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Cleft Lip

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2641. Palate morphology after unilateral and bilateral cleft lip and palate closure. (Abstract)

Palate morphology after unilateral and bilateral cleft lip and palate closure. The aim of the present study was to compare the morphology of the hard palate of patients with uni- and bilateral cleft lip and palate after palatoplasty using vomer and palatal pedicled flaps with the palatal morphology of non-cleft individuals. Eighty patients were enrolled into this retrospective study: 40 patients with cleft lip and palate (30 unilateral, 10 bilateral) and 40 non-cleft patients with class I (...) occlusion, who served as controls. Analysis of the development of the maxillary arch and evaluation of palatal morphology were accomplished from reformatted CT scans from plaster casts of the maxilla at the age of 4, 10 and 15 years (cleft patients) and 10 years (controls). Width and symmetry of the maxillary arch and morphology of the hard palate were assessed in the canine and molar region and compared both among the cleft groups and the controls. Maxillary arch width as assessed from plaster casts

2006 International Journal of Oral and Maxillofacial Surgery

2642. Influence of season on prevalence of cleft lip and palate. (Abstract)

Influence of season on prevalence of cleft lip and palate. According to the multifactorial threshold (MFT) model, cleft lip and palate (CLP) result from the influence of exogenous and endogenous genetic factors. Based on nearly 1000 cases of cleft lip with or without cleft palate treated in the clinic for maxillofacial surgery of the Martin-Luther University Halle-Wittenberg during the period 1946-1995, we investigated the influence of season on the occurrence of this malformation. Seasons (...) of the year reflect various exogenous factors that may be accessible to prevention. The calculated date of conception served as comparison value between the study group and controls. The clefts of type III (CLP; P = 0.48), isolated CP (P = 0.933) or male patients with clefts of type I showed no significant deviation from the distribution in the control group over the year. We proved a significant maximum in spring, summer and autumn and a minimum in winter for the conception date of female patients

2006 International Journal of Oral and Maxillofacial Surgery

2643. A study of PVRL1 mutations for non-syndromic cleft lip and/or palate among Taiwanese patients. (Abstract)

A study of PVRL1 mutations for non-syndromic cleft lip and/or palate among Taiwanese patients. Mutations of codons 185 and 323, especially the W185X mutation, of the PVRL1 gene among non-syndromic cleft lip and/or palate (CL/P) patients and normal controls in Taiwan were studied in order to determine whether there are mutations that play a part in the formation of non-syndromic CL/P. A total of 76 patients were enrolled; 66 sporadic non-syndromic CL/P patients and 10 normal controls

2006 International Journal of Oral and Maxillofacial Surgery

2644. Effect of ENU (ethylnitrosourea) mutagenesis in cleft lip and/or palate pathogenesis in mice. (Abstract)

Effect of ENU (ethylnitrosourea) mutagenesis in cleft lip and/or palate pathogenesis in mice. Cleft lip and/or palate (CL/P) are caused by many factors. The aim of this study was to investigate the effects of genetic point mutations in CL/P pathogenesis. ICR and AJ strain mice were used. Ethylnitrosourea (ENU) was injected into 10-week-old male mice (G0) intraperitoneally at a dose of 250 mg/kg. The males were bred with two untreated virgin females of the same strain on day 100 after injection (...) . The uterine contents (G1) of one female were examined on day 18.5 of pregnancy. From the other female, the offspring were delivered naturally, and F3 mice (G3) were also examined in the same way. In ICR strain mice, cleft palate only (CPO) was increased in both the G1 and G3. The frequency was significantly higher in the G3 than the G1 generation. Cleft lip was not observed. In AJ strain mice, CL/P increased in both the G1 and G3. In the G3, the frequency of CPO was increased significantly. Genes related

2005 International Journal of Oral and Maxillofacial Surgery

2645. Patient evaluation of outcomes of external rhinoplasty for unilateral cleft lip and palate. (Abstract)

Patient evaluation of outcomes of external rhinoplasty for unilateral cleft lip and palate. Thirty-five patients (range 16-59 years) with cleft-lip nasal deformity treated by external rhinoplasty were evaluated for satisfaction and perception of outcomes. Treatment involved alar base relocation and augmentation of the asymmetric nasal tip with auricular cartilage grafts. The patients completed a satisfaction survey and interview at the 2-year follow-up visit. A visual analogue scale (VAS (...) of nostrils (6.73). No patients suffered long-term pain for more than 2 months following surgery. All patients were prepared to undergo such procedure for a second time, if necessary. The unilateral cleft-lip nasal deformity can be improved in the eyes of the patient, using the combination of external rhinoplasty with alar base relocation, where necessary, and auricular cartilage augmentation of the nasal tip.

