1/39. Dental and craniofacial features of Aarskog syndrome: report of a case and review of literature.Aarskog syndrome is a rare syndrome with a typical triad of facial, digital and genital characteristics. The characteristic cephalometric finding in this patient was the unusually large upward slant of SN plane and a steep Ba-N plane. Though the patient presented with a class I skeletal pattern, both the maxilla and mandible were hypoplastic and retruded with respect to the cranial base. Other characteristic features regarding the mandibular morphology were a large FMA (37 degrees) and Sn-GoGn (44 degrees) angles, a large gonial angle (138 degrees), an increase in total anterior facial and lower anterior facial height.- - - - - - - - - - ranking = 1keywords = craniofacial (Clic here for more details about this article) |
2/39. Primary failure of tooth eruption: a unique case.Primary failure of tooth eruption rarely occurs. This case represents a rare clinical situation and appears to reflect a generalized disturbance in the eruptive process, inasmuch as (1) deciduous and permanent dentition are affected, (2) incisors, molars, and premolars are involved in all quadrants, (3) skeletal and craniofacial growth are within normal limits, and (4) no systemic/genetic anomalies were detected. This is the first such case reported in the literature; diagnosis and management are discussed.- - - - - - - - - - ranking = 0.25keywords = craniofacial (Clic here for more details about this article) |
3/39. Anomalies of craniofacial skeleton and teeth in cleidocranial dysplasia.Mutations involving the transcription factor CBFA1 cause cleidocranial dysplasia (CCD) in man. Recently, a mouse model of CCD has been generated (Cbfal /-) [Komori et al., 1997], and disturbances of osteoclast differentiation have been documented. It has been shown that these animals exhibit hypoplastic clavicles and nasal bones, and retarded ossification of parietal, interparietal, and supraoccipital bones. humans with CCD show all these features, including severely retarded ossification of the cranial base, strongly suggesting that both intramembranous ossification and endochondral ossification are affected. In addition, CCD patients have multiple supernumerary teeth and delayed tooth eruption. The present report presents 3D reconstructions of computerised tomography (CT) scans of the craniofacial region of a CCD boy examined at both 1 and 7 years of age. The anomalies in craniofacial skeleton and teeth are analysed and compared to the findings of our previous clinical studies and to the findings in the animal model. Based on the available information, we suggest that osteoblast, osteoclast, and dentinoclast differentiation may be disturbed in CCD.- - - - - - - - - - ranking = 1.5keywords = craniofacial (Clic here for more details about this article) |
4/39. Crouzon disease--a case report.The present case of a ten year old boy with craniofacial dysostosis with the features of midfacial hypoplasia is a disease known as Crouzon disease. This disease is characterised by cranial deformities, facial malformation, eye changes and occasional other associated abnormalities. The aim of this case is to discuss the clinical, radiographic features and management of the problems.- - - - - - - - - - ranking = 0.25keywords = craniofacial (Clic here for more details about this article) |
5/39. Orthodontic treatment for patients with turner syndrome.The purpose of this study was to review the distinctive characteristics of turner syndrome and to illustrate how they influenced the orthodontic treatment of a 17-year-old patient with turner syndrome. The rate and the timing of growth, the development of the craniofacial structures, and the somatic and dental morphology unique to turner syndrome significantly influence orthodontic treatment. Modifications that might be required to orthodontic treatment plans include (1) antibiotic prophylaxis, (2) occlusal adjustments to account for altered dental morphology, (3) altered treatment timing because of major differences in growth and differences between chronological and skeletal ages, and (4) growth hormone therapy. Orthodontists must understand the characteristics of turner syndrome and modify the patient's treatment plan accordingly.- - - - - - - - - - ranking = 0.25keywords = craniofacial (Clic here for more details about this article) |
