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Expression profiles of cIRF6, cLHX6 and cLHX7 in the facial primordia suggest specific roles during primary palatogenesis.

BACKGROUND: The LIM-homeodomain transcription factors LHX7 and LHX6 have been implicated in palatogenesis in mice and thus may also contribute to the incidence of isolated palatal clefts and/or clefts of the lip and primary palate (CL/P) in humans. Causative mutations in the transcription factor IRF6 have also been identified in two allelic CL/P syndromes and common polymorphisms in the same gene are significantly associated with non-syndromal CL/P in different populations. RESULTS: Here we report the isolation of chick orthologues of LHX7, LHX6 and IRF6 and the first characterisation of their profiles of expression during morphogenesis of the midface with emphasis on the period around formation of the primary palate. LHX7 and LHX6 expression was restricted to the ectomesenchyme immediately underlying the ectoderm of the maxillary and mandibular primordia as well as to the lateral globular projections of the medial nasal process, again underlying the pre-fusion primary palatal epithelia. In contrast, IRF6 expression was restricted to surface epithelia, with elevated levels around the frontonasal process, the maxillary primordia, and the nasal pits. Elsewhere, high expression was also evident in the egg tooth primordium and in the apical ectodermal ridge of the developing limbs. CONCLUSION: The restricted expression of both LHX genes and IRF6 in the facial primordia suggests roles for these gene products in promoting directed outgrowth and fusion of the primary palate. The manipulability, minimal cost and susceptibility of chicks to CL/P will enable more detailed investigations into how perturbations of IRF6, LHX6 and LHX7 contribute to common orofacial clefts.

Amino Acid Sequence↗

The craniofacial dysostosis syndromes: current surgical thinking and future directions.

Craniofacial dysostosis is the term applied to familial forms of craniosynostosis in which the sutural involvement generally includes the cranial vault, cranial base, and midfacial skeletal structures. The syndromic forms of craniofacial dysostosis were initially described by Carpenter, Apert, Crouzon, Saethre and Chotzen, Pfeiffer, and others. In addition to the dysmorphic cranial features, affected individuals may have profound alterations in facial skeletal development. Surgical reconstruction requires thoughtfully sequenced and staged procedures with consideration for the individual's specific malformations, craniofacial growth patterns, and psychosocial needs. Management of the craniofacial dysostosis syndromes is surgical, but the indications and the timing, type, and effectiveness of each stage of reconstruction have not been well evaluated and remains as much an art as a science. This article reviews the specific characteristic clinical features of the craniofacial dysostosis syndromes and presents current philosophy and rationale for the staging of reconstruction.

Craniofacial Dysostosis↗

Cleft lip and palate care in the United Kingdom--the Clinical Standards Advisory Group (CSAG) Study. Part 1: background and methodology.

OBJECTIVE: To review the history of cleft care in the United Kingdom and the rationale and methodology of a national study. DESIGN: Descriptive overview to introduce this series of papers. SETTING: Fifty National Health Service cleft teams. PATIENTS/PARTICIPANTS: Two age cohorts, 5-year-olds and 12-year-olds, with nonsyndromic complete unilateral cleft lip and palate and their parents. MAIN OUTCOME MEASURES: Facial development and appearance, quality of bone grafts, speech, oral health and patient/parent satisfaction. CONCLUSIONS: The case for a national assessment of cleft care is made. The methodology of the cross-sectional outcome study is described, together with a survey of training for recently appointed clinicians.

Attitude of Health Personnel↗

Skulls with facial clefts. Experimental surgery on the facial skeleton.

Skulls of adult, unoperated patients with facial clefts show additional anomalies specific for the type of cleft. In an experimental study we demonstrated that similar anomalies develop in rabbits with clefts that are artificially made in the fourth week after birth. Some surgical methods (Veau, primary osteoplasty) for correction of facial clefts are compared with regard to their effect upon later growth of the skull of young rabbits. The importance of experimental surgery preceding the introduction of new surgical techniques on the growing skull and its facial skeleton in particular is stressed.

Abnormalities, Multiple↗

Management of temporo-mandibular joint ankylosis in growing children.

Although temporo-mandibular joint (TMJ) ankylosis is one of the most common pathologies afflicting the facial skeleton, it is also the most overlooked and under-managed problem in children. The TMJ forms the very cornerstone of cranio-facial integrity and hence its ankylosis in growing children adversely affects the growth and development of the jaws and occlusion. Impairment of speech, difficulty in mastication, poor oral hygiene, rampant caries and acute compromise of the airway pose a severe psychologic burden on the tender minds of children. The aim of this article is to present an overview of efficient management strategies, based on a case report, so as to increase its awareness among all dental surgeons involved in the treatment of children.