2006 International Journal of Oral and Maxillofacial Surgery

2646. Long-term evaluation of bilateral cleft lip and palate patients after early secondary closure and premaxilla repositioning. (Abstract)

Long-term evaluation of bilateral cleft lip and palate patients after early secondary closure and premaxilla repositioning. The aim of this retrospective, mixed longitudinal study was to assess the long-term outcome of early secondary closure and premaxilla osteotomy in 40 bilateral cleft lip and palate patients who underwent early secondary osteotomy of the premaxilla and bone grafting at the age of 8-12 years. Clinical and cephalometric evaluations of profile, lip relation, nasolabial angle (...) and position of the maxilla preoperatively, postoperatively and at adolescence were compared to normal values of non-cleft individuals and the reported data of 90 bilateral cleft lip and palate patients treated in Oslo. In 68% of patients the profile was considered acceptable, but in 26 maxillary growth appeared to be impaired by cephalometric standards. In four patients a Le Fort I osteotomy was carried out and nine patients would have benefited from such a procedure. This study reveals a trend towards

2007 International Journal of Oral and Maxillofacial Surgery

2647. A 2-stage procedure combining maxillary advancement by distraction technique with mandibular setback surgery in patients with cleft lip and palate. (Abstract)

A 2-stage procedure combining maxillary advancement by distraction technique with mandibular setback surgery in patients with cleft lip and palate. A 2-stage procedure combining maxillary advancement by distraction technique with mandibular setback surgery was used to correct jaw deformities in 5 patients with severe maxillary retrusion secondary to cleft lip and palate. First, a Le Fort I maxillary osteotomy was performed. Immediately after maxillary distraction, the distraction device

2006 International Journal of Oral and Maxillofacial Surgery

2648. Neuroprotective peptides prevent some alcohol-induced alteration in gamma-aminobutyric acid A-beta3, which plays a role in cleft lip and palate and learning in fetal alcohol syndrome. (Abstract)

Neuroprotective peptides prevent some alcohol-induced alteration in gamma-aminobutyric acid A-beta3, which plays a role in cleft lip and palate and learning in fetal alcohol syndrome. Prenatal alcohol exposure affects 1 in 100 births in the United States and results in craniofacial dysmorphologic condition and learning disabilities. In a model for fetal alcohol syndrome, neuroprotective peptides prevented fetal death and learning deficits. The gamma-aminobutyric acid A (GABA) receptor subunit (...) was prevented by the peptides (P = .01). GABAbeta3 was higher in alcohol treated adult brains respect to the controls (P = .002); this rise was not prevented by the peptides.Treatment with the neuropeptides NAPVSIPQ and SALLRSIPA prevented the alcohol-induced decline in GABAbeta3 expression 10 days after alcohol exposure. Because palate formation continues through E18, NAPVSIPQ and SALLRSIPA may be beneficial for the prevention of cleft lip and palate.

2007 American Journal of Obstetrics and Gynecology

2649. Prevalence of cleft lip and palate in a hospital-based population in the Sudan. (Abstract)

Prevalence of cleft lip and palate in a hospital-based population in the Sudan. Cleft lip and palate represents one of the most common developmental deformities seen in oral surgery clinics. It is usually associated with problems which include not only cosmetic and dental abnormalities, but also speech, hearing and facial growth difficulties.The purpose of the present study was to determine the prevalence of cleft lip and palate in births taking place in hospitals in the Sudan.The records (...) of 15,890 Sudanese new-borns delivered at three hospitals during the period from 1997 to 2000 were examined.Thirteen cases of cleft lip and palate were found, demonstrating a prevalence of 0.9 per 1000. There were more girls than boys, with a male:female ratio of 3:10. Fifty-four per cent of the cases had cleft lip with cleft palate, 30% had only cleft palate and the remaining 16% had cleft lip alone.The present study was confined to hospital-based births in one city, and the true incidence of cleft lip

2005 International Journal of Paediatric Dentistry

2650. The self-concept of adolescents with cleft lip and palate: a pilot study using a multidimensional/hierarchical measurement instrument. Full Text available with Trip Pro

The self-concept of adolescents with cleft lip and palate: a pilot study using a multidimensional/hierarchical measurement instrument. To pilot the use of a multidimensional/hierarchical measurement instrument called the self-description questionnaire II to determine whether specific areas of self-concept in a group of adolescents with cleft lip and palate would be affected by their condition when compared with a normative sample.The self-concept of 23 adolescents with a cleft of the lip (...) ) and Physical Appearance (P < 0.01) self-concepts. These differences were in a positive direction. Global self-concept as measured by the General Self scale was not significantly different from the normative sample.These results suggest that adolescents with clefts of the lip and palate have normative if not better self-concept than their peers. The study also suggests that having a cleft of the lip and palate has specific rather than broad associations with psychosocial adjustment. This justifies the use

