoses , Osteogênese por Distração , Humanos , Disostose Craniofacial/cirurgia , Craniossinostoses/etiologia , Craniossinostoses/cirurgia , Seguimentos , Osteogênese por Distração/efeitos adversos , Osteotomia de Le FortRESUMO
ABSTRACT: The purpose of this study was to determine the cephalometric predictors of the future need for orthognathic surgery in patients with repaired unilateral cleft lip and palate (UCLP) using machine learning. This study included 56 Korean patients with UCLP, who were treated by a single surgeon and a single orthodontist with the same treatment protocol. Lateral cephalograms were obtained before the commencement of orthodontic/orthopedic treatment (T0; mean age, 6.3 years) and at at least of 15 years of age (T1; mean age, 16.7 years). 38 cephalometric variables were measured. At T1 stage, 3 cephalometric criteria (ANB ≤ -3°; Wits appraisal ≤ -5âmm; Harvold unit difference ≥34âmm for surgery group) were used to classify the subjects into the surgery group (nâ=â10, 17.9%) and non-surgery group (nâ=â46, 82.1%). Independent t-test was used for statistical analyses. The Boruta method and XGBoost algorithm were used to determine the cephalometric variables for the prediction model. At T0 stage, 2 variables exhibited a significant intergroup difference (ANB and facial convexity angle [FCA], all Pâ<â0.05). However, 18 cephalometric variables at the T1 stage and 14 variables in the amount of change (ΔT1-T0) exhibited significant intergroup differences (all, more significant than Pâ<â0.05). At T0 stage, the ANB, PP-FH, combination factor, and FCA were selected as predictive parameters with a cross-validation accuracy of 87.4%. It was possible to predict the future need for surgery to correct sagittal skeletal discrepancy in UCLP patients at the age of 6 years.
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Fenda Labial , Fissura Palatina , Cirurgia Ortognática , Adolescente , Cefalometria , Criança , Fenda Labial/diagnóstico por imagem , Fenda Labial/cirurgia , Fissura Palatina/diagnóstico por imagem , Fissura Palatina/cirurgia , Humanos , Aprendizado de Máquina , Estudos RetrospectivosRESUMO
Nasal retainers are common tools used in managing patients with cleft lip. The significance of nasal retainer in preventing nostril collapse or stenosis to maintain a symmetrical nose after the surgical procedures is already well known. We came up with a way to create a nasal retainer using a latex nelaton catheter. Custom-made nasal retainer using latex nelaton catheter was used postoperatively on a 10-month-old infant with median cleft lip after cheiloplasty. In postoperative day 7, her nostrils were large enough for premade silicone nasal retainer to fit. She was discharged with instructions given to use the retainer for 6 months. Custom-made nasal retainer can be used as an alternative to premade nasal retainers for patients with wide columella or small nostril cavities, or who cannot afford premade retainers.
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Fenda Labial , Látex , Catéteres , Fenda Labial/cirurgia , Feminino , Humanos , Lactente , NarizRESUMO
BACKGROUND: The purpose of this study is to evaluate the factors affecting the speech outcome following Le fort I conventional osteotomy(CO) or Le Fort I distraction osteogenesis(DO) in patients with cleft lip and palate at a single institution. METHOD: Records of cleft lip and palate patients who underwent orthognathic surgery between 2010 and 2015 were reviewed. Data included age at orthognathic surgery, sex, cleft lip and palate type, type of orthognathic surgery, the amount of maxillary advancement, and speech assessment. Speech outcomes were classified into 2 categories. Compared with the pre and post-operative Pittsburgh Weighted speech scale scores, in case that the post-operative total score is increased the authors define it as "Speech deterioration" and if not, the authors define it as "Speech preservation." RESULT: The 44 patients were identified, 33 patients underwent CO and 11 patients underwent DO. The mean age was 19.4â±â1.4. The mean period time of speech evaluation after orthognathic surgery was 1.0â±â0.46 year. The mean amount of maxillary advancement was 7.2â±â3.2âmm and show significant correlation with speech outcomes. (Pâ=â0.012) . In CO group, the patients who had the maxilla 1â¼5âmm advancement maintained their speech completely and 44% of patients with 6â¼8âmm deteriorated their speech. In DO group, patients with 9â¼10âmm maintained their speech completely, 50% of patients with 11â¼12âmm deteriorated their speech and 100% of patients with 13â¼16âmm deteriorated their speech. According to the relationship between the amount of maxillary advancement and speech outcomes, there was a statistically significant correlation in both CO and DO groups. (Pâ=â0.04, 0.029). CONCLUSION: It was found that speech of the patients with more amount of maxillary advancement tended to get worse. Also, it was observed that there exist some stable ranges of maxillary advancement for speech safety which does not effect on speech. (1â¼5âmm in CO group and 9â¼10âmm in DO group).
