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At least 631 records · Page 35Linked to original sources
PubMed1995

Molecular genetic approaches to the study of human craniofacial dysmorphologies.

Craniofacial dysmorphologies are common, ranging from simple facial disfigurement to complex malformations involving the whole head. With the advent of gene mapping and cloning techniques, the genetic element of both simple and complex human craniofacial dysmorphologies can be investigated. For many of the dysmorphic syndromes, it is possible to find families that display a particular phenotype in either an autosomal dominant, recessive, or X-linked manner. This article focuses on a subgroup of craniofacial dysmorphologies, covering these three main inheritance patterns, that are being studied using molecular biology techniques: DiGeorge syndrome, Treacher Collins syndrome, Greig cephalopolysyndactyly syndrome, acrocallosal syndrome, amelogenesis imperfecta, and X-linked cleft palate with ankyloglossia. Once the mutated or deleted gene or genes for each syndrome have been cloned, patterns of normal and abnormal craniofacial development should be elucidated. This should enhance both diagnosis and treatment of these common and disfiguring disorders.

Animals↗
PubMed1984

Retinal and optic nerve findings in Goldenhar-Gorlin syndrome.

Involvement of the posterior segment of the eye in Goldenhar-Gorlin syndrome is more common than is generally appreciated. We examined seven patients with this syndrome. Abnormalities included diminished visual acuity, tilted optic disc, optic nerve hypoplasia, tortuous retinal vessels, macular hypoplasia and heterotopia, microphthalmia and anophthalmia. In one case, pathologic study showed agenesis of the optic nerve. It is proposed that retinal, optic nerve and craniofacial abnormalities in this condition may reflect an asynchrony in the migration of the neural crest cells in the early stages of embryonal development.

Adolescent↗
PubMed2000

Treacher Collins syndrome with acute airway obstruction.

Treacher Collins syndrome presents with cranio-facial deformities of variable expressions and severity, but rarely is it associated with acute airway obstruction of such severity that it necessitates surgical intervention. The authors describe their experience with such a neonate, who presented with acute airway obstruction successfully managed with a tracheostomy. The relevant English literature is reviewed.

Acute Disease↗
PubMed2001

[Mandibular growth, its environmental interactions].

A thorough knowledge of mandibular growth is necessary for proper comprehension of growth disorders and malformations affecting the lateral cranio-facial region. The growth mechanisms are complex and multifactorial, the mandible itself is subdivided into two heterogeneous and interdependent segments, as far as growth is concerned. These are the ramus and the corpus. The ramus is interacting with the middle cranial base through the TMJ and the temporo-pterygoid muscle belt. The mandibular body is interacting with the maxilla and the anterior cranial base. Its occlusal role is a function of the adaptive capacity of the alveolodental region.

Alveolar Process↗
PubMed2001

[Morphological alterations of oto-mandibular syndromes].

Otomandibular dysplasia is a congenital malformation defined by a certain degree of temporomandibular or pterygomandibular hypoplasia. The syndrome is characterised by the variability of clinical findings, but the three major features are auricular, mandibular and maxillary hypoplasia. All the laterofacial structures may be affected. The deformity is usually unilateral but bilateral cases exist; a lot of associated malformations have been described. Multiple classification systems have been published. Some of them are very complex, but it is possible to define a simple diagnostic diagram based on ethiopathogenic data. Bilateral involvement affects predominantly the zygoma, and concerns hereditary syndromes. When the mandibular hypoplasia is evident Franceschetti or Goldenhar syndrome is suspected; otherwise Treacher-Collins syndrome is probable. Unilateral cases are not, in general, hereditary and the hypoplasia predominates on the mandible. The difference between hemifacial microsomia or mandibular dysplasia is made by the presence of associated laterofacial deformities.

Ear, External↗
PubMed2001

[Imaging of oto-mandibular dysplasias].

The modern imaging opened a possibility of precise exploration of otomandibular dysplasias. The techniques of browsing by IRM and CT Scan and the software of image processing which is associated with these techniques (three-dimensional, superposition and removing of the anatomical structures) allow to carry out a true anatomical dissection of the whole of these malformative syndromes. The study of the skeletal anomalies gains by specifying the disorders of development of the various mandibular segments: temporomandibular joint, ramus and horizontal branch and also, all cranio-maxillar structures: orbital rim, cranial basis. Study of masticatory muscles shows an important damage correlated with skeletal troubles and performs a grading of morphological and functional gravity. This analytical study emphasizes some malformative axis: malar axis in cases of mandibulofacial dystosis, temporomandibular axis in hemifacial microsomia. Modern imaging can appreciate, by development of three-dimensional cephalometry, growth anomalies and quantification of post therapeutical results. At last usefulness of imaging appears in therapeutic modelisation and in the field of Computer-Aided Planning of surgical techniques of osteotomies and overall maxillaries distractions.