2006 International Journal of Paediatric Dentistry

2651. Linkage disequilibrium between MSX1 and non-syndromic cleft lip/palate in the Chilean population. (Abstract)

Linkage disequilibrium between MSX1 and non-syndromic cleft lip/palate in the Chilean population. Non-syndromic cleft lip/palate (NSCLP) is a complex genetic trait. Linkage and association studies have suggested that a clefting locus could be located on chromosome 4p. Sixty Chilean families were recruited for this study; from these, we used unrelated trios to evaluate the possible linkage disequilibrium between MSX1 and NSCLP. An intragenic marker, MSX1-CA, and an extragenic marker, D4S432

2004 Journal of Dental Research

2652. Disruption of an AP-2alpha binding site in an IRF6 enhancer is associated with cleft lip. Full Text available with Trip Pro

Disruption of an AP-2alpha binding site in an IRF6 enhancer is associated with cleft lip. Previously we have shown that nonsyndromic cleft lip with or without cleft palate (NSCL/P) is strongly associated with SNPs in IRF6 (interferon regulatory factor 6). Here, we use multispecies sequence comparisons to identify a common SNP (rs642961, G>A) in a newly identified IRF6 enhancer. The A allele is significantly overtransmitted (P = 1 x 10(-11)) in families with NSCL/P, in particular those (...) with cleft lip but not cleft palate. Further, there is a dosage effect of the A allele, with a relative risk for cleft lip of 1.68 for the AG genotype and 2.40 for the AA genotype. EMSA and ChIP assays demonstrate that the risk allele disrupts the binding site of transcription factor AP-2alpha and expression analysis in the mouse localizes the enhancer activity to craniofacial and limb structures. Our findings place IRF6 and AP-2alpha in the same developmental pathway and identify a high-frequency

2008 Nature Genetics

2653. Craniofacial computerized tomography analysis of the midface of patients with repaired complete unilateral cleft lip and palate. Full Text available with Trip Pro

Craniofacial computerized tomography analysis of the midface of patients with repaired complete unilateral cleft lip and palate. Computerized tomography (CT) makes regional anatomy available as a series of cross-sectional axial images that can be analyzed unobstructed by other anatomic features. In this study, we aimed to analyze the maxillary dentoalveolar and deeper structures in patients with surgically repaired complete unilateral cleft lip and palate (CLP) and to investigate whether (...) , respectively, were compared by using the paired t test.Significant reduction of 19.05% in the nasal chamber width (2.54 +/- 1.49 mm; P <0.001), more distal position of the bony alar base (2.28 +/- 1.71 mm; P <0.001), and the sagittal position of the most prominent anterolateral point on the maxillary alveolar process (1.62 +/- 1.85 mm; P <0.01) were noted on the cleft side. The lower half of the bony nasal septum was consistently deviated toward the cleft side, and the nasal tip was deviated

2008 American journal of orthodontics and dentofacial orthopedics

2654. Evaluation of 3-dimensional tooth crown size in cleft lip and palate patients. (Abstract)

Evaluation of 3-dimensional tooth crown size in cleft lip and palate patients. The aims of this study were to evaluate 3-dimensional (3D) tooth crown sizes in patients with cleft lip and palate (CLP) and to compare them with those of a Class I control group.Orthodontic dental casts were used of 72 subjects with CLP (20 bilateral [BCLP], 34 unilateral left [ULCLP], 18 unilateral right [URCLP]) and 53 adolescents in the permanent dentition stage with Class I occlusion. Mesiodistal (MD (...) were smaller when compared with other CLP groups (P <0.001). In general, all LL and OG measurements were smaller in the CLP groups than in the Class I group in both dental arches.In general, MD, LL, and OG dimensions of CLP patients were smaller than those of the Class I subjects, not only in the affected maxillary dental arch, but also in the mandibular dental arch. Variations in 3D tooth dimensions were found among all CLP types. The lateral incisor in the cleft region was the smallest. A 3D

2008 American journal of orthodontics and dentofacial orthopedics

2655. Genomic screening identifies novel linkages and provides further evidence for a role of MYH9 in nonsyndromic cleft lip and palate. Full Text available with Trip Pro

Genomic screening identifies novel linkages and provides further evidence for a role of MYH9 in nonsyndromic cleft lip and palate. Nonsyndromic cleft lip with or without cleft palate (NSCLP) is a common birth anomaly that requires prolonged multidisciplinary rehabilitation. Although variation in several genes has been identified as contributing to NSCLP, most of the genetic susceptibility loci have yet to be defined. To identify additional contributory genes, a high-throughput genomic scan (...) not identify a single high-risk haplotype. Our results confirm a previous report that 8q21.3-24.12 may harbor a clefting gene and identify 22q12.2-12.3 as a new candidate region that contains MYH9. Most importantly, we confirm the previous report of an association with MYH9.