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Fenda Labial/cirurgia , Fissura Palatina/cirurgia , Maxila/cirurgia , Osteogênese por Distração , Osteotomia de Le Fort , Fala , Adolescente , Feminino , Humanos , Masculino , Procedimentos Cirúrgicos Ortognáticos , Resultado do Tratamento , Adulto JovemRESUMO
Wound healing is a complex mechanism involving a variety of factors and is a representative process of tissue growth and regeneration in our body. Surface-based interactions between the dressing material and the wound may significantly influence the healing phase. Advances in understanding the mechanism of wound healing have led to the development of numerous dressing materials that can accelerate the healing process. However, these materials have a passive role in wound healing. It is therefore necessary to develop novel wound dressing materials, especially effective for clinically problematic wounds. Chitosan-based dressing materials are considered suitable for clinically problematic wounds as they exhibit several characteristic features, such as facilitating hemostasis, enhanced wound healing during the inflammatory and proliferative phases, antimicrobial effect, etc. Here, we review the current status of clinically available dressing materials and studies on the biological characteristics of chitosan, and discuss the potential applications of chitosan in multi-functional dressing materials for accelarated wound healing.
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Bandagens , Quitosana , Cicatrização , Materiais Biocompatíveis , Hemostasia , HumanosRESUMO
BACKGROUND: Blood loss during cleft palate surgery has been investigated in previous research, but there is no report regarding blood loss when performing Furlow's double opposing Z-plasty (DOZ). In the present study, we evaluated intraoperative blood loss in patients with cleft palate who underwent the DOZ procedure. MATERIALS AND METHODS: Intraoperative blood loss was prospectively investigated in 59 patients undergoing palatoplasty with DOZ by a single surgeon between August 2012 and July 2013. Demographic factors and clinical status, including cleft type and palatal gap, were recorded. Blood loss was evaluated by measuring the change in weight of a suction bottle, suction line, and gauze balls. RESULTS: Mean blood loss was 16.61 ± 10.33 mL, which accounted for 5% of total blood volume. Male sex, older age, severe cleft type, larger palatal gap, relaxed incision, and increased operative time contributed to greater blood loss. The amount of intraoperative bleeding could be predicted by the following equation: Blood loss = -5.64 + 6.18 (male patients) + 7.58 (severe type cleft) + 0.88 X age (months) + 0.84 X palatal gap (mm). CONCLUSIONS: We found that the DOZ technique causes mild blood loss, but bleeding amount tended to increase in older male patients with a severe cleft and a larger palatal gap. The use of relaxed incisions during palatoplasty and prolonged operation times also contributed to greater blood loss.
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Perda Sanguínea Cirúrgica/estatística & dados numéricos , Fissura Palatina/cirurgia , Procedimentos Cirúrgicos Bucais/métodos , Procedimentos de Cirurgia Plástica/métodos , Feminino , Humanos , Lactente , Masculino , Duração da Cirurgia , Estudos Prospectivos , Fatores de RiscoRESUMO
Hyaluronic acid (HAc) hydrogel exhibits excellent biocompatibility, but it has limited biomedical application due to its poor biomechanical properties as well as too-fast enzymatic degradation. In this study, we have developed an in situ precipitation process for the fabrication of a HAc-calcium phosphate nanocomposite hydrogel, after the formation of the glycidyl methacrylate-conjugated HAc (GMHA) hydrogels via photo-cross-linking, to improve the mechanical and biological properties under physiological conditions. In particular, our process facilitates the rapid incorporation of calcium phosphate (CaP) nanoparticles of uniform size and with minimal agglomeration into a polymer matrix, homogeneously. Compared with pure HAc, the nanocomposite hydrogels exhibit improved mechanical behavior. Specifically, the shear modulus is improved by a factor of 4. The biostability of the nanocomposite hydrogel was also significantly improved compared with that of pure HAc hydrogels under both in vitro and in vivo conditions.
