1/21. Treatment planning and biomechanics of distraction osteogenesis from an orthodontic perspective.As in traditional combined surgical and orthodontic procedures, the orthodontist has a role in the planning and orthodontic support of patients undergoing distraction osteogenesis. This role includes predistraction assessment of the craniofacial skeleton and occlusal function in addition to planning both the predistraction and postdistraction orthodontic care. Based on careful clinical evaluation, dental study models, photographic analysis, cephalometric evaluation, and evaluation of three-dimensional computed tomographic scans, the orthodontist, in collaboration with the surgeon, plans distraction device placement and the predicted vectors of distraction. Both surgeon and orthodontist closely monitor the patient during the active distraction phase, using intermaxillary elastic traction, sometimes combined with guide planes, bite plates, and stabilization arches, to mold the newly formed bone (regenerate) while optimizing the developing occlusion. Postdistraction change caused by relapse is minimal. growth after mandibular distraction is variable and appears to be dependent on the genetic program of the native bone and the surrounding soft tissue matrix. A significant advantage of distraction osteogenesis is the gradual lengthening of the soft tissues and surrounding functional spaces. Distraction osteogenesis can be applied at an earlier age than traditional orthognathic surgery because the technique is relatively simple and bone grafts are not required for augmentation of the hypoplastic craniofacial skeleton. In this new technique, the surgeon and the orthodontist have become collaborators in a process that gradually alters the magnitude and direction of craniofacial growth.- - - - - - - - - - ranking = 1keywords = craniofacial (Clic here for more details about this article) |
2/21. Bilateral congenital choanal atresia and absence of respiratory distress.Bilateral congenital choanal atresia is considered a lethal congenital malformation in an obligatory nasal breathing neonate. Described herein are two cases of bilateral choanal atresia associated with craniofacial anomalies who did not present respiratory distress in the neonatal period. Our first patient had a complete unilateral cleft lip which facilitated oropharyngeal respiration. The second patient presented wory distress in the neonatal period by providing an oropharyngeal airway.- - - - - - - - - - ranking = 0.33333333333333keywords = craniofacial (Clic here for more details about this article) |
3/21. Moulding of the generate to control open bite during mandibular distraction osteogenesis.Distraction osteogenesis of the craniofacial skeleton has become a widely accepted, safe, and effective means of craniofacial reconstructive surgery. Despite excellent results in general, there are still some uncertainties related to the procedure, such as development of an anterior open bite (AOB) during mandibular distraction. The aim of this study was to examine whether 'moulding of the generate', i.e. use of intermaxillary elastics during the active distraction phase is possible to close the mandibular plane angle and open bite. Three subjects, 13- and 15-year-old males and a 7-year-old female, underwent mandibular linear and angular bilateral distraction osteogenesis with moulding of the generate. Lateral cephalograms were obtained before the introduction of elastics and following distraction, once the activation was stopped and the patients were ready for the consolidation phase. Conventional cephalometric measurements were used to assess possible changes in the mandibular plane angle and incisor position. Three different anchorage systems (dental, orthopaedic, and skeletal) were used for placement of the intermaxillary elastics. Cephalometric examination showed that the mandibular plane angle was decreased during active distraction osteogenesis with the introduction of elastics and angulation of the distraction device. Depending on the type of elastic anchorage system, smaller or greater amounts of extrusion of the incisors were noted. Moulding of the generate during active distraction can be performed to reduce the mandibular plane angle and open bite. To prevent unwanted dentoalveolar changes from occurring during elastic traction, skeletal rather than dental fixation of the elastics is recommended. Intrusive mechanics may be incorporated into the orthodontic appliances to balance extrusive force by the moulding elastics.- - - - - - - - - - ranking = 0.66666666666667keywords = craniofacial (Clic here for more details about this article) |
4/21. Pneumosinus dilatans as the aetiology of progressive bilateral blindness.Pneumosinus Dilatans is a rare condition of the craniofacial skeleton which was diagnosed in an adolescent male who presented with progressive bilateral blindness and many features of osteodysplasty (Melnick-needles syndrome). The clinical course and unusual pathology of this case which included the compression of both optic nerves within long tubes of bone are described, together with the surgical intervention performed to arrest the patient's loss of vision.- - - - - - - - - - ranking = 0.33333333333333keywords = craniofacial (Clic here for more details about this article) |
