1/40. Autosomal dominant osteosclerosis: report of a kindred.Autosomal dominant osteosclerosis (ADO), a rare inherited craniotubular bone disorder, is a generalized hyperostosis that manifests itself as increased cortical thickening of the skull, mandible, metacarpals, metatarsals, long bones, vertebral bodies, ribs, and clavicles. jaw abnormalities, which clinically resemble the widening and deepening of the mandible seen in cherubism, begin in childhood and have been reported to stabilize after puberty. Teeth and alveolar bone are normal. ADO must be distinguished from Van Buchem's disease, which is characterized by elevated serum alkaline phosphatase, neurologic complications, exopthalmos, periosteal excrescences, and an autosomal recessive pattern of inheritance, as well as from other craniotubular bone disorders such as osteopetrosis. We present clinical and radiographic documentation of members of a kindred representing 4 generations affected with ADO. At initial examination of the proband, a differential diagnosis included cherubism, fibrous dysplasia, osteopetrosis, and Paget's disease. Radiographic examination revealed extensive radiopacity of the inferior border and basal bone of the mandible. The proband's clavicles and humerus were also affected. All family members examined were similarly affected and had mandibular and palatal tori. Authors of a previously published report on the dental and dentoalveolar management of patients with craniotubular bone disorders have recommended prophylactic antibiotics to minimize risk of osteomyelitis in all such cases. The members of our kindred received extensive dental treatment before diagnosis, including extractions of severely carious teeth, preprosthetic dentoalveolar surgery, and endodontic therapy; there was no incidence of osteomyelitis or postsurgical complications. Therefore, the use of prophylactic antibiotics may not be warranted in patients with ADO who have otherwise normal medical histories.- - - - - - - - - - ranking = 1keywords = dental (Clic here for more details about this article) |
2/40. Accuracy of integration of dental casts in three-dimensional models.PURPOSE: This study investigated errors occurring in three-dimensional (3D) models when plaster dental casts are integrated into them. MATERIALS AND methods: Three-dimensional milling models of three patients with a jaw deformity were fabricated using the Endoplan system (SPARC International Inc, Santa Clara, CA). After this, plaster dental casts were integrated into the 3D models using a face-bow transfer system. Two cephalograms were then compared, one obtained from the patient and the other obtained from the 3D model painted with contrast medium. RESULTS: In two cases, the reproducibility of the dental position as determined by angle analysis was within 2 degrees, and that determined by distance analysis was within 2 mm. However, errors over 4 degrees and 4.2 mm, respectively, were observed in one case. CONCLUSION: It is clinically important to confirm the accuracy of the 3D model by cephalometric analysis, and it may be necessary to reposition the dental model based on the results.- - - - - - - - - - ranking = 4keywords = dental (Clic here for more details about this article) |
3/40. Severe acro-renal-uterine-mandibular syndrome.Although limb and renal defects occur together in a variety of patterns of multiple malformations, familial cases of acro-renal disorders are rare. In 1980, Halal et al. inverted question markAm J Med Genet 5:277-284 described two sisters with unusual limb deficiencies, renal anomalies, and mandibular hypoplasia and termed this condition acro-renal-mandibular syndrome. A girl reported earlier by Fitch and Lachance inverted question mark1972; Can Med Assoc J 107:653-656 had similarly limb and renal findings, but an apparently normal jaw. We document three sibs with unusual limb deficiencies, renal agenesis, uterine anomalies in the two females, and orofacial defects, who clearly have a similar but more severe type of acrorenal disorder, apparently inherited as an autosomal recessive condition. The sibs with limb deficiencies and renal agenesis reported by Hennekam et al. inverted question mark1994; Am J Med Genet 53:102-107 appear to be additional cases of this very rare disorder, the pathogenesis of which may be related to abnormal epithelial-mesenchymal interactions.- - - - - - - - - - ranking = 1.7909618675771keywords = hypoplasia (Clic here for more details about this article) |
4/40. Orodental abnormalities in lobster claw syndrome (a type of syndactyly).Lobster Claw syndrome is a type of syndactyly, where abnormalities in the hand-foot region as well as the orodental region occur. A case of a 14-year old boy with this syndrome is presented here.- - - - - - - - - - ranking = 2.5keywords = dental (Clic here for more details about this article) |
5/40. Transmission of the dysgnathia complex from mother to daughter.We report the first observation of parent-to-child transmission of dysgnathia, a rare disorder characterized by severe mandibular hypoplasia or agenesis, ear anomalies, microstomia, and microglossia. Patient 1 was noted prenatally by ultrasound to have severe micrognathia and, after birth, abnormal ears with canal stenosis and non-contiguous lobules located dorsally to the rest of the pinnae, normal zygomata, severe jaw immobility and microstomia with an opening of only 4 to 5 mm, hypoplastic tongue, and cleft palate. The 21-year-old mother of patient 1 was born with severe micrognathia requiring tracheostomy, microglossia, cleft palate with filiform alveolar bands, abnormal pinnae, and decreased conductive hearing. Dysgnathia is thought to result from a defect in the development of the first branchial arch. A similar phenotype has been seen in Otx2 haplo-insufficiency and endothelin-1 homozygous null mice, suggesting that these genes contribute to branchial arch development. Our report of a long-surviving mother and her daughter with non-syndromal dysgnathia may lead to identification of the molecular basis of these findings and provide insight into the genetics of first branchial arch formation. The survival of patient 1 and patient 2 beyond the neonatal period has implications for improvements in prenatal diagnosis and counseling and for neonatal treatment of this condition.- - - - - - - - - - ranking = 1.7909618675771keywords = hypoplasia (Clic here for more details about this article) |
