11/35. New oral findings in Cohen syndrome.Cohen syndrome is a hereditary disorder transmitted as an autosomal-recessive trait. Approximately 100 cases have been reported in the genetic and pediatric literature. Despite the fact that oral alterations are often observed in these cases, only 1 work has been published addressing this specific topic, and it tended to concentrate on periodontal abnormalities. The present study details 2 new patients, 2 brothers (8 and 11 years old), and mainly consists of an analysis of the dentomaxillary anomalies that until now have not been studied in depth. In this study, the mandible, characterized as hypoplastic in Cohen syndrome, appears to be in a normal position; what really exists is a maxillary hyperplasia of genetic origin. We also put forward an observation hitherto undescribed in the literature: dental agenesis.- - - - - - - - - - ranking = 1keywords = mandible (Clic here for more details about this article) |
12/35. Craniofacial morphology in a patient with Simpson-Golabi-Behmel syndrome.OBJECTIVE: We present the case of a 6-year-old boy with a coarse face, cleft palate, and malocclusion with anterior open bite who had been diagnosed with Simpson-Golabi-Behmel syndrome. Morphology of the craniofacial structures was examined on the basis of conventional radiographs, three-dimensional (3D) computed tomography (CT) and magnetic resonance (MR) scanning. PATIENT: This patient had 13 ribs on the right side, slight scoliosis, supernumerary nipples, a coarse face, hypertelorism, a short broad upturned nose, a wide mouth, a straight facial profile with incompetence of the lips, midline groove of tongue, and cleft palate. The patient also had severe anterior open bite, a distal step-type molar relationship, five congenitally missing teeth, and a supernumerary tooth. Lateral cephalometric analysis revealed a large anterior cranial base, a large maxilla and mandible, a large inferior face height, and skeletal Class I jaw relationship with a high mandibular plane angle and large gonial angle. The 3D CT image showed a large cranium, a long face height, and prominent skull sutures. The MR image showed a large tongue, midline groove of the tongue, and a small space between tongue and palate.- - - - - - - - - - ranking = 1.0645879591877keywords = mandible, jaw (Clic here for more details about this article) |
13/35. Advances in distraction techniques for craniofacial surgery.Distraction osteogenesis has been applied to the craniofacial skeleton as well as the long bones of the extremities. This technique does not require bone grafting and allows correction of craniofacial deformities with less invasion. Moreover, the distraction procedures can expand the overlying soft tissues simultaneously. We determined the indications of distraction osteogenesis, analyzed the types of devices available, and examined patients treated with distraction for the mandible, midface, and cranium. In all three sites, the devices tended to be the buried type and made of absorbable materials. Administration of some cytokines for shortening the consolidation period may be considered. Among disorders indicated for distraction osteogenesis, there are several syndromic craniosynostoses, which involve mutations in the fibroblast growth factor receptor (FGFR) 2 gene. The FGFR 2 mutation was suggested to clinically accelerate osteogenesis at the distraction site. The usefulness and appropriateness of the distraction protocol must be assessed for each individual disorder. Although distraction osteogenesis in the craniofacial skeleton has advanced technologically, all possible risks must be discussed with the patient and family members when obtaining preoperative informed consent, especially until establishment of fully safe distraction procedures.- - - - - - - - - - ranking = 1keywords = mandible (Clic here for more details about this article) |
14/35. Molding of the regenerate in mandibular distraction: clinical experience.Initial clinical experience with distraction osteogenesis has demonstrated the risk of developing postdistraction malocclusion that requires secondary orthodontic correction. In addition, optimal mandibular form is not always achieved. Both animal studies and preliminary clinical investigations have suggested that the regenerate can be successfully "molded" during active mandibular distraction. The authors have applied this concept clinically to obtain a more desirable occlusal relationship in a group of mandibular distraction patients. Eleven patients are described in whom angulation of the distraction device or intermaxillary/interdental elastics were employed to mold the regenerate. Two representative case studies are provided to illustrate the principles. When using elastic traction to close an anterior open bite, care must be taken that extrusion of individual teeth is minimized by distributing the force over the entire dental arch, especially the basilar portions of the jaws. The authors demonstrate that molding of the regenerate can be successfully accomplished not only during device activation but also early in the consolidation period. The outer limit of the time window in which molding is effective remains to be defined.- - - - - - - - - - ranking = 0.064587959187727keywords = jaw (Clic here for more details about this article) |
15/35. Craniofacial morphology in an unusual case with nasal aplasia studied by roentgencephalometry and three-dimensional CT scanning.OBJECTIVE: To examine the three-dimensional morphology of internal structures of the craniofacial region and present the orthodontic problems in an unusual case with nasal aplasia. PATIENT: The patient was an 11.5-year-old boy with aplasia of the nose and nasal cavity with extremely constricted nasopharyngeal airway. He did not have mental or somatic retardation. The patient had dacryostenosis. The morphology of the craniofacial structures was characterized by absence of septal structures, including cribriform plate, perpendicular plate of ethmoid bone, vomer, and septal cartilage; bony hypotelorism; midface hypoplasia; short and retrognathic maxilla with Class III jaw relationship; average mandibular plane angle; high arched palate; severe anterior open bite with bilateral posterior crossbites; and dental anomalies (agenesis of four maxillary permanent teeth, microdontia, taurodontism, and short roots). Thus, the patient had characteristic dentofacial phenotype, which might be caused by a combination of the primary anomaly and the functional disturbances secondary to the nasal obstruction.- - - - - - - - - - ranking = 0.064587959187727keywords = jaw (Clic here for more details about this article) |