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Materiais Biocompatíveis/síntese química , Hidrogéis/síntese química , Nanocompostos/química , Animais , Materiais Biocompatíveis/efeitos adversos , Materiais Biocompatíveis/química , Fosfatos de Cálcio/química , Linhagem Celular , Ácido Hialurônico/química , Hidrogéis/efeitos adversos , Hidrogéis/química , Camundongos , Nanocompostos/efeitos adversosRESUMO
The purpose of this study was to investigate the effect of secondary alveolar bone grafting (SABG) on the maxillary growth in patients with unilateral (UCLP) and bilateral cleft lip and palate (BCLP). The samples consisted of 40 Korean boy cleft patients who had the similar initial skeletal characteristics and were treated with the identical treatment protocol. They were divided into UCLP group (N = 25; mean SABG age, 9.9 years; mean follow-up duration, 42.3 months) and BCLP group (N = 15; mean SABG age, 10.2 years; mean follow-up duration, 40.6 months). In the lateral cephalograms taken 1 month before (T1) and at least 2 years after SABG (T2), cephalometric variables were measured. At T1 stage, the 2 groups did not exhibit significant differences in the cephalometric variables except posterior maxillary height (P-HRP) (P < 0.05). At T2 stage, both groups exhibited the reduced sagittal growth (UCLP, ANB, P < 0.001; AB to facial plane angle (AB-FPA), P < 0.01; BCLP, A to N perpendicular, P < 0.05; ANB and AB-FPA, P < 0.001) and the undisturbed vertical growth (A-HRP and P-HRP, all P < 0.001) of the maxilla. During T1 to T2, BCLP group experienced more aggravation of Class III skeletal pattern than UCLP group (ΔAB-FPA, P < 0.05). There, however, were no differences in the amounts of changes in the maxillary vertical position and mandibular plane angulation between the 2 groups. Two-stage SABG procedure subgroup in patients with BCLP demonstrated a more retrusive maxilla compared with 1-stage SABG procedure subgroup (ΔSNA, P < 0.05). Patients with BCLP, especially who underwent 2-stage SABG procedure, might have a possibility of poor sagittal growth of the maxilla compared with patients having UCLP.
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Enxerto de Osso Alveolar/métodos , Fenda Labial/cirurgia , Fissura Palatina/cirurgia , Maxila/crescimento & desenvolvimento , Pontos de Referência Anatômicos/patologia , Cefalometria/métodos , Criança , Fenda Labial/fisiopatologia , Fissura Palatina/fisiopatologia , Seguimentos , Humanos , Estudos Longitudinais , Masculino , Má Oclusão Classe III de Angle/etiologia , Má Oclusão Classe III de Angle/patologia , Mandíbula/crescimento & desenvolvimento , Mandíbula/patologia , Maxila/patologia , Osso Nasal/patologia , Retrognatismo/etiologia , Retrognatismo/patologia , Estudos Retrospectivos , Sela Túrcica/patologia , Dimensão VerticalRESUMO
The purpose of this study was to determine the cephalometric variables that can predict the future need for orthognathic surgery or distraction osteogenesis in Korean male patients with nonsyndromic cleft lip and alveolus (CLA) and unilateral (UCLP) and bilateral cleft lip and palate (BCLP). A total of 131 patients who were treated by one surgeon and one orthodontist using identical protocol were divided into CLA group (n = 35), UCLP group (n = 56), and BCLP group (n = 40). Lateral cephalograms were taken before secondary alveolar bone graft (T0; mean age, 9.3 years) and at the minimum of 15 years of age (T1; mean age, 17.3 years). The cephalometric variables of these cephalograms were measured. At T1 stage, 3 cephalometric criteria were used to divide the subjects into surgery and nonsurgery groups (ANB ≤ -3 degrees; Wits appraisal ≤ -5 mm; Harvold unit difference ≥ 34 mm for surgery group). The feature wrapping method was used to determine the cephalometric variables at T0 stage for a prediction model. At T1 stage, 27 (20.6%) of 131 subjects required surgical intervention to correct their sagittal skeletal discrepancies. Frequency was significantly different among the CLA, UCLP, and BCLP groups (8.5%, 21.4%, and 30.0%, respectively; P < 0.05; [CLA, UCLP]â<â[UCLP, BCLP]). A total of 10 cephalometric variables of T0 stage were selected as predictors, and weighted classification accuracy was 77.3%. The frequency of surgical intervention increased with cleft severity. Ten cephalometric variables might be regarded as effective predictors of the future need for surgery to correct their sagittal skeletal discrepancies.
