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Genetic aspects of some orofacial anomalies.

The increased importance of congenital anomalies in childhood disease is stressed. It is suggested that treatment and research in congential craniofacial anomalies would benefit from centralized registration. Their clinical management should be delegated to centres with a multidisciplinary team. Some problems related to genetic counselling and identification of microforms are illustrated by cleft lip and palate. An account is given of some orofacial anomalies.

Abnormalities, Multiple↗
PubMed1986

[3-dimensional display of computed tomographic studies of craniofacial anomalies].

Craniofacial anomalies are conventionally investigated by cephalometry using ordinary radiographs and by computed tomography. Both methods have the major disadvantage of trying to demonstrate a complex three-dimensional structure, such as the skull, in two dimensions and they therefore cannot display a true spatial image. We present the principle underlying a three-dimensional display derived from computer tomographic studies and discuss the clinical application in the diagnosis of craniofacial anomalies.

Adolescent↗
PubMed1977

Cleft palate in two syndromes.

Patients with cleft palates with or without cleft lip need more special attention at birth when the cleft is associated with other malformations. For example, the Pierre-Robin and the Klippel-Feil Syndromes are both well known for the special problems they present. Reported here are three cases, with Crouzon's and two with Franceschetti Syndromes, all of whom have palatal clefts.

Adult↗
PubMed1989

[Facial malformations and asymmetries caused by the first and second branchial arch syndrome].

The first and second branchial arch syndrome is a congenital affection, among whom one can range as well hereditary as non-hereditary entities. They always cause a badly mutilating facial asymmetry. In this article, the first and second branchial arch syndrome were divided in hemifacial and bilateral dysostoses. We pointed out the hemifacial microsomia, the most occurring entity in these series. From two case reports, we attempt to give a treatment synopsis of this syndrome, which is always done by a multi-disciplinary team, including dentists, orthodontists, maxillofacial surgeons and plastic surgeons.

Adult↗
PubMed1985

A comparative cephalometric study of the cranial base in craniofacial anomalies: Part I: Tensor analysis.

The method of mean tensor analysis was used to study the cranial base in six craniofacial anomalies: Crouzon's disease, Apert's syndrome, Pfeiffer's syndrome, craniofacial microsomia (CFM), Treacher Collins (TC) syndrome, and frontonasal dysplasia (FND). The form was represented by five landmarks: the nasion (N), basion (Ba), sella (S), frontomaxillonasal suture (FMN), and sphenoethmoidal registration point (SE), and the deformities were computed as mean deformations from age- and sex-matched normal mean forms. The cranial base in CFM is normal in shape. The other five syndromes manifest four distinct patterns of shape variation. Only in TC and Pfeiffer's syndrome is the cranial-base angle distinctive. In Apert's and Crouzon's syndromes, point SE is displaced anteriorly upon a cranial base, small in size but otherwise normal in shape. In TC syndrome and FND, point SE is displaced posteriorly toward the sella.

Acrocephalosyndactylia↗