6/39. Cranioectodermal dysplasia: a new patient with an inapparent, subtle phenotype.Cranioectodermal dysplasia is a rare syndrome characterized by craniofacial and skeletal anomalies and ectodermal dysplasia. life-threatening associated conditions (i.e., kidney failure and abnormal regulation of the parathyroid-bone axis) can also develop. We report a patient whose features are suggestive of an inapparent, subtle phenotype of the syndrome. The patient is a 4-year-old girl with only dolichocephaly and clinodactyly; microdontia, hypodontia, and taurodontia (i.e., cone-shaped teeth); anteverted nares, full cheeks, and everted lower lip; epicanthal folds, hypertelorism and hyperopia; and corpus callosum hypoplasia. She has no rhizomelic limb shortening or hair abnormalities. In view of the rarity of the cranioectodermal dysplasias, the variability of the phenotype, and the uncertain outcome of some previously described patients, we believe this inapparent, subtle case should reported to enable better understanding and treatment of this rare syndrome.- - - - - - - - - - ranking = 0.25keywords = craniofacial (Clic here for more details about this article) |
7/39. Schinzel-Giedion syndrome: interesting facial and orodental features, and dental management.Schinzel-Giedion syndrome comprises multiple congenital anomalies. The orofacial features include coarse facies, frontal bossing, ocular hypertelorism, anterior open bite and macrodontia. Two cases are presented in which the presence of specific craniofacial anomalies with bilateral hydronephrosis confirmed the diagnosis. In one patient, bottle-feeding was associated with caries in maxillary central and lateral incisors, but the second patient was permanently tube fed and did not experience any dental caries. Clinical management of these patients requires a coordinated approach from a team of medical and dental specialists.- - - - - - - - - - ranking = 0.25keywords = craniofacial (Clic here for more details about this article) |
8/39. Report of a patient with hypoglossia-hypodactylia syndrome and a review of the literature.OBJECTIVE: To present the morphology of the extremities, craniofacial structures, and the oral cavity based on roentgencephalometry and three-dimensional computed tomography and magnetic resonance imaging scanning in a patient with hypoglossia-hypodactylia syndrome, discuss the orthodontic treatment method, and review the literature for the syndrome. PATIENT: The patient was a 6-year-old boy diagnosed with hypoglossia-hypodactylia syndrome at birth. He had hypodactylia as well as micrognathia with steep inclination of the anterior surface of the mandible in relation to the lower mandibular plane. He had missing mandibular incisors with concomitant bone defect limited to the associated alveolar ridge and an absence of any malformations in the mandibular ramus and condylar head. The patient had a bilateral scissors bite with an extremely constricted mandibular dental arch, skeletal Class II jaw relationship with an average mandibular plane angle and maxillary incisors inclined palatally. He had extremely reduced tongue size and hypertrophy of the floor of the mouth. Anomalies of the central nervous system were not observed. There was no evidence of hearing loss.- - - - - - - - - - ranking = 0.25keywords = craniofacial (Clic here for more details about this article) |
9/39. hajdu-cheney syndrome (acro-osteolysis): a case report of dental interest.hajdu-cheney syndrome (acro-osteolysis) is a rare disorder of bone metabolism characterized by progressive lytic lesions in a number of bones. Constant features of this condition include an osteoporotic skeleton, acro-osteolysis, and a shortened lower third of face. The purpose of this report was to focus on the craniofacial and oral manifestations of the disorder in a 9-year-old boy.- - - - - - - - - - ranking = 0.25keywords = craniofacial (Clic here for more details about this article) |
10/39. A new syndrome of symphalangism, multiple frenula, postaxial polydactyly, dysplastic ears, dental anomalies, and exclusion of NOG and GDF5.A Thai girl with a unique combination of limb and craniofacial anomalies is reported. Manifestations include blepharoptosis; prominent nose; hypodontia; multiple, hyperplastic frenula; and dysplastic ears. Limb anomalies include short stature, postaxial polydactyly of both hands and the left foot, proximal and distal symphalangism of fingers, and congenital absence of the distal phalanges of toes 2-5. mutation analyses of NOG and GDF5, the genes responsible for symphalangism-related syndromes, were negative.- - - - - - - - - - ranking = 0.25keywords = craniofacial (Clic here for more details about this article) |
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