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Enxerto de Osso Alveolar/métodos , Fenda Labial/cirurgia , Fissura Palatina/cirurgia , Tomada de Decisões , Cirurgia Ortognática/métodos , Osteogênese por Distração/métodos , Adolescente , Cefalometria , Criança , Fenda Labial/diagnóstico , Fissura Palatina/diagnóstico , Humanos , Masculino , PrognósticoRESUMO
BACKGROUND: The aim of this research was to investigate whether covering of the distracted cranial bone segment with a polycaprolactone (PCL) membrane (guided bone formation) would accelerate the process of osteogenesis in high-speed distraction of adult rabbit. METHODS: Eighteen 24-weeks old, 3.0 to 3.5 âkg, male New Zealand rabbits underwent routine gradual cranium distraction (group 1), distraction at high speed without the membrane (group 2), and distraction at high speed with a PCL membrane covering the cranium at the distraction gap (group 3). Five days after the cranial osteotomy, the distraction process was initiated at 3â mm per day (1.5 âmm twice a day) in group 2 and group 3, and 1â mm per day (0.5 âmm twice a day) in group 1 until 10 âmm of length gain was achieved. At the consolidation 4 and 6 weeks, the bone mineral density was analyzed by a computerized tomography imaging protocol. The bone formation ratio of each group was compared with Hematoxylin and Eosin stain. The collagen formation of each group was analyzed by Massons' trichrome staining. RESULTS: Radiographic imaging and quantitative data indicated a significant increase in bone mineral density in group 1 and group 3 compared with group 2. Bone formation ratio in histologic analysis showed an increase in group 1 and group 3. Collagen synthesis in group 2 was significantly increased in distraction gap. In group 3, collagen fibers were significantly decreased underneath the PCL membrane. CONCLUSIONS: Polycaprolactone membrane covering the bone distraction gap provides an environment for faster bone formation in high-speed distraction.
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Mandíbula/cirurgia , Osteogênese por Distração/métodos , Osteogênese/fisiologia , Osteotomia/métodos , Poliésteres , Crânio/cirurgia , Animais , Densidade Óssea , Masculino , CoelhosRESUMO
The purposes of this study were to find a novel mutation of FGFR2 in Korean Crouzon syndrome patients and to identify the functional consequences of this mutation. The samples consisted of 16 Crouzon patients. Peripheral venous blood was collected from the patients. FGFR2 mutation screening was performed by direct PCR sequencing of all exons and part of the introns. Restriction fragment length polymorphism (RFLP) analysis was performed to confirm the novel mutation. For functional studies, we performed luciferase assay for Runx2 transcriptional activity, real-time PCR for the bone markers (osteocalcin and alkaline phosphatase), and Western blot for phosphorylated FGFR2 and ERK1/2-MAPK protein. Among 16 patients, 10 showed FGFR2 mutations that had already been reported elsewhere. A novel FGFR2 mutation associated with tyrosine kinase II (TK-II) domain, L617F, was found in one Crouzon syndrome patient by direct PCR sequencing. Presence of this mutation was confirmed using RFLP analysis. Runx2 transcriptional activity and expression of osteocalcin and alkaline phosphatase significantly increased in L617F-transfected cells compared to wild-type cells. FGFR2 autophosphorylation in L617F-transfected cells increased in 1% serum, but ERK1/2-MAPK protein was not activated. The FGFR2-L617F mutation associated with the TK domain is potentially related to premature suture closure in Crouzon syndrome patient.