Ear↗
PubMed2001

[Microtia in cases of oto-mandibular dysplasia].

The technique of reconstruction of a microtia, observed in cases of otomandibular dysplasia, does not differ in its principle from that of an isolated microtia. The technical approach depends primarily on the aspect of the auricular remnants whose forms are multiple and are in no way etiologically specific. However, when associated with other malformations of otomandibular dysplasia, the microtia presents some particularities and its correction must be integrated in the global treatment of hemifacial anomalies.

Adolescent↗
PubMed2001

[Mandibular elongation and remodeling by gradual distraction. An experience of 277 cases].

Distraction osteogenesis at the craniofacial skeleton offers superior results than using standard osteotomies and other surgical techniques when treating mandibular hypoplasia. After ten years, we have performed 221 patients with hemifacial microsomia and 56 patients with micrognathia using external mandibular distraction. For every single patient the distraction vector is different according to the degree of bone hypoplasia. The bone elongation ranged from 12 to 49 mm, and the results were excellent: facial symmetry was reestablished, the menton became horizontal and the long term result controls shows stability clinically and occlusally.

Adolescent↗
PubMed2001

[Hemifacial microsomia treated with mandibular lengthening using intraoral distractors. On precise indications].

Mandibular hypoplasia in the hemifacial microsomia have largely benefited from distraction techniques especially intraoral. In fact, these techniques are possible in the child, replacing him in a better morphological, psychological and functional configuration. They can redynamise growth and, in any case, diminish secondary distant effects. The authors, from precise criteria, propose a surgical classification to facilitate the therapeutic decision. Intraoral and sometimes bidirectional distraction, whose results are equivalent to external distraction, present numerous advantages that, in the majority of cases, make it preferred now to external distraction. Its three-dimensional (3D) effect on bone and soft tissues and the long term follow-up must be evaluated more objectively, as pre and post-distraction stereolithographic or 3D photogrammetric may get. It must be considered in the child as the first step of skeletic surgery preparing to a facilitated second step orthopedic, orthodontic or orthognathic after puberty. In the young adult, it is in competition with conventional surgery but the slow progressive process, kind with the temporo-mandibular joints, allows in mandibular asymmetries an adaptation having avoided the need for controlateral osteotomy or a bone graft.

Adolescent↗
PubMed1976

Anatomical classification facial, cranio-facial and latero-facial clefts.

Based on personal observations a new classification of facial, cranio-facial and latero-facial clefts is proposed. The orbit is used as the primary structure of reference. Fifteen locations for clefts can be differentiated. Their course through soft tissues and bone is described in detail and illustrated with typical cases. Combinations of several types of clefts and associated malformations are discussed. The new classification offers the following advantages: it eliminates the old confusing terminology (based on personal experience and case descriptions in the literature it seems to be complete); it facilitates recording of malformations (communication between observers becomes easier); it increases the appreciation for the scope and the tridimensional structure of cranio-facial deformities; and, finally, the better understanding of these rare malformations will lead to more careful investigations, and to more adequate planning of treatment. More complications in corrective surgery will be avoided and the results achieved will improve.

Classification↗
PubMed1977

A spectrum of application of autogenous costochondral grafts.

Autogenous costochondral grafts have found application in 26 variegated cases of congenital dysplasia, ankylosis, neoplastic disease, osteoarthritis, and post-traumatic dysfunction. The authors' experience with the grafting procedure has been generally favourable, but various difficulties have also arisen. There is a cursory discussion on the physiological superiority of rib cartilage grafts over those of other autogenous joints in reconstruction of the temporomandibular articulation.

Adolescent↗
PubMed1980

Overcorrection in mandibular advancement.

Seven cases of class II skeletal deformities of the face are described in which overcorrection into a class III relationship was performed. This concept is based on the unpredictability of the relapse tendency on the one hand and the anterior inclination of the lower front teeth in some cases (which may block mandibular advancement significantly) on the other hand. Depending on the degree of the relapse occurring, postoperative orthodontic treatment, a second segmental operation or mere occlusal grinding is required.

Adolescent↗