Ankylosis↗

[Prediction of growth and dento-facial orthopedics. Importance and accuracy of superposition].

For a long time, the orthodontists have tried to predict the growth to come. They have atempted to know if there are relationships between height and facial growth and if a better knowledge of general development of child had an interest to their therapeutic decision. The author compare the methods of estimation and prediction of height and facial growth; and he thinks that these methods are not enough exact and safe and they have a little utility to the practionner. The author purpose to replace them by the search of radiologics signs which need anyone measurement and which the good prediction value is very adequate to the daily dento-facial orthopedic pratice.

Age Determination by Skeleton↗

Guiding occlusal development with functional appliances.

Orthodontic treatment with functional appliances has been available since the beginning of this century. One of the first known functional appliances, 'the Monobloc', was introduced at a conference in Paris in 1926 by Pierre Robin. This appliance had been developed at the beginning of the century for the treatment of patients with Pierre Robin syndrome. Patients with this syndrome have extreme retrognathia of the mandible at birth and, as a result, the tongue is so far back in the pharynx that there is a risk of suffocation. Several years later, the same appliance was modified by Pierre Robin himself for the treatment of patients with Class II malocclusions which are in combination with extreme overjet.

Activator Appliances↗

Skeletal growth after muscular reconstruction for cleft lip, alveolus and palate.

OBJECTIVE: To compare two treatments for unilateral cleft lip and palate. DESIGN: Follow-up study. SETTING: University department, Germany. SUBJECTS: 110 children with unilateral cleft lip and palate, 50 of whom were treated between 1980 and 1988 (group 1) and 60 of whom were treated between 1970 and 1979 (group 2). INTERVENTIONS: Group 1 had no dentofacial treatment before operation. At the age of 3 months they had musculoperiosteal reconstruction with no orthopaedic treatment postoperatively. At the age of 9-12 months they had the velar muscle reconstructed but no orthopaedic treatment to follow. Group 2 had dentofacial treatment before operation. At the age of 6 months they had their lips operated on by Millard's technique. Postoperatively they had dentofacial orthopaedic treatment followed, at the age of 2.5 years, by palatal surgery by the Campbell-Widmaier technique. This was followed by more dentofacial orthopaedic treatment. Main outcome measure--Growth of the midface skeleton. RESULTS: The course of the anterior tooth segment was similar in the two groups, as was the transverse development of the maxilla. Analyses of both dental casts and lateral cephalometric radiographs showed that the skeletal development in group 1 (who had undergone reconstruction of the perioral and perinasal muscles) was better than in group 2 (who had not) despite the fact that group 1 had had no orthopaedic growth stimulation. CONCLUSION: If the primary and secondary growth centres are not joined at the first operation and the midfacial muscles are not reconstructed, even orthopaedic growth stimulation will not compensate for the defect.

Alveolar Process↗

Upper airway obstruction and craniofacial morphology.

Otolaryngologists are being asked with increasing frequency to assess adequacy of the upper airway and to treat upper airway obstructive problems in orthodontic patients. The incentive has been provided by recent studies that purport to relate upper airway obstruction to dental and craniomorphologic changes. It is hypothesized that prolonged oral respiration during critical growth periods in children initiates a sequence of events that commonly results in dental and skeletal changes. In the chronic mouth-breather excessive molar tooth eruption is almost a constant feature, causing a clockwise rotation of the growing mandible, with a disproportional increase in anterior lower vertical face height. Such increases in anterior lower vertical face height are often associated with retrognathia and open bites. Low tongue posture seen with oral respiration impedes the lateral expansion and anterior development of the maxilla. Otolaryngologists have the ability to objectively and accurately assess upper airway patency. Rhinometric assessment before and after application of topical nasal decongestant, in conjunction with clinical examination, provides valuable information regarding upper airway patency and the cause of any existing obstructive pathologic condition. Studies should be designed carefully to control the numerous variables that have an impact on the growing face of a young child so that meaningful data can be obtained in our own field regarding this challenging topic.

Child, Preschool↗

Testing for occlusal disharmonies.