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Disostose Craniofacial/genética , Mutação , Proteínas Tirosina Quinases/metabolismo , Receptor Tipo 2 de Fator de Crescimento de Fibroblastos/genética , Fosfatase Alcalina/genética , Fosfatase Alcalina/metabolismo , Pré-Escolar , Subunidade alfa 1 de Fator de Ligação ao Core/genética , Subunidade alfa 1 de Fator de Ligação ao Core/metabolismo , Disostose Craniofacial/etiologia , Face/anormalidades , Feminino , Humanos , Masculino , Maxila/anormalidades , Osteocalcina/genética , Osteocalcina/metabolismo , Fosforilação , Polimorfismo de Fragmento de Restrição , Estrutura Terciária de Proteína , Receptor Tipo 2 de Fator de Crescimento de Fibroblastos/metabolismoRESUMO
The purpose of this study was to analyze the statistical association between variable clinical phenotypes of the orbit, mandible, ear, nerve, soft tissue (OMENS) classification system in Oriental patients with hemifacial microsomia (HFM) and compare the data of Oriental children with data from other Western centers. One hundred consecutive patients with HFM referred to the Seoul National University Children Hospital were chosen between March 2009 and April 2010. They were classified according to the OMENS classification system, and statistical analyses were performed. The total OMENS scores were correlated significantly with all 5 component scores. In comparison with the previous data of Western centers, the orbit and ear components had statistically higher severity. Our retrospective study in Oriental children demonstrated that the pathophysiology of HFM is a complex process and that the components of HFM have a close relationship. For the ethnic difference, further etiologic studies on the molecular basis are required.
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Povo Asiático , Síndrome de Goldenhar/classificação , Síndrome de Goldenhar/etnologia , Fenótipo , Índice de Gravidade de Doença , Feminino , Humanos , Masculino , República da Coreia , Estudos Retrospectivos , Estatísticas não ParamétricasRESUMO
The purpose of this study was to statistically analyze mandibular growth disturbance in the Oriental cleft population and compare this with that of non-cleft children through cephalometric analysis. Thirty-six children with cleft palates, repaired using a modification of Furlow double opposing Z-plasty, were chosen. Comparative analyses among 3 types of cleft were performed. Statistical analyses of 8 linear and angular measurements were performed in cleft patients and the non-cleft population using Fisher Z-transformation. Comparative analysis showed no significant difference among the 3 types of cleft. In the Oriental cleft group treated with modified double opposing Z-plasty, the spatial position of the mandible showed significant differences compared with the non-cleft group. A backward inclination of the anterior surface of the mandible and downward rotation of the mandibular body were identified. Some of our results regarding gonial angle and length of the mandibular body conflicted with previous Western studies.
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Fenda Labial/cirurgia , Fissura Palatina/cirurgia , Mandíbula/crescimento & desenvolvimento , Povo Asiático , Criança , Pré-Escolar , Feminino , Humanos , Masculino , Procedimentos de Cirurgia Plástica/métodos , Estudos RetrospectivosRESUMO
One of the important clinical findings in hemifacial microsomia (HFM) is facial asymmetry (FA) with unilateral underdevelopment and malformation of the mandible and/or maxilla. Unilateral distraction osteogenesis (UDOG) of the mandible has been used for correction of FA in HFM patients. However, return to the original FA status often occurs because of contraction of the distracted bony segment of the mandible and insufficient compensatory downward growth and skeletal midline correction of the maxilla on the affected side. Although bimaxillary UDOG procedure in the maxilla with Le Fort I osteotomy and the mandible with ramus osteotomy was suggested as an alternative treatment modality, it has some disadvantages including elongation of the face, inaccuracy of vector control, and less predictable results. Occlusal plane canting, skeletal midline deviation of the maxilla, and unilateral mandibular hypoplasia might be simultaneously corrected by fixation of the maxilla into a proper position after Le Fort I osteotomy and UDOG of the mandible. Three-dimensional virtual simulation using three-dimensional computed tomography data could help to accurately predict surgical outcomes. In addition, transarch elastic traction from the orthodontic mini-implants on the unaffected side of the mandible to the maxillary posterior teeth on the affected side can produce a plastic molding of the regenerated bone in the distraction area of the mandible and induce compensatory dentoalveolar downward development of the maxillary posterior teeth. Therefore, the purpose of this article was to introduce a new approach for the surgico-orthodontic treatment consisting of simultaneous maxillary fixation and unilateral mandibular distraction with transarch elastic traction in HFM patients.