2008 European Journal of Human Genetics

2656. Nasolabial cyst in a patient with cleft lip and palate. (Abstract)

Nasolabial cyst in a patient with cleft lip and palate. Nasolabial cysts are rare non-odontogenic cysts that occur beneath the ala nasi, and debate about their complicated, unique pathogenesis continues. It is widely accepted that these lesions originate from the anlage of the nasolacrimal duct; however, some still think that nasolabial cysts arise from fissural cysts. The authors report a patient with a nasolabial cyst who also had a unilateral cleft lip and palate. This unusual finding may

2008 International Journal of Oral and Maxillofacial Surgery

2657. Two novel TP63 mutations associated with the ankyloblepharon, ectodermal defects, and cleft lip and palate syndrome: a skin fragility phenotype. Full Text available with Trip Pro

Two novel TP63 mutations associated with the ankyloblepharon, ectodermal defects, and cleft lip and palate syndrome: a skin fragility phenotype. Ankyloblepharon, ectodermal defects, and cleft lip and palate (AEC) syndrome is a rare autosomal dominant disorder caused by mutations in the sterile alpha motif region of TP63, a homologue of the tumor suppressor TP53. Recent structure-function studies have identified complexities in the genotype-phenotype correlation of the p63 syndromes.We report 2

2005 Archives of Dermatology

2658. Two- and three-dimensional sonographic assessment of the fetal face. 2. Analysis of cleft lip, alveolus and palate. (Abstract)

Two- and three-dimensional sonographic assessment of the fetal face. 2. Analysis of cleft lip, alveolus and palate. To describe the sonographic appearance of cleft lip with or without cleft palate (CL +/- P) using two-dimensional and three-dimensional (3D) ultrasound imaging. Also, to evaluate the accuracy of ultrasound to delineate with precision the bony extent of facial clefts, i.e. to differentiate clefts limited to the lips, or extending to the alveolus/premaxilla or the secondary (...) palate.This was a retrospective study based on the examination of fetuses diagnosed with an isolated CL +/- P. Cases included were either discovered at systematic screening or referred for further investigation. Clefts were characterized by their precise anatomical location and extent. The defect could include a cleft lip (CL), a cleft alveolus (CA), or a cleft of the secondary palate (CSP).We analyzed 96 cases of CL +/- P. The mean gestational age at examination was 28.2 +/- 4.1 weeks. The sonographic

2004 Ultrasound in Obstetrics and Gynecology

2659. Craniofacial variability index determined by three-dimensional ultrasound in isolated vs. syndromal fetal cleft lip/palate. (Abstract)

Craniofacial variability index determined by three-dimensional ultrasound in isolated vs. syndromal fetal cleft lip/palate. This study was undertaken to employ craniofacial pattern profile analysis in fetal facial clefts and to evaluate the craniofacial variability index (CVI) in distinguishing between isolated and syndromal clefts.Three-dimensional (3D) sonographic assessment of 16 different fetal craniofacial measurements was performed in each of eight pregnancies complicated by an isolated (...) abnormal Z-scores and a higher mean CVI were found in the group with more severe (bilateral) facial clefts. Most abnormal values were found in the facial width measurements. Syndromal cleft lip/palate was associated with significantly more abnormal Z-scores and a higher mean CVI than isolated cleft lip/palate (P < 0.05).Craniofacial variability index may be a valuable tool for distinguishing between isolated and syndromal fetal cleft lip/palate.

2007 Ultrasound in Obstetrics and Gynecology

2660. Study of the cephalometric features of parents of children with cleft lip and/or palate anomaly. (Abstract)

Study of the cephalometric features of parents of children with cleft lip and/or palate anomaly. The purpose of this retrospective case-control study was to compare the cephalometric features of parents of children with cleft anomalies to those of parents of normal children in the hope of finding potential markers of predisposition for this condition. There were 22 sets of parents of cleft children (study group) and 22 sets of parents of normal children (control group). A total of 88 lateral (...) ) in study group mothers was larger than in control group mothers. In conclusion, the craniofacial morphology of the parents of children with cleft anomalies differs from that of parents of normal children and may have some predictive value.

2007 International Journal of Oral and Maxillofacial Surgery

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