6/40. osteogenesis imperfecta and orthognathic surgery: case report with long-term follow-up.osteogenesis imperfecta (OI) is the product of the abnormal synthesis and/or production of Type I collagen. Successful surgical management of extremity and spinal skeletal problems secondary to OI is documented in the orthopedic literature. Reports of successful facial skeletal surgery in all types of OI are encouraging. The purpose of this paper is to report on the long-term results of an orthognathic surgery patient successfully treated to correct a severe dentofacial deformity. The patient underwent an uncomplicated Le Fort I osteotomy with homologous interpositional bone grafts to advance and inferiorly reposition the maxilla. Clinically, the patient appeared to heal without difficulty, and a stable Class I skeletal and dental relationship was achieved. Nine years after surgery, the patient has a Class I occlusion, with maintenance of his facial height and skeletal relationship. Craniomaxillofacial surgery can be predictably performed in patients with OI as long as the surgeon maintains strict adherence to proper surgical technique and bears in mind the deficiencies of bone density and other possible medical complications.- - - - - - - - - - ranking = 0.5keywords = dental (Clic here for more details about this article) |
7/40. "Distraction" of grafted alveolar bone in cleft case using endosseous implant.OBJECTIVE: Presentation of a specific surgical technique of vertical alveolar ridge distraction performed on an alveolar bone graft 1 year after the primary grafting procedure designed to correct a labioalveolar cleft. The graft had not obtained the results desired and presented heavily scarred mucosal tissue. TECHNIQUE: A dental implant placed within the graft once it has been entirely freed by osteotomies functions as an endless screw. To avoid vascular risk, the alveolar bone graft is detached as a whole along its total height. Consequently, no bone remains above it to provide support for a conventional alveolar distractor. An osteosynthetic miniplate, fastened in a horizontal position beneath the nasal mucosae above the graft, is used to replace the missing bony support and to stabilize the implant, which activates the distraction process. PATIENT: This study is based on our first clinical case with a follow-up of 18 months at present. RESULT: A vertical displacement of the entire graft was achieved, including its attached mucosal layer, which repositioned both bone and mucosa on a far better level in the cleft area. Thus, a normal length of abutment was obtained permitting prosthetic rehabilitation based on the same endosseous implant, which was left in place within the graft. CONCLUSION: This technique may prove particularly helpful in certain cases in which a primary alveolar bone grafting procedure has produced borderline results. In such cases, on the one hand, neither the reasonably satisfactory volume of the bone graft itself nor the poor quality of its scarred mucosal tissue argue in favor of a secondary grafting procedure. On the other hand, it is impossible to resort to currently available alveolar distracters since our choice of techniques leaves no bone support above the graft.- - - - - - - - - - ranking = 0.5keywords = dental (Clic here for more details about this article) |
8/40. Larsen's syndrome with dental anomalies: report of a case.Dental anomalies and mixed-type hearing loss are extremely rare symptoms of Larsen's syndrome that is characterized by multiple joint dislocation and flat face. A 15-year-old Turkish girl affected with Larsen's syndrome exhibiting many characteristic facial and skeletal features plus hitherto unreported oral and oral-facial anomalies including, maxillary prognathi, malocclusion, supernumerary teeth, macroglossia and microdontia is reported.- - - - - - - - - - ranking = 2keywords = dental (Clic here for more details about this article) |
9/40. Hemifacial microsomia and hypodontia: a case report.A 6-year-old girl with hemifacial microsomia was examined. Abnormalities found were: severely malformed pinna of the right ear, right malar hypoplasia and unilateral mandibular hypoplasia. Dental examination revealed normal intraoral soft tissues with all deciduous teeth present. Radiographic examination disclosed that the maxillary and mandibular right and left second premolars were not developing. The mandibular ramus was short in length and the mandibular condyle had not developed on the right. The patient had no renal, cardiac or skeletal anomalies and her hearing was normal. No previous publications have been located which report the association of hypodontia and hemifacial microsomia.- - - - - - - - - - ranking = 3.5819237351541keywords = hypoplasia (Clic here for more details about this article) |
10/40. Lacrimo-auriculo-dento-digital syndrome. Case report, review of the literature, and clinical spectrum.Lacrimo-auriculo-dento-digital (LADD) syndrome, also known as Levy-Hollister syndrome, is characterized by pronounced dysplasias in various organ systems. The resulting disturbances affect primarily the lacrimal glands, the inner and outer ear, the salivary glands, and the osseous framework. Although the degree to which the different organs are involved varies considerably, dental anomalies are regularly reported. An autosomal-dominant trait has been recognized for this syndrome, but the majority of cases described in the literature concerned sporadic new mutations. We report on this unusual and relatively unknown syndrome with reference to a 13-year-old girl suffering from LADD syndrome presenting symptoms of severe xerostomia and various craniofacial malformations, hearing loss, enamel dysplasia, microdontia, and hypodontia.- - - - - - - - - - ranking = 2.2881557173715keywords = enamel, dental (Clic here for more details about this article) |
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