16/35. The dentofacial features of Sanjad-Sakati syndrome: a case report.Sanjad-Sakati syndrome is an autosomal recessive disorder that was first reported by Sanjad et al. in 1988. It is characterized by congenital hypoparathyroidism, severe growth failure and dysmorphic features. The clinical features include deep set eyes, microcephaly, thin lips, depressed nasal bridge with beaked nose, external ear anomalies and learning difficulties. This report presents the case of a 4-year-old patient who was referred to our dental clinic because of pain in her mouth and poor dental health. Oral findings included micrognathic mandible and maxilla, microdontia, enamel hypoplasia as well as severely decayed teeth. Treatment was carried out under general anaesthesia to extract the most severely affected teeth and restore those which could be conserved. It was concluded that these patients have special dental needs; early diagnosis of the affected children is therefore important in order to commence preventive dental therapy and carry out appropriate dental treatment at the optimum time.- - - - - - - - - - ranking = 1keywords = mandible (Clic here for more details about this article) |
17/35. Amniotic band sequence: the use of bone grafting and distraction osteogenesis.Distraction has proven to be a useful technique in cases of severe bone deficiency in the craniofacial skeleton. This is a report of its use in the treatment of a child affected by amniotic band sequence. This 5 year old Hispanic female presented with transverse deficiences of one lower extremity and both upper extremities. Additionally, she was missing the mandible and overlying soft tissue from ramus to ramus. Although free fibula transfer was a possibility, due to the presence of only one lower extremity, it was elected to utilize the technique of distraction. After preliminary bone grafting to unite these two hemimanibles, the bone graft was distracted bilaterally to create a neomandible. Following consolidation and removal of the distraction devices, the child was markedly improved despite what appeared to be a pathologic fracture in the region of the mandibular angle. Repeat distraction was planned.- - - - - - - - - - ranking = 2keywords = mandible (Clic here for more details about this article) |
18/35. airway management during an EXIT procedure for a fetus with dysgnathia complex.Nonsyndromal dysgnathia is a rare disorder with a probable genetic basis characterized by a hypoplastic or absent mandible (agnathia), microstomia, microglossia, and ear anomalies secondary to a defect in the ventral portion of the first branchial arch caused by defective neural crest migration or proliferation. Dysgnathic newborn infants often suffer fatal respiratory failure from airway obstruction. Nineteen children with isolated dysgnathia complex are described in the literature--six were stillborn, eight died shortly after birth, and only five survived infancy. All survivors required tracheostomy to maintain an airway. It is difficult to intubate the trachea of these children and early airway management planning is important. We report a neonate who presented with a prenatal ultrasound diagnosis of severe micrognathia, polyhydramnios and a family history of severe micrognathia. airway management was achieved with fiberoptic intubation through a laryngeal mask airway (LMA) during an ex utero intrapartum treatment procedure. Fiberoptic intubation was hampered by copious amounts of amniotic fluid. This child and her sibling are the first two siblings with isolated dysgnathia complex to have survived infancy and provide further support for a genetic basis to this condition.- - - - - - - - - - ranking = 1keywords = mandible (Clic here for more details about this article) |
19/35. Craniometaphyseal dysplasia associated with obstructive sleep apnoea syndrome.Craniometaphyseal dysplasia (CMD) is a genetic craniotubular bone disorder characterized by early progressive hyperostosis and sclerosis of the craniofacial bones, and abnormal modelling of the metaphyses of the tubular bones. We present the case of a patient with a confirmed history of the autosomal dominant form of CMD, associated with symptoms of obstructive sleep apnoea syndrome. Examination and imaging studies revealed several unusual features in addition to the common findings of CMD such as: bimaxillary retrusion with hyperostosis of the mental area, severe notching of the external occipital protuberance, huge occipital horn, decreased angle of the mandible with notching of the body and thickening of the areas of muscle attachment, and macrodontia. The literature and differential diagnoses are reviewed.- - - - - - - - - - ranking = 1keywords = mandible (Clic here for more details about this article) |
20/35. Velocardiofacial syndrome with single central incisor.Three siblings and their mother are reported who all had cytogenetically proven velocardiofacial syndrome (VCFS). One boy had normal dental and craniofacial findings, except for an increased cranial base angle. His sister had only one central incisor in the maxilla. One central incisor had also been missing in the primary dentition. She had no labial frenulum present. cephalometry showed a small maxillary unit length indicating mild maxillary hypoplasia, an increased anterior face height, steep mandibular plane angle, retruded chin, and a large cranial base angle. Dental measurements showed retroclined lower incisors and increased interincisal angle. A second sister had a cleft of the secondary palate. All permanent teeth were present with the exception of a missing central incisor in the lower jaw: the single lower central incisor was situated in the midline. Her cephalometry showed similar findings as in her sister. All three siblings required palate surgery for speech. Mother was not available for detailed dental and other oral investigations. A single maxillary central incisor has previously been reported in VCFS, but to our knowledge a single central incisor in the mandible has not been reported previously in this entity.- - - - - - - - - - ranking = 1.0645879591877keywords = mandible, jaw (Clic here for more details about this article) |
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