5/21. Percutaneous bone-anchored hearing aid.patients born with severe dysmorphology involving the ears usually have hearing derangements as well as other areas of the craniofacial skeleton affected. To correct the functional problem, the usual and customary treatment is augmentation of the hearing with a bone conducting hearing device. The patients have to wear these devices with an external band. The new advances in osseointegrated implant allowed us today to utilize the same technology in the patient with the application of a bone anchored hearing device. The advantages are related to the obviation of the need to use a hearing band across the head, that most children object to, and the new device is much smaller than the large and cumbersome banded device. There is also an added advantage in the improvement of the hearing as the units are anchored internally in the bone. The device utilizes digital technology and can be calibrated easily. The patient can apply the device in the morning by a simple application click. The disadvantage to the patient is that the area requires consistent maintenance and care, however, this operation can be done very easily. A sleeper osseointegrated unit is kept as a spare that can be utilized if any osseo-integrated functional problem appears. The functional changes in the hearing and the audiological improvements are well documented.- - - - - - - - - - ranking = 0.33333333333333keywords = craniofacial (Clic here for more details about this article) |
6/21. mosaicism of a TCOF1 mutation in an individual clinically unaffected with Treacher Collins syndrome.Treacher Collins syndrome (TCS) or mandibulofacial dysostosis is an autosomal dominant disorder of craniofacial development with 60% of its cases arising de novo. Other modes of inheritance such as autosomal recessive, gonadal mosaicism, and chromosomal rearrangement have also been proposed. This syndrome can result from TCOF1 gene mutations. In this study we identified a TCOF1 1408delAG heterozygous mutation in a patient with the clinical diagnosis of TCS. This same mutation was found in the clinically unaffected mother's leukocytes, hair root bulbs, buccal mucosa, urine, and stool. The mother has a clinically unaffected child and the maternal grandparents do not have the mutation. Because the mother has the mutation in cells derived from all three germ layers, we suspected the mutation was nonpenetrant. However, we could not detect the mutation in her skin fibroblasts, suggesting she is mosaic secondary to cell type specific selection.- - - - - - - - - - ranking = 0.33333333333333keywords = craniofacial (Clic here for more details about this article) |
7/21. Nager syndrome (preaxial acrofacial dysostosis): a case report.The Nager syndrome is a rare condition associated with craniofacial malformations such as micrognathia, zygomatic hypoplasia, cleft palate, and preaxial limb deformities. This report features a case of the Nager syndrome occurring in a 4-year-old boy showing microdontia, thumb duplication and radioulnar synostosis, and ventricular septum defect, characteristics not usually encountered in the published cases.- - - - - - - - - - ranking = 0.33333333333333keywords = craniofacial (Clic here for more details about this article) |
8/21. Management of the difficult pediatric airway with Shikani Optical Stylet.The airways of children with many craniofacial and mandibulofacial malformations often present unique challenges to the anesthesiologists. We report the application of Shikani Optical Stylet (SOS) in facilitating the tracheal intubation of four children with history of difficult airway management. The SOS combines the benefits of the lightwand and fiberoptic bronchoscope.- - - - - - - - - - ranking = 0.33333333333333keywords = craniofacial (Clic here for more details about this article) |
9/21. Treacher Collins syndrome with choanal atresia: a case report and review of disease features.Treacher Collins syndrome--or mandibulofacial dysostosis--is a rare condition that presents several craniofacial deformities of different levels. This is a congenital malformation involving the first and second branchial arches. incidence is estimated to range between 1-40,000 to 1-70,000 of live births. The disorder is characterized by abnormalities of the auricular pinna, hypoplasia of facial bones, antimongoloid slanting palpebral fissures with coloboma of the lower eyelids and cleft palate. Treacher Collins syndrome is rarely associated with choanal atresia. A multidisciplinary team, including craniofacial surgeon, ophthalmologist, speech therapist, dental surgeon and otorhinolaryngologist, is the most appropriate setting to manage these patients. This study reports a rare case of Treacher Collins syndrome with choanal atresia, presenting literature review and multidisciplinary intervention.- - - - - - - - - - ranking = 0.66666666666667keywords = craniofacial (Clic here for more details about this article) |
10/21. Treacher Collins syndrome with a de Novo 5-bp deletion in the TCOF1 gene.Treacher Collins syndrome (TCS) is an autosomal dominant disorder of craniofacial development with features including malar hypoplasia, micrognathia, microtia, downward slanting palpebral fissures, lower eyelid coloboma, conductive hearing loss, and cleft palate. TCS is caused by mutations in the TCOF1 gene, which encodes the nuclear phosphoprotein treacle. Here, we describe a 1-day-old male infant with classical TCS presentation. A 5-bp deletion in exon 22 of the TCOF1 gene (3469del ACTCT) was found to cause a premature stop codon. This is the first report of TCOF1 gene mutation in the Taiwanese population.- - - - - - - - - - ranking = 0.33333333333333keywords = craniofacial (Clic here for more details about this article) |
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