Assuntos
Assimetria Facial/terapia , Ortodontia Corretiva/métodos , Osteogênese por Distração/métodos , Pré-Escolar , Terapia Combinada , Assimetria Facial/diagnóstico por imagem , Humanos , Fixadores Internos , Masculino , Mandíbula/diagnóstico por imagem , Mandíbula/cirurgia , Maxila/diagnóstico por imagem , Maxila/cirurgia , Ortodontia Corretiva/instrumentação , Osteogênese por Distração/instrumentação , Osteotomia de Le Fort , Tomografia Computadorizada por Raios X , Resultado do TratamentoRESUMO
BACKGROUND: Bone grafts can provide an optimal environment for permanent tooth to erupt and enhance the stability of the alveolar maxilla. Although autologous bone is an optimal source for osteogenesis, its inevitable donor site morbidity has led to active research on bone substitutes. This study was designed to evaluate the safety and feasibility of using biphasic calcium phosphate (BCP; Osteon) as a bone substitute in dogs. METHODS: Bilateral third and fourth premolars of four 15-week-old mongrel dogs were used. All teeth were extracted except the third premolar of the right mandible, which was used as a control. After extraction of the premolars, each dog was administered BCP (Osteon), demineralized bone matrix (DBM; DBX), and no graft in the hollow sockets of the right fourth premolar, left fourth premolar, and left third premolar, respectively. Radiographs were taken at 2-week intervals to check for tooth eruption. After 8 weeks, each dog was sacrificed, and tooth and bone biopsies were performed to check for the presence of tooth and bone substitute particle remnants. RESULTS: Four weeks after the operation, permanent tooth eruptions had started at all the extraction sites in each dog. Eight weeks after the operation, all teeth had normally erupted, and histological examination revealed BCP particles at the right fourth premolar. CONCLUSION: In all four dogs, no delay in the eruption of the teeth or shape disfigurement of permanent teeth was observed on gross inspection and radiologic evaluation. On histological examination, most of the BCP and DBM were replaced by new bone. Bone substitutes can be used as graft materials in patients with alveolar clefts.
RESUMO
OBJECTIVE: To investigate the cephalometric predictors of the future need for orthognathic surgery in Korean patients with unilateral cleft lip and palate (UCLP) despite long-term use of facemask with miniplate (FMMP). METHODS: The sample consisted of 53 UCLP patients treated by a single orthodontist using an identical protocol. Lateral cephalograms were taken before commencement of FMMP therapy (T0; mean age, 10.45 years), after FMMP therapy (T1; mean age, 14.72 years), and at follow-up (T2; mean age, 18.68 years). Twenty-eight cephalometric variables were measured. At T2 stage, the subjects were divided into FMMP-Nonsurgery (n = 33, 62.3%) and FMMP-Surgery (n = 20, 37.7%) groups according to cephalometric criteria (point A-nasion-point B [ANB] < -3°; Wits-appraisal < -5 mm; and Harvold unit difference [HUD] > 34 mm for FMMP-Surgery group). Statistical analyses including discrimination analysis were performed. RESULTS: In FMMP-Surgery group, the forward position of the mandible at T0 stage was maintained throughout the whole stages and Class III relationship worsened with significant growth of the mandibular body and ramus and counterclockwise rotation of the maxilla and mandible at the T1 and T2 stages. Six cephalometric variables at T0 stage including ANB, anteroposterior dysplasia indicator, Wits-appraisal, mandibular body length, HUD, and overjet were selected as effective predictors of the future need for surgical intervention to correct sagittal skeletal discrepancies. CONCLUSIONS: Despite long-term use of FMMP therapy, 37.7% of UCLP patients became candidates for orthognathic surgery. Therefore, differential diagnosis is necessary to predict the future need for orthognathic surgery at early age.