Occlusal disharmony can present in a variety of forms. It affects both very young and very old patients. Occlusal disharmony can directly cause problems within the stomatognathic system, or indirectly be the result of another condition. Moreover, occlusal disharmony may be implicated in conditions of multi-factorial origin such as mandibular pain disharmony can directly cause problems within the stomatognathic system, or indirectly be the result of another condition. treatment, prevent complications, and often yield improved results. In the last decade rapid technological advance has resulted in many testing procedures becoming available to supplement the traditional diagnostic methods. However, this technological development has been such that practising dentists will have difficulty in keeping up with the use of the newer diagnostic instrumentation. Advanced education will be required if dentists are to utilize the instrumentation to its full capability both at the diagnostic and therapeutic levels. It is also likely that the amount of expertise required to utilize the available technology will further emphasise the necessity of diagnosis at the team level for many of the more complex occlusal disharmonies.

Adult↗

Guiding occlusal development with functional appliances.

Functional appliances have been used in orthodontics since their introduction by Pierre Robin almost one hundred years ago, however, our understanding of how they bring about orthodontic correction is still limited. This article is a brief overview of their history, mode of action, advantages and disadvantages, and includes the results of a study of attempts to control and minimise their side-effects using a recent development in functional appliances: the 'Teuscher Appliance'. This appliance combines a high-pull headgear with the activator, and is designed to reduce the often reported side-effects of functional appliances. The skeletal and dentoalveolar effects of treatment with the Teuscher Appliance on 40 consecutively-treated patients are reported and illustrated with four individual case reports. The results showed that the skeletal effect on the maxilla was a retardation of the normal forward and downward growth in 80 per cent of the cases, and that mandibular growth in 70 per cent of cases was forward. In patients whose mandibular growth was primarily in a vertical direction, such growth could be ascribed mainly to posterior rotation of the maxilla and/or the fact that the acrylic covering the lower posterior teeth to correct a deep bite was removed, promoting the eruption of these teeth and increasing the anterior vertical development. The dentoalveolar changes were characterised by retroclination of the maxillary incisors in 90 per cent of the patients, and were due to insufficient torque control by the built-in torque springs, which need further development. The mandibular incisors were well controlled by capping. The statistical analysis showed an inverse correlation between the initial incisor inclination and the change during treatment. This suggests that proclination of the lower incisors, as previously reported, is not a contra-indication to functional appliance treatment, provided the appliance is correctly designed. Overall, this study showed considerable individual response to treatment, and that the occlusal correction occurred through a combination of skeletal and dentoalveolar changes.

Adolescent↗

Dental trauma due to sport in the pediatric patient.

Dental trauma due to sport is devastating to any athlete but is made worse when the athlete is young and still growing. Orofacial trauma is relatively common in the pediatric population and can have significant management problems. Continued maxillary and alveolar growth, development, and maturation of individual teeth, as well as behavioral considerations create unique challenges in the management of this population. Children and youths have increased risk factors for dental trauma due to sports, including untreated malocclusions, an increase in risk-taking behavior, and undeveloped coordination and skill levels. This review article aims to increase awareness as to the role of sports in dental and orofacial injuries, discuss risk factors for this trauma as well as its prevention, and outline risk minimization for the pediatric athlete.

Adolescent↗

Facial growth and development in unilateral cleft lip and palate from the time of palatoplasty to the onset of puberty: a longitudinal study.

X-ray cephalometry was used for the assessment of facial growth and development from the time of palate surgery to the onset of puberty (from 5 to 11 years) in 24 boys with unilateral cleft lip and palate treated with primary periosteoplasty (at 8 months) and palatal pushback supplemented by pharyngeal flap surgery (at 5 years). The lowest growth showed the depth of the maxilla and the height of the upper lip. An increasing protrusion of the mandible and in particular the increasing retrusion of the maxilla resulted in a flattening of the face and in an impairment of sagittal jaw relations. However, it was possible to attain an improvement of overjet produced by a substantial increase of the proclination of upper incisors and of the alveolar process. There was a deterioration of the prominence of the upper lip. Anterior growth rotation was absent during the development of the face, though a rotation in both directions was quite common in individual cases. The steepness of the mandibular body, vertical jaw relations, and facial vertical proportions remained unchanged. As compared to the pubertal period, the growth and development differed only by a more marked proclination of the dentoalveolar component of the maxilla and by an improvement of overjet. Facial convexity and sagittal jaw relations deteriorated in more than 90% of the patients, the overjet only in 20%, yet the prominence of the lip in 70%. Facial convexity and sagittal jaw relations were not correlated with mandibular rotation but they affected the overjet and the prominence of the upper lip.(ABSTRACT TRUNCATED AT 250 WORDS)

Cephalometry↗

Orthopedic coordination of dentofacial development in skeletal Class II malocclusion in conjunction with edgewise therapy. Part I.

The skeletal Class II malocclusion may be considered to develop as a failure of the coordinating process to maintain harmonious relationships within the developing dentofacial apparatus. If the skeletal elements are too far apart for adaptation to occur and/or if there are functional abnormalities of the orofacial musculature which inhibit coordination from taking place, a malocclusion will result. An orthopedic technique and appliance system has been developed with the intention of improving those factors responsible for the development and perpetuation of the skeletal Class II malocclusion in a primary stage of treatment. This is accomplished by means of restraint and redirection of forward maxillary growth and an increase in the velocity of mandibular growth. Concurrently, adverse soft-tissue influences are eliminated or ameliorated. Edgewise appliance therapy is subsequently carried out for the final correction. The subject is considered in two articles. This first article describes the effects of the restraint of maxillary growth on craniofacial development and the dental changes produced by a maxillary removable splint with extraoral traction and shows how they can be used clinically for correction of the skeletal Class II malocclusion. The experimental and clinical evidence supporting this approach is considered, and case histories show the clinical use of the maxillary splint. This form of maxillary therapy for the skeletal Class II malocclusion has limitations, and it is desirable for it to be incorporated into a comprehensive orthopedic system.

Cephalometry↗

Secondary cartilages in growth and development of the symphysis menti in the hamster.

The early growth and development of the symphyseal joint and pertinent adjacent regions of the mandible in the hamster, Mesocricetus auratus, are described. The joint is patent throughout life and in adults contains a large fibrocartilage which mediates mandibular movements. Evidence is presented to show that this cartilage is formed by the fusion of Meckel's cartilage with two secondary cartilages that appear within the joint space. Evidence is also presented which suggests that cells of the symphyseal cartilage have the capacity to produce bone.

Aging↗

TGF-beta signaling and its functional significance in regulating the fate of cranial neural crest cells.

Members of the transforming growth factor-beta (TGF-beta) superfamily regulate cell proliferation, differentiation, and apoptosis, and control the development and maintenance of most tissues. TGF-beta signal is transmitted through the phosphorylation of Smad proteins by TGF-beta receptor serine/threonine kinase. During craniofacial development, TGF-beta may regulate the fate specification of cranial neural crest cells. These cells are multipotent progenitors and capable of producing diverse cell types upon differentiation. Here we summarize evidence that TGF-beta ligands and their signaling intermediates have significant roles in patterning and specification of cranial neural crest cells. The biological function of TGF-beta is carried out through the regulation of transcriptional factors during embryogenesis.

Animals↗

Craniofacial maturity and perceived personality in children with Down syndrome.

In this pair of studies, we examined whether the common perception of a positive Down syndrome personality is associated with a youthful craniofacial appearance, similar to Zebrowitz's (1997) "babyface." In Study 1, 43 observers rated photographs of age-matched children with Down syndrome, another mental retardation syndrome (5p- syndrome), and typically developing children. Those with Down syndrome were perceived as being more physically babyfaced and more likely to behave in an immature manner. We controlled for the effect of familiarity with Down syndrome in Study 2 by employing a within-etiology design in which 128 observers rated 12 pictures of 10-year-old children with Down syndrome. Results showed that more physically babyfaced children with Down syndrome are more subject to the overgeneralization.

Adult↗

Removable partial denture in special clinical conditions.

Controversies concerning the prosthetic treatment through removable or fixed dentures until the age of 18 existed and continue to exist due to the structural instability of the stomatognathic system. In this communication we present a special experience, acquired about the treatment of some clinical situations in which the removable partial denture has been found appropriate with respect to certain features as bone substance loss as a consequence of traumatisms or tumor extirpations, oligodontia, cleft lip and palate. The clinical situations that will be presented refer to a group of 29 patients treated during a period of 10 years in the Department of Prosthetic Dentistry within the Faculty of Stomatology of Iaşi, Romania. Our conclusion is that the children and teenager patients prosthetic therapy differs with respect to the type and etiology of the teeth loss, the affected zone, the stage of the growth and development process of the stomatognathic system, the technological conditions, the patient social conditions, and last but not least, by his affective attitude regarding the teeth loss and the denture type. The removable partial denture for children and teenagers represents, with some exceptions, an emergency or transitory solution until the necessary conditions for other therapeutic stages are fulfilled. Taking into consideration the tissues lability on which it is applied, the removable partial denture requires a rigorous postprosthetic therapy.

Adolescent↗